[ { "text": "(5)房室结消融和起搏器植入作为反复发作或难治性心房内折返性心动过速的替代疗法。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "房室结消融" }, { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "起搏器植入" }, { "start_idx": 16, "end_idx": 33, "type": "dis", "entity": "反复发作或难治性心房内折返性心动过速" } ] }, { "text": "(6)发作一次伴血流动力学损害的室性心动过速(ventriculartachycardia),可接受导管消融者。", "entities": [ { "start_idx": 8, "end_idx": 21, "type": "dis", "entity": "血流动力学损害的室性心动过速" }, { "start_idx": 23, "end_idx": 44, "type": "dis", "entity": "ventriculartachycardia" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "导管消融" } ] }, { "text": "4.第三类(1)无症状性WPW综合征患者,年龄小于5岁。", "entities": [ { "start_idx": 8, "end_idx": 17, "type": "dis", "entity": "无症状性WPW综合征" } ] }, { "text": "(2)室上性心动过速可用常规抗心律失常药物控制,年龄小于5岁。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "室上性心动过速" } ] }, { "text": "(3)非持续性,不考虑为无休止性的阵发性室性心动过速(即一次监视数小时或任何一小时记录的心电图条带几乎均可出现),心室功能正常。", "entities": [ { "start_idx": 3, "end_idx": 25, "type": "dis", "entity": "非持续性,不考虑为无休止性的阵发性室性心动过速" }, { "start_idx": 44, "end_idx": 46, "type": "pro", "entity": "心电图" } ] }, { "text": "(4)非持续性室上性心动过速,不需其他治疗和(或)症状轻微。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "非持续性室上性心动过速" } ] }, { "text": "第十章胸膜疾病小儿胸膜疾病以胸膜炎最为常见,多继发于肺部感染,原发性或其他原因所致者较少见。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "胸膜疾病" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "小儿胸膜疾病" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "胸膜炎" } ] }, { "text": "第一节胸膜炎胸膜炎(pleurisy)分为三种:干性胸膜炎、浆液性胸膜炎和化脓性胸膜炎。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "胸膜炎" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "胸膜炎" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "pleurisy" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "干性胸膜炎" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "浆液性胸膜炎" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "化脓性胸膜炎" } ] }, { "text": "一、干性胸膜炎干性胸膜炎(dryorplasticpleurisy)又称纤维素性胸膜炎,常与肺部细菌感染有关,亦可发生于急性上呼吸道疾病过程中。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "干性胸膜炎" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "干性胸膜炎" }, { "start_idx": 13, "end_idx": 32, "type": "dis", "entity": "dryorplasticpleurisy" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "纤维素性胸膜炎" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "肺部细菌感染" }, { "start_idx": 60, "end_idx": 67, "type": "dis", "entity": "急性上呼吸道疾病" } ] }, { "text": "结缔组织疾病如风湿热患儿亦可发生。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "结缔组织疾病" } ] }, { "text": "深呼吸及咳嗽时疼痛加剧。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "深呼吸及咳嗽时疼痛加剧" } ] }, { "text": "病程早期可闻胸膜摩擦音在全部呼吸期间均可听到。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "闻胸膜摩擦音" }, { "start_idx": 11, "end_idx": 21, "type": "sym", "entity": "在全部呼吸期间均可听到" } ] }, { "text": "胸部X线透视和胸片可见患侧膈呼吸运动减弱肋膈角变钝流行性胸痛和带状疱疹前驱期的胸痛及肋骨骨折相鉴别。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "胸部X线透视" }, { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "胸片" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "患侧膈呼吸运动减弱" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "肋膈角变钝" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "流行性胸痛" }, { "start_idx": 31, "end_idx": 40, "type": "sym", "entity": "带状疱疹前驱期的胸痛" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "肋骨骨折" } ] }, { "text": "同时应分析胸膜炎的原因,注意肺部有无炎症,并进行必要的检查,尤其注意排除结核病。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "胸膜炎" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肺部" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "结核病" } ] }, { "text": "如非肺炎病例,宜用宽大胶布条紧缠患部以减少其呼吸动作或给镇咳剂抑制咳嗽。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "pro", "entity": "宽大胶布条紧缠患部" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "镇咳剂" } ] }, { "text": "肺炎患儿则不宜采用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肺炎" } ] }, { "text": "第十三节狂犬病狂犬病(rabies)又称恐水症(hydrophobia),是由狂犬病毒引起的中枢神经系统急性传染病,为人畜共患的自然疫源性疾病。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "狂犬病" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "狂犬病" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "rabies" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "恐水症" }, { "start_idx": 24, "end_idx": 34, "type": "dis", "entity": "hydrophobia" }, { "start_idx": 39, "end_idx": 42, "type": "mic", "entity": "狂犬病毒" }, { "start_idx": 46, "end_idx": 56, "type": "dis", "entity": "中枢神经系统急性传染病" }, { "start_idx": 64, "end_idx": 70, "type": "dis", "entity": "自然疫源性疾病" } ] }, { "text": "临床上以恐水、怕风、咽肌痉挛、进行性瘫痪为特征,病死率极高。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "恐水" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "怕风" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "咽肌" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "咽肌痉挛" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "瘫痪" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "进行性瘫痪" } ] }, { "text": "【病原和流行病学】狂犬病病毒(rabiesvirus)属弹状病毒科狂犬病病毒属。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "狂犬病病毒" }, { "start_idx": 15, "end_idx": 25, "type": "mic", "entity": "rabiesvirus" }, { "start_idx": 28, "end_idx": 31, "type": "mic", "entity": "弹状病毒" }, { "start_idx": 33, "end_idx": 37, "type": "mic", "entity": "狂犬病病毒" } ] }, { "text": "病毒基因为单负链RNA。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "病毒基因" } ] }, { "text": "狂犬病病毒能在多种细胞中生长,但绝大多数毒株不引起细胞病变(长期传代培养适应后才出现),故常采用免疫酶技术或动物接种试验检测病毒增殖。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "狂犬病病毒" }, { "start_idx": 48, "end_idx": 52, "type": "pro", "entity": "免疫酶技术" }, { "start_idx": 54, "end_idx": 59, "type": "pro", "entity": "动物接种试验" }, { "start_idx": 62, "end_idx": 63, "type": "mic", "entity": "病毒" } ] }, { "text": "在狂犬患者和动物神经细胞内存在狂犬病病毒特有的胞质内包涵体,又称内基小体(Negribodies)。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "mic", "entity": "狂犬病病毒" } ] }, { "text": "病毒对理化因素抵抗力较低,56℃30分钟或100℃2分钟、强酸、强碱、甲醛、升汞、脂溶剂、季铵类化合物都能很快杀灭之;紫外线和直射阳光可迅速降低病毒活力。", "entities": [ { "start_idx": 72, "end_idx": 73, "type": "mic", "entity": "病毒" } ] }, { "text": "狂犬病的传染源主要是犬,其次是猫和狼,其他野生动物如狐、浣熊、吸血蝙蝠也能传播本病。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "狂犬病" } ] }, { "text": "患病或带毒动物唾液中有大量病毒,通过咬伤、抓伤和舔伤皮肤黏膜而侵入,偶经食入带毒肉类而感染。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "病毒" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "皮肤黏膜" } ] }, { "text": "人群普通易感,被病犬咬伤而未预防接种者发病率为10%~70%,病死率近100%。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "咬伤" } ] }, { "text": "狂犬病在全球2/3的国家和地区流行。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "狂犬病" } ] }, { "text": "我国在20世纪50年代发病率很低,近几年随着“宠物热”的升温,发病率逐年增高,近5年全国狂犬病年疫报人数由160人(1996年)上升至899人(2001年),年死亡人数由140人(1996年)上升至862人(2001年)。", "entities": [ { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "狂犬病" } ] }, { "text": "【发病机制和病理改变】狂犬病病毒的靶细胞是神经细胞和肌细胞。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "狂犬病病毒" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "神经细胞" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "肌细胞" } ] }, { "text": "侵入后先在局部神经末梢或在附近肌细胞中增殖后再侵入神经末梢,沿周围传入神经轴索上行至脊髓前背根神经大量增殖,然后侵入脊髓和中枢神经系统,主要侵犯脑干、基底节、海马回及小脑等处神经元,引起弥漫性脑脊髓病变;再沿传出神经侵入各组织器官继续复制。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "局部神经末梢" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肌细胞" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "神经末梢" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "传入神经轴索" }, { "start_idx": 42, "end_idx": 48, "type": "bod", "entity": "脊髓前背根神经" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "脊髓" }, { "start_idx": 61, "end_idx": 66, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "脑干" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "基底节" }, { "start_idx": 79, "end_idx": 84, "type": "bod", "entity": "海马回及小脑" }, { "start_idx": 87, "end_idx": 89, "type": "bod", "entity": "神经元" }, { "start_idx": 93, "end_idx": 100, "type": "dis", "entity": "弥漫性脑脊髓病变" }, { "start_idx": 104, "end_idx": 107, "type": "bod", "entity": "传出神经" } ] }, { "text": "由于迷走神经、舌咽神经核及舌下神经核受损伤,可发生呼吸肌及吞咽肌痉挛。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "迷走神经" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "舌咽神经核" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "舌下神经核" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "吞咽肌" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "呼吸肌及吞咽肌痉挛" } ] }, { "text": "交感神经受累时,可致唾液分泌和出汗增多。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "交感神经" }, { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "交感神经受累" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "唾液" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "汗" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "唾液分泌和出汗增多" } ] }, { "text": "延髓和脊髓受损,则可引起各种类型的瘫痪。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "延髓" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "脊髓" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "延髓和脊髓受损" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "瘫痪" } ] }, { "text": "最终因脑实质损伤导致呼吸和循环衰竭而死亡。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "脑实质损伤" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "呼吸和循环衰竭" } ] }, { "text": "病毒侵入靶细胞的机制与病毒结合乙酰胆碱受体或其他受体有关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "病毒" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "乙酰胆碱受体" } ] }, { "text": "病毒抗原诱导的特异性中和抗体、特异性细胞免疫及其分泌的细胞因子,特别是干扰素在抗狂犬病病毒免疫中起重要作用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "病毒抗原" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "干扰素" }, { "start_idx": 40, "end_idx": 44, "type": "mic", "entity": "狂犬病病毒" } ] }, { "text": "主要病理改变为脑实质和脑膜水肿、充血,微小血管出血,尤以大脑海马、延髓、脑桥、小脑和咬伤部位相应的背根节及脊髓段最为严重。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "脑实质" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脑膜" }, { "start_idx": 7, "end_idx": 17, "type": "sym", "entity": "脑实质和脑膜水肿、充血" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "微小血管" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "微小血管出血" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "大脑海马" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "延髓" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "脑桥" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "小脑" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "背根节" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "脊髓段" } ] }, { "text": "显微镜下见神经细胞空泡形成,透明变性和染色体分解小神经胶质细胞浸润,血管周围单核细胞及浆细胞浸润。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "显微镜" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "神经细胞" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "神经细胞空泡" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "透明变性" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "染色体" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "染色体分解" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "小神经胶质细胞" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "小神经胶质细胞浸润" }, { "start_idx": 34, "end_idx": 41, "type": "bod", "entity": "血管周围单核细胞" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "浆细胞" }, { "start_idx": 34, "end_idx": 47, "type": "sym", "entity": "血管周围单核细胞及浆细胞浸润" } ] }, { "text": "70%~80%患者的神经细胞质中可发现内基小体,呈圆形或卵圆形,直径3~10μm,由狂犬病病毒核糖核蛋白聚集而成,有特异性诊断价值。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "神经细胞质" }, { "start_idx": 0, "end_idx": 39, "type": "sym", "entity": "70%~80%患者的神经细胞质中可发现内基小体,呈圆形或卵圆形,直径3~10μm" }, { "start_idx": 42, "end_idx": 51, "type": "mic", "entity": "狂犬病病毒核糖核蛋白" } ] }, { "text": "影响潜伏期的因素为年龄(儿童较短)、伤口部位(头面部发病早)、伤口性质(深咬伤较短)和入侵病毒的数量、毒力及宿主防御机制等。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "头面部" }, { "start_idx": 45, "end_idx": 46, "type": "mic", "entity": "病毒" } ] }, { "text": "(一)前驱期持续1~4天,常有低热、头痛、乏力、咽痛、腹痛、烦躁等。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "低热" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "头痛" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "乏力" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "咽痛" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "腹痛" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "烦躁" } ] }, { "text": "继之对强光、高声等刺激敏感而有咽喉紧迫感,进食时咽喉肌轻度痉挛,但尚能吞咽。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "咽喉" }, { "start_idx": 2, "end_idx": 19, "type": "sym", "entity": "对强光、高声等刺激敏感而有咽喉紧迫感" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "咽喉肌" }, { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "进食时咽喉肌轻度痉挛" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "尚能吞咽" } ] }, { "text": "约80%的患者伤口局部及其神经通路上有放射性疼痛、麻木、痒及感觉异常。", "entities": [ { "start_idx": 7, "end_idx": 33, "type": "sym", "entity": "伤口局部及其神经通路上有放射性疼痛、麻木、痒及感觉异常" } ] }, { "text": "(二)兴奋期反射性咽喉痉挛逐渐加重,当饮水,甚至听到讲水、看到水及咽水动作,或风、光、声、烟刺激也会引起咽喉部严重痉挛,出现典型的恐水症。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "咽喉" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "咽喉痉挛逐渐加重" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "咽喉部" }, { "start_idx": 18, "end_idx": 58, "type": "sym", "entity": "当饮水,甚至听到讲水、看到水及咽水动作,或风、光、声、烟刺激也会引起咽喉部严重痉挛" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "恐水症" } ] }, { "text": "常伴呼吸肌痉挛而发生呼吸困难,全身肌张力高,颈部强硬。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "呼吸肌痉挛" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "全身肌" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "全身肌张力高" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "颈部" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "颈部强硬" } ] }, { "text": "常出现躁狂与昏睡交替。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "躁狂" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "昏睡交替" } ] }, { "text": "发作时暴躁异常;发作间歇期则较安静,语言清晰。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "暴躁异常" }, { "start_idx": 10, "end_idx": 21, "type": "sym", "entity": "间歇期则较安静,语言清晰" } ] }, { "text": "由于交感神经亢进,有大汗、心率增快、血压升高、瞳孔扩大、唾液分泌增加等表现。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "交感神经" }, { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "交感神经亢进" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "大汗" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "心率增快" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "血压升高" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "瞳孔" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "瞳孔扩大" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "唾液" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "唾液分泌增加" } ] }, { "text": "患者可高热39~40℃,神志大多清晰,部分患者有精神失常,可在发作中死于呼吸衰竭或循环衰竭。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "高热" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "神志大多清晰" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "精神失常" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "呼吸衰竭" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "循环衰竭" } ] }, { "text": "(三)麻痹期痉挛减少或停止,患者渐安静,逐步发生全身弛缓性瘫痪,尤以肢体软瘫多见。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "痉挛减少或停止" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "渐安静" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "瘫痪" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "逐步发生全身弛缓性瘫痪" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "肢体软瘫" } ] }, { "text": "感觉减退,反射消失,呼吸变慢而不齐,心搏微弱,血压下降,神志不清,最终因呼吸麻痹和循环衰竭而死亡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "感觉减退" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "反射消失" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "呼吸变慢而不齐" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "心搏微弱" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "血压下降" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "神志不清" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "呼吸麻痹" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "循环衰竭" } ] }, { "text": "狂犬病整个病程3~5天,极少超过10天。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "狂犬病" } ] }, { "text": "极少见“麻痹型”病例,后者以高热、进行性麻痹为主,终至衰竭死亡,全病程约8~9天。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "高热" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "进行性麻痹" } ] }, { "text": "【实验室检查】(一)血、尿常规及脑脊液白细胞总数轻至中度升高,亦可高达30×109/L,以中性粒细胞占优势,可达80%以上。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "ite", "entity": "血、尿常规" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "脑脊液" }, { "start_idx": 19, "end_idx": 21, "type": "ite", "entity": "白细胞" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "中性粒细胞" } ] }, { "text": "尿常规有轻度蛋白尿,偶有透明管型。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "尿常规" }, { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "轻度蛋白尿" } ] }, { "text": "脑脊液压力在正常范围或稍增高,细胞数及蛋白量稍增多。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑脊液" } ] }, { "text": "(二)病原学检查1.病毒分离取患者唾液(病后4~24天)、脑脊液和尿沉渣(病后头2周内)或死后脑组织悬液接种敏感细胞,经检测病毒抗原鉴定病毒或脑内接种敏感动物,若接种动物在6~10天出现痉挛和麻痹或脑组织中找到内基小体即可诊断。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "病毒分离" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "唾液" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "脑脊液" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "尿沉渣" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "脑组织" }, { "start_idx": 62, "end_idx": 63, "type": "mic", "entity": "病毒" }, { "start_idx": 68, "end_idx": 69, "type": "mic", "entity": "病毒" }, { "start_idx": 73, "end_idx": 74, "type": "pro", "entity": "接种" }, { "start_idx": 93, "end_idx": 94, "type": "sym", "entity": "痉挛" }, { "start_idx": 96, "end_idx": 97, "type": "sym", "entity": "麻痹" }, { "start_idx": 99, "end_idx": 101, "type": "bod", "entity": "脑组织" }, { "start_idx": 105, "end_idx": 108, "type": "bod", "entity": "内基小体" } ] }, { "text": "2.内基小体检查取死者脑组织或咬人动物脑组织(最好是脑室底部)印压涂片或病理切片,染色直接镜检或免疫荧光法检查内基小体,阳性率为70%~80%。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "内基小体检查" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "脑组织" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "脑组织" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "脑室底部" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "印压涂片" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "病理切片" }, { "start_idx": 41, "end_idx": 46, "type": "pro", "entity": "染色直接镜检" }, { "start_idx": 48, "end_idx": 52, "type": "pro", "entity": "免疫荧光法" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "内基小体" } ] }, { "text": "3.病毒抗原检测取患者唾液、咽-气管分泌物、尿沉渣、有神经原纤维的皮肤(常取颈部发际处)活检标本,用免疫荧光法或ELISA或ELISA夹心法检测病毒抗原,具有较高敏感性和特异性。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "病毒抗原检测" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "唾液" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "咽-气管分泌物" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "尿沉渣" }, { "start_idx": 26, "end_idx": 34, "type": "bod", "entity": "有神经原纤维的皮肤" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "颈部发际" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "活检标本" }, { "start_idx": 50, "end_idx": 54, "type": "pro", "entity": "免疫荧光法" }, { "start_idx": 56, "end_idx": 60, "type": "pro", "entity": "ELISA" }, { "start_idx": 62, "end_idx": 69, "type": "pro", "entity": "ELISA夹心法" }, { "start_idx": 72, "end_idx": 75, "type": "mic", "entity": "病毒抗原" } ] }, { "text": "4.中和抗体测定患者存活一周以上可用中和试验或ELISA法检测血清中和抗体,未接种过疫苗的患者抗体水平低;接种过疫苗的患者在出现症状后6~10天中和抗体水平快速增高,滴度可≥1∶5000(疫苗免疫不能达到此水平)。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "中和抗体测定" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "中和试验" }, { "start_idx": 23, "end_idx": 28, "type": "pro", "entity": "ELISA法" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "血清" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "抗体" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "疫苗" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "疫苗" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "抗体" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "疫苗" } ] }, { "text": "病程晚期脑脊液中检出高水平抗体(疫苗不能诱导)亦有诊断意义。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "脑脊液" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "抗体" }, { "start_idx": 16, "end_idx": 17, "type": "dru", "entity": "疫苗" } ] }, { "text": "5.病毒RNA检查用斑点杂交法或RT-PCR法检测狂犬病病毒核衣壳序列,因有非特异性扩增带干扰,PCR产物需用Southern印迹法加以确认。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "病毒RNA检查" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "斑点杂交法" }, { "start_idx": 16, "end_idx": 22, "type": "pro", "entity": "RT-PCR法" }, { "start_idx": 25, "end_idx": 29, "type": "mic", "entity": "狂犬病病毒" }, { "start_idx": 48, "end_idx": 50, "type": "pro", "entity": "PCR" }, { "start_idx": 55, "end_idx": 65, "type": "pro", "entity": "Southern印迹法" } ] }, { "text": "确诊有赖于病原学检查或尸检脑组织发现内基小体。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "尸检" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "脑组织" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "内基小体" } ] }, { "text": "(二)鉴别诊断1.破伤风(tetanus)有外伤史,潜伏期短,多为6~14天。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "破伤风" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "tetanus" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "外伤" } ] }, { "text": "有牙关紧闭,角弓反张,全身阵发性强直性肌痉挛,持续时间长;而无高度兴奋和恐水现象,预后也较好。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "牙关紧闭" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "角弓反张" }, { "start_idx": 11, "end_idx": 21, "type": "sym", "entity": "全身阵发性强直性肌痉挛" }, { "start_idx": 30, "end_idx": 39, "type": "sym", "entity": "无高度兴奋和恐水现象" } ] }, { "text": "但须注意,狂犬患者被咬伤时,也可同时感染破伤风。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "狂犬" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "咬伤" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "破伤风" } ] }, { "text": "2.其他病毒所致脑炎和脑膜炎有神志改变,甚至昏迷,此与狂犬病患者神志清楚、惊恐不安的情况不同。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dis", "entity": "其他病毒所致脑炎和脑膜炎" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "神志改变" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "昏迷" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "狂犬病" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "神志清楚" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "惊恐不安" } ] }, { "text": "可通过脑脊液变化和病原学检查区别。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "脑脊液" } ] }, { "text": "【预防和治疗】(一)控制和消灭传染源加强犬等管理,野犬应尽量捕杀,家犬应登记,注射疫苗。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "pro", "entity": "控制和消灭传染源" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "加强犬等管理" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "注射疫苗" } ] }, { "text": "狂犬应立即击毙,焚毁或深埋,不可剥皮。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "pro", "entity": "狂犬应立即击毙,焚毁或深埋,不可剥皮" } ] }, { "text": "一时不能肯定为狂犬者,应隔离观察10天,取击毙或隔离期死亡动物的脑组织作病原学检查。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "隔离观察10天" }, { "start_idx": 20, "end_idx": 40, "type": "pro", "entity": "取击毙或隔离期死亡动物的脑组织作病原学检查" } ] }, { "text": "以20%肥皂水或0.1%苯扎溴铵彻底冲洗伤口至少半小时;再用白酒或70%乙醇、碘酊涂擦几次,以清除局部的病毒。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "肥皂水" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "苯扎溴铵" }, { "start_idx": 30, "end_idx": 31, "type": "dru", "entity": "白酒" }, { "start_idx": 36, "end_idx": 37, "type": "dru", "entity": "乙醇" }, { "start_idx": 39, "end_idx": 40, "type": "dru", "entity": "碘酊" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "涂擦" }, { "start_idx": 52, "end_idx": 53, "type": "mic", "entity": "病毒" } ] }, { "text": "除非伤及大血管需要紧急止血外,3天内不必包扎或缝合伤口。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "大血管" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "紧急止血" } ] }, { "text": "(三)疫苗接种疫苗接种是预防和控制狂犬病的重要措施之一。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "疫苗接种" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "疫苗接种" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "狂犬病" } ] }, { "text": "1.狂犬病病毒疫苗目前主要使用细胞培养疫苗,包括:①人二倍体细胞狂犬疫苗(humandiploidcell,HDCV):免疫原性强,不良反应很少,注射次数少,但制备困难,价格昂贵;②原代地鼠肾细胞狂犬病疫苗:效力在2.5IU以上,使用安全;③精制Vero狂犬病疫苗:免疫原性和不良反应与①相似,但价格低。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dru", "entity": "狂犬病病毒疫苗" }, { "start_idx": 19, "end_idx": 20, "type": "dru", "entity": "疫苗" }, { "start_idx": 26, "end_idx": 35, "type": "dru", "entity": "人二倍体细胞狂犬疫苗" }, { "start_idx": 37, "end_idx": 52, "type": "dru", "entity": "humandiploidcell" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "HDCV" }, { "start_idx": 91, "end_idx": 102, "type": "dru", "entity": "原代地鼠肾细胞狂犬病疫苗" }, { "start_idx": 121, "end_idx": 131, "type": "dru", "entity": "精制Vero狂犬病疫苗" } ] }, { "text": "其他有精制鸡胚狂犬病疫苗、精制鸭胚狂犬病疫苗和原代牛肾细胞狂犬病疫苗。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dru", "entity": "精制鸡胚狂犬病疫苗" }, { "start_idx": 13, "end_idx": 21, "type": "dru", "entity": "精制鸭胚狂犬病疫苗" }, { "start_idx": 23, "end_idx": 33, "type": "dru", "entity": "原代牛肾细胞狂犬病疫苗" } ] }, { "text": "2.接触前免疫对象为有职业危险者、接触狂犬病病毒的实验室工作人员及和狂犬患者密切接触者。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "mic", "entity": "狂犬病病毒" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "狂犬" } ] }, { "text": "推荐0、28日二剂和0、7、28或0、28、56日三剂接种方案,每次1.0ml肌内注射或深皮下注射,或0.1ml皮内注射。", "entities": [ { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "肌内注射" }, { "start_idx": 44, "end_idx": 48, "type": "pro", "entity": "深皮下注射" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "皮内注射" } ] }, { "text": "3.接触后免疫WHO推荐的标准免疫方案(HDCV疫苗)为0、3、7、14和28日各肌内注射1ml,第90日再加强1次。", "entities": [ { "start_idx": 20, "end_idx": 25, "type": "dru", "entity": "HDCV疫苗" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "肌内注射" } ] }, { "text": "佐剂地鼠肾细胞疫苗建议采用2-2-1程序,即0日肌内注射2剂(2ml),第7日和第21日各肌内注射1ml。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dru", "entity": "地鼠肾细胞疫苗" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "肌内注射" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "肌内注射" } ] }, { "text": "注射部位成人取三角肌,儿童注入腿前外侧。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "三角肌" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "腿前外侧" } ] }, { "text": "不宜接种于臂部。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "臂部" } ] }, { "text": "(四)注射免疫血清WHO推荐,在接种疫苗同时注射人狂犬病免疫球蛋白(HRIG),剂量为20IU/kg(马狂犬病免疫球蛋白剂量为40IU/kg)。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "注射免疫血清" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "接种疫苗" }, { "start_idx": 24, "end_idx": 32, "type": "dru", "entity": "人狂犬病免疫球蛋白" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "HRIG" }, { "start_idx": 51, "end_idx": 59, "type": "dru", "entity": "马狂犬病免疫球蛋白" } ] }, { "text": "先做皮试,阴性者一次肌内注射或一半剂量在伤口周围浸润注射,另半量肌内注射。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "皮试" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "肌内注射" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "肌内注射" } ] }, { "text": "皮试阳性者需行脱敏处理(0.05ml稀释20倍,0.05ml稀释10倍,0.1ml,0.2ml,0.5ml,不稀释抗血清分别皮下注射,每次观察15分钟)后方可注射。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "皮试" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "脱敏处理" }, { "start_idx": 62, "end_idx": 65, "type": "pro", "entity": "皮下注射" } ] }, { "text": "补充水电解质及热量,纠正酸碱平衡失调,维护心血管及呼吸功能。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "pro", "entity": "补充水电解质及热量,纠正酸碱平衡失调" }, { "start_idx": 19, "end_idx": 28, "type": "pro", "entity": "维护心血管及呼吸功能" } ] }, { "text": "兴奋期狂躁时可交替应用多种镇静剂,甚至应用吗啡或全身麻醉。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "dru", "entity": "吗啡" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "全身麻醉" } ] }, { "text": "咽喉痉挛不能控制导致窒息时可气管切开,间歇正压给氧。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "咽喉" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "咽喉痉挛不能控制" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "窒息" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "气管切开" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "间歇正压给氧" } ] }, { "text": "脑水肿时给脱水剂。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "脑" }, { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "脑水肿" }, { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "脱水剂" } ] }, { "text": "麻痹期可用呼吸循环兴奋剂、给氧或人工辅助呼吸。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "麻痹" }, { "start_idx": 5, "end_idx": 11, "type": "dru", "entity": "呼吸循环兴奋剂" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "给氧" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "人工辅助呼吸" } ] }, { "text": "三、核素检查放射性核素检查(简称核素检查),是利用放射性核素对疾病进行检查和对脏器功能进行测定的方法,已广泛应用于小儿肾脏疾病的诊断和病理生理研究,有以下常用的方法应用于临床。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "核素检查" }, { "start_idx": 6, "end_idx": 12, "type": "pro", "entity": "放射性核素检查" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "核素检查" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "脏器" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "小儿肾脏疾病" } ] }, { "text": "(一)肾动态显像和GRF、ERPF测定静脉注射由肾小球滤过或肾小管上皮细胞分泌而不被回吸收的显像剂(如99mTc-DTPA、131I-邻碘马尿酸和99mTc-EC等)后,用γ相机快速连续采集包括双肾和部分膀胱区域的放射性影像,可以依次观察到显像剂随血流灌注肾动脉和肾血管床的情况,然后是迅速聚集在肾实质,继而逐渐由肾实质流向肾盏、肾盂,最后经输尿管而达膀胱的整个过程,该检查提供两侧肾脏血流、肾功能和尿路是否通畅等多方面的信息,并可定量计算出双肾的GFR和ERPF。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "肾动态显像" }, { "start_idx": 9, "end_idx": 18, "type": "pro", "entity": "GRF、ERPF测定" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "静脉注射" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肾小球" }, { "start_idx": 30, "end_idx": 36, "type": "bod", "entity": "肾小管上皮细胞" }, { "start_idx": 46, "end_idx": 48, "type": "dru", "entity": "显像剂" }, { "start_idx": 51, "end_idx": 60, "type": "dru", "entity": "99mTc-DTPA" }, { "start_idx": 62, "end_idx": 71, "type": "dru", "entity": "131I-邻碘马尿酸" }, { "start_idx": 73, "end_idx": 80, "type": "dru", "entity": "99mTc-EC" }, { "start_idx": 86, "end_idx": 88, "type": "equ", "entity": "γ相机" }, { "start_idx": 97, "end_idx": 98, "type": "bod", "entity": "双肾" }, { "start_idx": 102, "end_idx": 103, "type": "bod", "entity": "膀胱" }, { "start_idx": 107, "end_idx": 111, "type": "pro", "entity": "放射性影像" }, { "start_idx": 120, "end_idx": 122, "type": "dru", "entity": "显像剂" }, { "start_idx": 124, "end_idx": 125, "type": "bod", "entity": "血流" }, { "start_idx": 128, "end_idx": 130, "type": "bod", "entity": "肾动脉" }, { "start_idx": 148, "end_idx": 150, "type": "bod", "entity": "肾实质" }, { "start_idx": 157, "end_idx": 159, "type": "bod", "entity": "肾实质" }, { "start_idx": 162, "end_idx": 163, "type": "bod", "entity": "肾盏" }, { "start_idx": 165, "end_idx": 166, "type": "bod", "entity": "肾盂" }, { "start_idx": 171, "end_idx": 173, "type": "bod", "entity": "输尿管" }, { "start_idx": 176, "end_idx": 177, "type": "bod", "entity": "膀胱" }, { "start_idx": 191, "end_idx": 192, "type": "bod", "entity": "肾脏" }, { "start_idx": 196, "end_idx": 196, "type": "bod", "entity": "肾" }, { "start_idx": 200, "end_idx": 201, "type": "bod", "entity": "尿路" }, { "start_idx": 221, "end_idx": 222, "type": "bod", "entity": "双肾" }, { "start_idx": 224, "end_idx": 226, "type": "ite", "entity": "GFR" }, { "start_idx": 228, "end_idx": 231, "type": "ite", "entity": "ERPF" } ] }, { "text": "本法十分灵敏,当血浆BUN>36mmol/L(100mg/dl)和Scr>800μmol/dl(10mg/dl),仍可使泌尿系显影。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "ite", "entity": "血浆BUN" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "Scr" }, { "start_idx": 60, "end_idx": 64, "type": "pro", "entity": "泌尿系显影" } ] }, { "text": "已成为GFR的“金标准”。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "GFR" } ] }, { "text": "动态肾显影还可作为肾血管性高血压的初筛方法,肾血管性高血压多为单侧肾动脉狭窄,典型表现为患侧肾血流灌注不良肾静态显像静脉注射能够聚集并滞留在肾实质内的显像剂(如99mTc-DMSA和99mTc-GH等),取后位进行肾区静态显像,所得影像即为肾实质影像。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "动态肾显影" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "肾血管性高血压" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "肾血管性高血压" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "单侧肾动脉狭窄" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "肾" }, { "start_idx": 44, "end_idx": 52, "type": "sym", "entity": "患侧肾血流灌注不良" }, { "start_idx": 53, "end_idx": 57, "type": "pro", "entity": "肾静态显像" }, { "start_idx": 75, "end_idx": 77, "type": "dru", "entity": "显像剂" }, { "start_idx": 80, "end_idx": 89, "type": "dru", "entity": "99mTc-DMSA" }, { "start_idx": 91, "end_idx": 98, "type": "dru", "entity": "99mTc-GH" }, { "start_idx": 107, "end_idx": 112, "type": "pro", "entity": "肾区静态显像" }, { "start_idx": 120, "end_idx": 122, "type": "bod", "entity": "肾实质" } ] }, { "text": "当血浆BUN>18mmol/L(50mg/dl),Scr>440μmol/L(5mg/dl)时,本法仍可显示残余的肾组织,较IVP灵敏。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "ite", "entity": "血浆BUN" }, { "start_idx": 62, "end_idx": 64, "type": "pro", "entity": "IVP" } ] }, { "text": "肾静态显像和动态显像是所有显像技术(包括静脉肾盂造影、肾动脉造影、X线计算机体层扫描以及超声检查等)中最直观者,能直接显示肾实质全影,因此十分有利于观察肾脏的位置、形态和大小。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "肾静态显像" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "动态显像" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "静脉肾盂造影" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "肾动脉造影" }, { "start_idx": 33, "end_idx": 41, "type": "pro", "entity": "X线计算机体层扫描" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "超声检查" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "肾脏" } ] }, { "text": "对先天性肾畸形,如蹄铁形肾(horseshoekidney)、孤立肾(singlekidney)、双肾一侧融合(unilateralfusion)、重复肾(duplicationofthekidney)和先天性肾盂输尿管连接部狭窄(pelviuretericobstruction)等,本法可一目了然,并可了解局部的功能状态。", "entities": [ { "start_idx": 14, "end_idx": 28, "type": "dis", "entity": "horseshoekidney" }, { "start_idx": 35, "end_idx": 46, "type": "dis", "entity": "singlekidney" }, { "start_idx": 56, "end_idx": 71, "type": "dis", "entity": "unilateralfusion" }, { "start_idx": 102, "end_idx": 114, "type": "dis", "entity": "先天性肾盂输尿管连接部狭窄" }, { "start_idx": 116, "end_idx": 139, "type": "dis", "entity": "pelviuretericobstruction" } ] }, { "text": "用本法显影受肾功能的影响很小,有人估计,只要残留肾功能在3%以上,本法即可勉强显影。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肾" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "肾" } ] }, { "text": "肾静态显像观察到急性肾盂肾炎患儿肾瘢痕的阳性率达50%左右,瘢痕征的表现为肾影中单个或多个局部放射性缺损或减低区,多位于上下极,典型者呈楔形,宽面向外,使整个肾影变形。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "肾静态显像" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "急性肾盂肾炎" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 30, "end_idx": 82, "type": "sym", "entity": "瘢痕征的表现为肾影中单个或多个局部放射性缺损或减低区,多位于上下极,典型者呈楔形,宽面向外,使整个肾影变形" } ] }, { "text": "本法的阳性率比超声检查和IVP高1倍左右。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "超声检查" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "IVP" } ] }, { "text": "由于瘢痕征并不全是真正的组织坏死后纤维化,而仍有较多的缺血和炎症成分,故可以由功能性肾静态显像发现,而难以被形态学检查法发现。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "瘢痕征" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "缺血" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "炎症" }, { "start_idx": 54, "end_idx": 59, "type": "pro", "entity": "形态学检查法" } ] }, { "text": "尿路感染患儿有肾瘢痕,说明感染已定位于肾实质,可作为小儿肾盂肾炎的诊断参考指标,对预后、正确治疗和疗效观察有重要意义。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尿路感染" }, { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "肾瘢痕" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "感染" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "小儿肾盂肾炎" } ] }, { "text": "由于瘢痕的上述性质,经过积极治疗可见缩小甚至消退。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "瘢痕" } ] }, { "text": "肾动态显像的肾实质影像和排出影像也可见到瘢痕肾多样异常表现,但因影像质量不如肾静态显像,故为诊断肾瘢痕应首选肾静态显像,动态显像仅有参考价值。", "entities": [ { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "瘢痕肾多样异常" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "肾瘢痕" } ] }, { "text": "(三)膀胱输尿管尿反流(vesicoureteralreflux,VUR)显像膀胱尿反流显像分直接和间接两种方法。", "entities": [ { "start_idx": 3, "end_idx": 38, "type": "pro", "entity": "膀胱输尿管尿反流(vesicoureteralreflux,VUR)显像" }, { "start_idx": 39, "end_idx": 45, "type": "pro", "entity": "膀胱尿反流显像" } ] }, { "text": "直接法是将99mTcO4-148MBq(4mCi)经导尿管注入膀胱内,然后用生理盐水缓缓灌注,待膀胱充盈完全后用力排尿,在此过程中,用γ相机以1~2秒一帧的速度连续照相,无论在充盈期或γ相机,输尿管或肾内有放射性出现,即可诊断为膀胱尿反流。", "entities": [ { "start_idx": 44, "end_idx": 46, "type": "equ", "entity": "导尿管" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "膀胱" }, { "start_idx": 110, "end_idx": 112, "type": "equ", "entity": "γ相机" } ] }, { "text": "间接法则是在进行肾肾动态显像很少时,令受检者尽量憋住尿,然后用力排尿,在此过程中用γ相机快速照相,也可观察有无膀胱输尿管反流的存在和程度。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肾" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "则是在进行肾" }, { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "肾动态显像" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "尿" } ] }, { "text": "核素法和X线法对膀胱尿反流的诊断率大致相同,且二者有约80%的X线法性,对重度反流为100%。", "entities": [ { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "X线法" } ] }, { "text": "针对X线法的使受检患儿性腺的辐射吸收剂量较大的突出的缺点,核素法所致小儿性腺的辐射吸收剂量明显要低,仅为上述剂量的1/100左右。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "pro", "entity": "核素法" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "性腺" } ] }, { "text": "第十三节尿道下裂尿道下裂是一种尿道发育畸形,即尿道开口在阴茎腹侧正常尿道口近端至会阴部的途径上小儿泌尿生殖系统最常见的畸形之一。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "尿道发育畸形" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "尿道" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "阴茎" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "会阴部" }, { "start_idx": 23, "end_idx": 46, "type": "sym", "entity": "尿道开口在阴茎腹侧正常尿道口近端至会阴部的途径上" }, { "start_idx": 47, "end_idx": 54, "type": "bod", "entity": "小儿泌尿生殖系统" } ] }, { "text": "近来,由于环境污染、农作物大量使用化肥和促生长剂,尿道下裂在欧美报道有上升之势。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "尿道下裂" } ] }, { "text": "尽管在尿道下裂的手术治疗上已经有了非常大的进步,但无论从医生的角度还是患儿或其父母的角度,尿道下裂的治疗结果远不如人意。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "尿道下裂" } ] }, { "text": "【胚胎学】胚胎第6周时,尿生殖窦的腹侧出现一个突起,称为生殖结节。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "尿生殖窦" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "生殖结节" } ] }, { "text": "在生殖结节的尾侧正中线上有一条浅沟,称为尿道沟。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "生殖结节" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "尿道沟" } ] }, { "text": "尿道沟两侧隆起部分为尿生殖褶。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "尿道沟" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "尿生殖褶" } ] }, { "text": "男性在双氢睾酮的作用下,生殖结节增长形成阴茎,尿生殖窦的下段伸入阴茎并开口于尿道沟,以后尿道沟两侧的尿生殖褶由近端向远端相互融合,形成尿道,此时尿道外口移到阴茎头冠状沟部。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "双氢睾酮" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "生殖结节" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "阴茎" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "尿生殖窦" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "阴茎" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "尿道沟" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "尿道沟" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "尿生殖褶" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "尿道" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "尿道" }, { "start_idx": 78, "end_idx": 84, "type": "bod", "entity": "阴茎头冠状沟部" } ] }, { "text": "在阴茎头顶部,外胚层向内生长出一个细胞索,以后细胞索中央与尿道沟相通,使尿道外口移到阴茎头顶端。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "阴茎头顶部" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "细胞索" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "细胞索" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "尿道沟" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "尿道外口" }, { "start_idx": 42, "end_idx": 46, "type": "bod", "entity": "阴茎头顶端" } ] }, { "text": "第12周时,阴茎头处形成皮肤反折,称为包皮。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "阴茎头" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "皮肤反折" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "包皮" } ] }, { "text": "生殖结节内的间质分化为阴茎海绵体及尿道海绵体。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "生殖结节" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "间质" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "阴茎海绵体" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "尿道海绵体" } ] }, { "text": "在胚胎期由于内分泌的异常或其他原因导致尿道沟融合不全时,即形成尿道下裂。", "entities": [ { "start_idx": 6, "end_idx": 25, "type": "sym", "entity": "内分泌的异常或其他原因导致尿道沟融合不全" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "尿道下裂" } ] }, { "text": "由于尿道远端的形成处于最后阶段,所以尿道口位于阴茎体远端的尿道下裂占比例最大。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "尿道远端" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "尿道口" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "阴茎体" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "尿道下裂" } ] }, { "text": "【病因】1.基因遗传尿道下裂发病有一定的家族倾向,但所涉及的基因或染色体的具体情况尚不清楚。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "基因" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "染色体" } ] }, { "text": "我们碰到数例父子或兄弟,特别是孪生兄弟同患此病的,有报道10%患儿的父亲及15%患儿的兄弟中有尿道下裂。", "entities": [ { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "尿道下裂" } ] }, { "text": "2.激素影响从胎睾中产生的激素影响男性外生殖器的形成。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "胎睾" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "激素" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "外生殖器" } ] }, { "text": "由绒毛膜促性腺激素刺激睾丸间质细胞(leydigcells)在孕期第8周开始产生睾酮,再转化形成双氢睾酮。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "bod", "entity": "绒毛膜促性腺激素" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "睾丸间质细胞" }, { "start_idx": 18, "end_idx": 28, "type": "bod", "entity": "leydigcells" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "睾酮" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "双氢睾酮" } ] }, { "text": "外生殖器的发育受双氢睾酮的调节。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "外生殖器" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "双氢睾酮" } ] }, { "text": "睾酮产生不足、过迟,或者睾酮转化成双氢睾酮的过程出现异常均可导致生殖器畸形。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "睾酮" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "双氢睾酮" }, { "start_idx": 0, "end_idx": 36, "type": "sym", "entity": "睾酮产生不足、过迟,或者睾酮转化成双氢睾酮的过程出现异常均可导致生殖器畸形" } ] }, { "text": "尿道下裂常伴发于隐睾、两性畸形就与此相关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "隐睾" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "两性畸形" } ] }, { "text": "母亲孕前、孕期的激素应用对胎儿生殖系统的发育也有一定的影响。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "激素" } ] }, { "text": "【临床表现】尿道下裂的临床表现非常有特点,①尿道开口异位尿道异位开口于从正常尿道口近端至会阴部的任何部位部分尿道口有轻度狭窄因其表面有一层非常薄的黏膜样组织不完全覆盖所致有时尿道的一段海绵体缺如,其壁菲薄尿线一般向后,故患儿常取蹲位排尿,尿道口位于阴茎体近端时更明显阴茎向腹侧弯曲,主要因尿道口远端的尿道板纤维组织增生阴茎腹侧和尿道壁各层组织缺乏以及阴茎海绵体背、腹两侧不对称所致包皮堆积于背侧,阴茎头腹侧包皮未能在中线融合包皮系带缺如,全部包皮转至阴茎背侧,呈帽状堆积尿道口位置尿道下裂分为四型,阴茎头型、阴茎体型、阴茎阴囊型和会阴型(图12-8)。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "尿道" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "尿道开口异位" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "尿道" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "尿道口" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "会阴部" }, { "start_idx": 28, "end_idx": 51, "type": "sym", "entity": "尿道异位开口于从正常尿道口近端至会阴部的任何部位" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "尿道口" }, { "start_idx": 52, "end_idx": 61, "type": "sym", "entity": "部分尿道口有轻度狭窄" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "黏膜" }, { "start_idx": 62, "end_idx": 84, "type": "sym", "entity": "因其表面有一层非常薄的黏膜样组织不完全覆盖所致" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "尿道" }, { "start_idx": 92, "end_idx": 94, "type": "bod", "entity": "海绵体" }, { "start_idx": 85, "end_idx": 101, "type": "sym", "entity": "有时尿道的一段海绵体缺如,其壁菲薄" }, { "start_idx": 119, "end_idx": 121, "type": "bod", "entity": "尿道口" }, { "start_idx": 124, "end_idx": 126, "type": "bod", "entity": "阴茎体" }, { "start_idx": 102, "end_idx": 132, "type": "sym", "entity": "尿线一般向后,故患儿常取蹲位排尿,尿道口位于阴茎体近端时更明显" }, { "start_idx": 133, "end_idx": 139, "type": "sym", "entity": "阴茎向腹侧弯曲" }, { "start_idx": 144, "end_idx": 146, "type": "bod", "entity": "尿道口" }, { "start_idx": 150, "end_idx": 154, "type": "bod", "entity": "尿道板纤维" }, { "start_idx": 141, "end_idx": 158, "type": "sym", "entity": "主要因尿道口远端的尿道板纤维组织增生" }, { "start_idx": 159, "end_idx": 160, "type": "bod", "entity": "阴茎" }, { "start_idx": 164, "end_idx": 166, "type": "bod", "entity": "尿道壁" }, { "start_idx": 175, "end_idx": 180, "type": "bod", "entity": "阴茎海绵体背" }, { "start_idx": 159, "end_idx": 189, "type": "sym", "entity": "阴茎腹侧和尿道壁各层组织缺乏以及阴茎海绵体背、腹两侧不对称所致" }, { "start_idx": 190, "end_idx": 191, "type": "bod", "entity": "包皮" }, { "start_idx": 198, "end_idx": 200, "type": "bod", "entity": "阴茎头" }, { "start_idx": 190, "end_idx": 211, "type": "sym", "entity": "包皮堆积于背侧,阴茎头腹侧包皮未能在中线融合" }, { "start_idx": 212, "end_idx": 213, "type": "bod", "entity": "包皮" }, { "start_idx": 221, "end_idx": 222, "type": "bod", "entity": "包皮" }, { "start_idx": 225, "end_idx": 226, "type": "bod", "entity": "阴茎" }, { "start_idx": 212, "end_idx": 234, "type": "sym", "entity": "包皮系带缺如,全部包皮转至阴茎背侧,呈帽状堆积" }, { "start_idx": 235, "end_idx": 237, "type": "bod", "entity": "尿道口" }, { "start_idx": 240, "end_idx": 243, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 249, "end_idx": 252, "type": "dis", "entity": "阴茎头型" }, { "start_idx": 254, "end_idx": 257, "type": "dis", "entity": "阴茎体型" }, { "start_idx": 259, "end_idx": 263, "type": "dis", "entity": "阴茎阴囊型" }, { "start_idx": 265, "end_idx": 267, "type": "dis", "entity": "会阴型" } ] }, { "text": "有些病例其阴茎弯曲非常严重,尿道开口仍可位于阴茎远端患儿甚至还可站立解尿,但一旦术中将纤维索带组织松解,尿道口即会退至阴茎近端尿道下裂的分型【伴发畸形】尿道下裂最常见的伴发畸形为腹股沟斜疝及睾丸下降不全,各占10%左右。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "阴茎" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "尿道" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "阴茎" }, { "start_idx": 0, "end_idx": 25, "type": "sym", "entity": "有些病例其阴茎弯曲非常严重,尿道开口仍可位于阴茎远端" }, { "start_idx": 26, "end_idx": 35, "type": "sym", "entity": "患儿甚至还可站立解尿" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "纤维索" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "尿道口" }, { "start_idx": 37, "end_idx": 62, "type": "sym", "entity": "但一旦术中将纤维索带组织松解,尿道口即会退至阴茎近端" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 76, "end_idx": 79, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 84, "end_idx": 100, "type": "sym", "entity": "伴发畸形为腹股沟斜疝及睾丸下降不全" } ] }, { "text": "尿道下裂越严重,伴发畸形的发生率也越高重,伴发畸形的发生率也越高。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "尿道下裂越严重,伴发畸形的发生率也越高" } ] }, { "text": "尿道下裂患儿伴发上尿路畸形的发生率1%~3%不等,如肾盂输尿管连接部梗阻及重复畸形等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 6, "end_idx": 23, "type": "sym", "entity": "伴发上尿路畸形的发生率1%~3%不等" }, { "start_idx": 26, "end_idx": 35, "type": "sym", "entity": "肾盂输尿管连接部梗阻" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "重复畸形" } ] }, { "text": "也有少数病人合并肛门直肠畸形。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "合并肛门直肠畸形" } ] }, { "text": "【诊断及鉴别诊断】尿道下裂的诊断由于其外观的特征性改变,并不困难,一望可知。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "尿道下裂" } ] }, { "text": "关键是应明确尿道下裂可能是某些严重泌尿生殖系畸形如两性畸形的表现。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "泌尿生殖系畸形" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "两性畸形" } ] }, { "text": "尤其是比较严重的尿道下裂,畸形伴发率高,要注意鉴别有无性别异常。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "尿道下裂" } ] }, { "text": "对外生殖器表现模棱两可的、尿道下裂伴隐睾的都要首先进行染色体检查;如明确为男性的46XY,则先行睾丸下降固定,同时进行性腺活检。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "外生殖器" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "伴隐睾" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "染色体检查" }, { "start_idx": 46, "end_idx": 62, "type": "pro", "entity": "先行睾丸下降固定,同时进行性腺活检" } ] }, { "text": "如为睾丸,但发育很差,加之阴茎发育很小的情况,要和家属商讨,考虑做成女性为好。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "睾丸" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "但发育很差" } ] }, { "text": "【手术治疗】手术行尿道成形是尿道下裂唯一的治疗方法。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "pro", "entity": "手术行尿道成形" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "尿道下裂" } ] }, { "text": "手术要求达到完全矫正阴茎下弯、尿道正位开完全矫正阴茎下弯、尿道正位开口于阴茎头、能站立排尿以及成年后能进行正常性生活的目的术方法有200多种,但至今尚无一种满意的被所有医师接受的术式。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "阴茎" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "阴茎" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "尿道" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "阴茎头" } ] }, { "text": "相对来说比较常用的手术方法有尿道口前移龟头成形术(meata尿道口前移龟头成形术ndgmeataladvancementandglanuloplastyincorporatedprocedure或FMAGPIFlapMathieu,Filp-Flap尿道成形术la加盖岛状皮瓣尿道成形术成形onlayislandflap尿道成形术皮内板横行岛状皮瓣尿道成形术包皮内板横行岛状皮瓣尿道成形术十年Duckett术多的在无阴茎下弯的尿道下裂中应用尿道板纵切卷管尿道成形术(也尿道板纵切卷管尿道成形术。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "龟头成形术" }, { "start_idx": 30, "end_idx": 39, "type": "pro", "entity": "尿道口前移龟头成形术" }, { "start_idx": 43, "end_idx": 96, "type": "pro", "entity": "meataladvancementandglanuloplastyincorporatedprocedure" }, { "start_idx": 99, "end_idx": 103, "type": "pro", "entity": "MAGPI" }, { "start_idx": 108, "end_idx": 114, "type": "pro", "entity": "Mathieu" }, { "start_idx": 116, "end_idx": 129, "type": "pro", "entity": "Filp-Flap尿道成形术" }, { "start_idx": 132, "end_idx": 142, "type": "pro", "entity": "加盖岛状皮瓣尿道成形术" }, { "start_idx": 145, "end_idx": 164, "type": "pro", "entity": "onlayislandflap尿道成形术" }, { "start_idx": 179, "end_idx": 193, "type": "pro", "entity": "包皮内板横行岛状皮瓣尿道成形术" }, { "start_idx": 196, "end_idx": 203, "type": "pro", "entity": "Duckett术" }, { "start_idx": 213, "end_idx": 216, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 234, "end_idx": 245, "type": "pro", "entity": "尿道板纵切卷管尿道成形术" } ] }, { "text": "波士顿儿Snodgrass术尿道下裂尿道成形术的50%是采用此方尿道成形术特点是手术简单,尿道外口美观,并发症低。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "pro", "entity": "Snodgrass术" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "尿道成形术" } ] }, { "text": "手术的主要并发症包括:尿瘘、尿道狭窄、尿道憩室形成尿瘘、尿道狭窄、尿道憩室形成、阴茎弯曲及阴茎扭转总体发生率约有30%左右其总体发生率约有30%左右尚难有效解决。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "尿瘘" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "尿道狭窄" }, { "start_idx": 25, "end_idx": 48, "type": "sym", "entity": "尿瘘、尿道狭窄、尿道憩室形成、阴茎弯曲及阴茎扭转" }, { "start_idx": 61, "end_idx": 73, "type": "sym", "entity": "其总体发生率约有30%左右" } ] }, { "text": "由于尿道下裂患儿多不能如正常地站立解尿,容易造成其心理负担,影响心理发育,现多主张早期手术。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "尿道下裂" } ] }, { "text": "第七篇感染性疾病第一章感染性疾病的诊断要则感染性疾病是指由病原微生物感染机体引起的一类疾病,这些病原微生物包括细菌、病毒、支原体、衣原体、立克次体、真菌、螺旋体、原虫和蠕虫等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 55, "end_idx": 56, "type": "mic", "entity": "细菌" }, { "start_idx": 58, "end_idx": 59, "type": "mic", "entity": "病毒" }, { "start_idx": 61, "end_idx": 63, "type": "mic", "entity": "支原体" }, { "start_idx": 65, "end_idx": 67, "type": "mic", "entity": "衣原体" }, { "start_idx": 69, "end_idx": 72, "type": "mic", "entity": "立克次体" }, { "start_idx": 74, "end_idx": 75, "type": "mic", "entity": "真菌" }, { "start_idx": 81, "end_idx": 82, "type": "mic", "entity": "原虫" } ] }, { "text": "其范畴远远超过以往定义的“传染病”,如外科的感染疾病、术后感染,耳鼻喉科的中耳炎和鼻窦炎,以及内科的呼吸道感染、肠道感染疾病等都属感染性疾病。", "entities": [ { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "医院内感染是相对于社区感染而言的概念,也属感染性疾病范畴。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "感染性疾病有其特异的病原微生物,因此病原学诊断尤为重要,是确诊的主要依据。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "感染性疾病的诊断过程中,要充分注意以下环节。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "如伤寒杆菌只可能在早期伤寒患者的血中发现,1周以后才能从患者粪便中查到此菌。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "mic", "entity": "伤寒杆菌" } ] }, { "text": "2.标本采集的最有价值部位是感染所在部位,如肺炎的标本应该是真正从下呼吸道取得的痰。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "肺炎" } ] }, { "text": "三、周围肺动脉狭窄自肺动脉瓣以后的肺动脉管道狭窄可发生于从主干至肺内动脉的各段,单发或多发,大多伴有其他畸形如肺动脉瓣狭窄,室间隔缺损、法洛四联症及主动脉瓣上狭窄(supravalvularaorticstenosis)等。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "周围肺动脉狭窄" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "肺动脉管道狭窄" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "主干" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "肺内动脉" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 68, "end_idx": 72, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 74, "end_idx": 80, "type": "dis", "entity": "主动脉瓣上狭窄" }, { "start_idx": 82, "end_idx": 108, "type": "dis", "entity": "supravalvularaorticstenosis" } ] }, { "text": "肺动脉的管腔狭窄依其部位可分为四型:①主干或其左右支;②主干分叉部,并延伸至左右支;③周围分支多发的梗阻;④主干及其周围分支狭窄。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "肺动脉的管腔狭窄" }, { "start_idx": 43, "end_idx": 51, "type": "dis", "entity": "周围分支多发的梗阻" }, { "start_idx": 54, "end_idx": 63, "type": "dis", "entity": "主干及其周围分支狭窄" } ] }, { "text": "狭窄部可能产生连续性杂音,尤其伴有左向右分流使肺动脉血流增多者。", "entities": [ { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "肺动脉血流" } ] }, { "text": "心导管可能发现在肺动脉的管道某部前后有明显压差,肺动脉造影可以看到狭窄的部位;为了避免狭窄部被重叠影像掩盖,必须用轴向位置方能将全貌暴露出来。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "心导管" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肺动脉" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "肺动脉造影" } ] }, { "text": "随着患儿的成长,狭窄可越趋严重,到青春期心排量大增时尤著;再者许多手术如法洛四联症,心内畸形纠正后右心室出路仍受本病的阻挡,应考虑解除的措施。", "entities": [ { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "心" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "心内畸形" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "右心室" } ] }, { "text": "四、超声检查应用实时B型超声波或三维实时超声。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "超声检查" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "B型超声波" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "三维实时超声" } ] }, { "text": "进食一定量液体时,能够有效地观察到胃窦、胃体、幽门以及十二指肠的动态运动情况,并可将胃排空情况量化。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "胃窦" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "胃体" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "幽门" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "十二指肠" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "胃" } ] }, { "text": "常用功能指标有:①收缩频率;②收缩幅度;③运动指数;④幽门开放时间;⑤胃十二指肠运动协调性;⑥十二指肠、胃反流征观察。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "ite", "entity": "运动指数" }, { "start_idx": 27, "end_idx": 32, "type": "ite", "entity": "幽门开放时间" }, { "start_idx": 35, "end_idx": 44, "type": "ite", "entity": "胃十二指肠运动协调性" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "十二指肠" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "胃" } ] }, { "text": "对胃的排空功能检查,能准确测量胃的半排空和完全排空时间。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "胃" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "胃" } ] }, { "text": "具体方法:给被检查者一定量的37℃的液体,然后每10分钟测量胃体、胃底及胃窦部前后径、上下径大小和面积,计算出胃窦、胃体面积减少的速度,从而得出胃排空和半排空时间。", "entities": [ { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "胃体" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "胃底" }, { "start_idx": 36, "end_idx": 41, "type": "bod", "entity": "胃窦部前后径" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "胃窦" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "胃体" }, { "start_idx": 72, "end_idx": 72, "type": "bod", "entity": "胃" } ] }, { "text": "国内已采用B超对小儿厌食、胃炎及功能性消化不良分别检测。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "B超" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "小儿厌食" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "胃炎" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "本方法准确可靠,重复性好,但技术要求高,适于观察液体排空,但不能确切显示固体胃排空。", "entities": [ { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "胃" } ] }, { "text": "四、手术技术的特殊性1.儿童肾移植常发生移植物大小和髂窝的空隙不成比例,需将移植肾置入腹膜后、盲肠后以及腹腔内。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "手术" }, { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "髂窝" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "肾" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "腹膜" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "盲肠" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "腹腔" } ] }, { "text": "体重>20kg的儿童移植手术方法与成人相同。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "儿童移植手术" } ] }, { "text": "<20kg的小儿取腹正中切口入腹腔,游离盲肠,将供肾移植于腹膜后,肾动、静脉分别与主动脉、腔静脉(或髂总动、静脉)吻合,成人供肾体积相对较大时,须将肾脏横置于右侧腹腔内,肾血管与腹部大血管吻合。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "腹正" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "腹腔" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "盲肠" }, { "start_idx": 25, "end_idx": 27, "type": "pro", "entity": "肾移植" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "腹膜" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "肾动、静脉" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "主动脉" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "腔静脉" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "髂总动" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "静脉" }, { "start_idx": 63, "end_idx": 63, "type": "bod", "entity": "肾" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "肾脏" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "腹腔" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "肾血管" }, { "start_idx": 89, "end_idx": 93, "type": "bod", "entity": "腹部大血管" } ] }, { "text": "2.儿童肾移植(<5岁)吻合技术需顾及儿童器官的生长过程,至少有半圈采用间断缝合,对边连续缝合进行吻合。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "器官" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "间断缝合" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "连续缝合" } ] }, { "text": "3.在开放移植肾血流时需考虑婴幼儿心搏出量及收缩压,当不能满足成人供肾血流动力学要求,应尽快输血100~200ml,保持患儿的足够血容量;当阻断主动脉进行血管吻合时,可继发酸中毒,宜给予5%NaHCO31~2ml/kg。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肾" }, { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "心搏出量" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "收缩压" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肾" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "输血" }, { "start_idx": 65, "end_idx": 67, "type": "ite", "entity": "血容量" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "主动脉" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "血管" }, { "start_idx": 86, "end_idx": 88, "type": "sym", "entity": "酸中毒" }, { "start_idx": 95, "end_idx": 100, "type": "dru", "entity": "NaHCO3" } ] }, { "text": "4.移植前切除双肾的对象经血透不能控制的恶性高血压、慢性肾盂肾炎或多囊肾合并感染不易控制时建议移植前切除双肾。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "移植" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "切除双肾" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "血透" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "恶性高血压" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "慢性肾盂肾炎" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "多囊肾合并感染" }, { "start_idx": 47, "end_idx": 48, "type": "pro", "entity": "移植" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "切除双肾" } ] }, { "text": "对进展迅速的肾小球肾炎,因血循环中存在抗肾小球基底膜抗体,为防止移植后复发,亦应切除病肾,待血循环中抗体消失6个月后再行肾移植手术。", "entities": [ { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "血" }, { "start_idx": 19, "end_idx": 25, "type": "bod", "entity": "抗肾小球基底膜" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "移植" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "肾" }, { "start_idx": 60, "end_idx": 64, "type": "pro", "entity": "肾移植手术" } ] }, { "text": "5.下尿路异常的矫正先天性泌尿道畸形患儿在肾移植前应检查膀胱功能,如膀胱输尿管反流伴肾积水,应施行肾、全输尿管切除术。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "尿路异常" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "先天性泌尿道畸形" }, { "start_idx": 21, "end_idx": 23, "type": "pro", "entity": "肾移植" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "膀胱" }, { "start_idx": 34, "end_idx": 44, "type": "sym", "entity": "膀胱输尿管反流伴肾积水" }, { "start_idx": 49, "end_idx": 57, "type": "pro", "entity": "肾、全输尿管切除术" } ] }, { "text": "膀胱有慢性炎症以及神经性膀胱病变,应移植前切除膀胱并在腹膜后形成回盲袋代膀胱。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "膀胱" }, { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "慢性炎症" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "神经性膀胱病变" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "移植" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "切除膀胱" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "腹膜" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "膀胱" } ] }, { "text": "二、爽身粉吸入婴幼儿使用爽身粉、痱子粉时误吸所致。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "爽身粉吸入" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "爽身粉" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "痱子粉" } ] }, { "text": "多含有矽酸镁或其他矽酸盐。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "矽酸镁" } ] }, { "text": "吸入肺部后造成细支气管阻塞间质性肺炎、肺纤维变性。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肺部" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "细支气管阻塞" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "间质性肺炎" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "肺纤维变性" } ] }, { "text": "开始为干咳,以后有痰有低热。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "开始为干咳" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "以后有痰" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "有低热" } ] }, { "text": "有的表现反复呼吸道感染两肺听诊可闻及干湿啰音呛咳、气喘、进行性呼吸困难、发绀等,未经处理可在1~2天内死亡。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "反复呼吸道感染" }, { "start_idx": 11, "end_idx": 21, "type": "sym", "entity": "两肺听诊可闻及干湿啰音" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "呛咳" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "气喘" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "进行性呼吸困难" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "发绀" }, { "start_idx": 40, "end_idx": 52, "type": "sym", "entity": "未经处理可在1~2天内死亡" } ] }, { "text": "胸部X线表现中下肺野有条索状、小片状、斑点状或网状阴影病程长、出现纤维化时,表现两下肺野细小网状影。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "胸部X线" }, { "start_idx": 6, "end_idx": 26, "type": "sym", "entity": "中下肺野有条索状、小片状、斑点状或网状阴影" }, { "start_idx": 27, "end_idx": 29, "type": "sym", "entity": "病程长" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "出现纤维化" }, { "start_idx": 40, "end_idx": 48, "type": "sym", "entity": "两下肺野细小网状影" } ] }, { "text": "合并感染时可有片絮状阴影。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "合并感染时可有片絮状阴影" } ] }, { "text": "以对症处理为主,急性大量吸入者可采用支气管镜下冲洗,立即在高湿度下吸氧。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "支气管镜下冲洗" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "高湿度下吸氧" } ] }, { "text": "早期使用肾上腺皮质激素可减轻炎症反应。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dru", "entity": "肾上腺皮质激素" } ] }, { "text": "七、异基因造血干细胞移植的植入证据1.异性间移植时可通过性染色体获得证据。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "异基因造血干细胞移植" }, { "start_idx": 20, "end_idx": 24, "type": "pro", "entity": "异性间移植" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "染色体" } ] }, { "text": "2.同性别异基因移植者可以通过DNA指纹证据。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "同性别异基因移植" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "DNA" }, { "start_idx": 18, "end_idx": 18, "type": "ite", "entity": "指" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "指" } ] }, { "text": "二、病理性黄疸新生儿病理性黄疸是新生儿早期除胆红素代谢的特点外,同时有使黄疸加重的疾病或致病因素存在。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "病理性黄疸" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "病理性黄疸" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "胆红素" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "黄疸" } ] }, { "text": "但广义的病理性黄疸还包括已过生理性黄疸时期而血清胆红素仍超过正常水平者。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "病理性黄疸" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "生理性黄疸" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "血清胆红素" } ] }, { "text": "部分病理性黄疸可致中枢神经系统受损,产生胆红素脑病。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "病理性黄疸" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "胆红素脑病" } ] }, { "text": "我国新生儿高胆的发病率各家报道不一,为9.1%~50.0%,甚至更高。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "高胆" } ] }, { "text": "1997年,徐放生等统计164所医院共收治患病新生儿39621例,其中黄疸患儿13918例,占患病新生儿总数的35.13%;高胆红素血症患儿共收治10365例,占患病新生儿总数的26.16%,黄疸患儿的74.47%;发生胆红素脑病216例,为高胆患儿的2.08%。", "entities": [ { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "黄疸" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "高胆红素血症" }, { "start_idx": 96, "end_idx": 97, "type": "dis", "entity": "黄疸" }, { "start_idx": 110, "end_idx": 114, "type": "dis", "entity": "胆红素脑病" }, { "start_idx": 121, "end_idx": 122, "type": "dis", "entity": "高胆" } ] }, { "text": "新生儿黄疸有下列情况之一时要考虑病理性黄疸:①生后24小时内出现黄疸,血清胆红素>102μmol/L(6mg/dl);②足月儿血清胆红素>220.6μmol/L(12.9mg/dl),早产儿>255μmol/L(15mg/dl);③血清结合胆红素>34μmol/L(2mg/dl);④血清胆红素每天上升>85μmol/L(5mg/dl);⑤黄疸持续时间较长,超过2~4周,或进行性加重。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "黄疸" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "病理性黄疸" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "黄疸" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "血清胆红素" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "血清胆红素" }, { "start_idx": 116, "end_idx": 122, "type": "bod", "entity": "血清结合胆红素" }, { "start_idx": 142, "end_idx": 146, "type": "bod", "entity": "血清胆红素" }, { "start_idx": 170, "end_idx": 171, "type": "dis", "entity": "黄疸" } ] }, { "text": "新生儿病理性黄疸按发病机制可分为红细胞破坏增多(溶血性、肝前性)、肝脏胆红素代谢功能低下(肝细胞性)和胆汁排出障碍(梗阻性、肝后性)三类。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "病理性黄疸" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "红细胞" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "肝脏胆红素" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "肝细胞性" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "胆汁" } ] }, { "text": "按实验室测定总胆红素和结合胆红素浓度的增高程度可分为高未结合胆红素血症和高结合胆红素血症,如两者同时存在则称混合性高胆红素血症。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "胆红素" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "结合胆红素" }, { "start_idx": 26, "end_idx": 34, "type": "dis", "entity": "高未结合胆红素血症" }, { "start_idx": 36, "end_idx": 43, "type": "dis", "entity": "高结合胆红素血症" }, { "start_idx": 54, "end_idx": 62, "type": "dis", "entity": "混合性高胆红素血症" } ] }, { "text": "(一)高未结合胆红素血症引起的原因有:①胆红素产生过多:如母婴血型不合、遗传性球形红细胞增多症、红细胞酶的缺陷(如G-6-PD、丙酮酸激酶、己糖激酶等)、血管外溶血、红细胞增多症等;②肝细胞摄取和结合低下:如肝脏酶系统功能不全引起的黄疸、甲状腺功能低下、进食减少等;③肠-肝循环增加:如胎粪排出延迟等。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "高未结合胆红素血症" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "胆红素" }, { "start_idx": 36, "end_idx": 46, "type": "dis", "entity": "遗传性球形红细胞增多症" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "红细胞酶" }, { "start_idx": 57, "end_idx": 62, "type": "bod", "entity": "G-6-PD" }, { "start_idx": 64, "end_idx": 68, "type": "bod", "entity": "丙酮酸激酶" }, { "start_idx": 70, "end_idx": 73, "type": "bod", "entity": "己糖激酶" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "血管" }, { "start_idx": 80, "end_idx": 88, "type": "dis", "entity": "溶血、红细胞增多症" }, { "start_idx": 92, "end_idx": 94, "type": "bod", "entity": "肝细胞" }, { "start_idx": 104, "end_idx": 106, "type": "bod", "entity": "肝脏酶" }, { "start_idx": 116, "end_idx": 117, "type": "dis", "entity": "黄疸" }, { "start_idx": 119, "end_idx": 121, "type": "bod", "entity": "甲状腺" }, { "start_idx": 127, "end_idx": 130, "type": "sym", "entity": "进食减少" }, { "start_idx": 134, "end_idx": 136, "type": "bod", "entity": "肠-肝" }, { "start_idx": 134, "end_idx": 140, "type": "sym", "entity": "肠-肝循环增加" } ] }, { "text": "1.新生儿溶血病因母子血型不合而引起的同族免疫性溶血称为新生儿溶血病(hemolyticdiseaseofnewborn)。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "新生儿溶血病" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "新生儿溶血病" }, { "start_idx": 35, "end_idx": 59, "type": "dis", "entity": "hemolyticdiseaseofnewborn" } ] }, { "text": "ABO血型不合者较Rh不合多见,大多数母亲为O型,子为A或B型,本病可见于第一胎,可能因其母孕前已受其他原因的刺激,如寄生虫感染,注射伤寒疫苗、破伤风或白喉抗毒素等,均可使机体发生初发免疫反应,当怀孕时再次刺激机体产生免疫抗体,即可通过胎盘进入胎儿引起溶血(详见本章第16节)。", "entities": [ { "start_idx": 76, "end_idx": 80, "type": "pro", "entity": "白喉抗毒素" }, { "start_idx": 118, "end_idx": 119, "type": "bod", "entity": "胎盘" }, { "start_idx": 126, "end_idx": 127, "type": "sym", "entity": "溶血" } ] }, { "text": "2.母乳性黄疸其特征为新生儿以母乳喂养后不久即出现黄疸,可持续数周到数月,而其他方面正常。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "母乳性黄疸" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "母乳" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "黄疸" } ] }, { "text": "20世纪60年代,文献报道发生率为1%~2%,随着对母乳性黄疸的认识的提高,从20世纪80年代报道的发生率有逐年上升的趋势。", "entities": [ { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "母乳性黄疸" } ] }, { "text": "其发生的原因目前认为主要是因为新生儿胆红素代谢的肠-肝循环增加有关,早发型母乳喂养性黄疸的预防和处理:鼓励尽早喂奶。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "新生儿胆红素" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肠-肝" }, { "start_idx": 34, "end_idx": 43, "type": "dis", "entity": "早发型母乳喂养性黄疸" } ] }, { "text": "晚发型母乳性黄疸,血清胆红素<257μmol/L(15mg/dl)时不需停母乳;>257μmol/L(15mg/dl)时暂停母乳3天,>342μmol/L(20mg/dl)时则加光疗,一般不需用清蛋白或血浆治疗。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "晚发型母乳性黄疸" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "血清胆红素" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "母乳" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "母乳" }, { "start_idx": 89, "end_idx": 90, "type": "pro", "entity": "光疗" }, { "start_idx": 97, "end_idx": 104, "type": "pro", "entity": "清蛋白或血浆治疗" } ] }, { "text": "(二)高结合胆红素血症新生儿结合胆红素增高的疾病,其临床均以阻塞性黄疸为特征,即皮肤、巩膜黄染,大便色泽变淡或呈灰白色如油灰状,小便深黄,肝脾大及肝功能损害等,亦称之为肝炎综合征。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "高结合胆红素血症" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "胆红素" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "阻塞性黄疸" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "皮肤" }, { "start_idx": 40, "end_idx": 62, "type": "sym", "entity": "皮肤、巩膜黄染,大便色泽变淡或呈灰白色如油灰状" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "小便深黄" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "肝脾" }, { "start_idx": 73, "end_idx": 73, "type": "bod", "entity": "肝" }, { "start_idx": 69, "end_idx": 77, "type": "sym", "entity": "肝脾大及肝功能损害" }, { "start_idx": 84, "end_idx": 88, "type": "dis", "entity": "肝炎综合征" } ] }, { "text": "1.新生儿肝炎多数为胎儿在宫内由病毒感染所致,国际上所指的CROTCHS或TORCH感染(即巨细胞病毒、风疹病毒、弓形虫、柯萨奇和其他肠道病毒、单纯疱疹和乙肝病毒、HIV以及其他病毒)均可为新生儿肝炎的病因。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "新生儿肝炎" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "胎儿" }, { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "病毒" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "CROTCHS" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "TORCH感染" }, { "start_idx": 46, "end_idx": 50, "type": "mic", "entity": "巨细胞病毒" }, { "start_idx": 52, "end_idx": 55, "type": "mic", "entity": "风疹病毒" }, { "start_idx": 57, "end_idx": 59, "type": "mic", "entity": "弓形虫" }, { "start_idx": 61, "end_idx": 63, "type": "mic", "entity": "柯萨奇" }, { "start_idx": 67, "end_idx": 70, "type": "mic", "entity": "肠道病毒" }, { "start_idx": 72, "end_idx": 75, "type": "mic", "entity": "单纯疱疹" }, { "start_idx": 77, "end_idx": 80, "type": "mic", "entity": "乙肝病毒" }, { "start_idx": 82, "end_idx": 84, "type": "mic", "entity": "HIV" }, { "start_idx": 89, "end_idx": 90, "type": "mic", "entity": "病毒" }, { "start_idx": 95, "end_idx": 99, "type": "dis", "entity": "新生儿肝炎" } ] }, { "text": "感染可经胎盘传给胎儿或在通过产道娩出时被感染。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "胎盘" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "产道" } ] }, { "text": "常在生后1~3周或更晚出现黄疸,经过一般处理后好转,病程约4~6周。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "黄疸" } ] }, { "text": "2.胆道闭锁其病因尚不清楚,发病率在亚洲比白种人为高,多在生后2周始显黄疸并呈进行性加重,粪色由浅黄转为白色,肝脏进行性增大,边缘硬而光滑;肝功能以结合胆红素升高为主。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "胆道闭锁" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "黄疸" }, { "start_idx": 45, "end_idx": 53, "type": "sym", "entity": "粪色由浅黄转为白色" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "肝脏" }, { "start_idx": 55, "end_idx": 61, "type": "sym", "entity": "肝脏进行性增大" }, { "start_idx": 70, "end_idx": 70, "type": "bod", "entity": "肝" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "胆红素" } ] }, { "text": "3.代谢性疾病由先天性代谢障碍所引起的一类疾病,部分可以在新生儿期间出现黄疸。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "代谢性疾病" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "黄疸" } ] }, { "text": "(三)混合性高胆红素血症感染是引起混合性高胆红素血症的重要原因,细菌和病毒都可引起黄疸。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "混合性高胆红素血症" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "混合性高胆红素血症" }, { "start_idx": 32, "end_idx": 33, "type": "mic", "entity": "细菌" }, { "start_idx": 35, "end_idx": 36, "type": "mic", "entity": "病毒" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "黄疸" } ] }, { "text": "患儿多伴有发热或体温不升、食欲缺乏、呼吸不规则、嗜睡和烦躁不安等症状。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "发热" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "体温不升" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "呼吸不规则" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "嗜睡" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "烦躁不安" } ] }, { "text": "表5-5、5-5示新生儿黄疸干预标准,主要针对非结合胆红素升高引起的黄疸(中华医学会儿科学分会新生儿学组,2000年9月)。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "黄疸" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "胆红素" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "黄疸" } ] }, { "text": "表5-2不同出生时龄的足月新生儿黄疸干预推荐标准注:括号内数值为mg/dl,1mg/dl=17.1μmol/L表5-3不同胎龄/出生体重的早产儿黄疸干预推荐标准注:括号内数值为mg/dl,1mg/dl=17.1μmol/L(朱建幸何振娟)", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "黄疸" }, { "start_idx": 72, "end_idx": 73, "type": "dis", "entity": "黄疸" } ] }, { "text": "第六节肺液的转运和清除(一)胎肺液和肺发育胎儿肺内上皮细胞分泌肺液(又称肺管腔液,以别于肺血管外水含量),充盈于气道和潜在肺泡,对于肺的宫内发育至关重要。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肺液" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "胎肺液" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肺" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "肺内上皮细胞" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肺液" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "肺管腔液" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "肺血管" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "气道" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "潜在肺泡" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "肺" } ] }, { "text": "肺液量由妊娠中期的4~6ml/kg增加到近足月的20ml/kg,并在出生前下降到6~10ml/kg。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "肺液量" } ] }, { "text": "当胎儿血供由胎盘转为宫外呼吸时,首先需要将肺液清除,以利于肺泡扩张和气体交换。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "胎盘" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "宫外" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肺液" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肺泡" } ] }, { "text": "在正常呼吸建立后,肺液在充气的成熟肺泡腔内非常少,可能只有0.5~1ml/kg,主要作为电解质、肺表面活性物质的承载与交换基质。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肺液" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肺泡腔" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "电解质" }, { "start_idx": 48, "end_idx": 54, "type": "bod", "entity": "肺表面活性物质" } ] }, { "text": "羊膜早破可能导致肺液显著减少,或膈疝使肺组织发育受外来压迫,均可造成肺泡发育延迟或停顿,为肺发育不良(hypoplasia)或不发育(agenesis)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "羊膜早破" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肺液" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "膈疝" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "肺" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "肺泡发育延迟或停顿" }, { "start_idx": 45, "end_idx": 75, "type": "sym", "entity": "肺发育不良(hypoplasia)或不发育(agenesis)" } ] }, { "text": "肺液与淋巴液、血浆的电解质、蛋白成分不同(表8-8),反映出肺液不是血浆或淋巴液的漏出或浓缩。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺液" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "淋巴液" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血浆" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "电解质" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "蛋白" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肺液" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "血浆" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "淋巴液" } ] }, { "text": "表8-3胎羊肺液、淋巴液和血浆成分比较(二)肺淋巴管肺淋巴管形成网络,分布于肺血管周围的结缔组织间质中,主要为肺泡外间质内,为水分子和大分子的回流通路。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "肺淋巴管" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "肺淋巴管" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "肺血管" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "结缔组织间质" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "肺泡外间质" } ] }, { "text": "其中包括大量淋巴细胞和血液有形成分。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血液" } ] }, { "text": "液体主要借助肺泡壁和肺泡间质之间的压力差作为推进动力,且流量随间质内液量增多而增加。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肺泡壁" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "肺泡间质" } ] }, { "text": "而肺间质也沿外周肺泡向肺门部呈压力下降趋势,以保持微血管滤出液的回流。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "肺间质" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "肺门部" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "微血管滤出液" } ] }, { "text": "滤出液先顺此压力梯度转移到大血管和气道周围结缔组织中,经淋巴管系统有效地回流过量液体。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "滤出液" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "大血管" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "气道" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "结缔组织" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "淋巴管系统" } ] }, { "text": "当滤出液量超过了淋巴回流能力时,液体先在肺门及大血管鞘积聚,形成肺水肿早期血管周围液体“袖口”特征。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "淋巴" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肺门" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "大血管鞘" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "肺水肿" } ] }, { "text": "在间质和肺泡水肿时,淋巴回流代偿能力丧失。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肺泡" }, { "start_idx": 1, "end_idx": 7, "type": "sym", "entity": "间质和肺泡水肿" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "淋巴" } ] }, { "text": "(三)水的跨壁转运肺泡上皮细胞、毛细血管内皮细胞和间质基质是肺泡和间质液体转运的主要屏障。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 16, "end_idx": 23, "type": "bod", "entity": "毛细血管内皮细胞" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "间质基质" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肺泡" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "间质" } ] }, { "text": "间质中的透明质酸、硫酸蛋白聚糖、清蛋白、内皮细胞多糖成分间相互作用,调节内皮细胞屏障对水的通透性。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "间质" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "透明质酸" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "硫酸蛋白聚糖" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "清蛋白" }, { "start_idx": 20, "end_idx": 25, "type": "bod", "entity": "内皮细胞多糖" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "内皮细胞" } ] }, { "text": "肺泡上皮细胞端面的离子通道、水通道、基底面的钠-钾ATP酶(钠泵)对钠、钾、氯离子的转运,使水分由肺泡向肺间质和血管转移。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 22, "end_idx": 28, "type": "bod", "entity": "钠-钾ATP酶" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "钠泵" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "肺泡" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "血管" } ] }, { "text": "第四节IgA肾病IgA肾病(IgAnephropathy)是1968年由Berger首先描述的,以系膜增生及系膜区显著弥漫的IgA沉积为特征的一组肾小球疾病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 14, "end_idx": 27, "type": "dis", "entity": "IgAnephropathy" }, { "start_idx": 50, "end_idx": 66, "type": "sym", "entity": "膜增生及系膜区显著弥漫的IgA沉积" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "肾小球疾病" } ] }, { "text": "本节主要介绍原发性IgA肾病,继发性IgA肾病请参阅各有关章节。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "原发性IgA肾病" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "继发性IgA肾病" } ] }, { "text": "现有的流行病学资料均是以同期肾活体组织检查乃至肾脏病住院人数作参照对象统计得来的。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "肾脏病" } ] }, { "text": "中华儿科学会肾脏病学组统计全国20个单位,1979~1994年共2315例肾活检标本中,IgA肾病168例,占7.3%。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "肾脏病" }, { "start_idx": 37, "end_idx": 39, "type": "pro", "entity": "肾活检" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "该病在年长儿及成人中更多见,在原发性肾小球疾病肾活体组织检查中,IgA肾病在北美占10%左右,欧洲10%~30%,亚太地区最高,我国为30%,日本甚至高达50%。", "entities": [ { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "原发性肾小球疾病" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "由于肾组织内有IgA、C3或/和IgA、IgG的沉积,因此IgA肾病是一种免疫复合物性肾炎,其发病与IgA免疫异常密切相关,目前有关研究已深入到IgA分子结构水平。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肾组织" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "IgA" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "C3" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "IgA" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "IgG" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 37, "end_idx": 44, "type": "dis", "entity": "免疫复合物性肾炎" }, { "start_idx": 50, "end_idx": 56, "type": "sym", "entity": "IgA免疫异常" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "IgA" } ] }, { "text": "(一)免疫球蛋白A的结构与特征IgA是一种重要的免疫球蛋白,约占血清总免疫球蛋白的15.2%,80%的血清IgA是以单体四条链的形式出现,单体间的连接靠二硫键和J链稳定。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "免疫球蛋白A" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "IgA" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 32, "end_idx": 39, "type": "bod", "entity": "血清总免疫球蛋白" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "血清IgA" } ] }, { "text": "依α重链抗原性不同,将IgA分为2个血清型,即IgA1和IgA2。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "IgA" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "IgA1" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "IgA2" } ] }, { "text": "IgA1是血清中的主要亚型,占80%~90%,IgA2仅占10%~20%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "IgA1" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血清" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "IgA2" } ] }, { "text": "IgA1绞链区比IgA2长1倍,IgA2又可分为IgA2m(1)和IgA2m(2),尽管血清IgA2浓度仅及IgA1的1/4,但分泌液中IgA2浓度与IgA1相等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "IgA1" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "IgA2" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "IgA2" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "IgA2" }, { "start_idx": 74, "end_idx": 79, "type": "bod", "entity": "血清IgA2" }, { "start_idx": 90, "end_idx": 93, "type": "bod", "entity": "IgA1" }, { "start_idx": 106, "end_idx": 108, "type": "bod", "entity": "分泌液" }, { "start_idx": 110, "end_idx": 112, "type": "bod", "entity": "IgA" }, { "start_idx": 123, "end_idx": 126, "type": "bod", "entity": "IgA1" } ] }, { "text": "在IgA2m(1)结构中,α链与轻链间无二硫键,靠非共价键连接,但轻链间及α链间则由二硫链相连接。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "IgA2" } ] }, { "text": "分泌型IgA与血清型不同,它是一个二聚体分子,带一个J链和另一个外分泌成分(SC)组成(IgA)2-J-SC复合物。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "分泌型IgA" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "IgA" } ] }, { "text": "而血清型则是(IgA)2-J组成。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "IgA" } ] }, { "text": "J链由137个氨基酸构成,分子量1500,是一种酸性糖蛋白,含8个胱氨酸残基,6个与链内二硫链形成有关,而2个与α链的连接有关。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "氨基酸" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "酸性糖蛋白" } ] }, { "text": "已知α链的C末端有18个额外的氨基酸残基,J链是通过与α链的C端的第2个半胱氨酸残基与α链相连的。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "氨基酸" } ] }, { "text": "SC是由黏膜组织或分泌腺体中的上皮细胞合成的,通过二硫键同人SIgA的两个单体IgA中的一个相连接,SC是由549~558个氨基酸组成的多肽链,分子量约7万,糖基含量高达20%。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "黏膜组织" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "腺体" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "SIgA" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "IgA" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "氨基酸" } ] }, { "text": "其多肽链上有5个同源区,每个同源区由104、114个氨基酸组成,这些同源区在立体结构上与Ig相似。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "氨基酸" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "Ig" } ] }, { "text": "SIgA的构型可能是:①一种堆加起来的Y型排列;②末端对末端的排列,两个IgA通过Fcα区相连接,组成双Y字形结构。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "SIgA" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "IgA" } ] }, { "text": "局部组织浆细胞产生的(IgA)2-J通过:①与上皮细胞基底侧表面的SC结合后,形成IgA-J-SC,转送到一个囊泡中的顶端表面而分泌出去;②(IgA)2-J经淋巴管进入血液循环,同肝细胞表面的SC结合而清除,再经肝细胞的囊泡机制而转送入胆道,并最终进入肠道。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "组织浆细胞" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "IgA" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "SC" }, { "start_idx": 41, "end_idx": 48, "type": "bod", "entity": "IgA-J-SC" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "囊泡" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "IgA" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "淋巴管" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "血液" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "肝细胞" }, { "start_idx": 96, "end_idx": 97, "type": "bod", "entity": "SC" }, { "start_idx": 106, "end_idx": 108, "type": "bod", "entity": "肝细胞" }, { "start_idx": 110, "end_idx": 111, "type": "bod", "entity": "囊泡" }, { "start_idx": 118, "end_idx": 119, "type": "bod", "entity": "胆道" }, { "start_idx": 126, "end_idx": 127, "type": "bod", "entity": "肠道" } ] }, { "text": "血清IgA末端相互连接可形成末端开放的多聚体,而且一个明显的特征是多聚体大小的异质性,血清中IgA有20%是以多聚体形或存在的,且沉降系数为10S、13S及15S不等,此外IgA有易于同其他蛋白质形成复合物的倾向,这都是由于α链的氨基酸残基极易于形成分子间的二硫键。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "血清IgA" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "血清" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "IgA" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "IgA" }, { "start_idx": 95, "end_idx": 97, "type": "bod", "entity": "蛋白质" }, { "start_idx": 115, "end_idx": 117, "type": "bod", "entity": "氨基酸" } ] }, { "text": "(二)IgA在肾小球系膜区的沉积在IgA肾病中,IgA沉积的方式与肾小球的病理变化是相平行的。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "IgA" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "肾小球系膜" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "IgA" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "肾小球" } ] }, { "text": "系膜区的IgA沉积伴随系膜增生,毛细血管上的沉积则伴随血管内皮的改变。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "系膜" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "毛细血管" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "血管内皮" } ] }, { "text": "引起IgA沉积的病理因素有:①抗原从黏膜处进入体内并刺激IgA免疫系统,抗原成分范围很广,包括微生物及食物(卵白蛋白、牛血清白蛋白、酪蛋白和胶)等;②IgA免疫反应异常导致高分子量的多聚IgA形成;③结合抗原的多聚IgA通过静电(λ链)、受体(FcaR)或与纤维连接蛋白结合而沉积于肾脏,已发现血清中IgA-纤维连接蛋白复合物是IgA肾病的特征;④其他IgA清除机制(如肝脏)的受损或饱和。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "IgA" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "黏膜" }, { "start_idx": 28, "end_idx": 34, "type": "bod", "entity": "IgA免疫系统" }, { "start_idx": 47, "end_idx": 49, "type": "mic", "entity": "微生物" }, { "start_idx": 75, "end_idx": 97, "type": "sym", "entity": "IgA免疫反应异常导致高分子量的多聚IgA形成" }, { "start_idx": 100, "end_idx": 142, "type": "sym", "entity": "结合抗原的多聚IgA通过静电(λ链)、受体(FcaR)或与纤维连接蛋白结合而沉积于肾脏" }, { "start_idx": 147, "end_idx": 148, "type": "bod", "entity": "血清" }, { "start_idx": 150, "end_idx": 162, "type": "bod", "entity": "IgA-纤维连接蛋白复合物" }, { "start_idx": 164, "end_idx": 168, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 176, "end_idx": 178, "type": "bod", "entity": "IgA" }, { "start_idx": 185, "end_idx": 186, "type": "bod", "entity": "肝脏" } ] }, { "text": "现有的研究表明,IgA肾病中在肾小球内沉积的IgA主要是多聚的λ-IgA1,IgA肾病患者的血清IgA1、多聚IgA和λ-IgA1水平均可见增高。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肾小球" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "IgA" }, { "start_idx": 31, "end_idx": 36, "type": "bod", "entity": "λ-IgA1" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 46, "end_idx": 51, "type": "bod", "entity": "血清IgA1" }, { "start_idx": 53, "end_idx": 57, "type": "bod", "entity": "多聚IgA" }, { "start_idx": 59, "end_idx": 64, "type": "bod", "entity": "λ-IgA1" } ] }, { "text": "患者B细胞存在β-1,3半乳糖基转移酶(β-1,3GT)的缺陷,导致IgA1绞链区O型糖基化时,末端链接的半乳糖减少,这一改变可能影响IgA1与肝细胞上的寡涎酸蛋白受体(ASGPR)结合而影响IgA的清除,而且能增加其与肾脏组织的结合而沉积。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "B细胞" }, { "start_idx": 7, "end_idx": 18, "type": "bod", "entity": "β-1,3半乳糖基转移酶" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "β-1,3GT" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "IgA1" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "IgA1" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "肝细胞" }, { "start_idx": 83, "end_idx": 89, "type": "bod", "entity": "寡涎酸蛋白受体" }, { "start_idx": 91, "end_idx": 95, "type": "bod", "entity": "ASGPR" }, { "start_idx": 102, "end_idx": 104, "type": "bod", "entity": "IgA" }, { "start_idx": 116, "end_idx": 119, "type": "bod", "entity": "肾脏组织" } ] }, { "text": "Harper等采用原位杂交技术研究发现IgA肾病肠道黏膜表达合成多聚IgA的必需成分J链mRNA水平降低,而骨髓则升高。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "肠道黏膜" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "多聚IgA" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "J链mRNA" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "骨髓" } ] }, { "text": "此外,扁桃体PIgA1产生也增多。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "bod", "entity": "扁桃体PIgA1" } ] }, { "text": "由于扁桃体PIgA产量远低于黏膜及骨髓,因此,沉积在肾组织中的PIgA1可能主要来源于骨髓而非扁桃体及黏膜。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "扁桃体PIgA" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "黏膜" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "骨髓" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "肾组织" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "PIgA1" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "骨髓" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "扁桃体" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "黏膜" } ] }, { "text": "(三)IgA肾病的免疫异常对IgA肾病体液及细胞免疫的广泛研究,表明IgA肾病患者存在免疫异常,包括:1.自身抗体Fornesier等已在肾病病人血清中发现有针对肾脏系膜细胞胞浆大分子成分的抗体。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "体液" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "细胞" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "免疫异常" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "抗体" }, { "start_idx": 69, "end_idx": 70, "type": "dis", "entity": "肾病" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "血清" }, { "start_idx": 81, "end_idx": 88, "type": "bod", "entity": "肾脏系膜细胞胞浆" }, { "start_idx": 95, "end_idx": 96, "type": "bod", "entity": "抗体" } ] }, { "text": "此外还有针对基底膜Ⅰ、Ⅱ、Ⅲ型胶原纤维、层黏蛋白及Gliadin等成分的抗体。", "entities": [ { "start_idx": 6, "end_idx": 18, "type": "bod", "entity": "基底膜Ⅰ、Ⅱ、Ⅲ型胶原纤维" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "层黏蛋白" } ] }, { "text": "在部分病人血液中还发现IgA型抗中性粒细胞胞浆抗体(IgA-ANCA)。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "血液" }, { "start_idx": 11, "end_idx": 24, "type": "bod", "entity": "IgA型抗中性粒细胞胞浆抗体" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "IgA-ANCA" } ] }, { "text": "IgA肾病接受同种肾移植后,在移植肾中重新出现IgA肾病病理改变者高达40%~50%,这些资料均说明自身抗体在IgA肾病的发病中起重要作用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "肾移植" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "移植肾" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "2.细胞免疫研究表明,细胞免疫功能的紊乱也在IgA肾病发病中起重要作用。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "细胞" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "细胞免疫功能的紊乱" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "IgA特异性抑制T细胞活性的下降导致B淋巴细胞合成IgA的增加。", "entities": [ { "start_idx": 0, "end_idx": 30, "type": "sym", "entity": "IgA特异性抑制T细胞活性的下降导致B淋巴细胞合成IgA的增加" } ] }, { "text": "T辅助细胞(Th)数在IgA肾病活动期也增高,因此活动期时Th/Ts增高。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "T辅助细胞" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "Th" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "Th/Ts" } ] }, { "text": "具有IgA特异性受体的T细胞称为Tα细胞,Tα细胞具有增加IgA产生的作用。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "bod", "entity": "IgA特异性受体的T细胞" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "Tα细胞" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "Tα细胞" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "IgA" } ] }, { "text": "有人发现IgA肾病尤其是表现为肉眼血尿的患者Tα明显增多,Tα辅助细胞明显增多导致了IgA合成的增多。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "Tα" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "Tα辅助细胞" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "IgA" } ] }, { "text": "3.细胞因子与炎症介质许多细胞因子参与了免疫系统的调节,包括淋巴因子、白介素(interleukin,IL)、肿瘤坏死因子以及多肽生长因子,这些细胞因子对于行使正常的免疫功能起重要作用,在异常情况下也会导致细胞因子网络的失调,从而产生免疫损伤。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "细胞因子" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "炎症介质" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "细胞因子" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "免疫系统" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "淋巴因子" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "白介素" }, { "start_idx": 39, "end_idx": 49, "type": "bod", "entity": "interleukin" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "IL" }, { "start_idx": 55, "end_idx": 60, "type": "bod", "entity": "肿瘤坏死因子" }, { "start_idx": 63, "end_idx": 68, "type": "bod", "entity": "多肽生长因子" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "细胞因子" }, { "start_idx": 103, "end_idx": 106, "type": "bod", "entity": "细胞因子" }, { "start_idx": 117, "end_idx": 120, "type": "sym", "entity": "免疫损伤" } ] }, { "text": "在肾小球系膜细胞增生的过程中,细胞因子与炎症介质(补体成分MAC、IL1、MCP-1及活性氧等)发挥着重要作用。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "bod", "entity": "肾小球系膜细胞" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "细胞因子" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "炎症介质" }, { "start_idx": 25, "end_idx": 31, "type": "bod", "entity": "补体成分MAC" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "IL1" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "MCP-1" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "活性氧" } ] }, { "text": "4.免疫遗传已有家族成员先后患IgA肾病的报道,提示遗传因素在IgA肾病中有重要作用。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "IgA肾病相关的HLA抗原位点也报道不一,欧美以Bw35,日本和我国以DR4多见,也有报道我国北方汉族以DRW12最多见,此外还有与B12、DR1以及IL-RN.2等位基因、ACED/D基因型相关的报道。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "HLA抗原" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "Bw35" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "DR4" }, { "start_idx": 64, "end_idx": 68, "type": "bod", "entity": "DRW12" }, { "start_idx": 84, "end_idx": 86, "type": "bod", "entity": "B12" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "DR1" }, { "start_idx": 99, "end_idx": 109, "type": "bod", "entity": "IL-RN.2等位基因" }, { "start_idx": 111, "end_idx": 119, "type": "bod", "entity": "ACED/D基因型" } ] }, { "text": "【病理】光镜表现为肾小球系膜增生,程度从局灶、节段性增生到弥漫性系膜增生不等。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "equ", "entity": "光镜" }, { "start_idx": 9, "end_idx": 37, "type": "sym", "entity": "肾小球系膜增生,程度从局灶、节段性增生到弥漫性系膜增生不等" } ] }, { "text": "部分系膜增生较重者可见系膜插入,形成节段性双轨。", "entities": [ { "start_idx": 0, "end_idx": 22, "type": "sym", "entity": "部分系膜增生较重者可见系膜插入,形成节段性双轨" } ] }, { "text": "有时还见节段性肾小球硬化毛细血管塌陷及球囊粘连。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肾小球" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "有时还见节段性肾小球硬化" }, { "start_idx": 12, "end_idx": 22, "type": "sym", "entity": "毛细血管塌陷及球囊粘连" } ] }, { "text": "个别病变严重者可出现透明样变和全球硬化,个别有毛细血管管袢形成Masson染色可见系膜区大量嗜复红沉积物,这些沉积物具有诊断价值。", "entities": [ { "start_idx": 2, "end_idx": 18, "type": "sym", "entity": "病变严重者可出现透明样变和全球硬化" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "毛细血管管袢" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "个别有毛细血管管袢形成" }, { "start_idx": 31, "end_idx": 38, "type": "pro", "entity": "Masson染色" }, { "start_idx": 39, "end_idx": 51, "type": "sym", "entity": "可见系膜区大量嗜复红沉积物" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "沉积物" } ] }, { "text": "Ⅰ、Ⅲ、Ⅳ型胶原及层黏蛋白、纤维结合蛋白在IgA肾病肾小球毛细血管袢的表达明显增加,Ⅰ、Ⅲ型胶原在系膜区表达也明显增加多数患者肾小管基底膜Ⅳ型胶原表达也增加电镜下主要为不同程度的系膜细胞和基质增生,在系膜区有较多的电子致密物沉积,有些致密物也可沉积于内皮下。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "Ⅰ、Ⅲ、Ⅳ型胶原" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "层黏蛋白" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "纤维结合蛋白" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "肾小球毛细血管袢" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "胶原" }, { "start_idx": 42, "end_idx": 58, "type": "sym", "entity": "Ⅰ、Ⅲ型胶原在系膜区表达也明显增加" }, { "start_idx": 63, "end_idx": 72, "type": "bod", "entity": "肾小管基底膜Ⅳ型胶原" }, { "start_idx": 59, "end_idx": 77, "type": "sym", "entity": "多数患者肾小管基底膜Ⅳ型胶原表达也增加" }, { "start_idx": 78, "end_idx": 79, "type": "equ", "entity": "电镜" }, { "start_idx": 84, "end_idx": 97, "type": "sym", "entity": "不同程度的系膜细胞和基质增生" }, { "start_idx": 99, "end_idx": 127, "type": "sym", "entity": "在系膜区有较多的电子致密物沉积,有些致密物也可沉积于内皮下" } ] }, { "text": "【临床表现】本病多见于年长儿童及青年,男女比为2∶1,起病前多常有上呼吸道感染的诱因,也有由腹泻及泌尿系感染等诱发的报告。", "entities": [ { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "腹泻" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "泌尿系感染" } ] }, { "text": "也有些患儿表现为血尿和蛋白尿,此时血尿既可为发作性肉眼血尿,也可为镜下血尿,蛋白尿多为轻-中度。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "血尿" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "血尿" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "发作性肉眼血尿" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "镜下血尿" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "蛋白尿" } ] }, { "text": "高血压是IgA肾病病情恶化的重要标志,多数伴有肾功能的迅速恶化。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "高血压" }, { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "肾功能的迅速恶化" } ] }, { "text": "【实验室检查】(一)免疫学检查约1/4~1/2病人血IgA增高,主要是多聚体IgA的增多;约1/5~2/3患儿血中可检出IgA循环免疫复合物和/或IgG循环免疫复合物;少数患者有抗“O”滴度升高;补体C3、C4多正常。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "免疫学检查" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "血IgA增高" }, { "start_idx": 35, "end_idx": 43, "type": "sym", "entity": "多聚体IgA的增多" }, { "start_idx": 55, "end_idx": 82, "type": "sym", "entity": "血中可检出IgA循环免疫复合物和/或IgG循环免疫复合物" }, { "start_idx": 89, "end_idx": 96, "type": "sym", "entity": "抗“O”滴度升高" }, { "start_idx": 98, "end_idx": 101, "type": "bod", "entity": "补体C3" }, { "start_idx": 109, "end_idx": 110, "type": "bod", "entity": "C4" } ] }, { "text": "IgA型类风湿因子以及IgA型ANCA也时常为阳性,有人认为血中升高的IgA-纤维结合蛋白复合物是IgA肾病的特征性改变,有较高诊断价值。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "IgA型类风湿因子" }, { "start_idx": 11, "end_idx": 18, "type": "bod", "entity": "IgA型ANCA" }, { "start_idx": 30, "end_idx": 30, "type": "ite", "entity": "血" }, { "start_idx": 35, "end_idx": 47, "type": "bod", "entity": "IgA-纤维结合蛋白复合物" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "(二)免疫病理肾脏免疫病理是确诊IgA肾病唯一关键的依据。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肾脏" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "有人进行皮肤免疫病理检查发现,20%~50%病人皮肤毛细血管壁上有IgA、C3及备解素的沉积,Bene等报告皮肤活体组织检查的特异性和敏感性分别为88%和75%。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "pro", "entity": "皮肤免疫病理检查" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "皮肤毛细血管壁" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "IgA" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "C3" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "备解素" }, { "start_idx": 60, "end_idx": 67, "type": "pro", "entity": "皮肤活体组织检查" } ] }, { "text": "【诊断】(一)诊断年长儿童反复发作性肉眼血尿并多有上呼吸道或肠道感染的诱因,应考虑本病;表现为单纯镜下血尿或肉眼血尿或伴中等度蛋白尿时,也应怀疑IgA肾病,争取尽早肾活体组织检查。", "entities": [ { "start_idx": 13, "end_idx": 21, "type": "sym", "entity": "反复发作性肉眼血尿" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "上呼吸道或肠道感染" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "单纯镜下血尿" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 60, "end_idx": 65, "type": "sym", "entity": "中等度蛋白尿" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 82, "end_idx": 88, "type": "pro", "entity": "肾活体组织检查" } ] }, { "text": "以肾病综合征、急进性肾炎综合征和高血压伴肾功能不全为表现者也应考虑本病,确诊有赖肾活体组织检查。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "肾病综合征" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "急进性肾炎综合征" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "高血压伴肾功能不全" }, { "start_idx": 40, "end_idx": 46, "type": "pro", "entity": "肾活体组织检查" } ] }, { "text": "(二)WHO对本病的病理分级Ⅰ级:光镜大多数肾小球正常,少数部位有轻度系膜增生伴/不伴细胞增生小管和间质损害。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肾小球" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "膜" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "细胞" }, { "start_idx": 17, "end_idx": 46, "type": "sym", "entity": "光镜大多数肾小球正常,少数部位有轻度系膜增生伴/不伴细胞增生" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "小管" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "间质" } ] }, { "text": "Ⅱ级:少于50%的肾小球有系膜增生,罕有硬化、粘连和小新月体,称轻微病变,无小管和间质损害局灶节段乃至弥漫性肾小球系膜增宽伴细胞增生偶有粘连和小新月体局灶节段性肾小球肾炎。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肾小球" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "新月体" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "小管" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "间质" }, { "start_idx": 3, "end_idx": 44, "type": "sym", "entity": "少于50%的肾小球有系膜增生,罕有硬化、粘连和小新月体,称轻微病变,无小管和间质损害" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "肾小球" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "细胞" }, { "start_idx": 45, "end_idx": 65, "type": "sym", "entity": "局灶节段乃至弥漫性肾小球系膜增宽伴细胞增生" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "新月体" }, { "start_idx": 66, "end_idx": 74, "type": "sym", "entity": "偶有粘连和小新月体" }, { "start_idx": 75, "end_idx": 84, "type": "dis", "entity": "局灶节段性肾小球肾炎" } ] }, { "text": "偶有局灶性间质水肿和轻度炎症细胞浸润全部肾小球示明显的弥漫性系膜增生和硬化伴不规则分布的、不同程度的细胞增生少于50%的肾小球有粘连和新月体弥漫性系膜增生性肾小球肾炎。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "局灶性间质水肿" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "细胞" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "轻度炎症细胞浸润" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肾小球" }, { "start_idx": 18, "end_idx": 36, "type": "sym", "entity": "全部肾小球示明显的弥漫性系膜增生和硬化" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "细胞" }, { "start_idx": 37, "end_idx": 53, "type": "sym", "entity": "伴不规则分布的、不同程度的细胞增生" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "肾小球" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "新月体" }, { "start_idx": 54, "end_idx": 69, "type": "sym", "entity": "少于50%的肾小球有粘连和新月体" }, { "start_idx": 70, "end_idx": 82, "type": "dis", "entity": "弥漫性系膜增生性肾小球肾炎" } ] }, { "text": "有明显的小管萎缩和间质炎症。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "小管萎缩" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "间质炎症" } ] }, { "text": "小管和间质的损害较Ⅳ级更严重。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "间质" } ] }, { "text": "(一)一般治疗儿童最多见的临床类型是反复发作性的肉眼血尿,且大多有诱因如急性上呼吸道感染等,因此要积极控制感染,清除病灶,注意休息。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "急性上呼吸道感染" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "感染" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "清除病灶" } ] }, { "text": "(二)肾上腺皮质激素及免疫抑制剂对于以肾病综合征或急进性肾炎综合征起病的患儿,应予以皮质激素及免疫抑制剂治疗。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "肾病综合征" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "急进性肾炎综合征" }, { "start_idx": 42, "end_idx": 45, "type": "dru", "entity": "皮质激素" }, { "start_idx": 47, "end_idx": 51, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "Kabayashi曾回顾性研究二组病人,一组为29例,蛋白尿>2g/d,泼尼松治疗1~3年,随访2~4年,结果表明早期的激素治疗(Ccr在70ml/min以上时)对于稳定肾功能及延缓疾病进展有益。", "entities": [ { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 36, "end_idx": 38, "type": "dru", "entity": "泼尼松" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "激素治疗" }, { "start_idx": 85, "end_idx": 85, "type": "bod", "entity": "肾" } ] }, { "text": "对另一组18例蛋白尿1~2g/d的IgA肾病也采用皮质激素治疗,同时以42例使用双嘧达莫及吲哚美辛的IgA患者作对照,治疗组在稳定肾功能及降压蛋白尿方面明显优于对照组。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "皮质激素治疗" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "IgA" }, { "start_idx": 65, "end_idx": 65, "type": "bod", "entity": "肾" }, { "start_idx": 71, "end_idx": 73, "type": "ite", "entity": "蛋白尿" } ] }, { "text": "Lai等报告了一个前瞻性随机对照试验结果,17例患者每日服用泼尼松4个月,与17例对照组相比,平均观察38个月,两组内生肌酐清除率无显著差异,泼尼松治疗对轻微病变的肾病综合征患者,可明显提高缓解率,但有一定不良反应。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "泼尼松" }, { "start_idx": 60, "end_idx": 64, "type": "ite", "entity": "肌酐清除率" }, { "start_idx": 71, "end_idx": 73, "type": "dru", "entity": "泼尼松" }, { "start_idx": 82, "end_idx": 86, "type": "sym", "entity": "肾病综合征" } ] }, { "text": "这一研究提示泼尼松治疗对于IgA肾病是有益的。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "泼尼松" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "有人报道一组对成人IgA肾病的对照研究以考察硫唑嘌呤和泼尼松的疗效。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "成人IgA肾病" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "泼尼松" } ] }, { "text": "66例病人使用硫唑嘌呤和泼尼松,结果表明其在减慢IgA肾病进展方面,与48例未接受该治疗的对照组比较是有益的。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "泼尼松" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "有关应用环孢霉素的报道较少,Lai等曾应用环孢素A进行了一个随机、单盲对照试验,治疗组及对照组各12例,患者蛋白尿大于1.5g/d,并有肌酐清除率减退[Ccr(77±6)ml/min],予环孢素A治疗12周,使血浆浓度水平控制在50~100ng/ml。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "环孢霉素" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "环孢素A" }, { "start_idx": 54, "end_idx": 56, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 68, "end_idx": 72, "type": "ite", "entity": "肌酐清除率" }, { "start_idx": 94, "end_idx": 97, "type": "dru", "entity": "环孢素A" }, { "start_idx": 105, "end_idx": 108, "type": "ite", "entity": "血浆浓度" } ] }, { "text": "结果显示蛋白排泄显著减少,同时伴随着血浆肌酐清除率提高,但这些变化在终止治疗后则消失。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "蛋白" }, { "start_idx": 18, "end_idx": 24, "type": "ite", "entity": "血浆肌酐清除率" } ] }, { "text": "总之,免疫抑制剂在治疗IgA肾病方面的功效仍有待评价。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "Woo和Wallker分别观察了环磷酰胺、华法林、双嘧达莫及激素的联合治疗效果,结果与对照组相比,在治疗期间可以降低蛋白尿并稳定肾功能,但随访2~5年后,肾功能保护方面与对照组相比较无明显差异。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "华法林" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 30, "end_idx": 31, "type": "dru", "entity": "激素" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "联合治疗" }, { "start_idx": 58, "end_idx": 60, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 64, "end_idx": 66, "type": "ite", "entity": "肾功能" }, { "start_idx": 77, "end_idx": 79, "type": "ite", "entity": "肾功能" } ] }, { "text": "(三)免疫球蛋白在一组开放的前瞻性的研究中,Rostoker等人采用大剂量免疫球蛋白静脉注射,每日1次,每次2g/kg,连用3个月,然后改为16.5%免疫球蛋白肌肉注射,每次0.35ml/kg,每半月1次,连用6个月,结果发现,治疗后尿蛋白排泄由5.2g/d降至2.2g/d,血尿及白细胞尿消失,肾小球滤过率每月递减速率由-3.78ml/min减慢至0。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "免疫球蛋白" }, { "start_idx": 37, "end_idx": 41, "type": "dru", "entity": "免疫球蛋白" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "静脉注射" }, { "start_idx": 75, "end_idx": 79, "type": "dru", "entity": "免疫球蛋白" }, { "start_idx": 80, "end_idx": 83, "type": "pro", "entity": "肌肉注射" }, { "start_idx": 117, "end_idx": 119, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 138, "end_idx": 139, "type": "sym", "entity": "血尿" }, { "start_idx": 141, "end_idx": 144, "type": "sym", "entity": "白细胞尿" }, { "start_idx": 148, "end_idx": 153, "type": "ite", "entity": "肾小球滤过率" } ] }, { "text": "(四)鱼油IgA肾病患者缺乏必需脂肪酸,而鱼油(fishoil)可补充必需脂肪酸,从而防止早期的肾小球损害。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "鱼油" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "脂肪酸" }, { "start_idx": 21, "end_idx": 22, "type": "dru", "entity": "鱼油" }, { "start_idx": 24, "end_idx": 30, "type": "dru", "entity": "fishoil" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "脂肪酸" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "肾小球" } ] }, { "text": "鱼油富含长链ω-3-多聚不饱和脂肪酸、EPA(eicosapentaenoicacid)及DHA,这些物质可代替花生四烯酸,作为脂氧化酶和环氧化酶的底物而发挥作用,改变膜流动性,降低血小板聚集。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "鱼油" }, { "start_idx": 56, "end_idx": 60, "type": "bod", "entity": "花生四烯酸" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "脂氧化酶" }, { "start_idx": 69, "end_idx": 72, "type": "bod", "entity": "环氧化酶" }, { "start_idx": 84, "end_idx": 84, "type": "bod", "entity": "膜" }, { "start_idx": 91, "end_idx": 93, "type": "bod", "entity": "血小板" } ] }, { "text": "早在1984年Hamazaki收集20例IgA肾病患者做了初步研究,治疗组接受鱼油治疗1年,肾功能维持稳定,而未接受鱼油的对照组,则显示血浆肌酐清除率的降低。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 39, "end_idx": 40, "type": "dru", "entity": "鱼油" }, { "start_idx": 46, "end_idx": 48, "type": "ite", "entity": "肾功能" }, { "start_idx": 58, "end_idx": 59, "type": "dru", "entity": "鱼油" }, { "start_idx": 68, "end_idx": 74, "type": "ite", "entity": "血浆肌酐清除率" } ] }, { "text": "共收集55例病人,每日口服12g鱼油为治疗组,51例病人服橄榄油为对照组,所选病例中68%的基础血肌酐值增高,初始观察终点是血肌酐上升>50%,结果为在治疗期间(2年),鱼油组仅6%的病人进展到观察终点,而对照组达33%,每年血肌酐的增高速率在治疗组为0.03mg/dl,对照组为0.14mg/dl。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dru", "entity": "鱼油" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "橄榄油" }, { "start_idx": 48, "end_idx": 50, "type": "ite", "entity": "血肌酐" }, { "start_idx": 62, "end_idx": 64, "type": "ite", "entity": "血肌酐" }, { "start_idx": 85, "end_idx": 86, "type": "dru", "entity": "鱼油" }, { "start_idx": 113, "end_idx": 115, "type": "ite", "entity": "血肌酐" } ] }, { "text": "4年后的终末期肾病发生率,对照组为40%,治疗组则为10%,结果有统计学显著意义,没有病人因不良反应而停止治疗。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "终末期肾病" } ] }, { "text": "表明鱼油可减慢GFR的下降率。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dru", "entity": "鱼油" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "GFR" } ] }, { "text": "该作者在1999年又报道了上述病例远期随访结果,表明早期并持续使用鱼油可明显延缓高危IgA肾病患者的肾衰竭出现时间。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "dru", "entity": "鱼油" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "(五)其他Copp最近组织了一个为期6年的前瞻多中心双盲随机对照研究,以探讨长效服用贝那普利(benazepril),0.2mg/(kg•d),对中等程度蛋白尿、肾功能较好的儿童和青年IgA肾病患者的治疗功效,试验于2004年已完成。", "entities": [ { "start_idx": 42, "end_idx": 45, "type": "dru", "entity": "贝那普利" }, { "start_idx": 47, "end_idx": 56, "type": "dru", "entity": "benazepril" }, { "start_idx": 77, "end_idx": 79, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 81, "end_idx": 83, "type": "ite", "entity": "肾功能" }, { "start_idx": 92, "end_idx": 96, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "以往有人采用苯妥英钠5mg/(kg•d)治疗IgA肾病,发现可降低血清中IgA及多聚IgA水平,且血尿发作次数减少,但循环免疫复合物未减低,且远期疗效不肯定,近年已很少使用。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "苯妥英钠" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "血清" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "IgA" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "多聚IgA" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "血尿" }, { "start_idx": 59, "end_idx": 65, "type": "dru", "entity": "循环免疫复合物" } ] }, { "text": "(六)透析及肾移植对终末期肾衰患者可行透析及移植治疗。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "透析" }, { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "肾移植" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "终末期肾衰" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "透析" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "移植治疗" } ] }, { "text": "【预后】成人IgA肾病10年后约15%进展到终末肾功衰竭,20年后升至25%~30%。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "终末肾功衰竭" } ] }, { "text": "儿童IgA肾病预后好于成人,Yoshikawa报道20年后10%进展到终末肾衰竭。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "终末肾衰竭" } ] }, { "text": "影响预后的因素很多,重度蛋白尿、高血压、肾小球硬化以及间质小管病变严重均是预后不良的指标;男性也易于进展;肉眼血尿与预后的关系尚存争议。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "重度蛋白尿" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "高血压" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "间质小管病变严重" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "肉眼血尿" } ] }, { "text": "据报道,IgA肾病患者从肾功能正常起每年GFR的减低速度为1~3ml/min,而表现为肾病综合征的IgA肾病病人GFR递减率为9ml/min。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "肾功能" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "GFR" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "肾病综合征" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 56, "end_idx": 61, "type": "ite", "entity": "GFR递减率" } ] }, { "text": "合并高血压时,GFR减低速度更是高达每年12ml/min,因此,控制血压和蛋白尿在IgA肾病治疗中至关重要。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "高血压" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "GFR" }, { "start_idx": 34, "end_idx": 35, "type": "ite", "entity": "血压" }, { "start_idx": 37, "end_idx": 39, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "第四节黏多糖病【概述】黏多糖病(mucopolysaccharidosis,MPS)是一组遗传性溶酶体贮积症,因降解各种黏多糖所需的溶酶体酶缺陷,造成不能完全降解的黏多糖在溶酶体中贮积,并有大量黏多糖从尿中排出。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "黏多糖病" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "黏多糖病" }, { "start_idx": 16, "end_idx": 36, "type": "dis", "entity": "mucopolysaccharidosis" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "MPS" }, { "start_idx": 45, "end_idx": 53, "type": "dis", "entity": "遗传性溶酶体贮积症" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "黏多糖" }, { "start_idx": 66, "end_idx": 69, "type": "bod", "entity": "溶酶体酶" }, { "start_idx": 75, "end_idx": 104, "type": "sym", "entity": "不能完全降解的黏多糖在溶酶体中贮积,并有大量黏多糖从尿中排出" } ] }, { "text": "根据临床表现和不同的酶缺陷,可将MPS分为Ⅰ~Ⅶ型,除MPSⅡ型为X连锁隐性遗传外,其余均属常染色体隐性遗传。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "MPS" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "Ⅰ~Ⅶ型" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "MPSⅡ型" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "常染色体" } ] }, { "text": "MPS各型之间存在明显的遗传异质性,我国以MPSⅡ型和Ⅰ型最为常见,症状最为典型。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "MPS" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "MPSⅡ型" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "Ⅰ型" } ] }, { "text": "【病理生理和发病机制】黏多糖是结缔组织细胞间的主要成分,广泛存在于各种细胞内。", "entities": [ { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "细胞内" } ] }, { "text": "黏多糖是带阴性电荷的多聚物,重要的黏多糖有硫酸皮肤素(dermatansulfate,DS)、硫酸肝素(heparansulfate,HS)、硫酸角质素(keratansulfate,KS)、硫酸软骨素(chondroitinsulfate,CS)及透明质酸(hyaluronicacid,HA)等,前3种是黏多糖病的主要病理性黏多糖。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "黏多糖" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "黏多糖" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "硫酸皮肤素" }, { "start_idx": 27, "end_idx": 41, "type": "bod", "entity": "dermatansulfate" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "DS" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "硫酸肝素" }, { "start_idx": 52, "end_idx": 65, "type": "bod", "entity": "heparansulfate" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "HS" }, { "start_idx": 71, "end_idx": 75, "type": "bod", "entity": "硫酸角质素" }, { "start_idx": 77, "end_idx": 90, "type": "bod", "entity": "keratansulfate" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "KS" }, { "start_idx": 96, "end_idx": 100, "type": "bod", "entity": "硫酸软骨素" }, { "start_idx": 102, "end_idx": 119, "type": "bod", "entity": "chondroitinsulfate" }, { "start_idx": 121, "end_idx": 122, "type": "bod", "entity": "CS" }, { "start_idx": 125, "end_idx": 128, "type": "bod", "entity": "透明质酸" }, { "start_idx": 130, "end_idx": 143, "type": "bod", "entity": "hyaluronicacid" }, { "start_idx": 145, "end_idx": 146, "type": "bod", "entity": "HA" }, { "start_idx": 154, "end_idx": 157, "type": "dis", "entity": "黏多糖病" }, { "start_idx": 164, "end_idx": 166, "type": "bod", "entity": "黏多糖" } ] }, { "text": "这些多糖都是直链杂多糖,由不同的双糖单位连接而成,包括N-乙酰氨基己糖和糖醛酸或者己糖组成。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "多糖" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "双糖" }, { "start_idx": 27, "end_idx": 34, "type": "bod", "entity": "N-乙酰氨基己糖" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "糖醛酸" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "己糖" } ] }, { "text": "每个氨基葡糖聚糖直链约由50~100个分子组成,许多直链又同时与一条蛋白质肽链结合,形成更大分子量的聚合体。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "bod", "entity": "氨基葡糖聚糖直链" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "蛋白质肽链" } ] }, { "text": "结缔组织便是由这类聚合体所形成。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "结缔组织" } ] }, { "text": "这种多糖链的降解必须在溶酶体中进行。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "多糖链" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "溶酶体" } ] }, { "text": "正常溶酶体中含有许多种糖苷酶、硫酸脂酶和乙酸转移酶,不同的黏多糖需不同的溶酶体酶进行降解。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "溶酶体" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "糖苷酶" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "硫酸脂酶" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "乙酸转移酶" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "黏多糖" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "溶酶体酶" } ] }, { "text": "已知有10余种溶酶体酶参与其降解过程。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "溶酶体酶" } ] }, { "text": "其中任何一种酶的缺陷都会造成氨基葡糖聚糖链分解障碍,积聚在溶酶体内,尿中排出增加。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "酶" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "尿" }, { "start_idx": 14, "end_idx": 39, "type": "sym", "entity": "氨基葡糖聚糖链分解障碍,积聚在溶酶体内,尿中排出增加" } ] }, { "text": "各型黏多糖病的酶缺陷(表14-14)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "黏多糖病" } ] }, { "text": "【临床表现】黏多糖是结缔组织的主要成分,因此黏多糖代谢异常累及全身器官,患者一般出生时正常,随年龄增大,临床症状逐渐明显,其共同特征是在出生一年左右出现生长落后。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "黏多糖" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "结缔组织" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "黏多糖代谢异常" }, { "start_idx": 67, "end_idx": 79, "type": "sym", "entity": "在出生一年左右出现生长落后" } ] }, { "text": "关节进行性畸变,有爪形手、椎体发育不良呈扁平,短颈,鸡胸,肋下缘外突,脊柱后凸或者侧凸,早期出现肝、脾肿大,耳聋,心脏增大等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "关节" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "肋下缘" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "脊柱" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "心脏" }, { "start_idx": 0, "end_idx": 61, "type": "sym", "entity": "关节进行性畸变,有爪形手、椎体发育不良呈扁平,短颈,鸡胸,肋下缘外突,脊柱后凸或者侧凸,早期出现肝、脾肿大,耳聋,心脏增大等" } ] }, { "text": "表14-19黏多糖病的分型、酶的缺陷和累积的代谢产物目前对引起黏多糖病的酶缺陷都已鉴定,共分为6大型。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "黏多糖病" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "酶" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "黏多糖病" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "酶缺陷" } ] }, { "text": "1.黏多糖病I-H型(Hurler综合征)本型是最严重的一种类型,常在10岁左右死亡,病因为缺乏α-L-艾杜糖醛酸苷酶,导致硫酸皮肤素和硫酸肝素在体内积聚,全身脏器如角膜、软骨、骨骼、皮肤、心肌内膜及血管结缔组织等均受累。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "黏多糖病I-H型" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "Hurler综合征" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "常在10岁左右死亡" }, { "start_idx": 48, "end_idx": 58, "type": "bod", "entity": "α-L-艾杜糖醛酸苷酶" }, { "start_idx": 62, "end_idx": 66, "type": "bod", "entity": "硫酸皮肤素" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "硫酸肝素" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "脏器" }, { "start_idx": 83, "end_idx": 84, "type": "bod", "entity": "角膜" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "软骨" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "骨骼" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "皮肤" }, { "start_idx": 95, "end_idx": 98, "type": "bod", "entity": "心肌内膜" }, { "start_idx": 100, "end_idx": 105, "type": "bod", "entity": "血管结缔组织" } ] }, { "text": "末梢血白细胞、淋巴细胞可见到异染的大小不等,形态不同的深染颗粒,有时呈空泡状,尿排出大量酸性黏多糖((>100mg/24h,正常为3~25mg/24h)。", "entities": [ { "start_idx": 0, "end_idx": 75, "type": "sym", "entity": "末梢血白细胞、淋巴细胞可见到异染的大小不等,形态不同的深染颗粒,有时呈空泡状,尿排出大量酸性黏多糖((>100mg/24h,正常为3~25mg/24h)" } ] }, { "text": "本病是常染色体隐性遗传病,α-L-艾杜糖醛酸苷酶基因已被鉴定,位于染色体4p16.3,有14个外显子,并在其中发现了不少基因突变。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "常染色体隐性遗传病" }, { "start_idx": 13, "end_idx": 25, "type": "bod", "entity": "α-L-艾杜糖醛酸苷酶基因" }, { "start_idx": 33, "end_idx": 41, "type": "bod", "entity": "染色体4p16.3" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "外显子" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "基因突变" } ] }, { "text": "临床表型与基因型分析发现,α-L-艾杜糖醛酸苷酶基因突变导致酶活性严重缺乏者称为黏多糖病I-H型,如在编码区70,或402出现终止密码。", "entities": [ { "start_idx": 13, "end_idx": 27, "type": "sym", "entity": "α-L-艾杜糖醛酸苷酶基因突变" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "酶活性" }, { "start_idx": 40, "end_idx": 47, "type": "dis", "entity": "黏多糖病I-H型" } ] }, { "text": "基因突变导致酶活性中度或轻度下降者,在临床上分类为黏多糖病I-S型。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "基因突变" }, { "start_idx": 6, "end_idx": 8, "type": "ite", "entity": "酶活性" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "黏多糖病I-S型" } ] }, { "text": "2.黏多糖病I-S型(Scheie综合征)原先分类为黏多糖病V型,属中等度严重类型黏多糖病,遗传类型和致病基因同黏多糖病I-H型。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "黏多糖病I-S型" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "Scheie综合征" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "黏多糖病V型" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "黏多糖病" }, { "start_idx": 56, "end_idx": 63, "type": "dis", "entity": "黏多糖病I-H型" } ] }, { "text": "智能发育正常,临床症状一般在5岁后出现。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "智能发育正常,临床症状一般在5岁后出现" } ] }, { "text": "3.黏多糖病Ⅱ型(Hunter综合征)临床重型与黏多糖I-H型相似,在2~6岁起病,有特殊面容和骨骼畸形,但脊椎无鸟嘴样畸形。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "黏多糖病Ⅱ型" }, { "start_idx": 9, "end_idx": 17, "type": "dis", "entity": "Hunter综合征" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "黏多糖I-H型" }, { "start_idx": 34, "end_idx": 61, "type": "sym", "entity": "在2~6岁起病,有特殊面容和骨骼畸形,但脊椎无鸟嘴样畸形" } ] }, { "text": "无角膜混浊。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "无角膜混浊" } ] }, { "text": "本型为X连锁隐性遗传,病因是艾杜糖醛酸硫酸酯酶缺陷,使硫酸皮肤素和硫酸肝素代谢障碍。", "entities": [ { "start_idx": 14, "end_idx": 22, "type": "bod", "entity": "艾杜糖醛酸硫酸酯酶" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "硫酸皮肤素" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "硫酸肝素" } ] }, { "text": "致病基因已克隆,位于染色体Xq28区,在脆性X综合征区域附近,有9个外显子,基因分析发现较多病人有大片段缺失,其他形式有点突变、小片段缺失或插入。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "染色体Xq28区" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "脆性X综合征" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "外显子" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "基因分析" }, { "start_idx": 44, "end_idx": 71, "type": "sym", "entity": "较多病人有大片段缺失,其他形式有点突变、小片段缺失或插入" } ] }, { "text": "有严重病变,如在核苷酸1129位上插入22个碱基或有基因缺失者病情较重,基因有点突变者相对属轻型临床表现。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "bod", "entity": "核苷酸1129位" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "碱基" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "基因缺失" } ] }, { "text": "4.黏多糖病Ⅲ型(Sanfilippo综合征)临床可分为4种亚型,分别由4种不同的酶缺陷所引起。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "黏多糖病Ⅲ型" }, { "start_idx": 9, "end_idx": 21, "type": "dis", "entity": "Sanfilippo综合征" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "酶缺陷" } ] }, { "text": "上述4种酶都是硫酸肝素降解所需要的酶,因此,当这些酶缺乏时均引起硫酸肝素在体内积聚,同时尿中排出量增加。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "酶" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "硫酸肝素" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "酶" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "酶缺乏" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "硫酸肝素" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "尿" } ] }, { "text": "本病4型均为常染色体隐性遗传性疾病,导致硫酸肝素在体内积聚。", "entities": [ { "start_idx": 6, "end_idx": 16, "type": "dis", "entity": "常染色体隐性遗传性疾病" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "硫酸肝素" } ] }, { "text": "ⅢD型的致病基因葡糖胺-6-硫酸酯酶已克隆,位于染色体12q14。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "ⅢD型" }, { "start_idx": 8, "end_idx": 17, "type": "bod", "entity": "葡糖胺-6-硫酸酯酶" }, { "start_idx": 24, "end_idx": 31, "type": "bod", "entity": "染色体12q14" } ] }, { "text": "5.黏多糖病Ⅳ型(Morquio综合征)临床特征与黏多糖病I-H型相似,但无智能障碍。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "黏多糖病Ⅳ型" }, { "start_idx": 9, "end_idx": 18, "type": "dis", "entity": "Morquio综合征" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "黏多糖病I-H型" } ] }, { "text": "两种亚型都属常染色体隐性遗传性疾病。", "entities": [ { "start_idx": 6, "end_idx": 16, "type": "dis", "entity": "常染色体隐性遗传性疾病" } ] }, { "text": "N-乙酰半乳糖胺-6-硫酸酯酶的全长cDNA已克隆,基因定位于染色体16q24.3,并在此基因上发现了一些突变位点。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "bod", "entity": "N-乙酰半乳糖胺-6-硫酸酯酶" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "cDNA" }, { "start_idx": 31, "end_idx": 40, "type": "bod", "entity": "染色体16q24.3" } ] }, { "text": "β-半乳糖酶基因也已克隆,定位于染色体3q21.33,并找到了突变位点。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "β-半乳糖酶基因" }, { "start_idx": 16, "end_idx": 25, "type": "bod", "entity": "染色体3q21.33" } ] }, { "text": "6.黏多糖病Ⅵ型(Maroteaux-Lamy综合征)临床表现同黏多糖病I-H型相似,但无智能落后。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "黏多糖病Ⅵ型" }, { "start_idx": 9, "end_idx": 25, "type": "dis", "entity": "Maroteaux-Lamy综合征" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "黏多糖病I-H型" } ] }, { "text": "尿中排出大量硫酸皮肤素,致病基因为芳基硫酸酯酶B,基因定位于染色体5q13-13q14,属常染色体隐性遗传性疾病。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "尿中排出大量硫酸皮肤素" }, { "start_idx": 17, "end_idx": 23, "type": "bod", "entity": "芳基硫酸酯酶B" }, { "start_idx": 30, "end_idx": 42, "type": "bod", "entity": "染色体5q13-13q14" }, { "start_idx": 45, "end_idx": 55, "type": "dis", "entity": "常染色体隐性遗传性疾病" } ] }, { "text": "7.黏多糖病Ⅶ型临床表现同黏多糖病I-H型,但个体轻重程度不一,变异较大,轻者可无智能落后,本型为常染色体隐性遗传性疾病,因β-葡萄糖醛酸酶缺乏,导致4/6硫酸软骨素在体内沉积。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "黏多糖病Ⅶ型" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "黏多糖病I-H型" }, { "start_idx": 49, "end_idx": 59, "type": "dis", "entity": "常染色体隐性遗传性疾病" }, { "start_idx": 62, "end_idx": 71, "type": "sym", "entity": "β-葡萄糖醛酸酶缺乏" }, { "start_idx": 78, "end_idx": 82, "type": "bod", "entity": "硫酸软骨素" } ] }, { "text": "基因定位于染色体7q21.11,有12个外显子。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "bod", "entity": "染色体7q21.11" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "外显子" } ] }, { "text": "【实验室检查】1.尿黏多糖测定通常用甲苯胺蓝法做定性试验,患者尿液呈阳性反应。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "尿黏多糖测定" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "甲苯胺蓝法" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "尿液呈阳性反应" } ] }, { "text": "2.骨骼X线检查骨质较疏松,骨皮质变薄,颅骨增大蝶鞍增大后凸或侧凸,椎体呈楔形或扁平,胸、腰椎体前下缘呈鱼唇样前突或呈鸟嘴突肋骨脊柱端细小胸骨端增宽飘带状,掌骨短粗,基底变尖,指骨远端窄圆,腕骨骨化成熟延迟。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "骨质较疏松" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "骨皮质变薄" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "颅骨" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "颅骨增大" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "蝶鞍" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "蝶鞍增大" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "后凸或侧凸" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "腰椎体" }, { "start_idx": 43, "end_idx": 61, "type": "sym", "entity": "胸、腰椎体前下缘呈鱼唇样前突或呈鸟嘴突" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "肋骨脊柱" }, { "start_idx": 62, "end_idx": 68, "type": "sym", "entity": "肋骨脊柱端细小" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "胸骨" }, { "start_idx": 69, "end_idx": 73, "type": "sym", "entity": "胸骨端增宽" }, { "start_idx": 74, "end_idx": 76, "type": "sym", "entity": "飘带状" }, { "start_idx": 78, "end_idx": 81, "type": "sym", "entity": "掌骨短粗" }, { "start_idx": 83, "end_idx": 86, "type": "sym", "entity": "基底变尖" }, { "start_idx": 88, "end_idx": 93, "type": "sym", "entity": "指骨远端窄圆" }, { "start_idx": 95, "end_idx": 102, "type": "sym", "entity": "腕骨骨化成熟延迟" } ] }, { "text": "3.醋酸纤维薄膜电泳可以区分尿中排出黏多糖的种类,并进行分型。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "醋酸纤维薄膜电泳" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "尿" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "黏多糖" } ] }, { "text": "4.酶学分析通过测定外周血白细胞,成纤维细胞中的特异性酶活性,以及尿中排出的黏多糖类型,可以对黏多糖病分型。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "酶学分析" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "外周血白细胞" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "成纤维细胞" }, { "start_idx": 24, "end_idx": 29, "type": "ite", "entity": "特异性酶活性" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "尿" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "黏多糖" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "黏多糖病" } ] }, { "text": "【诊断】1.根据临床特殊面容和体征、X线片表现以及尿黏多糖阳性,可以作出诊断。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "特殊面容" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "尿黏多糖阳性" } ] }, { "text": "2.家族史中有黏多糖病人对早期诊断有帮助。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "黏多糖" } ] }, { "text": "本病应与佝偻病、先天性甲状腺功能减低症、粘脂病各型、甘露糖贮积病以及GM1神经节苷脂沉积病等鉴别,这些疾病临床表现与黏多糖病相似,但尿中黏多糖排量不增加。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "佝偻病" }, { "start_idx": 8, "end_idx": 18, "type": "dis", "entity": "先天性甲状腺功能减低症" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "粘脂病各型" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "甘露糖贮积病" }, { "start_idx": 34, "end_idx": 44, "type": "dis", "entity": "GM1神经节苷脂沉积病" }, { "start_idx": 58, "end_idx": 61, "type": "dis", "entity": "黏多糖病" }, { "start_idx": 66, "end_idx": 75, "type": "sym", "entity": "尿中黏多糖排量不增加" } ] }, { "text": "【治疗】目前对各型黏多糖病无病因治疗方法,可做一些对症处理。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "黏多糖病" } ] }, { "text": "【预后】本组未治疗病例随年龄增大而病情加重,出现进行性智能障碍,骨、关节症状加重肺炎或心脏病,少数类型可存活至成人。", "entities": [ { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "进行性智能障碍" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "骨" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "关节" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "骨、关节症状加重" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "肺炎" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "心脏病" } ] }, { "text": "参考文献1.彭文伟.传染病学.第3版.北京:人民卫生出版社,19922.杨永弘.小儿细菌性肺炎及其病原学诊断.中华儿科杂志,1992,30(4):2563.杨永弘.儿科细菌感染性疾病及其病原学诊断中的几个问题.中华儿科杂志,1993,31(1):66-684.朱忠勇.实用医学检验学.北京:人民军医出版社,19925.李苏利.临床微生物检验的快速诊断技术研究进展.当代医学,2009,16:15-15", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "传染病" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 84, "end_idx": 90, "type": "dis", "entity": "细菌感染性疾病" }, { "start_idx": 165, "end_idx": 167, "type": "mic", "entity": "微生物" }, { "start_idx": 171, "end_idx": 176, "type": "pro", "entity": "快速诊断技术" } ] }, { "text": "四、Addis计数Addis计数是测定夜间12小时尿液中有形成分的数量,可以定量地反映尿中细胞及管型的数量。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "Addis计数" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "Addis计数" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "尿液" }, { "start_idx": 43, "end_idx": 43, "type": "ite", "entity": "尿" }, { "start_idx": 45, "end_idx": 46, "type": "ite", "entity": "细胞" } ] }, { "text": "正常参考值为管型0~5000个/12h尿,红细胞0~50万个/12h尿,白细胞0~100万个/12h尿。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "管型" }, { "start_idx": 19, "end_idx": 19, "type": "ite", "entity": "尿" }, { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "红细胞" }, { "start_idx": 34, "end_idx": 34, "type": "ite", "entity": "尿" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "白细胞" }, { "start_idx": 50, "end_idx": 50, "type": "ite", "entity": "尿" } ] }, { "text": "收集3小时尿再计算出1小时尿有形成分排泄率也可定量反映尿改变。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "ite", "entity": "尿" }, { "start_idx": 13, "end_idx": 13, "type": "ite", "entity": "尿" }, { "start_idx": 27, "end_idx": 27, "type": "ite", "entity": "尿" } ] }, { "text": "成人正常值为红细胞:男<3万个/h,女<4万/h;白细胞:男<7万个/h,女<14万个/h,小儿正常值为成人的1~2倍。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "ite", "entity": "红细胞" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "白细胞" } ] }, { "text": "第十四节流行性出血热流行性出血热(epidemichemorrhagicfever,EHF)是由汉坦病毒引起的自然疫源性疾病。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "流行性出血热" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "流行性出血热" }, { "start_idx": 17, "end_idx": 40, "type": "dis", "entity": "epidemichemorrhagicfever" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "EHF" }, { "start_idx": 48, "end_idx": 51, "type": "mic", "entity": "汉坦病毒" } ] }, { "text": "临床上以发热、出血、周围循环衰竭及肾脏损害为特征。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "发热" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "出血" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "周围循环衰竭" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肾脏" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "肾脏损害" } ] }, { "text": "【病原和流行病学】汉坦病毒(hantaviruses,HVs)归类为布尼亚病毒科汉坦病毒属。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "汉坦病毒" }, { "start_idx": 14, "end_idx": 25, "type": "mic", "entity": "hantaviruses" }, { "start_idx": 27, "end_idx": 29, "type": "mic", "entity": "HVs" }, { "start_idx": 34, "end_idx": 38, "type": "mic", "entity": "布尼亚病毒" }, { "start_idx": 40, "end_idx": 43, "type": "mic", "entity": "汉坦病毒" } ] }, { "text": "其中9个主要型别为汉滩型(hantaanvirus,HTNV)、汉城型(SEOV)、普马拉型(PUUV)、希望山型(PHV)、泰国型(ThAIV)、印度型(TPMV)、多布拉伐-贝尔格莱德型(DOBV)、无名病毒型(SNV)和纽约型(NYV)。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "汉滩型" }, { "start_idx": 13, "end_idx": 24, "type": "mic", "entity": "hantaanvirus" }, { "start_idx": 26, "end_idx": 29, "type": "mic", "entity": "HTNV" }, { "start_idx": 32, "end_idx": 34, "type": "mic", "entity": "汉城型" }, { "start_idx": 36, "end_idx": 39, "type": "mic", "entity": "SEOV" }, { "start_idx": 42, "end_idx": 45, "type": "mic", "entity": "普马拉型" }, { "start_idx": 47, "end_idx": 50, "type": "mic", "entity": "PUUV" }, { "start_idx": 53, "end_idx": 56, "type": "mic", "entity": "希望山型" }, { "start_idx": 58, "end_idx": 60, "type": "mic", "entity": "PHV" }, { "start_idx": 63, "end_idx": 65, "type": "mic", "entity": "泰国型" }, { "start_idx": 67, "end_idx": 71, "type": "mic", "entity": "ThAIV" }, { "start_idx": 74, "end_idx": 76, "type": "mic", "entity": "印度型" }, { "start_idx": 78, "end_idx": 81, "type": "mic", "entity": "TPMV" }, { "start_idx": 84, "end_idx": 94, "type": "mic", "entity": "多布拉伐-贝尔格莱德型" }, { "start_idx": 96, "end_idx": 99, "type": "mic", "entity": "DOBV" }, { "start_idx": 102, "end_idx": 105, "type": "mic", "entity": "无名病毒" }, { "start_idx": 108, "end_idx": 110, "type": "mic", "entity": "SNV" }, { "start_idx": 113, "end_idx": 115, "type": "mic", "entity": "纽约型" }, { "start_idx": 117, "end_idx": 119, "type": "mic", "entity": "NYV" } ] }, { "text": "我国目前仅发现汉滩型和汉城型,其宿主分别为黑线姬鼠和褐家鼠。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "汉滩型" }, { "start_idx": 11, "end_idx": 13, "type": "mic", "entity": "汉城型" } ] }, { "text": "病毒基因由3个负链RNA环状分子组成,外被核衣壳和包膜。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "病毒基因" } ] }, { "text": "包膜含血凝素抗原,在病毒黏附、穿入和脱衣壳中起重要作用。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "病毒" } ] }, { "text": "病毒可在多种细胞或细胞株中生长,但致细胞病变的作用较弱,其生长缓慢,病毒滴度一般在接种后7~14天达高峰。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 34, "end_idx": 35, "type": "mic", "entity": "病毒" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "接种" } ] }, { "text": "病毒对外环境抵抗力不强,75%乙醇、乙醚等脂溶剂、0.5%碘酒、戊二醛、酸(pH3~5)、56℃30分钟、紫外线可使之灭活。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 15, "end_idx": 16, "type": "dru", "entity": "乙醇" }, { "start_idx": 18, "end_idx": 19, "type": "dru", "entity": "乙醚" }, { "start_idx": 29, "end_idx": 30, "type": "dru", "entity": "碘酒" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "戊二醛" } ] }, { "text": "带病毒的鼠类是主要传染源和储存宿主。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "mic", "entity": "病毒" } ] }, { "text": "病毒有多种传播途径,以动物源传播为主,即人接触带病毒动物排泄物而感染;带毒排泄物形成的气溶胶能经呼吸道感染人体;其他如消化道及虫媒(寄生虫与带毒动物身上的螨类叮咬人体)传播也已证明,罕见人与人之间传播的报道。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "病毒" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "呼吸道" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "呼吸道感染" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "消化道" }, { "start_idx": 66, "end_idx": 68, "type": "mic", "entity": "寄生虫" } ] }, { "text": "已报道全球有78个国家和地区的人和动物感染汉坦病毒,我国疫情最重,占发病人数的90%以上,1997~1999年全国年报告病例达4.1万~4.6万余,2000年和2001年分别为35810和33872例。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "mic", "entity": "汉坦病毒" } ] }, { "text": "【发病机制和病理】EHF的发病机制并不十分明了。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "EHF" } ] }, { "text": "一般认为,早期损伤是病毒直接致病作用所致,晚期则是病毒介导的免疫性损伤。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "病毒" }, { "start_idx": 25, "end_idx": 26, "type": "mic", "entity": "病毒" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "免疫性损伤" } ] }, { "text": "HVs的组织细胞嗜性广泛,血管内皮细胞和多种免疫细胞如T细胞、B细胞、单核/巨噬细胞、脑胶质细胞及肝库普弗细胞等为其靶细胞。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "HVs" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "血管内皮细胞" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "T细胞" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "B细胞" }, { "start_idx": 35, "end_idx": 41, "type": "bod", "entity": "单核/巨噬细胞" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "脑胶质细胞" }, { "start_idx": 49, "end_idx": 54, "type": "bod", "entity": "肝库普弗细胞" } ] }, { "text": "病毒侵入人体后,首先累及单核-吞噬细胞系统,病毒入血致短暂病毒血症后出现全身广泛微血管损伤,表现为微血管充血、水肿、变性和坏死。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 12, "end_idx": 20, "type": "bod", "entity": "单核-吞噬细胞系统" }, { "start_idx": 22, "end_idx": 23, "type": "mic", "entity": "病毒" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "血" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "病毒血症" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "微血管" }, { "start_idx": 36, "end_idx": 44, "type": "sym", "entity": "全身广泛微血管损伤" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "微血管" }, { "start_idx": 49, "end_idx": 62, "type": "sym", "entity": "微血管充血、水肿、变性和坏死" } ] }, { "text": "血管通透性增加,血浆大量外渗,致有效循环血量减少。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血管" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "血管通透性增加" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "血浆" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "血浆大量外渗" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "有效循环血量减少" } ] }, { "text": "肾血流量减少和其滤过率下降,经刺激肾素-血管紧张素-醛固酮系统分泌增多导致少尿。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肾" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "肾血流量减少和其滤过率下降" }, { "start_idx": 17, "end_idx": 30, "type": "bod", "entity": "肾素-血管紧张素-醛固酮系统" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "尿" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "少尿" } ] }, { "text": "肾缺血致肾小管上皮坏死,加上肾间质水肿使肾小管狭窄甚至闭塞,加重少尿。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肾" }, { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "肾缺血" }, { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "肾小管上皮" }, { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "肾小管上皮坏死" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肾间质" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "肾间质水肿" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肾小管" }, { "start_idx": 20, "end_idx": 28, "type": "sym", "entity": "肾小管狭窄甚至闭塞" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "尿" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "少尿" } ] }, { "text": "大量免疫复合物沉积于血管和肾小球基底膜,参与血管壁和肾脏损伤机制。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "血管" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "肾小球基底膜" }, { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "大量免疫复合物沉积于血管和肾小球基底膜" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "血管壁" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肾脏" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "肾脏损伤" } ] }, { "text": "患者早期血中IgE明显增高,并可检出自身抗体,提示存在Ⅰ型和Ⅱ型变态反应。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "血" }, { "start_idx": 4, "end_idx": 12, "type": "sym", "entity": "血中IgE明显增高" } ] }, { "text": "最近研究显示,患者免疫细胞增生活跃,CD8+T细胞增加,NK细胞活性增强,认为细胞免疫在病毒清除和致病机制中起双重作用。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "免疫细胞" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "免疫细胞增生活跃" }, { "start_idx": 18, "end_idx": 37, "type": "sym", "entity": "CD8+T细胞增加" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "NK细胞" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "NK细胞活性增强" }, { "start_idx": 55, "end_idx": 56, "type": "mic", "entity": "病毒" } ] }, { "text": "典型病理改变为多发性出血、严重渗出和水肿及灶状坏死和细胞浸润。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "多发性出血" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "严重渗出" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "水肿" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "灶状坏死" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "细胞浸润" } ] }, { "text": "肾脏病变广泛,髓质充血、出血;肾小管上皮肿胀、变性、坏死;内皮脱落;包膜紧张可至肾破裂;全尿路均可出血。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肾脏" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "肾脏病变" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "髓质" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "髓质充血、出血" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "肾小管上皮" }, { "start_idx": 15, "end_idx": 27, "type": "sym", "entity": "肾小管上皮肿胀、变性、坏死" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "内皮脱落" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "包膜" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "肾" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "包膜紧张可至肾破裂" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "尿路" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "全尿路均可出血" } ] }, { "text": "心脏以右心房病变多见,心壁细胞变性、浸润及出血;纤维断裂。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心脏" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "右心房" }, { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "右心房病变" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "心壁细胞" }, { "start_idx": 11, "end_idx": 22, "type": "sym", "entity": "心壁细胞变性、浸润及出血" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "纤维断裂" } ] }, { "text": "脑垂体肿大,前叶明显充血、出血和坏死。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑垂体" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "前叶" }, { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "脑垂体肿大,前叶明显充血、出血和坏死" } ] }, { "text": "腹膜后胶冻样水肿为本病所特有。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "腹膜" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "腹膜后胶冻样水肿" } ] }, { "text": "(一)发热期相当于病毒血症期。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "病毒血症" } ] }, { "text": "血及尿中有病毒存在,故有传染性。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "血" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "尿" }, { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "病毒" } ] }, { "text": "主要表现有:1.发热及中毒症状几乎均有发热,多急起,体温可达38~40℃,以弛张热、稽留热型为多,热程一般3~7天。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "发热" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "中毒" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "发热" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "弛张热" }, { "start_idx": 42, "end_idx": 44, "type": "sym", "entity": "稽留热" } ] }, { "text": "常伴三痛症:头痛、腰痛及眼眶痛。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "头痛" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "腰痛" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "眼眶痛" } ] }, { "text": "由于脑膜及脑实质血管充血、水肿及出血,引起剧烈头痛。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "脑膜" }, { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "脑实质血管" }, { "start_idx": 2, "end_idx": 17, "type": "sym", "entity": "脑膜及脑实质血管充血、水肿及出血" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "剧烈头痛" } ] }, { "text": "腰痛和肾区叩痛与肾充血、水肿、包膜紧张及腹膜后水肿有关;眼眶痛系眼球周围软组织水肿出血引起眶压增高所致。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "腰痛" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾区" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "肾区叩痛" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肾" }, { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "肾充血" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "水肿" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "包膜" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "包膜紧张" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "腹膜" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "腹膜后水肿" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "眼眶痛" }, { "start_idx": 32, "end_idx": 38, "type": "bod", "entity": "眼球周围软组织" }, { "start_idx": 32, "end_idx": 42, "type": "sym", "entity": "眼球周围软组织水肿出血" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "眶压增高" } ] }, { "text": "患者常有口渴、食欲下降、恶心、呕吐和腹痛、腹泻。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "口渴" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "食欲下降" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "恶心" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "呕吐" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "腹痛" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "腹泻" } ] }, { "text": "大便可带黏血。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "大便" }, { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "大便可带黏血" } ] }, { "text": "重患者可有嗜睡或兴奋不安、谵语等。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "嗜睡" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "兴奋不安" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "谵语" } ] }, { "text": "2.毛细血管损害包括:①三红症:眼结膜、颜面、颈及上胸部明显充血潮红,呈酒醉貌;②出血:软腭、球结膜、腋下和胸背皮肤见针尖大小出血点,呈特征性搔抓样或条痕样排列。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "毛细血管" }, { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "毛细血管损害" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "眼结膜" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "颜面" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "颈" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "上胸部" }, { "start_idx": 16, "end_idx": 33, "type": "sym", "entity": "眼结膜、颜面、颈及上胸部明显充血潮红" }, { "start_idx": 36, "end_idx": 38, "type": "sym", "entity": "酒醉貌" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "出血" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "软腭" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "球结膜" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "腋下" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "胸背皮肤" }, { "start_idx": 44, "end_idx": 79, "type": "sym", "entity": "软腭、球结膜、腋下和胸背皮肤见针尖大小出血点,呈特征性搔抓样或条痕样排列" } ] }, { "text": "重症见大片皮肤瘀斑,血尿、呕血及便血,束臂试验强阳性;③渗出:常见球结膜和眼睑水肿,面部、四肢亦可肿胀,可有腹水。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "皮肤" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "大片皮肤瘀斑" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "血尿" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "呕血" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "便血" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "束臂试验强阳性" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "球结膜" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "眼睑" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "球结膜和眼睑水肿" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "面部" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "四肢" }, { "start_idx": 42, "end_idx": 50, "type": "sym", "entity": "面部、四肢亦可肿胀" }, { "start_idx": 53, "end_idx": 55, "type": "sym", "entity": "有腹水" } ] }, { "text": "3.肾脏损害蛋白尿为最常见的早期表现之一,可有血尿和尿量减少。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肾脏" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "肾脏损害" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "血尿" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "尿量减少" } ] }, { "text": "(二)低血压休克期发热后期(一般在病后4~6天)或退热同时可有血压下降甚至休克,呈现“热退症更重”的特点。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "休克" }, { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "低血压休克" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "发热" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "退热" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "血压" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "血压下降" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "休克" } ] }, { "text": "表现为:低血压:收缩压下降,脉压变小、脉快而弱、球结膜水肿,尿少,烦躁不安等;②休克:收缩压低于9.3kPa,脉压小于2.6kPa。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "低血压" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "收缩压" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "收缩压下降" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "脉压" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "脉压变小" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "脉快而弱" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "球结膜" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "球结膜水肿" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "尿" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "尿少" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "休克" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "收缩压" } ] }, { "text": "脉细弱或扪不到,球结膜水肿更重,出现明显消化道及精神神经症状。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "脉细弱或扪不到" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "球结膜" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "球结膜水肿更重" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "消化道" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "消化道及精神神经症状" } ] }, { "text": "尿少、蛋白尿及出血症状更明显。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "尿" }, { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "尿少" }, { "start_idx": 3, "end_idx": 5, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "出血" } ] }, { "text": "(三)少尿期多发生于病程第5~8天,持续约2~5天。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "少尿" } ] }, { "text": "症状以急性肾衰竭为主,表现为尿毒症、酸中毒、电解质紊乱、高血容量综合征(表现为脉搏洪大,静脉充血、水肿、血红蛋白下降等)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "急性肾衰竭" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "尿毒症" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "酸中毒" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "电解质紊乱" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "高血容量综合征" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "脉搏洪大" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "静脉" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "静脉充血" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "水肿" }, { "start_idx": 52, "end_idx": 55, "type": "ite", "entity": "血红蛋白" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "血红蛋白下降" } ] }, { "text": "前述各期的症状可加重,可因颅内压增高出现烦躁、谵妄甚至昏迷、抽搐等。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "颅内压" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "烦躁" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "谵妄" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "昏迷" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "抽搐" } ] }, { "text": "(四)多尿期于病程第9~14天进入多尿期,持续1~2周,可分为移行阶段(尿量从少尿增至正常尿量)、多尿早期和多尿后期。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "多尿" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "尿" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "多尿" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "尿量" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "尿" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "多尿" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "尿" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "多尿" } ] }, { "text": "在前二阶段,氮质血症仍逐日上升,症状可继续加重。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "氮质血症" } ] }, { "text": "进入多尿后期,患者症状逐渐减轻,氮质血症好转,酸中毒和高血容量得以纠正。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "尿" }, { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "多尿" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "氮质血症" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "酸中毒" }, { "start_idx": 23, "end_idx": 34, "type": "sym", "entity": "酸中毒和高血容量得以纠正" } ] }, { "text": "若过度利尿,失水、失盐,加之继发感染或出血等可诱发第二次休克或再次肾衰竭。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "继发感染" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "出血" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "休克" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "尿量减少至肾功能恢复,症状体征消失,各种化验逐渐正常。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "尿量" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "尿量减少" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "肾功能恢复" } ] }, { "text": "小儿出血热临床经过与成人基本相似,但5个病期经过不完全,临床表现除消化道症状明显外,其他症状与体征较成人轻,预后较好。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "小儿出血热" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "消化道" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "消化道症状明显" } ] }, { "text": "【实验室检查】(一)常规检查1.血常规检查白细胞总数于病程第3~4天开始增高,15×109~30×109/L,少数高达50×109/L以上。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "血常规" }, { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "白细胞总数" } ] }, { "text": "早期中性粒细胞增高,核左移,可见中毒颗粒和类白血病反应;病程第5~8天后淋巴细胞增高;异淋出现于病程早期。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "中性粒细胞增高" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "核左移" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "类白血病" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "淋巴细胞增高" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "异淋" } ] }, { "text": "血红蛋白因血液浓缩而升高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "血红蛋白" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "血液" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "血红蛋白因血液浓缩而升高" } ] }, { "text": "血小板有不同程度下降,DIC时下降更明显。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "血小板有不同程度下降" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "DIC" } ] }, { "text": "2.尿常规蛋白尿是出血热肾损害的最早征象。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "尿常规" }, { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "出血热肾损害" } ] }, { "text": "还可有血尿及管型尿。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "血尿" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "管型尿" } ] }, { "text": "少数病例尿中出现膜状物,为凝血块、蛋白和脱落上皮细胞的混合物。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "尿" }, { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "尿中出现膜状物" } ] }, { "text": "尿中溶菌酶、N-乙酰氨基糖苷酶(NAG)亦可阳性。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "尿" }, { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "溶菌酶" }, { "start_idx": 6, "end_idx": 14, "type": "mic", "entity": "N-乙酰氨基糖苷酶" }, { "start_idx": 16, "end_idx": 18, "type": "mic", "entity": "NAG" } ] }, { "text": "(二)血生化检查发热晚期血尿素氮及肌酐开始升高,少尿及多尿早期达高峰,常见代谢性酸中毒合并呼吸性碱中毒。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "血生化检查" }, { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "血尿素氮" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "肌酐" }, { "start_idx": 12, "end_idx": 22, "type": "sym", "entity": "血尿素氮及肌酐开始升高" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "尿" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "少尿" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "尿" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "多尿" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "酸中毒" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "代谢性酸中毒" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "呼吸性碱中毒" } ] }, { "text": "血钠、氯、钙在全病程均降低,磷、镁、铁升高。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "血钠" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "血钠、氯、钙在全病程均降低" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "磷、镁、铁升高" } ] }, { "text": "血钾在发热及休克期降低,少尿期升高。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "血钾" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "尿" }, { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "血钾在发热及休克期降低,少尿期升高" } ] }, { "text": "心肌受损时,血清肌酸磷酸激酶、乳酸脱氢酶和肌红蛋白升高。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心肌" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "心肌受损" }, { "start_idx": 6, "end_idx": 13, "type": "bod", "entity": "血清肌酸磷酸激酶" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "乳酸脱氢酶" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "肌红蛋白" }, { "start_idx": 6, "end_idx": 26, "type": "sym", "entity": "血清肌酸磷酸激酶、乳酸脱氢酶和肌红蛋白升高" } ] }, { "text": "(三)病原学检查病毒分离及鉴定取患者急性期血液、尿液或尸检材料制成10%悬液,接种于敏感单层细胞上,由于CPE不典型,需用免疫荧光法测定细胞内特异性抗原而检出病毒。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "病毒分离及鉴定" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "血液" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "尿液" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "接种" }, { "start_idx": 61, "end_idx": 65, "type": "pro", "entity": "免疫荧光法" }, { "start_idx": 79, "end_idx": 80, "type": "mic", "entity": "病毒" } ] }, { "text": "2.病毒抗原检测最早采用免疫荧光法检测组织细胞中的病毒抗原,早期患者白细胞中病毒抗原检出率达90%以上。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "病毒抗原检测" }, { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "免疫荧光法" }, { "start_idx": 25, "end_idx": 28, "type": "mic", "entity": "病毒抗原" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "白细胞" }, { "start_idx": 38, "end_idx": 41, "type": "mic", "entity": "病毒抗原" } ] }, { "text": "用免疫酶技术检测患者血和体液中病毒抗原敏感性低,早期标本阳性率不足20%,但用于组织内病毒抗原检测,阳性率可达100%。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "免疫酶技术" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "血" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "体液" }, { "start_idx": 15, "end_idx": 18, "type": "mic", "entity": "病毒抗原" }, { "start_idx": 43, "end_idx": 48, "type": "pro", "entity": "病毒抗原检测" } ] }, { "text": "3.特异性抗体测定特异性IgM出现早,感染1周后达高峰,维持半年左右,是近期感染的指标;双份血清特异性IgG≥4倍增高有确诊价值。", "entities": [ { "start_idx": 46, "end_idx": 53, "type": "ite", "entity": "血清特异性IgG" } ] }, { "text": "检测方法有免疫荧光法和ELISA法,将重组病毒抗原用于ELISA检测,敏感性提高。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "免疫荧光法" }, { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "ELISA法" }, { "start_idx": 21, "end_idx": 24, "type": "mic", "entity": "病毒抗原" }, { "start_idx": 27, "end_idx": 33, "type": "pro", "entity": "ELISA检测" } ] }, { "text": "4.病毒基因检测用原位杂交法和RT-PCR技术可检测组织细胞内病毒基因片段。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "病毒基因检测" }, { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "原位杂交法" }, { "start_idx": 15, "end_idx": 22, "type": "pro", "entity": "RT-PCR技术" }, { "start_idx": 31, "end_idx": 34, "type": "mic", "entity": "病毒基因" } ] }, { "text": "后者还是对汉坦病毒分离物进行基因分型的可靠方法之一。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "mic", "entity": "汉坦病毒" } ] }, { "text": "根据流行病学资料如在流行季节;发病前两个月内有疫区居住或逗留史;临床上有发热和中毒症状,充血、出血、渗出体征,以及肾损害三大主征和病程发展的五期经过,实验室检查有典型血、尿常规和血生化改变可作出临床诊断。", "entities": [ { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "发热" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "中毒" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "充血" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "出血" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "渗出" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "肾" }, { "start_idx": 57, "end_idx": 59, "type": "sym", "entity": "肾损害" }, { "start_idx": 83, "end_idx": 87, "type": "ite", "entity": "血、尿常规" }, { "start_idx": 89, "end_idx": 91, "type": "ite", "entity": "血生化" }, { "start_idx": 83, "end_idx": 93, "type": "sym", "entity": "血、尿常规和血生化改变" } ] }, { "text": "以发热为主症者,应与流感、钩端螺旋体病、败血症等相鉴别;以休克为主症者应与暴发型流脑、败血症休克、中毒型菌痢等区别;以出血为主症者应与伤寒肠出血,血小板减少性紫癜等相鉴别;以肾功能损害少尿为主症者,应与肾小球肾炎和其他原因引起的急性肾功能不全区别。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "发热" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "流感" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "钩端螺旋体病" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "败血症" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "休克" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "暴发型流脑" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "败血症休克" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "中毒型菌痢" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "出血" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "伤寒肠出血" }, { "start_idx": 73, "end_idx": 80, "type": "dis", "entity": "血小板减少性紫癜" }, { "start_idx": 87, "end_idx": 87, "type": "bod", "entity": "肾" }, { "start_idx": 93, "end_idx": 93, "type": "bod", "entity": "尿" }, { "start_idx": 87, "end_idx": 93, "type": "sym", "entity": "肾功能损害少尿" }, { "start_idx": 101, "end_idx": 105, "type": "dis", "entity": "肾小球肾炎" }, { "start_idx": 114, "end_idx": 120, "type": "dis", "entity": "急性肾功能不全" } ] }, { "text": "另外,小儿流行性出血热还应与登革出血热(Denguehemorrhagicfever)相鉴别:①登革出血热是由伊蚊传播登革病毒所致,在海南及广东流行。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "小儿流行性出血热" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "登革出血热" }, { "start_idx": 20, "end_idx": 41, "type": "dis", "entity": "Denguehemorrhagicfever" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "登革出血热" }, { "start_idx": 59, "end_idx": 62, "type": "mic", "entity": "登革病毒" } ] }, { "text": "②临床上以发热、多形性皮疹、多器官较大量出血为特征。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "发热" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "多形性皮疹" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "多器官较大量出血" } ] }, { "text": "病程中可有休克但无肾损害。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "休克" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "无肾损害" } ] }, { "text": "目前国内外已成功研制出3类疫苗,即纯化乳鼠脑灭活疫苗、细胞培养灭活疫苗和基因工程重组载体(活)疫苗。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dru", "entity": "疫苗" }, { "start_idx": 17, "end_idx": 25, "type": "dru", "entity": "纯化乳鼠脑灭活疫苗" }, { "start_idx": 27, "end_idx": 34, "type": "dru", "entity": "细胞培养灭活疫苗" }, { "start_idx": 36, "end_idx": 48, "type": "dru", "entity": "基因工程重组载体(活)疫苗" } ] }, { "text": "纯化乳鼠脑灭活疫苗Ⅲ期临床防病效果观察,保护率为96%。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dru", "entity": "纯化乳鼠脑灭活疫苗" } ] }, { "text": "两株细胞培养灭活疫苗,即金黄地鼠细胞(GHKC)和长爪沙鼠肾细胞(MGKC)灭活疫苗的Ⅲ期临床预防效果观察,前者保护率为97.63%;后者达95.27%。", "entities": [ { "start_idx": 12, "end_idx": 41, "type": "dru", "entity": "金黄地鼠细胞(GHKC)和长爪沙鼠肾细胞(MGKC)灭活疫苗" } ] }, { "text": "疫苗的应用将会大大降低我国EHF的发病率。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "EHF" } ] }, { "text": "【治疗】(一)综合疗法应做好“三早一就”(早发现、早休息、早治疗及就近治疗),以液体疗法为主,辅以对症治疗。", "entities": [ { "start_idx": 21, "end_idx": 36, "type": "pro", "entity": "早发现、早休息、早治疗及就近治疗" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "液体疗法" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "对症治疗" } ] }, { "text": "1.发热期治疗卧床休息,给以高热量、高维生素、易消化的食物。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "卧床休息" }, { "start_idx": 12, "end_idx": 28, "type": "pro", "entity": "给以高热量、高维生素、易消化的食物" } ] }, { "text": "高热以物理降温为主,体温过高、中毒症状重者给予地塞米松,每次0.2~0.4mg/kg,每4~6小时一次,疗程2~3天或热退即停。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "高热" }, { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "物理降温" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "体温过高" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "中毒" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "地塞米松" } ] }, { "text": "出血明显可用酚磺乙胺、云南白药或维生素K。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "云南白药" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "维生素K" } ] }, { "text": "呕吐不能进食者,给予静脉补液和止吐剂。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "呕吐" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "静脉补液" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "止吐剂" } ] }, { "text": "发生DIC时,可尽早试用肝素,低分子右旋糖酐及丹参注射液有抗凝作用,均可应用。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "DIC" }, { "start_idx": 12, "end_idx": 13, "type": "dru", "entity": "肝素" }, { "start_idx": 15, "end_idx": 21, "type": "dru", "entity": "低分子右旋糖酐" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "丹参注射液" } ] }, { "text": "2.低血压休克期治疗①补充血容量:按早期、快速、适量的原则,液体一般按3∶1的晶胶比例,先晶体后胶体,酌情补钾、钙。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "低血压休克" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "补充血容量" } ] }, { "text": "本期因血液浓缩,不宜用全血;②纠正酸中毒:1.4%~2.5%碳酸氢钠静脉注射或静脉滴注,至酸中毒纠正为止;③强心剂应用:在血容量基本补足情况下,心率仍快则可给予毛花苷丙或毒毛花苷K;④血管活性药物应用:血容量补足,血压仍不稳定者可选用血管活性药物,如多巴胺、间羟胺、异丙基肾上腺素或山莨菪碱等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血液" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "酸中毒" }, { "start_idx": 30, "end_idx": 42, "type": "pro", "entity": "碳酸氢钠静脉注射或静脉滴注" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "酸中毒" }, { "start_idx": 54, "end_idx": 56, "type": "dru", "entity": "强心剂" }, { "start_idx": 72, "end_idx": 73, "type": "ite", "entity": "心率" }, { "start_idx": 80, "end_idx": 83, "type": "dru", "entity": "毛花苷丙" }, { "start_idx": 85, "end_idx": 89, "type": "dru", "entity": "毒毛花苷K" }, { "start_idx": 92, "end_idx": 97, "type": "dru", "entity": "血管活性药物" }, { "start_idx": 101, "end_idx": 105, "type": "sym", "entity": "血容量补足" }, { "start_idx": 107, "end_idx": 108, "type": "ite", "entity": "血压" }, { "start_idx": 117, "end_idx": 122, "type": "dru", "entity": "血管活性药物" }, { "start_idx": 125, "end_idx": 127, "type": "dru", "entity": "多巴胺" }, { "start_idx": 129, "end_idx": 131, "type": "dru", "entity": "间羟胺" }, { "start_idx": 133, "end_idx": 139, "type": "dru", "entity": "异丙基肾上腺素" }, { "start_idx": 141, "end_idx": 144, "type": "dru", "entity": "山莨菪碱" } ] }, { "text": "3.少尿期治疗(包括移行阶段和多尿早期)本期主要稳定机体内环境,加强利尿,促进肾功能恢复。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "尿" }, { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "少尿" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "尿" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "多尿" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "加强利尿" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "肾" } ] }, { "text": "(1)稳定机体内环境:①控制氮质血症:给以高糖、高维生素、低蛋白饮食,维持热量;②严格限制入量:每日液体入量=[前一日尿量+每日不显性失水+吐泻丢失量]-内生水量;③维持电解质和酸碱平衡。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "氮质血症" }, { "start_idx": 20, "end_idx": 38, "type": "pro", "entity": "以高糖、高维生素、低蛋白饮食,维持热量" }, { "start_idx": 41, "end_idx": 46, "type": "pro", "entity": "严格限制入量" }, { "start_idx": 83, "end_idx": 92, "type": "pro", "entity": "维持电解质和酸碱平衡" } ] }, { "text": "(2)促进利尿:可用呋塞米等利尿剂和血管扩张剂如多巴胺等。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "利尿" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "呋塞米" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "利尿剂" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "血管扩张剂" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "多巴胺" } ] }, { "text": "(3)导泻和放血:有高血容量综合征时,可口服甘露醇粉剂、50%硫酸镁、中药大黄等导泻;出现心衰、肺水肿可考虑放血。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "导泻" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "放血" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "高血容量综合征" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "甘露醇粉剂" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "硫酸镁" }, { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "中药大黄" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "导泻" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "心衰" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "肺" }, { "start_idx": 48, "end_idx": 50, "type": "sym", "entity": "肺水肿" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "放血" } ] }, { "text": "(4)透析疗法:凡有明显氮质血症、高血钾、高血容量综合征者,可采用腹膜或血液透析治疗。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "透析" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "氮质血症" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "高血钾" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "高血容量综合征" }, { "start_idx": 33, "end_idx": 39, "type": "pro", "entity": "腹膜或血液透析" } ] }, { "text": "4.多尿期治疗主要保持水、电解质平衡,防止继发感染。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "尿" }, { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "多尿" }, { "start_idx": 9, "end_idx": 17, "type": "pro", "entity": "保持水、电解质平衡" } ] }, { "text": "随着尿量的增加适当补充液体和电解质,防止第二次肾衰竭,补液以口服为主。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "pro", "entity": "补充液体和电解质" }, { "start_idx": 23, "end_idx": 25, "type": "sym", "entity": "肾衰竭" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "口服" } ] }, { "text": "蛋白质宜逐步增加,以防止多尿性氮质血症。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "蛋白质宜逐步增加" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "多尿性氮质血症" } ] }, { "text": "5.恢复期治疗补充营养,逐步恢复活动。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "补充营养" }, { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "逐步恢复活动" } ] }, { "text": "6.其他对症治疗肾破裂时及时手术治疗;高血容量综合征、高血钾、心衰、肺水肿、呼吸衰竭、中枢神经并发症及腔道出血时及时采取有力抢救措施。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肾" }, { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "肾破裂" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "手术" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "高血容量综合征" }, { "start_idx": 27, "end_idx": 29, "type": "sym", "entity": "高血钾" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "心衰" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肺" }, { "start_idx": 34, "end_idx": 36, "type": "sym", "entity": "肺水肿" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "呼吸衰竭" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "中枢神经" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "中枢神经并发症" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "腔道" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "腔道出血" } ] }, { "text": "(二)抗病毒治疗1.利巴韦林(ribavirin)是我国学者首先找到的抗汉坦病毒药物,早期应用(病程头4天内)效佳。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "利巴韦林" }, { "start_idx": 15, "end_idx": 23, "type": "dru", "entity": "ribavirin" }, { "start_idx": 35, "end_idx": 41, "type": "dru", "entity": "抗汉坦病毒药物" } ] }, { "text": "有两种治疗方案:①大剂量疗法:首剂33mg/kg,以后16mg/kg,6小时一次,连续4天,第5~7天8mg/kg,8小时一次,静脉滴注,可将病死率降至<2%。", "entities": [ { "start_idx": 64, "end_idx": 67, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "副作用有可逆性骨髓抑制和红细胞减少。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "骨髓" }, { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "可逆性骨髓抑制" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "红细胞" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "红细胞减少" } ] }, { "text": "②小剂量疗法:按10~15mg/(kg•d)或700~750mg/d静脉滴注,疗程3天,可改善症状,降低病死率,无不良反应。", "entities": [ { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "但患者体内病毒是否被清除尚未证实。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "病毒" } ] }, { "text": "此外,利巴韦林治疗汉坦病毒肺综合征(HPS)无明显疗效,可能与该病进展快速有关。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "利巴韦林" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "汉坦病毒肺综合征" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "HPS" } ] }, { "text": "2.干扰素体外有抗病毒作用,体内疗效尚在评价中。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "干扰素" }, { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "病毒" } ] }, { "text": "第二节面肩肱型肌营养不良面肩肱型肌营养不良(facioscapulohumeraldystrophy,FSHD)是常染色体显性遗传病,发病率约为1∶20000。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "面肩肱型肌营养不良" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "面肩肱型肌营养不良" }, { "start_idx": 22, "end_idx": 49, "type": "dis", "entity": "facioscapulohumeraldystrophy" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "FSHD" }, { "start_idx": 57, "end_idx": 65, "type": "dis", "entity": "常染色体显性遗传病" } ] }, { "text": "【发病机制】Wijmenga等首先证实,FSHD基因定位于4号染色体上,进一步的研究还表明定位于4q35。", "entities": [ { "start_idx": 20, "end_idx": 25, "type": "bod", "entity": "FSHD基因" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "4号染色体" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "4q35" } ] }, { "text": "1992年,研究发现经EcoR1酶切后的片段中,用特异性探针(p13E-11)可检测到一个比正常人群短的DNA片段。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "EcoR1酶" }, { "start_idx": 25, "end_idx": 29, "type": "equ", "entity": "特异性探针" }, { "start_idx": 31, "end_idx": 37, "type": "equ", "entity": "p13E-11" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "DNA" } ] }, { "text": "约85%~95%临床诊断为FSHD患者(无论是家族性还是散发性),都证实有短片段存在。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "FSHD" } ] }, { "text": "许多研究表明,4q35区的缺失越大(形成的短片段越小),临床表现越严重。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "4q35" } ] }, { "text": "虽然4q35短片段与FSHD的关系已明确,但精确的基因定位或这种缺陷影响的基因还未明确,缺失与疾病的确切关系至今未明。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "4q35" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "FSHD" } ] }, { "text": "位置变异效应假说认为,此区域的串联重复序列缺失可能影响邻近基因(包括FSHD基因)的表达。", "entities": [ { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "FSHD" } ] }, { "text": "一些FSHD患者在缺失位点邻近区域有重组现象,支持了以上假说。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "FSHD" } ] }, { "text": "因此目前研究已转向确立4号染色体上邻近FSHD区域的基因及其特性。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "4号染色体" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "FSHD" } ] }, { "text": "另外,一些FSHD的家族患者与4号染色体无关,这说明其具有遗传异质性。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "FSHD" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "4号染色体" } ] }, { "text": "【临床表现】本病患者肌无力主要累及面肌及肩胛肌群。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肌无力" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "面肌" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "肩胛肌群" } ] }, { "text": "面肌主要累及眼轮匝肌和口轮匝肌,常为非对称性,患者出现奇怪的扭曲笑容,口角处出现凹陷无法撅嘴面部表情抑郁、平淡当要求患者吹口哨时,嘴唇常特征性地呈横向或水平位患者不能完全闭紧眼睛眼睑很容易睁开肩胛带肌肉包括背阔肌、斜方肌、菱形肌以及前锯肌。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "面肌" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "眼轮匝肌" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "口轮匝肌" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "扭曲笑容" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "口角" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "口角处出现凹陷" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "嘴" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "无法撅嘴" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "面部" }, { "start_idx": 46, "end_idx": 54, "type": "sym", "entity": "面部表情抑郁、平淡" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "嘴唇" }, { "start_idx": 55, "end_idx": 78, "type": "sym", "entity": "当要求患者吹口哨时,嘴唇常特征性地呈横向或水平位" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "眼睛" }, { "start_idx": 79, "end_idx": 88, "type": "sym", "entity": "患者不能完全闭紧眼睛" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "眼睑" }, { "start_idx": 89, "end_idx": 95, "type": "sym", "entity": "眼睑很容易睁开" }, { "start_idx": 96, "end_idx": 100, "type": "bod", "entity": "肩胛带肌肉" }, { "start_idx": 103, "end_idx": 105, "type": "bod", "entity": "背阔肌" }, { "start_idx": 107, "end_idx": 109, "type": "bod", "entity": "斜方肌" }, { "start_idx": 111, "end_idx": 113, "type": "bod", "entity": "菱形肌" }, { "start_idx": 116, "end_idx": 118, "type": "bod", "entity": "前锯肌" } ] }, { "text": "静止时,患者表现为斜肩姿势肩膀前转肩胛骨上升前臂上移或外展时,肩胛骨常常向前外向转动活动障碍,尤其是那些需应用肩胛肌肉的活动,如爬树、挥动高尔夫球棒以及投掷垒球等。", "entities": [ { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肩" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "斜肩姿势" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肩" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "膀" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "肩膀前转" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肩胛骨" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "肩胛骨上升" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "肩胛骨" }, { "start_idx": 22, "end_idx": 41, "type": "sym", "entity": "前臂上移或外展时,肩胛骨常常向前外向转动" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "活动障碍" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "肩胛肌肉" } ] }, { "text": "约有1/3的患者无症状,仅能依据体格检查进行诊断。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "体格检查" } ] }, { "text": "腹壁肌肉在疾病早期即可受累,但往往到疾病晚期才引起注意。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "腹壁肌肉" } ] }, { "text": "典型的表现是腹壁下部肌肉严重受累引起腹部突出Beevor征(患者在仰卧时屈曲颈部脐部可向上偶尔也会向下移动),它可作为可疑患者的一个早期表现,因为其他肌肉性疾病很少出现这种表现。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "腹壁" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肌肉" }, { "start_idx": 6, "end_idx": 15, "type": "sym", "entity": "腹壁下部肌肉严重受累" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "腹部" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "引起腹部突出" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "Beevor征" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "颈部" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "在仰卧时屈曲颈部" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "脐部" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "脐部可向上" }, { "start_idx": 45, "end_idx": 52, "type": "sym", "entity": "偶尔也会向下移动" }, { "start_idx": 75, "end_idx": 79, "type": "dis", "entity": "肌肉性疾病" } ] }, { "text": "大腿远端前群肌常常受累最早、最严重行走时足部拖地明显的足下垂导致频繁的摔倒或者行走不稳。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "大腿" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "前群肌" }, { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "大腿远端前群肌常常受累最早、最严重" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "足部" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "行走时足部拖地" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "足" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "明显的足下垂" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "导致频繁的摔倒" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "行走不稳" } ] }, { "text": "少数情况下,患者可出现较严重的骨盆肢带肌无力髋部屈肌和外展肌,造成早期相对较重的步态不稳。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "骨盆肢带肌" }, { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "严重的骨盆肢带肌无力" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "髋部屈肌" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "外展肌" } ] }, { "text": "患者延髓肌、咽肌、眼外肌和呼吸肌一般不受累,吞咽功能不受影响没有明显肌肉外受累表现高频性耳聋以及视网膜血管异常在FSHD人群中的发病率正逐渐上升,但常无临床意义。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "延髓肌" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "咽肌" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "眼外肌" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "吞咽功能" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "吞咽功能不受影响" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肌肉" }, { "start_idx": 30, "end_idx": 40, "type": "sym", "entity": "没有明显肌肉外受累表现" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "高频性耳聋" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "视网膜血管异常" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "FSHD" } ] }, { "text": "许多研究发现FSHD患者有发生房性心律紊乱实验室检查主要实验室指标如下:1.血清CK约75%患者血清CK升高,但常常为中度升高。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "FSHD" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "心律" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "房性心律紊乱" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "实验室检查" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "血清CK" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "血清CK" } ] }, { "text": "2.肌电图大多数患者临床受累的肌肉出现明显的多相低振幅短时相的动作单位电位肌肉活体组织检查肌肉活体组织检查对于疑似FSHD,尤其是家族史不确切的患者至关重要。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肌电图" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肌肉" }, { "start_idx": 15, "end_idx": 36, "type": "sym", "entity": "肌肉出现明显的多相低振幅短时相的动作单位电位" }, { "start_idx": 37, "end_idx": 44, "type": "pro", "entity": "肌肉活体组织检查" }, { "start_idx": 45, "end_idx": 52, "type": "pro", "entity": "肌肉活体组织检查" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "FSHD" } ] }, { "text": "常显示不同程度的改变,包括纤维直径的不同出现角形纤维中央核纤维坏死纤维再生纤维肥大纤维单核炎症细胞浸润明显的脂肪浸润和结缔组织增殖基因诊断基因检测不失为一种有用的诊断手段。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "纤维" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "纤维直径的不同" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "角形纤维" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "出现角形纤维" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "核纤维" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "中央核纤维" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "纤维" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "坏死纤维" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "纤维" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "再生纤维" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "纤维" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "肥大纤维" }, { "start_idx": 43, "end_idx": 48, "type": "bod", "entity": "单核炎症细胞" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "单核炎症细胞浸润" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "脂肪浸润" }, { "start_idx": 59, "end_idx": 64, "type": "dis", "entity": "结缔组织增殖" }, { "start_idx": 51, "end_idx": 64, "type": "sym", "entity": "明显的脂肪浸润和结缔组织增殖" }, { "start_idx": 65, "end_idx": 68, "type": "pro", "entity": "基因诊断" }, { "start_idx": 69, "end_idx": 72, "type": "pro", "entity": "基因检测" } ] }, { "text": "FSHD的基因定位于4q35,4q35基因缺失具有较高的敏感性和特异性,通过检测可疑患者4q35短片段,基本可以作出诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "FSHD" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "4q35" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "4q35" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "4q35" } ] }, { "text": "【治疗】目前尚无特殊的治疗,支持治疗是主要治疗手段。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "支持治疗" } ] }, { "text": "由于受累的肌肉不同,患者容易出现肩部、背部、腹部及腿部疼痛保守治疗包括非甾体类抗炎药、适当的运动锻炼以及理疗可以缓解一些不适主诉。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肌肉" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肩部" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "背部" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "腹部" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "腿部" }, { "start_idx": 16, "end_idx": 28, "type": "sym", "entity": "肩部、背部、腹部及腿部疼痛" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "保守治疗" }, { "start_idx": 35, "end_idx": 41, "type": "dru", "entity": "非甾体类抗炎药" }, { "start_idx": 52, "end_idx": 53, "type": "dru", "entity": "理疗" } ] }, { "text": "定做的塑料踝-足矫正器,可以减轻足下垂伴有明显腹部或者下背部疼痛腰背紧身胸衣或腹带,为无力的腹部肌肉群提供支撑。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "equ", "entity": "塑料踝-足矫正器" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "足" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "足下垂" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "腹部" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "背部" }, { "start_idx": 19, "end_idx": 31, "type": "sym", "entity": "伴有明显腹部或者下背部疼痛" }, { "start_idx": 32, "end_idx": 37, "type": "equ", "entity": "腰背紧身胸衣" }, { "start_idx": 39, "end_idx": 40, "type": "equ", "entity": "腹带" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "腹部" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "肌肉" } ] }, { "text": "目前尚无药物可改善FSHD患者的肌力或延缓疾病的进展。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "FSHD" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "肌力" } ] }, { "text": "曾有学者使用泼尼松治疗8例FSHD患者,但结果显示并无多大益处。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "泼尼松治疗" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "FSHD" } ] }, { "text": "【预后】肌无力发展呈下降型,首先累及面部肌肉然后下移至肩胛肌及肱肌最后影响骨盆肢带肌,对患者的寿命无明显影响。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "肌无力" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "发展呈下降型" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "面部" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肌肉" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "首先累及面部肌肉" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肩胛肌" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肱肌" }, { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "然后下移至肩胛肌及肱肌" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "最后影响骨盆肢带肌" } ] }, { "text": "二、哮喘的诱因(一)呼吸道感染1.呼吸道病毒感染在婴幼儿期主要有呼吸道合胞病毒(RSV),其次为副流感病毒、流感病毒和腺病毒,其他如麻疹病毒、腮腺炎病毒、肠道病毒、脊髓灰质炎病毒偶尔可见。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "哮喘" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "呼吸道病毒感染" }, { "start_idx": 32, "end_idx": 38, "type": "mic", "entity": "呼吸道合胞病毒" }, { "start_idx": 40, "end_idx": 42, "type": "mic", "entity": "RSV" }, { "start_idx": 48, "end_idx": 52, "type": "mic", "entity": "副流感病毒" }, { "start_idx": 54, "end_idx": 57, "type": "mic", "entity": "流感病毒" }, { "start_idx": 66, "end_idx": 69, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 71, "end_idx": 75, "type": "mic", "entity": "腮腺炎病毒" }, { "start_idx": 77, "end_idx": 80, "type": "mic", "entity": "肠道病毒" }, { "start_idx": 82, "end_idx": 88, "type": "mic", "entity": "脊髓灰质炎病毒" } ] }, { "text": "年长儿多见鼻病毒感染。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "鼻病毒感染" } ] }, { "text": "2.支原体感染由于婴幼儿免疫系统不成熟,支原体可以引起婴幼儿呼吸道慢性感染,若处理不恰当,可以导致反复不愈的咳嗽和喘息。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "支原体感染" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "免疫系统" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "支原体" }, { "start_idx": 27, "end_idx": 36, "type": "dis", "entity": "婴幼儿呼吸道慢性感染" }, { "start_idx": 49, "end_idx": 58, "type": "sym", "entity": "反复不愈的咳嗽和喘息" } ] }, { "text": "3.呼吸道局灶性感染慢性鼻窦炎、鼻炎、中耳炎、慢性扁桃体炎,是常见的儿童上呼吸道慢性局灶性病变,一方面可以引起反复的感染,另一方面又可以通过神经反射引起反复的咳喘,需要对这些病灶进行及时处理。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "呼吸道局灶性感染" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "慢性鼻窦炎" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "鼻炎" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "中耳炎" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "慢性扁桃体炎" }, { "start_idx": 34, "end_idx": 46, "type": "dis", "entity": "儿童上呼吸道慢性局灶性病变" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "感染" }, { "start_idx": 79, "end_idx": 80, "type": "dis", "entity": "咳喘" } ] }, { "text": "(二)吸入过敏物质持续低浓度变应原吸入可以诱发慢性气道变应性炎症,促进气道高反应形成,但短时间吸入高浓度变应原可以诱发急性哮喘发作。", "entities": [ { "start_idx": 23, "end_idx": 31, "type": "dis", "entity": "慢性气道变应性炎症" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "气道" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "急性哮喘" } ] }, { "text": "这类诱因诱发的哮喘发作较为突然,无上呼吸道感染症状,多数在环境中过敏原浓度较高的季节发作。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "哮喘" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "无上呼吸道感染症状" } ] }, { "text": "(三)胃食管反流由于解剖结构的原因,也有医源性因素(如应用氨茶碱、β受体兴奋药等)可以引起胃食管反流,在婴幼儿尤为多见,它是导致喘息反复不愈的重要原因之一。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 33, "end_idx": 38, "type": "dru", "entity": "β受体兴奋药" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "喘息" } ] }, { "text": "临床上多表现为入睡中出现剧烈的咳嗽、喘息,平时有回奶或呕吐现象。", "entities": [ { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "入睡中出现剧烈的咳嗽" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "喘息" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "回奶" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "呕吐" } ] }, { "text": "(四)其他吸入刺激性气体或剧烈运动、哭闹,以及油漆、煤烟、冷空气吸入均可作为非特异性刺激物诱发哮喘发作,其中油漆散发的气体可触发严重而持续的咳喘发作,应尽量避免。", "entities": [ { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "哮喘" }, { "start_idx": 70, "end_idx": 71, "type": "dis", "entity": "咳喘" } ] }, { "text": "剧烈运动、哭闹使呼吸运动加快,呼吸道温度降低或呼吸道内液体渗透压改变,而诱发哮喘发作。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "呼吸运动加快" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "哮喘" } ] }, { "text": "三、抗胆碱能药物正常人的气道有静态张力,主要受胆碱能神经(迷走神经)的控制。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "抗胆碱能药物" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "气道" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "胆碱能神经" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "迷走神经" } ] }, { "text": "哮喘患儿可能由于气道上皮破坏,神经末梢暴露或其他原因,使胆碱能神经的张力及反射加强,是引起气道高反应的重要因素之一;临床常用的抗胆碱能药物主要为阿托品和溴化异丙托品。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "哮喘" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "气道上皮" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "神经末梢" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "胆碱能神经" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "气道" }, { "start_idx": 63, "end_idx": 68, "type": "dru", "entity": "抗胆碱能药物" }, { "start_idx": 72, "end_idx": 74, "type": "dru", "entity": "阿托品" }, { "start_idx": 76, "end_idx": 81, "type": "dru", "entity": "溴化异丙托品" } ] }, { "text": "阿托品为叔胺类复合物,脂溶性强,容易从黏膜吸收,可以口服给药;但由于其体内分布广泛,系统性不良反应较多;而且可以进入血脑屏障,引起疲劳、烦躁、头痛症状,目前已很少用于哮喘的治疗。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "阿托品" }, { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "叔胺类复合物" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "黏膜" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "口服给药" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "血脑屏障" }, { "start_idx": 65, "end_idx": 72, "type": "sym", "entity": "疲劳、烦躁、头痛" }, { "start_idx": 83, "end_idx": 84, "type": "dis", "entity": "哮喘" } ] }, { "text": "溴化异丙托品为季胺类化合物,水溶性强,不容易吸收,全身不良反应小,但局部作用强;而且对支气管平滑肌M受体的选择性强,对分泌腺的影响小,不易出现气道分泌物干结现象。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "溴化异丙托品" }, { "start_idx": 7, "end_idx": 12, "type": "dru", "entity": "季胺类化合物" }, { "start_idx": 43, "end_idx": 51, "type": "bod", "entity": "支气管平滑肌M受体" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "分泌腺" }, { "start_idx": 71, "end_idx": 75, "type": "bod", "entity": "气道分泌物" }, { "start_idx": 67, "end_idx": 79, "type": "sym", "entity": "不易出现气道分泌物干结现象" } ] }, { "text": "由于胆碱能神经在气道的分布不均匀,在大气道分布多,而在小气道分布少,因此应用抗胆碱药后,主要使大气道松弛;当大气道收缩明显时,才有一定的松弛作用。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "胆碱能神经" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "气道" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "大气道" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "小气道" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "抗胆碱药" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "大气道" } ] }, { "text": "β肾上腺素受体激动药则对大、小气道均有明显的松弛作用,这与抗胆碱药有所不同。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dru", "entity": "β肾上腺素受体激动药" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "大、小气道" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "抗胆碱药" } ] }, { "text": "一般在哮喘治疗中抗胆碱药与β2受体激动剂合用而起到相互协同作用。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "哮喘" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "抗胆碱药" }, { "start_idx": 13, "end_idx": 30, "type": "dru", "entity": "β2受体激动剂" } ] }, { "text": "现有剂型为:①爱全乐pMDI:20μg/揿,1~2揿/次,3~4次/天;②爱全乐溶液:0.025%溶液,<2岁0.5ml,>2岁1ml,<4次/天,喷射雾化吸入。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dru", "entity": "爱全乐pMDI" }, { "start_idx": 37, "end_idx": 41, "type": "dru", "entity": "爱全乐溶液" }, { "start_idx": 74, "end_idx": 77, "type": "pro", "entity": "喷射雾化" } ] }, { "text": "第八节新生儿呕吐呕吐是新生儿期常见症状,是一系列复杂的神经反射活动。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "新生儿呕吐" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "呕吐" } ] }, { "text": "新生儿胃容量小、胃呈横位、贲门括约肌发育不完善、幽门括约肌发育较好、肠道蠕动的神经调节功能较差,由于这些解剖生理特点,新生儿容易发生呕吐。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "胃" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "胃" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "贲门括约肌" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "幽门括约肌" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肠道" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "呕吐" } ] }, { "text": "【病因】(一)消化系统疾病各种消化系统疾病都可引起呕吐,主要有消化道先天畸形、梗阻、炎症、感染、出血、功能失调等。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "消化系统疾病" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "消化系统疾病" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "呕吐" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "消化道先天畸形" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "梗阻" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "炎症" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "感染" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "出血" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "功能失调" } ] }, { "text": "1.消化系统功能紊乱如吞咽功能不协调、胃食管反流、贲门失弛缓症、幽门痉挛、胎粪性便秘、胎粪排出延迟等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "消化系统" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "吞咽功能不协调" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "贲门失弛缓症" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "幽门痉挛" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "胎粪性便秘" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "胎粪排出延迟" } ] }, { "text": "2.消化道黏膜受刺激如咽下综合征、胃出血、应激性溃疡、牛奶过敏等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "消化道黏膜" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "咽下综合征" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "胃出血" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "应激性溃疡" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "牛奶过敏" } ] }, { "text": "3.消化系统感染及炎症如急性胃炎、急性肠炎、坏死性小肠结肠炎、腹膜炎等。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "消化系统感染及炎症" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "急性胃炎" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "急性肠炎" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "坏死性小肠结肠炎" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "4.消化道梗阻多数为先天畸形所致。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "消化道梗阻" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "先天畸形" } ] }, { "text": "少见疾病有嵌顿疝、肠套叠等。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "嵌顿疝" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "肠套叠" } ] }, { "text": "(二)全身性疾病许多全身性疾病可引起呕吐,常见的有以下几方面:1.感染新生儿感染常引起呕吐,如败血症、呼吸道感染、泌尿系统感染等。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "呕吐" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "感染" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "新生儿感染" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "呕吐" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "败血症" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "泌尿系统感染" } ] }, { "text": "2.颅内压增高引起颅内压增高的疾病多会导致呕吐,如中枢神经系统感染、脑水肿、脑积水、颅内出血、颅内肿瘤等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "颅内压增高" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "呕吐" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "中枢神经系统感染" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "脑水肿" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "脑积水" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "颅内出血" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "颅内肿瘤" } ] }, { "text": "3.先天性代谢性疾病一些先天性代谢性疾病由于代谢紊乱而导致呕吐,如氨基酸代谢疾病(高氨血症、苯丙酮尿症、甘氨酸血症)、糖代谢疾病(半乳糖血症、枫糖尿症)、肾上腺皮质增生症等。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "先天性代谢性疾病" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "先天性代谢性疾病" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "呕吐" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "氨基酸代谢疾病" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "高氨血症" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "甘氨酸血症" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "糖代谢疾病" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "枫糖尿症" }, { "start_idx": 77, "end_idx": 84, "type": "dis", "entity": "肾上腺皮质增生症" } ] }, { "text": "(三)其他因素一些疾病因素也可引起新生儿呕吐。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "呕吐" } ] }, { "text": "2.药物许多药物可引起消化道反应,发生呕吐,如红霉素、两性霉素B等。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "消化道" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "呕吐" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "红霉素" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "两性霉素B" } ] }, { "text": "【临床特点】(一)溢乳和喂养不当1.溢乳新生儿溢乳比较常见,但溢乳没有神经反射参与,不属于真正的呕吐。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "溢乳" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "新生儿溢乳" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "溢乳" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "神经" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "呕吐" } ] }, { "text": "溢乳的原因与食管弹力组织和肌肉发育不完善有关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "溢乳" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "食管弹力组织" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肌肉" } ] }, { "text": "溢乳多发生在喂奶后不久,乳汁从口角边溢出,喂奶后体位改变可引起溢乳。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "溢乳" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "乳汁" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "溢乳" } ] }, { "text": "2.喂养不当新生儿喂养不当非常多见,主要原因有:喂奶次数过于频繁,喂奶量太多,浓度不适合,牛乳太热或太凉,配方乳多变;奶嘴孔过大或过小,乳母乳头下陷;喂奶后平卧,体位多动。", "entities": [ { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "乳母乳头" } ] }, { "text": "(二)与内科疾病有关的呕吐1.吞咽功能不协调喂奶时即呕吐,常伴有呛咳或吸入,一部分乳汁从鼻孔流出。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "呕吐" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "呕吐" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "鼻孔" } ] }, { "text": "2.胃食管反流(GER)是新生儿呕吐的常见原因,尤其是早产儿。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "新生儿呕吐" } ] }, { "text": "主要与新生儿食管下端括约肌较松弛、胃排空延迟、腹内压增高等因素有关。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "食管下端括约肌" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "胃" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "腹内压" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "腹内压增高" } ] }, { "text": "常在喂奶后不久出现呕吐或表现为溢乳,呕吐物常为不带胆汁的奶液。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "呕吐" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "溢乳" } ] }, { "text": "3.胃黏膜受刺激出生时咽下羊水或产道血液,刺激胃黏膜引起呕吐。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "羊水" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "血液" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "呕吐" } ] }, { "text": "未开奶前即可出现呕吐,开奶后呕吐加重,呕吐物为泡沫样黏液或带血性,用生理盐水洗胃1~2次,呕吐即可停止。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "呕吐" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "呕吐" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "生理盐水" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "洗胃" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "呕吐" } ] }, { "text": "4.幽门痉挛为幽门神经肌肉功能暂时性失调所致,解剖结构无异常。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "幽门痉挛" }, { "start_idx": 7, "end_idx": 12, "type": "bod", "entity": "幽门神经肌肉" } ] }, { "text": "呕吐常发生在生后2~3周,呈间隙性,可为喷射状,呕吐物不含胆汁,与幽门肥厚性狭窄较难鉴别,试用1∶1000阿托品可缓解。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "呕吐" }, { "start_idx": 47, "end_idx": 55, "type": "dru", "entity": "1∶1000阿托品" } ] }, { "text": "5.胎粪延迟排出正常新生儿在生后24小时内开始排胎粪,3天排完。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "胎粪延迟排出" } ] }, { "text": "6.感染性疾病肠道内感染或肠道外感染均可引起新生儿呕吐,常伴有感染表现如神萎、食欲缺乏,肠道内感染伴有腹泻、腹胀。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "肠道内感染" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "肠道外感染" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "新生儿呕吐" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "神萎" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "肠道内感染" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "腹泻" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "腹胀" } ] }, { "text": "7.先天性代谢性疾病发生呕吐时间无规律性,一般呕吐较频繁和剧烈,常伴有其他代谢病的临床表现,如酸中毒、电解质紊乱、脱水、肝脾大等。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "先天性代谢性疾病" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "呕吐" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "呕吐" }, { "start_idx": 47, "end_idx": 49, "type": "sym", "entity": "酸中毒" }, { "start_idx": 51, "end_idx": 55, "type": "sym", "entity": "电解质紊乱" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "脱水" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "肝脾" }, { "start_idx": 60, "end_idx": 62, "type": "sym", "entity": "肝脾大" } ] }, { "text": "(三)与外科疾病有关的呕吐1.食管闭锁和食管气管瘘食管闭锁者第一次喂奶(或喂水)时即发生呕吐,伴食管气管瘘者喂奶时出现呼吸困难、青紫,肺部闻湿啰音,每次喂奶时均出现类似情况。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dep", "entity": "外科" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "呕吐" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "食管闭锁" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "食管气管瘘" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "食管闭锁" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "呕吐" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "食管气管瘘者" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "青紫" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "肺部" }, { "start_idx": 67, "end_idx": 72, "type": "sym", "entity": "肺部闻湿啰音" } ] }, { "text": "2.幽门肥厚性狭窄常于生后第2周左右开始出现呕吐,呕吐量多,呕吐物为乳汁或乳凝块,酸臭味,无胆汁。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "幽门肥厚性狭窄" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "呕吐" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "呕吐" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "胆汁" } ] }, { "text": "呕吐常呈进行性加重,伴脱水、电解质紊乱、营养不良。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "呕吐" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "脱水" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "电解质紊乱" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "营养不良" } ] }, { "text": "腹部可见明显的胃型,右上腹可触及枣核大小的肿块。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "腹部" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "右上腹" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "肿块" } ] }, { "text": "3.十二指肠和小肠疾病患儿常有严重呕吐,呕吐物有绿色胆汁,位置较高者生后不久即呕吐,腹胀不明显,位置较低者呕吐出现晚一些,呕吐物为棕色粪便样物质,混有深色胆汁,腹胀明显,肠鸣音活跃,可见肠型、肠蠕动波。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "十二指肠" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "小肠" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "呕吐" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "呕吐" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "腹胀" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "呕吐" }, { "start_idx": 80, "end_idx": 83, "type": "sym", "entity": "腹胀明显" } ] }, { "text": "4.直肠肛门疾病一般先有腹胀,后出现呕吐,肠鸣音活跃,腹部平片显示肠腔扩张,多个液平。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "直肠肛门" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "腹胀" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "呕吐" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "肠鸣音活跃" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "腹部" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "肠腔扩张" } ] }, { "text": "先天性巨结肠患儿生后便秘,灌肠后腹胀减轻。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "先天性巨结肠" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "灌肠" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "腹胀" } ] }, { "text": "(四)呕吐所致的并发症新生儿呕吐时常发生一些并发症,需密切注意。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "呕吐" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "呕吐" } ] }, { "text": "1.窒息与猝死新生儿呕吐会使呕吐物进入呼吸道,发生窒息,如呕吐物多、没有及时发现可导致猝死。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "窒息" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "猝死" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "呕吐" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "呼吸道" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "窒息" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "猝死" } ] }, { "text": "2.吸入综合征呕吐物进入气道可发生吸入性肺炎,出现咳嗽、呼吸困难,长时间反复吸入可使吸入性肺炎迁延不愈。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "吸入综合征" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "气道" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "吸入性肺炎" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "咳嗽" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "吸入性肺炎" } ] }, { "text": "3.呼吸暂停早产儿呕吐可发生呼吸暂停。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "呼吸暂停" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "呕吐" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "呼吸暂停" } ] }, { "text": "4.出血剧烈呕吐可导致胃黏膜损伤,发生出血,呕吐物呈血性。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "出血" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "呕吐" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "出血" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "呕吐物呈血性" } ] }, { "text": "5.水电解质紊乱呕吐较频繁者,因丧失大量水分和电解质,导致水电解质平衡紊乱,患儿出现脱水、酸中毒、低钠血症等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "水电解质紊乱" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "呕吐" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "脱水" }, { "start_idx": 45, "end_idx": 47, "type": "sym", "entity": "酸中毒" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "低钠血症" } ] }, { "text": "【诊断与鉴别诊断】要详细询问病史,了解分娩时情况、发生呕吐的时间、呕吐特点、伴随症状等,仔细体格检查,初步考虑呕吐的定位和性质,并做进一步的检查,以明确诊断。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "呕吐" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "呕吐" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "呕吐" } ] }, { "text": "(一)定位根据呕吐发生的时间、呕吐特点、呕吐物、是否有腹胀、肠型、便秘等情况,初步判断消化道疾病的位置。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "呕吐" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "呕吐" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "腹胀" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "肠型" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "便秘" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "消化道疾病" } ] }, { "text": "1.上消化道呕吐出现时间早,呕吐物为乳汁或乳凝块,不含胆汁,腹胀不明显。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "上消化道" } ] }, { "text": "2.下消化道生后1~2天即呕吐,呕吐物含较多胆汁,腹胀不明显,提示病变在十二指肠或空肠上段。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "下消化道" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "呕吐" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "腹胀不明显" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "十二指肠" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "空肠上段" } ] }, { "text": "如呕吐物含黄绿色粪便样物质,腹部有较细的肠型和肠蠕动,提示病变在空肠下段或回肠。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "腹部" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "空肠下段" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "回肠" } ] }, { "text": "而直肠病变的呕吐常发生在出生3天以后,呕吐物含棕色粪便样物质,腹胀明显,肠型较粗大,可触及粪块。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "直肠" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "呕吐" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "腹胀明显" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肠型" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "肠型较粗大" } ] }, { "text": "(二)定性为使呕吐原发病得到及时治疗,要鉴别是内科疾病还是外科疾病所致。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "dep", "entity": "内科" }, { "start_idx": 29, "end_idx": 30, "type": "dep", "entity": "外科" } ] }, { "text": "1.内科疾病呕吐症状不剧烈,呕吐次数不频繁,呕吐物常不含胆汁或粪便,有较明显的消化系统以外的症状和体征,常提示呕吐为内科疾病所致。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dep", "entity": "内科" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "呕吐" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "呕吐" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "消化系统" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "呕吐" }, { "start_idx": 58, "end_idx": 59, "type": "dep", "entity": "内科" } ] }, { "text": "2.外科疾病呕吐出现早,频繁,较剧烈,呕吐物含胆汁、血液或粪便,伴脱水和电解质紊乱,常提示呕吐为外科疾病所致。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dep", "entity": "外科" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "呕吐" }, { "start_idx": 10, "end_idx": 30, "type": "sym", "entity": "早,频繁,较剧烈,呕吐物含胆汁、血液或粪便" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "脱水" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "电解质紊乱" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "呕吐" }, { "start_idx": 48, "end_idx": 49, "type": "dep", "entity": "外科" } ] }, { "text": "(三)进一步检查对呕吐原发病的位置和性质有初步判断后,应及时做进一步的检查,以明确诊断。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "呕吐" } ] }, { "text": "1.消化道影像学检查对消化道先天畸形的诊断有很大的帮助。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "消化道影像学检查" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "消化道先天畸形" } ] }, { "text": "对胃食管反流,可做放射性核素检查。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "放射性核素检查" } ] }, { "text": "对胃十二指肠、小肠部位的先天畸形,钡餐造影可帮助诊断,须注意检查结束时应洗胃,将胃内钡剂洗出,防止呕吐时钡剂。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "胃十二指肠" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "小肠" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "先天畸形" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "钡餐造影" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "洗胃" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "胃" }, { "start_idx": 42, "end_idx": 43, "type": "dru", "entity": "钡剂" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "呕吐" }, { "start_idx": 52, "end_idx": 53, "type": "dru", "entity": "钡剂" } ] }, { "text": "2.中枢神经系统检查如怀疑中枢感染,应查脑脊液,对颅内出血或其他占位病变,应做头颅B超或CT检查。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "中枢神经系统检查" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "脑脊液" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "颅内出血" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "头颅B超" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "CT检查" } ] }, { "text": "3.血气分析及生化检查可了解患儿是否存在酸中毒、电解质紊乱。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "血气分析" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "生化检查" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "酸中毒" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "电解质紊乱" } ] }, { "text": "4.内分泌及遗传代谢病检查如已排除消化道、中枢神经等疾病,而患儿仍然频繁呕吐,应进一步做内分泌、代谢病方面检查,如血氨、血糖等。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "内分泌及遗传代谢病检查" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "消化道" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "中枢神经" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "呕吐" }, { "start_idx": 57, "end_idx": 58, "type": "ite", "entity": "血氨" }, { "start_idx": 60, "end_idx": 61, "type": "ite", "entity": "血糖" } ] }, { "text": "【治疗】(一)对症治疗1.禁食对一些病因未清楚、怀疑外科疾病、消化道出血,可先行禁食,以免加重病情,同时给予补液,保证营养供给。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "禁食" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "消化道出血" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "禁食" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "补液" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "营养供给" } ] }, { "text": "2.洗胃对咽下综合征可先洗胃,用温生理盐水,一般洗2~3次即可,如洗胃后仍呕吐,应考虑其他疾病。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "洗胃" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "咽下综合征" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "洗胃" }, { "start_idx": 16, "end_idx": 20, "type": "dru", "entity": "温生理盐水" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "洗胃" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "呕吐" } ] }, { "text": "3.胃肠减压对外科疾病、呕吐较频繁、腹胀者,可先行胃肠减压,缓解症状,同时做有关检查。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "胃肠减压" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "呕吐" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "腹胀" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "胃肠减压" } ] }, { "text": "4.解痉止吐对病因诊断为胃食管反流,可用胃动力制剂或解痉剂。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "解痉止吐" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "胃动力制剂" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "解痉剂" } ] }, { "text": "5.体位对呕吐患儿,应提高头部和上身的体位,一般30°左右。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "呕吐" } ] }, { "text": "6.纠正水、电解质紊乱呕吐导致水、电解质紊乱,应及时纠正。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "纠正水" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "呕吐" } ] }, { "text": "(二)病因治疗1.手术对外科疾病需手术治疗,手术时机根据病情而定。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "手术" }, { "start_idx": 12, "end_idx": 13, "type": "dep", "entity": "外科" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "手术治疗" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "手术" } ] }, { "text": "2.抗感染对消化道感染或其他部位感染所致者,应给抗生素治疗。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "抗感染" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "消化道感染" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "抗生素" } ] }, { "text": "3.止血消化道出血者,可用维生素K1、酚磺乙胺等止血。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "止血" }, { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "消化道出血" }, { "start_idx": 13, "end_idx": 28, "type": "dru", "entity": "维生素K1" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "酚磺乙胺" } ] }, { "text": "4.解除颅内高压脑水肿者用20%甘露醇每次0.5g/kg,每6~8小时1次,呋塞米每次0.5mg/kg,每天1~2次。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "解除颅内高压" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "脑水肿" }, { "start_idx": 13, "end_idx": 18, "type": "dru", "entity": "20%甘露醇" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "呋塞米" } ] }, { "text": "颅内占位病变行手术治疗,脑积水行引流术。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "颅内" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "手术治疗" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "脑积水" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "引流术" } ] }, { "text": "十、L型菌肺炎L型菌肺炎是临床上难治性呼吸道感染的病原体之一。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "L型菌肺炎" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "L型菌肺炎" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "呼吸道感染" } ] }, { "text": "患儿常有肺炎不能解释的迁延发热,或原发病已愈,找不到继续发热的原因。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "肺炎" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "迁延发热" } ] }, { "text": "病情多不重,β-内酰胺类抗生素治疗无效。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "dru", "entity": "β-内酰胺类抗生素" } ] }, { "text": "X线改变无特异性,多呈间质性肺炎改变。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "X线" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "间质性肺炎" } ] }, { "text": "三、各型结核的治疗方案(表7-7)表7-1各型结核的治疗方案", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "结核" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "结核" } ] }, { "text": "第六章风湿性疾病相关的实验室检查风湿性疾病(rheumaticdiseases)包含一大类慢性全身性疾病,人们认识这类疾病经历了漫长的实践、变迁过程。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 22, "end_idx": 38, "type": "dis", "entity": "rheumaticdiseases" }, { "start_idx": 45, "end_idx": 51, "type": "dis", "entity": "慢性全身性疾病" } ] }, { "text": "在命名上,西方与中国古代医学早有“风湿病”的名称,但两者在概念及内容上大相径庭,不可类比。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "风湿病" } ] }, { "text": "本章中“风湿性疾病”仅涉及现代医学的“风湿病”范畴。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "风湿病" } ] }, { "text": "西方医学中,经典称谓的风湿病泛指“以疼痛为核心的骨、关节及肌肉等慢性疾病”。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "风湿病" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "骨" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "关节" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肌肉" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "慢性疾病" } ] }, { "text": "至1941年Klemperer总结了系统性红斑狼疮和硬皮病经验以及Klinge的研究成果,认为风湿性疾病均为机体胶原组织病理损伤的结果,因此提出“胶原性疾病”的概念。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "硬皮病" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 54, "end_idx": 59, "type": "bod", "entity": "机体胶原组织" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "胶原性疾病" } ] }, { "text": "但后来多数人认为风湿性疾病并不仅限于累及胶原组织,它可以是更广泛的结缔组织损伤。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "胶原组织" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "结缔组织" } ] }, { "text": "为此Ehrich建议将“胶原性疾病”更名为“结缔组织病”,故此命名又沿用了几十年。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "胶原性疾病" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "结缔组织病" } ] }, { "text": "至20世纪中后期,大量的临床研究与实验研究发现,在很多结缔组织病病人体内能检出针对自身抗原的自身抗体和与自身细胞反应的致敏淋巴细胞以及病变组织中有各种抗体,补体与免疫复合物沉积及免疫活性细胞浸润,因而主张将结缔组织病更名为“自身免疫性疾病”。", "entities": [ { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "抗体" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "细胞" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "病变组织" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "抗体" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "补体" }, { "start_idx": 81, "end_idx": 87, "type": "bod", "entity": "免疫复合物沉积" }, { "start_idx": 89, "end_idx": 96, "type": "dis", "entity": "免疫活性细胞浸润" }, { "start_idx": 103, "end_idx": 107, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 112, "end_idx": 118, "type": "dis", "entity": "自身免疫性疾病" } ] }, { "text": "20世纪末众多临床学家认为“结缔组织病”与“自身免疫性疾病”的命名强调了病理学与免疫学研究结果,但都不能全部涵盖风湿性疾病的特征,故主张仍使用风湿性疾病的名称。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 71, "end_idx": 75, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "但现代风湿病概念已特指自身免疫耐受破坏后,发生自身免疫损伤的一类风湿性疾病。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "现代风湿病" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "它不包含外伤、代谢异常以及退行性变等许多经典风湿性疾病。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "外伤" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "代谢异常" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "风湿性疾病的病因并不十分清楚,遗传因素、感染因素以及免疫异常是公认的值得深入研究的方向。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "风湿性疾病往往因累及的器官不同而表现出由各种症状和体征组合的临床综合征。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "器官" } ] }, { "text": "“胶原性疾病”、“结缔组织病”以及“自身免疫性疾病”的命名均从不同角度反映了风湿性疾病的基本特征,这些命名有助于深入理解和研究风湿性疾病。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "胶原性疾病" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "目前还缺乏针对各种风湿性疾病有特异性诊断价值的实验室检查项目,诊断主要依靠临床症状与实验室检查的综合分析,在除外其他疾病基础上,风湿性疾病的诊断才能成立。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "一、免疫学检查(一)免疫活化现象免疫活化现象有关的实验室检查结果是引导临床诊断风湿性疾病的简易途径:1.血清免疫球蛋白(immunoglobulin,Ig)Ig水平升高是风湿性疾病最常见的免疫活化现象,系统性红斑狼疮(SLE)、幼年特发性关节炎(JIA)、皮肌炎以及各种血管炎综合征均常有一种或几种免疫球蛋白显著升高B细胞增殖及分泌功能过度活化淋巴细胞表型分析免疫活化现象还可以在淋巴细胞表型分析检测发现CD3、CD4、CD8、CD16、CD19、CD20阳性细胞绝对数与相对数增加得到证实。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "免疫学检查" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "免疫活化现象" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "免疫活化现象" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 52, "end_idx": 58, "type": "bod", "entity": "血清免疫球蛋白" }, { "start_idx": 60, "end_idx": 73, "type": "bod", "entity": "immunoglobulin" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "Ig" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "Ig" }, { "start_idx": 85, "end_idx": 89, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 94, "end_idx": 99, "type": "sym", "entity": "免疫活化现象" }, { "start_idx": 101, "end_idx": 107, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 109, "end_idx": 111, "type": "dis", "entity": "SLE" }, { "start_idx": 114, "end_idx": 121, "type": "dis", "entity": "幼年特发性关节炎" }, { "start_idx": 123, "end_idx": 125, "type": "dis", "entity": "JIA" }, { "start_idx": 128, "end_idx": 130, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 135, "end_idx": 140, "type": "dis", "entity": "血管炎综合征" }, { "start_idx": 149, "end_idx": 153, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 149, "end_idx": 157, "type": "sym", "entity": "免疫球蛋白显著升高" }, { "start_idx": 158, "end_idx": 160, "type": "bod", "entity": "B细胞" }, { "start_idx": 158, "end_idx": 171, "type": "sym", "entity": "B细胞增殖及分泌功能过度活化" }, { "start_idx": 172, "end_idx": 175, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 180, "end_idx": 185, "type": "sym", "entity": "免疫活化现象" }, { "start_idx": 190, "end_idx": 193, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 224, "end_idx": 231, "type": "bod", "entity": "CD20阳性细胞" } ] }, { "text": "3.免疫病理各脏器组织免疫病理学检测可见多种Ig、补体、抗原抗体复合物沉积病变区域或血管内外以及大量淋巴细胞的组织浸润补体检测测定补体活性及其中某些成分有助于判断几种风湿性疾病的活动性,尤其是对SLE有诊断意义。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "脏器组织" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "免疫病理学检测" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "Ig" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "补体" }, { "start_idx": 28, "end_idx": 40, "type": "bod", "entity": "抗原抗体复合物沉积病变区域" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "血管" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "组织" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "组织浸润" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "补体检测" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "补体" }, { "start_idx": 83, "end_idx": 87, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 97, "end_idx": 99, "type": "dis", "entity": "SLE" } ] }, { "text": "C3、C4显著降低是补体消耗现象,也是免疫活化的标志。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "C3" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "C4" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "补体" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "免疫活化" } ] }, { "text": "一定要注意极少数原发性补体缺陷病以及与补体缺陷有关的风湿性疾病(表17-17)以上免疫活化现象仅是诊断各种风湿性疾病的提示指标。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "原发性补体缺陷病" }, { "start_idx": 19, "end_idx": 30, "type": "dis", "entity": "补体缺陷有关的风湿性疾病" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "免疫活化现象" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "表17-13补体缺陷与风湿性疾病(二)自身抗体与抗核抗体谱理论上讲,检出自身抗体是自身免疫性疾病的直接证据。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "补体缺陷" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "自身抗体" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "自身抗体" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "自身免疫性疾病" } ] }, { "text": "最早人们发现了抗细胞核自身抗体(ANA),并成为诊断SLE的有力工具,随后发现ANA并非SLE特异性诊断抗体,干燥综合征、皮肌炎、关节炎以及混合性结缔组织病均见ANA阳性。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "bod", "entity": "抗细胞核自身抗体" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "ANA" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "SLE" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "ANA" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "SLE" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "干燥综合征" }, { "start_idx": 61, "end_idx": 63, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "关节炎" }, { "start_idx": 70, "end_idx": 77, "type": "dis", "entity": "混合性结缔组织病" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "ANA" } ] }, { "text": "无任何临床症状的“健康人”也见低滴度ANA阳性(也可能是各种风湿病亚临床病人)。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "ANA" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "风湿病" } ] }, { "text": "近年研究成果发现依据针对不同细胞核成分可将抗核抗体分成多种抗核抗体亚类谱系,抗核抗体谱能更好地帮助临床鉴别诊断风湿性疾病。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "细胞核" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "但并不是所有抗核抗体亚类都具备非常高的特异性和敏感性。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "抗核抗体" } ] }, { "text": "1.类风湿因子(rheumatoidfactor,RF)属一种抗Ig稳定区(Fc)的自身抗体。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "类风湿因子" }, { "start_idx": 8, "end_idx": 23, "type": "bod", "entity": "rheumatoidfactor" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "RF" }, { "start_idx": 31, "end_idx": 45, "type": "bod", "entity": "抗Ig稳定区(Fc)的自身抗体" } ] }, { "text": "虽然最先在类风湿关节炎病人血清中发现RF,但它在疾病中确切的病理及生理意义并未弄清。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "类风湿关节炎" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "血清" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "RF" } ] }, { "text": "健康人血清中的RF为天然自身抗体,往往亲和力低,效价低,属IgM抗体,可能来自胚胎性免疫球蛋白基因。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血清" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "RF" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "自身抗体" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "IgM抗体" }, { "start_idx": 39, "end_idx": 48, "type": "bod", "entity": "胚胎性免疫球蛋白基因" } ] }, { "text": "由RF构成的免疫复合体还可以在滑膜液、滑膜组织中及皮肤溃疡、病变血管和吞噬细胞中检出。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "RF" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "免疫复合体" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "滑膜液" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "滑膜组织" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "皮肤溃疡" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "病变血管" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "吞噬细胞" } ] }, { "text": "急、慢性感染,恶性肿瘤病人RF常呈阳性,甚至正常人也有低效价RF被检出,年龄越大阳性结果可能性越大。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "慢性感染" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "RF" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "RF" } ] }, { "text": "虽然检测RF对JIA诊断并非关键,但RF阳性JIA患儿常伴有严重、顽固的关节炎及血管炎,值得重视。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "RF" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "JIA" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "RF阳性JIA" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "关节炎" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "血管炎" } ] }, { "text": "推测RF阳性JIA病人的发病机制可能与成人类风湿关节炎发病机制相同。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "RF阳性JIA" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "类风湿关节炎" } ] }, { "text": "2.隐匿性RF(hiddenrheumatoidfactor)沉降系数为19S,常规方法检测不出来,在血清中它与IgG结合在一起,用酸性凝胶滤过处理血清之后才能将其分离后再用ELISA方法测定。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "隐匿性RF" }, { "start_idx": 8, "end_idx": 29, "type": "bod", "entity": "hiddenrheumatoidfactor" }, { "start_idx": 31, "end_idx": 34, "type": "ite", "entity": "沉降系数" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "血清" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "IgG" }, { "start_idx": 66, "end_idx": 69, "type": "dru", "entity": "酸性凝胶" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "血清" }, { "start_idx": 87, "end_idx": 91, "type": "pro", "entity": "ELISA" } ] }, { "text": "有研究者发现65%~75%用经典方法检测RF阴性的JIA患儿隐匿性RF检测可能阳性。", "entities": [ { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "RF阴性的JIA" }, { "start_idx": 30, "end_idx": 36, "type": "pro", "entity": "隐匿性RF检测" } ] }, { "text": "Moore等人认为这种隐匿性RF与JIA活动有关,因此有助于诊断和治疗观察。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "隐匿性RF" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "JIA" } ] }, { "text": "3.抗核抗体(antinuclearantibodies,ANAs)是与各种细胞核成分反应的自身抗体的总称,包括针对脱氧核糖核酸(DNA)、可提取的核蛋白(snENP)、核糖核酸(RNA)、组蛋白、酶及核仁等细胞核成分的抗体。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 7, "end_idx": 27, "type": "bod", "entity": "antinuclearantibodies" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "ANAs" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "细胞核" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "自身抗体" }, { "start_idx": 58, "end_idx": 63, "type": "bod", "entity": "脱氧核糖核酸" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "DNA" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "核蛋白" }, { "start_idx": 78, "end_idx": 82, "type": "bod", "entity": "snENP" }, { "start_idx": 85, "end_idx": 88, "type": "bod", "entity": "核糖核酸" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "RNA" }, { "start_idx": 95, "end_idx": 97, "type": "bod", "entity": "组蛋白" }, { "start_idx": 99, "end_idx": 99, "type": "bod", "entity": "酶" }, { "start_idx": 101, "end_idx": 102, "type": "bod", "entity": "核仁" }, { "start_idx": 104, "end_idx": 106, "type": "bod", "entity": "细胞核" } ] }, { "text": "研究证明抗核抗体见于多种风湿性疾病,是筛查诊断SLE的指引性项目。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "SLE" } ] }, { "text": "几乎所有SLE病人,50%硬皮病,25%皮肌炎和超过50%小年龄JIA,如少关节型女性患儿及RF阳性多关节型患儿会有ANA阳性结果。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "SLE" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "硬皮病" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "JIA" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "RF" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "ANA" } ] }, { "text": "ANA阳性JIA常伴虹膜睫状体炎。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "ANA阳性JIA" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "虹膜睫状体炎" } ] }, { "text": "健康儿童也有2%~9%的人ANA阳性,有人通过5年随访也未见这些ANA阳性健康儿童发展成为风湿性疾病。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "ANA" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "ANA" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "Miles和Isenberg(1993)发现在风湿性疾病病人的一、二级亲属中ANA阳性检出率较高。", "entities": [ { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "ANA" } ] }, { "text": "ANA滴度与临床症状间并无必然联系,但它有助于疾病随访。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "ANA" } ] }, { "text": "在细菌、病毒及真菌感染,甚至患恶性肿瘤时ANA也见阳性。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "mic", "entity": "细菌" }, { "start_idx": 4, "end_idx": 5, "type": "mic", "entity": "病毒" }, { "start_idx": 7, "end_idx": 8, "type": "mic", "entity": "真菌" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "ANA" } ] }, { "text": "4.抗核抗体谱包括抗DNA抗体、抗组蛋白抗体、抗DNA组蛋白复合物抗体以及抗非组蛋白4种大类的自身抗体。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "抗DNA抗体" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "抗组蛋白抗体" }, { "start_idx": 23, "end_idx": 34, "type": "bod", "entity": "抗DNA组蛋白复合物抗体" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "抗非组蛋白" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "自身抗体" } ] }, { "text": "目前国内普遍采用德国欧蒙试剂的抗核抗体谱检测,包括抗SS-A、抗SS-B、抗-Sm、抗Scl-70、抗Jo-1、抗Ro-52抗核小体抗体、抗组蛋白抗体、抗CNEPB、核糖体P蛋白抗体和抗nRNP/Sm等12种抗原的IgG抗体。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "德国欧蒙试剂" }, { "start_idx": 15, "end_idx": 21, "type": "pro", "entity": "抗核抗体谱检测" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "抗SS-A" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "抗SS-B" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "抗-Sm" }, { "start_idx": 42, "end_idx": 48, "type": "bod", "entity": "抗Scl-70" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "抗Jo-1" }, { "start_idx": 56, "end_idx": 67, "type": "bod", "entity": "抗Ro-52抗核小体抗体" }, { "start_idx": 69, "end_idx": 74, "type": "bod", "entity": "抗组蛋白抗体" }, { "start_idx": 76, "end_idx": 81, "type": "bod", "entity": "抗CNEPB" }, { "start_idx": 83, "end_idx": 90, "type": "bod", "entity": "核糖体P蛋白抗体" }, { "start_idx": 92, "end_idx": 99, "type": "bod", "entity": "抗nRNP/Sm" }, { "start_idx": 107, "end_idx": 111, "type": "bod", "entity": "IgG抗体" } ] }, { "text": "除抗dsDNA外,可提取核抗原(ENA)抗体中诊断意义较大的有抗-Sm、抗nRNP/Sm、抗SS-A、抗SS-B、抗Scl-70以及抗Jo-1六种自身抗体:①抗dsDNA:抗dsDNA抗体检测对诊断SLE具极高特异性(>95%),且对SLE预后判断也有重要价值,但其敏感性只有30%~40%。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "抗dsDNA" }, { "start_idx": 12, "end_idx": 21, "type": "bod", "entity": "核抗原(ENA)抗体" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "抗-Sm" }, { "start_idx": 36, "end_idx": 43, "type": "bod", "entity": "抗nRNP/Sm" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "抗SS-A" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "抗SS-B" }, { "start_idx": 57, "end_idx": 63, "type": "bod", "entity": "抗Scl-70" }, { "start_idx": 66, "end_idx": 70, "type": "bod", "entity": "抗Jo-1" }, { "start_idx": 73, "end_idx": 76, "type": "bod", "entity": "自身抗体" }, { "start_idx": 79, "end_idx": 84, "type": "bod", "entity": "抗dsDNA" }, { "start_idx": 86, "end_idx": 95, "type": "pro", "entity": "抗dsDNA抗体检测" }, { "start_idx": 99, "end_idx": 101, "type": "dis", "entity": "SLE" }, { "start_idx": 117, "end_idx": 119, "type": "dis", "entity": "SLE" } ] }, { "text": "高滴度抗dsDNA抗体不仅表明SLE的活动性,而且提示病变累及肾脏的可能性极大。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "bod", "entity": "高滴度抗dsDNA抗体" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "SLE" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肾脏" } ] }, { "text": "dsDNA抗体阳性率易受皮质激素和免疫抑制剂使用影响。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "dsDNA抗体" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "皮质激素" }, { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "抗ssDNA抗体阳性率在SLE中高于抗dsDNA抗体,但其特异性不如后者;在其他AID,甚至病毒感染性疾病中也可见到抗ssDNA抗体阳性。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "抗ssDNA抗体" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "SLE" }, { "start_idx": 18, "end_idx": 25, "type": "bod", "entity": "抗dsDNA抗体" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "AID" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "病毒感染性疾病" }, { "start_idx": 58, "end_idx": 65, "type": "bod", "entity": "抗ssDNA抗体" } ] }, { "text": "②抗-Sm抗体:抗-Sm抗体也是SLE的标志性抗体,诊断特异性也超过95%,敏感性在40%左右。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "抗-Sm抗体" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "抗-Sm抗体" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "SLE" } ] }, { "text": "③抗nRNP/Sm:抗nRNP/Sm对混合性结缔组织病阳性率可达100%,但SLE及干燥综合征也有抗nRNP/Sm阳性检出。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "bod", "entity": "抗nRNP/Sm" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "抗nRNP/Sm" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "混合性结缔组织病" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "SLE" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "干燥综合征" }, { "start_idx": 49, "end_idx": 56, "type": "bod", "entity": "抗nRNP/Sm" } ] }, { "text": "④抗SS-A及抗SS-B:抗SS-A和抗SS-B在干燥综合征中阳性检出率分别为75%和60%,但也见于SLE、硬皮病、皮肌炎以及关节炎等患者。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "抗SS-A" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "抗SS-B" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "抗SS-A" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "抗SS-B" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "干燥综合征" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "SLE" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "硬皮病" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 64, "end_idx": 66, "type": "dis", "entity": "关节炎" } ] }, { "text": "⑤抗Scl-70:抗Scl-70为硬皮病标志性抗体,但阳性率不足50%。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "bod", "entity": "抗Scl-70" }, { "start_idx": 9, "end_idx": 15, "type": "bod", "entity": "抗Scl-70" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "硬皮病" } ] }, { "text": "⑥抗Jo-1:抗Jo-1见于多肌炎患者,阳性率仅25%~35%,常与合并肺间质纤维化有关。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "抗Jo-1" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "抗Jo-1" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "多肌炎" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "肺间质纤维化" } ] }, { "text": "5.其他细胞成分自身抗体这类抗体仅与专一的细胞成分反应,引起相应的细胞病变也较为局限,如:(1)抗细胞抗体:如抗淋巴细胞、红细胞、血小板及精子细胞抗体,引起临床所见淋巴细胞坏死性、增生性疾病及自身免疫性溶血症、血小板减少症和不孕症等疾病。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "细胞" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "自身抗体" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "抗体" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "细胞" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "细胞" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "抗细胞抗体" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "抗淋巴细胞" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "红细胞" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "血小板" }, { "start_idx": 69, "end_idx": 74, "type": "bod", "entity": "精子细胞抗体" }, { "start_idx": 82, "end_idx": 87, "type": "dis", "entity": "淋巴细胞坏死" }, { "start_idx": 90, "end_idx": 94, "type": "dis", "entity": "增生性疾病" }, { "start_idx": 96, "end_idx": 103, "type": "dis", "entity": "自身免疫性溶血症" }, { "start_idx": 105, "end_idx": 110, "type": "dis", "entity": "血小板减少症" }, { "start_idx": 112, "end_idx": 114, "type": "dis", "entity": "不孕症" } ] }, { "text": "(2)抗细胞质抗体:如抗线粒体抗体、核糖体抗体及溶酶体抗体等,引起相应的病变。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "抗细胞质抗体" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "抗线粒体抗体" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "核糖体抗体" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "溶酶体抗体" } ] }, { "text": "(3)抗受体抗体:如抗乙酰胆碱受体及抗胰岛素受体抗体等所引起的自身免疫性神经传导性病变(自身免疫性重症肌无力)和胰腺等神经内分泌相关疾病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "抗受体抗体" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "抗乙酰胆碱受体" }, { "start_idx": 18, "end_idx": 25, "type": "bod", "entity": "抗胰岛素受体抗体" }, { "start_idx": 31, "end_idx": 42, "type": "dis", "entity": "自身免疫性神经传导性病变" }, { "start_idx": 44, "end_idx": 53, "type": "dis", "entity": "自身免疫性重症肌无力" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "胰腺" } ] }, { "text": "(4)抗中性粒细胞胞浆抗体(ANCA):ANCA是近年研究较多的自身抗体之一,ANCA主要有两个亚型,即抗蛋白酶3(PR3)抗体,称为胞浆型ANCA(C-ANCA)和抗髓过氧化物酶(MPO)抗体,称为核周型ANCA(P-ANCA)。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "bod", "entity": "抗中性粒细胞胞浆抗体" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "ANCA" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "ANCA" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "自身抗体" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "ANCA" }, { "start_idx": 52, "end_idx": 63, "type": "bod", "entity": "抗蛋白酶3(PR3)抗体" }, { "start_idx": 67, "end_idx": 73, "type": "bod", "entity": "胞浆型ANCA" }, { "start_idx": 75, "end_idx": 80, "type": "bod", "entity": "C-ANCA" }, { "start_idx": 83, "end_idx": 89, "type": "bod", "entity": "抗髓过氧化物酶" }, { "start_idx": 91, "end_idx": 93, "type": "bod", "entity": "MPO" }, { "start_idx": 100, "end_idx": 106, "type": "bod", "entity": "核周型ANCA" }, { "start_idx": 108, "end_idx": 113, "type": "bod", "entity": "P-ANCA" } ] }, { "text": "ANCA主要出现在以系统性小血管炎为基础病变的AID患者血中,如Wegener肉芽肿、镜下多小动脉炎、系统性血管炎、川崎病、SLE以及过敏性紫癜等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "ANCA" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "系统性小血管炎" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "AID" }, { "start_idx": 32, "end_idx": 41, "type": "dis", "entity": "Wegener肉芽肿" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "镜下多小动脉炎" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "系统性血管炎" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "川崎病" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "SLE" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "过敏性紫癜" } ] }, { "text": "临床上把ANCA视为小血管炎并肾炎的特殊血清学标志。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "ANCA" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "小血管炎" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "肾炎" } ] }, { "text": "(5)抗磷脂抗体(aPL抗体):aPL抗体是一组非均一性抗体,磷脂是从牛心组织中提取的一种抗原,命名为心磷脂。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "抗磷脂抗体" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "aPL抗体" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "aPL抗体" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "磷脂" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "心磷脂" } ] }, { "text": "临床上aPL分为两种,即狼疮凝集物(LAC)和抗心磷脂抗体(aCL)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "aPL" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "狼疮凝集物" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "LAC" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "抗心磷脂抗体" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "aCL" } ] }, { "text": "LAC能与凝血酶原复合物中磷脂成分结合而抑制凝血作用,可能与SLE病人出血及凝血机制异常有关,实验发现aCL抗体有laG与IgM两种,IgG型与AID并流产、血栓形成及血小板减少有关;IgM型与AID并溶血性贫血、粒细胞减少、网状青斑及心内膜病变有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "LAC" }, { "start_idx": 5, "end_idx": 11, "type": "bod", "entity": "凝血酶原复合物" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "磷脂" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "SLE" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "aCL抗体" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "laG" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "IgM" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "IgG" }, { "start_idx": 72, "end_idx": 74, "type": "dis", "entity": "AID" }, { "start_idx": 76, "end_idx": 77, "type": "dis", "entity": "流产" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "血栓" }, { "start_idx": 84, "end_idx": 86, "type": "bod", "entity": "血小板" }, { "start_idx": 92, "end_idx": 94, "type": "bod", "entity": "IgM" }, { "start_idx": 97, "end_idx": 99, "type": "dis", "entity": "AID" }, { "start_idx": 101, "end_idx": 105, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 107, "end_idx": 111, "type": "dis", "entity": "粒细胞减少" }, { "start_idx": 113, "end_idx": 116, "type": "dis", "entity": "网状青斑" }, { "start_idx": 118, "end_idx": 122, "type": "dis", "entity": "心内膜病变" } ] }, { "text": "(6)抗神经节苷脂抗体(GM抗体):抗GM抗体针对髓鞘脂类成分,AID中抗GM抗体主要引起神经及精神病变,IgM型抗GM抗体与SLE脑病高度相关。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "bod", "entity": "抗神经节苷脂抗体" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "GM抗体" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "抗GM抗体" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "髓鞘脂类" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "AID" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "抗GM抗体" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "神经" }, { "start_idx": 53, "end_idx": 61, "type": "bod", "entity": "IgM型抗GM抗体" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "SLE脑病" } ] }, { "text": "检测抗GM抗体可能有预测SLE神经系统并发症的价值,尤其是脑脊液中抗GM抗体检测。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "抗GM抗体" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "SLE神经系统并发症" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "脑脊液" }, { "start_idx": 33, "end_idx": 39, "type": "pro", "entity": "抗GM抗体检测" } ] }, { "text": "6.器官特异性自身抗体同细胞专一成分的自身抗体一样,器官特异性自身抗体主要针对某些器官组织并引起器官特异性AID。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "bod", "entity": "器官特异性自身抗体" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "细胞" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "自身抗体" }, { "start_idx": 26, "end_idx": 34, "type": "bod", "entity": "器官特异性自身抗体" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "器官组织" }, { "start_idx": 48, "end_idx": 55, "type": "dis", "entity": "器官特异性AID" } ] }, { "text": "(1)抗脑组织抗体:有41.5%的精神分裂症患者、37%脑血管意外患者血清中可检出抗脑组织抗体,而正常人中该抗体阳性率仅1.4%,先天性脑发育不全者该抗体阳性率为9.1%。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "抗脑组织抗体" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "精神分裂症" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "脑血管" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "血清" }, { "start_idx": 41, "end_idx": 46, "type": "bod", "entity": "抗脑组织抗体" }, { "start_idx": 65, "end_idx": 72, "type": "dis", "entity": "先天性脑发育不全" } ] }, { "text": "37例癫痫患者中抗脑组织抗体阳性率也皆高于正常对照组,在风湿性疾病脑损害中的诊断价值有待研究。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "癫痫" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "抗脑组织抗体" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "脑损害" } ] }, { "text": "(2)抗心肌M受体抗体:抗心肌抗体的靶抗原十分复杂,可针对肌纤维、肌动蛋白、肌凝蛋白及Z带等抗原成分,因此在心肌梗死、风湿性心肌炎、病毒性心肌炎、心肌病、冠状动脉硬化性心脏病,甚至心脏手术之后,均可检出抗心肌抗体。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "bod", "entity": "抗心肌M受体抗体" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "抗心肌抗体" }, { "start_idx": 18, "end_idx": 20, "type": "mic", "entity": "靶抗原" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "肌纤维" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "肌动蛋白" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "肌凝蛋白" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "Z带" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "心肌梗死" }, { "start_idx": 59, "end_idx": 64, "type": "dis", "entity": "风湿性心肌炎" }, { "start_idx": 66, "end_idx": 71, "type": "dis", "entity": "病毒性心肌炎" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "心肌病" }, { "start_idx": 77, "end_idx": 86, "type": "dis", "entity": "冠状动脉硬化性心脏病" }, { "start_idx": 90, "end_idx": 93, "type": "pro", "entity": "心脏手术" }, { "start_idx": 101, "end_idx": 105, "type": "bod", "entity": "抗心肌抗体" } ] }, { "text": "目前难以确定是组织损伤,自身抗原改变刺激产生自身抗体,还是感染、药物等诱生的自身抗体。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "自身抗体" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "自身抗体" } ] }, { "text": "(3)抗甲状腺球蛋白及抗甲状腺微粒体抗体(TGA与TPO):在桥本甲状腺炎患者血清中高滴度TGA90%~95%,TPO检测也有相应诊断价值。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "bod", "entity": "抗甲状腺球蛋白" }, { "start_idx": 11, "end_idx": 19, "type": "bod", "entity": "抗甲状腺微粒体抗体" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "TGA" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "TPO" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "桥本甲状腺炎" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "血清" }, { "start_idx": 42, "end_idx": 46, "type": "ite", "entity": "高滴度TG" }, { "start_idx": 42, "end_idx": 46, "type": "bod", "entity": "高滴度TG" } ] }, { "text": "(4)类风湿关节炎特异性抗体:多年来在RA患者中(尤其是儿童RA)难以检出特异性抗体,一直是令医师头痛的问题。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "bod", "entity": "类风湿关节炎特异性抗体" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "RA" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "儿童RA" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "特异性抗体" } ] }, { "text": "最近发现一种被称为Sa的抗原物质,其相对分子质量为50000或55000,属于人体组织的正常成分。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "Sa" } ] }, { "text": "抗Sa抗体在成人RA中阳性率为31.9%,在SLE为4.3%,干燥综合征为3%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "抗Sa抗体" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "RA" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "SLE" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "干燥综合征" } ] }, { "text": "抗Sa抗体对RA诊断特异性为98.6%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "抗Sa抗体" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "RA" } ] }, { "text": "研究发现抗Sa抗体与RF、RA3、SSA、SSB、RNP、Sm、Jo-1及Scl-70等多种自身抗体无交叉反应性,遗憾的是Sa抗体对JIA的诊断价值罕见报告。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "抗Sa抗体" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "RF" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "RA3" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "SSA" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "SSB" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "RNP" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "Sm" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "Jo-1" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "自身抗体" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "Sa抗体" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "JIA" } ] }, { "text": "抗环瓜氨酸抗体(ACCP):研究表明,JIA患儿ACCP为阳性率不足2%,远低于成年(63%)。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "抗环瓜氨酸抗体" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "ACCP" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "JIA" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "ACCP" } ] }, { "text": "因此,ACCP难以作为JIA诊断的筛选手段。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "ACCP" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "JIA" } ] }, { "text": "但有报告13%的多关节型和2%的其他类型JIA血清中ACCP抗体为阳性,健康对照仅0.6%阳性,其中RF阳性多关节型JIA患儿中57%ACCP抗体为阳性。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "JIA" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "血清" }, { "start_idx": 26, "end_idx": 31, "type": "bod", "entity": "ACCP抗体" }, { "start_idx": 50, "end_idx": 60, "type": "dis", "entity": "RF阳性多关节型JIA" }, { "start_idx": 67, "end_idx": 72, "type": "bod", "entity": "ACCP抗体" } ] }, { "text": "HLA-DR4阳性多关节型患儿ACCP抗体阳性率高于HLA-DR4阴性的患儿,且与多关节型发病、病程和关节破坏程度有关。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "HLA-DR4" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "ACCP抗体" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "HLA-DR4" } ] }, { "text": "抗核周因子抗体(APF):NesherG检测64名JIA患儿(28名多关节型,26名少关节型,10名全身型),结果多关节型中APF10名阳性,少关节型中5名阳性,全身型中1名阳性。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "抗核周因子抗体" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "APF" }, { "start_idx": 13, "end_idx": 21, "type": "pro", "entity": "NesherG检测" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "JIA" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "APF" } ] }, { "text": "遗憾的是,以上自身抗体能提示某种器官损害,其特异性及阳性率报告差异极大,以器官疾病命名的自身抗体不一定具有诊断该病的高度特异性,临床医师绝不能僵化地看待自身抗体的报告。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "自身抗" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "自身抗" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "提示" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "极大" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "病命名的" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "化地看待" } ] }, { "text": "临床检测自身抗体要注意3点:①自身抗体有生理性与病理性之分,生理性自身抗体不但效价低,产生机制也可能不同;②多数自身抗体特异性欠佳,要特别强调临床综合分析;③自身抗体仅用于风湿性疾病诊断,在一些非风湿性疾病中也通过自身抗体检测获取临床信息,如肺癌诊断,淋巴细胞白血病分型等。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "自身抗体" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "自身抗体" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "自身抗体" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "自身抗体" }, { "start_idx": 79, "end_idx": 81, "type": "ite", "entity": "自身抗" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "自身抗" }, { "start_idx": 82, "end_idx": 86, "type": "dis", "entity": "体仅用于风" }, { "start_idx": 94, "end_idx": 98, "type": "dis", "entity": "在一些非风" }, { "start_idx": 103, "end_idx": 108, "type": "pro", "entity": "中也通过自身" }, { "start_idx": 117, "end_idx": 118, "type": "dis", "entity": "信息" }, { "start_idx": 122, "end_idx": 128, "type": "dis", "entity": "癌诊断,淋巴细" } ] }, { "text": "第七章心肌病心肌病(cardiomyopathy)为发生于心肌的疾病。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "心肌病" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "心肌病" }, { "start_idx": 10, "end_idx": 23, "type": "dis", "entity": "cardiomyopathy" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "心肌" } ] }, { "text": "该术语最初出现于1957年,当时指一组不能归因于冠状动脉病变的心肌病变。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "心肌" } ] }, { "text": "此后,心肌病的定义发生了变化。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "心肌病" } ] }, { "text": "目前,心肌病的定义为心肌的结构或功能异常,且无高血压或肺动脉高压、无心脏瓣膜病变、无先天性心脏病而言。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "心肌病" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心肌" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "高血压" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "心脏瓣膜病变" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "心脏病" } ] }, { "text": "以解剖与生理改变为依据,可将心肌病分为以下三型:①扩张(充血)型心肌病:此型左心室或双心室扩大,心肌收缩功能不同程度降低充血性心力衰竭的症状与体征。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "心肌病" }, { "start_idx": 25, "end_idx": 34, "type": "dis", "entity": "扩张(充血)型心肌病" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "心肌" }, { "start_idx": 48, "end_idx": 59, "type": "sym", "entity": "心肌收缩功能不同程度降低" }, { "start_idx": 60, "end_idx": 66, "type": "sym", "entity": "充血性心力衰竭" } ] }, { "text": "②肥厚性心肌病:先前称之为特发性肥厚性心肌病,以左心室肥厚左心室流出道梗阻舒张功能障碍或心律失常引起猝死。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "肥厚性心肌病" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "左心室" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "左心室肥厚" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "左心室" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "左心室流出道梗阻" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "舒张功能障碍" }, { "start_idx": 44, "end_idx": 45, "type": "ite", "entity": "心律" }, { "start_idx": 44, "end_idx": 51, "type": "sym", "entity": "心律失常引起猝死" } ] }, { "text": "③限制型心肌病(restrictivecardiomyopathy):心房显著扩大,一般心室大小及收缩功能正常,舒张功能损害,症状由肺及体循环静脉充血引起,也可出现晕厥。", "entities": [ { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "心室" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "肺" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "体循环" }, { "start_idx": 82, "end_idx": 83, "type": "sym", "entity": "晕厥" } ] }, { "text": "第一节扩张性心肌病【病因】【扩张性心肌病扩张性心肌病(dilatedcardiomyopathy,DCM)在各种类型心肌病中最为常见,在美国及欧洲,其年发病率约为2/10万~8/10万人口,据估计每10万人口中约有36人患有DCM。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "扩张性心肌病" }, { "start_idx": 27, "end_idx": 47, "type": "dis", "entity": "dilatedcardiomyopathy" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "DCM" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "心肌病" }, { "start_idx": 112, "end_idx": 114, "type": "dis", "entity": "DCM" } ] }, { "text": "此外,20%~30%的DCM患者为家族性的。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "DCM" } ] }, { "text": "表9-10扩张性心肌病的病因【病理】扩张性心肌病病变以心肌纤维化为主,心肌肥厚不显著心腔扩大明显二尖瓣环和三尖瓣环增大,乳头肌伸长心腔内附壁血栓心肌节律点及传导系统而引起心律失常。", "entities": [ { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "扩张性心肌病" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "心肌纤维化" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "心肌" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "心肌肥厚不显著" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "心腔" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "心腔扩大明显" }, { "start_idx": 48, "end_idx": 58, "type": "sym", "entity": "二尖瓣环和三尖瓣环增大" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "乳头肌" }, { "start_idx": 60, "end_idx": 64, "type": "sym", "entity": "乳头肌伸长" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "心腔" }, { "start_idx": 65, "end_idx": 71, "type": "sym", "entity": "心腔内附壁血栓" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "心肌" }, { "start_idx": 85, "end_idx": 88, "type": "sym", "entity": "心律失常" } ] }, { "text": "由于心肌纤维化,心肌收缩功能心力衰竭。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心肌" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "心肌收缩功能" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "心力衰竭" } ] }, { "text": "主要表现为心脏增大心力衰竭,心律失常小动脉栓塞心脏增大气喘、乏力、心悸、咳嗽、胸闷等症状,有的可有偏瘫。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心脏" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "心脏增大" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "心律" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "心律失常" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "动脉" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "小动脉栓塞" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心脏" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "心脏增大" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "气喘" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "乏力" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "心悸" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "咳嗽" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "胸闷" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "偏瘫" } ] }, { "text": "体格检查可见心尖搏动弥散或抬举心浊音界向左扩大,心率增快可闻及Ⅱ/Ⅵ~Ⅲ/Ⅵ级收缩期杂音(心力衰竭控制后杂音减轻或消失),肝脏增大下肢水肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "体格检查" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "心尖" }, { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "心尖搏动弥散或抬举" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "心浊音界向左扩大" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "心率" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "心率增快" }, { "start_idx": 28, "end_idx": 43, "type": "sym", "entity": "可闻及Ⅱ/Ⅵ~Ⅲ/Ⅵ级收缩期杂音" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "肝脏" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "肝脏增大" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "下肢" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "下肢水肿" } ] }, { "text": "【实验室检查】(一)胸部X线检查心影扩大,由左心室、左心房扩大引起。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "ite", "entity": "胸部X线检查" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "心影" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "左心室" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "左心房" } ] }, { "text": "常存在肺静脉充血,可发展为肺水肿。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肺静脉" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "肺水肿" } ] }, { "text": "左肺部分区域可因左心房扩大压迫左支气管而致不张胸腔积液。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "左肺" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "左心房" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "左支气管" }, { "start_idx": 0, "end_idx": 22, "type": "sym", "entity": "左肺部分区域可因左心房扩大压迫左支气管而致不张" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "胸腔" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "胸腔积液" } ] }, { "text": "(二)心电图及HOLTER大多数患儿心电图上呈窦性心动过速左心室肥大左右心房扩大右心室肥大HOLTER检查可发现心律失常。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "心电图" }, { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "HOLTER" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "心动" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "窦性心动过速" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "左心室" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "左心室肥大" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "心房" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "左右心房扩大" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "右心室" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "右心室肥大" }, { "start_idx": 45, "end_idx": 52, "type": "pro", "entity": "HOLTER检查" } ] }, { "text": "(三)超声心动图DCM患儿的超声心动图特征包括左心室、左心房扩大,缩短分数及射血分数减低,左心室射血前期与射血期比率增加等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "DCM" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "左心室" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "左心房" }, { "start_idx": 23, "end_idx": 31, "type": "sym", "entity": "左心室、左心房扩大" } ] }, { "text": "(四)心导管检查与活体组织检查由于DCM可由超声心动图检查确定,心导管检查主要用于排除异常的左冠状动脉起源,因这一情况在超声心动图检查时易于漏诊,必要时活体组织检查帮助确定心肌病的病因。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "心导管检查" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "活体组织检查" }, { "start_idx": 22, "end_idx": 28, "type": "pro", "entity": "超声心动图检查" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "心导管检查" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "左冠状动脉" }, { "start_idx": 60, "end_idx": 66, "type": "pro", "entity": "超声心动图检查" }, { "start_idx": 76, "end_idx": 81, "type": "pro", "entity": "活体组织检查" }, { "start_idx": 86, "end_idx": 88, "type": "dis", "entity": "心肌病" } ] }, { "text": "【治疗】扩张性心肌病的临床特征为心输出量减少、液体潴留及血管收缩活性增加,后者为神经体液因素作用以维持足够的灌注压。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "扩张性心肌病" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "心输出量减少" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "液体潴留" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "血管收缩活性增加" } ] }, { "text": "增强心肌收缩力的药物1.第一类为拟交感药物包括多巴胺、多巴酚丁胺及肾上腺素。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "多巴胺" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "多巴酚丁胺" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "肾上腺素" } ] }, { "text": "多巴胺小剂量时可改善肾脏功能,剂量加大可增强对心脏的作用,但也可引起外周血管阻力增加,并有可能致心律失常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "多巴胺" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肾脏" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心脏" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "血管" }, { "start_idx": 48, "end_idx": 49, "type": "ite", "entity": "心律" } ] }, { "text": "多巴酚丁胺致心律失常作用较弱,但有报道因可引起肺动脉楔压升高而致肺水肿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "多巴酚丁胺" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "心律" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "肺动脉" } ] }, { "text": "2.第二类增强心肌收缩力的药物为双吡啶衍生剂包括氨力农及米力农,可通过抑制磷酸二酯酶增加细胞内钙的浓度,有强心及扩张外周血管的作用。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心肌" }, { "start_idx": 16, "end_idx": 21, "type": "dru", "entity": "双吡啶衍生剂" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "氨力农" }, { "start_idx": 35, "end_idx": 41, "type": "dru", "entity": "抑制磷酸二酯酶" }, { "start_idx": 44, "end_idx": 45, "type": "mic", "entity": "细胞" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "血管" } ] }, { "text": "其可能的副作用为血小板减少、肝毒性及胃肠道刺激。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "血小板" } ] }, { "text": "地高辛为可长期应用的经典心肌收缩力增强药物,但在危重病例,因心肌损害严重及肾功能减退,应减量慎用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "地高辛" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "心肌" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "心肌" } ] }, { "text": "3.利尿剂改善液体内环境平衡在扩张性心肌病的治疗中至关重要。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "利尿剂" } ] }, { "text": "呋塞米(速尿)为首选的药物,但应注意监测电解质水平,尤其是血钾水平,必要时可适当补充钾盐,也可与螺内酯等类药物合用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "呋塞米" }, { "start_idx": 4, "end_idx": 5, "type": "dru", "entity": "速尿" }, { "start_idx": 29, "end_idx": 32, "type": "ite", "entity": "血钾水平" }, { "start_idx": 42, "end_idx": 43, "type": "dru", "entity": "钾盐" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "螺内酯" } ] }, { "text": "4.血管扩张剂硝普钠及肼屈嗪可有效扩张外周血管,从而降低后负荷,增加心输出量及减低充盈压。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "血管扩张剂" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "硝普钠" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "肼屈嗪" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "血管" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "心" } ] }, { "text": "ACE抑制剂还有一定的抑制甚至逆转心肌病时的心室重塑作用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "ACE抑制剂" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "心肌病" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "心室" } ] }, { "text": "5.其他治疗扩张性心肌病因心腔扩大血流淤滞,有可能发生血栓形成。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "心肌" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心腔" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "心腔扩大" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "血流淤滞" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "血栓" } ] }, { "text": "药物的选择依心动过速的原因而定。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "心动" } ] }, { "text": "6.心脏移植儿童心脏移植近年已增加,且改善了严重心肌病患儿的存活率。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "心脏移植" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "心脏移植" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "心肌病" } ] }, { "text": "因此,重症心肌病患儿如积极的内科治疗无效,应考虑心脏移植。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "心肌病" }, { "start_idx": 14, "end_idx": 15, "type": "dep", "entity": "内科" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "心脏移植" } ] }, { "text": "二、营养素的主要功能和角色营养素主要功能除了作为能源物质外,其次还作为“建筑”材料,构成和修补机体组织、满足生长发育以及合成机体的免疫物质和激素等。", "entities": [ { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "机体组织" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "免疫物质" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "激素" } ] }, { "text": "第四节支原体肺炎支原体肺炎(mycoplasmalpneumonia)由肺炎支原体(mycoplasmapneumoniae,MP)引起。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "支原体肺炎" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "支原体肺炎" }, { "start_idx": 14, "end_idx": 33, "type": "dis", "entity": "mycoplasmalpneumonia" }, { "start_idx": 36, "end_idx": 40, "type": "mic", "entity": "肺炎支原体" }, { "start_idx": 42, "end_idx": 61, "type": "mic", "entity": "mycoplasmapneumoniae" }, { "start_idx": 63, "end_idx": 64, "type": "mic", "entity": "MP" } ] }, { "text": "北京首都儿科研究所报道,MP肺炎占住院儿童肺炎的19.2%~21.9%。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "MP肺炎" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "肺炎" } ] }, { "text": "北美和欧洲的研究表明,MP占肺炎的15.0%~34.3%,并随年龄增长而增多。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "MP" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肺炎" } ] }, { "text": "【病因】该病病原体为MP,它是介于细菌和病毒之间的一种微生物,能在细胞外独立生活,具有RNA和DNA。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "MP" }, { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "细菌" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "病毒" }, { "start_idx": 43, "end_idx": 45, "type": "mic", "entity": "RNA" }, { "start_idx": 47, "end_idx": 49, "type": "mic", "entity": "DNA" } ] }, { "text": "患儿常有发热、畏寒、头痛、咽痛、咳嗽、全身不适、疲乏、食欲缺乏、恶心、呕吐、腹泻等症状,但鼻部卡他症状少见。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "发热" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "畏寒" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "头痛" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "咽痛" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "咳嗽" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "全身不适" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "疲乏" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "恶心" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "呕吐" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "腹泻" } ] }, { "text": "婴幼儿可表现为憋气,年长儿可感胸闷、胸痛。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "憋气" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "胸闷" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "胸痛" } ] }, { "text": "年长患儿肺部常无阳性体征,这是本病的特点之一。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肺部" } ] }, { "text": "少数病例呼吸音减弱,有干、湿啰音,这些体征常在X线改变之后出现。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "呼吸音减弱" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "干、湿啰音" } ] }, { "text": "此外,可发生肺脓疡、胸膜炎、肺不张、支气管扩张症、弥漫性间质性肺纤维化等。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "肺脓疡" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "胸膜炎" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肺不张" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "支气管扩张症" }, { "start_idx": 25, "end_idx": 34, "type": "dis", "entity": "弥漫性间质性肺纤维化" } ] }, { "text": "本病尚可并发神经系统、血液系统、心血管系统、皮肤、肌肉和关节等肺外并发症,如脑膜脑炎、神经根神经炎、心肌炎、心包炎、肾炎、血小板减少、溶血性贫血、噬血细胞综合征及皮疹,尤其是Stevens-Johnson综合征。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "神经系统" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "血液系统" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "心血管系统" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "皮肤" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肌肉" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "关节" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "肺" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "脑膜脑炎" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "神经根神经炎" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "心肌炎" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "心包炎" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "肾炎" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "血小板减少" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 73, "end_idx": 79, "type": "dis", "entity": "噬血细胞综合征" }, { "start_idx": 81, "end_idx": 82, "type": "dis", "entity": "皮疹" }, { "start_idx": 87, "end_idx": 104, "type": "dis", "entity": "Stevens-Johnson综合征" } ] }, { "text": "多发生在呼吸道症状出现后10天左右。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "呼吸道" } ] }, { "text": "【实验室检查】X线胸部摄片多表现为单侧病变,大多数侵犯下叶,以右下叶为多,常呈淡薄片状或云雾状浸润,从肺门延伸至肺野,呈支气管肺炎的改变。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "ite", "entity": "X线胸部摄片" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "支气管肺炎" } ] }, { "text": "少数呈均匀的实变阴影,类似大叶性肺炎。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "大叶性肺炎" } ] }, { "text": "外周血白细胞计数大多正常,但也有白细胞减少或偏高者。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "外周血白细胞计数" } ] }, { "text": "部分患儿血清转氨酶、乳酸脱氢酶、碱性磷酸酶增高。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "mic", "entity": "血清转氨酶" }, { "start_idx": 10, "end_idx": 14, "type": "mic", "entity": "乳酸脱氢酶" }, { "start_idx": 16, "end_idx": 20, "type": "mic", "entity": "碱性磷酸酶" } ] }, { "text": "血清抗体可通过补体结合试验、间接血球凝集试验、酶联免疫吸附试验、间接免疫荧光试验等方法测定,或通过检测抗原得到早期诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "血清抗体" }, { "start_idx": 7, "end_idx": 12, "type": "ite", "entity": "补体结合试验" }, { "start_idx": 14, "end_idx": 21, "type": "ite", "entity": "间接血球凝集试验" }, { "start_idx": 23, "end_idx": 30, "type": "ite", "entity": "酶联免疫吸附试验" }, { "start_idx": 32, "end_idx": 39, "type": "ite", "entity": "间接免疫荧光试验" } ] }, { "text": "【诊断与鉴别诊断】根据以下临床特征可初步诊断:①多发年龄5~18岁;②咳嗽突出而持久;③肺部体征少而X线改变出现早且严重;④用青霉素无效,红霉素治疗效果好;⑤外周血白细胞计数正常或升高;⑥血清冷凝集阳性。", "entities": [ { "start_idx": 69, "end_idx": 71, "type": "dru", "entity": "红霉素" } ] }, { "text": "确诊必须靠呼吸道分泌物中检出MP及特异性抗体IgM检查阳性。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "MP" } ] }, { "text": "早期诊断法有ELISA法、单克隆抗体法检测MP抗原,特异IgM及PCR法检测DNA等。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "ite", "entity": "ELISA法" }, { "start_idx": 13, "end_idx": 24, "type": "ite", "entity": "单克隆抗体法检测MP抗原" }, { "start_idx": 26, "end_idx": 40, "type": "ite", "entity": "特异IgM及PCR法检测DNA" } ] }, { "text": "【治疗】首选大环内酯类抗生素如红霉素,疗程一般较长,不少于2周,停药过早易于复发。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "dru", "entity": "大环内酯类抗生素" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "红霉素" } ] }, { "text": "近年来研究表明新合成的大环内酯类抗生素阿奇霉素、克拉霉素等具有与红霉素同等的抗菌活性,而且耐受性较好。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "阿奇霉素" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "克拉霉素" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "红霉素" } ] }, { "text": "近年来有人认为重症MP肺炎的发病可能与人体免疫反应有关,因此,对急性期病情较重者,或肺部病变迁延而出现肺不张、肺间质纤维化,支气管扩张者,或有肺外并发症者,可应用肾上腺皮质激素口服或静脉用药,一般疗程为3~5天。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "重症MP肺炎" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "肺不张" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "肺间质纤维化" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "支气管扩张" }, { "start_idx": 81, "end_idx": 87, "type": "dru", "entity": "肾上腺皮质激素" } ] }, { "text": "参考文献1.秦梅,樊绍曾.亚低温和非氨酯对新生鼠缺氧缺血性脑损伤的保护作用.中华儿科杂志,2001,3(1):11-112.MhairiGMacDonald,MarthaDMullett,MaryMKSeshia.Neonatology,PathophysiologyandManagementoftheNewborn.6thed.LippincottWilliams&Wilkins,Phladelphia,20053.BattinMR,DezoeteJA,GunnTR,etal.Neuro-developmentaloutcomeofinfantstreatedwithheadcoolingandmildhypothermiaafterperinatalasphyxia.Pediatrics,2001,107(3):480-4844.BehrmanRE,KliegmanRM,JensonHB,etal.NelsonTextbookofPediatrics.17thed.SciencePress,Harcourt2.AsiaandSaunders,20045.DaniC,MartelliE,RubaltelliFF.ProphylaxisofgroupBbetahemolyticstreptococcalinfections.ActaBiomedAteneoParmense,2000,71(Suppl1):541-5416.HansenT.Guidelinesfortreatmentofneonataljaundice,Isthereaplaceforevidence-basedmedicine?", "entities": [ { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "缺氧缺血性脑损伤" }, { "start_idx": 40, "end_idx": 41, "type": "dep", "entity": "儿科" } ] }, { "text": 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"entities": [ { "start_idx": 1472, "end_idx": 1473, "type": "dep", "entity": "儿科" }, { "start_idx": 1757, "end_idx": 1761, "type": "dis", "entity": "新生儿窒息" }, { "start_idx": 1822, "end_idx": 1823, "type": "dis", "entity": "梅毒" }, { "start_idx": 1886, "end_idx": 1895, "type": "dis", "entity": "新生儿持续肺动脉高压" }, { "start_idx": 1903, "end_idx": 1904, "type": "dep", "entity": "儿科" } ] }, { "text": "第十四章病毒性脑膜炎、脑炎第一节中枢神经系统病毒感染概述中枢神经系统病毒感染的临床表现多种多样,以急性无菌性脑膜炎或脑炎最为常见。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "病毒性脑膜炎" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "脑炎" }, { "start_idx": 16, "end_idx": 25, "type": "dis", "entity": "中枢神经系统病毒感染" }, { "start_idx": 28, "end_idx": 37, "type": "dis", "entity": "中枢神经系统病毒感染" }, { "start_idx": 49, "end_idx": 56, "type": "dis", "entity": "急性无菌性脑膜炎" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "脑炎" } ] }, { "text": "可以引起中枢神经系统感染的病毒多种多样,常见者包括单纯疱疹病毒(herpessimplexvirus,HSV)、肠道病毒以及EB病毒(Epstein-Barrvirus,EBV)等。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 27, "end_idx": 30, "type": "mic", "entity": "疱疹病毒" }, { "start_idx": 32, "end_idx": 49, "type": "mic", "entity": "herpessimplexvirus" }, { "start_idx": 51, "end_idx": 53, "type": "mic", "entity": "HSV" }, { "start_idx": 56, "end_idx": 59, "type": "mic", "entity": "肠道病毒" }, { "start_idx": 62, "end_idx": 65, "type": "mic", "entity": "EB病毒" }, { "start_idx": 85, "end_idx": 87, "type": "mic", "entity": "EBV" } ] }, { "text": "除少数病毒外,中枢神经系统病毒感染的治疗缺乏特效方法。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "例如虫媒病毒感染好发于相应虫媒生活的地域和季节。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "虫媒病毒感染" } ] }, { "text": "多数中枢神经系统病毒感染累及所有年龄人群,无明显的季节或地域差异,例如单纯疱疹病毒性脑炎。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "中枢神经系统病毒感染" }, { "start_idx": 35, "end_idx": 43, "type": "dis", "entity": "单纯疱疹病毒性脑炎" } ] }, { "text": "我国近年来随着脊髓灰质炎病毒疫苗的强化接种,野病毒株感染者已经消失。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "dru", "entity": "脊髓灰质炎病毒疫苗" } ] }, { "text": "目前疱疹病毒、肠道病毒(除脊髓灰质炎病毒外)和呼吸道病毒(如腺病毒)等几类病毒感染占本病的大多数。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "mic", "entity": "疱疹病毒" }, { "start_idx": 7, "end_idx": 10, "type": "mic", "entity": "肠道病毒" }, { "start_idx": 23, "end_idx": 27, "type": "mic", "entity": "呼吸道病毒" }, { "start_idx": 30, "end_idx": 32, "type": "mic", "entity": "腺病毒" } ] }, { "text": "【临床分类】根据起病和病程特点,神经系统病毒感染一般可分为四类:急性、亚急性、慢性和胚胎脑病(表16-16)。", "entities": [ { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "神经系统病毒感染" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "急性" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "亚急性" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "慢性" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "胚胎脑病" } ] }, { "text": "首先根据核酸类型,再根据核酸分子量、结构、衣壳的对称型、壳粒数、包膜和病毒形态与大小等进一步分类。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "核酸" } ] }, { "text": "比病毒更小的传染因子被称为亚病毒(subvirus),包括类病毒(viroid)、拟病毒(virusoid)和朊病毒。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "mic", "entity": "亚病毒" }, { "start_idx": 29, "end_idx": 31, "type": "mic", "entity": "类病毒" }, { "start_idx": 41, "end_idx": 43, "type": "mic", "entity": "拟病毒" }, { "start_idx": 55, "end_idx": 57, "type": "mic", "entity": "朊病毒" } ] }, { "text": "其中朊病毒是1982年美国学者Prusiner首先报道的一种对核酸酶有抵抗性的感染性蛋白质,被称为朊蛋白(prion),是引起疯牛(羊)病和人类CJD及库鲁(Kuru)病等中枢神经系统慢感染(以前称为慢病毒感染)的致癌因子表16-9神经系统病毒感染的临床类型与疾病*由朊蛋白(prion)感染所致表16-10DNA病毒的分类【常见病毒所致神经系统感染的临床特点】不同病毒所致神经系统感染的临床特点有所差异(表16-16)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "朊病毒" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "核酸酶" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "朊蛋白" }, { "start_idx": 86, "end_idx": 91, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 86, "end_idx": 94, "type": "sym", "entity": "中枢神经系统慢感染" }, { "start_idx": 100, "end_idx": 104, "type": "dis", "entity": "慢病毒感染" }, { "start_idx": 140, "end_idx": 151, "type": "dis", "entity": "朊蛋白(prion)感染" }, { "start_idx": 160, "end_idx": 164, "type": "mic", "entity": "DNA病毒" }, { "start_idx": 175, "end_idx": 180, "type": "dis", "entity": "神经系统感染" }, { "start_idx": 193, "end_idx": 198, "type": "dis", "entity": "神经系统感染" } ] }, { "text": "表16-11RNA病毒的分类表16-12常见病毒与神经系统疾病+++,关系极密切;++,关系密切;+,有一定关系;?", "entities": [ { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "神经系统疾病" } ] }, { "text": "五、经导管关闭房间隔缺损及室间隔缺损房间隔缺损与室间缺损是常见的先天性心脏病,通常需要开胸体外转流下进行直视关闭术。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "经导管关闭房间隔缺损" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "室间缺损" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 43, "end_idx": 56, "type": "pro", "entity": "开胸体外转流下进行直视关闭术" } ] }, { "text": "虽然手术死亡率低,但仍有一定的并发症、切口瘢痕形成与术后远期影响等问题。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "手术" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "切口瘢痕形成" } ] }, { "text": "因此,经导管关闭房、室间隔缺损一直为先天性心脏病介入性治疗研究的热点。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "经导管关闭房、室间隔缺损" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "另外,随着先天性心脏病手术技术的进展,复杂型心脏病应用生理纠正术,如高危单心室病人作Fontan手术后有较高的并发症及死亡率,术后多死于低心排伴中心静脉压增高。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "先天性心脏病手术" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "复杂型心脏病" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "生理纠正术" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "高危单心室" }, { "start_idx": 42, "end_idx": 49, "type": "pro", "entity": "Fontan手术" }, { "start_idx": 68, "end_idx": 78, "type": "dis", "entity": "低心排伴中心静脉压增高" } ] }, { "text": "因此,设计开窗的Fontan手术,术时于左右心房之间的房间隔补片上留有直径4~6mm的小窗,术后使留有不同程度的心房水平右向左分流,以维持左心室负荷、心排量及限制中心静脉压增加。", "entities": [ { "start_idx": 5, "end_idx": 15, "type": "pro", "entity": "开窗的Fontan手术" }, { "start_idx": 20, "end_idx": 31, "type": "bod", "entity": "左右心房之间的房间隔补片" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "心房" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "左心室" }, { "start_idx": 75, "end_idx": 75, "type": "bod", "entity": "心" }, { "start_idx": 83, "end_idx": 84, "type": "bod", "entity": "静脉" } ] }, { "text": "一旦病人术后循环获得调整,数天或数月后用导管法关闭房间隔交通。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "导管法" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "房间隔" } ] }, { "text": "由于这些病人再次开胸手术较困难及危险性较大,而经导管关闭房间隔缺损为最好选择,因此促进了非开胸关闭的装置和技术的研究。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "开胸手术" }, { "start_idx": 23, "end_idx": 32, "type": "dis", "entity": "经导管关闭房间隔缺损" }, { "start_idx": 44, "end_idx": 48, "type": "pro", "entity": "非开胸关闭" } ] }, { "text": "经导管关闭术的指征仍在研究中。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "经导管关闭术" } ] }, { "text": "室间隔缺损的堵塞器安置较房间隔缺损困难,并发症亦较多,目前进行的病例还不多,尚缺乏中远期随访。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 6, "end_idx": 8, "type": "equ", "entity": "堵塞器" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "(一)经导管房间隔缺损关闭术1976年,King及Mille首次报道经静脉双伞堵塞装置关闭继发孔型房间隔缺损获得成功,但需7.7mm(23F)递送系统。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "pro", "entity": "经导管房间隔缺损关闭术" }, { "start_idx": 34, "end_idx": 53, "type": "pro", "entity": "经静脉双伞堵塞装置关闭继发孔型房间隔缺损" } ] }, { "text": "随后Rashkind等应用堵塞动脉导管未闭的双伞装置关闭房间隔缺损,先后经单面伞状带钩的堵塞装置,双伞无钩的堵塞器,但仍需3.33mm(10F)递送系统。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "动脉导管未闭" }, { "start_idx": 22, "end_idx": 25, "type": "equ", "entity": "双伞装置" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 37, "end_idx": 47, "type": "equ", "entity": "单面伞状带钩的堵塞装置" }, { "start_idx": 49, "end_idx": 56, "type": "equ", "entity": "双伞无钩的堵塞器" }, { "start_idx": 72, "end_idx": 75, "type": "equ", "entity": "递送系统" } ] }, { "text": "以后Lock等改进该装置,成为蚌壳状式闭合器,以适合房间隔缺损的需要,但关闭房间隔缺损的结果仍不尽满意。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "equ", "entity": "蚌壳状式闭合器" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "为此,Sideris等研制成纽扣式双盘堵塞装置,该装置较以上的堵塞器安置方便、安全,而且可使递送系统的直径减至2.67mm(8F),使该技术适合婴儿病人使用,但并发症仍较高,且残余分流率也高。", "entities": [ { "start_idx": 14, "end_idx": 22, "type": "equ", "entity": "纽扣式双盘堵塞装置" }, { "start_idx": 31, "end_idx": 33, "type": "equ", "entity": "堵塞器" }, { "start_idx": 46, "end_idx": 49, "type": "equ", "entity": "递送系统" }, { "start_idx": 88, "end_idx": 91, "type": "sym", "entity": "残余分流" } ] }, { "text": "1998年Amplatzer双盘自膨性房间隔堵塞装置(AGAmedicalcorporation)面世,由于其具有自膨性、自向心性、完全封堵率高、并发症少、操作方便、递送导管小等优点而迅速风靡全球。", "entities": [ { "start_idx": 14, "end_idx": 25, "type": "equ", "entity": "双盘自膨性房间隔堵塞装置" }, { "start_idx": 27, "end_idx": 47, "type": "equ", "entity": "AGAmedicalcorporation" }, { "start_idx": 85, "end_idx": 86, "type": "equ", "entity": "导管" } ] }, { "text": "作者所在单位的临床应用表明技术成功率达100%,即刻残余分流率<7%,随访1月分流全部消失,无并发症发生,因而该术值得推广,但其中长期疗效尚需进一步随访研究。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "残余分流" } ] }, { "text": "1.适应证及禁忌证(适用于Amplatzer双盘自膨性房间隔缺损堵塞装置)(1)适应证:①继发孔型房间隔缺损;②小儿病例通常直径≤30mm;③右室扩大有右室容量负荷增加的证据;④左向右分流;⑤缺损边缘至冠状窦、房室瓣和右上叶肺静脉的距离≥5mm。", "entities": [ { "start_idx": 13, "end_idx": 35, "type": "equ", "entity": "Amplatzer双盘自膨性房间隔缺损堵塞装置" }, { "start_idx": 45, "end_idx": 53, "type": "dis", "entity": "继发孔型房间隔缺损" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "右室扩大" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "右室" }, { "start_idx": 96, "end_idx": 121, "type": "sym", "entity": "缺损边缘至冠状窦、房室瓣和右上叶肺静脉的距离≥5mm" } ] }, { "text": "(2)禁忌证:①伴有需胸外科手术的先天性心脏畸形;②原发孔型房间隔缺损;③静脉窦型房间隔缺损;④肺静脉异位引流(完全性或部分性);⑤严重肺动脉高压/双向或右向左分流。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "胸外科手术" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "先天性心脏畸形" }, { "start_idx": 26, "end_idx": 34, "type": "dis", "entity": "原发孔型房间隔缺损" }, { "start_idx": 37, "end_idx": 45, "type": "dis", "entity": "静脉窦型房间隔缺损" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "肺静脉异位引流" }, { "start_idx": 66, "end_idx": 72, "type": "dis", "entity": "严重肺动脉高压" } ] }, { "text": "2.方法全麻下经皮股静脉穿刺,各心腔全套血流动力学检查,包括压力及血氧饱和度检测。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "全麻" }, { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "经皮股静脉穿刺" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "心腔" }, { "start_idx": 30, "end_idx": 39, "type": "ite", "entity": "压力及血氧饱和度检测" } ] }, { "text": "四腔位行右上肺静脉造影以确定房间隔缺损大小,并与食管超声结果相比较。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "pro", "entity": "四腔位行右上肺静脉造影" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "食管超声" } ] }, { "text": "然后用球囊测量导管跨房间隔缺损以稀释造影剂扩张球囊,球囊两侧二凹陷点间的距离为房间隔缺损伸展直径。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "equ", "entity": "球囊" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "导管跨房间隔缺损" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "造影剂" }, { "start_idx": 23, "end_idx": 24, "type": "equ", "entity": "球囊" }, { "start_idx": 26, "end_idx": 27, "type": "equ", "entity": "球囊" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "房间隔缺损" } ] }, { "text": "同时在透视及食管超声下测量。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "透视" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "食管超声" } ] }, { "text": "再以同样量造影剂在体外扩张球囊,用测量板测量轻度变形时的球囊直径。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "造影剂" }, { "start_idx": 13, "end_idx": 14, "type": "equ", "entity": "球囊" }, { "start_idx": 17, "end_idx": 19, "type": "equ", "entity": "测量板" }, { "start_idx": 28, "end_idx": 29, "type": "equ", "entity": "球囊" } ] }, { "text": "选择腰部直径等于或大于房间隔缺损伸展直径1~2mm的Amplatzer房间隔缺损封堵器。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "房间隔缺损" }, { "start_idx": 26, "end_idx": 42, "type": "equ", "entity": "Amplatzer房间隔缺损封堵器" } ] }, { "text": "装置经股静脉途径在长引导鞘内推送,在透视及食管超声监视下先在左心房内释放左侧盘,回撤系统,使腰部卡于房间隔缺损,然后释放右盘。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "股静脉" }, { "start_idx": 9, "end_idx": 12, "type": "equ", "entity": "长引导鞘" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "透视" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "食管超声" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "左心房" }, { "start_idx": 36, "end_idx": 38, "type": "equ", "entity": "左侧盘" }, { "start_idx": 42, "end_idx": 43, "type": "equ", "entity": "系统" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "腰部" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 60, "end_idx": 61, "type": "equ", "entity": "右盘" } ] }, { "text": "3.疗效评价由于该法设计合理,操作较稳定安全,根据大系列报道该堵塞装置技术成功率高达97%,一年完全堵塞率达94.9%,但远期随访,包括血流动力学、心功能等其结果尚待进一步评价。", "entities": [ { "start_idx": 31, "end_idx": 34, "type": "equ", "entity": "堵塞装置" }, { "start_idx": 68, "end_idx": 72, "type": "ite", "entity": "血流动力学" }, { "start_idx": 74, "end_idx": 76, "type": "ite", "entity": "心功能" } ] }, { "text": "4.并发症由于Amplatzer堵塞器操作方便、安全、成功率高,因此该法并发症较少。", "entities": [ { "start_idx": 7, "end_idx": 18, "type": "equ", "entity": "Amplatzer堵塞器" } ] }, { "text": "除术时有一过性心律失常外,偶有堵塞器脱落、心包填塞、二尖瓣关闭不全、局部血管并发症等。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "过性心律失常" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "堵塞器脱落" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "心包填塞" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "二尖瓣关闭不全" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "局部血管并发症" } ] }, { "text": "5.随访术前3天及术后3~6个月内口服阿司匹林3~5mg/(kg•d)预防血小板在堵塞器上过度凝聚。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "口服" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "血小板" }, { "start_idx": 41, "end_idx": 43, "type": "equ", "entity": "堵塞器" } ] }, { "text": "术前后常规抗生素静脉点滴以预防感染。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "抗生素" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "静脉点滴" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "感染" } ] }, { "text": "术后1月、3月、6月、1年门诊随访。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dep", "entity": "门诊" } ] }, { "text": "随访内容包括超声心动图、胸片、心电图及血常规等,以确定是否有残余分流、装置异位及溶血等并发症。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "超声心动图" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "胸片" }, { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "心电图" }, { "start_idx": 19, "end_idx": 21, "type": "ite", "entity": "血常规" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "残余分流" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "装置异位" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "溶血" } ] }, { "text": "(二)经导管室间隔缺损关闭术自1988年Lock等首先报道应用双伞堵塞装置经导管关闭室间隔缺损以来,目前已有多种室间隔缺损封堵装置问世,包括蚌壳状封堵装置、Rashkind动脉导管未闭封堵装置、纽扣式补片装置以及近期推出的Amplatzer室间隔缺损封堵装置等。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "pro", "entity": "经导管室间隔缺损关闭术" }, { "start_idx": 31, "end_idx": 36, "type": "equ", "entity": "双伞堵塞装置" }, { "start_idx": 37, "end_idx": 46, "type": "pro", "entity": "经导管关闭室间隔缺损" }, { "start_idx": 56, "end_idx": 64, "type": "equ", "entity": "室间隔缺损封堵装置" }, { "start_idx": 70, "end_idx": 76, "type": "equ", "entity": "蚌壳状封堵装置" }, { "start_idx": 78, "end_idx": 95, "type": "equ", "entity": "Rashkind动脉导管未闭封堵装置" }, { "start_idx": 97, "end_idx": 103, "type": "equ", "entity": "纽扣式补片装置" }, { "start_idx": 111, "end_idx": 128, "type": "equ", "entity": "Amplatzer室间隔缺损封堵装置" } ] }, { "text": "由于经导管堵塞技术的复杂性、效果不确切及经验有限,目前尚不能确定哪一种装置更优越,或是否经导管室间隔缺损关闭术优于外科手术。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "经导管堵塞技术" }, { "start_idx": 44, "end_idx": 54, "type": "pro", "entity": "经导管室间隔缺损关闭术" }, { "start_idx": 57, "end_idx": 58, "type": "dep", "entity": "外科" } ] }, { "text": "目前可经导管关闭的室间隔缺损的类型主要为肌部室间隔缺损及部分膜部室间隔缺损。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "经导管关闭" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "肌部室间隔缺损" }, { "start_idx": 28, "end_idx": 36, "type": "dis", "entity": "部分膜部室间隔缺损" } ] }, { "text": "第四节军团菌感染1976年美国费城暴发军团菌肺炎,军团菌肺炎是一种以肺部感染为主、伴全身多系统损害的急性传染病,可引起任何年龄的感染。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "军团菌感染" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "军团菌肺炎" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "军团菌肺炎" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "肺部感染" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "全身多系统损害" } ] }, { "text": "在儿童的肺部感染性疾病中也很重要。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "dis", "entity": "儿童的肺部感染性疾病" } ] }, { "text": "【病原与流行病学】军团菌是一种机会致病菌,有42个种,64个血清型,其中至少19个种与人类疾病有关。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "军团菌" } ] }, { "text": "与人类关系最为密切的为嗜肺军团菌种(Legionellapneumophile,Lp),已发现15个血清型(L1~L15)。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "嗜肺军团菌" }, { "start_idx": 18, "end_idx": 38, "type": "mic", "entity": "Legionellapneumophile" }, { "start_idx": 40, "end_idx": 41, "type": "mic", "entity": "Lp" } ] }, { "text": "军团菌为需氧革兰染色阴性杆菌,是一种人类单核细胞和巨噬细胞的兼性细胞内寄生菌,培养和分离困难。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "军团菌" }, { "start_idx": 4, "end_idx": 13, "type": "mic", "entity": "需氧革兰染色阴性杆菌" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "单核细胞" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "巨噬细胞" } ] }, { "text": "流行病学调查发现军团菌暴发与冷却塔、热水系统、温泉浴等水装置或花盆肥料有关。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "军团菌" } ] }, { "text": "儿童感染军团菌的危险因素有慢性疾患及应用免疫抑制剂。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "mic", "entity": "军团菌" } ] }, { "text": "新生儿军团菌病的高危因素有早产、先天性心脏病、支气管发育不良和接受激素治疗。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "新生儿军团菌病" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "早产" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "支气管" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "支气管发育不良" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "激素治疗" } ] }, { "text": "小儿军团菌肺炎病情相对较重。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "小儿军团菌肺炎" } ] }, { "text": "污染有军团菌的直径小于5μm的小颗粒气溶胶可直接穿入支气管和肺部造成感染。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "mic", "entity": "军团菌" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "支气管" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肺部" } ] }, { "text": "军团菌产生的有害物质可造成组织损伤:外膜蛋白MIP可促进吞噬细胞对细菌的摄入并破坏细胞的杀菌功能;Lp的外毒素有消化卵黄囊和灭活抗糜蛋白酶的作用;脂多糖Lps作为内毒素有利于细菌黏附宿主细胞,保护细菌免受细胞内酶的破坏,促进单核/吞噬细胞对细菌的摄入,阻止吞噬体与溶酶体的融合;磷酸酶和蛋白酶影响吞噬细胞的杀菌功能。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "军团菌" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "组织损伤" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "吞噬细胞" }, { "start_idx": 49, "end_idx": 54, "type": "mic", "entity": "Lp的外毒素" }, { "start_idx": 91, "end_idx": 94, "type": "bod", "entity": "宿主细胞" }, { "start_idx": 112, "end_idx": 118, "type": "bod", "entity": "单核/吞噬细胞" } ] }, { "text": "【病理改变】肺炎急性期为多灶性纤维素性化脓性炎症,分两型:Ⅰ型为急性纤维素性化脓性肺炎95%,以大量纤维素渗出,中性粒细胞崩解,细胞碎片及巨噬细胞为主;Ⅱ型为急性弥漫性肺泡损伤。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肺炎" }, { "start_idx": 12, "end_idx": 23, "type": "dis", "entity": "多灶性纤维素性化脓性炎症" }, { "start_idx": 32, "end_idx": 42, "type": "dis", "entity": "急性纤维素性化脓性肺炎" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "大量纤维素渗出" }, { "start_idx": 56, "end_idx": 60, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 56, "end_idx": 62, "type": "sym", "entity": "中性粒细胞崩解" }, { "start_idx": 69, "end_idx": 72, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 79, "end_idx": 87, "type": "dis", "entity": "急性弥漫性肺泡损伤" } ] }, { "text": "病变中可见肺泡上皮增生、脱屑及透明膜形成,严重者可致蜂窝肺。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "肺泡上皮" }, { "start_idx": 5, "end_idx": 13, "type": "sym", "entity": "肺泡上皮增生、脱屑" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "透明膜形成" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "蜂窝肺" } ] }, { "text": "急性后期为机化性肺炎。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "机化性肺炎" } ] }, { "text": "胸膜病变为浆液性、浆液纤维素性胸膜炎或化脓性胸膜炎。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胸膜" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "胸膜病变" }, { "start_idx": 5, "end_idx": 17, "type": "dis", "entity": "浆液性、浆液纤维素性胸膜炎" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "化脓性胸膜炎" } ] }, { "text": "开始有短暂不适,急起高热,体温最高可达40℃以上,伴寒战,干咳,全身肌肉酸痛明显,恶心、腹痛、腹泻等胃肠道症状显著。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "不适" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "急起高热" }, { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "体温最高可达40℃以上" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "寒战" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "干咳" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肌肉" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "肌肉酸痛" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "恶心" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "腹痛" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "腹泻" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "胃肠道" }, { "start_idx": 50, "end_idx": 54, "type": "sym", "entity": "胃肠道症状" } ] }, { "text": "军团菌肺炎临床表现复杂,病情程度差异悬殊,轻者无明显临床症状,重者可引起以肺部感染为主的多器官损害,如ARDS、急性肾衰竭、休克和弥漫性血管内凝血。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "军团菌肺炎" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "肺部" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "肺部感染" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "多器官损害" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "ARDS" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "休克" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "血管" }, { "start_idx": 65, "end_idx": 72, "type": "sym", "entity": "弥漫性血管内凝血" } ] }, { "text": "常有以下较为特殊的临床表现:①与高热不相称的相对缓脉;②剧烈胸痛,在成人易误诊为肺栓塞;③胃肠道症状显著,为水样便,呈无菌性腹泻;④电解质紊乱,可合并低钠血症、低钾血症,此项表现有助于诊断和鉴别诊断;⑤多汗症状突出。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "高热" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "缓脉" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "剧烈胸痛" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "肺栓塞" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "胃肠道" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "胃肠道症状" }, { "start_idx": 54, "end_idx": 56, "type": "sym", "entity": "水样便" }, { "start_idx": 59, "end_idx": 63, "type": "sym", "entity": "无菌性腹泻" }, { "start_idx": 66, "end_idx": 70, "type": "sym", "entity": "电解质紊乱" }, { "start_idx": 75, "end_idx": 78, "type": "dis", "entity": "低钠血症" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "低钾血症" }, { "start_idx": 101, "end_idx": 102, "type": "sym", "entity": "多汗" } ] }, { "text": "【影像学检查】X线的表现呈多样性、缺乏特异性,可见斑片影、纱网影、边缘清楚的小结节影、条索影、弥漫肺浸润影等,胸腔积液相对多见。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "X线" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "斑片影" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "纱网影" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "边缘清楚的小结节影" }, { "start_idx": 43, "end_idx": 45, "type": "sym", "entity": "条索影" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "弥漫肺浸润影" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "胸腔" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "胸腔积液" } ] }, { "text": "肺CT可发现早期病灶。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "肺CT" } ] }, { "text": "吸收较慢,有人认为军团菌肺炎的吸收是最慢的。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "军团菌肺炎" } ] }, { "text": "【诊断】根据国内1992年制定的军团菌肺炎诊断标准:①临床表现如发热、寒战、咳嗽、胸痛等呼吸道感染症状;②X线胸片具有炎性阴影;③呼吸道分泌物、痰、血或胸水在活性炭母浸液琼脂培养基BCYE或其他特殊培养基上培养有军团菌生长;④呼吸道分泌物直接荧光法DFA检查阳性;⑤血间接荧光法IFA检查前后2次抗体滴度呈4倍或以上增高,达1∶128或以上;血试管凝集实验TAT检查前后2次抗体滴度呈4倍或以上增高,达1∶160或以上;血微量凝集实验MAA检查前后2次抗体滴度呈4倍或以上增高,达1∶64或以上。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "军团菌肺炎" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "发热" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "寒战" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "咳嗽" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "胸痛" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "呼吸道" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "呼吸道感染症状" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "X线胸片" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "炎性阴影" }, { "start_idx": 65, "end_idx": 70, "type": "bod", "entity": "呼吸道分泌物" }, { "start_idx": 72, "end_idx": 72, "type": "bod", "entity": "痰" }, { "start_idx": 74, "end_idx": 74, "type": "bod", "entity": "血" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "胸水" }, { "start_idx": 79, "end_idx": 89, "type": "equ", "entity": "活性炭母浸液琼脂培养基" }, { "start_idx": 106, "end_idx": 108, "type": "mic", "entity": "军团菌" }, { "start_idx": 113, "end_idx": 118, "type": "bod", "entity": "呼吸道分泌物" }, { "start_idx": 121, "end_idx": 128, "type": "pro", "entity": "荧光法DFA检查" }, { "start_idx": 133, "end_idx": 143, "type": "pro", "entity": "血间接荧光法IFA检查" }, { "start_idx": 171, "end_idx": 182, "type": "pro", "entity": "血试管凝集实验TAT检查" }, { "start_idx": 210, "end_idx": 219, "type": "pro", "entity": "血微量凝集实验MAA" } ] }, { "text": "凡具有以上①、②项,同时具有③、④、⑤项中任何一项者,即可诊断为军团菌肺炎。", "entities": [ { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "军团菌肺炎" } ] }, { "text": "对于IFA或TAT效价有一次增高,IFA=1∶256或TAT=1∶320,同时有临床及X线胸片炎症表现的病例可考虑为可疑军团菌肺炎。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "IFA" }, { "start_idx": 6, "end_idx": 8, "type": "ite", "entity": "TAT" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "IFA" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "TAT" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "X线胸片" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "炎症" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "军团菌肺炎" } ] }, { "text": "【治疗】(1)抗菌治疗。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "抗菌治疗" } ] }, { "text": "首选红霉素,30~50mg/(kg•d),14~21天,静脉滴注或口服分4次,疗程3周;或阿奇霉素10mg/(kg•d),3~5天,或联用利福平。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "红霉素" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "口服" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "阿奇霉素" }, { "start_idx": 69, "end_idx": 71, "type": "dru", "entity": "利福平" } ] }, { "text": "危重病例必要时可考虑选用喹诺酮类抗生素。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dru", "entity": "喹诺酮类抗生素" } ] }, { "text": "(2)对症治疗与支持治疗。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "支持治疗" } ] }, { "text": "重症军团菌病往往出现进行性呼吸困难致呼吸衰竭、肾衰竭、感染性休克、DIC和脑病。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "重症军团菌病" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "进行性呼吸困难" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "感染性休克" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "DIC" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "脑病" } ] }, { "text": "这些病往往是军团菌病死亡的重要原因,应积极防治。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "军团菌病" } ] }, { "text": "第四节气道反应性气道高反应性(BHR)是指气道对外界特异性或非特异性刺激过于强烈的反应,是儿童支气管哮喘的主要病理生理特征。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "气道反应性" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "气道高反应性" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "BHR" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "气道" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "支气管哮喘" } ] }, { "text": "气道反应性测定对儿童不典型哮喘、咳嗽变异型哮喘的诊断,以及支气管哮喘患儿用药治疗期间的疗效判断和何时停药显得格外重要。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "气道反应性" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "不典型哮喘" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "咳嗽变异型哮喘" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "支气管哮喘" } ] }, { "text": "一、支气管激发试验分为直接激发试验和间接激发试验,前者主要选用外源性非选择性直接激发剂,如组胺、乙酰甲胆碱。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "ite", "entity": "支气管激发试验" }, { "start_idx": 11, "end_idx": 16, "type": "ite", "entity": "直接激发试验" }, { "start_idx": 18, "end_idx": 23, "type": "ite", "entity": "间接激发试验" }, { "start_idx": 31, "end_idx": 42, "type": "dru", "entity": "外源性非选择性直接激发剂" }, { "start_idx": 45, "end_idx": 46, "type": "dru", "entity": "组胺" }, { "start_idx": 48, "end_idx": 52, "type": "dru", "entity": "乙酰甲胆碱" } ] }, { "text": "间接激发试验主要通过刺激支气管内炎性细胞使其释放多种能间接引起支气管狭窄的介质,作用于支气管平滑肌上特异性受体而引起气道收缩。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "间接激发试验" }, { "start_idx": 12, "end_idx": 19, "type": "bod", "entity": "支气管内炎性细胞" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "支气管" }, { "start_idx": 43, "end_idx": 48, "type": "bod", "entity": "支气管平滑肌" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "气道" } ] }, { "text": "目前间接激发试验不论在成人还是儿童应用都很少,尤其是儿童尚没有规范的量化标准。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "ite", "entity": "间接激发试验" } ] }, { "text": "而且特异性抗原刺激危险性较大,可诱发严重的哮喘。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "哮喘" } ] }, { "text": "目前大多数支气管激发试验选用前者。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "ite", "entity": "支气管激发试验" } ] }, { "text": "直接支气管激发试验检测方法目前主要有两种:1.用常规通气方法以吸药前的FEV1做对照,间歇吸入不同浓度(或相同浓度)的乙酰甲胆碱或组胺,每吸入1次,检测当时的FEV1。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "ite", "entity": "直接支气管激发试验" }, { "start_idx": 35, "end_idx": 49, "type": "ite", "entity": "FEV1" }, { "start_idx": 70, "end_idx": 74, "type": "dru", "entity": "乙酰甲胆碱" }, { "start_idx": 76, "end_idx": 77, "type": "dru", "entity": "组胺" }, { "start_idx": 90, "end_idx": 104, "type": "ite", "entity": "FEV1" } ] }, { "text": "一般以FEV1较对照值下降20%的最低累积剂量(PD20-FEV1)或最低累积浓度(PC20-FEV1)为试验的反应阈值,当FEV1下降大于20%对照值或基础值时为激发试验阳性。", "entities": [ { "start_idx": 3, "end_idx": 17, "type": "ite", "entity": "FEV1" }, { "start_idx": 95, "end_idx": 109, "type": "ite", "entity": "FEV1" } ] }, { "text": "2.连续呼吸的Astograph法原理是应用强迫振荡技术进行检测,在连续吸入不同浓度的乙酰甲胆碱时,不断监测呼吸阻力。", "entities": [ { "start_idx": 22, "end_idx": 27, "type": "ite", "entity": "强迫振荡技术" }, { "start_idx": 43, "end_idx": 47, "type": "dru", "entity": "乙酰甲胆碱" } ] }, { "text": "整个雾化系统包括12个雾化罐,第1罐为生理盐水,第12罐为支气管扩张剂,第2~11罐为浓度依次递增(49~25000μg/mL)的乙酰甲胆碱。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "equ", "entity": "雾化罐" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "生理盐水" }, { "start_idx": 29, "end_idx": 34, "type": "dru", "entity": "支气管扩张剂" }, { "start_idx": 65, "end_idx": 69, "type": "dru", "entity": "乙酰甲胆碱" } ] }, { "text": "以吸入生理盐水时的阻力为基础阻力,当呼吸阻力增加到基础阻力的2倍时,改为吸入支气管扩张剂。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "生理盐水" }, { "start_idx": 38, "end_idx": 43, "type": "dru", "entity": "支气管扩张剂" } ] }, { "text": "最后按照所测出的气道反应阈值得出诊断结果。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "气道" } ] }, { "text": "3.注意事项(1)检查前须停药:β受体兴奋剂(沙丁胺醇等)应停用12小时以上,缓释型停用24小时以上;甲基黄嘌呤类(茶碱)普通型停用12小时以上,缓释型停用24小时以上,抗胆碱能类药(异丙溴托胺等)停用12小时以上;抗组织胺类药停用48小时以上;糖皮质激素停用12小时以上,另外避免吸烟、咖啡、可乐饮料等6小时以上;(2)检测前常规肺功能:FEV1>预计值70%以上。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "dru", "entity": "β受体兴奋剂" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "沙丁胺醇" }, { "start_idx": 51, "end_idx": 56, "type": "dru", "entity": "甲基黄嘌呤类" }, { "start_idx": 58, "end_idx": 59, "type": "dru", "entity": "茶碱" }, { "start_idx": 85, "end_idx": 90, "type": "dru", "entity": "抗胆碱能类药" }, { "start_idx": 92, "end_idx": 96, "type": "dru", "entity": "异丙溴托胺" }, { "start_idx": 108, "end_idx": 113, "type": "dru", "entity": "抗组织胺类药" }, { "start_idx": 123, "end_idx": 127, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 164, "end_idx": 168, "type": "ite", "entity": "常规肺功能" }, { "start_idx": 170, "end_idx": 184, "type": "ite", "entity": "FEV1" } ] }, { "text": "(3)检测时监测:激发试验可能会诱发喘息,在检测过程中须加强肺部听诊,准备好支气管舒张剂和抢救设备。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "激发试验" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "喘息" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "肺部听诊" }, { "start_idx": 38, "end_idx": 43, "type": "dru", "entity": "支气管舒张剂" } ] }, { "text": "在检测结束时须给予支气管舒张剂吸入,待患儿常规肺功能恢复FEV1>预计值70%以上,方可以回家。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "支气管舒张剂" }, { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "常规肺功能" }, { "start_idx": 28, "end_idx": 42, "type": "ite", "entity": "FEV1" } ] }, { "text": "第三节右室双出口右室双出口(doubleoutletsofrightventricle)是指当两根大血管完全或接近完全起自右心室,占出生婴儿的0.032‰。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "右室双出口" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "右室双出口" }, { "start_idx": 14, "end_idx": 42, "type": "dis", "entity": "doubleoutletsofrightventricle" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "大血管" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "右心室" } ] }, { "text": "因此,右室双出口可与双心室、单心室或任何大血管相互关系及任何房室连接类型同时存在。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "右室双出口" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "双心室" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "单心室" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "大血管" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "房室" } ] }, { "text": "【病理解剖】右室双出口不是一种单一的先天性心脏畸形,该术语仅仅用于描述各种心脏畸形时大血管的位置。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "右室双出口" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "先天性心脏畸形" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "心脏畸形" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "血管" } ] }, { "text": "尽管这种心室大血管连接可合并任何类型的心脏节段连接类型,但绝大多数为心房正位,房室连接一致。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "心室" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血管" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心脏" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "心房" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "房室" } ] }, { "text": "大血管相互关系常见的类型有:①主、肺动脉瓣相对关系正常,主动脉瓣位于肺动脉的右后方,此型最多见;②大动脉总干相互平行,主动脉瓣位于右侧,即所谓“Taussing-Bing畸形”;③大动脉总干相互平行,主动脉瓣位于左侧,此型最少见。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "大血管" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "主、肺动脉瓣相对关系正常" }, { "start_idx": 28, "end_idx": 40, "type": "sym", "entity": "主动脉瓣位于肺动脉的右后方" }, { "start_idx": 49, "end_idx": 53, "type": "bod", "entity": "大动脉总干" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 72, "end_idx": 86, "type": "dis", "entity": "Taussing-Bing畸形" }, { "start_idx": 90, "end_idx": 94, "type": "bod", "entity": "大动脉总干" }, { "start_idx": 100, "end_idx": 103, "type": "bod", "entity": "主动脉瓣" } ] }, { "text": "也有两根大血管有螺旋交叉而主动脉位于肺动脉的左后方者。", "entities": [ { "start_idx": 2, "end_idx": 24, "type": "sym", "entity": "两根大血管有螺旋交叉而主动脉位于肺动脉的左后方" } ] }, { "text": "但近30%的右室双出口主动脉瓣位于右侧且与肺动脉瓣在同一水平,近54%的右室双出口主动脉瓣位于肺动脉瓣的右前方。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "右室双出口" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺动脉" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "右室双出口" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "肺动脉瓣" } ] }, { "text": "右室双出口最常见且被广泛接受的分型方法是依据室间隔缺损的位置分为四种类型,即:①伴有主动脉下室间隔缺损的右室双出口;②伴有肺动脉下室间隔缺损的右室双出口;③伴有双动脉下室间隔缺损的右室双出口;④伴有远离大动脉室间隔缺损的右室双出口。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "右室双出口" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "主动脉" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "右室双出口" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "肺动脉" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 71, "end_idx": 75, "type": "dis", "entity": "右室双出口" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "双动脉" }, { "start_idx": 84, "end_idx": 88, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 90, "end_idx": 94, "type": "dis", "entity": "右室双出口" }, { "start_idx": 101, "end_idx": 103, "type": "bod", "entity": "大动脉" }, { "start_idx": 104, "end_idx": 108, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 110, "end_idx": 114, "type": "dis", "entity": "右室双出口" } ] }, { "text": "在临床上的分类为:①伴主动脉下室间隔缺损,主动脉瓣位于肺动脉右侧及右室流出道狭窄(法四型右室双出口);②伴肺动脉下室间隔缺损,主动脉瓣位于肺动脉右侧;③伴主动脉下室间隔缺损,主动脉瓣位于肺动脉右侧,不伴有右心室流出道狭窄的右室双出口较常见。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "主动脉" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肺动脉" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "流出道" }, { "start_idx": 41, "end_idx": 48, "type": "dis", "entity": "法四型右室双出口" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "肺动脉" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "肺动脉" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "主动脉" }, { "start_idx": 81, "end_idx": 85, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 87, "end_idx": 89, "type": "bod", "entity": "主动脉" }, { "start_idx": 93, "end_idx": 95, "type": "bod", "entity": "肺动脉" }, { "start_idx": 102, "end_idx": 104, "type": "bod", "entity": "右心室" }, { "start_idx": 105, "end_idx": 107, "type": "bod", "entity": "流出道" }, { "start_idx": 111, "end_idx": 115, "type": "dis", "entity": "右室双出口" } ] }, { "text": "从病理解剖学上,无论室间隔缺损为何种类型,室间隔缺损的位置几乎是固定的,即位于隔束的两个分叉之间,而室间隔缺损位置的变化取决于大血管本身的排列、相互关系和流出道室间隔的大小。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "血管" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "流出道" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "室间隔" } ] }, { "text": "据此,在右室双出口的心脏,肌部室间隔仅有流入道及小梁部。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "右室双出口" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心脏" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肌部" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "室间隔" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "流入道" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "小梁部" } ] }, { "text": "当两根大血管均发自右心室,流出道室间隔(圆锥隔或漏斗部)完全是右心室内的结构。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "血管" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "右心室" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "流出道" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "室间隔" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "右心室" } ] }, { "text": "仅有的例外是当室间隔缺损是肌部流入道或心尖小梁部,或膜周流入道向后主要向流入道延伸,即形成远离大动脉型的室间隔缺损。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "肌部流入道" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "心尖小梁部" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "膜周流入道" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "流入道" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "大动脉" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "漏斗部的形态也有许多类型,大动脉瓣近端与室间隔缺损顶部的形态取决于心室漏斗部褶。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "大动脉瓣" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "心室" } ] }, { "text": "当两根大血管的空间关系基本正常的情况下,两根大血管短轴连线与室间隔是平行的;此时的流出道相对与室间隔是向右成角且流出道室隔几乎都是与隔束的前臂融合,此时,位于隔束的两臂之间的室间隔缺损位于主动脉下。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "血管" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "血管" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "室间隔" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "流出道" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "室间隔" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "流出道" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "前臂" }, { "start_idx": 83, "end_idx": 83, "type": "bod", "entity": "臂" }, { "start_idx": 87, "end_idx": 91, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "主动脉" } ] }, { "text": "在极少见的情况下,流出道室间隔明显发育不良甚至缺如时,位于隔束两臂间的室间隔缺损即为双动脉下。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "流出道" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "室间隔" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "发育不良" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "双动脉下" } ] }, { "text": "因此,在两根大血管的相互关系基本正常的情况下,室间隔缺损绝大多数是主动脉下,有时是双动脉下,较少见的有远离大动脉型。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血管" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "主动脉" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "双动脉" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "大动脉" } ] }, { "text": "至今尚未见到大动脉位置关系正常而室间隔缺损为肺动脉下的类型。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "大动脉" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肺动脉" } ] }, { "text": "当两根大血管基本平行且主动脉位于右侧时(Taussing-Bing畸形),两根大血管短轴连线与三尖瓣与动脉瓣间的心室漏斗部褶平行,仅有左位的肺动脉靠近室间隔缺损。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "血管" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "主动脉" }, { "start_idx": 20, "end_idx": 34, "type": "dis", "entity": "Taussing-Bing畸形" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "血管" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "三尖" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "动脉" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "心室" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "肺动脉" }, { "start_idx": 76, "end_idx": 79, "type": "dis", "entity": "间隔缺损" } ] }, { "text": "因此,发育良好的流出道间隔与心室漏斗部褶融合形成了肺动脉下室间隔缺损,在很少情况下,流出道室间隔明显发育不良甚至缺如时,室间隔缺损即为双动脉下。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "流出道" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心室" }, { "start_idx": 25, "end_idx": 33, "type": "dis", "entity": "肺动脉下室间隔缺损" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "流出道" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "双动脉" } ] }, { "text": "在双动脉下室间隔缺损时,有可能两根大血管都有骑跨,Brandt等称之为“双心室双出口”伴左位主动脉,流出道室隔亦可与隔束的前支融合,因此可以有室间隔缺损与右后位的肺动脉相通。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "dis", "entity": "双动脉下室间隔缺损" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "血管" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "双心室双出口" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "主动脉" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "流出道室隔" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "隔束" }, { "start_idx": 71, "end_idx": 75, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "肺动脉" } ] }, { "text": "室间隔缺损的底部边界通常由隔束的两个支组成;左右边界可以是流出道间隔、心室游离壁或心室漏斗部褶,主要取决于室间隔缺损本身为主动脉下、肺动脉下或双动脉下。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "隔束" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "流出道" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "心室游离壁" }, { "start_idx": 41, "end_idx": 46, "type": "bod", "entity": "心室漏斗部褶" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "主动脉下" }, { "start_idx": 66, "end_idx": 69, "type": "bod", "entity": "肺动脉下" }, { "start_idx": 71, "end_idx": 74, "type": "bod", "entity": "双动脉下" } ] }, { "text": "同时心室漏斗部褶本身亦可影响右室双出口的类型。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心室" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "漏斗部褶" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "右室双出口" } ] }, { "text": "当该心室漏斗部褶与隔束的后支融合时,室间隔缺损即有了后下边缘,此时对外科医生非常重要,因为该肌束内有传导束并起保护作用。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心室" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "漏斗部褶" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "隔束" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 34, "end_idx": 35, "type": "dep", "entity": "外科" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "肌束" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "传导束" } ] }, { "text": "相反,当后支未向心室-漏斗部褶延伸时,形成二、三尖瓣纤维连接,此时的室间隔缺损为膜周型,传导束通常多在缺损的后下边缘走行。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心室" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "传导束" } ] }, { "text": "但有一点可以用于区分,即是否在大动脉与房室瓣间有漏斗部肌性分隔。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "大动脉" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "房室" } ] }, { "text": "同样,当右室双出口伴有肺动脉瓣下室间隔缺损造成左心室血流直接进入肺动脉时,无论从血流动力学或解剖上均与完全性大血管转位相似,鉴别点是二尖瓣肺动脉瓣的纤维连接,如果存在纤维连接即为完全性大血管转位。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "右室双出口" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "左心室" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "肺动脉" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "血管" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 69, "end_idx": 72, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 93, "end_idx": 94, "type": "bod", "entity": "血管" } ] }, { "text": "同时,右室双出口合并心脾综合征并不少见,无脾综合征(双右心房结构)绝大多数合并有完全性肺静脉异位引流,多脾综合征(双左心房结构)常合并有体静脉的回流异常、特别是下腔静脉的奇静脉延续和共同房室瓣。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "右室双出口合并心脾综合征" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "无脾综合征" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "肺静脉异位" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "多脾综合征" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "体静脉" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "下腔静脉" }, { "start_idx": 85, "end_idx": 87, "type": "dis", "entity": "奇静脉" } ] }, { "text": "而且还要注意窦房结异位。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "窦房结异位" } ] }, { "text": "【病理生理】室间隔缺损与大血管的相对关系、有无合并流出道狭窄及体肺循环的相对阻力决定了患儿的血流动力学状态。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "血管" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "流出道" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "体肺循环" } ] }, { "text": "其血流动力学可类似于室间隔缺损、右心室型单心室、法洛四联症或完全性大血管转位。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "血流" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "右心室型单心室" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "法洛四联症" } ] }, { "text": "可有青紫、充血性心力衰竭或两者同时存在。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "青紫、充血性心力衰竭" } ] }, { "text": "几乎所有的患儿均有不同程度的低氧血症。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "低氧血症" } ] }, { "text": "(一)室间隔缺损位置与氧饱和度关系:所有伴有肺动脉下室间隔缺损的患儿,无论伴或不伴有肺动脉狭窄或肺血管阻塞性疾病,肺循环氧饱和度均高于体循环氧饱和度。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 11, "end_idx": 14, "type": "ite", "entity": "氧饱和度" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 48, "end_idx": 55, "type": "dis", "entity": "肺血管阻塞性疾病" }, { "start_idx": 57, "end_idx": 63, "type": "ite", "entity": "肺循环氧饱和度" }, { "start_idx": 67, "end_idx": 73, "type": "ite", "entity": "体循环氧饱和度" } ] }, { "text": "伴主动脉下室间隔缺损时,约60%的患儿体循环氧饱和度高于肺循环,伴或不伴有肺动脉狭窄或肺血管阻塞性疾病并不一定影响其氧饱和度变化。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 19, "end_idx": 25, "type": "ite", "entity": "体循环氧饱和度" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "肺循环" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 43, "end_idx": 50, "type": "dis", "entity": "肺血管阻塞性疾病" }, { "start_idx": 58, "end_idx": 61, "type": "ite", "entity": "氧饱和度" } ] }, { "text": "伴双动脉下室间隔缺损时,肺循环氧饱和度多高于体循环氧饱和度。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "双动脉" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 12, "end_idx": 18, "type": "ite", "entity": "肺循环氧饱和度" }, { "start_idx": 22, "end_idx": 28, "type": "ite", "entity": "体循环氧饱和度" } ] }, { "text": "而伴远离大动脉的室间隔缺损时,体、肺循环氧饱和度差异较大。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "大动脉" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 15, "end_idx": 23, "type": "ite", "entity": "体、肺循环氧饱和度" } ] }, { "text": "(二)心室、动脉压力由于主动脉发自右心室,右心室压力接近体循环。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心室" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "动脉" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "主动脉" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "右心室" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "右心室" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "体循环" } ] }, { "text": "当存在肺动脉瓣上或瓣下狭窄时,肺动脉压力将明显降低。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "肺动脉瓣上或瓣下狭窄" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肺动脉" } ] }, { "text": "【临床表现】患儿可有青紫、充血性心力衰竭的症状,亦可毫无症状。", "entities": [ { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "青紫、充血性心力衰竭" } ] }, { "text": "其他类型的右室双出口体肺循环平衡良好,往往在新生儿期后才逐渐出现青紫或缺氧发作。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "右室双出口" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "体肺循环" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "青紫" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "缺氧" } ] }, { "text": "伴肺动脉下室间隔缺损的右室双出口常表现为安静时轻度青紫,哭吵后青紫加剧。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "肺动脉" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "右室双出口" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "青紫" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "青紫" } ] }, { "text": "右室双出口无特异性的体征。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "右室双出口" } ] }, { "text": "(一)主动脉下室间隔缺损伴肺动脉狭窄临床表现与法洛四联症相似。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "主动脉下室间隔缺损" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "法洛四联症" } ] }, { "text": "第一心音正常,第二心音单一,有时在心尖区可及第三心音。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "第一心音正常" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "第二心音单一" }, { "start_idx": 14, "end_idx": 25, "type": "sym", "entity": "有时在心尖区可及第三心音" } ] }, { "text": "(二)肺动脉下室间隔缺损伴或不伴有肺动脉狭窄临床表现与完全性大血管转位伴室间隔缺损相似。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "肺动脉下室间隔缺损" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "大血管转位" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "伴室间隔缺损" } ] }, { "text": "通常在婴儿期即表现为青紫和充血性心力衰竭。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "青紫" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "充血性心力衰竭" } ] }, { "text": "如伴有肺动脉狭窄时,青紫出现时间更早但常无心力衰竭表现。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "青紫" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "心力衰竭" } ] }, { "text": "肺血流量增加者可在心尖部闻及滚动样舒张期杂音。", "entities": [ { "start_idx": 0, "end_idx": 21, "type": "sym", "entity": "肺血流量增加者可在心尖部闻及滚动样舒张期杂音" } ] }, { "text": "(三)主动脉下室间隔缺损不伴肺动脉狭窄临床表现与大型室间隔缺损伴肺动脉高压类似。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "主动脉下室间隔缺损" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "肺动脉高压" } ] }, { "text": "通常青紫轻微但气急和充血性心力衰竭表现明显,有反复的上呼吸道感染。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "青紫" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "气急" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "充血性心力衰竭" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "上呼吸道感染" } ] }, { "text": "(四)主动脉下室间隔缺损伴肺血管阻塞性疾病如上组类型伴有器质性肺高压时,此时肺动脉血流减少,心功能衰竭及反复呼吸道感染少见。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "主动脉下室间隔缺损" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "肺血管阻塞性疾病" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "器质性肺高压" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "肺动脉" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "心功能衰竭" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "反复呼吸道感染" } ] }, { "text": "可出现青紫和杵状指(趾)。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "青紫" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "杵状指(趾)" } ] }, { "text": "体格检查常无杂音闻及,第二心音响亮而单一,同时可及肺动脉反流所致的舒张期杂音。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "体格检查常无杂音闻及" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "第二心音响亮而单一" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肺动脉" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "舒张期杂音" } ] }, { "text": "【辅助检查】(一)心电图常表现为窦性心律、电轴右偏及不同程度的右心室肥大,右胸前导联QRS波常表现为qR型。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "心电图" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "窦性心律" }, { "start_idx": 21, "end_idx": 35, "type": "sym", "entity": "电轴右偏及不同程度的右心室肥大" }, { "start_idx": 37, "end_idx": 52, "type": "sym", "entity": "右胸前导联QRS波常表现为qR型" } ] }, { "text": "左心室常不扩大,如果有明显的左心室大则提示有可能合并限制性的室间隔缺损。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "左心室常不扩大" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "(二)胸部X线无特征性的改变,心脏大小及肺血多少取决于当时的血流动力学状态,可为小心脏,肺血减少,也可表现为大心脏,肺血明显增多。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "胸部X线" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心脏" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肺血" }, { "start_idx": 40, "end_idx": 42, "type": "sym", "entity": "小心脏" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "肺血减少" }, { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "肺血明显增多" } ] }, { "text": "(三)超声心动图剑突下扫查对诊断右室双出口非常有用(图9-9),诊断标准为两根大血管全部或大部分发自右心室,双圆锥常见但并不是诊断的必须条件。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "剑突" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "右室双出口" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "血管" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "右心室" } ] }, { "text": "胸骨旁长轴及短轴可检测大血管的相互关系及动脉下圆锥。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "胸骨旁长轴及短轴" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "血管" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "动脉" } ] }, { "text": "由于右心室双出口的解剖类型变化较多且伴发畸形多样,必须进行全面的循序分段诊断。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "右心室双出口" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "畸形" } ] }, { "text": "(四)心导管造影在心外手术以前,通常需要通过心导管及造影证实超声心动图诊断并获得详细的血流动力学信息。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "心导管造影" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "心外手术" }, { "start_idx": 22, "end_idx": 24, "type": "equ", "entity": "心导管" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "造影" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "超声心动图" } ] }, { "text": "其血流动力学表现可类似于大分流量的左向右分流、右心室型单心室、法洛四联症、完全性大血管转位及肺动脉狭窄。", "entities": [ { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "右心室型单心室" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 37, "end_idx": 44, "type": "dis", "entity": "完全性大血管转位" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "肺动脉狭窄" } ] }, { "text": "超声心动图所获得的信息可用于选择合适的透照位置以明确大血管的起源及室间隔缺损的位置。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "超声心动图" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血管" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "由于大动脉多为侧侧关系且半月瓣在同一水平,采用双向DSA行前后位及侧位投照较为理想(图9-9)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "大动脉" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "半月瓣" } ] }, { "text": "图9-30右室双出口经剑突下超声心动图主动脉、肺动脉分别位于右、左侧,半月瓣位于同一水平,均发自右心室,可见双圆锥,右心室肥厚明显图9-31右室双出口右心室造影主肺动脉均发自右心室,主动脉瓣位于肺动脉瓣的右前方且与肺动脉瓣在同一水平(A.右心室前后位造影;B.右心室侧位造影)【治疗】在治疗以前必须考虑以下几点:双心室修补是否可能?", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "右室双出口" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "超声心动图" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "主动脉" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "肺动脉" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "半月瓣" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "右心室" }, { "start_idx": 54, "end_idx": 56, "type": "sym", "entity": "双圆锥" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "右心室肥厚明显" }, { "start_idx": 70, "end_idx": 74, "type": "dis", "entity": "右室双出口" }, { "start_idx": 75, "end_idx": 79, "type": "pro", "entity": "右心室造影" }, { "start_idx": 80, "end_idx": 83, "type": "bod", "entity": "主肺动脉" }, { "start_idx": 87, "end_idx": 89, "type": "bod", "entity": "右心室" }, { "start_idx": 91, "end_idx": 94, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 97, "end_idx": 100, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 107, "end_idx": 110, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 119, "end_idx": 126, "type": "pro", "entity": "右心室前后位造影" }, { "start_idx": 130, "end_idx": 136, "type": "pro", "entity": "右心室侧位造影" }, { "start_idx": 156, "end_idx": 160, "type": "pro", "entity": "双心室修补" } ] }, { "text": "心室发育是否正常?", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心室" } ] }, { "text": "是否仅可进行Fantan一类的手术?", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "Fantan" } ] }, { "text": "如果有可能进行双心室修补,是否存在肺动脉狭窄?", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "双心室修补" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "肺动脉狭窄" } ] }, { "text": "是否需要人工带瓣管道?", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "人工带瓣管道" } ] }, { "text": "在小婴儿,由于生长发育较快,一般须避免使用人工带瓣管道。", "entities": [ { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "人工带瓣管道" } ] }, { "text": "手术而不采用以后必须更换的人工带瓣管道。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "人工带瓣管道" } ] }, { "text": "在室间隔缺损关闭后,是否有可能进行循环的转换手术,如有可能,是否可进行大动脉转换术(如冠状动脉是否适合做大动脉转换术)?", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 35, "end_idx": 40, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 52, "end_idx": 57, "type": "pro", "entity": "大动脉转换术" } ] }, { "text": "有时关闭室间隔缺损可使患儿得到生存,而有时关闭室间隔缺损并解除肺动脉狭窄可得到良好的效果。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "室间隔缺" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "肺动脉狭窄" } ] }, { "text": "一般主张在2岁以后才作根治术,手术的目的是:①建立左心室与主动脉间的通道:用Dacron补片在室间隔缺损与主动脉瓣下流出道间建立人工隧道,注意避免此连接的梗阻,有时需扩大室间隔缺损;②建立右心室与肺动脉间的通路:可通过右心室流出道扩大补片、肺动脉瓣切开或在右心室与肺动脉间用人工带瓣管道连接;③修复伴发畸形。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "根治术" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "左心室" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "主动脉" }, { "start_idx": 38, "end_idx": 45, "type": "equ", "entity": "Dacron补片" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 53, "end_idx": 57, "type": "bod", "entity": "主动脉瓣下" }, { "start_idx": 64, "end_idx": 67, "type": "pro", "entity": "人工隧道" }, { "start_idx": 85, "end_idx": 89, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "右心室" }, { "start_idx": 98, "end_idx": 100, "type": "bod", "entity": "肺动脉" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "右心室" }, { "start_idx": 112, "end_idx": 114, "type": "bod", "entity": "流出道" }, { "start_idx": 117, "end_idx": 118, "type": "equ", "entity": "补片" }, { "start_idx": 120, "end_idx": 123, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 128, "end_idx": 130, "type": "bod", "entity": "右心室" }, { "start_idx": 132, "end_idx": 134, "type": "bod", "entity": "肺动脉" }, { "start_idx": 137, "end_idx": 142, "type": "pro", "entity": "人工带瓣管道" }, { "start_idx": 151, "end_idx": 152, "type": "dis", "entity": "畸形" } ] }, { "text": "最近报道,右心室双出口在新生儿期行根治术的死亡率可低至4%~8%。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "右心室双出口" }, { "start_idx": 17, "end_idx": 19, "type": "pro", "entity": "根治术" } ] }, { "text": "四、新生儿常用基本通气模式(一)持续气道正压持续气道正压(continuouspositiveairwaypressure,CPAP)也称自主呼吸(spontaneousbreathing,Spont),是指有自主呼吸的婴儿在整个呼吸周期中(吸气和呼气)接受呼吸机供给的高于大气压的气体压力,其作用为吸气时气体易于进入肺内,减少呼吸功;呼气时可防止病变肺泡萎陷,增加FRC,改善肺泡通气、血流比,从而升高PaO2。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "持续气道正压" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "持续气道正压" }, { "start_idx": 29, "end_idx": 60, "type": "pro", "entity": "continuouspositiveairwaypressure" }, { "start_idx": 62, "end_idx": 65, "type": "pro", "entity": "CPAP" }, { "start_idx": 69, "end_idx": 72, "type": "pro", "entity": "自主呼吸" }, { "start_idx": 74, "end_idx": 93, "type": "pro", "entity": "spontaneousbreathing" }, { "start_idx": 95, "end_idx": 99, "type": "pro", "entity": "Spont" }, { "start_idx": 129, "end_idx": 131, "type": "equ", "entity": "呼吸机" }, { "start_idx": 159, "end_idx": 159, "type": "bod", "entity": "肺" }, { "start_idx": 176, "end_idx": 177, "type": "bod", "entity": "肺泡" }, { "start_idx": 183, "end_idx": 185, "type": "ite", "entity": "FRC" }, { "start_idx": 189, "end_idx": 190, "type": "bod", "entity": "肺泡" } ] }, { "text": "主要用于低氧血症、轻型RDS和频发的呼吸暂停。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "低氧血症" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "轻型RDS" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "呼吸暂停" } ] }, { "text": "多主张应用鼻塞CPAP,但因易吞入空气导致腹胀,使用时应放置胃管以排气;经气管插管作CPAP,可增加气道阻力和呼吸功,只是在应用或撤离呼吸机前的短时间内应用。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "鼻塞CPAP" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "腹胀" }, { "start_idx": 30, "end_idx": 31, "type": "equ", "entity": "胃管" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "气管插管" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "CPAP" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "气道" }, { "start_idx": 67, "end_idx": 69, "type": "equ", "entity": "呼吸机" } ] }, { "text": "压力一般为3~8cmH2O,压力>8cmH2O(尤其当肺顺应性改善时)可影响静脉回流及降低心排血量,还会造成潮气量减低和PaCO2升高。", "entities": [ { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "肺" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "静脉" }, { "start_idx": 67, "end_idx": 67, "type": "bod", "entity": "心" }, { "start_idx": 76, "end_idx": 78, "type": "ite", "entity": "潮气量" } ] }, { "text": "CPAP不宜使用纯氧作气源。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "CPAP" } ] }, { "text": "(二)间歇指令通气间歇指令通气(intermittentmandatoryventilation,IMV)也称为间歇正压通气(intermittentpositivepressureventilation,IPPV)。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "间歇指令通气" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "间歇指令通气" }, { "start_idx": 16, "end_idx": 47, "type": "pro", "entity": "intermittentmandatoryventilation" }, { "start_idx": 49, "end_idx": 51, "type": "pro", "entity": "IMV" }, { "start_idx": 56, "end_idx": 61, "type": "pro", "entity": "间歇正压通气" }, { "start_idx": 63, "end_idx": 101, "type": "pro", "entity": "intermittentpositivepressureventilation" }, { "start_idx": 103, "end_idx": 106, "type": "pro", "entity": "IPPV" } ] }, { "text": "IMV是指呼吸机以预设的频率、压力和吸、呼气时间对患儿施以正压通气,患儿如有自主呼吸,则按自己的频率和形式进行呼吸,其总的通气量=患儿自主呼吸的通气量+呼吸机正压通气量;患儿接受正压通气的频率=呼吸机的预设频率。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "IMV" }, { "start_idx": 5, "end_idx": 7, "type": "equ", "entity": "呼吸机" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "正压通气" }, { "start_idx": 76, "end_idx": 78, "type": "equ", "entity": "呼吸机" }, { "start_idx": 89, "end_idx": 92, "type": "pro", "entity": "正压通气" }, { "start_idx": 97, "end_idx": 99, "type": "equ", "entity": "呼吸机" } ] }, { "text": "当应用较高频率IMV时,呼吸机可提供完全的通气支持。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "IMV" }, { "start_idx": 12, "end_idx": 14, "type": "equ", "entity": "呼吸机" } ] }, { "text": "因此,当患儿无自主呼吸时,可应用较高频率的IMV;随着自主呼吸的出现和增强,应相应减低IMV的频率,撤机前则可使IMV的频率降到5~10次/分,减少呼吸机的正压通气,以增强患儿自主呼吸的能力,达到依靠自主呼吸能保证气体交换的目的。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "pro", "entity": "IMV" }, { "start_idx": 43, "end_idx": 45, "type": "pro", "entity": "IMV" }, { "start_idx": 56, "end_idx": 58, "type": "pro", "entity": "IMV" }, { "start_idx": 74, "end_idx": 76, "type": "equ", "entity": "呼吸机" }, { "start_idx": 78, "end_idx": 81, "type": "pro", "entity": "正压通气" } ] }, { "text": "此方式由于呼吸机送气经常与患儿的呼气相冲突即人机不同步,故可导致小气道损伤、慢性肺疾病、脑室内出血和脑室周围白质软化等的发生。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "equ", "entity": "呼吸机" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "小气道损伤" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "慢性肺疾病" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "脑室内出血" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "脑室周围白质软化" } ] }, { "text": "(三)同步间歇指令通气同步间歇指令通气(synchronizedintermittentmandatoryventilation,SIMV)是指呼吸机通过识别患儿吸气初期气道压力或气体流速或腹部阻抗的变化,触发呼吸机以预设的频率进行机械通气,即与患儿吸气同步;当患儿呼吸暂停或无自主呼吸时,呼吸机则以设定的频率控制通气。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "同步间歇指令通气" }, { "start_idx": 11, "end_idx": 18, "type": "pro", "entity": "同步间歇指令通气" }, { "start_idx": 20, "end_idx": 63, "type": "pro", "entity": "synchronizedintermittentmandatoryventilation" }, { "start_idx": 65, "end_idx": 68, "type": "pro", "entity": "SIMV" }, { "start_idx": 72, "end_idx": 74, "type": "equ", "entity": "呼吸机" }, { "start_idx": 85, "end_idx": 86, "type": "bod", "entity": "气道" }, { "start_idx": 95, "end_idx": 96, "type": "bod", "entity": "腹部" }, { "start_idx": 105, "end_idx": 107, "type": "equ", "entity": "呼吸机" }, { "start_idx": 116, "end_idx": 119, "type": "pro", "entity": "机械通气" }, { "start_idx": 145, "end_idx": 147, "type": "equ", "entity": "呼吸机" } ] }, { "text": "患儿的吸气只有在呼吸机按预设频率送气前的较短时间内才能触发呼吸机的机械通气,因此,患儿接受正压通气的频率=呼吸机的预设频率。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "equ", "entity": "呼吸机" }, { "start_idx": 29, "end_idx": 31, "type": "equ", "entity": "呼吸机" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "机械通气" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "正压通气" }, { "start_idx": 53, "end_idx": 55, "type": "equ", "entity": "呼吸机" } ] }, { "text": "SIMV从根本上解决了人机不同步现象,从而避免了IMV的副作用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "SIMV" }, { "start_idx": 24, "end_idx": 26, "type": "pro", "entity": "IMV" } ] }, { "text": "(四)助-控制通气助-控制通气(assist/controlventilation,A/C)也称为同步间歇正压通气(synchronizedintermittentpositivepressureventilation,SIPPV)。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "助-控制通气" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "助-控制通气" }, { "start_idx": 16, "end_idx": 40, "type": "pro", "entity": "assist/controlventilation" }, { "start_idx": 42, "end_idx": 44, "type": "pro", "entity": "A/C" }, { "start_idx": 49, "end_idx": 56, "type": "pro", "entity": "同步间歇正压通气" }, { "start_idx": 58, "end_idx": 108, "type": "pro", "entity": "synchronizedintermittentpositivepressureventilation" }, { "start_idx": 110, "end_idx": 114, "type": "pro", "entity": "SIPPV" } ] }, { "text": "所谓辅助通气是指患儿的自主吸气触发机械通气,机械通气的频率是由自主呼吸的频率所决定;所谓控制通气是指呼吸机按预设的频率进行机械通气。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "辅助通气" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "机械通气" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "机械通气" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "控制通气" }, { "start_idx": 50, "end_idx": 52, "type": "equ", "entity": "呼吸机" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "机械通气" } ] }, { "text": "A/C是将辅助通气与控制通气相结合的通气模式,当自主呼吸较强时,依靠自主吸气触发机械通气,提供与自主呼吸频率相同并且同步的机械通气;当呼吸微弱或无自主呼吸时,呼吸机则按预设的通气频率进行机械通气,以保证患儿需要的通气量。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "A/C" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "辅助通气" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "控制通气" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "机械通气" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "机械通气" }, { "start_idx": 79, "end_idx": 81, "type": "equ", "entity": "呼吸机" }, { "start_idx": 93, "end_idx": 96, "type": "pro", "entity": "机械通气" } ] }, { "text": "因此,应用A/C模式时,患儿接受机械通气的频率≥预设的频率。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "A/C" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "机械通气" } ] }, { "text": "当患儿自主呼吸较强和较快时,由于患儿接受机械通气的频率大于预设频率,可产生过度通气,故应及时调低压力或降低触发敏感度(增大其负值),一般触发敏感度设置既要避免过度敏感,导致过多触发,也要避免触发敏感度过低,造成费力触发。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "机械通气" } ] }, { "text": "此外,有关压力支持通气(pressuresupportventilation,PSV)、容量控制通气(volume-controlventilation,VCV)、压力调节容量控制通气(pressureregulatedvolume-controlventilation,PRVC)、适应性支持通气(adaptivesupportventilation,ASV)、压力释放通气(pressurereleaseventilation,FRV)、双相气道正压通气(biphasicpositiveairwaypressure,BI-PAP)、指令分钟通气(mandatoryminuteventilation,MMV)、容量支持通气(vo1umesupportventilation,VSV)及成比率通气(proportionalassistedventilation,PAV)等通气模式,在新生儿不常用或不宜使用,故在此不一一赘述。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "压力支持通气" }, { "start_idx": 12, "end_idx": 37, "type": "pro", "entity": "pressuresupportventilation" }, { "start_idx": 39, "end_idx": 41, "type": "pro", "entity": "PSV" }, { "start_idx": 44, "end_idx": 49, "type": "pro", "entity": "容量控制通气" }, { "start_idx": 51, "end_idx": 75, "type": "pro", "entity": "volume-controlventilation" }, { "start_idx": 77, "end_idx": 79, "type": "pro", "entity": "VCV" }, { "start_idx": 82, "end_idx": 91, "type": "pro", "entity": "压力调节容量控制通气" }, { "start_idx": 93, "end_idx": 134, "type": "pro", "entity": "pressureregulatedvolume-controlventilation" }, { "start_idx": 136, "end_idx": 139, "type": "pro", "entity": "PRVC" }, { "start_idx": 142, "end_idx": 148, "type": "pro", "entity": "适应性支持通气" }, { "start_idx": 150, "end_idx": 175, "type": "pro", "entity": "adaptivesupportventilation" }, { "start_idx": 177, "end_idx": 179, "type": "pro", "entity": "ASV" }, { "start_idx": 182, "end_idx": 187, "type": "pro", "entity": "压力释放通气" }, { "start_idx": 189, "end_idx": 214, "type": "pro", "entity": "pressurereleaseventilation" }, { "start_idx": 216, "end_idx": 218, "type": "pro", "entity": "FRV" }, { "start_idx": 221, "end_idx": 228, "type": "pro", "entity": "双相气道正压通气" }, { "start_idx": 230, "end_idx": 259, "type": "pro", "entity": "biphasicpositiveairwaypressure" }, { "start_idx": 261, "end_idx": 266, "type": "pro", "entity": "BI-PAP" }, { "start_idx": 269, "end_idx": 274, "type": "pro", "entity": "指令分钟通气" }, { "start_idx": 276, "end_idx": 301, "type": "pro", "entity": "mandatoryminuteventilation" }, { "start_idx": 303, "end_idx": 305, "type": "pro", "entity": "MMV" }, { "start_idx": 308, "end_idx": 313, "type": "pro", "entity": "容量支持通气" }, { "start_idx": 315, "end_idx": 338, "type": "pro", "entity": "vo1umesupportventilation" }, { "start_idx": 340, "end_idx": 342, "type": "pro", "entity": "VSV" }, { "start_idx": 345, "end_idx": 349, "type": "pro", "entity": "成比率通气" }, { "start_idx": 351, "end_idx": 381, "type": "pro", "entity": "proportionalassistedventilation" }, { "start_idx": 383, "end_idx": 385, "type": "pro", "entity": "PAV" } ] }, { "text": "第九章结核病第一节概况结核病(tuberculosis)是由结核杆菌感染引起的慢性感染性疾病。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "结核病" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "结核病" }, { "start_idx": 15, "end_idx": 26, "type": "dis", "entity": "tuberculosis" }, { "start_idx": 30, "end_idx": 33, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "慢性感染性疾病" } ] }, { "text": "全身各个器官都可累及,以肺结核病为最常见。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "器官" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "肺结核病" } ] }, { "text": "目前在全球范围内,由于耐药结核菌株的出现与扩展,结核病与艾滋病的双重感染,许多国家结核病控制规划不完善,使得全球结核病疫情明显上升并呈持续上升的趋势。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "mic", "entity": "结核菌" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "结核病" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "艾滋病" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "结核病" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "结核病" } ] }, { "text": "在全球所有感染性疾病中,结核病已成为成年人的首要死因。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "结核病" } ] }, { "text": "世界卫生组织于1993年4月向全世界宣布:全球处于结核病紧急状态。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "结核病" } ] }, { "text": "同样,我国结核病流行形势也十分严峻。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "结核病" } ] }, { "text": "30年来由于推广了卡介苗接种及应用抗结核治疗,结核病流行情况大为好转,但由于人口众多,结核病仍为我国常见病。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "卡介苗" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "接种" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "抗结核治疗" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "结核病" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "结核病" } ] }, { "text": "一、病原结核病的病原为结核杆菌,包括结核分枝杆菌、牛型分枝杆菌和非洲分枝杆菌,属放线菌目、分枝杆菌科。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "结核病" }, { "start_idx": 11, "end_idx": 14, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 18, "end_idx": 23, "type": "mic", "entity": "结核分枝杆菌" }, { "start_idx": 25, "end_idx": 30, "type": "mic", "entity": "牛型分枝杆菌" }, { "start_idx": 32, "end_idx": 37, "type": "mic", "entity": "非洲分枝杆菌" }, { "start_idx": 40, "end_idx": 42, "type": "mic", "entity": "放线菌" }, { "start_idx": 45, "end_idx": 48, "type": "mic", "entity": "分枝杆菌" } ] }, { "text": "结核杆菌为无芽胞、不运动、多形性,长2~4μm,稍弯曲的革兰弱阳性杆菌。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 28, "end_idx": 34, "type": "mic", "entity": "革兰弱阳性杆菌" } ] }, { "text": "所有分枝杆菌均具有抗酸特性。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "mic", "entity": "分枝杆菌" } ] }, { "text": "除了上述引起结核病的分枝杆菌,其他分枝杆菌则称为非结核分枝杆菌。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "结核病" }, { "start_idx": 10, "end_idx": 13, "type": "mic", "entity": "分枝杆菌" }, { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "分枝杆菌" }, { "start_idx": 24, "end_idx": 30, "type": "mic", "entity": "非结核分枝杆菌" } ] }, { "text": "这些分枝杆菌在健康人或免疫缺陷的患者,可以引起局限性病变,如局部的淋巴结炎。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "mic", "entity": "分枝杆菌" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "局限性病变" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "淋巴结炎" } ] }, { "text": "第十章小儿血尿的鉴别诊断血尿(hematuria)是儿科泌尿系统疾病最常见的症状,可分为肉眼及镜下血尿。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "小儿血尿的鉴别诊断" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "血尿" }, { "start_idx": 15, "end_idx": 23, "type": "sym", "entity": "hematuria" }, { "start_idx": 26, "end_idx": 27, "type": "dep", "entity": "儿科" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "肉眼及镜下血尿" } ] }, { "text": "首先要鉴别是真性血尿还是假性血尿,假性血尿(falsehematuria)常可见于:①非泌尿道出血:阴道出血混入,尤其青春期女孩应排除月经污染。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "真性血尿" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "假性血尿" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "假性血尿" }, { "start_idx": 22, "end_idx": 35, "type": "sym", "entity": "falsehematuria" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "泌尿道" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "非泌尿道出血" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "阴道" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "阴道出血" } ] }, { "text": "②红色尿:机体某些代谢产物如卟啉尿可使尿呈红色,酚红、刚果红、氨基比林及柔红霉素等也可使尿呈红色;新生儿期由于尿中排出较多尿酸盐也可使尿布红染;红色尿还见于血红蛋白尿及肌红蛋白尿;某些食物、蔬菜中的色素也使尿呈红色。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "sym", "entity": "红色尿" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "卟啉尿" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "氨基比林" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "柔红霉素" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "尿呈红色" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "尿" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "尿酸盐" }, { "start_idx": 55, "end_idx": 63, "type": "sym", "entity": "尿中排出较多尿酸盐" }, { "start_idx": 72, "end_idx": 74, "type": "sym", "entity": "红色尿" }, { "start_idx": 78, "end_idx": 82, "type": "sym", "entity": "血红蛋白尿" }, { "start_idx": 84, "end_idx": 88, "type": "sym", "entity": "肌红蛋白尿" }, { "start_idx": 103, "end_idx": 106, "type": "sym", "entity": "尿呈红色" } ] }, { "text": "鉴别点在于尿镜检时无红细胞。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "尿镜检" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "无红细胞" } ] }, { "text": "血尿的检查方法及标准为:取10ml清洁新鲜中段尿(以晨尿为好),以1500转/min离心5分钟,取沉渣镜检,正常人红细胞仅为0~2个/高倍视野(HPF),当红细胞>3个/HPF则考虑有病理意义。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "血尿" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "红细胞" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "红细胞" }, { "start_idx": 78, "end_idx": 87, "type": "sym", "entity": "红细胞>3个/HPF" } ] }, { "text": "出血量超过1ml/L尿液,则可见肉眼血尿。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "出血量超过1ml/L尿液" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "肉眼血尿" } ] }, { "text": "大量出血呈烟灰水样,酸性尿时色较暗红,肉眼血尿放置久也呈暗红色。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "大量出血呈烟灰水样" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "酸性尿时色较暗红" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "肉眼血尿" } ] }, { "text": "病毒感染及剧烈运动后偶有一过性血尿,排除方法为3次以上尿镜检。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "病毒感染" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "一过性血尿" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "3次以上尿镜检" } ] }, { "text": "【病因】血尿病因复杂,泌尿系统各部位的炎症、畸形、结石、外伤及肿瘤等均可引起血尿,还可见于全身血液系统疾病时因凝血机制障碍而致血尿。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "血尿" }, { "start_idx": 11, "end_idx": 29, "type": "dis", "entity": "泌尿系统各部位的炎症、畸形、结石、外伤" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "肿瘤" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "血尿" }, { "start_idx": 45, "end_idx": 52, "type": "dis", "entity": "全身血液系统疾病" }, { "start_idx": 55, "end_idx": 60, "type": "sym", "entity": "凝血机制障碍" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "血尿" } ] }, { "text": "但最多见的是肾小球性血尿。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "肾小球性血尿" } ] }, { "text": "(一)肾小球性血尿指血尿部位来源于肾小球。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "肾小球性血尿" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "血尿" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肾小球" } ] }, { "text": "1.原发性肾小球疾病如急性、慢性肾小球肾炎,肾病综合征(肾病),急进性肾炎,IgA肾病,遗传性肾炎等。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "原发性肾小球疾病" }, { "start_idx": 11, "end_idx": 20, "type": "dis", "entity": "急性、慢性肾小球肾炎" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "肾病" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "急进性肾炎" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "遗传性肾炎" } ] }, { "text": "2.继发性肾小球疾病如系统性红斑狼疮肾炎(LN),紫癜肾炎,乙型肝炎相关性肾炎,溶血尿毒综合征(HUS),肺出血肾炎综合征等。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "继发性肾小球疾病" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "系统性红斑狼疮肾炎" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "LN" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "紫癜肾炎" }, { "start_idx": 30, "end_idx": 38, "type": "dis", "entity": "乙型肝炎相关性肾炎" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "溶血尿毒综合征" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "HUS" }, { "start_idx": 53, "end_idx": 60, "type": "dis", "entity": "肺出血肾炎综合征" } ] }, { "text": "(二)非肾小球性血尿血尿来源于肾小球以下泌尿系统,肾盏、肾盂、输尿管、膀胱或尿道。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "非肾小球性血尿" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "血尿" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肾小球" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肾盏" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肾盂" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "输尿管" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "膀胱" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "尿道" } ] }, { "text": "1.泌尿道急性及慢性感染。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "泌尿道急性及慢性感染" } ] }, { "text": "2.肾盂、膀胱及输尿管结石。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "肾盂、膀胱及输尿管结石" } ] }, { "text": "3.特发性高钙尿症。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "特发性高钙尿症" } ] }, { "text": "4.左肾静脉压迫综合征(leftrenalveinentrapmentsyndrome,或称胡桃夹现象nutcrackerphenomenon)。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "左肾静脉压迫综合征" }, { "start_idx": 12, "end_idx": 42, "type": "dis", "entity": "leftrenalveinentrapmentsyndrome" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "胡桃夹现象" }, { "start_idx": 51, "end_idx": 70, "type": "sym", "entity": "nutcrackerphenomenon" } ] }, { "text": "6.肿瘤、外伤及异物。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "外伤" } ] }, { "text": "7.药物所致肾及膀胱损伤如环磷酰胺、磺胺类、氨基糖苷类抗生素如庆大霉素等。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "肾及膀胱损伤" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "磺胺类" }, { "start_idx": 22, "end_idx": 29, "type": "dru", "entity": "氨基糖苷类抗生素" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "庆大霉素" } ] }, { "text": "8.结核、原虫及螺旋体等感染。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "sym", "entity": "结核、原虫及螺旋体等感染" } ] }, { "text": "9.全身疾病引起的出血,如血小板减少性紫癜、新生儿自然出血症及血友病等。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "出血" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "血小板减少性紫癜" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "新生儿自然出血症" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "血友病" } ] }, { "text": "【诊断与鉴别诊断】病史及体检1.血尿有关病史既往有无肉眼血尿发作史和常规尿检查史;家族中有无肾脏病史及尿毒症者、有无耳聋患者;发病有无前驱感染及诱因;有无发热、皮疹、关节肿痛,便血或咯血史;抗生素、磺胺类药物及环磷酰胺等药物应用史。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "血尿" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 34, "end_idx": 38, "type": "pro", "entity": "常规尿检查" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "肾脏病" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "尿毒症" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "耳聋" }, { "start_idx": 67, "end_idx": 70, "type": "sym", "entity": "前驱感染" }, { "start_idx": 77, "end_idx": 78, "type": "sym", "entity": "发热" }, { "start_idx": 80, "end_idx": 81, "type": "sym", "entity": "皮疹" }, { "start_idx": 83, "end_idx": 84, "type": "bod", "entity": "关节" }, { "start_idx": 83, "end_idx": 86, "type": "sym", "entity": "关节肿痛" }, { "start_idx": 88, "end_idx": 89, "type": "sym", "entity": "便血" }, { "start_idx": 91, "end_idx": 92, "type": "sym", "entity": "咯血" }, { "start_idx": 95, "end_idx": 97, "type": "dru", "entity": "抗生素" }, { "start_idx": 99, "end_idx": 103, "type": "dru", "entity": "磺胺类药物" }, { "start_idx": 105, "end_idx": 108, "type": "dru", "entity": "环磷酰胺" } ] }, { "text": "引起血尿的原因按年龄来分:①新生儿期:可见于新生儿出血、严重缺氧、窒息、肾静脉血栓及急性肾乳头坏死等;②婴幼儿期最常见泌尿系感染及先天性尿路畸形,其次为肾胚胎瘤、肾母细胞瘤、HUS及重症遗传性肾炎,部分良性家族性血尿也在3岁前起病;③儿童期最常见为急性肾炎综合征及各类原发及继发性肾小球肾炎,其次为泌尿系感染、家族性良性血尿、遗传性进行性肾炎、高钙尿症及左肾静脉扩张等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "血尿" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "新生儿出血" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "严重缺氧" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "窒息" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "肾静脉" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "肾静脉血栓" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "急性肾乳头坏死" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "泌尿系" }, { "start_idx": 59, "end_idx": 63, "type": "sym", "entity": "泌尿系感染" }, { "start_idx": 65, "end_idx": 71, "type": "sym", "entity": "先天性尿路畸形" }, { "start_idx": 76, "end_idx": 79, "type": "dis", "entity": "肾胚胎瘤" }, { "start_idx": 81, "end_idx": 85, "type": "dis", "entity": "肾母细胞瘤" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "HUS" }, { "start_idx": 91, "end_idx": 97, "type": "dis", "entity": "重症遗传性肾炎" }, { "start_idx": 101, "end_idx": 107, "type": "sym", "entity": "良性家族性血尿" }, { "start_idx": 124, "end_idx": 130, "type": "dis", "entity": "急性肾炎综合征" }, { "start_idx": 134, "end_idx": 144, "type": "dis", "entity": "原发及继发性肾小球肾炎" }, { "start_idx": 149, "end_idx": 153, "type": "dis", "entity": "泌尿系感染" }, { "start_idx": 155, "end_idx": 161, "type": "sym", "entity": "家族性良性血尿" }, { "start_idx": 163, "end_idx": 170, "type": "dis", "entity": "遗传性进行性肾炎" }, { "start_idx": 172, "end_idx": 175, "type": "dis", "entity": "高钙尿症" }, { "start_idx": 177, "end_idx": 182, "type": "dis", "entity": "左肾静脉扩张" } ] }, { "text": "2.体格检查对血尿的初诊患儿要做全面的体格检查,包括生长发育状况,有无水肿、高血压及贫血貌;皮肤有无出血点、瘀斑及皮疹;腹部有无包块,肾区有无叩击痛;有无耳聋,特别是神经性耳聋,眼疾等;并检查外生殖器,特别是男孩有无包茎及包皮粘连。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "体格检查" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "血尿" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "体格检查" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "水肿" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "高血压" }, { "start_idx": 42, "end_idx": 44, "type": "sym", "entity": "贫血貌" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "皮肤" }, { "start_idx": 50, "end_idx": 52, "type": "sym", "entity": "出血点" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "瘀斑" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "皮疹" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "腹部" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "肾区" }, { "start_idx": 71, "end_idx": 73, "type": "sym", "entity": "叩击痛" }, { "start_idx": 77, "end_idx": 78, "type": "dis", "entity": "耳聋" }, { "start_idx": 83, "end_idx": 87, "type": "dis", "entity": "神经性耳聋" }, { "start_idx": 89, "end_idx": 90, "type": "dis", "entity": "眼疾" }, { "start_idx": 96, "end_idx": 99, "type": "bod", "entity": "外生殖器" }, { "start_idx": 108, "end_idx": 109, "type": "sym", "entity": "包茎" }, { "start_idx": 111, "end_idx": 114, "type": "sym", "entity": "包皮粘连" } ] }, { "text": "3.结合病史、临床表现及体格检查综合分析(1)血尿和感染有关:急性肾炎综合征常有较明显的前驱感染病史;病毒感染如腮腺炎或EB病毒感染可出现一过性血尿;细菌性心内膜炎可伴肾梗死出现血尿;流行性出血热有出血、发热和肾衰竭;HUS常有肠道感染史;肾结核不仅有血尿,更多伴脓尿;最常见的泌尿系感染可由细菌、病毒及衣原体等引起,表现血尿伴尿路刺激症状,但小婴儿可仅表现为发热、拒食、哭闹及体重不增等。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "体格检查" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "血尿" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "感染" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "急性肾炎综合征" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "前驱感染" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "病毒感染" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "EB病毒感染" }, { "start_idx": 69, "end_idx": 73, "type": "sym", "entity": "一过性血尿" }, { "start_idx": 75, "end_idx": 81, "type": "dis", "entity": "细菌性心内膜炎" }, { "start_idx": 84, "end_idx": 86, "type": "dis", "entity": "肾梗死" }, { "start_idx": 89, "end_idx": 90, "type": "sym", "entity": "血尿" }, { "start_idx": 92, "end_idx": 97, "type": "dis", "entity": "流行性出血热" }, { "start_idx": 99, "end_idx": 100, "type": "sym", "entity": "出血" }, { "start_idx": 102, "end_idx": 103, "type": "sym", "entity": "发热" }, { "start_idx": 105, "end_idx": 107, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 109, "end_idx": 111, "type": "dis", "entity": "HUS" }, { "start_idx": 114, "end_idx": 117, "type": "dis", "entity": "肠道感染" }, { "start_idx": 120, "end_idx": 122, "type": "dis", "entity": "肾结核" }, { "start_idx": 126, "end_idx": 127, "type": "sym", "entity": "血尿" }, { "start_idx": 132, "end_idx": 133, "type": "sym", "entity": "脓尿" }, { "start_idx": 139, "end_idx": 143, "type": "dis", "entity": "泌尿系感染" }, { "start_idx": 146, "end_idx": 147, "type": "mic", "entity": "细菌" }, { "start_idx": 149, "end_idx": 150, "type": "mic", "entity": "病毒" }, { "start_idx": 152, "end_idx": 154, "type": "mic", "entity": "衣原体" }, { "start_idx": 161, "end_idx": 162, "type": "sym", "entity": "血尿" }, { "start_idx": 164, "end_idx": 165, "type": "bod", "entity": "尿路" }, { "start_idx": 164, "end_idx": 169, "type": "sym", "entity": "尿路刺激症状" }, { "start_idx": 180, "end_idx": 181, "type": "sym", "entity": "发热" }, { "start_idx": 183, "end_idx": 184, "type": "sym", "entity": "拒食" }, { "start_idx": 186, "end_idx": 187, "type": "sym", "entity": "哭闹" }, { "start_idx": 189, "end_idx": 192, "type": "sym", "entity": "体重不增" } ] }, { "text": "(2)血尿伴蛋白尿、水肿和高血压:最常见为急性肾炎综合征,当血清补体C3下降且在8周内恢复正常,并有血清抗“O”升高,则可确诊为急性链球菌感染后肾小球肾炎;当血尿伴有进行性少尿及肾功能急骤恶化者考虑急进性肾炎可能性大;儿童期发作性肉眼血尿且和上呼吸道关系密切,则应考虑IgA肾病;持续低补体伴血尿及中度以上蛋白尿多见于膜增殖性肾炎;生长发育障碍、中度以上贫血、持续高血压及肾功能不全首先想到慢性肾炎;血尿伴大量蛋白尿则为肾炎性肾病,病理可见多种形态改变,特别应注意微小病变也有13%可出现镜下血尿。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "血尿" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "水肿" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "高血压" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "急性肾炎综合征" }, { "start_idx": 30, "end_idx": 35, "type": "bod", "entity": "血清补体C3" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "血清补体C3下降" }, { "start_idx": 50, "end_idx": 57, "type": "sym", "entity": "血清抗“O”升高" }, { "start_idx": 64, "end_idx": 70, "type": "dis", "entity": "急性链球菌感染" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "肾小球肾炎" }, { "start_idx": 79, "end_idx": 80, "type": "sym", "entity": "血尿" }, { "start_idx": 83, "end_idx": 87, "type": "sym", "entity": "进行性少尿" }, { "start_idx": 89, "end_idx": 91, "type": "ite", "entity": "肾功能" }, { "start_idx": 89, "end_idx": 95, "type": "sym", "entity": "肾功能急骤恶化" }, { "start_idx": 99, "end_idx": 103, "type": "dis", "entity": "急进性肾炎" }, { "start_idx": 109, "end_idx": 118, "type": "sym", "entity": "儿童期发作性肉眼血尿" }, { "start_idx": 121, "end_idx": 124, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 134, "end_idx": 138, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 140, "end_idx": 144, "type": "sym", "entity": "持续低补体" }, { "start_idx": 146, "end_idx": 147, "type": "sym", "entity": "血尿" }, { "start_idx": 149, "end_idx": 155, "type": "sym", "entity": "中度以上蛋白尿" }, { "start_idx": 159, "end_idx": 164, "type": "sym", "entity": "膜增殖性肾炎" }, { "start_idx": 166, "end_idx": 171, "type": "sym", "entity": "生长发育障碍" }, { "start_idx": 173, "end_idx": 178, "type": "sym", "entity": "中度以上贫血" }, { "start_idx": 180, "end_idx": 184, "type": "sym", "entity": "持续高血压" }, { "start_idx": 186, "end_idx": 190, "type": "sym", "entity": "肾功能不全" }, { "start_idx": 195, "end_idx": 198, "type": "dis", "entity": "慢性肾炎" }, { "start_idx": 200, "end_idx": 201, "type": "sym", "entity": "血尿" }, { "start_idx": 203, "end_idx": 207, "type": "sym", "entity": "大量蛋白尿" }, { "start_idx": 210, "end_idx": 214, "type": "dis", "entity": "肾炎性肾病" }, { "start_idx": 244, "end_idx": 247, "type": "sym", "entity": "镜下血尿" } ] }, { "text": "(3)系统性疾病及遗传性疾病的肾损害:典型的紫癜肾炎及LN不难诊断,但应注意不典型病例及急、重型病例。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "系统性疾病" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肾损害" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "紫癜肾炎" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "LN" } ] }, { "text": "我科有持续三年血尿伴轻~中度蛋白尿,反复查抗核抗体阴性,最终靠肾活体组织检查为典型免疫荧光表现,始确诊为LN的病例,更有学龄前4~5岁儿童以全身皮疹、贫血及镜下血尿起病的LN者;血尿伴不明原因的发热、消瘦、贫血及咯血史应疑为肺出血肾炎综合征;发热伴面、颈及上胸部潮红,热退后出现低血压、休克、少尿,继而出现血尿应考虑流行性出血热;HUS除血尿及少尿外还有皮肤黏膜出血及黄疸;家族性良性血尿常有明确家族史,当同时伴耳聋、眼疾及肾功能进行性恶化者,尤其是男孩,最多见为Alport综合征。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "血尿" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "轻~中度蛋白尿" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "抗核抗体阴性" }, { "start_idx": 31, "end_idx": 37, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "典型免疫荧光表现" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "LN" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "全身皮疹" }, { "start_idx": 75, "end_idx": 76, "type": "sym", "entity": "贫血" }, { "start_idx": 78, "end_idx": 81, "type": "sym", "entity": "镜下血尿" }, { "start_idx": 85, "end_idx": 86, "type": "dis", "entity": "LN" }, { "start_idx": 89, "end_idx": 90, "type": "sym", "entity": "血尿" }, { "start_idx": 97, "end_idx": 98, "type": "sym", "entity": "发热" }, { "start_idx": 100, "end_idx": 101, "type": "sym", "entity": "消瘦" }, { "start_idx": 103, "end_idx": 104, "type": "sym", "entity": "贫血" }, { "start_idx": 106, "end_idx": 108, "type": "sym", "entity": "咯血史" }, { "start_idx": 112, "end_idx": 119, "type": "dis", "entity": "肺出血肾炎综合征" }, { "start_idx": 121, "end_idx": 122, "type": "sym", "entity": "发热" }, { "start_idx": 124, "end_idx": 130, "type": "bod", "entity": "面、颈及上胸部" }, { "start_idx": 124, "end_idx": 132, "type": "sym", "entity": "面、颈及上胸部潮红" }, { "start_idx": 139, "end_idx": 141, "type": "sym", "entity": "低血压" }, { "start_idx": 143, "end_idx": 144, "type": "sym", "entity": "休克" }, { "start_idx": 146, "end_idx": 147, "type": "sym", "entity": "少尿" }, { "start_idx": 153, "end_idx": 154, "type": "sym", "entity": "血尿" }, { "start_idx": 158, "end_idx": 163, "type": "sym", "entity": "流行性出血热" }, { "start_idx": 165, "end_idx": 167, "type": "sym", "entity": "HUS" }, { "start_idx": 169, "end_idx": 170, "type": "sym", "entity": "血尿" }, { "start_idx": 172, "end_idx": 173, "type": "sym", "entity": "少尿" }, { "start_idx": 177, "end_idx": 180, "type": "bod", "entity": "皮肤黏膜" }, { "start_idx": 177, "end_idx": 182, "type": "sym", "entity": "皮肤黏膜出血" }, { "start_idx": 184, "end_idx": 185, "type": "dis", "entity": "黄疸" }, { "start_idx": 187, "end_idx": 193, "type": "sym", "entity": "家族性良性血尿" }, { "start_idx": 206, "end_idx": 207, "type": "dis", "entity": "耳聋" }, { "start_idx": 209, "end_idx": 210, "type": "dis", "entity": "眼疾" }, { "start_idx": 212, "end_idx": 219, "type": "dis", "entity": "肾功能进行性恶化" }, { "start_idx": 232, "end_idx": 240, "type": "dis", "entity": "Alport综合征" } ] }, { "text": "4.特殊类型血尿包括特发性高钙尿及胡桃夹现象。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "血尿" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "特发性高钙尿" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "胡桃夹现象" } ] }, { "text": "后者需通过尿红细胞形态、尿Ca/Cr比值、24小时尿钙定量及腹部B超鉴别。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "ite", "entity": "尿红细胞形态" }, { "start_idx": 12, "end_idx": 19, "type": "ite", "entity": "尿Ca/Cr比值" }, { "start_idx": 21, "end_idx": 28, "type": "ite", "entity": "24小时尿钙定量" }, { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "腹部B超" } ] }, { "text": "5.肉眼观察带有血凝块多来自下泌尿道,血块或混合黏膜样物质多来自膀胱,滴血多来自尿道。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "下泌尿道" }, { "start_idx": 6, "end_idx": 17, "type": "sym", "entity": "带有血凝块多来自下泌尿道" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "膀胱" }, { "start_idx": 19, "end_idx": 33, "type": "sym", "entity": "血块或混合黏膜样物质多来自膀胱" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "尿道" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "滴血多来自尿道" } ] }, { "text": "尿三杯试验:用三只白色透明容器收集患儿排尿过程中的初、中、终段的尿液(初及中段尿液不得少于20ml)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "尿三杯试验" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "尿液" } ] }, { "text": "仅有初段血尿表示病变在尿道;终末滴血示病变在膀胱颈部和三角区、后尿道及前列腺等处;全程血尿则提示肾、输尿管或膀胱。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "血尿" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "尿道" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "病变在尿道" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "终末滴血" }, { "start_idx": 22, "end_idx": 29, "type": "bod", "entity": "膀胱颈部和三角区" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "后尿道" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "前列腺" }, { "start_idx": 19, "end_idx": 37, "type": "sym", "entity": "病变在膀胱颈部和三角区、后尿道及前列腺" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "血尿" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "肾" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "输尿管" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "膀胱" } ] }, { "text": "6.肾小球性血尿特点肾小球性血尿为全程血尿,无血凝块;可有肾区钝痛;常合并蛋白尿及管型,特别是有红细胞管型更说明血尿来自肾实质;尿沉渣红细胞形态及容积分布曲线检查符合肾小球血尿。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "肾小球性血尿" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "肾小球性血尿" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "血尿" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "无血凝块" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肾区" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "肾区钝痛" }, { "start_idx": 35, "end_idx": 42, "type": "sym", "entity": "合并蛋白尿及管型" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "红细胞管型" }, { "start_idx": 56, "end_idx": 62, "type": "sym", "entity": "血尿来自肾实质" }, { "start_idx": 64, "end_idx": 80, "type": "pro", "entity": "尿沉渣红细胞形态及容积分布曲线检查" }, { "start_idx": 83, "end_idx": 87, "type": "sym", "entity": "肾小球血尿" } ] }, { "text": "【有关实验室检查】(一)尿常规检查常用多联试纸法,可作为过筛及普查,敏感性为90%,但假阳性较高,需进一步做尿沉渣镜检以明确,当离心尿红细胞>3个/HPF且三次以上则有病理意义;血尿如伴蛋白尿及红细胞管型则多为肾小球病变。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "尿常规检查" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "多联试纸法" }, { "start_idx": 54, "end_idx": 58, "type": "pro", "entity": "尿沉渣镜检" }, { "start_idx": 64, "end_idx": 69, "type": "ite", "entity": "离心尿红细胞" }, { "start_idx": 64, "end_idx": 76, "type": "sym", "entity": "离心尿红细胞>3个/HPF" }, { "start_idx": 89, "end_idx": 90, "type": "sym", "entity": "血尿" }, { "start_idx": 93, "end_idx": 95, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 97, "end_idx": 101, "type": "sym", "entity": "红细胞管型" }, { "start_idx": 105, "end_idx": 107, "type": "bod", "entity": "肾小球" }, { "start_idx": 105, "end_idx": 109, "type": "sym", "entity": "肾小球病变" } ] }, { "text": "(二)尿红细胞形态近年来采用相差显微镜及扫描电镜观察尿红细胞形态变化,肾实质病变时红细胞通过基底膜受挤压,并受肾小管渗透压作用而变形,故认为当尿中红细胞形态以变形红细胞为主时属肾小球性血尿,其变形程度和肾病变严重性相一致。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "ite", "entity": "尿红细胞形态" }, { "start_idx": 16, "end_idx": 18, "type": "equ", "entity": "显微镜" }, { "start_idx": 20, "end_idx": 23, "type": "equ", "entity": "扫描电镜" }, { "start_idx": 26, "end_idx": 31, "type": "ite", "entity": "尿红细胞形态" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "肾实质病变" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "红细胞" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "基底膜" }, { "start_idx": 41, "end_idx": 51, "type": "sym", "entity": "红细胞通过基底膜受挤压" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "肾小管" }, { "start_idx": 71, "end_idx": 71, "type": "bod", "entity": "尿" }, { "start_idx": 73, "end_idx": 77, "type": "ite", "entity": "红细胞形态" }, { "start_idx": 79, "end_idx": 83, "type": "bod", "entity": "变形红细胞" }, { "start_idx": 71, "end_idx": 85, "type": "sym", "entity": "尿中红细胞形态以变形红细胞为主" }, { "start_idx": 88, "end_idx": 93, "type": "sym", "entity": "肾小球性血尿" } ] }, { "text": "而红细胞形态基本正常、均一则为非肾小球性血尿,多由尿路血管破裂出血而造成。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "非肾小球性血尿" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "尿路血管" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "尿路血管破裂出血" } ] }, { "text": "当红细胞形态严重变形呈芽胞、环状及穿孔等改变,称为严重变形红细胞,当其数目>30%以上考虑为肾性血尿,如以均一型红细胞为主,或变形红细胞数目<10%应考虑为非肾性血尿。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "ite", "entity": "红细胞形态" }, { "start_idx": 1, "end_idx": 21, "type": "sym", "entity": "红细胞形态严重变形呈芽胞、环状及穿孔等改变" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "严重变形红细胞" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "肾性血尿" }, { "start_idx": 53, "end_idx": 58, "type": "ite", "entity": "均一型红细胞" }, { "start_idx": 52, "end_idx": 60, "type": "sym", "entity": "以均一型红细胞为主" }, { "start_idx": 63, "end_idx": 67, "type": "ite", "entity": "变形红细胞" }, { "start_idx": 63, "end_idx": 73, "type": "sym", "entity": "变形红细胞数目<10%" }, { "start_idx": 78, "end_idx": 82, "type": "sym", "entity": "非肾性血尿" } ] }, { "text": "临床诊断符合率各家报告均在95%左右,但需注意尿中红细胞<8000个/ml及低比重时不可靠。", "entities": [ { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "尿" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "红细胞" }, { "start_idx": 23, "end_idx": 36, "type": "sym", "entity": "尿中红细胞<8000个/ml" } ] }, { "text": "血细胞自动分析仪测定尿红细胞形态容积分布曲线,对判断血尿来源有一定意义,当尿红细胞平均容积(MCV)<72fl,且呈小细胞分布,则说明为肾小球血尿。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "equ", "entity": "血细胞自动分析仪" }, { "start_idx": 10, "end_idx": 15, "type": "ite", "entity": "尿红细胞形态" }, { "start_idx": 10, "end_idx": 21, "type": "sym", "entity": "尿红细胞形态容积分布曲线" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "血尿" }, { "start_idx": 37, "end_idx": 40, "type": "ite", "entity": "尿红细胞" }, { "start_idx": 37, "end_idx": 54, "type": "sym", "entity": "尿红细胞平均容积(MCV)<72fl" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "小细胞" }, { "start_idx": 58, "end_idx": 62, "type": "sym", "entity": "小细胞分布" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "肾小球" }, { "start_idx": 68, "end_idx": 72, "type": "sym", "entity": "肾小球血尿" } ] }, { "text": "本法不受、尿比重、pH及主观影响,有一定临床应用价值。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "尿比重" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "pH" } ] }, { "text": "(三)血常规及相应的血液系统检查如贫血程度、生血状况(网织红细胞计数、血小板计数、出凝血时间、凝血酶原时间、纤维蛋白原水平及血浆抗凝血酶-Ⅲ(AT-Ⅲ)等对于以血尿表现的各类原发及继发性肾炎、肾衰竭、合并血栓,或全身血液疾病所致血尿的诊断及鉴别诊断有意义。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "血常规" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "血液系统检查" }, { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "贫血程度" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "生血状况" }, { "start_idx": 27, "end_idx": 33, "type": "ite", "entity": "网织红细胞计数" }, { "start_idx": 35, "end_idx": 39, "type": "ite", "entity": "血小板计数" }, { "start_idx": 41, "end_idx": 45, "type": "ite", "entity": "出凝血时间" }, { "start_idx": 47, "end_idx": 52, "type": "ite", "entity": "凝血酶原时间" }, { "start_idx": 54, "end_idx": 60, "type": "ite", "entity": "纤维蛋白原水平" }, { "start_idx": 62, "end_idx": 69, "type": "ite", "entity": "血浆抗凝血酶-Ⅲ" }, { "start_idx": 71, "end_idx": 74, "type": "ite", "entity": "AT-Ⅲ" }, { "start_idx": 80, "end_idx": 81, "type": "sym", "entity": "血尿" }, { "start_idx": 90, "end_idx": 94, "type": "dis", "entity": "继发性肾炎" }, { "start_idx": 96, "end_idx": 98, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 100, "end_idx": 103, "type": "dis", "entity": "合并血栓" }, { "start_idx": 106, "end_idx": 111, "type": "dis", "entity": "全身血液疾病" }, { "start_idx": 114, "end_idx": 115, "type": "sym", "entity": "血尿" } ] }, { "text": "(四)其他肾性血尿相关化验血尿伴蛋白尿要进一步测定24小时尿蛋白定量,当尿蛋白>1g/24h多明确有肾实质病变;ASO、血补体C3及乙型肝炎相关抗原测定可鉴别肾炎性质;BUN、Cr及Ccr等指标可判断肾功能受损。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "肾性血尿相关化验" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "血尿伴蛋白尿" }, { "start_idx": 25, "end_idx": 33, "type": "ite", "entity": "24小时尿蛋白定量" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 36, "end_idx": 45, "type": "sym", "entity": "尿蛋白>1g/24h" }, { "start_idx": 50, "end_idx": 54, "type": "sym", "entity": "肾实质病变" }, { "start_idx": 56, "end_idx": 58, "type": "ite", "entity": "ASO" }, { "start_idx": 60, "end_idx": 64, "type": "ite", "entity": "血补体C3" }, { "start_idx": 66, "end_idx": 73, "type": "ite", "entity": "乙型肝炎相关抗原" }, { "start_idx": 79, "end_idx": 80, "type": "dis", "entity": "肾炎" }, { "start_idx": 84, "end_idx": 86, "type": "ite", "entity": "BUN" }, { "start_idx": 88, "end_idx": 89, "type": "ite", "entity": "Cr" }, { "start_idx": 91, "end_idx": 93, "type": "ite", "entity": "Ccr" }, { "start_idx": 100, "end_idx": 103, "type": "dis", "entity": "肾功能受" }, { "start_idx": 100, "end_idx": 103, "type": "pro", "entity": "肾功能受" } ] }, { "text": "(五)尿钙测定当随意尿Ca/Cr>0.21时,则进一步测定24小时尿钙定量,当尿Ca>4mg/(kg•d)则应疑为高钙尿症,应查2~3次才能确定。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "尿钙" }, { "start_idx": 10, "end_idx": 15, "type": "ite", "entity": "尿Ca/Cr" }, { "start_idx": 8, "end_idx": 21, "type": "sym", "entity": "随意尿Ca/Cr>0.21时" }, { "start_idx": 29, "end_idx": 36, "type": "ite", "entity": "24小时尿钙定量" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "尿Ca" }, { "start_idx": 39, "end_idx": 52, "type": "sym", "entity": "尿Ca>4mg/(kg•d)" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "高钙尿症" } ] }, { "text": "(六)尿细菌检查尿沉渣涂片找细菌、尿细菌计数及尿培养以确定泌尿系感染引起血尿的病因,反复发作者要除外伴有膀胱输尿管反流。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "尿细菌检查" }, { "start_idx": 8, "end_idx": 12, "type": "equ", "entity": "尿沉渣涂片" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "细菌" }, { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "尿细菌计数" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "尿培养" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "泌尿系" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "泌尿系感染" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "血尿" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "膀胱输尿管" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "膀胱输尿管反流" } ] }, { "text": "(七)特殊检查疑为结石引起做腹部平片;B型超声可观察肾脏大小、结构、肾静脉扩张、结石、畸形及肿物,对血尿诊断及鉴别诊断极为重要;静脉肾盂造影及膀胱逆行造影根据需要选用;数字减影血管造影可明确有无动静脉瘘、血管病变及血栓等;肾CT检查可除外占位性病变,但小儿应用较少。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "结石" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "腹部平片" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "B型超声" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肾脏" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "肾静脉" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "肾静脉扩张" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "结石" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "畸形" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "血尿" }, { "start_idx": 64, "end_idx": 69, "type": "pro", "entity": "静脉肾盂造影" }, { "start_idx": 71, "end_idx": 76, "type": "pro", "entity": "膀胱逆行造影" }, { "start_idx": 84, "end_idx": 91, "type": "pro", "entity": "数字减影血管造影" }, { "start_idx": 97, "end_idx": 100, "type": "dis", "entity": "动静脉瘘" }, { "start_idx": 102, "end_idx": 105, "type": "dis", "entity": "血管病变" }, { "start_idx": 107, "end_idx": 108, "type": "dis", "entity": "血栓" }, { "start_idx": 111, "end_idx": 115, "type": "ite", "entity": "肾CT检查" } ] }, { "text": "(八)肾活体组织检查可明确肾小球血尿的病因,对指导治疗及判断预后有一定帮助,以下指征可考虑做肾活体组织检查:①血尿伴尿蛋白定量>1~2g/24h,或伴高血压及氮质血症者;②伴持续补体C3下降者;③有肾炎家族史者;凡尿中的红细胞数量超过正常而无明确的临床症状、实验室改变及肾功能异常者,称为单纯性血尿,如持续半年以上,也应考虑肾活体组织检查。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肾小球" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "肾小球血尿" }, { "start_idx": 46, "end_idx": 52, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 55, "end_idx": 62, "type": "ite", "entity": "血尿伴尿蛋白定量" }, { "start_idx": 55, "end_idx": 71, "type": "sym", "entity": "血尿伴尿蛋白定量>1~2g/24h" }, { "start_idx": 75, "end_idx": 77, "type": "dis", "entity": "高血压" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "氮质血症" }, { "start_idx": 89, "end_idx": 92, "type": "bod", "entity": "补体C3" }, { "start_idx": 87, "end_idx": 94, "type": "sym", "entity": "持续补体C3下降" }, { "start_idx": 99, "end_idx": 100, "type": "dis", "entity": "肾炎" }, { "start_idx": 110, "end_idx": 114, "type": "ite", "entity": "红细胞数量" }, { "start_idx": 107, "end_idx": 139, "type": "sym", "entity": "尿中的红细胞数量超过正常而无明确的临床症状、实验室改变及肾功能异常" }, { "start_idx": 144, "end_idx": 148, "type": "sym", "entity": "单纯性血尿" }, { "start_idx": 162, "end_idx": 168, "type": "pro", "entity": "肾活体组织检查" } ] }, { "text": "【治疗和预后】血尿较重时适当注意休息,根据不同病因予以相应治疗。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "血尿" } ] }, { "text": "血尿是一个多病因的复杂问题,诊断不明确者应长期随访,特别是血尿伴蛋白尿者。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "血尿" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "血尿伴蛋白尿" } ] }, { "text": "二、诊断和鉴别诊断(一)临床诊断根据以上呼吸系统表现,加上神经系统、心血管、内脏功能变化的表现,结合血气分析分析,可以初步做出呼吸衰竭的临床诊断。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "神经系统" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "心血管" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "内脏" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "血气分析" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "(二)血气分析诊断一般认为在海平面大气压水平,吸入空气时,PaCO2>8kPa,PaO2<6.67kPa,提示呼吸衰竭。", "entities": [ { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "对于小儿急性和慢性呼吸衰竭的血气检查主要有以下特点。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "dis", "entity": "急性和慢性呼吸衰竭" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "血气检查" } ] }, { "text": "1.呼吸性酸中毒动脉血pH<7.35,PaCO2>7kPa,PaO2>8kPa,BE>-5mmol/L,>20mmol/L。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "呼吸性酸中毒" } ] }, { "text": "多见于急性梗阻性通气障碍、通气-灌流失调。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "急性梗阻性通气障碍" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "通气-灌流失调" } ] }, { "text": "2.混合性酸中毒动脉血pH<7.25,PaCO2>7kPa,PaO2<8kPa,BE<-5mmol/L,<20mmol/L。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "混合性酸中毒" } ] }, { "text": "多见于持续低氧血症伴通气、换气障碍,严重通气-灌流失调。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "低氧血症" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "通气、换气障碍" }, { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "严重通气-灌流失调" } ] }, { "text": "3.呼吸性碱中毒动脉血pH>7.45,PaCO2<4kPa,PaO2>8kPa,BE>5mmol/L,<20mmol/L。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "呼吸性碱中毒" } ] }, { "text": "多见于机械通气过度时。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "机械通气过度" } ] }, { "text": "4.代谢性酸中毒合并呼吸性碱中毒表现为动脉血pH<7.45,PaCO2<4kPa,PaO2>8kPa,BE<-5mmol/L,<20mmol/L。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "呼吸性碱中毒" } ] }, { "text": "可见于呼吸衰竭应用利尿剂后,以及机械通气纠正呼吸性酸中毒后。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "利尿剂" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "机械通气" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "呼吸性酸中毒" } ] }, { "text": "5.代谢性碱中毒合并呼吸性酸中毒发生代谢性碱中毒的原因与长时间应用碱液、呋塞米、甘露醇、肾上腺皮质激素等药物,吐泻引起的低钾,机械通气掌握不当,以及肾脏调节慢等有关。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "代谢性碱中毒" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "代谢性碱中毒" }, { "start_idx": 33, "end_idx": 34, "type": "dru", "entity": "碱液" }, { "start_idx": 36, "end_idx": 38, "type": "dru", "entity": "呋塞米" }, { "start_idx": 40, "end_idx": 42, "type": "dru", "entity": "甘露醇" }, { "start_idx": 44, "end_idx": 50, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "吐泻" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "低钾" } ] }, { "text": "6.氧合指数(oxygenationindex,OI)结合血气参数和机械通气参数可以判断呼吸衰竭的危重程度,可以采用OI[OI=FiO2×MAP×100/PaO2,MAP为平均气道压(cmH2O),可以从呼吸机直接读取,PaO2单位mmHg]。", "entities": [ { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 135, "end_idx": 137, "type": "equ", "entity": "呼吸机" } ] }, { "text": "OI<5,正常或轻度呼吸功能不全;OI=5~10,呼吸功能不全和呼吸衰竭,如果气体交换有明显障碍,需要机械通气;OI=10~20,中-重度呼吸衰竭,依赖机械通气;OI=20~30,严重呼吸衰竭,可能伴有肺内静-动脉分流,有应用气道滴入肺表面活性物质治疗指征。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "轻度呼吸功能不全" }, { "start_idx": 25, "end_idx": 35, "type": "dis", "entity": "呼吸功能不全和呼吸衰竭" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "气体交换有明显障碍" }, { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "机械通气" }, { "start_idx": 65, "end_idx": 72, "type": "dis", "entity": "中-重度呼吸衰竭" }, { "start_idx": 76, "end_idx": 79, "type": "pro", "entity": "机械通气" }, { "start_idx": 90, "end_idx": 95, "type": "dis", "entity": "严重呼吸衰竭" }, { "start_idx": 101, "end_idx": 101, "type": "bod", "entity": "肺" }, { "start_idx": 113, "end_idx": 114, "type": "bod", "entity": "气道" } ] }, { "text": "OI=30~40,严重呼吸衰竭伴有肺动脉高压和肺外右向左分流,有吸入一氧化氮,体外膜肺等特殊呼吸治疗、生命支持治疗指征。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 32, "end_idx": 37, "type": "pro", "entity": "吸入一氧化氮" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "体外膜肺" } ] }, { "text": "(三)鉴别诊断1.呼吸功能不全单纯使用血气值作为呼吸衰竭的诊断依据并不十分准确。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "呼吸功能不全" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "比如在吸入30%~40%氧后30~60分钟,患儿PaO2>8kPa,有可能为呼吸功能不全。", "entities": [ { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "呼吸功能不全" } ] }, { "text": "因此,在对呼吸困难症状出现时,采用持续非介入性正压通气、或气道插管机械通气和气道清洗使黏稠分泌物导致的气道阻塞复通后,呼吸困难症状迅速缓解。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "呼吸困难症状" }, { "start_idx": 17, "end_idx": 26, "type": "pro", "entity": "持续非介入性正压通气" }, { "start_idx": 29, "end_idx": 36, "type": "pro", "entity": "气道插管机械通气" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "气道清洗" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "气道阻塞" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "呼吸困难" } ] }, { "text": "因此,需要与单纯性原发于肺部或肺外疾病演变发展的严重呼吸困难加以区别。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肺部" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肺" } ] }, { "text": "2.急性呼吸窘迫综合征(ARDS)ARDS是与肺部和其他脏器感染等有关的急性肺部炎症损伤导致的临床综合征,因肺泡-毛细血管通透性增加而有严重肺水肿。", "entities": [ { "start_idx": 2, "end_idx": 16, "type": "dis", "entity": "急性呼吸窘迫综合征(ARDS)" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "ARDS" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺部" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "急性肺部炎症" }, { "start_idx": 70, "end_idx": 72, "type": "sym", "entity": "肺水肿" } ] }, { "text": "小儿ARDS多为急性起病,主要表现为呼吸窘迫症状,放射学检查为双侧肺弥漫性炎症和渗出改变,血气分析提示严重低氧血症,PaO2/FiO2<27kPa(200mmHg)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "小儿ARDS" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "呼吸窘迫症状" }, { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "放射学检查" }, { "start_idx": 31, "end_idx": 38, "type": "dis", "entity": "双侧肺弥漫性炎症" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "血气分析" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "严重低氧血症" } ] }, { "text": "可以合并严重肺内分流和肺动脉高压,应用常规机械通气往往效果差。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "机械通气" } ] }, { "text": "随着急救技术的提高和肺保护性策略的应用,临床预后已有明显改善。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "急救技术" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "肺保护性策略" } ] }, { "text": "3.感染性休克和全身性炎症反应综合征小儿感染性休克时可因心肌麻痹、肺血管痉挛、全身炎症反应时毒素刺激等,导致肺部严重损伤和呼吸功能障碍。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "感染性休克" }, { "start_idx": 8, "end_idx": 17, "type": "dis", "entity": "全身性炎症反应综合征" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "感染性休克" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "心肌麻痹" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "肺血管痉挛" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "全身炎症" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "肺部严重损伤" }, { "start_idx": 61, "end_idx": 66, "type": "dis", "entity": "呼吸功能障碍" } ] }, { "text": "此时应及时处理原发病因,采取抗感染和抗休克措施,解除导致呼吸功能障碍的主要原因。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "抗感染" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "抗休克措施" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "呼吸功能障碍" } ] }, { "text": "第八节肺的神经内分泌调节功能(一)呼吸道上皮细胞中的神经内分泌细胞分泌5-羟色胺,具有收缩血管、刺激肺呼吸作用在肺血管内皮被单胺氧化酶降解而灭活。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" }, { "start_idx": 17, "end_idx": 23, "type": "bod", "entity": "呼吸道上皮细胞" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "神经内分泌细胞" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "5-羟色胺" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "肺血管" }, { "start_idx": 62, "end_idx": 66, "type": "bod", "entity": "单胺氧化酶" } ] }, { "text": "血液中的去甲肾上腺素也在肺血管内皮中被单胺氧化酶和儿茶酚胺甲基转化酶降解。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血液" }, { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "去甲肾上腺素" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肺血管" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "单胺氧化酶" }, { "start_idx": 25, "end_idx": 33, "type": "bod", "entity": "儿茶酚胺甲基转化酶" } ] }, { "text": "循环中95%血管紧张素Ⅰ(AT-Ⅰ)在肺内皮细胞的血管紧张素转化酶作用下,由10肽变为8肽的AT-Ⅱ和7肽的AT-Ⅲ,活性分别提高50倍或25~30倍。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "血管紧张素Ⅰ" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "AT-Ⅰ" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "肺内皮细胞" }, { "start_idx": 25, "end_idx": 32, "type": "bod", "entity": "血管紧张素转化酶" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "AT-Ⅱ" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "AT-Ⅲ" } ] }, { "text": "肺内产生的或循环中的前列腺素如PGE、PGF也在肺血管内皮降解和灭活,但PGI2则不被灭活。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "前列腺素" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "PGE" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "PGF" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肺血管" }, { "start_idx": 36, "end_idx": 45, "type": "bod", "entity": "PGI2" } ] }, { "text": "(二)肥大细胞和IgE肥大细胞多分布在小支气管和细支气管黏膜浅层,其细胞质含有异染颗粒。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "肥大细胞" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "IgE" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "肥大细胞" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "小支气管" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "细支气管黏膜" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "细胞质" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "异染颗粒" } ] }, { "text": "肥大细胞膜表面有大量IgE的Fc段受体。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "肥大细胞膜" }, { "start_idx": 10, "end_idx": 18, "type": "bod", "entity": "IgE的Fc段受体" } ] }, { "text": "当IgE与受体结合后,肥大细胞即致敏,在同种小剂量抗原与细胞膜表面结合的IgE再次结合时,诱发经钙离子介导的细胞脱颗粒作用。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "IgE" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "肥大细胞" }, { "start_idx": 25, "end_idx": 26, "type": "mic", "entity": "抗原" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "细胞膜" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "IgE" } ] }, { "text": "(三)肺巨噬细胞巨噬细胞在肺组织内分布不同,肺泡内者称为肺泡巨噬细胞,直径20~40μm,具有活跃的吞噬功能,可以清除细菌、病毒、吸入物颗粒、衰老坏死的细胞,抗肿瘤,调节肺表面活性物质磷脂代谢。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "肺巨噬细胞" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肺组织" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肺泡" }, { "start_idx": 28, "end_idx": 33, "type": "bod", "entity": "肺泡巨噬细胞" }, { "start_idx": 59, "end_idx": 60, "type": "mic", "entity": "细菌" }, { "start_idx": 62, "end_idx": 63, "type": "mic", "entity": "病毒" }, { "start_idx": 85, "end_idx": 91, "type": "bod", "entity": "肺表面活性物质" } ] }, { "text": "巨噬细胞-粒细胞集落刺激因子调节巨噬细胞功能。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "巨噬细胞" } ] }, { "text": "先天性巨噬细胞-粒细胞集落刺激因子缺陷,可以使肺泡巨噬细胞无功能,导致肺泡磷脂代谢异常,可能为肺泡蛋白沉积症的发生原因。", "entities": [ { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "肺泡巨噬细胞" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肺泡" }, { "start_idx": 47, "end_idx": 53, "type": "dis", "entity": "肺泡蛋白沉积症" } ] }, { "text": "第五节新生儿呼吸窘迫综合征新生儿呼吸窘迫综合征(respiratorydistresssyndrome,RDS),也称为肺透明膜病(hyalinemembranedisease,HMD)。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "新生儿呼吸窘迫综合征" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "新生儿呼吸窘迫综合征" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "RDS" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "肺透明膜病" }, { "start_idx": 89, "end_idx": 91, "type": "dis", "entity": "HMD" } ] }, { "text": "其基本特点为肺发育不成熟、肺表面活性物质缺乏而导致的肺泡不张、肺液转运障碍、肺毛细血管-肺泡间高通透性渗出性病变。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肺" }, { "start_idx": 13, "end_idx": 19, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肺泡" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "肺泡不张" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肺液" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "肺液转运障碍" }, { "start_idx": 38, "end_idx": 45, "type": "bod", "entity": "肺毛细血管-肺泡" }, { "start_idx": 38, "end_idx": 55, "type": "sym", "entity": "肺毛细血管-肺泡间高通透性渗出性病变" } ] }, { "text": "以机械通气和肺表面活性物质替代疗法治疗为主的呼吸治疗和危重监护技术,已经能够使90%以上的RDS患儿存活。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "机械通气" }, { "start_idx": 6, "end_idx": 16, "type": "pro", "entity": "肺表面活性物质替代疗法" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "呼吸治疗" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "危重监护技术" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "RDS" } ] }, { "text": "【临床流行病学】RDS主要发生在早产儿,其发生率和严重程度与胎龄及出生体重呈反比。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "RDS" } ] }, { "text": "2006年,EuroNeoStat的数据显示RDS发病率在胎龄23~25周早产儿为91%,26~27周88%,28~29周74%,30~31周52%。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "RDS" } ] }, { "text": "RDS发病率占所有新生儿的1%,尤其多见于胎龄32周以下的早产儿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "RDS" } ] }, { "text": "美国资料显示,在胎龄29周内出生的早产儿中RDS的发病率可以高达60%,但在胎龄40周时基本不发生。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "RDS" } ] }, { "text": "发生RDS的高危因素包括男性、双胎,前一胎有RDS病史、母亲患糖尿病、剖宫产且无产程发动等。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "RDS" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "RDS" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "糖尿病" } ] }, { "text": "低龄怀孕、孕期吸烟、吸毒、药物、妊娠高血压等也与RDS发生相关。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "高血压" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "RDS" } ] }, { "text": "羊膜早破(分娩前24~48小时)则会降低RDS发生的危险性,可能为胎儿处于应激下,肾上腺激素分泌,促进了肺成熟;但一般认为胎儿宫内窘迫与RDS的发生没有直接关系,但会影响到早产儿生后早期的呼吸适应,如呼吸费力和肺液清除延缓等,其发生可以达50%。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "RDS" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "肾上腺激素" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "肺" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "RDS" }, { "start_idx": 100, "end_idx": 103, "type": "sym", "entity": "呼吸费力" }, { "start_idx": 105, "end_idx": 110, "type": "sym", "entity": "肺液清除延缓" } ] }, { "text": "肺表面活性物质可以降低RDS病死率。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "肺表面活性物质" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "RDS" } ] }, { "text": "Curosurf(固尔苏)临床研究中对照组病死率为50%,治疗组为30%,使RDS净存活率提高20%。", "entities": [ { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "RDS" } ] }, { "text": "20世纪90年代初的临床研究表明,肺表面活性物质治疗使RDS的生存率提高到75%,在多剂量治疗时可以提高到80%~90%。", "entities": [ { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "肺表面活性物质" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "RDS" } ] }, { "text": "美国在20世纪80年代末开始常规应用肺表面活性物质治疗RDS,在1989—1990年间1岁以下婴儿病死率由8.5%下降为6.3%,主要为RDS死亡率的下降。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "肺表面活性物质" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "RDS" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "RDS" } ] }, { "text": "【病因及发病机制】1.因肺发育不成熟,过低的表面活性物质使肺泡气液界面表面张力升高,肺泡萎陷,使功能余气量下降,肺顺应性曲线下移,顺应性下降,无效腔通气,呼吸做功显著增加,能量耗竭,导致全身脏器功能衰竭。", "entities": [ { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肺" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "表面活性物质" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肺泡" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "肺泡" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "肺" }, { "start_idx": 93, "end_idx": 100, "type": "sym", "entity": "全身脏器功能衰竭" } ] }, { "text": "2.不成熟肺的肺泡数量和通气面积太少,肺泡间隔宽,气体弥散和交换严重不足。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "肺" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肺泡" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肺泡" } ] }, { "text": "3.呼气末肺泡萎陷,通气困难,出现低氧血症,使肺泡上皮细胞合成表面活性物质能力下降。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肺泡" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "低氧血症" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 31, "end_idx": 36, "type": "bod", "entity": "表面活性物质" } ] }, { "text": "4.持续低氧导致肺血管痉挛,出现肺动脉高压,肺血流减少,肺外右向左分流,肺内动静脉分流,使通气-灌流比例失调,影响气血交换。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "持续低氧" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肺血管痉挛" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "肺动脉" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "肺动脉高压" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肺" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肺" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "肺内动静脉" }, { "start_idx": 22, "end_idx": 42, "type": "sym", "entity": "肺血流减少,肺外右向左分流,肺内动静脉分流" } ] }, { "text": "5.持续低氧和酸中毒可以造成心肌损害,心输出量下降,全身性低血压、低灌流,最后出现以呼吸衰竭为主的多脏器衰竭。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "持续低氧" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "酸中毒" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "心肌损害" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "心输出量下降" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "低血压" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "全身性低血压" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "低灌流" }, { "start_idx": 41, "end_idx": 53, "type": "sym", "entity": "以呼吸衰竭为主的多脏器衰竭" } ] }, { "text": "【病理组织学】大体解剖时,肺多为实变,外观显暗红色,水中下沉。", "entities": [ { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肺" } ] }, { "text": "机械通气后的肺泡可以局部扩张,未经机械通气的RDS患儿肺主要表现为不张、充血和水肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "机械通气" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肺泡" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "机械通气" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "RDS" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "肺" }, { "start_idx": 27, "end_idx": 40, "type": "sym", "entity": "肺主要表现为不张、充血和水肿" } ] }, { "text": "显微镜下肺泡萎陷,上皮细胞多立方状、少扁平状,肺泡间隔宽、充气少,细小支气管、肺泡导管和肺泡扩张,上皮细胞脱落坏死,有呈嗜伊红色膜内衬,为透明膜形成。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "显微镜" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肺泡" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺泡" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肺泡" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "肺泡" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 59, "end_idx": 66, "type": "sym", "entity": "呈嗜伊红色膜内衬" } ] }, { "text": "已经通过气的肺则主要为小气道损伤,为肺泡不张的继发性改变。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肺" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "小气道损伤" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肺泡" } ] }, { "text": "肺微血管和毛细血管中可以有血栓形成、出血。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肺微血管" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "毛细血管" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "血栓形成、出血" } ] }, { "text": "【病理生理】由于肺表面活性物质的分泌合成作用下降,肺表面活性物质再循环途径的阻断,或者因肺泡腔内液体过多(转运障碍、高渗出),均可以使肺表面活性物质不足。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 25, "end_idx": 31, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "肺泡" }, { "start_idx": 67, "end_idx": 73, "type": "bod", "entity": "肺表面活性物质" } ] }, { "text": "病理性渗出液含大量血浆蛋白,在肺泡腔内干扰和抑制肺表面活性物质功能。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "血浆蛋白" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺泡" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "肺表面活性物质" } ] }, { "text": "出生时吸入、肺炎、肺发育不良、肺出血以及窒息缺氧性损害等出生早期病况均可与上述病理生理相关。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肺炎" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "肺发育不良" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肺出血" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "窒息缺氧性损害" } ] }, { "text": "早产儿肺内肺表面活性物质的磷脂总量只有足月儿的10%~30%或更低,且缺乏SP-A、B、C等主要肺表面活性物质蛋白,因而在数量和质量上均劣于足月儿,是发生RDS的主要原因。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" }, { "start_idx": 5, "end_idx": 11, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 48, "end_idx": 56, "type": "bod", "entity": "肺表面活性物质蛋白" }, { "start_idx": 77, "end_idx": 79, "type": "dis", "entity": "RDS" } ] }, { "text": "应用外源性肺表面活性物质制剂可以迅速提高肺内的肺表面活性物质含量。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dru", "entity": "外源性肺表面活性物质制剂" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "肺" }, { "start_idx": 23, "end_idx": 29, "type": "bod", "entity": "肺表面活性物质" } ] }, { "text": "将肺表面活性物质经气道滴入RDS患儿肺内后,肺表面活性物质磷脂会立即被肺泡上皮细胞摄取,并逐渐强化内源性肺表面活性物质的功能活性,特别是促使SP-A、B、C的合成分泌。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "气道" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "RDS" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肺内" }, { "start_idx": 22, "end_idx": 30, "type": "bod", "entity": "肺表面活性物质磷脂" }, { "start_idx": 35, "end_idx": 40, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 52, "end_idx": 58, "type": "bod", "entity": "肺表面活性物质" } ] }, { "text": "【临床表现】RDS主要发生在早产儿,尤其在胎龄小于32周、出生体重低于2000g的早产儿。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "RDS" } ] }, { "text": "可以是刚一出生即出现症状或出生后6小时内发病,表现为呼吸困难症状,如呼吸频率加快(>60次/分)或呼吸浅弱,鼻翼扇动,呼气呻吟,锁骨上、肋间和胸骨下吸气性凹陷(“三凹征”),青紫。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "呼吸频率加快" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "呼吸浅弱" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "鼻翼" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "鼻翼扇动" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "呼气呻吟" }, { "start_idx": 64, "end_idx": 85, "type": "sym", "entity": "锁骨上、肋间和胸骨下吸气性凹陷(“三凹征”)" }, { "start_idx": 87, "end_idx": 88, "type": "sym", "entity": "青紫" } ] }, { "text": "这类症状呈进行性加重,并可发生呼吸暂停。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "呼吸暂停" } ] }, { "text": "典型的X线胸片显示RDS早期的肺部网状细颗粒影和后期的毛玻璃状(“白肺”)征象以及相对增强的支气管充气征,伴早产儿胸廓和肺容积偏小特征。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "X线胸片" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "RDS" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺部" } ] }, { "text": "血气分析显示酸中毒、低氧血症和高碳酸血症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "血气分析" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "酸中毒" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "低氧血症" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "高碳酸血症" } ] }, { "text": "如果持续低氧血症和酸中毒不能纠正,患儿可以并发肺动脉高压、呼吸与心力衰竭,可在48~72小时内死亡。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "低氧血症" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "酸中毒" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "呼吸与心力衰竭" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "死亡" } ] }, { "text": "经辅助或强制通气的患儿在3~5天后,随内源性肺表面活性物质增多,症状会好转,表现为自限性恢复的特点。", "entities": [ { "start_idx": 19, "end_idx": 26, "type": "bod", "entity": "内源性肺表面活性" } ] }, { "text": "【实验室检查】(一)卵磷脂/鞘磷脂比(L/S)羊水中L/S比值<1,胎儿发生RDS危险性可达100%;L/S>2,发生RDS的危险性<1%。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "L/S" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "RDS" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "RDS" } ] }, { "text": "同一胎龄小儿的L/S可以变化很大,因此单纯用L/S不能判断是否发生RDS,但可以作为预防的指征。", "entities": [ { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "RDS" } ] }, { "text": "羊水中磷脂酰甘油(PG)和SP-A也可以作为判断肺成熟的辅助指标,两者在接近出生前偏低,提示肺不成熟。", "entities": [ { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "肺" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "肺" } ] }, { "text": "在肺不成熟的胎儿,如果L/S、PG、SP-A均很低,发生RDS的危险性非常高。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肺" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "RDS" } ] }, { "text": "测定气道吸出液或出生后早期胃液的以上指标,也可以辅助判断RDS治疗效果及转归。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "气道" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胃液" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "RDS" } ] }, { "text": "也有研究应用显微镜微泡计数法,检测气道清洗液或胃液中微小气泡与大气泡比例,间接判断内源性肺表面活性物质含量与活性,可有助于床旁快速判断RDS疾病程度和治疗效果。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "equ", "entity": "显微镜" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "气道" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "胃液" }, { "start_idx": 41, "end_idx": 50, "type": "bod", "entity": "内源性肺表面活性物质" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "RDS" } ] }, { "text": "(二)血气分析为最主要实验室检查。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "血气分析" } ] }, { "text": "患儿呼吸治疗时必须测定动脉血氧分压(PaO2)、二氧化碳分压(PaCO2)和pH。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "ite", "entity": "动脉血氧分压" }, { "start_idx": 30, "end_idx": 35, "type": "ite", "entity": "二氧化碳分压" }, { "start_idx": 50, "end_idx": 51, "type": "ite", "entity": "pH" } ] }, { "text": "发病早期,PaO2<6.5kPa(50mmHg),PaCO2>8kPa(60mmHg),pH<7.20,BE<-5.0mmol/L,应考虑低氧血症、高碳酸血症、代谢性酸中毒,经吸氧或辅助通气治疗无改善,可转为气道插管和呼吸机治疗,避免发生严重呼吸衰竭。", "entities": [ { "start_idx": 56, "end_idx": 57, "type": "ite", "entity": "pH" }, { "start_idx": 81, "end_idx": 84, "type": "dis", "entity": "低氧血症" }, { "start_idx": 86, "end_idx": 90, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 92, "end_idx": 97, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 100, "end_idx": 101, "type": "pro", "entity": "吸氧" }, { "start_idx": 103, "end_idx": 108, "type": "pro", "entity": "辅助通气治疗" }, { "start_idx": 116, "end_idx": 119, "type": "pro", "entity": "气道插管" }, { "start_idx": 121, "end_idx": 125, "type": "pro", "entity": "呼吸机治疗" }, { "start_idx": 131, "end_idx": 136, "type": "sym", "entity": "严重呼吸衰竭" } ] }, { "text": "一般在开始机械通气后1~3小时以及随后2~3天的每12~24小时,需要检查动脉血气值,以判断病情转归和调节呼吸机参数,以保持合适的通气量和氧供。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "机械通气" }, { "start_idx": 37, "end_idx": 40, "type": "ite", "entity": "动脉血气" }, { "start_idx": 53, "end_idx": 55, "type": "equ", "entity": "呼吸机" } ] }, { "text": "【诊断与鉴别诊断】根据上述临床表现及胸部X线的表现,诊断不难。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "胸部X线" } ] }, { "text": "需要鉴别诊断的疾病有:(一)新生儿湿肺又称暂时性呼吸困难或肺液转运障碍。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "新生儿湿肺" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "暂时性呼吸困难" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "肺液转运障碍" } ] }, { "text": "X线胸片特征为肺门纹理增强,肺泡、叶间、间质积液,肺血管充血,肺气肿等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "X线胸片" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肺门" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肺泡" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "叶间" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "间质" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "间质积液" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肺血管" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "肺血管充血" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "肺气肿" } ] }, { "text": "如果经吸氧临床症状没有改善或吸氧加重时,宜采用持续气道正压通气(CPAP)或气道插管机械通气治疗,一般24~72小时X线检查见肺液快速吸收和呼吸急促症状的缓解。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "吸氧" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "吸氧" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "正压通气" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "CPAP" }, { "start_idx": 38, "end_idx": 47, "type": "pro", "entity": "气道插管机械通气治疗" }, { "start_idx": 58, "end_idx": 61, "type": "pro", "entity": "X线检查" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "肺液" }, { "start_idx": 63, "end_idx": 68, "type": "sym", "entity": "肺液快速吸收" }, { "start_idx": 70, "end_idx": 78, "type": "sym", "entity": "呼吸急促症状的缓解" } ] }, { "text": "(二)B族溶血性链球菌(GBS)肺炎可见于早产、近足月和足月新生儿,母亲妊娠后期有感染及羊膜早破史,临床发病特点同早产儿RDS,可以有细菌培养阳性。", "entities": [ { "start_idx": 3, "end_idx": 17, "type": "dis", "entity": "B族溶血性链球菌(GBS)肺炎" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "感染" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "羊膜早破" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "RDS" } ] }, { "text": "胸部X线检查表现为肺叶或节段炎症特征及肺泡萎陷征,临床有感染征象,病程1~2周。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肺叶" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "肺叶或节段炎症" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "肺泡萎陷征" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "感染" } ] }, { "text": "治疗以出生后最初3天采用联合广谱抗生素,如氨苄西林加庆大霉素,随后应用7~10天氨苄西林或青霉素,剂量要參考最小抑菌浓度,避免因剂量偏低导致失去作用。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dru", "entity": "联合广谱抗生素" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "庆大霉素" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "青霉素" } ] }, { "text": "(三)遗传性SP-B缺乏症又称为“先天性肺表面活性物质蛋白缺乏症”,于1993年在美国发现,目前全世界有100多例经分子生物学技术诊断明确的患儿。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "遗传性SP-B缺乏症" }, { "start_idx": 17, "end_idx": 31, "type": "dis", "entity": "先天性肺表面活性物质蛋白缺乏症" } ] }, { "text": "发病原因为调控SP-B合成的DNA序列碱基突变。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "SP-B" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "DNA" } ] }, { "text": "临床上表现为足月出生的小儿出现进行性呼吸困难,经任何治疗干预无效。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "进行性呼吸困难" } ] }, { "text": "肺病理表现类似早产儿RDS,肺活检发现SP-B蛋白和SP-BmRNA缺乏,并可以伴前SP-C合成与表达的异常,其肺组织病理类似肺泡蛋白沉积症。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "早产儿RDS" }, { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "肺活检" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "SP-B蛋白" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "SP-BmRNA" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "肺组织" }, { "start_idx": 63, "end_idx": 69, "type": "dis", "entity": "肺泡蛋白沉积症" } ] }, { "text": "外源性肺表面活性物质治疗仅能暂时缓解症状,患儿多依赖肺移植,否则多在1岁内死亡。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "外源性肺表面活性物质" }, { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "肺移植" } ] }, { "text": "【预防】预防RDS的主要手段包括预期产程并及时做好接生和早产儿复苏急救准备,还可以通过产前评估、产前母体糖皮质激素以及出生后肺表面活性物质的预防性给药,达到预防RDS发生的目的。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "RDS" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "糖皮质激素" }, { "start_idx": 62, "end_idx": 68, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 80, "end_idx": 82, "type": "dis", "entity": "RDS" } ] }, { "text": "肺表面活性物质在妊娠22~24周胎儿肺中出现,25周左右已可在羊水中检测出,在32~35周大量合成。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肺" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "羊水" } ] }, { "text": "肺表面活性物质在足月出生的新生儿肺内非常丰富,且具有很高的表面活性,但在32周以下出生的早产儿,特别是28周以下出生、体重低于1000g的超低出生体重儿,60%~80%可以发生呼吸窘迫。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肺内" }, { "start_idx": 88, "end_idx": 91, "type": "sym", "entity": "呼吸窘迫" } ] }, { "text": "产前给予糖皮质激素治疗,一般产前使用激素的最佳时间为分娩前24小时~7天,给予地塞米松每次6mg,2~4次,每次间隔12~24小时;或倍他米松,每次12mg,每天1次,共2次,可以显著降低24~34周早产新生儿RDS发生率和新生儿死亡接近50%,并可以减少新生儿脑室内出血。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "pro", "entity": "糖皮质激素治疗" }, { "start_idx": 39, "end_idx": 42, "type": "dru", "entity": "地塞米松" }, { "start_idx": 105, "end_idx": 107, "type": "dis", "entity": "RDS" }, { "start_idx": 131, "end_idx": 133, "type": "bod", "entity": "脑室内" }, { "start_idx": 128, "end_idx": 135, "type": "sym", "entity": "新生儿脑室内出血" } ] }, { "text": "对于早产儿出生后立即预防性气道内给予肺表面活性物质可以减少RDS发生。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "RDS" } ] }, { "text": "【治疗】(一)辅助呼吸治疗1.氧疗可以部分改善低氧血症,其作用原理为提高局部通气-灌流差的肺泡内氧分压,使局部痉挛血管舒张,减少右向左分流,提高动脉氧饱和度。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "辅助呼吸治疗" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "氧疗" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "低氧血症" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "肺泡" }, { "start_idx": 48, "end_idx": 50, "type": "ite", "entity": "氧分压" } ] }, { "text": "持续高氧(FiO2>0.5)24小时以上可以导致肺水肿和炎症,严重者出现支气管发育不良(BPD)和眼球后视神经血管损害。", "entities": [ { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "肺水肿" }, { "start_idx": 47, "end_idx": 53, "type": "dis", "entity": "支气管发育不良" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "BPD" }, { "start_idx": 60, "end_idx": 69, "type": "dis", "entity": "眼球后视神经血管损害" } ] }, { "text": "2.经鼻持续气道正压通气(CPAP)简易水封瓶CPAP装置,或带有湿化器的专用CPAP装置产品,比较简单,使用方便,但存在氧浓度无法控制和调节、压力不稳定、易诱发气胸等并发症的缺点。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "pro", "entity": "经鼻持续气道正压通气" }, { "start_idx": 23, "end_idx": 28, "type": "equ", "entity": "CPAP装置" }, { "start_idx": 33, "end_idx": 35, "type": "equ", "entity": "湿化器" }, { "start_idx": 39, "end_idx": 44, "type": "equ", "entity": "CPAP装置" }, { "start_idx": 81, "end_idx": 82, "type": "dis", "entity": "气胸" } ] }, { "text": "CPAP装置供氧浓度连续可调(21%~100%),气流流量可变(0~12L/min),并具有供气压力上限报警和安全卸压(11cmH2O)阀门装置,在治疗中可以保持供气压力稳定,显著提高使用的安全性和有效性,减少气胸等并发症,尤其适用于<1500g体重的早产儿和极低出生体重儿。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "equ", "entity": "CPAP装置" }, { "start_idx": 111, "end_idx": 112, "type": "dis", "entity": "气胸" } ] }, { "text": "经1~3天治疗后,如果PEEP可以下调至0~1cmH2O以下,供氧浓度在25%以下,仍可维持SpO2达到88%~93%,可以转为短时间头罩吸氧至停止呼吸治疗。", "entities": [ { "start_idx": 79, "end_idx": 82, "type": "pro", "entity": "头罩吸氧" }, { "start_idx": 86, "end_idx": 89, "type": "pro", "entity": "呼吸治疗" } ] }, { "text": "3.气道插管和呼吸机治疗应用指征一般考虑经头罩或CPAP治疗6~12小时以上病情无改善,且继续加重,可以考虑气道插管和机械通气。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "气道插管" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "呼吸机治疗" }, { "start_idx": 21, "end_idx": 22, "type": "equ", "entity": "头罩" }, { "start_idx": 24, "end_idx": 29, "type": "pro", "entity": "CPAP治疗" }, { "start_idx": 54, "end_idx": 57, "type": "pro", "entity": "气道插管" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "机械通气" } ] }, { "text": "临床采用机械通气的一般原则为:FiO2>0.5,呼吸机参数设定为吸气时间(Ti)最初在0.3~0.4秒,呼气末正压(PEEP)在3~6cmH2O,通气频率(f)为50~60次/分,气道峰压(PIP)在20~30cmH2O,以可见胸廓运动为适宜,潮气量(VT)通气6~8ml/kg体重,达到PaO2在50~70mmHg,PaCO2在45~55mmHg。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "机械通气" }, { "start_idx": 35, "end_idx": 37, "type": "equ", "entity": "呼吸机" } ] }, { "text": "如果出现呼吸对抗,可以考虑采用镇静剂和肌肉松弛剂,或调节同步触发通气。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "镇静剂" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "肌肉松弛剂" } ] }, { "text": "严重呼吸衰竭时伴有肺动脉高压者,可以吸入一氧化氮(NO),高频振荡通气(HFOV)也可以治疗早产儿RDS,在缺乏肺表面活性物质制剂或常频机械通气效果不良时选用HFOV,可能迅速改善通气障碍,缩短呼吸机治疗时间,并降低CLD发生危险性。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "呼吸衰竭" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "一氧化氮" }, { "start_idx": 25, "end_idx": 26, "type": "dru", "entity": "NO" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "高频振荡通气" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "HFOV" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "早产儿RDS" }, { "start_idx": 56, "end_idx": 64, "type": "dru", "entity": "肺表面活性物质制剂" }, { "start_idx": 66, "end_idx": 71, "type": "pro", "entity": "常频机械通气" }, { "start_idx": 79, "end_idx": 82, "type": "pro", "entity": "HFOV" }, { "start_idx": 97, "end_idx": 99, "type": "equ", "entity": "呼吸机" } ] }, { "text": "参数调节原则上以动脉PaCO2不出现急剧变化为适宜,避免导致脑血流迅速下降,诱发继发性缺血缺氧性脑损伤。", "entities": [ { "start_idx": 51, "end_idx": 61, "type": "dis", "entity": "继发性缺血缺氧性脑损伤" } ] }, { "text": "(二)液体治疗由于RDS早期有肺液转运障碍和肺血管高通透性水肿,出生后最初3天进液量可以控制在50~70ml/(kg•d),然后逐渐提高到80~100ml/(kg•d)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "液体治疗" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "RDS" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺液" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "肺液转运障碍" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肺血管" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "水肿" }, { "start_idx": 22, "end_idx": 30, "type": "sym", "entity": "肺血管高通透性水肿" } ] }, { "text": "密切监测血电解质,酌情给予钠盐,避免因皮肤薄、非显性失水等原因导致高钠血症和脑损害。", "entities": [ { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "高钠血症" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "脑损害" } ] }, { "text": "在用补液治疗高钠血症时,可能会导致高血糖,可以视情况经胃管输入液体。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dru", "entity": "补液" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "高钠血症" }, { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "血糖" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "高血糖" } ] }, { "text": "补充胶体液亦应谨慎,因由于高血管通透性会使输入蛋白沉着于肺间质,使间质胶体渗透压增加,加重间质肺液滞留。", "entities": [ { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "肺间质" } ] }, { "text": "碳酸氢钠液可以稀释后缓慢静脉推注,不主张持续滴注。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "碳酸氢钠液" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "静脉推注" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "持续滴注" } ] }, { "text": "RDS患儿会因低氧血症使细胞钠-钾ATP酶功能低下和肾功能不全,出现高钾血症,因此出生早期不必补钾。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "RDS" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "低氧血症" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "高钾血症" } ] }, { "text": "出生后会出现短时间甲状旁腺功能低下,可以适当补充钙剂。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "dru", "entity": "钙剂" } ] }, { "text": "(三)血压维持早产儿外周血压低于30mmHg时,脑血流低灌注可以导致脑损伤。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "血压维持" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "血压" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "脑损伤" } ] }, { "text": "低血压可能与血容量过低有关。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "血压" }, { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "低血压" } ] }, { "text": "可以按10~20ml/kg输入血浆等液体以提高血压,同时给予多巴胺和多巴酚丁胺5~15μg/(kg•min)。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dru", "entity": "血浆" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "血压" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "多巴胺" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "多巴酚丁胺" } ] }, { "text": "纠正低血压要避免剧烈血压波动,否则会诱发脑出血。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "低血压" }, { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "血压" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "脑出血" } ] }, { "text": "在有肺动脉高压时,目前不主张用全身性扩张血管药物,因可造成全身血管舒张导致低血压。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺动脉" }, { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "肺动脉高压" }, { "start_idx": 29, "end_idx": 39, "type": "sym", "entity": "全身血管舒张导致低血压" } ] }, { "text": "可以考虑应用关闭动脉导管药物和吸入NO等治疗方式。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "pro", "entity": "关闭动脉导管药物" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "吸入NO" } ] }, { "text": "(四)护理对极低体重新生儿RDS,可通过伺服控制方式,调节环境温度在36.5~37℃,控制肛温在37℃。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "护理" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "新生儿RDS" }, { "start_idx": 45, "end_idx": 46, "type": "ite", "entity": "肛温" } ] }, { "text": "不主张反复气道吸引、改变体位等护理,以减少因过多刺激带来脑血流剧烈波动导致颅内出血。", "entities": [ { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "颅内出血" } ] }, { "text": "动脉留置导管主要在发病早期稳定后应该及时拔掉,避免医源性损害。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "equ", "entity": "动脉留置导管" } ] }, { "text": "俯卧位可以应用于机械通气时,可以促进背部肺泡扩张,改善局部肺泡的通气灌流失调。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "机械通气" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肺泡" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肺泡" } ] }, { "text": "(五)营养在RDS急性期不给予脂肪乳剂,因脂肪乳剂会对于低氧性肺血流下降产生不利影响。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "营养" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "RDS" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "脂肪乳剂" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "脂肪乳剂" } ] }, { "text": "对于贫血者,可以输血和补充红细胞成分等,保持红细胞压积在40%~50%。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "贫血" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "输血" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "补充红细胞成分" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "红细胞" } ] }, { "text": "(六)抗生素应用如果考虑为GBS感染,在做血培养后应用氨苄西林和庆大霉素预防性治疗。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "抗生素" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "GBS感染" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "庆大霉素" } ] }, { "text": "如果血培养阴性,外周白细胞计数为正常范围,可以停用抗生素。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "抗生素" } ] }, { "text": "一般应用抗生素为1周。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "抗生素" } ] }, { "text": "如果母亲在分娩前已经应用过抗生素,对血培养阴性者必须根据临床状况处理。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "抗生素" } ] }, { "text": "对于呼吸机治疗过程中出现气道清洗液培养细菌阳性,可以根据是否为致病菌和药敏试验结果来决定抗生素是否应用。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "呼吸机" }, { "start_idx": 44, "end_idx": 46, "type": "equ", "entity": "抗生素" } ] }, { "text": "(七)肺表面活性物质治疗20世纪80~90年代,国际儿科新生儿医学最突出成果是应用外源性肺表面活性物质(pulmonarysurfactant)对RDS的研究在临床预防和治疗的成功。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "肺表面活性物质治疗" }, { "start_idx": 26, "end_idx": 27, "type": "dep", "entity": "儿科" }, { "start_idx": 41, "end_idx": 50, "type": "dru", "entity": "外源性肺表面活性物质" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "RDS" } ] }, { "text": "1959年,美国MaryEllenAvery医师首次提出HMD的病因是肺表面活性物质缺乏。", "entities": [ { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "HMD" }, { "start_idx": 35, "end_idx": 41, "type": "bod", "entity": "肺表面活性物质" } ] }, { "text": "1980年,日本藤原泽郎(TetsuroFujiwara)医师首次报道了应用牛肺表面活性物质制剂治疗10例HMD成功。", "entities": [ { "start_idx": 38, "end_idx": 47, "type": "dru", "entity": "牛肺表面活性物质制剂" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "HMD" } ] }, { "text": "1990年以来,发达国家和地区已普遍应用肺表面活性物质预防和治疗RDS。", "entities": [ { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "RDS" } ] }, { "text": "1.肺表面活性物质制剂目前国外常规应用的肺表面活性物质制剂为牛和猪肺提取物,富含磷脂和一定量的SP-B和C,不含SP-A,其中以Survanta(牛肺,美国)、Infasurf(小牛肺、美国)、Curosurf(猪肺,意大利)为代表。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dru", "entity": "肺表面活性物质制剂" }, { "start_idx": 20, "end_idx": 28, "type": "dru", "entity": "肺表面活性物质制剂" } ] }, { "text": "肺表面活性物质制剂应用指征仅限于新生儿RDS,但也有应用于新生儿和婴幼儿肺部炎症、吸入性损伤等的报道,有一定疗效。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dru", "entity": "肺表面活性物质制剂" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "新生儿RDS" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "吸入性损伤" } ] }, { "text": "外源性肺表面活性物质的代谢主要为肺泡Ⅱ型上皮细胞的摄取和再利用。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "外源性肺表面活性物质" }, { "start_idx": 16, "end_idx": 23, "type": "bod", "entity": "肺泡Ⅱ型上皮细胞" } ] }, { "text": "动物研究显示治疗剂量的肺表面活性物质磷脂的生物半衰期为30~40小时,肺内清除速率为每小时2%~4%。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "bod", "entity": "肺表面活性物质磷脂" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肺内" } ] }, { "text": "应用稳定同位素的人体研究发现,新生儿肺通过摄取原料合成肺表面活性物质磷脂(磷脂酰胆碱)的速率为每天肺内总量的2%~4%,或4.2mg/(kg•d),但半衰期长达5~6天。", "entities": [ { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肺" }, { "start_idx": 27, "end_idx": 35, "type": "bod", "entity": "肺表面活性物质磷脂" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "磷脂酰胆碱" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "肺" } ] }, { "text": "2.肺表面活性物质预防性治疗RDS的指征出生体重1000克以下常规应用,一般在出生后15~30分钟气道插管后滴入100mg/kg,以防止RDS的发生。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "RDS" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "气道插管" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "RDS" } ] }, { "text": "临床试验的的结论表明对于部分婴儿是有利的,但从经济上看,可能对相当一部分原本不发生RDS的婴儿做了不必要的治疗,因而不主张广泛使用,而局限于对小胎龄极低出生体重儿和珍贵儿有选择地使用。", "entities": [ { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "RDS" } ] }, { "text": "对于胎龄在30~35周、中度呼吸困难的RDS患儿,即使单纯呼吸机治疗,也可以在3~4天后恢复,而不需要依赖外源性表面活性物质治疗。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "中度呼吸困难" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "RDS" }, { "start_idx": 29, "end_idx": 31, "type": "equ", "entity": "呼吸机" }, { "start_idx": 53, "end_idx": 61, "type": "dru", "entity": "外源性表面活性物质" } ] }, { "text": "3.表面活性物质救治性(rescue)治疗RDS的指征对于已经出现RDS临床征象的早产儿,可以在机械通气下气道滴入100~200mg/kg,并调节呼吸机参数,保持合适的通气压力,避免出现气漏等并发症。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "表面活性物质" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "RDS" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "RDS" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "机械通气" }, { "start_idx": 73, "end_idx": 75, "type": "equ", "entity": "呼吸机" } ] }, { "text": "肺表面活性物质治疗的疗效首先为用药后短时期内氧合状况的改善。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "肺表面活性物质治疗" } ] }, { "text": "可以表现为血氧分压的迅速提高,一般给药后几分钟到1~2小时内可以使动脉氧分压提高50%以上,吸入氧浓度下调10%~20%以上。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "ite", "entity": "血氧分压" }, { "start_idx": 35, "end_idx": 37, "type": "ite", "entity": "氧分压" } ] }, { "text": "相应的可以将机械通气的吸气峰压减少3~4cmH2O。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "机械通气" } ] }, { "text": "疗效不佳的原因,除了表面活性物质制剂本身外,主要与RDS肺内有肺表面活性物质耗竭,缺氧对肺泡组织细胞合成肺表面活性物质的抑制,肺泡毛细血管高通透性致大量血浆蛋白渗出,抑制内源性肺表面活性物质活性有关。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dru", "entity": "表面活性物质制剂" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "RDS" }, { "start_idx": 31, "end_idx": 37, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "缺氧" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "肺泡组织细胞" }, { "start_idx": 52, "end_idx": 58, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 63, "end_idx": 68, "type": "bod", "entity": "肺泡毛细血管" }, { "start_idx": 85, "end_idx": 94, "type": "bod", "entity": "内源性肺表面活性物质" } ] }, { "text": "(一)支气管肺发育不良(bronchopulmonarydysplasia,BPD)为继发性慢性肺部病变,早产儿特别是经较长时间氧疗和机械通气可诱发,表现为生后2~3周对机械通气和吸入氧的依赖,严重病例肺部有放射学上纤维化的表现。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "支气管肺发育不良" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "BPD" }, { "start_idx": 43, "end_idx": 51, "type": "dis", "entity": "继发性慢性肺部病变" }, { "start_idx": 64, "end_idx": 65, "type": "pro", "entity": "氧疗" }, { "start_idx": 67, "end_idx": 70, "type": "pro", "entity": "机械通气" }, { "start_idx": 85, "end_idx": 88, "type": "pro", "entity": "机械通气" }, { "start_idx": 101, "end_idx": 102, "type": "bod", "entity": "肺部" }, { "start_idx": 101, "end_idx": 110, "type": "sym", "entity": "肺部有放射学上纤维化" } ] }, { "text": "呋塞米,静脉1mg/kg,一天2次,口服2mg/(kg•d);氢氯噻嗪,2mg/kg,一天2次,与氯化钾同时服用;氨茶碱剂量控制以血浓度保持为12~15mg/L为安全有效。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "呋塞米" }, { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "静脉" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "氢氯噻嗪" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "氯化钾" }, { "start_idx": 57, "end_idx": 59, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 65, "end_idx": 67, "type": "ite", "entity": "血浓度" } ] }, { "text": "地塞米松治疗在出生后第4周开始,0.25mg/kg,一天2次,每1~2天剂量减半至0.01~0.02mg/kg,一天2次,总疗程在5~7天,以尽量减少皮质激素的不良反应,如高血糖、消化道出血、肾上腺皮质功能抑制、败血症、生长迟缓等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "地塞米松" }, { "start_idx": 75, "end_idx": 78, "type": "dru", "entity": "皮质激素" }, { "start_idx": 86, "end_idx": 88, "type": "sym", "entity": "高血糖" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "消化道" }, { "start_idx": 90, "end_idx": 94, "type": "sym", "entity": "消化道出血" }, { "start_idx": 96, "end_idx": 100, "type": "bod", "entity": "肾上腺皮质" }, { "start_idx": 96, "end_idx": 104, "type": "sym", "entity": "肾上腺皮质功能抑制" }, { "start_idx": 106, "end_idx": 108, "type": "dis", "entity": "败血症" }, { "start_idx": 110, "end_idx": 113, "type": "sym", "entity": "生长迟缓" } ] }, { "text": "如果皮质激素治疗7天无效,应放弃该疗法。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "皮质激素" } ] }, { "text": "治疗效果以小儿依赖呼吸机和高氧治疗的状况缓解、体重增加、没有感染等并发症来判断。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "equ", "entity": "呼吸机" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "高氧治疗" }, { "start_idx": 13, "end_idx": 21, "type": "sym", "entity": "高氧治疗的状况缓解" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "体重增加" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "没有感染" } ] }, { "text": "(二)气胸及纵隔气漏气胸和气漏(纵隔气肿、间质气肿)是RDS的主要并发症,一般需要行胸腔插管闭式引流。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "气胸" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "纵隔气漏" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "气胸" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "气漏" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "纵隔气肿" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "间质气肿" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "RDS" }, { "start_idx": 42, "end_idx": 49, "type": "pro", "entity": "胸腔插管闭式引流" } ] }, { "text": "主要预防手段为柔和的复苏手法和小潮气量机械通气,或采用新型CPAP装置,可以通过稳定通气压力降低其发生率。", "entities": [ { "start_idx": 15, "end_idx": 22, "type": "pro", "entity": "小潮气量机械通气" }, { "start_idx": 27, "end_idx": 34, "type": "equ", "entity": "新型CPAP装置" } ] }, { "text": "目前,经肺表面活性物质治疗后的发生率可以减到10%以下。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "pro", "entity": "肺表面活性物质治疗" } ] }, { "text": "(三)肺出血肺出血为严重临床并发症,一般止血药物往往难以奏效。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "肺出血" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "肺出血" } ] }, { "text": "约有2%~7%的经肺表面活性物质治疗的新生儿可以并发肺出血。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "pro", "entity": "肺表面活性物质治疗" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "肺出血" } ] }, { "text": "有报道应用肺表面活性物质制剂治疗肺出血有效,但对于早产极低出生体重儿预后差。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dru", "entity": "肺表面活性物质制剂" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "肺出血" } ] }, { "text": "(四)持续动脉导管开放持续动脉导管开放(PDA)多见于经肺表面活性物质治疗后的RDS患儿。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "持续动脉导管开放" }, { "start_idx": 11, "end_idx": 18, "type": "pro", "entity": "持续动脉导管开放" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "PDA" }, { "start_idx": 28, "end_idx": 36, "type": "pro", "entity": "肺表面活性物质治疗" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "RDS" } ] }, { "text": "为使关闭动脉导管,可以在出生后第3天起,静脉给予吲哚美辛(indomethacin)或布洛芬(ibuprofen)治疗。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "equ", "entity": "动脉导管" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "静脉" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 43, "end_idx": 45, "type": "dru", "entity": "布洛芬" } ] }, { "text": "如果无效,可以手术结扎使之关闭。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "手术结扎" } ] }, { "text": "【足月儿RDS】(一)足月儿原发性RDS一般见于窒息后有肺水肿的足月儿。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "RDS" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "足月儿原发性RDS" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "窒息" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肺水肿" } ] }, { "text": "大部分没有胎粪污染羊水,而无早产儿发生RDS的情况,但可以有产前和产时窒息史,使肺泡上皮细胞的肺液清除功能下降。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "RDS" }, { "start_idx": 40, "end_idx": 45, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "肺液" } ] }, { "text": "同时,可以有胸片肺野渗出似炎症、心影大、二尖瓣和三尖瓣关闭不全、低血压、肝脏增大、少尿等症状。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肝脏" }, { "start_idx": 6, "end_idx": 42, "type": "sym", "entity": "胸片肺野渗出似炎症、心影大、二尖瓣和三尖瓣关闭不全、低血压、肝脏增大、少尿" } ] }, { "text": "彩超检查可以发现心脏收缩力和心输出量下降等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "彩超检查" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心脏" }, { "start_idx": 8, "end_idx": 19, "type": "sym", "entity": "心脏收缩力和心输出量下降" } ] }, { "text": "但一般在机械通气和纠正低氧、酸中毒后,会在24小时恢复。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "机械通气" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "低氧" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "酸中毒" } ] }, { "text": "(二)足月儿获得性RDS随着年龄的增加,发生RDS的比例逐渐降低,但是由于非医学适应证剖宫产比例的增高,即使达37周,发生RDS的比例仍可达4%左右。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "足月儿获得性RDS" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "RDS" }, { "start_idx": 61, "end_idx": 63, "type": "dis", "entity": "RDS" } ] }, { "text": "此类患儿与早产儿RDS在临床症状和放射学检查上相似,而不同于窒息后肺水肿,其没有窒息史及心功能低下。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "早产儿RDS" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "放射学检查" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "窒息" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "肺水肿" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "窒息" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "心功能低下" } ] }, { "text": "可能为表面活性物质相对缺乏,可以考虑用外源性表面活性物质治疗,如果效果不好,则可以用高频震荡通气(HFOV)等治疗手段。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "表面活性物质" }, { "start_idx": 19, "end_idx": 29, "type": "pro", "entity": "外源性表面活性物质治疗" }, { "start_idx": 42, "end_idx": 47, "type": "pro", "entity": "高频震荡通气" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "HFOV" } ] }, { "text": "(三)先天性肺泡蛋白沉积症和表面活性物质蛋白B缺乏发病原因为调控SP-B合成的DNA序列碱基突变。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "先天性肺泡蛋白沉积症" }, { "start_idx": 14, "end_idx": 24, "type": "dis", "entity": "表面活性物质蛋白B缺乏" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "SP-B" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "DNA" } ] }, { "text": "临床上表现为足月出生小儿进行性呼吸困难,经任何治疗干预无效。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "小儿进行性呼吸困难" } ] }, { "text": "肺病理表现类似早产儿RDS,肺活检发现SP-B蛋白和SP-BmRNA缺乏,而前SP-C(proSPC)基因表达提高。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "早产儿RDS" }, { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "肺活检" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "SP-B蛋白" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "SP-BmRNA" }, { "start_idx": 19, "end_idx": 35, "type": "sym", "entity": "SP-B蛋白和SP-BmRNA缺乏" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "前SP-C" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "proSPC" } ] }, { "text": "肺组织病理类似肺泡蛋白沉积症。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肺组织" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "肺泡蛋白沉积症" } ] }, { "text": "外源性肺表面活性物质治疗仅能暂时缓解症状,不能治愈,患儿多在1岁内死亡,或者依赖肺移植。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "pro", "entity": "外源性肺表面活性物质治疗" }, { "start_idx": 40, "end_idx": 42, "type": "pro", "entity": "肺移植" } ] }, { "text": "六、对重复肾活检的认识重复肾活检的目的在于了解疾病的演变过程,观察药物治疗效果及判断预后,在临床及科研工作中有重要的价值。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "重复肾活检" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "重复肾活检" } ] }, { "text": "除一些列为临床观察及科研的病例行重复肾活检外,无论哪种类型的肾脏疾病,只要治疗效果不佳,经过一定时期的随访,病理类型有可能发生变化者,均为重复肾活检的对象。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "重复肾活检" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "肾脏疾病" }, { "start_idx": 69, "end_idx": 73, "type": "pro", "entity": "重复肾活检" } ] }, { "text": "当然患者的临床及其他实验室检查条件必须符合一般肾活检的指征。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "实验室检查" }, { "start_idx": 23, "end_idx": 25, "type": "pro", "entity": "肾活检" } ] }, { "text": "临床工作中,较常进行重复肾活检的疾病包括下述三种:①各种原发及继发性肾小球疾病,如新月体性肾炎、抗GBM肾炎、狼疮性肾炎、紫癜性肾炎和坏死性血管炎肾损害等;②经正规治疗临床症状不缓解,血尿、蛋白尿持续者,如以蛋白尿为主的局灶节段性肾小球肾炎、膜性肾病、系膜病变及以血尿为主的IgA肾炎等;③临床过程发生突然变化如肾移植后排斥等。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "重复肾活检" }, { "start_idx": 28, "end_idx": 38, "type": "dis", "entity": "原发及继发性肾小球疾病" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "新月体性肾炎" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "抗GBM肾炎" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "狼疮性肾炎" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "紫癜性肾炎" }, { "start_idx": 67, "end_idx": 75, "type": "dis", "entity": "坏死性血管炎肾损害" }, { "start_idx": 92, "end_idx": 93, "type": "sym", "entity": "血尿" }, { "start_idx": 95, "end_idx": 97, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 104, "end_idx": 106, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 110, "end_idx": 119, "type": "dis", "entity": "局灶节段性肾小球肾炎" }, { "start_idx": 121, "end_idx": 124, "type": "dis", "entity": "膜性肾病" }, { "start_idx": 126, "end_idx": 129, "type": "dis", "entity": "系膜病变" }, { "start_idx": 132, "end_idx": 133, "type": "sym", "entity": "血尿" }, { "start_idx": 137, "end_idx": 141, "type": "dis", "entity": "IgA肾炎" }, { "start_idx": 156, "end_idx": 158, "type": "pro", "entity": "肾移植" } ] }, { "text": "二、新生儿后期贫血新生儿后期贫血主要是指出生1周以后发生的贫血,一般多为慢性贫血,主要有以下几种类型:(一)新生儿生理性贫血新生儿生理性贫血是指足月儿生后6~12周时血红蛋白下降至95~110g/L,主要原因有:在宫内,胎儿血氧饱和度约50%,相对缺氧状态使促红细胞生成素含量较高,红细胞较多,出生后血氧饱和度显著增高,促红细胞生成素分泌明显减少,红细胞产生减少。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "贫血" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "贫血" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "贫血" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "新生儿生理性贫血" }, { "start_idx": 62, "end_idx": 69, "type": "dis", "entity": "新生儿生理性贫血" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 112, "end_idx": 116, "type": "ite", "entity": "血氧饱和度" }, { "start_idx": 129, "end_idx": 135, "type": "bod", "entity": "促红细胞生成素" }, { "start_idx": 141, "end_idx": 143, "type": "bod", "entity": "红细胞" }, { "start_idx": 150, "end_idx": 154, "type": "ite", "entity": "血氧饱和度" }, { "start_idx": 160, "end_idx": 166, "type": "bod", "entity": "促红细胞生成素" }, { "start_idx": 174, "end_idx": 176, "type": "bod", "entity": "红细胞" } ] }, { "text": "新生儿红细胞寿命较短。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "红细胞" } ] }, { "text": "(二)早产儿贫血早产儿贫血是早产儿(尤其是极低出生体重儿)的常见现象,严重者影响早产儿的生长发育,因此早产儿贫血并非生理性。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "早产儿贫血" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "早产儿贫血" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "早产儿贫血" } ] }, { "text": "早产儿贫血的主要临床表现为苍白、气急、心率增快、烦躁不安或淡漠、食欲下降、喂养困难、体重不增。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "苍白" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "气急" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "心率增快" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "淡漠" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "食欲下降" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "喂养困难" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "体重不增" } ] }, { "text": "胎龄越小,出生体重越低,贫血出现越早,程度越严重,持续时间越长。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "胎龄越小" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "出生体重越低" } ] }, { "text": "(三)新生儿晚期贫血晚期贫血是指部分Rh血型不合溶血病患儿在生后2~6周发生明显贫血。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "新生儿晚期贫血" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "晚期贫血" }, { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "Rh血型不合溶血病" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "贫血" } ] }, { "text": "这是由于部分Rh血型不合溶血病患儿早期症状不严重,不需换血治疗,但Rh血型抗体却在体内持续存在较长时间(超过1~2个月),继续溶血而导致晚期贫血。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "dis", "entity": "部分Rh血型不合溶血病" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "换血治疗" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "Rh血型抗体" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "溶血" }, { "start_idx": 68, "end_idx": 71, "type": "dis", "entity": "晚期贫血" } ] }, { "text": "第五节微生物学检查呼吸道感染的病原学诊断极为重要,在指导抗生素的合理应用和疾病的预防中起关键作用。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "微生物学检查" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "抗生素" } ] }, { "text": "具体方法因病原菌而异。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "mic", "entity": "病原菌" } ] }, { "text": "一、细菌学检查对上呼吸道标本培养结果的解释要特别小心,因为上呼吸道正常情况下就存在许多微生物,包括小儿肺炎常见的病原菌,如肺炎链球菌、流感嗜血杆菌等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "细菌学检查" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "标本培养" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "小儿肺炎" }, { "start_idx": 56, "end_idx": 58, "type": "mic", "entity": "病原菌" }, { "start_idx": 61, "end_idx": 65, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 67, "end_idx": 72, "type": "mic", "entity": "流感嗜血杆菌" } ] }, { "text": "因而从呼吸道分泌物中分离出细菌并不代表它们在呼吸道感染中的致病作用。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "呼吸道分泌物" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "细菌" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "呼吸道感染" } ] }, { "text": "咽拭子细菌培养主要应用于链球菌咽炎的诊断。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "咽拭子细菌培养" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "链球菌咽炎" } ] }, { "text": "要压下舌根以免被口腔菌污染。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "舌根" }, { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "口腔菌" } ] }, { "text": "正常情况下喉、气管以下均为无菌区,这些部位采集的标本均可送细菌室分析,一旦培养阳性,理论上均有诊断意义。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "喉" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "气管" }, { "start_idx": 29, "end_idx": 31, "type": "dep", "entity": "细菌室" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "阳性" } ] }, { "text": "但在实际操作过程中,无一例外地受到口腔、鼻咽部细菌的污染,有时送检的可能不是痰液,而是唾液。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "口腔" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "鼻咽部" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "痰液" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "唾液" } ] }, { "text": "在细菌室,送检标本最多的是痰,而痰中培养出的细菌种类和数量与通过肺穿刺所得的结果一致性很差。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dep", "entity": "细菌室" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "痰" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "痰" }, { "start_idx": 22, "end_idx": 23, "type": "mic", "entity": "细菌" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "肺穿刺" } ] }, { "text": "因而必须用直接Gram染色镜检筛选痰标本,以确定痰标本的质量。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "pro", "entity": "Gram染色镜检" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "痰" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "痰" } ] }, { "text": "在低倍镜下(100倍)检查每个视野的鳞状上皮细胞如超过10个(也有人认为超过25个),即可判定为不合格痰标本。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "equ", "entity": "低倍镜" }, { "start_idx": 18, "end_idx": 23, "type": "equ", "entity": "鳞状上皮细胞" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "痰" } ] }, { "text": "除一般培养外,可根据病例情况进行特殊培养,如结核菌培养、厌氧菌培养。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "mic", "entity": "结核菌" }, { "start_idx": 28, "end_idx": 30, "type": "mic", "entity": "厌氧菌" } ] }, { "text": "痰定量培养可提供较为正确的病原学诊断,一般认为当细菌数超过105CFU/ml时有诊断意义。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "痰定量培养" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "细菌" } ] }, { "text": "为减少标本受污染的机会,可通过纤维支气管镜下保护性毛刷或特殊的气囊导管进行支气管肺泡灌洗取得标本进行培养,亦可通过肺穿刺吸引获取标本。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 22, "end_idx": 26, "type": "equ", "entity": "保护性毛刷" }, { "start_idx": 31, "end_idx": 34, "type": "equ", "entity": "气囊导管" }, { "start_idx": 37, "end_idx": 43, "type": "pro", "entity": "支气管肺泡灌洗" }, { "start_idx": 57, "end_idx": 59, "type": "pro", "entity": "肺穿刺" } ] }, { "text": "胸水、血培养阳性可作为细菌性肺炎的重要诊断依据,但其阳性率不高。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "胸水、血培养阳性" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "阳性" } ] }, { "text": "此外亦可通过乳胶凝集试验、对流免疫电泳等血清学方法测定血液或尿液中的细菌抗原进行诊断。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "乳胶凝集试验" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "对流免疫电泳" }, { "start_idx": 20, "end_idx": 24, "type": "pro", "entity": "血清学方法" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血液" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "尿液" }, { "start_idx": 34, "end_idx": 35, "type": "mic", "entity": "细菌" } ] }, { "text": "活泼好动,活动范围增大,大脑思维能力明显增强,智力发育迅速,是学习知识和培养生活自理能力的良好阶段。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "大脑" } ] }, { "text": "此期的儿童易缺乏的营养素为钙、铁、锌、维生素A和维生素B2等,而且正值乳牙更换期,充足供给钙质可保证恒牙的正常生长。", "entities": [ { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "乳牙" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "恒牙" } ] }, { "text": "五、肾活检绝对及相对禁忌证(一)绝对禁忌证指临床上有明显出血倾向的患者,孤立肾或对侧肾脏已切除者、多囊肾或肾脏囊性病变、肾脏肿瘤以及动脉瘤也都不宜行肾活检。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肾活检" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "出血倾向" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "孤立肾" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "肾脏" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "多囊肾" }, { "start_idx": 53, "end_idx": 58, "type": "dis", "entity": "肾脏囊性病变" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "肾脏肿瘤" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 74, "end_idx": 76, "type": "pro", "entity": "肾活检" } ] }, { "text": "(二)相对或暂时禁忌证1.终末期肾脏疾病慢性肾衰竭时作肾活检危险性大,易出现并发症,由于肾脏萎缩常使穿刺成功率降低,即使取得足够的肾组织,但由于病变严重,正常结构消失,已被纤维化的组织所替代,因而难以确定原发病,因此过去此类疾病不列为肾活检的指征。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肾脏" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "慢性肾衰竭" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "肾活检" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "肾脏" }, { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "穿刺" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "肾组织" }, { "start_idx": 117, "end_idx": 119, "type": "pro", "entity": "肾活检" } ] }, { "text": "近年来,由于对慢性肾衰竭患者治疗的进展、透析疗法的普及以及穿刺的安全性也有所提高,因此,我们主张对肾脏大小尚在正常范围内的肾衰竭患者应争取作肾活检,以确定是否存在活动性、可逆性病变。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "慢性肾衰竭" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "透析疗法" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "穿刺" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "肾脏" }, { "start_idx": 61, "end_idx": 63, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 70, "end_idx": 72, "type": "pro", "entity": "肾活检" } ] }, { "text": "对于萎缩肾,目前仍认为是穿刺的禁忌证。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "萎缩肾" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "穿刺" } ] }, { "text": "2.对侧肾功能不良为防止穿刺发生并发症,应避免行健侧。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "对侧肾功能不良" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "穿刺" } ] }, { "text": "3.肾脏感染性病变如肾结核、脓肿和活动性肾盂肾炎,穿刺易致感染扩散,因此不做肾穿刺。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "肾脏感染性病变" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾结核" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "脓肿" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "活动性肾盂肾炎" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "穿刺" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "易致" }, { "start_idx": 38, "end_idx": 40, "type": "pro", "entity": "肾穿刺" } ] }, { "text": "4.难以控制的高血压穿刺后出血机会明显增多。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "高血压" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "穿刺" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "出血" } ] }, { "text": "5.肾活检的相对禁忌证还包括严重贫血、血容量不足、心功能不全以及肾钙化等。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肾活检" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "贫血" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "血容量不足" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "心功能不全" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "肾钙化" } ] }, { "text": "九、水痘肺炎水痘肺炎(varicellapneumonia)由水痘-带状疱疹病毒引起,为全身性疾病,可发生支气管炎和间质性肺炎。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "水痘肺炎" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "水痘肺炎" }, { "start_idx": 11, "end_idx": 28, "type": "dis", "entity": "varicellapneumonia" }, { "start_idx": 31, "end_idx": 39, "type": "mic", "entity": "水痘-带状疱疹病毒" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "支气管炎" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "间质性肺炎" } ] }, { "text": "年龄越小越易发生肺炎。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肺炎" } ] }, { "text": "多在水痘发生1周内,表现咳嗽,肺部有湿性啰音,X线检查呈现双肺野结节性浸润阴影。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "水痘" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "咳嗽" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺部" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "湿性啰音" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "X线检查" } ] }, { "text": "水痘患儿如出现呼吸道症状和体征,应考虑本病。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "水痘" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "呼吸道" } ] }, { "text": "部分年幼婴儿,水痘肺炎可出现在皮疹之前,极易误诊和漏诊。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "水痘肺炎" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "皮疹" } ] }, { "text": "因而有明确水痘接触史者,如发生肺炎,亦应考虑本病,并予以隔离。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "水痘" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "肺炎" } ] }, { "text": "二、供肾的选择儿童肾移植具有更广泛的供肾来源,成人供肾移植给10岁以上的儿童,技术操作与成人肾移植无差别;成人肾移植给婴幼儿需克服手术操作中的一些困难;体重7~8kg以下的婴幼儿宜使用儿童供肾;取自5岁以下小儿的尸体肾移植给年龄较大的儿童可获满意的肾功能。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肾" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "肾移植" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "肾" }, { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "肾移植" }, { "start_idx": 46, "end_idx": 48, "type": "pro", "entity": "肾移植" }, { "start_idx": 55, "end_idx": 57, "type": "pro", "entity": "肾移植" }, { "start_idx": 95, "end_idx": 95, "type": "bod", "entity": "肾" }, { "start_idx": 108, "end_idx": 108, "type": "bod", "entity": "肾" }, { "start_idx": 124, "end_idx": 124, "type": "bod", "entity": "肾" } ] }, { "text": "儿童肾移植更容易从亲属中获取活体肾移植,无论在组织相容性还是缩短缺血时间上都更具优越性,从而降低排斥反应的发生率。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肾移植" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "肾移植" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "血" } ] }, { "text": "另有成功病例报道将新生儿尸体双肾连同主动脉、腔静脉一并切下并移植给先天性马蹄肾肾衰竭的患儿。", "entities": [ { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肾" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "主动脉" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "腔静脉" }, { "start_idx": 33, "end_idx": 41, "type": "dis", "entity": "先天性马蹄肾肾衰竭" } ] }, { "text": "三、葡萄糖-6-磷酸脱氢酶缺陷症红细胞葡萄糖-6-磷酸脱氢酶(glucose-6-phosphatedehydrogenase,G-6-PD)缺陷症是一种常见伴性不完全显性遗传性红细胞膜酶缺陷病,此类缺陷遍布全世界,据统计约有2亿多人具有此类缺陷。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "dis", "entity": "葡萄糖-6-磷酸脱氢酶缺陷症" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "红细胞" }, { "start_idx": 89, "end_idx": 96, "type": "dis", "entity": "红细胞膜酶缺陷病" } ] }, { "text": "编码该酶蛋白的基因位于X染色体长臂2区8带(Xq28)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "酶蛋白" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "X染色体" } ] }, { "text": "目前已证实至少有90种G-6-PD突变基因存在,大多为编码区内单个或多个碱基置换的突变,少数为缺失型。", "entities": [ { "start_idx": 11, "end_idx": 20, "type": "bod", "entity": "G-6-PD突变基因" } ] }, { "text": "出现慢性非球形红细胞溶血性贫血(CNSHA)者的基因突变发生于G-6-PD基因羧基末端第1089~1361核苷酸序列中,突变发生于氨基末端者病情较轻。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "慢性非球形红细胞溶血性贫血" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "CNSHA" } ] }, { "text": "我国已发现12种G-6-PD突变基因,均为点突变。", "entities": [ { "start_idx": 8, "end_idx": 17, "type": "bod", "entity": "G-6-PD突变基因" } ] }, { "text": "多种G-6-PD突变基因所表达的产物为各种变异酶,它们的活性大多降低或稳定性降低。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "bod", "entity": "G-6-PD突变基因" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "变异酶" } ] }, { "text": "到目前为止,已发现400多种G-6-PD变异酶,其中可引起各种溶血症者有100种以上。", "entities": [ { "start_idx": 14, "end_idx": 22, "type": "bod", "entity": "G-6-PD变异酶" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "溶血症" } ] }, { "text": "根据酶的活性和是否出现临床表现,可将它们分为五大类:①严重酶缺陷伴CNSHA;②酶活性严重缺乏,活性<10%;③酶活性轻~中度缺陷,活性为10%~60%;④酶活性轻度降低或正常,活性为60%~100%;⑤酶活性增加,高于正常的4~5倍。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "酶" }, { "start_idx": 27, "end_idx": 37, "type": "dis", "entity": "严重酶缺陷伴CNSHA" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "酶" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "酶" }, { "start_idx": 78, "end_idx": 78, "type": "bod", "entity": "酶" }, { "start_idx": 102, "end_idx": 102, "type": "bod", "entity": "酶" } ] }, { "text": "其中②③类多数表现为新生儿高胆红素血症及急性溶血性贫血,少数伴有CNSHA。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "新生儿高胆红素血症" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "急性溶血性贫血" } ] }, { "text": "根据发病种族的不同,将G-6-PD分为G-6-PDA和G-6-PDB两种变异型。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "G-6-PD" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "G-6-PDA" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "G-6-PDB" } ] }, { "text": "在我国广东,还发现另一种变异型,称为G-6-PDCanton(广东型),区域发病率可高达5%。", "entities": [ { "start_idx": 18, "end_idx": 29, "type": "dis", "entity": "G-6-PDCanton" } ] }, { "text": "【发病机制】G-6-PD在磷酸戊糖旁路中是6-磷酸葡萄糖(G-6-P)转变为6-磷酸葡萄糖酸(G-6-PG)反应中必需的酶。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "G-6-PD" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "磷酸戊糖旁路" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "6-磷酸葡萄糖" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "G-6-P" }, { "start_idx": 38, "end_idx": 45, "type": "bod", "entity": "6-磷酸葡萄糖酸" }, { "start_idx": 47, "end_idx": 52, "type": "bod", "entity": "G-6-PG" }, { "start_idx": 60, "end_idx": 60, "type": "bod", "entity": "酶" } ] }, { "text": "G-6-PD缺乏时,使还原型三磷酸吡啶核苷(NADPH)减少,不能维持生理浓度的还原型谷胱甘肽(GSH),从而使红细胞膜蛋白和酶蛋白中的巯基遭受氧化,改变了红细胞膜的完整性。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "G-6-PD" }, { "start_idx": 11, "end_idx": 20, "type": "bod", "entity": "还原型三磷酸吡啶核苷" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "NADPH" }, { "start_idx": 40, "end_idx": 46, "type": "bod", "entity": "还原型谷胱甘肽" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "GSH" }, { "start_idx": 56, "end_idx": 61, "type": "bod", "entity": "红细胞膜蛋白" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "酶蛋白" }, { "start_idx": 78, "end_idx": 81, "type": "bod", "entity": "红细胞膜" } ] }, { "text": "此外,NADPH减少时,使高铁血红蛋白(MHb)不能转变为氧合血红蛋白,MHb增加致红细胞内不可溶性变性珠蛋白小体(Heinz小体)形成明显增加,红细胞膜变硬,通过脾脏时Heinz小体被巨噬细胞摘出,随之丢失部分的红细胞膜,致表面积减少,可塑性降低,而易破裂溶血。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "NADPH" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "高铁血红蛋白" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "MHb" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "氧合血红蛋白" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "MHb" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "红细胞" }, { "start_idx": 46, "end_idx": 56, "type": "bod", "entity": "不可溶性变性珠蛋白小体" }, { "start_idx": 58, "end_idx": 64, "type": "bod", "entity": "Heinz小体" }, { "start_idx": 73, "end_idx": 76, "type": "bod", "entity": "红细胞膜" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "脾脏" }, { "start_idx": 85, "end_idx": 91, "type": "bod", "entity": "Heinz小体" }, { "start_idx": 93, "end_idx": 96, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 107, "end_idx": 110, "type": "bod", "entity": "红细胞膜" }, { "start_idx": 129, "end_idx": 130, "type": "sym", "entity": "溶血" } ] }, { "text": "【临床表现】本病一般可分为四种类型:(一)新生儿高胆红素血症新生儿红细胞G-6-PD缺乏时可受外源性或内源性氧化剂作用诱发或自发发生新生儿溶血症,导致高胆红素血症。", "entities": [ { "start_idx": 21, "end_idx": 29, "type": "dis", "entity": "新生儿高胆红素血症" }, { "start_idx": 33, "end_idx": 43, "type": "sym", "entity": "红细胞G-6-PD缺乏" }, { "start_idx": 66, "end_idx": 71, "type": "dis", "entity": "新生儿溶血症" }, { "start_idx": 75, "end_idx": 80, "type": "dis", "entity": "高胆红素血症" } ] }, { "text": "我国新生儿G-6-PD缺乏发生率约3.0%~8.7%,其中严重黄疸发生率为20%~50%。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "G-6-PD缺乏" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "黄疸" } ] }, { "text": "感染或药物可使症状加重。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "感染" } ] }, { "text": "黄疸多出现在生后48~96小时内,也可迟至2周才出现黄疸。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "黄疸" } ] }, { "text": "进展迅速,严重的于1周后发生胆红素脑病甚至死亡。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "胆红素脑病" } ] }, { "text": "(二)蚕豆病(favism)本病最初因食用蚕豆、及其制品后发生急性溶血性贫血、黄疸而得名,多见于我国南方以及地中海沿岸国家的某些人群。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "蚕豆病" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "favism" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "急性溶血性贫血" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "黄疸" } ] }, { "text": "溶血的轻重与食用蚕豆的量也无关系。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "溶血" } ] }, { "text": "目前认为,蚕豆中含有大量左旋多巴,在酪氨酸酶的作用下,可变成多巴醌,后者可使GSH含量减少而发生溶血。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "左旋多巴" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "酪氨酸酶" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "多巴醌" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "GSH" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "溶血" } ] }, { "text": "本病大多发生在每年产新鲜蚕豆季节,在食用蚕豆或其制品、吸入蚕豆花粉、或吸吮乳母吃过蚕豆的乳汁后数小时至数天内发生不同程度的急性血管内溶血。", "entities": [ { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "急性血管内溶血" } ] }, { "text": "血红蛋白迅速下降,多有黄疸。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "黄疸" } ] }, { "text": "极重者甚至出现惊厥、休克、急性肾衰竭;如不及时治疗可于1~2天内死亡。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "惊厥" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "急性肾衰竭" } ] }, { "text": "溶血期大多持续1~2天,最长1周左右,然后进入恢复期。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "溶血" } ] }, { "text": "(三)药物诱发溶血性贫血服用具有氧化作用的药物如:①乙酰苯胺(acetanild)、氨基比林等退热药;②伯氨喹、扑疟喹等抗疟药;③磺胺类药物;④其他如呋喃唑酮、呋喃坦丁、萘(naphthalene)、苯肼等可引起溶血。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "乙酰苯胺" }, { "start_idx": 31, "end_idx": 39, "type": "dru", "entity": "acetanild" }, { "start_idx": 42, "end_idx": 45, "type": "dru", "entity": "氨基比林" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "伯氨喹" }, { "start_idx": 56, "end_idx": 58, "type": "dru", "entity": "扑疟喹" }, { "start_idx": 60, "end_idx": 62, "type": "dru", "entity": "抗疟药" }, { "start_idx": 75, "end_idx": 78, "type": "dru", "entity": "呋喃唑酮" }, { "start_idx": 80, "end_idx": 83, "type": "dru", "entity": "呋喃坦丁" }, { "start_idx": 85, "end_idx": 85, "type": "dru", "entity": "萘" }, { "start_idx": 87, "end_idx": 97, "type": "dru", "entity": "naphthalene" }, { "start_idx": 100, "end_idx": 101, "type": "dru", "entity": "苯肼" }, { "start_idx": 106, "end_idx": 107, "type": "dru", "entity": "溶血" } ] }, { "text": "具体某一药物对G-6-PD缺乏者诱发溶血作用并不一致,如服用氯霉素可诱发地中海沿岸重度G-6-PD缺乏者出现溶血,而在A-型或广东型的G-6-PD缺乏患者则不引起溶血。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "G-6-PD缺乏" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "溶血" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "氯霉素" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "溶血" }, { "start_idx": 67, "end_idx": 74, "type": "sym", "entity": "G-6-PD缺乏" }, { "start_idx": 81, "end_idx": 82, "type": "dis", "entity": "溶血" } ] }, { "text": "急性溶血期持续1~2周,血红蛋白多于1周末降至最低点,此时出现血红蛋白尿,高铁血红蛋白血症,血浆GSH和结合珠蛋白下降,周围血涂片可见红细胞轻度大小不等,红细胞碎片,异型或嗜多染红细胞增多等。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "血红蛋白尿" }, { "start_idx": 37, "end_idx": 44, "type": "dis", "entity": "高铁血红蛋白血症" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "血浆GSH" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "结合珠蛋白" }, { "start_idx": 62, "end_idx": 64, "type": "pro", "entity": "血涂片" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "红细胞" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "红细胞" }, { "start_idx": 86, "end_idx": 91, "type": "bod", "entity": "嗜多染红细胞" } ] }, { "text": "肝、脾可轻度增大。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "肝、脾可轻度增大" } ] }, { "text": "2周后进入恢复期,网状红细胞、血红蛋白逐渐上升至正常,病情也明显好转,此时即使服用同类药物也无明显溶血,于4周后血红蛋白恢复正常。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "网状红细胞" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "此后进入代偿稳定期,由于骨髓代偿功能良好,病人无贫血或黄疸,网织红细胞正常。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "骨髓" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "贫血" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "黄疸" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "网织红细胞" } ] }, { "text": "但若于2~3月后再服此药物可再次诱发溶血。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "溶血" } ] }, { "text": "(四)感染诱发的溶血性贫血G-6-PD缺乏患儿,在有些病毒和细菌感染如急性传染性肝炎、传染性单核细胞增多症、上呼吸道感染、肺炎、败血症、肠炎、菌痢、伤寒等情况下均可诱发与伯氨喹型药物相似的溶血性贫血,主要机制是由于急性感染过程中体内氧化产物如H2O2等堆积,造成红细胞破坏所致。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "感染" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "G-6-PD缺乏" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "感染" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "急性传染性肝炎" }, { "start_idx": 43, "end_idx": 52, "type": "dis", "entity": "传染性单核细胞增多症" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 61, "end_idx": 62, "type": "dis", "entity": "肺炎" }, { "start_idx": 64, "end_idx": 66, "type": "dis", "entity": "败血症" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "肠炎" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "菌痢" }, { "start_idx": 74, "end_idx": 75, "type": "dis", "entity": "伤寒" }, { "start_idx": 85, "end_idx": 90, "type": "dru", "entity": "伯氨喹型药物" }, { "start_idx": 94, "end_idx": 98, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 109, "end_idx": 110, "type": "sym", "entity": "感染" }, { "start_idx": 143, "end_idx": 145, "type": "bod", "entity": "红细胞" } ] }, { "text": "【辅助检查】1.血象溶血发作时红细胞与血红蛋白迅速下降。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "血象溶血" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "红细胞" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "血红蛋白" } ] }, { "text": "出现变形和嗜多色性红细胞及红细胞碎片,网织红细胞增多,大多在5%~15%之间,最高可达20%。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "bod", "entity": "嗜多色性红细胞" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "红细胞" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "网织红细胞" } ] }, { "text": "白细胞可以增高,达10×109~20×109/L,甚至出现类白血病反应,血小板通常正常或偏高。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "白细胞" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "白血病" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "血小板" } ] }, { "text": "2.骨髓象粒细胞、红细胞系均增生,粒细胞增生程度与发病年龄呈负相关。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "骨髓象粒细胞" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "红细胞" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "粒细胞" } ] }, { "text": "3.尿常规检查根据溶血程度的不同,肉眼观察可见尿呈酱油色、浓茶色、茶色或黄色,尿隐血试验60%~70%病例呈阳性。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "尿常规检查" }, { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "肉眼观察" } ] }, { "text": "严重时可导致肾功能损害,出现蛋白尿、红细胞尿及管型尿,尿胆原和尿胆红素增加。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "肾功能损害" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "蛋白尿" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "红细胞尿" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "管型尿" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "尿胆红素" } ] }, { "text": "4.血清游离血红蛋白增加,结合珠蛋白降低。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血清" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 2, "end_idx": 11, "type": "sym", "entity": "血清游离血红蛋白增加" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "结合珠蛋白" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "结合珠蛋白降低" } ] }, { "text": "5.变性珠蛋白小体试验用结晶紫将Heinz小体染成紫色,然后计数含Heinz小体的红细胞的百分比。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "变性珠蛋白小体" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "Heinz小体" }, { "start_idx": 33, "end_idx": 39, "type": "bod", "entity": "Heinz小体" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "红细胞" } ] }, { "text": "在发病48小时内均可检出Heinz小体,溶血停止后即呈阴性。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "bod", "entity": "Heinz小体" } ] }, { "text": "但此试验并非特异性,因不稳定血红蛋白病、血红蛋白H病和其他酶缺陷也可呈阳性。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "血红蛋白病" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "血红蛋白H病" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "酶" } ] }, { "text": "6.高铁血红蛋白还原试验是目前常用的筛选试验。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "pro", "entity": "高铁血红蛋白还原试验" } ] }, { "text": "G-6-PD缺陷的患者由于NADPH生成减少,MHb的还原速度显著减慢,以此来间接测定G-6-PD活性。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "G-6-PD缺陷" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "MHb" }, { "start_idx": 43, "end_idx": 48, "type": "bod", "entity": "G-6-PD" } ] }, { "text": "定量法还原率大于75%为正常,74%~31%为中等度(杂合子)缺陷,小于31%为重度缺陷。", "entities": [ { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "重度缺陷" } ] }, { "text": "7.高铁血红蛋白洗脱法本试验是利用组织化学方法以观察每个红细胞中MHb被还原的能力,从而间接反映G-6-PD活性。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "高铁血红蛋白洗脱法" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "红细胞" }, { "start_idx": 48, "end_idx": 53, "type": "bod", "entity": "G-6-PD" } ] }, { "text": "G-6-PD缺陷的红细胞中的MHb不易还原,可被H2O2洗脱成为不着色的红细胞空影。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "G-6-PD缺陷" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "红细胞" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "MHb" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "红细胞" } ] }, { "text": "正常人空影红细胞小于2%,若超过80%为显著缺陷;杂合子在50%左右。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "红细胞" } ] }, { "text": "8.荧光斑点试验正常红细胞在紫外线波长340nm照射下可发生荧光,而患者的红细胞不发生荧光。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "荧光斑点试验" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "红细胞" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "红细胞" } ] }, { "text": "9.G-6-PD活性定量测定是一种直接测定法,对诊断有特异性。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "G-6-PD" } ] }, { "text": "患者G-6-PD活性多在正常人的10%以下。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "G-6-PD" } ] }, { "text": "【诊断】根据急性溶血、血红蛋白尿、病史中找到明确的诱因,并有阳性家族史和既往发作史,再结合以上实验室检查,多可作出诊断。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "急性溶血" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "血红蛋白尿" } ] }, { "text": "继发于感染时,应结合各种感染的特点加以综合考虑。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "感染" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "感染" } ] }, { "text": "本病需与不稳定血红蛋白病(uHb病)如HbH病、免疫性溶血性贫血等鉴别,前者在血红蛋白电泳时,可出现异常区带;后者Coombs试验阳性。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "血红蛋白病" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "uHb病" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "HbH病" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "免疫性溶血性贫血" }, { "start_idx": 39, "end_idx": 44, "type": "pro", "entity": "血红蛋白电泳" }, { "start_idx": 57, "end_idx": 64, "type": "pro", "entity": "Coombs试验" } ] }, { "text": "慢性溶血型应与其他遗传性溶血性疾病鉴别,可通过G-6-PD活性直接测定以资鉴别。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "遗传性溶血性疾病" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "G-6-PD" } ] }, { "text": "【治疗和预后】在高发病地区进行普查,凡证实有G-6-PD缺陷的,应避免食用蚕豆或具有氧化作用的药物,并应积极预防感染,以防止急性发作。", "entities": [ { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "G-6-PD缺陷" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "感染" } ] }, { "text": "急性发作时,应尽快去除诱因,贫血严重者(Hb<40g/L)应输血或浓缩红细胞1~2次,注意水和电解质紊乱,纠正酸中毒,防止高钾血症,以及碱化尿液防止急性肾衰竭。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "贫血" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "红细胞" }, { "start_idx": 45, "end_idx": 51, "type": "sym", "entity": "水和电解质紊乱" }, { "start_idx": 55, "end_idx": 57, "type": "sym", "entity": "酸中毒" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "高钾血症" }, { "start_idx": 68, "end_idx": 71, "type": "pro", "entity": "碱化尿液" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "新生儿时发病应注意同时防治高胆红素血症以及胆红素性脑病。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "高胆红素血症" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "胆红素性脑病" } ] }, { "text": "急性发作多呈自限性,因新生的红细胞G-6-PD活性正常,病情多于1周后逐渐稳定。", "entities": [ { "start_idx": 14, "end_idx": 22, "type": "bod", "entity": "红细胞G-6-PD" } ] }, { "text": "第六章胃炎胃炎(gastritis)是由多种病因引起的胃黏膜炎症,根据病程分为急性和慢性两类,前者多为继发性,后者以原发性多见。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "胃炎" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "胃炎" }, { "start_idx": 8, "end_idx": 16, "type": "dis", "entity": "gastritis" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "胃黏膜炎症" } ] }, { "text": "近几年随着胃镜在儿科的普及应用,儿童胃炎的检出率明显增高。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "equ", "entity": "胃镜" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "儿童胃炎" } ] }, { "text": "第一节急性胃炎急性胃炎(acutegastritis)系由不同病因引起的胃黏膜急性炎症。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "急性胃炎" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "急性胃炎" }, { "start_idx": 12, "end_idx": 25, "type": "dis", "entity": "acutegastritis" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "胃黏膜急性炎症" } ] }, { "text": "病变严重者可累及黏膜下层与肌层,甚至深达浆膜层。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "黏膜下层与肌层" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "浆膜层" } ] }, { "text": "儿童中以单纯性与糜烂性多见。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "单纯性" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "糜烂性" } ] }, { "text": "【病因】(一)微生物感染或细菌感染进食污染微生物和细菌毒素的食物后引起的急性胃炎中,多见沙门菌属、嗜盐杆菌及某些病毒等。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "微生物感染" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "细菌感染" }, { "start_idx": 19, "end_idx": 23, "type": "mic", "entity": "污染微生物" }, { "start_idx": 25, "end_idx": 28, "type": "mic", "entity": "细菌毒素" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "急性胃炎" }, { "start_idx": 44, "end_idx": 47, "type": "mic", "entity": "沙门菌属" }, { "start_idx": 49, "end_idx": 52, "type": "mic", "entity": "嗜盐杆菌" }, { "start_idx": 54, "end_idx": 57, "type": "mic", "entity": "某些病毒" } ] }, { "text": "(二)化学因素1.药物水杨酸盐类药物如阿司匹林及吲哚美辛等。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "dru", "entity": "水杨酸盐类药物" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "吲哚美辛" } ] }, { "text": "2.误食强酸(如硫酸、盐酸和硝酸)及强碱(如氢氧化钠和氢氧化钾)引起胃壁腐蚀性损伤。", "entities": [ { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "胃壁腐蚀性损伤" } ] }, { "text": "3.误食毒蕈、砷、灭虫药及杀鼠剂等化学毒物,均可刺激胃黏膜引起炎症。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "炎症" } ] }, { "text": "(三)物理因素进食过冷、过热的食品或粗糙食物均可损伤胃黏膜,引起炎症。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "炎症" } ] }, { "text": "(四)应激状态某些危重疾病如新生儿窒息、颅内出血、败血症、休克及大面积灼伤等使患儿处于严重的应激状态是导致急性糜烂性胃炎的主要原因。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "新生儿窒息" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "颅内出血" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "败血症" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "休克" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "大面积灼伤" }, { "start_idx": 53, "end_idx": 59, "type": "dis", "entity": "急性糜烂性胃炎" } ] }, { "text": "【发病机制】1.外源性病因可严重破坏胃黏液屏障,导致氢离子及胃蛋白酶的逆向弥散,引起胃黏膜的损伤而发生糜烂、出血。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "胃黏液屏障" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "氢离子" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "胃蛋白酶" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "胃黏膜的损伤" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "糜烂" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "出血" } ] }, { "text": "2.应激状态使去甲肾上腺素和肾上腺素大量分泌,内脏血管收缩,胃血流量减少,缺血、缺氧进一步使黏膜上皮的线粒体功能降低,影响氧化磷酸化过程,使胃黏膜的糖原贮存减少。", "entities": [ { "start_idx": 7, "end_idx": 21, "type": "sym", "entity": "去甲肾上腺素和肾上腺素大量分泌" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "内脏血管收缩" }, { "start_idx": 30, "end_idx": 35, "type": "sym", "entity": "胃血流量减少" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "缺血、缺氧" }, { "start_idx": 46, "end_idx": 57, "type": "sym", "entity": "黏膜上皮的线粒体功能降低" }, { "start_idx": 70, "end_idx": 79, "type": "sym", "entity": "胃黏膜的糖原贮存减少" } ] }, { "text": "而胃黏膜缺血时,不能清除逆向弥散的氢离子;缺氧和去甲肾上腺素又使碳酸氢根离子分泌减少,前列腺素合成减少,削弱胃黏膜屏障功能,导致胃黏膜急性糜烂性炎症。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "血" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "氢离子" }, { "start_idx": 32, "end_idx": 41, "type": "sym", "entity": "碳酸氢根离子分泌减少" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "前列腺素合成减少" }, { "start_idx": 52, "end_idx": 60, "type": "sym", "entity": "削弱胃黏膜屏障功能" }, { "start_idx": 64, "end_idx": 73, "type": "dis", "entity": "胃黏膜急性糜烂性炎症" } ] }, { "text": "【临床表现及分型】(一)急性单纯性胃炎起病较急,多在进食污染食物数小时后或24小时发病,症状轻重不一,表现上腹部不适、疼痛,甚至剧烈的腹部绞痛。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "急性单纯性胃炎" }, { "start_idx": 53, "end_idx": 60, "type": "sym", "entity": "上腹部不适、疼痛" }, { "start_idx": 64, "end_idx": 70, "type": "sym", "entity": "剧烈的腹部绞痛" } ] }, { "text": "若为药物或刺激性食物所致,症状则较轻,局限上腹部,体格检查有上腹部或脐周压痛,肠鸣音可亢进。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "上腹部" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "上腹部或脐周压痛" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "肠鸣音可亢进" } ] }, { "text": "(二)急性糜烂性胃炎多在机体处在严重疾病应激状态下诱发,起病急骤,常以呕血或黑粪为突出症状,大量出血可引起晕厥或休克,伴重度贫血。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "急性糜烂性胃炎" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "呕血" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "黑粪" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "晕厥" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "休克" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "重度贫血" } ] }, { "text": "(三)急性腐蚀性胃炎误服强酸、强碱史,除口腔黏膜糜烂、水肿外,中上腹剧痛、绞窄感、恶心、呕吐、呕血和黑粪,并发胃功能紊乱,急性期过后可遗留贲门或幽门狭窄,出现呕吐等梗阻症状。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "急性腐蚀性胃炎" }, { "start_idx": 20, "end_idx": 28, "type": "sym", "entity": "口腔黏膜糜烂、水肿" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "中上腹剧痛" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "绞窄感" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "恶心" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "呕吐" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "呕血" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "黑粪" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "胃功能紊乱" }, { "start_idx": 69, "end_idx": 75, "type": "sym", "entity": "贲门或幽门狭窄" }, { "start_idx": 79, "end_idx": 80, "type": "sym", "entity": "呕吐" }, { "start_idx": 82, "end_idx": 85, "type": "sym", "entity": "梗阻症状" } ] }, { "text": "【实验室检查】感染因素引起者其末梢血白细胞计数一般增高,中性粒细胞比例增大。", "entities": [ { "start_idx": 15, "end_idx": 22, "type": "ite", "entity": "末梢血白细胞计数" }, { "start_idx": 28, "end_idx": 34, "type": "ite", "entity": "中性粒细胞比例" } ] }, { "text": "【影像学检查】(一)内镜检查胃黏膜明显充血、水肿,黏膜表面覆盖厚的黏稠炎性渗出物,糜烂性胃炎则在上述病变上见到点、圆、片、线状或不规则形糜烂,中心为红色新鲜出血或棕红色陈旧性出血,伴白苔或黄苔,常为多发亦可为单个。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "内镜检查" }, { "start_idx": 14, "end_idx": 23, "type": "sym", "entity": "胃黏膜明显充血、水肿" }, { "start_idx": 25, "end_idx": 39, "type": "sym", "entity": "黏膜表面覆盖厚的黏稠炎性渗出物" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "糜烂性胃炎" }, { "start_idx": 55, "end_idx": 69, "type": "sym", "entity": "点、圆、片、线状或不规则形糜烂" }, { "start_idx": 71, "end_idx": 79, "type": "sym", "entity": "中心为红色新鲜出血" }, { "start_idx": 81, "end_idx": 88, "type": "sym", "entity": "棕红色陈旧性出血" }, { "start_idx": 91, "end_idx": 92, "type": "sym", "entity": "白苔" }, { "start_idx": 94, "end_idx": 95, "type": "sym", "entity": "黄苔" }, { "start_idx": 97, "end_idx": 105, "type": "sym", "entity": "常为多发亦可为单个" } ] }, { "text": "做胃镜时应同时取胃黏膜做幽门螺杆菌检测。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "equ", "entity": "胃镜" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "胃黏膜" }, { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "幽门螺杆菌检测" } ] }, { "text": "(二)X线检查胃肠钡餐检查病变黏膜粗糙,局部压痛,但不能发现糜烂性病变,且不能用于急性或活动性出血患者。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "X线检查" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "胃肠钡餐" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "病变黏膜粗糙" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "局部压痛" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "糜烂性病变" }, { "start_idx": 41, "end_idx": 48, "type": "dis", "entity": "急性或活动性出血" } ] }, { "text": "【诊断与鉴别诊断】急性胃炎无特征性临床表现,诊断主要依靠病史及内镜检查,以上腹痛为主要症状者应与下列疾病鉴别。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "急性胃炎" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "内镜检查" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "腹痛" } ] }, { "text": "(一)急性胰腺炎有突然发作的上腹部剧烈疼痛,放射至背部及腰部,血清淀粉酶升高,B超或CT显示胰腺肿大,严重患者腹腔穿刺可抽出血性液体且淀粉酶增高。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "急性胰腺炎" }, { "start_idx": 9, "end_idx": 20, "type": "sym", "entity": "突然发作的上腹部剧烈疼痛" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "放射至背部及腰部" }, { "start_idx": 31, "end_idx": 35, "type": "ite", "entity": "血清淀粉酶" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "B超" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "CT" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "胰腺肿大" }, { "start_idx": 55, "end_idx": 58, "type": "pro", "entity": "腹腔穿刺" }, { "start_idx": 59, "end_idx": 65, "type": "sym", "entity": "可抽出血性液体" }, { "start_idx": 67, "end_idx": 69, "type": "ite", "entity": "淀粉酶" } ] }, { "text": "(二)胆道蛔虫症骤然发生上腹部剧烈绞痛,可放射至左、右肩部及背部,发作时辗转不安,剑突下偏右压痛明显,可伴呕吐,有时吐出蛔虫,B超见胆总管内有虫体异物。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "胆道蛔虫症" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "骤然发生上腹部剧烈绞痛" }, { "start_idx": 20, "end_idx": 31, "type": "sym", "entity": "可放射至左、右肩部及背部" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "发作时辗转不安" }, { "start_idx": 41, "end_idx": 49, "type": "sym", "entity": "剑突下偏右压痛明显" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "呕吐" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "吐出蛔虫" }, { "start_idx": 63, "end_idx": 64, "type": "pro", "entity": "B超" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "胆总管" } ] }, { "text": "【治疗】1.单纯性胃炎以对症治疗为主,去除病因,解痉止吐,口服黏膜保护剂,对细菌感染尤其伴有腹泻者可选用小檗碱、卡那霉素及氨苄西林等抗生素。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "单纯性胃炎" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "解痉止吐" }, { "start_idx": 29, "end_idx": 35, "type": "pro", "entity": "口服黏膜保护剂" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "细菌感染" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "腹泻" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "小檗碱" }, { "start_idx": 56, "end_idx": 59, "type": "dru", "entity": "卡那霉素" }, { "start_idx": 61, "end_idx": 64, "type": "dru", "entity": "氨苄西林" } ] }, { "text": "有幽门螺杆菌者,则应做清除治疗。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "清除治疗" } ] }, { "text": "2.糜烂性胃炎应控制出血,去除应激因素,可用H2受体拮抗剂:西咪替丁20~40mg/(kg•d),法莫替丁0.4~0.8mg/(kg•d),或质子泵阻滞剂奥美拉唑0.6~0.8mg/(kg•d),以及应用止血药如立止血注射,凝血酶口服等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "糜烂性胃炎" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "控制出血" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "去除应激因素" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "西咪替丁" }, { "start_idx": 55, "end_idx": 58, "type": "dru", "entity": "法莫替丁" }, { "start_idx": 77, "end_idx": 86, "type": "dru", "entity": "质子泵阻滞剂奥美拉唑" }, { "start_idx": 108, "end_idx": 110, "type": "dru", "entity": "止血药" }, { "start_idx": 112, "end_idx": 116, "type": "pro", "entity": "立止血注射" }, { "start_idx": 118, "end_idx": 122, "type": "pro", "entity": "凝血酶口服" } ] }, { "text": "3.腐蚀性胃炎应根据腐蚀剂性质给予相应中和药物,如口服镁乳氢氧化铝、牛奶和鸡蛋清等治疗强酸剂腐蚀。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "腐蚀性胃炎" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "中和药物" }, { "start_idx": 25, "end_idx": 39, "type": "pro", "entity": "口服镁乳氢氧化铝、牛奶和鸡蛋清" } ] }, { "text": "第十三章常见的泌尿外科疾病第一节异位肾当肾脏未能正常发育到达肾窝的位置,即为肾脏异位。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "异位肾" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肾脏" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肾窝" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "肾脏异位" } ] }, { "text": "其与肾下垂的区别在于肾下垂的肾脏最初都是位于正常的位置,随后下垂而异常的,但异位肾的位置从来都是异常的。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肾下垂" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾下垂" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肾脏" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "异位肾" } ] }, { "text": "随着产前超声检查的应用,异位肾的检出率有提高之势。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "超声检查" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "异位肾" } ] }, { "text": "双侧异位肾很少见,仅占异位肾总数的10%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "双侧异位肾" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "异位肾" } ] }, { "text": "肾脏异位的原因尚未明了,其与输尿管芽发育不良、后肾发育异常、遗传异常、致畸因子作用以及母亲的某些疾病有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "肾脏异位" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "输尿管芽发育不良" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "后肾发育异常" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "遗传异常" } ] }, { "text": "肾脏异位的分类根据肾脏所处的具体位置而定。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "肾脏异位" } ] }, { "text": "异位肾在上述位置的分布上无明显突出之处,比较均匀。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "异位肾" } ] }, { "text": "异位肾一般较正常为小,外形上可与正常有一些区别,但结构多正常,一般是直立的,也可侧转90°呈水平位。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "异位肾" } ] }, { "text": "25%是由于Ⅲ级以上的膀胱输尿管反流引起的,另25%是由于肾旋转不良引起。", "entities": [ { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "肾旋转不良" } ] }, { "text": "异位肾的动脉供应依其所处的位置而定,可发自腹主动脉的末端或分叉处,也可来自髂总及髂外动脉。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "异位肾" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "腹主动脉" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "髂总" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "髂外动脉" } ] }, { "text": "患儿多伴生殖系统畸形,如女孩中的双角子宫、单角子宫、子宫缺如、阴道缺如和重复阴道等。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "生殖系统畸形" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "双角子宫" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "单角子宫" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "子宫缺如" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "阴道缺如" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "重复阴道" } ] }, { "text": "除了肾积水和肾结石外,异位肾并不意味具有更多的其他问题。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肾积水" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "肾结石" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "异位肾" } ] }, { "text": "但异位肾失去了肋骨的保护,易受外伤。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "异位肾" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肋骨" } ] }, { "text": "有报告提出由于异位肾的血供异常而出现肾血管性高血压。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "异位肾" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "肾血管性高血压" } ] }, { "text": "第九节链球菌感染一、A族链球菌感染A族链球菌(groupAstreptococcic,GAS)又称化脓性链球菌(streptococcuspyogenes),是一种常见的革兰阳性致病菌,GAS感染可以引起多种疾病,从急性咽炎、扁桃体炎到严重的侵袭性感染如坏死性筋膜炎和中毒性休克综合征,无菌性后遗症如风湿热、肾小球肾炎等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "链球菌感染" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "A族链球菌感染" }, { "start_idx": 17, "end_idx": 21, "type": "mic", "entity": "A族链球菌" }, { "start_idx": 23, "end_idx": 41, "type": "mic", "entity": "groupAstreptococcic" }, { "start_idx": 43, "end_idx": 45, "type": "mic", "entity": "GAS" }, { "start_idx": 49, "end_idx": 54, "type": "mic", "entity": "化脓性链球菌" }, { "start_idx": 56, "end_idx": 76, "type": "mic", "entity": "streptococcuspyogenes" }, { "start_idx": 85, "end_idx": 91, "type": "mic", "entity": "革兰阳性致病菌" }, { "start_idx": 93, "end_idx": 97, "type": "dis", "entity": "GAS感染" }, { "start_idx": 108, "end_idx": 111, "type": "dis", "entity": "急性咽炎" }, { "start_idx": 113, "end_idx": 116, "type": "dis", "entity": "扁桃体炎" }, { "start_idx": 118, "end_idx": 125, "type": "dis", "entity": "严重的侵袭性感染" }, { "start_idx": 127, "end_idx": 132, "type": "dis", "entity": "坏死性筋膜炎" }, { "start_idx": 134, "end_idx": 141, "type": "dis", "entity": "中毒性休克综合征" }, { "start_idx": 143, "end_idx": 148, "type": "dis", "entity": "无菌性后遗症" }, { "start_idx": 150, "end_idx": 152, "type": "dis", "entity": "风湿热" }, { "start_idx": 154, "end_idx": 158, "type": "dis", "entity": "肾小球肾炎" } ] }, { "text": "【病原学】链球菌是革兰阳性球菌,常呈对状或链状生长,以它溶解红细胞的能力分类,可分为完全溶血(β溶血)、部分溶血(又称草绿色溶血,α溶血)和不溶血(γ溶血)三种。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "mic", "entity": "链球菌" }, { "start_idx": 9, "end_idx": 14, "type": "mic", "entity": "革兰阳性球菌" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "红细胞" } ] }, { "text": "因为溶血本身不构成致病性,兰斯菲尔德进一步按细胞壁的碳水化合物成分的不同将链球菌分为A-H和K-V族。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "细胞壁" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "碳水化合物成分" }, { "start_idx": 37, "end_idx": 39, "type": "mic", "entity": "链球菌" } ] }, { "text": "A族β溶血性链球菌细胞壁由明显的三层结构所组成,外层部分包含几种抗原蛋白,其中非常重要的是M蛋白。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "bod", "entity": "A族β溶血性链球菌细胞壁" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "抗原蛋白" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "M蛋白" } ] }, { "text": "A族β溶血性链球菌按其M蛋白抗原的不同可分为80多个血清型,M蛋白是细菌主要的毒力因子,它可黏附于人类上皮细胞和抵抗吞噬作用。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "mic", "entity": "A族β溶血性链球菌" }, { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "M蛋白抗原" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "血清" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "M蛋白" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "细菌" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "上皮细胞" } ] }, { "text": "细胞壁的另一种成分即脂胞壁酸,是另一种毒力因子,它可通过上皮细胞表面的纤维素加速细菌作用在黏膜、皮肤上。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "细胞壁" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "脂胞壁酸" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "纤维素" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "细菌" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "黏膜" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "皮肤" } ] }, { "text": "而透明质酸荚膜抵抗吞噬作用,进一步增加细菌毒力。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "透明质酸荚膜" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "细菌" } ] }, { "text": "获得性免疫是直接针对M蛋白的。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "M蛋白" } ] }, { "text": "链球菌产生毒素、酶和溶血素,当在人体组织中生长时,A族β溶血性链球菌释放约有20多种细胞外抗原成分。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "链球菌" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "毒素" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "酶" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "溶血素" }, { "start_idx": 25, "end_idx": 33, "type": "mic", "entity": "A族β溶血性链球菌" }, { "start_idx": 42, "end_idx": 46, "type": "mic", "entity": "细胞外抗原" } ] }, { "text": "在这些细胞外产物中,致热性红斑毒素(A、B和C)、链球菌素O和S、链激酶、脱氧核糖核酸酶和蛋白酶,具有很重要的临床意义。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "细胞" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "致热性红斑毒素" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "链球菌素O" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "链激酶" }, { "start_idx": 37, "end_idx": 43, "type": "bod", "entity": "脱氧核糖核酸酶" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "蛋白酶" } ] }, { "text": "致热性红斑毒素可引起猩红热的皮疹和中毒休克样综合征中的休克症状。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "致热性红斑毒素" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "猩红热" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "皮疹" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "中毒休克样综合征" } ] }, { "text": "链球菌素S引起细胞的粘连,破坏中性粒细胞和血小板的膜壁结构。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "链球菌素S" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "细胞" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "血小板" } ] }, { "text": "链球菌素O溶解红细胞,并对中性粒细胞有毒性作用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "链球菌素O" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "红细胞" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "中性粒细胞" } ] }, { "text": "链球菌素O和S使得这一β溶血性链球菌菌株产生清楚的溶血环。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "链球菌素O" }, { "start_idx": 11, "end_idx": 17, "type": "mic", "entity": "β溶血性链球菌" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "溶血环" } ] }, { "text": "细胞外的消化酶使得该细菌能在组织中迅速蔓延,例如:链激酶溶解纤维蛋白,DNA酶帮助脓液液化,透明质酸酶可帮助细菌穿透基底膜组织,蛋白酶同严重侵袭性感染造成的组织损害有关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "细胞" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "消化酶" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "细菌" }, { "start_idx": 25, "end_idx": 33, "type": "bod", "entity": "链激酶溶解纤维蛋白" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "DNA酶" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "脓液" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "透明质酸酶" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "细菌" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "基底膜组织" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "蛋白酶" }, { "start_idx": 68, "end_idx": 74, "type": "dis", "entity": "严重侵袭性感染" } ] }, { "text": "抗链球菌素O抗体(ASO)、抗DNaseB、透明质酸酶和链激酶抗体在A族链球菌疾病的血清学诊断中有一定作用。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "抗链球菌素O抗体" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "ASO" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "抗DNaseB" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "透明质酸酶" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "链激酶抗体" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "A族链球菌疾病" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "血清" } ] }, { "text": "M型特异性抗体在感染后的4~8周可检测出来,而抗生素的治疗可阻断这一反应。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "M型特异性抗体" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "抗生素" } ] }, { "text": "【流行病学】由A族链球菌引起化脓性和非化脓性并发症的发病率在20世纪80年代晚期和90年代呈增加趋势。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "mic", "entity": "A族链球菌" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "非化脓性并发症" } ] }, { "text": "这种严重链球菌感染性疾病的复燃的确切原因尚不知道,可能同流行菌株较以前更具有毒力有关。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "严重链球菌感染性疾病" } ] }, { "text": "A族链球菌是人类正常口咽部的菌群,儿童带菌率是15%~20%,发病率取决于小儿的年龄、季节、气候和地理位置,以及和感染者接触的程度有关。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "A族链球菌" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "口咽部" } ] }, { "text": "一般来说,婴儿期发病率最低,可能是经胎盘获得型特异性抗体的保护作用,也可能是婴儿期咽部尚缺乏同链球菌结合的受体。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "胎盘" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "特异性抗体" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "咽部" }, { "start_idx": 47, "end_idx": 49, "type": "mic", "entity": "链球菌" } ] }, { "text": "小于6岁儿童的链球菌皮肤感染是很常见的,5~15岁儿童链球菌性咽炎最常见,小于3岁的小儿链球菌疾病,包括猩红热不常见。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "链球菌皮肤感染" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "链球菌性咽炎" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "小儿链球菌疾病" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "猩红热" } ] }, { "text": "严重的侵袭性的A族链球菌感染可在任何年龄组出现。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "A族链球菌感染" } ] }, { "text": "链球菌咽炎在温带地区发病率较高,发病率和严重程度在寒冷季节增加,尤其在学龄儿童。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "链球菌咽炎" } ] }, { "text": "脓疱病在热带和温热气候时最易发生。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脓疱病" } ] }, { "text": "A族β溶血性链球菌是经飞沫传播的,咽部带菌者是常见的传染源,感染也可经接触破损的皮肤伤口,或经食物、牛奶和水来传播。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "mic", "entity": "A族β溶血性链球菌" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "咽部" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "皮肤" } ] }, { "text": "链球菌的感染常和家庭、学校、部队或拥挤的居住条件有关,破损的皮肤易于感染脓皮疹或脓疱疹,如水痘就可造成皮肤的破损,它不仅是链球菌感染的侵入途径,还使得宿主对其感染的反应性降低。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "链球菌" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "皮肤" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "脓皮疹" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "脓疱疹" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "水痘" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "皮肤" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "链球菌感染" } ] }, { "text": "在链球菌感染的急性期3~5天内是极易传染给他人的。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "链球菌感染" } ] }, { "text": "而在带菌期传染力减弱,型特异性免疫力可以由带菌或明显感染所诱导,链球菌在成人期随着对更多流行的血清型产生了免疫力,患病的危险大大减少了。", "entities": [ { "start_idx": 32, "end_idx": 34, "type": "mic", "entity": "链球菌" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "血清" } ] }, { "text": "【发病机制】在吸入或食入链球菌后,细菌通过表面原纤维、细胞壁脂胞壁酸附着在呼吸道上皮细胞上,原纤维含有抗吞噬细胞的特异性M蛋白的抗原决定簇,M蛋白同荚膜透明质酸一起抵抗吞噬作用,细胞外消化性酶通过干扰局部血栓形成(链球菌素)和脓液形成(DNA酶)使得感染的蔓延加快;同时蛋白酶、透明质酸酶增加了对结缔组织的消化。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "mic", "entity": "链球菌" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "细菌" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "表面原纤维" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "细胞壁" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "脂胞壁酸" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "呼吸道" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "原纤维" }, { "start_idx": 51, "end_idx": 65, "type": "mic", "entity": "抗吞噬细胞的特异性M蛋白的抗原" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "M蛋白" }, { "start_idx": 74, "end_idx": 79, "type": "bod", "entity": "荚膜透明质酸" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "细胞" }, { "start_idx": 92, "end_idx": 95, "type": "bod", "entity": "消化性酶" }, { "start_idx": 102, "end_idx": 103, "type": "bod", "entity": "血栓" }, { "start_idx": 107, "end_idx": 110, "type": "bod", "entity": "链球菌素" }, { "start_idx": 113, "end_idx": 114, "type": "bod", "entity": "脓液" }, { "start_idx": 118, "end_idx": 121, "type": "bod", "entity": "DNA酶" }, { "start_idx": 135, "end_idx": 137, "type": "bod", "entity": "蛋白酶" }, { "start_idx": 139, "end_idx": 143, "type": "bod", "entity": "透明质酸酶" }, { "start_idx": 148, "end_idx": 151, "type": "bod", "entity": "结缔组织" } ] }, { "text": "局部炎症后的化脓性并发症如扁桃体周围脓肿咽后壁脓肿中耳炎、鼻窦炎、淋巴管(淋巴结炎)的蔓延和菌血症(败血症、肺炎、骨髓炎)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "局部炎症" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "化脓性并发症" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "扁桃体" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "扁桃体周围脓肿" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "咽后壁" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "咽后壁脓肿" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "中耳炎" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "淋巴管" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "淋巴结炎" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "菌血症" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "败血症" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "肺炎" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "骨髓炎" } ] }, { "text": "引起猩红热临床症状的链球菌同非致热原外毒素菌株相似,只是增加猩红热样皮疹,血清学明确的致热原外毒素(A~C),在无免疫力的个体产生皮疹,皮疹可因宿主产生特异性的抗毒素抗体而减少。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "猩红热" }, { "start_idx": 10, "end_idx": 12, "type": "mic", "entity": "链球菌" }, { "start_idx": 14, "end_idx": 22, "type": "mic", "entity": "非致热原外毒素菌株" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "猩红热样皮疹" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "血清" }, { "start_idx": 43, "end_idx": 48, "type": "mic", "entity": "致热原外毒素" }, { "start_idx": 65, "end_idx": 66, "type": "dis", "entity": "皮疹" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "皮疹" }, { "start_idx": 80, "end_idx": 84, "type": "bod", "entity": "抗毒素抗体" } ] }, { "text": "这些毒素具有致热原性和细胞毒性,它们增加了外毒素的效果,并同中毒休克样症状产生有关。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "毒素" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "细胞" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "外毒素" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "中毒休克样症状" } ] }, { "text": "链球菌致热外毒素A有部分氨基酸同葡萄球菌肠毒素B同源,而后者同葡萄球菌中毒休克综合征的发生有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "链球菌" }, { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "致热外毒素" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "氨基酸" }, { "start_idx": 16, "end_idx": 23, "type": "bod", "entity": "葡萄球菌肠毒素B" }, { "start_idx": 31, "end_idx": 41, "type": "dis", "entity": "葡萄球菌中毒休克综合征" } ] }, { "text": "【临床表现】最常见的感染部位是呼吸道、皮肤、软组织和血液。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "呼吸道" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "皮肤" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "软组织" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "血液" } ] }, { "text": "常见的疾病有:咽炎、扁桃体炎、肺炎、皮肤感染、阴道炎、肛周疾病、败血症和菌血症,以及猩红热、链球菌中毒休克综合征、严重的侵袭性链球菌疾病。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "咽炎" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "扁桃体炎" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "肺炎" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "皮肤感染" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "阴道炎" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "肛周疾病" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "败血症" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "菌血症" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "猩红热" }, { "start_idx": 46, "end_idx": 55, "type": "dis", "entity": "链球菌中毒休克综合征" }, { "start_idx": 57, "end_idx": 67, "type": "dis", "entity": "严重的侵袭性链球菌疾病" } ] }, { "text": "(一)咽炎、扁桃体炎6个月~3岁的婴幼儿多见,主要表现为鼻咽炎、流清涕、低热,常见上唇周围表皮剥脱鼻咽炎临床上不易与其他病因的呼吸道感染鉴别。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "咽炎" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "扁桃体炎" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "鼻咽炎" }, { "start_idx": 32, "end_idx": 34, "type": "sym", "entity": "流清涕" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "低热" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "上唇" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "表皮" }, { "start_idx": 41, "end_idx": 48, "type": "sym", "entity": "上唇周围表皮剥脱" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "鼻咽炎" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "呼吸道感染" } ] }, { "text": "3~12岁小儿常患急性咽炎、急性滤泡性扁桃体炎、化脓性扁桃体炎,患儿有发热、咽痛、轻重不等的感染中毒全身症状。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "急性咽炎" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "急性滤泡性扁桃体炎" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "化脓性扁桃体炎" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "发热" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "咽痛" }, { "start_idx": 41, "end_idx": 53, "type": "sym", "entity": "轻重不等的感染中毒全身症状" } ] }, { "text": "(二)皮肤感染由A族β溶血性族链球菌引起的皮肤感染最常见的是表皮层脓疱病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "皮肤感染" }, { "start_idx": 8, "end_idx": 17, "type": "mic", "entity": "A族β溶血性族链球菌" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "皮肤感染" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "表皮层脓疱病" } ] }, { "text": "细菌在无破损的皮肤上生长约10天,在虫咬、划伤、小的创伤后,细菌可在皮下繁殖,发展成为脓疱病、深脓疱和蜂窝织炎。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "细菌" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "皮肤" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "细菌" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "皮下" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "脓疱病" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "深脓疱" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "蜂窝织炎" } ] }, { "text": "皮肤的脓疱病感染株常和患者咽部携带的菌株相同。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "皮肤" }, { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脓疱病" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "咽部" } ] }, { "text": "深部软组织感染可以出现如丹毒,它是一种急性、易区别的皮肤感染,同时伴淋巴管炎,可以累及面部,常常和咽炎有关。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "深部软组织感染" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "丹毒" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "皮肤感染" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "淋巴管炎" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "面部" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "咽炎" } ] }, { "text": "(三)猩红热猩红热(scarletfever)常见于3岁以上小儿,为急性呼吸道传染病。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "猩红热" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "猩红热" }, { "start_idx": 10, "end_idx": 21, "type": "dis", "entity": "scarletfever" }, { "start_idx": 34, "end_idx": 41, "type": "dis", "entity": "急性呼吸道传染病" } ] }, { "text": "以发热、咽炎及皮疹为特征,此病可由不同型的A族β溶血性链球菌引起,其细菌的致热毒素有A、B、C三种。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "发热" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "咽炎" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "皮疹" }, { "start_idx": 21, "end_idx": 29, "type": "mic", "entity": "A族β溶血性链球菌" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "细菌" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "毒素" } ] }, { "text": "感染其中一种后,再遇到其他任一种,仍可发生第二次或第三次猩红热,但这种机会很少。", "entities": [ { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "猩红热" } ] }, { "text": "起病急骤,有寒战、发热、全身不适、咽痛和皮肤出现弥漫性猩红色、约针头大小的丘疹,触之呈粗砂纸样。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "寒战" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "发热" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "全身不适" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "咽痛" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "皮肤出现弥漫性猩红色" }, { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "约针头大小的丘疹" } ] }, { "text": "体格检查:面颊部潮红口周围皮肤苍白口周苍白圈。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "面颊部" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "面颊部潮红" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "口" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "皮肤" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "口周围皮肤苍白" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "口周苍白圈" } ] }, { "text": "咽及扁桃体显著充血可见脓性渗出物。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "咽" }, { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "扁桃体" }, { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "咽及扁桃体显著充血" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "可见脓性渗出物" } ] }, { "text": "舌质红舌乳头红肿如草莓舌颈部及颌下淋巴结增大有触痛。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "舌质" }, { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "舌质红" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "舌乳头" }, { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "舌乳头红肿如草莓舌" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "颈部" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "颌下淋巴结" }, { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "颈部及颌下淋巴结增大" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "有触痛" } ] }, { "text": "于皮肤皱褶处如腋窝、肘、腹股沟等处,皮疹密集、色深红,其间有针头大小出血点,形成深红色横行“帕氏症”(Pastia’ssign)。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "皮肤" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "腋窝" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肘" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "腹股沟" }, { "start_idx": 18, "end_idx": 25, "type": "sym", "entity": "皮疹密集、色深红" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "有针头大小出血点" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "帕氏症" }, { "start_idx": 51, "end_idx": 62, "type": "dis", "entity": "Pastia’ssign" } ] }, { "text": "(四)链球菌中毒休克样综合征链球菌中毒休克样综合征(TSS)这一疾病是同菌株产生致热外毒素有关,其特征是低血压,伴随多器官、系统功能障碍葡萄球菌中毒休克综合征相区别。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "链球菌中毒休克样综合征" }, { "start_idx": 14, "end_idx": 24, "type": "dis", "entity": "链球菌中毒休克样综合征" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "TSS" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "致热外毒素" }, { "start_idx": 52, "end_idx": 54, "type": "sym", "entity": "低血压" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "器官" }, { "start_idx": 56, "end_idx": 67, "type": "sym", "entity": "伴随多器官、系统功能障碍" }, { "start_idx": 68, "end_idx": 78, "type": "dis", "entity": "葡萄球菌中毒休克综合征" } ] }, { "text": "经常是有A族链球菌感染的病灶,如菌血症、肺炎、蜂窝织炎,而咽炎常不存在,咽培养的结果可以是阴性的,但可以从其他部位分离出细菌。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "A族链球菌感染" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "菌血症" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肺炎" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "蜂窝织炎" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "咽炎" }, { "start_idx": 36, "end_idx": 38, "type": "pro", "entity": "咽培养" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "细菌" } ] }, { "text": "(五)严重侵袭性链球菌疾病是一组非常严重的侵袭性A族链球菌疾病,包括脓毒败血症、重症的猩红热、坏死性筋膜炎、TSS和败血症,在世界范围内,它们的发病率已在增加,其原因可能是因为更有毒性的菌株的流行。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "严重侵袭性链球菌疾病" }, { "start_idx": 21, "end_idx": 30, "type": "dis", "entity": "侵袭性A族链球菌疾病" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "脓毒败血症" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "重症的猩红热" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "坏死性筋膜炎" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "TSS" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "败血症" } ] }, { "text": "这些菌株可能同在18世纪引起世界范围内暴发性脓毒败血症的菌株相似。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "脓毒败血症" } ] }, { "text": "这些严重形式的A族链球菌疾病需要早期认识和有效的治疗。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "A族链球菌疾病" } ] }, { "text": "10%~20%的正常学龄儿童咽培养可有A族链球菌出现,这些携带者没有发生感染和风湿热的危险。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "咽培养" }, { "start_idx": 19, "end_idx": 23, "type": "mic", "entity": "A族链球菌" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "风湿热" } ] }, { "text": "有30%患有咽痛的儿童咽培养可以阳性,仅有一半人有血清抗体增高,说明有感染,而不是带菌者。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "咽痛" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "咽培养" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "血清抗体" } ] }, { "text": "链球菌咽炎患儿发病年龄多在5岁以上,有发热、咽部渗出物颈前淋巴结肿大猩红热样皮疹和A族链球菌暴露接触史。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "链球菌咽炎" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "发热" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "咽部" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "咽部渗出物" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "颈" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "淋巴结" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "颈前淋巴结肿大" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "猩红热样皮疹" }, { "start_idx": 41, "end_idx": 45, "type": "mic", "entity": "A族链球菌" } ] }, { "text": "但是许多患有急性感染的小儿可能仅有轻度症状,通过临床不能判断患A族链球菌感染,需有咽培养和抗原检测来明确诊断。", "entities": [ { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "A族链球菌感染" }, { "start_idx": 41, "end_idx": 43, "type": "pro", "entity": "咽培养" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "抗原检测" } ] }, { "text": "【实验室检查】(一)咽培养咽培养是非常有价值的实验室检查,帮助患有扁桃体炎和咽炎患者明确诊断。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "咽培养" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "咽培养" }, { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "实验室检查" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "扁桃体炎" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "咽炎" } ] }, { "text": "扁桃体和咽后壁有力地拭抹对于获得足够的标本是必要的,选择培养基以羊血培养基效果好,在3%的双氧水中无气泡产生(触酶试验阴性),并有β溶血菌落可以定为链球菌,而A族链球菌的生长可被杆菌肽纸片抑制;乳胶凝集试验也可证实。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "扁桃体" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "咽后壁" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "双氧水" }, { "start_idx": 55, "end_idx": 58, "type": "pro", "entity": "触酶试验" }, { "start_idx": 65, "end_idx": 69, "type": "mic", "entity": "β溶血菌落" }, { "start_idx": 74, "end_idx": 76, "type": "mic", "entity": "链球菌" }, { "start_idx": 79, "end_idx": 83, "type": "mic", "entity": "A族链球菌" }, { "start_idx": 89, "end_idx": 93, "type": "dru", "entity": "杆菌肽纸片" }, { "start_idx": 97, "end_idx": 102, "type": "pro", "entity": "乳胶凝集试验" } ] }, { "text": "因为溶血性链球菌常常栖居在正常儿童的咽部,因此,在咽炎患儿的咽部分离出A族链球菌不一定说明炎症就是由这一病原引起的。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "mic", "entity": "溶血性链球菌" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "咽部" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "咽炎" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "咽部" }, { "start_idx": 35, "end_idx": 39, "type": "mic", "entity": "A族链球菌" } ] }, { "text": "若细菌由中、重度渗出性咽炎的患儿标本中分离,并且他们的腭部有瘀斑和颈部淋巴结炎,这一诊断则是确定的。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "细菌" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "咽炎" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "腭部" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "瘀斑" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "颈部淋巴结炎" } ] }, { "text": "到目前为止,细菌抗原的快速检测的敏感性尚不高,但特异性较好。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "mic", "entity": "细菌抗原" } ] }, { "text": "(二)血常规周围血白细胞计数可以增高,也可以不增高,白细胞增加可出现在许多细菌感染性疾病。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "血常规" }, { "start_idx": 8, "end_idx": 13, "type": "ite", "entity": "血白细胞计数" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "白细胞" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "细菌感染性疾病" } ] }, { "text": "同样,血沉和C反应蛋白的增高也不能作为诊断的依据。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "血沉" }, { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "C反应蛋白" } ] }, { "text": "(三)免疫学反应1.ASO抗体水平的检测在病程的3~6周,80%链球菌咽炎患儿ASO抗体滴度大于166todd单位。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "ASO抗体" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "链球菌咽炎" }, { "start_idx": 39, "end_idx": 45, "type": "ite", "entity": "ASO抗体滴度" } ] }, { "text": "通过早期、有效抗生素的应用,这一反应可以被减弱或消除。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "抗生素" } ] }, { "text": "ASO滴度在风湿热患者可以很高。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "ASO滴度" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "风湿热" } ] }, { "text": "相反,脓疱病患者这一抗体是弱阳性或很低。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脓疱病" } ] }, { "text": "在肾小球肾炎的患者这一抗体反应个体差异较大。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "肾小球肾炎" } ] }, { "text": "2.抗DNA酶B抗体(ADB)水平的检测患有脓疱病的患儿可能对其链球菌细胞外产物的刺激具有强烈的反应。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "bod", "entity": "抗DNA酶B抗体" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "ADB" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "脓疱病" }, { "start_idx": 32, "end_idx": 39, "type": "bod", "entity": "链球菌细胞外产物" } ] }, { "text": "感染发生6~8周时抗体水平在链球菌性脓疱病患儿开始增高,许多有链球菌咽炎者也可产生针对这一酶的抗体。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "链球菌性脓疱病" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "链球菌咽炎" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "酶" } ] }, { "text": "3.脓疱病和咽炎患者也可产生针对透明质酸酶抗体反应,但这一反应不像ASO反应那样有规律。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脓疱病" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "咽炎" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "透明质酸酶抗体" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "ASO" } ] }, { "text": "当考虑患儿有近期感染时,应该测定多种链球菌产物的抗体反应滴度。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "mic", "entity": "链球菌" } ] }, { "text": "4.快速抗原检测法(rapidantigendetectiontest,RADT)诊断咽部GAS感染的正确性较高,具有简单快捷的优势,但对症状较轻或使用过抗生素治疗的患儿的诊断效能有所下降。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "快速抗原检测法" }, { "start_idx": 10, "end_idx": 34, "type": "pro", "entity": "rapidantigendetectiontest" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "RADT" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "咽部GAS感染" }, { "start_idx": 77, "end_idx": 81, "type": "pro", "entity": "抗生素治疗" } ] }, { "text": "【鉴别诊断】(一)病毒性咽炎咽部细菌培养常阴性,而特异病毒培养可以阳性,血清特异性抗体可增加。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "病毒性咽炎" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "咽部细菌培养" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "特异病毒培养" }, { "start_idx": 36, "end_idx": 42, "type": "bod", "entity": "血清特异性抗体" } ] }, { "text": "(二)感染性单核细胞增多症其临床症状,末梢血非典型淋巴细胞存在,异嗜血细胞和EB病毒滴度增高都是此病的特征。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "感染性单核细胞增多症" }, { "start_idx": 19, "end_idx": 28, "type": "bod", "entity": "末梢血非典型淋巴细胞" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "异嗜血细胞" }, { "start_idx": 38, "end_idx": 43, "type": "ite", "entity": "EB病毒滴度" } ] }, { "text": "(三)其他病原引起的急性咽炎如白喉、支原体感染等适当的咽培养和血清学检测可以排出这些疾病。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "急性咽炎" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "白喉" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "支原体感染" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "咽培养" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "血清学检测" } ] }, { "text": "(四)葡萄球菌皮肤感染链球菌性脓皮病应该同葡萄球菌皮肤感染相鉴别,两种细菌经常同时存在,培养是鉴别的最好方法。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "葡萄球菌皮肤感染" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "链球菌性脓皮病" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "葡萄球菌皮肤感染" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "细菌" } ] }, { "text": "(五)链球菌败血症、脑膜炎、化脓性关节炎和肺炎其病状无特异性,只有培养证实。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "链球菌败血症" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "化脓性关节炎" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "肺炎" } ] }, { "text": "【并发症】并发症一般反映链球菌从鼻咽部蔓延过程,引起鼻窦炎、中耳炎、乳突炎、颈淋巴结炎、咽后壁脓肿支气管肺炎。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "mic", "entity": "链球菌" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "鼻咽部" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "中耳炎" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "乳突炎" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "颈淋巴结炎" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "咽后壁" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "咽后壁脓肿" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "支气管肺炎" } ] }, { "text": "链球菌血源性传播可引起脑膜炎、骨髓炎或化脓性关节炎。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "链球菌" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "骨髓炎" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "化脓性关节炎" } ] }, { "text": "非化脓性晚期并发症包括风湿热和肾小球肾炎。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "非化脓性晚期并发症" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "风湿热" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "肾小球肾炎" } ] }, { "text": "【治疗】治疗的目的是减轻症状,预防败血症、化脓和非化脓性并发症。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "败血症" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "非化脓性并发症" } ] }, { "text": "青霉素是首选药物。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "青霉素" } ] }, { "text": "迄今为止,所有A族链球菌菌株都对青霉素和许多头孢菌素敏感。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "mic", "entity": "A族链球菌" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "青霉素" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "头孢菌素" } ] }, { "text": "但在严重感染时推荐青霉素和克林霉素联合应用,对青霉素过敏时大环内酯类抗生素作为首选药物,但我国已经有大环内酯类高耐药率及高水平耐药的报道。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "青霉素" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "克林霉素" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "青霉素" }, { "start_idx": 30, "end_idx": 36, "type": "dru", "entity": "环内酯类抗生素" } ] }, { "text": "对红霉素耐药程度在不同国家和地区有明显差异,它取决于应用红霉素的广泛程度;克林霉素耐药株少见。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "红霉素" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "红霉素" }, { "start_idx": 37, "end_idx": 40, "type": "dru", "entity": "克林霉素" } ] }, { "text": "对于深部感染部位,A族链球菌生长缓慢时,青霉素治疗效果不好。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "A族链球菌" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "青霉素" } ] }, { "text": "在血液和组织中青霉素的杀菌浓度维持至少10天,以便彻底杀死链球菌。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "青霉素" }, { "start_idx": 29, "end_idx": 31, "type": "mic", "entity": "链球菌" } ] }, { "text": "儿童链球菌咽炎青霉素250~500mg/次,一日2次或一日3次,疗程10天。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "链球菌" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "咽炎" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "青霉素" } ] }, { "text": "青霉素G或青霉素V也可使用,后者更好些,因为即使在空腹时它也可达到满意的血浓度。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "青霉素G" }, { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "青霉素V" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "血浓度" } ] }, { "text": "单剂量肌内注射长效的苯唑青霉素G(22kg以下小儿,60万U/次;22kg以上儿童120万U/次),对于治疗和预防复发是非常有效的。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "单剂量肌内注射" }, { "start_idx": 10, "end_idx": 15, "type": "dru", "entity": "苯唑青霉素G" } ] }, { "text": "红霉素40mg/(kg•d)、克林霉素30mg/(kg•d)或一代头孢菌素类可以用来治疗青霉素过敏的咽炎患者,一般来说,复发率是很低的。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "红霉素" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "克林霉素" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "头孢菌素" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "青霉素" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "咽炎" } ] }, { "text": "四环素和磺胺嘧啶不能用于治疗。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "四环素" }, { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "磺胺嘧啶" } ] }, { "text": "当一个疗程青霉素治疗后,仍有链球菌持续存在,被定义为治疗失败。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "青霉素" }, { "start_idx": 14, "end_idx": 16, "type": "mic", "entity": "链球菌" } ] }, { "text": "这种情况在5%~20%的儿童中出现,且常出现在口服治疗的患者,可能是由于再感染、持续携带β内酰胺酶阳性的菌株、对青霉素耐药株的带菌状态。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "口服治疗" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "β内酰胺酶" }, { "start_idx": 56, "end_idx": 58, "type": "dru", "entity": "青霉素" } ] }, { "text": "在高危人群,青霉素足疗程治疗后,应反复咽培养检查,对于咽培养再次阳性的患者,一些临床医生建议给予第二次治疗疗程。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "青霉素" }, { "start_idx": 19, "end_idx": 21, "type": "pro", "entity": "咽培养" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "咽培养" } ] }, { "text": "如果第二个疗程后仍是带菌状况,这时发展为风湿热的危险性很小,不需进一步治疗。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "风湿热" } ] }, { "text": "患有严重猩红热、链球菌菌血症、肺炎、脑膜炎、深部软组织感染、丹毒、链球菌中毒休克样综合征或链球菌咽炎并发症的病例,应该肌内或最好是静脉给予青霉素,其用量和疗程则依疾病的自然病程而定。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "猩红热" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "链球菌菌血症" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "肺炎" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "深部软组织感染" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "丹毒" }, { "start_idx": 33, "end_idx": 43, "type": "dis", "entity": "链球菌中毒休克样综合征" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "链球菌咽炎" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "肌内" }, { "start_idx": 69, "end_idx": 71, "type": "dru", "entity": "青霉素" } ] }, { "text": "而非常严重感染的病例,应给予40万U/(kg•24h)的大剂量青霉素。", "entities": [ { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "青霉素" } ] }, { "text": "有研究建议深部或坏死性感染可能需要增加一种二线抗生素,如克林霉素,以确保彻底杀死细菌。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "深部或坏死性感染" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "抗生素" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "克林霉素" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "细菌" } ] }, { "text": "TSS也可能需要附加的治疗,包括早期积极的液体供应,静脉免疫球蛋白或糖皮质激素的应用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "TSS" }, { "start_idx": 26, "end_idx": 32, "type": "dru", "entity": "静脉免疫球蛋白" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "糖皮质激素" } ] }, { "text": "【预防】如果在症状发生前应用青霉素,可以预防多数链球菌感染发生,对风湿热以外的其他感染化学药物预防效果尚不清。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "青霉素" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "链球菌感染" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "风湿热" } ] }, { "text": "或者60万U苯唑青霉素连同60万U水剂普鲁卡因青霉素,给予一次性肌内注射,这一用法适用于一定范围内的流行。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "U苯唑青霉素" }, { "start_idx": 16, "end_idx": 25, "type": "dru", "entity": "U水剂普鲁卡因青霉素" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肌内" } ] }, { "text": "对无症状的恢复期患者的持续咽培养阳性可不必再次治疗,因为他们一般是带菌者,不会有持续的或者再发的感染存在。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "咽培养" } ] }, { "text": "一些携带者常患有病毒性呼吸道感染,很像链球菌感染,可能被认为是再发性链球菌感染。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "病毒性呼吸道感染" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "链球菌感染" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "链球菌感染" } ] }, { "text": "应用非青霉素类药物如头孢菌素类、红霉素、克林霉素对于消灭带菌症状是有效的,但是对一些少见病例应慎用。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "青霉素" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "头孢菌素" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "红霉素" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "克林霉素" } ] }, { "text": "目前尚无临床可应用的A族链球菌疫苗。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "mic", "entity": "A族链球菌疫苗" } ] }, { "text": "【预后】经适当治疗的链球菌感染的预后是非常好的,多数化脓性并发症是可以预防和治疗的。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "链球菌感染" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "化脓性并发症" } ] }, { "text": "早期治疗是防止非化脓性并发症和完全治愈的关键。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "非化脓性并发症" } ] }, { "text": "尽管应用足够的疗程,但仍有少数病例如新生儿或有免疫缺陷的儿童患暴发性肺炎、败血症而发生死亡。", "entities": [ { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "肺炎" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "败血症" } ] }, { "text": "二、肾小球功能检查肾小球功能主要为滤过和清除功能。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "肾小球功能检查" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肾小球" } ] }, { "text": "临床上常将血肌酐、尿素氮、尿酸以及肾小球滤过率(GFR)、血浆蛋白清除率等作为肾小球滤过和清除功能的指标。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "血肌酐" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "尿素氮" }, { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "尿酸" }, { "start_idx": 17, "end_idx": 22, "type": "ite", "entity": "肾小球滤过率" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "GFR" }, { "start_idx": 29, "end_idx": 35, "type": "ite", "entity": "血浆蛋白清除率" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "肾小球" } ] }, { "text": "(一)血尿素氮及血肌酐血尿素氮(BUN)及血肌酐(Scr)来评估肾小球滤过率(GFR),已在临床得到广泛应用。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "血尿素氮" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "血肌酐" }, { "start_idx": 11, "end_idx": 14, "type": "ite", "entity": "血尿素氮" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "BUN" }, { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "血肌酐" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "Scr" }, { "start_idx": 32, "end_idx": 37, "type": "ite", "entity": "肾小球滤过率" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "GFR" } ] }, { "text": "BUN正常值新生儿为1.8~6.4mmol/L,婴儿及儿童为2.5~6.4mmol/L,青春期为2.9~7.5mmol/L。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "BUN" } ] }, { "text": "尿素主要由肾小球滤过,可以从BUN上升来评估GFR下降的程度。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "尿素" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肾小球" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "BUN" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "GFR" } ] }, { "text": "但尿素的产生不如肌酐恒定,受多种因素影响:①肝脏合成尿素是将未合成蛋白质的氨基酸脱氨后再合成尿素,当肝内氨基酸代谢增加时(如高蛋白饮食),以及组织分解代谢旺盛而蛋白合成减少时,尿素生成增加,BUN上升。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "尿素" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肌酐" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肝脏" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "尿素" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "蛋白质" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "氨基酸" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "尿素" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "肝" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "氨基酸" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "组织" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "蛋白" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "尿素" }, { "start_idx": 95, "end_idx": 97, "type": "ite", "entity": "BUN" } ] }, { "text": "②严重肝病或低蛋白饮食时BUN会下降。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "严重肝病" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "BUN" } ] }, { "text": "③尿素排泄不仅决定于肾小球滤过,正常时约有40%~50%的尿素随着钠及水一起而被肾小管被动回吸收。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "尿素" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肾小球" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "尿素" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "钠" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "肾小管" } ] }, { "text": "血容量下降时,尿素可随着近曲小管钠的回吸收增加而增加使BUN升高,这时并不代表GFR下降。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "血" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "尿素" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "钠" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "BUN" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "GFR" } ] }, { "text": "④BUN与GFR之间的负相关并非直线关系而是呈反抛物线形式,只有当GFR下降至50%~60%以上才引起BUN上升。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "ite", "entity": "BUN" }, { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "GFR" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "GFR" }, { "start_idx": 51, "end_idx": 53, "type": "ite", "entity": "BUN" } ] }, { "text": "因此,在用BUN评估肾脏病进展时必须考虑多方面因素的影响,尤其是饮食中蛋白质含量。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "BUN" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾脏病" } ] }, { "text": "如GFR稳定时,饮食中蛋白质加倍,BUN将升高而造成肾功能下降的错觉。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "ite", "entity": "GFR" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "BUN" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "肾" } ] }, { "text": "此外,小儿分解代谢旺盛如发热时也可出现一过性BUN升高。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "发热" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "BUN" } ] }, { "text": "血肌酐(Scr)浓度不受蛋白质摄入量影响,是更可靠而有用的肾功能指标。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "血肌酐" }, { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "Scr" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "蛋白质" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "肾" } ] }, { "text": "肌酐是体内肌酸代谢的终产物,分子量为113D,因而正常人Scr浓度也极为恒定,人每日的动态变化小于10%。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肌酐" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肌酸" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "Scr" } ] }, { "text": "血肌酐的浓度直接与体内肌肉组织的总量有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "血肌酐" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "肌肉组织" } ] }, { "text": "故Scr的正常值与性别、年龄和身长有关。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "ite", "entity": "Scr" } ] }, { "text": "可参照下列两个公式:男孩Scr(mg/dl)=0.35+0.03×年龄女孩Scr(mg/dl)=0.37+0.02×年龄或Scr(mg/dl)=0.004×身长(cm)小儿血肌酐正常值约为27~62μmol/L,随年龄增长及肌肉发育而增高。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "Scr" }, { "start_idx": 37, "end_idx": 39, "type": "ite", "entity": "Scr" }, { "start_idx": 61, "end_idx": 63, "type": "ite", "entity": "Scr" }, { "start_idx": 86, "end_idx": 88, "type": "ite", "entity": "血肌酐" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "肌肉" } ] }, { "text": "正常小儿血肌酐初生时较高,接近成人水平,约2~4周下降至8.84~17.68μmol/L。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "血肌酐" } ] }, { "text": "当GFR降至正常70%以下时,Scr才可能升高。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "ite", "entity": "GFR" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "Scr" } ] }, { "text": "(二)肾小球滤过率肾小球滤过率(glomerularfiltrationrate,GFR)是指单位时间(分钟)内从双肾滤过的血浆毫升数。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "ite", "entity": "肾小球滤过率" }, { "start_idx": 9, "end_idx": 14, "type": "ite", "entity": "肾小球滤过率" }, { "start_idx": 16, "end_idx": 39, "type": "ite", "entity": "glomerularfiltrationrate" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "GFR" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "肾" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "血浆" } ] }, { "text": "临床上常用清除率来表示GFR。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "清除率" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "GFR" } ] }, { "text": "清除率指每分钟多少毫升血中的某物质被肾小球滤过清除,当这种物质既不被肾小管重吸收,也不被肾小管分泌时,可以认定该物质的清除率与GFR相等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "清除率" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "血" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肾小球" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "肾小管" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "肾小管" }, { "start_idx": 59, "end_idx": 61, "type": "ite", "entity": "清除率" }, { "start_idx": 63, "end_idx": 65, "type": "ite", "entity": "GFR" } ] }, { "text": "菊粉(inulin)是从植物块基中提取的不带电荷的果糖聚合物,分子量5.2kD,只从肾小球滤过,不被肾小管重吸收和分泌,故菊粉清除率(Cin)=GFR,Cin被认为是测定GFR的金标准。", "entities": [ { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "肾小球" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "肾小管" }, { "start_idx": 61, "end_idx": 65, "type": "ite", "entity": "菊粉清除率" }, { "start_idx": 67, "end_idx": 69, "type": "ite", "entity": "Cin" }, { "start_idx": 72, "end_idx": 74, "type": "ite", "entity": "GFR" }, { "start_idx": 76, "end_idx": 78, "type": "ite", "entity": "Cin" }, { "start_idx": 85, "end_idx": 87, "type": "ite", "entity": "GFR" } ] }, { "text": "但菊粉需给病人静脉注入,然后分时段多次采血后以高效液相色谱法分析后才能计算出Cin,过程繁琐,程序复杂,因而临床上仍普遍以内生肌酐廓清率(Ccr)来代替Cin。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dru", "entity": "菊粉" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "静脉注入" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "采血" }, { "start_idx": 23, "end_idx": 31, "type": "pro", "entity": "高效液相色谱法分析" }, { "start_idx": 38, "end_idx": 40, "type": "ite", "entity": "Cin" }, { "start_idx": 61, "end_idx": 67, "type": "ite", "entity": "内生肌酐廓清率" }, { "start_idx": 69, "end_idx": 71, "type": "ite", "entity": "Ccr" }, { "start_idx": 76, "end_idx": 78, "type": "ite", "entity": "Cin" } ] }, { "text": "内生肌酐由体内肌酸衍生而来,其产生及排泄均较稳定。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "内生肌酐" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肌酸" } ] }, { "text": "正常儿童尿中肌酐排出量约为[15+0.5×年龄(岁)]mg/(kg•24h),由肾小球滤过后,既不为肾小管回吸收,也不为肾脏代谢,但可由近曲小管少量分泌。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "尿" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肌酐" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "肾小球" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "肾小管" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "肾脏" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "近曲小管" } ] }, { "text": "因而,正常人肌酐排泄量超过滤过量的10%~20%。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "肌酐排泄量" } ] }, { "text": "但在血浆中也有10%~20%非肌酐的色素物质可与肌酐一起用苦味酸比色法测出,故恰与尿中多出的抵消。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血浆" }, { "start_idx": 14, "end_idx": 21, "type": "bod", "entity": "非肌酐的色素物质" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肌酐" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "苦味酸比色法" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "尿" } ] }, { "text": "因此,肌酐廓清率可准确反映肾小球滤过率(GFR)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "肌酐廓清率" }, { "start_idx": 13, "end_idx": 18, "type": "ite", "entity": "肾小球滤过率" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "GFR" } ] }, { "text": "GFR下降至<40ml/(min•1.73m2)时,滤过的肌酐减少,肾小管分泌的增加,此时Ccr将比实际GFR高出10%~40%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "GFR" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肌酐" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "肾小管" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "Ccr" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "GFR" } ] }, { "text": "肌酐廓清率的测定方法是测定同一时间内血及尿中的肌酐浓度,并准确测定尿量(留4小时或12小时尿),计算出肾脏每分钟清除血浆中肌酐的毫升数,再用患儿体表面积矫正即得出矫正肌酐廓清率:矫正廓清率=内生肌酐廓清率×1.73(m2)/小儿实际体表面积(m2)正常新生儿为25~27ml/(min•1.73m2),婴儿期60~80ml/(min1.73m2),2岁以后的小儿矫正内生肌酐廓清率为80~120ml/(min•1.73m2)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "肌酐廓清率" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "血" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "肌酐浓度" }, { "start_idx": 33, "end_idx": 34, "type": "ite", "entity": "尿量" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "肾脏" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "血浆" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "肌酐" }, { "start_idx": 81, "end_idx": 87, "type": "ite", "entity": "矫正肌酐廓清率" }, { "start_idx": 89, "end_idx": 93, "type": "ite", "entity": "矫正廓清率" }, { "start_idx": 95, "end_idx": 101, "type": "ite", "entity": "内生肌酐廓清率" }, { "start_idx": 205, "end_idx": 213, "type": "ite", "entity": "矫正内生肌酐廓清率" } ] }, { "text": "由于血肌酐浓度十分恒定,其变化与肾小球滤过率高低十分密切。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "血肌酐" }, { "start_idx": 16, "end_idx": 21, "type": "ite", "entity": "肾小球滤过率" } ] }, { "text": "因此不少人试图以血肌酐来拟算肾小球滤过率。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "血肌酐" }, { "start_idx": 14, "end_idx": 19, "type": "ite", "entity": "肾小球滤过率" } ] }, { "text": "Schwartz等研究Scr/GFR和身长间的关系,得出公式为:矫正Ccr=K×身长(cm)/Scr(mg/dl)式中K为单位体重排肌酐的常数,2~12岁小儿为0.55,≤1岁婴儿为0.45,≤1岁的低体重儿为0.33。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "Scr" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "GFR" }, { "start_idx": 34, "end_idx": 36, "type": "ite", "entity": "Ccr" }, { "start_idx": 47, "end_idx": 49, "type": "ite", "entity": "Scr" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "肌酐" } ] }, { "text": "第十章小儿腹泻病在未明确病因前,大便性状改变与大便次数比平时增多,统称为腹泻病(diarrhealdisease)。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "小儿腹泻病" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "大便性状改变" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "大便次数比平时增多" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "腹泻病" }, { "start_idx": 41, "end_idx": 56, "type": "dis", "entity": "diarrhealdisease" } ] }, { "text": "全球大约每年至少10亿人次发生腹泻,根据世界卫生组织调查,每天大约1万人死于腹泻。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "腹泻" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "腹泻" } ] }, { "text": "在我国,腹泻病同样是儿童的常见病,据有关资料,我国5岁以下儿童腹泻病的年发病率为201%,平均每年每个儿童年发病3.5次,其死亡率为0.51%。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "腹泻" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "儿童腹泻病" } ] }, { "text": "因此,对小儿腹泻病的防治十分重要。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "小儿腹泻病" } ] }, { "text": "根据病程腹泻病分为:急性腹泻病(acutediarrhealdisease)病程在2周以内;迁延性腹泻病(persistentdiarrhealdisease)病程在2周~2个月;慢性腹泻病(chronicdiarrhealdisease)病程在2个月以上。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "腹泻病" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "急性腹泻病" }, { "start_idx": 16, "end_idx": 36, "type": "dis", "entity": "acutediarrhealdisease" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "迁延性腹泻病" }, { "start_idx": 53, "end_idx": 78, "type": "dis", "entity": "persistentdiarrhealdisease" }, { "start_idx": 90, "end_idx": 94, "type": "dis", "entity": "慢性腹泻病" }, { "start_idx": 96, "end_idx": 118, "type": "dis", "entity": "chronicdiarrhealdisease" } ] }, { "text": "按病情分为:轻型:无脱水,无中毒症状;中型:轻度至中度脱水或有中毒症状;重型:重度脱水或有明显中毒症状(烦躁、精神萎靡、嗜睡、面色苍白。", "entities": [ { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "轻度至中度脱水" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "中毒" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "有中毒症状" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "重度脱水" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "明显中毒症状" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "烦躁" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "精神萎靡" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "嗜睡" }, { "start_idx": 63, "end_idx": 64, "type": "ite", "entity": "面色" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "面色苍白" } ] }, { "text": "高热或体温不升以及白细胞计数明显增高痢疾、霍乱、病毒及其他感染性腹泻等;非感染性,包括食饵性(饮食性)腹泻;症状性腹泻,如过敏性腹泻;其他腹泻病如乳糖不耐症及糖原性腹泻等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "高热" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "体温不升" }, { "start_idx": 9, "end_idx": 13, "type": "ite", "entity": "白细胞计数" }, { "start_idx": 9, "end_idx": 17, "type": "sym", "entity": "白细胞计数明显增高" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "痢疾" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "霍乱" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "感染性腹泻" }, { "start_idx": 43, "end_idx": 52, "type": "dis", "entity": "食饵性(饮食性)腹泻" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "症状性腹泻" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "过敏性腹泻" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "其他腹泻病" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "乳糖不耐症" }, { "start_idx": 79, "end_idx": 83, "type": "dis", "entity": "糖原性腹泻" } ] }, { "text": "(一)内在因素1.消化系统发育不成熟婴幼儿时期,胃酸及消化酶分泌不足,消化酶的活性较低,神经系统对胃肠道调节功能较差,不易适应食物的质和量,且生长发育快,营养物质的需要相对较多,胃肠道负担较大,消化功能经常处于紧张状态,易发生消化功能紊乱。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "胃酸" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "消化酶" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "消化酶" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "神经系统" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "胃肠道" }, { "start_idx": 89, "end_idx": 91, "type": "bod", "entity": "胃肠道" }, { "start_idx": 113, "end_idx": 118, "type": "dis", "entity": "消化功能紊乱" } ] }, { "text": "2.机体防御功能较差婴幼儿时期免疫功能相对不够成熟,血液中的免疫球蛋白和胃肠道sIgA均较低,胃肠屏障功能较弱,胃酸分泌量少,胃肠排空较快,对感染因素防御功能差。", "entities": [ { "start_idx": 30, "end_idx": 34, "type": "ite", "entity": "免疫球蛋白" }, { "start_idx": 36, "end_idx": 42, "type": "ite", "entity": "胃肠道sIgA" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "胃酸" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "胃肠" } ] }, { "text": "另外,新生儿,出生后尚未建立完善的肠道正常菌群,对侵入肠道的病原微生物拮抗能力弱,人工喂养者食物中缺乏母乳含有的大量免疫物质,且食物和食具污染机会较多,肠道感染的发生率明显高于母乳喂养儿。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肠道" }, { "start_idx": 30, "end_idx": 34, "type": "mic", "entity": "病原微生物" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "母乳" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "肠道" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "母乳" } ] }, { "text": "3.体液分布特点婴儿细胞间质液较多,且水代谢旺盛,肾功能调节差,易发生体液紊乱。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "体液" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "细胞间质液" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肾" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "体液紊乱" } ] }, { "text": "(二)感染因素1.肠道感染主要由细菌和病毒引起。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "肠道感染" }, { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "细菌" }, { "start_idx": 19, "end_idx": 20, "type": "mic", "entity": "病毒" } ] }, { "text": "(1)细菌:除法定传染病外。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "细菌" } ] }, { "text": "①大肠杆菌(Escherichiacoli,E.Coli):按其致病机制分类为致病性大肠杆菌(Entero-pathogenicE.Coli,EPEC)、产毒素性大肠杆菌(EnterotoxigenicE.Coli,ETEC)、侵袭性大肠杆菌(EnteroinvasiveE.Coli,EIEC)、出血性大肠杆菌(EnterohemorrhagicE.Coli,EHEC)以及黏附性大肠杆菌(EnteroadhesiveE.Coli,EAEC);②空肠弯曲菌(Campylobacterjejuni);③耶尔森菌(Yersiniaentero-colitica);④其他:鼠伤寒沙门菌(Salmonellatyphimurium)、变形杆菌(proteus)、铜绿假单胞菌(Pseudomonasaeruginosa)、克雷伯菌(Klebsiella)、金黄色葡萄球菌(Staphlococcusaureus)以及难辨梭状芽胞杆菌(Clostridiumdifficile)等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 6, "end_idx": 20, "type": "mic", "entity": "Escherichiacoli" }, { "start_idx": 22, "end_idx": 27, "type": "mic", "entity": "E.Coli" }, { "start_idx": 39, "end_idx": 45, "type": "mic", "entity": "致病性大肠杆菌" }, { "start_idx": 71, "end_idx": 74, "type": "mic", "entity": "EPEC" }, { "start_idx": 77, "end_idx": 84, "type": "mic", "entity": "产毒素性大肠杆菌" }, { "start_idx": 86, "end_idx": 106, "type": "mic", "entity": "EnterotoxigenicE.Coli" }, { "start_idx": 108, "end_idx": 111, "type": "mic", "entity": "ETEC" }, { "start_idx": 114, "end_idx": 120, "type": "mic", "entity": "侵袭性大肠杆菌" }, { "start_idx": 122, "end_idx": 141, "type": "mic", "entity": "EnteroinvasiveE.Coli" }, { "start_idx": 143, "end_idx": 146, "type": "mic", "entity": "EIEC" }, { "start_idx": 149, "end_idx": 155, "type": "mic", "entity": "出血性大肠杆菌" }, { "start_idx": 157, "end_idx": 179, "type": "mic", "entity": "EnterohemorrhagicE.Coli" }, { "start_idx": 181, "end_idx": 184, "type": "mic", "entity": "EHEC" }, { "start_idx": 188, "end_idx": 194, "type": "mic", "entity": "黏附性大肠杆菌" }, { "start_idx": 196, "end_idx": 215, "type": "mic", "entity": "EnteroadhesiveE.Coli" }, { "start_idx": 217, "end_idx": 220, "type": "mic", "entity": "EAEC" }, { "start_idx": 224, "end_idx": 228, "type": "mic", "entity": "空肠弯曲菌" }, { "start_idx": 230, "end_idx": 248, "type": "mic", "entity": "Campylobacterjejuni" }, { "start_idx": 252, "end_idx": 255, "type": "mic", "entity": "耶尔森菌" }, { "start_idx": 257, "end_idx": 279, "type": "mic", "entity": "Yersiniaentero-colitica" }, { "start_idx": 286, "end_idx": 291, "type": "mic", "entity": "鼠伤寒沙门菌" }, { "start_idx": 293, "end_idx": 313, "type": "mic", "entity": "Salmonellatyphimurium" }, { "start_idx": 316, "end_idx": 319, "type": "mic", "entity": "变形杆菌" }, { "start_idx": 321, "end_idx": 327, "type": "mic", "entity": "proteus" }, { "start_idx": 330, "end_idx": 335, "type": "mic", "entity": "铜绿假单胞菌" }, { "start_idx": 337, "end_idx": 357, "type": "mic", "entity": "Pseudomonasaeruginosa" }, { "start_idx": 360, "end_idx": 363, "type": "mic", "entity": "克雷伯菌" }, { "start_idx": 365, "end_idx": 374, "type": "mic", "entity": "Klebsiella" }, { "start_idx": 377, "end_idx": 383, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 385, "end_idx": 403, "type": "mic", "entity": "Staphlococcusaureus" }, { "start_idx": 407, "end_idx": 414, "type": "mic", "entity": "难辨梭状芽胞杆菌" }, { "start_idx": 416, "end_idx": 435, "type": "mic", "entity": "Clostridiumdifficile" } ] }, { "text": "(2)病毒:①轮状病毒(humanrotavirus),是引起婴幼儿腹泻的主要病原;②诺如病毒(Norovirus);③肠道腺病毒(adenovirus);④其他,如星状病毒(astrovirus)、杯状病毒(calicivirus)及冠状病毒(coronavirus)等。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "mic", "entity": "轮状病毒" }, { "start_idx": 12, "end_idx": 25, "type": "mic", "entity": "humanrotavirus" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "婴幼儿腹泻" }, { "start_idx": 43, "end_idx": 46, "type": "mic", "entity": "诺如病毒" }, { "start_idx": 48, "end_idx": 56, "type": "mic", "entity": "Norovirus" }, { "start_idx": 60, "end_idx": 64, "type": "mic", "entity": "肠道腺病毒" }, { "start_idx": 66, "end_idx": 75, "type": "mic", "entity": "adenovirus" }, { "start_idx": 83, "end_idx": 86, "type": "mic", "entity": "星状病毒" }, { "start_idx": 88, "end_idx": 97, "type": "mic", "entity": "astrovirus" }, { "start_idx": 100, "end_idx": 103, "type": "mic", "entity": "杯状病毒" }, { "start_idx": 105, "end_idx": 115, "type": "mic", "entity": "calicivirus" }, { "start_idx": 118, "end_idx": 121, "type": "mic", "entity": "冠状病毒" }, { "start_idx": 123, "end_idx": 133, "type": "mic", "entity": "coronavirus" } ] }, { "text": "(3)真菌和原虫:真菌感染主要为白色念珠菌(Candidaalbicans),一些原虫的感染如蓝氏贾第鞭毛虫(Giardialam-blia)、结肠小袋虫(Balantidiumcoli)、隐孢子虫(Cryptosporidi-um)及阿米巴原虫(Entamoebahistolytica)等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "真菌" }, { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "原虫" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "真菌感染" }, { "start_idx": 16, "end_idx": 20, "type": "mic", "entity": "白色念珠菌" }, { "start_idx": 22, "end_idx": 36, "type": "mic", "entity": "Candidaalbicans" }, { "start_idx": 47, "end_idx": 53, "type": "mic", "entity": "蓝氏贾第鞭毛虫" }, { "start_idx": 55, "end_idx": 69, "type": "mic", "entity": "Giardialam-blia" }, { "start_idx": 72, "end_idx": 76, "type": "mic", "entity": "结肠小袋虫" }, { "start_idx": 78, "end_idx": 92, "type": "mic", "entity": "Balantidiumcoli" }, { "start_idx": 95, "end_idx": 98, "type": "mic", "entity": "隐孢子虫" }, { "start_idx": 100, "end_idx": 115, "type": "mic", "entity": "Cryptosporidi-um" }, { "start_idx": 118, "end_idx": 122, "type": "mic", "entity": "阿米巴原虫" }, { "start_idx": 124, "end_idx": 143, "type": "mic", "entity": "Entamoebahistolytica" } ] }, { "text": "2.肠道外感染小儿患上呼吸道感染、肺炎、肾盂肾炎、中耳炎、皮肤感染及其他急性感染性疾病时可伴有腹泻。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肠道外感染" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "肺炎" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "中耳炎" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "皮肤感染" }, { "start_idx": 34, "end_idx": 42, "type": "dis", "entity": "其他急性感染性疾病" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "腹泻" } ] }, { "text": "这是由于发热及病原体毒素的影响,使消化功能紊乱,酶分泌减少肠蠕动增加。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "发热" }, { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "病原体" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "消化功能紊乱" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "酶" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "酶分泌减少" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "肠" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "肠蠕动增加" } ] }, { "text": "腹泻的主要原因之一。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "腹泻" } ] }, { "text": "过多过早喂哺大量淀粉类、脂肪类食物,突然改变食物品种和断奶等均可导致腹泻。", "entities": [ { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "腹泻" } ] }, { "text": "气候的突然变化,使肠蠕动增加,消化酶和胃酸分泌减少,可诱发腹泻。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肠" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "消化酶" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胃酸" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "腹泻" } ] }, { "text": "一些吸收不良综合征如乳糖不耐受症、糖原性腹泻、先天性氯化物性腹泻、遗传性果糖不耐受症、胰腺囊性纤维性变以及原发性肠吸收不良等都可引起腹泻。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "吸收不良综合征" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "乳糖不耐受症" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "糖原性腹泻" }, { "start_idx": 23, "end_idx": 31, "type": "dis", "entity": "先天性氯化物性腹泻" }, { "start_idx": 33, "end_idx": 41, "type": "dis", "entity": "遗传性果糖不耐受症" }, { "start_idx": 43, "end_idx": 50, "type": "dis", "entity": "胰腺囊性纤维性变" }, { "start_idx": 53, "end_idx": 60, "type": "dis", "entity": "原发性肠吸收不良" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "腹泻" } ] }, { "text": "牛奶蛋白过敏者,吃牛乳后48小时发生水样泻。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "水样泻" } ] }, { "text": "【发病机制】不同病因引起腹泻的机制不同,可通过以下几种机制致病。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "腹泻" } ] }, { "text": "(一)非感染因素主要是饮食的量和质不恰当,使婴儿消化道功能发生障碍,食物不能充分消化和吸收,积滞于肠道上部,同时酸度下降,有利于肠道下部细菌上移繁殖,使消化功能紊乱。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "消化道" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "肠道上部" }, { "start_idx": 56, "end_idx": 57, "type": "ite", "entity": "酸度" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "肠道下部" }, { "start_idx": 68, "end_idx": 69, "type": "mic", "entity": "细菌" }, { "start_idx": 76, "end_idx": 81, "type": "dis", "entity": "消化功能紊乱" } ] }, { "text": "肠道内产生大量的乳酸、乙酸等有机酸,使肠腔渗透压增强,引起腹泻。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肠道" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "乳酸" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "乙酸" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "有机酸" }, { "start_idx": 19, "end_idx": 23, "type": "ite", "entity": "肠腔渗透压" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "腹泻" } ] }, { "text": "(二)感染因素病原微生物能否引起肠道感染,取决于宿主防御功能的强弱、感染量的大小以及微生物毒力(黏附性、产毒性及侵袭性)、细胞毒性,其中微生物的黏附能力对于肠道感染至关重要。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "mic", "entity": "病原微生物" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "肠道感染" }, { "start_idx": 34, "end_idx": 36, "type": "ite", "entity": "感染量" }, { "start_idx": 42, "end_idx": 44, "type": "mic", "entity": "微生物" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "细胞" }, { "start_idx": 68, "end_idx": 70, "type": "mic", "entity": "微生物" } ] }, { "text": "1.细菌性肠炎主要通过细菌产生毒素作用及细菌侵袭性作用为主要发病机制。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "细菌" } ] }, { "text": "(1)肠毒素性肠炎:病原菌不侵入肠黏膜,不引起病理形态学上的变化,仅附着于完整的肠绒毛上,通过产生肠毒素致病。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "肠毒素性肠炎" }, { "start_idx": 10, "end_idx": 12, "type": "mic", "entity": "病原菌" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "肠黏膜" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "肠绒毛" } ] }, { "text": "典型的细菌为ETEC和霍乱弧菌。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "细菌" }, { "start_idx": 6, "end_idx": 9, "type": "mic", "entity": "ETEC" }, { "start_idx": 11, "end_idx": 14, "type": "mic", "entity": "霍乱弧菌" } ] }, { "text": "其他细菌也可产生肠毒素,如耶尔森菌、鼠伤寒沙门菌、金黄色葡萄球菌、变形杆菌及空肠弯曲菌等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "细菌" }, { "start_idx": 13, "end_idx": 16, "type": "mic", "entity": "耶尔森菌" }, { "start_idx": 18, "end_idx": 23, "type": "mic", "entity": "鼠伤寒沙门菌" }, { "start_idx": 25, "end_idx": 31, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 33, "end_idx": 36, "type": "mic", "entity": "变形杆菌" }, { "start_idx": 38, "end_idx": 42, "type": "mic", "entity": "空肠弯曲菌" } ] }, { "text": "以ETEC为例,通过其菌毛黏附在小肠微绒毛上,生长繁殖,产生大量肠毒素。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "mic", "entity": "ETEC" }, { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "菌毛" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "小肠微绒毛" }, { "start_idx": 32, "end_idx": 34, "type": "mic", "entity": "肠毒素" } ] }, { "text": "肠毒素有两种,即不耐热毒素(heatliabletoxin,LT)和耐热毒素(heatstabletoxin,ST)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "肠毒素" }, { "start_idx": 8, "end_idx": 12, "type": "mic", "entity": "不耐热毒素" }, { "start_idx": 14, "end_idx": 28, "type": "mic", "entity": "heatliabletoxin" }, { "start_idx": 30, "end_idx": 31, "type": "mic", "entity": "LT" }, { "start_idx": 34, "end_idx": 37, "type": "mic", "entity": "耐热毒素" }, { "start_idx": 39, "end_idx": 53, "type": "mic", "entity": "heatstabletoxin" }, { "start_idx": 55, "end_idx": 56, "type": "mic", "entity": "ST" } ] }, { "text": "LT的理化性质、免疫状态及作用机制与霍乱毒素相同。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "LT" }, { "start_idx": 18, "end_idx": 21, "type": "mic", "entity": "霍乱毒素" } ] }, { "text": "LT与小肠上皮细胞上的受体神经节苷脂结合,激活腺苷酸环化酶,使肠上皮细胞内三磷酸腺苷(ATP)转化为环磷酸腺苷(cAMP)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "LT" }, { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "小肠上皮细胞" }, { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "受体神经节苷脂" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "腺苷酸环化酶" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "肠上皮细胞" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "三磷酸腺苷" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "ATP" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "环磷酸腺苷" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "cAMP" } ] }, { "text": "ST通过激活鸟苷酸环化酶使三磷酸鸟苷(GTP)转化为环磷酸鸟苷(cGMP)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "ST" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "鸟苷酸环化酶" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "三磷酸鸟苷" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "GTP" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "环磷酸鸟苷" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "cGMP" } ] }, { "text": "二者均抑制肠黏膜对钠(同时对氯和水)的吸收,促进氯(同时对钠和水)的分泌。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肠黏膜" } ] }, { "text": "使水向肠腔内渗透,液体积聚于肠道,引起腹泻。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肠腔" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肠道" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "腹泻" } ] }, { "text": "(2)侵袭性肠炎:病原侵入肠黏膜固有层,引起充血、水肿、炎症细胞浸润、渗出性炎症病变、糜烂及溃疡等,造成腹泻。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "侵袭性肠炎" }, { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "病原" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "肠黏膜固有层" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "充血" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "水肿" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "炎症细胞浸润" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "渗出性炎症病变" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "糜烂" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "溃疡" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "腹泻" } ] }, { "text": "由各种侵袭性细菌所致,如志贺菌、沙门菌、EIEC、空肠弯曲菌、耶尔森菌和金黄色葡萄球菌等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "mic", "entity": "侵袭性细菌" }, { "start_idx": 12, "end_idx": 14, "type": "mic", "entity": "志贺菌" }, { "start_idx": 16, "end_idx": 18, "type": "mic", "entity": "沙门菌" }, { "start_idx": 20, "end_idx": 23, "type": "mic", "entity": "EIEC" }, { "start_idx": 25, "end_idx": 29, "type": "mic", "entity": "空肠弯曲菌" }, { "start_idx": 31, "end_idx": 34, "type": "mic", "entity": "耶尔森菌" }, { "start_idx": 36, "end_idx": 42, "type": "mic", "entity": "金黄色葡萄球菌" } ] }, { "text": "志贺菌、EIEC和金黄色葡萄球菌主要侵犯结肠;空肠弯曲菌主要病变在空肠和回肠,也可累及结肠;耶尔森菌多累及回肠;鼠伤寒沙门菌主要累及回肠和结肠。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "志贺菌" }, { "start_idx": 4, "end_idx": 7, "type": "mic", "entity": "EIEC" }, { "start_idx": 9, "end_idx": 15, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 23, "end_idx": 27, "type": "mic", "entity": "空肠弯曲菌" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "回肠" }, { "start_idx": 46, "end_idx": 49, "type": "mic", "entity": "耶尔森菌" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "回肠" }, { "start_idx": 56, "end_idx": 61, "type": "mic", "entity": "鼠伤寒沙门菌" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "结肠" } ] }, { "text": "这类病原菌均能引起痢疾样症状,粪便水分不多有脓血黏液痉挛样腹痛;同时具肠毒素作用和侵袭作用的菌株则可引起毒素性水样腹泻和痢疾样症状。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "病原菌" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "粪便" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "粪便水分不多" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "脓血黏液" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "有脓血黏液" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "痉挛样腹痛" }, { "start_idx": 35, "end_idx": 37, "type": "mic", "entity": "肠毒素" }, { "start_idx": 46, "end_idx": 47, "type": "mic", "entity": "菌株" }, { "start_idx": 52, "end_idx": 58, "type": "dis", "entity": "毒素性水样腹泻" } ] }, { "text": "2.病毒性肠炎(viralenteritis)目前对轮状病毒(rotavirus)研究较多。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "病毒性肠炎" }, { "start_idx": 8, "end_idx": 21, "type": "dis", "entity": "viralenteritis" }, { "start_idx": 26, "end_idx": 29, "type": "mic", "entity": "轮状病毒" }, { "start_idx": 31, "end_idx": 39, "type": "mic", "entity": "rotavirus" } ] }, { "text": "轮状病毒侵犯小肠上部,严重者累及整个小肠。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "轮状病毒" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "小肠" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "小肠" } ] }, { "text": "在小肠绒毛顶端的柱状上皮细胞内复制,细胞变性,微绒毛肿胀、不规则,从而使受累的小肠黏膜上皮细胞很快脱落。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "小肠绒毛顶端" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "柱状上皮细胞" }, { "start_idx": 39, "end_idx": 46, "type": "bod", "entity": "小肠黏膜上皮细胞" } ] }, { "text": "小肠隐窝部立方上皮细胞(分泌细胞)不受损害,增殖上移修复受损的黏膜上皮,但新生的上皮细胞不够成熟,其酶活性和转运功能较差。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "bod", "entity": "小肠隐窝部立方上皮细胞" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "分泌细胞" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "黏膜上皮" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "酶" } ] }, { "text": "由于肠黏膜上皮细胞脱落,造成吸收面积减少,使水和电解质吸收减少,而且绒毛裸露,造成水、电解质回渗,导致腹泻;微绒毛上双糖酶,尤其是乳糖酶活性降低,造成双糖如(乳糖)吸收障碍,不能分解的营养物质在肠腔内滞留,被肠道细菌分解,增加肠内渗透压,使水进入肠腔,导致腹泻加重。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "肠黏膜上皮细胞" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "绒毛" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "腹泻" }, { "start_idx": 54, "end_idx": 60, "type": "bod", "entity": "微绒毛上双糖酶" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "乳糖酶" }, { "start_idx": 75, "end_idx": 85, "type": "dis", "entity": "双糖如(乳糖)吸收障碍" }, { "start_idx": 97, "end_idx": 98, "type": "bod", "entity": "肠腔" }, { "start_idx": 104, "end_idx": 107, "type": "mic", "entity": "肠道细菌" }, { "start_idx": 113, "end_idx": 117, "type": "ite", "entity": "肠内渗透压" }, { "start_idx": 123, "end_idx": 124, "type": "bod", "entity": "肠腔" }, { "start_idx": 128, "end_idx": 129, "type": "dis", "entity": "腹泻" } ] }, { "text": "晚近国外研究发现,轮状病毒上的非结构蛋白NSP4引起类似于细菌毒素的作用,导致分泌性腹泻。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "轮状病毒" }, { "start_idx": 15, "end_idx": 23, "type": "mic", "entity": "非结构蛋白NSP4" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "分泌性腹泻" } ] }, { "text": "(三)脂肪、蛋白质和糖代谢紊乱由于肠道消化吸收功能减低,肠蠕动亢进,使营养素的消化和吸收发生障碍。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "脂肪、蛋白质和糖代谢紊乱" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肠道" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肠" } ] }, { "text": "营养物质的丢失主要是酶功能紊乱引起同化功能障碍所致。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "酶功能紊乱" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "同化功能障碍" } ] }, { "text": "碳水化合物的吸收也受到影响,糖耐量试验曲线低。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "糖耐量试验" } ] }, { "text": "但在急性腹泻,患儿胃肠的消化吸收功能未完全丧失,对营养素的吸收可达正常的60%~90%。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "急性腹泻" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胃肠" } ] }, { "text": "【临床表现】(一)消化道症状腹泻时大便次数增多,量增加,性质改变,大便每日3次以上,甚至10~20次/日可呈稀便、糊状便、水样便,或是黏液脓血便腹泻时粪便的形状比次数更重要。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "消化道" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "腹泻" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "大便" }, { "start_idx": 17, "end_idx": 31, "type": "sym", "entity": "大便次数增多,量增加,性质改变" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "大便" }, { "start_idx": 33, "end_idx": 51, "type": "sym", "entity": "大便每日3次以上,甚至10~20次/日" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "稀便" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "糊状便" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "水样便" }, { "start_idx": 67, "end_idx": 71, "type": "bod", "entity": "黏液脓血便" }, { "start_idx": 52, "end_idx": 71, "type": "sym", "entity": "可呈稀便、糊状便、水样便,或是黏液脓血便" }, { "start_idx": 72, "end_idx": 73, "type": "dis", "entity": "腹泻" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "粪便" } ] }, { "text": "如果便次增多而大便成形,不是腹泻。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "便次增多" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "大便" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "大便成形" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "腹泻" } ] }, { "text": "人乳喂养儿每天排便2~4次呈糊状,也不是腹泻。", "entities": [ { "start_idx": 5, "end_idx": 15, "type": "sym", "entity": "每天排便2~4次呈糊状" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "腹泻" } ] }, { "text": "恶心、呕吐是常见的伴发症状,严重者呕吐咖啡样物。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "恶心" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "呕吐" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "咖啡样物" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "呕吐咖啡样物" } ] }, { "text": "腹痛、腹胀及食欲缺乏等症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "腹痛" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "腹胀" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "食欲缺乏" } ] }, { "text": "(二)全身症状病情严重者全身症状明显,大多数有发热,体温38~40℃,少数高达40℃以上,可出现面色苍白烦躁不安、精神萎靡、嗜睡、惊厥甚至昏迷等表现。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "发热" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "体温" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "体温38~40℃" }, { "start_idx": 35, "end_idx": 43, "type": "sym", "entity": "少数高达40℃以上" }, { "start_idx": 48, "end_idx": 49, "type": "ite", "entity": "面色" }, { "start_idx": 45, "end_idx": 51, "type": "sym", "entity": "可出现面色苍白" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "精神萎靡" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "嗜睡" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "惊厥" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "昏迷" } ] }, { "text": "随着全身症状加重,可引起神经系统、心、肝及肾功能失调。", "entities": [ { "start_idx": 12, "end_idx": 25, "type": "dis", "entity": "神经系统、心、肝及肾功能失调" } ] }, { "text": "(三)水、电解质及酸碱平衡紊乱酸碱平衡紊乱主要为脱水及代谢性酸中毒,有时还有低钾血症和低钙血症。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "酸碱平衡紊乱" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "脱水" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "低钾血症" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "低钙血症" } ] }, { "text": "1.脱水由于腹泻与呕吐丢失大量的水和电解质,使体内保留水分的能力减低;严重呕吐、禁食、食欲减低或拒食,使食物和液体摄入量均减少;患儿发热、呼吸加快、酸中毒者呼吸加深,使不显性失水增加。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "脱水" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "腹泻" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "呕吐" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "水" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "电解质" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "严重呕吐" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "禁食" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "食欲减低" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "拒食" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "发热" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "呼吸加快" }, { "start_idx": 74, "end_idx": 76, "type": "dis", "entity": "酸中毒" }, { "start_idx": 78, "end_idx": 81, "type": "sym", "entity": "呼吸加深" }, { "start_idx": 84, "end_idx": 90, "type": "sym", "entity": "不显性失水增加" } ] }, { "text": "根据水、电解质损失的量及性质不同分为三种类型:等渗性脱水(血清钠浓度130~150mmol/L)、低渗性脱水(血清钠浓度<130mmol/L)及高渗性脱水(血清钠浓度>150mmol/L)。", "entities": [ { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "等渗性脱水" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "血清钠" }, { "start_idx": 55, "end_idx": 57, "type": "ite", "entity": "血清钠" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "高渗性脱水" }, { "start_idx": 78, "end_idx": 80, "type": "ite", "entity": "血清钠" } ] }, { "text": "大多数急性腹泻患儿为等渗性脱水。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "急性腹泻" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "等渗性脱水" } ] }, { "text": "一般表现为体重减轻,口渴不安,皮肤苍白前囟和眼眶凹陷黏膜干燥眼泪减少尿量减少。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "体重减轻" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "口渴不安" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "皮肤" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "皮肤苍白" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "前囟" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "眼眶" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "眼眶凹陷" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "黏膜" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "黏膜干燥" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "眼泪" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "眼泪减少" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "尿量减少" } ] }, { "text": "严重者可导致循环障碍。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "循环障碍" } ] }, { "text": "按脱水分为轻度、中度及重度。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "dis", "entity": "脱" }, { "start_idx": 1, "end_idx": 1, "type": "pro", "entity": "脱" } ] }, { "text": "脱水的评估(表13-13)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "脱水" } ] }, { "text": "表13-5脱水及液体丢失量的估计2.代谢性酸中毒脱水大多有不同程度的代谢性酸中毒,产生原因为:大量的碱性物质随粪便丢失;脱水时肾血流量不足,尿量减少,体内酸性代谢产物不能及时排出;肠道消化和吸收功能不良、摄入热量不足,脂肪氧化增加,代谢不全,致酮体堆积且不能及时被肾脏排出;严重脱水者组织灌容不足,组织缺氧,乳酸堆积。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "脱水" }, { "start_idx": 8, "end_idx": 12, "type": "ite", "entity": "液体丢失量" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "脱水" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "碱性物质" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "粪便" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "脱水" }, { "start_idx": 63, "end_idx": 63, "type": "bod", "entity": "肾" }, { "start_idx": 70, "end_idx": 71, "type": "ite", "entity": "尿量" }, { "start_idx": 75, "end_idx": 82, "type": "bod", "entity": "体内酸性代谢产物" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "肠道" }, { "start_idx": 122, "end_idx": 123, "type": "bod", "entity": "酮体" }, { "start_idx": 132, "end_idx": 133, "type": "bod", "entity": "肾脏" }, { "start_idx": 137, "end_idx": 140, "type": "dis", "entity": "严重脱水" }, { "start_idx": 154, "end_idx": 155, "type": "bod", "entity": "乳酸" } ] }, { "text": "主要表现为精神萎靡、嗜睡、呼吸深长呈叹息状,口唇樱红意识不清。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "精神萎靡" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "嗜睡" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "呼吸深长呈叹息状" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "口唇" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "口唇樱红" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "意识不清" } ] }, { "text": "新生儿及小婴儿呼吸代偿功能差,呼吸节律改变不明显,主要表现为嗜睡、面色苍白拒食及衰弱等,应注意早期发现。", "entities": [ { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "嗜睡" }, { "start_idx": 33, "end_idx": 34, "type": "ite", "entity": "面色" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "面色苍白" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "拒食" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "衰弱" } ] }, { "text": "3.低钾血症腹泻时水样便中钾浓度约在20~50mmol/L。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "低钾血症" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "腹泻" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "水样便" }, { "start_idx": 13, "end_idx": 13, "type": "ite", "entity": "钾" } ] }, { "text": "吐泻丢失过多以及摄入不足、钾不能补偿等可导致低血钾发生。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "低血钾" } ] }, { "text": "其症状多在脱水与酸中毒纠正,尿量增多酸中毒时细胞外液H+的进入细胞内,与K+交换,故细胞内K+下降,而血清钾不降低。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "脱水" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "酸中毒" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "尿量" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "尿量增多" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "酸中毒" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "细胞外液H+" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "K+" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "细胞内K+" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "血清钾" } ] }, { "text": "脱水时肾功能低下,钾由尿液排出减少。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "脱水" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "钾" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "尿液" } ] }, { "text": "在补液后,尤其是输入不含钾的溶液,血清钾被稀释并随尿排出增多,酸中毒纠正后钾又从细胞外转至细胞内,此时易出现低钾血症。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dru", "entity": "不含钾的溶液" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "血清钾" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "酸中毒" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "钾" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "细胞" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "细胞" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "低钾血症" } ] }, { "text": "病程在1周以上时逐渐出现低钾血症。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "低钾血症" } ] }, { "text": "在脱水未纠正前,因血液浓缩、酸中毒及尿少等原因,血钾浓度尚可维持正常,此时很少出现低钾血症。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "脱水" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "血液" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "酸中毒" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "血钾" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "低钾血症" } ] }, { "text": "而随着脱水和酸中毒逐步纠正和尿量的增多,再加补给钾含量不足从而逐渐出现。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "脱水" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "酸中毒" } ] }, { "text": "血清钾低于3.5mmol/L以下,表现为精神萎靡,肌张力减低腹胀临床表现心音低钝射减弱或消失。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "血清钾" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "心音低钝" } ] }, { "text": "严重者昏迷,肠麻痹呼吸肌麻痹心率减慢心尖部收缩期杂音,可危及生命。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "昏迷" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肠" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "肠麻痹" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "呼吸肌麻痹" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "心率" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "心率减慢" }, { "start_idx": 18, "end_idx": 25, "type": "sym", "entity": "心尖部收缩期杂音" } ] }, { "text": "4.低钙血症和低镁血症一般不会出现。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "低钙血症" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "低镁血症" } ] }, { "text": "腹泻持久,原有佝偻病或营养不良患儿,当酸中毒纠正后,血清结合钙增多,离子钙减少,可出现低血钙症状低镁血症一般在低钠、低钾及低钙纠正后出现。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "腹泻" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "佝偻病" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "营养不良" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "酸中毒" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "血清结合钙" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "离子钙" }, { "start_idx": 44, "end_idx": 45, "type": "ite", "entity": "血钙" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "低血钙症状" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "低镁血症" } ] }, { "text": "表现烦躁,手足搐搦惊厥。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "烦躁" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "手足" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "手足搐搦" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "惊厥" } ] }, { "text": "(四)几种常见感染性腹泻的临床表现特点1.轮状病毒性肠炎(rotavirusenteritis)好发于秋冬季,呈散发或小流行,病毒通过粪-口途径以及呼吸道传播。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "感染性腹泻" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "轮状病毒性肠炎" }, { "start_idx": 29, "end_idx": 46, "type": "dis", "entity": "rotavirusenteritis" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "粪-口" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "呼吸道" } ] }, { "text": "潜伏期1~3天,常伴发热和上呼吸道感染症状,腹泻,大便呈水样或蛋花汤样,无腥臭,每日数次至十余次。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "发热" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "上呼吸道感染症状" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "腹泻" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "大便" }, { "start_idx": 25, "end_idx": 34, "type": "sym", "entity": "大便呈水样或蛋花汤样" }, { "start_idx": 36, "end_idx": 38, "type": "sym", "entity": "无腥臭" }, { "start_idx": 40, "end_idx": 47, "type": "sym", "entity": "每日数次至十余次" } ] }, { "text": "病程1~3天内大量病毒从大便排出。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "大便" }, { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "大量病毒从大便排出" } ] }, { "text": "血清抗体一般3周后上升病毒较难分离,免疫电镜、ELISA或核酸电泳等均有助于诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "血清抗体" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "血清抗体一般3周后上升" }, { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "病毒" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "免疫电镜" }, { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "ELISA" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "核酸电泳" } ] }, { "text": "2.诺如病毒肠炎多见于较大儿童及成年人,临床表现与轮状病毒肠炎相似。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "诺如病毒肠炎" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "轮状病毒肠炎" } ] }, { "text": "3.大肠杆菌肠炎(escherichiacolienteritis)常发生于5~8月份,病情轻重不一。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "大肠杆菌肠炎" }, { "start_idx": 9, "end_idx": 32, "type": "dis", "entity": "escherichiacolienteritis" } ] }, { "text": "致病性大肠杆菌肠炎大便呈蛋花汤样腥臭,有较多的黏液偶见血丝或黏胨便。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "致病性大肠杆菌肠炎" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "大便" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "大便呈蛋花汤样" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "腥臭" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "黏液" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "有较多的黏液" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "便" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "偶见血丝或黏胨便" } ] }, { "text": "产毒素性大肠杆菌肠炎,起病较急,主要症状为呕吐、腹泻,大便呈水样无白细胞常发生明显的水、电解质和酸碱平衡紊乱侵袭性大肠杆菌肠炎,起病急,高热,腹泻频繁,大便呈黏胨状,带脓血,常伴恶心、腹痛及里急后重等症状,有时可出现严重中毒症状,甚至休克。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "产毒素性大肠杆菌肠炎" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "呕吐" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "腹泻" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "大便" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "大便呈水样" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "白细胞" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "无白细胞" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "水" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "电解质" }, { "start_idx": 36, "end_idx": 53, "type": "sym", "entity": "常发生明显的水、电解质和酸碱平衡紊乱" }, { "start_idx": 54, "end_idx": 62, "type": "dis", "entity": "侵袭性大肠杆菌肠炎" }, { "start_idx": 64, "end_idx": 66, "type": "sym", "entity": "起病急" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "高热" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "腹泻频繁" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "大便" }, { "start_idx": 76, "end_idx": 81, "type": "sym", "entity": "大便呈黏胨状" }, { "start_idx": 83, "end_idx": 85, "type": "sym", "entity": "带脓血" }, { "start_idx": 89, "end_idx": 90, "type": "sym", "entity": "恶心" }, { "start_idx": 92, "end_idx": 93, "type": "sym", "entity": "腹痛" }, { "start_idx": 95, "end_idx": 98, "type": "sym", "entity": "里急后重" }, { "start_idx": 110, "end_idx": 111, "type": "dis", "entity": "中毒" }, { "start_idx": 108, "end_idx": 113, "type": "sym", "entity": "严重中毒症状" }, { "start_idx": 117, "end_idx": 118, "type": "sym", "entity": "休克" } ] }, { "text": "出血性大肠杆菌肠炎,大便次数增多开始为黄色水样便后转为血水便有特殊臭味,大便镜检有大量红细胞,常无白细胞。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "出血性大肠杆菌肠炎" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "大便" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "大便次数增多" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "水样便" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "开始为黄色水样便" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "血水便" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "后转为血水便" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "有特殊臭味" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "大便镜检" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "红细胞" }, { "start_idx": 49, "end_idx": 51, "type": "ite", "entity": "白细胞" } ] }, { "text": "4.空肠弯曲菌肠炎(campylobacterjejunienteritis)全年均可发病,多见于夏季,可散发或暴发流行。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "空肠弯曲菌肠炎" }, { "start_idx": 10, "end_idx": 37, "type": "dis", "entity": "campylobacterjejunienteritis" } ] }, { "text": "发热、呕吐、腹痛、腹泻、大便呈黏液或脓血便,产毒菌株感染可引起水样便,大便镜检有大量白细胞及数量不等的红细胞,可并发严重的小肠结肠炎、败血症、肺炎、脑膜炎、心内膜炎及心包炎等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "发热" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "呕吐" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "腹痛" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "腹泻" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "大便" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "大便呈黏液或脓血便" }, { "start_idx": 22, "end_idx": 25, "type": "mic", "entity": "产毒菌株" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "水样便" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "大便镜检" }, { "start_idx": 42, "end_idx": 44, "type": "ite", "entity": "白细胞" }, { "start_idx": 51, "end_idx": 53, "type": "ite", "entity": "红细胞" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "小肠结肠炎" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "败血症" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "肺炎" }, { "start_idx": 74, "end_idx": 76, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 78, "end_idx": 81, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 83, "end_idx": 85, "type": "dis", "entity": "心包炎" } ] }, { "text": "5.耶尔森菌小肠结肠炎多发生于冬春季节,以婴幼儿多见。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "耶尔森菌小肠结肠炎" } ] }, { "text": "5岁以下患儿以腹泻为主要症状,粪便为水样、黏液样、脓样或带血大便镜检有大量白细胞,多伴腹痛、发热、恶心和呕吐。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "腹泻" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "粪便" }, { "start_idx": 15, "end_idx": 29, "type": "sym", "entity": "粪便为水样、黏液样、脓样或带血" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "大便镜检" }, { "start_idx": 37, "end_idx": 39, "type": "ite", "entity": "白细胞" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "腹痛" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "发热" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "恶心" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "呕吐" } ] }, { "text": "5岁以上及青少年,以下腹痛、血白细胞增高,血沉加快为主要表现,酷似急性阑尾炎。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "腹痛" }, { "start_idx": 14, "end_idx": 17, "type": "ite", "entity": "血白细胞" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "血白细胞增高" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "血沉加快" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "急性阑尾炎" } ] }, { "text": "6.鼠伤寒沙门菌肠炎全年发病,以4~9月份发病率最高。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "鼠伤寒沙门菌肠炎" } ] }, { "text": "经口传播。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "口" } ] }, { "text": "主要临床表现为发热、恶心、呕吐、腹痛、腹胀及“喷射”样腹泻,大便次数可达30次以上,呈黄色或墨绿色稀便、水样便、黏液便或脓血便。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "发热" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "恶心" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "呕吐" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "腹痛" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "“喷射”样腹泻" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "大便" }, { "start_idx": 30, "end_idx": 40, "type": "sym", "entity": "大便次数可达30次以上" }, { "start_idx": 42, "end_idx": 50, "type": "sym", "entity": "呈黄色或墨绿色稀便" }, { "start_idx": 52, "end_idx": 54, "type": "sym", "entity": "水样便" }, { "start_idx": 56, "end_idx": 58, "type": "sym", "entity": "黏液便" }, { "start_idx": 60, "end_idx": 62, "type": "sym", "entity": "脓血便" } ] }, { "text": "大便镜检可见大量白细胞及不同数量的红细胞,严重者可出现脱水、酸中毒及全身中毒症状休克,也可引起败血症及脑脊髓膜炎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "大便镜检" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "白细胞" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "红细胞" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "脱水" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "酸中毒" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "全身中毒" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "全身中毒症状" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "休克" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "败血症" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "脑脊髓膜炎" } ] }, { "text": "7.金黄色葡萄球菌肠炎很少为原发性,多继发于应用大量广谱抗生素后或继发于慢性疾病基础上。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "金黄色葡萄球菌肠炎" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "广谱抗生素" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "慢性疾病" } ] }, { "text": "起病急,中毒症状重发热、呕吐及频繁腹泻。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "起病急" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "中毒" }, { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "中毒症状重" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "发热" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "呕吐" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "频繁腹泻" } ] }, { "text": "不同程度脱水、电解质紊乱,严重者发生休克。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "脱水" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "电解质紊乱" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "休克" } ] }, { "text": "病初大便为黄绿色,3~4日后多转变为腥臭,海水样便,黏液多大便镜检有大量脓细胞及革兰阳性菌。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "大便" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "病初大便为黄绿色" }, { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "3~4日后多转变为腥臭" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "海水样便" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "黏液" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "黏液多" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "大便镜检" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "脓细胞" }, { "start_idx": 40, "end_idx": 44, "type": "mic", "entity": "革兰阳性菌" } ] }, { "text": "大便培养有葡萄球菌生长,凝固酶阳性伪膜性肠炎多见长期使用抗生素后,由于长期使用抗生素导致肠道菌群紊乱,使难辨梭状芽胞杆菌大量繁殖,产生坏死毒素所致。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "大便培养" }, { "start_idx": 5, "end_idx": 8, "type": "mic", "entity": "葡萄球菌" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "凝固酶" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "凝固酶阳性" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "伪膜性肠炎" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "抗生素" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "抗生素" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "肠道菌群紊乱" }, { "start_idx": 54, "end_idx": 59, "type": "mic", "entity": "梭状芽胞杆菌" }, { "start_idx": 67, "end_idx": 70, "type": "mic", "entity": "坏死毒素" } ] }, { "text": "主要症状为腹泻,大便呈黄稀水样或黏液便少数带血,有伪膜排出(肠管状)伴有发热、腹胀及腹痛。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "腹泻" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "大便" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "大便呈黄稀水样或黏液便" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "少数带血" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "伪膜" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "肠管状" }, { "start_idx": 24, "end_idx": 33, "type": "sym", "entity": "有伪膜排出(肠管状)" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "伴有发热" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "腹胀" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "腹痛" } ] }, { "text": "腹痛常先于腹泻或与腹泻同时出现。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "腹痛" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "腹泻" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "腹泻" } ] }, { "text": "轻型患儿一般于停药后5~8天腹泻停止,严重者发生脱水、休克甚至死亡。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "腹泻停止" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "脱水" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "休克" } ] }, { "text": "如果患儿腹泻发生于停药后或腹泻出现后持续用抗生素,则病程常迁延。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "腹泻" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "腹泻" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "抗生素" } ] }, { "text": "9.白色念珠菌肠炎(candidaalbicansenteritis)多发生于体弱及营养不良小儿,长期滥用广谱抗生素或肾上腺皮质肾上腺皮质激素腔内常伴有鹅口疮。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "白色念珠菌肠炎" }, { "start_idx": 10, "end_idx": 33, "type": "dis", "entity": "candidaalbicansenteritis" }, { "start_idx": 64, "end_idx": 70, "type": "dru", "entity": "肾上腺皮质激素" } ] }, { "text": "大便次数大便次数增多便或发绿,泡沫较多泡沫较多液,有时可见有时可见豆腐渣样细块(菌落)在镜下可见真菌孢子和假菌丝,做粪便真菌粪便真菌培养别。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "大便次数增多" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "泡沫较多" }, { "start_idx": 29, "end_idx": 42, "type": "sym", "entity": "有时可见豆腐渣样细块(菌落)" }, { "start_idx": 62, "end_idx": 67, "type": "pro", "entity": "粪便真菌培养" } ] }, { "text": "(五)小儿迁延性和慢性腹泻病因复杂,目前认为包括感染、过感染、先天性消化先天性消化酶缺陷缺陷、药物因素及先天畸形先天畸形中以感染后腹泻最为腹泻。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "感染" }, { "start_idx": 36, "end_idx": 43, "type": "dis", "entity": "先天性消化酶缺陷" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "先天畸形" }, { "start_idx": 69, "end_idx": 70, "type": "dis", "entity": "腹泻" } ] }, { "text": "对慢性腹泻患儿肠黏膜活肠黏膜活体组织检查,小肠黏膜结构和功能持续损害或正常修复机制受损是小儿腹泻小儿腹泻愈的重要原因。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "肠黏膜活体组织检查" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "小儿腹泻" } ] }, { "text": "但如宿主不能产生正常免疫反应,反复接触感染病原,或因感染严重损伤肠黏液,肠黏液性腹泻可转为慢性腹泻慢性腹泻因黏膜持续损伤致腹泻迁延腹泻愈,少数为感染原持续作用。", "entities": [ { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "肠黏液" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "慢性腹泻" }, { "start_idx": 65, "end_idx": 66, "type": "dis", "entity": "腹泻" } ] }, { "text": "十二指肠、空肠黏膜变薄,肠绒毛萎缩、溢出、脱落增加,微绒毛变性上皮细胞更新加速,这可能与肠黏膜表面肠黏膜物的黏附有关。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "十二指肠、空肠黏膜" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "十二指肠、空肠黏膜变薄" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肠绒毛" }, { "start_idx": 12, "end_idx": 24, "type": "sym", "entity": "肠绒毛萎缩、溢出、脱落增加" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "微绒毛" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "微绒毛变性" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "肠黏膜" } ] }, { "text": "由于黏膜再生黏膜不足,这些新生的上皮细胞类似于隐窝细胞隐窝细胞能低下。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "黏膜" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "隐窝细胞" } ] }, { "text": "双糖酶尤其是乳糖酶活性乳糖酶刷状缘肽酶活性降低,加上有效吸收面积的减少,引起各种营养物质的消化吸收不良。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "乳糖酶" } ] }, { "text": "另外,肠黏膜损肠黏膜损伤对病原因子和大分子物质的通透性,使黏膜对外来抗原致敏。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "肠黏膜损伤" } ] }, { "text": "②营养不良营养不良腹泻时小肠上段小肠上段所有细菌都显著增多肠内厌氧菌和酵母菌过度繁殖。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "营养不良" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "小肠上段" }, { "start_idx": 16, "end_idx": 28, "type": "sym", "entity": "小肠上段所有细菌都显著增多" } ] }, { "text": "由于大量细菌对胆细菌的脱结合作用,使游离胆酸增高。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "mic", "entity": "细菌" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "游离胆酸" } ] }, { "text": "高浓度游离胆酸有损害小肠细胞,还阻碍脂肪微粒的形成。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "小肠细胞" } ] }, { "text": "严重营养不良免疫功能缺陷,分泌型抗体分泌型抗体胞功能和补体水平补体,因而增加了对病原及食物蛋白抗原的易感性。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "严重营养不良" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "分泌型抗体" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "补体" } ] }, { "text": "总之,持续腹泻易发生营养不良,而营养不良又易使腹泻迁延腹泻愈,两者互为因果,形成恶性循环。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "营养不良" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "腹泻" } ] }, { "text": "【实验室检查】(一)粪便常规检查大便显微镜检查有无脓细胞、白细胞、与吞噬细胞,注意有无虫卵、寄生虫、和菌丝。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "粪便" }, { "start_idx": 16, "end_idx": 22, "type": "pro", "entity": "大便显微镜检查" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "白细胞" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "吞噬细胞" }, { "start_idx": 43, "end_idx": 44, "type": "mic", "entity": "虫卵" }, { "start_idx": 46, "end_idx": 48, "type": "mic", "entity": "寄生虫" } ] }, { "text": "有菌丝需反复几次才有意义,有助于腹泻病的病因和病原学诊断。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "mic", "entity": "菌丝" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "腹泻病" } ] }, { "text": "(二)大便培养大便培养腹泻病原有重要意义。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "大便培养" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "大便培养" } ] }, { "text": "1次粪便培养较低,需多做几次,新鲜标本立即培养可提高阳性检出率。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "粪便培养" } ] }, { "text": "(三)大便乳胶凝集试验对某些病毒性肠炎价值,如轮状病毒及肠道腺病毒较好的敏感性和特异性,对空肠弯曲菌肠炎的诊断有帮助。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "大便乳胶凝集试验" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "病毒性肠炎" }, { "start_idx": 23, "end_idx": 26, "type": "mic", "entity": "轮状病毒" }, { "start_idx": 28, "end_idx": 32, "type": "mic", "entity": "肠道腺病毒" }, { "start_idx": 45, "end_idx": 51, "type": "dis", "entity": "空肠弯曲菌肠炎" } ] }, { "text": "(四)酶联免疫吸附试验病毒有高度敏感性和特异性。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "酶联免疫吸附试验" } ] }, { "text": "有助于轮状病毒肠炎诊断。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "轮状病毒肠炎" } ] }, { "text": "(五)聚丙烯酰凝胶GE)电泳试验检测出轮状病毒亚群及不同电泳型,有助于轮状病毒分类研究。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "equ", "entity": "聚丙烯酰凝胶" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "电泳试验" }, { "start_idx": 19, "end_idx": 22, "type": "mic", "entity": "轮状病毒" }, { "start_idx": 35, "end_idx": 38, "type": "mic", "entity": "轮状病毒" } ] }, { "text": "(六)粪便还原糖检查双糖消化吸收不良时,粪便还原呈阳性,pH值<6.0。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "粪便还原糖检查" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "粪便" } ] }, { "text": "还原糖检查可用改良斑氏试剂或nitest试纸比色。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "斑氏试剂" }, { "start_idx": 14, "end_idx": 21, "type": "equ", "entity": "nitest试纸" } ] }, { "text": "继发性双糖酶缺乏发性多见,原发性者以蔗糖-异麦芽糖酶缺乏最常见。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "继发性双糖酶缺乏" } ] }, { "text": "(七)粪便电镜检查病毒性肠炎有诊断价值,如轮状病毒性肠炎等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "粪便电镜检查" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "病毒性肠炎" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "轮状病毒性肠炎" } ] }, { "text": "(八)血白细胞计数病毒性肠炎白细胞总数不增高。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "ite", "entity": "血白细胞计数" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "病毒性肠炎" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "白细胞" } ] }, { "text": "细菌性肠炎白细胞总数增高或不增高,半数以上的患儿有杆状核增高,杆状核>杆状核,有助于细菌感染的诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "细菌性肠炎" }, { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "白细胞" }, { "start_idx": 35, "end_idx": 37, "type": "ite", "entity": "杆状核" } ] }, { "text": "(九)血培养血培养痢疾、大肠杆菌和大肠杆菌等细菌性肠炎细菌性肠炎意义,血液细菌培养阳性者有助于诊断。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "血培养" }, { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "细菌性肠炎" } ] }, { "text": "(十)血生化检查较重的患儿,应及时检查血pH、血pH碳结合力、碳酸氢根、碳酸氢根、血钾、血血钾及血渗透压血渗透压断及治疗均有重要意义。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "血生化检查" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "血pH" }, { "start_idx": 36, "end_idx": 39, "type": "ite", "entity": "碳酸氢根" }, { "start_idx": 45, "end_idx": 46, "type": "ite", "entity": "血钾" }, { "start_idx": 52, "end_idx": 55, "type": "ite", "entity": "血渗透压" } ] }, { "text": "(十一)其他对迁延性和慢性腹泻者,必要时做乳糖、蔗乳糖、蔗糖或葡萄糖耐量试验试验(一种定量非侵入性测定碳水化合物吸收不良的方法,有条件可以应用),也可作纤维结肠镜纤维结肠镜检查诊断】根据发病季节、年龄、大便性状以及排便次数做出初步诊断,对于脱水程度和性质,有无酸中毒以酸中毒、钠等电解质缺乏,进行判断。", "entities": [ { "start_idx": 25, "end_idx": 37, "type": "pro", "entity": "乳糖、蔗糖或葡萄糖耐量试验" }, { "start_idx": 81, "end_idx": 87, "type": "pro", "entity": "纤维结肠镜检查" }, { "start_idx": 134, "end_idx": 136, "type": "dis", "entity": "酸中毒" } ] }, { "text": "必要时进行细菌、病细菌以及寄生虫等寄生虫检查,作为病因诊断。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "细菌" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "寄生虫" } ] }, { "text": "注意与以下疾病相鉴别:(一)生理性腹泻小儿外观小儿外观虚胖后不久大便次数即较多、稀薄,呈金黄色,但不伴呕吐不伴呕吐增加正常。", "entities": [ { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "小儿外观虚胖" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "不伴呕吐" } ] }, { "text": "至添加辅食添加辅食后大便逐渐转为正常二)急性坏死性小肠炎急性坏死性急性坏死性小肠炎变态反应是发病的重要因素。", "entities": [ { "start_idx": 5, "end_idx": 17, "type": "sym", "entity": "添加辅食后大便逐渐转为正常" }, { "start_idx": 33, "end_idx": 40, "type": "dis", "entity": "急性坏死性小肠炎" } ] }, { "text": "本病具有腹泻、腹胀、便血、高热及呕高热五大症状。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "腹胀" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "高热" } ] }, { "text": "大便初为大便初为水样便转暗红色、果酱样或血便,腹胀多较腹胀多较严重期出现休克,甚至昏迷、惊昏迷。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "大便初为水样便" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "腹胀多较严重" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "昏迷" } ] }, { "text": "(三)急性细菌性急性细菌性痢疾痢疾夏季发病率高,患儿多有不洁食物史,潜伏期24~72小时。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "急性细菌性痢疾" } ] }, { "text": "大多数患者起病急,起病急、腹痛、呕腹痛、腹泻、里腹泻,大便多呈大便多呈黏液脓血便次数每天数次至十多次。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "sym", "entity": "起病急" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "腹痛" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "腹泻" }, { "start_idx": 31, "end_idx": 39, "type": "sym", "entity": "大便多呈黏液脓血便" } ] }, { "text": "中毒性菌痢中毒性菌痢高热惊厥、嗜睡或嗜睡,甚至休克等症休克。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "中毒性菌痢" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "嗜睡" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "休克" } ] }, { "text": "【治疗】腹泻病的腹泻疗原则为预防脱水,纠正脱水纠正脱水饮食,合理用合理用药一)急性腹泻的治疗1.脱水的防脱水的防治预防和纠正在腹泻治疗腹泻占极重要的地位,世界卫生组织(WHO)推荐的口服补液盐(ORS)ORS服补液疗法具有有效、简便、价廉、安全等优点,已成为主要的补液途径,是腹泻治疗腹泻一个重要进展。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "腹泻" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "纠正脱水" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "合理用药" }, { "start_idx": 52, "end_idx": 56, "type": "pro", "entity": "脱水的防治" }, { "start_idx": 67, "end_idx": 68, "type": "dis", "entity": "腹泻" }, { "start_idx": 101, "end_idx": 103, "type": "dru", "entity": "ORS" }, { "start_idx": 142, "end_idx": 143, "type": "dis", "entity": "腹泻" } ] }, { "text": "口服补液治疗是基于小肠的N小肠葡萄糖偶联葡萄糖机制。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "小肠" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "葡萄糖" } ] }, { "text": "小肠微绒毛上皮细胞刷状缘上存在Na+和葡萄糖比例适当,有利于Na+和水的吸收。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "ORS" } ] }, { "text": "ORS中含有钾和碳酸氢盐,可补充腹泻时钾的丢失钾和纠正酸中毒。", "entities": [ { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "钾" } ] }, { "text": "(1)预防脱水预防脱水导致体内大量的水与电解水丢失。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "预防脱水" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "水" } ] }, { "text": "因此,患儿一开始腹泻,就腹泻该给口服足够的液体并继续给小儿喂养,尤其是婴幼儿母乳喂养,以防脱水。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "腹泻" } ] }, { "text": "选用以下方法:①ORS:ORS体为2/3张溶液,用于预防脱水时加等量或半量水稀释以降低电解质的张力。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "ORS" } ] }, { "text": "每次腹泻后,腹泻岁以下服50~100ml,2~10岁服100~200ml,大于10岁的能喝多少就给多少。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "腹泻" } ] }, { "text": "②米汤加盐米汤加盐溶液500ml+细盐1.75g或炒米粉25g+细盐1.75g+水500ml,煮2~3分钟。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "米汤加盐溶液" } ] }, { "text": "③糖盐水:白开水纠正脱水+蔗糖10g+细盐1.75g。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "纠正脱水" } ] }, { "text": "(2)纠正脱水:小儿腹泻发生的脱水,大脱水可通过口服补液疗法纠正。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "脱水" } ] }, { "text": "重度脱水重度脱水补液。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "重度脱水" } ] }, { "text": "1)口服补液口服补液于轻度、中度脱水者。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "口服补液" } ] }, { "text": "有严重腹胀严重腹胀、心肾功能不心肾功能其他较重的并发症以及新生儿,均不宜口服补液。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "严重腹胀" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "心肾功能" } ] }, { "text": "分两个阶段,即纠正脱水纠正脱水维持治疗阶段。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "纠正脱水" } ] }, { "text": "纠正脱水纠正脱水S;补充累积损失量,轻度脱水轻度脱水ml/kg,中度脱水50~80ml/kg,少量多次口服,以免呕吐影响疗效,所需液量在4~6小时内服完。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "纠正脱水" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "轻度脱水" } ] }, { "text": "脱水纠正后,ORS以ORS水稀释补充继续丢失量,随丢随补,也可按每次10ml/kg计算。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "ORS" } ] }, { "text": "2)静脉补液静脉补液脱水和新生儿腹泻患儿均宜静脉补液。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "静脉补液" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "新生儿腹泻" } ] }, { "text": "第一天补液:包补液累积损失量、继续损失量和生理需要量。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "补液" } ] }, { "text": "累积损失量根据脱水程度计算,轻度脱水轻度脱水/kg,中度脱水50~100ml/kg,重度脱水重度脱水120ml/kg。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "轻度脱水" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "重度脱水" } ] }, { "text": "溶液电解质和非电解质比例(即溶液种类)根据脱水性质而定,等渗性脱水用1/2~2/3张含钠液含钠液脱水用2/3等张含钠液,含钠液脱水用1/3张含钠液含钠液滴速宜稍快,一般在8~12小时补完,约每小时8~10ml/kg。", "entities": [ { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "含钠液" }, { "start_idx": 60, "end_idx": 62, "type": "dru", "entity": "含钠液" }, { "start_idx": 73, "end_idx": 75, "type": "dru", "entity": "含钠液" } ] }, { "text": "对重度脱水合并周围循环周围循环障碍2∶1等张液20ml/kg,于30~60分钟内静脉推注或快速滴注快速滴注增加血容量,改善循环和肾脏功能。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "周围循环障碍" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "快速滴注" } ] }, { "text": "在扩容后根据脱水性质选用前述不同溶液继续静脉滴注静脉滴注扣除扩容量。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "继续丢失量和生理需要量能口服则口服,对于不能口服、呕吐频繁呕吐频繁者,给予静脉补液静脉补液需要量每日60~80ml/kg,用1/5张含钠液补充,继续损失量是按“失多少补多少”,用1/3~1/2含钠溶液补充钠溶液两者合并,在余12~16小时补完,一般约每小时5ml/kg。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "呕吐频繁" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "静脉补液" }, { "start_idx": 102, "end_idx": 104, "type": "dru", "entity": "钠溶液" } ] }, { "text": "需静脉补液静脉补液生理需要量和继续丢失量两部分液体(计算方法同上所述)一并在24小时均匀补充。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "静脉补液" } ] }, { "text": "3)纠正酸中毒:轻、中度轻、中度酸中毒纠正,因为在输入的溶液中已含有一部分碱性溶液碱性溶液经过输液后循环和肾功能改善,酸中毒随即纠正。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "轻、中度酸中毒" }, { "start_idx": 41, "end_idx": 44, "type": "dru", "entity": "碱性溶液" } ] }, { "text": "严重酸中严重酸中毒后仍表现有酸中毒症状酸中毒,则需要用碱性药物。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "严重酸中毒" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "酸中毒" } ] }, { "text": "常用的碱性药物有碳酸氢钠和碳酸氢钠。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "碳酸氢钠" } ] }, { "text": "在无实验室检查条件时,可按5%碳酸氢钠5碳酸氢钠/kg或11.2乳酸钠3ml/kg,可提高CO2结合力5mmol/L。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "碳酸氢钠" } ] }, { "text": "需要同时扩充血容量者可直接用1.4%碳酸氢钠2碳酸氢钠/kg代替2∶1等张液,兼扩容和加快酸中毒纠正的作用。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "碳酸氢钠" } ] }, { "text": "已测知血气分析者,按以下公式计算:需补碱性液数(mmol)=(60-CO2结合力)×0.3×体重(kg)/2.24=BE×0.3×体重(kg)5%碳酸氢钠(碳酸氢钠)=BE×体重(kg)/2碱性药物先用半量。", "entities": [ { "start_idx": 84, "end_idx": 87, "type": "dru", "entity": "碳酸氢钠" } ] }, { "text": "4)钾的补充:低钾的低钾一般按KCl2~4mmol/(kg•d)或10%KCl3ml/(kg•d),浓度常为0.15%~0.3%,切勿超过0.3%,速度不宜过快,至少在6小时以上补给。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "低钾" } ] }, { "text": "但在重度脱水重度脱水较大量的钾丢失,补液后循环得到改善,血钾被稀血钾酸中毒纠正,钾向细胞钾内转移,所以易造成低血钾。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "重度脱水" }, { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "血钾" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "钾" } ] }, { "text": "低血钾脱水特别是原有营养不良营养不良长,多日不进食的患儿,及时补钾更必要。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "低血钾" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "营养不良" } ] }, { "text": "一般补钾4补钾天,严重缺钾者适当延长补钾时间补钾5)钙和镁的补充:一般患儿无须常规服用钙剂,对合并营养不良营养不良的患儿应早期给钙。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "补钾" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "补钾" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "营养不良" } ] }, { "text": "在输液过程输液如出现抽搐,可给予10%葡萄糖酸钙葡萄糖酸钙0ml,静脉缓注,必要时重复使用。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "输液" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "葡萄糖酸钙" } ] }, { "text": "个别抽搐患儿用钙剂无效钙剂应考虑到低镁血症的可能,经血镁测定血镁测定后可给25%硫酸镁,每次给0.2ml/kg,每天2~3次,深部肌内深部肌内注射消失后停药。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dru", "entity": "钙剂" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "血镁测定" }, { "start_idx": 67, "end_idx": 72, "type": "pro", "entity": "深部肌内注射" } ] }, { "text": "强调腹泻患儿继续喂养,饮食需适应患儿的消化吸收功能,根据个体情况,分别对待,最好参考患儿食欲及腹泻等情腹泻结合平时饮食习惯,采取循序渐进的原则,并适当补充微量元素和维生素。", "entities": [ { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "腹泻" } ] }, { "text": "维生素喂养者应继续母乳喂养,暂停辅食,缩短每次喂乳时间,少量多次喂哺。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "维生素" } ] }, { "text": "轻症腹泻轻症腹泻方牛奶(formulformulamilk大多耐受良好。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "轻症腹泻" }, { "start_idx": 18, "end_idx": 28, "type": "dru", "entity": "formulamilk" } ] }, { "text": "严重腹泻者,消化吸收消化吸收功能障碍双糖酶(尤其乳糖酶尤其乳糖酶受损,乳糖吸收不良,全乳喂养可加重腹泻症状腹泻甚至可引起酸中毒,先以稀释奶、发酵奶、奶谷类混合物及去乳糖配方奶喂哺,每天喂6次,保证足够的热量,逐渐增至全奶。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "消化吸收功能障碍" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "尤其乳糖酶" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "腹泻" } ] }, { "text": "母乳喂养或牛奶喂养者,如大便量、大便量、次数明显增多样稀便,带酸臭味带酸臭味,腹胀,肠腹胀亢进,又引起较严重的脱水和酸脱水毒,停止喂哺后症状减轻,测大便pH大便pH,还原物质>0.5%,考虑急性腹泻急性腹泻继发性乳糖酶缺乏吸收不良,改稀释牛奶、发酵奶或去乳糖配方奶(不含乳糖)喂养,并密切观察,一旦小儿能耐受即应恢复正常饮食。", "entities": [ { "start_idx": 16, "end_idx": 25, "type": "sym", "entity": "大便量、次数明显增多" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "带酸臭味" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "腹胀" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "脱水" }, { "start_idx": 78, "end_idx": 81, "type": "ite", "entity": "大便pH" }, { "start_idx": 99, "end_idx": 110, "type": "dis", "entity": "急性腹泻继发性乳糖酶缺乏" } ] }, { "text": "遇脱水严重脱水严重频繁的患儿,宜暂禁食,先纠正水和电解质紊乱,病情好转后恢复喂养。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "脱水严重" } ] }, { "text": "必要时对重症腹泻重症腹泻不良者采用静脉营养静脉营养停止后,应提供富有热量和营养价值高的饮食,并应超过平时需要量的10%~100%,一般2周内每日加餐一次,以较快地补偿生长发育,赶上正常生长。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "重症腹泻" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "静脉营养" } ] }, { "text": "3.药物治疗药物治疗抗生素治疗:根据感染性腹泻感染性腹泻和部分细菌性腹泻有自愈倾向的特点,WHO提出90%的腹泻不需腹泻抗菌药物治疗,国内专家提出大约70%的腹泻病不腹泻病也不应该用抗生素,抗生素适用于侵袭性细侵袭性细菌感染(约30%)。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "药物治疗" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "感染性腹泻" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "腹泻" }, { "start_idx": 83, "end_idx": 85, "type": "dis", "entity": "腹泻病" }, { "start_idx": 95, "end_idx": 97, "type": "dru", "entity": "抗生素" }, { "start_idx": 105, "end_idx": 111, "type": "dis", "entity": "侵袭性细菌感染" } ] }, { "text": "临床指征为:①血便;②有里急后重有里急后重便镜检白细胞满视野;④大便pH7大便pH7以上袭性细菌性腹泻重症、新生儿、小婴儿和原有严重消耗性疾病者如肝硬化、肝硬化、血液病及血液病等,使用抗生素指征抗生素。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "有里急后重" }, { "start_idx": 37, "end_idx": 43, "type": "sym", "entity": "大便pH7以上" }, { "start_idx": 77, "end_idx": 79, "type": "dis", "entity": "肝硬化" }, { "start_idx": 85, "end_idx": 87, "type": "dis", "entity": "血液病" }, { "start_idx": 97, "end_idx": 99, "type": "dru", "entity": "抗生素" } ] }, { "text": "1)喹诺酮类药:治疗腹泻抗菌药腹泻首选药物。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "腹泻" } ] }, { "text": "常用诺氟沙星(诺氟沙星)和环丙沙星。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "诺氟沙星" } ] }, { "text": "环丙沙星于细菌性痢疾,大肠杆菌、大肠杆菌曲菌、弧菌、耶弧菌菌及气单胞菌等气单胞菌的肠炎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "环丙沙星" }, { "start_idx": 16, "end_idx": 19, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 27, "end_idx": 28, "type": "mic", "entity": "弧菌" }, { "start_idx": 36, "end_idx": 39, "type": "mic", "entity": "气单胞菌" } ] }, { "text": "由于动物试验发现此类药物可致胚胎关节胚胎关节软骨损伤在儿童剂量不宜过大,疗程不宜过长(一般不超过1周)。", "entities": [ { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "胚胎关节软骨损伤" } ] }, { "text": "常规剂量:诺氟沙星每日15~20mg/kg,分2~3次口服;环丙沙星每环丙沙星0~15mg/kg,分2次口服或静脉滴注静脉滴注)小檗碱:用于轻型细菌性轻型细菌性肠炎稳定,不易耐药,不良反应小,与某些药物联合治疗,可提高疗效。", "entities": [ { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "环丙沙星" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 75, "end_idx": 81, "type": "dis", "entity": "轻型细菌性肠炎" } ] }, { "text": "口服)呋喃唑酮:每日5~7mg/kg,分3~4次口服。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "口服" } ] }, { "text": "在口服道可保持高药物浓度,不易产生耐药性。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "口服" } ] }, { "text": "有恶心、头恶心、皮疹、溶皮疹贫血及黄疸等不黄疸反应。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "恶心" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "皮疹" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "黄疸" } ] }, { "text": "4)氨基糖苷类:本类药临床疗效仅次于第三代头孢菌素与头孢菌素星,但对儿童副作用大,主要为肾及耳神肾及耳神经损害素已很少应用。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "头孢菌素" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "肾及耳神经损害" } ] }, { "text": "阿米卡星(阿米卡星那霉素)每日10~15mg/kg,分次肌内注射肌内注射滴注。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "阿米卡星" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "肌内注射" } ] }, { "text": "妥布霉素3妥布霉素mg/kg,分2次静脉滴注或肌内注射肌内注射米星4~16mg/kg,1次或分2次静脉滴注静脉滴注)第三代头孢菌素及氧头孢烯类氧头孢烯类腹泻的病原菌普遍病原菌对本类药敏感,包括治疗最为困难的多重耐药鼠伤寒沙门菌及志贺菌。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "妥布霉素" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "肌内注射" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 71, "end_idx": 75, "type": "dru", "entity": "氧头孢烯类" }, { "start_idx": 84, "end_idx": 86, "type": "mic", "entity": "病原菌" } ] }, { "text": "志贺菌疗效好,副作用少,但价格贵,需注射给药,故不作为临床第一线用药,仅用于重症及难治性患者。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "志贺菌" } ] }, { "text": "常用有头孢噻肟、头孢噻肟肟、头孢三嗪及头孢三嗪孢等。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "头孢噻肟" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "头孢三嗪" } ] }, { "text": "6)复方新诺明复方新诺明~50mg/(kg•d),分2~3次口服。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "复方新诺明" } ] }, { "text": "7)其他类抗其他类抗生素是治疗空肠弯曲菌空肠弯曲菌肠炎药,25~30mg/(kg•d),分4次口服或一次静脉滴注静脉滴注7天。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "其他类抗生素" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "空肠弯曲菌肠炎" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "隐孢子虫肠炎口服大蒜素口服。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "口服" } ] }, { "text": "真菌性采用制霉菌素、制霉菌素或克霉唑。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "制霉菌素" } ] }, { "text": "克霉唑肠炎停用原来抗生素,抗生素甲硝唑、万古霉素及万古霉素口服。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "克霉唑" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "抗生素" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "万古霉素" } ] }, { "text": "(2)肠黏膜保护剂肠黏膜保护剂石是一种天然的铝和镁的硅酸盐,能改善肠黏液的肠黏液量,加强肠黏膜屏障,吸附和固定各种细菌、病细菌及其毒素,有毒素受损肠黏膜修复和再生。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "肠黏膜保护剂" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肠黏液" }, { "start_idx": 61, "end_idx": 62, "type": "mic", "entity": "细菌" }, { "start_idx": 69, "end_idx": 70, "type": "mic", "entity": "毒素" } ] }, { "text": "临床证明其治疗腹泻具止腹泻、收敛和抑病毒作用,能缩短病程。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dru", "entity": "腹泻" } ] }, { "text": "(3)微生态疗法微生态疗法在于恢复肠道正常菌群的生态平衡,起到生物屏障作用,抵御病原菌的定植和侵入,有利于腹泻的恢腹泻。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "微生态疗法" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "腹泻" } ] }, { "text": "常用药:①乳酶生,也称表飞明,表飞明燥乳酸杆菌片剂,每次0.3g,每日3次;②口服嗜酸乳口服胶囊(LactobLactobacillusAcidophilusCapsule灭活的嗜酸乳酸杆菌及其代谢产物,每包含菌50亿,每次50亿~100亿,每日2次;③双歧杆菌活双歧杆菌活菌制剂胶囊含双歧杆菌0.5亿,每次1粒,每日2~3次;④枯草杆菌、枯草杆菌、肠球菌二联活菌多维颗粒维颗粒,为活菌制剂,每袋含粪链球菌1粪链球菌和枯草杆菌0.15亿,每次1袋,每日2~3次;⑤口服双歧杆口服、嗜酸乳杆菌、肠球菌三联活菌胶囊,为双歧杆菌、双歧杆菌、乳酸杆菌和肠球菌三联活菌制剂每次1~2粒,散剂每次1/2~1包,每日2~3次。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "表飞明" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "口服" }, { "start_idx": 55, "end_idx": 85, "type": "dru", "entity": "LactobacillusAcidophilusCapsule" }, { "start_idx": 132, "end_idx": 139, "type": "dru", "entity": "双歧杆菌活菌制剂" }, { "start_idx": 170, "end_idx": 185, "type": "dru", "entity": "枯草杆菌、肠球菌二联活菌多维颗粒" }, { "start_idx": 204, "end_idx": 207, "type": "mic", "entity": "粪链球菌" }, { "start_idx": 237, "end_idx": 238, "type": "pro", "entity": "口服" }, { "start_idx": 262, "end_idx": 280, "type": "dru", "entity": "双歧杆菌、乳酸杆菌和肠球菌三联活菌制剂" } ] }, { "text": "注意喂水和口喂水补液。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "喂水" } ] }, { "text": "防止呕吐后误呕吐入肺内。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "呕吐" } ] }, { "text": "勤换尿布,大便后冲洗臀部,以臀部防上行性尿路感染、尿布疹及尿布疹感染。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "臀部" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "尿布疹" } ] }, { "text": "(二)迁延性和迁延性和慢性腹泻1.预防、治疗脱水治疗脱水水、电解质和酸碱平衡紊乱。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "迁延性和慢性腹泻" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "治疗脱水" } ] }, { "text": "2.营养治疗此类患者多有营养障碍营养障碍黏膜持续损害、营养不良营养不良继发免疫功能低下循环是主要的发病因素。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "营养障碍" }, { "start_idx": 31, "end_idx": 42, "type": "dis", "entity": "营养不良继发免疫功能低下" } ] }, { "text": "饮食的选择,应考虑到患儿的消化功能及经济状况,母乳为合适饮食,或选用价格低廉、可口的乳类食品,具体参照“急性腹泻急性腹泻治疗。", "entities": [ { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "急性腹泻" } ] }, { "text": "要素饮食是慢性腹泻患儿最理想食品,含已消化的简单的氨基酸、葡萄糖和脂肪,仅需少量肠腔内和肠腔黏液消化,在严重小肠严重小肠黏液损害消化酶缺乏的情况下仍可吸收和耐受。", "entities": [ { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "肠腔" }, { "start_idx": 56, "end_idx": 63, "type": "dis", "entity": "严重小肠黏液损害" } ] }, { "text": "每天6~7次,经口摄入或胃管重力胃管重力间歇滴喂泻停止,体重增加,逐步恢复普通饮食。", "entities": [ { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "胃管重力间歇滴喂" } ] }, { "text": "对仅表现乳糖不耐乳糖不耐受乳糖配方奶、豆浆和酸豆浆。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "乳糖不耐受" }, { "start_idx": 23, "end_idx": 24, "type": "dru", "entity": "豆浆" } ] }, { "text": "对严重腹泻严重腹泻素饮食营养治疗后腹泻仍持续、营养状况恶化,需静脉营养。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "严重腹泻" } ] }, { "text": "静脉营养(TPN)TPN分是葡萄糖、脂肪、蛋脂肪、水溶性和水溶性维生素、电解质及电解质素。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "静脉营养" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "TPN" }, { "start_idx": 22, "end_idx": 23, "type": "dru", "entity": "脂肪" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "水溶性" }, { "start_idx": 40, "end_idx": 42, "type": "dru", "entity": "电解质" } ] }, { "text": "中国腹泻病方腹泻推荐配方为每日脂肪乳剂2~3g/kg,复方结晶复方结晶氨基酸.5g/g,葡萄糖12~15mg/kg,液体120~150ml/kg,热量209.2~376.6kJ/kg(70~90kcal/kg)。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "腹泻" }, { "start_idx": 31, "end_idx": 37, "type": "dru", "entity": "复方结晶氨基酸" } ] }, { "text": "葡萄糖是葡萄糖供能物质,浓度8%~12%,输注速度每分钟4~6mg/kg,最大可达12~15mg/kg。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "葡萄糖" } ] }, { "text": "氨基酸是蛋白质基本单位,是静脉营养静脉营养主要来源,小儿氨基酸代谢与成人不同,选用小儿专用氨基酸较氨基酸,目前小儿专用氨基酸配方有国产(18-AA-650)和德国产(16-AA-600),使用时从小量开始,每日0.5g/kg,每日递增0.25~0.5g/kg,至2.5~3.0g/kg。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "静脉营养" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "氨基酸" } ] }, { "text": "氨基酸可氨基酸葡萄糖共同输入。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "氨基酸" } ] }, { "text": "10%脂肪乳剂1脂肪乳剂20ml/kg,第3天起可增至20~40ml/kg,静脉输注>6小时,最好24小时均匀输入。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "脂肪乳剂" } ] }, { "text": "在应用上述营养液同时,还应补充电解质、电解质及微量元素(微量元素3-13)。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "电解质" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "微量元素" } ] }, { "text": "已有TPN专用的维生素和维生素素的剂型,水乐维他加水乐维他氨基酸中,维他利匹特维他利匹特乳剂中。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "维生素" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "水乐维他" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "维他利匹特" } ] }, { "text": "微量元素,微量元素5kg儿童选用安达美(addameaddamelN15kg者选用派达益儿(ped-ped-el表13-7静脉营养时电解质、电解质、微量元素的微量元素需要量长期TPN会导致肠黏液萎缩肠黏液萎缩分泌减少及胆汁黏稠胆汁黏稠长期输注葡萄糖,会影响食欲。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "微量元素" }, { "start_idx": 26, "end_idx": 33, "type": "dru", "entity": "addamelN" }, { "start_idx": 50, "end_idx": 55, "type": "dru", "entity": "ped-el" }, { "start_idx": 70, "end_idx": 72, "type": "dru", "entity": "电解质" }, { "start_idx": 79, "end_idx": 82, "type": "dru", "entity": "微量元素" }, { "start_idx": 99, "end_idx": 103, "type": "sym", "entity": "肠黏液萎缩" }, { "start_idx": 113, "end_idx": 116, "type": "sym", "entity": "胆汁黏稠" } ] }, { "text": "也可采用部分经口喂,部口喂静脉供给营养素和营养素。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "口喂" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "营养素" } ] }, { "text": "3.抗生素要抗生素分慎重,用于分离出特异病原的感染,并根据药敏试验药敏试验临床用药。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "抗生素" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "药敏试验" } ] }, { "text": "4.蒙脱石散具体见“急性腹泻急性腹泻5.微生态疗法具体见“急性腹泻急性腹泻6.中医治疗对慢性腹泻治疗有一定的疗效。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "急性腹泻" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "急性腹泻" } ] }, { "text": "四、机械通气的辅助给药(一)镇静剂常用的有水合氯醛、地西泮和巴比妥类药品。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "机械通气" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "镇静剂" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "水合氯醛" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "地西泮" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "巴比妥类" } ] }, { "text": "地西泮剂量在静脉给药时为0.1mg/kg,苯巴比妥静脉注射1~2mg/kg。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "地西泮" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "静脉给药" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "静脉注射" } ] }, { "text": "一般可以间断4~6小时使用,不良反应主要为呼吸抑制。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "呼吸抑制" } ] }, { "text": "在自主呼吸没有时,不必使用。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "自主呼吸没有" } ] }, { "text": "也有采用吗啡类镇痛药如芬太尼的,剂量为0.5~2mg/kg。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "吗啡类镇痛药" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "芬太尼" } ] }, { "text": "给药过快可以造成呼吸停止,要同时准备气道通气复苏设备。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 18, "end_idx": 25, "type": "equ", "entity": "气道通气复苏设备" } ] }, { "text": "对于比较烦躁的小儿,使用镇静剂是急救病房常规的手段,避免由于刺激和烦躁,造成血压和颅内压的骤然升降,导致血管破裂出血,或气道压过高,造成气胸和气道身体损伤。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "镇静剂" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "血压" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "颅内压" }, { "start_idx": 38, "end_idx": 48, "type": "sym", "entity": "血压和颅内压的骤然升降" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "血管" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "血管破裂出血" }, { "start_idx": 60, "end_idx": 62, "type": "ite", "entity": "气道压" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "气胸" }, { "start_idx": 71, "end_idx": 76, "type": "dis", "entity": "气道身体损伤" } ] }, { "text": "(二)肌松剂常用的有潘可罗宁(pancuroniumbromide)和琥珀胆碱(succinylcholine)。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "潘可罗宁" }, { "start_idx": 15, "end_idx": 32, "type": "dru", "entity": "pancuroniumbromide" }, { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "琥珀胆碱" }, { "start_idx": 40, "end_idx": 54, "type": "dru", "entity": "succinylcholine" } ] }, { "text": "静脉注射前者为0.1mg/kg,后者为1~2mg/kg。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "静脉注射" } ] }, { "text": "以往由于呼吸机没有同步触发功能,在小儿烦躁时,为避免人机对抗造成的气胸、颅内出血、低氧血症等并发症,肌松剂使用很频繁。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "equ", "entity": "呼吸机" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "烦躁" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "气胸" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "颅内出血" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "低氧血症" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "肌松剂" } ] }, { "text": "现在由于有了同步触发装置,特别是智能化的供气气流调节装置调节装置,使肌松剂的使用限制在较低程度。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "equ", "entity": "同步触发装置" }, { "start_idx": 16, "end_idx": 27, "type": "equ", "entity": "智能化的供气气流调节装置" }, { "start_idx": 34, "end_idx": 36, "type": "dru", "entity": "肌松剂" } ] }, { "text": "一般在用SIMV和A/C方式通气不能得到有效通气、或在测定呼吸力学参数时使用,以短时间消除自主呼吸对测定的干扰。", "entities": [ { "start_idx": 27, "end_idx": 34, "type": "ite", "entity": "测定呼吸力学参数" } ] }, { "text": "在使用肌松剂后,喉部肌肉的松弛,可以造成气道插管周围的漏气增加,在临床上要加以关注。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "肌松剂" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "喉部肌肉" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "松弛" }, { "start_idx": 20, "end_idx": 23, "type": "equ", "entity": "气道插管" } ] }, { "text": "绝对不可在没有气道插管、复苏和呼吸机支持的条件下给予肌松剂。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "equ", "entity": "气道插管" }, { "start_idx": 12, "end_idx": 13, "type": "equ", "entity": "复苏" }, { "start_idx": 15, "end_idx": 17, "type": "equ", "entity": "呼吸机" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "肌松剂" } ] }, { "text": "(三)利尿剂肺水肿时常用利尿剂为呋塞米,可以经静脉或气道给予,均可以起利尿和减少肺液潴留的问题,静脉给药剂量为1~2mg/kg,最大为5mg/kg。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "利尿剂" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "肺水肿" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "利尿剂" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "呋塞米" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "静脉或气道给予" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "肺液潴留" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "静脉给药" } ] }, { "text": "气道内给予呋塞米剂量为0.5mg/kg,对呼吸功能不全有迅速排尿和改善肺顺应性的作用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "气道" }, { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "呋塞米" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "呼吸功能不全" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "肺" } ] }, { "text": "第四节伤寒【流行病学】人类是伤寒(typhoid)杆菌的唯一自然传染源,直接或间接地同感染的人群接触是造成感染的原因。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "伤寒" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "伤寒" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "typhoid" } ] }, { "text": "细菌主要通过粪-口途径传播,一些污染水中放养的水生的贝壳类动物和牡蛎也是感染播散的方式。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "细菌" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "粪" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "口" } ] }, { "text": "伤寒带菌期可长达数十年,小儿带菌者少。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "伤寒带菌" } ] }, { "text": "苍蝇不仅可将带菌者的粪便带至食物上,而且本身还可带菌,从粪便中排菌而污染食物。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "粪便" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "粪便" } ] }, { "text": "【发病机制】病菌经口入胃,未被胃酸消灭者进入小肠的淋巴组织,再由淋巴管进入血液,引起第一次菌血症,病菌随血流进入多种脏器的单核-吞噬细胞系统,部分未被吞噬的细菌再次入血,出现第二次菌血症期,并出现临床症状。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "口" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "胃" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "胃酸" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "小肠" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "淋巴组织" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "淋巴管" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "血液" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "菌血症" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "血流" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "脏器" }, { "start_idx": 61, "end_idx": 69, "type": "bod", "entity": "单核-吞噬细胞系统" }, { "start_idx": 78, "end_idx": 79, "type": "mic", "entity": "细菌" }, { "start_idx": 83, "end_idx": 83, "type": "bod", "entity": "血" }, { "start_idx": 90, "end_idx": 92, "type": "dis", "entity": "菌血症" } ] }, { "text": "受累最严重的部位是集合淋巴结、肝、脾、骨髓等,病理表现有肠壁损伤、肝脾肿大、骨髓受到抑制,肝内病菌从肝管向肠腔排出时,通过胆囊,并在此繁殖。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "淋巴结" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肝" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "脾" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "骨髓" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "肠壁损伤" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "肝脾肿大" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "骨髓" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "骨髓受到抑制" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "肝" }, { "start_idx": 47, "end_idx": 48, "type": "mic", "entity": "病菌" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "肝管" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "肠腔" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "胆囊" } ] }, { "text": "当细菌栓塞末梢血管时造成血管炎,皮肤可见玫瑰疹;细菌进入肾脏并可由尿中排出,细菌在生长繁殖过程中,可释放大量内毒素,引起内毒素血症。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "mic", "entity": "细菌" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "末梢血管" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "血管炎" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "皮肤" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "玫瑰疹" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "细菌" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肾脏" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "尿" }, { "start_idx": 38, "end_idx": 39, "type": "mic", "entity": "细菌" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "内毒素血症" } ] }, { "text": "伤寒杆菌表面的Vi荚膜抗原干扰吞噬作用,它是通过防止补体C3在细胞表面的凝集来完成的,细菌在被巨噬细胞吞噬后可在其内生存,这是它的重要毒力物质即循环内毒素,是脂多糖细菌细胞壁成分,是延长发热和导致中毒症状的主要原因。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "伤寒杆菌" }, { "start_idx": 43, "end_idx": 44, "type": "mic", "entity": "细菌" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "巨噬细胞" } ] }, { "text": "内毒素诱导由巨噬细胞产生的细胞因子,可引起全身症状。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "巨噬细胞" } ] }, { "text": "细胞介导的免疫在人类抵抗伤寒病中起主要作用。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "伤寒病" } ] }, { "text": "T细胞数的减少是伤寒患者患病的关键原因,携带者的白细胞移动抑制试验显示对伤寒抗原的细胞反应受损,带菌者可将大量的病菌通过肠道,随粪便排泄,而不进入宿主的上皮细胞。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "T细胞" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "伤寒" }, { "start_idx": 24, "end_idx": 32, "type": "pro", "entity": "白细胞移动抑制试验" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "伤寒" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "粪便" }, { "start_idx": 76, "end_idx": 79, "type": "bod", "entity": "上皮细胞" } ] }, { "text": "年长儿表现与成人相似,起病即伴发热,逐渐升高,在第5~9天时超过39℃,如无并发症,在第2周开始下降,但也可持续发热1个月者,同时伴有头痛、肌痛、腹痛、厌食、乏力、腹胀、面色苍白、精神不振、相对缓脉。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "发热" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "头痛" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "肌痛" }, { "start_idx": 73, "end_idx": 74, "type": "sym", "entity": "腹痛" }, { "start_idx": 76, "end_idx": 77, "type": "sym", "entity": "厌食" }, { "start_idx": 79, "end_idx": 80, "type": "sym", "entity": "乏力" }, { "start_idx": 82, "end_idx": 83, "type": "sym", "entity": "腹胀" }, { "start_idx": 85, "end_idx": 88, "type": "sym", "entity": "面色苍白" }, { "start_idx": 90, "end_idx": 93, "type": "sym", "entity": "精神不振" }, { "start_idx": 95, "end_idx": 98, "type": "sym", "entity": "相对缓脉" } ] }, { "text": "第1周末出现肝脾肿大,重者伴黄疸,约在第2周,躯干部分见玫瑰斑疹。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肝脾" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "黄疸" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "躯干" }, { "start_idx": 23, "end_idx": 31, "type": "sym", "entity": "躯干部分见玫瑰斑疹" } ] }, { "text": "婴幼儿症状不典型,无相对缓脉,症状较轻,轻度发热,似病毒感染,而腹泻较常见,常被误诊为急性肠炎。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "相对缓脉" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "轻度发热" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "病毒感染" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "腹泻" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "急性肠炎" } ] }, { "text": "其他表现可有呼吸道感染的症状和体征。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "呼吸道感染" } ] }, { "text": "新生儿往往是母婴垂直传播引起,生后3天出现症状,以呕吐、腹泻、腹胀常见,体温变化不一;但可高达40℃,可抽搐、贫血、黄疸、肝大和体重明显减轻。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "呕吐" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "腹泻" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "腹胀" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "体温变化不一" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "高达40℃" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "抽搐" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "贫血" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "黄疸" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "肝大" }, { "start_idx": 64, "end_idx": 69, "type": "sym", "entity": "体重明显减轻" } ] }, { "text": "【并发症】肠出血和肠穿孔近年已见减少,胆道感染、胆囊穿孔、腮腺炎、中毒性脑病、伤寒杆菌脑膜炎都是少见的并发症。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肠出血" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "肠穿孔" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "胆道感染" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "胆囊穿孔" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "中毒性脑病" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "伤寒杆菌脑膜炎" } ] }, { "text": "【诊断】培养出伤寒杆菌可明确诊断。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "mic", "entity": "伤寒杆菌" } ] }, { "text": "疾病的早期,40%~60%的患者血培养阳性,粪便和尿培养在1周后转为阳性,粪便培养有时可在潜伏期已是阳性。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "血培养" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "血培养阳性" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "粪便和尿培养" }, { "start_idx": 22, "end_idx": 35, "type": "sym", "entity": "粪便和尿培养在1周后转为阳性" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "粪便培养" } ] }, { "text": "由于是间歇性和低水平的菌血症,对可疑病例应反复取血培养。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "菌血症" }, { "start_idx": 3, "end_idx": 13, "type": "sym", "entity": "间歇性和低水平的菌血症" }, { "start_idx": 24, "end_idx": 26, "type": "pro", "entity": "血培养" } ] }, { "text": "在疾病的后期当血培养转阴性时骨髓培养常可有阳性结果,此时小肠淋巴结、肝脾组织培养都可有阳性结果。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "血培养" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "血培养转阴性" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "骨髓培养" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "骨髓培养常可有阳性结果" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "小肠淋巴结" }, { "start_idx": 34, "end_idx": 39, "type": "pro", "entity": "肝脾组织培养" } ] }, { "text": "骨髓培养阳性率高,且可持续较长时间,是最敏感的诊断方法,阳性率可高达85%~90%,且不受早期抗生素的影响。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "骨髓培养" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "抗生素" } ] }, { "text": "在慢性带菌者,粪便或尿培养也可出现阳性,有些病例十二指肠引流液培养也有助于明确诊断。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "粪便或尿培养" }, { "start_idx": 24, "end_idx": 32, "type": "pro", "entity": "十二指肠引流液培养" } ] }, { "text": "早期快速诊断方法是使用单克隆抗体直接检测血中伤寒杆菌的和尿中伤寒杆菌Vi多糖抗原。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "单克隆抗体直接检测" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "血" }, { "start_idx": 22, "end_idx": 25, "type": "mic", "entity": "伤寒杆菌" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "尿" }, { "start_idx": 30, "end_idx": 33, "type": "mic", "entity": "伤寒杆菌" } ] }, { "text": "应用PCR技术测定血中特定伤寒基因片段,这种方法敏感性大大高于细菌培养。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "PCR技术" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "血" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "伤寒" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "细菌" } ] }, { "text": "血清肥达试验是测定血清抗O和H抗原的抗体,由于这一试验易出现假阴性和假阳性结果,所以易误诊。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "血清肥达试验" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "血清" } ] }, { "text": "【实验室检查】外周血白细胞总数减少,急性期不出现嗜酸性粒细胞,一旦出现说明病情有好转。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "ite", "entity": "外周血白细胞" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "外周血白细胞总数减少" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "嗜酸性粒细胞" }, { "start_idx": 18, "end_idx": 29, "type": "sym", "entity": "急性期不出现嗜酸性粒细胞" } ] }, { "text": "极期时可出现蛋白尿和管型。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "管型" } ] }, { "text": "肠出血时粪便潜血试验阳性,严重时可出现柏油样大便。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肠出血" }, { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "粪便潜血试验" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "柏油样大便" } ] }, { "text": "【鉴别诊断】在病程的初期,要与胃肠炎、病毒感染综合征、支气管炎或支气管肺炎鉴别。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "胃肠炎" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "病毒感染综合征" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "支气管炎" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "支气管肺炎" } ] }, { "text": "随着病情进展应同其他病原所致败血症及结核病等鉴别。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "败血症" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "结核病" } ] }, { "text": "【治疗】1.抗菌治疗抗生素是治疗伤寒的关键,由于细菌对抗生素耐药性增加,如何选用恰当的抗生素治疗是很重要的,也是人们争论之焦点。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "抗生素" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "抗生素" } ] }, { "text": "目前推荐用药量:氯霉素50mg/(kg•d),口服,或75mg/(kg•d),分4次静脉注射;氨苄西林100mg/(kg•d),分4~6次静脉滴注;阿莫西林100mg/(kg•d),分3次口服;复方磺胺甲唑(SMX)50mg/(kg•d),分2次口服。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "氯霉素" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "口服" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "静脉注射" }, { "start_idx": 47, "end_idx": 50, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 69, "end_idx": 72, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 74, "end_idx": 77, "type": "dru", "entity": "阿莫西林" }, { "start_idx": 94, "end_idx": 95, "type": "pro", "entity": "口服" }, { "start_idx": 97, "end_idx": 102, "type": "dru", "entity": "复方磺胺甲唑" }, { "start_idx": 104, "end_idx": 106, "type": "dru", "entity": "SMX" } ] }, { "text": "青霉素目前虽然可快速杀菌,但是容易复发和引起不良反应。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "青霉素" } ] }, { "text": "氯霉素是首选药。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "氯霉素" } ] }, { "text": "2.短程激素治疗仅用于中毒症状严重者,地塞米松3mg/kg首次剂量,随后1mg/kg每6小时一次,共用48小时。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "地塞米松" } ] }, { "text": "第三种疫苗是Vi荚膜多糖疫苗,对2岁以上小儿注射应用。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "dru", "entity": "Vi荚膜多糖疫苗" } ] }, { "text": "第七节卡氏肺孢子虫肺炎卡氏肺孢子虫肺炎(pneumocystisCariniipneumonia)又称为间质性浆细胞肺炎(interstitialplasmacellpneumonia),是宿主存在免疫缺陷的基础上发生的机会感染性疾病。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "卡氏肺孢子虫肺炎" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "卡氏肺孢子虫肺炎" }, { "start_idx": 20, "end_idx": 47, "type": "dis", "entity": "pneumocystisCariniipneumonia" }, { "start_idx": 52, "end_idx": 59, "type": "dis", "entity": "间质性浆细胞肺炎" }, { "start_idx": 61, "end_idx": 91, "type": "dis", "entity": "interstitialplasmacellpneumonia" }, { "start_idx": 110, "end_idx": 116, "type": "dis", "entity": "机会感染性疾病" } ] }, { "text": "卡氏肺孢子虫是原虫的一种。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "mic", "entity": "卡氏肺孢子虫" }, { "start_idx": 7, "end_idx": 8, "type": "mic", "entity": "原虫" } ] }, { "text": "外周血白细胞计数正常或稍高,偶见嗜酸性粒细胞增高,血气分析示PaO2显著降低,而PaCO2不增高。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "外周血白细胞计数" }, { "start_idx": 16, "end_idx": 21, "type": "ite", "entity": "嗜酸性粒细胞" } ] }, { "text": "呼吸道分泌物或肺组织,用环六亚甲基四胺硝酸银染色,可查见直径4~6μm的黑褐色圆形或椭圆形囊体。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "呼吸道分泌物" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肺组织" } ] }, { "text": "胸部X线摄片早期改变轻微,主要为肺纹理增多、肺门周围及下肺野可出现斑片状阴影。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "胸部X线摄片" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "肺纹理增多" }, { "start_idx": 22, "end_idx": 37, "type": "sym", "entity": "肺门周围及下肺野可出现斑片状阴影" } ] }, { "text": "胸膜少受累,但可发生气胸、纵隔气肿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "胸膜少受累" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "气胸" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "纵隔气肿" } ] }, { "text": "肺部阴影自肺门向周围伸展,两上肺病变较少。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "肺部阴影自肺门向周围伸展" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "两上肺病变较少" } ] }, { "text": "确诊有赖于痰或气道吸出物中查到病原体。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "ite", "entity": "痰" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "吸出物" } ] }, { "text": "本病治疗首选SMZ100mg/(kg•d),加TMP20mg/(kg•d),每日4次口服,亦可静脉滴注。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "SMZ" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "TMP" } ] }, { "text": "艾滋病患儿疗程3周,其他患儿2周。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "艾滋病" } ] }, { "text": "SMZ/TMP还可用于本病高危儿的预防。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "SMZ/TMP" } ] }, { "text": "以上药物无效或无法耐受者可选用喷他脒,但副作用较大。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "喷他脒" } ] }, { "text": "第三章儿科用药特点药物是治疗儿科疾病的很重要手段,而其副反应、过敏反应和毒性作用则常会对机体产生不良影响。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dep", "entity": "儿科" } ] }, { "text": "此外,新生儿期体液占体重的比例较大、肝脏酶系统发育不成熟肾清除率低血浆白蛋白含量低肝脏代谢药物的主要酶系统活性已成熟;肾小球滤过率和肾血流量在6~12个月可达到成人水平。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "肝脏酶系统" }, { "start_idx": 18, "end_idx": 27, "type": "sym", "entity": "肝脏酶系统发育不成熟" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肾" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "肾清除率低" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "血浆白蛋白" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "血浆白蛋白含量低" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "肝脏" }, { "start_idx": 59, "end_idx": 64, "type": "ite", "entity": "肾小球滤过率" }, { "start_idx": 66, "end_idx": 69, "type": "ite", "entity": "肾血流量" } ] }, { "text": "由于这一时期生长迅速,要密切注意药物通过不同的机制影响小儿的发育,如长期类固醇激素的应用可影响生长发育,中枢抑制性药物对智力有损害等。", "entities": [ { "start_idx": 36, "end_idx": 40, "type": "dru", "entity": "类固醇激素" }, { "start_idx": 52, "end_idx": 58, "type": "dru", "entity": "中枢抑制性药物" } ] }, { "text": "假如把身体划分为两部分,药物进入体内后首先迅速地分布于血液及血流供应充分的组织,如心、肝、肾、肺等,然后再由这些部位向血流不足的组织如肌肉、脂肪、皮肤等组织转运,达到平衡,这种模型称为“二室模型”。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血液" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "心" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "肝" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "肾" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "肺" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "肌肉" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "脂肪" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "皮肤" } ] }, { "text": "这一成比例的特性,结合对病人的监测,常被临床上用于调整药物的剂量;相反,某些药物如奥美拉唑、西咪替丁、水杨酸盐、茶碱、卡马西平、苯妥因钠等血液中药物浓度的变化与使用剂量不成比例,即呈非线性动力学特征。", "entities": [ { "start_idx": 41, "end_idx": 44, "type": "dru", "entity": "奥美拉唑" }, { "start_idx": 46, "end_idx": 49, "type": "dru", "entity": "西咪替丁" }, { "start_idx": 51, "end_idx": 54, "type": "dru", "entity": "水杨酸盐" }, { "start_idx": 56, "end_idx": 57, "type": "dru", "entity": "茶碱" }, { "start_idx": 59, "end_idx": 62, "type": "dru", "entity": "卡马西平" }, { "start_idx": 64, "end_idx": 67, "type": "dru", "entity": "苯妥因钠" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "血液" } ] }, { "text": "在通常情况下,这些药物在低剂量时遵循一级动力学过程,但随剂量增加由于与吸收有关的转运蛋白被饱和、血浆/组织蛋白结合过程被饱和、药物代谢酶被饱和、肾小管主动重吸收等任何过程被饱和都可以导致体内药物浓度增加,这时剂量稍有增加,常可导致血药浓度不成比例地增高,引起不良反应甚至中毒,并且由于半衰期延长,清除率明显降低,由非线性动力学而导致的血药浓度过高,可能产生严重的后果。", "entities": [ { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "转运蛋白" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "血浆" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "组织蛋白" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "药物代谢酶" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "肾小管" } ] }, { "text": "这一差异是药动学和药效学个体差异及许多生物变异的结果,如代谢、病理生理及遗传差异。", "entities": [ { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "病" } ] }, { "text": "由于存在药物疗效及毒性的个体差异,对特殊病人需调整给药方案,尤其是对某些药物,如血管活性药的剂量可根据患儿出现的即刻、易定量(如血压、心率等)的临床反应进行调整。", "entities": [ { "start_idx": 40, "end_idx": 44, "type": "dru", "entity": "血管活性药" }, { "start_idx": 64, "end_idx": 65, "type": "ite", "entity": "血压" }, { "start_idx": 67, "end_idx": 68, "type": "ite", "entity": "心率" } ] }, { "text": "而一种药物的药理或毒理反应可能直接与特异血清浓度范围有关。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "血清" } ] }, { "text": "这些治疗范围代表了平均值,仅49%的人群包括在均数±2SD范围内,因此血清药物浓度的临床监测只能作为药物干预和剂量调整的参考,使用时必须注意到个体化。", "entities": [ { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "血清" } ] }, { "text": "例如:一个病人的某药物血清浓度在低于有效治疗窗时即有完全的临床反应,而另一个病人,同一种疾病,用同一种药物,可能需要血清浓度在治疗浓度范围以上,才能获得相同程度的阳性治疗反应。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血清" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "血清" } ] }, { "text": "因此,血清药物浓度的治疗范围只能作为治疗的指导,最终必须通过临床反应来评价药物有效性。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血清" } ] }, { "text": "体液中药物浓度测定有助于减少药物毒性反应,同时达到最理想的治疗效果,为评价疾病治疗过程或药物相互作用对药物分布的影响提供了有效方法。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "体液" } ] }, { "text": "治疗性药物浓度监测并非对所有药物都是必须、必要和实用的。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "治疗性药物浓度监测" } ] }, { "text": "对药效学已较为清楚的药物,如利尿剂的利尿效果、抗高血压药降低血压作用等,并不需要常规监测血浓度。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "利尿剂" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "抗高血压药" } ] }, { "text": "应用血清药物浓度监测以指导治疗时,应首先了解该药物的药动学特性,以便确定用药后适当的采血时间及合理解释药物浓度和治疗反应。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "血清药物浓度监测" } ] }, { "text": "【钙】钙是人体内含量最多的元素之一,其中99%的钙集中在骨骼和牙齿,只有1%的钙以游离或结合的离子状态存在于其他组织和体液内,正常情况下,后者与骨骼内的钙维持动态平衡。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "骨骼" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "牙齿" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "组织" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "体液" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "骨骼" }, { "start_idx": 76, "end_idx": 76, "type": "bod", "entity": "钙" } ] }, { "text": "钙主要在小肠内吸收,维生素D、乳糖和蛋白质可促进钙的吸收,而植物中的植酸、草酸、膳食纤维和脂肪酸与钙结合可影响钙的吸收。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "钙" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "小肠" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "维生素D" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "蛋白质" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "钙" } ] }, { "text": "粪便是钙的主要排泄途径,其次是尿液,少量经汗液排出。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "粪便" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "钙" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "汗液" } ] }, { "text": "钙不仅是小儿骨骼和牙齿生长发育不可缺少的,而且还是维持肌肉神经兴奋性的重要物质,当血钙过低时,小儿容易哭闹和夜惊,甚至于出现手足抽搐等兴奋性增高的现象。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "钙" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "小儿骨骼" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "牙齿" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "肌肉神经" }, { "start_idx": 41, "end_idx": 42, "type": "ite", "entity": "血钙" }, { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "小儿容易哭闹和夜惊" }, { "start_idx": 57, "end_idx": 74, "type": "sym", "entity": "甚至于出现手足抽搐等兴奋性增高的现象" } ] }, { "text": "另外,钙还能激活体内多种酶的活性,如激活凝血酶原,使之成为凝血酶而发挥凝血功能。", "entities": [ { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "酶" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "激活凝血酶原" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "凝血酶" } ] }, { "text": "体内磷元素约80%存在于骨骼和牙齿中,是构成核酸、磷脂、酶等的原料,参与重要的生理代谢活动。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "骨骼" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "牙齿" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "核酸" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "磷脂" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "酶" } ] }, { "text": "维生素D有助于磷的吸收。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "维生素D" } ] }, { "text": "我国2000年全国学生体质健康调研显示6~18岁人群中缺铁性贫血的患病率为18.4%~22.4%。", "entities": [ { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "缺铁性贫血" } ] }, { "text": "2岁以下的婴幼儿患病率则更高(参见营养性疾病铁缺乏章节)。", "entities": [ { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "营养性疾病铁缺乏" } ] }, { "text": "新生婴儿由于体内铁元素的储存少,但此期生长发育快,因此,早产儿在出生2个月后,足月儿在出生4个月后,体内储存的铁元素已基本耗尽,再加上母乳中的铁含量低,牛奶中的铁吸收率低,很容易发生缺铁性贫血,故需及时补充铁元素。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "铁元素" }, { "start_idx": 91, "end_idx": 95, "type": "dis", "entity": "缺铁性贫血" } ] }, { "text": "【锌】微量元素锌分布于人体所有组织、器官、体液和分泌物中,95%以上存在于细胞内。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "组织" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "器官" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "体液" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "分泌物" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "细胞" } ] }, { "text": "锌也主要经小肠吸收,动物食物中的锌比植物食物中的锌容易吸收,铁可与锌竞争肠黏膜细胞上的受体而抑制其吸收。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "小肠" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "肠黏膜细胞" } ] }, { "text": "【碘】碘是组成甲状腺素的重要成分,甲状腺素有调节人体能量代谢以及三大营养素的合成和分解的作用,促进小儿生长发育。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "甲状腺素" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "甲状腺素" } ] }, { "text": "胎儿和新生儿缺碘不仅使生长发育迟缓,还可导致智力低下。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "缺碘" } ] }, { "text": "硒广泛分布在脂肪组织以外的所有组织,是机体内一种非特异抗氧化物质谷胱甘肽过氧化酶的重要成分之一,有清除体内过氧化物和自由基的作用,从而保护细胞膜和细胞器(如线粒体、微粒体和溶酶体)的膜。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "脂肪组织" }, { "start_idx": 24, "end_idx": 39, "type": "bod", "entity": "非特异抗氧化物质谷胱甘肽过氧化酶" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "过氧化物" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "自由基" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "细胞膜" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "细胞器" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "线粒体" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "微粒体" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "溶酶体" } ] }, { "text": "许多动物和临床流行病学研究显示硒对心血管和眼的健康有保护作用;而且与维生素E有重要的协同作用。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "心血管" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "眼" } ] }, { "text": "血硒浓度受土壤、水质和食物中硒含量的影响;海产品、动物肝脏、肾和肌肉及整粒谷类、洋葱等是硒的良好食物来源。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "血硒浓度" } ] }, { "text": "铜的生理功能有:参与结缔组织的合成,对骨骼、血管壁的健全起重要作用;参与铁代谢和造血功能;与中枢神经系统正常结构和功能有关;也与黑色素合成有关。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "结缔组织" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "骨骼" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "血管壁" }, { "start_idx": 46, "end_idx": 51, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "黑色素" } ] }, { "text": "【铬】铬在人体内的含量仅6mg,其主要的生理功能是促进胰岛素的作用,从而影响糖、脂肪和蛋白质的代谢。", "entities": [ { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "胰岛素" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "糖" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "脂肪" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "蛋白质" } ] }, { "text": "铬缺乏多发生在蛋白质-能量营养不良的儿童和应用全肠外营养的患者。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "铬缺乏" }, { "start_idx": 7, "end_idx": 16, "type": "dis", "entity": "蛋白质-能量营养不良" } ] }, { "text": "第四节哮喘持续状态哮喘持续状态是指对常规哮喘治疗反应差,呈急性进行性加重的严重发作,如不及时处理会发展成呼吸衰竭。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "哮喘" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "哮喘" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "常规哮喘治疗" }, { "start_idx": 30, "end_idx": 41, "type": "sym", "entity": "急性进行性加重的严重发作" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "疾病初期气道阻力非匀称增加,V/Q比例失调引起低氧血症,并代偿性出现PaCO2下降;气道阻进一步增加,代偿机制恶化,通气量明显下降,引起严重低氧血症和高碳酸血症;最后可以出现混合性酸中毒,肺动脉高压和右心功能及中枢神经系统功能异常。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "阻力非匀称增加" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "V/Q比例失调" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "低氧血症" }, { "start_idx": 76, "end_idx": 79, "type": "dis", "entity": "低氧血症" }, { "start_idx": 81, "end_idx": 85, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 93, "end_idx": 98, "type": "dis", "entity": "混合性酸中毒" }, { "start_idx": 100, "end_idx": 104, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 106, "end_idx": 120, "type": "dis", "entity": "右心功能及中枢神经系统功能异常" } ] }, { "text": "【诱发因素】如患儿哮喘治疗不当,长期应用β2受体激动剂,而未进行抗感染治疗;以及短期内吸入大量的过敏物质或强烈理化气体(如油漆)可以引起哮喘重度发作;此外脱水引起气道分泌物干燥分泌物干燥,痰栓阻塞气道;伴有各种并发症出现(气胸、肺不张等),造成哮喘治疗困难。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "哮喘" }, { "start_idx": 20, "end_idx": 32, "type": "dru", "entity": "β2受体激动剂" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "抗感染治疗" }, { "start_idx": 74, "end_idx": 75, "type": "dis", "entity": "哮喘" }, { "start_idx": 83, "end_idx": 84, "type": "dis", "entity": "脱水" }, { "start_idx": 87, "end_idx": 93, "type": "sym", "entity": "气道分泌物干燥" }, { "start_idx": 100, "end_idx": 105, "type": "sym", "entity": "痰栓阻塞气道" }, { "start_idx": 111, "end_idx": 113, "type": "dis", "entity": "并发症" }, { "start_idx": 117, "end_idx": 118, "type": "dis", "entity": "气胸" }, { "start_idx": 120, "end_idx": 122, "type": "dis", "entity": "肺不张" }, { "start_idx": 128, "end_idx": 131, "type": "pro", "entity": "哮喘治疗" } ] }, { "text": "【临床表现】除了明显喘憋、面色苍白、口唇发绀烦躁外,体格检查有助于判断疾病的严重程度:①呼吸是伴有明显的三凹征,提示FEV1和呼气流速峰值低于正常的50%;②奇脉血压超过2.93kPa(22mmHg)常提示PaCO2升高;③有呼气动作,但呼气音低,听不到哮鸣音,表明喘憋严重。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "明显喘憋" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "面色苍白" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "口唇" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "口唇发绀" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "烦躁外" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "体格检查" }, { "start_idx": 44, "end_idx": 54, "type": "sym", "entity": "呼吸是伴有明显的三凹征" }, { "start_idx": 58, "end_idx": 61, "type": "ite", "entity": "FEV1" }, { "start_idx": 63, "end_idx": 68, "type": "ite", "entity": "呼气流速峰值" }, { "start_idx": 79, "end_idx": 82, "type": "ite", "entity": "奇脉血压" }, { "start_idx": 118, "end_idx": 122, "type": "sym", "entity": "有呼气动作" }, { "start_idx": 124, "end_idx": 128, "type": "sym", "entity": "但呼气音低" }, { "start_idx": 130, "end_idx": 135, "type": "sym", "entity": "听不到哮鸣音" }, { "start_idx": 139, "end_idx": 140, "type": "dis", "entity": "喘憋" } ] }, { "text": "表8-26Wood临床评分标准注:≥5分,为呼吸功能不全;≥7分,伴PaCO2>8.7kPa为呼吸衰竭【治疗】1.吸氧给予吸入经湿化后的30%~50%浓度的氧,维持PaO260~80mmHg,SaO292%~95%。", "entities": [ { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "呼吸功能不全" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 63, "end_idx": 64, "type": "pro", "entity": "吸氧" }, { "start_idx": 65, "end_idx": 84, "type": "pro", "entity": "给予吸入经湿化后的30%~50%浓度的氧" }, { "start_idx": 86, "end_idx": 124, "type": "pro", "entity": "维持PaO260~80mmHg,SaO292%~95%" } ] }, { "text": "2.保持呼吸道湿润补充足够的液体,但补液速度不能过快;同时要避免环境过分干燥。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "保持呼吸道湿润" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "补充足够的液体" }, { "start_idx": 17, "end_idx": 25, "type": "pro", "entity": "但补液速度不能过快" }, { "start_idx": 27, "end_idx": 37, "type": "pro", "entity": "同时要避免环境过分干燥" } ] }, { "text": "3.支气管扩张药静脉用β受体兴奋剂的心血管不良反应较大,现已少用;目前多采用喷射式雾化吸入方法;吸入沙丁胺醇2.5~5mg/次,第一小时每20分钟一次,连用3次,然后每小时一次,根据喘息缓解情况,逐渐延长用药间隔。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "支气管扩张药" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "静脉" }, { "start_idx": 11, "end_idx": 16, "type": "dru", "entity": "β受体兴奋剂" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "心血管" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "心血管不良反应较大" }, { "start_idx": 38, "end_idx": 46, "type": "pro", "entity": "喷射式雾化吸入方法" }, { "start_idx": 48, "end_idx": 53, "type": "pro", "entity": "吸入沙丁胺醇" }, { "start_idx": 64, "end_idx": 74, "type": "pro", "entity": "第一小时每20分钟一次" }, { "start_idx": 76, "end_idx": 79, "type": "pro", "entity": "连用3次" }, { "start_idx": 81, "end_idx": 87, "type": "pro", "entity": "然后每小时一次" }, { "start_idx": 98, "end_idx": 105, "type": "pro", "entity": "逐渐延长用药间隔" } ] }, { "text": "反复用药时要监测心血管功能和血钾,保持心率<180次/分,无室性异位节律发生。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "pro", "entity": "反复用药时要监测心血管功能和血钾" }, { "start_idx": 17, "end_idx": 27, "type": "pro", "entity": "保持心率<180次/分" }, { "start_idx": 29, "end_idx": 37, "type": "pro", "entity": "无室性异位节律发生" } ] }, { "text": "同时可加用氨茶碱静脉注射;以每1mg/kg的负荷剂量增加血中氨茶碱浓度大约2μg/ml计算,对那些以前从未接受氨茶碱或口服茶碱制剂的患者,首次给予4~6mg/kg的氨茶碱负荷剂量以取得12μg/ml的水平;然后用维持量,剂量为每小时0.8~1mg/kg,严密观察毒性反应(胃不适、心律失常、抽搐)和氨茶碱水平,尽量维持在13~16μg/ml的稳定状态。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "静脉注射" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "血" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 55, "end_idx": 57, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 59, "end_idx": 64, "type": "dru", "entity": "口服茶碱制剂" }, { "start_idx": 71, "end_idx": 88, "type": "pro", "entity": "给予4~6mg/kg的氨茶碱负荷剂量" }, { "start_idx": 105, "end_idx": 108, "type": "pro", "entity": "用维持量" }, { "start_idx": 110, "end_idx": 125, "type": "pro", "entity": "剂量为每小时0.8~1mg/kg" }, { "start_idx": 127, "end_idx": 134, "type": "pro", "entity": "严密观察毒性反应" }, { "start_idx": 136, "end_idx": 138, "type": "sym", "entity": "胃不适" }, { "start_idx": 140, "end_idx": 143, "type": "sym", "entity": "心律失常" }, { "start_idx": 145, "end_idx": 146, "type": "sym", "entity": "抽搐" }, { "start_idx": 149, "end_idx": 151, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 155, "end_idx": 174, "type": "pro", "entity": "尽量维持在13~16μg/ml的稳定状态" } ] }, { "text": "除了喷射雾化吸入β2受体兴奋剂外,还可同时吸入抗胆碱能药物气道炎症够减轻气道炎症引起的局部迷走神经反射,与β2受体兴奋剂合用有互补作用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "喷射雾化" }, { "start_idx": 8, "end_idx": 21, "type": "dru", "entity": "β2受体兴奋剂外" }, { "start_idx": 29, "end_idx": 34, "type": "dru", "entity": "抗胆碱能药物" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "气道炎症" }, { "start_idx": 49, "end_idx": 56, "type": "dis", "entity": "局部迷走神经反射" }, { "start_idx": 59, "end_idx": 65, "type": "dru", "entity": "β2受体兴奋剂" } ] }, { "text": "具体剂量见表8-8,可以与β受体兴奋剂同时吸入。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "dru", "entity": "β受体兴奋剂" } ] }, { "text": "硫酸镁:每次25mg/kg+100ml生理盐水静脉滴注20~30分钟,有低血压、心动过缓、面色潮红等不良反应。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "硫酸镁" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "生理盐水" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 36, "end_idx": 38, "type": "sym", "entity": "低血压" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "心动过缓" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "面色潮红" } ] }, { "text": "4.应用糖皮质激素静脉用甲基泼尼松龙,第1次剂量2mg/kg,然后每6小时1次,每次1mg/kg;或氢化可的松,每6~8小时1次,每次5~10mg/kg。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dru", "entity": "应用糖皮质激素" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "静脉" }, { "start_idx": 12, "end_idx": 17, "type": "dru", "entity": "甲基泼尼松龙" }, { "start_idx": 19, "end_idx": 29, "type": "pro", "entity": "第1次剂量2mg/kg" }, { "start_idx": 33, "end_idx": 38, "type": "pro", "entity": "每6小时1次" }, { "start_idx": 40, "end_idx": 47, "type": "pro", "entity": "每次1mg/kg" }, { "start_idx": 50, "end_idx": 54, "type": "dru", "entity": "氢化可的松" }, { "start_idx": 56, "end_idx": 63, "type": "pro", "entity": "每6~8小时1次" }, { "start_idx": 65, "end_idx": 75, "type": "pro", "entity": "每次5~10mg/kg" } ] }, { "text": "可同时吸入Budesonide,每次1mg。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "dru", "entity": "Budesonide" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "每次1mg" } ] }, { "text": "5.控制感染尽管目前还有争论,但由于气道分泌物增加、环境条件差,加上大量糖皮质激素应用,应用抗生素有一定的合理性。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "气道分泌物" }, { "start_idx": 36, "end_idx": 40, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 46, "end_idx": 48, "type": "dru", "entity": "抗生素" } ] }, { "text": "6.观察和监护随访血气分析,分析气道阻塞程度;对长期应用β受体兴奋剂患儿要监测血电解质,注意低钾血症发生;对治疗效果不明显或病情恶化患儿,要注意肺部并发症存在,摄胸片观察是否伴有肺不张、气胸、气道异物。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "气道" }, { "start_idx": 28, "end_idx": 33, "type": "dru", "entity": "β受体兴奋剂" }, { "start_idx": 39, "end_idx": 42, "type": "ite", "entity": "血电解质" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "低钾血症" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "治疗效果不明显" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "病情恶化" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "肺部并发症" }, { "start_idx": 80, "end_idx": 82, "type": "ite", "entity": "摄胸片" }, { "start_idx": 89, "end_idx": 91, "type": "dis", "entity": "肺不张" }, { "start_idx": 93, "end_idx": 94, "type": "dis", "entity": "气胸" }, { "start_idx": 96, "end_idx": 99, "type": "dis", "entity": "气道异物" } ] }, { "text": "7.机械通气对经过以上处理病情不能改善,呼吸衰竭持续存在的情况下应考虑机械通气。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "机械通气" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "机械通气" } ] }, { "text": "机械通气指征:持续严重的呼吸困难,哮鸣音和呼吸音明显减弱;呼吸肌极度疲劳;在吸入纯氧下PaO2<8kPa(60mmHg),PaCO2>6.65kPa(50mmHg);有并发症(气胸、纵隔气肿等)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "机械通气" }, { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "持续严重的呼吸困难" }, { "start_idx": 17, "end_idx": 27, "type": "sym", "entity": "哮鸣音和呼吸音明显减弱" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "呼吸肌极度疲劳" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "吸入纯氧" }, { "start_idx": 106, "end_idx": 108, "type": "dis", "entity": "并发症" }, { "start_idx": 110, "end_idx": 111, "type": "dis", "entity": "气胸" }, { "start_idx": 113, "end_idx": 116, "type": "dis", "entity": "纵隔气肿" }, { "start_idx": 105, "end_idx": 118, "type": "sym", "entity": "有并发症(气胸、纵隔气肿等)" } ] }, { "text": "机械通气原则:①在尽量减少气压伤的基础上,维持足够的氧合和通气量直至其他治疗充分起效;②用定容型呼吸模式,以利控制合适的潮气量;③长呼气时间,低呼吸频率,保证足够的呼气时间;④呼气末正压应保持在低值,⑤通过呼吸机管路,吸入β受体兴奋药物;⑥机械通气下,伴有代酸患儿,可用NaHCO3纠酸。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "机械通气" }, { "start_idx": 103, "end_idx": 107, "type": "equ", "entity": "呼吸机管路" }, { "start_idx": 111, "end_idx": 117, "type": "dru", "entity": "β受体兴奋药物" }, { "start_idx": 120, "end_idx": 123, "type": "pro", "entity": "机械通气" }, { "start_idx": 128, "end_idx": 129, "type": "dis", "entity": "代酸" }, { "start_idx": 135, "end_idx": 152, "type": "dru", "entity": "NaHCO3" } ] }, { "text": "二、咽旁脓肿咽旁脓肿(lateralpharyngealabscess)病原学与咽后壁脓肿相同。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "咽旁脓肿" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "咽旁脓肿" }, { "start_idx": 12, "end_idx": 35, "type": "dis", "entity": "lateralpharyngealabscess" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "咽后壁脓肿" } ] }, { "text": "患儿病情常严重,伴高热、牙关紧闭、咽痛、吞咽困难,常有颈淋巴结肿大。", "entities": [ { "start_idx": 9, "end_idx": 23, "type": "sym", "entity": "高热、牙关紧闭、咽痛、吞咽困难" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "颈淋巴结" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "颈淋巴结肿大" } ] }, { "text": "由于受累部位肌肉痉挛引起斜颈。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肌肉" }, { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "受累部位肌肉痉挛" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "斜颈" } ] }, { "text": "治疗常需切开引流。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "切开引流" } ] }, { "text": "第三节Q热Q热(Qfever)是无皮疹的热性疾病,有急性型和慢性型两种。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "Q热" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "Q热" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "Qfever" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "无皮疹" } ] }, { "text": "【病原及流行病学】贝纳立克次体是本病的病原体,习惯上称Q热立克次体。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "mic", "entity": "贝纳立克次体" }, { "start_idx": 27, "end_idx": 32, "type": "mic", "entity": "Q热立克次体" } ] }, { "text": "贝纳立克次体是革兰染色阴性、嗜酸、耐热、专性细胞内寄生菌。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "mic", "entity": "贝纳立克次体" } ] }, { "text": "对人类的感染性特别强,在外界环境中存活久,是立克次体中唯一可不借助媒介节肢动物而通过气溶胶使人和动物发生感染的病原体,因而分布十分广泛。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "mic", "entity": "立克次体" } ] }, { "text": "全世界均有Q热报道。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "Q热" } ] }, { "text": "近年来以心内膜炎为特征的慢性Q热病例日益增多,其病情严重,抗生素疗效不佳,应引起足够重视。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "慢性Q热" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "抗生素" } ] }, { "text": "贝纳立克次体对人类和动物高度易感,少到一个病原体即可致病。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "mic", "entity": "贝纳立克次体" } ] }, { "text": "受感染动物和人的尿液、粪便、羊水、乳汁中均含有立克次体,经空气、食物、皮肤进入人体。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "尿液" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "粪便" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "羊水" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "乳汁" }, { "start_idx": 23, "end_idx": 26, "type": "mic", "entity": "立克次体" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "皮肤" } ] }, { "text": "【临床表现】急性Q热潜伏期2~3周。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "急性Q热" } ] }, { "text": "发病较急,病初即有发热,发热1~3周,同时有畏寒、全身乏力、酸痛、头痛,偶伴关节痛。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "发热" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "发热1~3周" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "畏寒" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "全身乏力" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "酸痛" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "关节痛" } ] }, { "text": "X线肺部检查见节段性和大叶性模糊阴影,多在肺下叶;肺门及支气管周围纹理增厚及有浸润影,肺炎经2~4周消失。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "X线肺部检查" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺下叶" }, { "start_idx": 7, "end_idx": 23, "type": "sym", "entity": "节段性和大叶性模糊阴影,多在肺下叶" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肺门" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "支气管" }, { "start_idx": 25, "end_idx": 41, "type": "sym", "entity": "肺门及支气管周围纹理增厚及有浸润影" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "肺炎" } ] }, { "text": "儿童很少有慢性Q热。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "慢性Q热" } ] }, { "text": "【诊断】注意流行病学资料,临床症状无特异性,Q热的确诊在于得到Ⅰ和Ⅱ相抗原的间接免疫荧光血清抗体滴定度增高或补体结合抗体滴定度增高。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "Q热" }, { "start_idx": 54, "end_idx": 64, "type": "sym", "entity": "补体结合抗体滴定度增高" } ] }, { "text": "单独Ⅱ相抗体的滴定度升高对急性Q热有特殊诊断价值。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "单独Ⅱ相抗体的滴定度升高" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "急性Q热" } ] }, { "text": "Ⅰ和Ⅱ相抗体持续升高用于诊断慢性Q热,Ⅰ相IgA抗体升高对诊断Q热心内膜炎有帮助。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "Ⅰ和Ⅱ相抗体持续升高" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "慢性Q热" }, { "start_idx": 19, "end_idx": 27, "type": "sym", "entity": "Ⅰ相IgA抗体升高" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "Q热心内膜炎" } ] }, { "text": "【治疗】采用四环素或多西环素治疗,但应争取早期治疗,过晚治疗对于缩短急性起病程没有作用。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "四环素" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "多西环素" } ] }, { "text": "第七章神经母细胞瘤神经母细胞瘤(neuroblastoma,NB)从原始神经嵴细胞演化而来,交感神经链、肾上腺髓质是最常见的原发部位。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "神经母细胞瘤" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "神经母细胞瘤" }, { "start_idx": 16, "end_idx": 28, "type": "dis", "entity": "neuroblastoma" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "NB" }, { "start_idx": 34, "end_idx": 40, "type": "bod", "entity": "原始神经嵴细胞" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "交感神经链" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "肾上腺髓质" } ] }, { "text": "不同年龄、肿瘤发生部位及不同的组织分化程度使其生物特性及临床表现有很大差异,部分可自然消退或转化成良性肿瘤,但另一部分病人却又十分难治,预后不良。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "组织" } ] }, { "text": "在过去的30年中,婴儿型或早期NB预后有了明显的改善,但大年龄晚期病人预后仍然十分恶劣。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "婴儿型或早期NB" } ] }, { "text": "在NB中有许多因素可影响预后,年龄、分期和N-MYC扩增仍然是最重要的因素。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "NB" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "N-MYC" } ] }, { "text": "【发病率】NB是儿童最常见的颅外实体瘤,占所有儿童肿瘤的8%~10%,一些高发地区如法国、以色列、瑞士、新西兰等的年发病率达11/100万(0~15岁),美国为25/100万,中国和印度的报告低于5/100万。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "NB" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "颅外实体瘤" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "【病理学】NB来自起源于神经嵴的原始多能交感神经细胞,形态为蓝色小圆细胞。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "NB" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "神经嵴" }, { "start_idx": 18, "end_idx": 25, "type": "bod", "entity": "多能交感神经细胞" } ] }, { "text": "从神经嵴移行后细胞的分化程度、类型及移行部位形成不同的交感神经系统正常组织,包括脊髓交感神经节、肾上腺嗜铬细胞。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "神经嵴" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "交感神经系统" }, { "start_idx": 40, "end_idx": 46, "type": "bod", "entity": "脊髓交感神经节" }, { "start_idx": 48, "end_idx": 54, "type": "bod", "entity": "肾上腺嗜铬细胞" } ] }, { "text": "NB组织学亚型与交感神经系统的正常分化模型相一致。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "NB" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "交感神经系统" } ] }, { "text": "典型的NB由一致的小细胞组成,约15%~50%的病例,母细胞周围有嗜酸性神经纤维网。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "NB" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "小细胞" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "母细胞" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "神经纤维网" } ] }, { "text": "另一种完全分化的、良性NB为神经节细胞瘤,由成熟的节细胞、神经纤维网及Schwann细胞组成。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "NB" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "神经节细胞瘤" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "节细胞" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "神经纤维网" }, { "start_idx": 35, "end_idx": 43, "type": "bod", "entity": "Schwann细胞" } ] }, { "text": "神经节母细胞瘤介于前两者之间,含有神经母细胞和节细胞混杂成分。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "神经节母细胞瘤" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "神经母细胞" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "节细胞" } ] }, { "text": "4个亚型即包括NB(Schwannin少基质型);GNB混合型(基质丰富型);GN成熟型和GNB结节型(包括少基质型和基质丰富型)。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "NB" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "GNB结节型" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "基质" } ] }, { "text": "前三型代表了NB的成熟过程,而最后一型则为多克隆性。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "NB" } ] }, { "text": "对NB而言,细胞分化分为3级,包括未分化、分化不良、分化型;细胞的有丝分裂指数(MKI)也分为低、中、高3级。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "NB" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "细胞" } ] }, { "text": "Shimada分类综合肿瘤细胞的分化程度、有丝分裂指数和年龄,将NB分为临床预后良好组(FH)和预后不良组(UFH),FH包括以下各类:①NB,MKI为低中度,年龄<1.5岁;②分化型NB,MKI为低度,年龄1.5~5岁;③GNB混合型;④GN。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "NB" }, { "start_idx": 69, "end_idx": 70, "type": "dis", "entity": "NB" }, { "start_idx": 89, "end_idx": 93, "type": "dis", "entity": "分化型NB" } ] }, { "text": "UFH包括①NB,MKI高级;②NB,MKI为中级,年龄1.5~5岁;③未分化或分化不良型NB,年龄1.5~5岁;④所有>5岁的NB;⑤GNB结节型。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "NB" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "不良型NB" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "NB" }, { "start_idx": 68, "end_idx": 73, "type": "dis", "entity": "GNB结节型" } ] }, { "text": "在病理上,除HE染色外,可进一步作免疫组化电镜检查来与其他小圆细胞肿瘤相鉴别,NB时神经特异性酯酶(NSE)阳性,电镜下可见典型的致密核,结合于膜上的神经分泌颗粒,在神经纤维网中有微丝和平行排列的微管。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "HE染色" }, { "start_idx": 17, "end_idx": 24, "type": "pro", "entity": "免疫组化电镜检查" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "小圆细胞肿瘤" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "NB" }, { "start_idx": 42, "end_idx": 48, "type": "bod", "entity": "神经特异性酯酶" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "NSE" }, { "start_idx": 57, "end_idx": 58, "type": "equ", "entity": "电镜" }, { "start_idx": 57, "end_idx": 67, "type": "sym", "entity": "电镜下可见典型的致密核" }, { "start_idx": 72, "end_idx": 72, "type": "bod", "entity": "膜" }, { "start_idx": 75, "end_idx": 80, "type": "bod", "entity": "神经分泌颗粒" }, { "start_idx": 69, "end_idx": 80, "type": "sym", "entity": "结合于膜上的神经分泌颗粒" }, { "start_idx": 83, "end_idx": 87, "type": "bod", "entity": "神经纤维网" }, { "start_idx": 98, "end_idx": 99, "type": "bod", "entity": "微管" }, { "start_idx": 82, "end_idx": 99, "type": "sym", "entity": "在神经纤维网中有微丝和平行排列的微管" } ] }, { "text": "其他常见部位为胸腔和颈部。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "胸腔" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "颈部" } ] }, { "text": "原发于腹部时以肾上腺及脊柱两侧交感神经链原发多见,一般在肿块较大时才出现症状,可有腹痛、腹围增大腰背部饱满扪及肿块、胃肠道症状胸腔时有纵隔压迫相关症状及呼吸道症状气促、咳嗽等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "腹部" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肾上腺" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脊柱" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "交感神经链" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "腹痛" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "腹" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "腹围增大" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "腰背部" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "腰背部饱满" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "胃肠道" }, { "start_idx": 53, "end_idx": 62, "type": "sym", "entity": "扪及肿块、胃肠道症状" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "胸腔" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "纵隔" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "呼吸道" }, { "start_idx": 63, "end_idx": 80, "type": "sym", "entity": "胸腔时有纵隔压迫相关症状及呼吸道症状" }, { "start_idx": 81, "end_idx": 82, "type": "sym", "entity": "气促" }, { "start_idx": 84, "end_idx": 85, "type": "sym", "entity": "咳嗽" } ] }, { "text": "晚期病人常有肢体疼痛贫血、发热、消瘦、眼眶部转移眼眶部转移形成具有特征性的熊猫眼,表现为眼球突出眶周青紫高血压及肿块部位相关压迫症状,如有椎管内浸润压迫时出现运动障碍大小便失禁等。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肢体" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "肢体疼痛" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "贫血" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "消瘦" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "眼眶部" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "眼眶部转移" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "眼眶部" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "眼球" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "眼球突出" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "眶" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "眶周青紫" }, { "start_idx": 52, "end_idx": 65, "type": "sym", "entity": "高血压及肿块部位相关压迫症状" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "椎管" }, { "start_idx": 69, "end_idx": 82, "type": "sym", "entity": "椎管内浸润压迫时出现运动障碍" }, { "start_idx": 83, "end_idx": 88, "type": "sym", "entity": "大小便失禁等" } ] }, { "text": "【实验室检查】尽量争取病理活检以明确诊断及分型。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "病理活检" } ] }, { "text": "85%~90%患儿尿中儿茶酚胺代谢产物同型香酸(HVA)、香草基杏仁酸(VMA)增高。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "尿" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "儿茶酚胺" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "同型香酸" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "HVA" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "香草基杏仁酸" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "VMA" }, { "start_idx": 7, "end_idx": 41, "type": "sym", "entity": "患儿尿中儿茶酚胺代谢产物同型香酸(HVA)、香草基杏仁酸(VMA)增高" } ] }, { "text": "细胞遗传学检查可发现1p-。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "细胞遗传学检查" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "1p" } ] }, { "text": "【诊断及分期】组织病理学检查是NB诊断的最重要手段,有时需结合免疫组织化学、电镜以明确诊断。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "NB" }, { "start_idx": 38, "end_idx": 39, "type": "equ", "entity": "电镜" } ] }, { "text": "影像学检查发现有与NB特征相符合的肿块,同时骨髓中发现NB肿瘤细胞,有明显增高的儿茶酚胺代谢产物(HVA或VMA)也可作出诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "影像学检查" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "NB" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肿块" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "骨髓" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "NB肿瘤细胞" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "儿茶酚胺" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "HVA" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "VMA" } ] }, { "text": "如病理诊断有困难时,染色体检查发现有1p-缺失或N-myc扩增支持NB诊断。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "染色体检查" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "1p" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "N-myc" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "NB" } ] }, { "text": "(二)生物学特征在NB中常见有N-myc扩增,N-myc对细胞分裂有正向调节作用,维A酸(RA)对N-myc表达有负向调节作用以致NB细胞停止增殖并分化,N-myc扩增>10倍为预后不良因素。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "NB" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "N-myc" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "N-myc" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "维A酸" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "RA" }, { "start_idx": 49, "end_idx": 53, "type": "bod", "entity": "N-myc" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "NB细胞" }, { "start_idx": 77, "end_idx": 81, "type": "bod", "entity": "N-myc" } ] }, { "text": "1p36.3缺失是易复发的因素,1p可能有肿瘤抑制因子,即使无N-myc扩增,1p36.3缺失仍有意义。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "1p36.3" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "1p" }, { "start_idx": 21, "end_idx": 26, "type": "bod", "entity": "肿瘤抑制因子" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "N-myc" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "1p36.3" } ] }, { "text": "17q获得(gain)时预后差。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "17q" } ] }, { "text": "NB表达酪胺酸激酶(Trk)家族受体激酶的研究进展较快,预后良好型表达TrkA、C;而预后不良型、N-myc扩增型表达TrkB。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "NB" }, { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "酪胺酸激酶" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "Trk" }, { "start_idx": 35, "end_idx": 40, "type": "bod", "entity": "TrkA、C" }, { "start_idx": 49, "end_idx": 53, "type": "bod", "entity": "N-myc" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "TrkB" } ] }, { "text": "【治疗】由于NB预后差异大,部分病人如小年龄、早期NB预后明显优于大年龄晚期组,因此应根据病人的预后因素如年龄、分期、N-myc扩增、1p缺失等采用分级治疗。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "NB" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "N-myc" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "1p" } ] }, { "text": "早期病人无N-myc扩增及1p缺失,可仅做手术,手术后随访。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "N-myc" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "1p" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "手术" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "手术" } ] }, { "text": "而大年龄、晚期,伴有N-myc扩增,1p缺失者需接受强化疗和手术,直至骨髓移植。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "N-myc" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "1p" }, { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "强化疗" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "手术" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "骨髓移植" } ] }, { "text": "手术、化疗、放疗仍为NB治疗的三大主要手段,根据其临床预后因素采用不同强度的治疗方案。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "化疗" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "放疗" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "NB" } ] }, { "text": "一般对局限性肿瘤主张先手术切除,再化疗。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "手术切除" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "化疗" } ] }, { "text": "而对估计手术不能切除者采用先化疗、再手术、再化疗或加放疗的策略。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "手术" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "化疗" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "手术" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "化疗" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "放疗" } ] }, { "text": "对NB敏感的药物有环磷酰胺、长春新碱、Vp-16、卡铂、顺铂、抗肿瘤抗生素(阿霉素)、异环磷酰胺等,各个协作组采用不同药物组合对晚期病人强化疗,但预后改善仍未令人满意。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "NB" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "长春新碱" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "Vp-16" }, { "start_idx": 25, "end_idx": 26, "type": "dru", "entity": "卡铂" }, { "start_idx": 28, "end_idx": 29, "type": "dru", "entity": "顺铂" }, { "start_idx": 31, "end_idx": 36, "type": "dru", "entity": "抗肿瘤抗生素" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "阿霉素" }, { "start_idx": 43, "end_idx": 47, "type": "dru", "entity": "异环磷酰胺" }, { "start_idx": 68, "end_idx": 70, "type": "pro", "entity": "强化疗" } ] }, { "text": "异基因移植与自体移植间结果无差异。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "异基因移植" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "自体移植" } ] }, { "text": "自体外周血干细胞移植时造血功能恢复要比骨髓干细胞移植快,并且肿瘤细胞污染的机会相对减少。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "自体外周血干细胞移植" }, { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "骨髓干细胞移植" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "肿瘤细胞" } ] }, { "text": "美国儿童肿瘤协作组对晚期病人在自身干细胞移植后随机分组进行13-顺维A酸治疗研究,一组病人接受160mg/(m2•d),每月用2周,共3~6月,另一组病人停化疗后不用药。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "pro", "entity": "自身干细胞移植" }, { "start_idx": 29, "end_idx": 35, "type": "dru", "entity": "13-顺维A酸" }, { "start_idx": 84, "end_idx": 85, "type": "pro", "entity": "化疗" } ] }, { "text": "结果为接受维A酸组3年EFS为47%,未接受组为25%,P=0.013。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "维A酸" } ] }, { "text": "在Ⅳ期病人及高危Ⅲ期病人中维A酸作用更为明显,分别为40%对22%,和77%对49%。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "维A酸" } ] }, { "text": "常用参考化疗方案(表11-11),一般21~28天为一疗程。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "化疗" } ] }, { "text": "表11-20NB时常用参考化疗方案说明:当环磷酰胺剂量>1.0g/m2时,应水化1500~2000ml/m2,并同时给予美斯纳1~2次,每次0.4g/m2。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "化疗" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "水化" }, { "start_idx": 72, "end_idx": 74, "type": "dru", "entity": "美斯纳" } ] }, { "text": "使用顺铂时需给予高渗盐水稀释(2~3张盐水),同时给予水化并补充钙、钾、镁以防电解质紊乱。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dru", "entity": "顺铂" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "水化" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "电解质" } ] }, { "text": "阿霉素累积剂量>320mg/m2,需谨慎应用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "阿霉素" } ] }, { "text": "病人<1岁,或Ⅰ、Ⅱ期,以OPEC、OPAC治疗为主,化疗剂量减25%。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "化疗" } ] }, { "text": "第二节肺容量和通气一、肺容量肺容量(图8-8)是指肺内容纳的气体量,是呼吸道和肺泡的总容量,反映了外呼吸的空间。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "肺容量" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "肺容量" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "肺容量" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肺" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "呼吸道" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肺泡" } ] }, { "text": "图8-7肺容量示意图TV:潮气量,IRV:补吸气量,ERV:补呼气量,RV:残气量,IC:深吸气量,FRC:功能残气量,VC:肺活量,TLC:肺总量(一)潮气量平静呼吸时,每次吸入或呼出的气量为潮气量(tidalvolume,VT)。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "肺容量" }, { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "TV" }, { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "潮气量" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "IRV" }, { "start_idx": 21, "end_idx": 24, "type": "ite", "entity": "补吸气量" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "ERV" }, { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "补呼气量" }, { "start_idx": 35, "end_idx": 36, "type": "ite", "entity": "RV" }, { "start_idx": 38, "end_idx": 40, "type": "ite", "entity": "残气量" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "IC" }, { "start_idx": 45, "end_idx": 48, "type": "ite", "entity": "深吸气量" }, { "start_idx": 50, "end_idx": 52, "type": "ite", "entity": "FRC" }, { "start_idx": 54, "end_idx": 58, "type": "ite", "entity": "功能残气量" }, { "start_idx": 60, "end_idx": 61, "type": "ite", "entity": "VC" }, { "start_idx": 63, "end_idx": 65, "type": "ite", "entity": "肺活量" }, { "start_idx": 67, "end_idx": 69, "type": "ite", "entity": "TLC" }, { "start_idx": 71, "end_idx": 73, "type": "ite", "entity": "肺总量" }, { "start_idx": 77, "end_idx": 79, "type": "ite", "entity": "潮气量" }, { "start_idx": 97, "end_idx": 99, "type": "ite", "entity": "潮气量" }, { "start_idx": 101, "end_idx": 111, "type": "ite", "entity": "tidalvolume" }, { "start_idx": 113, "end_idx": 114, "type": "ite", "entity": "VT" } ] }, { "text": "可用呼吸流速仪或肺量计测定。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "equ", "entity": "呼吸流速仪" }, { "start_idx": 8, "end_idx": 10, "type": "equ", "entity": "肺量计" } ] }, { "text": "为了校正体重对潮气量的影响,一般以ml/kg体重来表示。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "体重" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "潮气量" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "体重" } ] }, { "text": "小儿潮气量一般为6~10ml/kg。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "潮气量" } ] }, { "text": "小儿往往用“浅快型”方式呼吸以弥补潮气量、肺泡通气量不足和降低呼吸功。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "潮气量" }, { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "肺泡通气量" } ] }, { "text": "影响潮气量的主要因素是吸气肌功能,尤其是膈肌的活动。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "潮气量" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "吸气肌" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "膈肌" } ] }, { "text": "由于肺的通气储备极大,许多肺部疾病患者(如肺不张、肺实变及脓胸等),肺活量已明显减小,但潮气量仍无明显变化。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肺" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肺部" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "肺不张" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "肺实变" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "脓胸" }, { "start_idx": 34, "end_idx": 36, "type": "ite", "entity": "肺活量" }, { "start_idx": 44, "end_idx": 46, "type": "ite", "entity": "潮气量" } ] }, { "text": "只有当通气功能受损较严重或通气调节障碍时才会出现。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "通气功能受损较严重" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "通气调节障碍" } ] }, { "text": "在安静时,儿童仅用肺活量的12.5%来呼吸,而婴儿则需用30%左右,说明相比于大年龄儿童而言,婴儿的容量储备较差。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "肺活量" } ] }, { "text": "这也就是婴儿在呼吸道感染严重时易出现呼吸衰竭的原因。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "呼吸衰竭" } ] }, { "text": "(二)补吸气量平静吸气后所能再吸入的最大气量为补吸气量(inspiratoryreservevolume,IRV)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "补吸气量" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "补吸气量" }, { "start_idx": 28, "end_idx": 51, "type": "ite", "entity": "inspiratoryreservevolume" }, { "start_idx": 53, "end_idx": 55, "type": "ite", "entity": "IRV" } ] }, { "text": "(三)补呼气量平静呼气后所能继续呼出的最大气量为补呼气量(expiratoryreservevolume,ERV)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "补呼气量" }, { "start_idx": 24, "end_idx": 27, "type": "ite", "entity": "补呼气量" }, { "start_idx": 29, "end_idx": 51, "type": "ite", "entity": "expiratoryreservevolume" }, { "start_idx": 53, "end_idx": 55, "type": "ite", "entity": "ERV" } ] }, { "text": "体位对其有显著影响,在阻塞性通气障碍患者,细支气管在呼气相提早闭陷,补呼气量降低。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "阻塞性通气障碍" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "细支气管" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "补呼气量" } ] }, { "text": "(四)残气量补呼气后肺内不能呼出的残留气量为残气量(residualvolume,RV)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "残气量" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肺" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "残气量" }, { "start_idx": 26, "end_idx": 39, "type": "ite", "entity": "residualvolume" }, { "start_idx": 41, "end_idx": 42, "type": "ite", "entity": "RV" } ] }, { "text": "可以用体描仪和氦气稀释法或氮气洗出法测定。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "体描仪" }, { "start_idx": 7, "end_idx": 11, "type": "ite", "entity": "氦气稀释法" }, { "start_idx": 13, "end_idx": 17, "type": "ite", "entity": "氮气洗出法" } ] }, { "text": "婴幼儿残气量一般是肺总量的25%,它可以对吸入肺泡内的空气起缓冲作用。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "残气量" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "肺总量" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺泡" } ] }, { "text": "(五)深吸气量平静呼气后所能吸入的最大气量为深吸气量(inspiratorycapacityIC)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "深吸气量" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "深吸气量" }, { "start_idx": 27, "end_idx": 47, "type": "ite", "entity": "inspiratorycapacityIC" } ] }, { "text": "由VT+IRV组成。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "VT" }, { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "IRV" } ] }, { "text": "它是每分最大通气量和肺活量的主要成分(约占肺活量的75%),当深吸气量降低时,往往提示有限制性通气障碍可能。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "ite", "entity": "每分最大通气量" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "肺活量" }, { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "肺活量" }, { "start_idx": 31, "end_idx": 34, "type": "ite", "entity": "深吸气量" }, { "start_idx": 44, "end_idx": 50, "type": "dis", "entity": "限制性通气障碍" } ] }, { "text": "若每分最大通气量降低,而深吸气量正常时,可能与体质衰弱使呼吸肌无力有关。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "ite", "entity": "每分最大通气量" }, { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "深吸气量" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "体质衰弱" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "呼吸肌无力" } ] }, { "text": "(六)肺活量最大吸气后能呼出的最大气量为肺活量(vitalcapacity,VC)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "肺活量" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "肺活量" }, { "start_idx": 24, "end_idx": 36, "type": "ite", "entity": "vitalcapacity" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "VC" } ] }, { "text": "由IC+ERV组成,大致在50~70ml/kg。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "IC" }, { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "ERV" } ] }, { "text": "肺活量在婴幼儿测定难度大,有人提出用哭吵测定肺活量,但不准确,对于婴幼儿的实际意义并不很大。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "肺活量" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "肺活量" } ] }, { "text": "相对潮气量而言,肺活量虽有5~10倍的代偿潜力,但在病理情况下,婴幼儿的残气量增高加上无效腔大、基础呼吸快、气道易堵塞等因素使VC很难发挥应有的代偿效果。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "潮气量" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "肺活量" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "残气量" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "无效腔" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "气道" }, { "start_idx": 63, "end_idx": 64, "type": "ite", "entity": "VC" } ] }, { "text": "因此临床上婴幼儿肺炎呼吸衰竭的发生率远远高于年长儿。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "肺炎呼吸衰竭" } ] }, { "text": "(七)功能残气量平静呼气后肺内所含有的气量为功能残气量(functionalresidualcapacity,FRC),由ERV+RV组成。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "功能残气量" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肺" }, { "start_idx": 22, "end_idx": 26, "type": "ite", "entity": "功能残气量" }, { "start_idx": 28, "end_idx": 53, "type": "ite", "entity": "functionalresidualcapacity" }, { "start_idx": 55, "end_idx": 57, "type": "ite", "entity": "FRC" }, { "start_idx": 61, "end_idx": 63, "type": "ite", "entity": "ERV" }, { "start_idx": 65, "end_idx": 66, "type": "ite", "entity": "RV" } ] }, { "text": "可以用体描仪和氦气稀释法或氮气洗出法测定。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "体描仪" }, { "start_idx": 7, "end_idx": 11, "type": "ite", "entity": "氦气稀释法" }, { "start_idx": 13, "end_idx": 17, "type": "ite", "entity": "氮气洗出法" } ] }, { "text": "功能残气位时,吸气肌和呼气肌都处于松弛状态,此刻胸廓向外的牵张力与肺泡向内的弹性回缩力以及表面张力平衡,肺泡内压为零。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "吸气肌" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "呼气肌" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "胸廓" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肺泡" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "肺泡" } ] }, { "text": "功能残气在生理上起着稳定肺泡气体分压的作用,减小了通气间歇对肺泡内气体交换的影响。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "功能残气" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肺泡" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肺泡" } ] }, { "text": "FRC一般是肺总量的50%左右,足月儿20~30ml/kg,相当于出生时肺液的含量(15~20ml)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "FRC" }, { "start_idx": 6, "end_idx": 8, "type": "ite", "entity": "肺总量" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肺液" } ] }, { "text": "出生后最初几次呼吸时的压力-容积变化可以反映FRC的形成。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "FRC" } ] }, { "text": "RDS时应用肺表面活性物质(PS)或机械通气后期的肺恢复期体内自行产生的PS,可以提高FRC。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "RDS" }, { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "PS" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肺" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "PS" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "FRC" } ] }, { "text": "机械通气时应用呼气末正压(PEEP)也可以提高FRC。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "呼气末正压" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "PEEP" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "FRC" } ] }, { "text": "研究表明,婴幼儿的功能残气与体表面积、身高、体重及胸围均明显相关,尤其与身高呈直线相关,而与性别无相关。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "功能残气" }, { "start_idx": 14, "end_idx": 17, "type": "ite", "entity": "体表面积" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "身高" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "体重" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "胸围" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "身高" }, { "start_idx": 46, "end_idx": 47, "type": "ite", "entity": "性别" } ] }, { "text": "测FRC最常用技术是氦稀释技术,这项技术的原理是在未知肺容量和已知氦容积之间的气体平衡的基础上建立的。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "ite", "entity": "FRC" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "氦稀释" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "肺容量" } ] }, { "text": "通过氦浓度的变化即可计算出肺容量。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "肺容量" } ] }, { "text": "同样肺容积也可通过氮清洗技术获得。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "肺容积" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "氮清洗" } ] }, { "text": "测试时让婴儿吸入无氮气体,冲洗出肺泡内的氮气,通过快速反应的氮分析仪测定清洗出的氮量,最后计算出功能残气。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肺泡" }, { "start_idx": 30, "end_idx": 33, "type": "equ", "entity": "氮分析仪" }, { "start_idx": 48, "end_idx": 51, "type": "ite", "entity": "功能残气" } ] }, { "text": "现在较新的方法是用超声波流速仪和气体质谱技术,以惰性气体SF6洗入-洗出法来检测。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "equ", "entity": "超声波流速仪" }, { "start_idx": 16, "end_idx": 21, "type": "ite", "entity": "气体质谱技术" }, { "start_idx": 24, "end_idx": 36, "type": "ite", "entity": "惰性气体SF6洗入-洗出法" } ] }, { "text": "(八)肺总量深吸气后肺内所含有的总气量为肺总量(totallungcapacity,TLC),由VC+RV组成。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "肺总量" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肺" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "肺总量" }, { "start_idx": 24, "end_idx": 40, "type": "ite", "entity": "totallungcapacity" }, { "start_idx": 42, "end_idx": 44, "type": "ite", "entity": "TLC" }, { "start_idx": 48, "end_idx": 49, "type": "ite", "entity": "VC" }, { "start_idx": 51, "end_idx": 52, "type": "ite", "entity": "RV" } ] }, { "text": "四、并发症处理和临床转归(一)发展为严重肺损伤和急性呼吸窘迫综合征中枢性呼吸衰竭可以发展为呼吸机相关性肺炎和肺损伤。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "肺损伤" }, { "start_idx": 24, "end_idx": 32, "type": "dis", "entity": "急性呼吸窘迫综合征" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "中枢性呼吸衰竭" }, { "start_idx": 45, "end_idx": 52, "type": "dis", "entity": "呼吸机相关性肺炎" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "肺损伤" } ] }, { "text": "持续机械通气时,呼吸管理不善,可以导致气道肺泡发育不良,呼吸道细菌感染,发展为肺炎,加重呼吸衰竭。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "机械通气" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "气道肺泡发育不良" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "呼吸道细菌感染" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "肺炎" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "化疗和免疫抑制时、肠道缺血缺氧-再灌注性损伤性损伤等可以导致严重肺部感染性损伤,并发展为ARDS。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "免疫抑制" }, { "start_idx": 9, "end_idx": 21, "type": "dis", "entity": "肠道缺血缺氧-再灌注性损伤" }, { "start_idx": 30, "end_idx": 38, "type": "dis", "entity": "严重肺部感染性损伤" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "ARDS" } ] }, { "text": "(二)发展为肺外脏器功能衰竭呼吸衰竭时持续低氧血症可以导致肺部和肺外脏器功能衰竭。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "肺外脏器" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "低氧血症" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肺部" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "肺外脏器" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "功能衰竭" } ] }, { "text": "主要由于肺部炎症细胞大量集聚,释放促炎症介质进入循环,攻击肺外脏器,导致肺外脏器的多脏器结构损害,可以发展为多脏器功能障碍和衰竭。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "肺部炎症细胞" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "促炎症介质" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "肺外脏器" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "肺外脏器" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "多脏器" } ] }, { "text": "第八节阿米巴病【病原】阿米巴病(amoebiasis)是由溶组织阿米巴原虫所引起的疾病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "阿米巴病" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "阿米巴病" }, { "start_idx": 16, "end_idx": 25, "type": "dis", "entity": "amoebiasis" }, { "start_idx": 29, "end_idx": 36, "type": "mic", "entity": "溶组织阿米巴原虫" } ] }, { "text": "溶组织阿米巴(entamoebahistolytica)可分为滋养体和包囊两期。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "mic", "entity": "溶组织阿米巴" }, { "start_idx": 7, "end_idx": 26, "type": "mic", "entity": "entamoebahistolytica" } ] }, { "text": "滋养体直径为20~40μm,有伪足,可侵入肠组织,可于患者肠组织及稀粪便中见到。", "entities": [ { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "肠" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "稀粪便" } ] }, { "text": "不能侵入肠组织的滋养体在肠腔中形成包囊,由粪便排出体外。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肠腔" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "粪便" } ] }, { "text": "溶组织阿米巴的生活史:包囊通过污染的食物及饮水进入人的消化道,在小肠下段由于胰蛋白酶的消化作用,囊壁被破坏形成小阿米巴(滋养体),继续分裂繁殖,侵犯组织,形成包囊排出体外。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "mic", "entity": "溶组织阿米巴" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "消化道" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "小肠下段" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "胰蛋白酶" }, { "start_idx": 55, "end_idx": 58, "type": "mic", "entity": "小阿米巴" } ] }, { "text": "【发病机制及病理】在人机体免疫正常情况下,阿米巴在结肠腔内呈共栖状态,并不侵犯肠组织;当机体免疫受损,营养不良时,溶组织阿米巴侵入肠壁组织,致临床发病。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "阿米巴" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "结肠腔" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "肠组织" }, { "start_idx": 44, "end_idx": 49, "type": "sym", "entity": "机体免疫受损" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "营养不良" }, { "start_idx": 57, "end_idx": 62, "type": "mic", "entity": "溶组织阿米巴" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "肠壁组织" } ] }, { "text": "病变部位主要在大肠,以粪便停留较长的回盲部、升结肠、乙状结肠与直肠为多,有时也可侵犯回肠。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "大肠" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "粪便" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "回盲部" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "升结肠" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "乙状结肠" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "直肠" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "回肠" } ] }, { "text": "肠壁黏膜充血、水肿或溃疡溃疡呈底大、口小如烧瓶样。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肠壁黏膜" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "肠壁黏膜充血、水肿或溃疡" }, { "start_idx": 12, "end_idx": 23, "type": "sym", "entity": "溃疡呈底大、口小如烧瓶样" } ] }, { "text": "严重病变可穿过浆膜层,引起肠穿孔及腹膜炎。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "浆膜层" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肠穿孔" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "慢性期出现肠黏膜上皮增生溃疡底出现肉芽组织,致肠壁增厚、狭窄。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肠黏膜" }, { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "肠黏膜上皮增生" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "溃疡底出现肉芽组织" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肠壁" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "肠壁增厚、狭窄" } ] }, { "text": "如原虫进入门脉系统,可引起肝炎、肝脓肿。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "门脉系统" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "肝炎" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "肝脓肿" } ] }, { "text": "【临床表现】潜伏期为一周至数月不等,症状轻重不一,主要取决于阿米巴原虫所侵犯的组织和器官。", "entities": [ { "start_idx": 30, "end_idx": 34, "type": "mic", "entity": "阿米巴原虫" } ] }, { "text": "肠道是最常见的初发病变部位,原虫侵入大肠后引起以痢疾为主的症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肠道" }, { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "原虫" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "大肠" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "痢疾" } ] }, { "text": "轻者只有腹部不适食欲缺乏。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "腹部" }, { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "腹部不适" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "食欲缺乏" } ] }, { "text": "典型表现为腹痛,腹泻,大便如猪肝酱样每天大便5~6次,里急后重不明显。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "腹痛" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "腹泻" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "大便" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "大便如猪肝酱样" }, { "start_idx": 18, "end_idx": 33, "type": "sym", "entity": "每天大便5~6次,里急后重不明显" } ] }, { "text": "迁徙性感染可遍及全身各个器官和部位,出现相应的症状,如在眼睛可致棘阿米巴角膜炎。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "迁徙性感染" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "眼睛" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "棘阿米巴角膜炎" } ] }, { "text": "【并发症】(一)肝炎及肝脓肿以右叶多见,出现弛张型高热,腹痛,肝脏肿大,经皮肝穿刺能抽出牛奶咖啡样脓液能从脓汁中找到阿米巴滋养体。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肝炎" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肝脓肿" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "右叶" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "弛张型高热" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "腹痛" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肝脏" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "肝脏肿大" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "皮肝穿刺" }, { "start_idx": 36, "end_idx": 50, "type": "sym", "entity": "经皮肝穿刺能抽出牛奶咖啡样脓液" }, { "start_idx": 58, "end_idx": 63, "type": "mic", "entity": "阿米巴滋养体" }, { "start_idx": 51, "end_idx": 63, "type": "sym", "entity": "能从脓汁中找到阿米巴滋养体" } ] }, { "text": "(二)肺脓肿和脑脓肿偶见于血行播散的病儿,多在病程终末期或幼儿自母亲传染者。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "肺脓肿" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "(三)腹膜炎较少见。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "【实验室诊断】(一)肠阿米巴病以粪便中找到阿米巴原虫为最可靠依据,送检粪便应新鲜,挑选有黏液、脓血的部分,至少送检4~6次;若粪便检查屡阴性而临床不能排出本病时,可做阿米巴培养,或用乙状结肠镜直接观察黏膜溃疡,必要时取活检。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "肠阿米巴病" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "粪便" }, { "start_idx": 21, "end_idx": 25, "type": "mic", "entity": "阿米巴原虫" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "粪便" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "黏液" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "脓血" }, { "start_idx": 63, "end_idx": 69, "type": "sym", "entity": "粪便检查屡阴性" }, { "start_idx": 91, "end_idx": 95, "type": "equ", "entity": "乙状结肠镜" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "黏膜" }, { "start_idx": 109, "end_idx": 110, "type": "pro", "entity": "活检" } ] }, { "text": "(二)肠外阿米巴病可结合血清学查抗体,如间接荧光抗体、酶免疫方法及B超检查。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "肠外阿米巴病" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "抗体" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "间接荧光抗体" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "酶免疫方法" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "B超检查" } ] }, { "text": "【鉴别诊断】需要鉴别的疾病有细菌性痢疾、细菌性肝脓肿及胆道蛔虫症等。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "细菌性痢疾" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "细菌性肝脓肿" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "胆道蛔虫症" } ] }, { "text": "(二)病因治疗1.阿米巴痢疾急性期病例甲硝唑(灭滴灵)为首选,儿童35~50mg/(kg•d),每天最大剂量2200mg,分3次口服,5~7天为一疗程。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "阿米巴痢疾" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "甲硝唑" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "灭滴灵" }, { "start_idx": 64, "end_idx": 65, "type": "pro", "entity": "口服" } ] }, { "text": "对于不能服药的重型病例,可用吐根碱,2~5岁每次5~20mg,5~10岁30~45mg,每天1次,深层皮下注射;也可按1mg/(kg•d),分1~2次注射,疗程8~10天。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "吐根碱" }, { "start_idx": 49, "end_idx": 54, "type": "pro", "entity": "深层皮下注射" } ] }, { "text": "慢性期或复发的病例常用有机碘制剂。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "有机碘制剂" } ] }, { "text": "如喹碘仿,口服为每次20~25mg/kg,一日3次,8~10天为一疗程;也可灌肠,1~2g溶于100~200ml生理盐水内,保留灌肠,每天1次,连用8~10天。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "喹碘仿" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "口服" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "灌肠" }, { "start_idx": 64, "end_idx": 65, "type": "pro", "entity": "灌肠" } ] }, { "text": "另外,氯碘喹每次10~20mg/kg,每天3~4次,连服10天。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "氯碘喹" } ] }, { "text": "2.阿米巴肝脓肿甲硝唑为首选,剂量同前,10天为一疗程;磷酸氯喹,每次10mg/kg,每天2次,连服2天后改为每天1次,连服2周或更久。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "阿米巴肝脓肿" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "甲硝唑" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "磷酸氯喹" } ] }, { "text": "上述两种药可轮换应用,若有细菌感染,应同时加用抗生素。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "细菌感染" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "抗生素" } ] }, { "text": "脓肿大时,可同时穿刺抽脓。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "穿刺抽脓" } ] }, { "text": "3.胸、腹部阿米巴病的治疗诊断有困难时,可用吐根碱试验治疗,如3天内热度下降,既可给予全程治疗。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "胸、腹部阿米巴病" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "吐根碱" } ] }, { "text": "4.阿米巴包囊携带者的治疗选用一种作用于肠腔阿米巴药物,如甲硝唑、氯碘喹等,剂量同前。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "阿米巴" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肠腔" }, { "start_idx": 22, "end_idx": 24, "type": "mic", "entity": "阿米巴" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "甲硝唑" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "氯碘喹" } ] }, { "text": "【预防】包括:①注意饮食卫生,饭前洗手,水果及生吃的蔬菜要洗净;②治疗有阿米巴包囊携带者;③加强卫生宣教。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "注意饮食卫生" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "饭前洗手" }, { "start_idx": 20, "end_idx": 30, "type": "pro", "entity": "水果及生吃的蔬菜要洗净" }, { "start_idx": 46, "end_idx": 51, "type": "pro", "entity": "加强卫生宣教" } ] }, { "text": "第二节无菌性脑膜炎无菌性脑膜炎(asepticmeningitis)又称浆液性脑膜炎、淋巴细胞性脑膜炎或病毒性脑膜炎,是多种病毒性神经系统感染的常见表现。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "无菌性脑膜炎" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "无菌性脑膜炎" }, { "start_idx": 16, "end_idx": 32, "type": "dis", "entity": "asepticmeningitis" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "浆液性脑膜炎" }, { "start_idx": 43, "end_idx": 50, "type": "dis", "entity": "淋巴细胞性脑膜炎" }, { "start_idx": 62, "end_idx": 70, "type": "dis", "entity": "病毒性神经系统感染" } ] }, { "text": "一般无严重的脑实质损害症状,如瘫痪、昏迷或惊厥持续状态。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "脑实质损害症状" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "瘫痪" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "昏迷" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "惊厥" } ] }, { "text": "病毒性无菌性脑膜类的病程一般为数日至2周。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "病毒性无菌性脑膜类" } ] }, { "text": "多数病人急性期过后恢复完全,但有些病人在随后的几周内可仍有头晕、疲乏以及间歇性头痛等症状,个别甚至持续数月或数年。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "头晕" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "疲乏" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "间歇性头痛" } ] }, { "text": "远期随访还发现,病情恢复后数年内此类患儿出现学习困难、行为异常以及复发性惊厥等神经精神症状的危险性较高。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "学习困难" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "行为异常" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "复发性惊厥" } ] }, { "text": "复发性无菌性脑膜炎少见,Mollaret脑膜炎属其中之一。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "复发性无菌性脑膜炎" }, { "start_idx": 12, "end_idx": 22, "type": "dis", "entity": "Mollaret脑膜炎" } ] }, { "text": "该病的临床表现以良性复发性无菌性脑膜炎为特征。", "entities": [ { "start_idx": 7, "end_idx": 21, "type": "sym", "entity": "以良性复发性无菌性脑膜炎为特征" } ] }, { "text": "脑脊液表现与病毒性脑膜炎相似,但可见到内皮样细胞(Mollaret细胞),其体积较大,在体外很快溶解消失,故怀疑本病时应取新鲜脑脊液送检。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑脊液" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "病毒性脑膜炎" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "内皮样细胞" }, { "start_idx": 25, "end_idx": 34, "type": "bod", "entity": "Mollaret细胞" } ] }, { "text": "本病病因尚未完全明确,可能与单纯疱疹病毒4型(HSV-4)或EBV有关。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "单纯疱疹病毒4型" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "EBV" } ] }, { "text": "【实验室检查与诊断】无菌性脑膜炎的脑脊液多有异常改变,通常表现为轻度细胞和/或蛋白增多,糖和氯化物一般正常。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "无菌性脑膜炎" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "蛋白" }, { "start_idx": 32, "end_idx": 42, "type": "sym", "entity": "轻度细胞和/或蛋白增多" } ] }, { "text": "早期脑脊液炎性细胞中可以中性粒细胞为主,以后则以淋巴细胞为主。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "脑脊液炎性细胞" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "可于发病早期采集标本(脑脊液、粪便、血液、尿液、呼吸道黏膜,或必要时脑活检组织等)分离病毒。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "脑脊液" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "粪便" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "血液" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "尿液" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "呼吸道黏膜" }, { "start_idx": 34, "end_idx": 36, "type": "pro", "entity": "脑活检" } ] }, { "text": "某些病毒感染,如腮腺炎病毒和巨细胞病毒等,可于极期送检标本,检测早期IgM抗体,如为阳性则有助于早期确诊。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "mic", "entity": "腮腺炎病毒" }, { "start_idx": 14, "end_idx": 18, "type": "mic", "entity": "巨细胞病毒" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "送检标本" } ] }, { "text": "【治疗】多数病毒引起的无菌性脑膜炎缺乏特异性治疗。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "病毒" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "无菌性脑膜炎" } ] }, { "text": "第三章神经管畸形畸形如果妇女怀孕3个月之内,胚胎发育时神经管闭合过程受到影响即产生胎儿脑或脊髓发育异常儿童颅脑的畸形神经管畸形(neuraltubedefects,NTDs)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "神经管畸形" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "畸形" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "神经管" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "脑" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "脊髓" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "脑或脊髓发育异常" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "颅脑" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "畸形" }, { "start_idx": 51, "end_idx": 57, "type": "sym", "entity": "儿童颅脑的畸形" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "神经管畸形" }, { "start_idx": 64, "end_idx": 80, "type": "dis", "entity": "neuraltubedefects" }, { "start_idx": 82, "end_idx": 85, "type": "dis", "entity": "NTDs" } ] }, { "text": "NTDs是世界范围内的一个重要公共卫生问题,中国是世界上已知的NTDs高发国家。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "NTDs" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "NTDs" } ] }, { "text": "NTDs是造成孕妇流产及死胎主要原因之一,也是造成婴儿死亡和患者终身残疾的主要原因之一。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "NTDs" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "流产" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "死胎" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "残疾" } ] }, { "text": "目前NTDs的防治已被高度重视。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "NTDs" } ] }, { "text": "【流行病学】NTDs属于世界范围内的一种先天神经发育异常疾病,其发病率在各国、各地区是不同的,且同一地区各种族NTDs发病率也有所不同。", "entities": [ { "start_idx": 20, "end_idx": 29, "type": "dis", "entity": "先天神经发育异常疾病" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "NTDs" } ] }, { "text": "我国主要出生缺陷发生率顺位变化显示,1986—2006年NTDs发生率呈现下降趋势,从3.25‰降至0.72‰,从居各种出生缺陷之首降至第四。", "entities": [ { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "NTDs" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "出生缺陷" } ] }, { "text": "产前超声检查可对NTDs做出早期诊断,无脑畸形的准确度可达90%以上,脊柱裂达70%。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "超声检查" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "NTDs" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "无脑畸形" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "脊柱裂" } ] }, { "text": "此外孕母血清或羊水甲胎蛋白(AFP)的增高也有助于产前诊断。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "血清" }, { "start_idx": 7, "end_idx": 12, "type": "ite", "entity": "羊水甲胎蛋白" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "AFP" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "产前诊断" } ] }, { "text": "普通人群生育NTDs儿的风险为0.1%~0.3%。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "NTDs" } ] }, { "text": "已生过一胎NTDs再发风险为4%~5%,生过两胎缺陷者为10%,故再次怀孕前应进行遗传咨询。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "NTDs" } ] }, { "text": "美国2006年全球出生缺陷报告NTDs患儿达32.4万,居全球前5位常见严重出生缺陷第二位。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "出生缺陷" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "NTDs" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "严重出生缺陷" } ] }, { "text": "其中南美发病率最高,高达2‰,NTDs次高发的国家依次为墨西哥、挪威和法国,发病率分别为1.34‰、1.19‰和1.15‰。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "NTDs" } ] }, { "text": "与其他国家比较,我国NTDs发病率为0.82‰。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "NTDs" } ] }, { "text": "根据国际出生缺陷监测情报所统计资料,世界20多个国家近20年左右的NTDs发病率波动在0.3‰~2.1‰,据此推算全世界每年大约有30万~40万NTDs病例发生,其中女性发病要多于男性。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "出生缺陷" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "NTDs" } ] }, { "text": "北京医科大学中国妇婴保健中心与美国疾病控制中心合作,于1992年3月~1993年8月在河北、山西、江苏和浙江省等30个市、县进行的出生缺陷监测结果显示,北方地区NTDs发生率约为7‰,南方地区约为1.5‰。", "entities": [ { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "NTDs" } ] }, { "text": "这些资料表明,根据已有的报告数字,我国NTDs发病率在世界上是最高的国家之一。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "NTDs" } ] }, { "text": "我国NTDs发病率的分布具有北方高于南方,农村高于城市,夏秋季高于冬春季三大特点。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "NTDs" } ] }, { "text": "世界上英国NTDs发病率也很高,尤以北爱尔兰为多,1976年北爱尔兰NTDs发病率为6.4‰,其中贝尔法斯特市高达8.7‰。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "NTDs" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "NTDs" } ] }, { "text": "NTDs次高发的国家和地区是以色列、拉丁美洲和匈牙利等,NTDs较低发的国家和地区是丹麦、瑞典、芬兰和法国等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "NTDs" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "NTDs" } ] }, { "text": "在NTDs类型上,根据我国1996—2006年31个省市自治区出生缺陷监测结果,我国是以无脑畸形居首位,占51.2%左右,脊柱裂占34.6%左右,脑膨出占14.2%左右。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "NTDs" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "无脑畸形" }, { "start_idx": 61, "end_idx": 63, "type": "dis", "entity": "脊柱裂" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "脑膨出" } ] }, { "text": "而国际出生缺陷监测交换所19个国家和地区,以及欧洲共同体16个地区和澳大利亚新南威尔士则是脊柱裂占首位(均在50%以上),无脑畸形次之,脑膨出等更次之。", "entities": [ { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "脊柱裂" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "无脑畸形" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "脑膨出" } ] }, { "text": "许多统计资料也显示了NTDs的长期变化趋势,近几十年来NTDs发病率有逐渐下降的趋势,这个现象在世界上许多国家和地区都已观察到,例如英国和美国西北部NTDs在20世纪20年代有一个发病率高峰,到40年代下降,进入70年代又有所下降。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "NTDs" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "NTDs" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "NTDs" } ] }, { "text": "究其原因是广泛提高了孕期诊断技术而使NTDs发病率下降,并强烈显示出环境因素在NTDs发病中的作用,特别是多种维生素和叶酸的使用。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "NTDs" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "NTDs" } ] }, { "text": "另外,随着时间推移,NTDs临床医学和流行病学特征的改变,也提示了NTDs的病因异质性(不同临床表型之间存在的病因差异)。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "NTDs" }, { "start_idx": 14, "end_idx": 17, "type": "dep", "entity": "临床医学" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "NTDs" } ] }, { "text": "【病因与发病机制】研究NTDs病因,有必要搞清楚神经胚胎发育的正常过程。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "神经胚胎" } ] }, { "text": "在人类胚胎发育第16天脊索上方外胚层增厚形成神经板两侧神经板增高形成神经褶并向中线靠拢融合形成神经管神经管融合最初出现在第22天,位于第3体节水平,即未来脑干形成区域,融合是从视原基部位开始,并自脑干和视原基部位向头和尾两个方向发展。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "神经板" }, { "start_idx": 11, "end_idx": 24, "type": "sym", "entity": "脊索上方外胚层增厚形成神经板" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "两侧神经板" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "神经褶" }, { "start_idx": 25, "end_idx": 42, "type": "sym", "entity": "两侧神经板增高形成神经褶并向中线靠拢" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "神经管" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "融合形成神经管" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "神经管" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "脑干" }, { "start_idx": 98, "end_idx": 99, "type": "bod", "entity": "脑干" } ] }, { "text": "前神经管于胚胎发育第23~26天闭合,若闭合不全则形成颅裂及无脑畸形。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "前神经管" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "颅裂" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "无脑畸形" } ] }, { "text": "而后神经管于胚胎发育第26~30天闭合,若闭合不全则形成脊柱裂。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "神经管" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "脊柱裂" } ] }, { "text": "NTDs是一种多基因遗传病,其病因是极其复杂的,发病原因可能是多方面的,许多因素的干扰都会影响发病过程,根据动物实验、临床观察和流行病学研究认为NTDs是遗传因素和环境因素(子宫内环境)共同作用的结果。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "NTDs" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "多基因遗传病" }, { "start_idx": 72, "end_idx": 75, "type": "dis", "entity": "NTDs" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "子宫" } ] }, { "text": "(一)遗传因素在NTDs病因研究中,很难把多基因或多因素作用与复杂的环境因素作用区别开来,如某些家族可能与共同生活的环境有关。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "NTDs" } ] }, { "text": "这样,在病因中通常把某些特征归结为基因因素作用的结果,诸如不同地区和种族人群NTDs发病率的改变,近亲婚配NTDs发病率高,NTDs家族内的复发危险度高等。", "entities": [ { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "NTDs" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "NTDs" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "NTDs" } ] }, { "text": "家系研究表明,有NTDs家族史的孕妇,其NTDs婴儿的出生概率比一般人群高。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "NTDs" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "NTDs" } ] }, { "text": "有过2次及以上NTDs生育经历的母亲再生育NTDs婴儿的危险性则提高10%。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "NTDs" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "NTDs" } ] }, { "text": "另外,双胎中NTDs的发病率比一般人群要高,且单卵双胎又比双卵双胎NTDs发病率高。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "NTDs" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "NTDs" } ] }, { "text": "这些研究结果均支持遗传因素对NTDs的作用。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "NTDs" } ] }, { "text": "有关NTDs的遗传学研究结果不能用孟德尔遗传法则的单基因突变来解释,而是有多对基因遗传的基础,即微效基因。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "NTDs" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "单基因突变" } ] }, { "text": "(二)环境因素环境致畸因子在妊娠早期,通常在3个月内作用于母体,导致神经管发育过程中出现障碍而发生畸形。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "妊娠" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "神经管" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "畸形" } ] }, { "text": "研究较多的是母亲孕早期叶酸和其他多种维生素(包括维生素A、B1、B2、C、D、E以及尼克酸等)的缺乏与NTDs发生的关系,尤其是叶酸与NTDs关系自80年代以来已取得突破性进展,现已确定妇女怀孕早期叶酸缺乏是NTDs发生的主要原因。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "叶酸" }, { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "维生素" }, { "start_idx": 24, "end_idx": 27, "type": "ite", "entity": "维生素A" }, { "start_idx": 29, "end_idx": 30, "type": "ite", "entity": "B1" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "B2" }, { "start_idx": 47, "end_idx": 47, "type": "ite", "entity": "C" }, { "start_idx": 49, "end_idx": 49, "type": "ite", "entity": "D" }, { "start_idx": 51, "end_idx": 51, "type": "ite", "entity": "E" }, { "start_idx": 54, "end_idx": 56, "type": "ite", "entity": "尼克酸" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "NTDs" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "叶酸" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "NTDs" }, { "start_idx": 116, "end_idx": 119, "type": "dis", "entity": "NTDs" } ] }, { "text": "摄入不足、吸收不良、代谢障碍或需要增加等多种原因都可导致叶酸缺乏,致使DNA合成障碍,从而影响细胞分裂与增殖。", "entities": [ { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "叶酸缺乏" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "DNA" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "细胞" } ] }, { "text": "叶酸是一种水溶性维生素,是胎儿早期神经发育必需的一种营养物质。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "叶酸" } ] }, { "text": "怀孕早期正值胚胎分化以及胎盘形成阶段,细胞生长和分裂十分旺盛,如果孕妇叶酸缺乏,将影响胎儿神经系统雏形——神经管的正常发育,以后则将使颅骨或脊椎骨融合不良,出现NTDs,而引起自发性流产和死胎。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "细胞" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "叶酸缺乏" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "神经系统" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "神经管" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "NTDs" }, { "start_idx": 88, "end_idx": 92, "type": "sym", "entity": "自发性流产" } ] }, { "text": "其他微量元素如铜、钙和硒等的摄入不足也可诱发NTDs,但其确切作用尚不清楚。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "NTDs" } ] }, { "text": "病毒学研究表明,母亲孕早期感染巨细胞病毒或A型流感病毒胎儿中枢神经系统发育障碍NTDs。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "mic", "entity": "巨细胞病毒" }, { "start_idx": 21, "end_idx": 26, "type": "mic", "entity": "A型流感病毒" }, { "start_idx": 13, "end_idx": 26, "type": "sym", "entity": "感染巨细胞病毒或A型流感病毒" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 27, "end_idx": 38, "type": "sym", "entity": "胎儿中枢神经系统发育障碍" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "NTDs" } ] }, { "text": "母亲孕早期腹部或盆腔接受射线照射,其胎儿中枢神经系统发育往往受到影响,有的发生NTDs。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "腹部" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "盆腔" }, { "start_idx": 20, "end_idx": 25, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "NTDs" } ] }, { "text": "患有癫痫的孕妇服用丙戊酸、苯巴比妥和苯妥英钠等抗癫痫药物,其后代容易发生NTDs。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "癫痫" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "丙戊酸" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "苯妥英钠" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "NTDs" } ] }, { "text": "孕早期口服避孕药,服用某些抗肿瘤药物如氨甲蝶呤、维生素B4和巯基嘌呤等,以及大量或持续应用可的松或泼尼松龙,均可以诱发NTDs。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "避孕药" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "氨甲蝶呤" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "维生素B4" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "巯基嘌呤" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "可的松" }, { "start_idx": 55, "end_idx": 58, "type": "dru", "entity": "泼尼松龙" }, { "start_idx": 65, "end_idx": 68, "type": "dis", "entity": "NTDs" } ] }, { "text": "【临床表现】NTDs是一组具有多种不同临床表型的先天畸形,主要包括无脑畸形、脑膨出及脊柱裂等。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "NTDs" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "先天畸形" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "无脑畸形" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "脑膨出" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "脊柱裂" } ] }, { "text": "现将常见的几种NTDs分述如下。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "NTDs" } ] }, { "text": "(一)无脑畸形无脑畸形(anencephaly)是一种严重的NTDs,为脑的全部或大部缺如。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "无脑畸形" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "无脑畸形" }, { "start_idx": 12, "end_idx": 22, "type": "dis", "entity": "anencephaly" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "NTDs" }, { "start_idx": 36, "end_idx": 44, "type": "dis", "entity": "脑的全部或大部缺如" } ] }, { "text": "头颅的缺损从顶部开始,可延伸到其与枕骨大孔的任何部位。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "头颅" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "枕骨大孔" } ] }, { "text": "患儿因颅骨穹隆缺如造成面部特殊外貌前颅窝缩短和眼眶变浅眼球向前突出下颌紧贴胸骨口半张开,耳廓很厚,前突出于头的两侧呈非常奇特的“蛙状脸”。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "颅骨穹隆缺如" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "面部" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "面部特殊外貌" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "前颅窝" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "眼眶" }, { "start_idx": 17, "end_idx": 26, "type": "sym", "entity": "前颅窝缩短和眼眶变浅" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "眼球" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "眼球向前突出" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "下颌" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "下颌紧贴胸骨" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "口半张开" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "头" }, { "start_idx": 49, "end_idx": 56, "type": "sym", "entity": "前突出于头的两侧" }, { "start_idx": 57, "end_idx": 67, "type": "sym", "entity": "呈非常奇特的“蛙状脸”" } ] }, { "text": "几乎都伴有母体羊水过多颅裂、脑膜膨出与脑脑膜膨出颅裂纯属先天颅骨发育异常,表现为颅缝闭合不全而遗有缺损,形成一个缺口。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "羊水" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "母体羊水过多" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "颅裂" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "脑膜膨出" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "脑脑膜膨出" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "颅裂" } ] }, { "text": "一般多发生在颅盖骨或颅底骨的中线,少数偏于一侧。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "颅盖骨" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "颅底骨的中线" } ] }, { "text": "如果从裂孔处无脑膜或脑组织膨出,则称为隐性颅裂。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "隐性颅裂" } ] }, { "text": "反之,在颅裂的基础上,有脑膜或脑组织膨出则称囊性(显性)颅裂,其中囊内容物仅为脑脊液者称为脑膜膨出;囊内容物含有脑组织者称为脑脑膜膨出。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "颅裂" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "囊性(显性)颅裂" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "脑脊液" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "脑膜膨出" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "脑组织" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "脑脑膜膨出" } ] }, { "text": "其实脑膜膨出和脑脑膜膨出是脑膨出中最多见的两种类型。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "脑膜膨出" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "脑脑膜膨出" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "脑膨出" } ] }, { "text": "隐性颅裂在临床上多无症状,大多在做头颅X线检查时偶然发现,可见边缘光滑的颅骨缺损神经受损症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "隐性颅裂" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "头颅X线检查" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "颅骨缺损" }, { "start_idx": 31, "end_idx": 39, "type": "sym", "entity": "边缘光滑的颅骨缺损" }, { "start_idx": 40, "end_idx": 45, "type": "sym", "entity": "神经受损症状" } ] }, { "text": "囊性颅裂的脑膜膨出或脑脑膜膨出,可以有以下三方面的表现①局部症状:一般多为圆形或椭圆形的囊性膨出包块,如位于鼻根多为扁平状包块,其大小各异,大者近似儿头,小者直径可几厘米,有的生后即很大,有的逐渐长大厚者触之软而有弹性感,有的表面似有瘢痕状而较硬。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "囊性颅裂" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "脑膜膨出" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "脑脑膜膨出" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "鼻根" }, { "start_idx": 33, "end_idx": 99, "type": "sym", "entity": "一般多为圆形或椭圆形的囊性膨出包块,如位于鼻根多为扁平状包块,其大小各异,大者近似儿头,小者直径可几厘米,有的生后即很大,有的逐渐长大" }, { "start_idx": 100, "end_idx": 122, "type": "sym", "entity": "厚者触之软而有弹性感,有的表面似有瘢痕状而较硬" } ] }, { "text": "其基底部可为细的带状或为广阔基底。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "其基底部可为细的带状或为广阔基底" } ] }, { "text": "有的可触及骨缺损的边缘。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "骨缺损" } ] }, { "text": "囊性包块透光试验阳性,在脑脑膜膨出时有可能见到膨出的脑组织阴影。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "脑脑膜膨出" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "脑组织" } ] }, { "text": "如发生在鼻根部时,可一侧或双侧嗅觉丧失,如膨出突入眶内Ⅱ、Ⅲ、Ⅳ、Ⅵ颅神经及第Ⅴ颅神经的第一支受累。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "鼻根部" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "一侧或双侧嗅觉丧失" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "眶内" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "膨出突入眶内" }, { "start_idx": 27, "end_idx": 48, "type": "sym", "entity": "Ⅱ、Ⅲ、Ⅳ、Ⅵ颅神经及第Ⅴ颅神经的第一支受累" } ] }, { "text": "如发生在枕部的脑脑膜膨出,可有皮质盲及小脑受损症状器官的受压表现:膨出位于鼻根部者,常引起颜面畸形,鼻根扁宽,眼距加大,眶腔变小,有时眼睛呈三角形,双眼球被挤向外侧,可累及泪腺致泪囊炎。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "枕部" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "脑脑膜膨出" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "小脑" }, { "start_idx": 15, "end_idx": 24, "type": "sym", "entity": "皮质盲及小脑受损症状" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "器官" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "鼻根部" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "颜面畸形" } ] }, { "text": "突入鼻腔可影响呼吸或侧卧时才呼吸通畅。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "鼻腔" } ] }, { "text": "膨出突入眶内时,可致眼球突出及移位脊柱裂、脊膜膨出与脊髓脊膜膨出脊柱裂是胚胎早期椎弓发育障碍,椎管闭合不全。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "眶内" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "眼球" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "眼球突出及移位" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "脊柱裂" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "脊膜膨出" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "脊髓脊膜膨出" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "脊柱裂" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "椎弓发育障碍" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "椎管闭合不全" } ] }, { "text": "可发生在脊椎的任何部位,如颈椎、胸椎、腰椎和骶椎,以腰骶部最常见。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "脊椎" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "颈椎" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "胸椎" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "腰椎" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "骶椎" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "腰骶部" } ] }, { "text": "脊柱裂分为隐性与显性两类,前者是指有脊柱裂而无椎管内容物膨出;后者由于椎板闭合不全,椎管内容物通过缺损处向椎管外膨出,在背部皮下形成囊性包块。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脊柱裂" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脊柱裂" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "无椎管内容物膨出" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "椎板闭合不全" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "椎管" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "背部" } ] }, { "text": "显性脊柱裂又称为囊性脊柱裂,以脊膜膨出和脊髓脊膜膨出两种类型为多见,其中脊膜膨出的囊内充满脑脊液,无神经组织;而脊髓脊膜膨出的囊内既有脑脊液又有脊髓及其脊神经突入隐性脊柱裂大部分临床上无症状,大多是在X线检查中无意发现的,可见脊椎椎板缺损未闭合下肢无力、遗尿或大小便失禁等,缘于神经根与裂孔处有纤维带粘连或压迫所致。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "显性脊柱裂" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "囊性脊柱裂" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "脊膜膨出" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "脊髓脊膜膨出" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "脊膜膨出" }, { "start_idx": 56, "end_idx": 61, "type": "dis", "entity": "脊髓脊膜膨出" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "脑脊液" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "脊髓" }, { "start_idx": 76, "end_idx": 80, "type": "bod", "entity": "脊神经突入" }, { "start_idx": 63, "end_idx": 80, "type": "sym", "entity": "囊内既有脑脊液又有脊髓及其脊神经突入" }, { "start_idx": 81, "end_idx": 85, "type": "dis", "entity": "隐性脊柱裂" }, { "start_idx": 100, "end_idx": 103, "type": "pro", "entity": "X线检查" }, { "start_idx": 113, "end_idx": 116, "type": "bod", "entity": "脊椎椎板" }, { "start_idx": 113, "end_idx": 121, "type": "sym", "entity": "脊椎椎板缺损未闭合" }, { "start_idx": 122, "end_idx": 125, "type": "sym", "entity": "下肢无力" }, { "start_idx": 127, "end_idx": 128, "type": "sym", "entity": "遗尿" }, { "start_idx": 130, "end_idx": 134, "type": "sym", "entity": "大小便失禁" } ] }, { "text": "脊膜膨出和脊髓脊膜膨出的临床表现分为三个方面:①局部包块:患儿出生时,在背部中线颈、胸或腰骶部可见一大小不等的囊性包块,呈圆形或椭圆形,多数基底较宽,少数为带状表面皮肤正常,也有时为瘢痕样,而且菲薄婴儿哭闹时包块膨大,压迫包块则前囟门膨隆,显示膨出包块与蛛网膜下腔相通包块透光试验,单纯的脊膜膨出,透光程度高,而内含脊髓与神经根者,可见包块内有阴影。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "脊膜膨出" }, { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "脊髓脊膜膨出" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "局部包块" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "背部" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "胸" }, { "start_idx": 35, "end_idx": 79, "type": "sym", "entity": "在背部中线颈、胸或腰骶部可见一大小不等的囊性包块,呈圆形或椭圆形,多数基底较宽,少数为带状" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "皮肤" }, { "start_idx": 80, "end_idx": 98, "type": "sym", "entity": "表面皮肤正常,也有时为瘢痕样,而且菲薄" }, { "start_idx": 114, "end_idx": 116, "type": "bod", "entity": "前囟门" }, { "start_idx": 127, "end_idx": 131, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 99, "end_idx": 133, "type": "sym", "entity": "婴儿哭闹时包块膨大,压迫包块则前囟门膨隆,显示膨出包块与蛛网膜下腔相通" }, { "start_idx": 134, "end_idx": 139, "type": "ite", "entity": "包块透光试验" }, { "start_idx": 144, "end_idx": 147, "type": "dis", "entity": "脊膜膨出" }, { "start_idx": 149, "end_idx": 153, "type": "sym", "entity": "透光程度高" }, { "start_idx": 158, "end_idx": 159, "type": "bod", "entity": "脊髓" }, { "start_idx": 161, "end_idx": 163, "type": "bod", "entity": "神经根" }, { "start_idx": 166, "end_idx": 173, "type": "sym", "entity": "可见包块内有阴影" } ] }, { "text": "②神经损害症状:单纯的脊膜膨出,可以无神经系统症状。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "神经损害症状" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "脊膜膨出" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "神经系统" } ] }, { "text": "③其他症状:少数脊膜膨出向胸腔、腹腔以及盆腔内伸长,出现包块及压迫内脏的症状脊膜膨出患儿合并脑积水和其他畸形,出现相应症状。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胸腔" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "腹腔" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "盆腔" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "内脏" }, { "start_idx": 6, "end_idx": 37, "type": "sym", "entity": "少数脊膜膨出向胸腔、腹腔以及盆腔内伸长,出现包块及压迫内脏的症状" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "脊膜膨出" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "脑积水" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "畸形" } ] }, { "text": "【诊断】无脑畸形患儿的临床表现一目了然,无需做CT或MRI检查即可进行临床诊断。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "无脑畸形" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "CT" }, { "start_idx": 26, "end_idx": 30, "type": "pro", "entity": "MRI检查" } ] }, { "text": "但CT或MRI检查对产前诊断是有帮助的,特别是MRI,可显示宫内胎儿颅脑发育情况,如颅盖缺如脑组织缺如羊水过多。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "CT" }, { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "MRI检查" }, { "start_idx": 23, "end_idx": 25, "type": "pro", "entity": "MRI" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "宫内" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "颅脑" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "颅盖" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "颅盖缺如" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "脑组织" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "脑组织缺如" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "羊水过多" } ] }, { "text": "头颅X线片可发现有大小不等的颅骨缺损头颅CT平扫可显示颅骨缺损脑脊液同样密度的囊性肿物,如合并脑脑膜膨出则可见囊内有脑组织密度影。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "头颅X线片" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "颅骨" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "颅骨缺损" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "头颅CT平扫" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "颅骨" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "颅骨缺损" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "脑脊液" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "脑脑膜膨出" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "脑组织" } ] }, { "text": "头颅CT增强扫描可显示囊内脑组织强化,囊与蛛网膜下腔相交通头颅MRI扫描可见颅骨缺损及由此膨出的脑脊液、脑组织、脑血管及硬脑膜组织信号的肿物,并可见颅内其他结构的改变及畸形。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "头颅CT" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "脑组织" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 11, "end_idx": 28, "type": "sym", "entity": "囊内脑组织强化,囊与蛛网膜下腔相交通" }, { "start_idx": 29, "end_idx": 35, "type": "pro", "entity": "头颅MRI扫描" } ] }, { "text": "脊柱X线片显示椎板棘突缺如,椎弓根间距增宽,骨质缺损部位与软组织肿物相连接CT及MRI扫描可发现脊髓、脊神经及脊膜的膨出情况,以及局部粘连等病变。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "脊柱X线片" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "椎板棘突" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "椎弓根" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "软组织" }, { "start_idx": 7, "end_idx": 36, "type": "sym", "entity": "椎板棘突缺如,椎弓根间距增宽,骨质缺损部位与软组织肿物相连接" }, { "start_idx": 37, "end_idx": 38, "type": "pro", "entity": "CT" }, { "start_idx": 40, "end_idx": 44, "type": "pro", "entity": "MRI扫描" }, { "start_idx": 48, "end_idx": 71, "type": "sym", "entity": "脊髓、脊神经及脊膜的膨出情况,以及局部粘连等病变" } ] }, { "text": "【治疗】无脑畸形患儿不能外科手术治疗。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "无脑畸形" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "外科手术" } ] }, { "text": "单纯颅裂一般无需特殊治疗。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "颅裂" } ] }, { "text": "当合并脑膜膨出或脑脑膜膨出时,一般均需手术治疗。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "脑膜膨出" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "脑脑膜膨出" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "手术" } ] }, { "text": "手术时间过去认为在生后6~12个月为宜,近年来主张尽早手术,可在生后数天或数周内进行。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "手术" } ] }, { "text": "手术目的是切除膨出的囊,还纳膨出的脑组织等内容物,封闭颅骨缺损巨大脑膜膨出或脑脑膜膨出,合并神经系统症状,智力低下及明显的脑积水者,无需手术治疗。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 10, "end_idx": 10, "type": "sym", "entity": "囊" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "脑组织" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "颅骨" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "颅骨缺损" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "巨大脑膜膨出" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "脑脑膜膨出" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "神经系统" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "智力低下" }, { "start_idx": 61, "end_idx": 63, "type": "dis", "entity": "脑积水" }, { "start_idx": 68, "end_idx": 69, "type": "pro", "entity": "手术" } ] }, { "text": "脊髓脊膜膨出手术时,通常需要向上、向下扩大椎板切开范围,以探查脊髓与神经向脊膜囊内膨出的情况,有利于膨出的神经组织还纳于椎管内。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "脊髓脊膜膨出" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "椎板" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "脊髓" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "神经" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "神经组织" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "椎管" } ] }, { "text": "【预防】(一)增补叶酸迄今为止,对NTDs的研究已经历了30多年之久,积累了丰富的临床医学、流行病学及遗传学等方面的资料,特别是对叶酸预防NTDs的研究取得了突破性进展。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "叶酸" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "NTDs" }, { "start_idx": 41, "end_idx": 44, "type": "dep", "entity": "临床医学" }, { "start_idx": 46, "end_idx": 49, "type": "dep", "entity": "流行病学" }, { "start_idx": 51, "end_idx": 53, "type": "dep", "entity": "遗传学" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "叶酸" }, { "start_idx": 69, "end_idx": 72, "type": "dis", "entity": "NTDs" } ] }, { "text": "妇女怀孕早期体内叶酸缺乏是NTDs发生的主要原因;妇女如果能在怀孕前和怀孕早期及时增补叶酸,便可有效地预防大部分NTDs的发生。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "叶酸缺乏" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "NTDs" }, { "start_idx": 43, "end_idx": 44, "type": "dru", "entity": "叶酸" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "NTDs" } ] }, { "text": "近年来研究发现5,10-亚甲基四氢叶酸还原酶(MTHFR)是叶酸代谢过程中的一种关键酶,MTHFR的酶活性降低,可引起体内一种胚胎毒性物质同型半胱氨酸蓄积,可能是NTDs发生的危险因素,而妇女在怀孕前和怀孕早期服用叶酸可以弥补MTHFR酶活性的缺陷,使体内的5-甲基四氢叶酸(活性叶酸)增多,达到预防NTDs发生之目的。", "entities": [ { "start_idx": 7, "end_idx": 21, "type": "bod", "entity": "5,10-亚甲基四氢叶酸还原酶" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "MTHFR" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "叶酸" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "酶" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "MTHFR" }, { "start_idx": 81, "end_idx": 84, "type": "dis", "entity": "NTDs" }, { "start_idx": 107, "end_idx": 108, "type": "dru", "entity": "叶酸" }, { "start_idx": 113, "end_idx": 117, "type": "bod", "entity": "MTHFR" }, { "start_idx": 129, "end_idx": 136, "type": "bod", "entity": "5-甲基四氢叶酸" }, { "start_idx": 138, "end_idx": 141, "type": "bod", "entity": "活性叶酸" }, { "start_idx": 150, "end_idx": 153, "type": "dis", "entity": "NTDs" } ] }, { "text": "我国卫生部决定从1995年10月起,在全国实施妇女增补叶酸预防NTDs工作。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "dru", "entity": "叶酸" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "NTDs" } ] }, { "text": "至于我国育龄妇女体内叶酸缺乏的主要原因是膳食中摄入叶酸量较少,富含叶酸的食物如绿色蔬菜和水果等摄入量不足,另外由于烹调习惯的关系,使得食物中的叶酸大部分受到破坏。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "叶酸缺乏" }, { "start_idx": 25, "end_idx": 26, "type": "dru", "entity": "叶酸" }, { "start_idx": 33, "end_idx": 34, "type": "dru", "entity": "叶酸" }, { "start_idx": 71, "end_idx": 72, "type": "dru", "entity": "叶酸" } ] }, { "text": "需要指出的是,不能用普通的药用叶酸代替,因为那种片剂每片含叶酸5mg(5000μg),如果天天服用,由于剂量过大,可能反而影响胎儿发育。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "药用叶酸" }, { "start_idx": 29, "end_idx": 30, "type": "dru", "entity": "叶酸" } ] }, { "text": "另一类为复合制剂,专为孕产妇设计,其中不仅包含叶酸,同时还含有多种维生素、矿物质和微量元素。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "dru", "entity": "叶酸" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "维生素" } ] }, { "text": "这类制剂能够补充孕、产妇的全面营养,除能预防NTDs外,还有利于胎儿和婴儿营养需求,同时也有利于母婴的均衡营养。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "NTDs" } ] }, { "text": "(二)遗传咨询NTDs的遗传咨询主要包括婚前、孕前及孕期咨询,了解夫妇双方的家族史、孕妇既往的妊娠史、此次妊娠的饮食、服药情况和接触放射线、有害化学物质以及致病微生物等情况,对孕妇进行卫生保健以减少NTDs患儿的发生。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "NTDs" }, { "start_idx": 99, "end_idx": 102, "type": "dis", "entity": "NTDs" } ] }, { "text": "(三)产前检查NTDs的产前检查内容主要包括羊水、母亲血清甲胎蛋白(AFP)检测及B超检查等。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "NTDs" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "B超检查" } ] }, { "text": "B超可扫描胎儿的头颅形状及大小或脊柱部位的结构,如无脑畸形胎儿表现为胎头圆形光环消失,脑膜膨出胎儿在胎头的颅骨壁缺损处可见一囊性肿物与其连接,脊柱裂胎儿可发现脊柱裂口,脊膜膨出胎儿可在脊椎部发现边界规则而清晰的囊性膨出物等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "B超" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "头颅" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "脊柱" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "无脑畸形" }, { "start_idx": 34, "end_idx": 41, "type": "sym", "entity": "胎头圆形光环消失" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "脑膜膨出" }, { "start_idx": 50, "end_idx": 69, "type": "sym", "entity": "胎头的颅骨壁缺损处可见一囊性肿物与其连接" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "脊柱裂" }, { "start_idx": 79, "end_idx": 82, "type": "sym", "entity": "脊柱裂口" }, { "start_idx": 84, "end_idx": 87, "type": "dis", "entity": "脊膜膨出" }, { "start_idx": 92, "end_idx": 109, "type": "sym", "entity": "脊椎部发现边界规则而清晰的囊性膨出物" } ] }, { "text": "一旦产前检查发现胎儿NTDs,应立即终止妊娠,以减少NTDs患儿出生。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "NTDs" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "NTDs" } ] }, { "text": "【预后】无脑畸形患儿预后极差,绝大多数于出生时即为死胎,仅25%的患儿为活产,但极少能存活一周,多数于生后数小时死亡。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "无脑畸形" } ] }, { "text": "单纯的脑膜膨出,经过手术治疗后,一般效果较好,可减少或缓解神经系统的损害症状,降低死亡率。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "脑膜膨出" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "手术" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "神经系统" } ] }, { "text": "单纯的脊膜膨出,手术治疗疗效好,可以达到临床治愈。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "脊膜膨出" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "手术" } ] }, { "text": "三、新生儿出血症新生儿出血症(hemorrhagicdiseaseofthenewborn,HDN)为维生素K依赖的凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ减少而引起的出血,又称新生儿自然出血症、维生素K依赖性出血症、新生儿低凝血酶原血症。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "新生儿出血症" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "新生儿出血症" }, { "start_idx": 15, "end_idx": 44, "type": "dis", "entity": "hemorrhagicdiseaseofthenewborn" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "HDN" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "维生素K" }, { "start_idx": 58, "end_idx": 68, "type": "bod", "entity": "凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ" }, { "start_idx": 75, "end_idx": 76, "type": "sym", "entity": "出血" }, { "start_idx": 80, "end_idx": 87, "type": "dis", "entity": "新生儿自然出血症" }, { "start_idx": 89, "end_idx": 98, "type": "dis", "entity": "维生素K依赖性出血症" }, { "start_idx": 100, "end_idx": 109, "type": "dis", "entity": "新生儿低凝血酶原血症" } ] }, { "text": "【病因与发病机制】病因为维生素K缺乏。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "维生素K" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "维生素K缺乏" } ] }, { "text": "维生素K缺乏的原因有:①摄入不足:孕母维生素K只有10%可通过胎盘达到胎儿,胎儿维生素K贮量少,母乳中维生素K的含量(15μg/L)仅为牛奶(60μg/L)的1/4,初生母乳量又不足,母乳喂养者发病率较牛奶喂养者高15~20倍。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "维生素K" }, { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "维生素K缺乏" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "维生素K" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "胎盘" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "维生素K" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "维生素K" } ] }, { "text": "②合成不足:维生素K主要由正常肠道菌群合成,初生新生儿肠道菌群尚未建立,影响维生素K的合成。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "维生素K" }, { "start_idx": 27, "end_idx": 30, "type": "mic", "entity": "肠道菌群" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "维生素K" } ] }, { "text": "抗生素抑制肠道菌群,使维生素K合成不足。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "抗生素" }, { "start_idx": 5, "end_idx": 8, "type": "mic", "entity": "肠道菌群" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "维生素K" } ] }, { "text": "凝血因子Ⅱ、Ⅻ、Ⅸ、Ⅹ的谷氨酸残基需要羧化为γ-羧基谷氨酸,增加钙结合位点,才具有凝血活性。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "bod", "entity": "凝血因子Ⅱ、Ⅻ、Ⅸ、Ⅹ" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "谷氨酸" }, { "start_idx": 19, "end_idx": 28, "type": "bod", "entity": "羧化为γ-羧基谷氨酸" } ] }, { "text": "这一羧化过程需要维生素K的参与,如发生维生素K缺乏早发性出血比较少见,生后24小时内发病,与孕母用药有关,如抗凝药(双香豆素)、抗癫痫药(苯妥英钠、苯巴比妥)及抗结核药(利福平、异烟肼)等,这些药物可干扰胎儿维生素K的功能。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "维生素K" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "维生素K" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "维生素K缺乏" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "早发性出血" }, { "start_idx": 54, "end_idx": 56, "type": "dru", "entity": "抗凝药" }, { "start_idx": 58, "end_idx": 61, "type": "dru", "entity": "双香豆素" }, { "start_idx": 64, "end_idx": 67, "type": "dru", "entity": "抗癫痫药" }, { "start_idx": 69, "end_idx": 72, "type": "dru", "entity": "苯妥英钠" }, { "start_idx": 74, "end_idx": 77, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 80, "end_idx": 83, "type": "dru", "entity": "抗结核药" }, { "start_idx": 85, "end_idx": 87, "type": "dru", "entity": "利福平" }, { "start_idx": 89, "end_idx": 91, "type": "dru", "entity": "异烟肼" }, { "start_idx": 104, "end_idx": 107, "type": "bod", "entity": "维生素K" } ] }, { "text": "出血程度轻重不一,出血部位除皮肤外,也可有颅内、胸腔或腹腔出血。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "皮肤" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "胸腔" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "腹腔" } ] }, { "text": "2.典型的新生儿出血症近年已较少见,生后2~7天发病,早产儿可迟至2周。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "新生儿出血症" } ] }, { "text": "多见于母乳喂养儿,出血部位以胃肠道(便血和呕血)最常见,其他有脐带残端、皮肤、帽状腱膜下、颅内、注射部位或手术伤口的渗血等,早产儿可发生颅内出血。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "胃肠道" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "便血" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "呕血" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "脐带残端" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "皮肤" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "帽状腱膜下" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "颅内" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "颅内" }, { "start_idx": 68, "end_idx": 71, "type": "sym", "entity": "颅内出血" } ] }, { "text": "出血程度轻重不等,但有些轻度出血可为严重致命出血(如颅内出血)的前驱症状,少数病例可发生消化道或脐端大出血导致休克。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "出血" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "轻度出血" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "严重致命出血" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "颅内出血" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "消化道" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "脐端" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "脐端大出血" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "休克" } ] }, { "text": "3.晚发性维生素K缺乏出血生后2周~3个月发病,发生率约为1/1万~10/1万活产儿。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "晚发性维生素K缺乏出血" } ] }, { "text": "此外,与肝胆疾病、腹泻、使用广谱抗生素(抑制肠道菌群)、长期禁食或静脉营养时未补充维生素K有关。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肝胆" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "腹泻" }, { "start_idx": 14, "end_idx": 18, "type": "dru", "entity": "广谱抗生素" }, { "start_idx": 22, "end_idx": 25, "type": "mic", "entity": "肠道菌群" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "维生素K" } ] }, { "text": "出血部位主要为颅内出血,约占60%~80%。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "颅内" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "颅内出血" } ] }, { "text": "患儿出现惊厥、嗜睡、昏迷、前囟隆起脑疝,瞳孔固定、不等大,病死率高,后遗症多。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "惊厥" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "嗜睡" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "昏迷" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "前囟" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "前囟隆起" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "脑疝" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "瞳孔固定" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "不等大" } ] }, { "text": "【诊断与鉴别诊断】健康新生儿生后2~5天发生自然出血现象,血小板和出血时间正常,可考虑本病,若凝血酶原时间和部分凝血活酶时间延长、凝血时间正常或轻度延长,但出血时间正常,则可确诊。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "血小板" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "凝血活酶" } ] }, { "text": "注射维生素K1或输新鲜血浆等治疗有效,可为辅助诊断。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "新鲜血浆" } ] }, { "text": "1.胃肠道出血应与咽下综合征、应激性溃疡、消化道畸形和感染引起的消化道出血鉴别,这些患儿无凝血障碍。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "胃肠道" }, { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "胃肠道出血" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "咽下综合征" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "应激性溃疡" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "消化道畸形" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "感染" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "消化道出血" } ] }, { "text": "咽下综合征是新生儿出生时咽下母亲产道的血液或带血的羊水等,于生后不久即发生呕吐,呕吐物呈棕色,也可有血便,但血量均微,洗胃后可止吐。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "咽下综合征" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血液" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "血便" } ] }, { "text": "另外,碱变性试验(Apt试验)有助于鉴别母血及儿血,取1份呕吐物加水5份,混匀后离心,取上清液5份加1%氢氧化钠(0.25N)1份,混匀后静置2分钟,上清液仍为粉红色,说明血中含较多胎儿血红蛋白(HbF),出血来自新生儿;如上清液转变为棕黄色,则是吞入的母血。", "entities": [ { "start_idx": 93, "end_idx": 96, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 98, "end_idx": 100, "type": "bod", "entity": "HbF" } ] }, { "text": "因为新生儿Hb的80%~90%为FHb,成人的Hb97%为成人血红蛋白(HbA),HbF具有抗碱作用。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "Hb" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "HbA" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "HbF" } ] }, { "text": "2.产伤性出血多发生于分娩的先露部位,生后即出现。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "产伤性出血" } ] }, { "text": "3.其他如脐部出血应与脐带接扎不紧、脐部感染或肉芽肿等所致出血鉴别。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "脐部" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脐带" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "脐部感染" }, { "start_idx": 23, "end_idx": 25, "type": "pro", "entity": "肉芽肿" } ] }, { "text": "遗传性毛细血管扩张症可在新生儿早期发生消化道出血。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "遗传性毛细血管扩张症" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "消化道出血" } ] }, { "text": "【预防】出生后常规给维生素K11mg肌注1次,可有效防止本病的发生。", "entities": [ { "start_idx": 10, "end_idx": 20, "type": "dru", "entity": "维生素K1" } ] }, { "text": "母乳喂养者,哺乳母亲应多进食含维生素K丰富的食物,在生后2~3周再给新生儿注射维生素K15mg,以预防晚发性出血。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "维生素K" } ] }, { "text": "前几年曾认为注射维生素K会增加致癌几率,但经过临床对照研究这种观点已被否定。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "维生素K" } ] }, { "text": "【治疗】一旦怀疑本病,应立即给维生素K1治疗,治疗量为每次1~5mg缓慢静脉注射(1mg/min),注射速度过快可引起面色潮红、支气管痉挛、心动过速及血压下降等不良反应,静脉注射奏效最快,一般在注射后4小时内凝血酶原时间即可趋于正常。", "entities": [ { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "面色潮红" }, { "start_idx": 70, "end_idx": 74, "type": "sym", "entity": "支气管痉挛" }, { "start_idx": 76, "end_idx": 79, "type": "sym", "entity": "心动过速" }, { "start_idx": 81, "end_idx": 84, "type": "sym", "entity": "血压下降" } ] }, { "text": "应避免采用肌内注射,因易引起注射部位大量出血。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "注射部位大量出血" } ] }, { "text": "也可采用皮下注射,药物能被较快吸收,注射后可采用压迫止血。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "皮下注射" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "压迫止血" } ] }, { "text": "出血量较多的患儿,会导致急性失血性贫血和失血性休克,应立即给生理盐水纠正休克,同时根据患儿血红蛋白水平,给予输血,每次输新鲜血10~20ml/kg。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "急性失血性贫血" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "失血性休克" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "生理盐水" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "输血" } ] }, { "text": "轻者可输库存血浆以补充凝血因子。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血浆" } ] }, { "text": "早产儿肝功能不成熟,肝脏不能合成凝血因子,虽用维生素K1治疗,常不能迅速奏效,最好同时输新鲜血治疗。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "肝功能" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "肝功能不成熟" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肝脏" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "凝血因子" }, { "start_idx": 23, "end_idx": 35, "type": "pro", "entity": "维生素K1治疗" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "输新鲜血" } ] }, { "text": "脐部出血要做好包扎。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脐部" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "脐部出血" } ] }, { "text": "穿刺部位出血要压迫止血。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "穿刺部位" }, { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "穿刺部位出血" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "压迫止血" } ] }, { "text": "出血过多、治疗延误者可导致死亡,颅内出血者预后差,重者死亡,幸存者常留后遗症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "出血过多" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "颅内" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "颅内出血" } ] }, { "text": "五、幕上高度恶性胶质瘤幕上高度恶性胶质瘤相当于Kernohan分类中的Ⅲ、Ⅳ级,属于恶性程度很高的一类肿瘤。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "幕上高度恶性胶质瘤" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "幕上高度恶性胶质瘤" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "肿瘤" } ] }, { "text": "这类肿瘤占儿童神经系统肿瘤的7%~11%,主要位于大脑半球(66%),少部分位于间脑(20%)及后颅窝(14%),平均发病年龄为9~10岁。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "儿童神经系统肿瘤" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "大脑半球" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "间脑" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "后颅窝" } ] }, { "text": "有条件者亦可行立体定向放射治疗,如X-刀或γ-刀,可延长生存期。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "pro", "entity": "立体定向放射治疗" }, { "start_idx": 17, "end_idx": 19, "type": "pro", "entity": "X-刀" }, { "start_idx": 21, "end_idx": 23, "type": "pro", "entity": "γ-刀" } ] }, { "text": "化疗的作用和治疗方案的选择目前尚处于摸索阶段,应用价值还有争议。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" } ] }, { "text": "第十一章颅内出血颅内出血(intracranialhemorrhage,ICH)又称为出血性脑血管病或出血性卒中,系因脑血管破裂使血液外溢至颅腔所致。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "颅内出血" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "颅内出血" }, { "start_idx": 13, "end_idx": 34, "type": "dis", "entity": "intracranialhemorrhage" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "ICH" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "出血性脑血管病" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "出血性卒中" }, { "start_idx": 59, "end_idx": 63, "type": "sym", "entity": "脑血管破裂" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "血液外溢" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "颅腔" } ] }, { "text": "国外文献报道15岁以下儿童脑出血和蛛网膜下腔出血的发病率为2.5/10万。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "儿童脑出血" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "蛛网膜" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "蛛网膜下腔出血" } ] }, { "text": "无论何种原因所致的小儿ICH,其临床表现有颇多相似之处,但预后则视不同病因而有很大差异,且诊断与治疗是否及时也是直接影响预后的关键因素。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "小儿ICH" } ] }, { "text": "一、病因许多血液病、脑血管发育异常及颅内外其他病变均与小儿ICH的发生有关,其病因可以是单一的,亦可由多种病因联合所致。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "血液病" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "脑血管" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "脑血管发育异常" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "颅内外其他病变" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "小儿ICH" } ] }, { "text": "(一)脑血管畸形脑动静脉畸形是儿童时期ICH的常见原因之一,可分为先天性、感染性与外伤性。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "脑血管畸形" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "脑动静脉" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "脑动静脉畸形" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "ICH" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "先天性" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "感染性" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "外伤性" } ] }, { "text": "(二)血液病血液病是小儿脑血管病的重要病因,在尸检的血液病例中有50%发现自发性脑出血。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "血液病" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "血液病" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "小儿脑血管病" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "自发性脑出血" } ] }, { "text": "血友病患儿中2.2%~7.4%发生ICH。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "血友病" } ] }, { "text": "(三)颅内其他原因包括颅脑外伤,颅内肿瘤,脑动脉炎,中毒性脑病等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "颅内" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "颅脑外伤" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "脑动脉炎" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "中毒性脑病" } ] }, { "text": "(四)颅外其他原因包括维生素K缺乏症,维生素C缺乏症,肝病,高血压,感染或结缔组织病等其他各种原因所致的ICH。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "颅外" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "维生素C缺乏症" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "肝病" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "高血压" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "感染" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "ICH" } ] }, { "text": "(五)新生儿颅内出血原因新生儿颅内出血(neonatalintracranialhemorrhage,NICH)有其特殊的病因,主要发病因素为两大方面,即产伤及缺氧引起,前者正逐渐减少,后者有增加趋势。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "新生儿颅内出血" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "新生儿颅内出血" }, { "start_idx": 20, "end_idx": 49, "type": "dis", "entity": "neonatalintracranialhemorrhage" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "NICH" }, { "start_idx": 77, "end_idx": 78, "type": "sym", "entity": "产伤" }, { "start_idx": 80, "end_idx": 81, "type": "sym", "entity": "缺氧" } ] }, { "text": "NICH的发病率依不同的检测及统计方法不同而不同,其中在孕周<34周、出生体重<1500g的未成熟儿高达40%~50%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "NICH" } ] }, { "text": "(六)其他尚有部分小儿ICH的原因不明。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "小儿ICH" } ] }, { "text": "有文献报道尸检发现小儿特发性脑出血系由微小动脉瘤样血管畸形破裂所致,因此并非真正的原因不明。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "小儿特发性脑出血" }, { "start_idx": 19, "end_idx": 28, "type": "dis", "entity": "微小动脉瘤样血管畸形" } ] }, { "text": "只是因这种动脉瘤太小,用CT扫描和脑血管造影等神经影像学检查不能发现而已。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "CT扫描" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "脑血管造影" }, { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "影像学检查" } ] }, { "text": "第六节维生素B6缺乏症维生素B6有三种形式,即吡哆醇(pyridoxine,PN)、吡哆醛(pyridoxal,PA或PL)和吡哆胺(pyridoxamine,PM)。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "dru", "entity": "维生素B6" }, { "start_idx": 4, "end_idx": 21, "type": "dis", "entity": "维生素B6缺乏症" }, { "start_idx": 22, "end_idx": 31, "type": "dru", "entity": "维生素B6" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "吡哆醇" }, { "start_idx": 48, "end_idx": 57, "type": "dru", "entity": "pyridoxine" }, { "start_idx": 59, "end_idx": 60, "type": "dru", "entity": "PN" }, { "start_idx": 63, "end_idx": 65, "type": "dru", "entity": "吡哆醛" }, { "start_idx": 67, "end_idx": 75, "type": "dru", "entity": "pyridoxal" }, { "start_idx": 77, "end_idx": 78, "type": "dru", "entity": "PA" }, { "start_idx": 80, "end_idx": 81, "type": "dru", "entity": "PL" }, { "start_idx": 84, "end_idx": 86, "type": "dru", "entity": "吡哆胺" }, { "start_idx": 88, "end_idx": 99, "type": "dru", "entity": "pyridoxamine" }, { "start_idx": 101, "end_idx": 102, "type": "dru", "entity": "PM" } ] }, { "text": "这三种形式通过酶可互相转换。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "酶" } ] }, { "text": "PL及PM磷酸化后变为辅酶磷酸吡哆醛(PLP)及磷酸吡哆胺(PMP)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "PL" }, { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "磷酸吡哆醛" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "PLP" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "磷酸吡哆胺" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "PMP" } ] }, { "text": "吡哆醇为人工合成的产品,在植物中也有;在动物体内,多以辅酶PLP及PMP的形式存在。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "吡哆醇" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "辅酶PLP" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "PMP" } ] }, { "text": "【流行病学】1934年,Gyorgy首次证实吡哆醇即维生素B6,并于1938年阐明其化学结构与人工合成方法。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "吡哆醇" }, { "start_idx": 26, "end_idx": 35, "type": "dru", "entity": "维生素B6" } ] }, { "text": "原发性缺乏罕见,因为大多数食物中都含有维生素B6,但是人工喂养儿因配制奶中维生素B6缺乏可致病。", "entities": [ { "start_idx": 19, "end_idx": 28, "type": "dru", "entity": "维生素B6" }, { "start_idx": 47, "end_idx": 56, "type": "dru", "entity": "维生素B6" } ] }, { "text": "血液生化分析的结果显示维生素B6缺乏率约为12%~19%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "血液生化分析" }, { "start_idx": 11, "end_idx": 20, "type": "dru", "entity": "维生素B6" } ] }, { "text": "【病因】(一)膳食组成的影响因为5-磷酸吡哆醛氨基酸代谢中许多酶的辅酶,故蛋白质代谢需要维生素B6的参与,当膳食中蛋白质的摄入量高时,维生素B6的需要量也多,如以蛋白质摄入量为基础计算,摄取100g蛋白质,每天需摄入维生素B61.5~2.5mg。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "5-磷酸吡哆醛" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "酶" }, { "start_idx": 44, "end_idx": 58, "type": "dru", "entity": "维生素B6" } ] }, { "text": "(二)摄入不足婴儿由于母亲维生素B6摄入不足,引起乳汁中维生素B6的分泌量减少,或者人工喂养的婴儿,牛乳经过多次加热、煮沸,造成维生素B6的破坏,均可造成婴儿的维生素B6缺乏。", "entities": [ { "start_idx": 13, "end_idx": 22, "type": "dru", "entity": "维生素B6" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "乳汁" }, { "start_idx": 38, "end_idx": 47, "type": "dru", "entity": "维生素B6" }, { "start_idx": 84, "end_idx": 93, "type": "dru", "entity": "维生素B6" }, { "start_idx": 110, "end_idx": 119, "type": "dru", "entity": "维生素B6" }, { "start_idx": 110, "end_idx": 126, "type": "sym", "entity": "维生素B6缺乏" } ] }, { "text": "如小儿患结核、水痘、肺炎以及高热时,维生素B6的消耗增加,如未予及时补充,则造成维生素B6的缺乏。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "结核" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "水痘" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肺炎" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "高热" }, { "start_idx": 18, "end_idx": 27, "type": "dru", "entity": "维生素B6" }, { "start_idx": 50, "end_idx": 59, "type": "dru", "entity": "维生素B6" } ] }, { "text": "患甲状腺功能亢进时,维生素B6辅酶活力降低,维生素B6的需要量增加。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "甲状腺功能亢进" }, { "start_idx": 10, "end_idx": 26, "type": "bod", "entity": "维生素B6辅酶" }, { "start_idx": 32, "end_idx": 41, "type": "dru", "entity": "维生素B6" } ] }, { "text": "(四)药物影响异烟肼、环丝氨酸、L-多巴、肼苯达嗪、D-青霉胺、四环素等均可导致维生素B6缺乏。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "异烟肼" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "环丝氨酸" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "L-多巴" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "肼苯达嗪" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "D-青霉胺" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "四环素" } ] }, { "text": "异烟肼、肼苯达嗪与维生素B6形成非活性衍生物,加速了维生素B6排泄;青酶胺、环丝氨酸是维生素B6的抗代谢剂,均会加重维生素B6缺乏。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "异烟肼" }, { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "肼苯达嗪" }, { "start_idx": 54, "end_idx": 56, "type": "dru", "entity": "青酶胺" }, { "start_idx": 58, "end_idx": 61, "type": "dru", "entity": "环丝氨酸" } ] }, { "text": "(五)吸收障碍如患有消化系统疾病,如慢性腹泻、肠道感染、肠吸收不良综合征等疾病均可减少维生素B6的吸收。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "消化系统疾病" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "慢性腹泻" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "肠道感染" }, { "start_idx": 28, "end_idx": 35, "type": "dis", "entity": "肠吸收不良综合征" } ] }, { "text": "【临床表现】虽然明显缺乏维生素B6的症状较为少见,但是轻度缺乏却比较多见。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "明显缺乏" }, { "start_idx": 12, "end_idx": 21, "type": "dru", "entity": "维生素B6" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "轻度缺乏" } ] }, { "text": "当人体缺乏维生素B6时,常伴有其他营养素的缺乏,尤其是其他水溶性维生素的缺乏,特别是维生素B2,因维生素B2参与维生素B6的代谢。", "entities": [ { "start_idx": 39, "end_idx": 44, "type": "dru", "entity": "水溶性维生素" } ] }, { "text": "(一)生长发育不良维生素B6缺乏的患儿,氨基酸、蛋白质代谢异常,在婴儿期表现为生长发育迟缓。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "生长发育不良" }, { "start_idx": 9, "end_idx": 25, "type": "dis", "entity": "维生素B6缺乏" }, { "start_idx": 30, "end_idx": 40, "type": "sym", "entity": "氨基酸、蛋白质代谢异常" }, { "start_idx": 49, "end_idx": 54, "type": "sym", "entity": "生长发育迟缓" } ] }, { "text": "还可出现贫血。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "贫血" } ] }, { "text": "(二)皮肤脂溢性皮炎维生素B6缺乏可致眼、口腔和鼻周围皮肤脂溢性皮炎,并可向面部、前额、耳后等扩展。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "皮肤脂溢性皮炎" }, { "start_idx": 10, "end_idx": 19, "type": "dru", "entity": "维生素B6" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "眼" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "口腔" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "鼻" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "皮肤脂溢性皮炎" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "面部" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "前额" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "耳后" } ] }, { "text": "也可导致舌炎、口炎、口唇干裂。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "舌炎" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "口炎" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "口唇干裂" } ] }, { "text": "(三)神经精神系统症状个别伴有神经系统症状,如兴奋性增高、尖声哭叫、全身抽搐。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "神经系统" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "兴奋性增高" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "尖声哭叫" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "全身抽搐" } ] }, { "text": "6个月内的小儿可因频繁抽搐而导致智力发育障碍。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "频繁抽搐" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "智力发育障碍" } ] }, { "text": "(四)消化系统症状常伴有一些胃肠道症状,如恶心、呕吐、腹泻等。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "胃肠道" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "恶心" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "呕吐" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "腹泻" } ] }, { "text": "(五)感染维生素B6对免疫系统也有影响。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "感染" }, { "start_idx": 5, "end_idx": 14, "type": "dru", "entity": "维生素B6" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "免疫系统" } ] }, { "text": "维生素B6缺乏,细胞介导免疫系统受损。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dru", "entity": "维生素B6" }, { "start_idx": 18, "end_idx": 25, "type": "bod", "entity": "细胞介导免疫系统" }, { "start_idx": 18, "end_idx": 27, "type": "sym", "entity": "细胞介导免疫系统受损" } ] }, { "text": "Talbot和Meydani等人研究发现,如补充吡哆醇,对淋巴细胞增殖会产生有利的作用。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "吡哆醇" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "研究表明,维生素B6缺乏会损害DNA的合成,故对维持免疫功能很重要。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "dru", "entity": "维生素B6" }, { "start_idx": 5, "end_idx": 21, "type": "dis", "entity": "维生素B6缺乏" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "DNA" } ] }, { "text": "因此,如维生素B6缺乏,抗体生成减少,容易发生感染。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "dru", "entity": "维生素B6" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "抗体" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "感染" } ] }, { "text": "(一)直接法1.血浆磷酸吡哆醛(PLP)浓度测定血浆5-磷酸吡哆醛是肝脏维生素B6的主要存在形式,并且反映组织中的储存,但是血浆5-磷酸吡哆醛对该种维生素摄入量的反应相当缓慢,需要10天才能达到一个新的稳定状态。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "血浆" }, { "start_idx": 10, "end_idx": 21, "type": "ite", "entity": "磷酸吡哆醛(PLP)浓度" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "血浆" }, { "start_idx": 26, "end_idx": 32, "type": "dru", "entity": "5-磷酸吡哆醛" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肝脏" }, { "start_idx": 36, "end_idx": 45, "type": "dru", "entity": "维生素B6" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "血浆" }, { "start_idx": 74, "end_idx": 80, "type": "dru", "entity": "5-磷酸吡哆醛" }, { "start_idx": 84, "end_idx": 86, "type": "dru", "entity": "维生素" } ] }, { "text": "但在评价时应考虑可能影响PLP浓度的各种因素,如蛋白质的摄入增加、AKP药物的活性升高都可使血浆PLP浓度下降。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "ite", "entity": "PLP浓度" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "蛋白质" }, { "start_idx": 33, "end_idx": 40, "type": "ite", "entity": "AKP药物的活性" }, { "start_idx": 33, "end_idx": 42, "type": "sym", "entity": "AKP药物的活性升高" }, { "start_idx": 46, "end_idx": 52, "type": "ite", "entity": "血浆PLP浓度" } ] }, { "text": "目前是以20nmolL血浆PLP浓度为评价维生素B6营养状况的指标。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "ite", "entity": "血浆PLP浓度" }, { "start_idx": 21, "end_idx": 30, "type": "dru", "entity": "维生素B6" } ] }, { "text": "但胎儿体内5-PLP浓度非常高,出生后第一年内迅速降低,然后降低缓慢。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "ite", "entity": "5-PLP浓度" } ] }, { "text": "所以,评价新生儿及婴儿维生素B6的营养状况较困难。", "entities": [ { "start_idx": 11, "end_idx": 20, "type": "dru", "entity": "维生素B6" } ] }, { "text": "2.血浆总维生素B6浓度测定(包括游离维生素B6及吡哆醇磷酸盐)本方法较为简单,是了解体内维生素B6营养状况的敏感指标,但是测定值的波动较大,因此限制了它的使用价值。", "entities": [ { "start_idx": 2, "end_idx": 21, "type": "ite", "entity": "血浆总维生素B6浓度" }, { "start_idx": 27, "end_idx": 39, "type": "dru", "entity": "游离维生素B6<" }, { "start_idx": 45, "end_idx": 50, "type": "dru", "entity": "吡哆醇磷酸盐" }, { "start_idx": 65, "end_idx": 74, "type": "dru", "entity": "维生素B6" } ] }, { "text": "3.尿中维生素B6浓度测定尿中维生素B6排泄,特别是4-吡哆酸的排泄,已被广泛用于研究维生素B6的需要量。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "尿" }, { "start_idx": 4, "end_idx": 13, "type": "dru", "entity": "维生素B6" }, { "start_idx": 4, "end_idx": 20, "type": "ite", "entity": "维生素B6浓度" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "尿" }, { "start_idx": 25, "end_idx": 34, "type": "dru", "entity": "维生素B6" }, { "start_idx": 46, "end_idx": 50, "type": "dru", "entity": "4-吡哆酸" }, { "start_idx": 63, "end_idx": 72, "type": "dru", "entity": "维生素B6" } ] }, { "text": "吡哆酸的排泄量约占维生素B6摄入量的50%,4-吡哆酸的排出量反映近期膳食维生素B6摄入量的变化,正常尿内排泄4-吡哆酸大于0.8mgd,如果少于0.2mgd,即表明维生素B6缺乏。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "吡哆酸" }, { "start_idx": 32, "end_idx": 40, "type": "ite", "entity": "4-吡哆酸的排出量" }, { "start_idx": 47, "end_idx": 56, "type": "dru", "entity": "维生素B6" }, { "start_idx": 71, "end_idx": 71, "type": "bod", "entity": "尿" }, { "start_idx": 75, "end_idx": 79, "type": "ite", "entity": "4-吡哆酸" }, { "start_idx": 103, "end_idx": 112, "type": "dru", "entity": "维生素B6" }, { "start_idx": 103, "end_idx": 119, "type": "dis", "entity": "维生素B6缺乏" } ] }, { "text": "(二)间接法1.尿中色氨酸降解产物的水平(尿色氨酸负荷试验)尿中黄尿酸的排出量是维生素B6缺乏的最早标记物之一。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "尿" }, { "start_idx": 10, "end_idx": 19, "type": "ite", "entity": "色氨酸降解产物的水平" }, { "start_idx": 21, "end_idx": 26, "type": "ite", "entity": "尿色氨酸负荷" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "尿" }, { "start_idx": 32, "end_idx": 38, "type": "ite", "entity": "黄尿酸的排出量" }, { "start_idx": 40, "end_idx": 56, "type": "dis", "entity": "维生素B6缺乏" } ] }, { "text": "正常情况下,黄尿酸是一种微量的色氨酸降解产物,色氨酸降解的主要途径是通过5-磷酸吡哆醛依存的犬尿氨酸酶。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "黄尿酸" }, { "start_idx": 15, "end_idx": 21, "type": "dru", "entity": "色氨酸降解产物" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "色氨酸" }, { "start_idx": 36, "end_idx": 42, "type": "dru", "entity": "5-磷酸吡哆醛" }, { "start_idx": 46, "end_idx": 50, "type": "dru", "entity": "犬尿氨酸酶" } ] }, { "text": "微量黄尿酸也涉及5-磷酸吡哆醛依存的酶。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "黄尿酸" }, { "start_idx": 8, "end_idx": 14, "type": "dru", "entity": "5-磷酸吡哆醛" } ] }, { "text": "维生素B6缺乏时,色氨酸代谢产物及衍生物生成增加,由尿排出体外。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "dis", "entity": "维生素B6缺乏" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "衍生物" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "尿" } ] }, { "text": "黄尿酸能可靠地反映维生素B6的营养状况,给予色氨酸负荷剂量(色氨酸50~100mgkg,配成溶液,总量<2g),通过测定色氨降解产物来评价维生素B6的营养状况,维生素B6缺乏患者的尿中黄尿酸排出量>50mg。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "黄尿酸" }, { "start_idx": 9, "end_idx": 18, "type": "dru", "entity": "维生素B6" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "色氨酸" }, { "start_idx": 40, "end_idx": 42, "type": "dru", "entity": "色氨酸" }, { "start_idx": 79, "end_idx": 88, "type": "dru", "entity": "维生素B6" }, { "start_idx": 100, "end_idx": 109, "type": "dru", "entity": "维生素B6" }, { "start_idx": 120, "end_idx": 120, "type": "bod", "entity": "尿" }, { "start_idx": 122, "end_idx": 127, "type": "ite", "entity": "黄尿酸排出量" } ] }, { "text": "2.红细胞内依赖性维生素B6酶活性的测定红细胞内需要PLP酶,如谷丙酮酸转氨酶(EGPT)、谷草酰乙酸转氨酶(EGOT)、天门冬氨酸转氨酶(α-EAST)等,也是评价体内维生素B6营养状况的敏感指标。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "红细胞" }, { "start_idx": 6, "end_idx": 16, "type": "ite", "entity": "依赖性维生素B6酶活性" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "红细胞" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "PLP酶" }, { "start_idx": 32, "end_idx": 38, "type": "bod", "entity": "谷丙酮酸转氨酶" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "EGPT" }, { "start_idx": 46, "end_idx": 53, "type": "bod", "entity": "谷草酰乙酸转氨酶" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "EGOT" }, { "start_idx": 61, "end_idx": 68, "type": "bod", "entity": "天门冬氨酸转氨酶" }, { "start_idx": 70, "end_idx": 75, "type": "bod", "entity": "α-EAST" }, { "start_idx": 85, "end_idx": 94, "type": "dru", "entity": "维生素B6" } ] }, { "text": "常将红细胞加和不加PLP之比作为评价维生素B6营养状况的指标,加上PLP测定谷丙或谷草转氨酶活性,如活性上升20%以上,表明维生素B6缺乏疾病。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "红细胞" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "PLP" }, { "start_idx": 18, "end_idx": 27, "type": "dru", "entity": "维生素B6" }, { "start_idx": 43, "end_idx": 45, "type": "dru", "entity": "PLP" }, { "start_idx": 48, "end_idx": 49, "type": "dru", "entity": "谷丙" }, { "start_idx": 51, "end_idx": 57, "type": "ite", "entity": "谷草转氨酶活性" }, { "start_idx": 60, "end_idx": 61, "type": "ite", "entity": "活性" }, { "start_idx": 72, "end_idx": 81, "type": "dru", "entity": "维生素B6" } ] }, { "text": "EGOT指数=EGOT+PLPEGOT-PLPEGPT指数=EGPT+PLPEGPT-PLPEGOT活性指数≤1.80为正常,EGPT活性指数≤1.25为正常。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "EGOT" }, { "start_idx": 23, "end_idx": 28, "type": "ite", "entity": "EGPT指数" }, { "start_idx": 46, "end_idx": 51, "type": "ite", "entity": "EGOT活性" }, { "start_idx": 63, "end_idx": 66, "type": "dru", "entity": "EGPT" }, { "start_idx": 63, "end_idx": 68, "type": "ite", "entity": "EGPT活性" } ] }, { "text": "最近也有人测定天门冬氨酸酶的活性作为评价维生素B6营养状况的指标,但测定数值变异较大,使其应用受到了限制。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "ite", "entity": "测定天门冬氨酸酶" }, { "start_idx": 20, "end_idx": 29, "type": "dru", "entity": "维生素B6" } ] }, { "text": "3.血浆高半胱氨酸的含量最近提出以血浆高半胱氨酸作为评价维生素B6营养状况的指标。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血浆" }, { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "高半胱氨酸" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "血浆" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "高半胱氨酸" }, { "start_idx": 28, "end_idx": 37, "type": "dru", "entity": "维生素B6" } ] }, { "text": "因为高半胱氨酸的降解开始于转硫化到半胱氨酸的过程,涉及5-PLP依存酶。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "高半胱氨酸" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "半胱氨酸" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "5-PLP" }, { "start_idx": 27, "end_idx": 34, "type": "dru", "entity": "5-PLP依存酶" } ] }, { "text": "但最近有研究表明,叶酸和维生素B12与血浆高半胱氨酸的水平关系更密切。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "叶酸" }, { "start_idx": 12, "end_idx": 22, "type": "dru", "entity": "维生素B12" }, { "start_idx": 29, "end_idx": 38, "type": "ite", "entity": "血浆高半胱氨酸的水平" } ] }, { "text": "患儿是否有摄入不足、偏食厌食;是否合理膳食,各营养素的比例是否合理;有无妨碍吸收和利用的疾病,如慢性消耗疾病、胃肠道疾病等影响吸收的疾病;患者是否存在需要量增加的因素,如生长发育速度较快、发热等;近来是否服用影响维生素B6活性的药物。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "摄入不足" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "偏食" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "厌食" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "慢性消耗疾病" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "胃肠道疾病" }, { "start_idx": 85, "end_idx": 92, "type": "sym", "entity": "生长发育速度较快" }, { "start_idx": 106, "end_idx": 115, "type": "dru", "entity": "维生素B6" } ] }, { "text": "(二)临床表现婴儿有无生长发育不良,惊厥、抽搐等神经系统表现,以及末梢神经炎、皮炎、口腔、鼻周围皮肤脂溢性皮炎和贫血等表现。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "生长发育不良" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "惊厥" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "抽搐" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "神经系统" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "末梢神经炎" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "皮炎" }, { "start_idx": 42, "end_idx": 54, "type": "sym", "entity": "口腔、鼻周围皮肤脂溢性皮炎" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "贫血" } ] }, { "text": "(三)实验室检验可通过测定血浆中磷酸吡哆醛(PLP)浓度、血浆总维生素B6浓度、尿中的维生素B6浓度、尿中色氨酸降解产物的水平、红细胞内依赖性维生素B6酶活性、血浆高半胱氨酸的含量等方法帮助诊断。", "entities": [ { "start_idx": 13, "end_idx": 27, "type": "ite", "entity": "血浆中磷酸吡哆醛(PLP)浓度" }, { "start_idx": 29, "end_idx": 48, "type": "ite", "entity": "血浆总维生素B6浓度" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "尿" }, { "start_idx": 53, "end_idx": 69, "type": "ite", "entity": "维生素B6浓度" }, { "start_idx": 71, "end_idx": 71, "type": "bod", "entity": "尿" }, { "start_idx": 73, "end_idx": 82, "type": "ite", "entity": "色氨酸降解产物的水平" }, { "start_idx": 84, "end_idx": 86, "type": "bod", "entity": "红细胞" }, { "start_idx": 88, "end_idx": 108, "type": "ite", "entity": "依赖性维生素B6酶活性" }, { "start_idx": 110, "end_idx": 111, "type": "bod", "entity": "血浆" }, { "start_idx": 112, "end_idx": 119, "type": "ite", "entity": "高半胱氨酸的含量" } ] }, { "text": "【预防及治疗】(一)去除病因询问病史,了解患儿喂养情况及辅食添加情况,查明缺乏维生素B6的原因,治疗消化道疾病、慢性消耗性疾病及感染等造成维生素B6缺乏的疾病,以去除病因。", "entities": [ { "start_idx": 39, "end_idx": 48, "type": "dru", "entity": "维生素B6" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "消化道疾病" }, { "start_idx": 66, "end_idx": 72, "type": "dis", "entity": "慢性消耗性疾病" }, { "start_idx": 79, "end_idx": 88, "type": "dru", "entity": "维生素B6" }, { "start_idx": 79, "end_idx": 95, "type": "dis", "entity": "维生素B6缺乏" } ] }, { "text": "(二)调整饮食维生素B6推荐的每天适宜摄入量:6个月以下的婴儿为0.1mg,较大婴儿增加为0.3mg;1~3岁为0.5mg,4~6岁为0.6mg,7~13岁为0.7~0.9mg,14岁以后为1.1~1.2mg,乳母为1.9mg。", "entities": [ { "start_idx": 7, "end_idx": 16, "type": "dru", "entity": "维生素B6" } ] }, { "text": "合理补充含维生素B6丰富的食物,并注意合理搭配。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "dru", "entity": "维生素B6" } ] }, { "text": "高蛋白质、低碳水化合物饮食时,应适当增加维生素B6的摄入,如果乳母维生素B6缺乏,应及时予以补充,避免婴儿发生维生素B6缺乏症。", "entities": [ { "start_idx": 20, "end_idx": 29, "type": "dru", "entity": "维生素B6" }, { "start_idx": 43, "end_idx": 52, "type": "dru", "entity": "维生素B6" }, { "start_idx": 75, "end_idx": 84, "type": "dru", "entity": "维生素B6" }, { "start_idx": 75, "end_idx": 92, "type": "dis", "entity": "维生素B6缺乏症" } ] }, { "text": "人工喂养的婴儿,牛乳不宜经过多次加热、煮沸,避免造成维生素B6的破坏,造成婴儿的维生素B6缺乏。", "entities": [ { "start_idx": 26, "end_idx": 35, "type": "dru", "entity": "维生素B6" }, { "start_idx": 50, "end_idx": 59, "type": "dru", "entity": "维生素B6" } ] }, { "text": "如存在维生素B6缺乏,应多摄入含维生素B6丰富的食物,如肉类、水果、蔬菜、谷类食物,都含有一定量的维生素B6。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dru", "entity": "维生素B6" }, { "start_idx": 3, "end_idx": 19, "type": "dis", "entity": "维生素B6缺乏" }, { "start_idx": 26, "end_idx": 35, "type": "dru", "entity": "维生素B6" }, { "start_idx": 69, "end_idx": 78, "type": "dru", "entity": "维生素B6" } ] }, { "text": "(三)维生素B6治疗【维生素B6依赖症】(一)维生素B6依赖性惊厥这种疾病可能由于在神经系统中,PLP与谷氨酸脱氨酶的辅基酶蛋白不能合成,使γ-氨基丁酸(GABA)合成减少,GABA是中枢神经系统抑制性神经递质,其脑内浓度降低,造成惊厥阈降低。", "entities": [ { "start_idx": 21, "end_idx": 38, "type": "dis", "entity": "维生素B6依赖症" }, { "start_idx": 43, "end_idx": 62, "type": "dis", "entity": "维生素B6依赖性惊厥" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "神经系统" }, { "start_idx": 78, "end_idx": 80, "type": "dru", "entity": "PLP" }, { "start_idx": 82, "end_idx": 87, "type": "dru", "entity": "谷氨酸脱氨酶" }, { "start_idx": 89, "end_idx": 93, "type": "dru", "entity": "辅基酶蛋白" }, { "start_idx": 100, "end_idx": 105, "type": "dru", "entity": "γ-氨基丁酸" }, { "start_idx": 107, "end_idx": 110, "type": "dru", "entity": "GABA" }, { "start_idx": 117, "end_idx": 120, "type": "dru", "entity": "GABA" }, { "start_idx": 122, "end_idx": 127, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 131, "end_idx": 134, "type": "bod", "entity": "神经递质" }, { "start_idx": 137, "end_idx": 137, "type": "bod", "entity": "脑" } ] }, { "text": "多发生于出生数小时~3个月的婴儿,出现反复惊厥,抗癫痫药物治疗无效,静脉注射维生素B6后可缓解,通常使用维生素B65~10mg静脉注射,维持剂量为10~25mgd,该病治疗需维持终身。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "惊厥" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "抗癫痫药物" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "静脉注射" }, { "start_idx": 38, "end_idx": 47, "type": "dru", "entity": "维生素B6" }, { "start_idx": 62, "end_idx": 71, "type": "dru", "entity": "维生素B6" }, { "start_idx": 83, "end_idx": 86, "type": "pro", "entity": "静脉注射" } ] }, { "text": "如患儿出生后不积极予以治疗,可能出现智力低下。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "智力低下" } ] }, { "text": "(二)维生素B6依赖性小细胞低色素性贫血(三)高胱氨酸尿症患儿表现为智力低下、骨骼畸形、肌肉发育不良,其中80%患儿伴有视力障碍,30%患儿有类似Marfan综合征的心脏病。", "entities": [ { "start_idx": 3, "end_idx": 29, "type": "dis", "entity": "维生素B6依赖性小细胞低色素性贫血" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "高胱氨酸尿症" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "智力低下" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "骨骼畸形" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "肌肉" }, { "start_idx": 54, "end_idx": 59, "type": "sym", "entity": "肌肉发育不良" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "视力障碍" }, { "start_idx": 83, "end_idx": 91, "type": "dis", "entity": "Marfan综合征" } ] }, { "text": "部分病例给予大剂量维生素B6治疗,高胱氨酸尿消失,但也有部分病例无效。", "entities": [ { "start_idx": 9, "end_idx": 18, "type": "dru", "entity": "维生素B6" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "高胱氨酸" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "尿" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "高胱氨酸尿消失" } ] }, { "text": "(四)胱硫醚尿症胱硫醚酶是维生素B6依赖酶,如维生素B6缺乏,胱硫醚酶的活性降低,胱硫醚不能分解,积聚在体内,患儿表现为智力迟滞、肢端肥大、耳畸形、耳聋、血小板减少、肾性尿崩症,易发肾结石。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "胱硫醚尿症" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "胱硫醚酶" }, { "start_idx": 13, "end_idx": 22, "type": "dru", "entity": "维生素B6" }, { "start_idx": 33, "end_idx": 42, "type": "dru", "entity": "维生素B6" }, { "start_idx": 33, "end_idx": 49, "type": "sym", "entity": "维生素B6缺乏" }, { "start_idx": 51, "end_idx": 59, "type": "sym", "entity": "胱硫醚酶的活性降低" }, { "start_idx": 61, "end_idx": 63, "type": "dru", "entity": "胱硫醚" }, { "start_idx": 80, "end_idx": 83, "type": "sym", "entity": "智力迟滞" }, { "start_idx": 85, "end_idx": 88, "type": "sym", "entity": "肢端肥大" }, { "start_idx": 90, "end_idx": 92, "type": "sym", "entity": "耳畸形" }, { "start_idx": 94, "end_idx": 95, "type": "sym", "entity": "耳聋" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "血小板" }, { "start_idx": 97, "end_idx": 101, "type": "sym", "entity": "血小板减少" }, { "start_idx": 103, "end_idx": 107, "type": "sym", "entity": "肾性尿崩症" }, { "start_idx": 111, "end_idx": 113, "type": "sym", "entity": "肾结石" } ] }, { "text": "应用大剂量维生素B6治疗,具有一定的疗效。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "dru", "entity": "维生素B6" } ] }, { "text": "二、贫血的分类(一)形态学分类根据红细胞体积的大小以及血红蛋白在红细胞内的含量即色素的高低来判断贫血的类型。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "贫血" }, { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "红细胞体积" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "红细胞" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "含量" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "色素" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "贫血" } ] }, { "text": "代表红细胞体积大小的指标为平均红细胞体积(meancorpuscularvolume,MCV)和平均红细胞血红蛋白含量(meancorpuscularhemoglobin,MCH),代表红细胞内血红蛋白含量或色素高低的指标为平均红细胞血红蛋白浓度(MeanCorpuscularHemoglobinConcentration,MCHC)其正常值及有关贫血的种类见表10-10。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "ite", "entity": "红细胞体积" }, { "start_idx": 13, "end_idx": 19, "type": "ite", "entity": "平均红细胞体积" }, { "start_idx": 21, "end_idx": 41, "type": "ite", "entity": "meancorpuscularvolume" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "MCV" }, { "start_idx": 48, "end_idx": 58, "type": "ite", "entity": "平均红细胞血红蛋白含量" }, { "start_idx": 60, "end_idx": 84, "type": "ite", "entity": "meancorpuscularhemoglobin" }, { "start_idx": 86, "end_idx": 88, "type": "ite", "entity": "MCH" }, { "start_idx": 93, "end_idx": 95, "type": "bod", "entity": "红细胞" }, { "start_idx": 97, "end_idx": 100, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 101, "end_idx": 102, "type": "ite", "entity": "含量" }, { "start_idx": 104, "end_idx": 105, "type": "bod", "entity": "色素" }, { "start_idx": 112, "end_idx": 122, "type": "ite", "entity": "平均红细胞血红蛋白浓度" }, { "start_idx": 124, "end_idx": 161, "type": "ite", "entity": "MeanCorpuscularHemoglobinConcentration" }, { "start_idx": 163, "end_idx": 166, "type": "ite", "entity": "MCHC" }, { "start_idx": 175, "end_idx": 176, "type": "dis", "entity": "贫血" } ] }, { "text": "表10-2贫血的形态学分类(二)病因分类根据贫血发生的原因和发病机制进行分类,对临床诊断与治疗均有指导意义(表10-10)。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "贫血" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "贫血" }, { "start_idx": 40, "end_idx": 46, "type": "pro", "entity": "临床诊断与治疗" } ] }, { "text": "表10-3贫血的病因分类", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "贫血" } ] }, { "text": "第三节小儿药物治疗的影响因素小儿药物治疗的特点受体液的pH、细胞膜的通透性、药物与蛋白质的结合程度、药物在肝脏内的代谢和肾脏排泄等多种因素的影响。", "entities": [ { "start_idx": 24, "end_idx": 28, "type": "ite", "entity": "体液的pH" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "细胞膜" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "蛋白质" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "肝脏" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "肾脏" } ] }, { "text": "一、年龄对药物胃肠道吸收的影响血管外使用的药物在进入全身循环并分布到作用部位前,必须穿过许多生理膜从而影响其吸收率。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "胃肠道" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血管" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "全身循环" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "生理膜" } ] }, { "text": "虽然一些益生菌不被吸收,一些营养成分可通过主动转运和促进扩散(facilitateddiffusion)而吸收,但大多数药物在胃肠道经过被动扩散而吸收。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "mic", "entity": "益生菌" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "胃肠道" } ] }, { "text": "病人的一些重要因素可影响胃肠道吸收药物的速率和吸收量,如消化道pH、有无胃内容物及其种类、胃排空时间、胃肠动力情况等。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "胃肠道" }, { "start_idx": 28, "end_idx": 32, "type": "ite", "entity": "消化道pH" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "胃" } ] }, { "text": "在口服用药时应考虑下列因素:新生儿的胃液分泌、肠蠕动和胆汁分泌功能均较婴儿或儿童低下,胃排空时间较短;婴儿和儿童胃液分泌、肠蠕动和胆汁分泌功能正常,胃排空时间增加。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "胃液" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "肠" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "胆汁" }, { "start_idx": 43, "end_idx": 47, "type": "ite", "entity": "胃排空时间" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "胃液" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "肠" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "胆汁" }, { "start_idx": 74, "end_idx": 78, "type": "ite", "entity": "胃排空时间" } ] }, { "text": "小婴儿喂药时最好将小儿抱起或头略抬高,以免呛咳将药吐出。", "entities": [ { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "头" } ] }, { "text": "病情需要时可采用鼻饲给药。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "鼻饲" } ] }, { "text": "第七章消化性溃疡消化性溃疡(pepticulcer,PU)是指那些接触消化液(胃酸和胃蛋白酶)的胃肠黏膜及其深层组织的一种局限性黏膜缺损,其深度达到或穿透黏膜肌层。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 14, "end_idx": 24, "type": "dis", "entity": "pepticulcer" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "PU" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "消化液" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "胃酸" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "胃蛋白酶" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "胃肠黏膜" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "深层组织" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "局限性黏膜缺损" }, { "start_idx": 77, "end_idx": 80, "type": "bod", "entity": "黏膜肌层" } ] }, { "text": "本病95%以上发生在胃和十二指肠,即又称胃溃疡和十二指肠溃疡。", "entities": [ { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "胃" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "十二指肠" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "胃溃疡" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "十二指肠溃疡" } ] }, { "text": "近年来随着诊断技术的进步,尤为消化内镜在儿科的普及应用,该病的检出率明显上升,上海瑞金医院溃疡病平均检出率占胃镜检查的12%;成人中报道约有10%的人在其一生中有过溃疡病。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "equ", "entity": "消化内镜" }, { "start_idx": 20, "end_idx": 21, "type": "dep", "entity": "儿科" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "溃疡病" }, { "start_idx": 54, "end_idx": 57, "type": "pro", "entity": "胃镜检查" }, { "start_idx": 82, "end_idx": 84, "type": "dis", "entity": "溃疡病" } ] }, { "text": "【病因及发病机制】消化性溃疡的病因繁多,有遗传、精神、环境、饮食、吸烟及内分泌等因素,迄今尚无定论,发病机制多倾向于攻击因素-防御因素失衡学说。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "消化性溃疡" } ] }, { "text": "正常情况下胃黏膜分泌黏液,良好的血液运输、旺盛的细胞更新能力及胃液分泌的调节机制等防御因素处于优势,或与盐酸、胃蛋白酶及幽门螺杆菌等攻击因素保持平衡;一旦攻击因素增强或(和)防御因素削弱则可形成溃疡。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "黏液" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "血液" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "细胞" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "胃液" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "胃蛋白酶" }, { "start_idx": 60, "end_idx": 64, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 97, "end_idx": 98, "type": "dis", "entity": "溃疡" } ] }, { "text": "目前认为,在上述因素中两大环境因素对大多数溃疡患者的发病有重要意义,即幽门螺杆菌感染与非甾体类抗炎药(NSAIDs)的使用。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "溃疡" }, { "start_idx": 35, "end_idx": 39, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 43, "end_idx": 49, "type": "dru", "entity": "非甾体类抗炎药" }, { "start_idx": 51, "end_idx": 56, "type": "dru", "entity": "NSAIDs" } ] }, { "text": "(一)致消化性溃疡的有害因素消化性溃疡形成的基本因素是胃酸及胃蛋白酶分泌增加。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "胃酸" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "胃蛋白酶" } ] }, { "text": "1.胃酸1910年Schwartz提出“无酸无溃疡”的名言,现在仍然正确。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "胃酸" } ] }, { "text": "胃酸是由胃黏膜的壁细胞分泌,壁细胞上有3种受体即乙酰胆碱受体、胃泌素受体及组胺受体。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胃酸" }, { "start_idx": 4, "end_idx": 10, "type": "bod", "entity": "胃黏膜的壁细胞" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "壁细胞" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "受体" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "乙酰胆碱受体" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "胃泌素受体" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "组胺受体" } ] }, { "text": "这3种受体在接受相应物质乙酰胆碱、胃泌素及组胺的刺激后产生泌酸效应。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "受体" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "乙酰胆碱" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "胃泌素" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "组胺" } ] }, { "text": "迷走神经活动亦与胃酸分泌有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "迷走神经" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胃酸" } ] }, { "text": "(1)壁细胞泌酸过程可分3步:①组胺、胆碱能递质或胃泌素与细胞底一边膜上的相应受体结合;②经第二信息(AMP、Ca2+)介导,使刺激信号由细胞内向细胞顶端膜传递;③在刺激下,使H+-K+-ATP酶移至分泌性微管,将H+从胞浆泵向胃腔,生成胃酸。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "壁细胞" }, { "start_idx": 16, "end_idx": 23, "type": "bod", "entity": "组胺、胆碱能递质" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "胃泌素" }, { "start_idx": 29, "end_idx": 40, "type": "bod", "entity": "细胞底一边膜上的相应受体" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "细胞" }, { "start_idx": 79, "end_idx": 83, "type": "bod", "entity": "细胞顶端膜" }, { "start_idx": 94, "end_idx": 103, "type": "bod", "entity": "H+-K+-ATP酶" }, { "start_idx": 106, "end_idx": 110, "type": "bod", "entity": "分泌性微管" }, { "start_idx": 116, "end_idx": 117, "type": "bod", "entity": "胞浆" }, { "start_idx": 120, "end_idx": 121, "type": "bod", "entity": "胃腔" }, { "start_idx": 125, "end_idx": 126, "type": "bod", "entity": "胃酸" } ] }, { "text": "一般情况下组胺、乙酰胆碱和胃泌素除单独地促进胃酸分泌外,还有协同作用。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "胃泌素" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "胃酸" } ] }, { "text": "(2)正常人平均每日胃液分泌量1000~1500ml,盐酸40mmol/L;十二指肠溃疡(duodenalulcer,DU)患者每日胃液分泌量1500~2000ml,盐酸40~80mmol/L;而胃溃疡(gastriculcer,GU)患者每日胃液分泌量及盐酸多在正常范围。", "entities": [ { "start_idx": 10, "end_idx": 25, "type": "sym", "entity": "胃液分泌量1000~1500ml" }, { "start_idx": 27, "end_idx": 36, "type": "sym", "entity": "盐酸40mmol/L" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "十二指肠溃疡" }, { "start_idx": 45, "end_idx": 57, "type": "dis", "entity": "duodenalulcer" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "DU" }, { "start_idx": 66, "end_idx": 81, "type": "sym", "entity": "胃液分泌量1500~2000ml" }, { "start_idx": 83, "end_idx": 95, "type": "sym", "entity": "盐酸40~80mmol/L" }, { "start_idx": 98, "end_idx": 100, "type": "dis", "entity": "胃溃疡" }, { "start_idx": 102, "end_idx": 113, "type": "dis", "entity": "gastriculcer" }, { "start_idx": 115, "end_idx": 116, "type": "dis", "entity": "GU" }, { "start_idx": 122, "end_idx": 135, "type": "sym", "entity": "胃液分泌量及盐酸多在正常范围" } ] }, { "text": "胃酸分泌随着年龄改变而变化,小儿出生时胃液呈碱性,24~48小时游离酸分泌达高峰,此认为与来自母体的胃泌素通过胎盘有直接关系,2天后母体胃泌素减少,胃酸降低。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胃酸" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胃液" }, { "start_idx": 32, "end_idx": 34, "type": "ite", "entity": "游离酸" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "胃泌素" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "胎盘" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "胃泌素" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "胃酸" } ] }, { "text": "所以新生儿在出生2天后就可发生急性胃溃疡及胃穿孔。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "急性胃溃疡" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "胃穿孔" } ] }, { "text": "由于胃酸分泌随年龄增加,年长儿消化性溃疡较婴儿多。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "胃酸" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "消化性溃疡" } ] }, { "text": "(3)胃酸增高的原因1)壁细胞数量增加:正常男性为1.09×109,女性为0.82×109。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "胃酸" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "壁细胞" } ] }, { "text": "而DU为1.8×109(增加1倍多),GU为0.8×109(接近正常)。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "GU" } ] }, { "text": "2)促胃泌素:人促胃泌素G17(胃窦部最高)或G34(十二指肠最高),DU患者促胃泌素无增加。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "促胃泌素" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "促胃泌素G17" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "胃窦部" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "G34" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "十二指肠" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "DU" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "促胃泌素无增加" } ] }, { "text": "有人提出DU患者胃酸分泌增高可能与壁细胞对胃泌素刺激敏感有关。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "DU" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "胃酸分泌增高" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "壁细胞" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "胃泌素" } ] }, { "text": "Isenberg和Grossman曾给DU及非溃疡(NUD)患者注射8个不同剂量的促胃泌素,结果达到最大胃酸分泌量(MAO)时促胃液素半数有效量NDU的均值为148.2±30.3,DU为60.5±96,说明DU患者酸分泌过高是壁细胞对促胃液素敏感所致。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "DU" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "非溃疡" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "NUD" }, { "start_idx": 41, "end_idx": 44, "type": "dru", "entity": "促胃泌素" }, { "start_idx": 50, "end_idx": 56, "type": "ite", "entity": "最大胃酸分泌量" }, { "start_idx": 58, "end_idx": 60, "type": "ite", "entity": "MAO" }, { "start_idx": 63, "end_idx": 71, "type": "ite", "entity": "促胃液素半数有效量" }, { "start_idx": 72, "end_idx": 74, "type": "ite", "entity": "NDU" }, { "start_idx": 90, "end_idx": 91, "type": "dis", "entity": "DU" }, { "start_idx": 103, "end_idx": 104, "type": "dis", "entity": "DU" }, { "start_idx": 107, "end_idx": 107, "type": "bod", "entity": "酸" }, { "start_idx": 113, "end_idx": 115, "type": "bod", "entity": "壁细胞" }, { "start_idx": 117, "end_idx": 120, "type": "dis", "entity": "促胃液素" } ] }, { "text": "3)驱动胃酸分泌增加的其他因素:神经、内分泌及旁分泌等因素可影响胃酸分泌增加,消化性溃疡患者基础胃酸分泌量分泌的紧张度增加,敏感性也增加。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "胃酸" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "神经" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "胃酸" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 46, "end_idx": 67, "type": "sym", "entity": "基础胃酸分泌量分泌的紧张度增加,敏感性也增加" } ] }, { "text": "2.胃蛋白酶胃壁主细胞分泌胃蛋白酶原,按照免疫化学分型,分为蛋白酶原Ⅰ(PGⅠ)和蛋白酶原Ⅱ(PGⅡ)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "胃蛋白酶" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "胃壁主细胞" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "胃蛋白酶原" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "蛋白酶原Ⅰ" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "PGⅠ" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "蛋白酶原Ⅱ" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "PGⅡ" } ] }, { "text": "PGⅠ存在5种亚型,分布于胃体主细胞,PGⅡ存在于胃体及胃窦。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "PGⅠ" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "胃体主细胞" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "PGⅡ" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "胃体" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "胃窦" } ] }, { "text": "应用放免法可在30%~50%DU患者血中测出PGⅠ升高,当达到130μg/L,其致DU的危险较正常人增高3倍。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "DU" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "血中测出PGⅠ升高" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "DU" } ] }, { "text": "PGⅡ升高时致GU危险性增高3倍。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "PGⅡ" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "GU" } ] }, { "text": "胃蛋白酶的消化作用是与胃酸紧密联系在一起的,当胃酸pH1.8~2.5时胃蛋白酶活性达到最佳状态,当pH>4时胃蛋白酶失去活性,不起消化作用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "胃蛋白酶" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "胃酸" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "胃酸pH" }, { "start_idx": 35, "end_idx": 40, "type": "ite", "entity": "胃蛋白酶活性" }, { "start_idx": 49, "end_idx": 50, "type": "ite", "entity": "pH" }, { "start_idx": 54, "end_idx": 61, "type": "ite", "entity": "胃蛋白酶失去活性" } ] }, { "text": "故消化作用必须有足够的酸使pH达到3以下才能激活胃蛋白酶,胃酸与胃蛋白酶共同作用产生溃疡,但胃酸是主要因素。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "pH" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "胃蛋白酶" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "胃酸" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "胃蛋白酶" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "溃疡" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "胃酸" } ] }, { "text": "小儿出生时胃液中胃蛋白酶含量极微,以后缓慢增加,至青春期达到成人水平。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "胃液" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "胃蛋白酶" } ] }, { "text": "3.胆汁酸盐胆汁与胃溃疡的关系早有报道。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "胆汁" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "胃溃疡" } ] }, { "text": "在胃窦或十二指肠发生动力紊乱时,胆汁反流入胃,引起胃黏膜损伤,特别是胆汁和胰液在十二指肠互相混合生成溶血卵磷脂,后者破坏胃黏膜屏障,使氢离子反向弥散而损害胃黏膜。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "胃窦" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "十二指肠" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "动力紊乱" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "胆汁" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "胃" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "胃黏膜损伤" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "胆汁" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "胰液" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "十二指肠" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "溶血卵磷脂" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "胃黏膜屏障" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "胃黏膜" } ] }, { "text": "现认为胆汁对胃黏膜的损伤,主要是由胆汁酸(胆盐)所致。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "胆汁" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "胆汁酸" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "胆盐" } ] }, { "text": "胆盐有增加胃内氢离子的反向弥散和降低黏膜电位差的作用,与胃内的酸性环境和胆汁的浓度有密切关系。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胆盐" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "胃" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "胃" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "胆汁" } ] }, { "text": "动物实验表明氢离子反向弥散在胆汁高浓度和pH2的条件下反应最显著,低浓度和pH8的条件下反应轻微。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "胆汁" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "pH2" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "pH8" } ] }, { "text": "胆汁酸刺激肥大细胞释放组胺,组胺可使胃黏膜血管扩张,毛细血管壁的通透性增加,导致黏膜水肿、出血、发炎及糜烂,在这样的情况下黏膜很容易发展成溃疡。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "胆汁酸" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "肥大细胞" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "组胺" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "组胺" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "胃黏膜血管扩张" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "毛细血管壁的通透性增加" }, { "start_idx": 40, "end_idx": 52, "type": "sym", "entity": "黏膜水肿、出血、发炎及糜烂" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "黏膜" }, { "start_idx": 69, "end_idx": 70, "type": "dis", "entity": "溃疡" } ] }, { "text": "4.幽门螺杆菌感染幽门螺杆菌与慢性胃炎密切相关,抑制幽门螺杆菌使原发性消化性溃疡愈合率增加,消除幽门螺杆菌以后溃疡复发率显著下降,细菌的消除以及胃十二指肠炎的消退在很多研究中与溃疡不复发有关。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 26, "end_idx": 30, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "原发性消化性溃疡" }, { "start_idx": 48, "end_idx": 52, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "溃疡" }, { "start_idx": 65, "end_idx": 66, "type": "mic", "entity": "细菌" }, { "start_idx": 72, "end_idx": 77, "type": "dis", "entity": "胃十二指肠炎" }, { "start_idx": 88, "end_idx": 89, "type": "dis", "entity": "溃疡" } ] }, { "text": "文献报道,在未服用ASA及其他NSAIDs的胃十二指肠溃疡患者中,90%以上均有幽门螺杆菌感染引起的慢性活动性胃炎,仅约5%~10%的十二指肠溃疡患者及30%的胃溃疡患者无明确的幽门螺杆菌感染的证据。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "ASA" }, { "start_idx": 13, "end_idx": 20, "type": "dru", "entity": "其他NSAIDs" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "胃十二指肠溃疡" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "慢性活动性胃炎" }, { "start_idx": 67, "end_idx": 72, "type": "dis", "entity": "十二指肠溃疡" }, { "start_idx": 80, "end_idx": 82, "type": "dis", "entity": "胃溃疡" }, { "start_idx": 89, "end_idx": 93, "type": "dis", "entity": "幽门螺杆菌" } ] }, { "text": "且根除幽门螺杆菌后消化性溃疡1年复发率<10%,而幽门螺杆菌(+)的消化性溃疡愈合后1年复发率50%左右,2年复发率几乎达100%,所以,无酸无溃疡,有被“无幽门螺杆菌感染无溃疡”取代或者两者并存的趋势。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 25, "end_idx": 29, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 72, "end_idx": 73, "type": "dis", "entity": "溃疡" }, { "start_idx": 79, "end_idx": 85, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 87, "end_idx": 88, "type": "dis", "entity": "溃疡" } ] }, { "text": "幽门螺杆菌感染在胃黏膜的改变很大程度上可能与幽门螺杆菌的产物(细胞毒素及尿素酶)以及炎症过程有关。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 22, "end_idx": 26, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "细胞毒素" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "尿素酶" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "炎症" } ] }, { "text": "幽门螺杆菌感染和黏膜的炎症可破坏胃及十二指肠黏膜屏障的完整性,DU不伴幽门螺杆菌少见,但不清楚的是为什么只有一小部分感染了幽门螺杆菌的患者发展为消化性溃疡,其发病机制如何?", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "黏膜" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "炎症" }, { "start_idx": 16, "end_idx": 25, "type": "bod", "entity": "胃及十二指肠黏膜屏障" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "DU" }, { "start_idx": 35, "end_idx": 39, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 61, "end_idx": 65, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "消化性溃疡" } ] }, { "text": "(1)幽门螺杆菌菌株:不同的幽门螺杆菌菌株有不同的致病性,产生不同的临床结果,具有细胞空泡毒素(CagA及VagA)的幽门螺杆菌菌株感染,使患溃疡的机会增加。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "mic", "entity": "幽门螺杆菌菌株" }, { "start_idx": 14, "end_idx": 20, "type": "mic", "entity": "幽门螺杆菌菌株" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "细胞空泡" }, { "start_idx": 59, "end_idx": 65, "type": "mic", "entity": "幽门螺杆菌菌株" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "溃疡" } ] }, { "text": "目前已发现儿童溃疡患者感染此菌比例很高。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "溃疡" } ] }, { "text": "(2)宿主的遗传易感性:O型血的人较其他血型者DU发生率高30%~40%,血型物质不分泌型者发生DU的可能性高40%~50%,也有研究认为幽门螺杆菌感染和不同的血型抗原是DU发生中两个独立的因素。", "entities": [ { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "血" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "DU" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "血型物质" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "DU" }, { "start_idx": 69, "end_idx": 75, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 80, "end_idx": 83, "type": "bod", "entity": "血型抗原" }, { "start_idx": 85, "end_idx": 86, "type": "dis", "entity": "DU" } ] }, { "text": "(3)炎症反应:中性粒细胞引起氧化反应。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "炎症" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "中性粒细胞" } ] }, { "text": "幽门螺杆菌表面蛋白质激活单核细胞和巨噬细胞,分泌IL-1及TNF,合成血小板激活因子而产生严重的病理反应。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "幽门螺杆菌表面蛋白质" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "单核细胞" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "IL-1" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "TNF" }, { "start_idx": 35, "end_idx": 41, "type": "bod", "entity": "血小板激活因子" } ] }, { "text": "(4)酸分泌反应:有报道幽门螺杆菌感染者,食物蛋白胨等可引起胃窦G细胞促胃泌素的释放增加,细菌消除后恢复正常。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 30, "end_idx": 43, "type": "sym", "entity": "胃窦G细胞促胃泌素的释放增加" } ] }, { "text": "更多认为幽门螺杆菌感染导致胃窦部炎症,使胃窦部胃泌素释放增加,生长抑素分泌下降而致胃酸分泌增加。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "胃窦部炎症" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "胃窦部胃泌素释放增加" }, { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "生长抑素分泌下降" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "胃酸分泌增加" } ] }, { "text": "(5)十二指肠的胃上皮化生:幽门螺杆菌引起十二指肠胃黏膜化生,使十二指肠碳酸氢盐分泌降低,胃酸分泌增加。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "十二指肠" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "胃上皮" }, { "start_idx": 14, "end_idx": 18, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "十二指肠胃黏膜" }, { "start_idx": 32, "end_idx": 43, "type": "sym", "entity": "十二指肠碳酸氢盐分泌降低" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "胃酸分泌增加" } ] }, { "text": "另有人认为幽门螺杆菌产生的细胞空泡毒素在胃液中释放与激活,通过幽门到肠管,活化的空泡毒素在未被肠内一些蛋白酶消化前,即引起十二指肠上皮细胞空泡形成,于是在十二指肠缺乏幽门螺杆菌存在的条件下导致十二指肠溃疡。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "胃液" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肠管" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "肠" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "蛋白酶" }, { "start_idx": 61, "end_idx": 68, "type": "bod", "entity": "十二指肠上皮细胞" }, { "start_idx": 77, "end_idx": 80, "type": "bod", "entity": "十二指肠" }, { "start_idx": 83, "end_idx": 87, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 96, "end_idx": 101, "type": "dis", "entity": "十二指肠溃疡" } ] }, { "text": "5.药物因素引起消化性溃疡的药物中较重要的有三类:①阿司匹林(ASA);②非甾体抗炎药物(NSAIDs),如吲哚美辛及保泰松;③肾上腺皮质激素。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "ASA" }, { "start_idx": 37, "end_idx": 43, "type": "dru", "entity": "非甾体抗炎药物" }, { "start_idx": 45, "end_idx": 50, "type": "dru", "entity": "NSAIDs" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 59, "end_idx": 61, "type": "dru", "entity": "保泰松" }, { "start_idx": 64, "end_idx": 70, "type": "dru", "entity": "肾上腺皮质激素" } ] }, { "text": "ASA及大多数其他NSAIDs与消化性溃疡的相互作用表现在几个方面:小剂量时可致血小板功能障碍;稍大剂量可引起急性浅表性胃黏膜糜烂致出血,约2/3长期使用NSAIDs的患者存在胃十二指肠黏膜病变,其中大多数为浅表损害,约1/4长期应用药物的患者有溃疡病。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "ASA" }, { "start_idx": 7, "end_idx": 14, "type": "dru", "entity": "其他NSAIDs" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "血小板功能障碍" }, { "start_idx": 55, "end_idx": 64, "type": "dis", "entity": "急性浅表性胃黏膜糜烂" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "出血" }, { "start_idx": 77, "end_idx": 82, "type": "dru", "entity": "NSAIDs" }, { "start_idx": 88, "end_idx": 94, "type": "bod", "entity": "胃十二指肠黏膜" }, { "start_idx": 123, "end_idx": 125, "type": "dis", "entity": "溃疡病" } ] }, { "text": "但ASA/NSAIDs致胃溃疡机制尚不清楚,现认为是这些药物直接损伤胃黏膜,除使氢离子逆向弥散增加之外,还可抑制前列腺素合成,使胃酸及胃蛋白酶分泌增加,胃黏膜血液供应障碍,胃黏膜屏障功能下降。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "ASA" }, { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "NSAIDs" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "胃溃疡" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "前列腺素" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "胃酸" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "胃蛋白酶" }, { "start_idx": 76, "end_idx": 80, "type": "bod", "entity": "胃黏膜血液" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "胃黏膜屏障" } ] }, { "text": "6.遗传因素(1)GU和DU同胞患病比一般人群高1.8倍和2.6倍,GU易患GU、DU易患DU。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "GU" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "DU" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "GU" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "GU" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "DU" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "DU" } ] }, { "text": "儿童中DU患儿家族史明显。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "DU" } ] }, { "text": "O型血发生PUD高于其他血型35%左右,主要为DU;且溃疡伴出血、穿孔,并发症者以O型多见。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "PUD" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "DU" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "溃疡" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "出血" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "穿孔" } ] }, { "text": "调查发现,DU患儿男性多于女性,48.08%系DU家族史,家族发病率一级家属>二级家属>三级家属,一级家属的发病率高于普通人群的11倍,O型血多见,占患儿的44.23%,且症状严重。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "DU" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "DU" } ] }, { "text": "HLA血清分型发现HLA-B5、HLA-B12、HLA-BW35与DU有相关性。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "DU" } ] }, { "text": "HLA-DQA1*03基因与DU有关。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "DU" } ] }, { "text": "上海市瑞金医院对十二指肠溃疡患儿HLA-DQA1基因,DU患儿*03等位基因频率明显低于健康正常儿童,提示*03基因对DU有重要的抵抗作用。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "十二指肠溃疡" }, { "start_idx": 16, "end_idx": 24, "type": "ite", "entity": "HLA-DQA1基" }, { "start_idx": 16, "end_idx": 24, "type": "bod", "entity": "HLA-DQA1基" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "1基" }, { "start_idx": 67, "end_idx": 68, "type": "dis", "entity": "3基" } ] }, { "text": "(3)胃蛋白酶原(PG)是胃蛋白酶前体,分泌PGⅠ及PGⅡ,家系调查发现DU患者一半血清中PGⅠ含量增高,在高PGⅠ后代,50%也显示高PGⅠ,表明PGⅠ血症患者为单染色体显性遗传,支持DU遗传基因存在。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "胃蛋白酶原" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "PG" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "胃蛋白酶前体" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "PGⅠ" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "PGⅡ" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "DU" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "血清" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "PGⅠ" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "PGⅠ" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "PGⅠ" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "PGⅠ" }, { "start_idx": 93, "end_idx": 94, "type": "bod", "entity": "DU" } ] }, { "text": "7.精神因素15年前,对胃造瘘患者观察发现,人胃黏膜随人的情绪变化而出现不同的反应,兴奋时,胃黏膜充血,胃液分泌增多,胃运动加强;而抑郁和绝望时,胃黏膜苍白,胃运动减慢。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "胃造瘘" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "胃黏膜" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "胃黏膜充血" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "胃液分泌增多" }, { "start_idx": 59, "end_idx": 63, "type": "sym", "entity": "胃运动加强" }, { "start_idx": 73, "end_idx": 77, "type": "sym", "entity": "胃黏膜苍白" }, { "start_idx": 79, "end_idx": 83, "type": "sym", "entity": "胃运动减慢" } ] }, { "text": "胃肠道的功能,包括胃液分泌及胃肠运动都会在情绪、催眠和生物反馈抑制的影响下发生变化。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "胃肠道" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胃液" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "胃肠" } ] }, { "text": "应激时,胃酸分泌增加,胰腺分泌下降,胃的排空率明显下降,溃疡患者在应激时产生的恐惧程度高于健康人群。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "胃酸分泌增加" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "胰腺分泌下降" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "胃的排空率明显下降" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "溃疡" } ] }, { "text": "Mark等分析发现:溃疡患者多疑、固执,有较强的依赖感,处理事物能力差,不成熟,易冲动,易感到孤独,自我控制能力差,易处于受压和焦虑的状态。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "溃疡" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "多疑" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "固执" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "有较强的依赖感" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "处理事物能力差" }, { "start_idx": 36, "end_idx": 38, "type": "sym", "entity": "不成熟" }, { "start_idx": 40, "end_idx": 42, "type": "sym", "entity": "易冲动" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "易感到孤独" }, { "start_idx": 50, "end_idx": 56, "type": "sym", "entity": "自我控制能力差" }, { "start_idx": 58, "end_idx": 68, "type": "sym", "entity": "易处于受压和焦虑的状态" } ] }, { "text": "学龄儿童消化性溃疡发病率增加与学习负担过重、精神压力和心理因素逐渐复杂有关。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "消化性溃疡" } ] }, { "text": "8.食物因素中国南方食米区,消化性溃疡发病率较食面食为主的北方地区为高。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "消化性溃疡" } ] }, { "text": "乱吃冷饮,嗜好辛辣食品或暴饮暴食,早餐不吃,晚上贪吃,过食油炸食物、含汽饮料等不良习惯都对胃黏膜造成直接损伤。", "entities": [ { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "胃黏膜" } ] }, { "text": "(二)消化性溃疡的防御因素1.胃黏膜屏障作用胃黏膜屏障是由黏膜表层上皮细胞的细胞膜及细胞间隙的紧密连接所组成,黏膜抵抗氢离子反渗的作用过程有三个部分:①维持胃液中氢离子浓度与胃壁组织液中氢离子浓度的梯度差;②抵挡氢离子逆向弥散及其他有害物质如胆汁、药物及胃蛋白酶对黏膜的损害;③上皮和黏膜/黏膜下血循环营养黏膜,并促进愈合。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "胃黏膜屏障" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "胃黏膜屏障" }, { "start_idx": 29, "end_idx": 36, "type": "bod", "entity": "黏膜表层上皮细胞" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "细胞膜" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "细胞间隙" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "黏膜" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "胃液" }, { "start_idx": 87, "end_idx": 91, "type": "bod", "entity": "胃壁组织液" }, { "start_idx": 121, "end_idx": 122, "type": "bod", "entity": "胆汁" }, { "start_idx": 127, "end_idx": 130, "type": "bod", "entity": "胃蛋白酶" }, { "start_idx": 132, "end_idx": 133, "type": "bod", "entity": "黏膜" }, { "start_idx": 139, "end_idx": 140, "type": "bod", "entity": "上皮" }, { "start_idx": 142, "end_idx": 143, "type": "bod", "entity": "黏膜" }, { "start_idx": 145, "end_idx": 146, "type": "bod", "entity": "黏膜" }, { "start_idx": 153, "end_idx": 154, "type": "bod", "entity": "黏膜" } ] }, { "text": "2.黏液屏障作用胃黏膜表面覆盖着一层黏液,是由黏膜上皮细胞及胃隐窝处颈黏膜细胞分泌,内含大分子物质如糖蛋白、黏多糖、蛋白质及磷脂等,其厚度约为上皮细胞的10~20倍。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "黏液屏障" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "黏液" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "黏膜上皮细胞" }, { "start_idx": 30, "end_idx": 38, "type": "bod", "entity": "胃隐窝处颈黏膜细胞" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "糖蛋白" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "黏多糖" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "蛋白质" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "磷脂" }, { "start_idx": 71, "end_idx": 74, "type": "bod", "entity": "上皮细胞" } ] }, { "text": "使其下面的黏膜与胃腔内容物隔离,阻挡氢离子及胃蛋白酶的损害。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "黏膜" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胃腔" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "胃蛋白酶" } ] }, { "text": "3.碳酸氢盐分泌胃和十二指肠黏膜近端还能分泌小量碳酸氢盐进入黏膜层,中和黏膜层表面的酸,使上皮细胞表面能经常维持pH6~8的范围,抵挡氢离子的逆向弥散作用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "碳酸氢盐" }, { "start_idx": 8, "end_idx": 15, "type": "bod", "entity": "胃和十二指肠黏膜" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "碳酸氢盐" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "黏膜" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "黏膜层" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "酸" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 56, "end_idx": 57, "type": "ite", "entity": "pH" } ] }, { "text": "4.胃黏膜血液供应与上皮细胞再生能力胃、十二指肠黏膜层有丰富的血液供应,向黏膜细胞输送足够的营养物质及不断清除代谢产物,使上皮细胞及时更新。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "ite", "entity": "胃黏膜血液" }, { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "上皮细胞" }, { "start_idx": 18, "end_idx": 26, "type": "ite", "entity": "胃、十二指肠黏膜层" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "血液" }, { "start_idx": 37, "end_idx": 40, "type": "ite", "entity": "黏膜细胞" }, { "start_idx": 61, "end_idx": 64, "type": "ite", "entity": "上皮细胞" } ] }, { "text": "动物实验证实黏膜损伤后能在30分钟内迅速修复。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "黏膜损伤" } ] }, { "text": "因此脱落与更新之间维持在平衡状态,从而保持了黏膜的完整性。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "黏膜" } ] }, { "text": "当胃黏膜供血不足,黏膜缺血坏死,细胞再生更新延缓时,则有可能形成溃疡。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "黏膜" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "溃疡" } ] }, { "text": "5.前列腺素作用胃黏膜上皮细胞有不断合成及释放内源性前列腺素(PG)的作用,主要是PGE2;后者具有防止各种有害物质对消化道上皮细胞损伤和酸坏死的作用,这种作用称为细胞保护。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "前列腺素" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "胃黏膜上皮细胞" }, { "start_idx": 23, "end_idx": 29, "type": "bod", "entity": "内源性前列腺素" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "PG" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "PGE2" }, { "start_idx": 65, "end_idx": 71, "type": "bod", "entity": "消化道上皮细胞" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "细胞" } ] }, { "text": "具体表现为:①保护胃黏膜免遭有毒物质的损害;②减少NSAIDs所致消化道出血,凡在酸性pH下不解离并溶于脂肪的物质,在胃内很容易进入黏膜细胞,一旦进入细胞后,由于pH的改变而发生解离,其通透性降低,潴留在黏膜细胞内起毒性作用,如NSAIDs。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "NSAIDs" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "消化道出血" }, { "start_idx": 43, "end_idx": 44, "type": "ite", "entity": "pH" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "脂肪" }, { "start_idx": 59, "end_idx": 59, "type": "bod", "entity": "胃" }, { "start_idx": 66, "end_idx": 69, "type": "bod", "entity": "黏膜细胞" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "细胞" }, { "start_idx": 81, "end_idx": 82, "type": "ite", "entity": "pH" }, { "start_idx": 102, "end_idx": 105, "type": "bod", "entity": "黏膜细胞" }, { "start_idx": 114, "end_idx": 119, "type": "dis", "entity": "NSAIDs" } ] }, { "text": "PG细胞保护作用的机制:①促使胃黏膜上皮细胞分泌黏液及;②抑制基础胃酸及进餐后胃酸分泌;③加强黏膜的血液循环和蛋白质合成;④促进表面活性磷脂的释放,从而加强了胃黏膜表面的流水性;⑤清除氧自由基。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "PG细胞" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "胃黏膜上皮细胞" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "黏液" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "胃酸" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "胃酸" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "黏膜" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "血液" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "蛋白质" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "磷脂" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "胃黏膜" } ] }, { "text": "非甾体类消炎药抑制前列腺素合成,故可诱发溃疡。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "非甾体类消炎药" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "前列腺素" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "溃疡" } ] }, { "text": "除前列腺素外,一些脑肠肽如生长抑素、胰多肽及脑啡肽等也有细胞保护作用。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "前列腺素" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "脑肠肽" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "生长抑素" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "胰多肽" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "脑啡肽" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "细胞" } ] }, { "text": "6.表皮生长因子表皮生长因子(EGF)是从唾液腺、十二指肠黏液中的Brunner腺及胰腺等组织分泌的多肽。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "表皮生长因子" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "表皮生长因子" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "EGF" }, { "start_idx": 21, "end_idx": 30, "type": "bod", "entity": "唾液腺、十二指肠黏液" }, { "start_idx": 33, "end_idx": 40, "type": "bod", "entity": "Brunner腺" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "胰腺" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "多肽" } ] }, { "text": "已有不少报道,EGF在胃肠道内与胃黏膜的特异受体结合而发挥细胞保护作用。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "EGF" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "胃肠道" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "胃黏膜" } ] }, { "text": "如给予外源性的EGF后,能明显减轻乙醇及阿司匹林等有害物质对胃黏膜的损伤,初步的临床观察给消化性溃疡患者口服EGF后,可促进溃疡愈合。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "EGF" }, { "start_idx": 17, "end_idx": 18, "type": "dru", "entity": "乙醇" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 45, "end_idx": 51, "type": "dis", "entity": "消化性溃疡患者" }, { "start_idx": 54, "end_idx": 56, "type": "dru", "entity": "EGF" } ] }, { "text": "EGF保护胃黏膜促进溃疡愈合的作用,可能与EGF参与胃黏膜上皮细胞再生的调节,刺激消化道黏膜DNA合成,促进上皮再生与痊愈有关,也有报道EGF可使胃黏膜血流量增多。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "EGF" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "EGF" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "胃黏膜上皮细胞" }, { "start_idx": 41, "end_idx": 48, "type": "bod", "entity": "消化道黏膜DNA" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "上皮" }, { "start_idx": 68, "end_idx": 70, "type": "dru", "entity": "EGF" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "胃黏膜" } ] }, { "text": "【临床表现】(一)症状与体征小儿消化性溃疡临床表现各种各样,不同的年龄症状差异较大。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "消化性溃疡" } ] }, { "text": "1.新生儿期以突发性上消化道出血或穿孔为主要特征,常急性起病,以呕血、便血、腹胀及腹膜炎表现为主,易被误诊。", "entities": [ { "start_idx": 7, "end_idx": 18, "type": "sym", "entity": "突发性上消化道出血或穿孔" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "呕血" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "便血" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "腹胀" }, { "start_idx": 41, "end_idx": 43, "type": "sym", "entity": "腹膜炎" } ] }, { "text": "此期多为急性应激性溃疡,死亡率较高。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "急性应激性溃疡" } ] }, { "text": "2.婴幼儿期此期患儿以急性起病多见,突然呕血、黑便,前期可能有食欲减退、呕吐和腹痛,生长发育迟缓等。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "突然呕血" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "黑便" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "食欲减退" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "呕吐" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "腹痛" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "生长发育迟缓" } ] }, { "text": "3.学龄前期原发性溃疡逐渐增多,此期腹痛症状明显,多位于脐周,呈间歇性发作,与饮食关系不明确,恶心、呕吐与上消化道出血也较常见。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "原发性溃疡" }, { "start_idx": 18, "end_idx": 36, "type": "sym", "entity": "腹痛症状明显,多位于脐周,呈间歇性发作" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "恶心" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "呕吐" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "上消化道出血" } ] }, { "text": "4.学龄期以十二指肠溃疡多见,随着年龄递增,临床表现与成人接近,症状以上腹痛和脐周腹痛为主,有时有夜间痛,或泛酸、嗳气或慢性贫血。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "十二指肠溃疡" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "腹痛" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "脐周腹痛" }, { "start_idx": 49, "end_idx": 51, "type": "sym", "entity": "夜间痛" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "泛酸" }, { "start_idx": 58, "end_idx": 58, "type": "sym", "entity": "气" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "慢性贫血" } ] }, { "text": "少数人表现无痛性黑便、昏厥,甚至休克。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "无痛性黑便" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "昏厥" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "休克" } ] }, { "text": "(二)并发症1.出血出血的并发症有时可以是溃疡的首发症状,而无任何前驱表现。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "出血" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "出血" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "溃疡" } ] }, { "text": "2.穿孔穿孔较出血少见得多,溃疡穿孔常突然发生,可无任何先兆症状。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "穿孔" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "穿孔" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "出血" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "溃疡穿孔" } ] }, { "text": "少数儿童可无溃疡病史,以穿孔并发症为首发症状。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "溃疡" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "穿孔" } ] }, { "text": "经手术证实为十二指肠溃疡伴穿孔。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "十二指肠溃疡" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "穿孔" } ] }, { "text": "在新生儿早期也可见应激性胃溃疡穿孔,表现腹痛、腹胀。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "应激性胃溃疡穿孔" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "腹痛" } ] }, { "text": "【诊断】因小儿消化性溃疡症状不典型,所以,对临床凡有原因不明的反复发作性腹痛,长期呕吐、黑便、呕血、慢性贫血或在严重的全身性疾病基础上出现胃肠道症状时,都应考虑有消化性溃疡可能,需做进一步检查。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "反复发作性腹痛" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "长期呕吐" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "黑便" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "呕血" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "慢性贫血" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "全身性疾病" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "胃肠道" }, { "start_idx": 81, "end_idx": 85, "type": "dis", "entity": "消化性溃疡" } ] }, { "text": "(一)分类小儿消化性溃疡主要分为原发性与继发性溃疡两大类(表13-13)。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "原发性" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "继发性溃疡" } ] }, { "text": "表13-3小儿消化性溃疡分类(二)辅助检查1.内镜检查内镜检查是诊断消化性溃疡最重要的手段,溃疡在内镜下所见为圆形或椭圆形病灶,少数为线形,边界清楚,中央披有灰白色苔状物,周边黏膜轻微隆起或在同一平面。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "小儿消化性溃疡" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "内镜检查" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "内镜检查" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "溃疡" }, { "start_idx": 49, "end_idx": 50, "type": "equ", "entity": "内镜" }, { "start_idx": 55, "end_idx": 62, "type": "sym", "entity": "圆形或椭圆形病灶" }, { "start_idx": 64, "end_idx": 68, "type": "sym", "entity": "少数为线形" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "边界清楚" }, { "start_idx": 75, "end_idx": 84, "type": "sym", "entity": "中央披有灰白色苔状物" }, { "start_idx": 86, "end_idx": 99, "type": "sym", "entity": "周边黏膜轻微隆起或在同一平面" } ] }, { "text": "根据病程的不同,溃疡分为三个周期:活动期、愈合期及瘢痕期。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "溃疡" } ] }, { "text": "2.X线钡餐检查溃疡病的X线征象可分为直接和间接两种。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "X线钡餐检查" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "溃疡病" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "X线" } ] }, { "text": "钡剂充盈于溃疡的凹陷处形成龛影,为诊断溃疡病的直接征象,也为确诊依据。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "钡剂" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "溃疡" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "凹陷处形成龛影" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "溃疡病" } ] }, { "text": "溃疡周边被炎症和水肿组织包绕,龛影周边可出现透光圈。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "溃疡周边被炎症和水肿组织包绕" }, { "start_idx": 15, "end_idx": 24, "type": "sym", "entity": "龛影周边可出现透光圈" } ] }, { "text": "由于纤维组织增生,黏膜皱襞呈放射状向龛影集中,瘢痕形成和肌肉痉挛可使胃和十二指肠腔局部变形,出现的局部压痛、胃大弯侧痉挛性切迹、十二指肠球部激惹、充盈不佳以及畸形等均为间接征象,只能提示但不能确诊为溃疡。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "纤维组织" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "黏膜皱襞" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肌肉" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "胃" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "十二指肠腔" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "局部压痛" }, { "start_idx": 54, "end_idx": 62, "type": "sym", "entity": "胃大弯侧痉挛性切迹" }, { "start_idx": 64, "end_idx": 80, "type": "sym", "entity": "十二指肠球部激惹、充盈不佳以及畸形" }, { "start_idx": 99, "end_idx": 100, "type": "dis", "entity": "溃疡" } ] }, { "text": "气钡双重造影可使黏膜显示清晰,但小儿常不能配合完成。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "气钡双重造影" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "黏膜" } ] }, { "text": "在儿童急性溃疡时病灶浅表,愈合较快,X线钡餐检查常常易漏诊或误诊。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "急性溃疡" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "X线钡餐检查" } ] }, { "text": "3.幽门螺杆菌的检测幽门螺杆菌感染检测主要分为两方面:①侵入性方法:通过胃镜取胃黏膜活体组织做幽门螺杆菌培养,快速尿素酶测定,细菌染色检查。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "幽门螺杆菌的检测" }, { "start_idx": 10, "end_idx": 18, "type": "pro", "entity": "幽门螺杆菌感染检测" }, { "start_idx": 36, "end_idx": 37, "type": "equ", "entity": "胃镜" }, { "start_idx": 39, "end_idx": 45, "type": "bod", "entity": "胃黏膜活体组织" }, { "start_idx": 47, "end_idx": 53, "type": "pro", "entity": "幽门螺杆菌培养" }, { "start_idx": 55, "end_idx": 61, "type": "pro", "entity": "快速尿素酶测定" }, { "start_idx": 63, "end_idx": 68, "type": "pro", "entity": "细菌染色检查" } ] }, { "text": "②非侵入性方法:测定血清中幽门螺杆菌IgG作为幽门螺杆菌的筛查指标,以及尿素呼气试验,呼气试验阳性提示有活动性幽门螺杆菌感染。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "血清" }, { "start_idx": 13, "end_idx": 20, "type": "mic", "entity": "幽门螺杆菌IgG" }, { "start_idx": 23, "end_idx": 27, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 36, "end_idx": 41, "type": "pro", "entity": "尿素呼气试验" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "呼气试验阳性" }, { "start_idx": 52, "end_idx": 61, "type": "dis", "entity": "活动性幽门螺杆菌感染" } ] }, { "text": "【治疗】消化性溃疡的治疗目前已取得很大进展,过去常选用中和胃酸或抑制胃酸分泌的药物,仅可有效控制症状和溃疡暂时愈合,新的观点认为消化性溃疡是一种环境因素所致的疾病,如果明确并去除潜在的致病因素,即可得到永久性的治愈。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 27, "end_idx": 40, "type": "dru", "entity": "中和胃酸或抑制胃酸分泌的药物" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "溃疡" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "消化性溃疡" } ] }, { "text": "(二)饮食疗法过去主张少量多餐,近年发现所有食物,包括牛奶,进食后均可刺激胃酸分泌。", "entities": [ { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "胃酸" } ] }, { "text": "主张一般饮食,症状发作严重时,白天可每2小时进食一次,症状减轻改为一日三餐,限制咖啡、浓茶和汽水等饮料,忌用阿司匹林一类药物。", "entities": [ { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "阿司匹林" } ] }, { "text": "(三)幽门螺杆菌阴性消化性溃疡的传统治疗在下述药物中,以H2受体阻滞剂应用最多,其机制为抑制组胺对壁细胞的泌酸作用,但对于胆碱能神经或胃泌素合并的餐后胃酸分泌影响较小。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "幽门螺杆菌阴性消化性溃疡" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "组胺" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "壁细胞" }, { "start_idx": 67, "end_idx": 71, "type": "bod", "entity": "胆碱能神经" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "胃泌素" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "胃酸" } ] }, { "text": "1.抗酸治疗即中和胃酸,降低胃及十二指肠内的酸度,减轻胃酸对胃肠黏膜的损伤。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "抗酸治疗" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "中和胃酸" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "胃及十二指肠" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "胃酸" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "胃肠黏膜" } ] }, { "text": "如复方碳酸钙咀嚼片、铝碳酸镁、碳酸氢钠、氢氧化铝、氢氧化镁。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dru", "entity": "复方碳酸钙咀嚼片" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "铝碳酸镁" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "氢氧化铝" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "氢氧化镁" } ] }, { "text": "2.胃蛋白酶抑制剂(1)抗酸剂或酸分泌抑制剂:胃蛋白酶在碱性环境失活。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dru", "entity": "胃蛋白酶抑制剂" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "抗酸剂" }, { "start_idx": 16, "end_idx": 21, "type": "dru", "entity": "酸分泌抑制剂" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "胃蛋白酶" } ] }, { "text": "(2)硫酸支链淀粉:250mg每天3~4次,硫酸化多糖与胃蛋白酶结合,使之失活。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "硫酸支链淀粉" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "硫酸化多糖" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "胃蛋白酶" } ] }, { "text": "3.抗胆碱能药物阻断壁细胞的乙酰胆碱受体(M1分布胃黏膜,尤为壁细胞,M2分布心、膈肌、膀胱及胃肠平滑肌),乙酰胆碱对G细胞的作用,使胃酸及胃泌素分泌减少。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "抗胆碱能药物" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "壁细胞" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "乙酰胆碱受体" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "M1" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "壁细胞" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "M2" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "心" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "膈肌" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "膀胱" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "胃肠平滑肌" }, { "start_idx": 66, "end_idx": 69, "type": "dru", "entity": "乙酰胆碱" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "G细胞" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "胃酸" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "胃泌素" } ] }, { "text": "(1)非特异性胆碱能神经阻滞剂:如阿托品、654-654、胃安及胃欢等。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dru", "entity": "非特异性胆碱能神经阻滞剂" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "阿托品" }, { "start_idx": 21, "end_idx": 27, "type": "dru", "entity": "654-654" }, { "start_idx": 29, "end_idx": 30, "type": "dru", "entity": "胃安" }, { "start_idx": 32, "end_idx": 33, "type": "dru", "entity": "胃欢" } ] }, { "text": "阻断M1及M2受体,抑酸差,解痉镇痛好,限用于DU及少数有痉挛疼痛的GU患者,消化性溃疡有胃排空不良者不用。", "entities": [ { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "DU" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "痉挛疼痛" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "GU" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 57, "end_idx": 61, "type": "sym", "entity": "胃排空不良" } ] }, { "text": "与H2受体阻滞剂有协同作用,用于顽固消化性溃疡。", "entities": [ { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "顽固消化性溃疡" } ] }, { "text": "阻断M1受体,抑酸显著,对心及瞳孔等无副作用。", "entities": [ { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "心" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "瞳孔" } ] }, { "text": "4.组胺H2受体阻断剂阻断组胺与壁细胞膜H2受体结合,抑制胃酸分泌,是相当安全的药物。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "组胺" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "胃酸" } ] }, { "text": "不良反应:①可有头昏、疲乏、口干、轻泻、潮红及肌痛。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "头昏" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "疲乏" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "口干" }, { "start_idx": 18, "end_idx": 18, "type": "sym", "entity": "泻" }, { "start_idx": 21, "end_idx": 21, "type": "sym", "entity": "红" } ] }, { "text": "②偶有肝损。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肝损" } ] }, { "text": "③可引起急性间质性肾炎及肾衰竭。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "急性间质性肾炎" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "④可出现可逆性精神紊乱。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "可逆性精神紊乱" } ] }, { "text": "⑤偶见骨髓抑制,血小板减少。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "骨髓抑制" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "血小板减少" } ] }, { "text": "⑦本药为肝微粒体酶抑制剂,与细胞色素P450结合,降低药酶活性,因此不宜和氨茶碱、地西泮、地高辛、奎尼丁、咖啡因、酮康唑、氢氧化铝、氧化酶及甲氧氯普胺合用。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dru", "entity": "肝微粒体酶抑制剂" }, { "start_idx": 14, "end_idx": 21, "type": "bod", "entity": "细胞色素P450" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "氨茶碱" }, { "start_idx": 41, "end_idx": 43, "type": "dru", "entity": "地西泮" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "地高辛" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "奎尼丁" }, { "start_idx": 53, "end_idx": 55, "type": "dru", "entity": "咖啡因" }, { "start_idx": 57, "end_idx": 59, "type": "dru", "entity": "酮康唑" }, { "start_idx": 61, "end_idx": 64, "type": "dru", "entity": "氢氧化铝" }, { "start_idx": 66, "end_idx": 68, "type": "dru", "entity": "氧化酶" }, { "start_idx": 70, "end_idx": 74, "type": "dru", "entity": "甲氧氯普胺" } ] }, { "text": "⑧和硫糖铝合用会降低后者的疗效;和维拉帕米合用可提高后者生物利用度,使其不良反应增加;和阿司匹林合用使后者作用增强。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "硫糖铝" }, { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "维拉帕米合" }, { "start_idx": 44, "end_idx": 47, "type": "dru", "entity": "阿司匹林" } ] }, { "text": "⑨有与氨基糖苷类药物相似的神经阻断作用,且不被新斯的明对抗,只能被氯化钙对抗,如和氨基糖苷类合用有可能导致呼吸抑制或停止。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dru", "entity": "氨基糖苷类药物" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "氯化钙" }, { "start_idx": 41, "end_idx": 45, "type": "dru", "entity": "氨基糖苷类" }, { "start_idx": 53, "end_idx": 59, "type": "sym", "entity": "呼吸抑制或停止" } ] }, { "text": "(2)雷尼替丁(ranitidine):儿童4~5mg/(kg•d),2次/日,疗程6周。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "雷尼替丁" }, { "start_idx": 8, "end_idx": 17, "type": "dru", "entity": "ranitidine" } ] }, { "text": "注意:①婴儿及<8岁儿童慎用;②不良反应轻微,可有皮疹、便秘、腹泻、头痛、出汗及焦虑等;③偶有可逆性的细胞血小板减少,转氨酶升高;④可降低维生素B12的吸收;⑤可减少肝血流量,因而与普萘洛尔及利多卡因合用时可延缓此药的作用;⑥与普鲁卡因合用,可使普鲁卡因清除率减低。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "皮疹" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "便秘" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "腹泻" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "头痛" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "出汗" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "焦虑" }, { "start_idx": 47, "end_idx": 57, "type": "sym", "entity": "可逆性的细胞血小板减少" }, { "start_idx": 59, "end_idx": 63, "type": "sym", "entity": "转氨酶升高" }, { "start_idx": 87, "end_idx": 92, "type": "sym", "entity": "减少肝血流量" }, { "start_idx": 97, "end_idx": 100, "type": "dis", "entity": "普萘洛尔" }, { "start_idx": 102, "end_idx": 105, "type": "dis", "entity": "利多卡因" }, { "start_idx": 120, "end_idx": 123, "type": "dis", "entity": "普鲁卡因" }, { "start_idx": 129, "end_idx": 137, "type": "sym", "entity": "普鲁卡因清除率减低" } ] }, { "text": "(3)法莫替丁(famotidine):儿童0.8~1mg/(kg•d),2次/日。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "法莫替丁" }, { "start_idx": 8, "end_idx": 17, "type": "dru", "entity": "famotidine" } ] }, { "text": "注意:①肝、肾功能不好慎用;②应在排除肿瘤后再给药;③常见有头痛、便秘及腹泻等;④偶见皮疹、荨麻疹,白细胞减少,氨基转移酶升高;⑤罕见腹部胀满感、食欲缺乏及心率增加,血压升高,颜面潮红等。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "肿瘤" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "头痛" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "便秘" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "腹泻" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "皮疹" }, { "start_idx": 46, "end_idx": 48, "type": "sym", "entity": "荨麻疹" }, { "start_idx": 50, "end_idx": 54, "type": "sym", "entity": "白细胞减少" }, { "start_idx": 56, "end_idx": 62, "type": "sym", "entity": "氨基转移酶升高" }, { "start_idx": 67, "end_idx": 71, "type": "sym", "entity": "腹部胀满感" }, { "start_idx": 73, "end_idx": 76, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 78, "end_idx": 81, "type": "sym", "entity": "心率增加" }, { "start_idx": 83, "end_idx": 86, "type": "sym", "entity": "血压升高" }, { "start_idx": 88, "end_idx": 91, "type": "sym", "entity": "颜面潮红" } ] }, { "text": "5.质子泵阻断剂(protonpumpinhibitor,PPI)奥美拉唑(omeprazole)特异地作用于壁细胞,选择性抑制壁细胞的H+-K+-ATP酶,作用于胃酸分泌的最后一环节,对组胺、五肽胃泌素及乙酰胆碱引起的胃酸分泌均有抑制持续时间长、对壁细胞无毒性的作用,目前未发现明显副作用。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "质子泵阻断剂" }, { "start_idx": 9, "end_idx": 27, "type": "dru", "entity": "protonpumpinhibitor" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "PPI" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "奥美拉唑" }, { "start_idx": 38, "end_idx": 47, "type": "dru", "entity": "omeprazole" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "壁细胞" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "壁细胞" }, { "start_idx": 68, "end_idx": 77, "type": "bod", "entity": "H+-K+-ATP酶" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "胃酸" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "组胺" }, { "start_idx": 97, "end_idx": 101, "type": "bod", "entity": "五肽胃泌素" }, { "start_idx": 103, "end_idx": 106, "type": "bod", "entity": "乙酰胆碱" }, { "start_idx": 110, "end_idx": 111, "type": "bod", "entity": "胃酸" }, { "start_idx": 125, "end_idx": 127, "type": "bod", "entity": "壁细胞" } ] }, { "text": "注意:①不良反应发生与雷尼替丁相似。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "雷尼替丁" } ] }, { "text": "②有酶抑作用,可延长地西泮及苯妥英钠等药的半衰期。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "地西泮" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "苯妥英钠" } ] }, { "text": "同用后可出现共济失调、步态不稳及行走困难,但茶碱和普萘洛尔的代谢不受本品影响。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "共济失调" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "步态不稳及行走困难" }, { "start_idx": 22, "end_idx": 23, "type": "dru", "entity": "茶碱" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "普萘洛尔" } ] }, { "text": "③偶见恶心、呕吐、便秘、胀气、头痛、皮疹、一过性转氨酶及胆红素升高。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "恶心" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "呕吐" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "便秘" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "头痛" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "皮疹" }, { "start_idx": 21, "end_idx": 32, "type": "sym", "entity": "一过性转氨酶及胆红素升高" } ] }, { "text": "6.胃黏膜保护剂(1)生胃酮:使胃黏膜上皮生命延长,胃黏液分泌增加。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "胃黏膜保护剂" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "生胃酮" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "胃黏膜上皮生命延长" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "胃黏液分泌增加" } ] }, { "text": "不良反应有醛固酮效应,水、钠潴留,低血钾,高血压等。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "醛固酮效应" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "水、钠潴留" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "低血钾" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "高血压" } ] }, { "text": "(2)硫糖铝:硫酸化二糖和氢氧化铝的复合物,不被胃肠道吸收,黏附溃疡基底,形成保护层,防止H离子逆向弥散。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "硫糖铝" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "硫酸化二糖" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "氢氧化铝" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "胃肠道" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "溃疡基底" } ] }, { "text": "②主要副作用为便秘,偶有口干、恶心及胃痛等,可适当合用抗胆碱药。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "便秘" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "恶心" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "胃痛" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "抗胆碱药" } ] }, { "text": "③和多酶片合用,两者有拮抗作用,使疗效均降低。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "多酶片" } ] }, { "text": "④和西咪替丁合用,使本药疗效减低。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "西咪替丁" } ] }, { "text": "⑤与四环素、西咪替丁、苯妥因钠及地高辛合用时,可干扰和影响这些药物的吸收,故因间隔2小时后再服用上述药物。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "四环素" }, { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "西咪替丁" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "苯妥因钠" } ] }, { "text": "⑥肾功能不全,长期服用,可能会引起铝中毒。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "铝中毒" } ] }, { "text": "注意:①年幼儿一般不宜服用此药,肾功能不全者应慎用;②铋可使大便和舌苔、牙齿染黑及恶心、呕吐,停药后消失;③不宜与牛奶、茶、咖啡及含酒精饮料同服;④长期大量应用,可发生不可逆性脑病、精神紊乱及运动失调,有条件者应做血铋检测。", "entities": [ { "start_idx": 27, "end_idx": 27, "type": "dru", "entity": "铋" }, { "start_idx": 30, "end_idx": 39, "type": "sym", "entity": "大便和舌苔、牙齿染黑" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "恶心" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "呕吐" }, { "start_idx": 84, "end_idx": 89, "type": "dis", "entity": "不可逆性脑病" }, { "start_idx": 91, "end_idx": 94, "type": "dis", "entity": "精神紊乱" }, { "start_idx": 96, "end_idx": 99, "type": "dis", "entity": "运动失调" }, { "start_idx": 107, "end_idx": 110, "type": "pro", "entity": "血铋检测" } ] }, { "text": "其作用为细胞保护,增强胃肠黏膜防御能力,抑制胃酸及胃蛋白酶原的分泌。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "胃肠黏膜" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "胃酸" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "胃蛋白酶原" } ] }, { "text": "不良反应有腹泻及子宫收缩,孕妇忌用。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "腹泻" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "子宫收缩" } ] }, { "text": "前列腺素衍生物有恩前列腺素,成人35μg,每日2次,疗效与西咪替丁相似。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "前列腺素衍生物" }, { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "恩前列腺素" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "西咪替丁" } ] }, { "text": "此药是目前预防和治疗非甾体类消炎药引起的胃和十二指肠黏膜损伤最有效的药物。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dru", "entity": "非甾体类消炎药" }, { "start_idx": 20, "end_idx": 29, "type": "dis", "entity": "胃和十二指肠黏膜损伤" } ] }, { "text": "7.其他谷氨酰胺呱仑酸钠颗粒(抗炎、抗溃疡、促进组织修复),蒙脱石散等通过增加黏膜厚度及加强黏膜屏障功能,促进溃疡愈合。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "dru", "entity": "谷氨酰胺呱仑酸钠颗粒" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "蒙脱石散" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "黏膜" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "黏膜屏障" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "溃疡" } ] }, { "text": "(四)幽门螺杆菌阳性消化性溃疡的治疗目前幽门螺杆菌阳性合并有活动期溃疡的患者除给予传统抗溃疡药物治疗,如H2受体阻滞剂、质子泵抑制剂或硫糖铝促进溃疡愈合外,常同时给予抗生素根除幽门螺杆菌。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "幽门螺杆菌阳性消化性溃疡" }, { "start_idx": 20, "end_idx": 34, "type": "dis", "entity": "幽门螺杆菌阳性合并有活动期溃疡" }, { "start_idx": 66, "end_idx": 71, "type": "dru", "entity": "质子泵抑制剂" }, { "start_idx": 73, "end_idx": 75, "type": "dru", "entity": "硫糖铝" }, { "start_idx": 89, "end_idx": 91, "type": "dru", "entity": "抗生素" }, { "start_idx": 94, "end_idx": 98, "type": "mic", "entity": "幽门螺杆菌" } ] }, { "text": "虽然理论上抗菌治疗后根除幽门螺杆菌的同时亦可使溃疡愈合,但仍缺乏足够数量的单独应用抗菌药物治疗的病例研究。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "mic", "entity": "幽门螺杆菌" } ] }, { "text": "抗菌治疗目前在儿科应用最广泛,最廉价,被证实确实有效的抗幽门螺杆菌三联的方案:阿莫西林、甲硝唑和铋制剂(三钾二枸橼酸合铋及次水杨酸铋等)。", "entities": [ { "start_idx": 28, "end_idx": 32, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 39, "end_idx": 42, "type": "dru", "entity": "阿莫西林" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "甲硝唑" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "铋制剂" }, { "start_idx": 52, "end_idx": 59, "type": "dru", "entity": "三钾二枸橼酸合铋" }, { "start_idx": 61, "end_idx": 65, "type": "dru", "entity": "次水杨酸铋" } ] }, { "text": "对于应用甲硝唑出现明显不良作用或既往曾用过甲硝唑(幽门螺杆菌易对其产生耐药性)的患者,可用克拉霉素取代。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "甲硝唑" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "甲硝唑" }, { "start_idx": 25, "end_idx": 29, "type": "mic", "entity": "幽门螺杆菌" } ] }, { "text": "应用奥美拉唑、阿莫西林与克拉霉素的三联疗法。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "奥美拉唑" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "阿莫西林" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "克拉霉素" } ] }, { "text": "(五)消化性溃疡外科治疗主要适用于溃疡伴有出血、穿孔、梗阻等并发症或经内科治疗经久不愈患者。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "外科治疗" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "溃疡" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "出血" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "穿孔" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "梗阻" } ] }, { "text": "五、结核杆菌感染的阶段结核杆菌感染可以分为3个阶段:接触有传染性的结核患者、无症状结核感染和活动结核病。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "结核杆菌感染" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "结核杆菌感染" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "结核" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "无症状结核感染" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "活动结核病" } ] }, { "text": "和有传染性的结核患者接触的患儿,其结核菌素试验阴性,没有症状和体征,肺部X线片正常。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "结核" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "结核菌素试验" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "结核菌素试验阴性" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "肺部X线" } ] }, { "text": "他们不一定感染结核,但其感染结核和发展为活动结核病的危险性增加。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "结核" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "结核" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "活动结核病" } ] }, { "text": "无症状结核感染是指由结核杆菌感染引起的结核菌素试验阳性结核病灶,没有体征,X线胸片正常。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "无症状结核感染" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "结核杆菌感染" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "结核菌素试验" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "结核菌素试验阳性" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "结核" } ] }, { "text": "部分患儿可能有临床症状,如不规则发热或低热、精神不振、盗汗、疲乏、食欲缺乏等。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "不规则发热" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "低热" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "精神不振" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "盗汗" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "疲乏" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "食欲缺乏" } ] }, { "text": "无症状结核感染是否发展成为活动结核病,与患者的年龄以及其他危险因素有关。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "无症状结核感染" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "活动结核病" } ] }, { "text": "婴幼儿、老年人、伴有某种疾病或免疫功能抑制的患者,在无症状结核感染后易发展成活动结核病。", "entities": [ { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "无症状结核感染" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "活动结核病" } ] }, { "text": "无症状结核感染的患者,如不治疗,可能发展为活动结核病。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "无症状结核感染" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "活动结核病" } ] }, { "text": "活动结核病是指患者有结核杆菌感染的症状、体征、肺部影像学改变以及实验室检测到结核杆菌等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "活动结核病" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "结核杆菌感染" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺部" }, { "start_idx": 38, "end_idx": 41, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "这种感染可以累及肺内、肺外或两者同时受累。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肺内" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肺外" } ] }, { "text": "但以肺结核病最多见。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "肺结核病" } ] }, { "text": "九、颅咽管瘤颅咽管瘤(craniopharyngioma)占儿童中枢神经系统肿瘤的6%~9%,多发生在20岁以前,平均发病年龄为8岁。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "颅咽管瘤" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "颅咽管瘤" }, { "start_idx": 11, "end_idx": 27, "type": "dis", "entity": "craniopharyngioma" }, { "start_idx": 30, "end_idx": 39, "type": "dis", "entity": "儿童中枢神经系统肿瘤" } ] }, { "text": "可因孟氏孔阻塞引起脑积水颅内压增高压迫视交叉引起视力视野障碍;侵犯或压迫视丘下部导致内分泌功能紊乱如尿崩症、嗜睡、脂肪代谢或体温调节障碍及垂体性侏儒症MRI是最有效的诊断工具,能很好地显示肿瘤性质、范围及与周围结构的关系。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "脑积水" }, { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "可因孟氏孔阻塞引起脑积水颅内压增高" }, { "start_idx": 17, "end_idx": 29, "type": "sym", "entity": "压迫视交叉引起视力视野障碍" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "视丘下部" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "内分泌功能紊乱" }, { "start_idx": 31, "end_idx": 48, "type": "sym", "entity": "侵犯或压迫视丘下部导致内分泌功能紊乱" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "尿崩症" }, { "start_idx": 72, "end_idx": 74, "type": "dis", "entity": "侏儒症" }, { "start_idx": 49, "end_idx": 74, "type": "sym", "entity": "如尿崩症、嗜睡、脂肪代谢或体温调节障碍及垂体性侏儒症" }, { "start_idx": 75, "end_idx": 77, "type": "pro", "entity": "MRI" } ] }, { "text": "另外,由于肿瘤对周围重要结构的浸润压迫以及手术可能产生的影响,术前及术后均要检查下丘脑垂体轴、肾上腺功能及水、电解质平衡等。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "手术" }, { "start_idx": 40, "end_idx": 45, "type": "bod", "entity": "下丘脑垂体轴" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "肾上腺" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "水" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "电解质平衡" } ] }, { "text": "治疗原则是能够完全切除的肿瘤应尽量完整切除;体积大的肿瘤或与周围组织粘连严重时可做部分切除,术后辅以局部放射治疗;大的囊性单腔性颅咽管瘤可用32P行内放疗;而对于小的2~3cm的肿瘤可行立体定向放射外科治疗。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肿瘤" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "切除" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "肿瘤" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "部分切除" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "放射" }, { "start_idx": 59, "end_idx": 67, "type": "dis", "entity": "囊性单腔性颅咽管瘤" }, { "start_idx": 81, "end_idx": 82, "type": "pro", "entity": "放疗" }, { "start_idx": 95, "end_idx": 96, "type": "dis", "entity": "肿瘤" }, { "start_idx": 99, "end_idx": 104, "type": "pro", "entity": "立体定向放射" }, { "start_idx": 105, "end_idx": 106, "type": "dep", "entity": "外科" } ] }, { "text": "死亡主要与肿瘤复发或慢性神经内分泌功能紊乱慢性神经内分泌功能紊乱有关。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" }, { "start_idx": 10, "end_idx": 20, "type": "dis", "entity": "慢性神经内分泌功能紊乱" } ] }, { "text": "十、肺部其他病变(一)特发性肺含铁血黄素沉着症两肺野透亮度对称性减低,可呈毛玻璃状改变,内有细小颗粒状阴影;出血多时,呈现薄片状阴影,以肺门区为明显;静止期以颗粒状阴影为主。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肺部" }, { "start_idx": 11, "end_idx": 22, "type": "dis", "entity": "特发性肺含铁血黄素沉着症" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肺野" }, { "start_idx": 23, "end_idx": 52, "type": "sym", "entity": "两肺野透亮度对称性减低,可呈毛玻璃状改变,内有细小颗粒状阴影" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "肺门" }, { "start_idx": 54, "end_idx": 73, "type": "sym", "entity": "出血多时,呈现薄片状阴影,以肺门区为明显" }, { "start_idx": 75, "end_idx": 85, "type": "sym", "entity": "静止期以颗粒状阴影为主" } ] }, { "text": "(二)朗格汉斯组织细胞增生症弥漫性颗粒状阴影为最典型的表现,其他有片状、网状及囊泡样透亮区。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "朗格汉斯组织细胞增生症" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "弥漫性颗粒状阴影" }, { "start_idx": 33, "end_idx": 44, "type": "sym", "entity": "片状、网状及囊泡样透亮区" } ] }, { "text": "(三)系统性红斑狼疮肺部表现以小片状病灶,病变一侧或两侧,肺底部多见,可反复发作,在间质性肺炎时出现网状影,大多数患者出现胸膜反应与渗出。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肺部" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "小片状病灶" }, { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "病变一侧或两侧" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "肺" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "肺底部多见" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "间质性肺炎" }, { "start_idx": 50, "end_idx": 52, "type": "sym", "entity": "网状影" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "胸膜反应" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "渗出" } ] }, { "text": "支气管腔、病理改变气道黏膜充血、水肿,上皮细胞脱落、崩解;黏膜杯状细胞增多,黏液腺增生;包括炎性细胞(嗜酸性粒细胞、肥大细胞、T淋巴细胞、中性粒细胞等)、气道结构细胞(气道平滑肌细胞和上皮细胞等)明显增多;支气管平滑肌肥厚,基底膜变厚,使支气管壁增厚,重建;支气管腔内可见黏液或黏液栓,引起肺泡膨胀,过度充气或肺不张。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "支气管腔" }, { "start_idx": 9, "end_idx": 17, "type": "sym", "entity": "气道黏膜充血、水肿" }, { "start_idx": 19, "end_idx": 27, "type": "sym", "entity": "上皮细胞脱落、崩解" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "黏膜杯状细胞增多" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "黏液腺增生" }, { "start_idx": 103, "end_idx": 105, "type": "bod", "entity": "支气管" }, { "start_idx": 119, "end_idx": 122, "type": "bod", "entity": "支气管壁" }, { "start_idx": 129, "end_idx": 132, "type": "bod", "entity": "支气管腔" }, { "start_idx": 145, "end_idx": 148, "type": "dis", "entity": "肺泡膨胀" }, { "start_idx": 150, "end_idx": 153, "type": "dis", "entity": "过度充气" }, { "start_idx": 155, "end_idx": 157, "type": "dis", "entity": "肺不张" } ] }, { "text": "三、囊状淋巴管瘤囊状淋巴管瘤(cysticlymphangioma)又称囊状水瘤。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "囊状淋巴管瘤" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "囊状淋巴管瘤" }, { "start_idx": 15, "end_idx": 32, "type": "dis", "entity": "cysticlymphangioma" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "囊状水瘤" } ] }, { "text": "为淋巴性起源的含液病变,呈圆形、椭圆形,可分叶,囊内壁衬有上皮层,光滑透明,血管少。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "dis", "entity": "淋巴性起源的含液病变" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "囊" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "上皮层" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "血管" } ] }, { "text": "肿瘤的体积变异很大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" } ] }, { "text": "治疗包括:1.药物注射疗法近年来用抗肿瘤药物博来霉素作瘤内注射治疗,可使70%的囊状水瘤完全消失或显著缩小。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "药物注射疗法" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "博来霉素" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "瘤内注射治疗" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "囊状水瘤" } ] }, { "text": "作用机制可能是抑制淋巴管内皮细胞的生长,使间质纤维化。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "淋巴管" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "间质" } ] }, { "text": "用法:将博来霉素水剂,配成1mg/1ml的浓度,每次0.2~0.3mg/kg的剂量注入瘤体内,每周一次,3~10次为一疗程,总剂量不超过5mg/kg,否则可导致组织坏死,发生溃疡。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "博来霉素水剂" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "瘤体" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "组织" }, { "start_idx": 87, "end_idx": 88, "type": "dis", "entity": "溃疡" } ] }, { "text": "用法:0.1mgOK-432溶于10ml生理盐水中,抽取多少淋巴液,注射多少OK-432溶液,每次总量不超过0.3mg。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dru", "entity": "OK-432" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "生理盐水" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "淋巴液" }, { "start_idx": 38, "end_idx": 43, "type": "dru", "entity": "OK-432" } ] }, { "text": "2.手术疗法药物注射应该首选,如注射效果不佳或复发,手术是必要的。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "手术疗法" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "药物注射" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "注射" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "手术" } ] }, { "text": "手术解剖要细致,囊壁菲薄,如果破裂则很难找到其边缘,另外尽量避免损伤临近的重要的血管、神经。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "手术解剖" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "囊壁" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "血管" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "神经" } ] }, { "text": "残留的囊壁用1%碘酒涂擦。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "囊壁" }, { "start_idx": 8, "end_idx": 9, "type": "dru", "entity": "碘酒" } ] }, { "text": "个别巨大肿瘤,可分期手术。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "巨大肿瘤" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "手术" } ] }, { "text": "第二章消化道疾病的诊断技术第一节内镜检查消化道纤维(电子)内镜是当今诊断与治疗食管、胃、小肠、大肠及胰胆管腔道疾病最先进而有效的仪器。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "内镜检查" }, { "start_idx": 20, "end_idx": 30, "type": "equ", "entity": "消化道纤维(电子)内镜" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "食管" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "胃" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "小肠" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "大肠" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "胰胆管腔道" } ] }, { "text": "而内镜检查是一种直观、准确、敏感的胃肠疾病检查手段。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "内镜检查" } ] }, { "text": "自20世纪70年代初期,细径化、可屈式的纤维胃镜进入儿科领域后,儿童消化系统腔道疾病的诊断水平得到了很大的提高。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "equ", "entity": "纤维胃镜" }, { "start_idx": 32, "end_idx": 41, "type": "dis", "entity": "儿童消化系统腔道疾病" } ] }, { "text": "近20年来,随着消化内镜在儿科应用的普及及内镜医疗器械的进步,高难度操作技术的开展,儿科消化内镜学正迅速向治疗内镜学发展。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "equ", "entity": "消化内镜" }, { "start_idx": 13, "end_idx": 14, "type": "dep", "entity": "儿科" }, { "start_idx": 42, "end_idx": 43, "type": "dep", "entity": "儿科" } ] }, { "text": "一、胃镜检查(一)胃镜类型及儿童应用选择纤维胃镜类型很多按其先端部的形态和物镜的部位,分为三种类型:①直视式:接物镜在镜的前端,可观察食管、胃和十二指肠,但胃小弯不满意;②侧视式:接物镜在镜的前端侧面,多用于逆行胰胆管造影检查;③斜视式:接物镜向上斜30°,能较满意观察上消化道病变。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "胃镜检查" }, { "start_idx": 20, "end_idx": 23, "type": "equ", "entity": "纤维胃镜" }, { "start_idx": 55, "end_idx": 57, "type": "equ", "entity": "接物镜" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "食管" }, { "start_idx": 70, "end_idx": 70, "type": "bod", "entity": "胃" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "十二指肠" }, { "start_idx": 90, "end_idx": 92, "type": "equ", "entity": "接物镜" }, { "start_idx": 104, "end_idx": 112, "type": "pro", "entity": "逆行胰胆管造影检查" }, { "start_idx": 119, "end_idx": 121, "type": "equ", "entity": "接物镜" } ] }, { "text": "儿科临床应用较多的是直视式胃镜。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dep", "entity": "儿科" }, { "start_idx": 10, "end_idx": 14, "type": "equ", "entity": "直视式胃镜" } ] }, { "text": "适用于儿童的胃镜有两类:①小儿胃镜:专为儿童设计的直视式胃镜,如FujinonFG-100PE及PentaxFG-24X,该镜特点是镜身纤细,外径仅7~9mm,有效工作长度是1050mm,操作灵活方便,受检者痛苦少。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "equ", "entity": "小儿胃镜" }, { "start_idx": 25, "end_idx": 29, "type": "equ", "entity": "直视式胃镜" }, { "start_idx": 32, "end_idx": 46, "type": "equ", "entity": "FujinonFG-100PE" }, { "start_idx": 48, "end_idx": 59, "type": "equ", "entity": "PentaxFG-24X" } ] }, { "text": "②成人细径胃镜:外径在9.0~9.8mm范围,活检孔较大,适用于年长儿童及内镜治疗。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "equ", "entity": "成人细径胃镜" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "内镜治疗" } ] }, { "text": "对早产婴儿,文献报道,可用直径3.55mm的纤维支气管镜代替胃镜。", "entities": [ { "start_idx": 22, "end_idx": 27, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 30, "end_idx": 31, "type": "equ", "entity": "胃镜" } ] }, { "text": "(二)胃镜检查(gastroscopy)适应证及禁忌证1.适应证(1)反复发作性腹痛:尤为上腹痛,脐周痛;有半夜痛醒史;腹痛伴体重减轻;腹痛伴黑便;腹痛经驱虫及一般治疗无效者。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "胃镜检查" }, { "start_idx": 8, "end_idx": 18, "type": "pro", "entity": "gastroscopy" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "反复发作性腹痛" }, { "start_idx": 45, "end_idx": 47, "type": "sym", "entity": "上腹痛" }, { "start_idx": 49, "end_idx": 51, "type": "sym", "entity": "脐周痛" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "有半夜痛醒史" }, { "start_idx": 60, "end_idx": 66, "type": "sym", "entity": "腹痛伴体重减轻" }, { "start_idx": 68, "end_idx": 72, "type": "sym", "entity": "腹痛伴黑便" } ] }, { "text": "(3)经常性呕吐:呕吐伴出血,呕吐伴上腹痛,呕吐伴体重减轻者。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "经常性呕吐" }, { "start_idx": 9, "end_idx": 29, "type": "sym", "entity": "呕吐伴出血,呕吐伴上腹痛,呕吐伴体重减轻者" } ] }, { "text": "(5)消化不良:经常性腹胀、嗳气及纳呆,经一般治疗无效。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "消化不良" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "经常性腹胀、嗳气及纳呆" } ] }, { "text": "(7)不能用心肺疾病解释的胸闷及胸骨后疼痛。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "心肺疾病" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "胸闷" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "胸骨后疼痛" } ] }, { "text": "2.禁忌证有严重的心肺疾病不能耐受者;疑似胃、十二指肠穿孔者;处于休克、昏迷等严重状态;有发热、急性咽喉炎及肺部感染;吞食腐蚀物的急性期,癫痫反复发作等,均被列为儿童胃镜检查禁忌证。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "心肺疾病" }, { "start_idx": 19, "end_idx": 28, "type": "sym", "entity": "疑似胃、十二指肠穿孔" }, { "start_idx": 31, "end_idx": 42, "type": "sym", "entity": "处于休克、昏迷等严重状态" }, { "start_idx": 44, "end_idx": 57, "type": "sym", "entity": "有发热、急性咽喉炎及肺部感染" }, { "start_idx": 59, "end_idx": 75, "type": "sym", "entity": "吞食腐蚀物的急性期,癫痫反复发作等" }, { "start_idx": 81, "end_idx": 86, "type": "pro", "entity": "儿童胃镜检查" } ] }, { "text": "(三)胃镜检查的并发症及其预防纤维或电子胃镜检查是一项比较安全的技术,根据日本87万次成人胃镜检查资料,严重并发症发生率仅0.003%~0.044%。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "胃镜检查" }, { "start_idx": 15, "end_idx": 23, "type": "pro", "entity": "纤维或电子胃镜检查" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "胃镜检查" } ] }, { "text": "其发生类型主要有以下几种:①麻醉意外:多系全麻引起的药物过敏、气管痉挛及短暂性呼吸抑制等反应;②插管损伤:主要是操作者不熟练,强行插管引起;③其他如低氧血症、吸入窒息及胃肠穿孔也有报道,但罕见。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "全麻" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "药物过敏" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "气管痉挛" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "短暂性呼吸抑制" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "低氧血症" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "吸入窒息" }, { "start_idx": 84, "end_idx": 87, "type": "dis", "entity": "胃肠穿孔" } ] }, { "text": "预防的关键是尽量少使用全麻,已经有报道非麻醉下胃镜检查的时间仅是麻醉下的一半,且安全无麻醉意外。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "全麻" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "胃镜检查" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "麻醉" } ] }, { "text": "此外胃镜检查必须有专人进行,操作者动作必须熟练、轻柔、仔细,切不可盲目粗暴。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "胃镜检查" } ] }, { "text": "在胃镜检查过程中有专人护士给予安抚。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "胃镜检查" } ] }, { "text": "(四)胃镜检查术前准备及术后处理1.术前准备(1)检查所有器械是否完整,安装是否确切。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "胃镜检查" } ] }, { "text": "(4)检查前一天晚8点后禁食、禁水、禁药;哺乳期婴儿术前禁奶6小时,幽门梗阻患儿禁食3天。", "entities": [ { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "幽门梗阻" } ] }, { "text": "2.术前用药(1)除婴儿外术前均用2%丁卡因溶液做咽部麻醉,婴幼儿或精神紧张的年长儿术前15分钟给予10%水合氯醛0.5ml/kg保留灌肠或地西泮0.1~0.3mg/kg肌内注射,也可采用全身麻醉,氯胺酮4~6mg/kg,肌内注射,但须麻醉医师参与。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "丁卡因" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "咽部" }, { "start_idx": 53, "end_idx": 56, "type": "dru", "entity": "水合氯醛" }, { "start_idx": 67, "end_idx": 68, "type": "pro", "entity": "灌肠" }, { "start_idx": 70, "end_idx": 72, "type": "dru", "entity": "地西泮" }, { "start_idx": 85, "end_idx": 88, "type": "pro", "entity": "肌内注射" }, { "start_idx": 94, "end_idx": 97, "type": "pro", "entity": "全身麻醉" }, { "start_idx": 99, "end_idx": 101, "type": "dru", "entity": "氯胺酮" }, { "start_idx": 111, "end_idx": 114, "type": "pro", "entity": "肌内注射" } ] }, { "text": "(2)术前5分钟口服祛泡剂。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "祛泡剂" } ] }, { "text": "(3)阿托品0.01~0.015mg/kg,肌内注射。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "阿托品" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "肌内注射" } ] }, { "text": "3.术后处理胃镜术后应留观半小时,适当休息1天;术后禁食1小时,取活体组织检查者进流汁饮食1天,并嘱咐家长若有消化道出血,便来院急诊。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "胃镜" }, { "start_idx": 55, "end_idx": 59, "type": "sym", "entity": "消化道出血" } ] }, { "text": "(五)胃镜操作方法及注意事项1.患儿左侧卧位,松开领扣及裤带,双下肢屈曲,助手扶持患儿头部,把紧患儿口中牙垫。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "胃镜" } ] }, { "text": "2.插镜采用单人或双人插镜法,术者左手持操作部,并调节角度钮使胃镜前端稍向下弯曲,右手拿胃镜可屈部,通过牙垫轻轻插入咽部,让患者做吞咽动作,此时,右手将胃镜往下送,便可顺利到达食管上段。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "插镜" }, { "start_idx": 31, "end_idx": 32, "type": "equ", "entity": "胃镜" }, { "start_idx": 44, "end_idx": 45, "type": "equ", "entity": "胃镜" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "咽部" }, { "start_idx": 76, "end_idx": 77, "type": "equ", "entity": "胃镜" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "食管" } ] }, { "text": "3.当镜身通过咽喉部后,即应在直视下操作。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "咽喉部" } ] }, { "text": "依次自食管、贲门、胃体、胃窦直至幽门,进入球内观察,注气应适量,必要时将过多气体吸出。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "食管" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "贲门" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胃体" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胃窦" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "幽门" } ] }, { "text": "4.在退镜过程中,应上下、左右方向依次仔细观察胃内各部,特别是胃窦小弯后壁及胃角,对胃体、胃底及贲门部至少应采用高位翻转及正面两次观察。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "退镜" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "胃" }, { "start_idx": 31, "end_idx": 36, "type": "bod", "entity": "胃窦小弯后壁" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "胃角" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "胃体" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "胃底" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "贲门部" } ] }, { "text": "5.胃镜退出贲门时,应吸出胃内气体,然后退入食管观察,直至全部退出。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "equ", "entity": "胃镜" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "贲门" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "胃内气体" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "食管" } ] }, { "text": "(六)上消化道常见病变的胃镜诊断1.正常胃黏膜正常小儿胃黏液较成人更为细嫩柔软呈半透明状,表面有少量晶状稀薄黏液,使其湿润而光滑,色泽视血色素高低而不同,一般呈橘红色。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "胃镜诊断" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "小儿胃黏液" } ] }, { "text": "胃镜刚入胃腔时,可见到许多皱襞,呈曲折蜿蜒状态,自贲门沿着胃的长轴,走行至幽门部,注气量增多时,皱襞变平或消失。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "胃镜" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "胃腔" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "胃" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "幽门部" } ] }, { "text": "2.食管炎(esophagitis)儿童食管炎占胃镜检查的10%~12%,最常见的是反流性食管炎。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "食管炎" }, { "start_idx": 6, "end_idx": 16, "type": "dis", "entity": "esophagitis" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "食管炎" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "胃镜检查" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "反流性食管炎" } ] }, { "text": "据报道儿童食管炎中74%有胃食管反流。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "食管炎" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "胃食管反流" } ] }, { "text": "所以食管炎的发病机制多认为与食管下段括约肌功能不全以及酸清除能力下降有关。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "食管炎" }, { "start_idx": 14, "end_idx": 24, "type": "dis", "entity": "食管下段括约肌功能不全" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "酸清除能力下降" } ] }, { "text": "1992年消化内镜学会在通化市拟定了反流性食管炎的内镜诊断标准如下:①轻度:红色条纹和红斑,累及食管下1/3糜烂<1/2食管圆周,仅累及食管中、下段Ⅰ级:糜烂累及>1/2食管圆周,或已累及上段,或形成溃疡<1/3食管圆周,在食管任何部位;Ⅱ级:溃疡累及>1/3圆周食管,任何部位。", "entities": [ { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "反流性食管炎" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "食管" }, { "start_idx": 38, "end_idx": 53, "type": "sym", "entity": "红色条纹和红斑,累及食管下1/3" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "食管" }, { "start_idx": 54, "end_idx": 73, "type": "sym", "entity": "糜烂<1/2食管圆周,仅累及食管中、下段" }, { "start_idx": 74, "end_idx": 117, "type": "sym", "entity": "Ⅰ级:糜烂累及>1/2食管圆周,或已累及上段,或形成溃疡<1/3食管圆周,在食管任何部位" } ] }, { "text": "3.慢性胃炎慢性胃炎内镜表现和悉尼系统。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "慢性胃炎" } ] }, { "text": "(1)内镜黏膜形态学:1)充血:与邻区比较,黏膜色泽呈斑块状、线状或弥漫性鲜红。", "entities": [ { "start_idx": 13, "end_idx": 38, "type": "sym", "entity": "充血:与邻区比较,黏膜色泽呈斑块状、线状或弥漫性鲜红" } ] }, { "text": "2)水肿:黏膜肿胀,色泽较苍白,反光增强,胃小区轮廓更明显。", "entities": [ { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "胃" }, { "start_idx": 2, "end_idx": 28, "type": "sym", "entity": "水肿:黏膜肿胀,色泽较苍白,反光增强,胃小区轮廓更明显" } ] }, { "text": "4)花纹状:黏膜红白相间,红点与红点之间黏膜较苍白,似麻疹样改变。", "entities": [ { "start_idx": 2, "end_idx": 31, "type": "sym", "entity": "花纹状:黏膜红白相间,红点与红点之间黏膜较苍白,似麻疹样改变" } ] }, { "text": "5)微小结节形成:胃窦平坦时,黏膜呈微细或粗颗粒状、结节状。", "entities": [ { "start_idx": 2, "end_idx": 28, "type": "sym", "entity": "微小结节形成:胃窦平坦时,黏膜呈微细或粗颗粒状、结节状" } ] }, { "text": "6)糜烂:局散性或散在分布,可伴有新鲜或陈旧出血点。", "entities": [ { "start_idx": 2, "end_idx": 24, "type": "sym", "entity": "糜烂:局散性或散在分布,可伴有新鲜或陈旧出血点" } ] }, { "text": "糜烂位于黏液层内为平坦型糜烂。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "糜烂位于黏液层内为平坦型糜烂" } ] }, { "text": "高于黏膜面称隆起型糜烂,隆起呈圆形或椭圆形,顶部呈脐样凹陷。", "entities": [ { "start_idx": 0, "end_idx": 28, "type": "sym", "entity": "高于黏膜面称隆起型糜烂,隆起呈圆形或椭圆形,顶部呈脐样凹陷" } ] }, { "text": "7)出血斑点:黏膜散在分布点状或小片状新鲜或陈旧出血。", "entities": [ { "start_idx": 2, "end_idx": 25, "type": "sym", "entity": "出血斑点:黏膜散在分布点状或小片状新鲜或陈旧出血" } ] }, { "text": "(2)胃炎的悉尼系统分类:1990年8月在澳大利亚悉尼召开的国际胃肠病学学术会上,对慢性胃炎制订了一整套的分类和诊断方法,称为悉尼系统。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "胃炎" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "慢性胃炎" } ] }, { "text": "该系统强调内镜和病理密切结合,胃炎的诊断要包括组织学和内镜两部分。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "equ", "entity": "内镜" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "胃炎" } ] }, { "text": "组织学的核心是解决急性胃炎、慢性胃炎还是特殊类型胃炎以及炎症的部位,并尽可能找到病因或相关的病原,以及炎症的程度、活动性、萎缩程度、肠化分级、有无幽门螺杆菌等。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "急性胃炎" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "胃炎" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "幽门螺杆菌" } ] }, { "text": "4.十二指肠炎(duodenitis)十二指肠炎常多相伴其他部位的炎症,内镜下黏膜炎症的改变有四种类型:(1)充血型:黏膜充血、水肿,镜下反光增强。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "十二指肠炎" }, { "start_idx": 8, "end_idx": 17, "type": "dis", "entity": "duodenitis" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "十二指肠" }, { "start_idx": 36, "end_idx": 37, "type": "equ", "entity": "内镜" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "黏膜炎症" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "充血型" }, { "start_idx": 59, "end_idx": 72, "type": "sym", "entity": "黏膜充血、水肿,镜下反光增强" } ] }, { "text": "(2)肿胀型:黏膜水肿肥厚呈不规则形隆起,表面充血。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "肿胀型" }, { "start_idx": 7, "end_idx": 24, "type": "sym", "entity": "黏膜水肿肥厚呈不规则形隆起,表面充血" } ] }, { "text": "(3)颗粒型:黏膜呈滤泡样或颗粒状或结节状隆起,大小较均匀。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "颗粒型" }, { "start_idx": 7, "end_idx": 28, "type": "sym", "entity": "黏膜呈滤泡样或颗粒状或结节状隆起,大小较均匀" } ] }, { "text": "(4)出血糜烂型:黏膜充血处见点状、片状或蜂窝状糜烂,表面可有出血。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "出血糜烂型" }, { "start_idx": 9, "end_idx": 32, "type": "sym", "entity": "黏膜充血处见点状、片状或蜂窝状糜烂,表面可有出血" } ] }, { "text": "5.胃十二指肠溃疡溃疡在胃镜中所见为橘红色,黏膜上有鲜明的白色及灰白色病变,一般为圆形及椭圆形,边缘清楚,周围黏液在同一平面或仅轻微隆起,溃疡底面光滑,被有白色或灰白色苔状物,有时呈褐色,表示有血色素沉着,多数为单个溃疡,少数为多发性。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "胃十二指肠溃疡" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "溃疡" }, { "start_idx": 12, "end_idx": 13, "type": "equ", "entity": "胃镜" } ] }, { "text": "溃疡病分期:(1)活动期(activestage;A期):又称厚苔膜期。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "溃疡病" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "活动期" }, { "start_idx": 13, "end_idx": 23, "type": "dis", "entity": "activestage" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "A期" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "厚苔膜期" } ] }, { "text": "A1:溃疡苔厚而污秽,边缘肿胀,无皱襞集中,有较多分泌物;A2:溃疡苔膜厚而清洁,边缘肿胀逐渐消失,四周出现上皮再生形成的红晕,皱襞开始向溃疡集中。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "A1" }, { "start_idx": 3, "end_idx": 27, "type": "sym", "entity": "溃疡苔厚而污秽,边缘肿胀,无皱襞集中,有较多分泌物" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "A2" }, { "start_idx": 32, "end_idx": 72, "type": "sym", "entity": "溃疡苔膜厚而清洁,边缘肿胀逐渐消失,四周出现上皮再生形成的红晕,皱襞开始向溃疡集中" } ] }, { "text": "(2)愈合期(healingstage;H期):又称薄苔膜期。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "愈合期" }, { "start_idx": 7, "end_idx": 18, "type": "dis", "entity": "healingstage" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "H期" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "薄苔膜期" } ] }, { "text": "H1:苔膜变薄,溃疡缩小,周围有上皮再生;H2:接近愈合,但溃疡面仍有极少白薄苔。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "H1" }, { "start_idx": 3, "end_idx": 19, "type": "sym", "entity": "苔膜变薄,溃疡缩小,周围有上皮再生" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "H2" }, { "start_idx": 24, "end_idx": 39, "type": "sym", "entity": "接近愈合,但溃疡面仍有极少白薄苔" } ] }, { "text": "(3)瘢痕期(scarringstage;S期):又称无苔期。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "瘢痕期" }, { "start_idx": 7, "end_idx": 19, "type": "dis", "entity": "scarringstage" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "S期" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "无苔期" } ] }, { "text": "S1:溃疡白苔已消失,中央充血呈红色,又称红色瘢痕期;S2:中央充血已完全消退,变为白色瘢痕,亦称白色瘢痕期。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "S1" }, { "start_idx": 3, "end_idx": 17, "type": "sym", "entity": "溃疡白苔已消失,中央充血呈红色" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "红色瘢痕期" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "S2" }, { "start_idx": 30, "end_idx": 45, "type": "sym", "entity": "中央充血已完全消退,变为白色瘢痕" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "白色瘢痕期" } ] }, { "text": "四周有黏液纹辐射,表示溃疡已完全愈合。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "四周有黏液纹辐射,表示溃疡已完全愈合" } ] }, { "text": "儿童期溃疡以十二指肠多见,幼小儿童多见胃溃疡。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "溃疡" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "十二指肠" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "胃溃疡" } ] }, { "text": "十二指肠球部溃疡,大多位于前壁大弯侧,特点是溃疡病灶浅,愈合快,部分患儿不需治疗,常可自愈,溃疡灶周边充血、水肿显著,消退缓慢。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "十二指肠球部溃疡" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "前壁大弯侧" }, { "start_idx": 22, "end_idx": 30, "type": "sym", "entity": "溃疡病灶浅,愈合快" }, { "start_idx": 32, "end_idx": 44, "type": "pro", "entity": "部分患儿不需治疗,常可自愈" }, { "start_idx": 46, "end_idx": 62, "type": "sym", "entity": "溃疡灶周边充血、水肿显著,消退缓慢" } ] }, { "text": "胃镜检查常可发现溃疡已愈合,但充血,水肿仍明显的球炎状态。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "胃镜检查" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "溃疡" }, { "start_idx": 18, "end_idx": 27, "type": "sym", "entity": "水肿仍明显的球炎状态" } ] }, { "text": "所以有作者认为十二指肠球部溃疡是球炎的发展,而球炎是溃疡修复中的一个表现。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "十二指肠球部溃疡" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "球炎" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "球炎" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "溃疡" } ] }, { "text": "另在十二指肠球部溃疡中可见到下述两种特殊类型:①十二指肠多发性溃疡;前后壁各有一溃疡,中间有桥状皱襞连接,也称对口(接吻)溃疡;②霜斑样溃疡:表现一片充血的黏膜上,覆盖散在的、细微的小白斑或小白苔,给人以下过霜一样的外貌。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "二指肠球部溃疡" }, { "start_idx": 24, "end_idx": 32, "type": "dis", "entity": "十二指肠多发性溃疡" }, { "start_idx": 34, "end_idx": 51, "type": "sym", "entity": "前后壁各有一溃疡,中间有桥状皱襞连接" }, { "start_idx": 55, "end_idx": 62, "type": "dis", "entity": "对口(接吻)溃疡" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "霜斑样溃疡" }, { "start_idx": 71, "end_idx": 109, "type": "sym", "entity": "表现一片充血的黏膜上,覆盖散在的、细微的小白斑或小白苔,给人以下过霜一样的外貌" } ] }, { "text": "第六节过敏性紫癜肾炎过敏性紫癜(anaphylactoidpurpura,AP)(henochschonleinpurpura,HSP)是以皮肤紫癜、出血性胃肠炎、关节炎及肾小球肾炎为主要特点的临床综合征。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "过敏性紫癜肾炎" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "过敏性紫癜" }, { "start_idx": 16, "end_idx": 35, "type": "dis", "entity": "anaphylactoidpurpura" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "AP" }, { "start_idx": 41, "end_idx": 62, "type": "dis", "entity": "henochschonleinpurpura" }, { "start_idx": 64, "end_idx": 66, "type": "dis", "entity": "HSP" }, { "start_idx": 70, "end_idx": 73, "type": "dis", "entity": "皮肤紫癜" }, { "start_idx": 75, "end_idx": 80, "type": "dis", "entity": "出血性胃肠炎" }, { "start_idx": 82, "end_idx": 84, "type": "dis", "entity": "关节炎" }, { "start_idx": 86, "end_idx": 90, "type": "dis", "entity": "肾小球肾炎" } ] }, { "text": "HSP患儿中约有一半出现肾损害,此时称过敏性紫癜肾炎(henochschonleinpurpuranephritis,HSPN)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "HSP" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "肾损害" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "过敏性紫癜肾炎" }, { "start_idx": 27, "end_idx": 57, "type": "dis", "entity": "henochschonleinpurpuranephritis" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "HSPN" } ] }, { "text": "【病因及发病机制】HSP是白细胞碎裂性小血管炎,是主要由IgA免疫复合物沉积引起的免疫复合物病,其病因仍未完全明了,可能与下列因素有关:感染、疫苗接种、虫咬、寒冷刺激、药物过敏和食物过敏等。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "HSP" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "白细胞碎裂性小血管炎" }, { "start_idx": 28, "end_idx": 37, "type": "sym", "entity": "IgA免疫复合物沉积" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "免疫复合物病" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "感染" }, { "start_idx": 71, "end_idx": 74, "type": "pro", "entity": "疫苗接种" }, { "start_idx": 84, "end_idx": 87, "type": "sym", "entity": "药物过敏" }, { "start_idx": 89, "end_idx": 92, "type": "sym", "entity": "食物过敏" } ] }, { "text": "尽管这些因素都可能诱发HSP,但临床上仍难明确过敏原,脱敏治疗的效果往往难以令人满意。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "HSP" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "脱敏治疗" } ] }, { "text": "鉴于HSPN免疫病理的显著特点是系膜区颗粒状IgA沉积,与IgA肾病改变极为相似,因此推测IgA在发病中有重要作用,甚至有人认为它们本质上是同一种疾病。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "HSPN" }, { "start_idx": 16, "end_idx": 26, "type": "sym", "entity": "系膜区颗粒状IgA沉积" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "IgA" } ] }, { "text": "进一步研究发现两者免疫发病机制确有惊人的一致性,如均有血清IgA升高、单体以及多聚体IgA升高、λ-IgA1升高,两者血清中均有循环IgA免疫复合物;沉积在肾小球上的均以多聚IgA1为主,且有J链沉积;两者都有C4a、C4b亚型缺陷,都有IgA1O型糖基化异常等等。", "entities": [ { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "血清IgA升高" }, { "start_idx": 35, "end_idx": 46, "type": "sym", "entity": "单体以及多聚体IgA升高" }, { "start_idx": 48, "end_idx": 55, "type": "sym", "entity": "λ-IgA1升高" }, { "start_idx": 59, "end_idx": 73, "type": "sym", "entity": "血清中均有循环IgA免疫复合物" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "肾小球" }, { "start_idx": 75, "end_idx": 103, "type": "sym", "entity": "沉积在肾小球上的均以多聚IgA1为主" }, { "start_idx": 107, "end_idx": 110, "type": "sym", "entity": "J链沉积" }, { "start_idx": 116, "end_idx": 148, "type": "sym", "entity": "C4a、C4b亚型缺陷" }, { "start_idx": 152, "end_idx": 173, "type": "sym", "entity": "IgA1O型糖基化异常" } ] }, { "text": "华中科技大学同济医学院附属同济医院儿科对120例HSPN及31例IgA肾病进行了比较研究,发现HSPN6.3%有IgG沿肾小球毛细血管壁的线样沉积以及膜抗肾小球基底抗体阳性,而且12.5%不是以IgA为主要沉积物,因此,至少在一部分HSPN中,其发病机制与IgA肾病显著不同。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "HSPN" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "HSPN" }, { "start_idx": 60, "end_idx": 67, "type": "bod", "entity": "肾小球毛细血管壁" }, { "start_idx": 56, "end_idx": 72, "type": "sym", "entity": "IgG沿肾小球毛细血管壁的线样沉积" }, { "start_idx": 75, "end_idx": 85, "type": "sym", "entity": "膜抗肾小球基底抗体阳性" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "IgA" }, { "start_idx": 116, "end_idx": 119, "type": "dis", "entity": "HSPN" }, { "start_idx": 128, "end_idx": 132, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "HSPN的肾脏损伤中补体发挥重要作用,补体的激活可能是通过旁路途径实现的:①IgA无激活C1q的能力,而能直接激活C3;②在在肾小球系膜区证实有C3、备解素C3PA,而无C1q及C4;③C2缺乏的病人易患本病。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "HSPN" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "肾脏损伤" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "补体" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "补体" }, { "start_idx": 85, "end_idx": 90, "type": "bod", "entity": "肾小球系膜区" }, { "start_idx": 84, "end_idx": 156, "type": "sym", "entity": "在肾小球系膜区证实有C3、备解素C3PA,而无C1q及C4" }, { "start_idx": 159, "end_idx": 173, "type": "sym", "entity": "C2缺乏" } ] }, { "text": "【病理】HSPN的基本病理改变为肾小球系膜增生和系膜区IgA沉积,严重时尚有新月体形成和肾小管坏死,病理改变轻重差别很大,国际小儿肾脏病研究会根据光镜下肾小球的改变将HSPN分为6型:Ⅰ:肾小球轻微改变。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "HSPN" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "肾小球系膜增生" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "系膜区IgA沉积" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "有新月体形成" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "肾小管" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "肾小管坏死" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "小儿肾脏病" }, { "start_idx": 73, "end_idx": 74, "type": "equ", "entity": "光镜" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "肾小球" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "HSPN" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "肾小球" }, { "start_idx": 94, "end_idx": 100, "type": "sym", "entity": "肾小球轻微改变" } ] }, { "text": "Ⅱ:单纯性系膜增生,不伴毛细血管袢局灶性改变及新月体:Ⅱa:局灶节段性系膜增生;Ⅱb:弥漫性系膜增生。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "毛细血管" }, { "start_idx": 2, "end_idx": 25, "type": "sym", "entity": "单纯性系膜增生,不伴毛细血管袢局灶性改变及新月体" }, { "start_idx": 30, "end_idx": 38, "type": "sym", "entity": "局灶节段性系膜增生" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "弥漫性系膜增生" } ] }, { "text": "Ⅲ:系膜增生并出现局灶改变(血栓、坏死、新月体及硬化):Ⅲa:局灶性改变;Ⅲb:新月体出现,但<50%。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "sym", "entity": "系膜增生并出现局灶改变" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "血栓" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "坏死" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "新月体" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "硬化" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "局灶性改变" }, { "start_idx": 40, "end_idx": 50, "type": "sym", "entity": "新月体出现,但<50%" } ] }, { "text": "Ⅳ:系膜增生,50%~75%肾小球有新月体或局灶性改变:Ⅳa:局灶性病变;Ⅳb:新月体50%~75%。", "entities": [ { "start_idx": 2, "end_idx": 26, "type": "sym", "entity": "系膜增生,50%~75%肾小球有新月体或局灶性改变" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "局灶性病变" }, { "start_idx": 40, "end_idx": 49, "type": "sym", "entity": "新月体50%~75%" } ] }, { "text": "Ⅴ:>75%肾小球有新月体或局灶病变:Ⅴa:局灶性病变;Ⅴb:新月体。", "entities": [ { "start_idx": 2, "end_idx": 17, "type": "sym", "entity": ">75%肾小球有新月体或局灶病变" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "局灶性病变" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "新月体" } ] }, { "text": "Ⅵ:膜增生性肾小球肾炎。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "膜增生性肾小球肾炎" } ] }, { "text": "免疫荧光检查显示以系膜区团块状IgA沉积为主,可伴有C3、IgG和备解素,但荧光强度较IgA为弱。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "免疫荧光检查" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "系膜区" }, { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "系膜区团块状IgA沉积" }, { "start_idx": 49, "end_idx": 58, "type": "sym", "entity": "荧光强度较IgA为弱" } ] }, { "text": "即使光镜下病变呈局灶、节段分布,在免疫荧光镜下沉积物仍弥漫分布。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "光镜" }, { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "病变呈局灶、节段分布" }, { "start_idx": 17, "end_idx": 21, "type": "equ", "entity": "免疫荧光镜" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "沉积物仍弥漫分布" } ] }, { "text": "严重者延伸于毛细血管壁内。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "毛细血管壁" }, { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "延伸于毛细血管壁内" } ] }, { "text": "皮下及肠道小血管壁也常见IgA沉积。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "皮下及肠道小血管壁" }, { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "皮下及肠道小血管壁也常见IgA沉积" } ] }, { "text": "电镜下可见系膜细胞增生,系膜基质增多和系膜区大小不等的细颗粒状电子致密物沉积,沉积亦可在内皮下。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "电镜" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "系膜细胞" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "系膜细胞增生" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "系膜基质" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "系膜基质增多" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "系膜区" }, { "start_idx": 19, "end_idx": 37, "type": "sym", "entity": "系膜区大小不等的细颗粒状电子致密物沉积" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "内皮" } ] }, { "text": "免疫电镜证实沉积物中主要含IgA及少量C3和IgG。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "equ", "entity": "免疫电镜" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "IgA" }, { "start_idx": 19, "end_idx": 31, "type": "bod", "entity": "C3" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "IgG" } ] }, { "text": "这些改变轻者呈局灶性分布,重者呈弥漫性分布。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "局灶性分布" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "弥漫性分布" } ] }, { "text": "【临床表现】(一)肾外表现主要是过敏性紫癜所致的皮肤、胃肠及关节等方面的症状与体征。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肾" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "过敏性紫癜" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "皮肤" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "胃肠" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "关节" } ] }, { "text": "1.皮疹对称性分布于双下肢伸侧,严重时可波及臀部、下腹及肘部。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "皮疹" }, { "start_idx": 4, "end_idx": 14, "type": "sym", "entity": "对称性分布于双下肢伸侧" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "臀部" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "下腹" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肘部" }, { "start_idx": 16, "end_idx": 29, "type": "sym", "entity": "严重时可波及臀部、下腹及肘部" } ] }, { "text": "2.关节1/2~2/3病人出现关节肿痛,以膝关节和踝关节多见,活动可受限,一般数日内即可恢复。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "关节" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "关节" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "关节肿痛" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "膝关节" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "踝关节" } ] }, { "text": "3.胃肠道症状1/3病人出现阵发性腹部绞痛,脐周为主,可伴呕吐、黑便及呕血等,个别可出现肠梗阻、肠穿孔及肠套叠等。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "胃肠道" }, { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "胃肠道症状" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "腹部" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "阵发性腹部绞痛" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "脐周" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "呕吐、黑便及呕血" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "肠穿孔" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "肠套叠" } ] }, { "text": "4.其他如鼻出血、咯血及心肌炎,少数伴头痛和抽搐。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "鼻" }, { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "鼻出血" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "咯血" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "心肌炎" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "头痛" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "抽搐" } ] }, { "text": "(二)肾脏表现以血尿和蛋白尿为主。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "血尿" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "蛋白尿" } ] }, { "text": "1.血尿约一半病人出现肉眼血尿,均有镜下血尿。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "血尿" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "镜下血尿" } ] }, { "text": "2.蛋白尿程度不等。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "蛋白尿" } ] }, { "text": "3.水肿一般为轻~中度,非凹陷性,伴大量蛋白尿时可为凹陷性水肿。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "水肿" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "大量蛋白尿" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "凹陷性水肿" } ] }, { "text": "4.高血压。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "高血压" } ] }, { "text": "(三)其他表现HSP可累及中枢神经系统、心血管系统以及胸膜外分泌腺等而出现相应症状。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "HSP" }, { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "心血管系统" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "胸膜外分泌腺" } ] }, { "text": "【实验室检查】血常规及出凝血试验均可正常,ESR升高;IgA可升高,并可检出IgA类风湿因子。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "血常规" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "出凝血试验" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "ESR升高" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "IgA" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "IgA可升高" }, { "start_idx": 38, "end_idx": 45, "type": "bod", "entity": "IgA类风湿因子" } ] }, { "text": "其他如IgG、IgA及IgE均可增高或正常,血生化及肾功能可因临床表现类型的不同而正常或出现相应的异常改变。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "IgG" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "IgA" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "IgE" }, { "start_idx": 3, "end_idx": 20, "type": "sym", "entity": "IgG、IgA及IgE均可增高或正常" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "血生化" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "肾功能" } ] }, { "text": "尿液检查主要为血尿和蛋白尿,如有间质小管损害,可出现小分子蛋白如RBP、β2-微球蛋白及溶菌酶等增高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "尿液检查" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "血尿" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "间质小管损害" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "RBP" }, { "start_idx": 36, "end_idx": 53, "type": "bod", "entity": "β2-微球蛋白" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "溶菌酶" }, { "start_idx": 32, "end_idx": 60, "type": "sym", "entity": "RBP、β2-微球蛋白及溶菌酶等增高" } ] }, { "text": "【诊断和鉴别诊断】中华医学会儿科分会肾脏病学组于2000年11月珠海会议上制定的诊断标准为:(一)诊断标准在过敏性紫癜病程中(多数在6个月内),出现血尿和(或)蛋白尿。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "肾脏病" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "过敏性紫癜" }, { "start_idx": 74, "end_idx": 75, "type": "sym", "entity": "血尿" }, { "start_idx": 80, "end_idx": 82, "type": "sym", "entity": "蛋白尿" } ] }, { "text": "(二)临床分型1.孤立性血尿或孤立性蛋白尿。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "孤立性血尿" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "孤立性蛋白尿" } ] }, { "text": "2.血尿和蛋白尿。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "血尿" }, { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "蛋白尿" } ] }, { "text": "5.急进性肾炎型。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "急进性肾炎型" } ] }, { "text": "【治疗】(一)一般治疗急性期应卧床休息,如有明确过敏原,应脱敏治疗。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "脱敏治疗" } ] }, { "text": "有出血症状者应止血治疗,呕血者可静脉应用抗酸药物如甲氰米胍(cimetidine)10mg/(kg•d),或奥美拉唑(omeprazole,洛赛克,losec)每次0.3~0.5mg/kg,每日2次。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "出血症状" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "止血治疗" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "呕血" }, { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "静脉应用抗酸药物" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "甲氰米胍" }, { "start_idx": 30, "end_idx": 39, "type": "dru", "entity": "cimetidine" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "奥美拉唑" }, { "start_idx": 59, "end_idx": 68, "type": "dru", "entity": "omeprazole" }, { "start_idx": 70, "end_idx": 72, "type": "dru", "entity": "洛赛克" }, { "start_idx": 74, "end_idx": 78, "type": "dru", "entity": "losec" } ] }, { "text": "有水肿者可应用氢氯噻嗪、螺内酯或呋塞米等利尿剂。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "水肿" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "氢氯噻嗪" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "螺内酯" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "呋塞米" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "利尿剂" } ] }, { "text": "(二)肾上腺皮质激素肾上腺皮质激素对腹痛疗效好,对控制出血性皮疹、关节肿痛及蛋白尿有一定的效果,但对过敏性紫癜的血尿无效。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 10, "end_idx": 16, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "腹痛" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "出血性皮疹" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "关节" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "关节肿痛" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "过敏性紫癜" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "血尿" } ] }, { "text": "(三)免疫抑制剂对于Ⅳ~Ⅵ级HSPN,使用皮质激素的同时应给予环磷酰胺(CTX)治疗,可改善预后,具体疗法参见本章第四节。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "Ⅳ~Ⅵ级HSPN" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "皮质激素" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 36, "end_idx": 38, "type": "dru", "entity": "CTX" } ] }, { "text": "(四)抗血小板制剂长期口服双嘧达莫(dipyridamole,潘生丁,persantin)5~8mg/(kg•d),分2~3次口服,对预防和治疗HSPN有一定疗效。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "抗血小板制剂" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 18, "end_idx": 29, "type": "dru", "entity": "dipyridamole" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "潘生丁" }, { "start_idx": 35, "end_idx": 43, "type": "dru", "entity": "persantin" }, { "start_idx": 72, "end_idx": 75, "type": "dis", "entity": "HSPN" } ] }, { "text": "(五)中医中药可选用雷公藤多甙片,适用于I、Ⅱ及Ⅲ级HSPN。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dru", "entity": "中药" }, { "start_idx": 10, "end_idx": 15, "type": "dru", "entity": "雷公藤多甙片" }, { "start_idx": 20, "end_idx": 29, "type": "dis", "entity": "I、Ⅱ及Ⅲ级HSPN" } ] }, { "text": "初始剂量2mg/(kg•d),1个月后改为1.5mg/(kg•d)服用1个月,最后以1mg/(kg•d)维持1~2个月,对HSPN有较好疗效。", "entities": [ { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "HSPN" } ] }, { "text": "(六)其他有人报道应用硝苯地平(nifedipine,心痛定)每次0.25~0.5mg/kg,一日3~4次,可减轻HSPN的血管炎病变。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "硝苯地平" }, { "start_idx": 16, "end_idx": 25, "type": "dru", "entity": "nifedipine" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "心痛定" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "HSPN" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "血管炎病变" } ] }, { "text": "对肾衰竭病人可作透析及肾移植。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "透析" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "肾移植" } ] }, { "text": "【预后】一般认为本患儿童预后较成人佳,大部分能完全恢复或仅有轻微尿异常,预后与临床及病理类型相关。", "entities": [ { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "轻微尿异常" } ] }, { "text": "但Coup近年对儿童HSPN进行多中心研究后,认为遗留持续肾病或肾炎状态的患儿较多,而且15%在10年后,20%在20年后进展到终末肾衰竭,应引起重视。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "HSPN" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "肾病" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "肾炎" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "终末肾衰竭" } ] }, { "text": "第二节慢性胃炎慢性胃炎(chronicgastritis)是指各种原因持续反复作用于胃黏膜所引起的慢性炎症。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 12, "end_idx": 27, "type": "dis", "entity": "chronicgastritis" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "慢性炎症" } ] }, { "text": "慢性胃炎发病原因尚未明了,各种饮食、药物、微生物类、毒素以及胆汁反流,均可能与慢性胃炎的发病有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 18, "end_idx": 19, "type": "dru", "entity": "药物" }, { "start_idx": 21, "end_idx": 24, "type": "mic", "entity": "微生物类" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "胆汁" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "慢性胃炎" } ] }, { "text": "【病因及发病机制】(一)幽门螺杆菌自从1983年澳大利亚学者Warren和Marshall首次从慢性胃炎患者的胃黏液中分离出幽门螺杆菌以来,大量的研究表明,幽门螺杆菌与慢性胃炎密切相关。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "胃黏液" }, { "start_idx": 62, "end_idx": 66, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 78, "end_idx": 82, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 84, "end_idx": 87, "type": "dis", "entity": "慢性胃炎" } ] }, { "text": "在儿童中原发性胃炎幽门螺杆菌感染率高达40%,慢性活动性胃炎高达90%以上,而正常胃黏膜几乎很难检出幽门螺杆菌。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "原发性胃炎" }, { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "慢性活动性胃炎" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 50, "end_idx": 54, "type": "mic", "entity": "幽门螺杆菌" } ] }, { "text": "(二)化学性药物小儿时期经常感冒和发热,反复使用非甾体类药物如阿司匹林和吲哚美辛等,使胃黏膜内源性保护物质前列腺素E2减少,胃黏膜屏障功能降低,而致胃黏膜损伤。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "化学性药物" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "感冒" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "发热" }, { "start_idx": 24, "end_idx": 29, "type": "dru", "entity": "非甾体类药物" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 68, "end_idx": 76, "type": "sym", "entity": "胃黏膜屏障功能降低" }, { "start_idx": 80, "end_idx": 84, "type": "dis", "entity": "胃黏膜损伤" } ] }, { "text": "(三)不合理的饮食习惯食物过冷、过热、过酸、过辣、过咸,或经常暴饮暴食、饮食无规律等均可引起胃黏膜慢性炎症,食物中缺乏蛋白质及B族维生素也使慢性胃炎的易患性增加。", "entities": [ { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "胃黏膜慢性炎症" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "蛋白质" }, { "start_idx": 63, "end_idx": 67, "type": "dru", "entity": "B族维生素" }, { "start_idx": 70, "end_idx": 73, "type": "dis", "entity": "慢性胃炎" } ] }, { "text": "(四)细菌、病毒和(或)其毒素鼻腔、口咽部的慢性感染病灶,如扁桃腺炎、鼻旁窦炎等细菌或其毒素吞入胃内,长期慢性刺激可引起慢性胃黏膜炎症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "细菌" }, { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "病毒" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "鼻腔、口咽部" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "慢性感染病灶" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "扁桃腺炎" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "鼻旁窦炎" }, { "start_idx": 40, "end_idx": 41, "type": "mic", "entity": "细菌" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "胃黏膜炎症" } ] }, { "text": "(五)十二指肠液反流幽门括约肌功能失调时,使十二指肠液反流入胃增加。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "十二指肠液" }, { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "幽门括约肌功能失调" }, { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "十二指肠液反流入胃增加" } ] }, { "text": "十二指肠液中含有胆汁、肠液和胰液。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "十二指肠液" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胆汁" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肠液" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "胰液" } ] }, { "text": "【临床表现】小儿慢性胃炎的症状无特异性,多数有不同程度的消化不良症状,临床表现的轻重与胃黏膜的病变程度并非一致,且病程迁延。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 23, "end_idx": 33, "type": "sym", "entity": "不同程度的消化不良症状" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "胃黏膜" } ] }, { "text": "主要表现是反复腹痛,无明显规律性,通常在进食后加重。", "entities": [ { "start_idx": 5, "end_idx": 24, "type": "sym", "entity": "反复腹痛,无明显规律性,通常在进食后加重" } ] }, { "text": "疼痛部位不确切,多在脐周。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "疼痛部位不确切,多在脐周" } ] }, { "text": "体征多不明显,压痛部位可在中上腹或脐周,范围较广泛。", "entities": [ { "start_idx": 7, "end_idx": 18, "type": "sym", "entity": "压痛部位可在中上腹或脐周" } ] }, { "text": "【实验室检查】(一)胃酸测定浅表性胃炎胃酸正常或偏低,萎缩性胃炎则明显降低,甚至缺酸。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "胃酸测定" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "浅表性胃炎" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "胃酸正常或偏低" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "萎缩性胃炎" } ] }, { "text": "(二)幽门螺杆菌检测包括胃镜下取胃黏液直接涂片染色,组织切片染色找幽门螺杆菌,幽门螺杆菌培养,尿素酶检测。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "幽门螺杆菌检测" }, { "start_idx": 12, "end_idx": 13, "type": "equ", "entity": "胃镜" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "胃黏液" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "直接涂片染色" }, { "start_idx": 26, "end_idx": 31, "type": "pro", "entity": "组织切片染色" }, { "start_idx": 33, "end_idx": 37, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 39, "end_idx": 45, "type": "pro", "entity": "幽门螺杆菌培养" }, { "start_idx": 47, "end_idx": 51, "type": "pro", "entity": "尿素酶检测" } ] }, { "text": "其次是非侵袭法利用细菌的生物特性,特别是幽门螺杆菌的尿素酶水解尿素的能力而形成的呼气试验(13C-尿素呼气)检测幽门螺杆菌。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "尿素酶" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "尿素" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "呼气试验" }, { "start_idx": 62, "end_idx": 66, "type": "mic", "entity": "幽门螺杆菌" } ] }, { "text": "血清学幽门螺杆菌IgG抗体的测定,因不能提供细菌当前是否存在的依据,故不能用于目前感染的诊断,主要用于筛选或流行病学调查。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "pro", "entity": "幽门螺杆菌IgG抗体的测定" }, { "start_idx": 22, "end_idx": 23, "type": "mic", "entity": "细菌" } ] }, { "text": "以上方法中,以尿素酶法最为简便、快速,常一步完成。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "尿素酶法" } ] }, { "text": "13C-尿素呼气试验,因此法价格昂贵,临床普及受到限制。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "ite", "entity": "尿素呼气试验" } ] }, { "text": "(三)其他检查在A型萎缩性胃炎(胃体胃炎)血清中可出现壁细胞抗体、胃泌素抗体和内因子抗体等。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "A型萎缩性胃炎" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "胃体胃炎" }, { "start_idx": 21, "end_idx": 43, "type": "sym", "entity": "血清中可出现壁细胞抗体、胃泌素抗体和内因子抗体" } ] }, { "text": "多数萎缩性胃炎的血、尿胃蛋白酶原分泌减少,而浅表性胃炎多属正常。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "多数萎缩性胃炎的血、尿胃蛋白酶原分泌减少" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "浅表性胃炎" } ] }, { "text": "恶性贫血时血清维生素B12水平明显减少。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "恶性贫血" } ] }, { "text": "【X线钡餐检查】X线钡餐检查对慢性胃炎的诊断无多大帮助。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "X线钡餐检查" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "X线钡餐检查" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "慢性胃炎" } ] }, { "text": "依据国外资料,胃镜确诊为慢性胃炎者X线检查显示有胃黏膜炎症者仅20%~25%。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "X线检查" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "胃黏膜炎症" } ] }, { "text": "【胃镜检查】胃镜检查是慢性胃炎最主要的诊断方法,并可取黏膜活体组织做病理学检查。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "equ", "entity": "胃镜检查" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "胃镜检查" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "慢性胃炎" }, { "start_idx": 27, "end_idx": 32, "type": "ite", "entity": "黏膜活体组织" } ] }, { "text": "慢性胃炎在胃镜下表现为充血、水肿,反光增强,胃小凹明显,黏膜质脆易出血;黏液增多,微小结节形成,局限或大片状伴有新鲜或陈旧性出血点及糜烂。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "充血" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "水肿" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "反光增强" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "胃小凹明显" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "黏膜质脆易出血" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "黏液增多" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "微小结节形成" }, { "start_idx": 48, "end_idx": 57, "type": "sym", "entity": "局限或大片状伴有新鲜" }, { "start_idx": 59, "end_idx": 67, "type": "sym", "entity": "陈旧性出血点及糜烂" } ] }, { "text": "当胃黏膜有萎缩改变时,黏膜失去正常的橘红色,色泽呈灰色,皱襞变细,黏膜变薄,黏膜下血管显露。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "黏膜失去正常的橘红色" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "色泽呈灰色" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "皱襞变细" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "黏膜变薄" }, { "start_idx": 38, "end_idx": 44, "type": "sym", "entity": "黏膜下血管显露" } ] }, { "text": "病理组织学改变,上皮细胞变性,小凹上皮细胞增生,固有膜炎症细胞浸润,腺体萎缩,炎症细胞主要是淋巴细胞及浆细胞。", "entities": [ { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "小凹上皮细胞增生" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "膜炎症细胞浸润" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "腺体萎缩" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "浆细胞" } ] }, { "text": "【诊断与鉴别诊断】慢性胃炎无特殊性表现,单凭临床症状诊断较为困难,对反复腹痛与消化不良症状的患儿确诊主要依靠胃镜检查与病理组织活体检查。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "反复腹痛" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "消化不良" }, { "start_idx": 54, "end_idx": 57, "type": "pro", "entity": "胃镜检查" }, { "start_idx": 59, "end_idx": 66, "type": "pro", "entity": "病理组织活体检查" } ] }, { "text": "根据炎症程度分为轻度(炎症浸润仅限于黏液的浅表1/3)、中度(炎症累及黏膜的浅层1/3~2/3)及重度(炎症超过黏膜浅层2/3以上);若固有层内有中性粒细胞浸润则说明“活动性”。", "entities": [ { "start_idx": 73, "end_idx": 79, "type": "bod", "entity": "中性粒细胞浸润" } ] }, { "text": "此外,常规在胃窦大弯或后壁距幽门5cm内取组织切片染色,快速尿素酶试验或细菌培养,或13C-尿素呼气试验检查幽门螺杆菌,如阳性则诊断为“幽门螺杆菌相关性胃炎”。", "entities": [ { "start_idx": 6, "end_idx": 18, "type": "bod", "entity": "胃窦大弯或后壁距幽门5cm" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "切片染色" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "快速尿素酶试验" }, { "start_idx": 60, "end_idx": 64, "type": "ite", "entity": "幽门螺杆菌" }, { "start_idx": 74, "end_idx": 83, "type": "dis", "entity": "幽门螺杆菌相关性胃炎" } ] }, { "text": "发现幽门口收缩不良,反流增多,胆汁滞留胃内,病理切片示纤维组织增生,常提示胃炎与胆汁反流有关。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "胆汁" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "胃" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "胃炎" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "胆汁" } ] }, { "text": "【预防】早期去除各种诱发或加重胃炎的原因,避免精神过度紧张、疲劳与各种刺激性饮食,注意气候变化,防止受凉,积极治疗口腔及鼻咽部慢性感染灶,少用对胃黏膜有刺激的药物。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "胃炎" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "口腔" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "鼻咽部" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "胃黏膜" } ] }, { "text": "2.根除幽门螺杆菌对幽门螺杆菌引起的胃炎,尤为活动性胃炎,应给予抗幽门螺杆菌治疗。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 10, "end_idx": 14, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "胃炎" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "胃炎" }, { "start_idx": 32, "end_idx": 39, "type": "pro", "entity": "抗幽门螺杆菌治疗" } ] }, { "text": "4.高酸或胃炎活动期者,可给予H2受体阻滞剂:西咪替丁、雷尼替丁和法莫替丁。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "胃炎" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "西咪替丁" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "雷尼替丁" }, { "start_idx": 39, "end_idx": 42, "type": "dru", "entity": "法莫替丁" } ] }, { "text": "5.有胆汁反流者,给予胃达喜、熊去氧胆酸与胆汁酸结合及促进胆汁排空的药。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "胆汁反流" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "胃达喜" } ] }, { "text": "第二节非国家规划疫苗接种一、肺炎链球菌和流感嗜血杆菌疫苗肺炎链球菌和流感嗜血杆菌是严重危害儿童健康的主要致病菌,能引起化脓性脑膜炎、肺炎、败血症和急性中耳炎等感染性疾病。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dru", "entity": "疫苗" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "接种" }, { "start_idx": 14, "end_idx": 18, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 20, "end_idx": 27, "type": "dru", "entity": "流感嗜血杆菌疫苗" }, { "start_idx": 28, "end_idx": 32, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 34, "end_idx": 39, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 52, "end_idx": 54, "type": "mic", "entity": "致病菌" }, { "start_idx": 59, "end_idx": 64, "type": "dis", "entity": "化脓性脑膜炎" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "肺炎" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "败血症" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "急性中耳炎" } ] }, { "text": "WHO希望将这两种疫苗纳入各国的扩大免疫计划中去。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "疫苗" } ] }, { "text": "(一)肺炎链球菌疫苗荚膜多糖是肺炎链球菌的分型依据,也是致病的主要因素。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dru", "entity": "肺炎链球菌疫苗" }, { "start_idx": 15, "end_idx": 19, "type": "mic", "entity": "肺炎链球菌" } ] }, { "text": "到目前为止,可分型的肺炎链球菌有90种,但经常引起人类感染的只有20余种血清型,国外最重要的肺炎链球菌致病血清型是6B、9V、14、19A、19F和23F等。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 46, "end_idx": 50, "type": "mic", "entity": "肺炎链球菌" } ] }, { "text": "肺炎链球菌多糖疫苗由提纯的细菌荚膜多糖制备,有14价(型)和23价的多糖疫苗两种。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dru", "entity": "肺炎链球菌多糖疫苗" }, { "start_idx": 23, "end_idx": 37, "type": "dru", "entity": "14价(型)和23价的多糖疫苗" } ] }, { "text": "23价至少覆盖85%~90%在成人和儿童引起侵袭性感染的血清型,有效性为61%~75%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "23价" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "侵袭性感染" } ] }, { "text": "23价多糖疫苗可诱导特异性抗体产生,增强淋巴细胞和吞噬细胞的调理、吞噬和杀菌功能。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "23价多糖疫苗" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "诱导特异性抗体" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "吞噬细胞" } ] }, { "text": "注射后2~3周,80%以上健康年轻人血清中的特异性抗体升高2倍以上,并可维持5~10年。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "血清" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "特异性抗体" } ] }, { "text": "疫苗的安全性较好。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "疫苗" } ] }, { "text": "注射后一般出现轻微的局部不良反应(注射部位疼痛和红肿),48小时以内可消失。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "注射" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "注射部位疼痛和红肿" } ] }, { "text": "个别出现全身不良反应(发热和肌痛)和局部严重反应(局部硬结)。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "发热" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "肌痛" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "局部硬结" } ] }, { "text": "全身严重反应(过敏反应)很罕见。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "过敏反应" } ] }, { "text": "推荐使用多糖疫苗的对象:①>2岁儿童和>65岁老年人;②镰状细胞病;③功能性或解剖性无脾;④肾病综合征或慢性肾衰竭;⑤各种原因的免疫缺陷,包括器官移植和长期全身使用肾上腺皮质类固醇;⑥脑脊液漏;⑦HIV感染。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "多糖疫苗" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "镰状细胞病" }, { "start_idx": 35, "end_idx": 43, "type": "dis", "entity": "功能性或解剖性无脾" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "慢性肾衰竭" }, { "start_idx": 64, "end_idx": 67, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 71, "end_idx": 74, "type": "pro", "entity": "器官移植" }, { "start_idx": 82, "end_idx": 89, "type": "dru", "entity": "肾上腺皮质类固醇" }, { "start_idx": 92, "end_idx": 95, "type": "sym", "entity": "脑脊液漏" }, { "start_idx": 98, "end_idx": 102, "type": "dis", "entity": "HIV感染" } ] }, { "text": "多糖疫苗不足之处:①对<2岁婴幼儿无免疫原性,因为多糖是非T细胞依赖性半抗原。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "多糖疫苗" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "T细胞" } ] }, { "text": "而<2岁儿童又是侵袭性感染和黏膜感染的高发年龄组,因为此时从母体获得的抗体已经消失,自身产生的抗体水平很低。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "侵袭性感染" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "黏膜感染" } ] }, { "text": "②对某些患者提供的保护作用是有限的,例如免疫缺陷和血液恶性肿瘤。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "血液恶性肿瘤" } ] }, { "text": "③不能降低呼吸道黏膜肺炎链球菌的带菌率。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "呼吸道黏膜" }, { "start_idx": 10, "end_idx": 14, "type": "mic", "entity": "肺炎链球菌" } ] }, { "text": "新的为幼小儿童使用的肺炎链球菌结合疫苗已经研制成功,在多糖上加蛋白载体,由非T细胞依赖性抗原变为T细胞依赖性抗原,以增加免疫原性。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "dru", "entity": "肺炎链球菌结合疫苗" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "T细胞" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "T细胞" } ] }, { "text": "目前主要开发出5种结合疫苗,在多糖上分别加白喉类毒素(Pnc-D)、破伤风类毒素(Pnc-T)、CRM197蛋白(Pnc-CRM)、脑膜炎球菌外膜蛋白复合物(Pnc-OMPC)和既有白喉类毒素又有破伤风类毒素混合物(Pnc-TD)。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "结合疫苗" }, { "start_idx": 21, "end_idx": 25, "type": "mic", "entity": "白喉类毒素" }, { "start_idx": 27, "end_idx": 31, "type": "mic", "entity": "Pnc-D" }, { "start_idx": 34, "end_idx": 39, "type": "mic", "entity": "破伤风类毒素" }, { "start_idx": 41, "end_idx": 45, "type": "mic", "entity": "Pnc-T" }, { "start_idx": 48, "end_idx": 55, "type": "mic", "entity": "CRM197蛋白" }, { "start_idx": 57, "end_idx": 63, "type": "mic", "entity": "Pnc-CRM" }, { "start_idx": 66, "end_idx": 77, "type": "mic", "entity": "脑膜炎球菌外膜蛋白复合物" }, { "start_idx": 79, "end_idx": 86, "type": "mic", "entity": "Pnc-OMPC" }, { "start_idx": 91, "end_idx": 95, "type": "mic", "entity": "白喉类毒素" }, { "start_idx": 98, "end_idx": 103, "type": "mic", "entity": "破伤风类毒素" }, { "start_idx": 108, "end_idx": 113, "type": "mic", "entity": "Pnc-TD" } ] }, { "text": "结合疫苗不仅能够诱导产生足够的特异性抗体,还能诱导免疫回忆反应。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "结合疫苗" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "特异性抗体" } ] }, { "text": "因此可以抗侵袭性感染(肺炎、脑膜炎和败血症等)和急性中耳炎,还能降低肺炎链球菌在鼻咽部的带菌率。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "侵袭性感染" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "肺炎" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "败血症" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "急性中耳炎" }, { "start_idx": 34, "end_idx": 38, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "鼻咽部" } ] }, { "text": "以此为依据推出一种7价的结合疫苗。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dru", "entity": "7价的结合疫苗" } ] }, { "text": "临床研究表明可覆盖86%的菌血症,83%的脑膜炎,65%急性中耳炎。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "菌血症" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "另有9价和11价结合疫苗可覆盖高达92%的侵袭性感染。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dru", "entity": "9价和11价结合疫苗" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "侵袭性感染" } ] }, { "text": "注射1个月后健康成人血清中特异性抗体2~10倍升高,而在多糖疫苗组中只有2~6倍升高。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "血清" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "特异性抗体" } ] }, { "text": "WHO推荐这种结合疫苗到儿童计划免疫中。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "结合疫苗" } ] }, { "text": "目前,PCV9、PCV10、PCV11、PCV13疫苗陆续被研制成功,且增加了1、3、5、6A、7F、19A等常见和有增多趋势的IPD血清型。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "PCV9" }, { "start_idx": 14, "end_idx": 18, "type": "dru", "entity": "PCV11" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "PCV13" } ] }, { "text": "(二)流感嗜血杆菌疫苗流感嗜血杆菌(haemophilusinfluenzae,Hi)分为有荚膜和无荚膜两种。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dru", "entity": "流感嗜血杆菌疫苗" }, { "start_idx": 11, "end_idx": 16, "type": "dru", "entity": "流感嗜血杆菌" }, { "start_idx": 18, "end_idx": 38, "type": "dru", "entity": "haemophilusinfluenzae" }, { "start_idx": 40, "end_idx": 41, "type": "dru", "entity": "Hi" } ] }, { "text": "所有流感嗜血杆菌都可引起侵袭性感染,但b型是引起5岁以下儿童严重感染和死亡的最常见病原菌之一。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "侵袭性感染" } ] }, { "text": "目前有4种结合疫苗登记注册,在磷酸多核糖核醇(PRP)上分别加白喉类毒素(PRP-D)、破伤风类毒素(PRPT)、CRM197蛋白(PRP-CRM或HbOC)、脑膜炎球菌外膜蛋白复合物(PRP-OMP)。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "结合疫苗" }, { "start_idx": 31, "end_idx": 35, "type": "mic", "entity": "白喉类毒素" }, { "start_idx": 37, "end_idx": 41, "type": "mic", "entity": "PRP-D" }, { "start_idx": 44, "end_idx": 49, "type": "mic", "entity": "破伤风类毒素" }, { "start_idx": 51, "end_idx": 54, "type": "mic", "entity": "PRPT" }, { "start_idx": 57, "end_idx": 64, "type": "mic", "entity": "CRM197蛋白" }, { "start_idx": 66, "end_idx": 72, "type": "mic", "entity": "PRP-CRM" }, { "start_idx": 74, "end_idx": 77, "type": "mic", "entity": "HbOC" }, { "start_idx": 80, "end_idx": 84, "type": "mic", "entity": "脑膜炎球菌" } ] }, { "text": "在欧洲和美国,4种结合疫苗抗侵袭性感染的有效性>90%。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "结合疫苗" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "侵袭性感染" } ] }, { "text": "美国自从1988使用结合疫苗以来,1993年<5岁儿童中Hib侵袭性感染的发病率下降了95%以上。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "结合疫苗" }, { "start_idx": 28, "end_idx": 35, "type": "dis", "entity": "Hib侵袭性感染" } ] }, { "text": "在冈比亚,Hib结合疫苗抗侵袭性感染有效性与发达国家相同。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dru", "entity": "Hib结合疫苗" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "侵袭性感染" } ] }, { "text": "该国儿童脑膜炎发病率由使用疫苗前的每年200/10万人下降到使用疫苗后的21/10万人。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 13, "end_idx": 14, "type": "dru", "entity": "疫苗" } ] }, { "text": "另外从X线诊断学上判断,结合疫苗接种组的肺炎发病率减少>20%。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "X线" }, { "start_idx": 14, "end_idx": 15, "type": "dru", "entity": "疫苗" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "接种" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肺炎" } ] }, { "text": "这种结合疫苗可以降低疫苗接种者的鼻咽部的带菌率,减少病原体在易感小儿中的传播。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "鼻咽部" } ] }, { "text": "Hib结合疫苗能与DTP疫苗同时注射,不良反应比单独使用DTP时无明显增加。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "Hib结合疫苗" }, { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "DTP疫苗" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "注射" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "DTP" } ] }, { "text": "一般局部反应有发热、皮疹和易激惹等。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "发热" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "皮疹" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "易激惹" } ] }, { "text": "目前存在的问题:①流感嗜血杆菌在亚洲,包括我国的流行病学资料还很少;②流感嗜血杆菌疫苗接种时间与DPT和MMR(麻疹、腮腺炎、风疹)等同时,需要开发联合疫苗,即一针多苗,国外已有无细胞百、白、破与Hib的联合疫苗;③结合疫苗及联合多种疫苗的价格较贵。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 35, "end_idx": 42, "type": "dru", "entity": "流感嗜血杆菌疫苗" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "麻疹" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "风疹" }, { "start_idx": 98, "end_idx": 100, "type": "dis", "entity": "Hib" } ] }, { "text": "第三节儿童慢性粒细胞白血病慢性粒细胞白血病(chronicmyelogenousleukemia,CML)是一种骨髓异常增生性疾病,在儿童中分为成人型慢性粒细胞白血病和幼年型慢性粒细胞白血病(JCML),主要为骨髓多能造血干细胞病变。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "儿童慢性粒细胞白血病" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "慢性粒细胞白血病" }, { "start_idx": 22, "end_idx": 47, "type": "dis", "entity": "chronicmyelogenousleukemia" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "CML" }, { "start_idx": 56, "end_idx": 64, "type": "dis", "entity": "骨髓异常增生性疾病" }, { "start_idx": 72, "end_idx": 82, "type": "dis", "entity": "成人型慢性粒细胞白血病" }, { "start_idx": 84, "end_idx": 94, "type": "dis", "entity": "幼年型慢性粒细胞白血病" }, { "start_idx": 96, "end_idx": 99, "type": "dis", "entity": "JCML" }, { "start_idx": 105, "end_idx": 115, "type": "sym", "entity": "骨髓多能造血干细胞病变" } ] }, { "text": "与急性白血病比较病变细胞形态相对成熟,发病相对缓慢,尤其是CML自然病程可达数年。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "急性白血病" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "CML" } ] }, { "text": "儿童CML约占白血病总发病数的5%不到,其中JCML则更少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "儿童CML" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "白血病" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "JCML" } ] }, { "text": "一、慢性粒细胞白血病CML是白血病中最先被认识的一种血细胞疾病,1844年Doune首先描述了它的临床特征,至1870年Neumann提出本病起源于骨髓而不是脾脏,1960年Nowell和Hungerford在费城发现本病的细胞遗传学特征Ph1染色体。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "慢性粒细胞白血病" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "CML" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "白血病" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "骨髓" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "脾脏" }, { "start_idx": 128, "end_idx": 130, "type": "bod", "entity": "染色体" } ] }, { "text": "【流行病学】CML好发于中年人,40~50岁为发病高峰,在儿童中80%病人在4岁后发病,60%在6岁后发病,也有生后3个月即发病的报告。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "CML" } ] }, { "text": "【细胞遗传学及生物学特征】Ph1是CML的标志性染色体变化,由非随机t(9;22)(q34;q11)形成。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "Ph1" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "CML" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "染色体" } ] }, { "text": "在9号染色体断裂点上有c-abl基因,它的变异性可大于100kb。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "染色体" }, { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "c-abl基因" } ] }, { "text": "Bcr基因位于22号染色体,是一个变异性很小的5.8kbDNA小片段,易位后22q-和9q+结合部形成bcr/abl融合基因,编码一个特异的210kb蛋白质(P210),它是一种酪氨酸激酶,在肿瘤的发病中起作用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "Bcr基因" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "染色体" }, { "start_idx": 23, "end_idx": 30, "type": "bod", "entity": "5.8kbDNA" }, { "start_idx": 51, "end_idx": 61, "type": "bod", "entity": "bcr/abl融合基因" }, { "start_idx": 70, "end_idx": 77, "type": "bod", "entity": "210kb蛋白质" }, { "start_idx": 79, "end_idx": 82, "type": "bod", "entity": "P210" }, { "start_idx": 89, "end_idx": 93, "type": "bod", "entity": "酪氨酸激酶" }, { "start_idx": 96, "end_idx": 97, "type": "dis", "entity": "肿瘤" } ] }, { "text": "Ph1染色体在儿童CML中尚有它的特殊情况。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "儿童CML" } ] }, { "text": "(一)Ph1阴性CML约5%~10%有典型CML临床表现的病例Ph1阴性,可能有以下原因:①其他染色体片段结合于22q,使Ph1的22q-在细胞遗传学水平不易检测到;②9号染色体有断裂或基因重排,但22q11未断裂,分子生物学技术可检测出CMLPh1阴性时的这些变化。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "Ph1" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "CML" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "CML" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "Ph1" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "染色体" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "Ph1" }, { "start_idx": 104, "end_idx": 106, "type": "bod", "entity": "染色体" }, { "start_idx": 137, "end_idx": 142, "type": "bod", "entity": "CMLPh1" } ] }, { "text": "(二)Ph1阳性急性白血病Ph1染色体并不只存在于CML中,约3%~10%的儿童急性白血病有Ph1染色体。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "Ph1" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "急性白血病" }, { "start_idx": 19, "end_idx": 30, "type": "bod", "entity": "Ph1染色体" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "CML" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "儿童急性白血病" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "Ph1" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "染色体" } ] }, { "text": "Ph1阳性急性白血病可以是CML急变,也可能是原发的急性白血病。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "急性白血病" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "CML" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "急性白血病" } ] }, { "text": "在临床和血液学检查中区别这两种情况很困难,但细胞遗传学结合分子生物学技术检查可发现Ph1阳性急性白血病常为非CML特异的非随机的染色体异常t(9;22)(q34;q11),有bcr基因以外的基因重排,并产生190kb蛋白(P190),在治疗缓解后骨髓细胞中的Ph1染色体和P190即可消失。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "pro", "entity": "临床和血液学检查" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "Ph1" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "急性白血病" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "染色体" }, { "start_idx": 93, "end_idx": 97, "type": "bod", "entity": "bcr基因" }, { "start_idx": 109, "end_idx": 115, "type": "bod", "entity": "190kb蛋白" }, { "start_idx": 117, "end_idx": 120, "type": "bod", "entity": "P190" }, { "start_idx": 129, "end_idx": 132, "type": "bod", "entity": "骨髓细胞" }, { "start_idx": 135, "end_idx": 146, "type": "bod", "entity": "Ph1染色体" }, { "start_idx": 148, "end_idx": 151, "type": "bod", "entity": "P190" } ] }, { "text": "而CML则相反,有CML特异的非随机染色体异常t(9;22)(q34;q11),无论在疾病的哪一个时期,Ph1染色体和bcr基因重排所产生的210kb蛋白(P210)始终存在。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "CML" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "CML" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "染色体" }, { "start_idx": 52, "end_idx": 63, "type": "bod", "entity": "Ph1染色体" }, { "start_idx": 65, "end_idx": 69, "type": "bod", "entity": "bcr基因" }, { "start_idx": 76, "end_idx": 82, "type": "bod", "entity": "210kb蛋白" }, { "start_idx": 84, "end_idx": 87, "type": "bod", "entity": "P210" } ] }, { "text": "【生物学特征】(一)克隆特征CML是一个起源于骨髓单株多能干细胞的获得性疾病,由于多能干细胞有向多个细胞系发育的潜能,CML急变时具不均一性,如急淋变、急粒变、急单变。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "CML" }, { "start_idx": 23, "end_idx": 31, "type": "bod", "entity": "骨髓单株多能干细胞" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "多能干细胞" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "CML" } ] }, { "text": "(二)细胞生长特性CML从单个病变细胞增殖,直至骨髓、外周血、脾脏中堆积大量的CML细胞,多种机制参与了这一过程(表11-11)。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "CML" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "病变细胞" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "骨髓" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "外周血" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "脾脏" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "CML细胞" } ] }, { "text": "表11-9CML细胞异常增生机制(三)细胞动力学CML慢性期外周血粒细胞数数十倍甚至数百倍于正常粒细胞数,这些细胞可自由循环于骨髓、外周血、脾脏间;CML细胞的半衰期比正常人粒细胞长5~10倍,这些细胞形态学上为未完全成熟的粒细胞,同时形态学上完全成熟的粒细胞半衰期也比正常人长2~4倍。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "CML细胞" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "CML" }, { "start_idx": 30, "end_idx": 36, "type": "ite", "entity": "外周血粒细胞数" }, { "start_idx": 48, "end_idx": 51, "type": "ite", "entity": "粒细胞数" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "骨髓" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "外周血" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "脾脏" }, { "start_idx": 74, "end_idx": 78, "type": "bod", "entity": "CML细胞" }, { "start_idx": 112, "end_idx": 114, "type": "bod", "entity": "粒细胞" }, { "start_idx": 127, "end_idx": 129, "type": "bod", "entity": "粒细胞" } ] }, { "text": "CML细胞的增殖速度比正常人慢,因此细胞寿命延长是CML外周血中粒细胞明显增高的主要原因,而不是它的增殖速度。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "CML细胞" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "细胞" }, { "start_idx": 25, "end_idx": 34, "type": "bod", "entity": "CML外周血中粒细胞" } ] }, { "text": "CML慢性期时髓系定向干细胞明显增多,因此在干细胞培养中粒-单细胞集落形成单位(GM-CFU)数明显增多,可高于正常人10~20倍。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "CML" }, { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "髓系定向干细胞" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "干细胞" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "中粒-单细胞" } ] }, { "text": "(四)疾病转化基础CML起病时多能造血干细胞发生c-abl基因重排,形成肿瘤前期血细胞克隆,这一转化过程中的克隆、出现细胞遗传学变化,即形成Ph1染色体使bcr/abl融合,融合基因下调酪氨酸激酶活力,使细胞凋亡紊乱。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "CML" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "多能造血干细胞" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "c-abl基因" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "肿瘤" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "血细胞" }, { "start_idx": 70, "end_idx": 81, "type": "bod", "entity": "Ph1染色体" }, { "start_idx": 83, "end_idx": 91, "type": "bod", "entity": "bcr/abl融合" }, { "start_idx": 99, "end_idx": 103, "type": "bod", "entity": "酪氨酸激酶" } ] }, { "text": "这些细胞中基因的不稳定性增加和DNA复制过程中的自发性错误使病变细胞向更异常的方向发展,直至出现另一个新的细胞遗传学变化。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "DNA" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "病变细胞" } ] }, { "text": "随着这些变化,细胞的增殖与分化失去正常关系,异常克隆抑制正常克隆生长,异常的未成熟克隆呈优势,最终进入急性白血病期。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "细胞" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "急性白血病" } ] }, { "text": "【临床表现】CML分为三个阶段,即慢性期、加速期和急变期。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "CML" } ] }, { "text": "各阶段代表疾病的性质由高增生性、基本正常的相对成熟血细胞成分为主进展为分化停滞、未成熟细胞为优势的过程。", "entities": [ { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "成熟血细胞" } ] }, { "text": "(一)慢性期主要由造血细胞池扩张造成的一系列变化,此期粒细胞数明显增高,主要浸润于骨髓、外周血、脾脏和肝脏,血黏稠度增高。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "血细胞池" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "粒细胞" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "骨髓" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "外周血" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "脾脏" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "肝脏" } ] }, { "text": "病人有非特异性症状如发热、盗汗、乏力、左上腹饱满或疼痛、骨痛白细胞数,称为白血危象,造成血管阻塞并出现相应的临床情况如中枢神经系统症状、呼吸窘迫综合征、视力障碍等。", "entities": [ { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "骨" }, { "start_idx": 3, "end_idx": 29, "type": "sym", "entity": "非特异性症状如发热、盗汗、乏力、左上腹饱满或疼痛、骨痛" }, { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "白细胞数" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "白血危象" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "血管阻塞" }, { "start_idx": 59, "end_idx": 66, "type": "dis", "entity": "中枢神经系统症状" }, { "start_idx": 68, "end_idx": 74, "type": "dis", "entity": "呼吸窘迫综合征" }, { "start_idx": 76, "end_idx": 79, "type": "dis", "entity": "视力障碍" } ] }, { "text": "(二)加速期出现进展性加重的全身症状,如发热、盗汗、乏力、消瘦和出血倾向,肝、脾增大,化疗难于控制。", "entities": [ { "start_idx": 20, "end_idx": 35, "type": "sym", "entity": "发热、盗汗、乏力、消瘦和出血倾向" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "肝、脾增大" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "化疗" } ] }, { "text": "急变期临床表现与急性白血病相似,贫血、出血、发热,肝肿大并可伴有其他髓外浸润灶。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "急性白血病" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肝" }, { "start_idx": 16, "end_idx": 27, "type": "sym", "entity": "贫血、出血、发热,肝肿大" }, { "start_idx": 28, "end_idx": 38, "type": "sym", "entity": "并可伴有其他髓外浸润灶" } ] }, { "text": "【实验室检查】(一)外周血象1.慢性期轻度贫血,粒细胞数明显增高伴左移,计数在(8.0~80)×109/L之间,平均为25×109/L,大于50×109/L的病例较成人多见,涂片分类各期细胞均可见,但未完全成熟粒细胞小于15%,嗜酸及嗜碱粒细胞绝对值增高,并可见嗜酸嗜碱双染细胞。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "外周血象" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "轻度贫血" }, { "start_idx": 24, "end_idx": 34, "type": "sym", "entity": "粒细胞数明显增高伴左移" }, { "start_idx": 105, "end_idx": 106, "type": "pro", "entity": "涂片" }, { "start_idx": 146, "end_idx": 156, "type": "sym", "entity": "并可见嗜酸嗜碱双染细胞" } ] }, { "text": "血小板数常增高,接近500×109/L。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "血小板数常增高" } ] }, { "text": "2.加速期粒细胞数下降,但原始及幼稚细胞比例明显增高。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "粒细胞" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "幼稚细胞" } ] }, { "text": "血小板数下降。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" } ] }, { "text": "此期病人50%发展为急性白血病,另约45%逐渐发展为骨髓增生异常综合征样状态。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "急性白血病" }, { "start_idx": 26, "end_idx": 34, "type": "dis", "entity": "骨髓增生异常综合征" } ] }, { "text": "3.急变期血小板、血色素进一步下降,原始加幼稚细胞比例进一步增高与急性白血病相似。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "血小板" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "血色素" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "幼稚细胞" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "急性白血病" } ] }, { "text": "(二)骨髓象1.慢性期骨髓高度增生,以粒系为主,见各阶段细胞,以中晚幼粒细胞及杆状核粒细胞为主,原始加幼稚细胞比例小于5%。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "骨髓象" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "骨髓高度增生" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "以粒系为主" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "见各阶段细胞" }, { "start_idx": 31, "end_idx": 46, "type": "sym", "entity": "以中晚幼粒细胞及杆状核粒细胞为主" } ] }, { "text": "易见嗜碱和嗜酸细胞。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "易见嗜碱和嗜酸细胞" } ] }, { "text": "骨髓纤维化不明显。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "骨髓纤维化不明显" } ] }, { "text": "偶见类似高雪细胞和海蓝细胞的有脂质沉积的组织细胞组织化学染色示白细胞碱性磷酸酶(AKP)活力明显减低原始加幼稚细胞比例大于30%是急变的主要依据,加速期原始加幼稚细胞比例在5%~30%之间。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "高雪细胞" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "海蓝细胞" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "脂质" }, { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "偶见类似高雪细胞和海蓝细胞的有脂质沉积的组织细胞" }, { "start_idx": 31, "end_idx": 38, "type": "bod", "entity": "白细胞碱性磷酸酶" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "AKP" }, { "start_idx": 24, "end_idx": 49, "type": "sym", "entity": "组织化学染色示白细胞碱性磷酸酶(AKP)活力明显减低" }, { "start_idx": 50, "end_idx": 63, "type": "sym", "entity": "原始加幼稚细胞比例大于30%" }, { "start_idx": 79, "end_idx": 93, "type": "sym", "entity": "幼稚细胞比例在5%~30%之间" } ] }, { "text": "60%~70%病人向髓系急变,但此时过氧化物酶(POX)通常阴性,用单抗作表面抗原检测可发现幼稚细胞中也包含有少量巨核系、红系和单核系细胞。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "ite", "entity": "过氧化物酶" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "POX" }, { "start_idx": 34, "end_idx": 42, "type": "pro", "entity": "单抗作表面抗原检测" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "幼稚细胞" }, { "start_idx": 57, "end_idx": 68, "type": "bod", "entity": "巨核系、红系和单核系细胞" } ] }, { "text": "(三)细胞遗传学大部分病人Ph1染色体阳性,同时可检测到bcr/abl融合基因(P210)。", "entities": [ { "start_idx": 13, "end_idx": 24, "type": "bod", "entity": "Ph1染色体" }, { "start_idx": 34, "end_idx": 44, "type": "bod", "entity": "bcr/abl融合基因" } ] }, { "text": "急变期常有新的染色体变化,如Ph1复制,8-三体,19-三体,17q异构等。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "染色体" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "Ph1复制" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "8-三体" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "19-三体" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "17q异构" } ] }, { "text": "【预后】目前常用的治疗手段可使CML的中位生存期达5~5.5年,35%~40%的病人可生存7~8年。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "CML" } ] }, { "text": "影响慢性期长短的不利因素包括脾左肋下大于15cm,肝右肋下大于6cm,血小板低于150×109/L或大于500×109/L,外周血幼稚细胞大于1%或未完全成熟(核左移)细胞大于20%。", "entities": [ { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "脾" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肝" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "血小板" }, { "start_idx": 74, "end_idx": 80, "type": "bod", "entity": "外周血幼稚细胞" }, { "start_idx": 86, "end_idx": 97, "type": "bod", "entity": "未完全成熟(核左移)细胞" } ] }, { "text": "【治疗】常规化疗很难达到细胞遗传学水平缓解,即彻底清除Ph1染色体阳性细胞。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "化疗" }, { "start_idx": 27, "end_idx": 42, "type": "bod", "entity": "Ph1染色体阳性细胞" } ] }, { "text": "目前异基因骨髓移植仍是CML获得长期无病生存的唯一治疗手段。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "基因骨髓移植" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "CML" } ] }, { "text": "针对bcr/abl融合基因的靶向治疗在CML治疗中取得了举世瞩目的成绩,可获得细胞/分子遗传学水平缓解,但是否能达到根治,目前尚无结论。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "bod", "entity": "bcr/abl融合基因" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "靶向治疗" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "CML" } ] }, { "text": "(一)非特异性处理高尿酸血症,水、电解质紊乱,白血危象,高血小板血症需紧急对症处理(同急性白血病),有脑膜浸润时按脑膜白血病处理。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "高尿酸血症" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "水、电解质紊乱" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "白血危象" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "高血小板血症" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "急性白血病" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "脑膜浸润" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "脑膜白血病" } ] }, { "text": "(二)慢性期治疗1.单药化疗标准药物为马利兰或羟基脲,可有效地控制临床症状、体征和血液学变化,但不会使急变期延迟。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "化疗" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "马利兰" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "羟基脲" } ] }, { "text": "羟基脲是一种核苷酸还原酶抑制剂,抑制核苷酸转化为脱氧核苷酸,从而影响DNA合成,推荐剂量为10~20mg/(kg•d),根据临床情况作调整,它与马利兰疗效相仿,它的作用时间较短,因此相对安全,全身性的毒副作用也相对较小。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "羟基脲" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "核苷酸还原酶" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "核苷酸" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "脱氧核苷酸" }, { "start_idx": 72, "end_idx": 74, "type": "dru", "entity": "马利兰" } ] }, { "text": "马利兰是一种烷化剂,为非细胞周期特异性药物,常用剂量为0.06~0.1mg/(kg•d),用药10~14天后血细胞开始明显下跌、肝、脾缩小晚于血象变化,约3个月肝脾完全恢复正常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "马利兰" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "血细胞" }, { "start_idx": 64, "end_idx": 64, "type": "bod", "entity": "肝" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "脾" }, { "start_idx": 80, "end_idx": 80, "type": "bod", "entity": "肝" }, { "start_idx": 81, "end_idx": 81, "type": "bod", "entity": "脾" } ] }, { "text": "马利兰特点为作用反应出现较晚但持续时间长,因此在白细胞计数跌至(30~40)×109/L时药物剂量应减半,至20×109/L时应停药。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "马利兰" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "白细胞" } ] }, { "text": "停药后2~3周内白细胞计数仍可继续下跌。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "白细胞" } ] }, { "text": "除骨髓抑制外,马利兰尚有肺纤维化、色素沉着、消瘦和低血压等不良反应。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "骨髓抑制" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "马利兰" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "肺纤维化" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "色素沉着" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "消瘦" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "低血压" } ] }, { "text": "2.α-干扰素其作用机制未明确。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "α-干扰素" } ] }, { "text": "70%病人对干扰素治疗有效,达到血液学水平缓解。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "干扰素" } ] }, { "text": "α-干扰素和马利兰合用时骨髓抑制严重,缓解率不高于单用α-干扰素。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "α-干扰素" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "马利兰" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "骨髓" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "α-干扰素" } ] }, { "text": "与羟基脲合用时达缓解时间缩短,毒副反应可以耐受,但并不提高细胞遗传学水平的缓解率。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "羟基脲" } ] }, { "text": "与小剂量阿糖胞苷合用可提高细胞遗传学水平的缓解率和慢性期后期缓解率。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "阿糖胞苷" } ] }, { "text": "α-干扰素与羟基脲合用时推荐用法如下:先用羟基脲,在白细胞数降至(10~20)×109/L时减停,同时加用α-干扰素,α-干扰素从小剂量开始,3~7天后增加剂量,在2周内达全量。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "α-干扰素" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "羟基脲" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "羟基脲" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "白细胞" }, { "start_idx": 59, "end_idx": 63, "type": "dru", "entity": "α-干扰素" }, { "start_idx": 65, "end_idx": 69, "type": "dru", "entity": "α-干扰素" } ] }, { "text": "α-干扰素最好在睡前应用,并同时加用退热剂,以避免发热反应。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "α-干扰素" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "退热剂" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "发热反应" } ] }, { "text": "其他的不良反应尚有疲劳、抑郁、失眠等。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "疲劳" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "抑郁" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "失眠" } ] }, { "text": "当白细胞降至2×109/L或血小板低于50×109/L时减量。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "ite", "entity": "白细胞" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "血小板" } ] }, { "text": "α-干扰素应长期应用直至加速期或急变期或细胞遗传学水平缓解3年以上。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "α-干扰素" } ] }, { "text": "3.异基因骨髓移植目前仍是唯一已证实的可治愈CML的治疗方法(见干细胞移植章节)。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "异基因骨髓移植" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "CML" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "干细胞移植" } ] }, { "text": "CML慢性期非亲缘供体骨髓移植结果(表11-11)。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "pro", "entity": "非亲缘供体骨髓移植" } ] }, { "text": "表11-10CML慢性期非亲缘供体骨髓移植结果4.靶向治疗甲磺酸伊马替尼(imatiribmesylate,Gleevec,Glivec)通过特异性抑制肿瘤细胞酪氨酸激酶活性而抑制恶性肿瘤克隆,因具有高度特异性,故与其他化疗药物相比其毒副作用相对低,对CML非常有效。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "CML" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "骨髓移植" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "靶向治疗" }, { "start_idx": 29, "end_idx": 35, "type": "dru", "entity": "甲磺酸伊马替尼" }, { "start_idx": 37, "end_idx": 52, "type": "dru", "entity": "imatiribmesylate" }, { "start_idx": 54, "end_idx": 60, "type": "dru", "entity": "Gleevec" }, { "start_idx": 62, "end_idx": 67, "type": "dru", "entity": "Glivec" }, { "start_idx": 76, "end_idx": 84, "type": "bod", "entity": "肿瘤细胞酪氨酸激酶" }, { "start_idx": 92, "end_idx": 93, "type": "dis", "entity": "肿瘤" }, { "start_idx": 110, "end_idx": 111, "type": "pro", "entity": "化疗" }, { "start_idx": 126, "end_idx": 128, "type": "dis", "entity": "CML" } ] }, { "text": "经过大型临床研究(IRIS),伊马替尼每天400mg与IFN-α+Ara-C随机对照比较,伊马替尼有明显优势,包括血液缓解,细胞遗传学缓解及分子生物学缓解,同时亦延长存活及非进展存活,其后多个临床研究亦有相类结果。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "伊马替尼" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "IFN-α" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "Ara-C" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "伊马替尼" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "血液" } ] }, { "text": "伊马替尼现在已是很多临床指示的第一线治疗CML药物,但目前尚不明确治疗时间和是否能完全替代异基因造血干细胞移植治愈CML。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "伊马替尼" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "CML" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "CML" } ] }, { "text": "(三)急变后治疗CML急变后预后不良,对治疗常耐药,急变后平均生存期为3个月,急淋变生存期稍长,急变后可按急性白血病相应类型治疗。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "CML" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "白血病" } ] }, { "text": "第七节肺通气异常性疾病一、肺气肿肺气肿(emphysema)是指终末支气管远端部分,包括呼吸性细支气管,肺泡管、肺泡囊及肺泡的膨胀及过度充气,导致肺组织弹力减退和容积增大。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "肺通气异常性疾病" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肺气肿" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "肺气肿" }, { "start_idx": 20, "end_idx": 28, "type": "dis", "entity": "emphysema" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "终末支气管" }, { "start_idx": 44, "end_idx": 50, "type": "bod", "entity": "呼吸性细支气管" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "肺泡管" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "肺泡囊" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "肺泡" } ] }, { "text": "肺气肿可分三类(1)代偿性肺气肿:由于部分肺组织损坏,容积缩小,健康肺膨胀,填补空隙而形成代偿性肺气肿,多见于肺不张、脓胸、气胸等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肺气肿" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "代偿性肺气肿" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺组织" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肺" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "代偿性肺气肿" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "肺不张" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "脓胸" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "气胸" } ] }, { "text": "(2)梗阻性肺气肿:由于气管异物、支气管内膜结核、肺炎、支气管炎、百日咳、支气管哮喘等,导致支气管壁痉挛、狭窄及管腔内黏稠分泌物堵塞,形成活瓣,吸气时支气管腔扩大,吸入空气多,呼气时支气管管腔缩小,呼出空气少。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "梗阻性肺气肿" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "气管异物" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "支气管内膜结核" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "肺炎" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "支气管炎" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "百日咳" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "支气管哮喘" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "支气管壁" }, { "start_idx": 91, "end_idx": 95, "type": "bod", "entity": "支气管管腔" } ] }, { "text": "或由于心脏扩大、肺动脉扩张、淋巴结肿大、纵隔肿瘤等压迫导致外因性支气管阻塞。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肺动脉" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "淋巴结" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "纵隔肿瘤" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "支气管" } ] }, { "text": "(3)间质性肺气肿:剧烈咳嗽等情况下肺泡破裂,空气进入肺间质组织内而形成,空气可沿血管或淋巴管逆行至纵隔,形成纵隔气肿,亦可产生颈胸部皮下气肿和气胸。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "间质性肺气肿" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肺泡" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "肺间质组织" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "血管" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "淋巴管" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "纵隔气肿" }, { "start_idx": 64, "end_idx": 73, "type": "dis", "entity": "颈胸部皮下气肿和气胸" } ] }, { "text": "先天性肺叶气肿在本章第一节中讨论。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "先天性肺叶气肿" } ] }, { "text": "症状随病因及受累范围和肺膨胀程度不同而异。", "entities": [ { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "肺" } ] }, { "text": "一叶以上肺气肿常有严重呼吸困难、发绀等症状。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "肺气肿" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "严重呼吸困难" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "发绀" } ] }, { "text": "听诊肺呼吸音减弱、遥远或消失,叩诊肺部有轻度或明显的鼓音,若一侧发生重度肺气肿,则纵隔移向对侧。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "sym", "entity": "肺呼吸音减弱、遥远或消失" }, { "start_idx": 15, "end_idx": 27, "type": "sym", "entity": "叩诊肺部有轻度或明显的鼓音" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "纵隔移向对侧" } ] }, { "text": "X线透视起重要的诊断作用,表现为病侧肋间距较大,患区肺透亮度增强,膈肌运动受限、位置较低,心影移向健侧。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "X线透视" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "病侧肋间距较大" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "患区肺透亮度增强" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "膈肌运动受限" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "位置较低" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "心影移向健侧" } ] }, { "text": "两侧肺气肿者,心影较为狭小。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "心影较为狭小" } ] }, { "text": "治疗包括去除病因和对症治疗。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "去除病因" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "对症治疗" } ] }, { "text": "采用支气管解痉药及化痰药雾化吸入。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "支气管解痉药" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "化痰药雾化吸入" } ] }, { "text": "第五章骨髓增生异常综合征骨髓增生异常综合征(myelodysplasticsyndrome,MDS)是一组临床表现为难治性贫血、感染和出血,外周血象表现为血细胞减少,骨髓为活跃或明显活跃增生,三系有病态造血,或原始细胞和早期细胞增多的综合征。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "骨髓增生异常综合征" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "骨髓增生异常综合征" }, { "start_idx": 22, "end_idx": 44, "type": "dis", "entity": "myelodysplasticsyndrome" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "MDS" }, { "start_idx": 58, "end_idx": 62, "type": "sym", "entity": "难治性贫血" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "感染" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "出血" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "血细胞" }, { "start_idx": 107, "end_idx": 108, "type": "bod", "entity": "细胞" }, { "start_idx": 110, "end_idx": 119, "type": "dis", "entity": "早期细胞增多的综合征" } ] }, { "text": "1953年Block等首先称之为白血病前期(preleukemia),简称“白前”。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "白血病" } ] }, { "text": "但并非所有的“白前”均转化为白血病,“白前”的诊断仅合适于已转化为白血病的回顾性诊断,因此1976年巴黎会议建议将这一组疾病称之为骨髓增生异常综合征,并渐被广泛接受。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "白血病" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "白血病" }, { "start_idx": 65, "end_idx": 73, "type": "dis", "entity": "骨髓增生异常综合征" } ] }, { "text": "【分类】2003年Hasle等参照成人MDS的WHO诊断分型标准提出了一个儿童MDS的WHO分型标准(表10-10),并提出了儿童MDS的最低诊断标准,认为至少符合以下四项中的任何两项方可诊断为MDS:1.持续性不能解释的血细胞减少(中性粒细胞减少、血小板减少或贫血);2.至少二系有发育异常的形态学特征;3.造血细胞存在获得性克隆性细胞遗传学异常;4.原始细胞增高(≥5%)。", "entities": [ { "start_idx": 111, "end_idx": 113, "type": "bod", "entity": "血细胞" }, { "start_idx": 117, "end_idx": 121, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 125, "end_idx": 127, "type": "bod", "entity": "血小板" }, { "start_idx": 131, "end_idx": 132, "type": "dis", "entity": "贫血" }, { "start_idx": 155, "end_idx": 158, "type": "bod", "entity": "造血细胞" }, { "start_idx": 177, "end_idx": 180, "type": "bod", "entity": "原始细胞" } ] }, { "text": "按FAB标准诊断的儿童难治性贫血(RA)患儿与成人RA患者相比具有以下几点主要区别:①外周血贫血(Hb<100g/L)所占比例较低(46%),主要表现为中性粒细胞绝对值(ANC)减少(其中ANC<0.5×109/L比例为27%)和/或血小板数减低(<150×109/L比例为75%);②骨髓增生减低比例较高(43%);③粒细胞系统和巨核细胞系统发育异常的细胞形态学改变与疾病演进和预后无相关性。", "entities": [ { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "外周血贫血" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "Hb" }, { "start_idx": 76, "end_idx": 80, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "ANC" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "ANC" }, { "start_idx": 123, "end_idx": 125, "type": "bod", "entity": "血小板" }, { "start_idx": 155, "end_idx": 156, "type": "bod", "entity": "骨髓" }, { "start_idx": 172, "end_idx": 174, "type": "bod", "entity": "粒细胞" }, { "start_idx": 178, "end_idx": 181, "type": "bod", "entity": "巨核细胞" }, { "start_idx": 189, "end_idx": 190, "type": "bod", "entity": "细胞" } ] }, { "text": "表10-14儿童骨髓增生异常和骨髓增殖性疾病的诊断分类因此,采用难治性血细胞减少(RC)的定义而非RA。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "骨髓" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "骨髓" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "难治性血细胞减少" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "RC" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "RA" } ] }, { "text": "RC的确诊,特别是无克隆性染色体核型异常患儿,有时显得较困难。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "RC" } ] }, { "text": "首先需能除外感染、代谢性疾病、营养缺乏症、药物。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "感染" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "营养缺乏症" } ] }, { "text": "【临床表现】(一)MDS的临床表现多样,通常起病隐匿,症状轻重取决于贫血、白细胞和血小板减少的程度和速度。", "entities": [ { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "贫血" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "白细胞" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "血小板" } ] }, { "text": "出血常表现为皮肤黏膜瘀点和瘀斑,重者反复鼻衄、牙龈渗血、血尿、消化道出血,甚至颅内出血,有出血表现者约占MDS患者的60%~80%。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "皮肤黏膜瘀点" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "瘀斑" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "反复鼻衄" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "牙龈渗血" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "血尿" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "消化道出血" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "颅内出血" } ] }, { "text": "肝、脾大者较多见,但淋巴结增大者不多,约5%~20%。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肝" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "脾" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "淋巴结" } ] }, { "text": "(二)儿童MDSFAB亚型的特异表现儿童MDS与成人不同,以外周血细胞减少的增生低下型MDS多见,幼稚细胞增多向白细胞转化的MDS相对少见。", "entities": [ { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "血细胞" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "幼稚细胞" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "白细胞" } ] }, { "text": "幼年型慢性粒单核细胞白血病(juvenilemyelomonocyticleukemia,JMML)是儿童特有的MDS亚类。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "dis", "entity": "幼年型慢性粒单核细胞白血病" }, { "start_idx": 14, "end_idx": 43, "type": "dis", "entity": "juvenilemyelomonocyticleukemia" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "JMML" } ] }, { "text": "1.JMML也称JCMML在临床血液学、细胞生物学和分子学等方面与成人慢性髓系白血病(CML)明显不同。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "JMML" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "JCMML" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "白血病" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "CML" } ] }, { "text": "外周血中白细胞计数及单核细胞绝对数增多,贫血、血小板减少,血液中胎儿血红蛋白(HbF)持续性的明显增高细胞及单核细胞增多,巨核细胞减少,病态造血的特征常不明显,6%~24%的患儿表现有7号染色体单体(-7),体外培养CFU-GM呈自发性生长,对GM-CSF刺激敏感性增高,患儿对化疗反应不敏感,生存期短,但急性白血病转化率相对较低,多数患儿死于骨髓衰竭并发症。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "外周血" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "白细胞" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "单核细胞" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "贫血" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "血小板减少" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "血液" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "HbF" }, { "start_idx": 29, "end_idx": 50, "type": "sym", "entity": "血液中胎儿血红蛋白(HbF)持续性的明显增高" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "细胞" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "单核细胞" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "巨核细胞" }, { "start_idx": 153, "end_idx": 157, "type": "dis", "entity": "急性白血病" }, { "start_idx": 172, "end_idx": 178, "type": "dis", "entity": "骨髓衰竭并发症" } ] }, { "text": "患儿经常发生感染,肝、脾、淋巴结增大Down综合征、Fanconi综合征、神经纤维瘤Ⅰ型(NF-1)、Bloom综合征、先天性中性粒细胞减少、血小板储存池病、家族性-7综合征、线粒体细胞病、非特异性免疫缺陷以及不能分类的其他先天性异常等,这些患儿发病年龄大多大于2岁,AML的转化率较原发性儿童MDS为低。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "感染" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肝" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "脾" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "淋巴结" }, { "start_idx": 9, "end_idx": 17, "type": "sym", "entity": "肝、脾、淋巴结增大" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "Down综合征" }, { "start_idx": 26, "end_idx": 35, "type": "dis", "entity": "Fanconi综合征" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "神经纤维瘤Ⅰ型" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "NF-1" }, { "start_idx": 51, "end_idx": 58, "type": "dis", "entity": "Bloom综合征" }, { "start_idx": 60, "end_idx": 69, "type": "sym", "entity": "先天性中性粒细胞减少" }, { "start_idx": 71, "end_idx": 77, "type": "dis", "entity": "血小板储存池病" }, { "start_idx": 79, "end_idx": 86, "type": "dis", "entity": "家族性-7综合征" }, { "start_idx": 88, "end_idx": 93, "type": "dis", "entity": "线粒体细胞病" }, { "start_idx": 95, "end_idx": 102, "type": "sym", "entity": "非特异性免疫缺陷" } ] }, { "text": "成人WHOMDS诊断分型标准中按骨髓原始粒细胞比例将RAEB再分为RAEB-Ⅰ(骨髓原始细胞5%~9%)和RAEB-Ⅱ(骨髓原始细胞10%~19%)两型,此外,将MDS和AML骨髓原始细胞的分界降低为0.20,取消了RAEB-t亚型,但现有资料表明这并不适合儿童MDS。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "骨髓原始粒细胞" }, { "start_idx": 40, "end_idx": 45, "type": "bod", "entity": "骨髓原始细胞" }, { "start_idx": 60, "end_idx": 65, "type": "bod", "entity": "骨髓原始细胞" } ] }, { "text": "对于那些骨髓原始细胞比例在20%~30%的患儿,如无临床和儿童MDS特征性7号染色单体异常或前述原发性AML特征性染色体核型异常,应在几周后重复骨髓检查,如果骨髓原始细胞比例超过30%则诊断为AML,如果骨髓原始细胞比例保持稳定则诊断为RAEB-t。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "骨髓原始细胞" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "骨髓" }, { "start_idx": 102, "end_idx": 103, "type": "bod", "entity": "骨髓" } ] }, { "text": "【诊断】(一)外周血象常表现为一系或一系以上血细胞减少,部分患儿网织红细胞百分率有增高。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "血细胞" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "网织红细胞" } ] }, { "text": "贫血一般呈正细胞、正色素性,红细胞大小不一,可见单个核或多核有核红细胞及卵形大红细胞。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "正细胞" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "正色素性" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "红细胞" } ] }, { "text": "粒系形态变化较明显,核浆发育不平衡,可出现Pelgen-Huet畸形(分叶减少的中性粒细胞),也可伴分叶过多畸形,或中性粒细胞胞质中颗粒减少,或无颗粒以及其他的形态异常表现。", "entities": [ { "start_idx": 21, "end_idx": 33, "type": "sym", "entity": "Pelgen-Huet畸形" }, { "start_idx": 35, "end_idx": 44, "type": "bod", "entity": "分叶减少的中性粒细胞" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "畸形" } ] }, { "text": "单核细胞常可见增多。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "单核细胞" } ] }, { "text": "血小板及其颗粒常减少,可见大型血小板或形态异常,电镜下可呈空泡形成,糖原减少,微小管缺乏,小管系统扩张等变化。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "血小板" }, { "start_idx": 34, "end_idx": 53, "type": "sym", "entity": "糖原减少,微小管缺乏,小管系统扩张等变化" } ] }, { "text": "有些患儿血小板计数可正常,但有出血倾向,血小板对胶原、ADP等诱导的聚集作用异常,黏附性降低。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "血小板" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "血小板" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "ADP" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "黏附性降低" } ] }, { "text": "(二)骨髓涂片MDS的骨髓象呈现病态造血的现象。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "骨髓涂片" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "骨髓象" } ] }, { "text": "1/2~3/4患儿骨髓有核细胞增生亢进或正常,约1/4左右患儿骨髓增生减低,尤其是继发性MDS骨髓增生常低下,而骨髓增生活跃时常伴有纤维化,因此常出现骨髓不易抽出(“干抽”现象)。", "entities": [ { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "骨髓" } ] }, { "text": "红系病态造血表现为,红系增生过多(>60%)或过少(<5%),多数患儿的幼红细胞有巨幼样改变,出现环状铁粒幼红细胞、多核红细胞、核分裂、核凹陷以至核分叶、胞质染色不均匀、多嗜性红细胞及点彩红细胞,尤其MDS转变为白血病前,上述变化为较突出的表现。", "entities": [ { "start_idx": 49, "end_idx": 56, "type": "bod", "entity": "环状铁粒幼红细胞" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "多核红细胞" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "核分裂" }, { "start_idx": 77, "end_idx": 83, "type": "bod", "entity": "胞质染色不均匀" }, { "start_idx": 85, "end_idx": 90, "type": "bod", "entity": "多嗜性红细胞" }, { "start_idx": 92, "end_idx": 96, "type": "bod", "entity": "点彩红细胞" }, { "start_idx": 106, "end_idx": 108, "type": "dis", "entity": "白血病" } ] }, { "text": "粒系病态造血表现为,颗粒减少或缺如或过大,成熟粒细胞胞质仍嗜碱,呈核浆发育不平衡表现,细胞核分叶过少(Pelger-Hüet异常)或过多。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "核浆" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "细胞核分叶过少" }, { "start_idx": 51, "end_idx": 63, "type": "sym", "entity": "Pelger-Hüet异常" } ] }, { "text": "巨核系病态造血表现为巨核细胞减少,出现小巨核细胞、大单个核巨核细胞、多核巨核细胞、胞质中颗粒加大或形态异常。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "巨核细胞" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "巨核细胞" }, { "start_idx": 25, "end_idx": 32, "type": "bod", "entity": "大单个核巨核细胞" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "多核巨核细胞" } ] }, { "text": "小巨核细胞及巨大血小板偶尔出现在外周血中。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "小巨核细胞" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "巨大血小板" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "外周血" } ] }, { "text": "(三)骨髓活检除了观察骨髓中细胞学改变之外,还可见到下列主要的组织学变化红系前体细胞成熟过程障碍,常形成分化在同一阶段的幼红细胞岛,伴有早幼红细胞增多,骨髓中原粒细胞和早幼粒细胞离开骨小梁附近呈中心性簇生,这些异位的原粒和早幼粒细胞形成聚集(>5个粒系前体细胞)或小簇(3~5个粒系前体细胞),称为异位的不成熟前体细胞(abnormallocalizationofimmatureprecursor,ALIP),巨核细胞形态异常,表现为体积有显著的大小不一,细胞核呈低分叶的鹿角样和不规则的过多分叶,小型巨核细胞(体积仅为正常的1/6)普遍多见。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "骨髓活检" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "骨髓" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "幼红细胞" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "红细胞" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "骨髓" }, { "start_idx": 111, "end_idx": 115, "type": "bod", "entity": "早幼粒细胞" }, { "start_idx": 149, "end_idx": 158, "type": "bod", "entity": "异位的不成熟前体细胞" }, { "start_idx": 160, "end_idx": 198, "type": "bod", "entity": "abnormallocalizationofimmatureprecursor" }, { "start_idx": 200, "end_idx": 203, "type": "bod", "entity": "ALIP" }, { "start_idx": 206, "end_idx": 209, "type": "bod", "entity": "巨核细胞" }, { "start_idx": 250, "end_idx": 255, "type": "bod", "entity": "小型巨核细胞" } ] }, { "text": "骨髓组织内细胞增生活跃者(造血组织>50%)约60%~70%,部分患者增生正常(造血组织30%~50%),少数患者骨髓造血细胞增生减低(<30%)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "骨髓组织" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "细胞" }, { "start_idx": 57, "end_idx": 62, "type": "bod", "entity": "骨髓造血细胞" } ] }, { "text": "上述变化中,尤其是ALIP不仅有诊断价值,而且对估计MDS的预后有价值,有ALIP的患儿约有40%可发展成急性粒细胞白血病,平均生存期约16个月,无ALIP的MDS患儿仅10%发展成急性粒细胞白血病,平均生存期为33个月。", "entities": [ { "start_idx": 53, "end_idx": 60, "type": "dis", "entity": "急性粒细胞白血病" }, { "start_idx": 91, "end_idx": 98, "type": "dis", "entity": "急性粒细胞白血病" } ] }, { "text": "(四)细胞遗传学较常见的染色体异常有5q-,-7,+8,+21,7q-,假二倍体,亚二倍体,超二倍体,21-4体及-5等。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "染色体异常" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "5q-" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "-7" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "+8" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "+21" }, { "start_idx": 32, "end_idx": 34, "type": "sym", "entity": "7q-" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "假二倍体" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "亚二倍体" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "超二倍体" }, { "start_idx": 51, "end_idx": 55, "type": "sym", "entity": "21-4体" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "-5" } ] }, { "text": "极少数可出现ph染色体。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "ph染色体" } ] }, { "text": "5q-综合征患儿均有第5号染色体长臂缺失(其断裂点位置常在2区或3区)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "5q-综合征" } ] }, { "text": "细胞遗传学改变对MDS预后方面有以下共同特点:①正常核型者比异常核型者好;②单一异常者比多种异常者好(-7或7q-例外);③核型稳定者比核型演变者好。", "entities": [ { "start_idx": 24, "end_idx": 35, "type": "sym", "entity": "正常核型者比异常核型者好" }, { "start_idx": 38, "end_idx": 49, "type": "sym", "entity": "单一异常者比多种异常者好" }, { "start_idx": 62, "end_idx": 73, "type": "sym", "entity": "核型稳定者比核型演变者好" } ] }, { "text": "(五)造血干细胞培养一般采用Pike和Robinson建立的造血干细胞培养技术。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "造血干细胞" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "造血干细胞" } ] }, { "text": "MDS时有明显的粒细胞-单核细胞集落形成单位(CFU-GM)形成障碍。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "bod", "entity": "粒细胞-单核细胞" } ] }, { "text": "凡在琼脂中生长形成3~20个细胞的细胞团称为小簇,形成21~40个细胞者称为大簇,形成41个以上细胞者称为集落。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "细胞" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "细胞" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "细胞" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "细胞" } ] }, { "text": "正常人CFU-GM体外培养形成中性粒细胞、单核、巨噬细胞或粒细胞性混合集落,细胞分化和形态均正常。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "单核" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "粒细胞性" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "细胞" } ] }, { "text": "MDS的CFU-GM体外培养结果往往集落数低下,细胞集落和细胞簇中细胞成熟度及两者间比例显著低于正常对照组,为急性白血病相似的集落形成和细胞分化障碍。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "细胞" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "细胞" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "细胞" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "急性白血病" } ] }, { "text": "(六)MDS患者机体免疫功能有多种变化,有体液免疫异常和细胞免疫异常的各种表现,但无特异性,提示有免疫功能紊乱,主要以体液免疫和细胞免疫功能降低为主。", "entities": [ { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "体液免疫异常" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "细胞免疫异常" }, { "start_idx": 49, "end_idx": 54, "type": "sym", "entity": "免疫功能紊乱" } ] }, { "text": "【治疗】支持疗法是MDS最基本的治疗措施,贫血严重者输血或少浆红细胞,感染时用相应的抗生素。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "贫血" }, { "start_idx": 29, "end_idx": 33, "type": "dru", "entity": "少浆红细胞" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "感染" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "抗生素" } ] }, { "text": "(一)造血干细胞移植因造血干细胞移植唯一能使MDS治愈,如患儿一般情况好,应积极考虑作造血干细胞移植治疗,争取治愈。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "造血干细胞移植" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "造血干细胞移植" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "MDS" }, { "start_idx": 43, "end_idx": 49, "type": "pro", "entity": "造血干细胞移植" } ] }, { "text": "大约50%的患者可以通过造血干细胞移植得到治愈,但不同的MDS亚型移植时机是不一样的,伴有幼稚细胞增多的MDS因为随时可能向白血病转化,且一旦转化成白血病治疗难度是很大的,所以应该尽早移植。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "造血干细胞移植" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "MDS" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "幼稚细胞" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "MDS" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "白血病" }, { "start_idx": 74, "end_idx": 76, "type": "dis", "entity": "白血病" } ] }, { "text": "不伴有幼稚细胞增高的MDS一般病情进展缓慢,有较长的稳定期,研究发现早移植与晚移植的疗效是没有差别的,所以一般不需要马上移植,只有当病情进展到反复输血依赖时才需要尽早移植。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "幼稚细胞" } ] }, { "text": "对于伴有-7染色体异常的MDS,因为其病情进展比较快,所以也应该尽早移植。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "-7染色体异常" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "MDS" } ] }, { "text": "作为儿童MDS的特有亚型-JMML,造血干细胞移植前患者往往伴有明显肝脾大脾脏是否移植前需要切脾有一定的争议,虽然切脾有助于植入、有助于减少血小板的输注,但来自欧洲EWOG-MDS100例儿童JMML移植资料提示切脾并不能提高疗效,所以推荐移植前不必要切脾。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "儿童MDS" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "亚型-JMML" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "造血干细胞移植" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肝脾" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "明显肝脾大" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "脾脏" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "脾" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "脾" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "血小板" }, { "start_idx": 94, "end_idx": 99, "type": "dis", "entity": "儿童JMML" } ] }, { "text": "RAEBT患者移植前是否需要化疗就有很大争议,临床实践中往往从两个方面可以帮助我们做出决定,第一我们可以看看这些病人有否非随机的染色体异常,如:t(8,21)或inv16,如果伴有这样的染色体异常,即使幼稚细胞比例没有达到30%,也已经是经典的AML了,也可以在严密观察下随访等待看幼稚细胞是否马上升高。", "entities": [ { "start_idx": 101, "end_idx": 104, "type": "bod", "entity": "幼稚细胞" }, { "start_idx": 122, "end_idx": 124, "type": "dis", "entity": "AML" }, { "start_idx": 141, "end_idx": 144, "type": "bod", "entity": "幼稚细胞" } ] }, { "text": "因此目前一般认为伴有幼稚细胞增高的MDS患者不必要接受化疗,应该直接移植。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "幼稚细胞" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "MDS" } ] }, { "text": "因为移植治疗是MDS患者获得治愈的唯一希望,其移植指针应该比任何类型的白血病还要强,所以一旦诊断明确,应积极寻找供体准备移植,为了防止病情变化,RAEB、RAEBT患者不能花更多时间在选择供体上,即使是配型条件较差的非血缘相关供体甚至半相合供体都应积极考虑,以争取时间。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "MDS" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "白血病" } ] }, { "text": "(二)化学治疗1.小剂量阿糖胞苷剂量为10~20mg/M2,每日1~2次,皮下注射10日至10月,完全缓解者约30%,部分缓解者约30%,似乎延长存活期。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "阿糖胞苷" } ] }, { "text": "2.小剂量三尖杉酯碱0.5~1mg静滴,每日或隔日1次,10~15次为一疗程,休息5~10日,再接下一疗程。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "三尖杉酯碱" } ] }, { "text": "副作用是骨髓抑制。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "骨髓" } ] }, { "text": "3.联合化疗常用联合化疗方案有HOAP、HA、VP-16+Arc-C、COAP、DA等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "联合化疗" } ] }, { "text": "但联合化疗后骨髓抑制持续的时间比急性白血病化疗后骨髓抑制时间长,且不易恢复,病态造血也难以纠正,容易并发致死性的严重感染,故宜慎重。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "骨髓" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "急性白血病" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "骨髓" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "感染" } ] }, { "text": "(三)其他包括免疫抑制药(环孢霉素、ATG)和DNA甲基化酶抑制药[5-氮杂胞苷(azacytidine,5AC)和地西他滨(decitabine,DAC)],除有ATG治疗儿童MDS的小系列报道外,其他药物极少有用于儿童MDS的研究报道。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "免疫抑制药" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "环孢霉素" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "ATG" }, { "start_idx": 23, "end_idx": 32, "type": "dru", "entity": "DNA甲基化酶抑制药" }, { "start_idx": 34, "end_idx": 39, "type": "dru", "entity": "5-氮杂胞苷" }, { "start_idx": 41, "end_idx": 51, "type": "dru", "entity": "azacytidine" }, { "start_idx": 53, "end_idx": 55, "type": "dru", "entity": "5AC" }, { "start_idx": 58, "end_idx": 61, "type": "dru", "entity": "地西他滨" }, { "start_idx": 63, "end_idx": 72, "type": "dru", "entity": "decitabine" }, { "start_idx": 74, "end_idx": 76, "type": "dru", "entity": "DAC" }, { "start_idx": 87, "end_idx": 91, "type": "dis", "entity": "儿童MDS" }, { "start_idx": 109, "end_idx": 113, "type": "dis", "entity": "儿童MDS" } ] }, { "text": "全反式维A酸对MDS剂量为每日20~60mg/m2,疗程1~9个月。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "全反式维A酸" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "MDS" } ] }, { "text": "第六节幼儿急疹幼儿急疹(exanthemsubitum)又称婴儿玫瑰疹(roseolainfantum),是常见于婴幼儿的急性出疹性传染病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "幼儿急疹" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "幼儿急疹" }, { "start_idx": 12, "end_idx": 26, "type": "dis", "entity": "exanthemsubitum" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "婴儿玫瑰疹" }, { "start_idx": 36, "end_idx": 50, "type": "dis", "entity": "roseolainfantum" }, { "start_idx": 61, "end_idx": 68, "type": "dis", "entity": "急性出疹性传染病" } ] }, { "text": "临床特征为高热3~4天,然而骤然退热并出现皮疹,病情很快恢复。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "高热" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "骤然退热" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "皮疹" } ] }, { "text": "【病原和流行病学】1988年,从急疹患儿外周血淋巴细胞中分离到人类疱疹6型(humanherpervirus6,HHV-6)B组病毒,患者脑脊液中也可见HHV-6B病毒。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "急疹" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "外周血淋巴细胞" }, { "start_idx": 33, "end_idx": 65, "type": "mic", "entity": "疱疹6型(humanherpervirus6,HHV-6)B组病毒" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "脑脊液" }, { "start_idx": 76, "end_idx": 83, "type": "mic", "entity": "HHV-6B病毒" } ] }, { "text": "患者血清中抗HHV-6抗体有意义地升高。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血清" }, { "start_idx": 5, "end_idx": 12, "type": "bod", "entity": "抗HHV-6抗体" } ] }, { "text": "HHV-6还可引起婴儿发生无皮疹的急性发热性疾病。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "HHV-6" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "皮疹" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "急性发热性疾病" } ] }, { "text": "感染后获持久免疫,偶见第2次发病。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "感染" } ] }, { "text": "(一)发热期常突起高热,持续3~5天。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "突起高热" } ] }, { "text": "高热初期可伴惊厥。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "高热" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "惊厥" } ] }, { "text": "表现为高热与轻微的症状及体征不相称。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "高热" } ] }, { "text": "(二)出疹期病程第3~5天体温骤然退至正常,同时或稍后出现皮疹。", "entities": [ { "start_idx": 8, "end_idx": 20, "type": "sym", "entity": "第3~5天体温骤然退至正常" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "皮疹" } ] }, { "text": "皮疹散在,为玫瑰红色斑疹或斑丘疹,压之褪色,很少融合。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "皮疹散在" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "玫瑰红色斑疹" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "斑丘疹" } ] }, { "text": "偶有并发脑炎和血小板减少性紫癜的报告。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "脑炎" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "血小板减少性紫癜" } ] }, { "text": "【实验室检查】血常规检查见白细胞总数减少,伴中性粒细胞减少。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 7, "end_idx": 11, "type": "ite", "entity": "血常规检查" }, { "start_idx": 13, "end_idx": 17, "type": "ite", "entity": "白细胞总数" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "白细胞总数减少" }, { "start_idx": 22, "end_idx": 26, "type": "ite", "entity": "中性粒细胞" } ] }, { "text": "【诊断】在发热期诊断比较困难,不过,从患儿全身症状轻微与高热表现不一致,周围血象中白细胞总数减少,应考虑之。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "发热" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "高热" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "血象" }, { "start_idx": 41, "end_idx": 45, "type": "ite", "entity": "白细胞总数" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "白细胞总数减少" } ] }, { "text": "一旦高热骤退,同时出现皮疹,诊断就不难建立。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "高热" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "皮疹" } ] }, { "text": "在出现症状3天内可从外周血淋巴细胞和唾液中分离HHV-6,或用核酸杂交技术检测病毒基因进行病原诊断。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "外周血淋巴细胞" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "唾液" }, { "start_idx": 23, "end_idx": 27, "type": "mic", "entity": "HHV-6" }, { "start_idx": 31, "end_idx": 36, "type": "pro", "entity": "核酸杂交技术" }, { "start_idx": 39, "end_idx": 42, "type": "ite", "entity": "病毒基因" } ] }, { "text": "【治疗】一般不需特殊治疗,主要是对症处理,尤其对高热患者应予以退热镇静剂;加强水分和营养供给。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "高热" }, { "start_idx": 31, "end_idx": 35, "type": "dru", "entity": "退热镇静剂" }, { "start_idx": 37, "end_idx": 45, "type": "pro", "entity": "加强水分和营养供给" } ] }, { "text": "五、造血干细胞采集与保存(一)动员外周血干细胞移植时需作干细胞动员,供体接受粒细胞集落刺激因子(G-CSF)5~10μg/(kg•d)4~6天后,采集1~2天,即可满足需要。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "造血干细胞" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "动员" }, { "start_idx": 18, "end_idx": 25, "type": "pro", "entity": "外周血干细胞移植" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "干细胞动员" }, { "start_idx": 39, "end_idx": 47, "type": "dis", "entity": "粒细胞集落刺激因子" }, { "start_idx": 49, "end_idx": 53, "type": "dru", "entity": "G-CSF" } ] }, { "text": "(二)采集方式和目标细胞数量1.通过外周血细胞分离仪采集造血干细胞如需进一步去除其中的淋巴细胞时使用带有目的单克隆抗体磁珠的血细胞分选仪(如CD34+)分选出需要的细胞(如CD34阳性细胞)。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "细胞" }, { "start_idx": 18, "end_idx": 25, "type": "equ", "entity": "外周血细胞分离仪" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "造血干细胞" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "单克隆抗体" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "血细胞分选仪" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "CD34+" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "细胞" }, { "start_idx": 92, "end_idx": 99, "type": "bod", "entity": "CD34阳性细胞" } ] }, { "text": "2.骨髓采集需在无菌环境下进行(一般在手术室中),在腰麻或全身麻醉下于髂后上棘多部位穿刺采集,一般每个穿刺点采集5ml,根据有核细胞计数或CD34细胞定量决定采髓总量。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "骨髓" }, { "start_idx": 19, "end_idx": 21, "type": "equ", "entity": "手术室" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "腰麻" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "全身麻醉" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "髂" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "上棘" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "穿刺采集" }, { "start_idx": 62, "end_idx": 67, "type": "ite", "entity": "有核细胞计数" }, { "start_idx": 69, "end_idx": 76, "type": "ite", "entity": "CD34细胞定量" }, { "start_idx": 79, "end_idx": 80, "type": "pro", "entity": "采髓" } ] }, { "text": "3.脐带血采集在分娩室进行,断脐后立即在胎盘端脐静脉中采集,分离出脐血中单个核细胞冷冻保存待用。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "脐带血采集" }, { "start_idx": 8, "end_idx": 10, "type": "dep", "entity": "分娩室" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "胎盘" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "脐静脉" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "脐血" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "核细胞" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "冷冻保存" } ] }, { "text": "如受体为小年龄患儿,1个患儿的脐带血就足够。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "脐带血" } ] }, { "text": "4.干细胞数量Mifin等认为移植量后中性粒细胞及血小板的恢复迅速。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "干细胞" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "移植量" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "血小板" } ] }, { "text": "单个核细胞作为传统衡量标准,平均为4×108/kg;粒-单集落形成单位(GM-CFU)平均为5×105/kg;而自身移植时核细胞2×108/kg就已够。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "核细胞" }, { "start_idx": 26, "end_idx": 30, "type": "ite", "entity": "粒-单集落" }, { "start_idx": 36, "end_idx": 41, "type": "ite", "entity": "GM-CFU" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "自身移植" }, { "start_idx": 61, "end_idx": 63, "type": "ite", "entity": "核细胞" } ] }, { "text": "(三)保存与回输同种异体非脐血干细胞移植采集后可立即回输,无需保存。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "回输" }, { "start_idx": 10, "end_idx": 19, "type": "pro", "entity": "异体非脐血干细胞移植" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "回输" } ] }, { "text": "自身移植时则需根据预处理方案所需的时间,如在48~72小时内回输干细胞,则4℃保存;而超过72小时者,则需加二甲基亚砜及人血清清蛋白后,在-80~-196℃超低温保存。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "自身移植" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "回输" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "干细胞" }, { "start_idx": 54, "end_idx": 58, "type": "dru", "entity": "二甲基亚砜" }, { "start_idx": 60, "end_idx": 65, "type": "bod", "entity": "人血清清蛋白" }, { "start_idx": 78, "end_idx": 82, "type": "pro", "entity": "超低温保存" } ] }, { "text": "脐血干细胞均在-80~-196℃超低温保存待用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "脐血干细胞" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "超低温保存" } ] }, { "text": "异体非脐血移植时造血干细胞采集后直接经静脉输给病人。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "异体非脐血移植" }, { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "造血干细胞采集" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "静脉" } ] }, { "text": "自体和脐血移植时取出保存于液氮中的造血干细胞,在38℃的水箱中快速解冻后即输给病人。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "自体和脐血移植" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "造血干细胞" }, { "start_idx": 28, "end_idx": 29, "type": "equ", "entity": "水箱" } ] }, { "text": "4℃保存的自身干细胞可直接回输。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "干细胞" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "回输" } ] }, { "text": "第七节心脏电生理检查临床心电学在帮助对各种心脏疾病尤其是心律失常的诊断中发挥了重要的作用。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "心脏电生理检查" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "心律失常" } ] }, { "text": "但是心电图作为一种诊断手段受限于它的经验与推论应用原理,对心律失常的确切机制的认识以及复杂心律失常的诊断都是远不够的。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "心电图" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "心律失常" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "心律失常" } ] }, { "text": "自从1969年经静脉记录希氏束电图的导管技术应用于临床,有创性心脏电生理检查发展很快。", "entities": [ { "start_idx": 7, "end_idx": 21, "type": "pro", "entity": "经静脉记录希氏束电图的导管技术" }, { "start_idx": 28, "end_idx": 37, "type": "pro", "entity": "有创性心脏电生理检查" } ] }, { "text": "心脏电生理检查的基本内容是在自身心律或起搏引起的心律时,记录心内电活动,分析其表现和特征,加以推理,作出综合判断,为临床医生提供关于心律失常的正确诊断,发生机制研究,治疗方法选择和预后判断等方面的重要的甚或决定性的依据。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "心脏电生理检查" }, { "start_idx": 66, "end_idx": 69, "type": "dis", "entity": "心律失常" } ] }, { "text": "(一)指征心脏电生理检查的指征可分为诊断性与治疗性。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "心脏电生理检查" } ] }, { "text": "行导管消融术时的心脏电生理检查绝对必要,这也是大多数电生理实验室的最常用指征。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "导管消融术" }, { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "心脏电生理检查" }, { "start_idx": 26, "end_idx": 31, "type": "dep", "entity": "电生理实验室" } ] }, { "text": "1.缓慢性心律失常的诊断与处理获得电生理资料来证实一些患者的症状是由发作性缓慢性心律失常引起;确定房室传导阻滞发生的水平。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "缓慢性心律失常" }, { "start_idx": 34, "end_idx": 43, "type": "dis", "entity": "发作性缓慢性心律失常" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "房室" } ] }, { "text": "2.快速性心律失常的诊断与处理确定窄QRS心动过速的机制;确定宽QRS心动过速的机制;诱发临床上出现的窄QRS心动过速及宽QRS心动过速,并进行标测与经导管消融。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "快速性心律失常" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "窄QRS心动过速" }, { "start_idx": 31, "end_idx": 38, "type": "dis", "entity": "宽QRS心动过速" }, { "start_idx": 51, "end_idx": 58, "type": "dis", "entity": "窄QRS心动过速" }, { "start_idx": 60, "end_idx": 67, "type": "dis", "entity": "宽QRS心动过速" }, { "start_idx": 75, "end_idx": 79, "type": "pro", "entity": "经导管消融" } ] }, { "text": "3.不明原因晕厥的诊断评价窦房结功能与房室传导时间;在有晕厥与器质性心脏病的患者检查是否有可诱发的持续性室性快速性心律失常存在。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "晕厥" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "窦房结" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "房室" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "晕厥" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "器质性心脏病" }, { "start_idx": 49, "end_idx": 60, "type": "dis", "entity": "持续性室性快速性心律失常" } ] }, { "text": "4.一级预防方案在患有冠状动脉病变、心功能下降和非持续性室性心动过速的患者行电生理检查以了解是否有程序电刺激诱发的室性心动过速与室颤,筛选ICD治疗患者。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "冠状动脉病变" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "心功能下降" }, { "start_idx": 24, "end_idx": 33, "type": "dis", "entity": "非持续性室性心动过速" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "电生理检查" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "室性心动过速" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "室颤" }, { "start_idx": 69, "end_idx": 73, "type": "pro", "entity": "ICD治疗" } ] }, { "text": "5.其他个体化用途为接受植入心脏转律除颤器(ICD)治疗患者选择最佳ICD参数;评价心脏外科手术的疗效;评价抗心律失常药物的疗效(少用)。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "植入" }, { "start_idx": 14, "end_idx": 20, "type": "equ", "entity": "心脏转律除颤器" }, { "start_idx": 22, "end_idx": 24, "type": "equ", "entity": "ICD" }, { "start_idx": 34, "end_idx": 36, "type": "equ", "entity": "ICD" }, { "start_idx": 42, "end_idx": 45, "type": "dep", "entity": "心脏外科" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "心律失常" } ] }, { "text": "(二)设备与人员北美心脏起搏和电生理学会于1992年制订了关于进行导管消融术的人员和设备配置的基本要求,现介绍如下(供参考):1.设备(1)房间:1)为了有创性电生理研究的目的。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心脏" }, { "start_idx": 33, "end_idx": 37, "type": "pro", "entity": "导管消融术" } ] }, { "text": "2)有处理急性冠状动脉并发症(血栓血栓、冠状动脉痉挛、心包填塞)的仪器和装备。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "急性冠状动脉并发症" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "血栓" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "冠状动脉痉挛" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "心包填塞" } ] }, { "text": "(2)支援:1)随时可支援的心外科班子。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dep", "entity": "心外科" } ] }, { "text": "2)进行经皮冠状动脉腔内成形术(PTCA)必需设备和器材。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "pro", "entity": "经皮冠状动脉腔内成形术" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "PTCA" } ] }, { "text": "3)临时性和永久性心脏起搏器植入的必要条件。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "pro", "entity": "临时性和永久性心脏起搏器植入" } ] }, { "text": "2.人员(1)医生:1)经过导管消融术训练的合格医生1名。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "导管消融术" } ] }, { "text": "2)经过心导管术训练的操纵导管的医生或专科进修医生1名。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "心导管术" }, { "start_idx": 13, "end_idx": 14, "type": "equ", "entity": "导管" } ] }, { "text": "(2)实验室人员:1)护士或助理医生,监护患者,给予镇静药。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "镇静药" } ] }, { "text": "2)护士或技术员,进行与消融术有关的工作。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "消融术" } ] }, { "text": "3.仪器设备(1)X线:1)最低要求是可转动的C形臂式更佳,但并非必需。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "X线" } ] }, { "text": "3)质量先进的X线系统以减少X线曝光时间。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "X线" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "X线" } ] }, { "text": "(2)一般性的:为了电生理检查和导管消融治疗所用的各种仪器和器材。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "电生理检查" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "导管消融治疗" } ] }, { "text": "(三)导管的置入与定位在绝大多数患者,可通过经皮方法自上肢或下肢的血管将电极导管放置于心腔内。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "equ", "entity": "导管" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "经皮方法" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "上肢" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "下肢" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "血管" }, { "start_idx": 36, "end_idx": 39, "type": "equ", "entity": "电极导管" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "心腔" } ] }, { "text": "经皮穿刺的优点是快速,疼痛较轻,随时可改用电极导管,并且被穿刺过的血管(主要是静脉,有时是动脉)经几天便可恢复。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "经皮穿刺" }, { "start_idx": 21, "end_idx": 24, "type": "equ", "entity": "电极导管" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "穿刺" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "血管" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "静脉" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "动脉" } ] }, { "text": "电极导管可经由股静脉、上肢的正中静脉、锁骨下静脉以及颈内静脉进入右心房或右心室,其中以股静脉最常用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "equ", "entity": "电极导管" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "股静脉" }, { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "上肢的正中静脉" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "锁骨下静脉" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "颈内静脉" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "右心房" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "右心室" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "股静脉" } ] }, { "text": "为进入左心室,须经穿刺股动脉逆向地插入电极导管。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "左心室" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "经穿刺股动脉" }, { "start_idx": 19, "end_idx": 22, "type": "equ", "entity": "电极导管" } ] }, { "text": "还可通过房间隔穿刺或未闭卵圆孔进入左心房或左心室。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "房间隔穿刺" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "未闭卵圆孔" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "左心房" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "左心室" } ] }, { "text": "到达希氏束位置可通过股静脉、锁骨下静脉、贵要静脉及颈内静脉。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "股静脉" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "锁骨下静脉" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "贵要静脉" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "颈内静脉" } ] }, { "text": "进入窦状静脉窦可通过颈内静脉、锁骨下静脉及贵要静脉。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "窦状静脉窦" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "颈内静脉" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "锁骨下静脉" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "贵要静脉" } ] }, { "text": "为记录希氏束电图,可将电极导管置于房间隔右侧面下部。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "equ", "entity": "电极导管" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "房间隔" } ] }, { "text": "右心房上部电极导管置于心房后侧壁靠近上腔静脉入口处。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "右心房上部" }, { "start_idx": 5, "end_idx": 8, "type": "equ", "entity": "电极导管" }, { "start_idx": 11, "end_idx": 24, "type": "bod", "entity": "心房后侧壁靠近上腔静脉入口处" } ] }, { "text": "右心房下部电极导管置于右心房侧壁与下腔静脉交界处。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "右心房下部" }, { "start_idx": 5, "end_idx": 8, "type": "equ", "entity": "电极导管" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "右心房侧壁" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "下腔静脉" } ] }, { "text": "冠状窦电极导管经冠状窦口插入冠状窦。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "equ", "entity": "冠状窦电极导管" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "冠状窦口" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "冠状窦" } ] }, { "text": "右心室电极导管一般置于右心室心尖部。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "equ", "entity": "右心室电极导管" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "右心室心尖部" } ] }, { "text": "(四)传导时间的测量有了同步记录的希氏束电图,体表心电图上的PR间期可进一步分为三个间期,即PA、AH和HV间期。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "pro", "entity": "心电图" } ] }, { "text": "1.PA间期从体表心电图P波的起始时相至希氏束电图A波的高大转折波的起始点之间的距离称PA间期,反映激动从右心房上部至右心房下部靠近房室结处的传导时间,正常值约20~40ms。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "心电图" }, { "start_idx": 53, "end_idx": 57, "type": "bod", "entity": "右心房上部" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "右心房下部" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "房室结" } ] }, { "text": "通常把PA间期代表心房内传导时间,但实际上PA间期只能反映右心房内的传导时间,不能反映左心房的传导时间,在实际工作中PA间期的应用价值不大。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "心房" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "右心房" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "左心房" } ] }, { "text": "2.AH间期从A波的第一个高大转折波的起点至H波的起点之间的距离称为AH间期,反映激动从右心房下部靠近房室结处至希氏束的传导时间,正常值约60~140ms。", "entities": [ { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "右心房下部" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "房室结" } ] }, { "text": "通常把AH间期代表房室结传导时间,因为从右心房下部至希氏束之间的传导时间,主要为房室结内的传导,但又由于激动进入房室结的确切时相并无标志,故AH间期只能作为房室传导的近似值,为了测量的准确,要选择重复性较好的A波高大转折波起点作为测量点,以减少测量误差。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "房室结" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "右心房下部" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "房室结" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "房室结" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "房室" } ] }, { "text": "即使如此,在一次检查中各组激动测得的AH间期的数值,仍可能有参差,这是由于房室结传导时间受自主神经系统的影响,交感神经和副交感神经的张力分别可使AH间期缩短和延长。", "entities": [ { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "房室结" }, { "start_idx": 45, "end_idx": 50, "type": "bod", "entity": "自主神经系统" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "交感神经" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "副交感神经" } ] }, { "text": "观察AH间期对心脏起搏或对药物的反映,比单纯观察AH间期更能反映房室结的功能状况。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心脏" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "房室结" } ] }, { "text": "4.HV间期从H波的起点至V波或体表心电图QRS波起点之间的距离,称为HV间期,它反映激动自希氏束经房室束,分支,浦肯野纤维至心室肌的传导时间。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "pro", "entity": "心电图" }, { "start_idx": 57, "end_idx": 61, "type": "bod", "entity": "浦肯野纤维" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "心室肌" } ] }, { "text": "HV间期受自主神经影响较小,故在同一次检查中数值比较恒定。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "自主神经" } ] }, { "text": "(五)临床心电生理检查方法临床心电生理检查的基本方法是与体表导联心电图同步的经静脉和(或)经动脉的心腔内电图记录技术与心腔内刺激(起搏)技术心腔)技术相结合。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "临床心电生理检查" }, { "start_idx": 13, "end_idx": 20, "type": "pro", "entity": "临床心电生理检查" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "体表导联心电图" }, { "start_idx": 59, "end_idx": 69, "type": "pro", "entity": "心腔内刺激(起搏)技术" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "心腔" } ] }, { "text": "在心电生理检查前,应当停用各种心律失常药,以免药物改变心电生理特性和妨碍心律失常的诱发。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "心律失常" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "心律失常" } ] }, { "text": "用经皮穿刺插入电极导管的技术,把为记录心腔内电图和心腔刺激(起搏)用的电极导管顶端放置于适当的心腔内部位,并将它们的尾部分别连接于生理记录仪和程序刺激仪。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "心腔内电图" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "心腔刺激" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "心腔" }, { "start_idx": 71, "end_idx": 75, "type": "equ", "entity": "程序刺激仪" } ] }, { "text": "在窦性心律或起搏心律时,引进单个或多个程序的期前刺激是观察动态电生理学的方法。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "窦性心律" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "起搏心律" } ] }, { "text": "正常心脏以可预言的方式对这些干预作出反应。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心脏" } ] }, { "text": "了解并根据这些正常反应,程序刺激便可用来:1.刻划出房室传导系统、心房和心室的电生理特性。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "房室" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "心房" } ] }, { "text": "2.诱发心律失常并分析其发生机制。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "心律失常" } ] }, { "text": "3.评定药物、电刺激、导管消融和外科手术干预对房室传导系统、心房和心室功能的影响以及它们对心律失常的治疗效果。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "电刺激" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "导管消融" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "外科手术" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "房室传导系统" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "心房" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "心室" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "心律失常" } ] }, { "text": "临床心电生理检查时,体表导联至少应有:Ⅰ、Ⅱ、V1或Ⅰ、aVF、V1。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "临床心电生理检查" } ] }, { "text": "心腔内记录导联一般包括希氏束、高位右心房、冠状静脉窦及右心室心尖部。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心腔" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "高位右心房" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "冠状静脉窦" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "右心室心尖部" } ] }, { "text": "心室(心房)标测有激动标测及起搏标测。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心室" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心房" } ] }, { "text": "(六)并发症心脏电生理检查的并发症主要包括三大类:1.与动、静脉穿刺插管有关疼痛、药物不良反应、穿刺部位的感染或脓疡、败血症、出血或血肿、血栓性静脉炎、肺血栓栓塞、动脉撕裂或夹层动脉瘤、全身性血栓栓塞、一过性缺血发作或中风。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "pro", "entity": "心脏电生理检查" }, { "start_idx": 28, "end_idx": 35, "type": "pro", "entity": "动、静脉穿刺插管" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "药物不良反应" }, { "start_idx": 48, "end_idx": 57, "type": "sym", "entity": "穿刺部位的感染或脓疡" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "出血" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "血肿" }, { "start_idx": 76, "end_idx": 80, "type": "dis", "entity": "肺血栓栓塞" }, { "start_idx": 82, "end_idx": 85, "type": "dis", "entity": "动脉撕裂" }, { "start_idx": 93, "end_idx": 99, "type": "dis", "entity": "全身性血栓栓塞" }, { "start_idx": 109, "end_idx": 110, "type": "dis", "entity": "中风" } ] }, { "text": "2.与心腔内导管操纵与程序刺激有关心腔或冠状窦穿孔、心包积血或心包填塞、房颤或室颤、心肌缺血或心肌梗死、束支传导阻滞。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "心腔内导管操纵" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "心包积血" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "房颤" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "室颤" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "心肌缺血" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "心肌梗死" }, { "start_idx": 52, "end_idx": 57, "type": "dis", "entity": "束支传导阻滞" } ] }, { "text": "3.与经导管消融有关完全性房室传导阻滞、瓣膜损伤或新出现的瓣膜关闭不全、急性冠状动脉血栓形成和慢性冠状动脉狭窄、心律失常、心腔、冠状窦或腔静脉穿孔、心包炎、心肌坏死、心力衰竭或心源性休克、肺静脉狭窄、膈神经麻痹等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "经导管消融" }, { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "完全性房室传导阻滞" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "瓣膜损伤" }, { "start_idx": 25, "end_idx": 34, "type": "dis", "entity": "新出现的瓣膜关闭不全" }, { "start_idx": 36, "end_idx": 43, "type": "dis", "entity": "急性冠状动脉血栓" }, { "start_idx": 47, "end_idx": 54, "type": "dis", "entity": "慢性冠状动脉狭窄" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "心律失常" }, { "start_idx": 61, "end_idx": 72, "type": "dis", "entity": "心腔、冠状窦或腔静脉穿孔" }, { "start_idx": 74, "end_idx": 76, "type": "dis", "entity": "心包炎" }, { "start_idx": 78, "end_idx": 81, "type": "dis", "entity": "心肌坏死" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 88, "end_idx": 92, "type": "dis", "entity": "心源性休克" }, { "start_idx": 94, "end_idx": 98, "type": "dis", "entity": "肺静脉狭窄" } ] }, { "text": "第二节特发性弥漫性肺间质纤维化特发性弥漫性肺间质纤维化(idiopathicdiffuseinterstitialfibrosisofthelung)又称Hamman-Rich综合征,是一种弥漫性进行性肺间质纤维化状态,病因尚未明确,可能是各种炎症未控制的结果。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "特发性弥漫性肺间质纤维化" }, { "start_idx": 15, "end_idx": 26, "type": "dis", "entity": "特发性弥漫性肺间质纤维化" }, { "start_idx": 28, "end_idx": 73, "type": "dis", "entity": "idiopathicdiffuseinterstitialfibrosisofthelung" }, { "start_idx": 77, "end_idx": 90, "type": "dis", "entity": "Hamman-Rich综合征" }, { "start_idx": 101, "end_idx": 103, "type": "bod", "entity": "肺间质" } ] }, { "text": "肺泡巨噬细胞可释放纤维化相关的趋化因子和刺激素,如纤维连接蛋白、肺泡巨噬细胞源性生长因子等起到重要作用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "肺泡巨噬细胞" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "趋化因子" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "刺激素" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "纤维连接蛋白" }, { "start_idx": 32, "end_idx": 43, "type": "bod", "entity": "肺泡巨噬细胞源性生长因子" } ] }, { "text": "临床症状以干咳、气短、进行性呼吸困难、发绀为主,一般不发热,可有体重下降、乏力、食欲差。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "干咳" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "气短" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "进行性呼吸困难" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "发绀" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "一般不发热" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "体重下降" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "乏力" }, { "start_idx": 40, "end_idx": 42, "type": "sym", "entity": "食欲差" } ] }, { "text": "合并感染时有发热、咳脓痰、气急等。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "发热" }, { "start_idx": 9, "end_idx": 11, "type": "sym", "entity": "咳脓痰" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "气急" } ] }, { "text": "体格检查见患儿发育极度不良、肺叩诊清音,在肺底部可闻细小捻发音或高调“爆裂”性啰音。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "体格检查" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "发育极度不良" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "肺叩诊清音" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "在肺底部可闻细小捻发音" }, { "start_idx": 32, "end_idx": 40, "type": "sym", "entity": "高调“爆裂”性啰音" } ] }, { "text": "诊断主要根据临床、胸片及肺功能测定。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "ite", "entity": "肺功能测定" } ] }, { "text": "胸部X线变化往往与病理变化一致,显示中下肺野弥漫性网点状阴影,随纤维化加重,出现粗条索状阴影。", "entities": [ { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "纤维化加重" }, { "start_idx": 40, "end_idx": 45, "type": "sym", "entity": "粗条索状阴影" } ] }, { "text": "当肺间质纤维组织收缩时,肺泡及细支气管扩大,形成蜂窝状肺。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "bod", "entity": "肺间质纤维组织" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肺泡" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "支气管" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "肺" } ] }, { "text": "肺门淋巴结不肿大,肺活量减低。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "肺门淋巴结" } ] }, { "text": "肺泡灌洗液中有较多炎症细胞和肥大细胞。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "炎症细胞" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "肥大细胞" } ] }, { "text": "部分患者类风湿因子及抗核抗体可为阳性。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "ite", "entity": "类风湿因子" }, { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "抗核抗体" } ] }, { "text": "以对症治疗为主,吸氧、抗感染、控制心力衰竭等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "对症治疗" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "吸氧" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "抗感染" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "控制心力衰竭" } ] }, { "text": "皮质激素可缓解部分症状,但不能阻止其病情进展和改善肺功能。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "皮质激素" } ] }, { "text": "无效者可试用免疫抑制剂或氯喹。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 12, "end_idx": 13, "type": "dru", "entity": "氯喹" } ] }, { "text": "进行性者多于2年内死于呼吸衰竭及肺心病,慢性者可存活20余年。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "肺心病" } ] }, { "text": "第三节流行性感冒流行性感冒(influenza,简称流感),是由流行性感冒病毒(简称流感病毒)引起的一种常见急性呼吸道传染病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "流行性感冒" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "流行性感冒" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "influenza" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "流感" }, { "start_idx": 32, "end_idx": 38, "type": "mic", "entity": "流行性感冒病毒" }, { "start_idx": 42, "end_idx": 45, "type": "mic", "entity": "流感病毒" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "急性呼吸道传染病" } ] }, { "text": "【病原和流行病学】流感病毒(influenzavirus)属正黏病毒科,基因组为单股正链RNA,其结构包括核衣壳(含NP蛋白)、蛋白壳(含M1蛋白)和包膜。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "流感病毒" }, { "start_idx": 14, "end_idx": 27, "type": "mic", "entity": "influenzavirus" }, { "start_idx": 30, "end_idx": 33, "type": "mic", "entity": "正黏病毒" } ] }, { "text": "包膜来自病毒复制的宿主细胞,带有3种蛋白突起:①血凝素(HA);②神经氨酸酶(NA);③基质蛋白(M2)。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "mic", "entity": "病毒" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "宿主细胞" }, { "start_idx": 24, "end_idx": 26, "type": "mic", "entity": "血凝素" }, { "start_idx": 28, "end_idx": 29, "type": "mic", "entity": "HA" }, { "start_idx": 33, "end_idx": 37, "type": "mic", "entity": "神经氨酸酶" }, { "start_idx": 39, "end_idx": 40, "type": "mic", "entity": "NA" } ] }, { "text": "HA具有亚型和株特异性,能识别靶细胞表面受体,与靶细胞膜融合和诱导保护性中和抗体。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "HA" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "靶细胞" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "靶细胞膜" } ] }, { "text": "NA亦具亚型和株特异性,其功能尚未完全明了,可使病毒从含唾液酸结构中游离出来,通过黏液层结合上皮靶细胞;促进HA被蛋白酶水解;还可破坏宿主细胞的HA受体,协助新生病毒颗粒再吸附于易感细胞,并防止病毒本身发生聚积。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "NA" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "病毒" }, { "start_idx": 28, "end_idx": 30, "type": "mic", "entity": "唾液酸" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "黏液层" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "上皮靶细胞" }, { "start_idx": 54, "end_idx": 55, "type": "mic", "entity": "HA" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "宿主细胞" }, { "start_idx": 72, "end_idx": 73, "type": "mic", "entity": "HA" }, { "start_idx": 81, "end_idx": 82, "type": "mic", "entity": "病毒" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "细胞" }, { "start_idx": 97, "end_idx": 98, "type": "mic", "entity": "病毒" } ] }, { "text": "根据病毒NP和M1蛋白抗原性不同,流感病毒分为甲、乙、丙3个型,根据HA和NA抗原性又分为若干亚型。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "mic", "entity": "病毒NP" }, { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "流感病毒" }, { "start_idx": 34, "end_idx": 35, "type": "mic", "entity": "HA" }, { "start_idx": 37, "end_idx": 38, "type": "mic", "entity": "NA" } ] }, { "text": "流感病毒抗原性变异主要指HA和NA抗原性变异,有两种形式:①抗原性漂移(antigenicdrift):变异幅度小,属量变,往往引起中小型流行;②抗原性转换(antigenicshift):变异幅度大,系质变,形成新亚型。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "流感病毒" }, { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "HA" }, { "start_idx": 15, "end_idx": 16, "type": "mic", "entity": "NA" } ] }, { "text": "分子流行病学研究显示,与流感流行关系最为密切的是HA基因。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "流感" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "HA" } ] }, { "text": "具流行病学意义变异株的HA分子至少有4个以上氨基酸发生替换,并分布在2个以上抗原决定簇区。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "HA" } ] }, { "text": "流感病毒在鸡胚中生长较迅速,但传代易发生抗原性变异。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "流感病毒" } ] }, { "text": "病毒分离可采用原代人胚肾细胞、猴肾细胞和传代犬肾及牛肾细胞等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "人胚肾细胞" } ] }, { "text": "病毒对热、紫外线、乙醚等有机溶剂、甲醛和常用消毒剂均很敏感。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "乙醚" }, { "start_idx": 17, "end_idx": 18, "type": "dru", "entity": "甲醛" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "消毒剂" } ] }, { "text": "患者自潜伏期末即有传染性,持续约1周;隐性感染者带毒时间短。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "隐性感染" } ] }, { "text": "患者呼吸道分泌物中的颗粒可达1000000/ml以上,直径<10μm的飞沫在空气中悬浮时间长,故在人群密集场所感染率高。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "呼吸道分泌物" } ] }, { "text": "分泌物污染环境可间接传播病毒。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "分泌物" }, { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "病毒" } ] }, { "text": "病后或接种后获同型病毒的免疫力,维持时间不超过2年。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "病毒" } ] }, { "text": "我国流感流行存在南北地区差异:长江以南主要在冬、春季,长江以北主要在冬季。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "流感" } ] }, { "text": "【发病机制和病理改变】流感病毒进入上呼吸道后停留于上皮细胞表面的黏液中。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "mic", "entity": "流感病毒" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "黏液" } ] }, { "text": "若过去感染过类似毒株,其呼吸道局部抗体(主要为sIgA)能将病毒清除;若未感染过,病毒则进入细胞内复制,释放大量感染性病毒侵入邻近细胞,在1~2天内引起呼吸道广泛炎症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "感染" }, { "start_idx": 12, "end_idx": 18, "type": "bod", "entity": "呼吸道局部抗体" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "sIgA" }, { "start_idx": 30, "end_idx": 31, "type": "mic", "entity": "病毒" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "感染" }, { "start_idx": 41, "end_idx": 42, "type": "mic", "entity": "病毒" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "细胞" }, { "start_idx": 56, "end_idx": 60, "type": "mic", "entity": "感染性病毒" }, { "start_idx": 76, "end_idx": 82, "type": "dis", "entity": "呼吸道广泛炎症" } ] }, { "text": "在少数抵抗力差者,感染下行造成间质性肺炎。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "感染" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "间质性肺炎" } ] }, { "text": "当呼吸道黏膜被破坏时,部分病毒及其产物如HA、NA等进入血液,引起全身中毒症状。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "呼吸道黏膜" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "病毒" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "HA" }, { "start_idx": 23, "end_idx": 24, "type": "mic", "entity": "NA" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "血液" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "中毒" } ] }, { "text": "流感病毒感染后,近100%的感染者产生局部抗体sIgA,能中和同亚型内不同毒株;约50%产生血清IgA。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "流感病毒感染" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "感染" }, { "start_idx": 19, "end_idx": 26, "type": "bod", "entity": "局部抗体sIgA" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "血清IgA" } ] }, { "text": "特异性IgM和IgA在感染后2周内达峰值;而特异性IgG约在4~8周内达峰值。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "特异性IgM和IgA" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "感染" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "特异性IgG" } ] }, { "text": "抗HA抗体是主要的保护性中和抗体;抗NA抗体不能中和病毒,但能抑制病毒从感染细胞释放。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "抗HA抗体" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "抗NA抗体" }, { "start_idx": 26, "end_idx": 27, "type": "mic", "entity": "病毒" }, { "start_idx": 33, "end_idx": 34, "type": "mic", "entity": "病毒" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "感染细胞" } ] }, { "text": "特异性细胞毒性T细胞(CTL)可直接杀伤感染靶细胞,控制病毒在体内扩散;特异性CTL回忆反应能迅速清除再次感染的病毒而对再次感染有保护作用。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "特异性细胞毒性T细胞" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "CTL" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "感染靶细胞" }, { "start_idx": 28, "end_idx": 29, "type": "mic", "entity": "病毒" }, { "start_idx": 36, "end_idx": 41, "type": "bod", "entity": "特异性CTL" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "感染" }, { "start_idx": 56, "end_idx": 57, "type": "mic", "entity": "病毒" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "感染" } ] }, { "text": "流感时,由于细胞免疫功能受抑制,易继发细菌感染。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "流感" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "细胞" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "细菌感染" } ] }, { "text": "流感所致死亡多见于继发细菌感染或体弱并有其他慢性疾病者。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "流感" }, { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "死亡" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "细菌感染" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "体弱" } ] }, { "text": "呼吸道黏膜早期有单核细胞浸润及水肿,晚期见广泛上皮细胞坏死和出血性渗出物,但基底层细胞正常。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "呼吸道黏膜" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "单核细胞" }, { "start_idx": 5, "end_idx": 16, "type": "sym", "entity": "早期有单核细胞浸润及水肿" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 18, "end_idx": 35, "type": "sym", "entity": "晚期见广泛上皮细胞坏死和出血性渗出物" }, { "start_idx": 38, "end_idx": 44, "type": "sym", "entity": "基底层细胞正常" } ] }, { "text": "肺间质有水肿及炎性细胞浸润,肺泡内可有肺透明膜形成。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肺间质" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "炎性细胞" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "肺间质有水肿及炎性细胞浸润" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肺泡" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "肺透明膜" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "肺泡内可有肺透明膜形成" } ] }, { "text": "(一)典型流感起病急,呼吸道卡他症状轻,而全身中毒症状明显,不同年龄儿童的临床表现各有差异。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "起病急" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "呼吸道" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "呼吸道卡他症状轻" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "中毒" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "全身中毒症状明显" } ] }, { "text": "1.新生儿流感突起高热或体温不升、拒乳、不安、衰弱,类似败血症。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "新生儿流感" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "突起高热" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "体温不升" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "拒乳" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "不安" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "衰弱" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "败血症" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "类似败血症" } ] }, { "text": "但有鼻塞、流涕,提示病毒感染。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "鼻塞" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "流涕" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "病毒感染" } ] }, { "text": "2.幼儿流感可发生上呼吸道感染、喉炎、气管炎、支气管炎、毛细支气管炎和肺炎等症。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "幼儿流感" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "喉炎" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "气管炎" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "支气管炎" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "毛细支气管炎" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "肺炎" } ] }, { "text": "高热时易发生惊厥。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "高热" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "惊厥" } ] }, { "text": "3.学龄儿及青少年流感发病近似成人,急起畏寒高热,体温达39~41℃,面颊潮红,结膜充血,伴全身肌肉酸痛、头痛、乏力、食欲减退等全身症状及鼻塞、流涕、咽痛、干咳等呼吸道症状。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "学龄儿及青少年流感" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "急起畏寒高热" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "体温达39~41℃" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "面颊" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "面颊潮红" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "结膜" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "结膜充血" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "肌肉" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "全身肌肉酸痛" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "头痛" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "乏力" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "食欲减退" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "鼻塞" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "流涕" }, { "start_idx": 75, "end_idx": 76, "type": "sym", "entity": "咽痛" }, { "start_idx": 78, "end_idx": 79, "type": "sym", "entity": "干咳" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "呼吸道" } ] }, { "text": "无并发症者热程一般2~5天,热退后全身症状好转,但呼吸道症状常持续1~2周。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "呼吸道" } ] }, { "text": "丙型流感症状类似于普通感冒或典型流感,儿童少见。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "丙型流感" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "普通感冒" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "典型流感" } ] }, { "text": "(二)轻型流感急性起病,热度不高,呼吸道症状轻,全身症状不明显。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "轻型流感" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "急性起病" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "热度不高" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "呼吸道" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "呼吸道症状轻" } ] }, { "text": "(三)肺炎型流感(流感病毒性肺炎)见于老年、幼儿、体弱多病或正在使用免疫抑制剂者。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肺炎型流感" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "流感病毒性肺炎" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "起病与典型流感相似,1~2日内病情迅速加重,高热持续不退,剧咳带血样痰,烦躁不安,呼吸困难和发绀,可伴心力衰竭和脑病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "典型流感" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "高热持续不退" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "血" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "痰" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "剧咳带血样痰" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "发绀" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "脑病" } ] }, { "text": "两肺密布湿啰音和喘鸣音。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肺" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "两肺密布湿啰音和喘鸣音" } ] }, { "text": "【病原学诊断】(一)病毒分离病毒分离是发现新毒株的唯一方法。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "病毒分离" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "病毒分离" } ] }, { "text": "取发病5天内鼻咽分泌物,同时采用鸡胚羊膜腔接种和细胞培养可提高检出率。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "鼻咽分泌物" }, { "start_idx": 14, "end_idx": 27, "type": "pro", "entity": "采用鸡胚羊膜腔接种和细胞培养" } ] }, { "text": "(二)快速诊断直接检查病毒抗原和病毒核酸的方法有:①病毒抗原检测,用免疫荧光法或免疫酶法检测鼻咽分泌物脱落细胞中病毒抗原;②病毒颗粒检查,用电镜或免疫电镜在症状出现24小时鼻咽分泌物沉渣中直接镜检病毒颗粒;③病毒基因检测,采用核酸杂交法或RT-PCR法检测鼻咽分泌物中病毒特异性基因。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "mic", "entity": "病毒抗原" }, { "start_idx": 16, "end_idx": 19, "type": "mic", "entity": "病毒核酸" }, { "start_idx": 26, "end_idx": 29, "type": "mic", "entity": "病毒抗原" }, { "start_idx": 34, "end_idx": 38, "type": "pro", "entity": "免疫荧光法" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "免疫酶法" }, { "start_idx": 46, "end_idx": 54, "type": "bod", "entity": "鼻咽分泌物脱落细胞" }, { "start_idx": 56, "end_idx": 57, "type": "mic", "entity": "病毒" }, { "start_idx": 62, "end_idx": 67, "type": "pro", "entity": "病毒颗粒检查" }, { "start_idx": 70, "end_idx": 71, "type": "equ", "entity": "电镜" }, { "start_idx": 73, "end_idx": 76, "type": "equ", "entity": "免疫电镜" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "鼻咽分泌物" }, { "start_idx": 96, "end_idx": 97, "type": "pro", "entity": "镜检" }, { "start_idx": 98, "end_idx": 99, "type": "mic", "entity": "病毒" }, { "start_idx": 104, "end_idx": 109, "type": "pro", "entity": "病毒基因检测" }, { "start_idx": 113, "end_idx": 117, "type": "pro", "entity": "核酸杂交法" }, { "start_idx": 119, "end_idx": 125, "type": "pro", "entity": "RT-PCR法" }, { "start_idx": 128, "end_idx": 132, "type": "bod", "entity": "鼻咽分泌物" }, { "start_idx": 134, "end_idx": 135, "type": "mic", "entity": "病毒" } ] }, { "text": "(三)血清学诊断取双份血清(间隔2~4周),采用血凝抑制试验、特异性补体结合试验和中和试验检测相应特异性IgG抗体,滴度≥4倍增高有回顾性诊断意义。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "血清学诊断" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血清" }, { "start_idx": 24, "end_idx": 29, "type": "pro", "entity": "血凝抑制试验" }, { "start_idx": 31, "end_idx": 39, "type": "pro", "entity": "特异性补体结合试验" }, { "start_idx": 41, "end_idx": 56, "type": "pro", "entity": "中和试验检测相应特异性IgG抗体" } ] }, { "text": "用ELISA法检测特异性IgM和IgA可诊断之。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "ELISA法" }, { "start_idx": 9, "end_idx": 18, "type": "bod", "entity": "特异性IgM和IgA" } ] }, { "text": "【预防和治疗】(一)药物预防1.金刚烷胺(amantadine)主要用于甲型流感的预防。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "金刚烷胺" }, { "start_idx": 21, "end_idx": 30, "type": "dru", "entity": "amantadine" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "甲型流感" } ] }, { "text": "2.金刚乙胺(rimantadine)用于甲型流感的预防。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "金刚乙胺" }, { "start_idx": 7, "end_idx": 17, "type": "dru", "entity": "rimantadine" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "甲型流感" } ] }, { "text": "吸入给药已成功用于成人的预防,尚未见儿童群体预防用药的报道。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "吸入给药" } ] }, { "text": "(二)疫苗接种由于流感病毒抗原易变异,WHO流感协作中心每年定期发布次年流感疫苗抗原成分的建议。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "疫苗接种" }, { "start_idx": 9, "end_idx": 14, "type": "mic", "entity": "流感病毒抗原" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "WHO流感" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "流感疫苗" } ] }, { "text": "1.灭活疫苗目前多采用多价纯化的灭活疫苗或裂解的亚单位疫苗(保留HA和NA,去除核酸),接种者不良反应减少,但免疫原性不如纯化的全毒株疫苗。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "灭活疫苗" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "灭活疫苗" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "亚单位疫苗" }, { "start_idx": 32, "end_idx": 33, "type": "mic", "entity": "HA" }, { "start_idx": 35, "end_idx": 36, "type": "mic", "entity": "NA" }, { "start_idx": 61, "end_idx": 68, "type": "dru", "entity": "纯化的全毒株疫苗" } ] }, { "text": "将佐剂与亚单位疫苗一起应用,可提高疫苗的效果。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "亚单位疫苗" }, { "start_idx": 17, "end_idx": 18, "type": "dru", "entity": "疫苗" } ] }, { "text": "2.减毒活疫苗鼻内给药,使病毒只在上呼吸道增殖,刺激产生局部和体液免疫,已用于成人和儿童,显示良好的免疫原性。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "减毒活疫苗" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "鼻内给药" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "病毒" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "体液" } ] }, { "text": "(三)综合对症治疗卧床休息,多饮水,加强护理,预防并发症。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "治疗卧床休息" }, { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "多饮水" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "加强护理" } ] }, { "text": "剧咳者给予镇咳祛痰剂。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "剧咳" }, { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "镇咳祛痰剂" } ] }, { "text": "继发细菌感染时给予相应抗生素(一般不必预防性用药)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "细菌感染" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "抗生素" } ] }, { "text": "(四)抗病毒治疗1.金刚烷胺用于治疗无并发症的甲型流感。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "抗病毒治疗" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "金刚烷胺" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "甲型流感" } ] }, { "text": "2.扎那米韦用于治疗甲型和乙型流感。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "扎那米韦" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "甲型和乙型流感" } ] }, { "text": "儿童用量:吸入10mg,一日2次,疗程10天,病程早期(36小时内)开始用药。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "吸入" } ] }, { "text": "3.Oseltavirir为NA抑制剂。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dru", "entity": "Oseltavirir" }, { "start_idx": 14, "end_idx": 18, "type": "dru", "entity": "NA抑制剂" } ] }, { "text": "二、小儿神经系统的解剖生理特点(一)脑和脊髓的发育特点神经系统的发育在胎儿期最早开始。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "神经系统" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "脑" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "脊髓" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "神经系统" } ] }, { "text": "在婴儿期,甚至整个小儿时期,神经精神发育一直十分活跃。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "神经" } ] }, { "text": "出生时小儿脑重平均370g,占体重的10%~12%,为成人脑重(约1500g)的25%左右。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "小儿脑重" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "体重" }, { "start_idx": 27, "end_idx": 30, "type": "ite", "entity": "成人脑重" } ] }, { "text": "6个月婴儿脑重600~700g,1岁时达900g,2岁时达1000g左右,4~6岁时脑重已达成人脑重的85%~90%。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "脑重" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "脑重" }, { "start_idx": 46, "end_idx": 49, "type": "ite", "entity": "成人脑重" } ] }, { "text": "出生时大脑已有主要的沟回,但皮层较薄、沟裂较浅。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "大脑" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "沟回" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "皮层较薄" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "沟裂较浅" } ] }, { "text": "新生儿神经细胞数目与成人相同,但其树突与轴突少而短脑重的增加主要由于神经细胞体积增大和树突的增多、加长,以及神经髓鞘的形成和发育;3岁时神经细胞分化已基本完成,8岁时接近成人。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经细胞" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "轴突" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "树突与轴突少而短" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "脑重" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "神经髓鞘" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "神经细胞" } ] }, { "text": "神经纤维的发育较晚,始于胚胎7个月,到4岁时完成髓鞘化。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "神经纤维" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "髓鞘化" } ] }, { "text": "故婴儿期各种刺激引起的神经冲动传导缓慢,且易于泛化,不易形成兴奋灶,易于疲劳。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "易于泛化" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "不易形成兴奋灶" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "易于疲劳" } ] }, { "text": "出生时大脑皮质下中枢如丘脑、下丘脑以及苍白球等发育已较成熟,初生婴儿的活动主要由皮质下系统调节。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "bod", "entity": "大脑皮质下中枢" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "丘脑" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "下丘脑" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "苍白球" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "皮质" } ] }, { "text": "随着大脑皮层的发育成熟,运动逐渐转为由大脑皮层中枢调节,对皮质下中枢的抑制作用也趋明显。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "大脑皮层" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "大脑皮层中枢" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "皮质下中枢" } ] }, { "text": "足月新生儿出生时脊髓重2~6g,脊髓功能相对成熟。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "脊髓" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "脊髓" } ] }, { "text": "脊髓下端在胎儿时位于第2腰椎下缘,4岁时上移至第1腰椎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "脊髓下端" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "第2腰椎下缘" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "第1腰椎" } ] }, { "text": "作腰椎穿刺时应注意,婴幼儿脊髓下端位置较低。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "脊髓下端" } ] }, { "text": "脊髓的髓鞘由上而下逐渐形成,约于3岁时完成髓鞘化。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脊髓" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "髓鞘" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "髓鞘化" } ] }, { "text": "(二)脑脊液的正常值小儿时期脑脊液的正常值为:压力0.69~1.96(新生儿0.29~0.78)kPa,外观清亮透明,潘氏试验阴性,白细胞数0~5(新生儿或小婴儿0~20)×106/L,蛋白0.2~0.4(新生儿0.2~1.2)g/L,糖2.2~4.4mmol/L。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "脑脊液" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "脑脊液" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "外观清亮透明" }, { "start_idx": 59, "end_idx": 64, "type": "sym", "entity": "潘氏试验阴性" }, { "start_idx": 66, "end_idx": 69, "type": "ite", "entity": "白细胞数" }, { "start_idx": 99, "end_idx": 100, "type": "ite", "entity": "蛋白" }, { "start_idx": 124, "end_idx": 124, "type": "ite", "entity": "糖" } ] }, { "text": "(三)神经反射的发育特点正常足月儿出生时即具有觅食、吸吮、吞咽、拥抱及握持等一些先天性(原始)反射和对强光、寒冷及疼痛等的反应。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "神经" } ] }, { "text": "3~4个月前小儿肌张力较高,Kernig征可为阳性,2岁以下小儿Babinski征阳性(对称)亦可为生理现象。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "肌张力较高" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "Kernig征可为阳性" }, { "start_idx": 32, "end_idx": 46, "type": "sym", "entity": "Babinski征阳性(对称)" } ] }, { "text": "第十节缓慢性心律失常窦性静止与窦房传导阻滞在心电图上可表现为长间隙。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "缓慢性心律失常" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "窦性静止" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "窦房传导阻滞" }, { "start_idx": 22, "end_idx": 24, "type": "pro", "entity": "心电图" } ] }, { "text": "前者一般认为是因为激动在窦房结内形成异常在窦房传导时发生阻滞心律失常在儿科少见,可见于洋地黄中毒中毒或心房广泛手术后。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "窦房结内" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "在窦房结内形成异常" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "窦房" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "在窦房传导时发生阻滞" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "心律失常" }, { "start_idx": 35, "end_idx": 36, "type": "dep", "entity": "儿科" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "洋地黄中毒" }, { "start_idx": 51, "end_idx": 56, "type": "pro", "entity": "心房广泛手术" } ] }, { "text": "房室传导阻滞(atrialcapture)可分为三种类型,即一度房室传导阻滞、二度房室传导阻滞及三度房室传导阻滞。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "房室传导阻滞" }, { "start_idx": 7, "end_idx": 19, "type": "dis", "entity": "atrialcapture" }, { "start_idx": 30, "end_idx": 37, "type": "dis", "entity": "一度房室传导阻滞" }, { "start_idx": 39, "end_idx": 46, "type": "dis", "entity": "二度房室传导阻滞" }, { "start_idx": 48, "end_idx": 55, "type": "dis", "entity": "三度房室传导阻滞" } ] }, { "text": "一度房室传导阻滞:PR间期延长,但所有心房激动均能传到心室。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "一度房室传导阻滞" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心房" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "心室" } ] }, { "text": "在莫氏Ⅱ型,由于房室传导组织有效不应期延长,使心房搏动部分不能下传至心室,发生间歇性心室脱落心室脱落前后,下传的P波,其PR间期是恒定的。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "房室" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心房" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "心室" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "心室" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "间歇性心室脱落" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "心室" } ] }, { "text": "三度房室传导阻滞(完全性房室传导阻滞):心房激动完全不能到达心室。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "三度房室传导阻滞" }, { "start_idx": 9, "end_idx": 17, "type": "dis", "entity": "完全性房室传导阻滞" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "心房" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "心室" } ] }, { "text": "先天性完全性房室传导阻滞的发病率一般认为1/2万~1/2.5万活产婴儿,常为自身免疫引起。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dis", "entity": "先天性完全性房室传导阻滞" } ] }, { "text": "患儿的母亲常患有系统性红斑狼疮(可无症状),其体内的IgG抗体(anti-SSA/Ro、anti-SSB/La)可通过胎盘损害胎儿的传导组织。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "IgG抗体" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "胎盘" }, { "start_idx": 66, "end_idx": 69, "type": "bod", "entity": "传导组织" } ] }, { "text": "类风湿性关节炎、皮肌炎、或Sjögren综合征偶也可引起胎儿传导组织的自身免疫性损伤。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "类风湿性关节炎" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "Sjögren综合征" }, { "start_idx": 28, "end_idx": 33, "type": "bod", "entity": "胎儿传导组织" } ] }, { "text": "自身免疫原因占先天性完全性房室传导阻滞发生率的60%~70%,心脏结构正常患儿的80%。", "entities": [ { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "心脏" } ] }, { "text": "有些系统性红斑狼疮母亲的小儿出生时并无传导阻滞,生后3~6个月才发生。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "系统性红斑狼疮" } ] }, { "text": "其他原因包括先天性房室结缺如或房室结纤维化等。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "房室结纤维化" } ] }, { "text": "新生儿或婴幼儿可出现心力衰竭或心源性脑缺氧综合征。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "心源性脑缺氧综合征" } ] }, { "text": "年长儿往往因心率轻度减慢而无明显症状。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "心率" } ] }, { "text": "心电图除显示PP间期与RR间期各有其固定规律外,QRS间期及形态多正常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "心电图" } ] }, { "text": "后天获得性完全性房室传导阻滞的病因有:心肌炎、心脏肿瘤、心内膜炎所致的心肌脓疡、药物或电解质紊乱等,也可发生在心脏手术后。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "dis", "entity": "后天获得性完全性房室传导阻滞" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "心肌炎" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "心脏肿瘤" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "心肌脓疡" }, { "start_idx": 40, "end_idx": 47, "type": "sym", "entity": "药物或电解质紊乱" }, { "start_idx": 55, "end_idx": 58, "type": "pro", "entity": "心脏手术" } ] }, { "text": "阻滞部位可在房室结、房室束或其分支以下。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "房室结" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "房室束" } ] }, { "text": "心电图显示QRS波多宽大畸形,时间>0.1秒,且心室率较慢,多在40次以下,因而症状较明显,轻者可诉疲乏、无力、眩晕,重者可发生心力衰竭或急性心脑缺氧综合征。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "心电图" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "畸形" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "心室率" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "疲乏" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "无力" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "眩晕" }, { "start_idx": 64, "end_idx": 67, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 69, "end_idx": 77, "type": "dis", "entity": "急性心脑缺氧综合征" } ] }, { "text": "无症状且心率>55次/分者不需给予治疗,也不应限制患儿的活动。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "心率" } ] }, { "text": "心率较慢而无晕厥或心力衰竭者可口服阿托品、麻黄碱。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "心率" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "晕厥" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "口服阿托品" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "麻黄碱" } ] }, { "text": "危重患者可静滴异丙肾上腺素。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "静滴异丙肾上腺素" } ] }, { "text": "安置人工起搏器的指征为:①有心力衰竭或心源性脑缺氧综合征者;②心室率显著缓慢(新生儿<55次/分,婴儿<50次/分,儿童<40次/分);③有频发室早或室速者;④心脏手术后发生三度房室传导阻滞,观察2~4周未能恢复者(ACC/AHA指征为术后高二度或三度房室传导阻滞无缓解趋势或持续至少术后7天者)。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "安置人工起搏器" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 19, "end_idx": 27, "type": "dis", "entity": "心源性脑缺氧综合征" }, { "start_idx": 31, "end_idx": 33, "type": "ite", "entity": "心室率" }, { "start_idx": 80, "end_idx": 83, "type": "pro", "entity": "心脏手术" }, { "start_idx": 87, "end_idx": 94, "type": "dis", "entity": "三度房室传导阻滞" }, { "start_idx": 121, "end_idx": 131, "type": "dis", "entity": "二度或三度房室传导阻滞" } ] }, { "text": "参考文献1.V.E.Brown,C.A.Pilkington1,B.M.Feldman,etal.Aninternationalconsensussurveyofthediagnosticcriteriaforjuveniledermatomyositis(JDM).Rheumatology(Oxford),2006,45(8):990-9902.P.Riley,L.J.McCann,S.M.Maillard,etal.Effectivenessofinfliximabinthetreatmentofrefractoryjuveniledermatomyositiswithcalcinosis.Rheumatology(Oxford)2008,47(6):877-8803.蒋明等.风湿病学.北京:科学出版社,19984.BehrmanRE,KliegmanRM,JensonHB.NelsonTextbookofPediatrics.16thed.Philadelphia:W.B.SaunersCo,2000", "entities": [ { "start_idx": 129, "end_idx": 131, "type": "dis", "entity": "JDM" }, { "start_idx": 345, "end_idx": 347, "type": "dis", "entity": "风湿病" } ] }, { "text": "参考文献1.中华医学会风湿病学分会.风湿热诊治指南草案.中华风湿病学杂志,2004,8(3):504-5042.蒋明等.风湿病学.北京:科学出版社,19983.BehrmanRE,KliegmanRM,JensonHB.NelsonTextbookofPediatrics.16thed.Philadelphia:W.B.SaunersCo,20004.杨锡强等.儿童免疫学.北京:人民卫生出版社,2001", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "风湿病学" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "风湿热" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "风湿病" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "风湿病" } ] }, { "text": "参考文献1.BarkinRM.EmergencyPediatrics-aguidetoambulatorycare.5thed.Mosby.Louis,Missouri,1999:314-3142.BehrmanRE.TextbookofPediatrics.16thed.WBSaundersCompany,Philadelphia,USA,2000:287-2873.BarkinRM.PediatricEmergencyMedicine-conceptsandclinicalpractice.2nded.MosbyYear-bookIncStLouisMissouri,1997:489-4954.AshcraftKW.PediatricSurgery.3rded.WBSaundersCompany.Philadelphia,USA,2000:159-1595.胡亚美,江载芳.诸福棠实用儿科学.第7版.北京:人民卫生出版,2002:2479-24796.张金哲.实用小儿外科学.杭州:浙江科学技术出版社,2003:52-52", "entities": [ { "start_idx": 421, "end_idx": 422, "type": "dep", "entity": "儿科" }, { "start_idx": 463, "end_idx": 464, "type": "dep", "entity": "外科" } ] }, { "text": "四、毒蛇咬伤【病因】儿童野外玩耍可被毒蛇咬伤,蛇毒的成分复杂,但主要为神经毒素和血毒素两大类,神经毒素主要作用于神经系统,引起呼吸肌麻痹、肌肉麻痹等症状;血毒素主要引起心力衰竭、溶血、出血、凝血等。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "咬伤" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "咬伤" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "神经系统" }, { "start_idx": 63, "end_idx": 67, "type": "sym", "entity": "呼吸肌麻痹" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "肌肉麻痹" }, { "start_idx": 84, "end_idx": 87, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 89, "end_idx": 90, "type": "sym", "entity": "溶血" }, { "start_idx": 92, "end_idx": 93, "type": "sym", "entity": "出血" }, { "start_idx": 95, "end_idx": 96, "type": "sym", "entity": "凝血" } ] }, { "text": "【临床表现】血毒素主要表现为咬伤局部肿胀剧痛,可出现水疱、血泡、组织坏死、伤口流血不止,并迅即向近心端发展,全身出血或发生溶血、贫血、黄疸、血红蛋白尿及少尿、无尿,心音低钝,心律不齐,血压下降,呼吸急促、休克以致死亡。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "咬伤" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "局部肿胀剧痛" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "水疱" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "血泡" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "组织坏死" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "伤口流血不止" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "心端" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "全身出血" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "溶血" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "贫血" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "黄疸" }, { "start_idx": 70, "end_idx": 74, "type": "sym", "entity": "血红蛋白尿" }, { "start_idx": 76, "end_idx": 77, "type": "sym", "entity": "少尿" }, { "start_idx": 79, "end_idx": 80, "type": "sym", "entity": "无尿" }, { "start_idx": 82, "end_idx": 83, "type": "ite", "entity": "心音" }, { "start_idx": 82, "end_idx": 85, "type": "sym", "entity": "心音低钝" }, { "start_idx": 87, "end_idx": 88, "type": "ite", "entity": "心律" }, { "start_idx": 87, "end_idx": 90, "type": "sym", "entity": "心律不齐" }, { "start_idx": 92, "end_idx": 93, "type": "ite", "entity": "血压" }, { "start_idx": 92, "end_idx": 95, "type": "sym", "entity": "血压下降" }, { "start_idx": 97, "end_idx": 100, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 102, "end_idx": 103, "type": "sym", "entity": "休克" }, { "start_idx": 106, "end_idx": 107, "type": "sym", "entity": "死亡" } ] }, { "text": "【治疗】(一)现场急救咬伤后立即在伤口近端2~3cm处扎肢体,阻断静脉血液和淋巴液回流,减少吸收。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "咬伤" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "血液" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "淋巴液" } ] }, { "text": "紧扎处应每隔10分钟左右放松1~2分钟,以使被扎部分组织不会因血流循环受阻而坏死,并限制患肢活动。", "entities": [ { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "血流循环" } ] }, { "text": "(二)局部处理伤口局部用水冲洗后,并用5%依地酸二钠或1∶5000高锰酸钾水冲洗至流出的血水变为鲜红色为止。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "dru", "entity": "依地酸二钠" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "高锰酸钾水" } ] }, { "text": "局部封闭疗法是在伤口周围或肿胀上方3~4cm处皮下注射0.25%~0.5%普鲁卡因加地塞米松5mg,或用胰蛋白酶4000U溶于25%普鲁卡因5~20ml,以牙痕为中心,在伤口周围作皮下环封。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "局部封闭疗法" }, { "start_idx": 37, "end_idx": 40, "type": "dru", "entity": "普鲁卡因" }, { "start_idx": 42, "end_idx": 45, "type": "dru", "entity": "地塞米松" }, { "start_idx": 52, "end_idx": 55, "type": "dru", "entity": "胰蛋白酶" }, { "start_idx": 66, "end_idx": 69, "type": "dru", "entity": "普鲁卡因" }, { "start_idx": 90, "end_idx": 93, "type": "pro", "entity": "皮下环封" } ] }, { "text": "(三)全身处理尽快应用蛇药口服(如南通蛇药片、群生蛇药)或注射抗毒血清,注射前应先做皮试,阴性时作静脉注射,阳性者可做脱敏疗法,如无蛇药,可选用中草药解毒。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dru", "entity": "蛇药" }, { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "南通蛇药片" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "群生蛇药" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "注射抗毒血清" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "皮试" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "静脉注射" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "脱敏疗法" }, { "start_idx": 72, "end_idx": 74, "type": "dru", "entity": "中草药" } ] }, { "text": "常用的中草药如半枝莲、蛇果草、穿心莲、地丁草、蒲公英、马齿苋、拉拉藤、河白草、瓜子金、苦参、青木香、紫花地丁、凤尾草等。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "半枝莲" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "蛇果草" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "穿心莲" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "地丁草" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "蒲公英" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "马齿苋" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "拉拉藤" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "河白草" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "瓜子金" }, { "start_idx": 43, "end_idx": 44, "type": "dru", "entity": "苦参" }, { "start_idx": 46, "end_idx": 48, "type": "dru", "entity": "青木香" }, { "start_idx": 50, "end_idx": 53, "type": "dru", "entity": "紫花地丁" }, { "start_idx": 55, "end_idx": 57, "type": "dru", "entity": "凤尾草" } ] }, { "text": "参考文献1.BarrattTM,AvnerED,HarmonWE.PediatricNephrology,4thedition.Baltimore,LippincottWilliams&Wilkins.1999:565-5822.LashJP,ArrudaJA.Laboratoryevaluationofrenaltubularacidosis.ClinLabMed,1993,13(1):117-1173.FortenberryJD,KenneyRD.Distalrenaltubularacidosisastheinitialmanifestationofsystemiclupuserythematosusinanadolescent.JAdolescHealth,1991,12(2):148-1484.BolinaFM,VillegasMO,VehaskariVM.Renalacidificationinchildrenwithidiopathichypercalciuria.JPediatr,1994,124(4):529-5295.BrownMT.Progressivesebsorineuralhearinglossinassociationwithdistaltubularrenalacidosis.ArchOtolaryngolHeadNeckSurg,1993,119(4):458-4606.BaggaA.Renaltubularacidosisprecedingsystemiclupuserythematosus.PediatrNephrol,1993,7(6):735-7357.ChaftL,CaultMH.FirstmorningurinepHinthediagnosisofrenaltubularacidosiswithnephrolithiasis.ClinNephrol,1994,11:1598.RothKS,ChanJCM.Renaltubularacidosis:Anewlookatanoldproblem.ClinNephrol,1994,41(3):159-1599.FormanJW,RothKS.ThehumanrenalFanconisyndrome—thenandnow.Nephron,1989,51(3):301-30110.SantosF,ChanJCM.Renaltubularacidosisinchildren:diagnosis,treatmentandprognosis.AmJNephrol,1986,6(4):289-28911.Rodriguez-SorianoJ.Newinsightsintothepathogenesisofrenaltubularacidosis-fromfunctionaltomolecularstudies.PediatrNephrol,2000,14(12):1121-112112.Rodriguez-SorianoJ,ValloA.Renaltubularacidosis.PediatrNephrol,1990,4(3):268-26813.ForemanJW,RothKS.ThehumanrenalFanconisyndromethenandnow.Nephrology,1989,51(3):301-30114.RossiR,EhrichJH.PartialandcompletedeToni-Debre-Fanconisyndromeafterifosfamidechemotherapyofchildrenmalignancy.EurJClinPharmacol,1993,44(Suppl1):S43-4515.TolaymatA,SakaracanA,NeibergerR.IdiopathicFanconisyndromeinafamily.PartI.Clinicalaspects.JAmSocNephrol,1992,2(8):1310-131016.BrewerED.TheFanconisyndrome:ClinicalDisorders.In:GonickHC,BuckalenVM.RenalTubularDisorder.1sted.NY,MarcelDekker,1985:475-47517.ForemanJW.CystinosisandFanconisyndrome.In:BarrattTM,AvnerED,HarmanWE,eds.PediatricNephrology,4thedition.1998:593-60818.Rodriguez-SorianoJ.Tubulardisordersofelectrolyteregulation.In:BarrattTM,AvnerED,armanWE,eds.PediatricNephrology,4thedition.1998:545-54519.TownM,JeanG,CherquiS,etal.Anovelgeneencodinganintegralmembraneproteinismutatedinnephropathiccystinosis.NatGenet,1998,18(4):319-31920.AttardM,JeanG,ForestierL,etal.SeverityofphenotypeincystinosisvarieswithmutationsintheCTNSgene:predictedeffectonthemodelofcystinosin.HumMolGenet,1999,8(13):2507-250721.OnoJ,HaradaK,ManoT,etal.MRfindingsandneurologicmanifestationsinloweoculocerebrorenalsyndrome.PediatrNeurol,1996,14(2):162-16222.LinT,OrrisonBM,LeaheyAM,etal.SpectrumofmutationsintheOCRL1geneintheLoweoculocerebrorenalsyndrome.AmJHumGenet,1997,60(6):1384-138423.AlconcherLFCastroC,QuintanaD,eta1.Urinarycalciumexcretioninhealthyschoolchildren.PediatrNephrol,1997,11(2):186-18624.PerroneHC,ToporovskiJ,ScherN.UrinaryinhibitorsOfcrystallizationinhypercalciuricchildrenwithhematuriaandnephrolithiasis.PediatrNephrol,1996,10(4):435-43725.NietopnFN,FerrandezC,MongeM,etal.Bonemineraldensityinpediatricpatientswithidiopathichypercalciuria.PediatrNephrol,1997,11(5):578-57826.SantosF,SuarezD,MalagaS,etal.Idiopathichypercalciuriainchildren.pathophysiologicconsiderationsofrenalandabsorptivesubtypes.JPediatr,1987,110(2):238-23827.HeiliczerJD,CanonigoBB,BishofNA,etal.Noncalculiurinarytractdisorderssecondarytoidiopathichypercalciuriainchildren.PedClinNorAm,1987,34(3):711-71128.PronickaE,RowinskaE,KulczyckaH,etal.Persistenthypercaciuriaandelevated25-hydroxyvitaminD3inchildrenwithinfantilehypercalciuria.PediatrNephrol,1997,11(1):2-629.LamaG,StileV,RageneG,etal.Increasedserumcalcitonin,reducedserum1,25(OH)2-vitaminDandnormalparathormoneconcentrationsinidiopathichypercalciuria.ChildNephrolUrol,1988,8(3):99-9930.HymesLC,WarshawBL.Idiopathichypercalduria:renalandabsorpivesubtypesinchildren.AmJDisChild,1984(2),138:176-17631.TiederM,StarkH,Shainkin-kerstenbaumR.Pathophysiologicstudiesinidiopathichypercalciuriapresentinginchildhood.IntJPediatrNephrol,1983,4(3):197-19732.InsognaKL,BroadusAE,DreyerBE,etal.Elevetedproductionrateofl,25(OH)-dihydroxyvitaminDinpatientswithabsorptivehypercalciuria.JClinEndocrinolMetab,1985,61(3):490-49533.HollidayMA,BarrattTM,AvrnerED.PediatricNephrology.3ndedition.Baltirnore:Williams&Wilkins,1994:890-89034.MassrySG,GlassockRJ.TextbookofNephrology3nd.Baltimore:Williams&Wilkins,1995:1036-103635.杜学海.肾间质性损伤在肾脏疾病中的意义.中华肾脏病杂志,1995,11(4):242-24236.CameronS,DavisonAM,GruanfeldJ,KerrD,etal.OxfordTextbookofClinicalNephrology.Oxford:OxforduniversityPress,1992:1091-1091,1203-120337.RoxeDM,KrumlovskyFA.Toxicinterstitialnephropathyfrommetals,metabolites,andradiation.SeminNephrol,1998,8(1):72-8138.MullerGA,ZeisbergM,StrutzF.Theimportanceoftubulointerstitialdamageinprogressiverenaldisease.NephrolDialTransplant,2000,15(Suppl6):76-7639.WuthrichRP,SibalicV.Autoimmunetubulointerstitialnephritis:insightfromexperimentalmodels.ExpNephrol,1998,6(4):288-288", "entities": [ { "start_idx": 4324, "end_idx": 4329, "type": "dis", "entity": "肾间质性损伤" }, { "start_idx": 4331, "end_idx": 4332, "type": "dis", "entity": "肾脏" }, { "start_idx": 4342, "end_idx": 4344, "type": "dis", "entity": "肾脏病" } ] }, { "text": "附:维生素A过多症维生素A过多症(hypervitaminosisA),即维生素A中毒,根据发病情况,可分为急性中毒及慢性中毒两种。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "维生素A过多症" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "维生素A过多症" }, { "start_idx": 17, "end_idx": 33, "type": "dis", "entity": "hypervitaminosisA" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "维生素A中毒" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "急性中毒" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "慢性中毒" } ] }, { "text": "当血清维生素A浓度超过5.1μmolL(1500UL)时出现中毒症状。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "ite", "entity": "血清维生素A浓度" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "中毒" } ] }, { "text": "【急性中毒】比较多见。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "急性中毒" } ] }, { "text": "由于短期内大量摄入维生素A(剂量≥20万U)所致。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "维生素A" } ] }, { "text": "【慢性中毒】由于长期较大剂量摄入维生素A所致,发生中毒所需的累积量及时间因人而异,一般平均摄入量大于1.3万μgd(5万Ud),常发生在因慢性皮肤疾病服药的患儿。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "慢性中毒" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "维生素A" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "中毒" }, { "start_idx": 69, "end_idx": 74, "type": "dis", "entity": "慢性皮肤疾病" } ] }, { "text": "一般摄入数周或数月后出现症状,主要表现为慢性症状,如食欲减退、体重不增、激怒、脂溢性皮炎、皮肤瘙痒、脱发、口唇皲裂、肝脾大和肝功能损害。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "食欲减退" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "体重不增" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "激怒" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "脂溢性皮炎" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "皮肤" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "皮肤瘙痒" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "脱发" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "口唇皲裂" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "肝脾" }, { "start_idx": 58, "end_idx": 60, "type": "sym", "entity": "肝脾大" }, { "start_idx": 62, "end_idx": 62, "type": "bod", "entity": "肝" }, { "start_idx": 62, "end_idx": 66, "type": "sym", "entity": "肝功能损害" } ] }, { "text": "骨骼症状明显,有骨痛,尤以长骨为主,可有转移性。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "骨骼" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "骨痛" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "长骨" } ] }, { "text": "软组织肿胀、压痛而无红热,常误为脓肿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "软组织" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "软组织肿胀" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "压痛而无红热" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "脓肿" } ] }, { "text": "骨骼X线显示骨皮质肥厚、骨膜下积液和骨膜分离,尤以长骨中段更为明显。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "骨骼X线" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "骨皮质肥厚" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "骨膜" }, { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "骨膜下积液和骨膜分离" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "长骨中段" } ] }, { "text": "【处理】一旦发现维生素A过多,应立即停服维生素A制剂及对症处理。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "维生素A" }, { "start_idx": 20, "end_idx": 25, "type": "dru", "entity": "维生素A制剂" } ] }, { "text": "急性中毒者,待停服维生素A1~2天后症状缓解;慢性中毒者,待停服维生素A,1~2周后症状减轻或消失,但骨骼X线表现需要6个月左右恢复正常。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "急性中毒" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "维生素A" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "慢性中毒" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "维生素A" }, { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "骨骼X线" } ] }, { "text": "五、戊型肝炎【病原和流行病学】戊型肝炎病毒(hepatitisEvirus,HEV)属嵌杯病毒科,无包膜,核酸为单股正链RNA。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "戊型肝炎" }, { "start_idx": 15, "end_idx": 20, "type": "mic", "entity": "戊型肝炎病毒" }, { "start_idx": 22, "end_idx": 36, "type": "mic", "entity": "hepatitisEvirus" }, { "start_idx": 38, "end_idx": 40, "type": "mic", "entity": "HEV" }, { "start_idx": 43, "end_idx": 46, "type": "mic", "entity": "嵌杯病毒" } ] }, { "text": "病毒在体外不稳定,对高盐、氯化铯、氯仿等敏感。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" } ] }, { "text": "细胞培养尚未建立,多种非人灵长类动物可感染HEV。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "HEV" } ] }, { "text": "患者于潜伏期末至急性早期从粪便中排出大量病毒,是主要传染源。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "粪便" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "病毒" } ] }, { "text": "病毒经粪-口途径和接触传播。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "粪" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "口" } ] }, { "text": "我国各地区均有戊型肝炎(viralhepatitisE)发生,吉林、辽宁、河北、山东、内蒙古、新疆和北京曾有暴发流行,流行类型为食物型或水源型。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "戊型肝炎" }, { "start_idx": 12, "end_idx": 26, "type": "dis", "entity": "viralhepatitisE" } ] }, { "text": "【发病机制和病理改变】HEV主要侵犯肝脏,通过直接致病作用和(或)免疫性损伤引起肝细胞炎症和坏死。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "mic", "entity": "HEV" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肝脏" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "免疫性损伤" }, { "start_idx": 40, "end_idx": 47, "type": "dis", "entity": "肝细胞炎症和坏死" } ] }, { "text": "肝脏病理改变有肝细胞变性,灶性坏死,汇管区淋巴细胞、单核巨噬细胞和NK细胞浸润。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肝脏" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肝细胞" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "肝细胞变性" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "灶性坏死" }, { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "汇管区淋巴细胞" }, { "start_idx": 26, "end_idx": 31, "type": "bod", "entity": "单核巨噬细胞" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "NK细胞" }, { "start_idx": 18, "end_idx": 38, "type": "sym", "entity": "汇管区淋巴细胞、单核巨噬细胞和NK细胞浸润" } ] }, { "text": "急性黄疸型患者半数以上可见淤胆和胆栓形成。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "急性黄疸型" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "淤胆" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "胆栓" } ] }, { "text": "临床表现与甲肝类似,不发展成慢性。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "甲肝" } ] }, { "text": "儿童多为亚临床型感染。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "亚临床型感染" } ] }, { "text": "常见临床类型有:(一)急性黄疸型占显性感染的86.5%。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "黄疸" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "急性黄疸" } ] }, { "text": "临床三期经历同甲肝,前驱期症状可持续到黄疸出现后第4~5天。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "甲肝" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "黄疸" } ] }, { "text": "淤胆较为常见。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "淤胆" } ] }, { "text": "(二)急性无黄疸型表现与甲肝类似。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "黄疸" }, { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "急性无黄疸" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "甲肝" } ] }, { "text": "(三)淤胆型较为常见,病程可长达2个月以上。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "淤胆" } ] }, { "text": "高危因素包括:①妊娠妇女;②年老体弱者;③合并HBV感染。", "entities": [ { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "HBV感染" } ] }, { "text": "(五)与其他病毒混合感染1.HEV与HAV同时或先后感染者并不加重病情。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "病毒混合感染" }, { "start_idx": 14, "end_idx": 27, "type": "dis", "entity": "HEV与HAV同时或先后感染" } ] }, { "text": "2.HEV与HBV重叠感染者患者HBV常有活动性复制,HEV不易被清除。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "HEV与HBV重叠感染" }, { "start_idx": 16, "end_idx": 18, "type": "mic", "entity": "HBV" }, { "start_idx": 27, "end_idx": 29, "type": "mic", "entity": "HEV" } ] }, { "text": "病情易迁延或反复发作。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "病情易迁延或反复发作" } ] }, { "text": "病情重,发生重型者多,印度报告80.7%的急性重型和75.5%的亚急性重型为HBsAg携带者重叠HEV感染引起。", "entities": [ { "start_idx": 38, "end_idx": 42, "type": "mic", "entity": "HBsAg" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "重叠HEV感染" } ] }, { "text": "【病原学诊断】(一)病毒颗粒和抗原检查在潜伏期末至急性早期取粪便用免疫电镜检出病毒颗粒或用酶免疫法检测病毒抗原。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "mic", "entity": "病毒颗粒" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "粪便" }, { "start_idx": 33, "end_idx": 36, "type": "equ", "entity": "免疫电镜" }, { "start_idx": 39, "end_idx": 40, "type": "mic", "entity": "病毒" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "酶免疫法" }, { "start_idx": 51, "end_idx": 52, "type": "mic", "entity": "病毒" } ] }, { "text": "(二)血清学检查急性期特异性IgM阳性有临床诊断价值。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "血清学检查" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "急性期特异性IgM阳性" } ] }, { "text": "发病后2~3周,特异性IgG检出率为72.7%,4~8周时为84.9%。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "ite", "entity": "特异性IgG" } ] }, { "text": "采用完整的ORF2蛋白作抗原建立的EIA法具有较高的灵敏度和特异性。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "EIA法" } ] }, { "text": "(三)病毒基因检查用RT-PCR法可在血清和粪便中检测HEVRNA。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "病毒基因检查" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "RT-PCR法" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血清" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "粪便" }, { "start_idx": 27, "end_idx": 32, "type": "ite", "entity": "HEVRNA" } ] }, { "text": "【预防和治疗】(一)预防主要是保护水源、加强食品卫生管理、注意个人卫生和改善环境卫生。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "保护水源" }, { "start_idx": 20, "end_idx": 27, "type": "pro", "entity": "加强食品卫生管理" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "注意个人卫生" }, { "start_idx": 36, "end_idx": 41, "type": "pro", "entity": "改善环境卫生" } ] }, { "text": "人丙种球蛋白对本病无明显预防作用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "人丙种球蛋白" } ] }, { "text": "基因重组疫苗和核酸疫苗正在研究之中。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "基因重组疫苗" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "核酸疫苗" } ] }, { "text": "(二)治疗尚无特异性抗病毒药物。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "病毒" } ] }, { "text": "综合对症措施同甲型肝炎。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "甲型肝炎" } ] }, { "text": "二、病理生理(一)低氧血症及其对机体的影响1.氧摄取困难当通气不足或通气中氧含量太低时,会出现机体氧摄取不足。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "低氧血症" } ] }, { "text": "氧分压降低时,刺激颈动脉体和主动脉弓的化学感受器,通过兴奋呼吸中枢,增强呼吸活动。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "颈动脉体" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "化学感受器" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "呼吸中枢" } ] }, { "text": "慢性缺氧对刺激呼吸的影响,则主要通过促红细胞生成素调节机制,使红细胞生成增加,提高携带氧功能,以保证组织脏器供氧。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "促红细胞生成素" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "红细胞" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "组织脏器" } ] }, { "text": "全肺各部分通气-灌流比例实际上并不一致,只是理论上每一部分肺泡保持此比例,才能保持和发挥肺脏的最大换气效率。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "全肺" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肺泡" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "肺脏" } ] }, { "text": "如果肺泡通气量显著大于灌流,或肺灌流量显著减少,此部分通气-灌流比例显著大于1.0,则该部分肺泡不能保证血液氧和二氧化碳的交换,通气无效,无效腔通气量增加。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肺泡" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "肺泡" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "血液" } ] }, { "text": "如果通气量显著减少,此时肺内通气-灌流比例低于0.8,没有获得气体交换的血液经肺泡毛细血管流入肺静脉,出现静动脉分流。", "entities": [ { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肺" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "血液" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肺泡" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "毛细血管" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "肺静脉" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "静动脉" } ] }, { "text": "3.对脏器功能的影响小儿体内氧储存量较少,以10kg体重小儿为例,肺泡功能残气中氧含量50~60ml,血液中氧与血红蛋白的结合量约180ml,总计约240ml。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "脏器" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肺泡" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "血液" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "按动静脉氧含量差为33%(相当于SaO2由90%下降到60%),可以提供基础代谢所需耗氧60~80ml/min。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "静脉" } ] }, { "text": "体内储氧量仅够维持数分钟,且PaO2<4kPa时,大脑皮层出现不可复原的损伤。", "entities": [ { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "大脑皮层" } ] }, { "text": "从有氧代谢转化为无氧代谢,能量转化效率显著降低,产生大量乳酸,可以引起代谢性酸中毒等代谢紊乱和脏器系统功能失调。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "乳酸" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "酸中毒" } ] }, { "text": "(1)肺:持续处于低氧状态可以使肺小动脉痉挛,产生肺动脉高压和肺水肿,可以导致严重的肺通气-灌流失调。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "肺小动脉" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "肺水肿" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "肺" } ] }, { "text": "(2)心血管:缺氧通过交感神经兴奋使心率加快、血压升高、心输出量增加。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "心血管" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "交感神经" } ] }, { "text": "(3)中枢神经:随缺氧程度逐渐加重,可以出现脑细胞水肿,血-脑屏障通透性增加,脑血管扩张,脑血流增加,最终导致脑水肿和颅内高压,出现中枢性呼吸衰竭的症状。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "中枢神经" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "脑细胞水肿" }, { "start_idx": 28, "end_idx": 37, "type": "sym", "entity": "血-脑屏障通透性增加" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "脑血管扩张" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "脑血流增加" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "脑水肿" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "颅内高压" }, { "start_idx": 66, "end_idx": 72, "type": "dis", "entity": "中枢性呼吸衰竭" } ] }, { "text": "(4)肾脏:缺氧非常容易导致肾脏血管痉挛,肾血流显著下降,滤过减少,出现少尿和无尿。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "肾脏血管" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "肾" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "少尿" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "无尿" } ] }, { "text": "肾素-血管紧张素-醛固酮系统对血管张力、水、盐、电解质代谢的调节作用,亦随全身性低氧状况而丧失,进一步加重临床症状。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "bod", "entity": "肾素-血管紧张素-醛固酮系统" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血管" } ] }, { "text": "(5)胃肠道和肝脏:缺氧导致的循环障碍使胃肠道淤血,引起出血、坏死性小肠结肠炎,肝脏出现小叶中心坏死,功能受损失去对体内代谢产物的加工处理。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "胃肠道" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肝脏" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "胃肠道" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "出血" }, { "start_idx": 31, "end_idx": 38, "type": "dis", "entity": "坏死性小肠结肠炎" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "肝脏" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "小叶中心坏死" } ] }, { "text": "(6)造血系统:低氧可以增加促红细胞生成素(erythropoietin,EPO),刺激骨髓红细胞生成增加。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "造血系统" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "促红细胞生成素" }, { "start_idx": 22, "end_idx": 35, "type": "bod", "entity": "erythropoietin" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "EPO" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "骨髓红细胞" } ] }, { "text": "EPO主要在肾脏活化。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "EPO" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肾脏" } ] }, { "text": "红细胞增加可以提高携氧能力,代偿组织缺氧。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "红细胞" } ] }, { "text": "但在急性呼吸衰竭时,低氧对骨髓的抑制,使EPO的作用产生缓慢或不起作用。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "骨髓" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "EPO" } ] }, { "text": "(二)二氧化碳潴留及其对机体的影响1.中枢对二氧化碳的调节敏感性和反应性动脉血二氧化碳分压变化通过延髓和颈动脉体化学感受器影响呼吸运动强弱和通气量。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "中枢" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "动脉血" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "延髓" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "颈动脉" }, { "start_idx": 55, "end_idx": 60, "type": "bod", "entity": "体化学感受器" } ] }, { "text": "二氧化碳透过血-脑屏障,进入脑脊液,解离出氢离子,刺激感受器。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "血-脑屏障" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "脑脊液" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "感受器" } ] }, { "text": "反应机制中颈动脉体的作用占1/3,反应快;延脑作用占2/3,作用较持续。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "颈动脉体" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "延脑" } ] }, { "text": "当二氧化碳进一步升高时,可以抑制大脑皮质下层,出现嗜睡和呼吸抑制。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "大脑皮质下层" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "嗜睡" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "呼吸抑制" } ] }, { "text": "不同个体和不同疾病状况下,外周和中枢化学感受器对于二氧化碳的不同程度变化,其敏感性和反应性不同。", "entities": [ { "start_idx": 13, "end_idx": 22, "type": "bod", "entity": "外周和中枢化学感受器" } ] }, { "text": "对于呼吸衰竭出现兴奋烦躁、与呼吸机对抗者,可以通过应用镇静剂,以降低中枢对二氧化碳反应的敏感性,但仍然可以维持中枢对二氧化碳的调节反应性。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 14, "end_idx": 16, "type": "equ", "entity": "呼吸机" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "镇静剂" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "中枢" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "中枢" } ] }, { "text": "呼吸肌长期负担过重而导致动力性衰竭,或长期二氧化碳潴留,可以出现敏感性和反应性同时下降。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "动力性衰竭" } ] }, { "text": "过多使用肌松剂导致呼吸肌失用性变性变性等,也可以导致对二氧化碳呼吸调节作用的下降。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "肌松剂" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "呼吸肌失用性变性" } ] }, { "text": "2.二氧化碳潴留对脏器功能的影响(1)呼吸系统:机体二氧化碳代谢特点表现为由组织-循环血-肺泡的二氧化碳分压递降。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "组织" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "循环血" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "肺泡" } ] }, { "text": "呼吸衰竭时可因肺通气障碍,导致肺泡内二氧化碳排出困难,在通气不足时,组织、循环和肺泡内二氧化碳潴留,而呼出气二氧化碳分压降低。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺泡" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "组织" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "肺泡" } ] }, { "text": "当通气改善而肺血流灌注不良时,出现组织和循环二氧化碳潴留,肺泡内气和呼出气二氧化碳分压降低。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肺" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肺泡" } ] }, { "text": "当通气-灌流和肺换气功能改善,但外周循环没有改善,则循环血和呼出气二氧化碳分压逐渐提高,而且差别减小;当外周循环改善后,会出现循环血和呼出气二氧化碳分压增高的阶段,然后随组织二氧化碳潴留的解除,循环血和呼出气二氧化碳分压水平恢复到正常。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "循环血" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "循环血" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "循环血" } ] }, { "text": "二氧化碳增加可以使肺血管收缩,肺血流量下降。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肺血管" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肺" } ] }, { "text": "(2)中枢神经系统:正常人脑循环对于二氧化碳敏感。", "entities": [ { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "脑" } ] }, { "text": "当吸入气含CO25%时,或PaCO2提高1~2kPa时,脑血流量可提高40%~50%,可以出现颅内压上升,致头痛、视神经乳头水肿、肌张力增高、瞳孔变化等症状和体征。", "entities": [ { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "脑" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "颅内压上升" }, { "start_idx": 76, "end_idx": 76, "type": "bod", "entity": "头" }, { "start_idx": 79, "end_idx": 83, "type": "bod", "entity": "视神经乳头" }, { "start_idx": 87, "end_idx": 87, "type": "bod", "entity": "肌" }, { "start_idx": 93, "end_idx": 94, "type": "bod", "entity": "瞳孔" } ] }, { "text": "颅高压严重者可以发展为脑疝,延髓受压迫后中枢呼吸停止而死亡。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "脑疝" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "延髓" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "中枢" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "死亡" } ] }, { "text": "(3)心血管系统:二氧化碳分压升高可以使心率、血压、心输出量反射性增加。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "心血管系统" } ] }, { "text": "如果二氧化碳上升过高,可以出现心率、血压、心输出量降低,出现心律不齐,外周血管扩张症状。", "entities": [ { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "血管" } ] }, { "text": "(4)肾脏:二氧化碳潴留存在轻度酸中毒时,肾血管血流增加,促进肾脏排尿作用。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "酸中毒" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肾血管" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肾脏" } ] }, { "text": "当呼吸性酸中毒失代偿时,pH显著下降,肾血管痉挛、血流减少,尿量和钠离子排出量亦显著减少。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "pH显著下降" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "肾血管" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "肾血管痉挛" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "血流减少" }, { "start_idx": 30, "end_idx": 43, "type": "sym", "entity": "尿量和钠离子排出量亦显著减少" } ] }, { "text": "3.酸碱失衡和电解质紊乱二氧化碳潴留可以导致呼吸性酸中毒,并出现一系列电解质紊乱。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "酸碱失衡和电解质紊乱" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "酸中毒" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "电解质紊乱" } ] }, { "text": "(1)碳酸氢根/碳酸对酸中毒的调节失代偿:组织生成的CO2主要在红细胞和肾小管上皮细胞内,经碳酸酐酶催化生成碳酸,碳酸迅速解离成H+和离子。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "酸中毒" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "红细胞" }, { "start_idx": 47, "end_idx": 53, "type": "bod", "entity": "肾小管上皮细胞" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "碳酸酐酶" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "碳酸" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "碳酸" } ] }, { "text": "血液中5%的CO2溶解在血浆中,95%的CO2进入红细胞,而大部分的逸出红细胞外,相应的Cl-进入红细胞内(氯移现象),伴随血清氯离子减少。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血液" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "血浆" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "红细胞" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "红细胞" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "红细胞" }, { "start_idx": 95, "end_idx": 96, "type": "bod", "entity": "血清" } ] }, { "text": "血浆中CO2总量包括溶解的CO2和。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血浆" } ] }, { "text": "呼吸性酸中毒主要为PaCO2变化(升高)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "呼吸性酸中毒" } ] }, { "text": "当呼吸性酸中毒合并代谢性酸中毒时,可以出现的下降,还可出现严重电解质紊乱和阴离子间隙升高。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "(2)氢-钾交换:细胞内酸中毒时大量氢离子产生,细胞内钾离子和细胞外液中氢离子交换(3个钾离子与2个钠离子、1个氢离子交换),可以导致细胞内酸中毒和低钾。", "entities": [ { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "细胞外液" }, { "start_idx": 67, "end_idx": 72, "type": "dis", "entity": "细胞内酸中毒" }, { "start_idx": 74, "end_idx": 75, "type": "dis", "entity": "低钾" } ] }, { "text": "此外,远端肾小管氢-钾交换加强,随氢离子大量排出,血清钾水平升高。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肾小管" } ] }, { "text": "呼吸性酸中毒时血清钾离子水平与pH呈负相关,pH越低,血钾越高。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "呼吸性酸中毒" } ] }, { "text": "理论上血浆阳离子总数和阴离子总数相等,Na++UC=Cl-++UA,对上式移项得到:UA-UC=Na+-(Cl-+),正常范围为8~16mmol/L,平均12mmol/L。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血浆" } ] }, { "text": "呼吸衰竭时,由于组织缺氧,组织无氧代谢-糖酵解增强,可以出现乳酸增高伴酸中毒,为乳酸性酸中毒,常见于呼吸性酸中毒时,可以通过测定血乳酸、AG加以诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "组织" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "乳酸" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "酸中毒" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "乳酸性酸中毒" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 64, "end_idx": 66, "type": "ite", "entity": "血乳酸" }, { "start_idx": 68, "end_idx": 69, "type": "ite", "entity": "AG" } ] }, { "text": "尤其当治疗过程中补碱液、机体代偿使增加并恢复到正常范围,但酸中毒仍然存在时,如果AG增高,仍可以判断有代谢性紊乱。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "酸中毒" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "AG" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "代谢性紊乱" } ] }, { "text": "但危重呼吸衰竭时,应根据临床病情和处理综合判断。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "危重呼吸衰竭" } ] }, { "text": "三、出血性疾病的鉴别诊断(一)血管止血功能障碍引起的出血性疾病1.遗传性出血性毛细血管扩张症同一患者反复同一部位的出血,儿童以鼻出血、牙龈出血最为常见,成人还可见胃肠道、泌尿道等内脏出血。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "出血性疾病" }, { "start_idx": 16, "end_idx": 31, "type": "dis", "entity": "血管止血功能障碍引起的出血性疾病" }, { "start_idx": 34, "end_idx": 46, "type": "dis", "entity": "遗传性出血性毛细血管扩张症" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "出血" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "出血" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "胃肠道" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "泌尿道" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "内脏" } ] }, { "text": "体格检查可见口腔、鼻黏膜、甲床和手掌等部位的毛细血管扭曲扩张,呈细点状或结节状;全身皮肤、黏膜并无瘀点、瘀斑。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "口腔" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "鼻黏膜" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "甲床" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "手掌" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "毛细血管" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "皮肤" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "黏膜" } ] }, { "text": "出血时间延长、束臂试验阳性,毛细血管镜下可见扩张的毛细血管。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "equ", "entity": "毛细血管镜" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "毛细血管" } ] }, { "text": "共济失调毛细血管扩张症也可见眼结合膜和皮肤的毛细血管扩张,但一般很少出血,常有反复呼吸道感染、进行性共济失调和联合免疫缺陷,易与遗传性出血性毛细血管扩张症鉴别。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "眼结合膜" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "皮肤" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "毛细血管" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "呼吸道" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "呼吸道感染" }, { "start_idx": 47, "end_idx": 53, "type": "sym", "entity": "进行性共济失调" }, { "start_idx": 55, "end_idx": 60, "type": "sym", "entity": "联合免疫缺陷" }, { "start_idx": 64, "end_idx": 76, "type": "dis", "entity": "遗传性出血性毛细血管扩张症" } ] }, { "text": "2.过敏性紫癜四肢及臂部反复出现高于皮面、大小不等的紫癜,呈对称性分布,可同时出现血尿、胃肠道出血。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "过敏性紫癜" } ] }, { "text": "半数以上病人束臂试验阳性,而出凝血时间、血小板计数、血块退缩试验等均正常。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "血小板" } ] }, { "text": "不典型的过敏性紫癜应与ITP、风湿性关节炎、肾炎及急腹症等相鉴别。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "风湿性关节炎" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "肾炎" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "急腹症" } ] }, { "text": "3.单纯性紫癜多见于青春期女孩,月经期发作加重。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "单纯性紫癜" } ] }, { "text": "4.维生素C缺乏症维生素C不仅是体内强有力的还原剂,还参加体内多种物质如胶原纤维及细胞间粘合质等的合成,缺乏时引起出血和骨骼改变。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "维生素C缺乏症" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "维生素C" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "出血" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "骨骼" } ] }, { "text": "骨骼改变可见骨膜下出血,干骺脱位、分离或骨骺嵌入,膝、踝关节肿胀血浆维生素C含量<4mg/L,四肢长骨远端X线检查有较特异改变。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "骨骼" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "骨膜" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "出血" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "骨骺" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "膝" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "踝关节" }, { "start_idx": 20, "end_idx": 31, "type": "sym", "entity": "骨骺嵌入,膝、踝关节肿胀" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "血浆" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "维生素C" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "长骨" } ] }, { "text": "5.爱-唐(Ehlers-Danlos)综合征本病系常染色体显性遗传,因结缔组织中胶原纤维数量不足和质的异常,弹力纤维相对增多所致。", "entities": [ { "start_idx": 2, "end_idx": 22, "type": "dis", "entity": "爱-唐(Ehlers-Danlos)综合征" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "结缔组织" } ] }, { "text": "临床表现为皮肤弹性过度,牵拉后弹回原位时会伴以“拍击声”;关节过度伸展、行走困难;血管脆性增高,轻微外伤可致出血,如瘀斑、血肿等,后者钙化后X线检查时可见钙化小肿瘤;受累者可表现蓝色巩膜、血肿、斜视和视网膜血管纹等。", "entities": [ { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "瘀斑" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "血肿" }, { "start_idx": 77, "end_idx": 81, "type": "sym", "entity": "钙化小肿瘤" }, { "start_idx": 89, "end_idx": 92, "type": "sym", "entity": "蓝色巩膜" }, { "start_idx": 94, "end_idx": 95, "type": "sym", "entity": "血肿" }, { "start_idx": 97, "end_idx": 98, "type": "sym", "entity": "斜视" }, { "start_idx": 100, "end_idx": 102, "type": "bod", "entity": "视网膜" }, { "start_idx": 100, "end_idx": 105, "type": "sym", "entity": "视网膜血管纹" } ] }, { "text": "(二)血小板止血功能障碍引起的出血性疾病1.特发性血小板减少性紫癜ITP是一种自身免疫性疾病,约2/3的患者循环中存在以IgG为主的抗血小板抗体,ITP在起病前1~3周多有病毒感染史。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "血小板" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "出血性疾病" }, { "start_idx": 22, "end_idx": 32, "type": "dis", "entity": "特发性血小板减少性紫癜" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "ITP" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "IgG" }, { "start_idx": 66, "end_idx": 71, "type": "bod", "entity": "抗血小板抗体" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "ITP" } ] }, { "text": "临床以皮肤、黏膜的自发性出血为主,重者亦可有器官内出血。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "皮肤" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "黏膜" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "自发性出血" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "出血" } ] }, { "text": "实验室检查可见血小板计数减少,骨髓中巨核细胞数增多或正常,成熟障碍,血液中PAIgG增多或PAC3增多。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "血小板" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "骨髓" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "巨核细胞" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "血液" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "PAIgG" } ] }, { "text": "本病与继发性血小板减少性紫癜的鉴别,急慢性ITP型别的鉴别等请参见本章第一节内容。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "继发性血小板减少性紫癜" } ] }, { "text": "2.血栓性血小板减少性紫癜(thromboticthrombocytopenicpurpura,TTP)TTP在儿童少见,主要因为微小血管内皮细胞增生、损伤造成微血栓形成并阻塞管腔所致。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "血栓性血小板减少性紫癜" }, { "start_idx": 14, "end_idx": 46, "type": "dis", "entity": "thromboticthrombocytopenicpurpura" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "TTP" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "TTP" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "血管" } ] }, { "text": "临床病变广泛,主要表现为血小板减少性出血,溶血性贫血和神经系统受累,通常发病急骤并有高热,组织病理学检查可辅助诊断。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "血小板" } ] }, { "text": "TTP发病机制尚未完全清楚,临床表现与实验室检查酷似Evans综合征,溶血尿毒综合征和DIC,更有人认为溶血尿毒综合征与TTP实际上是一种疾病的两种型别。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "TTP" }, { "start_idx": 26, "end_idx": 33, "type": "dis", "entity": "Evans综合征" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "溶血尿毒综合征" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "DIC" }, { "start_idx": 52, "end_idx": 58, "type": "dis", "entity": "溶血尿毒综合征" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "TTP" } ] }, { "text": "3.原发性血小板增多症临床上血小板持续增多达600×109/L以上,伴反复自发性皮肤、黏膜出血、血栓形成和脾大为特征。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "原发性血小板增多症" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "血小板" } ] }, { "text": "4.血管瘤-血小板减少综合征先天性的皮肤或内脏海绵状血管瘤会随年龄增长而增大,血小板则相应减少。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dis", "entity": "血管瘤-血小板减少综合征" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "皮肤" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "内脏" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "血管瘤" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "血小板" } ] }, { "text": "究其原因可能系血小板在血管中凝聚或形成血小板栓子所致。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "血小板" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血管" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "血小板" } ] }, { "text": "病儿可在生后数周内也可致成年后发生皮肤、黏膜或内脏的出血,重者可致DIC甚至死亡。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "皮肤" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "DIC" } ] }, { "text": "5.周期性血小板减少症血小板呈周期性减少伴出血,通常10~25天为一周期,重者可出血致死。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "周期性血小板减少症" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "血小板" } ] }, { "text": "本症病因未明,临床可见有随月经周期变化的,有巨核细胞本身周期性减少、消失和伴成熟障碍的,也有血小板减少时骨髓巨核细胞反而增多等亚型。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "巨核细胞" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "血小板" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "骨髓" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "巨核细胞" } ] }, { "text": "6.先天性血小板功能障碍性疾病本组疾病病情请参见本章第二节有关内容,注意与获得性血小板功能障碍性疾病相鉴别。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "先天性血小板功能障碍性疾病" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "血小板" } ] }, { "text": "(三)凝血障碍引起的出血性疾病1.血友病凝血障碍性出血性疾病约占我国小儿出血性疾病的12%,其中血友病甲约占70%~80%,血友病乙约占10%~15%,血友病丙仅占1%~2%,前二者为性联隐性遗传,后者为不完全性常染色体隐性遗传。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "凝血障碍引起的出血性疾病" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "血友病" }, { "start_idx": 20, "end_idx": 29, "type": "dis", "entity": "凝血障碍性出血性疾病" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "血友病甲" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "血友病乙" }, { "start_idx": 76, "end_idx": 79, "type": "dis", "entity": "血友病丙" } ] }, { "text": "血友病的特点是轻微损伤后出血不止的倾向,重者常有软组织的深部血肿和关节腔出血APTT多延长,PT及TT正常,APTT纠正试验及凝血因子活性测定有助诊断。", "entities": [ { "start_idx": 7, "end_idx": 18, "type": "sym", "entity": "轻微损伤后出血不止的倾向" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "软组织" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "关节腔" }, { "start_idx": 24, "end_idx": 37, "type": "sym", "entity": "软组织的深部血肿和关节腔出血" }, { "start_idx": 38, "end_idx": 41, "type": "ite", "entity": "APTT" }, { "start_idx": 46, "end_idx": 47, "type": "ite", "entity": "PT" }, { "start_idx": 54, "end_idx": 57, "type": "ite", "entity": "APTT" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "凝血因子" } ] }, { "text": "2.血管性血友病本病为常染色体显性遗传,少数为隐性遗传。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "血管性血友病" } ] }, { "text": "因子Ⅷ是由低分子量的因子Ⅷ∶C和高分子量的vWF组成的蛋白复合物,因为vWF合成障碍导致vWF缺乏或分子结构异常是本病的基本缺陷,继而引起血小板对损伤血管壁黏附功能障碍,对瑞斯托霉素诱导的聚集反应降低和Ⅷ∶C降低。", "entities": [ { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "血小板" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "血管壁" }, { "start_idx": 86, "end_idx": 90, "type": "dru", "entity": "瑞斯托霉素" } ] }, { "text": "vWD以皮肤黏膜出血为多见,重者可有内脏出血及手术时异常出血,其中肌肉及关节出血少见。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "皮肤" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "黏膜" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肌肉" } ] }, { "text": "3.维生素K缺乏症凝血因子由前体蛋白转变成具有生物活性的过程中需维生素K参与。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "维生素K缺乏症" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "凝血因子" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "维生素K" } ] }, { "text": "新生儿及小婴儿时有发病,前者系孕母维生素K通过胎盘少,或用过影响维生素K代谢药物,患儿吸收减少所致。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "维生素K" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "胎盘" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "维生素K" } ] }, { "text": "实验室检查凝血时间(CT)轻度延长或正常,凝血酶原时间延长,血小板正常即可确诊。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "血小板" } ] }, { "text": "4.先天性纤维蛋白原缺乏症本病为常染色体隐性或不完全隐性遗传,临床罕见。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "先天性纤维蛋白原缺乏症" } ] }, { "text": "杂合子多无出血,纯合子可有出血表现,其中APTT、PT、TT均延长、血浆纤维蛋白原量显著减少;成年人中须注意与获得性纤维蛋白原缺乏症相鉴别。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "ite", "entity": "APTT" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "PT" }, { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "TT" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "血浆纤维蛋白" }, { "start_idx": 55, "end_idx": 65, "type": "dis", "entity": "获得性纤维蛋白原缺乏症" } ] }, { "text": "5.抗凝物质过多血友病甲反复应用因子Ⅷ制剂后,约6%~9%病例可产生抗体,在自身免疫性疾病、淋巴瘤、药物反应等中也可出现因子Ⅷ抗体。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "血友病甲" }, { "start_idx": 16, "end_idx": 20, "type": "dru", "entity": "因子Ⅷ制剂" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "抗体" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "因子Ⅷ抗体" } ] }, { "text": "抗原与抗体结合的结果,抑制了因子Ⅷ活性,临床表现与实验室检查结果均类似甲型血友病。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "抗体" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "因子Ⅷ" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "甲型血友病" } ] }, { "text": "肝功能衰竭、放射病、恶性肿瘤或肝素使用过量等可引起循环中肝素或类肝素物质增多,抑制Ⅴ、Ⅷ、Ⅹ、Ⅻ和对抗凝血酶、结果CT显著延长,PT中度延长,使用鱼精蛋白或甲苯胺蓝可纠正CT延长即能诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "肝功能衰竭" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "放射病" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 15, "end_idx": 16, "type": "dru", "entity": "肝素" }, { "start_idx": 28, "end_idx": 29, "type": "dru", "entity": "肝素" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "类肝素" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "凝血酶" }, { "start_idx": 57, "end_idx": 58, "type": "ite", "entity": "CT" }, { "start_idx": 64, "end_idx": 65, "type": "ite", "entity": "PT" }, { "start_idx": 73, "end_idx": 76, "type": "dru", "entity": "鱼精蛋白" }, { "start_idx": 78, "end_idx": 81, "type": "dru", "entity": "甲苯胺蓝" }, { "start_idx": 85, "end_idx": 86, "type": "bod", "entity": "CT" } ] }, { "text": "6.DIC本症是多种因素引起的一组严重的出血综合征。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "出血综合征" } ] }, { "text": "实验室检查发现有下列三项异常者可确定诊断:①血小板<100×109/L或进行性下降;②血浆纤维蛋白原含量<1.5g/L或进行性下降;③3P试验阳性或FDP>20mg/L;或血浆D-二聚体水平升高;④PT缩短或延长3秒以上或呈动态变化;⑤外周血破碎红细胞>2%。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "血小板" }, { "start_idx": 49, "end_idx": 55, "type": "ite", "entity": "血浆纤维蛋白原" }, { "start_idx": 73, "end_idx": 76, "type": "pro", "entity": "3P试验" }, { "start_idx": 105, "end_idx": 106, "type": "ite", "entity": "PT" }, { "start_idx": 124, "end_idx": 131, "type": "ite", "entity": "外周血破碎红细胞" } ] }, { "text": "此外纤溶酶原和AT-Ⅲ的含量及活性降低,因子Ⅷ∶C活性<50%等有助于疑难病例的诊断。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "纤溶酶原" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "AT-Ⅲ" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "因子Ⅷ∶C" } ] }, { "text": "第三章儿童恶性肿瘤诊断治疗工作模式第一节多学科联合工作模式儿童肿瘤是一个较为复杂的疾病。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "儿童恶性肿瘤" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "诊断及治疗涉及外科、内科肿瘤专业、病理科、影像学科、放疗科、免疫学、遗传学等多个学科。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dep", "entity": "外科" }, { "start_idx": 10, "end_idx": 11, "type": "dep", "entity": "内科" }, { "start_idx": 17, "end_idx": 19, "type": "dep", "entity": "病理科" }, { "start_idx": 21, "end_idx": 24, "type": "dep", "entity": "影像学科" }, { "start_idx": 26, "end_idx": 28, "type": "dep", "entity": "放疗科" } ] }, { "text": "(一)外科在儿童肿瘤中的作用除血液系统恶性肿瘤外,外科在儿童恶性肿瘤的诊断与治疗中起着至关重要的作用,手术是儿童实体瘤的三大主要治疗手段之一。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dep", "entity": "外科" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "血液系统恶性肿瘤" }, { "start_idx": 25, "end_idx": 26, "type": "dep", "entity": "外科" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "儿童恶性肿瘤" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "手术" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "儿童实体瘤" } ] }, { "text": "随着诊断与治疗技术的不断发展,外科医生越来越需要其他相关专业参与儿童肿瘤的诊治工作。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dep", "entity": "外科" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "外科医生在儿童肿瘤中的主要作用有以下几个方面:1.向肿瘤专业、影像学、病理科等相关学科提供临床资料,如肿块大小、质地、粘连及术中情况,辅助肿瘤专业、影像学科和病理科作出完善而正确的诊断。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dep", "entity": "外科" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 26, "end_idx": 29, "type": "dep", "entity": "肿瘤专业" }, { "start_idx": 31, "end_idx": 33, "type": "dep", "entity": "影像学" }, { "start_idx": 35, "end_idx": 37, "type": "dep", "entity": "病理科" }, { "start_idx": 69, "end_idx": 72, "type": "dep", "entity": "肿瘤专业" }, { "start_idx": 74, "end_idx": 77, "type": "dep", "entity": "影像学科" }, { "start_idx": 79, "end_idx": 81, "type": "dep", "entity": "病理科" } ] }, { "text": "2.在肿瘤不能完全或大部分切除时作肿块活检,提供诊断用标本。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肿瘤" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "大部分切除" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "肿块活检" } ] }, { "text": "3.对能完全切除的肿瘤做根治性手术,达到治愈的目的。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肿瘤" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "手术" } ] }, { "text": "或做肿瘤大部分切除术切除术,结合其他治疗方法,增加治愈机会。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "大部分切除术" } ] }, { "text": "4.以缓解症状改善生命质量为目的的姑息性手术,如造瘘术、转流术、减压术等。", "entities": [ { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "姑息性手术" }, { "start_idx": 24, "end_idx": 26, "type": "pro", "entity": "造瘘术" }, { "start_idx": 28, "end_idx": 30, "type": "pro", "entity": "转流术" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "减压术" } ] }, { "text": "(二)病理科的作用1.结合影像学、外科、肿瘤科及其他学科提供的临床资料作出正确的病理诊断、分型,指导临床治疗方案的选择。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dep", "entity": "病理科" }, { "start_idx": 13, "end_idx": 15, "type": "dep", "entity": "影像学" }, { "start_idx": 17, "end_idx": 18, "type": "dep", "entity": "外科" }, { "start_idx": 20, "end_idx": 22, "type": "dep", "entity": "肿瘤科" } ] }, { "text": "2.对切除的临近组织、器官、淋巴结作肿瘤浸润情况报告,提供临床分期依据,对治疗方案的强度选择至关重要。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "组织" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "器官" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "淋巴结" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "肿瘤" } ] }, { "text": "3.对残留病灶的再活检,提供组织细胞病理水平完全缓解依据,指导进一步治疗计划的制定。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "活检" } ] }, { "text": "(三)影像学科的作用通过肿瘤相关部位的X线平片、造影、超声检查、MRI、CT检查等向临床医生提供各种必要的信息。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dep", "entity": "影像学科" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肿瘤" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "X线平片" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "造影" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "超声检查" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "MRI" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "CT检查" } ] }, { "text": "1.向有关学科提供肿瘤影像学特征,如肿瘤大小、边界、钙化、液化及出血等,并作出影像学诊断,供临床医生及病理科参考。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "肿瘤" }, { "start_idx": 51, "end_idx": 53, "type": "dep", "entity": "病理科" } ] }, { "text": "2.手术前向外科医生提供肿瘤与邻近脏器、组织、血管的关系,与外科医生共同确定手术的可行性及手术范围,完善手术方案。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "手术" }, { "start_idx": 6, "end_idx": 7, "type": "dep", "entity": "外科" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肿瘤" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "脏器" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "组织" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "血管" }, { "start_idx": 30, "end_idx": 31, "type": "dep", "entity": "外科" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "手术" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "手术" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "手术" } ] }, { "text": "3.从影像学角度确定病变扩散范围,提供肿瘤分期依据。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "肿瘤" } ] }, { "text": "(四)肿瘤科的作用儿童肿瘤专业是一个新兴学科,既往儿童实体瘤主要在儿童外科诊治,随着化疗等技术的发展,许多儿童肿瘤在化疗介入后预后有了明显的改善。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dep", "entity": "肿瘤科" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "儿童实体瘤" }, { "start_idx": 33, "end_idx": 36, "type": "dep", "entity": "儿童外科" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "化疗" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 58, "end_idx": 59, "type": "pro", "entity": "化疗" } ] }, { "text": "由于化疗及其他诊治技术越来越复杂,就需要有一组专业性人员来负责包括化疗在内的诊治方案的制定、实施及观察、随访,并负责各学科之间的协作。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "化疗" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "化疗" } ] }, { "text": "在进展中逐渐形成了儿童肿瘤专业,这一专业的人员通常来自熟悉儿童白血病化疗及支持治疗的血液专业。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "儿童白血病" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "化疗" } ] }, { "text": "它的主要任务是与外科、病理科、放疗科、影像学等各学科协作,使诊断确切完善,治疗更合理有效,病人随访管理落实。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dep", "entity": "外科" }, { "start_idx": 11, "end_idx": 13, "type": "dep", "entity": "病理科" }, { "start_idx": 15, "end_idx": 17, "type": "dep", "entity": "放疗科" }, { "start_idx": 19, "end_idx": 21, "type": "dep", "entity": "影像学" } ] }, { "text": "2.结合外科、放疗科意见制定合理的综合治疗方案。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dep", "entity": "外科" }, { "start_idx": 7, "end_idx": 9, "type": "dep", "entity": "放疗科" } ] }, { "text": "(五)放疗科的作用与外科手术、内科化疗一样,放疗是儿童肿瘤三大主要治疗方法之一。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dep", "entity": "放疗科" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "外科手术" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "内科化疗" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "放疗" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "2.以治愈为目的的放疗方案的制定与实施,目前的研究表明放疗对儿童肾母细胞瘤、脑瘤、尤因肉瘤、横纹肌肉瘤的预后有明显良好的影响。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "放疗" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "放疗" }, { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "儿童肾母细胞瘤" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "脑瘤" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "尤因肉瘤" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "3.以减轻症状为目的的姑息性放疗,缓解一些晚期肿瘤压迫或浸润引起的剧烈疼痛。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "姑息性放疗" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "肿瘤" } ] }, { "text": "(六)各专业间协作工作模式——多专业联合讨论制对儿童肿瘤必须有多专业的协调工作才能按计划实施诊治计划,因此应建立一个常规的多专业(外科、病理科、血液/肿瘤科、影像学科和放疗科为基本组成单位)讨论制度,对所有新病例和随访诊治过程中需解决的问题进行讨论,对诊断、治疗方案达成共识,并落实讨论所提出的要求和各专业间病人的交接。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 65, "end_idx": 66, "type": "dep", "entity": "外科" }, { "start_idx": 68, "end_idx": 70, "type": "dep", "entity": "病理科" }, { "start_idx": 72, "end_idx": 77, "type": "dep", "entity": "血液/肿瘤科" }, { "start_idx": 79, "end_idx": 82, "type": "dep", "entity": "影像学科" }, { "start_idx": 84, "end_idx": 86, "type": "dep", "entity": "放疗科" } ] }, { "text": "以目前的治疗进展,儿童实体瘤治疗常包括外科手术,内科化疗以及放疗的综合治疗措施。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "儿童实体瘤" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "外科手术" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "内科化疗" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "放疗" } ] }, { "text": "手术和放疗常在短期内完成,而化疗为相对长期治疗,并需要在内科肿瘤专业医生的密切观察下进行,因此以内科肿瘤专业医生为主负责全程治疗计划实施和随访较为合理。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "放疗" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "化疗" }, { "start_idx": 28, "end_idx": 29, "type": "dep", "entity": "内科" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "肿瘤" }, { "start_idx": 48, "end_idx": 49, "type": "dep", "entity": "内科" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "肿瘤" } ] }, { "text": "对儿童实体肿瘤,由外科医生单独给予手术并进行术后不规则化疗和随访的诊治管理模式已不能适应当前儿童肿瘤诊断和治疗进展的需要。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "儿童实体肿瘤" }, { "start_idx": 9, "end_idx": 10, "type": "dep", "entity": "外科" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "手术" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "化疗" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "应将儿童恶性肿瘤的诊断、治疗过程视为是一个系统性工程,我国与儿童肿瘤诊断和治疗相关的各专业的设备与技术水平和发达国家相比并不十分落后,但总体治疗结果却相差较多,其主要原因是我们对诊治全过程的安排实施缺少有效的管理,各专业分割工作缺少相互沟通联系,随访机制也不完善。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "儿童恶性肿瘤" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "这些基本的工作虽然不如分子生物学那样深奥,却有十分重要的临床意义,有效的诊断治疗工作模式对儿童肿瘤的预后改善将起到事半功倍的效果,临床医务工作者应对此有足够的重视。", "entities": [ { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "(一)单核/巨噬细胞(MC/MΦ)SC在单粒细胞克隆刺激因子(MG-CSF)作用下形成集落形成单位(CFU),在单核-CSF(M-CSF)作用下发育成MΦ,在粒-CSF作用下发育成中性粒细胞(PMN)。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "bod", "entity": "单核/巨噬细胞" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "MC/MΦ" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "SC" }, { "start_idx": 20, "end_idx": 29, "type": "bod", "entity": "单粒细胞克隆刺激因子" }, { "start_idx": 31, "end_idx": 36, "type": "bod", "entity": "MG-CSF" }, { "start_idx": 56, "end_idx": 61, "type": "bod", "entity": "单核-CSF" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "M-CSF" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "MΦ" }, { "start_idx": 79, "end_idx": 83, "type": "bod", "entity": "粒-CSF" }, { "start_idx": 90, "end_idx": 94, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 96, "end_idx": 98, "type": "bod", "entity": "PMN" } ] }, { "text": "新生儿MΦ数量与成人无差别,但同PMN一样缺乏黏附分子和趋化分子刺激,其趋化功能相对较弱。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "新生儿MΦ" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "PMN" } ] }, { "text": "新生儿期淋巴细胞IFN-r产生不足,MΦ自身分泌的IFN-α也仅为成人一半左右,两者都可能影响MΦ吞噬和杀菌功能。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "bod", "entity": "淋巴细胞IFN-r" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "MΦ" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "IFN-α" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "MΦ" } ] }, { "text": "新生儿期MΦ内含较多前列腺素E2以及因血浆高甲胎蛋白水平等因素,抑制了单核-巨噬细胞表达人类白细胞抗原(HLA)DR,从而抑制了其抗原递呈能力。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "新生儿期MΦ" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "前列腺素E2" }, { "start_idx": 25, "end_idx": 31, "type": "bod", "entity": "血浆高甲胎蛋白" }, { "start_idx": 41, "end_idx": 47, "type": "bod", "entity": "单核-巨噬细胞" }, { "start_idx": 52, "end_idx": 56, "type": "mic", "entity": "白细胞抗原" }, { "start_idx": 58, "end_idx": 60, "type": "mic", "entity": "HLA" } ] }, { "text": "婴幼儿期低补体血症等也是造成MΦ功能不足的原因,MΦ大约在6岁前后才能达到成人水平。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "婴幼儿期低补体血症" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "MΦ" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "MΦ" } ] }, { "text": "(二)中性粒细胞(PMN)约在胚胎34周中性粒细胞的趋化、吞噬和杀菌功能即基本成熟,但在出生后头2周,上述功能却表现出暂时低下。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "PMN" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "中性粒细胞" } ] }, { "text": "这可能与分娩时缺氧、脑损伤以及母体高血压等因素有关,在严重感染时可以表现出边缘池中性粒细胞空虚和存活时间缩短以及一些酶的活性不足。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "缺氧" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "脑损伤" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "母体高血压" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "严重感染" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "中性粒细胞" } ] }, { "text": "补体水平低下、缺乏趋化因子及调理素也是婴幼儿中性粒细胞功能不足的重要原因。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "中性粒细胞" } ] }, { "text": "(三)T细胞及淋巴因子1.T细胞胸腺是T细胞发育成熟的重要场所,它由第3、4对鳃囊上皮细胞发育而来。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "T细胞" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "淋巴因子" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "T细胞" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "胸腺" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "T细胞" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "鳃囊上皮细胞" } ] }, { "text": "在胚胎7~8周,胸腺细胞开始分化,此后逐渐接受来自循环的造血干细胞,在胸腺上皮趋化因子诱导下前T细胞逐渐分化成熟。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "胸腺细胞" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "造血干细胞" }, { "start_idx": 35, "end_idx": 42, "type": "bod", "entity": "胸腺上皮趋化因子" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "前T细胞" } ] }, { "text": "出生时胸腺大小与功能已达高峰。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "胸腺" } ] }, { "text": "T细胞经胸腺诱导已能识别自身的主要组织相容性复合物(MHC)抗原,且形成了对自身组织的耐受性,并获得了细胞表面抗原CD3和CD11及T细胞受体(TCR)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "T细胞" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "胸腺" }, { "start_idx": 19, "end_idx": 31, "type": "mic", "entity": "相容性复合物(MHC)抗原" }, { "start_idx": 57, "end_idx": 59, "type": "mic", "entity": "CD3" }, { "start_idx": 61, "end_idx": 64, "type": "mic", "entity": "CD11" }, { "start_idx": 66, "end_idx": 70, "type": "mic", "entity": "T细胞受体" }, { "start_idx": 72, "end_idx": 74, "type": "mic", "entity": "TCR" } ] }, { "text": "成熟的T细胞主要由具辅助、诱导功能的CD4+T细胞和细胞毒性功能的CD8+T细胞组成,出生时T细胞自身发育已完善,但此时CD4+T细胞相对较多,CD4/CD8比例高达3~4,故出生后表现出CTL活性不足,易感染;TH2细胞功能相对亢进,易患过敏性疾病等特征。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "T细胞" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "T细胞" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "T细胞" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "T细胞" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "T细胞" }, { "start_idx": 112, "end_idx": 118, "type": "sym", "entity": "CTL活性不足" }, { "start_idx": 120, "end_idx": 122, "type": "sym", "entity": "易感染" }, { "start_idx": 142, "end_idx": 148, "type": "sym", "entity": "易患过敏性疾病" } ] }, { "text": "约2岁后CD8+T细胞水平才接近成人,抗感染能力逐渐增强。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "T细胞" } ] }, { "text": "新生儿未经抗原刺激的T细胞(CD45RA)比例较大,这些细胞寿命短,功能弱,而已经抗原刺激的T细胞(CD45RO)较少,虽然它们寿命长,功能强,但就整体而言T细胞尚不能充分发挥其功能。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "bod", "entity": "未经抗原刺激的T细胞" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "CD45RA" }, { "start_idx": 39, "end_idx": 48, "type": "bod", "entity": "已经抗原刺激的T细胞" }, { "start_idx": 50, "end_idx": 55, "type": "bod", "entity": "CD45RO" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "T细胞" } ] }, { "text": "小于胎龄儿及早产儿T细胞数量少,对有丝分裂原反应较低,但早产儿1个月后即可赶上足月儿,而小于胎龄儿要在1岁后才能赶上同龄正常儿童。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "T细胞" } ] }, { "text": "2.淋巴因子婴儿期T细胞在PHA诱导下分泌IL-2正常,但抗CD3单抗刺激产生IL-2却不足,新生儿期产生不足的因子有IL-3、IL-4、IL-5及IFN-γ,其水平仅为正常成人10%~25%,中度产生不足的细胞因子有GM-CSF和IL-6;轻度产生不足有TNF-α及G-CSF,未成熟儿细胞因子分泌功能更差,IL-6仅为成人25%。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "淋巴因子" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "婴儿期T细胞" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "IL-2" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "抗CD3单抗" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "IL-2" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "IL-3" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "IL-4" }, { "start_idx": 74, "end_idx": 78, "type": "bod", "entity": "IFN-γ" }, { "start_idx": 109, "end_idx": 114, "type": "bod", "entity": "GM-CSF" }, { "start_idx": 116, "end_idx": 119, "type": "bod", "entity": "IL-6" }, { "start_idx": 128, "end_idx": 132, "type": "bod", "entity": "TNF-α" }, { "start_idx": 134, "end_idx": 138, "type": "bod", "entity": "G-CSF" }, { "start_idx": 155, "end_idx": 158, "type": "bod", "entity": "IL-6" } ] }, { "text": "新生儿循环中分泌IL-2和γ-IFN的TH1类细胞相对不足,γ-IFN相当于成人水平的1/10~1/8,CTL活性明显低于年长儿和成人,因此由TH2类细胞分泌的细胞因子IL-4、5、6、10、13和IL-15占有相对优势(虽然IL-4也仅为成人1/3水平),TH1/TH2比例较正常水平低,T细胞应答向TH2偏移。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "IL-2" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "γ-IFN" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "TH1类细胞" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "γ-IFN" }, { "start_idx": 163, "end_idx": 165, "type": "bod", "entity": "T细胞" } ] }, { "text": "(四)B细胞发育及抗体合成骨髓是B细胞成熟的场所,相当于类囊的功能。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "B细胞" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "抗体" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "骨髓" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "B细胞" } ] }, { "text": "淋巴结是B细胞富集的器官,全身各处淋巴结发育先后不一,在胚胎10周时,颈部及肠系膜淋巴结最早发育,足月新生儿即可扪及腹股沟淋巴结,12岁时淋巴结发育达顶点;2岁后扁桃体才明显增大,6~7岁时扁桃体增大最为明显。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "淋巴结" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "B细胞" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "淋巴结" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "颈部" }, { "start_idx": 38, "end_idx": 43, "type": "bod", "entity": "肠系膜淋巴结" }, { "start_idx": 58, "end_idx": 63, "type": "bod", "entity": "腹股沟淋巴结" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "淋巴结" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "扁桃体" }, { "start_idx": 95, "end_idx": 97, "type": "bod", "entity": "扁桃体" } ] }, { "text": "B细胞功能在胚胎期已经成熟,但它要成为分泌抗体的浆细胞需要抗原与T细胞的多种信号辅助刺激(T细胞CD40配体,IL-4、5、6等),因而新生儿B细胞产生抗体能力差。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "B细胞" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "抗体" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "浆细胞" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "T细胞" }, { "start_idx": 45, "end_idx": 53, "type": "bod", "entity": "T细胞CD40配体" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "B细胞" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "抗体" } ] }, { "text": "虽然胎儿B细胞对抗原刺激能够产生IgM抗体,但有效的IgG抗体应答要在生后3个月后才出现。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "B细胞" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "IgM抗体" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "IgG抗体" } ] }, { "text": "小于胎龄儿抗体产生能力更差,其B细胞数量也低于足月儿,这不利于特异性抗体产生,易发生暂时性低丙种球蛋白血症。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "抗体" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "B细胞" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "抗体" }, { "start_idx": 45, "end_idx": 52, "type": "dis", "entity": "低丙种球蛋白血症" } ] }, { "text": "1.IgG孕期32周进入胎儿循环的母亲IgG明显增加,出生时脐血IgG水平甚至可高出母亲IgG水平10%,但此时自身合成抗体能力很差。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "IgG" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "IgG" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "IgG" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "IgG" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "抗体" } ] }, { "text": "早产儿及小于胎龄儿IgG水平低于母亲。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "IgG" } ] }, { "text": "生后3个月虽然自身合成能力增加,但来自母亲的IgG大量衰减,至6个月时全部消失,婴儿血清IgG水平降至最低点。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "IgG" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "血清" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "IgG" } ] }, { "text": "IgG含4种亚类成分,婴儿自身合成的IgG1及IgG3早于IgG2及IgG4;IgG1约在5岁达成人水平,IgG3在10岁左右,IgG4和IgG2要在14岁前后才达成人水平。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "IgG" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "IgG1" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "IgG3" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "IgG2" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "IgG4" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "IgG1" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "IgG3" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "IgG4" }, { "start_idx": 69, "end_idx": 72, "type": "bod", "entity": "IgG2" } ] }, { "text": "IgG1是主要针对细菌及病毒的蛋白质抗原,IgG2主要是抗多糖成分抗体。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "IgG1" }, { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "细菌" }, { "start_idx": 12, "end_idx": 19, "type": "mic", "entity": "病毒的蛋白质抗原" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "IgG2" }, { "start_idx": 28, "end_idx": 34, "type": "bod", "entity": "抗多糖成分抗体" } ] }, { "text": "IgG1约占总IgG总量70%,IgG2约为20%,IgG3占7%左右,IgG4约占3%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "IgG1" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "IgG2" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "IgG3" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "IgG4" } ] }, { "text": "若仅为某一种IgG亚类生成障碍,将会产生比较特殊的病原反复感染。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "IgG" } ] }, { "text": "2.IgM虽然胚胎12周时已能合成IgM,但因缺乏抗原刺激,胎儿自身合成的IgM量极少,母亲IgM不能通过胎盘,因而出生时脐血IgM水平很低,若发现脐血IgM水平升高(>0.2~0.3g/L),即提示胎儿有宫内感染。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "IgM" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "IgM" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "IgM" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "IgM" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "胎盘" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "IgM" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "IgM" }, { "start_idx": 103, "end_idx": 106, "type": "dis", "entity": "宫内感染" } ] }, { "text": "大约在生后3~4个月血IgM水平达成人50%,1~3岁时达成人水平75%。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "IgM" } ] }, { "text": "婴儿期低IgM血症,是易患革兰阴性菌感染的重要原因。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "婴儿期低IgM血症" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "革兰阴性菌感染" } ] }, { "text": "3.IgA脐血IgA水平很低(多<0.05g/L),若脐血IgA水平升高也提示宫内感染。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "脐血IgA" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "脐血IgA" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "宫内感染" } ] }, { "text": "分泌型IgA可从母乳中获取,且不易被水解蛋白酶分解,是黏膜局部抗感染分子。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "分泌型IgA" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "水解蛋白酶" }, { "start_idx": 27, "end_idx": 35, "type": "bod", "entity": "黏膜局部抗感染分子" } ] }, { "text": "婴幼儿期分泌型IgA低下是易患呼吸道、消化道以及尿路感染的原因。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "分泌型IgA" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "呼吸道" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "消化道" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "尿路感染" } ] }, { "text": "不同年龄IgG、A、M及IgG亚类水平(表17-17、表17-17),生后变化趋势(图17-17)。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "IgG" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "IgG" } ] }, { "text": "表17-1健康儿童血清免疫球蛋白含量(g/L)注:表内数字为均值±2s,括弧内为均值本表摘自:小儿内科学.第3版.北京:人民卫生出版社.1995:413.表17-2正常儿童血清IgG及其亚类水平(g/L)注:以均值±s表示,括弧内为95%可信区间。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "ite", "entity": "血清免疫球蛋白含量" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "血清" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "IgG" } ] }, { "text": "摘自:上海免疫学杂志,1990,10:161.图17-1生后血清免疫球蛋白浓度变化趋势4.IgD和IgE两者均难以通过胎盘,在新生儿血清中,IgD和IgE含量极少,IgD生物学功能不清楚,5岁时才达成人水平。", "entities": [ { "start_idx": 30, "end_idx": 36, "type": "bod", "entity": "血清免疫球蛋白" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "IgD" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "IgE" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "血清" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "IgD" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "IgE" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "IgD" } ] }, { "text": "IgE参与Ⅰ型变态反应,与过敏性疾病有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "IgE" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "过敏性疾病" } ] }, { "text": "生后可从母乳中获取部分IgE,自身合成IgE的能力并不弱,患过敏性疾病时血IgE水平可显著升高。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "IgE" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "IgE" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "过敏性疾病" }, { "start_idx": 36, "end_idx": 46, "type": "sym", "entity": "血IgE水平可显著升高" } ] }, { "text": "(五)补体系统新生儿各补体成分均低于成人,其C1、C2、C4、C3及C7成分仅为成人水平的35%~80%,C8及C9约为成人10%,B及P因子为35%~70%,补体旁路系统能力约为成人水平49%~65%。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "补体系统" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "补体" }, { "start_idx": 80, "end_idx": 85, "type": "bod", "entity": "补体旁路系统" } ] }, { "text": "补体分子的趋化作用是活化MΦ的重要因素之一。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "补体分子" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "MΦ" } ] }, { "text": "未成熟儿补体各成分水平更低。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "补体" } ] }, { "text": "约在生后6~12个月补体浓度或活性才接近成人水平。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "补体" } ] }, { "text": "(六)其他免疫分子血浆甘露聚糖结合蛋白(MBP)又称甘露聚糖结合凝集素(mannosebindinglectin,MBL),属于凝集素(collectin)家族。", "entities": [ { "start_idx": 9, "end_idx": 18, "type": "bod", "entity": "血浆甘露聚糖结合蛋白" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "MBP" }, { "start_idx": 26, "end_idx": 34, "type": "bod", "entity": "甘露聚糖结合凝集素" }, { "start_idx": 36, "end_idx": 55, "type": "bod", "entity": "mannosebindinglectin" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "MBL" }, { "start_idx": 64, "end_idx": 79, "type": "bod", "entity": "凝集素(collectin)家族" } ] }, { "text": "2岁以前的婴儿,获得性抗体反应尚不完善时,MBL在保护宿主免受感染中发挥天然抗感染的重要作用。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "抗体" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "MBL" } ] }, { "text": "未成熟儿MBL水平较成人为低,尤以中国未成熟儿更低,随胎龄增长而上升,约在生后10~20周达到足月新生儿水平。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "MBL" } ] }, { "text": "以纤维连接蛋白为例,纤维连接蛋白具有细胞黏附分子、趋化因子和调理素的功能,新生儿,尤其是未成熟儿水平低下,严重感染、缺氧以及窒息时纤维连接蛋白的水平将进一步下降,其感染的恢复明显不及成人。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "纤维连接蛋白" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "纤维连接蛋白" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "细胞黏附分子" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "严重感染" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "缺氧" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "窒息" }, { "start_idx": 65, "end_idx": 70, "type": "bod", "entity": "纤维连接蛋白" } ] }, { "text": "二、结核性脑膜炎结核性脑膜炎(简称结脑)是小儿结核病中最严重的疾病类型,常在初染后1年内发生。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "结脑" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "小儿结核病" } ] }, { "text": "多为全身性粟粒肺结核病的一部分,通过血行播散而来。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "全身性粟粒肺结核病" } ] }, { "text": "结核病变波及脑膜主要通过血行-脑脊液途径。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "结核病变" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脑膜" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "血行" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "脑脊液" } ] }, { "text": "此外,结脑亦可因脑实质或脑膜干酪灶破溃而引起。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "结脑" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "脑实质" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "脑膜干酪灶" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "脑实质或脑膜干酪灶破溃" } ] }, { "text": "1.前驱期(早期)约1~2周,前驱症状包括有精神状态的改变,如烦躁好哭,或精神呆滞,不喜游戏。", "entities": [ { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "精神状态的改变" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "烦躁好哭" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "精神呆滞" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "不喜游戏" } ] }, { "text": "此外可有低热、食欲减退、睡眠不安、消瘦、便秘或无原因的呕吐。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "低热" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "食欲减退" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "睡眠不安" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "消瘦" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "便秘" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "无原因的呕吐" } ] }, { "text": "年长儿可自诉头痛,初期多轻微或非持续性。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "头痛" } ] }, { "text": "婴幼儿可起病急骤,前驱期很短或无,一发病即出现脑膜刺激症状头痛持续并加重,呕吐加重并可变为喷射状。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "起病急骤" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "脑膜" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "脑膜刺激症状" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "头痛持续并加重" }, { "start_idx": 37, "end_idx": 47, "type": "sym", "entity": "呕吐加重并可变为喷射状" } ] }, { "text": "逐渐出现嗜睡,或嗜睡与烦躁不安相交替。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "逐渐出现嗜睡" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "嗜睡" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "烦躁不安" } ] }, { "text": "患儿可有感觉过敏,可有惊厥发作。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "感觉过敏" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "惊厥发作" } ] }, { "text": "此时期体征可有前囟饱满或膨隆,颈项强直,克氏征、布氏征及巴氏征阳性,浅反射一般减弱或消失,腱反射多亢进脑神经障碍症状。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "前囟饱满或膨隆" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "颈项强直" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "克氏征" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "布氏征" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "巴氏征阳性" }, { "start_idx": 34, "end_idx": 43, "type": "sym", "entity": "浅反射一般减弱或消失" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "腱" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "腱反射多亢进" }, { "start_idx": 51, "end_idx": 57, "type": "sym", "entity": "脑神经障碍症状" } ] }, { "text": "此期不少患儿已有明显颅压高及脑积水的症状及体征。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "明显颅压高" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "脑积水" } ] }, { "text": "3.昏迷期(晚期)约1~3周,以上症状逐渐加重,神志由意识曚昽、半昏迷而进入完全昏迷,多于惊厥后陷入昏迷。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "意识曚昽" }, { "start_idx": 32, "end_idx": 34, "type": "sym", "entity": "半昏迷" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "完全昏迷" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "惊厥" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "昏迷" } ] }, { "text": "阵挛性或强直性惊厥发作频繁。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "阵挛性或强直性惊厥发作频繁" } ] }, { "text": "颅压增高及脑积水现象更为明显,终因呼吸及心血管运动中枢麻痹死亡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "颅压增高" }, { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "脑积水" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "心血管运动中枢" }, { "start_idx": 17, "end_idx": 28, "type": "sym", "entity": "呼吸及心血管运动中枢麻痹" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "死亡" } ] }, { "text": "结核性脑膜炎的患儿可能产生下述并发症:脑积水、局限性脑梗死、颅内结核瘤、脑神经瘫痪、颅内压力增高、惊厥甚至死亡。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "脑积水" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "局限性脑梗死" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "颅内结核瘤" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "脑神经瘫痪" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "颅内压力增高" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "惊厥" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "死亡" } ] }, { "text": "(二)临床病型根据小儿结脑的病理变化、临床表现和病程轻重,可分为以下4型。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "结脑" } ] }, { "text": "1.浆液型其特点为浆液渗出物只局限于脑底,脑膜刺激症状和脑神经障碍不明显,脑脊液改变轻微,生化检查方面正常,经抗结核药治疗症状及脑脊液改变很快消失。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "脑底" }, { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "浆液渗出物只局限于脑底" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "脑膜" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "脑膜刺激" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "脑神经障碍" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "脑脊液" }, { "start_idx": 37, "end_idx": 43, "type": "sym", "entity": "脑脊液改变轻微" }, { "start_idx": 45, "end_idx": 48, "type": "ite", "entity": "生化检查" }, { "start_idx": 55, "end_idx": 58, "type": "dru", "entity": "抗结核药" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "脑脊液" }, { "start_idx": 64, "end_idx": 72, "type": "sym", "entity": "脑脊液改变很快消失" } ] }, { "text": "多在粟粒性结核病常规腰穿时发现。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "粟粒性结核病" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "腰穿" } ] }, { "text": "全部属早期病例,占所有早期结脑病例的1/2。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "结脑" } ] }, { "text": "2.脑底脑膜炎型为常见的一型。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "脑底脑膜炎" } ] }, { "text": "炎性病变主要位于脑底,但浆液纤维蛋白性渗出物可较弥漫。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "炎性病变" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "脑底" }, { "start_idx": 12, "end_idx": 25, "type": "sym", "entity": "浆液纤维蛋白性渗出物可较弥漫" } ] }, { "text": "其临床特征为有明显的脑膜刺激症状及脑神经障碍,可有程度不等的颅压高及脑积水症状,但没有脑局灶性症状;脑脊液有典型结脑变化结核杆菌为原发耐药菌,则疗效不佳,预后差。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "脑膜刺激" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "脑神经障碍" }, { "start_idx": 30, "end_idx": 32, "type": "sym", "entity": "颅压高" }, { "start_idx": 34, "end_idx": 36, "type": "sym", "entity": "脑积水" }, { "start_idx": 41, "end_idx": 48, "type": "sym", "entity": "没有脑局灶性症状" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "脑脊液" }, { "start_idx": 50, "end_idx": 59, "type": "sym", "entity": "脑脊液有典型结脑变化" }, { "start_idx": 60, "end_idx": 63, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "3.脑膜脑炎型当脑底脑膜炎型诊断或治疗延误,炎症病变常从脑膜蔓延到脑实质,病理上可见脑实质炎症充血或出血,多数为点状出血,少数呈弥漫性甚至大片出血。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "脑膜脑炎" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "脑底脑膜炎" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "炎症病变" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "脑膜" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "脑实质" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "脑实质炎症" }, { "start_idx": 42, "end_idx": 51, "type": "sym", "entity": "脑实质炎症充血或出血" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "点状出血" }, { "start_idx": 64, "end_idx": 72, "type": "sym", "entity": "弥漫性甚至大片出血" } ] }, { "text": "发生血管病变闭塞性动脉内膜炎时,可见到脑梗死和软化,部分病例可见到单发或多发结核瘤,并可引起局灶性症状。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血管" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "血管病变" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "闭塞性动脉内膜炎" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "脑梗死" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "结核瘤" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "局灶性症状" } ] }, { "text": "结核病变在脉络丛或脑室管膜可引起脑室管膜炎;当炎症波及间脑时可出现自主神经功能紊乱;炎症如蔓延到延髓或压迫延髓时可出现迷走神经综合征而导致死亡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "结核病变" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "脉络丛" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "脑室管膜" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "脑室管膜炎" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "炎症" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "自主神经功能紊乱" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "炎症" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "延髓" }, { "start_idx": 59, "end_idx": 65, "type": "sym", "entity": "迷走神经综合征" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "死亡" } ] }, { "text": "除脑膜刺激症状、脑神经受损症状及脑实质损害症状外,颅压最高及脑积水症状明显。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "脑膜刺激症状" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "脑神经受损症状" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "脑实质损害症状" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "颅压最高" }, { "start_idx": 30, "end_idx": 32, "type": "sym", "entity": "脑积水" } ] }, { "text": "脑脊液变化脑底脑膜炎型为轻,且可较快恢复正常,与临床好转不相平行。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑脊液" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "脑脊液变化" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "脑底脑膜炎" } ] }, { "text": "预后差,脑实质损害及脑积水严重者,即使临床恢复,亦常留有严重后遗症。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "脑实质损害" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "脑积水严重" } ] }, { "text": "4.结核性脑脊髓软、硬脑膜炎(或简称脊髓型)炎症病变不仅限于脑膜和脑实质,且蔓延到脊髓膜及脊髓。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "结核性脑脊髓软" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "硬脑膜炎" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "脊髓" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "炎症" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "脑膜" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "脑实质" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "脊髓膜" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "脊髓" } ] }, { "text": "除脑和脑膜症状外,又有脊髓及神经根的障碍,如截瘫、腱发射亢进、震颤、感觉障碍、括约肌功能障碍(尿潴留、顽固性便秘或大小便失禁)、神经性营养障碍(肢体水肿、褥疮)等。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "脑" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "脑膜" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脊髓" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "神经根" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "截瘫" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "腱" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "腱发射亢进" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "震颤" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "感觉障碍" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "括约肌" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "括约肌功能障碍" }, { "start_idx": 47, "end_idx": 49, "type": "sym", "entity": "尿潴留" }, { "start_idx": 51, "end_idx": 55, "type": "sym", "entity": "顽固性便秘" }, { "start_idx": 57, "end_idx": 61, "type": "sym", "entity": "大小便失禁" }, { "start_idx": 64, "end_idx": 70, "type": "sym", "entity": "神经性营养障碍" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "肢体水肿" }, { "start_idx": 77, "end_idx": 78, "type": "sym", "entity": "褥疮" } ] }, { "text": "脑脊液通路梗阻:有明显的蛋白、细胞分离现象,脑脊液可呈黄色,蛋白达10g/L(1g/dl)以上,甚至有达40~50g/L(4~5g/dl)之多。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "脑脊液通路" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "脑脊液通路梗阻" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "脑脊液" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "脑脊液可呈黄色" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "蛋白" } ] }, { "text": "多见于年长儿,病程长,临床恢复慢,如不合并脑积水,一般死亡率不高,但常遗留截瘫后遗症。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "脑积水" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "截瘫后遗症" } ] }, { "text": "(三)辅助检查1.脑脊液压力增高(200~360mmH2O),也可因炎性粘连、椎管梗阻而压力降低。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "脑脊液" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "压力增高" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "炎性粘连" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "椎管梗阻" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "压力降低" } ] }, { "text": "外观:早期多为无色透明,而中期或晚期可为混浊,呈玻璃样,浅黄或橙黄色。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "无色透明" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "混浊" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "玻璃样" } ] }, { "text": "65%的结脑患儿脑脊液标本静置24小时,可有薄膜形成,典型的薄膜呈漏斗状,从液面中央倒置至试管底部,用它做涂片更易找到结核杆菌。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "结脑" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "脑脊液" }, { "start_idx": 53, "end_idx": 54, "type": "pro", "entity": "涂片" }, { "start_idx": 59, "end_idx": 62, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "白细胞轻至中度增高淋巴细胞占优势,但在急性期或恶化期中性粒细胞可以占优势。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "白细胞" }, { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "白细胞轻至中度增高" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "中性粒细胞" } ] }, { "text": "一般经过1周左右,转变为淋巴细胞占优势。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "淋巴细胞占优势" } ] }, { "text": "偶见结脑患儿脑脊液白细胞数始终在正常范围。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "结脑" }, { "start_idx": 6, "end_idx": 12, "type": "ite", "entity": "脑脊液白细胞数" } ] }, { "text": "脑脊液蛋白增高大多在40~300mg之间。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "脑脊液蛋白" }, { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "脑脊液蛋白增高大多在40~300mg之间" } ] }, { "text": "蛋白含量显著最高多提示脑脊液循环发生障碍,脊髓蛛网膜炎性粘连,椎管梗阻脑脊液糖含量降低氯化物降低,较化脓性脑膜炎明显。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "蛋白含量" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "蛋白含量显著最高" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "脑脊液" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "脑脊液循环发生障碍" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "脊髓蛛网膜" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "脊髓蛛网膜炎性粘连" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "椎管" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "椎管梗阻" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "脑脊液糖" }, { "start_idx": 35, "end_idx": 42, "type": "sym", "entity": "脑脊液糖含量降低" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "氯化物降低" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "化脓性脑膜炎" } ] }, { "text": "糖和氯化物同时降低是结脑的典型改变。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "ite", "entity": "糖" }, { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "氯化物" }, { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "糖和氯化物同时降低" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "结脑" } ] }, { "text": "最可靠的诊断根据是从脑脊液查见结核杆菌。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "脑脊液" }, { "start_idx": 15, "end_idx": 18, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "2.头颅CT(图7-7)最常见异常为脑积水,其次为脑梗死、脑萎缩、脑水肿、结核瘤、钙化灶及硬膜下积液,只有10%患儿无异常发现。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "头颅CT" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "脑积水" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "脑梗死" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "脑萎缩" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "脑水肿" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "结核瘤" }, { "start_idx": 41, "end_idx": 43, "type": "sym", "entity": "钙化灶" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "硬膜下积液" } ] }, { "text": "图7-3头颅CT:第3、4脑室及两侧室扩大,周围可见低密度,大脑半球各叶沟回变平,基底池窄,密度增高,中线结构无移位3.脑电图急性期患儿绝大多数脑电图异常弥漫性慢活动,不对称。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "头颅CT" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "第3、4脑室" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "两侧室" }, { "start_idx": 9, "end_idx": 20, "type": "sym", "entity": "第3、4脑室及两侧室扩大" }, { "start_idx": 30, "end_idx": 37, "type": "bod", "entity": "大脑半球各叶沟回" }, { "start_idx": 30, "end_idx": 39, "type": "sym", "entity": "大脑半球各叶沟回变平" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "基底池" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "基底池窄" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "中线结构" }, { "start_idx": 51, "end_idx": 57, "type": "sym", "entity": "中线结构无移位" }, { "start_idx": 60, "end_idx": 62, "type": "pro", "entity": "脑电图" }, { "start_idx": 72, "end_idx": 74, "type": "pro", "entity": "脑电图" }, { "start_idx": 72, "end_idx": 76, "type": "sym", "entity": "脑电图异常" }, { "start_idx": 77, "end_idx": 86, "type": "sym", "entity": "弥漫性慢活动,不对称" } ] }, { "text": "可见不对称偶发尖式棘波;重度异常时可见明显不对称,多发尖、棘、尖-慢、棘-慢等病理波。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "不对称偶发尖式棘波" }, { "start_idx": 25, "end_idx": 41, "type": "sym", "entity": "多发尖、棘、尖-慢、棘-慢等病理波" } ] }, { "text": "于合并结核瘤或局部脑梗死时可见占位性或局灶性改变,表现为局部δ波。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "结核瘤" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "局部脑梗死" }, { "start_idx": 15, "end_idx": 23, "type": "sym", "entity": "占位性或局灶性改变" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "局部δ波" } ] }, { "text": "但脑电图的改变无特异性,仅可作为临床的辅助诊断,而对病原的鉴别诊断方面意义不大。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "pro", "entity": "脑电图" } ] }, { "text": "(四)治疗1.一般治疗严格卧床休息,营养丰富,细心护理,保证入量等。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "卧床休息" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "营养丰富" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "细心护理" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "保证入量" } ] }, { "text": "目前多采用INH、RFP和PZA联合治疗。", "entities": [ { "start_idx": 5, "end_idx": 19, "type": "pro", "entity": "INH、RFP和PZA联合治疗" } ] }, { "text": "其中INH为主要的药物,整个疗程自始至终应用。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "INH" } ] }, { "text": "疗程1~1.5年,或脑脊液正常后不少于半年。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "脑脊液" } ] }, { "text": "3.激素疗法必须与有效的抗结核药物同时应用,剂量和疗程要适中,在需要应用的病例越早用越好。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "激素疗法" } ] }, { "text": "激素对脑底脑膜炎型效果最好。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "激素" }, { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "脑底脑膜炎" } ] }, { "text": "激素的剂量要适中,泼尼松或泼尼松龙1.5~2mg/(kg•d),最大量不超过45mg/d;氢化可的松在急性期可静脉滴注1疗程1~2周,剂量5mg/(kg•d)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "激素" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "泼尼松" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "泼尼松龙" }, { "start_idx": 45, "end_idx": 49, "type": "dru", "entity": "氢化可的松" }, { "start_idx": 55, "end_idx": 58, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "对于中晚期结脑患儿用甲基泼尼松龙5mg/(kg•d),静脉滴注3~5天后逐渐减至1~2mg/(kg•d),总疗程1~2周,改为泼尼松口服,可以缩短昏迷时间,提高疗效。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "结脑" }, { "start_idx": 10, "end_idx": 15, "type": "dru", "entity": "甲基泼尼松龙" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 63, "end_idx": 65, "type": "dru", "entity": "泼尼松" } ] }, { "text": "4.脑积水的治疗治疗除常规使用激素外,可采用以下措施:(1)侧脑室穿刺、引流:适用于急性脑积水,用其他降颅压措施无效,或疑有脑疝形成时。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑积水" }, { "start_idx": 15, "end_idx": 16, "type": "dru", "entity": "激素" }, { "start_idx": 30, "end_idx": 37, "type": "pro", "entity": "侧脑室穿刺、引流" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "急性脑积水" }, { "start_idx": 51, "end_idx": 53, "type": "pro", "entity": "降颅压" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "脑疝" } ] }, { "text": "引流时应注意固定好侧脑室穿刺针,预防继发感染。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "引流" }, { "start_idx": 6, "end_idx": 14, "type": "pro", "entity": "固定好侧脑室穿刺针" } ] }, { "text": "(2)高渗液的应用:其作用原理为当静脉快速滴入高渗液后,由于血与脑脊液之间渗透压之差而产生降颅压作用。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "高渗液" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "静脉快速滴入" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "高渗液" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "血" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "脑脊液" } ] }, { "text": "常用的高渗液有30%尿素、20%甘露醇、25%山梨醇、50%葡萄糖或尿素和甘露醇混合液。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dru", "entity": "尿素" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "甘露醇" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "山梨醇" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 34, "end_idx": 35, "type": "dru", "entity": "尿素" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "甘露醇" } ] }, { "text": "剂量为每次1~1.5g/kg,于30分钟内快速静脉注入,必要时可1天2~3次。", "entities": [ { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "快速静脉注入" } ] }, { "text": "此外,亦可应用50%甘油糖浆口服,每次1~1.5/kg,1日可服3~4次,但效果较差。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "甘油糖浆" } ] }, { "text": "(3)分流手术:如果由于脑底脑膜粘连梗阻致发生梗阻性脑积水时,以上疗法均难以奏效时,在炎症基本控制的情况下,可考虑采用脑室腹腔分流术。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "分流手术" }, { "start_idx": 12, "end_idx": 19, "type": "sym", "entity": "脑底脑膜粘连梗阻" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "梗阻性脑积水" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "炎症" }, { "start_idx": 59, "end_idx": 65, "type": "pro", "entity": "脑室腹腔分流术" } ] }, { "text": "5.中医疗法及对症治疗。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "中医疗法" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "对症治疗" } ] }, { "text": "五、新生儿疾病(一)湿肺病变两侧对称。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "湿肺" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "病变两侧对称" } ] }, { "text": "轻者肺野透亮度降低,肺血管纹理增粗,胸腔少量积液,重者为白肺,但无支气管充气,数小时后就出现透亮度增强的改变,几天内可以完全吸收。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肺野" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺血管" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "胸腔" }, { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "轻者肺野透亮度降低,肺血管纹理增粗,胸腔少量积液" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "支气管" }, { "start_idx": 25, "end_idx": 37, "type": "sym", "entity": "重者为白肺,但无支气管充气" }, { "start_idx": 39, "end_idx": 63, "type": "sym", "entity": "数小时后就出现透亮度增强的改变,几天内可以完全吸收" } ] }, { "text": "(二)新生儿呼吸窘迫综合征两肺体积缩小。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "新生儿呼吸窘迫综合征" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "肺" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "两肺体积缩小" } ] }, { "text": "轻度:两肺透亮度暗淡,有细小颗粒状阴影;中度:两肺野出现毛玻璃状阴影,有支气管充气影。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "肺" }, { "start_idx": 3, "end_idx": 18, "type": "sym", "entity": "两肺透亮度暗淡,有细小颗粒状阴影" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肺野" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "支气管" }, { "start_idx": 23, "end_idx": 41, "type": "sym", "entity": "两肺野出现毛玻璃状阴影,有支气管充气影" } ] }, { "text": "重度:两肺显示白肺,心脏边缘消失,支气管充气征明显。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "肺" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "两肺显示白肺" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心脏" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "心脏边缘消失" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "支气管" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "支气管充气征明显" } ] }, { "text": "可出现气胸及支气管肺发育不良。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "气胸" }, { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "支气管肺发育不良" } ] }, { "text": "(三)新生儿吸入综合征胎粪吸入表现为颗粒状阴影,羊水吸入为薄片状阴影,两者兼有则既有颗粒状阴影,又有薄片状阴影。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "新生儿吸入综合征" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "胎粪吸入" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "颗粒状阴影" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "羊水吸入" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "薄片状阴影" }, { "start_idx": 40, "end_idx": 54, "type": "sym", "entity": "既有颗粒状阴影,又有薄片状阴影" } ] }, { "text": "可出现气胸。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "气胸" } ] }, { "text": "(四)新生儿支气管、肺发育不成熟两侧肺纹理增多,两肺野出现网粒状阴影,肺透亮度增高,有的则可出现囊状透亮区,与支气管-肺发育不良相似。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "新生儿支气管、肺发育不成熟" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肺" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "两侧肺纹理增多" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肺野" }, { "start_idx": 24, "end_idx": 33, "type": "sym", "entity": "两肺野出现网粒状阴影" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "肺" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "肺透亮度增高" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "囊状透亮区" }, { "start_idx": 55, "end_idx": 63, "type": "dis", "entity": "支气管-肺发育不良" } ] }, { "text": "(五)新生儿气胸继发于某些疾病,如新生儿呼吸窘迫综合征、湿肺、新生儿吸入综合征等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "新生儿气胸" }, { "start_idx": 17, "end_idx": 26, "type": "dis", "entity": "新生儿呼吸窘迫综合征" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "湿肺" }, { "start_idx": 31, "end_idx": 38, "type": "dis", "entity": "新生儿吸入综合征" } ] }, { "text": "大量气胸易识别,少量气胸位于纵隔、横膈旁者呈线条状,需引起重视。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "气胸" }, { "start_idx": 8, "end_idx": 24, "type": "sym", "entity": "少量气胸位于纵隔、横膈旁者呈线条状" } ] }, { "text": "三、适应证和禁忌证CPT主要适用于胸腔内中央气道内的分泌物清除,随着气道直径的逐渐缩小,其效应相对降低,因而不适用于肺泡、间质、血管及胸膜的疾病。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "CPT" }, { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "胸腔内中央气道" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "气道" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "肺泡" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "间质" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "血管" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "胸膜" } ] }, { "text": "此外还应注意以下几种情况不应用CPT治疗,以免加重病情:1.患出血性疾病、心血管外科手术后、支气管出血及心律失常的患儿。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "CPT" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "出血性疾病" }, { "start_idx": 37, "end_idx": 43, "type": "pro", "entity": "心血管外科手术" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "支气管出血" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "心律失常" } ] }, { "text": "2.患胸廓疾病,如肿瘤、感染、外伤等容易引起骨折的患儿。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "胸廓疾病" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肿瘤" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "感染" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "外伤" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "骨折" } ] }, { "text": "3.患有颅内压增高的疾病的患儿,CPT有进一步使颅内压增高的危险。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "CPT" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "颅内压增高" } ] }, { "text": "4.患毛细支气管炎和支气管哮喘等气道反应性增高的患儿,CPT容易引起气道痉挛。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "毛细支气管炎" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "支气管哮喘" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "气道" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "CPT" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "气道痉挛" } ] }, { "text": "5.一些疾病,如急性喉炎、急性会厌炎、大叶性或节段性肺实变、异物吸入等疗效不佳。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "急性喉炎" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "急性会厌炎" }, { "start_idx": 19, "end_idx": 28, "type": "dis", "entity": "大叶性或节段性肺实变" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "异物吸入" } ] }, { "text": "图8-14不同部位病变体位引流方法A.上叶尖段:患儿坐在床或引流台上,背靠枕头,后倾30度;B.上叶前段:患儿平卧在床或引流台上,下肢屈曲,膝关节下垫一枕头;C.上叶后段:患儿坐位,前倾30度,扒在枕头上;D.左上叶舌段:床呈15度倾斜,脚高头低,右侧卧位,略向后旋转1/4,膝关节略弯曲,背后可用枕头支撑;E.右中叶内段或外段:床呈15度倾斜,脚高头低,左侧卧位,略向后旋转1/4,膝关节略弯曲,背后可用枕头支撑;F.下叶背段:床平放,患儿俯卧位,并用枕头垫高臀部;G.下叶左或右前基底段:床呈30度倾斜,脚高头低,右肺病变左侧卧位,左肺病变右侧卧位,略向后旋转30度,膝关节下垫一枕头;H.下叶左或右外基底段:床呈30度倾斜,脚高头低,患儿呈俯卧位,向上旋转1/4,使病变部位朝上,上位下肢屈曲在枕头上,以作支撑;I.下叶左或右后基底段:床呈30度倾斜,脚高头低,患儿呈俯卧位图8-15胸部拍击法A.cupped手法;B.three-fingertenting手法", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "体位引流" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "上叶尖段" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "背" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "上叶前段" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "下肢" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "膝关节" }, { "start_idx": 81, "end_idx": 84, "type": "bod", "entity": "上叶后段" }, { "start_idx": 105, "end_idx": 109, "type": "bod", "entity": "左上叶舌段" }, { "start_idx": 119, "end_idx": 119, "type": "bod", "entity": "脚" }, { "start_idx": 138, "end_idx": 140, "type": "bod", "entity": "膝关节" }, { "start_idx": 145, "end_idx": 145, "type": "bod", "entity": "背" }, { "start_idx": 156, "end_idx": 163, "type": "bod", "entity": "右中叶内段或外段" }, { "start_idx": 173, "end_idx": 173, "type": "bod", "entity": "脚" }, { "start_idx": 175, "end_idx": 175, "type": "bod", "entity": "头" }, { "start_idx": 192, "end_idx": 194, "type": "bod", "entity": "膝关节" }, { "start_idx": 199, "end_idx": 199, "type": "bod", "entity": "背" }, { "start_idx": 210, "end_idx": 213, "type": "bod", "entity": "下叶背段" }, { "start_idx": 231, "end_idx": 232, "type": "bod", "entity": "臀部" }, { "start_idx": 236, "end_idx": 244, "type": "bod", "entity": "下叶左或右前基底段" }, { "start_idx": 254, "end_idx": 254, "type": "bod", "entity": "脚" }, { "start_idx": 259, "end_idx": 260, "type": "bod", "entity": "右肺" }, { "start_idx": 268, "end_idx": 269, "type": "bod", "entity": "左肺" }, { "start_idx": 286, "end_idx": 288, "type": "bod", "entity": "膝关节" }, { "start_idx": 297, "end_idx": 305, "type": "bod", "entity": "下叶左或右外基底段" }, { "start_idx": 315, "end_idx": 315, "type": "bod", "entity": "脚" }, { "start_idx": 317, "end_idx": 317, "type": "bod", "entity": "头" }, { "start_idx": 343, "end_idx": 346, "type": "bod", "entity": "上位下肢" }, { "start_idx": 361, "end_idx": 369, "type": "bod", "entity": "下叶左或右后基底段" }, { "start_idx": 379, "end_idx": 379, "type": "bod", "entity": "脚" }, { "start_idx": 382, "end_idx": 382, "type": "bod", "entity": "低" }, { "start_idx": 395, "end_idx": 399, "type": "pro", "entity": "胸部拍击法" } ] }, { "text": "附:外部性脑积水外部性脑积水(externalhydrocephalus,EH)是发生在婴儿期的一种良性、自愈性疾病。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "外部性脑积水" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "外部性脑积水" }, { "start_idx": 15, "end_idx": 35, "type": "dis", "entity": "externalhydrocephalus" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "EH" } ] }, { "text": "随着神经影像学的发展,临床发现有些头颅较大的婴儿,行头颅CT和MRI检查显示双侧额部或额顶部有蛛网膜下腔增宽,没有或仅有轻度脑室扩大,在2~3岁以后扩大的蛛网膜下腔又慢慢自行消失,这种现象被称为EH,由Dandy于1917年首先提出。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "头颅" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "头颅CT" }, { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "MRI" }, { "start_idx": 97, "end_idx": 98, "type": "dis", "entity": "EH" } ] }, { "text": "又称为良性蛛网膜下腔扩大、婴儿良性硬脑膜下积液脑室外梗阻性脑积水脑外积水等,属于假性脑积水。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "硬脑膜" }, { "start_idx": 13, "end_idx": 22, "type": "sym", "entity": "婴儿良性硬脑膜下积液" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "脑积水" }, { "start_idx": 23, "end_idx": 31, "type": "sym", "entity": "脑室外梗阻性脑积水" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "脑外积水" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "假性脑积水" } ] }, { "text": "原发性EH系指找不出明确原因的EH。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "EH" } ] }, { "text": "EH的发病机制尚不清楚,多数学者认为与颅外静脉阻塞引起颅内静脉压力增高上矢状窦压力升高蛛网膜颗粒水平的脑脊液吸收障碍有关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "EH" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "颅外静脉" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "颅内静脉" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "颅内静脉压力增高" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "上矢状窦" }, { "start_idx": 35, "end_idx": 42, "type": "sym", "entity": "上矢状窦压力升高" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "蛛网膜" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "脑脊液" } ] }, { "text": "新近有研究认为EH可能是源于蛛网膜功能发育延迟EH可能为脑与颅骨发育不均衡原发性EH的头颅CT或MRI表现可能就是部分正常婴儿的发育现象,无特殊病理意义。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "EH" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "蛛网膜功能" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "蛛网膜功能发育延迟" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "EH" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "脑" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "颅骨" }, { "start_idx": 28, "end_idx": 36, "type": "sym", "entity": "脑与颅骨发育不均衡" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "原发性EH" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "头颅CT" }, { "start_idx": 48, "end_idx": 50, "type": "pro", "entity": "MRI" } ] }, { "text": "【临床表现】本病发病年龄在1~1.5岁前囟未闭合之前,多发生于6个月左右婴儿。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "前囟" } ] }, { "text": "头颅CT或MRI扫描的表现为对称性的,显示:①额和额顶区蛛网膜下腔增宽>5mm(正常<2.3mm),其他区域蛛网膜下腔不增宽或稍宽脑前部纵裂池及侧裂池增宽基底池主要是鞍上池扩大额顶区脑沟加深加宽脑室不大或轻度扩大EH主要依据短期内头围增大及特有的头颅CT或MRI表现而予以诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "头颅CT" }, { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "MRI" }, { "start_idx": 23, "end_idx": 32, "type": "bod", "entity": "额和额顶区蛛网膜下腔" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 23, "end_idx": 64, "type": "sym", "entity": "额和额顶区蛛网膜下腔增宽>5mm(正常<2.3mm),其他区域蛛网膜下腔不增宽或稍宽" }, { "start_idx": 65, "end_idx": 70, "type": "bod", "entity": "脑前部纵裂池" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "侧裂池" }, { "start_idx": 65, "end_idx": 76, "type": "sym", "entity": "脑前部纵裂池及侧裂池增宽" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "基底池" }, { "start_idx": 83, "end_idx": 85, "type": "bod", "entity": "鞍上池" }, { "start_idx": 77, "end_idx": 87, "type": "sym", "entity": "基底池主要是鞍上池扩大" }, { "start_idx": 88, "end_idx": 92, "type": "bod", "entity": "额顶区脑沟" }, { "start_idx": 88, "end_idx": 96, "type": "sym", "entity": "额顶区脑沟加深加宽" }, { "start_idx": 97, "end_idx": 98, "type": "bod", "entity": "脑室" }, { "start_idx": 97, "end_idx": 105, "type": "sym", "entity": "脑室不大或轻度扩大" }, { "start_idx": 106, "end_idx": 107, "type": "dis", "entity": "EH" }, { "start_idx": 123, "end_idx": 126, "type": "pro", "entity": "头颅CT" }, { "start_idx": 128, "end_idx": 130, "type": "pro", "entity": "MRI" } ] }, { "text": "参考标准为:①患儿在短期内(1~3个月)头围异常增大,部分出现抽搐发作或前囟隆起发育及智力正常;③头颅CT或MRI扫描显示双侧额和额顶区蛛网膜下腔对称性局限性增宽,伴或不伴脑室轻度扩大;④随访观察扩大的蛛网膜下腔可自行慢慢恢复正常。", "entities": [ { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "前囟" }, { "start_idx": 31, "end_idx": 39, "type": "sym", "entity": "抽搐发作或前囟隆起" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "发育及智力正常" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "头颅CT" }, { "start_idx": 54, "end_idx": 58, "type": "pro", "entity": "MRI扫描" }, { "start_idx": 101, "end_idx": 105, "type": "bod", "entity": "蛛网膜下腔" } ] }, { "text": "需与脑萎缩及硬脑膜下积液脑萎缩患儿头围不大或更小些,在头颅CT或MRI上,呈现整个大脑脑沟普遍加深变宽,有时小脑沟也加深,脑室扩大,大部分病例无脑前部纵裂池增宽脑纵裂池增宽时,整个纵裂池均宽而不局限于前部硬脑膜下积液多由于脑膜炎和外伤引起,头颅CT或MRI扫描显示硬脑膜下积液不伴有基底池扩大及前纵裂增宽,多伴有脑室受压,其扩大腔内侧缘较平滑及左右两侧多不对称EH为良性自限性疾病,绝大多数患儿不需任何内外科治疗,可待其自行消失。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑萎缩" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "硬脑膜" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "硬脑膜下积液" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "脑萎缩" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "头颅CT" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "MRI" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "大脑脑沟" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "小脑沟" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "脑室" }, { "start_idx": 41, "end_idx": 79, "type": "sym", "entity": "大脑脑沟普遍加深变宽,有时小脑沟也加深,脑室扩大,大部分病例无脑前部纵裂池增宽" }, { "start_idx": 80, "end_idx": 83, "type": "bod", "entity": "脑纵裂池" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "纵裂池" }, { "start_idx": 80, "end_idx": 101, "type": "sym", "entity": "脑纵裂池增宽时,整个纵裂池均宽而不局限于前部" }, { "start_idx": 102, "end_idx": 104, "type": "bod", "entity": "硬脑膜" }, { "start_idx": 111, "end_idx": 113, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 115, "end_idx": 116, "type": "dis", "entity": "外伤" }, { "start_idx": 120, "end_idx": 123, "type": "pro", "entity": "头颅CT" }, { "start_idx": 125, "end_idx": 129, "type": "pro", "entity": "MRI扫描" }, { "start_idx": 132, "end_idx": 134, "type": "bod", "entity": "硬脑膜" }, { "start_idx": 156, "end_idx": 157, "type": "bod", "entity": "脑室" }, { "start_idx": 132, "end_idx": 179, "type": "sym", "entity": "硬脑膜下积液不伴有基底池扩大及前纵裂增宽,多伴有脑室受压,其扩大腔内侧缘较平滑及左右两侧多不对称" }, { "start_idx": 180, "end_idx": 181, "type": "dis", "entity": "EH" }, { "start_idx": 201, "end_idx": 203, "type": "dep", "entity": "内外科" } ] }, { "text": "继发性EH的轻症病例亦可自愈,有原发病者要积极治疗原发病。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "继发性EH" } ] }, { "text": "六、造血干细胞移植的预处理接受异体或自体干细胞移植之前,受体需接受移植前治疗,称为预处理,预处理治疗主要达到两个目的:摧毁受体的免疫系统,以保证移植物植入,抑制移植物的免疫功能,控制移植后GVHD;清除受体内残留肿瘤细胞或带有疾病基因的骨髓细胞。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "造血干细胞移植" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "预处理" }, { "start_idx": 15, "end_idx": 24, "type": "pro", "entity": "异体或自体干细胞移植" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "移植" }, { "start_idx": 41, "end_idx": 43, "type": "pro", "entity": "预处理" }, { "start_idx": 45, "end_idx": 49, "type": "pro", "entity": "预处理治疗" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "免疫系统" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "移植物" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "移植物" }, { "start_idx": 91, "end_idx": 92, "type": "pro", "entity": "移植" }, { "start_idx": 94, "end_idx": 97, "type": "dis", "entity": "GVHD" }, { "start_idx": 106, "end_idx": 109, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 113, "end_idx": 116, "type": "bod", "entity": "疾病基因" }, { "start_idx": 118, "end_idx": 121, "type": "bod", "entity": "骨髓细胞" } ] }, { "text": "自体移植时预处理的目的主要是清除受体内残留肿瘤细胞。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "自体移植" }, { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "预处理" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "肿瘤细胞" } ] }, { "text": "预处理的手段包括超剂量化疗、全身放疗和免疫抑制药物应用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "预处理" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "超剂量化疗" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "全身放疗" }, { "start_idx": 19, "end_idx": 24, "type": "dru", "entity": "免疫抑制药物" } ] }, { "text": "各单位、各种疾病预处理方案不完全相同,因为全身放疗的远期毒副反应,人们尽可能避免这种预处理,但其在ALL中仍然显示能明显提高疗效,所以仍广泛应用于ALL中,除此之外清髓性预处理基本被白消安所替代。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "预处理" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "全身放疗" }, { "start_idx": 42, "end_idx": 44, "type": "pro", "entity": "预处理" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "ALL" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "ALL" }, { "start_idx": 82, "end_idx": 87, "type": "pro", "entity": "清髓性预处理" }, { "start_idx": 91, "end_idx": 93, "type": "dru", "entity": "白消安" } ] }, { "text": "第二节基因诊断遗传性疾病是由于患者基因的缺陷或者变异,造成体内相应蛋白质合成的数量或者质量的异常,以致不能执行正常的生理功能而出现的疾病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "基因诊断" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "基因" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "基因的缺陷或者变异" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "蛋白质" }, { "start_idx": 33, "end_idx": 47, "type": "sym", "entity": "蛋白质合成的数量或者质量的异常" }, { "start_idx": 51, "end_idx": 61, "type": "sym", "entity": "不能执行正常的生理功能" } ] }, { "text": "基因诊断是在基因水平对疾病作出病因诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "基因诊断" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "基因" } ] }, { "text": "通过DNA或者RNA抽提、基因体外扩增(PCR)、分子杂交以及DNA测序等技术,检测特定基因是否有改变,从基因水平阐明病因,从而诊断患者是否有某种遗传病。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "pro", "entity": "DNA或者RNA抽提" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "基因体外扩增" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "PCR" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "分子杂交" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "DNA测序" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "基因" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "基因" } ] }, { "text": "对胎儿进行DNA检测,明确是否患有遗传病,从而指导孕妇作出是否继续妊娠的选择。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "DNA检测" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "遗传病" } ] }, { "text": "一、基因突变基因突变是指由于基因序列上DNA碱基对的置换、插入、缺失及重复等引起的基因结构的变化。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "基因" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "基因突变" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "基因" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "基因突变" }, { "start_idx": 14, "end_idx": 24, "type": "bod", "entity": "基因序列上DNA碱基对" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "基因" }, { "start_idx": 14, "end_idx": 47, "type": "sym", "entity": "基因序列上DNA碱基对的置换、插入、缺失及重复等引起的基因结构的变化" } ] }, { "text": "基因突变可发生在生殖细胞,也可发生在体细胞。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "基因" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "生殖细胞" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "体细胞" } ] }, { "text": "基因突变在生物界中是普遍存在的,它是生物变异的主要原因,是生物进化的主要因素。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "基因" } ] }, { "text": "但多数突变对生物体是有害的,影响氨基酸的组成,影响蛋白质功能,从而导致疾病,严重者影响胚胎发育而发生早期流产。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "氨基酸" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "蛋白质" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "胚胎" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "早期流产" } ] }, { "text": "绝大多数的人类遗传病,例如人的苯丙酮尿症、白化病及地中海贫血等遗传病,都是突变性状,是由基因突变造成的,这些病对人类健康构成了严重威胁。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "遗传病" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "白化病" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "地中海贫血" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "基因" } ] }, { "text": "突变是生物变异与进化的源泉,它也能为生物体提供新的基因。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "基因" } ] }, { "text": "通常将基因序列上单个碱基或少数碱基改变称为点突变。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "基因" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "碱基" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "碱基" } ] }, { "text": "突变有以下几种类型:1.碱基置换(basesubstitution)突变由一个错误的碱基对替代一个正确的碱基对的突变称为碱基置换突变。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "碱基" }, { "start_idx": 12, "end_idx": 35, "type": "sym", "entity": "碱基置换(basesubstitution)突变" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "碱基对" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "碱基对" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "碱基" } ] }, { "text": "例如在DNA分子中的GC碱基对由CG或AT或TA所代替,AT碱基对由TA或GC或CG所代替。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "DNA分子" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "GC碱基对" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "CG" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "AT" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "TA" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "AT碱基对" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "TA" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "GC" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "CG" } ] }, { "text": "碱基替换过程只改变被替换碱基的那个密码子,也就是说每一次碱基替换只改变一个密码子,不会涉及其他的密码子。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "碱基" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "碱基" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "密码子" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "碱基" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "密码子" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "密码子" } ] }, { "text": "碱基置换所致的基因突变既可发生在基因编码区,又可见于基因调控区,是最常见的基因点突变。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "碱基" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "基因" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "基因编码区" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "基因调控区" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "基因点" } ] }, { "text": "2.基因丢失(deletion)指基因核苷酸序列中核苷酸的丢失,这种丢失少则一个碱基,多至全基因或至几十个kb片段大小。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "基因" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "基因丢失" }, { "start_idx": 17, "end_idx": 27, "type": "bod", "entity": "基因核苷酸序列中核苷酸" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "碱基" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "基因" } ] }, { "text": "大片段DNA丢失可造成基因编码产物合成障碍,导致蛋白质完全或部分缺失,严重影响其功能。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "大片段DNA" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "基因" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "蛋白质" } ] }, { "text": "3.基因插入(insertion)与缺失相反,为插入一段顺序,可插入一个碱基,甚至好几百个或者更多碱基对插入。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "基因" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "基因插入" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "碱基" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "碱基对" } ] }, { "text": "4.基因融合指两个不同的核苷酸序列相接,形成一个新的基因。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "基因" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "基因融合" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "核苷酸序列" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "基因" } ] }, { "text": "如同源染色体之间的不等交换,或者染色体异位,例如染色体9q上癌基因ABL异位到22qBCR,形成BCRABL融合蛋白,导致慢性粒细胞性白血病。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "同源染色体" }, { "start_idx": 24, "end_idx": 35, "type": "bod", "entity": "染色体9q上癌基因ABL" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "22qBCR" }, { "start_idx": 48, "end_idx": 57, "type": "bod", "entity": "BCRABL融合蛋白" }, { "start_idx": 61, "end_idx": 69, "type": "dis", "entity": "慢性粒细胞性白血病" } ] }, { "text": "5.动态突变(dynamicmutation)是指DNA中的碱基重复序列拷贝数发生扩增而导致的突变。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "动态突变" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "DNA" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "碱基" }, { "start_idx": 30, "end_idx": 48, "type": "sym", "entity": "碱基重复序列拷贝数发生扩增而导致的突变" } ] }, { "text": "在人类基因中,当动态突变发生在转录序列内或附近时,就有可能对基因转录或其表达产物产生影响。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "基因" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "基因" } ] }, { "text": "通常重复序列的拷贝数有一个范围,超过这个范围,重复序列将变得不稳定,从而表现出疾病症状或在染色体上表现出脆性位点。", "entities": [ { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "染色体" } ] }, { "text": "至今已发现10余种遗传性疾病与动态突变有关,其中属于CGG/CCG重复的有脆性X综合征,以及Fraxe综合征;属于CAG/CTG重复的有重症肌无力(DM)、X连锁的脊柱和延髓肌萎缩、脊髓与小脑运动失调-1型(SCA1)、舞蹈症、亨廷顿病、齿状核与苍白球萎缩症,以苍白球萎缩症do-Josephdisease(MJD)等。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "动态突变" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "CGG/CCG" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "CGG/CCG重复" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "脆性X综合征" }, { "start_idx": 46, "end_idx": 53, "type": "dis", "entity": "Fraxe综合征" }, { "start_idx": 57, "end_idx": 63, "type": "bod", "entity": "CAG/CTG" }, { "start_idx": 57, "end_idx": 65, "type": "sym", "entity": "CAG/CTG重复" }, { "start_idx": 68, "end_idx": 72, "type": "dis", "entity": "重症肌无力" }, { "start_idx": 74, "end_idx": 75, "type": "dis", "entity": "DM" }, { "start_idx": 78, "end_idx": 83, "type": "dis", "entity": "X连锁的脊柱" }, { "start_idx": 85, "end_idx": 89, "type": "dis", "entity": "延髓肌萎缩" }, { "start_idx": 91, "end_idx": 102, "type": "dis", "entity": "脊髓与小脑运动失调-1型" }, { "start_idx": 104, "end_idx": 107, "type": "dis", "entity": "SCA1" }, { "start_idx": 110, "end_idx": 112, "type": "dis", "entity": "舞蹈症" }, { "start_idx": 114, "end_idx": 117, "type": "dis", "entity": "亨廷顿病" }, { "start_idx": 119, "end_idx": 121, "type": "dis", "entity": "齿状核" }, { "start_idx": 123, "end_idx": 128, "type": "dis", "entity": "苍白球萎缩症" }, { "start_idx": 131, "end_idx": 136, "type": "dis", "entity": "苍白球萎缩症" } ] }, { "text": "根据基因结构的改变方式,基因突变可分为碱基置换突变和移码突变两种类型。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "基因" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "基因" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "基因突变" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "碱基" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "碱基置换突变" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "移码" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "移码突变" } ] }, { "text": "碱基置换除了可改变编码区的密码子外,若发生在外显子与内含子连接部位(基因内含子剪接供体和受体位点)附近的内含子部位,突变内含子导致mRNA剪切异常,使mRNA短缺,mRNA异常,启动子区域的基因点突变可干扰基因的表达调控,干扰编码产物蛋白质的合成启动及合成速率,严重影响蛋白质的合成量。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "碱基" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "碱基置换" }, { "start_idx": 9, "end_idx": 15, "type": "bod", "entity": "编码区的密码子" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "外显子" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "内含子" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "基因内含子" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "供体" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "受体" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "内含子" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "突变内含子" }, { "start_idx": 65, "end_idx": 72, "type": "sym", "entity": "mRNA剪切异常" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "mRNA" }, { "start_idx": 75, "end_idx": 80, "type": "sym", "entity": "mRNA短缺" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "mRNA" }, { "start_idx": 103, "end_idx": 104, "type": "bod", "entity": "基因" }, { "start_idx": 89, "end_idx": 109, "type": "sym", "entity": "启动子区域的基因点突变可干扰基因的表达调控" }, { "start_idx": 117, "end_idx": 119, "type": "bod", "entity": "蛋白质" }, { "start_idx": 111, "end_idx": 129, "type": "sym", "entity": "干扰编码产物蛋白质的合成启动及合成速率" }, { "start_idx": 135, "end_idx": 137, "type": "bod", "entity": "蛋白质" }, { "start_idx": 133, "end_idx": 141, "type": "sym", "entity": "影响蛋白质的合成量" } ] }, { "text": "移码突变(frameshiftmutation)指基因中插入或者缺失一个或几个碱基对,使DNA的阅读框架(读码框)发生改变,导致插入或缺失部位之后的所有密码子都跟着发生变化,结果产生一种异常的多肽链。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "移码" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "移码突变" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "基因" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "碱基对" }, { "start_idx": 25, "end_idx": 41, "type": "sym", "entity": "基因中插入或者缺失一个或几个碱基对" }, { "start_idx": 43, "end_idx": 60, "type": "sym", "entity": "使DNA的阅读框架(读码框)发生改变" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "密码子" }, { "start_idx": 64, "end_idx": 85, "type": "sym", "entity": "插入或缺失部位之后的所有密码子都跟着发生变化" }, { "start_idx": 96, "end_idx": 98, "type": "bod", "entity": "多肽链" }, { "start_idx": 89, "end_idx": 98, "type": "sym", "entity": "产生一种异常的多肽链" } ] }, { "text": "根据遗传信息的改变方式,基因突变又可以分为同义突变、错义突变和无义突变三种类型。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "基因" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "基因突变" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "同义突变" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "错义突变" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "无义突变" } ] }, { "text": "1.同义突变(synonymousmutation)DNA的一个碱基对的改变并不影响它所编码的蛋白质的氨基酸序列,这是因为改变后的密码子和改变前的密码子,它们编码同一种氨基酸。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "同义突变" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "DNA" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "碱基对" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "蛋白质" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "氨基酸序列" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "密码子" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "密码子" }, { "start_idx": 84, "end_idx": 86, "type": "bod", "entity": "氨基酸" } ] }, { "text": "但目前发现个别原先认为是同义突变,可能会导致转录功能异常。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "同义突变" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "转录功能异常" } ] }, { "text": "2.错义突变(missensemutation)由于一对或几对碱基对的改变而使决定某一氨基酸的密码子变为决定另一种氨基酸的密码子,引起氨基酸序列和空间构象改变,导致它所编码的蛋白质部分或完全失活,例如人血红蛋白β链的基因如果将决定第6位氨基酸(谷氨酸)的密码子由CTT变为CAT,就会使它合成出的β链多肽的第6位氨基酸由谷氨酸变为缬氨酸,从而引起镰刀形红细胞贫血病。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "错义突变" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "碱基对" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "氨基酸" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "密码子" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "氨基酸" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "密码子" }, { "start_idx": 26, "end_idx": 63, "type": "sym", "entity": "一对或几对碱基对的改变而使决定某一氨基酸的密码子变为决定另一种氨基酸的密码子" }, { "start_idx": 67, "end_idx": 71, "type": "bod", "entity": "氨基酸序列" }, { "start_idx": 65, "end_idx": 78, "type": "sym", "entity": "引起氨基酸序列和空间构象改变" }, { "start_idx": 87, "end_idx": 89, "type": "bod", "entity": "蛋白质" }, { "start_idx": 80, "end_idx": 96, "type": "sym", "entity": "导致它所编码的蛋白质部分或完全失活" }, { "start_idx": 101, "end_idx": 109, "type": "bod", "entity": "血红蛋白β链的基因" }, { "start_idx": 115, "end_idx": 120, "type": "bod", "entity": "第6位氨基酸" }, { "start_idx": 122, "end_idx": 124, "type": "bod", "entity": "谷氨酸" }, { "start_idx": 127, "end_idx": 129, "type": "bod", "entity": "密码子" }, { "start_idx": 131, "end_idx": 133, "type": "bod", "entity": "CTT" }, { "start_idx": 136, "end_idx": 138, "type": "bod", "entity": "CAT" }, { "start_idx": 148, "end_idx": 151, "type": "bod", "entity": "β链多肽" }, { "start_idx": 153, "end_idx": 158, "type": "bod", "entity": "第6位氨基酸" }, { "start_idx": 160, "end_idx": 162, "type": "bod", "entity": "谷氨酸" }, { "start_idx": 165, "end_idx": 167, "type": "bod", "entity": "缬氨酸" }, { "start_idx": 173, "end_idx": 181, "type": "dis", "entity": "镰刀形红细胞贫血病" } ] }, { "text": "3.无义突变(nonsensemutation)如果某一碱基被另一碱基替代后,决定某一氨基酸的密码子变成一个终止密码子,使翻译过程提前终止,合成的肽链变短,此终止密码子称为无义突变。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "碱基" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "碱基" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "某一碱基被另一碱基替代" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "氨基酸" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "密码子" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "终止密码子" }, { "start_idx": 41, "end_idx": 58, "type": "sym", "entity": "某一氨基酸的密码子变成一个终止密码子" }, { "start_idx": 61, "end_idx": 68, "type": "sym", "entity": "翻译过程提前终止" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "肽链" }, { "start_idx": 70, "end_idx": 76, "type": "sym", "entity": "合成的肽链变短" }, { "start_idx": 79, "end_idx": 83, "type": "bod", "entity": "终止密码子" }, { "start_idx": 86, "end_idx": 89, "type": "sym", "entity": "无义突变" } ] }, { "text": "参考文献1.胡亚美,江载芳.诸福棠实用儿科学.第7版.北京:人民卫生出版社,2002:1562-15622.Nationalhighbloodpressureeducationprogramworkinggrouponhighbloodpressureinchildrenandadolescents.Thefourthreportonthediagnosis,evaluation,andtreatmentofhighbloodpressureinchildrenandadolescents.Pediatrics,2004,114:555-5553.刘冲,杜忠东,李霞,等.住院儿童高血压的病因分析及鉴别诊断.首都医科大学学报,2010,31:187-1874.胡云南,易岂建.儿童高血压的诊治进展.儿科药学杂志,2010,16:57-605.MitsnefesMM.Hypertensioninchildrenandadolescents.PediatrCliNAm,2006,53:493-4936.FlyninJT,etal.Pharmacologictreatmentofhypertensioninchildrenandadolescents.JPediatr,2006,149:746-7467.FivushB,etal.Acutehypertensivecrisesinchildren:emergenciesandurgencies.CurrOpinPediar,1997,9:233-2338.KayJD,etal.Pediatrichypertension.AmHeartJ,2001,142:422-4329.齐建光,杜军保.儿童高血压及高血压危象的治疗.中国社区医师,2006,18:10-1010.中国高血压防治指南修订委员会.2004年中国高血压防治指南(实用本).中华心血管病杂志,2004,32:1060-1060", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "dep", "entity": "儿科" }, { "start_idx": 291, "end_idx": 295, "type": "dis", "entity": "儿童高血压" }, { "start_idx": 341, "end_idx": 345, "type": "dis", "entity": "儿童高血压" }, { "start_idx": 352, "end_idx": 353, "type": "dep", "entity": "儿科" }, { "start_idx": 726, "end_idx": 730, "type": "dis", "entity": "儿童高血压" }, { "start_idx": 732, "end_idx": 736, "type": "dis", "entity": "高血压危象" }, { "start_idx": 766, "end_idx": 768, "type": "dis", "entity": "高血压" }, { "start_idx": 786, "end_idx": 788, "type": "dis", "entity": "高血压" }, { "start_idx": 801, "end_idx": 804, "type": "dis", "entity": "心血管病" } ] }, { "text": "七、腮腺炎病毒肺炎腮腺炎病毒肺炎(mumpspneumonia)常因其呼吸道症状不明显,易为腮腺肿大及其并发症所掩盖,以及极少进行X线肺部检查而漏诊。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "腮腺炎病毒肺炎" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "腮腺炎病毒肺炎" }, { "start_idx": 17, "end_idx": 30, "type": "dis", "entity": "mumpspneumonia" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "呼吸道" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "腮腺肿大" }, { "start_idx": 65, "end_idx": 70, "type": "pro", "entity": "X线肺部检查" } ] }, { "text": "临床表现大多较轻,一般无呼吸困难和发绀。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "无呼吸困难和发绀" } ] }, { "text": "肺部呈局限性呼吸音粗糙,少数可闻水泡音。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "局限性呼吸音粗糙" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "闻水泡音" } ] }, { "text": "外周血白细胞计数多不升高。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "外周血白细胞计数" } ] }, { "text": "X线表现肺野斑片状或大片状阴影,或呈毛玻璃样改变。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "X线" } ] }, { "text": "根据典型腮腺炎表现,加上述X线改变,可考虑本病。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "X线" } ] }, { "text": "三、脂肪肉瘤脂肪肉瘤(liposarcoma)起源于原始间叶细胞,成人多见,小儿少见,多发于10~15岁,男女无差异。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "脂肪肉瘤" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "脂肪肉瘤" }, { "start_idx": 11, "end_idx": 21, "type": "dis", "entity": "liposarcoma" }, { "start_idx": 26, "end_idx": 31, "type": "bod", "entity": "原始间叶细胞" } ] }, { "text": "脂肪肉瘤分五种组织类型:分化良好型、黏液样型、圆细胞型、多形型和混合型。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "脂肪肉瘤" } ] }, { "text": "脂肪肉瘤来自脂肪组织,故在全身各部位生长,最多发生于腹膜后,另为股部和膝部,肿瘤生长隐匿、缓慢,无明显界限,无特殊临床表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "脂肪肉瘤" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "脂肪组织" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "腹膜后" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "股部" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "膝部" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "肿瘤" } ] }, { "text": "治疗:手术完全切除是最好的治疗方法,放疗效果不确切,化疗只在手术无效或无法实施时才采用,常用药物为环磷酰胺、长春新碱、放线菌素D,但其效果也不明确。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "手术" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "放疗" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "化疗" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "手术" }, { "start_idx": 49, "end_idx": 52, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "长春新碱" }, { "start_idx": 59, "end_idx": 63, "type": "dru", "entity": "放线菌素D" } ] }, { "text": "二、出生后感染性肺炎【临床流行病学】出生后感染性肺炎发生率较高,常见的病原体有:金黄色葡萄球菌、大肠埃希菌、克雷伯杆菌、假单胞菌等细菌,呼吸道合胞病毒、腺病毒等病毒,以及卡氏肺囊虫、解脲支原体等。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "出生后感染性肺炎" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "出生后感染性肺炎" }, { "start_idx": 40, "end_idx": 46, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 48, "end_idx": 52, "type": "mic", "entity": "大肠埃希菌" }, { "start_idx": 54, "end_idx": 58, "type": "mic", "entity": "克雷伯杆菌" }, { "start_idx": 60, "end_idx": 63, "type": "mic", "entity": "假单胞菌" }, { "start_idx": 65, "end_idx": 66, "type": "mic", "entity": "细菌" }, { "start_idx": 68, "end_idx": 74, "type": "mic", "entity": "呼吸道合胞病毒" }, { "start_idx": 76, "end_idx": 78, "type": "mic", "entity": "腺病毒" }, { "start_idx": 80, "end_idx": 81, "type": "mic", "entity": "病毒" }, { "start_idx": 85, "end_idx": 89, "type": "mic", "entity": "卡氏肺囊虫" }, { "start_idx": 91, "end_idx": 95, "type": "mic", "entity": "解脲支原体" } ] }, { "text": "【病因机制和病理】出生后感染性肺炎的来源有:(一)接触传播接触新生儿者如患呼吸道感染,其病原体可经飞沫由上呼吸道向下传播至肺。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "出生后感染性肺炎" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 44, "end_idx": 46, "type": "mic", "entity": "病原体" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "呼吸道" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "肺" } ] }, { "text": "(二)血行传播脐炎、皮肤感染引起败血症时,病原体经血行传播至肺而引起肺炎。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "脐炎" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "皮肤感染" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "败血症" }, { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "病原体" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "肺" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "肺炎" } ] }, { "text": "(三)医源性传播由于医用器械(如吸痰器、雾化器、供氧面罩、气管插管等)消毒不严,暖箱湿度过高使水生菌易于繁殖,或使用呼吸机时间过长等引起肺炎;医护人员洗手不勤,将患儿的致病菌带给其他新生儿;广谱抗生素使用过久容易发生真菌性肺等。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "equ", "entity": "吸痰器" }, { "start_idx": 20, "end_idx": 22, "type": "equ", "entity": "雾化器" }, { "start_idx": 24, "end_idx": 27, "type": "equ", "entity": "供氧面罩" }, { "start_idx": 29, "end_idx": 32, "type": "equ", "entity": "气管插管" }, { "start_idx": 40, "end_idx": 41, "type": "equ", "entity": "暖箱" }, { "start_idx": 47, "end_idx": 49, "type": "mic", "entity": "水生菌" }, { "start_idx": 58, "end_idx": 60, "type": "equ", "entity": "呼吸机" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "肺炎" }, { "start_idx": 95, "end_idx": 99, "type": "dru", "entity": "广谱抗生素" }, { "start_idx": 108, "end_idx": 111, "type": "dis", "entity": "真菌性肺" } ] }, { "text": "镜检各病灶存在不同阶段的炎性反应。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "镜检" } ] }, { "text": "【临床表现】起病前有时有上呼吸道感染的症状,患儿常出现呼吸急促、呻吟、鼻扇、口吐白沫、发绀、发热或体温不升等,吸气时胸廓有三凹征,肺部体征有细湿啰音等。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "呻吟" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "鼻扇" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "口吐白沫" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "发绀" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "发热" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "体温不升" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "胸廓" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "肺部" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "细湿啰音" } ] }, { "text": "呼吸道合胞病毒性肺炎可表现为喘憋、咳嗽,肺部闻及哮鸣音。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "呼吸道合胞病毒性肺炎" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "喘憋" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "咳嗽" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肺部" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "肺部闻及哮鸣音" } ] }, { "text": "咽部分泌物等进行培养等检测,有助于病原学诊断。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "咽部" } ] }, { "text": "【治疗】(一)加强护理和监护注意保暖,使患儿皮温达36.5℃,环境湿度在50%以上。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "注意保暖" }, { "start_idx": 22, "end_idx": 29, "type": "pro", "entity": "皮温达36.5℃" }, { "start_idx": 31, "end_idx": 40, "type": "pro", "entity": "环境湿度在50%以上" } ] }, { "text": "吸净口咽、鼻部分泌物,保持呼吸道通畅、定期翻身拍背有利于痰液排出。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "口咽" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "鼻部" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "保持呼吸道通畅" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "定期翻身拍背" } ] }, { "text": "(二)供氧有低氧血症时可根据病情供氧,维持血氧在6.65~10.7kPa(50~80mmHg),不超过16.0kPa(120mmHg),以防氧中毒。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "供氧" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "低氧血症" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "氧中毒" } ] }, { "text": "(三)抗病原体治疗细菌性肺炎以早期静脉给予抗生素为宜,原则上根据病原菌选用抗生素,如金黄色葡萄球菌可用耐酶青霉素、第一代头孢菌素或阿米卡星;G-阴性菌可用第三代头孢菌素。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "静脉" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "抗生素" }, { "start_idx": 32, "end_idx": 34, "type": "mic", "entity": "病原菌" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "抗生素" }, { "start_idx": 42, "end_idx": 48, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 51, "end_idx": 55, "type": "dru", "entity": "耐酶青霉素" }, { "start_idx": 57, "end_idx": 63, "type": "dru", "entity": "第一代头孢菌素" }, { "start_idx": 65, "end_idx": 68, "type": "dru", "entity": "阿米卡星" }, { "start_idx": 83, "end_idx": 85, "type": "mic", "entity": "阴性菌" }, { "start_idx": 91, "end_idx": 94, "type": "dru", "entity": "头孢菌素" } ] }, { "text": "(四)支持疗法维持水、电解质平衡;输新鲜血或血浆:每次10ml/kg,根据病情可少量多次应用;丙种球蛋白增加免疫功能对某些肺炎有一定疗效,500mg/(kg•d),可用3~5天。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "支持疗法" }, { "start_idx": 7, "end_idx": 15, "type": "pro", "entity": "维持水、电解质平衡" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "输新鲜血或血浆" }, { "start_idx": 61, "end_idx": 62, "type": "dis", "entity": "肺炎" } ] }, { "text": "参考文献1.吴希如,林庆,主编.小儿神经系统疾病基础与临床(第2版).北京:人民卫生出版社,20092.林庆.婴幼儿发育医学.北京:人民卫生出版社,19853.PilzD,StoodleyN,GoldenJA.Neuronalmigration,cerebralcorticaldevelopment,andcerebralcorticalanomalies.JNeuropatholExpNeurol.2002,61(1):1-14.SowellER,TraunerDA,GamstA,etal.Developmentofcorticalandsubcorticalbrainstructuresinchildhoodandadolescence:astructuralMRIstudy.DevMedChildNeurol.2002,44(1):4-45.PraysonRA,SpreaficoR,VintersHV.Pathologiccharacteristicsofthecorticaldysplasias.NeurosurgClinNAm.2002,13(1):17-17", "entities": [ { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "小儿神经系统疾病" }, { "start_idx": 27, "end_idx": 28, "type": "dep", "entity": "临床" } ] }, { "text": "四、Kartagener综合征本病1933年由Kartagener首次报告,包括支气管扩张、鼻窦炎或鼻息肉、内脏转位(主要为右位心)。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "Kartagener综合征" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "支气管扩张" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "鼻息肉" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "内脏转位" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "右位心" } ] }, { "text": "病因尚未肯定,可能与遗传及原发性纤毛动力障碍(primaryciliadyskinesia,PCD)即纤毛不动综合征(immotileciliasyndrome)有关。", "entities": [ { "start_idx": 10, "end_idx": 21, "type": "dis", "entity": "遗传及原发性纤毛动力障碍" }, { "start_idx": 23, "end_idx": 44, "type": "dis", "entity": "primaryciliadyskinesia" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "PCD" }, { "start_idx": 51, "end_idx": 57, "type": "dis", "entity": "纤毛不动综合征" }, { "start_idx": 59, "end_idx": 79, "type": "dis", "entity": "immotileciliasyndrome" } ] }, { "text": "50%PCD患儿伴发本病。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "PCD" } ] }, { "text": "由于纤毛轴丝臂缺乏,引起纤毛活动能力丧失,黏液纤毛运输功能障碍,引起分泌物和细菌的潴留,导致持续感染,日久即演变为支气管扩张和鼻窦炎,亦可表现广泛性毛细支气管炎。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "纤毛轴丝臂" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "纤毛" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "纤毛" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "分泌物" }, { "start_idx": 38, "end_idx": 39, "type": "mic", "entity": "细菌" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "感染" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "支气管扩张" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 71, "end_idx": 79, "type": "dis", "entity": "广泛性毛细支气管炎" } ] }, { "text": "心脏及胃泡在右侧,肝浊音区在左侧。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心脏" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "胃泡" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肝" } ] }, { "text": "常合并先天性心脏病、脑积水、肛门闭锁、尿道下裂、重复肾等畸形。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "脑积水" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "肛门闭锁" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "重复肾" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "畸形" } ] }, { "text": "支气管造影显示支气管呈柱状或囊状扩张。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "支气管造影" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "支气管" }, { "start_idx": 7, "end_idx": 17, "type": "sym", "entity": "支气管呈柱状或囊状扩张" } ] }, { "text": "支气管黏膜活检组织电子显微镜下可观察到纤毛超微结构缺陷或有纤毛功能异常的证据。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "支气管黏膜活检" }, { "start_idx": 9, "end_idx": 13, "type": "equ", "entity": "电子显微镜" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "纤毛" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "纤毛超微结构缺陷" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "纤毛" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "纤毛功能异常" } ] }, { "text": "如有鼻窦炎或下呼吸道感染,应积极给予抗生素治疗。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "下呼吸道感染" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "抗生素" } ] }, { "text": "对局灶性支气管扩张伴反复感染、咯血等严重症状,且不易控制者可考虑手术切除。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "支气管" }, { "start_idx": 1, "end_idx": 8, "type": "sym", "entity": "局灶性支气管扩张" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "感染" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "咯血" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "手术" } ] }, { "text": "第三节肢带型肌营养不良最初的肢带型肌营养不良(limb-girdlemusculardystrophy,LGMD)的分型由Walton及Nattrass于1954年确定。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "肢带型肌营养不良" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "肢带型肌营养不良" }, { "start_idx": 23, "end_idx": 50, "type": "dis", "entity": "limb-girdlemusculardystrophy" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "LGMD" } ] }, { "text": "表16-14LGMDS分类【临床表现】所有类型患者都表现为肢带肌无力,而面肌、眼外肌及咽肌不受累肌无力的程度个体差异很大。", "entities": [ { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "肢带肌无力" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "眼外肌" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "咽肌" }, { "start_idx": 35, "end_idx": 47, "type": "sym", "entity": "而面肌、眼外肌及咽肌不受累" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "肌无力" } ] }, { "text": "据报道Calpain-3缺乏患者(LGMD2A)有腓肠肌挛缩,造成足趾行走。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "Calpain-3缺乏" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "LGMD2" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "腓肠肌挛缩" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "造成足趾行走" } ] }, { "text": "在某些家系中,受累者可有近端肌群或远端肌群受累的表现智能往往正常。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "远端肌群" }, { "start_idx": 12, "end_idx": 25, "type": "sym", "entity": "近端肌群或远端肌群受累的表现" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "智能往往正常" } ] }, { "text": "LGMD1B可合并心肌病,62.5%患者在50岁左右出现心脏传导系统紊乱,并造成心动过缓及晕厥,需要安装心脏起搏器,也可发生猝死。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "LGMD1" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "心肌病" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "心脏传导系统紊乱" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "心动过缓及晕厥" }, { "start_idx": 52, "end_idx": 56, "type": "equ", "entity": "心脏起搏器" }, { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "也可发生猝死" } ] }, { "text": "【诊断】(一)临床表现肌无力与阳性家族史。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肌无力" } ] }, { "text": "(二)实验室检查1.血清CK血清CK可增高,常染色体隐性遗传型LGMD患者比显性遗传型增高更明显,但由于有重叠现象,因此不可能依靠CK水平作出诊断。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "血清CK" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "血清CK" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "LGMD" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "CK" } ] }, { "text": "2.肌电图及肌肉活体组织检查肌电图示肌源性损伤,肌肉活体组织检查为非特异性肌源性改变。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肌电图" }, { "start_idx": 6, "end_idx": 13, "type": "pro", "entity": "肌肉活体组织检查" }, { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "肌电图" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "肌源性损伤" }, { "start_idx": 24, "end_idx": 31, "type": "pro", "entity": "肌肉活体组织检查" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "非特异性肌源性改变" } ] }, { "text": "3.其他检查如通过组织染色,以抗体检测肌聚糖复合物的成分,但缺乏特异性。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "抗体检测" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "肌聚糖复合物" } ] }, { "text": "4.基因诊断LGMDs或为常染色体显性遗传(LGMD1A、1B和1C),或为常染色体隐性遗传(LGMD2A~H)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "基因诊断" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "LGMDs" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "LGMD1A" }, { "start_idx": 47, "end_idx": 54, "type": "dis", "entity": "LGMD2A~H" } ] }, { "text": "对α-肌聚糖缺陷型研究较为深入,已确认了近40种不同的α-肌聚糖基因突变,大多数定位于细胞外区域,特别是在3号外显子,发现了12种不同的基因突变,Arg77Cys最为多见。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "α-肌聚糖缺陷型" }, { "start_idx": 27, "end_idx": 33, "type": "bod", "entity": "α-肌聚糖基因" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "细胞" } ] }, { "text": "在β-肌聚糖缺陷型中,大多数被确认的基因突变发生于细胞外的外显子3和4。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "β-肌聚糖缺陷型" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "细胞" } ] }, { "text": "γ-肌聚糖缺陷型中确定的基因突变则较少,而δ-肌聚糖缺陷型只有2种基因突变,也位于细胞外。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "γ-肌聚糖缺陷型" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "δ-肌聚糖缺陷型" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "细胞" } ] }, { "text": "【治疗】各种维持功能的治疗措施均对LGMD患者有利,伸展训练可减轻挛缩,支架及脊柱侧弯手术均可适用,指征同DMD患者。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "LGMD" }, { "start_idx": 36, "end_idx": 37, "type": "equ", "entity": "支架" }, { "start_idx": 39, "end_idx": 44, "type": "pro", "entity": "脊柱侧弯手术" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "DMD" } ] }, { "text": "同时,rAAV对宿主免疫系统无显著刺激。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "rAAV" } ] }, { "text": "参考文献1.FalkRJ,JennetteJC,NachmanPH.PrimaryGlomerulardisease.In:BrennerBM.BrennerandRector’stheKidney.1sted,NY,HarcourtPublisherslimited,1999,1263-12632.TejaniA,IngulliE.Poststrptococcalglomerulonephritis.Currentclinicalandpathologicconcepts.Nephron,1990,55:13.KrausW,OhyamaaK,SynderS,etal.AutoimmunesequenceofstreptococcalMproteinsharedwiththeintermediatefilamentprotein,vimentin.JExpMed,1989,169:4814.CroninWJ,LangerK.Immunologicevidencefortheinsitudepositionofacytoplasmicstreptococcalantigen(endostreptosin)ontheglomerularbasementmembraneinrats.ClinNephrol,1990,34:1435.CouserWG.Rapidlyprogressiveglomerulonephritis:classification,pathogeneticmechanismsandtherapy.AmJKidneyDis,1988,11:4496.BonsibSM.Glomerularbasementmembranenecrosisandcrescentorganization.KidneyInt,1988,33:9667.JardimHM,LeakeJ,RisdonRA,etal.Crescenticglomerulonephritisinchildren.PediatrNephrol,1992,6:2318.HellmarkT,JohanssonC,WieslanderJ.Characterizationofanti-GBMantibodiesinvolvedinGoodpasture’ssyndrome.KidneyInt.1994,46:8239.BruceMT,MendozaSA.Treatmentoftheidiopathicnephroticsyndrome:Regimensandoutcomesinchildrenandadults.JASN,1997,8:82510.BogenschiitzO,BohleA,WehrmannM,etal.IgAnephritis:Ontheimportanceofmorphologicalandclinicalparametersinthelongtermprognosisof239patients.AmJNephrol,1990,10:13711.IijmaK,ItoS,YoshikawaN.Multiplecombinedtherapyforseverehenoch-Schonleinnephritisinchildren.PediatrNephrol,1998,12:24412.LinCY.HepatitisBvirusassociatedmembraneousnephropathy:clinicalfeatures,immunologicalprofileandoutcome.Nephron,1990,55:3713.WhiteRHR,YoshiRawaN,FeehallyJ.IgANephoopathyandHenoch-Schonleinnepritis.In:PediatricNephrologyeditedbyBarrattTM,AvnerED,HarmanWE.4thedition.Baltimore,William&Wilkins,1998,691-69114.KnightJF.Therheumaticpoison:AsurveyofsomepublishedinvestigationsoftheimmunopathogenesisofHenoch-Schonleinpurpura.PediatrNephrol,1990,4:53315.DavinJC,WeeningJJ.Bergerdisease:Thirtyyearslater.EurJPediatr,1999,158:43716.KumadoK,SuzukiJ,KumeK,etal.ClinicopathologicalstudyofHenoch-SchonleinpurpuranephritiswithspecialreferencetoC3cdeposits.NipponJinzoGakkaiShi,1996,38:25917.EndoM,OhiH,OhsawaI,etal.Glomerulardepositionofmannose-bindinglectin(MBL)indicatesanovelmechanismofcomplementactivationinIgAnephropathy.NephrolDialTransplant,1998,13:198418.D’AmicoG.ImmunoglobulinAnephropathy.In:ThePrinciplesandPracticeofNephrology,editedbyJacobsonHR,StrikerGE,KlahrS.2ndedition,StLouis,Mosby-YearBookInc,1996,13319.AbeJ,KohsakaT,TanakaM,etal.GeneticstudyonHLAclassⅡandclassⅢregioninthediseaseassociatedwithIgAnepbropathy.Nephron,1993,65:1720.KoshikawaN,ItoH,NakamuraH.IgAnephropathyinchildrenfromJapan.ChildNephrolUrol,1989,9:19121.CoppoR,MazzucoG,CagnoliL,etal.Long-termprognosisofHenoch-Schonleinnephritisinadultsandchildren.NephrolDialTransplant,1997,12:227722.JohnsonRJ,CouserWG.HepatitisBinfectionandrenaldisease:Clinical,immunopathogeneticandtherapeuticconsiderations.KidneyInt,1990,37:66323.LinCY.TreatmentofhepatitisBvirus-relatedmembraneousnephropathywithrecombinantalphaa-interferon.KidneyInt,1995,47:22524.杨锡强主编.儿童免疫学.北京:人民卫生出版社,2001:59125.李永柏,杨锡强,沈锦.环磷酰胺冲击治疗儿童系统性红斑狼疮.实用儿科临床杂志,1994,9(6):321-32126.李永柏,沈锦,张恒言等.环磷酰胺两种用药方案治疗肾病综合征的对比研究.中华儿科杂志,1993,31(4):215-21527.易著文主编.小儿临床肾脏病学.北京:人民卫生出版社,1998,333", "entities": [ { "start_idx": 3060, "end_idx": 3063, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 3068, "end_idx": 3076, "type": "dis", "entity": "儿童系统性红斑狼疮" }, { "start_idx": 3119, "end_idx": 3122, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 3131, "end_idx": 3135, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 3180, "end_idx": 3182, "type": "dis", "entity": "肾脏病" } ] }, { "text": "第四节新生儿疾病筛查新生儿疾病筛查(neonatalscreening)是指医疗保健机构在新生儿群体中,用快速、简便、敏感的检验方法,对一些危及儿童生命、危害儿童生长发育、导致儿童智能障碍的一些先天性疾病和遗传性疾病进行群体筛检,从而使患儿在临床上未出现疾病表现,而其体内生化、激素水平已有明显变化时就做出早期诊断,结合有效治疗,避免患儿重要脏器出现不可逆的损害,保障儿童正常的体格发育和智能发育的系统服务。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "新生儿疾病筛查" }, { "start_idx": 38, "end_idx": 43, "type": "dep", "entity": "医疗保健机构" }, { "start_idx": 97, "end_idx": 101, "type": "dis", "entity": "先天性疾病" }, { "start_idx": 103, "end_idx": 107, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 136, "end_idx": 142, "type": "ite", "entity": "生化、激素水平" } ] }, { "text": "新生儿疾病筛查是预防医学的一项重要措施,目前已在世界范围内推广,成为人类卫生保健的重要内容之一。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "新生儿疾病筛查" } ] }, { "text": "经过40年的发展,新生儿疾病筛查的疾病病种逐步增多,由最初苯丙酮尿症一种增加到数十种,新生儿疾病筛查的概念被普遍认可,新生儿疾病筛查逐步由发达国家向发展中国家普及。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "新生儿疾病筛查" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 43, "end_idx": 49, "type": "pro", "entity": "新生儿疾病筛查" }, { "start_idx": 59, "end_idx": 65, "type": "pro", "entity": "新生儿疾病筛查" } ] }, { "text": "我国自1981年开始进行新生儿疾病筛查,目前正在由大城市向中小城市推广,由经济较发达的沿海地区向内地发展。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "新生儿疾病筛查" } ] }, { "text": "一、国际新生儿疾病筛查的历史自1934年挪威生化学家Folling首次报道了苯丙酮尿症(PKU)这种疾病以来,世界各国科学家对PKU进行了大量的研究。", "entities": [ { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "PKU" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "PKU" } ] }, { "text": "1953年德国Bickel医生首创使用饮食疗法治疗苯丙酮尿症获得成功,并提出早期诊断及早期治疗的重要性,由此设想把患儿尽早从正常人群中筛选出来,新生儿疾病筛查的概念因而形成。", "entities": [ { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 72, "end_idx": 78, "type": "pro", "entity": "新生儿疾病筛查" } ] }, { "text": "但是在当时条件下,实验诊断PKU的手段只有三氯化铁试验,其准确性和实用性都不适合新生儿群体普查。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "PKU" } ] }, { "text": "1961年美国Guthrie医生建立了细菌抑制法对血中苯丙氨酸中苯丙氨酸进行半定量测定,尤其是创立了干血滤纸片血样采集法,血片采集简便,标本运送方便,为开展大规模人群筛查提供了基本条件,苯丙酮尿症的新生儿筛查开始得以实施。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "细菌抑制法" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "血中苯丙氨酸" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "苯丙氨酸" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "半定量测定" }, { "start_idx": 50, "end_idx": 59, "type": "pro", "entity": "干血滤纸片血样采集法" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "血片采集" }, { "start_idx": 93, "end_idx": 97, "type": "dis", "entity": "苯丙酮尿症" } ] }, { "text": "先天性甲状腺机能减低症(CH)的新生儿筛查首先从美国Pittsburgh用脐血测定促甲状腺激素(TSH)开始,1973年Dussault等用干血滤纸片放射免疫方法测新生儿末梢血T4天的新生儿末梢血T4进行CH筛查。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "dis", "entity": "先天性甲状腺机能减低症" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "CH" }, { "start_idx": 37, "end_idx": 46, "type": "pro", "entity": "脐血测定促甲状腺激素" }, { "start_idx": 48, "end_idx": 50, "type": "pro", "entity": "TSH" }, { "start_idx": 70, "end_idx": 74, "type": "equ", "entity": "干血滤纸片" }, { "start_idx": 75, "end_idx": 80, "type": "pro", "entity": "放射免疫方法" }, { "start_idx": 82, "end_idx": 89, "type": "ite", "entity": "新生儿末梢血T4" }, { "start_idx": 108, "end_idx": 111, "type": "pro", "entity": "CH筛查" } ] }, { "text": "1975年Irie和Naruse在日本采用干血滤纸片法测定TSH的方法进行CH筛查。", "entities": [ { "start_idx": 21, "end_idx": 31, "type": "pro", "entity": "干血滤纸片法测定TSH" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "CH筛查" } ] }, { "text": "1982年在日本东京召开了第二届国际新生儿筛查大会,会上提出了适合大规模筛查的四种疾病:PKU、CH、半乳糖血症和先天性肾上腺皮质增生症。", "entities": [ { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "PKU" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "CH" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 57, "end_idx": 67, "type": "dis", "entity": "先天性肾上腺皮质增生症" } ] }, { "text": "随着对遗传代谢病的深入了解和技术的发展,其他一些疾病也被列入了筛查范围,例如葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏症、生物素酶缺乏、酪氨酸血症、镰刀状红细胞贫血、组氨酸血症、枫糖尿病、同型胱氨酸尿症、囊性纤维变性、高胱氨酸尿症以及地中海贫血等数十种疾病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 38, "end_idx": 59, "type": "dis", "entity": "葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏症" }, { "start_idx": 61, "end_idx": 66, "type": "dis", "entity": "生物素酶缺乏" }, { "start_idx": 68, "end_idx": 72, "type": "dis", "entity": "酪氨酸血症" }, { "start_idx": 74, "end_idx": 81, "type": "dis", "entity": "镰刀状红细胞贫血" }, { "start_idx": 83, "end_idx": 87, "type": "dis", "entity": "组氨酸血症" }, { "start_idx": 89, "end_idx": 92, "type": "dis", "entity": "枫糖尿病" }, { "start_idx": 94, "end_idx": 100, "type": "dis", "entity": "同型胱氨酸尿症" }, { "start_idx": 102, "end_idx": 107, "type": "dis", "entity": "囊性纤维变性" }, { "start_idx": 109, "end_idx": 114, "type": "dis", "entity": "高胱氨酸尿症" }, { "start_idx": 117, "end_idx": 121, "type": "dis", "entity": "地中海贫血" } ] }, { "text": "目前的共识是在众多的出生缺陷疾病中开展新生儿疾病筛查,应该结合本国国情,筛查的疾病选择一般应符合以下几个标准:1.疾病危害严重,可导致残疾或致死,已构成公共卫生问题。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "出生缺陷疾病" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "残疾" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "致死" } ] }, { "text": "6.筛查费用相对低廉,筛查、诊断和治疗所需的费用应低于发病后的诊断、治疗的支出费用,即投入、产出比的经济效益良好。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "筛查" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "诊断" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "治疗" } ] }, { "text": "以苯丙酮尿症为例,在国际上,日本人的PKU发病率最低,为1/80500,其成本/效益比达1∶2.5,根据计算,即使发病率为1/140000,其成本/效益比仍达到1∶1.7。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "PKU" } ] }, { "text": "我国的PKU发病率约为1∶11000,经济效益应该更为可观。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "PKU" } ] }, { "text": "由于各国的经济、文化、科技水平、疾病的流行与发病情况不同,开展的项目也不同,其中PKU和CH发病率较高,治疗效果好,多数国家都首先从这两种疾病开始筛查,逐步增加项目。", "entities": [ { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "PKU" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "CH" } ] }, { "text": "新生儿疾病筛查覆盖率由1977年的29.2%上升至1986年的99.8%,至今筛查已超过3千万新生儿,筛查疾病包括苯丙酮尿症、枫糖尿病、同型胱氨酸尿症、半乳糖血症、先天性甲状腺功能减低症和先天性肾上腺皮质增生症等6种疾病。", "entities": [ { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "枫糖尿病" }, { "start_idx": 68, "end_idx": 74, "type": "dis", "entity": "同型胱氨酸尿症" }, { "start_idx": 76, "end_idx": 80, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 82, "end_idx": 92, "type": "dis", "entity": "先天性甲状腺功能减低症" }, { "start_idx": 94, "end_idx": 104, "type": "dis", "entity": "先天性肾上腺皮质增生症" } ] }, { "text": "此外,日本还对婴儿进行了神经母细胞瘤筛查,调查发现发病率为1∶8000。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "神经母细胞瘤" } ] }, { "text": "目前全球每年有上千万新生儿进行疾病筛查,一些发达国家已将新生儿疾病筛查列入国家卫生法,或者采用行政手段实施,筛查覆盖率接近100%。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "疾病筛查" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "新生儿疾病筛查" } ] }, { "text": "美国每年有400万婴儿接受新生儿疾病筛查。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "pro", "entity": "新生儿疾病筛查" } ] }, { "text": "第二节恙虫病【病原及流行病学】恙虫病立克次体是恙虫病(scrubtyphus)的病原体,又称东方立克次体。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "恙虫病" }, { "start_idx": 15, "end_idx": 21, "type": "mic", "entity": "恙虫病立克次体" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "恙虫病" }, { "start_idx": 27, "end_idx": 37, "type": "dis", "entity": "scrubtyphus" }, { "start_idx": 46, "end_idx": 51, "type": "mic", "entity": "东方立克次体" } ] }, { "text": "恙虫病是一种自然疫源性疾病,恙螨既是恙虫病唯一的传播媒介,又是恙虫病立克次体的保存宿主。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "恙虫病" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "恙虫病" }, { "start_idx": 31, "end_idx": 37, "type": "mic", "entity": "恙虫病立克次体" } ] }, { "text": "恙螨幼虫需吸取人或动物的淋巴液或血液才能完成从幼虫到稚虫的发育过程,只有恙螨的幼虫才是恙虫病的传播媒介。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "恙螨幼虫" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "淋巴液" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "血液" }, { "start_idx": 36, "end_idx": 37, "type": "mic", "entity": "恙螨" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "恙虫病" } ] }, { "text": "携带恙虫病立克次体的啮齿类动物主要有黄毛鼠、黄胸鼠、褐家鼠等。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "mic", "entity": "恙虫病立克次体" } ] }, { "text": "恙虫病主要分布于东半球的日本、印度、巴基斯坦、澳大利亚、前苏联的西伯利亚、朝鲜、中国、菲律宾和泰国等地。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "恙虫病" } ] }, { "text": "恙虫病的分布过去认为比较局限,近年来不断扩大,病例数有急剧上升趋势。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "恙虫病" } ] }, { "text": "我国东南、西北各省区都有恙虫病病例报道。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "恙虫病" } ] }, { "text": "【发病机制和病理改变】带有病原体的恙螨幼虫叮咬人体后,立克次体从叮咬部位进入人体,局部繁殖后侵入血流到达全身各组织器官。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "mic", "entity": "立克次体" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "血流" } ] }, { "text": "全身及局部淋巴结有炎性细胞浸润、出血及灶性坏死。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "淋巴结" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "炎性细胞" }, { "start_idx": 0, "end_idx": 22, "type": "sym", "entity": "全身及局部淋巴结有炎性细胞浸润、出血及灶性坏死" } ] }, { "text": "被恙螨叮咬的局部可见焦痂。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "焦痂" } ] }, { "text": "心血管方面有间质性心肌炎、心包积液、冠状动脉和主动脉外膜炎;脑膜充血、大脑水肿、脑膜脑炎;肝脏呈间质性肝炎、中央性坏死、肝组织自溶;出血性脾炎、脾淤血、脾组织自溶;肺炎、肺出血、肺水肿;胃黏膜出血性糜烂,小肠黏膜淋巴细胞浸润,大肠黏膜水肿;泌尿生殖系统也可累及。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "心血管" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "间质性心肌炎" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "心包积液" }, { "start_idx": 18, "end_idx": 28, "type": "dis", "entity": "冠状动脉和主动脉外膜炎" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "脑膜" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "脑膜充血" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "大脑" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "大脑水肿" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "脑膜脑炎" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "肝脏" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "间质性肝炎" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "中央性坏死" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "肝组织" }, { "start_idx": 60, "end_idx": 64, "type": "sym", "entity": "肝组织自溶" }, { "start_idx": 66, "end_idx": 70, "type": "sym", "entity": "出血性脾炎" }, { "start_idx": 72, "end_idx": 74, "type": "sym", "entity": "脾淤血" }, { "start_idx": 76, "end_idx": 80, "type": "sym", "entity": "脾组织自溶" }, { "start_idx": 82, "end_idx": 83, "type": "sym", "entity": "肺炎" }, { "start_idx": 85, "end_idx": 87, "type": "sym", "entity": "肺出血" }, { "start_idx": 89, "end_idx": 91, "type": "sym", "entity": "肺水肿" }, { "start_idx": 93, "end_idx": 95, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 93, "end_idx": 100, "type": "sym", "entity": "胃黏膜出血性糜烂" }, { "start_idx": 102, "end_idx": 109, "type": "bod", "entity": "小肠黏膜淋巴细胞" }, { "start_idx": 102, "end_idx": 111, "type": "sym", "entity": "小肠黏膜淋巴细胞浸润" }, { "start_idx": 113, "end_idx": 116, "type": "bod", "entity": "大肠黏膜" }, { "start_idx": 113, "end_idx": 118, "type": "sym", "entity": "大肠黏膜水肿" }, { "start_idx": 120, "end_idx": 125, "type": "bod", "entity": "泌尿生殖系统" } ] }, { "text": "发病急,伴高热寒战,体温在39~41℃,持续2~3周。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "高热寒战" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "体温在39~41℃" } ] }, { "text": "年长儿能自诉头痛、腹痛或全身酸痛。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "头痛" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "腹痛" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "全身酸痛" } ] }, { "text": "尚有精神委靡、嗜睡、食欲减退。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "精神委靡" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "嗜睡" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "食欲减退" } ] }, { "text": "大多数患儿出现结膜充血,局部及全身浅表淋巴结肿大,肝脾肿大,四肢水肿。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "结膜" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "结膜充血" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "淋巴结" }, { "start_idx": 12, "end_idx": 23, "type": "sym", "entity": "局部及全身浅表淋巴结肿大" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肝脾" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "四肢" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "四肢水肿" } ] }, { "text": "起病后3~8日多出现皮疹,为疏散的斑丘疹,大小不等,初为鲜红色,后逐渐变暗红,重症病例有出血点、瘀斑,压之不褪色。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "皮疹" }, { "start_idx": 14, "end_idx": 37, "type": "sym", "entity": "疏散的斑丘疹,大小不等,初为鲜红色,后逐渐变暗红" }, { "start_idx": 43, "end_idx": 55, "type": "sym", "entity": "有出血点、瘀斑,压之不褪色" } ] }, { "text": "皮疹可持续7~12天,以后开始消退,无脱屑。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "皮疹" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "无脱屑" } ] }, { "text": "焦痂是恙虫病的特有症状,是恙虫叮咬的部位。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "焦痂" }, { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "恙虫病" } ] }, { "text": "为圆形或椭圆形,直径约1~2cm,边缘稍突起,高出于皮肤,周围有红晕,中央稍凹陷,表面黑色,不化脓,在软组织部位仅呈白色溃疡面,溃疡大多不痛,常不为患者注意。", "entities": [ { "start_idx": 29, "end_idx": 48, "type": "sym", "entity": "周围有红晕,中央稍凹陷,表面黑色,不化脓" }, { "start_idx": 58, "end_idx": 62, "type": "sym", "entity": "白色溃疡面" } ] }, { "text": "【诊断】(一)流行病学资料为恙虫病的自然疫源地区;1周前曾去过草地或鼠类出没的污秽环境。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "恙虫病" } ] }, { "text": "(二)临床症状和体征除发热和皮疹外,焦痂具有特殊的诊断价值。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "发热" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "皮疹" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "焦痂" } ] }, { "text": "外斐试验特异性敏感性低,不应作为恙虫病的诊断方法。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "外斐试验" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "恙虫病" } ] }, { "text": "免疫荧光试验检测患者血清中的抗恙虫病立克次体IgM、IgG效价,效价在1∶80以上有诊断意义。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "免疫荧光试验检测" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "血清" } ] }, { "text": "应用聚合酶链反应可在发病2天内检出恙虫病立克次体特异性DNA而作出病原学诊断。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "聚合酶链反应" }, { "start_idx": 17, "end_idx": 29, "type": "mic", "entity": "恙虫病立克次体特异性DNA" } ] }, { "text": "【治疗及预防】(一)治疗可用氯霉素、四环素、多西环素治疗。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "氯霉素" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "四环素" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "多西环素" } ] }, { "text": "重症可采用静脉滴注。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "同时应注意对症支持治疗。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "对症支持治疗" } ] }, { "text": "(二)预防重视环境卫生,消灭老鼠。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "消灭老鼠" } ] }, { "text": "目前尚未研制出高效理想的恙虫病疫苗。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "恙虫病疫苗" } ] }, { "text": "【预后】轻者于1周后可退热,重者于2~3周退热,一切症状减轻而进入恢复期,但并发肺炎、出现脑症状、胃肠道大出血者则预后不良。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "退热" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "退热" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "肺炎" }, { "start_idx": 45, "end_idx": 47, "type": "sym", "entity": "脑症状" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "胃肠道" }, { "start_idx": 49, "end_idx": 54, "type": "sym", "entity": "胃肠道大出血" } ] }, { "text": "参考文献1.王振义,李家增,阮长耿.血栓与止血-基础理论与临床.第2版.上海:上海科学技术出版社,1996:261-261,283-283,298-298,312-3122.陈淑容.血友病A//王振义,李家增,阮长耿.血栓与止血-基础理论与临床.第2版.上海:上海科学技术出版社,1996,283-2833.高怡瑾.血友病的诊断治疗研究进展.国外医学儿科学分册,1999,26(4)∶169-1694.李志广.血友病A基因突变及其检测方法的进展.国外医学临床生物化学与检验学分册,2000,21(6)∶310-3105.阮长耿.血管性血友病.//张之南.血液病诊断及治疗标准.第2版.北京:科学出版社,1998,312-3126.张之南.血液病诊断及疗效标准.第2版.北京:科学出版社,1998,268-2687.FeinsteinDI.InhibitorsinHemophilia//HoffmanR,BenzEJ,ShattilSJ,etal.Hematology:BasicPrinciplesandPractice.3rded.NewYork:ChurchillLivingstone,2001:1904-19048.GralnickHR,GinsburgD.vonWillebrandDisease//BeutlerE,LichtmanMA,CollerBS.WillimasHematology.5thed.NewYork:McGraw-HillInc,1995:1458-14589.HoffmanR.Hematology:BasicPrinciplesandPractice.3rded.NewYork:ChurchillLivingstone,2001:1904-1904,1904-1911,1946-194610.LanzkowskyP,ManualofPediatricHematologyandOncology3rded.SanDiego:AHarcourtSciencesandTechnologyCompany,2000:233-23311.LozierJN,KesslerCM.ClinicalAspectsandTherapyofHemophilia.In:HoffmanR,BenzEJ,ShattilSJ,etal.Hematology:BasicPrinciplesandPractice.3rded.NewYork:ChurchillLivingstone,2001:1883-188312.RobertsHR,HoffmanM.Hemophiliaandrelatedconditionsinheriteddeficienciesofprothrombin(factorⅡ),factorⅤ,andfactorⅦtoⅫ//BeutlerE,LichtmanMA,CollerBS.WillimasHematology.5thed.NewYork:McGraw-HillInc,1995:1413-141313.WhiteⅡGC,MontgomeryRR.ClinicalAspectsofandTheraphyforvonWillebrandDisease//HoffmanR,BenzEJ,ShattilSJ,etal.Hematology:BasicPrinciplesandPractice.3rded.NewYork:ChurchillLivingstone,2001:1946-1946", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "血栓" }, { "start_idx": 91, "end_idx": 94, "type": "dis", "entity": "血友病A" }, { "start_idx": 109, "end_idx": 110, "type": "dis", "entity": "血栓" }, { "start_idx": 158, "end_idx": 160, "type": "dis", "entity": "血友病" }, { "start_idx": 205, "end_idx": 208, "type": "dis", "entity": "血友病A" }, { "start_idx": 264, "end_idx": 269, "type": "dis", "entity": "血管性血友病" }, { "start_idx": 277, "end_idx": 279, "type": "dis", "entity": "血液病" }, { "start_idx": 319, "end_idx": 321, "type": "dis", "entity": "血液病" } ] }, { "text": "三、扁桃体周围脓肿扁桃体周围脓肿(peritonsillarabscess)常见的病原是A组溶血性链球菌和口腔厌氧菌。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "扁桃体周围脓肿" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "扁桃体周围脓肿" }, { "start_idx": 18, "end_idx": 37, "type": "dis", "entity": "peritonsillarabscess" }, { "start_idx": 45, "end_idx": 52, "type": "mic", "entity": "A组溶血性链球菌" }, { "start_idx": 54, "end_idx": 58, "type": "mic", "entity": "口腔厌氧菌" } ] }, { "text": "多数先有咽扁桃体炎病史。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "咽扁桃体炎" } ] }, { "text": "受累侧扁桃体明显充血肿大。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "扁桃体" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "受累侧扁桃体明显充血肿大" } ] }, { "text": "如既往无慢性扁桃体炎病史,复发率约10%,无需扁桃体切除。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "慢性扁桃体炎" }, { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "扁桃体切除" } ] }, { "text": "如既往有慢性扁桃体炎或扁桃体脓肿史,最好行扁桃体切除术。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "慢性扁桃体炎" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "扁桃体脓肿" }, { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "扁桃体切除术" } ] }, { "text": "第二节纤维支气管镜术自1964年日本Ikeda采用可曲式光导纤维支气管镜(flexiblefiberopticbronchoscope)以来,随着激光、荧光微波、电视等技术的进展以及临床的需要,纤维支气管镜的功能与用途不断扩展,目前已成为成人支气管及肺部疾病诊断和治疗的重要工具。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "pro", "entity": "纤维支气管镜术" }, { "start_idx": 26, "end_idx": 36, "type": "equ", "entity": "可曲式光导纤维支气管镜" }, { "start_idx": 38, "end_idx": 67, "type": "equ", "entity": "flexiblefiberopticbronchoscope" }, { "start_idx": 98, "end_idx": 103, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 122, "end_idx": 129, "type": "dis", "entity": "支气管及肺部疾病" } ] }, { "text": "由于小儿气道狭窄及配合不佳等原因,纤维支气管镜在儿科临床应用中起步较晚。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "气道狭窄" }, { "start_idx": 17, "end_idx": 22, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 24, "end_idx": 25, "type": "dep", "entity": "儿科" } ] }, { "text": "但近年来国内小儿纤维支气管镜检查与治疗已得到了很大的发展。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "pro", "entity": "小儿纤维支气管镜" } ] }, { "text": "一、小儿纤维支气管镜的检查和治疗技术(一)形态学检查纤维支气管镜纤细、柔软又可弯曲,在气管中可以随意调整前进方向。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "小儿纤维支气管镜" }, { "start_idx": 26, "end_idx": 31, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "气管" } ] }, { "text": "能进入硬支气管镜无法探及的左右上叶,并可插入到段、亚段支气管以下。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "equ", "entity": "硬支气管镜" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "左右上叶" }, { "start_idx": 23, "end_idx": 29, "type": "bod", "entity": "段、亚段支气管" } ] }, { "text": "从声门由上而下注意黏膜是否正常、管腔有无变形、管壁运动状况,有无赘生物、异物、出血点、窦道及分泌物情况。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "声门" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "黏膜" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "管腔" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "管壁" } ] }, { "text": "(二)活检技术可用毛刷和针吸取病理标本,主要用于细胞学检查。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "活检" }, { "start_idx": 9, "end_idx": 10, "type": "equ", "entity": "毛刷" }, { "start_idx": 12, "end_idx": 12, "type": "equ", "entity": "针" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "细胞学检查" } ] }, { "text": "活检钳则用于支气管黏膜和肺组织活检。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "活检钳" }, { "start_idx": 6, "end_idx": 16, "type": "pro", "entity": "支气管黏膜和肺组织活检" } ] }, { "text": "(三)病原学检测技术由于下呼吸道的致病菌与咽部存在的细菌并不一致,因而传统的咽拭子或痰培养对下呼吸道感染病原菌的诊断并不可靠。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "下呼吸道" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "致病菌" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "咽部" }, { "start_idx": 26, "end_idx": 27, "type": "mic", "entity": "细菌" }, { "start_idx": 38, "end_idx": 40, "type": "equ", "entity": "咽拭子" }, { "start_idx": 42, "end_idx": 44, "type": "ite", "entity": "痰培养" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "下呼吸道感染" }, { "start_idx": 52, "end_idx": 54, "type": "mic", "entity": "病原菌" } ] }, { "text": "纤维支气管镜下气管内导管吸引或毛刷可取得下呼吸道标本。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "气管" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "导管" }, { "start_idx": 15, "end_idx": 16, "type": "equ", "entity": "毛刷" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "下呼吸道" } ] }, { "text": "防污染毛刷、尤其是双腔管保护性标本刷(protectedspecimenbrush,PSB)的应用使污染率明显下降。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "equ", "entity": "防污染毛刷" }, { "start_idx": 9, "end_idx": 17, "type": "equ", "entity": "双腔管保护性标本刷" }, { "start_idx": 19, "end_idx": 40, "type": "equ", "entity": "protectedspecimenbrush" }, { "start_idx": 42, "end_idx": 44, "type": "equ", "entity": "PSB" } ] }, { "text": "由于毛刷隐藏在双层套管中,顶端有聚乙二醇堵塞,可避免咽喉部污染。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "equ", "entity": "毛刷" }, { "start_idx": 7, "end_idx": 10, "type": "equ", "entity": "双层套管" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "聚乙二醇" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "咽喉部" } ] }, { "text": "20世纪90年代以来,采用顶端带气囊的防污染导管进行支气管肺泡灌洗(bronchoalveolarlavage,BAL),既可获得大量的灌洗液(BALF)标本,又可使污染率下降至2%。", "entities": [ { "start_idx": 13, "end_idx": 23, "type": "equ", "entity": "顶端带气囊的防污染导管" }, { "start_idx": 26, "end_idx": 32, "type": "pro", "entity": "支气管肺泡灌洗" }, { "start_idx": 34, "end_idx": 54, "type": "pro", "entity": "bronchoalveolarlavage" }, { "start_idx": 56, "end_idx": 58, "type": "pro", "entity": "BAL" }, { "start_idx": 72, "end_idx": 75, "type": "pro", "entity": "BALF" } ] }, { "text": "但在儿童由于受到小儿纤维支气管镜本身的制约,以上防污染方法尚无法应用,仍只能通过活检孔取样。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "equ", "entity": "小儿纤维支气管镜" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "活检" } ] }, { "text": "在取样前应尽量避免通过活检孔吸引上呼吸道分泌物。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "上呼吸道" } ] }, { "text": "(四)BAL将纤维支气管镜伸至段或亚段支气管开口处,根据年龄每次注入生理盐水5~20ml,随即负压吸引,共2~3次。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "BAL" }, { "start_idx": 7, "end_idx": 12, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "段或亚段支气管" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "生理盐水" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "负压吸引" } ] }, { "text": "BALF除病原学检查外,还可进行免疫、炎症细胞等分析研究。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "BALF" }, { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "病原学检查" } ] }, { "text": "BAL作为支气管肺局部治疗方法,可有效清除下呼吸道黏稠分泌物、改善气道阻塞并注药治疗顽固性肺部炎症,对控制支气管肺内化脓性感染、治疗阻塞性肺不张或肺气肿有明显效果。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "BAL" }, { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "支气管肺局部治疗" }, { "start_idx": 21, "end_idx": 29, "type": "bod", "entity": "下呼吸道黏稠分泌物" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "气道" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "气道阻塞" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "顽固性肺部炎症" }, { "start_idx": 53, "end_idx": 62, "type": "dis", "entity": "支气管肺内化脓性感染" }, { "start_idx": 66, "end_idx": 75, "type": "dis", "entity": "阻塞性肺不张或肺气肿" } ] }, { "text": "一般先用0.5ml/kg的生理盐水多次冲洗,再用活检钳或毛刷清除肉芽和脓苔,然后再次冲洗。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "equ", "entity": "活检钳" }, { "start_idx": 28, "end_idx": 29, "type": "equ", "entity": "毛刷" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肉芽" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "脓苔" } ] }, { "text": "有专家认为,在管腔干净后可向局部气道注入青霉素、庆大霉素、甲硝唑或头孢菌素等,但其有效性、安全性与必要性尚有待研究。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "气道" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "青霉素" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "庆大霉素" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "甲硝唑" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "头孢菌素" } ] }, { "text": "(五)钳取深部支气管异物与硬质支气管镜相比,纤维支气管镜具有能够弯曲、深入、直观、且可越过异物从异物远端进行注气、注液治疗等优势,可钳取异物,或应用支气管冲洗加吸引的方法取异物。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "支气管" }, { "start_idx": 13, "end_idx": 18, "type": "equ", "entity": "硬质支气管镜" }, { "start_idx": 22, "end_idx": 27, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 54, "end_idx": 60, "type": "pro", "entity": "注气、注液治疗" }, { "start_idx": 74, "end_idx": 81, "type": "pro", "entity": "支气管冲洗加吸引" } ] }, { "text": "纤维支气管镜已成为诊断和治疗支气管深部异物的一种有效手段。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "支气管深部异物" } ] }, { "text": "二、产时出血(一)头颅血肿和帽状腱膜下出血为胎儿头颅在产道受压、牵拉、器械助产等所致,随着产前诊断、产程监护、手术方式的改进,本病发生率已明显减少。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "头颅血肿" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "帽状腱膜下出血" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "头颅" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "产道" } ] }, { "text": "【临床表现】1.头颅血肿又称骨膜下血肿,常位于一侧或两侧顶骨部,局部皮肤不肿、不变色骨膜下出血缓慢,血肿多在生后数小时或2~3天才明显,1周内达最大范围,以后渐吸收缩小。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "头颅血肿" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "骨膜下血肿" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "顶骨部" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "皮肤" }, { "start_idx": 32, "end_idx": 41, "type": "sym", "entity": "局部皮肤不肿、不变色" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "骨膜" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "血肿" } ] }, { "text": "血肿界限清楚,不越过骨缝,有波动感,局部皮肤颜色无改变,此可与头皮水肿及帽状腱膜下血肿骨缝,头皮水肿出生时即发现,界限不分明,压之柔软且可凹,无波动感,局部皮肤可呈红或紫色。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "血肿" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "骨缝" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "皮肤" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "头皮水肿" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "帽状腱膜" }, { "start_idx": 36, "end_idx": 42, "type": "sym", "entity": "帽状腱膜下血肿" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "骨缝" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "头皮水肿" }, { "start_idx": 57, "end_idx": 61, "type": "sym", "entity": "界限不分明" }, { "start_idx": 63, "end_idx": 69, "type": "sym", "entity": "压之柔软且可凹" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "无波动感" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "皮肤" } ] }, { "text": "头颅血肿位于枕骨部位者,需与脑膜膨出鉴别,后者随呼吸有起伏感,头颅X线片可见局部颅骨有缺损头颅血肿颅骨完整,偶见颅骨有线样骨折。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "头颅血肿" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "脑膜膨出" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "头颅" }, { "start_idx": 33, "end_idx": 35, "type": "pro", "entity": "X线片" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "颅骨" }, { "start_idx": 38, "end_idx": 44, "type": "sym", "entity": "局部颅骨有缺损" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "头颅血肿" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "颅骨" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "颅骨" } ] }, { "text": "巨大头颅血肿可致失血性贫血及高胆红素血症。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "头颅" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "失血性贫血" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "高胆红素血症" } ] }, { "text": "头颅血肿吸收较慢,因大小不同可在2周~3个月左右消退。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "头颅血肿" } ] }, { "text": "吸收时先在血肿边缘形成隆起的骨化的硬边,中央凹陷,呈火山口样改变。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "血肿" } ] }, { "text": "2.帽状腱膜下血肿生后不久即见头皮局限性肿胀,出血可通过软组织扩散,出血量较少时,血肿范围较局限;出血量多时,肿胀范围逐渐扩大,可累及整个头皮,甚至波及额、眼周、枕或颈背部。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "帽状腱膜下血肿" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "头皮" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "局限性肿胀" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "软组织" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "血肿" }, { "start_idx": 49, "end_idx": 51, "type": "ite", "entity": "出血量" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "头皮" }, { "start_idx": 76, "end_idx": 76, "type": "bod", "entity": "额" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "眼周" }, { "start_idx": 81, "end_idx": 81, "type": "bod", "entity": "枕" }, { "start_idx": 83, "end_idx": 85, "type": "bod", "entity": "颈背部" } ] }, { "text": "血肿有波动感,常使前囟不易扪清,所覆皮肤可呈青紫色。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血肿" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "前囟" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "皮肤" } ] }, { "text": "出血严重时可致贫血或低血容量休克,若不及时治疗可引起死亡。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "贫血" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "低血容量休克" } ] }, { "text": "【治疗】头颅血肿多可自行吸收,无需特殊治疗,出血较多引起贫血时,可适量输血;引起高胆红素血症时,需进行光疗。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "头颅血肿" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "贫血" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "输血" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "高胆红素血症" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "光疗" } ] }, { "text": "为避免感染不应抽吸血肿,若2个月后头颅血肿仍巨大,可手术清除之。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "血肿" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "头颅血肿" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "手术" } ] }, { "text": "可用维生素K1治疗,以防止因发生新生儿出血症而引起出血加重。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dru", "entity": "维生素K1" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "新生儿出血症" } ] }, { "text": "(二)内脏出血新生儿内脏出血主要发生在产时,常由产伤引起,生后1~3天即发生贫血、失血性休克等严重临床表现,有时内脏出血不容易及时发现,导致死亡,应予以重视。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "内脏出血" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "新生儿内脏出血" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "贫血" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "失血性休克" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "内脏出血" } ] }, { "text": "【临床表现】1.颅内出血颅内出血(详见颅内出血章节)是新生儿期常见的临床问题,出血部位包括硬膜下出血蛛网膜下腔出血脑室周围-脑室内出血小脑出血和脑实质出血蛛网膜下腔出血脑室周围-脑室内出血惊厥、两眼凝视、呼吸暂停、四肢肌张力增高或降低、前囟隆起贫血等。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "颅内出血" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "颅内出血" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "颅内出血" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "硬膜" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "硬膜下出血" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "蛛网膜" }, { "start_idx": 50, "end_idx": 56, "type": "sym", "entity": "蛛网膜下腔出血" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "脑室" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "周围-脑室" }, { "start_idx": 57, "end_idx": 66, "type": "sym", "entity": "脑室周围-脑室内出血" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "小脑" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "脑实质" }, { "start_idx": 67, "end_idx": 76, "type": "sym", "entity": "小脑出血和脑实质出血" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "蛛网膜" }, { "start_idx": 77, "end_idx": 83, "type": "sym", "entity": "蛛网膜下腔出血" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "脑室" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "周围-脑室" }, { "start_idx": 84, "end_idx": 93, "type": "sym", "entity": "脑室周围-脑室内出血" }, { "start_idx": 94, "end_idx": 95, "type": "sym", "entity": "惊厥" }, { "start_idx": 97, "end_idx": 100, "type": "sym", "entity": "两眼凝视" }, { "start_idx": 102, "end_idx": 105, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 107, "end_idx": 116, "type": "sym", "entity": "四肢肌张力增高或降低" }, { "start_idx": 118, "end_idx": 119, "type": "bod", "entity": "前囟" }, { "start_idx": 118, "end_idx": 121, "type": "sym", "entity": "前囟隆起" }, { "start_idx": 122, "end_idx": 123, "type": "dis", "entity": "贫血" } ] }, { "text": "2.肝脾破裂贫血、面色苍白,病情呈进行性加重,严重者出现失血性休克。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肝脾" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "肝脾破裂" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "贫血" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "面色苍白" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "失血性休克" } ] }, { "text": "肝脏出血包膜破裂腹腔出血腹胀、腹部叩诊有移动性浊音。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肝脏" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "肝脏出血" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "包膜" }, { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "包膜破裂" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "腹腔" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "腹腔出血" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "腹胀" }, { "start_idx": 15, "end_idx": 24, "type": "sym", "entity": "腹部叩诊有移动性浊音" } ] }, { "text": "3.肾上腺出血患儿突然出现贫血、面色苍白、循环衰竭、青紫、呼吸不规则、黄疸、四肢肌张力降低,侧腹部触及包块。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肾上腺" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "贫血" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "面色苍白" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "循环衰竭" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "青紫" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "呼吸不规则" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "黄疸" }, { "start_idx": 38, "end_idx": 44, "type": "sym", "entity": "四肢肌张力降低" }, { "start_idx": 46, "end_idx": 52, "type": "sym", "entity": "侧腹部触及包块" } ] }, { "text": "【诊断】1.病史多有异常分娩史,出生后要严密观察面色、四肢循环、心率、呼吸及腹部体征。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "ite", "entity": "四肢循环" }, { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "心率" }, { "start_idx": 35, "end_idx": 41, "type": "ite", "entity": "呼吸及腹部体征" } ] }, { "text": "2.影像学检查怀疑内脏出血者应及时做影像学检查。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "内脏" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "内脏出血" } ] }, { "text": "怀疑肝、脾破裂或肾上腺出血腹部B超或腹部X线平片。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肝" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "脾" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肾上腺" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "肾上腺出血" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "腹部" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "B超" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "腹部" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "X线平片" } ] }, { "text": "3.判断出血部位对出生后发生严重贫血者,要根据临床表现判断是否发生内脏出血出血量及是否发生失血性休克根据生命体征、血压、血红蛋白动态变化,及时作出判断。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "严重贫血" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "内脏" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "内脏出血" }, { "start_idx": 37, "end_idx": 39, "type": "ite", "entity": "出血量" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "失血性休克" }, { "start_idx": 52, "end_idx": 55, "type": "ite", "entity": "生命体征" }, { "start_idx": 57, "end_idx": 58, "type": "ite", "entity": "血压" }, { "start_idx": 60, "end_idx": 67, "type": "ite", "entity": "血红蛋白动态变化" } ] }, { "text": "【治疗】1.止血可用维生素K1、酚磺乙胺、氨甲苯酸(PAMBA)等。", "entities": [ { "start_idx": 10, "end_idx": 20, "type": "dru", "entity": "维生素K1" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "酚磺乙胺" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "氨甲苯酸" }, { "start_idx": 32, "end_idx": 36, "type": "dru", "entity": "PAMBA" } ] }, { "text": "2.纠正低血容量如发生失血性休克,应立即给生理盐水,先快速静脉滴注10~20ml/kg,然后根据病情继续维持。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "血容量" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "失血性休克" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "生理盐水" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "3.输血出现严重贫血或失血性休克,应及时输血,每次10~20ml/kg,病情严重者需多次输血。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "贫血" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "失血性休克" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "输血" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "输血" } ] }, { "text": "4.手术对肝脾破裂肾上腺出血糖皮质激素治疗,用氢化可的松,每天5~10mg/kg,静脉滴注。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肝脾" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "肝脾破裂" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肾上腺" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "肾上腺出血" }, { "start_idx": 14, "end_idx": 18, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "氢化可的松" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "第二章心肺脑复苏【概述】心肺复苏始于1958年,kouwenhoven于1960年将其标准化并广泛用于临床,80年代将脑复苏推向复苏前沿,即目前所称的心肺脑复苏(cardiopulmonarycerebralresuscitation,CPCR)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "心肺脑复苏" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 59, "end_idx": 61, "type": "pro", "entity": "脑复苏" }, { "start_idx": 75, "end_idx": 79, "type": "pro", "entity": "心肺脑复苏" }, { "start_idx": 81, "end_idx": 116, "type": "pro", "entity": "cardiopulmonarycerebralresuscitation" }, { "start_idx": 118, "end_idx": 121, "type": "pro", "entity": "CPCR" } ] }, { "text": "90年代,基础研究已深入到细胞分子水平,复苏操作上出现了一些新方法和新概念,复苏学已成为一门新学科。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "复苏操作" } ] }, { "text": "现今认为机体从有生命到死亡经历了临终状态(terminalstate)、心脏骤停(cardiacarrest)、临床死亡(clinicaldeath)、生物死亡(biologicdeath)。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "死亡" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "临终状态" }, { "start_idx": 21, "end_idx": 33, "type": "sym", "entity": "terminalstate" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "心脏" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "心脏骤停" }, { "start_idx": 41, "end_idx": 53, "type": "sym", "entity": "cardiacarrest" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "临床死亡" }, { "start_idx": 61, "end_idx": 73, "type": "sym", "entity": "clinicaldeath" }, { "start_idx": 76, "end_idx": 79, "type": "sym", "entity": "生物死亡" }, { "start_idx": 81, "end_idx": 93, "type": "sym", "entity": "biologicdeath" } ] }, { "text": "CPCR是对临床死亡及前期采取的心、肺、脑功能抢救措施。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "CPCR" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "死亡" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "心" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肺" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "脑" } ] }, { "text": "【呼吸心脏骤停的病因】心肺脑复苏的对象是各种原因引起的呼吸心脏骤停。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "呼吸心脏骤停" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "心肺脑复苏" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "呼吸心脏骤停" } ] }, { "text": "引起小儿呼吸、心搏骤停的病因如下:(一)呼吸系统疾病如上气道阻塞(如异物、反流、喉痉挛、喉水肿等)、下气道疾病(如继发于呼吸衰竭或呼吸停止的疾病)。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "呼吸、心搏骤停" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "呼吸系统疾病" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "上气道阻塞" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "异物" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "反流" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "喉痉挛" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "喉水肿" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "下气道疾病" }, { "start_idx": 60, "end_idx": 71, "type": "dis", "entity": "呼吸衰竭或呼吸停止的疾病" } ] }, { "text": "(二)感染如败血症、脑膜炎等。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "败血症" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "(三)中毒与药物过敏如药品(麻醉性抑制剂、镇静剂、抗节律不齐药物药物中毒)、农药、有害气体中毒、青霉素过敏等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "中毒" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "药物过敏" }, { "start_idx": 14, "end_idx": 35, "type": "dis", "entity": "麻醉性抑制剂、镇静剂、抗节律不齐药物药物中毒" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "有害气体中毒" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "青霉素过敏" } ] }, { "text": "(四)循环系统疾病如休克、先天性心脏病、心肌炎、心包炎、心律失常等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "循环系统疾病" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "休克" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "心肌炎" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "心包炎" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "心律失常" } ] }, { "text": "(五)中枢神经系统疾病如颅脑外伤、颅内感染、颅内出血、颅内肿瘤、脑疝等。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "中枢神经系统疾病" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "颅脑外伤" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "颅内感染" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "颅内出血" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "脑疝" } ] }, { "text": "(六)创伤和意外如窒息、溺水、婴儿猝死综合征等。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "窒息" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "溺水" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "婴儿猝死综合征" } ] }, { "text": "(七)代谢性疾病如酸碱和电解质紊乱等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "代谢性疾病" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "酸碱和电解质紊乱" } ] }, { "text": "【临床表现和诊断】心搏骤停常有如下临床表现:①突然出现昏迷,部分患儿有一过性抽搐;②瞳孔扩大;③大动脉搏动消失;④心音消失及心动过缓;⑤呼吸停止或严重呼吸困难;⑥心电图显示等电位和极缓慢心律。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "心搏骤停" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "突然出现昏迷" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "一过性抽搐" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "瞳孔" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "瞳孔扩大" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "大动脉" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "大动脉搏动消失" }, { "start_idx": 57, "end_idx": 65, "type": "sym", "entity": "心音消失及心动过缓" }, { "start_idx": 68, "end_idx": 71, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 73, "end_idx": 78, "type": "sym", "entity": "严重呼吸困难" }, { "start_idx": 81, "end_idx": 83, "type": "ite", "entity": "心电图" }, { "start_idx": 81, "end_idx": 88, "type": "sym", "entity": "心电图显示等电位" }, { "start_idx": 90, "end_idx": 94, "type": "sym", "entity": "极缓慢心律" } ] }, { "text": "但切不可等待出现上述临床表现时才考虑实施心肺脑复苏术。", "entities": [ { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "心肺脑复苏术" } ] }, { "text": "如能早期发现心搏骤停的先兆,及时判断并尽早实施心肺脑复苏术常可提高患儿存活率;心肺脑复苏术开始越早,复苏成功率越高。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "心搏骤停" }, { "start_idx": 23, "end_idx": 28, "type": "pro", "entity": "心肺脑复苏术" }, { "start_idx": 39, "end_idx": 44, "type": "pro", "entity": "心肺脑复苏术" } ] }, { "text": "要避免如下操作而延误时间,丧失抢救良机:①不断地试听患儿心音有无;②等待心电图显示结果;③反复检查患儿瞳孔和扪摸动脉。", "entities": [ { "start_idx": 21, "end_idx": 31, "type": "pro", "entity": "不断地试听患儿心音有无" }, { "start_idx": 34, "end_idx": 42, "type": "pro", "entity": "等待心电图显示结果" }, { "start_idx": 45, "end_idx": 57, "type": "pro", "entity": "反复检查患儿瞳孔和扪摸动脉" } ] }, { "text": "临床上迅速而准确的诊断依据是:①突然出现昏迷;②大动脉搏动消失;③呼吸停止。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "突然出现昏迷" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "大动脉" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "大动脉搏动消失" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "呼吸停止" } ] }, { "text": "【小儿呼吸心搏骤停的特点】1.心搏骤停在心电图上的常见三种表现形式为心室纤颤、心脏停搏和电机械分离。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "sym", "entity": "小儿呼吸心搏骤停" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "心搏骤停" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "心电图" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "心室" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "心室纤颤" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "心脏" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "心脏停搏" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "电机械分离" } ] }, { "text": "与成人相比,儿童很少由心室纤颤引起,而主要是极缓慢心律,其中多数是心室自主心律,小部分为极缓慢的窦性心动过缓。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "心室纤颤" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "极缓慢心律" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "心室自主心律" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "心" }, { "start_idx": 44, "end_idx": 53, "type": "sym", "entity": "极缓慢的窦性心动过缓" } ] }, { "text": "了解小儿心脏停搏的心电图特点,成人复苏时采用心前区捶击捶击的方法原则上不适用于小儿。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "心电图" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "复苏" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "心前区捶击" } ] }, { "text": "2.与成人相比,小儿心搏骤停较少原发于心脏疾病,而多为严重疾病的终末结果,在儿科多因呼吸停止造成的严重低氧血症和高碳酸血症所致,先引起呼吸骤停,继而心搏骤停。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "小儿心搏骤停" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "心脏疾病" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "严重低氧血症" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 67, "end_idx": 70, "type": "dis", "entity": "呼吸骤停" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "心搏骤停" } ] }, { "text": "所以,保持呼吸道通畅是复苏成败的关键措施之一。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "保持呼吸道通畅" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "复苏" } ] }, { "text": "上气道阻塞是复苏时需最先处理的问题。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "上气道" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "上气道阻塞" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "复苏" } ] }, { "text": "3.儿童心、肺、脑各个器官发育尚不成熟,易受体内外环境的影响,年龄愈小,呼吸心搏骤停发生率愈高。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "心" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肺" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "脑" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "呼吸心搏骤停" } ] }, { "text": "但小儿脑组织对缺氧耐受性比成人强,多器官衰竭的慢性病较少,故复苏成功率较成人高。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "脑组织" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "多器官衰竭" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "复苏" } ] }, { "text": "复苏成功的患儿遗留的后遗症相对比成人少。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "复苏" } ] }, { "text": "4.呼吸道感染是儿童最常见疾病,由呼吸道分泌物堵塞而致的窒息成为小儿呼吸心搏骤停的主要直接因素,恢复肺有效通气的简单、有效、最易于施行的措施是清理呼吸道和口对口人工呼吸。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "呼吸道分泌物" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "窒息" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "小儿呼吸心搏骤停" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "肺" }, { "start_idx": 71, "end_idx": 75, "type": "pro", "entity": "清理呼吸道" }, { "start_idx": 77, "end_idx": 83, "type": "pro", "entity": "口对口人工呼吸" } ] }, { "text": "若有简易复苏器和人工呼吸机行气管内插管予以辅助通气是首选方法,有条件应尽早使用,只是单纯鼻导管给氧是最错误的方法。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "equ", "entity": "复苏器" }, { "start_idx": 8, "end_idx": 12, "type": "equ", "entity": "人工呼吸机" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "气管内插管" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "辅助通气" }, { "start_idx": 44, "end_idx": 48, "type": "pro", "entity": "鼻导管给氧" } ] }, { "text": "此外,在呼吸停止时,不管心脏是否停搏,均应立即实施心肺脑复苏术。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "心脏" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "停搏" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "心肺脑复苏术" } ] }, { "text": "【标准心肺复苏程序】1973年,美国心脏病学会在美国医学杂志(JAMA)发表了心肺复苏指南和抢救规范条例,后几次修订,内容包括:①基本生命支持(basiclifesupport,BLS);②进一步生命支持(advancedlifesupport,ALS);③延续生命支持延续生命支持(prolongedlifesupport,PLS)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "心脏病" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 65, "end_idx": 70, "type": "pro", "entity": "基本生命支持" }, { "start_idx": 72, "end_idx": 87, "type": "pro", "entity": "basiclifesupport" }, { "start_idx": 89, "end_idx": 91, "type": "pro", "entity": "BLS" }, { "start_idx": 95, "end_idx": 101, "type": "pro", "entity": "进一步生命支持" }, { "start_idx": 103, "end_idx": 121, "type": "pro", "entity": "advancedlifesupport" }, { "start_idx": 123, "end_idx": 125, "type": "pro", "entity": "ALS" }, { "start_idx": 129, "end_idx": 134, "type": "pro", "entity": "延续生命支持" }, { "start_idx": 142, "end_idx": 161, "type": "pro", "entity": "prolongedlifesupport" }, { "start_idx": 163, "end_idx": 165, "type": "pro", "entity": "PLS" } ] }, { "text": "标准心肺复苏程序见表6-4。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "心肺复苏" } ] }, { "text": "(一)基本生命支持(BLS)由现场人员立即对患者进行抢救,是心、肺、脑复苏的第一阶段,主要目的是保持呼吸道通畅,建立人工呼吸和人工循环,保证供给重要生命器官的血液和氧气,给患者提供最基本的生命支持,即心肺复苏ABC。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "基本生命支持" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "BLS" }, { "start_idx": 30, "end_idx": 36, "type": "pro", "entity": "心、肺、脑复苏" }, { "start_idx": 48, "end_idx": 54, "type": "pro", "entity": "保持呼吸道通畅" }, { "start_idx": 56, "end_idx": 66, "type": "pro", "entity": "建立人工呼吸和人工循环" }, { "start_idx": 72, "end_idx": 77, "type": "bod", "entity": "重要生命器官" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "血液" }, { "start_idx": 88, "end_idx": 97, "type": "pro", "entity": "提供最基本的生命支持" }, { "start_idx": 100, "end_idx": 106, "type": "pro", "entity": "心肺复苏ABC" } ] }, { "text": "其中包括A(呼吸道通畅)、B(辅助呼吸)、C(辅助循环)。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "呼吸道通畅" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "辅助呼吸" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "辅助循环" } ] }, { "text": "1.保持呼吸道通畅保持呼吸道通畅是复苏成败的关键措施之一,只有呼吸道通畅复苏程序才能发挥作用。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "保持呼吸道通畅" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "保持呼吸道通畅" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "复苏" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "呼吸道" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "呼吸道通畅" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "复苏" } ] }, { "text": "方法有:表6-4心肺复苏程序(1)背部叩打加胸部压迫法(图6-6):医务人员取坐位,保持婴儿俯卧于救者前臂上,前臂可放于大腿上,用手指张开托住患儿的嘴并固定患儿的头,保持头低位。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 17, "end_idx": 26, "type": "pro", "entity": "背部叩打加胸部压迫法" }, { "start_idx": 34, "end_idx": 40, "type": "pro", "entity": "医务人员取坐位" }, { "start_idx": 42, "end_idx": 53, "type": "pro", "entity": "保持婴儿俯卧于救者前臂上" }, { "start_idx": 55, "end_idx": 62, "type": "pro", "entity": "前臂可放于大腿上" }, { "start_idx": 64, "end_idx": 81, "type": "pro", "entity": "用手指张开托住患儿的嘴并固定患儿的头" }, { "start_idx": 83, "end_idx": 87, "type": "pro", "entity": "保持头低位" } ] }, { "text": "用手掌根部在婴儿肩胛之间进行5次有力的拍打。", "entities": [ { "start_idx": 0, "end_idx": 20, "type": "pro", "entity": "用手掌根部在婴儿肩胛之间进行5次有力的拍打" } ] }, { "text": "拍背后将空闲的手放于婴儿背部,手指托住其头颈部,此时患儿处于两手之间,一手支持其头、颈、嘴、胸,一手支持其背部。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "pro", "entity": "拍背后将空闲的手放于婴儿背部" }, { "start_idx": 15, "end_idx": 22, "type": "pro", "entity": "手指托住其头颈部" }, { "start_idx": 24, "end_idx": 33, "type": "pro", "entity": "此时患儿处于两手之间" }, { "start_idx": 35, "end_idx": 46, "type": "pro", "entity": "一手支持其头、颈、嘴、胸" }, { "start_idx": 48, "end_idx": 54, "type": "pro", "entity": "一手支持其背部" } ] }, { "text": "当头、颈很好地托住后,小心地将婴儿反转过来,使其仰卧于另一手的前臂上,手臂置于大腿上,继续维持头低位。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "头" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "颈" }, { "start_idx": 11, "end_idx": 20, "type": "pro", "entity": "小心地将婴儿反转过来" }, { "start_idx": 22, "end_idx": 33, "type": "pro", "entity": "使其仰卧于另一手的前臂上" }, { "start_idx": 35, "end_idx": 41, "type": "pro", "entity": "手臂置于大腿上" }, { "start_idx": 43, "end_idx": 49, "type": "pro", "entity": "继续维持头低位" } ] }, { "text": "施行5次快速的胸部冲压,位置与胸部按压相同。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "pro", "entity": "施行5次快速的胸部冲压" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "胸部按压" } ] }, { "text": "若婴儿较大,可将婴儿置于救者大腿上,头置于膝部,用手可靠地固定住头部并保持头低位,在行5次拍背后,翻转过来进行5次胸部冲压,若能见到患儿口或鼻中有异物,应迅速用手指或器械除去异物。", "entities": [ { "start_idx": 7, "end_idx": 16, "type": "pro", "entity": "将婴儿置于救者大腿上" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "头置于膝部" }, { "start_idx": 24, "end_idx": 39, "type": "pro", "entity": "用手可靠地固定住头部并保持头低位" }, { "start_idx": 41, "end_idx": 47, "type": "pro", "entity": "在行5次拍背后" }, { "start_idx": 49, "end_idx": 60, "type": "pro", "entity": "翻转过来进行5次胸部冲压" }, { "start_idx": 68, "end_idx": 68, "type": "bod", "entity": "口" }, { "start_idx": 70, "end_idx": 70, "type": "bod", "entity": "鼻" }, { "start_idx": 73, "end_idx": 74, "type": "dis", "entity": "异物" }, { "start_idx": 68, "end_idx": 74, "type": "sym", "entity": "口或鼻中有异物" }, { "start_idx": 79, "end_idx": 88, "type": "pro", "entity": "用手指或器械除去异物" } ] }, { "text": "在急救现场如无适当的器械,保持呼吸道通畅的首要措施是用手或其他方法将上呼吸道异物清除,同时将患者放平,复苏者用一手托起患者下颌并使头轻度后仰,以减轻舌后坠导致的气道阻塞。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "equ", "entity": "器械" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "呼吸道" }, { "start_idx": 26, "end_idx": 41, "type": "pro", "entity": "用手或其他方法将上呼吸道异物清除" }, { "start_idx": 43, "end_idx": 49, "type": "pro", "entity": "同时将患者放平" }, { "start_idx": 51, "end_idx": 69, "type": "pro", "entity": "复苏者用一手托起患者下颌并使头轻度后仰" }, { "start_idx": 74, "end_idx": 74, "type": "bod", "entity": "舌" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "气道阻塞" } ] }, { "text": "图6-1背部叩打加胸部压迫法(引自:JAMA,1992,268(16):2251-2251)(2)口咽和鼻咽通气管的插入(图6-6):最简单的开放气道的器械是口咽和鼻咽通气导管。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "pro", "entity": "背部叩打加胸部压迫法" }, { "start_idx": 49, "end_idx": 56, "type": "equ", "entity": "口咽和鼻咽通气管" }, { "start_idx": 58, "end_idx": 59, "type": "pro", "entity": "插入" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "气道" }, { "start_idx": 79, "end_idx": 87, "type": "equ", "entity": "口咽和鼻咽通气导管" } ] }, { "text": "口咽和鼻咽通气导管的作用在于防止舌根后坠所致的上气道阻塞,适用于有意识障碍、舌根后坠,呼吸尚平稳、血气分析及血氧饱和度正常的患儿。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "equ", "entity": "口咽和鼻咽通气导管" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "舌根" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "舌根后坠" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "上气道阻塞" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "有意识障碍" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "舌根" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "舌根后坠" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "呼吸尚平稳" }, { "start_idx": 49, "end_idx": 52, "type": "ite", "entity": "血气分析" }, { "start_idx": 54, "end_idx": 58, "type": "ite", "entity": "血氧饱和度" }, { "start_idx": 49, "end_idx": 60, "type": "sym", "entity": "血气分析及血氧饱和度正常" } ] }, { "text": "(3)气管切开法:紧急状态下,可行环甲膜穿刺法或环甲膜造口术。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "气管切开法" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "环甲膜穿刺法" }, { "start_idx": 24, "end_idx": 29, "type": "pro", "entity": "环甲膜造口术" } ] }, { "text": "环甲膜穿刺法采用16或18号粗针与注射器相连,当注射器内有空气时表明已进入气管。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "环甲膜穿刺法" }, { "start_idx": 8, "end_idx": 15, "type": "equ", "entity": "16或18号粗针" }, { "start_idx": 17, "end_idx": 19, "type": "equ", "entity": "注射器" }, { "start_idx": 24, "end_idx": 26, "type": "equ", "entity": "注射器" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "气管" } ] }, { "text": "环甲膜造口术可由内科医生和五官科医生共同完成,只需气管切开包和气管切开导管等简单器械即可。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "环甲膜造口术" }, { "start_idx": 8, "end_idx": 9, "type": "dep", "entity": "内科" }, { "start_idx": 13, "end_idx": 15, "type": "dep", "entity": "五官科" }, { "start_idx": 25, "end_idx": 29, "type": "equ", "entity": "气管切开包" }, { "start_idx": 31, "end_idx": 36, "type": "equ", "entity": "气管切开导管" } ] }, { "text": "环甲膜穿刺法或环甲膜造口术是上气道堵塞时开放气道最简单、最迅速的方法。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "环甲膜穿刺法" }, { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "环甲膜造口术" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "上气道堵塞" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "气道" } ] }, { "text": "2.辅助呼吸在保持呼吸道通畅后,仍无自主呼吸的患儿要积极做辅助呼吸,保证供给重要生命器官的氧气。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "辅助呼吸" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "呼吸道" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "无自主呼吸" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "辅助呼吸" }, { "start_idx": 38, "end_idx": 43, "type": "bod", "entity": "重要生命器官" } ] }, { "text": "辅助呼吸的方法有:(1)口对口辅助呼吸法(图6-6):口对口辅助呼吸法是最简单、有效且易于施行的现场急救措施。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "辅助呼吸" }, { "start_idx": 12, "end_idx": 19, "type": "pro", "entity": "口对口辅助呼吸法" }, { "start_idx": 27, "end_idx": 34, "type": "pro", "entity": "口对口辅助呼吸法" } ] }, { "text": "如果复苏者吸气后再进行口对口辅助呼吸,患儿可吸入高达18%氧,而二氧化碳浓度可低至2%。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "口对口辅助呼吸" } ] }, { "text": "实施者首先通过看胸腹部的起伏,听有无呼气音,用面颊部感受由口呼出的气流来评价有无呼吸,这个过程不要超过10秒,一旦患儿无反应、无呼吸,立即做2次有效呼吸,口对口辅助呼吸法的具体操作是,复苏者站或跪在患儿一侧,将患者的头轻轻后仰使颈项平直,用一手抬起患儿下颌并打开口腔,同时另一手捏住患儿鼻孔,在吸一口气之后,用口将患儿口腔紧紧盖住,然后用力向患儿呼吸道吹气,直吹到其胸部相应抬起为止,若患儿较小,可不必捏住患儿鼻孔,而实行口对口鼻辅助呼吸,吹气后、复苏者移开自己的口腔,并放开患儿之鼻孔借患儿的胸廓与肺的弹性回缩自然呼气。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "胸腹部" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "听有无呼气音" }, { "start_idx": 22, "end_idx": 41, "type": "pro", "entity": "用面颊部感受由口呼出的气流来评价有无呼吸" }, { "start_idx": 59, "end_idx": 61, "type": "sym", "entity": "无反应" }, { "start_idx": 63, "end_idx": 65, "type": "sym", "entity": "无呼吸" }, { "start_idx": 69, "end_idx": 75, "type": "pro", "entity": "做2次有效呼吸" }, { "start_idx": 77, "end_idx": 84, "type": "pro", "entity": "口对口辅助呼吸法" }, { "start_idx": 92, "end_idx": 102, "type": "pro", "entity": "复苏者站或跪在患儿一侧" }, { "start_idx": 104, "end_idx": 117, "type": "pro", "entity": "将患者的头轻轻后仰使颈项平直" }, { "start_idx": 119, "end_idx": 132, "type": "pro", "entity": "用一手抬起患儿下颌并打开口腔" }, { "start_idx": 134, "end_idx": 144, "type": "pro", "entity": "同时另一手捏住患儿鼻孔" }, { "start_idx": 146, "end_idx": 152, "type": "pro", "entity": "在吸一口气之后" }, { "start_idx": 154, "end_idx": 164, "type": "pro", "entity": "用口将患儿口腔紧紧盖住" }, { "start_idx": 166, "end_idx": 177, "type": "pro", "entity": "然后用力向患儿呼吸道吹气" }, { "start_idx": 179, "end_idx": 190, "type": "pro", "entity": "直吹到其胸部相应抬起为止" }, { "start_idx": 205, "end_idx": 206, "type": "bod", "entity": "鼻孔" }, { "start_idx": 211, "end_idx": 218, "type": "pro", "entity": "口对口鼻辅助呼吸" }, { "start_idx": 232, "end_idx": 233, "type": "bod", "entity": "口腔" }, { "start_idx": 241, "end_idx": 242, "type": "bod", "entity": "鼻孔" }, { "start_idx": 247, "end_idx": 248, "type": "bod", "entity": "胸廓" }, { "start_idx": 250, "end_idx": 250, "type": "bod", "entity": "肺" } ] }, { "text": "操作要点是:①保持气道开放位;②每次送气时间1~1.5秒;③胸部抬起。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "保持气道开放位" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "送气" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "胸部抬起" } ] }, { "text": "对存在脉搏但呼吸停止的无反应患儿仅行人工呼吸,无需胸外按压,成人10~12次/分、儿童12~20次/分。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "存在脉搏" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "无反应" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "人工呼吸" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "胸外按压" } ] }, { "text": "图6-2简易通气道A.鼻咽通气道;B.口咽通气道(引自:樊寻梅.实用急救与危重症抢救技术图解.北京:人民卫生出版社,2000:110-110)图6-3口对口辅助呼吸(引自:JAMA,1992,268(16):2251-2251)图6-4面罩呼吸气囊辅助呼吸法(引自:樊寻梅.实用急救与危重症抢救技术图解.北京:人民卫生出版社,2000:13)(2)面罩或呼吸气囊辅助呼吸法(图6-4):面罩或呼吸气囊辅助呼吸,如给氧流量10L/min,FiO2可达30%~40%,使用时将呼吸气囊的面罩盖紧患儿的口鼻,同时用呼吸气囊的皮球的皮球给患儿送气。", "entities": [ { "start_idx": 76, "end_idx": 81, "type": "pro", "entity": "对口辅助呼吸" }, { "start_idx": 118, "end_idx": 128, "type": "pro", "entity": "面罩呼吸气囊辅助呼吸法" }, { "start_idx": 174, "end_idx": 185, "type": "pro", "entity": "面罩或呼吸气囊辅助呼吸法" }, { "start_idx": 193, "end_idx": 203, "type": "pro", "entity": "面罩或呼吸气囊辅助呼吸" }, { "start_idx": 242, "end_idx": 248, "type": "equ", "entity": "呼吸气囊的面罩" }, { "start_idx": 254, "end_idx": 255, "type": "bod", "entity": "口鼻" }, { "start_idx": 260, "end_idx": 266, "type": "equ", "entity": "呼吸气囊的皮球" }, { "start_idx": 273, "end_idx": 274, "type": "pro", "entity": "送气" } ] }, { "text": "但应注意引起胃胀气。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "胃胀气" } ] }, { "text": "(3)气管内插管和人工机械通气法:气管内插管是首选方法,有条件应尽早使用,并应做好充分的插管前准备,确保插管顺利完成。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "pro", "entity": "气管内插管和人工机械通气法" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "气管内插管" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "插管" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "插管" } ] }, { "text": "1)器械准备:①直接喉镜:注意检查喉镜柄和叶片的接头、喉镜光源,确保其性能良好;②气管导管:气管导管分为有囊导管和无囊导管,有囊导管主要用于年长儿,无囊导管主要用于新生儿和婴幼儿。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "equ", "entity": "直接喉镜" }, { "start_idx": 17, "end_idx": 19, "type": "equ", "entity": "喉镜柄" }, { "start_idx": 21, "end_idx": 25, "type": "equ", "entity": "叶片的接头" }, { "start_idx": 27, "end_idx": 28, "type": "equ", "entity": "喉镜" }, { "start_idx": 41, "end_idx": 44, "type": "equ", "entity": "气管导管" }, { "start_idx": 46, "end_idx": 49, "type": "equ", "entity": "气管导管" }, { "start_idx": 52, "end_idx": 55, "type": "equ", "entity": "有囊导管" }, { "start_idx": 57, "end_idx": 60, "type": "equ", "entity": "无囊导管" }, { "start_idx": 62, "end_idx": 65, "type": "equ", "entity": "有囊导管" }, { "start_idx": 74, "end_idx": 77, "type": "equ", "entity": "无囊导管" } ] }, { "text": "常用气管导管型号如表6-6;2岁以上儿童还可以按以下公式计算:管腔内径=4+年龄/4。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "equ", "entity": "气管导管" } ] }, { "text": "表6-5常用气管导管型号及插入深度2)药物准备:肾上腺素、阿托品、麻黄碱、利多卡因、地西泮、苯巴比妥、硫喷妥钠。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "equ", "entity": "气管导管" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "阿托品" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "麻黄碱" }, { "start_idx": 37, "end_idx": 40, "type": "dru", "entity": "利多卡因" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "地西泮" }, { "start_idx": 46, "end_idx": 49, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 51, "end_idx": 54, "type": "dru", "entity": "硫喷妥钠" } ] }, { "text": "3)其他:器械包括插管钳、牙垫、胶布,并注意给氧导管、皮囊接头、吸痰器是否完好、静脉通道是否通畅。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "equ", "entity": "插管钳" }, { "start_idx": 13, "end_idx": 14, "type": "equ", "entity": "牙垫" }, { "start_idx": 16, "end_idx": 17, "type": "equ", "entity": "胶布" }, { "start_idx": 22, "end_idx": 25, "type": "equ", "entity": "给氧导管" }, { "start_idx": 27, "end_idx": 30, "type": "equ", "entity": "皮囊接头" }, { "start_idx": 32, "end_idx": 34, "type": "equ", "entity": "吸痰器" }, { "start_idx": 40, "end_idx": 43, "type": "equ", "entity": "静脉通道" } ] }, { "text": "气管插管的具体步骤见其他章节。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "气管插管" } ] }, { "text": "人工呼吸机的应用见其他章节。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "equ", "entity": "人工呼吸机" } ] }, { "text": "3.辅助循环在保持呼吸道通畅后,应积极建立循环保证供给重要生命器官的血液。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "辅助循环" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "呼吸道" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "重要生命器官" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "血液" } ] }, { "text": "对心脏有节律性地按压,使心内血排出进入全身的动脉系统,可分为胸外按压和胸内按压。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "对心脏有节律性地按压" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "心内血" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "动脉系统" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "胸外按压" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "胸内按压" } ] }, { "text": "(1)胸外按压:在保持呼吸道通畅后,应积极建立循环保证供给重要生命器官的血液。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "胸外按压" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "呼吸道" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "重要生命器官" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "血液" } ] }, { "text": "对心脏有节律性地按压,使心内血排出入全身的动脉系统。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "对心脏有节律性地按压" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "心内血" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "动脉系统" } ] }, { "text": "专业人员则要求同时检查患者的脉搏搏动及观察循环的征象,时间不超过10秒。", "entities": [ { "start_idx": 9, "end_idx": 25, "type": "pro", "entity": "检查患者的脉搏搏动及观察循环的征象" } ] }, { "text": "1岁以上检查颈动脉搏动,1岁以下检查肱动脉或股动脉搏动,如果无呼吸但脉搏存在,则单做人工呼吸,每2分钟要检查一次脉搏。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "pro", "entity": "检查颈动脉搏动" }, { "start_idx": 16, "end_idx": 26, "type": "pro", "entity": "检查肱动脉或股动脉搏动" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "无呼吸但脉搏存在" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "人工呼吸" }, { "start_idx": 52, "end_idx": 57, "type": "pro", "entity": "检查一次脉搏" } ] }, { "text": "如果未触到脉搏或心率小于60次/分,且有体循环灌注不良表现时,即开始胸外按压。", "entities": [ { "start_idx": 2, "end_idx": 16, "type": "sym", "entity": "未触到脉搏或心率小于60次/分" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "体循环" }, { "start_idx": 19, "end_idx": 28, "type": "sym", "entity": "有体循环灌注不良表现" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "胸外按压" } ] }, { "text": "2005指南将胸外按压更加简化,按压的技术要求见表6-6。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "按压" } ] }, { "text": "表6-6小儿心外按压的技术要求(2)胸内按压:小儿开胸作胸内心脏按压的机会较少。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "小儿心外按压" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "胸内按压" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "开胸" }, { "start_idx": 28, "end_idx": 33, "type": "pro", "entity": "胸内心脏按压" } ] }, { "text": "仅在手术开胸后出现心搏骤停,当患儿有胸骨、脊柱畸形无法正确进行胸外按压等少数紧急情况下使用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "手术开胸" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "心搏骤停" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "胸骨" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "脊柱" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "脊柱畸形" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "胸外按压" } ] }, { "text": "(二)进一步生命支持(ALS)经过BLS后应采取进一步生命支持,一般在医院急诊室进行,是心肺脑复苏的第二阶段,主要目的是努力恢复自主心律和自主呼吸,以保证生命体征基本稳定,其中包括心肺复苏药物的使用、严密的心脏监护和电复律。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "进一步生命支持" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "ALS" }, { "start_idx": 17, "end_idx": 19, "type": "pro", "entity": "BLS" }, { "start_idx": 24, "end_idx": 30, "type": "pro", "entity": "进一步生命支持" }, { "start_idx": 37, "end_idx": 39, "type": "dep", "entity": "急诊室" }, { "start_idx": 44, "end_idx": 48, "type": "pro", "entity": "心肺脑复苏" }, { "start_idx": 90, "end_idx": 98, "type": "pro", "entity": "心肺复苏药物的使用" }, { "start_idx": 100, "end_idx": 106, "type": "pro", "entity": "严密的心脏监护" }, { "start_idx": 108, "end_idx": 110, "type": "pro", "entity": "电复律" } ] }, { "text": "(三)延续生命支持(PLS)在保证生命体征基本稳定的基础上,积极开展脑复苏,一般在医院重症监护室里进行,主要目的是防止中枢神经系统后遗症的发生,其中包括进一步明确诊断、脑复苏、加强医疗护理、多器官功能衰竭的治疗。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "延续生命支持" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "PLS" }, { "start_idx": 34, "end_idx": 36, "type": "pro", "entity": "脑复苏" }, { "start_idx": 43, "end_idx": 47, "type": "dep", "entity": "重症监护室" }, { "start_idx": 59, "end_idx": 64, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 76, "end_idx": 82, "type": "pro", "entity": "进一步明确诊断" }, { "start_idx": 84, "end_idx": 86, "type": "pro", "entity": "脑复苏" }, { "start_idx": 88, "end_idx": 93, "type": "pro", "entity": "加强医疗护理" }, { "start_idx": 95, "end_idx": 104, "type": "pro", "entity": "多器官功能衰竭的治疗" } ] }, { "text": "【心肺复苏药物的重新评价】近年来,国内外学者对心肺复苏药物从理论到临床都进行了深入研究,提出了许多新概念和新认识,了解这些进展,对提高CPCR的成功率具有重要的意义。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dru", "entity": "心肺复苏药物" }, { "start_idx": 23, "end_idx": 28, "type": "dru", "entity": "心肺复苏药物" }, { "start_idx": 67, "end_idx": 70, "type": "pro", "entity": "CPCR" } ] }, { "text": "(一)肾上腺素是公认CPR的首选药物,具有α、β受体兴奋作用,小剂量的肾上腺素主要兴奋β受体,扩张血管,增加心率和房室心肌收缩力;中等剂量肾上腺素兼有α和β受体兴奋作用;大剂量肾上腺素主要为α受体兴奋作用,即收缩外周血管,增高外周阻力,增高主动脉舒张压。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "CPR" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "α、β受体" }, { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "β受体" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "血管" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "房室心肌" }, { "start_idx": 69, "end_idx": 72, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 75, "end_idx": 79, "type": "bod", "entity": "α和β受体" }, { "start_idx": 88, "end_idx": 91, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 95, "end_idx": 97, "type": "bod", "entity": "α受体" }, { "start_idx": 106, "end_idx": 109, "type": "bod", "entity": "外周血管" }, { "start_idx": 113, "end_idx": 114, "type": "bod", "entity": "外周" }, { "start_idx": 120, "end_idx": 122, "type": "bod", "entity": "主动脉" } ] }, { "text": "2005年美国CPR新指南肾上腺素均用标准剂量0.01mg/kg(1∶10000溶液,0.1ml/kg)静脉或骨髓给药,气管给药0.1mg/kg(1∶1000溶液,0.1ml/kg);持续给药:0.1~1μg/(kg•min),心跳恢复后减量。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 52, "end_idx": 58, "type": "pro", "entity": "静脉或骨髓给药" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "气管给药" }, { "start_idx": 92, "end_idx": 95, "type": "pro", "entity": "持续给药" } ] }, { "text": "酸性环境可使肾上腺素药物效应减弱,肾上腺素亦不可与碱性液在同一管道输注,否则将使肾上腺素灭活影响药效。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "肾上腺素药物" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "碱性液" }, { "start_idx": 31, "end_idx": 32, "type": "equ", "entity": "管道" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "输注" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "肾上腺素" } ] }, { "text": "异丙肾上腺素为单纯β受体兴奋剂,由于其直接升压效果差,对改善冠状动脉灌注不利,且增加心肌耗氧量,已不作为复苏常规用药。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "异丙肾上腺素" }, { "start_idx": 7, "end_idx": 14, "type": "dru", "entity": "单纯β受体兴奋剂" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "心肌" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "复苏" } ] }, { "text": "去甲肾上腺素虽主要兴奋α受体,但可引起周围血管强烈收缩,增加外周血管阻力和心脏后负荷,所以亦不宜选用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "去甲肾上腺素" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "α受体" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "血管" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "血管" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "心脏" } ] }, { "text": "(二)5%NaHCO3循环一旦中止立即产生酸中毒,酸中毒可使窦房结与房室结传导减慢,导致心动过缓与心律不齐;还可直接减弱心肌收缩力。", "entities": [ { "start_idx": 3, "end_idx": 21, "type": "dru", "entity": "5%NaHCO3" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "酸中毒" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "酸中毒" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "窦房结" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "房室结" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "心动过缓" }, { "start_idx": 66, "end_idx": 69, "type": "dis", "entity": "心律不齐" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "心肌" } ] }, { "text": "酸中毒可使脑微循环自动调节功能丧失,脑血管扩张,同时毛细血管通透性增强,可引起血管源性脑水肿,使颅压增高。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "酸中毒" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "脑血管" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "脑血管扩张" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "毛细血管" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "毛细血管通透性增强" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "血管源性脑水肿" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "颅" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "颅压增高" } ] }, { "text": "酸中毒可使肝素灭活,促进弥散性血管内凝血,故多年来碳酸氢钠一直被推荐为CPCR的必要药物之一。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "酸中毒" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肝素" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "肝素灭活" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血管" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "促进弥散性血管内凝血" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "CPCR" } ] }, { "text": "但由于下述理由使这一传统观念受到异议:①心搏骤停后,出现的酸中毒一般为呼吸性酸中毒合并高乳酸性代酸,有效循环建立前,碳酸氢钠不易纠正这类酸中毒;②碱性液使氧离曲线左移加重组织缺氧;③大剂量碳酸氢钠还可引起或加重高钠、高渗血症,加重脑的损害;④碱血症使K+细胞内流可致心律失常。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "心搏骤停" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "酸中毒" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "高乳酸性代酸" }, { "start_idx": 58, "end_idx": 61, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "酸中毒" }, { "start_idx": 85, "end_idx": 88, "type": "dis", "entity": "组织缺氧" }, { "start_idx": 94, "end_idx": 97, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 105, "end_idx": 111, "type": "dis", "entity": "高钠、高渗血症" }, { "start_idx": 115, "end_idx": 115, "type": "bod", "entity": "脑" }, { "start_idx": 113, "end_idx": 118, "type": "sym", "entity": "加重脑的损害" }, { "start_idx": 121, "end_idx": 123, "type": "dis", "entity": "碱血症" }, { "start_idx": 144, "end_idx": 147, "type": "sym", "entity": "心律失常" } ] }, { "text": "基于上述原因,目前,对于心肺复苏来讲,碳酸氢钠不是第一线药物。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "碳酸氢钠" } ] }, { "text": "迅速建立有效的通气和恢复全身灌注是处理酸中毒和低氧血症的基本措施。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "迅速建立有效的通气" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "恢复全身灌注" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "酸中毒" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "低氧血症" } ] }, { "text": "使用碳酸氢钠前必须进行有效的通气和恢复有效的灌注,以免加重机体损害。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "通气" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "恢复有效的灌注" } ] }, { "text": "婴儿0.5mmol/kg,儿童1.0mmol/kg(5%NaHCO31ml=0.6mmol),快速静滴,碳酸氢钠进一步用量应根据血pH和PaCO2测定值而定,使用时应将5%NaHCO3稀释为1.4%。", "entities": [ { "start_idx": 63, "end_idx": 66, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 75, "end_idx": 77, "type": "ite", "entity": "血pH" }, { "start_idx": 79, "end_idx": 94, "type": "ite", "entity": "PaCO2" }, { "start_idx": 108, "end_idx": 119, "type": "dru", "entity": "NaHCO3" } ] }, { "text": "(三)阿托品阿托品为胆碱酯酶拮抗剂,能降低迷走神经兴奋性。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "阿托品" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "阿托品" }, { "start_idx": 10, "end_idx": 16, "type": "dru", "entity": "胆碱酯酶拮抗剂" } ] }, { "text": "适用于临床因心动过缓以及房室结的房室传导阻滞伴室率缓慢的患儿,气管插管时因咽部受到刺激,迷走神经兴奋性增高,心脏复跳后出现心动过缓的患儿均可使用。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "心动过缓" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "房室结的房室传导阻滞" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "室率缓慢" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "气管插管" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "咽部" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "迷走神经" }, { "start_idx": 44, "end_idx": 52, "type": "sym", "entity": "迷走神经兴奋性增高" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "心脏" } ] }, { "text": "每次0.02mg/kg静脉注射,5分钟后可重复应用,最大单一剂量1mg(青少年)、0.5mg(儿童)。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "静脉注射" } ] }, { "text": "(四)利多卡因当心脏出现多源性室性期前收缩,利多卡因为首选药物。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "利多卡因" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心脏" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "利多卡因" } ] }, { "text": "它能抑制心脏自律性和室性异位性期前收缩,常用于心室纤颤。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "心脏" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "心室纤颤" } ] }, { "text": "在没有或来不及作心脏监护情况下,可依据下列情况判断:原发病为心脏疾患、心脏停搏前曾有过快速性室性心律失常,一旦心脏停搏,多为心室纤颤,此时可给利多卡因。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "作心脏监护" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "心脏疾患" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "心脏停搏" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "室性心律失常" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "心脏停搏" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "心室纤颤" }, { "start_idx": 71, "end_idx": 74, "type": "dru", "entity": "利多卡因" } ] }, { "text": "1mg/kg+5%GS10ml静注,5~10分钟重复,总量<5mg/kg,当心律复转后,25~40μg/(kg•min),静脉维持6小时。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "静注" } ] }, { "text": "(五)钙剂钙剂为心肌兴奋-收缩偶联所必需,以往认为Ca2+和K+有拮抗作用,可降低高K+对心肌的抑制,可提高心肌张力和兴奋性。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "钙剂" }, { "start_idx": 5, "end_idx": 6, "type": "dru", "entity": "钙剂" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心肌" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "心肌" } ] }, { "text": "从1984年后发现,Ca2+升高可使室颤变得更不易纠正,并使死亡率增加。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "室颤" } ] }, { "text": "这主要是因为缺血、缺氧时钙泵、钠泵失活,细胞外钙入细胞,导致微循环障碍、不可逆的破坏、血管痉挛、复苏后延迟性低灌流。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "缺血" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "缺氧" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "钙泵、钠泵失活" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "细胞" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "细胞" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "微循环障碍" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "不可逆的破坏" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "血管" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "血管痉挛" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "复苏" }, { "start_idx": 51, "end_idx": 56, "type": "sym", "entity": "延迟性低灌流" } ] }, { "text": "目前,使用指征为高钾血症、低钙血症和钙通道阻滞剂中毒。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "高钾血症" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "低钙血症" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "钙通道阻滞剂中毒" } ] }, { "text": "(六)葡萄糖CPCR后伴高血糖的患者预后极差,而高糖血症常被忽略,其对机体的危害尚未引起临床医师重视。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "CPCR" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "高血糖" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "高糖血症" } ] }, { "text": "呼吸心搏骤停时,突发强烈刺激致儿茶酚胺、皮质醇、胰高糖素等分解代谢激素分泌增多,这些激素除直接刺激糖原分解、糖原异生增加外,还通过不同途径拮抗胰岛素的生物效应。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "呼吸心搏骤停" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "儿茶酚胺" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "皮质醇" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "胰高糖素" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "激素" }, { "start_idx": 15, "end_idx": 38, "type": "sym", "entity": "儿茶酚胺、皮质醇、胰高糖素等分解代谢激素分泌增多" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "激素" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "胰岛素" } ] }, { "text": "此外,在各种突发强烈刺激下,胰岛素受体数目及受体最大特异结合率可受其影响而降低,最终导致血糖升高和高糖性高渗血症。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "胰岛素受体" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "受体" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "血糖" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "血糖升高" }, { "start_idx": 49, "end_idx": 55, "type": "dis", "entity": "高糖性高渗血症" } ] }, { "text": "由于严重缺血、缺氧,葡萄糖转为无氧酵解代谢增多,其产物乳酸增多。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "缺血" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "缺氧" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "乳酸" } ] }, { "text": "而CPCR期间输入葡萄糖必将使无氧酵解加剧,加重组织酸中毒中毒。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "CPCR" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "组织酸中毒" } ] }, { "text": "高血糖又可致血浆渗透压增高,进一步加重高渗性脑损伤。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "高血糖" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血浆" }, { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "血浆渗透压增高" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "高渗性脑损伤" } ] }, { "text": "CPCR期间的高血糖多为一过性,常随CPCR的抢救成功以及外周组织胰岛素拮抗的改善逐渐缓解,处理原则为积极根治原发病和严格控制外源性葡萄糖的输入,故CPCR期间宜用无糖液或5%的低糖液,快速大量输注葡萄糖对CPCR是不利的。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "CPCR" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "高血糖" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "CPCR" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "外周组织胰岛素" }, { "start_idx": 66, "end_idx": 68, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 74, "end_idx": 77, "type": "pro", "entity": "CPCR" }, { "start_idx": 82, "end_idx": 84, "type": "dru", "entity": "无糖液" }, { "start_idx": 89, "end_idx": 91, "type": "dru", "entity": "低糖液" }, { "start_idx": 99, "end_idx": 101, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 103, "end_idx": 106, "type": "pro", "entity": "CPCR" } ] }, { "text": "儿童的糖原储备有限,当发生心肺功能障碍时可被很快消耗殆尽,导致低血糖。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "心肺功能障碍" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "低血糖" } ] }, { "text": "高血糖和低血糖均可产生脑损伤,因此对心肺功能不稳定的危重儿都应床旁监测血糖浓度。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "高血糖" }, { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "低血糖" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "脑损伤" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "心肺功能不稳定" }, { "start_idx": 33, "end_idx": 38, "type": "ite", "entity": "监测血糖浓度" } ] }, { "text": "如发生低血糖可补葡萄糖,剂量为0.5~1g/kg或10%葡萄糖5~10ml/kg。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "低血糖" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "葡萄糖" } ] }, { "text": "(七)钙拮抗剂钙拮抗剂可直接抑制腺苷磷酸酶的分解,保护线粒体,降低乳酸,降低纤颤域值,不影响电复律,解除小动脉痉挛,加快血流,抑制血小板凝聚,降低血黏度,改善循环,阻止钙内流,保护缺血细胞。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "钙拮抗剂" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "钙拮抗剂" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "腺苷磷酸酶" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "线粒体" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "乳酸" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "纤颤" }, { "start_idx": 46, "end_idx": 48, "type": "pro", "entity": "电复律" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "小动脉" }, { "start_idx": 52, "end_idx": 56, "type": "sym", "entity": "小动脉痉挛" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "血小板" }, { "start_idx": 63, "end_idx": 69, "type": "sym", "entity": "抑制血小板凝聚" }, { "start_idx": 73, "end_idx": 73, "type": "bod", "entity": "血" }, { "start_idx": 71, "end_idx": 75, "type": "sym", "entity": "降低血黏度" }, { "start_idx": 90, "end_idx": 93, "type": "bod", "entity": "缺血细胞" } ] }, { "text": "临床常用药物有:维拉帕米(异搏定),硝苯地平,尼莫地平。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "维拉帕米" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "异搏定" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "硝苯地平" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "尼莫地平" } ] }, { "text": "缺点:负性心肌作用、抑制A-V传导、心律失常、扩张外周血管,故钙拮抗剂在CPCR的应用有待研究。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心肌" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "负性心肌作用" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "抑制A-V传导" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "心律失常" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "外周血管" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "扩张外周血管" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "钙拮抗剂" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "CPCR" } ] }, { "text": "(八)呼吸兴奋剂儿科呼吸、心搏骤停的临床特点多因严重低氧血症和高碳酸血症所致,先引起呼吸骤停,继而心搏骤停,故小儿CPCR时有效的人工通气显得尤为重要。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "呼吸兴奋剂" }, { "start_idx": 8, "end_idx": 9, "type": "dep", "entity": "儿科" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "呼吸、心搏骤停" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "低氧血症" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "呼吸骤停" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "心搏骤停" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "CPCR" }, { "start_idx": 65, "end_idx": 68, "type": "pro", "entity": "人工通气" } ] }, { "text": "口对口人工呼吸,一人只能坚持3分钟,因此气管内插管是首选方法,有条件应尽早使用。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "口对口人工呼吸" }, { "start_idx": 20, "end_idx": 24, "type": "pro", "entity": "气管内插管" } ] }, { "text": "目前,呼吸兴奋剂在复苏过程中已不主张使用。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "呼吸兴奋剂" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "复苏" } ] }, { "text": "【给药途径的选择】及时的给药和输液在CPCR十分重要,鉴于CPCR时上腔静脉系统和肺循环尚存在,故首选静脉和气管内给药。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "给药" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "给药" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "输液" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "CPCR" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "CPCR" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "上腔静脉系统" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "肺" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "静脉" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "气管" }, { "start_idx": 57, "end_idx": 58, "type": "pro", "entity": "给药" } ] }, { "text": "有研究表明,静脉、气管内、心内注射三种给药途径发挥的作用时间无明显差别。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "静脉" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "气管" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心内" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "给药" } ] }, { "text": "上腔静脉系统给药最好,以中心静脉最佳(锁骨下静脉、颈内静脉),平均2分左右发挥作用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "上腔静脉系统" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "中心静脉" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "锁骨下静脉" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "颈内静脉" } ] }, { "text": "但经皮静脉穿刺最快、最易。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "皮静脉穿刺" } ] }, { "text": "由于下肢末端静脉循环和回流缓慢,常不作为首选部位。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "下肢末端静脉" } ] }, { "text": "气管内给药由于发挥作用时间和静脉给药相似,在静脉通道建立困难时,气管内给药可通过肺毛细血管迅速吸收,使药物最快到达心脏。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "气管内给药" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "静脉给药" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "静脉" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "气管内给药" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "肺毛细血管" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "心脏" } ] }, { "text": "此外,气管内给药不必中止心外按压,亦不易引起并发症。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "气管内给药" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "心外按压" } ] }, { "text": "肾上腺素、异丙肾上腺素、阿托品、利多卡因等均可气管内给药,采用生理盐水稀释,根据患儿年龄大小每次剂量1~5ml。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "异丙肾上腺素" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "阿托品" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "利多卡因" }, { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "气管内给药" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "生理盐水" } ] }, { "text": "心内注射虽然给药效果确切,但干扰胸部按压,难以准确注入心室,并可导致心肌、冠脉损伤,已不主张使用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "心内注射" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "给药" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "胸部按压" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "心室" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "心肌、冠脉损伤" } ] }, { "text": "2005年新指南仍强调并推荐骨髓给药,若静脉穿刺3次失败或90秒内未能建立可靠的静脉通道,可使用骨髓给药。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "骨髓给药" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "静脉穿刺" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "静脉" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "骨髓给药" } ] }, { "text": "【抢救时机的选择】CPCR的对象是各种原因引起的呼吸、心搏骤停患儿,经过基本生命支持和进一步生命支持后,患儿呼吸、心跳恢复,并不意味着CPCR成功,小儿脑复苏是CPCR最终达到的目的,只有脑功能得到完全恢复,才能说是CPCR成功,脑复苏不能使已死亡的脑细胞复活、再生,但能保护尚未发生不可逆损害的脑细胞,阻止其病理过程的发展。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "CPCR" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "呼吸、心搏骤停" }, { "start_idx": 36, "end_idx": 41, "type": "pro", "entity": "基本生命支持" }, { "start_idx": 43, "end_idx": 49, "type": "pro", "entity": "进一步生命支持" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "呼吸、心跳恢复" }, { "start_idx": 67, "end_idx": 70, "type": "pro", "entity": "CPCR" }, { "start_idx": 76, "end_idx": 78, "type": "pro", "entity": "脑复苏" }, { "start_idx": 80, "end_idx": 83, "type": "pro", "entity": "CPCR" }, { "start_idx": 94, "end_idx": 94, "type": "bod", "entity": "脑" }, { "start_idx": 108, "end_idx": 111, "type": "pro", "entity": "CPCR" }, { "start_idx": 115, "end_idx": 117, "type": "pro", "entity": "脑复苏" }, { "start_idx": 125, "end_idx": 127, "type": "bod", "entity": "脑细胞" }, { "start_idx": 148, "end_idx": 150, "type": "bod", "entity": "脑细胞" } ] }, { "text": "因此,脑功能在CPCR中是否能完全恢复,目前已作为复苏成功的首要评价标准。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "脑" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "CPCR" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "复苏" } ] }, { "text": "心搏骤停的时间直接影响脑复苏的成功率。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "心搏骤停" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "脑复苏" } ] }, { "text": "一般心搏骤停10~15秒可引起缺氧晕厥脑,>15秒可发生抽搐,30~40秒呼吸停止、瞳孔扩大,3~5分钟严重脑损害,>10分钟无成活希望。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "心搏骤停" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "缺氧晕厥脑" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "抽搐" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "瞳孔" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "瞳孔扩大" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "脑损害" } ] }, { "text": "因此,最关键的抢救时机是最初1~4分钟,并应及早实施脑复苏。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "脑复苏" } ] }, { "text": "应尽早恢复有效脑灌注,要早期给药,并且联合用药优于单一用药。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "脑" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "早期给药" } ] }, { "text": "心肺脑复苏早期成功的征象为:①瞳孔缩小和对光反射恢复;②睫毛反射出现;③肌张力增强甚至出现不自主运动;④自主呼吸恢复。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "心肺脑复苏" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "瞳孔" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "对光反射恢复" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "睫毛" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "睫毛反射出现" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "肌张力" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "肌张力增强" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "不自主运动" } ] }, { "text": "【死亡概念的新认识】心、肺、脑复苏的患儿有4种结果:①完全恢复,不留任何后遗症;②部分恢复,但留有系统后遗症;③去大脑皮层综合征,即植物状态;④脑死亡。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "死亡" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "心、肺、脑复苏" }, { "start_idx": 72, "end_idx": 74, "type": "sym", "entity": "脑死亡" } ] }, { "text": "死亡是生命的永久丧失,但确定死亡并非简单。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "死亡" } ] }, { "text": "长期以来,人们都把心跳和呼吸停止作为死亡的征象,然而现代医学的发展使死亡的观点发生了根本改变。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "死亡" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "死亡" } ] }, { "text": "目前,通过现代医疗技术即使患者在脑神经细胞的综合功能完全丧失的情况下(脑死亡),也能维持患者脑外器官(呼吸和心跳)的生活能力。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "脑神经细胞" }, { "start_idx": 35, "end_idx": 37, "type": "sym", "entity": "脑死亡" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "脑外器官" } ] }, { "text": "因此,现代医学认为脑的状态是确定死亡的可靠而根本的指标。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "脑" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "死亡" } ] }, { "text": "脑死亡概念是1968年美国哈佛大学医学院首先提出的,随后世界各国都作出了不同的诊断标准。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "脑死亡" } ] }, { "text": "脑死亡的现代定义是指脑神经细胞的综合功能完全的、永久的丧失,即大脑皮层、脑干的全部脑功能完全的、永久的丧失,此时不论心跳、呼吸和脊髓功能是否存在。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "脑神经细胞" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "大脑皮层" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "脑干" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "脑" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "脊髓" } ] }, { "text": "在脑死亡后继续进行重症支持以维护患者脑外器官(呼吸和心跳)的生活能力是可能的,但只是毫无意义的浪费,并给国家、家庭和个人带来沉重的负担,当今国际上只有两种情况(器官移植供体捐赠者和妊娠妇女脑死亡后胎儿仍然活着)可考虑继续进行重症支持。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "重症支持" }, { "start_idx": 14, "end_idx": 33, "type": "pro", "entity": "维护患者脑外器官(呼吸和心跳)的生活能力" }, { "start_idx": 94, "end_idx": 96, "type": "dis", "entity": "脑死亡" }, { "start_idx": 112, "end_idx": 115, "type": "pro", "entity": "重症支持" } ] }, { "text": "我国目前尚无脑死亡标准和法规,大多参考美国哈佛大学脑死亡标准和美国国立疾病(NIR)及卒中研究所(NINDS)制定的脑死亡标准(表6-6及表6-8),对下述情况经家属同意后可考虑停止复苏:①CPCR前心跳呼吸停止15分钟以上;②心肺复苏30分钟以上心跳仍未恢复或进一步生命支持10分钟以上仍无心电活动;③已知为终末期疾病。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑死亡" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "脑死亡" }, { "start_idx": 91, "end_idx": 92, "type": "pro", "entity": "复苏" }, { "start_idx": 95, "end_idx": 98, "type": "pro", "entity": "CPCR" }, { "start_idx": 100, "end_idx": 111, "type": "sym", "entity": "心跳呼吸停止15分钟以上" }, { "start_idx": 114, "end_idx": 117, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 131, "end_idx": 137, "type": "pro", "entity": "进一步生命支持" } ] }, { "text": "表6-7美国哈佛大学脑死亡标准表6-8美国国立疾病(NIR)及卒中研究所(NIND脑死亡制定的脑死亡标准植物状态是持久的意识丧失,又称无脑综合征,代表着一种生与死亡间的状脑死亡它既非脑死亡,但意识又永远不能恢复。", "entities": [ { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "脑死亡" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "脑死亡" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "植物状态" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "无脑综合征" }, { "start_idx": 85, "end_idx": 87, "type": "dis", "entity": "脑死亡" } ] }, { "text": "其表现包括:①无意识活动,不能执行指令;②保持自主呼吸和血压;③有睡眠觉醒周期;④不能理解或表达语言;⑤能自动睁眼或在刺激下睁眼;⑥丘脑下部及脑干功能基本保持;⑦可有无目的性眼球跟踪运动。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "无意识活动" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "不能执行指令" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "保持自主呼吸和血压" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "有睡眠觉醒周期" }, { "start_idx": 41, "end_idx": 49, "type": "sym", "entity": "不能理解或表达语言" }, { "start_idx": 52, "end_idx": 63, "type": "sym", "entity": "能自动睁眼或在刺激下睁眼" }, { "start_idx": 66, "end_idx": 69, "type": "bod", "entity": "丘脑下部" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "脑干" }, { "start_idx": 66, "end_idx": 78, "type": "sym", "entity": "丘脑下部及脑干功能基本保持" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "眼球" }, { "start_idx": 84, "end_idx": 92, "type": "sym", "entity": "目的性眼球跟踪运动" } ] }, { "text": "植物状态持续1个月以上即人们通常说的植物人。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "植物状态" } ] }, { "text": "植物人与脑死亡有本质的区别,植物状态患者其低级神经中枢的功能尚存,而高级神经中枢的功能已经丧失,而脑死亡为全脑的完全性死亡,无任何中枢的功能存在。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "脑死亡" }, { "start_idx": 21, "end_idx": 26, "type": "bod", "entity": "低级神经中枢" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "高级神经中枢" }, { "start_idx": 49, "end_idx": 51, "type": "sym", "entity": "脑死亡" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "全脑" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "中枢" } ] }, { "text": "确定脑死亡。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑死亡" } ] }, { "text": "确定脑死亡标准有助于决定在什么时候可宣布患儿死亡,什么时候不值得去延长一个人的生物学生命。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑死亡" } ] }, { "text": "我国目前尚无明确的关于死亡标准的法律规定,有很多工作尚待完成。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "死亡" } ] }, { "text": "第四章呼吸系统疾病的临床诊断和检查技术第一节影像诊断技术一、影像检查方法传统的胸部X线平片仍为检查胸部疾病的基本方法,以胸部平片检查为起点,也可采用其他特殊体位,如斜位、侧位、前弓位、高千伏摄影,卧位水平位、侧位水平位。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "呼吸系统疾病" }, { "start_idx": 39, "end_idx": 44, "type": "pro", "entity": "胸部X线平片" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "胸部疾病" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "胸部平片" } ] }, { "text": "其他检查方法包括透视、食管造影、肺血管造影、支气管造影、CT和MRI等。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "透视" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "食管造影" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "肺血管造影" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "支气管造影" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "CT" }, { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "MRI" } ] }, { "text": "CT可得到横断面的高质量图像,与常规技术比较,其密度分辨率高,可显示小病灶,测量CT值可决定某些组织的特性(如脂肪、钙化、液体等)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "CT" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "CT" } ] }, { "text": "目前CT已发展到多排CT(16排、32排等),这有利于多层面及三维的重建,现在CT应用到肺部来越普遍。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "CT" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "多排CT" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "CT" }, { "start_idx": 44, "end_idx": 44, "type": "ite", "entity": "肺" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "肺" } ] }, { "text": "对不合作的小儿应镇静,让他(她)睡熟后再扫描,小婴儿层厚5mm,年长儿层厚10mm,对感兴趣处可选择2~3mm,甚至1mm,增强扫描可使血管和某些病灶密度增高,同时提高各种正常组织与病理组织密度差异,有利于病变的检查、病灶性质和范围的判断。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "镇静" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "血管" } ] }, { "text": "使用造影剂一般选择非离子造影剂为宜,参考剂量为1~2ml/kg。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "造影剂" }, { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "非离子造影剂" } ] }, { "text": "瑞氏综合征@第十七章瑞氏综合征瑞氏综合征(Reye综合征,Reye’ssyndrome,RS),又名脑病伴内脏脂肪变性,由澳大利亚小儿病理学家Reye等于1963年首先报道,以急性脑病和肝脏脂肪变性为主要临床特征。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "Reye综合征" }, { "start_idx": 29, "end_idx": 42, "type": "dis", "entity": "Reye’ssyndrome" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "RS" }, { "start_idx": 50, "end_idx": 58, "type": "dis", "entity": "脑病伴内脏脂肪变性" }, { "start_idx": 88, "end_idx": 91, "type": "sym", "entity": "急性脑病" }, { "start_idx": 93, "end_idx": 98, "type": "sym", "entity": "肝脏脂肪变性" } ] }, { "text": "瑞氏综合征@RS是儿科的一种危重疾患,常在前驱病毒感染后发生,服用水杨酸制剂和其发病密切相关。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "RS" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "病毒感染" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "水杨酸制剂" } ] }, { "text": "瑞氏综合征@常见表现为急性颅内压增高意识障碍和惊厥等脑病症状,并出现肝功能异常、低血糖、高氨血症和其他代谢紊乱。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "颅内压" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "急性颅内压增高" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "意识障碍" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "惊厥" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "肝功能异常" }, { "start_idx": 40, "end_idx": 42, "type": "sym", "entity": "低血糖" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "高氨血症" } ] }, { "text": "瑞氏综合征@多数病例预后不良,因严重颅内压增高及脑疝致死,或遗留严重的神经系统后遗症。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "严重颅内压增高" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "脑疝" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "神经系统后遗症" } ] }, { "text": "瑞氏综合征@近年来国内外报告确诊病例很少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" } ] }, { "text": "瑞氏综合征@这一方面可能和水杨酸应用减少有关,更主要的则是临床对遗传代谢病的认识和诊断水平提高的结果。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "水杨酸" } ] }, { "text": "瑞氏综合征@很多符合RS临床诊断条件的患儿,最后确诊为脂肪酸或其他代谢障碍,实际上为“类Reye综合征”(Reye’s-likesyndromes)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "RS" }, { "start_idx": 27, "end_idx": 36, "type": "sym", "entity": "脂肪酸或其他代谢障碍" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "类Reye综合征" }, { "start_idx": 53, "end_idx": 72, "type": "sym", "entity": "Reye’s-likesyndromes" } ] }, { "text": "瑞氏综合征@已证实可表现为瑞氏综合征的遗传代谢病包括尿素循环障碍、某些亚型糖原累积病、原发性肉碱缺陷、遗传果糖不耐症、甲基丙二酸血症、3-羟-3-戊二酶血症及脂肪酸β氧化缺陷等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "尿素循环障碍" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "亚型糖原累积病" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "原发性肉碱缺陷" }, { "start_idx": 51, "end_idx": 57, "type": "dis", "entity": "遗传果糖不耐症" }, { "start_idx": 59, "end_idx": 65, "type": "dis", "entity": "甲基丙二酸血症" }, { "start_idx": 67, "end_idx": 77, "type": "dis", "entity": "3-羟-3-戊二酶血症" }, { "start_idx": 79, "end_idx": 86, "type": "dis", "entity": "脂肪酸β氧化缺陷" } ] }, { "text": "瑞氏综合征@【流行病学】美国18岁以下人群发病率为0.1/10万~0.88/10万,部分地区发病率曾高达2.4/10万~8.4/10万。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" } ] }, { "text": "瑞氏综合征@20世纪60年代以来,美国疾病控制中心(CDC)登记病例数达3000例以上,死亡率达26%~42%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" } ] }, { "text": "瑞氏综合征@从1967—1973年,年报告病例数为11~83。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" } ] }, { "text": "瑞氏综合征@在1974—1983年,报告病例明显增加,其中仅1979—1980年就达555例。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" } ] }, { "text": "瑞氏综合征@此后由于慎用阿司匹林,发病人数逐渐下降,死亡率也下降至10%~20%,目前Reye综合征已经十分罕见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "Reye综合征" } ] }, { "text": "瑞氏综合征@近年来RS在澳大利亚和新西兰等国也几乎消失。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "RS" } ] }, { "text": "瑞氏综合征@我国自1973年以来,广州、上海、福建、贵州、北京及湖南等地均有RS的报道,迄今已报道200余例,其中约100例经尸检证实。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "RS" } ] }, { "text": "瑞氏综合征@与欧美RS的临床特点不同,国内报告的RS患儿不一定有病毒感染病史,与阿司匹林的应用也无明显相关。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "欧美RS" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "RS" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "病毒感染" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "阿司匹林" } ] }, { "text": "瑞氏综合征@【病因和发病机制】RS的病因和发病机制迄今未明。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "RS" } ] }, { "text": "瑞氏综合征@研究发现RS病人存在线粒体形态异常肝脏线粒体内酶活性降低线粒体外酶活性保持正常血清中线粒体型GOT增加尿中二羧酸增加急性脂肪酸β氧化紊乱。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "RS" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "线粒体" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "线粒体形态异常" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肝脏" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "线粒体内酶" }, { "start_idx": 23, "end_idx": 33, "type": "sym", "entity": "肝脏线粒体内酶活性降低" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "线粒体外酶" }, { "start_idx": 34, "end_idx": 44, "type": "sym", "entity": "线粒体外酶活性保持正常" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "血清" }, { "start_idx": 48, "end_idx": 54, "type": "bod", "entity": "线粒体型GOT" }, { "start_idx": 45, "end_idx": 56, "type": "sym", "entity": "血清中线粒体型GOT增加" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "尿" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "二羧酸" }, { "start_idx": 57, "end_idx": 63, "type": "sym", "entity": "尿中二羧酸增加" }, { "start_idx": 64, "end_idx": 73, "type": "dis", "entity": "急性脂肪酸β氧化紊乱" } ] }, { "text": "瑞氏综合征@临床观察也发现RS的症状类似于伴有线粒体异常的遗传代谢疾病,而线粒体抑制剂或毒素(如柳酸盐及棘皮油等)可引起类似的临床病理改变。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "RS" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "线粒体" }, { "start_idx": 37, "end_idx": 42, "type": "dru", "entity": "线粒体抑制剂" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "柳酸盐" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "棘皮油" } ] }, { "text": "瑞氏综合征@因此多数学者认为本病与病毒感染或其他因素诱发的线粒体损伤有关。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "病毒感染" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "线粒体损伤" } ] }, { "text": "瑞氏综合征@国外证实本病的发生与B型流感和水痘等病毒感染的流行有关。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "B型流感" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "水痘" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "病毒感染" } ] }, { "text": "瑞氏综合征@【病理】RS的病理改变主要表现在脑和肝脏。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "RS" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "脑" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肝脏" } ] }, { "text": "瑞氏综合征@脑的病理改变主要是脑水肿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "脑" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "脑水肿" } ] }, { "text": "瑞氏综合征@外观肿胀,重量增加,脑回变平脑沟变浅瑞氏综合征@可见枕骨大孔小脑幕切迹疝瑞氏综合征@光镜下可见神经元损伤脑水肿和脑缺血的继发性病变。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "外观肿胀" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "重量增加" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "脑回" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "脑回变平" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "脑沟" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "脑沟变浅" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "枕骨" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "枕骨大孔" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "小脑" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "小脑幕切迹疝" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 48, "end_idx": 49, "type": "equ", "entity": "光镜" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "神经元" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "神经元损伤" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "脑水肿" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "脑缺血" } ] }, { "text": "瑞氏综合征@电镜下可见弥漫性神经元线粒体肿胀瑞氏综合征@星形胶质细胞水肿颗粒减少,并有空泡。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 6, "end_idx": 7, "type": "equ", "entity": "电镜" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "神经元线粒体" }, { "start_idx": 11, "end_idx": 21, "type": "sym", "entity": "弥漫性神经元线粒体肿胀" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "胶质细胞" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "星形胶质细胞水肿" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "颗粒减少" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "并有空泡" } ] }, { "text": "瑞氏综合征@肝脏外观呈浅黄至白色脂肪含量增加。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肝脏" }, { "start_idx": 6, "end_idx": 15, "type": "sym", "entity": "肝脏外观呈浅黄至白色" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "脂肪含量增加" } ] }, { "text": "瑞氏综合征@光镜下可见肝细胞脂肪变性瑞氏综合征@电镜检查可见线粒体肿胀和变形线粒体嵴可消失,肝细胞浆中可见许多细小的脂肪滴瑞氏综合征@肝活检发现上述典型的线粒体改变是确定诊断的重要病理依据。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 6, "end_idx": 7, "type": "equ", "entity": "光镜" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "肝细胞" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "肝细胞脂肪变性" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 24, "end_idx": 25, "type": "equ", "entity": "电镜" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "线粒体" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "线粒体肿胀和变形" }, { "start_idx": 38, "end_idx": 44, "type": "sym", "entity": "线粒体嵴可消失" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "肝细胞浆" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "脂肪滴" }, { "start_idx": 46, "end_idx": 60, "type": "sym", "entity": "肝细胞浆中可见许多细小的脂肪滴" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 67, "end_idx": 69, "type": "pro", "entity": "肝活检" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "线粒体" } ] }, { "text": "瑞氏综合征@【临床表现】典型RS呈“双相期”疾病特征。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "典型RS" } ] }, { "text": "瑞氏综合征@患儿常先有前驱期感染(如流感),可伴低热、咳嗽及流涕等症状。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "流感" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "低热" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "咳嗽" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "流涕" } ] }, { "text": "瑞氏综合征@3~7天后突发高烧、频繁呕吐、惊厥和意识障碍(昏睡或昏迷)等脑病症状瑞氏综合征@重症常有呼吸节律不整等中枢性呼吸衰竭症状,危重者可出现去皮层或去大脑强直。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "高烧" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "频繁呕吐" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "惊厥" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "意识障碍" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "昏睡" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "昏迷" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "脑" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "脑病症状" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 50, "end_idx": 55, "type": "sym", "entity": "呼吸节律不整" }, { "start_idx": 57, "end_idx": 65, "type": "sym", "entity": "中枢性呼吸衰竭症状" }, { "start_idx": 73, "end_idx": 81, "type": "sym", "entity": "去皮层或去大脑强直" } ] }, { "text": "瑞氏综合征@常伴循环紊乱、低血糖或腹泻等症状。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "循环紊乱" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "低血糖" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "腹泻" } ] }, { "text": "瑞氏综合征@常伴肝脏肿大质地韧或硬,一般不伴黄疸(表16-16)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肝脏" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "肝脏肿大" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "质地韧或硬" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "黄疸" } ] }, { "text": "瑞氏综合征@表16-13临床表现根据病程进展,美国国立卫生研究院(NIH)于1982年将RS分为5级。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" } ] }, { "text": "瑞氏综合征@一般于1~2天内由Ⅰ级进展至Ⅴ级,危重者多于起病后数日内死亡,重型RS来势凶猛,发展迅速,甚至在24小时内死亡。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "重型RS" } ] }, { "text": "瑞氏综合征@存活者于病情好转后多在2~3日内恢复。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" } ] }, { "text": "瑞氏综合征@重型,特别是婴儿存活病例中,脑病后遗症可占1/3~2/3,一般无肝脏后遗症。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "脑病" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "肝脏" } ] }, { "text": "瑞氏综合征@【辅助检查】1.肝功能血清谷草转氨酶、谷丙转氨酶以及肌酸磷酸激酶(CK)在病后明显上升,多于1周内恢复正常。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "辅助检查" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "肝" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "血清" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "谷草转氨酶" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "谷丙转氨酶" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "肌酸磷酸激酶" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "CK" } ] }, { "text": "瑞氏综合征@血氨明显增高瑞氏综合征@凝血酶原降低瑞氏综合征@2.其他代谢紊乱血清游离脂酸浓度上升瑞氏综合征@尿及血清中出现二羧酸。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血氨" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "血氨明显增高" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "凝血酶原降低" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 38, "end_idx": 43, "type": "bod", "entity": "血清游离脂酸" }, { "start_idx": 38, "end_idx": 47, "type": "sym", "entity": "血清游离脂酸浓度上升" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "尿" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "血清" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "二羧酸" } ] }, { "text": "瑞氏综合征@婴幼儿常出现低血糖。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "低血糖" } ] }, { "text": "瑞氏综合征@可出现低肉碱血症、低胆固醇血症、低脂蛋白血症和二羧酸血(尿)症。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "低肉碱血症" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "低胆固醇血症" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "低脂蛋白血症" }, { "start_idx": 29, "end_idx": 36, "type": "dis", "entity": "二羧酸血(尿)症" } ] }, { "text": "瑞氏综合征@血乳酸及丙酮酸增高瑞氏综合征@3.脑脊液压力多明显增高,脑脊液常规检查大多正常,低血糖明显者糖含量相应降低瑞氏综合征@4.肝活检可发现典型的RS肝病表现。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "血乳酸" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "丙酮酸" }, { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "血乳酸及丙酮酸增高" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "脑脊液" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "压力多明显增高" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "脑脊液" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "低血糖" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "糖" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "糖含量相应降低" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 67, "end_idx": 69, "type": "pro", "entity": "肝活检" }, { "start_idx": 76, "end_idx": 79, "type": "dis", "entity": "RS肝病" } ] }, { "text": "瑞氏综合征@【诊断】目前临床最常用的标准为美国疾病控制中心(CDC)所制定的诊断标准。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" } ] }, { "text": "瑞氏综合征@主要包括:①急性非炎症性脑病(意识障碍,CSF除外中枢神经系统感染,或组织学证实)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "急性非炎症性脑病" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "意识障碍" }, { "start_idx": 26, "end_idx": 38, "type": "sym", "entity": "CSF除外中枢神经系统感染" } ] }, { "text": "瑞氏综合征@②血清GOT、GPT增高2~3倍以上血氨增高,急性脂肪肝。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "血清GOT" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "GPT" }, { "start_idx": 7, "end_idx": 23, "type": "sym", "entity": "血清GOT、GPT增高2~3倍以上" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "血氨增高" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "急性脂肪肝" } ] }, { "text": "瑞氏综合征@可伴有血乳酸及丙酮酸增高凝血酶原降低CK升高婴幼儿常出现低血糖。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "血乳酸" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "丙酮酸" }, { "start_idx": 9, "end_idx": 17, "type": "sym", "entity": "血乳酸及丙酮酸增高" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "凝血酶原降低" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "CK" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "CK升高" }, { "start_idx": 28, "end_idx": 36, "type": "sym", "entity": "婴幼儿常出现低血糖" } ] }, { "text": "瑞氏综合征@③除外其他类似疾病,如急性中毒、遗传代谢病以及暴发性肝炎等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "急性中毒" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "暴发性肝炎" } ] }, { "text": "瑞氏综合征@如符合上述临床诊断标准而未做肝活检或尸检者称为临床诊断的RS(CRS)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "肝活检" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "RS" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "CRS" } ] }, { "text": "瑞氏综合征@如果肝活检或尸检符合RS诊断标准,则称之为确诊的RS(DRS)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "肝活检" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "尸检" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "RS" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "RS" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "DRS" } ] }, { "text": "瑞氏综合征@RS的症状可发生于很多类似于RS的疾病。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "RS" } ] }, { "text": "瑞氏综合征@因此需鉴别除外的疾病很多,如急性中枢神经系统感染、中毒性脑病以及遗传代谢病等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 20, "end_idx": 29, "type": "dis", "entity": "急性中枢神经系统感染" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "中毒性脑病" } ] }, { "text": "瑞氏综合征@由于上述RS诊断标准是非特异性的,甚至光镜呈“急性脂肪肝”,也无特异性,因此应尽可能行肝活检,电镜下观察肝细胞线粒体的改变以确定诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "RS" }, { "start_idx": 25, "end_idx": 26, "type": "equ", "entity": "光镜" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "急性脂肪肝" }, { "start_idx": 49, "end_idx": 51, "type": "pro", "entity": "肝活检" }, { "start_idx": 53, "end_idx": 54, "type": "equ", "entity": "电镜" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "肝细胞" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "线粒体" } ] }, { "text": "瑞氏综合征@肝活检应争取在起病后4~5日内进行,尸检标本不适于线粒体形态学或有关代谢的检查。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "肝活检" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "尸检" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "线粒体" } ] }, { "text": "瑞氏综合征@【治疗】本病预后不良,早期认识轻症患儿并给予及时治疗是争取改善预后的关键。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" } ] }, { "text": "瑞氏综合征@一旦出现严重的意识障碍则有很高的死亡率,幸存者也往往出现严重的神经系统后遗症。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" } ] }, { "text": "瑞氏综合征@迄今对RS的治疗尚缺乏特效疗法,以病情监护以及维持内环境稳定、降低颅内压、止惊、控制低血糖控制低血糖和凝血障碍等对症治疗为主。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "RS" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "颅内压" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "止惊" }, { "start_idx": 46, "end_idx": 50, "type": "pro", "entity": "控制低血糖" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "凝血障碍" } ] }, { "text": "瑞氏综合征@控制致命的脑水肿最为关键,可合用甘露醇、呋塞米及皮质激素。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "脑水肿" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "甘露醇" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "呋塞米" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "皮质激素" } ] }, { "text": "瑞氏综合征@适量输入葡萄糖和胰岛素,补充肉碱及瓜氨酸有助于缓解肝病进展。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "胰岛素" }, { "start_idx": 20, "end_idx": 21, "type": "dru", "entity": "肉碱" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "瓜氨酸" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "肝病" } ] }, { "text": "瑞氏综合征@(秦炯)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "瑞氏综合征" } ] }, { "text": "第五章尿路感染尿路感染(urinarytractinfection,UTI)简称尿感,是指病原微生物入侵泌尿系统并在尿中繁殖,侵入泌尿道黏膜或组织引起炎症反应。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "尿路感染" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "尿路感染" }, { "start_idx": 12, "end_idx": 32, "type": "dis", "entity": "urinarytractinfection" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "UTI" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "尿感" }, { "start_idx": 45, "end_idx": 49, "type": "mic", "entity": "病原微生物" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "尿" }, { "start_idx": 65, "end_idx": 69, "type": "bod", "entity": "泌尿道黏膜" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "组织" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "炎症反应" } ] }, { "text": "根据1986年全国21个省市224291例健康小儿尿筛查的结果,尿感发病率为8.25%。", "entities": [ { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "尿感" } ] }, { "text": "上尿路感染的危害较大,以婴幼儿发病率最高,反复感染可形成肾疤痕,严重者可致继发性高血压和慢性肾衰竭。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "尿路感染" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "感染" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肾疤痕" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "继发性高血压" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "慢性肾衰竭" } ] }, { "text": "【病因和发病机制】(一)病因各种病原微生物即为尿感的病因,大肠杆菌占75%~90%,其次为肺炎克雷伯杆菌、变形杆菌、产气杆菌和产碱杆菌,近年来革兰阳性球菌的比例升高,如肠链球菌和葡萄球菌。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "mic", "entity": "病原微生物" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "尿感" }, { "start_idx": 29, "end_idx": 32, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 45, "end_idx": 51, "type": "mic", "entity": "肺炎克雷伯杆菌" }, { "start_idx": 53, "end_idx": 56, "type": "mic", "entity": "变形杆菌" }, { "start_idx": 58, "end_idx": 61, "type": "mic", "entity": "产气杆菌" }, { "start_idx": 63, "end_idx": 66, "type": "mic", "entity": "产碱杆菌" }, { "start_idx": 71, "end_idx": 76, "type": "mic", "entity": "革兰阳性球菌" }, { "start_idx": 84, "end_idx": 87, "type": "mic", "entity": "肠链球菌" }, { "start_idx": 89, "end_idx": 92, "type": "mic", "entity": "葡萄球菌" } ] }, { "text": "(二)发病机制主要通过上行和血行感染,邻近器官感染的直接侵犯少见。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "上行和血行感染" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "邻近器官感染" } ] }, { "text": "正常泌尿道通过以下机制有抗感染作用:①定期排尿将细菌冲洗出尿道;②尿中有IgA、溶菌酶及有机酸等抗菌物质;③泌尿道黏膜产生的分泌型IgA及膀胱黏膜移行上皮细胞分泌的黏附分子(mucin),可有效减少细菌的黏附,所以只有在诱因存在,才为易发尿感的原因。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "泌尿道" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "感染" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "尿" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "IgA" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "溶菌酶" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "有机酸" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "泌尿道黏膜" }, { "start_idx": 62, "end_idx": 67, "type": "bod", "entity": "分泌型IgA" }, { "start_idx": 69, "end_idx": 78, "type": "bod", "entity": "膀胱黏膜移行上皮细胞" }, { "start_idx": 87, "end_idx": 91, "type": "bod", "entity": "mucin" }, { "start_idx": 99, "end_idx": 100, "type": "mic", "entity": "细菌" }, { "start_idx": 119, "end_idx": 120, "type": "dis", "entity": "尿感" } ] }, { "text": "1.上行感染正常小儿尿道有少许细菌存在,当机体抵抗力下降或尿道黏膜损伤时,细菌可入侵或沿尿道上行,引起膀胱、肾盂和肾间质的感染。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "上行感染" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "尿道" }, { "start_idx": 15, "end_idx": 16, "type": "mic", "entity": "细菌" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "抵抗力下降" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "尿道黏膜损伤" }, { "start_idx": 37, "end_idx": 38, "type": "mic", "entity": "细菌" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "尿道上行" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "膀胱" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "肾盂" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "肾间质" }, { "start_idx": 51, "end_idx": 62, "type": "sym", "entity": "膀胱、肾盂和肾间质的感染" } ] }, { "text": "正常输尿管蠕动可使尿液注入膀胱。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "输尿管" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "尿液" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "膀胱" } ] }, { "text": "女孩尿道短,上行感染机会比男孩多。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "尿道" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "上行感染" } ] }, { "text": "婴儿用尿布,外阴容易受粪便污染是婴幼儿容易发生上行性感染的原因之一,以下因素可促发上行感染:(1)小儿解剖生理特点:小儿输尿管长而弯曲,管壁肌肉弹力纤维发育不全,蠕动力弱,易于扩张,尿流不畅。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "外阴" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "上行性感染" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "上行感染" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "输尿管" }, { "start_idx": 68, "end_idx": 75, "type": "bod", "entity": "管壁肌肉弹力纤维" }, { "start_idx": 58, "end_idx": 94, "type": "sym", "entity": "小儿输尿管长而弯曲,管壁肌肉弹力纤维发育不全,蠕动力弱,易于扩张,尿流不畅" } ] }, { "text": "(2)膀胱输尿管反流(vesico-ureteralreflux,VUR):输尿管分腹段、盆腔段和膀胱段,正常输尿管进入膀胱呈一钝角,向内向下进入膀胱壁有一定的斜度和长度,输尿管膀胱连接处是由肌肉筋膜鞘组成,输尿管膀胱开口呈斜行裂隙状,起瓣膜作用。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 11, "end_idx": 31, "type": "dis", "entity": "vesico-ureteralreflux" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "VUR" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "输尿管" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "腹段" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "盆腔段" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "膀胱段" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "输尿管" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "膀胱" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "膀胱壁" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "输尿管" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "膀胱" }, { "start_idx": 96, "end_idx": 100, "type": "bod", "entity": "肌肉筋膜鞘" }, { "start_idx": 104, "end_idx": 106, "type": "bod", "entity": "输尿管" }, { "start_idx": 107, "end_idx": 108, "type": "bod", "entity": "膀胱" } ] }, { "text": "当输尿管进入膀胱的角度改变、在膀胱壁内行程太短、输尿管末端环形和纵行肌纤维数量和分布异常或输尿管膀胱开口的先天异常,以及脊髓脊膜膨出所致的神经源性膀胱均可引起输尿管膀胱反流。", "entities": [ { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "在膀胱壁内行程太短" }, { "start_idx": 24, "end_idx": 38, "type": "ite", "entity": "输尿管末端环形和纵行肌纤维数量" }, { "start_idx": 24, "end_idx": 43, "type": "sym", "entity": "输尿管末端环形和纵行肌纤维数量和分布异常" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "输尿管膀胱" }, { "start_idx": 45, "end_idx": 56, "type": "sym", "entity": "输尿管膀胱开口的先天异常" }, { "start_idx": 69, "end_idx": 74, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 79, "end_idx": 85, "type": "dis", "entity": "输尿管膀胱反流" } ] }, { "text": "婴儿期下尿道神经发育不成熟,在膀胱充盈期和排尿期产生的逼尿肌功能亢进,使膀胱内压增高,改变了膀胱壁和输尿管交界处的解剖关系也引起VUR,但为暂时性。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "尿道神经" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "膀胱" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "逼尿肌" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "逼尿肌功能亢进" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "膀胱内压增高" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "膀胱壁" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "输尿管" }, { "start_idx": 64, "end_idx": 66, "type": "dis", "entity": "VUR" } ] }, { "text": "尿液反流分5级。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尿液反流" } ] }, { "text": "Ⅰ级:反流仅见于输尿管;Ⅱ级:反流至肾盂及肾盏;Ⅲ级:输尿管轻~中度扩张与扭曲,肾盏中度扩张,穹隆无或轻度变钝;Ⅳ级:输尿管中度扩张,穹隆角完全消失;Ⅴ级:输尿管显著扩张与扭曲,肾盂肾盏显著扩张,多数肾盏不见乳头压迹。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "输尿管" }, { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "反流仅见于输尿管" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肾盂" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肾盏" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "反流至肾盂及肾盏" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "输尿管" }, { "start_idx": 27, "end_idx": 38, "type": "sym", "entity": "输尿管轻~中度扩张与扭曲" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "肾盏" }, { "start_idx": 40, "end_idx": 45, "type": "sym", "entity": "肾盏中度扩张" }, { "start_idx": 47, "end_idx": 54, "type": "sym", "entity": "穹隆无或轻度变钝" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "输尿管" }, { "start_idx": 59, "end_idx": 65, "type": "sym", "entity": "输尿管中度扩张" }, { "start_idx": 67, "end_idx": 73, "type": "sym", "entity": "穹隆角完全消失" }, { "start_idx": 78, "end_idx": 87, "type": "sym", "entity": "输尿管显著扩张与扭曲" }, { "start_idx": 89, "end_idx": 92, "type": "bod", "entity": "肾盂肾盏" }, { "start_idx": 89, "end_idx": 96, "type": "sym", "entity": "肾盂肾盏显著扩张" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "肾盏" }, { "start_idx": 100, "end_idx": 107, "type": "sym", "entity": "肾盏不见乳头压迹" } ] }, { "text": "尿液反流的危害在于可造成上行性尿感反复发作,引起肾疤痕,而且,Ⅲ级以上的尿液反流也可因肾盏内压力过高引起肾内反流和肾间质损害。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尿液反流" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "上行性尿感" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "肾疤痕" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "尿液反流" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "肾盏" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "肾盏内压力过高" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "肾内反流" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "肾间质损害" } ] }, { "text": "两者同时或单独存在均可致慢性肾衰竭。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "慢性肾衰竭" } ] }, { "text": "(3)其他先天畸形和尿路梗阻:如肾盂输尿管连接处狭窄、肾盂积水、后尿道瓣膜及多囊肾等均可使引流不畅而继发感染。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "先天畸形" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "尿路梗阻" }, { "start_idx": 16, "end_idx": 23, "type": "bod", "entity": "肾盂输尿管连接处" }, { "start_idx": 16, "end_idx": 25, "type": "sym", "entity": "肾盂输尿管连接处狭窄" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肾盂" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "肾盂积水" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "后尿道" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "后尿道瓣膜" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "多囊肾" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "继发感染" } ] }, { "text": "此外,还可由神经源性膀胱、结石及肿瘤等引起梗阻。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "结石" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "肿瘤" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "梗阻" } ] }, { "text": "(4)病原菌的致病力也是影响尿感的主要因素,以大肠杆菌为例,其菌体抗原和荚膜抗原K是决定大肠杆菌尿路致病性的必要条件。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "mic", "entity": "病原菌" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "尿感" }, { "start_idx": 23, "end_idx": 26, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 44, "end_idx": 47, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 44, "end_idx": 52, "type": "sym", "entity": "大肠杆菌尿路致病性" } ] }, { "text": "此外,大肠杆菌菌体表面有许多P菌毛,能表达黏附素。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "mic", "entity": "大肠杆菌菌体" } ] }, { "text": "能特异地与泌尿道上皮细胞表面的特异受体结合,使菌体紧密黏附于泌尿道上皮,避免被尿液冲洗,得以在局部繁殖,引起上行感染。", "entities": [ { "start_idx": 5, "end_idx": 18, "type": "bod", "entity": "泌尿道上皮细胞表面的特异受体" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "泌尿道上皮" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "上行感染" } ] }, { "text": "2.血行感染在败血症或其他病灶引起的菌血症时,细菌经血流进入肾皮质和肾盂引起尿感。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "血行感染" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "败血症" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "菌血症" }, { "start_idx": 23, "end_idx": 24, "type": "mic", "entity": "细菌" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "肾皮质" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肾盂" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "尿感" } ] }, { "text": "血行感染以新生儿多见。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "血行感染" } ] }, { "text": "【临床表现】因年龄和尿感部位不同而异,主要有三种表现形式:即肾盂肾炎、膀胱炎和无症状性菌尿。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "尿感" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "膀胱炎" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "无症状性菌尿" } ] }, { "text": "(一)肾盂肾炎婴幼儿占多数,以全身感染中毒症状为主要表现,常有38.5℃以上的发热,高热时可有惊厥或寒战。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "全身感染中毒症状" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "发热" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "高热" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "惊厥" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "寒战" } ] }, { "text": "(二)膀胱炎大多为年长女孩,有尿频、尿急、排尿困难、排尿不尽、下腹不适、耻骨上区疼痛及尿失禁的症状,有时尿恶臭,有外阴部湿疹。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "膀胱炎" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "尿频" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "尿急" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "排尿困难" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "排尿不尽" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "下腹" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "下腹不适" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "耻骨上区" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "耻骨上区疼痛" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "尿" }, { "start_idx": 43, "end_idx": 45, "type": "sym", "entity": "尿失禁" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "尿" }, { "start_idx": 52, "end_idx": 54, "type": "sym", "entity": "尿恶臭" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "外阴部" }, { "start_idx": 57, "end_idx": 61, "type": "sym", "entity": "外阴部湿疹" } ] }, { "text": "膀胱炎一般不引起发热。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "膀胱炎" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "发热" } ] }, { "text": "(三)无症状性菌尿无症状性菌尿指小儿尿培养阳性而无任何感染的临床症状。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "无症状性菌尿" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "无症状性菌尿" }, { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "尿培养" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "尿培养阳性" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "感染" } ] }, { "text": "几乎全是女孩,但若不治疗可能发展为有症状的尿路感染。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "尿路感染" } ] }, { "text": "【实验室检查】(一)血液检查急性肾盂肾炎常有血白细胞总数和中性粒细胞比例明显增高、血沉增快、C反应蛋白>20mg/L。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "血液检查" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "急性肾盂肾炎" }, { "start_idx": 22, "end_idx": 27, "type": "ite", "entity": "血白细胞总数" }, { "start_idx": 29, "end_idx": 33, "type": "ite", "entity": "中性粒细胞" }, { "start_idx": 22, "end_idx": 39, "type": "sym", "entity": "血白细胞总数和中性粒细胞比例明显增高" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "血沉增快" }, { "start_idx": 46, "end_idx": 50, "type": "ite", "entity": "C反应蛋白" }, { "start_idx": 46, "end_idx": 57, "type": "sym", "entity": "C反应蛋白>20mg/L" } ] }, { "text": "膀胱炎时上述实验指标多正常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "膀胱炎" } ] }, { "text": "(二)尿常规检查清洁中段尿离心镜检中WBC≥5/HP提示尿路感染,若见白细胞管型,提示肾盂肾炎。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "尿常规检查" }, { "start_idx": 8, "end_idx": 16, "type": "pro", "entity": "清洁中段尿离心镜检" }, { "start_idx": 18, "end_idx": 25, "type": "sym", "entity": "WBC≥5/HP" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "尿路感染" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "白细胞管型" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "肾盂肾炎" } ] }, { "text": "肾乳头或膀胱炎可有明显血尿。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "膀胱炎" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "血尿" } ] }, { "text": "尿路炎症严重者,可有短暂明显的蛋白尿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尿路炎症" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "短暂明显的蛋白尿" } ] }, { "text": "部分患儿可有血尿或终末血尿。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "血尿" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "终末血尿" } ] }, { "text": "(三)细菌学检查尿培养因可受前尿道和尿道周围杂菌的污染,故需在治疗前做清洁中段尿培养及菌落计数,若菌落计数≥105/ml有诊断意义,104~105/ml为可疑。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "细菌学检查" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "尿培养" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "前尿道" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "尿道" }, { "start_idx": 35, "end_idx": 41, "type": "ite", "entity": "清洁中段尿培养" }, { "start_idx": 43, "end_idx": 46, "type": "ite", "entity": "菌落计数" }, { "start_idx": 49, "end_idx": 52, "type": "ite", "entity": "菌落计数" } ] }, { "text": "但已有膀胱炎尿路刺激症状的患者,尿白细胞明显增多,尿培养菌落计数为103~104/ml亦应考虑尿感的诊断,此外,某些革兰阳性球菌如肠链球菌分裂慢,如为103/ml亦可诊断尿感。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "膀胱炎尿路" }, { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "膀胱炎尿路刺激症状" }, { "start_idx": 16, "end_idx": 19, "type": "ite", "entity": "尿白细胞" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "尿白细胞明显增多" }, { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "尿培养菌落计数" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "尿感" }, { "start_idx": 70, "end_idx": 75, "type": "mic", "entity": "革兰阳性球菌" }, { "start_idx": 77, "end_idx": 80, "type": "mic", "entity": "肠链球菌" }, { "start_idx": 103, "end_idx": 104, "type": "dis", "entity": "尿感" } ] }, { "text": "对婴幼儿和新生儿以及怀疑尿感而留尿困难的小儿,可作耻骨上膀胱穿刺培养,阳性培养即有诊断意义。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "尿感" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "留尿困难" }, { "start_idx": 25, "end_idx": 33, "type": "pro", "entity": "耻骨上膀胱穿刺培养" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "阳性培养" } ] }, { "text": "留做细菌培养的尿若不能及时送验时,应暂放4℃冰箱内,否则会影响结果。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "细菌培养" } ] }, { "text": "有发热的尿感应同时做血培养。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "发热" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "尿感" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "血培养" } ] }, { "text": "大量利尿或已应用抗菌治疗则影响尿培养的结果。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "抗菌治疗" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "尿培养" } ] }, { "text": "尿培养如阳性,应作药物敏感试验,指导治疗。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "尿培养" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "药物敏感试验" } ] }, { "text": "(四)尿直接涂片找细菌用一滴均匀新鲜尿液置玻片上烘干,用美蓝或革兰染色,在高倍或油镜下每视野若见到细菌≥1个,表示尿内菌落计数>105/ml。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "尿直接涂片找细菌" }, { "start_idx": 21, "end_idx": 22, "type": "equ", "entity": "玻片" }, { "start_idx": 37, "end_idx": 41, "type": "equ", "entity": "高倍或油镜" }, { "start_idx": 57, "end_idx": 62, "type": "ite", "entity": "尿内菌落计数" } ] }, { "text": "根据尿沉渣涂片革兰染色及细菌形态,可作为选用药物治疗的参考。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "尿沉渣涂片革兰染色" } ] }, { "text": "(五)菌尿辅助检查尿液亚硝酸盐还原试验,可作为过筛检查,阳性率可达80%。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "菌尿辅助检查" }, { "start_idx": 9, "end_idx": 18, "type": "pro", "entity": "尿液亚硝酸盐还原试验" } ] }, { "text": "(六)肾小管损伤的其他实验室指标尿β2-微球蛋白及尿N-乙酰-β-D-葡萄糖苷酶(NAG)增高,尿渗透压降低提示肾盂肾炎。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肾小管损伤" }, { "start_idx": 16, "end_idx": 29, "type": "ite", "entity": "尿β2-微球蛋白" }, { "start_idx": 31, "end_idx": 45, "type": "ite", "entity": "尿N-乙酰-β-D-葡萄糖苷酶" }, { "start_idx": 47, "end_idx": 49, "type": "ite", "entity": "NAG" }, { "start_idx": 16, "end_idx": 52, "type": "sym", "entity": "尿β2-微球蛋白及尿N-乙酰-β-D-葡萄糖苷酶(NAG)增高" }, { "start_idx": 54, "end_idx": 59, "type": "sym", "entity": "尿渗透压降低" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "肾盂肾炎" } ] }, { "text": "(七)影像学检查1.B超检查可探查泌尿系的结构和膀胱排泄功能有无异常,有无结石、梗阻及残余尿等引起感染诱因。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "影像学检查" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "B超检查" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "泌尿系" }, { "start_idx": 24, "end_idx": 29, "type": "ite", "entity": "膀胱排泄功能" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "结石" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "梗阻" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "残余尿" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "感染" } ] }, { "text": "2.X线检查静脉肾盂造影可显示泌尿系统有无先天畸形(如重肾和多囊肾等)、肾盂积水及其程度。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "X线检查" }, { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "静脉肾盂造影" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "先天畸形" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "重肾" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "多囊肾" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肾盂" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "肾盂积水" } ] }, { "text": "了解肾的大小,有无肾盂肾盏变形等慢性炎症和肾疤痕证据。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肾" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "肾盂肾盏" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "肾盂肾盏变形" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "慢性炎症" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "肾疤痕" } ] }, { "text": "3.核素检查同位素锝-99m二巯基丁二酸(99mTc-dimercaptosuccinicacid,DMSA)肾静态显像可作为上尿路感染诊断的可靠指标,对发现肾盂肾炎的敏感性和特异性均在90%以上。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "核素检查" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "上尿路感染" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "肾盂肾炎" } ] }, { "text": "当急性肾盂肾炎时肾的轮廓正常,由于肾实质的炎性细胞浸润,肾间质水肿及肾小管细胞坏死致DMSA减少,造成病变部位同位素分布的稀疏区,当炎症消散后此种稀疏区可消失。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "急性肾盂肾炎" }, { "start_idx": 17, "end_idx": 26, "type": "sym", "entity": "肾实质的炎性细胞浸润" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "肾间质水肿" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "肾小管细胞" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "肾小管细胞坏死" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "DMSA减少" }, { "start_idx": 51, "end_idx": 63, "type": "sym", "entity": "病变部位同位素分布的稀疏区" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "炎症" } ] }, { "text": "在慢性肾盂肾炎,肾疤痕形成时,病变部位的DMSA摄入更少,且肾外形可因疤痕收缩而缩小或见楔形缺损区。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "慢性肾盂肾炎" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "肾疤痕" }, { "start_idx": 15, "end_idx": 27, "type": "sym", "entity": "病变部位的DMSA摄入更少" }, { "start_idx": 30, "end_idx": 41, "type": "sym", "entity": "肾外形可因疤痕收缩而缩小" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "楔形缺损区" } ] }, { "text": "【诊断和鉴别诊断】患者多有感染或尿路刺激的临床症状,结合尿常规及尿培养菌落计数可以做出诊断。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "感染" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "尿路刺激" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "尿常规" }, { "start_idx": 32, "end_idx": 38, "type": "ite", "entity": "尿培养菌落计数" } ] }, { "text": "1.清洁中段尿,离心镜检中WBC≥5/HP,或有尿感症状;2.中段尿培养菌落计数≥105/ml;3.如无1条,应再做中段尿培养,同一细菌仍≥105/ml,可确诊。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "离心镜检" }, { "start_idx": 8, "end_idx": 20, "type": "sym", "entity": "离心镜检中WBC≥5/HP" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "尿感症状" }, { "start_idx": 31, "end_idx": 39, "type": "ite", "entity": "中段尿培养菌落计数" }, { "start_idx": 64, "end_idx": 68, "type": "pro", "entity": "中段尿培养" } ] }, { "text": "可称无症状性菌尿。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "无症状性菌尿" } ] }, { "text": "尿培养是确定诊断的重要证据,要求在抗生素应用前做,排尿前勿多饮水。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "尿培养" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "抗生素" } ] }, { "text": "留尿过程中要严格按常规操作,以免尿液污染。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "尿液" } ] }, { "text": "尿白细胞管型、血白细胞总数和中性粒细胞比例增高,血沉增快,C反应蛋白升高,提示肾盂肾炎,影像学DMSA检查,确认肾盂肾炎的存在,并了解炎症的范围和程度。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "尿白细胞" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "血白细胞" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 0, "end_idx": 22, "type": "sym", "entity": "尿白细胞管型、血白细胞总数和中性粒细胞比例增高" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "血沉增快" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "C反应蛋白升高" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 44, "end_idx": 52, "type": "pro", "entity": "影像学DMSA检查" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 67, "end_idx": 68, "type": "dis", "entity": "炎症" } ] }, { "text": "不伴泌尿道结构或/和功能异常的尿感为单纯性尿感;伴结构或/和功能异常的尿感为复杂性尿感,后者容易复发或反复感染,造成高血压和慢性肾衰竭的后果。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "泌尿道" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "不伴泌尿道结构或/和功能异常" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "尿感" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "单纯性尿感" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "结构或/和功能异常" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "尿感" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "复杂性尿感" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "感染" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "高血压" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "慢性肾衰竭" } ] }, { "text": "急性肾盂肾炎严重者可合并败血症,特别是在新生儿和有阻塞性肾病者,故有明显感染中毒症状及血白细胞20×109~25×109/L的患者,血培养。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "急性肾盂肾炎" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "败血症" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "阻塞性肾病" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "中毒" }, { "start_idx": 34, "end_idx": 41, "type": "sym", "entity": "明显感染中毒症状" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "血白细胞" }, { "start_idx": 43, "end_idx": 83, "type": "sym", "entity": "血白细胞20×109~25×109/L" }, { "start_idx": 88, "end_idx": 90, "type": "ite", "entity": "血培养" } ] }, { "text": "对一般抗菌治疗无效应和尿细菌培养多次无细菌生长的尿感,尚应结合胸片、OT试验、尿沉渣找抗酸杆菌、结核培养和静脉肾盂造影等除外泌尿系结核。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "抗菌治疗" }, { "start_idx": 11, "end_idx": 15, "type": "ite", "entity": "尿细菌培养" }, { "start_idx": 11, "end_idx": 22, "type": "sym", "entity": "尿细菌培养多次无细菌生长" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "尿感" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "胸片" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "OT试验" }, { "start_idx": 43, "end_idx": 46, "type": "mic", "entity": "抗酸杆菌" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "结核培养" }, { "start_idx": 53, "end_idx": 58, "type": "pro", "entity": "静脉肾盂造影" }, { "start_idx": 61, "end_idx": 66, "type": "dis", "entity": "外泌尿系结核" } ] }, { "text": "经驱虫,加强外阴护理和局部处理可缓解症状,不必口服抗菌药。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "抗菌药" } ] }, { "text": "(二)抗感染治疗1.药物选择细菌性尿感根据尿感的定位诊断及病原选药:①上尿路感染选用血和肾浓度高的药物,下尿路感染选用尿浓度高的药物;②根据检查的病原菌及其药物敏感试验选药;③尽可能用低毒的药物。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "抗感染治疗" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "细菌性尿感" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "尿感" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "上尿路感染" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "下尿路感染" }, { "start_idx": 78, "end_idx": 83, "type": "pro", "entity": "药物敏感试验" } ] }, { "text": "婴幼儿应采取积极有效的治疗,如伴有呕吐及精神萎靡者,建议静脉用药。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "呕吐" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "精神萎靡" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "静脉用药" } ] }, { "text": "头孢类抗生素,特别是第二、三代头孢菌素,有较好的效果,因氨苄西林耐药菌株有增多趋势,已有被安美汀(羟氨苄西林+β内酰胺酶抑制剂克拉维酸)替代趋势。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "头孢类抗生素" }, { "start_idx": 10, "end_idx": 18, "type": "dru", "entity": "第二、三代头孢菌素" }, { "start_idx": 28, "end_idx": 35, "type": "mic", "entity": "氨苄西林耐药菌株" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "安美汀" }, { "start_idx": 49, "end_idx": 66, "type": "dru", "entity": "羟氨苄西林+β内酰胺酶抑制剂克拉维酸" } ] }, { "text": "氨基糖苷类静脉滴注要慎用,时间不可长。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "氨基糖苷类静脉滴注" } ] }, { "text": "喹诺酮类药物抗菌作用较强,但7岁以下小儿慎用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "喹诺酮类药物" } ] }, { "text": "SMZco和呋喃妥因适用于下尿路感染的治疗,一般用药5~7日。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "SMZco" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "呋喃妥" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "下尿路感染" } ] }, { "text": "对真菌引起的尿路感染可用抗真菌药。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "尿路感染" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "抗真菌药" } ] }, { "text": "2.疗程由于儿童膀胱炎和肾盂肾炎临床上不易区分,新生儿和小婴儿尿路感染合并畸形的比例较高,短程疗法,包括单剂量疗法和3天疗法,在儿童中均不宜推广。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "儿童膀胱炎" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 24, "end_idx": 38, "type": "dis", "entity": "新生儿和小婴儿尿路感染合并畸形" }, { "start_idx": 45, "end_idx": 61, "type": "pro", "entity": "短程疗法,包括单剂量疗法和3天疗法" } ] }, { "text": "采用短程疗法的急性尿路感染儿童,其复发率和重新感染的机会均大于2周左右的常规疗法。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "短程疗法" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "急性尿路感染" } ] }, { "text": "只有年龄大于5岁,尿路没有畸形,才考虑采用短程疗法。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "尿路" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "尿路没有畸形" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "短程疗法" } ] }, { "text": "急性初次上尿路感染经有效抗菌治疗,多于2~3日高热渐降,尿常规迅速恢复正常,常规疗程为2周。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "急性初次上尿路感染" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "抗菌治疗" }, { "start_idx": 17, "end_idx": 26, "type": "sym", "entity": "多于2~3日高热渐降" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "尿常规" }, { "start_idx": 28, "end_idx": 36, "type": "sym", "entity": "尿常规迅速恢复正常" } ] }, { "text": "对治疗恢复不顺利者应根据尿培养及药敏试验及时更换抗生素,疗程需4~6周。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "尿培养" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "药敏试验" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "抗生素" } ] }, { "text": "初次尿感痊愈后第1、2、3、6、12个月应随访中段尿培养及菌落计数至少1年。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "尿感" }, { "start_idx": 23, "end_idx": 27, "type": "ite", "entity": "中段尿培养" }, { "start_idx": 29, "end_idx": 32, "type": "ite", "entity": "菌落计数" } ] }, { "text": "3.复发和再感染的治疗急性尿路感染经合理抗菌治疗,多数于数日内症状消失、治愈,但有50%的患儿可有复发,多在治疗后1个月内出现。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "感染" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "急性尿路感染" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "合理抗菌治疗" } ] }, { "text": "常见的原因有:①抗菌药物选择不当,包括未选用针对致病菌敏感的药物和仅选用了肾组织内浓度低的药物,因而达不到有效的杀菌目的。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "mic", "entity": "致病菌" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肾组织" } ] }, { "text": "②出现了耐药菌株,这在初次感染的病人很少见,如初次治疗后72小时症状和菌尿未消失,应及时按药敏结果更换抗生素。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "mic", "entity": "耐药菌株" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "感染" }, { "start_idx": 28, "end_idx": 39, "type": "sym", "entity": "72小时症状和菌尿未消失" }, { "start_idx": 44, "end_idx": 53, "type": "pro", "entity": "按药敏结果更换抗生素" } ] }, { "text": "③L-型细菌,占肾盂肾炎复发的20%,根据其仅能在肾髓质高渗条件下生存,可通过多饮水来降低肾髓质渗透压破坏其生存环境,同时选用红霉素和氯霉素等抑制蛋白质合成的药物重新治疗。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "mic", "entity": "L-型细菌" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肾髓质" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "肾髓质高渗" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "肾髓质" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "降低肾髓质渗透压" }, { "start_idx": 61, "end_idx": 84, "type": "pro", "entity": "选用红霉素和氯霉素等抑制蛋白质合成的药物重新治疗" } ] }, { "text": "④尿路结石。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "尿路结石" } ] }, { "text": "尿路结石的存在可为细菌提供有效的庇护所,逃脱抗菌药物的杀灭作用而得以幸存,常在治疗中止后,成为复发的病因。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尿路结石" }, { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "细菌" } ] }, { "text": "⑤病原菌除大肠杆菌外,变形杆菌是最常见的致病菌。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "mic", "entity": "病原菌" }, { "start_idx": 5, "end_idx": 8, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 11, "end_idx": 14, "type": "mic", "entity": "变形杆菌" }, { "start_idx": 20, "end_idx": 22, "type": "mic", "entity": "致病菌" } ] }, { "text": "在1岁以上的男童,初次感染的致病菌也以变形杆菌为主。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "感染" }, { "start_idx": 14, "end_idx": 16, "type": "mic", "entity": "致病菌" }, { "start_idx": 19, "end_idx": 22, "type": "mic", "entity": "变形杆菌" } ] }, { "text": "对这些病人应按药敏选用抗生素,剂量要大,疗程要长,至少在6周以上。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "抗生素" } ] }, { "text": "⑥如菌尿持续存在或经2次6周以上治疗仍频繁复发,则要选用长程低剂量抑菌疗法,以每晚睡前一次顿服为宜,剂量为常规治疗量的1/3~1/4,药物可选用SMZ+TMP、阿莫西林、头孢氨苄或呋喃妥因等,或两种交替使用,以防产生耐药菌株。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "菌尿持续存在" }, { "start_idx": 9, "end_idx": 22, "type": "sym", "entity": "经2次6周以上治疗仍频繁复发" }, { "start_idx": 28, "end_idx": 36, "type": "pro", "entity": "长程低剂量抑菌疗法" }, { "start_idx": 72, "end_idx": 78, "type": "dru", "entity": "SMZ+TMP" }, { "start_idx": 80, "end_idx": 83, "type": "dru", "entity": "阿莫西林" }, { "start_idx": 85, "end_idx": 88, "type": "dru", "entity": "头孢氨苄" }, { "start_idx": 90, "end_idx": 93, "type": "dru", "entity": "呋喃妥因" }, { "start_idx": 108, "end_idx": 111, "type": "mic", "entity": "耐药菌株" } ] }, { "text": "再感染多发生在初次治疗后1个月以上,常见于女童,占再发性尿路感染的80%。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "感染" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "再发性尿路感染" } ] }, { "text": "再感染均为不同菌株或同一菌株不同血清型的大肠杆菌所引起,常合并有尿路梗阻和膀胱输尿管反流等尿路畸形。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "感染" }, { "start_idx": 20, "end_idx": 23, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "尿路梗阻" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "尿路畸形" } ] }, { "text": "再感染的病人,应首先采用10~14天的常规治疗,如症状和菌尿消失,继之以小剂量抗生素预防重新感染,可供选择的药物有SMZ+TMP、呋喃妥因、阿莫西林或头孢氨苄等,剂量为常规治疗量的1/5~1/4。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "感染" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "抗生素" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "重新感染" }, { "start_idx": 57, "end_idx": 63, "type": "dru", "entity": "SMZ+TMP" }, { "start_idx": 65, "end_idx": 68, "type": "dru", "entity": "呋喃妥因" }, { "start_idx": 70, "end_idx": 73, "type": "dru", "entity": "阿莫西林" }, { "start_idx": 75, "end_idx": 78, "type": "dru", "entity": "头孢氨苄" } ] }, { "text": "如10~14天的常规治疗无效,应延长疗程至6周,有效者继续以小剂量抗生素预防,无效者或当时有效但随后再感染频发,宜选用长程低剂量抑菌疗法,方法同上,疗程至少1年以上,如确诊有尿路畸形,则需用至畸形被矫正或膀胱输尿管反流自行中止后1年为止。", "entities": [ { "start_idx": 30, "end_idx": 37, "type": "pro", "entity": "小剂量抗生素预防" }, { "start_idx": 39, "end_idx": 54, "type": "sym", "entity": "无效者或当时有效但随后再感染频发" }, { "start_idx": 59, "end_idx": 67, "type": "pro", "entity": "长程低剂量抑菌疗法" }, { "start_idx": 87, "end_idx": 90, "type": "dis", "entity": "尿路畸形" }, { "start_idx": 96, "end_idx": 100, "type": "sym", "entity": "畸形被矫正" }, { "start_idx": 102, "end_idx": 106, "type": "bod", "entity": "膀胱输尿管" }, { "start_idx": 102, "end_idx": 112, "type": "sym", "entity": "膀胱输尿管反流自行中止" } ] }, { "text": "4.无症状性菌尿的治疗无症状性菌尿大多不需治疗,因为抗菌治疗并不能降低再感染的发生率。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "无症状性菌尿" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "无症状性菌尿" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "抗菌治疗" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "感染" } ] }, { "text": "不过,如果患儿合并有尿路梗阻、膀胱输尿管反流等尿路畸形,或继往感染留下肾内陈旧性疤痕,则应给予积极治疗。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "尿路梗阻" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "尿路畸形" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "继往感染" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "肾内陈旧性疤痕" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "积极治疗" } ] }, { "text": "否则,菌尿及并存畸形可促进旧疤痕的发展和新疤痕的形成,导致肾脏功能受损,肾性高血压形成,直至终末期肾衰竭。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "菌尿及并存畸形" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "疤痕" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "疤痕" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "肾脏功能受损" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "肾性高血压" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "终末期肾衰竭" } ] }, { "text": "无症状菌尿的治疗,先采用10~14天常规疗法,菌尿转阴后,给予小剂量长期预防,药物选择、剂量和疗程与再感染病人的预防相同。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "无症状菌尿" }, { "start_idx": 12, "end_idx": 21, "type": "pro", "entity": "10~14天常规疗法" }, { "start_idx": 23, "end_idx": 59, "type": "pro", "entity": "菌尿转阴后,给予小剂量长期预防,药物选择、剂量和疗程与再感染病人的预防相同" } ] }, { "text": "5.慢性肾盂肾炎的治疗慢性肾盂肾炎常有肾皮质疤痕形成,并伴有肾乳头和肾盂肾盏的变形扩张,或持续的肾功能损害和肾脏挛缩。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "慢性肾盂肾炎" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "慢性肾盂肾炎" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "肾皮质疤痕" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "肾乳头" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "肾盂肾盏" }, { "start_idx": 30, "end_idx": 42, "type": "sym", "entity": "肾乳头和肾盂肾盏的变形扩张" }, { "start_idx": 48, "end_idx": 50, "type": "ite", "entity": "肾功能" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "肾功能损害" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "肾脏" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "肾脏挛缩" } ] }, { "text": "慢性肾盂肾炎大多伴有膀胱输尿管反流,少数有尿路梗阻,不伴畸形者极少见。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "慢性肾盂肾炎" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "膀胱输尿管" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "膀胱输尿管反流" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "尿路" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "尿路梗阻" } ] }, { "text": "慢性肾盂肾炎的治疗包括内科保守治疗和外科治疗。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "慢性肾盂肾炎" }, { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "内科保守治疗" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "外科治疗" } ] }, { "text": "对于有尿路畸形者或尿路梗阻者,应尽早手术。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "尿路畸形" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "尿路梗阻" } ] }, { "text": "6.尿路畸形的治疗输尿管肾盂连接处狭窄或肾结石引起的肾盂积水,后尿道瓣膜和膀胱输尿管反流Ⅲ级以上应予手术治疗。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "尿路畸形" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "输尿管" }, { "start_idx": 9, "end_idx": 18, "type": "sym", "entity": "输尿管肾盂连接处狭窄" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "肾结石" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肾盂" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "肾盂积水" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "后尿道" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "后尿道瓣膜" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "膀胱输尿管" }, { "start_idx": 37, "end_idx": 47, "type": "sym", "entity": "膀胱输尿管反流Ⅲ级以上" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "手术治疗" } ] }, { "text": "【预后】对于大多数慢性尿路感染患儿,随着尿路畸形的矫正和积极的抗感染治疗,尿路感染急性发作的次数可明显降低,肾疤痕形成的风险减少。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "慢性尿路感染" }, { "start_idx": 20, "end_idx": 26, "type": "pro", "entity": "尿路畸形的矫正" }, { "start_idx": 28, "end_idx": 35, "type": "pro", "entity": "积极的抗感染治疗" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "尿路感染" }, { "start_idx": 37, "end_idx": 52, "type": "sym", "entity": "尿路感染急性发作的次数可明显降低" }, { "start_idx": 54, "end_idx": 63, "type": "sym", "entity": "肾疤痕形成的风险减少" } ] }, { "text": "仅少数起病年龄早,就诊时已有广泛肾疤痕形成的慢性尿路感染的小儿,会发展成高血压,进行性肾损害,直到慢性肾衰竭。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "广泛肾疤痕形成" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "慢性尿路感染" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "高血压" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "进行性肾损害" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "慢性肾衰竭" } ] }, { "text": "所以,对儿童,尤其婴幼儿的尿路感染要引起足够的重视。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "尿路感染" } ] }, { "text": "三、治疗(一)氧疗对于呼吸功能不全者,吸入低-中浓度氧(FiO20.3~0.5)数小时,可以提高血氧饱和度(SpO2>90%),一般认为有效。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "氧疗" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "呼吸功能不全" }, { "start_idx": 22, "end_idx": 27, "type": "dru", "entity": "低-中浓度氧" }, { "start_idx": 60, "end_idx": 64, "type": "ite", "entity": "血氧饱和度" } ] }, { "text": "呼吸衰竭患者吸入氧12~24小时,可以解除低氧血症,发绀和呼吸困难逐渐消退。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 8, "end_idx": 8, "type": "dru", "entity": "氧" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "低氧血症" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "发绀" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "长时间吸入低浓度氧一般不会产生严重不良反应。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "dru", "entity": "氧" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "严重不良反应" } ] }, { "text": "但吸入氧>0.8,24~48小时可以导致气道炎症和水肿,甚至严重的气道黏膜过氧化损伤。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "pro", "entity": "吸入氧" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "炎症" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "水肿" }, { "start_idx": 33, "end_idx": 41, "type": "dis", "entity": "气道黏膜过氧化损伤" } ] }, { "text": "血氧水平过高,可以导致视网膜病变。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "血氧水平" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "视网膜" } ] }, { "text": "动脉氧水平的提高必须和缺氧症状的改善相联系,因组织摄取氧的能力受到氧解离曲线、血红蛋白水平、心输出量等因素影响。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "动脉氧水平" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "缺氧" }, { "start_idx": 33, "end_idx": 37, "type": "ite", "entity": "氧解离曲线" }, { "start_idx": 46, "end_idx": 49, "type": "ite", "entity": "心输出量" } ] }, { "text": "(二)气道管理保持呼吸道湿化和雾化,防止气道上皮细胞过于干燥而变性坏死。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "气道" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "呼吸道" }, { "start_idx": 20, "end_idx": 25, "type": "bod", "entity": "气道上皮细胞" } ] }, { "text": "清除气道分泌物可以采用拍背、气道雾化等方法,也可以使用沐舒坦等药物化痰。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "拍背" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "气道雾化" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "沐舒坦" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "化痰" } ] }, { "text": "对于先天性或获得性气道发育导致通气障碍者,或二氧化碳潴留者,应给予气道插管、机械通气和必要的手术处理,目的为解除气道阻塞、修复窦道等先天性畸形。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "气道" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "通气障碍" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "二氧化碳潴留" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "气道插管" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "机械通气" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "手术" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "气道" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "气道阻塞" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "窦道" }, { "start_idx": 66, "end_idx": 70, "type": "dis", "entity": "先天性畸形" } ] }, { "text": "气道插管后应每隔1~2小时向气道滴入生理盐水,然后行负压气道吸引。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "气道插管" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "气道" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "生理盐水" }, { "start_idx": 26, "end_idx": 31, "type": "pro", "entity": "负压气道吸引" } ] }, { "text": "(三)机械通气1.一般参数设置原则调节潮气和通气频率,保持通气量相对稳定,控制PaCO2在4.7~6kPa(35~45mmHg)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "机械通气" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "潮气" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "通气频率" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "通气量" } ] }, { "text": "新生儿和小于3个月的婴儿通气频率40~50次/分,幼儿为30~50次/分,儿童为20~40次/分。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "通气频率" } ] }, { "text": "容量控制或压力控制时的通气潮气量在6ml/kg体重。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "ite", "entity": "通气潮气量" } ] }, { "text": "2.机械通气效果判断对于肺泡通气量与血氧合状况是否合适,采用以下公式可以判断潜在通气和换气效率:a/A(PO2)=PaO2/PAO2,其中PAO2=FiO2×(PB-PH2O)-PaCO2/R,PAO2为肺泡气氧分压,PB为海平面大气压(760mmHg),PH2O为肺泡气水蒸发分压(47mmHg),R为呼吸商(0.8)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "机械通气" } ] }, { "text": "如果a/A>0.5,正常或轻度呼吸功能不全;a/A<0.5,呼吸衰竭或严重呼吸功能不全;a/A<0.3,严重呼吸衰竭,可以有呼吸窘迫。", "entities": [ { "start_idx": 10, "end_idx": 20, "type": "dis", "entity": "正常或轻度呼吸功能不全" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "严重呼吸功能不全" }, { "start_idx": 52, "end_idx": 57, "type": "dis", "entity": "严重呼吸衰竭" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "呼吸窘迫" } ] }, { "text": "表8-27肺泡通气和血气参数的关系3.过度通气目前不主张采用过度通气的方法,因可能导致新生儿和婴幼儿脑血流显著下降,诱发缺血缺氧性脑损伤。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "通气" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "通气" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "脑" }, { "start_idx": 50, "end_idx": 56, "type": "sym", "entity": "脑血流显著下降" }, { "start_idx": 60, "end_idx": 67, "type": "dis", "entity": "缺血缺氧性脑损伤" } ] }, { "text": "对于通气效果不佳者,可以容许存在一定程度的高碳酸血症,即PaCO2能够保持在7~9kPa(50~65mmHg),而不必调高通气潮气量和气道峰压。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "通气" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 28, "end_idx": 32, "type": "ite", "entity": "PaCO2" } ] }, { "text": "(四)呼吸兴奋剂对于中枢性急性呼吸衰竭,可以使用尼可刹米(可拉明)、盐酸洛贝林(山梗菜碱)等药物兴奋呼吸中枢,但疗效不持久,使用时必须确定气道通畅,新生儿一般不用。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "呼吸兴奋剂" }, { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "中枢性急性呼吸衰竭" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "尼可刹米" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "可拉明" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "盐酸洛贝林" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "山梗菜碱" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "呼吸中枢" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "气道" } ] }, { "text": "尼可刹米肌内、皮下或静脉注射,小于6个月75mg/次,1~3岁125mg/次,4~7岁175mg/次。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "尼可刹米" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肌内" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "皮下" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "静脉" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "注射" } ] }, { "text": "盐酸洛贝林皮下或肌内1~3mg/次,静脉注射0.3~3mg/次,必要时间隔30分钟可重复使用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "盐酸洛贝林" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "皮下" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肌内" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "静脉注射" } ] }, { "text": "(五)降低颅内压遇有脑水肿时,原则上采用“边脱边补”的方式,控制出入液量,达到轻度脱水程度。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "脑水肿" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "“边脱边补”" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "轻度脱水" } ] }, { "text": "常用药为甘露醇,静脉推注每次0.25~0.5g/kg,间隔4~6小时重复应用。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "甘露醇" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "静脉推注" } ] }, { "text": "一般用药后20分钟颅内压开始下降。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "颅内压开始下降" } ] }, { "text": "或采用甘露醇-复方甘油(0.5~1.0g/kg)交替应用,间隔4~6小时,直至症状缓解可逐渐停药。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dru", "entity": "甘露醇-复方甘油" } ] }, { "text": "利尿剂多采用呋塞米,肌内或静脉注射,每次1~2mg/kg,新生儿应间隔12~24小时脱水、低血压、低血钠、低血钾、低血氯、低血钙等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "利尿剂" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "呋塞米" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肌内" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "静脉" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "注射" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "脱水" }, { "start_idx": 45, "end_idx": 47, "type": "sym", "entity": "低血压" }, { "start_idx": 49, "end_idx": 51, "type": "sym", "entity": "低血钠" }, { "start_idx": 53, "end_idx": 55, "type": "sym", "entity": "低血钾" }, { "start_idx": 57, "end_idx": 59, "type": "sym", "entity": "低血氯" }, { "start_idx": 61, "end_idx": 63, "type": "sym", "entity": "低血钙" } ] }, { "text": "已经存在水、盐电解质紊乱者应注意及时纠正。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "水、盐电解质紊乱" } ] }, { "text": "(六)纠正酸中毒1.呼吸性酸中毒呼吸衰竭时的主要代谢失衡是呼吸性酸中毒。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "纠正酸中毒" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "呼吸性酸中毒" } ] }, { "text": "一般应保持气道通畅,兴奋呼吸,必要时采用机械通气方式,降低组织和循环血中的二氧化碳。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "保持气道通畅" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "机械通气" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "组织" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "循环血" } ] }, { "text": "2.代谢性酸中毒采用碱性药物,如碳酸氢钠,通过中和体内固定酸,提高血浆,纠正酸中毒。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "碱性药物" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "体内固定酸" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "血浆" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "酸中毒" } ] }, { "text": "此外,酸中毒可以刺激气道痉挛和降低支气管扩张剂的作用,碳酸氢钠可以缓解支气管痉挛。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "酸中毒" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "气道" }, { "start_idx": 17, "end_idx": 22, "type": "dru", "entity": "支气管扩张剂" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "支气管" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "支气管痉挛" } ] }, { "text": "低氧和酸中毒可以导致心肌麻痹及肺内小血管痉挛,补充碳酸氢钠可以起强心和舒张肺内血管作用,有利于改善肺内血液灌流。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "酸中毒" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心肌" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "心肌麻痹" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "肺内小血管" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "肺内小血管痉挛" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "心" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "肺内血管" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "肺内血液" } ] }, { "text": "一般应用5%碳酸氢钠,首剂可用1~1.5mmol/kg(1ml=0.6mmol)。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "碳酸氢钠" } ] }, { "text": "静脉滴注或慢推注时,可以将5%碳酸氢钠用乳酸-林格液或葡萄糖生理盐水稀释(1∶2vol/vol),以降低碱性液对静脉血管的刺激。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "慢推注" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 20, "end_idx": 25, "type": "dru", "entity": "乳酸-林格液" }, { "start_idx": 27, "end_idx": 33, "type": "dru", "entity": "葡萄糖生理盐水" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "碱性液" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "静脉血管" } ] }, { "text": "如果补充碱性液过快,或没有及时改善通气和外周循环,可能产生代谢性碱中毒,可以导致昏迷和心跳停止。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "碱性液" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "代谢性碱中毒" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "昏迷" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "心跳停止" } ] }, { "text": "在出现代谢性碱中毒时,可以迅速适当降低通气量产生呼吸性酸中毒、补充生理盐水,或给予口服氯化氨、静脉注射或口服氯化钾纠正。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "代谢性碱中毒" }, { "start_idx": 15, "end_idx": 21, "type": "pro", "entity": "适当降低通气量" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "生理盐水" }, { "start_idx": 41, "end_idx": 45, "type": "dru", "entity": "口服氯化氨" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "静脉注射" }, { "start_idx": 52, "end_idx": 56, "type": "dru", "entity": "口服氯化钾" } ] }, { "text": "(七)强心药和血管活性药的应用在持续低氧血症并发心力衰竭时可以使用洋地黄制剂、利尿剂、血管张力调节制剂等。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "强心药" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "血管活性药" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "低氧血症" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "洋地黄制剂" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "利尿剂" }, { "start_idx": 43, "end_idx": 50, "type": "dru", "entity": "血管张力调节制剂" } ] }, { "text": "1.毛花苷丙和地高辛在呼吸衰竭时心肌缺氧,容易导致洋地黄中毒,应考虑减少其用量。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "毛花苷丙" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "地高辛" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "心肌缺氧" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "洋地黄中毒" } ] }, { "text": "2.多巴胺和多巴酚丁胺兴奋心脏β1受体,扩张肾、脑、肺血管作用,增加肾血流量和尿量,为休克和难治性心力衰竭的主要药物。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "多巴胺" }, { "start_idx": 6, "end_idx": 10, "type": "dru", "entity": "多巴酚丁胺" }, { "start_idx": 13, "end_idx": 29, "type": "bod", "entity": "心脏β1受体" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "肾" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "脑" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肺血管" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "肾" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "休克" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "难治性心力衰竭" } ] }, { "text": "其半衰期非常短,必须连续静脉滴注。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "连续静脉滴注" } ] }, { "text": "多巴胺2~10μg/(kg•min),多巴酚丁胺2~20μg/(kg•min),可以联合应用,从低剂量开始。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "多巴胺" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "多巴酚丁胺" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "联合应用" } ] }, { "text": "3.酚妥拉明为α受体阻滞剂,可以直接扩张外周小动脉和毛细血管,显著降低周围血管阻力及心脏后负荷,提高心输出量。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "酚妥拉明" }, { "start_idx": 7, "end_idx": 12, "type": "dru", "entity": "α受体阻滞剂" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "外周小动脉" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "毛细血管" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "周围血管" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "心脏" }, { "start_idx": 50, "end_idx": 53, "type": "ite", "entity": "心输出量" } ] }, { "text": "适用于低氧引起的肺血管痉挛、重症肺炎、急性肺水肿、充血性心力衰竭等疾病时的呼吸衰竭。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肺血管" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "肺血管痉挛" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "重症肺炎" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "急性肺水肿" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "剂量为静脉滴注0.1~0.3mg/次,用5%葡萄糖盐水稀释,每分钟2~6μg速度滴入。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "葡萄糖盐水" } ] }, { "text": "应用中注意纠正低血压和心律失常,在伴有中毒性休克时应补充血容量。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "低血压" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "心律失常" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "中毒性休克" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "血容量" } ] }, { "text": "4.一氧化氮(NO)吸入新生儿低氧性呼吸衰竭伴持续肺动脉高压,可以吸入NO治疗。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "一氧化氮" }, { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "NO" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "低氧性呼吸衰竭" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肺动脉" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "肺动脉高压" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "吸入NO" } ] }, { "text": "起始剂量为10~20ppm3~6小时,随后改为5~10ppm,可以维持1~7天或更长时间,直到缺氧状况根本缓解(参见本篇第十三章第三节)。", "entities": [ { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "缺氧" } ] }, { "text": "(八)利尿剂在呼吸衰竭伴急性肺水肿、急性心力衰竭时,可以应用呋塞米促进肺液吸收、减轻心脏负荷。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "利尿剂" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "急性肺水肿" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "急性心力衰竭" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "呋塞米" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肺液" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "心脏" } ] }, { "text": "七、肾活检的并发症及处理肾活检属比较安全的手术,严格掌握操作步骤可降低并发症发生率,远较肝穿刺安全。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肾活检" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "肾活检" }, { "start_idx": 44, "end_idx": 46, "type": "pro", "entity": "肝穿刺" } ] }, { "text": "(一)血尿术后镜下血尿的发生率占穿刺病例的80%~90%,为组织损伤后常见的临床表现,并不视为并发症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "血尿" }, { "start_idx": 7, "end_idx": 7, "type": "equ", "entity": "镜" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "血尿" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "穿刺" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "组织" } ] }, { "text": "血尿通常在1~5天内消失,无需特殊处理。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "血尿" } ] }, { "text": "镜下血尿发生率为49.8%~86.7%,一般24~48小时后消失。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "equ", "entity": "镜" }, { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "血尿" } ] }, { "text": "肉眼血尿多在1天内消失,少数病例持续1周。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "肉眼血尿" } ] }, { "text": "术前有肉眼血尿,尤其IgA肾病患者以反复发作型肉眼血尿为临床表现者;持续性高血压;肾功能受损者;取出组织过长者出现肉眼血尿的机会较多。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "血尿" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "持续性高血压" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "肾功能受损者" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "组织" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "血尿" } ] }, { "text": "对于一过性肉眼血尿的患者,除严格卧床休息、多饮水增加尿量外,无特别治疗。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "血尿" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "尿量" } ] }, { "text": "若血尿持续不止,应进一步检查凝血功能,排除隐匿性凝血功能异常,并动态观察血中血红蛋白,对失血量较大或出血时间较长的患者,应及时输新鲜血,同时静脉给予维生素K1促进凝血因子合成,增强止血效果。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "血尿" }, { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "检查凝血功能" }, { "start_idx": 21, "end_idx": 29, "type": "dis", "entity": "隐匿性凝血功能异常" }, { "start_idx": 36, "end_idx": 36, "type": "ite", "entity": "血" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "血" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "血" }, { "start_idx": 64, "end_idx": 66, "type": "dru", "entity": "新鲜血" }, { "start_idx": 74, "end_idx": 78, "type": "dru", "entity": "维生素K1" }, { "start_idx": 87, "end_idx": 90, "type": "bod", "entity": "凝血因子" }, { "start_idx": 97, "end_idx": 97, "type": "bod", "entity": "血" } ] }, { "text": "为防止血块形成阻塞肾小管,予以适量碳酸氢钠碱化尿液。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血块" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肾小管" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "尿液" } ] }, { "text": "经上述治疗血尿无明显缓解者,可以用小剂量垂体后叶素由输液泵静脉持续泵入。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "血尿" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "垂体后叶素" }, { "start_idx": 26, "end_idx": 28, "type": "equ", "entity": "输液泵" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "静脉持续泵入" } ] }, { "text": "首次剂量为6~8U/h,尿色转淡后即减至4U/h,直到血尿完全消失24小时后。", "entities": [ { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "血尿" } ] }, { "text": "若经过以上措施出血仍不缓解,条件允许者还可行选择性肾动脉造影检查,明确出血灶后,采用插管的方法在局部注射硬化剂或自身血块栓塞血管,以达到止血的目的。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "出血" }, { "start_idx": 22, "end_idx": 31, "type": "pro", "entity": "选择性肾动脉造影检查" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "插管" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "硬化剂" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "血块" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "血管" } ] }, { "text": "(二)肾周围血肿文献报道肾周围血肿的发生率为0.5%~1.5%。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "肾周围血肿" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "肾周围血肿" } ] }, { "text": "一般患者仅诉腹痛、腰痛,体格检查时可发现穿刺部位有压痛或较对侧膨隆,若血红蛋白、血细胞比容下降血细胞则应作超声波或CT检查,以明确诊断并观察血肿大小,评估出血量。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "腹痛" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "腰痛" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "体格检查" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "压痛" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "较对侧膨隆" }, { "start_idx": 35, "end_idx": 38, "type": "ite", "entity": "血红蛋白" }, { "start_idx": 40, "end_idx": 44, "type": "ite", "entity": "血细胞比容" }, { "start_idx": 47, "end_idx": 49, "type": "ite", "entity": "血细胞" }, { "start_idx": 53, "end_idx": 60, "type": "pro", "entity": "超声波或CT检查" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "血肿" } ] }, { "text": "发生肾周围血肿时,患者应绝对卧床休息,适当应用抗生素,预防血肿感染。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "肾周围血肿" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "抗生素" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "血肿感染" } ] }, { "text": "一般病例保守治疗后出血可自行停止,少数血红蛋白下降较多者需输血。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "输血" } ] }, { "text": "严重者输血持续增大,则须手术止血。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "输血" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "手术止血" } ] }, { "text": "肾周围血肿分为有临床意义和无临床意义两种,前者是指实验室检查血细胞比容较穿刺前降低4%以上。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "肾周围血肿" }, { "start_idx": 30, "end_idx": 34, "type": "ite", "entity": "血细胞比容" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "穿刺" } ] }, { "text": "血肿多在1个月内自行吸收消失,出血量大导致血压、脉搏发生变化时需输血。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "血压" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "脉搏" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "输血" } ] }, { "text": "经内科治疗仍出血不止者需行手术止血,甚至切除肾脏。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dep", "entity": "内科" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "手术止血" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "切除肾脏" } ] }, { "text": "(三)动-静脉瘘文献报道肾穿刺引起动-静脉瘘发生率仅为15%~18%,其中多数患者无临床症状。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "动-静脉瘘" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "肾穿刺" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "动-静脉瘘" } ] }, { "text": "临床上存在高血压、肾间质纤维化、肾硬化及严重动脉病变的患者肾穿刺后较易出现此种并发症。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "高血压" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "肾间质纤维化" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "肾硬化" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "严重动脉病变" }, { "start_idx": 29, "end_idx": 31, "type": "pro", "entity": "肾穿刺" } ] }, { "text": "一般无临床症状,若体检时闻及肾区血管性杂音,静脉肾盂造影偶见肾盏扭曲,则可疑为动-静脉瘘。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "体检" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "肾区血管性杂音" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "静脉肾盂造影" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "肾盏扭曲" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "动-静脉瘘" } ] }, { "text": "肾动脉造影或彩色多普勒超声检查可以明确诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "肾动脉造影" }, { "start_idx": 6, "end_idx": 14, "type": "pro", "entity": "彩色多普勒超声检查" } ] }, { "text": "95%的动-静脉瘘患者在肾穿刺术后2~20个月内瘘管能自行闭合,很少要外科手术治疗,若出现大出血、高血压和心力衰竭时,应立即手术治疗,有人提出采用介入治疗,动脉插管注射氨基乙酸可以避免手术。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "动-静脉瘘" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "肾穿刺术" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "瘘管" }, { "start_idx": 35, "end_idx": 36, "type": "dep", "entity": "外科" }, { "start_idx": 45, "end_idx": 47, "type": "sym", "entity": "大出血" }, { "start_idx": 49, "end_idx": 51, "type": "sym", "entity": "高血压" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 62, "end_idx": 65, "type": "pro", "entity": "手术治疗" }, { "start_idx": 73, "end_idx": 76, "type": "pro", "entity": "介入治疗" }, { "start_idx": 78, "end_idx": 87, "type": "pro", "entity": "动脉插管注射氨基乙酸" } ] }, { "text": "其他并发症有腰痛、腹胀、腹痛和放射痛、尿潴留等,但短期随访症状均会消失。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "腰痛" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "腹胀" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "腹痛" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "放射痛" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "尿潴留" } ] }, { "text": "第二节生理特点肾脏的生理功能主要为排泄体内代谢产物如尿素和有机酸等;调节水和电解质平衡,维持内环境稳定以及内分泌功能如分泌肾素、前列腺素以及促红细胞生成素等。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肾脏" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "代谢产物" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "尿素" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "有机酸" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "水" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "电解质" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "内分泌" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "肾素" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "前列腺素" }, { "start_idx": 70, "end_idx": 76, "type": "bod", "entity": "促红细胞生成素" } ] }, { "text": "胚胎9~12周时肾脏开始形成尿液,但整个宫内时期胎儿内环境的稳定是靠胎盘维持,肾脏尚未发挥功能,胎尿为羊水的主要成分。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胚胎" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肾脏" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "尿液" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "宫" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "胎盘" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肾脏" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "胎尿" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "羊水" } ] }, { "text": "肾脏的发育约在胎龄36周时完成,此时肾单位数量已达成人量。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肾脏" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肾单位" } ] }, { "text": "足月新生儿出生后肾单位数量不再增加,但早产儿在出生后肾组织可继续发育,直至相当于胎龄36周为止,因此早产儿出生时肾功能较差。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肾单位" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "肾组织" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "肾" } ] }, { "text": "肾功能除与肾脏本身发育成熟有关外,尚受神经-内分泌以及体液分布等影响。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肾" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肾脏" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "神经-内分泌" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "体液" } ] }, { "text": "足月儿出生时已能完成肾脏的生理功能,但贮备能力差,调节功能不够成熟,当喂养不当、疾病或应激状态时易出现功能紊乱。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肾脏" }, { "start_idx": 34, "end_idx": 54, "type": "sym", "entity": "当喂养不当、疾病或应激状态时易出现功能紊乱" } ] }, { "text": "到1~2岁时各项肾功能按体表面积计算已接近成人水平。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肾" } ] }, { "text": "(一)肾小球的滤过作用新生儿出生时肾小球数目虽与成人相等,但肾小球滤过率甚低,平均约每分钟20ml/1.73m2,早产儿则更低。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肾小球" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肾小球" }, { "start_idx": 30, "end_idx": 35, "type": "ite", "entity": "肾小球滤过率" } ] }, { "text": "因此过量的水分和溶质不能迅速有效地排出,原因是:①新生儿肾滤过作用主要由近髓的成熟肾小球负担;皮质表面的肾小球发育差,血流供应量少,基本不参加滤过作用。", "entities": [ { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肾" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "髓" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "成熟肾小球" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "皮质表面" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "肾小球" }, { "start_idx": 59, "end_idx": 59, "type": "bod", "entity": "血" } ] }, { "text": "②入球小动脉阻力较高,影响肾小球的血流量。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "入球小动脉" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肾小球" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "血流量" } ] }, { "text": "③新生儿血压低,故肾小球滤过压也较低。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "血压" }, { "start_idx": 9, "end_idx": 14, "type": "ite", "entity": "肾小球滤过压" } ] }, { "text": "出生后一周开始,肾血管阻力下降,皮质浅表肾单位开放,肾血流量增加,肾小球滤过压升高,因此肾小球滤过率迅速增加,至1岁时已接近成人水平。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肾血管" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "皮质" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肾单位" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "肾" }, { "start_idx": 33, "end_idx": 38, "type": "ite", "entity": "肾小球滤过压" }, { "start_idx": 44, "end_idx": 49, "type": "ite", "entity": "肾小球滤过率" } ] }, { "text": "(二)肾小管的重吸收及排泄功能肾小管对肾小球滤液中的水及各种溶质选择性地重吸收,以保持机体内环境的稳定。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肾小管" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肾小管" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "肾小球滤液" } ] }, { "text": "肾小管的重吸收与肾小球滤过率保持密切的联系,这一现象称为球-管平衡。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肾小管" }, { "start_idx": 8, "end_idx": 13, "type": "ite", "entity": "肾小球滤过率" } ] }, { "text": "许多实验资料证明,尽管在形态上肾小管的发育落后于肾小球,但肾小管功能在出生后已能保持球-管平衡。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肾小管" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肾小球" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "肾小管" } ] }, { "text": "足月新生儿氨基酸及葡萄糖的重吸收能力正常,出生后已能维持钠平衡,但调节幅度有限,钠的重吸收主要在远端肾小管(成人主要在近端肾小管),此可能与新生儿血循环中醛固酮含量较高有关。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "氨基酸" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "钠" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "钠" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "近端肾小管" }, { "start_idx": 73, "end_idx": 73, "type": "bod", "entity": "血" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "醛固酮" } ] }, { "text": "同时由于肾小球滤过率较低,因此在钠负荷量过大时不能迅速排钠,而易致水肿。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "肾小球" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "钠" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "钠" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "水肿" } ] }, { "text": "早产儿肾功能尚不成熟,葡萄糖肾阈较低,易出现糖尿。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肾" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "肾" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "糖尿" } ] }, { "text": "低出生体重儿排钠较多,如摄入量过低,可出现钠的负平衡而致低钠血症。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "钠" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "钠" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "低钠血症" } ] }, { "text": "新生儿最初10~20天内对钾的排泄能力较差,故有高钾血症的倾向。", "entities": [ { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "钾" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "高钾血症" } ] }, { "text": "(三)尿的浓缩和稀释新生儿及幼婴对尿的稀释能力接近成人,新生儿已能将原尿稀释至40mmol/L[40mOsm/(kg•H2O)]。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "尿" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "尿" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "原尿" } ] }, { "text": "但由于肾小球滤过率低,利尿速度慢,大量水负荷时易出现水肿。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "ite", "entity": "肾小球滤过率" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "水肿" } ] }, { "text": "初生婴儿对尿的浓缩能力不及年长儿及成人,这主要由于婴儿蛋白合成代谢旺盛,而肾小球滤过率低,尿素排出较少,滤液中尿素量不足以在髓质中形成较高的渗透压梯度,因而影响尿的浓缩。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "尿" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "蛋白" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肾小球" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "尿素" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "滤液" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "尿素" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "髓质" }, { "start_idx": 70, "end_idx": 72, "type": "ite", "entity": "渗透压" }, { "start_idx": 80, "end_idx": 80, "type": "bod", "entity": "尿" } ] }, { "text": "婴儿每由尿内排出1mmol的溶质需水1.4~2.4ml,而成人仅需0.7ml。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "尿" } ] }, { "text": "婴儿尿最高渗透压仅达700mmol/L[700mOsm/(kg•H2O)],而成人可达1400mmol/L[1400mOsm/(kg•H2O)],在正常情况下,婴儿这种浓缩能力的缺陷并无重要影响,但在负荷过重的情况下则易发生脱水,甚至诱发急性肾功能不全。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "ite", "entity": "尿最高渗透压" }, { "start_idx": 124, "end_idx": 125, "type": "sym", "entity": "脱水" }, { "start_idx": 131, "end_idx": 137, "type": "dis", "entity": "急性肾功能不全" } ] }, { "text": "(四)酸碱平衡肾脏通过H+的排泌及的重吸收以维持酸碱平衡。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肾脏" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "H+" } ] }, { "text": "新生儿由于肾小管液中磷酸盐及NH3的浓度较低,实际能排出的H+较少,约1~2个月时尿中的可滴定酸可达成人水平,排氨能力至2岁方接近成人。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "肾小管液" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "磷酸盐" }, { "start_idx": 35, "end_idx": 36, "type": "ite", "entity": "H+" }, { "start_idx": 53, "end_idx": 53, "type": "ite", "entity": "尿" } ] }, { "text": "新生儿碳酸氢盐的肾阈较低(约比成人低4~6mmol/L),超过肾阈时即由尿排出。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "碳酸氢盐" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肾" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "肾" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "尿" } ] }, { "text": "婴儿在正常情况下酸碱平衡的调节能力已达最高限度,不足以应付病理状况下的额外负担,较易出现酸中毒。", "entities": [ { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "酸中毒" } ] }, { "text": "(五)肾脏的内分泌功能肾脏不仅是一个排泄器官,而且是一个重要的内分泌器官。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肾脏" } ] }, { "text": "它可以通过自分泌、旁分泌及胞分泌的形式产生一系列内分泌性生物活性物质,在调节身体血压、水电解质平衡、钙、磷等许多方面起重要作用。", "entities": [ { "start_idx": 24, "end_idx": 33, "type": "bod", "entity": "内分泌性生物活性物质" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "血压" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "水电解质" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "钙" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "磷" } ] }, { "text": "肾脏能分泌多种生物活性物质,如前列腺素、肾素、激肽释放酶、促红细胞生成素、1,25-二羟骨化醇以及内皮素与一氧化氮(NO)等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肾脏" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "前列腺素" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肾素" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "激肽释放酶" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "促红细胞生成素" }, { "start_idx": 39, "end_idx": 46, "type": "bod", "entity": "25-二羟骨化醇" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "内皮素" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "一氧化氮" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "NO" } ] }, { "text": "新生儿肾脏已具内分泌功能,释出肾素较多,新生儿血浆肾素、血管紧张素以及醛固酮均高于成人,生后2周内渐降低。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肾素" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "血浆肾素" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "血管紧张素" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "醛固酮" } ] }, { "text": "胚胎肾已能合成前列腺素,脐带血中前列腺素E2含量甚高。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肾" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "前列腺素" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "脐带血" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "前列腺素E2" } ] }, { "text": "前列腺素E2有扩张血管作用,参与肾血流量的调节。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "前列腺素E2" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血管" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肾" } ] }, { "text": "胎儿血氧分压较低,胚胎肾合成促红细胞生成素较多,生后随着血氧分压增高,促红细胞生成素合成减少。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "血氧分压" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "胚胎肾" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "促红细胞生成素" }, { "start_idx": 28, "end_idx": 31, "type": "ite", "entity": "血氧分压" }, { "start_idx": 35, "end_idx": 41, "type": "bod", "entity": "促红细胞生成素" } ] }, { "text": "二、血小板无力症瑞士医师Glanzmann于1918年首先报道本病,故又称Glanzmannthrombasthenia(GT)。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "血小板无力症" }, { "start_idx": 37, "end_idx": 59, "type": "dis", "entity": "Glanzmannthrombasthenia" }, { "start_idx": 61, "end_idx": 62, "type": "dis", "entity": "GT" } ] }, { "text": "其特点是血小板的形态、数量正常,出血时间延长,血块收缩不良或不收缩,聚集功能缺陷。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "血小板" }, { "start_idx": 4, "end_idx": 39, "type": "sym", "entity": "血小板的形态、数量正常,出血时间延长,血块收缩不良或不收缩,聚集功能缺陷" } ] }, { "text": "【发病机制】GT的生化缺陷在于血小板膜糖蛋白GPⅡb/Ⅲa复合物量的减少、缺失或质的异常。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "GT" }, { "start_idx": 15, "end_idx": 31, "type": "bod", "entity": "血小板膜糖蛋白GPⅡb/Ⅲa复合物" } ] }, { "text": "GPⅡb/Ⅲa在其周围微环境发生改变或因血小板激活使其构型改变时,可作为受体与纤维蛋白原、vWF、纤维连接蛋白、层素等黏附分子结合,介导血小板聚集。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "GPⅡb/Ⅲa" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "血小板" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "纤维蛋白原" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "vWF" }, { "start_idx": 49, "end_idx": 54, "type": "bod", "entity": "纤维连接蛋白" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "血小板" } ] }, { "text": "目前研究发现GPⅡb和GPⅢa由不同基因编码,都位于17号染色体上(17q21~23)GT是由于该基因发生了缺失、点突变或插入所致。", "entities": [ { "start_idx": 26, "end_idx": 31, "type": "bod", "entity": "17号染色体" } ] }, { "text": "【实验室检查】血小板数量及形态正常,但在血片上散在不聚集,出血时间延长,血块收缩不良或不收缩。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "血小板" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "血块" } ] }, { "text": "如能测定可进一步发现血小板GPⅡb/Ⅲa复合物量的减少或质的异常。", "entities": [ { "start_idx": 10, "end_idx": 22, "type": "bod", "entity": "血小板GPⅡb/Ⅲa复合物" } ] }, { "text": "(二)实验室检查1.血小板计数正常,血涂片上血小板散在分布,不聚集成堆。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "血小板" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "血小板" } ] }, { "text": "加瑞斯托霉素聚集正常或降低。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "加瑞斯托霉素" } ] }, { "text": "5.血小板玻珠滞留试验减低。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "血小板玻珠滞留试验" } ] }, { "text": "6.血小板膜糖蛋白(GP)Ⅱb/Ⅲa(CD41/CD61)减少或有质的异常。", "entities": [ { "start_idx": 2, "end_idx": 28, "type": "bod", "entity": "血小板膜糖蛋白(GP)Ⅱb/Ⅲa(CD41/CD61)" } ] }, { "text": "五、婴儿血管外皮细胞瘤1942年Stont和Murray首先描述血管外皮细胞瘤,在小儿有特点,常称“类血管外皮细胞瘤”,多见于1岁左右的婴儿,仅50%左右发展为恶性。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "婴儿血管外皮细胞瘤" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "血管外皮细胞瘤" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "类血管外皮细胞瘤" } ] }, { "text": "肿瘤为多房性,大小不一,界限清楚,显微镜下肿瘤内毛细血管紧密聚集,肿瘤由血管扁平细胞组成,血管间有大量外皮细胞增生·。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 17, "end_idx": 19, "type": "equ", "entity": "显微镜" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "肿瘤" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "毛细血管" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "毛细血管紧密聚集" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "肿瘤" }, { "start_idx": 36, "end_idx": 41, "type": "bod", "entity": "血管扁平细胞" }, { "start_idx": 33, "end_idx": 43, "type": "sym", "entity": "肿瘤由血管扁平细胞组成" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "血管" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "外皮细胞" }, { "start_idx": 45, "end_idx": 56, "type": "sym", "entity": "血管间有大量外皮细胞增生" } ] }, { "text": "肿瘤细胞向血管内生长,可见核分裂和坏死情况,在小儿中这并不提示恶性。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "血管" } ] }, { "text": "少数情况下,肿瘤可向周围组织浸润,甚至远处转移,则肯定为恶性。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肿瘤" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "组织" } ] }, { "text": "婴儿血管外皮细胞瘤(infantilehemangioendothelioma)多为良性,局部切除即可治愈,无需放疗、化疗。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "婴儿血管外皮细胞瘤" }, { "start_idx": 10, "end_idx": 38, "type": "dis", "entity": "infantilehemangioendothelioma" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "局部切除" }, { "start_idx": 56, "end_idx": 57, "type": "pro", "entity": "放疗" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "化疗" } ] }, { "text": "四、肾活检适应证凡肾脏有弥漫性病变,其病因、治疗和预后等问题尚未解决或不甚明确,又无禁忌证者,均为肾活检的适应证。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肾活检" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肾脏" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "弥漫性病变" }, { "start_idx": 49, "end_idx": 51, "type": "pro", "entity": "肾活检" } ] }, { "text": "它能对各种原发及继发性肾脏疾病的诊断提供直接依据,还可以了解病变的程度以及病情是否可逆等,从而直接指导临床治疗。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "dis", "entity": "原发及继发性肾脏疾病" } ] }, { "text": "1.肾病综合征儿童肾病综合征(NS)可由多种原因引起,原发性NS又有多种病理类型,治疗及预后各有不同。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "儿童肾病综合征" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "NS" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "原发性NS" } ] }, { "text": "对继发性NS以及对糖皮质激素治疗耐药和临床表现提示非单纯性NS的情况应行肾活检,以明确病理类型,指导治疗,判断预后。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "继发性NS" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "非单纯性NS" } ] }, { "text": "由于儿童微小病变较多,对糖皮质激素治疗敏感,对这类患儿是否及早肾活检尚有不同意见。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "肾活检" } ] }, { "text": "2.急性肾炎综合征多种肾小球疾病均可表现为急性肾炎综合征,仅根据临床表现及实验检查相似,难以鉴别诊断,但病情发展、治疗和预后则完全不同。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "急性肾炎综合征" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肾小球疾病" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "急性肾炎综合征" } ] }, { "text": "如IgA肾病、膜性肾病、新月体肾炎、急进性肾炎甚至系统性血管炎,因此对这类临床综合征应尽早行肾活检。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "膜性肾病" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "新月体肾炎" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "急进性肾炎" }, { "start_idx": 46, "end_idx": 48, "type": "pro", "entity": "肾活检" } ] }, { "text": "3.无症状性蛋白尿肾功能正常,蛋白尿持续6个月以上,伴有血尿和(或)24小时尿蛋白达1g/m2,是肾活检的指征。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肾" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "血尿" }, { "start_idx": 38, "end_idx": 40, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 55, "end_idx": 57, "type": "pro", "entity": "肾活检" } ] }, { "text": "已有肾功能轻度不全的患者,就诊时若不能肯定诊断,特别是伴有镜下血尿和(或)高血压者都是肾活检的指征。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肾" }, { "start_idx": 29, "end_idx": 29, "type": "equ", "entity": "镜" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "血尿" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "高血压" }, { "start_idx": 43, "end_idx": 45, "type": "pro", "entity": "肾活检" } ] }, { "text": "4.原因不明的血尿反复发作性肉眼血尿或持续性镜下血尿持续6个月以上,已排除肾外因素,并经尿路造影及膀胱镜检查未能确诊者,可考虑肾活检;血尿伴有蛋白尿及管型者,更应考虑肾活检。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "血尿" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "血尿" }, { "start_idx": 22, "end_idx": 22, "type": "equ", "entity": "镜" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "血尿" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "肾" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "尿路造影" }, { "start_idx": 49, "end_idx": 53, "type": "pro", "entity": "膀胱镜检查" }, { "start_idx": 63, "end_idx": 65, "type": "pro", "entity": "肾活检" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "血尿" }, { "start_idx": 71, "end_idx": 73, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 75, "end_idx": 76, "type": "sym", "entity": "管型" }, { "start_idx": 83, "end_idx": 85, "type": "pro", "entity": "肾活检" } ] }, { "text": "5.急性肾衰竭以前认为尿毒症时肾穿刺危险性大,但是近年来大多数作者倾向于对病因不明的急性肾衰竭患儿均应进行肾活检,这对确定原发病、选择治疗方案及判断预后有重要意义。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "尿毒症" }, { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "肾穿刺" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 53, "end_idx": 55, "type": "pro", "entity": "肾活检" } ] }, { "text": "特别近年来药物引起的急性间质性肾炎日益增多,但临床有时很难与其他各种疾病引起的急性肾衰竭或肾小管间质性疾病相区别,肾活检可以做出正确诊断并选择正确的治疗措施。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "急性间质性肾炎" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 45, "end_idx": 52, "type": "dis", "entity": "肾小管间质性疾病" }, { "start_idx": 57, "end_idx": 59, "type": "pro", "entity": "肾活检" } ] }, { "text": "6.结缔组织病肾脏为系统性红斑狼疮等多种结缔组织病最常受侵犯的脏器,有时为首发症状,肾脏病理改变可作为观察全身病变性质及程度的一个窗口。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肾脏" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "肾脏" } ] }, { "text": "肾活检不仅能够了解病因,明确病理类型,而且还有选择治疗及判断预后的价值。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "肾活检" } ] }, { "text": "因此结缔组织病应尽早行肾活检。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "肾活检" } ] }, { "text": "7.肾移植肾移植后发生肾功能减退的原因很多,临床鉴别十分困难。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肾移植" }, { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "肾移植" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "肾功能减退" } ] }, { "text": "因此,当存在以下疑问时,应尽早行肾活检明确引起肾功能减退的原因:①肾移植后出现急性肾衰竭者;②移植后患者发热、尿量减少、血尿素氮及肌酐轻度升高、不能区分感染或排异反应者;③发生严重的排异反应,判断继续保守疗法或手术切除有困难者;④术后出现持续性蛋白尿,要明确是原有肾脏疾病复发还是其他因素引起者。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "肾活检" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "肾功能减退" }, { "start_idx": 33, "end_idx": 35, "type": "pro", "entity": "肾移植" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 47, "end_idx": 48, "type": "pro", "entity": "移植" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "发热" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "尿量减少" }, { "start_idx": 60, "end_idx": 70, "type": "sym", "entity": "血尿素氮及肌酐轻度升高" }, { "start_idx": 72, "end_idx": 82, "type": "sym", "entity": "不能区分感染或排异反应" }, { "start_idx": 100, "end_idx": 103, "type": "pro", "entity": "保守疗法" }, { "start_idx": 105, "end_idx": 108, "type": "pro", "entity": "手术切除" }, { "start_idx": 115, "end_idx": 124, "type": "sym", "entity": "术后出现持续性蛋白尿" }, { "start_idx": 132, "end_idx": 135, "type": "dis", "entity": "肾脏疾病" } ] }, { "text": "8.慢性小管间质性疾病慢性小管-间质性肾炎既可以是先天性、遗传性的,也可以是特发性或继发性的,其临床表现多种多样,包括蛋白尿、血尿、无症状性脓尿或原因不明的肾功能减退及由此引起的与慢性肾炎相似的各种临床表现,肾活检有助于明确诊断,探讨病因。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "慢性小管间质性疾病" }, { "start_idx": 11, "end_idx": 20, "type": "dis", "entity": "慢性小管-间质性肾炎" }, { "start_idx": 59, "end_idx": 61, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "血尿" }, { "start_idx": 66, "end_idx": 71, "type": "sym", "entity": "无症状性脓尿" }, { "start_idx": 78, "end_idx": 78, "type": "bod", "entity": "肾" }, { "start_idx": 90, "end_idx": 93, "type": "dis", "entity": "慢性肾炎" }, { "start_idx": 104, "end_idx": 106, "type": "pro", "entity": "肾活检" } ] }, { "text": "第十八章重症肌无力重症肌无力(myastheniagravis,MG)包括三种综合征即新生儿MG、先天性MG及儿童MG,其中新生儿及儿童MG是一种发生在神经-肌肉接头处,乙酰胆碱受体(acetycholinereceptor,AChR)抗体介导、细胞免疫依赖的获得性自身免疫性疾病。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "重症肌无力" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "重症肌无力" }, { "start_idx": 15, "end_idx": 30, "type": "dis", "entity": "myastheniagravis" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "MG" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "新生儿MG" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "先天性MG" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "儿童MG" }, { "start_idx": 66, "end_idx": 69, "type": "dis", "entity": "儿童MG" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "神经" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "肌肉" }, { "start_idx": 85, "end_idx": 90, "type": "bod", "entity": "乙酰胆碱受体" }, { "start_idx": 92, "end_idx": 111, "type": "bod", "entity": "acetycholinereceptor" }, { "start_idx": 113, "end_idx": 116, "type": "bod", "entity": "AChR" }, { "start_idx": 130, "end_idx": 139, "type": "dis", "entity": "获得性自身免疫性疾病" } ] }, { "text": "肌无力通常表现为晨轻晚重,波动性明显。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肌无力" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "晨轻晚重" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "波动性明显" } ] }, { "text": "2/3病例累及眼外肌,常为早期症状,10%长期局限于眼肌,颜面肌、咽喉肌、躯干肌和肢体肌均可受累。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "眼外肌" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "眼肌" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "颜面肌" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "咽喉肌" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "躯干肌" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "肢体肌" } ] }, { "text": "【流行病学】国外流行病学调查显示MG年发病率为7.4/10万。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "MG" } ] }, { "text": "但近些年我国文献报道,患者发病年龄同期以儿童期多见,占MG56.4%,且发病年龄提前,多在1~5岁发病。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "MG" } ] }, { "text": "我国尚无流行病学研究报道,但从国内多个成组病例资料以及我院的资料显示,儿童MG小年龄患病比例较高。", "entities": [ { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "儿童MG" } ] }, { "text": "女性患者所生新生儿,其中约10%经过胎盘转运获得烟碱型乙酰胆碱受体抗体(nAChRab),可暂时出现肌无力症状。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "胎盘" }, { "start_idx": 24, "end_idx": 34, "type": "bod", "entity": "烟碱型乙酰胆碱受体抗体" }, { "start_idx": 36, "end_idx": 42, "type": "bod", "entity": "nAChRab" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "肌无力" } ] }, { "text": "【病因与发病机制】20世纪70年代由于烟碱型乙酰胆碱受体(nAChR)能够从电鱼放电器官中得到并纯化,可成功地产生实验性MG的模型,以及同位素标记的蛇毒α-神经毒素放射免疫分析的应用,MG的发病机制研究已经取得突破性的进展:MG其发病机制与遗传因素、致病性自身抗体、细胞因子、补体参与及胸腺肌细胞等复杂因素有关。", "entities": [ { "start_idx": 19, "end_idx": 27, "type": "bod", "entity": "烟碱型乙酰胆碱受体" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "nAChR" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "实验性MG" }, { "start_idx": 68, "end_idx": 72, "type": "pro", "entity": "同位素标记" }, { "start_idx": 92, "end_idx": 93, "type": "dis", "entity": "MG" }, { "start_idx": 112, "end_idx": 113, "type": "dis", "entity": "MG" }, { "start_idx": 125, "end_idx": 131, "type": "bod", "entity": "致病性自身抗体" }, { "start_idx": 133, "end_idx": 136, "type": "bod", "entity": "细胞因子" }, { "start_idx": 138, "end_idx": 139, "type": "bod", "entity": "补体" }, { "start_idx": 143, "end_idx": 147, "type": "bod", "entity": "胸腺肌细胞" } ] }, { "text": "(一)重症肌无力是横纹肌突触后膜nAChR自身免疫性疾病神经肌肉接头是通过接受乙酰胆碱(ACh)及烟碱等兴奋性递质传递与肌膜受体结合,导致离子通道开放,Na+内流,肌膜去极化,产生终板电位,肌丝滑行,因而引起肌肉收缩。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "重症肌无力" }, { "start_idx": 9, "end_idx": 27, "type": "dis", "entity": "横纹肌突触后膜nAChR自身免疫性疾病" }, { "start_idx": 28, "end_idx": 33, "type": "bod", "entity": "神经肌肉接头" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "乙酰胆碱" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "ACh" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "烟碱" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "兴奋性递质" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "肌膜受体" }, { "start_idx": 69, "end_idx": 72, "type": "bod", "entity": "离子通道" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "Na+" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "肌膜" }, { "start_idx": 101, "end_idx": 102, "type": "bod", "entity": "肌丝" }, { "start_idx": 110, "end_idx": 113, "type": "sym", "entity": "肌肉收缩" } ] }, { "text": "已知nChR是造成MG自体免疫应答高度特异性的抗原。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "nChR" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "MG" } ] }, { "text": "nAChR位于神经肌肉接头部的突触后膜中。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "nAChR" }, { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "神经肌肉接头部" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "突触后膜" } ] }, { "text": "实验证明MG患者胸腺上皮细胞内含肌原纤维骨骼肌存在共同抗原(nAChR)。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "MG" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胸腺" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 8, "end_idx": 19, "type": "sym", "entity": "胸腺上皮细胞内含肌原纤维" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 30, "end_idx": 34, "type": "mic", "entity": "nAChR" } ] }, { "text": "该抗原致敏T细胞,产生抗nAChR的抗体(nAChRab)。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "T细胞" }, { "start_idx": 11, "end_idx": 19, "type": "bod", "entity": "抗nAChR的抗体" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "nAChRab" } ] }, { "text": "该抗体对骨骼肌nAChR产生交差免疫应答,使受体被阻滞;并加速AChR的降解,通过激活补体,使肌膜受到损害。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "bod", "entity": "骨骼肌nAChR" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "受体" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "AChR" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "补体" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "肌膜" } ] }, { "text": "电镜检查显示突触后膜IgG和C3沉积辣根酶标记蛇毒神经毒素电镜检测运动终板超微结构显示:MG病理损害的特征是骨骼肌突触后膜皱襞表面面积减少nAChR活性降低肌无力症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "电镜检查" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "IgG" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "C3" }, { "start_idx": 6, "end_idx": 17, "type": "sym", "entity": "突触后膜IgG和C3沉积" }, { "start_idx": 18, "end_idx": 32, "type": "pro", "entity": "辣根酶标记蛇毒神经毒素电镜检测" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "MG" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "突触后膜" }, { "start_idx": 54, "end_idx": 68, "type": "sym", "entity": "骨骼肌突触后膜皱襞表面面积减少" }, { "start_idx": 69, "end_idx": 73, "type": "mic", "entity": "nAChR" }, { "start_idx": 69, "end_idx": 77, "type": "sym", "entity": "nAChR活性降低" }, { "start_idx": 78, "end_idx": 80, "type": "dis", "entity": "肌无力" } ] }, { "text": "(二)重症肌无力是T细胞依赖的自身免疫疾病体液免疫大量研究资料阐明nAChR作为MG的靶子遭到损害,是由nAChRab介导的;而nAChRab对nAChR免疫应答是T细胞依赖性的。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "重症肌无力" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "T细胞" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "体液" }, { "start_idx": 33, "end_idx": 37, "type": "mic", "entity": "nAChR" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "MG" }, { "start_idx": 52, "end_idx": 58, "type": "bod", "entity": "nAChRab" }, { "start_idx": 64, "end_idx": 70, "type": "bod", "entity": "nAChRab" }, { "start_idx": 72, "end_idx": 76, "type": "mic", "entity": "nAChR" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "T细胞" } ] }, { "text": "T细胞在MG自身免疫应答中起着关键作用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "T细胞" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "MG" } ] }, { "text": "nAChRab的产生必须有nAChR特异性T细胞的参与。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "nAChRab" }, { "start_idx": 13, "end_idx": 17, "type": "mic", "entity": "nAChR" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "T细胞" } ] }, { "text": "nAChR特异CD4+T细胞先通过其受体(TCR)对nAChR特异性位点的识别,然后由T辅助细胞(Th)将nAChR主要免疫原区特异性抗体提供给B细胞,促使B细胞分泌高致病性的nAChRab。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "nAChR" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "T细胞" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "受体" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "TCR" }, { "start_idx": 32, "end_idx": 36, "type": "mic", "entity": "nAChR" }, { "start_idx": 49, "end_idx": 53, "type": "bod", "entity": "T辅助细胞" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "Th" }, { "start_idx": 59, "end_idx": 63, "type": "mic", "entity": "nAChR" }, { "start_idx": 66, "end_idx": 74, "type": "bod", "entity": "免疫原区特异性抗体" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "B细胞" }, { "start_idx": 84, "end_idx": 86, "type": "bod", "entity": "B细胞" }, { "start_idx": 94, "end_idx": 100, "type": "bod", "entity": "nAChRab" } ] }, { "text": "Th细胞通过分泌细胞因子来实现对nAChRab分泌的调节。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "Th细胞" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "细胞因子" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "nAChRab" } ] }, { "text": "(三)遗传基因和病毒感染众所周知,重症肌无力是自身免疫应答异常,但启动自身免疫的病因尚未完全弄清。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "病毒感染" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "重症肌无力" } ] }, { "text": "目前认为MG发病与人类白血病抗原(HLA)有关,其相关性与人种及地域有关,且存在性别差异。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "MG" }, { "start_idx": 9, "end_idx": 15, "type": "mic", "entity": "人类白血病抗原" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "HLA" } ] }, { "text": "HLA-Ⅱ类抗原(包括D区的DP、DQ及DR等基因产物)在发生自体免疫过程中起重要作用。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "mic", "entity": "HLA-Ⅱ类抗原" } ] }, { "text": "采用PCR-RFLP技术检测发现我国非胸腺瘤MG与HLADQA1*0301基因显著相关。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "pro", "entity": "PCR-RFLP技术检测" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "非胸腺瘤MG" }, { "start_idx": 25, "end_idx": 31, "type": "mic", "entity": "HLADQA1" } ] }, { "text": "此外还发现与DQB1*0303及DPD1*1910基因相关显著,说明MG发病与多基因遗传有关。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "mic", "entity": "DQB1" }, { "start_idx": 22, "end_idx": 25, "type": "mic", "entity": "DPD1" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "MG" } ] }, { "text": "MG的发病除了与遗传基因有关外,还包括外在环境影响,如本病常因病毒感染而诱发或使病情加重。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "MG" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "病毒感染" } ] }, { "text": "胸腺为免疫中枢。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胸腺" } ] }, { "text": "不论是胸腺淋巴细胞(特别是T细胞),还是上皮细胞(特别是肌样细胞,含有nAChR特异性抗原),遭到免疫攻击,打破免疫耐受性,引起针对nAChR的自身免疫应答,因此使MG发病。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "胸腺淋巴细胞" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "T细胞" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "肌样细胞" }, { "start_idx": 35, "end_idx": 44, "type": "bod", "entity": "nAChR特异性抗原" }, { "start_idx": 66, "end_idx": 70, "type": "mic", "entity": "nAChR" }, { "start_idx": 82, "end_idx": 83, "type": "dis", "entity": "MG" } ] }, { "text": "(一)新生儿一过性重症肌无力(transientneonatalmyastheniagravis)仅见于母亲患MG所生的新生儿。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "新生儿一过性重症肌无力" }, { "start_idx": 15, "end_idx": 47, "type": "dis", "entity": "transientneonatalmyastheniagravis" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "MG" } ] }, { "text": "患儿出生后数小时~3天内出现肌无力,表现哭声低弱,吞咽及呼吸困难,患儿血中nAChR-Ab可增高呼吸衰竭。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肌无力" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "表现哭声低弱" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "吞咽及呼吸困难" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "血" }, { "start_idx": 37, "end_idx": 44, "type": "bod", "entity": "nAChR-Ab" }, { "start_idx": 33, "end_idx": 47, "type": "sym", "entity": "患儿血中nAChR-Ab可增高" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "(二)先天性肌无力综合征(congenitalmyastheniasyndrome)出生后以对称、持续存在、不完全眼外肌无力为特点,血清中无nAChR-Ab常染色体遗传有关,同胞中可有此病,但其母亲未患MG。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "先天性肌无力综合征" }, { "start_idx": 13, "end_idx": 40, "type": "dis", "entity": "congenitalmyastheniasyndrome" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "不完全眼外肌无力" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "血清" }, { "start_idx": 70, "end_idx": 77, "type": "bod", "entity": "nAChR-Ab" }, { "start_idx": 66, "end_idx": 77, "type": "sym", "entity": "血清中无nAChR-Ab" }, { "start_idx": 78, "end_idx": 81, "type": "bod", "entity": "常染色体" }, { "start_idx": 101, "end_idx": 102, "type": "dis", "entity": "MG" } ] }, { "text": "(三)少年时重症肌无力(juvenileneonatalmyastheniagravis)为后天获得性肌无力,可以查到血清中nAChR-Ab。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "少年时重症肌无力" }, { "start_idx": 12, "end_idx": 43, "type": "dis", "entity": "juvenileneonatalmyastheniagravis" }, { "start_idx": 46, "end_idx": 53, "type": "dis", "entity": "后天获得性肌无力" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "血清" }, { "start_idx": 62, "end_idx": 66, "type": "mic", "entity": "nAChR" } ] }, { "text": "国外病例大多在10岁以后发病,以全身型为主,而国内资料与香港及日本报道发病多在幼儿时期(2~3岁),眼肌型为主。", "entities": [ { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "眼肌型" } ] }, { "text": "此为儿童MG最常见的类型,现重点叙述如下。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "儿童MG" } ] }, { "text": "肌无力通常晨轻晚重,亦可多变,后期可处于不全瘫痪状态。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肌无力" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "晨轻晚重" }, { "start_idx": 15, "end_idx": 25, "type": "sym", "entity": "后期可处于不全瘫痪状态" } ] }, { "text": "眼外肌最常受累亦可局限于眼肌睁眼无力上眼睑下垂眼球运动受限出现斜视和复视,甚或眼球固定不动眼内肌一般不受影响瞳孔反射多正常眼肌型重症无力。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "眼外肌" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "眼外肌最常受累" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "眼肌" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "亦可局限于眼肌" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "眼" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "睁眼无力" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "上眼睑" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "上眼睑下垂" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "眼球" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "眼球运动受限" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "出现斜视和复视" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "眼球" }, { "start_idx": 37, "end_idx": 44, "type": "sym", "entity": "甚或眼球固定不动" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "眼内肌" }, { "start_idx": 45, "end_idx": 53, "type": "sym", "entity": "眼内肌一般不受影响" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "瞳孔" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "瞳孔反射多正常" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "眼肌型重症无力" } ] }, { "text": "面肌、舌肌、咀嚼肌及咽喉肌亦易受累闭眼不全额纹及鼻唇沟变浅咀嚼无力,吞咽困难,舌运动不自如无肌束颤动。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "面肌" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "舌肌" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "咀嚼肌" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "咽喉肌" }, { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "面肌、舌肌、咀嚼肌及咽喉肌亦易受累" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "眼" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "闭眼不全" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "额纹" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "鼻唇" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "额纹及鼻唇沟变浅" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "咀嚼无力" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "吞咽困难" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "舌" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "舌运动不自如" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "无肌束颤动" } ] }, { "text": "软腭肌无力发音呈鼻音谈话片刻后音调低沉或声嘶。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "软腭" }, { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肌无力" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "软腭肌无力" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "鼻" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "发音呈鼻音" }, { "start_idx": 10, "end_idx": 21, "type": "sym", "entity": "谈话片刻后音调低沉或声嘶" } ] }, { "text": "称为延髓型(或球型)重症肌无力。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "延髓型(或球型)重症肌无力" } ] }, { "text": "颈肌、躯干及四肢肌也可患病,尤其以肢体近端无力明显抬头困难手托头胸闷气短洗脸及穿衣乏累,行走困难,不能久行。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "颈肌" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "躯干" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "四肢肌" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肢体" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "肢体近端无力明显" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "头" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "抬头困难" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "手" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "头" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "手托头" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "胸" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "胸闷气短" }, { "start_idx": 36, "end_idx": 42, "type": "sym", "entity": "洗脸及穿衣乏累" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "行走困难" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "不能久行" } ] }, { "text": "有的只表现两下肢无力腱反射存在无感觉障碍。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "下肢" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "两下肢无力" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "腱" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "腱反射存在" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "无感觉障碍" } ] }, { "text": "称全身型重症肌无力。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "全身型重症肌无力" } ] }, { "text": "本病主要累及骨骼肌心肌损害MG患者尸检25%~50%有心肌损害重症肌无力伴有其他疾病,如胸腺瘤,其次为甲状腺功能亢进,并少数伴类风湿关节炎、多发性肌炎、红斑狼疮以及自身溶血性贫血等。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "累及骨骼肌" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "心肌" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "心肌损害" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "MG" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "心肌" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "心肌损害" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "重症肌无力" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "胸腺瘤" }, { "start_idx": 51, "end_idx": 57, "type": "dis", "entity": "甲状腺功能亢进" }, { "start_idx": 63, "end_idx": 68, "type": "dis", "entity": "类风湿关节炎" }, { "start_idx": 70, "end_idx": 74, "type": "dis", "entity": "多发性肌炎" }, { "start_idx": 76, "end_idx": 79, "type": "dis", "entity": "红斑狼疮" }, { "start_idx": 82, "end_idx": 88, "type": "dis", "entity": "自身溶血性贫血" } ] }, { "text": "2.MG分型为标明MG肌无力分布部位、程度及病程,一般还采用Ossernen改良法分为以下类型:Ⅰ型(眼肌型)病变仅眼外肌受累Ⅱ型(全身型)ⅡA型表现眼、面和肢体肌无力全身无力并有咽喉肌无力球麻痹型。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "MG" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "MG肌无力" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "Ⅰ型" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "眼肌型" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "眼外肌" }, { "start_idx": 58, "end_idx": 62, "type": "sym", "entity": "眼外肌受累" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "Ⅱ型" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "全身型" }, { "start_idx": 81, "end_idx": 81, "type": "bod", "entity": "眼" }, { "start_idx": 83, "end_idx": 83, "type": "bod", "entity": "面" }, { "start_idx": 85, "end_idx": 86, "type": "bod", "entity": "肢体" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "肌无力" }, { "start_idx": 81, "end_idx": 89, "type": "sym", "entity": "眼、面和肢体肌无力" }, { "start_idx": 90, "end_idx": 93, "type": "sym", "entity": "全身无力" }, { "start_idx": 96, "end_idx": 97, "type": "bod", "entity": "咽喉" }, { "start_idx": 96, "end_idx": 100, "type": "sym", "entity": "咽喉肌无力" }, { "start_idx": 101, "end_idx": 104, "type": "dis", "entity": "球麻痹型" } ] }, { "text": "Ⅲ型(爆发型)突发全身无力,极易发生肌无力危象Ⅳ型(迁缓型)病程反复2年以上,常由Ⅰ型或Ⅱ型发展而来。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "Ⅲ型" }, { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "爆发型" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肌无力" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "极易发生肌无力危象" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "Ⅳ型" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "迁缓型" } ] }, { "text": "Ⅴ型(肌萎缩型)少数病人有肌萎缩。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "Ⅴ型" }, { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "肌萎缩型" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肌萎缩" } ] }, { "text": "感冒、腹泻、激动、疲劳、月经、分娩或手术等常使病情加重,甚至出现危象,危及生命。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "感冒" }, { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "腹泻" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "手术" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "危象" } ] }, { "text": "3.MG危象是指肌无力突然加重呼吸肌(包括膈肌及肋间肌)及咽喉肌严重无力导致呼吸困难。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "MG危象" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "肌无力" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "指肌无力突然加重" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "膈肌" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肋间肌" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "咽喉肌" }, { "start_idx": 15, "end_idx": 35, "type": "sym", "entity": "呼吸肌(包括膈肌及肋间肌)及咽喉肌严重无力" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "导致呼吸困难" } ] }, { "text": "多在重型基础上诱发,感染是危象发生的最常见的诱发因素,伴有胸腺瘤者易发生危象。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "感染" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "胸腺瘤" } ] }, { "text": "危象可分为三种①肌无力危象:为疾病本身肌无力加重所致,此时胆碱酯酶抑制剂往往剂量不足,加大药量或静脉注射腾喜龙后肌力好转。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肌无力危象" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "肌无力" }, { "start_idx": 29, "end_idx": 35, "type": "dru", "entity": "胆碱酯酶抑制剂" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "静脉注射" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "腾喜龙" }, { "start_idx": 56, "end_idx": 57, "type": "ite", "entity": "肌力" } ] }, { "text": "常由感冒诱发,也可发生于应用神经-肌肉阻滞作用的药剂(如链霉素)、大剂量皮质类固醇、胸腺放射治疗或手术后。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "感冒" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "神经" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肌肉" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "链霉素" }, { "start_idx": 36, "end_idx": 40, "type": "dru", "entity": "皮质类固醇" }, { "start_idx": 42, "end_idx": 47, "type": "pro", "entity": "胸腺放射治疗" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "手术" } ] }, { "text": "②胆碱能危象:是由于胆碱酯酶抑制剂过量,使ACh免于水解,在突触积聚过多表现胆碱能毒性反应:肌无力加重肌束颤动烟碱样反应,终板膜过度除极化瞳孔缩小出汗,唾液增多毒素碱样反应);头痛精神紧张(中枢神经反应注射腾喜龙无力症状不见好转,反而加重。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "胆碱能危象" }, { "start_idx": 10, "end_idx": 16, "type": "dru", "entity": "胆碱酯酶抑制剂" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "ACh" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "突触" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "在突触积聚过多" }, { "start_idx": 36, "end_idx": 44, "type": "sym", "entity": "表现胆碱能毒性反应" }, { "start_idx": 46, "end_idx": 48, "type": "ite", "entity": "肌无力" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "肌无力加重" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "肌束" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "肌束颤动" }, { "start_idx": 55, "end_idx": 59, "type": "sym", "entity": "烟碱样反应" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "终板膜" }, { "start_idx": 61, "end_idx": 68, "type": "sym", "entity": "终板膜过度除极化" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "瞳孔" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "瞳孔缩小" }, { "start_idx": 73, "end_idx": 74, "type": "sym", "entity": "出汗" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "唾液" }, { "start_idx": 76, "end_idx": 79, "type": "sym", "entity": "唾液增多" }, { "start_idx": 80, "end_idx": 85, "type": "sym", "entity": "毒素碱样反应" }, { "start_idx": 88, "end_idx": 88, "type": "bod", "entity": "头" }, { "start_idx": 88, "end_idx": 89, "type": "sym", "entity": "头痛" }, { "start_idx": 90, "end_idx": 93, "type": "sym", "entity": "精神紧张" }, { "start_idx": 95, "end_idx": 98, "type": "bod", "entity": "中枢神经" }, { "start_idx": 95, "end_idx": 100, "type": "sym", "entity": "中枢神经反应" }, { "start_idx": 101, "end_idx": 105, "type": "pro", "entity": "注射腾喜龙" }, { "start_idx": 106, "end_idx": 113, "type": "sym", "entity": "无力症状不见好转" }, { "start_idx": 115, "end_idx": 118, "type": "sym", "entity": "反而加重" } ] }, { "text": "③反拗性危象:对胆碱酯酶抑制剂暂时失效,加大药量无济于事。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "反拗性危象" }, { "start_idx": 8, "end_idx": 14, "type": "dru", "entity": "胆碱酯酶抑制剂" } ] }, { "text": "儿科无此危象的报告。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dep", "entity": "儿科" } ] }, { "text": "【诊断】(一)确定是否重症肌无力主要根据病史,典型的临床表现即受累骨骼肌活动后疲劳无力肌群可分成眼外肌、颜面肌、咽喉肌、颈肌、躯干肌和肢体肌等,经休息或用胆碱酯酶抑制剂可以缓解;且无神经系统其他体征。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "重症肌无力" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 31, "end_idx": 42, "type": "sym", "entity": "受累骨骼肌活动后疲劳无力" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "肌群" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "眼外肌" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "颜面肌" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "咽喉肌" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "颈肌" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "躯干肌" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "肢体肌" }, { "start_idx": 73, "end_idx": 74, "type": "pro", "entity": "休息" }, { "start_idx": 77, "end_idx": 83, "type": "pro", "entity": "胆碱酯酶抑制剂" } ] }, { "text": "1.疲劳实验阳性受累肌群连续运动后症状明显加重肌疲劳现象肌无力较轻、检查配合的年长儿童可选择疲劳试验。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "疲劳实验" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肌群" }, { "start_idx": 8, "end_idx": 22, "type": "sym", "entity": "受累肌群连续运动后症状明显加重" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "肌" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "肌疲劳现象" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "肌无" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "肌无" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "童可选择" } ] }, { "text": "成人MG患者强调定量疲劳实验,即选择不同的受累肌群,让其持续用力收缩,测量出现病态疲劳现象所需的时间及疲劳程度,并且制定有专项的评定量表。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "成人MG" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "疲劳实验" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肌群" } ] }, { "text": "但儿童MG以年幼儿童发病为主,检查依从性差,尚缺少年龄相关的儿童专项定量疲劳实验量表。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "儿童MG" } ] }, { "text": "2.药物实验阳性甲基硫酸新斯的明实验:0.03~0.04mg/kg,肌注,比较注射前后半小时各受累肌群的肌力的变化,肌力明显改善者有助于MG的诊断腾喜龙试验:腾喜龙0.2mg/kg,以注射用水稀释至1ml,静脉注射,症状迅速缓解则为阳性,持续10分钟左右又恢复原状。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "药物实验" }, { "start_idx": 8, "end_idx": 17, "type": "pro", "entity": "甲基硫酸新斯的明实验" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "肌注" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "肌群" }, { "start_idx": 52, "end_idx": 53, "type": "ite", "entity": "肌力" }, { "start_idx": 58, "end_idx": 59, "type": "ite", "entity": "肌力" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "MG" }, { "start_idx": 58, "end_idx": 72, "type": "sym", "entity": "肌力明显改善者有助于MG的诊断" }, { "start_idx": 73, "end_idx": 77, "type": "pro", "entity": "腾喜龙试验" }, { "start_idx": 79, "end_idx": 81, "type": "dru", "entity": "腾喜龙" }, { "start_idx": 103, "end_idx": 106, "type": "pro", "entity": "静脉注射" } ] }, { "text": "对疲劳实验改善不明显者、肌无力较重病例以及疲劳实验不合作的年幼儿童选择药物试验。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "疲劳实验" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肌无" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "肌无" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "病例以及" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "儿童选择" } ] }, { "text": "3.肌电图神经低频重复电刺激示复合肌肉动作电位波幅衰减10%单纤维肌电图检查显示颤抖增宽,是目前敏感性及准确性最高的电生理检测手段。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肌电图" }, { "start_idx": 5, "end_idx": 13, "type": "pro", "entity": "神经低频重复电刺激" }, { "start_idx": 15, "end_idx": 24, "type": "ite", "entity": "复合肌肉动作电位波幅" }, { "start_idx": 15, "end_idx": 29, "type": "sym", "entity": "复合肌肉动作电位波幅衰减10%" }, { "start_idx": 30, "end_idx": 37, "type": "pro", "entity": "单纤维肌电图检查" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "颤抖增宽" }, { "start_idx": 58, "end_idx": 62, "type": "pro", "entity": "电生理检测" } ] }, { "text": "前者阴性不能排除MG,后者在国内,特别是儿童尚未广泛开展。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "MG" } ] }, { "text": "4.血清AChRab的检测AChRab检测是MG诊断重要的参考依据,若阳性者有助于诊断,阴性者不能排除MG。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "血清AChRab的检测" }, { "start_idx": 13, "end_idx": 20, "type": "pro", "entity": "AChRab检测" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "MG" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "MG" } ] }, { "text": "眼肌型及儿童MG病例AChRab多阴性。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "眼肌型" }, { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "儿童MG" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "AChRab" } ] }, { "text": "(二)明确是否合并胸腺瘤成人病例约75%胸腺增生,15%MG合并胸腺瘤;我院(复旦大学附属儿童医院)资料4%胸腺瘤,42%胸腺增生。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "胸腺瘤" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "胸腺增生" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "MG" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "胸腺瘤" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "胸腺瘤" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "胸腺增生" } ] }, { "text": "肿瘤常位于前上纵隔,除表现肌无力,一般无占位病变的症状和体征,易漏诊。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肌无力" }, { "start_idx": 19, "end_idx": 29, "type": "sym", "entity": "无占位病变的症状和体征" } ] }, { "text": "胸腺瘤多见于40岁以后男性患者,肌无力症状较重对胆碱酯酶抑制剂疗效不佳易发生危象。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "胸腺瘤" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "肌无力" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "肌无力症状较重" }, { "start_idx": 24, "end_idx": 30, "type": "dru", "entity": "胆碱酯酶抑制剂" }, { "start_idx": 23, "end_idx": 34, "type": "sym", "entity": "对胆碱酯酶抑制剂疗效不佳" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "易发生危象" } ] }, { "text": "侧位或正位X光胸片偶可发现异常,纵隔CT扫描可直接显示肿瘤部位、大小、形状以及与邻近器官的关系。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "侧位或正位X光胸片" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "纵隔CT扫描" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "肿瘤" } ] }, { "text": "免疫学检查:CAEab(又称胸腺瘤相关抗体)对MG患者提示胸腺瘤具有重要价值。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "免疫学检查" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "CAEab" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "胸腺瘤" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "MG" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "胸腺瘤" } ] }, { "text": "MG合并胸腺瘤CAEab阳性率高达80%~90%。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "MG" }, { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "胸腺瘤CAEab" } ] }, { "text": "诊断尚需结合临床和CT纵隔扫描,综合分析。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "CT纵隔扫描" } ] }, { "text": "(三)明确有无其他并存症MG作为自身免疫疾病中一种“姐妹病”,可伴有以下夹杂症:如甲状腺功能亢进,类风湿关节炎,系统性红斑狼疮,溶血性贫血,多发性肌炎或多发性硬化等。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "MG" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "姐妹病" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "甲状腺功能亢进" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "类风湿关节炎" }, { "start_idx": 56, "end_idx": 62, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 70, "end_idx": 74, "type": "dis", "entity": "多发性肌炎" }, { "start_idx": 76, "end_idx": 80, "type": "dis", "entity": "多发性硬化" } ] }, { "text": "(四)鉴别诊断MG急性肌无力应与其他急性瘫痪疾病鉴别:包括①周期性瘫痪。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "MG" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肌无力" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "瘫痪疾病" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "周期性瘫痪" } ] }, { "text": "常在夜间发病,醒来时发现四肢无力血钾低心电图出现U波补钾治疗有效。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "四肢" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "四肢无力" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "血钾" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "血钾低" }, { "start_idx": 19, "end_idx": 21, "type": "pro", "entity": "心电图" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "心电图出现U波" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "补钾治疗" } ] }, { "text": "②急性炎症性脱髓鞘多发神经根病。", "entities": [ { "start_idx": 1, "end_idx": 14, "type": "dis", "entity": "急性炎症性脱髓鞘多发神经根病" } ] }, { "text": "病初有发热或腹泻,除肢体瘫痪外,尚有神经根牵拉痛脑脊液有蛋白-细胞分离现象脊髓炎。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "发热" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "腹泻" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "肢体瘫痪" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "神经根" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "神经根牵拉痛" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "脑脊液" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "蛋白" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "细胞" }, { "start_idx": 24, "end_idx": 36, "type": "sym", "entity": "脑脊液有蛋白-细胞分离现象" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "脊髓炎" } ] }, { "text": "有发热及脊髓损害上运动神经元型瘫痪、横截型感觉障碍及排尿障碍)。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "发热" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "脊髓" }, { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "脊髓损害" }, { "start_idx": 8, "end_idx": 16, "type": "dis", "entity": "上运动神经元型瘫痪" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "横截型感觉障碍" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "排尿障碍" } ] }, { "text": "慢性肌无力需要和以下疾病鉴别:包括①动眼神经麻痹。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "慢性肌无力" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "动眼神经麻痹" } ] }, { "text": "麻痹侧除上睑下垂可见瞳孔散大眼球向上、下及内收运动受限神经炎或颅内动脉瘤。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "上睑" }, { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "上睑下垂" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "瞳孔" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "可见瞳孔散大" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "眼球" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "眼球向上、下及内收运动受限" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "神经炎" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "颅内动脉瘤" } ] }, { "text": "②多发性肌炎。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "多发性肌炎" } ] }, { "text": "四肢近端肌无力肌痛肌酶升高肌营养不良。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "四肢" }, { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "近端肌" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "四肢近端肌无力" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肌" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "肌痛" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肌酶" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "肌酶升高" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "肌营养不良" } ] }, { "text": "缓慢进行性肢体无力,肌萎缩,儿童患者翼状肩胛,腓肠肌假肥大血肌酶升高线粒体肌病。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "缓慢进行性肢体无力" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肌萎缩" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "翼状肩胛" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "腓肠肌" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "腓肠肌假肥大" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "血肌酶" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "血肌酶升高" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "线粒体肌病" } ] }, { "text": "骨骼肌极度不能耐受疲劳血乳酸升高,肌活体组织检查可见不整红边纤维电镜示异常线粒体糖原累积病。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "骨骼肌极度不能耐受疲劳" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "血乳酸升高" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "肌活体组织检查" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "纤维" }, { "start_idx": 17, "end_idx": 31, "type": "sym", "entity": "肌活体组织检查可见不整红边纤维" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "电镜" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "线粒体" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "电镜示异常线粒体" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "糖原累积病" } ] }, { "text": "其中尤其以Ⅱ型患者,酸性麦芽糖酶缺乏引起肢带肌无力,可出现呼吸肌麻痹,易误诊,肌活体组织检查PAS染色可见糖原积累癌性肌无力,主要多见于年老患者小细胞肺癌,肢体无力活动后缓解,高频反复电刺激神经肌电图示肌电位递增运动神经元病。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "酸性麦芽糖酶缺乏" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "肢带肌无力" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "呼吸肌麻痹" }, { "start_idx": 39, "end_idx": 50, "type": "pro", "entity": "肌活体组织检查PAS染色" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "糖原" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "糖原积累" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "癌性肌无力" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "小细胞肺癌" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "肢体" }, { "start_idx": 78, "end_idx": 81, "type": "sym", "entity": "肢体无力" }, { "start_idx": 82, "end_idx": 86, "type": "sym", "entity": "活动后缓解" }, { "start_idx": 88, "end_idx": 99, "type": "pro", "entity": "高频反复电刺激神经肌电图" }, { "start_idx": 101, "end_idx": 103, "type": "ite", "entity": "肌电位" }, { "start_idx": 88, "end_idx": 105, "type": "sym", "entity": "高频反复电刺激神经肌电图示肌电位递增" }, { "start_idx": 106, "end_idx": 111, "type": "dis", "entity": "运动神经元病" } ] }, { "text": "早期仅表现舌及肢体肌无力肌萎缩、肌纤维颤动或锥体束征则鉴别不难。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "舌" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肢体" }, { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "舌及肢体肌无力" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "肌萎缩" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "肌纤维颤动" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "锥体束征" } ] }, { "text": "【治疗】(一)胆碱酯酶抑制剂(AchEI)可选用溴化新斯的明,剂量每次0.5mg/kg日服3~4次;吡啶新斯的明,剂量每次2mg/kg,日服4次;溴化吡啶新斯的明,每次剂量7mg/kg,日服3次。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dru", "entity": "胆碱酯酶抑制剂" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "AchEI" }, { "start_idx": 24, "end_idx": 29, "type": "dru", "entity": "溴化新斯的明" }, { "start_idx": 50, "end_idx": 55, "type": "dru", "entity": "吡啶新斯的明" }, { "start_idx": 73, "end_idx": 80, "type": "dru", "entity": "溴化吡啶新斯的明" } ] }, { "text": "总之,胆碱酯酶抑制剂作为一种有效的对症、辅助治疗药物,不宜长期单独应用。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dru", "entity": "胆碱酯酶抑制剂" } ] }, { "text": "长期依赖,滥用胆碱酯酶抑制剂,有碍AchR修复,须避免此类药物的弊端。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dru", "entity": "胆碱酯酶抑制剂" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "AchR" } ] }, { "text": "辅助药物如氯化钾和麻黄碱等可加强新斯的明的作用。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "氯化钾" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "麻黄碱" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "新斯的明" } ] }, { "text": "忌用对神经-肌肉传递阻滞的药物,如各种氨基糖苷类的抗生素、奎宁、奎宁丁、普鲁卡因胺、普萘洛尔、氯丙嗪以及各种肌肉松弛剂。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "神经" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肌肉" }, { "start_idx": 19, "end_idx": 27, "type": "dru", "entity": "氨基糖苷类的抗生素" }, { "start_idx": 29, "end_idx": 30, "type": "dru", "entity": "奎宁" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "奎宁丁" }, { "start_idx": 36, "end_idx": 40, "type": "dru", "entity": "普鲁卡因胺" }, { "start_idx": 42, "end_idx": 45, "type": "dru", "entity": "普萘洛尔" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "氯丙嗪" }, { "start_idx": 54, "end_idx": 58, "type": "dru", "entity": "肌肉松弛剂" } ] }, { "text": "(二)免疫抑制剂1.皮质类固醇为最常用的免疫治疗药物,无论是眼肌型还是全身型都可选用泼尼松,1~1.5mg/(kg•d)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "皮质类固醇" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "眼肌型" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "全身型" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "泼尼松" } ] }, { "text": "或病初使用甲泼尼龙冲击疗法,儿童20mg/(kg•d),静脉滴注,连用3~5天,起效快,适用重症或危象患者,用药方便,甚至可取代血浆交换疗法。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "甲泼尼龙冲击疗法" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 64, "end_idx": 69, "type": "pro", "entity": "血浆交换疗法" } ] }, { "text": "但有一过性高血糖、高血压、继发感染及胃出血等不良反应,值得重视。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "一过性高血糖" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "高血压" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "继发感染" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "胃出血" } ] }, { "text": "病情缓解后逐渐减量改为泼尼松小剂量,隔日晨服,维持至少1年以上。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "泼尼松" } ] }, { "text": "大剂量类固醇可使病情加重,多发生在用药1周内,可促发危象。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "类固醇" } ] }, { "text": "发生机制是直接阻抑AChR离子通道。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "bod", "entity": "AChR离子通道" } ] }, { "text": "2.其他免疫抑制剂可选用环磷酰胺、硫唑嘌呤或环孢素,对难治病例、发生危象病例以及胸腺切除术后疗效不佳者有效。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "环孢素" }, { "start_idx": 40, "end_idx": 44, "type": "pro", "entity": "胸腺切除术" } ] }, { "text": "需注意血象和肝、肾功能的变化。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "血象" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肝" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "肾功能" } ] }, { "text": "(三)放射治疗至今胸腺放射治疗还是对MG一种确实有效的治疗方法。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "放射治疗" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "胸腺放射治疗" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "MG" } ] }, { "text": "被称作是“非手术的手术治疗”。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "非手术的手术治疗" } ] }, { "text": "适用于:①MG药物疗效不明显者,最好于发病2~3年内及早放射治疗;②巨大或多个胸腺瘤,无法手术或作为术前准备治疗;③恶性肿瘤术后追加放射治疗。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "MG" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "胸腺瘤" }, { "start_idx": 58, "end_idx": 61, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 66, "end_idx": 69, "type": "pro", "entity": "放射治疗" } ] }, { "text": "(四)胸腺切除胸腺切除仍然是MG的基本疗法。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "胸腺切除" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "胸腺切除" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "MG" } ] }, { "text": "适用于:①全身型MG,药物疗效不佳,宜尽早手术。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "全身型MG" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "手术" } ] }, { "text": "发病3~5年内中年女性患者手术疗效甚佳。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "手术" } ] }, { "text": "②伴有胸腺瘤的各型MG患者,疗效虽较差,应尽可能手术切除病灶。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "胸腺瘤" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "MG" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "手术" } ] }, { "text": "③儿童眼肌型患者,手术虽有效,是否值得手术仍有争议。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "儿童眼肌型" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "手术" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "手术" } ] }, { "text": "手术后继续用泼尼松1年。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "泼尼松" } ] }, { "text": "(五)血浆交换及血浆净化治疗能迅速清除血浆中AChRab及免疫复合物等,用于抢救危象。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "血浆交换" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "血浆净化治疗" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血浆" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "AChRab" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "危象" } ] }, { "text": "(六)丙种球蛋白用大剂量丙种球蛋,0.4g/(kg•d),静脉滴注,连用5天。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "丙种球蛋白" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "丙种球蛋" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "治疗病情严重全身型MG患者,迅速扭转危象,或用于手术前准备,安全有效。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "全身型MG" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "危象" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "手术" } ] }, { "text": "(七)危象的处理儿科病例危象发生率2.2%,病死率0.8%。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "危象" }, { "start_idx": 8, "end_idx": 9, "type": "dep", "entity": "儿科" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "危象" } ] }, { "text": "一旦发生危象,呼吸肌瘫痪气管插管或气管切开,应用人工呼吸器辅助呼吸,同时明确何种危象,进行对症处理。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "危象" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "呼吸肌瘫痪" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "气管插管" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "气管切开" }, { "start_idx": 24, "end_idx": 28, "type": "equ", "entity": "人工呼吸器" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "辅助呼吸" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "危象" } ] }, { "text": "在危象处理过程中保持气道护理的无菌操作、雾化吸入、保持呼吸道通畅、防止肺部感染及肺不张等并发症是抢救成功的关键。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "危象" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "气道" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "无菌操作" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "保持呼吸道通畅" }, { "start_idx": 33, "end_idx": 38, "type": "pro", "entity": "防止肺部感染" }, { "start_idx": 40, "end_idx": 42, "type": "pro", "entity": "肺不张" } ] }, { "text": "【预后】本病的预后,一些病例在发病后数月或数年后自行缓解;一些儿童期病例可持续到成人时期;眼肌型在青春前发病者预后较青春后发病者好;少数儿童病例病程迁延,其间可缓解、复发或恶化;多数病例经免疫抑制剂、胸腺切除及胸腺放疗等治疗可能得以治愈。", "entities": [ { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "眼肌型" }, { "start_idx": 94, "end_idx": 98, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 100, "end_idx": 103, "type": "pro", "entity": "胸腺切除" }, { "start_idx": 105, "end_idx": 108, "type": "pro", "entity": "胸腺放疗" } ] }, { "text": "第八节流行性腮腺炎流行性腮腺炎(mumps,epidemicparotitis)是由腮腺炎病毒引起的急性呼吸道传染病。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "流行性腮腺炎" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "流行性腮腺炎" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "mumps" }, { "start_idx": 22, "end_idx": 38, "type": "dis", "entity": "epidemicparotitis" }, { "start_idx": 42, "end_idx": 46, "type": "mic", "entity": "腮腺炎病毒" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "急性呼吸道传染病" } ] }, { "text": "病毒对腺体和神经组织具有亲和力。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "腺体" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "神经组织" } ] }, { "text": "【病原和流行病学】腮腺炎病毒(mumpsvirus)属副黏病毒。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "腮腺炎病毒" }, { "start_idx": 15, "end_idx": 24, "type": "mic", "entity": "mumpsvirus" } ] }, { "text": "病毒只有一个血清型,有6种主要结构蛋白。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" } ] }, { "text": "M蛋白在病毒包装中起决定作用。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "mic", "entity": "病毒" } ] }, { "text": "病毒体外可在许多原代细胞和细胞系内增殖。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "细胞" } ] }, { "text": "紫外线、甲醛、乙醚、56℃20分钟均可被灭活。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dru", "entity": "甲醛" }, { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "乙醚" } ] }, { "text": "4℃存活数天,加病毒保护剂-70℃可长期保存。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "mic", "entity": "病毒" } ] }, { "text": "在腮腺肿大前6天到后9天,均可自患者唾液中检出病毒。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "腮腺" }, { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "腮腺肿大" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "唾液" }, { "start_idx": 23, "end_idx": 24, "type": "mic", "entity": "病毒" } ] }, { "text": "病毒经呼吸道途径传播。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "呼吸道" } ] }, { "text": "如有再次患腮腺炎者可能系免疫缺陷患者或为其他病毒感染所致。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "病毒感染" } ] }, { "text": "婴儿因有母体被动抗体保护(维持9个月)而很少发病。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "抗体" } ] }, { "text": "孕妇患病可将病毒经胎盘感染胎儿。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "病毒" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胎盘" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胎儿" } ] }, { "text": "据估计,在应用疫苗前,妊娠期腮腺炎发病率为0.8/万~10/万。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "疫苗" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "腮腺炎" } ] }, { "text": "【发病机制和病理改变】病毒侵入后先在上呼吸道黏膜上皮细胞内增殖,再播散至引流淋巴结,随后发生病毒血症,病毒随血流传播至腺样组织或其他部位。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "病毒" }, { "start_idx": 18, "end_idx": 27, "type": "bod", "entity": "上呼吸道黏膜上皮细胞" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "淋巴结" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "病毒血症" }, { "start_idx": 51, "end_idx": 52, "type": "mic", "entity": "病毒" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "血流" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "腺样组织" } ] }, { "text": "唾液腺感染最为突出,其他包括内耳、胰腺、心脏、神经系统(脑膜和脑)、关节、肾、肝、性腺和甲状腺。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "唾液腺" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "唾液腺感染" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "内耳" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "胰腺" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "心脏" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "神经系统" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "脑膜" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "脑" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "关节" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "肾" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "肝" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "性腺" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "甲状腺" } ] }, { "text": "在唾液腺,病毒感染小管上皮细胞,引起腺管周围间质水肿和局部炎症反应。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "唾液腺" }, { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "病毒" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "小管上皮细胞" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "腺管周围间质" }, { "start_idx": 18, "end_idx": 32, "type": "sym", "entity": "腺管周围间质水肿和局部炎症反应" } ] }, { "text": "淋巴细胞、巨噬细胞浸润和受累细胞脱落使管腔阻塞。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "受累细胞" }, { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "淋巴细胞、巨噬细胞浸润和受累细胞脱落" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "管腔" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "管腔阻塞" } ] }, { "text": "病毒经感染单核细胞通过脉络丛侵入中枢神经系统,在脉络丛和室管膜细胞内增殖,感染细胞脱落进入脑脊液,进而引起脑膜炎。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "感染单核细胞" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "脉络丛" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "脉络丛" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "室管膜细胞" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "感染细胞" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "脑脊液" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "脑炎时,脑室周围单核细胞浸润,散在噬神经细胞病灶和小神经胶质细胞增生,并见脑室周围脱髓鞘病变。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "脑炎" }, { "start_idx": 4, "end_idx": 11, "type": "bod", "entity": "脑室周围单核细胞" }, { "start_idx": 4, "end_idx": 13, "type": "sym", "entity": "脑室周围单核细胞浸润" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "噬神经细胞" }, { "start_idx": 25, "end_idx": 31, "type": "bod", "entity": "小神经胶质细胞" }, { "start_idx": 15, "end_idx": 33, "type": "sym", "entity": "散在噬神经细胞病灶和小神经胶质细胞增生" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "脑室" }, { "start_idx": 37, "end_idx": 45, "type": "sym", "entity": "脑室周围脱髓鞘病变" } ] }, { "text": "睾丸炎时,病毒在细精管增殖,引起组织间质水肿和淋巴细胞浸润。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "睾丸炎" }, { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "病毒" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "细精管" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "组织间质" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 16, "end_idx": 28, "type": "sym", "entity": "组织间质水肿和淋巴细胞浸润" } ] }, { "text": "胰腺受累时,胰导管上皮细胞肿胀,坏死脱落,与炎性渗出物等阻塞管腔,致胰液潴留,大量淀粉酶反流入血。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胰腺" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "胰腺受累" }, { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "胰导管上皮细胞" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "管腔" }, { "start_idx": 6, "end_idx": 31, "type": "sym", "entity": "胰导管上皮细胞肿胀,坏死脱落,与炎性渗出物等阻塞管腔" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "胰液潴留" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "血" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "大量淀粉酶反流入血" } ] }, { "text": "【临床表现】潜伏期为12~25天,一般为16~18天,约30%~40%患者为隐性感染。", "entities": [ { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "隐性感染" } ] }, { "text": "临床上以腮腺炎最为常见,其他表现可在腮腺炎前、同时或后发生,也可发生在无腮腺炎时。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "腮腺炎" } ] }, { "text": "在5岁以下,可能仅引起上呼吸道感染,而无腮腺炎。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "腮腺炎" } ] }, { "text": "(一)腮腺炎典型病例先有发热、头痛、厌食和不适。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "发热" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "头痛" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "厌食" } ] }, { "text": "在24小时内,患者诉有“耳痛”,咀嚼时加剧。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "耳痛" } ] }, { "text": "次日腮腺逐渐肿大,于1~3天内达高峰。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "腮腺" }, { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "腮腺逐渐肿大" } ] }, { "text": "腮腺以耳垂为中心呈马鞍形肿大,触之有轻度压痛,腮腺急骤肿胀时疼痛比较严重,持续4~5天后逐渐缩小,整个过程为6~10天。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "腮腺" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "耳垂" }, { "start_idx": 0, "end_idx": 21, "type": "sym", "entity": "腮腺以耳垂为中心呈马鞍形肿大,触之有轻度压痛" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "腮腺" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "腮腺急骤肿胀" } ] }, { "text": "通常一侧腮腺先肿大,数日内对侧肿大,约25%患者只有单侧肿大。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "腮腺" }, { "start_idx": 2, "end_idx": 16, "type": "sym", "entity": "一侧腮腺先肿大,数日内对侧肿大" } ] }, { "text": "其他唾液腺如颌下腺或舌下腺可同时肿大,或单独肿大。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "唾液腺" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "颌下腺" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "舌下腺" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "肿大" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "肿大" } ] }, { "text": "(二)脑膜脑炎较常见。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "脑膜脑炎" } ] }, { "text": "常发生在腮腺炎后3~10天,也可在腮腺受累前。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "腮腺" } ] }, { "text": "脑脊液呈无菌性脑膜炎改变。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑脊液" }, { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "无菌性脑膜炎" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "脑脊液呈无菌性脑膜炎改变" } ] }, { "text": "(三)睾丸炎、附睾炎10岁后男性患者约有20%~35%发生,多为单侧。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "睾丸炎" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "附睾炎" } ] }, { "text": "约半数病例以后睾丸发生萎缩。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "睾丸" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "睾丸发生萎缩" } ] }, { "text": "因常为单侧受累,很少影响生育。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "单侧受累" } ] }, { "text": "(四)胰腺炎为严重的少见表现。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "轻型或亚临床型感染远较已诊断者多。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "轻型或亚临床型感染" } ] }, { "text": "(五)其他女性患者可有卵巢炎。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "卵巢炎" } ] }, { "text": "(六)宫内感染孕早期患腮腺炎时,胎儿病死率增高,有报告腮腺病毒与胎儿、新生儿原发性心内膜纤维弹力组织增生症有关,但有人持反对意见。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "宫内感染" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 27, "end_idx": 30, "type": "mic", "entity": "腮腺病毒" }, { "start_idx": 32, "end_idx": 52, "type": "dis", "entity": "胎儿、新生儿原发性心内膜纤维弹力组织增生症" } ] }, { "text": "对腮腺病毒致畸作用尚有争议。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "mic", "entity": "腮腺病毒" }, { "start_idx": 6, "end_idx": 6, "type": "dis", "entity": "畸" } ] }, { "text": "【诊断】根据流行病学资料、接触史和典型腮腺炎表现易确定诊断,缺乏腮腺炎表现或接种过疫苗的患儿需进行病原学诊断,方法包括:(一)病毒分离收集急性期唾液标本和脑膜脑炎发生后5天内脑脊液接种敏感细胞,病变细胞可见胞质内嗜酸性包涵体,形成多核巨细胞。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 41, "end_idx": 42, "type": "dru", "entity": "疫苗" }, { "start_idx": 63, "end_idx": 66, "type": "pro", "entity": "病毒分离" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "唾液" }, { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "脑膜脑炎" }, { "start_idx": 87, "end_idx": 89, "type": "bod", "entity": "脑脊液" }, { "start_idx": 103, "end_idx": 104, "type": "bod", "entity": "胞质" } ] }, { "text": "用特异性抗血清可早期快速检出培养物中病毒(免疫荧光法)。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "血清" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "病毒" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "免疫荧光法" } ] }, { "text": "(二)特异性抗体检测用补体结合试验、血凝抑制试验或ELISA法检测双份血清,特异IgG≥4倍增高可建立诊断,但因腮腺炎病毒与副流感病毒间存在交叉抗体反应,故此法并不理想。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "特异性抗体检测" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "血凝抑制试验" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "ELISA法" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "血清" }, { "start_idx": 38, "end_idx": 42, "type": "ite", "entity": "特异IgG" }, { "start_idx": 56, "end_idx": 60, "type": "mic", "entity": "腮腺炎病毒" }, { "start_idx": 62, "end_idx": 66, "type": "mic", "entity": "副流感病毒" } ] }, { "text": "特异性IgM阳性提示近期感染(IgM在疾病早期出现,持续60天)。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "特异性IgM阳性" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "感染" } ] }, { "text": "【鉴别诊断】(一)急性淋巴结炎耳前、颈浅上和下颌角淋巴结发炎肿大伴有周围组织水肿时,易于腮腺炎混淆。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "急性淋巴结炎" }, { "start_idx": 15, "end_idx": 27, "type": "bod", "entity": "耳前、颈浅上和下颌角淋巴结" }, { "start_idx": 15, "end_idx": 39, "type": "sym", "entity": "耳前、颈浅上和下颌角淋巴结发炎肿大伴有周围组织水肿" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "腮腺炎" } ] }, { "text": "而颌下和颏下淋巴结炎时,又易与颌下和舌下腺炎混淆。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "dis", "entity": "颌下和颏下淋巴结炎" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "颌下和舌下腺炎" } ] }, { "text": "这些淋巴结炎局部疼痛较重,且常有头面部或口咽部感染病灶,周围血象中白细胞总数和中性粒细胞均增高。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "淋巴结炎" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "头面部" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "口咽部" }, { "start_idx": 33, "end_idx": 37, "type": "ite", "entity": "白细胞总数" }, { "start_idx": 39, "end_idx": 43, "type": "ite", "entity": "中性粒细胞" } ] }, { "text": "(二)急性化脓性腮腺炎病原多为金黄色葡萄球菌。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "急性化脓性腮腺炎" }, { "start_idx": 15, "end_idx": 21, "type": "mic", "entity": "金黄色葡萄球菌" } ] }, { "text": "外周血白细胞总数和中性粒细胞增高。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "外周血白细胞总数" }, { "start_idx": 9, "end_idx": 13, "type": "ite", "entity": "中性粒细胞" }, { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "外周血白细胞总数和中性粒细胞增高" } ] }, { "text": "(三)复发性腮腺炎病因不明,可因感染和药物过敏所致,腮腺反复肿大,有些患者作腮腺管X线造影可见结石。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "复发性腮腺炎" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "腮腺" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "腮腺反复肿大" }, { "start_idx": 38, "end_idx": 44, "type": "pro", "entity": "腮腺管X线造影" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "结石" } ] }, { "text": "(四)其他病毒所致腮腺炎柯萨基病毒和副流感病毒可致腮腺炎,需借助病原学检查鉴别。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "病毒" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 12, "end_idx": 16, "type": "mic", "entity": "柯萨基病毒" }, { "start_idx": 18, "end_idx": 22, "type": "mic", "entity": "副流感病毒" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "腮腺炎" } ] }, { "text": "【预防】一般预防应隔离患者至腮腺肿胀完全消退为止。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "隔离患者" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "腮腺" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "腮腺肿胀" } ] }, { "text": "孕早期易感孕妇应避免接触患者,以免造成胎儿感染。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "感染" } ] }, { "text": "腮腺炎减毒活疫苗(JerylLynn株)接种后产生亚临床感染,诱生的抗体可维持至少20年。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dru", "entity": "腮腺炎减毒活疫苗" }, { "start_idx": 9, "end_idx": 18, "type": "dru", "entity": "JerylLynn株" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "亚临床感染" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "抗体" } ] }, { "text": "应用麻疹-腮腺炎-风疹(MMR)三联疫苗抗体阳转率可达95%以上,推荐对大于12月龄小儿、青春期和成年无自然感染史者普遍接种。", "entities": [ { "start_idx": 2, "end_idx": 19, "type": "dru", "entity": "麻疹-腮腺炎-风疹(MMR)三联疫苗" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "抗体" }, { "start_idx": 60, "end_idx": 61, "type": "pro", "entity": "接种" } ] }, { "text": "12月龄以内婴儿因存在母传抗体干扰,不宜接种。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "接种" } ] }, { "text": "孕期、严重发热性疾病、过敏、近期接受过免疫球蛋白和免疫抑制患者不能接种。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "严重发热性疾病" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "过敏" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "免疫球蛋白" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "接种" } ] }, { "text": "可用中药板蓝根口服口服或注射,或用青黛散调醋局部涂敷。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "中药板蓝根" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "口服" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "注射" }, { "start_idx": 17, "end_idx": 25, "type": "pro", "entity": "青黛散调醋局部涂敷" } ] }, { "text": "发生睾丸炎时,局部冷湿敷,并将阴囊吊起。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "睾丸炎" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "局部冷湿敷" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "阴囊" } ] }, { "text": "发生胰腺炎或脑膜炎时,应作相应处理。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "第四节曲霉菌病【病原学和发病机制】曲霉菌(aspergillosis)可分为18个群、132个种和18个变种,绝大多数分为非致病菌,对人类致病者有烟曲霉、黄曲霉、黑曲霉、土曲霉、构巢曲霉等,其中以烟曲霉最常见。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "曲霉菌病" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "曲霉菌" }, { "start_idx": 21, "end_idx": 33, "type": "mic", "entity": "aspergillosis" }, { "start_idx": 61, "end_idx": 64, "type": "mic", "entity": "非致病菌" }, { "start_idx": 73, "end_idx": 75, "type": "mic", "entity": "烟曲霉" }, { "start_idx": 77, "end_idx": 79, "type": "mic", "entity": "黄曲霉" }, { "start_idx": 81, "end_idx": 83, "type": "mic", "entity": "黑曲霉" }, { "start_idx": 85, "end_idx": 87, "type": "mic", "entity": "土曲霉" }, { "start_idx": 89, "end_idx": 92, "type": "mic", "entity": "构巢曲霉" }, { "start_idx": 98, "end_idx": 100, "type": "mic", "entity": "烟曲霉" } ] }, { "text": "在各种曲霉病中,可为单一曲霉菌的感染,也可两种以上曲霉合并感染。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "曲霉病" }, { "start_idx": 12, "end_idx": 14, "type": "mic", "entity": "曲霉菌" }, { "start_idx": 25, "end_idx": 26, "type": "mic", "entity": "曲霉" } ] }, { "text": "曲霉菌的致病方式有以下几种:(1)原发性侵袭型:机体抵抗力正常,吸入大量的病原体,使机体感染,引起急性肺炎表现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "曲霉菌" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "急性肺炎" } ] }, { "text": "(2)继发性侵袭型:机体患有严重疾病或长期应用大量抗生素、免疫抑制剂,此型较为常见。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "抗生素" }, { "start_idx": 29, "end_idx": 33, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "(3)变态反应型:因吸入大量曲霉孢子而引起过敏反应。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "mic", "entity": "曲霉孢子" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "过敏反应" } ] }, { "text": "(4)寄生型:曲霉菌寄生在支气管扩张的空腔内和肺结核的空洞内。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "曲霉菌" }, { "start_idx": 13, "end_idx": 20, "type": "bod", "entity": "支气管扩张的空腔" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "肺结核的空洞" } ] }, { "text": "本病为外源性感染,主要是肺部吸入大量的曲霉菌孢子,侵入血流播散至全身各器官。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "外源性感染" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肺部" }, { "start_idx": 19, "end_idx": 23, "type": "mic", "entity": "曲霉菌孢子" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血流" } ] }, { "text": "其次是皮肤创伤性接种。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "皮肤" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "接种" } ] }, { "text": "主要有渗出性炎症、脓肿、坏死溃疡、和肉芽肿等4种类型。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "渗出性炎症" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "脓肿" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "坏死溃疡" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "肉芽肿" } ] }, { "text": "脓肿中常可见到菌丝。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "脓肿中常可见到菌丝" } ] }, { "text": "HE染色呈蓝色略带红色,PAS染色红色,嗜银染色呈黑色。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "HE染色呈蓝色略带红色" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "PAS染色红色" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "嗜银染色呈黑色" } ] }, { "text": "慢性肺曲霉菌病特点为肺组织损伤显著,但曲霉菌成分仅少量,或菌丝发生变型,故称半侵袭性肺曲霉菌病,应注意仔细反复寻找。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "慢性肺曲霉菌病" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "肺组织损伤显著" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "曲霉菌成分仅少量" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "菌丝发生变型" }, { "start_idx": 38, "end_idx": 46, "type": "dis", "entity": "半侵袭性肺曲霉菌病" } ] }, { "text": "【临床类型】由于机体免疫状态和易感性不同,曲霉菌侵入肺部可以引起下列3种表现:曲霉菌球、过敏性支气管肺曲霉菌病、侵袭性肺部曲霉菌病。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "曲霉菌" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肺部" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "曲霉菌球" }, { "start_idx": 44, "end_idx": 54, "type": "dis", "entity": "过敏性支气管肺曲霉菌病" }, { "start_idx": 56, "end_idx": 64, "type": "dis", "entity": "侵袭性肺部曲霉菌病" } ] }, { "text": "1.曲霉菌球此型多在肺部存在空洞性病变的情况下,真菌在空腔内寄生,形成曲霉球。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "曲霉菌球" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "肺部存在空洞性病变" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "真菌" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "曲霉球" } ] }, { "text": "一般为单个出现,偶尔双肺同时出现。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "双肺" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "双肺同时出现" } ] }, { "text": "典型的X线表现:空洞内有一个新月形气体阴影,由于菌丝不侵袭空洞壁,较小的曲霉球可在空洞内移动,或随体位改变而移动。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "X线" }, { "start_idx": 8, "end_idx": 20, "type": "sym", "entity": "空洞内有一个新月形气体阴影" }, { "start_idx": 33, "end_idx": 55, "type": "sym", "entity": "较小的曲霉球可在空洞内移动,或随体位改变而移动" } ] }, { "text": "这些空洞也可为过敏性或侵袭性曲霉菌病的演变。", "entities": [ { "start_idx": 7, "end_idx": 17, "type": "dis", "entity": "过敏性或侵袭性曲霉菌病" } ] }, { "text": "侵袭性曲霉菌病引起肺炎,肺组织坏死,呈真菌性肺脓肿,脓腔形成,进一步形成球体。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "侵袭性曲霉菌病" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肺炎" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "肺组织坏死" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "真菌性肺脓肿" } ] }, { "text": "此外,过敏性支气管肺曲霉菌病亦可发生脓肿,最后形成曲霉球。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "过敏性支气管肺曲霉菌病" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "脓肿" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "曲霉球" } ] }, { "text": "2.过敏性支气管肺曲霉菌病(ABPA)是曲霉菌引起的过敏性肺疾病。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "过敏性支气管肺曲霉菌病" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "ABPA" }, { "start_idx": 20, "end_idx": 22, "type": "mic", "entity": "曲霉菌" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "过敏性肺疾病" } ] }, { "text": "绝大多数发生于哮喘或有过敏性疾病的患者。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "哮喘" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "过敏性疾病" } ] }, { "text": "ABPA典型的病理变化是中央气道扩张,常有黏液堵塞,远端气道通常正常。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "ABPA" }, { "start_idx": 12, "end_idx": 24, "type": "sym", "entity": "中央气道扩张,常有黏液堵塞" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "远端气道通常正常" } ] }, { "text": "曲霉菌的菌丝可与支气管壁紧密相邻,但界限清楚,不侵入管壁和血管,一些病例可形成肉芽肿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "曲霉菌" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "支气管壁" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "管壁" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "血管" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "肉芽肿" } ] }, { "text": "在临床上,ABPA支气管痉挛是短暂的,后期症状趋于慢性。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "ABPA支气管痉挛" } ] }, { "text": "外周血和痰液嗜酸性粒细胞升高。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "血" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "痰液" }, { "start_idx": 6, "end_idx": 11, "type": "ite", "entity": "嗜酸性粒细胞" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "外周血和痰液嗜酸性粒细胞升高" } ] }, { "text": "痰液涂片和培养可以发现菌丝。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "痰液涂片" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "痰液涂片和培养可以发现菌丝" } ] }, { "text": "血IgE明显升高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "血IgE" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "血IgE明显升高" } ] }, { "text": "对曲霉菌抗原速发型或迟发型皮肤超敏反应。", "entities": [ { "start_idx": 1, "end_idx": 12, "type": "dis", "entity": "曲霉菌抗原速发型或迟发型" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "皮肤" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "皮肤超敏反应" } ] }, { "text": "3.侵袭性肺部曲霉菌病即在肺组织发现曲霉菌菌丝或孢子,为侵袭性肺部曲霉菌病。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "侵袭性肺部曲霉菌病" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肺组织" }, { "start_idx": 18, "end_idx": 20, "type": "mic", "entity": "曲霉菌" }, { "start_idx": 28, "end_idx": 36, "type": "dis", "entity": "侵袭性肺部曲霉菌病" } ] }, { "text": "免疫功能受损越严重,越易发生急性肺部曲霉菌病。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "肺部曲霉菌病" } ] }, { "text": "影像学表现早期可为多发或单发小炎性结节,急性肺部曲霉菌病可为双肺弥漫性团块影、云絮影,也可为斑片影。", "entities": [ { "start_idx": 9, "end_idx": 18, "type": "sym", "entity": "多发或单发小炎性结节" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "急性肺部曲霉菌病" }, { "start_idx": 30, "end_idx": 48, "type": "sym", "entity": "双肺弥漫性团块影、云絮影,也可为斑片影" } ] }, { "text": "慢性肺部曲霉菌病多为上叶实变伴胸膜肥厚,实变区内有空洞(故又称慢性坏死性肺曲霉菌病)。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "慢性肺部曲霉菌病" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "上叶实变伴胸膜肥厚" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "实变区内有空洞" }, { "start_idx": 31, "end_idx": 40, "type": "dis", "entity": "慢性坏死性肺曲霉菌病" } ] }, { "text": "文献还报道慢性肺曲霉菌病的空洞内可见高密度阴影(似曲霉菌球),偶尔在高密度阴影内还可见到许多类似钙化的点状阴影,此征仅见于曲霉菌感染,而在其他真菌感染时不出现。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "慢性肺曲霉菌病" }, { "start_idx": 13, "end_idx": 22, "type": "sym", "entity": "空洞内可见高密度阴影" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "曲霉菌球" }, { "start_idx": 34, "end_idx": 54, "type": "sym", "entity": "高密度阴影内还可见到许多类似钙化的点状阴影" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "曲霉菌感染" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "真菌感染" } ] }, { "text": "“晕轮征”对于侵袭性肺曲霉菌病有一定的诊断意义。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "sym", "entity": "晕轮征" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "侵袭性肺曲霉菌病" } ] }, { "text": "急性侵袭性肺曲霉菌病可恶化为播散性曲霉菌病。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "急性侵袭性肺曲霉菌病" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "播散性曲霉菌病" } ] }, { "text": "慢性肺曲霉菌病进展缓慢,最后波及整个肺或胸腔、纵隔、胸壁等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "慢性肺曲霉菌病" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肺" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "胸腔" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "纵隔" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "胸壁" } ] }, { "text": "也可转化为急性曲霉菌肺炎。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "急性曲霉菌肺炎" } ] }, { "text": "菌丝易侵犯血管,随血流播散到全身各脏器,引起播散性曲霉菌病。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "血流" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "全身各脏器" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "播散性曲霉菌病" } ] }, { "text": "【实验室检查】1.直接镜检痰液、尿等标本,加一滴10%~20%氢氧化钾溶液,镜下见分隔菌丝、分生孢子,有时可见分生孢子梗、顶囊和小梗。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "直接镜检" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "痰液" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "尿" }, { "start_idx": 38, "end_idx": 49, "type": "sym", "entity": "镜下见分隔菌丝、分生孢子" }, { "start_idx": 51, "end_idx": 65, "type": "sym", "entity": "有时可见分生孢子梗、顶囊和小梗" } ] }, { "text": "2.培养标本接种于培养基上,48小时后即有菌丝和分生孢子头出现。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "培养" } ] }, { "text": "血液、心包液、脑脊液无菌,只要1次培养阳性,即有重要的诊断意义。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血液" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "心包液" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "脑脊液无菌" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "1次培养阳性" } ] }, { "text": "肺内咯出物活检组织或手术切除组织,做病理检查发现曲霉菌,有肯定意义。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "肺内咯出物活检组织" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "手术切除组织" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "病理检查发现曲霉菌" } ] }, { "text": "2次以上痰液或支气管-肺泡灌洗液(BALF)培养有曲霉菌生长,除外其他病原体感染,结合抗曲霉菌治疗有效,可作出临床诊断。", "entities": [ { "start_idx": 4, "end_idx": 29, "type": "sym", "entity": "痰液或支气管-肺泡灌洗液(BALF)培养有曲霉菌生长" }, { "start_idx": 43, "end_idx": 48, "type": "pro", "entity": "抗曲霉菌治疗" } ] }, { "text": "3.血清学检查(1)血清半乳糖甘露聚糖(GM)抗原检测:半乳糖甘露聚糖仅存在于曲霉细胞壁中,曲霉发生侵袭性感染时,可从细胞壁释放进入血液,在血清中可检测出,简称GM实验。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "血清学检查" }, { "start_idx": 10, "end_idx": 26, "type": "pro", "entity": "血清半乳糖甘露聚糖(GM)抗原检测" }, { "start_idx": 39, "end_idx": 43, "type": "mic", "entity": "曲霉细胞壁" }, { "start_idx": 46, "end_idx": 47, "type": "mic", "entity": "曲霉" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "血液" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "血清" }, { "start_idx": 80, "end_idx": 83, "type": "pro", "entity": "GM实验" } ] }, { "text": "GM实验阳性提示侵袭性曲霉感染。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "GM实验阳性" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "侵袭性曲霉感染" } ] }, { "text": "半乳糖甘露聚糖最早可在发病前5~8天从血液中检出。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "半乳糖甘露聚糖" } ] }, { "text": "假阳性率较高的人群为新生儿、自身抗体阳性、菌血症患者、使用半合成青霉素及异体骨髓抑制患者。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "菌血症" }, { "start_idx": 29, "end_idx": 34, "type": "dru", "entity": "半合成青霉素" }, { "start_idx": 36, "end_idx": 41, "type": "dru", "entity": "异体骨髓抑制" } ] }, { "text": "慢性肉芽病患儿发生慢性肺曲霉病时,GM检测呈阴性。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "慢性肉芽病" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "慢性肺曲霉病" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "GM检测呈阴性" } ] }, { "text": "(2)血清(1-3)-β-D-葡聚糖抗原检测:(1-3)-β-D-葡聚糖为真菌细胞壁成分,国内采用Fungitec-G法(中华鲎实验),简称G试验。", "entities": [ { "start_idx": 3, "end_idx": 21, "type": "pro", "entity": "血清(1-3)-β-D-葡聚糖抗原检测" }, { "start_idx": 49, "end_idx": 59, "type": "pro", "entity": "Fungitec-G法" }, { "start_idx": 61, "end_idx": 65, "type": "pro", "entity": "中华鲎实验" }, { "start_idx": 70, "end_idx": 72, "type": "pro", "entity": "G试验" } ] }, { "text": "阳性提示侵袭性真菌感染。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "阳性" }, { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "侵袭性真菌感染" } ] }, { "text": "【诊断】1.侵袭性肺曲霉病现行的诊断模式为基于宿主因素、临床特征、微生物学及病理组织学检查三种核心因素的综合诊断,诊断分级为确诊(proven)、临床诊断(probable)、拟诊(possible)(详见概况部分)。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "侵袭性肺曲霉病" } ] }, { "text": "肺组织活检发现曲霉菌丝,可以确诊。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "肺组织活检发现曲霉菌丝" } ] }, { "text": "气管内吸引物或合格痰标本直接镜检发现菌丝,且培养连续2次分离到同种真菌;BALF经直接镜检发现菌丝,真菌培养阳性;血清GM连续2次阳性,可临床诊断为侵袭性肺曲霉病。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "气管内吸引物或合格痰标本直接镜检发现菌丝" }, { "start_idx": 22, "end_idx": 34, "type": "sym", "entity": "培养连续2次分离到同种真菌" }, { "start_idx": 36, "end_idx": 55, "type": "sym", "entity": "BALF经直接镜检发现菌丝,真菌培养阳性" }, { "start_idx": 57, "end_idx": 66, "type": "sym", "entity": "血清GM连续2次阳性" }, { "start_idx": 74, "end_idx": 80, "type": "dis", "entity": "侵袭性肺曲霉病" } ] }, { "text": "2.ABPA目前依据Rosenberg-Patterson诊断标准,主要标准:①哮喘;②肺部影像学检查肺部浸润影;③曲霉菌抗原皮内试验快速反应阳性;④周围血嗜酸性粒细胞增多;⑤血清沉淀抗体IgG阳性;⑥血清总IgE升高(>1000U/L);⑦中央型支扩;⑧血清曲霉菌特异性IgG和IgE抗体两倍以上增高。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "ABPA" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "哮喘" }, { "start_idx": 44, "end_idx": 50, "type": "pro", "entity": "肺部影像学检查" }, { "start_idx": 51, "end_idx": 55, "type": "sym", "entity": "肺部浸润影" }, { "start_idx": 58, "end_idx": 72, "type": "sym", "entity": "曲霉菌抗原皮内试验快速反应阳性" }, { "start_idx": 75, "end_idx": 85, "type": "sym", "entity": "周围血嗜酸性粒细胞增多" }, { "start_idx": 88, "end_idx": 98, "type": "sym", "entity": "血清沉淀抗体IgG阳性" }, { "start_idx": 101, "end_idx": 118, "type": "sym", "entity": "血清总IgE升高(>1000U/L)" }, { "start_idx": 121, "end_idx": 125, "type": "sym", "entity": "中央型支扩" }, { "start_idx": 128, "end_idx": 150, "type": "sym", "entity": "血清曲霉菌特异性IgG和IgE抗体两倍以上增高" } ] }, { "text": "次要条件:①痰找到曲霉菌;②咳棕黑色黏液栓;③曲霉菌抗原迟发型皮试阳性。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "痰找到曲霉菌" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "咳棕黑色黏液栓" }, { "start_idx": 23, "end_idx": 34, "type": "sym", "entity": "曲霉菌抗原迟发型皮试阳性" } ] }, { "text": "根据患者是否出现中央型支气管扩张可将ABPA分为2个亚型,即ABPA-S(血清阳性型)和ABPA-CB(中央支气管扩张型)。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "sym", "entity": "出现中央型支气管扩张" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "ABPA" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "ABPA-S" }, { "start_idx": 44, "end_idx": 50, "type": "dis", "entity": "ABPA-CB" } ] }, { "text": "【治疗】1.侵袭性曲霉菌两性霉素B是传统治疗的首选药物,静脉应用,同时雾化给药。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "mic", "entity": "侵袭性曲霉菌" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "静脉应用" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "雾化给药" } ] }, { "text": "目前认为病情较重的可首选伏立康唑。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "伏立康唑" } ] }, { "text": "伊曲康唑用于轻-中患者。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "伊曲康唑" } ] }, { "text": "卡泊芬净用于其他药物治疗无效或不能耐受其他药物者。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "卡泊芬净" } ] }, { "text": "氟胞嘧啶对少数曲霉有一定的抗菌活性,常和两性霉素B或咪唑类联合应用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "氟胞嘧啶" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "咪唑类" } ] }, { "text": "氟康唑对曲霉菌无效。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "氟康唑" }, { "start_idx": 4, "end_idx": 6, "type": "mic", "entity": "曲霉菌" } ] }, { "text": "药物治疗后仍迁延不愈、合并大咯血、病变局限能耐受手术时可考虑外科手术切除病变。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "药物治疗后仍迁延不愈" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "合并大咯血" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "病变局限" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "外科手术" } ] }, { "text": "2.ABPA治疗皮质类固醇类激素目前依然是治疗的金标准,常规剂量,依据临床反应调整,可使大部分患者症状减轻、肺部阴影消退、延缓肺功能减退和肺纤维化等毁损肺的发生。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "ABPA" }, { "start_idx": 8, "end_idx": 15, "type": "dru", "entity": "皮质类固醇类激素" }, { "start_idx": 54, "end_idx": 59, "type": "sym", "entity": "肺部阴影消退" }, { "start_idx": 61, "end_idx": 67, "type": "sym", "entity": "延缓肺功能减退" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "肺纤维化" }, { "start_idx": 76, "end_idx": 76, "type": "bod", "entity": "肺" } ] }, { "text": "抗真菌治疗激素依赖性ABPA患者,可使部分患者有效。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "抗真菌治疗激素" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "依赖性ABPA" } ] }, { "text": "三、肾小管功能检查肾小管功能较为复杂,包括排泌、重吸收、浓缩稀释、酸化尿液以及离子转移等多方面。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "肾小管功能检查" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肾小管" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "尿液" } ] }, { "text": "(一)酚红排泄试验静脉注射0.6%酚红1ml后,94%由肾小管分泌排出,仅6%经肾小球滤过,故根据其排出的时间及量可判断近端肾小管细胞的分泌功能及肾血流量。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "酚红排泄试验" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "静脉注射" }, { "start_idx": 17, "end_idx": 18, "type": "dru", "entity": "酚红" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "肾小管" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "肾小球" }, { "start_idx": 62, "end_idx": 66, "type": "bod", "entity": "肾小管细胞" }, { "start_idx": 73, "end_idx": 73, "type": "bod", "entity": "肾" } ] }, { "text": "方法:注射前20分钟饮水200ml以上并在注射酚红同时排尿弃去。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "注射" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "注射酚红" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "尿" } ] }, { "text": "注射后15、30、60及120分钟各留尿一次。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "注射" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "尿" } ] }, { "text": "15分钟值在12%以下时,说明肾小管功能有明显损害,见于慢性肾小球肾炎及肾血管硬化者。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肾小管" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "慢性肾小球肾炎" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "肾血管硬化" } ] }, { "text": "(二)肾小管回吸收功能测定1.肾小管葡萄糖最大吸收量(TmG)测定TmG(tubularmaximalglucosereabsorptioncapacity)是测定近端肾小管最大重吸收能力的一种功能试验。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肾小管" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "TmG" }, { "start_idx": 39, "end_idx": 41, "type": "pro", "entity": "TmG" }, { "start_idx": 49, "end_idx": 89, "type": "ite", "entity": "tubularmaximalglucosereabsorptioncapacity" }, { "start_idx": 96, "end_idx": 98, "type": "bod", "entity": "肾小管" } ] }, { "text": "采用单位时间内肾小球滤过的葡萄糖量减去单位时间内尿中出现的葡萄糖量,即可计算出TmG值。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肾小球" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "葡萄" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "尿" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "TmG" } ] }, { "text": "TmG=PG×Cin-UG×VPG、UG分别为血、尿中葡萄糖浓度(mg/dl),V为尿量(ml),Cin为菊粉清除率(ml/min),3~15岁小儿正常参考值为(254±115)mg/(min•1.73m2)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "TmG" }, { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "PG" }, { "start_idx": 19, "end_idx": 24, "type": "ite", "entity": "Cin-UG" }, { "start_idx": 33, "end_idx": 34, "type": "ite", "entity": "PG" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "UG" }, { "start_idx": 53, "end_idx": 61, "type": "ite", "entity": "血、尿中葡萄糖浓度" }, { "start_idx": 72, "end_idx": 73, "type": "ite", "entity": "尿量" }, { "start_idx": 83, "end_idx": 87, "type": "ite", "entity": "菊粉清除率" } ] }, { "text": "在近端肾小管疾病时TmG降低。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "近端肾小管疾病" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "TmG" } ] }, { "text": "2.尿中低分子量蛋白的测定溶菌酶(lysozyme)、视黄醇结合蛋白(retinol-bindingprotein,RBP)以及尿中κ、λ轻链都属低分子量蛋白,绝大多数在肾近端小管重吸收,如果尿中浓度升高,往往说明肾小管回吸收功能减低。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "尿中低分子量蛋白的测定" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "溶菌酶" }, { "start_idx": 17, "end_idx": 24, "type": "bod", "entity": "lysozyme" }, { "start_idx": 27, "end_idx": 33, "type": "bod", "entity": "视黄醇结合蛋白" }, { "start_idx": 35, "end_idx": 56, "type": "bod", "entity": "retinol-bindingprotein" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "RBP" }, { "start_idx": 64, "end_idx": 64, "type": "bod", "entity": "尿" }, { "start_idx": 66, "end_idx": 70, "type": "bod", "entity": "κ、λ轻链" }, { "start_idx": 85, "end_idx": 89, "type": "bod", "entity": "肾近端小管" }, { "start_idx": 96, "end_idx": 96, "type": "bod", "entity": "尿" }, { "start_idx": 107, "end_idx": 109, "type": "bod", "entity": "肾小管" } ] }, { "text": "(三)尿液浓缩与稀释功能检查1.改良莫氏试验(Mosenthaltest)反映远端肾小管功能。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "pro", "entity": "尿液浓缩与稀释功能检查" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "改良莫氏试验" }, { "start_idx": 23, "end_idx": 35, "type": "pro", "entity": "Mosenthaltest" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "远端肾小管" } ] }, { "text": "试验前1天晚8时后禁水,试验当天正常饮食,餐间禁水,晨8时排尿弃去,以后每2小时留尿一次至晚8时共6次,自晚8时至次日晨留12小时尿,白天12小时尿量应为全日总量的1/2~3/4,比重应有一次>1.020,最高与最低尿比重之差应>0.009。", "entities": [ { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "尿" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "尿" }, { "start_idx": 65, "end_idx": 65, "type": "bod", "entity": "尿" }, { "start_idx": 73, "end_idx": 74, "type": "ite", "entity": "尿量" }, { "start_idx": 108, "end_idx": 110, "type": "ite", "entity": "尿比重" } ] }, { "text": "浓缩功能障碍见于急、慢性肾功能不全、肾间质损伤及肾小管疾病等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "浓缩功能障碍" }, { "start_idx": 8, "end_idx": 16, "type": "dis", "entity": "急、慢性肾功能不全" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "肾间质损伤" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "肾小管疾病" } ] }, { "text": "2.尿渗透压测定检测禁水12小时后的浓缩尿的渗透压,正常应达800mOsm/L以上,较莫氏试验简单,方便。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "尿渗透压测定" }, { "start_idx": 18, "end_idx": 24, "type": "ite", "entity": "浓缩尿的渗透压" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "莫氏试验" } ] }, { "text": "3.自由水清除率(clearanceoffreewater,CH2O)指单位时间内从血浆中清除到尿中的不含溶质的水量,计算方法:CH2O(ml/min)=V-Uosm×V/Posm,式中V为尿量,Uosm、Posm分别为尿和血的渗透压。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "ite", "entity": "自由水清除率" }, { "start_idx": 9, "end_idx": 28, "type": "ite", "entity": "clearanceoffreewater" }, { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "CH2O" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "血浆" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "尿" }, { "start_idx": 70, "end_idx": 73, "type": "ite", "entity": "CH2O" }, { "start_idx": 83, "end_idx": 88, "type": "ite", "entity": "V-Uosm" }, { "start_idx": 90, "end_idx": 90, "type": "ite", "entity": "V" }, { "start_idx": 92, "end_idx": 95, "type": "ite", "entity": "Posm" }, { "start_idx": 99, "end_idx": 99, "type": "ite", "entity": "V" }, { "start_idx": 101, "end_idx": 102, "type": "ite", "entity": "尿量" }, { "start_idx": 109, "end_idx": 112, "type": "ite", "entity": "Posm" }, { "start_idx": 116, "end_idx": 122, "type": "ite", "entity": "尿和血的渗透压" } ] }, { "text": "当Uosm=Posm时,CH2O=0,说明肾脏不起浓缩作用。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "ite", "entity": "Uosm" }, { "start_idx": 6, "end_idx": 9, "type": "ite", "entity": "Posm" }, { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "CH2O" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "肾脏不起浓缩作用" } ] }, { "text": "CH2O正值说明机体需排出较多水分以维持正常的血浆渗透压。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "CH2O" }, { "start_idx": 29, "end_idx": 33, "type": "ite", "entity": "血浆渗透压" } ] }, { "text": "CH2O负值越大,表示浓缩功能越强。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "CH2O" } ] }, { "text": "浓缩试验时,CH2O可达-0.4~10.7ml/min,稀释试验时CH2O为1~9ml/min。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "浓缩试验" }, { "start_idx": 6, "end_idx": 9, "type": "ite", "entity": "CH2O" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "稀释试验" }, { "start_idx": 33, "end_idx": 36, "type": "ite", "entity": "CH2O" } ] }, { "text": "(四)尿酸化功能检查近端肾小管和远端肾小管都有分泌H+、保的功能,这种酸化尿液的功能受损将导致肾小管酸中毒的发生。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "尿酸化功能检查" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "近端肾小管" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "远端肾小管" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "H+" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "尿液" }, { "start_idx": 53, "end_idx": 58, "type": "dis", "entity": "肾小管酸中毒" } ] }, { "text": "尿液酸化功能的检查包括尿pH、尿氨、可滴定酸的测定以及酸负荷试验、碱负荷试验和呋塞米试验等,方法详见本篇第四章第一节“肾小管性酸中毒”。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "尿液酸化功能的检查" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "尿pH" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "尿氨" }, { "start_idx": 18, "end_idx": 21, "type": "ite", "entity": "可滴定酸" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "酸负荷" }, { "start_idx": 33, "end_idx": 37, "type": "pro", "entity": "碱负荷试验" }, { "start_idx": 39, "end_idx": 43, "type": "pro", "entity": "呋塞米试验" }, { "start_idx": 59, "end_idx": 65, "type": "dis", "entity": "肾小管性酸中毒" } ] }, { "text": "(五)肾脏内分泌功能检查肾脏与内分泌关系主要包括:①产生内分泌激素的能力:如促红细胞生成素(Epo)、肾素、血管紧张素、1,25(OH)2D3以及前列腺素;②作为靶器官对外分泌激素的反应能力:如ADH和PTH等可作用于肾脏;③降解部分内分泌激素如胰岛素等。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "肾脏内分泌功能检查" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肾脏" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "内分泌激素" }, { "start_idx": 38, "end_idx": 44, "type": "bod", "entity": "促红细胞生成素" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "Epo" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "肾素" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "血管紧张素" }, { "start_idx": 85, "end_idx": 88, "type": "bod", "entity": "前列腺素" }, { "start_idx": 93, "end_idx": 95, "type": "bod", "entity": "靶器官" }, { "start_idx": 97, "end_idx": 101, "type": "bod", "entity": "外分泌激素" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "ADH" }, { "start_idx": 113, "end_idx": 115, "type": "bod", "entity": "PTH" }, { "start_idx": 121, "end_idx": 122, "type": "bod", "entity": "肾脏" }, { "start_idx": 129, "end_idx": 133, "type": "bod", "entity": "内分泌激素" }, { "start_idx": 135, "end_idx": 137, "type": "bod", "entity": "胰岛素" } ] }, { "text": "反映肾脏内分泌功能检测主要是血中Epo浓度、1,25(OH)2血浆肾素活性测定等方面。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肾脏" }, { "start_idx": 14, "end_idx": 14, "type": "ite", "entity": "血" }, { "start_idx": 16, "end_idx": 20, "type": "ite", "entity": "Epo浓度" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "血浆肾素" } ] }, { "text": "(六)尿酶检查肾脏受损时血液及肾组织中的某些酶可从尿中排出,现已证实可从尿液中检测出30~50种酶。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "尿酶检查" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "肾脏受损" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "血液" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肾组织" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "酶" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "尿" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "尿液" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "酶" } ] }, { "text": "临床上已有十几种尿酶测定应用于肾脏病的诊断和判断预后。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "ite", "entity": "尿酶测定" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肾脏病" } ] }, { "text": "但因大多数缺乏特异性且影响因素较多,故常需检查2种以上尿酶并结合临床资料综合判断。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "尿酶" } ] }, { "text": "尿酶有以下几类:①水解酶类:如N-乙酰-β-D氨基葡萄糖苷酶(N-acetyl-D-glucosaminidase,NAG)临床上应用较多,为溶酶体酶,不能为肾小球滤过,广泛存在于肾小管和泌尿道上皮细胞中。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "尿酶" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "水解酶类" }, { "start_idx": 15, "end_idx": 29, "type": "bod", "entity": "N-乙酰-β-D氨基葡萄糖苷酶" }, { "start_idx": 31, "end_idx": 56, "type": "bod", "entity": "N-acetyl-D-glucosaminidase" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "NAG" }, { "start_idx": 71, "end_idx": 74, "type": "bod", "entity": "溶酶体酶" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "肾小球" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "肾小管" }, { "start_idx": 94, "end_idx": 100, "type": "bod", "entity": "泌尿道上皮细胞" } ] }, { "text": "在急性肾炎、肾衰竭、肾病、狼疮肾和紫癜肾的急性期均较缓解期明显升高。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "急性肾炎" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肾病" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "狼疮肾" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "紫癜肾" } ] }, { "text": "NAG在尿中活性升高是肾脏损害的标志。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "NAG" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "尿" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "肾脏损害" } ] }, { "text": "NAG还可作为一灵敏的指标用于监测氨基甙类抗生素的中毒及肾移植后急性排异反应。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "NAG" }, { "start_idx": 28, "end_idx": 30, "type": "pro", "entity": "肾移植" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "急性排异" } ] }, { "text": "②转换酶类:如γ-谷氨酰转换酶(r-glutamyltransferase,γ-GT)为肾小管细胞刷状缘酶,增高可见于急性肾炎及肾病急性期,肾病合并泌尿系感染者增高尤为明显。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "转换酶类" }, { "start_idx": 7, "end_idx": 14, "type": "bod", "entity": "γ-谷氨酰转换酶" }, { "start_idx": 16, "end_idx": 36, "type": "bod", "entity": "r-glutamyltransferase" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "γ-GT" }, { "start_idx": 44, "end_idx": 52, "type": "bod", "entity": "肾小管细胞刷状缘酶" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "急性肾炎" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "肾病" }, { "start_idx": 70, "end_idx": 79, "type": "dis", "entity": "肾病合并泌尿系感染者" } ] }, { "text": "肾小球性血尿、肾小管疾病、肾结石及迁延性肾炎时也可见增高。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "肾小球性血尿" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "肾小管疾病" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肾结石" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "迁延性肾炎" } ] }, { "text": "③肽酶类:如亮氨酸氨基肽酶(leucineaminopeptidase,LAP)也是近端肾小管细胞刷状缘酶,对小管细胞的损伤较敏感。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "肽酶类" }, { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "亮氨酸氨基肽酶" }, { "start_idx": 14, "end_idx": 34, "type": "bod", "entity": "leucineaminopeptidase" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "LAP" }, { "start_idx": 42, "end_idx": 52, "type": "bod", "entity": "近端肾小管细胞刷状缘酶" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "小管细胞" } ] }, { "text": "其增高的意义与r-GT相似,临床上常两者同时检测。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "r-GT" } ] }, { "text": "④氧化还原酶类:如乳酸脱氢酶(lacticdehydrogenase,LDH)及其异构酶,可在急性肾炎及肾病时增高。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "氧化还原酶类" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "乳酸脱氢酶" }, { "start_idx": 15, "end_idx": 33, "type": "bod", "entity": "lacticdehydrogenase" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "LDH" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "异构酶" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "急性肾炎" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "肾病" } ] }, { "text": "LDH5增高提示为上泌尿系感染,还可用于肾移植后排异的早期诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "LDH5" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "上泌尿系感染" }, { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "肾移植" } ] }, { "text": "其他蛋白酶类还有如尿激酶和胰蛋白酶,裂解酶类有透明质酸酶等,但临床应用较少。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "蛋白酶类" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "尿激酶" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "胰蛋白酶" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "透明质酸酶" } ] }, { "text": "第十一节病态窦房结综合征病态窦房结综合征(sicksinussyndrome)由窦房结或心房传导组织的异常所引起。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "病态窦房结综合征" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "病态窦房结综合征" }, { "start_idx": 21, "end_idx": 37, "type": "dis", "entity": "sicksinussyndrome" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "窦房结" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "心房传导组织" } ] }, { "text": "可发生在无先天性心脏病的患儿,也有报道在同胞中出现,但最常见于先天性心脏病外科纠治术后,特别是大动脉错位Mustard或Senning手术后。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 31, "end_idx": 41, "type": "sym", "entity": "先天性心脏病外科纠治术" }, { "start_idx": 47, "end_idx": 68, "type": "sym", "entity": "大动脉错位Mustard或Senning手术" } ] }, { "text": "其临床表现主要取决于心率。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "心率" } ] }, { "text": "三、腹膜透析的基本方式(一)间歇性腹膜透析间歇性腹膜透析(IPD)透析方式运用于急性肾衰PD或慢性PD治疗的最初阶段,一般推荐透析交换8~10次/d(8~10个透析周期),每次透析液30~50m1/kg,最多不能超过2000ml/(m2•次),由于IPD透析方式腹透液置留腹腔内约30~40min,对中大分子物质清除效果差,因此不作为慢性腹透的常规。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 14, "end_idx": 20, "type": "pro", "entity": "间歇性腹膜透析" }, { "start_idx": 21, "end_idx": 27, "type": "pro", "entity": "间歇性腹膜透析" }, { "start_idx": 29, "end_idx": 31, "type": "pro", "entity": "IPD" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "透析" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "急性肾衰PD" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "慢性PD" }, { "start_idx": 63, "end_idx": 64, "type": "pro", "entity": "透析" }, { "start_idx": 88, "end_idx": 90, "type": "dru", "entity": "透析液" }, { "start_idx": 130, "end_idx": 134, "type": "pro", "entity": "IPD透析" }, { "start_idx": 137, "end_idx": 139, "type": "dru", "entity": "腹透液" }, { "start_idx": 142, "end_idx": 143, "type": "bod", "entity": "腹腔" }, { "start_idx": 173, "end_idx": 176, "type": "pro", "entity": "慢性腹透" } ] }, { "text": "(二)连续性非卧床腹膜透析连续性非卧床腹膜透析(continuousambulatorperitonealdialysis,CAPD)是慢性腹膜透析方式中使用最广的一种,其简单易行,费用低,不需特殊透析设备,具有持续性腹膜透析状态,缓慢持续的超滤脱水,生理状态稳定,血压控制满意。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "连续性非卧床腹膜透析" }, { "start_idx": 24, "end_idx": 60, "type": "pro", "entity": "continuousambulatorperitonealdialysis" }, { "start_idx": 68, "end_idx": 73, "type": "pro", "entity": "慢性腹膜透析" }, { "start_idx": 109, "end_idx": 112, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 123, "end_idx": 124, "type": "sym", "entity": "脱水" }, { "start_idx": 133, "end_idx": 134, "type": "ite", "entity": "血压" } ] }, { "text": "儿童标准的CAPD,每日4次,每次透析量为50ml/kg或2000ml/m2,白天交换3次,间隔4~6小时交换一次,夜间交换一次,透析液存留在腹腔中过夜8~10小时。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "CAPD" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "透析" }, { "start_idx": 71, "end_idx": 73, "type": "dru", "entity": "透析液" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "腹腔" } ] }, { "text": "患儿的CAPD次数并非一成不变,可根据病情及残余肾功能来增加或减少交换次数,当患儿常规透析仍出现高血钾或高分解状态或透析不充分时,可适当增加交换次数。", "entities": [ { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "肾" }, { "start_idx": 48, "end_idx": 62, "type": "sym", "entity": "高血钾或高分解状态或透析不充分" } ] }, { "text": "对于儿童患儿腹膜透析超滤脱水,可根据患儿尿量、水肿以及心功能状态酌情选用1.5%、2.5%及4.25%的葡萄糖透析液,一般情况白天采用1.5%透析液,夜间因腹透液置留腹腔时间过长,导致透析液葡萄糖经腹膜再吸收致使透析液渗透压下降而影响超滤,故应考虑使用2.5%或4.25%葡萄糖透析液。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "腹膜透析超滤脱水" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "尿量" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "水肿" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "心" }, { "start_idx": 52, "end_idx": 57, "type": "dru", "entity": "葡萄糖透析液" }, { "start_idx": 71, "end_idx": 73, "type": "dru", "entity": "透析液" }, { "start_idx": 78, "end_idx": 80, "type": "dru", "entity": "腹透液" }, { "start_idx": 83, "end_idx": 84, "type": "bod", "entity": "腹腔" }, { "start_idx": 92, "end_idx": 97, "type": "dru", "entity": "透析液葡萄糖" }, { "start_idx": 99, "end_idx": 100, "type": "bod", "entity": "腹膜" }, { "start_idx": 106, "end_idx": 108, "type": "dru", "entity": "透析液" }, { "start_idx": 136, "end_idx": 141, "type": "dru", "entity": "葡萄糖透析液" } ] }, { "text": "CAPD透析方式对于儿童患者需要其他人的帮助,同时影响学龄儿童正常上学,由于CAPD透析方式液体交换次数多,腹膜炎的发生率相对较高。", "entities": [ { "start_idx": 38, "end_idx": 43, "type": "pro", "entity": "CAPD透析" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "(三)持续循环腹膜透析持续循环腹膜透析(CCPD)是近年来随着自动腹膜透析机(APD)而发展起来的一种腹透方法,实际上它与CAPD相似,将CAPD白天3次透析液交换改在夜间利用腹膜透析机自动进行,晨起腹腔放入腹透液,持续到晚上重新循环开始。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "持续循环腹膜透析" }, { "start_idx": 11, "end_idx": 18, "type": "pro", "entity": "持续循环腹膜透析" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "CCPD" }, { "start_idx": 31, "end_idx": 37, "type": "equ", "entity": "自动腹膜透析机" }, { "start_idx": 39, "end_idx": 41, "type": "equ", "entity": "APD" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "腹透" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "CAPD" }, { "start_idx": 69, "end_idx": 72, "type": "pro", "entity": "CAPD" }, { "start_idx": 77, "end_idx": 79, "type": "dru", "entity": "透析液" }, { "start_idx": 88, "end_idx": 92, "type": "equ", "entity": "腹膜透析机" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "腹腔" }, { "start_idx": 104, "end_idx": 106, "type": "dru", "entity": "腹透液" } ] }, { "text": "对于儿童患者选用CCPD方式其优点是:①由于CCPD是由腹膜透析机换液,自动加温,控制液体出入量,减少了人工换液的次数,从而有效降低腹膜炎的发生率。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "CCPD" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "CCPD" }, { "start_idx": 28, "end_idx": 32, "type": "equ", "entity": "腹膜透析机" }, { "start_idx": 52, "end_idx": 55, "type": "pro", "entity": "人工换液" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "复旦大学附属儿科医院近年引进自动腹膜透析技术,患儿采用CCPD长达一年余未有腹膜炎发生。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dep", "entity": "儿科" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "自动腹膜透析" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "CCPD" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "②通过透析机可调整灌入量,适用于体表面积较小的儿童患者。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "透析机" } ] }, { "text": "③CCPD每天仅操作一次,最大限度地减少患儿父母对透析的参与,而患儿白天不需透析,可充分地利用白天时间学习。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "CCPD" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "透析" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "透析" } ] }, { "text": "④透析在夜间进行,白天可提高营养和热量的摄入。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "透析" } ] }, { "text": "⑤儿童腹膜表面积与单位体重之比较成年人大,腹膜对葡萄糖再吸收快。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "腹膜" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "腹膜" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "葡萄糖" } ] }, { "text": "因此对于儿童腹透患者选择透析方式CCPD明显优于CAPD。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "腹透" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "透析" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "CCPD" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "CAPD" } ] }, { "text": "(四)夜间间歇性腹膜透析夜间间歇性腹膜透析(nocturnalintermittentperitonealdialysis,NIPD)是在夜间交接,不影明白天的上课和正常的学习以及其他活动。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "夜间间歇性腹膜透析" }, { "start_idx": 12, "end_idx": 20, "type": "pro", "entity": "夜间间歇性腹膜透析" }, { "start_idx": 22, "end_idx": 60, "type": "pro", "entity": "nocturnalintermittentperitonealdialysis" }, { "start_idx": 62, "end_idx": 65, "type": "pro", "entity": "NIPD" } ] }, { "text": "第五节心房扑动心房扑动(简称房扑)系由于激动在心房内快速环形运动所产生的一种主动性快速而规则的心律失常。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "心房扑动" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "心房扑动" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "房扑" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "心律失常" } ] }, { "text": "较少见,占心律失常的2%左右。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "心律失常" } ] }, { "text": "室律规则(房室传导比例固定或完全房室传导阻滞)或不规则(房室传导不固定)。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "完全房室传导阻滞" } ] }, { "text": "新生儿房扑通常心脏正常。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "房扑" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心脏" } ] }, { "text": "房扑不控制易发生心力衰竭。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "房扑" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "心力衰竭" } ] }, { "text": "直流电复律可使房扑即刻转为窦性,在许多场合下是首选的方法。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "直流电复律" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "房扑" } ] }, { "text": "先天性心脏病患儿发生慢性房扑时,血栓栓塞与中风的机会增加,因此在电复律前应用抗凝剂。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "慢性房扑" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "血栓栓塞" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "中风" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "电复律" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "抗凝剂" } ] }, { "text": "洋地黄通过延长房室结传导时间来减慢心室率。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "洋地黄" } ] }, { "text": "洋地黄化后通常需给予Ⅰ类抗心律失常药如奎尼丁、普鲁卡因胺来维持疗效。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "洋地黄" }, { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "心律" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "奎尼丁" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "普鲁卡因胺" } ] }, { "text": "第五节乙型肝炎病毒相关肾炎乙型肝炎病毒相关肾炎(hepatitisBvirusassociatedglomerulonephritis,HBV-GN)是指继发于乙型肝炎病毒感染的肾小球肾炎。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "乙型肝炎病毒相关肾炎" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "乙型肝炎病毒相关肾炎" }, { "start_idx": 24, "end_idx": 66, "type": "dis", "entity": "hepatitisBvirusassociatedglomerulonephritis" }, { "start_idx": 68, "end_idx": 73, "type": "dis", "entity": "HBV-GN" }, { "start_idx": 80, "end_idx": 87, "type": "dis", "entity": "乙型肝炎病毒感染" }, { "start_idx": 89, "end_idx": 93, "type": "dis", "entity": "肾小球肾炎" } ] }, { "text": "【病因和发病机制】HBV是直径为42~45nm的球形颗粒(Dane颗粒),为DNA病毒,由双层外壳及内核组成,内含双股DNA及DNA多聚酶,其中一条负链为长链,约3.2kb,另一条正链是短链,约2.8kb,长链DNA上有4个阅读框架,分别编码HBsAg、HBcAg、HBeAg、DNA多聚酶和X蛋白。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "HBV" }, { "start_idx": 38, "end_idx": 42, "type": "mic", "entity": "DNA病毒" } ] }, { "text": "乙型肝炎病毒相关性肾炎中沉积于肾小球毛细血管壁的主要是HBsAg和HBeAg。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "dis", "entity": "乙型肝炎病毒相关性肾炎" }, { "start_idx": 15, "end_idx": 22, "type": "bod", "entity": "肾小球毛细血管壁" } ] }, { "text": "Ozawa和Hattor已分别从HBV-GN病人肾组织中洗脱并找到抗HBsAg抗体和抗HBeAg抗体;免疫电镜显示上述HBV抗原与免疫球蛋白是沉积在肾小球同一位点上的,这些结果均支持HBV-GN是由HBV抗原成分引起的一种免疫复合物性肾炎。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "HBV-GN" }, { "start_idx": 33, "end_idx": 40, "type": "bod", "entity": "抗HBsAg抗体" }, { "start_idx": 42, "end_idx": 49, "type": "bod", "entity": "抗HBeAg抗体" }, { "start_idx": 51, "end_idx": 54, "type": "equ", "entity": "免疫电镜" }, { "start_idx": 59, "end_idx": 61, "type": "mic", "entity": "HBV" }, { "start_idx": 65, "end_idx": 69, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "肾小球" }, { "start_idx": 91, "end_idx": 96, "type": "dis", "entity": "HBV-GN" }, { "start_idx": 111, "end_idx": 118, "type": "dis", "entity": "免疫复合物性肾炎" } ] }, { "text": "目前有人认为其肾小球基底膜上皮下免疫复合物为原位形成。", "entities": [ { "start_idx": 7, "end_idx": 20, "type": "bod", "entity": "肾小球基底膜上皮下免疫复合物" } ] }, { "text": "动物实验提示能穿过肾小球基底膜定位于上皮下的抗原多肽分子量一般小于300~500kD。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "肾小球基底膜" } ] }, { "text": "HBeAg分子量较小,即使结合上IgG也不超过300kD,且带正电荷(PI4.3~4.8),符合引起膜性肾病的条件。", "entities": [ { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "膜性肾病" } ] }, { "text": "HBsAg为3.7MD以上,PI4.0左右,HbcAg8MD以上,PI3.7~4.0,不仅分子量过大,且带阴电荷,因此不太可能穿透基底膜在上皮下形成原位复合物,而有可能沉积在系膜区而致病。", "entities": [ { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "基底膜" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "上皮" }, { "start_idx": 74, "end_idx": 78, "type": "bod", "entity": "原位复合物" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "系膜" } ] }, { "text": "尽管如此,临床上仍见大多数HBV-MN患儿肾小球上皮下有HBsAg沉积,因此,有人认为此时沉积在上皮下的HBsAg并非完整的分子,而是代谢后产生的含抗原决定簇的多肽亚单位,其分子量小,也能穿过基底膜并原位植入,最终导致膜性肾病的发生。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "HBV-MN" }, { "start_idx": 21, "end_idx": 34, "type": "sym", "entity": "肾小球上皮下有HBsAg沉积" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "上皮" }, { "start_idx": 96, "end_idx": 98, "type": "bod", "entity": "基底膜" }, { "start_idx": 109, "end_idx": 112, "type": "dis", "entity": "膜性肾病" } ] }, { "text": "此外,还有人认为HBV感染后诱发自身抗体而导致HBV-GN。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "HBV感染" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "抗体" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "HBV-GN" } ] }, { "text": "由于HBV可直接感染肾组织,因此,HBV直接感染肾脏致病也存在可能。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "HBV" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "感染" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肾组织" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "HBV" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "感染肾脏" } ] }, { "text": "【病理】亚洲小儿肾脏病研究会(Asianstudyofrenaldiseaseinchildren,ASRSC)报告儿童HBV-GN的66.1%为膜性肾病,16.1%为轻微病变,8.1%为膜增殖性肾炎。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "肾脏病" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "HBV-GN" }, { "start_idx": 73, "end_idx": 76, "type": "dis", "entity": "膜性肾病" }, { "start_idx": 94, "end_idx": 99, "type": "dis", "entity": "膜增殖性肾炎" } ] }, { "text": "其组织学改变与典型的膜性肾病有所区别:①往往伴有轻中度的系膜细胞增生且增生的系膜有插入,但多限于旁系膜区,很少伸及远端毛细血管内皮下;②基底膜及系膜区沉积的免疫球蛋白更多,使得免疫荧光镜下呈现粗颗粒甚至团块状,而非原发性膜性肾病的细颗粒样外观免疫荧光或酶标检查可发现HBeAg和/或HBsAg在肾小球内沉积,这也是诊断HBV-GN的必备条件。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "膜性肾病" }, { "start_idx": 24, "end_idx": 42, "type": "sym", "entity": "轻中度的系膜细胞增生且增生的系膜有插入" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "旁系膜" }, { "start_idx": 57, "end_idx": 64, "type": "bod", "entity": "远端毛细血管内皮" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "基底膜" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "系膜" }, { "start_idx": 78, "end_idx": 82, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 88, "end_idx": 92, "type": "equ", "entity": "免疫荧光镜" }, { "start_idx": 107, "end_idx": 113, "type": "dis", "entity": "原发性膜性肾病" }, { "start_idx": 88, "end_idx": 120, "type": "sym", "entity": "免疫荧光镜下呈现粗颗粒甚至团块状,而非原发性膜性肾病的细颗粒样外观" }, { "start_idx": 121, "end_idx": 129, "type": "pro", "entity": "免疫荧光或酶标检查" }, { "start_idx": 133, "end_idx": 152, "type": "sym", "entity": "HBeAg和/或HBsAg在肾小球内沉积" }, { "start_idx": 159, "end_idx": 164, "type": "dis", "entity": "HBV-GN" } ] }, { "text": "多隐匿起病,往往偶然查尿时才发现异常。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "查尿" } ] }, { "text": "2.水肿多不明显,且无明显尿少,但也有少数患儿呈明显凹陷性水肿并伴有腹水。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "水肿" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "尿少" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "凹陷性水肿" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "伴有腹水" } ] }, { "text": "3.血尿几乎均有镜下血尿,并持续存在,往往蛋白尿阴转后镜下血尿仍可持续一段时间。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "血尿" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "血尿" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "镜下血尿" } ] }, { "text": "部分病人在此基础上出现发作性肉眼血尿。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "发作性肉眼血尿" } ] }, { "text": "4.蛋白尿均有不同程度的蛋白尿,蛋白尿表现出较大的波动性,时轻时重,ASRSC报道约61.3%表现为肾病综合征,但对肾上腺皮质激素治疗一般无反应。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 50, "end_idx": 54, "type": "sym", "entity": "肾病综合征" }, { "start_idx": 58, "end_idx": 66, "type": "pro", "entity": "肾上腺皮质激素治疗" } ] }, { "text": "5.高血压多不明显,主要见于病变为膜增生性肾炎者。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "高血压" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "膜增生性肾炎" } ] }, { "text": "6.肾功能不全少见。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "肾功能不全" } ] }, { "text": "7.肝脏症状多不明显。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "肝脏症状" } ] }, { "text": "约一半患儿有肝大或肝功异常,表现为转氨酶升高,但黄疸者少见。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "肝大" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "肝功异常" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "转氨酶升高" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "黄疸" } ] }, { "text": "【实验室检查】1.尿液可出现血尿及蛋白尿、管型尿,尿蛋白主要为白蛋白。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "尿液" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "血尿" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "管型尿" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "白蛋白" } ] }, { "text": "2.血生化往往有白蛋白下降,胆固醇增高,谷丙转氨酶及谷草转氨酶可升高或正常,血浆蛋白电泳α2及β球蛋白升高,γ球蛋白则往往正常。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "血生化" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "白蛋白下降" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "胆固醇增高" }, { "start_idx": 20, "end_idx": 36, "type": "sym", "entity": "谷丙转氨酶及谷草转氨酶可升高或正常" }, { "start_idx": 60, "end_idx": 68, "type": "sym", "entity": "γ球蛋白则往往正常" } ] }, { "text": "3.乙肝血清学标记和HBV-DNA大多数病人为乙肝大三阳(HBsAg、HBeAg及HBcAb阳性),少数病人为小三阳(HBsAg、HBeAb及HBcAb阳性),单纯HBsAg阳性者极少。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "乙肝血清学标记" }, { "start_idx": 23, "end_idx": 48, "type": "sym", "entity": "乙肝大三阳(HBsAg、HBeAg及HBcAb阳性)" }, { "start_idx": 55, "end_idx": 78, "type": "sym", "entity": "小三阳(HBsAg、HBeAb及HBcAb阳性)" } ] }, { "text": "血中HBV-DNA一般阳性。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "ite", "entity": "血" } ] }, { "text": "4.免疫学检查有人认为血IgG和IgA增高,但也有报道50多例HBV-GN膜性肾病患儿均无IgG和IgA增高,相反,约1/3表现为血IgG降低。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "免疫学检查" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "血IgG和IgA增高" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "HBV-GN" }, { "start_idx": 45, "end_idx": 53, "type": "sym", "entity": "IgG和IgA增高" }, { "start_idx": 65, "end_idx": 70, "type": "sym", "entity": "血IgG降低" } ] }, { "text": "5.肾活体组织检查肾活体组织检查是确定HBV-GN的最终手段,是诊断HBV-GN的必备条件。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "HBV-GN" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "HBV-GN" } ] }, { "text": "【诊断】诊断参考1989年10月在北京召开的乙型肝炎(简称乙肝)病毒相关肾炎专题座谈会上对本病诊断的意见。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "乙型肝炎" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "乙肝" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "肾炎" } ] }, { "text": "诊断条件:1.血清乙肝病毒标志物阳性;2.患肾小球肾炎并可除外狼疮性肾炎等继发性肾小球疾病;3.肾组织切片中找到乙肝病毒(HBV)抗原或HBV-DNA;4.肾组织病理改变为膜性肾炎。", "entities": [ { "start_idx": 7, "end_idx": 17, "type": "sym", "entity": "血清乙肝病毒标志物阳性" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "肾小球肾炎" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "外狼疮性肾炎" }, { "start_idx": 37, "end_idx": 44, "type": "dis", "entity": "继发性肾小球疾病" }, { "start_idx": 48, "end_idx": 52, "type": "pro", "entity": "肾组织切片" }, { "start_idx": 56, "end_idx": 59, "type": "mic", "entity": "乙肝病毒" }, { "start_idx": 61, "end_idx": 63, "type": "mic", "entity": "HBV" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "肾组织" }, { "start_idx": 86, "end_idx": 89, "type": "dis", "entity": "膜性肾炎" } ] }, { "text": "值得说明的是:①符合第1、2、3条即可确诊,不论其肾组织病理改变如何;②符合诊断条件中的第1、2条且肾组织病理确诊为膜性肾炎时,尽管其肾组织切片中未查到HBV抗原或HBV-DNA,可作为拟诊;③我国为HBV感染高发地区,如肾小球疾病患者同时有HBV抗原血症,尚不足以作为HBV-GN相关肾炎的依据。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肾组织" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "肾组织" }, { "start_idx": 58, "end_idx": 61, "type": "dis", "entity": "膜性肾炎" }, { "start_idx": 67, "end_idx": 71, "type": "pro", "entity": "肾组织切片" }, { "start_idx": 76, "end_idx": 78, "type": "mic", "entity": "HBV" }, { "start_idx": 100, "end_idx": 102, "type": "mic", "entity": "HBV" }, { "start_idx": 111, "end_idx": 115, "type": "dis", "entity": "肾小球疾病" }, { "start_idx": 121, "end_idx": 127, "type": "dis", "entity": "HBV抗原血症" }, { "start_idx": 135, "end_idx": 140, "type": "dis", "entity": "HBV-GN" }, { "start_idx": 143, "end_idx": 144, "type": "dis", "entity": "肾炎" } ] }, { "text": "【治疗】(一)一般治疗包括低盐饮食、适量优质蛋白饮食;水肿明显时应利尿,可参考本章第三节给予各种口服利尿剂,严重水肿时可静脉应用呋塞米(furosemide,速尿)1~2mg/(kg•次);有高血压时应予硝苯地平(nifedipine,心痛定)每次0.25~0.5mg/kg一日3~4次或ACEI类药物口服治疗,如卡托普利(captopril,巯甲丙脯酸)1~2mg/(kg•d),一日2~3次。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "一般治疗" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "蛋白" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "水肿" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "利尿" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "利尿剂" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "水肿" }, { "start_idx": 60, "end_idx": 66, "type": "pro", "entity": "静脉应用呋塞米" }, { "start_idx": 68, "end_idx": 77, "type": "dru", "entity": "furosemide" }, { "start_idx": 79, "end_idx": 80, "type": "dru", "entity": "速尿" }, { "start_idx": 96, "end_idx": 98, "type": "sym", "entity": "高血压" }, { "start_idx": 102, "end_idx": 105, "type": "dru", "entity": "硝苯地平" }, { "start_idx": 107, "end_idx": 116, "type": "dru", "entity": "nifedipine" }, { "start_idx": 118, "end_idx": 120, "type": "dru", "entity": "心痛定" }, { "start_idx": 144, "end_idx": 150, "type": "dru", "entity": "ACEI类药物" }, { "start_idx": 157, "end_idx": 160, "type": "dru", "entity": "卡托普利" }, { "start_idx": 162, "end_idx": 170, "type": "dru", "entity": "captopril" }, { "start_idx": 172, "end_idx": 176, "type": "dru", "entity": "巯甲丙脯酸" } ] }, { "text": "(二)肾上腺皮质激素与免疫抑制治疗肾上腺皮质激素治疗一直存在争议。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "免疫抑制治疗" }, { "start_idx": 17, "end_idx": 25, "type": "pro", "entity": "肾上腺皮质激素治疗" } ] }, { "text": "对轻至中度蛋白尿的患儿应尽量避免使用肾上腺皮质激素及免疫抑制剂,以免加重病毒复制。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 18, "end_idx": 24, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "免疫抑制剂更不宜使用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "(三)抗病毒治疗主要有α-干扰素(α-interferon)、阿糖腺苷(Ara-A)和拉米呋定(lamivudine)等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "抗病毒治疗" }, { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "α-干扰素" }, { "start_idx": 17, "end_idx": 28, "type": "dru", "entity": "α-interferon" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "阿糖腺苷" }, { "start_idx": 36, "end_idx": 40, "type": "dru", "entity": "Ara-A" }, { "start_idx": 43, "end_idx": 46, "type": "dru", "entity": "拉米呋定" }, { "start_idx": 48, "end_idx": 57, "type": "dru", "entity": "lamivudine" } ] }, { "text": "目前应用α-干扰素治疗HBV-GN取得明显效果,且远期作用尤佳。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "pro", "entity": "α-干扰素治疗" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "HBV-GN" } ] }, { "text": "剂量为α-干扰素20万U/(kg•次),隔日肌注或皮下注射一次,疗程不少于半年,几乎均能收到较好疗效。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "肌注" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "皮下注射" } ] }, { "text": "往往在用药4个月左右能促使HBeAg阴转,少数患儿在治疗10个月左右还能使HBsAg阴转,蛋白尿均能明显阴转或明显减轻。", "entities": [ { "start_idx": 45, "end_idx": 47, "type": "sym", "entity": "蛋白尿" } ] }, { "text": "注射初期可出现发热及流感样症状,几天后即消失。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "发热" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "流感样症状" } ] }, { "text": "阿糖腺苷(Ara-A)也系抗病毒药物,台湾林氏首先用于治疗HBV-GN,取得一定疗效,剂量为15mg/(kg•d),缓慢静脉滴注,2周为一疗程。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "阿糖腺苷" }, { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "Ara-A" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "HBV-GN" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "还可并用胸腺肽以增加疗效,剂量为0.2~0.5mg/(kg•d),1次/日,疗程半年。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "胸腺肽" } ] }, { "text": "Ara-A不良反应大,疗效不及α-干扰素,因此现已被α-INF取代。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "Ara-A" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "α-干扰素" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "α-INF" } ] }, { "text": "拉米呋定(lamivudine)为新的核酸类抗病毒药,年长儿童每日100mg口服,疗程半年以上,对乙肝疗效与α-干扰素接近,用于治疗HBV-GN较少,疗效有待更多资料确定。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "拉米呋定" }, { "start_idx": 5, "end_idx": 14, "type": "dru", "entity": "lamivudine" }, { "start_idx": 19, "end_idx": 25, "type": "dru", "entity": "核酸类抗病毒药" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "乙肝" }, { "start_idx": 54, "end_idx": 58, "type": "dru", "entity": "α-干扰素" }, { "start_idx": 66, "end_idx": 71, "type": "dis", "entity": "HBV-GN" } ] }, { "text": "(四)抗血小板聚集药双嘧达莫(dipyridamole,潘生丁,persantin)5~8mg/(kg•d),分3次口服。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dru", "entity": "抗血小板聚集药" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 15, "end_idx": 26, "type": "dru", "entity": "dipyridamole" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "潘生丁" }, { "start_idx": 32, "end_idx": 40, "type": "dru", "entity": "persantin" } ] }, { "text": "(五)血管紧张素转换酶抑制剂(ACEI)可选用卡托普利(captopril)0.5~1mg/(kg•次),2~3次/日,或依那普利2.5~5mg/次,2~3次/日、西拉普利5~10mg/日,每日1次、福辛普利5~10mg/日,每日1次、贝那普利5mg/d,每日1次等,对降低蛋白尿及保护肾脏有一定效果。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dru", "entity": "血管紧张素转换酶抑制剂" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "ACEI" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "卡托普利" }, { "start_idx": 28, "end_idx": 36, "type": "dru", "entity": "captopril" }, { "start_idx": 61, "end_idx": 64, "type": "dru", "entity": "依那普利" }, { "start_idx": 82, "end_idx": 85, "type": "dru", "entity": "西拉普利" }, { "start_idx": 100, "end_idx": 103, "type": "dru", "entity": "福辛普利" }, { "start_idx": 118, "end_idx": 121, "type": "dru", "entity": "贝那普利" }, { "start_idx": 137, "end_idx": 139, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 143, "end_idx": 144, "type": "bod", "entity": "肾脏" } ] }, { "text": "(六)中医中药中药在护肝治疗及抑制HBV增殖上有一定效果。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dru", "entity": "中药" }, { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "中药" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "护肝治疗" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "HBV" } ] }, { "text": "作者曾试用三普乙肝健,A、B片各2~3片/次,1日3次,治疗3~6个月,收效尚满意;此外,亦可选用乙肝宁及乙肝解毒胶囊长期口服。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "三普乙肝健" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "乙肝宁" }, { "start_idx": 53, "end_idx": 58, "type": "dru", "entity": "乙肝解毒胶囊" } ] }, { "text": "第七节新生儿肺炎新生儿肺炎(neonatalpneumonia)一般指感染性肺炎,可发生于宫内、出生时或出生后。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "新生儿肺炎" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "新生儿肺炎" }, { "start_idx": 14, "end_idx": 30, "type": "dis", "entity": "neonatalpneumonia" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "感染性肺炎" } ] }, { "text": "一、宫内与出生时感染性肺炎【临床流行病学】(一)发病率宫内感染性肺炎占活产新生儿的0.5%。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "感染性肺炎" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "宫内感染性肺炎" } ] }, { "text": "(二)病原学在美国,以B族溶血性链球菌(GBS)为主要致病菌,孕妇阴道GBS的带菌率为20%~50%。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "mic", "entity": "B族溶血性链球菌" }, { "start_idx": 20, "end_idx": 22, "type": "mic", "entity": "GBS" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "阴道" }, { "start_idx": 35, "end_idx": 37, "type": "mic", "entity": "GBS" } ] }, { "text": "国内最近北京市妇产医院调查了1037名孕妇,其GBS带菌率为11.07%,新生儿的GBS带菌率为9.95%,然而,新生儿GBS带菌者的肺炎发生率(20%)与非GBS带菌者的肺炎发生率(14.92%)相比无统计学差异。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "mic", "entity": "GBS" }, { "start_idx": 60, "end_idx": 62, "type": "mic", "entity": "GBS" }, { "start_idx": 67, "end_idx": 68, "type": "dis", "entity": "肺炎" }, { "start_idx": 79, "end_idx": 81, "type": "mic", "entity": "GBS" }, { "start_idx": 86, "end_idx": 87, "type": "dis", "entity": "肺炎" } ] }, { "text": "【病因机制和病理】宫内感染的途径有:(一)血行传播途径母孕期受病毒、细菌、原虫、衣原体和支原体等感染,病原体经血行通过胎盘和羊膜侵袭胎儿。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "血行" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "病毒" }, { "start_idx": 34, "end_idx": 35, "type": "mic", "entity": "细菌" }, { "start_idx": 37, "end_idx": 38, "type": "mic", "entity": "原虫" }, { "start_idx": 40, "end_idx": 42, "type": "mic", "entity": "衣原体" }, { "start_idx": 44, "end_idx": 46, "type": "mic", "entity": "支原体" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "血行" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "胎盘" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "羊膜" } ] }, { "text": "(二)通过羊水感染羊膜早破24小时以上或羊膜绒毛膜炎时,产道内细菌上行性感染,或胎儿在宫内、出生时吸入污染羊水而致病。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "羊水" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "羊膜" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "细菌" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "羊水" } ] }, { "text": "宫内、出生时感染性肺炎的病理改变广泛,肺泡渗出液中含多核细胞、单核细胞和少量红细胞。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "感染性肺炎" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "肺泡渗出液" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "多核细胞" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "单核细胞" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "红细胞" } ] }, { "text": "镜检可见到羊水沉渣,如角化上皮细胞、胎儿皮脂和病原体等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "镜检" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "羊水" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "皮脂" }, { "start_idx": 23, "end_idx": 25, "type": "mic", "entity": "病原体" } ] }, { "text": "【临床表现】(一)宫内感染多在娩出后24小时内发病,婴儿出生时多有窒息,复苏后可见呼吸快、呻吟、体温不稳定、反应差,逐渐出现啰音等表现。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "宫内" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "宫内感染" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "窒息" }, { "start_idx": 41, "end_idx": 43, "type": "sym", "entity": "呼吸快" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "呻吟" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "体温不稳定" }, { "start_idx": 54, "end_idx": 56, "type": "sym", "entity": "反应差" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "啰音" } ] }, { "text": "严重病例可出现呼吸衰竭。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "呼吸衰竭" } ] }, { "text": "血行感染者常缺乏肺部体征,而以黄疸、肝脾大、脑膜炎等多系统受累为主。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肺部" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "黄疸" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "肝脾大" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "通过羊水感染者,常有明显的呼吸困难和肺部啰音(二)出生时感染出生时获得的感染需经过数天至数周潜伏期后始发病,如细菌性肺炎常在出生后3~5小时发病,疱疹病毒感染多在分娩后5~10天出现症状,而衣原体感染潜伏期则长达3~12周。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "羊水" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肺部" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "肺部啰音" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 73, "end_idx": 78, "type": "dis", "entity": "疱疹病毒感染" }, { "start_idx": 95, "end_idx": 99, "type": "dis", "entity": "衣原体感染" } ] }, { "text": "出生时感染的肺炎,患儿因病原不同而临床表现差别较大,且容易发生全身感染。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肺炎" } ] }, { "text": "宫内感染者,X线胸片常显示间质性肺炎改变;通过羊水感染者,X线胸片多显示支气管肺炎改变。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "宫内" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "X线胸片" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "间质性肺炎" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "羊水" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "X线胸片" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "支气管肺炎" } ] }, { "text": "【治疗】对羊膜早破的孕妇在分娩期可用抗生素预防胎儿感染。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "羊膜" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "抗生素" } ] }, { "text": "携带GBS的孕妇在分娩期可用青霉素或氨苄西林预防用药。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "GBS" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "青霉素" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "氨苄西林" } ] }, { "text": "呼吸困难者给氧或采用机械呼吸,加强营养,维持水、电解质和酸碱平衡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "给氧" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "机械呼吸" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "加强营养" }, { "start_idx": 20, "end_idx": 31, "type": "pro", "entity": "维持水、电解质和酸碱平衡" } ] }, { "text": "第四节先天性肾盂输尿管连接部梗阻先天性肾盂输尿管连接部梗阻(congenitalureteropelvicjunctionobstruction,UPJO)是最为常见的小儿泌尿系畸形之一,可导致肾盂积水。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "先天性肾盂输尿管连接部梗阻" }, { "start_idx": 16, "end_idx": 28, "type": "dis", "entity": "先天性肾盂输尿管连接部梗阻" }, { "start_idx": 30, "end_idx": 71, "type": "dis", "entity": "congenitalureteropelvicjunctionobstruction" }, { "start_idx": 73, "end_idx": 76, "type": "dis", "entity": "UPJO" }, { "start_idx": 84, "end_idx": 90, "type": "dis", "entity": "小儿泌尿系畸形" }, { "start_idx": 97, "end_idx": 100, "type": "dis", "entity": "肾盂积水" } ] }, { "text": "肾盂输尿管连接部梗阻包括三种类型:①内源性梗阻;②外源性梗阻;③继发性梗阻。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "肾盂输尿管连接部梗阻" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "内源性梗阻" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "外源性梗阻" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "继发性梗阻" } ] }, { "text": "由于连接部的梗阻,使得肾盂内的尿液无法通畅、及时地流入输尿管,导致肾脏集合系统的持续和进行性扩张,其结果进一步破坏肾盂的排空能力。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肾盂" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "尿液" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "输尿管" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肾脏" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "肾盂" } ] }, { "text": "【发病情况】肾盂输尿管连接部梗阻可以发生在任何年龄儿童,根据美国的统计资料大概有1/4的病例是发生于1岁以内。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "肾盂输尿管连接部梗阻" } ] }, { "text": "从最新的材料来看,随着产前超声的广泛应用,几乎所有的病例都可在围生期得以发现并做出诊断。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "超声" } ] }, { "text": "实际上UPJO是胎儿肾脏集合系统扩张最常见的原因,约占其80%,远远超过多囊性肾发育不良的发生率。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "UPJO" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "肾脏集合系统扩张" }, { "start_idx": 36, "end_idx": 43, "type": "dis", "entity": "多囊性肾发育不良" } ] }, { "text": "进入青春期和成人期,其发现的可能性变小,如果发现肾积水,多系供应肾下极皮质的迷走血管下极皮质的迷走血管跨于UPJO之上造成。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "肾积水" }, { "start_idx": 32, "end_idx": 41, "type": "bod", "entity": "肾下极皮质的迷走血管" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "UPJO" } ] }, { "text": "UPJO多发生于男孩,在新生儿期,男女比例可达2∶1;左侧稍多于右侧,新生儿中2/3发生于左侧。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "UPJO" } ] }, { "text": "双侧UPJO所占比例约为10%~40%,其多发生于新生儿期或小于6个月的婴幼儿。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "UPJO" } ] }, { "text": "已有一家中几代发生UPJO的报道,但遗传倾向尚待进一步证实。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "UPJO" } ] }, { "text": "【病因】尽管从胚胎学、组织学和解剖学进行了多方面的研究,UPJO准确的发生原因仍不清楚。", "entities": [ { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "UPJO" } ] }, { "text": "目前多认为系肾盂输尿管连接部处发育停滞或输尿管在胎儿期有一实质化和再腔化的过程,如再腔化不完全,则造成肾盂输尿管连接部的内源性梗阻。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "肾盂输尿管" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "输尿管" }, { "start_idx": 51, "end_idx": 64, "type": "dis", "entity": "肾盂输尿管连接部的内源性梗阻" } ] }, { "text": "(一)内源性梗阻1958年,Murnaghan发现肾盂输尿管连接部处环行肌肉发育停顿现象,其破坏了连接部的漏斗样结构,造成尿液引流不畅,而肾积水可加重漏斗样结构的破坏。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "内源性梗阻" }, { "start_idx": 25, "end_idx": 37, "type": "bod", "entity": "肾盂输尿管连接部处环行肌肉" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "尿液" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "肾积水" } ] }, { "text": "1968年Nutley,1976年Hanna等在电镜中发现,UPJO处的肌肉细胞排列方向是正常的,但胶原纤维的含量大大超标,由此导致肌纤维间的间距加大,许多肌细胞甚至发生萎缩,使得肾盂输尿管连接部肌肉收缩功能破坏,肾盂内尿液不能排空。", "entities": [ { "start_idx": 36, "end_idx": 47, "type": "sym", "entity": "肌肉细胞排列方向是正常的" }, { "start_idx": 50, "end_idx": 60, "type": "sym", "entity": "胶原纤维的含量大大超标" }, { "start_idx": 66, "end_idx": 74, "type": "sym", "entity": "肌纤维间的间距加大" }, { "start_idx": 76, "end_idx": 86, "type": "sym", "entity": "许多肌细胞甚至发生萎缩" }, { "start_idx": 90, "end_idx": 105, "type": "sym", "entity": "肾盂输尿管连接部肌肉收缩功能破坏" }, { "start_idx": 107, "end_idx": 115, "type": "sym", "entity": "肾盂内尿液不能排空" } ] }, { "text": "息肉一般不大,多位于肾盂输尿管连接部或输尿管的上段,可形成不全梗阻。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "息肉" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "肾盂输尿管连接部" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "输尿管" } ] }, { "text": "输尿管先天性黏膜皱襞尿管先天性黏膜皱襞是输尿管瓣膜尿管瓣膜的一种,在4个月以后胎儿的上段输尿管中是一种非常常见的现象,这种黏膜皱襞甚至可延续至新生儿期。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "输尿管先天性黏膜皱襞" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "输尿管瓣膜" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "输尿管" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "黏膜皱襞" } ] }, { "text": "一般来说,黏膜皱襞并不形成梗阻,随着生长发育可以消失,在年长的儿童或成人中是很少见的。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "黏膜皱襞" } ] }, { "text": "如这种胎儿黏膜皱襞持续存在并不断增厚,再有肌肉组织进入,或在肾盂输尿管连接部形成瓣膜样的结构,则可形成梗阻。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "黏膜皱襞" }, { "start_idx": 30, "end_idx": 37, "type": "bod", "entity": "肾盂输尿管连接部" } ] }, { "text": "手术时,包含瓣膜的输尿管部分必须切除。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "瓣膜的输尿管" } ] }, { "text": "(二)外源性梗阻外源性梗阻最常见的是支配肾下极皮质的迷走或副支血管压迫肾盂输尿管连接部,这支血管常在肾盂输尿管连接部或输尿管上端的前面穿过。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "外源性梗阻" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "外源性梗阻" }, { "start_idx": 20, "end_idx": 27, "type": "bod", "entity": "肾下极皮质的迷走" }, { "start_idx": 29, "end_idx": 42, "type": "bod", "entity": "副支血管压迫肾盂输尿管连接部" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "血管" }, { "start_idx": 50, "end_idx": 57, "type": "bod", "entity": "肾盂输尿管连接部" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "输尿管上端" } ] }, { "text": "迷走血管使输尿管折叠成角,当肾盂充盈时可在肾盂输尿管连接部和血管通过输尿管处两处形成梗阻而被迷走血管牵拉折叠向上的输尿管可以和肾盂之间形成筋膜粘连输尿管长期受压可以导致缺血、纤维化以及狭窄,故尽管有人认为松解粘连及游离血管即可解除UPJO,但还是将病变梗阻的输尿管切除为好。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "迷走血管" }, { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "输尿管折叠成角" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "肾盂输尿管" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "血管" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "输尿管" }, { "start_idx": 13, "end_idx": 43, "type": "sym", "entity": "当肾盂充盈时可在肾盂输尿管连接部和血管通过输尿管处两处形成梗阻" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "输尿管" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "肾盂" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "筋膜" }, { "start_idx": 44, "end_idx": 72, "type": "sym", "entity": "而被迷走血管牵拉折叠向上的输尿管可以和肾盂之间形成筋膜粘连" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "输尿管" }, { "start_idx": 84, "end_idx": 85, "type": "sym", "entity": "缺血" }, { "start_idx": 87, "end_idx": 93, "type": "sym", "entity": "纤维化以及狭窄" } ] }, { "text": "(三)继发性梗阻UPJO可由于严重的膀胱输尿管反流引起,占其总数的10%左右。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "继发性梗阻" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "UPJO" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "膀胱输尿管反流" } ] }, { "text": "反流可引起输尿管的扭曲、增粗及拉长粗及拉长,而因肾盂输尿管连接部的位置相对固定,产生折叠,导致梗阻。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "输尿管的扭曲" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "增粗及拉长" }, { "start_idx": 22, "end_idx": 48, "type": "sym", "entity": "而因肾盂输尿管连接部的位置相对固定,产生折叠,导致梗阻" } ] }, { "text": "同样原理UPJO也由于膀胱输尿管连接部梗阻引起。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "UPJO" }, { "start_idx": 11, "end_idx": 20, "type": "dis", "entity": "膀胱输尿管连接部梗阻" } ] }, { "text": "【伴发畸形】UPJO患儿伴发对侧肾脏畸形的几率相当高,也包括其他一些先天性畸形。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "UPJO" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "对侧肾脏畸形" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "先天性畸形" } ] }, { "text": "对侧UPJO的发生率报告为10%~40%,其他可以为肾发育不良及多囊性肾病等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "UPJO" }, { "start_idx": 26, "end_idx": 36, "type": "dis", "entity": "肾发育不良及多囊性肾病" } ] }, { "text": "UPJO也可发生在重复肾的上半肾或下半肾,也可发生于马蹄肾或异位肾。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "UPJO" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "重复肾" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "上半肾" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "下半肾" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "异位肾" } ] }, { "text": "UPJO患者轻度膀胱输尿管反流的发生率可达40%左右,其更多可能是尿路感染造成,可以自行消失。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "UPJO" }, { "start_idx": 6, "end_idx": 14, "type": "dis", "entity": "轻度膀胱输尿管反流" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "尿路感染" } ] }, { "text": "UPJO在Vater综合征中的发生率为21%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "UPJO" }, { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "Vater综合征" } ] }, { "text": "【症状】在新生儿或婴幼儿中UPJO导致肾积水可表现为无症状的腹块,在体检中被偶然发现。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "UPJO" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "肾积水" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "无症状的腹块" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "在体检中被偶然发现" } ] }, { "text": "随着孕期超声的常规使用,近年来许多UPJO导致肾积水的病例在胎儿期就诊断出来。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "超声" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "UPJO" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "肾积水" } ] }, { "text": "其他的表现尚包括生长发育迟缓、喂养困难、反复尿路感染和血尿等UPJO所致尿路梗阻由于是不完全的,病情进展缓慢,也无明确症状,有时在尿量较多时,因引流不畅致肾盂收缩而出现腹痛,但多难指出具体部位,可以伴恶心呕吐。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "生长发育迟缓" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "喂养困难" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "血尿" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "反复尿路感染和血尿等" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "UPJO" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "尿路梗阻" }, { "start_idx": 64, "end_idx": 69, "type": "sym", "entity": "在尿量较多时" }, { "start_idx": 71, "end_idx": 85, "type": "sym", "entity": "因引流不畅致肾盂收缩而出现腹痛" }, { "start_idx": 87, "end_idx": 103, "type": "sym", "entity": "但多难指出具体部位,可以伴恶心呕吐" } ] }, { "text": "在迷走血管导致的UPJO中,由于间隙性的梗阻,患儿有阵发性腹痛,有时可伴呕吐。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "迷走血管导致" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "UPJO" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "间隙性的梗阻" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "患儿有阵发性腹痛" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "有时可伴呕吐" } ] }, { "text": "血尿常由积水的肾盂黏膜血管破裂引起。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "血尿" }, { "start_idx": 7, "end_idx": 12, "type": "bod", "entity": "肾盂黏膜血管" } ] }, { "text": "年长的患儿,如青春期的常在大量饮水后出现腹痛。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "腹痛" } ] }, { "text": "大龄患者可出现高血压,原因是由于集合系统扩张,肾血流下降,肾脏功能性缺血,由肾素、血管紧张素介导产生。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "高血压" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "集合系统扩张" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "肾血流下降" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "肾脏功能性缺血" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "肾素" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "血管紧张素" } ] }, { "text": "【诊断】对胎儿期诊断有肾积水的患儿,出生后必须进行复查,时间最好是出生后和出生数周后(一般为4周)。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肾积水" } ] }, { "text": "在生后大约3个月以内,肾脏尚在发育中,肾锥体和髓质都是透声的,可以产生肾积水的假象,故出生后B超肾积水期检查非常必要。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肾脏" }, { "start_idx": 19, "end_idx": 28, "type": "sym", "entity": "肾锥体和髓质都是透声" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "B超" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "肾积水" } ] }, { "text": "目前在胎儿及新生儿的肾积水的程度估计上仍有困难。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾积水" } ] }, { "text": "从已有报告来看,轻中度的肾积水[肾盂扩张(2cm)的大部分患儿],在2年内可以自行缓解。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "轻中度的肾积水" } ] }, { "text": "这部分患儿的同位素肾图曲线上表现为对放射性核素的摄取正常,但排泄明显推迟。", "entities": [ { "start_idx": 1, "end_idx": 27, "type": "sym", "entity": "部分患儿的同位素肾图曲线上表现为对放射性核素的摄取正常" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "但排泄明显推迟" } ] }, { "text": "UPJO导致肾积水的实验室检查绝大部分患儿的尿常规都是正常的尿常规都是正常的,当出现尿路感染时,可以有白细胞出现;也有部分患儿表现为血尿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "UPJO" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "肾积水" }, { "start_idx": 15, "end_idx": 29, "type": "sym", "entity": "绝大部分患儿的尿常规都是正常的" }, { "start_idx": 39, "end_idx": 55, "type": "sym", "entity": "当出现尿路感染时,可以有白细胞出现" }, { "start_idx": 57, "end_idx": 67, "type": "sym", "entity": "也有部分患儿表现为血尿" } ] }, { "text": "除非是非常严重的双肾积水,一般肾积水的患儿其肾功能都在正常范围。", "entities": [ { "start_idx": 0, "end_idx": 30, "type": "sym", "entity": "除非是非常严重的双肾积水,一般肾积水的患儿其肾功能都在正常范围" } ] }, { "text": "(一)超声是最常用和最有效的无创检查手段,可以发现扩张而分离的肾盂及肾盏,并可测量出肾皮质的厚薄。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "超声" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肾盂" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肾盏" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "肾皮质" } ] }, { "text": "一般来说,UPJO致肾积水其输尿管口径基本正常。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "UPJO" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾积水" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "输尿管口径基本正常" } ] }, { "text": "超声对肾积水程度的判断除非是非常严重的巨大积水,其数据并不能反映肾积水的严重程度和肾功能的实际情况,但仍是首选的方法。", "entities": [ { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "肾积水" } ] }, { "text": "(二)排泄性静脉尿路造影(IVU)显示患肾肾盂扩张,肾盏扩张,肾盂输尿管连接部中断,输尿管不显示。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "排泄性静脉尿路造影" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "IVU" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "肾肾盂扩张" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "肾盏扩张" }, { "start_idx": 31, "end_idx": 40, "type": "sym", "entity": "肾盂输尿管连接部中断" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "输尿管不显示" } ] }, { "text": "我们常用76%泛影葡胺2.2ml/kg快速经静脉推入,肾脏开始显影时间的长短以及造影剂显示的程度可以反映患肾功能的变化。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "泛影葡胺2" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肾脏" }, { "start_idx": 40, "end_idx": 42, "type": "dru", "entity": "造影剂" } ] }, { "text": "从肾脏的大小、肾盂和肾盏扩张的程度以及造影剂排泄的时间可以反映积水的严重程度。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "肾脏" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肾盂" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肾盏" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "造影剂" } ] }, { "text": "依积水严重的程度,肾盏的表现可以从较轻的杯口变钝、变平,发展至严重的肾盏向外膨出、隆起及饱胀的肾盏向外膨出、隆起及饱胀。", "entities": [ { "start_idx": 9, "end_idx": 26, "type": "sym", "entity": "肾盏的表现可以从较轻的杯口变钝、变平" }, { "start_idx": 28, "end_idx": 45, "type": "sym", "entity": "发展至严重的肾盏向外膨出、隆起及饱胀" } ] }, { "text": "有时造影剂滞留于扩张的肾盏中而未进入肾盂,状如作画用的调色板。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "造影剂" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肾盂" } ] }, { "text": "但目前肾脏因积水严重而不显影时,多用MRU替代。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" } ] }, { "text": "UPJO一般情况下输尿管不显示,但即使是肾盂输尿管连接部远端的输尿管能够显示,仍可能在肾盂输尿管连接部处存在严重的梗阻。", "entities": [ { "start_idx": 20, "end_idx": 29, "type": "bod", "entity": "肾盂输尿管连接部远端" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "输尿管" }, { "start_idx": 43, "end_idx": 50, "type": "bod", "entity": "肾盂输尿管连接部" } ] }, { "text": "经膀胱镜输尿管逆行插管造影可以明确输尿管全程的情况,一般主张其在手术当日进行。", "entities": [ { "start_idx": 1, "end_idx": 12, "type": "pro", "entity": "膀胱镜输尿管逆行插管造影" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "输尿管" } ] }, { "text": "(三)利尿性肾图肾盂积水的另一重要诊断方法是利尿性肾图,采用同位素技术,对双肾的血流灌注、分肾功能及示踪剂排泄进行测定、分析。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "利尿性肾图" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "肾盂积水" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "同位素技术" } ] }, { "text": "一般来说肾图曲线的同位素摄取相评价肾功能,注射呋塞米后的排泄相可以确定梗阻的严重程度。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "pro", "entity": "肾图曲线的同位素摄取" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "呋塞米" } ] }, { "text": "对间隙性肾盂输尿管连接部梗阻的患儿来说,利尿性肾图尤其有价值。", "entities": [ { "start_idx": 1, "end_idx": 13, "type": "dis", "entity": "间隙性肾盂输尿管连接部梗阻" }, { "start_idx": 20, "end_idx": 24, "type": "pro", "entity": "利尿性肾图" } ] }, { "text": "这部分患儿仅在腹痛时才能发现PUJO的存在,而无症状时其排泄性尿路造影可以完全正常,上述情况多见于迷走血管压迫引起的肾积水。", "entities": [ { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "排泄性尿路造影" }, { "start_idx": 49, "end_idx": 60, "type": "dis", "entity": "迷走血管压迫引起的肾积水" } ] }, { "text": "注射呋塞米,尿量增加,肾盂排空无法及时完成,肾积水的症状得以出现。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "呋塞米" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "尿量" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肾盂" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "肾积水" } ] }, { "text": "当然有些患儿在做利尿性肾图时可以出现腹痛。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "利尿性肾图" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "出现腹痛" } ] }, { "text": "利尿性肾图避免了常规造影方法的放射性危害,探测敏感,即使功能很差的肾脏,如果有同位素示踪剂进入即可显示,可以提供分肾功能的指标,从而对术前、术后的情况进行比较。", "entities": [ { "start_idx": 39, "end_idx": 44, "type": "dru", "entity": "同位素示踪剂" } ] }, { "text": "从我们的经验来看,术后的肾图复查以术后6个月为宜,如在3个月以前进行利尿性肾图检查,可能因吻合口水肿影响尿液引流,仍呈机械性梗阻征象。", "entities": [ { "start_idx": 34, "end_idx": 38, "type": "ite", "entity": "利尿性肾图" } ] }, { "text": "(四)磁共振尿路显像(MRU)由于尿路梗阻导致积水、扩张,在磁共振的T2相上可以显示,尤其在肾功能不良,IVU和同位素肾图均不能显示的情况下,通过MRU可以显示尿路的解剖形态,提示梗阻的部位。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "磁共振尿路显像" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "MRU" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "尿路梗阻" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "积水、扩张" }, { "start_idx": 58, "end_idx": 60, "type": "pro", "entity": "IVU" }, { "start_idx": 62, "end_idx": 66, "type": "pro", "entity": "同位素肾图" } ] }, { "text": "(五)排尿性膀胱尿道造影肾积水的患儿都必须做排尿性膀胱尿道造影以排除膀胱输尿管反流导致的肾积水。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "排尿性膀胱尿道造影" }, { "start_idx": 22, "end_idx": 30, "type": "pro", "entity": "排尿性膀胱尿道造影" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "肾积水" } ] }, { "text": "【治疗】积水量少、肾功能正常,如果无严重的腹痛及血尿等症状,生长发育良好的患儿可以随访观察,依据其积水发展的情况再作决定。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "腹痛" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "血尿" } ] }, { "text": "对诊断明确,肾功能已有损害或肾功能尽管尚属正常,但有反复的腹痛、血尿及尿路感染等症状,均宜手术治疗。", "entities": [ { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "反复的腹痛" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "血尿及尿路感染" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "手术治疗" } ] }, { "text": "手术方式是成熟的,进行离断型肾盂输尿管成形术。", "entities": [ { "start_idx": 11, "end_idx": 21, "type": "pro", "entity": "离断型肾盂输尿管成形术" } ] }, { "text": "关于吻合口是否需支撑管的问题,日前尚有争论,美国的报告多不用支撑管,仅留置创面引流;国内有留置双“J”管的,问题是3~4个月后需再在麻醉下通过膀胱镜将其取出。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "equ", "entity": "支撑管" }, { "start_idx": 30, "end_idx": 32, "type": "equ", "entity": "支撑管" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "创面引流" }, { "start_idx": 66, "end_idx": 67, "type": "pro", "entity": "麻醉" }, { "start_idx": 71, "end_idx": 73, "type": "equ", "entity": "膀胱镜" } ] }, { "text": "如系小婴儿,膀胱镜恐不能进入,不得已要切开膀胱,总之都需要麻醉。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "equ", "entity": "膀胱镜" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "膀胱" } ] }, { "text": "我们常规采用8Fr的胃管,从肾盂置入输尿管,另一端从肾皮质穿过,固定于切口皮肤。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "胃管" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肾盂" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "输尿管" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "肾皮质" } ] }, { "text": "近十年来,内腔镜技术得到了比较快的发展,采用腹腔镜经腹腔或后腹膜途径进行肾盂成形术也有了不少报道。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "equ", "entity": "内腔镜" }, { "start_idx": 22, "end_idx": 24, "type": "equ", "entity": "腹腔镜" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "腹腔" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "腹膜" }, { "start_idx": 36, "end_idx": 40, "type": "pro", "entity": "肾盂成形术" } ] }, { "text": "由于难度较大的小切口肾盂成形术也达到了超过99%的手术成功率,且手术时间一般为1小时左右,比较多的意见认为腹腔镜肾盂成形术广泛开展尚待时机,尤其依赖于机器人腹腔镜技术的进一步发展。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "肾盂成形术" }, { "start_idx": 53, "end_idx": 60, "type": "pro", "entity": "腹腔镜肾盂成形术" }, { "start_idx": 75, "end_idx": 80, "type": "pro", "entity": "机器人腹腔镜" } ] }, { "text": "2002年美国儿科年会上波士顿儿童医院报告采用机器人腹腔镜进行肾盂成形术,受到了极大的关注,目前的主要问题是仪器及其使用的费用太高,难以普及。", "entities": [ { "start_idx": 23, "end_idx": 28, "type": "equ", "entity": "机器人腹腔镜" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "肾盂成形术" } ] }, { "text": "严重巨大肾积水这类病例现在已少见,由于小儿的肾脏多有相当的代偿功能,故对积水巨大的肾脏要谨慎处理,不宜草率作出切除肾脏的决定。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "严重巨大肾积水" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肾脏" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "肾脏" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "肾脏" } ] }, { "text": "我们对这类患儿先做肾造瘘,观察其肾功能恢复情况,从我们总结来看,36例造瘘肾经6~9个月的观察,其中32例肾功能有恢复,IVU上肾由不显影至显影,同位素肾图分肾功能增加,均做肾盂成形术,随访下来肾功能均能维持比较好的状态。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "肾造瘘" }, { "start_idx": 60, "end_idx": 62, "type": "pro", "entity": "IVU" }, { "start_idx": 73, "end_idx": 77, "type": "pro", "entity": "同位素肾图" }, { "start_idx": 87, "end_idx": 91, "type": "pro", "entity": "肾盂成形术" } ] }, { "text": "UPJO致肾积水尤其是胎儿及新生儿肾积水的手术时机一直存在争议。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "UPJO" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肾积水" } ] }, { "text": "20世纪80年代初,常见有胎儿肾脏减压以保护肾功能的报道,现在来看,仅在胎儿双侧肾积水伴羊水进行性减少时才要施用。", "entities": [ { "start_idx": 38, "end_idx": 45, "type": "dis", "entity": "双侧肾积水伴羊水" } ] }, { "text": "但胎儿肾积水减压后肾功能及肺功能恢复的程度目前都未证实。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "胎儿肾积水" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肾" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肺" } ] }, { "text": "对于肾积水患儿出生后的手术时机,争论也比较大。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肾积水" } ] }, { "text": "另一方面,也有许多医师,尤其是美国的小儿泌尿外科医师对肾积水的患儿进行长期随访,发现即使是肾盂扩张程度比较重的,肾功能已有损伤的新生儿,其积水也并未恶化。", "entities": [ { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "肾积水" } ] }, { "text": 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"entities": [ { "start_idx": 2036, "end_idx": 2038, "type": "dis", "entity": "代谢病" }, { "start_idx": 2085, "end_idx": 2089, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 2142, "end_idx": 2146, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 2148, "end_idx": 2158, "type": "dis", "entity": "先天性甲状腺功能减低症" }, { "start_idx": 2160, "end_idx": 2164, "type": "dis", "entity": "新生儿疾病" }, { "start_idx": 2295, "end_idx": 2299, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 2349, "end_idx": 2353, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 2364, "end_idx": 2365, "type": "dep", "entity": "儿科" }, { "start_idx": 2409, "end_idx": 2413, "type": "pro", "entity": "脆性X检测" }, { "start_idx": 2458, "end_idx": 2464, "type": "dis", "entity": "高苯丙氨酸血症" }, { "start_idx": 2519, "end_idx": 2524, "type": "dis", "entity": "黏多糖贮积症" }, { "start_idx": 2538, "end_idx": 2539, "type": "dep", "entity": "儿科" }, { "start_idx": 2573, "end_idx": 2575, "type": "dep", "entity": "儿科学" } ] }, { "text": "参考文献1.陈清棠.临床神经病学.北京科学技术出版社,2000:12.李庆有,等.肝豆状核变性的脑MRI与临床表现的相关分析.中华神经科杂志,1997,30(6):367-3673.吴斌.小儿肝豆状核变性的诊治新进展.国外医学儿科学分册,1997,24(4):202-2024.杨任民.肝豆状核变性的治疗.中国实用儿科杂志,2000,15(2):79-805.刘晓青,张雅芳,刘孜孜等.肝豆状核变性基因类型与临床表型关系研究.中华儿科杂志,2003,41(1):35-356.LoudianosG,GitlinJD.Wilson’sdisease.SeminLiverDis,2000,20(3):353-3537.RichardsRJ,HammittJK.Findingtheoptimalmultiple-teststrategyusingamethodanalogoustologisticregression:thediagnosisofhepatolenticulardegeneration(Wilson’sdisease).MedDecisMaking,1996,16(4):367-3678.RobertsEA,CoxDW.Wilsondisease.BaillieresClinGastroenterol,1998,12(2):237-2379.SmoloarekC,StremmelW.TherapyofWilsondisease.ZGastroenterol,1999,37(4):293-29310.中华医学会神经病学分会帕金森病及运动障碍学组.中华医学会神经病学分会神经遗传病学组.肝豆状核变性的诊断与治疗指南《中华神经科杂志》2008,41(8):566-566", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "神经病" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 48, "end_idx": 51, "type": "ite", "entity": "脑MRI" }, { "start_idx": 96, "end_idx": 101, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 143, "end_idx": 148, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 193, "end_idx": 198, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 667, "end_idx": 669, "type": "dis", "entity": "神经病" }, { "start_idx": 673, "end_idx": 676, "type": "dis", "entity": "帕金森病" }, { "start_idx": 678, "end_idx": 681, "type": "dis", "entity": "运动障碍" }, { "start_idx": 690, "end_idx": 692, "type": "dis", "entity": "神经病" }, { "start_idx": 704, "end_idx": 709, "type": "dis", "entity": "肝豆状核变性" } ] }, { "text": "四、弥漫性淋巴管瘤弥漫性淋巴管瘤又称淋巴管瘤性巨肢症。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "弥漫性淋巴管瘤" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "弥漫性淋巴管瘤" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "淋巴管瘤性巨肢症" } ] }, { "text": "系由胚胎早期的胚芽所生长,因弥漫性淋巴管瘤占领整个肢体,从指端到肩部,或从脚趾到腹股沟。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "胚胎" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "胚芽" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "弥漫性淋巴管瘤" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肢体" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "指端" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肩部" } ] }, { "text": "皮肤上有时有毛细血管斑痣肿瘤病变广泛,分布在肢体的所有组织,浸润肌肉和肌间隙,甚至骨膜。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "毛细血管" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "皮肤上有时有毛细血管斑痣" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肿瘤" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肢体" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "组织" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肌肉" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "肌间隙" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "骨膜" } ] }, { "text": "二、肺大疱肺大疱(pneumatoceles)又称为泡性肺气肿(bullousemphysematousblebsorcysts)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肺大疱" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肺大疱" }, { "start_idx": 9, "end_idx": 21, "type": "dis", "entity": "pneumatoceles" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "泡性肺气肿" }, { "start_idx": 32, "end_idx": 63, "type": "dis", "entity": "bullousemphysematousblebsorcysts" } ] }, { "text": "多数见于婴幼儿,最常见的病因为葡萄球菌性肺炎,由于支气管黏膜广泛充血水肿渗出,导致小气道狭窄,形成活瓣,肺泡内空气不断积聚,过度膨胀发生破裂,许多肺泡融合在一起,形成一个或多个肺大疱。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "葡萄球菌性肺炎" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "支气管黏膜" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "充血水肿渗出" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "小气道狭窄" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "形成活瓣" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "肺泡" }, { "start_idx": 55, "end_idx": 60, "type": "sym", "entity": "空气不断积聚" }, { "start_idx": 62, "end_idx": 69, "type": "sym", "entity": "过度膨胀发生破裂" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "肺泡" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "肺大疱" } ] }, { "text": "肺大疱体积小者可无任何症状,体积大而压力高者可致急性呼吸困难。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肺大疱" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "急性呼吸困难" } ] }, { "text": "诊断有赖于X线肺部摄片,胸片可见四周有薄壁构成的环状透亮阴影,含空气或含空气和液体,后者可见气液平面,且随体位而改变,其位置、透明区可迅速出现、迅速消失、忽大忽小,此为本病的特点。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "ite", "entity": "X线肺部摄片" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "胸片" }, { "start_idx": 16, "end_idx": 29, "type": "sym", "entity": "四周有薄壁构成的环状透亮阴影" }, { "start_idx": 31, "end_idx": 40, "type": "sym", "entity": "含空气或含空气和液体" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "气液平面" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "随体位而改变" }, { "start_idx": 60, "end_idx": 70, "type": "sym", "entity": "位置、透明区可迅速出现" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "迅速消失" }, { "start_idx": 77, "end_idx": 80, "type": "sym", "entity": "忽大忽小" } ] }, { "text": "本病预后大多良好,症状随呼吸道感染的痊愈及支气管梗阻的消除而消退。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "支气管梗阻" } ] }, { "text": "第四节新生儿颅内出血颅内出血(intracraninalhemorrhage)是新生儿期常见的临床问题,出血部位包括硬膜下出血、蛛网膜下腔出血、脑室周围-脑室内出血、小脑出血和脑实质出血。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "新生儿颅内出血" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "颅内出血" }, { "start_idx": 15, "end_idx": 37, "type": "dis", "entity": "intracraninalhemorrhage" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 64, "end_idx": 70, "type": "dis", "entity": "蛛网膜下腔出血" }, { "start_idx": 72, "end_idx": 81, "type": "dis", "entity": "脑室周围-脑室内出血" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "小脑出血" }, { "start_idx": 88, "end_idx": 92, "type": "dis", "entity": "脑实质出血" } ] }, { "text": "近年,由于产科技术的进步,产伤所致的硬膜下出血明显减少,而早产儿缺氧所致的脑室周围-脑室内出血已成为新生儿颅内出血最常见的类型。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dep", "entity": "产科" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "缺氧" }, { "start_idx": 37, "end_idx": 46, "type": "dis", "entity": "脑室周围-脑室内出血" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "新生儿颅内出血" } ] }, { "text": "一、脑室周围-脑室内出血脑室周围-脑室内出血(intraventricularhemorrhage,IVH)是早产儿最常见的颅内出血类型。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "脑室周围-脑室内出血" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "脑室周围-脑室内出血" }, { "start_idx": 23, "end_idx": 48, "type": "dis", "entity": "intraventricularhemorrhage" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "IVH" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "颅内出血" } ] }, { "text": "近年,随着新生儿医疗护理水平的改善,极低出生体重儿成活率显著提高,IVH已成为NICU早产儿的重要问题。", "entities": [ { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "IVH" } ] }, { "text": "【病因与发病机制】早产儿脑室周围室管膜下生发基质富含血管,这些血管在解剖学上是一种不成熟的毛细血管网,仅由一层内皮细胞组成,缺乏肌层和结缔组织支持,该区域对缺氧和高碳酸血症极为敏感,当缺氧致脑血流自我调节功能受损时,惊厥、气管吸引、快速扩容、静脉输注高渗溶液等可致血压波动而促发管破裂出血。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "脑室" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "室管膜" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "生发基质" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "血管" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "血管" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "毛细血管网" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "肌层" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "结缔组织" }, { "start_idx": 78, "end_idx": 79, "type": "sym", "entity": "缺氧" }, { "start_idx": 81, "end_idx": 85, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 92, "end_idx": 93, "type": "sym", "entity": "缺氧" }, { "start_idx": 108, "end_idx": 109, "type": "sym", "entity": "惊厥" }, { "start_idx": 132, "end_idx": 133, "type": "ite", "entity": "血压" } ] }, { "text": "此外,生发基质的毛细血管网在引流入静脉系统时的血流方向呈独特的U形,这在生发基质出血中起重要作用,当胎头娩出困难、颅骨过度受压时可使该处血流停滞而发生出血。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "生发基质" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "毛细血管" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "生发基质出血" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "颅骨" }, { "start_idx": 68, "end_idx": 71, "type": "sym", "entity": "血流停滞" }, { "start_idx": 75, "end_idx": 76, "type": "dis", "entity": "出血" } ] }, { "text": "生发基质的宽度在胎龄23~25周时为2.5mm,32周为1.4mm,36周时几乎完全退化,因此IVH主要发生在胎龄小于33周的早产儿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "生发基质" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "IVH" } ] }, { "text": "在生发基质出血的病例中,80%的患儿血液可进入侧脑室,血液通过马氏孔和路氏孔进入后颅凹的基底池,引起闭塞致使脑脊液循环障碍,血凝块也可阻塞大脑导水管和蛛网膜绒毛而引起出血后脑积水和脑室周围出血性梗死。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "生发基质出血" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "血液" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "侧脑室" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血液" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "后颅凹" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "脑脊液" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "血凝块" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "大脑" }, { "start_idx": 83, "end_idx": 84, "type": "dis", "entity": "出血" }, { "start_idx": 86, "end_idx": 88, "type": "dis", "entity": "脑积水" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "脑室" }, { "start_idx": 94, "end_idx": 98, "type": "dis", "entity": "出血性梗死" } ] }, { "text": "虽然IVH是早产儿的常见病,但足月儿也可发生,足月儿IVH的起源主要为脉络膜丛和室管膜下残存的生发基质。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "IVH" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "足月儿IVH" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "脉络膜丛" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "室管膜" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "生发基质" } ] }, { "text": "在足月儿IVH的发病机制中,产伤的作用比缺氧更为重要,其中30%的患儿有产钳分娩或臀位牵引史,还有25%的患儿可无明显诱因,既无产伤也无缺氧。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "足月儿IVH" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "缺氧" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "缺氧" } ] }, { "text": "近年研究发现早产儿IVH与机体凝血状况有关,某些凝血因子表达减少可能会加重IVH。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "早产儿IVH" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "IVH" } ] }, { "text": "【临床表现】IVH主要见于围生期窒息和早产儿,出血50%开始于生后第1天,30%发生在第2天,到生后72小时头颅超声可发现90%的IVH。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "IVH" }, { "start_idx": 54, "end_idx": 57, "type": "pro", "entity": "头颅超声" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "IVH" } ] }, { "text": "临床表现可有三种类型:急剧恶化型、断续进展型和临床寂静型。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "急剧恶化型" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "断续进展型" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "临床寂静型" } ] }, { "text": "以寂静型最为常见,占IVH病例的50%,无临床症状或体征,仅在超声或CT检查时发现。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "sym", "entity": "寂静型" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "IVH" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "超声" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "CT检查" } ] }, { "text": "【诊断】早产儿IVH的临床症状和体征较少,单凭临床表现很难诊断。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "早产儿IVH" } ] }, { "text": "影像学检查是IVH的主要诊断手段,要根据具体情况选择头颅B超或CT检查。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "影像学检查" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "IVH" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "头颅B超" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "CT检查" } ] }, { "text": "(一)头颅超声是诊断IVH的首选方法。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "头颅超声" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "IVH" } ] }, { "text": "床旁头颅超声对早产儿IVH的开始时间、出血部位及严重程度提供可靠的信息,而且价廉方便,又无放射线损伤。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "头颅超声" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "早产儿IVH" } ] }, { "text": "极低出生体重儿是易发生IVH的高危人群,应常规进行头颅超声的筛查。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "IVH" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "头颅超声" } ] }, { "text": "头颅超声检查可将IVH分为4级:①Ⅰ级:出血限于室管膜下,不伴脑室内出血;②Ⅱ级:不伴脑室扩张的IVH;③Ⅲ级:IVH(>50%脑室区域)伴脑室扩大;④Ⅳ级:脑室内出血合并脑实质出血或脑室周围出血性梗死。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "头颅超声检查" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "IVH" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "Ⅰ级" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "室管膜" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "脑室" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "脑室内出血" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "Ⅱ级" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "脑室" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "脑室扩张" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "IVH" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "Ⅲ级" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "IVH" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "脑室" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "脑室" }, { "start_idx": 76, "end_idx": 77, "type": "dis", "entity": "Ⅳ级" }, { "start_idx": 79, "end_idx": 83, "type": "dis", "entity": "脑室内出血" }, { "start_idx": 86, "end_idx": 90, "type": "dis", "entity": "脑实质出血" }, { "start_idx": 92, "end_idx": 100, "type": "dis", "entity": "脑室周围出血性梗死" } ] }, { "text": "(二)CT检查CT是证实IVH部位和程度的有效手段,对硬膜下出血、后颅凹出血、蛛网膜下腔出血和某些脑实质的损害,CT的诊断价值优于超声。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "CT检查" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "CT" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "IVH" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "后颅凹出血" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "蛛网膜下腔出血" }, { "start_idx": 56, "end_idx": 57, "type": "pro", "entity": "CT" }, { "start_idx": 65, "end_idx": 66, "type": "pro", "entity": "超声" } ] }, { "text": "但CT不能床旁进行,还有使患儿暴露于放射线的缺点。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "CT" } ] }, { "text": "(三)脑脊液检查IVH的脑脊液表现为出血早期脑脊液红细胞数量和蛋白含量增高,部分病例白细胞增高,然后脑脊液变为黄色,葡萄糖含量降低。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "IVH" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "脑脊液" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "脑脊液红细胞" }, { "start_idx": 18, "end_idx": 46, "type": "sym", "entity": "出血早期脑脊液红细胞数量和蛋白含量增高,部分病例白细胞增高" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "脑脊液" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "葡萄糖" } ] }, { "text": "但是,有些病例脑脊液不呈血性,因此不能将腰椎穿刺作为IVH的确诊手段。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "脑脊液" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "IVH" } ] }, { "text": "【预后】与出血的严重程度及部位有关,如出血仅限于生发基质或伴少量IVH者预后较好,很少发生脑室扩张。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "出血" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "出血" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "生发基质" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "IVH" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "脑室" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "脑室扩张" } ] }, { "text": "中度出血者,病死率略为增高,存活者中20%~30%发生脑积水。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "出血" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "脑积水" } ] }, { "text": "严重出血病例病死率20%~30%左右,存活者常发生脑积水。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "脑积水" } ] }, { "text": "重度IVH伴脑室周围出血性梗死者,病死率和脑积水发生率均较高,分别为40%和70%。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "IVH" }, { "start_idx": 6, "end_idx": 14, "type": "dis", "entity": "脑室周围出血性梗死" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "脑积水" } ] }, { "text": "IVH的远期预后取决于伴随的脑实质损害的程度,如伴有脑室周围白质软化,可发生四肢对称性痉挛性瘫痪,下肢重于上肢。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "IVH" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "脑实质损害" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "脑室" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "四肢" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "下肢" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "上肢" }, { "start_idx": 26, "end_idx": 54, "type": "sym", "entity": "脑室周围白质软化,可发生四肢对称性痉挛性瘫痪,下肢重于上肢" } ] }, { "text": "如伴有脑室周围出血性梗死,常造成早产儿痉挛性偏瘫。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "脑室周围出血性梗死" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "痉挛性偏瘫" } ] }, { "text": "4.纠正凝血异常,可应用维生素K1等药物。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "维生素K1" } ] }, { "text": "5.曾有人提出对极低出生体重儿出生后常规使用苯巴比妥预防IVH,但经过多中心对照试验未被证实能降低IVH的发生率或严重性,目前尚未在早产儿推荐应用。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "IVH" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "IVH" } ] }, { "text": "【治疗】(一)维持正常脑灌注大量IVH时,由于动脉压降低和颅内压增高,脑灌流减少,因此必须维持血压在足够的水平,同时避免血压的过度波动和脑血流速度的突然升高,没有必要的过分积极治疗反而会加重已经存在的脑损伤。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "维持正常脑灌注" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "IVH" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "动脉压" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "颅内压" }, { "start_idx": 47, "end_idx": 48, "type": "ite", "entity": "血压" }, { "start_idx": 60, "end_idx": 61, "type": "ite", "entity": "血压" }, { "start_idx": 100, "end_idx": 102, "type": "dis", "entity": "脑损伤" } ] }, { "text": "(二)支持疗法维持正常通气,维持水、电解质和酸碱平衡,维持体温和代谢正常等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "支持疗法" }, { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "维持正常通气" }, { "start_idx": 14, "end_idx": 25, "type": "pro", "entity": "维持水、电解质和酸碱平衡" }, { "start_idx": 27, "end_idx": 35, "type": "pro", "entity": "维持体温和代谢正常" } ] }, { "text": "(三)预防出血后脑积水脑脊液中的血液和蛋白质可引起蛛网膜炎及粘连,导致出血后脑积水,可连续腰椎穿刺放出血性脑脊液,在病情稳定后,每天或隔天1次,每次放2~3ml/kg,但连续腰椎穿刺对预防出血后脑积水的价值还有争议。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "出血" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "脑积水" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "脑脊液" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "血液" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "蛋白质" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "蛛网膜炎" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "出血" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "脑积水" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 87, "end_idx": 90, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 97, "end_idx": 99, "type": "dis", "entity": "脑积水" } ] }, { "text": "用纤溶药物已被尝试预防出血后脑积水的发生,但需要进一步证实。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "纤溶药物" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "出血" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "脑积水" } ] }, { "text": "(四)出血后脑室扩张的处理急性期过后,应随访颅脑超声,评估脑室大小,随访间隔时间根据病程而定,病情越重,间隔时间越短,一般5~10天随访1次。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脑室" }, { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "出血后脑室扩张" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "颅脑超声" } ] }, { "text": "根据超声测定脑室扩张的进展速率和严重程度,进行相应处理。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "超声" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脑室" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "脑室扩张" } ] }, { "text": "对快速进展的脑室扩张(每周头围增长速率>1.5~2cm),由于脑室扩张迅速,可在短期内发生明显的颅内压增高,应当积极治疗。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脑室" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "脑室扩张" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "脑室" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "脑室扩张" }, { "start_idx": 48, "end_idx": 50, "type": "ite", "entity": "颅内压" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "颅内压增高" } ] }, { "text": "对缓慢进展的脑室扩张(<4周),主要是严密观察,改变体位(床头抬高30°)有助于颅内压的降低。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脑室" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "脑室扩张" }, { "start_idx": 40, "end_idx": 42, "type": "ite", "entity": "颅内压" } ] }, { "text": "因为有相当部分的患儿脑室扩张可自发停止,过早的干预不能改善其神经系统的远期预后。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "脑室" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "脑室扩张" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "神经系统" } ] }, { "text": "对持续缓慢进展的脑室扩张,应该进行干预,治疗措施包括连续腰穿和应用药物减少脑脊液产生。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "脑室" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "脑室扩张" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "腰穿" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "脑脊液" } ] }, { "text": "可用碳酸酐酶抑制剂乙酰唑胺(diamox)或渗透性药物甘油,乙酰唑胺剂量每天100mg/kg可使脑脊液产生减少50%,与呋塞米(每天1mg/kg)联合应用疗效更好,但碳酸酐酶抑制剂在神经胶质发育过程中可能有不良反应,不宜长期大剂量应用。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dru", "entity": "碳酸酐酶抑制剂" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "乙酰唑胺" }, { "start_idx": 14, "end_idx": 19, "type": "dru", "entity": "diamox" }, { "start_idx": 22, "end_idx": 28, "type": "dru", "entity": "渗透性药物甘油" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "乙酰唑胺" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "脑脊液" }, { "start_idx": 60, "end_idx": 62, "type": "dru", "entity": "呋塞米" }, { "start_idx": 83, "end_idx": 89, "type": "dru", "entity": "碳酸酐酶抑制剂" } ] }, { "text": "参考文献1.李毅.儿科疾病诊断标准.北京:科学出版社,2001:386-3862.夏经,陈文静.癫痫持续状态的综合治疗.中国实用儿科杂志,1999,14(1):8-83.NishiyamaI,OhtsukaY,TsudaT,etal.AepidemiologicalstudyofchildrenwithstatusepilepticusinOkayama,JChildNeurol,2007,22(1):21-214.MinicucciF,BelliniA,FanelliG,etal.Statusepilepticus.NeurolSci,2006,27(Suppl1):S52-S545.ToniaSabo-Graham,AlanR.Managementofstatusepilepticusinchildren.PediatricsinReview.1998,19(9):306-3066.DanielH,Lowenstein,BrianK,etal.Statusepilepticus(currentconcepts).TheNewEnglandJournalofMedicine.1998,(2):970-9707.NinaGraves,PharmD.PharmacoeconomicConsiderationsinTreatmentOptionsforAcuteSeizures.JournalofChildNeurology.1998,13(1):S27-S298.GaryR,WilliamR,Stevenlelyveld,etal.Pediatricemergencymedicine.Secondedition.USA.TheMcGraw-HillCompanies.2002", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dep", "entity": "儿科" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "癫痫" } ] }, { "text": "第七节化脓性脑膜炎细菌性脑膜炎(bacterialmeningitis),简称化脑,是儿童期最严重的感染之一,具有高发病率、高病死率、临床症状多不典型和严重并发症的特点。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "化脓性脑膜炎" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "细菌性脑膜炎" }, { "start_idx": 16, "end_idx": 34, "type": "dis", "entity": "bacterialmeningitis" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "化脑" } ] }, { "text": "【病因学】化脑在0~2月龄内婴儿,其致病病原常反映母亲的带菌情况和婴儿的生活环境,常见病原有B族链球菌和革兰阴性肠杆菌等,偶尔也有流感嗜血杆菌b型(Hib)或不定型菌株。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "化脑" }, { "start_idx": 46, "end_idx": 50, "type": "mic", "entity": "B族链球菌" }, { "start_idx": 52, "end_idx": 58, "type": "mic", "entity": "革兰阴性肠杆菌" }, { "start_idx": 65, "end_idx": 72, "type": "mic", "entity": "流感嗜血杆菌b型" }, { "start_idx": 74, "end_idx": 76, "type": "mic", "entity": "Hib" } ] }, { "text": "在2月龄至12岁的儿童组中,其致病菌常是肺炎链球菌、脑膜炎双球菌或Hib。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 26, "end_idx": 31, "type": "mic", "entity": "脑膜炎双球菌" }, { "start_idx": 33, "end_idx": 35, "type": "mic", "entity": "Hib" } ] }, { "text": "在美国,没有应用Hib疫苗之前,约70%小于5岁儿童的化脑是由Hib引起。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "Hib疫苗" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "化脑" }, { "start_idx": 31, "end_idx": 33, "type": "mic", "entity": "Hib" } ] }, { "text": "另外,在一些有解剖结构缺陷或免疫功能缺陷的人群,少见病原引起脑膜炎的病例增加,如铜绿假单胞菌、金黄色葡萄球菌、凝固酶阴性葡萄球菌、沙门菌属和李斯特菌等。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "解剖结构缺陷" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "免疫功能缺陷" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 40, "end_idx": 45, "type": "mic", "entity": "铜绿假单胞菌" }, { "start_idx": 47, "end_idx": 53, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 55, "end_idx": 63, "type": "mic", "entity": "凝固酶阴性葡萄球菌" }, { "start_idx": 65, "end_idx": 67, "type": "mic", "entity": "沙门菌" }, { "start_idx": 70, "end_idx": 73, "type": "mic", "entity": "李斯特菌" } ] }, { "text": "【流行病学及病原学】细菌性脑膜炎的重要危险因素:其一为年幼儿对感染的病原缺乏免疫力;其二为近期有致病细菌的携带。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "细菌性脑膜炎" } ] }, { "text": "(一)肺炎链球菌肺炎链球菌脑膜炎的发病率约为1/10万~3/10万,一生都可以感染此菌,2岁以下婴幼儿和老年人中的发病率最高。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "肺炎链球菌肺炎" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "链球菌脑膜炎" } ] }, { "text": "其危险性同感染的肺炎链球菌血清型有关,血清型分布在不同国家和地区也不相同。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "mic", "entity": "肺炎链球菌" } ] }, { "text": "(二)流感嗜血杆菌流感嗜血杆菌是广泛寄居在正常人上呼吸道的微生物,在健康儿童中,约30%~80%都带有Hi,绝大多数是无荚膜不定型(NTHI),无致病性的,仅少数为有荚膜菌株,而侵袭性疾病大多数为Hib菌株引起。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 9, "end_idx": 14, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 83, "end_idx": 86, "type": "mic", "entity": "荚膜菌株" }, { "start_idx": 98, "end_idx": 102, "type": "mic", "entity": "Hib菌株" } ] }, { "text": "b型流感嗜血杆菌(Hib)带菌的高峰年龄主要在6个月~2岁半,然后很快下降,4岁后很少带菌。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "mic", "entity": "b型流感嗜血杆菌" }, { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "Hib" } ] }, { "text": "Hib的传播方式主要由呼吸道经空气、飞沫或经手传染,主要感染5岁以下的儿童,引起多器官、组织的侵袭性感染,其中占第一位而且危害最大的是脑膜炎。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "Hib" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "呼吸道" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "手" }, { "start_idx": 40, "end_idx": 51, "type": "sym", "entity": "多器官、组织的侵袭性感染" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "在美国未用此菌苗前,5岁以下儿童Hi脑膜炎发病率60/10万,死亡率为5%~10%,而由于中枢神经损伤所造成的后遗症发生率为30%~50%。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "儿童Hi脑膜炎" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "中枢神经" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "中枢神经损伤" } ] }, { "text": "近年来人们发现,由于耐药菌株的出现,尽管使用了有效的抗生素,仍有5%的患者死亡,30%的患者有中枢神经系统后遗症。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "抗生素" }, { "start_idx": 47, "end_idx": 52, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "中枢神经系统后遗症" } ] }, { "text": "在我国,1943年樊培禄首次在成都报告了2例Hi脑膜炎,虽经磺胺治疗,但均未免于死亡。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "Hi脑膜炎" }, { "start_idx": 30, "end_idx": 31, "type": "dru", "entity": "磺胺" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "死亡" } ] }, { "text": "1955年黄达枢报告浙江省儿童保健院2年中24例Hi脑膜炎,占该院(1952~1953年)收治化脓性脑膜炎149例的16.1%。", "entities": [ { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "Hi脑膜炎" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "化脓性脑膜炎" } ] }, { "text": "同年,上海儿科医院100例化脑Hi占11%。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "化脑Hi" } ] }, { "text": "值得注意的是,此时报道的小儿细菌性脑膜炎中,原因不明者高达24%~38%。", "entities": [ { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "小儿细菌性脑膜炎" } ] }, { "text": "由于各种原因,以后的近30年国内几乎无Hi脑膜炎的文献可寻。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "Hi脑膜炎" } ] }, { "text": "1988年11月至1989年年底,北京儿童医院应用CIE荚膜多糖抗原检测方法,结合细菌培养,对北京儿童医院128例化脑患儿进行了病原学研究。", "entities": [ { "start_idx": 25, "end_idx": 35, "type": "pro", "entity": "CIE荚膜多糖抗原检测" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "化脑" } ] }, { "text": "临床诊断为化脑的患儿只有4例培养出Hib,而抗原检测有37例Hib阳性,占全部小儿化脓性脑膜炎的28.9%。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "化脑" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "Hib" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "抗原检测" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "Hib阳性" }, { "start_idx": 39, "end_idx": 46, "type": "dis", "entity": "小儿化脓性脑膜炎" } ] }, { "text": "在此基础上,采用相同的试验方法,我们于1990~1992年在合肥市(100万人口)组织13家医院15个科室进行了小儿化脑的病因学和流行病学监测。", "entities": [ { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "小儿化脑" } ] }, { "text": "所有患儿标本,细菌培养的阳性率仅13.3%,3种细菌抗原CIE监测的阳性率为90%。", "entities": [ { "start_idx": 24, "end_idx": 32, "type": "pro", "entity": "细菌抗原CIE监测" } ] }, { "text": "抗原检测发现,Hib占所有化脑的51.3%,其中58.8%为1岁以下,80.5%为5岁以下,5岁以下儿童Hib脑膜炎的发病率为10.4/10万。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "抗原检测" }, { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "Hib" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "化脑" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "儿童Hib脑膜炎" } ] }, { "text": "这一结果表明,尽管我国小儿Hib脑膜炎的发病率低于欧美国家菌苗使用前的发病率,它仍是我国小儿化脓性脑膜炎重要致病菌。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "小儿Hib脑膜炎" }, { "start_idx": 44, "end_idx": 51, "type": "dis", "entity": "小儿化脓性脑膜炎" } ] }, { "text": "(三)脑膜炎球菌性脑膜炎至今仍是全球性疾病,世界各地都有发病。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "脑膜炎球菌性脑膜炎" } ] }, { "text": "在非洲脑脊髓膜炎的流行,A群脑膜炎球菌仍是最常见的病原菌。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "脑脊髓膜炎" }, { "start_idx": 12, "end_idx": 18, "type": "mic", "entity": "A群脑膜炎球菌" } ] }, { "text": "此外,在巴西、马里、尼日利亚等地,C群脑膜炎球菌引起过大暴发。", "entities": [ { "start_idx": 17, "end_idx": 23, "type": "mic", "entity": "C群脑膜炎球菌" } ] }, { "text": "在智利、古巴、挪威等地,B群脑膜炎球菌也和一些暴发有联系,而且由这种血清群引起的病例最近几年在北美已明显增多了。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "mic", "entity": "B群脑膜炎球菌" } ] }, { "text": "【发病机制】细菌抵达脑膜可通过多种途径,如外伤或手术直接接种、淋巴或血流播散等。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "脑膜" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "外伤" }, { "start_idx": 24, "end_idx": 29, "type": "pro", "entity": "手术直接接种" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "淋巴" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "血流" } ] }, { "text": "通常脑膜炎是由菌血症发展而来。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "菌血症" } ] }, { "text": "细菌多由上呼吸道侵入,先在鼻咽部隐匿、繁殖,继而进入血流,直接抵达营养中枢神经系统的血管,或在该处形成局部血栓,并释放出细菌栓子到血液循环中。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "鼻咽部" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "血流" }, { "start_idx": 35, "end_idx": 43, "type": "bod", "entity": "中枢神经系统的血管" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "局部血栓" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "血液" } ] }, { "text": "由于小儿防御、免疫功能均较成人弱,病原菌容易通过血-脑屏障到达脑膜引起化脑。", "entities": [ { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "血" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "脑" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "脑膜" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "化脑" } ] }, { "text": "婴幼儿的皮肤、黏膜、肠胃道以及新生儿的脐部也常是感染侵入门户。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "皮肤" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "黏膜" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肠胃道" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "脐部" } ] }, { "text": "鼻窦炎、中耳炎、乳突炎既可作为病灶窝藏细菌,也可因病变扩展直接波及脑膜。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "中耳炎" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "乳突炎" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "脑膜" } ] }, { "text": "颅骨外伤、骨折的并发症,特别是那些涉及鼻窦的骨折,更可形成颅内与外界的直接通道,成为细菌侵入的门户。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅骨外伤" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "骨折" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "鼻窦的骨折" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "颅内" } ] }, { "text": "先天性免疫球蛋白缺陷,细胞免疫缺陷或联合免疫缺陷,均影响婴儿预防感染的能力,容易发生严重感染乃至脑膜炎。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "先天性免疫球蛋白缺陷" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "细胞免疫缺陷" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "联合免疫缺陷" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "严重感染" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "具有大量荚膜的细菌在血流中生存力加强,在缺乏免疫力的年幼儿中,血清低浓度的抗荚膜IgM与IgG抗体、血清备解素、血清补体成分如C19、C3和C5缺乏或减少,都影响对细菌有效的调理吞噬作用,使其容易发生脑膜炎。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "血流" }, { "start_idx": 100, "end_idx": 102, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "细菌通过血-脑屏障进入脑脊液循环,因为脑脊液中的补体、抗体浓度明显低于血液循环,细菌可迅速繁殖,而化学趋化因子、肿瘤坏死因子、白细胞介素-1、前列腺素E和其他细胞因子或炎性介质的局部产生引起了局部炎症,细菌的细胞壁成分也可引起强烈的炎症反应。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "血" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "脑" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "脑脊液" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "脑脊液" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "补体" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "抗体" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "血液" }, { "start_idx": 96, "end_idx": 99, "type": "sym", "entity": "局部炎症" }, { "start_idx": 113, "end_idx": 117, "type": "sym", "entity": "强烈的炎症" } ] }, { "text": "继而,炎症造成白细胞浸润、血管通透性增加、血管闭塞,破坏了血-脑屏障。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "炎症" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "白细胞" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "白细胞浸润" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "血管" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "血管通透性增加" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "血管" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "血管闭塞" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "血" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "脑" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "破坏了血-脑屏障" } ] }, { "text": "在脑脊液中已无菌生长时,细胞因子引起的炎症还在继续,这也就造成了慢性炎症后遗症。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "脑脊液" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "炎症" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "慢性炎症后遗症" } ] }, { "text": "【临床表现】各种细菌所致化脑的临床表现大致相仿,可归纳为感染、颅压增高及脑膜刺激症状。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "化脑" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "感染" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "颅压增高" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "脑膜刺激" } ] }, { "text": "近年来,化脑的症状越来越不典型,可能与抗生素的滥用有关。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "化脑" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "抗生素" } ] }, { "text": "严重者在24小时内即出现惊厥、昏迷。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "惊厥" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "昏迷" } ] }, { "text": "由于前囟尚未闭合,骨缝可以裂开,而使颅内压增高及脑膜刺激症状出现较晚,临床表现不典型。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "前囟" }, { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "前囟尚未闭合" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "骨缝" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "骨缝可以裂开" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "脑膜" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "脑膜刺激症状" } ] }, { "text": "前囟饱满、布氏征阳性是重要体征,有时皮肤划痕试验阳性。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "前囟" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "前囟饱满" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "布氏征阳性" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "皮肤划痕" }, { "start_idx": 18, "end_idx": 25, "type": "sym", "entity": "皮肤划痕试验阳性" } ] }, { "text": "由于宫内感染引起的,可表现为出生时即呈不可逆性休克或呼吸暂停,很快死亡。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "宫内感染" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "休克" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "不可逆性休克" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "死亡" } ] }, { "text": "发热或有或无,甚至体温不升。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "发热" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "体温不升" } ] }, { "text": "体格检查仅见前囟张力增高,脑膜刺激征。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "前囟" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "前囟张力增高" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "脑膜" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "脑膜刺激征" } ] }, { "text": "前囟隆起亦出现较晚,极易误诊。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "前囟" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "前囟隆起" } ] }, { "text": "唯有腰穿检查脑脊液才能确诊。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "腰穿检查" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "脑脊液" } ] }, { "text": "【实验室检查】(一)血象白细胞总数及中性粒细胞明显增加,感染严重者有核左移,胞质中可见中毒颗粒。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "ite", "entity": "白细胞总数" }, { "start_idx": 18, "end_idx": 22, "type": "ite", "entity": "中性粒细胞" }, { "start_idx": 12, "end_idx": 26, "type": "sym", "entity": "白细胞总数及中性粒细胞明显增加" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "核" }, { "start_idx": 34, "end_idx": 36, "type": "sym", "entity": "核左移" }, { "start_idx": 38, "end_idx": 46, "type": "sym", "entity": "胞质中可见中毒颗粒" } ] }, { "text": "小儿血清中CPR对急性化脑的诊断、治疗和判断预后转归均具有重要的帮助作用,CPR可以作为小儿急性化脑病情发展的重要标志物。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血清" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "急性化脑" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "小儿急性化脑" } ] }, { "text": "贫血常见于流感嗜血杆菌脑膜炎。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "贫血" }, { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "流感嗜血杆菌脑膜炎" } ] }, { "text": "(二)血培养早期、未用抗生素治疗者可得阳性结果,能帮助确定病原菌。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "抗生素治疗" } ] }, { "text": "(四)瘀点涂片流脑患儿皮肤瘀点涂片并革兰染色找革兰阴性双球菌,阳性率可高达80%以上。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "瘀点涂片" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "流脑" }, { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "皮肤瘀点涂片" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "革兰染色" }, { "start_idx": 23, "end_idx": 29, "type": "mic", "entity": "革兰阴性双球菌" } ] }, { "text": "(五)脑脊液常规、涂片、培养脑脊液检查是诊断化脑的必需手段,可见典型化脓性改变。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "脑脊液" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "涂片" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "化脑" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "化脓" } ] }, { "text": "其外观混浊或稀米汤样,压力增高(当脓液黏稠、流出困难时,无法测量压力)。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "sym", "entity": "外观混浊或稀米汤样" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "压力增高" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "脓液" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "脓液黏稠、流出困难" } ] }, { "text": "显微镜下检查白细胞计数甚多,每立方毫米自数百至数万,每升可达数亿个,其中以多核白细胞为主。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "显微镜" }, { "start_idx": 6, "end_idx": 8, "type": "ite", "entity": "白细胞" }, { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "白细胞计数甚多" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "多核白细胞" } ] }, { "text": "糖定量试验,含量常在150mg/L以下。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "糖定量试验" } ] }, { "text": "糖定量不但可协助鉴别细菌或病毒感染,还能反映治疗效果。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "病毒感染" } ] }, { "text": "蛋白定性试验多为强阳性,定量试验明显增高。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "蛋白定性试验" } ] }, { "text": "将脑脊液离心沉淀,作涂片染色,常能查见病原菌,可作为早期选用抗生素治疗的依据。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "pro", "entity": "脑脊液离心沉淀" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "涂片染色" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "抗生素治疗" } ] }, { "text": "涂片检查用革兰染色,必要时加用亚甲蓝染色协助观察细菌形态。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "涂片检查" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "革兰染色" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "亚甲蓝染色" } ] }, { "text": "(六)特异性细菌抗原测定利用免疫学技术检查患儿脑脊液、血、尿中细菌抗原为快速确定病原菌的特异方法。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "特异性细菌抗原测定" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "脑脊液" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "血" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "尿" } ] }, { "text": "特别是脑脊液抗原检测最重要。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "脑脊液抗原检测" } ] }, { "text": "血、尿抗原阳性亦有参考价值。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "血" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "尿" } ] }, { "text": "国外在十余年前即已广泛开展此项工作,由于缺乏优质抗血清,我国尚未普及使用。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血清" } ] }, { "text": "常用的方法有:1.对流免疫电泳(CIE)此法系以已知抗体(特定的抗血清)检测脑脊液中的抗原如可溶性荚膜多糖,特异性高,1小时内即能获得结果,常用做流脑快速诊断,也用以检查嗜血流感杆菌、肺炎链球菌等,阳性率可达70%~80%。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "对流免疫电泳" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "CIE" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "抗体" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "脑脊液" }, { "start_idx": 85, "end_idx": 90, "type": "mic", "entity": "嗜血流感杆菌" }, { "start_idx": 92, "end_idx": 96, "type": "mic", "entity": "肺炎链球菌" } ] }, { "text": "北京儿童医院128例化脑抗原检测阳性率为86.7%。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "化脑抗原检测" } ] }, { "text": "2.乳胶凝集试验(LA)系用已知抗体检测未知抗原(或用已知抗原检测抗体)。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "乳胶凝集试验" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "LA" } ] }, { "text": "对脑膜炎双球菌与流感杆菌检测结果与用CIE方法所测结果相似,但对肺炎链球菌敏感性较差。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "mic", "entity": "脑膜炎双球菌" }, { "start_idx": 8, "end_idx": 11, "type": "mic", "entity": "流感杆菌" }, { "start_idx": 18, "end_idx": 20, "type": "pro", "entity": "CIE" }, { "start_idx": 32, "end_idx": 36, "type": "mic", "entity": "肺炎链球菌" } ] }, { "text": "此法较CIE敏感,但有假阳性可能。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "CIE" } ] }, { "text": "所用标本量较CIE多,试剂盒亦较昂贵。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "CIE" } ] }, { "text": "3.免疫荧光试验用荧光素标记已知抗体,再加入待检抗原(如脑脊液、血液标本),然后用荧光显微镜观察抗原抗体反应。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "免疫荧光试验" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "脑脊液" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "血液" }, { "start_idx": 41, "end_idx": 45, "type": "equ", "entity": "荧光显微镜" } ] }, { "text": "4.酶联免疫吸附试验(enzymelinkedimmunosorbentassay,ELISA)用酶标记已知抗体(或抗原)测定相应抗原(或抗体)。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "酶联免疫吸附试验" }, { "start_idx": 11, "end_idx": 40, "type": "pro", "entity": "enzymelinkedimmunosorbentassay" }, { "start_idx": 42, "end_idx": 46, "type": "pro", "entity": "ELISA" } ] }, { "text": "(七)头颅CT或MRI检查用于化脑的目的不是直接提供诊断性的依据,其实用价值在于疾病早期用于鉴别诊断,疾病后期特别是出现视神经乳头水肿和神经局限性体征等并发症时协助确定原因。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "头颅CT" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "MRI检查" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "化脑" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "视神经乳头" }, { "start_idx": 60, "end_idx": 66, "type": "sym", "entity": "视神经乳头水肿" }, { "start_idx": 68, "end_idx": 74, "type": "sym", "entity": "神经局限性体征" } ] }, { "text": "【诊断】遇有以下情况应考虑有化脑可能:①患儿有呼吸道或其他感染如上呼吸道感染、肺炎、中耳炎、乳突炎、骨髓炎、蜂窝织炎或败血症,同时伴有神经系统症状;②有头皮、脊背中线的孔窦畸形、头颅创伤,同时伴有神经系统症状;③婴儿不明原因的持续发热,经一般治疗无效;④婴幼儿初次高热伴惊厥,而不能用一般高热惊厥解释者。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "化脑" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "呼吸道" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "肺炎" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "中耳炎" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "乳突炎" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "骨髓炎" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "蜂窝织炎" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "败血症" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "神经系统" }, { "start_idx": 67, "end_idx": 72, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 76, "end_idx": 87, "type": "dis", "entity": "头皮、脊背中线的孔窦畸形" }, { "start_idx": 89, "end_idx": 92, "type": "dis", "entity": "头颅创伤" }, { "start_idx": 98, "end_idx": 101, "type": "bod", "entity": "神经系统" }, { "start_idx": 98, "end_idx": 103, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 115, "end_idx": 116, "type": "sym", "entity": "发热" }, { "start_idx": 132, "end_idx": 133, "type": "sym", "entity": "高热" }, { "start_idx": 135, "end_idx": 136, "type": "sym", "entity": "惊厥" }, { "start_idx": 144, "end_idx": 147, "type": "sym", "entity": "高热惊厥" } ] }, { "text": "新生儿早期脑膜炎病原菌刚刚开始侵入脑膜,脑脊液变化可能不明显,高度怀疑时应隔1、2日后重复检查。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "mic", "entity": "脑膜炎病原菌" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "脑膜" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "脑脊液" } ] }, { "text": "通过年龄、季节等流行病学资料与临床经过虽能对致病菌作出初步推测,但迄今仍无明显的症状和体征(流脑患者的典型瘀斑除外)可作为病原菌特异性诊断的根据。", "entities": [ { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "流脑" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "瘀斑" } ] }, { "text": "进一步确诊必须依靠脑脊液涂片、细菌培养、对流免疫电泳等抗原检查法。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "脑脊液涂片" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "细菌培养" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "对流免疫电泳" } ] }, { "text": "约15%~20%患者其脑脊液呈典型化脑改变,但始终未能找到细菌的患儿应按病原未明的化脑治疗。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "脑脊液" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "化脑" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "化脑" } ] }, { "text": "【鉴别诊断】患儿早期脑脊液未发生典型改变,或由于曾接受抗生素治疗,致脑脊液改变不典型时,常须与以下几种疾病鉴别。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "脑脊液" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "脑脊液" } ] }, { "text": "(一)病毒性脑膜脑炎病毒性脑膜脑炎(viralmeningitis)起病一般较急,脑脊液外观微毛或轻度浑浊,白细胞计数10×106~1000×106/L,早期多核细胞稍增多但以后即以单核细胞为主,蛋白轻度增高,糖、氯化物正常。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "病毒性脑膜脑炎" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "病毒性脑膜脑炎" }, { "start_idx": 18, "end_idx": 32, "type": "dis", "entity": "viralmeningitis" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "脑脊液" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "微毛" }, { "start_idx": 41, "end_idx": 52, "type": "sym", "entity": "脑脊液外观微毛或轻度浑浊" }, { "start_idx": 54, "end_idx": 58, "type": "ite", "entity": "白细胞计数" }, { "start_idx": 91, "end_idx": 94, "type": "bod", "entity": "多核细胞" }, { "start_idx": 91, "end_idx": 97, "type": "sym", "entity": "多核细胞稍增多" }, { "start_idx": 103, "end_idx": 106, "type": "bod", "entity": "单核细胞" }, { "start_idx": 110, "end_idx": 111, "type": "bod", "entity": "蛋白" }, { "start_idx": 110, "end_idx": 115, "type": "sym", "entity": "蛋白轻度增高" }, { "start_idx": 117, "end_idx": 117, "type": "bod", "entity": "糖" }, { "start_idx": 119, "end_idx": 121, "type": "bod", "entity": "氯化物" }, { "start_idx": 117, "end_idx": 123, "type": "sym", "entity": "糖、氯化物正常" } ] }, { "text": "(二)结核性脑膜炎结核性脑膜炎(tuberculousmeningitis)起病多较缓慢,常有1~2周全身不适的前驱症状。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 16, "end_idx": 36, "type": "dis", "entity": "tuberculousmeningitis" } ] }, { "text": "也有急骤起病者,尤其是粟粒性结核的婴儿。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "粟粒性结核" } ] }, { "text": "典型结核性脑膜炎脑脊液外观毛玻璃样,有时因蛋白含量过高而呈黄色。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "脑脊液" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "脑脊液外观毛玻璃样" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "蛋白" }, { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "蛋白含量过高而呈黄色" } ] }, { "text": "白细胞计数为数百至千,偶尔超过1000×106/L,单核细胞70%~80%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "白细胞计数" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "白细胞计数为数百至千" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "单核细胞" } ] }, { "text": "当脑脊液改变不典型时,常易引起误诊。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "脑脊液" }, { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "脑脊液改变" } ] }, { "text": "应仔细询问患者有无结核接触史,检查身体其他部位是否存在结核病灶,进行结核菌素试验,在痰及胃液中寻找结核菌等以协助诊断。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "结核" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "结核病灶" }, { "start_idx": 34, "end_idx": 39, "type": "pro", "entity": "结核菌素试验" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "痰" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "胃液" }, { "start_idx": 49, "end_idx": 51, "type": "mic", "entity": "结核菌" } ] }, { "text": "必要时用脑脊液作结核菌培养及动物接种,但这两种方法都不能做到早期诊断。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "脑脊液" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "动物接种" } ] }, { "text": "对高度怀疑而一时不易确诊的患者,应给予抗结核药物以观察治疗反应。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "抗结核药物" } ] }, { "text": "(三)真菌性(新型隐球菌)脑膜炎其临床表现、病程及脑脊液改变与结核性脑膜炎相似,起病缓慢症状更为隐匿,病程更长,病情可起伏加重,颅压增高,脑膜刺激症状可不十分明显。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "真菌性(新型隐球菌)脑膜炎" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "脑脊液" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "脑脊液改变" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "颅压增高" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "脑膜" }, { "start_idx": 69, "end_idx": 74, "type": "sym", "entity": "脑膜刺激症状" } ] }, { "text": "确诊靠脑脊液印度墨汁染色见到厚荚膜的发亮圆形菌体,在沙门培养基上有新型隐球菌生长。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "脑脊液印度墨汁染色" }, { "start_idx": 14, "end_idx": 23, "type": "sym", "entity": "厚荚膜的发亮圆形菌体" }, { "start_idx": 35, "end_idx": 37, "type": "mic", "entity": "隐球菌" } ] }, { "text": "【治疗原则】(一)抗菌治疗化脑预后好坏与是否早期明确病原菌,选择恰当的抗生素进行治疗密切相关。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "化脑" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "抗生素" } ] }, { "text": "经脑脊液确诊后,应尽快由静脉给予适当、足量的抗生素,以杀菌药物为佳,并根据病情按计划完成全部疗程。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "脑脊" }, { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "脑脊" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "应尽" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "、足量" } ] }, { "text": "治疗效果满意时,体温多于3天左右下降,症状减轻,脑脊液细菌消失,细胞数明显减少,其他生化指标亦有相应好转,此时可继用原来药物治疗,2周后再复查脑脊液。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "脑脊液" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "脑脊液细菌消失" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "细胞数明显减少" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "脑脊液" } ] }, { "text": "关于抗生素选择、使用方法应注意以下几点:①患儿年龄对抗生素选择有一定的指导意义:如年长儿童患流感杆菌脑膜炎较少;新生儿化脑大多数是肠道革兰阴性杆菌或链球菌所致,故应选择对这些细菌有效的药物;②保证药物在脑脊液中达到有效浓度:首先应选用易于透过血-脑屏障的药物,使脑脊液中抗生素浓度超过抑菌浓度10倍以上;③鞘内注射:如果选用的药物能很好通过血-脑屏障,原则上不需鞘内注射,以免出现不良反应及增加病儿痛苦;④脑室内注药:由于存在血-脑屏障及脑脊液单向循环,对并发脑室膜炎患儿,有人主张脑室注药以提高疗效。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "抗生素" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "抗生素" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "流感杆菌脑膜炎" }, { "start_idx": 65, "end_idx": 72, "type": "mic", "entity": "肠道革兰阴性杆菌" }, { "start_idx": 74, "end_idx": 76, "type": "mic", "entity": "链球菌" }, { "start_idx": 101, "end_idx": 103, "type": "bod", "entity": "脑脊液" }, { "start_idx": 121, "end_idx": 121, "type": "bod", "entity": "血" }, { "start_idx": 123, "end_idx": 123, "type": "bod", "entity": "脑" }, { "start_idx": 131, "end_idx": 133, "type": "bod", "entity": "脑脊液" }, { "start_idx": 135, "end_idx": 137, "type": "dru", "entity": "抗生素" }, { "start_idx": 153, "end_idx": 156, "type": "pro", "entity": "鞘内注射" }, { "start_idx": 170, "end_idx": 170, "type": "bod", "entity": "血" }, { "start_idx": 172, "end_idx": 172, "type": "bod", "entity": "脑" }, { "start_idx": 181, "end_idx": 184, "type": "pro", "entity": "鞘内注射" }, { "start_idx": 203, "end_idx": 207, "type": "pro", "entity": "脑室内注药" }, { "start_idx": 213, "end_idx": 213, "type": "bod", "entity": "血" }, { "start_idx": 215, "end_idx": 215, "type": "bod", "entity": "脑" }, { "start_idx": 219, "end_idx": 221, "type": "bod", "entity": "脑脊液" }, { "start_idx": 230, "end_idx": 233, "type": "dis", "entity": "脑室膜炎" }, { "start_idx": 241, "end_idx": 244, "type": "pro", "entity": "脑室注药" } ] }, { "text": "对革兰阴性杆菌和金黄色葡萄球菌至少用抗生素治疗3周。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 8, "end_idx": 14, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "抗生素" } ] }, { "text": "【并发症】(一)硬脑膜下积液常见于1岁以下的肺炎链球菌及流感杆菌脑膜炎患儿,但亦可见于流脑和其他细菌引起的化脑。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "硬脑膜下积液" }, { "start_idx": 22, "end_idx": 26, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 28, "end_idx": 34, "type": "mic", "entity": "流感杆菌脑膜炎" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "流脑" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "化脑" } ] }, { "text": "它可发生在化脑同时或症状出现数小时或数日之后,多发生在病后7~10天。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "化脑" } ] }, { "text": "当化脑患儿经恰当治疗不见好转,或在治疗过程中临床和脑脊液已逐渐好转,但忽又出现发热或烦躁、意识障碍、喷射性呕吐、惊厥等颅内压增高症状。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "脑脊液" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "发热" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "烦躁" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "意识障碍" }, { "start_idx": 50, "end_idx": 54, "type": "sym", "entity": "喷射性呕吐" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "惊厥" }, { "start_idx": 59, "end_idx": 63, "type": "sym", "entity": "颅内压增高" } ] }, { "text": "进一步做颅骨透照试验、头颅B超和CT证实。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "颅骨透照" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "头颅B超" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "CT证实" } ] }, { "text": "病情较重者可有硬脑膜下积脓,有时涂片或培养发现病原菌。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "硬脑膜下积脓" } ] }, { "text": "与脑积水相反硬脑膜下积液可发生于及时得到正确治疗的患者。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "脑积水" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "硬脑膜下积液" } ] }, { "text": "硬脑膜下积液的处理:若积液量不多,大多能在1~2个月自行吸收,若积液量多,同时有颅内压增高症状或硬脑膜下积脓时,均应穿刺放液。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "硬脑膜下积液" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 48, "end_idx": 53, "type": "sym", "entity": "硬脑膜下积脓" }, { "start_idx": 58, "end_idx": 61, "type": "pro", "entity": "穿刺放液" } ] }, { "text": "(二)颅内压增高症急性弥漫性脑水肿导致颅内压增高为常见并发症,如程度严重,进展急速,则可发生颞叶钩回疝或枕骨大孔疝。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "颅内压增高症" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "急性弥漫性脑水肿" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "颞叶钩回疝" }, { "start_idx": 52, "end_idx": 56, "type": "sym", "entity": "枕骨大孔疝" } ] }, { "text": "对此认识不足,未及早采用脱水疗法及时抢救,可以危及生命。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "脱水疗法" } ] }, { "text": "(三)脑室膜炎脑室膜炎亦是比较常见的并发症,是造成预后不良和严重后遗症的重要原因。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "脑室膜炎" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "脑室膜炎" } ] }, { "text": "革兰阴性杆菌所致者尤其多。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "mic", "entity": "革兰阴性杆菌" } ] }, { "text": "感染途径系经过血行播散,脉络膜裂隙蔓延或经脑脊液逆行扩散。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "脑脊液" } ] }, { "text": "治疗脑室膜炎除全身用药外,需向脑室内注入相应抗生素。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "脑室膜炎" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "脑室" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "抗生素" } ] }, { "text": "(四)脑积水患脑膜炎时,脓性渗出物易堵塞狭小孔道或发生粘连而引起脑脊液循环障碍,产生脑积水。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脑积水" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "脓性渗出物" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "脑脊液" }, { "start_idx": 12, "end_idx": 38, "type": "sym", "entity": "脓性渗出物易堵塞狭小孔道或发生粘连而引起脑脊液循环障碍" }, { "start_idx": 42, "end_idx": 44, "type": "sym", "entity": "脑积水" } ] }, { "text": "最突出的症状是头颅迅速扩大,骨缝分离,前囟扩大、饱满,头皮静脉扩张,颅部叩诊有破壶音。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "头颅" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "头颅迅速扩大" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "骨缝分离" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "前囟" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "前囟扩大、饱满" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "头皮" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "头皮静脉扩张" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "颅部" }, { "start_idx": 34, "end_idx": 41, "type": "sym", "entity": "颅部叩诊有破壶音" } ] }, { "text": "脑积液治疗分内科和外科两种。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑积液" } ] }, { "text": "(五)脑性低钠血症化脑患儿除因呕吐不进饮食等原因可引起水、电解质紊乱外,还可见脑性低钠血症,出现昏睡、惊厥、昏迷、全身软弱无力、四肢肌张力低下、尿少症状。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "脑性低钠血症" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "化脑" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "呕吐" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "水、电解质紊乱" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "脑性低钠血症" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "昏睡" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "惊厥" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "昏迷" }, { "start_idx": 57, "end_idx": 62, "type": "sym", "entity": "全身软弱无力" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "四肢" }, { "start_idx": 64, "end_idx": 70, "type": "sym", "entity": "四肢肌张力低下" }, { "start_idx": 72, "end_idx": 72, "type": "bod", "entity": "尿" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "尿少" } ] }, { "text": "其发生原理与感染影响脑神经垂体,抗利尿激素分泌过多导致水潴留有关。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "脑神经垂体" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "抗利尿激素" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "抗利尿激素分泌过多" }, { "start_idx": 27, "end_idx": 29, "type": "sym", "entity": "水潴留" } ] }, { "text": "(六)其他由于脑实质损害及粘连可使脑神经受累或出现肢体瘫痪,亦可发生脑脓肿、颅内动脉炎及继发性癫痫发作。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "脑实质损害及粘连" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "脑神经" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "脑神经受累" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肢体" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "肢体瘫痪" }, { "start_idx": 34, "end_idx": 36, "type": "sym", "entity": "脑脓肿" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "颅内动脉炎" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "继发性癫痫" } ] }, { "text": "此外,中耳炎、肺炎和关节炎也偶可发生。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "中耳炎" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "肺炎" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "关节炎" } ] }, { "text": "【预后】与化脑预后有关的因素是:患儿年龄、感染细菌种类、病情轻重,治疗早晚,有无并发症及细菌对抗生素的敏感性等。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "化脑" } ] }, { "text": "新生儿化脑病死率可达65%~75%,特别是宫内感染肠道细菌预后极差。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "新生儿化脑" }, { "start_idx": 25, "end_idx": 28, "type": "mic", "entity": "肠道细菌" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "宫内感染肠道细菌" } ] }, { "text": "金黄色葡萄球菌及肠道细菌引起者由于细菌耐药,治疗困难,病死率亦高。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 8, "end_idx": 11, "type": "mic", "entity": "肠道细菌" } ] }, { "text": "流感嗜血杆菌脑膜炎的病死率约10%~15%。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "流感嗜血杆菌脑膜炎" } ] }, { "text": "如多动性障碍、轻或重度精神发育迟缓、反复抽搐发作、语言发育障碍、脑积水、运动功能障碍、视力障碍和偏瘫等。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "多动性障碍" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "轻或重度精神发育迟缓" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "反复抽搐" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "语言发育障碍" }, { "start_idx": 32, "end_idx": 34, "type": "sym", "entity": "脑积水" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "运动功能障碍" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "视力障碍和偏瘫" } ] }, { "text": "听力障碍发生率高,脑膜炎球菌、流感嗜血杆菌、肺炎链球菌所致后遗症分别占5.4%、2.7%、7.8%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "听力障碍" }, { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "脑膜炎球菌" }, { "start_idx": 15, "end_idx": 20, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 22, "end_idx": 26, "type": "mic", "entity": "肺炎链球菌" } ] }, { "text": "二、儿童肿瘤的发病率世界各地各类肿瘤的发病率不完全相同,有些肿瘤有明显的地域差异,(表11-11)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "肿瘤" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "肿瘤" } ] }, { "text": "据报告尼日利亚的伊巴丹,儿童肿瘤年发病率最高为155.6/百万,斐济的印第安人发病率仅为39.7/百万。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "我国上海市有较完整的肿瘤登记系统,上海市儿童肿瘤的发病率(表11-11)。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "肿瘤" } ] }, { "text": "表11-1常见儿童肿瘤在不同国家地区的发病率差异如下表11-2上海市1972~1978年和1986~1992年0~15岁儿童肿瘤新病例数和标化发病率(1/百万)续表", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "第二章泌尿系统疾病的诊治技术第一节尿液检查血液经肾小球滤过膜滤过后形成的原尿,再经过肾小管选择性的吸收与排泌,最终通过输尿管、膀胱以及尿道而排出体外即成为尿液。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "泌尿系统疾病" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "尿液检查" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "血液" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "肾小球滤过膜" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "原尿" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "肾小管" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "输尿管" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "膀胱" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "尿道" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "尿液" } ] }, { "text": "因此,尿液中除含有大量的水分外,还含有蛋白质和核酸代谢的中间和终末产物(尿素、尿酸、肌酐和氨基酸)、各种有机酸盐类和无机盐类、激素以及酶等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "尿液" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "蛋白质" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "核酸" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "尿素" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "尿酸" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "肌酐" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "氨基酸" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "有机酸盐类" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "无机盐类" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "激素" }, { "start_idx": 67, "end_idx": 67, "type": "bod", "entity": "酶" } ] }, { "text": "还有一些正常或病理的有形成分,如细胞、细菌、寄生虫以及结晶等。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "细胞" }, { "start_idx": 19, "end_idx": 20, "type": "mic", "entity": "细菌" }, { "start_idx": 22, "end_idx": 24, "type": "mic", "entity": "寄生虫" } ] }, { "text": "因此,尿液是反映整个泌尿系统病变和功能状态的窗口,检查分析尿液可为相应的疾病提供诊断依据。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "尿液" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "泌尿系统病变" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "检查分析尿液" } ] }, { "text": "尿液检查分析的内容包括:尿液的一般性状检查、尿生化分析、尿沉渣分析和尿细菌学检查。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "尿液检查分析" }, { "start_idx": 12, "end_idx": 20, "type": "pro", "entity": "尿液的一般性状检查" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "尿生化分析" }, { "start_idx": 28, "end_idx": 32, "type": "pro", "entity": "尿沉渣分析" }, { "start_idx": 34, "end_idx": 39, "type": "pro", "entity": "尿细菌学检查" } ] }, { "text": "一、尿液的一般性状检查(一)尿量小儿24小时尿量变化较大,不仅与肾血流量、肾小球滤过率以及肾小管回吸收率相关,还受摄入液体量、活动量、出汗以及环境温度的影响。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "尿液的一般性状检查" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "尿量" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "尿量" }, { "start_idx": 32, "end_idx": 35, "type": "ite", "entity": "肾血流量" }, { "start_idx": 37, "end_idx": 42, "type": "ite", "entity": "肾小球滤过率" }, { "start_idx": 45, "end_idx": 51, "type": "ite", "entity": "肾小管回吸收率" }, { "start_idx": 68, "end_idx": 68, "type": "bod", "entity": "汗" } ] }, { "text": "新生儿每小时尿量约1~3ml/kg,每日尿量婴儿为400~500ml,幼儿500~600ml,学龄前期600~800ml,学龄期800~1400ml。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "尿量" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "尿量" } ] }, { "text": "(二)颜色正常尿液透明,呈淡黄色或黄色,主要是由于含有代谢产物尿色素(urochrome)、尿胆素(urobilin)和尿红质(uroerythrin)等有色物质。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "尿液" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "尿色素" }, { "start_idx": 35, "end_idx": 43, "type": "bod", "entity": "urochrome" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "尿胆素" }, { "start_idx": 50, "end_idx": 57, "type": "bod", "entity": "urobilin" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "尿红质" }, { "start_idx": 64, "end_idx": 74, "type": "bod", "entity": "uroerythrin" } ] }, { "text": "在疾病状态或特殊生理状态下,可出现尿液颜色的改变。", "entities": [ { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "尿液颜色的改变" } ] }, { "text": "红色尿最常见的是血尿,其次为血红蛋白尿和肌红蛋白尿,卟啉尿(porphyrinuria)外观如红葡萄酒样,见于先天性卟啉代谢异常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "红色尿" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "血尿" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "血红蛋白尿" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "肌红蛋白尿" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "卟啉尿" }, { "start_idx": 30, "end_idx": 42, "type": "sym", "entity": "porphyrinuria" }, { "start_idx": 44, "end_idx": 51, "type": "sym", "entity": "外观如红葡萄酒样" }, { "start_idx": 55, "end_idx": 63, "type": "dis", "entity": "先天性卟啉代谢异常" } ] }, { "text": "黄褐色尿见于黄疸病人以及服用含动物胆红素的药物后如牛黄解毒丸和熊胆粉等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "黄褐色尿" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "黄疸" }, { "start_idx": 25, "end_idx": 29, "type": "dru", "entity": "牛黄解毒丸" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "熊胆粉" } ] }, { "text": "淡绿色尿见于铜绿假单孢菌感染或使用亚甲蓝和阿米替林等药物后。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "淡绿色尿" }, { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "铜绿假单孢菌感染" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "亚甲蓝" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "阿米替林" } ] }, { "text": "乳白色尿常是由于脓尿、乳糜尿(chyluria)、磷酸盐或碳酸盐结晶所致。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "乳白色尿" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "脓尿" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "乳糜尿" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "chyluria" } ] }, { "text": "蓝色尿常见于由于色氨基吸收障碍,尿中排出大量尿蓝母的蓝尿布综合征。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "蓝色尿" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "色氨基吸收障碍" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "尿" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "尿蓝母" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "蓝尿布综合征" } ] }, { "text": "黑褐色尿除见于重症血尿和变性血红蛋白尿外,还见于胱氨酸病、酚中毒以及黑尿酸症等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "黑褐色尿" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "重症血尿" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "变性血红蛋白尿" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "胱氨酸病" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "酚中毒" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "黑尿酸症" } ] }, { "text": "(三)气味正常尿一般无味,但可受食物及代谢排泄物的影响而改变,如大蒜味。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "尿" } ] }, { "text": "细菌感染常使尿素转变为氨的气味。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "细菌感染" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "尿素" } ] }, { "text": "某些代谢病可产生特殊气味,如苯丙酮酸尿时尿有鼠尿样臭味,枫糖尿症时尿有甜味,糖尿病酮症酸中毒时,尿液呈烂苹果味。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "代谢病" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "苯丙酮酸尿" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "尿有鼠尿样臭味" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "枫糖尿症" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "尿有甜味" }, { "start_idx": 38, "end_idx": 45, "type": "dis", "entity": "糖尿病酮症酸中毒" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "尿液呈烂苹果味" } ] }, { "text": "服用某些特别的食物或药物后尿液也可出现特殊气味。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "尿液" } ] }, { "text": "(四)尿比重和尿渗透压尿比重表示尿中全部粒子的重量总和,是简单测定肾浓缩功能的指标。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "尿比重" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "尿渗透压" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "尿比重" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "尿" }, { "start_idx": 31, "end_idx": 37, "type": "pro", "entity": "测定肾浓缩功能" } ] }, { "text": "大多数情况下渗透压可根据尿比重以下列公式推算:尿渗透浓度(压)=[(尿比重-1000)×4000]mOsm/(kg•H2O)新生儿尿比重为1.006~1.008,尿渗透压为240mOsm/(kg•H2O),儿童尿比重范围为1.003~1.030间,渗透压在40~1400mOsm/(kg•H2O)范围内波动。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "ite", "entity": "渗透压" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "尿比重" }, { "start_idx": 23, "end_idx": 27, "type": "ite", "entity": "尿渗透浓度" }, { "start_idx": 34, "end_idx": 36, "type": "ite", "entity": "尿比重" }, { "start_idx": 71, "end_idx": 73, "type": "ite", "entity": "尿比重" }, { "start_idx": 87, "end_idx": 90, "type": "ite", "entity": "尿渗透压" }, { "start_idx": 117, "end_idx": 119, "type": "ite", "entity": "尿比重" }, { "start_idx": 136, "end_idx": 138, "type": "ite", "entity": "渗透压" } ] }, { "text": "当有大量的高分子量的粒子存在,如尿糖、尿蛋白及造影剂时尿比重明显升高,应予以矫正。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "尿糖" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "尿蛋白" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "造影剂" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "尿比重" } ] }, { "text": "尿液每100ml中含1g糖或蛋白时,可将所测得的比重减去0.003。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "尿液" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "糖" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "蛋白" } ] }, { "text": "低张尿比重<1.010,尿渗透压小于血浆渗透压,常≤200mOsm/(kg•H2O);高张尿比重>1.020,尿渗透压常>800mOsm/(kg•H2O);等张尿比重为1.010左右,尿渗透压等于血浆渗透透压[400mOsm/(kg•H2O)]。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "低张尿比重" }, { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "尿渗透压" }, { "start_idx": 18, "end_idx": 22, "type": "ite", "entity": "血浆渗透压" }, { "start_idx": 49, "end_idx": 53, "type": "ite", "entity": "高张尿比重" }, { "start_idx": 61, "end_idx": 64, "type": "ite", "entity": "尿渗透压" }, { "start_idx": 90, "end_idx": 94, "type": "ite", "entity": "等张尿比重" }, { "start_idx": 104, "end_idx": 107, "type": "ite", "entity": "尿渗透压" }, { "start_idx": 110, "end_idx": 115, "type": "ite", "entity": "血浆渗透透压" } ] }, { "text": "固定低张尿见于肾小管浓缩功能障碍、尿崩症、低钾血症及高钙血症所致肾小管功能受损等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "固定低张尿" }, { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "肾小管浓缩功能障碍" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "尿崩症" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "低钾血症" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "高钙血症" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "肾小管功能受损" } ] }, { "text": "等张尿见于急、慢性肾衰竭。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "等张尿" }, { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "急、慢性肾衰竭" } ] }, { "text": "高张尿见于脱水和糖尿病等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "高张尿" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "脱水" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "糖尿病" } ] }, { "text": "(五)酸碱度(pH)尿pH反映尿酸化的程度,随全身酸碱平衡而变化。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "ite", "entity": "酸碱度(pH)" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "尿pH" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "尿" } ] }, { "text": "正常尿一般为弱酸性(5.0~6.5),变动范围为4.5~7.5。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "尿" } ] }, { "text": "常用试纸法测定。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "试纸法" } ] }, { "text": "需准确测定pH时必须用pH计。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "pH" }, { "start_idx": 11, "end_idx": 13, "type": "equ", "entity": "pH计" } ] }, { "text": "pH>7.0~7.5提示产生尿素酶的奇异变形杆菌引起的泌尿系感染,因其可产生氨而使尿pH升高。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "pH" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "尿素酶" }, { "start_idx": 18, "end_idx": 31, "type": "dis", "entity": "奇异变形杆菌引起的泌尿系感染" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "尿pH" } ] }, { "text": "远端肾小管酸中毒时因尿酸化障碍,尿pH不能下降至6.0以下。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "远端肾小管酸中毒" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "尿酸化障碍" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "尿pH" } ] }, { "text": "参考文献1.殷雷,盛芳芸,曾纪骅,等.99mTc-DMSA肾静态显像对上尿路感染诊断的评价.中华儿科杂志,1999,36(10):610-6132.葛琳娟.小儿膀胱输尿管返流的诊治进展.中华小儿外科杂志,1998,19(5):307-3073.黄澄如,梁若馨,白继武,等.小儿原发性膀胱输尿管反流的诊断与手术治疗.中华泌尿外科杂志,2001,22(11):674-6774.MajdM,RushtonHG.RenalCorticalScintigraphyinthediagnosisofacutepyelonephritis.SeminNuclMed.1992,22(2):98-985.StanleyH.Urinarytractinfection’soldandnewconcepts.PediatrNephrol,1995,42(5):1433-14336.CormuC,CochatP.ColletJP,etal.Surveyoftheattituesmanagementofacutepyelonephritisinchildren.PediatrNephrol,1994,8(3):275-2757.BiggiA,DardanelliL,PomeroGetal.Acuterenalscintigraphyinchildrenwithafirsturinarytractinfection.PediatrNephrol,2001,16(9):733-7338.FunfstuekR,StraubeE,SchildbachO,etal.PreventionofreinfectionbyL-methionineinpatientswithrecurrenturinarytractinfection.MedKlin,1997,92(10):574-5749.NayirA,EmreS,SirinA,etal.Vaccinationwithinactivateduropathogenicbacteriainchildhoodrecurrenturinarytractinfection.KidneyInt,1995,48(3):1677-1677", "entities": [ { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "尿路感染" }, { "start_idx": 48, "end_idx": 49, "type": "dep", "entity": "儿科" }, { "start_idx": 78, "end_idx": 86, "type": "dis", "entity": "小儿膀胱输尿管返流" }, { "start_idx": 95, "end_idx": 98, "type": "dep", "entity": "小儿外科" }, { "start_idx": 136, "end_idx": 147, "type": "dis", "entity": "小儿原发性膀胱输尿管反流" }, { "start_idx": 159, "end_idx": 162, "type": "dep", "entity": "泌尿外科" } ] }, { "text": "第五节支气管扩张症支气管扩张症(bronchiectasis)以亚段支气管持续扩张为特征,伴支气管壁及支气管周围组织的炎症性破坏和管腔内渗出物积聚。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "支气管扩张症" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "支气管扩张症" }, { "start_idx": 17, "end_idx": 30, "type": "dis", "entity": "bronchiectasis" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "亚段支气管持续扩张" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "支气管壁" }, { "start_idx": 52, "end_idx": 58, "type": "bod", "entity": "支气管周围组织" }, { "start_idx": 66, "end_idx": 73, "type": "sym", "entity": "管腔内渗出物积聚" } ] }, { "text": "百日咳患儿可伴支气管扩张,但数个月后常可恢复正常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "百日咳" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "支气管扩张" } ] }, { "text": "国外报道囊性纤维化和HIV感染是儿童支气管扩张的重要原因。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "囊性纤维化" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "HIV感染" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "支气管扩张" } ] }, { "text": "其他因素包括异物吸入、支气管淋巴结结核、哮喘、肿瘤、各种原因引起的慢性肺炎等。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "异物吸入" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "支气管淋巴结结核" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "哮喘" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "肿瘤" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "慢性肺炎" } ] }, { "text": "易患反复下呼吸道感染,尤其在同一部位反复发生肺炎,甚至肺脓疡。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "下呼吸道感染" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "肺炎" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "肺脓疡" } ] }, { "text": "病程久者多有不同程度咯血、贫血、营养不良等。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "咯血" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "贫血" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "营养不良" } ] }, { "text": "肺部检查可在肺底部闻及水泡音或哮鸣音。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "肺部检查" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肺底部" }, { "start_idx": 6, "end_idx": 17, "type": "sym", "entity": "肺底部闻及水泡音或哮鸣音" } ] }, { "text": "如病变广泛,常因肺不张或纤维性病变致纵隔移向患侧。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "肺不张" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "纤维性病变" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "纵隔" } ] }, { "text": "支气管造影显示支气管呈柱状、梭状或囊状扩张。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "支气管造影" }, { "start_idx": 7, "end_idx": 20, "type": "sym", "entity": "支气管呈柱状、梭状或囊状扩张" } ] }, { "text": "当扩大的支气管走行与CT扫描层面平行时,即表现为“轨道征”;相垂直时,即构成“印戒征”。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "支气管" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "CT" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "轨道征" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "印戒征" } ] }, { "text": "清除分泌物后雾化吸入抗生素可能有益,但过长疗程也会增加耐药性。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "抗生素" } ] }, { "text": "应每年接种流感疫苗。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "接种" }, { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "流感疫苗" } ] }, { "text": "如病变限于一肺段或肺叶,经上述治疗病情无改善甚至恶化者,可考虑肺段或肺叶切除。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肺段" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肺叶" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肺段" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肺叶" } ] }, { "text": "三、内镜下逆行胰胆管造影术(ERCP)利用内镜操作技术,进行X线造影是检查胰腺及胆道疾病的重要手段。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "内镜下逆行胰胆管造影术" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "ERCP" }, { "start_idx": 21, "end_idx": 22, "type": "equ", "entity": "内镜" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "X线造影" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "胰腺" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "胆道疾病" } ] }, { "text": "ERCP是在电子十二指肠镜下由口腔经十二指肠乳头插管注入照影剂,从而逆行显示胰胆管的造影技术,是目前国际公认的胰胆管疾病诊断金标准,是确诊胆管结石的最佳方法。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "ERCP" }, { "start_idx": 6, "end_idx": 12, "type": "pro", "entity": "电子十二指肠镜" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "口腔" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "十二指肠乳头" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "照影剂" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "胰胆管" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "胰胆管疾病" }, { "start_idx": 69, "end_idx": 72, "type": "dis", "entity": "胆管结石" } ] }, { "text": "在ERCP基础上,可以同时进行Oddis括约肌切开术、胆管结石碎石取石术、胆总管支架植入术、内镜下鼻胆汁引流术以及内镜下碎石取石术等微创手术,对胆管结石等疾病能进行快速、安全、有效的治疗,是一种典型的、成功率极高的微创手术,代表了现代微创手术理念的发展趋向,已成为众多胆胰疾病首选的诊疗方法。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "ERCP" }, { "start_idx": 15, "end_idx": 25, "type": "pro", "entity": "Oddis括约肌切开术" }, { "start_idx": 27, "end_idx": 35, "type": "pro", "entity": "胆管结石碎石取石术" }, { "start_idx": 37, "end_idx": 44, "type": "pro", "entity": "胆总管支架植入术" }, { "start_idx": 46, "end_idx": 54, "type": "pro", "entity": "内镜下鼻胆汁引流术" }, { "start_idx": 57, "end_idx": 64, "type": "pro", "entity": "内镜下碎石取石术" }, { "start_idx": 66, "end_idx": 69, "type": "pro", "entity": "微创手术" }, { "start_idx": 72, "end_idx": 75, "type": "dis", "entity": "胆管结石" }, { "start_idx": 134, "end_idx": 137, "type": "dis", "entity": "胆胰疾病" } ] }, { "text": "目前用于先天性与后天性胆道及胰腺疾患的诊断。", "entities": [ { "start_idx": 4, "end_idx": 17, "type": "dis", "entity": "先天性与后天性胆道及胰腺疾患" } ] }, { "text": "适应证:凡疑似胰、胆疾病或属于胰、胆疾病皆为适应证,一般多在B超后进行,尤其对胆道疾病诊断价值最高。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "胰" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "胆疾病" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "胰" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "胆疾病" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "B超" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "胆道疾病" } ] }, { "text": "禁忌证:除同胃镜检查禁忌证外,上消化道梗阻、胰腺炎急性发作期及胆道感染伴发热者均为禁忌。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "胃镜检查" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "上消化道梗阻" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "胆道感染伴发热" } ] }, { "text": "碘过敏者慎用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "碘过敏" } ] }, { "text": "ERCP的并发症:急性药物性胰腺炎,急性胆管炎,造影剂后腹膜炎,其发生率国内报道0.57%~3.11%。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "急性药物性胰腺炎" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "急性胆管炎" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "造影剂后腹膜炎" } ] }, { "text": "儿童发生率高于成人。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "儿童发生率高于成人" } ] }, { "text": "二、经皮球囊瓣膜成形术经皮球囊瓣膜成形术20世纪80年代起应用于临床,最常应用的为肺动脉瓣狭窄扩张术(PBPV)。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "经皮球囊瓣膜成形术" }, { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "经皮球囊瓣膜成形术" }, { "start_idx": 41, "end_idx": 49, "type": "pro", "entity": "肺动脉瓣狭窄扩张术" }, { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "PBPV" } ] }, { "text": "目前已对PBPV术进行了系统研究,包括应用指征、方法学、效果及存在的问题。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "PBPV术" } ] }, { "text": "主动脉瓣狭窄球囊扩张术(PBAV)国内仅少数心血管中心开展,这一技术的开展,有助于与外科根治手术(Ross)内外科联合治疗。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "pro", "entity": "主动脉瓣狭窄球囊扩张术" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "PBAV" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "心血管" }, { "start_idx": 42, "end_idx": 47, "type": "pro", "entity": "外科根治手术" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "Ross" }, { "start_idx": 54, "end_idx": 56, "type": "dep", "entity": "内外科" } ] }, { "text": "(一)经皮球囊肺动脉瓣成形术近30年来,通过对PBPV的适应证、方法学、术前后血流动力学、作用机制及随访等进行深入综合研究,表明PBPV为简便、有效、安全、经济的治疗典型肺动脉瓣狭窄的首选方法,在大部分病例PBPV可替代外科开胸手术。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "pro", "entity": "经皮球囊肺动脉瓣成形术" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "PBPV" }, { "start_idx": 64, "end_idx": 67, "type": "pro", "entity": "PBPV" }, { "start_idx": 83, "end_idx": 90, "type": "dis", "entity": "典型肺动脉瓣狭窄" }, { "start_idx": 103, "end_idx": 106, "type": "pro", "entity": "PBPV" }, { "start_idx": 110, "end_idx": 115, "type": "pro", "entity": "外科开胸手术" } ] }, { "text": "另外对发育不良型、心房水平右向左分流重症肺动脉瓣狭窄、法洛四联症及复合畸形、室间隔完整的肺动脉瓣闭锁等进行PBPV术正在临床研究中。", "entities": [ { "start_idx": 3, "end_idx": 25, "type": "dis", "entity": "发育不良型、心房水平右向左分流重症肺动脉瓣狭窄" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "复合畸形" }, { "start_idx": 38, "end_idx": 49, "type": "dis", "entity": "室间隔完整的肺动脉瓣闭锁" }, { "start_idx": 53, "end_idx": 57, "type": "pro", "entity": "PBPV术" } ] }, { "text": "1.适应证由于方法学改进,大量PBPV术临床应用经验的积累,目前应用的指征更加明确且应用范围较前广泛,主要的指征为:(1)典型肺动脉瓣狭窄:肺动脉与右心室压差≥50mmHg。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "PBPV" }, { "start_idx": 61, "end_idx": 68, "type": "dis", "entity": "典型肺动脉瓣狭窄" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "肺动脉" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "右心室" } ] }, { "text": "对于新生儿重症肺动脉瓣狭窄亦可进行急诊球囊扩张术。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "新生儿重症肺动脉瓣狭窄" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "急诊球囊扩张术" } ] }, { "text": "(2)重症肺动脉瓣狭窄伴心房水平右向左分流:目前在有经验的心血管中心,大部分病例仍为适应证。", "entities": [ { "start_idx": 3, "end_idx": 20, "type": "dis", "entity": "重症肺动脉瓣狭窄伴心房水平右向左分流" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "心血管" } ] }, { "text": "(3)发育不良型肺动脉瓣狭窄:早期报道效果不良,目前约2/3病例仍可获得满意效果,但重症病例效果不满意。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "发育不良型肺动脉瓣狭窄" } ] }, { "text": "(4)其他:包括复杂型先心病的姑息疗法以缓解发绀和换瓣后引起再狭窄,室间隔完整的肺动脉瓣闭锁等。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "复杂型先心病" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "姑息疗法" }, { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "发绀和换瓣后引起再狭窄" }, { "start_idx": 34, "end_idx": 45, "type": "dis", "entity": "室间隔完整的肺动脉瓣闭锁" } ] }, { "text": "以上选择指征需要根据每一心血管中心介入性心导管术的经验、条件及人员设备等而定。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "心血管" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "介入性心导管术" } ] }, { "text": "开始PBPV时,应选择典型的PS为宜。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "PBPV" } ] }, { "text": "2.方法学在进行PBPV前,首先行右心导管术及右心室造影,以确定肺动脉瓣狭窄的类型及严重程度,然后按规范方法进行。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "PBPV" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "右心导管术" }, { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "右心室造影" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "肺动脉瓣狭窄" } ] }, { "text": "PBPV有两种方法,即单球囊肺动脉瓣膜成形术和双球囊肺动脉瓣成形术。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "PBPV" }, { "start_idx": 11, "end_idx": 21, "type": "pro", "entity": "单球囊肺动脉瓣膜成形术" }, { "start_idx": 23, "end_idx": 32, "type": "pro", "entity": "双球囊肺动脉瓣成形术" } ] }, { "text": "为了达到足够的球囊/瓣环比值,有些病例需作双球囊扩张术,分别由左右股静脉进行穿刺插入球囊导管,方法同单球囊扩张术。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "球囊" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "瓣环" }, { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "双球囊扩张术" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "左右股静脉" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "球囊导管" }, { "start_idx": 50, "end_idx": 55, "type": "pro", "entity": "单球囊扩张术" } ] }, { "text": "以下简述单球囊法。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "单球囊法" } ] }, { "text": "先以端孔导管或球囊端孔漂浮导管,经股静脉、下腔静脉、右心房、肺总动脉,最后到达肺小动脉,然后插入直头或弯头导引钢丝至肺下叶动脉,撤去端孔导管,用扩张管扩大股静脉穿刺口,使球囊导管顺利插入。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "equ", "entity": "端孔导管" }, { "start_idx": 7, "end_idx": 14, "type": "equ", "entity": "球囊端孔漂浮导管" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "股静脉" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "下腔静脉" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "右心房" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "肺总动脉" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "肺小动脉" }, { "start_idx": 48, "end_idx": 56, "type": "equ", "entity": "直头或弯头导引钢丝" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "肺下叶动脉" }, { "start_idx": 66, "end_idx": 69, "type": "equ", "entity": "端孔导管" }, { "start_idx": 72, "end_idx": 74, "type": "equ", "entity": "扩张管" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "股静脉" }, { "start_idx": 85, "end_idx": 88, "type": "equ", "entity": "球囊导管" } ] }, { "text": "球囊导管插入前应检查有无破损及漏气,同时应用稀释造影剂,扩张或吸瘪球囊数次以驱除空气,防止球囊破裂时空气释出而引起气栓并发症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "equ", "entity": "球囊导管" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "稀释造影剂" }, { "start_idx": 33, "end_idx": 34, "type": "equ", "entity": "球囊" }, { "start_idx": 45, "end_idx": 46, "type": "equ", "entity": "球囊" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "气栓并发症" } ] }, { "text": "推送球囊导管直至肺动脉瓣处。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "equ", "entity": "球囊导管" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "肺动脉瓣" } ] }, { "text": "先以少量造影剂注入球囊扩张导管,使球囊扩张以观察球囊是否恰跨在瓣环中心并呈腰凹症,如果球囊已到位,则用稀释造影剂以约3atm扩张球囊,开始显示肺动脉瓣狭窄处腰凹,随球囊腔内压力增加,腰凹随之消失。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "造影剂" }, { "start_idx": 9, "end_idx": 14, "type": "equ", "entity": "球囊扩张导管" }, { "start_idx": 17, "end_idx": 18, "type": "equ", "entity": "球囊" }, { "start_idx": 24, "end_idx": 25, "type": "equ", "entity": "球囊" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "瓣环" }, { "start_idx": 43, "end_idx": 44, "type": "equ", "entity": "球囊" }, { "start_idx": 51, "end_idx": 55, "type": "dru", "entity": "稀释造影剂" }, { "start_idx": 64, "end_idx": 65, "type": "equ", "entity": "球囊" }, { "start_idx": 71, "end_idx": 76, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 82, "end_idx": 83, "type": "equ", "entity": "球囊" } ] }, { "text": "球囊扩张时一旦球囊全部扩张,腰凹消失,即可吸瘪球囊。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "球囊" }, { "start_idx": 7, "end_idx": 8, "type": "equ", "entity": "球囊" }, { "start_idx": 23, "end_idx": 24, "type": "equ", "entity": "球囊" } ] }, { "text": "通常从开始扩张球囊至吸瘪球囊总时间<10秒,这样可减少由于右心室流出道血流中断时间过长而引起并发症。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "equ", "entity": "球囊" }, { "start_idx": 12, "end_idx": 13, "type": "equ", "entity": "球囊" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "右心室流出道" } ] }, { "text": "如果经过球囊扩张术后效果不满意是由于球/瓣比值不足引起,则可调用更大球囊或用双球囊进行扩张。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "球囊扩张术" }, { "start_idx": 34, "end_idx": 35, "type": "equ", "entity": "球囊" }, { "start_idx": 38, "end_idx": 40, "type": "equ", "entity": "双球囊" } ] }, { "text": "PBPV后,如扩张满意,即撤回球囊导管直至股静脉穿刺处,以堵塞穿刺口渗血。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "PBPV" }, { "start_idx": 15, "end_idx": 18, "type": "equ", "entity": "球囊导管" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "股静脉" } ] }, { "text": "15分钟后,由另一侧股静脉插入右心导管,重点血流动力学检测。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "股静脉" }, { "start_idx": 15, "end_idx": 18, "type": "equ", "entity": "右心导管" } ] }, { "text": "通常PBPV后ΔP<25mmHg,则效果良好。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "PBPV" } ] }, { "text": "并作左侧位右心室造影观察球囊扩张后效果及右心室漏斗部是否存在反应性狭窄。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "左侧位右心室造影" }, { "start_idx": 12, "end_idx": 13, "type": "equ", "entity": "球囊" }, { "start_idx": 20, "end_idx": 25, "type": "bod", "entity": "右心室漏斗部" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "反应性狭窄" } ] }, { "text": "3.球囊扩张术后反应性右心室漏斗部狭窄一部分病人在PBPV后发现瓣口梗阻虽已解除,但由于发生反应性漏斗部狭窄,使右心室压力不能满意下降,但连续曲线示肺动脉与漏斗部压差已解除,表明PBPV术有效。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "球囊扩张术" }, { "start_idx": 8, "end_idx": 18, "type": "dis", "entity": "反应性右心室漏斗部狭窄" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "PBPV" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "瓣口梗阻" }, { "start_idx": 46, "end_idx": 53, "type": "dis", "entity": "反应性漏斗部狭窄" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "右心室" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "肺动脉" }, { "start_idx": 89, "end_idx": 92, "type": "pro", "entity": "PBPV" } ] }, { "text": "10%~20%PBPV术后发生程度不一的漏斗部反应性狭窄。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "PBPV" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "漏斗部反应性狭窄" } ] }, { "text": "术后可给予普萘洛尔(心得安)口服,6个月内均可恢复正常。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "普萘洛尔" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "心得安" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "口服" } ] }, { "text": "4.并发症PBPV虽为安全而有效的治疗肺动脉瓣狭窄的非开胸方法,但仍有5%左右并发症,总死亡率<0.5%,多见于新生儿、小婴儿及重症病例。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "PBPV" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "肺动脉瓣狭窄" } ] }, { "text": "可能出现的并发症为球囊加压扩张时可引起一过性血压下降、心动过缓及期前收缩,另外血管损伤、右房室瓣损伤致关闭不全及心脏穿孔等偶有发生。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "equ", "entity": "球囊" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "一过性血压下降" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "心动过缓" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "期前收缩" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "血管损伤" }, { "start_idx": 44, "end_idx": 54, "type": "dis", "entity": "右房室瓣损伤致关闭不全" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "心脏穿孔" } ] }, { "text": "5.疗效评价与随访PBPV后即时及中长期随访表明,经皮球囊瓣膜成形术治疗肺动脉瓣狭窄效果最好,并发症最少,可达到开胸术疗效以替代外科开胸手术。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "PBPV" }, { "start_idx": 25, "end_idx": 33, "type": "pro", "entity": "经皮球囊瓣膜成形术" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 56, "end_idx": 58, "type": "pro", "entity": "开胸术" }, { "start_idx": 64, "end_idx": 69, "type": "pro", "entity": "外科开胸手术" } ] }, { "text": "术后随访包括临床、心电图、胸片及多普勒(彩色)超声心动图检查以评价疗效。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "心电图" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "胸片" }, { "start_idx": 16, "end_idx": 29, "type": "pro", "entity": "多普勒(彩色)超声心动图检查" } ] }, { "text": "(二)经皮球囊主动脉瓣成形术1984年,Lababidi等首先报道应用经皮球囊主动脉瓣成形术(PBAV)成功地治疗先天性或后天性主动脉瓣狭窄,引起广泛的重视。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "pro", "entity": "经皮球囊主动脉瓣成形术" }, { "start_idx": 35, "end_idx": 45, "type": "pro", "entity": "经皮球囊主动脉瓣成形术" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "PBAV" }, { "start_idx": 57, "end_idx": 69, "type": "dis", "entity": "先天性或后天性主动脉瓣狭窄" } ] }, { "text": "由于我国发病率较欧美国家为少,另外PBAV在技术上有别于PBPV,且有严重并发症发生,因此国内除上海新华医院一组报告外,尚无系统开展该技术的报道。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "PBAV" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "PBPV" } ] }, { "text": "根据1982~1986年204例小儿经皮球囊主动脉瓣形成术总结,95.1%获得即期成功。", "entities": [ { "start_idx": 18, "end_idx": 28, "type": "pro", "entity": "经皮球囊主动脉瓣形成术" } ] }, { "text": "随后对PBAV方法学上进行较多的研究,包括单球囊与双球囊扩张术及采用长的球囊进行扩张术,以后把球囊瓣膜成形术应用到新生儿病例,甚至进行胎儿主动脉瓣球囊成形术。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "PBAV" }, { "start_idx": 21, "end_idx": 30, "type": "dis", "entity": "单球囊与双球囊扩张术" }, { "start_idx": 32, "end_idx": 42, "type": "dis", "entity": "采用长的球囊进行扩张术" }, { "start_idx": 47, "end_idx": 53, "type": "dis", "entity": "球囊瓣膜成形术" }, { "start_idx": 67, "end_idx": 77, "type": "dis", "entity": "胎儿主动脉瓣球囊成形术" } ] }, { "text": "目前,正在对PBAV病人进行中、长期随访,以观察其疗效与心功能的改变。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "PBAV" } ] }, { "text": "1.经皮球囊主动脉瓣成形术的指征及反指征(1)主要指征:1)血流动力学:跨瓣收缩压差>50mmHg伴正常心排量;无或仅轻度主动脉瓣反流。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "经皮球囊主动脉瓣成形术" }, { "start_idx": 30, "end_idx": 34, "type": "ite", "entity": "血流动力学" }, { "start_idx": 36, "end_idx": 40, "type": "ite", "entity": "跨瓣收缩压" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "心" }, { "start_idx": 61, "end_idx": 66, "type": "sym", "entity": "主动脉瓣反流" } ] }, { "text": "2)主动脉瓣狭窄形态学:非瓣膜发育不良型、瓣膜薄且活动良好、无明显瓣环发育不良者为良好指征。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "主动脉瓣狭窄" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "瓣膜薄且活动良好" }, { "start_idx": 30, "end_idx": 38, "type": "sym", "entity": "无明显瓣环发育不良" } ] }, { "text": "(2)PBAV禁忌证:中度以上主动脉瓣反流以及心导管术禁忌者。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "PBAV" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "主动脉瓣反流" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "心导管术" } ] }, { "text": "2.方法可通过以下途径进行PBAV术:①逆行股动脉插管法,临床常用;②脐动脉、腋动脉及颈动脉插管法,适用于小婴儿;③经房间隔穿刺法球囊主动脉瓣成形术,该法一般适用于不宜经股动脉穿刺法者。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "PBAV" }, { "start_idx": 20, "end_idx": 27, "type": "pro", "entity": "逆行股动脉插管法" }, { "start_idx": 35, "end_idx": 48, "type": "pro", "entity": "脐动脉、腋动脉及颈动脉插管法" }, { "start_idx": 58, "end_idx": 73, "type": "pro", "entity": "经房间隔穿刺法球囊主动脉瓣成形术" } ] }, { "text": "3.疗效评价及随访PBAV的成功率为80%~97%,平均压差下降55%~70%。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "PBAV" } ] }, { "text": "与PBPV相比有较多严重并发症,主要为动脉栓塞(特别是婴幼儿)、明显主动脉反流、心律失常、心功能不全、心肌穿孔等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "PBPV" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "动脉栓塞" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "明显主动脉反流" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "心律失常" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "心功能不全" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "心肌穿孔" } ] }, { "text": "二、先天性肺囊肿先天性肺囊肿(congenitalpulmonarycysts)是常见的肺发育异常,系胚胎期肺芽发育障碍所致。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "先天性肺囊肿" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "先天性肺囊肿" }, { "start_idx": 15, "end_idx": 38, "type": "dis", "entity": "congenitalpulmonarycysts" } ] }, { "text": "病变肺组织可出现单个或多个囊肿,累及一个或多个肺叶。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺组织" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺叶" } ] }, { "text": "当囊肿黏液潴留过多或继发化脓性感染时,囊腔易与支气管相通,常形成单向活瓣样通气,导致肺泡腔内压力不断升高,形成张力性气囊肿,出现严重压迫症状。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "sym", "entity": "囊肿黏液潴留过多" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "化脓性感染" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "囊腔" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "支气管相通" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "单向活瓣样通气" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "肺泡腔内" }, { "start_idx": 55, "end_idx": 60, "type": "sym", "entity": "张力性气囊肿" } ] }, { "text": "囊肿小者可长期不出现任何症状,直至胸部X线检查时被发现。", "entities": [ { "start_idx": 17, "end_idx": 22, "type": "ite", "entity": "胸部X线检查" } ] }, { "text": "1岁以内患儿多因呼吸困难就诊,而肺部感染是年长儿的主要临床特征。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "肺部感染" } ] }, { "text": "单发闭合性肺囊肿在X线下显示一个圆形或类圆形阴影,密度均匀,边缘清晰,周围一般无明显浸润病灶。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "单发闭合性肺囊肿" } ] }, { "text": "多发性肺囊肿在X线平片上显示多数大小不等的圆形或类圆形阴影,阴影内可出现液平面,周围可伴炎性浸润。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "多发性肺囊肿" } ] }, { "text": "巨大的张力性气囊肿,有时易与张力性气胸相混淆,但张力性气囊肿在肺野的边缘如肺尖或肋膈角处仍可看到肺组织,而张力性气胸患侧肺组织被压到肺门区,肺野边缘部分看不到肺组织,且往往伴有胸膜反应。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "巨大的张力性气囊肿" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "张力性气胸" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "张力性气囊肿" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "肺野的边缘" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "肺尖" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "肋膈角" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "肺组织" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "张力性气胸" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "肺组织" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "肺门区" }, { "start_idx": 70, "end_idx": 75, "type": "bod", "entity": "肺野边缘部分" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "肺组织" }, { "start_idx": 88, "end_idx": 91, "type": "sym", "entity": "胸膜反应" } ] }, { "text": "对已出现张力性病变而引起严重压迫症状者,可先行胸腔减压,然后再手术。", "entities": [ { "start_idx": 4, "end_idx": 18, "type": "dis", "entity": "张力性病变而引起严重压迫症状者" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "胸腔减压" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "手术" } ] }, { "text": "三、其他病原学检查支原体病原学检查与病毒病原学检查相似。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "病原学检查" }, { "start_idx": 9, "end_idx": 16, "type": "pro", "entity": "支原体病原学检查" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "病毒病原学检查" } ] }, { "text": "呼吸道分泌物支原体分离和双份血清抗体测定可作为支原体感染诊断的金标准。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "pro", "entity": "呼吸道分泌物支原体分离" }, { "start_idx": 12, "end_idx": 19, "type": "pro", "entity": "双份血清抗体测定" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "支原体感染" } ] }, { "text": "临床上常应用咽拭子支原体DNA检测、支原体特异抗原测定及血清特异抗体IgM测定进行快速诊断,指导临床用药。", "entities": [ { "start_idx": 6, "end_idx": 16, "type": "pro", "entity": "咽拭子支原体DNA检测" }, { "start_idx": 18, "end_idx": 26, "type": "pro", "entity": "支原体特异抗原测定" }, { "start_idx": 28, "end_idx": 38, "type": "pro", "entity": "血清特异抗体IgM测定" } ] }, { "text": "与病毒不同的是,由于支原体可在细胞外单独存活,无需借助宿主细胞的细胞器,因而对呼吸道标本支原体的检测只需咽拭子,而不需要脱落细胞。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "mic", "entity": "病毒" }, { "start_idx": 10, "end_idx": 12, "type": "mic", "entity": "支原体" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "呼吸道" }, { "start_idx": 44, "end_idx": 46, "type": "mic", "entity": "支原体" }, { "start_idx": 52, "end_idx": 54, "type": "equ", "entity": "咽拭子" } ] }, { "text": "冷凝集试验是较早用于肺炎支原体感染早期诊断的一种方法,其原理是检查在4℃时有无凝集人类红细胞的IgM自身抗体。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "冷凝集试验" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "肺炎支原体感染" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "红细胞" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "IgM" } ] }, { "text": "但该方法并非特异性,当腺病毒等感染时亦可出现阳性,因而只能作为辅助诊断。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "mic", "entity": "腺病毒" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "阳性" } ] }, { "text": "对疑有真菌感染者,可采集痰标本、气管分泌物或血液等送检。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "真菌感染" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "痰" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "气管分泌物" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血液" } ] }, { "text": "直接涂片检查若发现真菌菌丝或孢子,可初步判定为真菌感染。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "直接涂片检查" }, { "start_idx": 9, "end_idx": 15, "type": "mic", "entity": "真菌菌丝或孢子" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "真菌感染" } ] }, { "text": "培养诊断价值较高,但多次培养阳性才有意义。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "阳性" } ] }, { "text": "亦可采用血清学、免疫学或分子生物学技术,如血清冷凝集试验、补体结合试验、免疫酶技术、免疫荧光技术、PCR技术等进行真菌病原的快速诊断。", "entities": [ { "start_idx": 21, "end_idx": 27, "type": "pro", "entity": "血清冷凝集试验" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "补体结合试验" }, { "start_idx": 36, "end_idx": 40, "type": "pro", "entity": "免疫酶技术" }, { "start_idx": 42, "end_idx": 47, "type": "pro", "entity": "免疫荧光技术" }, { "start_idx": 49, "end_idx": 53, "type": "pro", "entity": "PCR技术" }, { "start_idx": 57, "end_idx": 58, "type": "mic", "entity": "真菌" } ] }, { "text": "第六节溶血性贫血一、总论溶血性贫血(hemolyticanemia)是由多种病因引起红细胞寿命的缩短和过早地破坏,且红细胞的破坏超过了骨髓生血功能的代偿能力而发生循环中红细胞数和血红蛋白含量减少贫血。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 18, "end_idx": 32, "type": "dis", "entity": "hemolyticanemia" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "红细胞" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "红细胞" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "骨髓" }, { "start_idx": 84, "end_idx": 87, "type": "ite", "entity": "红细胞数" }, { "start_idx": 89, "end_idx": 94, "type": "ite", "entity": "血红蛋白含量" }, { "start_idx": 81, "end_idx": 96, "type": "sym", "entity": "循环中红细胞数和血红蛋白含量减少" }, { "start_idx": 97, "end_idx": 98, "type": "dis", "entity": "贫血" } ] }, { "text": "正常红细胞的寿命为110~120日左右。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "红细胞" } ] }, { "text": "正常情况下,每日约有1%的衰老红细胞在脾脏中被吞噬和破坏,由新生的红细胞补充代替之,从而维持红细胞数量的恒定以发挥正常的生理功能。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "红细胞" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "脾脏" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "红细胞" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "红细胞" } ] }, { "text": "正常小儿骨髓造血潜能很大,一般可增加到正常的6~8倍。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "骨髓" } ] }, { "text": "【分类】根据溶血因素存在的部位不同,可将溶血性贫血分为红细胞内和红细胞外两大类。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "溶血" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "红细胞" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "红细胞" } ] }, { "text": "(一)红细胞内因素1.红细胞膜的缺陷(1)遗传性球形细胞增多症。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "红细胞" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "红细胞膜" }, { "start_idx": 21, "end_idx": 30, "type": "dis", "entity": "遗传性球形细胞增多症" } ] }, { "text": "(2)遗传性椭圆形细胞增多症。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "遗传性椭圆形细胞增多症" } ] }, { "text": "(5)遗传性棘细胞增生症。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "遗传性棘细胞增生症" } ] }, { "text": "(6)阵发性睡眠性血红蛋白尿。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "阵发性睡眠性血红蛋白尿" } ] }, { "text": "2.红细胞酶的缺陷(1)红细胞糖分解酶缺乏:1)丙酮酸激酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "红细胞酶的缺陷" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "红细胞糖分解酶缺乏" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "丙酮酸激酶缺乏" } ] }, { "text": "3)磷酸果糖激酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "磷酸果糖激酶缺乏" } ] }, { "text": "4)丙糖磷酸异构酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "丙糖磷酸异构酶缺乏" } ] }, { "text": "5)己糖激酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "己糖激酶缺乏" } ] }, { "text": "6)磷酸甘油酸盐激酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "磷酸甘油酸盐激酶缺乏" } ] }, { "text": "7)醛缩酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "醛缩酶缺乏" } ] }, { "text": "8)二磷酸甘油酸盐变位酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dis", "entity": "二磷酸甘油酸盐变位酶缺乏" } ] }, { "text": "(2)红细胞核苷酸代谢异常:1)嘧啶5-核苷酸酶缺乏。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "红细胞核苷酸代谢异常" }, { "start_idx": 16, "end_idx": 25, "type": "dis", "entity": "嘧啶5-核苷酸酶缺乏" } ] }, { "text": "2)腺苷脱氨酶过多。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "腺苷脱氨酶过多" } ] }, { "text": "4)腺苷酸激酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "腺苷酸激酶缺乏" } ] }, { "text": "(3)戊糖磷酸盐通路及谷胱苷肽代谢有关的酶缺乏:1)葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏。", "entities": [ { "start_idx": 3, "end_idx": 22, "type": "dis", "entity": "戊糖磷酸盐通路及谷胱苷肽代谢有关的酶缺乏" }, { "start_idx": 26, "end_idx": 46, "type": "dis", "entity": "葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏" } ] }, { "text": "2)谷氨酰半胱氨酸合成酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dis", "entity": "谷氨酰半胱氨酸合成酶缺乏" } ] }, { "text": "3)谷胱甘肽合成酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "谷胱甘肽合成酶缺乏" } ] }, { "text": "3.血红蛋白异常(1)珠蛋白生成障碍性贫血。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 11, "end_idx": 20, "type": "dis", "entity": "珠蛋白生成障碍性贫血" } ] }, { "text": "(4)不稳定血红蛋白病。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "不稳定血红蛋白病" } ] }, { "text": "(5)其他同型合子血红蛋白病(CC,DI,EE)。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "其他同型合子血红蛋白病" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "CC" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "DI" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "EE" } ] }, { "text": "(6)双杂合子紊乱(HbSC,镰状细胞珠蛋白生成障碍性贫血)。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "双杂合子紊乱" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "HbSC" }, { "start_idx": 15, "end_idx": 28, "type": "dis", "entity": "镰状细胞珠蛋白生成障碍性贫血" } ] }, { "text": "(二)红细胞外因素1.免疫性溶血性贫血(1)错输血型不匹配血。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "红细胞" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "免疫性溶血性贫血" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "血" } ] }, { "text": "(3)自身免疫性溶血性贫血:1)温抗体所致的自身免疫性溶血性贫血。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "自身免疫性溶血性贫血" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "抗体" }, { "start_idx": 22, "end_idx": 31, "type": "dis", "entity": "自身免疫性溶血性贫血" } ] }, { "text": "2)冷抗体所致的自身免疫性溶血性贫血。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "抗体" }, { "start_idx": 8, "end_idx": 17, "type": "dis", "entity": "自身免疫性溶血性贫血" } ] }, { "text": "2.创伤性及微血管性溶血性贫血(1)人工瓣膜及其他心脏异常。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "创伤性及微血管性溶血性贫血" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "人工瓣膜" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "心脏" } ] }, { "text": "(5)血栓性血小板减少性紫癜。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "血栓性血小板减少性紫癜" } ] }, { "text": "(6)溶血尿毒综合征。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "溶血尿毒综合征" } ] }, { "text": "3.脾功能亢进4.血浆因素(1)肝脏疾病:如血浆胆固醇、磷脂过高所致脂肪肝、肝硬化等引起靴刺细胞(spurcell)贫血。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "脾功能亢进" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "血浆" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "血浆胆固醇" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "磷脂" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "脂肪肝" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "肝硬化" }, { "start_idx": 44, "end_idx": 59, "type": "dis", "entity": "靴刺细胞(spurcell)贫血" } ] }, { "text": "5.感染性(1)原虫:疟原虫、毒浆原虫、黑热病原虫等。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "mic", "entity": "原虫" }, { "start_idx": 11, "end_idx": 13, "type": "mic", "entity": "疟原虫" }, { "start_idx": 15, "end_idx": 18, "type": "mic", "entity": "毒浆原虫" }, { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "黑热病原虫" } ] }, { "text": "(2)细菌:梭状菌属感染(如梭状芽胞杆菌)、霍乱、伤寒等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "细菌" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "梭状菌属感染" }, { "start_idx": 14, "end_idx": 19, "type": "mic", "entity": "梭状芽胞杆菌" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "霍乱" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "伤寒" } ] }, { "text": "6.肝豆状核变性(Wilson病)7.化学品、药物及蛇毒(1)氧化性药物及化学制剂。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "Wilson病" }, { "start_idx": 31, "end_idx": 35, "type": "dru", "entity": "氧化性药物" } ] }, { "text": "(2)非氧化性药物。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "非氧化性药物" } ] }, { "text": "(3)新生儿维生素E缺乏。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "新生儿维生素E缺乏" } ] }, { "text": "(4)并存于尿毒症、血液透析。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "尿毒症" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "血液透析" } ] }, { "text": "【诊断】溶血性贫血的诊断主要依靠临床表现和实验室检查。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "溶血性贫血" } ] }, { "text": "当患儿有贫血伴有网织红细胞增高时,应考虑到溶血性贫血存在的可能性,应选择下列试验寻找红细胞破坏增加的直接和间接证据。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "贫血" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "网织红细胞" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "红细胞" } ] }, { "text": "(一)红细胞破坏增加的直接证据1.Na251CrO4标记红细胞测定红细胞寿命,其半寿命明显缩短。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "红细胞" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "红细胞" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "红细胞" } ] }, { "text": "2.血浆游离血红蛋白增多。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血浆" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "5.出现血红蛋白尿时,联苯胺试验阳性。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "血红蛋白尿" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "联苯胺试验" } ] }, { "text": "(二)红细胞破坏增加的间接证据1.网织红细胞明显增加。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "红细胞" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "网织红细胞" } ] }, { "text": "3.骨髓红系增生活跃,粒∶红比例降低甚至倒置。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "骨髓" } ] }, { "text": "4.由于红细胞生成代偿性增快,红细胞大小不一,形状不等,红细胞带有核或核残余。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "红细胞" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "生成代偿性增快" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "红细胞" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "大小不一" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "形状不等" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "红细胞" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "带有核或核残余" } ] }, { "text": "一旦红细胞破坏过多存在,溶血性贫血的诊断成立,然后,再根据抗人球蛋白(Coombs)直接及间接试验的阳性与否区分免疫性(Coombs试验阳性,血型不合溶血除外)与非免疫性(Coombs试验阴性);非免疫性溶血性贫血可根据红细胞形态、脆性试验、葡萄糖孵育脆性试验、高铁血红蛋白还原试验、酸溶血试验(Ham)、红细胞酶谱分析、红细胞CD55/CD59流式细胞仪分析、珠蛋白小体(Hein’sbody)、血红蛋白电泳等试验区分各种原因引起的溶血性贫血。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "红细胞" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 29, "end_idx": 48, "type": "pro", "entity": "抗人球蛋白(Coombs)直接及间接试验" }, { "start_idx": 98, "end_idx": 106, "type": "dis", "entity": "非免疫性溶血性贫血" }, { "start_idx": 110, "end_idx": 112, "type": "bod", "entity": "红细胞" }, { "start_idx": 116, "end_idx": 119, "type": "pro", "entity": "脆性试验" }, { "start_idx": 121, "end_idx": 129, "type": "pro", "entity": "葡萄糖孵育脆性试验" }, { "start_idx": 142, "end_idx": 146, "type": "pro", "entity": "酸溶血试验" }, { "start_idx": 148, "end_idx": 150, "type": "pro", "entity": "Ham" }, { "start_idx": 153, "end_idx": 159, "type": "pro", "entity": "红细胞酶谱分析" }, { "start_idx": 161, "end_idx": 179, "type": "pro", "entity": "红细胞CD55/CD59流式细胞仪分析" }, { "start_idx": 181, "end_idx": 185, "type": "pro", "entity": "珠蛋白小体" }, { "start_idx": 187, "end_idx": 196, "type": "pro", "entity": "Hein’sbody" }, { "start_idx": 199, "end_idx": 204, "type": "pro", "entity": "血红蛋白电泳" }, { "start_idx": 217, "end_idx": 221, "type": "dis", "entity": "溶血性贫血" } ] }, { "text": "【治疗】溶血性贫血种类多种多样,因此,其治疗应根据病因、类型拟定治疗方案。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "溶血性贫血" } ] }, { "text": "输血可改善贫血症状,但有时也有一定的危险性,例如给自身免疫性溶血性贫血病儿输血可发生严重的溶血反应。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "输血" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "贫血" }, { "start_idx": 25, "end_idx": 35, "type": "dis", "entity": "自身免疫性溶血性贫血病" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "输血" } ] }, { "text": "大量输血还可抑制病儿的造血机能。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "输血" } ] }, { "text": "对某些先天性或遗传性溶血性贫血除给予对症处理外,尚可采取切脾甚至造血干细胞移植等治疗。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "先天性或遗传性溶血性贫血" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "切脾" }, { "start_idx": 32, "end_idx": 38, "type": "pro", "entity": "造血干细胞移植" } ] }, { "text": "总之,溶血性贫血的治疗应针对某一特定缺陷来选择治疗方案。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "溶血性贫血" } ] }, { "text": "参考文献1.孙锟,陈树宝,周爱卿,等.二维超声技术检测右心室容量.中华医学杂志,1993;7:353-3532.孙锟,陈树宝,江海,等.婴儿心脏三维超声信息采集及重建的方法学研究.中国医学影像技术,1999;15(10):775-7753.周爱卿.心导管术-先天性心脏病的诊断与治疗.济南:山东科技出版社,19974.AllenHD,GutgesellHP,ClarkEB,DriscollDJ.MossandAdam’sheartdiseaseininfants,children,andadolescentsincludingthefetusandyoungadult(Sixthedition).Philadelphia:LippincottWilliams&wilkins,2001.162-1625.BalajiS,LauY,CaseC,etal.VentriculartachycardiaintetralogyofFallot.AmJCardiol,1997.80:1606.BudingerTF,BersonA,McVeighER,etal.CardiacMRimaging:reportofaworkinggroupsponsoredbytheNationalHeart,LungandBloodInstitute.Radiology,1998,208:573-5767.FischC.Evolutionoftheclinicalelectrocardiogram.JAmCollCardiol,1989,14:11278.KuglerJD,DanfordDA,DealBJ,etal.Radiofrequencycatheterablationfortachyarrhythmiasinchildrenandadolescents.NEnglJMed,1994,330:1481-14819.NASPEExpertConsensusConference:RadiofrequencyCatheterAblationinChildrenWithandWithoutCongenitalHeartDisease.ReportoftheWritingCommittee.PACE,2002,25(6):1000-100010.PihkalaJ,NykanenD,FreedomRM,etal.Interventionalcardiaccatheterization.PediatrClinNorthAm,1999,46(2):441-46011.ReichJD,AuldD,HulseE,etal.ThePediatricRadiofrequencyAblationRegistry’sexperiencewithEbstein’sanomaly.PediatricElectrophysiologySociety.JCardiovascElectrophysiol,1998,9:1370-137012.SchelagBJ,LauSH,HelfantRH,etal.CathetertechniquesforrecordingHisbundleactivityinman.Circulation,1969,39:1313.VanHareGF.Indicationsforradiofrequencyablationinthepediatricpopulation.JCardiovascElectrophysiol,1997,8:952-95214.WashingtonRL,BrickerJT,AlperBS,etal.Guidelinesforexercisetestinginthepediatricagegroup:Fromthecommitteeonatherosclerosisandhypertensioninchildrencounciloncardiovasculardiseaseintheyoung,theAmericanHeartAssociation.Circulation,1994,90:216615.ZipesDP,DiMarcoJP,GillettePC,etal.ACC/AHATaskForcereport.Guidelinesforclinicalintracardiacelectrophysiologicalandcatheterablationprocedures.Circulation,1995,92:673", "entities": [ { "start_idx": 19, "end_idx": 26, "type": "pro", "entity": "二维超声技术检测" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "右心室" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "心脏" }, { "start_idx": 72, "end_idx": 75, "type": "pro", "entity": "三维超声" }, { "start_idx": 124, "end_idx": 127, "type": "pro", "entity": "心导管术" }, { "start_idx": 129, "end_idx": 134, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "第九篇心血管系统疾病第一章心脏的胚胎发生学心脏由胚胎的中胚层组织演变而来,在胚胎的头三周内,中胚层细胞分化形成原始心管。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "心血管系统" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心脏" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "心脏" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "原始心管" } ] }, { "text": "此后,经过心管的分区、扭转及分隔逐渐形成完整的心脏。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心管" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心脏" } ] }, { "text": "一、心管管的形成早在胚胎15mm时,胚胎腹面咽喉下部的两侧下部的两侧,由原始基(primordia)形成两个管状结构,其内层为心内膜始基,外层为心外肌膜始基。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心管" }, { "start_idx": 20, "end_idx": 28, "type": "bod", "entity": "腹面咽喉下部的两侧" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "心内膜" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "心外肌膜" } ] }, { "text": "至22天左右,两个内皮管逐渐向正中移动,两个原始基相互融合成一腔,此即原始心管。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "内皮管" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "原始心管" } ] }, { "text": "参考文献1.王宏伟,杨锡强,朱启镕.小儿HIV感染和艾滋病诊断和处理建议.中华儿科杂志,2003,41(8)∶611-6122.杨锡强等.儿童免疫学.北京:人民卫生出版社,20013.JaneM,PamelaA,DavidW,etal.AntiretroviralAdherenceInterventions:AReviewofCurrentLiteratureandOngoingStudies.InternationalAIDSSocietyUSATopicsinHIVMedicine,2003,11(6):185-1854.OleskeJM.ReviewofrecentguidelinesforantiretroviraltreatmentofHIV-infectedchildren.TopHIVMed,2003,11(6):1805.SaloojeeH,ViolariA.Regularreview:HIVinfectioninchildren.BMJ.2001,323(7314):670", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "小儿HIV感染" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "艾滋病" } ] }, { "text": "第四节肾穿刺活体组织检查肾穿刺活体组织检查(简称肾活检)在临床及科研上运用已有60余年的历史。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "肾穿刺活体组织检查" }, { "start_idx": 12, "end_idx": 20, "type": "pro", "entity": "肾穿刺活体组织检查" }, { "start_idx": 24, "end_idx": 26, "type": "pro", "entity": "肾活检" } ] }, { "text": "近十年来,随着穿刺技术的不断革新,以及光学显微镜、电子显微镜、免疫荧光技术和免疫酶标技术的应用与推广,各种组织化学及分子病理学研究方法的逐步完善,使肾活检的成功率及安全性大大提高,并且成为研究肾脏病变的病因、发病机制、组织分型、病理演变过程、临床与病理形态学的联系,以及指导治疗、评估预后等方面的一种重要的手段,对肾脏病的迅速发展产生了重大影响。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "equ", "entity": "光学显微镜" }, { "start_idx": 25, "end_idx": 29, "type": "equ", "entity": "电子显微镜" }, { "start_idx": 31, "end_idx": 36, "type": "pro", "entity": "免疫荧光技术" }, { "start_idx": 38, "end_idx": 43, "type": "pro", "entity": "免疫酶标技术" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "组织" }, { "start_idx": 74, "end_idx": 76, "type": "pro", "entity": "肾活检" }, { "start_idx": 96, "end_idx": 97, "type": "bod", "entity": "肾脏" }, { "start_idx": 157, "end_idx": 159, "type": "dis", "entity": "肾脏病" } ] }, { "text": "一、肾活检方法肾活检主要方法有开放性肾活检和经皮肾穿刺活检两种。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肾活检" }, { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "肾活检" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "开放性肾活检" }, { "start_idx": 22, "end_idx": 28, "type": "pro", "entity": "经皮肾穿刺活检" } ] }, { "text": "1990年Mal等曾介绍经静脉活检,但临床较少采用。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "经静脉活检" } ] }, { "text": "(一)开放性肾活检通过手术直接采取肾脏活组织,又称直视下肾活检。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "开放性肾活检" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "手术" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "肾脏活组织" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "直视下肾活检" } ] }, { "text": "为肾活检的早期方法,现主要用于经皮肾活检有禁忌证或失败时采用,如孤立肾或对侧肾无功能,有定位好、组织块较大以及止血好等优点。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "pro", "entity": "肾活检" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "经皮肾活检" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "孤立肾" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "对侧肾无功能" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "组织" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "止血" } ] }, { "text": "还应注意直视下肾活检时,不宜采用锐器切割组织,仍应行穿刺术,减少损伤。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "直视下肾活检" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "组织" }, { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "穿刺术" } ] }, { "text": "(二)经皮肾穿刺活检是目前最常用、最安全的方法。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "经皮肾穿刺活检" } ] }, { "text": "穿刺部位的定位方法有体表解剖定位、静脉肾盂造影荧光屏直视定位和B型超声波导向定位。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "穿刺" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "体表解剖定位" }, { "start_idx": 17, "end_idx": 29, "type": "pro", "entity": "静脉肾盂造影荧光屏直视定位" }, { "start_idx": 31, "end_idx": 39, "type": "pro", "entity": "B型超声波导向定位" } ] }, { "text": "1.体表定位法儿童一般以背中线与外侧线连线中点内侧0.5~10cm,第一腰椎棘突水平,十二肋下0.5~1.0cm处为穿刺点。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "体表定位法" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "背" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "腰椎" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "肋" }, { "start_idx": 58, "end_idx": 59, "type": "pro", "entity": "穿刺" } ] }, { "text": "临床常需参考腹部平片上肾脏的位置,但成功率较低,特别是肾脏位置有变异时,与有效穿刺点的符合率较低。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "腹部" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肾脏" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肾脏" } ] }, { "text": "2.静脉肾盂造影荧光屏直视定位直接观察电视屏上的肾脏轮廓,确定穿刺部位及进针方向和深度。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "静脉肾盂造影" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肾脏" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "穿刺" } ] }, { "text": "成功率可达90%以上,并能及时发现是否存在肾脏畸形、转位不良以及肾盂积水等,并避免误刺肾门所致的大出血。", "entities": [ { "start_idx": 21, "end_idx": 29, "type": "dis", "entity": "肾脏畸形、转位不良" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "肾盂积水" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "肾门" }, { "start_idx": 48, "end_idx": 50, "type": "sym", "entity": "大出血" } ] }, { "text": "缺点有造影剂的肾毒性、接受X线照射以及需放射科医生协助等。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "造影剂" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肾" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "X线照射" }, { "start_idx": 20, "end_idx": 22, "type": "dep", "entity": "放射科" } ] }, { "text": "3.B型超声波导向定位临床上采用B型超声波定位后,大大提高了定位的正确性,并能精确地测得肾表面与皮肤之间的距离、吸气和呼气时肾脏下缘以及肾脏下极的厚度,使穿刺者能准确掌握位置和针的深度。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "pro", "entity": "B型超声波定位" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "肾表面" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "皮肤" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "肾脏" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "肾脏" }, { "start_idx": 77, "end_idx": 78, "type": "pro", "entity": "穿刺" } ] }, { "text": "我们体会,实际穿刺的深度常常大于B超探查的深度,最佳比例为1.3∶1~1.4∶1。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "穿刺" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "B超探查" } ] }, { "text": "20世纪90年代以后出现了应用穿刺枪作经皮肾穿刺活检的报道,并通过B超附有的特殊穿刺探头,在B超直接引导下进针。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "equ", "entity": "穿刺枪" }, { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "经皮肾穿刺活检" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "B超" }, { "start_idx": 40, "end_idx": 43, "type": "equ", "entity": "穿刺探头" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "B超" } ] }, { "text": "我院自20世纪80年代中期采用B型超声波定位,1999年开始应用自动穿刺枪在B超穿刺探头的指引下行肾穿刺术,例数已达1100余例,成功率为99%。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "pro", "entity": "B型超声波定位" }, { "start_idx": 32, "end_idx": 36, "type": "equ", "entity": "自动穿刺枪" }, { "start_idx": 38, "end_idx": 43, "type": "equ", "entity": "B超穿刺探头" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "肾穿刺术" } ] }, { "text": "二、补充累积损失量根据脱水程度及性质补充:即轻度脱水约30~50ml/kg(体重);中度为50~100ml/kg;重度为100~150ml/kg。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "脱水" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "轻度脱水" } ] }, { "text": "通常对低渗性脱水补2/3张含钠液;等渗性脱水补1/2张含钠液;高渗性脱水补1/5~1/3张含钠液。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "低渗性脱水" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "含钠液" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "等渗性脱水" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "含钠液" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "高渗性脱水" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "含钠液" } ] }, { "text": "补液的速度取决于脱水程度,原则上应先快后慢。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "脱水" } ] }, { "text": "对伴有循环不良和休克的重度脱水患儿,开始应快速输入等渗含钠液(生理盐水或2∶1液)按20ml/kg于30分钟~1小时输入。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "循环不良" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "休克" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "重度脱水" }, { "start_idx": 25, "end_idx": 29, "type": "dru", "entity": "等渗含钠液" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "生理盐水" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "2∶1液" } ] }, { "text": "酸碱平衡紊乱及其他电解质异常的纠正见(本章第二、三节)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "酸碱平衡紊乱" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "其他电解质异常" } ] }, { "text": "对于高渗性脱水,需缓慢纠正高钠血症(每24小时血钠下降<10mmol/L),也可在数天内纠正。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "高渗性脱水" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "高钠血症" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "血钠" } ] }, { "text": "有时需用张力较高,甚至等张液体,以防血钠迅速下降出现脑水肿。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "血钠" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "脑水肿" } ] }, { "text": "参考文献1.HuW,TongS,OldenburgB,etal.SerumvitaminAconcentrationsandgrowthinchildrenandadolescentsinGansuProvince,china.AsicPacJClinNutr,2001,10(1):63-662.JorgensenFS,FledingP,VintherS,etal.VitaminKtoneonates.Peroralversusintramuscularadministration.ActaPaediatrScand,1991,80(3):304-3043.Arteaga-VizcainoM,Torres-EspinaM,RedondoL,etal.EffectsoftheadministrationofvitaminKontheactivityoffactorⅡ、Ⅶ、Ⅹinhealthynewborns.InvestClin,1995,36(2):83-834.HathawayWE,IsarangkuraPB,MahasandanaC,etal.ComparisonoforalandparenteralvitaminKprophylaxisforpreventionoflatehemorrhagicdiseaseofthenewborn.JPediatr,1991,119(3):461-4645.HuysmanMW,SauerPJ.ThevitaminKcontroversy.CurrOpinPediatr,1994,6(2):129-1296.廖清奎.营养性疾病-小儿营养与营养性疾病.天津:天津科学技术出版社,1990:139-139,157-1577.顾景范,邵继智.临床营养学.上海:上海科学技术出版社,1990:222-2228.史奎雄.蛋白质-热能营养不良//医学营养学.上海:上海交通大学出版社,1998:114-1149.EKHARDEZIEGLER,LJFILER,JR.现代营养学.第7版.北京:人民卫生出版社,1998:116-11610.蒋月英.儿童营养学.北京:科学技术文献出版社,1992:77-7711.林良明,刘玉琳,鲁杰.婴儿维生素K110次口服预防效果评价.中国儿童保健杂志,2002,6(10):147-14712.中国营养学会.中国居民膳食营养素参考摄入量.北京:中国轻工业出版社,200013.ThomasGBaumgartner.ClinicalGuidetoParenteralMicronutrition.2nded.1991:193-19314.沈霞,李定国,姚建,等.现代生物化学检验与临床实践.上海:上海科学技术文献出版社,199915.葛可佑.中国营养科学全书.北京:人民卫生出版社,2004", "entities": [ { "start_idx": 689, "end_idx": 693, "type": "dis", "entity": "营养性疾病" }, { "start_idx": 695, "end_idx": 704, "type": "dis", "entity": "小儿营养与营养性疾病" }, { "start_idx": 786, "end_idx": 795, "type": "dis", "entity": "蛋白质-热能营养不良" }, { "start_idx": 1008, "end_idx": 1010, "type": "bod", "entity": "营养素" } ] }, { "text": "第六节疟疾【病原】疟疾(malaria)是感染疟原虫所引起的传染病。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "疟疾" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "疟疾" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "malaria" }, { "start_idx": 23, "end_idx": 25, "type": "mic", "entity": "疟原虫" } ] }, { "text": "疟原虫可分为四种:间日疟原虫(P.vivax)、三日疟原虫(P.malariae)、恶性疟原虫(P.falciparum)、卵形疟原虫(P.ovale)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "疟原虫" }, { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "间日疟原虫" }, { "start_idx": 15, "end_idx": 21, "type": "mic", "entity": "P.vivax" }, { "start_idx": 24, "end_idx": 28, "type": "mic", "entity": "三日疟原虫" }, { "start_idx": 30, "end_idx": 39, "type": "mic", "entity": "P.malariae" }, { "start_idx": 42, "end_idx": 46, "type": "mic", "entity": "恶性疟原虫" }, { "start_idx": 48, "end_idx": 59, "type": "mic", "entity": "P.falciparum" }, { "start_idx": 62, "end_idx": 66, "type": "mic", "entity": "卵形疟原虫" }, { "start_idx": 68, "end_idx": 74, "type": "mic", "entity": "P.ovale" } ] }, { "text": "疟原虫的发育包括两个阶段,在人体内进行无性生殖(裂体生殖),在蚊子体内完成有性生殖及进行孢子增殖。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "疟原虫" } ] }, { "text": "疟原虫在人体内的发育包括红细胞外期裂体增殖和红细胞内期裂体增殖。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "疟原虫" } ] }, { "text": "按蚊涎腺内的子孢子经血液进入肝细胞,每个子孢子进入一个肝细胞进行裂体增殖,形成潜隐子(裂殖子)。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "血液" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肝细胞" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肝细胞" } ] }, { "text": "被寄生的肝细胞破裂后,一部分潜隐子被吞噬细胞所消灭,一部分进入红细胞内寄生。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "肝细胞" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "吞噬细胞" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "红细胞" } ] }, { "text": "部分红细胞内期的裂殖子不再进行无性分裂,而是发育成雌的或雄的配子体。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "红细胞" } ] }, { "text": "【流行病学】疟疾在我国分布很广,全国大致可分为四个区:①黄河以北为低疟区,多为间日疟,少数三日疟,无恶性疟;②黄河与长江之间为中度疟区,间日疟多于恶性疟;③长江以南至沿海为高疟区,各种疟疾都有;④西北地区是高寒干热地带,为全国疟疾最轻地区。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "疟疾" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "间日疟" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "三日疟" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "恶性疟" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "间日疟" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "恶性疟" }, { "start_idx": 92, "end_idx": 93, "type": "dis", "entity": "疟疾" }, { "start_idx": 113, "end_idx": 114, "type": "dis", "entity": "疟疾" } ] }, { "text": "按蚊是疟原虫的终末宿主,也是传播疟疾的媒介。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "mic", "entity": "疟原虫" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "疟疾" } ] }, { "text": "【病理生理】引起疟疾临床症状主要是红细胞内期原虫。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "疟疾" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "红细胞" } ] }, { "text": "裂体增殖过程中,疟原虫的代谢产物——血红蛋白及破坏的红细胞碎片,进入人体血流,刺激体温中枢而引起人的发热及其他有关症状。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "疟原虫" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "红细胞" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "血流" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "体温中枢" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "发热" } ] }, { "text": "人体内残存的疟原虫产生抗原变异,在克服人体免疫后,重新大量繁殖,再次出现临床发作,称为再燃;间日疟因存在“红外期”,当产生的潜隐子重新侵入红细胞内繁殖时,又出现临床症状,称为复发。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "疟原虫" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "间日疟" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "红细胞" } ] }, { "text": "再燃和复发与人体免疫力降低及疟原虫虫株有关。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "人体免疫力降低" }, { "start_idx": 14, "end_idx": 16, "type": "mic", "entity": "疟原虫" } ] }, { "text": "【临床表现】(一)潜伏期间日疟的潜伏期为10~12日,三日疟为14~25日或更长,恶性疟为9~16日。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "间日疟" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "三日疟" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "恶性疟" } ] }, { "text": "潜伏期可伴有低热,乏力,全身酸痛等症状。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "低热" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "乏力" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "全身酸痛" } ] }, { "text": "(二)寒战期突然发作,畏寒,全身发抖,关节肌肉酸痛面色苍白,发绀,儿童常有抽搐。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "寒战" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "畏寒" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "全身发抖" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "关节" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肌肉" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "关节肌肉酸痛" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "面色苍白" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "发绀" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "抽搐" } ] }, { "text": "(三)高热期寒战过后,体温迅速升高达39~41℃,患儿面红耳热,结膜充血,头痛,烦躁不安,常发生谵妄,抽搐,昏迷。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "高热" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "寒战" }, { "start_idx": 11, "end_idx": 23, "type": "sym", "entity": "体温迅速升高达39~41℃" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "面红耳热" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "结膜充血" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "头痛" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "谵妄" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "抽搐" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "昏迷" } ] }, { "text": "(四)出汗退热期患儿全身大汗淋漓,皮肤变冷1~2小时内体温降至37℃以下。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "出汗退热" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "全身大汗淋漓" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "皮肤" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "皮肤变冷" }, { "start_idx": 21, "end_idx": 35, "type": "sym", "entity": "1~2小时内体温降至37℃以下" } ] }, { "text": "(五)周期性发作上述急性发作是周期性的。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "周期性发作" } ] }, { "text": "间歇时间因各型疟原虫在红细胞内进行裂体增殖所需时间的长短而不同。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "疟原虫" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "红细胞" } ] }, { "text": "恶性疟最严重,常出现以下凶险类型:1.脑型疟有高热,谵语,抽搐,昏迷,脑膜刺激征阳性。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "恶性疟" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "脑型疟" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "高热" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "谵语" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "抽搐" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "昏迷" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "脑膜刺激征阳性" } ] }, { "text": "2.胆汁型高热,黄疸,肝脾肿大,昏迷。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "高热" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "黄疸" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "昏迷" } ] }, { "text": "3.黑尿热型高热,葡萄酒样尿,黄疸,贫血,无尿等。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "高热" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "葡萄酒样尿" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "黄疸" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "贫血" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "无尿" } ] }, { "text": "4.休克虚脱型体温不升,血压下降,脉搏微弱。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "休克虚脱" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "体温不升" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "血压下降" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "脉搏微弱" } ] }, { "text": "另外,婴幼儿疟疾常不定型,各期不明显,易误诊。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "婴幼儿疟疾" } ] }, { "text": "(六)先天性疟疾指出生后不超过感染子孢子的潜伏期(间日疟为13~15天,三日疟为28~30天),并在疟疾流行区,在没有按蚊感染或输血等情况下发生。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "先天性疟疾" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "间日疟" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "三日疟" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "疟疾" }, { "start_idx": 64, "end_idx": 65, "type": "pro", "entity": "输血" } ] }, { "text": "其母孕期有疟疾史,若婴儿的疟原虫与母体的属同一型,则为先天性疟疾。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "疟疾" }, { "start_idx": 13, "end_idx": 15, "type": "mic", "entity": "疟原虫" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "先天性疟疾" } ] }, { "text": "(七)慢性疟疾多为重复感染或未正规治疗的结果,主要症状是精神不振,贫血,肝脾肿大,血液中可找到疟原虫。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "慢性疟疾" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "精神不振" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "贫血" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "血液" }, { "start_idx": 47, "end_idx": 49, "type": "mic", "entity": "疟原虫" }, { "start_idx": 41, "end_idx": 49, "type": "sym", "entity": "血液中可找到疟原虫" } ] }, { "text": "【并发症】(一)支气管炎或肺炎(二)肾炎多见于长期患疟疾的儿童(三)血红蛋白尿又称“黑尿热”,多发生在恶性疟疾。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "支气管炎" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "肺炎" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "肾炎" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "疟疾" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "血红蛋白尿" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "黑尿热" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "恶性疟疾" } ] }, { "text": "以前认为是疟原虫的毒素引起的溶血,现多数学者认为是患者的红细胞有遗传缺陷,主要是6-磷葡萄糖脱氢酶的含量减少,使氧化型谷胱甘肽还原不足,在服用抗疟药(如奎宁、伯氨喹)和某些退热药(如阿司匹林)后,红细胞在血管内大量溶解而发生血红蛋白尿。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "mic", "entity": "疟原虫" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "溶血" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "红细胞" }, { "start_idx": 40, "end_idx": 53, "type": "sym", "entity": "6-磷葡萄糖脱氢酶的含量减少" }, { "start_idx": 56, "end_idx": 62, "type": "bod", "entity": "氧化型谷胱甘肽" }, { "start_idx": 71, "end_idx": 73, "type": "dru", "entity": "抗疟药" }, { "start_idx": 76, "end_idx": 77, "type": "dru", "entity": "奎宁" }, { "start_idx": 79, "end_idx": 81, "type": "dru", "entity": "伯氨喹" }, { "start_idx": 86, "end_idx": 88, "type": "dru", "entity": "退热药" }, { "start_idx": 91, "end_idx": 94, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 98, "end_idx": 100, "type": "bod", "entity": "红细胞" }, { "start_idx": 102, "end_idx": 103, "type": "bod", "entity": "血管" }, { "start_idx": 112, "end_idx": 116, "type": "sym", "entity": "血红蛋白尿" } ] }, { "text": "【实验室诊断】(一)末梢血涂片找疟原虫(二)骨髓涂片(三)疟原虫的免疫诊断方法1.抗体的检测间接荧光抗体试验(IFAT)为当前国内外应用最广的一种检测疟原虫抗体的方法,另外还有间接红细胞凝集试验(IHA)、酶联免疫吸附试验(ELISA)。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "末梢血涂片" }, { "start_idx": 16, "end_idx": 18, "type": "mic", "entity": "疟原虫" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "骨髓涂片" }, { "start_idx": 29, "end_idx": 31, "type": "mic", "entity": "疟原虫" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "抗体" }, { "start_idx": 46, "end_idx": 53, "type": "pro", "entity": "间接荧光抗体试验" }, { "start_idx": 55, "end_idx": 58, "type": "pro", "entity": "IFAT" }, { "start_idx": 73, "end_idx": 79, "type": "pro", "entity": "检测疟原虫抗体" }, { "start_idx": 88, "end_idx": 96, "type": "pro", "entity": "间接红细胞凝集试验" }, { "start_idx": 98, "end_idx": 100, "type": "pro", "entity": "IHA" }, { "start_idx": 103, "end_idx": 110, "type": "pro", "entity": "酶联免疫吸附试验" }, { "start_idx": 112, "end_idx": 116, "type": "pro", "entity": "ELISA" } ] }, { "text": "2.抗原的检测放射免疫试验和酶联免疫吸附试验。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "放射免疫试验" }, { "start_idx": 14, "end_idx": 21, "type": "pro", "entity": "酶联免疫吸附试验" } ] }, { "text": "3.DNA探针杂交试验。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "DNA探针杂交试验" } ] }, { "text": "(四)用基因工程原理重组的蛋白质或多肽作为抗原诊断疟疾【治疗】(一)一般治疗发作时卧床休息,应进食营养丰富、富含维生素、易消化的食物。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "基因工程" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "疟疾" }, { "start_idx": 38, "end_idx": 44, "type": "pro", "entity": "发作时卧床休息" }, { "start_idx": 47, "end_idx": 65, "type": "pro", "entity": "进食营养丰富、富含维生素、易消化的食物" } ] }, { "text": "对贫血者给予铁剂,严重者输血。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "贫血" }, { "start_idx": 6, "end_idx": 7, "type": "dru", "entity": "铁剂" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "输血" } ] }, { "text": "(二)抗疟药物治疗根据抗疟药物的性能和作用,大致分为三类:主要用于控制临床症状,如氯喹、奎宁、青蒿素等,是杀灭滋养体及裂殖体的有效药物;主要用于复发和传播,如伯氨喹、扑疟喹,是杀灭肝内的潜隐子(红细胞外期)及配子体的特效药;主要用于预防疟疾的感染,如乙胺嘧啶是孢子增殖杀灭剂,对红细胞内期裂殖体有抑制作用。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "抗疟药物" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "抗疟药物" }, { "start_idx": 41, "end_idx": 42, "type": "dru", "entity": "氯喹" }, { "start_idx": 44, "end_idx": 45, "type": "dru", "entity": "奎宁" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "青蒿素" }, { "start_idx": 79, "end_idx": 81, "type": "dru", "entity": "伯氨喹" }, { "start_idx": 83, "end_idx": 85, "type": "dru", "entity": "扑疟喹" }, { "start_idx": 90, "end_idx": 90, "type": "bod", "entity": "肝" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "红细胞" }, { "start_idx": 118, "end_idx": 119, "type": "dis", "entity": "疟疾" }, { "start_idx": 125, "end_idx": 128, "type": "dru", "entity": "乙胺嘧啶" }, { "start_idx": 139, "end_idx": 141, "type": "bod", "entity": "红细胞" } ] }, { "text": "1.氯喹一般常规为3日疗法,首次量为10mg/kg(最大量不超过600mg),6小时后再服1次,5mg/kg;24小时后再服用5mg/kg;48小时后服最后1次5mg/kg;不良反应有恶心、呕吐、白细胞减少等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dru", "entity": "氯喹" }, { "start_idx": 92, "end_idx": 93, "type": "sym", "entity": "恶心" }, { "start_idx": 95, "end_idx": 96, "type": "sym", "entity": "呕吐" }, { "start_idx": 98, "end_idx": 102, "type": "sym", "entity": "白细胞减少" } ] }, { "text": "2.伯氨喹用于氯喹疗程之后,每片含基质7.5mg。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "伯氨喹" }, { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "氯喹" } ] }, { "text": "若发生溶血,白细胞减少,或高铁血红蛋白血症,应立即停药。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "溶血" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "白细胞减少" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "高铁血红蛋白血症" } ] }, { "text": "3.青蒿素及其衍生物青蒿素成人剂量为:口服每次0.6g,每天3次,连用3天为一疗程;肌内注射剂量为200~300mg/次,每天1~2次,连用3天。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "青蒿素" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "青蒿素" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "口服" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "肌内注射" } ] }, { "text": "青蒿琥酯片(每片20mg),成人5天疗程总剂量440mg,首剂80mg,6小时后服40mg,以后每天上,下午各40mg,共5天。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "青蒿琥酯片" } ] }, { "text": "复方蒿甲醚是由抗疟新药蒿甲醚和苯芴醇组成的新抗疟复方,在抗氯喹恶性疟流行区,具有广泛的应用前景。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "复方蒿甲醚" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "蒿甲醚" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "苯芴醇" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "抗氯喹恶性疟" } ] }, { "text": "4.乙胺嘧啶主要用于预防,一般与氯喹同用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "乙胺嘧啶" }, { "start_idx": 16, "end_idx": 17, "type": "dru", "entity": "氯喹" } ] }, { "text": "每隔10~14天口服乙胺嘧啶1次,年长儿为25mg,学龄前儿童为12.5mg,同时加氯喹0.25~0.5g。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "口服" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "乙胺嘧啶" }, { "start_idx": 42, "end_idx": 43, "type": "dru", "entity": "氯喹" } ] }, { "text": "(三)对脑型疟疾的救治脑型疟疾大都是恶性疟原虫引起,因此,对恶性疟要早诊早治,药物以青蒿素类药为首选。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "脑型疟疾" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "脑型疟疾" }, { "start_idx": 18, "end_idx": 22, "type": "mic", "entity": "恶性疟原虫" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "恶性疟" }, { "start_idx": 42, "end_idx": 46, "type": "dru", "entity": "青蒿素类药" } ] }, { "text": "【预防】措施有:①治疗带疟原虫的患者,消灭传染源;②消灭疟疾的传播媒介按蚊;③个人预防,避免蚊虫叮咬;④疟疾疫苗;⑤旅行疫区应用预防剂,服药应自进入流行区前1周开始,并持续到离开流行区后6~8周。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "mic", "entity": "疟原虫" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "消灭传染源" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "疟疾" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "个人预防" }, { "start_idx": 44, "end_idx": 49, "type": "pro", "entity": "避免蚊虫叮咬" }, { "start_idx": 52, "end_idx": 55, "type": "dru", "entity": "疟疾疫苗" }, { "start_idx": 64, "end_idx": 66, "type": "dru", "entity": "预防剂" } ] }, { "text": "如乙胺嘧啶。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "乙胺嘧啶" } ] }, { "text": "第十四章常见小儿外科消化系统疾病第一节幽门肥厚性狭窄幽门肥厚性狭窄(hypertrophicpyloricstenosis,HPS)是因幽门环状括约肌增厚幽门管延长正常结构消失,导致胃出口部梗阻胃代偿性扩张、肥厚和蠕动加快幽门平滑肌细胞肥大,而非增生幽门肥厚性狭窄的临床及病理表现,认为该病是一种先天性疾病。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dep", "entity": "外科" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "消化系统疾病" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "幽门肥厚性狭窄" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "幽门肥厚性狭窄" }, { "start_idx": 35, "end_idx": 61, "type": "dis", "entity": "hypertrophicpyloricstenosis" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "HPS" }, { "start_idx": 69, "end_idx": 75, "type": "bod", "entity": "幽门环状括约肌" }, { "start_idx": 69, "end_idx": 77, "type": "sym", "entity": "幽门环状括约肌增厚" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "幽门管" }, { "start_idx": 78, "end_idx": 82, "type": "sym", "entity": "幽门管延长" }, { "start_idx": 83, "end_idx": 88, "type": "sym", "entity": "正常结构消失" }, { "start_idx": 92, "end_idx": 94, "type": "bod", "entity": "胃出口" }, { "start_idx": 92, "end_idx": 97, "type": "sym", "entity": "胃出口部梗阻" }, { "start_idx": 98, "end_idx": 98, "type": "bod", "entity": "胃" }, { "start_idx": 98, "end_idx": 111, "type": "sym", "entity": "胃代偿性扩张、肥厚和蠕动加快" }, { "start_idx": 112, "end_idx": 118, "type": "bod", "entity": "幽门平滑肌细胞" }, { "start_idx": 112, "end_idx": 125, "type": "sym", "entity": "幽门平滑肌细胞肥大,而非增生" }, { "start_idx": 126, "end_idx": 132, "type": "dis", "entity": "幽门肥厚性狭窄" } ] }, { "text": "胃空肠吻合术是当时经典的治疗方法,死亡率高达60%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "胃空肠吻合术" } ] }, { "text": "黏膜外幽门成形术是另一种术式,但因为缝合易撕裂水肿的肌肉,导致大出血,疗效也不理想。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "黏膜外幽门成形术" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肌肉" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "大出血" } ] }, { "text": "自从1911年Ramstedt首次放弃缝合肌肉后,幽门环肌切开术成为标准术式。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "缝合肌肉" }, { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "幽门环肌切开术" } ] }, { "text": "【发病率】普通人群中幽门肥厚发病率为0.1%~1%,且有上升趋势,40年前的报告是1/900~1/300;而英国最近调查表明过去几十年以来,发病率已从0.1%~0.2%上升到0.3%~0.8%。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "幽门肥厚" } ] }, { "text": "MayoClinic的大样本人群调查显示:1950~1984年间美国Minnesota州Olmsted郡人群HPS总体发病率为0.26%,但至调查后期,发病率已接近0.5%。", "entities": [ { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "HPS" } ] }, { "text": "另一项前瞻性调查研究发现:随机抽取1400名足月新生儿接受B超检查,9名(0.65%)出现肥厚性幽门狭窄的患儿出生时幽门B超检查均无异常表现。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "B超检查" }, { "start_idx": 45, "end_idx": 51, "type": "dis", "entity": "肥厚性幽门狭窄" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "幽门" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "B超检查" } ] }, { "text": "此外,有这样一种观点:头胎男孩最易患HPS,但未获证实。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "HPS" } ] }, { "text": "幽门狭窄的遗传特征多变,母亲遗传的较父亲多见。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "幽门狭窄" } ] }, { "text": "【病因】HPS的病因尚不清楚,目前有几种假说。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "HPS" } ] }, { "text": "其中之一是胃蠕动和幽门松弛之间不协调胃内压升高时,幽门反而不恰当收缩幽门肌肉代偿性肥厚胃收宫内胃扩张患儿生后发生HPS的报道外,其他均未发现HPS患儿在生后数周内有胃排空异常。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "胃" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "幽门" }, { "start_idx": 5, "end_idx": 17, "type": "sym", "entity": "胃蠕动和幽门松弛之间不协调" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "胃" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "幽门" }, { "start_idx": 18, "end_idx": 33, "type": "sym", "entity": "胃内压升高时,幽门反而不恰当收缩" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "幽门肌肉" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "幽门肌肉代偿性肥厚" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "胃" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "胃收" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "宫内胃扩张" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "HPS" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "HPS" }, { "start_idx": 82, "end_idx": 86, "type": "dis", "entity": "胃排空异常" } ] }, { "text": "有人试图将血胃泌素升高、高胃酸与HPS联系在一起,但未获成功。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "HPS" } ] }, { "text": "事实上,任何原因所致胃排出口梗阻均可引起胃扩张,通过胃泌素使胃酸分泌增加。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "胃排出口梗阻" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "胃泌素" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "胃酸" } ] }, { "text": "有报道指出某些患儿的前列腺素(如PGE2和PGF2-α)水平升高可促使幽门收缩HPS。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "前列腺素" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "PGE2" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "幽门收缩" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "HPS" } ] }, { "text": "但是,临床上接受前列腺素治疗的新生儿,即使出现幽门痉挛性梗阻,亦未继发肌肉肥厚原发性肠神经系统发育异常学说来解释HPS的形成或许更能令人信服。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "前列腺素治疗" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "幽门痉挛性梗阻" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "肌肉肥厚" }, { "start_idx": 39, "end_idx": 50, "type": "dis", "entity": "原发性肠神经系统发育异常" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "HPS" } ] }, { "text": "这是因为一方面免疫组化研究发现,HPS患儿体内神经肽,如生长激素释放因子(GRP)、血管活性多肽(VIP)、胃泌素及P物质等较正常儿童明显减少;另一方面,有研究表明幽门区平滑肌松弛介质NO浓度降低是肥厚性幽门狭窄形成的重要因素。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "HPS" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "神经肽" }, { "start_idx": 28, "end_idx": 35, "type": "bod", "entity": "生长激素释放因子" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "血管活性多肽" }, { "start_idx": 54, "end_idx": 70, "type": "sym", "entity": "胃泌素及P物质等较正常儿童明显减少" }, { "start_idx": 82, "end_idx": 87, "type": "bod", "entity": "幽门区平滑肌" }, { "start_idx": 88, "end_idx": 91, "type": "bod", "entity": "松弛介质" }, { "start_idx": 99, "end_idx": 105, "type": "dis", "entity": "肥厚性幽门狭窄" } ] }, { "text": "正常情况下,幽门环肌、纵肌及肌间神经丛中都存在一定浓度的NO合成酶,而HPS患儿环肌层则缺乏该物质,且伴有ENS轴突形态的异常HPS患儿幽门肌间神经丛及肌肉内神经轴突退化HPS的病理原因是什么,其作用都是暂时的,因为术后罕见复发。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "幽门环肌" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "纵肌" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "肌间神经丛" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "NO合成酶" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "HPS" }, { "start_idx": 53, "end_idx": 62, "type": "sym", "entity": "ENS轴突形态的异常" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "HPS" }, { "start_idx": 68, "end_idx": 74, "type": "bod", "entity": "幽门肌间神经丛" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "肌肉" }, { "start_idx": 79, "end_idx": 84, "type": "sym", "entity": "神经轴突退化" }, { "start_idx": 85, "end_idx": 87, "type": "dis", "entity": "HPS" } ] }, { "text": "【临床表现】典型表现为足月男婴,生后3~6周起出现渐进性、非胆汁样喷射性呕吐食物过敏或胃食管反流,直至出现进食后呕吐这一特征性表现,才想到此病。", "entities": [ { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "胆汁" }, { "start_idx": 25, "end_idx": 37, "type": "sym", "entity": "渐进性、非胆汁样喷射性呕吐" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "食物过敏" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "进食后呕吐" } ] }, { "text": "因此,许多患儿就诊时已有严重脱水,但少见营养不良。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "严重脱水" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "营养不良" } ] }, { "text": "血生化指标提示低氯低钾性代谢性碱中毒,病情严重时可出现反常性酸性尿。", "entities": [ { "start_idx": 7, "end_idx": 17, "type": "dis", "entity": "低氯低钾性代谢性碱中毒" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "反常性酸性尿" } ] }, { "text": "临床可见重度低血糖甚至低血糖性抽搐;肝葡萄糖醛酸转移酶活性下降高非结合胆红素血症,可出现黄疸,但一旦胃出口部梗阻解除即好转。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "重度低血糖" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "低血糖性抽搐" }, { "start_idx": 18, "end_idx": 26, "type": "bod", "entity": "肝葡萄糖醛酸转移酶" }, { "start_idx": 18, "end_idx": 30, "type": "sym", "entity": "肝葡萄糖醛酸转移酶活性下降" }, { "start_idx": 31, "end_idx": 39, "type": "dis", "entity": "高非结合胆红素血症" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "黄疸" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "胃出口部梗阻" } ] }, { "text": "因而有人推测这种现象与胃和肝细胞之间的胃肠激素反馈异常有关。", "entities": [ { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "胃" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肝细胞" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "胃肠激素反馈异常" } ] }, { "text": "【诊断】上腹部触及一小而活动的橄榄样包块触诊方法:两或三个手指轻按上腹,慢慢沿肋缘向肝脏深面触探,接着滑向脐部,此时可感觉到似有橄榄样物在指间滑动。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "上腹" }, { "start_idx": 4, "end_idx": 19, "type": "sym", "entity": "上腹部触及一小而活动的橄榄样包块" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "触诊" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "上腹" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肋缘" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "脐部" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "橄榄样物" } ] }, { "text": "患儿保持安静合作是触诊探及包块的关键,有时可能耗时较长,甚至15~20分钟,才能摸到肥厚幽门。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "触诊" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "肥厚幽门" } ] }, { "text": "有时需置鼻胃管,抽出胃内容物,拔除胃管后再行触诊。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "equ", "entity": "鼻胃管" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "胃内容物" }, { "start_idx": 17, "end_idx": 18, "type": "equ", "entity": "胃管" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "触诊" } ] }, { "text": "若清楚触及肥厚幽门,即可明确诊断,无需其他检查。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "肥厚幽门" } ] }, { "text": "如果未能触及肥厚幽门,而临床症状典型,需进一步行影像学检查以明确之。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "肥厚幽门" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "影像学检查" } ] }, { "text": "诊断HPS的B超影像学指标由Teele和Smit提出,包括:幽门直径≥17mm、幽门壁厚度≥4mm幽门管长度≥17mm,其中后两项更常用。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "HPS" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "B超影像" }, { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "幽门直径" }, { "start_idx": 30, "end_idx": 38, "type": "sym", "entity": "幽门直径≥17mm" }, { "start_idx": 40, "end_idx": 43, "type": "ite", "entity": "幽门壁厚" }, { "start_idx": 40, "end_idx": 48, "type": "sym", "entity": "幽门壁厚度≥4mm" }, { "start_idx": 49, "end_idx": 53, "type": "ite", "entity": "幽门管长度" }, { "start_idx": 49, "end_idx": 58, "type": "sym", "entity": "幽门管长度≥17mm" } ] }, { "text": "30天以内婴儿如果壁厚达3mm就可诊断;若数据模棱两可(如壁厚2~3mm,管长12~16mm测量幽门容积,尤其是在小龄儿中。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "壁厚达3mm" }, { "start_idx": 29, "end_idx": 30, "type": "ite", "entity": "壁厚" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "壁厚2~3mm" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "管长" }, { "start_idx": 37, "end_idx": 45, "type": "sym", "entity": "管长12~16mm" }, { "start_idx": 46, "end_idx": 51, "type": "pro", "entity": "测量幽门容积" } ] }, { "text": "如果B超无法明确诊断,需行上消化道钡餐检查。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "B超" }, { "start_idx": 13, "end_idx": 20, "type": "pro", "entity": "上消化道钡餐检查" } ] }, { "text": "透视下可见幽门管狭窄、延长两侧肥厚幽门壁呈特征性的唇样结构凸入胃腔HPS患儿病程尚短,无脱水表现,血浆电解质、血糖水平及尿量正常,可立即手术。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "幽门管" }, { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "幽门管狭窄、延长" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "肥厚幽门壁" }, { "start_idx": 13, "end_idx": 32, "type": "sym", "entity": "两侧肥厚幽门壁呈特征性的唇样结构凸入胃腔" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "HPS" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "脱水" }, { "start_idx": 49, "end_idx": 53, "type": "ite", "entity": "血浆电解质" }, { "start_idx": 55, "end_idx": 56, "type": "ite", "entity": "血糖" }, { "start_idx": 60, "end_idx": 61, "type": "ite", "entity": "尿量" } ] }, { "text": "但许多患儿就诊时已存在较严重的脱水、低糖及低钾等,需术前积极准备24~48小时,调节水电解质平衡。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "脱水" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "低糖" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "水电解质" } ] }, { "text": "首先应立即静脉推注等渗Ringer液(10~20ml/kg),纠治严重脱水所致低灌注情况,直至血流动力学指标恢复正常。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "立即静脉推注" }, { "start_idx": 9, "end_idx": 17, "type": "dru", "entity": "等渗Ringer液" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "严重脱水" }, { "start_idx": 47, "end_idx": 53, "type": "ite", "entity": "血流动力学指标" } ] }, { "text": "轻、中度的水电解质失衡可静脉输注5%葡萄糖与0.45%NaCl混合液,速度为维持速度的1.5倍。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "dis", "entity": "轻、中度的水电解质失衡" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "静脉输注" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 27, "end_idx": 33, "type": "dru", "entity": "NaCl混合液" } ] }, { "text": "因患儿多有低钾和低氯,维持液中应常规添加氯化钾(浓度20~40mmol/L)。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "低钾" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "低氯" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "氯化钾" } ] }, { "text": "需要特别指出的是,碱中毒时,机体细胞外钾代偿性转移至细胞内,因此测得血钾水平低于体内实际水平。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "碱中毒" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "机体细胞" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "钾" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "细胞内" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "血钾水平" } ] }, { "text": "一旦循环总量、尿量、血钾及血氯恢复正常,血浆浓度低于30mmol/L,即可安全手术。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "循环总量" }, { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "尿量" }, { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "血氯" }, { "start_idx": 20, "end_idx": 23, "type": "ite", "entity": "血浆浓度" } ] }, { "text": "术中正确判断十二指肠边界很重要,因十二指肠黏液呈唇样向幽门远端凸出,若切口过分靠近幽门远端,一旦切入过深,可能损伤十二指肠。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "十二指肠" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "十二指肠黏液" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "幽门" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "幽门" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "十二指肠" } ] }, { "text": "无并发症的H.Pyloris术后处理比较简单,6~8小时可进少量糖水,每隔3小时逐渐增加量与浓度,直至开奶。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "pro", "entity": "H.Pyloris术" } ] }, { "text": "手术48小时后仍持续呕吐者少见,此时应考虑是否存在环肌切开不彻底或穿孔可能,消化道造影或B超检查对明确环肌切开是否彻底意义不大,因为环肌切开术前后的幽门超声与其他影像学表现基本相同,但造影可排除黏膜破裂致幽门内液体外溢压迫胃出口可能。", "entities": [ { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "环肌切开不彻底" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "穿孔" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "消化道造影" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "B超检查" }, { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "环肌切开" }, { "start_idx": 66, "end_idx": 70, "type": "pro", "entity": "环肌切开术" }, { "start_idx": 74, "end_idx": 77, "type": "pro", "entity": "幽门超声" }, { "start_idx": 92, "end_idx": 93, "type": "pro", "entity": "造影" }, { "start_idx": 97, "end_idx": 100, "type": "dis", "entity": "黏膜破裂" }, { "start_idx": 102, "end_idx": 105, "type": "bod", "entity": "幽门内液" }, { "start_idx": 111, "end_idx": 113, "type": "bod", "entity": "胃出口" } ] }, { "text": "如果既无穿孔又无完全性梗阻,应考虑可能存在环肌切开不全引起的不全梗阻,此时应至少等待2周再做探查。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "穿孔" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "完全性梗阻" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "环肌切开" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "不全梗阻" } ] }, { "text": "充分的术前准备与补液治疗,专业麻醉与标准术式,大大降低了该病死亡率。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "补液治疗" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "麻醉" } ] }, { "text": "虽然不同作者报道的十二指肠穿孔率从3%~30%不等,但少有超过10%。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "十二指肠穿孔" } ] }, { "text": "切口感染与裂开曾是严重并发症,如今已相当少见。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "切口感染" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "裂开" } ] }, { "text": "研究表明:曾经接受幽门环肌切开术的HPS患儿,数十年后,其胃排空及腹部情况无异于正常对照组。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "幽门环肌切开术" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "HPS" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "胃" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "腹部" } ] }, { "text": "第五节腹膜透析腹膜透析(peritonealdialysis,PD)是肾替代疗法的重要形式之一,主要用于儿童的急、慢性肾衰竭,急性药物或毒物中毒,严重的感染及败血症等疾病的治疗。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 12, "end_idx": 29, "type": "pro", "entity": "peritonealdialysis" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "PD" }, { "start_idx": 35, "end_idx": 39, "type": "pro", "entity": "肾替代疗法" }, { "start_idx": 55, "end_idx": 61, "type": "dis", "entity": "急、慢性肾衰竭" }, { "start_idx": 63, "end_idx": 71, "type": "dis", "entity": "急性药物或毒物中毒" }, { "start_idx": 76, "end_idx": 77, "type": "dis", "entity": "感染" }, { "start_idx": 79, "end_idx": 81, "type": "dis", "entity": "败血症" } ] }, { "text": "儿童腹膜对于中小分子物质的通透性与成人相似,但由于儿童腹膜面积相对较大,又无成年人血管硬化所致腹膜毛细血管的改变,因此儿童腹膜透析对溶质清除较成人充分。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "腹膜" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "腹膜" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "血管硬化" }, { "start_idx": 47, "end_idx": 52, "type": "bod", "entity": "腹膜毛细血管" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "腹膜透析" } ] }, { "text": "1978年,加拿大的一位3岁女孩成为世界上第一位接受连续性非卧床腹膜透析(CAPD)治疗的患儿,1981年当循环腹膜透析机治疗成人后,很快用于患儿,它能更有效地清除尿毒症的毒素及水分,纠正水电解质紊乱及代谢性酸中毒,并为患儿创造了等待肾脏移植的机会。", "entities": [ { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "CAPD" }, { "start_idx": 54, "end_idx": 60, "type": "equ", "entity": "循环腹膜透析机" }, { "start_idx": 82, "end_idx": 84, "type": "dis", "entity": "尿毒症" }, { "start_idx": 86, "end_idx": 87, "type": "mic", "entity": "毒素" }, { "start_idx": 94, "end_idx": 106, "type": "sym", "entity": "水电解质紊乱及代谢性酸中毒" }, { "start_idx": 117, "end_idx": 120, "type": "pro", "entity": "肾脏移植" } ] }, { "text": "根据北美、欧洲等许多发达国家报道,已经成为15岁以下慢性肾衰患儿最常用的透析方式。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "慢性肾衰" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "透析" } ] }, { "text": "一、腹膜透析的适应证和禁忌证(一)适应证1.急性肾衰竭有以下指征之一,即应早期积极透析:①出现尿毒症症状,如恶心、呕吐和嗜睡;②有重度水肿、心力衰竭、胸腹水等严重水、钠潴留;③血钾>6.5mmol/L;④血BUN>28.4mmol/L;⑤血二氧化碳结合力<7mmol/L。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "尿毒症" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "恶心" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "呕吐" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "嗜睡" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "水肿" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 75, "end_idx": 77, "type": "sym", "entity": "胸腹水" }, { "start_idx": 83, "end_idx": 85, "type": "sym", "entity": "钠潴留" }, { "start_idx": 88, "end_idx": 89, "type": "ite", "entity": "血钾" }, { "start_idx": 102, "end_idx": 105, "type": "ite", "entity": "血BUN" }, { "start_idx": 119, "end_idx": 126, "type": "ite", "entity": "血二氧化碳结合力" } ] }, { "text": "2.慢性肾衰竭患儿出现以下临床和生化指征时,要考虑腹膜透析:①出现少尿、肾性贫血或中度以上高血压;②出现明显水肿、腹水等水钠潴留表现;③严重恶心、呕吐等尿毒症症状;④肾小球滤过率GFR<5~10ml/(min•1.73m2)。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "慢性肾衰竭" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "少尿" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "肾性贫血" }, { "start_idx": 45, "end_idx": 47, "type": "sym", "entity": "高血压" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "腹水" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "水钠潴留" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "恶心" }, { "start_idx": 73, "end_idx": 74, "type": "sym", "entity": "呕吐" }, { "start_idx": 76, "end_idx": 78, "type": "dis", "entity": "尿毒症" }, { "start_idx": 83, "end_idx": 85, "type": "bod", "entity": "肾小球" }, { "start_idx": 89, "end_idx": 91, "type": "ite", "entity": "GFR" } ] }, { "text": "3.各种原因所致严重水、电解质紊乱,经一般治疗无效。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "水、电解质紊乱" } ] }, { "text": "4.多种毒物或药物中毒,严重感染合并多脏器衰竭等。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "毒物或药物中毒" } ] }, { "text": "(二)禁忌证1.绝对禁忌证①腹腔内广泛粘连;②腹腔或腹壁广泛感染;③严重胀气或大胀气或大量腹水.相对禁忌证①腹部手术3天内;②腹腔内有局限性炎性病灶,而腹透可使炎症扩散;③长期蛋白质和热量摄入不足者;④严重的呼吸功能不全。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "腹腔" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "胀气或大量腹水" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "腹部" }, { "start_idx": 76, "end_idx": 77, "type": "pro", "entity": "腹透" }, { "start_idx": 80, "end_idx": 81, "type": "dis", "entity": "炎症" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "蛋白质" }, { "start_idx": 104, "end_idx": 109, "type": "sym", "entity": "呼吸功能不全" } ] }, { "text": "第三节期前收缩期前收缩由异位起搏点发出冲动所致。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "期前收缩" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "期前收缩" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "异位起搏点" } ] }, { "text": "在某些情况下,期前收缩可由器质性心脏病(炎症、缺血、纤维化等)或引起,特别是洋地黄类。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "期前收缩" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "心脏病" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "炎症" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "缺血" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "纤维化" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "洋地黄类" } ] }, { "text": "如房性期前收缩落在前一QRS波群的不应期,则其后无QRS波群。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "房性期前收缩" } ] }, { "text": "房性期前收缩必须与室性期前收缩区别。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "房性期前收缩" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "室性期前收缩" } ] }, { "text": "房性期前收缩时,提前出现的QRS波群前有P波,其形态与正常窦性P波不同。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "房性期前收缩" } ] }, { "text": "房性期前收缩常重新调整窦房结起搏点计时,因而其后代偿间隙不完全。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "房性期前收缩" } ] }, { "text": "室性期前收缩常有完全性代偿间隙,但这不是可靠的鉴别标准。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "室性期前收缩" } ] }, { "text": "如形态不一,且联律间期不等,则称之为多源性室性期前收缩。", "entities": [ { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "多源性室性期前收缩" } ] }, { "text": "室性期前收缩后常为完全性代偿间隙。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "室性期前收缩" } ] }, { "text": "室性融合波的存在也提示着期前收缩起源于心室。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "期前收缩" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心室" } ] }, { "text": "室性期前收缩时搏量减少,如期前收缩提得很前,听诊器可听不到,桡动脉处也不能扪及。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "室性期前收缩" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "期前收缩" }, { "start_idx": 22, "end_idx": 24, "type": "equ", "entity": "听诊器" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "桡动脉" } ] }, { "text": "第十章生殖细胞性肿瘤生殖细胞性肿瘤(germcelltumor)是指由原始多潜能生殖细胞在分化、成熟和移行过程中形成的一组肿瘤,其原发部位及临床表现不一。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "生殖细胞性肿瘤" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "生殖细胞性肿瘤" }, { "start_idx": 18, "end_idx": 30, "type": "dis", "entity": "germcelltumor" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "生殖细胞" }, { "start_idx": 61, "end_idx": 62, "type": "dis", "entity": "肿瘤" } ] }, { "text": "15岁以下儿童生殖细胞性肿瘤发病率为2.4/100万,约占同年龄组儿童肿瘤的1%。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "儿童生殖细胞性肿瘤" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "【起源】胚胎发育第4周在卵黄囊区可见未分化的、无性别差异的胚胎性生殖腺,此后原始的生殖腺从卵黄囊移行至后腹膜的生殖脊,受性染色体信息指令调控发育成熟为卵巢或睾丸并渐分别下降至盆腔、阴囊。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "胚胎" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "卵黄囊区" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "生殖腺" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "生殖腺" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "卵黄囊" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "腹膜" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "生殖脊" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "性染色体" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "卵巢" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "睾丸" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "盆腔" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "阴囊" } ] }, { "text": "原始生殖腺可发生异位移行,如移行至松果体、纵隔、后腹膜、骶尾部等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "原始生殖腺" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "松果体" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "纵隔" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "后腹膜" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "骶尾部" } ] }, { "text": "因此生殖细胞性肿瘤可发生于任何一个原始生殖腺正常或异位移行的部位。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "生殖细胞性肿瘤" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "原始生殖腺" } ] }, { "text": "(图11-4)示生殖细胞性肿瘤的组织起源。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "生殖细胞性肿瘤" } ] }, { "text": "图11-4生殖细胞性肿瘤的组织起源【病理】(一)组织学分类儿童性腺及性腺外生殖细胞性肿瘤的组织学分类如下。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "生殖细胞性肿瘤" }, { "start_idx": 29, "end_idx": 43, "type": "dis", "entity": "儿童性腺及性腺外生殖细胞性肿瘤" } ] }, { "text": "1.卵巢原发(1)生殖细胞性:无性生殖细胞性;内胚窦瘤(卵黄囊瘤);畸胎瘤(成熟、未成熟、恶性);胚胎性癌;恶性混合性生殖细胞瘤;多胚瘤;绒毛膜癌;生殖腺母细胞瘤。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "卵巢" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "内胚窦瘤" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "卵黄囊瘤" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "畸胎瘤" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "胚胎性癌" }, { "start_idx": 54, "end_idx": 63, "type": "dis", "entity": "恶性混合性生殖细胞瘤" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "多胚瘤" }, { "start_idx": 69, "end_idx": 72, "type": "dis", "entity": "绒毛膜癌" }, { "start_idx": 74, "end_idx": 80, "type": "dis", "entity": "生殖腺母细胞瘤" } ] }, { "text": "2.睾丸原发(1)生殖细胞性:内胚窦瘤(卵黄囊瘤);畸胎瘤(成熟、未成熟、恶性);胚胎性癌;生殖腺母细胞瘤;其他如恶性混合性生殖细胞瘤、精原细胞瘤、绒毛膜癌。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "睾丸" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "内胚窦瘤" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "卵黄囊瘤" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "畸胎瘤" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "胚胎性癌" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "生殖腺母细胞瘤" }, { "start_idx": 57, "end_idx": 66, "type": "dis", "entity": "恶性混合性生殖细胞瘤" }, { "start_idx": 68, "end_idx": 72, "type": "dis", "entity": "精原细胞瘤" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "绒毛膜癌" } ] }, { "text": "(2)非生殖细胞性:性索-基质性肿瘤。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "性索-基质性肿瘤" } ] }, { "text": "3.生殖腺外生殖细胞瘤畸胎瘤(骶尾部、纵隔、后腹膜、松果体等):+/-卵黄囊瘤成分;+/-胚胎性癌成分。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dis", "entity": "生殖腺外生殖细胞瘤畸胎瘤" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "骶尾部" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "纵隔" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "后腹膜" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "松果体" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "+/-卵黄囊瘤" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "+/-胚胎性癌" } ] }, { "text": "(二)病理学特征1.同一类型的生殖细胞性肿瘤在生殖腺内和生殖腺外不同部位的病理形态学表现一致。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "生殖细胞性肿瘤" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "生殖腺" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "生殖腺" } ] }, { "text": "2.肿瘤可同时包含良性和恶性成分,其恶性成分决定它的临床特征。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" } ] }, { "text": "同一肿瘤的不同部位有不同的细胞成分,因此需作多层次病理切片以避免诊断误差。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "细胞" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "病理切片" } ] }, { "text": "【临床表现】生殖细胞性肿瘤在出生后至青少年期均可发病,但以婴幼儿、学龄前期发病较为多见。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "生殖细胞性肿瘤" } ] }, { "text": "不同的发病部位有不同的临床表现,常因可扪及肿块或增大的肿块产生压迫症状而就诊。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肿块" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肿块" }, { "start_idx": 18, "end_idx": 34, "type": "sym", "entity": "可扪及肿块或增大的肿块产生压迫症状" } ] }, { "text": "当肿瘤原发于骶尾部或盆腔并压迫直肠及尿路时发生大小便习惯改变,骶尾部外形改变或不对称骶尾部睾丸肿块较易早期发现,睾丸不对称肿大、硬卵巢肿块早期无症状而不易发现,常在腹围增大或扪及腹块时才就诊,卵巢肿块扭转可因急腹痛而就诊。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "骶尾部" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "盆腔" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "直肠" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "尿路" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "发生大小便习惯改变" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "骶尾部" }, { "start_idx": 31, "end_idx": 41, "type": "sym", "entity": "骶尾部外形改变或不对称" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "骶尾部" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "睾丸肿块" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "骶尾部睾丸肿块" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "睾丸" }, { "start_idx": 56, "end_idx": 64, "type": "sym", "entity": "睾丸不对称肿大、硬" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "卵巢" }, { "start_idx": 82, "end_idx": 90, "type": "sym", "entity": "腹围增大或扪及腹块" }, { "start_idx": 96, "end_idx": 99, "type": "bod", "entity": "卵巢肿块" }, { "start_idx": 104, "end_idx": 106, "type": "dis", "entity": "急腹痛" } ] }, { "text": "原发于纵隔的肿块可压迫血管或气道,产生上腔静脉压迫综合征及呼吸道压迫症状晚期肿瘤出现全身症状,如消瘦、发热、贫血及胸腹水等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "纵隔" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肿块" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血管" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "气道" }, { "start_idx": 19, "end_idx": 27, "type": "dis", "entity": "上腔静脉压迫综合征" }, { "start_idx": 17, "end_idx": 35, "type": "sym", "entity": "产生上腔静脉压迫综合征及呼吸道压迫症状" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "晚期肿瘤" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "消瘦" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "发热" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "贫血及胸腹水等" } ] }, { "text": "【实验室检查】(一)血液生化检查可作为辅助诊断及疾病的随访。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "血液生化检查" } ] }, { "text": "1.甲胎蛋白(α-FP)αFP是一种胚胎性蛋白,半衰期为5~7天,出生时水平较成人明显高,至1岁时降至成人水平。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "甲胎蛋白" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "α-FP" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "αFP" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "胚胎性蛋白" } ] }, { "text": "肝母细胞瘤、肝癌、肝细胞大量坏死后再生及生殖细胞性肿瘤时升高。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肝母细胞瘤" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肝癌" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肝细胞" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "生殖细胞性肿瘤" } ] }, { "text": "儿童生殖细胞性肿瘤好发部位肿块,伴有明显增高的α-FP提示肿块为生殖细胞性肿瘤可能较大。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "儿童生殖细胞性肿瘤" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肿块" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "α-FP" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肿块" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "生殖细胞性肿瘤" } ] }, { "text": "肿块α-FP组织化学染色阳性提示肿块内有恶性生殖细胞瘤成分。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "肿块α-FP" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "化学染色" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肿块" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "恶性生殖细胞瘤" } ] }, { "text": "随访过程中α-FP的水平可反映肿瘤负荷的变化。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "ite", "entity": "α-FP" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "肿瘤" } ] }, { "text": "对有α-FP增高的病例,在完全缓解和停药后随访α-FP的水平变化是必要的。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "α-FP" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "α-FP" } ] }, { "text": "正常婴儿α-FP的变化范围(表11-11)。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "ite", "entity": "α-FP" } ] }, { "text": "2.β-人绒毛膜促性腺激素(β-HCG)在孕期由胎盘滋养层合胞体细胞合成,在生殖细胞性肿瘤病人中如β-HCG升高表明肿瘤中存在滋养层合胞体细胞克隆,常见于绒毛膜癌、精原细胞瘤或无性生殖细胞瘤,偶见于成人的胚胎性癌。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "ite", "entity": "β-人绒毛膜促性腺激素" }, { "start_idx": 14, "end_idx": 18, "type": "ite", "entity": "β-HCG" }, { "start_idx": 24, "end_idx": 33, "type": "bod", "entity": "胎盘滋养层合胞体细胞" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "生殖细胞性肿瘤" }, { "start_idx": 49, "end_idx": 53, "type": "ite", "entity": "β-HCG" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "肿瘤" }, { "start_idx": 63, "end_idx": 70, "type": "bod", "entity": "滋养层合胞体细胞" }, { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "绒毛膜癌" }, { "start_idx": 82, "end_idx": 86, "type": "dis", "entity": "精原细胞瘤" }, { "start_idx": 88, "end_idx": 94, "type": "dis", "entity": "无性生殖细胞瘤" }, { "start_idx": 102, "end_idx": 105, "type": "dis", "entity": "胚胎性癌" } ] }, { "text": "3.血清乳酸脱氢酶(LDH)可非特异性升高,并与肿瘤负荷成正比。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "ite", "entity": "血清乳酸脱氢酶" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "LDH" }, { "start_idx": 24, "end_idx": 27, "type": "ite", "entity": "肿瘤负荷" } ] }, { "text": "表11-28正常婴儿α-FP值(二)影像学检查特征1.B型超声波骶尾部、睾丸、卵巢、盆腔、后腹膜肿块的大小、质地可由B型超声波检查得到证实,通常肿块密度不匀可伴有液化灶。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "α-FP" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "影像学检查" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "B型超声波" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "骶尾部" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "睾丸" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "卵巢" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "盆腔" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "后腹膜肿块" }, { "start_idx": 58, "end_idx": 62, "type": "pro", "entity": "B型超声波" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "肿块" }, { "start_idx": 72, "end_idx": 77, "type": "sym", "entity": "肿块密度不匀" }, { "start_idx": 78, "end_idx": 83, "type": "sym", "entity": "可伴有液化灶" } ] }, { "text": "畸胎瘤时可有类似骨组织样的强反光团CT或MRI示肿块大而质地不均一,伴有坏死灶或囊性变,畸胎瘤时也可见有密度较高的骨组织。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "畸胎瘤" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "骨组织" }, { "start_idx": 5, "end_idx": 16, "type": "sym", "entity": "有类似骨组织样的强反光团" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "CT" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "MRI" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肿块" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "畸胎瘤" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "骨组织" } ] }, { "text": "【治疗】生殖细胞瘤为一组在年龄、病理组织学类型、原发部位差异较大的疾病,因此在诊断和治疗过程中需因人给予相应的治疗措施。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "生殖细胞瘤" } ] }, { "text": "近年来由于化疗的进展,对手术的指征、时间及切除范围提出了新的建议。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "化疗" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "手术" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "切除" } ] }, { "text": "放疗在生殖细胞性肿瘤中的应用尚未确立。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "放疗" }, { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "生殖细胞性肿瘤" } ] }, { "text": "(一)手术对于局限性肿瘤及良性肿瘤如良性畸胎瘤等主要治疗手段为手术。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "手术" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "局限性肿瘤" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "良性肿瘤" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "良性畸胎瘤" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "手术" } ] }, { "text": "对恶性生殖细胞性肿瘤,估计肿瘤不能完全切除或切除可能损害重要的生命组织结构时,应考虑仅做活检。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "dis", "entity": "恶性生殖细胞性肿瘤" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "肿瘤" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "完全切除" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "切除" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "活检" } ] }, { "text": "在明确诊断后先行化疗,减少肿瘤负荷创造手术条件,待肿块明显缩小至基本能完全切除时再行根除手术。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "化疗" }, { "start_idx": 13, "end_idx": 16, "type": "ite", "entity": "肿瘤负荷" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "手术" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肿块" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "手术" } ] }, { "text": "对于第一次手术未能完全切除者,并在化疗后仍有残留病灶时可考虑第二次手术。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "手术" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "化疗" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "手术" } ] }, { "text": "(二)化疗对于手术不能完全切除的儿童恶性生殖细胞瘤,过去预后很差,近年来由于有效的化疗的介入,预后有了较大的改善。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "化疗" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "手术" }, { "start_idx": 16, "end_idx": 24, "type": "dis", "entity": "儿童恶性生殖细胞瘤" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "化疗" } ] }, { "text": "单药有效的药物包括放线菌素D、长春碱、博来霉素、阿霉素、顺铂、VP-16等,其他较新的药物如卡铂、异环磷酰胺也有效。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "放线菌素D" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "长春碱" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "博来霉素" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "阿霉素" }, { "start_idx": 28, "end_idx": 29, "type": "dru", "entity": "顺铂" }, { "start_idx": 31, "end_idx": 35, "type": "dru", "entity": "VP-16" }, { "start_idx": 46, "end_idx": 47, "type": "dru", "entity": "卡铂" }, { "start_idx": 49, "end_idx": 53, "type": "dru", "entity": "异环磷酰胺" } ] }, { "text": "临床上主要采用联合化疗,以长春碱为基础增加其他有效的化疗药物,结合手术使无病生存率进一步提高,近来顺铂的加入使治愈率明显上升。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "联合化疗" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "长春碱" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "手术" }, { "start_idx": 49, "end_idx": 50, "type": "dru", "entity": "顺铂" } ] }, { "text": "目前常用的联合化疗方案(表11-11)。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "联合化疗" } ] }, { "text": "表11-29生殖细胞瘤常用的联合化疗方案(一般21天为一周期)对于局限性肿瘤如Ⅰ期完全切除的睾丸肿瘤和卵巢肿瘤可以术后不作化疗,定期观察随访。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "生殖细胞瘤" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "联合化疗" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "局限性肿瘤" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "睾丸肿瘤" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "卵巢肿瘤" }, { "start_idx": 61, "end_idx": 62, "type": "pro", "entity": "化疗" } ] }, { "text": "(三)放疗放疗在生殖细胞性肿瘤治疗中的地位未确定。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "放疗" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "放疗" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "生殖细胞性肿瘤" } ] }, { "text": "克-雅病@二、克-雅病(Creutzfeldt-Jakobdisease,CJD)主要见于50~60岁成人,儿童少见。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "克-雅病" }, { "start_idx": 12, "end_idx": 35, "type": "dis", "entity": "Creutzfeldt-Jakobdisease" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "CJD" } ] }, { "text": "克-雅病@散发性发病。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" } ] }, { "text": "克-雅病@起病隐匿,首发症状多为疲倦、抑郁和体重减轻。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "疲倦" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "抑郁" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "体重减轻" } ] }, { "text": "克-雅病@精神异常发生较早,表现为记忆力减退、判断力下降、异常行为和人格改变,提示高级皮层的特异性受累。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "精神异常发生较早" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "记忆力减退" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "判断力下降" }, { "start_idx": 29, "end_idx": 37, "type": "sym", "entity": "异常行为和人格改变" }, { "start_idx": 39, "end_idx": 50, "type": "sym", "entity": "提示高级皮层的特异性受累" } ] }, { "text": "克-雅病@早期最常见的运动症状是协同障碍,常逐渐进展至典型的小脑性共济失调。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "协同障碍" }, { "start_idx": 21, "end_idx": 36, "type": "sym", "entity": "常逐渐进展至典型的小脑性共济失调" } ] }, { "text": "克-雅病@发病数周至数月以内出现其他神经体征,如强直,动作缓慢,静止时面部表情丧失,静止性震颤等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "强直" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "动作缓慢" }, { "start_idx": 32, "end_idx": 40, "type": "sym", "entity": "静止时面部表情丧失" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "静止性震颤" } ] }, { "text": "克-雅病@中期病人出现肌阵挛,常可为突然的感觉性刺激所诱发。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "肌阵挛" }, { "start_idx": 15, "end_idx": 28, "type": "sym", "entity": "常可为突然的感觉性刺激所诱发" } ] }, { "text": "克-雅病@稍晚出现惊厥。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "惊厥" } ] }, { "text": "克-雅病@CJD最常见的特征是痴呆。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "CJD" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "痴呆" } ] }, { "text": "克-雅病@可伴有视觉障碍、感觉障碍以及构音障碍等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "视觉障碍" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "感觉障碍" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "构音障碍" } ] }, { "text": "克-雅病@最终发展至植物状态并很快死亡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 5, "end_idx": 18, "type": "sym", "entity": "最终发展至植物状态并很快死亡" } ] }, { "text": "克-雅病@通常病程为1~15个月,平均6~7个月。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" } ] }, { "text": "克-雅病@CJD的脑电图均有异常发现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "CJD的脑电图" } ] }, { "text": "克-雅病@随病程进展呈进行性慢化,某些病例呈周期性异常,表现为高波幅慢波和尖波暴发,继以相对低平活动。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 31, "end_idx": 40, "type": "sym", "entity": "高波幅慢波和尖波暴发" }, { "start_idx": 42, "end_idx": 49, "type": "sym", "entity": "继以相对低平活动" } ] }, { "text": "克-雅病@脑脊液可仅有轻度蛋白升高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 5, "end_idx": 16, "type": "sym", "entity": "脑脊液可仅有轻度蛋白升高" } ] }, { "text": "克-雅病@本病诊断也有赖于病理检查,病理改变的特点是:大脑皮层各层均有神经细胞脱失,伴明显星形细胞增生,肉眼一般均可见大脑皮层萎缩。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 28, "end_idx": 40, "type": "sym", "entity": "脑皮层各层均有神经细胞脱失" }, { "start_idx": 42, "end_idx": 50, "type": "sym", "entity": "伴明显星形细胞增生" }, { "start_idx": 52, "end_idx": 64, "type": "sym", "entity": "肉眼一般均可见大脑皮层萎缩" } ] }, { "text": "克-雅病@海绵样变在皮质深层特别明显,镜下改变更为显著。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 5, "end_idx": 17, "type": "sym", "entity": "海绵样变在皮质深层特别明显" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "镜下改变更为显著" } ] }, { "text": "克-雅病@小脑改变以颗粒细胞减少为主,伴各层致密纤维胶质增生。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 5, "end_idx": 17, "type": "sym", "entity": "小脑改变以颗粒细胞减少为主" }, { "start_idx": 19, "end_idx": 29, "type": "sym", "entity": "伴各层致密纤维胶质增生" } ] }, { "text": "克-雅病@进展迅速的病例海绵样变更突出,死亡较晚的病例则出现更明显的胶质增生。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" }, { "start_idx": 5, "end_idx": 18, "type": "sym", "entity": "进展迅速的病例海绵样变更突出" }, { "start_idx": 20, "end_idx": 37, "type": "sym", "entity": "死亡较晚的病例则出现更明显的胶质增生" } ] }, { "text": "克-雅病@对本病目前尚缺乏特效治疗,只能给予对症性处理。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" } ] }, { "text": "克-雅病@(秦炯)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克-雅病" } ] }, { "text": "二、乙型肝炎【病原和流行病学】乙型肝炎病毒(hepatitisBvirus,HBV)又称Dane颗粒,属嗜肝DNA病毒科,有双层结构,外层为脂蛋白包膜,含糖蛋白,即表面抗原(HBsAg);内为核衣壳,含核心抗原(HBcAg)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "乙型肝炎" }, { "start_idx": 15, "end_idx": 20, "type": "mic", "entity": "乙型肝炎病毒" }, { "start_idx": 22, "end_idx": 36, "type": "mic", "entity": "hepatitisBvirus" }, { "start_idx": 38, "end_idx": 40, "type": "mic", "entity": "HBV" }, { "start_idx": 52, "end_idx": 58, "type": "mic", "entity": "嗜肝DNA病毒" } ] }, { "text": "后者被分泌入血或定位于感染细胞膜。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "血" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "感染细胞膜" } ] }, { "text": "根据HBsAg亚型抗原决定簇差异性,现已将HBV分为10个亚型,主要亚型为adw、adr、ayw和ayr。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "HBV" }, { "start_idx": 37, "end_idx": 39, "type": "mic", "entity": "adw" }, { "start_idx": 41, "end_idx": 43, "type": "mic", "entity": "adr" }, { "start_idx": 45, "end_idx": 47, "type": "mic", "entity": "ayw" }, { "start_idx": 49, "end_idx": 51, "type": "mic", "entity": "ayr" } ] }, { "text": "我国长江以北adr占优势,长江以南adr和adw混存,少数民族地区以adw为主。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "adr" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "adr" }, { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "adw" }, { "start_idx": 34, "end_idx": 36, "type": "mic", "entity": "adw" } ] }, { "text": "已发现多种HBV变异株,常见preC、C和S区基因变异。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "mic", "entity": "HBV" } ] }, { "text": "HBV对外界环境的抵抗力很强,能耐受干燥、60℃10小时、紫外线照射和一般消毒剂。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "HBV" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "紫外线照射" } ] }, { "text": "煮沸10分钟、高压灭菌法、89-89乙肝消毒剂(2%、5秒钟,0.5%、3~5分钟)、2%过氧乙酸浸泡2分钟可灭活之。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "高压灭菌法" }, { "start_idx": 13, "end_idx": 22, "type": "dru", "entity": "89-89乙肝消毒剂" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "过氧乙酸" } ] }, { "text": "HBV尚不能体外分离培养。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "HBV" } ] }, { "text": "黑猩猩是HBV最理想的实验模型动物。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "mic", "entity": "HBV" } ] }, { "text": "乙型肝炎(viralhepatitisB,简称乙肝),呈全球性流行,我国为高流行区,人群总感染率为35.5%~61.6%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "乙型肝炎" }, { "start_idx": 5, "end_idx": 19, "type": "dis", "entity": "viralhepatitisB" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "乙肝" } ] }, { "text": "约1.2亿人为HBsAg携带者,高峰年龄段为10岁以下和30~40岁组,传染源主要是乙肝患者和HBV携带者,病毒存在肝及肝外组织和血液中,通过各种体液排至体外。", "entities": [ { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "乙肝" }, { "start_idx": 47, "end_idx": 49, "type": "mic", "entity": "HBV" }, { "start_idx": 54, "end_idx": 55, "type": "mic", "entity": "病毒" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "肝" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "肝外组织" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "血液" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "体液" } ] }, { "text": "儿童HBV感染最主要的传播途径为母婴传播。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "儿童HBV感染" } ] }, { "text": "母婴传播率为40%~60%,如果母亲HBsAg和HBeAg双阳性,则高达90%,多发生于产时(经产道感染),宫内传播率为5%~15%,生后经密切接触和食入带病毒的母乳可获HBV感染。", "entities": [ { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "产道" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "产道感染" }, { "start_idx": 78, "end_idx": 79, "type": "mic", "entity": "病毒" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "母乳" }, { "start_idx": 85, "end_idx": 89, "type": "dis", "entity": "HBV感染" } ] }, { "text": "水平传播方式主要包括注射和输注血制品,医疗器材和操作如内镜、牙科或手术器械、血透或腹透等,以及生活密切接触传播如家庭内聚集现象和集体机构内流行。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "注射" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "输注血制品" }, { "start_idx": 27, "end_idx": 28, "type": "equ", "entity": "内镜" }, { "start_idx": 30, "end_idx": 36, "type": "equ", "entity": "牙科或手术器械" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "血透" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "腹透" } ] }, { "text": "【发病机制和病理改变】HBV经皮肤或黏膜进入血液循环,肝脏是其主要靶器官,但在胰腺、胆管上皮、肾小球、血管上皮、骨髓、性腺、胎盘、脾和外周血单个核细胞内都能找到病毒。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "mic", "entity": "HBV" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "皮肤" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "黏膜" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血液" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肝脏" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "靶器官" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "胰腺" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "胆管上皮" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "肾小球" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "血管上皮" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "骨髓" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "性腺" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "胎盘" }, { "start_idx": 65, "end_idx": 65, "type": "bod", "entity": "脾" }, { "start_idx": 67, "end_idx": 74, "type": "bod", "entity": "外周血单个核细胞" }, { "start_idx": 80, "end_idx": 81, "type": "mic", "entity": "病毒" } ] }, { "text": "HBV并不直接损伤靶细胞。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "HBV" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "靶细胞" } ] }, { "text": "目前认为,针对肝细胞表面病毒编码抗原的CTL是构成肝细胞损伤和最终清除病毒的机制。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肝细胞" }, { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "病毒" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肝细胞" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "肝细胞损伤" }, { "start_idx": 35, "end_idx": 36, "type": "mic", "entity": "病毒" } ] }, { "text": "最近在转基因鼠中发现存在非溶细胞性T细胞灭活病毒作用(通过CTL分泌IFN-γ和TNF-α或其他淋巴因子所介导),可能是机体清除病毒的另一途径。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "mic", "entity": "病毒" }, { "start_idx": 64, "end_idx": 65, "type": "mic", "entity": "病毒" } ] }, { "text": "如果病毒抗原不被识别或机体免疫应答不足,则形成持续病毒携带或表现为慢性肝炎;只有当机体产生有效的CTL和中和性抗体,才能清除病毒。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "mic", "entity": "病毒抗原" }, { "start_idx": 25, "end_idx": 26, "type": "mic", "entity": "病毒" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "慢性肝炎" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "抗体" }, { "start_idx": 62, "end_idx": 63, "type": "mic", "entity": "病毒" } ] }, { "text": "围生期或幼龄期感染HBV易发生持续病毒携带或慢性肝炎,与机体对HBV产生免疫耐受有关。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "HBV" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "持续病毒携带" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "慢性肝炎" }, { "start_idx": 31, "end_idx": 33, "type": "mic", "entity": "HBV" } ] }, { "text": "我国学者提出,对HBV包膜抗原(主要是HBsAg)的免疫耐受是其主要耐受机制。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "mic", "entity": "HBV包膜抗原" } ] }, { "text": "HBV可致膜性肾小球肾炎等肝外损害,与HBV抗体形成的免疫复合物性损伤有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "HBV" }, { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "膜性肾小球肾炎" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肝" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "肝外损害" }, { "start_idx": 19, "end_idx": 23, "type": "mic", "entity": "HBV抗体" } ] }, { "text": "乙肝的病理变化主要表现为肝细胞变性(常见水肿性和嗜酸性变)、不同程度坏死(点状、灶状、桥状或碎屑状坏死)和再生。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "乙肝" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肝细胞" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "肝细胞变性" }, { "start_idx": 20, "end_idx": 27, "type": "sym", "entity": "水肿性和嗜酸性变" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "坏死" }, { "start_idx": 37, "end_idx": 50, "type": "sym", "entity": "点状、灶状、桥状或碎屑状坏死" } ] }, { "text": "汇管区和肝实质可见淋巴细胞、单核细胞、浆细胞和组织细胞浸润。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "汇管区" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "肝实质" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "单核细胞" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "浆细胞" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "组织细胞" }, { "start_idx": 9, "end_idx": 28, "type": "sym", "entity": "淋巴细胞、单核细胞、浆细胞和组织细胞浸润" } ] }, { "text": "其他可见淤胆或胆栓形成、胆小管增生、纤维组织增生等。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "淤胆或胆栓形成" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "胆小管" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "胆小管增生" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "纤维组织" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "纤维组织增生" } ] }, { "text": "临床分为5型:急性肝炎、慢性肝炎、重型肝炎、淤胆型肝炎和肝炎肝硬化。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "急性肝炎" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "慢性肝炎" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "重型肝炎" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "淤胆型肝炎" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "肝炎肝硬化" } ] }, { "text": "幼龄或围生期感染易表现为慢性病毒携带状态。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "围生期感染" }, { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "病毒" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "慢性病毒携带" } ] }, { "text": "(一)急性肝炎分为急性无黄疸型和急性黄疸型,临床表现与甲肝类似。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "急性肝炎" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "急性无黄疸型" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "急性黄疸型" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "甲肝" } ] }, { "text": "(二)慢性肝炎急性肝炎病程超过半年或原有乙型肝炎HBsAg携带史,本次因HBV出现肝炎病情者。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "慢性肝炎" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "急性肝炎" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "乙型肝炎" }, { "start_idx": 36, "end_idx": 38, "type": "mic", "entity": "HBV" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "肝炎" } ] }, { "text": "1.轻度症状、体征轻微或缺如,肝功能指标仅1项或2项轻度异常,如ALT≤正常3倍、胆红素≤正常2倍、γ球蛋白≤21%、凝血酶原活动度(PTA)>70%。", "entities": [ { "start_idx": 15, "end_idx": 29, "type": "sym", "entity": "肝功能指标仅1项或2项轻度异常" }, { "start_idx": 32, "end_idx": 34, "type": "ite", "entity": "ALT" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "胆红素" }, { "start_idx": 50, "end_idx": 53, "type": "ite", "entity": "γ球蛋白" }, { "start_idx": 59, "end_idx": 65, "type": "ite", "entity": "凝血酶原活动度" }, { "start_idx": 67, "end_idx": 69, "type": "ite", "entity": "PTA" } ] }, { "text": "肝活检炎症活动度(G)分级为1~2,纤维化程度(S)分期为0~2。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "肝活检炎症活动度" }, { "start_idx": 18, "end_idx": 22, "type": "ite", "entity": "纤维化程度" } ] }, { "text": "肝活检呈G3级、S1~3期。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "肝活检" } ] }, { "text": "3.重度有明显或持续肝炎症状如乏力、食欲缺乏、腹胀等,伴肝掌、蜘蛛痣、脾大但无门脉高压者,ALT反复或持续升高。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肝炎" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "乏力" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "腹胀" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "肝掌" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "蜘蛛痣" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "脾" }, { "start_idx": 35, "end_idx": 42, "type": "sym", "entity": "脾大但无门脉高压" }, { "start_idx": 45, "end_idx": 47, "type": "ite", "entity": "ALT" }, { "start_idx": 45, "end_idx": 54, "type": "sym", "entity": "ALT反复或持续升高" } ] }, { "text": "此外,清蛋白明显下降,TB>正常5倍,PTA为60%~40%,胆碱酯酶<2500U/L,这4项指标中至少有一项符合,肝活检为G4级,S2~4期改变。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "清蛋白明显下降" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "TB" }, { "start_idx": 19, "end_idx": 21, "type": "ite", "entity": "PTA" }, { "start_idx": 31, "end_idx": 34, "type": "ite", "entity": "胆碱酯酶" }, { "start_idx": 58, "end_idx": 60, "type": "ite", "entity": "肝活检" } ] }, { "text": "(三)重型肝炎分为急性、亚急性和慢性重型肝炎。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "重型肝炎" }, { "start_idx": 9, "end_idx": 21, "type": "dis", "entity": "急性、亚急性和慢性重型肝炎" } ] }, { "text": "前两者临床表现同甲型重型肝炎。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "甲型重型肝炎" } ] }, { "text": "慢性重型肝炎是在慢性HBV携带或慢性肝炎或肝硬化基础上发生,起病时临床表现同亚急性重型肝炎,随病情发展而加重,有出血倾向(PTA<40%),黄疸加深(TB>正常10倍),腹水、肝性脑病等重症表现。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "慢性重型肝炎" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "慢性HBV携带" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "慢性肝炎" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "肝硬化" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "亚急性重型肝炎" }, { "start_idx": 61, "end_idx": 63, "type": "ite", "entity": "PTA" }, { "start_idx": 70, "end_idx": 71, "type": "dis", "entity": "黄疸" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "黄疸加深" }, { "start_idx": 75, "end_idx": 76, "type": "ite", "entity": "TB" }, { "start_idx": 85, "end_idx": 86, "type": "sym", "entity": "腹水" }, { "start_idx": 88, "end_idx": 91, "type": "dis", "entity": "肝性脑病" } ] }, { "text": "肝活检可见慢性肝病病变背景上出现大块性或亚大块性新鲜肝实质坏死。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "肝活检" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "慢性肝病" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "肝实质" }, { "start_idx": 16, "end_idx": 30, "type": "sym", "entity": "大块性或亚大块性新鲜肝实质坏死" } ] }, { "text": "(四)淤胆型肝炎可分为急性淤胆型和慢性淤胆型。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "淤胆型肝炎" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "急性淤胆型" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "慢性淤胆型" } ] }, { "text": "临床表现同甲型淤胆型肝炎,但慢性淤胆型发生在慢性肝炎基础上,黄疸持续时间更长,预后较急性淤胆型差。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "甲型淤胆型肝炎" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "慢性淤胆型" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "慢性肝炎" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "黄疸" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "急性淤胆型" } ] }, { "text": "(五)肝炎肝硬化是慢性肝炎的发展结果。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肝炎肝硬化" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "慢性肝炎" } ] }, { "text": "据肝活检报告,儿童HBV相关性肝硬化发生率为3.6%~32.6%,并可见于婴儿期。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "pro", "entity": "肝活检" }, { "start_idx": 7, "end_idx": 17, "type": "dis", "entity": "儿童HBV相关性肝硬化" } ] }, { "text": "肝活检有弥漫性肝纤维化及结节形成。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "肝活检" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肝" }, { "start_idx": 4, "end_idx": 15, "type": "sym", "entity": "弥漫性肝纤维化及结节形成" } ] }, { "text": "B超见肝脏缩小,表面凹凸不平,肝实质回声增强,呈结节状,门静脉和脾静脉内径增宽。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "B超" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肝脏" }, { "start_idx": 3, "end_idx": 13, "type": "sym", "entity": "肝脏缩小,表面凹凸不平" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肝实质" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "肝实质回声增强,呈结节状" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "门静脉" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "脾静脉" }, { "start_idx": 28, "end_idx": 38, "type": "sym", "entity": "门静脉和脾静脉内径增宽" } ] }, { "text": "1.代偿性肝硬化指早期肝硬化。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "代偿性肝硬化" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "早期肝硬化" } ] }, { "text": "可有门静脉高压症,但无腹水、肝性脑病或上消化道出血。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "门静脉高压症" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "肝性脑病" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "上消化道" }, { "start_idx": 10, "end_idx": 24, "type": "sym", "entity": "无腹水、肝性脑病或上消化道出血" } ] }, { "text": "2.失代偿性肝硬化指中晚期肝硬化。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "失代偿性肝硬化" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "中晚期肝硬化" } ] }, { "text": "有明显肝功能异常和失代偿征象。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "肝功能异常" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "失代偿征象" } ] }, { "text": "可有腹水、肝性脑病及门脉高压症引起的侧支血管明显曲张或出血。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "腹水" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "肝性脑病" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "门脉高压症" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "侧支血管" }, { "start_idx": 18, "end_idx": 28, "type": "sym", "entity": "侧支血管明显曲张或出血" } ] }, { "text": "【肝外损害】(一)肾脏损害1.乙型肝炎相关性肾炎多表现为膜性肾小球肾炎或肾病。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肝" }, { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "肝外损害" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肾脏" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "肾脏损害" }, { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "乙型肝炎相关性肾炎" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "膜性肾小球肾炎" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "肾病" } ] }, { "text": "肾组织免疫荧光检查有乙肝抗原、IgG、补体C3沉积。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "肾组织免疫荧光检查" }, { "start_idx": 10, "end_idx": 24, "type": "sym", "entity": "乙肝抗原、IgG、补体C3沉积" } ] }, { "text": "2.肾小管酸中毒慢性乙肝患者出现厌食、呕吐、多饮多尿、生长障碍;代谢性酸中毒伴碱性尿及相关电解质改变。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "肾小管酸中毒慢性乙肝" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "厌食" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "呕吐" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "多饮多尿" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "生长障碍" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "代谢性酸中毒" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "碱性尿" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "相关电解质改变" } ] }, { "text": "(二)血液系统损害1.再生障碍性贫血各型肝炎时均可发生,治疗效果差,病死率高。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "血液系统" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "血液系统损害" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "再生障碍性贫血" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肝炎" } ] }, { "text": "2.血小板减少性紫癜对治疗反应差,常伴抗心磷脂抗体阳性。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "血小板减少性紫癜" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "抗心磷脂抗体" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "抗心磷脂抗体阳性" } ] }, { "text": "(三)血清病样表现有皮疹、关节疼痛、短暂发热等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血清" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "皮疹" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "关节" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "关节疼痛" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "短暂发热" } ] }, { "text": "(四)婴儿丘疹样肢皮炎发生于婴儿HBV感染、HBsAg为ayw亚型者。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "婴儿丘疹样肢皮炎" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "婴儿HBV感染" }, { "start_idx": 28, "end_idx": 32, "type": "mic", "entity": "ayw亚型" } ] }, { "text": "面部和四肢有非化脓性红色丘疹。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "面部" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "四肢" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "面部和四肢有非化脓性红色丘疹" } ] }, { "text": "病变皮肤的微血管壁有HBsAg、IgG、C3免疫复合物沉积。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "病变皮肤的微血管壁" }, { "start_idx": 0, "end_idx": 28, "type": "sym", "entity": "病变皮肤的微血管壁有HBsAg、IgG、C3免疫复合物沉积" } ] }, { "text": "【病原学诊断】(一)血清HBV标志物(HBVmarkers)检测(常用ELISA法)1.乙肝表面抗原(HBsAg)和表面抗体(抗HBs)HBsAg是HBV感染的标志,高滴度阳性提示有HBV复制。", "entities": [ { "start_idx": 10, "end_idx": 31, "type": "pro", "entity": "血清HBV标志物(HBVmarkers)检测" }, { "start_idx": 35, "end_idx": 40, "type": "pro", "entity": "ELISA法" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "乙肝" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "抗体" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "抗HBs" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "HBV感染" }, { "start_idx": 91, "end_idx": 93, "type": "mic", "entity": "HBV" } ] }, { "text": "抗HBs为保护性中和抗体,在乙肝恢复期或乙肝疫苗免疫后出现。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "乙肝" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "乙肝疫苗" } ] }, { "text": "两者同时阳性见于疫苗免疫后HBV变异株感染。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "变异株感染" } ] }, { "text": "2.乙肝e抗原(HBeAg)和e抗体(抗HBe)HBeAg是HBV复制的标志。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "乙肝" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "e抗体" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "抗HBe" }, { "start_idx": 30, "end_idx": 32, "type": "mic", "entity": "HBV" } ] }, { "text": "HBeAg阴转和抗HBe出现表明病毒复制停止,见于:①急性感染恢复期;②慢性感染HBV非复制期;③HBV极低复制状态或慢性期pre-core基因突变时。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "抗HBe" }, { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "病毒" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "慢性感染HBV" }, { "start_idx": 49, "end_idx": 51, "type": "mic", "entity": "HBV" } ] }, { "text": "两者不会同时阳性,同时持续阴性提示pre-core变异株感染。", "entities": [ { "start_idx": 17, "end_idx": 29, "type": "dis", "entity": "pre-core变异株感染" } ] }, { "text": "3.乙肝核心抗原(HBcAg)和核心抗体(抗HBc)HBcAg是HBV复制的直接标志。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "乙肝" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "核心抗体" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "抗HBc" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "HBV" } ] }, { "text": "抗HBcIgM亦是HBV复制标志,急性期呈高滴度阳性,慢性感染HBV复制期亦呈阳性,但滴度较低。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "抗HBcIgM" }, { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "HBV" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "高滴度阳性" }, { "start_idx": 27, "end_idx": 36, "type": "dis", "entity": "慢性感染HBV复制期" } ] }, { "text": "抗HBcIgG在HBV感染后常持续存在,高滴度阳性提示有HBV复制。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "mic", "entity": "抗HBcIgG" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "HBV感染" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "高滴度阳性" }, { "start_idx": 28, "end_idx": 30, "type": "mic", "entity": "HBV" } ] }, { "text": "(二)血清HBVDNA是HBV复制的直接标志。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "bod", "entity": "血清HBVDNA" }, { "start_idx": 12, "end_idx": 14, "type": "mic", "entity": "HBV" } ] }, { "text": "可用PCR法进行定性或定量分析。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "PCR法" } ] }, { "text": "【预防】(一)乙肝疫苗乙肝疫苗是控制HBV感染的有效手段,除新生儿普种已纳入国家计划免疫外,其他易感人群,特别是儿童有条件时均应积极接种。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "乙肝疫苗" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "乙肝疫苗" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "HBV感染" }, { "start_idx": 66, "end_idx": 67, "type": "pro", "entity": "接种" } ] }, { "text": "现采用重组基因疫苗。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "重组基因疫苗" } ] }, { "text": "注射部位以上臂三角肌内注射最佳。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "上臂三角肌内注射" } ] }, { "text": "2.加强和复种基础免疫后应强调抗HBs的检测。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "复种" }, { "start_idx": 15, "end_idx": 21, "type": "pro", "entity": "抗HBs的检测" } ] }, { "text": "产生有效抗HBs表明免疫成功,无抗HBs产生者应再次全程免疫,免疫成功后抗HBs水平下降或消失应加强免疫(单剂接种剂接种即可)。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "抗HBs" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "抗HBs" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "单剂接种" } ] }, { "text": "(二)乙肝高效免疫球蛋白(HBIG)高危新生儿(母亲HBsAg阳性,特别是伴HBeAg阳性者)生后12小时内肌内注射HBIG200U;单次急性接触HBV(如输血制品、意外污染针头刺伤等)的成人或儿童在接触后24小时内肌内注射HBIG400U,推荐使用2剂,间隔15天。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dru", "entity": "乙肝高效免疫球蛋白" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "HBIG" }, { "start_idx": 54, "end_idx": 65, "type": "pro", "entity": "肌内注射HBIG200U" }, { "start_idx": 73, "end_idx": 75, "type": "mic", "entity": "HBV" }, { "start_idx": 87, "end_idx": 88, "type": "equ", "entity": "针头" }, { "start_idx": 89, "end_idx": 90, "type": "dis", "entity": "刺伤" }, { "start_idx": 108, "end_idx": 119, "type": "pro", "entity": "肌内注射HBIG400U" } ] }, { "text": "接触HBV达7天或超过7天不应使用HBIG。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "HBV" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "HBIG" } ] }, { "text": "HBIG与乙肝疫苗联合应用可更有效地阻断母婴传播HBV,即两者同时在不同部位注射时,200U的HBIG不足以干扰乙肝疫苗的免疫反应。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "HBIG" }, { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "乙肝疫苗" }, { "start_idx": 24, "end_idx": 26, "type": "mic", "entity": "HBV" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "注射" }, { "start_idx": 47, "end_idx": 50, "type": "dru", "entity": "HBIG" }, { "start_idx": 56, "end_idx": 59, "type": "dru", "entity": "乙肝疫苗" } ] }, { "text": "【治疗】(一)一般治疗同其他肝炎,包括合理营养、适宜活动、保护肝细胞、改善肝功能、预防肝纤维化(常采用活血化瘀中药)、调整免疫和对症治疗等综合治疗措施。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肝炎" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "合理营养" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "适宜活动" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "保护肝细胞" }, { "start_idx": 35, "end_idx": 39, "type": "pro", "entity": "改善肝功能" }, { "start_idx": 41, "end_idx": 46, "type": "pro", "entity": "预防肝纤维化" }, { "start_idx": 55, "end_idx": 56, "type": "dru", "entity": "中药" } ] }, { "text": "(二)抗病毒治疗其近期目标是抑制病毒增殖,改善症状和肝脏功能,减轻肝组织病变;远期目标是清除病毒,防止肝硬化和肝细胞癌的发生,提高生存率和改善生存质量。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "病毒" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肝脏" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "肝组织" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "肝组织病变" }, { "start_idx": 46, "end_idx": 47, "type": "mic", "entity": "病毒" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "肝硬化" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "肝细胞癌" } ] }, { "text": "目前在世界范围批准治疗慢性乙肝的药物只有干扰素和拉米夫定两种。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "慢性乙肝" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "干扰素" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "拉米夫定" } ] }, { "text": "一般认为,对于无肝损害或轻微肝病的HBV感染者宜医学观察,不推荐治疗。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肝" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "轻微肝病" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "HBV感染" } ] }, { "text": "可选用的治疗方法如下:1.干扰素α(IFN-α)血清HBVDNA>106copies/ml伴ALT异常的慢性患者适合IFN-α治疗。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "干扰素α" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "IFN-α" }, { "start_idx": 24, "end_idx": 31, "type": "ite", "entity": "血清HBVDNA" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "ALT异常" }, { "start_idx": 64, "end_idx": 70, "type": "pro", "entity": "IFN-α治疗" } ] }, { "text": "失代偿性肝硬化、患自身免疫性疾病或有重要脏器疾病者不宜使用。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "失代偿性肝硬化" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "重要脏器疾病" } ] }, { "text": "儿童剂量尚未统一,推荐剂量为3MU/m2(成人推荐每次5MU),皮下或肌内注射,每周3次,疗程≥6个月。", "entities": [ { "start_idx": 38, "end_idx": 44, "type": "pro", "entity": "皮下或肌内注射" } ] }, { "text": "治疗初期常见发热等感冒样综合征,在晚间或睡眠前用药可减轻不适反应。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "发热" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "感冒" } ] }, { "text": "粒细胞和血小板减少是常见的不良反应,前者经加服复方阿胶浆可获改善,当WBC计数<3.0×109/L或粒细胞计数<1.5×109/L或血小板计数<40×109/L时应停药,一般可自行恢复,恢复后可重新治疗。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "粒细胞" }, { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "血小板" }, { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "粒细胞和血小板减少" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "复方阿胶浆" }, { "start_idx": 34, "end_idx": 38, "type": "ite", "entity": "WBC计数" }, { "start_idx": 56, "end_idx": 60, "type": "ite", "entity": "粒细胞计数" }, { "start_idx": 78, "end_idx": 82, "type": "ite", "entity": "血小板计数" } ] }, { "text": "2.拉米呋定(lamivudine,LAM)为核苷类似物,适应证同IFN-α。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "拉米呋定" }, { "start_idx": 7, "end_idx": 16, "type": "dru", "entity": "lamivudine" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "LAM" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "IFN-α" } ] }, { "text": "用药期间应监测肝功能和血常规,若服药6个月以上病情复发,应考虑发生HBV变异而停药。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "监测肝功能" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "血常规" }, { "start_idx": 33, "end_idx": 35, "type": "mic", "entity": "HBV" } ] }, { "text": "一般停药3~6个月后,因LAM作用消除而病情复发,将LAM与IFN-α联合应用,可更早获得疗效。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "LAM" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "LAM" } ] }, { "text": "3.其他药物①阿地福韦(adefovir,ADV)用于治疗LAM耐药的HBV变异株感染。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "阿地福韦" }, { "start_idx": 12, "end_idx": 19, "type": "dru", "entity": "adefovir" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "ADV" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "LAM" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "HBV变异株感染" } ] }, { "text": "②胸腺素α1(Tα1)通过诱导和促进细胞免疫而清除病毒,其不良反应极小,12~16岁儿童1.6mg皮下注射,每周2次,共6个月。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dru", "entity": "胸腺素α1" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "细胞" }, { "start_idx": 37, "end_idx": 38, "type": "mic", "entity": "病毒" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "皮下注射" } ] }, { "text": "患者能很好耐受,适于对IFN和LAM不能耐受者和重型肝炎,可用于联合治疗。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "IFN" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "LAM" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "重型肝炎" } ] }, { "text": "4.肝移植国外对慢性失代偿性乙肝患者采用肝移植和LAM联合治疗(移植后持续服用LAM),5年存活率可达95%以上。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肝移植" }, { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "慢性失代偿性乙肝" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "肝移植" }, { "start_idx": 24, "end_idx": 30, "type": "pro", "entity": "LAM联合治疗" } ] }, { "text": "六、幼年性纤维瘤病幼年性纤维瘤病又称硬纤维瘤,是一种局部侵袭性纤维瘤,1838年Muller初次报道。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "幼年性纤维瘤病" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "幼年性纤维瘤病" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "硬纤维瘤" }, { "start_idx": 26, "end_idx": 33, "type": "dis", "entity": "局部侵袭性纤维瘤" } ] }, { "text": "有人认为本病是先天性肿瘤,也有人认为与雌性激素有关,还有人认为与遗传缺陷有关,总之,发病机制尚不完全清楚。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "雌性激素" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "遗传缺陷" } ] }, { "text": "镜下肿瘤为梭形的成熟成纤维细胞组成,被或多或少的胶原梭包围,可见成纤维细胞的核仁,但无明显的有丝分裂。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "equ", "entity": "镜" }, { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "纤维细胞" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "胶原梭" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "成纤维细胞" } ] }, { "text": "不同部位的肿瘤表现不同。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" } ] }, { "text": "肿瘤表面皮肤正常,不与肿瘤黏附,可活动。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "皮肤" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "肿瘤" } ] }, { "text": "多在腹直肌前鞘的后面,与后鞘融合,且与腹横肌紧密附着。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "腹直肌前鞘" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "后鞘" } ] }, { "text": "肿瘤质地非常坚硬,无痛,边界不清楚,无全身症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" } ] }, { "text": "手术切除是主要的治疗方法。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "手术切除" } ] }, { "text": "切除要彻底,一般要切除包括肿瘤边缘1~2cm正常组织,因为如有残留必定复发。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "肿瘤" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "组织" } ] }, { "text": "本瘤的复发率为50%~70%,应该争取再次手术切除,而且对复发病例,应加以放疗,对所有病例应长期随访。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "手术切除" } ] }, { "text": "四、腹膜透析的并发症(一)与技术有关的并发症主要为透析管道故障,多发生于插管后40天以内,40天后的并发症多为腹膜炎引起。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "透析管道故障" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "1.透析液外漏透析液沿管道自皮肤出口或皮肤切口漏出。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "透析液" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "透析液" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "皮肤" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "皮肤" } ] }, { "text": "多因腹膜切口过大以及缝合不严所致,腹壁松弛、应用糖皮质激素药物期间易于发生。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "腹膜" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "腹壁" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "糖皮质激素" } ] }, { "text": "插管术中严密缝合腹膜,术后5~14天才开始透析可以避免。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "插管术" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "腹膜" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "透析" } ] }, { "text": "一旦漏液发生后应排空透析液,停止透析1~2天,防止延迟愈合的因素如腹肌过度活动,一般休息数日后漏液自行停止。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "透析液" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "透析" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "腹肌" } ] }, { "text": "2.透析液引流不畅原因有:透析管端移位、漂浮,表现为单向阻滞;腹膜炎后形成纤维蛋白块,堵塞管道,透析液出入不畅,以排出不畅为主;腹膜粘连透析管周围形成包裹,表现为输入透析液不久患者即感腹痛,X线造影可确诊;腹腔内进入空气、肠胀气以及便秘等亦可导致透析液引流不畅。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "透析液" }, { "start_idx": 13, "end_idx": 15, "type": "equ", "entity": "透析管" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "腹膜炎" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "纤维蛋白块" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "透析液" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "腹膜" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "腹膜粘连" }, { "start_idx": 68, "end_idx": 70, "type": "equ", "entity": "透析管" }, { "start_idx": 83, "end_idx": 85, "type": "dru", "entity": "透析液" }, { "start_idx": 92, "end_idx": 93, "type": "sym", "entity": "腹痛" }, { "start_idx": 103, "end_idx": 104, "type": "bod", "entity": "腹腔" }, { "start_idx": 111, "end_idx": 113, "type": "sym", "entity": "肠胀气" }, { "start_idx": 116, "end_idx": 117, "type": "sym", "entity": "便秘" }, { "start_idx": 123, "end_idx": 125, "type": "dru", "entity": "透析液" } ] }, { "text": "应根据临床表现,结合X线检查,明确导致引流不畅的原因,分别处理。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "X线检查" } ] }, { "text": "遇到凝块堵塞,可用加有肝素以及尿激酶的生理盐水冲洗,应用尼龙丝线或细探针疏导。", "entities": [ { "start_idx": 10, "end_idx": 22, "type": "dru", "entity": "有肝素以及尿激酶的生理盐水" }, { "start_idx": 28, "end_idx": 31, "type": "equ", "entity": "尼龙丝线" }, { "start_idx": 33, "end_idx": 35, "type": "equ", "entity": "细探针" } ] }, { "text": "此外,腹部按摩以及灌肠可收效。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "腹部" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "灌肠" } ] }, { "text": "若上述措施无效,则需要更换透析管。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "equ", "entity": "透析管" } ] }, { "text": "(二)透析引起的继发性并发症1.腹膜炎儿童腹膜透析最常见的并发症是腹膜炎,其发生率为每13.4病人月发作一次,是导致腹透失败的常见原因。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "透析" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "腹膜炎" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "腹膜炎" }, { "start_idx": 58, "end_idx": 59, "type": "pro", "entity": "腹透" } ] }, { "text": "但最近由于先进的连接系统和自动腹膜透析机的使用,使儿童腹膜炎的发生大为减少,报道已减至30~50个病人月发生一次。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "equ", "entity": "自动腹膜透析机" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "儿童腹膜炎最常见的细菌为革兰阳性球菌,达60%~70%,如金黄色葡萄球菌,其次是表皮葡萄球菌。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "儿童腹膜炎" }, { "start_idx": 12, "end_idx": 17, "type": "mic", "entity": "革兰阳性球菌" }, { "start_idx": 29, "end_idx": 35, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 40, "end_idx": 45, "type": "mic", "entity": "表皮葡萄球菌" } ] }, { "text": "革兰阴性杆菌约占20%~30%,真菌感染约占3%~5%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "真菌" } ] }, { "text": "当腹透液白细胞总数>100/ml,多形核大于50%,要考虑腹膜炎的诊断。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "腹透液" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "体温升高常为最早表现,随之出现腹透液混浊、腹痛,同时伴有消化道症状如恶心、呕吐。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "腹透液" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "腹痛" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "恶心" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "呕吐" } ] }, { "text": "腹透液革兰染色和细菌培养可协助诊断并提供抗生素治疗选择依据。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "腹透液革兰染色" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "细菌培养" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "抗生素" } ] }, { "text": "由于患儿的腹膜表面积大,通透性高,腹腔内给抗生素能快速经腹膜吸收,局部药物浓度高。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "腹膜" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "腹腔" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "抗生素" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "腹膜" } ] }, { "text": "但<2岁婴儿,是需作血培养除外有无败血症并及时给予全身抗生素治疗,抗生素治疗时间一般为培养阴性后再持续7天,总疗程约14~21天。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "血培养" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "败血症" }, { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "全身抗生素治疗" }, { "start_idx": 33, "end_idx": 37, "type": "pro", "entity": "抗生素治疗" } ] }, { "text": "真菌性腹膜炎的抗真菌治疗效果较差,若应用两性霉素B或5-氟胞嘧啶等药物治疗10天后无效,可考虑拔除腹透管,拔管后继续抗真菌治疗10天。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "真菌性腹膜炎" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "抗真菌治疗" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 26, "end_idx": 31, "type": "dru", "entity": "5-氟胞嘧啶" }, { "start_idx": 49, "end_idx": 51, "type": "equ", "entity": "腹透管" }, { "start_idx": 58, "end_idx": 62, "type": "pro", "entity": "抗真菌治疗" } ] }, { "text": "2.腹透管外口及腹透管皮下隧道感染为儿童腹透常见并发症之一,腹透管外口感染表现为外口充血、皮肤炎症以及有脓性分泌物。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "腹透管" }, { "start_idx": 8, "end_idx": 10, "type": "equ", "entity": "腹透管" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "腹透" }, { "start_idx": 30, "end_idx": 32, "type": "equ", "entity": "腹透管" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "外口充血" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "皮肤炎症" }, { "start_idx": 51, "end_idx": 56, "type": "sym", "entity": "有脓性分泌物" } ] }, { "text": "分泌物培养阴性不能否定感染,培养阳性而无炎症时则可以否定感染的存在。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "分泌物培养" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "感染" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "炎症" } ] }, { "text": "感染可以单独存在,但常常伴有皮下隧道感染。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "感染" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "皮下隧道感染" } ] }, { "text": "皮下隧道感染表现为炎症累及涤纶套,皮肤充血,隧道沿途触痛、周围有蜂窝织炎。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "皮下隧道感染" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "炎症累及涤纶套" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "皮肤充血" } ] }, { "text": "当有以下指征时,要考虑拔除PD管:真菌性腹膜炎;伴PD管皮肤出口或皮下隧道感染,治疗无效;同一病菌引起反复发作的腹膜炎。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "equ", "entity": "PD管" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "真菌性腹膜炎" }, { "start_idx": 24, "end_idx": 38, "type": "dis", "entity": "伴PD管皮肤出口或皮下隧道感染" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "拔除PD管后若有必要,可在1~3周后重新置管。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "PD管" } ] }, { "text": "3.非感染性并发症随着PD感染性并发症的逐渐减少,非感染性并发症日益受到重视。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "非感染性并发症" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "PD感染性并发症" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "非感染性并发症" } ] }, { "text": "其发生主要与腹内压升高、长期透析对腹膜的非生理性影响和对全身代谢的影响有关:①蛋白质和氨基酸、维生素的丢失。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "ite", "entity": "腹内压" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "透析" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "腹膜" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "蛋白质" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "氨基酸" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "维生素" } ] }, { "text": "PD中难免要丢失体内的营养物质,蛋白质丢失的多少与血清蛋白质浓度、体表面积以及腹膜的通透性有关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "PD" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "蛋白质" }, { "start_idx": 25, "end_idx": 31, "type": "ite", "entity": "血清蛋白质浓度" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "体表" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "腹膜" } ] }, { "text": "在CAPD开始2周内,每日经透析液丢失蛋白质15~20g,以后丢失量减少,平均每日丢失5~11g,其中以白蛋白为主。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "CAPD" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "透析液" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "蛋白质" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "白蛋白" } ] }, { "text": "IPD每日丢失10~44g蛋白质。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "IPD" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "蛋白质" } ] }, { "text": "发生腹膜炎时蛋白质丢失量可增加1~30倍;增加透析次数、提高透析液的渗透压及发热时蛋白质丢失量也增多:氨基酸丢失量每日约2g,丢失种类与血中氨基酸成分相似,80%为非必需氨基酸。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "腹膜炎" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "蛋白质" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "透析" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "透析液" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "发热" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "蛋白质" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "氨基酸" }, { "start_idx": 68, "end_idx": 68, "type": "bod", "entity": "血" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "氨基酸" }, { "start_idx": 82, "end_idx": 87, "type": "bod", "entity": "非必需氨基酸" } ] }, { "text": "每日随透析液丢失大量维生素,主要是水溶性维生素,若不注意补充,在透析2~3个月后可以出现维生素缺乏的表现。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "透析液" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "维生素" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "水溶性维生素" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "维生素" } ] }, { "text": "有感染时可能需要另外补充蛋白质,如血浆和白蛋白。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "感染" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "蛋白质" }, { "start_idx": 17, "end_idx": 18, "type": "dru", "entity": "血浆" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "白蛋白" } ] }, { "text": "在透析液中加入氨基酸可以补充氨基酸的丢失。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "透析液" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "氨基酸" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "氨基酸" } ] }, { "text": "②高血糖、高血脂与肥胖。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "sym", "entity": "高血糖、高血脂与肥胖" } ] }, { "text": "PD中经腹膜吸收的葡萄糖每日约120~200g。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "PD" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "腹膜" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "葡萄糖" } ] }, { "text": "透析液渗透压越高,吸收的葡萄糖量越多。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "透析液" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "葡萄糖" } ] }, { "text": "若长期用较高浓度的葡萄糖液,可能会发生高血糖,甚至高渗性昏迷以及糖尿病。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "葡萄糖液" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "高血糖" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "高渗性昏迷" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "糖尿病" } ] }, { "text": "部分患者体重逐渐增加,血清甘油三酯以及胆固醇升高。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "血清甘油三酯" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "胆固醇" } ] }, { "text": "③水、电解质及酸碱失衡。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "sym", "entity": "水、电解质及酸碱失衡" } ] }, { "text": "PD患者很容易发生水代谢失衡。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "PD" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "水代谢失衡" } ] }, { "text": "若患者每日体重增加0.5kg,明显水肿,甚至出现肺水肿及脑水肿,提示体内水分过多,宜提高透析液渗透压,加强超滤;若患者显著口渴,短期内体重下降,乏力,发生体位性低血压,提示体内缺水,此时宜减少透析液中的葡萄糖用量,应用生理盐水或血浆扩容。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "水肿" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "肺水肿" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "脑水肿" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "水分" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "透析液" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "口渴" }, { "start_idx": 64, "end_idx": 70, "type": "sym", "entity": "短期内体重下降" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "乏力" }, { "start_idx": 75, "end_idx": 82, "type": "sym", "entity": "发生体位性低血压" }, { "start_idx": 89, "end_idx": 89, "type": "bod", "entity": "水" }, { "start_idx": 96, "end_idx": 98, "type": "dru", "entity": "透析液" }, { "start_idx": 101, "end_idx": 103, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 109, "end_idx": 112, "type": "dru", "entity": "生理盐水" }, { "start_idx": 114, "end_idx": 115, "type": "dru", "entity": "血浆" } ] }, { "text": "血中钠、钾、镁和碳酸氢根浓度过高或过低时,应调整透析液中的相应离子浓度来纠正。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "血" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "钠" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "钾" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "镁" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "碳酸氢根" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "透析液" } ] }, { "text": "④呼吸系统并发症。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "呼吸系统" } ] }, { "text": "由于腹腔内存有大量透析液,横膈抬高,肺底萎陷,分泌物易积蓄,患者可能发生呼吸系统并发症,包括支气管炎、肺炎、肺不张以及胸腔积液。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "腹腔" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "透析液" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "横膈抬高" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "肺底萎陷" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "分泌物" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "支气管炎" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "肺炎" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "肺不张" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "胸腔积液" } ] }, { "text": "⑤疝气:包括腹壁切口疝、脐疝、腹股沟疝和膈疝等,因腹内压增高所致。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "疝气" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "腹壁切口疝" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "脐疝" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "腹股沟疝" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "膈疝" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "腹内压" } ] }, { "text": "腹肌软弱者较易发生,常需手术修补。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "腹肌" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "手术" } ] }, { "text": "⑥其他可有腹痛", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "腹痛" } ] }, { "text": "第十章心律失常第一节概况儿童心律失常可以是一过性的或永久性的;可以是先天性的(心脏结构可正常或异常)或后天获得性的(风湿热,心肌炎);可以由毒素(白喉毒素)、可卡因、茶碱或一些抗心律失常药物引起;也可以是先天性心脏病外科手术后的后遗症。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "心律失常" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "心律失常" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "心脏" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "风湿热" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "心肌炎" }, { "start_idx": 70, "end_idx": 71, "type": "mic", "entity": "毒素" }, { "start_idx": 73, "end_idx": 76, "type": "mic", "entity": "白喉毒素" }, { "start_idx": 79, "end_idx": 81, "type": "dru", "entity": "可卡因" }, { "start_idx": 83, "end_idx": 84, "type": "dru", "entity": "茶碱" }, { "start_idx": 89, "end_idx": 92, "type": "dis", "entity": "心律失常" }, { "start_idx": 102, "end_idx": 111, "type": "pro", "entity": "先天性心脏病外科手术" } ] }, { "text": "心律失常的主要危险是严重心动过速或心动过缓,导致心输出量下降,或发展成更严重的心律失常,如心室颤动。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心律失常" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "心动过速" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "心动过缓" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "心输出量下降" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "心律失常" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "心室颤动" } ] }, { "text": "晕厥本身在某些情况下也很危险,如发生在游泳或驾车时。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "晕厥" } ] }, { "text": "当一个患有心律失常的患儿就诊时,首先需确定这种心律失常是否有可能发展成威胁生命的心动过速或心动过缓。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "心律失常" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "心律失常" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "心动过速" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "心动过缓" } ] }, { "text": "一些心律异常(如单纯性房性期前收缩和室性期前收缩)在无心脏疾患的儿童常见,在绝大多数情况下并不给这些患儿带来生命的危险。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "心律异常" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "室性期前收缩" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "心脏" } ] }, { "text": "成人抗心律失常药物的种类不断增加,但许多药物在儿科的应用并未广泛研究。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "心律失常" } ] }, { "text": "儿童抗心律失常药物的给予次数、顺应性、副作用及不同的反应性仍是有待解决的问题。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "心律失常" } ] }, { "text": "当患儿患快速性心律失常且耐药时,可予经导管射频消融或外科治疗。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "快速性心律失常" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "经导管射频消融" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "外科治疗" } ] }, { "text": "现今的起搏器体积很小,已可用于早产儿。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "起搏器" } ] }, { "text": "在高危恶性室性心律失常患儿,可安装自动植入式电复律除颤器(AICDs)。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "dis", "entity": "高危恶性室性心律失常" }, { "start_idx": 15, "end_idx": 27, "type": "pro", "entity": "安装自动植入式电复律除颤器" }, { "start_idx": 29, "end_idx": 33, "type": "equ", "entity": "AICDs" } ] }, { "text": "表9-9为儿科常用抗心律失常药物的剂量及注意事项,表9-9为美国心脏病学会/美国心脏协会(ACC/AHA)1998年更新的小儿及青少年永久心脏起搏的特殊指征,可供参考。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dep", "entity": "儿科" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "心脏病" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "心脏" }, { "start_idx": 69, "end_idx": 72, "type": "ite", "entity": "心脏起搏" } ] }, { "text": "表9-13儿科常用抗心律失常药物的剂量及注意事项续表注:CNS—中枢神经系统,bid—一天二次,tid—一天三次,PO—口服,IV—静脉应用。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dep", "entity": "儿科" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "心律失常" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "静脉" } ] }, { "text": "表9-14小儿与青少年永久心脏起搏的指征注:表中第一类为无争议的起搏指征,第二类为有争议的起搏指征,其中二a类为倾向于植入起搏器,二b类为倾向于不植入起搏器,第三类为无需起搏治疗的心律失常。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "ite", "entity": "心脏起搏" }, { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "起搏" }, { "start_idx": 45, "end_idx": 46, "type": "ite", "entity": "起搏" }, { "start_idx": 61, "end_idx": 63, "type": "equ", "entity": "起搏器" }, { "start_idx": 75, "end_idx": 77, "type": "equ", "entity": "起搏器" }, { "start_idx": 90, "end_idx": 93, "type": "dis", "entity": "心律失常" } ] }, { "text": "小儿病例不包含A级部分原因是儿科患儿数较少。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dep", "entity": "儿科" } ] }, { "text": "四、低度恶性胶质瘤低度恶性胶质瘤(glioma)指生长缓慢,非侵袭性,组织学表现相对良性的肿瘤,相当于Kernohan分类法中的Ⅰ、Ⅱ级肿瘤。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "低度恶性胶质瘤" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "低度恶性胶质瘤" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "glioma" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "肿瘤" } ] }, { "text": "包括星形细胞瘤、少突胶质细胞瘤、以上两种瘤细胞的混合型肿瘤及神经元和胶质细胞的混合型肿瘤。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "星形细胞瘤" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "少突胶质细胞瘤" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "瘤细胞" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "混合型肿瘤" }, { "start_idx": 30, "end_idx": 43, "type": "dis", "entity": "神经元和胶质细胞的混合型肿瘤" } ] }, { "text": "儿童幕上肿瘤35%为低度胶质瘤,1/3~1/2的肿瘤位于大脑半球,其余位于间脑中央深部(丘脑、下丘脑、三脑室)及基底节。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "儿童幕上肿瘤" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "低度胶质瘤" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "肿瘤" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "脑半球" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "间脑中央" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "丘脑" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "下丘脑" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "三脑室" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "基底节" } ] }, { "text": "低度胶质瘤在儿童幕下肿瘤中占10%~20%,主要为纤维型星形细胞瘤,平均发病年龄为6.5~9岁。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "低度胶质瘤" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "儿童幕下肿瘤" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "纤维型星形细胞瘤" } ] }, { "text": "临床表现常见症状有颅内高压(约75%)、癫痫不同部位的神经损害症状如偏瘫、失语、神经内分泌异常、视神经萎缩症状等检查CT及MRI可显示肿瘤部位、大小、血运是否丰富等。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "有颅内高压" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "癫痫" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "癫痫" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "神经损害" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "偏瘫" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "失语" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "神经内分泌异常" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "视神经萎缩" }, { "start_idx": 22, "end_idx": 55, "type": "sym", "entity": "不同部位的神经损害症状如偏瘫、失语、神经内分泌异常、视神经萎缩症状等" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "检查CT" }, { "start_idx": 61, "end_idx": 63, "type": "pro", "entity": "MRI" }, { "start_idx": 67, "end_idx": 68, "type": "dis", "entity": "肿瘤" } ] }, { "text": "手术是治疗的主要手段,要求在安全的前提下尽量完整切除肿瘤。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "肿瘤" } ] }, { "text": "统计肿瘤全切除病例,有报道7年存活率达80%,而次全切除的病例,7年存活率仅达15%~50%。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" } ] }, { "text": "放疗适用于进展性或复发性肿瘤,且仅需做局部放疗即可;全切除肿瘤不需放疗;不全切除肿瘤是否需要放疗尚有争议,多数学者认为应有再次手术或先化疗。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "放疗" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肿瘤" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "局部放疗" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "肿瘤" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "放疗" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "肿瘤" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "放疗" }, { "start_idx": 63, "end_idx": 64, "type": "pro", "entity": "手术" }, { "start_idx": 67, "end_idx": 68, "type": "pro", "entity": "化疗" } ] }, { "text": "报道105例儿童纤维细胞型星形细胞瘤,20年生存率达79%。", "entities": [ { "start_idx": 6, "end_idx": 17, "type": "dis", "entity": "儿童纤维细胞型星形细胞瘤" } ] }, { "text": "位于脑干、间脑及脊髓肿瘤的预后相对较差。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "脑干、间脑及脊髓肿瘤" } ] }, { "text": "二、腹膜透析置管术及相关并发症(一)腹膜透析管的选择质量好的腹透管是成功进行腹膜透析的基础。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "腹膜透析置管术" }, { "start_idx": 18, "end_idx": 22, "type": "equ", "entity": "腹膜透析管" }, { "start_idx": 30, "end_idx": 32, "type": "equ", "entity": "腹透管" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "腹膜透析" } ] }, { "text": "儿童和婴幼儿腹透管应随其年龄、身高和体重而选择,通常比成人腹透管更短,更小,还有新生儿专用管。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "equ", "entity": "腹透管" }, { "start_idx": 29, "end_idx": 31, "type": "equ", "entity": "腹透管" } ] }, { "text": "插入腹腔内透析管长度约相当于患儿脐至耻骨联合的距离,6岁以上、体重>30kg的儿童,可以应用成人型透析管。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "腹腔" }, { "start_idx": 5, "end_idx": 7, "type": "equ", "entity": "透析管" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "脐" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "耻骨" }, { "start_idx": 46, "end_idx": 51, "type": "equ", "entity": "成人型透析管" } ] }, { "text": "目前全世界范围内使用最广的仍是Tenckhoff透析管。", "entities": [ { "start_idx": 15, "end_idx": 26, "type": "equ", "entity": "Tenckhoff透析管" } ] }, { "text": "早期使用的Tenckhoff透析管均为直管,但考虑到减少注入腹透液疼痛及腹透液流出梗阻等问题,目前多倾向于使用弯曲Tenckhoff透析管。", "entities": [ { "start_idx": 5, "end_idx": 16, "type": "equ", "entity": "Tenckhoff透析管" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "腹透液" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "疼痛" }, { "start_idx": 36, "end_idx": 42, "type": "sym", "entity": "腹透液流出梗阻" }, { "start_idx": 55, "end_idx": 68, "type": "equ", "entity": "弯曲Tenckhoff透析管" } ] }, { "text": "据北美儿童肾移植合作研究组的观察结果显示415根直管有49根发生原发性流出梗阻(12%),而741根Tenckhoff弯管只有45根发生原发性流出梗阻(6%)。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "肾移植" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "原发性流出梗阻" }, { "start_idx": 50, "end_idx": 60, "type": "equ", "entity": "Tenckhoff弯管" }, { "start_idx": 68, "end_idx": 74, "type": "sym", "entity": "原发性流出梗阻" } ] }, { "text": "Tenckhoff导管有单涤纶套和双涤纶套,临床观察已证实双涤纶套透析管在减少腹膜炎的发生率及延长植管后第一次发作腹膜炎的时间方面的确优于单涤纶套导管。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "equ", "entity": "Tenckhoff导管" }, { "start_idx": 12, "end_idx": 15, "type": "equ", "entity": "单涤纶套" }, { "start_idx": 17, "end_idx": 20, "type": "equ", "entity": "双涤纶套" }, { "start_idx": 29, "end_idx": 35, "type": "equ", "entity": "双涤纶套透析管" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "腹膜炎" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "植管" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "腹膜炎" }, { "start_idx": 69, "end_idx": 74, "type": "equ", "entity": "单涤纶套导管" } ] }, { "text": "(二)透析管置入术因穿刺植入法的并发症较多,小儿腹膜透析管的安置多主张采用手术植入法,特别在新生儿期。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "透析管置入术" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "穿刺植入法" }, { "start_idx": 24, "end_idx": 28, "type": "equ", "entity": "腹膜透析管" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "手术植入法" } ] }, { "text": "手术植入法可准确地将导管放入小儿骨盆内,由于儿童难以配合,手术常于全麻下进行。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "手术植入法" }, { "start_idx": 10, "end_idx": 11, "type": "equ", "entity": "导管" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "骨盆" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "手术" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "全麻" } ] }, { "text": "切口常选择在脐与耻骨联合的连线上1/3处,可行正中或正中旁经腹直肌切口,也可选择麦氏切口,以经腹直肌旁正中切口最为常用。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "脐" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "耻骨" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "腹直肌" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "麦氏切口" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "腹直肌" } ] }, { "text": "Rinadi报道意大利20个透析中心的347次患儿腹膜透析管置管手术,136次采用正中切口,占39.2%,211次采用正中旁经腹直肌切口,占60.8%。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "透析" }, { "start_idx": 25, "end_idx": 33, "type": "pro", "entity": "腹膜透析管置管手术" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "腹直肌" } ] }, { "text": "按常规消毒、铺巾,切开皮肤,分离腹壁各层组织,暴露腹膜作0.5~0.8cm切口,将腹透管在导丝引导下紧贴腹膜后壁送入膀胱直肠窝,婴幼儿盆腔较浅,插入腹腔内透析管长度约相当于脐至耻骨联合的距离。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "消毒" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "皮肤" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "腹壁" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "组织" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "腹膜" }, { "start_idx": 41, "end_idx": 43, "type": "equ", "entity": "腹透管" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "腹膜" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "膀胱直肠窝" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "盆腔" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "腹腔" }, { "start_idx": 77, "end_idx": 79, "type": "equ", "entity": "透析管" }, { "start_idx": 86, "end_idx": 86, "type": "bod", "entity": "脐" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "耻骨" } ] }, { "text": "一般在切口缝合前,可灌注透析液500~1000ml,观察液体出入是否通畅,以示透析管腹腔段位置良好。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "透析液" }, { "start_idx": 39, "end_idx": 41, "type": "equ", "entity": "透析管" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "腹腔" } ] }, { "text": "在腹膜切口作荷包缝合,将腹透管周围的腹膜收紧,牢牢固定于透析管的深部涤纶套上,在观察局部无渗漏出后逐层关闭腹腔。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "腹膜" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "荷包缝合" }, { "start_idx": 12, "end_idx": 14, "type": "equ", "entity": "腹透管" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "腹膜" }, { "start_idx": 28, "end_idx": 30, "type": "equ", "entity": "透析管" }, { "start_idx": 34, "end_idx": 36, "type": "equ", "entity": "涤纶套" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "腹腔" } ] }, { "text": "浅部涤纶套在皮下隧道内离出口处至少2cm,防止因牵拉或水肿解除后皮下涤纶套脱出导致皮下隧道感染。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "equ", "entity": "浅部涤纶套" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "皮" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "水肿" }, { "start_idx": 32, "end_idx": 36, "type": "equ", "entity": "皮下涤纶套" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "皮下隧道感染" } ] }, { "text": "临床观察结果显示流出口方向向上的病人更易患腹膜炎和流出口感染。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "腹膜炎" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "流出口感染" } ] }, { "text": "Sieniawskaet尝试在婴幼儿使用鹅颈导管并使导管流出口定位在胸前,据观察可降低婴幼儿导管相关感染的发生率。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "equ", "entity": "鹅颈导管" }, { "start_idx": 46, "end_idx": 47, "type": "equ", "entity": "导管" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "感染" } ] }, { "text": "在手术中应注意:①因儿童腹膜薄、脆、嫩,为降低腹膜透析液外漏应特别注意采用腹膜荷包缝合使深部涤纶套固定腹膜中,但切勿过分牵拉腹膜造成腹膜撕裂;②儿童大网膜相对较长,常常造成大网膜包裹腹透导管堵塞使管路扭曲异位,为避免其发生,有人主张在术中对大网膜较长者行部分切除。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "腹膜" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "腹膜透析液" }, { "start_idx": 37, "end_idx": 42, "type": "pro", "entity": "腹膜荷包缝合" }, { "start_idx": 44, "end_idx": 48, "type": "equ", "entity": "深部涤纶套" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "腹膜" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "腹膜" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "腹膜" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "大网膜" }, { "start_idx": 91, "end_idx": 94, "type": "equ", "entity": "腹透导管" }, { "start_idx": 120, "end_idx": 122, "type": "bod", "entity": "大网膜" } ] }, { "text": "在慢性肾衰竭时,腹透管置入术后不主张立即进行透析,最好在术后2~4周开始。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "慢性肾衰竭" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "腹透管置入" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "透析" } ] }, { "text": "在此期间可用500~1000ml透析液冲洗腹腔后用肝素封闭透析管。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "pro", "entity": "透析液冲洗腹腔" }, { "start_idx": 29, "end_idx": 31, "type": "equ", "entity": "透析管" } ] }, { "text": "如病情需紧急透析,患儿应取作仰卧或半卧位,每次交换量15ml/kg为宜,这样可防止腹内压增加,有利于伤口组织愈合,避免发生透析液渗漏。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "透析" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "腹内压" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "伤口组织" }, { "start_idx": 61, "end_idx": 63, "type": "dru", "entity": "透析液" } ] }, { "text": "二、腺病毒肺炎腺病毒肺炎(adenoviralpneumonia)以腺病毒3型和7型为主。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "腺病毒肺炎" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "腺病毒肺炎" }, { "start_idx": 13, "end_idx": 31, "type": "dis", "entity": "adenoviralpneumonia" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "腺病毒3型" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "7型" } ] }, { "text": "起病大多急骤,先有上呼吸道感染症状,随后出现持续高热,咳嗽出现早,呈单声咳、频咳或阵咳,继而出现呼吸困难。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "上呼吸道感染" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "持续高热" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "咳嗽出现早" }, { "start_idx": 34, "end_idx": 36, "type": "sym", "entity": "单声咳" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "频咳" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "阵咳" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "肺部体征出现迟,多在高热3~4天后出现湿啰音。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "湿啰音" } ] }, { "text": "早期可出现中毒症状和多系统受累表现,如肝、脾肿大、嗜睡或烦躁不安,甚至中毒性脑病。", "entities": [ { "start_idx": 19, "end_idx": 19, "type": "sym", "entity": "肝" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "脾肿大" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "嗜睡" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "中毒性脑病" } ] }, { "text": "外周血白细胞计数大多轻度减少。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "外周血白细胞计数" } ] }, { "text": "X线改变以肺实变阴影及病灶融合为特点,其范围不受肺叶的限制。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "X线" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "肺" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肺叶" } ] }, { "text": "约1/6的病例可有胸膜炎。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "胸膜炎" } ] }, { "text": "根据上述临床表现,结合X线特点,诊断不难。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "X线" } ] }, { "text": "根据血清学和病毒学检查结果可确诊。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "血清学" }, { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "病毒学检查" } ] }, { "text": "十、恶性神经鞘瘤恶性神经鞘瘤(malignantschwannoma)又称神经纤维肉瘤,占全部小儿软组织肉瘤的3%。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "恶性神经鞘瘤" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "恶性神经鞘瘤" }, { "start_idx": 15, "end_idx": 33, "type": "dis", "entity": "malignantschwannoma" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "神经纤维肉瘤" }, { "start_idx": 47, "end_idx": 53, "type": "dis", "entity": "小儿软组织肉瘤" } ] }, { "text": "起源于周围神经或从神经纤维瘤病转化而来。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "神经" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "神经纤维瘤病" } ] }, { "text": "典型病变是在一较大的神经行径中,有一棱形肿块,脊神经的远、近端均匀变粗,表明肿瘤向神经外膜扩展。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "神经行径" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肿块" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "脊神经" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "肿瘤" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "神经外膜" } ] }, { "text": "肿瘤一般大于5cm,表面呈多肉状,可有出血或坏死区。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "表面呈多肉状" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "有出血或坏死区" } ] }, { "text": "光镜下与纤维肉瘤相似,但本瘤的细胞有不规则的轮廓,细胞核呈波形、弯形或逗号形。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "光镜" }, { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "纤维肉瘤" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "瘤的细胞" }, { "start_idx": 12, "end_idx": 23, "type": "sym", "entity": "本瘤的细胞有不规则的轮廓" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "细胞核" }, { "start_idx": 25, "end_idx": 37, "type": "sym", "entity": "细胞核呈波形、弯形或逗号形" } ] }, { "text": "临床表现:30%~43%的儿童恶性神经鞘瘤来自神经纤维瘤病,而成人50%来自主要神经干。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "童恶性神经鞘瘤" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "神经纤维瘤" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "神经干" } ] }, { "text": "对肿块突然增大或疼痛者,因其可能转为恶性,故要尽早做活检。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "活检" } ] }, { "text": "继发于神经纤维瘤病的患者,恶性程度更高。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "神经纤维瘤" } ] }, { "text": "由于肿瘤与主要神经干有关,一般局部完全切除肿瘤,还要截肢,不必做淋巴结清扫。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "神经干" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "局部完全切除" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "肿瘤" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "截肢" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "淋巴结清扫" } ] }, { "text": "该肿瘤放疗无效,可按横纹肌肉瘤方案化疗,但效果不肯定。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "放疗" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "化疗" } ] }, { "text": "第三节室间隔缺损室间隔缺损(ventricularseptaldefect,VSD)是最常见的先天性心血管畸形,可占先心病人的20%。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 14, "end_idx": 36, "type": "dis", "entity": "ventricularseptaldefect" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "VSD" }, { "start_idx": 47, "end_idx": 54, "type": "dis", "entity": "先天性心血管畸形" } ] }, { "text": "【病理解剖】在所有室间隔缺损的分类方法中,Soto等提出的分类法更有利于理解缺损的转归、累及的瓣膜和类似房室间通道的缺口大小。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "缺损" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "瓣膜" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "房室间通道" } ] }, { "text": "从右心室面观察,根据缺损边界,可将室间隔缺损分为膜周部缺损、肌部缺损及双动脉下型缺损(图9-9)。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "右心室" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "缺损" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "膜周部缺损" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "肌部缺损" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "双动脉下型缺损" } ] }, { "text": "图9-18间隔缺损的部位(一)膜周部缺损占室间隔缺损的85%,缺损的边缘由纤维组织构成。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "间隔缺损" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "膜周部缺损" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "缺损" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "纤维组织" } ] }, { "text": "缺损可以存在于室间隔肌部、流入部或流出部。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "缺损" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "室间隔肌部" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "流入部" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "流出部" } ] }, { "text": "若缺损累及房室瓣叶与膜部室间隔之间的接合部,二尖瓣和三尖瓣间的纤维连接将会增强。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "缺损" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "房室瓣叶" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "膜部室间隔" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "三尖瓣" } ] }, { "text": "正常情况下,流入部室间隔将右心室流入部和左心室流出部隔开,当此处的膜部室间隔缺损时,该处的间隔会变小,甚至出现左心室向右心房的分流。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "bod", "entity": "右心室流入部" }, { "start_idx": 20, "end_idx": 25, "type": "bod", "entity": "左心室流出部" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "左心室" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "右心房" } ] }, { "text": "缺损部位可部分或全部被三尖瓣纤维组织覆盖,形成“假性室隔瘤”;主动脉瓣脱垂(aorticvalveprolapse)也会盖于缺损的室间隔上,使心室间的分流量减少。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "缺损" }, { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "三尖瓣纤维组织" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "假性室隔瘤" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "主动脉瓣脱垂" }, { "start_idx": 38, "end_idx": 56, "type": "dis", "entity": "aorticvalveprolapse" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "缺损" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "室间隔" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "心室" } ] }, { "text": "(二)肌部缺损肌部缺损约占所有室间隔缺损的10%,边界全由肌性组织组成。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "肌部缺损" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "肌部缺损" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "肌性组织" } ] }, { "text": "缺损可位于心尖部、流入道或流出道的肌部室间隔。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "缺损" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "心尖部" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "流入道" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "流出道" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "肌部室间隔" } ] }, { "text": "它可以呈多发小孔,亦可伴有膜周部或双动脉下缺损。", "entities": [ { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "膜周部或双动脉下缺损" } ] }, { "text": "多发小孔的肌部缺损存在于心尖室间隔肌小梁之间,产生“Swiss-cheese”现象,它们可随年龄或肌小梁的肥厚而自行闭合;位于流出道部的肌部缺损也可随周围心肌的生长而自然闭合,此处分流量可为脱垂的主动脉瓣覆盖而减少;开口于流入道的肌部缺损可被三尖瓣瓣叶覆盖。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "肌部缺损" }, { "start_idx": 12, "end_idx": 19, "type": "bod", "entity": "心尖室间隔肌小梁" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "肌小梁" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "流出道部" }, { "start_idx": 68, "end_idx": 71, "type": "dis", "entity": "肌部缺损" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "心肌" }, { "start_idx": 98, "end_idx": 101, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 115, "end_idx": 118, "type": "dis", "entity": "肌部缺损" }, { "start_idx": 121, "end_idx": 125, "type": "bod", "entity": "三尖瓣瓣叶" } ] }, { "text": "这种类型缺损与膜周部缺损不同,其传导束位于缺损的前上方。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "缺损" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "膜周部缺损" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "缺损" } ] }, { "text": "(三)双动脉下型缺损此型在西方国家较少见,只占室缺的5%主动脉瓣在东方人中则有30%。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "双动脉下型缺损" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "室缺" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "主动脉瓣" } ] }, { "text": "其主要特征是在主动脉瓣和肺动脉瓣之间有纤维连接。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "主动脉瓣" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "肺动脉瓣" } ] }, { "text": "冠脉瓣脱垂可减少左向右分流,但却常引起主动脉反流。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "冠脉瓣脱垂" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "主动脉反流" } ] }, { "text": "此类型的传导束由缺损部位间接发出。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "缺损" } ] }, { "text": "【病理生理】室间隔缺损引起心脏左向右分流,其分流程度取决于缺损大小及肺循环阻力。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心脏" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "缺损" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肺" } ] }, { "text": "出生早期因肺静脉阻力高,分流量小;而后肺小血管肌层逐渐舒张,肺血管阻力下降,分流量遂增多。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肺静脉" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "肺小血管肌层" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "肺血管" } ] }, { "text": "大型缺损,因要避免肺血流过多,肺小血管收缩,这一过程往往延迟。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "缺损" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肺" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "肺小血管" } ] }, { "text": "若肺静脉回流血增多,会使左心房、左心室负荷增加,心脏容量超负荷及继发性肺高压可最终导致充血性心力衰竭产生。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "肺静脉" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "左心房" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "左心室" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "心脏" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "继发性肺高压" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "充血性心力衰竭" } ] }, { "text": "这种代偿机制包括Frank-Starling机制、交感兴奋及心肌肥厚。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "交感兴奋" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "心肌肥厚" } ] }, { "text": "肺动脉血流过多引起肺血管肌层肥厚,内膜增生,可导致肺小动脉结构破坏,产生不可逆的肺血管疾病,此时左向右的分流量可减少。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肺动脉" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "肺血管肌层肥厚" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "内膜增生" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "肺小动脉" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "肺血管疾病" } ] }, { "text": "当肺血管破坏进一步发展,肺循环阻力进一步增高,右心室压力明显增加,大于左心室内压力,可以出现右向左分流,体循环缺氧;极少情况下,小儿出生后未有肺小血管平滑肌舒张,肺循环阻力高,左右心室压力相近,存在双向分流而没有充血性心衰的症状和体征。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "肺血管" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肺" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "右心室" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "左心室" }, { "start_idx": 71, "end_idx": 77, "type": "bod", "entity": "肺小血管平滑肌" }, { "start_idx": 88, "end_idx": 91, "type": "bod", "entity": "左右心室" }, { "start_idx": 106, "end_idx": 110, "type": "dis", "entity": "充血性心衰" } ] }, { "text": "这两种情况,与Eisenmenger综合征晚期无多大区别。", "entities": [ { "start_idx": 7, "end_idx": 20, "type": "dis", "entity": "Eisenmenger综合征" } ] }, { "text": "除了肺血管疾病以外,其他导致左向右分流量减少的因素有:①右室圆锥部进行性肥厚造成狭窄,右心室流出道梗阻,临床上出现类似法洛四联症表现,而室缺本身症状被掩盖;②缺口由“瘤突”纤维或脱垂的主动脉瓣覆盖,而动脉下缺损常由脱垂的冠脉瓣覆盖,引起分流量的减少;③缺损可能自然缩小或完全关闭。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肺血管疾病" }, { "start_idx": 28, "end_idx": 37, "type": "dis", "entity": "右室圆锥部进行性肥厚" }, { "start_idx": 43, "end_idx": 50, "type": "dis", "entity": "右心室流出道梗阻" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "室缺" }, { "start_idx": 83, "end_idx": 84, "type": "dis", "entity": "瘤突" }, { "start_idx": 92, "end_idx": 95, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "动脉" }, { "start_idx": 103, "end_idx": 104, "type": "dis", "entity": "缺损" }, { "start_idx": 110, "end_idx": 112, "type": "bod", "entity": "冠脉瓣" }, { "start_idx": 126, "end_idx": 127, "type": "dis", "entity": "缺损" } ] }, { "text": "【临床表现】(一)小型缺损患儿无症状,通常是在体格检查时意外发现心脏杂音。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "小型缺损" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "体格检查" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "心脏" } ] }, { "text": "胸壁无畸形,左心室大小正常,外周血管搏动无异常。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "左心室" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "外周血管" } ] }, { "text": "主要体征为:胸骨左下缘有一响亮的收缩期杂音,常伴有震颤,杂音多为全收缩期;系动脉下缺损身体缩期杂音,常伴有震颤,杂音多为全收缩期;如系动脉下缺损,杂音和震颤则局限于胸骨左上缘。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "胸骨左下缘" }, { "start_idx": 6, "end_idx": 35, "type": "sym", "entity": "胸骨左下缘有一响亮的收缩期杂音,常伴有震颤,杂音多为全收缩期" }, { "start_idx": 82, "end_idx": 86, "type": "bod", "entity": "胸骨左上缘" } ] }, { "text": "对于小的肌部缺损,杂音特征为胸骨左下缘短促高亢的收缩期杂音,由于心肌收缩时肌小梁间的孔洞缩小或密闭,杂音于收缩中期终止。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "肌部缺损" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "胸骨左下缘" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "心肌" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肌小梁" } ] }, { "text": "心脏杂音的强弱与室间隔缺损的大小无直接关系。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心脏" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "(二)中型至大型缺损患儿常在生后1~2个月肺循环阻力下降时出现临床表现。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "缺损" } ] }, { "text": "由于肺循环流量大产生肺水肿,肺静脉压力增高,肺顺应性下降,出现吮乳困难,喂养时易疲劳、大量出汗,体重减轻,后渐出现身高发育延迟,呼吸急促,易反复呼吸道感染,进一步加剧心力衰竭形成。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "肺水肿" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "肺静脉压力增高" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "肺顺应性下降" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "吮乳困难" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "喂养时易疲劳" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "大量出汗" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "体重减轻" }, { "start_idx": 53, "end_idx": 62, "type": "sym", "entity": "后渐出现身高发育延迟" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 69, "end_idx": 76, "type": "sym", "entity": "易反复呼吸道感染" }, { "start_idx": 78, "end_idx": 88, "type": "sym", "entity": "进一步加剧心力衰竭形成" } ] }, { "text": "体格检查:小儿面色红润,反应稍差,脉率增快强弱正常,但当有严重心力衰竭或有很大的左向右分流时,脉搏减弱。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "体格检查" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "脉率" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "严重心力衰竭" }, { "start_idx": 47, "end_idx": 48, "type": "ite", "entity": "脉搏" } ] }, { "text": "患儿呼吸困难出现呼吸急促、肋间隙内陷。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "呼吸急促" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "肋间隙内陷" } ] }, { "text": "触诊,心尖搏动外移,有左心室抬举感,胸骨左下缘常可触及收缩期震颤。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "触诊" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "心尖搏动外移" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "左心室" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "胸骨左下缘" } ] }, { "text": "听诊第二心音响亮,如有肺高压时,胸骨左下缘可闻及典型的全收缩期杂音。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "听诊" }, { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "第二心音响亮" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肺高压" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "胸骨左下缘" } ] }, { "text": "如系动脉下缺损型,杂音通常以胸骨左缘第二肋间隙最为明显,当有大的左向右分流时,在心尖部可闻及第三心音及舒张中期隆隆样杂音。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "系动脉下缺损型" }, { "start_idx": 14, "end_idx": 22, "type": "bod", "entity": "胸骨左缘第二肋间隙" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "心尖部" } ] }, { "text": "与之相比,当小儿长至6月~2岁,心力衰竭比例反而可以下降。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "这可能由于缺损自然闭合、瓣膜纤维组织及脱垂的瓣叶覆盖缺口、右室圆锥部狭窄或肺循环阻力增高使左向右分流减少的缘故。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "缺损" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "瓣膜纤维组织" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "脱垂的瓣叶" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "右室圆锥部狭窄" } ] }, { "text": "随着肺血管压力增高,分流量的减少,心前区搏动逐渐减弱而仅出现严重的肺高压表现:第二心音亢进、单一,收缩期杂音短促最终消失。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺血管" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "肺高压" } ] }, { "text": "若有肺动脉反流,在胸骨左缘尚可闻及舒张期杂音;如出现三尖瓣相对关闭不全,有严重三尖瓣反流,则于胸骨左下缘可及全收缩期杂音。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺动脉" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "胸骨左缘" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "三尖瓣反流" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "胸骨左下缘" } ] }, { "text": "在十几岁的小儿中,更常见因出现右向左分流而引起的发绀。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "发绀" } ] }, { "text": "少数患儿,出生后肺循环压力未降,其主要表现为肺动脉高压,而心力衰竭症状不明显。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "当右室圆锥部进行性肥厚,右心室增大的体征可较左心室更明显。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "右室圆锥部" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "右心室增大" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "左心室" } ] }, { "text": "如出现右心室流出道梗阻时,第二心音变弱。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "右心室流出道梗阻" } ] }, { "text": "若狭窄进一步加重,左右心室收缩期压力平衡,全收缩期杂音减弱甚至消失,于胸骨左上缘可及响亮的收缩期喷射性杂音。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "左右心室" }, { "start_idx": 21, "end_idx": 32, "type": "sym", "entity": "全收缩期杂音减弱甚至消失" } ] }, { "text": "主动脉瓣脱垂可引起主动脉反流,因左心室舒张末期容量增加,可出现洪脉,心尖搏动外移及特征性的胸骨左缘高亢的舒张期吹风样杂音。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "左心室" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "洪脉" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "心尖" } ] }, { "text": "【辅助检查】(一)X线胸片小型室缺小儿X线胸片常完全正常。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "X线" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "胸片" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "小型室缺" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "X线" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "胸片" } ] }, { "text": "有大型缺损、分流量大、左心室超容的小儿,胸片表现为心影向左下扩大、左心房扩大、肺野淤血;如出现肺动脉高压,肺动脉干突出,右室肥厚,心尖上翘。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "左心室超容" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "胸片" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "心影向左下扩大" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "左心房" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "左心房扩大" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "肺野淤血" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "肺动脉" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "肺动脉高压" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "肺动脉干" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "右室" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "右室肥厚" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "心尖" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "心尖上翘" } ] }, { "text": "若系双动脉下型缺损,由于大量快速分流的血流直接撞击肺动脉,肺动脉干也突出。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "双动脉下型缺损" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肺动脉" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "肺动脉干" } ] }, { "text": "肺血管疾病的特征表现为肺动脉干及其主支很粗,但周围血管影不粗甚至变细。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肺血管疾病" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "肺动脉干" } ] }, { "text": "而因肺循环阻力很高,左向右分流量减少,所以心影可正常。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "心影" } ] }, { "text": "(二)心电图缺损小的儿童心电图类似完全正常。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "心电图" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "缺损" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "心电图" } ] }, { "text": "大型缺损儿童可出现左室肥厚表现:Ⅱ、Ⅲ、aVF、V5、V6深Q波,R波高大,T波高尖;左心房大,P波变宽。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "左室" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "左室肥厚" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "左心房" }, { "start_idx": 16, "end_idx": 73, "type": "sym", "entity": "Ⅱ、Ⅲ、aVF、V5、V6深Q波,R波高大,T波高尖;左心房大,P波变宽" } ] }, { "text": "流入部室间隔缺损可出现电轴左偏。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "有肺高压、右心室增大时,V1呈rsR。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "肺高压" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "右心室增大" } ] }, { "text": "右心室压力增高时,右胸导联R波高电压、T波直立。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "右胸" } ] }, { "text": "当有严重右心室流出道梗阻或肺血管病变时,心电图呈右心室占优势的图形。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "右心室流出道梗阻" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "肺血管病变" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "心电图" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "右心室" } ] }, { "text": "(三)超声心动图二维超声可直接显示缺损的位置。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "二维超声" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "缺损" } ] }, { "text": "流入道缺损可由心尖和肋下四腔位看到;稍向前移,所取的平面即可看到膜周部的缺损。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "流入道缺损" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心尖" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "肋下四腔位" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "缺损" } ] }, { "text": "以这些平面,还可看到来源于三尖瓣瓣叶的“瘤突”。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "三尖瓣瓣叶" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "瘤突" } ] }, { "text": "胸骨旁短轴平面时,若存在膜周部缺损和“瘤突”,则其位于10点位;漏斗部肥厚也可在此时看到。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胸骨" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "缺损" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "瘤突" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "漏斗部肥厚" } ] }, { "text": "另外,动脉下缺损于1点位可看到主肺动脉瓣联合部纤维。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "动脉下缺损" }, { "start_idx": 15, "end_idx": 24, "type": "bod", "entity": "主肺动脉瓣联合部纤维" } ] }, { "text": "如有主动脉瓣脱垂,可通过胸骨旁长、短轴清楚看到(图9-9)。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "主动脉瓣脱垂" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胸骨" } ] }, { "text": "前肌部缺损可通过长轴探察。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "前肌部缺损" } ] }, { "text": "心尖部的多发小孔可从心尖、剑突下、短轴到达二尖瓣、心尖等邻近部位观察。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "心尖部" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心尖" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "剑突下" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "短轴" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "心尖" } ] }, { "text": "彩色血流显像对上述缺损的定位更有帮助。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "彩色血流显像" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "缺损" } ] }, { "text": "图9-19经食管超声显示:贯穿动脉下室间隔缺损的右冠状动脉瓣脱垂(RCC)(箭头所示)LV:左心室RV:右心室通过无创的Doppler超声,运用Bernoulli校正公式可估计肺动脉压力。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "食管超声" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "贯穿动脉下室间隔缺损" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "右冠状动脉瓣脱垂" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "RCC" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "左心室" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "右心室" }, { "start_idx": 60, "end_idx": 68, "type": "pro", "entity": "Doppler超声" }, { "start_idx": 88, "end_idx": 92, "type": "ite", "entity": "肺动脉压力" } ] }, { "text": "心室间的压力阶差可由通过缺损处血流速度推算。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心室" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "缺损" } ] }, { "text": "收缩期肺动脉压力通过测收缩期体循环压力及心室间压力阶差后计算得出(假设无右心室流出道梗阻)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肺动脉" }, { "start_idx": 11, "end_idx": 18, "type": "ite", "entity": "收缩期体循环压力" }, { "start_idx": 20, "end_idx": 24, "type": "ite", "entity": "心室间压力" }, { "start_idx": 36, "end_idx": 43, "type": "dis", "entity": "右心室流出道梗阻" } ] }, { "text": "同样,左房、左室的方位为左向右分流的大小提供了足够的信息。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "左房" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "左室" } ] }, { "text": "(四)心导管目前,由于超声心动图能提供足够的解剖学及血流动力学方面的信息,故诊断性的心导管检查一般很少用。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "心导管" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "超声心动图" }, { "start_idx": 42, "end_idx": 46, "type": "pro", "entity": "心导管检查" } ] }, { "text": "但是,当存在中等大小的左向右分流时,仍然需通过心导管检查明确室间隔缺损的大小,以选择具体的手术方案。", "entities": [ { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "心导管检查" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "对怀疑可能有肺血管疾病的小儿,可行心导管检查明确肺血管病变的可逆程度。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "肺血管疾病" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "心导管检查" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "肺血管病变" } ] }, { "text": "若测得的优势氧饱和度高于正常,即说明在心室水平存在左向右分流,通过Fick原理,可计算出分流量。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "ite", "entity": "优势氧饱和度" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心室" } ] }, { "text": "当缺损呈中至大型,肺动脉压力可以升高,对于那些肺血管阻力过大而不能手术者,可通过吸入100%氧及NO气体,经心导管估计肺血管阻力下降程度。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "缺损" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肺动脉" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "肺血管" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "手术" }, { "start_idx": 40, "end_idx": 51, "type": "pro", "entity": "吸入100%氧及NO气体" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "心导管" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "肺血管" } ] }, { "text": "同样,虽然肺活体组织检查也可了解肺血管病变,但一旦取样不当仍可造成误诊。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "肺活体组织检查" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "肺血管病" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "肺血管病" } ] }, { "text": "左心导管可测得心室缺损的数量、大小、位置。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "左心导管" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "缺损" } ] }, { "text": "位于中部和心尖部的室间隔膜周部及肌部缺损及肌部缺损可通过长轴斜径或四腔位显示,而动脉下缺损和前室间隔肌部缺损可通过右前斜径显像。", "entities": [ { "start_idx": 9, "end_idx": 19, "type": "dis", "entity": "室间隔膜周部及肌部缺损" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "动脉下缺损" }, { "start_idx": 46, "end_idx": 53, "type": "bod", "entity": "前室间隔肌部缺损" } ] }, { "text": "对于需行导管闭合术的病人,一个清晰的血管造影定位尤其重要。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "导管闭合术" } ] }, { "text": "升主动脉造影术用来估计伴发的主动脉瓣脱垂主动脉瓣脱垂及主动脉反流的程度(图9-9)。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "升主动脉造影术" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "主动脉瓣脱垂" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "主动脉瓣脱垂" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "主动脉" } ] }, { "text": "右心室造影术可显示肺动脉漏斗部的狭窄程度。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "右心室造影术" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "肺动脉漏斗部" } ] }, { "text": "图9-20升主动脉血管造影右前斜位片显示:右冠状尖脱垂和畸形【治疗】(一)药物治疗小型缺损者无须治疗。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "升主动脉血管造影" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "右冠状尖脱垂和畸形" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "小型缺损" } ] }, { "text": "然而,在进行可能导致短暂菌血症如牙科或其他创伤性治疗以前,为避免细菌性心内膜炎的发生,需事先用抗生素预防。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "菌血症" }, { "start_idx": 16, "end_idx": 17, "type": "dep", "entity": "牙科" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "细菌性心内膜炎" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "抗生素" } ] }, { "text": "③血管扩张剂如依那普利和卡托普利能有效降低体循环的超负荷状况。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dru", "entity": "血管扩张剂" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "依那普利" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "卡托普利" } ] }, { "text": "在长期使用这些药的过程中,应定期检测血电解质、地高辛水平、肾功能情况。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "ite", "entity": "血电解质" }, { "start_idx": 23, "end_idx": 27, "type": "ite", "entity": "地高辛水平" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "肾功能" } ] }, { "text": "当药物治疗无效,则表明需尽早实施手术治疗。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "药物治疗" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "手术" } ] }, { "text": "(二)外科治疗对于不伴其他畸形的单纯室间隔缺损,手术指征为:药物不能控制心力衰竭;有大的左向右分流,出现活动受限、反应差的;肺高压反复肺部感染者。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dep", "entity": "外科" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "单纯室间隔缺损" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "手术" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 52, "end_idx": 59, "type": "sym", "entity": "活动受限、反应差" }, { "start_idx": 62, "end_idx": 70, "type": "dis", "entity": "肺高压反复肺部感染" } ] }, { "text": "若肺∶主动脉血流量大于2,说明至少存在中型缺损,需要行手术关闭缺口;该比值不到2∶1,不会出现肺动脉高压,一般先以药物治疗,1~2岁后复查心导管了解左向右分流量的变化及肺血管阻力;如果5~6岁小儿肺动脉压力仍持续高于主动脉50%,为减少肺血管疾病的发生,也需要进行手术。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肺" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "主动脉" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "中型缺损" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "手术关闭缺口" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "药物治疗" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "心导管" }, { "start_idx": 84, "end_idx": 86, "type": "bod", "entity": "肺血管" }, { "start_idx": 98, "end_idx": 100, "type": "bod", "entity": "肺动脉" }, { "start_idx": 108, "end_idx": 110, "type": "bod", "entity": "主动脉" }, { "start_idx": 118, "end_idx": 122, "type": "dis", "entity": "肺血管疾病" }, { "start_idx": 132, "end_idx": 133, "type": "pro", "entity": "手术" } ] }, { "text": "大多数病人在3~12个月时做手术,在这年龄阶段行室间隔缺损修补术,肺动脉压力可恢复正常。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "手术" }, { "start_idx": 24, "end_idx": 31, "type": "pro", "entity": "室间隔缺损修补术" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "肺动脉" } ] }, { "text": "在心血管治疗中心,现在行手术修补室间隔缺损的婴儿死亡率接近于0%。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "心血管" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "手术" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "术后早期并发症有:心室功能不良引起的心脏低位流出道综合征,完全性传导阻滞,肺动脉高压危象。", "entities": [ { "start_idx": 9, "end_idx": 27, "type": "dis", "entity": "心室功能不良引起的心脏低位流出道综合征" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "完全性传导阻滞" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "肺动脉高压危象" } ] }, { "text": "大多室间隔缺损可经心房路径修复,此外,通过该路径亦可切除肥厚的漏斗部肌肉;动脉下型缺损可经主动脉瓣路径,但对一些肌部缺损的关闭手术必须以左心室或右心室为入口;有多个孔洞的肌部缺损小儿手术难度较大,先行肺动脉环缩术可减少分流量,1~2年后再行缺损关闭手术。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "经心房路径修复" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "漏斗部肌肉" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "动脉下型缺损" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "肌部缺损" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "关闭手术" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "左心室" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "右心室" }, { "start_idx": 85, "end_idx": 88, "type": "dis", "entity": "肌部缺损" }, { "start_idx": 91, "end_idx": 92, "type": "pro", "entity": "手术" }, { "start_idx": 100, "end_idx": 105, "type": "pro", "entity": "肺动脉环缩术" }, { "start_idx": 120, "end_idx": 125, "type": "pro", "entity": "缺损关闭手术" } ] }, { "text": "近几年镶嵌治疗,即手术加导管治疗在治疗多发孔洞型室间隔缺损,尤其是肌部室间隔缺损越来越成熟、普遍。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "镶嵌治疗" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "手术加导管治疗" }, { "start_idx": 19, "end_idx": 28, "type": "dis", "entity": "多发孔洞型室间隔缺损" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "肌部室间隔缺损" } ] }, { "text": "动脉下缺损并发主动脉瓣疾病是早期手术的指征,而不是取决于分流量的大小。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "动脉下缺损" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "主动脉瓣疾病" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "手术" } ] }, { "text": "但是,对于无主动脉瓣畸形的室间隔缺损,手术治疗的必要性尚有争议:有人主张为了避免主动脉瓣并发症的出现,所有的动脉下型缺损均应手术治疗。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "无主动脉瓣畸形" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "手术" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "主动脉瓣并发症" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "动脉下型缺损" }, { "start_idx": 62, "end_idx": 63, "type": "pro", "entity": "手术" } ] }, { "text": "而最近研究表明,小于5mm的缺损不可能引起主动脉瓣畸形及主动脉反流,缺损小于5mm没有临床症状的病人可仅以药物保守治疗;对伴有严重主动脉瓣脱垂和主动脉反流的患儿,除了行缺损关闭术外,还需行主动脉瓣修复手术。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "缺损" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "主动脉瓣畸形" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "主动脉反流" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "缺损" }, { "start_idx": 53, "end_idx": 58, "type": "pro", "entity": "药物保守治疗" }, { "start_idx": 63, "end_idx": 70, "type": "dis", "entity": "严重主动脉瓣脱垂" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "主动脉反流" }, { "start_idx": 84, "end_idx": 89, "type": "pro", "entity": "缺损关闭术外" }, { "start_idx": 94, "end_idx": 101, "type": "pro", "entity": "主动脉瓣修复手术" } ] }, { "text": "大型室缺引起严重肺高压者,在决定是否手术以前,先要仔细了解肺血管阻力和肺血管扩张术后肺血管阻力下降程度。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "大型室缺" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "严重肺高压" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "肺血管" }, { "start_idx": 35, "end_idx": 40, "type": "pro", "entity": "肺血管扩张术" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "肺血管" } ] }, { "text": "行心导管术时,控制吸入100%氧及NO气体,可观察肺血管的反应程度。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "心导管术" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肺血管" } ] }, { "text": "肺循环阻力大于8Wood单位•平方米,通常列为手术禁忌。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "手术" } ] }, { "text": "(三)经导管介入治疗目前,已有很多填补装置用于经导管闭合室间隔缺损管闭合室间隔缺损的治疗中。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "经导管介入治疗" }, { "start_idx": 17, "end_idx": 20, "type": "equ", "entity": "填补装置" }, { "start_idx": 23, "end_idx": 32, "type": "pro", "entity": "经导管闭合室间隔缺损" } ] }, { "text": "用于填塞缺损的装置有Clamshell伞、Rashkind伞、Sideris纽扣等。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "缺损" }, { "start_idx": 10, "end_idx": 19, "type": "equ", "entity": "Clamshell伞" }, { "start_idx": 21, "end_idx": 29, "type": "equ", "entity": "Rashkind伞" }, { "start_idx": 31, "end_idx": 39, "type": "equ", "entity": "Sideris纽扣" } ] }, { "text": "这些装置最大的限制在于使用时需要有大的传导系统和相关的复杂置入技术,且对于填塞物的复位、调换及残留缺损的修复则无能为力。", "entities": [ { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "缺损" } ] }, { "text": "近来,Amplatzer室缺填塞装置,尤其对于肌部型缺损非常有用。", "entities": [ { "start_idx": 3, "end_idx": 17, "type": "equ", "entity": "Amplatzer室缺填塞装置" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "肌部型缺损" } ] }, { "text": "Thanopoulos等报道其对8名2~10岁患儿肌部缺损的治疗,2名患儿缺损即刻关闭,其余5名在术后24小时内缺口也关闭,1名在术后6个月仍存在小的分流残余。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "肌部缺损" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "缺损" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "缺口也关闭" }, { "start_idx": 73, "end_idx": 78, "type": "sym", "entity": "小的分流残余" } ] }, { "text": "不同于肌部缺损,膜周部缺损因其接近于主动脉和三尖瓣以及缺口较大,使缺损修补难度加大。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "肌部缺损" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "膜周部缺损" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "主动脉" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "缺损" } ] }, { "text": "常见的并发症包括填塞物移位、主动脉瓣穿孔等,改良的Rashkind伞、Sideris纽扣可用于晚期并发症的修复。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "填塞物移位" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "主动脉瓣穿孔" }, { "start_idx": 25, "end_idx": 33, "type": "equ", "entity": "Rashkind伞" }, { "start_idx": 35, "end_idx": 43, "type": "equ", "entity": "Sideris纽扣" } ] }, { "text": "最近,新的改良Amplatzer室缺填塞装置已在临床上使用,该装置为一个左侧偏心固定圆片,在填补膜周部室间隔缺损时,不影响主动脉瓣的活动。", "entities": [ { "start_idx": 7, "end_idx": 21, "type": "equ", "entity": "Amplatzer室缺填塞装置" }, { "start_idx": 36, "end_idx": 43, "type": "equ", "entity": "左侧偏心固定圆片" }, { "start_idx": 48, "end_idx": 55, "type": "dis", "entity": "膜周部室间隔缺损" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "主动脉瓣" } ] }, { "text": "但是目前大型的膜周部缺损仍需以手术治疗。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "膜周部缺损" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "手术" } ] }, { "text": "【预后】小型缺损预后良好,自然关闭率高达75%~80%,大多在5岁内关闭。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "小型缺损" } ] }, { "text": "但小儿有患感染性心内膜炎的可能。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "感染性心内膜炎" } ] }, { "text": "大约10%~15%婴儿会发展为充血性心衰,需要药物或手术治疗;早期伴有肺动脉高压的小儿,1岁时可产生肺血管疾病,那些出生后肺血管阻力未降的儿童,青紫可为首发症状;出现艾森门格综合征的病人,多可活到40岁以上,其死因包括咯血、心力衰竭、感染性心内膜炎、脑脓疡、血栓栓塞、妊娠及心律失常引起的猝死。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "充血性心衰" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "手术" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "肺血管疾病" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "肺血管" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "青紫" }, { "start_idx": 83, "end_idx": 89, "type": "dis", "entity": "艾森门格综合征" }, { "start_idx": 109, "end_idx": 110, "type": "dis", "entity": "咯血" }, { "start_idx": 112, "end_idx": 115, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 117, "end_idx": 123, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 125, "end_idx": 127, "type": "dis", "entity": "脑脓疡" }, { "start_idx": 129, "end_idx": 132, "type": "dis", "entity": "血栓栓塞" }, { "start_idx": 134, "end_idx": 135, "type": "dis", "entity": "妊娠" }, { "start_idx": 137, "end_idx": 140, "type": "dis", "entity": "心律失常" }, { "start_idx": 144, "end_idx": 145, "type": "sym", "entity": "猝死" } ] }, { "text": "大多手术病人的远期疗效良好,少数出现心脏完全性传导阻滞,需要安置起搏器。", "entities": [ { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "心脏完全性传导阻滞" }, { "start_idx": 32, "end_idx": 34, "type": "equ", "entity": "起搏器" } ] }, { "text": "至于一过性的术后心脏传导阻滞也可发生晚期猝死。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "过性的术后心脏传导阻滞" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "猝死" } ] }, { "text": "同样,术后心室异位也易引起心室心律失常导致猝死。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "心室异位" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "心室心律失常" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "猝死" } ] }, { "text": "可常见小的残余分流,通常这些残余室缺不会引起血流动力学紊乱,但是仍需终生用抗生素预防细菌性心内膜炎。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "小的残余分流" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "抗生素" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "细菌性心内膜炎" } ] }, { "text": "结核病特别易在缺乏营养、住房拥挤、无医疗条件和无固定住所的人群中流行。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "结核病" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "缺乏营养" } ] }, { "text": "根据卫生部2000年进行的第四次全国结核病流行病学调查结果,我国结核菌感染率31.7%,近4.2亿人感染了结核分枝杆菌,推算2000年的年感染率为0.72%。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "结核病" }, { "start_idx": 32, "end_idx": 34, "type": "mic", "entity": "结核菌" }, { "start_idx": 53, "end_idx": 58, "type": "mic", "entity": "结核分枝杆菌" } ] }, { "text": "肺结核的患病率为367/10万,现有肺结核患者450余万。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肺结核" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "肺结核" } ] }, { "text": "每年结核病死亡的人数约13万。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "结核病" } ] }, { "text": "0~14岁儿童平均感染率为9.0%,全年龄组的结核感染率随年龄的增长而增高。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "结核" } ] }, { "text": "我国儿童结核病流行仍然处于较高水平,结核病仍是儿童常见的传染病。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "结核病" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "结核病" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "传染病" } ] }, { "text": "(二)传播结核病最主要的传播途径是呼吸道。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "结核病" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "呼吸道" } ] }, { "text": "当含有病原菌的微粒核被结核患者咳到空气中,又被易感者吸入肺内,到达肺泡,结核菌即得到传播。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "mic", "entity": "病原菌" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肺" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肺泡" }, { "start_idx": 36, "end_idx": 38, "type": "mic", "entity": "结核菌" } ] }, { "text": "这种极小的含有病原菌的微粒直径1~5μm,可以悬浮在空气中几个小时。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "病原菌" } ] }, { "text": "环境因素,如和患者接触区域的空间大小、通风情况等,都影响空气中病原菌的浓度。", "entities": [ { "start_idx": 31, "end_idx": 33, "type": "mic", "entity": "病原菌" } ] }, { "text": "与患者接触的时间长短也影响结核病传播的可能性。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "结核病" } ] }, { "text": "许多专家认为,结核病的传播需要易感者暴露在有结核杆菌的空气中4~8小时,因此,家庭成员间的接触是最可能的传播途径。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "结核病" }, { "start_idx": 22, "end_idx": 25, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "活动性空洞肺结核成人咳痰且痰涂片可见抗酸杆菌(AFB)者是结核病典型的传染源。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "活动性空洞肺结核" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "咳痰" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "痰涂片" }, { "start_idx": 18, "end_idx": 21, "type": "mic", "entity": "抗酸杆菌" }, { "start_idx": 23, "end_idx": 25, "type": "mic", "entity": "AFB" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "结核病" } ] }, { "text": "成人肺外结核病,除了喉结核,一般不具有传染性,因为这些患者体内的病原菌无法在空气中传播。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肺外结核病" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "喉结核" } ] }, { "text": "曾有在接触患者的尿液和分泌物后被感染的病例发生。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "尿液" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "分泌物" } ] }, { "text": "如果活的病原菌被吞下,结核杆菌也可以经胃肠道传播。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "mic", "entity": "病原菌" }, { "start_idx": 11, "end_idx": 14, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "胃肠道" } ] }, { "text": "偶尔,病原菌可以通过引流的伤口或被污染的敷料在空气中传播。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "mic", "entity": "病原菌" } ] }, { "text": "小于12岁的儿童即使是活动性肺结核通常也没有传染性,这是因为儿童患者体内所含的病原菌数目少,不如成人咳嗽的力度大,因而不能把结核杆菌咳出来。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "活动性肺结核" }, { "start_idx": 39, "end_idx": 41, "type": "mic", "entity": "病原菌" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "咳嗽" }, { "start_idx": 62, "end_idx": 65, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "有报道,医护人员可能因为接触活动结核病患儿的胃液或支气管灌洗液而感染结核,但这种病例非常少见。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "结核病" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "胃液" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "支气管灌洗液" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "结核" } ] }, { "text": "四、平滑肌肉瘤平滑肌肉瘤(leiomyosarcoma)可以发生于任何含有平滑肌的器官或组织,最多见于胃肠道;成人、儿童均如此,但成人多见。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "平滑肌肉瘤" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "平滑肌肉瘤" }, { "start_idx": 13, "end_idx": 26, "type": "dis", "entity": "leiomyosarcoma" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "平滑肌" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "胃肠道" } ] }, { "text": "肿瘤质地坚韧,大小不一,中央常有坏死,无被膜覆盖光镜下细胞呈长胞质丰富,核分裂象多见。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "肿瘤质地坚韧" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "大小不一" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "中央常有坏死" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "被膜" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "无被膜覆盖" }, { "start_idx": 24, "end_idx": 25, "type": "equ", "entity": "光镜" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "光镜下细胞呈长" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "胞质丰富" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "核分裂象多见" } ] }, { "text": "儿童平滑肌肉瘤,主要发生部位除胃肠道外尚包括腹膜后、气管、支气管和肺内的平滑肌。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "儿童平滑肌肉瘤" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "胃肠道" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "腹膜后" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "气管" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "支气管" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "肺内的平滑肌" } ] }, { "text": "一般胃肠道和皮下组织的肿瘤预后较好,腹膜后、肠系膜的预后较差。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "胃肠道" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "皮下组织" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "肿瘤" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "腹膜后" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肠系膜" } ] }, { "text": "体检可触及肿块,患儿可有贫血、腹痛、消化道出血等胃肠道的诊断可做X线钡餐造影,典型征象为边缘整齐的充盈缺损,也可有溃疡表现纤维胃镜、肠镜在诊断上都有作用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "体检" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "贫血" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "腹痛" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "消化道出血" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "消化道出血等" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "胃肠道" }, { "start_idx": 32, "end_idx": 37, "type": "pro", "entity": "X线钡餐造影" }, { "start_idx": 44, "end_idx": 52, "type": "sym", "entity": "边缘整齐的充盈缺损" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "溃疡" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "有溃疡表现" }, { "start_idx": 61, "end_idx": 64, "type": "equ", "entity": "纤维胃镜" }, { "start_idx": 66, "end_idx": 67, "type": "equ", "entity": "肠镜" } ] }, { "text": "肿瘤可经血行转移至肝脏,或种植于大网膜、腹膜,可有淋巴结转移。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肝脏" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "大网膜" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "腹膜" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "淋巴结" } ] }, { "text": "放疗和化疗有一定的疗效。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "放疗" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "化疗" } ] }, { "text": "六、医学伦理与遗传病从遗传学的观点看,人类既有大量优质基因,也存在相当数量的有害基因,两者构成我们的基因库。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "遗传病" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "基因" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "基因" } ] }, { "text": "但是,对于晚发的遗传病,是否要早期告诉病人,让他(她)在发病前的几十年中生活在阴影之中?", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "遗传病" } ] }, { "text": "诸如:保险公司或人事部门是否有权力知道每个人所携带的致病基因(个人隐私权)?", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "基因" } ] }, { "text": "带有“癌症基因”或“糖尿病基因”者是否会在社会上受到“基因歧视”,人们常说“癌症基因”或“糖尿病基因”,但事实是基因只是决定病症的一部分原因。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "癌症基因" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "基因" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "癌症基因" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "糖尿病基因" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "基因" } ] }, { "text": "进一步明了基因功能将帮助人们准确判断环境因素的影响,并指导我们避免或者减轻致病基因的作用。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "基因" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "基因" } ] }, { "text": "第四节室上性心动过速室上性心动过速(简称室上速)是小儿最常见的异位快速心律失常。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "室上速" }, { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "异位快速心律失常" } ] }, { "text": "从广义上讲,室上速是指异位激动在希氏束分叉以上的心动过速,主要由折返性机制产生,少数为自律性增高或并行心律。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "室上速" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "希氏束分叉" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "心动过速" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "自律性增高" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "并行心律" } ] }, { "text": "折返性室上速以房室折返性心动过速(旁道参与)最常见,其次为房室结折返性心动过速(双径路),后者的发生近年有增加的趋势。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "折返性室上速" }, { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "房室折返性心动过速" }, { "start_idx": 29, "end_idx": 38, "type": "dis", "entity": "房室结折返性心动过速" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "双径路" } ] }, { "text": "房性和交界性异位心动过速通常与心脏病变(如心肌病)或先天性心脏病术后有关。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "交界性异位心动过速" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "心脏病变" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "心肌病" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "【临床表现】折返性室上速的特点是突发突止,可由急性感染促发,通常在安静时发作。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "折返性室上速" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "急性感染" } ] }, { "text": "唯一的主诉可能是患儿感到心跳快。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "心跳快" } ] }, { "text": "如发作时心率极快,或发作持续时间长,可感到心前区不适,甚至可发生心力衰竭。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "心率" }, { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "心率极快" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "心前区" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "心前区不适" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "心力衰竭" } ] }, { "text": "在这一年龄段,患儿不能主诉症状,通常情况下心率本身就快,即使无室上速,哭吵时心率更快。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "心率" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "室上速" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "心率" } ] }, { "text": "新生儿室上速发作时为窄QRS波群(<0.08秒),仅在50%~60%的新生儿可见到P波,但在食管电极多可见到。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "室上速" } ] }, { "text": "与窦性心动过速的鉴别比较困难。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "窦性心动过速" } ] }, { "text": "如发作时心率大于230次/分,P波电轴异常(正常P波在Ⅰ导联及avF正向),则室上速的可能性大。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "心率" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "室上速" } ] }, { "text": "此外,室上速时心率比较固定,而窦性心动过速时心率易受迷走及交感张力的变化而变化。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "室上速" }, { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "心率" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "窦性心动" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "心率" } ] }, { "text": "P波缺乏,QRS波群宽大,与窦性时不同,则室速的可能性大。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "室速" } ] }, { "text": "与室上速有关的旁道有两种:隐匿性旁道与显性旁道(WPW综合征或预激综合征)。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "室上速" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "WPW综合征" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "预激综合征" } ] }, { "text": "室上速发作时,如冲动自房室结前传、旁道逆传,则QRS波群正常;如冲动自旁道前传,房室结逆传,则QRS波群宽大畸形。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "室上速" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "房室结前传" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "旁道" } ] }, { "text": "有前传功能的旁道比较容易发展成更严重的心律失常,特别是如发生房性颤动时。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "心律失常" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "房性颤动" } ] }, { "text": "【实验室检查】24小时动态心电图(HOLTER)可用于监视治疗过程及发现短阵无症状性室上速。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "心电图" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "室上速" } ] }, { "text": "电生理检查时,多极导管可置于心脏的不同部位,根据激动顺序可识别异位起搏点或旁道的位置。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "电生理检查" }, { "start_idx": 7, "end_idx": 10, "type": "equ", "entity": "多极导管" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心脏" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "异位起搏点" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "旁道" } ] }, { "text": "电生理检查时还可诱发心动过速,并评价药物疗效。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "电生理检查" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "心动过速" } ] }, { "text": "【治疗】刺激迷走神经,如在年长儿将脸浸于冰水,或在婴儿将冰袋置于脸上可终止发作。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "迷走神经" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "脸" } ] }, { "text": "一旦患儿转为窦性心律,应选用长效药物维持治疗。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "窦性心律" } ] }, { "text": "应该认识到,大多数抗心律失常药有致心律失常作用及负性肌收缩力作用。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "心律" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "心律失常" } ] }, { "text": "生后3、4个月内发生室上速的患儿复发的机会较年长儿低。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "室上速" } ] }, { "text": "【几种特殊类型的室上性心动过速】(一)心房异位性心动过速在儿科不常见,特征为心率不固定(很少超过200次/分),可见P波,但电轴异常,可慢性持续性或慢性阵发性。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 19, "end_idx": 27, "type": "dis", "entity": "心房异位性心动过速" }, { "start_idx": 29, "end_idx": 30, "type": "dep", "entity": "儿科" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "心率" } ] }, { "text": "这种类型的房性心动过速通常由单个异位自律病灶引起,而不是通常的折返机制。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "房性心动过速" } ] }, { "text": "有一简单方法识别这两种机制,即迷走或药物治疗时观察心电图。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "心电图" } ] }, { "text": "折返机制时心动过速突然停止,自律性心动过速时心率逐渐减慢,然后又逐渐增快。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "心动过速突然停止" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "自律性心动过速" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "心率" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "心率逐渐减慢" } ] }, { "text": "房性异位性心动过速较通常的折返性心动过速难以用药物控制。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "房性异位性心动过速" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "折返性心动过速" } ] }, { "text": "(二)紊乱性或多源性房性心动过速特征为三种或以上的异位P波,三种或以上的PP间期,常见P波阻滞,PR间期不等。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "房性心动过速" } ] }, { "text": "这种心律失常最常发生在1岁以下的婴儿,通常无心脏病变,但有些病例可能与病毒性心肌炎有关。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "心律失常" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "心脏" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "病毒性心肌炎" } ] }, { "text": "这种类型的心律失常药物治疗难以奏效,常需多种药物联合应用。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "心律失常" } ] }, { "text": "(三)加速性交界性异位性心动过速(JET)为自律性(非折返性)心动过速,交界心率超过窦性心率,因而可出现房室分离。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "加速性交界性异位性心动过速" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "JET" }, { "start_idx": 22, "end_idx": 34, "type": "dis", "entity": "自律性(非折返性)心动过速" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "心率" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "窦性心率" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "房室" } ] }, { "text": "这种心律失常最常见于心脏外科手术后早期,非常难以控制。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "心律失常" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "心脏外科手术" } ] }, { "text": "这些患儿的JET常未经特殊治疗而自发消失。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "JET" } ] }, { "text": "JET也可以是洋地黄中毒的表现,在这种情况下,洋地黄应停用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "JET" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "洋地黄" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "洋地黄" } ] }, { "text": "术后JET胺碘酮治疗比较有效。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "JET" }, { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "胺碘酮" } ] }, { "text": "需长期用药的患儿可选用胺碘酮或索他洛尔。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "胺碘酮" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "索他洛尔" } ] }, { "text": "参考文献1.TryggvasonK,ZhouJ,LiisaS,etal.MoleculargeneticsofAlportsyndrome.KidneyInt,1993,43(1):38-442.ReedersST.MoleculargeneticsofhereditaryNephritis.KidneyInt,1992,42(3):783-7833.KashtanCE,SzaboP,RichS,etal.TheAlportlocusassociatedwithadefectintypeIVcollagenNCIdomainmapstoregionXq21.3~q22.KidneyInt,1989,35:2054.KastanCE,KleppelMM,ButkowskiRJ,etal.Alportsyndrome,basementmembranousandcollagen.PediatrNephrol,1990,4(5):523-5235.Kincaid.SmithPS:Thinbasementmembranedisease,inTextbookofNephrology(Pt13)ditedbyMassryS,GlassockRJ.Baltimore:Lippincot,WilliamsandWilkins,1995:760-7606.MarkBuzza,YanYanWang,HayatDagher,etal.COL4A4mutationinthinbasementdiseasepreviouslydescribedinAlportsyndrome.KidneyInt,2001,60(2):480-4837.董德长.实用肾脏病学.第1版.上海:上海科技出版社,1999:687-6928.BodziakKA,HammondWS,MolitorisBA.Inheriteddiseaseoftheglomerularbasementmembrane.AmJkidneyDis,1994,23(4):605-6189.SchroderCH,BontempsCM,AssmannKJ,etal.Renalbiopsyandfamilystudiesin65childrenwithisolatedhematuria.ActaPaediatrScand,1990,79(6-6):630-63610.NogueiraM,CartwrightJJr,HornK,etal.Thinbasementmembranediseasewithheavyproteinuriaornephroticsyndromeatpresentation.AmJKidneyDis,2000,35(4):E1511.FrascaGM,Onetti-MudaA,RenieriA.Thinglomerularbasementmembranedisease.JNephrol.2000,13(1):15-1512.LemminkHH,NillesenWN,MochizukiT,etal.BenignfamilialhematuriaduetomutationofthetypeIVcollagenα4gene.JClinInvest,1996,98(5):1114-111413.PicciniM,CasariG,ZhouJ,etal.Evidenceforgeneticheterogeneityinbenignfamilialhematuria.AmJNephrol,1999,19(4):464-46714.SaitoT,NishiS,KarasawaR,etal.Anultrastructuralstudyofglomerularbasementmembraneinrheumatoidarthritispatientswithurinaryabnormalities.Clinnephrol,1995,43(6):360-36015.ColemanM,StirlingJW,LangfordLR,etal.GlomerularbasementmembranethinninginapatientwithhematuriaandhemoptysismimickingGoodpasture’ssyndrome.AmJNephrol,1994,14(1):47-4716.MoghalNE,MilfordDV,WhiteRHR,etal.CoexistenceofthinmembraneandAlportnephropathiesinfamilieswithhaematuria.PediatrNephrol,1999,13(9):778-77817.HebertLA,BettsJA,SedmakDD,etal.Loinpain-hematuriasyndromeassociatedwiththinglomerularbasementmembranediseaseandhemorrhageintorenaltubules.kidneyInt,1996,49(1):168-16818.易著文.小儿临床肾脏病学.北京:人民卫生出版社,1998:363-36319.KanekoK,SuzukiY,KiyaK,MatsubaraT,FukudaY,YabutaK.Minimalchangelesionincongenitalnephriticsyndrome.Twocasereportsandareviewoftheliterature.Nephron,1998,79(3):379-37920.SmeetsHI,KnoersVV,vandeHeuvelLP,LemminkHH,SchroderCH,MonneusLA.Hereditarydisordersoftheglomerularbasementmembrane.PediatrNephrol,1996,10(6):779-77921.GhoshAK,SakhujaV,JoshiK,CbughKS.CongenitalnepkroticsyndromeofFinnishtypewithglomemlarcrescents.JAssocPhysiciansIndia,1994,42(5):415-417", "entities": [ { "start_idx": 722, "end_idx": 724, "type": "dis", "entity": "肾脏病" }, { "start_idx": 2153, "end_idx": 2155, "type": "dis", "entity": "肾脏病" } ] }, { "text": "第六节脱落细胞学检查脱落细胞学是通过观察临床标本中脱落上皮细胞的形态和结构的变化,而诊断疾病的一门学科。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "脱落细胞学检查" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "上皮细胞" } ] }, { "text": "近年来随着各种标本采集、细胞学检查技术的提高,脱落细胞学得到了很大的发展。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "细胞学检查技术" } ] }, { "text": "诱导痰、纤维支气管镜下细胞刷涂片、支气管肺泡灌洗液(BALF)细胞成分检查等技术,大大提高了肺部疾病的检出率。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "诱导痰" }, { "start_idx": 4, "end_idx": 15, "type": "pro", "entity": "纤维支气管镜下细胞刷涂片" }, { "start_idx": 17, "end_idx": 36, "type": "pro", "entity": "支气管肺泡灌洗液(BALF)细胞成分检查" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "肺部疾病" } ] }, { "text": "一、鼻咽部脱落细胞检查鼻咽部脱落细胞检查对病毒性呼吸道感染的诊断具有较重要的参考价值。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "鼻咽部脱落细胞检查" }, { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "鼻咽部脱落细胞检查" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "病毒性呼吸道感染" } ] }, { "text": "常通过鼻咽部负压吸引,收集脱落细胞,进行病毒分离、病毒抗原检测或核酸测定。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "鼻咽部负压吸引" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "病毒" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "病毒抗原检测" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "核酸测定" } ] }, { "text": "光学显微镜检查可见纤毛细胞坏变,即纤毛柱状上皮细胞体积增大、胞质淡染或着色不均匀,胞质固缩、碎裂等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "equ", "entity": "光学显微镜" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "纤毛细胞" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "纤毛细胞坏变" }, { "start_idx": 17, "end_idx": 24, "type": "bod", "entity": "纤毛柱状上皮细胞" }, { "start_idx": 17, "end_idx": 28, "type": "sym", "entity": "纤毛柱状上皮细胞体积增大" }, { "start_idx": 30, "end_idx": 39, "type": "sym", "entity": "胞质淡染或着色不均匀" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "胞质" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "胞质固缩、碎裂" } ] }, { "text": "在感染的上皮细胞胞质和(或)胞核内,可有嗜酸性或嗜碱性包涵体。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "上皮细胞胞质" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "胞核" }, { "start_idx": 20, "end_idx": 29, "type": "bod", "entity": "嗜酸性或嗜碱性包涵体" } ] }, { "text": "第八节肺吸虫病肺吸虫病(paragonimiasis)由肺吸虫寄生于人体所引起。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "肺吸虫病" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "肺吸虫病" }, { "start_idx": 12, "end_idx": 25, "type": "dis", "entity": "paragonimiasis" }, { "start_idx": 28, "end_idx": 30, "type": "mic", "entity": "肺吸虫" } ] }, { "text": "临床症状以咯血为主。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "咯血" } ] }, { "text": "但因虫体在胸腔内移行的途径及病变部位不同而异。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "虫体" } ] }, { "text": "若囊性空洞已形成并与支气管相通时,出现血痰和少量咯血,但大量咯血少见。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "支气管" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "血痰" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "少量咯血" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "大量咯血少见" } ] }, { "text": "典型的痰液呈饴糖样、巧克力样或黏稠的铁锈色。", "entities": [ { "start_idx": 3, "end_idx": 20, "type": "sym", "entity": "痰液呈饴糖样、巧克力样或黏稠的铁锈色" } ] }, { "text": "转为慢性时,呈脓性痰伴血丝;咳嗽、咳痰逐渐加剧,活动后或晨起时可出现呼吸困难。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "脓性痰伴血丝" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "咳嗽、咳痰逐渐加剧" }, { "start_idx": 24, "end_idx": 37, "type": "sym", "entity": "活动后或晨起时可出现呼吸困难" } ] }, { "text": "虫体寄生部位可涉及多处,尤以肺下叶多见。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "虫体" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肺下叶" } ] }, { "text": "有时伴胸水或气胸。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "胸水" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "气胸" } ] }, { "text": "此外可累及中枢神经系统、肠壁、肝、脾、腹膜及皮下组织等,并引起相应症状。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肠壁" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肝" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "脾" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "腹膜" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "皮下组织" } ] }, { "text": "有时可在痰和粪便中找到虫卵,对流免疫电泳、琼脂扩散、放射免疫电泳、酶联免疫吸附试验等方法有一定的诊断参考价值。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "ite", "entity": "痰" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "粪便" }, { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "虫卵" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "对流免疫电泳" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "琼脂扩散" }, { "start_idx": 26, "end_idx": 31, "type": "pro", "entity": "放射免疫电泳" }, { "start_idx": 33, "end_idx": 40, "type": "pro", "entity": "酶联免疫吸附试验" } ] }, { "text": "主要依靠流行病学特点及临床特征,遇有可疑病例必须仔细检查粪便、痰液的虫卵。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "粪便" } ] }, { "text": "治疗首选吡喹酮75mg/kg,分3次口服,1日即可,具有效果好、疗程短、服用方便、副作用少等优点。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "吡喹酮" } ] }, { "text": "六、治疗疗程一般CAP抗感染治疗用至热退和主要呼吸道症状明显改善后3~7天。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "CAP" }, { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "抗感染治疗" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "热退" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "呼吸道" } ] }, { "text": "而HAP治疗通常不少于10天,以免感染反复。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "HAP" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "感染" } ] }, { "text": "对于毒力强的病原或有并发症的难治性肺炎应适当延长疗程。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "肺炎" } ] }, { "text": "肺炎链球菌肺炎疗程7~10天;流感嗜血杆菌肺炎疗程14天左右;葡萄球菌肺炎尤其MRAS、MRSE疗程宜适当延长,平均28天;肠杆菌肺炎14~21天左右;铜绿假单胞菌等肺炎21~28天左右;支原体、衣原体或嗜肺军团菌肺炎需21天或更长;真菌性肺炎则需1~2个月。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "肺炎链球菌肺炎" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "MRAS" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "MRSE" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "肠杆菌肺炎" }, { "start_idx": 76, "end_idx": 81, "type": "mic", "entity": "铜绿假单胞菌" }, { "start_idx": 83, "end_idx": 84, "type": "dis", "entity": "肺炎" }, { "start_idx": 94, "end_idx": 108, "type": "dis", "entity": "支原体、衣原体或嗜肺军团菌肺炎" }, { "start_idx": 117, "end_idx": 121, "type": "dis", "entity": "真菌性肺炎" } ] }, { "text": "第七节贫血的鉴别诊断一、概述引起小儿贫血的原因多种多样,种类繁多,且多数贫血最终有赖于详尽的实验室检查才能明确诊断。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "贫血" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "贫血" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "贫血" } ] }, { "text": "以下将简述贫血的鉴别诊断。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "贫血" } ] }, { "text": "(一)小细胞低色素性贫血(MCV、MCH、MCHC均<正常低限)1.缺铁性贫血2.珠蛋白合成障碍性贫血3.铁粒幼细胞性贫血4.慢性感染5.铅中毒6.慢性失血7.缺铜性贫血8.严重营养不良(二)巨幼红细胞性贫血(MCV、MCH>正常低限,MCHC正常)1.正常新生儿2.巨幼红细胞贫血(维生素B12或叶酸缺乏)3.再生障碍性贫血4.肝病5.切脾后6.多种原因所致红系造血代偿性增加7.阻塞性黄疸8.甲状腺功能减退(三)正细胞性贫血(MCV、MCH、MCHC均正常)1.急性失血2.急性再生障碍性贫血3.急性感染4.慢性肾炎、肾衰竭5.缺铁早期6.结缔组织病7.骨髓肿瘤细胞浸润8.恶性肿瘤全身播散9.肝脏病10.其他红系造血障碍(四)特殊形态红细胞1.球形红细胞(1)先天性球形红细胞增多症(2)自身免疫性溶血(3)不稳定性血红蛋白病2.遗传性椭圆形红细胞增多症3.遗传性口形红细胞增多症4.靶形红细胞、珠蛋白生成障碍性贫血、血红蛋白病5.镰状红细胞性贫血6.盔形、三角形红细胞(1)DIC(2)微血管内溶血(3)溶血尿毒综合征(4)人工瓣膜、体外循环等7.棘形红细胞(1)遗传性棘形红细胞增多症(2)遗传性无β球蛋白血症(3)严重肝病(4)烧伤", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "小细胞低色素性贫血" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "MCV" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "MCH" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "MCHC" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "缺铁性贫血" }, { "start_idx": 41, "end_idx": 50, "type": "dis", "entity": "珠蛋白合成障碍性贫血" }, { "start_idx": 53, "end_idx": 60, "type": "dis", "entity": "铁粒幼细胞性贫血" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "慢性感染" }, { "start_idx": 69, "end_idx": 71, "type": "sym", "entity": "铅中毒" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "慢性失血" }, { "start_idx": 80, "end_idx": 84, "type": "dis", "entity": "缺铜性贫血" }, { "start_idx": 87, "end_idx": 92, "type": "sym", "entity": "严重营养不良" }, { "start_idx": 96, "end_idx": 103, "type": "dis", "entity": "巨幼红细胞性贫血" }, { "start_idx": 105, "end_idx": 107, "type": "bod", "entity": "MCV" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "MCH" }, { "start_idx": 118, "end_idx": 121, "type": "bod", "entity": "MCHC" }, { "start_idx": 134, "end_idx": 140, "type": "dis", "entity": "巨幼红细胞贫血" }, { "start_idx": 155, "end_idx": 156, "type": "bod", "entity": "叶酸" }, { "start_idx": 162, "end_idx": 168, "type": "dis", "entity": "再生障碍性贫血" }, { "start_idx": 171, "end_idx": 172, "type": "dis", "entity": "肝病" }, { "start_idx": 176, "end_idx": 176, "type": "bod", "entity": "脾" }, { "start_idx": 197, "end_idx": 201, "type": "dis", "entity": "阻塞性黄疸" }, { "start_idx": 204, "end_idx": 206, "type": "bod", "entity": "甲状腺" }, { "start_idx": 214, "end_idx": 219, "type": "dis", "entity": "正细胞性贫血" }, { "start_idx": 221, "end_idx": 223, "type": "bod", "entity": "MCV" }, { "start_idx": 225, "end_idx": 227, "type": "bod", "entity": "MCH" }, { "start_idx": 229, "end_idx": 232, "type": "bod", "entity": "MCHC" }, { "start_idx": 239, "end_idx": 242, "type": "dis", "entity": "急性失血" }, { "start_idx": 245, "end_idx": 253, "type": "dis", "entity": "急性再生障碍性贫血" }, { "start_idx": 256, "end_idx": 259, "type": "sym", "entity": "急性感染" }, { "start_idx": 262, "end_idx": 265, "type": "dis", "entity": "慢性肾炎" }, { "start_idx": 267, "end_idx": 269, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 278, "end_idx": 282, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 285, "end_idx": 290, "type": "bod", "entity": "骨髓肿瘤细胞" }, { "start_idx": 295, "end_idx": 298, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 305, "end_idx": 307, "type": "dis", "entity": "肝脏病" }, { "start_idx": 326, "end_idx": 328, "type": "bod", "entity": "红细胞" }, { "start_idx": 331, "end_idx": 335, "type": "bod", "entity": "球形红细胞" }, { "start_idx": 339, "end_idx": 349, "type": "dis", "entity": "先天性球形红细胞增多症" }, { "start_idx": 353, "end_idx": 359, "type": "dis", "entity": "自身免疫性溶血" }, { "start_idx": 363, "end_idx": 371, "type": "dis", "entity": "不稳定性血红蛋白病" }, { "start_idx": 374, "end_idx": 385, "type": "dis", "entity": "遗传性椭圆形红细胞增多症" }, { "start_idx": 388, "end_idx": 398, "type": "dis", "entity": "遗传性口形红细胞增多症" }, { "start_idx": 418, "end_idx": 422, "type": "dis", "entity": "血红蛋白病" }, { "start_idx": 425, "end_idx": 432, "type": "dis", "entity": "镰状红细胞性贫血" }, { "start_idx": 441, "end_idx": 443, "type": "bod", "entity": "红细胞" }, { "start_idx": 453, "end_idx": 458, "type": "dis", "entity": "微血管内溶血" }, { "start_idx": 462, "end_idx": 468, "type": "dis", "entity": "溶血尿毒综合征" }, { "start_idx": 486, "end_idx": 488, "type": "bod", "entity": "红细胞" }, { "start_idx": 492, "end_idx": 502, "type": "dis", "entity": "遗传性棘形红细胞增多症" }, { "start_idx": 506, "end_idx": 515, "type": "dis", "entity": "遗传性无β球蛋白血症" }, { "start_idx": 521, "end_idx": 522, "type": "dis", "entity": "肝病" }, { "start_idx": 526, "end_idx": 527, "type": "dis", "entity": "烧伤" } ] }, { "text": "第五节衣原体肺炎衣原体是一种细胞内寄生的微生物,含DNA和RNA。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "衣原体肺炎" }, { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "衣原体" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "细胞" }, { "start_idx": 25, "end_idx": 27, "type": "mic", "entity": "DNA" }, { "start_idx": 29, "end_idx": 31, "type": "mic", "entity": "RNA" } ] }, { "text": "有沙眼衣原体、肺炎衣原体和鹦鹉热衣原体三种,均可引起上呼吸道感染和肺炎。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "mic", "entity": "沙眼衣原体" }, { "start_idx": 7, "end_idx": 11, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 13, "end_idx": 18, "type": "mic", "entity": "鹦鹉热衣原体" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "肺炎" } ] }, { "text": "一、沙眼衣原体肺炎沙眼衣原体肺炎(chlamydiatrachomatispneumonia)为沙眼衣原体(CT)引起。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "沙眼衣原体肺炎" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "沙眼衣原体肺炎" }, { "start_idx": 17, "end_idx": 45, "type": "dis", "entity": "chlamydiatrachomatispneumonia" }, { "start_idx": 48, "end_idx": 52, "type": "mic", "entity": "沙眼衣原体" }, { "start_idx": 54, "end_idx": 55, "type": "mic", "entity": "CT" } ] }, { "text": "多由受染的母亲传染或眼部感染经鼻泪管传入呼吸道。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "眼部" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "鼻泪管" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "呼吸道" } ] }, { "text": "国内研究表明,CT占婴儿肺炎的18.4%。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "mic", "entity": "CT" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "婴儿肺炎" } ] }, { "text": "本病潜伏期2~3周,症状多在出生后3~12周出现,起病缓慢,先有鼻塞,然后出现咳嗽和气促,一般不发热。", "entities": [ { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "鼻塞" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "咳嗽" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "气促" } ] }, { "text": "胸部X线检查可表现为肺间质性病变、斑片状浸润和肺气肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "胸部X线" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "肺间质性病变" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "斑片状浸润" }, { "start_idx": 23, "end_idx": 25, "type": "sym", "entity": "肺气肿" } ] }, { "text": "血象中白细胞总数正常,50%~70%患儿可有轻、中度嗜酸性粒细胞增多。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "血象" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "白细胞" }, { "start_idx": 26, "end_idx": 31, "type": "mic", "entity": "嗜酸性粒细胞" } ] }, { "text": "血IgG、IgM和lgA可增高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "血IgG" }, { "start_idx": 5, "end_idx": 7, "type": "mic", "entity": "IgM" }, { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "lgA" } ] }, { "text": "新生儿出生后3~12周发生肺炎、尤其是无热性肺炎者应考虑本病,并及时送鼻咽部分泌物或血标本作病原学检测。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "肺炎" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "无热性肺炎" }, { "start_idx": 35, "end_idx": 40, "type": "pro", "entity": "鼻咽部分泌物" }, { "start_idx": 42, "end_idx": 44, "type": "pro", "entity": "血标本" } ] }, { "text": "【诊断】(一)目前诊断存在的问题抽动障碍的诊断目前仍以临床现象学诊断为主。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "这可以正确诊断抽动障碍和伴随的精神症状,有利于采取正确的治疗措施。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "国际研究有采用脑部功能磁共振(fMRI)和正电子扫描(PET)为辅助诊断,并引用基因诊断技术的趋势。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "磁共振" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "fMRI" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "正电子扫描" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "PET" } ] }, { "text": "根据抽动病程的长短与病情的轻重,人为地将抽动障碍分为短暂性抽动障碍、慢性抽动障碍和TS三种类型,它们三者之间具有连续性,其中TS是病程在一年以上的运动性和发声性抽动兼有的较重病例。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "TS" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "TS" } ] }, { "text": "由于抽动障碍的病因和发病机制迄今尚未明确,而各种检查包括神经系统软体征、脑电图(EEG)、诱发电位(EPs)、神经影像学检查(CT,MRI,SPECT,PET)、实验室检查和神经心理测验等,虽属客观指标,但这些检查仅在部分抽动障碍病人中发现有非特异性异常,只能作为诊断的辅助依据,目前尚未找到一种特异性的诊断手段来诊断本病。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 36, "end_idx": 38, "type": "pro", "entity": "脑电图" }, { "start_idx": 40, "end_idx": 42, "type": "pro", "entity": "EEG" }, { "start_idx": 55, "end_idx": 59, "type": "pro", "entity": "神经影像学" }, { "start_idx": 63, "end_idx": 64, "type": "pro", "entity": "CT" }, { "start_idx": 66, "end_idx": 68, "type": "pro", "entity": "MRI" }, { "start_idx": 70, "end_idx": 74, "type": "pro", "entity": "SPECT" }, { "start_idx": 76, "end_idx": 78, "type": "pro", "entity": "PET" }, { "start_idx": 81, "end_idx": 85, "type": "pro", "entity": "实验室检查" }, { "start_idx": 87, "end_idx": 92, "type": "pro", "entity": "神经心理测验" }, { "start_idx": 111, "end_idx": 114, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "头颅CT或MRI等检查对抽动障碍的价值不在于诊断,而在于排除其他脑器质性病变。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "头颅CT" }, { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "MRI" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "至于功能性磁共振成像(fMRI)、单光子发射计算机断层扫描(SPECT)、正电子发射扫描(PET)和经颅磁刺激(transcranialmagneticstimulation)等检查,能够用于抽动障碍脑功能研究。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "功能性磁共振成像" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "fMRI" }, { "start_idx": 17, "end_idx": 28, "type": "pro", "entity": "单光子发射计算机断层扫描" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "SPECT" }, { "start_idx": 37, "end_idx": 43, "type": "pro", "entity": "正电子发射扫描" }, { "start_idx": 45, "end_idx": 47, "type": "pro", "entity": "PET" }, { "start_idx": 50, "end_idx": 54, "type": "pro", "entity": "经颅磁刺激" } ] }, { "text": "在抽动障碍诊断方面,主要依据病人的临床表现(病史和临床症状)来进行诊断,国内外学者均采用临床描述性诊断方法来对抽动障碍进行诊断,并且必须排除风湿性舞蹈病、肝豆状核变性、癫痫肌阵挛性发作、药源性不自主抽动及其他锥体外系疾病。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 70, "end_idx": 75, "type": "dis", "entity": "风湿性舞蹈病" }, { "start_idx": 77, "end_idx": 82, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 84, "end_idx": 91, "type": "dis", "entity": "癫痫肌阵挛性发作" }, { "start_idx": 93, "end_idx": 100, "type": "dis", "entity": "药源性不自主抽动" } ] }, { "text": "抽动障碍的诊断通常被延误多年,多数病人在症状出现几年以后才被诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "将喉肌抽动而致的干咳误诊为慢性咽炎、气管炎;将眨眼、皱眉诊为眼结膜炎;动鼻诊为慢性鼻炎等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "喉肌抽动" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "慢性咽炎" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "气管炎" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "结膜炎" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "慢性鼻炎" } ] }, { "text": "(二)诊断标准1.短暂性抽动障碍(1)国际疾病分类第10版(ICD-10)关于暂时性抽动障碍的诊断标准:1)起病于儿童或青少年早期,以4~5岁儿童最常见。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "暂时性抽动障碍" } ] }, { "text": "2)有复发性、不自主、重复、快速、无目的的单一或多部位运动抽动,或发声抽动,以眨眼、扮鬼脸或头部抽动较常见。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "运动抽动" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "发声抽动" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "眨眼" }, { "start_idx": 42, "end_idx": 44, "type": "sym", "entity": "扮鬼脸" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "头部抽动" } ] }, { "text": "3)抽动能受意志克制短暂时间(数分钟至数小时),入睡后消失,检查未能发现神经系统障碍。", "entities": [ { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "神经系统障碍" } ] }, { "text": "4)抽动一天出现多次,几乎天天如此,至少持续2周,但病程不超过1年。", "entities": [ { "start_idx": 2, "end_idx": 32, "type": "sym", "entity": "抽动一天出现多次,几乎天天如此,至少持续2周,但病程不超过1年" } ] }, { "text": "5)排除风湿性舞蹈病、肝豆状核变性、癫痫肌阵挛发作、药源性不自主抽动和其他锥体外系病变。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "风湿性舞蹈病" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "癫痫肌阵挛发作" }, { "start_idx": 26, "end_idx": 33, "type": "dis", "entity": "药源性不自主抽动" } ] }, { "text": "(2)《美国精神疾病诊断与统计手册》第4版修订本(DSM-Ⅳ-TR)关于暂时性抽动障碍的诊断标准:1)一种或多种运动和或发声抽动。", "entities": [ { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "2)抽动一天发作多次,几乎每天发作持续时间至少4周,但不超过1年。", "entities": [ { "start_idx": 2, "end_idx": 31, "type": "sym", "entity": "抽动一天发作多次,几乎每天发作持续时间至少4周,但不超过1年" } ] }, { "text": "3)既往无慢性抽动障碍或TS病史。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "TS" } ] }, { "text": "5)抽动障碍症状不是直接由某些药物(如兴奋剂)或内科疾病(如亨廷顿舞蹈病或病毒感染后脑炎)所致。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "兴奋剂" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "亨廷顿舞蹈病" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "病毒感染后脑炎" } ] }, { "text": "(3)《中国精神障碍与诊断标准》第3版(CCMD-3)关于短暂性抽动障碍的诊断标准:1)有单个或多个运动性抽动或发声抽动,常表现为眨眼、扮鬼脸或头部抽动等简单抽动。", "entities": [ { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "眨眼" }, { "start_idx": 68, "end_idx": 70, "type": "sym", "entity": "扮鬼脸" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "头部抽动" } ] }, { "text": "4)不是由于TS、小舞蹈病、药物或神经系统其他疾病所致。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "TS" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "小舞蹈病" } ] }, { "text": "2.慢性抽动障碍(1)ICD-10关于慢性抽动障碍的诊断标准:1)有反复性、不自主、重复、快速、无目的的抽动,任何一次抽动不超过三组肌肉。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 34, "end_idx": 53, "type": "sym", "entity": "反复性、不自主、重复、快速、无目的的抽动" } ] }, { "text": "7)排除慢性锥体外系病变、癫痫肌阵挛发作、面肌痉挛和精神病装相等。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "慢性锥体外系病变" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "癫痫肌阵挛发作" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "面肌痉挛" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "精神病" } ] }, { "text": "(2)DSM-Ⅳ-TR关于慢性抽动障碍的诊断标准:1)一种或多种运动或发声抽动,但在病程中不同时出现。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "慢性抽动障碍" } ] }, { "text": "4)抽动障碍症状不是由某些药物(如兴奋剂)或内科疾病(如亨廷顿舞蹈病或病毒感染后脑炎)所致。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "兴奋剂" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "亨廷顿舞蹈病" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "病毒感染后脑炎" } ] }, { "text": "(3)CCMD-3关于慢性抽动障碍的诊断标准:1)不自主运动抽动或发声,可以不同时存在,常1天发生多次,可每天或间断出现。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "慢性抽动障碍" } ] }, { "text": "4)不是由于TS、小舞蹈病、药物或神经系统其他疾病所致。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "TS" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "小舞蹈病" } ] }, { "text": "3.TS通常推荐TS“必须诊断标准(essentialdiagnosticcriteria)”,包括:21岁以前发病,多发性不自主的运动抽动,一种或多种发声抽动,有一加重或减轻的病程,新的症状逐渐代替旧的症状,抽动缺乏其他医学解释和病程超过1年。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "TS" } ] }, { "text": "发声抽动对TS的诊断是必须的,但需要强调的是,本病的诊断标准中秽语不是必须的,因为秽语只发生在不到13病例中。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "TS" } ] }, { "text": "(1)ICD-10关于TS的诊断标准:1)起病于21岁以前,大多数在2~15岁之间。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "TS" } ] }, { "text": "2)有复发性、不自主、重复的、快速的、无目的的抽动,影响多组肌肉。", "entities": [ { "start_idx": 3, "end_idx": 31, "type": "sym", "entity": "复发性、不自主、重复的、快速的、无目的的抽动,影响多组肌肉" } ] }, { "text": "3)多种运动抽动和一种或多种发声抽动同时出现于某些时候,但不一定必须同时存在。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "多种运动抽动" } ] }, { "text": "6)抽动一天发作多次,几乎天天如此;病程超过1年以上,且在同一年之中症状缓解不超过2个月以上。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "抽动一天发作多次" } ] }, { "text": "7)排除风湿性舞蹈病、肝豆状核变性、癫痫肌阵挛发作、药源性不自主抽动和其他锥体外系病变。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "风湿性舞蹈病" }, { "start_idx": 11, "end_idx": 16, "type": "dru", "entity": "肝豆状核变性" }, { "start_idx": 18, "end_idx": 24, "type": "dru", "entity": "癫痫肌阵挛发作" }, { "start_idx": 26, "end_idx": 33, "type": "dru", "entity": "药源性不自主抽动" }, { "start_idx": 37, "end_idx": 42, "type": "dru", "entity": "锥体外系病变" } ] }, { "text": "(2)DSM-Ⅳ-TR关于TS的诊断标准:1)在病程中具有多种运动抽动及一种或多种发声抽动,而不必在同一时间出现。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dru", "entity": "TS" } ] }, { "text": "2)抽动可每天发作多次(通常为丛集性)或间歇发作,但间歇时间不超过3个月,抽动病程在1年以上。", "entities": [ { "start_idx": 2, "end_idx": 23, "type": "sym", "entity": "抽动可每天发作多次(通常为丛集性)或间歇发作" } ] }, { "text": "5)抽动障碍症状不是直接由某些药物(如兴奋剂)或内科疾病(如亨廷顿舞蹈病或病毒感染后脑炎)所致。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "抽动障碍" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "兴奋剂" }, { "start_idx": 24, "end_idx": 25, "type": "dep", "entity": "内科" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "亨廷顿舞蹈病" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "病毒感染后脑炎" } ] }, { "text": "(3)CCMD-3关于TS的诊断标准:TS是以进行性发展的多部位运动和发声抽动为特征的抽动障碍,部分患儿伴有模仿言语、模仿动作,或强迫、攻击、情绪障碍,及注意缺陷等行为障碍,起病于童年。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "TS" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "TS" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "模仿言语" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "模仿动作" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "强迫" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "攻击" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "情绪障碍" } ] }, { "text": "1)症状标准:表现为多种运动抽动和一种或多种发声抽动,多为复杂性抽动,二者多同时出现。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "多种运动抽动" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "一种或多种发声抽动" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "复杂性抽动" } ] }, { "text": "此外,难治性TS是近些年来小儿神经精神科临床逐渐形成的新概念,用于描述经过常规药物(氟哌啶醇及硫必利等)治疗效果不好,病程迁延不愈的TS病人。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "TS" }, { "start_idx": 42, "end_idx": 45, "type": "dru", "entity": "氟哌啶醇" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "硫必利" } ] }, { "text": "难治性Tourette综合征(intractableTourettesyndrome)的诊断标准:①符合ICD-10中TS的诊断标准,排除风湿性舞蹈病、肝豆状核变性、习惯性痉挛和癫痫;②YGTSS得分≥50;③经氟哌啶醇或和硫必利足量治疗1年以上无效。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "Tourette综合征" }, { "start_idx": 15, "end_idx": 41, "type": "dis", "entity": "intractableTourettesyndrome" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "TS" }, { "start_idx": 69, "end_idx": 74, "type": "dis", "entity": "风湿性舞蹈病" }, { "start_idx": 76, "end_idx": 81, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 83, "end_idx": 87, "type": "dis", "entity": "习惯性痉挛" }, { "start_idx": 89, "end_idx": 90, "type": "dis", "entity": "癫痫" }, { "start_idx": 106, "end_idx": 109, "type": "dru", "entity": "氟哌啶醇" }, { "start_idx": 112, "end_idx": 114, "type": "dru", "entity": "硫必利" } ] }, { "text": "三、新生儿免疫性血小板减少性紫癜在孕期中母亲的一部分抗体可通过胎盘进入胎儿体内,如果这部分抗体可引起血小板减少,则新生儿可出现血小板减少性紫癜。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "dis", "entity": "新生儿免疫性血小板减少性紫癜" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "抗体" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "血小板" }, { "start_idx": 63, "end_idx": 70, "type": "dis", "entity": "血小板减少性紫癜" } ] }, { "text": "广义的新生儿免疫性血小板减少性紫癜主要包括药物、新生儿ITP(NITP)、新生儿同种免疫性血小板减少性紫癜(neonatalalloimmunethrombocytopenicpurpura,NAIT)。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "dis", "entity": "新生儿免疫性血小板减少性紫癜" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "新生儿ITP" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "NITP" }, { "start_idx": 37, "end_idx": 52, "type": "dis", "entity": "新生儿同种免疫性血小板减少性紫癜" }, { "start_idx": 54, "end_idx": 94, "type": "dis", "entity": "neonatalalloimmunethrombocytopenicpurpura" }, { "start_idx": 96, "end_idx": 99, "type": "dis", "entity": "NAIT" } ] }, { "text": "1.NITP患有ITP孕母的抗血小板抗体可通过胎盘进入胎儿体内,破坏胎儿的血小板。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "NITP" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "ITP" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "抗血小板抗体" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "胎盘" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "血小板" } ] }, { "text": "不论患ITP孕母在孕期或分娩时有否血小板减少,约50%的新生儿可能出现血小板减少。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "ITP" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "血小板" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "血小板" } ] }, { "text": "在产前首先要分析孕妇状况对胎儿的影响,如孕母有否血小板降低及其减低程度,是否已切脾,是否分娩过血小板减少的新生儿。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "血小板" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "血小板" } ] }, { "text": "如果怀疑胎儿有血小板降低,有条件者可作经皮脐静脉采样测血小板数,当胎儿血小板<50×109/L时,母亲可试用静脉丙种球蛋白1g/(kg•d);如孕期已有足够长,必要时亦可进行剖宫产。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "血小板" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "皮脐静脉采样" }, { "start_idx": 27, "end_idx": 30, "type": "ite", "entity": "血小板数" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "血小板" } ] }, { "text": "产后的NITP处理原则基本同ITP,需要同新生儿科医师合作进行。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "NITP" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "ITP" } ] }, { "text": "2.NAIT发病机制与新生儿溶血相似,胎儿由父亲遗传获得不同于母亲的血小板抗原,通过胎盘进入母体刺激产生相应IgG抗体,该抗体在通过胎盘进入胎儿血循环并破坏其血小板。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "NAIT" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "抗体" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "胎盘" } ] }, { "text": "产后可于数分钟或数小时内发病,症状同血小板减少性紫癜,但常较重;颅内出血可发生于产前或产后,是常见的死因。", "entities": [ { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "血小板减少性紫癜" } ] }, { "text": "临床上如果母亲无ITP却娩出先天性血小板减少的新生儿,患儿巨核细胞数增加,此时应考虑NAIT。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "ITP" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "血小板" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "巨核细胞" } ] }, { "text": "确诊的条件需要鉴定母亲的血小板缺乏同种特异性抗原,血清中存在同种血小板抗体。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "血小板" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血清" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "血小板抗体" } ] }, { "text": "病情不重时可给肾上腺皮质激素、丙种球蛋白静滴,症状重时可输注血小板浓缩制剂,输入母亲的血小板为最安全有效。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "丙种球蛋白" }, { "start_idx": 30, "end_idx": 36, "type": "dru", "entity": "血小板浓缩制剂" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "血小板" } ] }, { "text": "第二节发育特点儿童发育除了具备体格生长的三个特点之外,还具有下述三个特点:(一)发育是成熟的过程儿童在生长的同时,也在不断发育,随着神经系统的成长和功能分化,儿童的行为也逐渐发生改变。", "entities": [ { "start_idx": 66, "end_idx": 69, "type": "bod", "entity": "神经系统" } ] }, { "text": "儿童认知发育(cognitivedevelopment)从手和眼的感觉运动到具体运算,最终达到逻辑运算这样一个过程。", "entities": [ { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "手" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "眼" } ] }, { "text": "二、吸入NO的临床应用(一)用于临床治疗1.医用NO气体浓度[NO]一般为1000±50×10-6(ppm);[NO2]<10ppm;容器为铝合金钢瓶+不锈钢减压阀,压力5~10mPa(50~100大气压)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "吸入NO" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "医用NO" } ] }, { "text": "2.用于NO/NO2浓度监测仪的定标气体[NO]为20~80ppm;[NO2]<1ppm,一般每周校正,避免由于监测仪工作状态漂移导致的吸入NO浓度过高。", "entities": [ { "start_idx": 4, "end_idx": 25, "type": "equ", "entity": "NO/NO2浓度监测仪" }, { "start_idx": 78, "end_idx": 80, "type": "equ", "entity": "监测仪" }, { "start_idx": 90, "end_idx": 93, "type": "pro", "entity": "吸入NO" } ] }, { "text": "3.适应证新生儿低氧性呼吸衰竭和持续肺动脉高压续肺动脉高压,潜在适应证为儿童复杂先天性心脏病合并肺动脉高压,儿童和成人急性肺损伤(ALI)和急性呼吸窘迫综合征(ARDS)。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "低氧性呼吸衰竭" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "持续肺动脉高压" }, { "start_idx": 36, "end_idx": 45, "type": "dis", "entity": "儿童复杂先天性心脏病" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "急性肺损伤" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "ALI" }, { "start_idx": 70, "end_idx": 78, "type": "dis", "entity": "急性呼吸窘迫综合征" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "ARDS" } ] }, { "text": "4.临床治疗研究标准①低氧血症性呼吸衰竭:呼吸机正压通气下,FiO2>0.6,SpO2<80%。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "低氧血症性呼吸衰竭" }, { "start_idx": 21, "end_idx": 23, "type": "equ", "entity": "呼吸机" } ] }, { "text": "肺动脉高压:彩超,导管或临床诊断,以出现动脉导管、卵圆孔的右向左分流、三尖瓣反流等为依据。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "彩超" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "导管" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "临床诊断" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "出现动脉导管" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "卵圆孔的右向左分流" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "三尖瓣反流" } ] }, { "text": "③疗效判断:FiO2下降>0.3,SpO2>85%,PaO2>50mmHg,PAP/SAP<0.7。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "ite", "entity": "FiO2" }, { "start_idx": 17, "end_idx": 31, "type": "ite", "entity": "SpO2" }, { "start_idx": 37, "end_idx": 51, "type": "ite", "entity": "PaO2" }, { "start_idx": 60, "end_idx": 62, "type": "ite", "entity": "PAP" }, { "start_idx": 64, "end_idx": 66, "type": "ite", "entity": "SAP" } ] }, { "text": "(二)临床研究的结果对于足月和近足月新生儿(>2500g、>35周)研究的文献中,确定吸入NO可以迅速提高血氧,改善低氧血症,显著减少对ECMO的依赖,并可以减少在NICU治疗的总费用。", "entities": [ { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "吸入NO" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "血氧" }, { "start_idx": 58, "end_idx": 61, "type": "dis", "entity": "低氧血症" }, { "start_idx": 68, "end_idx": 71, "type": "equ", "entity": "ECMO" }, { "start_idx": 82, "end_idx": 87, "type": "pro", "entity": "NICU治疗" } ] }, { "text": "对于早产儿(500~2500g、<35周)给予5ppm吸入NO治疗1~7天,可以迅速提高血氧,但不能显著减少病死率。", "entities": [ { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "吸入NO治疗" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "血氧" } ] }, { "text": "其死亡主要为极低出生体重和脏器发育不成熟。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "极低出生体重" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "脏器发育不成熟" } ] }, { "text": "吸入NO不加重颅内出血,但可以减轻发生慢性肺病的可能。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "吸入NO" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "颅内出" }, { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "颅内出" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "减轻发生" } ] }, { "text": "1.新生儿持续缺氧性呼吸衰竭伴肺动脉高压症(PPHN)多中心临床对照试验证实,吸入NO对于足月和近足月新生儿PPHN和危重缺氧性呼吸衰竭有效,主要效果表现为迅速降低肺动脉压、改善肺血流,减少对体外膜肺治疗的依赖,并弥补了机械通气和肺表面活性物质治疗效果不能持久的缺点,也反映出对近足月和足月新生儿低氧性呼吸衰竭采用肺表面活性物质应慎重考虑。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "持续缺氧性呼吸衰竭" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "肺动脉高压症" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "PPHN" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "吸入NO" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "PPHN" }, { "start_idx": 59, "end_idx": 67, "type": "dis", "entity": "危重缺氧性呼吸衰竭" }, { "start_idx": 82, "end_idx": 85, "type": "ite", "entity": "肺动脉压" }, { "start_idx": 78, "end_idx": 85, "type": "sym", "entity": "迅速降低肺动脉压" }, { "start_idx": 89, "end_idx": 89, "type": "bod", "entity": "肺" }, { "start_idx": 87, "end_idx": 91, "type": "sym", "entity": "改善肺血流" }, { "start_idx": 96, "end_idx": 101, "type": "pro", "entity": "体外膜肺治疗" }, { "start_idx": 110, "end_idx": 113, "type": "pro", "entity": "机械通气" }, { "start_idx": 115, "end_idx": 123, "type": "pro", "entity": "肺表面活性物质治疗" }, { "start_idx": 155, "end_idx": 163, "type": "pro", "entity": "采用肺表面活性物质" } ] }, { "text": "2.早产儿RDS可以在应用肺表面活性物质无效并存在肺动脉高压时使用,但必须密切观察是否出现颅内出血症状。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "RDS" }, { "start_idx": 11, "end_idx": 19, "type": "pro", "entity": "应用肺表面活性物质" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "颅内" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "颅内出血症状" } ] }, { "text": "目前是否可以对有指征的早产儿常规应用吸入NO有待研究。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "吸入NO" } ] }, { "text": "近年研究发现,新生儿出生后24小时呼出气NO浓度在3×10-6~5×10-6ppm,1周后下降,主要来自于鼻窦。", "entities": [ { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "鼻窦" } ] }, { "text": "提示出生早期自身NO对于调节肺血管张力起过渡性生理代偿作用,出生后立即气道插管机械通气的新生儿可能存在自身吸入和利用NO的障碍,可考虑将吸入NO作为替代治疗。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肺血管" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "气道插管" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "机械通气" }, { "start_idx": 51, "end_idx": 62, "type": "sym", "entity": "自身吸入和利用NO的障碍" }, { "start_idx": 68, "end_idx": 71, "type": "pro", "entity": "吸入NO" } ] }, { "text": "3.支气管肺发育不良(BPD)对于生后1~2周持续依赖机械通气和高浓度氧的新生儿,持续吸入低浓度NO对预防BPD发生有一定作用。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "支气管肺发育不良" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "BPD" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "机械通气" }, { "start_idx": 41, "end_idx": 49, "type": "pro", "entity": "持续吸入低浓度NO" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "BPD" } ] }, { "text": "有试验对预计长期呼吸机治疗小儿给予预防性吸入NO,但是已有的报道疗效不一。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "呼吸机治疗" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "预防性吸入NO" } ] }, { "text": "4.治疗方案和疗效观察目前治疗原发和继发的PPHN,起始NO浓度一般在10×10-6~20×10-6(10~20ppm),长时间吸入则降到5×10-6~10×10-6,甚至1×10-6~3×10-6。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "PPHN" }, { "start_idx": 86, "end_idx": 87, "type": "pro", "entity": "吸入" } ] }, { "text": "临床判断是否对吸入NO有反应,包括多普勒彩超确定肺外分流的改善、肺动脉压的下降,经皮氧饱和度和动脉氧分压的上升,血气参数的改善,可以将吸入氧浓度和呼吸机参数的下调等,作为临床治疗有效的依据。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "吸入NO" }, { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "多普勒彩超" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "肺" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "肺外分流的改善" }, { "start_idx": 32, "end_idx": 35, "type": "ite", "entity": "肺动脉压" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "肺动脉压的下降" }, { "start_idx": 41, "end_idx": 45, "type": "ite", "entity": "皮氧饱和度" }, { "start_idx": 47, "end_idx": 51, "type": "ite", "entity": "动脉氧分压" }, { "start_idx": 40, "end_idx": 54, "type": "sym", "entity": "经皮氧饱和度和动脉氧分压的上升" }, { "start_idx": 56, "end_idx": 59, "type": "ite", "entity": "血气参数" }, { "start_idx": 56, "end_idx": 62, "type": "sym", "entity": "血气参数的改善" }, { "start_idx": 67, "end_idx": 69, "type": "pro", "entity": "吸入氧" }, { "start_idx": 73, "end_idx": 75, "type": "equ", "entity": "呼吸机" } ] }, { "text": "可能原因为:①肺泡扩张不够,可以用CPAP、高频通气治疗,或应用气道滴入肺表面活性物质,使肺泡复张;②高铁血红蛋白血症,一般MetHb>3%可以有明显症状,可以降低吸入NO,如效果不佳,可以用维生素C、亚甲蓝(methyleneblue)或输血,以纠正;③肺血管器质性病变,对NO没有反应。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肺泡" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "肺泡扩张不够" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "用CPAP" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "高频通气治疗" }, { "start_idx": 32, "end_idx": 42, "type": "pro", "entity": "气道滴入肺表面活性物质" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "肺泡" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "肺泡复张" }, { "start_idx": 51, "end_idx": 58, "type": "dis", "entity": "高铁血红蛋白血症" }, { "start_idx": 82, "end_idx": 85, "type": "pro", "entity": "吸入NO" }, { "start_idx": 96, "end_idx": 99, "type": "dru", "entity": "维生素C" }, { "start_idx": 101, "end_idx": 103, "type": "dru", "entity": "亚甲蓝" }, { "start_idx": 105, "end_idx": 117, "type": "dru", "entity": "methyleneblue" }, { "start_idx": 120, "end_idx": 121, "type": "pro", "entity": "输血" }, { "start_idx": 128, "end_idx": 130, "type": "bod", "entity": "肺血管" }, { "start_idx": 128, "end_idx": 135, "type": "sym", "entity": "肺血管器质性病变" } ] }, { "text": "2.NO依赖在治疗中不能将NO浓度降低,或停止NO后立即出现低氧血症危象。", "entities": [ { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "低氧血症" } ] }, { "text": "目前由于大多数采用低浓度NO吸入(<10ppm),因此在3~7天内撤除NO一般没有困难。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "低浓度NO吸入" } ] }, { "text": "撤除后可以短时间适当提高FiO20.1~0.2,防止低氧血症。", "entities": [ { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "低氧血症" } ] }, { "text": "呼吸机参数不必大调。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "呼吸机" } ] }, { "text": "如果出现低氧血症和肺动脉高压危象,可以再将NO接入。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "低氧血症" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "肺动脉高压" } ] }, { "text": "第七节肺的局部免疫和防御机制呼吸道的主要功能除了调节气道阻力外,还有保护作用,主要为加温湿化作用和过滤清洁作用。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "呼吸道" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "气道" } ] }, { "text": "(一)气道的黏膜屏障1.黏膜屏障功能气管和支气管内壁黏膜层为假复层纤毛柱状上皮细胞,主要由纤毛上皮细胞、杯状细胞,基底细胞、神经内分泌细胞等组成,覆盖大、中、小支气管。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "气道" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "黏膜" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "黏膜" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "气管" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "支气管内壁黏膜" }, { "start_idx": 30, "end_idx": 40, "type": "bod", "entity": "假复层纤毛柱状上皮细胞" }, { "start_idx": 45, "end_idx": 50, "type": "bod", "entity": "纤毛上皮细胞" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "杯状细胞" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "基底细胞" }, { "start_idx": 62, "end_idx": 68, "type": "bod", "entity": "神经内分泌细胞" }, { "start_idx": 75, "end_idx": 82, "type": "bod", "entity": "大、中、小支气管" } ] }, { "text": "黏膜下层分布有浆液腺、黏液腺等结构。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "黏膜" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "浆液腺" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "黏液腺" } ] }, { "text": "随支气管逐级分支,柱状上皮和杯状细胞逐渐减少,至细支气管时,由假复层纤毛柱状上皮过渡为单层柱状上皮细胞和Clara细胞为主。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "支气管" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "柱状上皮" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "杯状细胞" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "细支气管" }, { "start_idx": 31, "end_idx": 39, "type": "bod", "entity": "假复层纤毛柱状上皮" }, { "start_idx": 43, "end_idx": 50, "type": "bod", "entity": "单层柱状上皮细胞" }, { "start_idx": 52, "end_idx": 58, "type": "bod", "entity": "Clara细胞" } ] }, { "text": "气道上皮细胞间、细胞和基底膜的连接依赖紧密连接、桥粒、半桥粒、中间连接等结构,起到黏附连接作用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "气道上皮细胞" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "细胞" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "基底膜" } ] }, { "text": "这些细胞的增殖、分化及功能发挥与细胞黏附分子和细胞外基质相关,这些黏附分子包括整合素(integrin)、细胞外基质蛋白(tenascin)、选择素(selectin)、细胞间黏附分子(intercellularadhesionmolecules,ICAMs)、纤维连接蛋白(fibronectin)、钙依赖黏附分子(cadherin)等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "细胞" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "细胞黏附分子" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "细胞外基质" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "整合素" }, { "start_idx": 43, "end_idx": 50, "type": "bod", "entity": "integrin" }, { "start_idx": 53, "end_idx": 59, "type": "bod", "entity": "细胞外基质蛋白" }, { "start_idx": 61, "end_idx": 68, "type": "bod", "entity": "tenascin" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "选择素" }, { "start_idx": 75, "end_idx": 82, "type": "bod", "entity": "selectin" }, { "start_idx": 85, "end_idx": 91, "type": "bod", "entity": "细胞间黏附分子" }, { "start_idx": 93, "end_idx": 122, "type": "bod", "entity": "intercellularadhesionmolecules" }, { "start_idx": 124, "end_idx": 128, "type": "bod", "entity": "ICAMs" }, { "start_idx": 131, "end_idx": 136, "type": "bod", "entity": "纤维连接蛋白" }, { "start_idx": 138, "end_idx": 148, "type": "bod", "entity": "fibronectin" }, { "start_idx": 151, "end_idx": 157, "type": "bod", "entity": "钙依赖黏附分子" }, { "start_idx": 159, "end_idx": 166, "type": "bod", "entity": "cadherin" } ] }, { "text": "细胞外基质(extracellularmetrix,ECM)包括胶原蛋白(collagen)、弹力蛋白(elastin)、蛋白多糖(proteoglycan)类如透明质酸、硫酸软骨素、肝素等,以及ECM糖蛋白如基膜粘连蛋白(laminin)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "细胞外基质" }, { "start_idx": 6, "end_idx": 24, "type": "bod", "entity": "extracellularmetrix" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "ECM" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "胶原蛋白" }, { "start_idx": 37, "end_idx": 44, "type": "bod", "entity": "collagen" }, { "start_idx": 52, "end_idx": 58, "type": "bod", "entity": "elastin" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "蛋白多糖" }, { "start_idx": 66, "end_idx": 77, "type": "bod", "entity": "proteoglycan" }, { "start_idx": 81, "end_idx": 84, "type": "bod", "entity": "透明质酸" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "硫酸软骨素" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "肝素" }, { "start_idx": 98, "end_idx": 103, "type": "bod", "entity": "ECM糖蛋白" }, { "start_idx": 105, "end_idx": 110, "type": "bod", "entity": "基膜粘连蛋白" }, { "start_idx": 112, "end_idx": 118, "type": "bod", "entity": "laminin" } ] }, { "text": "这些蛋白影响细胞间黏附、迁移、分化,参与细胞骨架构建及形态形成,在肺发育、损伤、修复中起重要的控制与调节作用。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "蛋白" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "细胞" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "细胞" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "肺" } ] }, { "text": "上皮细胞以及细胞下基质作为连续性界面,通过生物物理作用而成为生理屏障。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "细胞下基质" } ] }, { "text": "肺泡的黏膜屏障作用则主要依靠肺泡上皮细胞。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺泡" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "黏膜" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "肺泡上皮细胞" } ] }, { "text": "2.黏液-纤毛保护机制气道上皮细胞表面有大量黏液分泌,与上皮细胞端面的纤毛形成黏液-纤毛保护机制,并通过咳嗽作用,形成渐进式向上清除活动,可以将每日吸入的大量灰尘、颗粒、气雾、病原体清除出体外,或经咽喉部吞咽而清除。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "气道上皮细胞" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "咳嗽" }, { "start_idx": 88, "end_idx": 90, "type": "mic", "entity": "病原体" }, { "start_idx": 99, "end_idx": 101, "type": "bod", "entity": "咽喉部" } ] }, { "text": "纤毛结构发育上的异常、病理性损伤、呼吸管理不善导致纤毛结构和功能障碍,会影响黏液-纤毛保护功能。", "entities": [ { "start_idx": 25, "end_idx": 33, "type": "dis", "entity": "纤毛结构和功能障碍" } ] }, { "text": "3.气道上皮分泌抗微生物活性物质气道上皮具有分泌抗微生物活性物质,溶菌酶、分泌型免疫球蛋白A,肺表面活性物质蛋白A、D、乳铁蛋白等,通过溶菌、中和、调理等作用,直接或间接抑制病原体侵袭,对气道上皮起保护作用。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "气道" }, { "start_idx": 8, "end_idx": 15, "type": "bod", "entity": "抗微生物活性物质" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "气道" }, { "start_idx": 24, "end_idx": 31, "type": "bod", "entity": "抗微生物活性物质" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "溶菌酶" }, { "start_idx": 37, "end_idx": 45, "type": "bod", "entity": "分泌型免疫球蛋白A" }, { "start_idx": 47, "end_idx": 63, "type": "bod", "entity": "肺表面活性物质蛋白A、D、乳铁蛋白" }, { "start_idx": 87, "end_idx": 89, "type": "mic", "entity": "病原体" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "气道" } ] }, { "text": "4.抗过氧化物质气道上皮具有抗氧化活性物质,可以对抗氧自由基的细胞毒副作用,维持上皮细胞结构功能的完整。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "抗过氧化物质" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "气道" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "抗氧化活性物质" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "抗氧自由基" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "上皮细胞" } ] }, { "text": "如谷胱甘肽氧化还原系统中的还原型谷胱甘肽,是上皮细胞重要的细胞抗氧化物质,帮助过氧化氢、脂过氧化物等还原;超氧化物歧化酶(SOD)使超氧自由基转变为过氧化氢;过氧化氢酶使过氧化氢还原为水。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "bod", "entity": "谷胱甘肽氧化还原系统" }, { "start_idx": 13, "end_idx": 19, "type": "bod", "entity": "还原型谷胱甘肽" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "细胞抗氧化物质" }, { "start_idx": 53, "end_idx": 59, "type": "bod", "entity": "超氧化物歧化酶" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "SOD" }, { "start_idx": 79, "end_idx": 83, "type": "bod", "entity": "过氧化氢酶" } ] }, { "text": "5.抗蛋白酶肺内抗蛋白酶如α1-抗胰蛋白酶、α1-抗糜蛋白酶均为特异性的肺内蛋白酶抑制物。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "抗蛋白酶" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "肺内抗蛋白酶" }, { "start_idx": 13, "end_idx": 20, "type": "bod", "entity": "α1-抗胰蛋白酶" }, { "start_idx": 22, "end_idx": 29, "type": "bod", "entity": "α1-抗糜蛋白酶" }, { "start_idx": 36, "end_idx": 43, "type": "bod", "entity": "肺内蛋白酶抑制物" } ] }, { "text": "如果抗蛋白酶和蛋白酶之间的平衡被破坏,则带来组织细胞的破坏。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "抗蛋白酶" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "蛋白酶" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "组织细胞" } ] }, { "text": "(二)特异性局部免疫分泌型IgA(sIgA)由气道黏膜下层浆细胞合成IgA和J链,然后经气道上皮细胞合成的分泌片段结合后而形成。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "分泌型IgA" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "sIgA" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "气道黏膜" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "浆细胞" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "气道上皮细胞" } ] }, { "text": "在气道上皮表面发挥抑制外来抗原和一些细菌、病毒的作用。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "气道" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "抗原" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "细菌" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "病毒" } ] }, { "text": "新生儿和婴幼儿肺sIgA发育不完善,因此容易发生小气道的细菌或病毒感染。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "肺sIgA" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "小气道" }, { "start_idx": 28, "end_idx": 29, "type": "mic", "entity": "细菌" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "病毒" } ] }, { "text": "(三)肺泡巨噬细胞来源为骨髓幼单核细胞,经血流到达肺部后再发育成熟、转化而形成。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "肺泡巨噬细胞" }, { "start_idx": 12, "end_idx": 18, "type": "bod", "entity": "骨髓幼单核细胞" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肺部" } ] }, { "text": "直径约20~40μm,形态不规则,有伪足样突起,胞质含大量溶酶体、吞噬体。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "溶酶体" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "吞噬体" } ] }, { "text": "肺泡巨噬细胞吞噬细菌、尘粒和衰老死亡细胞,参与对肺表面活性物质磷脂重摄取代谢循环,并具有抗肿瘤作用,成为机体重要的非特异性防御功能的一道防线。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "肺泡巨噬细胞" }, { "start_idx": 8, "end_idx": 9, "type": "mic", "entity": "细菌" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "衰老死亡细胞" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "磷脂" } ] }, { "text": "第二十章进行性脊髓性肌萎缩症进行性脊髓性肌萎缩症(progressivespinalmuscularatrophy,SMA)是一类由脊髓前角运动神经元和脑干运动神经核变性导致肌无力及肌萎缩的疾病。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "dis", "entity": "进行性脊髓性肌萎缩症" }, { "start_idx": 14, "end_idx": 23, "type": "dis", "entity": "进行性脊髓性肌萎缩症" }, { "start_idx": 25, "end_idx": 56, "type": "dis", "entity": "progressivespinalmuscularatrophy" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "SMA" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "脊髓" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "运动神经元" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "脑干" }, { "start_idx": 78, "end_idx": 82, "type": "bod", "entity": "运动神经核" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "肌无力" }, { "start_idx": 91, "end_idx": 93, "type": "dis", "entity": "肌萎缩" } ] }, { "text": "SMA为常染色体隐性遗传病,根据发病年龄和肌无力严重程度临床分为3型。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "SMA" }, { "start_idx": 4, "end_idx": 12, "type": "dis", "entity": "常染色体隐性遗传病" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "肌无力" } ] }, { "text": "【流行病学】本病是一较常见的常染色体隐性遗传性疾病,发生率约为1/10万,基因携带率为1/50。", "entities": [ { "start_idx": 14, "end_idx": 24, "type": "dis", "entity": "常染色体隐性遗传性疾病" } ] }, { "text": "【发病机制】1990年Gillian等报道SMA基因位点在染色体5q11.2-2.3。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "SMA基因" }, { "start_idx": 29, "end_idx": 41, "type": "bod", "entity": "染色体5q11.2-2.3" } ] }, { "text": "1994年Meli等发现严重型SMA(Werdnig-Hoffmann型)患者在5q11.2-2.3发生较大的基因突变,而轻型患者(Kugelberg-Welander型)则无基因突变或突变较轻。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "严重型SMA" }, { "start_idx": 19, "end_idx": 35, "type": "dis", "entity": "Werdnig-Hoffmann型" }, { "start_idx": 40, "end_idx": 49, "type": "bod", "entity": "5q11.2-2.3" }, { "start_idx": 66, "end_idx": 84, "type": "dis", "entity": "Kugelberg-Welander型" } ] }, { "text": "目前发现的与SMA相关的基因有2种,即神经元凋亡抑制蛋白(neuronalapoptosisinhibitoryprotein,NAIP)基因和运动神经元(survivalmotoneuron,SMN)存活基因。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "SMA" }, { "start_idx": 19, "end_idx": 27, "type": "bod", "entity": "神经元凋亡抑制蛋白" }, { "start_idx": 29, "end_idx": 62, "type": "bod", "entity": "neuronalapoptosisinhibitoryprotein" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "NAIP" }, { "start_idx": 72, "end_idx": 76, "type": "bod", "entity": "运动神经元" }, { "start_idx": 78, "end_idx": 95, "type": "bod", "entity": "survivalmotoneuron" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "SMN" } ] }, { "text": "NAIP基因定位于5q13区,67%的SMA患者发生此基因突变,相比之下正常人群中突变率仅2%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "NAIP基因" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "5q13区" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "SMA" } ] }, { "text": "SMN基因也定位于5q13区,约98%以上的SMA患者发生此基因突变。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "SMN基因" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "5q13区" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "SMA" } ] }, { "text": "5q13连锁的SMA患者中,96.4%显示SMN1外显子7和8或者外显子7出现纯合缺失。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "5q13连锁" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "SMA" }, { "start_idx": 21, "end_idx": 30, "type": "bod", "entity": "SMN1外显子7和8" } ] }, { "text": "SMN基因有多种拷贝[SMNt(telomeric)、SMNc(centromeric)],以及不同外显子缺失的遗传异质性,给SMA的研究带来了巨大的挑战。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "SMN基因" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "SMNt" }, { "start_idx": 16, "end_idx": 24, "type": "bod", "entity": "telomeric" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "SMNc" }, { "start_idx": 32, "end_idx": 42, "type": "bod", "entity": "centromeric" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "SMA" } ] }, { "text": "有关SMN基因拷贝数与临床症状的严重程度的相关性尚在观察中。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "SMN基因" } ] }, { "text": "正常人的每个SMNt和SMNc都有2个等位基因,SMNt的两个等位基因的突变可能与疾病有关,而SMNc的突变与疾病很少或没有关联。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "SMNt" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "SMNc" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "SMNt" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "SMNc" } ] }, { "text": "目前研究表明在部分SMA-Ⅱ型和SMA-Ⅲ型患者中SMNt转化为SMNc,意味着随着SMNc拷贝数增加,临床症状的严重程度降低。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "SMA-Ⅱ型" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "SMA-Ⅲ型" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "SMNt" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "SMNc" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "SMNc" } ] }, { "text": "已知SMN基因的产物能与RNA结合蛋白相互作用,但其确切功能尚未阐明。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "SMN基因" }, { "start_idx": 12, "end_idx": 18, "type": "bod", "entity": "RNA结合蛋白" } ] }, { "text": "与正常人群相比反应产物在SMA-Ⅰ型患者的神经元中缺失,而在SMA-Ⅱ型和SMA-Ⅲ型中减少。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "SMA-Ⅰ型" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "神经元" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "SMA-Ⅱ型" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "SMA-Ⅲ型" } ] }, { "text": "正是由于基因的突变及转化引起脊髓前角运动神经元和脑干运动神经核变性,最终导致肌无力和肌萎缩。", "entities": [ { "start_idx": 14, "end_idx": 22, "type": "bod", "entity": "脊髓前角运动神经元" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "脑干运动神经核" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "肌无力" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "肌萎缩" } ] }, { "text": "【病理改变】各型SMA有不同的病理特点:(一)SMA-Ⅰ型肌肉病理特征是存在着大组分布的圆形萎缩肌纤维,常累及整个肌束亦见肥大纤维之中两型纤维均可受累并呈不完全同型肌群化萎缩肌纤维与那些未成熟纤维以及发育障碍与胚胎期肌纤维的外观相似,有作者称此为胚胎型或婴儿型肌纤维。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "SMA" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "SMA-Ⅰ型" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肌肉" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "萎缩肌纤维" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "肌束" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "常累及整个肌束" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "肥大纤维" }, { "start_idx": 59, "end_idx": 66, "type": "sym", "entity": "亦见肥大纤维之中" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "两型纤维" }, { "start_idx": 67, "end_idx": 74, "type": "sym", "entity": "两型纤维均可受累" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "肌群" }, { "start_idx": 75, "end_idx": 84, "type": "sym", "entity": "并呈不完全同型肌群化" }, { "start_idx": 85, "end_idx": 89, "type": "bod", "entity": "萎缩肌纤维" }, { "start_idx": 93, "end_idx": 97, "type": "bod", "entity": "未成熟纤维" }, { "start_idx": 100, "end_idx": 110, "type": "bod", "entity": "发育障碍与胚胎期肌纤维" }, { "start_idx": 123, "end_idx": 132, "type": "bod", "entity": "胚胎型或婴儿型肌纤维" } ] }, { "text": "(二)SMA-Ⅱ型肌肉病理形态改变类似SMA-Ⅰ型,但大组萎缩肌纤维不常见同型肌群化现象则更为突出继发性肌性损害改变,包括中央核增多肌纤维撕裂SMA-Ⅲ型本型在肌肉病理上可有多种表现。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "SMA-Ⅱ型" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肌肉" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "SMA-Ⅰ型" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "萎缩肌纤维" }, { "start_idx": 27, "end_idx": 36, "type": "sym", "entity": "大组萎缩肌纤维不常见" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肌群" }, { "start_idx": 37, "end_idx": 48, "type": "sym", "entity": "同型肌群化现象则更为突出" }, { "start_idx": 49, "end_idx": 55, "type": "dis", "entity": "继发性肌性损害" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "中央核" }, { "start_idx": 61, "end_idx": 65, "type": "sym", "entity": "中央核增多" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "肌纤维" }, { "start_idx": 66, "end_idx": 70, "type": "sym", "entity": "肌纤维撕裂" }, { "start_idx": 71, "end_idx": 76, "type": "dis", "entity": "SMA-Ⅲ型" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "肌肉" } ] }, { "text": "某些病例仅显示轻微变化,如小组同型肌群化,少量萎缩肌纤维等;其形态大致正常。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "小组同型肌群化" }, { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "少量萎缩肌纤维" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "形态大致正常" } ] }, { "text": "多数严重病例,肌肉活体组织检查表现与病期相关。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "pro", "entity": "肌肉活体组织检查" } ] }, { "text": "儿童早期,以萎缩小纤维为主可见同型肌群化以同型肌群化为主要特征合并成组或成束小点状萎缩肌纤维本型肌纤维肥大改变十分突出,直径可达100~150μm,常合并继发性肌原损害,包括纤维撕裂中央核改变NADH染色见蛾噬样指纹状纤维少量坏死和再生纤维巨噬细胞浸润间质脂肪结缔组织增生SMA-Ⅰ型、SMA-Ⅱ型以及SMA-Ⅲ型。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "萎缩小纤维" }, { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "以萎缩小纤维为主" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "同型肌群" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "可见同型肌群化" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "同型肌群" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "以同型肌群化为主要特征" }, { "start_idx": 38, "end_idx": 45, "type": "bod", "entity": "小点状萎缩肌纤维" }, { "start_idx": 31, "end_idx": 45, "type": "sym", "entity": "合并成组或成束小点状萎缩肌纤维" }, { "start_idx": 46, "end_idx": 52, "type": "sym", "entity": "本型肌纤维肥大" }, { "start_idx": 74, "end_idx": 83, "type": "sym", "entity": "常合并继发性肌原损害" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "纤维" }, { "start_idx": 87, "end_idx": 90, "type": "sym", "entity": "纤维撕裂" }, { "start_idx": 91, "end_idx": 93, "type": "bod", "entity": "中央核" }, { "start_idx": 91, "end_idx": 95, "type": "sym", "entity": "中央核改变" }, { "start_idx": 96, "end_idx": 99, "type": "bod", "entity": "NADH" }, { "start_idx": 96, "end_idx": 105, "type": "sym", "entity": "NADH染色见蛾噬样" }, { "start_idx": 109, "end_idx": 110, "type": "bod", "entity": "纤维" }, { "start_idx": 106, "end_idx": 110, "type": "sym", "entity": "指纹状纤维" }, { "start_idx": 111, "end_idx": 114, "type": "sym", "entity": "少量坏死" }, { "start_idx": 118, "end_idx": 119, "type": "bod", "entity": "纤维" }, { "start_idx": 116, "end_idx": 119, "type": "sym", "entity": "再生纤维" }, { "start_idx": 120, "end_idx": 123, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 120, "end_idx": 125, "type": "sym", "entity": "巨噬细胞浸润" }, { "start_idx": 126, "end_idx": 133, "type": "bod", "entity": "间质脂肪结缔组织" }, { "start_idx": 126, "end_idx": 135, "type": "sym", "entity": "间质脂肪结缔组织增生" }, { "start_idx": 136, "end_idx": 141, "type": "dis", "entity": "SMA-Ⅰ型" }, { "start_idx": 143, "end_idx": 148, "type": "dis", "entity": "SMA-Ⅱ型" }, { "start_idx": 151, "end_idx": 156, "type": "dis", "entity": "SMA-Ⅲ型" } ] }, { "text": "(一)SMA-Ⅰ型也称为婴儿型脊髓性肌萎缩症或Werdnig-Hoffmann病。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "SMA-Ⅰ型" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "婴儿型脊髓性肌萎缩症" }, { "start_idx": 23, "end_idx": 39, "type": "dis", "entity": "Werdnig-Hoffmann病" } ] }, { "text": "本型在三型中最为严重,约1/3病例在宫内发病,胎动变弱,半数在出生时或出生后的最初几个月即可发病,且几乎均在5个月内发病。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "胎动变弱" } ] }, { "text": "患者有严重肌无力(以四肢近端为主,躯干肌也可受累肌张力的低下括约肌的张力和感觉仍保持正常仰卧时腿呈蛙式肋间肌无力影响呼吸胸廓凹陷畸形双侧腹部脏器突出膈肌麻痹面肌和咀嚼肌无力吮吸及吞咽困难;由于不能摄取足够的能量,造成营养不良;除了手指和脚趾之外,无自发活动,常常有手指的细微震颤多发性微小肌阵挛(polyminimyoclonus)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "严重肌无力" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "四肢近端" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "躯干肌" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "躯干肌也可受累" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "肌张力" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "肌张力的低下" }, { "start_idx": 30, "end_idx": 35, "type": "ite", "entity": "括约肌的张力" }, { "start_idx": 30, "end_idx": 43, "type": "sym", "entity": "括约肌的张力和感觉仍保持正常" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "腿" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "仰卧时腿呈蛙式" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "肋间肌无力" }, { "start_idx": 51, "end_idx": 59, "type": "sym", "entity": "肋间肌无力影响呼吸" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "胸廓" }, { "start_idx": 60, "end_idx": 65, "type": "sym", "entity": "胸廓凹陷畸形" }, { "start_idx": 66, "end_idx": 71, "type": "bod", "entity": "双侧腹部脏器" }, { "start_idx": 66, "end_idx": 73, "type": "sym", "entity": "双侧腹部脏器突出" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "膈肌" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "膈肌麻痹" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "面肌" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "咀嚼肌" }, { "start_idx": 78, "end_idx": 85, "type": "sym", "entity": "面肌和咀嚼肌无力" }, { "start_idx": 86, "end_idx": 92, "type": "sym", "entity": "吮吸及吞咽困难" }, { "start_idx": 108, "end_idx": 111, "type": "sym", "entity": "营养不良" }, { "start_idx": 115, "end_idx": 116, "type": "bod", "entity": "手指" }, { "start_idx": 118, "end_idx": 119, "type": "bod", "entity": "脚趾" }, { "start_idx": 132, "end_idx": 133, "type": "bod", "entity": "手指" }, { "start_idx": 132, "end_idx": 138, "type": "sym", "entity": "手指的细微震颤" }, { "start_idx": 139, "end_idx": 146, "type": "dis", "entity": "多发性微小肌阵挛" }, { "start_idx": 148, "end_idx": 164, "type": "dis", "entity": "polyminimyoclonus" } ] }, { "text": "舌肌束颤较为常见腱反射常常消失呼吸功能不全误吸,任何轻微的上呼吸道感染可迅速演变为重症肺炎,危及生命。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "舌肌" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "舌肌束颤较为常见" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "腱" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "腱反射常常消失" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "呼吸功能" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "呼吸功能不全" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "误吸" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "重症肺炎" } ] }, { "text": "患者的社会交往能力和运动功能的丧失有明显差异。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "ite", "entity": "交往能力" }, { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "运动功能" } ] }, { "text": "(二)SMA-Ⅱ型也称为少年型SMA、中间型SMA或慢性SMA,发病较Ⅰ型稍迟,多于1岁内起病。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "SMA-Ⅱ型" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "少年型SMA" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "中间型SMA" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "慢性SMA" } ] }, { "text": "患儿在6~8个月时生长发育正常,多数病例表现以近端为主的严重肌无力下肢重于上肢多发性微小肌阵挛呼吸肌以及吞咽肌不受累括约肌功能正常SMA-Ⅲ型也称为Wohlfart-Kugelberg-Welander综合征或轻度SMA,是SMA中表现最轻的一类。", "entities": [ { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "严重肌无力" }, { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "以近端为主的严重肌无力" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "下肢" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "上肢" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "下肢重于上肢" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "微小肌" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "多发性微小肌阵挛" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "吞咽肌" }, { "start_idx": 47, "end_idx": 57, "type": "sym", "entity": "呼吸肌以及吞咽肌不受累" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "括约肌" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "括约肌功能正常" }, { "start_idx": 65, "end_idx": 70, "type": "dis", "entity": "SMA-Ⅲ型" }, { "start_idx": 74, "end_idx": 103, "type": "dis", "entity": "Wohlfart-Kugelberg-Welander综合征" }, { "start_idx": 105, "end_idx": 109, "type": "dis", "entity": "轻度SMA" }, { "start_idx": 112, "end_idx": 114, "type": "dis", "entity": "SMA" } ] }, { "text": "能行走的SMA-Ⅲ型患儿可出现蹒跚步态,腰椎前突腹部凸起腱反射可有可无肌无力的发病年龄密切相关,2岁前发病者将在15岁左右不能行走,2岁后发病者可一直保持行走能力至50岁左右。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "SMA-Ⅲ型" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "蹒跚步态" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "腰椎" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "腰椎前突" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "腹部" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "腹部凸起" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "腱" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "腱反射可有可无" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "肌无力" } ] }, { "text": "大量的前瞻性临床研究表明,SMA-Ⅱ型和Ⅲ型在数年内肌无力症状进展缓慢或没有进展。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "SMA-Ⅱ型" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "肌无力" } ] }, { "text": "另外,不典型SMA进行性延髓麻痹(Fazio-Londe病),患者脑干运动核进行性受损数量逐渐减少,引起进行性延髓麻痹,但不伴或很少伴有脊髓前角运动神经元受损现象明显的面肌无力脑神经运动神经核受累症状脑神经以下的神经核,眼外肌一般不受累。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "不典型SMA进行性延髓麻痹" }, { "start_idx": 17, "end_idx": 28, "type": "dis", "entity": "Fazio-Londe病" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "脑干运动核" }, { "start_idx": 33, "end_idx": 42, "type": "sym", "entity": "脑干运动核进行性受损" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "数量逐渐减少" }, { "start_idx": 52, "end_idx": 58, "type": "dis", "entity": "进行性延髓麻痹" }, { "start_idx": 68, "end_idx": 76, "type": "bod", "entity": "脊髓前角运动神经元" }, { "start_idx": 66, "end_idx": 80, "type": "sym", "entity": "伴有脊髓前角运动神经元受损现象" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "面肌" }, { "start_idx": 81, "end_idx": 87, "type": "sym", "entity": "明显的面肌无力" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "脑神经" }, { "start_idx": 88, "end_idx": 99, "type": "sym", "entity": "脑神经运动神经核受累症状" }, { "start_idx": 100, "end_idx": 102, "type": "bod", "entity": "脑神经" }, { "start_idx": 106, "end_idx": 108, "type": "bod", "entity": "神经核" }, { "start_idx": 110, "end_idx": 112, "type": "bod", "entity": "眼外肌" } ] }, { "text": "最近,分子生物学研究证实至少有部分SMA患者可伴有关节屈曲呼吸衰竭和关节屈曲SMN基因缺失,而另外2名无关节挛缩的婴儿无SMN基因缺失。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "SMA" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "关节" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "关节屈曲" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "关节" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "关节屈曲" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "SMN基因" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "SMN基因" } ] }, { "text": "这些发现提示伴有关节弯曲肌无力或肌张力低下SMN基因突变的检测。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "关节" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "关节弯曲" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "肌无力" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "肌张力" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "SMN基因" } ] }, { "text": "【实验室检查】(一)基因诊断自从SMN基因发现以来,SMA的诊断流程发生了改变,可通过血DNA分析检测SMN基因突变,从而诊断疾病。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "基因诊断" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "SMN基因" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "SMA" }, { "start_idx": 43, "end_idx": 48, "type": "pro", "entity": "血DNA分析" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "SMN基因" } ] }, { "text": "一旦发现SMN基因突变,则不需要再作其他检查,即可确诊为SMA。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "SMN基因" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "SMA" } ] }, { "text": "Ⅲ型患者SMN基因缺失率低,通过检测SMN基因7、8外显子进行基因诊断时需谨慎。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "SMN基因" }, { "start_idx": 18, "end_idx": 28, "type": "bod", "entity": "SMN基因7、8外显子" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "基因诊断" } ] }, { "text": "NAIP基因在SMA发病中的作用尚不清楚,有待进一步研究。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "NAIP基因" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "SMA" } ] }, { "text": "如果无SMN基因缺失,需作下列一些传统的检查方法以明确诊断。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "SMN基因" } ] }, { "text": "检查方法有血清肌酸磷酸激酶(CK)测定;电生理检查包括神经传导速度(NCV)和肌电图(EMG)的检测及肌肉活体组织检查。", "entities": [ { "start_idx": 5, "end_idx": 18, "type": "pro", "entity": "血清肌酸磷酸激酶(CK)测定" }, { "start_idx": 20, "end_idx": 32, "type": "pro", "entity": "电生理检查包括神经传导速度" }, { "start_idx": 34, "end_idx": 36, "type": "pro", "entity": "NCV" }, { "start_idx": 39, "end_idx": 49, "type": "pro", "entity": "肌电图(EMG)的检测" }, { "start_idx": 51, "end_idx": 58, "type": "pro", "entity": "肌肉活体组织检查" } ] }, { "text": "(二)血清CPKSMA-Ⅰ型正常,Ⅱ型偶见增高。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "血清CPK" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "SMA-Ⅰ型" } ] }, { "text": "Ⅲ型常增高,甚至可达正常值10倍以上,同工酶变化以MM为主,随着肌损害的发展而增加,至晚期肌肉萎缩时,CK才开始下降,这与肌营养不良不同,后者于婴幼儿期即达到高峰,以后渐降。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "同工酶" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "肌肉萎缩" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "CK" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "肌营养不良" } ] }, { "text": "(三)电生理检查(NCV和EMG)电生理检查可反映SMA的严重程度和进展程度,但各型EMG改变相似,包括纤颤电位、复合运动单位动作电位(MVAPS)波幅时限增加,以及干扰相减少。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "电生理检查" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "NCV" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "EMG" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "电生理检查" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "SMA" }, { "start_idx": 42, "end_idx": 44, "type": "pro", "entity": "EMG" }, { "start_idx": 52, "end_idx": 55, "type": "ite", "entity": "纤颤电位" }, { "start_idx": 57, "end_idx": 66, "type": "ite", "entity": "复合运动单位动作电位" }, { "start_idx": 68, "end_idx": 72, "type": "ite", "entity": "MVAPS" } ] }, { "text": "纤颤电位及正锐波在各型SMA均可出现,但SMA-Ⅰ型更明显。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "纤颤电位" }, { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "正锐波" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "SMA" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "SMA-Ⅰ型" } ] }, { "text": "随意运动时,各型SMA均见干扰相减少,尤其是Ⅰ型SMA仅呈单相。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "SMA" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "Ⅰ型SMA" } ] }, { "text": "在较晚期Ⅲ型SMA可见类似于肌源性损害的低波幅多相电位。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "Ⅲ型SMA" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肌源性损害" } ] }, { "text": "电生理检查NCV示运动传导速度可减慢,在Ⅰ型减慢,而其他类型正常;感觉传导速度正常。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "电生理检查NCV" }, { "start_idx": 9, "end_idx": 17, "type": "sym", "entity": "运动传导速度可减慢" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "感觉传导速度正常" } ] }, { "text": "检测婴儿运动NCV有一定难度,这是因为婴儿的肢体较小且刺激点和记录电极的距离较短,检测结果常常是正常传导速度,或有时比预期的传导速度还快。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "NCV" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肢体" } ] }, { "text": "(四)肌肉活体组织检查肌肉活体组织检查对确诊SMA具有重要意义,其病理表现特征是具有失神经和神经再支配现象。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "肌肉活体组织检查" }, { "start_idx": 11, "end_idx": 18, "type": "pro", "entity": "肌肉活体组织检查" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "SMA" }, { "start_idx": 42, "end_idx": 52, "type": "sym", "entity": "失神经和神经再支配现象" } ] }, { "text": "各型SMA有不同的肌肉病理特点,病程早期有同型肌群化,晚期可有肌纤维坏死。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "SMA" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肌肉" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "同型肌群化" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "肌纤维坏死" } ] }, { "text": "【治疗】目前尚无有效治疗,治疗措施主要是预防或治疗SMA的各种并发症。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "SMA" } ] }, { "text": "由于肋间肌和膈肌的肌无力,引起通气不足以及咳嗽微弱;长期卧床可造成坠积;误吸也可造成肺炎。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肋间肌" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "膈肌" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "肌无力" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "咳嗽" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "肺炎" } ] }, { "text": "预防肺炎的有效措施有辅助咳嗽、胸部叩击治疗及间歇正压通气。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肺炎" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "辅助咳嗽" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "胸部叩击治疗" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "间歇正压通气" } ] }, { "text": "即使在没有急性呼吸道感染的情况下,患者也需保持良好的肺部通气状态,预防发生进行性肺不张。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "急性呼吸道感染" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "肺部通气" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "进行性肺不张" } ] }, { "text": "除急性感染患者,氧疗一般不适用,因为限制性肺病患者在出现低氧血症前就已有CO2潴留,氧疗可能会引起呼吸功能抑制,呼吸暂停,最终导致死亡。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "急性感染" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "氧疗" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "限制性肺病" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "低氧血症" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "氧疗" }, { "start_idx": 55, "end_idx": 60, "type": "sym", "entity": "呼吸功能抑制" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "呼吸暂停" } ] }, { "text": "血气和FVC是有效的监测手段,当患者出现CO2潴留,可应用非侵入性通气、正压或负压通气。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "血气" }, { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "FVC" }, { "start_idx": 35, "end_idx": 40, "type": "pro", "entity": "非侵入性通气" }, { "start_idx": 42, "end_idx": 48, "type": "pro", "entity": "正压或负压通气" } ] }, { "text": "患者常由于吮吸乏力、气道不畅营养不良和生长障碍。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "吮吸乏力" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "气道" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "气道不畅" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "营养不良" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "生长障碍" } ] }, { "text": "部分不能经口摄入足够热量的患者,需要予以鼻饲喂养。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "鼻饲喂养" } ] }, { "text": "脊柱矫正法常不能预防或延缓脊柱侧弯,但可以帮助患者坐起。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "脊柱矫正法" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "脊柱侧弯" } ] }, { "text": "无论有无脊柱矫正,患者的肺功能均应予以监测。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "脊柱矫正" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "肺功能" } ] }, { "text": "脊柱手术的时间至关重要,因为必须让患儿充分生长,并等待时机直至弯曲已十分严重,同时只有肺功能相对正常时才有望进行手术。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "脊柱手术" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "肺功能" } ] }, { "text": "脊柱融合术后,脊柱侧弯的程度将明显改善,同时肺活量、坐、平衡以及舒适感也明显改善。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "脊柱融合术" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "脊柱侧弯" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "肺活量" } ] }, { "text": "另外,有学者对6例SMA病例进行促甲状腺激素释放激素(TRH)治疗,剂量为每次0.1mg/kg,通过经皮静脉导管给药。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "SMA" }, { "start_idx": 16, "end_idx": 32, "type": "pro", "entity": "促甲状腺激素释放激素(TRH)治疗" }, { "start_idx": 50, "end_idx": 57, "type": "pro", "entity": "经皮静脉导管给药" } ] }, { "text": "结果显示患儿腓神经传导速度比对照组明显增快,且患儿家长也反映患儿某些功能明显改善。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "ite", "entity": "腓神经传导速度" } ] }, { "text": "因此TRH不失为一种有用的治疗手段,但需要进一步研究证实。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "TRH" } ] }, { "text": "随着SMN和NAIP基因的确认及深入研究,基因治疗或体外基因活化治疗将是非常有希望的治疗手段。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "bod", "entity": "SMN和NAIP基因" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "基因治疗" }, { "start_idx": 26, "end_idx": 33, "type": "pro", "entity": "体外基因活化治疗" } ] }, { "text": "【预后】目前为止,SMA无特异治疗,预后主要与疾病的类型有关,Ⅰ型患者一般生存期在2岁以内,Ⅱ型患者生存期在5岁以内,而Ⅲ型患者可存活至成人,部分患者不影响寿命。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "SMA" } ] }, { "text": "参考文献1.李家宜,苏祖佑,陈新民.心肌炎//杨思源.小儿心脏病学.第2版.北京:人民卫生出版社,1994:359-3592.中华医学会儿科学分会心血管学组.中华儿科杂志编辑委员会.病毒性心肌炎诊断标准(修订草案).中华儿科杂志,2000,38(2):753.AingerLE,LawyerNG,FitchCW.Neonatalrubellamyocarditis.BrHeartJ,1996,28:691-6974.BowlesNE,TowbinJA.Molecularaspectsofmyocarditis.CurrOpinCardiol,1998,13:179-1795.FriedmanRA,SchowengerdtKO,TowbinJA.Myocarditis.In:BrickerJT,GarsonAJr,FisherDJ,etal.Thescienceandpracticeofpediatriccardiology,2nded.Baltimore:Williams&Wilkins,1998,1777-17776.LiuPP,MasonJW.Advancesintheunderstandingofmyocarditis.Circulation,2001,104:1076-10767.MasonJW,O’ConnellJB,HerskowitzA,etal.Aclinicaltrialofimmunosuppressivetherapyformyocarditis.NEnglJMed,1995,33:269-2698.ParrilloJE.Myocarditis:howshouldwetreatin1998?", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "心肌炎" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "心脏病" }, { "start_idx": 68, "end_idx": 69, "type": "dep", "entity": "儿科" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "心血管" }, { "start_idx": 91, "end_idx": 96, "type": "dis", "entity": "病毒性心肌炎" }, { "start_idx": 110, "end_idx": 111, "type": "dep", "entity": "儿科" } ] }, { "text": "三、在经验性治疗前,应进行病原菌培养和药敏试验尽管呼吸道感染病原学检查阳性率不高,而且结果有明显的滞后性,但阳性的结果对治疗有很高的指导价值,尤其在初次经验性治疗失败后,有助于对治疗方案的修正,改善治疗效果。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "ite", "entity": "病原菌培养" }, { "start_idx": 20, "end_idx": 23, "type": "ite", "entity": "药敏试验" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "病原学检查" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "阳性" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "阳性" } ] }, { "text": "为了尽可能减少药物治疗对病原学检查结果的影响,在经验性治疗之前,进行必要的病原学检查。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "病原学检查" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "病原学检查" } ] }, { "text": "病原学检查主要对痰、胸腔积液、经皮肺穿刺物和肺泡灌洗液进行涂片后革兰染色、抗酸染色、免疫荧光或酶标抗体染色,细菌培养或病原核酸测定。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "病原学检查" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "痰" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "胸腔积液" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "经皮肺穿刺物" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肺泡" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "涂片" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "革兰染色" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "抗酸染色" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "免疫荧光" }, { "start_idx": 47, "end_idx": 52, "type": "pro", "entity": "酶标抗体染色" }, { "start_idx": 54, "end_idx": 57, "type": "ite", "entity": "细菌培养" }, { "start_idx": 59, "end_idx": 64, "type": "ite", "entity": "病原核酸测定" } ] }, { "text": "革兰染色可以显示:镰刀状成串排列双球菌(肺炎链球菌)、成簇分布的革兰阳性球菌(金黄色葡萄球菌)、革兰阴性球杆菌(流感嗜血杆菌)、革兰阴性杆菌(肺炎克雷伯杆菌或肠杆菌);抗酸染色阳性,提示为结核杆菌感染;必要时可以用免疫荧光或酶标抗体染色后进行确认。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "革兰染色" }, { "start_idx": 16, "end_idx": 18, "type": "mic", "entity": "双球菌" }, { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 32, "end_idx": 37, "type": "mic", "entity": "革兰阳性球菌" }, { "start_idx": 39, "end_idx": 45, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 48, "end_idx": 54, "type": "mic", "entity": "革兰阴性球杆菌" }, { "start_idx": 56, "end_idx": 61, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 64, "end_idx": 69, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 71, "end_idx": 77, "type": "mic", "entity": "肺炎克雷伯杆菌" }, { "start_idx": 79, "end_idx": 81, "type": "mic", "entity": "肠杆菌" }, { "start_idx": 84, "end_idx": 87, "type": "pro", "entity": "抗酸染色" }, { "start_idx": 84, "end_idx": 89, "type": "sym", "entity": "抗酸染色阳性" }, { "start_idx": 94, "end_idx": 99, "type": "dis", "entity": "结核杆菌感染" }, { "start_idx": 107, "end_idx": 110, "type": "pro", "entity": "免疫荧光" }, { "start_idx": 112, "end_idx": 117, "type": "pro", "entity": "酶标抗体染色" } ] }, { "text": "只有25%~50%的CAP痰培养阳性,即使有菌血症的患儿,痰培养阳性率也仅为40%~60%。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "CAP" }, { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "痰培养" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "菌血症" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "痰培养" } ] }, { "text": "由于一些致病菌,如肺炎链球菌和流感嗜血杆菌可以是正常口腔菌群的一部分,从痰标本分离出的病原菌不一定是致病的原因;而且非典型病原中如肺炎支原体、肺炎衣原体以及厌氧菌,常规痰培养难以获得阳性结果,因而痰培养的结果也只能作为临床治疗的参考。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "mic", "entity": "致病菌" }, { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 15, "end_idx": 20, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "口腔" }, { "start_idx": 36, "end_idx": 38, "type": "equ", "entity": "痰标本" }, { "start_idx": 43, "end_idx": 45, "type": "mic", "entity": "病原菌" }, { "start_idx": 65, "end_idx": 69, "type": "mic", "entity": "肺炎支原体" }, { "start_idx": 71, "end_idx": 75, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 78, "end_idx": 80, "type": "mic", "entity": "厌氧菌" }, { "start_idx": 84, "end_idx": 86, "type": "ite", "entity": "痰培养" }, { "start_idx": 98, "end_idx": 100, "type": "ite", "entity": "痰培养" } ] }, { "text": "必要时,通过经皮肺穿刺或纤维支气管镜获取肺泡灌洗液进行培养,有助于明确下呼吸道感染局部的病原,对难治性下呼吸道感染有重要价值。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "经皮肺穿刺" }, { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "纤维支气管镜" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肺泡" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "下呼吸道感染" } ] }, { "text": "血液或胸腔积液中分离出的病原菌具有高度的特异性,但住院肺炎患儿的血培养阳性率仅为5%~20%,伴有胸腔积液的肺炎只占住院肺炎患儿15%。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血液" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "胸腔积液" }, { "start_idx": 12, "end_idx": 14, "type": "mic", "entity": "病原菌" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "肺炎" }, { "start_idx": 32, "end_idx": 34, "type": "ite", "entity": "血培养" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "胸腔积液" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "肺炎" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "肺炎" } ] }, { "text": "此外还可以进行血清病原特异性抗体测定,如肺炎支原体抗体、结核抗体等。", "entities": [ { "start_idx": 7, "end_idx": 17, "type": "pro", "entity": "血清病原特异性抗体测定" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "肺炎支原体抗体" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "结核抗体" } ] }, { "text": "但特异性抗体的产生和消失具有明显的滞后性,要明确本次感染的病原,必须进行动态观察抗体滴度的改变。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "特异性抗体" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "感染" } ] }, { "text": "参考文献1.周吕.胃肠生理学基础与临床.北京:科学出版社,1991:3432.江米足,叶瑞云,欧弼悠,等.24小时食管pH值小儿胃食管反流.中华儿科杂志,1997,35:2603.MazioIL,GiacobleA,ConoscitoreP,etal.Evaluationoftheuseofultrasonographyinthestudyofliquidgastricemptying.AmJGastroenterol,1989,84:4964.HillemierAC.Gastroesophagealreflux:diagnosticandtherapeuticapproaches.PediatricClinNorthAm,1996,43:197", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胃肠" }, { "start_idx": 57, "end_idx": 61, "type": "ite", "entity": "食管pH值" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "小儿胃食管反流" } ] }, { "text": "二、红细胞沉降率与急性期反应物希腊人很早就发现某些风湿性疾病患者有血红细胞沉降率(ESR,以下简称血沉)加快现象,现已证明ESR反映了红细胞凝集性变化,多种生物机制都可能影响其凝集性,尤其是血浆中某些蛋白质量增加,特别是纤维蛋白。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "ite", "entity": "红细胞沉降率" }, { "start_idx": 9, "end_idx": 14, "type": "ite", "entity": "急性期反应物" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 33, "end_idx": 39, "type": "ite", "entity": "血红细胞沉降率" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "ESR" }, { "start_idx": 49, "end_idx": 50, "type": "ite", "entity": "血沉" }, { "start_idx": 61, "end_idx": 63, "type": "ite", "entity": "ESR" }, { "start_idx": 67, "end_idx": 69, "type": "ite", "entity": "红细胞" }, { "start_idx": 95, "end_idx": 96, "type": "ite", "entity": "血浆" }, { "start_idx": 100, "end_idx": 101, "type": "ite", "entity": "蛋白" }, { "start_idx": 110, "end_idx": 113, "type": "ite", "entity": "纤维蛋白" } ] }, { "text": "急性期反应蛋白成分增加是炎症状态下的非特异性现象,也是ESR加快的重要原因。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "ite", "entity": "急性期反应蛋白成分" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "ESR" } ] }, { "text": "相反,红细胞增多症、镰状细胞贫血以及变形红细胞增多等均将出现ESR减慢。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "红细胞增多症" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "镰状细胞贫血" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "变形红细胞增多" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "ESR" } ] }, { "text": "但并不是所有活动性炎症病人ESR都会升高,ESR检测正常也不能除外风湿性疾病活动的可能性。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "ESR" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "ESR检测" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "检测ESR或任何一种急性期反应物对风湿性疾病的诊断价值甚微。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "ESR" }, { "start_idx": 10, "end_idx": 15, "type": "ite", "entity": "急性期反应物" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "第七节弓形虫病弓形虫病(toxoplasmosis)是一种由刚地弓形虫(toxoplasmagondii)所致的一种人畜共患的寄生虫性传染病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "弓形虫病" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "弓形虫病" }, { "start_idx": 12, "end_idx": 24, "type": "dis", "entity": "toxoplasmosis" }, { "start_idx": 30, "end_idx": 34, "type": "mic", "entity": "刚地弓形虫" }, { "start_idx": 36, "end_idx": 51, "type": "mic", "entity": "toxoplasmagondii" }, { "start_idx": 63, "end_idx": 69, "type": "dis", "entity": "寄生虫性传染病" } ] }, { "text": "【病原】刚地弓形虫是一种专性细胞内寄生的原虫,属顶端复合物亚门,孢子纲,真球虫目。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "mic", "entity": "刚地弓形虫" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "原虫" } ] }, { "text": "弓形虫以三种形态存在:滋养体、囊殖子、卵囊。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "弓形虫" } ] }, { "text": "滋养体大小为3μm×7μm,呈新月形,滋养体可感染所有哺乳动物的细胞,是急性感染的特征;囊殖子的大小与滋养体相同,可在宿主体内的组织中持续终生,是感染复发的来源;卵囊大小约10μm×12μm,随粪便排出体外的卵囊发育成孢子囊并产生孢子。", "entities": [ { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "急性感染" }, { "start_idx": 73, "end_idx": 74, "type": "dis", "entity": "感染" }, { "start_idx": 97, "end_idx": 98, "type": "bod", "entity": "粪便" } ] }, { "text": "刚地弓形虫基因组含有8×107个碱基对。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "刚地弓形虫" } ] }, { "text": "【流行病学】弓形虫病在世界上分部广泛,感染率约为25%~50%左右,欧美某些国家高达50%以上。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "弓形虫病" } ] }, { "text": "根据卫生部2001年6月~2004年年底在全国进行的人体重要寄生虫病现状调查,弓形虫的血清学阳性率为7.88%,贵州最高15.09%,黑龙江最低为0.55%。", "entities": [ { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "寄生虫病" }, { "start_idx": 39, "end_idx": 41, "type": "mic", "entity": "弓形虫" } ] }, { "text": "免疫功能正常的母亲在妊娠期间受到初次感染,或者免疫受损的母亲受到急性或慢性感染,胎儿可获得先天性感染。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "感染" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "免疫受损" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "急性或慢性感染" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "先天性感染" } ] }, { "text": "母婴总的传播率为29%,孕妇弓形虫IgM血清转化出现时间与其子女临床症状的发生有明显相关,当孕期13周出现IgM阳性,其子女发生临床症状的危险率为61%,26周时为25%。", "entities": [ { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "IgM阳性" } ] }, { "text": "【临床表现】弓形虫病在不同人群的表现差异较大。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "弓形虫病" } ] }, { "text": "(一)在免疫功能正常的年长儿中的感染原发性感染为无症状性,或引起淋巴结肿大,伴或不伴发热,引起脑、心、眼等组织损伤者少见。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "原发性感染" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "淋巴结肿大" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "发热" }, { "start_idx": 47, "end_idx": 56, "type": "dis", "entity": "脑、心、眼等组织损伤" } ] }, { "text": "淋巴结肿大最常见于颈部,但也可累及所有解剖部位的淋巴结,可有触痛,不发生化脓,病程可持续1年。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "淋巴结肿大" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "颈部" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "淋巴结" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "触痛" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "化脓" } ] }, { "text": "(二)在免疫受损的年长儿中的感染原发感染,或慢性、既往隐性感染的再活化,可累及所有器官,但最常见的是中枢神经系统。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "免疫受损" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "原发感染" }, { "start_idx": 22, "end_idx": 30, "type": "dis", "entity": "慢性、既往隐性感染" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "器官" }, { "start_idx": 50, "end_idx": 55, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "40%的艾滋病患者发生活动性弓形虫病。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "艾滋病" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "活动性弓形虫病" } ] }, { "text": "在伴有艾滋病的患者中,脑是最常受累的器官,但肺、眼和多个器官也可受累。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "艾滋病" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "脑" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肺" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "眼" } ] }, { "text": "伴有艾滋病的患者表现为局限性神经系统体征(包括偏瘫,语言异常)、惊厥、帕金森体征、痴呆、精神异常。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "艾滋病" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "局限性神经系统体征" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "偏瘫" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "语言异常" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "惊厥" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "帕金森体征" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "痴呆" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "精神异常" } ] }, { "text": "(三)宫内获得性感染先天性弓形虫感染较后天性感染严重。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "宫内获得性感染" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "先天性弓形虫感染" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "后天性感染" } ] }, { "text": "在妊娠4、5、6个月传播的感染若不治疗,出生时也常出现弓形虫病症状;在妊娠7、8、9个月获得的感染在新生儿期常无症状,若不治疗,几乎同样引起后遗症,如脉络膜视网膜炎和视力受损,也有认知和运动功能障碍、惊厥和听力受损。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "弓形虫病" }, { "start_idx": 75, "end_idx": 81, "type": "dis", "entity": "脉络膜视网膜炎" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "视力受损" }, { "start_idx": 90, "end_idx": 98, "type": "dis", "entity": "认知和运动功能障碍" }, { "start_idx": 100, "end_idx": 101, "type": "dis", "entity": "惊厥" }, { "start_idx": 103, "end_idx": 106, "type": "dis", "entity": "听力受损" } ] }, { "text": "(一)患儿的血液、骨髓、淋巴穿刺液或脑脊液沉淀等涂片用吉姆萨或瑞氏染色可能找到病原,但阳性率不高。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血液" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "骨髓" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "淋巴穿刺液" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "脑脊液沉淀" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "涂片" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "吉姆萨" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "瑞氏染色" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "阳性率不高" } ] }, { "text": "(二)血清学检查方法1.亚甲蓝染色试验在感染早期(10~14天)即开始阳性,第3~5周效价可达高峰,可维持数月和数年,而从母亲得来的抗体在生后3~6个月内消失。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "亚甲蓝染色试验" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "抗体" } ] }, { "text": "2.间接免疫荧光试验所测抗体是抗弓形虫IgG抗体,其临床意义与亚甲蓝染色试验相仿。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "间接免疫荧光试验" }, { "start_idx": 15, "end_idx": 23, "type": "bod", "entity": "抗弓形虫IgG抗体" }, { "start_idx": 31, "end_idx": 37, "type": "pro", "entity": "亚甲蓝染色试验" } ] }, { "text": "3.聚合酶链反应可以早期、快速地明确诊断。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "聚合酶链反应" } ] }, { "text": "【治疗】弓形虫病的治疗因不同的感染人群及免疫功能而不同。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "弓形虫病" } ] }, { "text": "(一)急性弓形虫病的孕妇若胎儿未感染,螺旋霉素1g,8小时1次,不与食物同服,疗程到发现胎儿感染为止,或在18~20周停用;若发现胎儿感染,可换用乙胺嘧啶、磺胺嘧啶和四氢叶酸,足月为止。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "急性弓形虫病" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "螺旋霉素" }, { "start_idx": 73, "end_idx": 76, "type": "dru", "entity": "乙胺嘧啶" }, { "start_idx": 78, "end_idx": 81, "type": "dru", "entity": "磺胺嘧啶" }, { "start_idx": 83, "end_idx": 86, "type": "dru", "entity": "四氢叶酸" } ] }, { "text": "(二)妊娠17周后肯定胎儿感染或母亲在妊娠最后几周内获得感染乙胺嘧啶负荷量:100mg/d,分为2次,2天后改为50mg/d;磺胺嘧啶负荷量:75mg/(kg•d),分为2次,(最大量4g/d),2天后改为100mg/(kg•d)分2次(最大量4g/d);四氢叶酸5~20mg/d。", "entities": [ { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "乙胺嘧啶" }, { "start_idx": 63, "end_idx": 66, "type": "dru", "entity": "磺胺嘧啶" }, { "start_idx": 128, "end_idx": 131, "type": "dru", "entity": "四氢叶酸" } ] }, { "text": "疗程到足月为止(在乙胺嘧啶停用后,四氢叶酸继用1周)。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "乙胺嘧啶" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "四氢叶酸" } ] }, { "text": "(三)婴儿先天性弓形虫感染乙胺嘧啶负荷量:2mg/(kg•d),2天后改为1mg/(kg•d),持续2~6个月后改为每周一、三、五各服1次;磺胺嘧啶100mg/(kg•d),分2次;四氢叶酸5~10mg,每周3次。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "婴儿先天性弓形虫感染" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "乙胺嘧啶" }, { "start_idx": 70, "end_idx": 73, "type": "dru", "entity": "磺胺嘧啶" }, { "start_idx": 91, "end_idx": 94, "type": "dru", "entity": "四氢叶酸" } ] }, { "text": "疗程1年(在乙胺嘧啶停用后,四氢叶酸继用一周)。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "乙胺嘧啶" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "四氢叶酸" } ] }, { "text": "(四)年长儿活动性脉络膜视网膜炎乙胺嘧啶负荷量:2mg/(kg•d)(最大剂量50mg),2天后改为1mg/(kg•d),最大剂量25mg维持。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "年长儿活动性脉络膜视网膜炎" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "乙胺嘧啶" } ] }, { "text": "磺胺嘧啶负荷量:75mg/(kg•d),然后以50mg/(kg•d)维持,12小时1次。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "磺胺嘧啶" } ] }, { "text": "四氢叶酸5~20mg,每周3次。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "四氢叶酸" } ] }, { "text": "皮质类固醇1mg/(kg•d),分为2次。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "皮质类固醇" } ] }, { "text": "(五)免疫功能正常的儿童单纯淋巴结肿大者,不需治疗;若重要器官损害,危及生命,治疗同“年长儿活动性脉络膜视网膜炎”,无需肾上腺皮质类固醇,疗程常为4~6周或在体征和症状消退后继续用2周。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "淋巴结肿大" }, { "start_idx": 43, "end_idx": 55, "type": "dis", "entity": "年长儿活动性脉络膜视网膜炎" }, { "start_idx": 60, "end_idx": 67, "type": "dru", "entity": "肾上腺皮质类固醇" } ] }, { "text": "做好人、畜的粪便的管理。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "pro", "entity": "做好人、畜的粪便的管理" } ] }, { "text": "肉、蛋、乳类食物要煮熟,饭前洗手。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "pro", "entity": "肉、蛋、乳类食物要煮熟" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "饭前洗手" } ] }, { "text": "孕妇早期用血清学方法检测抗体,若胎儿已受感染者可终止妊娠。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "抗体" } ] }, { "text": "二、病毒感染性口炎病毒感染性口炎中,疱疹性口炎(herpeticstomatitis)的发病率最高。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "病毒感染性口炎" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "病毒感染性口炎" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "疱疹性口炎" }, { "start_idx": 24, "end_idx": 41, "type": "dis", "entity": "herpeticstomatitis" } ] }, { "text": "【病因】疱疹性口炎又称疱疹性齿龈口炎,由疱疹病毒感染而引起,通过飞沫和接触传染。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "疱疹性口炎" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "疱疹性齿龈口炎" }, { "start_idx": 20, "end_idx": 23, "type": "mic", "entity": "疱疹病毒" } ] }, { "text": "一般不用抗生素,局部可用疱疹净(研细涂之)或中药锡类散等。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "抗生素" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "疱疹净" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "中药锡类散" } ] }, { "text": "进食前为减轻疼痛可用2%利多卡因局部涂之有发热者给予退热剂,患病期间应加强全身支持治疗如给予高维生素高营养流质,或静脉补充营养。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "利多卡因" }, { "start_idx": 30, "end_idx": 54, "type": "pro", "entity": "患病期间应加强全身支持治疗如给予高维生素高营养流质" }, { "start_idx": 57, "end_idx": 62, "type": "pro", "entity": "静脉补充营养" } ] }, { "text": "第十九章进行性肌营养不良进行性肌营养不良(progressivemusculardystrophy)是一组遗传性骨骼肌变性疾病,临床表现为进行性肌无力和肌萎缩,最终完全丧失运动功能。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "进行性肌营养不良" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "进行性肌营养不良" }, { "start_idx": 21, "end_idx": 48, "type": "dis", "entity": "progressivemusculardystrophy" }, { "start_idx": 53, "end_idx": 62, "type": "dis", "entity": "遗传性骨骼肌变性疾病" }, { "start_idx": 69, "end_idx": 74, "type": "sym", "entity": "进行性肌无力" }, { "start_idx": 76, "end_idx": 78, "type": "sym", "entity": "肌萎缩" }, { "start_idx": 80, "end_idx": 89, "type": "sym", "entity": "最终完全丧失运动功能" } ] }, { "text": "肌营养不良主要分为8大类:假肥大型、Emery-DreiFuss型、肢带型、面肩肱型、远端型、强直型、眼咽型和先天性肌营养不良。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肌营养不良" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "假肥大型" }, { "start_idx": 18, "end_idx": 32, "type": "dis", "entity": "Emery-DreiFuss型" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "肢带型" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "面肩肱型" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "远端型" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "强直型" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "眼咽型" }, { "start_idx": 55, "end_idx": 62, "type": "dis", "entity": "先天性肌营养不良" } ] }, { "text": "近年来随着分子生物学研究的不断深入,其中不少类型的基因已经定位,基因产物已经分离,进行基因诊断、基因携带者检出以及产前诊断已成为可能。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "基因" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "基因" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "基因诊断" }, { "start_idx": 48, "end_idx": 54, "type": "pro", "entity": "基因携带者检出" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "产前诊断" } ] }, { "text": "尽管肌营养不良的研究有了很大的进展,但至今本病的主要治疗方法仍是支持和对症治疗,尚无提高患者肌力或者延缓肌无力进展的有效药物。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肌营养不良" }, { "start_idx": 46, "end_idx": 47, "type": "ite", "entity": "肌力" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "肌无力" } ] }, { "text": "第一节假肥大型肌营养不良假肥大型肌营养不良包括DMD(Duchennemusculardystrophy,DMD)和BMD(Beckermusculardystrophy,BMD)两型,是X-染色体隐性遗传的等位基因病。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "假肥大型肌营养不良" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "假肥大型肌营养不良" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "DMD" }, { "start_idx": 27, "end_idx": 51, "type": "dis", "entity": "Duchennemusculardystrophy" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "DMD" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "BMD" }, { "start_idx": 62, "end_idx": 84, "type": "dis", "entity": "Beckermusculardystrophy" }, { "start_idx": 86, "end_idx": 88, "type": "dis", "entity": "BMD" }, { "start_idx": 94, "end_idx": 98, "type": "bod", "entity": "X-染色体" } ] }, { "text": "【发病机制】应用分子生物学方法已将DMD的基因定位于X染色体Xp21.1~Xp21.3,致病基因为dystrophin基因,它是至今发现的最大的人类基因,约2000kb以上,含有79个外显子编码,1个14kb的转录区。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "分子生物学方法" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "DMD" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "基因" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "X染色体" }, { "start_idx": 30, "end_idx": 35, "type": "bod", "entity": "Xp21.1" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "基因" }, { "start_idx": 49, "end_idx": 58, "type": "bod", "entity": "dystrophin" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "基因" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "基因" } ] }, { "text": "研究表明60%~70%的DMD是由于基因缺失或重复突变所致。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "DMD" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "基因" } ] }, { "text": "基因缺失呈非随机性分布,主要发生在基因的中央区(80%),少数发生在5'端(20%)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "基因" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "基因" } ] }, { "text": "大的基因缺失常常开始于基因的5'端,基因缺失造成开放的读码框的破坏,导致DMD表现。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "基因" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "基因" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "基因" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "DMD" } ] }, { "text": "BMD患者,缺失基因保持了翻译读码框,并能产生一个具有一半功能、长度缩短的蛋白质。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "BMD" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "基因" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "翻译读码框" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "蛋白质" } ] }, { "text": "“读码框”假说解释了92%的DMD/BMD患者不同的临床类型。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "DMD" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "BMD" } ] }, { "text": "Dystrophin是dystrophin糖蛋白复合物(DGC)的一部分,DGC是膜相关蛋白的综合体,跨越肌纤维膜,连接细胞内的细胞骨架和细胞外的基质。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "Dystrophin" }, { "start_idx": 11, "end_idx": 26, "type": "bod", "entity": "dystrophin糖蛋白复合物" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "DGC" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "DGC" }, { "start_idx": 41, "end_idx": 49, "type": "bod", "entity": "膜相关蛋白的综合体" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "肌纤维膜" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "细胞" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "细胞骨架" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "细胞" } ] }, { "text": "Duchenne肌营养不良患者,由于dystrophin的缺失导致DGC成分的减少,虽能正常合成但不能正确地装配和整合至肌纤维膜上。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "dis", "entity": "Duchenne肌营养不良" }, { "start_idx": 18, "end_idx": 27, "type": "bod", "entity": "dystrophin" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "DGC" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "肌纤维膜" } ] }, { "text": "由此推测由于DGC的受损,引发一系列连锁反应,导致DMD的肌细胞坏死。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "DGC" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "DMD" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "肌细胞坏死" } ] }, { "text": "Dystrophin的缺乏使肌纤维膜下的细胞骨架和细胞外基质的联系受到破坏,造成肌纤维膜不稳定,膜撕裂,肌细胞坏死。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "Dystrophin" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "肌纤维膜" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "细胞骨架" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "细胞外基质" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "肌纤维膜不稳定" }, { "start_idx": 48, "end_idx": 50, "type": "sym", "entity": "膜撕裂" }, { "start_idx": 52, "end_idx": 56, "type": "sym", "entity": "肌细胞坏死" } ] }, { "text": "【病理改变】各型有不同病理改变,在实验室检查中分别叙述。", "entities": [ { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "实验室检查" } ] }, { "text": "【临床表现】(一)骨骼肌DMD患者儿童期发病,一般在4~6岁时走路易跌,奔跑困难,逐渐出现走路和上楼困难,下蹲站起困难。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "DMD" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "走路易跌" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "奔跑困难" }, { "start_idx": 41, "end_idx": 51, "type": "sym", "entity": "逐渐出现走路和上楼困难" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "下蹲站起困难" } ] }, { "text": "神经系统检查可见四肢肌力低下,肌肉萎缩,腱反射减弱。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "神经系统检查" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "可见四肢肌力低下" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "肌肉萎缩" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "腱反射减弱" } ] }, { "text": "由于骨盆带肌肉无力而呈典型的鸭步,肩带肌肉萎缩无力形成翼状肩或游离肩,腹肌和髂腰肌的萎缩无力形成特征性的Gowers征。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "骨盆带肌肉无力" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "鸭步" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "肩带肌肉萎缩无力" }, { "start_idx": 27, "end_idx": 29, "type": "sym", "entity": "翼状肩" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "游离肩" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "腹肌" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "髂腰肌" }, { "start_idx": 48, "end_idx": 58, "type": "dis", "entity": "特征性的Gowers征" } ] }, { "text": "绝大多数患儿有腓肠肌假性肥大舌肌假性肥大心脏大多数DMD患者无心血管症状,只有在疾病晚期和反复感染的应激情况下才出现心力衰竭和心律失常,很少有明显的充血性心力衰竭。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "腓肠肌" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "腓肠肌假性肥大" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "舌肌" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "舌肌假性肥大" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "心脏" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "DMD" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "心血管" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "心律失常" }, { "start_idx": 74, "end_idx": 80, "type": "sym", "entity": "充血性心力衰竭" } ] }, { "text": "(三)胃肠道胃肠道的平滑肌也可受累。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "胃肠道" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "胃肠道" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "平滑肌" } ] }, { "text": "急性胃扩张可导致死亡,死于此症的患者尸检显示胃的纵行肌外层有退行性改变便秘。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "急性胃扩张" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "尸检" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "胃" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "纵行肌外层" }, { "start_idx": 22, "end_idx": 34, "type": "sym", "entity": "胃的纵行肌外层有退行性改变" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "便秘" } ] }, { "text": "(四)神经系统DMD和BMD患者可有中枢神经系统功能障碍,尤其是智能迟缓,患者平均IQ在正常值的1个标准差以下。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经系统" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "DMD" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "BMD" }, { "start_idx": 18, "end_idx": 27, "type": "dis", "entity": "中枢神经系统功能障碍" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "智能迟缓" }, { "start_idx": 37, "end_idx": 54, "type": "sym", "entity": "患者平均IQ在正常值的1个标准差以下" } ] }, { "text": "智能迟缓的神经病理机制目前尚未明确,是否由于dystrophin蛋白在肌肉和中枢神经系统都有表达有关、尚未肯定。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "智能迟缓" }, { "start_idx": 22, "end_idx": 33, "type": "bod", "entity": "dystrophin蛋白" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肌肉" }, { "start_idx": 38, "end_idx": 43, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "有研究报道患者癫痫的发病率增高,尤其是BMD型。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "癫痫" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "BMD型" } ] }, { "text": "DMD患者易出现情感/行为问题、认知功能下降及学习困难。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "DMD" }, { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "易出现情感/行为问题" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "认知功能下降" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "学习困难" } ] }, { "text": "(五)其他Larson等提出能行走的DMD患者的腰椎骨密度轻度降低,而不能行走的则明显降低。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "DMD" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "腰椎骨密度轻度降低" } ] }, { "text": "资料显示44%的患儿可出现骨折,44%尚能行走的患儿骨折后,就不能再行走。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "骨折" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "骨折" } ] }, { "text": "疾病的自然病程可以鉴别DMD和BMD。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "DMD" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "BMD" } ] }, { "text": "BMD的临床与DMD相似,但发病年龄稍晚,进展缓慢,病情较轻,预后较好,存活时间较长。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "BMD" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "DMD" } ] }, { "text": "【诊断】(一)典型的临床表现和特殊的遗传方式是诊断的基础,但实验室检查是确定本病诊断的依据。", "entities": [ { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "实验室检查" } ] }, { "text": "(二)实验室检查1.血清生化检查肌酸磷酸激酶(CK)明显升高,达15000~20000IU/L,甚至更高。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "血清生化检查" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "肌酸磷酸激酶" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "CK" } ] }, { "text": "血清CK升高可出现于出生时,疾病后期略有降低。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "血清CK升高" } ] }, { "text": "2.肌电图为肌源性改变,病变肌肉呈低电位,波形持续时间缩短,而多相波增高。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肌电图" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "肌源性改变" }, { "start_idx": 12, "end_idx": 19, "type": "sym", "entity": "病变肌肉呈低电位" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "波形持续时间缩短" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "多相波增高" } ] }, { "text": "3.肌肉活体组织检查特征性的病理改变有散在的退行性变和坏死肌纤维。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "肌肉活体组织检查" }, { "start_idx": 10, "end_idx": 31, "type": "sym", "entity": "特征性的病理改变有散在的退行性变和坏死肌纤维" } ] }, { "text": "随着时间的延长,出现肌内膜结缔组织增加以及肌纤维的丧失,脂肪组织的替代。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "肌内膜结缔组织增加" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "肌纤维的丧失" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "脂肪组织的替代" } ] }, { "text": "4.基因诊断DMD基因定位于Xp21.1或21.3,基因编码的蛋白质为dystrophin。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "基因诊断" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "DMD" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "基因" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "Xp21.1" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "基因编码的蛋白质" }, { "start_idx": 35, "end_idx": 44, "type": "dis", "entity": "dystrophin" } ] }, { "text": "【治疗】(一)药物治疗常用的药物有:维生素E、肌苷、三磷酸腺苷以及中药等。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "药物治疗" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "维生素E" }, { "start_idx": 23, "end_idx": 24, "type": "dru", "entity": "肌苷" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "三磷酸腺苷" }, { "start_idx": 33, "end_idx": 34, "type": "dru", "entity": "中药" } ] }, { "text": "此外,用钙拮抗药维拉帕米治疗也有一定效果。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dru", "entity": "钙拮抗药维拉帕米" } ] }, { "text": "(二)支持治疗为保持肌肉功能及预防挛缩,进行适度运动甚为重要,不宜久卧床上。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肌肉" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "挛缩" } ] }, { "text": "对症治疗包括肌肉、关节被动运动和按摩,注意并防止并发症。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肌肉" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "关节" } ] }, { "text": "(三)外科治疗DMD患者常发展为进行性脊柱侧弯,常需行脊柱后融合术。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "外科治疗" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "DMD" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "进行性脊柱侧弯" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "脊柱后融合术" } ] }, { "text": "(四)基因治疗DMD基因治疗从质粒直接注射到应用不同类型的载体组装的DMD基因转染,在动物实验中取得了成功,在动物骨骼肌中dystrophin进行表达。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "基因治疗" }, { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "DMD基因治疗" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "DMD基因" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 61, "end_idx": 70, "type": "bod", "entity": "dystrophin" } ] }, { "text": "加拿大学者开展了对DMD患者骨骼肌注射同种异体正常肌前体细胞的研究,并确认了供体来源的dystrophin表达。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "DMD" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "骨骼肌注射" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "肌前体细胞" }, { "start_idx": 43, "end_idx": 52, "type": "bod", "entity": "dystrophin" } ] }, { "text": "【预后】DMD预后不良,随着疾病的进展,出现关节挛缩,功能受限(尤其是踝、髋关节),脊柱侧弯较多见,一般在12岁左右发展为需依靠轮椅生活。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "DMD" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "关节挛缩" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "功能受限" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "踝" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "髋关节" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "脊柱侧弯较多见" } ] }, { "text": "大多于20岁左右因并发呼吸衰竭或心力衰竭而死亡。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "死亡" } ] }, { "text": "BMD则预后良好,病程较长,通常可活至中年。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "BMD" } ] }, { "text": "二、遗传性球形红细胞增多症遗传性球形红细胞增多症(hereditaryspherocytosis,HS)是红细胞膜缺陷中最常见的一种溶血性贫血。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "遗传性球形红细胞增多症" }, { "start_idx": 13, "end_idx": 23, "type": "dis", "entity": "遗传性球形红细胞增多症" }, { "start_idx": 25, "end_idx": 47, "type": "dis", "entity": "hereditaryspherocytosis" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "HS" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "红细胞" }, { "start_idx": 66, "end_idx": 70, "type": "dis", "entity": "溶血性贫血" } ] }, { "text": "其临床特点是外周血中球形红细胞增加、渗透脆性增加伴有脾大、间隙性黄疸以及不同程度的贫血,常染色体显性遗传,极少数病人为常染色体隐性遗传,多数具有家族史。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "bod", "entity": "外周血中球形红细胞" }, { "start_idx": 6, "end_idx": 16, "type": "sym", "entity": "外周血中球形红细胞增加" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "脾" }, { "start_idx": 18, "end_idx": 27, "type": "sym", "entity": "渗透脆性增加伴有脾大" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "黄疸" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "间隙性黄疸" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "贫血" }, { "start_idx": 36, "end_idx": 42, "type": "sym", "entity": "不同程度的贫血" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "常染色体" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "染色体" } ] }, { "text": "约25%的病例无家族史,其中大多数为新突变引起,极少数为未被发现的隐性遗传,其家族中未能发现球形红细胞和红细胞渗透脆性增高现象。", "entities": [ { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "隐性遗传" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "球形红细胞" }, { "start_idx": 52, "end_idx": 56, "type": "ite", "entity": "红细胞渗透" }, { "start_idx": 46, "end_idx": 60, "type": "sym", "entity": "球形红细胞和红细胞渗透脆性增高" } ] }, { "text": "【缺陷类型】HS的主要分子缺陷是维持红细胞形态的细胞骨架蛋白-收缩蛋白(spectrin,SP)和锚蛋白(ankyrin)异常。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "HS" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "红细胞" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "细胞骨架蛋白" }, { "start_idx": 36, "end_idx": 43, "type": "bod", "entity": "spectrin" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "SP" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "锚蛋白" }, { "start_idx": 53, "end_idx": 59, "type": "bod", "entity": "ankyrin" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "异常" } ] }, { "text": "收缩蛋白缺陷主要与隐性遗传有关,而β收缩蛋白和蛋白3(是与细胞骨架蛋白相互作用的一种蛋白)缺陷则为显性遗传为主。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "收缩蛋白缺陷" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "β收缩蛋白" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "蛋白3" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "细胞骨架蛋白" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "蛋白" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "缺陷" } ] }, { "text": "遗传带有异质性,现已发现以下几种类型:①单独SP缺乏(约占45%),其中75%为β-SP轻度缺陷,其含量约为正常人的63%~81%,属常染色体显性遗传。", "entities": [ { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "单独SP缺乏" }, { "start_idx": 40, "end_idx": 47, "type": "dis", "entity": "β-SP轻度缺陷" } ] }, { "text": "约25%为α-SP明显缺乏,其含量仅为正常人的30%~74%,属常染色体隐性遗传;②锚蛋白缺乏(约占16%):为常染色体显性遗传。", "entities": [ { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "染色体" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "锚蛋白缺乏" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "染色体" } ] }, { "text": "由于锚蛋白缺少,不能连接SP,剩余的SP迅速降解,引起继发性SP减少,造成锚蛋白与SP联合减少;③蛋白3缺乏(约占22%):为常染色体显性遗传;④蛋白4.2缺乏:为常染色体隐性遗传;⑤膜收缩蛋白与蛋白4.1的结合缺陷:为常染色体显性遗传。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "锚蛋白" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "SP" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "SP" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "SP减少" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "锚蛋白" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "SP" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "蛋白3缺乏" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "常染色体" }, { "start_idx": 73, "end_idx": 79, "type": "dis", "entity": "蛋白4.2缺乏" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "常染色体" }, { "start_idx": 92, "end_idx": 107, "type": "dis", "entity": "膜收缩蛋白与蛋白4.1的结合缺陷" }, { "start_idx": 110, "end_idx": 113, "type": "bod", "entity": "常染色体" } ] }, { "text": "【发病机制】上述缺陷可以导致下列病理生理改变:①脂质双层骨架在垂直方向的连接力减弱,使之“脱耦联”(uncoupling),随后红细胞膜以出芽形式形成微囊泡而丢失,而体积减少相对不明显,从而使红细胞表面积缩小,表面积与体积之比下降,变成球形细胞;②膜对阳离子的通透性和转运增加,三磷酸腺苷(ATP)利用增加,糖代谢增强;出现钠离子和水进入胞内,钾离子则透出胞外;钙ATP酶抑制,致使细胞内钙离子浓度增加并沉积在细胞膜上,使红细胞膜的柔韧性及变形能力下降。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "脂质" }, { "start_idx": 45, "end_idx": 47, "type": "sym", "entity": "脱耦联" }, { "start_idx": 50, "end_idx": 59, "type": "sym", "entity": "uncoupling" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "红细胞膜" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "微囊泡" }, { "start_idx": 83, "end_idx": 91, "type": "sym", "entity": "体积减少相对不明显" }, { "start_idx": 96, "end_idx": 98, "type": "bod", "entity": "红细胞" }, { "start_idx": 118, "end_idx": 121, "type": "bod", "entity": "球形细胞" }, { "start_idx": 96, "end_idx": 121, "type": "sym", "entity": "红细胞表面积缩小,表面积与体积之比下降,变成球形细胞" }, { "start_idx": 124, "end_idx": 137, "type": "sym", "entity": "膜对阳离子的通透性和转运增加" }, { "start_idx": 154, "end_idx": 158, "type": "sym", "entity": "糖代谢增强" }, { "start_idx": 160, "end_idx": 170, "type": "sym", "entity": "出现钠离子和水进入胞内" }, { "start_idx": 172, "end_idx": 179, "type": "sym", "entity": "钾离子则透出胞外" }, { "start_idx": 181, "end_idx": 185, "type": "bod", "entity": "钙ATP酶" }, { "start_idx": 191, "end_idx": 192, "type": "bod", "entity": "细胞" }, { "start_idx": 194, "end_idx": 200, "type": "sym", "entity": "钙离子浓度增加" }, { "start_idx": 211, "end_idx": 214, "type": "bod", "entity": "红细胞膜" }, { "start_idx": 211, "end_idx": 225, "type": "sym", "entity": "红细胞膜的柔韧性及变形能力下降" } ] }, { "text": "当这些红细胞通过仅2~3μm的脾脏微血管和脾血窦时,很容易被破坏而发生血管外溶血。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "红细胞" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "脾脏微血管" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "脾血窦" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "血管外溶血" } ] }, { "text": "因此,脾切除能明显延长红细胞的寿命而治愈或减轻贫血。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "脾切除" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "红细胞" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "贫血" } ] }, { "text": "而膜收缩蛋白的缺乏程度与红细胞球形率、溶血程度以及脾切除后的疗效密切相关。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "膜收缩蛋白" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "红细胞" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "球形率" }, { "start_idx": 25, "end_idx": 27, "type": "pro", "entity": "脾切除" } ] }, { "text": "发生于新生儿时,可出现新生儿溶血症,表现为典型的球形红细胞、溶血增加和网织红细胞增高,早期即可出现高胆红素血症,甚至发生核黄疸。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "新生儿溶血症" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "红细胞" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "球形红细胞" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "网织红细胞" }, { "start_idx": 30, "end_idx": 41, "type": "sym", "entity": "溶血增加和网织红细胞增高" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "高胆红素血症" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "核黄疸" } ] }, { "text": "由于骨髓代偿性增生,颅骨髓腔加宽,额骨和颞骨突起,珠蛋白生成障碍性贫血明显。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "颅骨髓腔加宽" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "颞骨" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "额骨和颞骨突起" }, { "start_idx": 25, "end_idx": 34, "type": "dis", "entity": "珠蛋白生成障碍性贫血" } ] }, { "text": "有些轻症病例平时可无症状,但在急性感染、B19微小病毒感染以及其他病毒感染后可诱发急性溶血、再障危象或骨髓增生低下危象。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "急性感染" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "病毒感染" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "急性溶血" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "再障危象" }, { "start_idx": 57, "end_idx": 64, "type": "dis", "entity": "骨髓增生低下危象" } ] }, { "text": "此时贫血加重,血红蛋白可降至20~30g/L,血细胞比容可降至10%以下,此危象可持续2周左右,多数自然缓解。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "贫血" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 23, "end_idx": 27, "type": "ite", "entity": "血细胞比容" } ] }, { "text": "有些病儿在生后6~8月内虽有轻重不等的贫血,间歇或持续性黄疸,但随着年龄的增长骨髓造血代偿功能不断增强,症状逐渐减轻。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "贫血" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "间歇或持续性黄疸" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "骨髓" } ] }, { "text": "脾脏于婴儿期以后逐渐增大,个别轻症病例也可不大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脾脏" } ] }, { "text": "肝脏不大或轻度大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肝脏" } ] }, { "text": "【实验室检查】1.外周血改变贫血多为轻度或中度,血红蛋白多在60~100g/L。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "网织红细胞约在6%~20%(平均10%)左右,溶血危象发作时可增至50%~70%,但在再生障碍危象时网织红细胞减低。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "网织红细胞" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "溶血危象" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "再生障碍危象" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "网织红细胞" } ] }, { "text": "胞体染色深,无中央淡染区,一般占红细胞的25%~42%;约有20%~25%的病例红细胞形态变化不明显。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "胞体染色深" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "无中央淡染区" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "红细胞" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "形态变化不明显" } ] }, { "text": "蛋白3缺乏者可见针刺状球形细胞,收缩蛋白和锚蛋白联合缺乏者可呈不规则形。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "蛋白3缺乏者" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "针刺状球形细胞" } ] }, { "text": "白细胞及血小板正常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "白细胞" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "血小板" } ] }, { "text": "2.骨髓象红细胞系增生极度活跃,以中、晚幼红细胞居多。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "骨髓" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "红细胞" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "晚幼红细胞" } ] }, { "text": "粒∶红细胞比例可倒置,但在再障危象或增生低下危象时,红细胞系增生不良。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "红细胞" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "再障危象" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "增生低下危象" } ] }, { "text": "但如合并叶酸缺乏,则可见巨幼样变。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "叶酸" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "巨幼样变" } ] }, { "text": "3.红细胞渗透脆性试验当红细胞放入低渗氯化钠盐水溶液中时,水即可进入细胞内,使胞体膨胀。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "红细胞渗透脆性试验" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "红细胞" }, { "start_idx": 17, "end_idx": 25, "type": "dru", "entity": "低渗氯化钠盐水溶液" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "细胞" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "胞体膨胀" } ] }, { "text": "正常红细胞呈盘状,可容许一定量的水分进入细胞内而不致破坏,而球形红细胞表面积缩小,少量水分进入细胞内即可导致红细胞破裂。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "红细胞" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "细胞" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "球形红细胞" }, { "start_idx": 30, "end_idx": 39, "type": "sym", "entity": "球形红细胞表面积缩小" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "红细胞" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "红细胞破裂" } ] }, { "text": "约75%的病例红细胞渗透脆性增加。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "红细胞" }, { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "红细胞渗透脆性增加" } ] }, { "text": "一般0.5%~0.70%开始溶血,0.4%时则完全溶血,比正常对照明显增高。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "溶血" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "溶血" } ] }, { "text": "有10%~20%的病人红细胞渗透脆性可正常,但若将红细胞置37℃温箱中孵育24小时后,红细胞渗透脆性明显增加,0.70%~0.80%以上即可出现溶血,阳性率高达100%。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "红细胞" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "红细胞渗透脆性可正常" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "红细胞" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "红细胞" }, { "start_idx": 43, "end_idx": 53, "type": "sym", "entity": "红细胞渗透脆性明显增加" }, { "start_idx": 72, "end_idx": 73, "type": "dis", "entity": "溶血" } ] }, { "text": "4.自身溶血试验对诊断有一定帮助。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "自身溶血试验" } ] }, { "text": "在无菌条件下,将脱纤维蛋白的血标本置37℃温箱中孵育48小时,离心后可见溶血增加。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "脱纤维蛋白" }, { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "血标本" }, { "start_idx": 21, "end_idx": 22, "type": "equ", "entity": "温箱" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "孵育" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "离心" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "溶血" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "溶血增加" } ] }, { "text": "正常人溶血少于5%,HS者可达15%~45%。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "溶血" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "HS" } ] }, { "text": "若于孵育前加入少量葡萄糖或ATP,可使溶血减轻,称为纠正试验阳性。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "ATP" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "溶血减轻" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "纠正试验" } ] }, { "text": "5.酸化甘油试验(AGLT)不同红细胞在酸化甘油中的破裂速度不同,在pH为6.85,离子强度为0.3M的甘油溶液中,红细胞可发生缓慢溶血,光密度随溶血增加而下降。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "酸化甘油试验" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "AGLT" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "红细胞" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "酸化甘油" }, { "start_idx": 52, "end_idx": 53, "type": "dru", "entity": "甘油" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "红细胞" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "缓慢溶血" }, { "start_idx": 73, "end_idx": 74, "type": "dis", "entity": "溶血" } ] }, { "text": "本法较敏感,阳性检测率可达100%。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "阳性检测率" } ] }, { "text": "6.红细胞膜蛋白电泳分析采用十二磺酸钠聚丙烯酰胺凝胶电泳(SDS-PAGE),可对膜蛋白主要成分进行定性或半定量分析,有助于了解膜蛋白的缺陷。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "红细胞膜蛋白" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "电泳分析" }, { "start_idx": 14, "end_idx": 27, "type": "pro", "entity": "十二磺酸钠聚丙烯酰胺凝胶电泳" }, { "start_idx": 29, "end_idx": 36, "type": "pro", "entity": "SDS-PAGE" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "膜蛋白" }, { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "定性" }, { "start_idx": 53, "end_idx": 57, "type": "pro", "entity": "半定量分析" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "膜蛋白" } ] }, { "text": "7.放射免疫法或ELISA法直接测定膜蛋白含量,此法比电泳法更敏感。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "放射免疫法" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "ELISA法" }, { "start_idx": 18, "end_idx": 22, "type": "ite", "entity": "膜蛋白含量" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "电泳法" } ] }, { "text": "9.血清间接胆红素增加,珠蛋白含量下降,尿中尿胆原增加,溶血性贫血,出现不同程度的黄疸以及脾大,血涂片见球形红细胞超过10%,红细胞渗透脆性和孵育后渗透脆性增加,具有阳性家族史,脾切除疗效佳,并排除其他原因引起的球形红细胞增多症者,可确诊本病。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血清" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "胆红素" }, { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "血清间接胆红素增加" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "珠蛋白" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "珠蛋白含量下降" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "尿胆原" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "尿中尿胆原增加" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "黄疸" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "脾" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "脾大" }, { "start_idx": 48, "end_idx": 50, "type": "pro", "entity": "血涂片" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "球形红细胞" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "红细胞" }, { "start_idx": 63, "end_idx": 79, "type": "sym", "entity": "红细胞渗透脆性和孵育后渗透脆性增加" }, { "start_idx": 89, "end_idx": 91, "type": "pro", "entity": "脾切除" }, { "start_idx": 106, "end_idx": 113, "type": "dis", "entity": "球形红细胞增多症" } ] }, { "text": "免疫性溶血性贫血病儿外周血中可出现大量球形红细胞,其中新生儿同族免疫性溶血性贫血,尤其是ABO血型不合存在大量球形红细胞时很像HS。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "免疫性溶血性贫血" }, { "start_idx": 30, "end_idx": 39, "type": "dis", "entity": "同族免疫性溶血性贫血" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "球形红细胞" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "HS" } ] }, { "text": "其他较少见的病因如烧伤、产气荚膜杆菌败血症、肝豆状核变性,也可见球形红细胞增多。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "烧伤" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "产气荚膜杆菌败血症" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "球形红细胞" } ] }, { "text": "【治疗】脾切除是本病的首选疗法,可以预防溶血危象和再生障碍危象的发生,避免出现持续的高胆红素血症和继发胆石症。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "pro", "entity": "脾切除" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "溶血危象" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "再生障碍危象" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "高胆红素血症" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "继发胆石症" } ] }, { "text": "手术后,贫血、网织红细胞增多和未结合胆红素增高现象得以纠正,红细胞寿命恢复或接近正常,临床症状消失。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "贫血" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "网织红细胞" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "胆红素" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "红细胞" } ] }, { "text": "但脾切除不能根治先天性缺陷,手术后红细胞膜的缺陷和球形红细胞依然存在,自体溶血现象虽然减轻,但渗透脆性试验仍不正常。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "pro", "entity": "脾切除" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "先天性缺陷" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "红细胞膜" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "球形红细胞" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "自体溶血" }, { "start_idx": 47, "end_idx": 52, "type": "pro", "entity": "渗透脆性试验" } ] }, { "text": "手术年龄依溶血的轻重而异,轻症或代偿功能好的可推迟,但一般不宜晚于10岁,因10岁以后继发胆石症的可能性逐渐增高。", "entities": [ { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "继发胆石症" } ] }, { "text": "婴儿时期作脾切除者,手术后发生严重感染的危险性较大。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "脾切除" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "严重感染" } ] }, { "text": "若在婴儿期即有反复发作的严重贫血和黄疸,为了避免影响病人的生长发育和早期出现胆石症,可以考虑在3岁以前做脾切除。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "严重贫血" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "黄疸" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "胆石症" }, { "start_idx": 52, "end_idx": 54, "type": "pro", "entity": "脾切除" } ] }, { "text": "但必须采取预防术后发生严重感染的措施。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "严重感染" } ] }, { "text": "由于50%的感染原为肺炎双球菌,故凡5岁以内切脾的,可每月注射长效青霉素,年龄为2~5岁者应间断地注射多价肺炎双球菌疫苗,以降低肺炎双球菌感染的发生率。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "感染" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "肺炎双球菌" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "切脾" }, { "start_idx": 31, "end_idx": 35, "type": "dru", "entity": "长效青霉素" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "肺炎双球菌" }, { "start_idx": 64, "end_idx": 70, "type": "sym", "entity": "肺炎双球菌感染" } ] }, { "text": "脾切除术后可合并白细胞和血小板增多,甚至发生脑和其他组织栓塞,故术后应密切观察血象变化以便及时采取相应措施。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "脾切除" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "白细胞" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "血小板" } ] }, { "text": "手术过程中应注意寻找有无副脾,如不切除副脾,则有复发的可能。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "副脾" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "副脾" } ] }, { "text": "近年来,采用大部分脾栓塞疗法治疗HS有一定近期疗效,对5岁以前患儿应用该疗法,可以避免免疫功能的严重下降。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "脾栓塞疗法" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "HS" } ] }, { "text": "但这部分病例将来是否必要作脾切除尚无定论。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "脾切除" } ] }, { "text": "贫血严重的病人由于骨髓代偿性增生旺盛,叶酸消耗增加,应给叶酸5mg/日,直至脾切除。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "贫血" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "骨髓代偿性" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "叶酸" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "叶酸" }, { "start_idx": 38, "end_idx": 40, "type": "pro", "entity": "脾切除" } ] }, { "text": "第十五节隐睾隐睾,也称睾丸未降或睾丸下降不全,是指睾丸未能按照正常发育程序从腰部腹膜后下降至阴囊。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "节隐睾" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "隐睾" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "睾丸未降" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "睾丸下降不全" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "睾丸" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "腰部腹膜" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "阴囊" } ] }, { "text": "隐睾的睾丸在生长发育中逐渐降低,早产儿的发病率约30%,新生儿为4%,1岁时为0.66%,成年人为0.3%,表明睾丸的下降是一个渐进的过程,在出生后睾丸仍可继续下降。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "隐睾" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "睾丸" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "睾丸" } ] }, { "text": "【睾丸的下降】胚胎睾丸形第12胸椎其位置相当于第12胸椎。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "睾丸" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "胚胎睾丸" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "第12胸椎" } ] }, { "text": "随着胚胎时期脊柱的第12胸椎,第12胸椎向颅侧移行,睾丸丸受相对固定的睾丸引带约束不能随第12胸椎上移,其位置反而靠近腹股沟管内环处。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脊柱" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "第12胸椎" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "颅" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "睾丸" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "睾丸" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "第12胸椎" }, { "start_idx": 59, "end_idx": 65, "type": "bod", "entity": "腹股沟管内环处" } ] }, { "text": "这一过程可能也与苗勒管(副中肾管)抑制物质(Müllerianinhibitsubstance,MIS)调节有关。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "苗勒管" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "副中肾管" }, { "start_idx": 22, "end_idx": 46, "type": "bod", "entity": "Müllerianinhibitsubstance" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "MIS" } ] }, { "text": "随之,睾丸从腹股沟管内环经腹股沟管出外环而进入阴囊,是谓睾丸下降过程。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "睾丸" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "腹股沟管内环" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "阴囊" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "睾丸下降" } ] }, { "text": "1.睾丸引带的牵拉睾丸引带的近端附着于睾丸和附睾,末端主要附着于阴囊底部,另有部分分别附着于耻骨结节、会阴部或股内侧部。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "睾丸" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "睾丸引带" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "睾丸" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "附睾" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "阴囊" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "耻骨结节" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "会阴部" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "股内侧部" } ] }, { "text": "在睾酮的作用下,引带容积增大、肿胀带容积增大、肿胀,精索血管也延长增粗呈曲张状睾丸即沿着引带扩张过的腹股沟管如果睾丸在其下降过程中停留在腹股沟管或内、外环附近下降不全。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "睾酮" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "引带容积增大、肿胀" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "精索血管" }, { "start_idx": 26, "end_idx": 38, "type": "sym", "entity": "精索血管也延长增粗呈曲张状" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "睾丸" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "腹股沟管" }, { "start_idx": 39, "end_idx": 53, "type": "sym", "entity": "睾丸即沿着引带扩张过的腹股沟管" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "睾丸" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "腹股沟管" }, { "start_idx": 54, "end_idx": 78, "type": "sym", "entity": "如果睾丸在其下降过程中停留在腹股沟管或内、外环附近" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "下降不全" } ] }, { "text": "如睾丸沿睾丸引带末端的其他分支下降至耻骨部、会阴部或股部,未降至阴囊底部异位睾丸。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "睾丸" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "睾丸" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "耻骨部" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "会阴部" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "股部" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "阴囊" }, { "start_idx": 0, "end_idx": 35, "type": "sym", "entity": "如睾丸沿睾丸引带末端的其他分支下降至耻骨部、会阴部或股部,未降至阴囊底部" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "异位睾丸" } ] }, { "text": "但睾丸引带的形态结构及功能效应并不明确,人类胚胎中,引带和阴囊仅有微弱的附着,可能不足以支持对睾丸的任何牵拉。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "引带" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "阴囊" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "睾丸" } ] }, { "text": "2.腹内压作用腹内压增高造成睾丸离开腹部进入腹股沟管先天性腹壁肌肉发育不良的综合征,李状腹综合征(Prune-bellysyndrome),常伴有双侧腹腔内隐睾。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "睾丸" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "腹股沟管" }, { "start_idx": 7, "end_idx": 25, "type": "sym", "entity": "腹内压增高造成睾丸离开腹部进入腹股沟管" }, { "start_idx": 26, "end_idx": 40, "type": "dis", "entity": "先天性腹壁肌肉发育不良的综合征" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "李状腹综合征" }, { "start_idx": 49, "end_idx": 67, "type": "dis", "entity": "Prune-bellysyndrome" }, { "start_idx": 73, "end_idx": 79, "type": "dis", "entity": "双侧腹腔内隐睾" } ] }, { "text": "3.内分泌调节内分泌因素在促进睾丸降入阴囊过程中肯定起了重要作用,但其具体的机制尚不明了。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "睾丸" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "阴囊" } ] }, { "text": "【病因】睾丸下降的机制尚未阐明,对隐睾的病因也不太清楚。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "睾丸下降" } ] }, { "text": "1.内分泌失调临床研究表明,下丘脑-垂体-睾丸轴失衡导致隐睾患者睾酮水平低于正常睾酮水平密切相关,睾酮-双氢睾酮与精索和阴囊表面的受体蛋白结合,促使睾丸下降五节隐睾隐睾,也称睾丸未降或睾丸下降不全,是指睾丸未能按照正常发育程序从腰部腹膜后下降至阴囊。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "内分泌失调" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "下丘脑" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "垂体" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "睾丸轴" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "睾酮" }, { "start_idx": 14, "end_idx": 39, "type": "sym", "entity": "下丘脑-垂体-睾丸轴失衡导致隐睾患者睾酮水平低于正常" }, { "start_idx": 40, "end_idx": 43, "type": "ite", "entity": "睾酮水平" }, { "start_idx": 49, "end_idx": 77, "type": "sym", "entity": "睾酮-双氢睾酮与精索和阴囊表面的受体蛋白结合,促使睾丸下降" }, { "start_idx": 82, "end_idx": 83, "type": "dis", "entity": "隐睾" }, { "start_idx": 87, "end_idx": 90, "type": "dis", "entity": "睾丸未降" }, { "start_idx": 92, "end_idx": 97, "type": "dis", "entity": "睾丸下降不全" }, { "start_idx": 101, "end_idx": 123, "type": "sym", "entity": "睾丸未能按照正常发育程序从腰部腹膜后下降至阴囊" } ] }, { "text": "隐睾的发病率在生长发育中逐渐降低,早产儿的发病率约30%,新生儿为4%,1岁时为0.66%,成年人为0.3%,表明睾丸的下降是一个渐进的过程,在出生后睾丸仍可继续下降。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "隐睾" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "睾丸" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "睾丸" } ] }, { "text": "【睾丸的下降】胚胎睾丸形成时,其位置相当于第12胸椎。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "睾丸" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "睾丸" } ] }, { "text": "随着胚胎时期脊柱的迅速发育,第12胸椎向颅侧移行,睾丸受相对固定的睾丸引带约束不能随第12胸椎上移,其位置反而靠近腹股沟管内环处。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "睾丸" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "腹股沟管" } ] }, { "text": "随之,睾丸从腹股沟管内环经腹股沟管出外环而进入阴囊,是谓睾丸下降过程。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "睾丸" } ] }, { "text": "1.睾丸引带的牵拉睾丸引带的近端附着于睾丸和附睾,末端主要附着于阴囊底部,另有部分分别附着于耻骨结节、会阴部或股内侧部。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "睾丸" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "睾丸" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "睾丸" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "附睾" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "阴囊" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "耻骨结节" } ] }, { "text": "在睾酮的作用下,引带容积增大、肿胀,精索血管也延长增粗呈曲张状,睾丸即沿着引带扩张过的腹股沟管进入阴囊底部。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "睾酮" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "精索血管" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "睾丸" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "腹股沟管" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "阴囊底部" } ] }, { "text": "如果睾丸在其下降过程中停留在腹股沟管或内、外环附近,则为下降不全。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "睾丸" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "腹股沟管" } ] }, { "text": "如睾丸沿睾丸引带末端的其他分支下降至耻骨部、会阴部或股部,未降至阴囊底部,则成为异位睾丸。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "睾丸" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "睾丸" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "耻骨部" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "会阴部" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "股部" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "阴囊" } ] }, { "text": "2.腹内压作用腹内压增高造成睾丸离开腹部进入腹股沟管,其证据是一种先天性腹壁肌肉发育不良的综合征,李状腹综合征(Prune-bellysyndrome),常伴有双侧腹腔内隐睾。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "睾丸" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "腹股沟管" }, { "start_idx": 80, "end_idx": 86, "type": "sym", "entity": "双侧腹腔内隐睾" } ] }, { "text": "3.内分泌调节内分泌因素在促进睾丸降入阴囊过程中肯定起了重要作用,但其具体的机制尚不明了。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "睾丸" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "阴囊" } ] }, { "text": "【病因】睾丸下降的机制尚未阐明,对隐睾的病因也不太清楚。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "睾丸" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "隐睾" } ] }, { "text": "1.内分泌失调临床研究表明,下丘脑-垂体-睾丸轴失衡导致隐睾患者睾酮水平低于正常。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "下丘脑" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "垂体" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "睾丸轴" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "睾酮" } ] }, { "text": "睾丸下降过程与睾酮水平密切相关,睾酮-双氢睾酮与精索和阴囊表面的受体蛋白单侧隐睾?", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "睾丸" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "睾酮" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "睾酮" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "精索" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "阴囊" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "受体蛋白" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "单侧隐睾" } ] }, { "text": "2.副中肾管抑制物质(MIS)不足胚胎初期,胎儿同时具有副中肾管和中肾管。", "entities": [ { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "副中肾管" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "中肾管" } ] }, { "text": "随着男性胎儿原始性腺发育为睾丸,睾丸内间质细胞分泌睾酮,支持细胞分泌副中肾管抑制物(MIS),抑制副中肾管发育。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "原始性腺" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "睾丸" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "睾丸" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "间质细胞" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "睾酮" }, { "start_idx": 34, "end_idx": 40, "type": "bod", "entity": "副中肾管抑制物" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "MIS" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "副中肾管" } ] }, { "text": "如果MIS不足,副中肾管可残留或完全没有退化,对睾丸的下降造成障碍。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "副中肾管" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "睾丸" } ] }, { "text": "3.解剖障碍隐睾者鞘状突多终止于耻骨结节或阴囊上方,而异常的引带残余及筋膜覆盖阴囊入口,这些都可阻止睾丸下降隐睾睾丸常有不同程度的发育不全,体积明显小于健侧,质地松软,有时还有附睾和输精管发育畸形,发生率约为36%~79%。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "耻骨结节" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "阴囊" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "筋膜" }, { "start_idx": 6, "end_idx": 53, "type": "sym", "entity": "隐睾者鞘状突多终止于耻骨结节或阴囊上方,而异常的引带残余及筋膜覆盖阴囊入口,这些都可阻止睾丸下降" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "隐睾睾丸" }, { "start_idx": 58, "end_idx": 82, "type": "sym", "entity": "常有不同程度的发育不全,体积明显小于健侧,质地松软" }, { "start_idx": 84, "end_idx": 110, "type": "sym", "entity": "有时还有附睾和输精管发育畸形,发生率约为36%~79%" } ] }, { "text": "隐睾的病理组织学主要表现为生殖细胞发育的障碍,其次是间质细胞数量亦有减少。", "entities": [ { "start_idx": 0, "end_idx": 21, "type": "sym", "entity": "隐睾的病理组织学主要表现为生殖细胞发育的障碍" }, { "start_idx": 23, "end_idx": 35, "type": "sym", "entity": "其次是间质细胞数量亦有减少" } ] }, { "text": "隐睾的曲细精管平均直径较正常者小,曲细精管隐睾的病理组织学改变随年龄增大而愈加明显,很多研究认为,2岁以后睾丸的组织病理学改变将难以恢复,故手术应在2岁以前完成。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "曲细精管" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "曲细精管" }, { "start_idx": 0, "end_idx": 20, "type": "sym", "entity": "隐睾的曲细精管平均直径较正常者小,曲细精管" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "隐睾" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "睾丸" } ] }, { "text": "成人的隐睾,其曲细精管退行性变,几乎看不到正常精子。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "隐睾" }, { "start_idx": 6, "end_idx": 24, "type": "sym", "entity": "其曲细精管退行性变,几乎看不到正常精子" } ] }, { "text": "病理学改变的程度也和隐睾位置有关,位置越高,病理损害越严重,越接近阴囊部位,病理损害就越轻微。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "阴囊" } ] }, { "text": "【隐睾并发症】隐睾可发生于单侧或双侧,以单侧较为多见。", "entities": [ { "start_idx": 7, "end_idx": 25, "type": "sym", "entity": "隐睾可发生于单侧或双侧,以单侧较为多见" } ] }, { "text": "单侧隐睾者,右侧的发生率略高于左侧。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "单侧隐睾者,右侧的发生率略高于左侧" } ] }, { "text": "但即使是双侧隐睾,仍有适量的雄激素产生,可维持男性第二性征的发育,也很少影响成年后的性行为。", "entities": [ { "start_idx": 0, "end_idx": 44, "type": "sym", "entity": "但即使是双侧隐睾,仍有适量的雄激素产生,可维持男性第二性征的发育,也很少影响成年后的性行为" } ] }, { "text": "1.生育能力下降或不育隐睾的病理主要是生殖细胞发育障碍,因此影响生育能力。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "隐睾" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "生殖细胞发育障碍" } ] }, { "text": "一般而言,双侧隐睾者生育能力明显下降,但如隐睾位置较低,可望留有部分生育能力。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "双侧隐睾者" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "生育能力明显下降" }, { "start_idx": 21, "end_idx": 37, "type": "sym", "entity": "隐睾位置较低,可望留有部分生育能力" } ] }, { "text": "单侧隐睾的生育能力,如对侧降入阴囊的睾丸与附睾发育正常,则影响不大。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "阴囊" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "睾丸" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "附睾" }, { "start_idx": 0, "end_idx": 32, "type": "sym", "entity": "单侧隐睾的生育能力,如对侧降入阴囊的睾丸与附睾发育正常,则影响不大" } ] }, { "text": "2.鞘状突未闭隐睾的鞘状突基本都未闭,有肠管疝入则形成斜疝,可发生嵌顿,手术要同时结扎鞘状突。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "隐睾" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肠管" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "斜疝" }, { "start_idx": 41, "end_idx": 45, "type": "pro", "entity": "结扎鞘状突" } ] }, { "text": "3.睾丸损伤由于睾丸处在腹股沟管内或耻骨结节附近,比较表浅,容易受到外力的直接损伤。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "睾丸" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "睾丸" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "腹股沟管" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "耻骨结节" } ] }, { "text": "4.隐睾扭转未降睾丸发生扭转的几率较阴囊内睾丸高20~50倍隐睾扭转一般表现为腹股沟部疼痛性肿块颇似腹股沟疝嵌顿但无明显胃肠道症状右侧腹内隐睾扭转,其症状与体征颇似急性阑尾炎。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "隐睾扭转" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "阴囊" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "睾丸" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "腹股沟部" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "腹股" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "胃肠道" } ] }, { "text": "5.隐睾恶变隐睾恶变的几率比正常睾丸高18~40倍。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "隐睾" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "隐睾恶变" }, { "start_idx": 11, "end_idx": 24, "type": "sym", "entity": "几率比正常睾丸高18~40倍" } ] }, { "text": "高位隐睾,特别是腹内隐睾,其恶变发生率比低位隐睾高6倍。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "高位隐睾" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "腹内隐睾" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "恶变发生率比低位隐睾高6倍" } ] }, { "text": "6岁以前行睾丸固定术而后发生恶变的,比7岁以后手术者低得多。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "睾丸固定术" } ] }, { "text": "隐睾恶变的发病年龄多在30岁之后。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "隐睾恶变" }, { "start_idx": 5, "end_idx": 15, "type": "sym", "entity": "发病年龄多在30岁之后" } ] }, { "text": "【诊断】诊断并不困难,体检可见患侧阴囊扁平,双侧者阴囊发育较差。", "entities": [ { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "体检可见患侧阴囊扁平" }, { "start_idx": 22, "end_idx": 30, "type": "sym", "entity": "双侧者阴囊发育较差" } ] }, { "text": "触诊时,患儿阴囊空虚,无睾丸。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "患儿阴囊空虚" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "无睾丸" } ] }, { "text": "大约80%的隐睾可在腹股沟管区扪及,压之有胀痛感,活动度大,不能被推入阴囊。", "entities": [ { "start_idx": 0, "end_idx": 36, "type": "sym", "entity": "大约80%的隐睾可在腹股沟管区扪及,压之有胀痛感,活动度大,不能被推入阴囊" } ] }, { "text": "小儿因提睾肌反射比较活跃,受到某些刺激,如寒冷或惊吓后,提睾肌收缩,可将本来位于阴囊内的睾丸提至阴囊近端,甚至进入腹股沟管内,临床表现颇似隐睾。", "entities": [ { "start_idx": 0, "end_idx": 32, "type": "sym", "entity": "小儿因提睾肌反射比较活跃,受到某些刺激,如寒冷或惊吓后,提睾肌收缩" }, { "start_idx": 34, "end_idx": 61, "type": "sym", "entity": "可将本来位于阴囊内的睾丸提至阴囊近端,甚至进入腹股沟管内" } ] }, { "text": "另有些睾丸可从腹股沟部被逐渐地推入阴囊,但松手后,睾丸即上缩回原来位置,应属于隐睾。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "睾丸" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "腹股沟部" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "隐睾" } ] }, { "text": "体检应注意仔细探查股部、耻骨部和会阴部,以除外异位睾丸。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "股部" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "耻骨部" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "会阴部" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "异位睾丸" } ] }, { "text": "约20%的隐睾在触诊时难以触及,但这并不意味着这些隐睾都位于腹内。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "约20%的隐睾在触诊时难以触及" } ] }, { "text": "触不到的隐睾在手术中,约80%可在腹股沟管内或内环附近被发现,而其余的20%经手术探查,仍未能发现。", "entities": [ { "start_idx": 0, "end_idx": 48, "type": "sym", "entity": "触不到的隐睾在手术中,约80%可在腹股沟管内或内环附近被发现,而其余的20%经手术探查,仍未能发现" } ] }, { "text": "如为一侧找不到睾丸,称为单睾或单侧睾丸缺如,发生率约占隐睾手术探查的3%~5%,约5000个男性中有1例单侧睾丸缺如;如双侧隐睾经探查,均未能发现睾丸,称为无睾畸形,约20000个男性中仅有1例。", "entities": [ { "start_idx": 22, "end_idx": 57, "type": "sym", "entity": "发生率约占隐睾手术探查的3%~5%,约5000个男性中有1例单侧睾丸缺如" }, { "start_idx": 59, "end_idx": 74, "type": "sym", "entity": "如双侧隐睾经探查,均未能发现睾丸" }, { "start_idx": 83, "end_idx": 96, "type": "sym", "entity": "约20000个男性中仅有1例" } ] }, { "text": "对于不能触及的隐睾,至今尚无满意的方法可于术前来判断睾丸的存在与否及隐睾所处的位置。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "隐睾" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "睾丸" } ] }, { "text": "超声、CT及磁共振检查,由于肠道气体的影响往往难以获得满意的结果。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "超声" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "CT" }, { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "磁共振检查" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肠道" } ] }, { "text": "双侧触不到睾丸者,视外生殖器情况要做染色体检查,并可通过HCG激发试验来初步判断睾丸的存在与否,但手术探查是唯一可靠的办法。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "染色体检查" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "HCG激发试验" } ] }, { "text": "【治疗】隐睾常在新生儿体检时发现,一经诊断,即应随访。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "隐睾" } ] }, { "text": "生后6个月,如睾丸仍未下降,则自行下降的机会已经极少。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "睾丸" } ] }, { "text": "1.激素治疗隐睾的发生可能与内分泌失调有关,激素用于治疗隐睾受到普通重视。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "激素治疗" }, { "start_idx": 22, "end_idx": 23, "type": "dru", "entity": "激素" } ] }, { "text": "绒毛膜促性腺激素主要成分是黄体生成激素(LH),LH刺激间质细胞,产生睾酮。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dru", "entity": "绒毛膜促性腺激素" }, { "start_idx": 13, "end_idx": 18, "type": "dru", "entity": "黄体生成激素" }, { "start_idx": 20, "end_idx": 21, "type": "dru", "entity": "LH" }, { "start_idx": 24, "end_idx": 31, "type": "bod", "entity": "LH刺激间质细胞" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "睾酮" } ] }, { "text": "应用时可出现阴茎增大及睾丸胀痛,如果剂量掌握不当,或较长期使用,可导致骨骺早期愈合。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "sym", "entity": "出现阴茎增大及睾丸胀痛" }, { "start_idx": 16, "end_idx": 40, "type": "sym", "entity": "如果剂量掌握不当,或较长期使用,可导致骨骺早期愈合" } ] }, { "text": "目前已有报道提出,在1~3岁的小儿中使用HCG反而引起睾丸发育不良。", "entities": [ { "start_idx": 9, "end_idx": 32, "type": "sym", "entity": "在1~3岁的小儿中使用HCG反而引起睾丸发育不良" } ] }, { "text": "我们一般使用一个疗程,总计量5000~10000单位,分10次,间隔1~3天,注射完成。", "entities": [ { "start_idx": 2, "end_idx": 42, "type": "pro", "entity": "一般使用一个疗程,总计量5000~10000单位,分10次,间隔1~3天,注射完成" } ] }, { "text": "从效果来看,并不如文献报道的那样好,尤其是睾丸完全降入阴囊的病例很少。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "睾丸" } ] }, { "text": "LHRH作用于垂体前叶,促使垂体释放LH和FSH,被释放的LH发挥与HCG相同的作用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "LHRH" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "垂体前叶" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "垂体" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "LH" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "FSH" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "LH" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "HCG" } ] }, { "text": "LHRH的优点是已可采用鼻黏膜喷雾给药,每侧鼻孔200μg,每天3次,每天总量1.2mg,连续28天,鼻LHRH治疗药无任何痛苦,即使感冒流涕仍可继续治疗。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "LHRH" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "鼻黏膜" }, { "start_idx": 20, "end_idx": 49, "type": "pro", "entity": "每侧鼻孔200μg,每天3次,每天总量1.2mg,连续28天" }, { "start_idx": 52, "end_idx": 57, "type": "pro", "entity": "LHRH治疗" } ] }, { "text": "对经术前应用LHRH治疗,睾丸未能下降的隐睾进行活检,结果显示其组织学表现较之未接受激素治疗者有明显改善。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "睾丸" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "隐睾" } ] }, { "text": "总之,激素治疗隐睾,由于目前对隐睾尚无统一的分类,疗效也缺乏统一客观的评价标准,各报告者之间有很大差异。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "隐睾" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "隐睾" } ] }, { "text": "腹内隐睾的激素治疗几乎无效。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "隐睾" } ] }, { "text": "我们也碰到HCG注射时睾丸下降,注射一结束,睾丸又回缩的情况。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "HCG注射" } ] }, { "text": "2.手术治疗隐睾经确诊后均可接受手术治疗,手术应在2岁之前进行,为睾丸下降固定术。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "隐睾" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "手术治疗" }, { "start_idx": 33, "end_idx": 39, "type": "pro", "entity": "睾丸下降固定术" } ] }, { "text": "一般可在腹股沟管及其附近找到睾丸,在结扎鞘状突后充分游离精索,使其能无张力地降入阴囊,固定于阴囊肉膜外。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "腹股沟管" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "睾丸" }, { "start_idx": 18, "end_idx": 29, "type": "pro", "entity": "结扎鞘状突后充分游离精索" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "阴囊" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "阴囊肉膜" } ] }, { "text": "如在腹股沟管附近未能发现睾丸,可切开鞘状突,于腹膜腔内探查。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "腹股沟管" } ] }, { "text": "或于内环部切开腹内斜肌和腹横肌2~3cm,于腹膜后去探查输精管和睾丸。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "腹横肌" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "腹膜" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "输精管" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "睾丸" } ] }, { "text": "如经上述步骤仍未发现睾丸,在做出无睾的诊断前一定要明确看到精索,并其末端为盲端。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "睾丸" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "精索" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "盲端" } ] }, { "text": "广泛游离精索时,要保护好睾丸动、静脉,以避免睾丸缺血萎缩。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "精索" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "睾丸动、静脉" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "睾丸" } ] }, { "text": "如果对输精管周围组织做过多剥离,则可能引起输精管蠕动障碍而失去输送精子的能力。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "输精管" }, { "start_idx": 21, "end_idx": 37, "type": "sym", "entity": "输精管蠕动障碍而失去输送精子的能力" } ] }, { "text": "精索经广泛游离后,几乎都能将睾丸无张力地置入阴囊。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "精索" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "睾丸" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "阴囊" } ] }, { "text": "少数病例虽经广泛游离,精索长度仍不足以将睾丸无张力放入阴囊,可先将睾丸固定于当前所能达到的最低位置,半年至一年后再次手术,期间可应用HCG,绝大多数病例可将睾丸放入阴囊。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "精索" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "睾丸" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "阴囊" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "睾丸" }, { "start_idx": 66, "end_idx": 68, "type": "dru", "entity": "HCG" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "睾丸" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "阴囊" } ] }, { "text": "另一可考虑的手术是Fowler-Stephens术,在尽可能高位切断精索血管,使高位隐睾能一次性降入阴囊,但保留输精管与精索血管间系膜样结构,通过侧支血供来供应睾丸隐睾,在腹股沟管内未能找到睾丸,但如发现有精索盲端,则提示已无睾丸,不必再作广泛探查。", "entities": [ { "start_idx": 9, "end_idx": 24, "type": "pro", "entity": "Fowler-Stephens术" }, { "start_idx": 27, "end_idx": 51, "type": "pro", "entity": "尽可能高位切断精索血管,使高位隐睾能一次性降入阴囊" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "输精管" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "精索血管" }, { "start_idx": 82, "end_idx": 83, "type": "dis", "entity": "隐睾" }, { "start_idx": 86, "end_idx": 89, "type": "bod", "entity": "腹股沟管" }, { "start_idx": 95, "end_idx": 96, "type": "bod", "entity": "睾丸" }, { "start_idx": 103, "end_idx": 106, "type": "bod", "entity": "精索盲端" }, { "start_idx": 113, "end_idx": 114, "type": "bod", "entity": "睾丸" } ] }, { "text": "如果只发现盲端输精管或附睾,应考虑输精管、附睾可能与睾丸完全分离,必须继续在腹膜后探查,直至睾丸原始发育的部位。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "盲端输精管" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "附睾" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "输精管" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "附睾" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "睾丸" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "腹膜" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "睾丸" } ] }, { "text": "睾丸原始发育虽为腹膜后器官,但不少高位隐睾在腹膜腔内,精索周围常有腹膜包裹,形成系膜,在探查时应加以注意。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "睾丸" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "腹膜" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "腹膜腔" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "精索" } ] }, { "text": "三、心脏内部结构的演变一个简单的心管发育成为一个复杂的心脏。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "心管" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "心脏" } ] }, { "text": "这一过程体现了由低等动物的心脏到高等动物心脏的种系发展过程。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心脏" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "心脏" } ] }, { "text": "心脏胚胎成分与解剖结构关系见表9-9。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "心脏胚胎" } ] }, { "text": "表9-1心脏胚胎成分与解剖结构关系(一)房间隔的形成当胚胎第四周时,心房身体正中线背侧,右肺静脉的右侧脊向房室交界处延伸成为第一房间隔(原发隔,Ⅰ),将心房分为左右两半。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "心脏胚胎" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "房间隔" }, { "start_idx": 34, "end_idx": 42, "type": "bod", "entity": "心房身体正中线背侧" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "右肺静脉" }, { "start_idx": 49, "end_idx": 57, "type": "bod", "entity": "右侧脊向房室交界处" }, { "start_idx": 62, "end_idx": 66, "type": "bod", "entity": "第一房间隔" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "原发隔" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "心房" } ] }, { "text": "第一房间隔近内膜处留一镰状孔道即为第一房室孔(原发孔),胚胎在心房水平的血流可由此自右向左通过。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "第一房间隔" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "内膜" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "第一房室孔" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "原发孔" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "心房" } ] }, { "text": "第一孔因第一隔下缘与心内膜垫融合而逐渐闭合,与此同时第一隔的后上方(头端)局部组织逐渐被吸收成为筛孔状孔道,此为第二孔,或继发孔。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "第一孔" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "第一隔" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "心内膜垫" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "第一隔" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "第二孔" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "继发孔" } ] }, { "text": "约在胚胎第7周时,于第一隔的右侧又发生一片状隔膜,此为第二隔(继发隔,Ⅱ)。", "entities": [ { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "第二隔" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "继发隔" } ] }, { "text": "第二隔朝向房室管方向生长,但中途停止,因此与房室管间留有卵圆形通道,此为卵圆孔。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "第二隔" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "房室管" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "房室管" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "卵圆孔" } ] }, { "text": "此时第一隔上的第二孔为第二隔所遮盖,第二隔上的卵圆孔则为第一隔所遮盖,此时卵圆孔成为卵圆窝。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "第一隔" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "第二孔" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "第二隔" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "第二隔" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "第一隔" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "卵圆窝" } ] }, { "text": "胚胎时肺组织未膨胀,肺循环阻力大,右心房压力高于左心房压力。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肺组织" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "右心房" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "左心房" } ] }, { "text": "右心房血流可冲开卵圆窝底部的第一隔,斜经第二孔入左心房。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心房" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "卵圆窝" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "第一隔" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "第二孔" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "左心房" } ] }, { "text": "而出生后两肺开始呼吸,肺循环开放,肺动脉压力下降,右心房压力低于左心房,第一隔受左心房压力作用紧贴于卵圆孔上,左右心房间的分流现象即告终止,此即卵圆孔的功能性闭合。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "两肺" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肺动脉" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "右心房" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "左心房" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "第一隔" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "左心房" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "左右心房" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "卵圆孔" } ] }, { "text": "卵圆孔的功能性闭合早于解剖闭合,胎儿出生后4~12月内解剖闭合才逐渐形成,而成年人中尚有25%~30%未实现解剖闭合,仍可探测到存在自左向右的血流通过,而此种情况不作房间隔缺损论。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 83, "end_idx": 85, "type": "bod", "entity": "房间隔" } ] }, { "text": "房间隔的发育异常:第一孔未及时闭合即为房间隔缺损的原发孔型,如第二孔未闭则为房间隔缺损的继发孔型。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "房间隔" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "第一孔" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "房间隔" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "第二孔" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "房间隔" } ] }, { "text": "房间孔形成时,孔中有残余组织未被吸收,在房间孔上形成网状结构,此即Charis网。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "房间孔" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "组织" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "房间孔" }, { "start_idx": 33, "end_idx": 39, "type": "bod", "entity": "Charis网" } ] }, { "text": "部分房间隔缺损的病例中可有Charis网的存在。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "房间隔" }, { "start_idx": 13, "end_idx": 19, "type": "bod", "entity": "Charis网" } ] }, { "text": "覆盖卵圆窝的第一房间隔组织松弛,呈袋囊状向右房膨出,此一现象称为房隔瘤。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "卵圆窝" }, { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "第一房间隔组织" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "右房" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "呈袋囊状向右房膨出" }, { "start_idx": 32, "end_idx": 34, "type": "sym", "entity": "房隔瘤" } ] }, { "text": "(二)心内膜垫的胚胎发育原始心管的心室和心房两个膨大之间为一凹陷的共同房室环,房室环中有三层组织所构成,其外层为心外膜,中间为心肌层,内层为心内膜。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "心内膜垫" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心管" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "心室" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "心房" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "房室环" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "房室环" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "心外膜" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "心肌层" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "心内膜" } ] }, { "text": "共同房室环构成房室共同管。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "房室环" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "房室共同" } ] }, { "text": "胚胎第4周末(28天左右)房室共同管的心内膜增生,形成突起形成心内膜垫。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "共同管" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "心内膜" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "心内膜垫" } ] }, { "text": "此时心内膜与心外膜直接融合。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "心内膜" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "心外膜" } ] }, { "text": "共同房室管内共有6个内膜垫:背侧左、右心内膜垫,腹侧左、右心内膜垫,左侧内膜垫与右侧内膜垫。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "共同房室管" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "内膜垫" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "右心内膜垫" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "右心内膜垫" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "右侧内膜垫" } ] }, { "text": "1.心内膜垫与房室孔、房室分隔的形成胚胎6周时,房室环的背侧内膜垫与腹侧内膜垫相互融合成为房室环的中间间隔而出现房室分隔,同时将房室管分为左右两半形成左、右房室孔。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "心内膜垫" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "房室孔" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "房室" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "房室环" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "内膜垫" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "内膜垫" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "房室环" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "房室管" }, { "start_idx": 75, "end_idx": 80, "type": "bod", "entity": "左、右房室孔" } ] }, { "text": "两者的内界为中间间隔,外界为房室环的左右侧壁。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "bod", "entity": "房室环的左右侧壁" } ] }, { "text": "2.心内膜垫与房室瓣的形成三尖瓣主要由右侧的腹、背侧及右侧三个心内膜垫及圆锥隔所组成。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "心内膜垫" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "房室瓣" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "心内膜垫" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "圆锥隔" } ] }, { "text": "其中隔瓣由右腹、背侧心内膜垫及右侧心内膜垫底一部分组成;后瓣由右侧心内膜垫组成;前瓣则大部分由圆锥隔组成。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "隔瓣" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "心内膜垫" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "心内膜垫底" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "后瓣" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "心内膜垫" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "前瓣" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "圆锥隔" } ] }, { "text": "二尖瓣均由左侧的三个内膜垫所组成。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "内膜垫" } ] }, { "text": "前瓣的前外部与后内部分别由腹侧垫和背侧垫所组成;后瓣由背侧垫组成;而后内侧联合及前外联合分别由左侧垫与背侧垫、腹侧垫共同组成。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "前瓣" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "腹侧垫" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "背侧垫" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "后瓣" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "背侧垫" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "左侧垫" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "背侧垫" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "腹侧垫" } ] }, { "text": "当左侧的腹背内膜垫融合不全可出现二尖瓣前叶裂,而当腹侧和背侧的左右心内膜垫相互融合时即形成共同房室瓣。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "内膜垫" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "二尖瓣前叶裂" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "心内膜垫" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "房室瓣" } ] }, { "text": "3.室间隔的发育胚胎第4周心房内原发隔形成时,心室底部突出肌性的室间隔基胚并向房室管方向生长,形成室间隔的小梁部,使心室分成左右两半,但在其上缘与房室管下缘间形成半月形的室间孔,室间孔周围组织向心性生长形成室间隔光滑部。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "室间隔" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心房" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "原发隔" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心室" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "室间隔" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "房室管" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "室间隔" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "心室" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "房室管" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "室间孔" }, { "start_idx": 89, "end_idx": 91, "type": "bod", "entity": "室间孔" }, { "start_idx": 103, "end_idx": 108, "type": "bod", "entity": "室间隔光滑部" } ] }, { "text": "至胚胎第7周时室间隔上缘的结缔组织、漏斗部及心内膜垫融合成膜部室间隔使室间孔完全闭合,室间隔分隔完成。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "结缔组织" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "漏斗部" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "心内膜垫" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "膜部室间隔" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "室间孔" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "室间隔" } ] }, { "text": "室间隔发育过程中任何部分出现异常即可出现室间隔缺损,其中以室间隔膜周部缺损最常见。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "室间隔" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "室间隔" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "室间隔膜" } ] }, { "text": "4.漏斗部的形成胚胎第3周时,心球膨大向右扭转并向尾端移行,至胚胎第4周时心球与心室均移至心房的尾端,心球此时分成头端与尾端两个部分,尾端发育成右心室的流入道,头端发育成流出道或称漏斗部。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "漏斗部" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心球" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "心球" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "心室" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "心房" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "心球" }, { "start_idx": 72, "end_idx": 78, "type": "bod", "entity": "右心室的流入道" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "流出道" }, { "start_idx": 89, "end_idx": 92, "type": "bod", "entity": "称漏斗部" } ] }, { "text": "此时的漏斗部为左右心室的共同流出道。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "漏斗部" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "左右心室" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "共同流出道" } ] }, { "text": "此后,在心球头尾端之间的腹背侧分别向心形成突起成为腹侧及背侧心球嵴并逐渐相互融合,最后与心内膜垫及心室间隔融合而将漏斗部分成左右两半,其中右侧发育成肺动脉漏斗部,与右心室相通;左侧发育成主动脉漏斗部,与左心室相通,但此后主动脉漏斗部被逐渐吸收,正常情况下不形成主动脉下圆锥。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "心球" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "心球嵴" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "心内膜垫" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "心室间隔" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "漏斗" }, { "start_idx": 74, "end_idx": 79, "type": "bod", "entity": "肺动脉漏斗部" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "右心室" }, { "start_idx": 93, "end_idx": 98, "type": "bod", "entity": "主动脉漏斗部" }, { "start_idx": 101, "end_idx": 103, "type": "bod", "entity": "左心室" }, { "start_idx": 110, "end_idx": 115, "type": "bod", "entity": "主动脉漏斗部" }, { "start_idx": 130, "end_idx": 132, "type": "bod", "entity": "主动脉" } ] }, { "text": "如果肺动脉及主动脉漏斗部出现吸收异常,可出现双漏斗部及漏斗部缺如畸形。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺动脉" }, { "start_idx": 5, "end_idx": 11, "type": "bod", "entity": "及主动脉漏斗部" } ] }, { "text": "5.动脉干的分隔动脉干位于心球的头端,在胚胎发育第5~7周时动脉干内膜左右两侧各发生一纵嵴,分别为Ⅰ嵴和Ⅲ嵴,分别与漏斗部的腹侧、背侧心球嵴相连。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "动脉干" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "动脉干" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心球" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "动脉干内膜" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "纵嵴" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "Ⅰ嵴" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "Ⅲ嵴" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "漏斗部" }, { "start_idx": 65, "end_idx": 69, "type": "bod", "entity": "背侧心球嵴" } ] }, { "text": "Ⅰ嵴和Ⅲ嵴相互融合形成动脉干的纵隔即主肺动脉隔,将动脉干分成前后两部分,前侧为肺动脉,后侧为主动脉。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "Ⅰ嵴" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "Ⅲ嵴" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "动脉干" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "纵隔" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "肺动脉隔" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "动脉干" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "肺动脉" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "主动脉" } ] }, { "text": "6.心球纵隔、动脉干纵隔的扭转心球纵隔、动脉干纵隔融合后,心球纵隔作顺时针180°的扭转,然后动脉干纵隔同样顺时针方向扭转,最终使两者呈45°的旋转关系,因而肺动脉自右前方转向左后方,主动脉由左后方转向右前方。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "心球纵隔" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "动脉干纵隔" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "心球纵隔" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "动脉干纵隔" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "心球纵隔" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "动脉干纵隔" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "肺动脉" }, { "start_idx": 92, "end_idx": 94, "type": "bod", "entity": "主动脉" } ] }, { "text": "心球、动脉干隔的发育异常可分为三种类型:①分隔不均畸形:纵隔偏右可造成右侧漏斗部狭窄,反之可造成主动脉下狭窄。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心球" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "动脉干隔" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "分隔" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "纵隔" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "右侧漏斗部" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "主动脉" } ] }, { "text": "②扭转不良畸形:动脉干纵隔扭转不全可导致主肺动脉异位性畸形(大动脉转位)。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "动脉干纵隔" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "主肺动脉" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "大动脉" } ] }, { "text": "③分隔不全畸形:动脉干纵隔发育不全可形成主肺动脉隔缺损;动脉干纵隔缺如即形成永存动脉干畸形。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "分隔" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "动脉干纵隔" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "主肺动脉隔" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "动脉干纵隔" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "动脉干" } ] }, { "text": "7.半月瓣的形成在胚胎5~7周时,动脉干内部分隔同时其表面发生两条相应的纵沟,使肺动脉及主动脉完全分离。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "半月瓣" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "动脉干" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "隔" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "肺动脉" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "主动脉" } ] }, { "text": "纵沟的形成使动脉干纵嵴一分为二,主动脉及肺动脉各得一半,随后头端大部分消失,留下的近心端(位于动脉干与心球交界水平)逐渐各自分化为三个半月瓣。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "纵沟" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "动脉干纵嵴" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "主动脉" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肺动脉" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "近心端" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "动脉干" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "心球" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "半月瓣" } ] }, { "text": "8.静脉窦的演变胚胎早期静脉窦分为左右两个,相互对称,一端与原始心房相连,另一端则各自连接三个左右对称的静脉:总主静脉、脐静脉及卵黄静脉。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "静脉窦" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "静脉窦" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "原始心房" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "静脉" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "总主静脉" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "脐静脉" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "卵黄静脉" } ] }, { "text": "以后两侧的脐静脉及左卵黄静脉退化,在胚胎发育第二个月末期右心房发育迅速,左、右静脉窦均被右房吸收。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "脐静脉" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "左卵黄静脉" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "右心房" }, { "start_idx": 36, "end_idx": 41, "type": "bod", "entity": "左、右静脉窦" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "右房" } ] }, { "text": "右总主静脉发育成上腔静脉,右卵黄静脉发育成下腔静脉。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "右总主静脉" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "右卵黄静脉" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "下腔静脉" } ] }, { "text": "原左静脉窦接收的左总主静脉发育差,形成心外的冠状窦及冠状静脉。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "原左静脉窦" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "左总主静脉" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "心" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "冠状窦" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "冠状静脉" } ] }, { "text": "9.左心房与肺静脉的发育胚胎36天左右时,原始窦-房腔的背侧中胚层组织呈现两个突起,位于静脉窦部的突起逐渐被吸收而消失,而位于心房的突起发展成肺静脉主干。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "左心房" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肺静脉" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "窦-房腔" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "静脉窦部" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "心房" }, { "start_idx": 71, "end_idx": 75, "type": "bod", "entity": "肺静脉主干" } ] }, { "text": "肺静脉主干形成分支,左右各二,随后肺静脉主干被左心房吸收,使左右肺静脉直接进入左心房,左心房内四支肺静脉中间部分属静脉性起源,称为Keir前庭。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "肺静脉主干" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "肺静脉主干" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "左心房" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "左右肺静脉" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "左心房" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "左心房" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "肺静脉" }, { "start_idx": 65, "end_idx": 70, "type": "bod", "entity": "Keir前庭" } ] }, { "text": "第十六节鞘膜积液胚胎发育时,腹膜在下腹部形成一突起,进入腹股沟管并伸延至阴囊底部,称为鞘状突。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "鞘膜积液" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "腹膜" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "腹股沟管" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "阴囊" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "鞘状突" } ] }, { "text": "鞘状突覆盖精索及睾丸的大部分。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "鞘状突" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "精索" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "睾丸" } ] }, { "text": "出生前鞘状突从腹股沟管内环处和近睾丸端两个部位开始闭塞,闭塞过程由两端向中间延续,使覆盖精索的鞘状突形成纤维索带,仅覆盖睾丸部的鞘膜留有间隙,成为睾丸固有鞘膜腔,与腹膜腔不通。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "鞘状突" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "腹股沟管" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "睾丸" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "精索" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "鞘状突" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "纤维索带" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "睾丸" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "鞘膜" }, { "start_idx": 73, "end_idx": 79, "type": "bod", "entity": "睾丸固有鞘膜腔" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "腹膜腔" } ] }, { "text": "鞘状突的闭塞过程可能出现异常,在腹膜腔和睾丸固有鞘膜腔之间的不同水平,覆盖精索的鞘状突未完全闭合,使得腹腔液进入未完全闭合的鞘状突,并在已闭合的近端积聚,即形成临床所见的鞘膜积液。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "鞘状突" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "腹膜腔" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "鞘膜腔" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "鞘状突" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "腹腔液" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "鞘状突" }, { "start_idx": 85, "end_idx": 88, "type": "sym", "entity": "鞘膜积液" } ] }, { "text": "根据鞘突管闭合异常的部位,鞘膜积液基本上分为两个类型:①精索鞘膜积液:鞘状突在近睾丸部闭塞,而其近端至腹膜腔未闭,腹膜腔内液体经内环流入鞘状突临床表现为腹股沟囊性的肿块,但睾丸清晰可及睾丸鞘膜积液:整个鞘状突均未闭合,腹膜腔内液体经鞘状突固有鞘膜腔阴囊内囊性肿块睾丸被囊液所包围积液量大,睾丸难以触及睾丸鞘膜腔与腹膜腔之间未闭的鞘状突一般细小,液体不容易倒流回腹膜腔,故鞘膜腔内积液的张力很大未闭的鞘状突比较粗,则表现为交通性鞘膜积液,积液的张力一般不大,特征性的是,腹股沟或阴囊的肿块在患儿晚上上床时很明显一夜平卧后,晨起肿块明显缩小或消失。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "鞘突管闭合异常" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "鞘膜积液" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "精索鞘膜积液" }, { "start_idx": 35, "end_idx": 55, "type": "sym", "entity": "鞘状突在近睾丸部闭塞,而其近端至腹膜腔未闭" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "腹膜腔" }, { "start_idx": 57, "end_idx": 70, "type": "sym", "entity": "腹膜腔内液体经内环流入鞘状突" }, { "start_idx": 71, "end_idx": 83, "type": "sym", "entity": "临床表现为腹股沟囊性的肿块" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "睾丸" }, { "start_idx": 85, "end_idx": 91, "type": "sym", "entity": "但睾丸清晰可及" }, { "start_idx": 92, "end_idx": 97, "type": "dis", "entity": "睾丸鞘膜积液" }, { "start_idx": 101, "end_idx": 103, "type": "bod", "entity": "鞘状突" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "腹膜腔" }, { "start_idx": 116, "end_idx": 118, "type": "bod", "entity": "鞘状突" }, { "start_idx": 121, "end_idx": 123, "type": "bod", "entity": "鞘膜腔" }, { "start_idx": 99, "end_idx": 123, "type": "sym", "entity": "整个鞘状突均未闭合,腹膜腔内液体经鞘状突固有鞘膜腔" }, { "start_idx": 124, "end_idx": 125, "type": "bod", "entity": "阴囊" }, { "start_idx": 124, "end_idx": 130, "type": "sym", "entity": "阴囊内囊性肿块" }, { "start_idx": 131, "end_idx": 132, "type": "bod", "entity": "睾丸" }, { "start_idx": 131, "end_idx": 138, "type": "sym", "entity": "睾丸被囊液所包围" }, { "start_idx": 144, "end_idx": 145, "type": "bod", "entity": "睾丸" }, { "start_idx": 139, "end_idx": 149, "type": "sym", "entity": "积液量大,睾丸难以触及" }, { "start_idx": 150, "end_idx": 154, "type": "bod", "entity": "睾丸鞘膜腔" }, { "start_idx": 156, "end_idx": 158, "type": "bod", "entity": "腹膜腔" }, { "start_idx": 164, "end_idx": 166, "type": "bod", "entity": "鞘状突" }, { "start_idx": 172, "end_idx": 182, "type": "sym", "entity": "液体不容易倒流回腹膜腔" }, { "start_idx": 185, "end_idx": 187, "type": "bod", "entity": "鞘膜腔" }, { "start_idx": 184, "end_idx": 195, "type": "sym", "entity": "故鞘膜腔内积液的张力很大" }, { "start_idx": 196, "end_idx": 216, "type": "sym", "entity": "未闭的鞘状突比较粗,则表现为交通性鞘膜积液" }, { "start_idx": 218, "end_idx": 226, "type": "sym", "entity": "积液的张力一般不大" }, { "start_idx": 234, "end_idx": 236, "type": "bod", "entity": "腹股沟" }, { "start_idx": 238, "end_idx": 239, "type": "bod", "entity": "阴囊" }, { "start_idx": 234, "end_idx": 253, "type": "sym", "entity": "腹股沟或阴囊的肿块在患儿晚上上床时很明显" }, { "start_idx": 254, "end_idx": 270, "type": "sym", "entity": "一夜平卧后,晨起肿块明显缩小或消失" } ] }, { "text": "鞘膜积液可双侧同时发生,女孩也有,称为Nuck囊肿,位于腹股沟。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "鞘膜积液可双侧同时发生" }, { "start_idx": 12, "end_idx": 30, "type": "sym", "entity": "女孩也有,称为Nuck囊肿,位于腹股沟" } ] }, { "text": "新生儿鞘膜积液相当常见,由于出生后鞘状突继续发生闭合,部分病例可逐渐自行消退,一般认为可观察至1岁,其后自行消退的可能性很小。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "鞘膜积液" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "鞘状突" } ] }, { "text": "临床应引起重视的是睾丸肿瘤也可伴有鞘膜积液,在积液量多、张力高、未触及睾丸的情况下,尤其容易误诊。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "睾丸肿瘤" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "鞘膜积液" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "张力高" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "睾丸" } ] }, { "text": "我们就有以鞘膜积液诊断手术而术中发现为睾丸肿瘤的经历。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "鞘膜积液诊断手术" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "睾丸肿瘤" } ] }, { "text": "新生儿鞘膜积液至1岁后鞘膜鞘膜积液后发现的鞘膜积液均应手术治疗。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "新生儿鞘膜积液" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "鞘膜鞘膜积液" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "鞘膜积液" } ] }, { "text": "有主张如果积液张力较高,可能影响睾丸血液循环,导致睾丸萎缩,应早期手术治疗。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "睾丸" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "睾丸" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "手术治疗" } ] }, { "text": "鞘膜翻转缝合或鞘膜切除,主要用于成人鞘膜积液,小儿中完全结扎鞘状鞘状突放出积液关键是完全结扎鞘状突,放出积液。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "鞘膜翻转缝合" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "鞘膜切除" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "成人鞘膜积液" }, { "start_idx": 26, "end_idx": 38, "type": "pro", "entity": "完全结扎鞘状鞘状突放出积液" }, { "start_idx": 42, "end_idx": 53, "type": "pro", "entity": "完全结扎鞘状突,放出积液" } ] }, { "text": "第三章继发性免疫缺陷病临床实践中逐步发现很多免疫功能缺陷病为继发性免疫缺陷病(secondaryimmunodeficiencydisease,SID),SID与原发性免疫缺陷病(PID)的重要区别在于:①PID几乎都是特定的单基因缺失,导致相应的免疫活性细胞或免疫分子受损,表现出这种功能的完全缺失,且为不可逆的改变;而SID常为免疫系统多环节受损,但受损程度较PID轻,仅为部分功能受损,表现为免疫功能低下(immunocompromise);②PID系关键位基因突变,除非免疫重建,否则其免疫功能缺陷将为终身性,SID为后天环境因素致免疫功能缺陷,虽也能影响基因表达,但仅系基因不完全性表达障碍,去除不利因素之后,免疫功能将可能恢复正常。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "继发性免疫缺陷病" }, { "start_idx": 30, "end_idx": 37, "type": "dis", "entity": "继发性免疫缺陷病" }, { "start_idx": 39, "end_idx": 70, "type": "dis", "entity": "secondaryimmunodeficiencydisease" }, { "start_idx": 72, "end_idx": 74, "type": "dis", "entity": "SID" }, { "start_idx": 77, "end_idx": 79, "type": "dis", "entity": "SID" }, { "start_idx": 81, "end_idx": 88, "type": "dis", "entity": "原发性免疫缺陷病" }, { "start_idx": 90, "end_idx": 92, "type": "dis", "entity": "PID" }, { "start_idx": 103, "end_idx": 105, "type": "dis", "entity": "PID" }, { "start_idx": 124, "end_idx": 129, "type": "bod", "entity": "免疫活性细胞" }, { "start_idx": 131, "end_idx": 134, "type": "bod", "entity": "免疫分子" }, { "start_idx": 161, "end_idx": 163, "type": "dis", "entity": "SID" }, { "start_idx": 182, "end_idx": 184, "type": "dis", "entity": "PID" }, { "start_idx": 199, "end_idx": 204, "type": "sym", "entity": "免疫功能低下" }, { "start_idx": 206, "end_idx": 221, "type": "sym", "entity": "immunocompromise" }, { "start_idx": 225, "end_idx": 227, "type": "dis", "entity": "PID" }, { "start_idx": 259, "end_idx": 261, "type": "dis", "entity": "SID" } ] }, { "text": "【病因】(一)感染感染即是免疫缺陷病的临床表现之一,同时也是致SID的原因。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "免疫缺陷病" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "SID" } ] }, { "text": "人类免疫缺陷病毒(HIV)感染致获得性免疫缺陷病(aquiredimmunodeficiencysyndrome,AIDS)是感染引起SID的典型例子。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "mic", "entity": "人类免疫缺陷病毒" }, { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "HIV" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "获得性免疫缺陷病" }, { "start_idx": 25, "end_idx": 55, "type": "dis", "entity": "aquiredimmunodeficiencysyndrome" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "AIDS" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "SID" } ] }, { "text": "(二)营养紊乱(五)医源性因素药物是临床实践中较常见的免疫低下的重要原因,如糖皮质激素、细胞毒性药物及放射性照射等均可引起SID。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "营养紊乱" }, { "start_idx": 38, "end_idx": 42, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 44, "end_idx": 49, "type": "dru", "entity": "细胞毒性药物" }, { "start_idx": 51, "end_idx": 55, "type": "pro", "entity": "放射性照射" }, { "start_idx": 61, "end_idx": 63, "type": "dis", "entity": "SID" } ] }, { "text": "在自身免疫性疾病及肿瘤等多种疾病的治疗中采用这些手段均要警惕SID的发生。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肿瘤" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "SID" } ] }, { "text": "引起SID因素复杂,现列简表如下(表17-17)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "SID" } ] }, { "text": "表17-6各种维生素和微量元素缺乏引起的免疫功能缺陷注:+轻度抑制,++中度抑制,+++严重抑制,*动物模型结果,尚未在人体证实表17-7导致继发性免疫缺陷病的因素【临床表现与治疗】SID的临床表现与PID大致相同,但其程度往往轻于后者,治疗效果也较好,反复感染是突出表现,并发肿瘤与自身免疫性疾病的机会相对较少。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "维生素" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "微量元素" }, { "start_idx": 77, "end_idx": 84, "type": "dis", "entity": "继发性免疫缺陷病" }, { "start_idx": 97, "end_idx": 99, "type": "dis", "entity": "SID" }, { "start_idx": 106, "end_idx": 108, "type": "dis", "entity": "PID" }, { "start_idx": 145, "end_idx": 146, "type": "dis", "entity": "肿瘤" }, { "start_idx": 148, "end_idx": 154, "type": "dis", "entity": "自身免疫性疾病" } ] }, { "text": "SID治疗原则上是根治原发病及免疫替代治疗和对症治疗,替代治疗和对症治疗均要视免疫缺陷的类型及后果而定,因在何种因素影响下,产生何种特殊免疫异常并不十分明了,故只有根治原发病,才有可能彻底纠正SID。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "SID" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "免疫替代治疗" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "对症治疗" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "替代治疗" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "对症治疗" }, { "start_idx": 96, "end_idx": 98, "type": "dis", "entity": "SID" } ] }, { "text": "三、CVS临床表现1.CVS分期和分级CVS分为4个时期:①间歇期:几乎没有症状;②前驱期:有接近于发作的表现,通过药物尚能控制;③呕吐期:持续而强烈的恶心、呕吐、干呕和其他症状;④恢复期:恶心很快停止,患者恢复食欲及精神状态。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "CVS" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "CVS" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "CVS" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "几乎没有症状" }, { "start_idx": 46, "end_idx": 54, "type": "sym", "entity": "有接近于发作的表现" }, { "start_idx": 70, "end_idx": 88, "type": "sym", "entity": "持续而强烈的恶心、呕吐、干呕和其他症状" }, { "start_idx": 95, "end_idx": 100, "type": "sym", "entity": "恶心很快停止" }, { "start_idx": 102, "end_idx": 112, "type": "sym", "entity": "患者恢复食欲及精神状态" } ] }, { "text": "2.CVS临床表现特点CVS以反复发生、刻板发作的剧烈恶心、呕吐为特征,持续数小时到数天。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "CVS" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "CVS" }, { "start_idx": 15, "end_idx": 31, "type": "sym", "entity": "反复发生、刻板发作的剧烈恶心、呕吐" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "持续数小时到数天" } ] }, { "text": "间歇期无症状,可持续数周到数月。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "间歇期无症状" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "可持续数周到数月" } ] }, { "text": "每日发作时间比较固定,通常在晚上或凌晨。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "每日发作时间比较固定,通常在晚上或凌晨" } ] }, { "text": "一旦发作,在最初的数小时内便达到最大强度,发作和停止却非常快速,呈一种“开-关”刻板模式。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "一旦发作,在最初的数小时内便达到最大强度" }, { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "发作和停止却非常快速" }, { "start_idx": 32, "end_idx": 43, "type": "sym", "entity": "呈一种“开-关”刻板模式" } ] }, { "text": "二、HSCT适应证HSCT医疗费用高、移植风险大,因此必须把握好移植指征,只有当移植治疗疗效明显优于其他治疗方法时才有必要采用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "HSCT" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "HSCT" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "移植" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "移植" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "指征" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "移植" } ] }, { "text": "(一)异基因HSCT常见适应证1.造血系统恶性疾病(1)急性淋巴细胞白血病(ALL):①高度难治或高危复发危险的ALL,如初治35天未达完全缓解者;有t(9;22)或t(4;11)并对早期治疗反应不良者(如诱导治疗第14~19天骨髓涂片肿瘤细胞仍大于5%)。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "异基因HSCT" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "造血系统恶性疾病" }, { "start_idx": 28, "end_idx": 36, "type": "dis", "entity": "急性淋巴细胞白血病" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "ALL" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "ALL" }, { "start_idx": 103, "end_idx": 106, "type": "pro", "entity": "诱导治疗" }, { "start_idx": 114, "end_idx": 117, "type": "pro", "entity": "骨髓涂片" }, { "start_idx": 118, "end_idx": 121, "type": "bod", "entity": "肿瘤细胞" } ] }, { "text": "②复发ALL再CR者(低危停药半年以上晚期复发的CR2B-ALL仍应以化疗为主)。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "复发ALL再CR" }, { "start_idx": 11, "end_idx": 31, "type": "dis", "entity": "低危停药半年以上晚期复发的CR2B-ALL" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "化疗" } ] }, { "text": "非CR状态的ALL无移植指征。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "非CR状态的ALL" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "移植" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "指征" } ] }, { "text": "(2)AML:①经正规诱导治疗1个疗程恢复造血后幼稚细胞仍然大于15%或2个疗程未CR者;②复发后再CR者;③高度难治,多个疗程后仍然未CR但幼稚细胞比例不很高者;④MDS转化而来的AML、治疗相关的AML患者、M0、M7患者[排除t(1;22)];⑤若为同胞相合供体,所有非M3、非低危AML均应考虑HSCT。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "AML" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "诱导治疗" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "幼稚细胞" }, { "start_idx": 71, "end_idx": 74, "type": "bod", "entity": "幼稚细胞" }, { "start_idx": 83, "end_idx": 85, "type": "dis", "entity": "MDS" }, { "start_idx": 91, "end_idx": 93, "type": "dis", "entity": "AML" }, { "start_idx": 100, "end_idx": 102, "type": "dis", "entity": "AML" }, { "start_idx": 106, "end_idx": 113, "type": "dis", "entity": "M0" }, { "start_idx": 115, "end_idx": 122, "type": "dis", "entity": "M7" }, { "start_idx": 149, "end_idx": 151, "type": "dis", "entity": "非M3" }, { "start_idx": 153, "end_idx": 158, "type": "dis", "entity": "非低危AML" }, { "start_idx": 163, "end_idx": 166, "type": "pro", "entity": "HSCT" } ] }, { "text": "(3)慢性粒细胞性白血病。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "慢性粒细胞性白血病" } ] }, { "text": "(4)非霍奇金淋巴瘤伴有骨髓浸润并经正规治疗后复发者。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "非霍奇金淋巴瘤" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "伴有骨髓浸润" } ] }, { "text": "(5)骨髓增生异常综合征。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "骨髓增生异常综合征" } ] }, { "text": "2.预后不良的造血系统衰竭性疾病(1)重型再生障碍性贫血(SAA)。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "造血系统衰竭性疾病" }, { "start_idx": 19, "end_idx": 27, "type": "dis", "entity": "重型再生障碍性贫血" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "SAA" } ] }, { "text": "(2)继发性造血系统衰竭(如放射病)。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "继发性造血系统衰竭" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "放射病" } ] }, { "text": "3.先天性造血、免疫系统疾病(1)家族性噬血细胞增生征。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dis", "entity": "先天性造血、免疫系统疾病" }, { "start_idx": 17, "end_idx": 26, "type": "dis", "entity": "家族性噬血细胞增生征" } ] }, { "text": "(2)免疫缺陷:重型联合免疫缺陷、慢性肉芽肿病、X-连锁高IgM血症、Wiskott-Aldrich综合征、Chediak-Higashi综合征等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "重型联合免疫缺陷" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "慢性肉芽肿病" }, { "start_idx": 24, "end_idx": 33, "type": "dis", "entity": "X-连锁高IgM血症" }, { "start_idx": 35, "end_idx": 52, "type": "dis", "entity": "Wiskott-Aldrich综合征" }, { "start_idx": 54, "end_idx": 71, "type": "dis", "entity": "Chediak-Higashi综合征" } ] }, { "text": "(3)重型地中海贫血、先天性粒细胞缺乏症、药物治疗无效的先天性纯红再障。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "重型地中海贫血" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "先天性粒细胞缺乏症" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "先天性纯红再障" } ] }, { "text": "4.先天代谢异常如黏多糖累积症Ⅰ、Ⅱ、Ⅵ型,肾上腺脑白质营养不良、甘露糖苷储积症、戈谢病、尼曼匹克病、石骨症等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "先天代谢异常" }, { "start_idx": 9, "end_idx": 20, "type": "dis", "entity": "黏多糖累积症Ⅰ、Ⅱ、Ⅵ型" }, { "start_idx": 22, "end_idx": 31, "type": "dis", "entity": "肾上腺脑白质营养不良" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "甘露糖苷储积症" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "戈谢病" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "尼曼匹克病" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "石骨症" } ] }, { "text": "(二)自体移植的适应证1.无骨髓浸润的化疗一定程度敏感的晚期恶性实体瘤,如神经母细胞瘤、脑瘤、尤因肉瘤、横纹肌肉瘤、恶性生殖细胞性肿瘤等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "自体移植" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "骨髓" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "化疗" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "晚期恶性实体瘤" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "神经母细胞瘤" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "脑瘤" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "尤因肉瘤" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 58, "end_idx": 66, "type": "dis", "entity": "恶性生殖细胞性肿瘤" } ] }, { "text": "2.难治性自身免疫性疾病。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "难治性自身免疫性疾病" } ] }, { "text": "第四节成分输血与其他血制品的应用在血液系统、肿瘤性疾病和其他一些危重疾病的治疗过程中常涉及血制品的应用,不同的临床状况需应用不同的血制品。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "成分输血" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "血制品" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "血液系统" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "肿瘤性疾病" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "血制品" }, { "start_idx": 65, "end_idx": 67, "type": "dru", "entity": "血制品" } ] }, { "text": "一、血制品的种类及应用指征(一)血细胞成分制品1.全血即为未祛除任何成分的血液。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "血制品" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "血细胞" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "全血" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "血液" } ] }, { "text": "正常人新鲜全血含有各种有效的血细胞及血浆成分,库存血随着库存时间的延长,根据半衰期不同逐渐丧失某些成分的活性,如新鲜全血中含有凝血因子Ⅷ,在无其他途径获得Ⅷ因子时,可采用新鲜全血作替代治疗,但Ⅷ因子半衰期仅为8~12小时,因此库存血就不能用作Ⅷ因子缺乏的替代治疗。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "全血" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "血细胞" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "血浆" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "全血" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "凝血因子Ⅷ" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "Ⅷ因子" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "全血" }, { "start_idx": 96, "end_idx": 98, "type": "bod", "entity": "Ⅷ因子" }, { "start_idx": 121, "end_idx": 123, "type": "bod", "entity": "Ⅷ因子" } ] }, { "text": "目前全血的应用日趋减少,主要有以下原因:①绝大多数疾病状态仅缺乏血液的某些成分;②某些特定成分缺乏时如采用全血,很难达到有效水平,因此影响疗效;③血液成分的分离技术越来越完善;④采用全血时浪费所需成分以外的其他血液成分。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "全血" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "血液" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "全血" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "血液" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "全血" }, { "start_idx": 105, "end_idx": 106, "type": "bod", "entity": "血液" } ] }, { "text": "2.以红细胞为主要成分的少浆血根据需要在全血中提取血浆、血小板、白细胞后的以红细胞为主要成分的悬液,是应用最为广泛的血液成分,根据被提取的成分不同可分别称为少浆全血、去白细胞少浆血、去血小板少浆血。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "红细胞" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "浆血" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "全血" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血浆" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "血小板" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "白细胞" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "红细胞" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "悬液" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "血液" }, { "start_idx": 78, "end_idx": 81, "type": "dru", "entity": "少浆全血" }, { "start_idx": 83, "end_idx": 89, "type": "dru", "entity": "去白细胞少浆血" }, { "start_idx": 91, "end_idx": 97, "type": "dru", "entity": "去血小板少浆血" } ] }, { "text": "少浆血可应用于各种原因引起的贫血。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "少浆血" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "贫血" } ] }, { "text": "在无明显失血或急性溶血时,输注10ml/kg少浆血可提高血色素20g/L(上海儿童医学中心100例输血疗效分析结果)。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "失血" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "急性溶血" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "少浆血" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "血色素" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "输血" } ] }, { "text": "每次输血量一般为5~10ml/kg,对较为慢性病程的病人,贫血越严重,单次输血量应越少,并需缓慢输入,以避免因血容量急剧增多而致心力衰竭。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "输血量" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "贫血" }, { "start_idx": 37, "end_idx": 39, "type": "ite", "entity": "输血量" }, { "start_idx": 55, "end_idx": 57, "type": "ite", "entity": "血容量" }, { "start_idx": 64, "end_idx": 67, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "3.血小板通过血细胞分离仪从单个供血者采集的血小板称为单采血小板,从2000ml全血中分离的血小板量为1个单采单位,容量为200ml。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "血小板" }, { "start_idx": 7, "end_idx": 12, "type": "equ", "entity": "血细胞分离仪" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "血小板" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "单采血小板" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "全血" }, { "start_idx": 46, "end_idx": 49, "type": "ite", "entity": "血小板量" } ] }, { "text": "从已采集的非同一供体的全血中分离的血小板为多采血小板,从200ml全血中分离的血小板量为1个多采单位,容量为25ml,10个多采单位相当于1个单采单位。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "全血" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "血小板" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "血小板" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "全血" }, { "start_idx": 39, "end_idx": 42, "type": "ite", "entity": "血小板量" } ] }, { "text": "血小板输注主要用于血小板明显减少或功能异常并伴有出血倾向者。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "输注" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "血小板" }, { "start_idx": 9, "end_idx": 25, "type": "sym", "entity": "血小板明显减少或功能异常并伴有出血" } ] }, { "text": "输注10ml/kg单采血小板可提高血小板50×109/L(上海儿童医学中心50例单采血小板输注疗效分析结果)。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "血小板" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "血小板" }, { "start_idx": 48, "end_idx": 52, "type": "pro", "entity": "血小板输注" } ] }, { "text": "在血小板相关抗体明显增高时输注效果减低。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "血小板" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "抗体" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "输注" } ] }, { "text": "血小板寿命短,需新鲜采集使用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" } ] }, { "text": "4.白细胞通过血细胞分离仪可分离得白细胞,但白细胞输注的疗效尚未被充分肯定,主要用于粒细胞极度低下及粒细胞功能障碍。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "白细胞" }, { "start_idx": 7, "end_idx": 12, "type": "equ", "entity": "血细胞分离仪" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "白细胞" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "白细胞输注" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "粒细胞极度低下" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "粒细胞功能障碍" } ] }, { "text": "白细胞寿命短,也需新鲜采集使用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "白细胞" } ] }, { "text": "5.造血干细胞输注见干细胞移植章节。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "造血干细胞" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "输注" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "干细胞移植" } ] }, { "text": "(二)血浆成分1.全血浆新鲜全血浆或新鲜冰冻全血浆含所有正常血浆成分,主要用于非失血性低血容量性休克的扩容、低蛋白血症、低免疫球蛋白血症、各种凝血因子缺乏症和其他血浆成分缺乏的替代治疗,常用剂量为5~10ml/kg。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血浆" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "全血浆" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "全血浆" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "血浆" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "血浆" }, { "start_idx": 39, "end_idx": 49, "type": "dis", "entity": "非失血性低血容量性休克" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "低蛋白血症" }, { "start_idx": 60, "end_idx": 67, "type": "dis", "entity": "低免疫球蛋白血症" }, { "start_idx": 71, "end_idx": 77, "type": "dis", "entity": "凝血因子缺乏症" }, { "start_idx": 81, "end_idx": 82, "type": "dis", "entity": "血浆" } ] }, { "text": "2.丙种球蛋白静脉用丙种球蛋白主要用于低丙种球蛋白血症的替代治疗及感染性疾病的辅助支持治疗,常用剂量为200~500mg/kg。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "丙种球蛋白" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "静脉" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "丙种球蛋白" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "低丙种球蛋白血症" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "替代治疗" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 39, "end_idx": 44, "type": "pro", "entity": "辅助支持治疗" } ] }, { "text": "大剂量(≥1克/千克体重)时有免疫封闭作用,可作为特发性血小板减少性紫癜和再障的免疫抑制治疗。", "entities": [ { "start_idx": 25, "end_idx": 35, "type": "dis", "entity": "特发性血小板减少性紫癜" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "再障" }, { "start_idx": 40, "end_idx": 45, "type": "pro", "entity": "免疫抑制治疗" } ] }, { "text": "3.凝血因子Ⅷ从血浆中提纯,主要用于血友病甲的替代治疗,1单位/千克体重可提高Ⅷ因子活力2%。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "凝血因子Ⅷ" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "血浆" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "血友病甲" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "替代治疗" }, { "start_idx": 34, "end_idx": 35, "type": "ite", "entity": "体重" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "Ⅷ因子" } ] }, { "text": "4.凝血酶原复合物用于多种内外源凝血因子缺乏的补充及替代治疗,如Ⅶ、Ⅷ、Ⅸ、Ⅹ因子缺乏,包括血友病及维生素K依赖性凝血因子缺乏症。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "凝血酶原复合物" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "内外源凝血因子缺乏" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "替代治疗" }, { "start_idx": 32, "end_idx": 42, "type": "sym", "entity": "Ⅶ、Ⅷ、Ⅸ、Ⅹ因子缺乏" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "血友病" }, { "start_idx": 50, "end_idx": 63, "type": "dis", "entity": "维生素K依赖性凝血因子缺乏症" } ] }, { "text": "5.纤维蛋白原复合物主要用于纤维蛋白原缺乏症和异常纤维蛋白原血症。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "bod", "entity": "纤维蛋白原复合物" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "纤维蛋白原缺乏症" }, { "start_idx": 23, "end_idx": 31, "type": "dis", "entity": "异常纤维蛋白原血症" } ] }, { "text": "(三)特殊处理的血制品1.三洗红细胞用生理盐水三次洗涤的红细胞,主要洗去可加重免疫性溶血的补体,用于免疫性溶血性贫血需输血的病人。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "血制品" }, { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "三洗红细胞" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "生理盐水" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "洗涤" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "红细胞" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "免疫性溶血" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "补体" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "免疫性溶血性贫血" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "输血" } ] }, { "text": "2.照光血制品强烈化放疗免疫功能极度抑制病人、细胞免疫功能不全、异体移植后免疫功能未完全重建者输注含有淋巴细胞的血制品时,血制品需先经1800cGy照光,以灭活可能导致移植物抗宿主病的淋巴细胞。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "照光血制品" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "强烈化放疗" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "细胞" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "异体移植" }, { "start_idx": 47, "end_idx": 48, "type": "pro", "entity": "输注" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 56, "end_idx": 58, "type": "dru", "entity": "血制品" }, { "start_idx": 61, "end_idx": 63, "type": "dru", "entity": "血制品" }, { "start_idx": 84, "end_idx": 90, "type": "dis", "entity": "移植物抗宿主病" }, { "start_idx": 92, "end_idx": 95, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "3.去白细胞成分血正常人白细胞携带CMV机会较高,异体骨髓移植受体输注后易发生CMV感染并可导致死亡,对异体骨髓移植者可输注去白细胞成分血。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "白细胞" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "血" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "白细胞" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "CMV" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "异体骨髓移植" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "输注" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "CMV感染" }, { "start_idx": 52, "end_idx": 57, "type": "pro", "entity": "异体骨髓移植" }, { "start_idx": 60, "end_idx": 61, "type": "pro", "entity": "输注" }, { "start_idx": 62, "end_idx": 68, "type": "dru", "entity": "去白细胞成分血" } ] }, { "text": "五、弥散性血管内凝血弥散性血管内凝血(DIC)是许多疾病严重阶段的并发症,机体在某些致病因素作用下,凝血系统被激活,凝血过程加速,微循环内发生纤维蛋白沉积和血小板凝集,导致血管内广泛微血栓形成,由于凝血因子被消耗,发生广泛出血。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "弥散性血管内凝血" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "弥散性血管内凝血" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "DIC" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "并发症" }, { "start_idx": 71, "end_idx": 74, "type": "bod", "entity": "纤维蛋白" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "血小板" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "血管" } ] }, { "text": "新生儿由于易患严重疾病,DIC的发生率较高。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "DIC" } ] }, { "text": "【病因与发病机制】1.感染严重感染是导致新生儿DIC的主要原因。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "感染" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "严重感染" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "DIC" } ] }, { "text": "2.缺氧缺氧导致酸中毒、血黏度增高。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "血黏度" } ] }, { "text": "3.寒冷损伤由于寒冷及皮下脂肪变硬,微循环灌流不足,毛细血管损伤。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "皮下脂肪" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "毛细血管" } ] }, { "text": "4.溶血由于红细胞破坏,释放大量磷脂类凝血活酶物质,促发DIC。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "红细胞" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "磷脂类凝血活酶" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "DIC" } ] }, { "text": "5.其他早产儿因易发生各种疾病及凝血机制不完善,DIC发生率较高。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "DIC" } ] }, { "text": "某些产科因素,如胎盘早期剥离、前置胎盘、严重妊娠高血压等,由于胎盘损伤释放组织凝血活酶进入胎儿循环,促发DIC。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胎盘" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "严重妊娠高血压" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "胎盘" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "凝血活酶" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "DIC" } ] }, { "text": "【临床表现】1.出血因凝血因子大量被消耗、血小板减少及继发纤溶亢进,发生出血。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "凝血因子" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "血小板" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "继发纤溶亢进" } ] }, { "text": "常见皮肤瘀点、瘀斑、脐部渗血、穿刺点渗血,严重者出现消化道、泌尿道、肺出血等全身广泛出血。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "皮肤瘀点" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "瘀斑" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "脐部渗血" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "穿刺点渗血" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "消化道、泌尿道、肺出血" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "全身广泛出血" } ] }, { "text": "2.休克由于微循环广泛血栓形成,通路受阻,发生循环障碍,出现休克。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "休克" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "血栓形成" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "通路受阻" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "循环障碍" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "休克" } ] }, { "text": "3.栓塞由于微循环广泛血栓形成,受累脏器发生缺氧缺血损伤,出现多脏器功能衰竭。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "栓塞" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "血栓" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "脏器" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "缺氧缺血损伤" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "脏器" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "功能衰竭" } ] }, { "text": "4.溶血因红细胞变形受损,发生微血管病性溶血,出现血红蛋白尿、黄疸、发热。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "红细胞" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "微血管病性溶血" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "血红蛋白尿" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "黄疸" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "发热" } ] }, { "text": "2.DIC的主要指标监测D-二聚体、血小板、凝血时间、凝血酶原时间、3P试验等项目的动态变化。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "DIC" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "D-二聚体" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "血小板" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "凝血酶原" } ] }, { "text": "3.使用肝素早期使用小剂量肝素,20U/kg,皮下注射,每天2~3次。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dru", "entity": "肝素" } ] }, { "text": "4.改善微循环低分子右旋糖酐,10~20ml/kg,静脉滴注。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "右旋糖酐" } ] }, { "text": "三、影像学检查影像学检查(超声,X线,CT,MRI等)主要是作为关节炎及其他器官损害的定位与程度评价,除极少数特征性骨关节病变外,是重要的鉴别诊断及疗效评价方法,基本上没有病因诊断价值。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "影像学检查" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "影像学检查" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "超声" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "X线" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "CT" }, { "start_idx": 22, "end_idx": 24, "type": "pro", "entity": "MRI" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "关节炎" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "器官" }, { "start_idx": 55, "end_idx": 62, "type": "dis", "entity": "特征性骨关节病变" } ] }, { "text": "二、选择常见病原菌的敏感抗生素类型,进行初始经验性治疗不论CAP还是HAP,绝大多数肺炎患儿的治疗是经验性的;在初始选择抗生素时候,应当覆盖大多数可能的病原,同时避免使用多种或过于贵重以及毒性较强的抗生素。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "病原菌" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "抗生素" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "CAP" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "HAP" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "肺炎" }, { "start_idx": 61, "end_idx": 63, "type": "dru", "entity": "抗生素" }, { "start_idx": 100, "end_idx": 102, "type": "dru", "entity": "抗生素" } ] }, { "text": "对于重危患儿,为了能够尽快控制病情,在没有得到明确的病原学诊断以前,就可以开始用广谱的静脉抗生素治疗。", "entities": [ { "start_idx": 43, "end_idx": 49, "type": "pro", "entity": "静脉抗生素治疗" } ] }, { "text": "常用的抗生素分类及使用剂量(表8-8~表8-8)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "抗生素" } ] }, { "text": "表8-8药效动力学因素对剂量方案和体内效能相关参数的影响注:MIC,最低抑菌浓度;AUC,曲线下面积;MBC,最低杀菌浓度表8-9常用抗生素分类续表续表表8-10呼吸道感染常用抗生素剂量与方法续表*氨基糖苷类抗生素在6岁以下小儿原则不用,6岁以上慎用。", "entities": [ { "start_idx": 67, "end_idx": 69, "type": "dru", "entity": "抗生素" }, { "start_idx": 81, "end_idx": 85, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 88, "end_idx": 90, "type": "dru", "entity": "抗生素" }, { "start_idx": 110, "end_idx": 117, "type": "dru", "entity": "氨基糖苷类抗生素" } ] }, { "text": "im,肌内注射;ivgtt,静脉滴注;po,口服肺炎链球菌:青霉素敏感者首选青霉素,或羟氨苄青霉素;青霉素低度耐药者仍可首选青霉素,但剂量要加大,也可选用第1代或第2代头孢菌素,备选头孢曲松或头孢噻肟或万古霉素。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "im" }, { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "肌内注射" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "ivgtt" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "po" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "口服" }, { "start_idx": 24, "end_idx": 28, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "青霉素" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "青霉素敏感" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "青霉素" }, { "start_idx": 43, "end_idx": 48, "type": "dru", "entity": "羟氨苄青霉素" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "青霉素" }, { "start_idx": 50, "end_idx": 56, "type": "sym", "entity": "青霉素低度耐药" }, { "start_idx": 62, "end_idx": 64, "type": "dru", "entity": "青霉素" }, { "start_idx": 77, "end_idx": 87, "type": "dru", "entity": "第1代或第2代头孢菌素" }, { "start_idx": 91, "end_idx": 94, "type": "dru", "entity": "头孢曲松" }, { "start_idx": 96, "end_idx": 99, "type": "dru", "entity": "头孢噻肟" }, { "start_idx": 101, "end_idx": 104, "type": "dru", "entity": "万古霉素" } ] }, { "text": "青霉素高度耐药或存在危险因素者首选万古霉素或头孢曲松或头孢噻肟。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "青霉素" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "万古霉素" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "头孢曲松" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "头孢噻肟" } ] }, { "text": "流感嗜血杆菌:首选羟氨苄青霉素/克拉维酸或氨苄西林/舒巴坦,备选第2~3代头孢菌素或新大环内酯类(罗红霉素、阿奇霉素、克拉霉素)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 9, "end_idx": 19, "type": "dru", "entity": "羟氨苄青霉素/克拉维酸" }, { "start_idx": 21, "end_idx": 28, "type": "dru", "entity": "氨苄西林/舒巴坦" }, { "start_idx": 32, "end_idx": 40, "type": "dru", "entity": "第2~3代头孢菌素" }, { "start_idx": 42, "end_idx": 47, "type": "dru", "entity": "新大环内酯类" }, { "start_idx": 49, "end_idx": 52, "type": "dru", "entity": "罗红霉素" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "阿奇霉素" }, { "start_idx": 59, "end_idx": 62, "type": "dru", "entity": "克拉霉素" } ] }, { "text": "葡萄球菌:MSSA、MSSE,首选苯唑西林或氯唑青霉素,备选第1代、第2代头孢菌素。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "葡萄球菌" }, { "start_idx": 5, "end_idx": 8, "type": "mic", "entity": "MSSA" }, { "start_idx": 10, "end_idx": 13, "type": "mic", "entity": "MSSE" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "苯唑西林" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "氯唑青霉素" }, { "start_idx": 30, "end_idx": 40, "type": "dru", "entity": "第1代、第2代头孢菌素" } ] }, { "text": "MRSA、MRSE,首选万古霉素或联用利福平。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "MRSA" }, { "start_idx": 5, "end_idx": 8, "type": "mic", "entity": "MRSE" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "万古霉素" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "利福平" } ] }, { "text": "卡他莫拉菌:首选羟氨苄青霉素/克拉维酸,备选第2代或第3代头孢菌素或新大环内酯类。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "卡他莫拉菌" }, { "start_idx": 8, "end_idx": 18, "type": "dru", "entity": "羟氨苄青霉素/克拉维酸" }, { "start_idx": 22, "end_idx": 32, "type": "dru", "entity": "第2代或第3代头孢菌素" }, { "start_idx": 34, "end_idx": 39, "type": "dru", "entity": "新大环内酯类" } ] }, { "text": "肠杆菌科(大肠杆菌、克雷伯杆菌、变形杆菌等):首选头孢曲松或头孢噻肟,单用或联用丁胺卡那霉素,备选有替卡西林/克拉维酸或氨曲南或亚胺培南或第3代头孢菌素如头孢吡肟等或庆大霉素。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "肠杆菌科" }, { "start_idx": 5, "end_idx": 8, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 10, "end_idx": 14, "type": "mic", "entity": "克雷伯杆菌" }, { "start_idx": 16, "end_idx": 19, "type": "mic", "entity": "变形杆菌" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "头孢曲松" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "头孢噻肟" }, { "start_idx": 40, "end_idx": 45, "type": "dru", "entity": "丁胺卡那霉素" }, { "start_idx": 50, "end_idx": 58, "type": "dru", "entity": "替卡西林/克拉维酸" }, { "start_idx": 60, "end_idx": 62, "type": "dru", "entity": "氨曲南" }, { "start_idx": 64, "end_idx": 67, "type": "dru", "entity": "亚胺培南" }, { "start_idx": 69, "end_idx": 75, "type": "dru", "entity": "第3代头孢菌素" }, { "start_idx": 77, "end_idx": 80, "type": "dru", "entity": "头孢吡肟" }, { "start_idx": 83, "end_idx": 86, "type": "dru", "entity": "庆大霉素" } ] }, { "text": "铜绿假单胞杆菌:首选替卡西林/克拉维酸或哌拉西林/他唑巴坦,或美洛西林或头孢他啶或头孢哌酮/舒巴坦或头孢吡肟,单用或联用氨基糖苷类抗生素(丁胺卡那霉素或庆大霉素,应注意使用指征:6岁以下小儿原则不用,6岁以上慎用),备选有氨基糖苷类联用氨曲南或亚胺培南。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "mic", "entity": "铜绿假单胞杆菌" }, { "start_idx": 10, "end_idx": 18, "type": "dru", "entity": "替卡西林/克拉维酸" }, { "start_idx": 20, "end_idx": 28, "type": "dru", "entity": "哌拉西林/他唑巴坦" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "美洛西林" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "头孢他啶" }, { "start_idx": 41, "end_idx": 48, "type": "dru", "entity": "头孢哌酮/舒巴坦" }, { "start_idx": 50, "end_idx": 53, "type": "dru", "entity": "头孢吡肟" }, { "start_idx": 60, "end_idx": 67, "type": "dru", "entity": "氨基糖苷类抗生素" }, { "start_idx": 69, "end_idx": 74, "type": "dru", "entity": "丁胺卡那霉素" }, { "start_idx": 76, "end_idx": 79, "type": "dru", "entity": "庆大霉素" }, { "start_idx": 111, "end_idx": 115, "type": "dru", "entity": "氨基糖苷类" }, { "start_idx": 118, "end_idx": 120, "type": "dru", "entity": "氨曲南" }, { "start_idx": 122, "end_idx": 125, "type": "dru", "entity": "亚胺培南" } ] }, { "text": "B族链球菌:首选青霉素,或羟氨苄青霉素或氨苄西林,青霉素剂量要加大。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "B族链球菌" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "青霉素" }, { "start_idx": 13, "end_idx": 18, "type": "dru", "entity": "羟氨苄青霉素" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "青霉素" } ] }, { "text": "厌氧菌:首选青霉素,联用克林霉素或甲硝唑,或羟氨苄青霉素/克拉维酸或氨苄西林/舒巴坦。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "厌氧菌" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "青霉素" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "克林霉素" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "甲硝唑" }, { "start_idx": 22, "end_idx": 32, "type": "dru", "entity": "羟氨苄青霉素/克拉维酸" }, { "start_idx": 34, "end_idx": 41, "type": "dru", "entity": "氨苄西林/舒巴坦" } ] }, { "text": "单核细胞增多性李斯特菌:首选羟氨苄青霉素或氨苄西林。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "mic", "entity": "单核细胞增多性李斯特菌" }, { "start_idx": 14, "end_idx": 19, "type": "dru", "entity": "羟氨苄青霉素" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "氨苄西林" } ] }, { "text": "百日咳杆菌、支原体、衣原体:选用大环内酯类抗生素。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "百日咳杆菌" }, { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "支原体" }, { "start_idx": 10, "end_idx": 12, "type": "mic", "entity": "衣原体" }, { "start_idx": 16, "end_idx": 23, "type": "dru", "entity": "大环内酯类抗生素" } ] }, { "text": "参考文献1.全国人体重要寄生虫病现状调查办公室.全国人体重要寄生虫病现状调查报告.中国寄生虫学与寄生虫病杂志,2005,23(5):332-3322.王战胜.小儿鼻蛲虫病16例分析.临床儿科杂志,2000,18:3193.詹斌,肖树华,李铁华,等.钩虫流行现状及疫苗研制进展.中华寄生虫学及寄生虫病杂志,2000,18:182-1824.陈应文,揭盛华.预防日本血吸虫病口服药物研究进展.湖北预防医学杂志,1999,10(5):16-165.苏正明,胡敏,徐兴建,等.微波酶联免疫吸附试验诊断日本血吸虫病.中国血吸虫病防治杂志,2004,16(3):178-1786.谢闻悦.血吸虫致弱疫苗的发展现状及展望.中国寄生虫病防治杂志,2000,13(3):221-2217.娄培安.重组抗原在诊断疟疾上的应用.国外医学寄生虫分册,2000,27(6):248-2488.周家莲,杨恒林.抗疟药研究现状与发展趋势.中国病原生物学杂志,2008,3(11):865-8679.张述义.先天性弓形虫感染的产前研究的国内、外进展.中国优生与遗传杂志,2000,8(4):124-12410.张历浊.儿童棘阿米巴角膜炎1例.中国斜视与小儿眼科杂志,2001,9(1):311.黄德珉,叶鸿瑁,罗风珍,译.儿科感染性疾病(下).沈阳:辽宁教育出版社,200012.BehrmanR,KliegmanRM,JensonH.NelsonTextbookofPediatrics.16thedition.London:W.B.Saunderscompany,2000", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "寄生虫病" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "寄生虫病" }, { "start_idx": 43, "end_idx": 45, "type": "mic", "entity": "寄生虫" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "寄生虫病" }, { "start_idx": 79, "end_idx": 84, "type": "dis", "entity": "小儿鼻蛲虫病" }, { "start_idx": 93, "end_idx": 94, "type": "dep", "entity": "儿科" }, { "start_idx": 124, "end_idx": 125, "type": "mic", "entity": "钩虫" }, { "start_idx": 131, "end_idx": 132, "type": "dru", "entity": "疫苗" }, { "start_idx": 140, "end_idx": 142, "type": "mic", "entity": "寄生虫" }, { "start_idx": 145, "end_idx": 148, "type": "dis", "entity": "寄生虫病" }, { "start_idx": 179, "end_idx": 184, "type": "dis", "entity": "日本血吸虫病" }, { "start_idx": 185, "end_idx": 186, "type": "pro", "entity": "口服" }, { "start_idx": 234, "end_idx": 243, "type": "pro", "entity": "微波酶联免疫吸附试验" }, { "start_idx": 246, "end_idx": 251, "type": "dis", "entity": "日本血吸虫病" }, { "start_idx": 255, "end_idx": 258, "type": "dis", "entity": "血吸虫病" }, { "start_idx": 288, "end_idx": 294, "type": "dru", "entity": "血吸虫致弱疫苗" }, { "start_idx": 306, "end_idx": 309, "type": "dis", "entity": "寄生虫病" }, { "start_idx": 346, "end_idx": 347, "type": "dis", "entity": "疟疾" }, { "start_idx": 357, "end_idx": 359, "type": "mic", "entity": "寄生虫" }, { "start_idx": 391, "end_idx": 393, "type": "dru", "entity": "抗疟药" }, { "start_idx": 438, "end_idx": 445, "type": "dis", "entity": "先天性弓形虫感染" }, { "start_idx": 493, "end_idx": 501, "type": "dis", "entity": "儿童棘阿米巴角膜炎" }, { "start_idx": 507, "end_idx": 508, "type": "dis", "entity": "斜视" }, { "start_idx": 512, "end_idx": 513, "type": "dep", "entity": "眼科" }, { "start_idx": 547, "end_idx": 551, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "二、肺动脉瓣下狭窄孤立性弥漫性肺动脉瓣下狭窄而肺动脉瓣正常极其罕见。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "肺动脉瓣下狭窄" }, { "start_idx": 9, "end_idx": 21, "type": "dis", "entity": "孤立性弥漫性肺动脉瓣下狭窄" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "肺动脉瓣" } ] }, { "text": "纤维或纤维肌性环可出现在瓣下右心室流出道的任何水平,并造成梗阻。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "纤维" }, { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "纤维肌性环" }, { "start_idx": 12, "end_idx": 19, "type": "bod", "entity": "瓣下右心室流出道" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "梗阻" } ] }, { "text": "大的异常肌束还可将右室腔分成两个单独腔(右室双腔),并造成梗阻,常伴有室间隔缺损,也可单独存在。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肌束" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "右室腔" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "单独腔" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "梗阻" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "心肌肿瘤,特别是涉及室间隔的肿瘤,也可引起右心室流出道梗阻。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心肌肿瘤" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "室间隔" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肿瘤" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "右心室流出道梗阻" } ] }, { "text": "纠正性大动脉转位伴心房通常位患儿的30%~50%存在肺动脉瓣下形态学左室流出道的梗阻。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "纠正性大动脉转位" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "心房通常位" }, { "start_idx": 26, "end_idx": 41, "type": "dis", "entity": "肺动脉瓣下形态学左室流出道的梗阻" } ] }, { "text": "这种情况通常伴有室间隔缺损,也可室间隔完整。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "室间隔" } ] }, { "text": "上述病损的临床表现类似于肺动脉瓣狭窄,但喀喇音可听不到,瓣后肺动脉瓣扩张也可不明显或缺乏。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "喀喇音可听不到" }, { "start_idx": 28, "end_idx": 33, "type": "bod", "entity": "瓣后肺动脉瓣" }, { "start_idx": 28, "end_idx": 43, "type": "sym", "entity": "瓣后肺动脉瓣扩张也可不明显或缺乏" } ] }, { "text": "收缩期杂音在胸骨左缘第三、第四肋间最响。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "收缩期杂音" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "胸骨左缘" } ] }, { "text": "三、蛛网膜下腔出血原发性蛛网膜下腔出血(primarysubarachnoidhemorrhage)是新生儿常见的颅内出血类型,多见于早产儿,也可见于足月儿,前者主要与缺氧有关,后者则多由产伤所致。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "蛛网膜下腔出血" }, { "start_idx": 9, "end_idx": 18, "type": "dis", "entity": "原发性蛛网膜下腔出血" }, { "start_idx": 20, "end_idx": 48, "type": "dis", "entity": "primarysubarachnoidhemorrhage" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "颅内出血" }, { "start_idx": 84, "end_idx": 85, "type": "sym", "entity": "缺氧" } ] }, { "text": "新生儿蛛网膜下腔出血起源于软脑膜丛的小静脉或蛛网膜下腔的桥静脉。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "新生儿蛛网膜下腔出血" }, { "start_idx": 13, "end_idx": 20, "type": "bod", "entity": "软脑膜丛的小静脉" }, { "start_idx": 22, "end_idx": 30, "type": "bod", "entity": "蛛网膜下腔的桥静脉" } ] }, { "text": "【临床表现】轻度蛛网膜下腔出血可无症状或症状轻微。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "蛛网膜下腔出血" } ] }, { "text": "中度出血可引起惊厥,常开始于生后第2天,惊厥发作间期患儿情况良好。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "中度出血" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "惊厥" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "惊厥" } ] }, { "text": "大量蛛网膜下腔出血可致患儿病情迅速恶化和死亡。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "蛛网膜下腔出血" } ] }, { "text": "蛛网膜下腔出血的诊断常因其他原因腰穿发现均匀一致的血性脑脊液而提示,确诊需通过CT检查,头颅超声对蛛网膜下腔出血不够敏感。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "蛛网膜下腔出血" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "血性脑脊液" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "CT检查" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "头颅超声" }, { "start_idx": 49, "end_idx": 55, "type": "dis", "entity": "蛛网膜下腔出血" } ] }, { "text": "血性脑脊液是提示蛛网膜下腔或脑室内出血的一个线索,但需与腰椎穿刺损伤鉴别。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "血性脑脊液" }, { "start_idx": 8, "end_idx": 18, "type": "dis", "entity": "蛛网膜下腔或脑室内出血" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "腰椎穿刺" } ] }, { "text": "非急性期颅内出血的脑脊液特征为脑脊液黄色、红细胞数量增多和蛋白含量增高,脑脊液中糖常常降低(<30mg/dl),甚至可低达10mg/dl,并可持续数周甚至数月。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "非急性期颅内出血" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "脑脊液" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "脑脊液" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "红细胞" }, { "start_idx": 15, "end_idx": 34, "type": "sym", "entity": "脑脊液黄色、红细胞数量增多和蛋白含量增高" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "脑脊液" } ] }, { "text": "脑脊液中糖的降低可能系出血损伤葡萄糖向脑脊液转运的机制。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑脊液" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "脑脊液" } ] }, { "text": "当脑脊液糖降低,伴淋巴细胞增多和蛋白含量增高时,很难与细菌性脑膜炎鉴别。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "脑脊液" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "蛋白" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "细菌性脑膜炎" } ] }, { "text": "【治疗】新生儿原发性蛛网膜下腔出血预后较好,90%随访正常,治疗以对症为主,如有惊厥可用地西泮类药物抗惊厥。", "entities": [ { "start_idx": 4, "end_idx": 16, "type": "dis", "entity": "新生儿原发性蛛网膜下腔出血" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "惊厥" }, { "start_idx": 44, "end_idx": 49, "type": "dru", "entity": "地西泮类药物" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "惊厥" } ] }, { "text": "大量蛛网膜下腔出血的主要后遗症是出血后脑积水,但其发展过程比脑室内出血后脑积水缓慢,预后比脑室内出血好。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "蛛网膜下腔出血" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "出血" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "脑积水" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "脑积水" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "脑室内出血" } ] }, { "text": "蛛网膜下腔出血后脑积水的处理同脑室内出血。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "蛛网膜下腔出血" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "脑积水" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "脑室内出血" } ] }, { "text": "二、海绵状淋巴管瘤海绵状淋巴管瘤(cavernouslymphangioma)系由许多小的多房性腔隙组成,内含淋巴或血液混合的液体,腔隙脏壁衬有内皮细胞层,因有时混有血管瘤,又称淋巴血管瘤。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "海绵状淋巴管瘤" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "海绵状淋巴管瘤" }, { "start_idx": 17, "end_idx": 37, "type": "dis", "entity": "cavernouslymphangioma" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "淋巴" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "血液" }, { "start_idx": 72, "end_idx": 76, "type": "bod", "entity": "内皮细胞层" }, { "start_idx": 83, "end_idx": 85, "type": "dis", "entity": "血管瘤" }, { "start_idx": 89, "end_idx": 93, "type": "dis", "entity": "淋巴血管瘤" } ] }, { "text": "多见于上肢、颈、腋窝、肩胛、口腔和舌等处。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "上肢" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "颈" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "腋窝" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肩胛" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "口腔" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "舌" } ] }, { "text": "肿瘤的体积一般很大,侵入舌部的可引起巨舌,侵犯口腔则吞咽、呼吸甚至说话困难,肿瘤的表面有增厚的皮肤,易感染,与周围的组织、器官发生粘连。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "舌部" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "巨舌" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "口腔" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "吞咽、呼吸甚至说话困难" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "肿瘤" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "皮肤" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "感染" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "组织" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "器官" } ] }, { "text": "切除是主要的治疗方法,对有扩张趋势,或影响呼吸和进食的颈部、口底的巨大淋巴管瘤有绝对的手术指征。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "切除" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "颈部" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "口底" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "巨大淋巴管" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "手术" } ] }, { "text": "术中要注意出血和休克的应对措施。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "休克" } ] }, { "text": "尽量避免损伤血管和神经。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血管" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "神经" } ] }, { "text": "第十四章急性中毒【概述】毒物是指在一定条件下,以各种形式和剂量作用于人体,产生对人体有害的生物学反应和病理变化,导致机体功能严重损害甚至危及生命的物质,包括化学品、药物、植物和气体等。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "急性中毒" } ] }, { "text": "毒物进入人体后,在体内与体液、组织相互作用后可引起一系列中毒症状表现,组织代谢和器官功能障碍,严重者可导致患儿终身残疾或死亡。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "人体" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "体液" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "组织" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "中毒" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "中毒症状" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "组织" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "器官" }, { "start_idx": 35, "end_idx": 45, "type": "sym", "entity": "组织代谢和器官功能障碍" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "残疾" } ] }, { "text": "因摄入毒物而产生的一系列危及生命的病理生理改变和相应症状称为中毒。", "entities": [ { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "中毒" } ] }, { "text": "摄入毒物后数小时至数天内出现中毒表现者称为急性中毒。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "中毒" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "急性中毒" } ] }, { "text": "凡能引起中毒的物质均被视为毒物。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "中毒" } ] }, { "text": "由于年幼儿有一定的活动能力,但认知能力和生活经验不足,对某些毒物和药物的危害缺乏认识,因此中毒发生率在此年龄组较高。", "entities": [ { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "中毒" } ] }, { "text": "文献报道中,年龄小于5岁的中毒群体虽发病率较高,但大多属于无意中毒,其摄入的中毒物质剂量不大、毒物种类单一,其病死率低于青少年患者,而青少年患者有相当部分在存在精神抑郁或心理障碍情况下自伤性服毒,其服毒剂量通常较大,病死率相对较高。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "中毒" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "中毒" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "中毒" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "精神抑郁" }, { "start_idx": 85, "end_idx": 88, "type": "dis", "entity": "心理障碍" } ] }, { "text": "小儿发生中毒后被送至医院时,经常会遇到患儿家长不能准确提供毒物种类及毒物摄入量的病史,以致无法实施有针对性的解毒措施,可在短时间内导致病孩死亡。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "中毒" } ] }, { "text": "3.药物过量中毒许多药物(包括中药)过量均可导致中毒,如地高辛、抗癫痫药、退热药、麻醉镇静药、抗心律失常药等。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dru", "entity": "中药" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "中毒" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "地高辛" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "抗癫痫药" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "退热药" }, { "start_idx": 41, "end_idx": 45, "type": "dru", "entity": "麻醉镇静药" }, { "start_idx": 47, "end_idx": 52, "type": "dru", "entity": "抗心律失常药" } ] }, { "text": "此外,根据毒物的物理状态还可分为挥发性与非挥发性毒物,根据毒物吸收方式分为食入、吸入、皮肤接触吸收性毒物等。", "entities": [ { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "皮肤" } ] }, { "text": "进入途径以胃肠道最多,其他还有呼吸道、皮肤、五官、创口、注射等。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "胃肠道" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "呼吸道" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "皮肤" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "五官" } ] }, { "text": "毒物的作用部位直接影响中毒程度,当毒物作用于氧代谢、神经系统、心脏等代谢关键环节或重要脏器时,可使患者很快出现严重症状甚至死亡。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "神经系统" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "心脏" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "脏器" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "严重症状" } ] }, { "text": "大多数毒物进入体内经肝脏代谢转化后毒性减弱或消失,并由肾脏排泄,一些毒物亦可为原形经肾脏排泄。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肝脏" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肾脏" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "肾脏" } ] }, { "text": "少数毒物可由皮肤汗腺、乳腺、泪液、呼吸道、胆道或肠道排泄。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "皮肤汗腺" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "乳腺" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "泪液" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "呼吸道" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "胆道" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肠道" } ] }, { "text": "各毒物间的排泄速度差异很大,主要取决于毒物本身特性和患者肾脏功能,毒物排泄时间最长可达数周甚至数月。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肾脏" } ] }, { "text": "治疗中的促进毒物排泄方法对于中毒早期毒物大部分积聚于血流中的患者效果较好,当毒物的分布在体内达到平衡时,大多数毒物仅有5%左右存在于血液中,此时仅采用排泄治疗效果较差。", "entities": [ { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "血液" } ] }, { "text": "此外,毒物脂溶性高或血浆蛋白结合率高、中毒时毒物剂量较大、休克等因素亦会导致毒物排泄速度减慢。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "血浆蛋白" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "中毒" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "休克" } ] }, { "text": "但对毒物一时不能明确者,临床表现也有助于鉴别中毒的毒物种类和病情程度。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "中毒" } ] }, { "text": "生命体征及心、脑、肾等主要器官功能受损常提示中毒病情严重,以下是中毒时各系统常见的症状和表现。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "心" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "脑" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肾" }, { "start_idx": 5, "end_idx": 18, "type": "sym", "entity": "心、脑、肾等主要器官功能受损" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "中毒" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "中毒" } ] }, { "text": "表6-20常见中毒症状体征与可能毒物(一)消化系统在急性中毒时,胃肠道症状通常最为显著。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "中毒" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "中毒症状" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "消化系统" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "急性中毒" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "胃肠道" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "胃肠道症状" } ] }, { "text": "毒物大多数均为食入中毒,少数为非食入中毒。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "食入中毒" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "非食入中毒" } ] }, { "text": "因此,对于小儿不明原因下突然出现急性消化道症状,应注意鉴别是否存在中毒。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "消化道" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "急性消化道症状" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "中毒" } ] }, { "text": "肝脏是毒物代谢转化的主要场所,由消化道进入的毒物,大多经肝脏代谢后毒性下降或失去毒性。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肝脏" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "消化道" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肝脏" } ] }, { "text": "肝脏受到毒物侵犯后可发生不同程度的损害,出现黄疸、肝炎症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肝脏" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "黄疸" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "肝炎" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "黄疸、肝炎症状" } ] }, { "text": "原先有肝功能障碍者可因解毒功能下降而使中毒症状加重。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肝功能障碍" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "中毒" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "中毒症状" } ] }, { "text": "(二)循环系统大部分中毒患儿均会出现循环系统症状,如心动过速、周围循环灌注变差等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "循环系统" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "中毒" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "循环系统" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "循环系统症状" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "心动过速" }, { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "周围循环灌注变差" } ] }, { "text": "自主神经对心血管系统影响较大,具有交感神经激动作用的毒物可使血压升高、心率快速和心律失常,而拟副交感神经毒物则会引起心动过缓。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "自主神经" }, { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "心血管系统" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "交感神经" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "血压升高" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "心率快速" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "心律失常" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "交感神经" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "心动过缓" } ] }, { "text": "(三)呼吸系统许多毒物(包括吸入有毒气体)会损害呼吸系统功能。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "呼吸系统" } ] }, { "text": "(四)泌尿系统肾脏是毒物和毒物代谢产物排泄的主要器官,中毒后循环、呼吸障碍导致的肾脏缺血缺氧,可引起不同程度的肾脏损害症状,表现为血尿、蛋白尿、水肿、尿量减少等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肾脏" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "中毒" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "呼吸障碍" }, { "start_idx": 40, "end_idx": 45, "type": "sym", "entity": "肾脏缺血缺氧" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "肾脏损害" }, { "start_idx": 55, "end_idx": 60, "type": "sym", "entity": "肾脏损害症状" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "血尿" }, { "start_idx": 68, "end_idx": 70, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "水肿" }, { "start_idx": 75, "end_idx": 76, "type": "ite", "entity": "尿量" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "尿量减少" } ] }, { "text": "部分毒物还具有选择性的肾脏毒性,直接损害肾脏。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肾脏" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肾脏" } ] }, { "text": "肾脏损害中以急性肾衰竭最为严重。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "肾脏损害" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "后者通常表现为短期内出现尿闭、高血压、氮质血症,重者还可出现意识改变、抽搐和急性肺水肿。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "尿闭" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "高血压" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "氮质血症" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "意识改变" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "抽搐" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "肺" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "急性肺水肿" } ] }, { "text": "(五)神经系统中枢神经系统是人体高级生命活动器官和调节机体生理功能的重要器官。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经系统" }, { "start_idx": 7, "end_idx": 12, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "瞳孔是脑功能观察的重要体征,并可在一定程度上鉴别毒物种类和脑功能状况。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "瞳孔" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "脑" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "脑" } ] }, { "text": "吗啡、乙醇、有机磷等中毒时,瞳孔通常显著缩小;而曼陀罗类、镇静剂中毒时,则瞳孔扩大。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "瞳孔" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "瞳孔通常显著缩小" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "瞳孔扩大" } ] }, { "text": "瞳孔扩大伴对光反应消失提示脑功能损害严重。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "瞳孔" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "瞳孔扩大伴对光反应消失" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "脑功能损害" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "脑功能损害严重" } ] }, { "text": "(六)其他有些毒物能抑制骨髓造血功能,破坏红细胞,引起贫血、溶血等。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "骨髓" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "红细胞" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "贫血" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "溶血" } ] }, { "text": "应激、休克和缺氧还可诱发DIC,引起皮肤、消化道等部位广泛出血。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "应激" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "休克" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "DIC" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "皮肤" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "消化道" }, { "start_idx": 18, "end_idx": 30, "type": "sym", "entity": "皮肤、消化道等部位广泛出血" } ] }, { "text": "细胞呼吸抑制剂可引起细胞能量代谢障碍而死亡(如氰化物)。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "细胞能量代谢障碍" } ] }, { "text": "临床医师可以根据中毒患者的面容、呼出气味、症状、其他体征、排泄物的性状等,结合病史,综合分析,得出初步诊断;此外,还可根据所在地域流行病学发病率较高的中毒毒物进行筛选和鉴别。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "中毒" } ] }, { "text": "各种毒物引起的急性中毒常具有各自的特征,这些特征是中毒诊断的重要线索和依据。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "急性中毒" } ] }, { "text": "采集标本应及早进行,包括原毒物样品、胃液或呕吐物、血液、尿液等。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "胃液" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血液" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "尿液" } ] }, { "text": "毒物确定后,还必须了解毒物服用剂量、发病时间和脏器受累表现以及就诊前处理等,以便确定相应的处理方案。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "脏器" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "脏器受累" } ] }, { "text": "对于这些患儿,应常规监测肝、肾等各脏器功能,为病情判断和支持处理提供依据。", "entities": [ { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肝" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "肾" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "脏器" } ] }, { "text": "【治疗】(一)对于存在呼吸、心搏骤停、休克、惊厥的患儿应首先现场进行抢救,保持良好的氧供和循环,严密监护并维持生命体征稳定,纠正内环境紊乱。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "呼吸、心搏骤停" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "休克" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "惊厥" } ] }, { "text": "对存在呼吸衰竭者应及早给予呼吸支持。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "呼吸衰竭" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "呼吸支持" } ] }, { "text": "血压不稳者给予多巴胺5~10μg/(kg•min)静脉维持。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "血压" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "血压不稳" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "多巴胺" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "静脉维持" } ] }, { "text": "(二)清除毒物1.清除尚未吸收的毒物其目的为防止已与体表、体腔接触,但尚未进入体内的毒物,以最大限度降低毒物进入体内的量和减轻病情。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "体表" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "体腔" } ] }, { "text": "洗胃一般于食入毒物后4~6小时内进行,操作时动作要轻巧迅速,危重患者取平卧位,头偏向一侧,胃内容物要尽量抽净,反复灌洗,直至洗出胃液清晰为止。", "entities": [ { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "胃" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "胃液" } ] }, { "text": "洗胃过程中应注意观察患者的反应和呕吐情况,防止胃内容物反流入肺内引起窒息。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "洗胃" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "呕吐" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "胃" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "肺" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "窒息" } ] }, { "text": "洗胃后可随即给患者50%硫酸镁0.5ml/kg导泻,加速毒物从肠道排出,或胃内注入活性炭0.5~1g/kg吸附毒物。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "洗胃" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "硫酸镁" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "导泻" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "胃" }, { "start_idx": 39, "end_idx": 43, "type": "pro", "entity": "注入活性炭" } ] }, { "text": "对强酸、强碱中毒或惊厥者不宜用洗胃、催吐及导泻法,以免发生消化道穿孔。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "中毒" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "洗胃" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "催吐" }, { "start_idx": 21, "end_idx": 23, "type": "pro", "entity": "导泻法" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "消化道穿孔" } ] }, { "text": "近来国外文献报道胃肠道脱毒并不能显著改变患者的症状、病程和预后。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "胃肠道" } ] }, { "text": "许多学者不主张采用催吐法治疗小儿急性中毒。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "催吐法" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "小儿急性中毒" } ] }, { "text": "但也有人认为食入毒物一小时内洗胃才有效。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "洗胃" } ] }, { "text": "如遇皮肤接触毒物者(如有机磷农药中毒)应立即脱去全部衣裤,以微温清水冲洗全身,并注意五官、毛发、指甲部位的清洗,一般洗5~15分钟左右。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "皮肤" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "中毒" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "五官" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "毛发" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "指甲" } ] }, { "text": "对体表不溶于水的毒物,可用适当溶剂,例如用10%乙醇或植物油冲洗酚类毒物,也可用适当的解毒剂加入水中冲洗。", "entities": [ { "start_idx": 43, "end_idx": 45, "type": "dru", "entity": "解毒剂" } ] }, { "text": "(2)高锰酸钾:系氧化剂,可与各种有机物相互作用,使巴比妥、阿片镇痛药、士的宁、毒扁豆碱、奎宁及烟碱等药物失活。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "巴比妥" }, { "start_idx": 30, "end_idx": 34, "type": "dru", "entity": "阿片镇痛药" }, { "start_idx": 36, "end_idx": 38, "type": "dru", "entity": "士的宁" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "毒扁豆碱" }, { "start_idx": 45, "end_idx": 46, "type": "dru", "entity": "奎宁" }, { "start_idx": 48, "end_idx": 49, "type": "dru", "entity": "烟碱" } ] }, { "text": "由于高锰酸钾对皮肤黏膜有刺激作用,使用时切勿将高锰酸钾结晶直接接触口腔及胃黏膜。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "皮肤黏膜" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "口腔" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "胃黏膜" } ] }, { "text": "美曲膦酯中毒不可采用本溶液,后者可使毒物毒性增强。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "美曲膦酯中毒" } ] }, { "text": "(4)活性炭(10%~20%)悬液:可强力吸附多种药物和化学物质,能吸附的物质范围较广,能有效地阻止药物在胃肠道中的吸收,适用于有机及无机毒物中毒,但对氟化物无效。", "entities": [ { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "胃肠道" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "中毒" } ] }, { "text": "目前,国内外大多数提倡采用此悬液进行洗胃。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "洗胃" } ] }, { "text": "临床用于氟化物或草酸盐中毒。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "dis", "entity": "氟化物或草酸盐中毒" } ] }, { "text": "(6)氧化镁溶液(4%):为弱碱性溶液,可中和酸性物质,用于阿司匹林、强酸及草酸等中毒。", "entities": [ { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "中毒" } ] }, { "text": "(7)米汤、稀面糊(含1%~10%淀粉):能结合并还原碘,使之失活,用于碘中毒。", "entities": [ { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "碘中毒" } ] }, { "text": "操作时应彻底洗胃,至洗出液清晰,不显蓝色为止。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "洗胃" } ] }, { "text": "(8)氯化钠1%~2%溶液:常用于毒物不明的急性中毒。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "急性中毒" } ] }, { "text": "2.促进毒物排泄大多毒物由肾脏排泄,积极利尿有利于加速毒物排泄,可每天酌情给予5%葡萄糖电解质溶液500~1000ml或10~20ml/(kg•d)静滴,同时静脉注射呋塞米(速尿)每次1mg/kg。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肾脏" }, { "start_idx": 41, "end_idx": 48, "type": "dru", "entity": "葡萄糖电解质溶液" }, { "start_idx": 74, "end_idx": 75, "type": "pro", "entity": "静滴" }, { "start_idx": 79, "end_idx": 82, "type": "pro", "entity": "静脉注射" }, { "start_idx": 83, "end_idx": 85, "type": "dru", "entity": "呋塞米" }, { "start_idx": 87, "end_idx": 88, "type": "dru", "entity": "速尿" } ] }, { "text": "经补液及给利尿剂后,水溶性和与蛋白结合很弱的化合物(如苯巴比妥、甲丙氨酯、苯丙胺等)较易从体内排出。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "利尿剂" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "蛋白" } ] }, { "text": "有些化合物(如巴比妥酸盐、水杨酸盐及异烟肼等)在碱性环境下离子化程度增加,处理时如在补液中适当补给碳酸氢钠以碱化尿液,可减少其在肾小管内重吸收,提高排出率。", "entities": [ { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "尿液" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "肾小管" } ] }, { "text": "血液透析对于水溶性较高和蛋白结合率不高的毒物排泄效果较好,如对乙酰氨基酚、水杨酸盐、非那西汀、苯巴比妥、甲丙氨酯、水合氯醛、海洛因、甲醇、乙醇、乙二醇、异丙醇、苯丙胺、锂盐、异烟肼、苯妥英钠、钾、铁、锌、铜、硼酸盐等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "血液透析" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "蛋白" } ] }, { "text": "脂溶性毒物及与蛋白质结合紧密的毒物则透析效果较差,如速效巴比妥盐类、阿米替林及地西泮等。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "蛋白质" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "透析" } ] }, { "text": "血液净化适应证为:①急性中毒其症状严重,无相应解毒剂,但毒物或其代谢产物能被透析出体外;②预计毒物摄入剂量很大,估计会出现严重并发症和严重不良后果;③发生急性肾衰竭。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血液" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "急性中毒" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "急性中毒其症状严重" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "解毒剂" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "透析" }, { "start_idx": 61, "end_idx": 65, "type": "sym", "entity": "严重并发症" }, { "start_idx": 67, "end_idx": 72, "type": "sym", "entity": "严重不良后果" }, { "start_idx": 77, "end_idx": 81, "type": "sym", "entity": "急性肾衰竭" } ] }, { "text": "血液净化相对禁忌证为:①严重心功能不全;②有严重贫血、血小板减少低于50×109/L、出血倾向,或全身应用抗凝药物;③休克虽经治疗仍不能维持收缩血压在12kPa以上。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "心功能不全" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "贫血" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "血小板" }, { "start_idx": 27, "end_idx": 52, "type": "sym", "entity": "血小板减少低于50×109/L" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "出血倾向" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "休克" }, { "start_idx": 81, "end_idx": 84, "type": "ite", "entity": "收缩血压" }, { "start_idx": 81, "end_idx": 92, "type": "sym", "entity": "收缩血压在12kPa以上" } ] }, { "text": "一般药物及毒物中毒的透析治疗应争取在中毒后8~16小时内进行,严重中毒者在3小时内进行效果更好,因为大多数毒物的血液浓度在此时段内浓度处于高峰阶段,透析可以达到最佳效果。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "透析治疗" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "中毒" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "血液" }, { "start_idx": 74, "end_idx": 75, "type": "pro", "entity": "透析" } ] }, { "text": "腹膜透析具有安全、方便、对循环影响较小等特点,在儿科中较血液透析应用更广,其应用指征同血液透析。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "血液透析" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "血液透析" } ] }, { "text": "其透析特性与血液透析相似,但小分子物质透析效果较血液透析略差。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "透析" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "血液透析" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "透析" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "血液透析" } ] }, { "text": "腹膜透析的禁忌证为:①腹腔感染、肠梗阻、腹膜广泛粘连、腹壁皮肤感染;②严重呼吸衰竭;③腹腔手术后3天以内。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "腹腔感染" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "腹膜广泛粘连" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "腹壁皮肤感染" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "严重呼吸衰竭" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "腹腔" } ] }, { "text": "腹膜透析方法为每次注入30~40ml/kg,保留30~45分钟后排出,每天4~8次,直至病情缓解。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "腹膜透析" } ] }, { "text": "对于中毒严重、毒物不能通过透析等方法得到有效排除者,早期还可采用换血或多次部分换血(每次换血量10ml/kg)的处理来达到排除体内毒物的目的。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "中毒" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "中毒严重" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "透析" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "换血" }, { "start_idx": 35, "end_idx": 40, "type": "pro", "entity": "多次部分换血" } ] }, { "text": "3.尽早使用有效拮抗剂这些药物目前种类很有限,仅少数有特异性拮抗剂(或解毒剂),一些常用拮抗剂见表6-6。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "拮抗剂" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "拮抗剂" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "解毒剂" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "拮抗剂" } ] }, { "text": "表6-21一些常见毒物的拮抗剂(三)对症处理适当镇静,避免烦躁;控制惊厥;高热者给予物理及药物退热;预防继发感染;维持水、电解质及酸碱平衡;积极防治各重要脏器功能衰竭。", "entities": [ { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "惊厥" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "高热" }, { "start_idx": 42, "end_idx": 48, "type": "pro", "entity": "物理及药物退热" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "继发感染" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "脏器" } ] }, { "text": "肾上腺皮质激素具有增强机体应激能力、改善毛细血管通透性、减少渗出、稳定细胞膜及溶酶体、减少细胞损害等作用,临床用于严重中毒伴中毒性脑病、肺水肿、急性呼吸窘迫综合征、中毒性肝肾功能损害以及化学物引起的溶血性贫血等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "肾上腺皮质激素" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "毛细血管" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "细胞膜" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "溶酶体" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "细胞" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "中毒" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "中毒性脑病" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "肺水肿" }, { "start_idx": 72, "end_idx": 80, "type": "dis", "entity": "急性呼吸窘迫综合征" }, { "start_idx": 82, "end_idx": 90, "type": "dis", "entity": "中毒性肝肾功能损害" }, { "start_idx": 99, "end_idx": 103, "type": "dis", "entity": "溶血性贫血" } ] }, { "text": "药物可选择地塞米松0.5mg/kg或琥珀酰氢化可的松5~10mg/kg,每天1~2次,静脉注射,疗程3~5天。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "地塞米松" }, { "start_idx": 18, "end_idx": 25, "type": "dru", "entity": "琥珀酰氢化可的松" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "静脉注射" } ] }, { "text": "(四)小儿一些常见药物中毒的主要处理原则1.水杨酸盐以5%碳酸氢钠或1∶5000高锰酸钾溶液洗胃;5%碳酸氢钠6ml/kg及维生素K110mg,每天1次静滴,补液利尿,碱化尿液。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dru", "entity": "中毒" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "洗胃" }, { "start_idx": 51, "end_idx": 54, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 87, "end_idx": 88, "type": "pro", "entity": "静滴" } ] }, { "text": "2.巴比妥类及苯二氮类1∶5000高锰酸钾洗胃;安钠咖6~12mg/kg,肌内注射;或贝美格1mg/kg,静注;或纳洛酮0.01mg/kg,肌内注射;4~6小时后可重复1次,直至神志转清。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "安钠咖" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "肌内注射" }, { "start_idx": 43, "end_idx": 45, "type": "dru", "entity": "贝美格" }, { "start_idx": 53, "end_idx": 54, "type": "pro", "entity": "静注" }, { "start_idx": 57, "end_idx": 59, "type": "dru", "entity": "纳洛酮" }, { "start_idx": 70, "end_idx": 73, "type": "pro", "entity": "肌内注射" }, { "start_idx": 89, "end_idx": 90, "type": "ite", "entity": "神志" }, { "start_idx": 89, "end_idx": 92, "type": "sym", "entity": "神志转清" } ] }, { "text": "注意呼吸和循环支持。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "呼吸和循环支持" } ] }, { "text": "3.颠茄类以1∶5000高锰酸钾洗胃、硫酸镁导泻;用毛果芸香碱0.1~0.2mg/kg,每1~4小时皮下注射1次;或新斯的明0.02~0.04mg/kg,每3~4小时肌内注射1次。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "洗胃" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "导泻" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "毛果芸香碱" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "皮下注射" }, { "start_idx": 58, "end_idx": 61, "type": "pro", "entity": "新斯的明" }, { "start_idx": 83, "end_idx": 86, "type": "pro", "entity": "肌内注射" } ] }, { "text": "直至瞳孔缩小。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "瞳孔" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "瞳孔缩小" } ] }, { "text": "4.氨茶碱反复以1∶5000高锰酸钾洗胃、导泻,洗胃后胃管内注入活性炭,注意镇静止痉、纠正低血钾、休克及心律失常、补液利尿。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "洗胃" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "导泻" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "洗胃" }, { "start_idx": 27, "end_idx": 28, "type": "equ", "entity": "胃管" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "注入活性炭" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "低血钾" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "休克" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "心律失常" } ] }, { "text": "5.麻黄碱氯丙嗪1mg/kg,肌内注射;或酚妥拉明每分钟1~3μg/kg,静脉滴注维持,血压正常后逐步撤除。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "氯丙嗪" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "肌内注射" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "酚妥拉明" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 44, "end_idx": 45, "type": "ite", "entity": "血压" } ] }, { "text": "6.乙醇中毒1小时内用温开水或2%NaHCO3洗胃,静注25%~50%高渗葡萄糖20~40ml/次,纠正电解质紊乱和酸中毒,并补充维生素B1及维生素B6。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "中毒" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "洗胃" }, { "start_idx": 37, "end_idx": 38, "type": "pro", "entity": "静注" }, { "start_idx": 46, "end_idx": 50, "type": "dru", "entity": "高渗葡萄糖" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "电解质紊乱" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "酸中毒" } ] }, { "text": "伴肝肾功能障碍者可考虑透析治疗。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "肝肾功能障碍" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "透析" } ] }, { "text": "7.氯丙嗪以1∶5000高锰酸钾反复洗胃、导泻,洗胃后可胃管内注入活性炭,保暖,减少搬动,防止直立性休克;酌情给予升压药(多巴胺或间羟胺),控制惊厥,呼吸支持。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "洗胃" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "导泻" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "洗胃" }, { "start_idx": 28, "end_idx": 29, "type": "equ", "entity": "胃管" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "注入活性炭" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "直立性休克" }, { "start_idx": 57, "end_idx": 59, "type": "dru", "entity": "升压药" }, { "start_idx": 61, "end_idx": 63, "type": "dru", "entity": "多巴胺" }, { "start_idx": 65, "end_idx": 67, "type": "dru", "entity": "间羟胺" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "惊厥" }, { "start_idx": 75, "end_idx": 78, "type": "pro", "entity": "呼吸支持" } ] }, { "text": "8.蟾蜍以1∶5000高锰酸钾洗胃、导泻(忌用油类泻药),维生素C1~2g用葡萄糖溶液稀释后静滴,纠正心律失常(阿托品、肾上腺素),地塞米松0.25~0.5mg/kg,静注;防治心源性休克。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "洗胃" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "导泻" }, { "start_idx": 25, "end_idx": 26, "type": "dru", "entity": "泻药" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "维生素C" }, { "start_idx": 38, "end_idx": 42, "type": "dru", "entity": "葡萄糖溶液" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "静滴" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "心律失常" }, { "start_idx": 56, "end_idx": 58, "type": "dru", "entity": "阿托品" }, { "start_idx": 60, "end_idx": 63, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 66, "end_idx": 69, "type": "dru", "entity": "地塞米松" }, { "start_idx": 84, "end_idx": 85, "type": "pro", "entity": "静注" }, { "start_idx": 89, "end_idx": 93, "type": "sym", "entity": "心源性休克" } ] }, { "text": "毒液接触眼者用生理盐水或3%硼酸液局部冲洗。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "眼" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "生理盐水" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "硼酸液" } ] }, { "text": "9.毒蕈类以1∶5000高锰酸钾或活性炭悬液反复洗胃,硫酸镁导泻;用二巯基丙磺酸钠每次5mg/kg,每天2~3次,肌内注射;阿托品0.05mg/kg,肌内注射,每15分钟1次,直至阿托品化后减量。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "洗胃" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "硫酸镁" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "导泻" }, { "start_idx": 34, "end_idx": 40, "type": "dru", "entity": "二巯基丙磺酸钠" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "肌内注射" }, { "start_idx": 62, "end_idx": 64, "type": "dru", "entity": "阿托品" }, { "start_idx": 75, "end_idx": 78, "type": "pro", "entity": "肌内注射" }, { "start_idx": 90, "end_idx": 92, "type": "dru", "entity": "阿托品" } ] }, { "text": "10.氰化物给予吸氧及生命支持。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "吸氧" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "生命支持" } ] }, { "text": "食入者用1∶5000高锰酸钾、5%硫代硫酸钠、3%过氧化氢及0.5%活性炭悬液洗胃;立即吸入亚硝酸异戊酯0.2~0.4ml/次,吸30秒钟,5分钟可重复1次;随后静注3%亚硝酸钠6~12mg/kg,再静注25%硫代硫酸钠溶液0.25g/kg(每药均推注10~15分钟);效果不满意时可重复使用。", "entities": [ { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "洗胃" }, { "start_idx": 46, "end_idx": 51, "type": "dru", "entity": "亚硝酸异戊酯" }, { "start_idx": 81, "end_idx": 82, "type": "pro", "entity": "静注" }, { "start_idx": 85, "end_idx": 88, "type": "dru", "entity": "亚硝酸钠" }, { "start_idx": 100, "end_idx": 101, "type": "pro", "entity": "静注" }, { "start_idx": 105, "end_idx": 109, "type": "dru", "entity": "硫代硫酸钠" } ] }, { "text": "无硫代硫酸钠时可用1%亚甲蓝1~2mg/kg缓慢静注代替。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dru", "entity": "硫代硫酸钠" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "亚甲蓝" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "静注" } ] }, { "text": "11.汞中毒(误食)立即用5%活性炭悬液或2%NaHCO3洗胃,并口服牛奶或蛋清;硫酸镁导泻。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "汞中毒" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "洗胃" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "导泻" } ] }, { "text": "有全身中毒症状者可用5%二巯基丙磺酸钠0.1~0.2ml/kg肌内注射,每天一次。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "全身" }, { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "中毒" }, { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "全身中毒症状" }, { "start_idx": 12, "end_idx": 18, "type": "dru", "entity": "二巯基丙磺酸钠" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "肌内注射" } ] }, { "text": "肾衰竭者给予透析治疗。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "透析" } ] }, { "text": "12.铅中毒急性食入中毒者以1%硫酸钠溶液洗胃,并口服牛奶或蛋清;及早应用依地酸钙钠(EDTA)25~50mg/kg,稀释至500ml葡萄糖液静滴,或二巯丁二钠20~30mg/kg静脉缓注。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "铅中毒" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "急性食入中毒" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "洗胃" }, { "start_idx": 37, "end_idx": 41, "type": "dru", "entity": "依地酸钙钠" }, { "start_idx": 43, "end_idx": 46, "type": "dru", "entity": "EDTA" }, { "start_idx": 67, "end_idx": 70, "type": "dru", "entity": "葡萄糖液" }, { "start_idx": 71, "end_idx": 72, "type": "pro", "entity": "静滴" }, { "start_idx": 75, "end_idx": 79, "type": "dru", "entity": "二巯丁二钠" }, { "start_idx": 90, "end_idx": 93, "type": "pro", "entity": "静脉缓注" } ] }, { "text": "13.一氧化碳中毒开窗通风或脱离中毒环境,吸氧,补充大量维生素C。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "一氧化碳中毒" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "中毒" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "吸氧" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "维生素C" } ] }, { "text": "伴昏迷或抽搐者提示病情较重,应及早给予高压氧治疗,并控制惊厥,提供生命支持。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "昏迷" }, { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "抽搐" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "高压氧治疗" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "惊厥" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "生命支持" } ] }, { "text": "甘露醇0.5g/kg静注,每天2~4次,胞磷胆碱每次0.125~0.25g,静滴。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "甘露醇" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "静注" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "胞磷胆碱" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "静滴" } ] }, { "text": "14.强酸碱类皮肤、五官等接触部分用清水冲洗,去除衣物。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "皮肤" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "五官" } ] }, { "text": "若非大量毒物食入,一般禁止洗胃及;强酸中毒可口服4%氢氧化铝10~20ml或蛋清、豆浆等;强碱类则口服1%醋酸或食醋等。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "洗胃" }, { "start_idx": 16, "end_idx": 15, "type": "pro", "entity": "" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "强酸中毒" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "氢氧化铝" } ] }, { "text": "注意生命功能支持,症状严重者可给予地塞米松0.25~0.5mg/kg,静注。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "地塞米松" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "静注" } ] }, { "text": "15.有机磷以2%碳酸氢钠溶液(DDT中毒忌用)或1∶5000高锰酸钾(马拉硫磷中毒忌用)洗胃,硫酸镁导泻。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "DDT中毒" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "马拉硫磷中毒" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "洗胃" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "硫酸镁" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "导泻" } ] }, { "text": "皮肤接触者用清水或苏打水冲洗皮肤及五官,去除衣物。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "皮肤" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "皮肤" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "五官" } ] }, { "text": "阿托品0.05~0.1mg/kg,静注,以后改0.05mg/kg,每10分钟1次,直至瞳孔散大后改0.02~0.03mg/kg,每30分钟1次,神志恢复后减为0.01~0.02mg/kg,每小时1次,以后根据病情逐渐延长给药间隔,直至停用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "阿托品" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "静注" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "瞳孔" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "瞳孔散大" }, { "start_idx": 72, "end_idx": 73, "type": "ite", "entity": "神志" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "神志恢复" } ] }, { "text": "治疗中应注意控制惊厥并防治呼吸衰竭。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "惊厥" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "16.鼠药(氟乙酸钠、氟化钠)用0.2~0.5%氯化钠洗胃,每天肌注乙酰胺0.1~0.3g/kg,分2~3次,疗程一周左右。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "洗胃" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "肌注" }, { "start_idx": 34, "end_idx": 36, "type": "dru", "entity": "乙酰胺" } ] }, { "text": "控制惊厥。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "惊厥" } ] }, { "text": "17.阿片口服中毒者可予1∶5000高锰酸钾或活性炭悬液洗胃;纳洛酮0.01mg/kg肌内注射或静注,数分钟后可重复应用;给予呼吸及生命支持;抗惊厥;防治脑水肿及肺水肿。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "中毒" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "洗胃" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "纳洛酮" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "肌内注射" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "静注" }, { "start_idx": 63, "end_idx": 69, "type": "pro", "entity": "呼吸及生命支持" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "惊厥" }, { "start_idx": 77, "end_idx": 79, "type": "dis", "entity": "脑水肿" }, { "start_idx": 81, "end_idx": 83, "type": "dis", "entity": "肺水肿" } ] }, { "text": "18.亚硝酸盐中毒6小时内给予洗胃、导泄,清除胃内余下毒物。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "中毒" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "洗胃" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "导泄" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "胃" } ] }, { "text": "1%亚甲蓝以每次0.1~0.2ml/kg,稀释后缓慢推注,或维生素C以0.5~1g用葡萄糖20ml稀释后静注;气促者给予吸氧。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "亚甲蓝" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "推注" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "维生素C" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "静注" } ] }, { "text": "19.毒蛇咬伤中毒伤肢制动,以减慢毒素扩散;肢体伤口的近心端2~3cm处用绳或布条缚扎,每15~30分钟放松1~2分钟。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "中毒" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "伤肢制动" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肢体" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "缚扎" } ] }, { "text": "20.蜂、蝎及毒蜘蛛螫伤中毒拔除伤口内毒刺,以3%氨水或苏打水等碱性溶液洗敷伤口。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "中毒" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "洗敷" } ] }, { "text": "毒蝎、毒蜘蛛螫伤者应在缚扎伤口近心端肢体,并扩创排毒(同毒蛇咬伤)。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "缚扎" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "扩创" } ] }, { "text": "参考文献1.宋家其.11例小儿急性胰腺炎治疗体会和预后评估.临床儿科杂志,1997,15(4):251-2512.施诚仁.儿童急性胰腺炎的概念与认识.中华小儿外科杂志,1997,18(2):124-1243.吴曰杰,郭永年,王开荣,等.小儿及青少年钙化性胰腺炎.实用儿科临床杂志,1996,11(6):366-3664.王志敏.青少年慢性胰腺炎.国外医学•儿科学分册,1995,22(3):1565.萧树东.江绍基胃肠病学.上海:上海科学技术出版社,2001:4876.徐家裕.临床胰腺病学.上海:上海科学技术出版社,1990:1207.徐家裕.急性重症胰腺炎的诊断进展.临床儿科杂志,1997,15(4):244-2448.姚希贤.临床消化病学.天津:天津科学技术出版社,1999:12789.徐家裕.急性重症胰腺炎的治疗进展.临床儿科杂志,1997,15(5):310-31010.杨镇主译,裘法祖审阅.慢性胰腺炎.北京:人民卫生出版社,1994:2111.叶孝礼.小儿消化系统疾病学.天津:天津科学技术出版社,1992:22612.张富生,周世杰.丙戊酸治疗10年后引起坏死性胰腺炎.国外医学•儿科学分册,1996,23(3):15513.张汝慧,王玲,陈俊仪,等.流行性腮腺炎致多脏器损害662例临床分析.临床儿科杂志,1996,14(4):220-22014.赵惠君,王耀平,顾龙君,等.左旋门冬酰胺酶在儿童淋巴系统恶性肿瘤化疗中的严重毒副反应.临床儿科杂志,1998,16(5):301-30115.周雪莲,欧弼悠.小儿急性坏死性胰腺炎16例诊治分析.急诊医学,1996,5(2)∶11416.BraganzaJM:Thepathogenesisofchronicpancreatitis.QJMed,1996,89:243-24317.HaddochG,CouparG,YoungsonGG,etal:Acutepancreatitisinchildren:a15yearreview.JPediatrSurg,1994,29(6):719-71918.KeimV,IovannaJL,DagornJC:Theacutephasereactionoftheexocrinepancreas.Digestion,1994,55:65-6519.LehmanGA,ShermanS.Pancreasdivisum.GastrointestEndoscClinNorthAm,1995,5(1):145-14520.MathewP,WyllieR,CaulfieldM,etal:Chronicpancreatitisinlatechildhoodandadolescence.ClinPediatr,1994,33:88-8821.ParentiDM,SteinbergW,KangP:Infectiouscausesofacutepancreatitis.Pancreas,1996,13(4):356-35622.PerraultJ.Hereditarypancreatitis.GastroenterolClinNorthAm,1994,23(4):743-74323.SteerML,WaxmanI,FreedmanS.ChronicPancreatitis.NEnglJMed,1995,332(22):1482-1482", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 127, "end_idx": 129, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 169, "end_idx": 171, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 277, "end_idx": 279, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 356, "end_idx": 358, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 406, "end_idx": 408, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 491, "end_idx": 493, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 671, "end_idx": 673, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "第十节冠状动脉异常冠状动脉畸形种类很多,包括起源、通路和走向的异常,重者可影响心肌的供血,轻者在心脏手术时可因误伤而使手术失败。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "冠状动脉异常" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "冠状动脉畸形" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "心肌的供血" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "心脏" } ] }, { "text": "(一)左冠状动脉异常畸形左冠状动脉起源于肺动脉,右冠状动脉起源正常。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "左冠状动脉异常" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "畸形左冠状动脉" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肺动脉" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "右冠状动脉" } ] }, { "text": "在胎儿期,由于肺动脉与主动脉的压力和氧饱和度相似,心肌的灌注与氧合正常。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肺动脉" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "主动脉" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "心肌" } ] }, { "text": "然而,出生后,随着肺动脉压力的降低,血液可自右冠状动脉经侧支进入左冠状动脉,最终进入肺动脉。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肺动脉" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "右冠状动脉" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "左冠状动脉" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "肺动脉" } ] }, { "text": "这样就产生了少量的左向右分流,应该供应心肌的血液通过这些通道进入了肺动脉。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心肌" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血液" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "肺动脉" } ] }, { "text": "通常影响最多的是左室的前侧壁。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "左室的前侧壁" } ] }, { "text": "临床表现一般与心力衰竭有关,后者由心肌缺血甚或心肌梗死引起。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "心肌" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心肌" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "心肌梗死" } ] }, { "text": "常可听到第三心音与第四心音。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "第三心音" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "第四心音" } ] }, { "text": "心电图显示前侧壁梗死图形,表现为Ⅰ、aVF及左胸导联宽深的Q波,同时伴有这些导联的持续性ST段及T波变化。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "心电图" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "前侧壁梗死" }, { "start_idx": 16, "end_idx": 30, "type": "sym", "entity": "Ⅰ、aVF及左胸导联宽深的Q波" }, { "start_idx": 41, "end_idx": 51, "type": "sym", "entity": "持续性ST段及T波变化" } ] }, { "text": "左心室电势通常增加。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "左心室电势通常增加" } ] }, { "text": "由于本病可根治,因此凡遇婴儿不明原因的左心衰竭应进行这一方面的检查。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "左心衰竭" } ] }, { "text": "二维超声心动图可怀疑本病,但确诊需进行心导管及心血管造影术。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "二维超声心动图" }, { "start_idx": 19, "end_idx": 28, "type": "pro", "entity": "心导管及心血管造影术" } ] }, { "text": "(二)冠状动脉主动脉起源异常左冠状动脉偶尔也可起源于右乏氏窦,或右冠状动脉起源于左乏氏窦。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "冠状动脉主动脉起源异常" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "左冠状动脉" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "右乏氏窦" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "右冠状动脉" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "左乏氏窦" } ] }, { "text": "这种起源异常的冠状动脉走行于主肺动脉间到达其供应区域。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "主肺动脉" } ] }, { "text": "患有本畸形的患儿易于发生胸痛、晕厥、甚至猝死。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "胸痛" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "晕厥" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "猝死" } ] }, { "text": "不幸的是有些患儿的首发表现即为猝死。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "猝死" } ] }, { "text": "本畸形临床难以诊断,确诊有赖于心导管及心血管造影术或CT。", "entities": [ { "start_idx": 15, "end_idx": 24, "type": "pro", "entity": "心导管及心血管造影术" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "CT" } ] }, { "text": "治疗方法为异常起源的冠状动脉重新植入到正确的乏氏窦。", "entities": [ { "start_idx": 5, "end_idx": 24, "type": "pro", "entity": "异常起源的冠状动脉重新植入到正确的乏氏窦" } ] }, { "text": "(三)冠状动静脉瘘本畸形冠状动脉的一支可直接与右心室(最常见位置)或右心房(直接或通过冠状窦)交通。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "冠状动静脉瘘" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "右心室" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "右心房" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "冠状窦" } ] }, { "text": "与左心室、左心房或肺动脉的交通比较少见。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "左心室" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "左心房" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肺动脉" } ] }, { "text": "本病最显著的临床特征为在胸骨左下缘可听到连续性浅表杂音。", "entities": [ { "start_idx": 12, "end_idx": 26, "type": "sym", "entity": "胸骨左下缘可听到连续性浅表杂音" } ] }, { "text": "分流量一般不大,否则可出现心搏增强及舒张中期隆隆样杂音。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "分流量一般不大" }, { "start_idx": 13, "end_idx": 26, "type": "sym", "entity": "心搏增强及舒张中期隆隆样杂音" } ] }, { "text": "可扪及连续性震颤。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "连续性震颤" } ] }, { "text": "二维多普勒超声及造影超声可作出诊断,但特异性诊断还需借助心导管及心血管造影术。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "二维多普勒超声" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "造影超声" }, { "start_idx": 28, "end_idx": 37, "type": "pro", "entity": "心导管及心血管造影术" } ] }, { "text": "治疗方法有结扎瘘口、修补瘘口(当与右心室相交通时)或经导管封堵。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "结扎瘘口" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "修补瘘口" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "右心室" } ] }, { "text": "(四)其他的冠状动脉异常单支冠状动脉:除了如发生冠状动脉粥样化出现相应症状外,这些患儿偶可猝死,这常发生在单支血管走行于主肺动脉间。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "冠状动脉异常" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "单支冠状动脉" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "冠状动脉粥样化" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "猝死" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "单支血管" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "主肺动脉" } ] }, { "text": "本病的临床表现与诊断与冠状动脉异常起源于主动脉相同。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "主动脉" } ] }, { "text": "偶尔整个冠状动脉系统可发育不良。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "可发育不良" } ] }, { "text": "有些患儿冠状动脉与主动脉的连接正常,但开口呈裂缝样,其起始部分斜形而不是通常的垂直自主动脉发出。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "主动脉" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "主动脉" } ] }, { "text": "这些患儿也可发生猝死。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "猝死" } ] }, { "text": "四、其他药物参见本篇第十一章支气管哮喘。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "支气管哮喘" } ] }, { "text": "第三章贫血第一节概况贫血(anemia)是指外周血液中单位容积内红细胞计数、血红蛋白含量及血细胞比容低于正常低限。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "贫血" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "贫血" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "anemia" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "血液" }, { "start_idx": 32, "end_idx": 36, "type": "ite", "entity": "红细胞计数" }, { "start_idx": 38, "end_idx": 43, "type": "ite", "entity": "血红蛋白含量" }, { "start_idx": 45, "end_idx": 49, "type": "ite", "entity": "血细胞比容" } ] }, { "text": "按世界卫生组织(WHO)对海拔0米时小儿贫血血红蛋白的标准规定:6个月~6岁,<110g/L;6~12岁,<120g/L时称为贫血。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "小儿贫血" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "贫血" } ] }, { "text": "海拔每升高1000米,血红蛋白低限标准应相应提高4%。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "<6个月婴儿,因生理性贫血等因素,血红蛋白变化较大,目前尚无统一标准。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "生理性贫血" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "一、贫血的分级小儿贫血分级见表10-10。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "贫血" }, { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "小儿贫" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "小儿贫" } ] }, { "text": "表10-1小儿贫血的分级", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "小儿贫" }, { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "小儿贫" } ] }, { "text": "第十三章小儿呼吸机治疗和吸入一氧化氮疗法第一节常规机械通气一、呼吸机和通气模式常规机械通气(conventionalmechanicalventilation,CMV)治疗用呼吸机一般为成人/小儿兼用型,按对气流控制方式具备以下基本模式:容量控制(volumecontrol,VCV,也称定容通气);压力控制(pressurecontrol,PCV,也称定压通气)。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "机械通气" }, { "start_idx": 32, "end_idx": 34, "type": "equ", "entity": "呼吸机" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "机械通气" }, { "start_idx": 47, "end_idx": 79, "type": "pro", "entity": "conventionalmechanicalventilation" }, { "start_idx": 81, "end_idx": 83, "type": "pro", "entity": "CMV" }, { "start_idx": 88, "end_idx": 90, "type": "equ", "entity": "呼吸机" } ] }, { "text": "辅助通气指患儿主要通过自主呼吸以适应、或以自主呼吸带动呼吸机供气,从而减轻呼吸做功。", "entities": [ { "start_idx": 27, "end_idx": 29, "type": "equ", "entity": "呼吸机" } ] }, { "text": "在辅助通气时又分为持续气道正压通气(continuouspositiveairwaypressure,CPAP)和压力支持通气(pressuresupportventilation,PSV)两种基本功能,某些呼吸机也用患者触发的通气(patienttriggeredventilation,PTV)表示,新型呼吸机还有容量支持通气(volumesupportventilation,VSV)功能。", "entities": [ { "start_idx": 104, "end_idx": 106, "type": "equ", "entity": "呼吸机" }, { "start_idx": 152, "end_idx": 156, "type": "equ", "entity": "新型呼吸机" } ] }, { "text": "缺点为气道峰压不控制,随通气量、气道阻力和肺组织顺应性而变化。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺组织" } ] }, { "text": "适用于没有或较轻外周肺损伤、手术、中枢性病变、或神经肌肉病变时的通气。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "外周肺损伤" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "手术" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "中枢性病变" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "神经肌肉" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "神经肌肉病变" } ] }, { "text": "目前新型呼吸机均设有潮气量目标化的气道压力自动调节的定容通气模式,也可以安全地应用于新生儿及小儿急性外周性肺损伤和呼吸衰竭。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "equ", "entity": "新型呼吸机" }, { "start_idx": 46, "end_idx": 55, "type": "dis", "entity": "小儿急性外周性肺损伤" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "每次供气压力限定时,通气量则随供气流量、气道阻力和肺组织顺应性而变化。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肺组织" } ] }, { "text": "新型呼吸机均设置吸气上升时间控制,或平台压控制旋钮来控制气道压的形成状态。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "equ", "entity": "新型呼吸机" } ] }, { "text": "适用于外周肺有病变损伤时的通气。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "外周肺" }, { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "外周肺有病变损伤" } ] }, { "text": "新型呼吸机具备供气和呼出气潮气量的精确监测,可以提高通气控制的稳定性。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "equ", "entity": "新型呼吸机" } ] }, { "text": "(四)同步间歇指令通气和间歇指令通气SIMV按照患儿自主呼吸的要求,提供预设的通气,避免与患儿自己的呼吸相冲突、对抗。", "entities": [ { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "通气" } ] }, { "text": "作用原理:在一次呼吸周期的呼气相后期(或呼气相全程),一旦患儿出现自主呼吸,气道压力和气流产生变化,呼吸机在极短时间内感知并发出指令,呼吸机立即提供通气气流,完成一次通气。", "entities": [ { "start_idx": 50, "end_idx": 52, "type": "equ", "entity": "呼吸机" }, { "start_idx": 67, "end_idx": 69, "type": "equ", "entity": "呼吸机" } ] }, { "text": "IMV为非同步间歇指令通气,其主要缺点为机械通气时出现患者与呼吸机对抗,因而鼓励使用SIMV和A/C通气模式的同步触发通气功能。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "机械通气" }, { "start_idx": 30, "end_idx": 32, "type": "equ", "entity": "呼吸机" } ] }, { "text": "在选择了SIMV(或A/C)模式时,应将触发控制功能打开,通过调节触发灵敏度来控制通气频率,以此获得目标分钟通气量和理想通气效果,SIMV应该与PSV联合应用(见后,呼吸机调节部分)。", "entities": [ { "start_idx": 83, "end_idx": 85, "type": "equ", "entity": "呼吸机" } ] }, { "text": "(五)压力调节容量控制通气新型呼吸机(如瑞典西门子300型、德国德尔格Babylog8000型)设有微电脑控制的“压力调节容量控制”(pressureregulatedvolumecontrol,PRVC),或称为“容量保证”(volumeguaranty)功能,将定容和定压的优点结合在一起,避免了各自的缺点,成为目前儿科患儿机械通气治疗的主要模式之一。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "equ", "entity": "新型呼吸机" }, { "start_idx": 164, "end_idx": 169, "type": "pro", "entity": "机械通气治疗" } ] }, { "text": "(六)压力支持通气呼吸机在一次通气的呼气相设有一特殊供气方式。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "equ", "entity": "呼吸机" } ] }, { "text": "在患儿自主呼吸发动时,经机内设置的触发反应机制将阀门打开,气流立即经气道进入肺内,在压力达到预设水平,且气流量衰减到最大峰流量的一定水平(如下降到峰流量的5%~25%),阀门关闭,供气气流中断,压力迅速回复到基线压水平。", "entities": [ { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "气道" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "肺内" } ] }, { "text": "这种通气的优点在于呼吸机随患者的需要而供气,可以提高并保证自主呼吸时的通气潮气量和每分通气量,而患者的吸气做功可以大大降低。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "equ", "entity": "呼吸机" } ] }, { "text": "此外,在保持每分通气量相似的条件下,PSV时的平均气道压(MAP)可以较A/C或IMV时降低30%~50%,可以降低气压伤危险性。", "entities": [ { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "气压伤" } ] }, { "text": "(七)持续气道正压通气CPAP是在整个呼吸周期,经鼻塞、面罩或气道插管将连续气流加于小儿鼻腔或气道,并产生高于大气压的鼻腔/气道压力,使小儿在吸气相得到较高的供气气压和流量,降低吸气做功;同时在呼气相,得到高于外界大气压的压力,避免肺泡塌陷。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "equ", "entity": "鼻塞" }, { "start_idx": 28, "end_idx": 29, "type": "equ", "entity": "面罩" }, { "start_idx": 31, "end_idx": 34, "type": "equ", "entity": "气道插管" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "鼻腔" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "气道" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "鼻腔" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "气道" }, { "start_idx": 116, "end_idx": 117, "type": "bod", "entity": "肺泡" } ] }, { "text": "新型专用于婴儿的CPAP气流供气系统采用特制的CPAP压力发生装置,使通气时压力恒定,效率大大提高,并可将供氧浓度调节在0.21~0.40,降低了高氧导致新生儿肺损伤的危险。", "entities": [ { "start_idx": 77, "end_idx": 82, "type": "dis", "entity": "新生儿肺损伤" } ] }, { "text": "参考文献1.ZulianF,WooP,AthreyaBH,etal.ThePediatricRheumatologyEuropeanSociety/AmericanCollegeofRheumatology/EuropeanLeagueagainstRheumatismprovisionalclassificationcriteriaforjuvenilesystemicsclerosis[J].ArthritisRheum,2007,57(2):203-2032.OchsHD,SmithCIE,PuckJM.PrimaryImmunodeficiencyDiseases:AMolecularandApproach.OxfordUniversityPress.NewYork,Oxford,19993.韩玉昆等.儿科实用诊断治疗学.合肥:安徽科技出版社,19994.杨锡强等.儿童免疫学.北京:人民卫生出版社,20015.StiehmER.ImmunologicDisorderinInfantsandChildren.3rded.W.B.SaundersCo,1996", "entities": [ { "start_idx": 360, "end_idx": 361, "type": "dep", "entity": "儿科" } ] }, { "text": "第五节先天性卵巢发育不全综合征【概述】本病由Turner于1938年首先报道,故称为Turner综合征(TS)。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "先天性卵巢发育不全综合征" }, { "start_idx": 42, "end_idx": 50, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "TS" } ] }, { "text": "该病也是人类唯一能生存的单体综合征。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "单体综合征" } ] }, { "text": "TS的主要临床特征是身材明显矮小、青春期不发育、原发性闭经、颈蹼及肘外翻等,其性腺呈纤维条索状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "TS" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "身材明显矮小" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "青春期不发育" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "原发性闭经" }, { "start_idx": 30, "end_idx": 35, "type": "sym", "entity": "颈蹼及肘外翻" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "性腺呈纤维条索状" } ] }, { "text": "【病理生理和发病机制】Turner综合征是由于细胞内X染色体缺失或结构发生改变所致。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 23, "end_idx": 29, "type": "bod", "entity": "细胞内X染色体" }, { "start_idx": 23, "end_idx": 38, "type": "sym", "entity": "细胞内X染色体缺失或结构发生改变" } ] }, { "text": "有关X单体染色体的遗传基础还不清楚,目前已排除遗传印迹的可能性,认为可能的机制为:①亲代生殖细胞的减数分裂发生不分离;②合子卵裂中姐妹染色单体不分离;③在有丝分裂过程中X染色体的部分丢失。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "X单体染色体" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "亲代生殖细胞" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "合子" }, { "start_idx": 65, "end_idx": 70, "type": "bod", "entity": "姐妹染色单体" }, { "start_idx": 84, "end_idx": 87, "type": "bod", "entity": "X染色体" } ] }, { "text": "由于在胚胎发育早期,女性两条X染色体之一就已失活而不转录,因此仅有一条X染色体在人体的发育中起积极作用。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "X染色体" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "X染色体" } ] }, { "text": "由此看来,Turner综合征表型并非由X单体所致,可能涉及某些至今尚未明确的Turner综合征相关基因的缺陷。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "X单体" }, { "start_idx": 38, "end_idx": 46, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "基因" } ] }, { "text": "在胚胎发育早期及X染色体失活之前,涉及人体细胞发育的X连锁基因可能即已表达,并呈双倍剂量的基因表达。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "胚胎" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "X染色体" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "细胞" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "X连锁基因" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "基因" } ] }, { "text": "失活X染色体上的X连锁基因似乎并未完全失活,可能存在不完全失活区域,由于该区域的基因表达是造成Turner综合征临床表型的关键因素,因此提示Turner综合征相关基因很可能位于该区域内。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "失活X染色体" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "X连锁基因" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "基因" }, { "start_idx": 47, "end_idx": 55, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 70, "end_idx": 78, "type": "dis", "entity": "Turner综合征" } ] }, { "text": "有关Y染色体的研究结果表明,由于部分Turner综合征患者伴有Y染色体着丝粒衍生物,因此人们认为性别决定基因和Turner综合征相关基因在遗传学上是两个完全独立的基因控制位点。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "Y染色体" }, { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 31, "end_idx": 40, "type": "bod", "entity": "Y染色体着丝粒衍生物" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "基因" }, { "start_idx": 55, "end_idx": 63, "type": "dis", "entity": "Turner综合征" } ] }, { "text": "研究还发现,部分患者中存在涉及Turner综合征相关的Y染色体基因(RPS4Y)与X染色体基因(RPS4X)连锁,并能编码一种40S的蛋白异构体,提示该连锁基因可能是Turner综合征的候选基因。", "entities": [ { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "Y染色体基因" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "RPS4Y" }, { "start_idx": 41, "end_idx": 46, "type": "bod", "entity": "X染色体基因" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "RPS4X" }, { "start_idx": 63, "end_idx": 71, "type": "bod", "entity": "40S的蛋白异构体" }, { "start_idx": 83, "end_idx": 91, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 93, "end_idx": 96, "type": "bod", "entity": "候选基因" } ] }, { "text": "目前有关TS表型特征的相关分子遗传学研究显示,身材矮小相关基因(SHOX)和FOXC2基因分别与Turner骨骼异常及淋巴管膨胀及淋巴水肿有关。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "TS" }, { "start_idx": 38, "end_idx": 44, "type": "bod", "entity": "FOXC2基因" }, { "start_idx": 48, "end_idx": 57, "type": "dis", "entity": "Turner骨骼异常" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "淋巴管膨胀" }, { "start_idx": 65, "end_idx": 68, "type": "dis", "entity": "淋巴水肿" } ] }, { "text": "SHOX基因定位于Xp22的伪长染色体区1(pseudoautosomalregion1,PAR1),包含7个外显子,分别编码292和225个氨基酸残基组成的两种转录蛋白(SHOXa和SHOXb),目前推测SHOX基因缺陷所致相关蛋白单倍剂量表达不足是与TS患者矮身材及骨骼畸形有关,但对TS的其他表型无重要作用。", "entities": [ { "start_idx": 9, "end_idx": 20, "type": "bod", "entity": "Xp22的伪长染色体区1" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "PAR1" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "外显子" }, { "start_idx": 71, "end_idx": 75, "type": "dis", "entity": "氨基酸残基" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "SHOXa" }, { "start_idx": 92, "end_idx": 96, "type": "bod", "entity": "SHOXb" }, { "start_idx": 103, "end_idx": 110, "type": "dis", "entity": "SHOX基因缺陷" }, { "start_idx": 115, "end_idx": 116, "type": "bod", "entity": "蛋白" }, { "start_idx": 127, "end_idx": 128, "type": "dis", "entity": "TS" }, { "start_idx": 131, "end_idx": 133, "type": "sym", "entity": "矮身材" }, { "start_idx": 135, "end_idx": 138, "type": "sym", "entity": "骨骼畸形" }, { "start_idx": 144, "end_idx": 145, "type": "dis", "entity": "TS" } ] }, { "text": "FOXC2基因定位于16号染色体长臂(16q),仅1个外显子,mRNA1.5kb,相关转录蛋白涉足胎儿相关发育旁路途径。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "FOXC2基因" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "16号染色体长臂" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "16q" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "外显子" }, { "start_idx": 31, "end_idx": 39, "type": "bod", "entity": "mRNA1.5kb" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "转录蛋白" } ] }, { "text": "该基因缺陷可致患者淋巴管发育不全、淋巴阻塞和继发性淋巴水肿。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "淋巴管发育不全" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "淋巴阻塞" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "继发性淋巴水肿" } ] }, { "text": "总之,目前Turner综合征致病机制正在向分子遗传学方向深入。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "Turner综合征" } ] }, { "text": "【临床表现】典型的Turner综合征患者在出生时就有身高和体重发育落后,在新生儿时期可见颈后皮肤过度折叠以及手、足背发生水肿等特殊症状。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "身高和体重发育落后" }, { "start_idx": 44, "end_idx": 51, "type": "sym", "entity": "颈后皮肤过度折叠" }, { "start_idx": 54, "end_idx": 66, "type": "sym", "entity": "手、足背发生水肿等特殊症状" } ] }, { "text": "除生殖器、乳腺不发育、原发性闭经及缺乏第二性征之外,尚有呆板面容、智力正常或稍低,约有18%的患者有智能落后。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "sym", "entity": "生殖器" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "乳腺不发育" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "呆板面容" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "智力正常或稍低" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "智能落后" } ] }, { "text": "最近有报道,用超声心动图检查Turner综合征患者,查出有34%病例并发主动脉瓣二叶型,但无狭窄。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "超声心动图检查" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "主动脉瓣二叶型" }, { "start_idx": 45, "end_idx": 47, "type": "sym", "entity": "无狭窄" } ] }, { "text": "患者还可并发肾脏畸形。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "肾脏畸形" } ] }, { "text": "【实验室检查】1.染色体核型分析临床确诊TS的关键检测手段,用外周血淋巴细胞培养技术进行核型分析,先天性卵巢发育不全综合征的异常核型有以下类型:(1)单体型,45,X是最多见的一种,约占60%。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "染色体核型分析" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "TS" }, { "start_idx": 31, "end_idx": 41, "type": "pro", "entity": "外周血淋巴细胞培养技术" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "核型分析" }, { "start_idx": 49, "end_idx": 60, "type": "dis", "entity": "先天性卵巢发育不全综合征" }, { "start_idx": 75, "end_idx": 77, "type": "dis", "entity": "单体型" }, { "start_idx": 82, "end_idx": 82, "type": "bod", "entity": "X" } ] }, { "text": "(2)嵌合型,核型为45,X/46,XX,约占该病的25%。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "嵌合型" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "X" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "XX" } ] }, { "text": "细胞类型以46,XX为主的个体临床症状较轻,约20%的患者可有月经来潮,部分有生育能力。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "XX" } ] }, { "text": "若患者以45,X细胞为主,其表型与单体型相似。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "X细胞" } ] }, { "text": "(3)X染色体结构异常,46,Xdel(Xq)或者46,Xdel(Xp),即1条X染色体长臂或短臂缺失,同时伴有X染色体易位;46,Xi(Xq),即一条X染色体的短臂缺失而形成了等长臂X染色体。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "X染色体结构异常" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "X染色体" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "X染色体" }, { "start_idx": 76, "end_idx": 79, "type": "bod", "entity": "X染色体" }, { "start_idx": 89, "end_idx": 95, "type": "bod", "entity": "等长臂X染色体" }, { "start_idx": 74, "end_idx": 95, "type": "sym", "entity": "一条X染色体的短臂缺失而形成了等长臂X染色体" } ] }, { "text": "2.内分泌激素检查垂体促性腺激素黄体生成激素(LH)及促卵泡生长激素(FSH)明显升高,E2降低,提示卵巢功能衰竭。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "内分泌激素检查" }, { "start_idx": 9, "end_idx": 21, "type": "ite", "entity": "垂体促性腺激素黄体生成激素" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "LH" }, { "start_idx": 27, "end_idx": 33, "type": "ite", "entity": "促卵泡生长激素" }, { "start_idx": 35, "end_idx": 37, "type": "ite", "entity": "FSH" }, { "start_idx": 9, "end_idx": 42, "type": "sym", "entity": "垂体促性腺激素黄体生成激素(LH)及促卵泡生长激素(FSH)明显升高" }, { "start_idx": 44, "end_idx": 51, "type": "ite", "entity": "E2" }, { "start_idx": 44, "end_idx": 53, "type": "sym", "entity": "E2降低" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "卵巢" }, { "start_idx": 57, "end_idx": 62, "type": "sym", "entity": "卵巢功能衰竭" } ] }, { "text": "部分患者血清生长激素(GH)激发峰值常可小于10ng/ml,血清胰岛素样生长因子1(IGF-1)分泌低下。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "ite", "entity": "血清生长激素" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "GH" }, { "start_idx": 4, "end_idx": 28, "type": "sym", "entity": "血清生长激素(GH)激发峰值常可小于10ng/ml" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "血清胰岛素" }, { "start_idx": 30, "end_idx": 51, "type": "sym", "entity": "血清胰岛素样生长因子1(IGF-1)分泌低下" } ] }, { "text": "3.B超检查显示子宫及卵巢发育不良,严重者呈纤维条索状。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "B超检查" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "子宫" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "卵巢" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "子宫及卵巢发育不良" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "严重者呈纤维条索状" } ] }, { "text": "【诊断及鉴别诊断】如发现女孩身材矮小,肘外翻,颈蹼,青春期无第二性征,又伴有某些先天畸形时应怀疑此病。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "身材矮小" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "肘外翻" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "颈蹼" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "青春期无第二性征" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "先天畸形" } ] }, { "text": "确诊需女孩身材矮小学检查。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "女孩身材矮小" } ] }, { "text": "在鉴别诊断时应考虑下列疾病:1.先天畸形症患者多无畸形,其生长激素分泌不足,部分病人还出现促甲状腺激素及促肾上腺皮质激素不足。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "先天畸形" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "患者多无畸形" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "生长激素" }, { "start_idx": 28, "end_idx": 36, "type": "sym", "entity": "其生长激素分泌不足" }, { "start_idx": 45, "end_idx": 50, "type": "bod", "entity": "促甲状腺激素" }, { "start_idx": 52, "end_idx": 59, "type": "bod", "entity": "促肾上腺皮质激素" }, { "start_idx": 43, "end_idx": 61, "type": "sym", "entity": "出现促甲状腺激素及促肾上腺皮质激素不足" } ] }, { "text": "2.青春期发育延迟虽青春期较正常儿延迟数年出现促甲状腺激素到发育正常水平,其内分泌功能亦正常,无血TSH及LH升高。", "entities": [ { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "出现促甲状腺激素" }, { "start_idx": 48, "end_idx": 54, "type": "ite", "entity": "血TSH及LH" } ] }, { "text": "3.出生低体重儿以足月出生,但体重明显低于正常儿以及体格发育始终无血TSH及LH升高为其特征。", "entities": [ { "start_idx": 15, "end_idx": 29, "type": "sym", "entity": "体重明显低于正常儿以及体格发育" }, { "start_idx": 32, "end_idx": 41, "type": "sym", "entity": "无血TSH及LH升高" } ] }, { "text": "其生长激素及骨龄接近正常。", "entities": [ { "start_idx": 1, "end_idx": 11, "type": "sym", "entity": "生长激素及骨龄接近正常" } ] }, { "text": "4.Noonan综合征临床表现与Turner综合征相似,智能发育迟缓者较多,部分病人合并心血管畸形,其中以肺动脉狭窄及房间隔缺损最常见,其核型为正常男性或者女性。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "Noonan综合征" }, { "start_idx": 16, "end_idx": 24, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "智能发育迟缓者" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "合并心血管畸形" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "肺动脉" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "房间隔" }, { "start_idx": 53, "end_idx": 63, "type": "sym", "entity": "肺动脉狭窄及房间隔缺损" } ] }, { "text": "【治疗】本病的治疗以改善其肺动脉狭窄身高、促进性征发育、辅助生殖技术、社会心理治疗及相关疾病防治为目标。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "肺动脉狭窄" } ] }, { "text": "重组人生长激素对TS患儿身高改善有一定作用,明确诊断后每晚临睡前皮下注射0.15U/kg。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "TS" } ] }, { "text": "2.雌性激素替代治疗在青春期可用雌激素进行替代疗法,一般从12~14岁开始,先用小剂量结合雌性激素疗6~12个月,逐步增加到成年人替代治疗剂量,以促使乳房及外阴发育。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "雌性激素替代治疗" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "雌激素" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "雌性激素" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "乳房" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "外阴" } ] }, { "text": "2年后可进行周期性的雌激素-孕激素疗法(人工周期治疗),有助于患者的第二性征发育及提高生活质量。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "pro", "entity": "雌激素-孕激素疗法" } ] }, { "text": "由于性激素具有促进骨骺愈合,限制骨骼生长的作用,故在青春期前忌用,12岁后方可考虑使用。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "性激素" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "骨骺" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "骨骼" } ] }, { "text": "诱导性激素性发育须遵循个体化原则。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "性激素" } ] }, { "text": "极少数嵌合型患者可能有生育能力,但其流产或者死胎率极高,30%后代患有染色体畸变。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "流产" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "死胎率" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "染色体畸变" } ] }, { "text": "二、呼吸机参数设定及其呼吸生理基础(一)肺通气量有关通气量、压力和流量示意(图8-19)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "呼吸机" }, { "start_idx": 20, "end_idx": 23, "type": "ite", "entity": "肺通气量" } ] }, { "text": "图8-19气道压力、肺容量、气流流量变化示意图1.潮气(tidalvolume,VT)每一次自主呼吸或机械通气时,进入或排出肺的气体量。", "entities": [ { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "机械通气" }, { "start_idx": 62, "end_idx": 62, "type": "bod", "entity": "肺" } ] }, { "text": "机械通气下,儿童与成人为5~7ml/kg体重,足月新生儿为6~8ml/kg,早产儿为8~10ml/kg。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "机械通气" } ] }, { "text": "2.通气频率(frequency)和吸呼比值(inspiration:expirationratio,I∶E)通气频率指每分钟机械通气的次数。", "entities": [ { "start_idx": 63, "end_idx": 66, "type": "pro", "entity": "机械通气" } ] }, { "text": "完成一次机械通气所需时间为通气周期,为频率的倒数×60。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "机械通气" } ] }, { "text": "吸呼比值则指一次机械通气时,供气与排气的时间比例,呼吸机一般只调节供气时间的长短以获得所需的吸呼比值,但有的呼吸机如美国Sechrist型、德国德尔格Babylog8000型,则可以分别改变供气和排气时间来改变吸呼比,且会影响到通气频率。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "机械通气" }, { "start_idx": 25, "end_idx": 27, "type": "equ", "entity": "呼吸机" }, { "start_idx": 54, "end_idx": 56, "type": "equ", "entity": "呼吸机" }, { "start_idx": 58, "end_idx": 68, "type": "equ", "entity": "美国Sechrist型" }, { "start_idx": 70, "end_idx": 86, "type": "equ", "entity": "德国德尔格Babylog8000型" } ] }, { "text": "3.每分通气量(minutevolume,MV)为潮气量和呼吸频率的乘积,指每分钟进出肺部的气量,用于判断通气量的大小,较单用潮气量要全面。", "entities": [ { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "肺部" } ] }, { "text": "机械通气下,一个3~4kg小儿一般要维持在0.8~1.2L/min。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "机械通气" } ] }, { "text": "当血气值测定后要求对呼吸机参数调整时,也应设定新的每分通气量值。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "ite", "entity": "血气值测定" }, { "start_idx": 10, "end_idx": 12, "type": "equ", "entity": "呼吸机" } ] }, { "text": "一般呼吸机均有每分通气量上下限报警。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "呼吸机" } ] }, { "text": "如果上限报警,可能因通气频率加快(触发增加)或潮气量过大(定压模式);如果下限报警,可能为供气量不足,供气回路管道或接口漏气,潮气量过低(定压模式),或呼吸机主供气气流不稳定(须检查压缩空气和氧气气源压力)。", "entities": [ { "start_idx": 76, "end_idx": 78, "type": "equ", "entity": "呼吸机" } ] }, { "text": "但在小婴儿,由于气道插管在声门处的漏气,或呼吸机监测装置装置的偏差,可以出现不一致。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "声门" }, { "start_idx": 21, "end_idx": 27, "type": "equ", "entity": "呼吸机监测装置" } ] }, { "text": "(二)通气压力1.气道峰压(peakinspiratorypressure,PIP)PIP是指在一次机械通气周期内,气道内压力达到的最大值,其内涵包括:①供气压力高于大气压(基线压)的最大变化水平;②达到最大峰压值所需时间;③达到最大值后的维持时间(图8-8)。", "entities": [ { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "气道" } ] }, { "text": "机械通气或自主呼吸的吸气末和呼气末的气流为零。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "机械通气" } ] }, { "text": "PIP设置的高低在于使肺泡张开并维持其张开达到适当时间。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肺泡" } ] }, { "text": "不同的压力波型可以对肺泡张开和肺泡内压水平,产生不同效果,并可应用于不同的肺内病变。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肺泡" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺泡" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "肺内病变" } ] }, { "text": "通过呼吸机设置吸气气流上升时间改变压力波形可以得到较安全有效的通气。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "呼吸机" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "通气" } ] }, { "text": "有的呼吸机采用设置PIP水平高于PEEP水平,因此实际的PIP水平应该为其设置值加上设置的PEEP值。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "呼吸机" } ] }, { "text": "在弥漫性肺损伤和肺泡萎陷时,要获得比较好的氧合,需要在机械通气时保持压力的平台,以使肺泡持续扩张,气体在不同时间常数的肺泡单位内移动,达到压力平台,使得到较好的肺泡通气/灌流,减少肺内分流。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "弥漫性肺损伤" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "肺泡萎陷" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "机械通气" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "肺泡" } ] }, { "text": "图8-20机械通气气道峰压示意图压力上升缓慢,没有气道峰压平台;压力上升较快,出现气道峰压平台;压力上升迅速,气道峰压平台时间较长,趋于平台4.呼气末正压(PEEP)呼吸机在排气管道设置的阻力阀门,使呼气相管道阻力增加,管道压力高于大气压。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "equ", "entity": "机械通气气道" }, { "start_idx": 83, "end_idx": 85, "type": "equ", "entity": "呼吸机" } ] }, { "text": "其效果主要为保持在呼气时肺泡不至于立即关闭,提高功能余气量,有利于气血交换,并减少呼吸做功。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肺泡" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "气血" } ] }, { "text": "一般用ml/min或L/min表示,在肺功能监护仪上显示一个呼吸周期的通气流量随时间或潮气有大小、位相的变化。", "entities": [ { "start_idx": 19, "end_idx": 24, "type": "equ", "entity": "肺功能监护仪" } ] }, { "text": "当自主呼吸增强或患儿触发的机械通气次数增加,实测每分流量可以高于设定每分流量。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "机械通气" } ] }, { "text": "3.峰流量在一次通气开始时,供气阀门打开,气流在瞬间达到最大值,以后随气道阻力和肺顺应性的反应,供气气流下降,为减速气流,为目前呼吸机常设。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "通气" }, { "start_idx": 64, "end_idx": 66, "type": "equ", "entity": "呼吸机" } ] }, { "text": "如果在气流达到最大后,尽管有气道阻力的增加,供气气流仍然维持在恒定水平,此为持续恒定气流,为一部分儿童型呼吸机供气的主要方式。", "entities": [ { "start_idx": 49, "end_idx": 54, "type": "equ", "entity": "儿童型呼吸机" } ] }, { "text": "在新型呼吸机上,小流量的偏流气流使流量触发功能得以发挥。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "equ", "entity": "新型呼吸机" } ] }, { "text": "第十三章硬皮病【概述】硬皮病(scleroderma)是儿童时期少见的慢性结缔组织病。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "硬皮病" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "硬皮病" }, { "start_idx": 15, "end_idx": 25, "type": "dis", "entity": "scleroderma" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "慢性结缔组织病" } ] }, { "text": "它可分为局限性硬皮病(localizedscleroderma)和系统性硬化症(systemicsclerosis)两种类型。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "局限性硬皮病" }, { "start_idx": 11, "end_idx": 30, "type": "dis", "entity": "localizedscleroderma" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "系统性硬化症" }, { "start_idx": 40, "end_idx": 56, "type": "dis", "entity": "systemicsclerosis" } ] }, { "text": "前者以局限性皮肤增厚和纤维化为主,后者除皮肤弥漫性增厚和纤维化,内脏器官如心、肺、肾也可受侵犯。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "局限性皮肤增厚" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "纤维化" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "皮肤" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "皮肤弥漫性增厚" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "纤维化" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "心" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "肺" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "肾" }, { "start_idx": 32, "end_idx": 46, "type": "sym", "entity": "内脏器官如心、肺、肾也可受侵犯" } ] }, { "text": "个别局限性硬皮病可转变为系统性硬化症而累及内脏,故认为两者是同一病理过程的不同类型。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "局限性硬皮病" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "系统性硬化症" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "内脏" } ] }, { "text": "【病因和发病机制】病因未明,很多报道提示是在遗传因素(如HLADR1和DR3频率较高)的参与下,由于某种因子刺激所导致的自身免疫性疾病。", "entities": [ { "start_idx": 72, "end_idx": 78, "type": "dis", "entity": "自身免疫性疾病" } ] }, { "text": "并认为本病的纤维化病变与5-羟色胺代谢异常有关。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "纤维化病变" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "5-羟色胺" } ] }, { "text": "内脏肌肉纤维变性、萎缩以及肌间纤维组织增生而造成脏器硬化。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "脏器" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "脏器硬化" } ] }, { "text": "【临床表现】(一)起病常隐袭。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "起病常隐袭" } ] }, { "text": "(二)皮肤、黏膜开始皮肤病变见于双侧手指及面部,后向躯干蔓延。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "皮肤" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "黏膜" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "皮肤" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "手指" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "面部" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "躯干" }, { "start_idx": 3, "end_idx": 29, "type": "sym", "entity": "皮肤、黏膜开始皮肤病变见于双侧手指及面部,后向躯干蔓延" } ] }, { "text": "经历水肿期(皮肤变厚、紧张、苍白和皮温降低)、硬化期(皮肤增厚、变硬如皮革,呈蜡样光泽、面部呈假面具状、皱纹消失和张口困难)、最后萎缩期(皮肤光滑而细薄如羊皮纸紧贴于皮下骨面)。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "皮肤" }, { "start_idx": 6, "end_idx": 20, "type": "sym", "entity": "皮肤变厚、紧张、苍白和皮温降低" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "皮肤" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "面部" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "口" }, { "start_idx": 27, "end_idx": 60, "type": "sym", "entity": "皮肤增厚、变硬如皮革,呈蜡样光泽、面部呈假面具状、皱纹消失和张口困难" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "皮肤" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "皮下骨面" }, { "start_idx": 69, "end_idx": 86, "type": "sym", "entity": "皮肤光滑而细薄如羊皮纸紧贴于皮下骨面" } ] }, { "text": "黏膜(如口腔及阴道黏膜)可硬化、萎缩。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "黏膜" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "口腔" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "阴道黏膜" }, { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "黏膜(如口腔及阴道黏膜)可硬化、萎缩" } ] }, { "text": "(三)雷诺现象约为70%患者的首发症状,有时为硬皮病早期唯一表现,是该病的典型症状之一。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "雷诺现象" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "硬皮病" } ] }, { "text": "(四)关节和肌肉关节炎或关节痛,以手指关节常见,指端可因缺血而造成指垫丧失,指骨溶解、吸收而缩短。肌肉无力和萎缩。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "关节" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "肌肉关节炎" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "关节" }, { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "关节和肌肉关节炎或关节痛" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "手指关节" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "指端" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "缺血" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "指垫" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "指垫丧失" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "指骨" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "肌肉" }, { "start_idx": 38, "end_idx": 55, "type": "sym", "entity": "指骨溶解、吸收而缩短。肌肉无力和萎缩" } ] }, { "text": "(五)消化系统食管受累而引起吞咽困难,反流性食道炎,吸收不良综合征等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "消化系统食管" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "吞咽困难" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "反流性食道炎" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "吸收不良综合征" } ] }, { "text": "(六)肺脏间质性肺炎、纤维化,通气及换气功能受损。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肺脏" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "间质性肺炎" }, { "start_idx": 3, "end_idx": 23, "type": "sym", "entity": "肺脏间质性肺炎、纤维化,通气及换气功能受损" } ] }, { "text": "(七)心脏心脏增大、心力衰竭、心包炎、心律失常和肺动脉高压等是死亡重要原因之一。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心脏" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "心脏增大" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "心包炎" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "心律失常" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "肺动脉高压" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "死亡" } ] }, { "text": "(八)肾脏约17%受累,蛋白尿、血尿,有时出现硬皮病危象(急进性高血压及进行性肾衰竭),是重要死因之一。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "血尿" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "硬皮病" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "急进性高血压" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "进行性肾衰竭" } ] }, { "text": "(九)其他发热,多发性神经炎等。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "发热" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "多发性神经炎" } ] }, { "text": "(十)分型1.局限型病变限于皮肤,预后较好。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "局限型" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "皮肤" } ] }, { "text": "此型有一组特殊临床表现,称为CREST综合征(皮下钙化、雷诺征、食管运动功能障碍、硬指和毛细血管扩张)。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "CREST综合征" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "皮下" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "食管" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "硬指" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "毛细血管" }, { "start_idx": 23, "end_idx": 49, "type": "sym", "entity": "皮下钙化、雷诺征、食管运动功能障碍、硬指和毛细血管扩张" } ] }, { "text": "2.弥漫型皮损累及全身,进展快,内脏器官受累。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "弥漫型皮损" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "内脏器官" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "内脏器官受累" } ] }, { "text": "3.重叠型局限或弥漫型伴有另一种结缔组织病。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "重叠型" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "弥漫型" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "结缔组织病" } ] }, { "text": "【实验室检查】(一)血沉加快。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "血沉" } ] }, { "text": "(二)自身抗体1.抗核抗体阳性,斑点型及核仁型为主。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "抗体" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "抗核抗体阳性" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "斑点型" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "核仁型" } ] }, { "text": "2.抗Scl-70抗体阳性,是弥漫型SSc标记抗体。", "entities": [ { "start_idx": 15, "end_idx": 24, "type": "bod", "entity": "弥漫型SSc标记抗体" } ] }, { "text": "3.抗着丝点抗体是局限型SSc标记抗体,特别是CREST综合征时阳性。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "抗着丝点抗体" }, { "start_idx": 9, "end_idx": 18, "type": "bod", "entity": "局限型SSc标记抗体" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "CREST综合征" } ] }, { "text": "4.抗核仁抗体阳性(三)皮肤活体组织检查胶原纤维增生、硬化和萎缩,结缔组织细胞浸润,小血管壁增厚,管腔变小和闭塞。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "抗核仁抗体" }, { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "抗核仁抗体阳性" }, { "start_idx": 12, "end_idx": 19, "type": "pro", "entity": "皮肤活体组织检查" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "胶原纤维" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "胶原纤维增生" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "硬化" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "萎缩" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "结缔组织细胞" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "结缔组织细胞浸润" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "小血管壁" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "小血管壁增厚" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "管腔" }, { "start_idx": 49, "end_idx": 55, "type": "sym", "entity": "管腔变小和闭塞" } ] }, { "text": "(四)X线检查食道蠕动减弱、管壁僵硬。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "X线检查" }, { "start_idx": 7, "end_idx": 17, "type": "sym", "entity": "食道蠕动减弱、管壁僵硬" } ] }, { "text": "间质性肺炎及肺纤维化等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "间质性肺炎" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "肺纤维化" } ] }, { "text": "(五)肺功能测定肺容量及弥散功能减低。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" } ] }, { "text": "【诊断与鉴别诊断】(一)诊断标准2007年ACR与欧洲风湿病联盟共同修订的SSc分类标准:1.主要指标近端硬皮:对称性手指及掌指或跖趾近端皮肤增厚、紧硬,类似病变亦见于整个四肢、面颈及躯干(胸腹)。", "entities": [ { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "风湿病" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "手指" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "掌指" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "跖趾" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "四肢" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "面颈" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "躯干" }, { "start_idx": 95, "end_idx": 96, "type": "bod", "entity": "胸腹" } ] }, { "text": "2.次要指标(1)硬指:上述皮肤改变仅限于手指。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "皮肤" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "手指" } ] }, { "text": "(2)外周血管:雷诺现象或指端可凹性瘢痕或指垫变薄、丧失。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "外周血管" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "雷诺现象" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "指端" }, { "start_idx": 13, "end_idx": 27, "type": "sym", "entity": "指端可凹性瘢痕或指垫变薄、丧失" } ] }, { "text": "(3)胃肠道:胃十二指肠反流及吞咽困难。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "胃肠道" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "胃十二指肠" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "吞咽困难" } ] }, { "text": "(4)呼吸系统:肺底部纤维化及肺动脉高压。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "肺底部纤维化" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "肺动脉高压" } ] }, { "text": "(5)心脏:心律失常及心功能衰竭。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "心律" }, { "start_idx": 6, "end_idx": 15, "type": "sym", "entity": "心律失常及心功能衰竭" } ] }, { "text": "(6)肾脏:肾危象及肾血管性高血压。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 6, "end_idx": 16, "type": "sym", "entity": "肾危象及肾血管性高血压" } ] }, { "text": "(7)神经系统:出现神经系统病变骨骼肌肉(8)骨骼肌肉:肌腱摩擦音、关节炎和肌炎。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经系统" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "骨骼肌肉" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "骨骼肌肉" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肌腱" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "关节炎" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "肌炎" }, { "start_idx": 28, "end_idx": 39, "type": "sym", "entity": "肌腱摩擦音、关节炎和肌炎" } ] }, { "text": "(9)血清学:ANA阳性或特异性抗体(如Scl70)阳性。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "特异性抗体" }, { "start_idx": 7, "end_idx": 27, "type": "sym", "entity": "ANA阳性或特异性抗体(如Scl70)阳性" } ] }, { "text": "具备1项主要指标和2项次要指标者可诊断为SSc。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "SSc" } ] }, { "text": "(二)鉴别诊断1.局部性硬皮病局部皮肤变硬呈线状或斑点状,界限清楚,无血清学及内脏病变。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "局部性硬皮病" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "皮肤" }, { "start_idx": 15, "end_idx": 32, "type": "sym", "entity": "局部皮肤变硬呈线状或斑点状,界限清楚" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "内脏" } ] }, { "text": "2.混合性结缔组织病该病有手指肿胀及雷诺现象,易与SSc混淆,但它兼有狼疮及肌炎表现,如蛋白尿、肌无力及肌酶增高,高滴度抗RNP抗体可鉴别。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "混合性结缔组织病" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "手指" }, { "start_idx": 13, "end_idx": 21, "type": "sym", "entity": "手指肿胀及雷诺现象" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "SSc" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "狼疮" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "肌炎" }, { "start_idx": 44, "end_idx": 55, "type": "sym", "entity": "蛋白尿、肌无力及肌酶增高" } ] }, { "text": "3.嗜酸性筋膜炎肢体局部压痛、肿胀和硬结,但一般不影响手、足和面部,嗜酸粒细胞增多,无雷诺现象及内脏损害,自身抗体阴性,活体组织检查可见深筋膜、皮下组织广泛炎症和硬化。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "嗜酸性筋膜炎" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肢体" }, { "start_idx": 8, "end_idx": 19, "type": "sym", "entity": "肢体局部压痛、肿胀和硬结" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "手" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "足" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "面部" }, { "start_idx": 60, "end_idx": 65, "type": "pro", "entity": "活体组织检查" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "皮下组织" }, { "start_idx": 68, "end_idx": 82, "type": "sym", "entity": "深筋膜、皮下组织广泛炎症和硬化" } ] }, { "text": "4.自限性硬肿病皮肤发硬,但①病损发展快,短期内可累及全身皮肤,手、足常不受累;②无雷诺现象;③抗Scl-70抗体等常阴性;④病程常自限性;⑤发病前常有感染史,如流感、咽炎及扁桃体炎等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "自限性硬肿病" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "皮肤" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "皮肤发硬" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "皮肤" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "手" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "足" }, { "start_idx": 81, "end_idx": 82, "type": "dis", "entity": "流感" }, { "start_idx": 84, "end_idx": 85, "type": "dis", "entity": "咽炎" }, { "start_idx": 87, "end_idx": 90, "type": "dis", "entity": "扁桃体炎" } ] }, { "text": "(一)一般治疗保暖,营养,避免劳累及精神紧张,清除感染。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "保暖" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "营养" }, { "start_idx": 13, "end_idx": 21, "type": "pro", "entity": "避免劳累及精神紧张" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "清除感染" } ] }, { "text": "(二)糖皮质激素用于系统性硬化症,缓解炎性肌病及肺纤维化,但不能阻止本病的进展。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "系统性硬化症" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "炎性肌病" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "肺纤维化" } ] }, { "text": "泼尼松1~2mg/(kg•d)分次服用,症状缓解后逐渐减量。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "泼尼松" } ] }, { "text": "(三)免疫抑制剂氨甲蝶呤0.25~0.5mg/kg,每周一次口服。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "氨甲蝶呤" } ] }, { "text": "(四)青霉胺适用于严重病例,3mg/kg,2个月后每月增加2~3mg/kg,最后达每日10~15mg/kg,一般剂量250~500mg/d。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "青霉胺" } ] }, { "text": "(五)扩张血管药硝苯地平0.5~1.0mg/(kg•d),分次服用。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "扩张血管药" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "硝苯地平" } ] }, { "text": "(六)中药可选用复方参片口服或用复方丹参注射液静滴。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "中药" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "复方参片" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "复方丹参" }, { "start_idx": 20, "end_idx": 24, "type": "pro", "entity": "注射液静滴" } ] }, { "text": "(七)血浆置换用于重症及药物疗效不佳患者。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "血浆置换" } ] }, { "text": "【预后】局限性硬皮病一般无生命危险,皮肤损害可持续进展多年,个别损害可逐渐变软,但很少完全恢复正常。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "局限性硬皮病" } ] }, { "text": "肺和心脏受累是本病的严重表现。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心脏" } ] }, { "text": "肺纤维化和心功能不全是主要致死的原因。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "肺纤维化" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "心功能不全" } ] }, { "text": "第四节维生素K缺乏症维生素K分为两大类:一类是脂溶性维生素K1(从植物中提取)和K2(从微生物中提取,也可由肠内细菌制造),另一类是水溶性维生素K3和K4(由人工合成),其中以K1和K2最为重要。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "维生素K缺乏症" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "维生素K" }, { "start_idx": 23, "end_idx": 30, "type": "bod", "entity": "脂溶性维生素K1" }, { "start_idx": 66, "end_idx": 69, "type": "mic", "entity": "肠内细菌" }, { "start_idx": 78, "end_idx": 85, "type": "bod", "entity": "水溶性维生素K3" } ] }, { "text": "维生素K是促进血液凝固的化学物质之一,是四种凝血蛋白(凝血酶原、转变加速因子、抗血友病因子和司徒因子)在肝内合成必不可少的物质。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "维生素K" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血液" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "凝血蛋白" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "转变加速因子" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "抗血友病因子" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "司徒因子" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "肝" } ] }, { "text": "维生素K的缺乏将导致凝血功能失常而出现出血。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "维生素K" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "凝血功能失常" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "出血" } ] }, { "text": "【流行病学】维生素K缺乏是婴儿和新生儿出血性疾病的主要原因,其发病急,病死率高,严重危害婴儿健康,1991年城市颅内出血死亡率71.5/105,1993年为106.6/105。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "维生素K缺乏" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "出血性疾病" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "颅内出血" } ] }, { "text": "本病发病高峰年龄为4~8周,发病的男女比例为2.62∶1,纯母乳喂养者占89%,92%患儿并发颅内出血;农村多于城市。", "entities": [ { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "颅内出血" } ] }, { "text": "根据死亡率推算:我国婴儿颅内出血每年死亡2.5万人。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "颅内出血" } ] }, { "text": "近几年来,随着母乳喂养率不断提高,母乳维生素K相对不足,可能导致婴儿维生素K缺乏,因此维生素K缺乏已是危害我国婴儿健康的严重疾病之一。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "维生素K" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "维生素K缺乏" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "维生素K缺乏" } ] }, { "text": "1997年,首都儿科研究所和全国维生素K协作组在7省自治区调查了31649名婴儿维生素K缺乏出血症的情况,其发生率为2.4‰。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "维生素K" }, { "start_idx": 40, "end_idx": 48, "type": "dis", "entity": "维生素K缺乏出血症" } ] }, { "text": "【病因】本病的发病原因是由于体内维生素K缺乏,使凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ在肝内合成不足,从而引起出血。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "维生素K缺乏" }, { "start_idx": 24, "end_idx": 34, "type": "bod", "entity": "凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "肝" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "出血" } ] }, { "text": "在婴儿出生后第一小时内即可出现,可导致致命性出血。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "致命性出血" } ] }, { "text": "发病原因如下:1.母体缺乏维生素K,维生素K经胎盘转运不足,经放射免疫方法检测大部分新生儿脐血中维生素K缺乏。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "维生素K" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "维生素K" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "胎盘" }, { "start_idx": 31, "end_idx": 38, "type": "pro", "entity": "放射免疫方法检测" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "脐血" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "维生素K" } ] }, { "text": "2.孕期药物影响母亲怀孕期间服用影响维生素K代谢及合成的药物能导致新生儿期维生素K缺乏。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "孕期药物" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "维生素K" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "维生素K缺乏" } ] }, { "text": "如果长期应用抑制肠道内细菌生长的药物,如广谱抗生素和肠道内不易吸收的磺胺类药物,能抑制肠道内寄生的非致病菌,减少肠道内维生素K的合成,导致维生素K的缺乏。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肠道" }, { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "细菌" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "广谱抗生素" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肠道" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "磺胺类药物" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "肠道" }, { "start_idx": 49, "end_idx": 52, "type": "mic", "entity": "非致病菌" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "肠道" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "维生素K" }, { "start_idx": 69, "end_idx": 72, "type": "bod", "entity": "维生素K" } ] }, { "text": "摄入过量的维生素A,也能抑制维生素K2的肠内合成,并且因为维生素K1、K2均为脂溶性物质,其他脂溶性维生素(如A和D)都能影响其吸收。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "维生素A" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "维生素K2" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "肠" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "维生素K1" }, { "start_idx": 65, "end_idx": 70, "type": "bod", "entity": "脂溶性维生素" } ] }, { "text": "口服抗凝药物(如双香豆素)的结构与维生素K相似,可与维生素K竞争,减少凝血酶原在肝脏内的合成;孕妇服用抗惊厥药物后,可经胎盘输送,并以类似抗凝药物的作用来抑制维生素K的生成,引起新生儿维生素K的缺乏。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "抗凝药物" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "双香豆素" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "维生素K" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "维生素K" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "肝脏" }, { "start_idx": 51, "end_idx": 55, "type": "dru", "entity": "抗惊厥药物" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "胎盘" }, { "start_idx": 69, "end_idx": 72, "type": "dru", "entity": "抗凝药物" }, { "start_idx": 79, "end_idx": 82, "type": "bod", "entity": "维生素K" }, { "start_idx": 92, "end_idx": 95, "type": "bod", "entity": "维生素K" } ] }, { "text": "其原因为:1.单纯母乳喂养母乳喂养是婴儿最佳的喂养方式已得到公认,应该大力提倡和推广,但由于人乳中含维生素K的量极低,平均为15μg/L(牛奶中含量为60μg/L)。", "entities": [ { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "维生素K" } ] }, { "text": "故如单纯母乳喂养的婴儿未给予适当量的维生素K的补充,很容易导致维生素K的缺乏。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "维生素K" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "维生素K" } ] }, { "text": "据相关文献报道,90%以上的维生素K缺乏出血是发生在母乳喂养的婴儿中。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "维生素K缺乏出血" } ] }, { "text": "2.吸收利用功能不良新生儿(特别是早产儿)胆汁分泌有限,且胆汁中胆酸含量低,脂肪及脂溶性维生素的吸收有限,影响维生素K的吸收;新生儿及早产儿肝脏功能未发育成熟,使凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ在肝内合成不足,以至维生素K依赖因子生成减少。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "胆汁" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "胆汁" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "胆酸" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "脂肪" }, { "start_idx": 41, "end_idx": 46, "type": "bod", "entity": "脂溶性维生素" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "维生素K" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "肝脏" }, { "start_idx": 81, "end_idx": 91, "type": "bod", "entity": "凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ" }, { "start_idx": 93, "end_idx": 93, "type": "bod", "entity": "肝" }, { "start_idx": 102, "end_idx": 109, "type": "bod", "entity": "维生素K依赖因子" } ] }, { "text": "肠道细菌可合成一部分维生素K,但新生儿出生时肠道内无细菌,维生素K合成减少。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "肠道细菌" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "维生素K" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肠道" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "维生素K" } ] }, { "text": "发病原因如下:1.摄入不足新生儿吃奶量少且母乳中维生素含量低,初乳中几乎不含维生素K,如长期单纯母乳喂养,未及时添加辅食,未添加含维生素K丰富的蔬菜、水果,均可引起维生素K缺乏。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "维生素" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "维生素K" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "维生素K" }, { "start_idx": 82, "end_idx": 87, "type": "dis", "entity": "维生素K缺乏" } ] }, { "text": "2.吸收不良因慢性腹泻、溃疡性结肠炎、肠切除、囊性纤维化等疾病引起的小儿肠道吸收不良维生素K吸收障碍;胆道阻塞、胆瘘等胆道梗阻性疾病、胆汁缺乏性疾病,也可影响维生素K的吸收。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "慢性腹泻" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "溃疡性结肠炎" }, { "start_idx": 19, "end_idx": 21, "type": "pro", "entity": "肠切除" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "囊性纤维化" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肠道" }, { "start_idx": 34, "end_idx": 41, "type": "sym", "entity": "小儿肠道吸收不良" }, { "start_idx": 42, "end_idx": 49, "type": "sym", "entity": "维生素K吸收障碍" }, { "start_idx": 51, "end_idx": 57, "type": "sym", "entity": "胆道阻塞、胆瘘" }, { "start_idx": 59, "end_idx": 65, "type": "dis", "entity": "胆道梗阻性疾病" }, { "start_idx": 67, "end_idx": 73, "type": "dis", "entity": "胆汁缺乏性疾病" }, { "start_idx": 79, "end_idx": 82, "type": "bod", "entity": "维生素K" } ] }, { "text": "3.利用障碍新生儿肝炎、新生儿败血症及病毒感染等任何原因引起的肝脏损害均可影响维生素K依赖因子的合成。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肝炎" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "新生儿败血症" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "病毒感染" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肝脏" }, { "start_idx": 39, "end_idx": 46, "type": "bod", "entity": "维生素K依赖因子" } ] }, { "text": "4.合成减少肠道细菌也可合成部分维生素K,在婴儿于肠道菌落出现后,维生素K缺乏则明显减少,长期应用抗生素抑制肠道内的正常细菌的生长。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "mic", "entity": "肠道细菌" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "维生素K" }, { "start_idx": 25, "end_idx": 28, "type": "mic", "entity": "肠道菌落" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "维生素K缺乏" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "抗生素" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "肠道" } ] }, { "text": "【临床表现】临床上以出血为主要表现。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "出血" } ] }, { "text": "早发型者可有头颅血肿颅内、胸腔内出血脐带出血胃肠道出血脐部出血脐带结扎不良来解释,轻者为渗血,重者则出血不止;胃肠道出血则表现为不同程度吐血和便血。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "头颅" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "头颅血肿" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "颅内" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胸腔" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "颅内、胸腔内出血" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "脐带" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "脐带出血" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "胃肠道" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "胃肠道出血" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "脐部" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "脐部出血" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "脐带结扎" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "渗血" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "出血不止" }, { "start_idx": 55, "end_idx": 59, "type": "sym", "entity": "胃肠道出血" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "吐血" }, { "start_idx": 71, "end_idx": 72, "type": "sym", "entity": "便血" } ] }, { "text": "其次是皮肤出血,多见于分娩时挤压处,轻者为瘀点和紫癜,重者可形成大片瘀斑和血肿;也可见于采血及注射部位、术后伤口处渗血不止。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "皮肤出血" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "分娩" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "瘀点" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "紫癜" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "瘀斑" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "血肿" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "采血" } ] }, { "text": "颅内出血少见,但早产儿由于毛细血管脆性增加,往往预后不良。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "颅内出血" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "毛细血管" } ] }, { "text": "同时可伴有出血性贫血。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "出血性贫血" } ] }, { "text": "【实验室检查】凝血酶原时间延长,多数延长至正常对照的2倍以上,轻度维生素K缺乏只有凝血酶原时间延长,临床无出血倾向。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "实验室检查" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "凝血酶原时间延长" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "维生素K缺乏" }, { "start_idx": 41, "end_idx": 48, "type": "sym", "entity": "凝血酶原时间延长" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "出血" } ] }, { "text": "陶土部分凝血活酶时间延长,凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ因子活性明显降低,第Ⅶ因子首先降至最低,第Ⅶ因子减低后凝血酶原水平即下降但较缓慢,第Ⅸ、Ⅹ因子也有不同程度地减少。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "陶土部分凝血活酶时间延长" }, { "start_idx": 13, "end_idx": 31, "type": "sym", "entity": "凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ因子活性明显降低" }, { "start_idx": 33, "end_idx": 42, "type": "sym", "entity": "第Ⅶ因子首先降至最低" }, { "start_idx": 44, "end_idx": 63, "type": "sym", "entity": "第Ⅶ因子减低后凝血酶原水平即下降但较缓慢" }, { "start_idx": 65, "end_idx": 79, "type": "sym", "entity": "第Ⅸ、Ⅹ因子也有不同程度地减少" } ] }, { "text": "凝血酶原检测是维生素K缺乏的可靠证据。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "凝血酶原检测" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "维生素K缺乏" } ] }, { "text": "如疑有颅内出血者应进行B超、CT或MRI检查,以了解出血情况。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "颅内出血" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "B超" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "CT" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "MRI检查" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "出血" } ] }, { "text": "必要时可行维生素K的检测。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "维生素K的检测" } ] }, { "text": "多见于单纯母乳喂养儿,生后3个月内的婴儿,未接受过维生素K预防。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "维生素K" } ] }, { "text": "(二)观察病情新生儿出血症多见于出生后1~7天,以胃肠道出血凝血酶原时间延长,血小板、出血时间均正常,予维生素K治疗效果良好,数小时或24小时后出血倾向明显好转。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "新生儿出血症" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "胃肠道" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "胃肠道出血" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "凝血酶原时间延长" }, { "start_idx": 39, "end_idx": 49, "type": "sym", "entity": "血小板、出血时间均正常" }, { "start_idx": 52, "end_idx": 55, "type": "dru", "entity": "维生素K" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "出血" } ] }, { "text": "不伴其他部位出血的患儿,易误诊为颅内感染,而迟发性新生儿出血症表现为突然起病,无明显感染中毒症状,贫血发展迅速而严重,故可与颅内感染相鉴别。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "出血" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "颅内感染" }, { "start_idx": 22, "end_idx": 30, "type": "dis", "entity": "迟发性新生儿出血症" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "感染" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "中毒" }, { "start_idx": 49, "end_idx": 57, "type": "sym", "entity": "贫血发展迅速而严重" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "颅内感染" } ] }, { "text": "辅助检查也有助于该诊断,脑脊液检查呈现均匀一致的血性和皱缩红细胞,但脑脊液检查正常也不可以完全排除此病,且病情危重者不宜进行该项检查。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "辅助检查" }, { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 19, "end_idx": 31, "type": "sym", "entity": "均匀一致的血性和皱缩红细胞" }, { "start_idx": 34, "end_idx": 38, "type": "pro", "entity": "脑脊液检查" } ] }, { "text": "【治疗】有出血现象时,应立即注射维生素K2mg,可迅速改善出血,胃肠道出血者应暂禁食,给予静脉营养支持,止血后应根据适当情况纠正贫血,严重者可输全血或血浆10~20ml/kg。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "出血" }, { "start_idx": 14, "end_idx": 20, "type": "pro", "entity": "注射维生素K2" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "出血" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "胃肠道出血" }, { "start_idx": 45, "end_idx": 50, "type": "pro", "entity": "静脉营养支持" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "贫血" }, { "start_idx": 72, "end_idx": 73, "type": "dru", "entity": "全血" }, { "start_idx": 75, "end_idx": 76, "type": "dru", "entity": "血浆" } ] }, { "text": "如有颅内出血,首先要加强护理,保持安静,维持通气,抬高头肩部,推迟喂奶,控制补液;如有高声尖叫、频繁呕吐、反复抽搐等表现,应对症止惊,降低颅内压,恢复脑细胞功能;同时要及时止血、纠正贫血。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "颅内出血" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "头" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肩" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "高声尖叫" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "频繁呕吐" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "反复抽搐" }, { "start_idx": 64, "end_idx": 65, "type": "pro", "entity": "止惊" }, { "start_idx": 67, "end_idx": 71, "type": "pro", "entity": "降低颅内压" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "脑细胞" }, { "start_idx": 86, "end_idx": 87, "type": "pro", "entity": "止血" }, { "start_idx": 91, "end_idx": 92, "type": "sym", "entity": "贫血" } ] }, { "text": "严重者可手术清除血肿。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "手术清除血肿" } ] }, { "text": "【预防】预防新生儿维生素K缺乏症应从孕妇开始,分娩前数周即可口服维生素K20mg,能预防新生儿维生素K缺乏所致的低凝血酶原血症。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "维生素K缺乏症" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "分娩" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "维生素K" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "维生素K缺乏" }, { "start_idx": 56, "end_idx": 62, "type": "dis", "entity": "低凝血酶原血症" } ] }, { "text": "乳母应多吃蔬菜、水果以提高乳汁中维生素K的含量。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "维生素K" } ] }, { "text": "自从1961年美国儿科学会营养委员会提出所有新生儿应在出生后肌内注射维生素K10.5~1mg作为预防新生儿出血以来,维生素K1用来预防和根治新生儿维生素K缺乏性出血已在许多国家得到广泛应用。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dep", "entity": "儿科" }, { "start_idx": 30, "end_idx": 38, "type": "pro", "entity": "肌内注射维生素K1" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "出血" }, { "start_idx": 64, "end_idx": 68, "type": "pro", "entity": "维生素K1" }, { "start_idx": 85, "end_idx": 93, "type": "dis", "entity": "维生素K缺乏性出血" } ] }, { "text": "荷兰CornelissenEA等人实验证明,在新生儿出生后3个月内,每周口服维生素K1mg可有效纠正维生素K缺乏且不会引起维生素K在体内的积聚。", "entities": [ { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "维生素K" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "维生素K缺乏" }, { "start_idx": 61, "end_idx": 64, "type": "dru", "entity": "维生素K" } ] }, { "text": "加拿大儿科协会建议足月产的新生儿应在出生后6小时内口服或肌注维生素K1mg;早产儿、低体重儿及难产儿均需在产后6小时内肌注维生素K1mg;因脂肪吸收不良而有迟发性出血性疾病危险性的新生儿需每天口服维生素K1mg或每月肌注维生素K一次以预防维生素K缺乏性出血症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dep", "entity": "儿科" }, { "start_idx": 25, "end_idx": 33, "type": "pro", "entity": "口服或肌注维生素K" }, { "start_idx": 59, "end_idx": 64, "type": "pro", "entity": "肌注维生素K" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "脂肪" }, { "start_idx": 78, "end_idx": 85, "type": "dis", "entity": "迟发性出血性疾病" }, { "start_idx": 98, "end_idx": 101, "type": "dru", "entity": "维生素K" }, { "start_idx": 108, "end_idx": 113, "type": "pro", "entity": "肌注维生素K" }, { "start_idx": 119, "end_idx": 128, "type": "dis", "entity": "维生素K缺乏性出血症" } ] }, { "text": "我国林良明等于2002年报道中国7省协作对19751例活产婴儿进行对照研究发现,采用给婴儿出生后口服维生素K12mg,以后每隔10天1次,服满3个月,共10次,对预防维生素K缺乏性出血有相当好的效果。", "entities": [ { "start_idx": 50, "end_idx": 54, "type": "dru", "entity": "维生素K1" }, { "start_idx": 89, "end_idx": 97, "type": "dis", "entity": "维生素K缺乏性出血" } ] }, { "text": "参考文献1.程国强,洪建国.痰液分析在哮喘临床研究中的作用.国外医学儿科学分册,2000,27(2):82-852.中华医学会儿科学分会呼吸学组儿科支气管镜协作组.儿科支气管镜术指南(2009年版).中华儿科杂志,2009,47(10):740-7403.EfratiO,Sadeh-GornikU,Modan-MosesD,etal.Flexiblebronchoscopyandbronchoalveolarlavageinpediatricpatientswithlungdisease.PediatrCritCareMed,2009,10(1):80-844.HasanRA,ReddyR.Sedationwithpropofolforflexiblebronchoscopyinchildren.PediatrPulmonol,2009,44(4):373-3735.NielsonDW,KuPLandEggerM.Topicallidocaineexaggerateslaryngomalaciaduringflexiblebronchoscopy.AmJRespirCritCareMed,2000,161(1):147-1476.Vuori-HolopainenE,PeltolaH.Reappraisaloflungtap:reviewofanoldmethodforbetteretiologicdiagnosisofchildhoodpneumonia.ClinInfectDis,2001,32(5):715-715", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "痰液" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "哮喘" }, { "start_idx": 34, "end_idx": 35, "type": "dep", "entity": "儿科" }, { "start_idx": 63, "end_idx": 64, "type": "dep", "entity": "儿科" }, { "start_idx": 72, "end_idx": 73, "type": "dep", "entity": "儿科" }, { "start_idx": 74, "end_idx": 77, "type": "equ", "entity": "支气管镜" }, { "start_idx": 82, "end_idx": 83, "type": "dep", "entity": "儿科" }, { "start_idx": 84, "end_idx": 88, "type": "pro", "entity": "支气管镜术" }, { "start_idx": 102, "end_idx": 103, "type": "dep", "entity": "儿科" } ] }, { "text": "参考文献1.蒋明.风湿病学.北京:科学出版社,19982.李永柏,杨锡强.自身抗体测定及其临床意义.中国实用儿科杂志,2000,15(11):691-6933.滕庆,何晓唬.隐匿性类风湿因子与幼年类风湿关节炎.实用儿科临床杂志,1997,12:2704.BehrmanRE,KliegmanRM,JensonHB.NelsonTextbookofPediatrics.16thed.Philadelphia:W.B.SaunersCo,20005.周厚清,董敏,马路.ANA、抗dsDNA和ENA多肽抗体与自身免疫性疾病的关系.中国卫生检验杂志,2008:18(12):2675-26756.李永柏.自身抗体检测及其临床应用.实用儿科临床杂志,2005,20(11):1062-10627.FerucciED,MajkaDS,ParrishLA,etal.AntibodiesagainstcycliccitrullinatedpeptideareassociatedwithHLA-DR4insimplexandmultiplexpolyarticular-onsetjuvenilerheumatoidarthritis.ArthritisRheum,2005Jan,52(1):239-239", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "风湿病" }, { "start_idx": 87, "end_idx": 94, "type": "bod", "entity": "隐匿性类风湿因子" }, { "start_idx": 96, "end_idx": 103, "type": "dis", "entity": "幼年类风湿关节炎" }, { "start_idx": 234, "end_idx": 236, "type": "bod", "entity": "ANA" }, { "start_idx": 238, "end_idx": 243, "type": "bod", "entity": "抗dsDNA" }, { "start_idx": 245, "end_idx": 251, "type": "bod", "entity": "ENA多肽抗体" }, { "start_idx": 253, "end_idx": 259, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 300, "end_idx": 305, "type": "pro", "entity": "自身抗体检测" } ] }, { "text": "五、免疫性溶血性贫血由于免疫因素如抗体、补体等导致红细胞损伤而过早地破坏,产生溶血和贫血症状者称为免疫性溶血性贫血。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "免疫性溶血性贫血" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "抗体" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "补体" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "红细胞" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "溶血" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "贫血" }, { "start_idx": 49, "end_idx": 56, "type": "dis", "entity": "免疫性溶血性贫血" } ] }, { "text": "可发生于任何年龄阶段,小儿时期最常见的是新生儿同族免疫性贫血(见有关章节),其次是自身免疫性溶血性贫血(autoimmunehemolyticanemia,AIHA)。", "entities": [ { "start_idx": 20, "end_idx": 29, "type": "dis", "entity": "新生儿同族免疫性贫血" }, { "start_idx": 41, "end_idx": 50, "type": "dis", "entity": "自身免疫性溶血性贫血" }, { "start_idx": 52, "end_idx": 76, "type": "dis", "entity": "autoimmunehemolyticanemia" }, { "start_idx": 78, "end_idx": 81, "type": "dis", "entity": "AIHA" } ] }, { "text": "AIHA是一种获得性免疫性贫血,由患儿体内产生抗自身红细胞膜抗原的抗体,引起红细胞过早地破坏而产生溶血性贫血所致。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "AIHA" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "获得性免疫性贫血" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "红细胞膜" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "抗体" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "红细胞" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "溶血性贫血" } ] }, { "text": "【分类】AIHA的分类有两种,一种是根据病因进行分类,另一种是根据抗体的种类进行分类。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "AIHA" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "抗体" } ] }, { "text": "(2)继发性:发病率约占80%,常见病因有:1)感染:可由细菌、病毒、支原体或疫苗接种等引起,病原体包括伤寒、链球菌、金黄色葡萄球菌、结核、肝炎病毒、巨细胞包涵体病毒、EB病毒、疱疹病毒、流感病毒、腺病毒、腮腺炎病毒及肺炎支原体等。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "感染" }, { "start_idx": 32, "end_idx": 33, "type": "mic", "entity": "病毒" }, { "start_idx": 35, "end_idx": 37, "type": "mic", "entity": "支原体" }, { "start_idx": 39, "end_idx": 40, "type": "dru", "entity": "疫苗" }, { "start_idx": 47, "end_idx": 49, "type": "mic", "entity": "病原体" }, { "start_idx": 52, "end_idx": 53, "type": "mic", "entity": "伤寒" }, { "start_idx": 55, "end_idx": 57, "type": "mic", "entity": "链球菌" }, { "start_idx": 59, "end_idx": 65, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 67, "end_idx": 68, "type": "mic", "entity": "结核" }, { "start_idx": 70, "end_idx": 73, "type": "mic", "entity": "肝炎病毒" }, { "start_idx": 75, "end_idx": 82, "type": "mic", "entity": "巨细胞包涵体病毒" }, { "start_idx": 84, "end_idx": 87, "type": "mic", "entity": "EB病毒" }, { "start_idx": 89, "end_idx": 92, "type": "mic", "entity": "疱疹病毒" }, { "start_idx": 94, "end_idx": 97, "type": "mic", "entity": "流感病毒" }, { "start_idx": 99, "end_idx": 101, "type": "mic", "entity": "腺病毒" }, { "start_idx": 103, "end_idx": 107, "type": "mic", "entity": "腮腺炎病毒" }, { "start_idx": 109, "end_idx": 113, "type": "mic", "entity": "肺炎支原体" } ] }, { "text": "2)免疫性疾病:常见于系统性红斑狼疮、类风湿性关节炎、皮肌炎、免疫性血小板减少症、无丙种球蛋白血症、异常丙种球蛋白血症等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "免疫性疾病" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "类风湿性关节炎" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 31, "end_idx": 39, "type": "dis", "entity": "免疫性血小板减少症" }, { "start_idx": 41, "end_idx": 48, "type": "dis", "entity": "无丙种球蛋白血症" }, { "start_idx": 50, "end_idx": 58, "type": "dis", "entity": "异常丙种球蛋白血症" } ] }, { "text": "3)恶性肿瘤:如白血病、淋巴瘤、霍奇金病等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "白血病" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "霍奇金病" } ] }, { "text": "4)多种药物:可通过半抗原药物依赖性非特异性抗体(如青霉素类、头孢霉素类等)或通过免疫复合物(如奎宁、奎尼丁等)或诱导真性自身抗体(如甲基多巴、左旋多巴等)而破坏红细胞,发生溶血性贫血。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "青霉素" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "头孢霉素" }, { "start_idx": 48, "end_idx": 49, "type": "dru", "entity": "奎宁" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "奎尼丁" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "甲基多巴" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "左旋多巴" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "红细胞" }, { "start_idx": 87, "end_idx": 91, "type": "dis", "entity": "溶血性贫血" } ] }, { "text": "2.根据抗体种类分类根据自身抗体作用在红细胞引起溶血所需要的最适温度可将AIHA分为温抗体型和冷抗体型两种,前者通常为IgG,少数为IgA和IgM;IgG亚类则以IgG1和IgG3为主,而IgG2及IgG4少见,最适温度为37℃;后者较少见,抗体属IgM,少数为IgG,最适温度为4℃。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "抗体" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "红细胞" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "溶血" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "IgG" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "IgA" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "IgM" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "IgG" }, { "start_idx": 145, "end_idx": 146, "type": "bod", "entity": "抗体" }, { "start_idx": 148, "end_idx": 150, "type": "bod", "entity": "IgM" }, { "start_idx": 155, "end_idx": 157, "type": "bod", "entity": "IgG" } ] }, { "text": "两类抗体引起的AIHA,其发病机制、诊断、治疗方法和预后不尽相同。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "AIHA" } ] }, { "text": "【发病机制】AIHA的发病机制尚未完全阐明。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "AIHA" } ] }, { "text": "自身温抗体型通过IgG可变区Fab段吸附于红细胞膜上,其恒定区Fc段则暴露于膜外,一是通过激活补体破坏红细胞,二是Fc段可被位于单核巨噬细胞膜上的Fc受体所识别,借此单核巨噬细胞便可进行吞噬和毒性溶解被抗体包被的红细胞。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "IgG" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "红细胞膜" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "补体" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "红细胞" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "受体" }, { "start_idx": 83, "end_idx": 88, "type": "bod", "entity": "单核巨噬细胞" }, { "start_idx": 101, "end_idx": 102, "type": "bod", "entity": "抗体" }, { "start_idx": 106, "end_idx": 108, "type": "bod", "entity": "红细胞" } ] }, { "text": "冷抗体型免疫性溶血性贫血可分为冷凝集素综合征(coldhemagglutininsyndrome)或冷凝集病和阵发性寒冷性血红蛋白尿;前者由病儿自身冷凝集素IgM引起,少数可由IgG或IgA引起;后者为IgG型冷抗体。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dis", "entity": "冷抗体型免疫性溶血性贫血" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "冷凝集素综合征" }, { "start_idx": 23, "end_idx": 47, "type": "dis", "entity": "coldhemagglutininsyndrome" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "冷凝集病" }, { "start_idx": 55, "end_idx": 65, "type": "dis", "entity": "阵发性寒冷性血红蛋白尿" }, { "start_idx": 74, "end_idx": 77, "type": "bod", "entity": "冷凝集素" }, { "start_idx": 101, "end_idx": 107, "type": "bod", "entity": "IgG型冷抗体" } ] }, { "text": "冷抗体型常继发于各种感染,可能由各种病原微生物和人类红细胞表面抗原相类似引起,所谓交叉抗原性(crossantigenicity);也有人认为病原微生物代谢产物在体内与红细胞膜的蛋白质结合,使蛋白变性,成为一种新的抗原,因而刺激人体免疫系统产生自身抗体。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "感染" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "抗原" }, { "start_idx": 89, "end_idx": 91, "type": "bod", "entity": "蛋白质" } ] }, { "text": "AIHA还可与免疫系统增生性疾病并发,如淋巴细胞白血病、恶性淋巴瘤等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "AIHA" }, { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "免疫系统增生性疾病" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "淋巴细胞白血病" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "恶性淋巴瘤" } ] }, { "text": "此外,在胶原血管疾病中也常有AI淋巴细胞白血病药物诱发的AIHA主要有三种类型:①青霉素型:亦称药物吸附型。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "胶原血管疾病" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "AI淋巴细胞白血病" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "AIHA" } ] }, { "text": "药物吸附于红细胞表面形成新的抗原,免疫系统制造抗体,通常是IgG与之结合而发生溶血。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "红细胞" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "抗原" } ] }, { "text": "青霉素、先锋霉素、四环素等所引起的AIHA均属这一类型。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "青霉素" }, { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "先锋霉素" } ] }, { "text": "②甲基青霉素型(methyldopatype):α甲基多巴引起的AIHA属自身免疫性,60%见于HLA-B7阳性患儿;③免疫复合物型:这是由于IgM与药物反应,激活了补体系统,C3b沉积于红细胞表面,进而导致巨噬细胞对带有C3b的红细胞发生攻击和吞噬。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dru", "entity": "甲基青霉素型" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "IgM" } ] }, { "text": "这种抗体与红细胞膜上的血型P抗原结合,通过激活补体而发生溶血性贫血。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "抗体" } ] }, { "text": "温抗体型自身免疫性溶血性贫血:【临床表现】有三种临床类型:1.急性暂时型占70%~8温抗体型自身免疫性溶血性贫血的儿童,偶见于新生儿,男多于女。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "dis", "entity": "温抗体型自身免疫性溶血性贫血" }, { "start_idx": 42, "end_idx": 55, "type": "dis", "entity": "温抗体型自身免疫性溶血性贫血" } ] }, { "text": "起病大多急骤,伴有虚脱、苍白、黄疸、发热、血红蛋白尿等,病程呈自限性,通常2周内自行停止,最长不超过6个月。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "虚脱" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "血红蛋白尿" } ] }, { "text": "严重溶血者,可发生急性肾功能不全,出现少尿、无尿和氮质血症等。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "少尿" } ] }, { "text": "部分病例起病也可稍缓慢,主要表现为疲劳和苍白。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "苍白" } ] }, { "text": "由青霉素引起者,与青霉素剂量有关,若每日用量超过120万单位,则很少出现溶血。", "entities": [ { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "溶血" } ] }, { "text": "偶尔继发于系统性红斑狼疮等结缔组织病。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "结缔组织病" } ] }, { "text": "起病缓慢,主要症状有贫血、黄疸、肝脾大等,溶血可持续数月或数年,最长可达20年,可反复发作。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "贫血" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "黄疸" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "肝脾大" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "溶血" } ] }, { "text": "合并感染可加重病情,甚肝脾大溶血危象。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "肝脾大" } ] }, { "text": "常并发其他血细胞成分异常,如合并中性粒细胞或血小板减少(Even综合征)。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "中性粒细胞" } ] }, { "text": "Even综合征者,常可并发慢性疾病如系统性红斑狼疮等,因此预后大多不良。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "Even综合征" } ] }, { "text": "3.抗人球蛋白试验(Coombs)阴性型与红细胞膜结合的抗体分子数过少(<260)有关。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "红细胞膜" } ] }, { "text": "【实验室检查】1.外周血象大多数病例贫血严重,血红蛋白<60g/L,球形和嗜多色性红细胞多见,网织红细胞可高达50%,可见有核红细胞。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "贫血" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "核红细胞" } ] }, { "text": "慢性迁延型者,网织红细胞大多减少,主要原因是IgG抗体可以与幼红细胞和网织红细胞结合,使骨髓中的幼红细胞和网织红细胞减少,严重时可发生再障危象。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "IgG抗体" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "幼红细胞" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "网织红细胞" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "骨髓" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "幼红细胞" } ] }, { "text": "白细胞总数通常升高,可出现类白血病反应。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "白血病" } ] }, { "text": "3.Coombs试验直接试验强阳性,而间接试验(测定血清中游离的抗红细胞抗体)阴性,偶尔间接试验也可呈阳性,与预后有关。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "Coombs试验" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "抗红细胞抗体" } ] }, { "text": "从有些病人血清中或从病人红细胞上洗脱下来的抗体还可以与其他人的红细胞发生凝集,这些抗体被认为是泛凝集素(panagglutinins),事实上,它们约与70%左右病人的红细胞Rh系统抗原呈特异性结合反应。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "红细胞" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "凝集素" } ] }, { "text": "部分病例的红细胞上可检测到与IgG结合的补体成分,通常为C3b。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "红细胞" } ] }, { "text": "一般来说,红细胞表面至少有260~500个抗体分子时,Coombs试验才能出现阳性,因此少数病例Coombs试验可因敏感度不足而呈阴性反应。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "抗体" } ] }, { "text": "此时,需要采用特殊的诊断试验(如125I葡萄球菌蛋白A试验、放射免疫直接抗人球蛋白试验等)才能证实抗红细胞抗体的存在。", "entities": [ { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "抗体" } ] }, { "text": "4.胆红素和珠蛋白测定血清间接胆红素增加,尿中尿胆原增加,结合珠蛋白降低或消失。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "胆红素" } ] }, { "text": "【诊断与鉴别诊断】近数月内无输胆红素特殊药物接触史,根据临床表现和实验室检查,尤其是直结合珠蛋白mbs试验阳性者可确诊,但后者阴性时不能否定。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "胆红素" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "直结合珠蛋白" } ] }, { "text": "本病应与其他溶血性贫血如珠蛋白生成障碍性贫血、溶血尿毒综合征、血栓性血小板减少性紫癜、传染性单核细胞增多症合并溶血等鉴别。", "entities": [ { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "溶血尿毒综合征" } ] }, { "text": "【治疗】1.一般治疗积极控制原发病,防治感染,以免引起溶血危象。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "感染" } ] }, { "text": "危重病例需注意水电解质平衡及心肾功能,溶血危象者宜采取碱化尿液的措施,应用低分子右旋糖苷以防DIC发生等。", "entities": [ { "start_idx": 37, "end_idx": 43, "type": "dru", "entity": "低分子右旋糖苷" } ] }, { "text": "2.药物治疗(1)激素:为温抗体型AIHA治疗的首选药,其主要药理机制是通过抑制抗体的产生、减少红细胞表面被覆盖的抗体量、干扰巨噬细胞表面的IgG及补体C3b受体以阻止其吞噬红细胞作用。", "entities": [ { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "红细胞" }, { "start_idx": 93, "end_idx": 95, "type": "bod", "entity": "红细胞" } ] }, { "text": "当出现溶血危象或再生障碍危象的重症或常规剂量无效者,可试用大剂量冲击疗法。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "溶血危象" } ] }, { "text": "也可应用大剂量地塞米松10mg/(M2•d)静滴,有效后改用泼尼松口服。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "地塞米松" } ] }, { "text": "(2)丙种球蛋白静滴:剂量400mg/(kg•d),连用5天为1个疗程,每隔3~5天可再用,至Hb达正常水平后1个月,再用1~2个疗程至Coombs试验转阴性后停药。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "丙种球蛋白静滴" }, { "start_idx": 68, "end_idx": 75, "type": "pro", "entity": "Coombs试验" } ] }, { "text": "(3)免疫抑制剂:皮质激素治疗无效或泼尼松维持量每日>10~15mg者,可应用免疫抑制剂或联合用药。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "皮质激素治疗" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "泼尼松" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "常用制剂有硫唑嘌呤(AZP)、6-巯基嘌呤(6-MP)、环磷酰胺(CTX)、环胞霉素A(CsA)等。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "AZP" }, { "start_idx": 15, "end_idx": 20, "type": "dru", "entity": "6-巯基嘌呤" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "6-MP" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "CTX" }, { "start_idx": 38, "end_idx": 42, "type": "dru", "entity": "环胞霉素A" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "CsA" } ] }, { "text": "3.脾切除对上述治疗疗效均差的温抗体型AIHA者,可考虑行脾切除术,有效率可达60%。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "脾切除" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "脾切除术" } ] }, { "text": "4.输血及血浆置换AIHA患儿一般不宜作输血治疗,因为,体内自身抗红细胞抗体可致自体或异体红细胞破坏溶血,且自身抗体尚可干扰血型配型等特点,输血后很容易发生更严重的溶血反应。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "输血治疗" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "抗体" }, { "start_idx": 40, "end_idx": 51, "type": "sym", "entity": "自体或异体红细胞破坏溶血" }, { "start_idx": 82, "end_idx": 83, "type": "dis", "entity": "溶血" } ] }, { "text": "如果确因病情严重而需输血者,也应严格掌握适应证:①急性溶血进展迅速而致严重贫血(Hb<40g/L),或发生溶血危象,心功能失代偿或严重脑缺氧和全身衰竭者;②急性溶血不能用糖皮质激素和免疫抑制剂控制者。", "entities": [ { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "心功能失代偿" }, { "start_idx": 91, "end_idx": 95, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "用洗涤浓缩红细胞可减少输血反应。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "红细胞" } ] }, { "text": "六、α1-抗胰蛋白酶缺乏症α1-抗胰蛋白酶缺乏症(α1-antitrypsindeficiency)简称α1-AT缺乏症,是一种常染色体隐性遗传性疾病。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "α1-抗胰蛋白酶缺乏症" }, { "start_idx": 13, "end_idx": 23, "type": "dis", "entity": "α1-抗胰蛋白酶缺乏症" }, { "start_idx": 25, "end_idx": 48, "type": "dis", "entity": "α1-antitrypsindeficiency" }, { "start_idx": 52, "end_idx": 59, "type": "dis", "entity": "α1-AT缺乏症" } ] }, { "text": "以婴儿期出现胆汁淤积性黄疸、进行性肝功能损害和青年后期出现肺气肿为主要临床表现,常有家族史。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "胆汁淤积性黄疸" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "进行性肝功能损害" }, { "start_idx": 23, "end_idx": 31, "type": "sym", "entity": "青年后期出现肺气肿" } ] }, { "text": "儿童期累及肺部者罕见。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肺部" } ] }, { "text": "目前普遍认为蛋白酶溶解学说是肺气肿的发病机制。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "蛋白酶" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肺气肿" } ] }, { "text": "α1-AT和其他抗蛋白酶在灭活死亡细菌及中性粒细胞释放的蛋白溶解酶过程中起重要作用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "α1-AT" }, { "start_idx": 8, "end_idx": 11, "type": "mic", "entity": "抗蛋白酶" }, { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "细菌" }, { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "中性粒细胞" }, { "start_idx": 28, "end_idx": 32, "type": "mic", "entity": "蛋白溶解酶" } ] }, { "text": "α1-AT严重缺乏者在炎症等刺激时不能提高分泌,而中性粒细胞和巨噬细胞在防御作用中释放的蛋白溶解酶过多积聚,引起肺组织蛋白溶解破坏和肺气肿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "α1-AT" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "炎症" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "不能提高分泌" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 44, "end_idx": 48, "type": "mic", "entity": "蛋白溶解酶" }, { "start_idx": 56, "end_idx": 60, "type": "bod", "entity": "肺组织蛋白" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "肺气肿" } ] }, { "text": "少数患儿可出现呼吸困难、咳喘、弥漫性肺气肿及桶状胸、杵状指(趾),肺部叩诊为过清音,伴生长发育障碍。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "咳喘" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "弥漫性肺气肿" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "桶状胸" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "杵状指(趾)" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肺部" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "过清音" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "生长发育障碍" } ] }, { "text": "胸部X线检查可见两侧肺气肿和膈肌下降。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "胸部X线检查" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肺气肿" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "膈肌下降" } ] }, { "text": "吸烟可显著增加发生肺气肿的危险性。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "肺气肿" } ] }, { "text": "血清α1-AT定量及胰蛋白酶抑制活性测定有助诊断。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "ite", "entity": "血清α1-AT定量及胰蛋白酶抑制活性测定" } ] }, { "text": "酶替代治疗可能成为本症的主要治疗方法。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "酶替代治疗" } ] }, { "text": "美国FDA已批准使用人血源性纯化酶用于某些纯合子患者。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "mic", "entity": "人血源性纯化酶" } ] }, { "text": "通过重组DNA技术亦已获得纯化酶。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "重组DNA技术" }, { "start_idx": 13, "end_idx": 15, "type": "mic", "entity": "纯化酶" } ] }, { "text": "重症患者可能需要外科干预,包括肺减容术和肺移植。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "肺减容术" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "肺移植" } ] }, { "text": "三、室管膜瘤室管膜瘤(ependymoma)发生于脑室的室管膜细胞,占儿童中枢神经系统原发性肿瘤5%~10%。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "室管膜瘤" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "室管膜瘤" }, { "start_idx": 11, "end_idx": 20, "type": "dis", "entity": "ependymoma" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "脑室" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "室管膜细胞" }, { "start_idx": 35, "end_idx": 47, "type": "dis", "entity": "儿童中枢神经系统原发性肿瘤" } ] }, { "text": "好发于后颅窝,约60%。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "后颅窝" } ] }, { "text": "瘤体多位于脑室内,少数在脑室旁组织内,呈缓慢、浸润性生长。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "瘤体" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "脑室" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "脑室旁" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "组织" } ] }, { "text": "瘤细胞亦可脱落于蛛网膜下腔产生播散性种植。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "瘤细胞" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "蛛网膜下腔" } ] }, { "text": "肿瘤呈灰白色或紫红色质软,有的较硬,呈颗粒状,可有钙化。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肿瘤" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "肿瘤呈灰白色或紫红色" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "质软" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "有的较硬" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "呈颗粒状" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "可有钙化" } ] }, { "text": "有包膜分界体积较大时多有囊肿形成瘤细胞较致密排列成腺泡或腺管状,形成假菊形团。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "包膜" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "有包膜分界" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "囊肿" }, { "start_idx": 5, "end_idx": 15, "type": "sym", "entity": "体积较大时多有囊肿形成" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "瘤细胞" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "瘤细胞较致密" }, { "start_idx": 22, "end_idx": 30, "type": "sym", "entity": "排列成腺泡或腺管状" } ] }, { "text": "核为圆形或椭圆形,间质内有胶质纤维形成的网状结构,血管较多。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "间质" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "胶质纤维" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血管" } ] }, { "text": "初期可有头痛、呕吐颅内压增高症状。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "头痛" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "呕吐" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "颅内压增高" } ] }, { "text": "辅助检查CT及MRI检查可清楚地显示肿瘤大小、形状、有无钙化及其与周围结构的关系。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "CT" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "MRI检查" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肿瘤" } ] }, { "text": "手术切除为治疗本病的主要手段,如肿瘤自第四脑室底部长出,术中可残留一薄层肿瘤组织,以免脑干损伤,术中务必使原先脑脊液梗阻放疗和化疗。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "手术切除" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "肿瘤" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "第四脑室底部" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "肿瘤组织" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "脑干" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "脑脊液" }, { "start_idx": 55, "end_idx": 59, "type": "sym", "entity": "脑脊液梗阻" }, { "start_idx": 60, "end_idx": 61, "type": "pro", "entity": "放疗" }, { "start_idx": 63, "end_idx": 64, "type": "pro", "entity": "化疗" } ] }, { "text": "间变性肿瘤或幕下肿瘤,应做全脑及脊髓轴的放疗,防止肿瘤种植到脑的其他部位或脊髓;低度肿瘤放疗范围尚有争议,目前多数主张局部照射,而不作大范围预防性放疗。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "间变性肿瘤" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "幕下肿瘤" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "全脑" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "脊髓轴" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "放疗" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "肿瘤" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "脑" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "脊髓" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "放疗" }, { "start_idx": 73, "end_idx": 74, "type": "pro", "entity": "放疗" } ] }, { "text": "本病的预后与肿瘤切除程度有关。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "肿瘤切除" } ] }, { "text": "三、肾穿刺操作步骤1.负压抽吸法嘱患儿排空膀胱后俯卧于检查台上,体位端正,腹部肋下垫一直径为10~15cm的硬枕,以固定肾脏,两上肢置于头前。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肾穿刺" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "负压抽吸法" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "膀胱" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "腹部" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "肾脏" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "上肢" }, { "start_idx": 68, "end_idx": 68, "type": "bod", "entity": "头" } ] }, { "text": "常规皮肤消毒、铺巾,B超定位后行局部麻醉(2%利多卡因1ml皮下注射);以22号腰穿针为探针,如探及肾包囊,可见针芯随呼吸头尾摆动,计算测得距离;在穿刺点上做一小切口,进针深度=皮肾距离(cm)+取肾组织长度(1.0~2.0cm)+肾退让距离(1~1.5cm);进针方向与肾纵轴垂直。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "皮肤消毒" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "B超定位" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "局部麻醉" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "利多卡因" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "皮下注射" }, { "start_idx": 40, "end_idx": 42, "type": "equ", "entity": "腰穿针" }, { "start_idx": 44, "end_idx": 45, "type": "equ", "entity": "探针" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "肾包囊" }, { "start_idx": 56, "end_idx": 57, "type": "equ", "entity": "针芯" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "皮肾" }, { "start_idx": 99, "end_idx": 101, "type": "bod", "entity": "肾组织" }, { "start_idx": 116, "end_idx": 116, "type": "bod", "entity": "肾" }, { "start_idx": 136, "end_idx": 136, "type": "bod", "entity": "肾" } ] }, { "text": "将穿刺针直至肾包膜处,拔出针芯,于针管内放置针栓(防止肾组织吸至负压注射器内),连接负压装置,嘱受检者屏气,肾固定后在助手配合下进行穿刺,一次穿刺动作于0.5秒内完成。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "equ", "entity": "穿刺针" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肾包膜" }, { "start_idx": 13, "end_idx": 14, "type": "equ", "entity": "针芯" }, { "start_idx": 17, "end_idx": 18, "type": "equ", "entity": "针管" }, { "start_idx": 22, "end_idx": 23, "type": "equ", "entity": "针栓" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肾组织" }, { "start_idx": 32, "end_idx": 36, "type": "equ", "entity": "负压注射器" }, { "start_idx": 42, "end_idx": 45, "type": "equ", "entity": "负压装置" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "肾" }, { "start_idx": 66, "end_idx": 67, "type": "pro", "entity": "穿刺" }, { "start_idx": 71, "end_idx": 72, "type": "pro", "entity": "穿刺" } ] }, { "text": "2.自动穿刺枪20世纪90年代后出现了应用穿刺枪作经皮肾穿刺活检的报道,穿刺枪体积小,重量轻,便于一人控制和操作。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "equ", "entity": "自动穿刺枪" }, { "start_idx": 21, "end_idx": 23, "type": "equ", "entity": "穿刺枪" }, { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "经皮肾穿刺活检" }, { "start_idx": 36, "end_idx": 38, "type": "equ", "entity": "穿刺枪" } ] }, { "text": "穿刺部位皮肤切口小,在B超引导下进针,进针方向与肾纵轴垂直线呈10°~15°夹角,穿刺针至肾包膜后,嘱患者屏气,扣动开关,在快速的强力弹簧驱动下,活检针迅速精确地进针穿刺。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "穿刺" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "皮肤" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "B超" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "肾" }, { "start_idx": 41, "end_idx": 43, "type": "equ", "entity": "穿刺针" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "肾包膜" }, { "start_idx": 73, "end_idx": 75, "type": "equ", "entity": "活检针" }, { "start_idx": 83, "end_idx": 84, "type": "pro", "entity": "穿刺" } ] }, { "text": "穿刺枪的应用使操作简化,单手操作整个过程,取材成功率高,减少穿刺后出血等并发症,对周围组织的损伤最小。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "穿刺枪" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "穿刺" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "出血" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "组织" } ] }, { "text": "3.肾活检后的处理穿刺后常规按压穿刺部位15分钟,以纱布覆盖伤口,用多头带包紧腰部,患者仰卧用推床推床将小儿送回病房,平卧24小时。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肾活检" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "穿刺" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "穿刺" }, { "start_idx": 26, "end_idx": 27, "type": "equ", "entity": "纱布" }, { "start_idx": 35, "end_idx": 36, "type": "equ", "entity": "头带" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "腰部" }, { "start_idx": 47, "end_idx": 48, "type": "equ", "entity": "推床" } ] }, { "text": "穿刺后每小时测血压及脉搏,连续3~4次,如无异常可改为4小时测1次至24小时止。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "穿刺" }, { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "血压" }, { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "脉搏" } ] }, { "text": "肾穿刺后连续检查尿液3次,若无血尿则可停止检查监测,若有血尿则应连续监测,直到血尿消失。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "肾穿刺" }, { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "尿液" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "血尿" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "血尿" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "血尿" } ] }, { "text": "有肉眼血尿者则应给予补液,或多饮水,使尿量增加以防血块形成堵塞输尿管。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "血尿" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "补液" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "尿量" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血块" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "输尿管" } ] }, { "text": "患儿应在1周内适当限制活动,观察有无肾周血肿的出现,如腰酸、腹部胀痛,腰部出现包块或血红蛋白下降等,可疑时应做超声波检查。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "肾周血肿" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "腰酸" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "腹部胀痛" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "腰部出现包块" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "血红蛋白下降" }, { "start_idx": 55, "end_idx": 59, "type": "pro", "entity": "超声波检查" } ] }, { "text": "例如,维生素E是生物膜和脂蛋白最重要的氧自由基清除剂,抑制脂质过氧化作用,对预防动脉粥样硬化和婴幼儿视网膜病变很重要;维生素C可抑制膳食中亚硝胺的致癌作用;许多流行病学调查证明,体内β-胡萝卜素水平的增加,可减少癌症和心血管疾病的危险性。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "生物膜" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "脂蛋白" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "动脉粥样硬化" }, { "start_idx": 47, "end_idx": 54, "type": "dis", "entity": "婴幼儿视网膜病变" }, { "start_idx": 106, "end_idx": 107, "type": "dis", "entity": "癌症" }, { "start_idx": 109, "end_idx": 113, "type": "dis", "entity": "心血管疾病" } ] }, { "text": "(一)维生素A主要功能是促进生长发育、维持表皮的完整性和视觉功能、促进生殖功能和维持骨细胞的代谢平衡等,近年研究表明其还有抗肿瘤作用。", "entities": [ { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "骨细胞" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "肿瘤" } ] }, { "text": "(二)维生素D主要功能是促进钙和磷在肠道内的吸收(钙和磷的比例为1~2∶1时吸收最佳)、增加肾脏对钙的重吸收,对骨骼形成极为重要,促使骨的生长和软骨骨化,与甲状旁腺一起维持血钙正常水平,防止骨质疏松和低钙痉挛。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肠道" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "肾脏" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "骨骼" }, { "start_idx": 67, "end_idx": 67, "type": "bod", "entity": "骨" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "软骨" }, { "start_idx": 78, "end_idx": 81, "type": "bod", "entity": "甲状旁腺" }, { "start_idx": 86, "end_idx": 87, "type": "ite", "entity": "血钙" }, { "start_idx": 95, "end_idx": 98, "type": "dis", "entity": "骨质疏松" }, { "start_idx": 100, "end_idx": 103, "type": "dis", "entity": "低钙痉挛" } ] }, { "text": "(四)维生素K维生素K是凝血酶原的主要成分,还能促使肝脏合成凝血酶原,临床上常作为止血药应用。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肝脏" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "凝血酶原" } ] }, { "text": "一部分维生素K可由人体回肠内细菌合成被吸收利用;另一部分由食物获得,主要来源于绿叶蔬菜、动物内脏、肉类和奶类。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "回肠内细菌" } ] }, { "text": "估计每天总的需要量为2μg/kg,如肠道功能不正常或长期应用抗生素者,有时需要补充一定量的维生素K来预防出血倾向。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肠道" } ] }, { "text": "主要概括为以下方面:促进胶原和神经递质的合成,类固醇化合物羟化,抗体生成,促使叶酸的活化;可防治坏血病,保护细胞膜,提高铁的吸收和利用,无论在治疗缺铁性还是巨幼红细胞性贫血胆固醇有协同作用;有抗氧化和清除自由基作用;另外,还可促进胆固醇的排出,防止动脉粥样硬化形成,并有提高机体免疫、增加白细胞的吞噬功能。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胶原" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "神经递质" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "抗体" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "细胞膜" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "胆固醇" }, { "start_idx": 124, "end_idx": 129, "type": "dis", "entity": "动脉粥样硬化" }, { "start_idx": 144, "end_idx": 146, "type": "bod", "entity": "白细胞" } ] }, { "text": "(二)维生素B1维生素B1又称硫胺素,是构成脱羧酶的辅酶,参与丙酮酸等的氧化脱羧反应,如缺乏可使丙酮酸在神经组织和末梢血管沉积而致脚气病。", "entities": [ { "start_idx": 74, "end_idx": 77, "type": "bod", "entity": "神经组织" }, { "start_idx": 79, "end_idx": 82, "type": "bod", "entity": "末梢血管" }, { "start_idx": 70, "end_idx": 84, "type": "sym", "entity": "丙酮酸在神经组织和末梢血管沉积" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "脚气病" } ] }, { "text": "(三)维生素B2维生素B2又称核黄素,其主要功能是构成核黄素辅酶参与体内多种物质的氧化还原反应,是一种重要的营养素,如缺乏将影响物质和能量代谢,会出现多种临床症状,常见的有舌炎、口角炎、口腔溃疡、脂溢性皮炎等。", "entities": [ { "start_idx": 108, "end_idx": 109, "type": "sym", "entity": "舌炎" }, { "start_idx": 111, "end_idx": 113, "type": "sym", "entity": "口角炎" }, { "start_idx": 115, "end_idx": 116, "type": "bod", "entity": "口腔" }, { "start_idx": 115, "end_idx": 118, "type": "sym", "entity": "口腔溃疡" }, { "start_idx": 120, "end_idx": 124, "type": "sym", "entity": "脂溢性皮炎" } ] }, { "text": "(四)维生素B6维生素B6又称吡哆素,在体内与磷酸结合构成多种酶的辅酶,参与三大营养素的代谢,并与血红素的合成有关,缺乏时可引起低色素性贫血。", "entities": [ { "start_idx": 86, "end_idx": 91, "type": "dis", "entity": "低色素性贫血" } ] }, { "text": "主要生理功能是提高叶酸的利用率,促进红细胞的发育和成熟;还与神经髓鞘的物质代谢密切相关。", "entities": [ { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "神经髓鞘" } ] }, { "text": "故缺乏时可导致巨细胞性贫血并出现神经系统症状。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "巨细胞性贫血" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "神经系统" } ] }, { "text": "维生素B12主要存在于动物性食物中,人体肠道内细菌也能大量合成,由于其吸收需在胃壁细胞分泌的内因子作用下在回肠部被吸收,故当胃或回肠切除后等胃肠功能减退时会发生缺乏,也可见于严格素食者和老年人中。", "entities": [ { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "肠道内细菌" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "回肠部" }, { "start_idx": 73, "end_idx": 73, "type": "bod", "entity": "胃" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "回肠" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "胃肠" } ] }, { "text": "(六)烟酸又称尼克酸或维生素PP,经小肠吸收后形成脱氢酶辅酶,在体内代谢中起着递氢的作用。", "entities": [ { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "脱氢酶辅酶" } ] }, { "text": "严重缺乏时会出现皮炎、腹泻和痴呆的癞皮病典型症状。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "皮炎" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "腹泻" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "痴呆的癞皮病" } ] }, { "text": "皮肤症状表现为肢体暴露部位的对称性皮炎,包括急性红斑和褶烂、慢性肥厚和萎缩、色素沉着等;消化系统症状包括舌炎、口角炎、恶心呕吐、慢性胃炎、便秘或腹泻等;神经系统症状可产生精神错乱、神志不清甚至痴呆等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "皮肤" }, { "start_idx": 7, "end_idx": 18, "type": "sym", "entity": "肢体暴露部位的对称性皮炎" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "急性红斑和褶烂" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "慢性肥厚和萎缩" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "色素沉着" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "消化系统" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "舌炎" }, { "start_idx": 55, "end_idx": 57, "type": "sym", "entity": "口角炎" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "恶心呕吐" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "慢性胃炎" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "便秘" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "腹泻" }, { "start_idx": 76, "end_idx": 79, "type": "bod", "entity": "神经系统" }, { "start_idx": 85, "end_idx": 88, "type": "sym", "entity": "精神错乱" }, { "start_idx": 90, "end_idx": 93, "type": "sym", "entity": "神志不清" }, { "start_idx": 96, "end_idx": 97, "type": "sym", "entity": "痴呆" } ] }, { "text": "由于抗结核药异烟肼与烟酸拮抗,故应用该药时需注意烟酸的补充。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "异烟肼" } ] }, { "text": "除了食物中很易得到外,肠内细菌也能合成,故通常不会缺乏。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "肠内细菌" } ] }, { "text": "缺乏时表现为皮炎、舌乳头萎缩、恶心呕吐和食欲减退等。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "皮炎" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "舌乳头" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "舌乳头萎缩" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "恶心呕吐" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "食欲减退" } ] }, { "text": "(九)叶酸在体内的主要生理功能是促进红细胞的生成,缺乏时红细胞的发育和成熟会受到影响,引起巨幼红细胞性贫血;还与胎儿的神经管的发育有关,孕妇叶酸缺乏时可致胎儿神经管的发育畸形。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "红细胞" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "红细胞" }, { "start_idx": 45, "end_idx": 52, "type": "dis", "entity": "巨幼红细胞性贫血" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "神经管" }, { "start_idx": 70, "end_idx": 73, "type": "dis", "entity": "叶酸缺乏" }, { "start_idx": 77, "end_idx": 86, "type": "dis", "entity": "胎儿神经管的发育畸形" } ] }, { "text": "近年研究发现叶酸缺乏可引起高同型半胱氨酸血症,被认为是心血管疾病的危险因素,可影响胚胎早期心血管的发育。", "entities": [ { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "高同型半胱氨酸血症" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "心血管疾病" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "心血管" } ] }, { "text": "第四节肺泡微石症肺泡微石症(pulmonaryalveolarmicrolithiasis)以肺泡内形成以钙为主成分、广泛存在的播散性小结石为特征。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肺泡微石症" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肺泡微石症" }, { "start_idx": 14, "end_idx": 44, "type": "dis", "entity": "pulmonaryalveolarmicrolithiasis" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "肺泡内" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "钙" } ] }, { "text": "病因不明,体内无钙、磷或其他代谢障碍。", "entities": [ { "start_idx": 5, "end_idx": 17, "type": "sym", "entity": "体内无钙、磷或其他代谢障碍" } ] }, { "text": "胸片示细砂粒、粟粒状播散钙化影,以中肺野及肺底部最明显,以后阴影于肺门处融合,并蔓延到肺尖及周边,有时肺尖部可见气肿性肺大疱。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "胸片" }, { "start_idx": 3, "end_idx": 5, "type": "sym", "entity": "细砂粒" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "粟粒状播散钙化影" }, { "start_idx": 17, "end_idx": 26, "type": "sym", "entity": "中肺野及肺底部最明显" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肺门" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "肺尖及周边" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "肺尖部" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "气肿性肺大疱" } ] }, { "text": "无特殊方法,以对症治疗及支持疗法为主,注意预防呼吸道感染。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "对症治疗" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "支持疗法" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "呼吸道感染" } ] }, { "text": "第五节风疹风疹(rubella,Germanmeasles)是由风疹病毒引起的急性出疹性传染病,以前驱期短、发热1~2天出疹及耳后、枕后和颈部淋巴结肿大为其临床特征。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "风疹" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "风疹" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "rubella" }, { "start_idx": 16, "end_idx": 28, "type": "dis", "entity": "Germanmeasles" }, { "start_idx": 32, "end_idx": 35, "type": "mic", "entity": "风疹病毒" }, { "start_idx": 39, "end_idx": 46, "type": "dis", "entity": "急性出疹性传染病" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "耳后" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "枕后" }, { "start_idx": 69, "end_idx": 73, "type": "bod", "entity": "颈部淋巴结" }, { "start_idx": 49, "end_idx": 75, "type": "sym", "entity": "前驱期短、发热1~2天出疹及耳后、枕后和颈部淋巴结肿大" } ] }, { "text": "胎儿早期感染可致严重先天畸形。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "感染" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "严重先天畸形" } ] }, { "text": "【病原和流行病学】风疹病毒(rubellaVirus)属披膜病毒科,由核衣壳和包膜构成。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "风疹病毒" }, { "start_idx": 14, "end_idx": 25, "type": "mic", "entity": "rubellaVirus" }, { "start_idx": 28, "end_idx": 31, "type": "mic", "entity": "披膜病毒" } ] }, { "text": "病毒核酸为单股正链RNA。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "病毒核酸" } ] }, { "text": "包膜含2种蛋白,E1具凝血作用,能刺激机体产生中和抗体和血凝抑制抗体,E2抗原性不如E1强,亦能诱导中和抗体。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "抗体" }, { "start_idx": 28, "end_idx": 33, "type": "bod", "entity": "血凝抑制抗体" } ] }, { "text": "此病毒较不稳定,可被脂溶剂、甲醛、紫外线、强酸和热等灭活,干燥冰冻保存9个月。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "mic", "entity": "病毒" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "脂溶剂" }, { "start_idx": 14, "end_idx": 15, "type": "dru", "entity": "甲醛" } ] }, { "text": "风疹病毒可在各种猴、兔和人原代、传代及二倍体细胞中增殖。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "风疹病毒" } ] }, { "text": "病毒存在于患者或隐性感染者鼻咽分泌物、血、粪和尿中。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "隐性感染" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "鼻咽分泌物" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "血" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "粪" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "尿" } ] }, { "text": "在出疹前7天和疹退后14天内可从鼻咽部毒。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "出疹" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "鼻咽" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "鼻咽" }, { "start_idx": 15, "end_idx": 14, "type": "mic", "entity": "" } ] }, { "text": "先天性风疹综合征患者生后排大量病毒可达数月至数年(1年者约11%)。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "先天性风疹综合征" }, { "start_idx": 15, "end_idx": 16, "type": "mic", "entity": "病毒" } ] }, { "text": "孕妇感染时,在病毒血症血症期可将病毒经胎盘传给胎儿。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "感染" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "病毒血症" }, { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "病毒" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胎盘" } ] }, { "text": "感染者排毒污染手或环境后,可经污染物—手—呼吸道或手—呼吸道途径传播病毒。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "手" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "手" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "呼吸道" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "手" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "呼吸道" }, { "start_idx": 34, "end_idx": 35, "type": "mic", "entity": "病毒" } ] }, { "text": "我国育龄妇女风疹病毒IgG抗体阳性率不同地区调查结果不同,为76%~98%,平均88%。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "bod", "entity": "风疹病毒IgG抗体" } ] }, { "text": "早期妇女特异性IgM阳性率为0.46%。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "特异性IgM" } ] }, { "text": "【发病机制和病理改变】病毒侵入上呼吸道,在黏膜和颈部、颈下和耳后淋巴结内增殖,而后入血,形成2次病毒血症。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "病毒" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "黏膜" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "颈部" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "颈下" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "耳" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "淋巴结" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "血" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "病毒血症" } ] }, { "text": "风疹病毒所致的抗原抗体复合物引起真皮上层毛细血管炎,形成皮疹。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "风疹病毒" }, { "start_idx": 16, "end_idx": 24, "type": "dis", "entity": "真皮上层毛细血管炎" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "皮疹" } ] }, { "text": "淋巴结肿大,呼吸道见轻度炎症。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "淋巴结" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "淋巴结肿大" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "呼吸道" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "呼吸道见轻度炎症" } ] }, { "text": "先天性风疹的发病机制并不十分明确。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "先天性风疹" } ] }, { "text": "风疹病毒导致血管内皮细胞受损是胎儿供血不足、组织细胞代谢失调和脏器发育不良的重要原因;病毒抑制感染细胞有丝分裂,致染色体断裂,使器官组织分化发育障碍;特异性免疫复合物和自身抗体形成可能是组织脏器损伤的另一机制。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "风疹病毒" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "血管内皮细胞" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "血管内皮细胞受损" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "血" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "胎儿供血不足" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "组织细胞" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "组织细胞代谢失调" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "脏器" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "脏器发育不良" }, { "start_idx": 43, "end_idx": 44, "type": "mic", "entity": "病毒" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "感染细胞" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "染色体" }, { "start_idx": 57, "end_idx": 61, "type": "sym", "entity": "染色体断裂" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "器官组织" }, { "start_idx": 64, "end_idx": 73, "type": "sym", "entity": "器官组织分化发育障碍" }, { "start_idx": 93, "end_idx": 98, "type": "dis", "entity": "组织脏器损伤" } ] }, { "text": "风疹病毒持续性感染可解释患儿生后出现的迟发性疾病,如生后某时期出现的听力障碍、白内障及进行性全脑炎。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "风疹病毒持续性感染" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "迟发性疾病" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "听力障碍" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "白内障" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "进行性全脑炎" } ] }, { "text": "【临床表现】(一)后天性风疹潜伏期14~21天,平均18天。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "后天性风疹" } ] }, { "text": "可有低热、不适、轻微上呼吸道炎症表现,如咳嗽、流涕、结合膜充血和咽红等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "低热" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "上呼吸道炎症" }, { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "轻微上呼吸道炎症" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "咳嗽" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "流涕" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "结合膜" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "结合膜充血" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "咽红" } ] }, { "text": "软腭上可见细小红疹,能融合成片。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "软腭" }, { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "软腭上可见细小红疹,能融合成片" } ] }, { "text": "2.出疹期通常于发热第1~2天开始出疹。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "发热" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "出疹" } ] }, { "text": "皮疹首先见于脸部,然后迅速遍及颈部、躯干和四肢。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脸部" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "颈部" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "躯干" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "四肢" }, { "start_idx": 0, "end_idx": 22, "type": "sym", "entity": "皮疹首先见于脸部,然后迅速遍及颈部、躯干和四肢" } ] }, { "text": "出疹第一天末,全身遍布浅红色斑丘疹,略大于猩红热的疹点,散在分布。", "entities": [ { "start_idx": 7, "end_idx": 31, "type": "sym", "entity": "全身遍布浅红色斑丘疹,略大于猩红热的疹点,散在分布" } ] }, { "text": "次日脸部皮疹开始消退,很少脱皮。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "脸部" }, { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "次日脸部皮疹开始消退,很少脱皮" } ] }, { "text": "另一典型表现为枕后、耳后或颈部淋巴结肿大,可在皮疹出现前发生,持续1周或更久。", "entities": [ { "start_idx": 7, "end_idx": 19, "type": "dis", "entity": "枕后、耳后或颈部淋巴结肿大" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "皮疹" } ] }, { "text": "有的患者伴轻度脾脏肿大,可在3~4周后恢复正常。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "轻度脾脏肿大" } ] }, { "text": "部分患者可无皮疹,仅有淋巴结肿大。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "皮疹" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "淋巴结" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "淋巴结肿大" } ] }, { "text": "(二)先天性风疹综合征先天感染风疹病毒后可发生死胎、流产、畸形新生儿;或在出生时正常,以后出现病损,也可为隐性感染。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "先天性风疹综合征" }, { "start_idx": 15, "end_idx": 18, "type": "mic", "entity": "风疹病毒" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "死胎" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "流产" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "畸形" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "隐性感染" } ] }, { "text": "重者出生时有低体重、肝脾肿大、血小板减少性紫癜、先天性心脏病、白内障、小头畸形、骨发育不良和脑脊液异常等。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "低体重" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肝脾" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "血小板" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "血小板减少性紫癜" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "白内障" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "小头畸形" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "骨" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "骨发育不良" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "脑脊液" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "脑脊液异常" } ] }, { "text": "迟发性疾病包括听力丧失、内分泌病、白内障或青光眼和进行性全脑炎。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "听力丧失" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "内分泌病" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "白内障" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "青光眼" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "进行性全脑炎" } ] }, { "text": "【病原学诊断】(一)病毒分离普通风疹患者取出疹前5天至出疹后6天鼻咽分泌物分离病毒的阳性率较高。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "病毒" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "风疹" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "鼻咽分泌物" }, { "start_idx": 39, "end_idx": 40, "type": "mic", "entity": "病毒" } ] }, { "text": "孕妇原发感染后,取羊水或胎盘绒毛分离病毒是诊断胎儿风疹病毒感染最可靠的方法之一。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "原发感染" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "羊水" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "胎盘绒毛" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "病毒" }, { "start_idx": 23, "end_idx": 30, "type": "dis", "entity": "胎儿风疹病毒感染" } ] }, { "text": "先天性风疹患儿常采集鼻咽分泌物、尿、脑脊液、骨髓等标本分离病毒。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "先天性风疹" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "鼻咽分泌物" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "尿" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "脑脊液" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "骨髓" }, { "start_idx": 29, "end_idx": 30, "type": "mic", "entity": "病毒" } ] }, { "text": "(二)特异性抗体检测特异性IgM在出疹时出现,疹后5~14天水平较高,持续约30天,是近期感染的指标。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "特异性抗体检测" }, { "start_idx": 10, "end_idx": 15, "type": "ite", "entity": "特异性IgM" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "出疹" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "感染" } ] }, { "text": "双份血清(间隔1~2周采血)特异性IgG≥4倍升高有诊断意义。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血清" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "采血" }, { "start_idx": 14, "end_idx": 19, "type": "ite", "entity": "特异性IgG" } ] }, { "text": "先天性风疹患儿特异性IgM在生后6个月内持续升高,1岁以内均可检测到。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "先天性风疹" }, { "start_idx": 7, "end_idx": 12, "type": "ite", "entity": "特异性IgM" } ] }, { "text": "胎血中检出特异性IgM可证实胎儿感染,但由于胎儿在20周后才能产生IgM,故妊娠头3个月胎儿的风疹病毒感染不能依靠IgM检测。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胎血" }, { "start_idx": 5, "end_idx": 10, "type": "ite", "entity": "特异性IgM" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "胎儿" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "胎儿" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "IgM" }, { "start_idx": 44, "end_idx": 52, "type": "dis", "entity": "胎儿的风疹病毒感染" }, { "start_idx": 57, "end_idx": 61, "type": "pro", "entity": "IgM检测" } ] }, { "text": "(三)病毒抗原检测采用免疫印迹法检测胎盘绒毛或胎儿活检标本中风疹病毒抗原。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "病毒抗原检测" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "免疫印迹法" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "胎盘绒毛" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "胎儿活检" }, { "start_idx": 30, "end_idx": 35, "type": "ite", "entity": "风疹病毒抗原" } ] }, { "text": "(四)病毒基因测定采用核酸杂交技术或PCR法检测羊水、绒毛膜或绒毛中病毒基因。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "病毒基因测定" }, { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "核酸杂交技术" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "PCR法" }, { "start_idx": 24, "end_idx": 37, "type": "ite", "entity": "羊水、绒毛膜或绒毛中病毒基因" } ] }, { "text": "【预防和治疗】(一)一般预防预防重点是妊娠期妇女,尤其在孕早期,无论是否患过风疹或接种过风疹疫苗,均应尽量避免与风疹患者接触,以免感染或再感染。", "entities": [ { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "风疹" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "接种" }, { "start_idx": 44, "end_idx": 47, "type": "dru", "entity": "风疹疫苗" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "风疹" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "感染" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "感染" } ] }, { "text": "(二)主动免疫风疹减毒活疫苗已广泛应用,接种者95%产生抗体。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dru", "entity": "风疹减毒活疫苗" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "抗体" } ] }, { "text": "不良反应主要有小关节疼痛、一过性发热或皮疹。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "小关节" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "小关节疼痛" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "一过性发热" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "皮疹" } ] }, { "text": "尚无疫苗致畸的证据。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dru", "entity": "疫苗" }, { "start_idx": 5, "end_idx": 5, "type": "dis", "entity": "畸" } ] }, { "text": "(三)被动免疫妊娠早期孕妇于接触风疹患者3天内肌内注射高效价免疫球蛋白20ml,可起到预防作用。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "风疹" }, { "start_idx": 23, "end_idx": 34, "type": "pro", "entity": "肌内注射高效价免疫球蛋白" } ] }, { "text": "(四)治疗主要为对症治疗,宜卧床休息,给予富营养又易消化的食物。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "卧床休息" }, { "start_idx": 19, "end_idx": 30, "type": "pro", "entity": "给予富营养又易消化的食物" } ] }, { "text": "可给清热解毒类中药。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "pro", "entity": "给清热解毒类中药" } ] }, { "text": "对先天性风疹综合征患者的各种缺陷,应作相应处理。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "先天性风疹综合征" } ] }, { "text": "二、幼年型慢性粒细胞白血病【临床特征】幼年型慢性粒细胞白血病(juvenilechronicmyelogenousleukemia,JCML)在FAB分类中划入骨髓增生异常综合征(MDS),与CML一样、JCML累及多能造血干细胞,外周血白细胞数增高、脾大、碱性磷酸酶活力减低CML来说尚有以下特征:外周血单核细胞比例>10%,幼稚细胞比例<5%;骨髓未成熟单核细胞增高,幼稚细胞比例<30%;Hb-F升高;高丙种球蛋白血症。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "幼年型慢性粒细胞白血病" }, { "start_idx": 19, "end_idx": 29, "type": "dis", "entity": "幼年型慢性粒细胞白血病" }, { "start_idx": 31, "end_idx": 64, "type": "dis", "entity": "juvenilechronicmyelogenousleukemia" }, { "start_idx": 66, "end_idx": 69, "type": "dis", "entity": "JCML" }, { "start_idx": 80, "end_idx": 88, "type": "dis", "entity": "骨髓增生异常综合征" }, { "start_idx": 90, "end_idx": 92, "type": "dis", "entity": "MDS" }, { "start_idx": 96, "end_idx": 98, "type": "dis", "entity": "CML" }, { "start_idx": 102, "end_idx": 105, "type": "dis", "entity": "JCML" }, { "start_idx": 108, "end_idx": 114, "type": "bod", "entity": "多能造血干细胞" }, { "start_idx": 119, "end_idx": 121, "type": "bod", "entity": "白细胞" }, { "start_idx": 126, "end_idx": 126, "type": "bod", "entity": "脾" }, { "start_idx": 129, "end_idx": 133, "type": "bod", "entity": "碱性磷酸酶" }, { "start_idx": 116, "end_idx": 137, "type": "sym", "entity": "外周血白细胞数增高、脾大、碱性磷酸酶活力减低" }, { "start_idx": 138, "end_idx": 140, "type": "dis", "entity": "CML" }, { "start_idx": 150, "end_idx": 156, "type": "ite", "entity": "外周血单核细胞" }, { "start_idx": 164, "end_idx": 167, "type": "ite", "entity": "幼稚细胞" }, { "start_idx": 174, "end_idx": 184, "type": "sym", "entity": "骨髓未成熟单核细胞增高" }, { "start_idx": 186, "end_idx": 189, "type": "ite", "entity": "幼稚细胞" }, { "start_idx": 197, "end_idx": 202, "type": "sym", "entity": "Hb-F升高" }, { "start_idx": 204, "end_idx": 211, "type": "dis", "entity": "高丙种球蛋白血症" } ] }, { "text": "MDS伴有7号染色体单体时与JCML临床表现相似,但Hb-F不高。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "MDS" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "染色体单体" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "JCML" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "Hb-F不高" } ] }, { "text": "JCML与CML的鉴别见表11-11。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "JCML" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "CML" } ] }, { "text": "表11-11JCML与成人型CML的鉴别【治疗与疗效】化疗疗效有限,可单独用6-巯基鸟嘌呤或与阿糖胞苷联合应用。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "JCML" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "成人型CML" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "化疗" }, { "start_idx": 38, "end_idx": 44, "type": "dru", "entity": "6-巯基鸟嘌呤" }, { "start_idx": 47, "end_idx": 50, "type": "dru", "entity": "阿糖胞苷" } ] }, { "text": "在多数病例中单纯的对症及支持治疗与采用化疗者生存期相似。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "化疗" } ] }, { "text": "联合强化疗在少数病人中取得达2年的缓解期。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "化疗" } ] }, { "text": "移植后复发仍是个棘手的问题。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "移植" } ] }, { "text": "第十节原发性膀胱输尿管反流正常的输尿管膀胱连接部具有活瓣样功能,其只允许尿液从输尿管流进膀胱,而不允许尿液从膀胱向输尿管的倒流。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "原发性膀胱输尿管反流" }, { "start_idx": 16, "end_idx": 23, "type": "bod", "entity": "输尿管膀胱连接部" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "尿液" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "输尿管" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "膀胱" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "尿液" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "膀胱" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "输尿管" } ] }, { "text": "输尿管膀胱连接部抗反流机制在公元150年已被认识到,1883年Pozzi在动物实验和人体中观察到膀胱输尿管反流,但尚不能确定是否为异常现象。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "输尿管膀胱连接部" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "膀胱输尿管反流" } ] }, { "text": "其后Bumpus和Gruber等发现反流与泌尿系感染之间的关系,但当时更多的认为膀胱输尿管反流是继发于膀胱颈部梗阻,故1950年前后,人们施行了许多不必要的手术如膀胱颈部成形去解除并不存在的膀胱颈梗阻。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "泌尿系感染" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "膀胱颈部梗阻" }, { "start_idx": 81, "end_idx": 84, "type": "bod", "entity": "膀胱颈部" }, { "start_idx": 95, "end_idx": 99, "type": "dis", "entity": "膀胱颈梗阻" } ] }, { "text": "从20世纪60年代起,出现了大量关于膀胱输尿管反流与尿路感染和肾瘢痕之间关系的研究,并逐步认识到膀胱输尿管反流可导致高血压和肾衰竭。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "尿路感染" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "高血压" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "【发病率】原发性膀胱输尿管反流在正常婴儿和儿童的发生率很难知道,统计的发生率为1%~18.5%,但70%发生在尿路感染的患儿中。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "dis", "entity": "原发性膀胱输尿管反流" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "尿路感染" } ] }, { "text": "反流有一定的性别倾向,女孩占85%,男孩如有尿路感染其反流可能性大,包皮环切对其发生也有影响,环切者,反流率低。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "尿路感染" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "包皮环切" } ] }, { "text": "年龄与反流发生率成反比,4岁20%,12岁15%,成人5.2%,由此也提示随着膀胱及输尿管的发育(输尿管隧道延长),反流可自然缓解。", "entities": [ { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "膀胱" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "输尿管" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "输尿管" } ] }, { "text": "【病因和机制】1.输尿管膀胱连接部正常解剖和抗反流机制输尿管全部的肌层几乎都是由松散的、不规则的螺旋形肌纤维构成,只有膀胱壁段输尿管的肌纤维才是纵行。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "bod", "entity": "输尿管膀胱连接部" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "输尿管" }, { "start_idx": 59, "end_idx": 65, "type": "bod", "entity": "膀胱壁段输尿管" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "肌纤维" } ] }, { "text": "进入膀胱后肌纤维成扇形构成三角区肌肉的浅层,并向前延伸达后尿道精阜部。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "膀胱" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肌纤维" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "尿道精阜部" } ] }, { "text": "输尿管穿入膀胱壁时由一纤维鞘(Waldeyer)包绕,位于膀胱黏膜下,使其能适应膀胱的充盈和空虚状态。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "输尿管" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "膀胱壁" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "纤维鞘" }, { "start_idx": 15, "end_idx": 22, "type": "bod", "entity": "Waldeyer" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "膀胱黏膜" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "膀胱" } ] }, { "text": "输尿管膀胱连接部的活瓣作用取决于膀胱内黏膜下段输尿管的长度和三角区肌层保持这个长度的能力,以及膀胱逼尿肌对该段输尿管后壁足够的支撑作用。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "输尿管膀胱连接部" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "活瓣" }, { "start_idx": 16, "end_idx": 25, "type": "bod", "entity": "膀胱内黏膜下段输尿管" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "膀胱逼尿肌" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "输尿管后壁" } ] }, { "text": "当膀胱内压上升时,黏膜下段输尿管被压缩而不产生反流,这种活瓣机制是被动的。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "膀胱" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "输尿管" } ] }, { "text": "但输尿管的蠕动能力和输尿管口的关闭能力在防止反流中也起一部分作用。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "输尿管" } ] }, { "text": "2.反流原因输尿管膀胱连接部的先天性异常,主要是输尿管膀胱壁内段的纵行肌肉发育不良,致使输尿管口外移,黏膜下段输尿管缩短,从而失去抗反流的能力。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "bod", "entity": "输尿管膀胱连接部" }, { "start_idx": 24, "end_idx": 70, "type": "sym", "entity": "输尿管膀胱壁内段的纵行肌肉发育不良,致使输尿管口外移,黏膜下段输尿管缩短,从而失去抗反流的能力" } ] }, { "text": "另一原因是黏膜下段输尿管的长度与其口径不相称。", "entities": [ { "start_idx": 5, "end_idx": 21, "type": "sym", "entity": "黏膜下段输尿管的长度与其口径不相称" } ] }, { "text": "正常无反流时,黏膜下段输尿管的长度与其直径的比例为5∶1,而有反流者仅为1.4∶1。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "黏膜下段输尿管" } ] }, { "text": "【反流分级】一般根据排尿性膀胱尿道造影(VCUG)的结果,将原发性膀胱输尿管反流分为反流仅达输尿管-12):Ⅰ级反流仅达输尿管;Ⅱ级反流至肾盂肾盏,但输尿管无扩张;Ⅲ级输尿管轻度扩张或(和)弯曲,肾盂轻度扩张和穹隆轻度变钝;轻度扩张或(和)弯曲,肾盂轻度扩张和穹隆轻度变钝;Ⅳ级输尿管中度扩张和弯曲,肾盂肾盏中度扩张,但多数肾盏仍维持乳头形态;Ⅴ级输尿管严重扩张和迂曲,肾盂肾盏严重扩张,多数肾盏中乳头形态消失。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "pro", "entity": "排尿性膀胱尿道造影" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "VCUG" }, { "start_idx": 30, "end_idx": 39, "type": "dis", "entity": "原发性膀胱输尿管反流" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "反流仅达输尿管" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "输尿管" }, { "start_idx": 66, "end_idx": 80, "type": "sym", "entity": "反流至肾盂肾盏,但输尿管无扩张" }, { "start_idx": 84, "end_idx": 111, "type": "sym", "entity": "输尿管轻度扩张或(和)弯曲,肾盂轻度扩张和穹隆轻度变钝;" }, { "start_idx": 112, "end_idx": 135, "type": "sym", "entity": "轻度扩张或(和)弯曲,肾盂轻度扩张和穹隆轻度变钝" }, { "start_idx": 139, "end_idx": 170, "type": "sym", "entity": "输尿管中度扩张和弯曲,肾盂肾盏中度扩张,但多数肾盏仍维持乳头形态" }, { "start_idx": 174, "end_idx": 204, "type": "sym", "entity": "输尿管严重扩张和迂曲,肾盂肾盏严重扩张,多数肾盏中乳头形态消失" } ] }, { "text": "【反流演进】反流使部分尿液在膀胱排空后仍停留在输尿管中,从而为细菌从膀胱上行到肾脏提供了通路,因此反流常并发尿路感染,可表现为急性肾盂肾炎或无症状的慢性肾盂肾炎过程,80%的反流肾其组织学改变与肾盂肾炎一致。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "尿液" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "膀胱" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "输尿管" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "细菌" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "膀胱" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肾脏" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "尿路感染" }, { "start_idx": 63, "end_idx": 68, "type": "dis", "entity": "急性肾盂肾炎" }, { "start_idx": 74, "end_idx": 79, "type": "dis", "entity": "慢性肾盂肾炎" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "反流肾" }, { "start_idx": 97, "end_idx": 100, "type": "dis", "entity": "肾盂肾炎" } ] }, { "text": "肾瘢痕的产生与反流的严重程度相关,反流越严重,发生瘢痕进展或新瘢痕的机会越高。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肾瘢痕" } ] }, { "text": "新生儿及婴儿的集合管相对粗大,易于发生肾内反流,故患重度反流的小婴儿更易产生肾瘢痕。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "集合管" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "肾瘢痕" } ] }, { "text": "图12-7膀胱输尿管反流分级反流对肾功能的影响与尿路不完全性梗阻对肾脏的影响是一样的。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "膀胱输尿管" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "尿路不完全性梗阻" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肾脏" } ] }, { "text": "反流时上尿路内压增加,远端肾单位首受其害,因此肾小管功能受损早于肾小球。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "反流时上尿路内压增加,远端肾单位首受其害" }, { "start_idx": 23, "end_idx": 34, "type": "sym", "entity": "肾小管功能受损早于肾小球" } ] }, { "text": "无菌反流影响肾小管的浓缩能力,且持续时间较长。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肾小管" } ] }, { "text": "肾小球功能在有肾实质损害时受影响,并与肾实质损害的程度成正比。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肾小球" } ] }, { "text": "反流可以影响肾脏的发育,如抑制其胚胎发生,导致肾发育不全或肾发育异常或肾发育异常;而长期反流的患儿发生肾脏不生长等情况。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "影响肾脏的发育" }, { "start_idx": 13, "end_idx": 33, "type": "sym", "entity": "抑制其胚胎发生,导致肾发育不全或肾发育异常" }, { "start_idx": 41, "end_idx": 58, "type": "sym", "entity": "而长期反流的患儿发生肾脏不生长等情况" } ] }, { "text": "反流病人发生高血压的机会较高。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "反流病人发生高血压的机会较高" } ] }, { "text": "如反流未能有效控制,肾瘢痕进行性发展可导致肾衰竭。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "原发性膀胱输尿管反流一般随年龄增长逐渐好转,可能是因输尿管膀胱壁内段和膀胱三角区肌肉的生长和成熟之故。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "原发性膀胱输尿管反流" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "输尿管膀胱壁内段" }, { "start_idx": 35, "end_idx": 41, "type": "bod", "entity": "膀胱三角区肌肉" } ] }, { "text": "如静脉尿路造影显示输尿管口径正常,原发反流85%可自然消失。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "静脉尿路造影" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "输尿管" } ] }, { "text": "反流的自然消失与反流累及的范围也有关系,单侧反流自然消失率可达65%;双侧反流,输尿管无扩张者,自然消失率是50%,有输尿管扩张的仅10%左右。", "entities": [ { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "输尿管" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "输尿管" } ] }, { "text": "感染及肾瘢痕并不直接影响反流的消失,但肾瘢痕多见于严重反流的病例,反流自行消失机会少。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "肾瘢痕" } ] }, { "text": "原发性膀胱输尿管反流在青年和成人中的发展趋势尚有争论。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "原发性膀胱输尿管反流" } ] }, { "text": "【临床表现】原发性膀胱输尿管反流的症状主要从两方面表现,肾积水和尿路感染。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "原发性膀胱输尿管反流" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肾积水" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "尿路感染" } ] }, { "text": "反流导致上尿路内的尿液无法排空,到一定程度即会产生肾盂和输尿管的扩张尿路内的尿液无法排空,到一定程度即会产生肾盂和输尿管的扩张,在超声上反映出来。", "entities": [ { "start_idx": 0, "end_idx": 33, "type": "sym", "entity": "反流导致上尿路内的尿液无法排空,到一定程度即会产生肾盂和输尿管的扩张" } ] }, { "text": "尿路感染在儿童中更多的表现是非特异性的,包括发热、嗜睡无力只允许尿液从输尿管流进膀胱,而不允许尿液从膀胱向输尿管的倒流。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尿路感染" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "发热" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "嗜睡" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "无力" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "输尿管" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "膀胱" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "膀胱" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "输尿管" } ] }, { "text": "输尿管膀胱连接部抗反流机制在公元150年已被认识到,1883年Pozzi在动物实验和人体中观察到膀胱输尿管反流,但尚不能确定是否为异常现象。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "输尿管膀胱连接部" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "膀胱输尿管反流" } ] }, { "text": "其后Bumpus和Gruber等发现反流与泌尿系感染之间的关系,但当时更多的认为膀胱输尿管反流是继发于膀胱颈部梗阻,故1950年前后,人们施行了许多不必要的手术如膀胱颈部成形去解除并不存在的膀胱颈梗阻。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "泌尿系感染" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "膀胱颈部梗阻" }, { "start_idx": 81, "end_idx": 89, "type": "pro", "entity": "膀胱颈部成形去解除" }, { "start_idx": 95, "end_idx": 99, "type": "dis", "entity": "膀胱颈梗阻" } ] }, { "text": "从20世纪60年代起,出现了大量关于膀胱输尿管反流与尿路感染和肾瘢痕之间关系的研究,并逐步认识到膀胱输尿管反流可导致高血压和肾衰竭。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "尿路感染" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "高血压" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "【发病率】原发性膀胱输尿管反流在正常婴儿和儿童的发生率很难知道,统计的发生率为1%~18.5%,但70%发生在尿路感染的患儿中。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "dis", "entity": "原发性膀胱输尿管反流" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "尿路感染" } ] }, { "text": "反流有一定的性别倾向,女孩占85%,男孩如有尿路感染其反流可能性大,包皮环切对其发生也有影响,环切者,反流率低。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "尿路感染" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "包皮环切" } ] }, { "text": "年龄与反流发生率成反比,4岁20%,12岁15%,成人5.2%,由此也提示随着膀胱及输尿管的发育(输尿管隧道延长),反流可自然缓解。", "entities": [ { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "膀胱" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "输尿管" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "输尿管" } ] }, { "text": "图12-7膀胱输尿管反流分级反流对肾功能的影响与尿路不完全性梗阻对肾脏的影响是一样的。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肾脏" } ] }, { "text": "反流时上尿路内压增加,远端肾单位首受其害,因此肾小管功能受损早于肾小球。", "entities": [ { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "肾小球" } ] }, { "text": "无菌反流影响肾小管的浓缩能力,且持续时间较长。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肾小管" } ] }, { "text": "肾小球功能在有肾实质损害时受影响,并与肾实质损害的程度成正比。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肾小球" } ] }, { "text": "反流可以影响肾脏的发育,如抑制其胚胎发生,导致肾发育不全或肾发育异常;而长期反流的患儿发生肾脏不生长等情况。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肾脏" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "肾发育不全" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "肾发育异常" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "肾脏" } ] }, { "text": "反流病人发生高血压的机会较高。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "高血压" } ] }, { "text": "如反流未能有效控制,肾瘢痕进行性发展可导致肾衰竭。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾瘢痕" } ] }, { "text": "原发性膀胱输尿管反流一般随年龄增长逐渐好转,可能是因输尿管膀胱壁内段和膀胱三角区肌肉的生长和成熟之故。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "原发性膀胱输尿管反流" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "输尿管膀胱壁内段" }, { "start_idx": 35, "end_idx": 41, "type": "bod", "entity": "膀胱三角区肌肉" } ] }, { "text": "如静脉尿路造影显示输尿管口径正常,原发反流85%可自然消失。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "静脉尿路造影" } ] }, { "text": "反流的自然消失与反流累及的范围也有关系,单侧反流自然消失率可达65%;双侧反流,输尿管无扩张者,自然消失率是50%,有输尿管扩张的仅10%左右。", "entities": [ { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "输尿管" } ] }, { "text": "感染及肾瘢痕并不直接影响反流的消失,但肾瘢痕多见于严重反流的病例,反流自行消失机会少。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "肾瘢痕" } ] }, { "text": "原发性膀胱输尿管反流在青年和成人中的发展趋势尚有争论。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "原发性膀胱输尿管反流" } ] }, { "text": "【临床表现】原发性膀胱输尿管反流的症状主要从两方面表现,肾积水和尿路感染。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "原发性膀胱输尿管反流" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肾积水" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "尿路感染" } ] }, { "text": "反流导致上尿路内的尿液无法排空,到一定程度即会产生肾盂和输尿管的扩张,在超声上反映出来。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肾盂" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "输尿管" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "超声" } ] }, { "text": "在婴幼儿无菌反流可表现为肾绞痛,但表现不典型。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "肾绞痛" } ] }, { "text": "大儿童可指出在膀胱充盈或排尿时肋部疼痛年长儿在并发急性肾盂肾炎时也有腰腹部疼痛和触痛。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "膀胱" }, { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "大儿童可指出在膀胱充盈或排尿时肋部疼痛" }, { "start_idx": 19, "end_idx": 41, "type": "sym", "entity": "年长儿在并发急性肾盂肾炎时也有腰腹部疼痛和触痛" } ] }, { "text": "【诊断】排肾同位素尿道DMSA(VCUG)是确定膀胱输尿管反流诊断和分级的金标准瘢痕凡超声检查发现肾小球水和肾小管泌尿系感染发作的均应进行VCUG。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "肾同位素" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "DMSA" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "VCUG" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "瘢痕" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "肾小球" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "肾小管" } ] }, { "text": "由于小儿对检查的恐惧和不合作原发性膀胱输尿管反流结果,造影时可予镇静剂,适当时要重复进行。", "entities": [ { "start_idx": 14, "end_idx": 23, "type": "dis", "entity": "原发性膀胱输尿管反流" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "镇静剂" } ] }, { "text": "放射性核素膀胱造影,能准确确定有无反流,但对确定反流控制感染,保护肾功能,防止并发症究。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "放射性核素膀胱造影" }, { "start_idx": 26, "end_idx": 41, "type": "pro", "entity": "控制感染,保护肾功能,防止并发症" } ] }, { "text": "静脉尿路造影可很好地显示肾脏形态,通过所显示的肾轮廓,可计算肾实质的厚度和肾的生长情况,但后一方面,超声更加简单易行。", "entities": [ { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "超声" } ] }, { "text": "肾同位素(DMSA)扫描可清晰显示肾瘢痕情况,用于随访患儿有无新瘢痕形成,并可评价肾小球和肾小管的功能,确定分肾功能,比较手术前后的肾功能等。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "pro", "entity": "肾同位素(DMSA)扫描" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "肾小球" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "肾小管" } ] }, { "text": "【治疗】由于原发性膀胱输尿管反流在许多小儿随生长发育可自然消失,而且无菌尿的反流不引起肾损害,原发性膀胱输尿管反流的治疗原则是控制尿液染,保护肾功能,防止并发症。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "原发性膀胱输尿管反流" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "尿液" } ] }, { "text": "即使是严重的反流,如患儿年龄小,肾功能好,无生长发育障碍预防性抗生素虑药物治疗。", "entities": [ { "start_idx": 28, "end_idx": 33, "type": "dru", "entity": "预防性抗生素" } ] }, { "text": "(一)药物治疗所选择的药物应当是抗菌谱广、易服用、价廉、对患儿毒身高小、体重内血压高及对体内正常尿常规影响血红蛋白的抗白细胞计数应以其最小剂量肾功能测定制感染为宜。", "entities": [ { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "身高" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "体重" }, { "start_idx": 39, "end_idx": 40, "type": "ite", "entity": "血压" }, { "start_idx": 48, "end_idx": 50, "type": "pro", "entity": "尿常规" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "血红蛋白" }, { "start_idx": 59, "end_idx": 63, "type": "pro", "entity": "白细胞计数" }, { "start_idx": 71, "end_idx": 75, "type": "pro", "entity": "肾功能测定" } ] }, { "text": "感染发作时使用治疗量,感染被控制尿液用预防量,预防量应为治疗量的1/2~1/3,这样很少引起不良反应。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "尿液" } ] }, { "text": "预超声前服用,是肾脏夜间尿液在肾盂内输尿管间最长,更易排尿性膀胱尿道造影间一直持续到反流消失为止。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "超声" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肾脏" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肾盂" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "输尿管" }, { "start_idx": 27, "end_idx": 35, "type": "pro", "entity": "排尿性膀胱尿道造影" } ] }, { "text": "反流程度明显减轻的大龄儿是否应继续服用预防性抗生素,目前放射性同位素膀胱造影治疗期间,患儿应定期随访手术治疗个月做一次体格检查,记录身高、体重及血压。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "抗生素" }, { "start_idx": 28, "end_idx": 37, "type": "pro", "entity": "放射性同位素膀胱造影" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "手术治疗" }, { "start_idx": 66, "end_idx": 67, "type": "ite", "entity": "身高" }, { "start_idx": 69, "end_idx": 70, "type": "ite", "entity": "体重" }, { "start_idx": 72, "end_idx": 73, "type": "ite", "entity": "血压" } ] }, { "text": "实验室检查包括尿常规、血红蛋白及白细胞计数等,每年做一次肾功进行性肾瘢痕扩展查也要根据患儿的病情随时调整。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "尿常规" }, { "start_idx": 11, "end_idx": 14, "type": "ite", "entity": "血红蛋白" }, { "start_idx": 16, "end_idx": 20, "type": "ite", "entity": "白细胞计数" }, { "start_idx": 30, "end_idx": 37, "type": "dis", "entity": "进行性肾瘢痕扩展" } ] }, { "text": "为膀胱输尿管反流和梗阻并存、异位输尿管开口、或伴有较大的输尿管旁憩室、或输尿管开口于膀胱憩室内肾脏的发育手术肾盂抗反流的输尿管膀胱再吻合术膀胱尿道输尿管膀胱再植术个月重复检查,以后每经膀胱外12经膀胱内一膀胱内外联合操作查也可改用放射性同位素膀胱造影。", "entities": [ { "start_idx": 1, "end_idx": 46, "type": "sym", "entity": "膀胱输尿管反流和梗阻并存、异位输尿管开口、或伴有较大的输尿管旁憩室、或输尿管开口于膀胱憩室内" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "手术" }, { "start_idx": 56, "end_idx": 68, "type": "pro", "entity": "抗反流的输尿管膀胱再吻合术" }, { "start_idx": 73, "end_idx": 80, "type": "pro", "entity": "输尿管膀胱再植术" }, { "start_idx": 91, "end_idx": 94, "type": "pro", "entity": "经膀胱外" }, { "start_idx": 97, "end_idx": 100, "type": "pro", "entity": "经膀胱内" }, { "start_idx": 102, "end_idx": 109, "type": "pro", "entity": "膀胱内外联合操作" } ] }, { "text": "Cohen输尿管膀胱再吻合术疗Politano-Leadbetter输尿管膀胱再吻合术不是Glenn-Anderson输尿管膀胱再吻合术或不能防止感染复发;有进行性肾瘢痕扩展或新瘢痕形成时要膀胱镜术治疗。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "pro", "entity": "Cohen输尿管膀胱再吻合术" }, { "start_idx": 15, "end_idx": 42, "type": "pro", "entity": "Politano-Leadbetter输尿管膀胱再吻合术" }, { "start_idx": 45, "end_idx": 67, "type": "pro", "entity": "Glenn-Anderson输尿管膀胱再吻合术" }, { "start_idx": 95, "end_idx": 97, "type": "equ", "entity": "膀胱镜" } ] }, { "text": "膀胱输尿管反流和梗阻并存Teflon管胶原蛋白或伴有较大的输尿管旁憩室、或输尿管开口于膀胱憩室内时首先考虑手术。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dru", "entity": "Teflon" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "胶原蛋白" }, { "start_idx": 24, "end_idx": 47, "type": "sym", "entity": "伴有较大的输尿管旁憩室、或输尿管开口于膀胱憩室内" } ] }, { "text": "抗反流的输尿管膀胱再吻合术(或称输尿管膀胱再Teflon多种,术式分为经膀胱外肺经脑胱内和膀胱内外联合操作三大类。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "pro", "entity": "抗反流的输尿管膀胱再吻合术" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "输尿管膀胱" }, { "start_idx": 22, "end_idx": 27, "type": "dru", "entity": "Teflon" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "膀胱" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "肺" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "脑" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "膀胱" } ] }, { "text": "目前较肉芽肿的术式有,Cohen输尿管膀胱再吻合术、Politano-LeDefluxer输尿管葡聚糖颗粒合术及Gle高分子透明质酸钠on输尿管膀胱再吻合术等。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "肉芽肿" }, { "start_idx": 11, "end_idx": 24, "type": "dis", "entity": "Cohen输尿管膀胱再吻合术" }, { "start_idx": 37, "end_idx": 42, "type": "dru", "entity": "Deflux" }, { "start_idx": 48, "end_idx": 52, "type": "dru", "entity": "葡聚糖颗粒" }, { "start_idx": 59, "end_idx": 66, "type": "dru", "entity": "高分子透明质酸钠" } ] }, { "text": "Teflon微粒由于可渗入血流,引起肺、脑等关键脏器的栓塞,或在注射局部形成肉芽肿,至今尚未获得美国食品和药品监督局(FDA)的批准。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dru", "entity": "Teflon微粒" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肺" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "脑" } ] }, { "text": "Deflux,一种葡聚糖颗粒和1%的高分子透明质酸钠各半混合而成的悬液,作为注射材料已有产品供应。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "Deflux" }, { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "葡聚糖颗粒" }, { "start_idx": 18, "end_idx": 25, "type": "dru", "entity": "高分子透明质酸钠" } ] }, { "text": "第二节房间隔缺损房间隔缺损(atrialseptaldefect,ASD)是指心房间隔任何部位出现缺损造成心房水平的交通。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 14, "end_idx": 31, "type": "dis", "entity": "atrialseptaldefect" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "ASD" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "心房间隔" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "心房" } ] }, { "text": "【病理解剖】在胚胎发育达4mm时,原始心房内相继长出第一及第二房间隔,经与中心心内膜垫会合后,将单腔的原始心房一分为二。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心房" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "第一及第二房间隔" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "中心心内膜垫" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "原始心房" } ] }, { "text": "在房间隔发育的同时,静脉窦也不断发育和移位,静脉窦移至右心房并扩大成为右心房的主要部分,使上腔静脉、下腔静脉、冠状静脉窦分别开口于右心房内,构成右心房的静脉窦部,而原始的右心房侧发育成为右心耳及右心房外侧壁,构成右心房的体部。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "房间隔" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "静脉窦" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "静脉窦" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "右心房" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "右心房" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "下腔静脉" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "冠状静脉窦" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "右心房" }, { "start_idx": 72, "end_idx": 79, "type": "bod", "entity": "右心房的静脉窦部" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "右心房" }, { "start_idx": 93, "end_idx": 95, "type": "bod", "entity": "右心耳" }, { "start_idx": 97, "end_idx": 102, "type": "bod", "entity": "右心房外侧壁" }, { "start_idx": 106, "end_idx": 108, "type": "bod", "entity": "右心房" } ] }, { "text": "心房形成及分隔过程出现异常,就可出现相应的畸形,根据胚胎发生,将房间隔缺损房间隔缺损分为四个类型:(一)原发孔型房间隔缺损房室瓣未被累及,少见。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心房" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 52, "end_idx": 60, "type": "dis", "entity": "原发孔型房间隔缺损" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "房室瓣" } ] }, { "text": "缺损位于冠状静脉窦开口的前方,缺损的下缘即为左右房室环的接合部,前方接近主动脉壁,后缘接近房室结。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "bod", "entity": "冠状静脉窦开口" }, { "start_idx": 22, "end_idx": 30, "type": "bod", "entity": "左右房室环的接合部" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "主动脉壁" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "房室结" } ] }, { "text": "(二)继发孔型房间隔缺损(中央型)占总数约70%,可以呈单孔,少数为多发型,也有筛孔状者。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "继发孔型房间隔缺损" } ] }, { "text": "(三)静脉窦型房间隔缺损占4%,其上方为上腔静脉开口,下缘为房间隔,卵圆窝和冠状静脉窦口均存在。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "静脉窦型房间隔缺损" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "房间隔" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "卵圆窝" }, { "start_idx": 38, "end_idx": 43, "type": "bod", "entity": "冠状静脉窦口" } ] }, { "text": "几乎均伴有右上肺静脉异位引流。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "右上肺静脉异位引流" } ] }, { "text": "可分为三种亚型:①上腔静脉窦型房间隔缺损:位于上腔静脉入口处,多数伴有1支或数支右上肺静脉或右肺上、中叶静脉向上移位,进入上腔静脉根部;②下腔静脉窦型房间隔缺损:此型罕见。", "entities": [ { "start_idx": 9, "end_idx": 19, "type": "dis", "entity": "上腔静脉窦型房间隔缺损" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "上肺静脉" }, { "start_idx": 46, "end_idx": 53, "type": "bod", "entity": "右肺上、中叶静脉" }, { "start_idx": 61, "end_idx": 66, "type": "bod", "entity": "上腔静脉根部" }, { "start_idx": 69, "end_idx": 79, "type": "dis", "entity": "下腔静脉窦型房间隔缺损" } ] }, { "text": "在卵圆窝后下方腔静脉入口处出现裂隙状小缺损,Kirklin等称之为后房间隔缺损,常伴有右下肺静脉1支或数支向下移位进入下腔静脉中。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "卵圆窝" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "腔静脉" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "裂隙状小缺损" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "后房间隔缺损" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "右下肺静脉" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "下腔静脉" } ] }, { "text": "因右下肺静脉造影时右心下缘呈弯刀状放射影,也称为弯刀综合征(scimitarsyndrome)。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "pro", "entity": "右下肺静脉造影" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "右心下缘" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "弯刀状放射影" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "弯刀综合征" }, { "start_idx": 30, "end_idx": 45, "type": "dis", "entity": "scimitarsyndrome" } ] }, { "text": "位于正常冠状窦口处,缺损后缘为心房壁。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "冠状窦口" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "心房壁" } ] }, { "text": "有两种亚型:冠状静脉窦顶盖部分或全部缺如(unroofedcoronarysinus),伴残存左上腔静脉入冠状静脉窦或左房者占90%;异位肺静脉入冠状静脉窦(三房心的一种),不伴左上腔静脉。", "entities": [ { "start_idx": 6, "end_idx": 19, "type": "dis", "entity": "冠状静脉窦顶盖部分或全部缺如" }, { "start_idx": 21, "end_idx": 41, "type": "dis", "entity": "unroofedcoronarysinus" }, { "start_idx": 45, "end_idx": 57, "type": "dis", "entity": "残存左上腔静脉入冠状静脉窦" }, { "start_idx": 67, "end_idx": 77, "type": "dis", "entity": "异位肺静脉入冠状静脉窦" }, { "start_idx": 89, "end_idx": 93, "type": "dis", "entity": "左上腔静脉" } ] }, { "text": "(四)单心房此型多并发其他复杂性先天性心脏病。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "单心房" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "复杂性先天性心脏病" } ] }, { "text": "【病理生理】除非缺损较小,通常通过房间隔缺损分流方向及分流量取决于两个下游心室的相对顺应性,与房间隔缺损的大小无关。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "缺损" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "下游心室" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "通常右心室顺应性较左心室佳,因此,多数情况下为左向右分流。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "右心室" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "左心室" } ] }, { "text": "在婴儿期,由于右心室肥厚、顺应性不佳,心房水平的左向右分流少。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "右心室肥厚" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心房" } ] }, { "text": "在出生后第一周,随着肺血管阻力下降,右心室顺应性改善,左向右分流增加。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺血管" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "右心室" } ] }, { "text": "绝大多数的单纯房间隔缺损婴儿无临床症状,亦有出现心功能衰竭的报道,但此类患儿心导管检查除心房水平左向右分流外,多无其他异常发现,心力衰竭的发病机制尚不明了,且易伴发心外畸形、生长发育迟缓。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "单纯房间隔缺损" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "心功能衰竭" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "心导管检查" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "心房" }, { "start_idx": 64, "end_idx": 67, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 82, "end_idx": 85, "type": "dis", "entity": "心外畸形" }, { "start_idx": 87, "end_idx": 92, "type": "dis", "entity": "生长发育迟缓" } ] }, { "text": "后者即使在房隔缺损关闭后亦不改善。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "房隔缺损" } ] }, { "text": "通常情况下,患儿肺动脉血流量较正常高3~4倍,而肺动脉压力仅轻度升高,肺血管阻力维持正常范围。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肺动脉" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肺动脉" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "肺血管" } ] }, { "text": "但亦有在出生后3个月即发现有肺动脉阻塞性疾病的报道。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "肺动脉阻塞性疾病" } ] }, { "text": "房间隔缺损伴有由肺动脉阻塞性疾病所致的严重青紫少见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "肺动脉阻塞性疾病" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "严重青紫" } ] }, { "text": "继发孔型房间隔缺损患儿出现青紫的另一种原因是较大的冠状窦静脉瓣、瓯氏瓣或塞氏瓣(Thebesianvalve)直接将血流从下腔静脉导入房间隔缺损。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "继发孔型房间隔缺损" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "青紫" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "冠状窦静脉瓣" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "瓯氏瓣" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "塞氏瓣" }, { "start_idx": 40, "end_idx": 53, "type": "bod", "entity": "Thebesianvalve" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "血流" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "下腔静脉" }, { "start_idx": 67, "end_idx": 71, "type": "bod", "entity": "房间隔缺损" } ] }, { "text": "此时,必须手术关闭房间隔缺损。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "pro", "entity": "手术关闭房间隔缺损" } ] }, { "text": "【临床表现】多数房间隔缺损婴儿因无症状而被忽略,少数可有生长发育迟缓、反复上呼吸道感染甚至心衰。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "生长发育迟缓" }, { "start_idx": 35, "end_idx": 42, "type": "sym", "entity": "反复上呼吸道感染" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "心衰" } ] }, { "text": "只有大分流量的患儿才出现明显的气促和乏力并随年龄的增长逐年加重。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "明显的气促" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "乏力" } ] }, { "text": "体格检查可见心前区隆起心房水平左向右分流明显时可见心尖搏动明显。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "体格检查" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "心前区" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "心前区隆起" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "心房" } ] }, { "text": "听诊可及三种特征:①典型的第二音固定分裂;②在左侧胸骨旁第二肋间可及柔和的收缩期杂音在左侧胸骨旁下缘可及早-中期舒张期杂音。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "听诊" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "典型的第二音固定分裂" }, { "start_idx": 23, "end_idx": 31, "type": "bod", "entity": "左侧胸骨旁第二肋间" }, { "start_idx": 22, "end_idx": 41, "type": "sym", "entity": "在左侧胸骨旁第二肋间可及柔和的收缩期杂音" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "左侧胸骨" }, { "start_idx": 42, "end_idx": 60, "type": "sym", "entity": "在左侧胸骨旁下缘可及早-中期舒张期杂音" } ] }, { "text": "第二心音分裂的原因与以下两个原因有关:①由于在房缺时右心室收缩期搏出血量增多而使肺动脉瓣第二音出现延迟;②由于肺动脉明显扩张,造成肺动脉关闭的动脉内张力上升延迟,而使肺动脉瓣关闭滞后。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "第二心音分裂" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "房缺" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "右心室" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "肺动脉" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "肺动脉" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "动脉" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "肺动脉瓣" } ] }, { "text": "由于通过肺动脉瓣的血流量明显增加,在左侧胸骨旁上缘可及喷射性收缩期杂音,并向肺部传导。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "左侧胸骨旁上缘" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "喷射性收缩期杂音" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "肺部" } ] }, { "text": "心房水平左向右分流使舒张期通过三尖瓣的血流量增加,造成三尖瓣区舒张早中期杂音。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心房" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "三尖瓣" } ] }, { "text": "【实验室检查】(一)心电图通常为正常窦性心律,在年长儿可有交界性心律和室上性心动过速。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "心电图" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "室上性心动过速" } ] }, { "text": "由于心房内及希氏束心室肌间传导延缓,年长儿可见PR间期延长,出现Ⅰ°房室传导阻滞。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心房" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "希氏束心室肌" } ] }, { "text": "近半数患者可有P波改变,几乎所有的病例存在不同程度的V1导联rsR'或RSR'的不完全性右束支传导阻滞的表现,并伴有右心室大胸部X线心脏通常扩大,心胸比例>0.5,肺血管影随着年龄增长及左向右分流量的增加而增加。", "entities": [ { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "右心室" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "右心室大" }, { "start_idx": 73, "end_idx": 76, "type": "pro", "entity": "胸部X线" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "心脏" }, { "start_idx": 84, "end_idx": 84, "type": "bod", "entity": "心" }, { "start_idx": 85, "end_idx": 85, "type": "bod", "entity": "胸" }, { "start_idx": 93, "end_idx": 96, "type": "pro", "entity": "肺血管影" } ] }, { "text": "当出现肺血管梗阻性疾病时,主肺动脉明显扩大而外周肺野血管影稀少。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "肺血管梗阻性疾病" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "主肺动脉" }, { "start_idx": 22, "end_idx": 28, "type": "pro", "entity": "外周肺野血管影" } ] }, { "text": "(三)超声心动图1.二维超声心动图(1)直接征象:①在心尖四腔切面时因为超声束与房间隔几乎平行易产生回声失落现象。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "二维超声心动图" } ] }, { "text": "剑下两腔切面、四腔切面为最佳切面,因房间隔束与房间隔几乎垂直,再结合胸骨旁四腔切面及大动脉短轴切面帮助检出,且要多个切面结合起来诊断。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "房间隔" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "房间隔" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "胸骨旁四腔" }, { "start_idx": 42, "end_idx": 46, "type": "bod", "entity": "大动脉短轴" } ] }, { "text": "房间隔缺损的游离端呈球状增厚,形如火柴头,又称“T”字征,以此特征明确缺损的位置、大小及数目比较可靠。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "缺损" } ] }, { "text": "②明确所有肺静脉与左房的关系,以排除肺静脉异位引流。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肺静脉" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "左房" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "肺静脉异位引流" } ] }, { "text": "(2)间接征象:右心房、右心室增大、肺动脉增宽、室间隔运动平坦或与左心室后壁呈同向运动2.脉冲多普勒超声将取样容积定位于分流的右心房侧,注意让血流方向与声束夹角尽可能小,一般可以得到舒张期1~3个正向波和1个收缩早期负向波,其最大流速一般在1.3m/s以下。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "右心房" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "右心室" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "右心房、右心室增大" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肺动脉" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "肺动脉增宽" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "室间隔" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "左心室后壁" }, { "start_idx": 24, "end_idx": 42, "type": "sym", "entity": "室间隔运动平坦或与左心室后壁呈同向运动" }, { "start_idx": 45, "end_idx": 51, "type": "pro", "entity": "脉冲多普勒超声" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "右心房" } ] }, { "text": "三尖瓣流速增快,跨肺动脉血流流速加快,但一般很少超过2.5m/s,如超过要注意合并肺动脉瓣狭窄。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "跨肺动脉血流" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "肺动脉瓣狭窄" } ] }, { "text": "3.彩色多普勒血流显像通常左心房压高于右心房,故能显示由左心房入右心房的穿隔血流束,血流位于房隔的中部,上部或多条分流束,以此判断缺损的类型,也可以估计流量的大小,缺损的大小。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "彩色多普勒血流显像" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "左心房" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "右心房" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "左心房" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "右心房" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "房隔" }, { "start_idx": 65, "end_idx": 66, "type": "dis", "entity": "缺损" }, { "start_idx": 82, "end_idx": 83, "type": "dis", "entity": "缺损" } ] }, { "text": "注意分流程度并不完全取决于缺损的大小,重要的是取决于右心室的顺应性。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "缺损" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "右心室" } ] }, { "text": "值得注意的是左上腔残存的患者易与冠状静脉窦型房缺并存可结合彩色多普勒和临床其他检查以免漏诊。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "左上腔" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "冠状静脉窦型房缺" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "彩色多普勒" } ] }, { "text": "4.三维超声心动图二维超声只能从平面结构上显示房间隔缺损病变及分流束的方向与大小。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "三维超声心动图" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "二维超声" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "房间隔缺损病变" } ] }, { "text": "需观察多个不同方位上二维切面图像来想象出房间隔缺损整体形态及其毗邻结构的立体解剖结构关系,这种想象通常十分困难且不准确。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "三维超声心动图则能以三维视角观察房间隔缺损的特征、空间位置及其与周围结构的空间关系,可从右心侧(L2a)或左心侧(L1a)直接观察缺损部位的整体形态、面积、大小及与上腔静脉、下腔静脉、冠状窦等的毗邻结构关系,还能观察二维超声心动图所不能显示的面积随心动周期对称收缩的动态变化特征,从而对房间隔缺损全面病理解剖诊断,进行正确的分型及准确测量缺损大小(图9-9)。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "三维超声心动图" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "右心侧" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "左心侧" }, { "start_idx": 65, "end_idx": 66, "type": "dis", "entity": "缺损" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 87, "end_idx": 90, "type": "bod", "entity": "下腔静脉" }, { "start_idx": 92, "end_idx": 94, "type": "bod", "entity": "冠状窦" }, { "start_idx": 108, "end_idx": 114, "type": "pro", "entity": "二维超声心动图" }, { "start_idx": 143, "end_idx": 147, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 169, "end_idx": 170, "type": "dis", "entity": "缺损" } ] }, { "text": "早在1993年Belohlavek等就报道了三维超声对正常和异常房间隔能获良好显示,此后有关研究更加深入。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "三维超声" }, { "start_idx": 27, "end_idx": 34, "type": "dis", "entity": "正常和异常房间隔" } ] }, { "text": "Marx等的研究中,16例房缺病人中有13例进行了成功的动态三维重建,并能以三维视角观察缺损的特征、空间位置及其与周围结构的空间关系,如主动脉瓣与房间隔的关系,正常连接的肺静脉入口处等;Dall’Agata等对23例要外科修补的Ⅱ孔型房缺进行经胸和经食管动态三维重建,发现与手术的相关性高达0.90以上,还发现Ⅱ孔型房缺并非是单纯的两房之间的孔洞,从右房侧看,它存在于房间隔上一个形状相对独立的折叠区域内,也具有三维的结构。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "房缺" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "缺损" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "房间隔" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "肺静脉" }, { "start_idx": 109, "end_idx": 110, "type": "dep", "entity": "外科" }, { "start_idx": 114, "end_idx": 118, "type": "dis", "entity": "Ⅱ孔型房缺" }, { "start_idx": 121, "end_idx": 132, "type": "pro", "entity": "经胸和经食管动态三维重建" }, { "start_idx": 137, "end_idx": 138, "type": "pro", "entity": "手术" }, { "start_idx": 155, "end_idx": 159, "type": "dis", "entity": "Ⅱ孔型房缺" }, { "start_idx": 175, "end_idx": 176, "type": "bod", "entity": "右房" }, { "start_idx": 184, "end_idx": 186, "type": "bod", "entity": "房间隔" } ] }, { "text": "许多研究表明,三维超声可提供心脏解剖结构更为详细的空间活动信息,从而提高房间隔缺损的诊断正确性。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "三维超声" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心脏" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "图9-17三维超声心动图L2a剖视面,正对房间隔缺损观察,显示房间隔缺损大小、形态及与周围结构的关系(四)心导管及心血管造影通常对于继发孔型房间隔缺损的诊断,不必进行心导管检查。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "三维超声心动图" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 53, "end_idx": 61, "type": "pro", "entity": "心导管及心血管造影" }, { "start_idx": 66, "end_idx": 74, "type": "dis", "entity": "继发孔型房间隔缺损" }, { "start_idx": 83, "end_idx": 87, "type": "pro", "entity": "心导管检查" } ] }, { "text": "只有怀疑合并有肺动脉阻塞性疾病或其他并发畸形时才进行。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "肺动脉阻塞性疾病" } ] }, { "text": "心导管时,如果右心房的氧饱和度明显高于上、下腔静脉(>10%),应考虑有房间隔缺损的存在。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "心导管" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "右心房" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "上、下腔静脉" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "但室间隔缺损合并三尖瓣反流、左心室右心房分流、部分性或完全性房室间隔缺损、肺静脉异位引流至右心房或腔静脉或体循环动静脉瘘均可导致有心房血氧饱和度升高。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "三尖瓣反流" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "左心室右心房分流" }, { "start_idx": 23, "end_idx": 35, "type": "dis", "entity": "部分性或完全性房室间隔缺损" }, { "start_idx": 37, "end_idx": 59, "type": "dis", "entity": "肺静脉异位引流至右心房或腔静脉或体循环动静脉瘘" }, { "start_idx": 65, "end_idx": 71, "type": "ite", "entity": "心房血氧饱和度" } ] }, { "text": "在大型房间隔缺损,左右心房的收缩压或平均压相等。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "大型房间隔缺损" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "左右心房" } ] }, { "text": "右心室压轻度上升,多在25~35mmHg之间,在少数患儿可有右心室压中度上升。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "右心室压" } ] }, { "text": "有时在右心室与肺动脉间可测到15~30mmHg的压力阶差。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "右心室" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肺动脉" } ] }, { "text": "肺动脉压力多正常或轻度增高。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肺动脉" } ] }, { "text": "通常情况下,肺动脉阻力在40Um2以下。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肺动脉" } ] }, { "text": "【治疗】(一)外科治疗对于绝大多数房间隔缺损患儿,即是症状很轻甚至无症状,仍然需要选择性外科治疗。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dep", "entity": "外科" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 44, "end_idx": 45, "type": "dep", "entity": "外科" } ] }, { "text": "通常婴儿对房间隔缺损已有较好的耐受,故选择性手术时间多在2~4岁。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "手术" } ] }, { "text": "延迟手术并无任何裨益,如青春期后手术,长期的容量负荷过重可造成右心房、右心室某些不可逆的变化而导致房性心律失常甚至死亡。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "手术" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "手术" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "右心房" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "右心室" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "房性心律失常" } ] }, { "text": "如有合并心功能衰竭或肺动脉高压时应尽早手术。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "心功能衰竭" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "手术" } ] }, { "text": "(二)经导管封堵治疗自1976年King和Mills首先用双伞形补片装置成功关闭继发性房间隔缺损以来,经导管介入性治疗房间隔缺损(ASD)得到迅速发展,封堵装置先后经历了Rashkind双面伞、Lock蚌壳、Sideris可调纽扣式补片等,1997年AmplatzK推出的Amplatzer蘑菇状封堵器成为当前广泛使用的封堵装置。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "经导管封堵治疗" }, { "start_idx": 29, "end_idx": 35, "type": "equ", "entity": "双伞形补片装置" }, { "start_idx": 40, "end_idx": 47, "type": "dis", "entity": "继发性房间隔缺损" }, { "start_idx": 51, "end_idx": 63, "type": "pro", "entity": "经导管介入性治疗房间隔缺损" }, { "start_idx": 65, "end_idx": 67, "type": "pro", "entity": "ASD" }, { "start_idx": 76, "end_idx": 79, "type": "equ", "entity": "封堵装置" }, { "start_idx": 85, "end_idx": 95, "type": "equ", "entity": "Rashkind双面伞" }, { "start_idx": 97, "end_idx": 102, "type": "equ", "entity": "Lock蚌壳" }, { "start_idx": 104, "end_idx": 117, "type": "equ", "entity": "Sideris可调纽扣式补片" }, { "start_idx": 136, "end_idx": 150, "type": "equ", "entity": "Amplatzer蘑菇状封堵器" }, { "start_idx": 160, "end_idx": 163, "type": "equ", "entity": "封堵装置" } ] }, { "text": "而超声心动图在ASD经导管封堵治疗的术前筛查、术中监视及术后效果评价中起着重要作用。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "超声心动图" }, { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "ASD" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "经导管封堵治疗" } ] }, { "text": "封堵术的并发症有残余分流、装置结构折断、装置脱落栓塞等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "封堵术" } ] }, { "text": "第十六篇神经肌肉疾病第一章儿童神经系统的解剖生理特点一、人类神经系统发育形成的主要过程人类神经系统结构和功能非常复杂。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "神经肌肉疾病" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "神经系统" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "神经系统" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "神经系统" } ] }, { "text": "据估计,人脑至少有1011个神经元。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "人脑" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "神经元" } ] }, { "text": "神经元之间以错综复杂的联系形成神经网络,在突触处又有多种化学活性物质参与神经信息的传递。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "神经元" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "神经" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "突触" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "神经" } ] }, { "text": "神经系统调节人体所有的生理功能以及学习、记忆和思维等高级神经活动。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "神经系统" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "神经" } ] }, { "text": "脑的正常发育对以后神经系统的结构和功能至关重要。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "脑" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "神经系统" } ] }, { "text": "传统的观点认为神经元代表神经系统主要结构单位,而胶质细胞的作用被降低至被动地位。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "神经元" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "神经系统" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "胶质细胞" } ] }, { "text": "近年大量研究证实,胶质细胞可对神经元发育与功能起到多方面的调控作用。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "胶质细胞" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "神经元" } ] }, { "text": "神经元与胶质细胞的动态相互作用已被认为是神经系统的基础功能单位。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "神经元" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "胶质细胞" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "神经系统" } ] }, { "text": "在发育过程中遗传与环境因素共同作用而产生神经细胞存活与死亡的平衡,控制其过度增殖,从而保证各型神经细胞应有的数量。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "神经细胞" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "存活" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "死亡" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "过度增殖" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "神经细胞" } ] }, { "text": "在无脊椎动物,其中枢神经系统(CNS)相对简单,生命周期较短,因此其发育主要受遗传因素所指导。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "CNS" } ] }, { "text": "脊椎动物CNS发育则反映了遗传/环境作用的连续性,环境因素对神经系统的发育产生了更多的影响。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "CNS" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "神经系统" } ] }, { "text": "人类神经系统发育的主要程序包括诱导及原始神经胚形成(发生高峰在妊娠3~4周)、前脑发育(发生高峰在2~3个月)、神经细胞增殖与分化(发生高峰在妊娠3~4个月),以及神经细胞移行与分化(发生高峰于妊娠3~5个月),神经组织过程包括突触连结及神经回路建立、树突发芽、膜兴奋性形成成(发生高峰于妊娠5个月~出生后数年)以及髓鞘化(发生高峰在出生~出生后数年)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "神经系统" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "原始神经胚" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "前脑" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "神经细胞" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "神经细胞" }, { "start_idx": 106, "end_idx": 109, "type": "bod", "entity": "神经组织" }, { "start_idx": 131, "end_idx": 136, "type": "sym", "entity": "膜兴奋性形成" }, { "start_idx": 158, "end_idx": 160, "type": "sym", "entity": "髓鞘化" } ] }, { "text": "(一)诱导及神经胚形成人类胚胎发育的第3周,外胚层在脊索中胚层的诱导下分化为神经外胚层。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "神经胚" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "脊索" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "神经" } ] }, { "text": "神经外胚层通过细胞增殖、增厚而形成神经板(neuralplate),进而向内凹陷形成神经沟,神经沟闭合形成神经管(neuraltube)沿神经板内陷的每侧边缘处出现神经嵴神经管在其内不断升高的液体压力作用下,前端膨胀形成三个囊逐渐形成前、中、后脑中枢神经系统和周围神经系统的一部分。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "神经" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "神经管" }, { "start_idx": 57, "end_idx": 66, "type": "bod", "entity": "neuraltube" }, { "start_idx": 46, "end_idx": 67, "type": "sym", "entity": "神经沟闭合形成神经管(neuraltube)" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "神经嵴" }, { "start_idx": 68, "end_idx": 84, "type": "sym", "entity": "沿神经板内陷的每侧边缘处出现神经嵴" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "神经管" }, { "start_idx": 112, "end_idx": 112, "type": "bod", "entity": "囊" }, { "start_idx": 104, "end_idx": 112, "type": "sym", "entity": "前端膨胀形成三个囊" }, { "start_idx": 117, "end_idx": 122, "type": "bod", "entity": "前、中、后脑" }, { "start_idx": 113, "end_idx": 122, "type": "sym", "entity": "逐渐形成前、中、后脑" }, { "start_idx": 123, "end_idx": 128, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 132, "end_idx": 135, "type": "bod", "entity": "神经系统" } ] }, { "text": "神经嵴细胞在神经管背中线处起源,在神经管闭合时或稍后即开始移行,逐渐分化成脑神经节、脊神经节和自主神经系统。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "神经嵴细胞" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "神经管背中线" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "神经管" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "脑神经节" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "脊神经节" }, { "start_idx": 47, "end_idx": 52, "type": "bod", "entity": "自主神经系统" } ] }, { "text": "(二)前脑发育(proencephalicdevelopment)前脑发育的高峰期为妊娠2~3个月,主要包括以下过程:①前脑形成:开始于神经管头端,自妊娠1个月末前神经孔闭合时开始。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "前脑" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "前脑" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "前脑" }, { "start_idx": 68, "end_idx": 72, "type": "bod", "entity": "神经管头端" } ] }, { "text": "②前脑分裂:高峰期为妊娠5~6周,主要包括水平方向形成一对视囊、嗅球及嗅径横贯分开端脑及间脑前脑中线发育:高峰期在妊娠2~3个月后,是形成胼胝体、透明隔、视交叉及下丘脑结构的重要基础。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "前脑" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "视囊" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "嗅球" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "嗅径" }, { "start_idx": 21, "end_idx": 36, "type": "sym", "entity": "水平方向形成一对视囊、嗅球及嗅径" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "端脑" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "间脑" }, { "start_idx": 37, "end_idx": 45, "type": "sym", "entity": "横贯分开端脑及间脑" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "前脑中线" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "胼胝体" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "透明隔" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "视交叉" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "下丘脑" } ] }, { "text": "(三)神经细胞增殖(proliferation)与分化(differentiation)人类胚胎发育的第3~4个月是神经元增殖的主要时期,此后至出生1年小脑外颗粒层仍在继续增殖,其他神经元出生后则已停止增殖。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经细胞" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "神经元" }, { "start_idx": 76, "end_idx": 81, "type": "bod", "entity": "小脑外颗粒层" }, { "start_idx": 91, "end_idx": 93, "type": "bod", "entity": "神经元" } ] }, { "text": "(四)神经细胞的移行(migration)与分化在神经系统发育过程中,神经细胞往往从其“出生地”出发,经过长短不等的路程,迁移到预定的位置。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经细胞" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "神经系统" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "神经细胞" } ] }, { "text": "这对于从一个薄壁的神经管演化成结构复杂的脑是十分必要的。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "神经管" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "脑" } ] }, { "text": "神经元移行发生高峰出现于妊娠3~5个月。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "神经元" } ] }, { "text": "这一时期的遗传和环境的任何异常因素均可导致神经元移行障碍,例如脑裂畸形(schizencephaly)、无脑回(lissencephaly)、多小脑回(polymicrogyria)以及灰质异位(heterotopia),临床常表现为智力和运动发育障碍及惊厥发作。", "entities": [ { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "神经元移行障碍" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "脑裂畸形" }, { "start_idx": 36, "end_idx": 49, "type": "dis", "entity": "schizencephaly" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "无脑回" }, { "start_idx": 56, "end_idx": 68, "type": "dis", "entity": "lissencephaly" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "多小脑回" }, { "start_idx": 76, "end_idx": 89, "type": "dis", "entity": "polymicrogyria" }, { "start_idx": 93, "end_idx": 96, "type": "dis", "entity": "灰质异位" }, { "start_idx": 98, "end_idx": 108, "type": "dis", "entity": "heterotopia" }, { "start_idx": 117, "end_idx": 130, "type": "dis", "entity": "智力和运动发育障碍及惊厥发作" } ] }, { "text": "随着影像诊断学技术进步,尤其是高分辨磁共振技术发展,皮层发育不良已经成为儿童难治性癫痫的重要原因。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dep", "entity": "影像诊断学" }, { "start_idx": 15, "end_idx": 22, "type": "pro", "entity": "高分辨磁共振技术" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "皮层发育不良" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "儿童难治性癫痫" } ] }, { "text": "(五)脑发育的组织过程(organization)正常脑发育的组织过程十分复杂,包括板层结构形成、突触连结及神经回路建立、神经突起生长发芽、膜兴奋性形成成以及胶质细胞增殖分化等。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "脑" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "脑" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "板层结构形成" }, { "start_idx": 70, "end_idx": 75, "type": "sym", "entity": "膜兴奋性形成" } ] }, { "text": "脑发育的组织过程起始于妊娠5个月,直至出生后数年仍在继续。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "脑" } ] }, { "text": "脑发育的组织过程可因多种因素引起异常,造成各类发育性脑病。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "脑" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "发育性脑病" } ] }, { "text": "例如智力低下及婴儿孤独症等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "智力低下" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "婴儿孤独症" } ] }, { "text": "(六)髓鞘化(myelination)人类神经系统的髓鞘化在出生后即开始,在出生后前8个月最快,可持续至生后数年。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "sym", "entity": "髓鞘化" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "神经系统" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "髓鞘化" } ] }, { "text": "第七节囊性纤维化囊性纤维化(cysticfibrosis,CF)是一种常染色体隐性遗传性疾病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "囊性纤维化" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "囊性纤维化" }, { "start_idx": 14, "end_idx": 27, "type": "dis", "entity": "cysticfibrosis" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "CF" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "常染色体" } ] }, { "text": "这一基因编码一种1480个氨基酸的蛋白质,称为CF跨膜调节蛋白(CFtransmembraneregulator,CFTR)。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "蛋白质" }, { "start_idx": 23, "end_idx": 30, "type": "bod", "entity": "CF跨膜调节蛋白" }, { "start_idx": 32, "end_idx": 55, "type": "bod", "entity": "CFtransmembraneregulator" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "CFTR" } ] }, { "text": "后者表达于气道、胃肠道(包括胰、胆道系统)、汗腺、泌尿生殖道上皮,具有离子通道和调节功能。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "气道" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "胃肠道" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "胰、胆道系统" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "汗腺" }, { "start_idx": 25, "end_idx": 31, "type": "bod", "entity": "泌尿生殖道上皮" } ] }, { "text": "CF基因突变可能影响上皮细胞Cl-分泌,而Na+吸收过量,导致气道表面水分不足,分泌物变得干燥黏稠、不易排出,因而易发生金黄色葡萄球菌和铜绿假单胞菌的呼吸道定植和继发感染。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "CF基因突变" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "气道" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "分泌物" }, { "start_idx": 82, "end_idx": 88, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 90, "end_idx": 95, "type": "mic", "entity": "铜绿假单胞菌" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "呼吸道" }, { "start_idx": 105, "end_idx": 106, "type": "dis", "entity": "感染" } ] }, { "text": "全身黏液分泌腺阻塞扩张,继而萎缩纤维变性。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "黏液分泌腺" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "萎缩纤维变性" } ] }, { "text": "由于胰腺外分泌不足,导致脂肪吸收障碍和营养不良。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "胰腺" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "脂肪吸收障碍" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "营养不良" } ] }, { "text": "新生儿可由于胰液消化力弱,致胎粪呈硬性灰白色油状物,引起胎粪性肠梗阻。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "胰液" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "胎粪呈硬性灰白色油状物" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "胎粪性肠梗阻" } ] }, { "text": "由于汗腺导管的功能是吸收Cl-而不是分泌Cl-,因而CF患儿汗液中Cl-重吸收障碍,导致汗液中Cl-和Na+水平升高。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "汗腺导管" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "CF" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "汗液" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "汗液" } ] }, { "text": "本病是引起儿童慢性严重肺部疾病的主要原因,并与胰腺外分泌功能不全有关。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "慢性严重肺部疾病" }, { "start_idx": 23, "end_idx": 31, "type": "dis", "entity": "胰腺外分泌功能不全" } ] }, { "text": "临床上以气道梗阻和感染及消化不良为特征。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "气道梗阻" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "感染" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "消化不良" } ] }, { "text": "根据美国20000例CF患者的调查,表现急性或持续呼吸道症状者占50.5%,生长发育障碍、营养不良者占42.9%,大便异常(如脂肪泻)占35.0%,胎粪性肠梗阻为18.8%,有家族史者占16.8%。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "CF" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "急性或持续呼吸道症状" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "生长发育障碍" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "营养不良" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "大便异常" }, { "start_idx": 63, "end_idx": 65, "type": "sym", "entity": "脂肪泻" }, { "start_idx": 74, "end_idx": 79, "type": "dis", "entity": "胎粪性肠梗阻" } ] }, { "text": "有些患儿可长时间无症状,或仅有反复肺炎或急性呼吸道感染迁延不愈。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "肺炎" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "急性呼吸道感染" } ] }, { "text": "婴儿常闻哮鸣音。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "哮鸣音" } ] }, { "text": "婴儿反复呼吸道感染若伴有消化不良,须考虑本病之可能。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "消化不良" } ] }, { "text": "用毛果芸香碱电离子透入法收集并测定汗液中Cl-含量,如超过60mmol/L,结合下列一项或一项以上可诊断本病:典型慢性阻塞性肺疾病,胰腺外分泌功能不全,阳性家族史。", "entities": [ { "start_idx": 1, "end_idx": 11, "type": "pro", "entity": "毛果芸香碱电离子透入法" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "汗液" }, { "start_idx": 68, "end_idx": 75, "type": "dis", "entity": "慢性阻塞性肺疾病" }, { "start_idx": 77, "end_idx": 85, "type": "dis", "entity": "胰腺外分泌功能不全" } ] }, { "text": "近年来推荐新的诊断标准:典型临床特征(呼吸道症状、胃肠道、泌尿生殖系统症状),或同胞中有CF患儿,或新生儿筛查试验阳性,加下列CFTR功能紊乱的实验室证据之一即可诊断:两个不同日期采集的汗液Cl-含量升高,鉴定出两种CF突变,鼻上皮电位差测定异常。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "呼吸道" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "胃肠道" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "泌尿生殖系统" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "CF" }, { "start_idx": 50, "end_idx": 56, "type": "ite", "entity": "新生儿筛查试验" }, { "start_idx": 63, "end_idx": 70, "type": "sym", "entity": "CFTR功能紊乱" }, { "start_idx": 93, "end_idx": 94, "type": "bod", "entity": "汗液" }, { "start_idx": 119, "end_idx": 122, "type": "dis", "entity": "CF突变" }, { "start_idx": 124, "end_idx": 131, "type": "ite", "entity": "鼻上皮电位差测定" } ] }, { "text": "设法去除呼吸道内黏稠分泌物,加强肺部物理治疗,尤其是胸部叩打和体位引流,根据病情每天1~4次;注意口服补液或静脉补液,尤其是炎热季节及伴发急性胃肠炎时,防止脱水和气道分泌物干燥黏稠;雾化吸入0.45~0.9%氯化钠可湿化气道、稀化痰液,亦可加用人重组DNA酶(2.5mg,每天1次)雾化吸入。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "呼吸道" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "黏稠分泌物" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "肺部物理治疗" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "胸部叩打" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "体位引流" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "口服补液" }, { "start_idx": 54, "end_idx": 57, "type": "pro", "entity": "静脉补液" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "急性胃肠炎" }, { "start_idx": 78, "end_idx": 79, "type": "sym", "entity": "脱水" }, { "start_idx": 81, "end_idx": 89, "type": "sym", "entity": "气道分泌物干燥黏稠" }, { "start_idx": 91, "end_idx": 94, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 104, "end_idx": 106, "type": "dru", "entity": "氯化钠" }, { "start_idx": 110, "end_idx": 111, "type": "bod", "entity": "气道" }, { "start_idx": 115, "end_idx": 116, "type": "bod", "entity": "痰液" }, { "start_idx": 122, "end_idx": 128, "type": "dru", "entity": "人重组DNA酶" }, { "start_idx": 141, "end_idx": 144, "type": "pro", "entity": "雾化吸入" } ] }, { "text": "N-乙酰半胱氨酸可溶解黏液,但可损害纤毛上皮,应避免反复使用。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dru", "entity": "N-乙酰半胱氨酸" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "纤毛上皮" } ] }, { "text": "伴可逆性气道梗阻者可应用β受体兴奋剂或色苷酸钠、盐酸异丙托品。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "可逆性气道梗阻" }, { "start_idx": 12, "end_idx": 17, "type": "dru", "entity": "β受体兴奋剂" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "色苷酸钠" }, { "start_idx": 24, "end_idx": 29, "type": "dru", "entity": "盐酸异丙托品" } ] }, { "text": "适当放宽抗生素应用指征,剂量为一般轻症感染的2~3倍。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "抗生素" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "轻症感染" } ] }, { "text": "轻症感染可口服给药,主要针对常见病原如金黄色葡萄球菌、不定型流感嗜血杆菌、铜绿假单胞菌等,最好根据细菌培养和药物敏感试验结果选用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "轻症感染" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "口服" }, { "start_idx": 19, "end_idx": 25, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 27, "end_idx": 35, "type": "mic", "entity": "不定型流感嗜血杆菌" }, { "start_idx": 37, "end_idx": 42, "type": "mic", "entity": "铜绿假单胞菌" }, { "start_idx": 49, "end_idx": 52, "type": "ite", "entity": "细菌培养" }, { "start_idx": 54, "end_idx": 59, "type": "ite", "entity": "药物敏感试验" } ] }, { "text": "严重感染者应静脉给药,疗程不少于2周。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "感染" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "静脉给药" } ] }, { "text": "为控制炎症过程,延缓肺部病变的发展,有人提出应用皮质激素吸入治疗,但迄今尚缺乏肯定有效的证据,常规应用仅限于伴有严重反应性气道疾病者。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "炎症" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "肺部病变" }, { "start_idx": 24, "end_idx": 31, "type": "pro", "entity": "皮质激素吸入治疗" }, { "start_idx": 56, "end_idx": 64, "type": "dis", "entity": "严重反应性气道疾病" } ] }, { "text": "非甾体类抗炎药物如布洛芬可用于肺部病变较轻、一秒用力呼气量(FEV1)在预计值60%以上的5~12岁儿童。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dru", "entity": "非甾体类抗炎药物" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "布洛芬" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "肺部病变" }, { "start_idx": 22, "end_idx": 28, "type": "ite", "entity": "一秒用力呼气量" }, { "start_idx": 30, "end_idx": 44, "type": "ite", "entity": "FEV1" } ] }, { "text": "此外,应给予高热量、高蛋白饮食,并注意补充多种维生素。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "pro", "entity": "高热量、高蛋白饮食" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "维生素" } ] }, { "text": "一些新的治疗手段目前正在积极研究中,如基因治疗、突变CFTR的药物上调、刺激其他Cl-转运机制等。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "基因治疗" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "突变CFTR" } ] }, { "text": "第五章小儿艾滋病小儿艾滋病即小儿时期的获得性免疫缺陷综合征(acquiredimmunodeficiencysyndrome,AIDS),由人类免疫缺陷病毒(humanimmunodeficiencyvirus,HIV)引起。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "小儿艾滋病" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "小儿艾滋病" }, { "start_idx": 14, "end_idx": 28, "type": "dis", "entity": "小儿时期的获得性免疫缺陷综合征" }, { "start_idx": 30, "end_idx": 61, "type": "dis", "entity": "acquiredimmunodeficiencysyndrome" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "AIDS" }, { "start_idx": 70, "end_idx": 77, "type": "dis", "entity": "人类免疫缺陷病毒" }, { "start_idx": 79, "end_idx": 104, "type": "mic", "entity": "humanimmunodeficiencyvirus" }, { "start_idx": 106, "end_idx": 108, "type": "mic", "entity": "HIV" } ] }, { "text": "AIDS为目前人类最为严重的传染病之一,小儿AIDS发生率的增长比成人快,每年约有40万儿童新感染HIV,截至2000年,全球约有800万儿童为HIV病毒携带者或AIDS患者。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "AIDS" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "小儿AIDS" }, { "start_idx": 49, "end_idx": 51, "type": "mic", "entity": "HIV" }, { "start_idx": 72, "end_idx": 76, "type": "mic", "entity": "HIV病毒" }, { "start_idx": 81, "end_idx": 84, "type": "dis", "entity": "AIDS" } ] }, { "text": "这些受HIV感染的儿童大部分出生在发展中国家,而在北美及西欧,新生婴儿HIV病毒的感染率近年已明显下降。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "HIV感染" }, { "start_idx": 35, "end_idx": 37, "type": "mic", "entity": "HIV" }, { "start_idx": 38, "end_idx": 39, "type": "mic", "entity": "病毒" } ] }, { "text": "儿童HIV感染后的病理进程较成人更快,一些儿童可在2岁内因AIDS死亡,这是因为AIDS儿童的病毒负荷较成人大,被感染的CD4+T淋巴细胞(CD4+Tlymphocyte)在体内消耗的速度较成人更快的缘故。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "HIV感染" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "AIDS" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "AIDS" }, { "start_idx": 47, "end_idx": 48, "type": "mic", "entity": "病毒" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "T淋巴细胞" } ] }, { "text": "我国目前已成为HIV感染的高发地区。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "HIV感染" } ] }, { "text": "1998年年初的统计表明,我国HIV感染者为9970人。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "mic", "entity": "HIV" } ] }, { "text": "至2002年年初,HIV感染者已急剧上升为80余万人,其中约40万人为AIDS现症病人。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "HIV感染" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "AIDS" } ] }, { "text": "因此小儿AIDS的防治,已成为儿科学界新的严峻挑战。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "AIDS" } ] }, { "text": "【病原和传播途径】(一)病原HIV属于反转录病毒科,慢病毒属中的灵长类免疫缺陷病毒亚属。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "mic", "entity": "HIV" }, { "start_idx": 22, "end_idx": 23, "type": "mic", "entity": "病毒" }, { "start_idx": 26, "end_idx": 29, "type": "mic", "entity": "慢病毒属" }, { "start_idx": 32, "end_idx": 42, "type": "mic", "entity": "灵长类免疫缺陷病毒亚属" } ] }, { "text": "目前已发现的HIV有两型,即HIV-1和HIV-2。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "HIV" }, { "start_idx": 14, "end_idx": 18, "type": "mic", "entity": "HIV-1" }, { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "HIV-2" } ] }, { "text": "HIV-1是引起全球性AIDS蔓延的主要病原体,而HIV-2的流行主要局限西非地区。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "HIV-1" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "AIDS" }, { "start_idx": 25, "end_idx": 29, "type": "mic", "entity": "HIV-2" } ] }, { "text": "HIV病毒颗粒是一个直径大约为100nm的圆球体,核心有两个单股正链RNA和由病毒编码的p17、p24、p7及p6等核蛋白构成,病毒最外面的包膜则由病毒编码的糖蛋白gp120和gp41等以及类脂组成。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "mic", "entity": "HIV病毒颗粒" }, { "start_idx": 30, "end_idx": 36, "type": "bod", "entity": "单股正链RNA" }, { "start_idx": 39, "end_idx": 40, "type": "mic", "entity": "病毒" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "核蛋白" }, { "start_idx": 64, "end_idx": 65, "type": "mic", "entity": "病毒" }, { "start_idx": 74, "end_idx": 75, "type": "mic", "entity": "病毒" }, { "start_idx": 79, "end_idx": 86, "type": "bod", "entity": "糖蛋白gp120" }, { "start_idx": 88, "end_idx": 91, "type": "bod", "entity": "gp41" } ] }, { "text": "(二)传播途径流行病学研究证实,HIV的传播主要通过三个途径:1.性传播即通过同性及异性间的性接触传播。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "mic", "entity": "HIV" } ] }, { "text": "为HIV的主要传播途径,全球70%~80%的HIV感染是通过性传播的。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "mic", "entity": "HIV" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "HIV感染" } ] }, { "text": "2.静脉药瘾者共用注射器传播输血和血液制品传播以及移植手术和针刺意外传播等。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "equ", "entity": "注射器" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "输血" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "血液制品" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "移植手术" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "针刺" } ] }, { "text": "3.母婴传播即垂直传播为小儿感染HIV的主要途径,其传播率为22%~65%。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "小儿感染HIV" } ] }, { "text": "垂直传播途径:①胎儿期经胎盘传播;②分娩中经母亲的血液和分泌物传播;③分娩后经母乳传播。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胎盘" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血液" } ] }, { "text": "其中以宫内感染为多见。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "宫内感染" } ] }, { "text": "【发病机制】HIV致病的中心环节,是选择性地使CD4+T淋巴细胞大量消耗,导致免疫功能缺陷。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "HIV" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "T淋巴细胞" } ] }, { "text": "当HIV侵入人体后,首先识别CD4+T淋巴细胞和巨噬细胞,由病毒外膜的包膜蛋白gp120与细胞表面的CD4受体及辅助受体结合,使gp120构象改变,暴露出与细胞趋化因子受体结合的位点,并与之作用,从而介导HIV对CD4+细胞的吸附。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "mic", "entity": "HIV" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "T淋巴细胞" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 36, "end_idx": 37, "type": "mic", "entity": "病毒" }, { "start_idx": 41, "end_idx": 49, "type": "bod", "entity": "包膜蛋白gp120" }, { "start_idx": 56, "end_idx": 60, "type": "bod", "entity": "CD4受体" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "辅助受体" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "gp120" }, { "start_idx": 84, "end_idx": 91, "type": "bod", "entity": "细胞趋化因子受体" }, { "start_idx": 108, "end_idx": 110, "type": "mic", "entity": "HIV" } ] }, { "text": "引起病毒脱衣壳,病毒核心部分进入细胞,在反转录酶的作用下,病毒的RNA转录为双链DNA,双链DNA在整合酶作用下与宿主细胞染色体DNA整合并形成前病毒DNA,随染色体复制而复制。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "病毒" }, { "start_idx": 8, "end_idx": 9, "type": "mic", "entity": "病毒" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "细胞" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "反转录酶" }, { "start_idx": 29, "end_idx": 30, "type": "mic", "entity": "病毒" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "RNA" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "双链DNA" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "双链DNA" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "整合酶" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "宿主细胞" }, { "start_idx": 61, "end_idx": 66, "type": "bod", "entity": "染色体DNA" }, { "start_idx": 72, "end_idx": 77, "type": "bod", "entity": "前病毒DNA" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "染色体" } ] }, { "text": "在人类,从黏膜感染到出现最初的病毒血症大约需要4~11天。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "黏膜感染" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "病毒血症" } ] }, { "text": "经过短暂的高滴度病毒血症期后,病毒播散到全身淋巴组织。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "高滴度病毒血症" }, { "start_idx": 15, "end_idx": 16, "type": "mic", "entity": "病毒" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "淋巴组织" } ] }, { "text": "由于机体的免疫应答,病毒复制被抑制,血中病毒含量迅速下降。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "病毒" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "血" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "病毒" } ] }, { "text": "此后,HIV感染分为两种不同的发展趋势,一种为快速进展,感染者迅速发病及死亡;另一种为缓慢进展,感染者进入慢性持续感染阶段。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "HIV感染" } ] }, { "text": "影响HIV感染向何种趋势发展的因素尚不清楚,可能与病毒的毒力、含量、宿主细胞辅助受体及免疫应答能力的差异有关。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "HIV" }, { "start_idx": 25, "end_idx": 26, "type": "mic", "entity": "病毒" }, { "start_idx": 34, "end_idx": 41, "type": "bod", "entity": "宿主细胞辅助受体" } ] }, { "text": "CD4+T淋巴细胞消耗的机制:HIV可感染CD4+T淋巴细胞以及表达CD4分子的单核/巨噬细胞和树突状细胞。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "T淋巴细胞" }, { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "HIV" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "T淋巴细胞" }, { "start_idx": 52, "end_idx": 58, "type": "bod", "entity": "单核/巨噬细胞" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "树突状细胞" } ] }, { "text": "病毒在易感细胞内复制、表达、整合并形成融合细胞,提示HIV可干扰或抑制细胞的正常功能,甚至对细胞造成直接损伤。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "细胞" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "细胞" }, { "start_idx": 26, "end_idx": 28, "type": "mic", "entity": "HIV" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "细胞" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "细胞" } ] }, { "text": "但是,针以HIV的特异性免疫应答,则是导致HIV感染细胞损伤的直接原因。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "mic", "entity": "HIV" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "HIV感染细胞" } ] }, { "text": "被感染的CD4+T细胞由于表面表达HIV抗原,可被机体的细胞毒性T淋巴细胞(cytotoxicTlymphocyte,CTL)所识别和攻击,CTL清除病毒的同时破坏了大量受感染的CD4+T淋巴细胞。", "entities": [ { "start_idx": 23, "end_idx": 27, "type": "mic", "entity": "HIV抗原" }, { "start_idx": 34, "end_idx": 42, "type": "bod", "entity": "细胞毒性T淋巴细胞" }, { "start_idx": 44, "end_idx": 63, "type": "bod", "entity": "cytotoxicTlymphocyte" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "CTL" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "CTL" }, { "start_idx": 81, "end_idx": 82, "type": "mic", "entity": "病毒" }, { "start_idx": 105, "end_idx": 109, "type": "bod", "entity": "T淋巴细胞" } ] }, { "text": "但HIV具有高度变异性,虽然大量病毒被清除,变异的病毒株仍可存活下来,又侵入新生的CD4+T淋巴细胞,并在其中复制表达;再为CTL识别、攻击和清除,CD4+T淋巴细胞又大量被破坏。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "mic", "entity": "HIV" }, { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "病毒" }, { "start_idx": 25, "end_idx": 26, "type": "mic", "entity": "病毒" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "T淋巴细胞" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "CTL" }, { "start_idx": 90, "end_idx": 94, "type": "bod", "entity": "T淋巴细胞" } ] }, { "text": "如此周而复始,最后CD4+T淋巴细胞被消耗殆尽,形成继发性免疫缺陷,各种机会性病原体乘虚而入,在体内繁殖并引起疾病。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "T淋巴细胞" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "继发性免疫缺陷" } ] }, { "text": "【临床表现】小儿感染HIV后,约经过5年左右的潜伏期才出现症状,绝大多数宫内HIV感染的婴儿在出生时无临床症状,体格检查正常。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "mic", "entity": "HIV" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "宫内HIV感染" } ] }, { "text": "约15%~25%围生期HIV感染的婴儿在生后数月发病,以后每年约以10%的比例增加。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "围生期HIV感染" } ] }, { "text": "故小儿艾滋病的临床经过较成人艾滋病更为凶险。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "小儿艾滋病" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "艾滋病" } ] }, { "text": "在确诊艾滋病之前,患儿常出现一些非特异性的临床表现,包括轻度生长迟缓,肝脾肿大全身淋巴结肿大间歇发热,非特异性间歇性腹泻和慢性皮肤感染HIV感染的可能。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "艾滋病" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "轻度生长迟缓" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "肝" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "脾" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "淋巴结" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "全身淋巴结肿大" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "间歇发热" }, { "start_idx": 51, "end_idx": 59, "type": "sym", "entity": "非特异性间歇性腹泻" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "皮肤" }, { "start_idx": 61, "end_idx": 66, "type": "sym", "entity": "慢性皮肤感染" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "HIV感染" } ] }, { "text": "(一)临床分期1994年美国疾病控制中心根据临床表现将HIV感染分为以下4期:无临床表现(N)、轻度临床表现(A)、中度临床表现(B)和严重临床表现(C)。", "entities": [ { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "HIV感染" } ] }, { "text": "2.轻微临床表现期(A)具有下列2个或更多的表现,但无中度和严重临床表现期的临床征象:(1)淋巴结肿大肝脏肿大脾脏肿大皮炎;(5)腮腺炎;(6)反复或持续性上呼吸道感染鼻窦炎或中耳炎。", "entities": [ { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "淋巴结" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "淋巴结肿大" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "肝脏" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "肝脏肿大" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "脾脏" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "脾脏肿大" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "皮炎" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 78, "end_idx": 83, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 72, "end_idx": 83, "type": "sym", "entity": "反复或持续性上呼吸道感染" }, { "start_idx": 84, "end_idx": 86, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 88, "end_idx": 90, "type": "dis", "entity": "中耳炎" } ] }, { "text": "3.中度临床表现期(B)除A期的表现外,尚有以下表现:(1)贫血(Hb<80g/L),中性粒细胞减少(<1×109/L),或血小板减少(<100×109/L),持续≥30天;(2)细菌性脑膜炎、肺炎或败血症;(3)6个月内婴儿持续2个月以上的口腔念珠菌病;(4)心肌病;(5)生后1个月内发生巨细胞病毒感染(CMV);(6)反复和慢性腹泻;(7)肝炎;(8)反复发生单纯疱疹病毒性口腔炎(1年内≥2次);(9)生后1个月发生单纯疱疹病毒性毛细支气管炎、肺炎或食管炎;(10)带状疱疹至少发作2次或出现不同的皮损部位;(11)平滑肌肉瘤伴有EB病毒感染;(12)淋巴样间质性肺炎或肺淋巴样增生综合征;(13)肾脏病变;(14)奴卡菌病(nocardiosis);(15)持续发热1个月以上;(16)出生1个月内发生弓形虫感染播散性水痘(有并发症的水痘)细菌感染,包括脓毒血症、肺炎、脑膜炎、骨关节感染和深部脓肿;(2)念珠菌感染累及食管、气管、支气管和肺;(3)深部真菌感染,呈播散性(肺、肺门和颈淋巴结以外的区域);(4)肺外隐球菌病;(5)隐孢子菌感染伴持续腹泻1个月以上;(6)出生后1个月的巨细胞病毒感染,累及肝、脾和淋巴结以外的部位;(7)脑病:以下表现之一,病程至少持续2个月以上,而不能以其他原因解释者:①发育滞后或倒退,智能倒退;②脑发育受损,头围测定证实为后天性小头畸形或CT/MRI检查发现有脑萎缩后天性系统性运动功能障碍,表现为下列2个或2个以上征象:麻痹,病理性反射征,共济失调步态不稳;(8)单纯疱疹病毒性黏膜溃疡持续1个月以上,或出生1个月以后的小儿患单纯疱疹病毒性支气管炎;(9)组织胞浆菌病累及肺、肺门和颈淋巴结以外的区域;(10)Kaposi肉瘤;(11)原发性脑内淋巴瘤;(12)Burkitt淋巴瘤,B细胞性或免疫学表型不明的大细胞性淋巴瘤;(13)播散性或肺外性结核病;(14)卡氏肺囊虫性肺炎(PCP);(15)进行性多发性白质性脑病;(16)沙门菌属(非伤寒)脓毒血症,反复发作;(17)出生1个月以后发生脑弓形虫感染;(18)消耗综合征:①体重连续减轻10%以上;②大于1岁者的体重-年龄曲线下降25个百分位;③出生1个月后的体重-身高曲线下降5个百分位;④并伴有慢性腹泻(每天至少2次稀便持续1个月以上)或发热1个月以上(持续或间歇性)。", "entities": [ { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "贫血" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "中性粒细胞减少" }, { "start_idx": 68, "end_idx": 72, "type": "sym", "entity": "血小板减少" }, { "start_idx": 102, "end_idx": 107, "type": "dis", "entity": "细菌性脑膜炎" }, { "start_idx": 109, "end_idx": 110, "type": "dis", "entity": "肺炎" }, { "start_idx": 112, "end_idx": 114, "type": "dis", "entity": "败血症" }, { "start_idx": 133, "end_idx": 138, "type": "dis", "entity": "口腔念珠菌病" }, { "start_idx": 143, "end_idx": 145, "type": "dis", "entity": "心肌病" }, { "start_idx": 158, "end_idx": 164, "type": "dis", "entity": "巨细胞病毒感染" }, { "start_idx": 166, "end_idx": 168, "type": "dis", "entity": "CMV" }, { "start_idx": 174, "end_idx": 180, "type": "sym", "entity": "反复和慢性腹泻" }, { "start_idx": 185, "end_idx": 186, "type": "dis", "entity": "肝炎" }, { "start_idx": 224, "end_idx": 236, "type": "dis", "entity": "单纯疱疹病毒性毛细支气管炎" }, { "start_idx": 238, "end_idx": 239, "type": "dis", "entity": "肺炎" }, { "start_idx": 241, "end_idx": 243, "type": "dis", "entity": "食管炎" }, { "start_idx": 249, "end_idx": 252, "type": "dis", "entity": "带状疱疹" }, { "start_idx": 274, "end_idx": 278, "type": "dis", "entity": "平滑肌肉瘤" }, { "start_idx": 281, "end_idx": 286, "type": "dis", "entity": "EB病毒感染" }, { "start_idx": 292, "end_idx": 299, "type": "dis", "entity": "淋巴样间质性肺炎" }, { "start_idx": 301, "end_idx": 309, "type": "dis", "entity": "肺淋巴样增生综合征" }, { "start_idx": 315, "end_idx": 318, "type": "sym", "entity": "肾脏病变" }, { "start_idx": 324, "end_idx": 327, "type": "dis", "entity": "奴卡菌病" }, { "start_idx": 329, "end_idx": 339, "type": "dis", "entity": "nocardiosis" }, { "start_idx": 346, "end_idx": 354, "type": "sym", "entity": "持续发热1个月以上" }, { "start_idx": 368, "end_idx": 372, "type": "dis", "entity": "弓形虫感染" }, { "start_idx": 360, "end_idx": 372, "type": "sym", "entity": "出生1个月内发生弓形虫感染" }, { "start_idx": 373, "end_idx": 377, "type": "dis", "entity": "播散性水痘" }, { "start_idx": 373, "end_idx": 386, "type": "sym", "entity": "播散性水痘(有并发症的水痘)" }, { "start_idx": 387, "end_idx": 390, "type": "dis", "entity": "细菌感染" }, { "start_idx": 394, "end_idx": 397, "type": "dis", "entity": "脓毒血症" }, { "start_idx": 399, "end_idx": 400, "type": "dis", "entity": "肺炎" }, { "start_idx": 402, "end_idx": 404, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 406, "end_idx": 410, "type": "dis", "entity": "骨关节感染" }, { "start_idx": 412, "end_idx": 415, "type": "dis", "entity": "深部脓肿" }, { "start_idx": 420, "end_idx": 424, "type": "dis", "entity": "念珠菌感染" }, { "start_idx": 427, "end_idx": 428, "type": "bod", "entity": "食管" }, { "start_idx": 430, "end_idx": 431, "type": "bod", "entity": "气管" }, { "start_idx": 433, "end_idx": 435, "type": "bod", "entity": "支气管" }, { "start_idx": 437, "end_idx": 437, "type": "bod", "entity": "肺" }, { "start_idx": 442, "end_idx": 447, "type": "dis", "entity": "深部真菌感染" }, { "start_idx": 454, "end_idx": 454, "type": "bod", "entity": "肺" }, { "start_idx": 456, "end_idx": 457, "type": "bod", "entity": "肺门" }, { "start_idx": 459, "end_idx": 462, "type": "bod", "entity": "颈淋巴结" }, { "start_idx": 473, "end_idx": 478, "type": "dis", "entity": "肺外隐球菌病" }, { "start_idx": 483, "end_idx": 488, "type": "dis", "entity": "隐孢子菌感染" }, { "start_idx": 489, "end_idx": 498, "type": "sym", "entity": "伴持续腹泻1个月以上" }, { "start_idx": 510, "end_idx": 516, "type": "dis", "entity": "巨细胞病毒感染" }, { "start_idx": 520, "end_idx": 520, "type": "bod", "entity": "肝" }, { "start_idx": 522, "end_idx": 522, "type": "bod", "entity": "脾" }, { "start_idx": 524, "end_idx": 526, "type": "bod", "entity": "淋巴结" }, { "start_idx": 536, "end_idx": 537, "type": "dis", "entity": "脑病" }, { "start_idx": 571, "end_idx": 582, "type": "sym", "entity": "发育滞后或倒退,智能倒退" }, { "start_idx": 585, "end_idx": 585, "type": "bod", "entity": "脑" }, { "start_idx": 591, "end_idx": 594, "type": "pro", "entity": "头围测定" }, { "start_idx": 598, "end_idx": 604, "type": "dis", "entity": "后天性小头畸形" }, { "start_idx": 606, "end_idx": 607, "type": "pro", "entity": "CT" }, { "start_idx": 609, "end_idx": 613, "type": "pro", "entity": "MRI检查" }, { "start_idx": 617, "end_idx": 619, "type": "dis", "entity": "脑萎缩" }, { "start_idx": 585, "end_idx": 619, "type": "sym", "entity": "脑发育受损,头围测定证实为后天性小头畸形或CT/MRI检查发现有脑萎缩" }, { "start_idx": 620, "end_idx": 631, "type": "dis", "entity": "后天性系统性运动功能障碍" }, { "start_idx": 648, "end_idx": 649, "type": "dis", "entity": "麻痹" }, { "start_idx": 651, "end_idx": 656, "type": "dis", "entity": "病理性反射征" }, { "start_idx": 658, "end_idx": 665, "type": "sym", "entity": "共济失调步态不稳" }, { "start_idx": 670, "end_idx": 680, "type": "dis", "entity": "单纯疱疹病毒性黏膜溃疡" }, { "start_idx": 701, "end_idx": 711, "type": "dis", "entity": "单纯疱疹病毒性支气管炎" }, { "start_idx": 716, "end_idx": 721, "type": "dis", "entity": "组织胞浆菌病" }, { "start_idx": 724, "end_idx": 724, "type": "bod", "entity": "肺" }, { "start_idx": 726, "end_idx": 727, "type": "bod", "entity": "肺门" }, { "start_idx": 729, "end_idx": 732, "type": "bod", "entity": "颈淋巴结" }, { "start_idx": 743, "end_idx": 750, "type": "dis", "entity": "Kaposi肉瘤" }, { "start_idx": 756, "end_idx": 763, "type": "dis", "entity": "原发性脑内淋巴瘤" }, { "start_idx": 769, "end_idx": 778, "type": "dis", "entity": "Burkitt淋巴瘤" }, { "start_idx": 780, "end_idx": 782, "type": "bod", "entity": "B细胞" }, { "start_idx": 793, "end_idx": 799, "type": "dis", "entity": "大细胞性淋巴瘤" }, { "start_idx": 805, "end_idx": 814, "type": "dis", "entity": "播散性或肺外性结核病" }, { "start_idx": 820, "end_idx": 827, "type": "dis", "entity": "卡氏肺囊虫性肺炎" }, { "start_idx": 829, "end_idx": 831, "type": "dis", "entity": "PCP" }, { "start_idx": 838, "end_idx": 848, "type": "dis", "entity": "进行性多发性白质性脑病" }, { "start_idx": 854, "end_idx": 866, "type": "dis", "entity": "沙门菌属(非伤寒)脓毒血症" }, { "start_idx": 886, "end_idx": 891, "type": "dis", "entity": "脑弓形虫感染" }, { "start_idx": 904, "end_idx": 914, "type": "sym", "entity": "体重连续减轻10%以上" }, { "start_idx": 917, "end_idx": 937, "type": "sym", "entity": "大于1岁者的体重-年龄曲线下降25个百分位" }, { "start_idx": 940, "end_idx": 960, "type": "sym", "entity": "出生1个月后的体重-身高曲线下降5个百分位" }, { "start_idx": 963, "end_idx": 1002, "type": "sym", "entity": "并伴有慢性腹泻(每天至少2次稀便持续1个月以上)或发热1个月以上(持续或间歇性)" } ] }, { "text": "(二)小儿HIV感染分类(表17-17)一旦定类,即使病情好转,也不能降至较轻一类。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "小儿HIV感染" } ] }, { "text": "表17-11小儿HIV感染分类注:如不能确定已有HIV感染,则在上述符合前加“E”,例如EN2(三)小儿AIDS的主要临床征象1.持续性全身淋巴结肿大全身淋巴结肿大病原微生物感染常出现反复腹泻、皮疹、肝脾肿大、口腔鹅口疮以及皮肤黏膜念珠菌病等。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "小儿HIV感染" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "HIV感染" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "小儿AIDS" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "淋巴结" }, { "start_idx": 65, "end_idx": 74, "type": "sym", "entity": "持续性全身淋巴结肿大" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "淋巴结" }, { "start_idx": 75, "end_idx": 81, "type": "sym", "entity": "全身淋巴结肿大" }, { "start_idx": 82, "end_idx": 88, "type": "dis", "entity": "病原微生物感染" }, { "start_idx": 92, "end_idx": 95, "type": "sym", "entity": "反复腹泻" }, { "start_idx": 97, "end_idx": 98, "type": "dis", "entity": "皮疹" }, { "start_idx": 100, "end_idx": 103, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 105, "end_idx": 109, "type": "dis", "entity": "口腔鹅口疮" }, { "start_idx": 112, "end_idx": 119, "type": "dis", "entity": "皮肤黏膜念珠菌病" } ] }, { "text": "随疾病进展可发生严重败血症、细菌性肺炎、脑膜炎、泌尿系感染、蜂窝组织炎、慢性中耳炎、慢性鼻窦炎和卡氏肺囊虫病,各脏器阿米巴病、结核菌感染、EB病毒感染以及李斯忒菌感染等。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "严重败血症" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "泌尿系感染" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "蜂窝组织炎" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "慢性中耳炎" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "慢性鼻窦炎" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "卡氏肺囊虫病" }, { "start_idx": 56, "end_idx": 61, "type": "dis", "entity": "脏器阿米巴病" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "结核菌感染" }, { "start_idx": 69, "end_idx": 74, "type": "dis", "entity": "EB病毒感染" }, { "start_idx": 77, "end_idx": 82, "type": "dis", "entity": "李斯忒菌感染" } ] }, { "text": "反复细菌、病毒、真菌和其他病原微生物感染为小儿AIDS的主要临床表现之一。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "细菌" }, { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "病毒" }, { "start_idx": 8, "end_idx": 9, "type": "mic", "entity": "真菌" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "病原微生物感染" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "小儿AIDS" } ] }, { "text": "最主要的病理所见为慢性肺炎,多为淋巴细胞性间质性肺炎,X线胸部摄片可见网状和结节状浸润影。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "慢性肺炎" }, { "start_idx": 16, "end_idx": 25, "type": "dis", "entity": "淋巴细胞性间质性肺炎" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "X线胸部摄片" }, { "start_idx": 35, "end_idx": 43, "type": "sym", "entity": "网状和结节状浸润影" } ] }, { "text": "已明确有HIV感染的患儿如果出现下列机会感染,应做出AIDS病的推测诊断:①念珠菌性食道炎;②巨细胞病毒性视网膜炎;③卡氏肺囊虫性肺炎;④脑弓形虫病(1月龄以后);⑤弥漫性慢性非典型性分枝杆菌感染。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "HIV感染" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "AIDS病" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "念珠菌性食道炎" }, { "start_idx": 47, "end_idx": 56, "type": "dis", "entity": "巨细胞病毒性视网膜炎" }, { "start_idx": 59, "end_idx": 66, "type": "dis", "entity": "卡氏肺囊虫性肺炎" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "脑弓形虫病" }, { "start_idx": 83, "end_idx": 97, "type": "dis", "entity": "弥漫性慢性非典型性分枝杆菌感染" } ] }, { "text": "具有下列组织学诊断依据的机会感染,则可确诊AIDS:①弥漫性;②弥漫性组织浆菌病;③肺外隐球菌病;④肺外结核病;⑤复发性沙门菌属败血症;⑥弥漫性/持续性单纯疱疹。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "AIDS" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "弥漫性组织浆菌病" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "肺外隐球菌病" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "肺外结核病" }, { "start_idx": 57, "end_idx": 66, "type": "dis", "entity": "复发性沙门菌属败血症" }, { "start_idx": 69, "end_idx": 79, "type": "dis", "entity": "弥漫性/持续性单纯疱疹" } ] }, { "text": "3.中枢神经系统并发症主要指AIDS脑病,小儿AIDS患者发生率较高。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "中枢神经系统并发症" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "AIDS" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "脑病" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "小儿AIDS" } ] }, { "text": "在围生期HIV感染儿中发生率约为23%,其发作常伴免疫缺陷的恶化。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "HIV感染" } ] }, { "text": "最严重的临床经过为亚急性脑病,常于症状出现后数周至数月死亡。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "亚急性脑病" } ] }, { "text": "其组织病理改变主要为脑萎缩。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "脑萎缩" } ] }, { "text": "4.其他并发症状消化系统常见消耗综合征(定义见临床分型)。", "entities": [ { "start_idx": 8, "end_idx": 18, "type": "dis", "entity": "消化系统常见消耗综合征" } ] }, { "text": "有资料表明,约0.5%的小儿AIDS发生恶性肿瘤,常见肿瘤类型为非霍奇金淋巴瘤、Kaposi肉瘤、B淋巴细胞性白血病以及肝母细胞瘤等。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "小儿AIDS" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "肿瘤" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "非霍奇金淋巴瘤" }, { "start_idx": 40, "end_idx": 47, "type": "dis", "entity": "Kaposi肉瘤" }, { "start_idx": 49, "end_idx": 57, "type": "dis", "entity": "B淋巴细胞性白血病" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "肝母细胞瘤" } ] }, { "text": "小儿AIDS的心血管并发症近年已引起重视。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "小儿AIDS" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "心血管并发症" } ] }, { "text": "伴随CD4+T淋巴细胞减少,可见渐进性左室功能障碍。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "T淋巴细胞" } ] }, { "text": "HIV感染儿童的血液系统异常常表现为白细胞减少贫血和血小板减少特异性抗体检测可用于HIV感染的流行病学调查和现症患者的诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "HIV感染" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "血液系统" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "白细胞" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "白细胞减少" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "贫血" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "血小板" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "血小板减少" }, { "start_idx": 31, "end_idx": 37, "type": "pro", "entity": "特异性抗体检测" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "HIV感染" } ] }, { "text": "但由于早期感染血清抗体出现较迟,一般在感染后22~27天才能检出,因此抗体阴性不能排除HIV早期感染,应在2~4周后复查。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "血清抗体" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "抗体" }, { "start_idx": 43, "end_idx": 45, "type": "mic", "entity": "HIV" } ] }, { "text": "应注意,18个月以下婴儿可存在来自母体的被动抗体。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "抗体" } ] }, { "text": "(1)筛选试验:采用ELISA或免疫荧光试验检查血清HIV抗体,阳性者应做验证试验,以排除假阳性反应。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "筛选试验" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "ELISA" }, { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "免疫荧光试验检查" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "血清HIV抗体" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "验证试验" } ] }, { "text": "(2)验证试验:用免疫印迹试验检查血清抗gp120、抗gp41和抗gp24。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "验证试验" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "免疫印迹试验" }, { "start_idx": 17, "end_idx": 24, "type": "bod", "entity": "血清抗gp120" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "抗gp41" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "抗gp24" } ] }, { "text": "阳性可以确立HIV感染的诊断。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "HIV感染" } ] }, { "text": "2.抗原检查检查血清中p24抗原,其出现早于血清抗体,因此可用于早期诊断。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "抗原检查" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "血清" }, { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "p24抗原" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "血清抗体" } ] }, { "text": "3.HIV核酸检查用PCR技术检测血清中HIVRNA,其阳性结果常较p24抗原检测早3~5天,较抗体~3周,并可定量检测。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "HIV核酸检查" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "血清" }, { "start_idx": 34, "end_idx": 38, "type": "mic", "entity": "p24抗原" }, { "start_idx": 48, "end_idx": 48, "type": "ite", "entity": "抗" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "抗" } ] }, { "text": "所以是早期诊断、判断预后和抗病毒药物疗效的理想指标。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "抗病毒药物" } ] }, { "text": "用原位杂交技术可以检查组织或细胞内的HIV核酸。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "组织" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "细胞" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "HIV核酸" } ] }, { "text": "1.血细胞检查包括白细胞、血小板及红细胞计数减少淋巴细胞检查T淋巴细胞亚群计数:正常CD4+T细胞/CD8+T细胞比值为1.5~2.0,AIDS时低于1.0。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "血细胞检查" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "白细胞" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "血小板" }, { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "红细胞计数" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "红细胞计数减少" }, { "start_idx": 24, "end_idx": 29, "type": "pro", "entity": "淋巴细胞检查" }, { "start_idx": 30, "end_idx": 38, "type": "ite", "entity": "T淋巴细胞亚群计数" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "AIDS" } ] }, { "text": "此外,CD4+T细胞绝对计数有助于疾病的分期和判断疗效。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "ite", "entity": "T细胞绝对计数" } ] }, { "text": "3.皮肤迟发性变态反应试验HIV感染者低下或阴性。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "皮肤迟发性变态反应试验" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "HIV感染" } ] }, { "text": "4.免疫球蛋白、补体、免疫复合物及自身抗体等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "补体" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "自身抗体" } ] }, { "text": "(三)免疫状况分类各年龄小儿CD4+T淋巴细胞正常值存在差异,在判别T淋巴细胞受抑制时应注意年龄特点。", "entities": [ { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "T淋巴细胞" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "T淋巴细胞" } ] }, { "text": "美国疾病控制中心(CDC)以周围血中CD4+T淋巴细胞绝对值或CD4+T淋巴细胞占淋巴细胞总数的百分率来表示病人的免疫状况,做以下分类(表17-17)。", "entities": [ { "start_idx": 28, "end_idx": 35, "type": "ite", "entity": "T淋巴细胞绝对值" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "T淋巴细胞" }, { "start_idx": 53, "end_idx": 58, "type": "ite", "entity": "淋巴细胞总数" } ] }, { "text": "表17-12基于CD4+T淋巴细胞计数和年龄特点的免疫状况分类【诊断】HIV感染母亲所生婴儿HIV感染的诊断原则:1.≥18个月婴儿的确定诊断具备ELISA检测抗体2次阳性和证实试验(免疫印迹或免疫荧光检测)1次阳性;或在不同样本任何2项病毒检测试验(HIV分离、HIV基因和p24抗原测定)阳性;或存在一项儿科AIDS定义疾病(见临床分型)。", "entities": [ { "start_idx": 8, "end_idx": 18, "type": "ite", "entity": "CD4+T淋巴细胞计数" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "HIV感染" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "HIV感染" }, { "start_idx": 73, "end_idx": 79, "type": "pro", "entity": "ELISA检测" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "抗体" }, { "start_idx": 87, "end_idx": 90, "type": "pro", "entity": "证实试验" }, { "start_idx": 92, "end_idx": 95, "type": "pro", "entity": "免疫印迹" }, { "start_idx": 97, "end_idx": 102, "type": "pro", "entity": "免疫荧光检测" }, { "start_idx": 119, "end_idx": 124, "type": "pro", "entity": "病毒检测试验" }, { "start_idx": 126, "end_idx": 130, "type": "pro", "entity": "HIV分离" }, { "start_idx": 132, "end_idx": 136, "type": "pro", "entity": "HIV基因" }, { "start_idx": 138, "end_idx": 144, "type": "pro", "entity": "p24抗原测定" }, { "start_idx": 156, "end_idx": 159, "type": "dis", "entity": "AIDS" } ] }, { "text": "≥18个月婴儿的推测诊断:具备一项病毒检测试验(同上)阳性(除外脐血)。", "entities": [ { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "病毒检测试验" } ] }, { "text": "2.<18个月婴儿的确定诊断具备在不同样本任何2项病毒检测试验(同上)阳性;或存在一项儿科AIDS定义疾病。", "entities": [ { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "病毒检测试验" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "AIDS" } ] }, { "text": "3.除外先天性免疫缺陷病。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "先天性免疫缺陷病" } ] }, { "text": "【治疗】HIV感染与艾滋病的临床表现复杂,迄今尚无特效疗法,疫苗仍处在研制阶段,故临床治疗甚为困难。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "HIV感染" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "艾滋病" } ] }, { "text": "目前治疗包括抗病毒治疗、提高免疫功能治疗、抗感染治疗和抗肿瘤治疗等。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "抗病毒治疗" }, { "start_idx": 12, "end_idx": 19, "type": "pro", "entity": "提高免疫功能治疗" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "抗感染治疗" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "抗肿瘤治疗" } ] }, { "text": "(一)抗病毒治疗1.齐多夫定(zidovudine,AZT)为核苷反转录酶抑制剂。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "抗病毒治疗" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "齐多夫定" }, { "start_idx": 15, "end_idx": 24, "type": "dru", "entity": "zidovudine" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "AZT" }, { "start_idx": 31, "end_idx": 39, "type": "dru", "entity": "核苷反转录酶抑制剂" } ] }, { "text": "新生儿2mg/kg口服,1.5mg/kg静脉注射,每6小时1次。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "静脉注射" } ] }, { "text": "儿童90~180mg/m2口服,每6~8小时1次(与其他抗病毒药物合用时可为180mg/m2,每12小时1次);或120mg/m2间隔性静脉注射,每6小时1次;或每小时20mg/m2持续静脉滴注。", "entities": [ { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "抗病毒药物" }, { "start_idx": 86, "end_idx": 89, "type": "pro", "entity": "静脉注射" }, { "start_idx": 117, "end_idx": 120, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "AZT常见的不良反应为血液学毒性,包括粒细胞减少症及贫血,常有头痛。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "AZT" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "粒细胞减少症" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "贫血" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "头痛" } ] }, { "text": "不常见的副作用为肌病、肌炎和肝毒性。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肌病" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "肌炎" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肝毒性" } ] }, { "text": "肾功能减退者,应减少剂量;严重而持久的粒细胞减少症和贫血时,应暂时停药,待骨髓功能恢复再重新开始给药。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "肾功能" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "粒细胞减少症" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "贫血" }, { "start_idx": 37, "end_idx": 40, "type": "ite", "entity": "骨髓功能" } ] }, { "text": "也可减少AZT剂量和合并使用红细胞生成素。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "AZT" }, { "start_idx": 14, "end_idx": 19, "type": "dru", "entity": "红细胞生成素" } ] }, { "text": "2.Nevirapine(NVP)为非核苷酸转录酶抑制剂。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dru", "entity": "Nevirapine" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "NVP" }, { "start_idx": 18, "end_idx": 27, "type": "dru", "entity": "非核苷酸转录酶抑制剂" } ] }, { "text": "NVP的主要不良反应是皮疹,可发展为危及生命的大疱性渗出性红斑,应即刻停药。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "NVP" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "皮疹" }, { "start_idx": 23, "end_idx": 30, "type": "dis", "entity": "大疱性渗出性红斑" } ] }, { "text": "其他不良反应有嗜睡、头痛、腹泻和恶心。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "嗜睡" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "头痛" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "腹泻" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "恶心" } ] }, { "text": "偶尔发生肝炎和肝功能损害NVP诱导肝脏细胞色素P4503A(CYP3A),能与多种药物发生相互反应,应予以重视。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "肝炎" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "肝功能" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "偶尔发生肝炎和肝功能损害" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "NVP" }, { "start_idx": 17, "end_idx": 28, "type": "bod", "entity": "肝脏细胞色素P4503A" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "CYP3A" } ] }, { "text": "这些药物包括利福平和利福布丁,口服避孕药、安眠药、口服抗凝剂、地高辛、苯妥英钠和茶碱。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "利福平" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "利福布丁" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "安眠药" }, { "start_idx": 25, "end_idx": 29, "type": "dru", "entity": "口服抗凝剂" } ] }, { "text": "常见不良反应为恶心、呕吐、腹泻、厌食和腹痛肝酶增高甘油三酯和胆固醇增高高血糖,以及酮症酸中毒等。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "恶心" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "呕吐" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "腹泻" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "厌食" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "腹" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "腹痛" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肝酶" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "肝酶增高" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "甘油三酯" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "胆固醇" }, { "start_idx": 25, "end_idx": 34, "type": "sym", "entity": "甘油三酯和胆固醇增高" }, { "start_idx": 35, "end_idx": 37, "type": "sym", "entity": "高血糖" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "酮症酸中毒" } ] }, { "text": "该药的代谢主要受肝脏细胞色素P450A3(CYP3A)的影响,不能与抗组胺类,阿普唑仑、咪达唑仑和三唑仑等安眠药,钙离子通道阻断剂硝苯地平,麦角碱衍生物、苯丙胺、西沙必利、华法林、利福平类和某些抗精神病药物合用。", "entities": [ { "start_idx": 8, "end_idx": 19, "type": "bod", "entity": "肝脏细胞色素P450A3" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "CYP3A" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "抗组胺类" }, { "start_idx": 39, "end_idx": 42, "type": "dru", "entity": "阿普唑仑" }, { "start_idx": 44, "end_idx": 47, "type": "dru", "entity": "咪达唑仑" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "三唑仑" }, { "start_idx": 53, "end_idx": 55, "type": "dru", "entity": "安眠药" }, { "start_idx": 57, "end_idx": 68, "type": "dru", "entity": "钙离子通道阻断剂硝苯地平" }, { "start_idx": 70, "end_idx": 75, "type": "dru", "entity": "麦角碱衍生物" }, { "start_idx": 77, "end_idx": 79, "type": "dru", "entity": "苯丙胺" }, { "start_idx": 81, "end_idx": 84, "type": "dru", "entity": "西沙必利" }, { "start_idx": 86, "end_idx": 88, "type": "dru", "entity": "华法林" }, { "start_idx": 90, "end_idx": 93, "type": "dru", "entity": "利福平类" }, { "start_idx": 97, "end_idx": 102, "type": "dru", "entity": "抗精神病药物" } ] }, { "text": "为减少胃肠道反应,开始剂量宜小,在5天内逐渐增加到足量。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "胃肠道" } ] }, { "text": "抗病毒治疗的指征:①有HIV感染的临床症状,包括临床表现期A、B或C;②CD4+T淋巴细胞绝对数或百分率下降,达到中度或严重免疫抑制;③年龄在1岁以内的患儿,无论其临床免疫学和病毒负荷状况;④年龄大于1岁患儿无临床症状者,应严密观察未开始治疗的病例其临床、免疫学和病毒负荷状况,一旦发现以下情况即开始治疗:HIVRVA复制物数量极高或进行性增高;CD4+T淋巴细胞绝对数或百分率很快下降,达到中度免疫抑制,出现临床症状。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "抗病毒治疗" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "HIV感染" }, { "start_idx": 46, "end_idx": 53, "type": "ite", "entity": "T淋巴细胞绝对数" }, { "start_idx": 94, "end_idx": 95, "type": "mic", "entity": "病毒" }, { "start_idx": 138, "end_idx": 139, "type": "mic", "entity": "病毒" }, { "start_idx": 159, "end_idx": 167, "type": "dru", "entity": "HIVRVA复制物" }, { "start_idx": 189, "end_idx": 196, "type": "ite", "entity": "T淋巴细胞绝对数" } ] }, { "text": "(二)免疫学治疗1.IL-2每日300万~1800万U,静脉或皮下注射,5天为一疗程;休息至少8周后开始下一疗程。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "免疫学治疗" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "IL-2" } ] }, { "text": "应监测血浆病毒负荷,控制在50复制物/μl以下。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "病毒" } ] }, { "text": "2.IL-12IL-12是另一个有治疗价值的细胞因子,体外实验表明IL-12能增强免疫细胞杀伤被HIV感染细胞的能力。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "IL-12" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "IL-12" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "细胞因子" }, { "start_idx": 33, "end_idx": 37, "type": "pro", "entity": "IL-12" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "免疫细胞" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "HIV感染细胞" } ] }, { "text": "(三)支持治疗静脉注射丙种球蛋白(IVIG)可减少AIDS患儿合并细菌感染的发生率和缩短住院时间。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "支持治疗" }, { "start_idx": 7, "end_idx": 15, "type": "pro", "entity": "静脉注射丙种球蛋白" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "IVIG" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "AIDS" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "细菌感染" } ] }, { "text": "IVIG对预防弓形体感染无效,也不能延长患儿的生命。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "IVIG" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "弓形体感染" } ] }, { "text": "每月定期使用IVIG的指征为低丙种球蛋白血症,抗体反应低下和适当的抗微生物不能控制的反复感染。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "IVIG" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "低丙种球蛋白血症" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "抗体" } ] }, { "text": "(四)抗感染治疗AIDS患儿由于免疫功能低下,极易病原微生物感染及机会感染。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "抗感染治疗" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "AIDS" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "病原微生物感染" } ] }, { "text": "应根据临床病原的种类,积极进行抗感染治疗及必要的预防治疗。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "抗感染治疗" } ] }, { "text": "如针对肺炎、败血症和脑膜炎等细菌感染疾病,选用敏感抗生素治疗;针对结核病进行抗结核治疗,治疗时间应适当延长,不得少于12个月。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肺炎" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "败血症" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "细菌感染疾病" }, { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "结核病" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "抗结核治疗" } ] }, { "text": "有结核接触史或PPD皮肤试验强阳性的HIV感染儿童,应用异烟肼预防性治疗9~12个月。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "PPD皮肤试验" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "HIV感染" }, { "start_idx": 28, "end_idx": 35, "type": "pro", "entity": "异烟肼预防性治疗" } ] }, { "text": "卡氏肺囊虫肺炎是AIDS常见的机会感染,如果CD4+T细胞计数1~5岁<500/μl或6~12岁<200/μl,并有临床表现者,应及时预防,常用TMP/SMZ每天150mg/m2,分2次口服,连服3天停药4天。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "卡氏肺囊虫肺炎" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "AIDS" }, { "start_idx": 32, "end_idx": 36, "type": "ite", "entity": "T细胞计数" } ] }, { "text": "如临床有病毒、真菌及弓形虫等感染的表现,应选用相应的药物治疗。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "mic", "entity": "病毒" }, { "start_idx": 7, "end_idx": 8, "type": "mic", "entity": "真菌" }, { "start_idx": 10, "end_idx": 12, "type": "mic", "entity": "弓形虫" } ] }, { "text": "对AIDS的治疗,目前主张联合用药。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "AIDS" } ] }, { "text": "包括抗病毒治疗+免疫学治疗联合用药以及不同类型抗病毒药物间联合用药。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "抗病毒治疗" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "免疫学治疗" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "抗病毒药物" } ] }, { "text": "如AZT+NVP+IL-2联合用药治疗。", "entities": [ { "start_idx": 1, "end_idx": 18, "type": "pro", "entity": "AZT+NVP+IL-2联合用药治疗" } ] }, { "text": "【预防】预防小儿艾滋病的关键是预防育龄妇女感染HIV和筛查献血员。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "小儿艾滋病" }, { "start_idx": 23, "end_idx": 25, "type": "mic", "entity": "HIV" } ] }, { "text": "母亲如果是AIDS病人或HIV感染者,应采取严格措施,以防传给后代,因为在怀孕、分娩和哺乳期间均可使小儿感染。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "AIDS" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "HIV感染" } ] }, { "text": "严禁进口与使用污染AIDS病毒的血液制品。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "mic", "entity": "AIDS病毒" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "血液" } ] }, { "text": "应广泛宣传AIDS的流行情况及其严重性。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "AIDS" } ] }, { "text": "【预后】小儿AIDS预后极为恶劣,约75%垂直感染的AIDS患儿在发病后1年内死亡。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "小儿AIDS" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "AIDS" } ] }, { "text": "近年新的诊断技术的临床应用和抑制HIV复制的新型药物的研制开发,正为预防和控制人类的这种灾难性疾病带来曙光。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "pro", "entity": "抑制HIV复制" } ] }, { "text": "二、遗传病分类基因组的内部结构及复杂的功能使其对损伤极为敏感,而导致疾病。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "遗传病" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "基因组" } ] }, { "text": "遗传性疾病的程度根据基因突变的不同类型有很大的区别,可从丢失或多一条染色体到改变一个基因的碱基对,其后果亦表现多种多样,有些根本不能存活而流产,有些产生特异的临床综合征,有些不出现临床症状。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "基因" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "染色体" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "基因" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "碱基对" } ] }, { "text": "根据Mckusick的统计,到1966年总共描述了148种遗传病,1986年为3907种,随着分子克隆和人类基因组计划的进展,2001年已经报道的遗传病达到13000多种,2010年达到20000余种。", "entities": [ { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "基因组" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "遗传病" } ] }, { "text": "儿科领域的遗传病种类繁多,机制复杂,研究又细分为细胞遗传学、生化遗传学、免疫遗传学及药物遗传学等。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "遗传病" } ] }, { "text": "遗传因素造成的心血管、呼吸、消化、肾脏、神经、血液、骨骼、结缔组织、皮肤及五官等多系统器官遗传病及先天畸形均可在新生儿时期发现。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "心血管" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "呼吸" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "消化" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肾脏" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "神经" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "血液" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "骨骼" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "结缔组织" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "皮肤" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "五官" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "先天畸形" } ] }, { "text": "根据遗传物质的结构和功能改变的不同,可将遗传病分为五类:1.染色体病(chromosomaldisorders)指染色体数目异常,或者染色体结构异常,包括缺失、易位、倒位、环形染色体和等臂染色体等,造成许多基因物质的得失而引起疾病,已经明确的染色体畸变综合征有数百种。", "entities": [ { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "染色体病" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "染色体" }, { "start_idx": 57, "end_idx": 63, "type": "sym", "entity": "染色体数目异常" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "染色体" }, { "start_idx": 67, "end_idx": 73, "type": "sym", "entity": "染色体结构异常" }, { "start_idx": 77, "end_idx": 78, "type": "sym", "entity": "缺失" }, { "start_idx": 80, "end_idx": 81, "type": "sym", "entity": "易位" }, { "start_idx": 83, "end_idx": 84, "type": "sym", "entity": "倒位" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "环形染色体" }, { "start_idx": 92, "end_idx": 96, "type": "bod", "entity": "等臂染色体" }, { "start_idx": 103, "end_idx": 104, "type": "bod", "entity": "基因" }, { "start_idx": 121, "end_idx": 128, "type": "dis", "entity": "染色体畸变综合征" } ] }, { "text": "2.单基因遗传病(singlegenediseases)单基因病是指由单个基因突变所致的遗传病,每种单基因病均源自相关基因的突变,此类疾病目前报道已达数千余种,但每种疾病的发病率非常低。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "单基因遗传病" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "单基因" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "基因" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "遗传病" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "单基因" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "基因" } ] }, { "text": "在一对基因中只要有一个致病基因存在就能表现性状的称显性基因,一对基因需2个基因同时存在病变时才能表现性状的称隐性基因。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "基因" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "基因" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "基因" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "基因" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "基因" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "隐性基因" } ] }, { "text": "单基因遗传病按不同遗传模式分为以下5类遗传方式(表14-14)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "单基因遗传病" } ] }, { "text": "表14-1常见单基因遗传病与基因缺陷关系表续表(1)常染色体显性遗传(autosomaldominantinheritance):致病基因在常染色体上,亲代只要有一个显性致病基因传递给子代,子代就会表现性状。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "单基因遗传病" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "基因缺陷" }, { "start_idx": 26, "end_idx": 33, "type": "dis", "entity": "常染色体显性遗传" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "基因" }, { "start_idx": 70, "end_idx": 73, "type": "bod", "entity": "常染色体" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "基因" } ] }, { "text": "例如软骨发育不全及成骨不全。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "软骨发育不全" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "成骨不全" } ] }, { "text": "但是,有时由于疾病外显率的不同,可表现为完全显性、不完全显性及延迟显性(杂合子Aa在生命早期显性基因并不表达,待一定年龄后才显达,如遗传性舞蹈病等)等。", "entities": [ { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "基因" }, { "start_idx": 66, "end_idx": 71, "type": "dis", "entity": "遗传性舞蹈病" } ] }, { "text": "(2)常染色体隐性遗传(autosomalrecessiveinheritance):致病基因在常染色体上,为一对隐性基因。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "常染色体隐性遗传" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "致病基因" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "常染色体" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "隐性基因" } ] }, { "text": "只带1个致病隐性基因的个体不发病,为致病基因携带者,只有致病纯合子才致病。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "致病隐性基因" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "致病基因" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "致病纯合子" } ] }, { "text": "多数遗传代谢病为常染色体隐性遗传,如苯丙酮尿症及白化病等。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "常染色体隐性遗传" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "苯丙酮尿症" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "白化病" } ] }, { "text": "(3)X连锁隐性遗传(X-linkedrecessiveinheritance):定位于X染色体上的致病基因随X染色体而传递疾病。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "X连锁隐性遗传" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "X染色体" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "致病基因" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "X染色体" } ] }, { "text": "女性带有一个隐性致病基因,为表型正常的致病基因携带者。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "隐性致病基因" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "致病基因" } ] }, { "text": "男性只有一条X染色体,即使是隐性基因,也会发病,如血友病及进行性肌营养不良等。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "X染色体" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "隐性基因" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "血友病" }, { "start_idx": 29, "end_idx": 36, "type": "dis", "entity": "进行性肌营养不良" } ] }, { "text": "(4)X连锁显性遗传(X-linkeddominantinheritance):X连锁显性遗传致病基因在X染色体上。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "X连锁显性遗传" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "X连锁显性遗传" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "致病基因" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "染色体" } ] }, { "text": "女性患者病情较轻,如抗维生素D佝偻病。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "抗维生素D佝偻病" } ] }, { "text": "(5)Y连锁遗传(Y-linkedinheritance):Y连锁遗传致病基因位于Y染色体上,只有男性出现症状,由父传子,例如性别决定基因(SRY基因)突变所致的性反转等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "Y连锁遗传" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "Y连锁遗传" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "致病基因" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "Y染色体" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "基因" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "SRY基因" } ] }, { "text": "3.多基因遗传病(multifactorialdiseases)疾病由多对异常基因及环境因素共同作用。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "多基因遗传病" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "异常基因" } ] }, { "text": "每对基因作用微小,但有积累效应,致使超出阈值而发病。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "基因" } ] }, { "text": "这些微效基因的总和加上环境因素的影响,就决定了个体的疾病性状。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "微效基因" } ] }, { "text": "例如2型糖尿病、高血压、神经管缺陷及兔唇等都属多基因遗传病。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "2型糖尿病" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "高血压" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "神经管缺陷" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "兔唇" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "多基因遗传病" } ] }, { "text": "4.线粒体病(mitochondrialdiseases)人类细胞中有一部分DNA存在于胞浆内,称为线粒体DNA,按母系遗传。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "线粒体病" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "DNA" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "胞浆" }, { "start_idx": 50, "end_idx": 55, "type": "bod", "entity": "线粒体DNA" } ] }, { "text": "基因突变为一组较为独特的遗传病,目前已发现60余种疾病与线粒体基因突变或线粒体结构异常有关,例如脂肪酸氧化障碍、呼吸链酶缺陷及特殊类型的糖尿病等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "基因" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "线粒体" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "线粒体" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "脂肪酸氧化障碍" }, { "start_idx": 56, "end_idx": 61, "type": "dis", "entity": "呼吸链酶缺陷" }, { "start_idx": 63, "end_idx": 70, "type": "dis", "entity": "特殊类型的糖尿病" } ] }, { "text": "5.基因组印记(genomicimprinting)基因根据来源亲代的不同而有不同的表达,控制某一表型的一对等位基因因亲源不同而呈差异性表达,即两条等位基因如皆来自父源或母源则有不同的表现形式。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "基因组印记" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "基因" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "等位基因" }, { "start_idx": 65, "end_idx": 69, "type": "sym", "entity": "差异性表达" }, { "start_idx": 74, "end_idx": 77, "type": "bod", "entity": "等位基因" } ] }, { "text": "例如,Prader-Willi综合征和Angelman综合征都是15q11-11缺失,Prader-Willi综合征是父源性15q11-11缺失(母源单亲二体),Angelman综合征为母源性15q11-11缺失(父源单亲二体)。", "entities": [ { "start_idx": 3, "end_idx": 17, "type": "dis", "entity": "Prader-Willi综合征" }, { "start_idx": 19, "end_idx": 29, "type": "dis", "entity": "Angelman综合征" }, { "start_idx": 32, "end_idx": 41, "type": "sym", "entity": "15q11-11缺失" }, { "start_idx": 43, "end_idx": 57, "type": "dis", "entity": "Prader-Willi综合征" }, { "start_idx": 59, "end_idx": 71, "type": "sym", "entity": "父源性15q11-11缺失" }, { "start_idx": 81, "end_idx": 91, "type": "dis", "entity": "Angelman综合征" }, { "start_idx": 93, "end_idx": 105, "type": "sym", "entity": "母源性15q11-11缺失" } ] }, { "text": "基因组印记还影响某些遗传病的表现度及外显率等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "基因组印记" } ] }, { "text": "参考文献1.方鹤松,段恕诚,于卫力,等.中国腹泻病诊断治疗方案.临床儿科杂志,1992,10(4):2392.姜天安,叶瑞云,叶华英.慢性腹泻患儿肠黏液超微结构观察.中华医学杂志,1991,71:683.姜天安,叶瑞云.对腹泻患儿的小肠黏液形态、乳糖酶及乳糖水解率的研究.中华医学杂志,1987,69:4994.姜天安,叶瑞云.婴幼儿慢性腹泻时的肠道营养治疗.中华儿科杂志,1992,30:45.江米足,叶瑞云.婴幼儿腹泻时空肠黏液刷状缘肽酶和蛋白质代谢的研究.中华儿科杂志,1996,34:326.刘湘云.急性腹泻病理生理及药物治疗研究进展.中华儿科杂志,1989,22(3):1827.BranskiD,LernerA,LebenthalE.Chronicdiarrheaandmalabsorption.PediatrClinNorthAm,1996,43:3078.CormierDaireV,BonnefontJP,RustinP,etal.MitochondrialDNArearrangementswithonsetaschronicdiarrheawithvillousatrophy.JPediatr,1994,124:639.DeBoissieuC,ChaussainM,BadoualJ,etal.Smallbowelbacterialovergrowthinchildrenwithchronicdiarrhea,abdominalpain,orboth.JPediatr,1996,128:20310.KneepkensCM,HoekstraJH.Chronicnonspecificdiarrheaofchildhood:pathophysiologyandmanagement.PediatrClinNorthAm,1996,43:375", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "dru", "entity": "腹泻" }, { "start_idx": 67, "end_idx": 70, "type": "dru", "entity": "慢性腹泻" }, { "start_idx": 111, "end_idx": 112, "type": "dis", "entity": "腹泻" }, { "start_idx": 116, "end_idx": 119, "type": "bod", "entity": "小肠黏液" }, { "start_idx": 123, "end_idx": 125, "type": "bod", "entity": "乳糖酶" }, { "start_idx": 127, "end_idx": 131, "type": "ite", "entity": "乳糖水解率" }, { "start_idx": 167, "end_idx": 170, "type": "dis", "entity": "慢性腹泻" }, { "start_idx": 173, "end_idx": 178, "type": "pro", "entity": "肠道营养治疗" }, { "start_idx": 209, "end_idx": 210, "type": "dis", "entity": "腹泻" }, { "start_idx": 212, "end_idx": 220, "type": "bod", "entity": "空肠黏液刷状缘肽酶" }, { "start_idx": 222, "end_idx": 224, "type": "bod", "entity": "蛋白质" }, { "start_idx": 254, "end_idx": 257, "type": "dis", "entity": "急性腹泻" } ] }, { "text": "第二节软组织肉瘤软组织肉瘤(softpartsarcoma)是发生在结缔组织的恶性肿瘤,包括皮下组织、肌肉、肌腱、血管、结缔组织间隙以及空腔器官支柱基质等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "软组织肉瘤" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "软组织肉瘤" }, { "start_idx": 14, "end_idx": 28, "type": "dis", "entity": "softpartsarcoma" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "结缔组织" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "皮下组织" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "肌肉" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "肌腱" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "血管" }, { "start_idx": 60, "end_idx": 65, "type": "bod", "entity": "结缔组织间隙" }, { "start_idx": 68, "end_idx": 75, "type": "bod", "entity": "空腔器官支柱基质" } ] }, { "text": "但发生在骨骼、网状内皮系统、神经胶质等部位的除外。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "骨骼" }, { "start_idx": 7, "end_idx": 12, "type": "bod", "entity": "网状内皮系统" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "神经胶质" } ] }, { "text": "软组织肉瘤的细胞起源为原始间叶干细胞,位于非节段性中胚层,故可生长在身体不同部位。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "软组织肉瘤" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "间叶干细胞" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "中胚层" } ] }, { "text": "一、纤维肉瘤纤维肉瘤(fibrosarcoma)起源于成纤维细胞,是纤维组织的恶性肿瘤,多见于5岁以下,有些患儿出生时即发现,也称“先天性及婴儿纤维肉瘤”,男孩略多于女孩。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "纤维肉瘤" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "纤维肉瘤" }, { "start_idx": 11, "end_idx": 22, "type": "dis", "entity": "fibrosarcoma" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "成纤维细胞" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "纤维组织" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 66, "end_idx": 75, "type": "dis", "entity": "先天性及婴儿纤维肉瘤" } ] }, { "text": "肿块圆形或椭圆形切面灰白或黄白,大小不一,小的肿瘤大的分界不清,往往浸润组织。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肿块" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "肿块圆形或椭圆形" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "切面灰白或黄白" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "大小不一" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "肿瘤" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "小的肿瘤" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "大的分界不清" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "往往浸润组织" } ] }, { "text": "光镜下肿瘤由呈囊状交织排列的梭形成纤维细胞组成胞质少各束细胞间由胶原纤维分开组织学形态比较一致。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "光镜" }, { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肿瘤" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "纤维细胞" }, { "start_idx": 0, "end_idx": 22, "type": "sym", "entity": "光镜下肿瘤由呈囊状交织排列的梭形成纤维细胞组成" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "胞质" }, { "start_idx": 23, "end_idx": 25, "type": "sym", "entity": "胞质少" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "胶原纤维" }, { "start_idx": 26, "end_idx": 37, "type": "sym", "entity": "各束细胞间由胶原纤维分开" }, { "start_idx": 38, "end_idx": 46, "type": "sym", "entity": "组织学形态比较一致" } ] }, { "text": "纤维肉瘤可发生于任何部位,下肢最多见约占50%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "纤维肉瘤" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "下肢" } ] }, { "text": "烧伤瘢痕、视网膜母细胞瘤、霍奇金淋巴瘤放疗后易生长本瘤。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "视网膜母细胞瘤" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "霍奇金淋巴瘤" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "放疗" } ] }, { "text": "肿瘤为生长迅速的无痛性肿块,可在2~3周内增长一倍,其边界常不清晰,当压迫神经如腓总神经或其分支时,可产生压迫症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肿块" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "神经" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "腓总神经" } ] }, { "text": "有同一肢体几个分开较远的肿瘤同时存在的报道。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肿瘤" } ] }, { "text": "X线检查,可见软组织块影或肢体长骨骨皮质增厚极少数可出现骨质破坏肺转移。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "X线检查" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "软组织" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "骨皮质" }, { "start_idx": 5, "end_idx": 21, "type": "sym", "entity": "可见软组织块影或肢体长骨骨皮质增厚" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "骨质" }, { "start_idx": 22, "end_idx": 31, "type": "sym", "entity": "极少数可出现骨质破坏" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "肺" } ] }, { "text": "切除不彻底可致复发,需再行手术,必要时考虑截肢。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "切除" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "手术" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "截肢" } ] }, { "text": "纤维肉瘤对放射敏感性低,故有主张大剂量放疗的(6Gy),特别是在多次复发或避免截肢时。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "纤维肉瘤" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "放射" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "放疗" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "截肢" } ] }, { "text": "化疗的意见不一致,有人主张按横纹肌肉瘤方案,用在复发和转移病例,也有人主张术前放疗和化疗。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 37, "end_idx": 37, "type": "pro", "entity": "术" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "放疗" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "化疗" } ] }, { "text": "纤维肉瘤的预后较好,5年治愈率可达85%左右。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "纤维肉瘤" } ] }, { "text": "五、免疫抑制剂的应用目前,在儿童肾移植中使用的免疫抑制剂包括:糖皮质激素(Pred)、硫唑嘌呤(Aza)、环孢霉素(CsA)、霉酚酸酯(MMF)、瑞帕霉素(sirolimusRapamycin,SRL)、他克莫司FK506(tacrolimus)、抗胸腺球蛋白(ATG)以及单克隆CD3抗体(OKT3)等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 31, "end_idx": 35, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 37, "end_idx": 40, "type": "dru", "entity": "Pred" }, { "start_idx": 43, "end_idx": 46, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "Aza" }, { "start_idx": 53, "end_idx": 56, "type": "dru", "entity": "环孢霉素" }, { "start_idx": 58, "end_idx": 60, "type": "dru", "entity": "CsA" }, { "start_idx": 63, "end_idx": 66, "type": "dru", "entity": "霉酚酸酯" }, { "start_idx": 68, "end_idx": 70, "type": "dru", "entity": "MMF" }, { "start_idx": 73, "end_idx": 76, "type": "dru", "entity": "瑞帕霉素" }, { "start_idx": 78, "end_idx": 95, "type": "dru", "entity": "sirolimusRapamycin" }, { "start_idx": 97, "end_idx": 99, "type": "dru", "entity": "SRL" }, { "start_idx": 102, "end_idx": 110, "type": "dru", "entity": "他克莫司FK506" }, { "start_idx": 112, "end_idx": 121, "type": "dru", "entity": "tacrolimus" }, { "start_idx": 124, "end_idx": 129, "type": "dru", "entity": "抗胸腺球蛋白" }, { "start_idx": 131, "end_idx": 133, "type": "dru", "entity": "ATG" }, { "start_idx": 137, "end_idx": 144, "type": "dru", "entity": "单克隆CD3抗体" }, { "start_idx": 146, "end_idx": 149, "type": "dru", "entity": "OKT3" } ] }, { "text": "儿童由于其生理、代谢特点,应用免疫抑制剂有独特性:①儿童的免疫防御较成人强,儿童肾移植术较成人更易发生急性排斥反应;②糖皮质激素用量较高,将抑制儿童的生长发育;③儿童对CsA代谢较成人快,难以达到稳定理想的CsA浓度,要兼顾移植后排斥反应及CsA肾毒性两方面的效应。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 38, "end_idx": 43, "type": "pro", "entity": "儿童肾移植术" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "急性排斥" }, { "start_idx": 59, "end_idx": 63, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 84, "end_idx": 86, "type": "dru", "entity": "CsA" }, { "start_idx": 103, "end_idx": 105, "type": "dru", "entity": "CsA" }, { "start_idx": 112, "end_idx": 113, "type": "pro", "entity": "移植" }, { "start_idx": 115, "end_idx": 118, "type": "sym", "entity": "排斥反应" }, { "start_idx": 120, "end_idx": 122, "type": "dru", "entity": "CsA" }, { "start_idx": 123, "end_idx": 123, "type": "bod", "entity": "肾" } ] }, { "text": "(一)糖皮质激素在儿童肾移植中应用,一般认为>0.5mg/(kg•d)可抑制生长发育,而隔日疗法对生长发育影响较小,并可减少不良反应;在保持移植肾功能良好的情况下超过2年后完全停药,但部分患儿则因排斥反应而需重新服用激素。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "抑制生长发育" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "隔日疗法" }, { "start_idx": 72, "end_idx": 72, "type": "bod", "entity": "肾" }, { "start_idx": 98, "end_idx": 101, "type": "sym", "entity": "排斥反应" }, { "start_idx": 108, "end_idx": 109, "type": "dru", "entity": "激素" } ] }, { "text": "(二)环孢霉素(CsA)在小儿体内清除率高、半衰期短,故较成人小儿需使用较大的剂量。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "环孢霉素" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "CsA" } ] }, { "text": "在移植肾功能未恢复前不宜使用CsA。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肾" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "CsA" } ] }, { "text": "<5岁小儿给予12mg/(kg•d)(分2~3次给予)6个月后,血药浓度宜控制在50~125ng/ml。", "entities": [ { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "血" } ] }, { "text": "近年来采用CsA的微乳制剂(新山地明或新赛斯平)具易吸收、药物浓度稳定的优点,适宜应用于儿童肾移植。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "CsA" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "微乳制剂" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "新山地明" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "新赛斯平" }, { "start_idx": 44, "end_idx": 48, "type": "pro", "entity": "儿童肾移植" } ] }, { "text": "意大利学者在临床随机对照实验中将CsA+Pred+Aza与CsA+Pred与CsA单药三种方案进行比较,发现移植后生存率与器官功能长期随访结果并无明显差异,故提出CsA单药方案在一定程度上安全有效并降低了肾外并发症的发生率。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "CsA" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "Pred" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "Aza" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "CsA" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "CsA" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "移植" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "器官" }, { "start_idx": 81, "end_idx": 83, "type": "dru", "entity": "CsA" }, { "start_idx": 102, "end_idx": 106, "type": "dis", "entity": "肾外并发症" } ] }, { "text": "广州军区武汉总医院在临床中发现,肾移植患者长期将CsA与盐酸小檗碱(黄连素)联合应用能增加患者CsA血药浓度,且并不增加CsA毒性反应,可减少CsA用量。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "肾移植" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "CsA" }, { "start_idx": 28, "end_idx": 32, "type": "dru", "entity": "盐酸小檗碱" }, { "start_idx": 34, "end_idx": 36, "type": "dru", "entity": "黄连素" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "CsA" }, { "start_idx": 60, "end_idx": 66, "type": "sym", "entity": "CsA毒性反应" }, { "start_idx": 71, "end_idx": 73, "type": "dru", "entity": "CsA" } ] }, { "text": "另外,澳大利亚研究者1997年提出,Pred+Aza+ATG为较常用的三联疗法。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "Pred" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "Aza" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "ATG" } ] }, { "text": "(三)瑞帕霉素(SRL)有英国研究者在欧洲、澳大利亚和加拿大三国的临床实践中将SRL+CsA+Pred与SRL+Pred相比较,提出SRL+CsA+Pred移植后治疗3个月,继而CsA减量的方案更为安全有效,在肾功能及血压控制上效果更佳。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "瑞帕霉素" }, { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "SRL" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "SRL" }, { "start_idx": 43, "end_idx": 45, "type": "dru", "entity": "CsA" }, { "start_idx": 47, "end_idx": 50, "type": "dru", "entity": "Pred" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "SRL" }, { "start_idx": 56, "end_idx": 59, "type": "dru", "entity": "Pred" }, { "start_idx": 66, "end_idx": 68, "type": "dru", "entity": "SRL" }, { "start_idx": 70, "end_idx": 72, "type": "dru", "entity": "CsA" }, { "start_idx": 78, "end_idx": 79, "type": "pro", "entity": "移植" }, { "start_idx": 89, "end_idx": 91, "type": "dru", "entity": "CsA" }, { "start_idx": 105, "end_idx": 105, "type": "bod", "entity": "肾" }, { "start_idx": 109, "end_idx": 110, "type": "ite", "entity": "血压" } ] }, { "text": "(四)他克莫司FK506在肾移植患儿发生急慢性排斥反应而对大剂量激素治疗无效时,FK506(tacrolimus)是一种有效的药物。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dru", "entity": "他克莫司FK506" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "肾移植" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "急慢性排斥反应" }, { "start_idx": 32, "end_idx": 33, "type": "dru", "entity": "激素" }, { "start_idx": 40, "end_idx": 44, "type": "dru", "entity": "FK506" } ] }, { "text": "美国Emory大学研究发现,许多这样的病例由于激素的抵抗,将免疫抑制剂从CsA(或MMF)换成FK506,获得了良好的疗效,未见明显的近期不良反应;但有研究显示FK506长期应用与移植后远期糖尿病的发生有关。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "dru", "entity": "激素" }, { "start_idx": 30, "end_idx": 34, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 36, "end_idx": 38, "type": "dru", "entity": "CsA" }, { "start_idx": 41, "end_idx": 43, "type": "dru", "entity": "MMF" }, { "start_idx": 47, "end_idx": 51, "type": "dru", "entity": "FK506" }, { "start_idx": 80, "end_idx": 84, "type": "dru", "entity": "FK506" }, { "start_idx": 90, "end_idx": 91, "type": "pro", "entity": "移植" }, { "start_idx": 95, "end_idx": 97, "type": "dis", "entity": "糖尿病" } ] }, { "text": "五、肌电描记(一)胃电图胃平滑肌始终存在电活动,控制胃平滑肌的收缩和舒张,了解胃电活动也是检查胃运动功能的主要方法。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "胃电图" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "胃平滑肌" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "胃平滑肌" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "胃" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "胃" } ] }, { "text": "在人体应用的方法有腔内胃电记录和体表胃电记录。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "腔内胃电记录" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "体表胃电记录" } ] }, { "text": "前者将电极直接置于胃窦及胃体黏膜,可准确记录到胃平滑肌电活动情况,是诊断胃平滑肌异常活动的有效的方法,然而因其侵入性难以在临床普遍开展。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胃窦" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "胃体黏膜" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "胃平滑肌" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "胃平滑肌" } ] }, { "text": "20世纪50年代首次报道应用体表胃电记录技术,即胃电图(electrogastrography,EGG)。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "pro", "entity": "胃电图" }, { "start_idx": 28, "end_idx": 46, "type": "pro", "entity": "electrogastrography" }, { "start_idx": 48, "end_idx": 50, "type": "pro", "entity": "EGG" } ] }, { "text": "它是一种非侵入性检查方法,操作简单,因准确性强和可重复性以及与胃运动的关系不断得到认可,最近解释的EGG已从肉眼判断到光谱分析。", "entities": [ { "start_idx": 49, "end_idx": 51, "type": "pro", "entity": "EGG" } ] }, { "text": "定量指标包括EGG的主频率,正常胃慢波所占时间百分比,胃动过速、胃动过缓及其他动力紊乱所占比例。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "EGG" } ] }, { "text": "EGG面临的问题是如何分析所获结果,使之规范化,统一化。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EGG" } ] }, { "text": "应用EGG诊断具体器质性疾病尚为时过早。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "EGG诊断" } ] }, { "text": "(二)小肠及结肠肌电描记已有研究,但对其临床意义有相当大的争论。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "小肠及结肠肌电描记" } ] }, { "text": "(三)肛门括约肌和耻骨直肠肌电描记有助于肛门括约肌协调症的诊断。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "肛门括约肌" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "耻骨直肠肌" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "肛门括约肌协调症" } ] }, { "text": "第六篇重症监护和急救第一章儿科重症监护室的设施和组织【概述】危重症医学(criticalcaremedicine)是利用现代生物医学技术,对各种危重患者,特别是对有器官或多器官系统功能衰竭综合征(multipleorgandysfunctionsyndrome,MODS)患者的生命活动进行连续、系统的观察,并提供特殊形式的生命支持,协助患者度过生命危险期,以提高患者存活的一门科学。", "entities": [ { "start_idx": 82, "end_idx": 96, "type": "dis", "entity": "器官或多器官系统功能衰竭综合征" }, { "start_idx": 98, "end_idx": 129, "type": "dis", "entity": "multipleorgandysfunctionsyndrome" }, { "start_idx": 131, "end_idx": 134, "type": "dis", "entity": "MODS" } ] }, { "text": "重症监护病房(intensivecareunit,ICU)是指用现代医疗设备装备起来,能对各种危重患者的生命活动进行连续、系统的观察,并提供特殊生命支持的治疗单位。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dep", "entity": "重症监护病房" }, { "start_idx": 7, "end_idx": 23, "type": "dep", "entity": "intensivecareunit" }, { "start_idx": 25, "end_idx": 27, "type": "dep", "entity": "ICU" } ] }, { "text": "儿科重症监护病房(PICU)是集中治疗儿科危重患儿,并提供特殊生命支持的治疗单位。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dep", "entity": "儿科重症监护病房" }, { "start_idx": 9, "end_idx": 12, "type": "dep", "entity": "PICU" }, { "start_idx": 19, "end_idx": 20, "type": "dep", "entity": "儿科" } ] }, { "text": "据记载,早在1860年,FlorenceNightingle首先在医院开辟一个病区,把重症患者集中起来观察和看护,这就是萌芽期的ICU。", "entities": [ { "start_idx": 64, "end_idx": 66, "type": "dep", "entity": "ICU" } ] }, { "text": "ICU的优越性在第二次世界大战时得到了进一步的证明,当时战地医生将伤员集中起来治疗,发现死亡率明显降低,这使医生充分认识到建立ICU的重要性。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dep", "entity": "ICU" }, { "start_idx": 63, "end_idx": 65, "type": "dep", "entity": "ICU" } ] }, { "text": "20世纪50年代,DwightHarken建立了第一个心脏术后ICU,随后60年代发展到为中毒、颅脑外伤、高危产妇以及婴幼儿和胎儿的监护病房相继成立,如呼吸重症监护病房(RICU)、神经外科重症监护病房(NSICU)、儿科重症监护病房(PICU)、新生儿重症监护病房(NICU)等。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "心脏" }, { "start_idx": 31, "end_idx": 33, "type": "dep", "entity": "ICU" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "中毒" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "颅脑外伤" }, { "start_idx": 76, "end_idx": 83, "type": "dep", "entity": "呼吸重症监护病房" }, { "start_idx": 85, "end_idx": 88, "type": "dep", "entity": "RICU" }, { "start_idx": 91, "end_idx": 100, "type": "dep", "entity": "神经外科重症监护病房" }, { "start_idx": 102, "end_idx": 106, "type": "dep", "entity": "NSICU" }, { "start_idx": 109, "end_idx": 116, "type": "dep", "entity": "儿科重症监护病房" }, { "start_idx": 118, "end_idx": 121, "type": "dep", "entity": "PICU" }, { "start_idx": 124, "end_idx": 132, "type": "dep", "entity": "新生儿重症监护病房" }, { "start_idx": 134, "end_idx": 137, "type": "dep", "entity": "NICU" } ] }, { "text": "近年来,随着监测、医疗护理技术的不断提高,ICU在现代化医院中的地位显得越来越重要,ICU在国际先进医疗机构中心已成为非常普遍和高度发展的重要部门。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dep", "entity": "ICU" }, { "start_idx": 42, "end_idx": 44, "type": "dep", "entity": "ICU" } ] }, { "text": "与之相比,我国由于对专业技术的发展认识不够,且建立ICU仪器设备昂贵,投资量大,并缺乏专业人才,故ICU发展相对较慢。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "dep", "entity": "ICU" }, { "start_idx": 49, "end_idx": 51, "type": "dep", "entity": "ICU" } ] }, { "text": "对此,我国卫生部门非常重视ICU的发展,卫生部于1989年已把设立ICU列为衡量医院等级的条例内容。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dep", "entity": "ICU" }, { "start_idx": 33, "end_idx": 35, "type": "dep", "entity": "ICU" } ] }, { "text": "1982年,卫生部妇幼司与联合国儿童基金会在我国北京、上海、重庆、沈阳等地创建了最早的10个儿童重症监护中心。", "entities": [ { "start_idx": 46, "end_idx": 53, "type": "dep", "entity": "儿童重症监护中心" } ] }, { "text": "另外,随着我国国民经济的不断发展,近年来,国内各大儿童医院甚至于县级医院也相继建立了ICU。", "entities": [ { "start_idx": 42, "end_idx": 44, "type": "dep", "entity": "ICU" } ] }, { "text": "我们既要充分认识ICU在现代医学中的作用,又要防止不顾资金、技术条件和病员来源造成的资源浪费,因而了解ICU的设施和组织,对更好地发挥ICU部门的作用,提高我国危重患儿的诊治水平十分重要。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dep", "entity": "ICU" }, { "start_idx": 51, "end_idx": 53, "type": "dep", "entity": "ICU" }, { "start_idx": 67, "end_idx": 69, "type": "dep", "entity": "ICU" } ] }, { "text": "【ICU的位置】ICU应建立在具有较好条件的大型医院里(三甲或二甲医院),其布局因地而异。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dep", "entity": "ICU" }, { "start_idx": 8, "end_idx": 10, "type": "dep", "entity": "ICU" } ] }, { "text": "为了便于抢救危重患儿,理想的位置是接近急诊室和住院部手术室,便于实施紧急心肺复苏、气管插管和抢救手术。", "entities": [ { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "心肺复苏" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "气管插管" }, { "start_idx": 46, "end_idx": 49, "type": "pro", "entity": "抢救手术" } ] }, { "text": "另外,ICU最好和放射科、检验科、血库、供应室、急救车库比较接近,便于患儿的转运和检查。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dep", "entity": "放射科" }, { "start_idx": 13, "end_idx": 15, "type": "dep", "entity": "检验科" }, { "start_idx": 17, "end_idx": 18, "type": "dep", "entity": "血库" }, { "start_idx": 20, "end_idx": 22, "type": "dep", "entity": "供应室" } ] }, { "text": "【ICU的结构】ICU以敞开式大房间为主并配合隔离单间病房,房间涂以适于清洁消毒的光滑材料,常以淡绿色和淡黄色为主,室内必须设有空调或中央空调,保持室温24~26℃,湿度在50%~70%。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dep", "entity": "ICU" }, { "start_idx": 8, "end_idx": 10, "type": "dep", "entity": "ICU" } ] }, { "text": "室内最好有空气过滤装置,使泵入的新鲜空气单向流动,以减少空气中的细菌和尘埃。", "entities": [ { "start_idx": 32, "end_idx": 33, "type": "mic", "entity": "细菌" } ] }, { "text": "每个床单位设监护墙壁,内置监护仪、人工呼吸机、输液泵、抢救用具、多用电源插头(10~15个)、中央供氧、中央供空气、中央负压吸引管道和开关。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "equ", "entity": "监护仪" }, { "start_idx": 17, "end_idx": 21, "type": "equ", "entity": "人工呼吸机" }, { "start_idx": 23, "end_idx": 25, "type": "equ", "entity": "输液泵" }, { "start_idx": 47, "end_idx": 50, "type": "equ", "entity": "中央供氧" }, { "start_idx": 52, "end_idx": 56, "type": "equ", "entity": "中央供空气" }, { "start_idx": 58, "end_idx": 65, "type": "equ", "entity": "中央负压吸引管道" } ] }, { "text": "房顶装设有悬吊输液瓶用的U形滑道和隔离床帘。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "equ", "entity": "U形滑道" }, { "start_idx": 17, "end_idx": 20, "type": "equ", "entity": "隔离床帘" } ] }, { "text": "病房中央设护士工作站和中央监护站,护士工作站与每个病床能直视相对,随时监测每个患儿的病情变化;中央监护站与每个床边的监护仪联网,方便护士和医生随时了解病房内所有患儿的生命指标参数。", "entities": [ { "start_idx": 58, "end_idx": 60, "type": "equ", "entity": "监护仪" } ] }, { "text": "每间病房应设有1~2个洗手池,便于医护人员检查患儿前后的消毒洗手。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "equ", "entity": "洗手池" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "消毒洗手" } ] }, { "text": "此外,ICU备有充足的复苏和麻醉药品,并常规置于急救车和急救箱中。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dep", "entity": "ICU" } ] }, { "text": "ICU除病房外还需必要的工作用房,工作用房占总面积的30%~40%左右,其中包括:(一)医护办公室设在离病室近又能清楚观察患儿的地方,用玻璃墙隔开,设有报警记录系统。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dep", "entity": "ICU" } ] }, { "text": "(三)家属接待室紧邻ICU应设置家属接待室,便于医师和家属医患沟通,使他们了解患儿的病情、治疗措施、医疗费用,并在诊治中得到家属的理解和支持。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dep", "entity": "ICU" } ] }, { "text": "(四)专用化验室有条件的ICU内应设专用化验室,便于检测血气、电解质、渗透压、心电图等需频繁监测的项目;此外,先进的ICU还配有小型移动床旁X线机和B超,即便是机械通气的患儿也能随时检查。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dep", "entity": "ICU" }, { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "血气" }, { "start_idx": 31, "end_idx": 33, "type": "ite", "entity": "电解质" }, { "start_idx": 35, "end_idx": 37, "type": "ite", "entity": "渗透压" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "心电图" }, { "start_idx": 58, "end_idx": 60, "type": "dep", "entity": "ICU" }, { "start_idx": 68, "end_idx": 72, "type": "equ", "entity": "床旁X线机" }, { "start_idx": 74, "end_idx": 75, "type": "equ", "entity": "B超" }, { "start_idx": 80, "end_idx": 83, "type": "pro", "entity": "机械通气" } ] }, { "text": "(五)专用仪器室ICU内应设专用仪器室,主要放置各种人工呼吸机、监护仪和其他抢救设备。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dep", "entity": "ICU" }, { "start_idx": 26, "end_idx": 30, "type": "equ", "entity": "人工呼吸机" }, { "start_idx": 32, "end_idx": 34, "type": "equ", "entity": "监护仪" } ] }, { "text": "【ICU的病床设置】ICU的床位数以全院总床数的2%~8%为宜。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dep", "entity": "ICU" }, { "start_idx": 10, "end_idx": 12, "type": "dep", "entity": "ICU" } ] }, { "text": "一般一个敞开式病房设8~12张病床,大的重症监护中心可划分成多个监护病房,每个监护病房收治不同的监护患儿,如脑外科、胸外科、神内科、胸内科等。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "equ", "entity": "病床" }, { "start_idx": 54, "end_idx": 56, "type": "dep", "entity": "脑外科" }, { "start_idx": 58, "end_idx": 60, "type": "dep", "entity": "胸外科" }, { "start_idx": 62, "end_idx": 64, "type": "dep", "entity": "神内科" }, { "start_idx": 66, "end_idx": 68, "type": "dep", "entity": "胸内科" } ] }, { "text": "一个抢救单元(床位)应配备一套基本设备,包括各种监护仪器、呼吸器、氧疗设备、吸引器、复苏囊、输液泵等。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "equ", "entity": "监护仪器" }, { "start_idx": 29, "end_idx": 31, "type": "equ", "entity": "呼吸器" }, { "start_idx": 33, "end_idx": 36, "type": "equ", "entity": "氧疗设备" }, { "start_idx": 38, "end_idx": 40, "type": "equ", "entity": "吸引器" }, { "start_idx": 42, "end_idx": 44, "type": "equ", "entity": "复苏囊" }, { "start_idx": 46, "end_idx": 48, "type": "equ", "entity": "输液泵" } ] }, { "text": "【ICU人员结构及安排】ICU需要经专业培训、精通业务、技术熟练的医护人员;医师与病床之比为0.8~1∶1,包括科主任、主治医师及住院医师,最好由内科、外科和麻醉科的医师共同组成,医师除已掌握儿科各专业的一般知识外,还需掌握较为广泛的病理、生理、生化等基础知识,具有独立诊断及处理各器官衰竭的能力和熟练运用各种生命体征监护抢救仪器的技能。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dep", "entity": "ICU" }, { "start_idx": 12, "end_idx": 14, "type": "dep", "entity": "ICU" }, { "start_idx": 73, "end_idx": 74, "type": "dep", "entity": "内科" }, { "start_idx": 76, "end_idx": 77, "type": "dep", "entity": "外科" }, { "start_idx": 79, "end_idx": 81, "type": "dep", "entity": "麻醉科" }, { "start_idx": 96, "end_idx": 97, "type": "dep", "entity": "儿科" }, { "start_idx": 141, "end_idx": 144, "type": "dis", "entity": "器官衰竭" } ] }, { "text": "重症监护室的住院医师除在本科接受3年以上的培训外,通常应到麻醉科、普外科、心脏科、耳鼻喉科和放射科轮转进行规范化培训,在熟练掌握心肺复苏技术、气管插管技术和指征、熟练应用各种呼吸机和监护仪、能进行各种穿刺(经皮放置周围动静脉导管、胸腔穿刺、腰椎穿刺和脑室内穿刺)后,可固定在ICU长期工作。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "dep", "entity": "麻醉科" }, { "start_idx": 33, "end_idx": 35, "type": "dep", "entity": "普外科" }, { "start_idx": 37, "end_idx": 39, "type": "dep", "entity": "心脏科" }, { "start_idx": 41, "end_idx": 44, "type": "dep", "entity": "耳鼻喉科" }, { "start_idx": 46, "end_idx": 48, "type": "dep", "entity": "放射科" }, { "start_idx": 64, "end_idx": 69, "type": "pro", "entity": "心肺复苏技术" }, { "start_idx": 71, "end_idx": 74, "type": "pro", "entity": "气管插管" }, { "start_idx": 78, "end_idx": 79, "type": "sym", "entity": "指征" }, { "start_idx": 87, "end_idx": 89, "type": "equ", "entity": "呼吸机" }, { "start_idx": 91, "end_idx": 93, "type": "equ", "entity": "监护仪" }, { "start_idx": 100, "end_idx": 101, "type": "pro", "entity": "穿刺" }, { "start_idx": 109, "end_idx": 113, "type": "pro", "entity": "动静脉导管" }, { "start_idx": 115, "end_idx": 118, "type": "pro", "entity": "胸腔穿刺" }, { "start_idx": 120, "end_idx": 123, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 125, "end_idx": 129, "type": "pro", "entity": "脑室内穿刺" }, { "start_idx": 137, "end_idx": 139, "type": "dep", "entity": "ICU" } ] }, { "text": "ICU的护士从专科毕业后通常应经过6~12个月的ICU专业化培训。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "dep", "entity": "ICU" } ] }, { "text": "先进的ICU还配备有固定的营养专家和心理医师,此外还配有医学生物和电子工程师,负责维修保养各种仪器设备。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dep", "entity": "ICU" } ] }, { "text": "【ICU及监护系统】ICU集中了各种诊疗仪器和监护系统,包括各类型人工呼吸机、电除颤起搏器、颅压监护仪、输液泵和监护系统等,此外还有气管导管、喉镜、纤维支气管镜、引流瓶及其装置、各种温控设备、吸痰器、气管切开器材和停电后的供电设备等。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "equ", "entity": "IC" }, { "start_idx": 1, "end_idx": 2, "type": "dep", "entity": "IC" }, { "start_idx": 6, "end_idx": 8, "type": "dep", "entity": "护系统" }, { "start_idx": 29, "end_idx": 33, "type": "equ", "entity": "括各类型人" }, { "start_idx": 35, "end_idx": 40, "type": "equ", "entity": "呼吸机、电除" }, { "start_idx": 42, "end_idx": 46, "type": "equ", "entity": "起搏器、颅" }, { "start_idx": 48, "end_idx": 50, "type": "equ", "entity": "监护仪" }, { "start_idx": 52, "end_idx": 55, "type": "equ", "entity": "输液泵和" }, { "start_idx": 62, "end_idx": 65, "type": "equ", "entity": "此外还有" }, { "start_idx": 67, "end_idx": 68, "type": "equ", "entity": "管导" }, { "start_idx": 70, "end_idx": 75, "type": "equ", "entity": "、喉镜、纤维" }, { "start_idx": 77, "end_idx": 79, "type": "equ", "entity": "气管镜" }, { "start_idx": 92, "end_idx": 94, "type": "equ", "entity": "控设备" } ] }, { "text": "ICU最基本和最重要的诊疗仪器和监护系统如下:(一)抢救复苏设备氧气源、给氧鼻导管、简易呼吸器(面罩、复苏皮囊)、气管插管导管、喉镜、中心静脉及外周静脉导管、留置针等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dep", "entity": "ICU" }, { "start_idx": 32, "end_idx": 34, "type": "equ", "entity": "氧气源" }, { "start_idx": 36, "end_idx": 40, "type": "equ", "entity": "给氧鼻导管" }, { "start_idx": 42, "end_idx": 46, "type": "equ", "entity": "简易呼吸器" }, { "start_idx": 48, "end_idx": 49, "type": "equ", "entity": "面罩" }, { "start_idx": 51, "end_idx": 54, "type": "equ", "entity": "复苏皮囊" }, { "start_idx": 57, "end_idx": 62, "type": "equ", "entity": "气管插管导管" }, { "start_idx": 64, "end_idx": 65, "type": "equ", "entity": "喉镜" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "静脉" }, { "start_idx": 74, "end_idx": 77, "type": "equ", "entity": "静脉导管" }, { "start_idx": 79, "end_idx": 81, "type": "equ", "entity": "留置针" } ] }, { "text": "(二)人工呼吸机机械通气是ICU的主要任务之一,正确使用人工呼吸机能起到挽救患儿生命的作用。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "equ", "entity": "人工呼吸机" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "机械通气" }, { "start_idx": 13, "end_idx": 15, "type": "dep", "entity": "ICU" }, { "start_idx": 28, "end_idx": 32, "type": "equ", "entity": "人工呼吸机" } ] }, { "text": "应用人工呼吸机的目的是维持代谢所需的肺泡通气,纠正高碳酸血症;提供适合的氧浓度,纠正难治性低氧血症;改善氧输送,减少呼吸功,降低氧耗量,缓解呼吸肌疲劳。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "equ", "entity": "人工呼吸机" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肺泡" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "低氧血症" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "呼吸肌" } ] }, { "text": "目前,国内外常用人工呼吸机品牌众多,性能各不相同,通气理论、技术、模式也在不断更新,如西门子伺服(SIEMENSServo)系列呼吸机、熊牌(Bear)系列呼吸机、星牌婴儿(Infantstar)系列呼吸机、纽帮(Newport)系列呼吸机、Drager系列呼吸机、PB系列呼吸机等,充分了解各类呼吸机的性能十分重要(呼吸机的使用见有关章节)。", "entities": [ { "start_idx": 64, "end_idx": 66, "type": "equ", "entity": "呼吸机" }, { "start_idx": 78, "end_idx": 80, "type": "equ", "entity": "呼吸机" }, { "start_idx": 100, "end_idx": 102, "type": "equ", "entity": "呼吸机" }, { "start_idx": 117, "end_idx": 119, "type": "equ", "entity": "呼吸机" }, { "start_idx": 129, "end_idx": 131, "type": "equ", "entity": "呼吸机" }, { "start_idx": 137, "end_idx": 139, "type": "equ", "entity": "呼吸机" }, { "start_idx": 148, "end_idx": 150, "type": "equ", "entity": "呼吸机" }, { "start_idx": 159, "end_idx": 161, "type": "equ", "entity": "呼吸机" } ] }, { "text": "(三)输液泵输液泵分为推注式注射器输液泵和蠕动式输液泵,是一种利用机械推动流体进入血管系统的一种电子机械装置,可按要求以恒定的速度输注定量的液体。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "输液泵" }, { "start_idx": 6, "end_idx": 8, "type": "equ", "entity": "输液泵" }, { "start_idx": 11, "end_idx": 19, "type": "equ", "entity": "推注式注射器输液泵" }, { "start_idx": 21, "end_idx": 26, "type": "equ", "entity": "蠕动式输液泵" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "血管系统" } ] }, { "text": "推注式注射器输液泵容量小、精确度高,故多用于输注需要精确控制剂量药物或半衰期特别短、需持续滴入的药物。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "equ", "entity": "推注式注射器" } ] }, { "text": "蠕动输液泵输液速度能控制于1~999ml/h的范围,为常规保证治疗时使用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "equ", "entity": "蠕动输液泵" } ] }, { "text": "(四)电除颤仪电击除颤仪由可调的高压直流电源(电池)、电容器及限制电流的限流线圈构成。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "equ", "entity": "电除颤仪" }, { "start_idx": 7, "end_idx": 11, "type": "equ", "entity": "电击除颤仪" } ] }, { "text": "电除颤及电复律是让一个电压极高、时间极短、电流极小的电流通过纤维颤动的心脏,使心肌纤维同时除极,然后同时复极,从而使心肌的收缩协调。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "电除颤" }, { "start_idx": 4, "end_idx": 6, "type": "pro", "entity": "电复律" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "心脏" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "心肌纤维" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "心肌" } ] }, { "text": "(五)负压吸引装置为清除患儿口、鼻和呼吸道痰液和异物的装置,一般婴儿负压<150mmHg,儿童负压<200mmHg。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "equ", "entity": "负压吸引装置" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "口" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "鼻" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "呼吸道痰液" } ] }, { "text": "(六)监护系统ICU治疗及监护是相辅相成,缺一不可,针对不同的病种及疾病的不同病理状态给予不同的监护治疗。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dep", "entity": "ICU" } ] }, { "text": "由基本监护和特殊监护组成,基本监护由床旁监护仪和中心监护站(包括控制系统、显示系统、记录系统)组成;特殊监护为针对不同病理状态及各系统特殊治疗要求,对特殊科室的危重患儿提供的特殊而有效监护。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "equ", "entity": "床旁监护仪" } ] }, { "text": "1.基本监护(1)床旁监护仪:由具有心脏监护、呼吸监护、血压监护、经皮血氧饱和度监测(TcSO2)和体温监护的多功能监护仪组成:①心脏监护:主要用于持续监测心电活动,荧光屏上连续显示心电波形和心率数值。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "equ", "entity": "床旁监护仪" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "心脏" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "呼吸" }, { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "血压" }, { "start_idx": 35, "end_idx": 39, "type": "ite", "entity": "血氧饱和度" }, { "start_idx": 43, "end_idx": 46, "type": "ite", "entity": "TcSO" }, { "start_idx": 61, "end_idx": 62, "type": "ite", "entity": "体温" }, { "start_idx": 69, "end_idx": 71, "type": "equ", "entity": "监护仪" }, { "start_idx": 76, "end_idx": 79, "type": "pro", "entity": "心脏监护" }, { "start_idx": 89, "end_idx": 90, "type": "ite", "entity": "心电" }, { "start_idx": 102, "end_idx": 103, "type": "ite", "entity": "心电" }, { "start_idx": 107, "end_idx": 108, "type": "ite", "entity": "心率" } ] }, { "text": "通过振幅调节键调节所需振幅,并可设置报警上下限,当心率超过预设范围时,仪器发出声响报警。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "心率" } ] }, { "text": "有的仪器可在报警时自动记录心率或心电波形,或有趋向显示储存前24小时内心率情况。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "心率" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "心电" }, { "start_idx": 35, "end_idx": 36, "type": "ite", "entity": "心率" } ] }, { "text": "荧光屏上显示的心电波形,主要为了解心率和心律变化,不能作为分析S-T段和各波形的依据。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "心电" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "心率" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "心律" } ] }, { "text": "因此,当心电波出现异常时需结合临床,必要时作常规心电图检查,以判断监测结果。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "心电波" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "心电图" } ] }, { "text": "②呼吸监护:主要用于持续监测呼吸活动,荧光屏上连续显示呼吸波形和呼吸频率,也有报警装置,当呼吸频率或呼吸停止时间超过预设范围时,仪器发出音响报警。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "呼吸" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "呼吸" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "呼吸" }, { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "呼吸" }, { "start_idx": 45, "end_idx": 46, "type": "ite", "entity": "呼吸" }, { "start_idx": 50, "end_idx": 51, "type": "ite", "entity": "呼吸" } ] }, { "text": "注意调节监护仪的合适敏感度,敏感度过低时,浅表的呼吸不能通过其胸廓阻抗的改变而显示出呼吸频率,遂发出呼吸暂停的警报。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "equ", "entity": "监护仪" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "胸廓" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "呼吸" } ] }, { "text": "敏感度过高,又可将心脏搏动引起的胸廓阻抗改变显示出呼吸,影响结果的判断。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "心脏" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "胸廓" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "呼吸" } ] }, { "text": "③血压监护:一般常用间接测压法(无创伤性测压法)。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "血压" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "间接测压法" }, { "start_idx": 16, "end_idx": 22, "type": "pro", "entity": "无创伤性测压法" } ] }, { "text": "可采用Dinamap血压测定仪,应用示波技术与微计算机,以特制的袖带束缚上臂,仪器自动充气放气,测得收缩压、舒张压、平均压和心率,以数字显示。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "equ", "entity": "Dinamap血压测定仪" }, { "start_idx": 32, "end_idx": 33, "type": "equ", "entity": "袖带" }, { "start_idx": 50, "end_idx": 52, "type": "ite", "entity": "收缩压" }, { "start_idx": 54, "end_idx": 56, "type": "ite", "entity": "舒张压" }, { "start_idx": 58, "end_idx": 60, "type": "ite", "entity": "平均压" }, { "start_idx": 62, "end_idx": 63, "type": "ite", "entity": "心率" } ] }, { "text": "本监护方法简便,但不如直接测压法准确。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "直接测压法" } ] }, { "text": "④经皮血氧饱和度监测(TcSO2):利用红光(660nm)和红外光(940nm)穿透外周血管床后吸收比例不同来测定动脉血氧饱和度,目前已广泛应用于临床。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "ite", "entity": "经皮血氧饱和度" }, { "start_idx": 11, "end_idx": 14, "type": "ite", "entity": "TcSO" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "血管床" }, { "start_idx": 68, "end_idx": 74, "type": "ite", "entity": "动脉血氧饱和度" } ] }, { "text": "使用时将探头夹于指(趾)端甲床、耳垂、手掌或足底部,数秒钟后荧光屏上即可显示氧饱和度数值。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "equ", "entity": "探头" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "指(趾)端甲床" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "耳垂" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "手掌" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "足底" }, { "start_idx": 38, "end_idx": 41, "type": "ite", "entity": "氧饱和度" } ] }, { "text": "经皮血氧饱和度(TcSO2)在末梢循环正常、没有涂抹染指情况下,TcSO2几乎和动脉血氧饱和度(SaO2)数值一致。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "ite", "entity": "经皮血氧饱和度" }, { "start_idx": 8, "end_idx": 11, "type": "ite", "entity": "TcSO" }, { "start_idx": 43, "end_idx": 46, "type": "ite", "entity": "TcSO" }, { "start_idx": 62, "end_idx": 68, "type": "ite", "entity": "动脉血氧饱和度" }, { "start_idx": 70, "end_idx": 72, "type": "ite", "entity": "SaO" } ] }, { "text": "但当体温<35℃、血压<50mmHg、用血管收缩药或血液中存在正铁血红蛋白和亚甲蓝等情况下,可影响SaO2准确性。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "体温" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "血压" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "血管收缩药" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "血液" }, { "start_idx": 31, "end_idx": 36, "type": "bod", "entity": "正铁血红蛋白" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "亚甲蓝" }, { "start_idx": 49, "end_idx": 51, "type": "ite", "entity": "SaO" } ] }, { "text": "本法对低氧血症有监护意义,但不能监测高氧血症。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "低氧血症" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "高氧血症" } ] }, { "text": "⑤体温监护:将探头置于患儿腋下或肛门直肠内便于长时间观察患儿体温变化情况,并可设置报警上下限,方便进行各种抢救治疗护理操作。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "体温" }, { "start_idx": 7, "end_idx": 8, "type": "equ", "entity": "探头" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "腋下" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "肛门直肠内" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "体温" }, { "start_idx": 53, "end_idx": 54, "type": "pro", "entity": "抢救" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "治疗" }, { "start_idx": 57, "end_idx": 58, "type": "pro", "entity": "护理" } ] }, { "text": "(2)中心监护站:中心监护站是对各床旁监护仪资料的汇集,当监测参数超过报警线时,可发出中心报警信号。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "equ", "entity": "监护仪" } ] }, { "text": "2.特殊监护(1)脑电监护:为脑电信号的连续监测,可作各种原因引起的脑功能紊乱的监护、抗惊厥药物疗效的监测,对不典型的惊厥发作提供诊断依据,为脑死亡诊断作出客观评价,临床上有较大的应用价值。", "entities": [ { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "脑" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "惊厥" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "惊厥" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "脑死亡" } ] }, { "text": "监测过程中,荧光屏可分别显示代表左右两侧大脑半球的两个标推导联的脑电图信号及功率谱阵图。", "entities": [ { "start_idx": 16, "end_idx": 23, "type": "bod", "entity": "左右两侧大脑半球" } ] }, { "text": "(2)颅内压监护:临床应用的颅内压监护方法有2种:一是应用前囟测压计监护,适用于新生儿及前囟未闭的婴儿,为非损伤性测压法。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "颅内压" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "颅内压" }, { "start_idx": 29, "end_idx": 33, "type": "equ", "entity": "前囟测压计" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "前囟" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "前囟未闭" }, { "start_idx": 53, "end_idx": 59, "type": "pro", "entity": "非损伤性测压法" } ] }, { "text": "将传感器置于前囟,利用压力换能器将所测得的压力换成电能,直接读数;二是持续直接测压法,为损伤性测压法,将传感器直接置于硬膜外、蛛网膜下腔或脑室内,借助压力传感器将颅内压力转换为电能输入监护仪,经处理后转变为波形及数字,显示于荧光屏上,同时可描记其压力曲线。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "equ", "entity": "传感器" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "前囟" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "直接测压法" }, { "start_idx": 44, "end_idx": 49, "type": "pro", "entity": "损伤性测压法" }, { "start_idx": 52, "end_idx": 54, "type": "equ", "entity": "传感器" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "硬膜外" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "脑室" }, { "start_idx": 77, "end_idx": 79, "type": "equ", "entity": "传感器" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "颅内" }, { "start_idx": 92, "end_idx": 94, "type": "equ", "entity": "监护仪" } ] }, { "text": "(3)呼气末二氧化碳(PetCO2)分压监测:采用无创性呼气末二氧化碳浓度监测仪可以及时了解机械通气的情况,而不必间歇抽血检查。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "ite", "entity": "呼气末二氧化碳" }, { "start_idx": 11, "end_idx": 15, "type": "ite", "entity": "PetCO" }, { "start_idx": 36, "end_idx": 50, "type": "equ", "entity": "无创性呼气末二氧化碳浓度监测仪" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "机械通气" }, { "start_idx": 70, "end_idx": 71, "type": "pro", "entity": "抽血" } ] }, { "text": "在健康成人个体,动脉血二氧化碳分压(PaCO2)与PetCO2差值小于6mmHg,当发生肺血液灌流量减少时,使PetCO2降低,可导致PaCO2与PetCO2差值增大。", "entities": [ { "start_idx": 8, "end_idx": 16, "type": "ite", "entity": "动脉血二氧化碳分压" }, { "start_idx": 18, "end_idx": 21, "type": "ite", "entity": "PaCO" }, { "start_idx": 36, "end_idx": 40, "type": "ite", "entity": "PetCO" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "肺" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "血液" }, { "start_idx": 77, "end_idx": 81, "type": "ite", "entity": "PetCO" }, { "start_idx": 100, "end_idx": 103, "type": "ite", "entity": "PaCO" }, { "start_idx": 117, "end_idx": 121, "type": "ite", "entity": "PetCO" } ] }, { "text": "【ICU的管理】(一)ICU的收入指征医院要充分发挥ICU的作用,必须开辟和完善抢救绿色通道,形成前沿,为院前急救、急诊预诊、急诊室、抢救室,对内为观察室,为各病区服务。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dep", "entity": "ICU" }, { "start_idx": 11, "end_idx": 13, "type": "dep", "entity": "ICU" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "指征" }, { "start_idx": 26, "end_idx": 28, "type": "dep", "entity": "ICU" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "急救" }, { "start_idx": 58, "end_idx": 61, "type": "pro", "entity": "急诊预诊" } ] }, { "text": "加强科间会诊,各病区危重患者随时送重症监护室,增强急诊科与各临床科室间联系,形成完善的院内急救系统。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "dep", "entity": "急诊科" } ] }, { "text": "ICU所有医护人员必须清楚儿科急诊范围(表6-6)、危重患儿急救绿色通道范围(表6-6)和ICU的收入指征(表6-6)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dep", "entity": "ICU" }, { "start_idx": 13, "end_idx": 14, "type": "dep", "entity": "儿科" }, { "start_idx": 45, "end_idx": 47, "type": "dep", "entity": "ICU" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "指征" } ] }, { "text": "遇有儿科急诊范围情况的患儿,急诊预诊护士应安排优先就诊,遇有危重患儿急救绿色通道范围患儿应直送ICU。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dep", "entity": "儿科" }, { "start_idx": 47, "end_idx": 49, "type": "dep", "entity": "ICU" } ] }, { "text": "此外,全院各科患儿只要达到ICU的收入指征均应转入ICU治疗。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dep", "entity": "ICU" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "指征" }, { "start_idx": 25, "end_idx": 27, "type": "dep", "entity": "ICU" } ] }, { "text": "当危重患儿病情稳定后应及时转出ICU,为专科医师提供原发病的诊治机会。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dep", "entity": "ICU" } ] }, { "text": "表6-1儿科急诊范围表6-2危重患儿急救绿色通道范围表6-3ICU的收入指征脑死亡患儿及恶性肿瘤终末期的患儿不属收入ICU的对象。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dep", "entity": "儿科" }, { "start_idx": 30, "end_idx": 32, "type": "dep", "entity": "ICU" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "指征" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "脑死亡" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 58, "end_idx": 60, "type": "dep", "entity": "ICU" } ] }, { "text": "(二)ICU岗位责任制1.凡进入ICU的患儿多属危急重症,应争分夺秒,紧急抢救。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dep", "entity": "ICU" }, { "start_idx": 16, "end_idx": 18, "type": "dep", "entity": "ICU" } ] }, { "text": "对来到ICU患儿,无论是何科疾病,均应作应急处理,首先是维持生命体征,然后根据具体情况请专科会诊,协同处置。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dep", "entity": "ICU" }, { "start_idx": 28, "end_idx": 33, "type": "pro", "entity": "维持生命体征" } ] }, { "text": "ICU内患儿病情危重,免疫功能低下,容易发生交叉感染,加之长期强有力抗生素的使用,容易产生耐药性细菌株,故无菌监测至关重要。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dep", "entity": "ICU" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "免疫功能" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "交叉感染" }, { "start_idx": 34, "end_idx": 36, "type": "dru", "entity": "抗生素" }, { "start_idx": 48, "end_idx": 50, "type": "mic", "entity": "细菌株" } ] }, { "text": "(四)ICU仪器保管ICU仪器设备大多数价格昂贵,应由专人管理,操作规程及注意事项制成卡片挂于仪器一侧,做到定期检查、维修及消毒。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dep", "entity": "ICU" }, { "start_idx": 10, "end_idx": 12, "type": "dep", "entity": "ICU" } ] }, { "text": "工作人员每天交班,保持仪器设备清洁,保证各种配件完好后备用,通常一台呼吸机至少保持2套管道备用,每周除湿一次,每次12~24小时。", "entities": [ { "start_idx": 34, "end_idx": 36, "type": "equ", "entity": "呼吸机" }, { "start_idx": 43, "end_idx": 44, "type": "equ", "entity": "管道" }, { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "除湿" } ] }, { "text": "【ICU发展趋势】ICU的建立和重症监护技术的发展带动了对专业技术人才的培养和学科的发展,危重病医学本身也超越了传统医学专科的专业界限,形成了一门多学科、多专业互相交叉、互相渗透的边缘科学,急诊医学也逐渐成为了一门高度发展的专业。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dep", "entity": "ICU" }, { "start_idx": 9, "end_idx": 11, "type": "dep", "entity": "ICU" } ] }, { "text": "因此,ICU的医护人员必须经常学习和进修培训,不断提高本专业的学术水平。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dep", "entity": "ICU" } ] }, { "text": "今后5~10年本学科的发展趋势将有如下几个方面:1.开展儿科危重病临床流行病学调查、儿科危重病严重度评分和诊断。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "dep", "entity": "儿科" }, { "start_idx": 42, "end_idx": 43, "type": "dep", "entity": "儿科" } ] }, { "text": "2.危重病治疗学方面,开展各种无创伤性监测技术,如血压、血气、氧饱和度、CO2连续监测、床边B超监测等,可大大减轻患儿痛苦,及时获得资料。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "无创伤性监测" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "血压" }, { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "血气" }, { "start_idx": 31, "end_idx": 34, "type": "ite", "entity": "氧饱和度" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "CO" }, { "start_idx": 55, "end_idx": 60, "type": "pro", "entity": "床边B超监测" } ] }, { "text": "各种呼吸机辅助支持模式、肺表面活性物质替代治疗、一氧化氮吸入治疗、膜肺(ECMO)等技术开展,可大大提高呼吸衰竭抢救成功率。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "呼吸机" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肺" }, { "start_idx": 24, "end_idx": 31, "type": "pro", "entity": "一氧化氮吸入治疗" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "膜肺" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "ECMO" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "分子生物学技术在儿科急诊临床和基础研究中的应用将揭示器官衰竭疾病的发病机制。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dep", "entity": "儿科" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "器官衰竭" } ] }, { "text": "计算机信息技术在ICU工作中的普及,它不仅可反映情况,供临床参考,并可用来教学、培训和科研工作之用。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dep", "entity": "ICU" } ] }, { "text": "此外,监护技术的提高,促进了对许多过去不能施行的手术得以积极开展,ICU的发展为心血管外科和脑外科专业的迅速发展提供了可靠保证。", "entities": [ { "start_idx": 33, "end_idx": 35, "type": "dep", "entity": "ICU" }, { "start_idx": 40, "end_idx": 44, "type": "dep", "entity": "心血管外科" }, { "start_idx": 46, "end_idx": 48, "type": "dep", "entity": "脑外科" } ] }, { "text": "5.在我国还面临建立适合国情的儿科ICU和儿童急诊医疗体制、高新技术和适宜技术结合以及中西医理论和中西药结合等一系列课题的研究。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dep", "entity": "ICU" } ] }, { "text": "第四章小儿胃食管反流病胃食管反流(gastroesophagealreflux,GER)有生理性和病理性两种。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "小儿胃食管反流病" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 17, "end_idx": 38, "type": "dis", "entity": "gastroesophagealreflux" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "GER" } ] }, { "text": "正常人每天都有短暂的、无症状的生理性胃食管反流,这并不引起食管黏膜的损伤。", "entities": [ { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "生理性胃食管反流" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "食管黏膜" } ] }, { "text": "当胃内容物反流至食管导致组织损伤而引起症状则为病理性反流,随之出现的一系列疾病症状,统称为胃食管反流病(gastroesophagealrefluxdisease,GERD)。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "胃" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "病理性反流" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "胃食管反流病" }, { "start_idx": 52, "end_idx": 80, "type": "dis", "entity": "gastroesophagealrefluxdisease" }, { "start_idx": 82, "end_idx": 85, "type": "dis", "entity": "GERD" } ] }, { "text": "小儿胃食管反流症是指由于胃内容物不受控制地从胃反流入食管,甚至口腔而引起的一系列顽固性呕吐、反胃及食管炎症状,呼吸道症状,甚至神经精神症状的上消化道运动障碍性疾病。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "小儿胃食管反流症" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "口腔" }, { "start_idx": 12, "end_idx": 44, "type": "sym", "entity": "胃内容物不受控制地从胃反流入食管,甚至口腔而引起的一系列顽固性呕吐" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "反胃" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "食管炎症状" }, { "start_idx": 55, "end_idx": 59, "type": "sym", "entity": "呼吸道症状" }, { "start_idx": 63, "end_idx": 80, "type": "sym", "entity": "神经精神症状的上消化道运动障碍性疾病" } ] }, { "text": "它可以导致小儿营养不良、生长发育迟缓、食管炎、反复发作的肺炎、支气管炎、哮喘,甚至婴儿猝死综合征(SIDS)。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "小儿营养不良" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "生长发育迟缓" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "食管炎" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "反复发作的肺炎" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "支气管炎" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "哮喘" }, { "start_idx": 39, "end_idx": 47, "type": "dis", "entity": "甚至婴儿猝死综合征" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "SIDS" } ] }, { "text": "小儿胃食管反流病是一种消化系统常见病,据报道,美国GERD的人群发病率在25%~35%之间。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "小儿胃食管反流病" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "消化系统常见病" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "GERD" } ] }, { "text": "我国,由胃食管反流反流性食管身体炎GERD在儿童,尤其在新生儿及早产儿中有较高的发病率,并认为它与早产儿的呼吸暂停、喂养困难及吸入性肺炎胃食管反流问题已经越来越被人们所关注,并作了广泛的研究。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "反流性食管身体炎" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "GERD" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "喂养困难" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "肺" }, { "start_idx": 63, "end_idx": 67, "type": "sym", "entity": "吸入性肺炎" }, { "start_idx": 68, "end_idx": 72, "type": "dis", "entity": "胃食管反流" } ] }, { "text": "【病因及发病机制】目前认为GERD的发生和发展是多种因素综合作用的过程,包括防止过度胃食管反流和迅速清除食管内有害物质两种机制的功能障碍。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "GERD" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "胃食管" } ] }, { "text": "(一)抗反流机制1.食管下端括约肌张力减低食管下端括约肌(loweresophagealsphincter,LES)是一段位于食管远端长约1.0~3.5cm特化的环行肌,它能产生并维持超过胃内压约1.33~5.33kPa(10~40mmHg)的静息压来防止反流,还可在咳嗽、打喷嚏或用力而使腹内压突然增高时迅速做出反应。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "食管" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "括约肌" }, { "start_idx": 10, "end_idx": 20, "type": "sym", "entity": "食管下端括约肌张力减低" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "食管下端括约肌" }, { "start_idx": 29, "end_idx": 52, "type": "bod", "entity": "loweresophagealsphincter" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "LES" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "食管" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "环行肌" }, { "start_idx": 94, "end_idx": 96, "type": "ite", "entity": "胃内压" }, { "start_idx": 122, "end_idx": 124, "type": "ite", "entity": "静息压" }, { "start_idx": 145, "end_idx": 147, "type": "ite", "entity": "腹内压" } ] }, { "text": "20世纪80年代前,许多学者认为食管下端并无括约肌存在,只是经测压证实该处有一段高压区,有括约肌样作用。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "食管" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "括约肌" } ] }, { "text": "近年来,随着微解剖研究的深入,提示这种肌肉结构确实存在,并由此构成食管腹段至膈上的2~4cm的高压带,其压力随胃内压的增高而增加,构成最有效的抗反流屏障。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "食管" }, { "start_idx": 55, "end_idx": 57, "type": "ite", "entity": "胃内压" } ] }, { "text": "LES的功能受神经及体液双重调节。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "LES" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "神经" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "体液" } ] }, { "text": "迷走神经及胃泌素使食管下端括约肌静息压LESP)升高,而胰泌素、胆囊收缩素(CCK)及肠抑胃肽(GIP)等则使其下降。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "迷走神经" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "胃泌素" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "食管" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "括约肌" }, { "start_idx": 19, "end_idx": 22, "type": "ite", "entity": "LESP" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "胰泌素" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "胆囊收缩素" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "CCK" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "肠抑胃肽" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "GIP" } ] }, { "text": "LES的成熟还与受孕后日龄(胎龄+出生后日龄)呈正相关,故新生儿、尤其早产儿更易发生胃食管反流。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "LES" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "胃食管反流" } ] }, { "text": "当LESP低下时就不能有效地对抗腹腔与胸腔之间的正性压力梯度而导致持续的胃食管反流,在腹内压突然增加时也不能做出充分的反应,则胃内容物将被逆排入食管GERD患者、尤其是伴重度食管炎及Barrett食管患者的LESP明显低于正常人,因而食管下端括约肌(LES)功能不全以及食管下端括约肌静息压(LESP)降低是GERD最重要的发病因素之一。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "ite", "entity": "LESP" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "腹腔" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胸腔" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "持续的胃食管反流" }, { "start_idx": 63, "end_idx": 63, "type": "bod", "entity": "胃" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "食管" }, { "start_idx": 43, "end_idx": 73, "type": "sym", "entity": "腹内压突然增加时也不能做出充分的反应,则胃内容物将被逆排入食管" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "GERD" }, { "start_idx": 85, "end_idx": 89, "type": "dis", "entity": "重度食管炎" }, { "start_idx": 91, "end_idx": 99, "type": "dis", "entity": "Barrett食管" }, { "start_idx": 103, "end_idx": 106, "type": "ite", "entity": "LESP" }, { "start_idx": 117, "end_idx": 123, "type": "bod", "entity": "食管下端括约肌" }, { "start_idx": 125, "end_idx": 127, "type": "bod", "entity": "LES" }, { "start_idx": 135, "end_idx": 144, "type": "ite", "entity": "食管下端括约肌静息压" }, { "start_idx": 146, "end_idx": 149, "type": "ite", "entity": "LESP" } ] }, { "text": "然而多项研究表明,LESP正常者也会发生胃食管反流,而较轻型的GERD患者的LESP也往往是正常的。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "LESP" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "GERD" }, { "start_idx": 38, "end_idx": 41, "type": "ite", "entity": "LESP" } ] }, { "text": "研究中还发现新生儿LESP并不低于年长儿及成人,所以GERD的发生可能不仅仅是由于LESP的降低。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "LESP" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "GERD" }, { "start_idx": 41, "end_idx": 44, "type": "ite", "entity": "LESP" } ] }, { "text": "目前研究认为LES一过性松弛(TLESR)是正常人生理性胃食管反流及LESP正常的GERD患者的主要发病机制。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "LES一过性松弛" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "TLESR" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "生理性胃食管反流" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "LESP" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "GERD" } ] }, { "text": "在原发性蠕动(由吞咽引起的蠕动)过程中,LES松弛3~10秒以允许吞咽的食团进入胃内,而LES一过性松弛并不发生于正常蠕动之后,持续时间也较长,约10~45秒。", "entities": [ { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "胃" } ] }, { "text": "在此过程中,LESP下降至0时括约肌即不再具有抗反流作用了。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "括约肌" } ] }, { "text": "这就解释了正常人的生理性反流及LESP正常的GERD患者的发病原因。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "生理性反流" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "LESP" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "GERD" } ] }, { "text": "国外文献报道,约50%以上的GERD属于TLESR,TLESR伴发酸反流的发生率达82%。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "GERD" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "TLESR" }, { "start_idx": 26, "end_idx": 35, "type": "dis", "entity": "TLESR伴发酸反流" } ] }, { "text": "正常受试者中40%~50%的TLESR伴胃酸反流,GERD患者中TLESR伴胃酸反流则达60%~70%。", "entities": [ { "start_idx": 14, "end_idx": 23, "type": "dis", "entity": "TLESR伴胃酸反流" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "GERD" }, { "start_idx": 32, "end_idx": 41, "type": "dis", "entity": "TLESR伴胃酸反流" } ] }, { "text": "这些都提示了TLESR是引起胃食管反流的主要因素。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "TLESR" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "胃食管反流" } ] }, { "text": "2.解剖因素除了LES外,这段食管的一些解剖因素无疑也起着抗当腹内压升高时,食管腹段被钳夹呈扁形腹段被钳夹呈扁形,从而起到抗反流作用,因此食管腹段越长,此功能则越完善。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "食管" }, { "start_idx": 30, "end_idx": 47, "type": "sym", "entity": "当腹内压升高时,食管腹段被钳夹呈扁形" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "食管" } ] }, { "text": "3个月以下的婴儿食管腹段很短,所以极易发生胃食管反流;胃食管交角(His角)为锐角,能使胃黏液在食管口外侧形成一活瓣而抗反流。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "食管" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "胃食管" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "胃黏液" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "食管" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "活瓣" } ] }, { "text": "食管手术及食管裂孔疝可令此角变钝,抗反流作用减弱;另外,膈角在吸气时可主动收缩,起到了食管外括约肌的作用,可加强LES的抗反流能力。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "食管手术" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "膈" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "食管" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "括约肌" } ] }, { "text": "而食管裂孔疝的形成破坏了外括约肌抗反流机制,因此这类患儿亦常伴有胃食管反流。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "食管裂孔疝" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "胃食管反流" } ] }, { "text": "(二)食管清除机制胃食管反流发生后,如果侵蚀性物质被很快地清除出食管,那么食管黏膜并不会受到损伤。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "食管" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "食管黏膜" } ] }, { "text": "正常情况下,在重力、食管蠕动、唾液及食管内产生的碳酸氢盐的共同作用下,食管通过两个步骤进行酸的清除。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "食管" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "唾液" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "碳酸氢盐" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "食管" } ] }, { "text": "第一步容量清除:大部分反流物由于其自身重力和1~2次食管蠕动性收缩的联合作用而被迅速清除,但食管黏膜仍为酸性;第二步由吞下的碱性唾液及食管黏膜自身产生的碳酸氢盐缓冲,中和残留在食管壁上的酸性物质。", "entities": [ { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "食管黏膜" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "唾液" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "食管黏膜" }, { "start_idx": 76, "end_idx": 79, "type": "bod", "entity": "碳酸氢盐" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "食管壁" } ] }, { "text": "GERD与食管这种清除能力的削弱密切相关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "GERD" } ] }, { "text": "在一些GERD患儿中常可见食管蠕动振幅降低,继发性蠕动减弱或消失。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "GERD" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "食管" } ] }, { "text": "另外,睡眠中发生的反流尤其容易损伤食管。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "食管" } ] }, { "text": "因为平卧睡眠时,反流物失去了重力的作用因而清除的速度被延缓了;其次,人在睡眠时实际上停止了吞咽和大量分泌唾液,所以既无原发性蠕动也无充分的唾液可用于中和食管内的酸。", "entities": [ { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "唾液" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "唾液" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "食管" } ] }, { "text": "(三)食管黏液屏障正常的食管黏膜屏障包括3部分:①上皮前屏障屏障,指附着的黏液,含不移动水及碳酸氢根,能对胃蛋白酶起到阻挡作用H+物中的H+;②上皮屏障障,指上皮间紧密排列的多层鳞状上皮细胞,使反流物难以通过;③上皮后屏障,主要指黏膜下丰富的毛细血管及其提供的,又称血管屏障。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "食管黏液" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "食管黏膜" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "上皮前屏障" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "黏液" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "碳酸氢根" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "胃蛋白酶" }, { "start_idx": 63, "end_idx": 70, "type": "bod", "entity": "H+" }, { "start_idx": 84, "end_idx": 87, "type": "bod", "entity": "上皮屏障" }, { "start_idx": 99, "end_idx": 106, "type": "bod", "entity": "多层鳞状上皮细胞" }, { "start_idx": 118, "end_idx": 122, "type": "bod", "entity": "上皮后屏障" }, { "start_idx": 127, "end_idx": 128, "type": "bod", "entity": "黏膜" }, { "start_idx": 133, "end_idx": 136, "type": "bod", "entity": "毛细血管" }, { "start_idx": 145, "end_idx": 148, "type": "bod", "entity": "血管屏障" } ] }, { "text": "当食管黏膜屏障防御机制不全时,胃酸和胃蛋白酶以及十二指肠反流物——胆酸及胰液刺激食管,损伤黏膜,引起反流性食管炎、Barrett食管甚至食管腺癌。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "食管黏膜" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "胃酸" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "胃蛋白酶" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "十二指肠反流物" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "胆酸" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "胰液" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "食管" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "黏膜" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "反流性食管炎" }, { "start_idx": 57, "end_idx": 65, "type": "dis", "entity": "Barrett食管" }, { "start_idx": 68, "end_idx": 71, "type": "dis", "entity": "食管腺癌" } ] }, { "text": "近来有研究表明,食管黏膜的损伤程度与每一次反流的时间长短密切相关,时间越长损伤程度越深。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "食管黏膜" } ] }, { "text": "(四)其他1.胃排空功能目前认为餐后胃排空延迟可使胃内容量增大,胃内压增高,从而刺激胃酸分泌并使LES腹内功能区长度缩短,同时可诱发TLESR参与GERD的发病。", "entities": [ { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "胃" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "胃" }, { "start_idx": 32, "end_idx": 34, "type": "ite", "entity": "胃内压" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "胃酸分泌" }, { "start_idx": 66, "end_idx": 70, "type": "dis", "entity": "TLESR" }, { "start_idx": 73, "end_idx": 76, "type": "dis", "entity": "GERD" } ] }, { "text": "文献报道大约有50%的GERD患儿同时伴有胃排空延迟。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "GERD" } ] }, { "text": "2.药物影响阿司匹林和其他非甾体类抗炎药物(NSAIDS)对黏膜都具有侵蚀性。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 13, "end_idx": 20, "type": "dru", "entity": "非甾体类抗炎药物" }, { "start_idx": 22, "end_idx": 27, "type": "dru", "entity": "NSAIDS" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "黏膜" } ] }, { "text": "流行病学研究提示,服用这类药物可引发GERD。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "GERD" } ] }, { "text": "有食管狭窄的患者尤其易感NASIDS引发的食管损伤。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "食管" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "NASIDS" } ] }, { "text": "而没有食管狭窄的患者,NASIDS引发GERD的机制尚不明了。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "食管" }, { "start_idx": 11, "end_idx": 16, "type": "dru", "entity": "NASIDS" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "GERD" } ] }, { "text": "【临床表现】(一)临床症状GERD的临床表现轻重不一,随年龄而不同。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "GERD" } ] }, { "text": "新生儿常表现为喷射状呕吐乳汁或奶块;婴幼儿则表现反复呕吐,严重的可导致营养不良和生长发育迟缓;年长儿可自诉反酸或餐后及平卧时有酸性液体反流至口腔。", "entities": [ { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "喷射状呕吐乳汁或奶块" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "反复呕吐" }, { "start_idx": 29, "end_idx": 45, "type": "sym", "entity": "严重的可导致营养不良和生长发育迟缓" }, { "start_idx": 53, "end_idx": 71, "type": "sym", "entity": "反酸或餐后及平卧时有酸性液体反流至口腔" } ] }, { "text": "这是一种位于胸骨后的不适或烧灼样感觉,多起源于上腹部,放射至胸部甚至咽喉部或背部烧灼样感觉,多起源于上腹部,放射至胸部甚至咽喉部或背部。", "entities": [ { "start_idx": 4, "end_idx": 39, "type": "sym", "entity": "位于胸骨后的不适或烧灼样感觉,多起源于上腹部,放射至胸部甚至咽喉部或背部" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "上腹部" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "胸部" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "咽喉部" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "背部" } ] }, { "text": "(二)并发症1.食管炎及其后遗症这是GERD最主要的并发症,它的发生与LESP异常及食管廓清能力减弱密切相关。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "食管炎" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "GERD" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "LESP异常" }, { "start_idx": 42, "end_idx": 49, "type": "sym", "entity": "食管廓清能力减弱" } ] }, { "text": "由于反流物不断地刺激食管壁而令其充血水肿,年长儿会感到胸骨下烧灼痛,胸闷饱胀,甚至吞咽困难或疼痛,严重的还可发生呕血、黑便及贫血可发生呕血、黑便及贫血。", "entities": [ { "start_idx": 2, "end_idx": 19, "type": "sym", "entity": "反流物不断地刺激食管壁而令其充血水肿" }, { "start_idx": 21, "end_idx": 32, "type": "sym", "entity": "年长儿会感到胸骨下烧灼痛" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "胸闷饱胀" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "吞咽困难或疼痛" }, { "start_idx": 49, "end_idx": 63, "type": "sym", "entity": "严重的还可发生呕血、黑便及贫血" } ] }, { "text": "除了反流因素外,幽门螺杆菌(H.pylori)的感染也可促进Barrett食管的发生。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 14, "end_idx": 21, "type": "mic", "entity": "H.pylori" }, { "start_idx": 30, "end_idx": 38, "type": "dis", "entity": "Barrett食管" } ] }, { "text": "内镜下见到大段红色和丝绒样质地的柱状上皮从胃食管交界处向上延伸,与临近苍白、光滑的鳞状上皮形成鲜明对比为其特征性内镜表现。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "内镜" }, { "start_idx": 3, "end_idx": 30, "type": "sym", "entity": "见到大段红色和丝绒样质地的柱状上皮从胃食管交界处向上延伸" }, { "start_idx": 32, "end_idx": 59, "type": "sym", "entity": "与临近苍白、光滑的鳞状上皮形成鲜明对比为其特征性内镜表现" } ] }, { "text": "Barrett上皮不引起症状,因此大多数患者仅有GERD的基本表现,甚至并无GERD症状。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "Barrett" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "GERD" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "GERD" } ] }, { "text": "但它是胃食管交界处发生腺癌的重要危险因素,发病率较正常人群高30~50倍。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "腺癌" } ] }, { "text": "2.呼吸道症状有文献报道,胃食管反流是儿童反复、慢性咳嗽的主要因素之一。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "呼吸道" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 19, "end_idx": 27, "type": "sym", "entity": "儿童反复、慢性咳嗽" } ] }, { "text": "国内对哮喘患儿的胃食管反流研究显示,哮喘儿的各项反流指标均高于对照组,其病理性GER39%。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "哮喘" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "哮喘" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "病理性GE" }, { "start_idx": 36, "end_idx": 40, "type": "ite", "entity": "病理性GE" } ] }, { "text": "各种原因的哮喘患者都易发生GER,而GER又可诱发或加剧哮喘的发生。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "哮喘" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "GER" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "GER" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "哮喘" } ] }, { "text": "【诊断】对于有典型病史的患者,如自诉有典型的胃灼热、反酸,且经抑酸治疗迅速好转的,GERD的诊断即可成立。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "胃灼热" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "反酸," }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "GERD" } ] }, { "text": "钡餐可显示食管炎的征象,如食管壁的糜烂、溃疡及狭窄糜烂、溃疡及狭窄,还可显示钡剂的反流从而提示反流程度。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "钡餐" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "食管炎" }, { "start_idx": 13, "end_idx": 24, "type": "sym", "entity": "食管壁的糜烂、溃疡及狭窄" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "钡剂的反流" } ] }, { "text": "但钡餐对食管炎的诊内镜检查度不如内镜检查,内镜检查不仅可以直观黏膜损伤情况,还可从任何异常部位取活体组织检查。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "钡餐" }, { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "食管炎" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "内镜检查" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "内镜检查" } ] }, { "text": "另外,24小时食管pH监测则是一种在诊断GERD中具有更高灵敏性、特异性,且更方便、快捷、先进的方法。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "ite", "entity": "食管pH监测" } ] }, { "text": "大量文献报道,该方法弥补了症状分析及内镜检查的局限性,对鉴别生理性与病理性GER,深入了解GER与食管炎的关系,特别是对GERD的诊断与疗效判定提供了可靠的依据。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "内镜检查" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "GER" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "GER" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "食管炎" } ] }, { "text": "【治疗】GERD的治疗一般根据症状的轻重不同可分为非系统性治疗、系统性内科治疗和外科手术治疗。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "GERD" } ] }, { "text": "目的在于加强食管的抗反流防御机制,减少胃食管反流;减缓症状,预防和治疗并发症以及防止复发。", "entities": [ { "start_idx": 4, "end_idx": 15, "type": "pro", "entity": "加强食管的抗反流防御机制" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "减少胃食管反流" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "减缓症状" }, { "start_idx": 30, "end_idx": 43, "type": "pro", "entity": "预防和治疗并发症以及防止复发" } ] }, { "text": "(一)非系统性治疗对于症状较轻、无器质性病变的患儿可采用保守疗法,通过改变饮食和体位来达到治疗目的。", "entities": [ { "start_idx": 35, "end_idx": 48, "type": "pro", "entity": "改变饮食和体位来达到治疗目的" } ] }, { "text": "在此基础上如仍有症状可服用抗酸剂制酸剂、黏膜保护剂及促胃动力药。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "抗酸剂" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "制酸剂" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "黏膜保护剂" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "促胃动力药" } ] }, { "text": "1.抑制酸分泌药(1)H2受体阻滞剂:它能阻断组胺与壁细胞膜上H2受体结合,从而减少胃酸分泌,减少反流物的酸度和量。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "抑制酸分泌药" }, { "start_idx": 11, "end_idx": 23, "type": "dru", "entity": "H2受体阻滞剂" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "组胺" }, { "start_idx": 32, "end_idx": 46, "type": "bod", "entity": "壁细胞膜上H2受体" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "减少胃酸分泌" }, { "start_idx": 59, "end_idx": 68, "type": "sym", "entity": "减少反流物的酸度和量" } ] }, { "text": "临床上常用的有西咪替丁、雷尼替丁和法莫替丁等。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "西咪替丁" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "雷尼替丁" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "法莫替丁" } ] }, { "text": "(2)质子泵抑制剂:它通过抑制壁细胞上的H+-K+-ATP酶活力阻断胃酸的分泌。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "质子泵抑制剂" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "壁细胞" }, { "start_idx": 20, "end_idx": 29, "type": "bod", "entity": "H+-K+-ATP酶" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "胃酸" } ] }, { "text": "目前认为,质子泵抑制剂能更快地缓解反流症状,加速反流性食管炎的愈合,尤其对中重度食管炎及其并发症,此药应作为首选。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "质子泵抑制剂" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "反流性食管炎" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "中重度食管炎" } ] }, { "text": "有研究证实,质子泵抑制剂在成人中长期使用(1年以上)能有效控制GERD并且安全。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "质子泵抑制剂" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "GERD" } ] }, { "text": "在儿童,曾有研究人员对患有GERD的弱智儿童群体长期随访,证实该类药物对各种程度的反流性食管炎都相当有效,且未发现不良反应。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "GERD" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "反流性食管炎" } ] }, { "text": "由此可见,质子泵抑制剂是一种有效且安全的GERD治疗药。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "质子泵抑制剂" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "GERD" } ] }, { "text": "2.黏膜保护剂常用的为铝碳酸镁。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "黏膜保护剂" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "铝碳酸镁" } ] }, { "text": "其独特的网络状结构,不仅可以迅速中和胃酸,还能吸附胆汁,对胃酸和胆汁反流引起的症状均有较好的疗效。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "胃酸" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "胆汁" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "胃酸" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "胆汁" } ] }, { "text": "另外,临床上还经常使用硫糖铝及蒙脱石散,能增加黏膜对酸的抵抗力及促进黏膜上皮皮的修复。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "硫糖铝" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "蒙脱石散" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "黏膜" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "黏膜上皮" } ] }, { "text": "3.促胃动力药GERD是一种上消化道动力障碍性疾病,因此,对GERD的治疗首先应该改善消化道动力。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "GERD" }, { "start_idx": 14, "end_idx": 24, "type": "dis", "entity": "上消化道动力障碍性疾病" } ] }, { "text": "(1)甲氧氯普胺:为周围及中枢神经系统多巴胺受体拮抗剂,能促进内源性乙酰胆碱的释放,增加食管收缩幅度并促进胃排空。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "甲氧氯普胺" }, { "start_idx": 13, "end_idx": 26, "type": "dru", "entity": "中枢神经系统多巴胺受体拮抗剂" }, { "start_idx": 31, "end_idx": 37, "type": "bod", "entity": "内源性乙酰胆碱" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "食管" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "胃" } ] }, { "text": "但因其对神经系统副作用明显,故临床上逐渐少用。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "神经系统" } ] }, { "text": "(2)多潘立酮:此药为外周多巴胺受体拮抗剂,能促进胃排空,协调胃、十二指肠运动,增强食管蠕动和LES张力。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "多潘立酮" }, { "start_idx": 11, "end_idx": 20, "type": "dru", "entity": "外周多巴胺受体拮抗剂" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "胃" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "胃" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "十二指肠" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "食管" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "LES" } ] }, { "text": "该药对血-脑屏障渗透力差,对脑内多巴胺受体几乎无抑制作用,故无精神与神经不良反应,但1岁以下婴儿血-脑屏障功能发育尚不完全,仍应慎用。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "血-脑屏障" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "多巴胺受体" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "血-脑屏障" } ] }, { "text": "(3)西沙比利:为第三代胃肠动力药。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "西沙比利" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "胃肠动力药" } ] }, { "text": "它通过促进胃肠道肌层神经丛副交感神经节后纤维乙酰胆碱释放来加强食管、胃、小肠及结肠的推进性运动,加快胃肠道排空,增加食管下端括约肌张力。", "entities": [ { "start_idx": 5, "end_idx": 21, "type": "bod", "entity": "胃肠道肌层神经丛副交感神经节后纤维" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "乙酰胆碱" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "食管" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "胃" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "小肠" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "结肠" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "胃肠道" }, { "start_idx": 58, "end_idx": 64, "type": "bod", "entity": "食管下端括约肌" } ] }, { "text": "(三)抗反流手术儿科GERD需要进行手术治疗的比较少见,大约仅占5%~15%,这些患儿往往是由于食管外症状,如反复吸入性肺炎及窒息等呼吸道症状,才需要手术治疗。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "抗反流手术" }, { "start_idx": 8, "end_idx": 9, "type": "dep", "entity": "儿科" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "GERD" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "吸入性肺炎" } ] }, { "text": "当前,抗反流手术的方式很多,国外开展最多的是Nissan胃底折叠术。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "抗反流手术" }, { "start_idx": 22, "end_idx": 32, "type": "pro", "entity": "Nissan胃底折叠术" } ] }, { "text": "其机制是人工造成一个加强的食管下端高压区以利抵抗胃内容物反流。", "entities": [ { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "胃" } ] }, { "text": "另外,近年来利用腹腔镜腔镜下行Nissan胃底折叠术日益增多。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "equ", "entity": "腹腔镜" }, { "start_idx": 15, "end_idx": 25, "type": "pro", "entity": "Nissan胃底折叠术" } ] }, { "text": "Lobe和Schier分别在1993和1994年报道了小儿GERD在腹腔镜下的Nissan术。", "entities": [ { "start_idx": 34, "end_idx": 36, "type": "equ", "entity": "腹腔镜" }, { "start_idx": 39, "end_idx": 45, "type": "pro", "entity": "Nissan术" } ] }, { "text": "理论上,腹腔镜下胃底折叠术有手术更安全、损伤更小以及恢复时间更快等优点,但对它的远期疗效尚有争议。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "pro", "entity": "腹腔镜下胃底折叠术" } ] }, { "text": "有研究显示,这种方法的远期疗效无论从临床上还是各种检查上,都显示出很高的失败率,尤其在重度GERD患者中。", "entities": [ { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "GERD" } ] }, { "text": "然而,这一技术无疑为小儿GERD的治疗开辟了新途径,并且随着这一新技术的日益成熟,它必将在GERD治疗中发挥重要作用。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "小儿GERD" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "GERD" } ] }, { "text": "参考文献1.BredaL,NozziM,DeSanctisS,etal.Laboratorytestsinthediagnosisandfollow-upofpediatricrheumaticdiseases:anupdate.SeminArthritisRheum.2010,40(1):53-532.MokMY.TheimmunologicalbasisofB-celltherapyinsystemiclupuserythematosus.IntRheumDis.2010,13(1):3-33.陈瑜,王宏伟,周建华.儿童系统性红斑狼疮国内外诊断标准比较.实用儿科临床杂志,2007,22(9):715-7154.BehrmanRE,KliegmanRM,JensonHB.NelsonTextbookofPediatrics.16thed.Philadelphia:W.B.SaunersCo,20005.杨锡强等主编.儿童免疫学.北京:人民卫生出版社,20016.PeterM.Izmirly,CarolinaLlanos,LelaA.Lee,etal.Cutaneousmanifestationsofneonatallupusandriskofsubsequentcongenitalheartblock.Arthritis&Rheumatism,2010,62(4):1153-1153", "entities": [ { "start_idx": 265, "end_idx": 271, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 284, "end_idx": 285, "type": "dep", "entity": "儿科" } ] }, { "text": "第六章中枢神经系统肿瘤一、概述中枢神经系统肿瘤是小儿时期比较常见的肿瘤之一,其发生率在15岁以下儿童肿瘤中占据第二位。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "中枢神经系统肿瘤" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "中枢神经系统肿瘤" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "肿瘤" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "美国1973~1989年流行病学调查中发现,每年10万个儿童中就有约2.8例患颅内肿瘤。", "entities": [ { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "颅内肿瘤" } ] }, { "text": "颅内肿瘤好发年龄主要在二个年龄段,第一高峰在10岁以内,发病率为2.2/10万~2.5/10万人口/年,男性稍多于女性(1.1∶1)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内肿瘤" } ] }, { "text": "第二高峰从30~40岁开始,60岁以上达到顶点,这一阶段为肿瘤的最好发时期。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "肿瘤" } ] }, { "text": "【病因】中枢神经系统肿瘤尚未发现确切的病因,但是目前根据某些肿瘤发病特点、病理以及一些基础实验研究,提出几种学说。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "中枢神经系统肿瘤" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "肿瘤" } ] }, { "text": "1.遗传学说在神经外科领域中,某些肿瘤具有明显的家族倾向性,如视网膜母细胞瘤、血管网织细胞瘤、多发性神经纤维瘤等,一般认为它们均为常染色体显性遗传性肿瘤,外显率很高。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dep", "entity": "神经外科" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "肿瘤" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "视网膜母细胞瘤" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "血管网织细胞瘤" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "常染色体" }, { "start_idx": 74, "end_idx": 75, "type": "dis", "entity": "肿瘤" } ] }, { "text": "2.病毒学说实验研究表明,一些病毒包括DNA病毒和RNA病毒,若接种于动物脑内可诱发脑瘤。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "mic", "entity": "DNA病毒" }, { "start_idx": 25, "end_idx": 29, "type": "mic", "entity": "RNA病毒" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "脑瘤" } ] }, { "text": "3.理化学说物理因素中被确认的具有致肿瘤可能的是放射线,已有许多关于头颅放疗后引起颅内肿瘤的报道。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "肿瘤" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "头颅放疗" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "颅内肿瘤" } ] }, { "text": "4.免疫抑制学说器官移植免疫抑制剂的应用,会增加颅内或外周肿瘤发生的风险。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "器官移植" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "颅内或外周肿瘤" } ] }, { "text": "5.胚胎残余学说颅咽管瘤、上皮样及皮样囊肿、畸胎瘤、脊索瘤明显发生于残留于脑内的胚胎组织,这些残余组织具有增殖分化的潜力,在一定条件下可发展为肿瘤。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "颅咽管瘤" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "上皮样及皮样囊肿" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "畸胎瘤" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "脊索瘤" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "脑内" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "胚胎组织" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "肿瘤" } ] }, { "text": "【分类】中枢神经系统颅内肿瘤分类方法很多,比较具有代表性的主要有以下几种(见表11-11)。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "dis", "entity": "中枢神经系统颅内肿瘤" } ] }, { "text": "Balley和Cushing的分类能反映肿瘤组织的来源及恶性程度,长期以来被世界多数地区的神经外科和病理科所采用。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "肿瘤组织" }, { "start_idx": 45, "end_idx": 48, "type": "dep", "entity": "神经外科" }, { "start_idx": 50, "end_idx": 52, "type": "dep", "entity": "病理科" } ] }, { "text": "在此基础上衍生出一些新的分类方法,如Kernohan的Ⅰ~Ⅳ级分类法,Russell的胶质瘤分类。", "entities": [ { "start_idx": 35, "end_idx": 45, "type": "dis", "entity": "Russell的胶质瘤" } ] }, { "text": "但此分类法也有缺陷,如同一肿瘤在不同部位,细胞分化即可不同,有些混合瘤无法分级,而有些肿瘤分级意义不大。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "肿瘤" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "肿瘤" } ] }, { "text": "在此基础上世界卫生组织制定了新的分类法,其特点是分类细致,包括了颅内发生的各种肿瘤类型,吸收了既往各种分类的特点和长处,既反映了肿瘤的形态学,又表明肿瘤的来源,并采用间变这一概念,可认为是目前最好的分类。", "entities": [ { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "颅内" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "肿瘤" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "肿瘤" }, { "start_idx": 74, "end_idx": 75, "type": "dis", "entity": "肿瘤" } ] }, { "text": "【临床表现】中枢神经系统肿瘤的发病机制及其临床表现,具有缓慢进行性加重的特征。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "中枢神经系统肿瘤" }, { "start_idx": 26, "end_idx": 37, "type": "sym", "entity": "具有缓慢进行性加重的特征" } ] }, { "text": "不同的年龄,不同部位的肿瘤,其临床表现各不相同。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "肿瘤" } ] }, { "text": "1.颅内压增高中枢神经系统肿瘤分类比较肿瘤压迫、浸润脑组织、阻塞脑脊液增高头痛呕吐、嗜睡及视神经乳头水肿间隙性头痛学习成绩下降、易疲劳及性格改变。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "颅内压" }, { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "中枢神经系统肿瘤" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "脑组织" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "脑脊液" }, { "start_idx": 19, "end_idx": 36, "type": "sym", "entity": "肿瘤压迫、浸润脑组织、阻塞脑脊液增高" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "头" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "头痛" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "呕吐" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "视神经乳头" }, { "start_idx": 42, "end_idx": 51, "type": "sym", "entity": "嗜睡及视神经乳头水肿" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "头" }, { "start_idx": 52, "end_idx": 56, "type": "sym", "entity": "间隙性头痛" }, { "start_idx": 57, "end_idx": 62, "type": "sym", "entity": "学习成绩下降" }, { "start_idx": 65, "end_idx": 71, "type": "sym", "entity": "疲劳及性格改变" } ] }, { "text": "在婴幼儿期多表现激惹、食欲下降、生长发育延迟甚至退步、头围异常增大骨缝分离及两眼太阳落山征神经系统定位症状。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "激惹" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "食欲下降" }, { "start_idx": 16, "end_idx": 25, "type": "sym", "entity": "生长发育延迟甚至退步" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "头" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "头围异常增大" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "骨" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "两眼" }, { "start_idx": 33, "end_idx": 44, "type": "sym", "entity": "骨缝分离及两眼太阳落山征" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "神经系统" } ] }, { "text": "续表2.癫痫癫痫是颅内肿瘤常见的症状之一,大约1/4的儿童幕上肿瘤是以癫痫为首发症状,其发生率仅次于头痛,位居第二。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "癫痫" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "癫痫" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "儿童幕上肿瘤" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "癫痫" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "头痛" } ] }, { "text": "出现以下情况应高度怀疑颅内占位性病变的存在:有癫痫史的病人其癫痫类型发生了改变;癫痫持续难治性癫痫伴有局部神经系统损害的癫痫癫痫以及多数难以解释的全身发作性癫痫需做CT或MRI检查,了解有无颅内占位。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "颅内" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "癫痫" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "癫痫" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "癫痫持续" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "癫痫" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "难治性癫痫" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "神经系统" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "癫痫" }, { "start_idx": 49, "end_idx": 61, "type": "sym", "entity": "伴有局部神经系统损害的癫痫" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "癫痫" }, { "start_idx": 78, "end_idx": 79, "type": "dis", "entity": "癫痫" }, { "start_idx": 82, "end_idx": 83, "type": "pro", "entity": "CT" }, { "start_idx": 85, "end_idx": 89, "type": "pro", "entity": "MRI检查" }, { "start_idx": 95, "end_idx": 96, "type": "bod", "entity": "颅内" } ] }, { "text": "3.局部症状因肿瘤所在部位和大小而异。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "肿瘤" } ] }, { "text": "若大脑半球肿瘤接近中央前回者可有对侧偏瘫;在左侧额下回后部者可有运动性失语;蝶鞍区肿瘤可有视神经原发性萎缩及视力视野改变;肿瘤影响垂体-视丘下部可有生长发育紊乱、肥胖或消瘦、多饮、多尿及体温调节障碍;幕下肿瘤多有走路不稳、眼球震颤肌张力及肌腱反射减退脑干部位肿瘤则有一侧脑神经损害及对侧锥体束征(交叉性麻痹)松果体区肿瘤可有眼球上视障碍及性早熟等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "大脑半球" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "有对侧偏瘫" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "额下回后部" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "有运动性失语" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "蝶鞍" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "肿瘤" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "视神经" }, { "start_idx": 44, "end_idx": 55, "type": "sym", "entity": "有视神经原发性萎缩及视力" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "视野改变" }, { "start_idx": 61, "end_idx": 62, "type": "dis", "entity": "肿瘤" }, { "start_idx": 65, "end_idx": 71, "type": "bod", "entity": "垂体-视丘下部" }, { "start_idx": 73, "end_idx": 79, "type": "sym", "entity": "有生长发育紊乱" }, { "start_idx": 81, "end_idx": 85, "type": "sym", "entity": "肥胖或消瘦" }, { "start_idx": 87, "end_idx": 88, "type": "sym", "entity": "多饮" }, { "start_idx": 90, "end_idx": 98, "type": "sym", "entity": "多尿及体温调节障碍" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "幕下" }, { "start_idx": 102, "end_idx": 103, "type": "dis", "entity": "肿瘤" }, { "start_idx": 105, "end_idx": 109, "type": "sym", "entity": "有走路不稳" }, { "start_idx": 111, "end_idx": 112, "type": "bod", "entity": "眼球" }, { "start_idx": 111, "end_idx": 114, "type": "sym", "entity": "眼球震颤" }, { "start_idx": 119, "end_idx": 120, "type": "bod", "entity": "肌腱" }, { "start_idx": 115, "end_idx": 124, "type": "sym", "entity": "肌张力及肌腱反射减退" }, { "start_idx": 125, "end_idx": 126, "type": "bod", "entity": "脑干" }, { "start_idx": 129, "end_idx": 130, "type": "dis", "entity": "肿瘤" }, { "start_idx": 135, "end_idx": 137, "type": "bod", "entity": "脑神经" }, { "start_idx": 133, "end_idx": 153, "type": "sym", "entity": "一侧脑神经损害及对侧锥体束征(交叉性麻痹)" }, { "start_idx": 154, "end_idx": 157, "type": "bod", "entity": "松果体区" }, { "start_idx": 158, "end_idx": 159, "type": "dis", "entity": "肿瘤" }, { "start_idx": 161, "end_idx": 172, "type": "sym", "entity": "有眼球上视障碍及性早熟等" } ] }, { "text": "第十一篇儿童常见肿瘤0~15岁儿童恶性肿瘤发病率只占全部肿瘤病人的一小部分,在工业发达地区约占2%。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肿瘤" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "儿童恶性肿瘤" } ] }, { "text": "但在儿童死亡原因中,恶性肿瘤却高达10%,是主要原因之一。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "第一章儿童恶性肿瘤的流行病学一、常用的流行病学术语比、百分比与率是最常用的流行病学术语。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "儿童恶性肿瘤" } ] }, { "text": "比(ratio)说明两个数字之间的关系,如儿童急性白血病(acuteleukemia)男女发病例数比为869∶695,或1.29∶1。", "entities": [ { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "儿童急性白血病" }, { "start_idx": 29, "end_idx": 41, "type": "dis", "entity": "acuteleukemia" } ] }, { "text": "率(rate)表示在一定期限内危险人群中的事件发生数,即发病率(incidence),也是一种成分比,肿瘤发病率常以百万计算,如某地区在3960740个0~14岁人口中1年内共有514例新发儿童肿瘤,即该地区0~14岁儿童年发病率为514/3960740约129.77/百万。", "entities": [ { "start_idx": 95, "end_idx": 98, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "第十一章婴儿猝死综合征【概述】婴儿猝死综合征(suddeninfantdeathsyndrome,SIDS)是指婴儿时期突然死亡,死亡前后均不能从病史、症状体征甚至死亡后尸检等各种检查中得到相关疾病的诊断。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "婴儿猝死综合征" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "婴儿猝死综合征" }, { "start_idx": 23, "end_idx": 47, "type": "dis", "entity": "suddeninfantdeathsyndrome" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "SIDS" } ] }, { "text": "据近年来的大量研究认为,SIDS并非由单一因素所致,而是由小儿发育、周围环境及多种病理生理因素造成。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "SIDS" } ] }, { "text": "目前研究仍未能揭示整个发病过程,仅在一些解剖和生理方面发现这些患儿存在一些问题,主要集中在肺部、脑干及一些神经功能方面(图6-6)。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "解剖" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "肺部" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "脑干" } ] }, { "text": "图6-6SIDS发病机制尽管常规病理解剖不能发现明显的致死原因,但从死者解剖研究仍可发现SIDS患者存在一些病理征象,如轻度肺水肿、肺淤血和皮肤瘀点、瘀斑。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "SIDS" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "解剖" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "SIDS" }, { "start_idx": 62, "end_idx": 62, "type": "bod", "entity": "肺" }, { "start_idx": 62, "end_idx": 64, "type": "sym", "entity": "肺水肿" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "肺" }, { "start_idx": 66, "end_idx": 68, "type": "sym", "entity": "肺淤血" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "皮肤" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "皮肤瘀点" }, { "start_idx": 75, "end_idx": 76, "type": "sym", "entity": "瘀斑" } ] }, { "text": "研究证实有2/3死者存在慢性窒息,这些患者脑脊液中内皮细胞生长因子低于正常婴儿。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "慢性窒息" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "脑脊液" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "内皮细胞" } ] }, { "text": "SIDS患者被发现存在脑干神经结构和神经递质异常。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "SIDS" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "脑干神经结构" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "神经递质" } ] }, { "text": "异常包括局灶性星形胶质细胞增生、树突状棘突及髓鞘发育不良、髓内星形细胞反应性增多。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "树突状棘突" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "髓鞘" }, { "start_idx": 0, "end_idx": 39, "type": "sym", "entity": "异常包括局灶性星形胶质细胞增生、树突状棘突及髓鞘发育不良、髓内星形细胞反应性增多" } ] }, { "text": "位于延髓心血管呼吸中枢,与唤醒、自主神经及化学感受神经有关的反射弧发育不良,该部位的神经受体亦存在功能低下和受体量减少,如钾通道受体、毒蕈碱胆碱能受体等,二氧化碳、血压等其他神经感受器亦受累及。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "bod", "entity": "延髓心血管呼吸中枢" }, { "start_idx": 61, "end_idx": 65, "type": "bod", "entity": "钾通道受体" }, { "start_idx": 67, "end_idx": 74, "type": "bod", "entity": "毒蕈碱胆碱能受体" }, { "start_idx": 82, "end_idx": 83, "type": "ite", "entity": "血压" }, { "start_idx": 0, "end_idx": 95, "type": "sym", "entity": "位于延髓心血管呼吸中枢,与唤醒、自主神经及化学感受神经有关的反射弧发育不良,该部位的神经受体亦存在功能低下和受体量减少,如钾通道受体、毒蕈碱胆碱能受体等,二氧化碳、血压等其他神经感受器亦受累及" } ] }, { "text": "此外,脑干区域、迷走神经核及脑干网状结构的酪氨酸羟化酶改变亦提示肾上腺素和去甲肾上腺素神经功能异常。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "脑干" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "迷走神经核" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "脑干" }, { "start_idx": 21, "end_idx": 26, "type": "bod", "entity": "酪氨酸羟化酶" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "去甲肾上腺素" } ] }, { "text": "近期临床研究主要集中于SIDS的危险因素。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "SIDS" } ] }, { "text": "基础研究方面,除解剖生理外,基因研究主要就心脏离子通道异常基因(钠通道、钾通道)、五羟色胺转运基因(5-HTT)以及自主神经系统发育和炎症反应的某些基因调控。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "心脏" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "钠" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "钾" }, { "start_idx": 41, "end_idx": 48, "type": "bod", "entity": "五羟色胺转运基因" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "5-HTT" }, { "start_idx": 58, "end_idx": 63, "type": "bod", "entity": "自主神经系统" }, { "start_idx": 67, "end_idx": 68, "type": "dis", "entity": "炎症" } ] }, { "text": "表6-14SIDS的高危因素【临床表现】本病发病年龄高峰为生后第2~4个月,早产儿为1~2个月,以后发病率减少。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "SIDS" } ] }, { "text": "前驱可有呼吸道感染和发热病史。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "发热" } ] }, { "text": "本病一般于午夜至清晨时段发病,患儿起病前常无明显、烦躁不适症状,在睡眠中呼吸、心跳突然停止。", "entities": [ { "start_idx": 39, "end_idx": 40, "type": "ite", "entity": "心跳" }, { "start_idx": 15, "end_idx": 44, "type": "sym", "entity": "患儿起病前常无明显、烦躁不适症状,在睡眠中呼吸、心跳突然停止" } ] }, { "text": "过度出汗提示存在发热、过度约束或存在自主神经功能缺陷。", "entities": [ { "start_idx": 0, "end_idx": 25, "type": "sym", "entity": "过度出汗提示存在发热、过度约束或存在自主神经功能缺陷" } ] }, { "text": "很难在制定SIDS,其内容和项目除应尽可能精确鉴别和找出那些最终会死于SIDS的患儿,也应考虑到表格的有效性和实用性,必须忽略假阴性和允许存在一定程度的假阳性率。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "SID" }, { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "SID" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "终会死于" } ] }, { "text": "此外,呼吸描记图(pneumogram)和多导睡眠图(polysomnogram)可用于持续观察呼吸形态和心跳异常情况,但目前研究未证实其有足够的敏感度和特异性,临床上尚不能作为SIDS的筛查方法。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "equ", "entity": "呼吸描记图" }, { "start_idx": 9, "end_idx": 18, "type": "equ", "entity": "pneumogram" }, { "start_idx": 21, "end_idx": 25, "type": "equ", "entity": "多导睡眠图" }, { "start_idx": 27, "end_idx": 39, "type": "equ", "entity": "polysomnogram" }, { "start_idx": 53, "end_idx": 54, "type": "ite", "entity": "心跳" }, { "start_idx": 89, "end_idx": 92, "type": "dis", "entity": "SIDS" } ] }, { "text": "亦未证实有早产儿呼吸暂停史婴儿的SIDS危险度高于无呼吸暂停的适于胎龄儿。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "SIDS" } ] }, { "text": "关于SIDS的鉴别诊断见图6-7。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "SIDS" } ] }, { "text": "图6-7SIDS的鉴别诊断【治疗】关于SIDS的防治,至今尚无可行的有效干预方法和用于初生婴儿生后SIDS的发病的预测方法。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "SIDS" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "SIDS" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "SIDS" } ] }, { "text": "心电节律、呼吸类型以及自主神经系统的异常变化至今未能找出可用于临床观察的敏感监测指标,因而无法对SIDS病情作出即时和准确的判断,也无法制定相应的针对性干预措施。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "心电节律" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "自主神经系统" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "SIDS" } ] }, { "text": "虽然部分患者在电子监护中发现存在QT延长性心律失常,但对婴儿患者尚未建立统一的安全治疗规范。", "entities": [ { "start_idx": 16, "end_idx": 24, "type": "dis", "entity": "QT延长性心律失常" } ] }, { "text": "咖啡因和茶碱已用于治疗早产儿呼吸暂停。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "咖啡因" }, { "start_idx": 4, "end_idx": 5, "type": "dru", "entity": "茶碱" } ] }, { "text": "在成年人中,咖啡因可降低听觉唤醒阈值,但在婴儿尚无相应研究报道。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "咖啡因" } ] }, { "text": "亦无该药会增加SIDS危险度的有关报道。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "SIDS" } ] }, { "text": "【预防】由于SIDS在临床观察上存在困难,目前主要针对有高危因素的婴儿进行临床保护性干预。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "SIDS" } ] }, { "text": "部分欧美国家报道,通过这些措施后,近10年SIDS发病率下降了0.5个百分点。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "SIDS" } ] }, { "text": "表6-15SIDS预防措施(美国儿科学会)(陆铸今)", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "SIDS" } ] }, { "text": "二、通气功能所谓通气是指肺泡气体与外环境进行气体交换的过程(图8-8)。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肺泡" } ] }, { "text": "图8-8最大用力流速-容量环VOKYNE:容积,FLOW:流速,TLC:肺总量,RV:残气量,FIF50:50%肺活量时的吸气流速,PEF:呼气峰流速,MEF75(FEF25):75%肺活量时呼气流速;MEF50(FEF50):50%肺活量时的呼流速;MEF25(FEF75):25%肺活量时的呼气速(一)每分通气量(MV)是指每分钟呼出或吸入的气量,即潮气与呼吸频率的乘积。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "ite", "entity": "VOKYNE" }, { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "容积" }, { "start_idx": 24, "end_idx": 27, "type": "ite", "entity": "FLOW" }, { "start_idx": 29, "end_idx": 30, "type": "ite", "entity": "流速" }, { "start_idx": 32, "end_idx": 34, "type": "ite", "entity": "TLC" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "肺总量" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "RV" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "残气量" }, { "start_idx": 47, "end_idx": 62, "type": "ite", "entity": "FIF50" }, { "start_idx": 67, "end_idx": 69, "type": "ite", "entity": "肺活量" }, { "start_idx": 77, "end_idx": 79, "type": "ite", "entity": "PEF" }, { "start_idx": 81, "end_idx": 85, "type": "ite", "entity": "呼气峰流速" }, { "start_idx": 87, "end_idx": 102, "type": "ite", "entity": "MEF75" }, { "start_idx": 104, "end_idx": 119, "type": "ite", "entity": "FEF25" }, { "start_idx": 125, "end_idx": 127, "type": "ite", "entity": "肺活量" }, { "start_idx": 134, "end_idx": 149, "type": "ite", "entity": "MEF50" }, { "start_idx": 151, "end_idx": 166, "type": "ite", "entity": "FEF50" }, { "start_idx": 172, "end_idx": 174, "type": "ite", "entity": "肺活量" }, { "start_idx": 181, "end_idx": 196, "type": "ite", "entity": "MEF25" }, { "start_idx": 198, "end_idx": 213, "type": "ite", "entity": "FEF75" }, { "start_idx": 219, "end_idx": 221, "type": "ite", "entity": "肺活量" }, { "start_idx": 230, "end_idx": 234, "type": "ite", "entity": "每分通气量" }, { "start_idx": 236, "end_idx": 237, "type": "ite", "entity": "MV" }, { "start_idx": 254, "end_idx": 255, "type": "ite", "entity": "潮气" }, { "start_idx": 257, "end_idx": 260, "type": "ite", "entity": "呼吸频率" } ] }, { "text": "足月儿每分通气量140~220ml/kg,相当于3500~4000ml/m2,与成人相似。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "每分通气量" } ] }, { "text": "(二)肺泡通气量(VA)在静息状态下每分钟吸入气量中能到达肺泡进行有效气体交换的通气量为:(潮气量-无效腔气量)×呼吸频率,足月儿是100~150ml/kg。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "肺泡通气量" }, { "start_idx": 9, "end_idx": 21, "type": "ite", "entity": "VA" }, { "start_idx": 29, "end_idx": 35, "type": "ite", "entity": "每分钟吸入气量" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "肺泡" }, { "start_idx": 57, "end_idx": 59, "type": "ite", "entity": "潮气量" }, { "start_idx": 61, "end_idx": 65, "type": "ite", "entity": "无效腔气量" }, { "start_idx": 68, "end_idx": 71, "type": "ite", "entity": "呼吸频率" } ] }, { "text": "(三)最大通气量(MVV)是指在单位时间内以最深最快的呼吸所能达到的最大通气量,通常以每分钟计算。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "最大通气量" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "MVV" }, { "start_idx": 34, "end_idx": 38, "type": "ite", "entity": "最大通气量" } ] }, { "text": "(四)时间肺活量(又称用力肺活量,FVC)是指深吸气至肺总量,然后用力快速呼气直至残气位时测得的肺活量。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "时间肺活量" }, { "start_idx": 11, "end_idx": 15, "type": "ite", "entity": "用力肺活量" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "FVC" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "肺总量" }, { "start_idx": 48, "end_idx": 50, "type": "ite", "entity": "肺活量" } ] }, { "text": "婴幼儿无法配合,故有人研究用啼哭肺活量(CVC)作为婴幼儿FVC指标。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "ite", "entity": "啼哭肺活量" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "CVC" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "FVC" } ] }, { "text": "测定第1秒时间内呼出的气量称为第一秒用力呼气量(FEV1)或简称一秒量,用FEV1除以最大肺活量即为一秒率,一秒量和秒率是常规肺功能检测中反映气道阻塞最重要的两个指标。", "entities": [ { "start_idx": 15, "end_idx": 22, "type": "ite", "entity": "第一秒用力呼气量" }, { "start_idx": 24, "end_idx": 38, "type": "ite", "entity": "FEV1" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "一秒量" }, { "start_idx": 48, "end_idx": 62, "type": "ite", "entity": "FEV1" }, { "start_idx": 65, "end_idx": 69, "type": "ite", "entity": "最大肺活量" }, { "start_idx": 72, "end_idx": 74, "type": "ite", "entity": "一秒率" }, { "start_idx": 76, "end_idx": 78, "type": "ite", "entity": "一秒量" }, { "start_idx": 80, "end_idx": 81, "type": "ite", "entity": "秒率" }, { "start_idx": 83, "end_idx": 89, "type": "pro", "entity": "常规肺功能检测" }, { "start_idx": 93, "end_idx": 94, "type": "bod", "entity": "气道" }, { "start_idx": 93, "end_idx": 96, "type": "sym", "entity": "气道阻塞" } ] }, { "text": "(五)呼气峰流速(PEF)即呼气相最高流速。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "呼气峰流速" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "PEF" }, { "start_idx": 14, "end_idx": 20, "type": "ite", "entity": "呼气相最高流速" } ] }, { "text": "在呼气中,流速与肺弹性回缩力以及气道阻力有关。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "流速" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肺" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "气道" } ] }, { "text": "最有意义的是呼气峰流速,其与年龄、身高、体重、胸围等均有关系,尤与身高关系密切。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "呼气峰流速" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "年龄" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "身高" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "体重" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "胸围" }, { "start_idx": 33, "end_idx": 34, "type": "ite", "entity": "身高" } ] }, { "text": "在阻塞性疾病(如婴幼儿哮喘),患者由于气道痉挛、痰液阻塞、小气道提早关闭,故PEF下降。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "阻塞性疾病" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "婴幼儿哮喘" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "气道" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "气道痉挛" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "痰液" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "痰液阻塞" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "小气道" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "小气道提早关闭" }, { "start_idx": 38, "end_idx": 40, "type": "ite", "entity": "PEF" } ] }, { "text": "PEF还存在昼夜波动,据此结合临床症状,可对哮喘进行分级:PEF昼夜波动率=(日内最高PEF-日内最低PEF)/1/2(同日内最高PEF+最低PEF)×100%哮喘患儿发作时此值往往大于15%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "PEF" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "PEF还存在昼夜波动" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "哮喘" }, { "start_idx": 29, "end_idx": 36, "type": "ite", "entity": "PEF昼夜波动率" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "PEF" }, { "start_idx": 51, "end_idx": 53, "type": "ite", "entity": "PEF" }, { "start_idx": 65, "end_idx": 67, "type": "ite", "entity": "PEF" }, { "start_idx": 71, "end_idx": 73, "type": "ite", "entity": "PEF" }, { "start_idx": 80, "end_idx": 81, "type": "dis", "entity": "哮喘" } ] }, { "text": "(六)其他以下一些流速指标有助于我们了解小气道功能(图8-8)。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "流速" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "小气道" } ] }, { "text": "MEF75(FEF25):75%肺活量时的呼气流速;MEF50(FEF50):50%肺活量时的呼气流速;MEF25(FEF75):75%肺活量时的呼气流速;及最大呼气中期流速(MMEF25~75)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "MEF75" }, { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "FEF25" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "肺活量" }, { "start_idx": 26, "end_idx": 30, "type": "ite", "entity": "MEF50" }, { "start_idx": 32, "end_idx": 36, "type": "ite", "entity": "FEF50" }, { "start_idx": 42, "end_idx": 44, "type": "ite", "entity": "肺活量" }, { "start_idx": 52, "end_idx": 56, "type": "ite", "entity": "MEF25" }, { "start_idx": 58, "end_idx": 62, "type": "ite", "entity": "FEF75" }, { "start_idx": 68, "end_idx": 70, "type": "ite", "entity": "肺活量" }, { "start_idx": 88, "end_idx": 96, "type": "ite", "entity": "MMEF25~75" } ] }, { "text": "随着肺活量的逐渐减少,MEF越能反映出小气道的情况。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "肺活量" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "MEF" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "小气道" } ] }, { "text": "参考文献1.BehrmanRE,etal.NelsonTextbookofPediatrics.16thed,2000:15772.杨霁云,白克敏主编.小儿肾脏病临床与基础.北京:人民卫生出版社,2000:1983.林善锬主编.当代肾脏病学.上海:上海科技教育出版社,2001:313", "entities": [ { "start_idx": 84, "end_idx": 86, "type": "dis", "entity": "肾脏病" }, { "start_idx": 122, "end_idx": 124, "type": "dis", "entity": "肾脏病" } ] }, { "text": "第六节三尖瓣闭锁三尖瓣闭锁(tricuspidatresia)是一种不多见的心血管畸形,占所有先心病的1.3%~1.7%。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "三尖瓣闭锁" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "三尖瓣闭锁" }, { "start_idx": 14, "end_idx": 29, "type": "dis", "entity": "tricuspidatresia" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "心血管畸形" } ] }, { "text": "【病理解剖】右心房和右心室被闭锁的三尖瓣完全隔开,多为肌性闭锁,少数为纤维型。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "右心房" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "右心室" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "肌性闭锁" } ] }, { "text": "右心室常发育不良流出道组成,还包括发育幼稚的肌小梁成分。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "右心室常发育不良" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "流出道" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肌小梁" } ] }, { "text": "若室间隔完整,右心室发育极其不全甚至缺如,伴肺动脉瓣闭锁;反之,若存在大的室间隔缺损右心室则发育良好。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "室间隔" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "右心室" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "室间隔" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "室间隔缺损" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "右心室" } ] }, { "text": "本病心房间的交通必然存在,它是小儿存活必不可少的条件,通常属于非限制型,可以是卵圆孔未闭、继发性房间隔缺损或者是原发性房间隔缺损伴完全型房室隔缺损。", "entities": [ { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "卵圆孔未闭" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "房间隔" }, { "start_idx": 45, "end_idx": 52, "type": "sym", "entity": "继发性房间隔缺损" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "房间隔" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "房室隔" }, { "start_idx": 56, "end_idx": 72, "type": "sym", "entity": "原发性房间隔缺损伴完全型房室隔缺损" } ] }, { "text": "心室大动脉连接位置可以正常,亦可转位。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "心室大动脉" } ] }, { "text": "当存在大动脉转位心血管畸形左上腔静脉、水肿、动脉导管未闭肺动脉为非限制型,则可见到主动脉发育不良水肿。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "动脉" }, { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "大动脉转位" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "心血管畸形" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "左上腔静脉" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "水肿" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "动脉" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "动脉导管未闭" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "肺动脉" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "主动脉" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "主动脉发育不良" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "水肿" } ] }, { "text": "其他伴随畸形尚有左右心耳并置永存动脉干、部分型房室间隔缺损。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心耳" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "左右心耳并置" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "永存动脉干" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "房室" }, { "start_idx": 20, "end_idx": 28, "type": "sym", "entity": "部分型房室间隔缺损" } ] }, { "text": "肺血流量的多寡取决于肺动脉狭窄程度,从而产生相应的临床症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "肺血流量" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺动脉" } ] }, { "text": "当动脉位置正常,室间隔缺损太小会引起肺血管梗阻大动脉转位梗阻常由于肺动脉下及肺动脉瓣狭窄造成。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "动脉" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肺" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血管" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "肺血管梗阻" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "动脉" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "大动脉转位" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "梗阻" } ] }, { "text": "若伴有肺动脉瓣闭锁肺血流量取决于动脉导管未闭和(或)主肺动脉间形成的侧支大小。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肺动脉" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "肺动脉瓣闭锁" }, { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "肺血流量" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "动脉" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "主肺动脉" } ] }, { "text": "【病理生理】本病心房间交通形成了血液被动性的右向左分流。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心房" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "血液" } ] }, { "text": "当房间隔缺损太小,血流受阻,右心房压力增高而使体静脉回血及肺血流量减少心搏出量减少青紫严重;若房间隔缺损大,其病理生理学改变主要依赖于肺血管流出道梗阻情况,即肺血流量的多少。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "房间隔" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "血流受阻" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "右心房" }, { "start_idx": 29, "end_idx": 32, "type": "ite", "entity": "肺血流量" }, { "start_idx": 14, "end_idx": 34, "type": "sym", "entity": "右心房压力增高而使体静脉回血及肺血流量减少" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "心" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "心搏出量减少" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "青紫严重" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "房间隔" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "肺血管" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "流出道" }, { "start_idx": 79, "end_idx": 82, "type": "ite", "entity": "肺血流量" } ] }, { "text": "当肺动脉显著狭窄青紫,随着室间隔缺损不断变小、圆锥部渐进性变窄或动脉导管的变细,青紫可进一步加重,若肺血量极度减少时,可发生缺氧发作;相反,若肺血管流出道正常,肺血流量会增多,大多表现为心功能不全发绀不明显,这种患儿常伴有大动脉转位肺血管梗阻性疾病及肺高压。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "肺动脉" }, { "start_idx": 1, "end_idx": 7, "type": "sym", "entity": "肺动脉显著狭窄" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "青紫" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "室间隔" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "圆锥部" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "动脉" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "青紫" }, { "start_idx": 50, "end_idx": 52, "type": "ite", "entity": "肺血量" }, { "start_idx": 71, "end_idx": 76, "type": "bod", "entity": "肺血管流出道" }, { "start_idx": 80, "end_idx": 83, "type": "ite", "entity": "肺血流量" }, { "start_idx": 93, "end_idx": 93, "type": "bod", "entity": "心" }, { "start_idx": 93, "end_idx": 97, "type": "sym", "entity": "心功能不全" }, { "start_idx": 98, "end_idx": 102, "type": "sym", "entity": "发绀不明显" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "动脉" }, { "start_idx": 111, "end_idx": 115, "type": "sym", "entity": "大动脉转位" }, { "start_idx": 116, "end_idx": 123, "type": "dis", "entity": "肺血管梗阻性疾病" }, { "start_idx": 125, "end_idx": 127, "type": "dis", "entity": "肺高压" } ] }, { "text": "【临床表现】(一)肺血增多大动脉转位常会出现肺血流过多呼吸急促喂养困难、出汗多、活动量减少、反复肺部感染青紫较轻甚至无发绀。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肺血" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "肺血增多" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "动脉" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "大动脉转位" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肺" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "肺血流过多" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "呼吸" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "喂养困难" }, { "start_idx": 36, "end_idx": 38, "type": "sym", "entity": "出汗多" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "活动量减少" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "肺部" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "反复肺部感染" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "青紫" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "发绀" } ] }, { "text": "此类患儿在生后2~3个月可发展为充血性心力衰竭,主要表现为心脏扩大肝大呼吸困难听诊可及第三心音、舒张期隆隆样杂音,收缩期杂音多不明显。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "心脏" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "肝" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "肝大" }, { "start_idx": 35, "end_idx": 36, "type": "ite", "entity": "呼吸" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "听诊可及第三心音" }, { "start_idx": 48, "end_idx": 55, "type": "sym", "entity": "舒张期隆隆样杂音" }, { "start_idx": 57, "end_idx": 65, "type": "sym", "entity": "收缩期杂音多不明显" } ] }, { "text": "(二)肺血减少大多数患儿在新生儿期即可出现发绀或心脏杂音青紫最常见于中央型青紫,其严重程度与肺血管流出道梗阻程度有关,如肺血流进一步减少,则在婴儿时期就出现缺氧发作。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "肺血减少" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "心脏" }, { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "发绀或心脏杂音" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "青紫" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "中央型青紫" }, { "start_idx": 46, "end_idx": 53, "type": "sym", "entity": "肺血管流出道梗阻" }, { "start_idx": 60, "end_idx": 60, "type": "bod", "entity": "肺" }, { "start_idx": 78, "end_idx": 79, "type": "sym", "entity": "缺氧" } ] }, { "text": "年长儿中可表现为呼吸急促活动后蹲踞。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "呼吸" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "活动后蹲踞" } ] }, { "text": "严重青紫及有红细胞增多症脑血管栓塞,尤其在铁质缺乏时更是如此。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "青紫" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "红细胞" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "红细胞增多症" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "脑血管栓塞" } ] }, { "text": "体格检查:小儿出现中至重度中央型青紫,年长儿杵状指(趾)明显心前区隆起心尖搏动增强听诊第一心音单一肺动脉狭窄、肺动脉瓣闭锁或伴大动脉转位,则第二心音单一喀喇音偶可于胸骨左缘闻及肺动脉流出道狭窄或限制性室间隔缺损而产生的收缩期喷射性杂音。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "体格检查" }, { "start_idx": 9, "end_idx": 17, "type": "sym", "entity": "中至重度中央型青紫" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "趾" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "杵状指(趾)明显" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "心前区" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "心前区隆起" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "心尖" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "搏动" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "心尖搏动增强" }, { "start_idx": 41, "end_idx": 42, "type": "ite", "entity": "听诊" }, { "start_idx": 41, "end_idx": 48, "type": "sym", "entity": "听诊第一心音单一" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "肺动脉瓣闭锁" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 72, "end_idx": 73, "type": "ite", "entity": "心音" }, { "start_idx": 70, "end_idx": 75, "type": "sym", "entity": "第二心音单一" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "胸骨" }, { "start_idx": 76, "end_idx": 87, "type": "sym", "entity": "喀喇音偶可于胸骨左缘闻及" }, { "start_idx": 88, "end_idx": 95, "type": "dis", "entity": "肺动脉流出道狭窄" }, { "start_idx": 97, "end_idx": 104, "type": "dis", "entity": "限制性室间隔缺损" }, { "start_idx": 109, "end_idx": 116, "type": "sym", "entity": "收缩期喷射性杂音" } ] }, { "text": "随着肺动脉流出道梗阻加重或室间隔缺损的缩小,杂音强度可逐渐降低。", "entities": [ { "start_idx": 22, "end_idx": 30, "type": "sym", "entity": "杂音强度可逐渐降低" } ] }, { "text": "如颈静脉搏动有明显“a”波、肝脏收缩前心房间存在限制性分流。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肝脏" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "肝脏收缩" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心房" } ] }, { "text": "【辅助检查】(一)胸部X线肺血流量少者,肺野肺血减少,肺动脉干凹陷心胸比值正常或轻度增大右房缘凸出明显肺血流量增加、有心力衰竭的小儿,其显著特征为心脏扩大、肺充血。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "胸部X线" }, { "start_idx": 13, "end_idx": 16, "type": "ite", "entity": "肺血流量" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "肺动脉干" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "肺动脉干凹陷" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "心" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "胸" }, { "start_idx": 33, "end_idx": 43, "type": "sym", "entity": "心胸比值正常或轻度增大" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "右房缘" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "右房缘凸出明显" }, { "start_idx": 51, "end_idx": 54, "type": "ite", "entity": "肺血流量" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 78, "end_idx": 78, "type": "bod", "entity": "肺" }, { "start_idx": 78, "end_idx": 80, "type": "sym", "entity": "肺充血" } ] }, { "text": "(二)心电图该病心电图特点是:QRS额面电轴偏左上,右心房肥大,右心室电势减小或消失(图9-9)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "心电图" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "心电图" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "右心房" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "右心室" } ] }, { "text": "如有大型室间隔缺损,右心室发育较好,右心室电压可变得明显。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "右心室" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "右心室" } ] }, { "text": "当肺血流量多,左心室容量负荷增加,左胸导联示ST段压低,T波改变,并可见V6深Q波、R波高尖。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "ite", "entity": "肺血流量" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "左心室" } ] }, { "text": "图9-35三尖瓣闭锁患儿(2月)典型心电图显示:电轴左偏,右房肥大和右、心室低电压。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "三尖瓣闭锁" }, { "start_idx": 18, "end_idx": 20, "type": "pro", "entity": "心电图" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "右房" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "右、心室" }, { "start_idx": 29, "end_idx": 40, "type": "sym", "entity": "右房肥大和右、心室低电压" } ] }, { "text": "(三)超声心动图剑突下及心尖四腔位可清楚捕捉到三尖瓣缺如和右心室发育不良心房间交通的大小,结合Doppler显像估计左右心房间的压力阶差,从而了解心房间的分流程度。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "心尖四腔位" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "右心室" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "右心室发育不良" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "心房" }, { "start_idx": 47, "end_idx": 55, "type": "pro", "entity": "Doppler显像" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "心房" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "心房" } ] }, { "text": "若房间隔向左房膨出则表明左右心房分流受限。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "房间隔" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "左房" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心房" } ] }, { "text": "二维超声心动图同时尚能看出室间隔缺损的大小、心室大动脉连接和肺动脉流出道梗阻的情况。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "二维超声心动图" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "心室" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "动脉" } ] }, { "text": "脉冲及连续Doppler还能明确肺动脉流出道收缩期压力阶差。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "脉冲" }, { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "连续Doppler" }, { "start_idx": 16, "end_idx": 24, "type": "dis", "entity": "肺动脉流出道收缩期" } ] }, { "text": "(四)心导管三尖瓣闭锁通常可通过超声心动图明确诊断,而在行Fontan手术之前,诊断性心导管术及心导管造影则有利于显示肺动脉解剖结构、肺动脉压力和阻力、左心室功能、二尖瓣活动情况和体肺静脉形态。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "心导管" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "三尖瓣闭锁" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "超声心动图" }, { "start_idx": 29, "end_idx": 36, "type": "pro", "entity": "Fontan手术" }, { "start_idx": 48, "end_idx": 52, "type": "pro", "entity": "心导管造影" }, { "start_idx": 59, "end_idx": 63, "type": "pro", "entity": "肺动脉解剖" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "肺动脉" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "左心室" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "体肺" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "静脉" } ] }, { "text": "心导管术还能看到大动脉转位、主动脉下狭窄及主动脉弓畸形。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "心导管术" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "主动脉下狭窄" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "主动脉弓畸形" } ] }, { "text": "行顺行静脉插管到达右心房。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "equ", "entity": "插管" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "右心房" } ] }, { "text": "若为限制性卵圆孔或房间隔缺损,右心房压力增高,两房间压力阶差显著,>3mm时记录右房压力曲线可见大的“a”波。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "房间隔" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "右心房" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "右房" } ] }, { "text": "利用漂浮导管,经心房间交通进入左心房、左心室。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "equ", "entity": "漂浮导管" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心房" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "左心房" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "左心室" } ] }, { "text": "在大动脉转位且无肺血管梗阻病例中,导管通常可经左心室插入肺动脉,如大血管连接正常,导管偶尔也可通过缺损的室间隔入发育不全的右心室,再直接进入肺动脉。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 17, "end_idx": 18, "type": "equ", "entity": "导管" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "左心室" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "肺动脉" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "血管" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "室间" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "右心室" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "肺动脉" } ] }, { "text": "但是,如肺血管流出道严重梗阻,这种方法可引起缺氧发作,操作需十分谨慎。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "sym", "entity": "肺血管流出道严重梗阻" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "缺氧" } ] }, { "text": "当导管无法插入肺动脉,平均肺动脉压可通过测肺静脉楔压得出。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肺动脉" }, { "start_idx": 13, "end_idx": 16, "type": "ite", "entity": "肺动脉压" }, { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "肺静脉楔压" } ] }, { "text": "肺动脉中度狭窄,其压力常正常;而肺动脉无狭窄,肺血流增多,肺血管阻力增高,肺高压形成,此种情况多出现在伴大动脉转位的该病中。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肺动脉" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "肺动脉" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "肺血流" }, { "start_idx": 29, "end_idx": 33, "type": "ite", "entity": "肺血管阻力" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "肺高压" }, { "start_idx": 52, "end_idx": 56, "type": "sym", "entity": "大动脉转位" } ] }, { "text": "心室大血管连接不一致,可行逆行动脉插管,直接越过主动脉瓣进入右心室;如有主-肺动脉分流,肺动脉分流,肺动脉还可通过主动脉或其分支进入。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心室" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血管" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "逆行动脉插管" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "右心室" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "主-肺动脉" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "肺动脉" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "主动脉" } ] }, { "text": "如同时伴有限制性室间隔缺损,右心室收缩期压力下降,低于左心室压力,左心室和升主动脉间存在压力阶差。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "限制性室间隔缺损" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "右心室" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "左心室" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "主动脉" } ] }, { "text": "但超声心动图诊断三尖瓣闭锁效果良好,一般很少行选择性右心房造影;左心室造影可见发育程度不同的右心室,像附着于前侧壁居中的一个憩室,还可清楚描绘出室间隔缺损、大动脉位置关系、肺血管流出道梗阻及肺动脉解剖结构(图9-9),左室舒张末期容积增加。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "pro", "entity": "超声心动图" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "三尖瓣闭锁" }, { "start_idx": 26, "end_idx": 30, "type": "pro", "entity": "右心房造影" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "左心室造影" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "右心室" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "动脉" }, { "start_idx": 86, "end_idx": 92, "type": "dis", "entity": "肺血管流出道梗" }, { "start_idx": 86, "end_idx": 92, "type": "pro", "entity": "肺血管流出道梗" }, { "start_idx": 91, "end_idx": 93, "type": "bod", "entity": "道梗阻" }, { "start_idx": 105, "end_idx": 106, "type": "bod", "entity": "-9" } ] }, { "text": "若伴有大动脉转位、限制性室间隔缺损,则需行主动脉造影,估计主动脉弓发育程度及水肿情况。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "限制性室间隔缺损" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "主动脉造影" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "水肿" } ] }, { "text": "图9-36左心室造影(左前斜位)显示:右心室发育不良和心室-大血管连接不一致,肺动脉已环缩Ao:主动脉LV:左心室PA:肺动脉RV:右心室【治疗】早期治疗原则为:保持心房间交通的通畅;保证一定量的肺血流。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "左心室造影" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "右心室" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "心室-大血管" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "肺动脉" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "主动脉" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "左心室" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "肺动脉" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "右心室" }, { "start_idx": 83, "end_idx": 84, "type": "bod", "entity": "心房" } ] }, { "text": "有严重低氧血症的新生儿,因其肺动脉血主要来源于未闭的动脉导管,故应静脉注射前列腺素E1或E2以维持动脉导管的开放,肺血流增加,体循环氧饱和度提高。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "低氧血症" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "肺动脉血" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "动脉导管" }, { "start_idx": 33, "end_idx": 45, "type": "pro", "entity": "静脉注射前列腺素E1或E2" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "动脉导管" }, { "start_idx": 57, "end_idx": 59, "type": "ite", "entity": "肺血流" }, { "start_idx": 63, "end_idx": 69, "type": "ite", "entity": "体循环氧饱和度" } ] }, { "text": "改良的Blalock-Taussig分流术避免了Waterston分流术(升主动脉-右肺动脉吻合)或Potts分流术(降主动脉-左肺动脉吻合)术后肺血流量过多肺动脉高压、心力衰竭、肺动脉扭曲等并发症。", "entities": [ { "start_idx": 3, "end_idx": 20, "type": "pro", "entity": "Blalock-Taussig分流术" }, { "start_idx": 24, "end_idx": 35, "type": "pro", "entity": "Waterston分流术" }, { "start_idx": 38, "end_idx": 45, "type": "bod", "entity": "主动脉-右肺动脉" }, { "start_idx": 50, "end_idx": 57, "type": "pro", "entity": "Potts分流术" }, { "start_idx": 60, "end_idx": 67, "type": "bod", "entity": "主动脉-左肺动脉" }, { "start_idx": 73, "end_idx": 76, "type": "ite", "entity": "肺血流量" }, { "start_idx": 73, "end_idx": 78, "type": "sym", "entity": "肺血流量过多" }, { "start_idx": 79, "end_idx": 83, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 85, "end_idx": 88, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 90, "end_idx": 94, "type": "dis", "entity": "肺动脉扭曲" } ] }, { "text": "患儿长至6个月以上,可行腔静脉-肺动脉双向分流术:切断右上腔静脉,远心端与右肺动脉上缘作端侧吻合,缝合近心端。", "entities": [ { "start_idx": 12, "end_idx": 23, "type": "pro", "entity": "腔静脉-肺动脉双向分流术" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "右上腔静脉" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "心端" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "右肺动脉" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "心端" } ] }, { "text": "此方案上腔静脉将体循环1/3血流送至左右肺动脉,而不增加左心室容量负荷,较改良的Blalock-Taussig分流术更符合生理学纠正标准,且可作为全腔静脉肺动脉连接术前的一期手术。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "体循环" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肺动脉" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "左心室" }, { "start_idx": 40, "end_idx": 57, "type": "pro", "entity": "Blalock-Taussig分流术" }, { "start_idx": 73, "end_idx": 82, "type": "pro", "entity": "全腔静脉肺动脉连接术" } ] }, { "text": "如伴左上腔静脉残存,若左无名静脉粗大,为防止血液流入冠状窦及右心房,术中需结扎左上腔静脉;若连接静脉很细或缺如,则应将上腔静脉两侧与肺动脉作双向分流。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "左上腔静脉" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "静脉" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "冠状窦" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "右心房" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "左上腔静脉" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "静脉" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "肺动脉" } ] }, { "text": "但在小于6个月的婴儿中,因其肺动脉细小,肺血管阻力尚高,此类手术不宜进行。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肺动脉" }, { "start_idx": 20, "end_idx": 24, "type": "ite", "entity": "肺血管阻力" } ] }, { "text": "上腔静脉-肺动脉连接术术后并发症包括静脉间侧支形成、肺动-静脉侧支形成,但其发生率大大少于标准Glenn手术(Glenn手术是将右肺动脉末端与上腔静脉作部分或完全性端侧吻合)。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "pro", "entity": "上腔静脉-肺动脉连接术" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "肺动-静脉侧支" }, { "start_idx": 47, "end_idx": 53, "type": "pro", "entity": "Glenn手术" }, { "start_idx": 55, "end_idx": 61, "type": "pro", "entity": "Glenn手术" }, { "start_idx": 64, "end_idx": 69, "type": "bod", "entity": "右肺动脉末端" }, { "start_idx": 71, "end_idx": 74, "type": "bod", "entity": "上腔静脉" } ] }, { "text": "半Fontan手术与上腔静脉-肺动脉双向分流术相似,它是将上腔静脉两个断端均与右肺动脉相连,右心房上腔静脉入口处以补片关闭,因而二期Fontan手术仅需移动该补片。", "entities": [ { "start_idx": 10, "end_idx": 22, "type": "pro", "entity": "上腔静脉-肺动脉双向分流术" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "右肺动脉" }, { "start_idx": 46, "end_idx": 52, "type": "bod", "entity": "右心房上腔静脉" }, { "start_idx": 57, "end_idx": 58, "type": "equ", "entity": "补片" }, { "start_idx": 66, "end_idx": 73, "type": "pro", "entity": "Fontan手术" }, { "start_idx": 79, "end_idx": 80, "type": "equ", "entity": "补片" } ] }, { "text": "对于存在肺血流增加,出现充血性心力衰竭的患儿,出生后3个月内以抗心力衰竭药物治疗,同时行肺动脉环缩术,防止肺高压、肺血管疾病的发展,控制心力衰竭症状。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "肺血流" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 44, "end_idx": 49, "type": "pro", "entity": "肺动脉环缩术" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "肺高压" }, { "start_idx": 68, "end_idx": 71, "type": "sym", "entity": "心力衰竭" } ] }, { "text": "伴有完全性大动脉转位的小儿,可有限制性室间隔缺损、主动脉瓣下狭窄,应切除主动脉瓣下组织,或实行Damus-Kaye-Stansel改良术或Norwood手术,建立肺动脉干-升主动脉吻合。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "完全性大动脉转位" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "限制性室间隔缺损" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "主动脉瓣下狭窄" }, { "start_idx": 34, "end_idx": 42, "type": "pro", "entity": "切除主动脉瓣下组织" }, { "start_idx": 47, "end_idx": 67, "type": "pro", "entity": "Damus-Kaye-Stansel改良术" }, { "start_idx": 69, "end_idx": 77, "type": "pro", "entity": "Norwood手术" }, { "start_idx": 81, "end_idx": 89, "type": "bod", "entity": "肺动脉干-升主动脉" } ] }, { "text": "Fontan手术:主要用于早期姑息性手术后仍存在低氧血症、出现活动量明显减少的患儿。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "Fontan手术" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "姑息性手术" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "低氧血症" } ] }, { "text": "手术适应证为:年龄大于2岁,平均肺动脉压力等于或小于15mmHg,肺血管阻力低(<2Wood单位/平方米),肺动脉粗无狭窄,左室收缩舒张功能良好,没有明显二尖瓣反流体静脉回血直接引入肺部,使静脉血不再汇入动脉血中,从而解决了体循环低氧血症的问题。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "ite", "entity": "肺动脉压力" }, { "start_idx": 33, "end_idx": 37, "type": "ite", "entity": "肺血管阻力" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "肺动脉" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "左室" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 77, "end_idx": 81, "type": "sym", "entity": "二尖瓣反流" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "体静脉" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "肺部" }, { "start_idx": 95, "end_idx": 97, "type": "bod", "entity": "静脉血" }, { "start_idx": 102, "end_idx": 104, "type": "bod", "entity": "动脉血" } ] }, { "text": "早期的手术方案有右心房-肺动脉直接吻合和右心房-右心室导管连接加室间隔缺损修补术两种。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "右心房-肺动脉" }, { "start_idx": 32, "end_idx": 39, "type": "pro", "entity": "室间隔缺损修补术" } ] }, { "text": "但这两种术式都会引起渐进性右心房扩大,导致体循环回流房性心律失常肺静脉回流潜在梗阻静-肺动脉双向分流;②右房内置膜片直接将下腔静脉与右肺动脉下壁连接。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "体循环" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "体循环回流" }, { "start_idx": 28, "end_idx": 29, "type": "ite", "entity": "心律" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "房性心律失常" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "肺静脉" }, { "start_idx": 32, "end_idx": 40, "type": "sym", "entity": "肺静脉回流潜在梗阻" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "静-肺动脉" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "右房" }, { "start_idx": 56, "end_idx": 57, "type": "equ", "entity": "膜片" }, { "start_idx": 66, "end_idx": 71, "type": "bod", "entity": "右肺动脉下壁" } ] }, { "text": "这避免了右心房扩大血液湍流减少心外将下腔静脉远心端与右肺动脉远心端相接,这种方法无须在心脏和肺动脉间建立旁道,可减少房性心律失常的发生。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "右心房" }, { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "右心房扩大" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "血液" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "血液湍流减少" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心外" }, { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "下腔静脉远心端" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "右肺动脉远心端" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "心脏" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "肺动脉" }, { "start_idx": 60, "end_idx": 61, "type": "ite", "entity": "心律" }, { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "房性心律失常" } ] }, { "text": "对于一些高危病人,应在右心房膜片或心外导管上作一开窗(fenestration),降低静脉系统压力及右向左分流,使体循环血液回流通畅,心排出量提高,明显改善患儿胸膜渗出、右心衰竭、心低排血量状况,以便病人度过术后危险期。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "equ", "entity": "右心房膜片" }, { "start_idx": 17, "end_idx": 20, "type": "equ", "entity": "心外导管" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "体循环" }, { "start_idx": 67, "end_idx": 70, "type": "ite", "entity": "心排出量" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "胸膜" }, { "start_idx": 85, "end_idx": 88, "type": "sym", "entity": "右心衰竭" }, { "start_idx": 90, "end_idx": 94, "type": "sym", "entity": "心低排血量" } ] }, { "text": "术后小儿可有轻度发绀。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "发绀" } ] }, { "text": "待心功能好转后,可于监护病房内缝合窗口或在心导管室用各种装置填塞(图9-9)。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "心导管室" } ] }, { "text": "图9-37Fontan手术后,心房间交通被Amplatzer房间隔缺损闭塞器堵塞术后应给予阿司匹林或其他抗凝剂防止静脉血栓形成。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "Fontan手术" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心房" }, { "start_idx": 21, "end_idx": 37, "type": "equ", "entity": "Amplatzer房间隔缺损闭塞器" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "抗凝剂" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "静脉血栓" } ] }, { "text": "多数病人还应服用利尿剂、ACEI、地高辛。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "利尿剂" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "ACEI" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "地高辛" } ] }, { "text": "血管扩张剂能降低外周血管阻力,减轻左心室后负荷,使肺血流量和心排出量增加。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "血管扩张剂" }, { "start_idx": 8, "end_idx": 13, "type": "ite", "entity": "外周血管阻力" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "左心室" }, { "start_idx": 25, "end_idx": 28, "type": "ite", "entity": "肺血流量" }, { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "心排出量" } ] }, { "text": "近年来,已证明NO可降低肺血管阻力,这提示在Fontan术后可能有内源性NO生成减少,对于这些病人,应通过治疗促进肺动脉内皮细胞释放NO。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "ite", "entity": "肺血管阻力" }, { "start_idx": 22, "end_idx": 28, "type": "pro", "entity": "Fontan术" } ] }, { "text": "【预后】本病的自然病程取决于肺动脉流出道梗阻情况、是否存在左室梗阻、有无左心室功能不全等因素。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "肺动脉流出道梗阻" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "左室梗阻" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "左心室功能不全" } ] }, { "text": "若体肺循环血流量平衡,患儿早期可存活,但如不治疗,90%的病人在10岁以前死亡,一些患者可活至20~30岁,不过长期左心室超负荷必然会引起左心室功能不全二尖瓣功能障碍心排出量减少心室大动脉连接不一致者,其病情变化较连接一致者更严重,部分患者同时伴有水肿或主动脉断离,不经治疗常在1岁以内夭折。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "ite", "entity": "体肺循环血流量" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "左心室" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "左心室" }, { "start_idx": 69, "end_idx": 75, "type": "sym", "entity": "左心室功能不全" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 76, "end_idx": 82, "type": "sym", "entity": "二尖瓣功能障碍" }, { "start_idx": 83, "end_idx": 86, "type": "ite", "entity": "心排出量" }, { "start_idx": 83, "end_idx": 88, "type": "sym", "entity": "心排出量减少" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "心室" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "动脉" }, { "start_idx": 124, "end_idx": 125, "type": "sym", "entity": "水肿" } ] }, { "text": "随着病源选择的严谨及手术技术的提高,Fontan术后的死亡率已低于10%,手术预后较功能性单心室更好。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "Fontan术" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "单心室" } ] }, { "text": "对复杂型畸形患儿,行腔静脉-肺动脉双向分流加右心房开窗术更能降低术后罹病率及死亡率,但术后早期仍可出现一些并发症,如胸腔积液、腹水、心包积液室上性心律失常心脏传导阻滞体循环静脉血栓形成心排出量减少等。", "entities": [ { "start_idx": 9, "end_idx": 27, "type": "pro", "entity": "行腔静脉-肺动脉双向分流加右心房开窗术" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "胸腔积液" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "腹水" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "心包" }, { "start_idx": 66, "end_idx": 69, "type": "sym", "entity": "心包积液" }, { "start_idx": 73, "end_idx": 74, "type": "ite", "entity": "心律" }, { "start_idx": 70, "end_idx": 76, "type": "sym", "entity": "室上性心律失常" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "心脏" }, { "start_idx": 77, "end_idx": 82, "type": "sym", "entity": "心脏传导阻滞" }, { "start_idx": 83, "end_idx": 85, "type": "bod", "entity": "体循环" }, { "start_idx": 83, "end_idx": 91, "type": "sym", "entity": "体循环静脉血栓形成" }, { "start_idx": 92, "end_idx": 97, "type": "sym", "entity": "心排出量减少" } ] }, { "text": "这可能与术后心脏结构重建、左心室功能变化引起心肌休整功能紊乱有关,术后早期使用血管扩张剂可有助于提高心排出量。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "心脏" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "左心室" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "心肌" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "血管扩张剂" }, { "start_idx": 50, "end_idx": 53, "type": "ite", "entity": "心排出量" } ] }, { "text": "大多数手术病人临床症状及运动量均有明显改善,体循环氧饱和度恢复正常,血栓栓塞、脑脓疡的发生率也减少。", "entities": [ { "start_idx": 22, "end_idx": 28, "type": "ite", "entity": "体循环氧饱和度" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "血栓栓塞" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "脑脓疡" } ] }, { "text": "但是,即使在一些没有临床症状的患儿中,也存在心排出量低于正常范围的现象,活动时体静脉压可进一步升高。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "心排出量" }, { "start_idx": 39, "end_idx": 42, "type": "ite", "entity": "体静脉压" } ] }, { "text": "长期的体静脉高压左室功能不全心房-肺动脉直接相连必然会使右心房逐渐扩大,易产生房性心律失常肺静脉梗阻血栓形成。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "静脉" }, { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "体静脉高压" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "左室" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "左室功能不全" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "心房-肺动脉" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "右心房" }, { "start_idx": 41, "end_idx": 42, "type": "ite", "entity": "心律" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "房性心律失常" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "肺静脉" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "肺静脉梗阻" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "血栓" } ] }, { "text": "除了饮食摄取及定期静脉滴注白蛋白外,其他根治性治疗方法效果仍不理想。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "静脉滴注白蛋白" } ] }, { "text": "排除肺动脉狭窄球囊扩张术或先置入stent补片,再行Fontan术,疗效颇佳;心房开窗术也可降低体静脉压力,但会加重青紫现象;还可选择腔静脉-肺动脉双向分流术或心脏移植手术。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺动脉" }, { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "肺动脉狭窄" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "球囊扩张术" }, { "start_idx": 16, "end_idx": 22, "type": "equ", "entity": "stent补片" }, { "start_idx": 26, "end_idx": 32, "type": "pro", "entity": "Fontan术" }, { "start_idx": 39, "end_idx": 43, "type": "pro", "entity": "心房开窗术" }, { "start_idx": 48, "end_idx": 52, "type": "ite", "entity": "体静脉压力" }, { "start_idx": 58, "end_idx": 59, "type": "pro", "entity": "青紫" }, { "start_idx": 67, "end_idx": 78, "type": "pro", "entity": "腔静脉-肺动脉双向分流术" }, { "start_idx": 80, "end_idx": 85, "type": "pro", "entity": "心脏移植手术" } ] }, { "text": "最近,在尚未产生明显体静脉高压肾上腺皮质激素和高分子肝素已获得成功,其作用机制目前仍不清楚。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "体静脉" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "体静脉高压" }, { "start_idx": 15, "end_idx": 21, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "高分子肝素" } ] }, { "text": "此外,其他晚期并发症有肝酶轻度升高、肝硬化凝血因子缺乏等,C蛋白、S蛋白及凝血因子Ⅶ的缺乏还可引起血栓栓塞。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "肝酶轻度升高" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肝" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "肝硬化" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "凝血因子缺乏" }, { "start_idx": 29, "end_idx": 44, "type": "sym", "entity": "C蛋白、S蛋白及凝血因子Ⅶ的缺乏" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "血栓栓塞" } ] }, { "text": "在Fontan术后长期随访的病人中,有些人出现心功能逐渐衰退的情况,有报道称术后早中期患者心功能不全以舒张功能不全为主。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "pro", "entity": "Fontan术" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "心功能逐渐衰退" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "心功能不全" }, { "start_idx": 51, "end_idx": 56, "type": "sym", "entity": "舒张功能不全" } ] }, { "text": "一份最新研究进一步证实,术后心肌持续工作能导致左室顺应性降低,从而引起肺动脉压力升高、肺淤血心排出量下降心功能衰退的机制之一。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心肌" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "左室" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "肺动脉压力升高" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "肺" }, { "start_idx": 43, "end_idx": 45, "type": "sym", "entity": "肺淤血" }, { "start_idx": 46, "end_idx": 49, "type": "ite", "entity": "心排出量" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "心排出量下降" }, { "start_idx": 52, "end_idx": 56, "type": "sym", "entity": "心功能衰退" } ] }, { "text": "总而言之,从后期并发症来看,Fontan手术仍是目前最佳的治疗手段。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "pro", "entity": "Fontan手术" } ] }, { "text": "有20%~25%的病人可通过后期手术或心导管干预而好转。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "equ", "entity": "心导管" } ] }, { "text": "第二节病毒性疾病的治疗病毒性疾病的发生和发展取决于病毒和机体两方面诸多因素,其治疗措施应包括针对病毒的抗病毒疗法,以及针对机体调节和提高其免疫功能,以清除细胞内病毒。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "病毒性疾病" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "病毒性疾病" }, { "start_idx": 25, "end_idx": 26, "type": "mic", "entity": "病毒" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "机体" }, { "start_idx": 48, "end_idx": 49, "type": "mic", "entity": "病毒" }, { "start_idx": 51, "end_idx": 55, "type": "pro", "entity": "抗病毒疗法" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "细胞" }, { "start_idx": 80, "end_idx": 81, "type": "mic", "entity": "病毒" } ] }, { "text": "由于很多急性病毒性疾病呈自限性,并缺乏特效的抗病毒药物,故综合对症处理常为其主要疗法。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "急性病毒性疾病" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "抗病毒药物" } ] }, { "text": "一、综合对症治疗主要包括:①一般处理:适宜休息,合理饮食,补充营养素,中枢神经系统感染时生命体征监护和呼吸管理等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "综合对症治疗" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "适宜休息" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "合理饮食" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "补充营养素" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "中枢神经系统感染" }, { "start_idx": 44, "end_idx": 49, "type": "pro", "entity": "生命体征监护" }, { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "呼吸管理" } ] }, { "text": "②对症治疗:如高温时退热,颅高压症时降低颅内压和止惊,止咳祛痰,降酶退黄等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "对症治疗" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "高温" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "退热" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "颅高压症" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "降低颅内压" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "止惊" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "止咳祛痰" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "降酶退黄" } ] }, { "text": "③保护脏器功能:如肝炎时的护肝治疗,脑炎时保护脑细胞处理。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "脏器" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肝炎" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "护肝治疗" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "脑炎" }, { "start_idx": 21, "end_idx": 27, "type": "pro", "entity": "保护脑细胞处理" } ] }, { "text": "④预防继发感染:有些病毒感染可抑制机体免疫功能,易继发细菌等其他病原感染如麻疹时,应注意预防。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "继发感染" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "病毒感染" }, { "start_idx": 27, "end_idx": 28, "type": "mic", "entity": "细菌" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "感染" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "麻疹" } ] }, { "text": "一般不主张预防性使用抗生素。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "抗生素" } ] }, { "text": "⑤恢复期康复治疗:主要在中枢神经系统感染有明显神经性损伤者如脑炎、脊髓炎时,应加强康复治疗,以减少或减轻后遗症。", "entities": [ { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "中枢神经系统感染" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "神经性损伤" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "脑炎" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "脊髓炎" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "康复治疗" } ] }, { "text": "三、真菌感染性口炎鹅口疮(thrush):念珠菌感染引起的口炎中以白色念珠菌致病力最强,儿童期感染常称之为鹅口疮。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "真菌感染性口炎" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "鹅口疮" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "thrush" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "念珠菌感染" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "口炎" }, { "start_idx": 33, "end_idx": 37, "type": "mic", "entity": "白色念珠菌" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "鹅口疮" } ] }, { "text": "念珠菌是人体常见的寄生菌,其致病力弱,仅在一定条件下感染致病,故为条件致病菌,近年来随着抗生素及肾上腺皮质激素的广泛应用,使念珠菌感染日益增多。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "念珠菌" }, { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "寄生菌" }, { "start_idx": 33, "end_idx": 37, "type": "mic", "entity": "条件致病菌" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "抗生素" }, { "start_idx": 48, "end_idx": 54, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "念珠菌感染" } ] }, { "text": "【病因】为白色念珠菌感染。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "白色念珠菌感染" } ] }, { "text": "诱因有营养不良、腹泻及长期使用抗生素、肾上腺皮质激素等,这些诱因加上乳具污染,便可引起鹅口疮。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "营养不良" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "腹泻" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "抗生素" }, { "start_idx": 19, "end_idx": 25, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "鹅口疮" } ] }, { "text": "【临床表现及诊断】鹅口疮的特点是口腔黏膜上出现白色乳凝块样物,分布于颊黏膜、舌、齿龈和上腭表面。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "鹅口疮" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "口腔黏膜" }, { "start_idx": 16, "end_idx": 29, "type": "sym", "entity": "口腔黏膜上出现白色乳凝块样物" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "颊黏膜" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "舌" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "齿龈" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "上腭" } ] }, { "text": "初起时呈小点状和小片状,渐融合成大片,不易擦去,若强行擦拭后局部潮红,可有溢血。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "初起时呈小点状和小片状,渐融合成大片" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "不易擦去" }, { "start_idx": 24, "end_idx": 33, "type": "sym", "entity": "若强行擦拭后局部潮红" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "可有溢血" } ] }, { "text": "患儿一般情况良好,无痛,不影响吃奶,偶有个别因累及消化道、呼吸道而出现呕吐、声嘶或呼吸困难鹅口疮的治疗,主要是用碱性药物及制霉菌素。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "患儿一般情况良好,无痛,不影响吃奶" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "消化道" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "呼吸道" }, { "start_idx": 18, "end_idx": 44, "type": "sym", "entity": "偶有个别因累及消化道、呼吸道而出现呕吐、声嘶或呼吸困难" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "鹅口疮" }, { "start_idx": 56, "end_idx": 59, "type": "dru", "entity": "碱性药物" }, { "start_idx": 61, "end_idx": 64, "type": "dru", "entity": "制霉菌素" } ] }, { "text": "局部治疗,因为口腔的碱性环境可抑制白色念珠菌的生长繁殖。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "口腔" }, { "start_idx": 17, "end_idx": 21, "type": "mic", "entity": "白色念珠菌" } ] }, { "text": "一般用2%碳酸氢钠清洗口腔后,局部涂抹2%甲紫或冰硼散,每日1~2次,数日后便可痊愈。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "口腔" }, { "start_idx": 21, "end_idx": 22, "type": "dru", "entity": "甲紫" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "冰硼散" } ] }, { "text": "若病变广泛者可用制霉菌素10万单位,加水1~2ml涂患处,每日3~4次。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "制霉菌素" } ] }, { "text": "第二节生理性贫血刚出生的正常足月新生儿红细胞计数可高达5×1012~7×1012/L,血红蛋白可达190~220g/L。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "生理性贫血" }, { "start_idx": 19, "end_idx": 23, "type": "ite", "entity": "红细胞计数" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "在无任何病理因素存在的情况下,出生后1周内红细胞计数与血红蛋白均逐渐下降,直至8周后方停止。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "红细胞计数" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "出生后2月~3月时,红细胞计数与血红蛋白含量降至最低点,前者可低至3×1012/L,后者可降至90g/L,呈现轻度贫血的表现。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "ite", "entity": "红细胞计数" }, { "start_idx": 16, "end_idx": 21, "type": "ite", "entity": "血红蛋白含量" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "轻度贫血" } ] }, { "text": "由于这种轻度贫血的过程是生理性的,故称生理性贫血(physiologicalanemia)。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "轻度贫血" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "生理性贫血" }, { "start_idx": 25, "end_idx": 43, "type": "dis", "entity": "physiologicalanemia" } ] }, { "text": "目前认为,这种贫血是胎儿出生后子宫外生命的生理性适应过程。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "贫血" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "子宫" } ] }, { "text": "(一)可能的发生机制①红细胞生成停止:婴儿出生后自主呼吸的建立,动脉血氧饱和度上升至95%时,红细胞生成作用迅速停止;②红细胞生成素(EPO)水平较低:可能是由于新生儿期EPO的产生部位在肝脏而非肾脏所致,因为肝脏EPO的释放对组织低氧相对不敏感,且EPO的半衰期缩短;③血液稀释:生后头3个月,体重迅速增加,血容量也相应增加,导致血液稀释而使红细胞计数和血红蛋白含量下降。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "红细胞" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "动脉" }, { "start_idx": 34, "end_idx": 38, "type": "ite", "entity": "血氧饱和度" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "红细胞" }, { "start_idx": 60, "end_idx": 65, "type": "bod", "entity": "红细胞生成素" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "EPO" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "EPO" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "肝脏" }, { "start_idx": 98, "end_idx": 99, "type": "bod", "entity": "肾脏" }, { "start_idx": 105, "end_idx": 106, "type": "bod", "entity": "肝脏" }, { "start_idx": 107, "end_idx": 109, "type": "bod", "entity": "EPO" }, { "start_idx": 125, "end_idx": 127, "type": "bod", "entity": "EPO" }, { "start_idx": 136, "end_idx": 137, "type": "bod", "entity": "血液" }, { "start_idx": 155, "end_idx": 157, "type": "ite", "entity": "血容量" }, { "start_idx": 166, "end_idx": 167, "type": "bod", "entity": "血液" }, { "start_idx": 172, "end_idx": 176, "type": "ite", "entity": "红细胞计数" }, { "start_idx": 178, "end_idx": 183, "type": "ite", "entity": "血红蛋白含量" } ] }, { "text": "(二)生理性贫血时机体的生理性适应过程当血红蛋白降至110g/L以下时,为了满足机体供氧需要,新生儿期血清2,3-二磷酸甘油醛(2,3-diphosphoglycocerate,2,3-DPG)增加,有利于正常成人型血红蛋白(HbA)中氧的释放,减少组织低氧的程度。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "生理性贫血" }, { "start_idx": 20, "end_idx": 23, "type": "ite", "entity": "血红蛋白" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "血清" }, { "start_idx": 53, "end_idx": 62, "type": "bod", "entity": "2,3-二磷酸甘油醛" }, { "start_idx": 64, "end_idx": 87, "type": "bod", "entity": "2,3-diphosphoglycocerate" }, { "start_idx": 89, "end_idx": 95, "type": "bod", "entity": "2,3-DPG" }, { "start_idx": 108, "end_idx": 111, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 113, "end_idx": 115, "type": "bod", "entity": "HbA" } ] }, { "text": "随着婴儿肾脏的不断成熟,肾脏分泌EPO的能力逐渐增加,红细胞生成作用也随之增加。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肾脏" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肾脏" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "EPO" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "红细胞" } ] }, { "text": "如无喂养不当以及其他病理因素存在的情况下,血红蛋白含量将于生后5~6个月恢复至110g/L以上。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "(三)未成熟儿的生理性贫血早产儿也可发生生理性贫血,发生因素与足月儿相同,但这些因素的作用更加明显,血红蛋白下降更快、更严重。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "生理性贫血" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "生理性贫血" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "血红蛋白最低可降至70~90g/L,通常发生于出生后3周~6周龄。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "与足月儿不同,未成熟儿EPO产生的代偿能力较差,致血红蛋白浓度的下降更加明显。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "EPO" }, { "start_idx": 25, "end_idx": 30, "type": "ite", "entity": "血红蛋白浓度" } ] }, { "text": "由于早产儿输入了含有HbA的成人血后,其氧离曲线向右漂移,有利于氧在组织中的释放。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "HbA" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "血" }, { "start_idx": 20, "end_idx": 23, "type": "ite", "entity": "氧离曲线" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "氧" } ] }, { "text": "因此,对早产儿贫血的定义以及需不需要输血,不仅应根据Hb水平,而且还要根据氧需要和婴儿循环中Hb释放氧的能力来决定。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "贫血" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "输血" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "Hb" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "氧" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "Hb" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "氧" } ] }, { "text": "(四)可加重生理性贫血的因素1.生理性贫血的程度可因生后早期出现严重贫血的各种溶血过程增加而加重。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "生理性贫血" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "生理性贫血" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "贫血" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "溶血" } ] }, { "text": "2.伴有EPO水平下降的情况如网织红细胞计数降低的低再生性(hyporegenerative)贫血、宫内输血的胎儿、一些伴有先天性支气管肺发育不良的婴儿等,均可以因EPO产生缺陷而发生贫血,因此,有必要对这些病人进行EPO治疗试验。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "EPO" }, { "start_idx": 25, "end_idx": 48, "type": "dis", "entity": "低再生性(hyporegenerative)贫血" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "宫内输血" }, { "start_idx": 62, "end_idx": 72, "type": "dis", "entity": "先天性支气管肺发育不良" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "EPO" }, { "start_idx": 92, "end_idx": 93, "type": "dis", "entity": "贫血" }, { "start_idx": 108, "end_idx": 110, "type": "bod", "entity": "EPO" } ] }, { "text": "3.营养饮食因素如缺铁、叶酸缺乏等。", "entities": [ { "start_idx": 10, "end_idx": 10, "type": "dru", "entity": "铁" }, { "start_idx": 12, "end_idx": 13, "type": "dru", "entity": "叶酸" } ] }, { "text": "但在生后3个月内,如无明显失血,不会发生缺铁性贫血。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "缺铁性贫血" } ] }, { "text": "对于出生时铁贮备足够的小婴儿来说,在体重未达到2倍于出生体重前,不会因食物中铁缺乏而致贫血。", "entities": [ { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "贫血" } ] }, { "text": "(五)治疗由于生理性贫血是正常发育过程中的一种表现,因此通常不需要治疗。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "生理性贫血" } ] }, { "text": "喂养良好、生长正常的早产婴儿如无明显医源性失血时极少需要输血。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "输血" } ] }, { "text": "健康早产婴儿即使Hb水平低至65g/L时通常也能很好地耐受,未必一定需要输血。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "ite", "entity": "Hb水平" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "输血" } ] }, { "text": "血细胞比容的监测是输红细胞的最佳观测指标。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "血细胞比容" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "输红细胞" } ] }, { "text": "红细胞输注似并不影响呼吸暂停(apneicspells)和心率缓慢的发作。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "红细胞输注" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "apneicspells" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "心率缓慢" } ] }, { "text": "国外报道,严重贫血的未成熟儿可用重组人EPO250IU/kg,每周3次,皮下注射或(和)铁剂如蔗糖铁6mg/kg,每周一次静脉注射,或硫酸亚铁6mg/(kg•24h),分3次口服治疗6周,经上述治疗者需要输血的次数明显减少,但应用EPO治疗的费用远比输血要高。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "严重贫血" }, { "start_idx": 16, "end_idx": 21, "type": "dru", "entity": "重组人EPO" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "皮下注射" }, { "start_idx": 44, "end_idx": 45, "type": "dru", "entity": "铁剂" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "蔗糖铁" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "静脉注射" }, { "start_idx": 67, "end_idx": 70, "type": "dru", "entity": "硫酸亚铁" }, { "start_idx": 102, "end_idx": 103, "type": "pro", "entity": "输血" }, { "start_idx": 115, "end_idx": 119, "type": "pro", "entity": "EPO治疗" }, { "start_idx": 125, "end_idx": 126, "type": "pro", "entity": "输血" } ] }, { "text": "二、新生儿的体格生长体格生长的常用指标新生儿的生长主要反映在体格方面,其衡量指标包括体重、身长、头围、胸围等指标。", "entities": [ { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "体重" }, { "start_idx": 45, "end_idx": 46, "type": "ite", "entity": "身长" }, { "start_idx": 48, "end_idx": 49, "type": "ite", "entity": "头围" }, { "start_idx": 51, "end_idx": 52, "type": "ite", "entity": "胸围" } ] }, { "text": "1.体重体重是机体各部重量的总和,这是体格生长的重要指标之一。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "体重" }, { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "体重" } ] }, { "text": "我国城市婴儿出生体重男婴为(3.32±0.39)kg,女婴为(3.21±0.36)kg(2005年9市城区正常男童和女童体格发育指标)。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "体重" } ] }, { "text": "出生后体重曲线本应是胎儿曲线的延续,但因初生数天内,新生儿摄入不足,胎粪及水分等的丢失,致使体重有下降趋势,大都在出生后3~4日降至最低点,可达出生体重的6%~9%生理性体重下降”。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "体重" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "摄入不足" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "胎粪及水分等的丢失" }, { "start_idx": 46, "end_idx": 47, "type": "ite", "entity": "体重" }, { "start_idx": 46, "end_idx": 81, "type": "sym", "entity": "体重有下降趋势,大都在出生后3~4日降至最低点,可达出生体重的6%~9%" }, { "start_idx": 82, "end_idx": 88, "type": "sym", "entity": "生理性体重下降" } ] }, { "text": "然而需要注意的是,一旦新生儿体重丢失超过出生体重的10%,或10天仍未恢复至出生时的体重,则应考虑为病理性或喂养不足所致。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "体重" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "体重" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "体重" } ] }, { "text": "一般来说,在7~10天,新生儿恢复到出生时体重,早产儿体重恢复较迟。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "体重" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "体重" } ] }, { "text": "以后新生儿体重就不断增加,年龄越小,体重的增加约25~30g体重要求用婴儿磅秤或特别的杠杆秤。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "体重" }, { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "体重" }, { "start_idx": 13, "end_idx": 29, "type": "sym", "entity": "年龄越小,体重的增加约25~30g" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "体重" }, { "start_idx": 35, "end_idx": 38, "type": "equ", "entity": "婴儿磅秤" }, { "start_idx": 43, "end_idx": 45, "type": "equ", "entity": "杠杆秤" } ] }, { "text": "称体重时应卧位,迅速调整游锤至杠杆正中水平,所示读数记录以kg为单位,至小数点后两位。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "体重" }, { "start_idx": 12, "end_idx": 13, "type": "equ", "entity": "游锤" }, { "start_idx": 15, "end_idx": 16, "type": "equ", "entity": "杠杆" } ] }, { "text": "2.身长身长代表着头、脊柱、下肢长度的总和。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "身长" }, { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "身长" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "头" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脊柱" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "下肢" } ] }, { "text": "身长在出生时平均约50cm。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "身长" } ] }, { "text": "新生儿测量身长用标准的量床或携带式量板。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "身长" } ] }, { "text": "坐高:是由头顶到坐骨结节的长度。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "坐高" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "头顶" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "坐骨结节" } ] }, { "text": "测量时取仰卧位,测量从头顶至臀部的距离,读刻度至0.1cm。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "头顶" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "臀部" } ] }, { "text": "新生儿坐高约占身长的66%。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "坐高" } ] }, { "text": "上部量和下部量:人体的全部长度以耻骨联合上缘为界可分为上、下两部分,上部分即从头顶至耻骨联合上缘的长度称为上部量;下部分即从耻骨联合上缘至足底的长度为下部量。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "上部量" }, { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "下部量" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "耻骨" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "头顶" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "耻骨" }, { "start_idx": 53, "end_idx": 55, "type": "ite", "entity": "上部量" }, { "start_idx": 62, "end_idx": 67, "type": "bod", "entity": "耻骨联合上缘" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "足底" }, { "start_idx": 75, "end_idx": 77, "type": "ite", "entity": "下部量" } ] }, { "text": "上部量主要表示脊柱的生长,下部代表下肢长骨的生长。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "上部量" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "脊柱" }, { "start_idx": 13, "end_idx": 16, "type": "ite", "entity": "下部代表" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "下肢长骨" } ] }, { "text": "出生时上部量约为身长的60%(30cm),下部量为40%(19.5cm),故身长的中点位于脐上,外表显示下肢短头围头围是指眉弓上方最突出处经枕后结节绕头一周的长度。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "上部量" }, { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "身长" }, { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "下部量" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "身长" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "脐上" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "下肢" }, { "start_idx": 52, "end_idx": 54, "type": "sym", "entity": "下肢短" }, { "start_idx": 55, "end_idx": 56, "type": "ite", "entity": "头围" }, { "start_idx": 57, "end_idx": 58, "type": "ite", "entity": "头围" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "眉弓上方" }, { "start_idx": 70, "end_idx": 73, "type": "bod", "entity": "枕后结节" } ] }, { "text": "新生儿头围平均为34cm,测量头围可知颅骨及脑的生长发育情况,读数至0.1cm。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "头围" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "头围" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "颅骨" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "脑" } ] }, { "text": "前囟及骨缝的变化也是颅骨生长的指标。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "前囟" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "骨缝" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "颅骨" } ] }, { "text": "前囟由两额骨与两顶骨相交接的骨缝的构成,出生时斜径约2.5cm。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "前囟" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "额骨" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "顶骨" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "骨缝" } ] }, { "text": "如有先天性佝偻病,前囟可增大,骨缝前面可延至额部后囟门由两顶骨与两枕骨的骨缝构成,呈三角形,在出生时或出生后2~3个月闭合。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "先天性佝偻病" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "前囟" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "骨缝" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "额部" }, { "start_idx": 9, "end_idx": 23, "type": "sym", "entity": "前囟可增大,骨缝前面可延至额部" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "后囟门" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "顶骨" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "枕骨" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "骨缝" } ] }, { "text": "如果出生时摸不到前囟门,要区别是否为颅骨畸形。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "囟门" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "颅骨畸形" } ] }, { "text": "前囟饱满颅内压增加,囟门凹陷严重脱水及营养不良。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "前囟" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "前囟饱满" }, { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "颅内压增加" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "囟门" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "囟门凹陷" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "严重脱水" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "营养不良" } ] }, { "text": "4.胸围胸围是指胸前乳头下缘向后绕经后背的两肩胛骨下角下缘,取平静呼、吸气时的中间读数至0.1cm。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "胸围" }, { "start_idx": 4, "end_idx": 5, "type": "ite", "entity": "胸围" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "胸前乳头下缘" }, { "start_idx": 22, "end_idx": 28, "type": "bod", "entity": "肩胛骨下角下缘" } ] }, { "text": "新生儿出生时胸廓呈圆筒状,胸围小于头围约1~2cm。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "胸廓" }, { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "胸围" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "头围" } ] }, { "text": "第二节维生素A缺乏症维生素A族的原形化合物是全反式视黄醇,天然维生素A只存在于动物体内,并分两种类型:维生素A1(视黄醇)和维生素A2(3-脱氢视黄醇)。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "维生素A" }, { "start_idx": 22, "end_idx": 27, "type": "dru", "entity": "全反式视黄醇" }, { "start_idx": 51, "end_idx": 54, "type": "dru", "entity": "维生素A" }, { "start_idx": 67, "end_idx": 69, "type": "dru", "entity": "视黄醇" }, { "start_idx": 72, "end_idx": 75, "type": "dru", "entity": "维生素A" }, { "start_idx": 88, "end_idx": 94, "type": "dru", "entity": "3-脱氢视黄醇" } ] }, { "text": "【流行病学】维生素A缺乏是导致儿童严重视觉损害和失明的主要原因,同时也是增加儿童严重感染性疾病危险和死亡风险的主要原因之一,维生素A缺乏被世界卫生组织确认为四大营养缺乏病之一。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "维生素A缺乏" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "视觉损害" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "失明" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "维生素A缺乏" } ] }, { "text": "1991年,Quito营养调查表明:2%的1~5岁儿童血清维生素A水平低于10μgdL,18%低于20μgdL。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "血清" } ] }, { "text": "同期菲律宾调查农村学龄前儿童,29%的儿童血清维生素A低于20μgdL,6%低于1OμgdL。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "血清" } ] }, { "text": "可见发展中国家维生素A缺乏发病率高于发达国家。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "维生素A缺乏" } ] }, { "text": "在我国,卫生部委托首都儿科研究所对14个省42个市县8669例0~5岁儿童于1999年12月~2000年3月协作进行维生素A缺乏情况调查,结果显示,<6个月婴儿为33.4%,2岁以上儿童维生素A缺乏的发生率为0.15%。", "entities": [ { "start_idx": 58, "end_idx": 63, "type": "dis", "entity": "维生素A缺乏" }, { "start_idx": 93, "end_idx": 98, "type": "dis", "entity": "维生素A缺乏" } ] }, { "text": "亚临床型维生素A缺乏发生率较高,已成为儿童广泛的缺乏症之一而备受关注。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "dis", "entity": "临床型维生素A缺乏" } ] }, { "text": "据WHO统计,1995年,全球近2.51亿儿童有亚临床型维生素A缺乏。", "entities": [ { "start_idx": 24, "end_idx": 33, "type": "dis", "entity": "亚临床型维生素A缺乏" } ] }, { "text": "根据2002年WHO的报道,全球有80万儿童(1.4%)死于维生素A缺乏症,1.8%的消耗性疾病也是由于维生素A缺乏症所造成的。", "entities": [ { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 52, "end_idx": 58, "type": "dis", "entity": "维生素A缺乏症" } ] }, { "text": "调查显示,接近12的维生素A缺乏症和干眼症发生在非洲、南亚和东南亚地区。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "干眼症" } ] }, { "text": "2000年,南非的调查发现,有13的0~4岁儿童患维生素A缺乏症,在同年调查3000名死亡儿童中发现,28%的儿童死于因维生素A缺乏症导致的腹泻,23%死于因维生素A缺乏症导致的麻疹,21%死于因维生素A缺乏症导致的疟疾。", "entities": [ { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 60, "end_idx": 66, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "腹泻" }, { "start_idx": 79, "end_idx": 85, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 89, "end_idx": 90, "type": "dis", "entity": "麻疹" }, { "start_idx": 98, "end_idx": 104, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 108, "end_idx": 109, "type": "dis", "entity": "疟疾" } ] }, { "text": "维生素A缺乏症是该地区极其严重的公共卫生问题。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "维生素A缺乏症" } ] }, { "text": "2006年,朝鲜光州调查显示,2.4%的儿童患维生素A缺乏症,42.3%患轻度维生素A缺乏症。", "entities": [ { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 37, "end_idx": 45, "type": "dis", "entity": "轻度维生素A缺乏症" } ] }, { "text": "印度于2007年进行的全国调查显示,每年有52000名儿童因维生素A缺乏症而导致失明。", "entities": [ { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "失明" } ] }, { "text": "2006年,中国疾病控制中心调查显示,我国6岁以下儿童维生素A缺乏症的发生率是12.2%,严重维生素A缺乏症占0.5%,1岁以上儿童发病率最高的西部地区占17.4%。", "entities": [ { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 45, "end_idx": 53, "type": "dis", "entity": "严重维生素A缺乏症" } ] }, { "text": "【发病机制及病因】(一)摄入不足初生时维生素A在肝脏中的贮存量很少。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "维生素A" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肝脏" } ] }, { "text": "出生后维生素A的主要来源是食物。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "维生素A" } ] }, { "text": "故婴儿时期,应提倡母乳喂养,人工喂养时,须给含脂肪的牛乳,婴儿如果单靠炼乳、脱脂牛乳、豆浆、米粉等食品喂养,容易发生维生素A缺乏。", "entities": [ { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "维生素A缺乏" } ] }, { "text": "早产儿肝脏内维生素A的贮存量更少,且脂肪吸收能力也有限,生长发育的速度又较快,故更容易发生维生素A缺乏症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肝脏" }, { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "维生素A" }, { "start_idx": 45, "end_idx": 51, "type": "dis", "entity": "维生素A缺乏症" } ] }, { "text": "如在疾病状态下,长期静脉补液未补充维生素A;或因饮食受到限制,也将导致维生素A缺乏。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "维生素A" }, { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "维生素A" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "维生素A缺乏" } ] }, { "text": "(二)吸收减少维生素A缺乏可见于多种临床情况,如吸收障碍综合征、慢性腹泻、慢性痢疾、慢性肝炎、胆道梗阻、胆囊纤维化、钩虫病、肠道感染等均可影响维生素A的吸收。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "维生素A缺乏" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "吸收障碍综合征" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "慢性腹泻" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "慢性痢疾" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "慢性肝炎" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "胆道梗阻" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "胆囊纤维化" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "钩虫病" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "肠道感染" }, { "start_idx": 71, "end_idx": 74, "type": "dru", "entity": "维生素A" } ] }, { "text": "(三)锌摄入不足当锌缺乏时,维生素A结合蛋白、前清蛋白、维生素A还原酶都降低,使维生素A不能利用而排出体外,造成维生素A缺乏。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "dru", "entity": "锌" }, { "start_idx": 9, "end_idx": 9, "type": "dru", "entity": "锌" }, { "start_idx": 9, "end_idx": 11, "type": "sym", "entity": "锌缺乏" }, { "start_idx": 14, "end_idx": 21, "type": "dru", "entity": "维生素A结合蛋白" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "前清蛋白" }, { "start_idx": 28, "end_idx": 34, "type": "dru", "entity": "维生素A还原酶" }, { "start_idx": 14, "end_idx": 37, "type": "sym", "entity": "维生素A结合蛋白、前清蛋白、维生素A还原酶都降低" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "维生素A" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "维生素A缺乏" } ] }, { "text": "Rahman等证实锌的缺乏限制了维生素A的生物利用率,锌和维生素A的缺乏经常同时存在于营养不良的小儿,同时给予维生素A和锌的补充可以改善维生素A的缺乏。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "dru", "entity": "锌" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "维生素A" }, { "start_idx": 27, "end_idx": 27, "type": "dru", "entity": "锌" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "维生素A" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "营养不良" }, { "start_idx": 55, "end_idx": 58, "type": "dru", "entity": "维生素A" }, { "start_idx": 60, "end_idx": 60, "type": "dru", "entity": "锌" }, { "start_idx": 68, "end_idx": 71, "type": "dru", "entity": "维生素A" } ] }, { "text": "近来有报道指出,铁的不足对维生素A的利用也有影响。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "dru", "entity": "铁" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "维生素A" } ] }, { "text": "(四)消耗增加当小儿患结核、麻疹、水痘、肺炎以及高热时,维生素A的消耗增加,如此时未予及时补充,则造成维生素A的血浆浓度降低。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "结核" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "麻疹" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "水痘" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肺炎" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "高热" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "维生素A" }, { "start_idx": 51, "end_idx": 54, "type": "dru", "entity": "维生素A" }, { "start_idx": 51, "end_idx": 61, "type": "sym", "entity": "维生素A的血浆浓度降低" } ] }, { "text": "(五)利用障碍如小儿患有肝脏、肾脏、甲状腺疾病、胰腺囊性纤维变性及蛋白-能量营养不良时,将导致血浆中视黄醇结合蛋白(RBP)代谢异常,导致维生素A缺乏。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肝脏" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肾脏" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "甲状腺" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "胰腺囊性纤维变性" }, { "start_idx": 33, "end_idx": 41, "type": "dis", "entity": "蛋白-能量营养不良" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "血浆" }, { "start_idx": 50, "end_idx": 56, "type": "dru", "entity": "视黄醇结合蛋白" }, { "start_idx": 58, "end_idx": 60, "type": "dru", "entity": "RBP" }, { "start_idx": 47, "end_idx": 65, "type": "sym", "entity": "血浆中视黄醇结合蛋白(RBP)代谢异常" }, { "start_idx": 69, "end_idx": 72, "type": "dru", "entity": "维生素A" }, { "start_idx": 69, "end_idx": 74, "type": "sym", "entity": "维生素A缺乏" } ] }, { "text": "【临床表现】由于维生素A和维生素A原缺乏所引起的营养缺乏病,临床上首先出现暗适应能力下降,小婴儿此症状不明显,如不仔细观察,容易被忽视。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "维生素A" }, { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "维生素A原" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "营养缺乏病" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "暗适应能力" } ] }, { "text": "首先由母亲发现,患儿在暗环境下安静,视物不清,行走、定向困难。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "暗环境下安静" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "视物不清" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "行走、定向困难" } ] }, { "text": "数周及数月后出现结膜干燥症,结膜干燥,失去光泽,主要是由于结膜和附近腺体组织增生,分泌减少,继而发生干燥。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "结膜干燥症" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "结膜干燥" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "结膜" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "腺体" } ] }, { "text": "在眼球巩膜近角膜缘外侧,由脱落的角膜上皮形成三角形白色泡沫状斑块称结膜干燥斑(Bitot斑)。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "bod", "entity": "眼球巩膜近角膜缘" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "角膜上皮" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "结膜干燥斑" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "Bitot斑" } ] }, { "text": "丘疹坚实而干燥,色暗棕,多为毛囊性,针头大至米粒大,圆锥形。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "丘疹" }, { "start_idx": 0, "end_idx": 28, "type": "sym", "entity": "丘疹坚实而干燥,色暗棕,多为毛囊性,针头大至米粒大,圆锥形" } ] }, { "text": "丘疹的中央有棘刺状角质栓,触之坚硬,去除后留下坑状凹陷,无炎症,无主观症状,丘疹密集犹似蟾蜍皮,称蟾蜍皮病(phrynoderma)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "丘疹" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "丘疹" }, { "start_idx": 38, "end_idx": 46, "type": "sym", "entity": "丘疹密集犹似蟾蜍皮" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "蟾蜍皮病" }, { "start_idx": 54, "end_idx": 64, "type": "dis", "entity": "phrynoderma" } ] }, { "text": "皮疹发生在面部,可有许多黑头。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "皮疹" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "面部" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "有许多黑头" } ] }, { "text": "患者毛发干燥,缺少光泽,易脱落,呈弥漫稀疏,指甲变脆,表面有纵横沟纹或点状凹陷。", "entities": [ { "start_idx": 2, "end_idx": 20, "type": "sym", "entity": "毛发干燥,缺少光泽,易脱落,呈弥漫稀疏" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "指甲" }, { "start_idx": 22, "end_idx": 38, "type": "sym", "entity": "指甲变脆,表面有纵横沟纹或点状凹陷" } ] }, { "text": "维生素A缺乏对骨骼(特别是长骨)的伸长也有明显影响,使骨变得又短又厚。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "维生素A缺乏" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "骨骼" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "长骨" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "骨" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "使骨变得又短又厚" } ] }, { "text": "HuW等人通过色层分析法测定维生素A浓度,证明维生素A浓度和体重以及BMI有明显的统计学意义,提示维生素A对儿童的生长发育有明显的影响。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "色层分析法" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "维生素A" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "维生素A" }, { "start_idx": 49, "end_idx": 52, "type": "dru", "entity": "维生素A" } ] }, { "text": "维生素A缺乏时,对呼吸系统也有不同程度的影响,使气管及支气管的上皮细胞中间层的细胞增殖,变成鳞状、角化,并使上皮细胞的纤毛脱落,失去上皮组织的正常保护功能,容易发生呼吸系统的感染。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "维生素A缺乏" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "气管" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "支气管" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "细胞" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "细胞增殖" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 54, "end_idx": 62, "type": "sym", "entity": "上皮细胞的纤毛脱落" }, { "start_idx": 66, "end_idx": 69, "type": "bod", "entity": "上皮组织" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "呼吸系统" } ] }, { "text": "【实验室检查】(一)视觉暗适应功能测定维生素A缺乏症患者的暗适应能力比正常人差,但是其他因素也可引起暗适应能力降低,如视神经萎缩、色素性视网膜炎、睡眠不足等。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "pro", "entity": "视觉暗适应功能测定" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 29, "end_idx": 38, "type": "sym", "entity": "暗适应能力比正常人差" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "视神经" }, { "start_idx": 65, "end_idx": 71, "type": "dis", "entity": "色素性视网膜炎" }, { "start_idx": 73, "end_idx": 76, "type": "sym", "entity": "睡眠不足" } ] }, { "text": "(二)血清维生素A水平测定是评价维生素A营养状况的常用指标,也是最可靠的指标,正常值为300~500μgL,若低于200μgL为缺乏。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "血清维生素A水平测定" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "维生素A" } ] }, { "text": "(三)血浆中视黄醇结合蛋白测定(RBP)近来有人认为RBP与人体维生素A水平呈正相关,RBP的含量可反映人体维生素A的营养水平。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "pro", "entity": "血浆中视黄醇结合蛋白测定" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "RBP" }, { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "RBP" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "维生素A" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "维生素A" } ] }, { "text": "正常儿童的血浆RBP的含量为23.1mgL。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "RBP" } ] }, { "text": "(四)维生素A的相对剂量反应试验当血清中维生素A浓度在正常范围时,肝脏维生素A已有耗尽的可能,因此采用相对剂量反应(RDR)法间接评价个体体内维生素A的贮存量。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "pro", "entity": "维生素A的相对剂量反应试验" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "血清" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "维生素A" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肝脏" }, { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "维生素A" }, { "start_idx": 71, "end_idx": 74, "type": "dru", "entity": "维生素A" } ] }, { "text": "口服1000mg维生素A棕榈酸,分别于口服前和口服后5小时测定血清维生素A浓度。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dru", "entity": "维生素A棕榈酸" }, { "start_idx": 29, "end_idx": 38, "type": "pro", "entity": "测定血清维生素A浓度" } ] }, { "text": "若服后5小时的血清维生素A浓度增高幅度,即RDR(relativedosereation,RDR)率≥20%,表示肝脏内维生素A的贮存已处于临界状态。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "维生素A" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "肝脏" }, { "start_idx": 60, "end_idx": 63, "type": "dru", "entity": "维生素A" } ] }, { "text": "用此方法可以进一步确定亚临床状态维生素A缺乏。", "entities": [ { "start_idx": 11, "end_idx": 21, "type": "dis", "entity": "亚临床状态维生素A缺乏" } ] }, { "text": "【诊断】仔细询问病史,如患者存在维生素A摄入不足,或者存在维生素A的吸收、利用障碍,或引起维生素A消耗过多的疾病,同时合并暗适应障碍、夜盲、结膜干燥、角膜软化或四肢伸侧有毛囊性角化丘疹,通过暗适应检查和血浆维生素A浓度的测定可基本作出诊断。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "维生素A" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "维生素A" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "维生素A" }, { "start_idx": 67, "end_idx": 68, "type": "dis", "entity": "夜盲" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "角膜" }, { "start_idx": 80, "end_idx": 83, "type": "bod", "entity": "四肢伸侧" }, { "start_idx": 85, "end_idx": 91, "type": "dis", "entity": "毛囊性角化丘疹" }, { "start_idx": 61, "end_idx": 91, "type": "sym", "entity": "暗适应障碍、夜盲、结膜干燥、角膜软化或四肢伸侧有毛囊性角化丘疹" }, { "start_idx": 95, "end_idx": 99, "type": "pro", "entity": "暗适应检查" }, { "start_idx": 101, "end_idx": 111, "type": "pro", "entity": "血浆维生素A浓度的测定" } ] }, { "text": "WHO推荐的诊断标准为:血清维生素A<0.7μmolL为维生素A缺乏;0.7~1.4μmolL为亚临床维生素A缺乏(维生素A存在不足);1.4~2.79μmolL为维生素贮存充足。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "血清" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "维生素A" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "维生素A缺乏" }, { "start_idx": 48, "end_idx": 56, "type": "dis", "entity": "亚临床维生素A缺乏" }, { "start_idx": 58, "end_idx": 61, "type": "dru", "entity": "维生素A" }, { "start_idx": 82, "end_idx": 84, "type": "dru", "entity": "维生素" } ] }, { "text": "若血清维生素A水平在正常低值,此时肝内维生素A的储存也可能已耗竭。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "血清" }, { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "维生素A" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肝内" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "维生素A" } ] }, { "text": "在这种可疑的情况下,可采用敏感而可靠的相对剂量反应试验来进一步确定亚临床维生素A的缺乏。", "entities": [ { "start_idx": 33, "end_idx": 42, "type": "dis", "entity": "亚临床维生素A的缺乏" } ] }, { "text": "亚临床维生素A缺乏已成为儿童广泛的营养缺乏症而受关注。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "亚临床维生素A缺乏" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "营养缺乏症" } ] }, { "text": "亚临床维生素A缺乏是指儿童因维生素A摄入不足导致的轻度维生素A缺乏,其特点是无典型的临床表现。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "亚临床维生素A缺乏" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "维生素A" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "维生素A摄入不足" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "轻度维生素A缺乏" } ] }, { "text": "【治疗】如患儿因为疾病引起维生素A缺乏,应首先去除病因,同时给予维生素A丰富的饮食。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "维生素A缺乏" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "维生素A" } ] }, { "text": "每天补充维生素A2.5万U(1U的维生素A=0.3μg的视黄醇),口服或肌注均可,共1~2周(或大剂量1次20万U),同时给予高蛋白饮食,以后再给予预防量。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "维生素A" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "维生素A" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "视黄醇" } ] }, { "text": "如有角膜软化则给水溶性维生素A10万U,1周后再给20万U,然后给预防量。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dru", "entity": "水溶性维生素A" } ] }, { "text": "夜盲症可于治疗后数小时好转,干眼于2~3天后改善。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "夜盲症" } ] }, { "text": "必要时保持两眼清洁,使用抗生素眼膏,角膜溃疡者用1%阿托品滴眼防止虹膜粘连。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "抗生素眼膏" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "阿托品" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "虹膜" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "虹膜粘连" } ] }, { "text": "【预防】应提倡母乳喂养,对稍大的儿童,应及时添加含有维生素A的辅食,如鱼肝油、动物肝脏、肾脏、蛋黄、胡萝卜汁及番茄汁等,避免偏食,增加维生素A的摄入量,避免维生素A的缺乏。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "维生素A" }, { "start_idx": 67, "end_idx": 70, "type": "dru", "entity": "维生素A" }, { "start_idx": 78, "end_idx": 81, "type": "dru", "entity": "维生素A" } ] }, { "text": "如小儿因患有疾病而影响了维生素A吸收和利用时,应首先去除病因,然后及时补充维生素A。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "维生素A" }, { "start_idx": 37, "end_idx": 40, "type": "dru", "entity": "维生素A" } ] }, { "text": "维生素A每天推荐摄入量婴儿期为1500U,12岁以下的儿童为1500~2500U,如饮食中维生素A含量丰富,可不必另外补充维生素A。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "维生素A" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "维生素A" }, { "start_idx": 61, "end_idx": 64, "type": "dru", "entity": "维生素A" } ] }, { "text": "四、治疗ICH治疗原则是迅速控制出血,适时进行外科手术治疗,预防并发症与后遗症。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "ICH" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "控制出血" }, { "start_idx": 23, "end_idx": 28, "type": "pro", "entity": "外科手术治疗" } ] }, { "text": "(一)稳定治疗稳定治疗措施包括优化呼吸管理、控制体循环高血压、防治癫痫发作和针对颅内压增高的医学管理等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "稳定治疗" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "优化呼吸管理" }, { "start_idx": 22, "end_idx": 29, "type": "pro", "entity": "控制体循环高血压" }, { "start_idx": 31, "end_idx": 36, "type": "pro", "entity": "防治癫痫发作" }, { "start_idx": 38, "end_idx": 44, "type": "pro", "entity": "针对颅内压增高" } ] }, { "text": "ICH急性期应绝对卧床,保持安静,不宜搬动,避免引起血压增高和颅内压增高的因素。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "ICH急性期" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "卧床" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "保持安静" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "不宜搬动" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "血压" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "血压增高" }, { "start_idx": 31, "end_idx": 33, "type": "ite", "entity": "颅内压" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "颅内压增高" } ] }, { "text": "如因特殊情况如急诊检查和手术治疗等,需要搬动病人,应保持头部固定。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "急诊检查" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "手术治疗" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "头部" } ] }, { "text": "还应保持水电解质平衡及足够的热量供给。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "保持水电解质平衡" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "足够的热量供给" } ] }, { "text": "另外,针对蛛网膜下腔出血患儿来说,控制血管痉挛。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血管" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "血管痉挛" } ] }, { "text": "而对于一般出血病例,需要待病人病情稳定后再实施手术治疗,包括清除血肿和对局部畸形血管的处理等,通常以发病后2周左右为宜。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "手术治疗" } ] }, { "text": "目前尚无明显证据显示幕上实质内血肿外科手术摘除术对任何年龄都有效。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "dep", "entity": "外科" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "手术摘除术" } ] }, { "text": "Mendelow及其同事研究显示,在1033名非外伤性幕上出血的成人随机试验中,在血肿发生24小时内进行血肿取出术对病人无明显受益。", "entities": [ { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "血肿" }, { "start_idx": 52, "end_idx": 56, "type": "pro", "entity": "血肿取出术" } ] }, { "text": "另外一项小样本研究,给予了较早(小于4小时)血肿取出术的11名病例中,有4例因为再出血给予了暂停早期血肿清除手术。", "entities": [ { "start_idx": 50, "end_idx": 55, "type": "pro", "entity": "血肿清除手术" } ] }, { "text": "也有无对照研究证据显示,在选择人群中血肿清除可能缓解脑疝发生。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "血肿" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "脑疝" } ] }, { "text": "这种外科手术对于小脑出血以及大脑半球较大范围出血病灶病人可能获益更多。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "外科手术" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "小脑出血" }, { "start_idx": 14, "end_idx": 23, "type": "sym", "entity": "大脑半球较大范围出血" } ] }, { "text": "反复腰穿放脑脊液适用于脑室及蛛网膜下腔出血患者,可减少脑积水的发生,并可迅速缓解蛛网膜下腔出血所引起的颅内高压,减轻脑膜刺激症状。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "腰穿" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脑室" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "脑积水" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "颅内高压" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "脑膜" } ] }, { "text": "但如果患儿头痛剧烈、呕吐频繁或极度烦躁,甚至已出现脑疝的早期征象,则应禁忌腰穿,以免诱发脑疝。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "患儿头痛剧烈" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "呕吐频繁" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "极度烦躁" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "脑疝" }, { "start_idx": 37, "end_idx": 38, "type": "pro", "entity": "腰穿" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "脑疝" } ] }, { "text": "硬膜下穿刺适用于硬膜下出血的治疗,前囟未闭。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "硬膜下穿刺" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "前囟" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "前囟未闭" } ] }, { "text": "穿刺1次,穿刺成功后应让液体自动流出,不应抽吸,每次引流量不宜过大,一般不超过15ml,否则可能诱发再出血。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "穿刺" } ] }, { "text": "可穿刺10~15次,液体量不多者逐渐延长间隔并停止穿刺。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "穿刺" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "穿刺" } ] }, { "text": "脑血管畸形的手术处理可以防止再次破裂出血。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "脑血管畸形" } ] }, { "text": "对凝血缺陷和血液系统疾病的治疗可减少继发性出血的危险。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "凝血缺陷" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "血液系统疾病" } ] }, { "text": "血小板计数在200×109/L以上时脑出血很少发生。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" } ] }, { "text": "即使血小板数很低,在没有创伤的情况下,自发性颅内出血极少见。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "血小板" } ] }, { "text": "获得性同种免疫血小板减少症患者的脑出血通常伴有全身性病毒感染,可能是由于感染刺激机体产生大量的抗血小板抗体,导致血小板减少。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "脑出血" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "全身性病毒感染" }, { "start_idx": 47, "end_idx": 52, "type": "bod", "entity": "抗血小板抗体" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "血小板减少" } ] }, { "text": "同样,Ⅶ因子缺乏患儿通过补充Ⅶ因子可减少或预防外伤性颅内出血。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "Ⅶ因子" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "Ⅶ因子" } ] }, { "text": "(四)康复治疗ICH患儿在病情好转后即应进行医学康复训练,包括物理治疗、作业治疗和语言治疗等。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "ICH" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "医学康复训练" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "物理治疗" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "作业治疗" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "语言治疗" } ] }, { "text": "还应辅以针灸、推拿、理疗以及高压氧等,以减轻神经损害后遗症。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "针灸" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "推拿" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "理疗" }, { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "高压氧" } ] }, { "text": "同时可给予心理支持和行为治疗。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "心理支持" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "行为治疗" } ] }, { "text": "儿童ICH治疗和评估推荐如下:1.Ⅰ级推荐(1)对于非外伤性脑出血患儿,当非侵入性检查不能确定原因时,应该进行全面的危险因素评估,包括常规脑血管造影,从而在再次脑出血发生前确定可治疗的危险因素(Ⅰ级,C水平证据)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "ICH" } ] }, { "text": "(2)严重凝血因子缺乏症患儿需要接受凝血因子替代治疗(Ⅰ级,A水平证据)。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "凝血因子缺乏症" } ] }, { "text": "(3)先天性血管异常患儿有发生反复脑出血的危险,这种损害应该予以及时识别和矫治。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "先天性血管异常" } ] }, { "text": "(4)脑出血病人的稳定治疗包括呼吸治疗、控制血压、控制癫痫发作和降低颅内压(Ⅰ级,C水平证据)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脑出血" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "呼吸治疗" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "控制血压" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "控制癫痫发作" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "降低颅内压" } ] }, { "text": "2.Ⅱ级推荐(1)对于无临床症状的颅内动脉瘤病人,有必要每1~5年应用颅脑MRA进行追踪随访(Ⅱa级,C水平证据)。", "entities": [ { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "颅内动脉瘤" }, { "start_idx": 35, "end_idx": 39, "type": "pro", "entity": "颅脑MRA" } ] }, { "text": "当病人出现可以用颅内动脉瘤解释的临床症状时,即使颅脑MRA未发现颅内动脉瘤,也应考虑采用CT血管造影(CTangiography,CTA)或导管血管造影(catheterangiography,CA)检查(Ⅱb级,C水平证据)。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "颅内动脉瘤" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "颅脑MRA" }, { "start_idx": 44, "end_idx": 49, "type": "pro", "entity": "CT血管造影" }, { "start_idx": 51, "end_idx": 63, "type": "pro", "entity": "CTangiography" }, { "start_idx": 65, "end_idx": 67, "type": "pro", "entity": "CTA" }, { "start_idx": 70, "end_idx": 75, "type": "pro", "entity": "导管血管造影" } ] }, { "text": "然而,对于可能发生脑疝或颅内压很高的患者,应该进行外科手术治疗。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "脑疝" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "颅内压" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "外科手术治疗" } ] }, { "text": "(2)尽管有证据表明镰状细胞病(sicklecelldisease,SCD)患者周期性的输血会造成缺血性梗死,但没有证据表明镰状细胞病患者周期性的输血能够减少ICH的发生率(Ⅲ级,B水平证据)。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "镰状细胞病" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "缺血性梗死" }, { "start_idx": 62, "end_idx": 66, "type": "bod", "entity": "镰状细胞病" }, { "start_idx": 79, "end_idx": 81, "type": "dis", "entity": "ICH" } ] }, { "text": "参考文献1.中华医学会.临床诊疗指南(癫痫病分册).北京:人民卫生出版社,20072.吴逊,林庆.神经病学(第13卷:癫痫和发作性疾病).北京:人民军医出版社,20013.周水珍,孙道开.抗癫痫新药的临床应用.中国实用儿科杂志,1997,12(6):372-3724.周水珍.儿童癫痫发作与非癫痫发作.中国临床神经科学杂志,2000,8(4):2975.周水珍.小儿癫痫抗癫痫药物治疗相关问题及其对策.实用儿科临床杂志,2006,21(24):1686-16866.吴希如.癫痫遗传学的研究进展.中华儿科杂志,2000,38(11):717-7177.周水珍,陈天兰,孙道开.非惊厥性癫痫持续状态的临床分析.中华儿科杂志,1995,33(5):2908.林庆.小儿癫痫发作的分类及最新进展.中华儿科杂志,2002,40(5):313-3139.蒋梨,蔡方成.小儿癫痫的诊断.中华儿科杂志,2002,40(6):380-38010.魏克伦,孙梅,刘春峰(主译).儿科临床手册(HandbookofPediatrics).北京:人民卫生出版社,200111.ToniaSabo-Graham,AlanR.ManagementofStatusEpilepticusinChildren.PediatricinReview.1998;19(9):306-30612.ChristopherC,Giza,RamanSankar.Pathogenesisofthedevelopmentalepilepsis.CurrentOpinioninPediatrics.1998,10:567-56713.AbdelazizY,HarbA,HishamM.TextbookofClinicalPediatrics.USA:LippincottWilliamsandWilkins.200114.EngelJJr.Aproposeddiagnosticschemeforpeoplewithepilepticseizuresandwithepilepsy:reportoftheILAEtaskforceonclassificationandterminology.Epilepsia.2001,42:796-80315.RamaMaganti,PaulaGerber,CorneliaDrees,et,al.Nonconvulsivestatusepilepticus.Epilepsy&Behavior.12(2008)572-57216.JeanAicardi,MartinB,ChristopherGillberg.ClinicinDevelopmentalMedicine:DiseasesoftheNervoussysteminChildhood3rdEdition.MacKeithPress,2009", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dep", "entity": "中华医学会" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "癫痫" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "神经病学" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "癫痫" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "发作性疾病" }, { "start_idx": 95, "end_idx": 96, "type": "dis", "entity": "癫痫" }, { "start_idx": 109, "end_idx": 110, "type": "dep", "entity": "儿科" }, { "start_idx": 138, "end_idx": 141, "type": "dis", "entity": "儿童癫痫" }, { "start_idx": 145, "end_idx": 149, "type": "dis", "entity": "非癫痫发作" }, { "start_idx": 153, "end_idx": 154, "type": "dep", "entity": "临床" }, { "start_idx": 181, "end_idx": 184, "type": "dis", "entity": "小儿癫痫" }, { "start_idx": 203, "end_idx": 204, "type": "dep", "entity": "儿科" }, { "start_idx": 237, "end_idx": 238, "type": "dis", "entity": "癫痫" }, { "start_idx": 250, "end_idx": 251, "type": "dep", "entity": "儿科" }, { "start_idx": 288, "end_idx": 293, "type": "dis", "entity": "非惊厥性癫痫" }, { "start_idx": 306, "end_idx": 307, "type": "dep", "entity": "儿科" }, { "start_idx": 330, "end_idx": 333, "type": "dis", "entity": "小儿癫痫" }, { "start_idx": 347, "end_idx": 348, "type": "dep", "entity": "儿科" }, { "start_idx": 379, "end_idx": 382, "type": "dis", "entity": "小儿癫痫" }, { "start_idx": 389, "end_idx": 390, "type": "dep", "entity": "儿科" }, { "start_idx": 430, "end_idx": 431, "type": "dep", "entity": "儿科" } ] }, { "text": "四、超声内镜超声内镜术(EUS)是超声与内镜相结合的一种新的检测技术,在内镜的顶端安置微型超声探头,当内镜插入消化道后,既可通过内镜直接观察消化道黏膜的形态学变化,又可通过内镜顶端的超声探头直接接触消化道黏膜进行扫描,从而获得管壁各层次的组织学特性及周围重要脏器的超声影像,增加了内镜的诊断范畴,提高了内镜的诊断能力。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "超声内镜" }, { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "超声内镜术" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "EUS" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "超声" }, { "start_idx": 20, "end_idx": 21, "type": "equ", "entity": "内镜" }, { "start_idx": 36, "end_idx": 37, "type": "equ", "entity": "内镜" }, { "start_idx": 43, "end_idx": 48, "type": "equ", "entity": "微型超声探头" }, { "start_idx": 51, "end_idx": 52, "type": "equ", "entity": "内镜" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "消化道" }, { "start_idx": 64, "end_idx": 65, "type": "equ", "entity": "内镜" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "消化道黏膜" }, { "start_idx": 86, "end_idx": 94, "type": "equ", "entity": "内镜顶端的超声探头" }, { "start_idx": 99, "end_idx": 103, "type": "bod", "entity": "消化道黏膜" }, { "start_idx": 113, "end_idx": 114, "type": "bod", "entity": "管壁" }, { "start_idx": 129, "end_idx": 130, "type": "bod", "entity": "脏器" }, { "start_idx": 140, "end_idx": 141, "type": "equ", "entity": "内镜" }, { "start_idx": 151, "end_idx": 152, "type": "equ", "entity": "内镜" } ] }, { "text": "内镜超声扫描有2种方式:①水囊法:于内镜顶端超声探头的周围固定一橡皮囊,通过内镜的固定管道孔注入脱气水3~5ml。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "内镜超声扫描" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "水囊法" } ] }, { "text": "适用于食管、十二指肠升部和降部的超声扫描。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "食管" }, { "start_idx": 6, "end_idx": 14, "type": "bod", "entity": "十二指肠升部和降部" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "超声扫描" } ] }, { "text": "②脱气水充盈法:通过内镜的固定管道向胃腔内注入脱气水300~600ml,使胃壁膨胀,超声探头完全浸入水中。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "脱气水充盈法" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "胃腔" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "胃壁" }, { "start_idx": 42, "end_idx": 45, "type": "equ", "entity": "超声探头" } ] }, { "text": "适用于观察胃壁的各层结构及胃周邻近脏器,例如肝、胆、胰、脾脏及门静脉等。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "胃壁" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肝" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "胆" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "胰" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "门静脉" } ] }, { "text": "此外,在消化道腔内进行超声扫描明显缩短了超声探头与靶器官的距离,避免了腹壁脂肪、肠管积气和骨骼系统对超声波传导的影响和干扰,使位于腹壁深部的器官病变显示更清晰。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "消化道腔" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "超声扫描" }, { "start_idx": 20, "end_idx": 23, "type": "equ", "entity": "超声探头" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "靶器官" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "腹壁脂肪" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "肠管" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "骨骼系统" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "腹壁深部" } ] }, { "text": "因而超声胃镜不仅具备内镜和超声双重功能,而且提高了内镜和超声在某些方面的诊断水平,弥补了这两种影像诊断的不足点。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "equ", "entity": "超声胃镜" } ] }, { "text": "五、尿液微生物学检查对诊断感染性疾病有意义。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "尿液微生物学检查" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "包括:(一)细菌学检查1.直接细菌学检查取尿10ml,1500转/分离心30分钟,沉渣涂片行革兰染色、抗酸染色(结核杆菌)以及吕氏美蓝染色(淋病萘瑟菌)以发现病原菌。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "细菌学检查" }, { "start_idx": 13, "end_idx": 19, "type": "pro", "entity": "直接细菌学检查" }, { "start_idx": 21, "end_idx": 21, "type": "ite", "entity": "尿" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "涂片" }, { "start_idx": 46, "end_idx": 49, "type": "pro", "entity": "革兰染色" }, { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "抗酸染色" }, { "start_idx": 56, "end_idx": 59, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 63, "end_idx": 68, "type": "pro", "entity": "吕氏美蓝染色" }, { "start_idx": 70, "end_idx": 74, "type": "mic", "entity": "淋病萘瑟菌" }, { "start_idx": 79, "end_idx": 81, "type": "mic", "entity": "病原菌" } ] }, { "text": "2.细菌培养可作一般细菌、结核杆菌、厌氧菌及L型菌培养(滴养)并进行菌落计数,以鉴别是否为尿路感染。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "细菌培养" }, { "start_idx": 13, "end_idx": 16, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 18, "end_idx": 20, "type": "mic", "entity": "厌氧菌" }, { "start_idx": 22, "end_idx": 24, "type": "mic", "entity": "L型菌" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "滴养" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "菌落计数" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "尿路感染" } ] }, { "text": "菌落数>105/ml则为感染,菌落数<103/ml多为污染,介于两者之间为可疑。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "菌落数" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "感染" }, { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "菌落数" } ] }, { "text": "如果定量接种前在室温下放置时间超过2小时,即使计数≥105/ml亦可能是污染。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "定量接种" } ] }, { "text": "3.免疫荧光检查检查抗体包裹细菌可用于区别上、下泌尿道感染以及非淋菌性尿道炎的诊断。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "免疫荧光检查" }, { "start_idx": 8, "end_idx": 15, "type": "pro", "entity": "检查抗体包裹细菌" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "上、下泌尿道感染" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "非淋菌性尿道炎" } ] }, { "text": "(二)其他病原微生物包括病毒、支原体和衣原体等,一般采用培养分离,免疫荧光检查等方法。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "mic", "entity": "病原微生物" }, { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "病毒" }, { "start_idx": 15, "end_idx": 17, "type": "mic", "entity": "支原体" }, { "start_idx": 19, "end_idx": 21, "type": "mic", "entity": "衣原体" }, { "start_idx": 26, "end_idx": 31, "type": "pro", "entity": "采用培养分离" }, { "start_idx": 33, "end_idx": 38, "type": "pro", "entity": "免疫荧光检查" } ] }, { "text": "第三节恶性组织细胞增生症本组疾病中包括急性单核细胞性白血病(急单)、真正的恶性组织细胞病、恶性组织细胞肉瘤和少见的恶性郎罕细胞病,参见恶性肿瘤有关章节。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "恶性组织细胞增生症" }, { "start_idx": 19, "end_idx": 28, "type": "dis", "entity": "急性单核细胞性白血病" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "急单" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "恶性组织细胞病" }, { "start_idx": 45, "end_idx": 52, "type": "dis", "entity": "恶性组织细胞肉瘤" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "恶性郎罕细胞病" }, { "start_idx": 67, "end_idx": 70, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "第八节右位心右位心(dextrocardia)由Fabricius在1606年首先报道,右位心伴内脏位置正常者在活产婴儿中的发生率在1∶7500~1∶29000之间,而在儿科中,单纯性右位心较右位心伴内脏反位右位心及90%的孤立性右位心伴有先天性心脏畸形,多数属青紫型。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "右位心" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "右位心" }, { "start_idx": 10, "end_idx": 21, "type": "dis", "entity": "dextrocardia" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "右位心" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "内脏" }, { "start_idx": 96, "end_idx": 98, "type": "dis", "entity": "右位心" }, { "start_idx": 100, "end_idx": 101, "type": "bod", "entity": "内脏" }, { "start_idx": 100, "end_idx": 103, "type": "sym", "entity": "内脏反位" }, { "start_idx": 104, "end_idx": 106, "type": "dis", "entity": "右位心" }, { "start_idx": 115, "end_idx": 117, "type": "dis", "entity": "右位心" }, { "start_idx": 120, "end_idx": 126, "type": "dis", "entity": "先天性心脏畸形" } ] }, { "text": "【胚胎发生学】正常情况下原始心管在胚胎发育22~23天时向右侧成襻,在此后10~12天,心脏及大血管开始分隔,心尖开始由右侧胸腔迁移至左侧胸腔的正常位置。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心管" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "心脏" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "血管" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "心尖" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "胸腔" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "胸腔" } ] }, { "text": "在内脏位置正常时,右位心被认为是由于此迁移过程缺如所致。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "内脏" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "右位心" } ] }, { "text": "同样,在内脏位置反位者,同样已有从左向右的心脏迁移过程。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "内脏" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "心脏" } ] }, { "text": "【命名与分类】(一)右移心(dextroposition)或称为继发性右位心,由于外部因素将心脏整体推向右侧胸腔。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "右移心" }, { "start_idx": 14, "end_idx": 27, "type": "dis", "entity": "dextroposition" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "继发性右位心" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "心脏" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "胸腔" } ] }, { "text": "(二)孤立性右位心(isolateddextrocardia)内脏位置正常,心尖位于右侧胸腔。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "孤立性右位心" }, { "start_idx": 10, "end_idx": 29, "type": "dis", "entity": "isolateddextrocardia" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "内脏" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "心尖" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "胸腔" } ] }, { "text": "(三)混合性右位心(mixeddextrocardia)右位心伴房室连接不一致。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "混合性右位心" }, { "start_idx": 10, "end_idx": 26, "type": "dis", "entity": "mixeddextrocardia" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "房室" } ] }, { "text": "(四)镜像右位心(mirrordextrocardia)内脏反位,心脏正常位于右侧胸腔。", "entities": [ { "start_idx": 9, "end_idx": 26, "type": "dis", "entity": "mirrordextrocardia" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "内脏" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "心脏" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "胸腔" } ] }, { "text": "因此,应先将右移心排除后,依据内脏位置确定右位心的类型。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "右移心" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "内脏" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "右位心" } ] }, { "text": "【临床诊断】尽管右位心可通过体格检查发现,但通常需经胸部X线平片进行诊断。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "右位心" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "体格检查" } ] }, { "text": "右位心可在在新生儿表现为青紫、呼吸困难和心脏杂音右位心是在常规体格检查时发现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "右位心" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "青紫" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "心脏" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "心脏杂音" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "右位心" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "体格检查" } ] }, { "text": "右位心伴发有膈疝等其他先天畸形时提示伴有心内结构异常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "右位心" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "心内结构异常" } ] }, { "text": "胸片不但可用于排除心外原因引起的右移心肝脏及胃泡位置、气管支气管类型的判断确定内脏位置而对右位心进行分类。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "胸片" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "心" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "右移心" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肝脏" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "胃泡" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "气管" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "支气管" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "内脏" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "右位心" } ] }, { "text": "同时尚需明确有无间隔缺损瓣膜反流或狭窄肺静脉回流心脏伴发畸形心导管造影检查明确诊断。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "间隔" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "间隔缺损" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "瓣膜" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "瓣膜反流或狭窄" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "肺静脉" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "肺静脉回流" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "心脏" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "心脏伴发畸形" }, { "start_idx": 30, "end_idx": 36, "type": "pro", "entity": "心导管造影检查" } ] }, { "text": "【伴发畸形】(一)内脏正位孤立性右位心绝大多数伴有心内结构畸形。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "内脏" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "孤立性右位心" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "心内结构畸形" } ] }, { "text": "心室大血管连接不一致发生率较高,近半数合并有房室连接不一致及心室大血管连接不一致,即纠正型大血管转位。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "心室大血管连接不一致" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "房室连接不一致" }, { "start_idx": 30, "end_idx": 39, "type": "sym", "entity": "心室大血管连接不一致" }, { "start_idx": 42, "end_idx": 49, "type": "sym", "entity": "纠正型大血管转位" } ] }, { "text": "肺动脉狭窄或闭锁常见,占60%,右位主动脉弓少见,占5%。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "肺动脉狭窄或闭锁" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "右位主动脉弓" } ] }, { "text": "短弯刀综合征是一种少见但合并右位心的心血管畸形。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "短弯刀综合征" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "右位心" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "心血管畸形" } ] }, { "text": "主要表现为右位心、心房内脏位置正位右肺发育不良异常体动脉血流供应右肺右肺静脉异常引流入上腔静脉胸部X线平片上,异位引流的肺静脉在右肺野表现为“短弯刀”样阴影。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "右位心" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "心房" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "内脏" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "心房内脏位置正位" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "右肺" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "右肺发育不良" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "体动脉" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "右肺" }, { "start_idx": 23, "end_idx": 33, "type": "sym", "entity": "异常体动脉血流供应右肺" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "右肺" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "静脉" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 34, "end_idx": 46, "type": "sym", "entity": "右肺静脉异常引流入上腔静脉" }, { "start_idx": 47, "end_idx": 52, "type": "pro", "entity": "胸部X线平片" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "肺静脉" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "右肺野" } ] }, { "text": "临床上,在婴儿可表现为青紫、呼吸困难、活动后气促,通常伴有肺动脉高压心内复杂畸形反复上呼吸道感染胸部X线表现。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "青紫" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "活动后气促" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "肺动脉" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "肺动脉高压" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "心内" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "心内复杂畸形" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "呼吸道" }, { "start_idx": 40, "end_idx": 47, "type": "sym", "entity": "反复上呼吸道感染" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "胸部X线" } ] }, { "text": "(二)内脏反位右位心伴内脏反位较前者少见。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "内脏反位" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "右位心伴内脏反位" } ] }, { "text": "在已报告的病例中,近1/3至2/3合并有右心室双出口、肺动脉狭窄或闭锁及室间隔缺损。", "entities": [ { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "右心室双出口" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "肺动脉狭窄" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "室间隔缺损" } ] }, { "text": "主动脉弓通常为右位。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "主动脉弓通常为右位" } ] }, { "text": "表9-9右位心伴内脏正位及右位心伴内脏反位伴发畸形比较尽管绝大多数内脏反位的患儿心脏结构正常,但肺部疾患的发病率较正常儿有明显升高。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "右位心" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "内脏" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "畸形" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "内脏反位" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "心脏" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "肺部" } ] }, { "text": "第二节新生儿窒息新生儿窒息(asphyxiaofnewborn)是指由于产前、产时或产后的各种病因,在生后1分钟内无自主呼吸或未能建立规律呼吸,导致低氧血症和高碳酸血症,若持续存在,可出现代谢性酸中毒。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "新生儿窒息" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "新生儿窒息" }, { "start_idx": 14, "end_idx": 30, "type": "dis", "entity": "asphyxiaofnewborn" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "低氧血症" }, { "start_idx": 79, "end_idx": 83, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 94, "end_idx": 99, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "在分娩过程中,胎儿的呼吸和循环系统经历剧烈变化,绝大多数胎儿能够顺利完成这种从子宫内到子宫外环境的转变,从而建立有效的呼吸和循环,保证机体新陈代谢和各器官功能的正常,仅有少数患儿发生窒息。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "呼吸和循环系统" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "子宫内" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "子宫外" }, { "start_idx": 91, "end_idx": 92, "type": "dis", "entity": "窒息" } ] }, { "text": "国外文献报道活产婴儿的围生期窒息发生率约为1%~1.5%,而胎龄大于36周仅为5‰。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "窒息" } ] }, { "text": "【病因】窒息的本质是缺氧,凡能造成胎儿或新生儿血氧浓度降低的因素均可引起窒息,一种病因可通过不同途经影响机体,也可多种病因同时作用。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "窒息" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "缺氧" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "血氧浓度" }, { "start_idx": 17, "end_idx": 28, "type": "sym", "entity": "胎儿或新生儿血氧浓度降低" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "窒息" } ] }, { "text": "常见病因如下:1.孕母因素①缺氧性疾病:如呼吸衰竭、青紫型先天性心脏病、严重贫血及CO中毒等;②障碍胎盘循环的疾病:如充血性心力衰竭、妊娠高血压综合征、慢性肾炎、失血、休克、糖尿病和感染性疾病等;③其他:孕母吸毒、吸烟或被动吸烟、孕母年龄≥35岁或<16岁、多胎妊娠等,其胎儿窒息发生率增高。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "缺氧性疾病" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 26, "end_idx": 34, "type": "dis", "entity": "青紫型先天性心脏病" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "严重贫血" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "CO中毒" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "胎盘" }, { "start_idx": 59, "end_idx": 65, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 67, "end_idx": 74, "type": "dis", "entity": "妊娠高血压综合征" }, { "start_idx": 76, "end_idx": 79, "type": "dis", "entity": "慢性肾炎" }, { "start_idx": 81, "end_idx": 82, "type": "dis", "entity": "失血" }, { "start_idx": 84, "end_idx": 85, "type": "dis", "entity": "休克" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "糖尿病" }, { "start_idx": 91, "end_idx": 95, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 129, "end_idx": 132, "type": "sym", "entity": "多胎妊娠" }, { "start_idx": 138, "end_idx": 139, "type": "dis", "entity": "窒息" } ] }, { "text": "2.胎盘异常如前置胎盘、胎盘早剥和胎盘功能不全等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "胎盘" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "前置胎盘" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "胎盘早剥" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "胎盘功能不全" } ] }, { "text": "3.脐带异常如脐带受压、过短、过长致绕颈或绕体、脱垂、扭转或打结等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "脐带" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "脐带" }, { "start_idx": 7, "end_idx": 31, "type": "sym", "entity": "脐带受压、过短、过长致绕颈或绕体、脱垂、扭转或打结" } ] }, { "text": "4.分娩因素如难产、高位产钳、臀位、胎头吸引不顺利;产程中麻醉药、镇痛药及催产药使用不当等。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "难产" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "高位产钳" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "麻醉药" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "镇痛药" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "催产药" } ] }, { "text": "5.胎儿因素①早产儿、小于胎龄儿、巨大儿等;②各种畸形如后鼻孔闭锁、喉蹼、肺膨胀不全、先天性心脏病及宫内感染所致神经系统受损等;③胎粪吸入致使呼吸道阻塞等。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "畸形" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "后鼻孔" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "后鼻孔闭锁" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "喉蹼" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "肺膨胀不全" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "宫内感染" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "神经系统" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "神经系统受损" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "胎粪" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "呼吸道" }, { "start_idx": 71, "end_idx": 75, "type": "sym", "entity": "呼吸道阻塞" } ] }, { "text": "若由于上述各种病因导致窒息,则出现一系列病理生理变化。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "窒息" } ] }, { "text": "(一)窒息后细胞损伤缺氧可导致细胞代谢及功能障碍和结构异常甚至死亡,是细胞损伤从可逆到不可逆的演变过程。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "窒息" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "缺氧" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "细胞" } ] }, { "text": "不同细胞对缺氧的易感性各异,其中脑细胞最敏感,其次是心肌、肝和肾上腺细胞,而纤维、上皮及骨骼肌细胞对缺氧的耐受性较强。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "细胞" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "缺氧" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "脑细胞" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "心肌" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "肝" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "肾上腺细胞" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "纤维" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "上皮" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "骨骼肌细胞" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "缺氧" } ] }, { "text": "1.可逆性细胞损伤细胞所需能量主要由线粒体生成的ATP供给。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "细胞" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "细胞" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "ATP" } ] }, { "text": "缺氧首先是细胞有氧代谢即线粒体内氧化磷酸化发生障碍,使ATP产生减少甚至停止。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "缺氧" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "细胞" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "氧化磷酸化" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "ATP" } ] }, { "text": "由于能源缺乏,加之缺氧,导致细胞代谢及功能异常:①葡萄糖无氧酵解增强:无氧酵解使葡萄糖和糖原消耗增加,易出现低血糖;同时也使乳酸增多,引起代谢性酸中毒。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "缺氧" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "细胞代谢及功能异常" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "糖原" }, { "start_idx": 54, "end_idx": 56, "type": "sym", "entity": "低血糖" }, { "start_idx": 69, "end_idx": 74, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "②细胞水肿:由于ATP缺乏,钠泵主动转运障碍,使钠、水潴留。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "细胞水肿" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "ATP" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "钠泵" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "钠" } ] }, { "text": "③钙离子内流增加:由于钙泵主动转运的障碍,使钙向细胞内流动增多。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "钙离子" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "钙泵" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "钙" } ] }, { "text": "④核蛋白脱落:由于核蛋白从粗面内质网脱落,使蛋白和酶等物质的合成减少。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "核蛋白" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "核蛋白" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "蛋白" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "酶" } ] }, { "text": "本阶段如能恢复血流灌注和供氧,上述变化可恢复,一般不留后遗症。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "血流灌注" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "供氧" } ] }, { "text": "2.不可逆性细胞损伤若窒息持续存在或严重缺氧,将导致不可逆性细胞损伤:①严重的线粒体形态和功能异常:不能进行氧化磷酸化、ATP产生障碍,线粒体产能过程中断;②细胞膜严重损伤:丧失其屏障和转运功能;③溶酶体破裂:由于溶酶体膜损伤,溶酶体酶扩散到细胞质中,消化细胞内各种成分(自溶)。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "细胞" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "窒息" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "严重缺氧" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "细胞" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "线粒体" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "ATP" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "细胞膜" }, { "start_idx": 99, "end_idx": 101, "type": "bod", "entity": "溶酶体" }, { "start_idx": 107, "end_idx": 110, "type": "bod", "entity": "溶酶体膜" }, { "start_idx": 114, "end_idx": 117, "type": "bod", "entity": "溶酶体酶" }, { "start_idx": 121, "end_idx": 123, "type": "bod", "entity": "细胞质" }, { "start_idx": 126, "end_idx": 129, "type": "bod", "entity": "消化细胞" } ] }, { "text": "此阶段即使恢复血流灌注和供氧,上述变化亦不可完全恢复。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "血流灌注" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "供氧" } ] }, { "text": "3.血流再灌注损伤复苏后,由于血流再灌注可导致细胞内钙超载和氧自由基增加,从而引起细胞的进一步损伤。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血流" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血流" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "细胞" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "钙" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "氧自由基" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "细胞" } ] }, { "text": "(二)窒息发展过程1.原发性呼吸暂停(primaryapnea)当胎儿或新生儿发生低氧血症、高碳酸血症和代谢性酸中毒时,由于儿茶酚胺分泌增加,呼吸和心率增快,机体血流重新分布即选择性血管收缩,使次要的组织和器官(如肺、肠、肾、肌肉、皮肤等)血流量减少,而主要的生命器官(如脑、心肌、肾上腺)的血流量增多,血压增高,心输出量增加。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "窒息" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "原发性呼吸暂停" }, { "start_idx": 19, "end_idx": 30, "type": "dis", "entity": "primaryapnea" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "低氧血症" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 52, "end_idx": 57, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "儿茶酚胺" }, { "start_idx": 71, "end_idx": 72, "type": "ite", "entity": "呼吸" }, { "start_idx": 74, "end_idx": 75, "type": "ite", "entity": "心率" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "血流" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "血管" }, { "start_idx": 107, "end_idx": 107, "type": "bod", "entity": "肺" }, { "start_idx": 109, "end_idx": 109, "type": "bod", "entity": "肠" }, { "start_idx": 111, "end_idx": 111, "type": "bod", "entity": "肾" }, { "start_idx": 113, "end_idx": 114, "type": "bod", "entity": "肌肉" }, { "start_idx": 116, "end_idx": 117, "type": "bod", "entity": "皮肤" }, { "start_idx": 120, "end_idx": 120, "type": "bod", "entity": "血" }, { "start_idx": 136, "end_idx": 136, "type": "bod", "entity": "脑" }, { "start_idx": 138, "end_idx": 139, "type": "bod", "entity": "心肌" }, { "start_idx": 141, "end_idx": 143, "type": "bod", "entity": "肾上腺" }, { "start_idx": 146, "end_idx": 150, "type": "sym", "entity": "血流量增多" }, { "start_idx": 152, "end_idx": 155, "type": "sym", "entity": "血压增高" }, { "start_idx": 157, "end_idx": 162, "type": "sym", "entity": "心输出量增加" } ] }, { "text": "如低氧血症和酸中毒持续存在则出现呼吸停止,称为原发性呼吸暂停。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "低氧血症" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "酸中毒" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "原发性呼吸暂停" } ] }, { "text": "此时肌张力存在,血压仍高,循环尚好,但发绀加重,伴有心率减慢。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "血压仍高" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "发绀加重" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "心率减慢" } ] }, { "text": "在此阶段若病因解除,经过清理呼吸道和物理刺激即可恢复自主呼吸。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "呼吸道" } ] }, { "text": "2.继发性呼吸暂停(secondaryapnea)若病因未解除,低氧血症持续存在,肺、肠、肾、肌肉和皮肤等血流量严重减少,脑、心肌和肾上腺的血流量也减少,可导致机体各器官功能和形态损伤,如脑和心肌损伤、休克、应激性溃疡等。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "继发性呼吸暂停" }, { "start_idx": 10, "end_idx": 23, "type": "dis", "entity": "secondaryapnea" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "低氧血症" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "肺" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "肠" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "肾" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "肌肉" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "皮肤" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "血" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "脑" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "心肌" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "肾上腺" }, { "start_idx": 70, "end_idx": 70, "type": "bod", "entity": "血" }, { "start_idx": 94, "end_idx": 94, "type": "bod", "entity": "脑" }, { "start_idx": 96, "end_idx": 97, "type": "bod", "entity": "心肌" }, { "start_idx": 94, "end_idx": 99, "type": "sym", "entity": "脑和心肌损伤" }, { "start_idx": 101, "end_idx": 102, "type": "sym", "entity": "休克" }, { "start_idx": 104, "end_idx": 108, "type": "sym", "entity": "应激性溃疡" } ] }, { "text": "在原发性呼吸暂停后出现几次喘息样呼吸,继而出现呼吸停止,即所谓的继发性呼吸暂停。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "原发性呼吸暂停" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "喘息样呼吸" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "继发性呼吸暂停" } ] }, { "text": "此时肌张力消失,苍白,心率和血压持续下降,出现心力衰竭及休克等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "肌张力消失" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "苍白" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "心率" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "血压" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "休克" } ] }, { "text": "此阶段对清理呼吸道和物理刺激无反应,需正压通气方可恢复自主呼吸。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "呼吸道" } ] }, { "text": "否则将死亡,存活者可留有后遗症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "死亡" } ] }, { "text": "窒息是从原发性呼吸暂停到继发性呼吸暂停的发展过程,但两种呼吸暂停的表现均为无呼吸和心率低于100次/分,故临床上难以鉴别,为了不延误抢救时机,对生后无呼吸者都应按继发性呼吸暂停进行处理。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "窒息" }, { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "原发性呼吸暂停" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "继发性呼吸暂停" }, { "start_idx": 41, "end_idx": 42, "type": "ite", "entity": "心率" }, { "start_idx": 81, "end_idx": 87, "type": "dis", "entity": "继发性呼吸暂停" } ] }, { "text": "(三)窒息后血液生化和代谢改变在窒息应激状态时,儿茶酚胺及胰高血糖素释放增加,使早期血糖正常或增高;当缺氧持续,动用糖增加、糖原贮存空虚,出现低血糖症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "窒息" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血液" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "窒息" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "儿茶酚胺" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "胰高血糖素" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "血糖" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "缺氧" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "低血糖症" } ] }, { "text": "此外,酸中毒抑制胆红素与清蛋白结合,降低肝内酶的活力而致高间接胆红素血症;由于左心房心钠素分泌增加,造成低钠血症等。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "酸中毒" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肝内酶" }, { "start_idx": 28, "end_idx": 35, "type": "dis", "entity": "高间接胆红素血症" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "左心房" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "低钠血症" } ] }, { "text": "【临床表现】(一)胎儿缺氧表现先出现胎动增加、胎心增快,胎心率≥160次/分;晚期则胎动减少(<20次/12小时),甚至消失,胎心减慢,胎心率<100次/分,严重时甚至心脏停搏;窒息可导致肛门括约肌松弛,排出胎便,使羊水呈黄绿色。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "胎儿缺氧表现" }, { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "胎动" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "胎心" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "胎心率" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "胎动" }, { "start_idx": 63, "end_idx": 64, "type": "ite", "entity": "胎心" }, { "start_idx": 68, "end_idx": 70, "type": "ite", "entity": "胎心率" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "心脏" }, { "start_idx": 84, "end_idx": 87, "type": "sym", "entity": "心脏停搏" }, { "start_idx": 89, "end_idx": 90, "type": "dis", "entity": "窒息" }, { "start_idx": 94, "end_idx": 98, "type": "bod", "entity": "肛门括约肌" }, { "start_idx": 94, "end_idx": 100, "type": "sym", "entity": "肛门括约肌松弛" }, { "start_idx": 108, "end_idx": 113, "type": "sym", "entity": "羊水呈黄绿色" } ] }, { "text": "(二)窒息判定Apgar评分是临床评价出生窒息的经典而简易的方法。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "dis", "entity": "窒" }, { "start_idx": 3, "end_idx": 3, "type": "pro", "entity": "窒" }, { "start_idx": 17, "end_idx": 17, "type": "dis", "entity": "评" }, { "start_idx": 17, "end_idx": 17, "type": "pro", "entity": "评" } ] }, { "text": "1分钟评分与动脉血pH相关,但不完全一致,如母亲分娩时用麻醉药或止痛药使新生儿生后呼吸抑制,Apgar评分虽低,但无宫内缺氧,血气改变相对较轻。", "entities": [ { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "麻醉药" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "止痛药" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "呼吸抑制" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "宫内缺氧" }, { "start_idx": 63, "end_idx": 64, "type": "ite", "entity": "血气" } ] }, { "text": "2.Apgar评分内容包括皮肤颜色(appearance)、心率(pulse)、对刺激的反应(grimace)、肌张力(activity)和呼吸(respiration)。", "entities": [ { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "心率" }, { "start_idx": 33, "end_idx": 37, "type": "ite", "entity": "pulse" } ] }, { "text": "1分钟Apgar评分8~10为正常,4~7分应密切注意窒息的可能性,0~3分为窒息。", "entities": [ { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "窒息" } ] }, { "text": "4.评估的意义1分钟评分反映窒息严重程度;5分钟及10分钟评分除反映窒息的严重程度外,还可反映复苏抢救的效果。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "窒息" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "窒息" } ] }, { "text": "5.注意事项应客观、快速及准确地进行评估;胎龄小的早产儿成熟度低,虽无窒息,但评分较低;单凭Apgar评分不应作为评估低氧或产时窒息以及神经系统预后的唯一指标。", "entities": [ { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "窒息" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "窒息" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "神经系统" } ] }, { "text": "(三)并发症由于窒息不同,发生器官损害的种类及严重程度各异。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "dis", "entity": "窒" }, { "start_idx": 8, "end_idx": 8, "type": "pro", "entity": "窒" } ] }, { "text": "【辅助检查】对宫内缺氧胎儿,可通过羊膜镜了解胎粪污染羊水的程度,或在胎头露出宫口时取胎儿头皮血进行血气分析,以估计宫内缺氧程度;生后应检测动脉血气、血糖、电解质、血尿素氮和肌酐等生化指标。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "equ", "entity": "羊膜镜" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "头皮血" }, { "start_idx": 49, "end_idx": 52, "type": "ite", "entity": "血气分析" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "缺氧" }, { "start_idx": 69, "end_idx": 72, "type": "ite", "entity": "动脉血气" }, { "start_idx": 74, "end_idx": 75, "type": "ite", "entity": "血糖" }, { "start_idx": 77, "end_idx": 79, "type": "ite", "entity": "电解质" }, { "start_idx": 81, "end_idx": 84, "type": "ite", "entity": "血尿素氮" }, { "start_idx": 86, "end_idx": 87, "type": "ite", "entity": "肌酐" } ] }, { "text": "【诊断】目前,我国新生儿窒息的诊断及程度判定仍依赖单独Apgar评分,但由于Apgar评分受多种因素的影响,单凭Apgar评分并不能准确诊断窒息及预测神经发育结局。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "新生儿窒息" } ] }, { "text": "因此,1996年,美国儿科学会(AAP)和妇产科学会(ACOG)将围生期窒息定义为:①严重的代谢性酸中毒(pH<7);②5分钟后Apgar评分仍≤3分;③有新生儿脑病表现;④伴有多器官功能障碍。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dep", "entity": "儿科" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "围生期窒息" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 78, "end_idx": 82, "type": "dis", "entity": "新生儿脑病" } ] }, { "text": "【治疗与预防】复苏(resuscitation)必须分秒必争,由儿科医生和助产士(师)合作进行。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "复苏" }, { "start_idx": 32, "end_idx": 33, "type": "dep", "entity": "儿科" } ] }, { "text": "(一)复苏方案采用国际公认的ABCDE复苏方案:①A(airway):清理呼吸道;②B(breathing):建立呼吸;③C(circulation):恢复循环;④D(drugs):药物治疗;⑤E(evaluationandenvironment):评估和环境(保温)。", "entities": [ { "start_idx": 35, "end_idx": 39, "type": "pro", "entity": "清理呼吸道" }, { "start_idx": 55, "end_idx": 58, "type": "pro", "entity": "建立呼吸" }, { "start_idx": 76, "end_idx": 79, "type": "pro", "entity": "恢复循环" }, { "start_idx": 91, "end_idx": 94, "type": "pro", "entity": "药物治疗" }, { "start_idx": 125, "end_idx": 133, "type": "pro", "entity": "评估和环境(保温)" } ] }, { "text": "其中评估和保温(E)贯穿于整个复苏过程中。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "评估和保温" } ] }, { "text": "执行ABCD每一步骤的前后,应对评价指标即呼吸、心率(计数6秒钟心率然后乘10)和皮肤颜色进行评估。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "呼吸" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "心率" }, { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "心率" } ] }, { "text": "复苏初期建议用纯氧(目前证据尚不足以证明空气复苏的有效性),以后通过监测动脉血气值或经皮血氧饱和度,逐步调整吸入气的氧浓度。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "纯氧" }, { "start_idx": 36, "end_idx": 40, "type": "ite", "entity": "动脉血气值" } ] }, { "text": "随着复苏理论和实践的进步,已证实一些复苏方法存在很多弊端,临床复苏时应予注意:①气道未清理干净前(尤其是胎粪污染儿),切忌刺激新生儿使其大哭,以免将气道内吸入物进一步吸入肺内。", "entities": [ { "start_idx": 85, "end_idx": 85, "type": "bod", "entity": "肺" } ] }, { "text": "清理呼吸道和触觉刺激后30秒钟仍无自主呼吸,应视为继发性呼吸暂停,即刻改用正压通气。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "呼吸道" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "继发性呼吸暂停" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "正压通气" } ] }, { "text": "②复苏过程中禁用呼吸兴奋剂。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "呼吸兴奋剂" } ] }, { "text": "③复苏过程中禁用高张葡萄糖,因为应激时血糖已升高,给予高张葡萄糖可增加颅内出血发生的机会,同时糖的无氧酵解增加,加重代谢性酸中毒。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "高张葡萄糖" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "血糖" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "高张葡萄糖" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "颅内出血" }, { "start_idx": 58, "end_idx": 63, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "用温热毛巾揩干头部及全身,以减少散热;摆好体位,肩部以布卷垫高2~3cm,使颈部轻微伸仰,然后进行复苏。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "全身" } ] }, { "text": "1.清理呼吸道(A)新生儿娩出后,应立即吸净口和鼻腔的黏液,因鼻腔较敏感,受刺激后易触发呼吸,故应先吸口腔,后吸鼻腔(图5-5);如羊水混有胎粪,无论胎粪是稠是稀,胎儿一经娩出后,立刻进行有无活力评估,有活力的新生儿继续初步复苏,无活力者应立即气管插管,吸净气道内的胎粪,然后再建立呼吸(有活力的定义是呼吸规则、肌张力好及心率>100次/分,以上三项中有一项不好即为无活力)。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "清理呼吸道" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "鼻腔" }, { "start_idx": 122, "end_idx": 125, "type": "pro", "entity": "气管插管" }, { "start_idx": 139, "end_idx": 142, "type": "pro", "entity": "建立呼吸" }, { "start_idx": 156, "end_idx": 159, "type": "sym", "entity": "肌张力好" }, { "start_idx": 161, "end_idx": 162, "type": "ite", "entity": "心率" } ] }, { "text": "2.建立呼吸(B)包括触觉刺激和正压通气:①触觉刺激:清理呼吸道后拍打或弹足底1~2次或沿长轴快速摩擦腰背皮肤1~2次(图5-5、5-5)(切忌不要超过2次或粗暴拍打),如出现正常呼吸,心率>100次/分,肤色红润可继续观察。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "建立呼吸" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "正压通气" }, { "start_idx": 44, "end_idx": 58, "type": "pro", "entity": "沿长轴快速摩擦腰背皮肤1~2次" }, { "start_idx": 93, "end_idx": 94, "type": "ite", "entity": "心率" } ] }, { "text": "②正压通气:触觉刺激后仍呼吸暂停或抽泣样呼吸,或心率<100次/分,或持续的中心性发绀,需用面罩正压通气(图5-4)。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "正压通气" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "心率" }, { "start_idx": 35, "end_idx": 42, "type": "sym", "entity": "持续的中心性发绀" } ] }, { "text": "通气频率40~60次/分,吸呼比1∶2,压力20~40cmH2O,即可见胸廓扩张和听诊呼吸音正常为宜。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "通气" } ] }, { "text": "气囊面罩正压通气30秒后,如自主呼吸不充分或心率<100次/分,需继续气囊面罩或气管插管正压通气。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "equ", "entity": "气囊面罩" }, { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "正压通气" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "心率" }, { "start_idx": 35, "end_idx": 38, "type": "equ", "entity": "气囊面罩" } ] }, { "text": "图5-1吸引先口腔后鼻腔图5-2拍打足底及弹足底图5-3摩擦后背图5-4面罩正压通气3.恢复循环(C)即胸外心脏按压。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "pro", "entity": "吸引先口腔后鼻腔" }, { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "拍打足底及弹足底" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "摩擦后背" }, { "start_idx": 36, "end_idx": 37, "type": "equ", "entity": "面罩" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "正压通气" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "恢复循环" }, { "start_idx": 52, "end_idx": 57, "type": "pro", "entity": "胸外心脏按压" } ] }, { "text": "如气管插管正压通气30秒后,心率<60次/分或心率在60~80次/分不再增加,应在继续正压通气的同时,进行胸外心脏按压。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "正压通气" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "心率" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "心率" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "正压通气" }, { "start_idx": 53, "end_idx": 58, "type": "pro", "entity": "胸外心脏按压" } ] }, { "text": "方法是:采用双拇指或中食指按压胸骨体下1/3处,频率为90次/分,胸外按压和正压通气的比例为3∶1(每按压3次,正压通气1次),按压深度为胸廓前后径的1/3(图5-5)。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "双拇指" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "中食指" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "胸骨" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "胸外按压" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "正压通气" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "正压通气" } ] }, { "text": "按压或抬起过程中,双拇指或中食指指端不能离开胸骨按压部位,也不宜用力过大以免损伤。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "双拇指" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "中食指指端" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "胸骨" } ] }, { "text": "4.药物治疗(D)目的是改善心脏功能、增加组织灌流和恢复酸碱平衡。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心脏" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "组织" } ] }, { "text": "(1)肾上腺素:①作用:可直接兴奋心肌起搏组织和传导系统的β受体,使心率加快,心输出量增加,同时兴奋血管α受体,使血管收缩,血压增高;②指征:心率为0或胸外心脏按压30秒后,心率仍持续<60次/分;③方法:给予1∶10000肾上腺素,0.1~0.3ml/kg静脉注入,或0.3~1ml/kg气管内注入,3~5分钟重复一次;④疗效评价:给药30秒后,有效者心率≥100次/分;无效者应考虑是否存在代谢性酸中毒和有效血容量减少等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 34, "end_idx": 35, "type": "ite", "entity": "心率" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "血压增高" }, { "start_idx": 71, "end_idx": 72, "type": "ite", "entity": "心率" }, { "start_idx": 76, "end_idx": 81, "type": "pro", "entity": "胸外心脏按压" }, { "start_idx": 87, "end_idx": 88, "type": "ite", "entity": "心率" }, { "start_idx": 112, "end_idx": 115, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 129, "end_idx": 132, "type": "pro", "entity": "静脉注入" }, { "start_idx": 177, "end_idx": 178, "type": "ite", "entity": "心率" }, { "start_idx": 197, "end_idx": 202, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "(2)扩容剂:①作用:增加血容量,改善循环。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "扩容剂" } ] }, { "text": "②指征:有急性失血的病史,疑似失血或休克(伴有血容量减少表现)。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "失血" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "休克" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "血容量减少" } ] }, { "text": "③方法:可给予等渗透晶体液,如生理盐水,对大量失血者可选择红细胞悬液。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "生理盐水" }, { "start_idx": 29, "end_idx": 33, "type": "dru", "entity": "红细胞悬液" } ] }, { "text": "④疗效:有效者脉搏有力、血压上升、皮肤转红及代谢性酸中毒减轻。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "脉搏" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "血压" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "(3)纳洛酮(naloxone):①作用:是半合成吗啡拮抗剂,阻断吗啡样物质与其受体结合,从而拮抗所有吗啡类镇痛药的呼吸抑制、缩瞳、胆总管痉挛及致幻作用,并降低镇痛效应。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "纳洛酮" }, { "start_idx": 22, "end_idx": 29, "type": "dru", "entity": "半合成吗啡拮抗剂" }, { "start_idx": 51, "end_idx": 56, "type": "dru", "entity": "吗啡类镇痛药" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "呼吸抑制" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "缩瞳" }, { "start_idx": 66, "end_idx": 70, "type": "sym", "entity": "胆总管痉挛" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "致幻" } ] }, { "text": "②指征:生后有呼吸抑制表现,其母亲产前4小时内用过吗啡类麻醉镇痛药者。", "entities": [ { "start_idx": 25, "end_idx": 32, "type": "dru", "entity": "吗啡类麻醉镇痛药" } ] }, { "text": "③方法:应给予纳洛酮,每次0.1mg/kg,静脉或肌肉注射或气管内注入,均应快速输入。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "纳洛酮" }, { "start_idx": 22, "end_idx": 28, "type": "pro", "entity": "静脉或肌肉注射" } ] }, { "text": "但应注意,纳洛酮不选择作为产房有呼吸抑制新生儿开始复苏的措施,应在保证通气情况下,使用该药物。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "纳洛酮" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "呼吸抑制" } ] }, { "text": "图5-5胸外心脏按压(三)复苏后的监护与转运复苏后需监测肤色、体温、呼吸、心率、血压、尿量、血气、血糖和电解质等。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "胸外心脏按压" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "体温" }, { "start_idx": 34, "end_idx": 35, "type": "ite", "entity": "呼吸" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "心率" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "血压" }, { "start_idx": 43, "end_idx": 44, "type": "ite", "entity": "尿量" }, { "start_idx": 46, "end_idx": 47, "type": "ite", "entity": "血气" }, { "start_idx": 49, "end_idx": 50, "type": "ite", "entity": "血糖" }, { "start_idx": 52, "end_idx": 54, "type": "ite", "entity": "电解质" } ] }, { "text": "如并发症严重,需转运到NICU治疗,转运中需注意保温、监护生命指标和予以必要的治疗。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dep", "entity": "NICU" } ] }, { "text": "【预防】①加强围生期保健,及时处理高危妊娠;②加强胎儿监护,避免和及时纠正宫内缺氧;③密切监测临产孕妇,避免难产;④培训接产人员熟练掌握复苏技术;⑤医院产房内需配备复苏设备,高危妊娠分娩时必须有掌握复苏技术的人员在场。", "entities": [ { "start_idx": 82, "end_idx": 85, "type": "equ", "entity": "复苏设备" } ] }, { "text": "第三节呼吸的控制和肺部气体交换多种原因要求理解和掌握小儿呼吸控制系统的原理。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肺部" } ] }, { "text": "在小儿出生后的呼吸适应涉及肺、心脏和中枢神经系统功能和结构上的调节;临床生理和病理生理变化使呼吸控制问题居于首位,比如气道阻塞、重症哮喘、心力衰竭、通气不足、低氧血症等;神经系统在调节呼吸上的重要作用显得尤为重要,比如新生儿缺氧缺血性脑损害、超低出生体重新生儿脑发育和呼吸暂停、小儿颅内感染和损伤等。", "entities": [ { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肺" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心脏" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "气道阻塞" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "哮喘" }, { "start_idx": 69, "end_idx": 72, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "通气不足" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "低氧血症" }, { "start_idx": 85, "end_idx": 88, "type": "bod", "entity": "神经系统" }, { "start_idx": 109, "end_idx": 119, "type": "dis", "entity": "新生儿缺氧缺血性脑损害" }, { "start_idx": 123, "end_idx": 126, "type": "ite", "entity": "出生体重" }, { "start_idx": 130, "end_idx": 130, "type": "bod", "entity": "脑" }, { "start_idx": 121, "end_idx": 137, "type": "sym", "entity": "超低出生体重新生儿脑发育和呼吸暂停" }, { "start_idx": 139, "end_idx": 147, "type": "sym", "entity": "小儿颅内感染和损伤" } ] }, { "text": "(一)呼吸中枢呼吸中枢为中枢神经系统内产生和调节呼吸运动的神经细胞群。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "呼吸中枢" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "呼吸中枢" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "神经细胞群" } ] }, { "text": "呼吸的节律性来自呼吸中枢的节律性活动,由产生和调节呼吸运动的神经细胞群组成。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "呼吸中枢" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "神经细胞群" } ] }, { "text": "这些细胞群广泛分布在大脑皮层、间脑、脑桥、延髓和脊髓部位,并在产生和调节呼吸运动时发挥不同的作用,并彼此协调和制约,对于传入的冲动加以整合。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "大脑皮层" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "间脑" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "脑桥" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "延髓" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "脊髓" } ] }, { "text": "1.脊髓脊髓中支配呼吸肌的运动神经元位于颈3~5(支配膈肌)和胸段(支配肋间肌和腹肌)前角。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "脊髓" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "脊髓中" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "运动神经元" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "颈" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "膈肌" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "胸" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "肋间肌" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "腹肌" } ] }, { "text": "脊髓不产生节律性呼吸,主要为联系高位脑和呼吸肌的中继站,但也作为整合某些呼吸反射的初级中枢。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脊髓" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "高位脑" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "呼吸肌" } ] }, { "text": "2.低位脑干脑桥和延髓组成低位脑干,是产生呼吸节律的部位。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "低位脑干" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脑桥" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "延髓" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "低位脑干" } ] }, { "text": "脑桥上部为抑制呼吸的结构,脑桥中下部有长吸气中枢,为呼吸调整中枢。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脑桥" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "脑桥" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "长吸气中枢" } ] }, { "text": "延髓中有呼吸神经元,包括吸气神经元、呼气神经元、吸气-呼气神经元、呼气-吸气神经元。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "延髓" }, { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "呼吸神经元" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "吸气神经元" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "呼气神经元" }, { "start_idx": 24, "end_idx": 31, "type": "bod", "entity": "吸气-呼气神经元" }, { "start_idx": 33, "end_idx": 40, "type": "bod", "entity": "呼气-吸气神经元" } ] }, { "text": "这些神经元在延髓中主要集中在背侧和腹侧,其轴突交叉到对侧并下行至脊髓颈段,支配膈神经运动神经元,或支配脊髓肋间呼吸内、外肌和腹肌的运动神经元。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "神经元" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "延髓" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "背侧" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "腹侧" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "脊髓颈段" }, { "start_idx": 39, "end_idx": 46, "type": "bod", "entity": "膈神经运动神经元" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "脊髓" }, { "start_idx": 53, "end_idx": 60, "type": "bod", "entity": "肋间呼吸内、外肌" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "腹肌" }, { "start_idx": 65, "end_idx": 69, "type": "bod", "entity": "运动神经元" } ] }, { "text": "但也可经同侧作为舌咽神经和迷走神经传出,支配咽喉部呼吸辅助肌。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "舌咽神经" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "迷走神经" }, { "start_idx": 22, "end_idx": 29, "type": "bod", "entity": "咽喉部呼吸辅助肌" } ] }, { "text": "产生呼吸节律的神经结构较广泛,使呼吸节律发源于多部位,不易因局灶性损害而丧失呼吸节律。", "entities": [ { "start_idx": 27, "end_idx": 41, "type": "sym", "entity": "不易因局灶性损害而丧失呼吸节律" } ] }, { "text": "3.高位脑大脑皮层、边缘系统、下丘脑等作为高位脑,可以随意控制呼吸、屏气、加强加快呼吸,是随意呼吸调节系统,而低位脑干对呼吸调节系统是不随意的自主节律呼吸调节系统。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "高位脑" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "大脑皮层" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "边缘系统" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "下丘脑" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "高位脑" }, { "start_idx": 47, "end_idx": 52, "type": "bod", "entity": "呼吸调节系统" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "低位脑干" }, { "start_idx": 60, "end_idx": 65, "type": "bod", "entity": "呼吸调节系统" }, { "start_idx": 71, "end_idx": 80, "type": "bod", "entity": "自主节律呼吸调节系统" } ] }, { "text": "新生儿和婴幼儿的自主呼吸节律自出生后一直处于发育中,不断通过呼吸肌运动和肺扩张运动,并经反射刺激呼吸中枢的发育、调节和整合功能,并在儿童早期基本完成随意呼吸运动及调节的发育成熟。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "肺" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "呼吸中枢" } ] }, { "text": "此外,呼吸和吞咽的运动协调,呼吸对心血管功能的调节,呼吸和全身运动等,均随小儿生理发育而成熟。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "心血管" } ] }, { "text": "(二)中枢对呼吸的调节作用呼吸中枢通过反馈机制控制呼吸活动,以保持全身性氧和二氧化碳代谢平衡。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "中枢" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "呼吸中枢" }, { "start_idx": 36, "end_idx": 41, "type": "mic", "entity": "氧和二氧化碳" } ] }, { "text": "其作用依靠中枢和外周化学感受器、外周牵张感受器。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "中枢" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "外周化学感受器" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "外周牵张感受器" } ] }, { "text": "呼吸肌与胸廓依靠中枢发放的冲动保持呼吸运动,但容易因过度疲劳而衰竭。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "胸廓" } ] }, { "text": "1.化学感受器位于颈动脉和主动脉的化学感受器对外周动脉血氧、二氧化碳分压和pH水平敏感,冲动经窦神经和迷走神经传入延髓。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "化学感受器" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "颈动脉" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "主动脉" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "化学感受器" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "冲动经窦神经" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "迷走神经" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "延髓" } ] }, { "text": "低氧、二氧化碳增高、pH增高可以刺激呼吸加深加快,并主要经主动脉体带来血液循环变化。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "主动脉体" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "血液" } ] }, { "text": "延髓中枢化学感受器主要对二氧化碳变化敏感,而不感受氧水平的变化。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "延髓中枢化学感受器" } ] }, { "text": "血液中的二氧化碳能够迅速通过血脑屏障,导致延髓化学感受区周围液体中[H+]升高,引起中枢呼吸兴奋。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血液" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "血脑屏障" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "延髓化学感受区" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "中枢呼吸兴奋" } ] }, { "text": "在一定范围内,二氧化碳水平升高可以刺激呼吸兴奋,超过一定范围,则表现为呼吸抑制。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "呼吸兴奋" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "呼吸抑制" } ] }, { "text": "二氧化碳刺激呼吸兴奋的作用主要依靠中枢化学感受器(80%),其次为外周化学感受器(20%)。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "呼吸兴奋" }, { "start_idx": 17, "end_idx": 23, "type": "bod", "entity": "中枢化学感受器" }, { "start_idx": 33, "end_idx": 39, "type": "bod", "entity": "外周化学感受器" } ] }, { "text": "低氧则主要通过外周化学感受器使呼吸兴奋,而对于中枢则是呼吸抑制。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "外周化学感受器" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "呼吸兴奋" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "中枢" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "呼吸抑制" } ] }, { "text": "如果持续低氧,对外周化学感受器刺激的反射作用不能抵消对中枢化学感受器呼吸抑制的刺激,则出现呼吸抑制。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "外周化学感受器" }, { "start_idx": 27, "end_idx": 33, "type": "bod", "entity": "中枢化学感受器" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "呼吸抑制" } ] }, { "text": "2.外周牵张感受器肺牵张反射又称为赫-布反射(Hering-Breuerreflex),为吸气相限制反射。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "外周牵张感受器" } ] }, { "text": "当肺扩张时,气道牵张作用于气管至细支气管平滑肌内的感受器,经迷走神经传入中枢延髓,可以出现抑制性吸气神经元冲动,避免肺过度扩张,也称为迷走反射。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肺" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "气道" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "气管" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "细支气管平滑肌" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "感受器" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "迷走神经" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "中枢延髓" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "吸气神经元" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "肺" } ] }, { "text": "亥氏反射(Head’sreflex)为深吸气反射,当肺扩张时出现的加强性吸气。", "entities": [ { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "肺" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "加强性吸气" } ] }, { "text": "3.防御性呼吸反射分布在呼吸道黏膜上皮的感受器对机械或化学刺激敏感,经迷走神经传入延髓,触发一系列协调的反射效应,为咳嗽反射。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "防御性呼吸反射" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "呼吸道黏膜" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "感受器" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "迷走神经" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "延髓" } ] }, { "text": "咳嗽反射包括深吸气、声门紧闭、呼气肌强烈收缩、肺内压和胸腔内压骤然上升、声门突然打开等序列过程,气流以极高的速度从肺内冲出,并将气道内异物和分泌物带出。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "深吸气" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "声门紧闭" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "呼气肌强烈收缩" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "肺内压" }, { "start_idx": 27, "end_idx": 30, "type": "ite", "entity": "胸腔内压" }, { "start_idx": 23, "end_idx": 34, "type": "sym", "entity": "肺内压和胸腔内压骤然上升" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "声门突然打开" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "肺" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "气道" } ] }, { "text": "剧烈咳嗽可以导致胸腔内压急剧升高,使静脉压和脑脊液压升高。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "ite", "entity": "胸腔内压" }, { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "静脉压" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "脑脊液压" } ] }, { "text": "如果刺激主要作用在鼻腔黏膜,则出现喷嚏反射,感受器经三叉神经传入冲动,带来软腭下垂,呼出气主要从鼻腔喷出,达到清除鼻腔中的刺激物。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "鼻腔黏膜" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "感受器" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "三叉神经" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "软腭" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "鼻腔" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "鼻腔" } ] }, { "text": "(三)呼吸肌和呼吸功能肺通气动力为呼吸肌运动,分为吸气(进气)和呼气(排气)过程。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "肺" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "呼吸肌" } ] }, { "text": "自然呼吸时收缩呼吸肌作用使胸腔内负压增大,胸廓增大同时使肺扩张,即肺泡的膨胀。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "收缩呼吸肌" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胸腔" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "胸廓" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肺" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肺泡" } ] }, { "text": "肺内压力低于外界大气压力,气流经气道进入肺泡,在吸气末肺泡内压与外界大气压相同时,气流流动停止,进气结束。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "气道" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肺泡" } ] }, { "text": "呼气时收缩呼吸肌舒张(或伴有呼气肌收缩),胸廓缩小,或者在肺组织弹性回缩作用下,使肺泡内压力高于外界大气压力,肺内气体向肺外流动,在呼气末肺内压与外界大气压相等时,气流流动停止,呼气结束。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "收缩呼吸肌" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "呼气肌" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "胸廓" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "肺" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "肺泡" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "肺" }, { "start_idx": 69, "end_idx": 69, "type": "bod", "entity": "肺" } ] }, { "text": "新生儿和小婴儿肋间呼吸肌发育不全,呼吸运动主要依赖膈肌,为腹式呼吸。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "肋间呼吸肌" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "膈肌" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "腹式呼吸" } ] }, { "text": "到幼儿和儿童期,随着肋间肌发育逐渐完善,可以同时有胸式和腹式呼吸,且中枢意识控制的随意呼吸功能完善。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肋间肌" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "胸" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "腹" }, { "start_idx": 22, "end_idx": 31, "type": "sym", "entity": "同时有胸式和腹式呼吸" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "中枢" } ] }, { "text": "(四)肺的气体交换功能1.呼吸频率和节律呼吸频率指每分钟呼吸次数,呼吸节律指呼吸活动的时间规律性。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" } ] }, { "text": "新生儿多表现为呼吸节律不齐。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "呼吸节律不齐" } ] }, { "text": "周期性呼吸(periodicrespiration)指呼吸时短暂性停止20秒以下,然后又恢复。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "周期性呼吸" }, { "start_idx": 6, "end_idx": 24, "type": "sym", "entity": "periodicrespiration" } ] }, { "text": "呼吸暂停指呼吸停止>20秒。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "呼吸暂停" } ] }, { "text": "主要因为随着肺发育长大,气道至外周肺泡距离增加,气流充盈肺泡及排出气体所需要时间相应延长,使呼吸周期延长,但也因为肺泡总表面积显著增加,满足了机体代谢需要。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肺" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "气道" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "外周肺泡" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肺泡" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "肺泡" } ] }, { "text": "小儿肺部病变时,如果出现气道通气障碍,或肺部炎症使有效气体交换面积和效率下降时,主要依靠加快呼吸频率来保持气体交换,以满足机体代谢需要。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "肺部病变" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "气道通气障碍" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "肺部炎症" } ] }, { "text": "2.肺通气量基本肺容积包括潮气量、吸气储备量(补吸气量)、呼气储备量(补呼气量)、残余气量。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肺" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肺" }, { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "潮气" }, { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "吸气储备量" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "补吸气量" }, { "start_idx": 29, "end_idx": 33, "type": "ite", "entity": "呼气储备量" }, { "start_idx": 35, "end_idx": 38, "type": "ite", "entity": "补呼气量" }, { "start_idx": 41, "end_idx": 44, "type": "ite", "entity": "残余气量" } ] }, { "text": "潮气量指一次呼吸时进入或排出肺部的气量。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "潮气量" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肺部" } ] }, { "text": "小儿潮气量一般以体重修正,为6~8ml/kg。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "潮气量" }, { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "体重" } ] }, { "text": "肺容量为基本肺容积中任两项或两项以上的联合气量。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "肺容量" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肺" } ] }, { "text": "其中深吸气量(inspiratorycapacity)为平静呼气末做最大吸气时所能吸入的气体量,相当于潮气量与补吸气量之和。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "深吸气量" }, { "start_idx": 7, "end_idx": 25, "type": "ite", "entity": "inspiratorycapacity" }, { "start_idx": 51, "end_idx": 53, "type": "ite", "entity": "潮气量" }, { "start_idx": 55, "end_idx": 58, "type": "ite", "entity": "补吸气量" } ] }, { "text": "功能残气量(functionalresidualcapacity,FRC)为平静呼气末尚存留于肺内的气量,相当于残气量和补呼气量之和。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "功能残气量" }, { "start_idx": 6, "end_idx": 31, "type": "ite", "entity": "functionalresidualcapacity" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "FRC" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "肺" }, { "start_idx": 56, "end_idx": 58, "type": "ite", "entity": "残气量" }, { "start_idx": 60, "end_idx": 63, "type": "ite", "entity": "补呼气量" } ] }, { "text": "新生儿出生早期肺液排出后,获得FRC约20~25ml/kg,至儿童期保持为25~30ml/kg水平,至成年可以达到30~35ml/kg。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肺液" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "FRC" } ] }, { "text": "FRC的生理意义为缓冲呼吸过程中肺泡气中氧和二氧化碳分压的过度变化,使肺泡气和动脉血的PaO2和PaCO2不会随呼吸而发生大幅度波动,以利于气体交换。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "FRC" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肺泡" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肺泡" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "动脉血" }, { "start_idx": 43, "end_idx": 57, "type": "ite", "entity": "PaO2" }, { "start_idx": 59, "end_idx": 74, "type": "ite", "entity": "PaCO2" } ] }, { "text": "在病理情况下,弥漫性肺泡损伤使FRC下降,哮喘、肺气肿、老年肺弹性回缩力减退时,FRC增高。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "弥漫性肺泡损伤" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "FRC" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "哮喘" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "肺气肿" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "肺弹性回缩力减退" }, { "start_idx": 40, "end_idx": 42, "type": "ite", "entity": "FRC" } ] }, { "text": "对于没有随意呼吸控制的小婴儿和幼儿,不能测定补吸气量和补呼气量,也不能直接测定出残余气量。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "补吸气量" }, { "start_idx": 27, "end_idx": 30, "type": "ite", "entity": "补呼气量" }, { "start_idx": 40, "end_idx": 43, "type": "ite", "entity": "残余气量" } ] }, { "text": "3.换气功能进入肺部的气体约1/3在大小气道管腔,不参与气体交换,为解剖无效腔;2/3达到呼吸性细支气管和肺泡,参与气体交换。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肺部" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "气道管腔" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "解剖无效腔" }, { "start_idx": 45, "end_idx": 51, "type": "bod", "entity": "呼吸性细支气管" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "肺泡" } ] }, { "text": "在由呼吸性细支气管、肺泡导管、肺泡囊和肺泡组成的呼吸性功能单位中,通过成千上万个微小肺泡,吸入气体的氧气弥散通过肺泡隔进入肺泡毛细血管并进入循环血液,血液中二氧化碳气体进入肺泡,随呼出气排出体外。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "呼吸性细支气管" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "肺泡导管" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肺泡囊" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肺泡" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "肺泡" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "肺泡隔" }, { "start_idx": 61, "end_idx": 66, "type": "bod", "entity": "肺泡毛细血管" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "血液" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "肺泡" } ] }, { "text": "动脉血液将氧气带到外周组织并释放,使组织获得氧供,并将组织代谢产生的二氧化碳带回到肺部,从而保证机体的氧和二氧化碳代谢平衡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "动脉血液" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "外周组织" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "肺部" } ] }, { "text": "在肺部气体交换保持合适的通气和血液灌流比例,在新生儿期,通气-灌流比接近1,儿童期与成人相似,为0.8。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "肺部" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血液" } ] }, { "text": "如果因肺外分流(动脉导管、卵圆孔未闭,房、室间隔缺损)肺部血流显著减少,或肺内动静脉分流,参与气体交换的毛细血管网减少,则使通气-灌流比例增大。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "动脉导管" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "卵圆孔未闭" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "房、室间隔缺损" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肺部" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "肺内动静脉" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "毛细血管" } ] }, { "text": "没有参加气体交换的肺泡部分成为生理无效腔。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肺泡" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "生理无效腔" } ] }, { "text": "二、胃肠测压法目前低顺应性的关注导管系统和腔内微型传感器导管系统已成为定型的测压技术设备。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "胃肠测压法" }, { "start_idx": 16, "end_idx": 19, "type": "equ", "entity": "导管系统" }, { "start_idx": 21, "end_idx": 31, "type": "equ", "entity": "腔内微型传感器导管系统" } ] }, { "text": "利用连续灌注导管测压系统工作原理,用微泵向导管内注水,水流速度恒定,导管末端测孔逸水时克服的阻力即为胃肠腔内压力。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "equ", "entity": "灌注导管测压系统" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "胃肠腔" } ] }, { "text": "常用PCPolygrafHR型胃肠动力监测系统,有4通道和8通道的。", "entities": [ { "start_idx": 2, "end_idx": 22, "type": "equ", "entity": "PCPolygrafHR型胃肠动力监测系统" } ] }, { "text": "(一)食管测压常用4通道测压导管,可测不同部位及不同方向的压力。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "食管测压" }, { "start_idx": 9, "end_idx": 15, "type": "equ", "entity": "4通道测压导管" } ] }, { "text": "液压毛细管灌注系统内的水流速度0.5cm/min。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "equ", "entity": "液压毛细管灌注系统" } ] }, { "text": "检查前禁食4~6小时,小婴儿适当用镇静剂,将测压导管经鼻腔或口插入,直至4个通道的测孔都到达胃内,采用定点牵拉法测定胃内压、上下食管括约肌压力、长度及松弛情况、食管体部蠕动情况等。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "镇静剂" }, { "start_idx": 22, "end_idx": 25, "type": "equ", "entity": "测压导管" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "鼻腔" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "口" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "胃" }, { "start_idx": 58, "end_idx": 60, "type": "ite", "entity": "胃内压" }, { "start_idx": 62, "end_idx": 70, "type": "ite", "entity": "上下食管括约肌压力" }, { "start_idx": 80, "end_idx": 87, "type": "ite", "entity": "食管体部蠕动情况" } ] }, { "text": "食管测压是研究和诊断食管动力障碍性疾病的基本方法,对食管裂孔疝可作出诊断,并可用于评价贲门失弛缓症、弥漫性食管痉挛的药物和手术治疗效果及胃底折叠术抗反流的疗效,筛选、评价作用于食管的药物,如促动力药等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "食管测压" }, { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "食管动力障碍性疾病" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "食管裂孔疝" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "贲门失弛缓症" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "弥漫性食管痉挛" }, { "start_idx": 68, "end_idx": 75, "type": "pro", "entity": "胃底折叠术抗反流" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "食管" }, { "start_idx": 95, "end_idx": 98, "type": "dru", "entity": "促动力药" } ] }, { "text": "(二)直肠肛门测压直肠肛门紧闭与舒张存在着一定的压力变化。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "直肠肛门测压" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "直肠肛门" } ] }, { "text": "肛门内外括约肌的压力变化与直肠内压力变化有密切关系,并且是连续性反射性活动。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "ite", "entity": "肛门内外括约肌的压力" }, { "start_idx": 13, "end_idx": 17, "type": "ite", "entity": "直肠内压力" } ] }, { "text": "1967年,SchnauferheLawson等先后报道用直肠肛门测压法发现了先天性巨结肠患儿直肠肛门反射消失的报告,使直肠肛门测压法首先在小儿外科得到广泛应用。", "entities": [ { "start_idx": 29, "end_idx": 35, "type": "pro", "entity": "直肠肛门测压法" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "先天性巨结肠" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "直肠肛门" }, { "start_idx": 60, "end_idx": 66, "type": "pro", "entity": "直肠肛门测压法" }, { "start_idx": 70, "end_idx": 73, "type": "dep", "entity": "小儿外科" } ] }, { "text": "作为一种研究直肠肛门生理、病理及诊断疾病和判定直肠肛门功能的方法,直肠肛门测压法得到了越来越多的应用。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "直肠肛门" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "直肠肛门" }, { "start_idx": 33, "end_idx": 39, "type": "pro", "entity": "直肠肛门测压法" } ] }, { "text": "测压导管带有气囊,在测定腔内压力的同时,可观察不同气囊内压时直肠和括约肌压力的变化及反射情况。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "equ", "entity": "测压导管" }, { "start_idx": 6, "end_idx": 7, "type": "equ", "entity": "气囊" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "直肠" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "括约肌" } ] }, { "text": "小儿常用4通道导管,每通道测孔相隔1cm。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "equ", "entity": "4通道导管" } ] }, { "text": "检查前排空大、小便,检查时取左侧卧位,将测压管插入直肠内,采用定点牵拉法测定压力,并向气囊内注气以观察松弛反射及直肠收缩反射,检测内外括约肌协调功能。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "equ", "entity": "测压管" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "直肠" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "定点牵拉法" }, { "start_idx": 43, "end_idx": 44, "type": "equ", "entity": "气囊" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "直肠" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "括约肌" } ] }, { "text": "检测指标:压力(包括直肠静息压,肛门内、外括约肌压力);反射(直肠收缩反射、内括约肌松弛反射、外括约肌松弛反射);直肠耐受性;括约肌长度。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "ite", "entity": "直肠静息压" }, { "start_idx": 16, "end_idx": 25, "type": "ite", "entity": "肛门内、外括约肌压力" }, { "start_idx": 31, "end_idx": 36, "type": "ite", "entity": "直肠收缩反射" }, { "start_idx": 38, "end_idx": 45, "type": "ite", "entity": "内括约肌松弛反射" }, { "start_idx": 47, "end_idx": 54, "type": "ite", "entity": "外括约肌松弛反射" }, { "start_idx": 57, "end_idx": 61, "type": "ite", "entity": "直肠耐受性" }, { "start_idx": 63, "end_idx": 67, "type": "ite", "entity": "括约肌长度" } ] }, { "text": "临床上用于先天性巨结肠的诊断,评估慢性便秘、特发性大便失禁患儿的直肠、肛门功能以及肛门部手术后的疗效评价。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "先天性巨结肠" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "慢性便秘" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "特发性大便失禁" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "直肠" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肛门" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "肛门" } ] }, { "text": "(三)其他胃内压测定以及Oddi括约肌压力测定等。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "胃内压测定" }, { "start_idx": 12, "end_idx": 22, "type": "pro", "entity": "Oddi括约肌压力测定" } ] }, { "text": "第六节青春期的生长发育青春(adolescence)期开始于生长突增,终止于骨骺完全愈合、躯体停止生长、性发育成熟。", "entities": [ { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "骨骺" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "躯体" } ] }, { "text": "在这个时期,生长突增,性腺、生殖器官及第二性征迅速发育,内分泌及心理发生明显的变化,它是人体生长发育的最后阶段。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "性腺" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "生殖器官" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "内分泌" } ] }, { "text": "(一)体格生长青春早期出现身高的突增,突增的幅度女孩为每年增加8~10cm,整个青春期可增加25cm,男孩每年增加9~12cm,整个青春期可增加28cm。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "身高" } ] }, { "text": "由于男孩的身高突增比女性晚2年,骨骼停止时间亦晚2年,且突增的幅度较大,因此在青春期结束时男性的平均身高比女性高10cm。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "身高" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "骨骼" }, { "start_idx": 50, "end_idx": 51, "type": "ite", "entity": "身高" } ] }, { "text": "生长加速从远端开始,最早是手和足增大,随后是臂和腿增长,最后才是躯干。", "entities": [ { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "手" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "足" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "臂" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "腿" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "躯干" } ] }, { "text": "四肢长度增长快于躯干,使坐高与身高之比缩小,当长骨的生长速度减慢时脊柱的生长相对较快,使坐高与身高的比例达到成人正常比例。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "四肢" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "躯干" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "身高" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "长骨" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "脊柱" }, { "start_idx": 47, "end_idx": 48, "type": "ite", "entity": "身高" } ] }, { "text": "体重的变化规律与身高相似,但生长突增不如身高显著,增长持续的时间较长,在青春期后仍可继续增长。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "体重" }, { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "身高" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "身高" } ] }, { "text": "青春期男、女儿童的各种身体成分总量都在增加,但各种成分的比例有所不同,男性的瘦体重(leanbodymass)增加迅速,尤其是骨骼肌的增加明显。", "entities": [ { "start_idx": 38, "end_idx": 40, "type": "ite", "entity": "瘦体重" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "骨骼肌" } ] }, { "text": "而女性的体脂量在整个青春期持续增长,尤以青春后期更为明显。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "体脂量" } ] }, { "text": "各种围长和宽度如胸围、大腿围、小腿围、臀围、骨盆宽都有增长,但有性别差异,男孩的肩宽、胸围增幅大,女孩则骨盆宽增加明显,最终形成男性身材高大、肌肉发达、上体宽的体格特征,而女性显示身材较矮、体脂丰满、下体宽的体型。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "胸围" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "大腿围" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "小腿围" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "臀围" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "骨盆宽" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "肩宽" }, { "start_idx": 43, "end_idx": 44, "type": "ite", "entity": "胸围" }, { "start_idx": 52, "end_idx": 54, "type": "ite", "entity": "骨盆宽" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "肌肉" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "上体" }, { "start_idx": 95, "end_idx": 96, "type": "ite", "entity": "体脂" } ] }, { "text": "(二)骨骼发育青春期各骨化中心相继钙化,并与骨干的骨骺端愈合。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "骨骼" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "骨干" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "骨骺端" } ] }, { "text": "长骨骨干与骨骺约15、16岁(女性)、17、18岁(男性)愈合,椎骨体与骨骺到20岁以后才能完全愈合。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "骨干" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "骨骺" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "椎骨体" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "骨骺" } ] }, { "text": "判断骨骼发育程度可应用骨骼年龄(骨龄boneage)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "骨骼" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "骨骼" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "骨龄" } ] }, { "text": "测定骨龄的理想部位为手腕部,通过骨骼X线摄片观察儿童手腕部骨骼钙化程度,并与骨龄标准比较,从而确定儿童的骨龄,反映儿童体格生长情况。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "骨龄" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "手腕部" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "骨骼" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "X线摄片" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "手腕部" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "骨骼" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "骨龄" }, { "start_idx": 52, "end_idx": 53, "type": "ite", "entity": "骨龄" } ] }, { "text": "1.性器官和性功能卵巢一般在8~10岁开始发育增快,重量增加,外形由纺锤形变为扁圆形,功能也逐渐完善,出现周期性排卵和分泌雌激素,月经初潮是女性青春期发育过程中的重要标志和评定指标。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "性器官" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "卵巢" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "雌激素" } ] }, { "text": "睾丸增大是男性青春期发育的第一个信号,睾丸开始增大的年龄最早为9.5岁,阴茎增大迟于睾丸增大的半年至1年后,睾丸的作用是产生精子和分泌雄激素,男性遗精的年龄平均为15、16岁。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "睾丸" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "睾丸" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "阴茎" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "睾丸" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "睾丸" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "精子" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "雄激素" } ] }, { "text": "2.第二性征发育乳房大约在8~13岁开始发育,阴毛在乳房开始发育后的6个月到1年出现,腋毛则在阴毛出现后的半年至1年后出现。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "乳房" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "阴毛" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "乳房" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "腋毛" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "阴毛" } ] }, { "text": "身高生长突增的开始是青春期最早的征象,多数女孩乳房增大和身高突增同时出现。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "乳房" } ] }, { "text": "男性的第二性征表现在阴毛、腋毛、胡须、变声、喉结等方面。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "阴毛" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "腋毛" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "胡须" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "变声" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "喉结" } ] }, { "text": "阴毛出现的年龄个体差异很大,在阴毛出现后1~2年出现腋毛,在腋毛出现后一年才出现胡须。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "阴毛" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "阴毛" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "腋毛" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "腋毛" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "胡须" } ] }, { "text": "喉节从12岁开始出现,13岁声音变粗,18岁时喉节和变声完成。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "喉节" } ] }, { "text": "表3-3青春期性发育顺序(四)功能发育青春期的功能发育常以心肺功能、肌肉力量及运动能力反映之。", "entities": [ { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肌肉" } ] }, { "text": "2.肌肉力量青春期时男性的握力(表示手及臂部肌肉力量)可增长25~35kg,女性增长15~20kg,年增长值男性4~10kg,女性2~5kg,男性握力值始终高于女性,随年龄增长,这种性别差异增大。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肌肉" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肌肉" } ] }, { "text": "3.运动能力青春期的运动能力有较明显的增强,肌肉活动中所表现出一定的力量、速度、灵敏及柔韧性。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肌肉" } ] }, { "text": "第十二章小儿呼吸衰竭第一节呼吸衰竭的病因及病理生理呼吸衰竭(respiratoryfailure)是指由于各种原因导致中枢和(或)外周性呼吸生理功能障碍,使动脉血氧分压(PaO2)<8kPa(60mmHg)和动脉二氧化碳分压(PaCO2)>6.67kPa(50mmHg),并存在呼吸困难症状的临床综合征。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 30, "end_idx": 47, "type": "dis", "entity": "respiratoryfailure" }, { "start_idx": 59, "end_idx": 75, "type": "dis", "entity": "中枢和(或)外周性呼吸生理功能障碍" }, { "start_idx": 78, "end_idx": 83, "type": "ite", "entity": "动脉血氧分压" }, { "start_idx": 115, "end_idx": 122, "type": "ite", "entity": "动脉二氧化碳分压" }, { "start_idx": 161, "end_idx": 164, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "小儿多见急性呼吸衰竭。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "急性呼吸衰竭" } ] }, { "text": "目前随着小儿危重病救治技术的完善和提高,尤其在急诊室和重症监护救治手段的应用,对于传统意义上的呼吸衰竭定义和认识有待进一步的发展。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "dep", "entity": "急诊室" }, { "start_idx": 27, "end_idx": 30, "type": "dep", "entity": "重症监护" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "一、病因和分类(一)根据年龄分类1.新生儿阶段一般指出生后28天内出现的呼吸系统或其他系统疾病导致的呼吸衰竭。", "entities": [ { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "多因窒息、缺氧、肺发育不成熟、吸入羊水胎粪、肺部或全身感染导致。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "窒息" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "缺氧" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肺" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肺部" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "全身" } ] }, { "text": "此外,先天性畸形和发育障碍导致上、下呼吸道梗阻,膈疝使肺部受压迫等,也可以导致呼吸衰竭。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "先天性畸形" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "发育障碍" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "上、下呼吸道梗阻" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "膈疝" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肺部" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "此阶段气道免疫系统发育尚不完善,容易感染细菌和病毒,导致呼吸衰竭的原因多为支气管肺炎、中枢感染等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "气道免疫系统" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "细菌" }, { "start_idx": 23, "end_idx": 24, "type": "mic", "entity": "病毒" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "支气管肺炎" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "中枢感染" } ] }, { "text": "3.儿童阶段多可因肺炎、先天性心脏病、哮喘持续状态、感染性疾病、肺外脏器功能衰竭等发展而来。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肺炎" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "哮喘" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "肺外脏器功能衰竭" } ] }, { "text": "此外,外伤、手术创伤、气道异物、溺水、中毒等也会严重影响到呼吸功能,导致急性呼吸衰竭。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "外伤" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "手术创伤" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "气道" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "中毒" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "急性呼吸衰竭" } ] }, { "text": "(二)根据中枢性和外周性病因的分类1.中枢性原发病对脑部的伤害、脑水肿或颅内高压影响呼吸中枢的正常功能,导致中枢呼吸运动神经元的冲动发放异常,而出现呼吸频率和节律异常,临床主要为通气功能异常。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "脑部" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "脑水肿" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "颅内高压" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "呼吸中枢" }, { "start_idx": 74, "end_idx": 80, "type": "ite", "entity": "呼吸频率和节律" }, { "start_idx": 89, "end_idx": 94, "type": "sym", "entity": "通气功能异常" } ] }, { "text": "如颅内感染、出血、头颅创伤,窒息和缺氧等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "颅内感染" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "出血" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "头颅创伤" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "窒息" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "缺氧" } ] }, { "text": "药物中毒、酸中毒、肝肾功能障碍也可以导致中枢性呼吸衰竭。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "药物中毒" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "酸中毒" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "肝肾功能障碍" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "中枢性呼吸衰竭" } ] }, { "text": "2.外周性原发于呼吸器官,如气道、肺、胸廓和呼吸肌病变,或继发于肺部及胸腔以外脏器系统病变的各种疾病。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "呼吸器官" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "气道" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "肺" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胸廓" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肺部" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "胸腔" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "脏器系统" } ] }, { "text": "(三)根据感染和非感染性病因的分类1.感染性疾病如细菌、病毒、真菌、原虫性肺炎并发呼吸衰竭,或脓毒症等全身性感染导致急性肺部炎症、损伤、水肿、出血等病变。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "mic", "entity": "细菌" }, { "start_idx": 28, "end_idx": 29, "type": "mic", "entity": "病毒" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "真菌" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "原虫性肺炎" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "脓毒症" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "全身" }, { "start_idx": 58, "end_idx": 63, "type": "dis", "entity": "急性肺部炎症" }, { "start_idx": 65, "end_idx": 66, "type": "dis", "entity": "损伤" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "水肿" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "出血" } ] }, { "text": "中枢感染也是导致呼吸衰竭的重要原因。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "中枢感染" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "2.非感染性如手术、创伤、吸入、淹溺、中毒等导致的中枢性和外周性呼吸衰竭。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "手术" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "创伤" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "淹溺" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "中毒" }, { "start_idx": 25, "end_idx": 35, "type": "dis", "entity": "中枢性和外周性呼吸衰竭" } ] }, { "text": "(四)直接根据疾病种类的分类直接根据原发疾病所出现的呼吸衰竭加以分类区别,如肺炎合并呼吸衰竭,脑炎、脑膜炎合并呼吸衰竭,或者多脏器功能衰竭合并呼吸衰竭。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "肺炎" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "脑炎" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "多脏器功能衰竭" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "(五)根据病理生理特点的分类1.急性呼吸衰竭多为急性发作并出现持续低氧血症,依赖紧急复苏抢救。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "急性呼吸衰竭" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "低氧血症" }, { "start_idx": 40, "end_idx": 45, "type": "pro", "entity": "紧急复苏抢救" } ] }, { "text": "2.慢性呼吸衰竭多表现为肺部基础疾病进行性损害,导致失代偿,出现高碳酸血症和酸中毒。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "慢性呼吸衰竭" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肺部" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "酸中毒" } ] }, { "text": "偶尔也可见于肺外疾病,如Duchenne型肌营养不良时进行性膈肌无力导致的气体交换不足。", "entities": [ { "start_idx": 12, "end_idx": 21, "type": "bod", "entity": "Duchenne型肌" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "营养不良" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "进行性膈肌无力" } ] }, { "text": "3.血氧和二氧化碳水平根据血气分析临床可诊断呼吸衰竭为Ⅰ型(低氧血症型)和Ⅱ型(低氧血症伴高碳酸血症)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "血氧" }, { "start_idx": 5, "end_idx": 8, "type": "ite", "entity": "二氧化碳" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "血气分析" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "低氧血症" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "低氧血症" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "高碳酸血症" } ] }, { "text": "第四节转移性骨肿瘤原发于身体其他部位的肿瘤,主要是恶性肿瘤,通过各种途径转移至骨骼并在骨内继续生长,形成子肿瘤。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "转移性骨肿瘤" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "肿瘤" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "骨骼" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "骨" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "子肿瘤" } ] }, { "text": "在骨肿瘤中占有一定的比例。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "骨肿瘤" } ] }, { "text": "【转移】转移性骨肿瘤的产生主要通过淋巴或血液两条途径,原发肿瘤可以来自全身各处。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "转移性骨肿瘤" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "淋巴" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "血液" } ] }, { "text": "【临床表现】转移到肢体骨骼的肿瘤主要以局部肿块最先发现,而躯干部的转移性骨肿瘤往往是疼痛为首发表现。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "肢体骨骼" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肿瘤" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "局部肿块" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "躯干部" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "转移性骨肿瘤" } ] }, { "text": "位于深部的骨骼转移,早期肿块不易发现,只反映出局部的疼痛,靠近关节附近的肿瘤可以引起关节功能的障碍,肿瘤增大在重要的神经附近时可以有或多或少的压迫症状,产生麻木、肌肉无力或萎缩,不少病例的诊断是在病理骨折发生时才发现了骨骼的病变,要特别引起重视。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "早期肿块不易发现" }, { "start_idx": 19, "end_idx": 27, "type": "sym", "entity": "只反映出局部的疼痛" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "关节" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "肿瘤" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "肿瘤" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "神经" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "压迫症状" }, { "start_idx": 76, "end_idx": 79, "type": "sym", "entity": "产生麻木" }, { "start_idx": 81, "end_idx": 87, "type": "sym", "entity": "肌肉无力或萎缩" }, { "start_idx": 98, "end_idx": 101, "type": "dis", "entity": "病理骨折" }, { "start_idx": 109, "end_idx": 110, "type": "bod", "entity": "骨骼" } ] }, { "text": "【诊断】(一)X线表现转移性骨肿瘤的X线表现多数为肿瘤发生的骨骼产生各种骨骼破坏性改变,病变多局限在骨骼内,边缘不清,有时与原发性骨肿瘤不易鉴别。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "X线" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "转移性骨肿瘤" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "X线" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "肿瘤" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "骨骼" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "骨骼" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "骨骼" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "原发性骨肿瘤" } ] }, { "text": "转移性骨肿瘤可单发或多发,单发性系发生于某一骨内的转移,局限于一处产生骨质破坏,使邻近骨皮质扩大膨胀,X线上可以为同一的骨质破坏或者形成大小不等的囊性腔隙也可以是广泛的骨质破坏骨内,也可以表现为两种形式,一种是广泛地散在于多数骨内,一种是连续侵犯相邻近的几个骨,如侵犯同侧的髂骨及股骨近端,肩胛骨及肱骨近端。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "转移性骨肿瘤" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "骨内" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "骨质" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "骨皮质" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "X线" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "骨质" }, { "start_idx": 51, "end_idx": 63, "type": "sym", "entity": "X线上可以为同一的骨质破坏" }, { "start_idx": 73, "end_idx": 76, "type": "bod", "entity": "囊性腔隙" }, { "start_idx": 64, "end_idx": 76, "type": "sym", "entity": "或者形成大小不等的囊性腔隙" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "骨质" }, { "start_idx": 77, "end_idx": 87, "type": "sym", "entity": "也可以是广泛的骨质破坏" }, { "start_idx": 88, "end_idx": 88, "type": "bod", "entity": "骨" }, { "start_idx": 113, "end_idx": 113, "type": "bod", "entity": "骨" }, { "start_idx": 129, "end_idx": 129, "type": "bod", "entity": "骨" }, { "start_idx": 137, "end_idx": 138, "type": "bod", "entity": "髂骨" }, { "start_idx": 140, "end_idx": 141, "type": "bod", "entity": "股骨" }, { "start_idx": 145, "end_idx": 147, "type": "bod", "entity": "肩胛骨" }, { "start_idx": 149, "end_idx": 150, "type": "bod", "entity": "肱骨" } ] }, { "text": "(二)实验室检查转移性骨肿瘤的诊断假如是在原发肿瘤的诊断之后,相对容易。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "转移性骨肿瘤" } ] }, { "text": "根据儿童常见肿瘤的发病次序,选择性地进行必要的实验室检查,可以帮助作出正确的诊断。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肿瘤" } ] }, { "text": "(三)活体组织检查对于骨骼的肿瘤性病变,活体组织检查是明确诊断最直接的方法,应该尽早进行。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "活体组织检查" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "骨骼" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肿瘤性病变" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "活体组织检查" } ] }, { "text": "单一的病灶,以直接挖取病灶组织为主要方法,多发性病灶则应该考虑从相对较易取得病变组织的部位着手。", "entities": [ { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "病变组织" } ] }, { "text": "【治疗】转移性骨肿瘤的治疗在诊断明确之后,应及时采用综合治疗的方法,原发性肿瘤病变的治疗是整个治疗中的主要环节。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "转移性骨肿瘤" } ] }, { "text": "第九章肾血管性高血压儿童高血压中65%~80%为继发性高血压。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "肾血管性高血压" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "儿童高血压" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "继发性高血压" } ] }, { "text": "肾血管性高血压(renovascularhypertension,RVH)即为其中之一。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "肾血管性高血压" }, { "start_idx": 8, "end_idx": 31, "type": "dis", "entity": "renovascularhypertension" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "RVH" } ] }, { "text": "肾血管性高血压,主要指肾动脉狭窄,系指单侧或双侧动脉及/或其分支病变使肾脏缺血引起的高血压。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "肾血管性高血压" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "肾动脉" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "肾动脉狭窄" }, { "start_idx": 19, "end_idx": 31, "type": "bod", "entity": "单侧或双侧动脉及/或其分支" }, { "start_idx": 19, "end_idx": 33, "type": "sym", "entity": "单侧或双侧动脉及/或其分支病变" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肾脏" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "肾脏缺血" }, { "start_idx": 42, "end_idx": 44, "type": "sym", "entity": "高血压" } ] }, { "text": "虽然在高血压中的发病率<5%。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "高血压" } ] }, { "text": "但是可根治的小儿高血压的病因之一,由于诊断方法、治疗技术、血管显微外科和肾移植的进展,本病的早期诊断和治疗后的效果有很大改观。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "小儿高血压" }, { "start_idx": 29, "end_idx": 34, "type": "dep", "entity": "血管显微外科" }, { "start_idx": 36, "end_idx": 38, "type": "pro", "entity": "肾移植" } ] }, { "text": "【病因】(一)先天性肾动脉纤维肌肉发育不良是国外报道的小儿和青少年RVH的主要病因,病变多发生于肾动脉的中段或远段。", "entities": [ { "start_idx": 7, "end_idx": 20, "type": "dis", "entity": "先天性肾动脉纤维肌肉发育不良" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "RVH" }, { "start_idx": 48, "end_idx": 56, "type": "bod", "entity": "肾动脉的中段或远段" } ] }, { "text": "常累及其分支,常见有几种:①内膜纤维增生:主要是肾动脉主干的狭窄和变形,血管造影显示中段有局灶性狭窄;②中层纤维增生:呈间断性破坏和增厚,多蔓延至肾动脉中远段,血管造影呈念珠状阴影;③纤维肌肉增生:肾动脉壁呈同心性增厚,肾动脉造影示肾动脉及其分支有光滑的狭窄;④外膜下纤维增生:致肾动脉严重狭窄,动脉造影示不规则狭窄及丰富的侧支循环。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "内膜纤维" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "内膜纤维增生" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "肾动脉主干" }, { "start_idx": 24, "end_idx": 34, "type": "sym", "entity": "肾动脉主干的狭窄和变形" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "血管造影" }, { "start_idx": 42, "end_idx": 49, "type": "sym", "entity": "中段有局灶性狭窄" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "中层纤维" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "中层纤维增生" }, { "start_idx": 60, "end_idx": 67, "type": "sym", "entity": "间断性破坏和增厚" }, { "start_idx": 73, "end_idx": 78, "type": "bod", "entity": "肾动脉中远段" }, { "start_idx": 69, "end_idx": 78, "type": "sym", "entity": "多蔓延至肾动脉中远段" }, { "start_idx": 80, "end_idx": 83, "type": "pro", "entity": "血管造影" }, { "start_idx": 85, "end_idx": 89, "type": "sym", "entity": "念珠状阴影" }, { "start_idx": 92, "end_idx": 95, "type": "bod", "entity": "纤维肌肉" }, { "start_idx": 92, "end_idx": 97, "type": "sym", "entity": "纤维肌肉增生" }, { "start_idx": 99, "end_idx": 102, "type": "bod", "entity": "肾动脉壁" }, { "start_idx": 99, "end_idx": 108, "type": "sym", "entity": "肾动脉壁呈同心性增厚" }, { "start_idx": 110, "end_idx": 114, "type": "pro", "entity": "肾动脉造影" }, { "start_idx": 116, "end_idx": 122, "type": "bod", "entity": "肾动脉及其分支" }, { "start_idx": 116, "end_idx": 128, "type": "sym", "entity": "肾动脉及其分支有光滑的狭窄" }, { "start_idx": 131, "end_idx": 132, "type": "bod", "entity": "外膜" }, { "start_idx": 131, "end_idx": 137, "type": "sym", "entity": "外膜下纤维增生" }, { "start_idx": 140, "end_idx": 142, "type": "bod", "entity": "肾动脉" }, { "start_idx": 140, "end_idx": 146, "type": "sym", "entity": "肾动脉严重狭窄" }, { "start_idx": 148, "end_idx": 151, "type": "pro", "entity": "动脉造影" }, { "start_idx": 153, "end_idx": 165, "type": "sym", "entity": "不规则狭窄及丰富的侧支循环" } ] }, { "text": "(二)多发性大动脉炎一种非特异性慢性血管炎症性疾病,是我国成人和小儿发生RVH的主要病因。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "多发性大动脉炎" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "非特异性慢性血管炎症" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "RVH" } ] }, { "text": "基本病变是动脉中层的弹力纤维组织增生变性和不同程度的小圆形细胞浸润,最终导致血管壁增厚,疤痕形成,血管壁弹性消失,管腔狭窄或动脉瘤样膨出。", "entities": [ { "start_idx": 5, "end_idx": 15, "type": "bod", "entity": "动脉中层的弹力纤维组织" }, { "start_idx": 5, "end_idx": 19, "type": "sym", "entity": "动脉中层的弹力纤维组织增生变性" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "小圆形细胞" }, { "start_idx": 21, "end_idx": 32, "type": "sym", "entity": "不同程度的小圆形细胞浸润" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "血管壁" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "血管壁增厚" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "疤痕形成" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "血管壁" }, { "start_idx": 49, "end_idx": 55, "type": "sym", "entity": "血管壁弹性消失" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "管腔" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "管腔狭窄" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "动脉" }, { "start_idx": 62, "end_idx": 67, "type": "sym", "entity": "动脉瘤样膨出" } ] }, { "text": "病变常位于肾动脉于腹主动脉的起始部狭窄,引起高血压。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肾动脉" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "腹主动脉" }, { "start_idx": 5, "end_idx": 18, "type": "sym", "entity": "肾动脉于腹主动脉的起始部狭窄" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "高血压" } ] }, { "text": "其他血管病变如溶血尿毒综合征、结节性多动脉炎、Ehlers-Danlos综合征及川崎病等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "血管病变" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "溶血尿毒综合征" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "结节性多动脉炎" }, { "start_idx": 23, "end_idx": 38, "type": "dis", "entity": "Ehlers-Danlos综合征" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "川崎病" } ] }, { "text": "(三)其他肾动脉血栓形成或栓塞,见于外伤或新生儿时期有脐静脉插管史者,肾动脉静脉瘘,肾动脉瘤,移植后肾动脉狭窄及先天性肾动脉异常(肾动脉均匀细小扭曲或狭窄),肾发育不良和成神经纤维瘤病的肾动脉受累,以及其他肾肿瘤和肾囊肿使肾动脉受纤维索带及动脉旁淋巴结压迫等。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肾动脉" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "肾动脉血栓" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "栓塞" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "脐静脉插管" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "肾动脉" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "肾动脉静脉瘘" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "肾动脉瘤" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "肾动脉" }, { "start_idx": 50, "end_idx": 54, "type": "sym", "entity": "肾动脉狭窄" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "肾动脉" }, { "start_idx": 56, "end_idx": 63, "type": "sym", "entity": "先天性肾动脉异常" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "肾动脉" }, { "start_idx": 65, "end_idx": 76, "type": "sym", "entity": "肾动脉均匀细小扭曲或狭窄" }, { "start_idx": 79, "end_idx": 79, "type": "bod", "entity": "肾" }, { "start_idx": 79, "end_idx": 83, "type": "sym", "entity": "肾发育不良" }, { "start_idx": 86, "end_idx": 91, "type": "dis", "entity": "神经纤维瘤病" }, { "start_idx": 93, "end_idx": 95, "type": "bod", "entity": "肾动脉" }, { "start_idx": 93, "end_idx": 97, "type": "sym", "entity": "肾动脉受累" }, { "start_idx": 103, "end_idx": 105, "type": "dis", "entity": "肾肿瘤" }, { "start_idx": 107, "end_idx": 109, "type": "dis", "entity": "肾囊肿" }, { "start_idx": 111, "end_idx": 113, "type": "bod", "entity": "肾动脉" }, { "start_idx": 115, "end_idx": 118, "type": "bod", "entity": "纤维索带" }, { "start_idx": 120, "end_idx": 125, "type": "bod", "entity": "动脉旁淋巴结" }, { "start_idx": 111, "end_idx": 127, "type": "sym", "entity": "肾动脉受纤维索带及动脉旁淋巴结压迫" } ] }, { "text": "也有根据病变所在的部位分为主要侵犯肾门的肾动脉疾病、肾内肾动脉疾病和肾动脉外的病变。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肾门" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "侵犯肾门" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "肾动脉疾病" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "肾内肾动脉疾病" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "肾动脉外的病变" } ] }, { "text": "【发病机制】在肾血管性高血压中肾素-血管紧张素-醛固酮系统(RAAS)起主要作用。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "肾血管性高血压" }, { "start_idx": 15, "end_idx": 28, "type": "bod", "entity": "肾素-血管紧张素-醛固酮系统" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "RAAS" } ] }, { "text": "第一种双肾单夹,类似于单侧RVH。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "单侧RVH" } ] }, { "text": "系钳夹肾动脉的一侧使钳夹侧肾动脉血流量减少。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "系钳夹肾动脉的一侧" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "钳夹侧肾动脉" }, { "start_idx": 10, "end_idx": 20, "type": "sym", "entity": "钳夹侧肾动脉血流量减少" } ] }, { "text": "通过刺激压力和化学感受器致密斑,使肾素分泌增多。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肾素" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "肾素分泌增多" } ] }, { "text": "血管紧张素Ⅱ(ATⅡ)形成增多后,通过①直接使全身小动脉收缩;②刺激醛固酮释放,致水、钠潴留;③ATⅡ能刺激交感神经,使其活力增强,还可刺激肾上腺髓质合成并释放去甲肾上腺素增多,引起高血压。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "血管紧张素Ⅱ" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "ATⅡ" }, { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "血管紧张素Ⅱ(ATⅡ)形成增多" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "全身小动脉" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "全身小动脉收缩" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "醛固酮" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "水、钠潴留" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "ATⅡ" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "交感神经" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "肾上腺髓质" }, { "start_idx": 80, "end_idx": 85, "type": "bod", "entity": "去甲肾上腺素" }, { "start_idx": 78, "end_idx": 87, "type": "sym", "entity": "释放去甲肾上腺素增多" }, { "start_idx": 91, "end_idx": 93, "type": "dis", "entity": "高血压" } ] }, { "text": "但此作用机制可通过对侧肾压力性排尿作用部分代偿,最终不出现钠潴留,其结果是:①缺血侧肾素分泌增多;②对侧正常肾由于血压升高及钠潴留的负反馈作用,使肾素分泌抑制;③缺血肾血流减少;④ATⅡ诱发血管收缩导致高血压。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "钠潴留" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "缺血侧肾素" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "缺血侧肾素分泌增多" }, { "start_idx": 57, "end_idx": 58, "type": "ite", "entity": "血压" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "血压升高" }, { "start_idx": 62, "end_idx": 64, "type": "sym", "entity": "钠潴留" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "肾素" }, { "start_idx": 73, "end_idx": 78, "type": "sym", "entity": "肾素分泌抑制" }, { "start_idx": 81, "end_idx": 87, "type": "sym", "entity": "缺血肾血流减少" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "ATⅡ" }, { "start_idx": 95, "end_idx": 96, "type": "bod", "entity": "血管" }, { "start_idx": 95, "end_idx": 98, "type": "sym", "entity": "血管收缩" }, { "start_idx": 101, "end_idx": 103, "type": "dis", "entity": "高血压" } ] }, { "text": "ATⅡ受体拮抗剂或血管紧张素转移抑制剂(ACEI)可使ATⅡ作用减弱,血压下降,但抑制了对侧肾素的反馈抑制,肾素及ATⅡ反而增加。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dru", "entity": "ATⅡ受体拮抗剂" }, { "start_idx": 9, "end_idx": 18, "type": "dru", "entity": "血管紧张素转移抑制剂" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "ACEI" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "ATⅡ" }, { "start_idx": 35, "end_idx": 36, "type": "ite", "entity": "血压" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "血压下降" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "对侧肾素" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "肾素" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "ATⅡ" } ] }, { "text": "为钳夹一例肾动脉,而对侧肾被切除,孤立肾与此种模型较为一致。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "bod", "entity": "钳夹一例肾动脉" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肾" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "肾" } ] }, { "text": "这样,压力性利尿排钠作用不再发生,导致钠潴留。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "压力性利尿排钠" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "钠潴留" } ] }, { "text": "同时反馈抑制了肾素分泌,外周血浆肾素水平在急性期后正常或降低。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肾素" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "外周血浆肾素" }, { "start_idx": 12, "end_idx": 29, "type": "sym", "entity": "外周血浆肾素水平在急性期后正常或降低" } ] }, { "text": "单用ACEI不能防止高血压的发生,若同时利尿排钠,可使血压下降。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "ACEI" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "高血压" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "利尿排钠" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "血压" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "血压下降" } ] }, { "text": "此种RVH可以是肾素依赖型,也可以是容量依赖型。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "RVH" } ] }, { "text": "双侧肾动脉狭窄是否与此一致尚有争论。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "双侧肾动脉" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "双侧肾动脉狭窄" } ] }, { "text": "此外激肽释放酶-激肽-前列腺素系统在RVH的发病机制中也有一定作用,激肽由激肽释放酶激活后,促进前列腺素合成。", "entities": [ { "start_idx": 2, "end_idx": 16, "type": "bod", "entity": "激肽释放酶-激肽-前列腺素系统" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "RVH" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "激肽" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "激肽释放酶" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "前列腺素" } ] }, { "text": "两者可使全身小动脉扩张,外周血管阻力降低,肾血管扩张,肾血流量增加,促进了水钠排出。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "全身小动脉" }, { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "全身小动脉扩张" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "外周血管" }, { "start_idx": 12, "end_idx": 19, "type": "sym", "entity": "外周血管阻力降低" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肾血管" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "肾血管扩张" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "肾血流量增加" } ] }, { "text": "在Glordblatt动物模型中,尿中激肽释放酶活力降低,以上因素又参与了高血压的发生。", "entities": [ { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "尿" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "激肽释放酶" }, { "start_idx": 17, "end_idx": 27, "type": "sym", "entity": "尿中激肽释放酶活力降低" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "高血压" } ] }, { "text": "RVH的发生与肾动脉狭窄发生的速度和时期有一定关系,肾动脉发生栓塞的数分钟内可出现高血压,即急性期,此时多为肾素依赖性高血压,如应用ACEI可使血压迅速有效地下降,数日或数周后进入过渡期,此期的血浆肾素和血管紧张素仍维持较高水平,但钠、水潴留已起作用,ACEI的应用仍可使血压下降,但速度减慢。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "RVH" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肾动脉" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "肾动脉狭窄" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "肾动脉" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "肾动脉发生栓塞" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "高血压" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "肾素依赖性高血压" }, { "start_idx": 66, "end_idx": 69, "type": "dru", "entity": "ACEI" }, { "start_idx": 72, "end_idx": 73, "type": "ite", "entity": "血压" }, { "start_idx": 72, "end_idx": 80, "type": "sym", "entity": "血压迅速有效地下降" }, { "start_idx": 97, "end_idx": 100, "type": "bod", "entity": "血浆肾素" }, { "start_idx": 102, "end_idx": 106, "type": "bod", "entity": "血管紧张素" }, { "start_idx": 97, "end_idx": 113, "type": "sym", "entity": "血浆肾素和血管紧张素仍维持较高水平" }, { "start_idx": 116, "end_idx": 120, "type": "sym", "entity": "钠、水潴留" }, { "start_idx": 126, "end_idx": 129, "type": "dru", "entity": "ACEI" }, { "start_idx": 136, "end_idx": 137, "type": "ite", "entity": "血压" }, { "start_idx": 136, "end_idx": 139, "type": "sym", "entity": "血压下降" } ] }, { "text": "最后是慢性期,水、钠潴留和血容量扩张对肾素的分泌起了抑制作用。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "水、钠潴留" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "血容量扩张" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肾素" } ] }, { "text": "【临床表现】(一)症状肾血管性高血压可发生在任何年龄,已有许多婴儿病例报告,最小者仅为7~10天。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "肾血管性高血压" } ] }, { "text": "小婴儿可有呕吐、发育营养差、充血性心力衰竭及急性肾衰竭等表现。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "呕吐" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "发育营养差" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "大多数患儿是由于严重高血压已存在相当时间,诊断时多已出现心、脑、肾等靶器官受累的症状。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "高血压" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "心" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "脑" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "肾" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "靶器官" }, { "start_idx": 28, "end_idx": 38, "type": "sym", "entity": "心、脑、肾等靶器官受累" } ] }, { "text": "病因为大动脉炎患者,尚可伴有低热、乏力和关节痛等症状。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "大动脉炎" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "低热" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "乏力" } ] }, { "text": "(二)体征1.高血压几乎一半的患儿在常规体检时发现高血压。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "高血压" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "高血压" } ] }, { "text": "小儿收缩压或舒张压超过该年龄及性别组的第95百分位数值(P95),相当于超过同年龄、同性别组平均值2个标准差为高血压;介于P95~P99者,为有意义的高血压,超过P99者为严重高血压,RVH患者均为严重高血压。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "收缩压" }, { "start_idx": 6, "end_idx": 8, "type": "ite", "entity": "舒张压" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "高血压" }, { "start_idx": 75, "end_idx": 77, "type": "dis", "entity": "高血压" }, { "start_idx": 88, "end_idx": 90, "type": "dis", "entity": "高血压" }, { "start_idx": 92, "end_idx": 94, "type": "dis", "entity": "RVH" }, { "start_idx": 101, "end_idx": 103, "type": "dis", "entity": "高血压" } ] }, { "text": "眼底检查可呈现不同程度高血压眼底改变。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "眼底检查" } ] }, { "text": "Ⅰ度正常眼底;Ⅱ度有局灶性小动脉痉挛;Ⅲ度有渗出伴或不伴有出血;Ⅳ度视乳头水肿。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "正常眼底" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "小动脉" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "局灶性小动脉痉挛" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "乳头" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "乳头水肿" } ] }, { "text": "2.血管杂音约有1/3~2/3患儿(多为大动脉炎患者)在中上腹部和/或腰背肋脊角处可闻及血管杂音,空腹时更易听到呈收缩期和舒张期连续性杂音,若听诊器从中上腹向旁平行移动时杂音增强则更有临床意义。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血管" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "血管杂音" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "大动脉炎" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "中上腹部" }, { "start_idx": 35, "end_idx": 40, "type": "bod", "entity": "腰背肋脊角处" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "血管" }, { "start_idx": 28, "end_idx": 47, "type": "sym", "entity": "中上腹部和/或腰背肋脊角处可闻及血管杂音" }, { "start_idx": 57, "end_idx": 68, "type": "sym", "entity": "收缩期和舒张期连续性杂音" }, { "start_idx": 71, "end_idx": 73, "type": "equ", "entity": "听诊器" }, { "start_idx": 75, "end_idx": 88, "type": "sym", "entity": "中上腹向旁平行移动时杂音增强" } ] }, { "text": "此外尚需注意大动脉炎所致的缺血症状,若累及无名动脉,可出现桡、肱动脉搏动减弱或消失;若累及髂动脉,可致跛行、手足凉,股动脉及足背动脉搏动减弱或消失。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "大动脉炎" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "缺血症状" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "无名动脉" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "累及无名动脉" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "桡、肱动脉" }, { "start_idx": 29, "end_idx": 40, "type": "sym", "entity": "桡、肱动脉搏动减弱或消失" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "髂动脉" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "累及髂动脉" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "跛行" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "手足" }, { "start_idx": 54, "end_idx": 56, "type": "sym", "entity": "手足凉" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "股动脉" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "足背动脉" }, { "start_idx": 58, "end_idx": 72, "type": "sym", "entity": "股动脉及足背动脉搏动减弱或消失" } ] }, { "text": "(三)实验室检查血常规、尿常规及尿细菌学检查,血尿素、肌酐、钾、钠、钙、氯化物及血气分析应列为常规检查。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "血常规" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "尿常规" }, { "start_idx": 16, "end_idx": 21, "type": "ite", "entity": "尿细菌学检查" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "血尿素" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "肌酐" }, { "start_idx": 30, "end_idx": 30, "type": "ite", "entity": "钾" }, { "start_idx": 32, "end_idx": 32, "type": "ite", "entity": "钠" }, { "start_idx": 34, "end_idx": 34, "type": "ite", "entity": "钙" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "氯化物" }, { "start_idx": 40, "end_idx": 43, "type": "ite", "entity": "血气分析" } ] }, { "text": "当严重高血压有继发性肾损害时可出现蛋白尿、血尿素和肌酐升高。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "高血压" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "继发性肾损害" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "血尿素" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "肌酐升高" } ] }, { "text": "心电图多呈左心室高电压或左心室肥大。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "心电图" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "左心室" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "左心室高电压" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "左心室" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "左心室肥大" } ] }, { "text": "【诊断】对高血压患儿需进一步检查血清内生肌酐清除率,钾、钠、氯化物及血气分析,血、尿醛固酮、尿儿茶酚胺及其代谢产物和香草苦杏仁酸(VMA)测定,以初步除外肾实质性高血压以及内分泌系、神经系、心血管系等疾病所致者,如:主动脉缩窄、原发性醛固酮增多症和嗜铬细胞瘤等。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "高血压" }, { "start_idx": 16, "end_idx": 24, "type": "ite", "entity": "血清内生肌酐清除率" }, { "start_idx": 26, "end_idx": 26, "type": "ite", "entity": "钾" }, { "start_idx": 28, "end_idx": 28, "type": "ite", "entity": "钠" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "氯化物" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "血气分析" }, { "start_idx": 39, "end_idx": 39, "type": "ite", "entity": "血" }, { "start_idx": 41, "end_idx": 44, "type": "ite", "entity": "尿醛固酮" }, { "start_idx": 46, "end_idx": 50, "type": "ite", "entity": "尿儿茶酚胺" }, { "start_idx": 58, "end_idx": 63, "type": "ite", "entity": "香草苦杏仁酸" }, { "start_idx": 65, "end_idx": 67, "type": "ite", "entity": "VMA" }, { "start_idx": 77, "end_idx": 83, "type": "dis", "entity": "肾实质性高血压" }, { "start_idx": 86, "end_idx": 101, "type": "dis", "entity": "内分泌系、神经系、心血管系等疾病" }, { "start_idx": 108, "end_idx": 112, "type": "dru", "entity": "主动脉缩窄" }, { "start_idx": 114, "end_idx": 122, "type": "dis", "entity": "原发性醛固酮增多症" }, { "start_idx": 124, "end_idx": 128, "type": "dis", "entity": "嗜铬细胞瘤" } ] }, { "text": "拟诊为RVH者,进行以下检查以明确有无肾动脉狭窄存在,并了解肾动脉狭窄的部位、病变性质和程度。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "RVH" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "肾动脉" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "肾动脉狭窄" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "肾动脉" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "肾动脉狭窄" } ] }, { "text": "病理资料表明,当肾动脉管腔截面积减少>50%~80%以上才有可能发生RVH。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "肾动脉管腔" }, { "start_idx": 8, "end_idx": 25, "type": "sym", "entity": "肾动脉管腔截面积减少>50%~80%" } ] }, { "text": "(一)筛选检查1.快速连续静脉肾盂造影(IVP)在注射造影剂后1、2、3、5、10、15分钟摄片,了解双肾大小、肾脏显影及排泄情况,阳性标准是:①缺血侧肾脏长径缩短1~1.5cm以上(正常小儿左肾稍大于右肾0.8cm);②患肾肾盂肾盏显影延迟,不显影和/或显影浓度降低;③后期造影剂排泄延迟。", "entities": [ { "start_idx": 9, "end_idx": 18, "type": "pro", "entity": "快速连续静脉肾盂造影" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "IVP" }, { "start_idx": 73, "end_idx": 79, "type": "ite", "entity": "缺血侧肾脏长径" }, { "start_idx": 73, "end_idx": 90, "type": "sym", "entity": "缺血侧肾脏长径缩短1~1.5cm以上" }, { "start_idx": 113, "end_idx": 120, "type": "sym", "entity": "肾盂肾盏显影延迟" }, { "start_idx": 136, "end_idx": 144, "type": "sym", "entity": "后期造影剂排泄延迟" } ] }, { "text": "此法在小儿的符合率为42%~65%,假阴性者多为双肾动脉狭窄或肾动脉分支狭窄。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "双肾动脉" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "双肾动脉狭窄" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "肾动脉分支" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "肾动脉分支狭窄" } ] }, { "text": "检查时还可静脉注射利尿剂,可使健侧肾盂造影剂迅速“洗脱”,患肾由于肾小球滤过率少,造影剂排出缓慢,从而扩大了两肾排泄造影剂的差别,有利于提高RVH的诊断。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "静脉注射" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "利尿剂" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "肾小球" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "肾小球滤过率少" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "造影剂排出缓慢" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "RVH" } ] }, { "text": "2.彩色多普勒超声检查可通过二维超声图像了解双肾大小有无差异,如一侧肾动脉狭窄,患肾比健侧明显缩小。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "彩色多普勒超声检查" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "一侧肾动脉" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "一侧肾动脉狭窄" }, { "start_idx": 40, "end_idx": 48, "type": "sym", "entity": "患肾比健侧明显缩小" } ] }, { "text": "又可通过多普勒超声检查探查双肾动脉直径、血流量及流速,了解有无肾动脉主干狭窄,为一种快速、无创、重复性好的筛选检查。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "pro", "entity": "多普勒超声检查探查" }, { "start_idx": 13, "end_idx": 18, "type": "ite", "entity": "双肾动脉直径" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "血流量" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "流速" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "肾动脉主干" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "肾动脉主干狭窄" } ] }, { "text": "3.放射性核素检查可初步了解分侧肾的血流灌注、分泌和排泄功能。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "放射性核素检查" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "分侧肾" } ] }, { "text": "近年来应用99m锝二巯基丁二酸(99mTc-DTPA)肾动态显像,可更全面地反映两侧肾的大小、肾灌注高峰出现时间、肾功能及两肾间差异程度,还可做ACEI抑制试验以增强健肾和患肾对示踪剂在双肾的灌注、分泌和排泄的差异程度,提高了该检查的敏感性和特异性。", "entities": [ { "start_idx": 5, "end_idx": 53, "type": "pro", "entity": "99m锝二巯基丁二酸(99mTc-DTPA)肾动态显像" }, { "start_idx": 62, "end_idx": 67, "type": "ite", "entity": "两侧肾的大小" }, { "start_idx": 69, "end_idx": 77, "type": "ite", "entity": "肾灌注高峰出现时间" }, { "start_idx": 79, "end_idx": 81, "type": "ite", "entity": "肾功能" }, { "start_idx": 83, "end_idx": 89, "type": "ite", "entity": "两肾间差异程度" }, { "start_idx": 94, "end_idx": 101, "type": "pro", "entity": "ACEI抑制试验" } ] }, { "text": "4.血浆肾素活性和血管紧张素抑制试验。", "entities": [ { "start_idx": 2, "end_idx": 17, "type": "pro", "entity": "血浆肾素活性和血管紧张素抑制试验" } ] }, { "text": "(1)外周血浆肾素活性(PRA)测定:肾素分泌有昼夜节律性,PRA活性上午8时许最低,中午至晚上8时分泌量最高,PRA与高血压不呈简单的平行关系。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "ite", "entity": "外周血浆肾素活性" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "PRA" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肾素" }, { "start_idx": 30, "end_idx": 34, "type": "ite", "entity": "PRA活性" }, { "start_idx": 56, "end_idx": 58, "type": "ite", "entity": "PRA" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "高血" }, { "start_idx": 60, "end_idx": 61, "type": "pro", "entity": "高血" } ] }, { "text": "由于RVH存在的时间、单侧或双侧及严重程度的不同,PRA值的变化很大。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "RVH" }, { "start_idx": 25, "end_idx": 28, "type": "ite", "entity": "PRA值" } ] }, { "text": "患者的PRA可显著增高,少数正常或降低。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "PRA" }, { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "PRA可显著增高" } ] }, { "text": "需在停用降压和利尿药2周后检测,而停用降压利尿药有发生高血压严重并发症的危险,又因小儿原发性高血压、肾实质性高血压,PRA亦可升高,故对RVH的诊断的敏感性和特异性均很差。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "降压和利尿药" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "降压利尿药" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "高血压" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "高血压严重并发症" }, { "start_idx": 41, "end_idx": 48, "type": "dis", "entity": "小儿原发性高血压" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "肾实质性高血压" }, { "start_idx": 58, "end_idx": 60, "type": "ite", "entity": "PRA" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "PRA亦可升高" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "RVH" } ] }, { "text": "(2)血管紧张素抑制试验:比较方便的是采用口服卡托普利(captopril),通过口服卡托普利,可阻断ATⅡ生成,通过负反馈效应使肾素分泌显著增多,以提高检查的敏感性和特异性,观察试验前后PRA的变化。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "血管紧张素抑制试验" }, { "start_idx": 21, "end_idx": 26, "type": "dru", "entity": "口服卡托普利" }, { "start_idx": 28, "end_idx": 36, "type": "dru", "entity": "captopril" }, { "start_idx": 43, "end_idx": 46, "type": "dru", "entity": "卡托普利" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "ATⅡ" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "肾素" }, { "start_idx": 65, "end_idx": 72, "type": "sym", "entity": "肾素分泌显著增多" }, { "start_idx": 94, "end_idx": 96, "type": "ite", "entity": "PRA" } ] }, { "text": "方法:试前2周停服利尿及降压药,给普食,患儿取平卧位,卡托普利(开博通)按0.7mg/kg加水20ml口服(服药后盛药容器需以温开水冲洗,再次服下),在服药前30分钟及服药后1小时采血测PRA及血压,阳性结果是:①舒张压下降≥15%;②血PRA用药前>5ngAI/(ml•h),用药后>10ngAI/(ml•h),用药后比用药前PRA之差>4ngAI/(ml•h)。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "利尿及降压药" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "卡托普利" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "开博通" }, { "start_idx": 93, "end_idx": 95, "type": "ite", "entity": "PRA" }, { "start_idx": 97, "end_idx": 98, "type": "ite", "entity": "血压" }, { "start_idx": 107, "end_idx": 109, "type": "ite", "entity": "舒张压" }, { "start_idx": 107, "end_idx": 115, "type": "sym", "entity": "舒张压下降≥15%" }, { "start_idx": 118, "end_idx": 121, "type": "ite", "entity": "血PRA" }, { "start_idx": 118, "end_idx": 181, "type": "sym", "entity": "血PRA用药前>5ngAI/(ml•h),用药后>10ngAI/(ml•h),用药后比用药前PRA之差>4ngAI/(ml•h)" } ] }, { "text": "用卡托普利后,ATⅡ生成减少,钠潴留反馈抑制肾素分泌解除,肾素分泌增加。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "卡托普利" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "ATⅡ" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "钠潴留" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肾素" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "抑制肾素分泌" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肾素" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "肾素分泌增加" } ] }, { "text": "此试验观察用药后PRA上升幅度比血压下降更有诊断意义。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "PRA" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "血压" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "血压下降" } ] }, { "text": "(二)确诊检查1.数字减影血管造影(digitalsubstractionangiography,DSA)为一项以电子计算机为辅助的X线成像技术,其原理是应用数字式视频影像处理系统,在一张血管造影片中,减去一张尿路平片的骨骼及软组织等阴影。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "pro", "entity": "数字减影血管造影" }, { "start_idx": 18, "end_idx": 47, "type": "pro", "entity": "digitalsubstractionangiography" }, { "start_idx": 49, "end_idx": 51, "type": "pro", "entity": "DSA" }, { "start_idx": 110, "end_idx": 111, "type": "bod", "entity": "骨骼" }, { "start_idx": 113, "end_idx": 115, "type": "bod", "entity": "软组织" } ] }, { "text": "由于消除了其他组织阴影,只剩下唯一的肾动脉图像,使肾动脉显影的清晰度明显提高,可辨认到肾实质内直径<1mm大小的血管。", "entities": [ { "start_idx": 43, "end_idx": 48, "type": "ite", "entity": "肾实质内直径" }, { "start_idx": 43, "end_idx": 52, "type": "sym", "entity": "肾实质内直径<1mm" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "血管" } ] }, { "text": "2.肾动脉造影对筛选试验阳性或筛选试验阴性而仍高度怀疑RVH者可作此项检查。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "肾动脉造影" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "RVH" } ] }, { "text": "采取经皮穿刺插管做血管造影可较好显示包括了弓状动脉在内的肾动脉及其分支的病变、部位、范围、狭窄程度及侧支循环情况,为确诊RVH的可靠方法。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "经皮穿刺插管做血管造影" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "弓状动脉" }, { "start_idx": 28, "end_idx": 34, "type": "bod", "entity": "肾动脉及其分支" }, { "start_idx": 21, "end_idx": 37, "type": "sym", "entity": "弓状动脉在内的肾动脉及其分支的病变" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "RVH" } ] }, { "text": "必要时于造影同时还可施行腔内血管扩张术(PTA)治疗,幼年儿童因血管细小,且又不合作,有时需在静脉麻醉辅助下施行,有一定的危险性。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "腔内血管扩张术" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "PTA" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "血管" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "静脉麻醉" } ] }, { "text": "此项检查前有如伤口出血、血管栓塞和急性肾衰竭等并发症,故应慎重选择病例,术前需作好充分准备。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "伤口出血" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "血管栓塞" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "如造影前,应控制高血压,以防伤口出血,造影后立即静注20%甘露醇20~40ml,继予补液,以减少急性肾衰竭和血管栓塞的并发症。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "高血压" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "静注" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "甘露醇" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "血管栓塞" } ] }, { "text": "3.磁共振血管成像(magneticresonanceangiography,MRA)是一种可靠的非创伤性检查方法,对RVH诊断的准确性可与DSA相同或更完美,因为它是三维空间肾动脉的血管像,可清晰显示肾动脉在主动脉开口处的情况。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "磁共振血管成像" }, { "start_idx": 10, "end_idx": 37, "type": "pro", "entity": "magneticresonanceangiography" }, { "start_idx": 39, "end_idx": 41, "type": "pro", "entity": "MRA" }, { "start_idx": 49, "end_idx": 56, "type": "pro", "entity": "非创伤性检查方法" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "RVH" }, { "start_idx": 70, "end_idx": 72, "type": "pro", "entity": "DSA" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "肾动脉" }, { "start_idx": 101, "end_idx": 103, "type": "bod", "entity": "肾动脉" }, { "start_idx": 105, "end_idx": 107, "type": "bod", "entity": "主动脉" } ] }, { "text": "同时它可以避免由肾动脉造影可能引起的碘过敏,出血、血栓形成等危险和并发症。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "肾动脉造影" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "碘过敏" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "出血" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "血栓形成" } ] }, { "text": "适用于对血管造影剂过敏,心、肾功能不全或有出血素质者。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "血管造影剂过敏" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "心、肾功能不全" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "有出血素质" } ] }, { "text": "药物治疗原则为控制高血压,以防止发生高血压严重并发症的危险,避免肾功能损害,或使已受损的肾功能得到改善,减少心、眼、脑等靶器官的损伤。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "高血压" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "肾功能损害" }, { "start_idx": 44, "end_idx": 46, "type": "ite", "entity": "肾功能" }, { "start_idx": 40, "end_idx": 50, "type": "sym", "entity": "已受损的肾功能得到改善" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "心" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "眼" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "脑" }, { "start_idx": 52, "end_idx": 65, "type": "sym", "entity": "减少心、眼、脑等靶器官的损伤" } ] }, { "text": "作为手术前准备或其他原因不能或不愿进行手术者,常联合应用以下药物以达到控制血压的目的。", "entities": [ { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "血压" } ] }, { "text": "1.β受体阻滞剂可通过抑制肾素而减少醛固酮分泌和水钠潴留而起降压作用,可用盐酸普萘洛尔(心得安)1~3mg/(kg•d),分3次服,阿替洛尔(氨酰心安)或美托洛尔(倍他洛克)学龄前小儿按成人剂量1/4~1/2给药,每日1~2次。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "β受体阻滞剂" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "抑制肾素" }, { "start_idx": 16, "end_idx": 27, "type": "pro", "entity": "减少醛固酮分泌和水钠潴留" }, { "start_idx": 37, "end_idx": 42, "type": "dru", "entity": "盐酸普萘洛尔" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "心得安" }, { "start_idx": 66, "end_idx": 69, "type": "dru", "entity": "阿替洛尔" }, { "start_idx": 71, "end_idx": 74, "type": "dru", "entity": "氨酰心安" }, { "start_idx": 77, "end_idx": 80, "type": "dru", "entity": "美托洛尔" }, { "start_idx": 82, "end_idx": 85, "type": "dru", "entity": "倍他洛克" } ] }, { "text": "2.血管紧张素抑制剂(ACEI)可抑制ATⅡ的血管收缩和醛固酮分泌作用,对RVH有良好的效应,尤其对合并心力衰竭者更为合适。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dru", "entity": "血管紧张素抑制剂" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "ACEI" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "ATⅡ" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "血管" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "醛固酮" }, { "start_idx": 17, "end_idx": 32, "type": "sym", "entity": "抑制ATⅡ的血管收缩和醛固酮分泌" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "RVH" }, { "start_idx": 50, "end_idx": 55, "type": "sym", "entity": "合并心力衰竭" } ] }, { "text": "但对一侧肾已有严重肾实质病变,一侧为肾动脉狭窄(RAS)或双侧RAS患者,可能诱发急性暂时性肾功能不全,不宜应用,因ACEI可减少ATⅡ生成使肾小球出球动脉舒张致肾小球滤过率下降,以致用药后肾功能恶化。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "一侧肾" }, { "start_idx": 2, "end_idx": 13, "type": "sym", "entity": "一侧肾已有严重肾实质病变" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肾动脉" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "一侧为肾动脉狭窄" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "RAS" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "双侧RAS" }, { "start_idx": 46, "end_idx": 48, "type": "ite", "entity": "肾功能" }, { "start_idx": 41, "end_idx": 50, "type": "sym", "entity": "急性暂时性肾功能不全" }, { "start_idx": 58, "end_idx": 61, "type": "dru", "entity": "ACEI" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "ATⅡ" }, { "start_idx": 63, "end_idx": 69, "type": "sym", "entity": "减少ATⅡ生成" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "肾小球" }, { "start_idx": 71, "end_idx": 79, "type": "sym", "entity": "肾小球出球动脉舒张" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "肾小球" }, { "start_idx": 81, "end_idx": 88, "type": "sym", "entity": "肾小球滤过率下降" }, { "start_idx": 95, "end_idx": 97, "type": "ite", "entity": "肾功能" }, { "start_idx": 95, "end_idx": 99, "type": "sym", "entity": "肾功能恶化" } ] }, { "text": "用药1周后要随访血尿素氮和肌酐,并定期以B超声检查随访肾脏大小变化。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "ite", "entity": "血尿素氮" }, { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "肌酐" } ] }, { "text": "新一代高效低毒长效的ACEI如依那普利(enalapril)和福辛普利(fosinpril,monopril)已应用于临床,后者从肾和胆汁两条途径排泄,对肾功能已有不全者(Cr30~60ml/min)亦可应用。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "ACEI" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "依那普利" }, { "start_idx": 20, "end_idx": 28, "type": "dru", "entity": "enalapril" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "福辛普利" }, { "start_idx": 36, "end_idx": 44, "type": "dru", "entity": "fosinpril" }, { "start_idx": 46, "end_idx": 53, "type": "dru", "entity": "monopril" }, { "start_idx": 65, "end_idx": 65, "type": "bod", "entity": "肾" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "胆汁" }, { "start_idx": 77, "end_idx": 79, "type": "ite", "entity": "肾功能" }, { "start_idx": 77, "end_idx": 83, "type": "sym", "entity": "肾功能已有不全" } ] }, { "text": "3.利尿剂常与其他降压药联合使用,单独使用效果欠佳,对低肾素性高血压最有效,常用有氢氯噻嗪,按1~2mg/(kg•d),分2~3次口服,注意低血钾副作用。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "利尿剂" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "低肾素性高血压" }, { "start_idx": 41, "end_idx": 44, "type": "dru", "entity": "氢氯噻嗪" }, { "start_idx": 70, "end_idx": 72, "type": "sym", "entity": "低血钾" } ] }, { "text": "4.钙离子通透阻滞剂如硝苯地平,可减低血管阻力,保持肾脏血流灌注,通过扩张血管而起降压作用,安全可靠,用于治疗单侧或双侧肾动脉狭窄性高血压。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dru", "entity": "钙离子通透阻滞剂" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "硝苯地平" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血管" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "减低血管阻力" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肾脏" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "保持肾脏血流灌注" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "血管" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "扩张血管" }, { "start_idx": 55, "end_idx": 68, "type": "dis", "entity": "单侧或双侧肾动脉狭窄性高血压" } ] }, { "text": "舌下含片3~5分钟可起降压作用,亦用于高血压危象的紧急处理。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "高血压" } ] }, { "text": "5.硝普钠严重高血压,伴有心、脑器官损害,心功能不全及肾功能不全者,应紧急处理,但降压不能过快或降至正常,以控制血压不发生高血压脑病水平为宜。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "硝普钠" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "严重高血压" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "心、脑器官损害" }, { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "心功能" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "心功能不全" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "肾功能" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "肾功能不全" }, { "start_idx": 35, "end_idx": 69, "type": "pro", "entity": "紧急处理,但降压不能过快或降至正常,以控制血压不发生高血压脑病水平为宜" } ] }, { "text": "静脉滴注硝普钠效果可靠,剂量为1~8μg/(kg•min)持续静滴,以后每分钟增加0.1~0.2μg/kg,直至生效或出现不良反应,停止输注后药效只维持2~5分钟,因其作用时间短暂,应同时与其他降压药联合应用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "硝普钠" } ] }, { "text": "(二)外科治疗1.腔内血管扩张术(PTA)在行动脉造影确立诊断时即可行该手术,如PTA未成功或扩张后发生再狭窄可重复再扩张,对PTA无效者可作自体肾移植术或血管重建术。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "腔内血管扩张术" }, { "start_idx": 17, "end_idx": 19, "type": "pro", "entity": "PTA" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "动脉造影" }, { "start_idx": 40, "end_idx": 42, "type": "ite", "entity": "PTA" }, { "start_idx": 40, "end_idx": 60, "type": "sym", "entity": "PTA未成功或扩张后发生再狭窄可重复再扩张" }, { "start_idx": 63, "end_idx": 65, "type": "ite", "entity": "PTA" }, { "start_idx": 71, "end_idx": 76, "type": "pro", "entity": "自体肾移植术" }, { "start_idx": 78, "end_idx": 82, "type": "pro", "entity": "血管重建术" } ] }, { "text": "若一侧肾已失去功能或旁路手术失败,对侧肾功能良好者可根据病情施行部分或全肾切除术。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "一侧肾" }, { "start_idx": 1, "end_idx": 8, "type": "sym", "entity": "一侧肾已失去功能" }, { "start_idx": 19, "end_idx": 21, "type": "ite", "entity": "肾功能" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "对侧肾功能良好" }, { "start_idx": 32, "end_idx": 39, "type": "pro", "entity": "部分或全肾切除术" } ] }, { "text": "近年来由于肾移植和显微外科的发展,肾脏冷却保存可达24小时,有充分时间修复肾动脉,又有肾动脉体外整形术以治疗RAH。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "肾移植" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "肾脏冷却保存" }, { "start_idx": 35, "end_idx": 39, "type": "pro", "entity": "修复肾动脉" }, { "start_idx": 43, "end_idx": 50, "type": "pro", "entity": "肾动脉体外整形术" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "RAH" } ] }, { "text": "2.肾动脉腔内支架术以膨胀性支架放置于经球囊导管扩张的狭窄肾动脉获得成功,为本病治疗开创了新途径。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "肾动脉腔内支架术" }, { "start_idx": 11, "end_idx": 15, "type": "equ", "entity": "膨胀性支架" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "球囊导管" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "肾动脉" }, { "start_idx": 19, "end_idx": 31, "type": "sym", "entity": "经球囊导管扩张的狭窄肾动脉" } ] }, { "text": "第七节主动脉弓异常主动脉弓异常(abnormalitiesoftheaorticarch)指主动脉弓及其分支发育异常。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "主动脉弓异常" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "主动脉弓异常" }, { "start_idx": 16, "end_idx": 43, "type": "dis", "entity": "abnormalitiesoftheaorticarch" }, { "start_idx": 46, "end_idx": 53, "type": "bod", "entity": "主动脉弓及其分支" } ] }, { "text": "主要有血管环血管环(vascularring)及主动脉分支悬带悬带(vascularsling),可致气管及(或)食管受压迫。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "血管环" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "血管环" }, { "start_idx": 10, "end_idx": 21, "type": "bod", "entity": "vascularring" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "主动脉分支悬带" }, { "start_idx": 34, "end_idx": 46, "type": "bod", "entity": "vascularsling" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "气管" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "食管" } ] }, { "text": "主动脉双弓、右位主动脉弓伴迷走左锁骨下动脉及左侧动脉导管或动脉韧带、左肺动脉异常或肺动脉悬带为其中较多见的3种类型。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "主动脉双弓" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "右位主动脉弓" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "左锁骨下动脉" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "左侧动脉导管" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "动脉韧带" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "肺动脉悬带" } ] }, { "text": "在先天性心脏血管畸形中,主动脉弓及其分支畸形仅占1%~2%。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "dis", "entity": "先天性心脏血管畸形" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "主动脉弓及其分支畸形" } ] }, { "text": "在胚胎发育的第4周,从主动脉囊发出6对腮动脉弓并与背主动脉相连接。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "主动脉囊" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "腮动脉弓" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "背主动脉" } ] }, { "text": "此后,第Ⅰ、Ⅱ对鳃动脉弓退化消失。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "鳃动脉弓" } ] }, { "text": "第Ⅲ对鳃动脉弓形成颈总动脉和一部分颈内动脉,第Ⅳ对鳃动脉弓左侧形成主动脉弓,右侧形成无名动脉和右锁骨下动脉。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "鳃动脉弓" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "颈总动脉" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "颈内动脉" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "鳃动脉弓" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "无名动脉" }, { "start_idx": 47, "end_idx": 52, "type": "bod", "entity": "右锁骨下动脉" } ] }, { "text": "第Ⅴ对鳃动脉弓不久亦退化。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "鳃动脉弓" } ] }, { "text": "第6对鳃动脉弓形成肺动脉,其右侧远端与背主动脉分离,左侧在胎儿期持续存在称为动脉导管(图9-9)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "鳃动脉弓" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肺动脉" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "背主动脉" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "动脉导管" } ] }, { "text": "图9-21正常大动脉发育一、血管环血管环(vascularring)最常见类型是主动脉双弓及右位主动脉弓伴迷走左锁骨下动脉及左侧动脉导管或动脉韧带,多为单独畸形,占先天性心脏病1%以下。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "大动脉" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "血管环" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "血管环" }, { "start_idx": 21, "end_idx": 32, "type": "dis", "entity": "vascularring" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "主动脉双弓" }, { "start_idx": 46, "end_idx": 51, "type": "bod", "entity": "右位主动脉弓" }, { "start_idx": 55, "end_idx": 60, "type": "bod", "entity": "左锁骨下动脉" }, { "start_idx": 62, "end_idx": 67, "type": "bod", "entity": "左侧动脉导管" }, { "start_idx": 69, "end_idx": 72, "type": "bod", "entity": "动脉韧带" }, { "start_idx": 82, "end_idx": 87, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "Edwards建立了主动脉弓畸形模型,使对主动脉弓的5种类型容易理解(图9-22)。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "主动脉弓畸形" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "主动脉弓" } ] }, { "text": "【病理解剖】(一)主动脉双弓主动脉双弓畸形是由于任何部分的左弓或右弓未正常退化所致。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "主动脉双弓" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "主动脉双弓畸形" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "左弓" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "右弓" } ] }, { "text": "双侧第4鳃动脉弓均存在并形成主动脉弓。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "鳃动脉弓" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "主动脉弓" } ] }, { "text": "升主动脉正常,在心包膜外分为左、右两支主动脉弓。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "升主动脉" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "心包膜" }, { "start_idx": 14, "end_idx": 22, "type": "bod", "entity": "左、右两支主动脉弓" } ] }, { "text": "左侧主动脉弓在气管前方从右向左行走,越过左主支气管,在脊柱左侧与右侧主动脉弓汇合成降主动脉。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "左侧主动脉弓" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "气管" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "左主支气管" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "脊柱左侧" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "右侧主动脉弓" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "降主动脉" } ] }, { "text": "右侧主动脉弓跨越右侧主支气管在脊柱前方、食管后方,越过中线向左向下行,与左侧主动脉弓汇合成降主动脉。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "右侧主动脉弓" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "右侧主支气管" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "脊柱" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "食管" }, { "start_idx": 36, "end_idx": 41, "type": "bod", "entity": "左侧主动脉弓" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "降主动脉" } ] }, { "text": "双侧主动脉弓均存在并完全包绕气管和食管,每个弓分别发出两根大动脉。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "气管" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "食管" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "大动脉" } ] }, { "text": "绝大多数的动脉导管位于左侧。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "动脉导管" } ] }, { "text": "在约90%的患儿,右弓较左弓粗大且位置较高。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "右弓" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "左弓" } ] }, { "text": "有时左弓在颈总动脉和锁骨下动脉间或在左锁骨下动脉远端出现闭锁。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "左弓" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "颈总动脉" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "锁骨下动脉" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "左锁骨下动脉" } ] }, { "text": "(二)右位主动脉弓伴左侧锁骨下动脉迷走右弓伴左锁骨下动脉迷走为胚胎发育时左弓在左颈总和左锁骨下之间退化所致,常不伴有其他心内畸形。", "entities": [ { "start_idx": 3, "end_idx": 18, "type": "dis", "entity": "右位主动脉弓伴左侧锁骨下动脉迷走" }, { "start_idx": 19, "end_idx": 29, "type": "dis", "entity": "右弓伴左锁骨下动脉迷走" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "左弓" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "左颈总" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "左锁骨" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "心内畸形" } ] }, { "text": "左锁骨下动脉起源于第Ⅳ腮弓,在主动脉弓后方发出,在食管后方走行并供应左上肢。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "左锁骨下动脉" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "第Ⅳ腮弓" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "食管" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "左上肢" } ] }, { "text": "左侧动脉导管在左锁骨下动脉起始部的主动脉憩室发出,连接与左肺动脉,将主动脉及憩室拉向前方形成血管环并压迫食管和气管。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "左侧动脉导管" }, { "start_idx": 7, "end_idx": 15, "type": "bod", "entity": "左锁骨下动脉起始部" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "主动脉憩室" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "主动脉" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "憩室" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "血管环" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "食管" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "气管" } ] }, { "text": "通常该血管环较松,但当动脉导管较短时可引起压迫症状。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "血管环" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "动脉导管" } ] }, { "text": "【临床表现】表现为气管压迫,可有喘鸣、气急和犬吠样咳嗽并在运动和进食时加剧。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "气管" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "气管压迫" }, { "start_idx": 14, "end_idx": 36, "type": "sym", "entity": "可有喘鸣、气急和犬吠样咳嗽并在运动和进食时加剧" } ] }, { "text": "食管压迫症状较少见,在婴儿可有呕吐、噫塞和喂养困难,在儿童可表现为吞咽困难。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "食管压迫" }, { "start_idx": 10, "end_idx": 24, "type": "sym", "entity": "在婴儿可有呕吐、噫塞和喂养困难" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "在儿童可表现为吞咽困难" } ] }, { "text": "多数在儿童期随着主动脉及锁骨上动脉的增大而出现症状。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "主动脉" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "锁骨上动脉" } ] }, { "text": "在右位主动脉弓伴左侧锁骨下动脉迷走时,由于动脉导管较短或主动脉憩室较大时可出现同样的气管、食管压迫症状。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "右位主动脉弓" }, { "start_idx": 8, "end_idx": 16, "type": "bod", "entity": "左侧锁骨下动脉迷走" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "动脉导管" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "主动脉憩室" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "气管" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "食管" }, { "start_idx": 42, "end_idx": 50, "type": "sym", "entity": "气管、食管压迫症状" } ] }, { "text": "图9-22Edwards主动脉弓畸形模型DA:降主动脉AA:升主动脉RS:右锁骨下动脉LS:左锁骨下动脉RC:右颈总动脉LC:左颈总动脉E:为食管T:为气管A.主动脉双弓;B.正常左位主动脉弓;C.左弓右锁骨下动脉迷走;D.右位主动脉弓;E.右弓左锁骨下动脉迷走听诊可及气道的喘鸣音,可有轻度的青紫出现,喜欢背屈、仰颈体位。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "主动脉弓畸形" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "降主动脉" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "升主动脉" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "右锁骨下动脉" }, { "start_idx": 46, "end_idx": 51, "type": "bod", "entity": "左锁骨下动脉" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "右颈总动脉" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "左颈总动脉" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "食管" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "气管" }, { "start_idx": 80, "end_idx": 84, "type": "bod", "entity": "主动脉双弓" }, { "start_idx": 88, "end_idx": 95, "type": "bod", "entity": "正常左位主动脉弓" }, { "start_idx": 99, "end_idx": 108, "type": "dis", "entity": "左弓右锁骨下动脉迷走" }, { "start_idx": 112, "end_idx": 117, "type": "bod", "entity": "右位主动脉弓" }, { "start_idx": 121, "end_idx": 130, "type": "dis", "entity": "右弓左锁骨下动脉迷走" }, { "start_idx": 131, "end_idx": 132, "type": "pro", "entity": "听诊" }, { "start_idx": 135, "end_idx": 136, "type": "bod", "entity": "气道" }, { "start_idx": 135, "end_idx": 140, "type": "sym", "entity": "气道的喘鸣音" }, { "start_idx": 142, "end_idx": 150, "type": "sym", "entity": "可有轻度的青紫出现" }, { "start_idx": 152, "end_idx": 155, "type": "sym", "entity": "喜欢背屈" }, { "start_idx": 157, "end_idx": 160, "type": "sym", "entity": "仰颈体位" } ] }, { "text": "【辅助检查】(一)胸部X线检查右位主动脉弓为血管环诊断的重要提示,正常情况下气管稍偏右侧,在平片无法清晰显示主动脉弓时,如平片上气管居中提示右位主动脉弓的存在。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "右位主动脉弓" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "血管环" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "气管" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "气管" }, { "start_idx": 70, "end_idx": 75, "type": "bod", "entity": "右位主动脉弓" } ] }, { "text": "双主动脉弓病例可显示双侧主动脉弓球形隆起,右侧更为明显。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "双主动脉弓" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "双侧主动脉弓" } ] }, { "text": "食管造影检查可显示在胸椎第3、4水平上段食管两侧压迹,右主动脉弓造成的压迹较大且位置较高,左主动脉弓造成的压迹较小且位置较低。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "食管造影" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "胸椎" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "食管" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "右主动脉弓" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "左主动脉弓" } ] }, { "text": "CT检查可能显示气管腔受压迫的征象。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "CT检查" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "气管腔" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "气管腔受压迫" } ] }, { "text": "左锁骨下动脉迷走患儿食管造影可显示食管后壁受血管压迫呈现斜行或螺旋形的压迹。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "左锁骨下动脉迷走" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "食管造影" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "食管后壁" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血管" } ] }, { "text": "婴儿作食管造影检查宜用碘油或水溶性造影剂,因钡剂造影剂如吸入气管支气管内,有加重呼吸困难或引致吸入性肺炎的危险。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "食管造影检查" }, { "start_idx": 11, "end_idx": 12, "type": "dru", "entity": "碘油" }, { "start_idx": 14, "end_idx": 19, "type": "dru", "entity": "水溶性造影剂" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "钡剂造影剂" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "气管支气管" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "吸入性肺炎" } ] }, { "text": "(二)支气管镜检查支气管镜检查可以明确气管受压迫的部位,并可在受压处观察到血管搏动,但引起的呼吸道黏膜创伤和水肿可加重呼吸道梗阻,必须十分慎重。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "支气管镜检查" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "支气管镜检查" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "气管" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "血管" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "呼吸道黏膜创伤" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "水肿" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "呼吸道梗阻" } ] }, { "text": "(三)主动脉造影检查主动脉造影是确诊主动脉弓及其分支畸形最升主动脉诊断方法。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "主动脉造影检查" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "主动脉造影" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "主动脉弓" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "升主动脉" } ] }, { "text": "于升主动脉内插入导管,注入造影剂进行主动脉及其分支造影,双向电影摄片检查可显示主动脉弓及其分支的起源、走向、粗细和其他异常,从而明确诊断。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "equ", "entity": "导管" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "造影剂" }, { "start_idx": 18, "end_idx": 26, "type": "pro", "entity": "主动脉及其分支造影" }, { "start_idx": 28, "end_idx": 35, "type": "pro", "entity": "双向电影摄片检查" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "主动脉弓" } ] }, { "text": "【治疗】对所有有症状的血管环患儿均应进行外科手术治疗。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "血管环" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "外科手术" } ] }, { "text": "手术治疗时需经左胸径路,双弓畸形者应将较小的主动脉弓离断(通常为左侧),右位主动脉弓伴左侧锁骨下动脉迷走者则离断动脉导管韧带以松开气管、食管的压迫。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "左胸径" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "双弓畸形" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "右位主动脉弓" }, { "start_idx": 43, "end_idx": 51, "type": "dis", "entity": "左侧锁骨下动脉迷走" }, { "start_idx": 56, "end_idx": 61, "type": "bod", "entity": "动脉导管韧带" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "气管" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "食管" } ] }, { "text": "手术患者在术后仍有肺功能异常,可能与压迫后气道器质性残余梗阻有关。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肺" }, { "start_idx": 21, "end_idx": 29, "type": "dis", "entity": "气道器质性残余梗阻" } ] }, { "text": "七、淋巴管肉瘤淋巴管肉瘤(lymphangiosarcoma)在小儿极为罕见,高度恶性。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "淋巴管肉瘤" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "淋巴管肉瘤" }, { "start_idx": 13, "end_idx": 29, "type": "dis", "entity": "lymphangiosarcoma" } ] }, { "text": "肿瘤起源于淋巴管内皮,其原因与放疗等原因引起的慢性淋巴水肿有关,先天型淋巴水肿也可发生。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "淋巴管内皮" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "放疗" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "慢性淋巴水肿" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "先天型淋巴水肿" } ] }, { "text": "肿瘤内见不规则的脉管结构,衬以恶性内皮细胞,细胞呈卵圆形及棱形胞质。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "脉管" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "肿瘤内见不规则的脉管结构" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "恶性内皮细胞" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "衬以恶性内皮细胞" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "细胞" }, { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "细胞呈卵圆形及棱形胞质" } ] }, { "text": "第Ⅷ因子相关抗原也是本瘤的一个标志。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "抗原" } ] }, { "text": "肿瘤呈现一个坚韧的肿块,如色青的瘀斑,高出皮面相近的皮肤、皮下组织有纤维化肿瘤多发生于四肢,向近远端发展。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肿块" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "色青的瘀斑" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "皮面" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "高出皮面" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "皮肤" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "皮下组织" }, { "start_idx": 23, "end_idx": 36, "type": "sym", "entity": "相近的皮肤、皮下组织有纤维化" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "肿瘤" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "四肢" } ] }, { "text": "如有肿瘤症状,即作根治手术。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "手术" } ] }, { "text": "四、并发症儿科纤维支气管镜检查的并发症较少见,大多因麻醉不当或操作不熟练所致。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dep", "entity": "儿科" }, { "start_idx": 7, "end_idx": 14, "type": "pro", "entity": "纤维支气管镜检查" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "麻醉" } ] }, { "text": "(一)喉水肿是最常见的并发症,多在术后2小时内出现。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "喉水肿" } ] }, { "text": "应选择粗细合适的纤维支气管镜,操作动作轻柔,检查时间不宜过长,术毕要观察半小时左右再送出手术室。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 44, "end_idx": 46, "type": "dep", "entity": "手术室" } ] }, { "text": "一旦出现喉水肿,在氧气吸入的同时,静脉注射地塞米松、雾化吸入肾上腺素和布地奈德能有效防止喉梗阻的发生。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "喉水肿" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "氧气吸入" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "静脉注射" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "地塞米松" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "布地奈德" }, { "start_idx": 44, "end_idx": 46, "type": "sym", "entity": "喉梗阻" } ] }, { "text": "(二)喉痉挛多由于麻醉不充分,刺激喉部发生。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "sym", "entity": "喉痉挛" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "麻醉" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "喉部" } ] }, { "text": "加深麻醉或对喉头进行表面麻醉后可消失。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "麻醉" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "喉头" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "表面麻醉" } ] }, { "text": "(三)黏膜出血多由于气道黏膜炎症、负压吸引及取异物、活检创伤所致。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "黏膜出血" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "气道黏膜炎症" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "负压吸引" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "活检" } ] }, { "text": "用纤维支气管镜直接压迫出血处或注入少量1∶10000肾上腺素液多能止血。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "压迫出血处" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "肾上腺素液" } ] }, { "text": "少数患儿可引起大咯血,甚至气道堵塞、窒息死亡。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "大咯血" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "气道堵塞" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "窒息死亡" } ] }, { "text": "活检时尤其应该小心谨慎,一旦发生严重出血,应及时抽吸积血,并肌内注射或经镜管内滴入垂体后叶素、酚磺乙胺等药物。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "活检" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "严重出血" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "抽吸积血" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "肌内注射" }, { "start_idx": 36, "end_idx": 37, "type": "equ", "entity": "镜管" }, { "start_idx": 41, "end_idx": 45, "type": "dru", "entity": "垂体后叶素" }, { "start_idx": 47, "end_idx": 50, "type": "dru", "entity": "酚磺乙胺" } ] }, { "text": "(四)缺氧或发绀纤维支气管镜术中可引起短暂性PaO2下降,如频繁吸引可造成通气不足而缺氧,其他原因包括原有肺功能不全、呼吸抑制、支气管痉挛、用药过量等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "缺氧" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "发绀" }, { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "纤维支气管镜术" }, { "start_idx": 22, "end_idx": 36, "type": "ite", "entity": "PaO2" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "通气不足而缺氧" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "原有肺功能不全" }, { "start_idx": 70, "end_idx": 73, "type": "dis", "entity": "呼吸抑制" }, { "start_idx": 75, "end_idx": 79, "type": "dis", "entity": "支气管痉挛" } ] }, { "text": "可经纤维支气管镜活检孔给氧或口鼻腔给氧,新生儿超细纤维支气管镜检查时可通过附加管道气管内给氧。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "equ", "entity": "纤维支气管镜活检孔" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "给氧" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "口鼻腔" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "给氧" }, { "start_idx": 20, "end_idx": 32, "type": "pro", "entity": "新生儿超细纤维支气管镜检查" }, { "start_idx": 37, "end_idx": 40, "type": "equ", "entity": "附加管道" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "气管" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "给氧" } ] }, { "text": "(五)其他如气胸或纵隔气肿、麻醉药物过敏、继发感染、心动过速等。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "气胸" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "纵隔气肿" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "麻醉药物过敏" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "继发感染" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "心动过速" } ] }, { "text": "纤维支气管镜的应用价值和前景毋容置疑,具体应用中的主要问题是确保安全。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "纤维支气管镜" } ] }, { "text": "三、新生儿贫血的治疗应根据新生儿贫血的不同类型、严重程度,给予相应治疗。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "新生儿贫血" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "新生儿贫血" } ] }, { "text": "对无症状的轻、中度贫血,以病因治疗为主,并补充维生素E和铁剂等,尽量减少输血。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "贫血" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "维生素E" }, { "start_idx": 28, "end_idx": 29, "type": "dru", "entity": "铁剂" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "输血" } ] }, { "text": "对重度贫血、急性失血性贫血,应进行输血治疗。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "重度贫血" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "急性失血性贫血" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "输血" } ] }, { "text": "(一)失血性贫血的治疗1.纠正低血容量性休克急性失血可导致休克,须紧急处理,应先给生理盐水扩容,20ml/kg,快速静脉滴注。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "失血性贫血" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "低血容量性休克" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "急性失血" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "休克" }, { "start_idx": 41, "end_idx": 44, "type": "dru", "entity": "生理盐水" }, { "start_idx": 58, "end_idx": 61, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "如血压仍不稳定,可适当增加扩容量。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "血压" } ] }, { "text": "如血压稳定,给维持量补液。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "血压" } ] }, { "text": "2.纠正贫血经过扩容,血压稳定后,如贫血较明显者,考虑输血,以纠正贫血。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "贫血" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "血压" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "贫血" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "输血" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "贫血" } ] }, { "text": "(二)溶血性贫血的治疗详见本篇第十六节新生儿溶血病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "溶血性贫血" } ] }, { "text": "(三)早产儿贫血的治疗1.使用重组人促红细胞生成素(rhEPO)(1)预防:对出生体重小于1500g的早产儿,生后7天,给rhEPO200U/kg,每周3次,皮下注射或静脉滴注,疗程4周。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "早产儿贫血" }, { "start_idx": 15, "end_idx": 24, "type": "dru", "entity": "重组人促红细胞生成素" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "rhEPO" }, { "start_idx": 79, "end_idx": 82, "type": "pro", "entity": "皮下注射" }, { "start_idx": 84, "end_idx": 87, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "(2)治疗:对已发生贫血者,给rhEPO300U/kg,每周3次,疗程4周。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "贫血" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "rhEPO" } ] }, { "text": "使用rhEPO可缓解贫血严重程度,减少输血次数,但不能避免输血。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "rhEPO" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "贫血" } ] }, { "text": "2.补充维生素E和铁剂在使用rhEPO的同时给维生素E10mg/d,分2次口服,1周后给3%硫酸亚铁3mg/(kg•d),分2次口服,每周增加2mg/(kg•d),至7mg/(kg•d)维持。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "维生素E" }, { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "铁剂" }, { "start_idx": 14, "end_idx": 18, "type": "dru", "entity": "rhEPO" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "维生素E" }, { "start_idx": 44, "end_idx": 49, "type": "dru", "entity": "3%硫酸亚铁" } ] }, { "text": "3.输血如血红蛋白低于80g/L并出现以下情况者需输血:胎龄小于30周,安静时呼吸增快>50次/分、心率加快>160次/分,进食易疲劳,每天体重增加<25g,血乳酸>1.8mmol/L。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "输血" }, { "start_idx": 39, "end_idx": 40, "type": "ite", "entity": "呼吸" }, { "start_idx": 50, "end_idx": 51, "type": "ite", "entity": "心率" }, { "start_idx": 70, "end_idx": 71, "type": "ite", "entity": "体重" }, { "start_idx": 79, "end_idx": 81, "type": "ite", "entity": "血乳酸" } ] }, { "text": "输血量每次10~15ml/kg。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "输血量" } ] }, { "text": "(四)输血疗法1.输血量根据贫血类型及轻重缓急而不同,也可根据公式计算,所需全血量(ml)=体重(kg)×(预期达到Hb-实际Hb)×6(6ml血提高1gHb),如输压缩红细胞,为全血量的1/2。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "贫血" } ] }, { "text": "2.注意事项重度贫血输血速度应缓慢。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "重度贫血" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "输血" } ] }, { "text": "充血性心力衰竭患儿,为防止输血引起血容量进一步增加,输血前可静脉注射呋塞米0.5mg/kg。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "输血" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "血容量" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "输血" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "静脉注射" }, { "start_idx": 34, "end_idx": 36, "type": "dru", "entity": "呋塞米" } ] }, { "text": "(五)其他治疗要积极治疗原发病及并发症。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "原发病" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "并发症" } ] }, { "text": "二、GINA治疗方案(一)GINA治疗方案的形成与演变1994年在美国国立卫生院心肺血液研究所与世界卫生组织的共同努力下,17个国家的30多位专家组成小组,制定了关于哮喘管理和预防的全球策略,即《全球哮喘防治创议》(GlobalInitiativeforAsthma,GINA),用来规范哮喘的防治。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "GINA治疗" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "GINA治疗" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "心" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "肺" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "血液" }, { "start_idx": 83, "end_idx": 84, "type": "dis", "entity": "哮喘" }, { "start_idx": 144, "end_idx": 145, "type": "dis", "entity": "哮喘" } ] }, { "text": "早期GINA是根据症状、气流受限的程度以及肺功能的改变,对哮喘病情进行严重程度的分级(即间歇、轻度持续、中度持续、重度持续),并根据分级采用相应的治疗方案。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "GINA" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "肺" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "哮喘" } ] }, { "text": "但随着在临床上广泛的推广,也感觉哮喘严重程度既涉及疾病本身的严重性,也涉及其对治疗的反应。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "哮喘" } ] }, { "text": "而且哮喘严重程度在具体某一哮喘患儿也不是一成不变的,可能在不同季节或环境改变后发生改变。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "哮喘" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "哮喘" } ] }, { "text": "所以,就哮喘管理的持续性而言,根据控制水平对哮喘进行分类更符合实际情况(表8-8)。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "哮喘" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "哮喘" } ] }, { "text": "表8-22喷射性雾化吸入用药注:普米克令舒可与0.9%生理盐水、特布他林、沙丁胺醇、色甘酸钠和溴化异丙托品混合使用表8-23儿童哮喘严重度分级注:①评估过去2~4周日间症状、夜间症状/憋醒、应急缓解药使用和活动受限情况;②患儿只要具有某级严重程度的任一项特点,就将其列为该级别;③任何级别严重程度,包括间歇状态,都可以出现严重的急性发作表8-24儿童哮喘控制水平分级注:①评估过去2~4周日间症状、夜间症状/憋醒、应急缓解药使用和活动受限情况;②出现任何一次急性发作都应复核维持治疗方案是否需要调整(二)确定长期治疗方案根据年龄分为5岁及以上儿童哮喘和5岁以下儿童哮喘的长期治疗方案。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "喷射性雾化" }, { "start_idx": 16, "end_idx": 20, "type": "dru", "entity": "普米克令舒" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "生理盐水" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "特布他林" }, { "start_idx": 37, "end_idx": 40, "type": "dru", "entity": "沙丁胺醇" }, { "start_idx": 42, "end_idx": 45, "type": "dru", "entity": "色甘酸钠" }, { "start_idx": 47, "end_idx": 52, "type": "dru", "entity": "溴化异丙托品" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "儿童哮喘" }, { "start_idx": 173, "end_idx": 176, "type": "dis", "entity": "儿童哮喘" }, { "start_idx": 280, "end_idx": 283, "type": "dis", "entity": "儿童哮喘" } ] }, { "text": "如哮喘控制,并维持至少3个月,治疗方案可考虑降级,直至确定维持哮喘控制的最小剂量。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "哮喘" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "哮喘" } ] }, { "text": "(三)常用哮喘维持治疗药物吸入糖皮质激素(ICS)种类ICS治疗哮喘的高效性和局部选择性的主要化学基础是在于激素甾体核的16α和17α或17β位置上有一个亲脂基团的置换。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "哮喘" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "ICS" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "ICS" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "哮喘" } ] }, { "text": "当甾体核的D环上用亲脂基团替代可得到三种重要特性:①与激素受体有非常高度的亲和性,这是在呼吸道黏膜发挥作用所必需的;②能增加局部摄取(浓度)和延长在组织中储存时间;③全身吸收后,易被肝脏转化而快速灭活。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "激素受体" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "呼吸道黏膜" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "肝脏" } ] }, { "text": "但一定程度的水溶性也十分重要,ICS必须首先溶解在气道黏液中,然后才能作用于气道组织,因而一个理想的ICS除了较强的脂溶性外,还需要一定的水溶性。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "ICS" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "气道黏液" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "气道组织" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "ICS" } ] }, { "text": "ICS的局部/全身作用的比例取决于:①药物在气道中的局部活性;②下呼吸道与口咽部药物沉积之比;③药物经肺或胃肠道吸收和首过代谢的周身活性。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "ICS" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "下呼吸道" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "口咽部" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "肺" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "胃肠道" } ] }, { "text": "目前临床上常用的ICS有以下三大类:(1)二丙酸倍氯米松(beclomethasonide,BDP):如必可酮、贝可乐;BDP是丙酸倍氯米松(BMP)的前体,BMP比BDP具有更高的受体亲和力,BDP水溶性低,在肺组织中转化成BMP。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "ICS" }, { "start_idx": 21, "end_idx": 27, "type": "dru", "entity": "二丙酸倍氯米松" }, { "start_idx": 29, "end_idx": 44, "type": "dru", "entity": "beclomethasonide" }, { "start_idx": 46, "end_idx": 48, "type": "dru", "entity": "BDP" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "必可酮" }, { "start_idx": 56, "end_idx": 58, "type": "dru", "entity": "贝可乐" }, { "start_idx": 60, "end_idx": 62, "type": "dru", "entity": "BDP" }, { "start_idx": 64, "end_idx": 69, "type": "dru", "entity": "丙酸倍氯米松" }, { "start_idx": 71, "end_idx": 73, "type": "dru", "entity": "BMP" }, { "start_idx": 79, "end_idx": 81, "type": "dru", "entity": "BMP" }, { "start_idx": 83, "end_idx": 85, "type": "dru", "entity": "BDP" }, { "start_idx": 97, "end_idx": 99, "type": "dru", "entity": "BDP" }, { "start_idx": 106, "end_idx": 108, "type": "bod", "entity": "肺组织" }, { "start_idx": 113, "end_idx": 115, "type": "dru", "entity": "BMP" } ] }, { "text": "肝脏灭活速度慢,并且在肝脏代谢后会产生另一种活性产物(倍氯米松);因而全身不良反应相对较大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肝脏" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肝脏" } ] }, { "text": "(2)布地奈德(budesonide,BUD):普米克都保或pMDI、英福美;BUD比BDP有较高的受体亲和性和水溶性,而与BMP接近。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "布地奈德" }, { "start_idx": 8, "end_idx": 17, "type": "dru", "entity": "budesonide" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "BUD" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "普米克都保" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "pMDI" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "英福美" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "BUD" }, { "start_idx": 43, "end_idx": 45, "type": "dru", "entity": "BDP" }, { "start_idx": 62, "end_idx": 64, "type": "dru", "entity": "BMP" } ] }, { "text": "BUD肝脏灭活速度较BMP快,肝脏通过两种代谢途径进行代谢,首过代谢为90%,半衰期2.8小时。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "BUD" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肝脏" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "BMP" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肝脏" } ] }, { "text": "图8-16≥5岁儿童哮喘的长期治疗方案(3)氟地卡松(fluticasonepropionate,FP):如辅舒酮pMDI。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "儿童哮喘" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "氟地卡松" }, { "start_idx": 27, "end_idx": 47, "type": "dru", "entity": "fluticasonepropionate" }, { "start_idx": 49, "end_idx": 50, "type": "dru", "entity": "FP" }, { "start_idx": 54, "end_idx": 60, "type": "dru", "entity": "辅舒酮pMDI" } ] }, { "text": "FP与BDP一样水溶性低,但受体亲和力高;FP只通过一种代谢途径,首过代谢为99%,半衰期8~14小时。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "FP" }, { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "BDP" }, { "start_idx": 21, "end_idx": 22, "type": "dru", "entity": "FP" } ] }, { "text": "长半衰期增加了反复用药的危险性,可导致组织内药物高浓度;FP的长半衰期可能与其高亲脂性有关,可增加组织结合和分布容积。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "dru", "entity": "FP" } ] }, { "text": "应用ICS应注意根据年龄选择合适的吸入装置,以增加吸入效率(表8-8)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "ICS" } ] }, { "text": "图8-17<5岁儿童哮喘的长期治疗方案表8-25不同年龄的吸入策略(四)白三烯受体拮抗剂白三烯是人体三种必需脂肪酸之一的花生四烯酸的脂氧化酶代谢产物,包括LTA4、LTB4、LTC4、LTD4和LTE4;其中LTC4、LTD4和LTE4被称为“半胱氨酰白三烯”,因为它们都包含一个硫醚连接的肽,主要由嗜酸性粒细胞、肥大细胞、巨噬细胞、单核细胞和嗜碱粒细胞产生。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "儿童哮喘" }, { "start_idx": 36, "end_idx": 43, "type": "dru", "entity": "白三烯受体拮抗剂" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "花生四烯酸" }, { "start_idx": 66, "end_idx": 69, "type": "mic", "entity": "脂氧化酶" }, { "start_idx": 198, "end_idx": 203, "type": "bod", "entity": "嗜酸性粒细胞" }, { "start_idx": 205, "end_idx": 208, "type": "bod", "entity": "肥大细胞" }, { "start_idx": 210, "end_idx": 213, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 215, "end_idx": 218, "type": "bod", "entity": "单核细胞" }, { "start_idx": 220, "end_idx": 224, "type": "bod", "entity": "嗜碱粒细胞" } ] }, { "text": "半胱氨酰白三烯是引起哮喘慢性气道炎症的重要炎性介质之一。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "哮喘慢性气道炎症" } ] }, { "text": "孟鲁司特钠和扎鲁司特是口服的选择性白三烯受体拮抗剂,能特异性抑制半胱氨酰白三烯(CysLT1)受体,以阻断白三烯引起的气道炎症;与糖皮质激素合用,可减少激素用量。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "孟鲁司特钠" }, { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "扎鲁司特" }, { "start_idx": 14, "end_idx": 24, "type": "dru", "entity": "选择性白三烯受体拮抗剂" }, { "start_idx": 32, "end_idx": 48, "type": "mic", "entity": "半胱氨酰白三烯(CysLT1)受体" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "气道炎症" }, { "start_idx": 65, "end_idx": 69, "type": "dru", "entity": "糖皮质激素" } ] }, { "text": "常用药物为孟鲁司特钠(montelukastsodium),商品名为顺尔宁颗粒剂或咀嚼片:6~14岁5mg,2~5岁4mg,每晚服。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "孟鲁司特钠" }, { "start_idx": 11, "end_idx": 27, "type": "dru", "entity": "montelukastsodium" }, { "start_idx": 34, "end_idx": 43, "type": "dru", "entity": "顺尔宁颗粒剂或咀嚼片" } ] }, { "text": "(五)肥大细胞膜稳定剂是一种非糖皮质激素类抗炎制剂,可抑制肥大细胞释放介质,对其他炎症细胞释放介质也有选择性抑制作用;主要用于轻中度哮喘患者。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dru", "entity": "肥大细胞膜稳定剂" }, { "start_idx": 14, "end_idx": 24, "type": "dru", "entity": "非糖皮质激素类抗炎制剂" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "肥大细胞" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "介质" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "炎症细胞" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "介质" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "轻中度哮喘" } ] }, { "text": "此类药物包括:色甘酸钠、尼多酸钠和酮替酚。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "色甘酸钠" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "尼多酸钠" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "酮替酚" } ] }, { "text": "(六)长效或缓释支气管扩张剂主要用于缓解期的轻中度咳喘症状,特别是夜间咳喘以及运动后咳喘。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dru", "entity": "长效或缓释支气管扩张剂" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "轻中度咳喘" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "夜间咳喘" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "运动后咳喘" } ] }, { "text": "1.长效或控释β2受体激动剂(1)沙丁胺醇缓释片(Etinoline)每片4mg。", "entities": [ { "start_idx": 23, "end_idx": 29, "type": "dru", "entity": "沙丁胺醇缓释片" }, { "start_idx": 31, "end_idx": 39, "type": "dru", "entity": "Etinoline" } ] }, { "text": "(2)丙卡特罗(Meptin)每片25μg。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "丙卡特罗" }, { "start_idx": 8, "end_idx": 13, "type": "dru", "entity": "Meptin" } ] }, { "text": "2.氨茶碱控释片(1)舒弗美:每片100mg。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "氨茶碱控释片" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "舒弗美" } ] }, { "text": "第二节偏头痛偏头痛(migraine)是一种反复发作的神经血管性头痛,多在单侧,每次发作性质与过程相似,间歇期正常。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "偏头痛" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "偏头痛" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "神经血管性头痛" } ] }, { "text": "可伴发恶心、呕吐、视觉改变以及对光和声音的过度敏感等症状。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "恶心" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "呕吐" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "视觉改变" }, { "start_idx": 15, "end_idx": 24, "type": "sym", "entity": "对光和声音的过度敏感" } ] }, { "text": "【流行病学】在小儿神经门诊初诊病人中,22%的患儿以头痛为主诉,其中约1/2为小儿偏头痛。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dep", "entity": "小儿神经门诊" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "头痛" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "小儿偏头痛" } ] }, { "text": "关于偏头痛的流行病学调查,由于调查的年龄范围、诊断标准及调查方式不同,调查结果往往存在差异。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "偏头痛" } ] }, { "text": "偏头痛可见于任何年龄的儿童,特别是青春期前后的女孩。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "偏头痛" } ] }, { "text": "小儿偏头痛发病年龄多为6~10岁,平均7.5岁;但6岁以前发病也不少见,文献报告有5个月起病者。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "小儿偏头痛" } ] }, { "text": "一般来讲,在6~12岁儿童中,偏头痛的患病率为2%~5%;此后随年龄增加而逐渐增多,14岁左右患病率约为10%;成人患病率为10%~30%。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "偏头痛" } ] }, { "text": "在所有儿童中,偏头痛的发病率为3%~7%。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "偏头痛" } ] }, { "text": "【病因与发病机制】偏头痛真正的病因与发病机制尚未明确,提出了许多学说,但偏头痛发作时颅内、外血管舒缩障碍已被证实。", "entities": [ { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "偏头痛" }, { "start_idx": 42, "end_idx": 51, "type": "sym", "entity": "颅内、外血管舒缩障碍" } ] }, { "text": "目前认为偏头痛是在遗传素质基础上形成的局部颅内外血管对神经-体液调节机制的阵发性异常反应。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "偏头痛" }, { "start_idx": 19, "end_idx": 43, "type": "sym", "entity": "局部颅内外血管对神经-体液调节机制的阵发性异常反应" } ] }, { "text": "(一)遗传因素现认为偏头痛与遗传有关,其阳性家族史为50%~80%。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "偏头痛" } ] }, { "text": "双亲都患偏头痛的,其子女患偏头痛的约占70%;单亲患偏头痛的,子女的患病机会约50%;单卵双胎共同发生率为50%以上。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "偏头痛" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "偏头痛" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "偏头痛" } ] }, { "text": "但基底动脉型偏头痛和家族性偏瘫型偏头痛例外,呈常染色体显性遗传。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "基底动脉型偏头痛" }, { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "家族性偏瘫型偏头痛" } ] }, { "text": "家族性偏瘫型偏头痛的致病基因可能定位于19p13.1-1.2。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "家族性偏瘫型偏头痛" } ] }, { "text": "Ducros等于1997年将家族性偏瘫型偏头痛的致病基因定位于1q21-23,提示该病具有遗传异质性。", "entities": [ { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "家族性偏瘫型偏头痛" } ] }, { "text": "(二)血管源学说认为偏头痛的先兆症状与颅内血管的收缩有关,随后由于颅内、外血管的扩张,血管周围组织产生血管活性多肽,导致无菌性炎症而诱发头痛。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "偏头痛" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "颅内血管" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "颅内、外血管的扩张" }, { "start_idx": 43, "end_idx": 56, "type": "sym", "entity": "血管周围组织产生血管活性多肽" }, { "start_idx": 60, "end_idx": 69, "type": "sym", "entity": "无菌性炎症而诱发头痛" } ] }, { "text": "20世纪90年代Olsen进一步发展了血管源学说,提出有先兆和没有先兆的偏头痛是血管痉挛程度不同的同一疾病。", "entities": [ { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "偏头痛" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "血管痉挛" } ] }, { "text": "(三)神经源学说认为偏头痛时神经功能变化是首要的,血流量的变化是继发的。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "偏头痛" } ] }, { "text": "1.神经递质假说5-HT在偏头痛的发病中具有重要作用,它可使血管壁产生无菌性炎症或通过受体使脑血管收缩导致局部脑血流下降引起头痛。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "偏头痛" }, { "start_idx": 30, "end_idx": 39, "type": "sym", "entity": "血管壁产生无菌性炎症" }, { "start_idx": 53, "end_idx": 63, "type": "sym", "entity": "局部脑血流下降引起头痛" } ] }, { "text": "β-内啡肽、甲硫脑啡肽、P物质、儿茶酚胺、组织胺、血管活性肽和前列环素等神经递质,亦与偏头痛的发生有关。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "β-内啡肽" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "甲硫脑啡肽" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "P物质" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "儿茶酚胺" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "组织胺" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "血管活性肽" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "前列环素" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "偏头痛" } ] }, { "text": "2.扩散性抑制假说是指各种因素刺激大脑皮层后出现的由刺激部位向周围组织呈波浪式扩展的皮层电活动抑制。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "大脑皮层" } ] }, { "text": "这种抑制以波的形式非常缓慢地通过皮质区,皮层扩散性抑制伴有明显的大脑血流减少(持续2~6个小时)。", "entities": [ { "start_idx": 29, "end_idx": 37, "type": "sym", "entity": "明显的大脑血流减少" } ] }, { "text": "此假说可以充分解释偏头痛发作的神经功能障碍,但不能成功地解释头痛。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "偏头痛" } ] }, { "text": "(四)三叉神经血管反射学说是指三叉神经传入纤维末梢释放P物质及其他神经递质,传出神经作用于颅内外血管,引起头痛和血管扩张。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "三叉神经血管" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "三叉神经" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "纤维末梢" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "P物质" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "颅内外血管" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "头痛" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "血管扩张" } ] }, { "text": "偏头痛作为一种不稳定的三叉神经-血管反射,伴有疼痛控制通路中的节段性缺陷,使得从三叉神经脊核来的过量冲动发放以及对三叉丘脑束或皮质延髓束来的过量传入冲动发生应答,最终引起脑干与颅内血管发生相互作用。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "三叉神经-血管" }, { "start_idx": 23, "end_idx": 35, "type": "sym", "entity": "疼痛控制通路中的节段性缺陷" }, { "start_idx": 40, "end_idx": 45, "type": "bod", "entity": "三叉神经脊核" }, { "start_idx": 57, "end_idx": 61, "type": "bod", "entity": "三叉丘脑束" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "皮质延髓束" }, { "start_idx": 85, "end_idx": 86, "type": "bod", "entity": "脑干" }, { "start_idx": 88, "end_idx": 91, "type": "bod", "entity": "颅内血管" } ] }, { "text": "(五)其他学说有关偏头痛发病机制尚有低镁学说、高钾诱导血管痉挛学说、自主神经功能紊乱学说及大脑细胞电流紊乱学说等。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "偏头痛" } ] }, { "text": "【临床表现】小儿偏头痛的临床表现与成人基本相似,但与成人比较又有许多不同之处,小儿偏头痛发作时的症状不如成人鲜明,但胃肠道症状非常突出。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "小儿偏头痛" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "小儿偏头痛" } ] }, { "text": "(一)有先兆的偏头痛旧称经典型偏头痛,多数患儿先兆先于头痛发作,少数与头痛同时发作,偶尔在头痛后发作,个别病例只有先兆而没有偏头痛发作。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "有先兆的偏头痛" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "经典型偏头痛" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "头痛" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "头痛" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "偏头痛" } ] }, { "text": "先兆以视觉症状最常见,如眼前出现不同形状的闪烁暗点、“冒金星”、城垛样闪光、视物模糊不清、偏盲以及黑蒙等,亦可出现视幻觉和视物变形或变色,持续数分钟至数小时。", "entities": [ { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "出现不同形状的闪烁暗点" }, { "start_idx": 27, "end_idx": 29, "type": "sym", "entity": "冒金星" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "城垛样闪光" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "视物模糊不清" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "偏盲" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "黑蒙" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "幻觉" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "视物变形" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "变色" }, { "start_idx": 69, "end_idx": 77, "type": "sym", "entity": "持续数分钟至数小时" } ] }, { "text": "头痛发作往往开始于一侧额颞部、眶上或眶后,偶尔出现在顶部或枕部,呈搏动性(跳痛)或胀痛,可扩展到半侧头部或全头部,亦有左右交替发作者。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "额颞部" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "眶上" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "眶后" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "顶部" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "枕部" }, { "start_idx": 33, "end_idx": 42, "type": "sym", "entity": "搏动性(跳痛)或胀痛" }, { "start_idx": 45, "end_idx": 55, "type": "sym", "entity": "扩展到半侧头部或全头部" }, { "start_idx": 59, "end_idx": 64, "type": "sym", "entity": "左右交替发作" } ] }, { "text": "头痛时有伴随症状,如恶心、呕吐、腹痛及面色苍白等。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "恶心" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "呕吐" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "腹痛" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "面色苍白" } ] }, { "text": "(二)没有先兆的偏头痛旧称普通型偏头痛,最常见,是青春期前儿童最常见的头痛发作形式。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "偏头痛" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "普通型偏头痛" } ] }, { "text": "常为双侧额或颞部疼痛,大约一半患儿头痛性质为搏动性,头痛程度比经典型偏头痛轻,持续时间0.5~2小时。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "双侧额或颞部疼痛" } ] }, { "text": "(三)特殊类型的偏头痛1.偏瘫型偏头痛头痛开始或头痛不久出现头痛对侧肢体瘫痪,可伴有瘫痪肢体麻木,持续时间长时可致瘫痪肢体抽搐。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "偏头痛" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "偏瘫型偏头痛" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "头痛" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "瘫痪肢体麻木" }, { "start_idx": 49, "end_idx": 62, "type": "sym", "entity": "持续时间长时可致瘫痪肢体抽搐" } ] }, { "text": "偏瘫一般较轻,持续数小时至1~2日,重者可达数日,一般均能完全恢复。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "偏瘫一般较轻" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "持续数小时至1~2日" } ] }, { "text": "2.基底动脉型偏头痛多见于儿童(女孩多于男孩)或年轻女性。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "基底动脉型偏头痛" } ] }, { "text": "先兆症状多持续数分钟或数十分钟,而后出现枕部搏动性疼痛,常伴恶心和呕吐,发作持续数小时。", "entities": [ { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "枕部搏动性疼痛" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "恶心" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "呕吐" }, { "start_idx": 36, "end_idx": 42, "type": "sym", "entity": "发作持续数小时" } ] }, { "text": "有时头痛也可先出现或与诸多神经症状同时发生。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "头痛" } ] }, { "text": "3.眼肌瘫痪型偏头痛多在12岁以前发病,有时见于婴幼儿。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "眼肌瘫痪型偏头痛" } ] }, { "text": "眼眶部疼痛伴有动眼神经完全性或不全性麻痹,部分病例同时累及滑车和展神经,出现眼球运动障碍。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "眼眶部疼痛" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "动眼神经完全性" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "不全性麻痹" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "眼球运动障碍" } ] }, { "text": "眼肌瘫痪可在头痛前或后或同时发生,以上眼睑下垂最常见,重者眼外肌全部瘫痪,伴瞳孔散大,眼球固定,光反应消失。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "眼肌瘫痪" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "眼外肌全部瘫痪" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "瞳孔散大" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "眼球固定" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "光反应消失" } ] }, { "text": "疼痛可持续数小时,眼肌瘫痪可持续数日至数周。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "疼痛可持续数小时" }, { "start_idx": 9, "end_idx": 20, "type": "sym", "entity": "眼肌瘫痪可持续数日至数周" } ] }, { "text": "4.可能为偏头痛先驱或与偏头痛有关的周期性综合征即过去所称的偏头痛等位症,是指临床出现短暂性神经功能障碍而当时头痛只是次要症状,甚至不出现头痛的一组综合征。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "偏头痛" }, { "start_idx": 43, "end_idx": 51, "type": "sym", "entity": "短暂性神经功能障碍" } ] }, { "text": "【诊断】关于偏头痛的诊断,目前还没有一个客观的生物学指标,主要根据临床症状及阳性家族史加以诊断。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "偏头痛" } ] }, { "text": "至于辅助检查对偏头痛的诊断是不必要的,其价值在于排除非偏头痛疾病。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "辅助检查" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "偏头痛" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "非偏头痛" } ] }, { "text": "2004年IHS制定了没有先兆的偏头痛诊断标准是至少有5次发作符合下列条件:①小儿头痛发作持续1~72小时;②头痛至少具有下列4项中的2项特点:a.单侧头痛,b.搏动性头痛,c.中度或重度头痛,影响日常生活,d.日常体力活动(如上楼梯)时头痛加重;③头痛时至少有下列2项中的1项表现:a.恶心和(或)呕吐,b.畏光及畏声;④病史、体检及各项检查未发现全身或中枢神经系统器质性疾病,如有其他疾病需有证据说明与头痛发作无关。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "偏头痛" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "小儿头痛" }, { "start_idx": 45, "end_idx": 52, "type": "sym", "entity": "持续1~72小时" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "单侧头痛" }, { "start_idx": 81, "end_idx": 85, "type": "sym", "entity": "搏动性头痛" }, { "start_idx": 89, "end_idx": 95, "type": "sym", "entity": "中度或重度头痛" }, { "start_idx": 119, "end_idx": 122, "type": "sym", "entity": "头痛加重" }, { "start_idx": 144, "end_idx": 145, "type": "sym", "entity": "恶心" }, { "start_idx": 150, "end_idx": 151, "type": "sym", "entity": "呕吐" }, { "start_idx": 155, "end_idx": 156, "type": "sym", "entity": "畏光" }, { "start_idx": 158, "end_idx": 159, "type": "sym", "entity": "畏声" }, { "start_idx": 178, "end_idx": 188, "type": "dis", "entity": "中枢神经系统器质性疾病" }, { "start_idx": 203, "end_idx": 204, "type": "dis", "entity": "头痛" } ] }, { "text": "有先兆的偏头痛诊断标准是:a.符合以下b~d特点的发作≥2次。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "偏头痛" } ] }, { "text": "c.至少符合以下两条:①至少1种先兆症状逐渐发展时间≥5分钟和(或)不同先兆症状接连出现≥5分钟;②先兆症状持续时间5~60分钟;③视觉症状和(或)感觉症状。", "entities": [ { "start_idx": 16, "end_idx": 29, "type": "sym", "entity": "先兆症状逐渐发展时间≥5分钟" }, { "start_idx": 34, "end_idx": 47, "type": "sym", "entity": "不同先兆症状接连出现≥5分钟" }, { "start_idx": 50, "end_idx": 63, "type": "sym", "entity": "先兆症状持续时间5~60分钟" }, { "start_idx": 66, "end_idx": 69, "type": "sym", "entity": "视觉症状" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "感觉症状" } ] }, { "text": "此外,病史、体检及各项检查应未发现全身或中枢神经系统器质性疾病,如果有其他疾病需有证据说明与头痛发作无关。", "entities": [ { "start_idx": 20, "end_idx": 25, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "HIS制定的偏头痛诊断标准过于繁琐及严格,不便于临床工作时应用。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "偏头痛" } ] }, { "text": "并且此诊断标准是面对整个人群的,由于小儿偏头痛的症状主诉、发作方式与成人不尽相同(比如小儿的发作时间较短,单侧性和畏声在小儿较少见),故有不少针对儿童的修改性意见。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "小儿偏头痛" }, { "start_idx": 43, "end_idx": 51, "type": "sym", "entity": "小儿的发作时间较短" }, { "start_idx": 53, "end_idx": 55, "type": "sym", "entity": "单侧性" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "畏声" } ] }, { "text": "比较认同的有以下几点:①发作头痛时伴有腹痛、恶心或呕吐;②偏侧头痛;③头痛性质呈跳动或搏动性、刺痛性;④经短暂时间后能完全缓解;⑤有视觉、感觉或运动性先兆;⑥在一级亲属中有一个或更多成员有头痛史。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "腹痛" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "恶心" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "呕吐" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "偏侧头痛" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "跳动" }, { "start_idx": 43, "end_idx": 45, "type": "sym", "entity": "搏动性" }, { "start_idx": 47, "end_idx": 49, "type": "sym", "entity": "刺痛性" }, { "start_idx": 66, "end_idx": 76, "type": "sym", "entity": "视觉、感觉或运动性先兆" } ] }, { "text": "头痛特征如具有以上几项中之三项以上,则较支持偏头痛的诊断。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "头痛" } ] }, { "text": "迄今尚无一致公认的偏头痛诊断标准,但以下几点受到普遍的赞同:①反复发作性的头痛,间歇期完全正常,排除其他器质性疾病引起的头痛;②具备以下6条中的3条:a.头痛发作时伴有恶心、呕吐,头痛时或不头痛时有发作性腹痛;b.偏侧头痛;c.搏动性头痛;d.短期休息或睡眠后缓解;e.有视觉异常等先兆;f.有偏头痛家族史。", "entities": [ { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "反复发作性的头痛" }, { "start_idx": 84, "end_idx": 85, "type": "sym", "entity": "恶心" }, { "start_idx": 87, "end_idx": 88, "type": "sym", "entity": "呕吐" }, { "start_idx": 90, "end_idx": 91, "type": "sym", "entity": "头痛" }, { "start_idx": 99, "end_idx": 103, "type": "sym", "entity": "发作性腹痛" }, { "start_idx": 107, "end_idx": 110, "type": "sym", "entity": "偏侧头痛" }, { "start_idx": 114, "end_idx": 118, "type": "sym", "entity": "搏动性头痛" }, { "start_idx": 122, "end_idx": 125, "type": "pro", "entity": "短期休息" }, { "start_idx": 127, "end_idx": 131, "type": "pro", "entity": "睡眠后缓解" }, { "start_idx": 147, "end_idx": 149, "type": "dis", "entity": "偏头痛" } ] }, { "text": "这比较符合Prensky提出的小儿偏头痛诊断标准。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "小儿偏头痛" } ] }, { "text": "伴恶心、呕吐者可用甲氧氯普胺或氯丙嗪;伴眩晕或头晕者可用地芬尼多或东莨菪碱等治疗。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "恶心" }, { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "甲氧氯普胺" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "氯丙嗪" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "眩晕" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "地芬尼多" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "东莨菪碱" } ] }, { "text": "1.解热镇痛剂常用对乙酰氨基酚(paracetamol)每次10~15mg/kg、阿司匹林(aspirin)每次10~15mg/kg、布洛芬(ibuprofen)每次5~10mg/kg以及奈普生(naproxen)每次5~10mg/kg等,在头痛早期服用有效。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "对乙酰氨基酚" }, { "start_idx": 41, "end_idx": 44, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 67, "end_idx": 69, "type": "dru", "entity": "布洛芬" }, { "start_idx": 94, "end_idx": 96, "type": "dru", "entity": "奈普生" } ] }, { "text": "2.麦角胺制剂如麦角胺及双氢麦角胺等,对颅外动脉有收缩作用。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "麦角胺制剂" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "麦角胺" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "双氢麦角胺" } ] }, { "text": "常用的复方片剂为麦角胺咖啡因(每片含麦角胺1mg和咖啡因100mg),学龄儿童用量每次1片,对终止头痛发作有效。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dru", "entity": "麦角胺咖啡因" } ] }, { "text": "3.曲普坦类药物如舒马曲坦(sumatriptan),是一种选择性5-羟色胺受体激动剂,具有高度选择性地收缩颈动脉作用,为治疗偏头痛急性发作有效而昂贵的药物。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "舒马曲坦" } ] }, { "text": "(二)预防性治疗偏头痛的预防第一步就是要消除或减少发作的诱发因素,如避免情绪紧张、劳累、睡眠不足、声及光刺激,不进食含奶酪食物等。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "偏头痛" }, { "start_idx": 34, "end_idx": 47, "type": "pro", "entity": "避免情绪紧张、劳累、睡眠不足" }, { "start_idx": 49, "end_idx": 53, "type": "pro", "entity": "声及光刺激" }, { "start_idx": 55, "end_idx": 62, "type": "pro", "entity": "不进食含奶酪食物" } ] }, { "text": "1.β受体阻断剂常用普萘洛尔(propranolol),剂量为2mg/(kg•d),分3次口服。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "β受体阻断剂" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "普萘洛尔" } ] }, { "text": "为防止低血压及心率减慢副作用发生,应从小量0.5~1mg/(kg•d)开始,缓慢加量直至可以耐受。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "低血压" } ] }, { "text": "2.组胺受体阻断剂常用赛庚啶(cyprohepladine),剂量为0.2~0.4mg/(kg•d),疗程6~12个月或更长。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "赛庚啶" } ] }, { "text": "3.5-羟色胺受体阻断剂常用苯噻啶(pizolifen),兼有组胺受体拮抗作用。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "苯噻啶" } ] }, { "text": "4.钙通道阻滞剂常用氟桂利嗪(flunarizine),剂量为每晚睡前服2.5~5mg,一般疗程2~3个月。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "氟桂利嗪" } ] }, { "text": "5.其他药物丙戊酸(valproicacid),卡马西平(carbamazepine),托吡酯(topiramate),可乐定(clonidine),苯乙肼(phenelzine),阿米替林(amitriptyline)等。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "丙戊酸" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "卡马西平" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "托吡酯" }, { "start_idx": 60, "end_idx": 62, "type": "dru", "entity": "可乐定" }, { "start_idx": 75, "end_idx": 77, "type": "dru", "entity": "苯乙肼" }, { "start_idx": 91, "end_idx": 94, "type": "dru", "entity": "阿米替林" } ] }, { "text": "【预后】偏头痛病程较长,但预后良好。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "偏头痛" } ] }, { "text": "近年有报道小儿偏头痛发作过程中有时并发脑梗死,被称为偏头痛脑卒中(migrainousstroke),其机制不明,可能与血小板聚集后的微血栓形成有关。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "小儿偏头痛" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "脑梗死" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "偏头痛脑卒" }, { "start_idx": 33, "end_idx": 48, "type": "dis", "entity": "migrainousstroke" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "血小板" } ] }, { "text": "五、混合性酸碱平衡紊乱当有两种或以上的酸碱紊乱分别同时作用于呼吸或代谢系统称为混合性酸碱平衡紊乱(mixeddisorders)。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "混合性酸碱平衡紊乱" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "酸碱紊乱" }, { "start_idx": 30, "end_idx": 36, "type": "bod", "entity": "呼吸或代谢系统" }, { "start_idx": 39, "end_idx": 47, "type": "dis", "entity": "混合性酸碱平衡紊乱" }, { "start_idx": 49, "end_idx": 62, "type": "dis", "entity": "mixeddisorders" } ] }, { "text": "当代偿能力在预计范围之外时,就应考虑存在混合性酸碱平衡紊乱(表2-8)。", "entities": [ { "start_idx": 20, "end_idx": 28, "type": "dis", "entity": "混合性酸碱平衡紊乱" } ] }, { "text": "例如糖尿病酮症酸中毒病人同时存在肺气肿,呼吸窘迫综合征(respiratorydistresssyndrome,RDS)病人有呼吸性酸中毒与代谢性酸中毒同时存在时。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "糖尿病酮症酸中毒" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "肺气肿" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "呼吸窘迫综合征" }, { "start_idx": 28, "end_idx": 54, "type": "dis", "entity": "respiratorydistresssyndrome" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "RDS" }, { "start_idx": 63, "end_idx": 68, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 70, "end_idx": 75, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "呼吸系统本身的疾病存在阻碍了降低PaCO2的代偿机制,结果使pH下降显著慢性呼吸性酸中毒伴有充血性心力衰竭时,如过度使用利尿剂可出现代谢性碱中毒,此时血浆水平和pH将高于单纯的慢性呼吸性酸中毒。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 16, "end_idx": 20, "type": "ite", "entity": "PaCO2" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "pH" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "使pH下降显著" }, { "start_idx": 42, "end_idx": 49, "type": "dis", "entity": "慢性呼吸性酸中毒" }, { "start_idx": 52, "end_idx": 58, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 66, "end_idx": 68, "type": "dru", "entity": "利尿剂" }, { "start_idx": 72, "end_idx": 77, "type": "dis", "entity": "代谢性碱中毒" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "血浆" }, { "start_idx": 86, "end_idx": 87, "type": "ite", "entity": "pH" }, { "start_idx": 94, "end_idx": 101, "type": "dis", "entity": "慢性呼吸性酸中毒" } ] }, { "text": "肝功能衰竭时可出现代谢性酸中毒与呼吸性碱中毒,此时pH可能变化不大,但血浆和PaCO2显著降低。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肝功能衰竭" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "呼吸性碱中毒" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "pH" }, { "start_idx": 38, "end_idx": 42, "type": "ite", "entity": "PaCO2" }, { "start_idx": 25, "end_idx": 52, "type": "sym", "entity": "pH可能变化不大,但血浆和PaCO2显著降低" } ] }, { "text": "第三章心血管系统疾病的诊疗技术第一节胸部X线检查过去,胸部X线检查需结合详细病史,体格检查和心电图才能对先心病做出可能的诊断。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "心血管系统疾病" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "体格检查" }, { "start_idx": 46, "end_idx": 48, "type": "pro", "entity": "心电图" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "先心病" } ] }, { "text": "毫无疑问,如今胸部X线检查的作用愈不如前。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "胸部X线检查" } ] }, { "text": "超声心动图技术的迅速发展为先心病诊断提供了一条快捷准确的诊断方式,二维超声心动图可显示心内的解剖结构,体循环和肺静脉连接,大血管关系和瓣膜异常,使先心病的诊断较前更加便捷准确。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "超声心动图" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "先心病" }, { "start_idx": 33, "end_idx": 39, "type": "pro", "entity": "二维超声心动图" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "心内" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "肺静脉" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "大血管" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "瓣膜" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "先心病" } ] }, { "text": "同时由于超声心动图的广泛应用,先心病得以早期发现,年长儿的典型X线表现已不多见。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "超声心动图" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "先心病" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "X线" } ] }, { "text": "尽管非侵入性诊断方法的应用日渐增多,X线作为一种价廉的影像技术,在先心病的筛查方面仍然起着重要的作用。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "X线" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "先心病" } ] }, { "text": "而且,胸片X线检查可提供心脏的血流动力学(如左向右分流量、青紫型先心病的肺血流多少)、充血性心力衰竭时心脏扩大的随访及手术后及经导管介入治疗效果等一系列信息。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "胸片X线检查" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "心脏" }, { "start_idx": 22, "end_idx": 27, "type": "ite", "entity": "左向右分流量" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "青紫型先心病" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "肺血流" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "心脏" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "手术" }, { "start_idx": 63, "end_idx": 69, "type": "pro", "entity": "经导管介入治疗" } ] }, { "text": "(一)胸片分析步骤胸部后前位是常规的摄片位,一般不用加拍侧位片,斜位片目前已少用。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "胸片" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胸部" } ] }, { "text": "摄片时宜取吸气状态,如呼气相摄片可能易误认为有心脏肥大或肺充血。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "心脏肥大" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "肺充血" } ] }, { "text": "如果膈肌位于第9、10后肋水平,说明摄片时处于理想的吸气状态。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "膈肌" } ] }, { "text": "当分析正位片时,应将心脏大小、心缘影与肺血管影结合起来分析,还应注意脊柱和肋骨有无异常,内脏位置,尤其是胃泡及肝脏的位置应与心脏的位置一起分析。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心脏" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "肺血管" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "脊柱" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "肋骨" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "胃泡" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "肝脏" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "心脏" } ] }, { "text": "(二)心脏大小判断一般通过正位片观察心脏大小。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "心脏" } ] }, { "text": "影响心脏大小的因素很多,吸气相或仰卧位时心影增大,婴儿和幼儿的正常胸腺可能被误诊为心脏增大。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心脏" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "胸腺" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "心脏增大" } ] }, { "text": "胸腺可向下扩展,占据胸骨后间隙,与心影重叠。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胸腺" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "胸骨" } ] }, { "text": "增大的胸腺如位于上纵隔可形成类似于心上型肺静脉异常连接的“雪人形”心影,二维超声可明确诊断。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "胸腺" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "上纵隔" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "心上型肺静脉" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "二维超声" } ] }, { "text": "心脏大小由心胸比率来定量,心胸比率即心脏最大横径与右膈最高点水平的胸廓内径之比。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心脏" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "心脏" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "右膈" } ] }, { "text": "如在成人或年长儿心胸比例超过0.5,婴儿超过0.6即为心脏增大。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "心脏增大" } ] }, { "text": "绝大多数的先心病伴有心脏扩大。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "先心病" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "心脏扩大" } ] }, { "text": "明确有心脏增大后,仔细分析心缘各段变化可确定扩大的腔室。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "心脏增大" } ] }, { "text": "(三)心脏外形1.右心房右房增大时,右心缘弧度增大,向右肺野突出,单纯的右房增大不多见,典型征象见于Ebstein畸形,在新生儿期即可出现明显的右房增大(图9-9)。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "右心房" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "右房" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "右房增大时" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "右心缘弧度增大" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "向右肺野突出" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "右房" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "右房增大" }, { "start_idx": 50, "end_idx": 58, "type": "dis", "entity": "Ebstein畸形" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "右房" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "右房增大" } ] }, { "text": "图9-1Ebstein畸形(正位片)示:右心房扩大可见右心缘膨出,肺血管纹理减少2.右心室右心室在正位片时不构成左下心缘,轻度的右室肥大或扩大,心影变化不明显,如右室继续增大可导致心尖向侧、上移位,当心影扩大不明显而右心室肥厚时,心尖上翘明显,侧位片心脏前缘向胸骨方向扩大。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "dis", "entity": "Ebstein畸形" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "右心房" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "右心房扩大" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "右心缘膨出" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "肺血管" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "肺血管纹理减少" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "右心室" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "右心室" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "右室" }, { "start_idx": 61, "end_idx": 70, "type": "sym", "entity": "轻度的右室肥大或扩大" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "右室" }, { "start_idx": 81, "end_idx": 97, "type": "sym", "entity": "右室继续增大可导致心尖向侧、上移位" }, { "start_idx": 108, "end_idx": 112, "type": "sym", "entity": "右心室肥厚" }, { "start_idx": 115, "end_idx": 120, "type": "sym", "entity": "心尖上翘明显" }, { "start_idx": 122, "end_idx": 135, "type": "sym", "entity": "侧位片心脏前缘向胸骨方向扩大" } ] }, { "text": "3.左心房在正位片,当左房扩大时,显示左心缘位于肺动脉干影下方有一小的膨出,如胶片透光良好,可显示双房影。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "左心房" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "左房" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "左心缘" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肺动脉" } ] }, { "text": "左房增大明显时,左主支气管受压上抬,隆凸角度增大,侧位片可见左主支气管移位。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "左房" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "左主支气管" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "左主支气管" } ] }, { "text": "4.左心室当左室扩张时,心尖向侧下方移位,而右室扩大时心尖上翘。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "左心室" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "左室" }, { "start_idx": 5, "end_idx": 19, "type": "sym", "entity": "当左室扩张时,心尖向侧下方移位" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "右室" }, { "start_idx": 22, "end_idx": 30, "type": "sym", "entity": "右室扩大时心尖上翘" } ] }, { "text": "左室肥大时,心影变化不明显。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "左室" } ] }, { "text": "(四)肺血管评估肺血管状况需要有高质量的胸片及丰富的经验。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肺血管" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肺血管" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "胸片" } ] }, { "text": "过度曝光会低估肺血供;在新生儿,如曝光过度尚可能遮盖中等程度左向右分流导致的肺血增多的表现。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肺" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "肺血增多" } ] }, { "text": "由于心脏病变引起的左向右分流造成肺血流增多时,心腔因容纳额外的血液而扩大。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "心脏病变" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "肺血流增多" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心腔" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "血液" } ] }, { "text": "在左向右分流先心病,如肺动脉血流量与主动脉血流量之比小于2∶1,肺血管影可正常。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "左向右分流先心病" }, { "start_idx": 11, "end_idx": 16, "type": "ite", "entity": "肺动脉血流量" }, { "start_idx": 18, "end_idx": 23, "type": "ite", "entity": "主动脉血流量" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "肺血管影" } ] }, { "text": "若肺血增加而心影正常则可能是肺静脉淤血;若肺血正常或减少而心影增大,应考虑为左向右分流之外的其他病变。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "肺血" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "心影" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肺静脉淤血" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肺血" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "心影" } ] }, { "text": "因此,肺血是否增多,需与心脏病变相联系,还应考虑众多背景因素包括年龄、呼吸深浅、X线的透光度。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "肺血" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "心脏病变" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "X线" } ] }, { "text": "1.非发绀型先心病肺充血如前所述,肺循环体循环血流比小于2∶1时肺血可正常。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "非发绀型先心病肺充血" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肺血" } ] }, { "text": "如肺血流量继续增加,肺血管扩张,则肺血管影向肺外带延伸(图9-9);在肺动脉血流增加的同时,肺静脉血回流至左心房亦增加,导致左心房扩大。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "肺血" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺血管" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肺血管" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肺外带" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "肺动脉血流" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "肺静脉血" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "左心房" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "左心房" }, { "start_idx": 62, "end_idx": 66, "type": "sym", "entity": "左心房扩大" } ] }, { "text": "由于心腔接纳大量的额外血流,心脏常扩大且X线可显示心影增大。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心腔" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血流" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心脏" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "X线" } ] }, { "text": "常见于:房间隔缺损(ASD)、室间隔缺损(VSD)、房室间隔缺损、动脉导管未闭(PDA);左心房、左心室增大见于大的VSD、房室间隔缺损或ASD;右心房、右心室增大见于大的ASD。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "ASD" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "VSD" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "房室间隔缺损" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "PDA" }, { "start_idx": 45, "end_idx": 53, "type": "sym", "entity": "左心房、左心室增大" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "VSD" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "房室间隔缺损" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "ASD" }, { "start_idx": 73, "end_idx": 81, "type": "sym", "entity": "右心房、右心室增大" }, { "start_idx": 86, "end_idx": 88, "type": "dis", "entity": "ASD" } ] }, { "text": "如大量左向右分流逐渐减,即为肺血管病变逐渐进展而致肺血管逐渐减少,扩张的肺血管影及外周肺血管影均减少。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肺血管病变" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "肺血管逐渐减少" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "扩张的肺血管影" }, { "start_idx": 41, "end_idx": 49, "type": "sym", "entity": "外周肺血管影均减少" } ] }, { "text": "图9-2房室间隔缺损(正位片)示:高肺动脉血流量导致肺纹理增多和心脏扩大2.发绀型肺血增多肺血增多和心脏肥大也见于发绀型先天性心脏病(congenitalheartdisease),见于完全性大动脉转位(transpositionofthegreatarteries)、共同通道畸形(无梗阻性完全性肺静脉异位连接、心室双入口不伴肺动脉狭窄及永存动脉干)、右向左分流伴肺血流增加(四联症伴肺动脉闭锁(pulmonaryatresia)合并大型的主肺动脉侧支或分流形成)。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "房室间隔缺损" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "高肺动脉血流量" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "肺纹理增多" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "发绀型肺血增多" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "肺血增多" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "心脏肥大" }, { "start_idx": 57, "end_idx": 65, "type": "dis", "entity": "发绀型先天性心脏病" }, { "start_idx": 67, "end_idx": 88, "type": "dis", "entity": "congenitalheartdisease" }, { "start_idx": 93, "end_idx": 100, "type": "dis", "entity": "完全性大动脉转位" }, { "start_idx": 102, "end_idx": 132, "type": "dis", "entity": "transpositionofthegreatarteries" }, { "start_idx": 135, "end_idx": 140, "type": "dis", "entity": "共同通道畸形" }, { "start_idx": 142, "end_idx": 155, "type": "dis", "entity": "无梗阻性完全性肺静脉异位连接" }, { "start_idx": 157, "end_idx": 168, "type": "dis", "entity": "心室双入口不伴肺动脉狭窄" }, { "start_idx": 170, "end_idx": 174, "type": "dis", "entity": "永存动脉干" }, { "start_idx": 177, "end_idx": 187, "type": "dis", "entity": "右向左分流伴肺血流增加" }, { "start_idx": 189, "end_idx": 197, "type": "dis", "entity": "四联症伴肺动脉闭锁" }, { "start_idx": 199, "end_idx": 214, "type": "dis", "entity": "pulmonaryatresia" }, { "start_idx": 221, "end_idx": 226, "type": "bod", "entity": "主肺动脉侧支" }, { "start_idx": 228, "end_idx": 229, "type": "bod", "entity": "分流" } ] }, { "text": "3.发绀性肺血减少心影正常或稍增大伴有右向左分流所致的肺血减少,与先天性心脏病肺动脉狭窄和梗阻有关。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "发绀性肺血减少" }, { "start_idx": 33, "end_idx": 43, "type": "dis", "entity": "先天性心脏病肺动脉狭窄" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "梗阻" } ] }, { "text": "肺血减少程度与肺血流量有关,后者与肺动脉狭窄程度或导管依赖性病变的动脉导管大小有关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺血" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "肺血流量" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肺动脉" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "导管" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "动脉导管" } ] }, { "text": "胸片表现为肺部过度充气。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "胸片" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肺部" } ] }, { "text": "典型病变有:法洛四联症伴或不伴肺动脉闭锁、室间隔完整型肺动脉闭锁(图9-9)、Ebstein畸形、三尖瓣闭锁(tricuspidatresia)伴肺动脉闭锁或狭窄。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "肺动脉闭锁" }, { "start_idx": 21, "end_idx": 31, "type": "dis", "entity": "室间隔完整型肺动脉闭锁" }, { "start_idx": 39, "end_idx": 47, "type": "dis", "entity": "Ebstein畸形" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "三尖瓣闭锁" }, { "start_idx": 55, "end_idx": 70, "type": "dis", "entity": "tricuspidatresia" }, { "start_idx": 73, "end_idx": 80, "type": "dis", "entity": "肺动脉闭锁或狭窄" } ] }, { "text": "法洛四联症心影呈靴形,心尖上翘(图9-4)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "心影呈靴形,心尖上翘" } ] }, { "text": "Ebstein畸形则表现为心影明显扩大。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "心影明显扩大" } ] }, { "text": "4.肺静脉淤血肺静脉淤血伴肺动脉高压在儿童并不多见。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肺静脉淤血" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "肺静脉淤血" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "肺动脉高压" } ] }, { "text": "见于肺静脉异位连接、三房心、二尖瓣狭窄(mitralstenosis)或限制型心房间交通伴二尖瓣闭锁。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "肺静脉异位连接" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "三房心" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "二尖瓣狭窄" }, { "start_idx": 20, "end_idx": 33, "type": "dis", "entity": "mitralstenosis" }, { "start_idx": 36, "end_idx": 49, "type": "dis", "entity": "限制型心房间交通伴二尖瓣闭锁" } ] }, { "text": "肺动脉毛细血管压超过12mmHg时,上下叶血管影相当或上叶略有增多,压力超过15mmHg时,可见肺间质水肿,位于外周的肺叶间隙水肿增厚,显示为KerleyB线,即从可见在肺下野两侧直达肺脏边缘的水平线,肺血管模糊不清。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "肺动脉毛细血管压" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "上下叶血管" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "上叶" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "肺间质水肿" }, { "start_idx": 54, "end_idx": 66, "type": "sym", "entity": "位于外周的肺叶间隙水肿增厚" }, { "start_idx": 85, "end_idx": 89, "type": "bod", "entity": "肺下野两侧" }, { "start_idx": 101, "end_idx": 103, "type": "bod", "entity": "肺血管" } ] }, { "text": "肺门部的肺叶间隙增厚,分散在中野或上野的KerleyA线也较明显。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "肺门部的肺叶间隙增厚" } ] }, { "text": "肺毛细血管压超过25mmHg时,肺泡水肿形成蝶翼征。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "肺毛细血管压" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肺泡" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "肺泡水肿形成蝶翼征" } ] }, { "text": "该阴影在双侧肺门区域最明显,对称性向外逐渐消散(图9-9)。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肺门" } ] }, { "text": "图9-3室间隔完整型的肺动脉闭锁(正位片)示:肺血流量减少,肺野缺血图9-4法洛四联症(正位片)示:心脏呈典型的“靴形心”,心尖上翘,肺动脉影凹陷和右位主动脉弓(五)心血管结构异常判别1.水肿本病在较大儿童和青年伴心脏肥大者少见。", "entities": [ { "start_idx": 4, "end_idx": 15, "type": "dis", "entity": "室间隔完整型的肺动脉闭锁" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "肺血流量减少" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "肺野缺血" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 50, "end_idx": 60, "type": "sym", "entity": "心脏呈典型的“靴形心”" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "心尖上翘" }, { "start_idx": 67, "end_idx": 79, "type": "sym", "entity": "肺动脉影凹陷和右位主动脉弓" }, { "start_idx": 83, "end_idx": 89, "type": "dis", "entity": "心血管结构异常" }, { "start_idx": 94, "end_idx": 95, "type": "dis", "entity": "水肿" }, { "start_idx": 107, "end_idx": 110, "type": "dis", "entity": "心脏肥大" } ] }, { "text": "有时因左锁骨下动脉扩张呈现狭窄部近端动脉扩张及降主动脉狭窄后扩张。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "左锁骨下动脉" }, { "start_idx": 0, "end_idx": 31, "type": "sym", "entity": "有时因左锁骨下动脉扩张呈现狭窄部近端动脉扩张及降主动脉狭窄后扩张" } ] }, { "text": "双侧肋骨切迹通常位于第3、4后肋下缘,与肋间动脉侧支血管形成有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "双侧肋骨" }, { "start_idx": 20, "end_idx": 27, "type": "bod", "entity": "肋间动脉侧支血管" } ] }, { "text": "2.右位主动脉弓(aorticarch)即主动脉弓上升于右支气管前,在气管的右侧形成弓后,向下走行。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "右位主动脉弓" }, { "start_idx": 9, "end_idx": 18, "type": "dis", "entity": "aorticarch" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "右支气管" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "气管" }, { "start_idx": 21, "end_idx": 48, "type": "sym", "entity": "主动脉弓上升于右支气管前,在气管的右侧形成弓后,向下走行" } ] }, { "text": "后者在先心病中较少见,但本病与法洛四联症、永存动脉干和大动脉转位均有密切关系。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "先心病" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "永存动脉干" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "大动脉转位" } ] }, { "text": "3.肺动脉瓣狭窄可见肺总动脉和左肺动脉扩张,其余肺动脉段发育正常,肺动脉扩张与狭窄后扩张有关。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "肺总动脉" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 10, "end_idx": 20, "type": "sym", "entity": "肺总动脉和左肺动脉扩张" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "其余肺动脉段" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "肺动脉" } ] }, { "text": "(六)骨骼异常肋骨畸形、脊柱侧突和椎骨的异常如脊椎畸形,均与先心病有关。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "骨骼异常" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "肋骨畸形" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "脊柱侧突" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "椎骨的异常" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "脊椎畸形" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "先心病" } ] }, { "text": "脊柱侧突和漏斗胸见于二尖瓣脱垂、马方综合征;蝶形椎体见于阿拉吉尔综合征(Alagillesyndrome)伴外周肺动脉狭窄。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "脊柱侧突" }, { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "漏斗胸" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "二尖瓣脱垂" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "马方综合征" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "阿拉吉尔综合征" }, { "start_idx": 36, "end_idx": 51, "type": "dis", "entity": "Alagillesyndrome" }, { "start_idx": 54, "end_idx": 60, "type": "dis", "entity": "外周肺动脉狭窄" } ] }, { "text": "肺骨关节病可有骨髓腔扩大,继发于发绀型先天性心脏病的低氧血症。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肺骨关节病" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "骨髓腔扩大" }, { "start_idx": 16, "end_idx": 29, "type": "dis", "entity": "发绀型先天性心脏病的低氧血症" } ] }, { "text": "后肋下缘切迹如前所述继发于水肿侧支血管形成。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "水肿侧支血管" } ] }, { "text": "肋骨畸形也可继发于既往的心脏手术。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "肋骨畸形" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "心脏手术" } ] }, { "text": "图9-5完全性肺静脉异位引流伴右心房异构(正位片)示:因肺水肿胸片呈“蝶形”,肺部阴影集中在肺门部并向周边呈对称性消退(七)心脏和腹部脏器位置右位心(dextrocardia)是指心脏的大部位于胸骨右侧,心尖指向左侧。", "entities": [ { "start_idx": 4, "end_idx": 19, "type": "dis", "entity": "完全性肺静脉异位引流伴右心房异构" }, { "start_idx": 28, "end_idx": 37, "type": "sym", "entity": "肺水肿胸片呈“蝶形”" }, { "start_idx": 39, "end_idx": 58, "type": "sym", "entity": "肺部阴影集中在肺门部并向周边呈对称性消退" }, { "start_idx": 62, "end_idx": 70, "type": "dis", "entity": "心脏和腹部脏器位置" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "右位心" }, { "start_idx": 75, "end_idx": 86, "type": "dis", "entity": "dextrocardia" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "心脏" }, { "start_idx": 97, "end_idx": 98, "type": "bod", "entity": "胸骨" }, { "start_idx": 102, "end_idx": 103, "type": "bod", "entity": "心尖" } ] }, { "text": "当心脏的所有心腔反位伴有腹腔脏器全部反位时称为镜像性右位心(图9-6),镜像右位心不伴有任何先天性心脏病者临床上少见。", "entities": [ { "start_idx": 1, "end_idx": 19, "type": "sym", "entity": "心脏的所有心腔反位伴有腹腔脏器全部反位" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "镜像性右位心" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "镜像右位心" } ] }, { "text": "大多数右位心合并先天性心脏病,X线胸片表现为心尖指向右侧、胃泡在正常左侧位。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "右位心" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "X线胸片" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "心尖指向右侧" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "胃泡在正常左侧位" } ] }, { "text": "部分右位心可伴有右肺发育不全。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "右位心" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "右肺发育不全" } ] }, { "text": "图9-6右心房异构,房室间隔缺损和肺动脉瓣狭窄(正位片)示:右位心和肺野缺血,横位的肝脏居中提示合并复杂型先心病通过辨别胃泡及肝脏右叶的位置可判定内脏位置。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "右心房异构" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "房室间隔缺损" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "右位心" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "肺野缺血" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "横位的肝脏居中" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "复杂型先心病" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "胃泡" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "肝脏右叶" } ] }, { "text": "绝大多数人表现为心房内脏正位,少数人两者均成呈镜像反位。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "心房内脏正位" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "镜像反位" } ] }, { "text": "有时可有双左或双右侧内脏结构(心耳、肺脏、支气管等),称为左或右侧异构,常与复杂先心病有关;亦可见肝脏横位或胃肠位于腹腔正中。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "sym", "entity": "双左或双右侧内脏结构" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心耳" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肺脏" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "支气管" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "左或右侧异构" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "复杂先心病" }, { "start_idx": 49, "end_idx": 61, "type": "sym", "entity": "肝脏横位或胃肠位于腹腔正中" } ] }, { "text": "由于支气管X线断层扫描广泛应用,支气管位置也已成为定位依据。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "支气管X线断层扫描" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "支气管" } ] }, { "text": "双右异构时,左侧、右侧主支气管短,上叶支气管向上走行;双左异构时则表现为两侧主支气管均较长。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "双右异构" }, { "start_idx": 6, "end_idx": 15, "type": "sym", "entity": "左侧、右侧主支气管短" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "上叶支气管向上走行" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "双左异构" }, { "start_idx": 36, "end_idx": 44, "type": "sym", "entity": "两侧主支气管均较长" } ] }, { "text": "目前通过超声心动图探查腹主动脉与下腔静脉关系可准确反映心房的位置。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "超声心动图" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "腹主动脉" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "下腔静脉" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "心房" } ] }, { "text": "第六节低磷血症性抗维生素D佝偻病低磷血症性抗维生素D佝偻病,又称家族性低磷血症性维生素D难治性佝偻病,多为性联显性遗传性疾病。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "低磷血症性抗维生素D佝偻病" }, { "start_idx": 16, "end_idx": 28, "type": "dis", "entity": "低磷血症性抗维生素D佝偻病" }, { "start_idx": 32, "end_idx": 49, "type": "dis", "entity": "家族性低磷血症性维生素D难治性佝偻病" }, { "start_idx": 53, "end_idx": 61, "type": "dis", "entity": "性联显性遗传性疾病" } ] }, { "text": "亦有人称本病为肾性磷丢失症。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "肾性磷丢失症" } ] }, { "text": "对一般治疗剂量的维生素D无效。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "维生素D" } ] }, { "text": "【病因和发病机制】本病多为性联显性遗传的肾小管功能缺陷,少数为常染色体显性或隐性遗传。", "entities": [ { "start_idx": 13, "end_idx": 26, "type": "dis", "entity": "性联显性遗传的肾小管功能缺陷" }, { "start_idx": 31, "end_idx": 41, "type": "sym", "entity": "常染色体显性或隐性遗传" } ] }, { "text": "由于染色体的先天病变致肾近曲小管细胞膜刷状缘钠-磷转运系统异常,肾小管对磷的重吸收障碍,引起大量磷从肾脏排出;另外,染色体的异常也造成成骨细胞功能不良,致成骨缺陷,造成临床上低磷血症及骨发育不良。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "染色体的先天病变" }, { "start_idx": 11, "end_idx": 21, "type": "bod", "entity": "肾近曲小管细胞膜刷状缘" }, { "start_idx": 11, "end_idx": 30, "type": "sym", "entity": "肾近曲小管细胞膜刷状缘钠-磷转运系统异常" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "肾小管" }, { "start_idx": 32, "end_idx": 42, "type": "sym", "entity": "肾小管对磷的重吸收障碍" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "肾脏" }, { "start_idx": 46, "end_idx": 53, "type": "sym", "entity": "大量磷从肾脏排出" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "染色体" }, { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "染色体的异常" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "成骨细胞" }, { "start_idx": 67, "end_idx": 74, "type": "sym", "entity": "成骨细胞功能不良" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "成骨" }, { "start_idx": 77, "end_idx": 80, "type": "sym", "entity": "成骨缺陷" }, { "start_idx": 87, "end_idx": 90, "type": "dis", "entity": "低磷血症" }, { "start_idx": 92, "end_idx": 96, "type": "dis", "entity": "骨发育不良" } ] }, { "text": "目前认为与下列发病机制有关:(一)肾小管保留磷和肠道吸收磷障碍认为肾小管本身功能障碍,可能缺乏一种磷结合蛋白,因为肾小管和肠黏膜上皮细胞的磷结合蛋白可能受同一基因位点的密码控制,当此基因缺陷,可出现尿磷丢失增加及肠道磷摄取障碍。", "entities": [ { "start_idx": 17, "end_idx": 30, "type": "dis", "entity": "肾小管保留磷和肠道吸收磷障碍" }, { "start_idx": 33, "end_idx": 41, "type": "dis", "entity": "肾小管本身功能障碍" }, { "start_idx": 49, "end_idx": 53, "type": "bod", "entity": "磷结合蛋白" }, { "start_idx": 45, "end_idx": 53, "type": "sym", "entity": "缺乏一种磷结合蛋白" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "肾小管" }, { "start_idx": 61, "end_idx": 67, "type": "bod", "entity": "肠黏膜上皮细胞" }, { "start_idx": 69, "end_idx": 73, "type": "bod", "entity": "磷结合蛋白" }, { "start_idx": 99, "end_idx": 104, "type": "sym", "entity": "尿磷丢失增加" }, { "start_idx": 106, "end_idx": 112, "type": "sym", "entity": "肠道磷摄取障碍" } ] }, { "text": "(二)肾脏1-α羟化酶缺陷肾脏1-α羟化酶活性减低,使1,25(OH)2D3合成减少,肠道钙吸收减少,尿磷排出增加,同时引起继发性甲状旁腺功能亢进,甲状旁腺素(PTH)增加,加重尿磷排出,最后导致骨钙化不全而造成佝偻病或骨软化症。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "肾脏1-α羟化酶缺陷" }, { "start_idx": 13, "end_idx": 20, "type": "bod", "entity": "肾脏1-α羟化酶" }, { "start_idx": 13, "end_idx": 24, "type": "sym", "entity": "肾脏1-α羟化酶活性减低" }, { "start_idx": 27, "end_idx": 54, "type": "bod", "entity": "1,25(OH)2D3" }, { "start_idx": 27, "end_idx": 58, "type": "sym", "entity": "1,25(OH)2D3合成减少" }, { "start_idx": 60, "end_idx": 66, "type": "sym", "entity": "肠道钙吸收减少" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "尿磷" }, { "start_idx": 68, "end_idx": 73, "type": "sym", "entity": "尿磷排出增加" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "甲状旁腺" }, { "start_idx": 79, "end_idx": 89, "type": "sym", "entity": "继发性甲状旁腺功能亢进" }, { "start_idx": 91, "end_idx": 95, "type": "bod", "entity": "甲状旁腺素" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "PTH" }, { "start_idx": 91, "end_idx": 102, "type": "sym", "entity": "甲状旁腺素(PTH)增加" }, { "start_idx": 106, "end_idx": 107, "type": "bod", "entity": "尿磷" }, { "start_idx": 104, "end_idx": 109, "type": "sym", "entity": "加重尿磷排出" }, { "start_idx": 115, "end_idx": 119, "type": "sym", "entity": "骨钙化不全" }, { "start_idx": 123, "end_idx": 125, "type": "dis", "entity": "佝偻病" }, { "start_idx": 127, "end_idx": 130, "type": "dis", "entity": "骨软化症" } ] }, { "text": "患儿多在1岁半出现症状,常表现为生长发育障碍,身材矮小;骨骼呈佝偻病样表现,特别是下肢短小和畸形。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "生长发育障碍" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "身材矮小" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "骨骼" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "骨骼呈佝偻病样" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "下肢" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "下肢短小和畸形" } ] }, { "text": "成人则发生骨软化症。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "骨软化症" } ] }, { "text": "在较严重的病人,其临床特征与维生素D缺乏性佝偻病相同。", "entities": [ { "start_idx": 14, "end_idx": 23, "type": "dis", "entity": "维生素D缺乏性佝偻病" } ] }, { "text": "儿童在6岁左右可出现典型的佝偻病;严重骨骼畸形及侏儒症;剧烈骨痛。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "佝偻病" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "严重骨骼畸形" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "侏儒症" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "剧烈骨痛" } ] }, { "text": "有些病人可因骨骼疼痛及至不能行走。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "骨骼" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "骨骼疼痛" } ] }, { "text": "小儿出现骨病前,早期常出现牙齿病变(牙折断、磨损、脱落、釉质矿质过少或发育不全)。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "骨病" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "牙齿" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "牙齿病变" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "牙" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "牙折断" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "磨损" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "脱落" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "釉质矿质过少" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "发育不全" } ] }, { "text": "维生素D治疗效果欠佳。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "维生素D" } ] }, { "text": "【实验室检查】血磷显著降低,常为0.32~0.78mmol/L(1~2.4mg/dl),24小时尿磷升高达21mg/kg以上(正常24小时12~20mg/kg)。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "血磷" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "血磷显著降低" }, { "start_idx": 48, "end_idx": 49, "type": "ite", "entity": "尿磷" }, { "start_idx": 44, "end_idx": 61, "type": "sym", "entity": "24小时尿磷升高达21mg/kg以上" } ] }, { "text": "血钙正常,尿钙正常,血钙磷乘积降低,常<30。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "血钙" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "血钙正常" }, { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "尿钙" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "尿钙正常" }, { "start_idx": 10, "end_idx": 14, "type": "ite", "entity": "血钙磷乘积" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "血钙磷乘积降低" } ] }, { "text": "活动期血碱性磷酸酶升高,血甲状旁腺激素正常或轻度升高,血l,25-(OH)2D3多正常。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "ite", "entity": "血碱性磷酸酶" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "活动期血碱性磷酸酶升高" }, { "start_idx": 12, "end_idx": 18, "type": "ite", "entity": "血甲状旁腺激素" }, { "start_idx": 12, "end_idx": 25, "type": "sym", "entity": "血甲状旁腺激素正常或轻度升高" } ] }, { "text": "骨X线表现为典型佝偻病及骨软化象征。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "骨X线" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "典型佝偻病" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "骨软化象征" } ] }, { "text": "本病应与下述疾病作鉴别:①维生素D缺乏性佝偻病:有缺乏维生素D的病因,对常规剂量维生素D治疗后反应良好,可资鉴别。", "entities": [ { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "维生素D缺乏性佝偻病" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "缺乏维生素D" }, { "start_idx": 36, "end_idx": 45, "type": "pro", "entity": "常规剂量维生素D治疗" } ] }, { "text": "此外,尿磷不增加,血甲状旁腺激素含量增加;尿cAMP升高,亦有助于鉴别。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "尿磷" }, { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "尿磷不增加" }, { "start_idx": 9, "end_idx": 15, "type": "ite", "entity": "血甲状旁腺激素" }, { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "血甲状旁腺激素含量增加" }, { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "尿cAMP" }, { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "尿cAMP升高" } ] }, { "text": "②还需注意与维生素D依赖性佝偻病、范可尼综合征、Lowe综合征、肾小管性酸中毒以及慢性肾功能不全等鉴别。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "维生素D依赖性佝偻病" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "范可尼综合征" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "Lowe综合征" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "肾小管性酸中毒" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "慢性肾功能不全" } ] }, { "text": "【治疗】(一)补充维生素D大剂量维生素D2,可用20000~100000U/d,或肌肉注射维生素D325000~50000U/d。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "补充维生素D" }, { "start_idx": 52, "end_idx": 85, "type": "pro", "entity": "肌肉注射维生素D325000~50000U/d" } ] }, { "text": "有条件应服用活性维生素D30.5~1μg/d。", "entities": [ { "start_idx": 4, "end_idx": 32, "type": "pro", "entity": "服用活性维生素D30.5~1μg/d" } ] }, { "text": "在治疗期间应根据病人的血钙、血磷、尿钙及骨X线征来调节剂量,防止发生高钙血症。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "血钙" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "血磷" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "尿钙" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "骨X线征" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "高钙血症" } ] }, { "text": "单独补充维生素D不能纠正低磷血症及生长迟缓。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "单独补充维生素D" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "低磷血症" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "生长迟缓" } ] }, { "text": "(二)补充磷用磷酸盐合剂20ml,每日4~5次。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "dru", "entity": "磷" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "磷酸盐合剂" } ] }, { "text": "其配方为:磷酸二氢钠18g,磷酸氢二钠145g,水加至1000ml。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "磷酸二氢钠" }, { "start_idx": 14, "end_idx": 18, "type": "dru", "entity": "磷酸氢二钠" } ] }, { "text": "补给磷后可减少维生素D用量,口服磷和1,25(OH)2D3可使近90%的病例骨痛明显减轻。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "dru", "entity": "磷" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "维生素D" }, { "start_idx": 16, "end_idx": 16, "type": "dru", "entity": "磷" }, { "start_idx": 60, "end_idx": 65, "type": "sym", "entity": "骨痛明显减轻" } ] }, { "text": "(三)其他治疗给予维生素C(降低尿pH)及钙剂可加强肾对磷的再吸收。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "维生素C" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "尿pH" }, { "start_idx": 21, "end_idx": 22, "type": "dru", "entity": "钙剂" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "肾" }, { "start_idx": 28, "end_idx": 28, "type": "dru", "entity": "磷" } ] }, { "text": "骨骼畸形明显而病情已静止,X线及生化检查已正常者,于12岁以后可作矫形手术,术前2周停服维生素D,以避免高钙血症的肾损害发生。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "骨骼" }, { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "骨骼畸形明显" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "X线" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "生化检查" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "矫形手术" }, { "start_idx": 44, "end_idx": 47, "type": "dru", "entity": "维生素D" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "高钙血症" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "肾损害" } ] }, { "text": "第三节缺铁性贫血缺铁性贫血(irondeficiencyanemia,IDA)是婴幼儿时期最常见的一种贫血。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "缺铁性贫血" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "缺铁性贫血" }, { "start_idx": 14, "end_idx": 33, "type": "dis", "entity": "irondeficiencyanemia" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "IDA" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "贫血" } ] }, { "text": "其发生的根本病因是体内铁缺乏致使血红蛋白合成减少而发生的一种小细胞低色素性贫血。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 30, "end_idx": 38, "type": "dis", "entity": "小细胞低色素性贫血" } ] }, { "text": "【铁的代谢】(一)铁在体内的分布体内大部分铁主要分布在血红蛋白中,少量存在于肌红蛋白中,两者占体内铁总量的60%~70%。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "铁" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "铁" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "肌红蛋白" } ] }, { "text": "细胞色素、过氧化氢酶及血浆中运输状态中的铁,仅占极小部分。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "细胞色素" }, { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "过氧化氢酶" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血浆" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "铁" } ] }, { "text": "其余约30%~40%的铁则以铁蛋白和含铁血黄素的形式贮存于骨髓、肝、脾、淋巴结等网状内皮系统中。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "骨髓" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "肝" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "脾" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "淋巴结" }, { "start_idx": 40, "end_idx": 45, "type": "bod", "entity": "网状内皮系统" } ] }, { "text": "内源性铁主要来自更新破坏的红细胞。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "红细胞" } ] }, { "text": "这种衰老红细胞经机体网状内皮系统消化降解的铁可被重新利用。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "红细胞" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "网状内皮系统" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "铁" } ] }, { "text": "此外,小儿在正常发育过程中,还可以通过消化道、皮肤、泌尿生殖道黏膜上皮细胞脱落而丢失一部分铁。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "消化道" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "皮肤" }, { "start_idx": 26, "end_idx": 36, "type": "bod", "entity": "泌尿生殖道黏膜上皮细胞" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "铁" } ] }, { "text": "因此,一个15岁以内的小儿每天从食物中吸收的铁至少需要1mg才能满足铁的正平衡。", "entities": [ { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "铁" } ] }, { "text": "铁吸收的主要部位在十二指肠及空肠上段,部分借助于位于十二指肠中的几种辅助铁吸收蛋白。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "十二指肠" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "空肠" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "十二指肠" }, { "start_idx": 34, "end_idx": 40, "type": "bod", "entity": "辅助铁吸收蛋白" } ] }, { "text": "适量胃酸的存在对铁的吸收颇为重要,它们能将食物中的三价铁转化成二价铁;维生素C能将三价铁还原成二价铁而有助于铁的吸收。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "胃酸" } ] }, { "text": "由于人体铁极少排泄,因此,铁吸收的调节是维持体内铁平衡的主要机制。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "铁" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "铁" } ] }, { "text": "肠黏膜上皮细胞具有控制铁吸收的能力,它们可根据体内铁的需要程度来增减铁的吸收量。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "肠黏膜上皮细胞" } ] }, { "text": "铁的吸收效率因食物种类不同而异,大多数素食中的铁吸收效率较差,平均仅5%左右(1.7%~7.9%),而黄豆、肉类和血红蛋白中铁的吸收效率可高达15%~20%。", "entities": [ { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "从食物中所吸收的铁有两种去向,其中一部分铁吸收后进入肠黏膜上皮细胞内,与其中的去铁蛋白(apoferritin)结合而形成铁蛋白;大部分吸收铁直接进入血液循环,并与血浆中的转铁蛋白(transferrin)结合而被转运。", "entities": [ { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "肠黏膜上皮细胞" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "去铁蛋白" }, { "start_idx": 44, "end_idx": 54, "type": "bod", "entity": "apoferritin" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "铁蛋白" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "血液" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "血浆" }, { "start_idx": 86, "end_idx": 89, "type": "bod", "entity": "转铁蛋白" }, { "start_idx": 91, "end_idx": 101, "type": "bod", "entity": "transferrin" } ] }, { "text": "(五)铁的转运被吸收的二价铁进入血液循环后又被氧化成三价铁,并与转铁蛋白结合。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "血液" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "转铁蛋白" } ] }, { "text": "通常,二分子Fe3+需一分子的转铁蛋白。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "转铁蛋白" } ] }, { "text": "与转铁蛋白相结合的铁称为血清铁(serumiron,SI)。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "转铁蛋白" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "血清铁" }, { "start_idx": 16, "end_idx": 24, "type": "bod", "entity": "serumiron" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "SI" } ] }, { "text": "SI被转运至骨髓幼红细胞和网织红细胞胞质内,约80%~90%进入幼红细胞的铁被线粒体摄取,并与原卟啉Ⅸ结合形成血红素,血红素再与珠蛋白结合而成血红蛋白。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "SI" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "骨髓幼红细胞" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "网织红细胞" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "胞质" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "幼红细胞" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "铁" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "线粒体" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "原卟啉Ⅸ" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "血红素" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "血红素" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "珠蛋白" }, { "start_idx": 71, "end_idx": 74, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "转铁蛋白存在于血浆β球蛋白组分中,是一种糖蛋白,主要在肝脏中合成,在465nm波长处有最大吸收峰。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "转铁蛋白" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血浆" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "β球蛋白" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "糖蛋白" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肝脏" } ] }, { "text": "转铁蛋白分子的表面有许多铁结合位点,在正常情况下,仅1/3的转铁蛋白铁结合位点被铁结合,换言之,转铁蛋白铁结合的饱和度仅33.3%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "转铁蛋白分子" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "转铁蛋白" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "转铁蛋白" } ] }, { "text": "转铁蛋白的主要功能是为骨髓造红细胞提供原料。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "转铁蛋白" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "骨髓造红细胞" } ] }, { "text": "它们将所结合的铁通过位于幼红细胞膜上转铁蛋白受体(TfR)的协助下转入幼红细胞和网织红细胞内后,在胞质低pH(5.5)条件下,转铁蛋白迅速释放出铁,而转铁蛋白本身又回到血浆中重新执行运铁的功能,如此循环往复,周而复始。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "幼红细胞膜" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "转铁蛋白受体" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "TfR" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "幼红细胞" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "网织红细胞" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "胞质" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "转铁蛋白" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "转铁蛋白" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "血浆" } ] }, { "text": "转铁蛋白的血浆半寿期平均为9(8~10.4)天。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "转铁蛋白" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "血浆" } ] }, { "text": "血清中转铁蛋白能结合SI的总量称为总铁结合力(totalironbindingcapacity,TIBC),未被铁结合的转铁蛋白铁结合能力称为未饱和铁结合力(unboundironbindingcapacity,UIBC)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血清" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "转铁蛋白" }, { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "总铁结合力" }, { "start_idx": 23, "end_idx": 46, "type": "ite", "entity": "totalironbindingcapacity" }, { "start_idx": 48, "end_idx": 51, "type": "ite", "entity": "TIBC" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "转铁蛋白" }, { "start_idx": 71, "end_idx": 77, "type": "ite", "entity": "未饱和铁结合力" }, { "start_idx": 79, "end_idx": 104, "type": "ite", "entity": "unboundironbindingcapacity" }, { "start_idx": 106, "end_idx": 109, "type": "ite", "entity": "UIBC" } ] }, { "text": "因此,这三者之间的关系可用一个公式(TIBC=SI+UIBC)来表示。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "ite", "entity": "TIBC" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "SI" }, { "start_idx": 26, "end_idx": 29, "type": "ite", "entity": "UIBC" } ] }, { "text": "幼红细胞中未被利用的铁以小粒的形式存在于胞质中,亚铁氰化钾可将其染成蓝色。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "幼红细胞" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "胞质" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "亚铁氰化钾" } ] }, { "text": "在缺铁情况下,幼红细胞中的铁小粒显著减少甚至消失,而在体内贮存铁增多时,幼红细胞中铁小粒也增多。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "缺铁" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "幼红细胞" }, { "start_idx": 7, "end_idx": 23, "type": "sym", "entity": "幼红细胞中的铁小粒显著减少甚至消失" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "幼红细胞" }, { "start_idx": 36, "end_idx": 46, "type": "sym", "entity": "幼红细胞中铁小粒也增多" } ] }, { "text": "含有铁粒的幼红细胞称为铁粒幼细胞。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "幼红细胞" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "铁粒幼细胞" } ] }, { "text": "(六)铁的贮存铁主要贮存于肝、脾与骨髓中。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "铁" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "铁" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肝" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "脾" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "骨髓" } ] }, { "text": "贮存的主要形式为铁蛋白与含铁血黄素。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "铁蛋白" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "含铁血黄素" } ] }, { "text": "铁蛋白中含铁可高达23%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "铁蛋白" } ] }, { "text": "含铁血黄素为颗粒状物质,含铁37%,见于铁蛋白含量最高的组织中。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "含铁血黄素" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "铁蛋白" } ] }, { "text": "(七)铁的排泄正常小儿铁的排泄量极微,主要排泄途径为胆汁、尿、粪、汗、脱落的毛发及剥落的皮肤、黏膜细胞。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "铁" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "铁" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "胆汁" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "尿" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "粪" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "汗" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "毛发" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "皮肤" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "黏膜细胞" } ] }, { "text": "【病因及发病机制】在正常情况下,铁吸收和排泄基本是平衡的。", "entities": [ { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "铁" } ] }, { "text": "如果铁的消耗超过体内所能供给的量,就会发生缺铁。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "铁" } ] }, { "text": "引起缺铁的可能因素有:(一)贮存铁不足早产儿、母亲怀孕期严重缺铁、胎儿宫内失血等均可出现贮存铁不足。", "entities": [ { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "铁" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "铁" } ] }, { "text": "(二)饮食中铁含量不足以牛乳、米、面粉等为主的食物进行人工喂养的婴儿,由于食物中含铁较少,不足以适应生长的需要,故易发生缺铁或缺铁性贫血,早产儿尤易如此。", "entities": [ { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "铁" }, { "start_idx": 60, "end_idx": 67, "type": "dis", "entity": "缺铁或缺铁性贫血" } ] }, { "text": "(三)吸收障碍消化系统的疾病如长期慢性腹泻、脂肪泻等均可影响铁的吸收。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "消化系统" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "长期慢性腹泻" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "脂肪泻" } ] }, { "text": "(四)需要量增加婴幼儿尤其是早产儿生长发育快,青春期前后发育也快,如饮食中无足够的铁供应,即可发生缺铁性贫血。", "entities": [ { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "缺铁性贫血" } ] }, { "text": "(五)失血长期慢性失血见于消化性溃疡、钩虫病、多发性肠息肉、血管瘤、梅克尔憩室炎或者炎症性肠病等,急性失血见于外伤、鼻出血等。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "长期慢性失血" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "钩虫病" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "多发性肠息肉" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "血管瘤" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "梅克尔憩室炎" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "炎症性肠病" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "外伤" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "鼻出血" } ] }, { "text": "铁是血红蛋白的必要组成成分。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "铁" }, { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "当体内缺铁或铁的利用发生障碍时,血红素合成不足,因而血红蛋白合成减少,形成小细胞低色素性贫血。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "铁" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "铁" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "血红素" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 37, "end_idx": 45, "type": "dis", "entity": "小细胞低色素性贫血" } ] }, { "text": "同时细胞色素、过氧化氢酶等也因缺铁而活性降低,细胞呼吸发生障碍。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "细胞色素" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "过氧化氢酶" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "细胞" } ] }, { "text": "贫血发生后,含铁酶活性的降低和长期携氧不足而影响消化、呼吸、循环、神经和免疫等系统的功能。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "贫血" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "含铁酶" }, { "start_idx": 27, "end_idx": 40, "type": "bod", "entity": "呼吸、循环、神经和免疫等系统" } ] }, { "text": "值得提出的是体内刚出现缺铁时并非立刻出现贫血。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "缺铁" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "贫血" } ] }, { "text": "当体内已经有缺铁存在但尚无血红蛋白降低者称为缺铁(irondeficiency,ID);只有当缺铁同时伴有血红蛋白下降者才称为缺铁性贫血(irondeficiencyanemia,IDA)。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "缺铁" }, { "start_idx": 25, "end_idx": 38, "type": "dis", "entity": "irondeficiency" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "ID" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "缺铁" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "缺铁性贫血" }, { "start_idx": 69, "end_idx": 88, "type": "dis", "entity": "irondeficiencyanemia" }, { "start_idx": 90, "end_idx": 92, "type": "dis", "entity": "IDA" } ] }, { "text": "如缺铁进一步加重,铁蛋白几近耗竭时,血红蛋白才开始下降,临床上出现明显的贫血表现。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "缺铁" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "铁蛋白" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "贫血" } ] }, { "text": "【临床表现】IDA的发病高峰年龄在6个月~3周岁,患儿常有皮肤和黏膜苍白,软弱无力,心悸、气急、食欲差、不愿活动,精神不振,对环境不感兴趣,易烦躁、哭闹。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "IDA" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "皮肤和黏膜苍白" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "软弱无力" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "心悸" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "气急" }, { "start_idx": 48, "end_idx": 50, "type": "sym", "entity": "食欲差" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "不愿活动" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "精神不振" }, { "start_idx": 62, "end_idx": 68, "type": "sym", "entity": "对环境不感兴趣" }, { "start_idx": 70, "end_idx": 72, "type": "sym", "entity": "易烦躁" }, { "start_idx": 74, "end_idx": 75, "type": "sym", "entity": "哭闹" } ] }, { "text": "可见口角炎、舌乳头萎缩、肛门皮肤发炎、反甲、皮肤干枯、毛发脆易断、失去光泽等。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "口角炎" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "舌乳头萎缩" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "肛门皮肤发炎" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "反甲" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "皮肤干枯" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "毛发脆易断" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "失去光泽" } ] }, { "text": "严重者出现异食癖。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "严重者出现异食癖" } ] }, { "text": "新生儿或小婴儿可有屏气发作,也称呼吸暂停症(breathholdingspell,BHS)。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "屏气发作" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "呼吸暂停症" }, { "start_idx": 22, "end_idx": 39, "type": "dis", "entity": "breathholdingspell" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "BHS" } ] }, { "text": "贫血严重时可有心脏扩大和心功能不全。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "贫血严重时可有心脏扩大和心功能不全" } ] }, { "text": "IDA患儿还可以出现免疫功能低下,容易合并感染。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "IDA" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "免疫功能低下" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "合并感染" } ] }, { "text": "【诊断与鉴别诊断】(一)病史及临床表现IDA的诊断应结合喂养史、出生体重、发病年龄及临床症状和体征等综合判断。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "IDA" } ] }, { "text": "患儿血红蛋白量比红细胞数降低明显,红细胞体积较小、中空、色淡,MCV<80μm3(fl),MCH<26pg,MCHC<320g/L,红细胞平均直径6.5μm。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "红细胞" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "红细胞" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "红细胞" } ] }, { "text": "网织红细胞在治疗前通常在正常范围或稍高,但治疗后7~10天可出现明显升高,但极少超过10%以上。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "网织红细胞" } ] }, { "text": "偶尔外周血中可出现有核红细胞。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "外周血" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "核红细胞" } ] }, { "text": "血小板计数大多在正常范围,但也可以出现血小板增多,甚至高达(600~1000)×109/L,贫血较重者血小板可减少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "血小板计数" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "血小板" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "贫血" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "血小板" } ] }, { "text": "血小板计数的改变机制不明,可能为缺铁的直接结果。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "血小板计数" } ] }, { "text": "(三)骨髓象有核红细胞增生活跃,严重患儿也可增生低下。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "核红细胞" } ] }, { "text": "轻度至中度红系细胞增多,幼红细胞比例增多,重度贫血患儿幼红细胞胞质较少,体积较小,边缘不整齐,胞质着色偏蓝,出现核、浆发育不平衡的表现:胞质发育落后于胞核。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "中度红系细胞" }, { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "中度红系细胞增多" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "幼红细胞" }, { "start_idx": 12, "end_idx": 19, "type": "sym", "entity": "幼红细胞比例增多" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "重度贫血" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "幼红细胞胞质较少" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "体积较小" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "边缘不整齐" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "胞质着色偏蓝" }, { "start_idx": 54, "end_idx": 66, "type": "sym", "entity": "出现核、浆发育不平衡的表现" }, { "start_idx": 68, "end_idx": 76, "type": "sym", "entity": "胞质发育落后于胞核" } ] }, { "text": "早幼红细胞和中幼红细胞比例增高,而晚幼红细胞减少。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "早幼红细胞和中幼红细胞比例增高" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "晚幼红细胞减少" } ] }, { "text": "骨髓涂片铁染色示细胞内、外铁均明显减少或缺如,铁粒幼细胞减少或不见。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "骨髓涂片铁染色示细胞内" }, { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "外铁均明显减少或缺如" }, { "start_idx": 23, "end_idx": 32, "type": "sym", "entity": "铁粒幼细胞减少或不见" } ] }, { "text": "白细胞和巨核细胞系统正常。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "白细胞和巨核细胞系统正常" } ] }, { "text": "(四)大便隐血试验约有1/3的病儿可有大便隐血试验阳性。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "大便隐血" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "大便隐血" } ] }, { "text": "(五)血生化指标改变SI明显降低,常低于350μg/L。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "血生化指标" }, { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "SI" } ] }, { "text": "TIBC增加,往往高于6700μg/L。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "TIBC" } ] }, { "text": "血清铁饱和度明显降低,常在15%以下。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血清" } ] }, { "text": "血清铁蛋白耗竭。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "血清铁蛋白" } ] }, { "text": "血清游离TfR增加。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血清" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "TfR" } ] }, { "text": "血清TfR/铁蛋白对数比值是诊断IDA的敏感指标,该比值明显增加时有利于IDA的诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "血清TfR" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "铁蛋白" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "IDA" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "IDA" } ] }, { "text": "红细胞内游离原卟啉可明显增高,可高达1000~6000μg/L(正常值为420±180μg/L)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "红细胞" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "原卟啉" } ] }, { "text": "(六)含铁酶的变化在发生缺铁性贫血以后,含铁酶细胞色素C、琥珀酸脱氢酶、单氨氧化酶等活性均可明显下降。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "含铁酶" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "缺铁性贫血" }, { "start_idx": 20, "end_idx": 27, "type": "bod", "entity": "含铁酶细胞色素C" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "琥珀酸脱氢酶" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "单氨氧化酶" } ] }, { "text": "【治疗】IDA的治疗除应加强护理、去除病因、防止感染外,重点应包括以下几方面:(一)改善饮食尤其原来喂养不当者。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "IDA" } ] }, { "text": "(二)铁剂治疗铁剂是治疗IDA的特效药物。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "铁剂" }, { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "铁剂" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "IDA" } ] }, { "text": "常用的制剂有硫酸亚铁、葡萄糖酸亚铁、富马酸亚铁等。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "硫酸亚铁" }, { "start_idx": 11, "end_idx": 16, "type": "dru", "entity": "葡萄糖酸亚铁" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "富马酸亚铁" } ] }, { "text": "由于牛奶含磷较多,可影响铁的吸收,故口服铁剂时不宜饮用牛奶。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "dru", "entity": "铁剂" } ] }, { "text": "注射铁剂疗效并不比口服好,且易出现毒性反应,因此仅在那些不宜口服治疗如伴有吸收不良的患儿才考虑使用;通常的制剂为右旋糖酐铁。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "注射" }, { "start_idx": 2, "end_idx": 3, "type": "dru", "entity": "铁剂" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "口服" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "口服" }, { "start_idx": 56, "end_idx": 60, "type": "dru", "entity": "右旋糖酐铁" } ] }, { "text": "铁剂服量过大可产生中毒现象,患儿可出现恶心、呕吐、不安,严重者可发生昏迷、肝坏死、胃肠道出血或末梢循环衰竭。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "铁剂" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "恶心" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "呕吐" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "不安" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "昏迷" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "肝坏死" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "胃肠道出血" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "末梢循环衰竭" } ] }, { "text": "铁剂治疗的效果可利用网织红细胞百分数作为观察指标,通常治疗后3天网织红细胞开始上升,第7~10天达高峰。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "铁剂" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "网织红细胞" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "网织红细胞" } ] }, { "text": "一周内红细胞和血红蛋白逐渐上升,连续治疗3~4周,血红蛋白可恢复正常。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "红细胞" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "红细胞和血红蛋白逐渐上升" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "此时,铁剂治疗不能立刻停止,而仍需继续治疗2~3月,以补充贮存铁。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "铁剂" } ] }, { "text": "(三)输血轻度贫血无需输血。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "轻度贫血" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "输血" } ] }, { "text": "重度贫血致组织缺氧甚至危及心脏功能者应给予少量多次输血,通常每次给予5~7ml/kg,千万不可操之过急,一次大量输血可造成急性心功能衰竭而危及病儿生命。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "重度贫血" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "组织缺氧" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心脏" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "输血" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "急性心功能衰竭" } ] }, { "text": "【预防】IDA是可以预防的疾病。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "IDA" } ] }, { "text": "对容易发生IDA的小儿,应尽早预防:对婴儿要及时添加适当的辅助食品,对未成熟儿早给铁剂。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "IDA" }, { "start_idx": 41, "end_idx": 42, "type": "dru", "entity": "铁剂" } ] }, { "text": "参考文献1.柴毅明,周水珍.脑性瘫患合并癫痫.国外医学儿科学分册,2001,(2):94-942.王清江,郑之卿主编.临床小儿神经病学.北京:人民军医出版社,2001:103.林庆,李松.小儿脑性瘫痪.北京:北京医科大学出版社,2000:64.吴希如,林庆.小儿神经系统疾病基础与临床.北京:人民卫生出版社,2000:622-6325.林庆,李松,刘建蒙等.我国六省(区)小儿脑瘫患病率及临床类型的调查分析.中华儿科杂志,2001,39:613-6156.GabriellaE.Molnar;Michael.Alexan.Pediatricrehabilitation.ed3.Philadelphia:HanceyandBelfus.1999,193-1937.ArpinoC,CuratoloP,StaziMA,etal.DIFferingriskfactorsforcerebralpalsyinthepresenceofmentalretardationandepilepsy.J-Child-Neurol.1999,14(3):151-1518.JeanAicardi,MartinBax,ChristopherGillberg.DiseasesofthenervoussysteminChildood,3ed.MacKeithPressDistributedbyWiley-Blackwell.2009,210-2109.JeanAicardi,MartinB,ChristopherGillberg.ClinicinDevelopmentalMedicine:DiseasesoftheNervoussysteminChildhood3rdEdition.MacKeithPress,2009", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "脑性瘫患" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "癫痫" }, { "start_idx": 27, "end_idx": 28, "type": "dep", "entity": "儿科" }, { "start_idx": 94, "end_idx": 99, "type": "dis", "entity": "小儿脑性瘫痪" }, { "start_idx": 129, "end_idx": 136, "type": "dis", "entity": "小儿神经系统疾病" }, { "start_idx": 140, "end_idx": 141, "type": "dep", "entity": "临床" }, { "start_idx": 186, "end_idx": 189, "type": "dis", "entity": "小儿脑瘫" }, { "start_idx": 206, "end_idx": 207, "type": "dep", "entity": "儿科" } ] }, { "text": "第十四篇遗传性疾病第一章遗传概述遗传性疾病是由于遗传物质结构或功能改变所导致的疾病,简称遗传病(geneticdisease),在儿科学中占重要的地位。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "遗传" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 24, "end_idx": 34, "type": "sym", "entity": "遗传物质结构或功能改变" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "遗传病" }, { "start_idx": 48, "end_idx": 61, "type": "dis", "entity": "geneticdisease" } ] }, { "text": "据统计,活产新生儿中,患不同遗传病者约占4%~6%,其中单基因病占1%以上,多基因病占2%~3%,染色体病占0.5%,其他占0.5%~1%。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "遗传病" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "单基因病" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "多基因病" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "染色体病" } ] }, { "text": "临床上尽管单一遗传病的发病率很低,但在总体上,遗传病在儿科疾病中所占的比例非常高。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "遗传病" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "遗传病" }, { "start_idx": 27, "end_idx": 28, "type": "dep", "entity": "儿科" } ] }, { "text": "特别是随着科学和社会的进步,急性感染性疾病和营养不良性疾病得到了较有效的控制,儿童的疾病谱发生了很大的改变,遗传病所占的地位越来越重要。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "急性感染性疾病" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "营养不良性疾病" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "遗传病" } ] }, { "text": "根据统计,在儿科专科医院住院患者中,25%~39%是因遗传原因导致发病,11%的儿童期死亡患者与遗传因素有关。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dep", "entity": "儿科" } ] }, { "text": "遗传病种类繁多,涉及全身各个系统,分散在临床各专业,导致畸形、代谢异常、神经和肌肉功能障碍,病死率和残疾率均较高。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "遗传病" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "畸形" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "代谢异常" }, { "start_idx": 36, "end_idx": 44, "type": "dis", "entity": "神经和肌肉功能障碍" } ] }, { "text": "本章重点介绍染色体病和遗传代谢病。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "染色体病" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "遗传代谢病" } ] }, { "text": "图14-1基因结构示意图一、染色体与基因1.染色体正常人体细胞(somaticcell)内含46条或23对染色体,配子即精子或卵子所含的染色体数目为体细胞染色体的一半。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "基因" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "染色体" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "基因" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "染色体" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "体细胞" }, { "start_idx": 32, "end_idx": 42, "type": "bod", "entity": "somaticcell" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "染色体" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "精子" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "卵子" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "染色体" }, { "start_idx": 74, "end_idx": 79, "type": "bod", "entity": "体细胞染色体" } ] }, { "text": "体细胞染色体数目为双倍体(diploid),即2n=46,配子的染色体数目为23(n),称单倍体(haploid),染色体数目倍增(3n,4n....)称多倍体(polyploid),染色体数目比2n多或少一个至多个时称非整倍体(aneuploid),46条染色体中44条男女都一样,称常染色体(autosomes)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "体细胞染色体" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "染色体" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "染色体" }, { "start_idx": 92, "end_idx": 94, "type": "bod", "entity": "染色体" }, { "start_idx": 129, "end_idx": 131, "type": "bod", "entity": "染色体" }, { "start_idx": 143, "end_idx": 146, "type": "bod", "entity": "常染色体" } ] }, { "text": "另外两条染色体男女不同,男性为XY,女性为XX。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "染色体" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "XY" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "XX" } ] }, { "text": "X与Y称性染色体。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "X" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "Y" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "染色体" } ] }, { "text": "染色体的结构畸变包括染色体部分缺失、倒位、重复、易位及插入等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "染色体" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "染色体的结构畸变" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "染色体" }, { "start_idx": 10, "end_idx": 28, "type": "sym", "entity": "染色体部分缺失、倒位、重复、易位及插入" } ] }, { "text": "2.基因与基因组基因是遗传的基本功能单位,是DNA双螺旋链上的一段负载一定的遗传信息,并在特定条件下表达,产生特定的生理功能的DNA片段。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "基因" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "基因组" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "基因" }, { "start_idx": 22, "end_idx": 28, "type": "bod", "entity": "DNA双螺旋链" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "DNA片段" } ] }, { "text": "基因是编码蛋白质肽链和RNA所必需的核苷酸顺序,人类基因组学发现大约有38000个基因。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "基因" }, { "start_idx": 3, "end_idx": 9, "type": "bod", "entity": "编码蛋白质肽链" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "RNA" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "核苷酸" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "基因组" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "基因" } ] }, { "text": "细胞的遗传信息几乎都储存在染色体的DNA分子长链上。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "细胞" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "染色体" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "DNA分子" } ] }, { "text": "人类细胞中的全部基因称为基因组(genome)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "细胞" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "基因" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "基因组" } ] }, { "text": "人类基因组由31.6亿个碱基对组成,共有3万~3.5万个基因。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "基因组" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "碱基对" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "基因" } ] }, { "text": "一条染色体是一个DNA分子,一个人单倍体细胞含有23个DNA分子,储存着人的全部信息。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "染色体" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "DNA分子" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "单倍体细胞" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "DNA分子" } ] }, { "text": "DNA是由两条多核苷酸链组成的双螺旋结构,脱氧核苷酸上的碱基有4种,即腺嘌呤(A)、胸腺嘧啶(T〕、鸟嘌呤(G)和胞嘧啶(C)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "DNA" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "多核苷酸链" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "脱氧核苷酸" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "碱基" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "腺嘌呤" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "A" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "胸腺嘧啶" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "T" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "鸟嘌呤" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "G" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "胞嘧啶" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "C" } ] }, { "text": "染色体中的所有基因均具有一定的基本单元结构。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "染色体" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "基因" } ] }, { "text": "典型的结构基因由外显子、内含子及调节顺序组成。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "基因" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "内含子" } ] }, { "text": "人类绝大多数的结构基因都存在内含子。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "基因" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "内含子" } ] }, { "text": "内含子是非编码基因序列,外显子才是基因的编码部分。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "内含子" }, { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "非编码基因" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "基因" } ] }, { "text": "在RNA转录中,内含子与外显子一起被转录,称为前体RNA,然后除去内含子剪接为成熟RNA。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "RNA" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "内含子" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "前体RNA" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "内含子" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "成熟RNA" } ] }, { "text": "基因结构(图14-14)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "基因" } ] }, { "text": "在结构基因的两端是非翻译区,又称侧翼区,分别为5'端(上游)侧翼区和3'端(下游)侧翼区。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "基因" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "非翻译区" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "侧翼区" }, { "start_idx": 34, "end_idx": 43, "type": "bod", "entity": "3'端(下游)侧翼区" } ] }, { "text": "其中5'端侧翼区包含基因的启动子区和一些保守序列,涉及基因的转录启动和表达调控。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "5'端侧翼区" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "基因的启动子区" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "基因" } ] }, { "text": "在上游侧翼区的-25~-33以及-50~-100区域内存在TATA盒及CCAAT盒。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "上游侧翼区" } ] }, { "text": "这些DNA元件能与RNA聚合酶及一些转录调控因子相互结合,在转录起始中发挥作用。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "DNA" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "RNA聚合酶" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "转录调控因子" } ] }, { "text": "在3'端侧翼区存在多聚腺苷酸加尾信号。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "3'端侧翼区" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "多聚腺苷酸" } ] }, { "text": "每个基因在染色体上都有自己精确的位置,称为基因位点,二倍体同一对染色体上同一位点的基因及其变异叫等位基因,等位基因中一个异常,一个正常,称为病态杂合子,两个异常者称为病态纯合子。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "基因" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "染色体" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "基因" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "染色体" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "基因" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "等位基因" }, { "start_idx": 70, "end_idx": 74, "type": "bod", "entity": "病态杂合子" }, { "start_idx": 83, "end_idx": 87, "type": "bod", "entity": "病态纯合子" } ] }, { "text": "大部分的遗传物质都在细胞核内,但是胞浆中的线粒体含有自己特殊的遗传物质,包含了16000个碱基片段。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "细胞核" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "胞浆" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "线粒体" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "碱基片段" } ] }, { "text": "所有的线粒体都由母系遗传(因为精子通常不携带线粒体到受精卵内),细胞内的线粒体主要反映了母系的遗传。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "线粒体" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "精子" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "线粒体" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "受精卵" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "细胞" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "线粒体" } ] }, { "text": "线粒体为细胞的运动、收缩、生物合成、主动运输以及信号传导等耗能的过程提供能源。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "线粒体" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "细胞" } ] }, { "text": "线粒体作为细胞的供能装置,将细胞氧化还原产生的能量以高能磷酸键形式暂时储存起来,是糖、脂肪和蛋白质代谢的最终通路。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "线粒体" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "细胞" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "细胞" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "高能磷酸键" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "糖" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "脂肪" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "蛋白质" } ] }, { "text": "线粒体基因组(mitochondrialgenome,mtDNA))是独立于细胞核染色体外的基因组,具有自我复制、转录和编码功能。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "线粒体" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "基因组" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "DNA" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "细胞" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "染色体" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "基因组" } ] }, { "text": "线粒体中所含的DNA为环状双链结构的DNA分子(mtDNA),编码多种与细胞氧化磷酸化有关的酶,是独立于细胞核染色体外的遗传物质,这些基因突变所导致的疾病称线粒体基因病。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "线粒体" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "DNA" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "DNA分子" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "DNA" }, { "start_idx": 36, "end_idx": 42, "type": "bod", "entity": "细胞氧化磷酸化" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "酶" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "细胞" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "染色体" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "基因" }, { "start_idx": 78, "end_idx": 83, "type": "dis", "entity": "线粒体基因病" } ] }, { "text": "人体基因除以上结构基因之外还存在有一定结构特征的其他序列。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "基因" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "基因" } ] }, { "text": "最为突出的是含有很多重复序列,例如微卫星DNA,可作为基因组的一种多态性标记。", "entities": [ { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "微卫星DNA" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "基因组" } ] }, { "text": "另外,目前发现基因组的单核苷酸多态性(singlenucleotidepolymorphism,SNP)发布广泛,数量达数百万,在分子遗传学连锁分析、种群多样性研究、亲子鉴定以及功能研究等领域中具有重要意义。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "基因组" }, { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "单核苷酸多态性" } ] }, { "text": "3.遗传信息的表达(1)DNA的复制:DNA分子把遗传信息传给下一代是通过DNA的复制机制来实现的,复制是指双链DNA分子由一个分子变成与之相同的两个分子的过程,是在DNA聚合酶的催化下完成的。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "DNA" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "DNA" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "DNA" }, { "start_idx": 54, "end_idx": 60, "type": "bod", "entity": "双链DNA分子" }, { "start_idx": 83, "end_idx": 88, "type": "bod", "entity": "DNA聚合酶" } ] }, { "text": "(2)遗传信息的转录(transcription):系DNA分子上的遗传信息传递到信使RNA(mRNA)的过程。", "entities": [ { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "DNA分子" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "信使RNA" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "mRNA" } ] }, { "text": "转录时,细胞核中DNA分子的双链脱开,以其中一条正义链作为RNA合成的“模板”,在酶的作用下,将不编码的内含子切去,然后将编码的外显子拼接起来,合成信使mRNA,mRNA与模板DNA之间按碱基互补的原则进行碱基配对。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "bod", "entity": "细胞核中DNA分子" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "RNA" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "酶" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "内含子" }, { "start_idx": 74, "end_idx": 79, "type": "bod", "entity": "信使mRNA" }, { "start_idx": 81, "end_idx": 84, "type": "bod", "entity": "mRNA" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "模板DNA" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "碱基" }, { "start_idx": 103, "end_idx": 104, "type": "bod", "entity": "碱基" } ] }, { "text": "但A不是与T相配对,而是与U(尿嘧啶)相配,因为RNA分子中不含胸腺嘧啶(T),而是含有尿嘧啶(U)。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "A" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "T" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "U" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "尿嘧啶" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "RNA" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "胸腺嘧啶" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "T" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "尿嘧啶" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "U" } ] }, { "text": "在RNA聚合酶的催化下,合成一条mRNA的单链,其中含有DNA的遗传信息。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "RNA聚合酶" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "mRNA" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "DNA" } ] }, { "text": "以后mRNA再与DNA“模板”分开,通过核膜进入胞浆。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "mRNA" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "DNA" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "核膜" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "胞浆" } ] }, { "text": "(3)遗传信息的翻译(translation):mRNA以遗传密码的方式控制着特定蛋白质的合成,称作翻译,这个过程是在细胞质的核糖体上进行的。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "mRNA" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "蛋白质" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "细胞质" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "核糖体" } ] }, { "text": "各种蛋白质基本上是由20种氨基酸以不同方式和数目组合而成。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "蛋白质" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "氨基酸" } ] }, { "text": "这些氨基酸连接成多肽链,最后形成具有空间结构的蛋白质。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "氨基酸" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "多肽链" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "蛋白质" } ] }, { "text": "现在已知道,mRNA分子中每三个相邻的碱基决定多肽链中的一个氨基酸,这三个相邻的碱基(即三联体)称为密码子(codon)。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "mRNA" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "碱基" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "多肽链" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "氨基酸" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "碱基" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "三联体" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "密码子" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "codon" } ] }, { "text": "由于RNA分子中的碱基是U、A、G、C4种,由于三个相邻的碱基(三联体)代表一个氨基酸的信息,那么就有43=64种密码子。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "RNA" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "碱基" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "U" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "A" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "G" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "C" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "碱基" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "三联体" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "氨基酸" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "密码子" } ] }, { "text": "在64种密码子中,有61种属氨基酸的密码,其中AUG是代表起始密码,也是甲硫氨基酸的密码。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "密码子" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "氨基酸" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "AUG" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "甲硫氨基酸" } ] }, { "text": "还有3种密码UAA、UAG及UGA不决定任何氨基酸,是“终止密码”,代表蛋白质合成终止的信号。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "UAA" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "UAG" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "UGA" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "氨基酸" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "蛋白质" } ] }, { "text": "二、开胸活检开胸活检是指在切开胸壁组织后,直视下取材。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "开胸活检" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "开胸活检" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "胸壁" } ] }, { "text": "近年来随着胸腔镜、纤维支气管镜的大力开展,及细针肺穿刺活检技术的应用,单纯开胸肺活检已较少采用。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "equ", "entity": "胸腔镜" }, { "start_idx": 9, "end_idx": 14, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 22, "end_idx": 28, "type": "pro", "entity": "细针肺穿刺活检" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "开胸肺活检" } ] }, { "text": "对经上述非创伤或微创伤性检查方法无法确诊者,尤其是罕见肺部疾病的诊断或可能需手术切除病灶者可考虑采用。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "pro", "entity": "非创伤或微创伤性检查" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "肺部疾病" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "手术" } ] }, { "text": "三、抗病毒治疗(一)抗病毒药物的选择性作用位点已知病毒复制过程包括:①病毒吸附(absorption):与靶细胞接触并吸附至细胞膜(存在病毒的特殊受体)。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "抗病毒治疗" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "抗病毒药物" }, { "start_idx": 25, "end_idx": 26, "type": "mic", "entity": "病毒" }, { "start_idx": 35, "end_idx": 36, "type": "mic", "entity": "病毒" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "靶细胞" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "细胞膜" }, { "start_idx": 68, "end_idx": 69, "type": "mic", "entity": "病毒" } ] }, { "text": "②穿入(penetration)细胞和脱衣壳(uncoating):病毒一般通过胞饮、吞噬作用或直接穿入而进入细胞。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "细胞" }, { "start_idx": 34, "end_idx": 35, "type": "mic", "entity": "病毒" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "细胞" } ] }, { "text": "某些有包膜的病毒则经与细胞膜融合穿入。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "病毒" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "细胞膜" } ] }, { "text": "③细胞内增殖:病毒增殖是一个极其复杂的过程,其中很多环节都有宿主细胞酶的参与。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "细胞" }, { "start_idx": 7, "end_idx": 8, "type": "mic", "entity": "病毒" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "宿主细胞酶" } ] }, { "text": "另外,病毒基因组类型众多,有DNA或RNA、单股或双股、环状、线状或分节状,各类病毒的核酸复制常有各自独特的形式。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "病毒" }, { "start_idx": 40, "end_idx": 41, "type": "mic", "entity": "病毒" } ] }, { "text": "④病毒装配、成熟和子代病毒释出:此过程涉及基因组和病毒结构蛋白的装配。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "mic", "entity": "病毒" }, { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "病毒" }, { "start_idx": 25, "end_idx": 30, "type": "mic", "entity": "病毒结构蛋白" } ] }, { "text": "理论上认为,病毒复制过程中任何一个环节均可作为抗病毒治疗的靶点。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "病毒" }, { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "抗病毒治疗" } ] }, { "text": "一种安全、有效的抗病毒药物必须具备以下特性:能到达靶细胞,在细胞内外均有活性,代谢过程稳定和抑制病毒增殖时不损害宿主细胞功能。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "抗病毒药物" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "靶细胞" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "细胞" }, { "start_idx": 48, "end_idx": 49, "type": "mic", "entity": "病毒" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "宿主细胞" } ] }, { "text": "由于病毒具有严格的细胞内寄生特性,复制时依赖宿主细胞的多种功能,做到两全其美十分困难。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "病毒" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "细胞" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "宿主细胞" } ] }, { "text": "迄今为止,特异、有效的抗病毒药物仍然很少,1999年美国市场上销售的抗病毒药物就只有24种。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "抗病毒药物" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "抗病毒药物" } ] }, { "text": "(二)抗病毒药物的种类1.化学制剂(1)核苷类似物:其结构与核酸前体类似,可竞争性抑制核酸合成酶,并可中断核苷酸链的延长而阻止病毒核酸合成。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "抗病毒药物" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "核苷类似物" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "核酸前体" }, { "start_idx": 63, "end_idx": 66, "type": "mic", "entity": "病毒核酸" } ] }, { "text": "嘌呤和嘧啶类核苷都是先在细胞内转化成三磷酸盐而活化。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "嘌呤和嘧啶类核苷" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "细胞" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "三磷酸盐" } ] }, { "text": "由于病毒DNA聚合酶对核苷三磷酸有选择性,病毒激酶参与其磷酸化,故有较高的选择性和安全性。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "mic", "entity": "病毒DNA聚合酶" }, { "start_idx": 21, "end_idx": 24, "type": "mic", "entity": "病毒激酶" } ] }, { "text": "(2)非核苷类似物:是一类对HIV-1的反转录酶有高度专一抑制作用的非竞争性抑制剂,易发生耐药,需联合或交替用药。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "非核苷类似物" }, { "start_idx": 14, "end_idx": 23, "type": "mic", "entity": "HIV-1的反转录酶" }, { "start_idx": 49, "end_idx": 55, "type": "pro", "entity": "联合或交替用药" } ] }, { "text": "(3)硫酸化多糖:如硫酸葡聚糖、硫酸右旋糖酐和肝素等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "硫酸化多糖" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "硫酸葡聚糖" }, { "start_idx": 16, "end_idx": 21, "type": "dru", "entity": "硫酸右旋糖酐" }, { "start_idx": 23, "end_idx": 24, "type": "dru", "entity": "肝素" } ] }, { "text": "具抑制HIV作用,其机制可能是干扰病毒吸附和抑制反转录酶活性。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "mic", "entity": "HIV" }, { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "病毒" }, { "start_idx": 24, "end_idx": 27, "type": "mic", "entity": "反转录酶" } ] }, { "text": "(4)取代嘧啶酮类化合物具较强的抗病毒和干扰素诱导活性。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dru", "entity": "取代嘧啶酮类化合物" }, { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "病毒" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "干扰素" } ] }, { "text": "但肾毒性大,需进一步进行结构改造。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肾" } ] }, { "text": "(5)其他:如金刚烷胺和金刚乙胺,作用于流感病毒M2蛋白而阻断病毒的穿入和脱衣壳。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "金刚烷胺" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "金刚乙胺" }, { "start_idx": 20, "end_idx": 27, "type": "mic", "entity": "流感病毒M2蛋白" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "病毒" } ] }, { "text": "神经氨酸酶抑制剂(zanamivir和oseltamivir)经抑制流感病毒的神经氨酸酶而阻止病毒释出和播散。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dru", "entity": "神经氨酸酶抑制剂" }, { "start_idx": 9, "end_idx": 17, "type": "dru", "entity": "zanamivir" }, { "start_idx": 19, "end_idx": 29, "type": "dru", "entity": "oseltamivir" }, { "start_idx": 34, "end_idx": 37, "type": "mic", "entity": "流感病毒" }, { "start_idx": 39, "end_idx": 43, "type": "mic", "entity": "神经氨酸酶" }, { "start_idx": 47, "end_idx": 48, "type": "mic", "entity": "病毒" } ] }, { "text": "膦甲酸为DNA聚合酶和反转录酶抑制剂,可抑制疱疹类病毒和HIV-1。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "膦甲酸" }, { "start_idx": 4, "end_idx": 17, "type": "dru", "entity": "DNA聚合酶和反转录酶抑制剂" }, { "start_idx": 22, "end_idx": 26, "type": "mic", "entity": "疱疹类病毒" }, { "start_idx": 28, "end_idx": 32, "type": "mic", "entity": "HIV-1" } ] }, { "text": "其他还有作用于HIV的蛋白酶抑制剂。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "HIV" }, { "start_idx": 11, "end_idx": 16, "type": "dru", "entity": "蛋白酶抑制剂" } ] }, { "text": "2.生物因子干扰素是最常用的生物因子,已证实长疗程大剂量治疗慢性丙型肝炎和乙型肝炎有效。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "干扰素" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "慢性丙型肝炎" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "乙型肝炎" } ] }, { "text": "人工合成或克隆表达的可溶性CD4蛋白(sCD4)可阻断HIV与靶细胞结合而保护正常细胞。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dru", "entity": "可溶性CD4蛋白" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "sCD4" }, { "start_idx": 27, "end_idx": 29, "type": "mic", "entity": "HIV" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "靶细胞" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "细胞" } ] }, { "text": "免疫球蛋白中和病毒,可阻断病毒与细胞结合,如狂犬病时联合应用狂犬病病毒免疫球蛋白和狂犬疫苗可降低病死率。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 7, "end_idx": 8, "type": "mic", "entity": "病毒" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "病毒" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "细胞" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "狂犬病" }, { "start_idx": 30, "end_idx": 39, "type": "dru", "entity": "狂犬病病毒免疫球蛋白" }, { "start_idx": 41, "end_idx": 44, "type": "dru", "entity": "狂犬疫苗" } ] }, { "text": "3.治疗性疫苗(1)HSV治疗性疫苗:用重组表达的HSV-2包膜抗原gD2加佐剂用于生殖道疱疹患者,可使生殖道疱疹的复发率和严重病损明显降低。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "治疗性疫苗" }, { "start_idx": 10, "end_idx": 17, "type": "dru", "entity": "HSV治疗性疫苗" }, { "start_idx": 25, "end_idx": 36, "type": "mic", "entity": "HSV-2包膜抗原gD2" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "生殖道疱疹" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "生殖道疱疹" } ] }, { "text": "(2)HIV治疗性疫苗:用重组HIV包膜抗原gp160加佐剂可使HIV感染者产生特异性抗体和细胞免疫。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dru", "entity": "HIV治疗性疫苗" }, { "start_idx": 13, "end_idx": 26, "type": "mic", "entity": "重组HIV包膜抗原gp160" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "HIV感染" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "特异性抗体" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "细胞" } ] }, { "text": "(3)HBV治疗性疫苗:包括重组含preS2疫苗、抗原-抗体复合物型疫苗、核心抗原合成肽疫苗和DNA疫苗,经诱导机体特异性免疫应答而清除体内HBV病毒。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dru", "entity": "HBV治疗性疫苗" }, { "start_idx": 17, "end_idx": 23, "type": "dru", "entity": "preS2疫苗" }, { "start_idx": 25, "end_idx": 35, "type": "dru", "entity": "抗原-抗体复合物型疫苗" }, { "start_idx": 43, "end_idx": 45, "type": "dru", "entity": "肽疫苗" }, { "start_idx": 47, "end_idx": 51, "type": "dru", "entity": "DNA疫苗" }, { "start_idx": 70, "end_idx": 74, "type": "mic", "entity": "HBV病毒" } ] }, { "text": "(4)核酶:是一类具双重特性的RNA分子,能与靶RNA序列特异结合,并能切割降解靶RNA。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "核酶" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "靶RNA" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "靶RNA" } ] }, { "text": "目前已研究设计针对HIV、肝炎病毒、流感病毒等的核酶,显示其潜在应用价值。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "HIV" }, { "start_idx": 13, "end_idx": 16, "type": "mic", "entity": "肝炎病毒" }, { "start_idx": 18, "end_idx": 21, "type": "mic", "entity": "流感病毒" }, { "start_idx": 24, "end_idx": 25, "type": "dru", "entity": "核酶" } ] }, { "text": "(5)反义核苷酸:包括反义寡脱氧核苷酸(asODN)和反义寡核苷酸(asON),主要通过与病毒关键DNA或RNA结合,抑制或阻断其复制与转录;与mRNA结合形成二聚体,致其被核酶降解,阻断蛋白质合成。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "反义核苷酸" }, { "start_idx": 11, "end_idx": 18, "type": "dru", "entity": "反义寡脱氧核苷酸" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "asODN" }, { "start_idx": 27, "end_idx": 32, "type": "dru", "entity": "反义寡核苷酸" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "asON" }, { "start_idx": 45, "end_idx": 46, "type": "mic", "entity": "病毒" }, { "start_idx": 87, "end_idx": 88, "type": "dru", "entity": "核酶" } ] }, { "text": "(6)抗病毒中药:已发现一些中药有抗病毒效应,如大蒜新素在体内外均具抗HCMV活性。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "抗病毒中药" }, { "start_idx": 14, "end_idx": 15, "type": "dru", "entity": "中药" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "病毒" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "大蒜新素" }, { "start_idx": 35, "end_idx": 38, "type": "mic", "entity": "HCMV" } ] }, { "text": "有报道中药XQ9302治疗晚期艾滋病可缓解症状和增加CD4细胞。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dru", "entity": "中药XQ9302" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "晚期艾滋病" } ] }, { "text": "中药是中国乃至世界医学的宝库,应积极开发利用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "中药" } ] }, { "text": "(三)合理使用抗病毒药物的原则和有关问题1.正确诊断病毒性疾病由于每种药物的设计都是特异性抑制某种病毒的复制,故在使用药物之前,应确诊感染的病毒,方法详见本章第一节。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "抗病毒药物" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "病毒性疾病" }, { "start_idx": 49, "end_idx": 50, "type": "mic", "entity": "病毒" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "感染" }, { "start_idx": 70, "end_idx": 71, "type": "mic", "entity": "病毒" } ] }, { "text": "2.合理选用抗病毒药物首先要掌握所选药物的作用机制和其敏感病毒谱。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dru", "entity": "抗病毒药物" }, { "start_idx": 29, "end_idx": 30, "type": "mic", "entity": "病毒" } ] }, { "text": "由于已用于临床的抗病毒药物都具不同程度的毒副作用,故而即使正确诊断出病毒性疾病,亦应根据病情和患者的基本状况,权衡利弊后合理选用药物。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "病毒" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "病毒性疾病" } ] }, { "text": "(1)轻症不必给予毒副作用大的药物:例如,婴儿无黄疸型HCMV肝炎肝损害较轻,一般给予护肝等对症治疗都能恢复,预后佳。", "entities": [ { "start_idx": 24, "end_idx": 32, "type": "dis", "entity": "黄疸型HCMV肝炎" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "肝" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "肝损害" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "护肝" }, { "start_idx": 46, "end_idx": 49, "type": "pro", "entity": "对症治疗" } ] }, { "text": "对这类患者就不必选用具有骨髓毒性的更昔洛韦治疗,以免造成不必要的医源性损伤。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "骨髓" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "更昔洛韦" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "医源性损伤" } ] }, { "text": "实际上,核苷类似物是病毒增殖的抑制剂,停药后抑制效应常随之消失,很难根除慢性感染的细胞内病毒。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "核苷类似物" }, { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "病毒" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "慢性感染" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "细胞" }, { "start_idx": 44, "end_idx": 45, "type": "mic", "entity": "病毒" } ] }, { "text": "例如,应用更昔洛韦时不宜同时使用其他骨髓毒性药物,包括AZT。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "更昔洛韦" }, { "start_idx": 18, "end_idx": 23, "type": "dru", "entity": "骨髓毒性药物" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "AZT" } ] }, { "text": "此外,患者已有相应功能障碍如肾功能不全时,不宜使用肾毒性抗病毒药物或应减量使用。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肾功能不全" } ] }, { "text": "(3)严格按照适应证用药:例如拉米呋定只对复制状态的HBV有抑制作用,即对慢性乙型肝炎、血清HBVDNA和HBeAg阳性患者有一定疗效。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "拉米呋定" }, { "start_idx": 26, "end_idx": 28, "type": "mic", "entity": "HBV" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "慢性乙型肝炎" }, { "start_idx": 44, "end_idx": 51, "type": "dis", "entity": "血清HBVDNA" }, { "start_idx": 53, "end_idx": 59, "type": "dis", "entity": "HBeAg阳性" } ] }, { "text": "而对慢性HBV感染非复制期(HBVDNA和HBeAg阴性)病毒无明显作用,对失代偿肝硬化者疗效差。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "慢性HBV感染" }, { "start_idx": 14, "end_idx": 27, "type": "dis", "entity": "HBVDNA和HBeAg阴性" }, { "start_idx": 29, "end_idx": 30, "type": "mic", "entity": "病毒" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "失代偿肝硬化" } ] }, { "text": "3.严密监测和及时处理毒副作用首先要求了解和熟悉各种药物可能发生的毒副作用;其次在用药前应了解患者相应器官功能如肝、肾功能等;再次,在用药期间需严密监测,如出现严重毒副作用时,一般宜停用药物。", "entities": [ { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "肝" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "肾" } ] }, { "text": "毒副作用发生时要及时处理,如α干扰素易致粒细胞减少,及时加服或加大复方阿胶浆用量,可有效提高粒细胞水平而不必中止治疗。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "α干扰素" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "粒细胞" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "粒细胞减少" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "复方阿胶浆" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "粒细胞" } ] }, { "text": "这种状况多与临床医生未能了解药物的抗病毒机制和适应证有关,例如,阿昔洛韦投放市场后,有医生将其用于治疗感冒、肺炎等呼吸道感染;更昔洛韦问世后,又如法炮制,当成广谱抗病毒药使用。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "病毒" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "阿昔洛韦" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "感冒" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "肺炎" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 63, "end_idx": 66, "type": "dru", "entity": "更昔洛韦" }, { "start_idx": 82, "end_idx": 83, "type": "mic", "entity": "病毒" } ] }, { "text": "事实上,这2种药物均依赖疱疹类病毒胸苷激酶的磷酸化作用而活化,故只对这些病毒起作用,而对引起呼吸道感染的常见病毒如流感病毒、鼻病毒、腺病毒等无抑制效应。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "mic", "entity": "疱疹类病毒" }, { "start_idx": 36, "end_idx": 37, "type": "mic", "entity": "病毒" }, { "start_idx": 46, "end_idx": 50, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 54, "end_idx": 55, "type": "mic", "entity": "病毒" }, { "start_idx": 57, "end_idx": 60, "type": "mic", "entity": "流感病毒" }, { "start_idx": 62, "end_idx": 64, "type": "mic", "entity": "鼻病毒" }, { "start_idx": 66, "end_idx": 68, "type": "mic", "entity": "腺病毒" } ] }, { "text": "再如干扰素理论上有广谱抗病毒作用,但实际上并非如此。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "干扰素" }, { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "病毒" } ] }, { "text": "实践证明,干扰素对HCMV感染无效,但临床上就有医生对HCMV感染患儿长期使用干扰素。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "干扰素" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "HCMV感染" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "HCMV感染" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "干扰素" } ] }, { "text": "滥用抗病毒药物对病儿百害而无一利,不仅造成浪费、对患儿产生医源性损害,还会诱生耐药毒株。", "entities": [ { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "医源性损害" } ] }, { "text": "有些药物可致基因突变,有潜在致癌的危险。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "基因突变" }, { "start_idx": 15, "end_idx": 15, "type": "dis", "entity": "癌" } ] }, { "text": "5.正确评估抗病毒药物的疗效对抗病毒药物疗效的评估应包括两个方面:一方面是临床疗效评估,另一方面是病毒学评估。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "抗病毒药物" }, { "start_idx": 49, "end_idx": 50, "type": "mic", "entity": "病毒" } ] }, { "text": "而后者尤为重要,是抗病毒效应最为可靠的评价指标。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "病毒" } ] }, { "text": "其方法有:①病毒分离或空斑试验计算病毒含量;②病毒基因定性和定量分析;③病毒抗原定性和半定量分析。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "病毒" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "空斑试验" }, { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "病毒" }, { "start_idx": 23, "end_idx": 33, "type": "pro", "entity": "病毒基因定性和定量分析" }, { "start_idx": 36, "end_idx": 47, "type": "pro", "entity": "病毒抗原定性和半定量分析" } ] }, { "text": "病毒特异性IgM抗体不宜作为观察指标,因为特异性IgM水平除与病毒增殖状况有关外,还受机体免疫反应强弱和抗体降解速率等因素的影响,而与病毒量无明显关系。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "病毒特异性IgM抗体" }, { "start_idx": 21, "end_idx": 28, "type": "ite", "entity": "特异性IgM水平" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "病毒" }, { "start_idx": 67, "end_idx": 68, "type": "mic", "entity": "病毒" } ] }, { "text": "第十一节神经源性膀胱神经源性膀胱(neurogenicbladder)是由于中枢或周围神经部分或完全性损害造成的膀胱、尿道贮尿或/和排尿功能障碍,也可将其称之为下尿路功能障碍。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 17, "end_idx": 33, "type": "dis", "entity": "neurogenicbladder" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "中枢" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "周围神经" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "膀胱" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "尿道" }, { "start_idx": 66, "end_idx": 71, "type": "dis", "entity": "排尿功能障碍" }, { "start_idx": 81, "end_idx": 86, "type": "dis", "entity": "尿路功能障碍" } ] }, { "text": "在小儿泌尿外科,至少有25%的临床问题是由于先天性神经损害影响到下尿路功能造成的。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dep", "entity": "小儿泌尿外科" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "先天性神经损害" } ] }, { "text": "其原因主要为脊髓发育不良,包括脊髓脊膜膨出、脊髓栓系、脊髓纵裂及腰骶椎发育不良等;其他原因包括外伤、肿瘤、经骶尾部或盆腔的手术、神经系统炎症和VATER综合征等。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "脊髓发育不良" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "脊髓脊膜膨出" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "脊髓栓系" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "脊髓纵裂" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "腰骶椎发育不良" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "肿瘤" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "骶尾部" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "盆腔" }, { "start_idx": 64, "end_idx": 69, "type": "dis", "entity": "神经系统炎症" }, { "start_idx": 71, "end_idx": 78, "type": "dis", "entity": "VATER综合征" } ] }, { "text": "临床上常可能仅注重对神经系统病变或原发病的治疗而忽视了神经源性膀胱所造成的影响,尤其在膀胱充盈期压力长期增高,引起膀胱输尿管反流可危害到患儿的肾功能,甚至导致肾衰竭。", "entities": [ { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 55, "end_idx": 73, "type": "sym", "entity": "引起膀胱输尿管反流可危害到患儿的肾功能" }, { "start_idx": 75, "end_idx": 81, "type": "sym", "entity": "甚至导致肾衰竭" } ] }, { "text": "【分类】在泌尿外科疾病中,神经源性膀胱是分类方法最多,也是最复杂和概念最混淆的一种疾病,迄今为止还没有一种分类能将神经源性膀胱的解剖、生理、病理和症状特点结合统一。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dep", "entity": "泌尿外科" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "另外,根据尿流动力学进行分类也越来越受到关注和应用,它在评价膀胱逼尿肌和尿道括约肌情况的同时可综合考虑其相互关系,将膀胱和尿道分为正常、亢进及无反射三类的同时又分为膀胱逼尿肌与尿道括约肌协调、不协调两种情况。", "entities": [ { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "膀胱逼尿肌" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "尿道括约肌" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "膀胱" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "尿道" }, { "start_idx": 65, "end_idx": 66, "type": "dis", "entity": "正常" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "亢进" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "无反射" }, { "start_idx": 82, "end_idx": 86, "type": "dis", "entity": "膀胱逼尿肌" }, { "start_idx": 88, "end_idx": 94, "type": "dis", "entity": "尿道括约肌协调" }, { "start_idx": 96, "end_idx": 98, "type": "dis", "entity": "不协调" } ] }, { "text": "【诊断】小儿神经源性膀胱的病因主要为先天性的,故大多可于生后早期发现,而如外伤等获得性者也可通过病史得出结论。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "小儿神经源性膀胱" } ] }, { "text": "虽然尿流动力学检查十分重要,但常常由于患儿无法合作或在麻醉下进行以至无法获得完全而真实的评价,因此常需要通过详细的病史询问和体格检查、完善的实验室检查和影像学检查以及一些必要的特殊检查来弥补上述不足。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "麻醉" } ] }, { "text": "(一)病史及体格检查脊髓脊膜膨出等先天性脊髓发育不良和椎管发育不全常导致患儿生后出现排尿(排便)异常,脊柱表面皮肤病变,下肢、足畸形和步态异常等情况,在新生儿或婴幼儿往往多因后两种情况而就诊;在大年龄患儿,通常在可控制大小便和行走的阶段出现异常而被家长发现。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "体格检查" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "脊髓脊膜膨出" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "先天性脊髓发育不良" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "椎管发育不全" }, { "start_idx": 51, "end_idx": 58, "type": "sym", "entity": "脊柱表面皮肤病变" }, { "start_idx": 60, "end_idx": 73, "type": "sym", "entity": "下肢、足畸形和步态异常等情况" } ] }, { "text": "脊柱尤其是骶尾部的体格检查可了解患儿有无脊柱裂等椎骨发育异常;神经系统的检查如马鞍区感觉减退或消失,肛门括约肌反射和球海绵体肌反射亢进、减退或消失等都有助于对患儿神经损害的程度和定位进行评价。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脊柱" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "骶尾部" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "体格检查" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "脊柱裂" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "椎骨发育异常" }, { "start_idx": 31, "end_idx": 37, "type": "pro", "entity": "神经系统的检查" }, { "start_idx": 39, "end_idx": 48, "type": "sym", "entity": "马鞍区感觉减退或消失" }, { "start_idx": 50, "end_idx": 72, "type": "sym", "entity": "肛门括约肌反射和球海绵体肌反射亢进、减退或消失" } ] }, { "text": "病史和体格检查除可对患儿的情况提供一个概括外,还具有以下的作用:①对可能与大小便功能有关的病史作出鉴别,如遗尿史及患儿的排尿类型等;②对神经系统损害进行定位以便证实、解释尿流动力学检查结果;③了解患儿的预后可能,为治疗方案提供参考。", "entities": [ { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "遗尿" }, { "start_idx": 68, "end_idx": 73, "type": "dis", "entity": "神经系统损害" }, { "start_idx": 85, "end_idx": 91, "type": "ite", "entity": "尿流动力学检查" } ] }, { "text": "(二)实验室检查神经源性膀胱最常见的并发症为尿路感染,但症状多不典型,如合并膀胱输尿管反流则可能加速肾脏的损害;在新生儿或婴幼儿可能导致水电解质紊乱、酸碱平衡失调以及肾衰竭等威胁患儿生命。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "尿路感染" }, { "start_idx": 36, "end_idx": 54, "type": "sym", "entity": "合并膀胱输尿管反流则可能加速肾脏的损害" }, { "start_idx": 56, "end_idx": 92, "type": "sym", "entity": "在新生儿或婴幼儿可能导致水电解质紊乱、酸碱平衡失调以及肾衰竭等威胁患儿生命" } ] }, { "text": "(三)影像学检查X线脊柱平片检查可发现脊柱畸形(如椎体畸形)和脊柱裂。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "影像学检查" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "X线脊柱平片" }, { "start_idx": 19, "end_idx": 33, "type": "sym", "entity": "脊柱畸形(如椎体畸形)和脊柱裂" } ] }, { "text": "排尿性膀胱尿道造影多可发现典型的“圣诞树”样膀胱,可了解充盈期膀胱的形态和容量,是否存在膀胱输尿管反流及反流的程度;在排尿时观察尿道和膀胱颈部开放的情况,对是否存在逼尿肌和括约肌不协调具有一定的诊断意义,并可排除后尿道瓣膜等先天性下尿路梗阻性疾病。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "排尿性膀胱尿道造影" }, { "start_idx": 11, "end_idx": 23, "type": "sym", "entity": "发现典型的“圣诞树”样膀胱" }, { "start_idx": 42, "end_idx": 56, "type": "sym", "entity": "存在膀胱输尿管反流及反流的程度" }, { "start_idx": 59, "end_idx": 75, "type": "ite", "entity": "排尿时观察尿道和膀胱颈部开放的情况" }, { "start_idx": 82, "end_idx": 91, "type": "sym", "entity": "逼尿肌和括约肌不协调" }, { "start_idx": 106, "end_idx": 110, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 112, "end_idx": 122, "type": "dis", "entity": "先天性下尿路梗阻性疾病" } ] }, { "text": "B超因其无创伤性,多用于了解上尿路情况以及患儿的长期随访。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "B超" } ] }, { "text": "(四)尿流动力学检查包括膀胱测压、外括约肌肌电测定、尿道压力测定和尿流率测定等。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "尿流动力学检查" }, { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "膀胱测压" }, { "start_idx": 17, "end_idx": 24, "type": "ite", "entity": "外括约肌肌电测定" }, { "start_idx": 26, "end_idx": 31, "type": "ite", "entity": "尿道压力测定" }, { "start_idx": 33, "end_idx": 37, "type": "ite", "entity": "尿流率测定" } ] }, { "text": "在检查方法上有常规尿流动力学检查、影像尿流动力学检查以及动态尿流动力学检查等。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "ite", "entity": "常规尿流动力学检查" }, { "start_idx": 17, "end_idx": 25, "type": "pro", "entity": "影像尿流动力学检查" }, { "start_idx": 28, "end_idx": 36, "type": "ite", "entity": "动态尿流动力学检查" } ] }, { "text": "除尿流率测定外,作为一种非自然的膀胱尿道功能的刺激性检查,尿流动力学检查有一定的局限性,但其某些客观的发现,如膀胱存在无抑制性收缩以及括约肌有去神经支配的肌电图表现等情况,仍可为临床提供重要的诊断资料,并且它对于神经源性膀胱的分类及治疗有一定的指导意义。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "ite", "entity": "尿流率测定" }, { "start_idx": 12, "end_idx": 27, "type": "pro", "entity": "非自然的膀胱尿道功能的刺激性检查" }, { "start_idx": 29, "end_idx": 35, "type": "pro", "entity": "尿流动力学检查" }, { "start_idx": 55, "end_idx": 84, "type": "sym", "entity": "膀胱存在无抑制性收缩以及括约肌有去神经支配的肌电图表现等情况" }, { "start_idx": 106, "end_idx": 111, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "对于小儿的尿流动力学检查,还应考虑到其下尿路神经支配的发育程度,以防止得出片面的结论。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "ite", "entity": "小儿的尿流动力学检查" } ] }, { "text": "【治疗】正如尚无单一的分类一样,迄今为止对神经源性膀胱的治疗也没有一种简便而单一的方法,常需要进行个体化的综合治疗,即不同患儿的治疗方案因病情可以完全不同,但又存在着一些根本的治疗原则:①保护肾功能;②防止及控制尿路感染;③尽量避免留置导尿和尿流改道;④尽可能实现既能控制失禁又能基本排空膀胱和尿道以改善患儿的生活质量。", "entities": [ { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 94, "end_idx": 98, "type": "pro", "entity": "保护肾功能" }, { "start_idx": 101, "end_idx": 109, "type": "pro", "entity": "防止及控制尿路感染" }, { "start_idx": 112, "end_idx": 124, "type": "pro", "entity": "尽量避免留置导尿和尿流改道" }, { "start_idx": 127, "end_idx": 158, "type": "pro", "entity": "尽可能实现既能控制失禁又能基本排空膀胱和尿道以改善患儿的生活质量" } ] }, { "text": "同时,患儿及其家长对治疗的理解和配合也是治疗成功与否的关键,应对以下情况进行充分的了解,以合理选用治疗方案:①患儿的意识和智力情况;②患儿是否具有治疗所需的能力,如是否能自行进行间歇性清洁导尿或排尿训练等;③患儿和家长对神经源性膀胱及选用治疗方案的理解;④患儿及家长的合作程度及其经济能力等。", "entities": [ { "start_idx": 89, "end_idx": 100, "type": "pro", "entity": "间歇性清洁导尿或排尿训练" }, { "start_idx": 110, "end_idx": 115, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "另外,也可将众多的治疗方法分为非手术和手术治疗两类。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "非手术" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "手术治疗" } ] }, { "text": "(一)非手术治疗1.导尿术以导尿管引流尿液,是一种有长久历史的治疗手段,具有操作简便、效果好及经济等优点,至今仍被普遍应用。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "非手术治疗" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "导尿术" }, { "start_idx": 14, "end_idx": 16, "type": "equ", "entity": "导尿管" } ] }, { "text": "主要包括留置导尿、无菌性间歇性导尿和间歇性清洁导尿(clearintermittentcatheterization,CIC)。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "留置导尿" }, { "start_idx": 9, "end_idx": 16, "type": "pro", "entity": "无菌性间歇性导尿" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "间歇性清洁导尿" }, { "start_idx": 26, "end_idx": 57, "type": "pro", "entity": "clearintermittentcatheterization" }, { "start_idx": 59, "end_idx": 61, "type": "pro", "entity": "CIC" } ] }, { "text": "由于传统的留置导尿法常易引起尿路感染,故多数情况下,主张首选间歇性清洁导尿。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "留置导尿法" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "尿路感染" }, { "start_idx": 30, "end_idx": 36, "type": "pro", "entity": "间歇性清洁导尿" } ] }, { "text": "(1)间歇性清洁导尿和无菌性间歇性导尿:对于任何不能自行排空膀胱的患儿均可考虑,其适应证为:①暂时性或永久性尿潴留;②逼尿肌反射亢进及膀胱逼尿肌、尿道外括约肌不协调;③有手术禁忌的膀胱出口梗阻者。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "间歇性清洁导尿" }, { "start_idx": 11, "end_idx": 18, "type": "pro", "entity": "无菌性间歇性导尿" }, { "start_idx": 47, "end_idx": 56, "type": "sym", "entity": "暂时性或永久性尿潴留" }, { "start_idx": 59, "end_idx": 81, "type": "sym", "entity": "逼尿肌反射亢进及膀胱逼尿肌、尿道外括约肌不协调" }, { "start_idx": 84, "end_idx": 96, "type": "sym", "entity": "有手术禁忌的膀胱出口梗阻者" } ] }, { "text": "尿道畸形、严重尿道炎和尿道周围脓肿则为其禁忌证。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尿道畸形" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "严重尿道炎" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "尿道周围脓肿" } ] }, { "text": "有实验证明正常的膀胱具有抗感染能力,膀胱黏膜的完整和膀胱自身的排空是机体的一种防御机制,因此,即使因导尿管或导尿过程中可能使细菌进入膀胱,也完全可以依赖这一机制以及间歇性导尿(如同形成正常的排尿周期)加以消灭或清除,从而最大程度地避免尿路感染。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "膀胱" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "膀胱黏膜" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "膀胱" }, { "start_idx": 50, "end_idx": 52, "type": "equ", "entity": "导尿管" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "导尿" }, { "start_idx": 62, "end_idx": 63, "type": "mic", "entity": "细菌" }, { "start_idx": 82, "end_idx": 86, "type": "pro", "entity": "间歇性导尿" }, { "start_idx": 117, "end_idx": 120, "type": "dis", "entity": "尿路感染" } ] }, { "text": "尽管无菌性间歇性导尿略优于间歇性清洁导尿,但相对而言后者更为简单方便,尤其适于需长期在家导尿又无法达到无菌要求的患儿。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "无菌性间歇性导尿" }, { "start_idx": 13, "end_idx": 19, "type": "pro", "entity": "间歇性清洁导尿" } ] }, { "text": "间歇性清洁导尿方法为:使用一次性消毒或普通的导尿管(后者每次用前开水煮沸20分钟,用后及时洗净置于干净容器内),每次导尿前用肥皂和清水洗净手和阴部,男孩需使用润滑剂,大年龄的女孩如能自行导尿则借助镜子或感觉,一手将阴唇分开,另一手插导尿管。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "间歇性清洁导尿方法" }, { "start_idx": 22, "end_idx": 24, "type": "equ", "entity": "导尿管" }, { "start_idx": 107, "end_idx": 108, "type": "bod", "entity": "阴唇" }, { "start_idx": 116, "end_idx": 118, "type": "equ", "entity": "导尿管" } ] }, { "text": "(2)留置导尿术:虽然多数情况下间歇性导尿要优于留置导尿,但下列情况应考虑留置导尿:①低压性膀胱输尿管反流;②严重的双侧上尿路积水;③伴有膀胱输尿管反流的急性重症肾盂肾炎;④患儿不合作。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "留置导尿术" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "间歇性导尿" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "留置导尿" }, { "start_idx": 43, "end_idx": 52, "type": "dis", "entity": "低压性膀胱输尿管反流" }, { "start_idx": 58, "end_idx": 64, "type": "dis", "entity": "双侧上尿路积水" }, { "start_idx": 67, "end_idx": 84, "type": "dis", "entity": "伴有膀胱输尿管反流的急性重症肾盂肾炎" } ] }, { "text": "留置导尿的适应证还包括:①重症和虚弱患儿存在膀胱排空不完全、完全性尿潴留或尿失禁;②可进行间歇性导尿但因某些原因无法实施者。", "entities": [ { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "完全性尿潴留" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "尿失禁" } ] }, { "text": "留置导尿期间需预防以下并发症:①尿路感染;②膀胱结石;③膀胱挛缩;④血尿;⑤导尿管堵塞等。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "尿路感染" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "膀胱结石" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "膀胱挛缩" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "血尿" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "导尿管堵塞" } ] }, { "text": "2.药物治疗神经源性膀胱的药物应用完全是以膀胱、尿道神经支配、自主神经受体分布和药物对膀胱尿道平滑肌作用为基础的,基本可分为兴奋或抑制膀胱逼尿肌、兴奋或抑制膀胱颈和后尿道的药物两大类。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "膀胱" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "尿道神经支配" }, { "start_idx": 31, "end_idx": 36, "type": "bod", "entity": "自主神经受体" }, { "start_idx": 43, "end_idx": 49, "type": "bod", "entity": "膀胱尿道平滑肌" }, { "start_idx": 62, "end_idx": 71, "type": "dru", "entity": "兴奋或抑制膀胱逼尿肌" }, { "start_idx": 73, "end_idx": 87, "type": "dru", "entity": "兴奋或抑制膀胱颈和后尿道的药物" } ] }, { "text": "药物可以是在下尿路存在或形成的、可影响膀胱尿道功能的神经递质,也可以是模拟、增强或抑制某一种或数种神经递质功能的物质,包括经典的神经递质如乙酰胆碱酰胆碱、去甲肾上腺素和组胺,以及其他非胆碱能非肾上腺素能递质如前列腺素、5-羟色胺、多巴胺、NO和P物质等神经肽。", "entities": [ { "start_idx": 19, "end_idx": 29, "type": "bod", "entity": "膀胱尿道功能的神经递质" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "神经递质" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "神经递质" }, { "start_idx": 69, "end_idx": 72, "type": "dru", "entity": "乙酰胆碱" }, { "start_idx": 77, "end_idx": 82, "type": "dru", "entity": "去甲肾上腺素" }, { "start_idx": 84, "end_idx": 85, "type": "dru", "entity": "组胺" }, { "start_idx": 91, "end_idx": 102, "type": "dru", "entity": "非胆碱能非肾上腺素能递质" }, { "start_idx": 104, "end_idx": 107, "type": "dru", "entity": "前列腺素" }, { "start_idx": 109, "end_idx": 113, "type": "dru", "entity": "5-羟色胺" }, { "start_idx": 115, "end_idx": 117, "type": "dru", "entity": "多巴胺" }, { "start_idx": 119, "end_idx": 120, "type": "dru", "entity": "NO" }, { "start_idx": 122, "end_idx": 124, "type": "dru", "entity": "P物质" }, { "start_idx": 126, "end_idx": 128, "type": "dru", "entity": "神经肽" } ] }, { "text": "药物治疗的主要根据是尿流动力学的检查结果,从应用的范围来看,主要是针对膀胱逼尿肌和尿道括约肌的。", "entities": [ { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "膀胱逼尿肌" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "尿道括约肌" } ] }, { "text": "逼尿肌反射亢进是临床碰到的最为常见的情况,可采用抗胆碱能药物以降低膀胱逼尿肌的收缩。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "逼尿肌反射亢进" }, { "start_idx": 24, "end_idx": 29, "type": "dru", "entity": "抗胆碱能药物" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "膀胱逼尿肌" } ] }, { "text": "抗胆碱能药物包括普鲁苯辛及颠茄合剂等;也可使用兼有抗胆碱及松弛平滑肌作用的药物如氯化羟丁宁和双环胺等。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "普鲁苯辛" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "颠茄合剂" }, { "start_idx": 25, "end_idx": 38, "type": "dru", "entity": "抗胆碱及松弛平滑肌作用的药物" }, { "start_idx": 40, "end_idx": 44, "type": "dru", "entity": "氯化羟丁宁" }, { "start_idx": 46, "end_idx": 48, "type": "dru", "entity": "双环胺" } ] }, { "text": "对伴有尿道平滑肌痉挛的逼尿肌反射亢进,可应用α受体阻滞剂酚苄明(松弛膀胱出口和尿道平滑肌),可单用或与抗胆碱能药物普鲁苯辛或与氯化羟丁宁合用。", "entities": [ { "start_idx": 1, "end_idx": 17, "type": "dis", "entity": "伴有尿道平滑肌痉挛的逼尿肌反射亢进" }, { "start_idx": 22, "end_idx": 30, "type": "dru", "entity": "α受体阻滞剂酚苄明" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "膀胱出口" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "尿道平滑肌" }, { "start_idx": 51, "end_idx": 60, "type": "dru", "entity": "抗胆碱能药物普鲁苯辛" }, { "start_idx": 63, "end_idx": 67, "type": "dru", "entity": "氯化羟丁宁" } ] }, { "text": "以达到减轻逼尿肌收缩和解除尿道平滑肌痉挛的作用。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "逼尿肌" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "尿道平滑肌" } ] }, { "text": "对伴有尿道外括约肌痉挛的逼尿肌反射亢进,为减轻逼尿肌收缩和解除尿道外括约肌痉挛,降低排尿阻力,可使用骨骼肌松弛剂如硝苯呋海因钠,多突触抑制剂如肼双二乙胺三嗪、氯苯氨丁酸和地西泮以及抗胆碱能药物。", "entities": [ { "start_idx": 3, "end_idx": 18, "type": "dis", "entity": "尿道外括约肌痉挛的逼尿肌反射亢进" }, { "start_idx": 50, "end_idx": 55, "type": "dru", "entity": "骨骼肌松弛剂" }, { "start_idx": 57, "end_idx": 62, "type": "dru", "entity": "硝苯呋海因钠" }, { "start_idx": 64, "end_idx": 69, "type": "dru", "entity": "多突触抑制剂" }, { "start_idx": 71, "end_idx": 77, "type": "dru", "entity": "肼双二乙胺三嗪" }, { "start_idx": 79, "end_idx": 83, "type": "dru", "entity": "氯苯氨丁酸" }, { "start_idx": 85, "end_idx": 87, "type": "dru", "entity": "地西泮" }, { "start_idx": 90, "end_idx": 95, "type": "dru", "entity": "抗胆碱能药物" } ] }, { "text": "膀胱逼尿肌反射低下或无反射,治疗的目的在于增加逼尿肌收缩,同时降低流出阻力。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "膀胱逼尿肌反射低下或无反射" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "逼尿肌" } ] }, { "text": "联合应用氨基甲酰甲基胆碱(拟胆碱药)和酚苄明可达此目的。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dru", "entity": "氨基甲酰甲基胆碱" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "拟胆碱药" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "酚苄明" } ] }, { "text": "前者单用效果不甚满意,因为氨基甲酰甲基胆碱能增强逼尿肌收缩,同时亦增加尿道平滑肌的收缩。", "entities": [ { "start_idx": 13, "end_idx": 21, "type": "dru", "entity": "氨基甲酰甲基胆碱能" } ] }, { "text": "加用酚苄明后流出阻力有效地降低,而又不改变增强了的逼尿肌收缩力,是一种极好地联合用药方法。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "酚苄明" } ] }, { "text": "丙咪嗪(Imipramine)是目前较为常用的药物,临床上主要用于小儿遗尿症,由于其具有抑制逼尿肌异常反射、增加膀胱容量以及增加尿道内压力的作用,也可治疗因无抑制收缩膀胱导致尿失禁的神经源性膀胱患儿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "丙咪嗪" }, { "start_idx": 4, "end_idx": 13, "type": "dru", "entity": "Imipramine" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "小儿遗尿症" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "逼尿肌" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "膀胱" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "尿道" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "尿失禁" }, { "start_idx": 91, "end_idx": 96, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "其缺点主要为可产生口干等胃肠道症状、易耐受或复发。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "胃肠道症状" } ] }, { "text": "近年来,有关神经肽在下尿路中的生理和病理作用越来越受到重视,有实验证明P物质不但作为痛觉和炎症的调节因子,还可通过特异性受体对下尿路平滑肌产生收缩作用,临床上也据此采用辣椒素(capsaicin)进行膀胱内灌注以耗竭P物质,减少逼尿肌收缩,改善逼尿肌反射亢进和膀胱过敏等症状,取得较好的疗效。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "神经肽" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "P物质" }, { "start_idx": 63, "end_idx": 68, "type": "bod", "entity": "下尿路平滑肌" }, { "start_idx": 84, "end_idx": 86, "type": "dru", "entity": "辣椒素" }, { "start_idx": 88, "end_idx": 96, "type": "dru", "entity": "capsaicin" }, { "start_idx": 108, "end_idx": 110, "type": "dru", "entity": "P物质" }, { "start_idx": 114, "end_idx": 116, "type": "bod", "entity": "逼尿肌" }, { "start_idx": 122, "end_idx": 128, "type": "dis", "entity": "逼尿肌反射亢进" }, { "start_idx": 130, "end_idx": 133, "type": "dis", "entity": "膀胱过敏" } ] }, { "text": "3.神经阻滞疗法骶神经根或阴部神经阻滞治疗的目的在于减轻或消除膀胱反射亢进以及减轻或消除外括约肌痉挛。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "神经阻滞疗法" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "骶神经根" }, { "start_idx": 26, "end_idx": 36, "type": "pro", "entity": "减轻或消除膀胱反射亢进" }, { "start_idx": 39, "end_idx": 49, "type": "pro", "entity": "减轻或消除外括约肌痉挛" } ] }, { "text": "由于技术要求高,定位难度大,易发生治疗后大便失禁和阳痿等并发症,且小儿难以合作等原因,目前神经阻滞治疗很少被使用,骶神经根阻滞术仅用于预测骶神经根切断的效果。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "大便失禁" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "阳痿" }, { "start_idx": 45, "end_idx": 50, "type": "pro", "entity": "神经阻滞治疗" }, { "start_idx": 57, "end_idx": 63, "type": "pro", "entity": "骶神经根阻滞术" } ] }, { "text": "4.其他方法排尿训练及生物反馈等方法旨在诱导、强化正确的感觉反馈,以形成反射性排尿,对神经源性膀胱患儿有一定的作用。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "排尿训练" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "生物反馈" } ] }, { "text": "(1)排尿训练1)“扳机点”排尿:通过用手摩擦大腿、轻叩耻骨上区等方法找到最易诱发膀胱收缩、括约肌松弛而引起排尿的刺激部位(扳机点),并配合以一定的姿势或动作增加排尿的强度。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "排尿训练" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "“扳机点”排尿" }, { "start_idx": 17, "end_idx": 85, "type": "pro", "entity": "通过用手摩擦大腿、轻叩耻骨上区等方法找到最易诱发膀胱收缩、括约肌松弛而引起排尿的刺激部位(扳机点),并配合以一定的姿势或动作增加排尿的强度" } ] }, { "text": "该方法适用于诱导逼尿肌反射亢进的患儿产生反射性排尿。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "逼尿肌反射亢进" } ] }, { "text": "2)Crede手法和Valsalva动作:对于因逼尿肌无反射或反射减退而无Crede手法,即嘱患儿取端坐位,以一手或二手四指压迫耻骨上区,持续压迫膀胱,增加膀胱内压使膀胱颈也可同时辅以Valsalva动作(用力吸气后屏住呼吸做呼气的动作),逼尿肌反射亢进为其禁忌证Kegel训练:目的在于增强盆底肌肉尤其是耻骨尾骨肌的功能。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "Crede手法" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "逼尿肌" }, { "start_idx": 24, "end_idx": 36, "type": "sym", "entity": "逼尿肌无反射或反射减退而无" }, { "start_idx": 37, "end_idx": 43, "type": "pro", "entity": "Crede手法" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "耻骨" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "膀胱" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "膀胱" }, { "start_idx": 83, "end_idx": 85, "type": "bod", "entity": "膀胱颈" }, { "start_idx": 120, "end_idx": 122, "type": "bod", "entity": "逼尿肌" }, { "start_idx": 132, "end_idx": 138, "type": "pro", "entity": "Kegel训练" }, { "start_idx": 146, "end_idx": 149, "type": "bod", "entity": "盆底肌肉" }, { "start_idx": 153, "end_idx": 157, "type": "bod", "entity": "耻骨尾骨肌" } ] }, { "text": "应指导患儿进行会阴部收缩或放松,做类似于抑制排大便(提肛)和中断排尿(收缩尿道外括约肌)的动作。", "entities": [ { "start_idx": 0, "end_idx": 46, "type": "pro", "entity": "应指导患儿进行会阴部收缩或放松,做类似于抑制排大便(提肛)和中断排尿(收缩尿道外括约肌)的动作" } ] }, { "text": "在女性压力性尿失禁,还可将手指置于阴道内指导患儿通过收缩阴道肌肉进行挤压来增强耻骨尾骨肌的收缩逼尿肌与括约肌不协调的患儿,也可通过该训练指导他们松弛会阴,在不用劲的情况下维持尿流的连续性。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "压力性尿失禁" }, { "start_idx": 12, "end_idx": 46, "type": "pro", "entity": "将手指置于阴道内指导患儿通过收缩阴道肌肉进行挤压来增强耻骨尾骨肌的收缩" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "逼尿肌" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "括约肌" }, { "start_idx": 63, "end_idx": 92, "type": "pro", "entity": "通过该训练指导他们松弛会阴,在不用劲的情况下维持尿流的连续性" } ] }, { "text": "(2)生物反馈:生物反馈的定义为应用检测仪器将体内生理过程记录并加以放大,使之成为可被利用的信息。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "生物反馈" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "生物反馈" } ] }, { "text": "尿流动力学仪器通过膀胱压力容积测定和会阴肌肌电图描计将得到的信息反馈给患者:对于排尿困难者,可训练其增加膀胱内压,克服出口阻力,并同时松弛外括约肌;对于急迫性尿失禁的患儿,可训练其抑制逼尿肌收缩训练以增加膀胱出口阻力手术治疗1.降低膀胱出口阻力的手术(1)神经根切断术:因上运动神经元病变引起逼尿肌反射亢进或痉挛性膀胱,经骶神经阻滞试验、尿流动力学测定证实效果显著且保守治疗无效时,可考虑进行骶神经根切断术,切断骶髓反射弧使上运动神经元病变转变为下运动神经元病变,由此产生一无反射性膀胱,从而增加膀胱容量,降低膀胱内压力以减少对肾脏的威胁骶神经根切断术后,25例转变为有一定容量的无反射性膀胱,23例能满意地排空膀胱,无需再用导尿管。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "pro", "entity": "膀胱压力容积测定" }, { "start_idx": 18, "end_idx": 25, "type": "pro", "entity": "会阴肌肌电图描计" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "膀胱" }, { "start_idx": 69, "end_idx": 72, "type": "bod", "entity": "外括约肌" }, { "start_idx": 86, "end_idx": 96, "type": "pro", "entity": "可训练其抑制逼尿肌收缩" }, { "start_idx": 97, "end_idx": 107, "type": "pro", "entity": "训练以增加膀胱出口阻力" }, { "start_idx": 108, "end_idx": 111, "type": "pro", "entity": "手术治疗" }, { "start_idx": 114, "end_idx": 124, "type": "pro", "entity": "降低膀胱出口阻力的手术" }, { "start_idx": 128, "end_idx": 133, "type": "pro", "entity": "神经根切断术" }, { "start_idx": 137, "end_idx": 143, "type": "dis", "entity": "运动神经元病变" }, { "start_idx": 146, "end_idx": 152, "type": "dis", "entity": "逼尿肌反射亢进" }, { "start_idx": 154, "end_idx": 158, "type": "dis", "entity": "痉挛性膀胱" }, { "start_idx": 161, "end_idx": 167, "type": "pro", "entity": "骶神经阻滞试验" }, { "start_idx": 169, "end_idx": 175, "type": "pro", "entity": "尿流动力学测定" }, { "start_idx": 196, "end_idx": 202, "type": "pro", "entity": "骶神经根切断术" }, { "start_idx": 212, "end_idx": 218, "type": "dis", "entity": "上运动神经元病" }, { "start_idx": 223, "end_idx": 230, "type": "dis", "entity": "下运动神经元病变" }, { "start_idx": 237, "end_idx": 242, "type": "dis", "entity": "无反射性膀胱" }, { "start_idx": 248, "end_idx": 249, "type": "bod", "entity": "膀胱" }, { "start_idx": 255, "end_idx": 256, "type": "bod", "entity": "膀胱" }, { "start_idx": 269, "end_idx": 275, "type": "pro", "entity": "骶神经根切断术" }, { "start_idx": 290, "end_idx": 295, "type": "dis", "entity": "无反射性膀胱" }, { "start_idx": 306, "end_idx": 307, "type": "bod", "entity": "膀胱" }, { "start_idx": 313, "end_idx": 315, "type": "equ", "entity": "导尿管" } ] }, { "text": "然而阳痿和大便失禁是骶神经根切断术的常见并发症,上述报道中有85%的病人出现阳痿症状。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "阳痿" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "大便失禁" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "骶神经根切断术" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "阳痿" } ] }, { "text": "近年采用选择性骶神经根切断术的报告中,结果较为满意且阳痿的发生率较低。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "pro", "entity": "选择性骶神经根切断术" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "阳痿" } ] }, { "text": "在20世纪50和60年代单侧或双侧性阴部神经切断术也是治疗小儿尿路出口梗阻的一种常规手术,但因阳痿发生率也较高,并可能出现术后会阴和肛门周围皮肤感觉丧失以及大便失禁,目前已少采用。", "entities": [ { "start_idx": 12, "end_idx": 24, "type": "pro", "entity": "单侧或双侧性阴部神经切断术" }, { "start_idx": 29, "end_idx": 36, "type": "dis", "entity": "小儿尿路出口梗阻" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "阳痿" }, { "start_idx": 61, "end_idx": 81, "type": "sym", "entity": "术后会阴和肛门周围皮肤感觉丧失以及大便失禁" } ] }, { "text": "(2)经尿道膀胱颈、尿道外括约肌切开术:Braasch和Thompson在1953年首次经尿道行膀胱颈切开术,以后这一手术为Emmett所提倡。", "entities": [ { "start_idx": 3, "end_idx": 18, "type": "pro", "entity": "经尿道膀胱颈、尿道外括约肌切开术" }, { "start_idx": 44, "end_idx": 53, "type": "pro", "entity": "经尿道行膀胱颈切开术" } ] }, { "text": "手术切开部位主要为膀胱截石位5点、7点和/或12点。", "entities": [ { "start_idx": 9, "end_idx": 24, "type": "bod", "entity": "膀胱截石位5点、7点和/或12点" } ] }, { "text": "对于无反射性膀胱,膀胱颈部不能适当开放但又无明显外括约肌痉挛者效果较好,可结合腹部用力或Crede法排尿而获得满意的排空效果。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "无反射性膀胱" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "膀胱颈部" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "外括约肌痉挛" } ] }, { "text": "但该方法不适用于外括约肌痉挛或膀胱逼尿肌与尿道括约肌不协调的病人。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "外括约肌痉挛" }, { "start_idx": 15, "end_idx": 28, "type": "dis", "entity": "膀胱逼尿肌与尿道括约肌不协调" } ] }, { "text": "由于目前尚无单纯作用于外括约肌的药物,经尿道外括约肌切开术常应用于因上运动神经元病变造成外括约肌痉挛或膀胱逼尿肌与尿道括约肌不协调的病例。", "entities": [ { "start_idx": 34, "end_idx": 41, "type": "dis", "entity": "上运动神经元病变" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "外括约肌痉挛" }, { "start_idx": 51, "end_idx": 64, "type": "dis", "entity": "膀胱逼尿肌与尿道括约肌不协调" } ] }, { "text": "术前尿流动力学评价十分重要:膀胱有足够容量,逼尿肌收缩较好,尿道压力显示括约肌压力等于或大于逼尿肌压力,或存在膀胱逼尿肌与尿道括约肌不协调时采用经尿道外括约肌切开术效果较好。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "膀胱有足够容量" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "逼尿肌收缩较好" }, { "start_idx": 30, "end_idx": 50, "type": "sym", "entity": "尿道压力显示括约肌压力等于或大于逼尿肌压力" }, { "start_idx": 52, "end_idx": 85, "type": "sym", "entity": "或存在膀胱逼尿肌与尿道括约肌不协调时采用经尿道外括约肌切开术效果较好" } ] }, { "text": "该手术为一种不可逆的手术方法,如患者膀胱颈部功能不正常(无正常控制排尿能力),尿失禁是其唯一结果,病人常需在术后使用集尿装置。", "entities": [ { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "尿失禁" }, { "start_idx": 58, "end_idx": 61, "type": "equ", "entity": "集尿装置" } ] }, { "text": "标准切口从近精阜下端起到尿道球部止,长约2.5~3cm,深度为必须切断该处的弹性纤维环、环行平滑肌和外括约肌组织。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "精阜下端" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "尿道球部" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "弹性纤维环" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "环行平滑肌" }, { "start_idx": 50, "end_idx": 55, "type": "bod", "entity": "外括约肌组织" } ] }, { "text": "经尿道外括约肌切开术的并发症为切口出血、穿孔、尿外渗及尿路感染、尿道狭窄等,有作者认为12点切口是并发症最少最安全的切口,而3点和9点切口出现阳痿的可能性较大。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "经尿道外括约肌切开术" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "切口出血" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "穿孔" }, { "start_idx": 23, "end_idx": 25, "type": "sym", "entity": "尿外渗" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "尿路感染" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "尿道狭窄" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "阳痿" } ] }, { "text": "(3)Y-V膀胱颈成形术:应用于因慢性炎症或导管慢性刺激造成膀胱颈部纤维狭窄的神经源性膀胱病人,常与其他手术如纠正膀胱输尿管反流等同时进行,或对完全性尿失禁病人于本手术同时植入人工尿道括约肌。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "Y-V膀胱颈成形术" }, { "start_idx": 30, "end_idx": 37, "type": "dis", "entity": "膀胱颈部纤维狭窄" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 72, "end_idx": 77, "type": "dis", "entity": "完全性尿失禁" }, { "start_idx": 86, "end_idx": 94, "type": "pro", "entity": "植入人工尿道括约肌" } ] }, { "text": "Y-V成形术一般能起到在保持尿不失禁的情况下改善膀胱排空的作用,虽可能损害近端括约肌机制,但较轻微。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "Y-V成形术" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "近端括约肌" } ] }, { "text": "2.增加膀胱出口阻力的手术(1)膀胱颈悬吊术:多用于治疗膀胱出口阻力低且不伴逼尿肌不稳定的尿失禁患者。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "增加膀胱出口阻力的手术" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "膀胱颈悬吊术" }, { "start_idx": 28, "end_idx": 43, "type": "sym", "entity": "膀胱出口阻力低且不伴逼尿肌不稳定" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "尿失禁" } ] }, { "text": "目前主要采用悬吊术(slingprocedures),利用机体各种组织片或合成替代物作膀胱颈悬吊术。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "悬吊术" }, { "start_idx": 10, "end_idx": 24, "type": "pro", "entity": "slingprocedures" }, { "start_idx": 43, "end_idx": 48, "type": "pro", "entity": "膀胱颈悬吊术" } ] }, { "text": "Kurzrock等采用膀胱前壁带蒂肌瓣环绕膀胱颈部并将其悬吊于耻骨及筋膜的方法治疗膀胱出口阻力低且顺应性差的神经源性膀胱患儿,获得较好的治疗效果。", "entities": [ { "start_idx": 9, "end_idx": 38, "type": "pro", "entity": "采用膀胱前壁带蒂肌瓣环绕膀胱颈部并将其悬吊于耻骨及筋膜的方法" }, { "start_idx": 41, "end_idx": 52, "type": "sym", "entity": "膀胱出口阻力低且顺应性差" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "(2)特氟隆(teflon)等生物材料注射手术:1973年Politano报道应用特氟隆作为硬化剂,注射于膜部尿道治疗压力性尿失禁,治愈率达76%,引起各界注意。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "pro", "entity": "特氟隆(teflon)" }, { "start_idx": 15, "end_idx": 22, "type": "pro", "entity": "生物材料注射手术" }, { "start_idx": 41, "end_idx": 43, "type": "dru", "entity": "特氟隆" }, { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "注射" }, { "start_idx": 59, "end_idx": 64, "type": "dis", "entity": "压力性尿失禁" } ] }, { "text": "如今,尽管美国FDA未批准使用,内镜特氟隆注射疗法已用于漏尿压较低的神经源性尿失禁患儿的初次治疗,或通过手术不能完全控制的失禁病例。", "entities": [ { "start_idx": 16, "end_idx": 24, "type": "pro", "entity": "内镜特氟隆注射疗法" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "神经源性尿失禁" } ] }, { "text": "理想的内镜治疗应满足下列标准:内镜检查发现后尿道和膀胱颈可以进行黏膜下注射,而尿道必须容易导尿;如膀胱功能亢进且药物无法控制,逼尿肌不稳定、膀胱顺应性低以及逼尿肌括约肌失协调者不宜作内镜治疗。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "内镜治疗" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "内镜检查" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "后尿道" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "膀胱颈" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "黏膜" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "膀胱功能亢进" }, { "start_idx": 63, "end_idx": 68, "type": "sym", "entity": "逼尿肌不稳定" }, { "start_idx": 70, "end_idx": 86, "type": "sym", "entity": "膀胱顺应性低以及逼尿肌括约肌失协调" }, { "start_idx": 91, "end_idx": 94, "type": "pro", "entity": "内镜治疗" } ] }, { "text": "内镜注射疗法常采用经尿道操作,用Williams针穿过膀胱镜的操作道,女性在膀胱截石位3、6、9、12点,男性在5、7、12点(男性6点处不注射,以免伤及精阜和前列腺组织)位置穿入尿道括约肌段黏膜或括约肌,每一部位注射0.75~2.5ml的特氟隆,以隆起的黏膜完全闭合管腔为度。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "内镜注射疗法" }, { "start_idx": 27, "end_idx": 29, "type": "equ", "entity": "膀胱镜" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "膀胱" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "精阜" }, { "start_idx": 80, "end_idx": 84, "type": "bod", "entity": "前列腺组织" }, { "start_idx": 90, "end_idx": 97, "type": "bod", "entity": "尿道括约肌段黏膜" }, { "start_idx": 99, "end_idx": 101, "type": "bod", "entity": "括约肌" }, { "start_idx": 120, "end_idx": 122, "type": "dru", "entity": "特氟隆" }, { "start_idx": 128, "end_idx": 129, "type": "bod", "entity": "黏膜" } ] }, { "text": "患儿术后需留置8~10FrFoley导尿管,后再行数小时的间歇导尿以避免术后暂时性的尿潴留。", "entities": [ { "start_idx": 11, "end_idx": 20, "type": "equ", "entity": "FrFoley导尿管" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "尿潴留" } ] }, { "text": "除特氟隆外,戊二醛交联的牛胶原蛋白也是一种常用的生物注射材料,但它们可能引起特氟隆颗粒迁移至肺和脑实质、“特氟隆性肉芽肿”或胶原蛋白导致的急/慢性免疫反应等并发症。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "特氟隆" }, { "start_idx": 6, "end_idx": 16, "type": "dru", "entity": "戊二醛交联的牛胶原蛋白" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "肺" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "脑实质" }, { "start_idx": 52, "end_idx": 76, "type": "dis", "entity": "“特氟隆性肉芽肿”或胶原蛋白导致的急/慢性免疫反应" } ] }, { "text": "(3)人工尿道括约肌:适用于神经源性尿失禁患者。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "人工尿道括约肌" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "神经源性尿失禁" } ] }, { "text": "严重逼尿肌反射亢进、膀胱顺应性差、合并严重的膀胱输尿管反流以及尿道梗阻者不宜应用。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "逼尿肌反射亢进" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "膀胱顺应性差" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "尿道梗阻" } ] }, { "text": "3.增加膀胱容量的手术目前应用的主要为胃(肠)膀胱扩大成形术。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "增加膀胱容量的手术" }, { "start_idx": 19, "end_idx": 29, "type": "pro", "entity": "胃(肠)膀胱扩大成形术" } ] }, { "text": "胃、肠膀胱扩大成形术的目的在于使患者膀胱有足够容量,减少排尿次数,增加尿量,并能使患者随意地进行排尿。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "胃、肠膀胱扩大成形术" } ] }, { "text": "膀胱容量小,且有尿潴留的患者作膀胱扩大术后,进行间歇性导尿的次数可大为减少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "膀胱容量小" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "且有尿潴留" } ] }, { "text": "也可用于逼尿肌反射亢进的病人,但如患有逼尿肌括约肌不协调或膀胱出口梗阻者可能影响膀胱扩大术的效果。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "逼尿肌反射亢进" }, { "start_idx": 19, "end_idx": 34, "type": "dis", "entity": "逼尿肌括约肌不协调或膀胱出口梗阻" }, { "start_idx": 40, "end_idx": 44, "type": "pro", "entity": "膀胱扩大术" } ] }, { "text": "膀胱扩大术可选用胃、回肠、盲肠或乙状结肠作为材料,并可产生相应的并发症:①残余尿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "膀胱扩大术" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "胃" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "回肠" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "盲肠" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "乙状结肠" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "残余尿" } ] }, { "text": "②尿路感染。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "尿路感染" } ] }, { "text": "③结石形成。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "结石形成" } ] }, { "text": "④电解质紊乱,多见于尿路梗阻患者。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "电解质紊乱" } ] }, { "text": "⑤尿液酸度升高,产生尿痛等刺激症状,多见于胃膀胱扩大成形术。", "entities": [ { "start_idx": 1, "end_idx": 28, "type": "sym", "entity": "尿液酸度升高,产生尿痛等刺激症状,多见于胃膀胱扩大成形术" } ] }, { "text": "⑥膀胱出口梗阻导致手术失败。", "entities": [ { "start_idx": 1, "end_idx": 12, "type": "sym", "entity": "膀胱出口梗阻导致手术失败" } ] }, { "text": "上运动神经元病变常引起逼尿肌括约肌收缩不协调,不能产生正常排尿功能,故采用膀胱扩大术的同时,作Y-V成形术或括约肌切开术,以改善术后排尿功能。", "entities": [ { "start_idx": 0, "end_idx": 32, "type": "sym", "entity": "上运动神经元病变常引起逼尿肌括约肌收缩不协调,不能产生正常排尿功能" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "膀胱扩大术" }, { "start_idx": 47, "end_idx": 52, "type": "pro", "entity": "Y-V成形术" }, { "start_idx": 54, "end_idx": 59, "type": "pro", "entity": "括约肌切开术" } ] }, { "text": "⑦有黏膜恶变可能。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "sym", "entity": "有黏膜恶变可能" } ] }, { "text": "自体膀胱扩大术可增加膀胱容量和顺应性,降低膀胱贮尿期压力,从而减少对上尿路的损害,对于因神经源性膀胱引起的膀胱顺应性差、容量小以及膀胱压力高等情况尤为有效,而且与采用胃(肠道)代膀胱手术以扩大膀胱容量相比,该手术具有创伤小,手术、住院时间短,无需进行特别的术前准备,不会出现术后代谢紊乱或尿路结石、感染等并发症,也不影响以后其他膀胱扩大手术方法的应用的优点,但其长期效果有待观察。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "自体膀胱扩大术" }, { "start_idx": 139, "end_idx": 142, "type": "dis", "entity": "代谢紊乱" }, { "start_idx": 144, "end_idx": 147, "type": "dis", "entity": "尿路结石" }, { "start_idx": 149, "end_idx": 150, "type": "dis", "entity": "感染" }, { "start_idx": 164, "end_idx": 169, "type": "pro", "entity": "膀胱扩大手术" } ] }, { "text": "4.增加膀胱逼尿肌收缩能力的手术(1)电刺激治疗1954年McGuire在截瘫病人中首次试用了可植入性膀胱刺激装置,但效果不理想。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "pro", "entity": "增加膀胱逼尿肌收缩能力的手术" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "电刺激治疗" } ] }, { "text": "(2)单层肠浆肌层膀胱加强术:应用带血管神经蒂的肠段,去除肠黏膜,沿系膜对侧缘剪开,形成单层肠浆肌层,包绕缝合于膀胱表面,加强膀胱逼尿肌收缩能力,也可能促进逼尿肌神经的再分布,重新建立排尿反射。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "pro", "entity": "单层肠浆肌层膀胱加强术" }, { "start_idx": 15, "end_idx": 49, "type": "pro", "entity": "应用带血管神经蒂的肠段,去除肠黏膜,沿系膜对侧缘剪开,形成单层肠浆肌层" }, { "start_idx": 51, "end_idx": 95, "type": "pro", "entity": "包绕缝合于膀胱表面,加强膀胱逼尿肌收缩能力,也可能促进逼尿肌神经的再分布,重新建立排尿反射" } ] }, { "text": "该手术的效果可能与膀胱逼尿肌的纤维疤痕化程度有关。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "膀胱逼尿肌" } ] }, { "text": "5.其他尿流改道常作为最后保护和挽救肾功能的重要措施之一,极少用于神经源性膀胱的最初治疗。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "尿流改道" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "主要适用于尿路感染和尿道梗阻持续存在,药物或外科治疗无法解决者;膀胱功能完全丧失,无力排空或出现完全性尿失禁者;膀胱失代偿导致上尿路积水者。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "尿路感染" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "尿道梗阻" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "完全性尿失禁" }, { "start_idx": 56, "end_idx": 67, "type": "sym", "entity": "膀胱失代偿导致上尿路积水" } ] }, { "text": "尿流改道方法繁多,以膀胱为界可分成三大类:①膀胱以下:导尿术及会阴尿道造瘘术;②膀胱部位:耻骨上膀胱造瘘术及膀胱皮肤造瘘术;③膀胱以上:输尿管皮肤造瘘术、输尿管乙状结肠吻合术、输尿管肠吻合造瘘术及肾造瘘术等。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "膀胱" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "导尿术" }, { "start_idx": 31, "end_idx": 37, "type": "pro", "entity": "会阴尿道造瘘术" }, { "start_idx": 45, "end_idx": 52, "type": "pro", "entity": "耻骨上膀胱造瘘术" }, { "start_idx": 54, "end_idx": 60, "type": "pro", "entity": "膀胱皮肤造瘘术" }, { "start_idx": 68, "end_idx": 75, "type": "pro", "entity": "输尿管皮肤造瘘术" }, { "start_idx": 77, "end_idx": 86, "type": "pro", "entity": "输尿管乙状结肠吻合术" }, { "start_idx": 88, "end_idx": 96, "type": "pro", "entity": "输尿管肠吻合造瘘术" }, { "start_idx": 98, "end_idx": 101, "type": "pro", "entity": "肾造瘘术" } ] }, { "text": "选择合适的尿流改道手术,应首先考虑肾功能情况,其次是手术部位(膀胱、输尿管及肾脏)、转流道(应用导管、肠段及尿路本身器官)、暂时或永久改道等手术方面的选择。", "entities": [ { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "膀胱" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "输尿管" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "肾脏" } ] }, { "text": "神经源性膀胱患者采用膀胱以上的尿流改道手术并不多见,并且由于间歇性导尿的广泛应用,永久性尿流改道多用于对尿失禁的控制。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "膀胱" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "尿流改道手术" } ] }, { "text": "(三)并发症的治疗1.尿路感染是神经源性膀胱最常见的并发症,主要由于残余尿积聚或留置导尿造成。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "尿路感染" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "间歇性清洁导尿可明显降低尿路感染率,该方法不主张应用抗生素或化学杀菌剂作为预防用药,以防耐药菌产生,仅主张于有明显感染症状时用。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "抗生素" }, { "start_idx": 30, "end_idx": 34, "type": "dru", "entity": "化学杀菌剂" } ] }, { "text": "2.尿路结石神经源性膀胱患者中尿潴留、尿路感染、组织碎片和上皮细胞脱落等为结石形成创造了有利条件。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "尿路结石" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "尿潴留" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "尿路感染" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "组织碎片" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "上皮细胞脱落" } ] }, { "text": "多见于膀胱结石,尤其在留置导尿的患者。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "膀胱结石" } ] }, { "text": "3.膀胱输尿管反流治疗的基本原则是保持膀胱排空和降低膀胱内压,如上述方法不能使反流消失,应考虑进行抗反流手术。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 17, "end_idx": 29, "type": "pro", "entity": "保持膀胱排空和降低膀胱内压" } ] }, { "text": "4.上尿路积水引起输尿管和/或肾盂积水的原因,主要是由于膀胱残余尿量增加,膀胱内压升高,影响膀胱输尿管连接部抗反流的正常机制。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "上尿路积水" }, { "start_idx": 28, "end_idx": 61, "type": "sym", "entity": "膀胱残余尿量增加,膀胱内压升高,影响膀胱输尿管连接部抗反流的正常机制" } ] }, { "text": "治疗上尿路积水的关键在于早期处理,通过导尿术等方法减少残余尿,降低膀胱内压力。", "entities": [ { "start_idx": 0, "end_idx": 37, "type": "pro", "entity": "治疗上尿路积水的关键在于早期处理,通过导尿术等方法减少残余尿,降低膀胱内压力" } ] }, { "text": "如儿童期的钙摄入充足,可使成人期的骨质疏松和骨折的机会减少。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "骨质疏松" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "骨折" } ] }, { "text": "第一节婴儿期(0~1岁)的膳食特点【婴儿期的营养需要】此期的生长发育是一生中最快的,一年内体重增加3倍,身长增加50%,故各种营养素的需求量大,但此时胃肠功能不成熟,存在矛盾;肝脏功能不完善,氨基酸要求高;肾脏功能不全,负担不能过大。", "entities": [ { "start_idx": 75, "end_idx": 81, "type": "sym", "entity": "胃肠功能不成熟" }, { "start_idx": 88, "end_idx": 94, "type": "sym", "entity": "肝脏功能不完善" }, { "start_idx": 103, "end_idx": 108, "type": "sym", "entity": "肾脏功能不全" } ] }, { "text": "热能需要高于成人3~4倍,1岁以内的婴儿母乳喂养70~85kcal/(kg•d),人工喂养75~90kcal/(kg•d)。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "母乳" } ] }, { "text": "蛋白质2~4g/(kg•d)(母乳2g/kg,牛乳3.5g/kg,大豆蛋白4g/kg)。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "母乳" } ] }, { "text": "由于肝脏不成熟,某些氨基酸(如组氨酸)不能合成,胱氨酸、酪氨酸合成低下;另外,由于支链氨基酸不增加肝脏负担;精氨酸有促进生长的作用,防止高氨血症,提高免疫功能;牛磺酸参与胆汁酸的代谢,且是神经系统及视网膜发育不可缺少的成分。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "肝脏不成熟" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "胱氨酸" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "酪氨酸" }, { "start_idx": 24, "end_idx": 34, "type": "sym", "entity": "胱氨酸、酪氨酸合成低下" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "支链氨基酸" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "肝脏" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "精氨酸" }, { "start_idx": 68, "end_idx": 71, "type": "dis", "entity": "高氨血症" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "牛磺酸" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "胆汁酸" }, { "start_idx": 94, "end_idx": 97, "type": "bod", "entity": "神经系统" }, { "start_idx": 99, "end_idx": 101, "type": "bod", "entity": "视网膜" } ] }, { "text": "所以,氨基酸品种需要多,必需氨基酸的需要量明显高于成人。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "氨基酸" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "必需氨基酸" } ] }, { "text": "脂肪提供的热能占总热能的35%~50%,必需脂肪酸不低于1%~3%。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脂肪" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "必需脂肪酸" } ] }, { "text": "婴儿(尤其是早产儿)不能从亚油酸、亚麻酸合成花生四烯酸和二十二碳六烯酸,而这些成分也是神经细胞膜和视网膜发育不可缺少的,所以,必须由食物中补充。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "亚油酸" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "亚麻酸" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "花生四烯酸" }, { "start_idx": 28, "end_idx": 34, "type": "bod", "entity": "二十二碳六烯酸" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "神经细胞膜" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "视网膜" } ] }, { "text": "乳类包括母乳、牛乳、羊乳和婴儿配方乳。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "母乳" } ] }, { "text": "母乳是婴儿首选的最佳食品,现公认4~6个月内婴儿应采用纯母乳喂养。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "母乳" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "母乳" } ] }, { "text": "1.母乳喂养(1)人乳的优点:人乳的优越性是其他任何乳类所不能比拟的,其所含的营养素一般都能满足婴儿的需求,对婴儿的生长发育最有利。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "母乳" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "人乳" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "人乳" } ] }, { "text": "各期人乳所含营养成分见表4-4。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "人乳" } ] }, { "text": "人乳中乳清蛋白比酪蛋白多,且以人乳清蛋白为主,在胃中形成的凝块小,易消化,不易引起过敏。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "人乳" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "乳清蛋白" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "酪蛋白" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "乳清蛋白" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "胃" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "过敏" } ] }, { "text": "人乳所含的氨基酸比值恰当。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "氨基酸" } ] }, { "text": "牛磺酸含量是牛乳的10倍,它具有促进大脑发育的作用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "牛磺酸" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "大脑" } ] }, { "text": "此外,还含有乳铁蛋白、分泌型IgA(SIgA)、溶菌酶等抗感染的蛋白质。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "乳铁蛋白" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "分泌型IgA" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "SIgA" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "溶菌酶" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "感染" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "蛋白质" } ] }, { "text": "人乳含不饱和必需脂肪酸较多,还含卵磷脂和鞘磷脂,对婴儿中枢神经系统发育极为重要。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "人乳" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "必需脂肪酸" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "卵磷脂" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "鞘磷脂" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "乳糖含量高,且以乙型乳糖为主,有助于类脂物的完全氧化及肝糖原的贮存,可促进乳酸杆菌生长,使pH值降低,有助于钙的吸收和抑制大肠杆菌生长,人乳乳糖较葡萄糖更易合成脑苷脂类,有利于大脑发育。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "乳糖" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "乙型乳糖" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "类脂物" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肝糖原" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "乳酸杆菌" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "大肠杆菌" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "人乳乳糖" }, { "start_idx": 80, "end_idx": 83, "type": "bod", "entity": "脑苷脂类" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "大脑" } ] }, { "text": "人乳中矿物质的总量比牛乳低,对于肾功能尚未发育完善的初生婴儿是有利的。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "人乳" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "肾功能" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "肾功能尚未发育完善" } ] }, { "text": "初乳含微量元素锌和铜较高,但人乳中维生素D和维生素K的含量较少,故应及时补充。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "人乳" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "维生素D" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "维生素K" } ] }, { "text": "另外,人乳中含有大量免疫物质,能增进婴儿的抗感染能力。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "人乳" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "感染" } ] }, { "text": "人乳含有IgA、IgG、IgM及IgE等免疫球蛋白,尤以初乳中SIgA量最多,它具有抗肠道微生物的作用以及抗呼吸道病毒病毒的作用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "人乳" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "IgA" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "IgG" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "IgM" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "IgE" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "SIgA" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "呼吸道病毒" } ] }, { "text": "人乳含有各类免疫细胞,其中大多数为巨噬细胞,约占90%;其余是中性粒细胞、T和B淋巴细胞、浆细胞。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "人乳" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 37, "end_idx": 43, "type": "bod", "entity": "T和B淋巴细胞" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "浆细胞" } ] }, { "text": "母乳喂哺不易污染,温度适宜,方便经济,不易引起婴儿过敏;可增进母婴感情,使婴儿获得满足感及安全感,也有利于教养,促进婴儿的心理发育。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "母乳" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "过敏" } ] }, { "text": "大量的研究资料证明母乳喂养的婴儿患病率、死亡率低于非母乳喂养的婴儿。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "母乳" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "母乳" } ] }, { "text": "表4-2各期人乳的成分(g/L)母乳喂养对早产儿来说更重要,早产儿母乳中的成分与足月母乳又有不同,其营养价值和生物学功能更适合早产儿的需求(表4-4)。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "人乳" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "母乳" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "母乳" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "母乳" } ] }, { "text": "如蛋白质含量高,利于早产儿的快速生长;乳清蛋白与酪蛋白的比例为70∶30,脂肪和乳糖含量偏低,易于吸收;钠盐较高,利于补充早产儿钠的丢失;长链多不饱和脂肪酸,如二十二碳六烯酸(DHA)、花生四烯酸(AA)和牛磺酸,是足月成熟乳含量的1.5~2倍,对促进早产儿中枢神经系统和视网膜的发育有着积极的意义。", "entities": [ { "start_idx": 69, "end_idx": 77, "type": "bod", "entity": "长链多不饱和脂肪酸" }, { "start_idx": 80, "end_idx": 86, "type": "bod", "entity": "二十二碳六烯酸" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "DHA" }, { "start_idx": 93, "end_idx": 97, "type": "bod", "entity": "花生四烯酸" }, { "start_idx": 99, "end_idx": 100, "type": "bod", "entity": "AA" }, { "start_idx": 103, "end_idx": 105, "type": "bod", "entity": "牛磺酸" }, { "start_idx": 129, "end_idx": 134, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 136, "end_idx": 138, "type": "bod", "entity": "视网膜" } ] }, { "text": "但由于极低出生体重儿的特点,纯母乳喂养不能满足其对蛋白质及多种营养素的需求,单纯喂养时生长速度较慢;母乳内钙磷含量较低,这些矿物质的不足会刺激骨的重吸收以保证血清钙浓度的正常,具有造成早产儿骨发育不良和代谢性骨病的危险。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "母乳" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "母乳" }, { "start_idx": 92, "end_idx": 99, "type": "dis", "entity": "早产儿骨发育不良" }, { "start_idx": 101, "end_idx": 105, "type": "dis", "entity": "代谢性骨病" } ] }, { "text": "目前,国际上推荐母乳喂养的低出生体重早产儿添加含蛋白质、矿物质和维生素的母乳强化剂(HMF)以确保满足预期的营养和生长需求。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "母乳" }, { "start_idx": 36, "end_idx": 40, "type": "dru", "entity": "母乳强化剂" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "HMF" } ] }, { "text": "表4-3早产母乳与足月母乳的主要成分(U/L)(2)哺乳技术:正常新生儿生后0.5~1小时可开始哺乳。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "母乳" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "母乳" } ] }, { "text": "两侧乳房喂哺先后应相互交替,利于乳汁充分吸空,保持下次乳汁的分泌。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "两侧乳房" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "乳汁" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "乳汁" } ] }, { "text": "正确的哺乳姿势首先要在母亲体位舒适的前提下,确保婴儿口腔与母亲乳头的正确含接。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "口腔" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "乳头" } ] }, { "text": "正确的乳头含接姿应该让婴儿含入乳晕的大部分,将乳晕下的乳房组织(包括储存乳汁的乳窦部位)也含入口内,此时婴儿的舌头向前伸出盖住牙龈,两侧向上卷曲裹住乳窦部位,这样婴儿吸吮的是母亲部分乳房而不是乳头(图4-4甲),如果婴儿吸吮乳头而没有含住乳晕,此时婴儿的舌头挤压不到储存乳汁的乳窦部位,吸不到乳汁,常常表现为哭闹,实际上是乳头含接不良(图4-4乙)。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "乳头" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "乳晕" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "乳晕" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "乳房组织" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "乳汁" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "乳窦部位" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "舌头" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "牙龈" }, { "start_idx": 74, "end_idx": 77, "type": "bod", "entity": "乳窦部位" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "乳房" }, { "start_idx": 96, "end_idx": 97, "type": "bod", "entity": "乳头" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "乳头" }, { "start_idx": 119, "end_idx": 120, "type": "bod", "entity": "乳晕" }, { "start_idx": 127, "end_idx": 128, "type": "bod", "entity": "舌头" }, { "start_idx": 135, "end_idx": 136, "type": "bod", "entity": "乳汁" }, { "start_idx": 138, "end_idx": 141, "type": "bod", "entity": "乳窦部位" }, { "start_idx": 146, "end_idx": 147, "type": "bod", "entity": "乳汁" }, { "start_idx": 161, "end_idx": 162, "type": "bod", "entity": "乳头" } ] }, { "text": "从外观上判断正确的含接姿势:婴儿的下颌接触乳房,口张大,下唇外翻,婴儿口上方露出的乳晕比口下方多(图4-4丙)。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "乳房" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "下唇" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "乳晕" } ] }, { "text": "含接不良的姿势:下颌不接触乳房,口张得不大而向前噘,下唇不向外翻,口上下方露出的乳晕一样多(图4-4丁)。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "乳房" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "下唇" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "乳晕" } ] }, { "text": "哺乳期母亲应少吃刺激性食物,不饮酒、不抽烟,忌服或慎服能从乳汁排出的药物,如红霉素、氯霉素、链霉素、溴化物、碘化物、水杨酸盐、抗甲状腺药、抗凝血药、阿托品等,以免婴儿发生药物中毒或不良反应。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "乳汁" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "红霉素" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "氯霉素" }, { "start_idx": 46, "end_idx": 48, "type": "dru", "entity": "链霉素" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "溴化物" }, { "start_idx": 54, "end_idx": 56, "type": "dru", "entity": "碘化物" }, { "start_idx": 58, "end_idx": 61, "type": "dru", "entity": "水杨酸盐" }, { "start_idx": 63, "end_idx": 67, "type": "dru", "entity": "抗甲状腺药" }, { "start_idx": 74, "end_idx": 76, "type": "dru", "entity": "阿托品" }, { "start_idx": 85, "end_idx": 88, "type": "dis", "entity": "药物中毒" } ] }, { "text": "图4-1婴儿与母乳头的含接(3)哺乳禁忌证:有活动性结核、急性传染病、艾滋病、严重心肾疾病、恶性肿瘤及精神病者以及接受放射性核素治疗或服用前述药物的母亲,不宜给婴儿哺乳。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "母乳头" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "活动性结核" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "急性传染病" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "艾滋病" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "严重心肾疾病" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "精神病" }, { "start_idx": 59, "end_idx": 65, "type": "pro", "entity": "放射性核素治疗" } ] }, { "text": "急性呼吸道感染时,乳母宜戴口罩。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "急性呼吸道感染" } ] }, { "text": "一侧患乳腺炎,应暂停患侧直接哺乳,但仍应按时挤出或吸出乳汁,以免病愈后无奶。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "乳腺炎" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "乳汁" } ] }, { "text": "由于夏季时婴儿特别容易发生消化不良和腹泻,因而,如断奶时间正好遇到夏季,建议推迟至秋季再断奶。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "消化不良" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "腹泻" } ] }, { "text": "婴儿配方奶粉在制造时参照母乳的营养成分,在普通奶粉中增加、减少或置换某些营养素的含量,使其更适合婴儿的生理功能和营养需要特点,这种经过改良后的奶粉比普通奶粉和鲜奶更符合婴儿生长的需要,但与母乳相比还是逊色得多。", "entities": [ { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "母乳" } ] }, { "text": "总而言之,人工喂养不如母乳喂养质优、经济、方便,又易污染。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "母乳" } ] }, { "text": "足月婴儿可选择普通婴儿奶粉,而对于早产儿来说,由于其生长要求更高,消化道功能不成熟,需要更多易消化吸收的营养素,故需要喂养早产儿配方奶。", "entities": [ { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "消化道功能不成熟" } ] }, { "text": "必需脂肪酸(包括亚油酸和亚麻酸)的含量和比例适宜,并强化了长链多不饱和脂肪酸,使其达到母乳含量,利于早产儿神经系统的发育。", "entities": [ { "start_idx": 29, "end_idx": 37, "type": "bod", "entity": "长链多不饱和脂肪酸" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "母乳" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "神经系统" } ] }, { "text": "这种配方奶中各种营养素和能量介于早产儿配方奶和标准婴儿配方奶之间,以帮助早产儿实现追赶性生长,适用于人工喂养的早产/低出生体重儿或作为母乳的补充(表4-4)。", "entities": [ { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "母乳" } ] }, { "text": "6个月以内的婴儿混合喂养时,母乳喂哺次数一般不变,可先哺母乳,将乳房吸空,再补授人工乳品,这样可维持母乳一定的分泌量。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "母乳" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "母乳" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "乳房" } ] }, { "text": "(二)及时添加辅食无论是接受母乳喂养,还是人工喂养的婴儿,从第4~6个月起就需要及时添加辅食。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "母乳" } ] }, { "text": "此时,母乳分泌量往往也相对不足,随着婴儿月龄的增长,单纯乳类喂养已不能满足婴儿的生长需求;而且,小儿体内的储备铁已消耗完,而乳类中所含维生素及铁的量均不能满足生长发育的需要,如不及时补充,易患缺铁性贫血,还会造成以后喂养的困难。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "母乳" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "乳类" }, { "start_idx": 96, "end_idx": 100, "type": "dis", "entity": "缺铁性贫血" } ] }, { "text": "第二节过敏性紫癜【概述】过敏性紫癜(anaphylactoidpurpura)也称亨-舒综合征(Henöch-Schöhleinsyndrome,Henoöch-Schönleinpurpura,HSP),是一种以小血管炎为主要病变的系统性血管炎,临床表现为皮肤紫癜,常伴关节炎、腹痛、便血和肾小球肾炎;多发于学龄前和学龄期儿童,男孩多于女孩,一年四季均有发病,以春秋两季居多,国内报告HSP患病率有逐年增高趋势。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "过敏性紫癜" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "过敏性紫癜" }, { "start_idx": 18, "end_idx": 37, "type": "dis", "entity": "anaphylactoidpurpura" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "亨-舒综合征" }, { "start_idx": 48, "end_idx": 71, "type": "dis", "entity": "Henöch-Schöhleinsyndrome" }, { "start_idx": 73, "end_idx": 96, "type": "dis", "entity": "Henoöch-Schönleinpurpura" }, { "start_idx": 98, "end_idx": 100, "type": "dis", "entity": "HSP" }, { "start_idx": 107, "end_idx": 110, "type": "dis", "entity": "小血管炎" }, { "start_idx": 117, "end_idx": 122, "type": "dis", "entity": "系统性血管炎" }, { "start_idx": 129, "end_idx": 132, "type": "sym", "entity": "皮肤紫癜" }, { "start_idx": 136, "end_idx": 138, "type": "dis", "entity": "关节炎" }, { "start_idx": 140, "end_idx": 141, "type": "sym", "entity": "腹痛" }, { "start_idx": 143, "end_idx": 144, "type": "sym", "entity": "便血" }, { "start_idx": 146, "end_idx": 150, "type": "dis", "entity": "肾小球肾炎" }, { "start_idx": 193, "end_idx": 195, "type": "dis", "entity": "HSP" } ] }, { "text": "【病因和发病机制】HSP发病机制尚未明确,下列情况可能为诱因如食物过敏(蛋类、乳类和豆类等)、药物(阿司匹林和抗生素等)、微生物(细菌、病毒和寄生虫等)、疫苗接种、麻醉以及恶性病变等。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "HSP" }, { "start_idx": 50, "end_idx": 53, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 55, "end_idx": 57, "type": "dru", "entity": "抗生素" }, { "start_idx": 65, "end_idx": 66, "type": "mic", "entity": "细菌" }, { "start_idx": 68, "end_idx": 69, "type": "mic", "entity": "病毒" }, { "start_idx": 71, "end_idx": 73, "type": "mic", "entity": "寄生虫" } ] }, { "text": "有报告HSP患儿中,50%以上有链球菌感染史,提示链球菌起触发作用,但随后研究发现链球菌感染史在HSP和健康儿童对照间并无差别。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "HSP" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "链球菌感染" }, { "start_idx": 25, "end_idx": 27, "type": "mic", "entity": "链球菌" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "HSP" } ] }, { "text": "30%~50%患儿血清IgA浓度升高;HSP急性期血循环中表面IgA阳性的B淋巴细胞数、IgA类免疫复合物或冷球蛋白均增高;HSP患儿的淋巴细胞可自发合成大量的IgA;IgA、补体C3和纤维蛋白沉积于肾小球系膜、皮肤和肠道毛细血管而导致相应症状。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "血清IgA" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "HSP" }, { "start_idx": 31, "end_idx": 41, "type": "bod", "entity": "IgA阳性的B淋巴细胞" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "冷球蛋白" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "HSP" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "IgA" }, { "start_idx": 84, "end_idx": 86, "type": "bod", "entity": "IgA" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "补体C" }, { "start_idx": 99, "end_idx": 102, "type": "bod", "entity": "纤维蛋白" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "皮肤" }, { "start_idx": 115, "end_idx": 120, "type": "bod", "entity": "肠道毛细血管" } ] }, { "text": "本病有一定遗传倾向,但肯定的HLA连锁尚未确定,有人认为HLA-DW35者易患本病,部分患儿伴有C2补体成分缺乏。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "HLA" }, { "start_idx": 28, "end_idx": 35, "type": "dis", "entity": "HLA-DW35" } ] }, { "text": "【病理】HSP病理变化为广泛的白细胞碎裂性小血管炎,以毛细血管炎为主,亦可波及静脉和小动脉;血管壁可见胶原纤维肿胀和坏死,中性粒细胞浸润,周围有散在核碎片;间质水肿,有浆液性渗出,同时可见渗出的红细胞;内皮细胞肿胀,可有血栓形成。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "HSP" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "毛细血管炎" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "静脉" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "小动脉" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "血管壁" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "胶原纤维" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "中性粒细胞" }, { "start_idx": 78, "end_idx": 99, "type": "sym", "entity": "间质水肿,有浆液性渗出,同时可见渗出的红细胞" }, { "start_idx": 101, "end_idx": 113, "type": "sym", "entity": "内皮细胞肿胀,可有血栓形成" } ] }, { "text": "病变累及皮肤、肾脏、关节及胃肠道,少数涉及心、肺等脏器。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "皮肤" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肾脏" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "关节" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "胃肠道" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "心" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "肺" } ] }, { "text": "在皮肤和肾脏,荧光显微镜下可见IgA为主的免疫复合物沉积。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "皮肤" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肾脏" }, { "start_idx": 7, "end_idx": 11, "type": "equ", "entity": "荧光显微镜" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "IgA" } ] }, { "text": "【临床表现】多为急性起病,首发症状以皮肤紫癜为主,部分病例腹痛、关节炎或肾脏症状上呼吸道感染史。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "皮肤紫癜" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "关节炎" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肾脏" }, { "start_idx": 29, "end_idx": 39, "type": "sym", "entity": "腹痛、关节炎或肾脏症状" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "上呼吸道感染" } ] }, { "text": "(一)皮肤紫癜病程中反复出现皮肤紫癜为本病特征,多见于四肢及臀部及躯干较少;初起呈紫红色斑丘疹,高出皮面,继而呈棕褐色而消退,可伴有荨麻疹和血管神经性水肿,重症患儿紫癜可融合成大疱伴出血性坏死。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "皮肤紫癜" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "皮肤紫癜" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "四肢" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "臀部" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "躯干" }, { "start_idx": 24, "end_idx": 36, "type": "sym", "entity": "多见于四肢及臀部及躯干较少" }, { "start_idx": 41, "end_idx": 61, "type": "sym", "entity": "紫红色斑丘疹,高出皮面,继而呈棕褐色而消退" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "荨麻疹" }, { "start_idx": 70, "end_idx": 76, "type": "dis", "entity": "血管神经性水肿" }, { "start_idx": 82, "end_idx": 83, "type": "sym", "entity": "紫癜" }, { "start_idx": 88, "end_idx": 95, "type": "sym", "entity": "大疱伴出血性坏死" } ] }, { "text": "(二)消化道症状半数以上患儿出现反复的阵发性腹痛,位于脐周或下腹部,疼痛剧烈,可伴呕吐,但呕血少见;部分患儿有黑便或血便、腹泻或便秘,偶见并发肠套叠、肠梗阻或肠穿孔。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "消化道" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "阵发性腹痛" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "脐周" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "下腹部" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "疼痛剧烈" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "呕吐" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "呕血" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "腹泻" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "便秘" }, { "start_idx": 69, "end_idx": 81, "type": "sym", "entity": "并发肠套叠、肠梗阻或肠穿孔" } ] }, { "text": "(三)关节症状出现膝、踝、肘、腕等大关节肿痛,活动受限,呈单发或多发,关节腔有积液,可在数月内消失,不留后遗症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "关节" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "膝" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "踝" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肘" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "腕" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "大关节" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "大关节肿痛" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "关节腔" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "积液" } ] }, { "text": "(四)肾脏症状本病引起的肾脏病变是小儿期最常见的继发性肾小球疾患。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肾脏" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肾脏" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "继发性肾小球" } ] }, { "text": "肾脏症状轻重不一,多数患儿出现血尿、蛋白尿和管型,伴血压增高及水肿,称为紫癜性肾炎,少数呈肾病综合征表现;肾脏症状绝大多数在起病1个月内出现,亦可在病程更晚期发生,大多数能完全恢复,少数发展为慢性肾炎,死于慢性肾衰竭。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肾脏" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "血尿" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "管型" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "血压" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "血压增高及水肿" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "紫癜性肾炎" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "肾脏" }, { "start_idx": 96, "end_idx": 99, "type": "dis", "entity": "慢性肾炎" }, { "start_idx": 103, "end_idx": 107, "type": "dis", "entity": "慢性肾衰竭" } ] }, { "text": "(五)其他偶可发生颅内出血,导致惊厥、昏迷及失语,还可有鼻、牙龈出血循环系统发生心肌炎、心包炎,或累及呼吸系统发生喉头水肿、哮喘和肺出血。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "颅内出血" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "惊厥" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "昏迷" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "失语" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "鼻" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "牙龈" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "鼻、牙龈出血" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "循环系统" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "心肌炎" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "心包炎" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 57, "end_idx": 67, "type": "sym", "entity": "喉头水肿、哮喘和肺出血" } ] }, { "text": "【实验室检查】(一)血象白细胞正常或增加,中性或嗜酸性粒细胞可增高;除非严重出血,一般无贫血;血小板计数正常甚至升高,出血和凝血时间正常,血块退缩试验正常,部分患儿毛细血管脆性试验阳性。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "血象白细胞" }, { "start_idx": 21, "end_idx": 29, "type": "bod", "entity": "中性或嗜酸性粒细胞" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "严重出血" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "血小板" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "出血" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "凝血" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "毛细血管" } ] }, { "text": "(二)尿常规可有红细胞、蛋白及管型,少数有肉眼血尿。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "尿常规" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "红细胞" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "蛋白" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "管型" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "血尿" } ] }, { "text": "(三)有消化道症状者大便隐血试验多阳性。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "消化道" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "大便隐血" } ] }, { "text": "(四)血沉正常或增快血清IgA可升高,IgG、IgM正常亦可轻度升高;C3、C4正常或升高;抗核抗体及RF阴性;重症血浆黏度增高。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血沉" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "血清IgA" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "IgG" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "IgM" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "C3" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "C4" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "重症血浆" } ] }, { "text": "(五)腹部超声波检查有利于早期诊断肠套叠等外科急腹症;有中枢神经系统症状患儿可予头颅MRI助诊;肾脏症状较重和迁延患儿可行肾穿刺以了解病情给予相应治疗。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "腹部超声波检查" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "肠套叠" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "外科急腹症" }, { "start_idx": 28, "end_idx": 33, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "头颅" }, { "start_idx": 42, "end_idx": 44, "type": "pro", "entity": "MRI" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "肾脏" }, { "start_idx": 61, "end_idx": 63, "type": "pro", "entity": "肾穿刺" } ] }, { "text": "【诊断和鉴别诊断】典型病例诊断不难,若临床表现不典型,皮肤紫癜未出现时,容易误诊为其他疾病,需与原发性血小板减少性紫癜、风湿性关节炎及外科急腹症等鉴别。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "皮肤紫癜" }, { "start_idx": 48, "end_idx": 58, "type": "dis", "entity": "原发性血小板减少性紫癜" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "风湿性关节炎" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "外科急腹症" } ] }, { "text": "【治疗】(一)一般治疗卧床休息,积极寻找和去除致病因素,控制感染,补充维生素。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "卧床休息" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "控制感染" }, { "start_idx": 33, "end_idx": 37, "type": "pro", "entity": "补充维生素" } ] }, { "text": "(二)对症治疗有荨麻疹或血管神经性水肿时,应用抗组胺药物和钙剂;腹痛时应用解痉剂,消化道出血时应禁食,可静脉注射西咪替丁20~40mg/(kg•d),必要时输血。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "荨麻疹" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "血管神经性水肿" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "抗组胺药物" }, { "start_idx": 29, "end_idx": 30, "type": "dru", "entity": "钙剂" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "腹痛" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "解痉剂" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "消化道" }, { "start_idx": 52, "end_idx": 55, "type": "pro", "entity": "静脉注射" }, { "start_idx": 56, "end_idx": 59, "type": "dru", "entity": "西咪替丁" }, { "start_idx": 78, "end_idx": 79, "type": "pro", "entity": "输血" } ] }, { "text": "(三)肾上腺皮质激素急性期对腹痛和关节痛可予缓解,但不能预防肾脏损害的发生,亦不能影响预后。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "腹痛" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "关节痛" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肾脏" } ] }, { "text": "(四)抗血小板聚集药物阿司匹林3~5mg/kg,或25~50mg/d,每天1次服用;双嘧达莫(潘生丁)3~5mg/(kg•d),分次服用。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dru", "entity": "抗血小板聚集药物" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 42, "end_idx": 45, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "潘生丁" } ] }, { "text": "(五)其他中成药如复方丹参片和银杏叶片,口服3~6个月,可补肾益气和活血化瘀。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "复方丹参片" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "银杏叶片" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "肾" } ] }, { "text": "【预后】本病预后一般良好,除少数重症患儿可死于肠出血、肠套叠、肠坏死或急性肾衰竭外,大多痊愈。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "sym", "entity": "肠出血" }, { "start_idx": 27, "end_idx": 29, "type": "sym", "entity": "肠套叠" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "肠坏死" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "病程一般约1~2周至1~2个月,少数可长达数月或一年以上;肾脏病变常较迁延,可持续数月或数年,大多自行缓解,部分病例有复发倾向。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肾脏" } ] }, { "text": "葡萄球菌肺炎@三、葡萄球菌肺炎葡萄球菌肺炎(staphylococcalpneumonia)多发生于新生儿和婴儿。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 22, "end_idx": 44, "type": "dis", "entity": "staphylococcalpneumonia" } ] }, { "text": "葡萄球菌肺炎@Goel等报道100例患儿中,1岁以内占78%,平均年龄5个月。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" } ] }, { "text": "葡萄球菌肺炎@金黄色葡萄球(金葡菌)和表皮葡萄球菌均可致病,但以前者致病最强。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 7, "end_idx": 12, "type": "mic", "entity": "金黄色葡萄球" }, { "start_idx": 19, "end_idx": 24, "type": "mic", "entity": "表皮葡萄球菌" } ] }, { "text": "葡萄球菌肺炎@由于金葡菌可产生多种毒素和酶,具有高度组织破坏性和化脓趋势,因而金葡菌肺炎以广泛出血性坏死、多发性小脓疡形成为特点。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "金葡菌" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "金葡菌肺炎" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "出血性坏死" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "多发性小脓疡" } ] }, { "text": "葡萄球菌肺炎@临床上以起病急、发展快、变化大、化脓性并发症多为特征。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "起病急" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "发展快" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "变化大" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "化脓性并发症" } ] }, { "text": "葡萄球菌肺炎@一开始可有1~2天的上呼吸道感染症状,或皮肤疖肿史,病情迅速恶化,出现高热、咳嗽、呻吟、喘憋、气急、发绀,肺部体征出现较早。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "上呼吸道感染" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "皮肤疖肿" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "高热" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "咳嗽" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "呻吟" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "喘憋" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "气急" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "发绀" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "肺部" } ] }, { "text": "葡萄球菌肺炎@易出现脓胸、脓气胸、肺大疱等并发症。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "脓胸" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "脓气胸" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "肺大疱" } ] }, { "text": "葡萄球菌肺炎@外周血白细胞计数常明显升高,以中性粒细胞为主。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 7, "end_idx": 14, "type": "ite", "entity": "外周血白细胞计数" }, { "start_idx": 22, "end_idx": 26, "type": "mic", "entity": "中性粒细胞" } ] }, { "text": "葡萄球菌肺炎@可伴轻至中度贫血。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "中度贫血" } ] }, { "text": "葡萄球菌肺炎@胸片改变特点:发展快、变化多、吸收慢。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "胸片" } ] }, { "text": "葡萄球菌肺炎@肺部病灶可在数小时内发展成为多发性小脓疡或肺大疱,并出现脓胸、脓气胸等并发症。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肺部" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "多发性小脓疡" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肺大疱" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "脓胸" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "脓气胸" } ] }, { "text": "葡萄球菌肺炎@X线改变吸收缓慢,可持续2个月或更久。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "X线" } ] }, { "text": "葡萄球菌肺炎@1岁以下、尤其是3月龄以内的小婴儿,如肺炎病情发展迅速,伴肺大疱、脓胸或肺脓疡形成者应高度怀疑本病。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "肺炎" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "肺大疱" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "脓胸" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "肺脓疡" } ] }, { "text": "葡萄球菌肺炎@在抗生素使用前必须进行痰、鼻咽拭子、浆膜腔液、血液或肺穿刺物的培养。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "抗生素" }, { "start_idx": 18, "end_idx": 18, "type": "pro", "entity": "痰" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "鼻咽拭子" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "浆膜腔液" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "血液" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "肺穿刺物" } ] }, { "text": "葡萄球菌肺炎@痰或胸水涂片染色可发现中性粒细胞和革兰阳性球菌呈葡萄串链状排列。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "痰" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胸水" }, { "start_idx": 18, "end_idx": 22, "type": "mic", "entity": "中性粒细胞" }, { "start_idx": 24, "end_idx": 29, "type": "mic", "entity": "革兰阳性球菌" } ] }, { "text": "葡萄球菌肺炎@血清中磷壁酸抗体测定可作为病原学诊断的补充。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "血清" }, { "start_idx": 10, "end_idx": 16, "type": "ite", "entity": "磷壁酸抗体测定" } ] }, { "text": "葡萄球菌肺炎@合适的抗生素治疗和脓液的引流是治疗的关键。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "脓液的引流" } ] }, { "text": "葡萄球菌肺炎@在获取培养标本后应立即给予敏感的杀菌药物,并足量、联合、静脉用药。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 29, "end_idx": 38, "type": "pro", "entity": "足量、联合、静脉用药" } ] }, { "text": "葡萄球菌肺炎@宜首选耐青霉素酶窄谱青霉素类,如苯唑西林等,可联合头孢霉素类使用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "耐青霉素" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "窄谱青霉素" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "苯唑西林" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "头孢霉素" } ] }, { "text": "葡萄球菌肺炎@如为耐甲氧西林金葡菌(MRSA)引起,应选用万古霉素治疗。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "葡萄球菌肺炎" }, { "start_idx": 9, "end_idx": 16, "type": "mic", "entity": "耐甲氧西林金葡菌" }, { "start_idx": 18, "end_idx": 21, "type": "mic", "entity": "MRSA" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "万古霉素" } ] }, { "text": "三、临床特点(一)原发部位恶性肿瘤一般均有原发部位,但多种儿童恶性肿瘤如淋巴瘤、神经母细胞瘤、尤因肉瘤等在疾病早期就可转移。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "儿童恶性肿瘤" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "神经母细胞瘤" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "尤因肉瘤" } ] }, { "text": "常见儿童肿瘤的原发灶和转移部位(表11-11),不同原发部位常见的肿瘤(表11-4)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "肿瘤" } ] }, { "text": "(二)转移特点儿童肿瘤在就诊时已有远处转移者较多见,原因有以下两点:①疾病早期即可发生远处转移是儿童恶性肿瘤的特点之一;②当肿瘤原发于非体表部位,未形成压迫症状或有全身症状时不易发现,在出现症状而就诊时已存在转移灶。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "儿童恶性肿瘤" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "肿瘤" } ] }, { "text": "即使影像学检查未发现明显转移灶,也有可能已有微小的亚临床转移。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "影像学检查" } ] }, { "text": "非霍奇金淋巴瘤、神经母细胞瘤、横纹肌肉瘤、尤因肉瘤、骨肉瘤就诊时就存在转移的机会较多,除局部扩散外,易发生淋巴、血行远处转移。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "非霍奇金淋巴瘤" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "神经母细胞瘤" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "尤因肉瘤" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "骨肉瘤" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "淋巴" } ] }, { "text": "常见的远处转移部位是肝、脾、淋巴结、骨髓、骨、肺和颅内(见表11-11)。", "entities": [ { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肝" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "脾" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "淋巴结" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "骨髓" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "骨" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "肺" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "颅内" } ] }, { "text": "表11-3常见儿童肿瘤的原发灶和转移部位表11-4不同原发部位常见的肿瘤(三)临床症状及体征儿童血液系统恶性肿瘤全身症状突出,主要有贫血、出血、发热和肝、脾、淋巴结肿大。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "肿瘤" }, { "start_idx": 46, "end_idx": 55, "type": "dis", "entity": "儿童血液系统恶性肿瘤" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "贫血" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "出血" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "发热" }, { "start_idx": 75, "end_idx": 75, "type": "bod", "entity": "肝" }, { "start_idx": 77, "end_idx": 77, "type": "bod", "entity": "脾" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "淋巴结" }, { "start_idx": 75, "end_idx": 83, "type": "sym", "entity": "肝、脾、淋巴结肿大" } ] }, { "text": "表11-5常见部位的肿瘤压迫症状(四)实验室检查1.具特殊意义的血、尿生化学检查(1)尿VMA、HVA、HVA检查:80%~90%神经母细胞瘤患儿尿VMA、HVA明显增高,具特异性,可作为诊断及疾病随访指标。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 32, "end_idx": 39, "type": "pro", "entity": "血、尿生化学检查" }, { "start_idx": 43, "end_idx": 50, "type": "ite", "entity": "尿VMA、HVA" }, { "start_idx": 52, "end_idx": 56, "type": "pro", "entity": "HVA检查" }, { "start_idx": 65, "end_idx": 70, "type": "dis", "entity": "神经母细胞瘤" }, { "start_idx": 73, "end_idx": 80, "type": "ite", "entity": "尿VMA、HVA" } ] }, { "text": "(2)血αFP:肝母细胞瘤、生殖细胞瘤时升高,可作为诊断及疾病随访指标。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "血αFP" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肝母细胞瘤" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "生殖细胞瘤" } ] }, { "text": "(3)血LDH:多种肿瘤有非特异性增高,在淋巴系恶性肿瘤、神经母细胞瘤中较为突出。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "血LDH" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "淋巴系恶性肿瘤" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "神经母细胞瘤" } ] }, { "text": "增高水平与肿瘤负荷相平行,缓解时降至正常,复发时再次增高。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" } ] }, { "text": "2.影像学检查影像学检查是肿瘤诊断的基础手段,主要包括X线平片、B型超声检查、CT、MRI、骨扫描。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "影像学检查" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "影像学检查" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "肿瘤" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "X线平片" }, { "start_idx": 32, "end_idx": 37, "type": "pro", "entity": "B型超声检查" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "CT" }, { "start_idx": 42, "end_idx": 44, "type": "pro", "entity": "MRI" }, { "start_idx": 46, "end_idx": 48, "type": "pro", "entity": "骨扫描" } ] }, { "text": "影像学检查可确定肿块部位、大小、与邻近组织和血管的关系、包膜的完整性、瘤内钙化灶、出血坏死灶、囊性变等,可据此作出初步诊断及手术方案。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "影像学检查" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血管" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "包膜" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "瘤内钙化灶" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "出血坏死灶" } ] }, { "text": "腹腔常见肿瘤的影像学特征(表11-6)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "腹腔" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "肿瘤" } ] }, { "text": "表11-6腹腔常见肿瘤的影像学特征3.骨髓涂片急性白血病时骨髓中幼稚细胞比例明显升高,超过骨髓有核细胞的30%,为诊断白血病的主要依据。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肿瘤" }, { "start_idx": 19, "end_idx": 22, "type": "ite", "entity": "骨髓涂片" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "急性白血病" }, { "start_idx": 29, "end_idx": 54, "type": "sym", "entity": "骨髓中幼稚细胞比例明显升高,超过骨髓有核细胞的30%" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "白血病" } ] }, { "text": "多种儿童恶性肿瘤易发生骨髓转移,如非霍奇金淋巴瘤、神经母细胞瘤、尤因肉瘤等,因此这些肿瘤应常规作骨髓涂片,检查有无肿瘤细胞浸润。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "儿童恶性肿瘤" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "骨髓" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "非霍奇金淋巴瘤" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "神经母细胞瘤" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "尤因肉瘤" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "肿瘤" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "骨髓涂片" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "肿瘤" } ] }, { "text": "参考文献1.张皓,任慈芳,王立波,等.单阻断法测135例婴幼儿肺炎患者呼吸道阻力和顺应性的变化.临床儿科杂志,2001,19(6):330-3302.BeydonN,DavisSD,LombardiE,etal.AnofficialAmericanThoracicSociety/EuropeanRespiratorySocietystatement:pulmonaryfunctiontestinginpreschoolchildren.AmJRespirCritCareMed,2007,175(12):1304-13043.HiroyukiMochizuki.Bronchialsensitivityandbronchialreactivityinchildrenwithcoughvariantasthma.Chest,2005,128(4):2427-24274.MillerMR,HankinsonJ,Brusasco.Standardisationofspirometry.EurRespirJ,2005,26:319-3195.OostveenE,MacleodD,LorinoH,etal.Theforcedoscillationtechniqueinclinicalpractice:methodology,recommendationsandfuturedevelopments.EurRespirJ,2003,22(6):1026-10266.SlyPD,MorganW.Respiratoryfunctiontestingininfantsandpreschool-agedchildren.In:TaussigLMandLandauLIeds.PediatricRespiratorymedicine.2nded.Philadelphia:Mosby,2008:163-1637.TurnerDJ,LanteriCJ,LeSouefKL,etal.Improveddetectionofabnormalrespiratoryfunctionusingforcedexpirationfromrausedlungvolumeininfantswithcysticfibrosis.EurRespirJ,1994,7(11):1995-19958.TurnerDJ,StickSM,LeSouefKL,etal.Anewtechniquetogenerateandassessforcedexpirationfromraisedlungvolumeininfants.AmJRespirCritCareMed,1995,151(5):1441-14419.WangerJ,ClausenJL,CoatesA,etal.Standardisationofthemeasurementoflungvolumes.EurRespirJ,2005,26(3):511-511", "entities": [ { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "肺炎" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "呼吸道" }, { "start_idx": 41, "end_idx": 43, "type": "ite", "entity": "顺应性" }, { "start_idx": 48, "end_idx": 51, "type": "dep", "entity": "临床儿科" } ] }, { "text": "第二节新生儿辅助机械通气辅助机械通气是治疗呼吸衰竭的重要手段。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "辅助机械通气" }, { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "辅助机械通气" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "新生儿呼吸系统代偿能力低下,当患呼吸系统疾病时极易发生呼吸衰竭,故在NICU中使用机械通气的频率较高。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "呼吸系统疾病" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 34, "end_idx": 37, "type": "dep", "entity": "NICU" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "机械通气" } ] }, { "text": "因此,新生儿急救医生应熟练、全面、准确地掌握机械通气相关的肺力学知识、气体交换方式、主要参数的作用、常用的通气模式及其临床应用。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dep", "entity": "急救" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "机械通气" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "肺" } ] }, { "text": "目前,有很多新类型呼吸机供新生儿选用,但持续气流、压力限定-时间转换型呼吸机(continuousflow,pressurelimitedandtime-cycledventilator)仍是新生儿基本而常用的呼吸机类型。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "equ", "entity": "呼吸机" }, { "start_idx": 20, "end_idx": 37, "type": "equ", "entity": "持续气流、压力限定-时间转换型呼吸机" }, { "start_idx": 39, "end_idx": 92, "type": "equ", "entity": "continuousflow,pressurelimitedandtime-cycledventilator" }, { "start_idx": 105, "end_idx": 107, "type": "equ", "entity": "呼吸机" } ] }, { "text": "持续气流是指呼吸机在吸气相和呼气相均持续向其管道内送气,在吸气相,呼气阀关闭气体送入肺内,过多气体通过泄压阀排入大气;在呼气相,呼气阀开放,气体排入大气。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "equ", "entity": "呼吸机" }, { "start_idx": 29, "end_idx": 31, "type": "equ", "entity": "吸气相" }, { "start_idx": 33, "end_idx": 35, "type": "equ", "entity": "呼气阀" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "肺" }, { "start_idx": 51, "end_idx": 53, "type": "equ", "entity": "泄压阀" }, { "start_idx": 60, "end_idx": 62, "type": "equ", "entity": "呼气相" }, { "start_idx": 64, "end_idx": 66, "type": "equ", "entity": "呼气阀" } ] }, { "text": "压力限定是预调的呼吸机管道和气道内在吸气相时的最高压力,当压力超过所调定的压力时,气体即通过泄压阀排出,使呼吸机管道和气道内的最高压力等于调定压力。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "equ", "entity": "呼吸机管道" }, { "start_idx": 14, "end_idx": 15, "type": "equ", "entity": "气道" }, { "start_idx": 46, "end_idx": 48, "type": "equ", "entity": "泄压阀" }, { "start_idx": 53, "end_idx": 57, "type": "equ", "entity": "呼吸机管道" }, { "start_idx": 59, "end_idx": 60, "type": "equ", "entity": "气道" } ] }, { "text": "时间转换即根据需要直接调定吸气时间和频率,呼气时间和吸、呼比呼吸机自动计算并直接显示。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "equ", "entity": "呼吸机" } ] }, { "text": "该类型呼吸机可供调节的参数为吸气峰压、呼气末正压、呼吸频率、吸气时间、吸入气氧分数和气体流速。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "呼吸机" } ] }, { "text": "一、机械通气相关肺力学不论自主呼吸还是辅助机械通气,均需口和肺泡间存在一定的压力差,方能克服肺及胸壁弹性(顺应性)和气道阻力,从而完成吸气和呼气。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "机械通气" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肺" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "辅助机械通气" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肺泡" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "肺" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "胸壁" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "气道" } ] }, { "text": "(一)肺顺应性肺顺应性(complianceoflungs,CL)是指肺的弹性阻力,常以施加单位压力时肺容积改变的大小来表示,其公式为:顺应性(L/cmH2O)=容量(L)/压力(cmH2O)从公式可见,当施给一定压力时,顺应性值越大,容积变化越大。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "肺顺应性" }, { "start_idx": 12, "end_idx": 28, "type": "ite", "entity": "complianceoflungs" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "CL" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "肺" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "肺" } ] }, { "text": "呼吸系统的总顺应性是由胸壁顺应性与肺顺应性构成,但由于新生儿胸壁弹性好,其顺应性常忽略不计,故通常肺顺应性即可代表呼吸系统的总顺应性。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "ite", "entity": "呼吸系统的总顺应性" }, { "start_idx": 11, "end_idx": 15, "type": "ite", "entity": "胸壁顺应性" }, { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "肺顺应性" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "胸壁" }, { "start_idx": 49, "end_idx": 52, "type": "ite", "entity": "肺顺应性" }, { "start_idx": 57, "end_idx": 65, "type": "ite", "entity": "呼吸系统的总顺应性" } ] }, { "text": "正常新生儿肺顺应性为0.003~0.006L/cmH2O;呼吸窘迫综合征(respiratorydistresssyndrome,RDS)时肺顺应性降低,仅为0.0005~0.001L/cmH2O,其含义为:在相同的压力下,送入其肺内的潮气量将明显减少,若获得正常的潮气量,则需要更高的压力。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "ite", "entity": "肺顺应性" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "呼吸窘迫综合征" }, { "start_idx": 48, "end_idx": 74, "type": "dis", "entity": "respiratorydistresssyndrome" }, { "start_idx": 76, "end_idx": 78, "type": "dis", "entity": "RDS" }, { "start_idx": 81, "end_idx": 84, "type": "ite", "entity": "肺顺应性" }, { "start_idx": 137, "end_idx": 137, "type": "bod", "entity": "肺" }, { "start_idx": 140, "end_idx": 142, "type": "ite", "entity": "潮气量" }, { "start_idx": 155, "end_idx": 157, "type": "ite", "entity": "潮气量" } ] }, { "text": "(二)气道阻力气道阻力(resistance,R)是指气道对气流的阻力。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "气道阻力" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "气道阻力" }, { "start_idx": 12, "end_idx": 21, "type": "ite", "entity": "resistance" }, { "start_idx": 23, "end_idx": 23, "type": "ite", "entity": "R" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "气道" } ] }, { "text": "常以单位流速流动的气体所需要的压力来表示,其公式为:气道阻力[cmH2O/(L•sec)]=压力(cmH2O)/流速(L/sec)正常新生儿总气道阻力为20~40cmH2O/(L•sec);气管插管时为50~150cmH2O/(L•sec);胎粪吸入综合征(meconiumaspirationsyndrome,MAS)为100~140cmH2O/(L•sec)或更高。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "ite", "entity": "气道阻力" }, { "start_idx": 92, "end_idx": 96, "type": "ite", "entity": "总气道阻力" }, { "start_idx": 128, "end_idx": 131, "type": "pro", "entity": "气管插管" }, { "start_idx": 165, "end_idx": 171, "type": "dis", "entity": "胎粪吸入综合征" }, { "start_idx": 173, "end_idx": 198, "type": "dis", "entity": "meconiumaspirationsyndrome" }, { "start_idx": 200, "end_idx": 202, "type": "dis", "entity": "MAS" } ] }, { "text": "(三)时间常数时间常数(timeconstant,TC)是指在一定压力下,送入肺内或呼出一定量气体所需要的时间单位,取决于呼吸系统的顺应性及气道阻力,其计算公式为:TC(sec)=CL(L/cmH2O)×R[cmH2O/(L•sec)]由公式可见:顺应性愈差,气道阻力(包括气管插管和呼吸机管道)愈小,送入肺内气体或呼出气体愈迅速,所需时间愈短,反之亦然。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "时间常数" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "时间常数" }, { "start_idx": 12, "end_idx": 23, "type": "ite", "entity": "timeconstant" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "肺" }, { "start_idx": 61, "end_idx": 73, "type": "ite", "entity": "呼吸系统的顺应性及气道阻力" }, { "start_idx": 82, "end_idx": 83, "type": "ite", "entity": "TC" }, { "start_idx": 152, "end_idx": 155, "type": "ite", "entity": "气道阻力" }, { "start_idx": 159, "end_idx": 162, "type": "pro", "entity": "气管插管" }, { "start_idx": 164, "end_idx": 168, "type": "equ", "entity": "呼吸机管道" }, { "start_idx": 175, "end_idx": 175, "type": "bod", "entity": "肺" } ] }, { "text": "正常足月儿:TC=0.005L/cmH2O×30cmH2O/(L•sec)=0.15sec;RDS患儿:TC=0.001L/cmH2O×30cmH2O/(L•sec)=0.03sec;MAS患儿:TC=0.003L/cmH2O×120cmH2O/(L•sec)=0.36sec。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "TC" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "RDS" }, { "start_idx": 74, "end_idx": 75, "type": "ite", "entity": "TC" }, { "start_idx": 136, "end_idx": 138, "type": "dis", "entity": "MAS" }, { "start_idx": 142, "end_idx": 143, "type": "ite", "entity": "TC" } ] }, { "text": "送入肺内或呼出一定量气体后剩余的潮气量与时间常数有关,其计算公式为:V/Vo=e-TC式中,V:送入肺内或呼出一定量气体后剩余的潮气量;Vo:潮气量;e=2.7134。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肺" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "潮气量" }, { "start_idx": 41, "end_idx": 42, "type": "ite", "entity": "TC" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "肺" }, { "start_idx": 64, "end_idx": 66, "type": "ite", "entity": "潮气量" }, { "start_idx": 71, "end_idx": 73, "type": "ite", "entity": "潮气量" } ] }, { "text": "以呼气时间(timeofexpiration,TE)为例,当TE为一个时间常数(TC=1)时,根据公式V/Vo=0.37,V=Vo×0.37即肺内剩余的气量为潮气量的37%,也就是说,当TE为一个时间常数(TC=1)时,可呼出潮气量的63%;当TE分别为2、3、4、5个时间常数时,呼出气量分别为潮气量的86%、95%、98%、99%。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "ite", "entity": "呼气时间" }, { "start_idx": 6, "end_idx": 21, "type": "ite", "entity": "timeofexpiration" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "TE" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "TE" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "TC" }, { "start_idx": 71, "end_idx": 71, "type": "bod", "entity": "肺" }, { "start_idx": 79, "end_idx": 81, "type": "ite", "entity": "潮气量" }, { "start_idx": 93, "end_idx": 94, "type": "ite", "entity": "TE" }, { "start_idx": 98, "end_idx": 101, "type": "ite", "entity": "时间常数" }, { "start_idx": 103, "end_idx": 104, "type": "ite", "entity": "TC" }, { "start_idx": 113, "end_idx": 115, "type": "ite", "entity": "潮气量" }, { "start_idx": 122, "end_idx": 123, "type": "ite", "entity": "TE" }, { "start_idx": 148, "end_idx": 150, "type": "ite", "entity": "潮气量" } ] }, { "text": "理论上,吸气时间、呼气时间若为5个时间常数,近乎全部的潮气量能进入肺内或排出体外,但临床实践中吸、呼气时间达3~5个时间常数即可。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "时间常数" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "潮气量" }, { "start_idx": 58, "end_idx": 61, "type": "ite", "entity": "时间常数" } ] }, { "text": "当吸气时间(timeofinspiration,TI)短于3~5个时间常数时,调定压力下的潮气量不能全部送入肺内,使实际的吸气峰压(PIP)低于调定的PIP,称为非调定的PIP下降,此时平均气道压力(meanairwaypressure,MAP)也随之下降,故也称为非调定的MAP下降,其结果导致PaO2降低及PaCO2升高;当TE短于3~5个时间常数时,即可产生非调定的呼气末正压。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "ite", "entity": "吸气时间" }, { "start_idx": 6, "end_idx": 22, "type": "ite", "entity": "timeofinspiration" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "TI" }, { "start_idx": 33, "end_idx": 36, "type": "ite", "entity": "时间常数" }, { "start_idx": 45, "end_idx": 47, "type": "ite", "entity": "潮气量" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "肺" }, { "start_idx": 61, "end_idx": 64, "type": "ite", "entity": "吸气峰压" }, { "start_idx": 66, "end_idx": 68, "type": "ite", "entity": "PIP" }, { "start_idx": 75, "end_idx": 77, "type": "ite", "entity": "PIP" }, { "start_idx": 85, "end_idx": 87, "type": "ite", "entity": "PIP" }, { "start_idx": 93, "end_idx": 98, "type": "ite", "entity": "平均气道压力" }, { "start_idx": 100, "end_idx": 117, "type": "ite", "entity": "meanairwaypressure" }, { "start_idx": 119, "end_idx": 121, "type": "ite", "entity": "MAP" }, { "start_idx": 137, "end_idx": 139, "type": "ite", "entity": "MAP" }, { "start_idx": 194, "end_idx": 197, "type": "ite", "entity": "时间常数" } ] }, { "text": "(四)非调定的呼气末正压当应用高呼吸频率(respiratoryrate,RR)通气时,TE短于3个TC,由于呼气时间不够,肺泡内气体不能完全排出,造成气体潴留,使肺泡内呼气末压力高于调定的呼气末正压(positiveend-expirationpressure,PEEP),其高出的PEEP值称为非调定的呼气末正压(inadvertentpositiveend-expirationpressure,iPEEP)。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "ite", "entity": "呼气末正压" }, { "start_idx": 16, "end_idx": 19, "type": "ite", "entity": "呼吸频率" }, { "start_idx": 21, "end_idx": 35, "type": "ite", "entity": "respiratoryrate" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "RR" }, { "start_idx": 44, "end_idx": 45, "type": "ite", "entity": "TE" }, { "start_idx": 50, "end_idx": 51, "type": "ite", "entity": "TC" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "肺泡" }, { "start_idx": 82, "end_idx": 83, "type": "bod", "entity": "肺泡" }, { "start_idx": 95, "end_idx": 99, "type": "ite", "entity": "呼气末正压" }, { "start_idx": 101, "end_idx": 130, "type": "ite", "entity": "positiveend-expirationpressure" }, { "start_idx": 132, "end_idx": 135, "type": "ite", "entity": "PEEP" }, { "start_idx": 142, "end_idx": 145, "type": "ite", "entity": "PEEP" }, { "start_idx": 149, "end_idx": 157, "type": "ite", "entity": "非调定的呼气末正压" }, { "start_idx": 159, "end_idx": 199, "type": "ite", "entity": "inadvertentpositiveend-expirationpressure" }, { "start_idx": 201, "end_idx": 205, "type": "ite", "entity": "iPEEP" } ] }, { "text": "此时功能残气量(functionalresidualcapacity,FRC)增加,肺顺应性和潮气量降低,每分通气量及心搏量减少,PaO2降低及PaCO2升高。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "ite", "entity": "功能残气量" }, { "start_idx": 8, "end_idx": 33, "type": "ite", "entity": "functionalresidualcapacity" }, { "start_idx": 35, "end_idx": 37, "type": "ite", "entity": "FRC" }, { "start_idx": 42, "end_idx": 45, "type": "ite", "entity": "肺顺应性" }, { "start_idx": 47, "end_idx": 49, "type": "ite", "entity": "潮气量" }, { "start_idx": 53, "end_idx": 57, "type": "ite", "entity": "每分通气量" }, { "start_idx": 59, "end_idx": 61, "type": "ite", "entity": "心搏量" } ] }, { "text": "如果调定的PEEP较低,iPEEP则可使萎陷的肺泡在呼气末恢复正常FRC,改善氧合,这可能是对RDS患儿有时增加频率后氧合陡度增加的原因。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "ite", "entity": "PEEP" }, { "start_idx": 12, "end_idx": 16, "type": "ite", "entity": "iPEEP" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺泡" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "FRC" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "RDS" } ] }, { "text": "当然,当产生iPEEP时,呼吸系统也将代偿和限制气体进一步潴留,高FRC使肺顺应性降低,气体潴留则使小气道开放,气道阻力下降,从而缩短相应肺泡的时间常数,在原有TE内,呼出比原来更多的气体,同时高FRC使潮气量减少,故呼出潮气量所需的时间也短,从而缓解气体潴留,达到新的平衡。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "iPEEP" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "潴留" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "FRC" }, { "start_idx": 37, "end_idx": 40, "type": "ite", "entity": "肺顺应性" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "气体潴留" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "小气道" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "气道" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "肺泡" }, { "start_idx": 80, "end_idx": 81, "type": "ite", "entity": "TE" }, { "start_idx": 98, "end_idx": 100, "type": "ite", "entity": "FRC" }, { "start_idx": 102, "end_idx": 104, "type": "ite", "entity": "潮气量" }, { "start_idx": 111, "end_idx": 113, "type": "ite", "entity": "潮气量" } ] }, { "text": "这也可能是调定的PEEP愈高气体潴留愈少和当存在不特别严重气体潴留时肺泡并未破裂的道理所在。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "ite", "entity": "PEEP" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "气体潴留" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肺泡" } ] }, { "text": "气管插管较细及气道分泌物增多使气道阻力增加,也是引起气体潴留的重要原因。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "equ", "entity": "气管插管" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "气道分泌物" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "气道" } ] }, { "text": "值得注意的是呼吸机经近气道测量的PEEP值不能准确反映肺泡内呼气末压力。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "equ", "entity": "呼吸机" }, { "start_idx": 16, "end_idx": 19, "type": "ite", "entity": "PEEP" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肺泡" }, { "start_idx": 30, "end_idx": 34, "type": "ite", "entity": "呼气末压力" } ] }, { "text": "首先根据疾病的种类或肺功能监测,推断和观察CL、R和TC,结合所调定的TE预测其可能性,肺顺应性高或气道阻力大的患儿易引起iPEEP,可应用长TE。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "TC" }, { "start_idx": 35, "end_idx": 36, "type": "ite", "entity": "TE" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "肺顺应性高" }, { "start_idx": 50, "end_idx": 54, "type": "sym", "entity": "气道阻力大" }, { "start_idx": 71, "end_idx": 72, "type": "ite", "entity": "TE" } ] }, { "text": "气体潴留的表现为:桶状胸,胸动幅度小,呼吸音减弱;PaCO2升高;循环障碍,如血压下降、代谢性酸中毒、中心静脉压升高等;胸片示呼气末膈肌低位;肺功能及呼气末闭合气管插管测量其食道或气道压力等方法对发现iPEEP也有一定帮助。", "entities": [ { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "胸" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "胸" }, { "start_idx": 9, "end_idx": 23, "type": "sym", "entity": "桶状胸,胸动幅度小,呼吸音减弱" }, { "start_idx": 25, "end_idx": 42, "type": "sym", "entity": "PaCO2升高" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "循环障碍" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "血压下降" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 62, "end_idx": 68, "type": "sym", "entity": "中心静脉压升高" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "膈肌" }, { "start_idx": 71, "end_idx": 80, "type": "sym", "entity": "胸片示呼气末膈肌低位" }, { "start_idx": 82, "end_idx": 82, "type": "bod", "entity": "肺" }, { "start_idx": 91, "end_idx": 94, "type": "pro", "entity": "气管插管" }, { "start_idx": 98, "end_idx": 99, "type": "bod", "entity": "食道" }, { "start_idx": 101, "end_idx": 102, "type": "bod", "entity": "气道" } ] }, { "text": "有的呼吸机可通过呼气保持按钮获得iPEEP。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "呼吸机" } ] }, { "text": "(五)TC相关的治疗策略TC是针对不同疾病制定机械通气策略的重要理论依据。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "TC" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "TC" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "机械通气" } ] }, { "text": "如上所述,RDS患儿肺顺应性小而气道阻力尚属正常,1个TC仅为0.03秒,3个TC为0.09秒,即使5个TC也只有0.15秒,因此,对RDS极期患儿进行机械通气时,可采用较高频率通气,RDS不至于产生iPEEP;由于RDS以缺氧为主,增加TI可提高MAP即提高PaO2,而RDS所需TE很短,故理论上可应用倒置的吸、呼比即2∶1~4∶1,长TI虽可提高PaO2,但容易造成肺气压伤,故临床已极少应用。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "TC" }, { "start_idx": 39, "end_idx": 40, "type": "ite", "entity": "TC" }, { "start_idx": 52, "end_idx": 53, "type": "ite", "entity": "TC" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "RDS" }, { "start_idx": 76, "end_idx": 79, "type": "pro", "entity": "机械通气" }, { "start_idx": 92, "end_idx": 94, "type": "dis", "entity": "RDS" }, { "start_idx": 112, "end_idx": 113, "type": "sym", "entity": "缺氧" }, { "start_idx": 147, "end_idx": 149, "type": "dis", "entity": "RDS" }, { "start_idx": 152, "end_idx": 153, "type": "ite", "entity": "TE" }, { "start_idx": 208, "end_idx": 211, "type": "dis", "entity": "肺气压伤" } ] }, { "text": "MAS患儿气道阻力明显增加,肺顺应性仅略减小,1个TC仅为0.36秒,3个TC则为1.08秒,因此,对MAS应用机械通气,宜选择慢频率和长TE,如果提高频率,则应降低PEEP,以免造成iPEEP;还可根据MAS病理改变(肺不张、肺气肿和正常肺泡同时存在)进行通气,气肿的肺泡TC长为慢肺泡,而正常的肺泡TC相对短为快肺泡,如果以正常肺泡为通气目标,可根据正常肺泡的TC(3~5个TC为0.45~0.75秒)确定TI和TE,采用中等频率,这样既可保证快肺泡有效通气,又可使进出慢肺泡的气体量减少,避免气肿的肺泡破裂,造成气胸;若以气肿肺泡,气肿肺泡TC确定TI和TE,采用慢频率、长TI和长TE,这样虽保证气肿肺泡的有效通气,却使正常肺泡过度通气,容易发生气胸。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "MAS" }, { "start_idx": 5, "end_idx": 8, "type": "ite", "entity": "气道阻力" }, { "start_idx": 14, "end_idx": 17, "type": "ite", "entity": "肺顺应性" }, { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "TC" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "TC" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "MAS" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "机械通气" }, { "start_idx": 69, "end_idx": 70, "type": "ite", "entity": "TE" }, { "start_idx": 102, "end_idx": 104, "type": "dis", "entity": "MAS" }, { "start_idx": 110, "end_idx": 112, "type": "sym", "entity": "肺不张" }, { "start_idx": 114, "end_idx": 116, "type": "sym", "entity": "肺气肿" }, { "start_idx": 132, "end_idx": 133, "type": "sym", "entity": "气肿" }, { "start_idx": 135, "end_idx": 136, "type": "bod", "entity": "肺泡" }, { "start_idx": 137, "end_idx": 138, "type": "ite", "entity": "TC" }, { "start_idx": 141, "end_idx": 143, "type": "bod", "entity": "慢肺泡" }, { "start_idx": 149, "end_idx": 150, "type": "bod", "entity": "肺泡" }, { "start_idx": 157, "end_idx": 159, "type": "bod", "entity": "快肺泡" }, { "start_idx": 166, "end_idx": 167, "type": "bod", "entity": "肺泡" }, { "start_idx": 179, "end_idx": 180, "type": "bod", "entity": "肺泡" }, { "start_idx": 182, "end_idx": 183, "type": "ite", "entity": "TC" }, { "start_idx": 189, "end_idx": 190, "type": "ite", "entity": "TC" }, { "start_idx": 224, "end_idx": 226, "type": "bod", "entity": "快肺泡" }, { "start_idx": 237, "end_idx": 239, "type": "bod", "entity": "慢肺泡" }, { "start_idx": 249, "end_idx": 250, "type": "sym", "entity": "气肿" }, { "start_idx": 259, "end_idx": 260, "type": "dis", "entity": "气胸" }, { "start_idx": 266, "end_idx": 267, "type": "bod", "entity": "肺泡" }, { "start_idx": 264, "end_idx": 267, "type": "sym", "entity": "气肿肺泡" }, { "start_idx": 271, "end_idx": 272, "type": "bod", "entity": "肺泡" }, { "start_idx": 269, "end_idx": 272, "type": "sym", "entity": "气肿肺泡" }, { "start_idx": 273, "end_idx": 274, "type": "ite", "entity": "TC" }, { "start_idx": 302, "end_idx": 305, "type": "sym", "entity": "气肿肺泡" }, { "start_idx": 316, "end_idx": 317, "type": "bod", "entity": "肺泡" }, { "start_idx": 327, "end_idx": 328, "type": "dis", "entity": "气胸" } ] }, { "text": "第八章溶血尿毒综合征【概述】溶血尿毒综合征(hemolyticuremicsyndrome,HUS)是一种累及多系统、以Grasser三联症(即微血管病性溶血、急性肾衰竭和血小板减少)为主要特征的临床综合征,是小儿急性肾衰竭常见的病因之一。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "溶血尿毒综合征" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "溶血尿毒综合征" }, { "start_idx": 22, "end_idx": 44, "type": "dis", "entity": "hemolyticuremicsyndrome" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "HUS" }, { "start_idx": 60, "end_idx": 69, "type": "dis", "entity": "Grasser三联症" }, { "start_idx": 72, "end_idx": 78, "type": "sym", "entity": "微血管病性溶血" }, { "start_idx": 80, "end_idx": 84, "type": "sym", "entity": "急性肾衰竭" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "血小板" }, { "start_idx": 86, "end_idx": 90, "type": "sym", "entity": "血小板减少" }, { "start_idx": 105, "end_idx": 111, "type": "dis", "entity": "小儿急性肾衰竭" } ] }, { "text": "1/3以上的HUS患儿可有神经系统受累的表现。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "HUS" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "神经系统" } ] }, { "text": "由于HUS与血栓性血小板减少性紫癜(thromboticthrombocytopenicpurpura,TTP)在病因、发病机制、病理改变和临床表现方面难以精确区分,目前越来越多的学者认为两者是同一疾病不同的临床表现,可统称之为HUS/TTP或血栓性微血管病(thromboticmicroangiopathy,TMA)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "HUS" }, { "start_idx": 6, "end_idx": 16, "type": "dis", "entity": "血栓性血小板减少性紫癜" }, { "start_idx": 18, "end_idx": 50, "type": "dis", "entity": "thromboticthrombocytopenicpurpura" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "TTP" }, { "start_idx": 114, "end_idx": 120, "type": "dis", "entity": "HUS/TTP" }, { "start_idx": 122, "end_idx": 128, "type": "dis", "entity": "血栓性微血管病" }, { "start_idx": 130, "end_idx": 154, "type": "dis", "entity": "thromboticmicroangiopathy" }, { "start_idx": 156, "end_idx": 158, "type": "dis", "entity": "TMA" } ] }, { "text": "HUS为病变以肾脏累及为主的肾限性TMA,肾衰竭是其主要特征;TTP则为系统性TMA,表现以神经系统症状为主。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "HUS" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肾脏" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "肾限性TMA" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "肾衰竭" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "TTP" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "系统性TMA" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "神经系统" } ] }, { "text": "随着诊疗技术的日趋完善,HUS/TTP的预后已有所改观。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "HUS/TTP" } ] }, { "text": "【流行病学】全球不同国家和地区虽均有HUS散发或流行的报道,但本病有一定的地域性,以阿根廷、荷兰、南非、美国加利福尼亚州和加拿大魁北克省居多。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "HUS" } ] }, { "text": "美国儿童HUS发病率为0.3/10万~10/10万,澳大利亚等国5岁以下儿童为1.35/10万~5.8/10万,日本的HUS病例数则有逐年增多趋势。", "entities": [ { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "HUS" } ] }, { "text": "各年龄段均可发生HUS,伴以腹泻的HUS发病高峰年龄为6个月~4岁,无性别差异。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "HUS" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "腹泻" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "HUS" } ] }, { "text": "【病因及发病机制】(一)病因尚不明确,下列外源或内源性因素可能与HUS的发病有关。", "entities": [ { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "HUS" } ] }, { "text": "1.感染是诱发儿童HUS的首要因素,根据诱因可以志贺样毒素相关HUS和非志贺样毒素相关HUS。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "HUS" }, { "start_idx": 24, "end_idx": 33, "type": "dis", "entity": "志贺样毒素相关HUS" }, { "start_idx": 35, "end_idx": 45, "type": "dis", "entity": "非志贺样毒素相关HUS" } ] }, { "text": "儿童暴发流行的EHECO157:H7感染中,可有高达53%的患者发生HUS。", "entities": [ { "start_idx": 7, "end_idx": 19, "type": "dis", "entity": "EHECO157:H7感染" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "HUS" } ] }, { "text": "2.药物长春新碱、丝裂霉素、顺铂、氟尿嘧啶、柔红霉素、阿糖胞苷等抗肿瘤药物可引起化疗相关性HUS,环孢霉素等免疫抑制剂也可诱发HUS,偶见奎宁引起HUS的报道。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "长春新碱" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "丝裂霉素" }, { "start_idx": 14, "end_idx": 15, "type": "dru", "entity": "顺铂" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "氟尿嘧啶" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "柔红霉素" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "阿糖胞苷" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "HUS" }, { "start_idx": 49, "end_idx": 52, "type": "dru", "entity": "环孢霉素" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "HUS" }, { "start_idx": 69, "end_idx": 70, "type": "dru", "entity": "奎宁" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "HUS" } ] }, { "text": "3.器官移植骨髓移植及肾移植后均可发生HUS,发生率分别为3.4%和6%~9%。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "器官移植" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "骨髓移植" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "肾移植" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "HUS" } ] }, { "text": "一旦发生骨髓移植后HUS,预后凶险,可能与大剂量化疗、放疗、排异反应、感染等有关。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "骨髓移植" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "HUS" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "化疗" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "放疗" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "排异反应" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "感染" } ] }, { "text": "4.免疫缺陷病如先天性无丙种球蛋白血症和胸腺无淋巴细胞增生症。", "entities": [ { "start_idx": 8, "end_idx": 18, "type": "dis", "entity": "先天性无丙种球蛋白血症" }, { "start_idx": 20, "end_idx": 29, "type": "dis", "entity": "胸腺无淋巴细胞增生症" } ] }, { "text": "5.遗传及基因突变HUS可在同一家族的兄弟姐妹中相继发病。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "HUS" } ] }, { "text": "目前认为其为常染色体隐性遗传,系血管性血友病因子裂解蛋白酶(vonWillebrandfactorcleavingprotease,vWF-CP又称ADAMTS-13,adisintegrinmetalloproteinasewiththrombospondintypeImotif,member13)重度缺乏,导致vWF多聚体增多,损伤内皮细胞。", "entities": [ { "start_idx": 16, "end_idx": 28, "type": "bod", "entity": "血管性血友病因子裂解蛋白酶" }, { "start_idx": 30, "end_idx": 64, "type": "bod", "entity": "vonWillebrandfactorcleavingprotease" }, { "start_idx": 66, "end_idx": 71, "type": "bod", "entity": "vWF-CP" }, { "start_idx": 74, "end_idx": 82, "type": "bod", "entity": "ADAMTS-13" }, { "start_idx": 84, "end_idx": 140, "type": "bod", "entity": "adisintegrinmetalloproteinasewiththrombospondintypeImotif" }, { "start_idx": 142, "end_idx": 149, "type": "bod", "entity": "member13" }, { "start_idx": 158, "end_idx": 163, "type": "bod", "entity": "vWF多聚体" }, { "start_idx": 169, "end_idx": 172, "type": "bod", "entity": "内皮细胞" } ] }, { "text": "家族性HUS预后不良,病死率达68%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "家族性HUS" } ] }, { "text": "近年来,也从一些HUS患者中发现有补体调节因子基因突变现象,如补体因子H(complementfactorH,CFH)、补体因子Ⅰ(complementfactorⅠ,CFI)及补体膜辅助蛋白(membranecofactorprotein,MCP)基因。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "HUS" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "补体调节因子" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "补体因子H" }, { "start_idx": 37, "end_idx": 53, "type": "bod", "entity": "complementfactorH" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "CFH" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "补体因子Ⅰ" }, { "start_idx": 66, "end_idx": 82, "type": "bod", "entity": "complementfactorⅠ" }, { "start_idx": 84, "end_idx": 86, "type": "bod", "entity": "CFI" }, { "start_idx": 89, "end_idx": 95, "type": "bod", "entity": "补体膜辅助蛋白" }, { "start_idx": 97, "end_idx": 119, "type": "bod", "entity": "membranecofactorprotein" }, { "start_idx": 121, "end_idx": 123, "type": "bod", "entity": "MCP" } ] }, { "text": "6.其他一些自身免疫相关性疾病如系统性红斑狼疮、类风湿性关节炎、抗磷脂抗体综合征、恶性肿瘤及妊娠,均可引起HUS,成人多见。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "类风湿性关节炎" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "抗磷脂抗体综合征" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "妊娠" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "HUS" } ] }, { "text": "(二)发病机制HUS的发病机制尚不明确。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "HUS" } ] }, { "text": "不同致病因素引起HUS的发病机制不尽相同,但毛细血管内皮细胞损伤是其共同的致病途径。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "HUS" }, { "start_idx": 22, "end_idx": 29, "type": "bod", "entity": "毛细血管内皮细胞" }, { "start_idx": 22, "end_idx": 31, "type": "sym", "entity": "毛细血管内皮细胞损伤" } ] }, { "text": "受损的内皮细胞启动凝血系统,致血小板在局部聚集、血栓形成和纤维蛋白沉积,使红细胞和血小板流经时遭受机械损伤而破坏,引起微血管性血栓、溶血性贫血和血小板减少;在肾脏,微血管性血栓致肾内循环障碍,进而发生急性肾衰竭。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "血小板" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "血小板在局部聚集" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "血栓形成" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "纤维蛋白" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "纤维蛋白沉积" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "红细胞" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "血小板" }, { "start_idx": 37, "end_idx": 55, "type": "sym", "entity": "红细胞和血小板流经时遭受机械损伤而破坏" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "血小板" }, { "start_idx": 57, "end_idx": 76, "type": "sym", "entity": "引起微血管性血栓、溶血性贫血和血小板减少" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "肾脏" }, { "start_idx": 82, "end_idx": 87, "type": "sym", "entity": "微血管性血栓" }, { "start_idx": 89, "end_idx": 89, "type": "bod", "entity": "肾" }, { "start_idx": 100, "end_idx": 104, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "近年的研究认为HUS发病机制涉及以下几个方面:1.内毒素及神经氨酸酶致内皮细胞受损EHEC在肠道内产生内毒素,主要有两种:一是志贺样毒素(shiga-liketoxin,SLT),又称维罗毒素(verotoxin,VT),可结合到内皮细胞表面的糖脂质受体(globotriaosylceramide,GB3)上,经吞噬进入胞质后分解为A链和B链。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "HUS" }, { "start_idx": 25, "end_idx": 27, "type": "mic", "entity": "内毒素" }, { "start_idx": 29, "end_idx": 33, "type": "mic", "entity": "神经氨酸酶" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "EHEC" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "肠道" }, { "start_idx": 63, "end_idx": 67, "type": "mic", "entity": "志贺样毒素" }, { "start_idx": 69, "end_idx": 83, "type": "mic", "entity": "shiga-liketoxin" }, { "start_idx": 85, "end_idx": 87, "type": "mic", "entity": "SLT" }, { "start_idx": 92, "end_idx": 95, "type": "mic", "entity": "维罗毒素" }, { "start_idx": 97, "end_idx": 105, "type": "mic", "entity": "verotoxin" }, { "start_idx": 107, "end_idx": 108, "type": "mic", "entity": "VT" }, { "start_idx": 115, "end_idx": 118, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 122, "end_idx": 126, "type": "bod", "entity": "糖脂质受体" }, { "start_idx": 128, "end_idx": 148, "type": "bod", "entity": "globotriaosylceramide" }, { "start_idx": 150, "end_idx": 163, "type": "bod", "entity": "GB3" }, { "start_idx": 172, "end_idx": 173, "type": "bod", "entity": "胞质" } ] }, { "text": "A链可裂解核糖体转运RNA的腺嘌呤,使蛋白合成障碍致细胞受损或死亡;SLTs尚有诱导肾细胞凋亡作用,细胞凋亡在HUS的发病过程中起一定作用,且凋亡细胞数与疾病严重度相关。", "entities": [ { "start_idx": 5, "end_idx": 16, "type": "bod", "entity": "核糖体转运RNA的腺嘌呤" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "细胞" }, { "start_idx": 34, "end_idx": 37, "type": "mic", "entity": "SLTs" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "肾细胞" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "HUS" } ] }, { "text": "另一种为细菌脂多糖(lipopolysaccharide,LPS),LPS通过上调纤溶酶原激活抑制剂(plasminogenactivatorinhibitor,PAI)和下调血栓调节素表达而损伤内皮细胞,促进血栓形成。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "mic", "entity": "细菌脂多糖" }, { "start_idx": 10, "end_idx": 27, "type": "mic", "entity": "lipopolysaccharide" }, { "start_idx": 29, "end_idx": 31, "type": "mic", "entity": "LPS" }, { "start_idx": 34, "end_idx": 36, "type": "mic", "entity": "LPS" }, { "start_idx": 41, "end_idx": 49, "type": "bod", "entity": "纤溶酶原激活抑制剂" }, { "start_idx": 51, "end_idx": 79, "type": "bod", "entity": "plasminogenactivatorinhibitor" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "PAI" }, { "start_idx": 88, "end_idx": 92, "type": "bod", "entity": "血栓调节素" }, { "start_idx": 98, "end_idx": 101, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 105, "end_idx": 106, "type": "dis", "entity": "血栓" } ] }, { "text": "LPS尚可促进白细胞和血小板黏附在内皮细胞上。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "LPS" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "白细胞" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "血小板" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "内皮细胞" } ] }, { "text": "肺炎链球菌产生的神经氨酸酶可分解掉N-乙酰神经氨酸,使被其掩盖的T-F抗原(Thomson-Friedenreich抗原)暴露于循环IgM抗体,IgM抗体与血小板和内皮细胞上的T-F抗原结合,导致血小板凝聚和内皮细胞损伤。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "神经氨酸酶" }, { "start_idx": 17, "end_idx": 24, "type": "bod", "entity": "N-乙酰神经氨酸" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "T-F抗原" }, { "start_idx": 38, "end_idx": 59, "type": "bod", "entity": "Thomson-Friedenreich抗原" }, { "start_idx": 66, "end_idx": 70, "type": "bod", "entity": "IgM抗体" }, { "start_idx": 72, "end_idx": 76, "type": "bod", "entity": "IgM抗体" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "血小板" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 88, "end_idx": 92, "type": "bod", "entity": "T-F抗原" }, { "start_idx": 98, "end_idx": 100, "type": "bod", "entity": "血小板" }, { "start_idx": 98, "end_idx": 102, "type": "sym", "entity": "血小板凝聚" }, { "start_idx": 104, "end_idx": 107, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 104, "end_idx": 109, "type": "sym", "entity": "内皮细胞损伤" } ] }, { "text": "2.细胞因子作用许多细胞因子参与HUS发病,肿瘤坏死因子(tumornecrosisfactor,TNF)、白细胞介素-6(IL-6)、IL-8、IL-1β等释放增加。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "细胞因子" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "细胞因子" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "HUS" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "肿瘤坏死因子" }, { "start_idx": 29, "end_idx": 47, "type": "bod", "entity": "tumornecrosisfactor" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "TNF" }, { "start_idx": 54, "end_idx": 60, "type": "bod", "entity": "白细胞介素-6" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "IL-6" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "IL-8" }, { "start_idx": 73, "end_idx": 77, "type": "bod", "entity": "IL-1β" } ] }, { "text": "TNF可诱导上皮细胞促凝血活性及GB3受体表达;IL-6是疾病活动性的一个标志物,与疾病严重程度和预后有关;IL-8是一种白细胞激活剂,白细胞激活后释放弹力蛋白酶,使其与内皮细胞黏附性增高,参与发病并加重病损。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "TNF" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 16, "end_idx": 29, "type": "bod", "entity": "GB3" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "IL-6" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "IL-8" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "白细胞" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "白细胞" }, { "start_idx": 87, "end_idx": 91, "type": "bod", "entity": "弹力蛋白酶" }, { "start_idx": 96, "end_idx": 99, "type": "bod", "entity": "内皮细胞" } ] }, { "text": "3.前列环素(prostacyclin,PGI2)和血栓素A2(thromboxane,TXA2)失衡正常内皮细胞可合成PGI2,具扩张血管和抑制血小板聚集作用,与促进血小板凝聚的TXA2保持动态平衡。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "前列环素" }, { "start_idx": 7, "end_idx": 18, "type": "bod", "entity": "prostacyclin" }, { "start_idx": 20, "end_idx": 34, "type": "bod", "entity": "PGI2" }, { "start_idx": 37, "end_idx": 52, "type": "bod", "entity": "血栓素A2" }, { "start_idx": 54, "end_idx": 64, "type": "bod", "entity": "thromboxane" }, { "start_idx": 66, "end_idx": 80, "type": "bod", "entity": "TXA2" }, { "start_idx": 86, "end_idx": 89, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 93, "end_idx": 107, "type": "bod", "entity": "PGI2" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "血管" }, { "start_idx": 117, "end_idx": 119, "type": "bod", "entity": "血小板" }, { "start_idx": 128, "end_idx": 130, "type": "bod", "entity": "血小板" }, { "start_idx": 134, "end_idx": 148, "type": "bod", "entity": "TXA2" } ] }, { "text": "本征患者PGI2低下,可能与发病有关,推测患儿缺少某种刺激产生PGI2的血浆因子或存在PGI2合成酶抑制物,尚有可能HUS患者对PGI2降解加快有关。", "entities": [ { "start_idx": 4, "end_idx": 18, "type": "bod", "entity": "PGI2" }, { "start_idx": 42, "end_idx": 56, "type": "bod", "entity": "PGI2" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "血浆因子" }, { "start_idx": 65, "end_idx": 85, "type": "bod", "entity": "PGI2合成酶抑制物" }, { "start_idx": 91, "end_idx": 93, "type": "dis", "entity": "HUS" }, { "start_idx": 97, "end_idx": 111, "type": "bod", "entity": "PGI2" } ] }, { "text": "4.凝血与纤溶系统异常促血小板凝聚物质如血小板激活因子(PAF)、体内存在vWF-CP抗体使血管性血友病因子(vWF)多聚体异常增多;血小板释放产物如β-血栓球蛋白(β-TG)等增加;内皮细胞释放组织因子,激活凝血系统,微血栓广泛形成;纤溶破坏,D-二聚体和纤溶酶原激活物抑制因子(plasminogenactivatorinhibitor,PAI)降低。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "凝血" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "纤溶系统" }, { "start_idx": 11, "end_idx": 18, "type": "bod", "entity": "促血小板凝聚物质" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "血小板激活因子" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "PAF" }, { "start_idx": 37, "end_idx": 44, "type": "bod", "entity": "vWF-CP抗体" }, { "start_idx": 46, "end_idx": 61, "type": "bod", "entity": "血管性血友病因子(vWF)多聚体" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "血小板" }, { "start_idx": 75, "end_idx": 81, "type": "bod", "entity": "β-血栓球蛋白" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "β-TG" }, { "start_idx": 92, "end_idx": 95, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 98, "end_idx": 101, "type": "bod", "entity": "组织因子" }, { "start_idx": 105, "end_idx": 108, "type": "bod", "entity": "凝血系统" }, { "start_idx": 110, "end_idx": 112, "type": "sym", "entity": "微血栓" }, { "start_idx": 123, "end_idx": 139, "type": "bod", "entity": "D-二聚体和纤溶酶原激活物抑制因子" }, { "start_idx": 141, "end_idx": 169, "type": "bod", "entity": "plasminogenactivatorinhibitor" }, { "start_idx": 171, "end_idx": 173, "type": "bod", "entity": "PAI" } ] }, { "text": "5.其他有学者注意到,内皮素-一氧化氮轴和免疫功能紊乱在HUS的发病中也可能起到一定作用。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "bod", "entity": "内皮素-一氧化氮轴" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "免疫功能紊乱" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "HUS" } ] }, { "text": "【病理】肾脏病理改变以血管内血小板聚集伴纤维素沉积、微血栓形成为特点,分三型。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肾脏" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血管" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "血小板" }, { "start_idx": 11, "end_idx": 24, "type": "sym", "entity": "血管内血小板聚集伴纤维素沉积" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "微血栓" } ] }, { "text": "(一)肾小球型小儿多见,肾小球毛细血管内皮细胞肿胀、脱落,内皮细胞下间歇增宽,可见系膜细胞插入现象。", "entities": [ { "start_idx": 12, "end_idx": 22, "type": "bod", "entity": "肾小球毛细血管内皮细胞" }, { "start_idx": 12, "end_idx": 27, "type": "sym", "entity": "肾小球毛细血管内皮细胞肿胀、脱落" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 29, "end_idx": 37, "type": "sym", "entity": "内皮细胞下间歇增宽" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "系膜细胞" }, { "start_idx": 39, "end_idx": 48, "type": "sym", "entity": "可见系膜细胞插入现象" } ] }, { "text": "肾小球毛细血管腔狭窄、有微血栓形成和节段性纤维素性坏死。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "bod", "entity": "肾小球毛细血管腔" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "肾小球毛细血管腔狭窄" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "微血栓" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "节段性纤维素性坏死" } ] }, { "text": "可见新月体形成。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "可见新月体形成" } ] }, { "text": "(二)血管型以入球小动脉、小叶间和弓状动脉分支为主,可见动脉内膜水肿、纤维素坏死、血管腔内血栓形成、血栓机化、血管内膜葱皮样增生。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "入球小动脉" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "小叶间" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "弓状动脉分支" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "动脉内膜" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "动脉内膜水肿" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "纤维素" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "纤维素坏死" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "血管腔" }, { "start_idx": 41, "end_idx": 48, "type": "sym", "entity": "血管腔内血栓形成" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "血栓机化" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "血管内膜" }, { "start_idx": 55, "end_idx": 63, "type": "sym", "entity": "血管内膜葱皮样增生" } ] }, { "text": "(三)皮质坏死型是较大的肾内动脉血栓形成和闭塞的后果。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "肾内动脉" } ] }, { "text": "免疫荧光检查可见肾小球内纤维蛋白原沉积,有时见IgM及C3沉积在肾小球毛细血管壁。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "免疫荧光检查" }, { "start_idx": 8, "end_idx": 16, "type": "bod", "entity": "肾小球内纤维蛋白原" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "肾小球内纤维蛋白原沉积" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "IgM" }, { "start_idx": 27, "end_idx": 39, "type": "bod", "entity": "C3" }, { "start_idx": 43, "end_idx": 50, "type": "bod", "entity": "肾小球毛细血管壁" }, { "start_idx": 20, "end_idx": 50, "type": "sym", "entity": "有时见IgM及C3沉积在肾小球毛细血管壁" } ] }, { "text": "【临床表现】临床表现典型者常有前驱症状,以胃肠道表现为主,多有腹痛、腹泻和呕吐,可有发热、嗜睡、乏力、食欲缺乏等非特异性表现。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "胃肠道" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "腹痛" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "腹泻" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "呕吐" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "发热" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "嗜睡" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "乏力" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "食欲缺乏" } ] }, { "text": "患儿明显苍白,临床所见黄疸不显著或仅面部呈柠檬黄色。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "患儿明显苍白" }, { "start_idx": 7, "end_idx": 24, "type": "sym", "entity": "临床所见黄疸不显著或仅面部呈柠檬黄色" } ] }, { "text": "心血管系统受损表现为高血压、心律失常和心功能不全;胰腺受损者可出现暂时性或永久性胰腺内分泌功能不全;可有短暂的肝损害,偶见胆汁郁积性黄疸;肺、肌肉、皮肤及视网膜损害少见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "心血管系统" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "高血压" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "心律失常" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "心功能不全" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "胰腺" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "胰腺" }, { "start_idx": 25, "end_idx": 48, "type": "sym", "entity": "胰腺受损者可出现暂时性或永久性胰腺内分泌功能不全" }, { "start_idx": 50, "end_idx": 57, "type": "sym", "entity": "可有短暂的肝损害" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "胆汁" }, { "start_idx": 59, "end_idx": 67, "type": "sym", "entity": "偶见胆汁郁积性黄疸" }, { "start_idx": 69, "end_idx": 69, "type": "bod", "entity": "肺" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "肌肉" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "皮肤" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "视网膜" }, { "start_idx": 69, "end_idx": 83, "type": "sym", "entity": "肺、肌肉、皮肤及视网膜损害少见" } ] }, { "text": "临床依病情轻重分为轻型和重型,重型标准包括:血红蛋白<60g/L、BUN≥17.9mmol/L及有少尿或无尿和(或)严重并发症(如高血压脑病、肺水肿等)。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "高血压脑病" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "肺水肿" } ] }, { "text": "【实验室检查】(一)血液检查血常规示血红蛋白和血细胞比容下降、血小板下降,镜检可见异型红细胞及碎片,网织红细胞计数增高。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "血液检查" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "血常规" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "血细胞" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "血小板" }, { "start_idx": 37, "end_idx": 38, "type": "pro", "entity": "镜检" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "红细胞" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "碎片" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "网织红细胞" }, { "start_idx": 39, "end_idx": 58, "type": "sym", "entity": "可见异型红细胞及碎片,网织红细胞计数增高" } ] }, { "text": "生化检查示有代谢性酸中毒、高血钾、高血磷和低血钙、稀释性低血钠、氮质血症、胆红素及转氨酶增高、总蛋白和清蛋白降低。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "生化检查" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "代谢性酸中毒" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "高血钾" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "高血磷" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "低血钙" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "稀释性低血钠" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "氮质血症" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "胆红素" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "转氨酶" }, { "start_idx": 37, "end_idx": 45, "type": "sym", "entity": "胆红素及转氨酶增高" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "总蛋白" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "清蛋白" }, { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "总蛋白和清蛋白降低" } ] }, { "text": "血乳酸脱氢酶增高。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "血乳酸脱氢酶" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "血乳酸脱氢酶增高" } ] }, { "text": "可见补体C3水平降低。", "entities": [ { "start_idx": 2, "end_idx": 16, "type": "bod", "entity": "补体C3" }, { "start_idx": 0, "end_idx": 20, "type": "sym", "entity": "可见补体C3水平降低" } ] }, { "text": "累及胰腺者有高血糖。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "胰腺" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "高血糖" } ] }, { "text": "凝血因子检查结果与病程有关,早期可有凝血酶原时间延长、纤维蛋白原降低、纤维蛋白降解产物增高及凝血Ⅱ、Ⅷ、Ⅸ及Ⅹ因子减少,但数天后即恢复正常。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "凝血因子检查" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 18, "end_idx": 25, "type": "sym", "entity": "凝血酶原时间延长" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "纤维蛋白原" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "纤维蛋白原降低" }, { "start_idx": 35, "end_idx": 42, "type": "bod", "entity": "纤维蛋白降解产物" }, { "start_idx": 35, "end_idx": 44, "type": "sym", "entity": "纤维蛋白降解产物增高" }, { "start_idx": 46, "end_idx": 56, "type": "bod", "entity": "凝血Ⅱ、Ⅷ、Ⅸ及Ⅹ因子" }, { "start_idx": 46, "end_idx": 58, "type": "sym", "entity": "凝血Ⅱ、Ⅷ、Ⅸ及Ⅹ因子减少" } ] }, { "text": "(二)尿检查血尿、蛋白尿和血红蛋白尿,尿沉渣镜检有红细胞碎片、白细胞及管型。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "尿检查" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "血尿" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 13, "end_idx": 17, "type": "ite", "entity": "血红蛋白尿" }, { "start_idx": 19, "end_idx": 23, "type": "ite", "entity": "尿沉渣镜检" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "红细胞碎片" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "白细胞" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "管型" } ] }, { "text": "(三)粪便检查典型的腹泻后HUS有赖于粪便细菌培养和血清学分型。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "粪便检查" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "腹泻" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "HUS" }, { "start_idx": 19, "end_idx": 24, "type": "ite", "entity": "粪便细菌培养" } ] }, { "text": "用免疫磁分离技术(immunomagneticseparation)分离EHECO157:H7,较培养方便快速。", "entities": [ { "start_idx": 36, "end_idx": 46, "type": "bod", "entity": "EHECO157:H7" } ] }, { "text": "【诊断】患儿有前驱胃肠症状史,临床见急性溶血性贫血、血小板减少和肾功能急性减退,表现为苍白、尿量减少,尿检红细胞、蛋白及管型,血象呈贫血状,血小板下降,涂片见异型红细胞和碎片,血生化示急性肾衰竭改变,即可诊断本症。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胃肠" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "急性溶血性贫血" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "血小板" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "血小板减少" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "肾功能急性减退" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "苍白" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "尿量减少" }, { "start_idx": 51, "end_idx": 52, "type": "ite", "entity": "尿检" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "红细胞" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "蛋白" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "管型" }, { "start_idx": 51, "end_idx": 61, "type": "sym", "entity": "尿检红细胞、蛋白及管型" }, { "start_idx": 63, "end_idx": 68, "type": "sym", "entity": "血象呈贫血状" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "血小板" }, { "start_idx": 70, "end_idx": 74, "type": "sym", "entity": "血小板下降" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "红细胞" }, { "start_idx": 76, "end_idx": 86, "type": "sym", "entity": "涂片见异型红细胞和碎片" }, { "start_idx": 88, "end_idx": 90, "type": "ite", "entity": "血生化" }, { "start_idx": 88, "end_idx": 98, "type": "sym", "entity": "血生化示急性肾衰竭改变" } ] }, { "text": "婴儿期应注意与中毒性或缺血性肾小管坏死区别,年长儿则应与结缔组织病所致肾脏病变鉴别。", "entities": [ { "start_idx": 7, "end_idx": 18, "type": "dis", "entity": "中毒性或缺血性肾小管坏死" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "肾脏" } ] }, { "text": "【治疗】对HUS的治疗强调加强支持、早期透析和积极对症处理的原则。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "HUS" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "早期透析" } ] }, { "text": "(一)支持疗法及早加强营养支持、维持水和电解质平衡及控制高血压。", "entities": [ { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "高血压" } ] }, { "text": "(二)透析疗法早期透析可明显改善急性期症状,降低病死率。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "透析" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "早期透析" } ] }, { "text": "适应证为无尿>12小时、氮质血症伴脑病或BUN>53.55mmol/L、血钾>6mmol/L和(或)心功能衰竭、顽固性高血压者。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "无尿>12小时" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "氮质血症" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "脑病" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "心功能衰竭" }, { "start_idx": 56, "end_idx": 61, "type": "dis", "entity": "顽固性高血压" }, { "start_idx": 12, "end_idx": 62, "type": "sym", "entity": "氮质血症伴脑病或BUN>53.55mmol/L、血钾>6mmol/L和(或)心功能衰竭、顽固性高血压者" } ] }, { "text": "目前,在儿科较为广泛使用的是腹膜透析,也可采用血液透析。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "血液透析" } ] }, { "text": "(三)血浆置换疗法传统采用血浆输入技术,近年来血浆置换技术被广泛采用。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "血浆置换疗法" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "血浆" }, { "start_idx": 23, "end_idx": 28, "type": "pro", "entity": "血浆置换技术" } ] }, { "text": "由于除了补充血浆成分,血浆置换可以清除血液中的有害毒素和炎症因子,已经证实,血浆置换比血浆输入效果显著。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血浆" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "血浆置换" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血液" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "血浆置换" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "血浆输入" } ] }, { "text": "血浆补充或置换能补充刺激PGI2生成的血浆因子,去除PGI2合成的抑制物。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "血浆补充或置换" }, { "start_idx": 12, "end_idx": 26, "type": "bod", "entity": "PGI2" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "血浆因子" }, { "start_idx": 37, "end_idx": 51, "type": "bod", "entity": "PGI2" } ] }, { "text": "当出现肾功能不全或者心力衰竭时,血浆交换更是第一选择,或合用血液透析技术。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "肾功能不全" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "血浆交换" }, { "start_idx": 30, "end_idx": 35, "type": "pro", "entity": "血液透析技术" } ] }, { "text": "血浆处理应在征兆出现的24小时内,通常血浆交换量每次40ml/kg,每天或隔天置换1次,3~4次后逐渐减少,增加血浆置换量能提高治疗效果;不耐受患儿,可以每天分2次进行置换,以减少输入的循环血浆,血浆的置换量第一天30~40ml/kg,此后10~20ml/kg,每天或隔天置换1次,3~4次后逐渐减少,直至完全缓解。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血浆" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血浆" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "血浆置换" }, { "start_idx": 84, "end_idx": 85, "type": "pro", "entity": "置换" }, { "start_idx": 95, "end_idx": 96, "type": "bod", "entity": "血浆" }, { "start_idx": 98, "end_idx": 99, "type": "bod", "entity": "血浆" } ] }, { "text": "(四)甲基泼尼松龙冲击治疗能控制溶血的发展,促进肾损伤的恢复。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "甲基泼尼松龙冲击治疗" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "溶血" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "肾" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "肾损伤" } ] }, { "text": "(五)其他疗法抗生素、肝素及链激酶、抗血栓制剂(阿司匹林、双嘧达莫)、纤溶药物和维生素E等疗效不确切,一般并不提倡。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "抗生素" }, { "start_idx": 11, "end_idx": 12, "type": "dru", "entity": "肝素" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "链激酶" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "抗血栓制剂" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "纤溶药物" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "维生素E" } ] }, { "text": "对有血小板聚集者,可用PGI2静滴,其机制可能为抑制肾小球内血栓形成,利于肾功能恢复。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "血小板" }, { "start_idx": 11, "end_idx": 25, "type": "dru", "entity": "PGI2" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "静滴" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肾小球" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "肾" } ] }, { "text": "初始剂量为每分钟2.5ng/kg,1周内逐渐加量,疗程8~12天;也可用前列腺素E110μg/次,1~2次/天,用7~10天。", "entities": [ { "start_idx": 36, "end_idx": 52, "type": "dru", "entity": "前列腺素E1" } ] }, { "text": "剂量大时可致低血压及心律改变。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "低血压" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "心律改变" } ] }, { "text": "关于急性期后治疗:急性期后指患儿溶血停止,以乳酸脱氢酶下降、血红蛋白和血小板开始回升为标志。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "溶血停止" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "乳酸脱氢酶" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "乳酸脱氢酶下降" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "血小板" }, { "start_idx": 30, "end_idx": 41, "type": "sym", "entity": "血红蛋白和血小板开始回升" } ] }, { "text": "此时,患儿仍有持续尿检异常、反复高血压和肾功能不全。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "尿检" }, { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "仍有持续尿检异常" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "反复高血压和肾功能不全" } ] }, { "text": "有国内学者建议参照中华医学会儿科学分会肾脏病学组制定的“小儿肾小球疾病的临床分类、诊断及治疗方案”中关于紫癜性肾炎和狼疮性肾炎的“临床分型”和“根据临床表现参照病理类型制定治疗方案”的内容进行用药,可明显改善预后。", "entities": [ { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "小儿肾小球疾病" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "紫癜性肾炎" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "狼疮性肾炎" } ] }, { "text": "有高血压者可长期用ACEI控制。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "sym", "entity": "高血压" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "ACEI" } ] }, { "text": "对急性期过后暂时无高血压者需进行长期随访,必要时行24小时血压监测或踏步车试验,以便早期发现和治疗高血压,延缓肾损害。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "ite", "entity": "血压监测" }, { "start_idx": 34, "end_idx": 38, "type": "pro", "entity": "踏步车试验" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "高血压" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "肾" } ] }, { "text": "【预后】由于对HUS认识的提高和透析技术的广泛应用,病死率已降至10%以下。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "HUS" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "透析" } ] }, { "text": "远期预后与临床肾脏损害程度及肾脏组织学受损范围有关。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肾脏" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "肾脏组织学" } ] }, { "text": "二、尿液生化检查(一)蛋白质正常健康小儿尿中有微量的蛋白,主要有白蛋白、溶菌酶、糖蛋白和脂蛋白等,其中一半来自血浆,另一半为脱落上皮、腺体分泌物及肾小管分泌的Tamm-Horsfall黏蛋白。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "尿液生化检查" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "蛋白质" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "尿" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "蛋白" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "白蛋白" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "溶菌酶" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "糖蛋白" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "脂蛋白" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "血浆" }, { "start_idx": 64, "end_idx": 71, "type": "bod", "entity": "上皮、腺体分泌物" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "肾小管" } ] }, { "text": "正常时每天由尿中排泄的蛋白<150mg。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "尿" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "蛋白" } ] }, { "text": "1.尿蛋白定性测量临床常用的定性方法有磺基水杨酸法、蛋白试纸法及加热醋酸法。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "尿蛋白定性测量" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "磺基水杨酸法" }, { "start_idx": 26, "end_idx": 30, "type": "pro", "entity": "蛋白试纸法" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "加热醋酸法" } ] }, { "text": "(1)磺基水杨酸法:用20%的该试剂2~3滴加入放有尿液的平板上或试管中,在黑色背景下观察,根据其浑浊度判定结果。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "磺基水杨酸法" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "尿液" }, { "start_idx": 33, "end_idx": 34, "type": "equ", "entity": "试管" } ] }, { "text": "该方法敏感度高,可检出0.05~0.1g/L的蛋白尿,但在高浓度的尿酸、草酸盐和黏蛋白存在时或当青霉素钾盐≥4万U/ml时可出现假阳性。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "尿酸" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "草酸盐" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "黏蛋白" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "青霉素钾盐" } ] }, { "text": "(2)蛋白试纸法:用四溴酚蓝作蛋白指示剂,当有蛋白存在时,蘸有尿液的试纸在10~60秒钟内由黄色变为绿色,再根据指示剂所标定的标准定度,其检测的最小浓度为0.15~0.3g/L。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "蛋白试纸法" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "四溴酚蓝" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "蛋白" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "蛋白" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "尿液" } ] }, { "text": "强碱性尿可致假阳性,大剂量青霉素时可出现假阴性。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "尿" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "青霉素" } ] }, { "text": "从定性可粗略估计尿蛋白。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "尿蛋白" } ] }, { "text": "表12-12示一般情况下用上述两种方法测定的结果彼此相符,且与尿蛋白浓度一致,但有时这两种方法可有不同的结果。", "entities": [ { "start_idx": 31, "end_idx": 33, "type": "ite", "entity": "尿蛋白" } ] }, { "text": "蛋白试纸法对白蛋白敏感,而磺基水杨酸则对所有蛋白均敏感,因而,当尿中存在低分子蛋白时,用试纸法可能为阴性。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "蛋白试纸法" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "白蛋白" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "磺基水杨酸" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "蛋白" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "尿" }, { "start_idx": 39, "end_idx": 40, "type": "ite", "entity": "蛋白" }, { "start_idx": 44, "end_idx": 46, "type": "pro", "entity": "试纸法" } ] }, { "text": "因此在多发性骨髓瘤患者尿排出大量免疫球蛋白轻链时,不用磺基水杨酸法,否则可能漏诊。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "多发性骨髓瘤" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "免疫球蛋白轻链" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "磺基水杨酸法" } ] }, { "text": "表12-1尿蛋白定性判断随尿蛋白浓度关系(3)醋酸加热法:灵敏度为0.15g/L,假阳性和假阴性较前两种方法要少,除本-周蛋白(Bence-Jones蛋白)外,所有蛋白均可在加热变性后沉淀。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 23, "end_idx": 27, "type": "pro", "entity": "醋酸加热法" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "本-周蛋白" }, { "start_idx": 64, "end_idx": 76, "type": "bod", "entity": "Bence-Jones蛋白" }, { "start_idx": 82, "end_idx": 83, "type": "ite", "entity": "蛋白" } ] }, { "text": "检测免疫球蛋白轻链(本-周蛋白)也可用醋酸加热法,当加热至45~55℃时蛋白沉淀,继续加热至100℃时又溶解。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "免疫球蛋白轻链" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "本-周蛋白" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "醋酸加热法" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "蛋白" } ] }, { "text": "如果用磺基水杨酸法得到的阳性结果较试纸法强提示为非白蛋白尿,两者得到相同的结果则尿蛋白主要为白蛋白。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "磺基水杨酸法" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "白蛋白尿" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "尿蛋白" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "白蛋白" } ] }, { "text": "2.尿蛋白定量测定尿蛋白定性常不能评估疾病的严重性,因为排出同等量的蛋白时可因尿量的不同得出不同的结果,因此需进行尿蛋白定量分析。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "尿蛋白定量测定" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 34, "end_idx": 35, "type": "ite", "entity": "蛋白" }, { "start_idx": 39, "end_idx": 40, "type": "ite", "entity": "尿量" }, { "start_idx": 57, "end_idx": 59, "type": "ite", "entity": "尿蛋白" } ] }, { "text": "通常采用24小时尿蛋白定量,来判断病情的轻重。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "尿蛋白" } ] }, { "text": "常用定量检查方法有双缩脲法、Esbach法、磺基水杨酸比浊法及各种染料比色法。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "双缩脲法" }, { "start_idx": 14, "end_idx": 20, "type": "pro", "entity": "Esbach法" }, { "start_idx": 22, "end_idx": 29, "type": "pro", "entity": "磺基水杨酸比浊法" }, { "start_idx": 31, "end_idx": 37, "type": "pro", "entity": "各种染料比色法" } ] }, { "text": "正常小儿尿蛋白定量:①24小时小于150mg;②<4mg/(h•m2);③尿蛋白/尿肌酐比值<0.2。", "entities": [ { "start_idx": 43, "end_idx": 49, "type": "ite", "entity": "尿蛋白/尿肌酐" } ] }, { "text": "肾病综合征时,尿蛋白定量:①>50mg/(kg•d);②>40mg/(h•m2);③尿蛋白/尿肌酐比值>2.0。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 48, "end_idx": 54, "type": "ite", "entity": "尿蛋白/尿肌酐" } ] }, { "text": "蛋白尿的轻重程度分为:轻度<500mg/24h、中度为500mg~2g/24h、重度为>2g/24h。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "蛋白尿" } ] }, { "text": "轻至中度蛋白尿见于直立性蛋白尿、急或慢性肾小球肾炎、泌尿系感染及某些药物中毒。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "直立性蛋白尿" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "泌尿系感染" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "药物中毒" } ] }, { "text": "重度蛋白尿主要见于肾病综合征。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "肾病综合征" } ] }, { "text": "3.尿蛋白成分分析尿蛋白按其来源可分为:①肾小球性蛋白尿:由于肾小球毛细管壁通透性增加或其静电屏障作用减弱所致。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "尿蛋白成分分析" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "尿蛋白" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "肾小球性蛋白尿" } ] }, { "text": "以白蛋白为主,既可含有少量β2-微球蛋白等小分子量蛋白,又可含有免疫球蛋白等大分子量蛋白。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "白蛋白" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "蛋白" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "免疫球蛋白" } ] }, { "text": "蛋白尿以白蛋白为主时称选择性蛋白尿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "白蛋白" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "蛋白尿" } ] }, { "text": "含有大量的高分子量蛋白如IgG、IgA和IgM时为非选择性蛋白尿。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "蛋白" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "IgG" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "IgM" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "蛋白尿" } ] }, { "text": "②肾小管性蛋白尿:由于近端小管重吸收功能减低所致。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "bod", "entity": "肾小管性蛋白尿" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "小管" } ] }, { "text": "以β2-微球蛋白、溶菌酶和视黄醇结合蛋白(RBP)等小分子蛋白升高为主。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "溶菌酶" }, { "start_idx": 19, "end_idx": 25, "type": "bod", "entity": "视黄醇结合蛋白" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "RBP" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "蛋白" } ] }, { "text": "测定尿β2-微球蛋白/白蛋白比值也有助于肾小管性蛋白尿的判断。", "entities": [ { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "肾小管性蛋白尿" } ] }, { "text": "正常人尿中β2-微球蛋白/白蛋白=1∶100,肾小管病变时为1∶7,肾小球病变时该比值为1∶750。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "尿" }, { "start_idx": 14, "end_idx": 21, "type": "ite", "entity": "微球蛋白/白蛋白" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "肾小管病变" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "肾小球病变" } ] }, { "text": "③混合性蛋白尿:既有肾小球性蛋白尿,又有肾小管性蛋白尿,称混合性蛋白尿。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "混合性蛋白尿" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "肾小球性蛋白尿" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "肾小管性蛋白尿" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "混合性蛋白尿" } ] }, { "text": "④溢出性蛋白尿:血中低分子量蛋白异常增高(如免疫球蛋白轻链和肌红蛋白),超过肾小管重吸收极限时,大量随尿排出,即为溢出性蛋白尿。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "溢出性蛋白尿" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "血" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "蛋白" }, { "start_idx": 22, "end_idx": 28, "type": "bod", "entity": "免疫球蛋白轻链" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "肌红蛋白" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "肾小管" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "尿" }, { "start_idx": 57, "end_idx": 62, "type": "bod", "entity": "溢出性蛋白尿" } ] }, { "text": "常见于多发性骨髓瘤、轻链病、原发性冷球蛋白血症以及肌红蛋白尿等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "多发性骨髓瘤" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "轻链病" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "原发性冷球蛋白血症" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "肌红蛋白尿" } ] }, { "text": "⑤组织性蛋白尿:由肾小管、输尿管、膀胱和尿道局部组织破坏或分泌产生的蛋白尿,见于泌尿道感染、损伤和肿瘤等。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "bod", "entity": "组织性蛋白尿" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肾小管" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "输尿管" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "膀胱" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "尿道" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "蛋白尿" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "泌尿道感染" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "损伤" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "肿瘤" } ] }, { "text": "对尿蛋白成分可采用:①十二烷基磺酸钠-聚丙烯酰胺凝胶电泳(sodiumdodecylsulfate-polyacrylamidegelelectrophoresis,SDS-PAGE):可采用盘状电泳或垂直板式电泳,板式电泳敏感度更高,两者都是按分子量大小来区分尿蛋白;②等电聚焦电泳:按蛋白质电荷量(PI值)来分离蛋白;③二维电泳:可区分更多蛋白,但操作复杂,要求较高;④反相高效液相色谱分析:分析速度快,灵敏度高,但仪器价格昂贵;⑤毛细管电泳:分析速度快,准确可靠,上样量小,但成本较高。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "尿蛋白" }, { "start_idx": 11, "end_idx": 27, "type": "pro", "entity": "十二烷基磺酸钠-聚丙烯酰胺凝胶电泳" }, { "start_idx": 29, "end_idx": 81, "type": "pro", "entity": "sodiumdodecylsulfate-polyacrylamidegelelectrophoresis" }, { "start_idx": 83, "end_idx": 90, "type": "pro", "entity": "SDS-PAGE" }, { "start_idx": 96, "end_idx": 99, "type": "pro", "entity": "盘状电泳" }, { "start_idx": 101, "end_idx": 106, "type": "pro", "entity": "垂直板式电泳" }, { "start_idx": 108, "end_idx": 111, "type": "pro", "entity": "板式电泳" }, { "start_idx": 136, "end_idx": 141, "type": "pro", "entity": "等电聚焦电泳" }, { "start_idx": 144, "end_idx": 149, "type": "ite", "entity": "蛋白质电荷量" }, { "start_idx": 151, "end_idx": 153, "type": "ite", "entity": "PI值" }, { "start_idx": 158, "end_idx": 159, "type": "bod", "entity": "蛋白" }, { "start_idx": 162, "end_idx": 165, "type": "pro", "entity": "二维电泳" }, { "start_idx": 172, "end_idx": 173, "type": "bod", "entity": "蛋白" }, { "start_idx": 187, "end_idx": 196, "type": "pro", "entity": "反相高效液相色谱分析" }, { "start_idx": 218, "end_idx": 222, "type": "pro", "entity": "毛细管电泳" } ] }, { "text": "尿蛋白可按分子量分为三类。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "尿蛋白" } ] }, { "text": "其一是高分子量蛋白,分子量>9万,主要为免疫球蛋白和Tamn-Horsfall蛋白;其二是中分子量蛋白,分子量在4万~9万间,主要为白蛋白;其三是低分子量蛋白,分子量<4万,主要由溶菌酶、β2-微球蛋白、α1-微球蛋白、免疫球蛋白Fc片段和自由轻链等组成。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "高分子量蛋白" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 26, "end_idx": 40, "type": "bod", "entity": "Tamn-Horsfall蛋白" }, { "start_idx": 45, "end_idx": 50, "type": "bod", "entity": "中分子量蛋白" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "白蛋白" }, { "start_idx": 73, "end_idx": 78, "type": "bod", "entity": "低分子量蛋白" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "溶菌酶" }, { "start_idx": 103, "end_idx": 106, "type": "bod", "entity": "微球蛋白" }, { "start_idx": 117, "end_idx": 120, "type": "bod", "entity": "微球蛋白" }, { "start_idx": 122, "end_idx": 130, "type": "bod", "entity": "免疫球蛋白Fc片段" }, { "start_idx": 132, "end_idx": 135, "type": "bod", "entity": "自由轻链" } ] }, { "text": "此外,也可采用蛋白尿选择性指数的测定来区别尿中排出大分子蛋白质的多少。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "ite", "entity": "蛋白尿选择性指数" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "尿" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "大分子蛋白质" } ] }, { "text": "选择性蛋白尿指数(selectiveproteinuriaindex,SpI)的计算如下:当尿中排出大分子IgG量少时为选择性好,排出大量IgG时为选择性差。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "选择性蛋白尿指数" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "尿" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "IgG" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "IgG" } ] }, { "text": "SpI>0.2为选择性差,见于膜性肾病和膜增殖性肾小球肾炎;SpI0.1~0.2为选择性一般;SpI<0.1为选择性好,见于微小病变性肾病。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "膜性肾病" }, { "start_idx": 20, "end_idx": 28, "type": "dis", "entity": "膜增殖性肾小球肾炎" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "微小病变性肾病" } ] }, { "text": "(二)糖近端肾小管可将滤过的葡萄糖完全回吸收,正常小儿尿糖极微,定量约0.56~5.0mmol/24h,浓度<2.8mmol/L,定性为阴性。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "糖" }, { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "近端肾小管" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "尿糖" } ] }, { "text": "儿童肾糖阈为8.88mmol/L(160mg/dl)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "肾糖" } ] }, { "text": "如血糖超过肾糖阈及肾糖阈降低时,均可出现尿糖。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "血糖" }, { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "肾糖" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "肾糖" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "尿糖" } ] }, { "text": "肾性糖尿为肾糖阈降低的肾小管疾病,此种疾病血糖及糖耐量正常而表现为尿糖阳性,可见于各种药物性肾损害、先天性肾性糖尿病、严重感染、胱氨酸尿症、Fanconi综合征、Wilson病以及部分糖原累积症Ⅰ型患儿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "肾性糖尿" }, { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "肾糖" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肾小管疾病" }, { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "血糖" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "糖耐量" }, { "start_idx": 33, "end_idx": 34, "type": "ite", "entity": "尿糖" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "药物性肾损害" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "先天性肾性糖尿病" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "严重感染" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "胱氨酸尿症" }, { "start_idx": 70, "end_idx": 79, "type": "dis", "entity": "Fanconi综合征" }, { "start_idx": 81, "end_idx": 87, "type": "dis", "entity": "Wilson病" }, { "start_idx": 92, "end_idx": 96, "type": "dis", "entity": "糖原累积症" } ] }, { "text": "20%的足月儿及未成熟儿在生后第1周可出现一过性糖尿,为肾单位发育不成熟所致。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "一过性糖尿" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "肾单位" } ] }, { "text": "(三)氨基酸血及尿中常见的氨基酸约有17种左右,大多经肾小球滤过后由近端肾小管上皮细胞主动回吸收,其回吸收率为92%(组氨酸)到99.8%(缬氨酸)不等。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "氨基酸" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "血" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "尿" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "氨基酸" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肾小球" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "组氨酸" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "缬氨酸" } ] }, { "text": "氨基酸尿可分为全氨基酸尿或一组氨基酸尿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "氨基酸尿" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "全氨基酸尿" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "一组氨基酸尿" } ] }, { "text": "前者为滤过的所有氨基酸回吸收率降低,见于Lowe综合征及Fanconi综合征。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "sym", "entity": "滤过的所有氨基酸回吸收率降低" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "Lowe综合征" }, { "start_idx": 28, "end_idx": 37, "type": "dis", "entity": "Fanconi综合征" } ] }, { "text": "一组氨基酸尿可见于胱氨酸尿症(cystinuria),主要为二碱基氨基酸(精氨酸、赖氨酸、鸟氨酸及胱氨酸)在尿中排出增加。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "一组氨基酸尿" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "胱氨酸尿症" }, { "start_idx": 15, "end_idx": 24, "type": "dis", "entity": "cystinuria" }, { "start_idx": 30, "end_idx": 59, "type": "sym", "entity": "二碱基氨基酸(精氨酸、赖氨酸、鸟氨酸及胱氨酸)在尿中排出增加" } ] }, { "text": "单氨基酸尿如苯丙酮尿症。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "单氨基酸尿" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "苯丙酮尿症" } ] }, { "text": "其他氨基酸尿尚可见继发于药物及重金属中毒、肾病和维生素D缺乏性佝偻病等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "氨基酸尿" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "重金属中毒" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "肾病" }, { "start_idx": 24, "end_idx": 33, "type": "dis", "entity": "维生素D缺乏性佝偻病" } ] }, { "text": "常用过筛或半定量法测定尿中游离氨基酸,更精确的方法是用氨基酸分析仪测定。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "过筛或半定量法" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "氨基酸" }, { "start_idx": 27, "end_idx": 34, "type": "pro", "entity": "氨基酸分析仪测定" } ] }, { "text": "(四)酮体包括丙酮、乙酰乙酸和β-羟丁酸,现通用化学试带法检查,其显色反应的原理是亚硝基铁氰化钠与酮体产生紫红色化合物。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "酮体" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "丙酮" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "乙酰乙酸" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "β-羟丁酸" }, { "start_idx": 24, "end_idx": 30, "type": "equ", "entity": "化学试带法检查" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "酮体" } ] }, { "text": "灵敏度高,缺点是不能检测β-羟丁酸。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "β-羟丁酸" } ] }, { "text": "正常小儿为阴性,糖尿病酮症酸中毒以及重症感染时可出现阳性。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "糖尿病酮症酸中毒" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "重症感染" } ] }, { "text": "(五)电解质电解质测定对了解某些疾病的病情变化有很大意义。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "电解质" }, { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "电解质测定" } ] }, { "text": "正常人尿钠的排泄通常反映饮食钠摄入情况,因此尿钠浓度波动范围较大。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "尿钠" } ] }, { "text": "在限制钠摄入情况下,血钠可降低或维持正常,当尿钠<10mmol/L,常见于严重腹泻脱水、心力衰竭及肾病水肿期。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "ite", "entity": "血钠" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "尿钠" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "腹泻脱水" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "肾病水肿" } ] }, { "text": "如尿钠>20mmol/L,则可能是抗利尿激素不适当分泌综合征(SIADH)、水中毒、慢性肾炎以及急性肾衰竭等;如尿钠>40mmol/L,则应考虑有无肾小管坏死。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "尿钠" }, { "start_idx": 17, "end_idx": 29, "type": "dis", "entity": "抗利尿激素不适当分泌综合征" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "SIADH" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "水中毒" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "慢性肾炎" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 56, "end_idx": 57, "type": "ite", "entity": "尿钠" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "肾小管坏死" } ] }, { "text": "肾脏保钾能力小于保钠能力,即使限制钾的摄入,尿钾仍可>10mmol/L。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肾脏" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "尿钾" } ] }, { "text": "尿钾增高见于应用排钾利尿剂和肾上腺皮质激素等药物,也见于原发性醛固酮增多症、库欣综合征、Batter综合征以及肾小管酸中毒等疾病。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "尿钾增高" }, { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "排钾利尿剂" }, { "start_idx": 14, "end_idx": 20, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 28, "end_idx": 36, "type": "dis", "entity": "原发性醛固酮增多症" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "库欣综合征" }, { "start_idx": 44, "end_idx": 52, "type": "dis", "entity": "Batter综合征" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "肾小管酸中毒" } ] }, { "text": "在正常饮食下(钙摄入量约500mg/d),尿钙应<4mg/(kg•d),尿Ca/Cr<0.21,(一般24小时尿钙<2mg/kg,随意尿Ca/Cr<0.12);尿钙>4mg/(kg•d),见于特发性高钙尿症和肾小管酸中毒等疾病。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "尿钙" }, { "start_idx": 36, "end_idx": 41, "type": "ite", "entity": "尿Ca/Cr" }, { "start_idx": 55, "end_idx": 56, "type": "ite", "entity": "尿钙" }, { "start_idx": 67, "end_idx": 72, "type": "ite", "entity": "尿Ca/Cr" }, { "start_idx": 80, "end_idx": 81, "type": "ite", "entity": "尿钙" }, { "start_idx": 96, "end_idx": 102, "type": "dis", "entity": "特发性高钙尿症" }, { "start_idx": 104, "end_idx": 109, "type": "dis", "entity": "肾小管酸中毒" } ] }, { "text": "尿磷的排泄与肾小管功能以及血浆中甲状旁腺激素(PTH)水平密切相关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "尿磷" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肾小管" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "血浆" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "甲状旁腺激素" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "PTH" } ] }, { "text": "尿磷增多见于Fanconi综合征、原发或者继发性甲状旁腺功能亢进、药物或重金属肾损害、应用大量皮质激素、利尿剂以及痛风、烧伤和各种佝偻病等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "尿磷增多" }, { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "Fanconi综合征" }, { "start_idx": 17, "end_idx": 31, "type": "dis", "entity": "原发或者继发性甲状旁腺功能亢进" }, { "start_idx": 33, "end_idx": 41, "type": "dis", "entity": "药物或重金属肾损害" }, { "start_idx": 45, "end_idx": 50, "type": "dru", "entity": "大量皮质激素" }, { "start_idx": 52, "end_idx": 54, "type": "dru", "entity": "利尿剂" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "痛风" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "烧伤" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "佝偻病" } ] }, { "text": "正常尿磷:婴儿<6.4mmol/d,儿童<0.5~0.6mmol/(kg•d)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "尿磷" } ] }, { "text": "尿铜增高见于Wilson病。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "尿铜" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "Wilson病" } ] }, { "text": "第四节营养性巨幼红细胞性贫血营养性巨幼红细胞性贫血(nutritionalmegaloblasticanemia)又称大细胞性贫血,主要由叶酸或(和)维生素B12直接或间接缺乏所致,大多因摄入不足而导致直接缺乏引起。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "营养性巨幼红细胞性贫血" }, { "start_idx": 14, "end_idx": 24, "type": "dis", "entity": "营养性巨幼红细胞性贫血" }, { "start_idx": 26, "end_idx": 55, "type": "dis", "entity": "nutritionalmegaloblasticanemia" }, { "start_idx": 59, "end_idx": 64, "type": "dis", "entity": "大细胞性贫血" }, { "start_idx": 69, "end_idx": 70, "type": "dru", "entity": "叶酸" }, { "start_idx": 75, "end_idx": 80, "type": "dru", "entity": "维生素B12" } ] }, { "text": "其血细胞形态学特点是红细胞体积较大,中性粒细胞核分叶过多,骨髓中巨幼红细胞增生。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "血细胞" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "红细胞" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "中性粒细胞核" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "骨髓" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "巨幼红细胞" } ] }, { "text": "【叶酸与维生素B12的代谢】(一)叶酸的代谢和利用食物中的叶酸(蝶酰谷氨酸)摄入后,与位于小肠黏膜上皮细胞上的叶酸结合蛋白结合而被吸收。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "叶酸" }, { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "维生素B12" }, { "start_idx": 23, "end_idx": 24, "type": "dru", "entity": "叶酸" }, { "start_idx": 35, "end_idx": 36, "type": "dru", "entity": "叶酸" }, { "start_idx": 38, "end_idx": 42, "type": "dru", "entity": "蝶酰谷氨酸" }, { "start_idx": 51, "end_idx": 58, "type": "bod", "entity": "小肠黏膜上皮细胞" }, { "start_idx": 61, "end_idx": 66, "type": "bod", "entity": "叶酸结合蛋白" } ] }, { "text": "蝶酰单谷氨酸较蝶酰多谷氨酸容易吸收。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "蝶酰单谷氨酸" }, { "start_idx": 7, "end_idx": 12, "type": "dru", "entity": "蝶酰多谷氨酸" } ] }, { "text": "而位于刷状缘的蝶酰谷氨酸羟化酶具有促进蝶酰多谷氨酸向蝶酰单谷氨酸转化,从而有利于叶酸的吸收。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "刷状缘" }, { "start_idx": 7, "end_idx": 14, "type": "bod", "entity": "蝶酰谷氨酸羟化酶" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "蝶酰多谷氨酸" }, { "start_idx": 26, "end_idx": 31, "type": "bod", "entity": "蝶酰单谷氨酸" }, { "start_idx": 40, "end_idx": 41, "type": "dru", "entity": "叶酸" } ] }, { "text": "叶酸具有肠肝循环。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "叶酸" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肠肝" } ] }, { "text": "血浆中大多数叶酸与血清清蛋白结合,叶酸本身并无生物学活性,必须在二氢叶酸还原酶的作用下还原成四氢叶酸,而后被转运入组织细胞内。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血浆" }, { "start_idx": 6, "end_idx": 7, "type": "dru", "entity": "叶酸" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "血清清蛋白" }, { "start_idx": 17, "end_idx": 18, "type": "dru", "entity": "叶酸" }, { "start_idx": 32, "end_idx": 38, "type": "bod", "entity": "二氢叶酸还原酶" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "四氢叶酸" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "组织细胞" } ] }, { "text": "正常成人的叶酸需要量为每日100μg,怀孕期母亲可增至每日350μg,以千克体重计算,婴幼儿的叶酸需要量比成人为高。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dru", "entity": "叶酸" }, { "start_idx": 47, "end_idx": 48, "type": "dru", "entity": "叶酸" } ] }, { "text": "(二)维生素B12的代谢和利用维生素B12来源于动物性食物中的钴胺素(cobalamin),人类不能合成维生素B12,但肠道细菌却能合成并能供人体使用。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "维生素B12" }, { "start_idx": 21, "end_idx": 26, "type": "dru", "entity": "维生素B12" }, { "start_idx": 43, "end_idx": 45, "type": "dru", "entity": "钴胺素" }, { "start_idx": 47, "end_idx": 55, "type": "dru", "entity": "cobalamin" }, { "start_idx": 64, "end_idx": 69, "type": "dru", "entity": "维生素B12" }, { "start_idx": 78, "end_idx": 81, "type": "mic", "entity": "肠道细菌" } ] }, { "text": "食物中的钴胺素在胃酸的作用下释放出来,并立即与R蛋白及内因子结合后通过十二指肠,其中的R蛋白被胰腺蛋白酶水解,残余的维生素B12内因子复合物在回肠末端借助于一种位于回肠末端黏膜上皮细胞上的钴胺素内因子复合物的特殊受体而吸收。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "钴胺素" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胃酸" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "R蛋白" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "十二指肠" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "R蛋白" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "胰腺蛋白酶" }, { "start_idx": 58, "end_idx": 63, "type": "dru", "entity": "维生素B12" }, { "start_idx": 77, "end_idx": 80, "type": "bod", "entity": "回肠末端" }, { "start_idx": 88, "end_idx": 97, "type": "bod", "entity": "回肠末端黏膜上皮细胞" }, { "start_idx": 100, "end_idx": 102, "type": "bod", "entity": "钴胺素" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "受体" } ] }, { "text": "此外,当给予大剂量维生素B12时,也可在口腔和小肠黏膜通过梯度弥散机制而吸收。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "维生素B12" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "口腔" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "小肠黏膜" } ] }, { "text": "血浆中的维生素B12与转钴胺素蛋白(transcobalamin,TC)Ⅰ、Ⅱ、Ⅲ结合,其中TCⅡ尤为重要。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血浆" }, { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "维生素B12" }, { "start_idx": 17, "end_idx": 46, "type": "bod", "entity": "转钴胺素蛋白(transcobalamin,TC)Ⅰ、Ⅱ、Ⅲ" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "TCⅡ" } ] }, { "text": "TCⅡ钴胺素通过特殊的受体介导内吞作用进入细胞内,然后钴胺素被转化成甲基钴胺素和腺苷钴胺素,后两者为活性形式,参与转甲基作用和合成DNA。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "TCⅡ钴胺素" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "受体" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "细胞" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "钴胺素" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "甲基钴胺素" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "腺苷钴胺素" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "转甲基" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "DNA" } ] }, { "text": "【叶酸、维生素B12缺乏的原因】(一)喂养不当叶酸主要存在于绿叶蔬菜中,其他如酵母、肝、肾等食物中也较多。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dru", "entity": "叶酸" }, { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "维生素B12" }, { "start_idx": 29, "end_idx": 30, "type": "dru", "entity": "叶酸" }, { "start_idx": 45, "end_idx": 46, "type": "mic", "entity": "酵母" } ] }, { "text": "而维生素B12则主要位于动物肝、肌肉和肾中。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dru", "entity": "维生素B12" } ] }, { "text": "当单纯母乳喂养而未及时添加辅食、人工喂养不当及严重偏食的小儿,其饮食中缺乏肉类、动物肝、肾及蔬菜,常常可以引起维生素B12和叶酸的缺乏。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "母乳" }, { "start_idx": 55, "end_idx": 60, "type": "dru", "entity": "维生素B12" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "叶酸" } ] }, { "text": "羊奶中所含叶酸甚微,因此,单纯以羊奶喂养者,容易出现叶酸的缺乏。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dru", "entity": "叶酸" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "叶酸" } ] }, { "text": "(三)疾病因素叶酸的主要吸收部位是小肠上段,而维生素B12的主要吸收部位则位于回肠末端。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "叶酸" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "小肠上段" }, { "start_idx": 23, "end_idx": 28, "type": "dru", "entity": "维生素B12" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "回肠末端" } ] }, { "text": "因此,任何原因导致小肠病变均可使叶酸和维生素B12的吸收障碍,从而导致两者的缺乏,如慢性腹泻可严重影响叶酸、维生素B12的吸收。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "小肠" }, { "start_idx": 16, "end_idx": 17, "type": "dru", "entity": "叶酸" }, { "start_idx": 19, "end_idx": 24, "type": "dru", "entity": "维生素B12" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "慢性腹泻" }, { "start_idx": 57, "end_idx": 58, "type": "dru", "entity": "叶酸" }, { "start_idx": 60, "end_idx": 65, "type": "dru", "entity": "维生素B12" } ] }, { "text": "空肠外科切除可引起叶酸缺乏,而回肠切除则可引起维生素B12的缺乏。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "空肠" }, { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "外科切除" }, { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "叶酸" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "回肠" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "切除" }, { "start_idx": 23, "end_idx": 28, "type": "dru", "entity": "维生素B12" } ] }, { "text": "此外,肝脏病变可影响叶酸的正常代谢,使叶酸的生物转化发生障碍而致病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "肝脏病变" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "叶酸" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "叶酸" } ] }, { "text": "(四)先天性缺陷1.小肠先天性叶酸吸收缺陷是一种常染色体隐性遗传性疾病,可导致叶酸的吸收障碍。", "entities": [ { "start_idx": 10, "end_idx": 20, "type": "dis", "entity": "小肠先天性叶酸吸收缺陷" }, { "start_idx": 24, "end_idx": 34, "type": "dis", "entity": "常染色体隐性遗传性疾病" }, { "start_idx": 39, "end_idx": 40, "type": "dru", "entity": "叶酸" } ] }, { "text": "2.幼年型恶性贫血该病罕见,属于常染色体隐性遗传。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "幼年型恶性贫血" } ] }, { "text": "病因为胃壁细胞不能分泌内因子(IF)而使维生素B12吸收障碍所致。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "胃壁细胞" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "内因子" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "IF" }, { "start_idx": 20, "end_idx": 25, "type": "dru", "entity": "维生素B12" } ] }, { "text": "3.先天性转钴胺蛋白缺陷转钴胺蛋白Ⅱ(TCⅡ)是维生素B12的主要转运蛋白,先天性转钴胺蛋白缺陷可以导致维生素B12转运障碍,从而出现维生素B12的间接缺陷。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "先天性转钴胺蛋白缺陷" }, { "start_idx": 12, "end_idx": 22, "type": "bod", "entity": "转钴胺蛋白Ⅱ(TCⅡ)" }, { "start_idx": 24, "end_idx": 29, "type": "dru", "entity": "维生素B12" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "转运蛋白" }, { "start_idx": 44, "end_idx": 53, "type": "dis", "entity": "先天性转钴胺蛋白缺陷" }, { "start_idx": 58, "end_idx": 63, "type": "dru", "entity": "维生素B12" }, { "start_idx": 79, "end_idx": 84, "type": "dru", "entity": "维生素B12" } ] }, { "text": "该病属于常染色体隐性遗传。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "常染色体" } ] }, { "text": "【发病机制】叶酸和维生素B12是脱氧核糖核酸(DNA)合成过程中重要的辅酶,主要起转甲基作用。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dru", "entity": "叶酸" }, { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "维生素B12" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "脱氧核糖核酸" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "DNA" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "辅酶" } ] }, { "text": "缺乏时,尿嘧啶脱氧核苷酸不能甲基化,从而阻碍了合成DNA的重要原料胸腺嘧啶脱氧核苷酸的合成,进而影响DNA的合成。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "bod", "entity": "尿嘧啶脱氧核苷酸" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "DNA" }, { "start_idx": 33, "end_idx": 41, "type": "bod", "entity": "胸腺嘧啶脱氧核苷酸" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "DNA" } ] }, { "text": "在正常情况下,当细胞内DNA增加到两倍(4n)时才发生细胞分裂。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "细胞" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "DNA" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "细胞" } ] }, { "text": "叶酸和维生素B12缺乏时,DNA合成发生障碍,幼红细胞分裂延迟,而血红蛋白则仍然在继续合成,这样,幼红细胞血红蛋白合成越来越多,体积越来越大,而DNA含量却始终未能达到细胞分裂所必需的要求,使幼红细胞发生巨幼变。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "叶酸" }, { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "维生素B12" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "DNA" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "幼红细胞" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "幼红细胞" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "DNA" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "细胞" }, { "start_idx": 102, "end_idx": 105, "type": "bod", "entity": "幼红细胞" } ] }, { "text": "这种巨幼红细胞很容易在骨髓内破坏,造成无效造血。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "巨幼红细胞" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "骨髓" } ] }, { "text": "叶酸和维生素B12缺乏对细胞分裂的影响不仅见于红系,也见于粒系和巨核细胞系。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "叶酸" }, { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "维生素B12" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "细胞" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "红系" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "粒系" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "巨核细胞系" } ] }, { "text": "晚幼粒和杆状核粒细胞体积大、核肿胀、结构疏松,核分叶过多,可多至5叶以上。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "晚幼粒" }, { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "杆状核粒细胞" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "体积大" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "核肿胀" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "结构疏松" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "核分叶过多" } ] }, { "text": "由于粒细胞生存期短,故这种变化先于红系,可作为叶酸或维生素B12缺乏早期诊断的依据。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "粒细胞" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "红系" }, { "start_idx": 23, "end_idx": 24, "type": "dru", "entity": "叶酸" }, { "start_idx": 26, "end_idx": 31, "type": "dru", "entity": "维生素B12" } ] }, { "text": "巨核细胞的体积也增大,核分叶过多,血小板生成障碍,可见巨大血小板。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "巨核细胞" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "体积也增大" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "核分叶过多" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "血小板生成障碍" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "可见巨大血小板" } ] }, { "text": "维生素B12缺乏时,除可出现血液系统改变外,尚可影响神经精神系统。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "维生素B12" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "血液系统" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "神经精神系统" } ] }, { "text": "这是由于维生素B12缺乏可引起神经系统有鞘神经纤维脂质代谢障碍。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "维生素B12" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "神经系统" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "鞘神经纤维脂质" }, { "start_idx": 21, "end_idx": 36, "type": "sym", "entity": "神经系统有鞘神经纤维脂质代谢障碍" } ] }, { "text": "有鞘神经纤维的鞘中含有丰富的鞘磷脂,脂质代谢过程中的中间代谢产物为甲基丙二酸,后者在维生素B12的参与下,转变成琥珀酸进而进入三羧酸循环。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "鞘神经纤维" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "鞘磷脂" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "脂质" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "甲基丙二酸" }, { "start_idx": 42, "end_idx": 47, "type": "dru", "entity": "维生素B12" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "琥珀酸" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "三羧酸" } ] }, { "text": "维生素B12缺乏时,甲基丙二酸不能转变成琥珀酸而使甲基丙二酸在神经鞘中堆积,破坏了神经鞘的形成,从而出现神经精神症状。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "维生素B12" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "甲基丙二酸" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "琥珀酸" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "甲基丙二酸" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "神经鞘" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "神经鞘" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "神经精神" } ] }, { "text": "全身症状轻重和贫血不一定成正比。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "dis", "entity": "贫" }, { "start_idx": 7, "end_idx": 7, "type": "pro", "entity": "贫" } ] }, { "text": "常有舌面光滑、厌食、恶心、呕吐、腹泻,偶有吞咽困难、声音嘶哑。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "舌面" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "舌面光滑" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "厌食" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "恶心" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "呕吐" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "腹泻" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "吞咽困难" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "声音嘶哑" } ] }, { "text": "由于髓外造血的关系,肝、脾可出现不同程度的肿大,与发病年龄有关,年龄越小,肝、脾大就越明显。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "髓" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肝" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "脾" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "肿大" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "肝" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "脾" } ] }, { "text": "白细胞减少者易患细菌性感染。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "白细胞" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "细菌性感染" } ] }, { "text": "维生素B12缺乏时,除也有上述表现外,尚可出现明显的神经精神症状。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "维生素B12" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "神经精神" } ] }, { "text": "【实验室检查】(一)外周血象红细胞数较血红蛋白量降低得更明显。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "外周血象" }, { "start_idx": 14, "end_idx": 17, "type": "ite", "entity": "红细胞数" }, { "start_idx": 19, "end_idx": 23, "type": "ite", "entity": "血红蛋白量" } ] }, { "text": "早期血红蛋白尚在正常范围时,红细胞数就可已经明显减少。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "红细胞" } ] }, { "text": "红细胞体积增大,可有轻度大小不等,以大细胞为主。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "红细胞" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "大细胞" } ] }, { "text": "红细胞内血红蛋白充盈度良好,中央淡染区缩小。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "红细胞" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "血红蛋白" } ] }, { "text": "平均红细胞体积(MCV)及平均红细胞血红蛋白含量(MCH)均大于正常,但平均红细胞血红蛋白浓度(MCHC)则在正常范围,说明此种贫血为单纯大细胞性贫血。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "红细胞" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "MCV" }, { "start_idx": 13, "end_idx": 23, "type": "ite", "entity": "平均红细胞血红蛋白含量" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "MCH" }, { "start_idx": 36, "end_idx": 46, "type": "ite", "entity": "平均红细胞血红蛋白浓度" }, { "start_idx": 48, "end_idx": 51, "type": "ite", "entity": "MCHC" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "贫血" }, { "start_idx": 67, "end_idx": 74, "type": "dis", "entity": "单纯大细胞性贫血" } ] }, { "text": "粒细胞体积增大、数量减少、核染色质疏松,核分叶较多,多者可达5叶以上,如核分叶5叶以上的细胞超过5%则有诊断价值。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "粒细胞" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "粒细胞体积增大" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "数量减少" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "核染色质" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "核染色质疏松" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "核分叶" }, { "start_idx": 20, "end_idx": 33, "type": "sym", "entity": "核分叶较多,多者可达5叶以上" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "核分叶" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "细胞" } ] }, { "text": "血小板数可减少、体积增大,出血时间延长。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "血小板数" }, { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "血小板数可减少、体积增大,出血时间延长" } ] }, { "text": "(二)骨髓象骨髓细胞大多代偿性增生旺盛,也有增生正常或增生低下者,但均有红细胞巨幼变,胞体大、核染色质松、胞质嗜酸性强,核、浆发育不平衡,胞核的发育落后于胞质。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "骨髓细胞" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "红细胞" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "红细胞巨幼变" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "胞体" }, { "start_idx": 43, "end_idx": 45, "type": "sym", "entity": "胞体大" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "核染色质" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "核染色质松" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "胞质" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "胞质嗜酸性强" }, { "start_idx": 60, "end_idx": 60, "type": "bod", "entity": "核" }, { "start_idx": 62, "end_idx": 62, "type": "bod", "entity": "浆" }, { "start_idx": 60, "end_idx": 67, "type": "sym", "entity": "核、浆发育不平衡" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "胞核" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "胞质" }, { "start_idx": 69, "end_idx": 78, "type": "sym", "entity": "胞核的发育落后于胞质" } ] }, { "text": "粒细胞体大、核分叶多、核右移,巨核细胞核分叶过多、胞质中颗粒减少,骨髓中血小板也较大。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "粒细胞体" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "粒细胞体大" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "核分叶" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "核分叶多" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "核" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "核右移" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "巨核细胞核分叶" }, { "start_idx": 15, "end_idx": 23, "type": "sym", "entity": "巨核细胞核分叶过多" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "胞质" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "胞质中颗粒减少" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "骨髓中血小板" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "骨髓中血小板也较大" } ] }, { "text": "(三)血浆叶酸及维生素B12定量测定叶酸或维生素B12减少或两者皆减少。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血浆" }, { "start_idx": 5, "end_idx": 6, "type": "dru", "entity": "叶酸" }, { "start_idx": 8, "end_idx": 13, "type": "dru", "entity": "维生素B12" }, { "start_idx": 24, "end_idx": 25, "type": "dru", "entity": "叶酸" }, { "start_idx": 27, "end_idx": 32, "type": "dru", "entity": "维生素B12" } ] }, { "text": "当血浆叶酸含量<3μg/L(67nmol/L)和(或)血浆维生素B12含量<100ng/L时有助于确诊。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "血浆" }, { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "叶酸" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血浆" }, { "start_idx": 29, "end_idx": 34, "type": "dru", "entity": "维生素B12" } ] }, { "text": "(四)胃酸测定患儿胃酸常降低,经治疗后可恢复。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "胃酸" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胃酸" } ] }, { "text": "为鉴别维生素B12缺乏抑或内因子缺乏,可采用51Cr标记的维生素B12进行Schilling试验。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "维生素B12" }, { "start_idx": 41, "end_idx": 46, "type": "dru", "entity": "维生素B12" }, { "start_idx": 55, "end_idx": 65, "type": "pro", "entity": "Schilling试验" } ] }, { "text": "巨幼红细胞性贫血应与红白血病、先天性脑发育不全症鉴别,红白血病时恶性巨幼红细胞中糖原染色(PAS)呈现巨大PAS阳性颗粒,而巨幼红细胞性贫血时却无此种表现;先天性脑发育不全的智力障碍发生于出生时,而巨幼红细胞性贫血者的智力下降是继发的,出生时智力正常,不难鉴别。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "巨幼红细胞性贫血" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "红白血病" }, { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "先天性脑发育不全症" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "红白血病" }, { "start_idx": 32, "end_idx": 38, "type": "bod", "entity": "恶性巨幼红细胞" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "糖原染色" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "PAS" }, { "start_idx": 51, "end_idx": 59, "type": "sym", "entity": "巨大PAS阳性颗粒" }, { "start_idx": 62, "end_idx": 69, "type": "dis", "entity": "巨幼红细胞性贫血" }, { "start_idx": 78, "end_idx": 85, "type": "dis", "entity": "先天性脑发育不全" }, { "start_idx": 87, "end_idx": 90, "type": "sym", "entity": "智力障碍" }, { "start_idx": 99, "end_idx": 107, "type": "dis", "entity": "巨幼红细胞性贫血者" }, { "start_idx": 109, "end_idx": 112, "type": "sym", "entity": "智力下降" } ] }, { "text": "对于不能根治的先天性缺陷,只能采用补充或替代疗法。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "先天性缺陷" } ] }, { "text": "叶酸不能改善维生素B12缺乏引起的神经症状,故在无明显神经症状的巨幼红细胞性贫血可用叶酸进行治疗。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "叶酸" }, { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "维生素B12" }, { "start_idx": 38, "end_idx": 45, "type": "dis", "entity": "巨幼红细胞性贫血" }, { "start_idx": 48, "end_idx": 49, "type": "dru", "entity": "叶酸" } ] }, { "text": "每日口服叶酸5~15mg,维生素C300mg;后者可加强前者的疗效。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dru", "entity": "叶酸" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "维生素C" } ] }, { "text": "营养因素引起的维生素B12缺乏者,可给予维生素B12每3日肌肉注射0.1mg,共2~3周。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dru", "entity": "维生素B12" }, { "start_idx": 26, "end_idx": 31, "type": "dru", "entity": "维生素B12" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "肌肉注射" } ] }, { "text": "其他原因引起或病情严重者可每月1次,每次1mg,待血象正常后,减量维持。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "血象" } ] }, { "text": "为改善神经系统症状,可适当加用维生素B6。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经系统" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "维生素B6" } ] }, { "text": "严重贫血已引起心功能不全者,应小量多次输血,以减少慢性缺氧。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "严重贫血" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "心功能不全" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "输血" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "慢性缺氧" } ] }, { "text": "输血时点滴速度要缓慢。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "输血" } ] }, { "text": "如有原发病应积极治疗。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "原发病" } ] }, { "text": "第三章极低出生体重儿的特点及护理极低出生体重儿(verylowbirthweightinfant,VLBW)是指出生体重小于1500g的新生儿,其中绝大部分为早产儿,胎龄小于32周。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "极低出生体重儿" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "极低出生体重儿" }, { "start_idx": 25, "end_idx": 48, "type": "dis", "entity": "verylowbirthweightinfant" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "VLBW" }, { "start_idx": 59, "end_idx": 60, "type": "ite", "entity": "体重" }, { "start_idx": 57, "end_idx": 67, "type": "sym", "entity": "出生体重小于1500g" } ] }, { "text": "VLBW的死亡数占新生儿死亡数的很大一部分,而且,即使存活也容易出现神经系统发育的障碍。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "神经系统" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "神经系统发育的障碍" } ] }, { "text": "美国1981—1997年VLBW的发生率为1.1%~1.4%,国内20世纪80年代的资料与之相近。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "VLBW" } ] }, { "text": "一、生理特点(一)呼吸系统VLBW呼吸中枢的发育、呼吸器官的发育和相关因子的产生均不完善,很容易发生呼吸暂停和低氧血症的发生。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "VLBW" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "呼吸中枢" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "呼吸器官" }, { "start_idx": 17, "end_idx": 43, "type": "sym", "entity": "呼吸中枢的发育、呼吸器官的发育和相关因子的产生均不完善" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "低氧血症" } ] }, { "text": "VLBW的胸壁薄、呼吸肌发育差、小支气管的软骨少,肺泡发育未完成,导致其功能残气量低,肺顺应性差,通气/血流比严重失调;同时,由于其气道的管径小,造成气道阻力高、有效通气量减少。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "胸壁" }, { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "胸壁薄" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "呼吸肌发育差" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "小支气管" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "软骨" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "小支气管的软骨少" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肺泡" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "肺泡发育未完成" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "功能残气量低" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "肺" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "肺顺应性差" }, { "start_idx": 49, "end_idx": 54, "type": "ite", "entity": "通气/血流比" }, { "start_idx": 49, "end_idx": 58, "type": "sym", "entity": "通气/血流比严重失调" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "气道" }, { "start_idx": 66, "end_idx": 71, "type": "sym", "entity": "气道的管径小" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "气道" }, { "start_idx": 75, "end_idx": 87, "type": "sym", "entity": "气道阻力高、有效通气量减少" } ] }, { "text": "对于胎龄小于32周的VLBW,其肺泡Ⅱ型上皮细胞及其功能的发育尚未完善,缺乏产生肺表面活性物质的能力或数量极少,导致新生儿呼吸窘迫综合征的发生率增高。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "VLBW" }, { "start_idx": 16, "end_idx": 23, "type": "bod", "entity": "肺泡Ⅱ型上皮细胞" }, { "start_idx": 16, "end_idx": 34, "type": "sym", "entity": "肺泡Ⅱ型上皮细胞及其功能的发育尚未完善" }, { "start_idx": 40, "end_idx": 46, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 36, "end_idx": 54, "type": "sym", "entity": "缺乏产生肺表面活性物质的能力或数量极少" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "呼吸窘迫综合征" } ] }, { "text": "(二)循环系统胎龄小的VLBW的心肌纤维胶原含量低而且缺乏张力,心肌处于一种低反应状态,表现为收缩期的收缩力和舒张期张力均较低,对于维持心功能不利。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "循环系统" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "VLBW" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "心肌纤维胶原" }, { "start_idx": 16, "end_idx": 30, "type": "sym", "entity": "心肌纤维胶原含量低而且缺乏张力" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "心肌" }, { "start_idx": 32, "end_idx": 42, "type": "sym", "entity": "心肌处于一种低反应状态" }, { "start_idx": 47, "end_idx": 62, "type": "sym", "entity": "收缩期的收缩力和舒张期张力均较低" }, { "start_idx": 68, "end_idx": 70, "type": "ite", "entity": "心功能" } ] }, { "text": "VLBW发育尚未达到适应外界生活的需要,因而出生后容易发生潜水反射,即全身血流重新分布,以保证重要脏器心、脑、肾上腺的血液供应。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "心" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "脑" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "肾上腺" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "血液" } ] }, { "text": "神经系统对心血管的调节为副交感神经占优势,在对VLBW进行口、鼻腔操作时容易引起迷走神经反射亢进,发生缓脉等情况。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "神经系统" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "心血管" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "副交感神经" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "VLBW" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "口" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "鼻腔" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "迷走神经" }, { "start_idx": 40, "end_idx": 47, "type": "sym", "entity": "迷走神经反射亢进" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "缓脉" } ] }, { "text": "对血压自身调节能力的发育尚未完善,如躯体血压的变动直接可影响脑血压的变化。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "血压" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "血压" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "脑血压" } ] }, { "text": "另外,对于部分重度宫内发育迟缓的VLBW,生后即可发生心功能不全,并在生后2~3天开始恶化,发病后1~2天达高峰,如能度过这一高峰期,则存活率增高。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "重度宫内发育迟缓" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "VLBW" }, { "start_idx": 27, "end_idx": 29, "type": "ite", "entity": "心功能" } ] }, { "text": "在部分死亡病例的尸解中发现心肌纤维与相应胎龄比较明显地更细。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "心肌纤维" } ] }, { "text": "(三)中枢神经系统因发育不完善致反射和协调功能差;四肢肌张力明显低下。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "反射和协调功能差" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "四肢" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "四肢肌张力明显低下" } ] }, { "text": "脑血管结构从软脑膜开始,沿很长的小动脉向侧脑室方向行进,终止于深部白质的脑室脚间动脉,与从侧脑室开始向深部白质行进的较短脑室动脉形成终末供血区。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑血管" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "软脑膜" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "小动脉" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "侧脑室" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "深部白质" }, { "start_idx": 36, "end_idx": 41, "type": "bod", "entity": "脑室脚间动脉" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "侧脑室" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "深部白质" }, { "start_idx": 58, "end_idx": 63, "type": "bod", "entity": "较短脑室动脉" } ] }, { "text": "这一部位容易受低血压或低血流所伴的缺血性变化的影响,造成脑室周围白质软化症的发生。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "低血压" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "低血流" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "脑室" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "白质软化症" } ] }, { "text": "(四)泌尿系统VLBW的肾功能差,肾小球滤过率较低;肾脏浓缩功能亦差,肾小管对葡萄糖的重吸收阈值低,给予高浓度葡萄糖补液可以造成高血糖和渗透性利尿。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "VLBW" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "肾功能" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "肾功能差" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肾小球" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "肾小球滤过率较低" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肾脏" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "肾脏浓缩功能亦差" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "肾小管" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 35, "end_idx": 48, "type": "sym", "entity": "肾小管对葡萄糖的重吸收阈值低" }, { "start_idx": 52, "end_idx": 59, "type": "dru", "entity": "高浓度葡萄糖补液" }, { "start_idx": 64, "end_idx": 66, "type": "dis", "entity": "高血糖" }, { "start_idx": 68, "end_idx": 72, "type": "dis", "entity": "渗透性利尿" } ] }, { "text": "新生儿少尿的定义为<1ml/(kg•h),但是对于VLBW在尿量<2ml/(kg•h)时也要考虑少尿的存在。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "少尿" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "VLBW" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "尿量" }, { "start_idx": 30, "end_idx": 42, "type": "sym", "entity": "尿量<2ml/(kg•h)" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "少尿" } ] }, { "text": "(五)消化系统小肠的自律性活动从胎龄6~7个月已经开始,但尚无输送能力,肠道输送能力实际是从胎龄34周开始的。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "消化系统" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "小肠" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肠道" } ] }, { "text": "VLBW的消化器官未能正常活动是因为:肠道神经丛处于未成熟阶段,加上低血糖及其他刺激,使交感神经的兴奋性增高;潜水反射使进入肠道的血流减少,导致肠道活动能力低下;母亲的某些用药,如控制妊高征的硫酸镁可以使乙酰胆碱释放减少,并使神经-肌紧张反射功能低下。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "消化器官" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "肠道神经丛" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "低血糖" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "交感神经" }, { "start_idx": 44, "end_idx": 53, "type": "sym", "entity": "交感神经的兴奋性增高" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "肠道" }, { "start_idx": 72, "end_idx": 79, "type": "sym", "entity": "肠道活动能力低下" }, { "start_idx": 92, "end_idx": 94, "type": "dis", "entity": "妊高征" }, { "start_idx": 96, "end_idx": 98, "type": "dru", "entity": "硫酸镁" }, { "start_idx": 102, "end_idx": 105, "type": "dru", "entity": "乙酰胆碱" }, { "start_idx": 113, "end_idx": 116, "type": "bod", "entity": "神经-肌" }, { "start_idx": 113, "end_idx": 124, "type": "sym", "entity": "神经-肌紧张反射功能低下" } ] }, { "text": "无论是潜水反射造成的缺氧,或是肠道喂养后发生的缺氧(开始肠道喂养后,肠道本身对氧的需求增加,而肠道血流量未明显增加,造成一种肠道组织的低氧状态),都是发生NEC的危险因素。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "缺氧" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肠道" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "缺氧" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肠道" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肠道" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "肠道" }, { "start_idx": 47, "end_idx": 56, "type": "sym", "entity": "肠道血流量未明显增加" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "肠道组织" }, { "start_idx": 77, "end_idx": 79, "type": "dis", "entity": "NEC" } ] }, { "text": "感染对发生NEC的影响与肠道的局部防御能力有关。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "NEC" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肠道" } ] }, { "text": "VLBW的胆汁酸分泌量很少,其胆汁酸池也很小,容易造成胆汁排出困难,形成胆汁淤积症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "胆汁酸" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "胆汁酸池" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "胆汁" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "胆汁排出困难" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "胆汁淤积症" } ] }, { "text": "VLBW在出生前体内已经有蛋白分解酶的活性存在,尽管其浓度与成人比还是处于明显的低水平;胎龄24周已经具备对氨基酸等的转运能力。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 13, "end_idx": 17, "type": "mic", "entity": "蛋白分解酶" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "氨基酸" } ] }, { "text": "胎龄25周时唾液腺已经开始分泌能消化脂肪的脂酶,同时人乳中存在的胆盐刺激脂酶(bilesaltstimulatinglipase,BSSL)能促使脂肪酸分解和消化脂肪。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "唾液腺" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "脂肪" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "脂酶" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "人乳" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "胆盐" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "脂酶" }, { "start_idx": 39, "end_idx": 63, "type": "bod", "entity": "bilesaltstimulatinglipase" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "BSSL" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "脂肪酸" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "脂肪" } ] }, { "text": "小肠黏膜绒毛膜刷状缘有活性双糖分解酶在胎龄10~12周已经出现,在26~34周时几乎达到成人的水平;乳糖酶、β-葡萄糖苷酶的活性在胎龄24周才开始增加;但缺乏消化多糖体所需要的淀粉酶,母乳中抗胃酸的淀粉酶直接到达小肠,可以帮助消化多糖体。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "小肠黏膜绒毛膜刷状缘" }, { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "活性双糖分解酶" }, { "start_idx": 50, "end_idx": 52, "type": "mic", "entity": "乳糖酶" }, { "start_idx": 54, "end_idx": 60, "type": "mic", "entity": "β-葡萄糖苷酶" }, { "start_idx": 88, "end_idx": 90, "type": "mic", "entity": "淀粉酶" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "母乳" }, { "start_idx": 96, "end_idx": 97, "type": "bod", "entity": "胃酸" }, { "start_idx": 99, "end_idx": 101, "type": "mic", "entity": "淀粉酶" }, { "start_idx": 106, "end_idx": 107, "type": "bod", "entity": "小肠" } ] }, { "text": "(六)水、电解质和酸碱平衡VLBW的体表面积相对较大,不显性失水量较大,体重<1000g新生儿的不显性失水几乎是1500g新生儿的2倍。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "VLBW" }, { "start_idx": 18, "end_idx": 25, "type": "sym", "entity": "体表面积相对较大" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "不显性失水量较大" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "体重" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "体重<1000g" } ] }, { "text": "出生后3天内,由于不显性失水的增加以及排尿和使用含钠盐溶液,可以造成高钠血症的出现。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "钠盐溶液" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "高钠血症" } ] }, { "text": "而对于出生3天后的VLBW,由于其肾小管重吸收功能差,尿中排钠量高,母乳中钠含量随出生后天数的增加而相应地持续减少,如加上利尿剂的使用,可以造成低钠血症。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "VLBW" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "肾小管" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "肾小管重吸收功能差" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "尿" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "尿中排钠量高" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "母乳" }, { "start_idx": 61, "end_idx": 63, "type": "dru", "entity": "利尿剂" }, { "start_idx": 72, "end_idx": 75, "type": "dis", "entity": "低钠血症" } ] }, { "text": "酸中毒往往发生在生后初期,主要与缺氧有关,通过改善氧供和呼吸循环的功能可以及时纠正;生后2周以后发生的晚发性代谢性酸中毒,可能与蛋白质负荷高、尿酸化能力低(肾小管分泌H+功能差)和排出阈值低有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "酸中毒" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "缺氧" }, { "start_idx": 51, "end_idx": 59, "type": "dis", "entity": "晚发性代谢性酸中毒" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "蛋白质" }, { "start_idx": 64, "end_idx": 69, "type": "sym", "entity": "蛋白质负荷高" }, { "start_idx": 71, "end_idx": 71, "type": "bod", "entity": "尿" }, { "start_idx": 71, "end_idx": 76, "type": "sym", "entity": "尿酸化能力低" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "肾小管" }, { "start_idx": 78, "end_idx": 98, "type": "sym", "entity": "肾小管分泌H+功能差" }, { "start_idx": 101, "end_idx": 105, "type": "sym", "entity": "排出阈值低" } ] }, { "text": "代谢性碱中毒的发生,可能与排钠钾性利尿剂的持续使用有关,造成肾小管对钠钾的重吸收障碍,同时利尿剂抑制了对氯离子的重吸收,则发生低氯性代谢性碱中毒。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "代谢性碱中毒" }, { "start_idx": 13, "end_idx": 19, "type": "dru", "entity": "排钠钾性利尿剂" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "肾小管" }, { "start_idx": 30, "end_idx": 41, "type": "sym", "entity": "肾小管对钠钾的重吸收障碍" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "利尿剂" }, { "start_idx": 63, "end_idx": 71, "type": "dis", "entity": "低氯性代谢性碱中毒" } ] }, { "text": "低血钙的发生可能无临床症状,血钙最低可<3mmol/L,一般在7天后可自行纠正。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "低血钙" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "血钙" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "血钙最低可<3mmol/L" } ] }, { "text": "(七)代谢VLBW的基础代谢率比足月新生儿低,但是其糖原、脂肪的储存量很少,能量的摄取能力极差。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "VLBW" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "糖原" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "脂肪" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "糖原、脂肪的储存量很少" }, { "start_idx": 38, "end_idx": 46, "type": "sym", "entity": "能量的摄取能力极差" } ] }, { "text": "同时,高血糖会造成肝细胞对胰岛素的反应低下。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "高血糖" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肝细胞" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "胰岛素" } ] }, { "text": "VLBW发生低血糖情况较少见。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "低血糖" } ] }, { "text": "VLBW本身血清蛋白含量低,同时能量供应不足的情况下,蛋白质被用于能量供应,造成负氮平衡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "血清蛋白" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "蛋白质" } ] }, { "text": "VLBW的卡泥汀合成能力低,对脂肪酸的分解不利。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "卡泥汀" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "脂肪酸" } ] }, { "text": "对静脉给予的脂肪利用度小,容易造成一过性高脂血症。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "静脉" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脂肪" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "一过性高脂血症" } ] }, { "text": "脂肪吸收率低,容易影响脂溶性维生素的吸收。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脂肪" }, { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "脂肪吸收率低" } ] }, { "text": "胎龄小于36周的新生儿难以从肠道吸收锌,容易存在锌缺乏。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肠道" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "锌缺乏" } ] }, { "text": "VLBW的体温调节能力低,通常需要37~38℃的环境温度(包括暖箱温度)方能维持其肛门温度为37℃,否则容易出现低体温。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "体温调节能力低" }, { "start_idx": 31, "end_idx": 32, "type": "equ", "entity": "暖箱" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "肛门" } ] }, { "text": "同时,由于汗腺功能发育不完善,环境温度过高,容易产生发热。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "汗腺" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "发热" } ] }, { "text": "(八)其他感染是造成VLBW死亡的重要原因,主要与免疫功能发育不完善有关,包括从母体获得的特异性免疫球蛋白量少于足月新生儿。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "感染" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "VLBW" }, { "start_idx": 45, "end_idx": 52, "type": "bod", "entity": "特异性免疫球蛋白" } ] }, { "text": "VLBW在黄疸不高的情况下,由于低蛋白血症和血脑屏障的发育不完善,也容易发生核黄疸,有人认为体重在1000g的新生儿,一旦其总胆红素为10mg/dl就应换血,以避免核黄疸的发生。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 5, "end_idx": 5, "type": "dis", "entity": "黄" }, { "start_idx": 5, "end_idx": 5, "type": "pro", "entity": "黄" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "下,由于低" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "白血症和" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "容易发" }, { "start_idx": 42, "end_idx": 43, "type": "ite", "entity": "有人" }, { "start_idx": 58, "end_idx": 61, "type": "ite", "entity": ",一旦其" }, { "start_idx": 58, "end_idx": 69, "type": "sym", "entity": ",一旦其总胆红素为10m" }, { "start_idx": 72, "end_idx": 73, "type": "pro", "entity": "dl" }, { "start_idx": 78, "end_idx": 80, "type": "dis", "entity": ",以避" } ] }, { "text": "第六章上呼吸道疾病第一节先天性喉喘鸣先天性喉喘鸣(congenitallaryngealstridor),常发生于出生后不久,主要症状为吸气时发生喘鸣。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "上呼吸道疾病" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "先天性喉喘鸣" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "先天性喉喘鸣" }, { "start_idx": 26, "end_idx": 51, "type": "dis", "entity": "congenitallaryngealstridor" }, { "start_idx": 69, "end_idx": 75, "type": "sym", "entity": "吸气时发生喘鸣" } ] }, { "text": "喉软化和气管软化是最主要的原因。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "喉软化" }, { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "气管软化" } ] }, { "text": "由于吸气时负压较大,使会厌边缘与杓状软骨接近,喉部成活瓣状关闭,从而发生喉喘鸣。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "会厌" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "杓状软骨" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "喉部" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "喉喘鸣" } ] }, { "text": "35%喉软化患儿伴有其他病变,如气管软化、声门下狭窄、声带麻痹等。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "喉软化" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "气管软化" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "声门下狭窄" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "声带麻痹" } ] }, { "text": "重者喘鸣为持续性,并出现三凹征。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "喘鸣" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "三凹征" } ] }, { "text": "由于影响哺乳及睡眠,常有不同程度的营养不良。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "营养不良" } ] }, { "text": "同时,由于呼吸困难及长期缺氧,可见明显的漏斗胸或鸡胸。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "长期缺氧" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "漏斗胸" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "鸡胸" } ] }, { "text": "明显喉喘鸣持续数个月至1年,随喉软骨逐渐发育,多数患儿在18个月内消失。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "喉喘鸣" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "喉软骨" } ] }, { "text": "少数可于呼吸道感染、运动或哭吵时出现喘鸣症状。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "喘鸣" } ] }, { "text": "根据出生后不久即出现喉喘鸣,无其他疾病的病史、体征,一般不难做出诊断。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "喉喘鸣" } ] }, { "text": "注意防治呼吸道感染。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "呼吸道感染" } ] }, { "text": "一般不需气管切开。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "气管" } ] }, { "text": "如并发其他解剖畸形,应进行相应处理。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "解剖畸形" } ] }, { "text": "第三节病毒性肺炎一、呼吸道合胞病毒性肺炎呼吸道合胞病毒(RSV)是婴儿下呼吸道感染的主要病原,尤其易发生于2~4月龄的小婴儿。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "病毒性肺炎" }, { "start_idx": 10, "end_idx": 19, "type": "dis", "entity": "呼吸道合胞病毒性肺炎" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "呼吸道合胞病毒" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "RSV" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "下呼吸道" } ] }, { "text": "据浙江大学医学院附属儿童医院观察,冬春季节RSV感染占3岁以下婴幼儿肺炎的35%左右。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "RSV感染" }, { "start_idx": 27, "end_idx": 35, "type": "dis", "entity": "3岁以下婴幼儿肺炎" } ] }, { "text": "RSV毛细支气管炎的发病机制尚不明确,但有证据表明,免疫损伤可能参与了其发病过程。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "RSV毛细支气管炎" } ] }, { "text": "初期上呼吸道感染症状突出,如鼻塞、流涕,继而咳嗽、低热、喘鸣。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "鼻塞" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "流涕" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "咳嗽" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "低热" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "喘鸣" } ] }, { "text": "随病情进展,出现呼吸困难、鼻扇、呼气延长、呼吸时呻吟和三凹征等。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "鼻扇" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "呼气延长" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "呼吸时呻吟" }, { "start_idx": 27, "end_idx": 29, "type": "sym", "entity": "三凹征" } ] }, { "text": "易并发急性心力衰竭。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "急性心力衰竭" } ] }, { "text": "初期听诊呼吸音减弱、哮鸣音为主,而后可闻细湿啰音。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "哮鸣音" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "细湿啰音" } ] }, { "text": "X线检查见肺纹理增粗或点片状阴影,部分见肺不张或以肺气肿为主要表现。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "X线" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "肺不张" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "肺气肿" } ] }, { "text": "鼻咽部脱落细胞病毒免疫荧光或免疫酶检查,均可在数小时内获得结果。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "mic", "entity": "鼻咽部脱落细胞病毒" }, { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "免疫荧光" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "免疫酶" } ] }, { "text": "急性期可有RSV特异IgM升高。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "RSV" } ] }, { "text": "年龄小、喘憋出现早是本病的特点,但确诊要靠血清学和病毒学检查。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "血清学" }, { "start_idx": 25, "end_idx": 29, "type": "ite", "entity": "病毒学检查" } ] }, { "text": "六、胶囊内镜全称为“智能胶囊消化道内镜系统”,又称“医用无线内镜”。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "equ", "entity": "胶囊内镜" }, { "start_idx": 10, "end_idx": 20, "type": "equ", "entity": "智能胶囊消化道内镜系统" }, { "start_idx": 26, "end_idx": 31, "type": "equ", "entity": "医用无线内镜" } ] }, { "text": "原理是受检者通过口服内置摄像与信号传输装置的智能胶囊,借助消化道蠕动使之在消化道内运动并拍摄图像,医生利用体外的图像记录仪和影像工作站,了解受检者的整个消化道情况,从而对其病情做出诊断。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "消化道" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "消化道" }, { "start_idx": 56, "end_idx": 60, "type": "equ", "entity": "图像记录仪" }, { "start_idx": 62, "end_idx": 66, "type": "equ", "entity": "影像工作站" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "消化道" } ] }, { "text": "胶囊内镜具有检查方便、无创伤、无导线、无痛苦、无交叉感染以及不影响患者的正常工作等优点,扩展了消化道检查的视野,克服了传统的插入式内镜所具有的耐受性差、不适用于年老体弱和病情危重等缺陷,可作为消化道疾病尤其是小肠疾病诊断的首选方法。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "equ", "entity": "胶囊内镜" }, { "start_idx": 62, "end_idx": 66, "type": "equ", "entity": "插入式内镜" }, { "start_idx": 96, "end_idx": 100, "type": "dis", "entity": "消化道疾病" }, { "start_idx": 104, "end_idx": 107, "type": "dis", "entity": "小肠疾病" } ] }, { "text": "二、免疫调节治疗通过增强和调节机体免疫功能,达到清除体内病毒的目的。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "免疫调节治疗" }, { "start_idx": 28, "end_idx": 29, "type": "mic", "entity": "病毒" } ] }, { "text": "如胸腺素α1,能促进T细胞分化和成熟、使IL-2和IL-2受体表达增加、增加Th细胞功能等,使机体能有效发挥免疫防护功能。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "胸腺素α1" }, { "start_idx": 20, "end_idx": 30, "type": "bod", "entity": "IL-2和IL-2受体" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "Th细胞" } ] }, { "text": "已证实,其单用或联合应用治疗慢性丙型肝炎效佳;并可用于重型丙型和乙型肝炎的治疗。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "慢性丙型肝炎" }, { "start_idx": 27, "end_idx": 35, "type": "dis", "entity": "重型丙型和乙型肝炎" } ] }, { "text": "再如,聚肌胞(人工合成的双链RNA)是高效干扰素诱生剂,具广谱抗病毒作用。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "聚肌胞" }, { "start_idx": 7, "end_idx": 16, "type": "dru", "entity": "人工合成的双链RNA" }, { "start_idx": 21, "end_idx": 26, "type": "dru", "entity": "干扰素诱生剂" }, { "start_idx": 32, "end_idx": 33, "type": "mic", "entity": "病毒" } ] }, { "text": "下述治疗性疫苗实际上都是通过调节和提高特异性免疫功能而发挥其抗病毒效应。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "治疗性疫苗" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "病毒" } ] }, { "text": "参考文献1.韩玉昆,等.儿科实用诊断治疗学.合肥:安徽科技出版社,19992.杨锡强,等.儿童免疫学.北京:人民卫生出版社,20013.杨锡强.原发性免疫缺陷病.中国实用儿科杂志,2000,15(8):5044.OchsHD,SmithCIE,PuckJM.PrimaryImmunodeficiencyDiseases:AMolecularandApproach.OxfordUniversityPress.NewYork,Oxford,19995.RosenFS,WedgwoodRJP,EiblM.Primaryimmunodeficiencydisease:ReportofaWHOScientificGroup,ClinExpImmunol,1997,109(Supl):1-16.StiehmER.ImmunologicDisorderinInfantsandChildren.3rded,W.B.SaundersCo,19967.GrezM,BeckerS,SaulnierS,etal.Genetherapyofchronicgranulomatousdisease.BoneMarrowTransplant,2000,25:S99", "entities": [ { "start_idx": 72, "end_idx": 79, "type": "dis", "entity": "原发性免疫缺陷病" } ] }, { "text": "参考文献1.罗学荣,苏林雁,万国斌,等.品行障碍儿童家庭环境的logistic回归分析.中国心理卫生杂志,1994,(8):2612.沈晓明.儿童铅中毒.北京:人民卫生出版社,1996;53-533.陶国泰.儿童少年精神医学.南京:江苏科学技术出版社,1999:438-4424.叶广俊.现代儿童少年卫生学.北京:人民卫生出版社,1999:332-3325.AmericanAcademyofPediatrics.Committeeonpsychosocialaspectsofchildandfamilyhealth:thepediatrician’sroleinhelpingchildrenandfamiliesdealwithseparationanddivorce.Pediatrics,1994,(1):1196.BischofGP,StithSM,WhitneyML.Familyenvironmentsofadolescent,sexoffendersandotherjuveniledelinquents.Adolescence,1995,(117):1577.BrentDA.Depressionandsuicideinchildrenandadolescents.PediatrRev,1993,(10):3808.DuRrantRH,GettsA,CadenheadC,etal.Exposuretoviolenceandvictimizationanddepression,hopelessnessandpurposeinlifeamongadolescentslivinginandaroundpublichousing.JDevelBehavPediatr,1995,(4):2339.HofmannAD,GreydanusDE.Adolescentmedicine.3rded.Appleton&Lange:Stanford,1997:835-83510.HindelangRL,DwyerWO.Adolescentrisk-takingbehavior:Areviewoftheroleofparentalinvolvement.CurrentproblemsinPediatrics,2001,(3):6311.SmetanaJG.Parentingstylesandconceptionsofparentalauthorityduringadolescence.ChildDevel,1995,(2):29912.WenderEH.Attention-deficithyperactivitydisordersinadolescence.JDevelBehavPediatr,1995,(3):192", "entities": [ { "start_idx": 71, "end_idx": 75, "type": "dis", "entity": "儿童铅中毒" } ] }, { "text": "第六节主动脉狭窄有几种类型的先天性心血管畸形可阻挡血液自左室喷射出。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "主动脉狭窄" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "先天性心血管畸形" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血液" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "左室" } ] }, { "text": "最常见为主动脉瓣本身的异常,其他的原因可包括:二尖瓣或乳头肌的位置异常、室间隔肌性肥厚、主动脉瓣下纤维环、主动脉瓣下薄膜样结构仅留一小孔、主动脉瓣上狭窄。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "乳头肌" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "室间隔肌性肥厚" }, { "start_idx": 44, "end_idx": 51, "type": "dis", "entity": "主动脉瓣下纤维环" }, { "start_idx": 53, "end_idx": 62, "type": "bod", "entity": "主动脉瓣下薄膜样结构" }, { "start_idx": 69, "end_idx": 75, "type": "dis", "entity": "主动脉瓣上狭窄" } ] }, { "text": "由于主动脉瓣狭窄(aorticstenosis)是最常见的主动脉狭窄类型,本节将作一详述。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "主动脉瓣狭窄" }, { "start_idx": 9, "end_idx": 22, "type": "dis", "entity": "aorticstenosis" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "主动脉狭窄" } ] }, { "text": "一、主动脉瓣狭窄大约85%的先天性主动脉瓣狭窄为二瓣畸形,这二个主动脉瓣一大一小,其间有一偏心的鱼嘴样开口。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "主动脉瓣狭窄" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "先天性主动脉瓣狭窄" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "二瓣畸形" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "主动脉瓣" } ] }, { "text": "梗阻部分是由于瓣叶交界融合所形成的小孔,部分是由于瓣叶增厚、活动缺乏。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "梗阻" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "瓣叶" } ] }, { "text": "如在宫内时狭窄已严重,可发生左室血液转移,因而可出现左心室及升主动脉发育不良。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "左室血液" }, { "start_idx": 26, "end_idx": 37, "type": "dis", "entity": "左心室及升主动脉发育不良" } ] }, { "text": "约20%伴其他畸形,如主动脉瓣关闭不全、水肿、升主动脉发育不良、动脉导管未闭,以及二尖瓣关闭不全及狭窄。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "主动脉瓣关闭不全" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "水肿" }, { "start_idx": 23, "end_idx": 30, "type": "dis", "entity": "升主动脉发育不良" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 41, "end_idx": 50, "type": "dis", "entity": "二尖瓣关闭不全及狭窄" } ] }, { "text": "【病理生理】如左心室收缩压高、左心室肥厚、且主动脉舒张压低、舒张期短,心内膜下血流可不足,心内膜下缺血因此发生。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "左心室" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "左心室收缩压高" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "左心室" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "左心室肥厚" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "主动脉" }, { "start_idx": 22, "end_idx": 33, "type": "sym", "entity": "主动脉舒张压低、舒张期短" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "心内膜" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "心内膜" } ] }, { "text": "这解释了为什么严重主动脉瓣狭窄时会发生心内膜下坏死及纤维化。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "主动脉瓣狭窄" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "心内膜" }, { "start_idx": 19, "end_idx": 28, "type": "sym", "entity": "心内膜下坏死及纤维化" } ] }, { "text": "轻型患者只有杂音而无左室肥厚。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "杂音" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "左室" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "左室肥厚" } ] }, { "text": "反之,如卵圆孔未闭,心房水平存在明显的左向右分流,左心房压升高不多,此时肺水肿可不明显,但心输出量降低。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "卵圆孔未闭" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心房" }, { "start_idx": 10, "end_idx": 23, "type": "sym", "entity": "心房水平存在明显的左向右分流" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "左心房" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "左心房压升高不多" }, { "start_idx": 36, "end_idx": 38, "type": "sym", "entity": "肺水肿" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "心" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "心输出量降低" } ] }, { "text": "在上述任一情况,左心室均可显著扩大,并发生左心功能不全。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "左心室" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "左心功能不全" } ] }, { "text": "在较轻型病例左心室肥厚足以克服梗阻,因而心输出量可维持。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "左心室肥厚" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "梗阻" } ] }, { "text": "先天性主动脉瓣狭窄的自然病程往往有日趋恶化的趋势。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "先天性主动脉瓣狭窄" } ] }, { "text": "当患儿生长时,心排量必需增长,但狭窄瓣口并不能相应扩大,所以左心室与主动脉的压差愈益扩大,尤其在患儿生长较快的年龄阶段狭窄程度可较快变化。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "心" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "瓣口" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "左心室" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "主动脉" } ] }, { "text": "【临床表现】症状集中在幼婴和少儿两个年龄阶段;严重的狭窄在新生儿期即有表现,呼吸急速,心率增快,面色苍白,肺有水泡音,肝可肿大,患儿这些心力衰竭的表现可急剧恶化而夭折。", "entities": [ { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "呼吸急速" }, { "start_idx": 43, "end_idx": 44, "type": "ite", "entity": "心率" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "心率增快" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "面色苍白" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "肺" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "肺有水泡音" }, { "start_idx": 59, "end_idx": 59, "type": "bod", "entity": "肝" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "肝可肿大" }, { "start_idx": 68, "end_idx": 71, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "左心房的压力增高即可撑开未闭的卵圆孔向右心房分流,所以右心室可有容量的超负荷。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "左心房" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "右心房" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "右心室" } ] }, { "text": "X线示心脏增大,肺有充血及淤血;心电图示右心室占优势,患婴有典型的左心室肥厚者少。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "X线" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "心脏增大" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肺" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "肺有充血及淤血" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "心电图" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "右心室" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "右心室占优势" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "左心室肥厚" } ] }, { "text": "杂音往往为诊断本病的线索,少数患儿诉易疲劳、腹痛或胸痛;典型的心绞痛在小儿很少见。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "杂音" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "疲劳" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "腹痛" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "胸痛" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "心绞痛" } ] }, { "text": "如有劳力型气急和昏厥,往往为严重狭窄的表现;但患儿因对症状无知,家长亦可并不介意,所以必须详细询问病史。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "劳力型气急" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "昏厥" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "严重狭窄" } ] }, { "text": "脉搏往往正常。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "脉搏" } ] }, { "text": "典型的脉压缩小,脉跳的起落缓渐需至成年方明显。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "脉压" } ] }, { "text": "心尖搏动持重有力;常可触到震颤,多位于胸骨右缘第二肋间,亦可在胸骨左缘下部或心尖部最清楚,且可向胸骨柄上窝及颈动脉传导。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "可触到震颤" }, { "start_idx": 19, "end_idx": 26, "type": "bod", "entity": "胸骨右缘第二肋间" }, { "start_idx": 31, "end_idx": 36, "type": "bod", "entity": "胸骨左缘下部" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "心尖部" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "胸骨柄上窝" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "颈动脉" } ] }, { "text": "如无震颤,心尖搏动亦属正常,则狭窄往往很轻,压差常不超过30mmHg。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心尖" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "狭窄" } ] }, { "text": "第一心音正常,胸骨左缘可听到收缩早期喀喇音,系由于瓣膜突然绷紧所致。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "胸骨左缘" }, { "start_idx": 0, "end_idx": 20, "type": "sym", "entity": "第一心音正常,胸骨左缘可听到收缩早期喀喇音" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "瓣膜" } ] }, { "text": "洪亮而粗糙的收缩期杂音为本病最重要体征,在胸骨右缘上部最响,并向胸骨柄上窝,背面及颈部传导,也可在左缘最响,甚至在心尖部也可很响;杂音自第一音后开始,以后渐强,其高峰视狭窄的程度而异,狭窄愈重,高峰出现愈迟,至收缩晚期又渐弱或听不到,至主动脉瓣关闭前完全消失。", "entities": [ { "start_idx": 21, "end_idx": 26, "type": "bod", "entity": "胸骨右缘上部" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "背面" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "心尖部" }, { "start_idx": 84, "end_idx": 85, "type": "dis", "entity": "狭窄" }, { "start_idx": 92, "end_idx": 93, "type": "dis", "entity": "狭窄" }, { "start_idx": 118, "end_idx": 121, "type": "bod", "entity": "主动脉瓣" } ] }, { "text": "响度为4~5/6级,有时可通过骨传导在右肘部亦能听到,使凭听诊量血压完全失真。", "entities": [ { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "骨" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "右肘部" }, { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "血压" } ] }, { "text": "在胸骨左缘中部有时可听到2/6~3/6级渐弱的吹风样舒张期杂音,为主动脉瓣关闭不全所致。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "胸骨左缘" }, { "start_idx": 33, "end_idx": 40, "type": "dis", "entity": "主动脉瓣关闭不全" } ] }, { "text": "由于左心室收缩射血费时较长,所以第二心音中主动脉瓣关闭音推迟,与肺动脉瓣关闭音接近,使正常所有的第二音分裂时距缩短;狭窄重者左心室射血时间更形延长,使主动脉瓣与肺动脉瓣关闭音相重叠;如极度狭窄,主动脉瓣关闭音甚至落后于肺动脉瓣关闭音,而且呼吸的影响与正常相反,吸气时分裂较短,而呼气分裂扩大,形成所谓“矛盾分裂”。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "左心室" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "狭窄" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "左心室" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 80, "end_idx": 83, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 97, "end_idx": 100, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 109, "end_idx": 112, "type": "bod", "entity": "肺动脉瓣" } ] }, { "text": "心尖部常可听到第三心音,严重狭窄甚至可听到左房使劲收缩充盈左室的第四心音。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "左房" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "左室" }, { "start_idx": 12, "end_idx": 35, "type": "sym", "entity": "严重狭窄甚至可听到左房使劲收缩充盈左室的第四心音" } ] }, { "text": "【实验室检查】心电图示左心室肥大,但其程度不一定能反映狭窄的轻重。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "心电图" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "左心室" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "左心室肥大" } ] }, { "text": "严重者右心亦有增大,肺野充血。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "右心" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "严重者右心亦有增大,肺野充血" } ] }, { "text": "Doppler超声可估算压差和反流量。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "Doppler超声" } ] }, { "text": "由于超声检查的完善使心导管检查渐少用,逆行主动脉插管可通过狭窄的主动脉瓣口进入左心室,左心室压和由左心室拉回主动脉时压力连续曲线可显示狭窄的部位和左心室与主动脉之间的压差峰值。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "超声检查" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "心导管检查" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "主动脉插管" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "主动脉瓣口" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "左心室" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "左心室" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "左心室" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "主动脉" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "左心室" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "主动脉" } ] }, { "text": "左心室造影可显示左心室流出道的梗阻解剖,室壁厚度和二尖瓣有否反流。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "左心室造影" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "左心室流出道" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "室壁" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "二尖瓣" } ] }, { "text": "婴儿期主动脉逆行插管不易成功,可由右心导管穿过卵圆孔至左心房,再顺流入左心室,可测得左心室压力和行左心室造影。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "主动脉逆行插管" }, { "start_idx": 17, "end_idx": 20, "type": "equ", "entity": "右心导管" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "左心房" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "左心室" }, { "start_idx": 42, "end_idx": 46, "type": "ite", "entity": "左心室压力" }, { "start_idx": 49, "end_idx": 53, "type": "pro", "entity": "左心室造影" } ] }, { "text": "【治疗】当有心肌缺血或主动脉瓣口很小时应予手术。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "心肌缺血" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "主动脉瓣口" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "手术" } ] }, { "text": "如瓣口面积少于0.65cm2/M2而心排量正常,则左心室与主动脉的收缩期峰值压差已达到或超过70mmHg,这在大多数中心是行瓣膜切开术的指征。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "瓣口" }, { "start_idx": 40, "end_idx": 40, "type": "dis", "entity": "心" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "左心室" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "主动脉" }, { "start_idx": 84, "end_idx": 88, "type": "pro", "entity": "瓣膜切开术" } ] }, { "text": "瓣膜切开术只是姑息性的,术中切开瓣膜连合处以减少梗阻,但瓣膜连合处切开不能过大,因为这样的话可引起主动脉瓣明显反流。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "瓣膜切开术" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "瓣膜" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "梗阻" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "瓣膜" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "主动脉瓣" } ] }, { "text": "这种姑息术可挽救生命,并维持良好功能状态数年,但术后再狭窄的发生率很高,常与钙化有关。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "姑息术" } ] }, { "text": "最终,大多数严重主动脉瓣狭窄的患儿可能需要换瓣。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "dis", "entity": "严重主动脉瓣狭窄" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "换瓣" } ] }, { "text": "换瓣如可能应尽量推迟至成人,这样可避免因成长问题而需再次换瓣。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "换瓣" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "换瓣" } ] }, { "text": "在某些患儿可施行Ross手术,术后由于主动脉位置是自己的肺动脉瓣,因而无需抗凝,瓣膜的寿命也会延长。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "Ross手术" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "主动脉" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "瓣膜" } ] }, { "text": "1984年,Lababidi等首先报道应用经皮球囊主动脉瓣成形术(PBAV)成功地治疗先天性或后天性主动脉瓣狭窄,引起广泛的重视。", "entities": [ { "start_idx": 21, "end_idx": 31, "type": "pro", "entity": "经皮球囊主动脉瓣成形术" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "PBAV" }, { "start_idx": 43, "end_idx": 55, "type": "dis", "entity": "先天性或后天性主动脉瓣狭窄" } ] }, { "text": "由于我国发病率较欧美国家为少,另外PBAV在技术上有别于PBPV,且有严重并发症发生,因此国内除上海新华医院一组报告外,尚无系统开展该技术的报道。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "PBAV" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "PBPV" } ] }, { "text": "根据1982~1986年204例小儿经皮球囊主动脉瓣形成术总结,95.1%获得即期成功。", "entities": [ { "start_idx": 18, "end_idx": 28, "type": "pro", "entity": "经皮球囊主动脉瓣形成术" } ] }, { "text": "随后对PBAV方法学上进行较多的研究,包括单球囊与双球囊扩张术及采用长的球囊进行扩张术,以后把球囊瓣膜成形术应用到新生儿病例,甚至进行胎儿主动脉瓣球囊成形术。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "PBAV" }, { "start_idx": 21, "end_idx": 30, "type": "pro", "entity": "单球囊与双球囊扩张术" }, { "start_idx": 34, "end_idx": 42, "type": "pro", "entity": "长的球囊进行扩张术" }, { "start_idx": 47, "end_idx": 53, "type": "pro", "entity": "球囊瓣膜成形术" }, { "start_idx": 67, "end_idx": 77, "type": "pro", "entity": "胎儿主动脉瓣球囊成形术" } ] }, { "text": "目前,正在对PBAV病人进行中、长期随访,以观察其疗效与心功能的改变。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "PBAV" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "心" } ] }, { "text": "第四节鼻窦炎儿童鼻窦炎(sinusitis)较为常见,但不易识别。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "儿童鼻窦炎" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "sinusitis" } ] }, { "text": "急性鼻窦炎常继发于急性鼻炎,以肺炎链球菌、卡他莫拉菌和不定型流感嗜血杆菌引起者居多。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "急性鼻窦炎" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "急性鼻炎" }, { "start_idx": 15, "end_idx": 19, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 21, "end_idx": 25, "type": "mic", "entity": "卡他莫拉菌" }, { "start_idx": 27, "end_idx": 35, "type": "mic", "entity": "不定型流感嗜血杆菌" } ] }, { "text": "慢性鼻窦炎大多由于急性鼻窦炎反复发作所致,营养不良、免疫功能低下、过敏性体质、腺样体肥大等亦为常见致病因素。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "慢性鼻窦炎" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "急性鼻窦炎" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "腺样体" } ] }, { "text": "常见病原包括A组溶血性链球菌、金黄色葡萄球菌和厌氧菌,且常为混合感染。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "mic", "entity": "A组溶血性链球菌" }, { "start_idx": 15, "end_idx": 21, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 23, "end_idx": 25, "type": "mic", "entity": "厌氧菌" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "混合感染" } ] }, { "text": "儿童鼻窦炎常缺乏头痛、面部疼痛、水肿、触痛。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "儿童鼻窦炎" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "面部" }, { "start_idx": 6, "end_idx": 20, "type": "sym", "entity": "缺乏头痛、面部疼痛、水肿、触痛" } ] }, { "text": "咳嗽于平卧时加剧。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "咳嗽于平卧时加剧" } ] }, { "text": "分泌物引流不畅可致持久发热、头痛,相应的鼻窦部位压痛,鼻黏膜充血、水肿,中、下鼻道有黏稠脓液。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "持久发热" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "头" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "头痛" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "鼻窦" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "相应的鼻窦部位压痛" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "鼻黏膜" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "鼻黏膜充血、水肿" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "中、下鼻道" }, { "start_idx": 36, "end_idx": 45, "type": "sym", "entity": "中、下鼻道有黏稠脓液" } ] }, { "text": "可合并中耳炎、眼眶蜂窝织炎、眼眶脓肿等。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "中耳炎" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "眼眶蜂窝织炎" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "眼眶脓肿" } ] }, { "text": "慢性鼻窦炎主要症状为鼻塞、流脓涕及张口呼吸。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "慢性鼻窦炎" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "鼻塞" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "流脓涕" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "张口呼吸" } ] }, { "text": "可有疲乏、体重不增、食欲缺乏、贫血等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "疲乏" }, { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "体重" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "体重不增" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "贫血" } ] }, { "text": "多数上呼吸道感染症状在7~10天内缓解,如症状持续10天未见缓解,应怀疑急性鼻窦炎。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "急性鼻窦炎" } ] }, { "text": "如患儿病程虽在10天以内,但高热不退、伴流脓涕、头痛、眼眶肿胀,亦应考虑到本病可能。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "高热不退" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "伴流脓涕" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "头痛" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "眼眶肿胀" } ] }, { "text": "病程超过90天者为慢性鼻窦炎。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "慢性鼻窦炎" } ] }, { "text": "根据鼻咽部分泌物细菌培养和药物敏感试验结果选用适当的抗生素。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "鼻咽部分泌物" }, { "start_idx": 8, "end_idx": 11, "type": "ite", "entity": "细菌培养" }, { "start_idx": 13, "end_idx": 18, "type": "ite", "entity": "药物敏感试验" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "抗生素" } ] }, { "text": "可首选羟氨苄青霉素,如为产β-内酰胺酶的卡他莫拉菌或流感嗜血杆菌感染,或上述治疗失败,应选用含β-内酰胺酶抑制剂的复合青霉素类、大环内酯类、第二或第三代头孢霉素。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "羟氨苄青霉素" }, { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "卡他莫拉菌" }, { "start_idx": 26, "end_idx": 31, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 47, "end_idx": 55, "type": "dru", "entity": "β-内酰胺酶抑制剂" }, { "start_idx": 57, "end_idx": 62, "type": "dru", "entity": "复合青霉素类" }, { "start_idx": 64, "end_idx": 68, "type": "dru", "entity": "大环内酯类" }, { "start_idx": 70, "end_idx": 79, "type": "dru", "entity": "第二或第三代头孢霉素" } ] }, { "text": "局部可短期使用麻黄素等滴鼻,以保证引流通畅。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "麻黄素" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "滴鼻" } ] }, { "text": "对有过敏性鼻炎基础的患儿可考虑加用抗组胺药物、抗白三烯药物或鼻吸糖皮质激素治疗。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "过敏性鼻炎" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "抗组胺" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "抗白三烯" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "鼻吸" }, { "start_idx": 32, "end_idx": 36, "type": "dru", "entity": "糖皮质激素" } ] }, { "text": "但抗组胺药可使分泌物黏稠而影响鼻窦引流,无过敏背景患儿应避免使用。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "抗组胺药" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "分泌物黏稠" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "鼻窦" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "过敏" } ] }, { "text": "流感嗜血杆菌肺炎@二、流感嗜血杆菌肺炎流感嗜血杆菌(Hi)肺炎(hemophilusinfluenzaepneumonia)常见于5岁以下婴儿和年幼儿童。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "流感嗜血杆菌" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "Hi" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "肺炎" }, { "start_idx": 32, "end_idx": 60, "type": "dis", "entity": "hemophilusinfluenzaepneumonia" } ] }, { "text": "流感嗜血杆菌肺炎疾病@应用特异性免疫血清可将Hi分为a~f6型,其中以b型(Hib)致病力最强。", "entities": [ { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "b型(Hib)" } ] }, { "text": "流感嗜血杆菌肺炎@由于Hib疫苗的接种,20世纪90年代以后美国等发达国家Hib所致肺炎下降了95%。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "Hib疫苗" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "肺炎" } ] }, { "text": "流感嗜血杆菌肺炎@近年来也有较多非b型Hi感染的报道。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" } ] }, { "text": "流感嗜血杆菌肺炎@本病临床表现无特异性。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" } ] }, { "text": "流感嗜血杆菌肺炎@但起病多较缓慢,病程可长达数周之久。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" } ] }, { "text": "流感嗜血杆菌肺炎@幼婴常伴有菌血症,易出现脓胸、心包炎等化脓性并发症。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "菌血症" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "脓胸" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "心包炎" } ] }, { "text": "流感嗜血杆菌肺炎@外周血白细胞计数常中度升高。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 11, "end_idx": 14, "type": "ite", "entity": "血白细胞" } ] }, { "text": "流感嗜血杆菌肺炎@多数患儿X线表现为大叶性或节段性病灶,下叶多受累。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "X线" } ] }, { "text": "流感嗜血杆菌肺炎@幼婴常伴胸膜受累。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "胸膜受累" } ] }, { "text": "流感嗜血杆菌肺炎@本病诊断有赖于从血、胸水或肺穿刺液中分离到病菌。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 17, "end_idx": 25, "type": "bod", "entity": "血、胸水或肺穿刺液" } ] }, { "text": "流感嗜血杆菌肺炎@由于Hi在正常人群的咽部中有一定的携带率,托幼机构中更高,因而呼吸道标本诊断价值不大。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "咽部" } ] }, { "text": "流感嗜血杆菌肺炎@治疗时必须注意Hi的耐药问题。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" } ] }, { "text": "流感嗜血杆菌肺炎@目前分离的Hi主要耐药机制是产生β-内酰胺酶,美国、我国香港等地Hi菌株产酶率已高达30%以上。", "entities": [ { "start_idx": 25, "end_idx": 30, "type": "mic", "entity": "β-内酰胺酶" } ] }, { "text": "流感嗜血杆菌肺炎@国内各地关于氨苄西林耐药率和产酶率差异较大。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "氨苄西林" } ] }, { "text": "流感嗜血杆菌肺炎@如对病菌不产酶,可使用氨苄西林,如不能明确其是否产酶,首选头孢噻肟、头孢曲松等。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "头孢噻肟" }, { "start_idx": 43, "end_idx": 46, "type": "dru", "entity": "头孢曲松" } ] }, { "text": "流感嗜血杆菌肺炎@如最初反应良好,可改为口服,疗程为10~14天。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "口服" } ] }, { "text": "流感嗜血杆菌肺炎@在大环内酯类中,阿奇霉素、克拉霉素对Hi有较好的敏感性。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "流感嗜血杆菌肺炎" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "阿奇霉素" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "克拉霉素" } ] }, { "text": "参考文献1.AllahabadiaA,DaykinJ,SheppardMC,etal.Radioiodinetreatmentofhyperthyroidism-prognosticfactorsforoutcome.JClinEndocrinolMetab.2001,86:3611-36112.ArslanogluI,KutluH,IsguvenP,etal.DiagnosticvalueofpituitaryMRIindifferentiationofchildrenwithnormalgrowthhormonesecretion,isolatedgrowthhormonedeficiencyandmultiplepituitaryhormonedeficiency.JPediatrEndocrinolMetab.2001,14:517-5173.CarelJC,EugsterEA,RogolA,etal.ConsensusStatementontheuseofgonadotropin-releasinghormoneanalogsinchildren.Pediatrics,2009,123(4):e752-7524.GillamMP,KoppP.Geneticregulationofthyroiddevelopment.CurrOpinPediatr.2001,13:358-3585.GlaserNS,StyneDM,OrganizationofPediatricEndocrinologistsofNorthernCaliforniaCollaborativeGraves’DiseaseStudyGroup.PredictingthelikelihoodofremissioninchildrenwithGraves’disease:aprospective,multicenterstudy.Pediatrics.2008,121:e481-4886.LazarL,PadoaA,PhillipM.GrowthPatternandFinalHeightafterCessationofGonadotropin-SuppressiveTherapyinGirlswithCentralsexualprecocity.JClinEndocrinolMetab,2007,92:3483-34837.Behrman,kliegman,Jenson.NelsonTextbookofPediatric.17thedition,20048.KyriakieSarafoglou.PediatricEndocrinologyandInbornErrorsofMetabolism.McGrawHillMedical,20099.SpeiserPW,AzzizR,BaskinLS,etal.Congenitaladrenalhyperplasiaduetosteroid21-hydroxylasedeficiency:anEndocrineSocietyclinicalpracticeguideline.JClinEndocrinolMetab,2010,95:4133-416010.SexualPrecocity.JClinEndocrinolMetab,92:3483-348311.SpeiserPW,AzzizR,BaskinLS,etal.Congenitaladrenalhyperplasiaduetosteroid21-hydroxylasedeficiency:anEndocrineSocietyclinicalpracticeguideline.JClinEndocrinolMetab.2010,95:4133-416012.沈水仙,付华,陈兆文,等.上海市儿童1型糖尿病发病率调查(1980—1991).中华内分泌代谢杂志,1994(10):20213.王伟,江静,孙文鑫,等.国产重组人生长激素治疗55例儿童生长激素缺乏症临床综合分析.中华儿科杂志,1999,37:206-20614.曾畿生,王德芬.现代儿科内分泌学基础与临床.上海:上海科学技术文献出版社,2001:355-35515.中华医学会儿科学分会内分泌遗传代谢学组.矮身材儿童诊治指南.中华儿科杂志,2008,46:428-43016.中华医学会儿科学分会内分泌遗传代谢学组.儿童糖尿病酮症酸中毒诊疗指南.中华儿科杂志,2009,47:421-42517.中华医学会儿科学分会内分泌遗传代谢学组.中枢性(真性)性早熟诊治指南.中华儿科杂志,2007,45:426-42718.胡亚美.诸福堂实用儿科学.第7版.北京:人民卫生出版社,2002", "entities": [ { "start_idx": 1604, "end_idx": 1610, "type": "dis", "entity": "儿童1型糖尿病" }, { "start_idx": 1667, "end_idx": 1673, "type": "pro", "entity": "重组人生长激素" }, { "start_idx": 1679, "end_idx": 1687, "type": "dis", "entity": "儿童生长激素缺乏症" }, { "start_idx": 1688, "end_idx": 1689, "type": "dep", "entity": "临床" }, { "start_idx": 1697, "end_idx": 1698, "type": "dep", "entity": "儿科" }, { "start_idx": 1730, "end_idx": 1731, "type": "dep", "entity": "儿科" }, { "start_idx": 1739, "end_idx": 1740, "type": "dep", "entity": "临床" }, { "start_idx": 1777, "end_idx": 1778, "type": "dep", "entity": "儿科" }, { "start_idx": 1792, "end_idx": 1794, "type": "sym", "entity": "矮身材" }, { "start_idx": 1832, "end_idx": 1833, "type": "dep", "entity": "儿科" }, { "start_idx": 1847, "end_idx": 1856, "type": "dis", "entity": "儿童糖尿病酮症酸中毒" }, { "start_idx": 1892, "end_idx": 1893, "type": "dep", "entity": "儿科" }, { "start_idx": 1907, "end_idx": 1916, "type": "dis", "entity": "中枢性(真性)性早熟" }, { "start_idx": 1956, "end_idx": 1957, "type": "dep", "entity": "儿科" } ] }, { "text": "第四节血吸虫病血吸虫病(schistosomiasis)是血吸虫寄生人体所致的疾病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "血吸虫病" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "血吸虫病" }, { "start_idx": 12, "end_idx": 26, "type": "dis", "entity": "schistosomiasis" }, { "start_idx": 29, "end_idx": 31, "type": "mic", "entity": "血吸虫" } ] }, { "text": "在人体寄生的血吸虫主要有三种:即日本血吸虫(Schis-tosomajaponicum)、曼氏血吸虫(Schistosomamansoni)和埃及血吸虫(Schistosomahaematobium)。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "血吸虫" }, { "start_idx": 16, "end_idx": 20, "type": "mic", "entity": "日本血吸虫" }, { "start_idx": 22, "end_idx": 42, "type": "mic", "entity": "Schis-tosomajaponicum" }, { "start_idx": 45, "end_idx": 49, "type": "mic", "entity": "曼氏血吸虫" }, { "start_idx": 51, "end_idx": 68, "type": "mic", "entity": "Schistosomamansoni" }, { "start_idx": 71, "end_idx": 75, "type": "mic", "entity": "埃及血吸虫" }, { "start_idx": 77, "end_idx": 98, "type": "mic", "entity": "Schistosomahaematobium" } ] }, { "text": "日本血吸虫流行于日本、中国、菲律宾、印度尼西亚等国家。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "日本血吸虫" } ] }, { "text": "在此仅叙述在我国流行的日本血吸虫病。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "日本血吸虫病" } ] }, { "text": "【病因学】日本血吸虫成虫雌雄异体。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "mic", "entity": "日本血吸虫" } ] }, { "text": "尾蚴体壁侵入终宿主皮肤后,脱去尾部并迅速改变体壁一个单位膜(3层)为两个单位膜(7层)的构造,以适应宿主体内血清-盐水环境,以后至发育为成虫阶段均称童虫。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "皮肤" } ] }, { "text": "然后经淋巴管或小静脉进入血液循环。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "淋巴管" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "小静脉" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "血液" } ] }, { "text": "约在感染后3~5天到达肝内门静脉分支,发育至性成熟期,约在感染后第25天雌虫开始产卵,感染后35天左右粪便中可发现虫卵。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "肝内门静脉分支" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "粪便" } ] }, { "text": "【流行病学】(一)传染源患者为主要传染源,牛、羊、猪、马、猫、鼠、兔等30多种动物均可感染本病,因此血吸虫病是人畜共患的疾病。", "entities": [ { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "血吸虫病" } ] }, { "text": "(二)传染途径1.粪便入水方式很多,如河边涮洗马桶,河边粪坑外溢,随地大便,渔民、船民的粪便入河,粪船外溢,以及各种动物、尤以耕牛随处大便均可污染水源。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "粪便" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "大便" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "粪便" } ] }, { "text": "2.钉螺的存在有钉螺的地区才有血吸虫病流行,因此血吸虫病的分布基本与钉螺的分布一致,具有地方性。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "血吸虫病" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "血吸虫病" } ] }, { "text": "3.接触疫水人体感染本病主要系因在生产或生活活动中接触疫水,5岁以上小儿多于生活中接触疫水,渔民小儿自幼即在疫水中洗涤臀部、洗手、洗脚、洗澡而感染,此种情况随着渔民陆居而减少。", "entities": [ { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "臀部" }, { "start_idx": 63, "end_idx": 63, "type": "bod", "entity": "手" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "脚" } ] }, { "text": "(三)人群的易感性一般来说,人群对血吸虫普遍易感,通常5岁以下儿童感染率低,10岁以后感染率上升,15~30岁最高,然后逐渐下降;但这一流行的年龄曲线在重流行区并不明显。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "血吸虫" } ] }, { "text": "此外流行区居民多重复感染,提示感染血吸虫后宿主虽能产生免疫,并不能完全防御再感染。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "血吸虫" } ] }, { "text": "【发病机制及病理变化】自尾蚴进入人体后,血吸虫在人体的各个发育阶段,如童虫、成虫及虫卵均对人体有不同程度的致病作用,但其中以虫卵最为重要。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "mic", "entity": "血吸虫" } ] }, { "text": "虫卵的致病作用是临床血吸虫病的主要病理基础。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "血吸虫病" } ] }, { "text": "尾蚴侵入皮肤后转变为童虫,在局部皮肤引起以瘙痒和小丘疹为特点的尾蚴性皮炎。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "皮肤" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "局部皮肤引起以瘙痒和小丘疹" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "尾蚴性皮炎" } ] }, { "text": "童虫移行经过肺脏时,可引起肺脏点状出血和血管周围嗜酸性粒细胞和巨噬细胞浸润。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肺脏" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "血管" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "嗜酸性粒细胞" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "巨噬细胞" } ] }, { "text": "病理变化主要是虫卵沉积在肝、结肠等脏器内所致的组织损伤,即虫卵肉芽肿的形成。", "entities": [ { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肝" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "结肠" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "虫卵肉芽肿" } ] }, { "text": "这种病变主要发生在成熟虫卵沉着的小血管内。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "血管" } ] }, { "text": "此种组织学病理变化与单个结核结节极相似,故又称为假结核结节。", "entities": [ { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "假结核结节" } ] }, { "text": "日本血吸虫的寄生部位及其虫卵沉着的部位通常在门静脉系统的分支中,所以主要的器官病理变化也在与这系统相连的脏器内,尤以结肠和肝脏为最多、最严重。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "日本血吸虫" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "门静脉系统" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "结肠" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "肝脏" } ] }, { "text": "肝脏病变可分早、晚两期。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肝脏" } ] }, { "text": "早期主要由虫卵在门静脉细支内形成嗜酸性脓肿,发生坏死性血管炎,形成血栓;病变表面尚光滑,唯可见粟粒状黄色颗粒。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "门静脉细支" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "嗜酸性脓肿" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "坏死性血管炎" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "血栓" }, { "start_idx": 47, "end_idx": 53, "type": "sym", "entity": "粟粒状黄色颗粒" } ] }, { "text": "晚期见汇管区较大门静脉分支管腔阻塞及周围渐显结缔组织增生,增生显著者可致肝脏发生纤维性变血吸虫病肝硬化。", "entities": [ { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肝脏" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "肝脏发生纤维性变" }, { "start_idx": 44, "end_idx": 50, "type": "dis", "entity": "血吸虫病肝硬化" } ] }, { "text": "【临床表现】按病程发展及主要临床表现,可分为急性、慢性及晚期血吸虫病。", "entities": [ { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "血吸虫病" } ] }, { "text": "(一)急性血吸虫病多见于夏秋季,以小儿及青壮年为多。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "急性血吸虫病" } ] }, { "text": "主要症状如下:1.发热急性感染者均有发热,热型不一,常为弛张热、间歇热或不规则热;体温多达39℃以上,早晨低,晚间高,开始有寒冷感。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "发热" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "发热" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "弛张热" }, { "start_idx": 32, "end_idx": 34, "type": "sym", "entity": "间歇热" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "不规则热" }, { "start_idx": 41, "end_idx": 64, "type": "sym", "entity": "体温多达39℃以上,早晨低,晚间高,开始有寒冷感" } ] }, { "text": "2.消化系统症状要表现为结肠炎症状,腹泻最多见;重症或重复感染患者常排黏血便,且有腹痛、腹胀,偶有便秘。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "消化系统" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "结肠炎" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "腹泻" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "排黏血便" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "腹痛" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "腹胀" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "便秘" } ] }, { "text": "此外有食欲缺乏、恶心等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "恶心" } ] }, { "text": "肝脏中等度肿大压痛,脾脏轻度肿大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肝脏" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "肝脏中等度肿大" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "压痛" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "脾脏轻度肿大" } ] }, { "text": "3.呼吸系统症状约半数以上病例可出现咳嗽、胸痛、血痰等症状,系虫卵沉积肺部引起的组织反应,体征不明显。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "咳嗽" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "胸痛" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "血痰" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "虫卵" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肺部" } ] }, { "text": "(二)慢性血吸虫病流行区所见的患者大多为慢性早期血吸虫病,分无症状型及有症状型两种。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "慢性血吸虫病" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "血吸虫病" } ] }, { "text": "早期肝脏肿大,质地可稍硬,肝功能可正常或轻度损害,脾脏亦可轻度肿大血吸虫病系指慢性期病情较严重的情况。", "entities": [ { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "肝功能可正常或轻度损害" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "脾脏" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "脾脏亦可轻度肿大" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "血吸虫病" } ] }, { "text": "病理上呈典型的干线型肝纤维化改变。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "干线型肝纤维化改变" } ] }, { "text": "临床上主要表现为门脉高压全身代谢紊乱,甚至肝功能减退等症状和体征。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "门脉" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "门脉高压" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "全身代谢紊乱" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "肝" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "肝功能减退" } ] }, { "text": "根据突出的临床表现,一般分为巨脾、腹水及侏儒三型。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "巨脾" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "腹水" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "侏儒" } ] }, { "text": "巨脾型最为多见,脾肿大往往达脐部或脐下,超过中线;或脾肿大虽未达脐或超过中线,但已伴白细胞及血小板减少、贫血等脾功能亢进现象。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "巨脾" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "脾" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "脐部" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "脐下" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "脾" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "脐" }, { "start_idx": 42, "end_idx": 50, "type": "sym", "entity": "白细胞及血小板减少" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "贫血" }, { "start_idx": 55, "end_idx": 59, "type": "sym", "entity": "脾功能亢进" } ] }, { "text": "腹水型是晚期血吸虫病的严重症状,由门脉阻塞、营养不良、低蛋白血症、淋巴循环障碍等原因引起。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "腹水" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "血吸虫病" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "门脉阻塞" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "营养不良" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "低蛋白血症" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "淋巴循环障碍" } ] }, { "text": "异位损害及并发症系指成虫或虫卵寄生及迷走在门静脉系统之外的器官所致的病变。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "成虫" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "虫卵" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "门静脉系统" } ] }, { "text": "异位寄生主要见于大量尾蚴感染时,因侵入人体的童虫过多引起满溢现象,或离开了移行常轨而被阻留于异常部位所致,或正常寄生部位成虫排卵随血管交通支沉着于门脉系统以外的脏器。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "mic", "entity": "童虫" }, { "start_idx": 73, "end_idx": 76, "type": "bod", "entity": "门脉系统" } ] }, { "text": "肺、脑异位损害最为常见,肺脏的异位损害多为虫卵经过肝窦或侧支循环到达肺脏,偶亦见于成虫寄生于肺小血管产卵引起,可有咳嗽、咳白色痰等症状。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "肺脏的异位损害" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "虫卵" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肝窦" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "侧支循环" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肺脏" }, { "start_idx": 41, "end_idx": 42, "type": "mic", "entity": "成虫" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "肺小血管" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "咳嗽" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "咳白色痰" } ] }, { "text": "脑部造成的异位损害,常见者主要为急性感染时童虫移行至脑血管中,致使成虫产卵沉积在脑部,急性期可发生类似脑炎、脑膜炎的症状,经病原学治疗症状很快消失。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脑部" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "异位损害" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "急性感染" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "童虫" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "脑血管" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "脑部" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "脑炎" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "【实验室诊断】(一)粪便查虫卵直接涂片找虫卵,或经沉淀后涂片找虫卵。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "粪便" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "虫卵" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "虫卵" }, { "start_idx": 31, "end_idx": 32, "type": "mic", "entity": "虫卵" } ] }, { "text": "大面积普查均用粪便沉淀孵化法。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "粪便" } ] }, { "text": "另外,改良加藤厚涂片法,为定量检查粪便中虫卵的常用方法。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "改良加藤厚涂片" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "粪便" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "虫卵" } ] }, { "text": "(二)直肠黏膜活组织压片检查一般用直肠镜,于距肛门10~12cm处夹取三小块组织活检,可查见活卵、变性卵及死卵。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "pro", "entity": "直肠黏膜活组织压片检查" }, { "start_idx": 17, "end_idx": 19, "type": "equ", "entity": "直肠镜" }, { "start_idx": 46, "end_idx": 47, "type": "mic", "entity": "活卵" }, { "start_idx": 49, "end_idx": 51, "type": "mic", "entity": "变性卵" }, { "start_idx": 53, "end_idx": 54, "type": "mic", "entity": "死卵" } ] }, { "text": "(三)免疫学检查1.成虫抗原皮内试验现用成虫干粉抗原,可作为初筛方法。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "pro", "entity": "成虫抗原皮内试验" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "成虫" } ] }, { "text": "2.环卵沉淀试验(COPT)阳性率平均为97.3%,假阳性率3.1%,对肺吸虫及丝虫病交叉反应,3~4年阴转率为82.5%,可作为考核疗效的参考。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "环卵沉淀试验" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "COPT" } ] }, { "text": "3.间接血细胞凝集试验(IHA)具有高度敏感性及一定的特异性。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "间接血细胞凝集试验" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "IHA" } ] }, { "text": "4.酶联免疫吸附试验(enzymelinkedimmunsorbentassay,ELISA)阳性率达97.6%~100%,假阳性仅0%~2.4%,但晚期血吸虫患者血中特异性IgE抗体的阳性率仅27.5%,远比IgG抗体阳性率低,治疗后6~8个月各种抗体的阴转率不同。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "酶联免疫吸附试验" }, { "start_idx": 11, "end_idx": 39, "type": "pro", "entity": "enzymelinkedimmunsorbentassay" }, { "start_idx": 41, "end_idx": 45, "type": "pro", "entity": "ELISA" }, { "start_idx": 47, "end_idx": 60, "type": "sym", "entity": "阳性率达97.6%~100%" }, { "start_idx": 62, "end_idx": 72, "type": "sym", "entity": "假阳性仅0%~2.4%" }, { "start_idx": 77, "end_idx": 79, "type": "mic", "entity": "血吸虫" }, { "start_idx": 82, "end_idx": 82, "type": "bod", "entity": "血" }, { "start_idx": 84, "end_idx": 91, "type": "ite", "entity": "特异性IgE抗体" } ] }, { "text": "可有助于鉴别急性和慢性血吸虫病和疗效考核。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "dis", "entity": "急性和慢性血吸虫病" } ] }, { "text": "5.血清中循环抗原的检测在感染血吸虫的宿主体液内可检出3种游离的血吸虫循环抗原,即肠相关抗原(gutassociatedantigens,GAA)、膜相关抗原(membraneassociatedantigens,MAA)、可溶性虫卵抗原(SEA),通常在感染后1~4周出现,GAA出现最早。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "mic", "entity": "血吸虫" }, { "start_idx": 32, "end_idx": 34, "type": "mic", "entity": "血吸虫" } ] }, { "text": "【鉴别诊断】1.急性期需与上呼吸道感染、急性胃肠炎、肠炎、痢疾、伤寒和急性粟粒结核、败血症等鉴别。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "急性胃肠炎" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "肠炎" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "痢疾" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "伤寒" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "急性粟粒结核" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "败血症" } ] }, { "text": "2.慢性期应与无黄疸性肝炎鉴别,后者肝功能损害较明显,嗜酸性粒细胞增多,粪便孵化始终阴性。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "黄疸性肝炎" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "肝功能损害" }, { "start_idx": 27, "end_idx": 32, "type": "ite", "entity": "嗜酸性粒细胞" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "嗜酸性粒细胞增多" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "粪便" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "粪便孵化始终阴性" } ] }, { "text": "血吸虫肝纤维化慢性腹泻,少见黄疸、蜘蛛痣及肝掌等,而巨脾与食管下端静脉曲张消灭传染源在流行区每年进行普查、普治。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "血吸虫" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肝" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "血吸虫肝纤维化" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "慢性腹泻" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "黄疸" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "蜘蛛痣" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "肝掌" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "巨脾" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "食管下端静脉" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "食管下端静脉曲张" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "消灭传染源" } ] }, { "text": "目前人畜同步化疗已列为消灭传染源的措施。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "消灭传染源" } ] }, { "text": "2.切断传染环节粪便管理和消灭钉螺。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "切断传染环节" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "粪便管理" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "消灭钉螺" } ] }, { "text": "3.保护易感人群尽量避免接触疫水。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "保护易感人群" }, { "start_idx": 8, "end_idx": 15, "type": "pro", "entity": "尽量避免接触疫水" } ] }, { "text": "免疫预防,血吸虫疫苗仍在试验阶段。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "血吸虫疫苗" } ] }, { "text": "4.药物预防包括吡喹酮、蒿甲醚和青蒿琥酯。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "吡喹酮" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "蒿甲醚" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "青蒿琥酯" } ] }, { "text": "【治疗】(一)一般疗法除特异性疗法以外应注意一般疗法,如增进营养、补充蛋白质与维生素等,特别是晚期营养不良、贫血、肝功能不佳者更应注意支持疗法。", "entities": [ { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "增进营养" }, { "start_idx": 33, "end_idx": 37, "type": "pro", "entity": "补充蛋白质" }, { "start_idx": 39, "end_idx": 41, "type": "pro", "entity": "维生素" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "营养不良" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "贫血" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "肝功能不佳" } ] }, { "text": "(二)驱虫疗法吡喹酮具有高效、低毒、口服简便、疗程短等特点,便于推广,可用于急、慢性各期以及伴有并发症的血吸虫病的治疗。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "吡喹酮" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "口服" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "血吸虫病" } ] }, { "text": "有建议用120mg/kg,儿童140mg/kg的六日疗法治疗急性血吸虫病,其中1/2量在第1、2天内服用,余下的1/2量分4天内服完。", "entities": [ { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "急性血吸虫病" } ] }, { "text": "2.不良反应少而轻,且多为一过性,可有神经肌肉系统症状,如头昏、头痛、乏力、四肢酸痛、眩晕等较为多见,其次为晕厥、肌颤动、视力模糊、失眠、嗜睡、多汗、肢端麻木、耳鸣、步态不稳等。", "entities": [ { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "神经肌肉系统" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "头昏" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "头痛" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "乏力" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "四肢酸痛" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "眩晕" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "晕厥" }, { "start_idx": 57, "end_idx": 59, "type": "sym", "entity": "肌颤动" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "视力模糊" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "失眠" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "嗜睡" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "多汗" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "肢端麻木" }, { "start_idx": 80, "end_idx": 81, "type": "sym", "entity": "耳鸣" }, { "start_idx": 83, "end_idx": 86, "type": "sym", "entity": "步态不稳" } ] }, { "text": "心血管系统症状为胸闷、心悸、期前收缩等较为多见,偶见室上性心动过速。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "心血管系统" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "胸闷" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "心悸" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "期前收缩" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "室上性心动过速" } ] }, { "text": "3.疗效多数患者于治疗半个月后粪便检查转阴。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "粪便检查" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "粪便检查转阴" } ] }, { "text": "(三)外科治疗门脉高压引起食管静脉曲张者,或巨脾Ⅲ级及Ⅱ级并发脾功能亢进者,为脾切除和分流手术的指征。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "门脉高压" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "食管静脉曲张" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "巨脾Ⅲ级及Ⅱ级" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "脾功能亢进" }, { "start_idx": 39, "end_idx": 41, "type": "pro", "entity": "脾切除" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "分流手术" } ] }, { "text": "二、正常新生儿的外观特点(一)整体外观新生儿头大、躯干长四肢短头部和全身的比例为1∶4。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "头大" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "躯干" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "躯干长" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "四肢" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "四肢短" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "头部" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "全身" } ] }, { "text": "初生新生儿往往呈屈曲状,这与胎儿在母亲宫内呈屈曲状姿势有关。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "初生新生儿往往呈屈曲状" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "宫" } ] }, { "text": "(二)皮肤外观1.胎脂出生后皮肤覆盖一层白色脂质物,有保护皮肤的作用;若呈黄色,提示有黄疸、窒息或过期产存在;若呈黄绿色,提示有胎粪污染羊水黄疸生理性黄疸多在生后2~3天内出现,5~6天左右为高峰,1周后消失。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "皮肤" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胎脂" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "皮肤" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "白色脂质物" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "皮肤" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "黄疸" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "窒息" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "过期产" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "胎粪" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "羊水" }, { "start_idx": 64, "end_idx": 69, "type": "sym", "entity": "胎粪污染羊水" }, { "start_idx": 70, "end_idx": 71, "type": "dis", "entity": "黄疸" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "生理性黄疸" } ] }, { "text": "3.水肿生后3~5天在手、足、小腿、耻骨区及眼窝等处有明显的水肿,2~3天后可以消退,可能与新生儿水代谢有关。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "水肿" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "手" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "足" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "小腿" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "耻骨区" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "眼窝" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "水肿" } ] }, { "text": "4.新生儿红斑生后1~2天在头面部、躯干和四肢有散在的多形红斑,大小不等,1~2天退清。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "新生儿红斑" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "头面部" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "躯干" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "四肢" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "多形红斑" } ] }, { "text": "5.粟粒疹在鼻尖、鼻翼、面颊部因皮脂腺堆积形成针头样黄白色皮疹,蜕皮后自然消退。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "粟粒疹" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "鼻尖" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "鼻翼" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "面颊部" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "皮脂腺" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "黄白色皮疹" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "皮" } ] }, { "text": "6.汗疱疹因新生儿汗腺功能欠佳,在前胸和前额多见针头样水疱疹,与周围温度过高有关。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "汗疱疹" }, { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "汗腺功能" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "前胸" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "前额" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "水疱疹" } ] }, { "text": "7.青记在新生儿的背部或臀部有蓝绿色色斑,为色素细胞沉着有关,随年龄增加而自动消退。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "青记" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "背部" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "臀部" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "蓝绿色色斑" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "色素细胞" } ] }, { "text": "(三)头面颈部外观1.头颅新生儿颅骨较软骨缝可呈分离状产道时受到挤压而有骨缝重叠,数天后可以自行恢复;另有部分新生儿可有头皮水肿眼部生儿经常闭眼部分新生儿可以有球结膜毛细血管破裂而出血数天后可自行吸收。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "头" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "面" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "颈" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "头颅" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "颅骨" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "颅骨较软" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "骨缝" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "骨缝可呈分离状" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "产道" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "头皮" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "头皮水肿" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "眼部" }, { "start_idx": 71, "end_idx": 71, "type": "bod", "entity": "眼" }, { "start_idx": 66, "end_idx": 71, "type": "sym", "entity": "生儿经常闭眼" }, { "start_idx": 80, "end_idx": 88, "type": "dis", "entity": "球结膜毛细血管破裂" }, { "start_idx": 90, "end_idx": 91, "type": "dis", "entity": "出血" }, { "start_idx": 72, "end_idx": 91, "type": "sym", "entity": "部分新生儿可以有球结膜毛细血管破裂而出血" }, { "start_idx": 92, "end_idx": 99, "type": "sym", "entity": "数天后可自行吸收" } ] }, { "text": "3.鼻新生儿鼻梁较低鼻腔黏膜容易肿胀透气困难而张口呼吸。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "鼻" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "鼻梁" }, { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "新生儿鼻梁较低" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "鼻腔黏膜" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "鼻腔黏膜容易肿胀" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "透气困难" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "张口呼吸" } ] }, { "text": "4.口腔新生儿牙龈上可有黄白色小颗粒板牙”或“马牙”,此系上皮细胞堆积或黏液包裹,不应挑破;在硬腭中线上有大小为2~4mm的黄色小结,称为彭氏结,也系上皮细胞堆积,数周后自行消退;新生儿两颊各有一个隆起的黄色脂肪垫,俗称“螳螂嘴”,这是新生儿吸吮的重要工具,不可损伤。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "口腔" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "牙龈" }, { "start_idx": 4, "end_idx": 17, "type": "sym", "entity": "新生儿牙龈上可有黄白色小颗粒" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "板牙" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "马牙" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "黏液" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "硬腭" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "黄色小结" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "彭氏结" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 93, "end_idx": 94, "type": "bod", "entity": "两颊" }, { "start_idx": 104, "end_idx": 106, "type": "bod", "entity": "脂肪垫" }, { "start_idx": 111, "end_idx": 113, "type": "bod", "entity": "螳螂嘴" } ] }, { "text": "5.耳新生儿耳部的大小、外形、结构和坚硬度,均和遗传及成熟度相关,在新生儿胎龄评分中,耳廓的坚硬程度是其中的一项重要指标。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "耳" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "耳部" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "耳廓" } ] }, { "text": "6.颈部新生儿颈短皱褶处容易潮湿和糜烂胸部新生儿胸部呈圆筒状剑突有时可以上翘肋外翻乳晕增深可触及乳房小结激素经过胎盘到达胎儿所致,2~3周后可以自行消退;也是判定新生儿胎龄的指标之一。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "颈部" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "颈" }, { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "新生儿颈短" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "皱褶处" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "糜烂" }, { "start_idx": 9, "end_idx": 18, "type": "sym", "entity": "皱褶处容易潮湿和糜烂" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胸部" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "胸部" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "新生儿胸部呈圆筒状" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "剑突" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "剑突有时可以上翘" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "肋" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "肋外翻" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "乳晕" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "乳晕增深" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "乳房小结" }, { "start_idx": 45, "end_idx": 51, "type": "sym", "entity": "可触及乳房小结" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "激素" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "胎盘" } ] }, { "text": "(五)腹部新生儿腹部有时可以较为膨隆蛙状腹”;由于新生儿肋间肌不能完成呼吸做功,需要膈肌的升降来帮助呼吸,所以新生儿可有腹式呼吸脐带结扎后,留有的脐残端经无菌包扎后,一般在7天内自行脱落;部分新生儿脐残端脱落前后,脐部可有浆液性分泌物或渗血肛门与生殖器为排除新生儿畸形,应做肛指检查,胎粪的排出情况可提示新生儿肛门有无异常。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "腹部" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "腹部" }, { "start_idx": 5, "end_idx": 17, "type": "sym", "entity": "新生儿腹部有时可以较为膨隆" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "蛙状腹" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "肋间肌" }, { "start_idx": 35, "end_idx": 36, "type": "ite", "entity": "呼吸" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "膈肌" }, { "start_idx": 50, "end_idx": 51, "type": "ite", "entity": "呼吸" }, { "start_idx": 60, "end_idx": 60, "type": "bod", "entity": "腹" }, { "start_idx": 62, "end_idx": 63, "type": "ite", "entity": "呼吸" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "腹式呼吸" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "脐带" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "脐残端" }, { "start_idx": 77, "end_idx": 80, "type": "pro", "entity": "无菌包扎" }, { "start_idx": 99, "end_idx": 101, "type": "bod", "entity": "脐残端" }, { "start_idx": 107, "end_idx": 108, "type": "bod", "entity": "脐部" }, { "start_idx": 111, "end_idx": 116, "type": "bod", "entity": "浆液性分泌物" }, { "start_idx": 119, "end_idx": 119, "type": "bod", "entity": "血" }, { "start_idx": 118, "end_idx": 119, "type": "sym", "entity": "渗血" }, { "start_idx": 120, "end_idx": 121, "type": "bod", "entity": "肛门" }, { "start_idx": 123, "end_idx": 125, "type": "bod", "entity": "生殖器" }, { "start_idx": 137, "end_idx": 140, "type": "pro", "entity": "肛指检查" }, { "start_idx": 142, "end_idx": 143, "type": "bod", "entity": "胎粪" }, { "start_idx": 155, "end_idx": 156, "type": "bod", "entity": "肛门" } ] }, { "text": "部分新生儿生后可以有会阴部水肿,数天后可自行消退;少数男婴可以有双侧或单侧睾丸未降睾丸在腹股沟处单侧或双侧鞘膜积液假月经”,此系母体来源的雌激素撤退后引起,表现为新生女婴在生后2~7天内有灰白色或血性黏液从阴道流出,可持续2周左右。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "阴部水肿" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "睾丸" }, { "start_idx": 32, "end_idx": 40, "type": "sym", "entity": "双侧或单侧睾丸未降" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "睾丸" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "腹股沟处" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "睾丸在腹股沟处" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "鞘膜" }, { "start_idx": 48, "end_idx": 56, "type": "sym", "entity": "单侧或双侧鞘膜积液" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "假月经" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "雌激素" }, { "start_idx": 94, "end_idx": 106, "type": "sym", "entity": "灰白色或血性黏液从阴道流出" } ] }, { "text": "参考文献1.许积德.小儿内科学.北京:人民卫生出版社,1995:122-1222.尹音,胡敏.儿童口腔学.北京:人民军医出版社,1995:34-343.郑麟蕃,张震康,余光岩.实用口腔科学.北京:人民卫生出版社,1999:143-143", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dep", "entity": "小儿内科" }, { "start_idx": 47, "end_idx": 50, "type": "dep", "entity": "儿童口腔" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "口腔" } ] }, { "text": "四、耐药结核菌的治疗耐药结核病一般分为4类:1.单耐药(monoresistance)结核病患者感染的结核分枝杆菌经体外证实对1种抗结核药物耐药。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "mic", "entity": "结核菌" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "耐药结核病" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "结核病" }, { "start_idx": 51, "end_idx": 56, "type": "mic", "entity": "结核分枝杆菌" }, { "start_idx": 65, "end_idx": 69, "type": "dru", "entity": "抗结核药物" } ] }, { "text": "2.多耐药(polyresistance)结核病患者感染的结核分枝杆菌经体外证实对1种以上的抗结核药物耐药,但不包括同时耐异烟肼、利福平。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "结核病" }, { "start_idx": 29, "end_idx": 34, "type": "mic", "entity": "结核分枝杆菌" }, { "start_idx": 46, "end_idx": 50, "type": "dru", "entity": "抗结核药物" }, { "start_idx": 61, "end_idx": 63, "type": "dru", "entity": "异烟肼" }, { "start_idx": 65, "end_idx": 67, "type": "dru", "entity": "利福平" } ] }, { "text": "3.耐多药(multidrugresistance,MDR)结核病患者感染的结核分枝杆菌经体外证实至少同时对异烟肼、利福平耐药。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "结核病" }, { "start_idx": 38, "end_idx": 43, "type": "mic", "entity": "结核分枝杆菌" }, { "start_idx": 54, "end_idx": 56, "type": "dru", "entity": "异烟肼" }, { "start_idx": 58, "end_idx": 60, "type": "dru", "entity": "利福平" } ] }, { "text": "4.广泛耐药(extensivelydrugresistance,XDR)结核病患者感染的结核分枝杆菌体外被证实除至少同时对异烟肼、利福平耐药外,还对任何氟喹诺酮类药物产生耐药,以及3种二线抗结核注射药物(卷曲霉素、卡那霉素和阿米卡星)中的至少1种耐药。", "entities": [ { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "结核病" }, { "start_idx": 45, "end_idx": 50, "type": "mic", "entity": "结核分枝杆菌" }, { "start_idx": 62, "end_idx": 64, "type": "dru", "entity": "异烟肼" }, { "start_idx": 66, "end_idx": 68, "type": "dru", "entity": "利福平" }, { "start_idx": 77, "end_idx": 83, "type": "dru", "entity": "氟喹诺酮类药物" }, { "start_idx": 103, "end_idx": 106, "type": "dru", "entity": "卷曲霉素" }, { "start_idx": 108, "end_idx": 111, "type": "dru", "entity": "卡那霉素" }, { "start_idx": 113, "end_idx": 116, "type": "dru", "entity": "阿米卡星" } ] }, { "text": "对耐药结核病的预防胜于治疗。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "耐药结核病" } ] }, { "text": "首先要实施国家控制结核病规划(NTP),做好原发性耐药结核病易感人群的防治。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "结核病" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "原发性耐药结核病" } ] }, { "text": "耐异烟肼或耐利福平者,根据病情可选择氟喹诺酮中的氧氟沙星或左氧氟沙星。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "异烟肼" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "利福平" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "氟喹诺酮" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "氧氟沙星" }, { "start_idx": 29, "end_idx": 33, "type": "dru", "entity": "左氧氟沙星" } ] }, { "text": "耐链霉素者可选用乙胺丁醇替代,也可选择1种二线注射剂药物。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "链霉素" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "乙胺丁醇" } ] }, { "text": "PZA可全疗程应用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "PZA" } ] }, { "text": "单耐利福平者疗程一般为12个月,单耐其他药物者疗程至少9个月,耐2种药物者疗程为12个月,耐3~4种药物者疗程至少18个月。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "利福平" } ] }, { "text": "对病变广泛者宜同时选择氟喹诺酮类药物和注射剂,或适当延长疗程。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "dru", "entity": "氟喹诺酮类药物" } ] }, { "text": "参考文献1.吴育锦,隋邦森,贾礼明,等.脑内小脓肿40例临床研究.中华神经精神科杂志,1988,21:352-3522.董宇为,夏学巍,杜怡庆等.儿童脑脓肿的显微外科治疗体会.中华小儿外科杂志,2010,31(1):1-13.SuTM,LinYC,LuCH,etal.Streptococcalbrainabscess:analysisofclinicalfeaturesin20patients.SurgNeurol.2001,56(3):189-1894.HabibAA,MozaffarT.Brainabscess.ArchNeurol.2001,58(8):1302-13025.Bair-MerrittMH,ShahSS,ZaoutisTE,BellLM,FeudtnerC.Suppurativeintracranialcomplicationsofsinusitisinpreviouslyhealthychildren.PediatrInfectDisJ.2005,24(4):384-3846.MarquardtG,SchickU,Moller-HartmannW.Brainabscess.JNeurosurg.2001,94(6):1020-10207.HakanT,CeranN,ErdemI,BerkmanMZ,GoktasP.Bacterialbrainabscesses:anevaluationof96cases.JInfect.2006,52(5):359-3598.TononE,ScottonPG,GallucciM,etal.Brainabscess:clinicalaspectsof100patients.IntJInfectDis.2006,10(2):103-1039.Shachor-MeyouhasY,Bar-JosephG,GuilburdJN,etal.Brainabscessinchildren-epidemiology,predisposingfactorsandmanagementinthemodernmedicineera.ActaPaediatr.2010,99(8):1163-1163", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "脑内小脓肿" }, { "start_idx": 35, "end_idx": 39, "type": "dep", "entity": "神经精神科" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "儿童脑脓肿" }, { "start_idx": 81, "end_idx": 82, "type": "dep", "entity": "外科" }, { "start_idx": 92, "end_idx": 93, "type": "dep", "entity": "外科" } ] }, { "text": "二、常见疾病的影像诊断(一)食管闭锁和食管气管瘘先天性食管闭锁及食管气管瘘(congenitalesophagealatresiaandtracheoesophagealfistula)是新生儿常见的胃肠道畸形。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "食管闭锁" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "食管气管瘘" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "先天性食管闭锁" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "食管气管瘘" }, { "start_idx": 38, "end_idx": 91, "type": "dis", "entity": "congenitalesophagealatresiaandtracheoesophagealfistula" }, { "start_idx": 100, "end_idx": 104, "type": "dis", "entity": "胃肠道畸形" } ] }, { "text": "根据畸形的解剖关系将食管闭锁分为五型:A型:食管近、远段均闭锁,无食管气管瘘B型:食管远段呈盲端C型:食管近段闭锁,远段有瘘与气管相通D型:食管近、远两段均与气管相通;E型:食管无闭锁,仅有食管气管瘘生后数小时开始流口水、吐白沫、喂奶时咳呛、呕吐甚至气促青紫为特征。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "食管闭锁" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "A型" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "食管" }, { "start_idx": 22, "end_idx": 37, "type": "sym", "entity": "食管近、远段均闭锁,无食管气管瘘" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "B型" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "食管" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "食管远段呈盲端" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "C型" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "食管" }, { "start_idx": 51, "end_idx": 66, "type": "sym", "entity": "食管近段闭锁,远段有瘘与气管相通" }, { "start_idx": 67, "end_idx": 68, "type": "dis", "entity": "D型" }, { "start_idx": 70, "end_idx": 82, "type": "sym", "entity": "食管近、远两段均与气管相通" }, { "start_idx": 84, "end_idx": 85, "type": "dis", "entity": "E型" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "食管" }, { "start_idx": 87, "end_idx": 99, "type": "sym", "entity": "食管无闭锁,仅有食管气管瘘" }, { "start_idx": 100, "end_idx": 119, "type": "sym", "entity": "生后数小时开始流口水、吐白沫、喂奶时咳呛" }, { "start_idx": 121, "end_idx": 128, "type": "sym", "entity": "呕吐甚至气促青紫" } ] }, { "text": "X线表现:①胸腹部平片:由于食管气管瘘的存在,大多数均有吸入性肺炎,右上肺最常受累。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "X线" }, { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "胸腹部平片" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "食管气管瘘" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "吸入性肺炎" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "右上肺" } ] }, { "text": "上纵隔内显示一充气盲囊影,为上段食管闭锁之盲袋。", "entities": [ { "start_idx": 0, "end_idx": 22, "type": "sym", "entity": "上纵隔内显示一充气盲囊影,为上段食管闭锁之盲袋" } ] }, { "text": "多数患儿因有食管远段一气管瘘,腹部有气体;少数病例远段食管与气管无交通,腹部无气体。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "多数患儿因有食管远段一气管瘘,腹部有气体" }, { "start_idx": 21, "end_idx": 40, "type": "sym", "entity": "少数病例远段食管与气管无交通,腹部无气体" } ] }, { "text": "食管闭锁常合并其他消化道、心血管及骨骼畸形,因此常规胸腹部照片是不可缺少的。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "食管闭锁" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "消化道" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "心血管" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "骨骼畸形" }, { "start_idx": 26, "end_idx": 30, "type": "pro", "entity": "胸腹部照片" } ] }, { "text": "但平片不能最后确诊。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "平片" } ] }, { "text": "②自鼻胃管内注入1~2ml稀钡剂,透视下点片,转动体位至侧卧位便于瘘管显影。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "自鼻胃管" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "稀钡剂" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "瘘管显影" } ] }, { "text": "(二)食管裂孔疝食管裂孔疝(hiatushernia)是小儿常见的呕吐原因。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "食管裂孔疝" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "食管裂孔疝" }, { "start_idx": 14, "end_idx": 25, "type": "dis", "entity": "hiatushernia" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "呕吐" } ] }, { "text": "系食管下端和周围膈食管裂孔的先天发育缺陷食管裂孔滑疝、食管旁疝和混合型疝三种类型。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "食管" }, { "start_idx": 1, "end_idx": 19, "type": "sym", "entity": "食管下端和周围膈食管裂孔的先天发育缺陷" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "食管裂孔滑疝" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "食管旁疝" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "混合型疝" } ] }, { "text": "最多见的为食管裂孔滑疝,其胸腹部平片无特征,钡餐显示:①食管持续反流胃底疝入膈上2~4cm,胃黏膜较粗、数目多、弯曲食管环位于膈上食管裂孔增大,食管胃角增大变钝胃食管反流当胃食管抗反流屏障作用发生异常,则极易导致病理性胃食管反流。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "食管裂孔滑疝" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "胸腹部平片" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "钡餐" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "食管" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "食管持续反流" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 34, "end_idx": 57, "type": "sym", "entity": "胃底疝入膈上2~4cm,胃黏膜较粗、数目多、弯曲" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "食管" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "食管环位于膈上" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "食管胃角" }, { "start_idx": 65, "end_idx": 79, "type": "sym", "entity": "食管裂孔增大,食管胃角增大变钝" }, { "start_idx": 80, "end_idx": 84, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 86, "end_idx": 93, "type": "bod", "entity": "胃食管抗反流屏障" }, { "start_idx": 106, "end_idx": 113, "type": "dis", "entity": "病理性胃食管反流" } ] }, { "text": "生后3个月以上仍有反流应考虑为病理性。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "反流" } ] }, { "text": "胃内容物反流到食管,食管下端黏膜受到反流液的侵袭而导致反流性食管炎。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "食管" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "食管下端黏膜" }, { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "胃内容物反流到食管,食管下端黏膜受到反流液的侵袭" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "反流性食管炎" } ] }, { "text": "临床表现为呕吐、肺部反复感染,并发生反流性食管炎。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "呕吐" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "反流性食管炎" } ] }, { "text": "影像诊断:①钡餐检查:仰卧位见胃内钡剂进入食管即可诊断,但需注意5分钟内反流3次方能诊断。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "钡餐检查" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "胃" }, { "start_idx": 17, "end_idx": 18, "type": "dru", "entity": "钡剂" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "食管" } ] }, { "text": "另外,还可见反流性食管炎的X线表现。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "反流性食管炎" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "X线" } ] }, { "text": "早期和轻度的食管炎引起食管痉挛性收缩,在钡剂造影时常可以看到食管下端数厘米的一段轻度狭窄,其上方有少量钡剂存留,尚可通过食管黏膜面呈针尖状钡点影或颗粒状小结节影超声检查:食管有一半以上充盈,且在下段食管有液体来回运动为阳性反流的诊断标准。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "食管炎" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "食管痉挛性收缩" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "钡剂造影" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "食管" }, { "start_idx": 51, "end_idx": 52, "type": "dru", "entity": "钡剂" }, { "start_idx": 19, "end_idx": 59, "type": "sym", "entity": "在钡剂造影时常可以看到食管下端数厘米的一段轻度狭窄,其上方有少量钡剂存留,尚可通过" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "食管黏膜" }, { "start_idx": 60, "end_idx": 79, "type": "sym", "entity": "食管黏膜面呈针尖状钡点影或颗粒状小结节影" }, { "start_idx": 80, "end_idx": 83, "type": "pro", "entity": "超声检查" }, { "start_idx": 85, "end_idx": 86, "type": "bod", "entity": "食管" }, { "start_idx": 97, "end_idx": 100, "type": "bod", "entity": "下段食管" }, { "start_idx": 85, "end_idx": 117, "type": "sym", "entity": "食管有一半以上充盈,且在下段食管有液体来回运动为阳性反流的诊断标准" } ] }, { "text": "(四)胃及十二指肠疾病1.先天性肥厚性幽门狭窄先天性肥厚性幽门狭窄多见于足月儿,男多于女,发生率男女之比约5∶1。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "胃" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "十二指肠" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "先天性肥厚性幽门狭窄" }, { "start_idx": 23, "end_idx": 32, "type": "dis", "entity": "先天性肥厚性幽门狭窄" } ] }, { "text": "生后2~3周出现进食后呕吐,进行性加重呈喷射性呕吐,呕吐物不含胆汁。", "entities": [ { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "生后2~3周出现进食后呕吐,进行性加重呈喷射性呕吐" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "呕吐物不含胆汁" } ] }, { "text": "可见胃蠕动波,右上腹可触及橄榄样包块。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "可见胃蠕动波,右上腹可触及橄榄样包块" } ] }, { "text": "(1)腹部直立位平片:可见典型的单气泡征。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "腹部直立位平片" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "单气泡征" } ] }, { "text": "钡餐检查具特异性,其特征改变为:①幽门管梗阻征象:钡剂排空延迟,胃蠕动增强幽门管细长呈线样征及鸟嘴征;③幽门环肌肥厚产生的征象:肥厚的环肌在胃窦的压迹呈肩样征,在十二指肠球底压迹呈蕈样征超声检查:可探得幽门管的长度和幽门壁的厚度以协助诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "钡餐检查" }, { "start_idx": 25, "end_idx": 26, "type": "dru", "entity": "钡剂" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "胃" }, { "start_idx": 17, "end_idx": 36, "type": "sym", "entity": "幽门管梗阻征象:钡剂排空延迟,胃蠕动增强" }, { "start_idx": 37, "end_idx": 49, "type": "sym", "entity": "幽门管细长呈线样征及鸟嘴征" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "幽门环肌" }, { "start_idx": 70, "end_idx": 71, "type": "dru", "entity": "胃窦" }, { "start_idx": 81, "end_idx": 85, "type": "bod", "entity": "十二指肠球" }, { "start_idx": 52, "end_idx": 92, "type": "sym", "entity": "幽门环肌肥厚产生的征象:肥厚的环肌在胃窦的压迹呈肩样征,在十二指肠球底压迹呈蕈样征" }, { "start_idx": 93, "end_idx": 96, "type": "pro", "entity": "超声检查" }, { "start_idx": 101, "end_idx": 103, "type": "bod", "entity": "幽门管" }, { "start_idx": 108, "end_idx": 110, "type": "bod", "entity": "幽门壁" } ] }, { "text": "超声显像诊断本病的标准是:①幽门管直径≥1.3cm幽门壁厚度≥0.4cm幽门肌长度≥1.9cm胃及十二指肠球部溃疡小儿消化性溃疡并不少见,从新生儿到学龄儿童均可发生。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "超声显像" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "幽门管" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "幽门管直径≥1.3cm" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "幽门壁" }, { "start_idx": 25, "end_idx": 35, "type": "sym", "entity": "幽门壁厚度≥0.4cm" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "幽门肌" }, { "start_idx": 36, "end_idx": 46, "type": "sym", "entity": "幽门肌长度≥1.9cm" }, { "start_idx": 47, "end_idx": 56, "type": "dis", "entity": "胃及十二指肠球部溃疡" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "小儿消化性溃疡" } ] }, { "text": "儿童十二指肠球部溃疡较胃溃疡更多见。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "十二指肠球部溃疡" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "胃溃疡" } ] }, { "text": "钡餐检查的主要征象是龛影,胃溃疡常位于胃小弯,龛影周围有放射状黏膜,皱襞向溃疡集中。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "钡餐检查" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "龛影" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "胃溃疡" }, { "start_idx": 17, "end_idx": 40, "type": "sym", "entity": "位于胃小弯,龛影周围有放射状黏膜,皱襞向溃疡集中" } ] }, { "text": "十二指肠溃疡显示除胃溃疡的征象外,还可见球部变形,造影剂通过球部较快。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "十二指肠溃疡" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "胃溃疡" }, { "start_idx": 20, "end_idx": 33, "type": "sym", "entity": "球部变形,造影剂通过球部较快" } ] }, { "text": "必须注意的是继发性溃疡一般表浅,难于在一般X线检查时发现。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "继发性溃疡" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "X线检查" } ] }, { "text": "3.十二指肠梗阻(duodenalobstruction)十二指肠梗阻的原因多为先天性,如十二指肠闭锁和狭窄、十二指肠腔内隔膜狭窄、肠旋转不良以及环状胰腺等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "十二指肠梗阻" }, { "start_idx": 9, "end_idx": 27, "type": "dis", "entity": "duodenalobstruction" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "十二指肠梗阻" }, { "start_idx": 45, "end_idx": 53, "type": "dis", "entity": "十二指肠闭锁和狭窄" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "十二指肠" }, { "start_idx": 59, "end_idx": 64, "type": "sym", "entity": "腔内隔膜狭窄" }, { "start_idx": 66, "end_idx": 70, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 73, "end_idx": 76, "type": "dis", "entity": "环状胰腺" } ] }, { "text": "腹部平片显示胃及十二指肠扩张呈双泡征或三泡征。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "腹部平片" }, { "start_idx": 6, "end_idx": 21, "type": "sym", "entity": "胃及十二指肠扩张呈双泡征或三泡征" } ] }, { "text": "不完全梗阻则梗阻点远端可见充气肠曲。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "不完全梗阻" }, { "start_idx": 6, "end_idx": 16, "type": "sym", "entity": "梗阻点远端可见充气肠曲" } ] }, { "text": "完全梗阻则梗阻点远端肠管不充气。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "完全梗阻" }, { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "梗阻点远端肠管不充气" } ] }, { "text": "先天性肠旋转不良(congenitalintestinalmalrotation)的诊断依靠X线钡餐检查及钡灌肠。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "先天性肠旋转不良" }, { "start_idx": 9, "end_idx": 39, "type": "dis", "entity": "congenitalintestinalmalrotation" }, { "start_idx": 46, "end_idx": 51, "type": "pro", "entity": "X线钡餐检查" }, { "start_idx": 53, "end_idx": 55, "type": "pro", "entity": "钡灌肠" } ] }, { "text": "钡餐检查显示十二指肠降段或水平段狭窄,狭窄以上肠管及胃扩张空肠位于右上腹。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "钡餐检查" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肠管" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "胃" }, { "start_idx": 6, "end_idx": 28, "type": "sym", "entity": "十二指肠降段或水平段狭窄,狭窄以上肠管及胃扩张" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "空肠位于右上腹" } ] }, { "text": "伴肠扭转时,十二指肠及上段空肠沿中腹部呈螺旋形下降。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "伴肠" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "十二指肠" }, { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "伴肠扭转时,十二指肠及上段空肠沿中腹部呈螺旋形下降" } ] }, { "text": "钡灌肠显示盲肠位于右上腹或上腹部,结肠大部在左腹部迂回。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "钡灌肠" }, { "start_idx": 5, "end_idx": 26, "type": "sym", "entity": "盲肠位于右上腹或上腹部,结肠大部在左腹部迂回" } ] }, { "text": "(五)小肠及结肠疾病1.肠套叠肠套叠为婴儿期常见的急腹症,近端肠管套入远端肠管小肠低位梗阻。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "小肠及结肠疾病" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "肠套叠" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肠套叠" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "急腹症" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "远端肠管" }, { "start_idx": 29, "end_idx": 38, "type": "sym", "entity": "近端肠管套入远端肠管" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "小肠低位梗阻" } ] }, { "text": "婴儿肠套叠大多为原发性,可能由多种原因引起的肠蠕动紊乱所致。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肠套叠" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "肠蠕动紊乱" } ] }, { "text": "也可为继发性,见于梅克尔憩室、肠道炎症、过敏性紫癜、息肉及肿瘤等。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "梅克尔憩室" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "肠道炎症" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "过敏性紫癜" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "息肉" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "肿瘤" } ] }, { "text": "临床以阵发性哭吵、呕吐、果酱样大便及腹部包块为特征。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "阵发性哭吵" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "呕吐" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "果酱样大便" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "腹部包块" } ] }, { "text": "腹部平片可见低位小肠梗阻。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "腹部平片" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "低位小肠梗阻" } ] }, { "text": "进一步可作钡灌肠或空气灌肠明确诊断。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "钡灌肠" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "空气灌肠" } ] }, { "text": "钡剂或空气注入后,在套叠处可以看到受阻影像。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "钡剂" } ] }, { "text": "套入肠管的头端呈圆形,透视下钡剂呈螺旋状或杯口形。", "entities": [ { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "套入肠管的头端呈圆形,透视下钡剂呈螺旋状或杯口形" } ] }, { "text": "空气灌肠不如钡剂显示清晰,但可见类似的影像即可得出诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "空气灌肠" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "钡剂" } ] }, { "text": "空气灌肠经济简便,整复较快,减少了X线暴露时间。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "空气灌肠" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "X线" } ] }, { "text": "凡病程在48小时以内,腹部无肠坏死征象者均可采用空气灌肠诊断和治疗。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "腹部无肠坏死" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "空气灌肠" } ] }, { "text": "2.先天性巨结肠先天性巨结肠为婴幼儿较常见的先天性结肠病变。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "先天性巨结肠" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "先天性巨结肠" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "先天性结肠病变" } ] }, { "text": "本病因结肠壁肌间神经节细胞缺如或减少,使病变肠段不能松弛,呈痉挛状态,而近段肠管代偿性扩张和肥厚。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "bod", "entity": "结肠壁肌间神经节细胞" }, { "start_idx": 3, "end_idx": 33, "type": "sym", "entity": "结肠壁肌间神经节细胞缺如或减少,使病变肠段不能松弛,呈痉挛状态" }, { "start_idx": 35, "end_idx": 47, "type": "sym", "entity": "而近段肠管代偿性扩张和肥厚" } ] }, { "text": "一般分型为:①超短段型,病变局限于直肠远端;②短段型,病变位于直肠中段远端,距肛门距离不超过6.5cm;③常见型,病变位于直肠近端或直肠乙状结肠交界处,距肛门约9cm;④长段型,病变延至乙状结肠或降结肠;⑤全结肠型,病变波及全部结肠及回肠,距回盲瓣30cm以内;⑥全肠型,病变波及全部结肠以及回肠30cm以上。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "超短段型" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "病变局限于直肠远端" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "短段型" }, { "start_idx": 31, "end_idx": 36, "type": "bod", "entity": "直肠中段远端" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肛门" }, { "start_idx": 27, "end_idx": 50, "type": "sym", "entity": "病变位于直肠中段远端,距肛门距离不超过6.5cm" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "常见型" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "直肠近端" }, { "start_idx": 66, "end_idx": 71, "type": "bod", "entity": "直肠乙状结肠" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "肛门" }, { "start_idx": 57, "end_idx": 82, "type": "sym", "entity": "病变位于直肠近端或直肠乙状结肠交界处,距肛门约9cm" }, { "start_idx": 85, "end_idx": 87, "type": "dis", "entity": "长段型" }, { "start_idx": 89, "end_idx": 100, "type": "sym", "entity": "病变延至乙状结肠或降结肠" }, { "start_idx": 103, "end_idx": 106, "type": "dis", "entity": "全结肠型" }, { "start_idx": 114, "end_idx": 115, "type": "bod", "entity": "结肠" }, { "start_idx": 117, "end_idx": 118, "type": "bod", "entity": "回肠" }, { "start_idx": 108, "end_idx": 129, "type": "sym", "entity": "病变波及全部结肠及回肠,距回盲瓣30cm以内" }, { "start_idx": 132, "end_idx": 134, "type": "dis", "entity": "全肠型" }, { "start_idx": 136, "end_idx": 153, "type": "sym", "entity": "病变波及全部结肠以及回肠30cm以上" } ] }, { "text": "肛门指诊指肠空虚,肛查后有大量气体和粪便排出。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "肛门指诊" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "指肠" }, { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "指肠空虚" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "肛查" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "粪便" }, { "start_idx": 9, "end_idx": 21, "type": "sym", "entity": "肛查后有大量气体和粪便排出" } ] }, { "text": "(1)腹部平片:显示低位肠梗阻,结肠与小肠有不同程度扩张,有时见液平钡剂灌肠:是很有价值的确诊方法。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "腹部平片" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "低位肠梗阻" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "结肠" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "小肠" }, { "start_idx": 8, "end_idx": 33, "type": "sym", "entity": "显示低位肠梗阻,结肠与小肠有不同程度扩张,有时见液平" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "钡剂灌肠" } ] }, { "text": "典型表现显示痉挛段、移行段与扩张段三部分。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "扩张段" } ] }, { "text": "①痉挛段:为长短不一的狭窄肠段,边缘呈不规则花边状、锯齿状;②移行段:位于痉挛段的近端,多呈漏斗状;③扩张段:移行段近端肠管明显扩张,黏膜粗厚。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "sym", "entity": "痉挛段" }, { "start_idx": 5, "end_idx": 28, "type": "sym", "entity": "为长短不一的狭窄肠段,边缘呈不规则花边状、锯齿状" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "移行段" }, { "start_idx": 35, "end_idx": 48, "type": "sym", "entity": "位于痉挛段的近端,多呈漏斗状" }, { "start_idx": 51, "end_idx": 53, "type": "sym", "entity": "扩张段" }, { "start_idx": 55, "end_idx": 70, "type": "sym", "entity": "移行段近端肠管明显扩张,黏膜粗厚" } ] }, { "text": "注意事项:①钡剂灌肠前不作清洁灌肠,尤其对新生儿,以免影响扩张段显示;②注钡肛管宜用细导尿管,且不能放置过深;③注钡压力不能过高;④摄正侧位片;⑤如遇不能确诊者,24小时后重复透视。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "钡剂灌肠" }, { "start_idx": 36, "end_idx": 39, "type": "equ", "entity": "注钡肛管" }, { "start_idx": 42, "end_idx": 45, "type": "equ", "entity": "细导尿管" }, { "start_idx": 66, "end_idx": 70, "type": "pro", "entity": "摄正侧位片" } ] }, { "text": "3.结肠息肉在良性肠肿瘤中,以结肠息肉多见。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "结肠息肉" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "良性肠肿瘤" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "结肠息肉" } ] }, { "text": "息肉可分为三类:①幼年性息肉:此型最多见,95%为单发,多见于直肠或乙状结肠家族性腺瘤病:有遗传性,息肉为多发性;③Peutz-Jephens综合征:小肠、结肠多发性息肉,同时面部皮肤、唇及口腔黏膜色素沉着钡餐造影显示圆形或椭圆形、大小不等的充盈缺损。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "幼年性息肉" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "直肠" }, { "start_idx": 15, "end_idx": 37, "type": "sym", "entity": "此型最多见,95%为单发,多见于直肠或乙状结肠" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "家族性腺瘤病" }, { "start_idx": 45, "end_idx": 55, "type": "sym", "entity": "有遗传性,息肉为多发性" }, { "start_idx": 58, "end_idx": 73, "type": "dis", "entity": "Peutz-Jephens综合征" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "小肠" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "结肠" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "皮肤" }, { "start_idx": 93, "end_idx": 93, "type": "bod", "entity": "唇" }, { "start_idx": 95, "end_idx": 98, "type": "bod", "entity": "口腔黏膜" }, { "start_idx": 75, "end_idx": 102, "type": "sym", "entity": "小肠、结肠多发性息肉,同时面部皮肤、唇及口腔黏膜色素沉着" }, { "start_idx": 103, "end_idx": 106, "type": "pro", "entity": "钡餐造影" }, { "start_idx": 109, "end_idx": 124, "type": "sym", "entity": "圆形或椭圆形、大小不等的充盈缺损" } ] }, { "text": "气钡双重造影显示钡剂涂布息肉表层显示完整或呈波浪状的边缘。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "气钡双重造影" }, { "start_idx": 8, "end_idx": 27, "type": "sym", "entity": "钡剂涂布息肉表层显示完整或呈波浪状的边缘" } ] }, { "text": "(六)肝、胆、胰1.肝脓肿(hepaticabscess)可以是阿米巴性或细菌性。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肝" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "胆" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "胰" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肝脓肿" }, { "start_idx": 14, "end_idx": 27, "type": "bod", "entity": "hepaticabscess" }, { "start_idx": 32, "end_idx": 35, "type": "mic", "entity": "阿米巴性" }, { "start_idx": 37, "end_idx": 39, "type": "mic", "entity": "细菌性" } ] }, { "text": "脓肿可单发或多发,多见于肝右叶。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "脓肿" }, { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "单发或多发,多见于肝右叶" } ] }, { "text": "临床上,主要表现为寒战、高热或弛张热、右季肋部钝痛,胆增大并有明显压痛。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "寒战" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "高热" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "弛张热" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "右季肋部钝痛" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "胆增大并有明显压痛" } ] }, { "text": "影像检查:①超声检查是诊断肝脓肿的首选方法。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "超声检查" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肝脓肿" } ] }, { "text": "典型声像图表现为液性暗区,移动探头可显示脓肿呈球形。", "entities": [ { "start_idx": 2, "end_idx": 24, "type": "sym", "entity": "声像图表现为液性暗区,移动探头可显示脓肿呈球形" } ] }, { "text": "还可见坏死组织等形成的点状或线状回声。", "entities": [ { "start_idx": 1, "end_idx": 17, "type": "sym", "entity": "可见坏死组织等形成的点状或线状回声" } ] }, { "text": "脓肿边缘较厚,内有散在细小光点。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "脓肿边缘较厚,内有散在细小光点" } ] }, { "text": "②X线检查可见膈肌升高、运动减弱,可有轻微胸膜反应使肋膈角或有少量胸腔积液在肝区可见含气或含液的空腔先天性胆总管囊肿(congenitalcholedochocyst)先天性胆总管囊肿为常见的一种先天性胆道囊性扩张症,男与女的比例大约为1∶4。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "X线检查" }, { "start_idx": 5, "end_idx": 15, "type": "sym", "entity": "可见膈肌升高、运动减弱" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "胸腔积液" }, { "start_idx": 17, "end_idx": 36, "type": "sym", "entity": "可有轻微胸膜反应使肋膈角或有少量胸腔积液" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "肝" }, { "start_idx": 37, "end_idx": 49, "type": "sym", "entity": "在肝区可见含气或含液的空腔" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "先天性胆总管囊肿" }, { "start_idx": 59, "end_idx": 82, "type": "dis", "entity": "congenitalcholedochocyst" }, { "start_idx": 84, "end_idx": 91, "type": "dis", "entity": "先天性胆总管囊肿" }, { "start_idx": 98, "end_idx": 107, "type": "dis", "entity": "先天性胆道囊性扩张症" } ] }, { "text": "本病的病因多数认为与先天性胆道或胰胆管发育异常有关。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "先天性胆道" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "胰胆管发育异常" } ] }, { "text": "腹痛、黄疸及腹部肿块为本病的三个典型症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "腹痛" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "黄疸" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "腹部肿块" } ] }, { "text": "B超检查:肝脏下方显示界限清楚的低回声区,并可查明肝内胆管扩张的程度和范围X线检查:①腹部平片:胆总管囊肿较大时,于右上腹可见密度均匀的、与肝影相连的软组织样肿块并可见胃及结肠被推移钡餐检查:可见胃窦部推向左上方,十二指肠段向右前移十二指肠环增大。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "B超检查" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肝脏" }, { "start_idx": 5, "end_idx": 36, "type": "sym", "entity": "肝脏下方显示界限清楚的低回声区,并可查明肝内胆管扩张的程度和范围" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "X线检查" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "腹部平片" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "胆总管囊肿" }, { "start_idx": 48, "end_idx": 80, "type": "sym", "entity": "胆总管囊肿较大时,于右上腹可见密度均匀的、与肝影相连的软组织样肿块" }, { "start_idx": 84, "end_idx": 84, "type": "bod", "entity": "胃" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "结肠" }, { "start_idx": 81, "end_idx": 90, "type": "sym", "entity": "并可见胃及结肠被推移" }, { "start_idx": 91, "end_idx": 94, "type": "pro", "entity": "钡餐检查" }, { "start_idx": 98, "end_idx": 100, "type": "bod", "entity": "胃窦部" }, { "start_idx": 107, "end_idx": 110, "type": "bod", "entity": "十二指肠" }, { "start_idx": 96, "end_idx": 115, "type": "sym", "entity": "可见胃窦部推向左上方,十二指肠段向右前移" }, { "start_idx": 116, "end_idx": 122, "type": "sym", "entity": "十二指肠环增大" } ] }, { "text": "③CT检查:CT可明确胆总管囊肿的大小,胆总管远端狭窄的程度,以及胆囊及肝管的解剖等,有助于手术方式的选择。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "CT检查" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "CT" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "胆总管囊肿" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "胆总管" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "胆囊" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肝管" } ] }, { "text": "3.胰腺炎小儿胰腺炎少见,多为急性胰腺炎。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "急性胰腺炎" } ] }, { "text": "临床表现多为剧烈腹痛,伴恶心、呕吐及发热等。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "剧烈腹痛" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "伴恶心、呕吐及发热等" } ] }, { "text": "B超:显示胰腺体积增大,密度降低,并可确定有无假性囊肿的存在。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "B超" }, { "start_idx": 3, "end_idx": 29, "type": "sym", "entity": "显示胰腺体积增大,密度降低,并可确定有无假性囊肿的存在" } ] }, { "text": "腹部平片:胃、横结肠、十二指肠和上部空肠扩张、积气,并可见“横结肠截断征CT:显示胰腺普遍增大,密度降低,胰腺轮廓不清慢性胰腺炎可见胰实质钙化。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "腹部平片" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "胃" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "横结肠" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "十二指肠" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "上部空肠" }, { "start_idx": 5, "end_idx": 35, "type": "sym", "entity": "胃、横结肠、十二指肠和上部空肠扩张、积气,并可见“横结肠截断征" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "CT" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "胰腺" }, { "start_idx": 41, "end_idx": 58, "type": "sym", "entity": "胰腺普遍增大,密度降低,胰腺轮廓不清" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "慢性胰腺炎" }, { "start_idx": 66, "end_idx": 70, "type": "sym", "entity": "胰实质钙化" } ] }, { "text": "五、双气囊小肠镜2001年,日本学者Yamamoto在世界上率先报道了使用推进式双气囊小肠镜(double-balloonenteroscopy,DBE)进行全小肠检查。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "equ", "entity": "双气囊小肠镜" }, { "start_idx": 40, "end_idx": 45, "type": "equ", "entity": "双气囊小肠镜" }, { "start_idx": 47, "end_idx": 71, "type": "equ", "entity": "double-balloonenteroscopy" }, { "start_idx": 73, "end_idx": 75, "type": "equ", "entity": "DBE" }, { "start_idx": 80, "end_idx": 83, "type": "pro", "entity": "小肠检查" } ] }, { "text": "推进式双气囊小肠镜是在原先的推进式小肠镜外加上一个顶端带气囊的外套管,同时也在小肠镜顶端加装一个气囊。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "equ", "entity": "推进式双气囊小肠镜" }, { "start_idx": 14, "end_idx": 19, "type": "equ", "entity": "推进式小肠镜" }, { "start_idx": 31, "end_idx": 33, "type": "equ", "entity": "外套管" }, { "start_idx": 39, "end_idx": 41, "type": "equ", "entity": "小肠镜" }, { "start_idx": 48, "end_idx": 49, "type": "equ", "entity": "气囊" } ] }, { "text": "双气囊小肠镜的问世与应用,使消化内镜对消化道检查已拓展至主深部小肠,原先无法抵达的部位和不能直接检出的情况发生了根本性变化,上下结合方式的双气囊小肠镜检查基本完成了对整个消化道的彻底无盲区的检查。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "equ", "entity": "双气囊小肠镜" }, { "start_idx": 14, "end_idx": 17, "type": "equ", "entity": "消化内镜" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "消化道检查" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "主深部小肠" }, { "start_idx": 69, "end_idx": 76, "type": "pro", "entity": "双气囊小肠镜检查" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "消化道" } ] }, { "text": "除了具有检查范围广的优势外,双气囊小肠镜还具有普通电子内镜的共同优点:图像清晰,操作可控制,能取活检等特点,使病变性质得以明确。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "equ", "entity": "双气囊小肠镜" } ] }, { "text": "双气囊小肠镜不仅可直视检查整个小肠的病变情况,而且可对病灶进行活检和治疗,该技术的应用,为小肠疾病检查提供了全新的方法。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "equ", "entity": "双气囊小肠镜" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "小肠" }, { "start_idx": 45, "end_idx": 50, "type": "pro", "entity": "小肠疾病检查" } ] }, { "text": "四、先天性心脏病血管堵塞术以往异常血管通道的外科治疗,是通过切除含有小的血管瘤的病理组织,结扎异常血管通道或直视切开心腔进行修补缺损达到治疗目的。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "先天性心脏病血管堵塞术" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "血管" }, { "start_idx": 22, "end_idx": 23, "type": "dep", "entity": "外科" }, { "start_idx": 30, "end_idx": 43, "type": "pro", "entity": "切除含有小的血管瘤的病理组织" }, { "start_idx": 45, "end_idx": 52, "type": "pro", "entity": "结扎异常血管通道" }, { "start_idx": 54, "end_idx": 65, "type": "pro", "entity": "直视切开心腔进行修补缺损" } ] }, { "text": "而经导管堵塞术,则是将特殊的堵塞材料经递送导管达特定的位置,达到与外科手术同样的治疗目的,从而免除了外科手术。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "经导管堵塞术" }, { "start_idx": 14, "end_idx": 17, "type": "equ", "entity": "堵塞材料" }, { "start_idx": 19, "end_idx": 22, "type": "equ", "entity": "递送导管" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "外科手术" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "外科手术" } ] }, { "text": "常用的治疗方法有:①微血管栓塞法,如对于肺小动静脉瘘(arteriovenousfistula),可应用明胶海绵、Ivalon碎片等对整个肺小动静脉瘘进行栓塞,达到与外科进行肺叶或肺段切除同样的作用,但血管堵塞法可尽量保持正常的肺组织及肺功能,使治疗更符合生理要求;②堵塞远端血管或动静脉畸形的上流血管,如主动脉-肺侧支循环、肺动静脉畸形、冠状动静脉瘘等,常采用弹簧圈,可脱卸球囊等达到堵断异常血管的目的;③关闭心内外异常缺损或粗的异常血管通道,如动脉导管未闭、冠状动静脉瘘、左上腔静脉入左心房等需要借助于特种堵塞装置,如多种类型伞状堵塞装置等。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "微血管栓塞法" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "肺小动静脉瘘" }, { "start_idx": 27, "end_idx": 46, "type": "dis", "entity": "arteriovenousfistula" }, { "start_idx": 52, "end_idx": 55, "type": "equ", "entity": "明胶海绵" }, { "start_idx": 57, "end_idx": 64, "type": "equ", "entity": "Ivalon碎片" }, { "start_idx": 69, "end_idx": 74, "type": "dis", "entity": "肺小动静脉瘘" }, { "start_idx": 77, "end_idx": 78, "type": "pro", "entity": "栓塞" }, { "start_idx": 83, "end_idx": 84, "type": "dep", "entity": "外科" }, { "start_idx": 87, "end_idx": 93, "type": "pro", "entity": "肺叶或肺段切除" }, { "start_idx": 101, "end_idx": 105, "type": "pro", "entity": "血管堵塞法" }, { "start_idx": 114, "end_idx": 116, "type": "bod", "entity": "肺组织" }, { "start_idx": 118, "end_idx": 118, "type": "bod", "entity": "肺" }, { "start_idx": 134, "end_idx": 150, "type": "pro", "entity": "堵塞远端血管或动静脉畸形的上流血管" }, { "start_idx": 153, "end_idx": 161, "type": "dis", "entity": "主动脉-肺侧支循环" }, { "start_idx": 163, "end_idx": 168, "type": "dis", "entity": "肺动静脉畸形" }, { "start_idx": 170, "end_idx": 175, "type": "dis", "entity": "冠状动静脉瘘" }, { "start_idx": 181, "end_idx": 183, "type": "equ", "entity": "弹簧圈" }, { "start_idx": 186, "end_idx": 189, "type": "pro", "entity": "脱卸球囊" }, { "start_idx": 197, "end_idx": 198, "type": "bod", "entity": "血管" }, { "start_idx": 204, "end_idx": 221, "type": "pro", "entity": "关闭心内外异常缺损或粗的异常血管通道" }, { "start_idx": 224, "end_idx": 229, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 231, "end_idx": 236, "type": "dis", "entity": "冠状动静脉瘘" }, { "start_idx": 238, "end_idx": 246, "type": "dis", "entity": "左上腔静脉入左心房" }, { "start_idx": 255, "end_idx": 258, "type": "equ", "entity": "堵塞装置" }, { "start_idx": 261, "end_idx": 270, "type": "equ", "entity": "多种类型伞状堵塞装置" } ] }, { "text": "本节介绍目前广泛应用的经导管动脉导管未闭堵塞术。", "entities": [ { "start_idx": 11, "end_idx": 22, "type": "pro", "entity": "经导管动脉导管未闭堵塞术" } ] }, { "text": "虽然Porstmann早在1967年就首次应用海绵塞法成功堵塞动脉导管未闭,但由于该法操作复杂、并发症多,且仅限用于年长儿及成人,因此目前已很少应用。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "海绵塞法" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "动脉导管未闭" } ] }, { "text": "此后Rashkind设计的双盘堵塞装置,包括其改良型,如蚌壳状关闭式(Lock)、纽扣式双盘装置(Sideris)等,因术后残余分流发生率高、需要较粗的递送导管,较难适用于小婴儿及过小的或过大的动脉导管未闭。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "equ", "entity": "双盘堵塞装置" }, { "start_idx": 28, "end_idx": 33, "type": "equ", "entity": "蚌壳状关闭式" }, { "start_idx": 35, "end_idx": 38, "type": "equ", "entity": "Lock" }, { "start_idx": 41, "end_idx": 47, "type": "equ", "entity": "纽扣式双盘装置" }, { "start_idx": 49, "end_idx": 55, "type": "equ", "entity": "Sideris" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "残余分流" }, { "start_idx": 76, "end_idx": 79, "type": "equ", "entity": "递送导管" }, { "start_idx": 97, "end_idx": 102, "type": "dis", "entity": "动脉导管未闭" } ] }, { "text": "因此,在20世纪90年代之前,经导管堵塞动脉导管未闭在国内外未获推广应用。", "entities": [ { "start_idx": 15, "end_idx": 25, "type": "pro", "entity": "经导管堵塞动脉导管未闭" } ] }, { "text": "1992年,Combiel等首先报道应用弹簧圈(coil)堵塞小型动脉导管未闭获得成功,上海第二医科大学附属新华医院也在国内最早报告用该法堵塞动脉导管未闭。", "entities": [ { "start_idx": 18, "end_idx": 38, "type": "pro", "entity": "应用弹簧圈(coil)堵塞小型动脉导管未闭" }, { "start_idx": 69, "end_idx": 76, "type": "pro", "entity": "堵塞动脉导管未闭" } ] }, { "text": "由于弹簧圈堵塞术具有操作简便、疗效好、递送导管细、损伤小及可用于小婴儿等优点,深受使用者欢迎。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "弹簧圈堵塞术" }, { "start_idx": 19, "end_idx": 22, "type": "equ", "entity": "递送导管" } ] }, { "text": "但对于中等以上的动脉导管未闭动脉导管未闭仍无合适的堵塞装置。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 25, "end_idx": 28, "type": "equ", "entity": "堵塞装置" } ] }, { "text": "1998年,Masura等报道应用自膨性蘑菇伞堵塞装置堵塞动脉导管未闭。", "entities": [ { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "动脉导管未闭" } ] }, { "text": "该法安全、简便、几乎无残余分流,可应用于中、大型动脉导管未闭,使动脉导管未闭的介入治疗获得突破性进展。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "无残余分流" }, { "start_idx": 20, "end_idx": 29, "type": "dis", "entity": "中、大型动脉导管未闭" }, { "start_idx": 32, "end_idx": 42, "type": "pro", "entity": "动脉导管未闭的介入治疗" } ] }, { "text": "目前,根据作者的经验及文献综述,弹簧圈堵塞术及自膨性蘑菇伞堵塞术的合理选择、操作方法规范并掌握一定的要点,几乎所有类型的动脉导管未闭(包括婴幼儿及合并肺动脉高压者)均能有效经导管关闭。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "弹簧圈堵塞术" }, { "start_idx": 23, "end_idx": 31, "type": "pro", "entity": "自膨性蘑菇伞堵塞术" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 75, "end_idx": 79, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 86, "end_idx": 90, "type": "pro", "entity": "经导管关闭" } ] }, { "text": "(一)经导管弹簧圈动脉导管未闭堵塞术1.指征目前主要适应证为:直径≤2.5mm的动脉导管未闭,未经手术或外科手术后残余分流者,适合的解剖类型主要为管型或漏斗型。", "entities": [ { "start_idx": 3, "end_idx": 17, "type": "pro", "entity": "经导管弹簧圈动脉导管未闭堵塞术" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "手术" }, { "start_idx": 52, "end_idx": 55, "type": "pro", "entity": "外科手术" } ] }, { "text": "2.方法经皮股动静脉插管,分别测压和血氧测定,估价分流严重性。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "pro", "entity": "经皮股动静脉插管" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "测压" }, { "start_idx": 18, "end_idx": 21, "type": "ite", "entity": "血氧测定" } ] }, { "text": "导管置于升主动脉进行左侧位造影,观察动脉导管大小,形态及走向,并排除其他心血管畸形,精确测量动脉导管最狭窄处直径。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "导管" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "升主动脉" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "左侧位造影" }, { "start_idx": 18, "end_idx": 21, "type": "equ", "entity": "动脉导管" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "心血管畸形" }, { "start_idx": 46, "end_idx": 49, "type": "equ", "entity": "动脉导管" } ] }, { "text": "pfm公司的双螺旋双圆锥弹簧圈及Cook公司的管形弹簧圈操作方法有所不同,但基本原则相似,有经股静脉顺向法及经股动脉逆向法。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "equ", "entity": "双螺旋双圆锥弹簧圈" }, { "start_idx": 23, "end_idx": 27, "type": "equ", "entity": "管形弹簧圈" }, { "start_idx": 46, "end_idx": 52, "type": "pro", "entity": "经股静脉顺向法" }, { "start_idx": 54, "end_idx": 60, "type": "pro", "entity": "经股动脉逆向法" } ] }, { "text": "3.并发症及其处理弹簧圈释放后可产生残余分流,如释放后尚未脱钩前如有中等分流时需再增加一个弹簧圈,如仅为极少量分流,随访观察表明大部分在短期内消失。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "equ", "entity": "弹簧圈" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "残余分流" }, { "start_idx": 45, "end_idx": 47, "type": "equ", "entity": "弹簧圈" } ] }, { "text": "少量病例发生溶血,多是由于较明显残余分流引起红细胞破坏所致。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "溶血" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "残余分流" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "红细胞" } ] }, { "text": "经短期内科对症处理及密切观察下无效,超声检查示左向右分流明显,需再次增加安置弹簧圈或作外科手术处理。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dep", "entity": "内科" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "超声检查" }, { "start_idx": 38, "end_idx": 40, "type": "equ", "entity": "弹簧圈" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "外科手术" } ] }, { "text": "弹簧圈脱落常由于选择太小或操作不当引起,常向肺动脉方向至肺,可经肺动脉插入圈套装置将弹簧圈取出。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "弹簧圈" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肺动脉" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肺" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "肺动脉" }, { "start_idx": 37, "end_idx": 40, "type": "equ", "entity": "圈套装置" }, { "start_idx": 42, "end_idx": 44, "type": "equ", "entity": "弹簧圈" } ] }, { "text": "根据上海第二医科大学附属新华医院应用经验,经导管弹簧圈动脉导管未闭堵塞术具有操作简便、疗效好、递送导管细、损伤小及可用于小婴儿等优点,特别适用于直径≤2mm的动脉导管未闭。", "entities": [ { "start_idx": 21, "end_idx": 35, "type": "pro", "entity": "经导管弹簧圈动脉导管未闭堵塞术" }, { "start_idx": 47, "end_idx": 50, "type": "equ", "entity": "递送导管" }, { "start_idx": 72, "end_idx": 84, "type": "dis", "entity": "直径≤2mm的动脉导管未闭" } ] }, { "text": "但对于>2mm的动脉导管未闭,还是推荐应用下面将叙述的自膨性蘑菇伞堵塞装置堵塞。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": ">2mm的动脉导管未闭" }, { "start_idx": 27, "end_idx": 36, "type": "equ", "entity": "自膨性蘑菇伞堵塞装置" } ] }, { "text": "(二)经导管自膨性蘑菇伞动脉导管未闭堵塞术1.适应证及禁忌证(1)适应证:直径>2.5mm、位置正常的动脉导管未闭,不管形状及动脉导管未闭大小,血流动力学监测无器质性肺高压者均为适应症,病人的体重通常应>5kg。", "entities": [ { "start_idx": 3, "end_idx": 20, "type": "pro", "entity": "经导管自膨性蘑菇伞动脉导管未闭堵塞术" }, { "start_idx": 37, "end_idx": 56, "type": "sym", "entity": "直径>2.5mm、位置正常的动脉导管未闭" }, { "start_idx": 63, "end_idx": 68, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 72, "end_idx": 76, "type": "ite", "entity": "血流动力学" }, { "start_idx": 80, "end_idx": 85, "type": "dis", "entity": "器质性肺高压" }, { "start_idx": 96, "end_idx": 104, "type": "sym", "entity": "体重通常应>5kg" } ] }, { "text": "(2)禁忌证:①重症肺动脉高压,经规范方法(压力、阻力、肺小动脉造影及堵塞试验)评价为器质性肺高压或临界病例者;②伴有需胸外手术的先天性心脏畸形;③体重<5kg。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "重症肺动脉高压" }, { "start_idx": 28, "end_idx": 33, "type": "pro", "entity": "肺小动脉造影" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "堵塞试验" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "器质性肺高压" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "临界病例" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "胸外手术" }, { "start_idx": 65, "end_idx": 71, "type": "dis", "entity": "先天性心脏畸形" }, { "start_idx": 74, "end_idx": 79, "type": "sym", "entity": "体重<5kg" } ] }, { "text": "2.方法经股静脉方法,将引导鞘自肺动脉通过动脉导管未闭定位于降主动脉。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "经股静脉方法" }, { "start_idx": 12, "end_idx": 14, "type": "equ", "entity": "引导鞘" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "肺动脉" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "降主动脉" } ] }, { "text": "选取比动脉导管未闭最狭窄处直径大3~6mm的堵塞装置。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 22, "end_idx": 25, "type": "equ", "entity": "堵塞装置" } ] }, { "text": "释放钢丝穿过装载器,其头端与封堵器螺纹相接。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "equ", "entity": "钢丝" }, { "start_idx": 6, "end_idx": 8, "type": "equ", "entity": "装载器" }, { "start_idx": 14, "end_idx": 15, "type": "equ", "entity": "封堵" } ] }, { "text": "封堵装置与装载器一起浸于盐水中,然后装置被拉入装载器。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "equ", "entity": "封堵装置" }, { "start_idx": 5, "end_idx": 7, "type": "equ", "entity": "装载器" }, { "start_idx": 18, "end_idx": 19, "type": "equ", "entity": "装置" }, { "start_idx": 23, "end_idx": 25, "type": "equ", "entity": "装载器" } ] }, { "text": "装载器与引导鞘对接,装置由释放钢丝推送至降主动脉(推送过程中不能旋转)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "装载器" }, { "start_idx": 4, "end_idx": 6, "type": "equ", "entity": "引导鞘" }, { "start_idx": 10, "end_idx": 11, "type": "equ", "entity": "装置" }, { "start_idx": 15, "end_idx": 16, "type": "equ", "entity": "钢丝" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "降主动脉" } ] }, { "text": "滞留盘放出,然后拉向动脉导管未闭口,这可通过透视、或可感到与主动脉搏动同步的拖拉感。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "滞留盘" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "主动脉" } ] }, { "text": "引导鞘后撤,装置的圆柱形部分便安全展开于动脉导管未闭内。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "引导鞘" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "动脉导管未闭" } ] }, { "text": "再行主动脉造影以确定装置位置正确,如不满意可将装置回收入鞘内重新定位。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "主动脉造影" }, { "start_idx": 10, "end_idx": 11, "type": "equ", "entity": "装置" }, { "start_idx": 23, "end_idx": 24, "type": "equ", "entity": "装置" } ] }, { "text": "一旦证实装置的位置正确,可逆向旋转释放钢丝释放封堵装置。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "equ", "entity": "装置" }, { "start_idx": 19, "end_idx": 20, "type": "equ", "entity": "钢丝" }, { "start_idx": 23, "end_idx": 26, "type": "equ", "entity": "封堵装置" } ] }, { "text": "3.疗效评价作者所在单位近200余例自膨性蘑菇伞动脉导管未闭堵塞术的临床应用显示技术成功率100%,术后即刻残余分流<5%,1月后均完全封堵,无溶血、异位等并发症,与国外的报道类似,因而认为本方法安全、有效、操作方便、适应证广,值得推广,但其中长期疗效尚需进一步随访研究。", "entities": [ { "start_idx": 18, "end_idx": 32, "type": "pro", "entity": "自膨性蘑菇伞动脉导管未闭堵塞术" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "即刻残余分流" }, { "start_idx": 72, "end_idx": 73, "type": "dis", "entity": "溶血" }, { "start_idx": 75, "end_idx": 76, "type": "dis", "entity": "异位" } ] }, { "text": "4.并发症及处理残余分流:通常术后可存在少量残余分流,随访时不久即消失。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "残余分流" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "残余分流" } ] }, { "text": "但对于明显残余分流,多由于堵塞装置太小或移位,一些病例发生溶血,常于术后数小时至10余小时,呈典型溶血表现,需及时内科处理,无效时需要再加弹簧圈堵塞或外科手术。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "残余分流" }, { "start_idx": 13, "end_idx": 16, "type": "equ", "entity": "堵塞装置" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "溶血" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "溶血" }, { "start_idx": 57, "end_idx": 58, "type": "dep", "entity": "内科" }, { "start_idx": 69, "end_idx": 71, "type": "equ", "entity": "弹簧圈" }, { "start_idx": 75, "end_idx": 78, "type": "pro", "entity": "外科手术" } ] }, { "text": "偶见堵塞装置脱落,多由于操作不当或选择堵塞装置太小所致,可用异物钳抓取,必要时需外科手术。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "equ", "entity": "堵塞装置" }, { "start_idx": 19, "end_idx": 22, "type": "equ", "entity": "堵塞装置" }, { "start_idx": 30, "end_idx": 32, "type": "equ", "entity": "异物钳" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "外科手术" } ] }, { "text": "五、腹膜平衡试验和透析充分性对于慢性肾衰竭的长期腹膜透析,必须考虑患儿的透析效果,并且最大限度地保护腹膜功能,腹膜平衡试验和透析充分性是两个重要部分。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "腹膜平衡试验" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "透析" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "慢性肾衰竭" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "透析" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "腹膜" }, { "start_idx": 55, "end_idx": 60, "type": "pro", "entity": "腹膜平衡试验" }, { "start_idx": 62, "end_idx": 63, "type": "pro", "entity": "透析" } ] }, { "text": "(一)腹膜平衡试验由于每个患者的腹膜对溶质转运能力各异,因此腹透疗效亦不同。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "腹膜平衡试验" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "腹膜" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "腹透" } ] }, { "text": "腹膜平衡试验(peritonealequilibrationtest,PET)是通过透析液灌入腹腔4小时中,观察腹透液葡萄糖浓度的变化,以及透析液肌酐与血肌酐比值的变化,来了解每位患者腹膜对葡萄糖的吸收能力,对肌酐的清除情况以及对水的超滤能力。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "腹膜平衡试验" }, { "start_idx": 7, "end_idx": 33, "type": "pro", "entity": "peritonealequilibrationtest" }, { "start_idx": 35, "end_idx": 37, "type": "pro", "entity": "PET" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "透析液" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "腹腔" }, { "start_idx": 56, "end_idx": 58, "type": "dru", "entity": "腹透液" }, { "start_idx": 59, "end_idx": 61, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 76, "end_idx": 78, "type": "dru", "entity": "血肌酐" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "腹膜" }, { "start_idx": 95, "end_idx": 97, "type": "dru", "entity": "葡萄糖" } ] }, { "text": "并根据腹膜对溶质转运个体差异来协助患者制定出合理的腹膜透析处方。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "腹膜" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "腹膜透析" } ] }, { "text": "因此PET是作为腹透患者常规、定期评价腹膜功能的工具。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "PET" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "腹透" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "腹膜" } ] }, { "text": "具体操作方法是:①试验前腹透液留置腹腔内8~12小时;②晨起采取立位或坐位,将腹腔内透析液完全排空,记录超滤量;③取平卧位,用2.5%葡萄糖透析液,按50ml/kg透析量在10min内均匀缓慢注入腹腔,并每2min做一次翻身动作,在10min灌完后计时为0小时;④透析液留置在腹腔4小时后,采用坐位或立位彻底排空腹透液记录超滤量;⑤分别在0、2、4小时留取透析液标本,2小时抽血2ml,所有标本送检测肌酐和葡萄糖;⑥计算腹膜对肌酐的清除(D/Pcr)及腹膜对葡萄糖吸收(D/Do)。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "腹透液" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "腹腔" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "腹腔" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "透析液" }, { "start_idx": 52, "end_idx": 54, "type": "ite", "entity": "超滤量" }, { "start_idx": 67, "end_idx": 72, "type": "dru", "entity": "葡萄糖透析液" }, { "start_idx": 98, "end_idx": 99, "type": "bod", "entity": "腹腔" }, { "start_idx": 132, "end_idx": 134, "type": "dru", "entity": "透析液" }, { "start_idx": 138, "end_idx": 139, "type": "bod", "entity": "腹腔" }, { "start_idx": 156, "end_idx": 158, "type": "dru", "entity": "腹透液" }, { "start_idx": 161, "end_idx": 163, "type": "ite", "entity": "超滤量" }, { "start_idx": 178, "end_idx": 180, "type": "dru", "entity": "透析液" }, { "start_idx": 188, "end_idx": 188, "type": "ite", "entity": "血" }, { "start_idx": 200, "end_idx": 201, "type": "ite", "entity": "肌酐" }, { "start_idx": 203, "end_idx": 205, "type": "ite", "entity": "葡萄糖" }, { "start_idx": 210, "end_idx": 211, "type": "bod", "entity": "腹膜" }, { "start_idx": 213, "end_idx": 214, "type": "ite", "entity": "肌酐" }, { "start_idx": 219, "end_idx": 223, "type": "ite", "entity": "D/Pcr" }, { "start_idx": 226, "end_idx": 227, "type": "bod", "entity": "腹膜" }, { "start_idx": 229, "end_idx": 231, "type": "ite", "entity": "葡萄糖" }, { "start_idx": 235, "end_idx": 238, "type": "ite", "entity": "D/Do" } ] }, { "text": "儿童PET的结果与成年腹透患者的PET比较,证明了与儿童的腹膜表面积大有关,而且这样特征伴随年龄越小更突出。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "PET" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "腹透" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "PET" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "腹膜" } ] }, { "text": "PET试验的结果证实,腹膜对肌酐的清除能力与腹膜对葡萄糖转运呈正相关,而超滤量与肌酐清除呈负相关。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "PET试验" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "腹膜" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肌酐" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "腹膜" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "超滤量" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "肌酐" } ] }, { "text": "由于腹膜表面大同时也增加了有效滤过面积,抵消了腹膜对葡萄糖吸收率高引起超滤下降,因此认为对于儿童腹透患者使用低渗透析液仍可得到满意的超滤效果。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "腹膜" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "腹膜" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "腹透" }, { "start_idx": 55, "end_idx": 58, "type": "dru", "entity": "渗透析液" } ] }, { "text": "(二)透析充分性腹膜透析作为肾脏替代疗法起到了清除毒素和保持体内水平衡作用,但由于是肾脏替代治疗,并不能完全替代正常人体肾脏功能,因此透析只能使尿毒症症状减轻,随着残余肾功能丧失,腹膜透析充分与否与腹透患儿的营养状态、生长发育及生存率密切相关。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "透析" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "肾脏替代疗法" }, { "start_idx": 25, "end_idx": 26, "type": "mic", "entity": "毒素" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "水" }, { "start_idx": 42, "end_idx": 47, "type": "pro", "entity": "肾脏替代治疗" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "肾脏" }, { "start_idx": 67, "end_idx": 68, "type": "pro", "entity": "透析" }, { "start_idx": 72, "end_idx": 74, "type": "dis", "entity": "尿毒症" }, { "start_idx": 84, "end_idx": 84, "type": "bod", "entity": "肾" }, { "start_idx": 90, "end_idx": 93, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 99, "end_idx": 100, "type": "pro", "entity": "腹透" } ] }, { "text": "评估儿童腹膜透析充分性包括:1.充分透析剂量通常需要50ml/kg或1100ml/m2,每日4~5次,此透析液量才能使人体每天代谢产物的产出达到平衡,同时由于儿童与成年人比较处于高代谢状态,给予充分透析剂量下监测尿素氮水平,BUN应<50mg/dl(17.86mmol/L)。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "透析" }, { "start_idx": 58, "end_idx": 60, "type": "dru", "entity": "透析液" }, { "start_idx": 112, "end_idx": 114, "type": "ite", "entity": "尿素氮" } ] }, { "text": "2.透析充分指标(1)KT/Vurea:KT/Vurea又称尿素清除指数,是指单位时间内机体对尿素的清除量,包括腹膜和残余肾单位,K为尿液和腹透液中尿素的清除量,T为每周透析天数,V为尿素的分布容积。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "透析" }, { "start_idx": 11, "end_idx": 18, "type": "ite", "entity": "KT/Vurea" }, { "start_idx": 20, "end_idx": 27, "type": "ite", "entity": "KT/Vurea" }, { "start_idx": 30, "end_idx": 35, "type": "ite", "entity": "尿素清除指数" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "尿素" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "腹膜" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "肾单位" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "尿液" }, { "start_idx": 70, "end_idx": 72, "type": "dru", "entity": "腹透液" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "尿素" }, { "start_idx": 85, "end_idx": 86, "type": "pro", "entity": "透析" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "尿素" } ] }, { "text": "通过计算KT/Vurea来作为透析充分的参考指标,CAPDKT/Vurea为2.0/w,最低不小于1.9/w,CCPDKT/Vurea为2.1/w,NIPDKT/Vurea为2.2/w。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "ite", "entity": "KT/Vurea" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "透析" }, { "start_idx": 25, "end_idx": 36, "type": "ite", "entity": "CAPDKT/Vurea" }, { "start_idx": 55, "end_idx": 66, "type": "ite", "entity": "CCPDKT/Vurea" } ] }, { "text": "(2)肌酐清除率(creatinineclearancerate,Ccr):与KT/Vurea相比,对肌酐清除率的计算能反映腹膜对大分子的溶质清除,因此对于透析充分性除有KT/Vurea值以外,临床上常用Ccr来作为判断透析效能的指标。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "肌酐清除率" }, { "start_idx": 9, "end_idx": 31, "type": "ite", "entity": "creatinineclearancerate" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "Ccr" }, { "start_idx": 39, "end_idx": 46, "type": "ite", "entity": "KT/Vurea" }, { "start_idx": 51, "end_idx": 55, "type": "ite", "entity": "肌酐清除率" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "腹膜" }, { "start_idx": 78, "end_idx": 79, "type": "pro", "entity": "透析" }, { "start_idx": 85, "end_idx": 92, "type": "ite", "entity": "KT/Vurea" }, { "start_idx": 102, "end_idx": 104, "type": "ite", "entity": "Ccr" }, { "start_idx": 110, "end_idx": 111, "type": "pro", "entity": "透析" } ] }, { "text": "如果将KT/Vurea与Ccr指标综合判断能提高其准确性。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "ite", "entity": "KT/Vurea" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "Ccr" } ] }, { "text": "其标准为CAPD时肌酐为60L/w或9L/d;CCPD为63L/w,NIPD为66L/w。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "CAPD" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "肌酐" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "CCPD" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "NIPD" } ] }, { "text": "3.透析充分的临床标准患者自我感觉良好,血压稳定,无恶心、呕吐、乏力和厌食,生长发育正常。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "透析" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "血压" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "恶心" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "呕吐" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "乏力" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "厌食" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "生长发育正常" } ] }, { "text": "贫血改善;电解质正常,尿素氮<50mg/dl(<17.86mmol/L)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "贫血改善" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "电解质正常" } ] }, { "text": "第八节吸入性肺炎吸入性肺炎(aspirationpneumonia)是指呼吸道直接吸入有机或无机物质造成的肺部炎性病变。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "吸入性肺炎" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "吸入性肺炎" }, { "start_idx": 14, "end_idx": 32, "type": "dis", "entity": "aspirationpneumonia" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "呼吸道" } ] }, { "text": "大多见于早产、弱小婴儿、重度营养不良或有腭裂的婴儿,如平卧喂奶或小儿哭叫时强迫服药易造成吸入;也见于用麻醉剂、中枢神经系统疾病等导致咽部反射或咳嗽反射失灵的患儿。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "早产" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "弱小" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "重度营养不良" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "有腭裂" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "麻醉剂" }, { "start_idx": 55, "end_idx": 62, "type": "dis", "entity": "中枢神经系统疾病" }, { "start_idx": 71, "end_idx": 76, "type": "sym", "entity": "咳嗽反射失灵" } ] }, { "text": "吸入物进入呼吸道后可产生物理或化学刺激,初期多为细支气管和毛细支气管痉挛,导致肺气肿或不张,以后可发生肺实质、肺间质、支气管的炎性病变。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "呼吸道" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "肺气肿或不张" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "肺实质" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "肺间质" }, { "start_idx": 59, "end_idx": 66, "type": "dis", "entity": "支气管的炎性病变" } ] }, { "text": "一、类脂性肺炎类脂性肺炎(lipoidpneumonia)系鱼肝油、石蜡油、油性滴鼻剂等油脂性物质吸入造成的一种肺炎,病理特征为慢性间质性肺炎。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "类脂性肺炎" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "类脂性肺炎" }, { "start_idx": 13, "end_idx": 27, "type": "dis", "entity": "lipoidpneumonia" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "肺炎" }, { "start_idx": 64, "end_idx": 70, "type": "dis", "entity": "慢性间质性肺炎" } ] }, { "text": "重者可出现阵发性呼吸暂停及发绀。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "阵发性呼吸暂停及发绀" } ] }, { "text": "急性期外周血白细胞数增高肺部可闻湿啰音、痰鸣音肺实变体征胸部X线检查常见肺门阴影增大变浓,重症可见两肺气肿、肺门旁及肺野内有片絮状密度增深阴影条索状间质性浸润。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "急性期外周血白细胞数增高" }, { "start_idx": 12, "end_idx": 22, "type": "sym", "entity": "肺部可闻湿啰音、痰鸣音" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "肺实变体征" }, { "start_idx": 28, "end_idx": 33, "type": "ite", "entity": "胸部X线检查" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "肺门阴影增大" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "变浓" }, { "start_idx": 45, "end_idx": 70, "type": "sym", "entity": "重症可见两肺气肿、肺门旁及肺野内有片絮状密度增深阴影" }, { "start_idx": 71, "end_idx": 78, "type": "sym", "entity": "条索状间质性浸润" } ] }, { "text": "根据年龄及病史,病变不易吸收,痰中找到含油滴的巨噬细胞即可以确诊。", "entities": [ { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "痰" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "巨噬细胞" } ] }, { "text": "婴幼儿慎用油类口服药物,尤其勿强制灌药。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "强制灌药" } ] }, { "text": "半昏迷时更应避免,并禁止油剂滴鼻。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "油剂滴鼻" } ] }, { "text": "四、肺隔离症肺隔离症(pulmonarysequestration)的特征为一部分胚胎性囊性肺组织与正常的肺组织相隔离,其血供来自体循环。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "肺隔离症" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "肺隔离症" }, { "start_idx": 11, "end_idx": 32, "type": "dis", "entity": "pulmonarysequestration" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "肺组织" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "肺组织" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "血" } ] }, { "text": "按其病理解剖特点分为:(一)肺内型较常见,张雷等报道42例中,37例(88%)为肺内型。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肺内型" } ] }, { "text": "病变在某肺叶中,由共同的胸膜包被。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肺叶" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胸膜" } ] }, { "text": "病变内的囊腔可与支气管相通。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "囊腔" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "支气管" } ] }, { "text": "其血液供应来源于胸主动脉或腹主动脉,通过肺韧带进入肺内。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "血液" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "胸主动脉" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "腹主动脉" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肺韧带" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肺" } ] }, { "text": "(二)肺外型隔离的肺组织位于肺叶外,且不与支气管相通,其血液供应多来源于腹主动脉异常分支,跨过横膈的食管裂隙或主动脉裂隙进入隔离的肺组织。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "肺外型" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肺组织" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肺叶外" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "支气管" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "血液" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "腹主动脉" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "横膈" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "食管裂隙" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "主动脉裂隙" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "肺组织" } ] }, { "text": "多发生于左侧肺下叶后基底部与横膈之间,常伴有横膈缺损。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "多发生于左侧肺下叶后基底部与横膈之间" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "常伴有横膈缺损" } ] }, { "text": "本病可能系胚胎发育异常所致,但亦有人认为肺内型可能是感染和炎症的结果或是一种囊腺瘤样畸形。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "胚胎发育异常" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "肺内型" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "感染" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "炎症" } ] }, { "text": "两型隔离肺均易并发其他先天性畸形,如膈疝、肠重复畸形、先天性心脏病等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "两型隔离肺" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "先天性畸形" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "膈疝" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "肠重复畸形" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "肺内型隔离肺多与支气管相通,易引起肺部感染,主要表现为反复发作的肺部感染。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肺内型隔离" }, { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "肺多与支气管相通" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "肺部感染" }, { "start_idx": 27, "end_idx": 35, "type": "sym", "entity": "反复发作的肺部感染" } ] }, { "text": "囊内如出现液平,提示囊腔与支气管相通。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "囊内如出现液平" }, { "start_idx": 8, "end_idx": 17, "type": "sym", "entity": "提示囊腔与支气管相通" } ] }, { "text": "支气管造影显示造影剂不能进入病变区,邻近的正常支气管阴影受到挤压出现移位。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "支气管造影" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "造影剂" }, { "start_idx": 18, "end_idx": 35, "type": "sym", "entity": "邻近的正常支气管阴影受到挤压出现移位" } ] }, { "text": "选择性血管造影可显示隔离肺、异常血管及其途径,有助于诊断并判断异常血管的方位。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "选择性血管造影" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肺" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "异常血管" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "血管" } ] }, { "text": "肺隔离症一般应考虑手术切除,肺外型一般做病灶切除即可,肿内型则需做肺叶切除。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "肺隔离症" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "手术" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肺外型" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "肿内型" } ] }, { "text": "Lopoo等随访14例胎儿隔离肺,2例因张力性水胸行宫内胸腔羊膜腔分流术,4例出生前病变完全退缩,10例择期手术均获成功。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "胎儿隔离肺" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "张力性水胸" }, { "start_idx": 26, "end_idx": 35, "type": "pro", "entity": "宫内胸腔羊膜腔分流术" } ] }, { "text": "第二节急性上呼吸道感染急性上呼吸道感染(acuteupperrespiratoryinfection,AURI)简称上感,是小儿最常见的疾病。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "急性上呼吸道感染" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "急性上呼吸道感染" }, { "start_idx": 21, "end_idx": 50, "type": "dis", "entity": "acuteupperrespiratoryinfection" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "AURI" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "上感" } ] }, { "text": "婴幼儿由于上呼吸道的解剖生理特点和免疫特点易患本病。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "上呼吸道" } ] }, { "text": "若有全身诱因如维生素D缺乏性佝偻病、营养不良等疾病;或护理不当、气候改变和不良环境因素等,则易致反复感染或使病程迁延。", "entities": [ { "start_idx": 7, "end_idx": 16, "type": "dis", "entity": "维生素D缺乏性佝偻病" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "营养不良" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "感染" } ] }, { "text": "与患儿年龄、感染病原及机体抵抗力不同有关,年长儿症状较轻,而婴幼儿常较重。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "感染" } ] }, { "text": "一、急性鼻咽炎急性鼻咽炎(acutenasopharyngitis)俗称伤风或感冒(commoncold),病原体侵犯鼻及鼻咽部为主,主要病原为鼻病毒、副流感病毒、呼吸道合胞病毒和冠状病毒,其他病原少见。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "急性鼻咽炎" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "急性鼻咽炎" }, { "start_idx": 13, "end_idx": 32, "type": "dis", "entity": "acutenasopharyngitis" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "伤风" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "感冒" }, { "start_idx": 42, "end_idx": 51, "type": "dis", "entity": "commoncold" }, { "start_idx": 59, "end_idx": 59, "type": "bod", "entity": "鼻" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "鼻咽部" }, { "start_idx": 72, "end_idx": 74, "type": "mic", "entity": "鼻病毒" }, { "start_idx": 76, "end_idx": 80, "type": "mic", "entity": "副流感病毒" }, { "start_idx": 82, "end_idx": 88, "type": "mic", "entity": "呼吸道合胞病毒" }, { "start_idx": 90, "end_idx": 93, "type": "mic", "entity": "冠状病毒" } ] }, { "text": "年长儿症状较轻,常于受凉后1~3天出现上述卡他症状;有些在发病早期可有阵发性脐周疼痛,与发热所致阵发性肠痉挛或肠系膜淋巴结炎有关。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "卡他症状" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "脐周" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "阵发性脐周疼痛" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "发热" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "阵发性肠痉挛" }, { "start_idx": 55, "end_idx": 61, "type": "dis", "entity": "肠系膜淋巴结炎" } ] }, { "text": "第十一节新生儿破伤风新生儿破伤风(neonataltetanus)是由破伤风厌氧芽胞梭状杆菌由脐部侵入引起的一种急性感染性疾病。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "新生儿破伤风" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "新生儿破伤风" }, { "start_idx": 17, "end_idx": 31, "type": "dis", "entity": "neonataltetanus" }, { "start_idx": 35, "end_idx": 45, "type": "mic", "entity": "破伤风厌氧芽胞梭状杆菌" }, { "start_idx": 56, "end_idx": 62, "type": "dis", "entity": "急性感染性疾病" } ] }, { "text": "常在生后7天左右发病,临床上以全身骨骼肌的强直性痉挛、牙关紧闭为特征,故有“脐风”、“七日风”、“锁口风”之称。", "entities": [ { "start_idx": 15, "end_idx": 25, "type": "sym", "entity": "全身骨骼肌的强直性痉挛" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "牙关紧闭" } ] }, { "text": "【临床流行病学】(一)发病率和病死率新生儿破伤风在世界各国的发病率有很大差异,自19世纪80年代无菌接生法和妊娠期破伤风免疫预防的推广,其发病率和死亡率已有所下降。", "entities": [ { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "新生儿破伤风" } ] }, { "text": "据WHO调查,在1994年每年有约51万名新生儿死于破伤风,其中约80%发生于东南亚和非洲的国家。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "破伤风" } ] }, { "text": "(二)病原学1.病原菌特点破伤风杆菌为革兰染色阳性、产芽胞的、梭形厌氧菌,长2~5μm,宽0.3~0.5μm,无荚膜,有周身鞭毛,能运动。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "病原菌" }, { "start_idx": 13, "end_idx": 17, "type": "mic", "entity": "破伤风杆菌" }, { "start_idx": 33, "end_idx": 35, "type": "mic", "entity": "厌氧菌" } ] }, { "text": "破伤风杆菌不是组织侵袭性细菌,仅通过破伤风痉挛毒素致病;破伤风毒素是已知毒素中排位第二的毒素,仅次于肉毒毒素,其致死量约10-6mg/kg。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "破伤风杆菌" }, { "start_idx": 18, "end_idx": 24, "type": "mic", "entity": "破伤风痉挛毒素" }, { "start_idx": 28, "end_idx": 32, "type": "mic", "entity": "破伤风毒素" }, { "start_idx": 50, "end_idx": 53, "type": "mic", "entity": "肉毒毒素" } ] }, { "text": "2.感染方式用未消毒的剪刀、线绳来断脐、结扎脐带;接生者的手或包盖脐带残端的棉花纱布未严格消毒时,破伤风梭菌即可由此侵入。", "entities": [ { "start_idx": 49, "end_idx": 53, "type": "mic", "entity": "破伤风梭菌" } ] }, { "text": "【发病机制】坏死的脐残端及其上的覆盖物使该处氧化还原电势降低,有利于破伤风梭菌出芽繁殖并产生破伤风痉挛毒素而致病。", "entities": [ { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "氧化还原电势降低" }, { "start_idx": 34, "end_idx": 38, "type": "mic", "entity": "破伤风梭菌" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "破伤风梭菌出芽繁殖" }, { "start_idx": 46, "end_idx": 52, "type": "mic", "entity": "破伤风痉挛毒素" }, { "start_idx": 44, "end_idx": 52, "type": "sym", "entity": "产生破伤风痉挛毒素" } ] }, { "text": "破伤风毒素经淋巴液中淋巴细胞入血,附在球蛋白到达中枢神经系统;也可由肌肉神经结合处吸收,通过外周神经的内膜和外膜间隙或运动神经轴上行至脊髓和脑干。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "破伤风毒素" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "淋巴液" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "球蛋白" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "肌肉神经" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "运动神经轴" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "脊髓" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "脑干" } ] }, { "text": "此毒素一旦与中枢神经组织中的神经节苷脂结合,抗毒素也不能中和。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "中枢神经组织" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "神经节苷脂" } ] }, { "text": "毒素与灰质中突触小体膜的神经节苷脂结合后,使它不能释放抑制性神经介质(甘氨酸、氨基丁酸),以致运动神经系统对传入刺激的反射强化,导致屈肌与伸肌同时强烈地持续收缩。", "entities": [ { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "甘氨酸" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "氨基丁酸" }, { "start_idx": 47, "end_idx": 52, "type": "bod", "entity": "运动神经系统" }, { "start_idx": 66, "end_idx": 79, "type": "sym", "entity": "屈肌与伸肌同时强烈地持续收缩" } ] }, { "text": "活动越频繁的肌群,越先受累,故咀嚼肌痉挛使牙关紧闭,面肌痉挛而呈苦笑面容,腹背肌当痉挛较强后,形成角弓反张。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "咀嚼肌痉挛" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "牙关紧闭" }, { "start_idx": 26, "end_idx": 35, "type": "sym", "entity": "面肌痉挛而呈苦笑面容" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "形成角弓反张" } ] }, { "text": "此毒素亦可兴奋交感神经,导致心动过速、高血压、多汗等表现。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "兴奋交感神经" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "心动过速" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "高血压" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "多汗" } ] }, { "text": "一般以哭吵不安起病,患儿想吃,但口张不大,吸吮困难。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "哭吵不安" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "口张不大" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "吸吮困难" } ] }, { "text": "随后牙关紧闭,眉举额皱,口角上牵,出现“苦笑”面容,双拳紧握,上肢过度屈曲,下肢伸直,成角弓反张状。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "牙关紧闭" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "眉举额皱" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "口角上牵" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "出现“苦笑”面容" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "双拳紧握" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "上肢过度屈曲" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "下肢伸直" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "成角弓反张状" } ] }, { "text": "强直性痉挛阵阵发作,间歇期肌肉收缩仍继续存在,轻微刺激(声、光、轻触、饮水、轻刺等)常诱发痉挛发作。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "强直性痉挛阵阵发作" }, { "start_idx": 10, "end_idx": 21, "type": "sym", "entity": "间歇期肌肉收缩仍继续存在" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "轻微刺激" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "诱发痉挛" } ] }, { "text": "患儿神志清醒,早期多不发热,以后体温升高可因全身肌肉反复强直痉挛引起,亦可因肺炎等继发感染所致。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "体温升高" }, { "start_idx": 22, "end_idx": 31, "type": "sym", "entity": "全身肌肉反复强直痉挛" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "肺炎" } ] }, { "text": "否则,因越发越频,缺氧窒息或继发感染而死亡。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "缺氧窒息" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "感染" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "死亡" } ] }, { "text": "【实验室检查】常规实验室检查多正常,周围血象中白细胞可因脐带继发感染或持续痉挛引起的应激反应而升高。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "白细胞" }, { "start_idx": 18, "end_idx": 48, "type": "sym", "entity": "周围血象中白细胞可因脐带继发感染或持续痉挛引起的应激反应而升高" } ] }, { "text": "脐部分泌物培养仅部分患儿阳性。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脐部" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "脐部分泌物培养仅部分患儿阳性" } ] }, { "text": "【诊断】破伤风的症状最有特征性,根据消毒不严的接生史、出生后典型发作表现,一般容易诊断;早期尚无典型表现时,可用压舌板检查患儿咽部,若越用力下压,压舌板反被咬得越紧,也可确诊。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "破伤风" } ] }, { "text": "同时,肌注青霉素3~4天及破伤风抗毒素1500~3000U或人体破伤风免疫球蛋白75~250U。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "青霉素" }, { "start_idx": 13, "end_idx": 18, "type": "dru", "entity": "破伤风抗毒素" }, { "start_idx": 35, "end_idx": 39, "type": "dru", "entity": "免疫球蛋白" } ] }, { "text": "3.对不能保证无菌接生的孕妇,于妊娠晚期可注射2次破伤风类毒素0.5ml,相隔1个月,第二次至少在产前2周(最好1个月时)肌注。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "注射" }, { "start_idx": 25, "end_idx": 30, "type": "dru", "entity": "破伤风类毒素" }, { "start_idx": 61, "end_idx": 62, "type": "pro", "entity": "肌注" } ] }, { "text": "【治疗】(一)一般治疗1.护理保持室内安静、避光,减少刺激,避免扰动,必需的操作(如测体温、翻身等)尽量集中同时进行。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "保持室内安静" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "避光" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "减少刺激" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "避免扰动" } ] }, { "text": "及时清除痰液,保持呼吸道通畅及口腔、皮肤清洁。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "清除痰液" }, { "start_idx": 7, "end_idx": 16, "type": "pro", "entity": "保持呼吸道通畅及口腔" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "皮肤清洁" } ] }, { "text": "2.保证营养和水分供给后期可鼻饲乳品,如痉挛频繁不能鼻饲,可用静脉营养。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "保证营养" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "水分供给" } ] }, { "text": "3.有缺氧及青紫时给氧,如窒息、呼吸衰竭者应用呼吸机辅助通气。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "窒息" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "呼吸机辅助通气" } ] }, { "text": "有脑水肿者应用甘露醇等脱水剂。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "甘露醇" } ] }, { "text": "1.地西泮有松弛肌肉及抗惊厥作用,每次0.2~0.3mg/kg,缓慢静注,4~6小时1次,若止痉效果不佳,可逐渐增加至每次1mg/kg,痉挛好转后再鼻饲给药,可每次0.5~1mg/kg,必要时还可加大剂量,口服地西泮的半衰期长达10余小时~3天。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "地西泮" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "惊厥" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "静注" }, { "start_idx": 105, "end_idx": 107, "type": "dru", "entity": "地西泮" } ] }, { "text": "近年来,国内有报道应用大剂量地西泮治疗重症新生儿破伤风有较好疗效,即患儿入院后先用地西泮3~5mg注射,15分钟后未达“地西泮化”者加用7.5mg,静脉缓推,最大量每次10mg,达“地西泮化”后每2~3小时给地西泮化量的地西泮1次维持,一般要求用量达到“地西泮化”标准,即患儿浅睡,咳嗽吞咽反射存在,体检时无抽搐,仅在注射、穿刺或吸痰时出现短暂肌强硬,下次给药前可有轻微而短暂的抽搐,但无明显发绀。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "地西泮" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "破伤风" }, { "start_idx": 41, "end_idx": 43, "type": "dru", "entity": "地西泮" }, { "start_idx": 74, "end_idx": 77, "type": "pro", "entity": "静脉缓推" }, { "start_idx": 110, "end_idx": 112, "type": "dru", "entity": "地西泮" }, { "start_idx": 150, "end_idx": 155, "type": "sym", "entity": "体检时无抽搐" }, { "start_idx": 159, "end_idx": 160, "type": "pro", "entity": "注射" }, { "start_idx": 162, "end_idx": 163, "type": "pro", "entity": "穿刺" }, { "start_idx": 165, "end_idx": 166, "type": "pro", "entity": "吸痰" }, { "start_idx": 170, "end_idx": 174, "type": "sym", "entity": "短暂肌强硬" } ] }, { "text": "2.苯巴比妥(鲁米那)负荷量10~20mg/kg,静脉或肌内注射,12小时后维持量5mg/(kg•d)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "鲁米那" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "注射" } ] }, { "text": "3.氯丙嗪每次0.5~1mg/kg,稀释后静滴,每6~8小时可重复一次。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "氯丙嗪" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "静滴" } ] }, { "text": "但剂量过大或持续时间过长可出现软瘫或体温下降,故不宜多用。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "软瘫" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "体温下降" } ] }, { "text": "4.水合氯醛止痉作用快,作为痉挛发作时临时性增加药物。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "水合氯醛" } ] }, { "text": "常用10%溶液每次0.5ml/kg,灌肠或鼻饲注入。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "灌肠" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "鼻饲" } ] }, { "text": "5.副醛止惊效果快而安全,但主要由肺排出刺激呼吸道黏膜,有肺炎时不宜采用。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dru", "entity": "副醛" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "呼吸道黏膜" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "肺炎" } ] }, { "text": "多为临时使用一次,每次0.1~0.2ml/kg(稀释成5%溶液)静注或0.2~0.3ml/kg每次肌注或灌肠。", "entities": [ { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "静注" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "肌注" }, { "start_idx": 52, "end_idx": 53, "type": "pro", "entity": "灌肠" } ] }, { "text": "6.泮库溴铵(pavulon,pancuronium)神经肌肉阻滞药或肌松药,每次0.05~1mg/kg,静脉注射,2~3小时1次,对重症患儿在使用人工呼吸机的情况下可以采用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "泮库溴铵" }, { "start_idx": 7, "end_idx": 13, "type": "dru", "entity": "pavulon" }, { "start_idx": 15, "end_idx": 25, "type": "dru", "entity": "pancuronium" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "肌松药" } ] }, { "text": "一般认为,大剂量地西泮和鲁苯巴比妥交替鼻饲,止痉效果确切,可作为新生儿破伤风止痉的首选搭配,临时可增加水合氯醛或副醛,以上治疗无效时,可给予普鲁卡因6~8mg/(kg•d),稀释后缓慢静脉滴入。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "地西泮" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "鲁苯巴比妥" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "鼻饲" }, { "start_idx": 51, "end_idx": 54, "type": "dru", "entity": "水合氯醛" }, { "start_idx": 56, "end_idx": 57, "type": "dru", "entity": "副醛" }, { "start_idx": 70, "end_idx": 73, "type": "dru", "entity": "普鲁卡因" }, { "start_idx": 92, "end_idx": 95, "type": "pro", "entity": "静脉滴入" } ] }, { "text": "(三)破伤风抗毒素的应用只能中和尚未与神经组织结合的毒素。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "抗毒素" } ] }, { "text": "精制破伤风抗毒素(TAT)1万~2万U肌内注射或静脉注射,用前须作皮试。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "破伤风抗毒素" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "注射" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "静脉注射" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "皮试" } ] }, { "text": "人体破伤风免疫球蛋白(TIG)不会产生血清病等过敏反应,其血浓度较高,半衰期长达24天,故更理想,但其价格昂贵不易获得,新生儿肌注500~1500U即可。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dru", "entity": "破伤风免疫球蛋白" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "血清病" } ] }, { "text": "(四)抗生素青霉素:能杀灭破伤风梭菌,10万~20万U/(kg•d),每天分2次,疗程10天左右。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "抗生素" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "青霉素" }, { "start_idx": 13, "end_idx": 17, "type": "mic", "entity": "破伤风梭菌" } ] }, { "text": "甲硝唑:首剂15mg/kg,然后15mg/(kg•d)或30mg/(kg•d),分2次静滴,1个疗程7天,有报告其疗效略优于青霉素。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "甲硝唑" }, { "start_idx": 62, "end_idx": 64, "type": "dru", "entity": "青霉素" } ] }, { "text": "(五)脐部处理用氧化消毒剂(3%过氧化氢或1∶4000高锰酸钾溶液)清洗脐部,再涂以碘酒以消灭残余破伤风梭菌。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "过氧化氢" }, { "start_idx": 27, "end_idx": 32, "type": "dru", "entity": "高锰酸钾溶液" }, { "start_idx": 49, "end_idx": 53, "type": "mic", "entity": "破伤风梭菌" } ] }, { "text": "五、预后ICH的预后与其发病年龄、病因、出血部位及出血量大小等有关。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "ICH" } ] }, { "text": "脑动静脉畸形易反复出血,复发者病死率较高;如血液流入脑室系统与蛛网膜下腔后,易致脑脊液循环通路阻塞,吸收障碍,产生脑积水。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "脑动静脉畸形" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "反复出血" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血液" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 22, "end_idx": 35, "type": "sym", "entity": "血液流入脑室系统与蛛网膜下腔" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "脑脊液" }, { "start_idx": 40, "end_idx": 48, "type": "sym", "entity": "脑脊液循环通路阻塞" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "吸收障碍" }, { "start_idx": 55, "end_idx": 59, "type": "sym", "entity": "产生脑积水" } ] }, { "text": "脑动脉瘤破裂常产生脑实质内出血,80%以上的病例于早期死亡,幸存者多留有神经系统后遗症。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "脑动脉瘤破裂" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "脑实质内出血" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "死亡" }, { "start_idx": 36, "end_idx": 42, "type": "sym", "entity": "神经系统后遗症" } ] }, { "text": "继发于全身性疾病的ICH预后与原发病、出血部位及其产生的病理反应有关。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "ICH" } ] }, { "text": "脑室周围-脑室内出血的近期预后与出血量大小有关,出血量越大,并发脑积水的发生率或病死率越高;远期随访,出血量大者多发生严重智能减退和运动功能障碍等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "脑室周围" }, { "start_idx": 32, "end_idx": 44, "type": "sym", "entity": "脑积水的发生率或病死率越高" }, { "start_idx": 59, "end_idx": 64, "type": "sym", "entity": "严重智能减退" }, { "start_idx": 66, "end_idx": 71, "type": "sym", "entity": "运动功能障碍" } ] }, { "text": "新生儿蛛网膜下腔出血主要系静脉破裂所致,出血量较小,大多预后良好;少数也可因先天性颅内动脉瘤破裂所致,病情多危重,预后较差,病死率高达40%。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "新生儿蛛网膜下腔出血" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "出血量较小" } ] }, { "text": "幕上硬膜下出血预后相对较好,而幕下硬膜下出血预后差。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "幕上硬膜" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "幕下硬膜" } ] }, { "text": "二、肺炎衣原体感染【病原和流行病学】20世纪60年代在研究沙眼中首次分离到肺炎衣原体,以后用血清学方法证实该病原引起1978年芬兰学生的轻症肺炎流行,1986年从患急性呼吸道疾病的大学生呼吸道分离到该病原,并于1986年被正式命名为肺炎衣原体TWAR株。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "肺炎衣原体感染" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "沙眼" }, { "start_idx": 37, "end_idx": 41, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 68, "end_idx": 71, "type": "dis", "entity": "轻症肺炎" }, { "start_idx": 82, "end_idx": 88, "type": "dis", "entity": "急性呼吸道疾病" }, { "start_idx": 93, "end_idx": 95, "type": "bod", "entity": "呼吸道" }, { "start_idx": 116, "end_idx": 125, "type": "mic", "entity": "肺炎衣原体TWAR株" } ] }, { "text": "肺炎衣原体属革兰阴性菌,没有细胞壁,是人呼吸道的常见病原,尚未发现动物储存宿主。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 6, "end_idx": 10, "type": "mic", "entity": "革兰阴性菌" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "呼吸道" } ] }, { "text": "通过呼吸道飞沫在人与人之间传播,在家庭成员中造成感染传播。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "呼吸道飞沫" } ] }, { "text": "任何年龄均可感染肺炎衣原体。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "mic", "entity": "肺炎衣原体" } ] }, { "text": "【临床表现】临床上,肺炎衣原体感染不易与其他病原体感染特别是肺炎支原体感染相鉴别。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "肺炎衣原体感染" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "病原体感染" }, { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "肺炎支原体感染" } ] }, { "text": "肺炎患者常表现为非典型肺炎,症状轻到中等,有发热、不适、头痛和咳嗽等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肺炎" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "非典型肺炎" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "发热" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "不适" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "头痛" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "咳嗽" } ] }, { "text": "胸部X线表现多较临床症状重,示肺叶浸润影,并可有胸腔积液。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "胸部X线" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺叶" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "肺叶浸润影" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "胸腔" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "胸腔积液" } ] }, { "text": "听诊可闻及湿啰音并常有哮鸣音。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "听诊可闻及湿啰音并常有哮鸣音" } ] }, { "text": "与肺炎支原体感染一样,肺炎衣原体感染也可有肺外表现,如结节性红斑、甲状腺炎、脑炎和吉兰-巴雷综合征等。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "肺炎支原体感染" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "肺炎衣原体感染" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "肺" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "结节性红斑" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "甲状腺炎" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "脑炎" }, { "start_idx": 41, "end_idx": 48, "type": "dis", "entity": "吉兰-巴雷综合征" } ] }, { "text": "【诊断】肺炎衣原体感染的特异性诊断依据组织培养、病原分离和血清学检查,衣原体的分离培养相对较困难,目前多用血清抗体水平作为诊断衣原体感染的依据。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "肺炎衣原体感染" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "病原分离" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "血清学检查" }, { "start_idx": 35, "end_idx": 37, "type": "mic", "entity": "衣原体" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "衣原体感染" } ] }, { "text": "血清学诊断可采用微量免疫荧光法(microimmunoflurescence,MIF),急性衣原体感染的血清学诊断标准是:IgG滴度4倍升高;或IgM滴度≥1∶16;或IgG滴度≥1∶512。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "免疫荧光法" }, { "start_idx": 16, "end_idx": 37, "type": "pro", "entity": "microimmunoflurescence" }, { "start_idx": 39, "end_idx": 41, "type": "pro", "entity": "MIF" }, { "start_idx": 44, "end_idx": 50, "type": "dis", "entity": "急性衣原体感染" }, { "start_idx": 61, "end_idx": 65, "type": "ite", "entity": "IgG滴度" }, { "start_idx": 72, "end_idx": 76, "type": "ite", "entity": "IgM滴度" }, { "start_idx": 84, "end_idx": 88, "type": "ite", "entity": "IgG滴度" } ] }, { "text": "酶联免疫吸附法(ELISA)可用于痰标本中肺炎衣原体抗原的检测。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "酶联免疫吸附法" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "ELISA" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "痰" }, { "start_idx": 21, "end_idx": 27, "type": "mic", "entity": "肺炎衣原体抗原" } ] }, { "text": "DNA探针检测衣原体具有较高的特异性和敏感性,聚合酶链技术快速、准确,也具有广泛的应用前景。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "equ", "entity": "DNA探针" }, { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "衣原体" }, { "start_idx": 23, "end_idx": 28, "type": "pro", "entity": "聚合酶链技术" } ] }, { "text": "【治疗】肺炎衣原体由于缺乏细胞壁,对β内酰胺类抗生素无效。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 18, "end_idx": 25, "type": "dru", "entity": "β内酰胺类抗生素" } ] }, { "text": "较为有效的抗菌药物主要包括大环内酯类、四环素类和氟喹诺酮类。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "大环内酯类" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "四环素类" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "氟喹诺酮类" } ] }, { "text": "大环内酯类中以阿奇霉素和克拉霉素效果较好,其中四环素类和氟喹诺酮类不推荐在儿童中使用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "大环内酯类" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "阿奇霉素" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "克拉霉素" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "四环素类" }, { "start_idx": 28, "end_idx": 32, "type": "dru", "entity": "氟喹诺酮类" } ] }, { "text": "此外由于利福平及其衍生物具有较高细胞内浓度,因此,对于专性细胞内寄生的衣原体有十分明显的抗菌作用。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "利福平" }, { "start_idx": 35, "end_idx": 37, "type": "mic", "entity": "衣原体" } ] }, { "text": "肺炎衣原体感染的治疗简单而有效,临床上耐药不多见,但主张长疗程,以避免复发。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "肺炎衣原体感染" } ] }, { "text": "第七章真菌感染性疾病第一节概况真菌(fungus)亦称霉菌,广泛分布于自然界,是医学微生物学的一个重要组成部分,和其他病原微生物一样,也可引起各种类型的疾病。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "真菌感染性疾病" }, { "start_idx": 15, "end_idx": 16, "type": "mic", "entity": "真菌" }, { "start_idx": 18, "end_idx": 23, "type": "mic", "entity": "fungus" }, { "start_idx": 27, "end_idx": 28, "type": "mic", "entity": "霉菌" } ] }, { "text": "真菌是异养的真核生物,有真正的细胞核和细胞器,能进行有丝分裂。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "真菌" } ] }, { "text": "真菌种类繁多,数目有10万种以上,其中能引起人或动物感染的仅占极少部分,约400种。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "真菌" } ] }, { "text": "与医院感染有关的有200余种,包括致病真菌、条件致病真菌(机会真菌,opportuniticfungi)、致敏真菌和产毒、促癌及致癌真菌。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "致病真菌" }, { "start_idx": 22, "end_idx": 27, "type": "mic", "entity": "条件致病真菌" }, { "start_idx": 29, "end_idx": 32, "type": "mic", "entity": "机会真菌" }, { "start_idx": 34, "end_idx": 50, "type": "mic", "entity": "opportuniticfungi" }, { "start_idx": 53, "end_idx": 56, "type": "mic", "entity": "致敏真菌" }, { "start_idx": 64, "end_idx": 67, "type": "mic", "entity": "致癌真菌" } ] }, { "text": "同一种疾病可由不同种类的真菌引起,一种真菌也可引起不同种类的疾病。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "mic", "entity": "真菌" }, { "start_idx": 19, "end_idx": 20, "type": "mic", "entity": "真菌" } ] }, { "text": "放线菌介于细菌和真菌之间,并不属于真菌,但因其所引起的疾病症状和体征都酷似真菌感染,故传统上一直将放线菌病归在真菌病中描述。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "放线菌" }, { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "细菌" }, { "start_idx": 8, "end_idx": 9, "type": "mic", "entity": "真菌" }, { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "真菌" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "真菌感染" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "放线菌病" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "真菌病" } ] }, { "text": "(一)真菌感染的影响因素1.真菌因素当机体缺乏对致病真菌的特异性免疫时,致病真菌可使正常健康人感染发病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "真菌感染" }, { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "真菌" }, { "start_idx": 24, "end_idx": 27, "type": "mic", "entity": "致病真菌" }, { "start_idx": 36, "end_idx": 39, "type": "mic", "entity": "致病真菌" } ] }, { "text": "在机体抗感染能力低下时,条件致病真菌可进入人正常的无菌部位或不常寄生的组织器官,其种类最多,感染率最高,危害性最大。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "mic", "entity": "条件致病真菌" } ] }, { "text": "2.宿主因素除致病真菌外,宿主的易感性也与感染密切相关。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "mic", "entity": "致病真菌" } ] }, { "text": "机体缺乏抵抗力,如婴儿,由于体内产生抗体的器官尚未发育完善,抗体缺乏,全身抵抗力低下;伴有影响机体抵抗力的各种疾病,如白血病、恶性肿瘤、结核、糖尿病、严重烧伤、营养不良、肝脏疾病及维生素缺乏等某些疾病后机体抵抗力受损或低下;特别是中性粒细胞减少的患者最容易并发念珠菌感染等真菌感染,此类患者使用抗生素后仍有发热,并有肌痛、关节痛、内眼炎,或有心动过速、呼吸困难或皮肤有红斑丘疹结节,高度提示有系统性或播散性念珠菌病的可能。", "entities": [ { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "白血病" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "结核" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "糖尿病" }, { "start_idx": 75, "end_idx": 78, "type": "dis", "entity": "严重烧伤" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "营养不良" }, { "start_idx": 85, "end_idx": 88, "type": "dis", "entity": "肝脏疾病" }, { "start_idx": 90, "end_idx": 94, "type": "dis", "entity": "维生素缺乏" }, { "start_idx": 101, "end_idx": 110, "type": "sym", "entity": "机体抵抗力受损或低下" }, { "start_idx": 115, "end_idx": 119, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 115, "end_idx": 121, "type": "sym", "entity": "中性粒细胞减少" }, { "start_idx": 130, "end_idx": 134, "type": "dis", "entity": "念珠菌感染" }, { "start_idx": 136, "end_idx": 139, "type": "dis", "entity": "真菌感染" }, { "start_idx": 147, "end_idx": 149, "type": "dru", "entity": "抗生素" }, { "start_idx": 153, "end_idx": 154, "type": "sym", "entity": "发热" }, { "start_idx": 158, "end_idx": 159, "type": "sym", "entity": "肌痛" }, { "start_idx": 161, "end_idx": 163, "type": "sym", "entity": "关节痛" }, { "start_idx": 165, "end_idx": 167, "type": "sym", "entity": "内眼炎" }, { "start_idx": 171, "end_idx": 174, "type": "sym", "entity": "心动过速" }, { "start_idx": 176, "end_idx": 179, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 181, "end_idx": 182, "type": "bod", "entity": "皮肤" }, { "start_idx": 181, "end_idx": 189, "type": "sym", "entity": "皮肤有红斑丘疹结节" }, { "start_idx": 196, "end_idx": 206, "type": "dis", "entity": "系统性或播散性念珠菌病" } ] }, { "text": "另外,机体某些特殊部位,如眼的前房和后房、脑膜、关节、心内膜和尿道,对微生物侵袭的抵抗力很低,尤易受条件致病真菌的感染。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "bod", "entity": "眼的前房和后房" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "脑膜" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "关节" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "心内膜" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "尿道" }, { "start_idx": 52, "end_idx": 55, "type": "mic", "entity": "致病真菌" } ] }, { "text": "3.其他因素实施某些使机体抵抗力降低的诊疗措施,如使用抗生素、激素、免疫抑制剂或接受放疗和化疗的肿瘤患者、脾摘除、器官移植及外伤等患者,易受条件致病真菌感染。", "entities": [ { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "抗生素" }, { "start_idx": 31, "end_idx": 32, "type": "dru", "entity": "激素" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "放疗" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "化疗" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "肿瘤" }, { "start_idx": 53, "end_idx": 55, "type": "pro", "entity": "脾摘除" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "器官移植" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "外伤" }, { "start_idx": 72, "end_idx": 77, "type": "dis", "entity": "致病真菌感染" } ] }, { "text": "手术后异物,缝线和修补手术埋入的材料,以及留置导管等均可引起局部的真菌感染。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "缝线" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "修补手术" }, { "start_idx": 23, "end_idx": 24, "type": "equ", "entity": "导管" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "局部的真菌感染" } ] }, { "text": "(二)真菌感染的来源1.内源性感染是指某些真菌平时就在机体内寄生,成为正常菌群一个组成部分,并不致病,如寄生在口腔、肠道和阴道等处。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "真菌感染" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "真菌" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "口腔" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "肠道" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "阴道" } ] }, { "text": "由于某些诱因,机体免疫功能降低时则可引起严重的真菌病。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "真菌病" } ] }, { "text": "2.外源性感染这类真菌平时在人体内并不存在,而存在于自然界,经呼吸道、胃肠道或有损伤的皮肤侵入人体而发病。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "真菌" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "呼吸道" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "胃肠道" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "皮肤" } ] }, { "text": "3.条件致病真菌感染条件致病真菌感染可以是内源性的也可以是外源性的。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "条件致病真菌感染" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "条件致病真菌感染" } ] }, { "text": "无病原性或病原性极弱的一部分真菌,在机体特异或非特异性免疫功能低下或由于机体内菌群失调时而引起的感染,称为条件致病真菌感染。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "真菌" }, { "start_idx": 53, "end_idx": 60, "type": "dis", "entity": "条件致病真菌感染" } ] }, { "text": "(三)真菌感染的途径由于病原性真菌的种类和在自然界中的分布,以及患者的年龄、生活环境的不同,其感染途径也是多种多样的。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "真菌感染" }, { "start_idx": 12, "end_idx": 16, "type": "mic", "entity": "病原性真菌" } ] }, { "text": "常见的几种真菌感染途径为:假丝酵母菌(如白色念珠菌)可经常存在于健康人的皮肤、黏膜、肠道等处,多为内源性感染;有时可由接触发生交叉感染。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "mic", "entity": "假丝酵母菌" }, { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "白色念珠菌" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "皮肤" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "黏膜" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "肠道" } ] }, { "text": "隐球菌病为外源性感染,可通过吸入或皮肤接触而引起感染。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "隐球菌病" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "皮肤" } ] }, { "text": "毛霉菌常因吸入而引起肺部感染或食入含孢子的食物而引起肠道感染。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "毛霉菌" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "肺部感染" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "肠道感染" } ] }, { "text": "例如内脏念珠菌病多为继发性感染,属于内源性,大量广谱抗生素、放疗、化疗和肾上腺皮质激素促进了上皮表面,尤其是口咽部和消化道念珠菌的大量生长和侵入,而肺部感染多由吸入念珠菌引起。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "内脏念珠菌病" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "广谱抗生素" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "放疗" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "化疗" }, { "start_idx": 36, "end_idx": 42, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "上皮表面" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "口咽部" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "消化道" }, { "start_idx": 61, "end_idx": 63, "type": "mic", "entity": "念珠菌" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "肺部感染" }, { "start_idx": 82, "end_idx": 84, "type": "mic", "entity": "念珠菌" } ] }, { "text": "(四)真菌感染的类型根据侵犯部位及范围分为以下几种:1.浅部真菌病主要侵犯皮肤及其附属器官(也就是癣菌病,简称“癣”),一般症状较轻,90%以上的真菌属此类。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "真菌感染" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "浅部真菌病" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "皮肤" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "癣菌病" }, { "start_idx": 56, "end_idx": 56, "type": "dis", "entity": "癣" }, { "start_idx": 73, "end_idx": 74, "type": "mic", "entity": "真菌" } ] }, { "text": "2.深部真菌病主要侵犯皮下组织、黏膜深处,内脏器官,骨骼,中枢神经系统等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "深部真菌病" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "皮下组织" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "黏膜深处" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "内脏器官" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "骨骼" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "局限性真菌病,是指真菌侵犯特定的器官、组织,如头癣、甲癣等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "局限性真菌病" }, { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "真菌" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "头癣" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "甲癣" } ] }, { "text": "全身性真菌感染,如疾病一开始就侵犯深部组织器官或病变从局部开始进一步发展侵犯各脏器、组织形成感染灶,则为全身侵袭性或系统性真菌感染。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "全身性真菌感染" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "脏器" }, { "start_idx": 52, "end_idx": 64, "type": "dis", "entity": "全身侵袭性或系统性真菌感染" } ] }, { "text": "真菌也可引起败血症,称为真菌败血症。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "真菌" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "败血症" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "真菌败血症" } ] }, { "text": "(五)真菌感染性疾病的免疫学特点正常情况下人对真菌具有高度的非特异性抵抗力。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "真菌感染性疾病" }, { "start_idx": 23, "end_idx": 24, "type": "mic", "entity": "真菌" } ] }, { "text": "这些非特异性抵抗力主要表现为:机体的屏障作用、吞噬细胞对真菌的吞噬功能、正常体液中非特异性抗真菌因素、内分泌因素和微生态环境。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "吞噬细胞" }, { "start_idx": 28, "end_idx": 29, "type": "mic", "entity": "真菌" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "体液" } ] }, { "text": "但免疫功能基本正常的儿童,由于吸入大量真菌孢子,如空调污染、密切接触鸽类以及接触有真菌存在的环境等,超过机体抵抗力而发病,多见于肺隐球菌病,其次是侵袭性肺曲霉菌病。", "entities": [ { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "肺隐球菌病" }, { "start_idx": 73, "end_idx": 80, "type": "dis", "entity": "侵袭性肺曲霉菌病" } ] }, { "text": "在严重的免疫缺陷患者,合并机会性感染常无明显的感染灶,组织反应和炎细胞浸润也比较轻微。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "炎细胞" }, { "start_idx": 27, "end_idx": 41, "type": "sym", "entity": "组织反应和炎细胞浸润也比较轻微" } ] }, { "text": "(六)真菌的检测真菌的检测包括直接镜检、培养检查、特殊染色检查、生化试验、动物接种和免疫学检查等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "真菌" }, { "start_idx": 8, "end_idx": 9, "type": "mic", "entity": "真菌" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "直接镜检" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "培养检查" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "特殊染色检查" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "生化试验" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "动物接种" }, { "start_idx": 42, "end_idx": 46, "type": "pro", "entity": "免疫学检查" } ] }, { "text": "1.病原学检查(1)直接镜检:从病损部位直接采取标本,进行镜检。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "直接镜检" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "镜检" } ] }, { "text": "将标本置于玻片上,加一滴5%~10%氢氧化钾溶液,覆以盖玻片,轻微加热后于显微镜下检查。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "equ", "entity": "玻片" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "氢氧化钾" }, { "start_idx": 27, "end_idx": 29, "type": "equ", "entity": "盖玻片" }, { "start_idx": 37, "end_idx": 39, "type": "equ", "entity": "显微镜" } ] }, { "text": "(2)真菌培养:根据真菌感染部位确定采取标本。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "真菌" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "真菌感染" } ] }, { "text": "理想的培养标本是通过无菌技术采取的血液、骨髓、胸水、腹水和脑脊液,检出真菌时的临床意义较大。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "血液" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "骨髓" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "胸水" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "腹水" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "脑脊液" }, { "start_idx": 35, "end_idx": 36, "type": "mic", "entity": "真菌" } ] }, { "text": "从脑脊液、血液中培养出致病性真菌,如新型隐球菌,一次培养阳性即有诊断意义。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "脑脊液" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "血液" }, { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "致病性真菌" }, { "start_idx": 18, "end_idx": 22, "type": "mic", "entity": "新型隐球菌" } ] }, { "text": "从痰、尿、大便致病菌,如念珠菌、曲霉菌、毛霉菌则有可能是标本污染,或是正常寄生菌,必须多次培养阳性且为同一种菌,并结合临床、内镜和病理检查方可确诊。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "痰" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "尿" }, { "start_idx": 5, "end_idx": 5, "type": "ite", "entity": "大" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "大" }, { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "病菌," }, { "start_idx": 12, "end_idx": 14, "type": "mic", "entity": "念珠菌" }, { "start_idx": 16, "end_idx": 18, "type": "mic", "entity": "曲霉菌" }, { "start_idx": 58, "end_idx": 59, "type": "pro", "entity": "合临" } ] }, { "text": "(3)动物接种:将真菌感染的标本,如脑脊液、血液等,接种于敏感动物,如小白鼠等,饲养数周后处死,相应的动物组织中可见到大量的真菌,可以确诊。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "动物接种" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "真菌感染" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "脑脊液" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血液" }, { "start_idx": 62, "end_idx": 63, "type": "mic", "entity": "真菌" } ] }, { "text": "2.病理检查真菌感染的组织病理变化与感染的菌种和感染的部位有关,也随病程的不同程度而改变。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "病理检查" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "真菌感染" } ] }, { "text": "一般病变早期多为化脓性改变,而在晚期多为肉芽肿性改变。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "化脓性改变" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "肉芽肿性改变" } ] }, { "text": "但是,真菌组织反应不具特异性,所以病理上的组织反应只对真菌感染的诊断具有参考意义。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "真菌" } ] }, { "text": "只有在病变组织中发现有真菌才能确诊为真菌感染。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "真菌" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "真菌感染" } ] }, { "text": "3.其他检查(1)血液标本真菌细胞壁成分1,3-β-D葡聚糖抗原(G试验):1,3-β-D葡聚糖广泛存在于真菌细胞壁中,但不包括隐球菌和接合菌,而其他微生物、动物及人体细胞均无此成分。", "entities": [ { "start_idx": 9, "end_idx": 36, "type": "pro", "entity": "血液标本真菌细胞壁成分1,3-β-D葡聚糖抗原(G试验)" }, { "start_idx": 53, "end_idx": 57, "type": "mic", "entity": "真菌细胞壁" }, { "start_idx": 64, "end_idx": 66, "type": "mic", "entity": "隐球菌" }, { "start_idx": 68, "end_idx": 70, "type": "mic", "entity": "接合菌" } ] }, { "text": "(2)血液标本曲霉半乳甘露聚糖抗原(GM)检测(ELISA):连续2次吸光度值(I)>0.8或单次I>1.5;GM检测患儿的假阳性率比成年患者高,因为儿童会比成年人食用更多含有GM的食物,特别是牛奶,而哌拉西林钠他/唑巴坦钠等青霉素类药物的使用也可能引起GM水平增高,产生假阳性结果;相反,接受抗真菌预防性或经验性治疗则会产生假阴性结果。", "entities": [ { "start_idx": 3, "end_idx": 29, "type": "pro", "entity": "血液标本曲霉半乳甘露聚糖抗原(GM)检测(ELISA)" }, { "start_idx": 55, "end_idx": 58, "type": "pro", "entity": "GM检测" }, { "start_idx": 101, "end_idx": 106, "type": "dru", "entity": "哌拉西林钠他" }, { "start_idx": 108, "end_idx": 111, "type": "dru", "entity": "唑巴坦钠" }, { "start_idx": 113, "end_idx": 115, "type": "dru", "entity": "青霉素" }, { "start_idx": 127, "end_idx": 130, "type": "ite", "entity": "GM水平" }, { "start_idx": 147, "end_idx": 158, "type": "pro", "entity": "抗真菌预防性或经验性治疗" } ] }, { "text": "(3)血液、脑脊液或支气管肺泡灌洗液乳胶凝集法检测隐球菌荚膜多糖抗原:目前临床上诊断隐球菌感染的最重要方法之一,不仅可有助于早期诊断,尚可用于疗效和预后的评估,敏感性和特异性均达到90%以上。", "entities": [ { "start_idx": 3, "end_idx": 22, "type": "pro", "entity": "血液、脑脊液或支气管肺泡灌洗液乳胶凝集法" }, { "start_idx": 25, "end_idx": 33, "type": "mic", "entity": "隐球菌荚膜多糖抗原" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "隐球菌感染" } ] }, { "text": "(七)侵袭性肺部真菌感染的诊断侵袭性肺部真菌感染(invasivepulmonaryfungalinfections,IPFIs)指真菌侵入气管支气管及肺组织引起的感染,不包括真菌寄生和过敏引起的肺部病变。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "侵袭性肺部真菌感染" }, { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "侵袭性肺部真菌感染" }, { "start_idx": 25, "end_idx": 57, "type": "dis", "entity": "invasivepulmonaryfungalinfections" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "IPFIs" }, { "start_idx": 66, "end_idx": 67, "type": "mic", "entity": "真菌" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "气管" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "支气管" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "肺组织" }, { "start_idx": 88, "end_idx": 89, "type": "mic", "entity": "真菌" }, { "start_idx": 93, "end_idx": 94, "type": "dis", "entity": "过敏" }, { "start_idx": 98, "end_idx": 99, "type": "bod", "entity": "肺部" }, { "start_idx": 98, "end_idx": 101, "type": "sym", "entity": "肺部病变" } ] }, { "text": "【IPFIs的诊断】IPFIs的诊断采用分级诊断模式,诊断依据由宿主(危险)因素、临床证据、微生物学证据和组织病理学4部分组成,分为确诊、临床诊断和拟诊三个级别。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "IPFIs" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "IPFIs" } ] }, { "text": "诊断依据:1.宿主和(或)环境(危险)因素(1)基础疾病:早产儿、低出生体重儿、先天发育异常、慢性疾病和重度营养不良等。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "早产" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "低出生体重" }, { "start_idx": 40, "end_idx": 45, "type": "sym", "entity": "先天发育异常" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "慢性疾病" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "重度营养不良" } ] }, { "text": "(2)原发性免疫缺陷病:各类原发性免疫缺陷病,尤其是联合免疫缺陷病、细胞免疫缺陷病和慢性肉芽肿病(CGD)等。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "原发性免疫缺陷病" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "原发性免疫缺陷病" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "联合免疫缺陷病" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "细胞免疫缺陷病" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "慢性肉芽肿病" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "CGD" } ] }, { "text": "(3)继发性免疫功能低下:抗肿瘤药物导致外周血中性粒细胞减少;长期应用广谱抗菌药物、糖皮质激素以及其他免疫抑制剂;骨髓移植和器官移植后以及HIV感染和其他严重病毒感染等。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "继发性免疫功能低下" }, { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "抗肿瘤药物" }, { "start_idx": 20, "end_idx": 27, "type": "bod", "entity": "外周血中性粒细胞" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "外周血中性粒细胞减少" }, { "start_idx": 35, "end_idx": 40, "type": "dru", "entity": "广谱抗菌药物" }, { "start_idx": 42, "end_idx": 46, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 51, "end_idx": 55, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "骨髓移植" }, { "start_idx": 62, "end_idx": 65, "type": "pro", "entity": "器官移植" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "HIV感染" }, { "start_idx": 77, "end_idx": 82, "type": "dis", "entity": "严重病毒感染" } ] }, { "text": "(4)侵入性操作:包括血管内留置导管、留置导尿管、气管插管或气管切开、机械通气、腹膜透析、血液净化和胃肠外营养等。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血管" }, { "start_idx": 16, "end_idx": 17, "type": "equ", "entity": "导管" }, { "start_idx": 21, "end_idx": 23, "type": "equ", "entity": "导尿管" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "气管插管" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "气管切开" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "机械通气" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "血液净化" }, { "start_idx": 50, "end_idx": 54, "type": "pro", "entity": "胃肠外营养" } ] }, { "text": "(5)环境危险因素:免疫功能基本正常的儿童,由于吸入大量真菌孢子,如空调污染、密切接触鸽类以及接触有真菌存在的环境等,超过机体抵抗力而发病,多见于肺隐球菌病,其次是侵袭性肺曲霉菌病。", "entities": [ { "start_idx": 28, "end_idx": 31, "type": "mic", "entity": "真菌孢子" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "肺隐球菌病" }, { "start_idx": 82, "end_idx": 89, "type": "dis", "entity": "侵袭性肺曲霉菌病" } ] }, { "text": "2.临床证据(1)发热、咳嗽和肺部体征经抗菌药物治疗无好转,或好转后再次出现发热、咳嗽和肺部体征。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "发热" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "咳嗽" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺部" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "发热" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "咳嗽" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "肺部" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "肺部体征" } ] }, { "text": "(2)影像学提示肺部病变经抗菌药物治疗无好转或肺部出现新的非原发病的浸润影。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肺部" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "肺部病变" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺部" }, { "start_idx": 23, "end_idx": 36, "type": "sym", "entity": "肺部出现新的非原发病的浸润影" } ] }, { "text": "3.微生物学证据有临床诊断意义的微生物学证据:(1)合格痰标本直接镜检发现菌丝,且培养连续2次以上分离到同种真菌。", "entities": [ { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "痰" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "直接镜检" }, { "start_idx": 54, "end_idx": 55, "type": "mic", "entity": "真菌" } ] }, { "text": "(2)支气管肺泡灌洗液经直接镜检发现菌丝,真菌培养阳性。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "bod", "entity": "支气管肺泡灌洗液" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "直接镜检" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "真菌" } ] }, { "text": "(3)合格痰液或支气管肺泡灌洗液直接镜检或培养发现新生隐球菌。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "痰液" }, { "start_idx": 8, "end_idx": 15, "type": "bod", "entity": "支气管肺泡灌洗液" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "直接镜检" }, { "start_idx": 27, "end_idx": 29, "type": "mic", "entity": "隐球菌" } ] }, { "text": "(4)血液标本曲霉半乳甘露聚糖抗原(GM)检测(ELISA)连续2次吸光度值(I)>0.8或单次I>1.5。", "entities": [ { "start_idx": 3, "end_idx": 22, "type": "pro", "entity": "血液标本曲霉半乳甘露聚糖抗原(GM)检测" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "ELISA" }, { "start_idx": 34, "end_idx": 37, "type": "ite", "entity": "吸光度值" } ] }, { "text": "(5)血液标本真菌细胞壁成分1,3-β-D葡聚糖抗原(G试验)连续2次阳性。", "entities": [ { "start_idx": 3, "end_idx": 25, "type": "pro", "entity": "血液标本真菌细胞壁成分1,3-β-D葡聚糖抗原" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "G试验" } ] }, { "text": "(6)血液隐球菌抗原阳性有确诊意义的微生物学证据:(1)肺组织真菌培养阳性(2)胸腔积液真菌培养阳性(3)血液真菌培养阳性(曲霉和除马尼菲青霉以外的青霉需除外污染)。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血液" }, { "start_idx": 5, "end_idx": 9, "type": "mic", "entity": "隐球菌抗原" }, { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "血液隐球菌抗原阳性" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "肺组织" }, { "start_idx": 28, "end_idx": 36, "type": "sym", "entity": "肺组织真菌培养阳性" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "胸腔积液" }, { "start_idx": 40, "end_idx": 49, "type": "sym", "entity": "胸腔积液真菌培养阳性" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "血液" }, { "start_idx": 53, "end_idx": 60, "type": "sym", "entity": "血液真菌培养阳性" } ] }, { "text": "(4)合格痰液或支气管肺泡灌洗液发现肺孢子菌包囊、滋养体或囊内小体。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "痰液" }, { "start_idx": 8, "end_idx": 15, "type": "bod", "entity": "支气管肺泡灌洗液" }, { "start_idx": 5, "end_idx": 32, "type": "sym", "entity": "痰液或支气管肺泡灌洗液发现肺孢子菌包囊、滋养体或囊内小体" } ] }, { "text": "(5)胸腔积液和血液直接镜检发现新生隐球菌。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "胸腔积液" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "血液" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "直接镜检" }, { "start_idx": 16, "end_idx": 20, "type": "mic", "entity": "新生隐球菌" }, { "start_idx": 3, "end_idx": 20, "type": "sym", "entity": "胸腔积液和血液直接镜检发现新生隐球菌" } ] }, { "text": "4.组织病理学证据肺组织标本进行组织病理学检查发现真菌感染的病理改变以及菌丝或孢子等真菌成分。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肺组织" }, { "start_idx": 36, "end_idx": 37, "type": "mic", "entity": "菌丝" }, { "start_idx": 39, "end_idx": 40, "type": "mic", "entity": "孢子" }, { "start_idx": 42, "end_idx": 43, "type": "mic", "entity": "真菌" }, { "start_idx": 9, "end_idx": 45, "type": "sym", "entity": "肺组织标本进行组织病理学检查发现真菌感染的病理改变以及菌丝或孢子等真菌成分" } ] }, { "text": "诊断标准为:确诊(proven):宿主因素+临床证据+肺组织病理学和(或)有确诊意义的微生物学证据。", "entities": [ { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肺组织" } ] }, { "text": "(八)抗真菌药物治疗包括多烯类、唑类、嘧啶类、棘白菌素类等药物。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "抗真菌药物治疗" } ] }, { "text": "前两类较常用,嘧啶类中的氟胞嘧啶,抗真菌谱窄,仅对酵母菌有较高的抗菌活性,但与其他抗真菌药有协同作用,生物利用度较高。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "氟胞嘧啶" }, { "start_idx": 25, "end_idx": 27, "type": "mic", "entity": "酵母菌" } ] }, { "text": "此外,还有抗真菌中草药,如大蒜素注射液等。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "抗真菌中草药" }, { "start_idx": 13, "end_idx": 18, "type": "dru", "entity": "大蒜素注射液" } ] }, { "text": "真菌细胞壁内含有固醇的细胞膜,是两性霉素B、制霉菌素、丙烯胺类和唑类抗真菌药物的作用场所。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "真菌细胞壁" }, { "start_idx": 16, "end_idx": 20, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "制霉菌素" }, { "start_idx": 32, "end_idx": 38, "type": "dru", "entity": "唑类抗真菌药物" } ] }, { "text": "1.多烯类抗生素主要有两性霉素B、制霉菌素等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "多烯类抗生素" }, { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "制霉菌素" } ] }, { "text": "儿童剂量为0.5~1mg/(kg•d);静脉注射时常见的药物反应有:高热、寒战、恶心、呕吐、食欲缺乏、静脉炎、低血压及心脏毒性;长期应用会出现低钾血症、低镁血症、贫血和肾毒性;大剂量时可致肾小管坏死、钙化。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "静脉注射" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "高热" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "寒战" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "恶心" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "呕吐" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 51, "end_idx": 53, "type": "sym", "entity": "静脉炎" }, { "start_idx": 55, "end_idx": 57, "type": "sym", "entity": "低血压" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "心脏" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "心脏毒性" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "低钾血症" }, { "start_idx": 76, "end_idx": 79, "type": "sym", "entity": "低镁血症" }, { "start_idx": 81, "end_idx": 82, "type": "sym", "entity": "贫血" }, { "start_idx": 84, "end_idx": 86, "type": "sym", "entity": "肾毒性" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "肾小管" }, { "start_idx": 94, "end_idx": 98, "type": "sym", "entity": "肾小管坏死" }, { "start_idx": 100, "end_idx": 101, "type": "sym", "entity": "钙化" } ] }, { "text": "而两性霉素B脂质体既能降低毒性又不降低抗菌作用,已经试用于儿童。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dru", "entity": "两性霉素B脂质体" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "降低毒性" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "不降低抗菌作用" } ] }, { "text": "两性霉素B脂质复合物3~5mg/(kg•d)。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dru", "entity": "两性霉素B脂质复合物" } ] }, { "text": "(2)制霉菌素:具广谱抗真菌作用,对念珠菌属的抗菌活性尤为明显,新型隐球菌和曲霉菌对该药也敏感。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "制霉菌素" }, { "start_idx": 18, "end_idx": 20, "type": "mic", "entity": "念珠菌" }, { "start_idx": 34, "end_idx": 36, "type": "mic", "entity": "隐球菌" }, { "start_idx": 38, "end_idx": 40, "type": "mic", "entity": "曲霉菌" } ] }, { "text": "2.唑类抗生素包含咪唑类和三唑类,能干扰真菌细胞膜的合成,常用的有氟康唑、咪康唑、克霉唑、酮康唑、伊曲康唑、伏立康唑等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "唑类抗生素" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "咪唑类" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "三唑类" }, { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "真菌细胞膜" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "氟康唑" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "咪康唑" }, { "start_idx": 41, "end_idx": 43, "type": "dru", "entity": "克霉唑" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "酮康唑" }, { "start_idx": 49, "end_idx": 52, "type": "dru", "entity": "伊曲康唑" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "伏立康唑" } ] }, { "text": "口服制剂6~8mg/(kg•d),分2次服用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "口服" } ] }, { "text": "2~12岁:每次7mg/kg,每12小时1次,静脉注射;或第1天6mg/kg,每12小时1次,静脉注射,随后每次4mg/kg,每12小时1次,静脉注射。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "静脉注射" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "静脉注射" }, { "start_idx": 71, "end_idx": 74, "type": "pro", "entity": "静脉注射" } ] }, { "text": "口服剂量:<40kg,100mg/次,每12小时1次;>40kg,200mg/次,每12小时1次。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "口服" } ] }, { "text": "3.棘白菌素类卡泊芬净:适应证为念珠菌和曲霉感染,对隐球菌、镰刀霉菌属以及毛霉菌属无活性。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dru", "entity": "棘白菌素类卡泊芬净" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "念珠菌和曲霉感染" }, { "start_idx": 26, "end_idx": 28, "type": "mic", "entity": "隐球菌" }, { "start_idx": 30, "end_idx": 33, "type": "mic", "entity": "镰刀霉菌" }, { "start_idx": 37, "end_idx": 39, "type": "mic", "entity": "毛霉菌" } ] }, { "text": "抗真菌治疗的时间长短,因病情而异,患侵袭性肺部真菌病的患儿一般均在免疫功能低下的情况下发病,给药时间不宜过短,一般要6~12周,甚至更长,一般治疗至临床证候消失,影像学示病变基本吸收。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "抗真菌治疗" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "侵袭性肺部真菌病" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "免疫功能低下" } ] }, { "text": "第二节临床资料的整理和分析(一)资料归纳将病史问诊、体格检查和各项辅助检查的结果进行整理,去粗存精,有条理、系统地进行归类并列出条目。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "体格检查" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "辅助检查" } ] }, { "text": "要求有高度的概括性,围绕主诉、突出重点,将主要症状的特点、体格检查阳性发现及重要的阴性体征、实验室检查的异常结果列出条目。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "体格检查" }, { "start_idx": 46, "end_idx": 50, "type": "pro", "entity": "实验室检查" } ] }, { "text": "以下是一病例临床归纳的特点:1.男性,1岁;2.持续发热2周伴不规则皮疹;3.咽充血双侧扁桃体Ⅱ度肿大颈部浅表淋巴结轻度肿大肝中度肿大,脾轻度肿大;6.外周血象白细胞总数正常淋巴细胞为主,轻度贫血,血小板计数正常尿常规正常;7.一般情况可,无头痛呕吐,无咳嗽气急,无腹痛、腹泻,无尿频、尿急、尿痛。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "发热" }, { "start_idx": 30, "end_idx": 35, "type": "sym", "entity": "伴不规则皮疹" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "咽" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "咽充血" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "扁桃体" }, { "start_idx": 42, "end_idx": 50, "type": "sym", "entity": "双侧扁桃体Ⅱ度肿大" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "颈部" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "淋巴结" }, { "start_idx": 51, "end_idx": 61, "type": "sym", "entity": "颈部浅表淋巴结轻度肿大" }, { "start_idx": 62, "end_idx": 72, "type": "sym", "entity": "肝中度肿大,脾轻度肿大" }, { "start_idx": 80, "end_idx": 84, "type": "ite", "entity": "白细胞总数" }, { "start_idx": 76, "end_idx": 86, "type": "sym", "entity": "外周血象白细胞总数正常" }, { "start_idx": 87, "end_idx": 90, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 94, "end_idx": 97, "type": "sym", "entity": "轻度贫血" }, { "start_idx": 99, "end_idx": 103, "type": "ite", "entity": "血小板计数" }, { "start_idx": 99, "end_idx": 105, "type": "sym", "entity": "血小板计数正常" }, { "start_idx": 106, "end_idx": 110, "type": "sym", "entity": "尿常规正常" }, { "start_idx": 121, "end_idx": 122, "type": "sym", "entity": "头痛" }, { "start_idx": 123, "end_idx": 124, "type": "sym", "entity": "呕吐" }, { "start_idx": 127, "end_idx": 130, "type": "sym", "entity": "咳嗽气急" }, { "start_idx": 133, "end_idx": 134, "type": "sym", "entity": "腹痛" }, { "start_idx": 136, "end_idx": 137, "type": "sym", "entity": "腹泻" }, { "start_idx": 140, "end_idx": 141, "type": "sym", "entity": "尿频" }, { "start_idx": 143, "end_idx": 144, "type": "sym", "entity": "尿急" }, { "start_idx": 146, "end_idx": 147, "type": "sym", "entity": "尿痛" } ] }, { "text": "而这个初步印象会在接下来的问诊、体格检查过程中起一定的导向作用。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "体格检查" } ] }, { "text": "以此为基础,进一步收集临床资料如辅助检查,尤其是一些具有特异性诊断价值的项目,以确诊或排除。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "辅助检查" } ] }, { "text": "在儿科疾病诊断的临床思维过程中,具体还应注意下列问题:1.首先考虑常见病儿科疾病谱中,先天性、遗传性和感染性疾病占较大比例,在诊断时应首先考虑。", "entities": [ { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "如遇发热待查患儿,病因有很多,如感染、结缔组织病、恶性肿瘤及血液病、变态反应性疾病、体温中枢病变或调节失常、组织破坏与吸收、代谢和内分泌失调等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "发热" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "感染" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "血液病" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "变态反应性疾病" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "体温中枢病变" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "调节失常" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "组织破坏" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "内分泌失调" } ] }, { "text": "但婴幼儿由于免疫功能低下,以感染性疾病最为常见,故诊断思路应首先想到感染性疾病。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "免疫功能低下" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "在病原方面,也应多考虑常见的细菌或病毒,其次再考虑支原体、衣原体、真菌、寄生虫。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "mic", "entity": "细菌" }, { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "病毒" }, { "start_idx": 25, "end_idx": 27, "type": "mic", "entity": "支原体" }, { "start_idx": 29, "end_idx": 31, "type": "mic", "entity": "衣原体" }, { "start_idx": 33, "end_idx": 34, "type": "mic", "entity": "真菌" }, { "start_idx": 36, "end_idx": 38, "type": "mic", "entity": "寄生虫" } ] }, { "text": "然后通过一系列的实验室检查,如外周血象、C-反应蛋白、血培养、血清学检查、分子生物学等方法来证实推断。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "实验室检查" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "外周血象" }, { "start_idx": 20, "end_idx": 25, "type": "ite", "entity": "C-反应蛋白" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "血培养" }, { "start_idx": 31, "end_idx": 35, "type": "pro", "entity": "血清学检查" } ] }, { "text": "如惊厥是儿科的常见症状之一,如果发生于新生儿,首先考虑围生期因素或代谢异常,如缺氧缺血性脑病、颅内出血、低血糖、低血钙等。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "惊厥" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "缺氧缺血性脑病" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "颅内出血" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "低血糖" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "低血钙" } ] }, { "text": "如果发生于小婴儿,首先考虑颅内感染、热性惊厥等。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "颅内感染" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "热性惊厥" } ] }, { "text": "如果是较大儿童,多考虑脑炎、癫痫等。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "脑炎" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "癫痫" } ] }, { "text": "3.切忌生搬硬套有些疾病缺乏特异性的实验室检查,而依靠一些非特异性的临床及辅助检查指标来进行诊断。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "实验室检查" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "辅助检查" } ] }, { "text": "4.重视典型临床表现的积累图2-1发绀的鉴别诊断有些疾病凭外观直觉就立即能作出诊断,如21-三体综合征有特殊的面容,过敏性紫癜有典型的皮肤表现;另外可以通过关联思维来获得诊断,如新生儿有阴茎短小并伴有低血糖,很容易想到先天性垂体功能低下的诊断。", "entities": [ { "start_idx": 93, "end_idx": 96, "type": "dis", "entity": "阴茎短小" }, { "start_idx": 100, "end_idx": 102, "type": "dis", "entity": "低血糖" }, { "start_idx": 109, "end_idx": 117, "type": "dis", "entity": "先天性垂体功能低下" } ] }, { "text": "它将关键的临床表现和辅助检查按顺序及逻辑关系进行排列,形成流程表。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "辅助检查" } ] }, { "text": "6.注意诊断的全面性及完整性诊断必须全面完整的诊断应包括主要诊断:系统器官定位(肺、肝)、性质(炎症、出血)、病程(如急性、慢性)、可能的病原(细菌性、支原体)、病理(如支气管肺炎、大叶性肺炎)、病情程度(轻、重)以及并发症(脓胸、气胸)、功能诊断(如呼吸衰竭)等。", "entities": [ { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "肺" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "肝" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "炎症" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "出血" }, { "start_idx": 76, "end_idx": 78, "type": "mic", "entity": "支原体" }, { "start_idx": 85, "end_idx": 89, "type": "dis", "entity": "支气管肺炎" }, { "start_idx": 91, "end_idx": 95, "type": "dis", "entity": "大叶性肺炎" }, { "start_idx": 113, "end_idx": 114, "type": "dis", "entity": "脓胸" }, { "start_idx": 116, "end_idx": 117, "type": "dis", "entity": "气胸" }, { "start_idx": 126, "end_idx": 129, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "有时还有次要诊断如贫血、血小板减少症等,都应完整列出。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "贫血" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "血小板减少症" } ] }, { "text": "二、阻力呼吸系统阻力从物理性质可分为三种:弹性阻力、黏性阻力、惯性阻力,三者之和为呼吸总阻抗;呼吸总阻抗可用强迫振荡法测定。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "ite", "entity": "呼吸系统阻力" }, { "start_idx": 21, "end_idx": 24, "type": "ite", "entity": "弹性阻力" }, { "start_idx": 26, "end_idx": 29, "type": "ite", "entity": "黏性阻力" }, { "start_idx": 31, "end_idx": 34, "type": "ite", "entity": "惯性阻力" }, { "start_idx": 41, "end_idx": 45, "type": "ite", "entity": "呼吸总阻抗" }, { "start_idx": 47, "end_idx": 51, "type": "ite", "entity": "呼吸总阻抗" }, { "start_idx": 54, "end_idx": 58, "type": "ite", "entity": "强迫振荡法" } ] }, { "text": "按部位可分为:气道阻力、肺阻力和胸廓阻力。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "气道阻力" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "肺阻力" }, { "start_idx": 16, "end_idx": 19, "type": "ite", "entity": "胸廓阻力" } ] }, { "text": "气道阻力是指气道的黏性阻力,是单位流量所需的压力差(R=△P/V)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "气道阻力" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "气道" } ] }, { "text": "气道阻力取决于管径大小和气体流速,管道气流与管腔半径的4次方成反比,故小儿气道阻力大于成人。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "气道阻力" }, { "start_idx": 37, "end_idx": 40, "type": "ite", "entity": "气道阻力" } ] }, { "text": "成人气道阻力一般在1~3cmH2O/(L•s),婴幼儿气道较狭窄,其阻力较高,约为成人的10倍。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "气道阻力" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "气道" } ] }, { "text": "气道管径随发育而增大,阻力随年龄而递减。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "气道" } ] }, { "text": "婴幼儿肺炎时,气道管腔黏膜肿胀,分泌物增加,支气管痉挛,故管腔更为狭小,气道阻力增加。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肺炎" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "气道管腔" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "气道管腔黏膜肿胀" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "分泌物增加" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "支气管" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "支气管痉挛" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "管腔" }, { "start_idx": 36, "end_idx": 39, "type": "ite", "entity": "气道阻力" } ] }, { "text": "七、pH监测采用柔软的pH电极(儿科常用单晶锑电极),放置在食管或胃中监测pH,数据存储在可携带的pH记录仪上,可持续监测24~96小时,由电脑进行数据处理。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "pH监测" }, { "start_idx": 11, "end_idx": 14, "type": "equ", "entity": "pH电极" }, { "start_idx": 16, "end_idx": 17, "type": "dep", "entity": "儿科" }, { "start_idx": 20, "end_idx": 24, "type": "equ", "entity": "单晶锑电极" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "食管" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "胃" }, { "start_idx": 49, "end_idx": 53, "type": "equ", "entity": "pH记录仪" }, { "start_idx": 70, "end_idx": 71, "type": "equ", "entity": "电脑" } ] }, { "text": "目前常用DigitrapperMK型pH监测仪。", "entities": [ { "start_idx": 4, "end_idx": 22, "type": "equ", "entity": "DigitrapperMK型pH监测仪" } ] }, { "text": "(一)食管pH监测检查前禁食4~6小时,检查时将pH电极准确置于下端食道括约肌上缘以上3cm处,受检时照常进食,但忌酸性饮料和食物,并记录进餐、体位变化的起止时间和症状发生的时间,不限制活动,力求接近生理状态。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "食管pH监测" }, { "start_idx": 24, "end_idx": 27, "type": "equ", "entity": "pH电极" }, { "start_idx": 32, "end_idx": 40, "type": "bod", "entity": "下端食道括约肌上缘" } ] }, { "text": "食管pH监测主要用于胃食管反流病(GERD)的诊断,判断GERD治疗效果。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "食管pH监测" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "胃食管反流病" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "GERD" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "GERD" } ] }, { "text": "可以发现反流,区分生理性反流还是病理性反流,了解反流与症状、体位进食的关系。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "反流" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "生理性反流" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "病理性反流" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "反流" } ] }, { "text": "(二)胃pH监测将pH电极置于下端食管括约肌下5cm,主要监测胃pH值平均数,胃pH中位数,胃pH值>1、>2、>3、>4的时间百分比,以及胃pH值密度分布。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "胃pH监测" }, { "start_idx": 9, "end_idx": 12, "type": "equ", "entity": "pH电极" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "下端食管括约肌" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "胃" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "胃" }, { "start_idx": 46, "end_idx": 49, "type": "ite", "entity": "胃pH值" }, { "start_idx": 70, "end_idx": 70, "type": "bod", "entity": "胃" } ] }, { "text": "主要用于评价酸相关性疾病的疗效,以及探讨消化性溃疡及胃泌素瘤等疾病的发病机制和愈合过程与胃酸变化的关系,对检测十二指肠胃反流有帮助。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "胃泌素瘤" }, { "start_idx": 55, "end_idx": 61, "type": "dis", "entity": "十二指肠胃反流" } ] }, { "text": "第二节范可尼综合征Fanconi(范可尼)于1931年首先描述1例小儿有蛋白尿、非糖尿病性葡萄糖尿、生长迟缓伴低血磷性佝偻病,此综合征因此得名。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "范可尼综合征" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "范可尼" }, { "start_idx": 36, "end_idx": 38, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 40, "end_idx": 48, "type": "sym", "entity": "非糖尿病性葡萄糖尿" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "生长迟缓" }, { "start_idx": 55, "end_idx": 61, "type": "dis", "entity": "低血磷性佝偻病" } ] }, { "text": "本病以多种肾小管功能紊乱为特征,导致氨基酸、葡萄糖、磷酸盐、碳酸氢盐和其他由近端或远端肾小管处的有机物或无机物从尿中丢失过多,因而出现酸中毒、低磷酸盐血症、低钙血症、脱水、佝偻病、骨质疏松以及生长过缓等表现。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "肾小管功能紊乱" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "氨基酸" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "磷酸盐" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "碳酸氢盐" }, { "start_idx": 38, "end_idx": 45, "type": "bod", "entity": "近端或远端肾小管" }, { "start_idx": 18, "end_idx": 61, "type": "sym", "entity": "氨基酸、葡萄糖、磷酸盐、碳酸氢盐和其他由近端或远端肾小管处的有机物或无机物从尿中丢失过多" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "酸中毒" }, { "start_idx": 71, "end_idx": 76, "type": "dis", "entity": "低磷酸盐血症" }, { "start_idx": 78, "end_idx": 81, "type": "dis", "entity": "低钙血症" }, { "start_idx": 83, "end_idx": 84, "type": "sym", "entity": "脱水" }, { "start_idx": 86, "end_idx": 88, "type": "dis", "entity": "佝偻病" }, { "start_idx": 90, "end_idx": 93, "type": "dis", "entity": "骨质疏松" }, { "start_idx": 96, "end_idx": 99, "type": "sym", "entity": "生长过缓" } ] }, { "text": "【病因和分类】幼儿大多与遗传有关,年长儿多继发于免疫性疾病、毒物或药物中毒以及各种肾脏病。", "entities": [ { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "免疫性疾病" }, { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "毒物或药物中毒" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "肾脏病" } ] }, { "text": "(一)原发性(原因不明或无全身性疾病)包括遗传性[常染色体显性(AD)、常染色体隐性(AR)及X连锁隐性(XLR)]、散发性及特殊型(即刷状缘缺失型)。", "entities": [ { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "常染色体显性" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "AD" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "常染色体隐性" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "AR" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "X连锁隐性" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "XLR" }, { "start_idx": 68, "end_idx": 73, "type": "dis", "entity": "刷状缘缺失型" } ] }, { "text": "(二)继发性(症状型)1.先天性代谢障碍(1)氨基酸代谢障碍:①胱氨酸病(常染色体隐性,AR);②酪氨酸血症Ⅰ型(AR);③Busby综合征(AR);④Ludersheldon综合征(AD)。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "先天性代谢障碍" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "氨基酸代谢障碍" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "胱氨酸病" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "常染色体隐性" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "AR" }, { "start_idx": 49, "end_idx": 55, "type": "dis", "entity": "酪氨酸血症Ⅰ型" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "AR" }, { "start_idx": 62, "end_idx": 69, "type": "dis", "entity": "Busby综合征" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "AR" }, { "start_idx": 76, "end_idx": 90, "type": "dis", "entity": "Ludersheldon综合征" }, { "start_idx": 92, "end_idx": 93, "type": "dis", "entity": "AD" } ] }, { "text": "(2)碳水化合物代谢障碍:①糖原累积病Ⅰ型(Fanconi-Bickel综合征,AR);②半乳糖血症(AR);③遗传性果糖不耐受症(AR)。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "碳水化合物代谢障碍" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "糖原累积病Ⅰ型" }, { "start_idx": 22, "end_idx": 38, "type": "dis", "entity": "Fanconi-Bickel综合征" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "AR" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "AR" }, { "start_idx": 56, "end_idx": 64, "type": "dis", "entity": "遗传性果糖不耐受症" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "AR" } ] }, { "text": "(3)其他:①Lowe综合征(XLR);②肝豆状核变性(AR);③细胞色素C氧化酶缺陷(AR);④Dent病(家族性近端肾小管疾病,XLR);⑤Pearson综合征;⑥Wilson病;⑦维生素B12缺乏。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "Lowe综合征" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "XLR" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "肝豆状核变性" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "AR" }, { "start_idx": 33, "end_idx": 40, "type": "bod", "entity": "细胞色素C氧化酶" }, { "start_idx": 33, "end_idx": 42, "type": "sym", "entity": "细胞色素C氧化酶缺陷" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "AR" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "Dent病" }, { "start_idx": 55, "end_idx": 64, "type": "dis", "entity": "家族性近端肾小管疾病" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "XLR" }, { "start_idx": 72, "end_idx": 81, "type": "dis", "entity": "Pearson综合征" }, { "start_idx": 84, "end_idx": 90, "type": "dis", "entity": "Wilson病" }, { "start_idx": 93, "end_idx": 104, "type": "bod", "entity": "维生素B12" }, { "start_idx": 93, "end_idx": 106, "type": "sym", "entity": "维生素B12缺乏" } ] }, { "text": "2.获得性疾病如:①多发性骨髓瘤;②肾病综合征;③肾移植;④肿瘤;⑤糖尿病;⑥急、慢性间质性肾炎;⑦急性肾小管坏死;⑧营养不良;⑨巴尔干肾病;⑩严重低钾血症。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "多发性骨髓瘤" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "肾移植" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "肿瘤" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "糖尿病" }, { "start_idx": 39, "end_idx": 47, "type": "dis", "entity": "急、慢性间质性肾炎" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "急性肾小管坏死" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "营养不良" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "巴尔干肾病" }, { "start_idx": 72, "end_idx": 77, "type": "dis", "entity": "严重低钾血症" } ] }, { "text": "3.药物损伤及中毒如:①重金属(汞、钠、铅及镉);②化学毒剂马来酸、来苏儿、甲苯、甲酚及硝苯等;③过期四环素及丙酸;④顺铂、异环磷酰胺(Ifosfamide)、氨基糖苷类抗生素及维生素中毒;⑤雷米替丁、西咪替丁及中草药如马兜铃肾损害等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "药物损伤" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "中毒" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "四环素" }, { "start_idx": 55, "end_idx": 56, "type": "dru", "entity": "丙酸" }, { "start_idx": 59, "end_idx": 93, "type": "dis", "entity": "顺铂、异环磷酰胺(Ifosfamide)、氨基糖苷类抗生素及维生素中毒" }, { "start_idx": 96, "end_idx": 99, "type": "dru", "entity": "雷米替丁" }, { "start_idx": 101, "end_idx": 104, "type": "dru", "entity": "西咪替丁" }, { "start_idx": 106, "end_idx": 108, "type": "dru", "entity": "中草药" }, { "start_idx": 110, "end_idx": 115, "type": "dis", "entity": "马兜铃肾损害" } ] }, { "text": "【发病机制】本病发病机制尚未完全清楚,有以下几种可能:①内流缺陷,管腔内向组织内流减少,见于刷状缘缺失型;②细胞内回漏到肾小管腔增加,如马来酸中毒型;③通过基底侧细胞膜回流减少,致细胞内物质堆积;影响回吸收,如Fanconi-Bickel综合征;④从血液向细胞灌注增加,通过细胞紧密连接处反流管腔增加,如细胞色素C氧化酶缺乏型。", "entities": [ { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "内流缺陷" }, { "start_idx": 33, "end_idx": 42, "type": "sym", "entity": "管腔内向组织内流减少" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "刷状缘缺失型" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "肾小管腔" }, { "start_idx": 54, "end_idx": 65, "type": "sym", "entity": "细胞内回漏到肾小管腔增加" }, { "start_idx": 68, "end_idx": 73, "type": "dis", "entity": "马来酸中毒型" }, { "start_idx": 78, "end_idx": 87, "type": "sym", "entity": "基底侧细胞膜回流减少" }, { "start_idx": 90, "end_idx": 96, "type": "sym", "entity": "细胞内物质堆积" }, { "start_idx": 105, "end_idx": 121, "type": "dis", "entity": "Fanconi-Bickel综合征" }, { "start_idx": 125, "end_idx": 133, "type": "sym", "entity": "血液向细胞灌注增加" }, { "start_idx": 135, "end_idx": 149, "type": "sym", "entity": "通过细胞紧密连接处反流管腔增加" }, { "start_idx": 152, "end_idx": 162, "type": "dis", "entity": "细胞色素C氧化酶缺乏型" } ] }, { "text": "肾小管膜的输送异常在病理组织学检查中未见特异性表现。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "肾小管膜的输送异常" } ] }, { "text": "有实验提示本征的细胞内ATP活性的转运功能不全是由于磷酸盐耗竭,引起细胞内腺嘌呤核苷酸降解,因而发生ATP消耗。", "entities": [ { "start_idx": 8, "end_idx": 22, "type": "sym", "entity": "细胞内ATP活性的转运功能不全" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "磷酸盐耗竭" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "腺嘌呤核苷酸" }, { "start_idx": 34, "end_idx": 44, "type": "sym", "entity": "细胞内腺嘌呤核苷酸降解" } ] }, { "text": "图12-1范可尼综合征病理生理与临床表现【临床表现】本病临床表现取决于肾小管功能障碍的类型和程度。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "范可尼综合征" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "肾小管功能障碍" } ] }, { "text": "全氨基酸尿、糖尿以及高磷酸盐尿导致低磷血症为本症的三大特征,但不完全性Fanconi综合征不是全部具备上述三个特征,往往只具备其中1、2项。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "全氨基酸尿" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "糖尿" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "高磷酸盐尿" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "低磷血症" }, { "start_idx": 31, "end_idx": 44, "type": "dis", "entity": "不完全性Fanconi综合征" } ] }, { "text": "(一)原发性Fanconi综合征1.婴儿型也称急性型,特点有:①起病早,6~12月龄发病;②常因烦渴、多饮、多尿、脱水、消瘦、呕吐、便秘及无力而就诊;③生长迟缓及发育障碍,出现抗维生素D佝偻病及营养不良、骨质疏松甚至骨折等表现;④肾性全氨基酸尿,但血浆氨基酸正常;⑤低血钾,低血磷,碱性磷酸酶活性增高,高氯血症性代谢性酸中毒,尿中可滴定酸及可减少,尿糖微量或增多,血糖正常;⑥预后较差,可死于尿毒症性酸中毒或继发感染。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "原发性Fanconi综合征" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "烦渴" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "多饮" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "多尿" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "脱水" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "消瘦" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "呕吐" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "便秘" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "无力" }, { "start_idx": 76, "end_idx": 84, "type": "sym", "entity": "生长迟缓及发育障碍" }, { "start_idx": 88, "end_idx": 95, "type": "dis", "entity": "抗维生素D佝偻病" }, { "start_idx": 97, "end_idx": 100, "type": "sym", "entity": "营养不良" }, { "start_idx": 102, "end_idx": 105, "type": "sym", "entity": "骨质疏松" }, { "start_idx": 108, "end_idx": 109, "type": "sym", "entity": "骨折" }, { "start_idx": 124, "end_idx": 130, "type": "sym", "entity": "血浆氨基酸正常" }, { "start_idx": 133, "end_idx": 135, "type": "sym", "entity": "低血钾" }, { "start_idx": 137, "end_idx": 139, "type": "sym", "entity": "低血磷" }, { "start_idx": 141, "end_idx": 149, "type": "sym", "entity": "碱性磷酸酶活性增高" }, { "start_idx": 151, "end_idx": 161, "type": "dis", "entity": "高氯血症性代谢性酸中毒" }, { "start_idx": 174, "end_idx": 180, "type": "dis", "entity": "尿糖微量或增多" }, { "start_idx": 182, "end_idx": 185, "type": "dis", "entity": "血糖正常" }, { "start_idx": 196, "end_idx": 202, "type": "dis", "entity": "尿毒症性酸中毒" }, { "start_idx": 206, "end_idx": 207, "type": "dis", "entity": "感染" } ] }, { "text": "2.幼儿型起病较晚(2岁以后),症状较婴儿型轻,以抗维生素D佝偻病及生长迟缓为最突出表现。", "entities": [ { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "抗维生素D佝偻病" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "生长迟缓" } ] }, { "text": "3.成人型特点有:①10~20岁或更晚发病;②多种肾小管功能障碍;如糖尿、全氨基酸尿、高磷酸盐尿、低血钾及高氯酸中毒;③软骨病往往是突出表现;④晚期可出现肾衰竭。", "entities": [ { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "肾小管功能障碍" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "糖尿" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "全氨基酸尿" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "高磷酸盐尿" }, { "start_idx": 49, "end_idx": 51, "type": "sym", "entity": "低血钾" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "高氯酸中毒" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "软骨病" }, { "start_idx": 77, "end_idx": 79, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "(二)继发性Fanconi综合征因病因不同表现有所不同(表12-4)。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "继发性Fanconi综合征" } ] }, { "text": "表12-4常见范可尼综合征的特征及鉴别注:PAH,对氨基马尿酸;+,轻度受损;++,中度受损;+++,严重受损;AD,常染色体显性;AR,常染色体隐性;XLR,X连锁隐性遗传【诊断与鉴别诊断】本病无特异诊断试验,根据生长迟缓、佝偻病、多尿及脱水、酸中毒、电解质紊乱相应的临床表现,血生化检查见低血钾、低血磷、低血钠、低血氯性酸中毒、高AKP、低血尿酸、糖尿而血糖正常,全氨基酸尿、尿pH低而尿氨和可滴定酸低,X线检查有骨质疏松及佝偻病表现均有助于骨质疏松注意询问家族史。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "范可尼综合征" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "AD" }, { "start_idx": 59, "end_idx": 64, "type": "sym", "entity": "常染色体显性" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "AR" }, { "start_idx": 69, "end_idx": 74, "type": "sym", "entity": "常染色体隐性" }, { "start_idx": 76, "end_idx": 78, "type": "sym", "entity": "XLR" }, { "start_idx": 80, "end_idx": 86, "type": "sym", "entity": "X连锁隐性遗传" }, { "start_idx": 108, "end_idx": 111, "type": "sym", "entity": "生长迟缓" }, { "start_idx": 113, "end_idx": 115, "type": "sym", "entity": "佝偻病" }, { "start_idx": 117, "end_idx": 118, "type": "sym", "entity": "多尿" }, { "start_idx": 120, "end_idx": 121, "type": "sym", "entity": "脱水" }, { "start_idx": 123, "end_idx": 125, "type": "dis", "entity": "酸中毒" }, { "start_idx": 127, "end_idx": 131, "type": "sym", "entity": "电解质紊乱" }, { "start_idx": 146, "end_idx": 148, "type": "sym", "entity": "低血钾" }, { "start_idx": 150, "end_idx": 152, "type": "sym", "entity": "低血磷" }, { "start_idx": 154, "end_idx": 156, "type": "sym", "entity": "低血钠" }, { "start_idx": 158, "end_idx": 164, "type": "dis", "entity": "低血氯性酸中毒" }, { "start_idx": 166, "end_idx": 169, "type": "sym", "entity": "高AKP" }, { "start_idx": 171, "end_idx": 174, "type": "sym", "entity": "低血尿酸" }, { "start_idx": 176, "end_idx": 177, "type": "sym", "entity": "糖尿" }, { "start_idx": 184, "end_idx": 188, "type": "sym", "entity": "全氨基酸尿" }, { "start_idx": 190, "end_idx": 193, "type": "sym", "entity": "尿pH低" }, { "start_idx": 195, "end_idx": 202, "type": "sym", "entity": "尿氨和可滴定酸低" }, { "start_idx": 204, "end_idx": 207, "type": "pro", "entity": "X线检查" }, { "start_idx": 209, "end_idx": 212, "type": "sym", "entity": "骨质疏松" }, { "start_idx": 214, "end_idx": 216, "type": "dis", "entity": "佝偻病" }, { "start_idx": 223, "end_idx": 226, "type": "dis", "entity": "骨质疏松" } ] }, { "text": "由于多种类型Fanconi综合征可通过特异性治疗及对症处理取得良好疗效,因此病因诊断尤为重要,表12-4为各种不同病因下Fanconi综合征的特点和区别。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "Fanconi综合征" }, { "start_idx": 60, "end_idx": 69, "type": "dis", "entity": "Fanconi综合征" } ] }, { "text": "【治疗】(一)病因治疗对已了解代谢缺陷类型的继发性Fanconi综合征,可进行特异性治疗。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "代谢缺陷" }, { "start_idx": 22, "end_idx": 34, "type": "dis", "entity": "继发性Fanconi综合征" } ] }, { "text": "通过饮食疗法减少或避免有毒代谢产物积聚的疾病有半乳糖血症、遗传性果糖不耐受以及酪氨酸血症Ⅰ型。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "有毒代谢产物积聚" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "遗传性果糖不耐受" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "酪氨酸血症Ⅰ型" } ] }, { "text": "通过促进排泄治疗的疾病有Wilson病和重金属中毒。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "Wilson病" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "重金属中毒" } ] }, { "text": "由药物引起的Fanconi综合征,清除体内药物可纠正肾小管功能障碍。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "Fanconi综合征" } ] }, { "text": "对于由肾脏疾病或全身疾病后引起的Fanconi综合征则相应针对原发病治疗。", "entities": [ { "start_idx": 16, "end_idx": 25, "type": "dis", "entity": "Fanconi综合征" } ] }, { "text": "。(二)对症治疗1.纠正酸中毒根据肾小管受损的程度给予碱性药物,剂量2~10mmol/(kg•d),可采用碳酸氢钠或者枸橼酸钠钾合剂,全天剂量分4~5次口服,然后根据血中浓度调整剂量。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "纠正酸中毒" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "肾小管受损" }, { "start_idx": 53, "end_idx": 56, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 59, "end_idx": 63, "type": "dru", "entity": "枸橼酸钠钾" } ] }, { "text": "应注意同时补钾,如碱性药物用量过大,可合用氢氯噻嗪,促进回吸收。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "氢氯噻嗪" } ] }, { "text": "2.纠正低磷血症口服中性磷酸盐以纠正低磷血症,剂量为1~3g/d,分次服,每4~5小时用药1次,不良反应有胃肠不适和腹泻,减少用量可减轻上述症状。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "纠正低磷血症" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "中性磷酸盐" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "纠正低磷血症" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "胃肠" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "胃肠不适" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "腹泻" } ] }, { "text": "在部分患者,应用磷酸盐可加重低钙血症,诱发甲状旁腺功能亢进,可口服钙剂和维生素D预防。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "磷酸盐" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "低钙血症" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "加重低钙血症" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "甲状旁腺功能亢进" }, { "start_idx": 31, "end_idx": 39, "type": "pro", "entity": "口服钙剂和维生素D" } ] }, { "text": "中性磷酸盐配方:Na2HPO4•7H2O145g,NaH2PO4•H2O18.2g,加水至1000ml,每1000ml供磷2g。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "中性磷酸盐" } ] }, { "text": "3.其他应补充血容量,防脱水,纠正低钾血症。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "补充血容量" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "防脱水" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "纠正低钾血症" } ] }, { "text": "对于低尿酸血症、氨基酸尿、糖尿及蛋白尿,目前尚缺乏有效的治疗方法。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "低尿酸血症" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "氨基酸尿" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "糖尿" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "蛋白尿" } ] }, { "text": "肾功能不全者,则酌情采用保守式肾脏替代治疗。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "肾功能" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "肾功能不全" }, { "start_idx": 12, "end_idx": 20, "type": "pro", "entity": "保守式肾脏替代治疗" } ] }, { "text": "【预后】本病预后取决于所累及的脏器以及治疗开始的早晚、持续性和原发病等因素,严重患者最终多死于严重水、电解质紊乱及肾衰竭。", "entities": [ { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "严重水、电解质紊乱" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "冷抗体型免疫性溶血性贫血可分为冷凝集素病和阵发性寒冷性血红蛋白尿两种。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dis", "entity": "冷抗体型免疫性溶血性贫血" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "冷凝集素病" }, { "start_idx": 21, "end_idx": 31, "type": "dis", "entity": "阵发性寒冷性血红蛋白尿" } ] }, { "text": "1.冷凝集素病常继发于肺炎支原体、EB病毒感染或继发于淋巴增生性疾病,产生识别I/i系统寡糖抗原的抗体,通常为IgM,需补体参与。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "冷凝集素病" }, { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "肺炎支原体" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "EB病毒感染" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "淋巴增生性疾病" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "抗体" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "IgM" } ] }, { "text": "急性型者继发于支原体肺炎或传染性单核细胞增多症,呈一过性,预后良好;慢性型者寒冷季节恶化,温热季节缓解,病情长期持续反复。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "支原体肺炎" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "传染性单核细胞增多症" } ] }, { "text": "实验室检查:冷凝集素滴度常在1∶10000左右,肺炎支原体感染时,可高达1∶30000以上。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "冷凝集素" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "肺炎支原体感染" } ] }, { "text": "直接Coombs试验可阳性,37℃时,间接试验阴性,但在低温条件下则阳性。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "Coombs试验" } ] }, { "text": "由IgM型冷凝抗体引起者,脾切除效果不佳,因为这类受损红细胞很少由脾脏清除。", "entities": [ { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "红细胞" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "脾脏" } ] }, { "text": "2.阵发性寒冷性血红蛋白尿本病占儿童AIHA病例的30%,多见于先天性梅毒患儿,也可继发于麻疹、流行性腮腺炎、水痘、传染性单核细胞增多症等病,少数为原发性。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "阵发性寒冷性血红蛋白尿" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "AIHA" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "先天性梅毒" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "麻疹" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "流行性腮腺炎" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "水痘" }, { "start_idx": 58, "end_idx": 67, "type": "dis", "entity": "传染性单核细胞增多症" } ] }, { "text": "它是与自身免疫和寒冷有关的急性溶血性贫血。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "急性溶血性贫血" } ] }, { "text": "患儿体内诱生的主要为IgG冷抗体即D-L型冷溶血素,该抗体在低温(<20℃)时能与红细胞膜上的抗原结合,并可固定大量补体,当温度上升时,大量红细胞发生破裂而溶血。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "抗体" }, { "start_idx": 17, "end_idx": 24, "type": "bod", "entity": "D-L型冷溶血素" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "抗体" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "红细胞膜" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "红细胞" } ] }, { "text": "本病也可呈慢性溶血过程,而与寒冷无关。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "溶血" } ] }, { "text": "临床上主要表现为身体暴露于低温条件下10~20分钟后复温时,发生溶血。", "entities": [ { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "溶血" } ] }, { "text": "发作时患儿出现寒战、高热、头痛、腰痛、肢痛及血红蛋白尿,并可出现恶性、呕吐、腹泻,肌强直等,症状持续数小时,发作后可有黄疸,反复发作者可出现轻度脾大。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "寒战" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "高热" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "头痛" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "腰痛" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "肢痛" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "血红蛋白尿" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "恶性" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "呕吐" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "腹泻" }, { "start_idx": 41, "end_idx": 43, "type": "sym", "entity": "肌强直" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "黄疸" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "轻度脾大" } ] }, { "text": "实验室检查:Donath-Landsteiner试验阳性为确诊本病的依据。", "entities": [ { "start_idx": 6, "end_idx": 25, "type": "pro", "entity": "Donath-Landsteiner试验" } ] }, { "text": "溶血发作时Coombs直接试验强阳性,发作后转为阴性;间接试验均阴性。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "溶血" }, { "start_idx": 5, "end_idx": 14, "type": "pro", "entity": "Coombs直接试验" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "间接试验" } ] }, { "text": "冷凝集试验阴性。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "冷凝集试验" } ] }, { "text": "慢性者可应用糖皮质激素或免疫抑制剂。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "重度贫血者可输注洗涤红细胞。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "贫血" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "红细胞" } ] }, { "text": "表10-12出血性疾病的临床鉴别(一)病史1.家族史家族中已有同样患者,提示为遗传性疾患,需仔细询问遗传方式等,如女性传递男性发病者多见于血友病甲或血友病乙;每一代中都有患者的为常染色体显性遗传如血管性血友病、毛细血管扩张症、储存池病、巨大血小板综合征等。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "出血性疾病" }, { "start_idx": 69, "end_idx": 72, "type": "dis", "entity": "血友病甲" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "血友病乙" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "染色体" }, { "start_idx": 98, "end_idx": 103, "type": "dis", "entity": "血管性血友病" }, { "start_idx": 105, "end_idx": 111, "type": "dis", "entity": "毛细血管扩张症" }, { "start_idx": 113, "end_idx": 116, "type": "dis", "entity": "储存池病" }, { "start_idx": 118, "end_idx": 125, "type": "dis", "entity": "巨大血小板综合征" } ] }, { "text": "2.发病年龄与性别遗传性出血性疾病发病年龄偏小,获得性者发病年龄多偏大,但维生素K缺乏症、原发性血小板减少性紫癜的发病年龄可小至数月至数天。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "遗传性出血性疾病" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "维生素K缺乏症" }, { "start_idx": 45, "end_idx": 55, "type": "dis", "entity": "原发性血小板减少性紫癜" } ] }, { "text": "男性患者伴关节腔出血者多为血友病甲或乙。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "关节腔出血" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "血友病甲或乙" } ] }, { "text": "3.出血方式皮肤、黏膜自发性出血、青春期月经量过多常提示血小板质或量的异常;轻微外伤后出血不止提示凝血功能异常。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "皮肤" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "黏膜" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "自发性出血" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "月经量过多" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "血小板质" } ] }, { "text": "4.药物史不少药物分别可损伤血管壁,影响血小板功能,抑制凝血,常用的如磺胺药、异烟肼、阿司匹林、双嘧达莫、肝素等;抗癌药则可直接抑制骨髓使血小板减少。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "血管壁" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "血小板" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "磺胺药" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "异烟肼" }, { "start_idx": 43, "end_idx": 46, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 48, "end_idx": 51, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 53, "end_idx": 54, "type": "dru", "entity": "肝素" }, { "start_idx": 57, "end_idx": 59, "type": "dru", "entity": "抗癌药" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "骨髓" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "血小板" } ] }, { "text": "5.其他疾病史肝肾疾病、结缔组织病、再生障碍性贫血、白血病等可引起继发性出血。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "肝肾疾病" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "再生障碍性贫血" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "白血病" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "继发性出血" } ] }, { "text": "(二)体格检查1.出血特征注意出血部位、性质、程度,四肢高出皮面的紫癜,对称性分布者是过敏性紫癜的特点。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "紫癜" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "过敏性紫癜" } ] }, { "text": "2.其他体征全身淋巴结及肝、脾大者提示恶性疾病如白血病、淋巴瘤等;巨脾应考虑到继发于寄生虫感染、溶血性疾病、肝硬化等的脾功能亢进;皮肤湿疹、反复感染及出血倾向应考虑到皮肤湿疹-感染-血小板减少综合征。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "淋巴结" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肝" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "脾" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "白血病" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "寄生虫感染" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "溶血性疾病" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "肝硬化" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "脾功能亢进" }, { "start_idx": 65, "end_idx": 68, "type": "dis", "entity": "皮肤湿疹" }, { "start_idx": 70, "end_idx": 76, "type": "sym", "entity": "反复感染及出血" }, { "start_idx": 83, "end_idx": 98, "type": "dis", "entity": "皮肤湿疹-感染-血小板减少综合征" } ] }, { "text": "(三)实验室检查实验室检查结果在出血性疾病的诊断中起着十分重要的作用,但是出、凝血的实验室检查项目繁多,临床医师必须仔细考虑病史和物理检查所揭示出的病人出血特点,结合血、大小便常规和骨髓涂片检查结果,排除由于全身性疾病引起的继发性出血,然后初步确立出血性疾病的实验室检查方向,以简易筛查试验来印证,再进一步检查确立最后诊断。", "entities": [ { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "凝血" }, { "start_idx": 83, "end_idx": 83, "type": "bod", "entity": "血" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "骨髓" }, { "start_idx": 112, "end_idx": 116, "type": "sym", "entity": "继发性出血" } ] }, { "text": "所以,出血性疾病的诊断可分为三步,首先确定是否出血性疾病,其次明确在血管、血小板、凝血因素中何种功能障碍引起的出血,最后确立最后诊断。", "entities": [ { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "血管" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "血小板" } ] }, { "text": "表10-13出血性疾病的简易筛查试验2.诊断试验根据临床特点及简易筛查试验结果,一般均能确定方向,明确是何种止血功能障碍引起的出血,接下来便是分门别类地确立进一步实验室检查的程序,以决定最后诊断。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "出血性疾病" } ] }, { "text": "例如疑为遗传性毛细血管扩张症者,应用毛细血管镜检查甲床可发现扭曲、排列不齐的扩张的毛细血管。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "dis", "entity": "遗传性毛细血管扩张症" }, { "start_idx": 18, "end_idx": 22, "type": "equ", "entity": "毛细血管镜" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "毛细血管" } ] }, { "text": "血小板数量正常疑为功能障碍者,应分别检查阿司匹林耐量试验、血小板黏附、聚集功能试验、血小板因子3(PF3)、血小板膜糖蛋白等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "血小板" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "血小板因子3" }, { "start_idx": 60, "end_idx": 66, "type": "bod", "entity": "血小板膜糖蛋白" } ] }, { "text": "例如激活的部分凝血活酶时间(APTT)延长,凝血酶原消耗试验(PCT)不良,凝血酶原时间(PT)正常,凝血酶时间(TT)正常,当属凝血活酶生成障碍。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "凝血活酶" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "APTT" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "PCT" }, { "start_idx": 45, "end_idx": 46, "type": "ite", "entity": "PT" }, { "start_idx": 57, "end_idx": 58, "type": "ite", "entity": "TT" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "凝血活酶" } ] }, { "text": "属于此阶段异常通常为血友病,可通过测定因子Ⅷ、Ⅸ、Ⅺ促凝血活性或APTT纠正试验以确定诊断。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "血友病" }, { "start_idx": 19, "end_idx": 25, "type": "bod", "entity": "因子Ⅷ、Ⅸ、Ⅺ" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "APTT" } ] }, { "text": "当APTT延长或正常,PCT正常、PT延长、TT正常时,应属凝血酶生成障碍,涉及因子Ⅱ、Ⅴ、Ⅶ、Ⅹ等异常,可进一步作PT纠正试验。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "ite", "entity": "APTT" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "PCT" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "PT" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "TT" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "凝血酶生成障碍" }, { "start_idx": 58, "end_idx": 59, "type": "ite", "entity": "PT" } ] }, { "text": "参考文献1.胡亚美,江载芳.诸福棠实用儿科学.第7版.北京:人民卫生出版社,20022.江载芳.实用小儿呼吸病学.北京:人民卫生出版社,2010:2253.中华医学会儿科分会呼吸学组.儿童侵袭性肺部真菌感染诊治指南(儿童侵袭性真菌感染州医学.2009,22(3):183-1834.肖晶,周东风,孟浦.儿童侵袭性真菌感染早期诊断的研究进展.国际儿科学杂志,2009,36(5):523-5235.KlontRR,MenninkKerstenMH,RuegebrinkW,etal.ParadoxicalincreaseincirculatingHspergillusantigenduringtreatmentwithcaspofungininapatientwithpulmonaryaspergillosis.ClinInfectDis,2006,43(3):23-236.AgarwalR.Allergicbronchopulmonaryaspergillosis.Chest,2009,135:805-8267.ChabraSK,SahayandS,RamarajuK.Allergicbronchopulmonaryaspergillosiscomplicatingchildhoodasthma.IndianJPediatric,2009,76(3):331-331", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "dep", "entity": "儿科" }, { "start_idx": 83, "end_idx": 84, "type": "dep", "entity": "儿科" }, { "start_idx": 108, "end_idx": 116, "type": "dis", "entity": "儿童侵袭性真菌感染" }, { "start_idx": 153, "end_idx": 159, "type": "dis", "entity": "侵袭性真菌感染" }, { "start_idx": 172, "end_idx": 173, "type": "dep", "entity": "儿科" } ] }, { "text": "四、液体疗法时常用补液溶液常用液体包括非电解质和电解质溶液。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "液体疗法" } ] }, { "text": "其中非电解质溶液常用5%或10%葡萄糖液,因葡萄糖输入体内将被氧化成水,故属无张力溶液。", "entities": [ { "start_idx": 10, "end_idx": 19, "type": "dru", "entity": "5%或10%葡萄糖液" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "葡萄糖" } ] }, { "text": "电解质溶液包括氯化钠、氯化钾、乳酸钠、碳酸氢钠和氯化铵等,以及它们的不同配制液(表2-2)。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "氯化钠" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "氯化钾" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "乳酸钠" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "氯化铵" } ] }, { "text": "表2-11常用溶液成分续表口服补液盐(oralrehydrationsalts,ORS)ORS是世界卫生组织推荐用以治疗急性腹泻合并脱水的一种溶液,经临床应用取得了良好效果,对发展中国家尤其适用。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "口服补液盐" }, { "start_idx": 19, "end_idx": 38, "type": "dru", "entity": "oralrehydrationsalts" }, { "start_idx": 40, "end_idx": 42, "type": "dru", "entity": "ORS" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "ORS" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "急性腹泻" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "脱水" } ] }, { "text": "其理论基础是基于小肠的Na+-葡萄糖偶联转运吸收机制,即小肠上皮细胞刷状缘的膜上存在着Na+-葡萄糖共同载体,此载体上有Na+-葡萄糖两个结合位点,当Na+-葡萄糖同时与结合位点相结合时即能运转,并显著增加钠和水的吸收。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "小肠" }, { "start_idx": 34, "end_idx": 44, "type": "bod", "entity": "小肠上皮细胞刷状缘的膜" } ] }, { "text": "目前有多种ORS配方。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "ORS" } ] }, { "text": "WHO2002年推荐的低渗透压口服补液盐配方较传统配方减少了糖和盐的量,但同样有效,且更为安全。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "dru", "entity": "低渗透压口服补液" } ] }, { "text": "该配方中各种电解质浓度为Na+75mmol/L、K+20mmol/L、Cl-65mmol/L、枸橼酸根10mmol/L、葡萄糖75mmol/L。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "Na+" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "K+" }, { "start_idx": 47, "end_idx": 49, "type": "ite", "entity": "Cl-" }, { "start_idx": 65, "end_idx": 68, "type": "ite", "entity": "枸橼酸根" }, { "start_idx": 78, "end_idx": 80, "type": "ite", "entity": "葡萄糖" } ] }, { "text": "可用NaCl2.6g、枸橼酸钠2.9g、氯化钾1.5g、葡萄糖13.5g,加水到1000ml配成。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "NaCl" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "枸橼酸钠" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "氯化钾" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "葡萄糖" } ] }, { "text": "ORS一般适用于轻度或中度脱水无严重呕吐者,在用于补充继续损失量和生理需要量时需适当稀释。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "ORS" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "轻度或中度脱水" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "严重呕吐" } ] }, { "text": "第四章非青紫型先天性心脏病第一节动脉导管未闭动脉导管是由第六对支气管动脉弓远端演化而成。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "非青紫型先天性心脏病" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "动脉导管" }, { "start_idx": 28, "end_idx": 38, "type": "bod", "entity": "第六对支气管动脉弓远端" } ] }, { "text": "在胎儿循环时,它将大部分右室入肺动脉的血流导入降主动脉送往胎盘进行氧合。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "右室" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肺动脉" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "降主动脉" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "胎盘" } ] }, { "text": "出生后,动脉导管未闭可作为一个独立病变存在(可单独存在),也可与其他心血管畸形合并存在,如主动脉弓缩窄或中断、严重的主动脉狭窄、左心发育不全综合征及肺动脉闭锁,严重的肺动脉狭窄或者作为血管环的一部分。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "心血管畸形" }, { "start_idx": 45, "end_idx": 53, "type": "dis", "entity": "主动脉弓缩窄或中断" }, { "start_idx": 55, "end_idx": 62, "type": "dis", "entity": "严重的主动脉狭窄" }, { "start_idx": 64, "end_idx": 72, "type": "dis", "entity": "左心发育不全综合征" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "肺动脉闭锁" }, { "start_idx": 80, "end_idx": 87, "type": "dis", "entity": "严重的肺动脉狭窄" }, { "start_idx": 92, "end_idx": 94, "type": "bod", "entity": "血管环" } ] }, { "text": "单纯的动脉导管未闭占所有先天性心脏病的12%,占活产婴儿的0.04%~0.06%。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "【病理解剖】动脉导管常位于左侧,肺动脉端开口起自左肺动脉近主肺动脉分叉处,主动脉端开口位于主动脉弓与降主动脉之间。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "动脉导管" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "肺动脉端开口" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "主肺动脉" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "主动脉端开口" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "降主动脉" } ] }, { "text": "如为右位主动脉弓,动脉导管可位于右侧。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "右位主动脉弓" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "动脉导管" } ] }, { "text": "少数左位动脉导管发生于右位主动脉弓,连接左肺动脉与左头臂动脉。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "左位动脉导管" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "右位主动脉弓" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "左头臂动脉" } ] }, { "text": "极少数动脉导管为双侧。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "动脉导管" } ] }, { "text": "组织学上,动脉导管管壁中段主要由数层环状的沿长轴方向排列的平滑肌构成,而与肺动脉和主动脉连接之处主要由环状的弹力纤维构成。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "动脉导管管壁" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "平滑肌" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肺动脉" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "主动脉" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "弹力纤维" } ] }, { "text": "出生后12小时,由于中层平滑肌收缩,动脉导管出现功能性关闭。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "平滑肌" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "动脉导管" } ] }, { "text": "此后,由于内皮细胞增生、肌纤维由纤维组织替代而最终转化成纤维索,形成动脉韧带导致解剖性关闭,此过程约需3周到3个月。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肌纤维" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "纤维组织" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "纤维索" }, { "start_idx": 34, "end_idx": 44, "type": "dis", "entity": "动脉韧带导致解剖性关闭" } ] }, { "text": "【病理生理】单纯的动脉导管未闭(patentductusarteriosus)的血流方向通常由降主动脉向肺动脉分流,从而形成左向右分流。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 16, "end_idx": 37, "type": "dis", "entity": "patentductusarteriosus" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "降主动脉" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "肺动脉" } ] }, { "text": "分流量取决于导管的粗细和体循环与肺循环血管阻力之间的压差。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "equ", "entity": "导管" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血管" } ] }, { "text": "生后早期肺血管阻力较高,分流量较小。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "肺血管" } ] }, { "text": "2个月后,随着肺血管阻力的降低,左向右分流逐渐增加。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肺血管" } ] }, { "text": "如导管管径较粗,左向右分流量多,肺静脉回流增多,则使左心容量负荷过重,左心房、左心室扩大。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "equ", "entity": "导管" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "肺静脉" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "左心" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "左心房" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "左心室" } ] }, { "text": "左心室容量负荷过重使左室舒张末期压力增大,导致继发性左心房压力增高,最终出现左心衰竭症状。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "左心室" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "左心房" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "左心衰竭" } ] }, { "text": "粗大的动脉导管未闭使肺循环血量增加过多,可造成肺动脉压力增高,体、肺循环的压力接近相等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肺" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "肺动脉" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "肺" } ] }, { "text": "肺血流量的增多将会进一步减慢或阻止肺小动脉平滑肌的退化,导致持续肺动脉高压。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 17, "end_idx": 23, "type": "bod", "entity": "肺小动脉平滑肌" }, { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "持续肺动脉高压" } ] }, { "text": "持续的大型左向右分流可使肺小动脉中膜增厚,内膜增生,导致肺小动脉破坏,形成不可逆性的肺血管病变。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "肺小动脉中膜" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "肺小动脉" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "肺血管病变" } ] }, { "text": "随着肺血管的进一步破坏以及肺血管阻力的增加,出现右向左分流,导致体循环血氧含量降低。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺血管" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肺血管" }, { "start_idx": 32, "end_idx": 36, "type": "ite", "entity": "体循环血氧" } ] }, { "text": "如通过FrankStarling机制左心室舒张末期容量增大以增加每搏量;增强交感神经兴奋性以增加心率和心肌收缩力,使心输出量进一步增加。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "左心室" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "交感神经" }, { "start_idx": 48, "end_idx": 49, "type": "ite", "entity": "心率" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "心肌" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "心" } ] }, { "text": "此外,大的动脉导管在心脏舒张期的窃血现象可造成肠系膜动脉在舒张期出现血流缺如甚至逆向血流,易导致坏死性小肠结肠炎。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "动脉导管" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心脏" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "窃血" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "肠系膜动脉" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "血流缺如" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "逆向血流" }, { "start_idx": 48, "end_idx": 55, "type": "dis", "entity": "坏死性小肠结肠炎" } ] }, { "text": "【临床表现】(一)早产儿血氧分压升高可使动脉导管收缩,而前列腺素E可使之扩张,此反应的灵敏程度与胎龄有关。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "血氧分压" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "动脉导管" }, { "start_idx": 28, "end_idx": 32, "type": "dru", "entity": "前列腺素E" } ] }, { "text": "肺泡表面活性剂的应用改善了呼吸窘迫综合征的症状,使肺血管阻力降低,临床症状常在生后3~4天左右才开始出现。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺泡" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "呼吸窘迫综合征" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "肺血管" } ] }, { "text": "肺动脉瓣区第二心音增强。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "肺动脉瓣区" } ] }, { "text": "未用呼吸机的患儿可表现为三凹征,腹部肝脏肿大。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "呼吸机" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "三凹征" }, { "start_idx": 16, "end_idx": 16, "type": "ite", "entity": "腹" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "腹" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "征,腹部" } ] }, { "text": "(二)婴儿和年长儿婴儿或年长儿,较小的动脉导管未闭可不引起任何症状,只是在常规体检时偶然发现心脏杂音才引起重视,生长发育不受影响。", "entities": [ { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "常规体检" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "心脏杂音" } ] }, { "text": "心搏出量正常或轻度增加,无心脏肥大或心脏搏动异常,第一、第二心音正常。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "心搏出量" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "无心脏肥大" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "心脏搏动异常" } ] }, { "text": "胸骨左上缘或左锁骨下可听到特征性的连续性杂音,杂音起初柔和,强度逐渐增强,到第二心音最响,至舒张期逐渐减弱。", "entities": [ { "start_idx": 0, "end_idx": 21, "type": "sym", "entity": "胸骨左上缘或左锁骨下可听到特征性的连续性杂音" } ] }, { "text": "细小的导管未闭临床可能仅表现为局限于收缩期的柔和的喷射性杂音。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "细小的导管未闭" } ] }, { "text": "心前区心尖搏动增强,呈抬举样搏动及心脏肥大的征象。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "心前区心尖搏动增强" }, { "start_idx": 10, "end_idx": 23, "type": "sym", "entity": "呈抬举样搏动及心脏肥大的征象" } ] }, { "text": "胸骨左上缘或左侧锁骨下可触及收缩期震颤。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "胸骨左上缘" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "左侧锁骨下" } ] }, { "text": "在有肺动脉高压时,右心室肥大,于胸骨左侧可触及心脏搏动。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "右心室肥大" }, { "start_idx": 16, "end_idx": 26, "type": "sym", "entity": "胸骨左侧可触及心脏搏动" } ] }, { "text": "听诊第二心音亢进,第一、第二心音可被响亮的动脉导管杂音所掩盖,胸骨左上缘可闻及多发的收缩期喀喇音,乃因来自动脉导管与右心室的相对血流冲撞产生湍流所致。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "听诊" }, { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "第二心音亢进" }, { "start_idx": 31, "end_idx": 47, "type": "sym", "entity": "胸骨左上缘可闻及多发的收缩期喀喇音" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "动脉导管" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "右心室" } ] }, { "text": "由于肺血管阻力的增加,左向右分流逐渐减少,左心衰竭症状减轻。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺血管" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "左心衰竭" } ] }, { "text": "症状的改变常出现于生后8~16个月,表现为脉搏减弱,心前区搏动减弱,第二心音增强且单一,舒张期杂音消失,收缩期杂音时相变短并逐渐消失。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "脉搏减弱" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "心前区搏动减弱" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "第二心音增强且单一" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "舒张期杂音消失" }, { "start_idx": 52, "end_idx": 65, "type": "sym", "entity": "收缩期杂音时相变短并逐渐消失" } ] }, { "text": "随着右向左分流的产生,肢体末端出现发绀明显,心前区体征呈严重肺动脉高压时的表现。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "肢体末端" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "发绀" }, { "start_idx": 22, "end_idx": 38, "type": "sym", "entity": "心前区体征呈严重肺动脉高压时的表现" } ] }, { "text": "生后15~18个月,肺血管即可发生不可逆性改变。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺血管" } ] }, { "text": "【鉴别诊断】无发绀患儿周围血管搏动明显伴连续性杂音,如杂音不在导管的典型部位,要考虑其他先天性或后天性心脏疾患,如主肺动脉窗、冠状动静脉瘘冠状动静脉瘘(coronaryarteriovenousfistula)、主动脉乏氏窦瘤破裂及伴室隔缺损的主动脉瓣反流。", "entities": [ { "start_idx": 6, "end_idx": 24, "type": "sym", "entity": "无发绀患儿周围血管搏动明显伴连续性杂音" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "主肺动脉窗" }, { "start_idx": 63, "end_idx": 68, "type": "dis", "entity": "冠状动静脉瘘" }, { "start_idx": 69, "end_idx": 74, "type": "dis", "entity": "冠状动静脉瘘" }, { "start_idx": 76, "end_idx": 103, "type": "dis", "entity": "coronaryarteriovenousfistula" }, { "start_idx": 106, "end_idx": 114, "type": "dis", "entity": "主动脉乏氏窦瘤破裂" }, { "start_idx": 116, "end_idx": 127, "type": "dis", "entity": "伴室隔缺损的主动脉瓣反流" } ] }, { "text": "【检查】(一)X线胸片导管细小时胸片正常。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "X线" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "胸片" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "导管" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "胸片" } ] }, { "text": "存在中到大量左向右分流时,左室增大,左心房增大明显,主动脉、肺动脉段突出,外周肺血管影增多,肺野充血。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "左室" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "左心房" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "主动脉" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "肺动脉段" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "肺血管" } ] }, { "text": "早产儿增粗的肺血管影较难与呼吸窘迫综合征所致的肺实质病变及慢性肺部疾病相鉴别。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肺血管" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "呼吸窘迫综合征" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "肺实质病变" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "慢性肺部疾病" } ] }, { "text": "(二)心电图导管细小时心电图完全正常,导管直径越大,左向右分流加大,心电图显示左室肥大,Ⅱ、Ⅲ、aVF、V5~V6等导联R波高耸,左胸导联T波倒置;左房增大呈宽大P波;如有肺动脉高压,则T波高尖,右胸导联R波增大。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "心电图" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "导管" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "心电图" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "导管" }, { "start_idx": 34, "end_idx": 36, "type": "pro", "entity": "心电图" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "左室肥大" }, { "start_idx": 98, "end_idx": 102, "type": "dis", "entity": "肺动脉高压" } ] }, { "text": "早产儿患肺部疾病时,右胸导联占优势。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "肺部疾病" } ] }, { "text": "(三)超声心动图二维超声和多普勒可显示动脉导管分流量的大小。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "二维超声" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "多普勒" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "动脉导管" } ] }, { "text": "在高位胸骨旁切面,探头置于胸骨左缘第一、二肋间隙,逆时针稍作旋转,即可得到导管大小及形态的清晰图像(图9-9)。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "胸骨" }, { "start_idx": 9, "end_idx": 10, "type": "equ", "entity": "探头" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "胸骨左缘" } ] }, { "text": "左心房、左心室大小可反映左心室容量负荷。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "左心房" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "左心室" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "左心室" } ] }, { "text": "M型超声左心房与主动脉根部内径比值超过1.3表示有较大的左向右的分流。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "M型超声" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "左心房" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "主动脉" } ] }, { "text": "彩色多普勒可快速显示细小的左向右分流,尤其适于检测手术后或经导管介入治疗后有无残余分流。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "彩色多普勒" }, { "start_idx": 29, "end_idx": 35, "type": "pro", "entity": "经导管介入治疗" } ] }, { "text": "连续多普勒超声适于估测穿过导管的射流速度,并可以此估测体循环与肺循环之间的压力差。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "多普勒超声" }, { "start_idx": 13, "end_idx": 14, "type": "equ", "entity": "导管" } ] }, { "text": "已知体循环动脉压后可用此方法估测肺动脉压力。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "ite", "entity": "体循环动脉压" }, { "start_idx": 16, "end_idx": 20, "type": "ite", "entity": "肺动脉压力" } ] }, { "text": "图9-14超声心动图示:中等大小的动脉导管未闭(箭头所示)(四)心导管现今的心导管术主要是用于动脉导管未闭封堵治疗而不是以诊断为目的。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "超声心动图" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "心导管" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "心导管术" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "动脉导管未闭" } ] }, { "text": "心导管检测肺动脉干氧饱和度升高表示动脉导管水平由左向右分流。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "pro", "entity": "心导管检测肺动脉干氧饱和度" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "动脉导管" } ] }, { "text": "但这一指标并不特异,主肺动脉窗、双动脉下室缺亦有相似结果。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "主肺动脉窗" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "双动脉下室" } ] }, { "text": "中到大型动脉导管未闭者肺动脉压升高。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 11, "end_idx": 14, "type": "ite", "entity": "肺动脉压" } ] }, { "text": "因体循环舒张压下降,收缩压增加,导致体循环脉压增宽。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "舒张压" }, { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "收缩压" }, { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "脉压" } ] }, { "text": "降主动脉造影可显示动脉导管解剖学形态。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "降主动脉造影" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "动脉导管" } ] }, { "text": "侧位造影可显示动脉导管的走向和形态(图9-9),左前斜位可减小动脉导管与降主动脉的重叠影。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "侧位造影" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "动脉导管" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "动脉导管" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "降主动脉" } ] }, { "text": "多数情况下主动脉端壶腹部较宽,越近肺动脉端直径越细。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "主动脉" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "肺动脉端" } ] }, { "text": "无节段性狭窄的管样动脉导管较少见。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "管样动脉" } ] }, { "text": "心导管造影前及造影中均不可将导管穿过动脉导管,否则,由此造成的动脉导管痉挛会影响对其大小的判断,从而影响填塞装置大小的选择。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "心导管造影" }, { "start_idx": 14, "end_idx": 15, "type": "equ", "entity": "导管" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "动脉导管" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "动脉导管痉挛" }, { "start_idx": 52, "end_idx": 55, "type": "equ", "entity": "填塞装置" } ] }, { "text": "图9-15降主动脉造影侧位片示:存在动脉导管未闭(箭头所示)【治疗】(一)早产儿对于早产儿,早期就需限制液体和钠盐的摄入量。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "降主动脉造影" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "动脉导管未闭" } ] }, { "text": "但有肾功能不全、坏死性小肠结肠炎及有出血倾向的患儿禁用,且治疗过程中需密切检测肾功能。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肾功能不全" }, { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "坏死性小肠结肠炎" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "出血倾向" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "肾" } ] }, { "text": "近来使用异丁苯乙酸(布洛芬)静脉滴注促进动脉导管闭合,疗效与吲哚美辛相当,且较少引起少尿。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "异丁苯乙酸" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "布洛芬" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "动脉导管" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "少尿" } ] }, { "text": "布洛芬分3次给予,首次剂量为10mg/kg,之后的24小时按每5mg/kg再用2次,导管闭合率可达70%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "布洛芬" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "导管" } ] }, { "text": "动脉导管结扎术可在新生儿重症监护室实施,以避免转往手术室过程中可能出现的紧急状态。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "动脉导管结扎术" }, { "start_idx": 9, "end_idx": 16, "type": "dep", "entity": "新生儿重症监护室" }, { "start_idx": 25, "end_idx": 27, "type": "dep", "entity": "手术室" } ] }, { "text": "对出生体重≤1000g的早产儿,在出生当天进行预防性的动脉导管结扎,经临床随机、对照研究发现可降低坏死性小肠结肠炎的发生率。", "entities": [ { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "动脉导管结扎" }, { "start_idx": 49, "end_idx": 56, "type": "dis", "entity": "坏死性小肠结肠炎" } ] }, { "text": "(二)足月儿和年长儿有严重左向右分流的患儿,关闭动脉导管可以纠正心力衰竭并消除最终发展成为肺血管疾病的危险性。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "左向右分流" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "关闭动脉导管" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "肺血管疾病" } ] }, { "text": "为预防感染性心内膜,即使是小的分流也建议结扎动脉导管,尤其是这种手术的并发症和死亡率都比较低,但单纯为消除发生感染性心内膜炎的危险性而行动脉导管结扎术尚有争议。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "感染性心内膜" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "结扎动脉导管" }, { "start_idx": 55, "end_idx": 61, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 68, "end_idx": 74, "type": "pro", "entity": "动脉导管结扎术" } ] }, { "text": "(三)经皮穿刺经导管动脉导管堵塞术目前,经导管介入治疗堵塞未闭的动脉导管的方法优于手术结扎。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "pro", "entity": "经皮穿刺经导管动脉导管堵塞术" }, { "start_idx": 20, "end_idx": 35, "type": "pro", "entity": "经导管介入治疗堵塞未闭的动脉导管" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "手术结扎" } ] }, { "text": "这一部分内容将在介入性心导管治疗术中详细介绍。", "entities": [ { "start_idx": 8, "end_idx": 16, "type": "pro", "entity": "介入性心导管治疗术" } ] }, { "text": "从20世纪70年代早期到80年代后期Rashkind双伞装置被广泛使用之前,动脉导管经心导管阻塞术只是偶尔有过尝试(图9-16A)。", "entities": [ { "start_idx": 18, "end_idx": 29, "type": "equ", "entity": "Rashkind双伞装置" }, { "start_idx": 38, "end_idx": 48, "type": "pro", "entity": "动脉导管经心导管阻塞术" } ] }, { "text": "但Rashkind双伞由于价格昂贵,需较大的经静脉管鞘及相对较高的近10%~20%的残余分流率,目前亦已少用。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "equ", "entity": "Rashkind双伞" }, { "start_idx": 22, "end_idx": 26, "type": "equ", "entity": "经静脉管鞘" } ] }, { "text": "据报道有少数病人尚可发生血管内溶血和左肺动脉血流紊乱。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "血管内溶血" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "左肺动脉血流紊乱" } ] }, { "text": "图9-16经导管用(A)Rashkind伞阻塞未闭的动脉导管已经过时,现多用(B)Gianturco旋管和(C)Amplatzer导管闭塞器近来,充满涤纶纤维的不锈钢弹簧圈正在越来越多地应用于小到中型的动脉导管的堵封闭(图9-9B)。", "entities": [ { "start_idx": 5, "end_idx": 29, "type": "pro", "entity": "经导管用(A)Rashkind伞阻塞未闭的动脉导管" }, { "start_idx": 38, "end_idx": 52, "type": "equ", "entity": "(B)Gianturco旋管和" }, { "start_idx": 53, "end_idx": 69, "type": "equ", "entity": "(C)Amplatzer导管闭塞器" }, { "start_idx": 80, "end_idx": 85, "type": "equ", "entity": "不锈钢弹簧圈" }, { "start_idx": 96, "end_idx": 108, "type": "pro", "entity": "小到中型的动脉导管的堵封闭" } ] }, { "text": "具有可控性释放机制的可拆卸弹簧圈因其潜在的易控制、易于取回重新放置的优点而得到发展。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "equ", "entity": "可拆卸弹簧圈" } ] }, { "text": "弹簧圈的优势是价格便宜,只需要较小的递送导管,封堵率达95%以上。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "弹簧圈" }, { "start_idx": 18, "end_idx": 21, "type": "equ", "entity": "递送导管" } ] }, { "text": "关闭4毫米及以上的大的动脉导管有较高的技术要求,须植入多个弹簧圈。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "动脉导管" }, { "start_idx": 29, "end_idx": 31, "type": "equ", "entity": "弹簧圈" } ] }, { "text": "弹簧圈封堵术的并发症不多见,包括弹簧圈导致的肺动脉分支栓塞、堵塞不全所致的血管内溶血及植入多个弹簧圈导致的左肺动脉狭窄。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "弹簧圈封堵术" }, { "start_idx": 16, "end_idx": 18, "type": "equ", "entity": "弹簧圈" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "肺动脉分支栓塞" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "血管内溶血" }, { "start_idx": 47, "end_idx": 49, "type": "equ", "entity": "弹簧圈" }, { "start_idx": 53, "end_idx": 58, "type": "dis", "entity": "左肺动脉狭窄" } ] }, { "text": "对于大的动脉导管未闭,纽式装置和近来备受推崇的Amplatzer导管封堵器已取得良好疗效(图9-9C)。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 11, "end_idx": 14, "type": "equ", "entity": "纽式装置" }, { "start_idx": 23, "end_idx": 36, "type": "equ", "entity": "Amplatzer导管封堵器" } ] }, { "text": "新近的多中心研究表明,纽扣式堵闭器用纽式装置是可行的和十分安全的。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "equ", "entity": "纽扣式堵闭器" }, { "start_idx": 18, "end_idx": 21, "type": "equ", "entity": "纽式装置" } ] }, { "text": "在284例年龄在3个月至92岁之间,动脉导管直径在1~15mm间的患者中,278例(98%)患者术后1年、2年、5年的残余分流检出率分别为21%、14%和0%。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "动脉导管" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "残余分流" } ] }, { "text": "同时,在婴儿和年长儿中采用Amplatzer导管封堵器堵塞中到大型动脉导管亦已积累了大量经验。", "entities": [ { "start_idx": 11, "end_idx": 36, "type": "pro", "entity": "采用Amplatzer导管封堵器堵塞中到大型动脉导管" } ] }, { "text": "Bilkis等近来报道了209例年龄在2个月至50岁,动脉导管在1.8~12.5mm之间,Amplatzer导管封堵器对98%患者有效,术后24小时内和1月内关闭率分别为66%和97%。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "动脉导管" }, { "start_idx": 45, "end_idx": 58, "type": "equ", "entity": "Amplatzer导管封堵器" } ] }, { "text": "术后并发症较少,包括堵塞装置引起栓塞、轻度主动脉狭窄,血管内溶血更少见。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "equ", "entity": "堵塞装置" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "栓塞" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "轻度主动脉狭窄" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "血管内溶血" } ] }, { "text": "(四)手术治疗手术结扎导管临床上有0.4%到3.1%的残余分流率。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "手术结扎导管" } ] }, { "text": "使用较敏感的彩色多普勒超声探查,残余分流率可能会更高,因此,对于一些再通和管径超过7~10毫米的患者需手术时应切断动脉导管。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "pro", "entity": "彩色多普勒超声探查" }, { "start_idx": 55, "end_idx": 60, "type": "pro", "entity": "切断动脉导管" } ] }, { "text": "近来,对新生儿、婴儿经腋下小切口胸廓切开术已经开展,取得了广为认可的效果。", "entities": [ { "start_idx": 10, "end_idx": 20, "type": "pro", "entity": "经腋下小切口胸廓切开术" } ] }, { "text": "手术步骤包括胸腔镜直视下放置血管夹。", "entities": [ { "start_idx": 6, "end_idx": 16, "type": "pro", "entity": "胸腔镜直视下放置血管夹" } ] }, { "text": "该新技术扩展应用于早产儿和婴儿,并发症包括气胸、喉返神经损伤。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "气胸" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "喉返神经损伤" } ] }, { "text": "(五)无症状动脉导管未闭随着彩色多普勒技术的应用,一些无临床症状的细小的动脉导管引起的微弱的湍流亦可被探及。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "无症状动脉导管未闭" }, { "start_idx": 14, "end_idx": 20, "type": "pro", "entity": "彩色多普勒技术" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "动脉导管" } ] }, { "text": "据报道,因发现与动脉导管未闭血流无关的杂音而行彩超检查的儿童中约0.5%发现有无症状性动脉导管未闭。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "彩超检查" }, { "start_idx": 39, "end_idx": 48, "type": "dis", "entity": "无症状性动脉导管未闭" } ] }, { "text": "曾有报道示1例无症状性导管未闭患者患有动脉内膜炎,是否需常规关闭无症状性导管尚需进一步探讨。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "无症状性导管未闭" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "动脉内膜炎" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "导管" } ] }, { "text": "【预后】未经治疗的动脉导管未闭患者的完整自然病程尚不清楚。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "动脉导管未闭" } ] }, { "text": "预期寿命的数据多是在常规使用抗生素之前收集的,由于感染性心内膜炎是患儿死亡的重要原因,预防性使用抗生素使情况与以往有明显不同。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "抗生素" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "抗生素" } ] }, { "text": "并发症包括感染性动脉内膜炎、进行性肺血管疾病和动脉瘤形成。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "感染性动脉内膜炎" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "进行性肺血管疾病" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "动脉瘤" } ] }, { "text": "目前感染性动脉内膜炎的危险性已较前大大降低。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "感染性动脉内膜炎" } ] }, { "text": "近期来自瑞典的研究表明,270例动脉导管未闭患者的随访研究中,无一例患感染性动脉内膜炎。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "感染性动脉内膜炎" } ] }, { "text": "经导管动脉导管堵塞术很少引起动脉内膜炎,使用Rashkind双伞封堵术的发病率为0.1%。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "pro", "entity": "导管动脉导管堵塞术" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "动脉内膜炎" }, { "start_idx": 22, "end_idx": 34, "type": "pro", "entity": "Rashkind双伞封堵术" } ] }, { "text": "但术后通常应用了抗生素进行预防性治疗。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "抗生素" } ] }, { "text": "常规的方法是在彩色多普勒证实导管完全堵闭后,继续连续应用抗生素6~12个月进行预防。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "彩色多普勒" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "抗生素" } ] }, { "text": "由于早期发现和治疗,进行性肺血管病变并不常见,偶有病人发展为肺血管病变和不可逆性肺动脉高压,此时为关闭动脉导管的禁忌证,这些病人常考虑行肺移植。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "进行性肺血管病变" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "肺血管病变" }, { "start_idx": 36, "end_idx": 44, "type": "dis", "entity": "不可逆性肺动脉高压" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "动脉导管" }, { "start_idx": 68, "end_idx": 70, "type": "pro", "entity": "肺移植" } ] }, { "text": "动脉导管瘤较少见,一旦出现,导管可能增大并压迫肺动脉,损伤喉返神经或出现瘤破裂。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "动脉导管瘤" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "肺动脉" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "损伤喉返神经" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "出现瘤破裂" } ] }, { "text": "【导管依赖性循环】动脉导管未闭在许多先天性心脏病中具有重要作用。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "未闭的动脉导管保证了充足的肺血流、体循环血流及体肺循环间血液的混合,此称为“导管依赖性循环”。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "动脉导管" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肺血流" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "体循环血流" }, { "start_idx": 23, "end_idx": 29, "type": "bod", "entity": "体肺循环间血液" } ] }, { "text": "导管依赖性肺循环见于肺动脉瓣闭锁、肺动脉狭窄及严重的法洛四联征;导管依赖性体循环见于严重水肿或离断、严重的主动脉瓣狭窄、左心发育不良综合征。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "肺动脉瓣闭锁" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 23, "end_idx": 30, "type": "dis", "entity": "严重的法洛四联征" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "严重水肿" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "离断" }, { "start_idx": 50, "end_idx": 58, "type": "dis", "entity": "严重的主动脉瓣狭窄" }, { "start_idx": 60, "end_idx": 68, "type": "dis", "entity": "左心发育不良综合征" } ] }, { "text": "对于完全性大动脉转位的患者,必须通过动脉导管使体肺循环血液得到充分混合。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "完全性大动脉转位" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "动脉导管" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "体肺循环血液" } ] }, { "text": "【维持动脉导管开放】(一)前列腺素E1和E2前列腺素E具有保持动脉导管开放的作用。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "动脉导管" }, { "start_idx": 13, "end_idx": 21, "type": "dru", "entity": "前列腺素E1和E2" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "前列腺素E" } ] }, { "text": "持续静脉点滴前列腺素E1或E2可用于导管依赖性循环的婴儿。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "静脉点滴" }, { "start_idx": 6, "end_idx": 14, "type": "dru", "entity": "前列腺素E1或E2" } ] }, { "text": "在特殊情况下,如早产儿,延长前列腺素E的使用时间仍很安全,且耐受量无变化。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dru", "entity": "前列腺素E" } ] }, { "text": "前列腺素E2也可口服,但次数多且吸收不稳定。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "前列腺素E2" } ] }, { "text": "前列腺素E的副作用有发热、呼吸暂停、心动过缓、低血压和癫痫样运动。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "前列腺素E" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "发热" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "心动过缓" }, { "start_idx": 23, "end_idx": 25, "type": "sym", "entity": "低血压" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "癫痫样运动" } ] }, { "text": "(二)动脉导管支架在实施手术困难的患儿,可植入自体扩张支架或球囊扩张支架以防止动脉导管关闭。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "equ", "entity": "动脉导管支架" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "手术" }, { "start_idx": 23, "end_idx": 28, "type": "equ", "entity": "自体扩张支架" }, { "start_idx": 30, "end_idx": 35, "type": "equ", "entity": "球囊扩张支架" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "动脉导管" } ] }, { "text": "这种血管内支架的姑息植入适用于左心发育不良综合征、肺动脉环缩和复杂型肺动脉闭锁的患儿。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血管" }, { "start_idx": 5, "end_idx": 6, "type": "equ", "entity": "支架" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "姑息植入" }, { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "左心发育不良综合征" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "肺动脉环缩" }, { "start_idx": 31, "end_idx": 38, "type": "dis", "entity": "复杂型肺动脉闭锁" } ] }, { "text": "姑息手术的效果只是暂时的,术后仍需重复通过球囊成形术来减少血管内膜肥厚所带来的影响。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "姑息手术" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "球囊成形术" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "血管内膜肥厚" } ] }, { "text": "而且,在导管依赖性心脏畸形婴儿中,导管内支架的作用仍需进一步研究。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "dis", "entity": "导管依赖性心脏畸形" }, { "start_idx": 17, "end_idx": 21, "type": "equ", "entity": "导管内支架" } ] }, { "text": "第三节神经电生理技术一、脑电图脑电图(electroencephalogram,EEG)描记法是应用电子放大技术将脑部自发的生物电流活动放大100万倍,通过头皮上两点间的电位差,或者头皮和无关电极值的电位差描记出脑波图线,临床上称为脑电图。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "脑电图" }, { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "脑电图" }, { "start_idx": 19, "end_idx": 38, "type": "pro", "entity": "electroencephalogram" }, { "start_idx": 40, "end_idx": 42, "type": "pro", "entity": "EEG" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "脑部" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "头皮" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "头皮" }, { "start_idx": 116, "end_idx": 118, "type": "pro", "entity": "脑电图" } ] }, { "text": "(一)原理脑电图所见的节律性电活动是发生于大脑皮质锥体细胞,经过突触后传送,它实际是代表着突触后电位,而不是从轴突传出的电位。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "脑电图" }, { "start_idx": 21, "end_idx": 28, "type": "bod", "entity": "大脑皮质锥体细胞" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "突触" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "突触" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "轴突" } ] }, { "text": "脑电图上的曲线并不代表几个神经细胞所发放的电流,而是大脑某一区域内许多神经元在一定生理生化代谢情况下的同步化电位。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "脑电图" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "神经细胞" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "神经元" } ] }, { "text": "由于大脑各区情况不同,同步的神经元数目也有差别,所以作为脑电活动信号的波率及波幅等也就不一致。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "神经元" } ] }, { "text": "主要见于枕顶区,为年长儿及正常成人的基本节律。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "枕顶区" } ] }, { "text": "α波在清醒安静闭目时即出现,睁眼、注意及思考问题时消失。", "entities": [ { "start_idx": 0, "end_idx": 26, "type": "sym", "entity": "α波在清醒安静闭目时即出现,睁眼、注意及思考问题时消失" } ] }, { "text": "注意、情绪紧张或服用安眠药时可增多。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "安眠药" }, { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "注意、情绪紧张或服用安眠药时可增多" } ] }, { "text": "3.θ波频率在4~7Hz,波幅20~40μV,见于顶区及额颞区。", "entities": [ { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "额颞区" } ] }, { "text": "在病理状态下,局限性慢波出现于局限性癫痫、脑肿瘤、脑脓肿、脑外伤性血肿和伴有软化灶的脑血管病等,有定位诊断价值。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "局限性癫痫" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "脑肿瘤" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "脑外伤性血肿" }, { "start_idx": 36, "end_idx": 45, "type": "dis", "entity": "伴有软化灶的脑血管病" } ] }, { "text": "弥漫性慢活动见于某些感染、中毒、低血糖、颅内压增高和各种原因引起的昏迷等。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "感染" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "中毒" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "低血糖" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "昏迷" } ] }, { "text": "5.κ节律在进行思维活动时,有时于额颞部出现一种6~12Hz,10~40μV的节律,开眼一般不抑制。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "额颞部" } ] }, { "text": "6.λ波在枕区出现的3~5Hz,10~40μV正相尖波,常为注视所诱发。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "枕区" } ] }, { "text": "7.μ节律在中央区出现的7~11Hz的节律,常为弧形,与希腊字母的μ相似,见于3%~13%正常人,常被握拳所抑制,开眼时不消失。", "entities": [ { "start_idx": 49, "end_idx": 62, "type": "sym", "entity": "常被握拳所抑制,开眼时不消失" } ] }, { "text": "8.顶尖波主要是负相尖波,在顶区最明显,常见于浅睡期。", "entities": [ { "start_idx": 13, "end_idx": 25, "type": "sym", "entity": "在顶区最明显,常见于浅睡期" } ] }, { "text": "9.σ节律又称睡梭或睡波,为约14Hz的节律,常发生于中睡期。", "entities": [ { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "常发生于中睡期" } ] }, { "text": "10.κ复合波是由顶尖波和σ节律组成的复合波,可在睡眠中自发性出现或被突然的声音刺激诱发出现。", "entities": [ { "start_idx": 23, "end_idx": 45, "type": "sym", "entity": "可在睡眠中自发性出现或被突然的声音刺激诱发出现" } ] }, { "text": "棘波是大脑皮层神经细胞过度兴奋癫痫,多棘波见于肌阵挛性发作。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "bod", "entity": "大脑皮层神经细胞" }, { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "大脑皮层神经细胞过度兴奋" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "癫痫" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "肌阵挛性发作" } ] }, { "text": "12.尖波是周期为70~200ms的三角形波,与背景脑电图有区别,出现于各种类型癫痫,三相尖波见于肝性昏迷。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "脑电图" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "癫痫" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "肝性昏迷" } ] }, { "text": "13.棘慢综合波是由1个棘波和1个慢波组成的复合波,棘波周期短于770ms,慢波的周期在200~500ms之间,出现于局限性癫痫。", "entities": [ { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "局限性癫痫" } ] }, { "text": "两侧对称同步3Hz持续的有规律的棘慢节律见于失神发作。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "失神发作" } ] }, { "text": "14.多棘慢波由2个/2个以上的棘波和1个慢波组成的复合波,见于肌阵挛性发作。", "entities": [ { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "见于肌阵挛性发作" } ] }, { "text": "15.尖慢综合波由1个尖波和1个慢波组成,尖波的周期在70~200ms之间,慢波周期在500~1000ms之间,出现于各种类型癫痫。", "entities": [ { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "癫痫" } ] }, { "text": "16.高度失律为不规则的高波幅慢活动,杂以棘波和尖波,一般不会形成典型的复合波,呈发作性或游走性,见于婴儿痉挛。", "entities": [ { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "痉挛" } ] }, { "text": "17.懒波一侧大脑半球有病变时,在病侧可出现α波减弱或消失,以及β波和睡眠波的减弱或消失。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "大脑半球" }, { "start_idx": 16, "end_idx": 43, "type": "sym", "entity": "在病侧可出现α波减弱或消失,以及β波和睡眠波的减弱或消失" } ] }, { "text": "18.平坦活动各种频率的电活动有不同程度的抑制,为大脑严重损害或各种原因引起的极度昏迷病人的脑波。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "大脑" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "极度昏迷" } ] }, { "text": "19.爆发性抑制活动即在平坦活动背景上,突然出现高波幅慢活动,可合并尖波,是大脑皮层和皮层下广泛损害的表现,见于婴儿痉挛、恶性胶质瘤、脑炎极期或麻醉过深脑电图的参数包括频率、波幅、波形、位相及调节与调幅。", "entities": [ { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "大脑皮层" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "皮层" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "婴儿痉挛" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "恶性胶质瘤" }, { "start_idx": 67, "end_idx": 68, "type": "dis", "entity": "脑炎" }, { "start_idx": 72, "end_idx": 73, "type": "pro", "entity": "麻醉" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "麻醉过深" }, { "start_idx": 76, "end_idx": 78, "type": "pro", "entity": "脑电图" } ] }, { "text": "脑电图的波幅,常以枕部描记出来的那些基本节律的波高度为标准。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "脑电图" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "枕部" } ] }, { "text": "(四)儿童正常脑电图1.安静时脑电图(1)新生儿期:以低波幅δ活动和20~50μV的θ活动及散在性低波幅的α波和β波为特征。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "脑电图" }, { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "脑电图" }, { "start_idx": 27, "end_idx": 57, "type": "sym", "entity": "低波幅δ活动和20~50μV的θ活动及散在性低波幅的α波和β波" } ] }, { "text": "(2)婴儿期:特征为频率2~6Hz,波幅20~50μV的慢活动。", "entities": [ { "start_idx": 7, "end_idx": 30, "type": "sym", "entity": "特征为频率2~6Hz,波幅20~50μV的慢活动" } ] }, { "text": "背景活动表现为基线不稳。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "背景活动表现为基线不稳" } ] }, { "text": "6个月以前以δ活动占优势,半岁以后θ活动为主。", "entities": [ { "start_idx": 0, "end_idx": 21, "type": "sym", "entity": "6个月以前以δ活动占优势,半岁以后θ活动为主" } ] }, { "text": "此期α波和β波很少,常散在性或短程出现。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "此期α波和β波很少,常散在性或短程出现" } ] }, { "text": "(3)幼儿期:特点是30~60μV的θ活动,背景活动表现为基线不稳。", "entities": [ { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "背景活动表现为基线不稳" } ] }, { "text": "1岁半以前以θ活动占优势,1岁半岁以后有13%以α活动占主导。", "entities": [ { "start_idx": 0, "end_idx": 29, "type": "sym", "entity": "1岁半以前以θ活动占优势,1岁半岁以后有13%以α活动占主导" } ] }, { "text": "此期β活动仍很少。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "此期β活动仍很少" } ] }, { "text": "(4)学龄前期:特征为7~10Hz波幅40~120μV的θ活动,背景活动表现为基线不稳。", "entities": [ { "start_idx": 8, "end_idx": 42, "type": "sym", "entity": "特征为7~10Hz波幅40~120μV的θ活动,背景活动表现为基线不稳" } ] }, { "text": "多数(88%)以α活动占优势,少数(12%)以7~8Hzθ活动为主。", "entities": [ { "start_idx": 0, "end_idx": 32, "type": "sym", "entity": "多数(88%)以α活动占优势,少数(12%)以7~8Hzθ活动为主" } ] }, { "text": "(5)儿童期:以8~11Hz,40~120μV的α活动占优势,背景活动表现为基线不稳或欠稳各占一半左右。", "entities": [ { "start_idx": 7, "end_idx": 50, "type": "sym", "entity": "以8~11Hz,40~120μV的α活动占优势,背景活动表现为基线不稳或欠稳各占一半左右" } ] }, { "text": "(6)青少年期:以9~12Hz,20~100μV的α节律占优势,2/3表现为背景活动基线欠稳。", "entities": [ { "start_idx": 8, "end_idx": 45, "type": "sym", "entity": "以9~12Hz,20~100μV的α节律占优势,2/3表现为背景活动基线欠稳" } ] }, { "text": "2.睡眠脑电图睡眠时脑电图改变很大,但有一定规律性,一般把睡眠过程分为下列四期:(1)思睡期:开始α节律波幅增高,区域扩大,然后α节律减少,频率变慢,波幅减低,常短阵或成对出现。", "entities": [ { "start_idx": 47, "end_idx": 87, "type": "sym", "entity": "开始α节律波幅增高,区域扩大,然后α节律减少,频率变慢,波幅减低,常短阵或成对出现" } ] }, { "text": "低波幅β活动和θ活动增多。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "低波幅β活动和θ活动增多" } ] }, { "text": "(2)浅睡期:α节律逐渐消失,出现低波幅4~7Hzθ活动和高波幅尖波,后者通常在顶区明显,称顶尖波,为此期特点。", "entities": [ { "start_idx": 7, "end_idx": 43, "type": "sym", "entity": "α节律逐渐消失,出现低波幅4~7Hzθ活动和高波幅尖波,后者通常在顶区明显" } ] }, { "text": "(3)中睡期:慢活动增多,波幅增高而频率进一步减低,自1~6Hz不等,常出现约14Hz的δ节律,为此期特点。", "entities": [ { "start_idx": 7, "end_idx": 46, "type": "sym", "entity": "慢活动增多,波幅增高而频率进一步减低,自1~6Hz不等,常出现约14Hz的δ节律" } ] }, { "text": "(4)深睡期:δ节律和κ复合波减少至消失。", "entities": [ { "start_idx": 7, "end_idx": 19, "type": "sym", "entity": "δ节律和κ复合波减少至消失" } ] }, { "text": "1~2Hz高波幅δ活动逐渐增加而占优势。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "1~2Hz高波幅δ活动逐渐增加而占优势" } ] }, { "text": "极度深睡时持续出现弥漫性0.5~1Hz的高波幅δ活动。", "entities": [ { "start_idx": 0, "end_idx": 25, "type": "sym", "entity": "极度深睡时持续出现弥漫性0.5~1Hz的高波幅δ活动" } ] }, { "text": "在儿童思睡期或被叫醒时,可呈现持续性或阵发性高波幅慢活动。", "entities": [ { "start_idx": 13, "end_idx": 27, "type": "sym", "entity": "呈现持续性或阵发性高波幅慢活动" } ] }, { "text": "大脑半球有病变时,在病变部位睡眠波(顶尖波、δ节律或κ复合波)可减弱或消失。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "大脑半球" }, { "start_idx": 9, "end_idx": 36, "type": "sym", "entity": "在病变部位睡眠波(顶尖波、δ节律或κ复合波)可减弱或消失" } ] }, { "text": "在药物睡眠时,除慢活动外,还重叠有很多快活动。", "entities": [ { "start_idx": 0, "end_idx": 21, "type": "sym", "entity": "在药物睡眠时,除慢活动外,还重叠有很多快活动" } ] }, { "text": "在昏迷时只有不同程度的慢活动,而不出现睡眠波。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "昏迷" } ] }, { "text": "(五)儿童异常脑电图对儿童脑电图异常的判断较成人困难,需要熟悉儿童各年龄期的脑电图特点。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "脑电图" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "脑电图" }, { "start_idx": 38, "end_idx": 40, "type": "pro", "entity": "脑电图" } ] }, { "text": "如果某一儿童的脑电图不符合或有异于该年龄组别的脑电图式样,为异常脑电图,提示该儿童的大脑发育出现障碍,或大脑受到某些疾病的损害。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "脑电图" }, { "start_idx": 23, "end_idx": 25, "type": "pro", "entity": "脑电图" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "脑电图" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "大脑" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "大脑" } ] }, { "text": "2岁以后很多每秒4~6周波慢波,6岁以后有中量每秒4~7周波慢活动均属不正常。", "entities": [ { "start_idx": 0, "end_idx": 32, "type": "sym", "entity": "2岁以后很多每秒4~6周波慢波,6岁以后有中量每秒4~7周波慢活动" } ] }, { "text": "出现棘波、尖波、病理复合波或高度失律,以及爆发性抑制活动或平坦活动,局限性高幅快波或慢波以及经常不对称,则不论任何年龄组均应视为异常。", "entities": [ { "start_idx": 0, "end_idx": 50, "type": "sym", "entity": "出现棘波、尖波、病理复合波或高度失律,以及爆发性抑制活动或平坦活动,局限性高幅快波或慢波以及经常不对称" } ] }, { "text": "(六)临床应用脑电图检查对癫痫、颅内占位性病变以及中枢神经系统感染性疾病等阳性率高达80%~90%。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "脑电图" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "癫痫" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "颅内占位性病变" }, { "start_idx": 25, "end_idx": 35, "type": "dis", "entity": "中枢神经系统感染性疾病" } ] }, { "text": "脑电图也常用于脑外伤、脑血管疾病或躯体性疾病引起中枢神经系统功能失调或损害的检查。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "脑电图" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "脑外伤" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "脑血管疾病" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "躯体性疾病" }, { "start_idx": 24, "end_idx": 36, "type": "sym", "entity": "中枢神经系统功能失调或损害" } ] }, { "text": "根据异常脑电波出现是弥漫性还是局限性,可判断病变的范围,如系局限性的异常则有定位诊断的作用。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "pro", "entity": "脑电波" } ] }, { "text": "病变部位可出现局灶性δ波。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "病变部位可出现局灶性δ波" } ] }, { "text": "脑电图检查时常规使用一些诱发方法,使不明显的异常电活动诱发出来,如视反应、过度换气以及闪光刺激等,还可做睡眠诱发及药物诱发试验等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "脑电图" } ] }, { "text": "另外,还可加用特殊电极引导出某部位的异常脑波,如鼻咽电极、蝶骨电极、枕下电极、皮层电极以及深部电极等。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "equ", "entity": "鼻咽电极" }, { "start_idx": 29, "end_idx": 32, "type": "equ", "entity": "蝶骨电极" }, { "start_idx": 34, "end_idx": 37, "type": "equ", "entity": "枕下电极" }, { "start_idx": 39, "end_idx": 42, "type": "equ", "entity": "皮层电极" }, { "start_idx": 45, "end_idx": 48, "type": "equ", "entity": "深部电极" } ] }, { "text": "近年还开展了24小时动态监测脑电图和视屏同步监测脑电图,使异常脑电波发现率明显增高。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "脑电图" }, { "start_idx": 24, "end_idx": 26, "type": "pro", "entity": "脑电图" }, { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "脑电波" } ] }, { "text": "动态脑电图是脑电图的一个重要分支,与常规脑电图相比,动态脑电图有以下特点:①导联组合不能随意变换,一般动态脑电图以时间/事件为记录目的,采用24小时导联不变的记录方式;②记录脑电图信息不等,常规脑电图仅记录20~30分钟的包括额、中央、顶、枕及颞五个部位的脑电活动,动态脑电图可记录24小时脑电活动,信息量相当于普通脑电图的46~71倍;③记录状态不同常规脑电图通常在静息状态描记,而动态脑电图可记录静息、活动、立、卧、坐等不同体位不同状态下的随意脑电图。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "动态脑电图" }, { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "脑电图" }, { "start_idx": 20, "end_idx": 22, "type": "pro", "entity": "脑电图" }, { "start_idx": 26, "end_idx": 30, "type": "pro", "entity": "动态脑电图" }, { "start_idx": 51, "end_idx": 55, "type": "pro", "entity": "动态脑电图" }, { "start_idx": 87, "end_idx": 89, "type": "pro", "entity": "脑电图" }, { "start_idx": 95, "end_idx": 99, "type": "pro", "entity": "常规脑电图" }, { "start_idx": 113, "end_idx": 113, "type": "bod", "entity": "额" }, { "start_idx": 115, "end_idx": 116, "type": "bod", "entity": "中央" }, { "start_idx": 118, "end_idx": 118, "type": "bod", "entity": "顶" }, { "start_idx": 120, "end_idx": 120, "type": "bod", "entity": "枕" }, { "start_idx": 122, "end_idx": 122, "type": "bod", "entity": "颞" }, { "start_idx": 133, "end_idx": 137, "type": "pro", "entity": "动态脑电图" }, { "start_idx": 158, "end_idx": 160, "type": "pro", "entity": "脑电图" }, { "start_idx": 176, "end_idx": 180, "type": "pro", "entity": "常规脑电图" }, { "start_idx": 192, "end_idx": 196, "type": "pro", "entity": "动态脑电图" }, { "start_idx": 224, "end_idx": 226, "type": "pro", "entity": "脑电图" } ] }, { "text": "动态脑电图在儿科的适应证:①监测正常人群中异常脑电活动情况,及早发现潜在病灶;②用于癫痫诊断和脑电图鉴别及分型;③用于昏迷病人及危重病人的实时脑电监测;④各种器质性病变严重程度及危险性估计;⑤麻醉的保护;⑥晕厥等不明原因的发作性疾患的病因诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "动态脑电图" }, { "start_idx": 6, "end_idx": 7, "type": "dep", "entity": "儿科" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "癫痫" }, { "start_idx": 47, "end_idx": 49, "type": "pro", "entity": "脑电图" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "昏迷" }, { "start_idx": 96, "end_idx": 97, "type": "pro", "entity": "麻醉" }, { "start_idx": 103, "end_idx": 104, "type": "dis", "entity": "晕厥" } ] }, { "text": "动态脑电图只能记录脑电图的表现,而病人的临床症状医生很难观察到,视频脑电在临床实时记录病人发作期或发作间期的脑电活动及体态活动,为医生提供最为直接、准确的诊断依据,是一非常重要的神经诊断技术。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "动态脑电图" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "脑电图" } ] }, { "text": "视频脑电图在儿科的适应证:①可疑癫痫病人的确诊。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "视频脑电图" }, { "start_idx": 6, "end_idx": 7, "type": "dep", "entity": "儿科" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "癫痫" } ] }, { "text": "对多数病人,常规脑电图已能提供足够的脑电活动信息,但约20%的病人在临床上没有明显的发作,通过实时脑电图记录,对癫痫类型的判定和癫痫分类,尤其对全身性癫痫棘波灶的起源和定位提供了前所未有的有利依据。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "常规脑电图" }, { "start_idx": 47, "end_idx": 51, "type": "pro", "entity": "实时脑电图" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "癫痫" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "癫痫" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "全身性癫痫" } ] }, { "text": "②癫痫的鉴别诊断。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "癫痫" } ] }, { "text": "一些非癫痫发作疾病(如屏气发作、晕厥、睡眠障碍及心因性疾患等)被错误诊断为癫痫的发作性疾患,依靠视频脑电图在临床发作期的敏感性和特异性,可得到鉴别。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "屏气发作" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "晕厥" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "睡眠障碍" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "心因性疾患" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "癫痫" }, { "start_idx": 48, "end_idx": 52, "type": "pro", "entity": "视频脑电图" } ] }, { "text": "③难治性癫痫术前定位。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "难治性癫痫" } ] }, { "text": "可有助于局灶性难治性癫痫的致痫灶的定位,通过临床表现、影像学、功能性检查尤其是视频脑电图发现的病灶进行联合定位,对手术切除癫痫灶提供可靠信息。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "局灶性难治性癫痫" }, { "start_idx": 39, "end_idx": 43, "type": "pro", "entity": "视频脑电图" }, { "start_idx": 57, "end_idx": 58, "type": "pro", "entity": "手术" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "癫痫灶" } ] }, { "text": "惊跳及呼吸不规则等新生儿和婴儿期特有的行为有时被误认为癫痫样发作,可经实时脑电监控得到识别。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "惊跳" }, { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "呼吸不规则" } ] }, { "text": "⑤儿童癫痫综合征的分类。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "儿童癫痫综合征" } ] }, { "text": "大田原综合征及West综合征等经常规脑电图如未能发现特异性脑电变化的,可经视频脑电描记,结合同时记录的临床发作而得以及早诊断。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "大田原综合征" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "West综合征" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "常规脑电图" } ] }, { "text": "第六节真菌性肺炎引起真菌性肺炎(fungalpneumonia)的病原有白色念珠菌、隐球菌、曲菌、毛霉菌、放线菌、组织胞质菌、芽生霉菌等,其中以白色念珠菌最常见,致病力最强。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "真菌性肺炎" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "真菌性肺炎" }, { "start_idx": 16, "end_idx": 30, "type": "dis", "entity": "fungalpneumonia" }, { "start_idx": 36, "end_idx": 40, "type": "mic", "entity": "白色念珠菌" }, { "start_idx": 42, "end_idx": 44, "type": "mic", "entity": "隐球菌" }, { "start_idx": 46, "end_idx": 47, "type": "mic", "entity": "曲菌" }, { "start_idx": 49, "end_idx": 51, "type": "mic", "entity": "毛霉菌" }, { "start_idx": 53, "end_idx": 55, "type": "mic", "entity": "放线菌" }, { "start_idx": 57, "end_idx": 61, "type": "mic", "entity": "组织胞质菌" }, { "start_idx": 63, "end_idx": 66, "type": "mic", "entity": "芽生霉菌" }, { "start_idx": 72, "end_idx": 76, "type": "mic", "entity": "白色念珠菌" } ] }, { "text": "由于该菌广泛存在于自然界,可寄生在正常人的皮肤、口腔、肠道、阴道等处黏膜上,在正常情况下不致病,当人体抵抗力低下时可致病。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "皮肤" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "口腔" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肠道" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "阴道" } ] }, { "text": "叶启慈报道经病理解剖证实的真菌性肺炎35例,以念珠菌最常见(48.5%),曲菌次之,其中新生儿14例(占40%),继发于其他疾病19例,胸腺发育异常25例(71%)。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "真菌性肺炎" }, { "start_idx": 23, "end_idx": 25, "type": "mic", "entity": "念珠菌" }, { "start_idx": 37, "end_idx": 38, "type": "mic", "entity": "曲菌" } ] }, { "text": "胸部X线检查有点片状阴影,可似粟粒性结核,以两肺中下部多见,或肺门阴影增浓、肺纹理增多,可有大片实变病灶,少数有胸腔积液及心包积液等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "胸部X线" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "肺门阴影增浓" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "肺纹理增多" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "胸腔积液" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "心包积液" } ] }, { "text": "有基础疾病的患儿,肺炎病程迁延不愈,抗生素治疗无效甚至恶化,应考虑本病可能。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "抗生素治疗" } ] }, { "text": "从痰等下呼吸道标本中找到真菌孢子及假菌丝,或培养阳性可诊断。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "mic", "entity": "真菌孢子" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "假菌丝" } ] }, { "text": "两性霉素B脂质体可提高疗效,减少不良反应。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dru", "entity": "两性霉素B脂质体" } ] }, { "text": "广谱抗真菌药氟康唑对念珠菌、隐球菌抗菌活性最强,但对曲菌的作用差,可用于治疗念珠菌、隐球菌感染。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "氟康唑" }, { "start_idx": 10, "end_idx": 12, "type": "mic", "entity": "念珠菌" }, { "start_idx": 14, "end_idx": 16, "type": "mic", "entity": "隐球菌" }, { "start_idx": 26, "end_idx": 27, "type": "mic", "entity": "曲菌" }, { "start_idx": 38, "end_idx": 40, "type": "mic", "entity": "念珠菌" }, { "start_idx": 42, "end_idx": 44, "type": "mic", "entity": "隐球菌" } ] }, { "text": "可酌情加用转移因子等免疫调节剂。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "免疫调节剂" } ] }, { "text": "二、恶性纤维组织细胞瘤恶性纤维组织细胞瘤(malignantfibroushistocytoma,MFH),也称纤维组织细胞肉瘤、纤维黄色肉瘤、恶性纤维黄色瘤等。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "恶性纤维组织细胞瘤" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "恶性纤维组织细胞瘤" }, { "start_idx": 21, "end_idx": 47, "type": "dis", "entity": "malignantfibroushistocytoma" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "MFH" }, { "start_idx": 56, "end_idx": 63, "type": "dis", "entity": "纤维组织细胞肉瘤" }, { "start_idx": 65, "end_idx": 70, "type": "dis", "entity": "纤维黄色肉瘤" }, { "start_idx": 72, "end_idx": 78, "type": "dis", "entity": "恶性纤维黄色瘤" } ] }, { "text": "肿瘤多呈结节状大小不一,从2cm到20cm,多无包膜切面为灰白色,鱼肉状,可有出血坏死,有时含有囊腔。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "肿瘤多呈结节状" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "大小不一" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "从2cm到20cm" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "包膜" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "多无包膜" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "切面为灰白色" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "鱼肉状" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "可有出血坏死" } ] }, { "text": "显微镜下肿瘤主要由组织细胞样细胞和成纤维细胞样细胞构成,呈多形性,典型和不典型核分裂象多见于组织细胞样细胞。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "显微镜" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "肿瘤" }, { "start_idx": 9, "end_idx": 15, "type": "bod", "entity": "组织细胞样细胞" }, { "start_idx": 17, "end_idx": 24, "type": "bod", "entity": "成纤维细胞样细胞" }, { "start_idx": 46, "end_idx": 52, "type": "bod", "entity": "组织细胞样细胞" } ] }, { "text": "肿瘤多发于四肢,其次是躯干,腹腔内、腹膜后、骨骼、乳房也均有可能。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "四肢" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "躯干" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "腹腔内" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "腹膜后" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "骨骼" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "乳房" } ] }, { "text": "肿块呈单个结节状,开始时可很少,但常易侵犯局部深筋膜,有时呈溃疡状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肿块" }, { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "肿块呈单个结节状,开始时可很少" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "深筋膜" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "有时呈溃疡状" } ] }, { "text": "肿瘤早期即可向区域淋巴结和远处转移,深部肿瘤可发生远处转移灶的症状先于原发灶的情况。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "淋巴结" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肿瘤" } ] }, { "text": "第一节川崎病川崎病(Kawasakidisease,KD)又称皮肤黏膜淋巴结综合征(mucocutaneouslymphnodesyndrome,MCLS),是一种病因未明的全身性血管炎综合征。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "川崎病" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "川崎病" }, { "start_idx": 10, "end_idx": 24, "type": "bod", "entity": "Kawasakidisease" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "KD" }, { "start_idx": 31, "end_idx": 40, "type": "bod", "entity": "皮肤黏膜淋巴结综合征" }, { "start_idx": 42, "end_idx": 71, "type": "bod", "entity": "mucocutaneouslymphnodesyndrome" }, { "start_idx": 73, "end_idx": 76, "type": "bod", "entity": "MCLS" }, { "start_idx": 87, "end_idx": 95, "type": "bod", "entity": "全身性血管炎综合征" } ] }, { "text": "表现为发热,皮疹,球结合膜、口腔黏膜充血,手足红斑、硬性水肿及颈淋巴结肿大。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "发热" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "皮疹" }, { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "球结合膜、口腔黏膜充血" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "手足红斑" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "硬性水肿" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "颈淋巴结肿大" } ] }, { "text": "主要病理改变为全身性中、小动脉炎,最严重的危害是病程中、后期发生的中大动脉损伤,尤其是冠状动脉损害,是儿童最重要的后天性心脏病之一。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "全身性中、小动脉炎" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "中大动脉损伤" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "冠状动脉损害" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "后天性心脏病" } ] }, { "text": "据统计,发热10天内未经及时治疗者,冠状动脉病变发生率达20%~25%。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "发热" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "冠状动脉" } ] }, { "text": "即使经阿司匹林治疗也有约15%患儿发生冠脉病变,因而其危害性应予高度关注。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "冠脉" } ] }, { "text": "【病因与发病机制】病因不明,感染是本病重要的致病因素之一,在川崎患儿体内常可发现链球菌、葡萄球菌、反转录病毒以及支原体等病原感染的证据。", "entities": [ { "start_idx": 40, "end_idx": 42, "type": "mic", "entity": "链球菌" }, { "start_idx": 44, "end_idx": 47, "type": "mic", "entity": "葡萄球菌" }, { "start_idx": 49, "end_idx": 53, "type": "mic", "entity": "反转录病毒" }, { "start_idx": 56, "end_idx": 58, "type": "mic", "entity": "支原体" } ] }, { "text": "有关发病机制有下列学说:(一)免疫过度活化研究认为免疫反应亢进是致病的重要环节,突出表现在急性期CD30+T细胞增多,部分细胞因子分泌异常,B细胞多克隆活化,循环中有抗内皮细胞毒性抗体及抗中性粒胞浆抗体等体液免疫反应亢进现象;异常增高的IL-6能抑制淋巴细胞p53基因表达,淋巴细胞凋亡时间明显延迟,最终导致免疫细胞过度活化。", "entities": [ { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "T细胞" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "细胞因子" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "B细胞" }, { "start_idx": 89, "end_idx": 97, "type": "bod", "entity": "抗内皮细胞毒性抗体" }, { "start_idx": 99, "end_idx": 106, "type": "bod", "entity": "抗中性粒胞浆抗体" }, { "start_idx": 124, "end_idx": 127, "type": "bod", "entity": "IL-6" }, { "start_idx": 131, "end_idx": 139, "type": "bod", "entity": "淋巴细胞p53基因" }, { "start_idx": 143, "end_idx": 146, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 160, "end_idx": 163, "type": "bod", "entity": "免疫细胞" } ] }, { "text": "(二)超抗原近年有人证实葡萄球菌内毒素和链球菌红斑毒素可作为一种超抗原启动暂时性异常免疫反应。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "mic", "entity": "葡萄球菌" }, { "start_idx": 20, "end_idx": 22, "type": "mic", "entity": "链球菌" }, { "start_idx": 23, "end_idx": 26, "type": "mic", "entity": "红斑毒素" } ] }, { "text": "超抗原可不经抗原递呈细胞(APC)处理即能直接激活T细胞或与APC表面MHCⅡ类抗原结合后刺激T细胞活化,释放大量淋巴因子如IL-4及IL-6,从而介导或放大自身免疫损伤。", "entities": [ { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "T细胞" }, { "start_idx": 30, "end_idx": 41, "type": "bod", "entity": "APC表面MHCⅡ类抗原" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "T细胞" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "淋巴因子" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "IL-4" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "IL-6" } ] }, { "text": "(三)热休克蛋白因细菌体热休克蛋白65(HSP65)成分与人类HSP60有高度同源性,川崎患儿细菌感染后,其HSP65诱导人体局限于血管组织的HSP60表达增强,通过抗原分子间的模拟机制刺激机体产生了针对自身血管的免疫损伤。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "细菌" }, { "start_idx": 26, "end_idx": 30, "type": "mic", "entity": "HSP65" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "HSP60" }, { "start_idx": 65, "end_idx": 66, "type": "mic", "entity": "细菌" }, { "start_idx": 72, "end_idx": 76, "type": "mic", "entity": "HSP65" }, { "start_idx": 90, "end_idx": 93, "type": "bod", "entity": "血管组织" }, { "start_idx": 95, "end_idx": 99, "type": "mic", "entity": "HSP60" }, { "start_idx": 134, "end_idx": 135, "type": "bod", "entity": "血管" } ] }, { "text": "【病理】病初以小血管炎为主,以后累及主动脉等中、大动脉,特别好发于冠状动脉及其分支,未经及时治疗的病例其病理改变大致可分为4期:Ⅰ期:1~9天,主要是小血管炎、微血管周围炎以及中等大小动脉周围炎,如冠状动脉周围炎;在心肌间质、心包及心内膜有中性粒细胞、嗜酸性粒细胞和淋巴细胞浸润。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "小血管炎" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "主动脉" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "中、大动脉" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 75, "end_idx": 78, "type": "dis", "entity": "小血管炎" }, { "start_idx": 80, "end_idx": 85, "type": "dis", "entity": "微血管周围炎" }, { "start_idx": 88, "end_idx": 96, "type": "dis", "entity": "中等大小动脉周围炎" }, { "start_idx": 99, "end_idx": 105, "type": "dis", "entity": "冠状动脉周围炎" }, { "start_idx": 108, "end_idx": 111, "type": "bod", "entity": "心肌间质" }, { "start_idx": 113, "end_idx": 114, "type": "bod", "entity": "心包" }, { "start_idx": 116, "end_idx": 118, "type": "bod", "entity": "心内膜" }, { "start_idx": 120, "end_idx": 124, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 126, "end_idx": 131, "type": "bod", "entity": "嗜酸性粒细胞" }, { "start_idx": 133, "end_idx": 136, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "Ⅱ期:12~25天,小血管炎减轻,冠状动脉主要分支等中等大小动脉全层血管炎(内膜、外膜及中膜均有炎性细胞浸润)突出,伴有坏死、水肿,血管弹力纤维和肌层断裂,出现冠状动脉扩张,易发生冠状动脉瘤及血栓。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "小血管炎" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 26, "end_idx": 31, "type": "bod", "entity": "中等大小动脉" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "血管炎" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "内膜" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "外膜" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "中膜" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "炎性细胞" }, { "start_idx": 60, "end_idx": 64, "type": "sym", "entity": "坏死、水肿" }, { "start_idx": 80, "end_idx": 83, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 80, "end_idx": 85, "type": "sym", "entity": "冠状动脉扩张" }, { "start_idx": 90, "end_idx": 94, "type": "dis", "entity": "冠状动脉瘤" }, { "start_idx": 96, "end_idx": 97, "type": "dis", "entity": "血栓" } ] }, { "text": "Ⅲ期:28~45天,小血管及微血管炎消退,中动脉发生肉芽肿及血栓,纤维组织增生,血管内膜增厚,冠状动脉一些分支可全部或部分阻塞,有冠状动脉瘤破裂急性血管炎消失,已经发生的血管内膜增厚,瘢痕、动脉瘤或血栓有一个漫长的吸收、修复过程。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "小血管及微血管炎" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "中动脉" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "肉芽肿" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "血栓" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "纤维组织" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "血管内膜" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "冠状动脉瘤" }, { "start_idx": 33, "end_idx": 71, "type": "sym", "entity": "纤维组织增生,血管内膜增厚,冠状动脉一些分支可全部或部分阻塞,有冠状动脉瘤破裂" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "急性血管炎" }, { "start_idx": 85, "end_idx": 90, "type": "sym", "entity": "血管内膜增厚" }, { "start_idx": 92, "end_idx": 93, "type": "sym", "entity": "瘢痕" }, { "start_idx": 95, "end_idx": 97, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 99, "end_idx": 100, "type": "dis", "entity": "血栓" } ] }, { "text": "狭窄、阻塞的血管可能修复、再通,心肌可能遗留永久的疤痕。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血管" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "心肌" } ] }, { "text": "早期严重心肌炎、中后期动脉瘤破裂与血管栓塞是本病死亡的主要风险。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "心肌炎" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "血管栓塞" } ] }, { "text": "【临床表现】(一)主要症状1.发热若无早期治疗,一般持续7~12天,少数有更长时间(2周至月余),多在39℃以上,呈稽留热或弛张热,抗生素治疗无效。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "发热" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "稽留热或弛张热" }, { "start_idx": 66, "end_idx": 68, "type": "dru", "entity": "抗生素" } ] }, { "text": "2.皮疹为多形性弥漫性红斑,有些近似麻疹样,一般无疱疹与结痂,躯干部多见,面部及四肢也可见上述皮疹。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "皮疹" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "麻疹" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "躯干部" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "面部" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "四肢" }, { "start_idx": 5, "end_idx": 44, "type": "sym", "entity": "多形性弥漫性红斑,有些近似麻疹样,一般无疱疹与结痂,躯干部多见,面部及四肢也可见" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "皮疹" } ] }, { "text": "发热后2~4天出疹,持续4~5天后消退。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "发热后2~4天出疹,持续4~5天后消退" } ] }, { "text": "3.双眼球结合膜充血无脓性分泌物,一般无糜烂。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "双眼球结合膜充血" }, { "start_idx": 10, "end_idx": 21, "type": "sym", "entity": "无脓性分泌物,一般无糜烂" } ] }, { "text": "4.唇红、干燥、皲裂口咽黏膜充血,舌乳头隆起似杨梅充血症状持续于整个发热期。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "唇红、干燥、皲裂" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "舌乳头" }, { "start_idx": 10, "end_idx": 24, "type": "sym", "entity": "口咽黏膜充血,舌乳头隆起似杨梅" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "充血" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "发热" } ] }, { "text": "5.手足硬肿手足掌现弥漫性红斑,趾、指末端硬肿突出,伴疼痛和僵直,9~14天开始出现特征性趾、指末端沿甲床膜状或薄片状脱屑,肛周也见类似脱屑。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "手足硬肿" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "弥漫性红斑" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "趾、指末端" }, { "start_idx": 16, "end_idx": 31, "type": "sym", "entity": "趾、指末端硬肿突出,伴疼痛和僵直" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "趾、指末端" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "肛周" }, { "start_idx": 42, "end_idx": 69, "type": "sym", "entity": "特征性趾、指末端沿甲床膜状或薄片状脱屑,肛周也见类似脱屑" } ] }, { "text": "6.颈部非化脓性淋巴结炎一过性淋巴结肿大,直径约0.5~1.5cm,多为颈侧淋巴结,单侧多见,压痛轻,质较硬,不化脓。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "颈部非化脓性淋巴结炎" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "淋巴结" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "淋巴结肿大" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "颈侧淋巴结" }, { "start_idx": 42, "end_idx": 57, "type": "sym", "entity": "单侧多见,压痛轻,质较硬,不化脓" } ] }, { "text": "(二)其他症状心脏损害并不少见,可因冠状动脉炎伴动脉瘤和血栓栓塞而引起猝死。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心脏" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "冠状动脉炎" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "血栓栓塞" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "猝死" } ] }, { "text": "有不同程度心肌炎、心包炎、心内膜炎和心律失常,偶可闻奔马律、心音低钝和心音分裂,可发生心肌梗死、心力衰竭、高血压及心源性休克等;少数患儿有惊厥、昏迷、中枢性、外周性神经麻痹以及精神、情绪异常等无菌性脑炎、脑膜炎症状;也有关节痛、关节炎,咳嗽、间质性肺炎的报告,上述症状多于病程1~6周出现。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "心肌炎" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "心包炎" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "心律失常" }, { "start_idx": 23, "end_idx": 38, "type": "sym", "entity": "偶可闻奔马律、心音低钝和心音分裂" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "心肌梗死" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "高血压" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "心源性休克" }, { "start_idx": 96, "end_idx": 100, "type": "dis", "entity": "无菌性脑炎" }, { "start_idx": 102, "end_idx": 104, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 69, "end_idx": 106, "type": "sym", "entity": "惊厥、昏迷、中枢性、外周性神经麻痹以及精神、情绪异常等无菌性脑炎、脑膜炎症状" }, { "start_idx": 114, "end_idx": 116, "type": "dis", "entity": "关节炎" }, { "start_idx": 121, "end_idx": 125, "type": "dis", "entity": "间质性肺炎" }, { "start_idx": 110, "end_idx": 125, "type": "sym", "entity": "关节痛、关节炎,咳嗽、间质性肺炎" } ] }, { "text": "极少数患儿可合并巨噬细胞活化(MAS)等严重并发症,甚至可威胁生命。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "巨噬细胞活化" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "MAS" } ] }, { "text": "【辅助检查】轻度贫血,外周血白细胞数增加,以中性粒细胞增加为主,有核左移现象血小板早期正常,第2~3周显著增高,血液呈高凝状态,血浆黏度增高,血浆纤维蛋白原增加。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 6, "end_idx": 37, "type": "sym", "entity": "轻度贫血,外周血白细胞数增加,以中性粒细胞增加为主,有核左移现象" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "血小板" } ] }, { "text": "血清IgG、IgA、IgM、IgE和血循环免疫复合物升高,类风湿因子及抗核抗体均阴性。", "entities": [ { "start_idx": 0, "end_idx": 27, "type": "sym", "entity": "血清IgG、IgA、IgM、IgE和血循环免疫复合物升高" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "类风湿因子" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "抗核抗体" } ] }, { "text": "部分患儿ALT和AST升高,血清蛋白电泳可见球蛋白升高。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "ALT" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "AST" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "血清蛋白" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "电泳" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "球蛋白升高" } ] }, { "text": "尿沉渣中白细胞数增多,轻度蛋白尿。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "白细胞" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "尿沉渣中白细胞数增多" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "轻度蛋白尿" } ] }, { "text": "病程第1周常见各类心电图异常,如心动过速,ST-T改变,各种房室传导阻滞,T波改变及心律紊乱。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "心电图" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "心动过速" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "ST-T改变" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "各种房室传导阻滞" }, { "start_idx": 37, "end_idx": 45, "type": "sym", "entity": "T波改变及心律紊乱" } ] }, { "text": "【诊断标准】多采用日本MCLS研究会或第三次国际川崎病研讨会提出的诊断标准。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "MCLS" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "川崎病" } ] }, { "text": "1.不明原因发热5天以上;2.双侧球结膜弥漫性充血,无渗出物;3.口唇潮红,皲裂,口咽黏膜充血,杨梅舌;4.病初(1~9天)手足指趾肿胀,掌跖潮红,恢复期(9~21天)出现指趾端膜状脱屑或肛周脱屑;5.躯干及四肢多形充血性红斑;6.颈淋巴结非化脓性肿大,直径达1.5cm或更大。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "sym", "entity": "不明原因发热5天以上" }, { "start_idx": 15, "end_idx": 29, "type": "sym", "entity": "双侧球结膜弥漫性充血,无渗出物" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "口咽黏膜" }, { "start_idx": 33, "end_idx": 50, "type": "sym", "entity": "口唇潮红,皲裂,口咽黏膜充血,杨梅舌" }, { "start_idx": 62, "end_idx": 67, "type": "sym", "entity": "手足指趾肿胀" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "掌跖潮红" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "肛周" }, { "start_idx": 86, "end_idx": 97, "type": "sym", "entity": "指趾端膜状脱屑或肛周脱屑" }, { "start_idx": 104, "end_idx": 105, "type": "bod", "entity": "四肢" }, { "start_idx": 101, "end_idx": 112, "type": "sym", "entity": "躯干及四肢多形充血性红斑" }, { "start_idx": 116, "end_idx": 125, "type": "sym", "entity": "颈淋巴结非化脓性肿大" } ] }, { "text": "【鉴别诊断】(一)猩红热皮疹发生早(1~2天),粟粒样均匀丘疹,疹间皮肤潮红,发病年龄普遍>3岁,青霉素治疗有效。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "猩红热" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "皮疹" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "粟粒样均匀丘疹" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "疹间皮肤潮红" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "青霉素" } ] }, { "text": "(二)幼年特发性关节炎(JIA)可为高热,反复隐现多型皮疹(热退疹隐),热程反复、迁延,常为关节肿痛。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "幼年特发性关节炎" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "JIA" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "高热" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "多型皮疹" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "热退疹隐" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "关节肿痛" } ] }, { "text": "(三)渗出性红斑常见口唇、眼角多处黏膜糜烂,常有脓性渗出,假膜形成。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "渗出性红斑" }, { "start_idx": 10, "end_idx": 32, "type": "sym", "entity": "口唇、眼角多处黏膜糜烂,常有脓性渗出,假膜形成" } ] }, { "text": "皮疹广泛、大片,有水泡和结痂。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "皮疹广泛、大片,有水泡和结痂" } ] }, { "text": "(四)系统性红斑狼疮面部蝶形、盘状红斑、脱发、关节炎、白细胞减少、血小板减少及抗核抗体阳性阿司匹林发热时用量30~50mg/(kg•d),热退后2~3天可根据血小板数及血凝状态调整剂量,一般为5~10mg/(kg•d)再用6~8周。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "关节炎" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "白细胞" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "血小板" }, { "start_idx": 10, "end_idx": 44, "type": "sym", "entity": "面部蝶形、盘状红斑、脱发、关节炎、白细胞减少、血小板减少及抗核抗体阳性" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "血小板" } ] }, { "text": "有冠状动脉病变者用药疗程延至冠状动脉病变恢复正常。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "冠状动脉病变" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "冠状动脉病变" } ] }, { "text": "(二)静脉注射用丙种球蛋白(IVIG)治疗本病疗效突出。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "静脉注射" }, { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "丙种球蛋白" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "IVIG" } ] }, { "text": "发热及充血症状可在24小时左右缓解。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "发热及充血" } ] }, { "text": "与单用阿司匹林治疗比较,冠状动脉病变发生率从18%降至4%左右,单次大剂量(2g/kg)比分次给药(每天400mg/kg,连用5天)有更佳疗效,急性期症状缓解更快,冠状动脉病变发生率更低。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "冠状动脉病变" }, { "start_idx": 82, "end_idx": 87, "type": "dis", "entity": "冠状动脉病变" } ] }, { "text": "但IVIG给药若在10天之后,冠状动脉病变预防效果将显著降低。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "IVIG" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "冠状动脉病变" } ] }, { "text": "IVIG输注同时和输注后1~2个月仍需合用阿司匹林以取得最佳疗效,防止恢复期高凝状态。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "IVIG" }, { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "输注" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "输注" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "阿司匹林" } ] }, { "text": "IVIG虽为目前最佳药物,但鉴于经济原因也有人主张适用于冠状动脉病变高危患儿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "IVIG" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "冠状动脉病变" } ] }, { "text": "高危因素包括:①年龄<1岁,男性;②血细胞压积<0.35;③血浆蛋白<35g/L;④血C反应蛋白强阳性;⑤血小板数第1周低于200×109/L。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "血细胞" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "血浆蛋白" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "血C反应蛋白" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "血小板" } ] }, { "text": "目前有关对IVIG治疗无反应(5%~10%)的临床报告在逐渐增加。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "IVIG" } ] }, { "text": "(三)双嘧达莫加用双嘧达莫3~5mg/(kg•d),可抗血小板聚集的作用。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "双嘧达莫" } ] }, { "text": "(四)皮质激素具有强大抗炎、抗过敏,抑制免疫反应的药理作用,能减轻血管内皮损伤,进而减轻冠脉损害。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "皮质激素" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "冠脉" } ] }, { "text": "近年主张皮质激素用于IVIG无反应川崎患儿,在首剂给予IVIG2g/kg后36小时发热不退,追加IVIG2g/kg,36小时仍发热,除外感染之后,可考虑短程小剂量使用皮质激素。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "皮质激素" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "IVIG" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "IVIG2" }, { "start_idx": 48, "end_idx": 51, "type": "dru", "entity": "IVIG" }, { "start_idx": 83, "end_idx": 86, "type": "dru", "entity": "皮质激素" } ] }, { "text": "(五)其他治疗急性期很快发生冠状动脉或心外动脉血栓者可用尿激酶或腹蛇抗栓酶治疗。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "冠状动脉" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "心外动脉血栓" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "尿激酶" }, { "start_idx": 32, "end_idx": 36, "type": "dru", "entity": "腹蛇抗栓酶" } ] }, { "text": "Kato等人用尿激酶(8000~10000U/kg),通过插管滴入冠状动脉内治疗15例巨大冠脉内血栓形成患者,结果血栓完全消失3/15例,部分消失4/15例,1例已有心肌梗死者出现再通,7例无变化,随访2年无死亡或心肌梗死病例发生。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "尿激酶" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "巨大冠脉内血栓" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "血栓" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "心肌梗死" }, { "start_idx": 104, "end_idx": 105, "type": "sym", "entity": "死亡" }, { "start_idx": 107, "end_idx": 110, "type": "dis", "entity": "心肌梗死" } ] }, { "text": "对于极少数难治性川崎病,近年来有使用生物制剂治疗的报道。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "川崎病" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "生物制剂" } ] }, { "text": "(六)随访本病退热出院后2个月内每2~4周随访心电图、B超及血小板一次,此后应每3个月随访一次到1~2年,有冠脉病变者要随访至病变消失后数年。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "pro", "entity": "心电图" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "B超" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "血小板" } ] }, { "text": "三、新生儿的神经心理发育儿童发育的基础与神经系统,尤其是脑的发育有密切的关系。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "神经系统" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "脑" } ] }, { "text": "新生儿脑重约390g,占出生体重的8%,为成人脑重的1/3。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "脑重" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "体重" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "脑重" } ] }, { "text": "大脑皮质细胞的分化从胎儿第5个月开始,出生时已具备了成人脑所具备的沟和回,但比成人的浅。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "大脑皮质细胞" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "脑" } ] }, { "text": "在组织学上也具备了大脑皮质层的6层基本结构。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "大脑皮质层" } ] }, { "text": "新生儿大脑的皮质下中枢,如苍白球、纹状体系统发育较成熟,而皮质的发育尚未成熟,所以新生儿出现肌张力增高及不自主的动作,兴奋及抑制过程容易扩散。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "大脑" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "皮质下中枢" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "苍白球" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "纹状体系统" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "皮质" } ] }, { "text": "随着大脑皮质的发育,对皮质下起的抑制作用也逐渐明显。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "大脑皮质" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "皮质下" } ] }, { "text": "(一)感知觉的发育1.视感知(1)眼的生长发育:正常足月新生儿出生时眼的大小为成人的3/4,在生后第1年内,发育最快,以后发育速度降低。", "entities": [ { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "眼" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "眼" } ] }, { "text": "一般来说,在出生时眼的前部结构相对较大,而较后部结构发育慢。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "眼" } ] }, { "text": "这种发育特征使小儿眼球形状处在不断变化之中,最后眼球近似球形。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "眼球" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "眼球" } ] }, { "text": "新生儿角膜相对较大,角膜弯曲度随年龄的增加而趋于平坦,因此眼的屈光度也在不断变化之中。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "角膜" }, { "start_idx": 10, "end_idx": 14, "type": "ite", "entity": "角膜弯曲度" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "眼" }, { "start_idx": 31, "end_idx": 33, "type": "ite", "entity": "屈光度" } ] }, { "text": "正常角膜无色透明,未成熟儿可呈暂时性的乳白色迷雾状新生儿前房较浅,并有角形结构眼内压的维持是重要的。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "角膜" }, { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "正常角膜无色透明,未成熟儿可呈暂时性的乳白色迷雾状" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "前房" }, { "start_idx": 25, "end_idx": 38, "type": "sym", "entity": "新生儿前房较浅,并有角形结构" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "眼内压" } ] }, { "text": "新生儿瞳孔色泽较成人浅,脉络膜血管高度清晰可见,黄斑特别是中央凹的光反射界限不够清楚眼底镜检查。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "瞳孔" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "脉络膜血管" }, { "start_idx": 0, "end_idx": 41, "type": "sym", "entity": "新生儿瞳孔色泽较成人浅,脉络膜血管高度清晰可见,黄斑特别是中央凹的光反射界限不够清楚" }, { "start_idx": 42, "end_idx": 46, "type": "pro", "entity": "眼底镜检查" } ] }, { "text": "有瞳孔对光反应,但感觉敏锐度较差,出生后1天的视力为20/150。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "瞳孔" } ] }, { "text": "视物最早的刺激是母亲的脸,特别是在哺乳时,在安静觉醒时,能注视人脸。", "entities": [ { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "脸" } ] }, { "text": "新生儿喜欢轮廓鲜明和深浅颜色对比强烈的图形,可能这种图形对视网膜刺激更大,因此黑白相间的棋盘比一块单纯白布更能吸引新生儿的注意力。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "视网膜" } ] }, { "text": "2.听感知发育很多研究证明胎儿在宫内即有听力,已能区别声音的强弱、声调的高低、熟悉或不熟悉的声音,甚至已能辨别声音来源的方向。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "宫内" } ] }, { "text": "新生儿从一出生即有声音的定向力,在新生儿觉醒状态,头向前方,在距小儿耳旁10~15cm发出柔和的格格声和铃声,新生儿会眨眼或转动头向声音发出的方向。", "entities": [ { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "头" }, { "start_idx": 55, "end_idx": 72, "type": "sym", "entity": "新生儿会眨眼或转动头向声音发出的方向" } ] }, { "text": "3.皮肤感觉的发育触觉器官最大,全身皮肤都有灵敏的触觉,出生后的新生儿身体喜欢紧贴着温暖的环境,因此在怀抱新生儿时,他们会紧贴在抱者的怀里。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "皮肤" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "触觉器官" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "皮肤" } ] }, { "text": "尤其当他们哭闹时,成人通过触觉刺激,将手放在新生儿的腹部,并按住他们两个手臂,就能使他们安静下来。", "entities": [ { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "手" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "腹部" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "手臂" } ] }, { "text": "新生儿的触觉有高度的灵敏性,尤其是在眼、前额、口周、手掌、足底等部位,而大腿、前臂、躯干处却比较迟钝,这可以解释新生儿吸吮手指躯干的有些反射出现与触觉的敏感性有关。", "entities": [ { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "眼" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "前额" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "口周" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "手掌" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "足底" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "大腿" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "前臂" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "躯干" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "手指" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "吸吮手指" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "躯干" } ] }, { "text": "新生儿痛觉不甚敏感,尤其在躯干、眼、腋下部位的痛刺激出现泛化现象。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "躯干" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "眼" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "腋下" } ] }, { "text": "哺乳时,当闻到乳的香味时就会积极地寻找乳头,而当闻到不愉快的气味时则转过头去。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "乳头" } ] }, { "text": "他们喜欢较甜的糖水,吸吮浓度较高的糖水比浓度较低的糖水量多,吸吮力强。", "entities": [ { "start_idx": 10, "end_idx": 33, "type": "sym", "entity": "吸吮浓度较高的糖水比浓度较低的糖水量多,吸吮力强" } ] }, { "text": "对于咸的、酸的或苦的液体有不愉快的表情。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "对于咸的、酸的或苦的液体有不愉快的表情" } ] }, { "text": "其睡眠生理与年长儿不同,其快速眼动睡眠每天约8~9小时。", "entities": [ { "start_idx": 0, "end_idx": 26, "type": "sym", "entity": "其睡眠生理与年长儿不同,其快速眼动睡眠每天约8~9小时" } ] }, { "text": "入睡的模式是从醒觉至快速眼动睡眠,新生儿的快速眼动睡眠周期短,约50~60分钟出现1次快速眼动,而成人则为90~100分钟。", "entities": [ { "start_idx": 17, "end_idx": 46, "type": "sym", "entity": "新生儿的快速眼动睡眠周期短,约50~60分钟出现1次快速眼动" } ] }, { "text": "此外,新生儿上半夜的快速眼动与下半夜的一样多。", "entities": [ { "start_idx": 3, "end_idx": 21, "type": "sym", "entity": "新生儿上半夜的快速眼动与下半夜的一样多" } ] }, { "text": "新生儿的觉醒和睡眠按不同程度分为6种表现状态,即两种睡眠状态,安静睡眠(深睡)和活动睡眠(浅睡);三种觉醒状态,安静觉醒、活动觉醒和哭;另一种是介于睡眠和觉醒之间的过渡形式,即瞌睡状态。", "entities": [ { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "安静睡眠(深睡)" }, { "start_idx": 40, "end_idx": 47, "type": "sym", "entity": "活动睡眠(浅睡)" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "安静觉醒" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "活动觉醒" }, { "start_idx": 66, "end_idx": 66, "type": "sym", "entity": "哭" }, { "start_idx": 88, "end_idx": 91, "type": "sym", "entity": "瞌睡状态" } ] }, { "text": "1.安静觉醒状态新生儿在这种状态下很机敏,喜欢看东西,特别是圆形和色彩鲜艳的东西。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "安静觉醒状态" } ] }, { "text": "还喜欢注视父母的脸,专心地听他们说话。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "脸" } ] }, { "text": "这种安静觉醒的时间很短,刚生下的新生儿约有40分钟的安静觉醒时间。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "安静觉醒" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "安静觉醒" } ] }, { "text": "2.活动觉醒状态吃奶前或烦躁时,活动增加,眼和脸部活动也增加,并发出声音有时运动很剧烈,甚至出现自发的惊跳。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "活动觉醒状态" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "烦躁" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "眼" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "脸部" }, { "start_idx": 16, "end_idx": 35, "type": "sym", "entity": "活动增加,眼和脸部活动也增加,并发出声音" }, { "start_idx": 36, "end_idx": 52, "type": "sym", "entity": "有时运动很剧烈,甚至出现自发的惊跳" } ] }, { "text": "有时运动呈阵发性,伴有特殊的节律。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "有时运动呈阵发性,伴有特殊的节律" } ] }, { "text": "在这种状态下,如新生儿受到不愉快的刺激,则可使其活动增强或惊跳。", "entities": [ { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "活动增强或惊跳" } ] }, { "text": "3.哭的状态新生儿哭时四肢有力地活动,眼可张开或紧闭,脸有时变得很红哭是新生儿表示意愿的一种方式,如饿了、尿布湿了或身体不适时哭,求助于父母能满足他们的要求。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "sym", "entity": "哭" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "眼" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "脸" }, { "start_idx": 9, "end_idx": 33, "type": "sym", "entity": "哭时四肢有力地活动,眼可张开或紧闭,脸有时变得很红" }, { "start_idx": 34, "end_idx": 34, "type": "sym", "entity": "哭" } ] }, { "text": "也可在刚醒时,哭一会儿进入安静觉醒状态。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "安静觉醒状态" } ] }, { "text": "4.瞌睡状态通常发生在刚醒后或入睡前。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "瞌睡状态" } ] }, { "text": "眼半睁半闭,眼睑出现闪动,眼闭和前眼球可能向上滚动有时微笑、皱眉或噘嘴唇,目光呆滞,反应迟钝。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "眼" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "眼睑" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "眼闭" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "前眼球" }, { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "眼半睁半闭,眼睑出现闪动,眼闭和前眼球可能向上滚动" }, { "start_idx": 25, "end_idx": 45, "type": "sym", "entity": "有时微笑、皱眉或噘嘴唇,目光呆滞,反应迟钝" } ] }, { "text": "对声音或图形表现茫然。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "对声音或图形表现茫然" } ] }, { "text": "常伴有轻度惊跳,这是觉醒和睡眠之间的过渡阶段,持续时间较短。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "轻度惊跳" } ] }, { "text": "5.活动睡眠状态新生儿在活动睡眠时,眼睛通常是闭合的,但偶然短暂地睁一下,眼睑有时颤动,经常可见到眼球在眼睑下快速运动小儿呼吸不规则,比安静睡眠时稍快。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "活动睡眠状态" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "活动睡眠" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "眼睛" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "眼睑" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "眼球" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "眼睑" }, { "start_idx": 18, "end_idx": 58, "type": "sym", "entity": "眼睛通常是闭合的,但偶然短暂地睁一下,眼睑有时颤动,经常可见到眼球在眼睑下快速运动" }, { "start_idx": 59, "end_idx": 74, "type": "sym", "entity": "小儿呼吸不规则,比安静睡眠时稍快" } ] }, { "text": "手臂、腿和整个身体偶然有些活动,且脸上常显出可笑的表情如做出怪相、微笑和皱眉有时出现吸吮动作或咀嚼状态。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "手臂" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "腿" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "身体" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "脸" }, { "start_idx": 0, "end_idx": 37, "type": "sym", "entity": "手臂、腿和整个身体偶然有些活动,且脸上常显出可笑的表情如做出怪相、微笑和皱眉" }, { "start_idx": 38, "end_idx": 50, "type": "sym", "entity": "有时出现吸吮动作或咀嚼状态" } ] }, { "text": "新生儿除了前述的视觉和听觉定向各能力外,能在帮助下竖起头,还有反射性的运动能力,如身体站立,在牵拉新生儿时,新生儿会像拉单杠似的腾空而起。", "entities": [ { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "头" } ] }, { "text": "在给新生儿做视知觉或行为检查中,如果新生儿处于良好的安静觉醒状态下,当你发出轻柔语声时,新生儿会对你的说话感兴趣,向你凝视,并作出友好的应答,面露笑容,嘴唇嗫嚅犹如与你说话一样,表现十分轻松、惬意颈屈肌和颈伸肌的主动收缩,在帮助下可使头竖立。", "entities": [ { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "安静觉醒状态" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "嘴唇" }, { "start_idx": 44, "end_idx": 97, "type": "sym", "entity": "新生儿会对你的说话感兴趣,向你凝视,并作出友好的应答,面露笑容,嘴唇嗫嚅犹如与你说话一样,表现十分轻松、惬意" }, { "start_idx": 98, "end_idx": 100, "type": "bod", "entity": "颈屈肌" }, { "start_idx": 102, "end_idx": 104, "type": "bod", "entity": "颈伸肌" }, { "start_idx": 117, "end_idx": 117, "type": "bod", "entity": "头" } ] }, { "text": "例如,我们用双手在新生儿乳间连线水平固定其身体,自仰卧位慢慢将新生儿扶起,在刚扶起时新生儿头向后垂,当将躯体与床位成垂直时因颈屈肌的主动收缩头会竖立。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "双手" } ] }, { "text": "主动肌张力的另一个运动是牵拉反射,即当你取得新生儿的握持反射后,在新生儿紧紧握住你的手指时的刹那立即将双手举起,新生儿靠上肢肌肉的主动收缩会使自己的躯体腾空而起。", "entities": [ { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "手指" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "双手" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "上肢肌肉" } ] }, { "text": "在这阶段中,儿童接受各种感觉刺激,并作出反射性应答,如吸吮、抓握等,在逐渐修正反射的过程中进入第二阶段。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "吸吮" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "抓握" } ] }, { "text": "三、主动脉瓣上狭窄在主动脉窦(乏氏窦)上缘发生的局限性或弥漫性狭窄,少见。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "主动脉瓣上狭窄" }, { "start_idx": 10, "end_idx": 20, "type": "bod", "entity": "主动脉窦(乏氏窦)上缘" } ] }, { "text": "冠状动脉开口在狭窄之前,所以压力很高,常扩张扭曲,易致动脉硬化,周围动脉和肺动脉也可有狭窄。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "动脉硬化" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "动脉" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肺动脉" } ] }, { "text": "这种疾病可单独发生,也可伴有特发性婴儿高钙血症。", "entities": [ { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "特发性婴儿高钙血症" } ] }, { "text": "磁共振成像及超声心动图可显示主动脉瓣上狭窄,多普勒可评估跨狭窄段压差。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "磁共振成像" }, { "start_idx": 6, "end_idx": 10, "type": "pro", "entity": "超声心动图" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "主动脉瓣上狭窄" }, { "start_idx": 22, "end_idx": 24, "type": "pro", "entity": "多普勒" } ] }, { "text": "不提倡经导管球囊扩张。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "经导管球囊扩张" } ] }, { "text": "目前,儿童的疾病谱正在发生变化,既往影响儿童健康最严重的感染性疾病和营养性疾病已经明显下降,而先天性畸形、恶性肿瘤、意外损伤、慢性疾病、心理行为性疾病和环境因素有关的疾病成为儿童健康新的威胁,多数疾病不单纯是生物因素的作用,还受心理和社会诸因素的制约,有许多疾病的生物因素也要通过心理与社会因素起作用。", "entities": [ { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "营养性疾病" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "先天性畸形" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 58, "end_idx": 61, "type": "dis", "entity": "意外损伤" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "慢性疾病" }, { "start_idx": 68, "end_idx": 74, "type": "dis", "entity": "心理行为性疾病" } ] }, { "text": "参考文献1.洪流,于皆平,沈志祥,等.小儿大肠息肉特点.内镜.1994,11(5):2902.李铁一.儿科X线诊断学.天津:天津科学技术出版社,1990:1253.林庚金,吴云林主编.内镜临床应用进展.上海:上海科学技术文献出版社,1991:684.尚克中,陈九如.胃肠道造影原理与诊断.上海:上海科学技术文献出版社,1995.3575.童尔昌,季海萍.小儿腹部外科学.北京:人民卫生出版社,19916.吴恩惠.影像诊断学.第3版.北京:人民卫生出版社,1995:1407.许春娣,徐家裕.儿童食管胃静脉曲张的内镜诊断与治疗.见吴云林主编.食管和胃静脉曲张出血的现代治疗.上海:上海科学技术文献出版社,1996:978.许春娣,肖丽萍,张太锦,等.小儿慢性胃炎的临床表现与内镜特点.临床儿科杂志,1994,12(6):4169.许春娣,吴云林,梅红,等.上消化道出血急诊胃镜检查与止血初步探讨.中华儿科杂志,1995,33(1):2210.于中麟.反流性食道炎内镜诊断及协作治疗方案(试行).内镜,1993,10:4811.BeatlieRM,WaikerJA,MurchSH.Indicationforinvestigationofchronicgastrointestinalsymptoms.ArchDisChild,1995,73(4):35412.ChundiXU,JiayuXU,etal.EndoscopyManagementofgastrointestinalpolypsinchildren.JGastroenterolHepatol,1995,10:28713.GoenkaAS,DAsilvaMS,Cleghon,etal.Thereputicuppergastrointestinalendoscopyinchildren:Auauditof443procedureandliteraturereview.JGastroenterolHepatol,1993,8:44", "entities": [ { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "小儿大肠息肉" }, { "start_idx": 28, "end_idx": 29, "type": "equ", "entity": "内镜" }, { "start_idx": 51, "end_idx": 56, "type": "pro", "entity": "儿科X线诊断" }, { "start_idx": 92, "end_idx": 93, "type": "equ", "entity": "内镜" }, { "start_idx": 133, "end_idx": 137, "type": "pro", "entity": "胃肠道造影" }, { "start_idx": 179, "end_idx": 180, "type": "bod", "entity": "腹部" }, { "start_idx": 206, "end_idx": 209, "type": "equ", "entity": "影像诊断" }, { "start_idx": 245, "end_idx": 253, "type": "dis", "entity": "儿童食管胃静脉曲张" }, { "start_idx": 255, "end_idx": 258, "type": "pro", "entity": "内镜诊断" }, { "start_idx": 270, "end_idx": 279, "type": "dis", "entity": "食管和胃静脉曲张出血" }, { "start_idx": 324, "end_idx": 329, "type": "dis", "entity": "小儿慢性胃炎" }, { "start_idx": 336, "end_idx": 337, "type": "bod", "entity": "内镜" }, { "start_idx": 343, "end_idx": 344, "type": "dep", "entity": "儿科" }, { "start_idx": 377, "end_idx": 388, "type": "pro", "entity": "上消化道出血急诊胃镜检查" }, { "start_idx": 424, "end_idx": 433, "type": "pro", "entity": "反流性食道炎内镜诊断" }, { "start_idx": 446, "end_idx": 447, "type": "equ", "entity": "内镜" } ] }, { "text": "四、呼吸性碱中毒呼吸性碱中毒(respiratoryalkalosis)是由于肺泡通气过度增加致血二氧化碳分压降低。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "呼吸性碱中毒" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肺泡" }, { "start_idx": 41, "end_idx": 56, "type": "sym", "entity": "通气过度增加致血二氧化碳分压降低" } ] }, { "text": "其原发病因可为心理因素所致的呼吸过度、机械通气时每分通气量太大,也可见于水杨酸中毒所致的呼吸中枢过度刺激、对CO2的敏感性太高所致的呼吸增加。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "呼吸过度" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "机械通气" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "水杨酸中毒" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "呼吸中枢" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "呼吸增加" } ] }, { "text": "低氧、贫血、CO中毒时呼吸加快,也可使PaCO2降低出现碱中毒。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "低氧" }, { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "贫血" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "CO中毒" }, { "start_idx": 19, "end_idx": 23, "type": "ite", "entity": "PaCO2" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "碱中毒" }, { "start_idx": 11, "end_idx": 36, "type": "sym", "entity": "呼吸加快,也可使PaCO2降低出现碱中毒" } ] }, { "text": "急性低碳酸血症可使神经肌肉兴奋性增加低血钙所致的肢体感觉异常。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "急性低碳酸血症" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "神经肌肉" }, { "start_idx": 8, "end_idx": 17, "type": "sym", "entity": "使神经肌肉兴奋性增加" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "低血钙" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "肢体感觉异常" } ] }, { "text": "血气分析见pH增加、PaCO2降低、血浓度降低、尿液常呈酸性。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "血气分析" }, { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "pH" }, { "start_idx": 10, "end_idx": 14, "type": "ite", "entity": "PaCO2" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "血浓度" }, { "start_idx": 5, "end_idx": 35, "type": "sym", "entity": "pH增加、PaCO2降低、血浓度降低、尿液常呈酸性" } ] }, { "text": "四、副流感病毒肺炎副流感病毒肺炎(parainfluenzapneumonia)易感对象为3个月至1岁的婴儿。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "副流感病毒肺炎" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "副流感病毒肺炎" }, { "start_idx": 17, "end_idx": 38, "type": "dis", "entity": "parainfluenzapneumonia" } ] }, { "text": "其发病率仅次于RSV。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "RSV" } ] }, { "text": "多有3~5日的中等程度发热或高热及呼吸困难、哮吼样咳嗽、三凹征、肺部干湿啰音等,但多数患儿表现较轻,一般无中毒症状,病程较短。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "中等程度发热" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "高热" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "哮吼样咳嗽" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "三凹征" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "肺部干湿啰音" } ] }, { "text": "X线检查肺野可有小片状阴影。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "X线检查" } ] }, { "text": "临床上无法与其他病毒性肺炎相区别,根据血清学和病毒学检查结果确定诊断。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "病毒性肺炎" }, { "start_idx": 19, "end_idx": 27, "type": "ite", "entity": "血清学和病毒学检查" } ] }, { "text": "七、先天性膈疝先天性膈疝(congenitaldiaphragmatichernia)是由于胚胎期膈肌发育缺陷,出现较大的裂隙或缺损,致生后一部分腹腔脏器疝入胸腔,压迫胸腔内组织,引起呼吸、循环障碍,或胃肠道梗阻、绞窄、出血甚至坏死。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "先天性膈疝" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "先天性膈疝" }, { "start_idx": 13, "end_idx": 41, "type": "dis", "entity": "congenitaldiaphragmatichernia" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "膈肌" }, { "start_idx": 73, "end_idx": 76, "type": "bod", "entity": "腹腔脏器" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "胸腔" }, { "start_idx": 84, "end_idx": 88, "type": "bod", "entity": "胸腔内组织" }, { "start_idx": 92, "end_idx": 98, "type": "sym", "entity": "呼吸、循环障碍" }, { "start_idx": 101, "end_idx": 115, "type": "sym", "entity": "胃肠道梗阻、绞窄、出血甚至坏死" } ] }, { "text": "按发生部位及临床特点分为三型:①胸腹裂孔疝(即Bochdalek疝),约占90%,症状出现早,多于婴儿期就诊;②胸骨后疝(又称Morgagni疝),较少见,约占3%;③食管裂孔疝,多见于中老年人,儿童偶见。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "胸腹裂孔疝" }, { "start_idx": 23, "end_idx": 32, "type": "dis", "entity": "Bochdalek疝" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "胸骨后疝" }, { "start_idx": 63, "end_idx": 71, "type": "dis", "entity": "Morgagni疝" }, { "start_idx": 84, "end_idx": 88, "type": "dis", "entity": "食管裂孔疝" } ] }, { "text": "临床症状的严重程度和出现时间与下列因素有关:①进入胸腔的腹部脏器的种类和容量;②是否有肺发育不全和肺动脉高压;③是否有肠道梗阻或其他先天性畸形。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "胸腔" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "腹部脏器" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "肺发育不全" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "肠道梗阻" }, { "start_idx": 64, "end_idx": 70, "type": "dis", "entity": "其他先天性畸形" } ] }, { "text": "多数患儿在生后24小时内出现呼吸困难,喂奶及哭闹时加重,患侧卧位或半坐位时则稍减轻;反复发生肺炎、呕吐及营养不良;体格检查时可发现患侧胸壁呼吸运动减弱,心界向对侧移位,患侧叩诊呈鼓音,肺呼吸音减低或消失,肺部可闻肠鸣音,并呈舟状腹。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "喂奶" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "哭闹时加重" }, { "start_idx": 28, "end_idx": 40, "type": "sym", "entity": "患侧卧位或半坐位时则稍减轻" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "肺炎" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "呕吐" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "营养不良" }, { "start_idx": 57, "end_idx": 60, "type": "ite", "entity": "体格检查" }, { "start_idx": 65, "end_idx": 74, "type": "sym", "entity": "患侧胸壁呼吸运动减弱" }, { "start_idx": 76, "end_idx": 82, "type": "sym", "entity": "心界向对侧移位" }, { "start_idx": 84, "end_idx": 90, "type": "sym", "entity": "患侧叩诊呈鼓音" }, { "start_idx": 92, "end_idx": 100, "type": "sym", "entity": "肺呼吸音减低或消失" }, { "start_idx": 102, "end_idx": 108, "type": "sym", "entity": "肺部可闻肠鸣音" }, { "start_idx": 110, "end_idx": 114, "type": "sym", "entity": "并呈舟状腹" } ] }, { "text": "产前超声波检查多数可做出诊断。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "ite", "entity": "超声波检查" } ] }, { "text": "胸片可发现有疝入侧的胸部出现胃或肠管阴影、或透亮的团块状阴影、纵隔和心脏向对侧移位等。", "entities": [ { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "透亮的团块状阴影" } ] }, { "text": "对于不易确诊的病例,须做钡餐检查以明确其类型和位置,然后决定手术方法。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "钡餐检查" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "手术" } ] }, { "text": "本病均需手术治疗,一旦确诊应尽早手术。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "手术治疗" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "手术" } ] }, { "text": "第六章急性肾衰竭见第十二篇第十一章。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "二、护理要点(一)保温和保湿VLBW出生后,应在远红外暖床上立即擦拭干净其体表的水分,送入暖箱,以防止低体温的出现。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "VLBW" }, { "start_idx": 25, "end_idx": 29, "type": "equ", "entity": "远红外暖床" }, { "start_idx": 46, "end_idx": 47, "type": "equ", "entity": "暖箱" } ] }, { "text": "在保温的同时应注意保湿,因为其不显性失水相对较多,容易出现脱水。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "脱水" } ] }, { "text": "用塑料布或薄膜覆盖VLBW的全身,可以明显减少不显性失水。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "VLBW" } ] }, { "text": "通过这些方法,可以将不显性失水的量减少30%~60%,从而减少输液量,这对VLBW而言是有重要意义的。", "entities": [ { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "VLBW" } ] }, { "text": "控制液体的供给,是预防动脉导管开放的重要措施之一,并可以减轻肾脏的负担。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "控制液体的供给" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "动脉导管开放" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肾脏" } ] }, { "text": "但是,也有人提出暖箱内如此高的湿度是否会增加感染的机会,尤其是通过水分传播的铜绿假单胞菌感染。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "equ", "entity": "暖箱" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "感染" }, { "start_idx": 38, "end_idx": 45, "type": "dis", "entity": "铜绿假单胞菌感染" } ] }, { "text": "高湿度可造成皮肤感染,这种感染对于VLBW而言是致命的。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "皮肤感染" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "感染" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "VLBW" } ] }, { "text": "其实,只要加强医疗行为中预防感染的措施,并将水汽雾化使用的时间控制在生后3~7天内,应当是安全的。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "感染" } ] }, { "text": "(二)呼吸管理首先应防止胎龄小于32周的早产儿发生因肺表面活性物质的缺乏而出现呼吸窘迫综合征,及时补充肺表面活性物质是有效的措施。", "entities": [ { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 26, "end_idx": 35, "type": "sym", "entity": "肺表面活性物质的缺乏" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "呼吸窘迫综合征" }, { "start_idx": 47, "end_idx": 57, "type": "pro", "entity": "及时补充肺表面活性物质" } ] }, { "text": "如果药物治疗效果不佳,并出现严重低氧血症的,可以使用呼吸机进行治疗。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "低氧血症" }, { "start_idx": 26, "end_idx": 28, "type": "equ", "entity": "呼吸机" } ] }, { "text": "(三)喂养和营养VLBW的喂养不应过迟,尽早喂养可以减轻生理性体重下降的程度,防止低血糖的发生,降低核黄疸的危险性。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "VLBW" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "体重" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "低血糖" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "核黄疸" } ] }, { "text": "喂养应以母乳为最佳,可以减少坏死性小肠结肠炎的发生率,并给予母乳强化剂。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "母乳" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "坏死性小肠结肠炎" }, { "start_idx": 30, "end_idx": 34, "type": "dru", "entity": "母乳强化剂" } ] }, { "text": "如果没有母乳,可以选用适合早产儿使用的配方奶进行喂养,在用配方奶之前可以先试用糖水,且糖水的浓度不可超过5%,高渗性糖水对VLBW的胃肠道不利,容易引起坏死性小肠结肠炎。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "母乳" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "VLBW" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "胃肠道" }, { "start_idx": 76, "end_idx": 83, "type": "dis", "entity": "坏死性小肠结肠炎" } ] }, { "text": "喂养的方式尽可能选用让小儿自己吸吮的方式,若有困难,可以使用鼻胃管或鼻肠管的方式。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "equ", "entity": "鼻胃管" }, { "start_idx": 34, "end_idx": 36, "type": "equ", "entity": "鼻肠管" } ] }, { "text": "需要注意的是,在使用鼻胃管或鼻肠管喂养时,应注意给奶的速度,切忌快速注入,应根据小儿的体重给予喂养的量,开始剂量为2~5ml左右。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "equ", "entity": "鼻胃管" }, { "start_idx": 14, "end_idx": 16, "type": "equ", "entity": "鼻肠管" }, { "start_idx": 43, "end_idx": 44, "type": "ite", "entity": "体重" } ] }, { "text": "事实上,大部分VLBW需经2~3周的全肠外营养(TPN)或部分肠外营养才能防止严重EUGR的发生。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "VLBW" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "全肠外营养" }, { "start_idx": 24, "end_idx": 26, "type": "pro", "entity": "TPN" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "部分肠外营养" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "EUGR" } ] }, { "text": "在给予TPN时应注意营养素的全面和均衡。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "TPN" } ] }, { "text": "(四)预防感染对VLBW的操作过程应严格执行消毒隔离制度,注意其环境和用具的清洁。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "VLBW" } ] }, { "text": "对于使用气管插管、鼻胃管或鼻肠管的小儿,应至少每周换管1次,并对换下的管道进行细菌学检测,以指导抗生素的应用。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "equ", "entity": "气管" }, { "start_idx": 6, "end_idx": 7, "type": "equ", "entity": "插管" }, { "start_idx": 9, "end_idx": 11, "type": "equ", "entity": "鼻胃管" }, { "start_idx": 13, "end_idx": 15, "type": "equ", "entity": "鼻肠管" }, { "start_idx": 35, "end_idx": 36, "type": "equ", "entity": "管道" }, { "start_idx": 39, "end_idx": 43, "type": "pro", "entity": "细菌学检测" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "抗生素" } ] }, { "text": "对VLBW伴有低丙种球蛋白血症的,可以给予静脉输注丙种球蛋白,以提高对感染的抵抗力。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "VLBW" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "低丙种球蛋白血症" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "静脉输注" }, { "start_idx": 25, "end_idx": 29, "type": "dru", "entity": "丙种球蛋白" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "感染" } ] }, { "text": "(五)动脉导管开放(PDA)的处理对存在PDA的VLBW,可以首先控制每天液体的输注量,以降低心脏负荷。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "动脉导管开放" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "PDA" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "PDA" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "VLBW" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "心脏" } ] }, { "text": "其次,可以使用吲哚美辛治疗。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "吲哚美辛" } ] }, { "text": "在使用吲哚美辛后,应注意肾功能的变化和有无坏死性小肠结肠炎的发生。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "肾功能" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "坏死性小肠结肠炎" } ] }, { "text": "在使用吲哚美辛关闭动脉导管后24~72小时,可有部分小儿的动脉导管重新开放。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "动脉导管" }, { "start_idx": 29, "end_idx": 36, "type": "dis", "entity": "动脉导管重新开放" } ] }, { "text": "(六)预防核黄疸对于VLBW,其黄疸的换血胆红素指标应在10~15mg/dl,要及时纠正低蛋白血症,并尽早开始光疗。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "核黄疸" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "VLBW" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "黄疸" }, { "start_idx": 19, "end_idx": 23, "type": "ite", "entity": "换血胆红素" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "低蛋白血症" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "光疗" } ] }, { "text": "(七)对于脑室内出血的预防和治疗要防止反复低氧的发生、血压的波动和高渗性补液的输注(VLBW应用的葡萄糖溶液浓度应低于8%),同时应用头颅B超或CT对小儿颅内情况进行连续监测,部分资料显示的应用苯巴比妥或吲哚美辛(消炎痛)及布洛芬都有部分预防脑室内出血的结果,但尚缺乏大样本多中心临床试验结果的支持。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "脑室内出血" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "反复低氧" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "血压" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "高渗性补液" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "VLBW" }, { "start_idx": 49, "end_idx": 51, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 67, "end_idx": 73, "type": "pro", "entity": "头颅B超或CT" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "颅内" }, { "start_idx": 97, "end_idx": 100, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 102, "end_idx": 105, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 107, "end_idx": 109, "type": "dru", "entity": "消炎痛" }, { "start_idx": 112, "end_idx": 114, "type": "dru", "entity": "布洛芬" }, { "start_idx": 121, "end_idx": 125, "type": "dis", "entity": "脑室内出血" } ] }, { "text": "最近的资料显示,目前临床上部分应用的连续腰穿疗法,对治疗由脑室内出血所造成的脑积水其疗效难以肯定,需要进一步的论证。", "entities": [ { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "连续腰穿疗法" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "脑室内出血" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "脑积水" } ] }, { "text": "第三节细菌性痢疾细菌性痢疾(bacillarydysentery)简称菌痢,系由细菌引起的常见肠道传染病,由四种志贺菌属引起,它们是志贺菌(A群)、福氏菌(B群)、鲍氏菌(C群)、宋内(D群)痢疾杆菌。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "细菌性痢疾" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "细菌性痢疾" }, { "start_idx": 14, "end_idx": 31, "type": "dis", "entity": "bacillarydysentery" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "菌痢" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "细菌" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "肠道传染病" }, { "start_idx": 56, "end_idx": 58, "type": "mic", "entity": "志贺菌" }, { "start_idx": 66, "end_idx": 72, "type": "mic", "entity": "志贺菌(A群)" }, { "start_idx": 74, "end_idx": 80, "type": "mic", "entity": "福氏菌(B群)" }, { "start_idx": 82, "end_idx": 88, "type": "mic", "entity": "鲍氏菌(C群)" }, { "start_idx": 90, "end_idx": 99, "type": "mic", "entity": "宋内(D群)痢疾杆菌" } ] }, { "text": "【临床流行病学】痢疾的发病有明显的季节性,多为夏季或热带地区的雨季,以婴幼儿的发病率最高,多见于2~3岁,小于6个月的婴儿很少发病,这可能是母乳喂养时婴儿从母乳中获得带有抗毒性质粒编码的抗脂多糖抗体。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "痢疾" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "母乳" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "母乳" } ] }, { "text": "在发达国家,宋内痢疾杆菌引起的感染较常见,福氏菌占第二位;在发展中国家如我国,福氏菌感染占第一位,宋内菌占第二位,志贺菌1型是引起大规模流行的菌型,在亚洲的某些地区,它也是主要的流行菌型。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "mic", "entity": "宋内痢疾杆菌" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "感染" }, { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "福氏菌" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "福氏菌感染" }, { "start_idx": 49, "end_idx": 51, "type": "mic", "entity": "宋内菌" }, { "start_idx": 57, "end_idx": 61, "type": "mic", "entity": "志贺菌1型" } ] }, { "text": "污染的蔬菜、瓜果或粪便管理不当,水源污染是引起痢疾流行或暴发的常见原因,生活的接触也是感染的主要方式。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "粪便" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "痢疾" } ] }, { "text": "人类对痢疾有普遍的易感性,感染后的免疫反应不一,亦不长久,所以可多次反复感染。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "痢疾" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "感染" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "感染" } ] }, { "text": "有报道分泌性IgA和血清抗体在感染的数天或数周内产生,目前已知有抗脂多糖抗体,抗毒力质粒编码的多肽抗体,但它们抗感染的主要保护性决定簇尚不清楚。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "分泌性IgA" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "血清" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "抗脂多糖抗体" } ] }, { "text": "有资料表明这种抗感染作用是血清型特异性的,但也有不同程度的交叉,细胞免疫也可能有一些保护作用,作用不大。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "血清" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "细胞" } ] }, { "text": "【发病机制】所有痢疾杆菌的基本毒力因素是它们能够侵入结肠上皮细胞,这是由一个120~140MDa的大质粒编码的一组多肽造成的侵袭性和杀伤性作用,丢失此质粒的菌株则不再致病。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "mic", "entity": "痢疾杆菌" }, { "start_idx": 26, "end_idx": 31, "type": "bod", "entity": "结肠上皮细胞" } ] }, { "text": "此外,染色体编码的因子也具有毒力作用,如编码脂多糖合成的染色体,由1型志贺菌合成的志贺毒素,由福氏菌2a型合成的志贺肠毒素1。", "entities": [ { "start_idx": 33, "end_idx": 37, "type": "mic", "entity": "1型志贺菌" }, { "start_idx": 41, "end_idx": 44, "type": "mic", "entity": "志贺毒素" }, { "start_idx": 47, "end_idx": 52, "type": "mic", "entity": "福氏菌2a型" }, { "start_idx": 56, "end_idx": 61, "type": "mic", "entity": "志贺肠毒素1" } ] }, { "text": "1型志贺菌可产生大量志贺毒素。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "1型志贺菌" }, { "start_idx": 10, "end_idx": 13, "type": "mic", "entity": "志贺毒素" } ] }, { "text": "而在痢疾感染的水泻阶段,很可能主要是志贺肠毒素1所致,少量的痢疾杆菌即可致病。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "痢疾感染" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "水泻" }, { "start_idx": 18, "end_idx": 23, "type": "mic", "entity": "志贺肠毒素1" }, { "start_idx": 30, "end_idx": 33, "type": "mic", "entity": "痢疾杆菌" } ] }, { "text": "临床可见下列类型:1.急性菌痢起病急,典型症状为严重的腹痛、高热、呕吐、食欲缺乏、全身中毒症状重,体征有腹胀、压痛、肠鸣音亢进。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "急性菌痢" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "腹痛" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "高热" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "呕吐" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "全身中毒症状重" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "腹胀" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "压痛" }, { "start_idx": 58, "end_idx": 62, "type": "sym", "entity": "肠鸣音亢进" } ] }, { "text": "指诊时直肠触痛,大便开始为稀水样,继而见黏冻样、脓冻样或脓血便,便次多,量小,便数不等,年长儿有里急后重,婴幼儿可无脓便及脓血便,易误诊为其他细菌引起的肠炎或病毒性肠炎,病程5~7天。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "指诊" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "直肠" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "指诊时直肠触痛" }, { "start_idx": 8, "end_idx": 30, "type": "sym", "entity": "大便开始为稀水样,继而见黏冻样、脓冻样或脓血便" }, { "start_idx": 32, "end_idx": 42, "type": "sym", "entity": "便次多,量小,便数不等" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "里急后重" }, { "start_idx": 53, "end_idx": 63, "type": "sym", "entity": "婴幼儿可无脓便及脓血便" }, { "start_idx": 71, "end_idx": 72, "type": "mic", "entity": "细菌" }, { "start_idx": 76, "end_idx": 77, "type": "dis", "entity": "肠炎" }, { "start_idx": 79, "end_idx": 83, "type": "dis", "entity": "病毒性肠炎" } ] }, { "text": "非典型痢疾不发热或只有微热,也无中毒症状,只有粪便培养阳性才能确诊,以被忽视,常为痢疾的传播者。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "非典型痢疾" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "发热" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "微热" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "中毒症状" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "粪便" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "粪便培养阳性" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "痢疾" } ] }, { "text": "2.中毒性菌痢本病多见于2~7岁小儿,起病急骤,高热甚至超高热,在24小时内出现惊厥、昏迷为主的脑型或以循环衰竭为主的休克型或两者俱存的混合型,有或无脓血便,甚至不出现腹泻。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "中毒性菌痢" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "高热" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "超高热" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "惊厥" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "昏迷" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "循环衰竭" }, { "start_idx": 75, "end_idx": 77, "type": "sym", "entity": "脓血便" }, { "start_idx": 84, "end_idx": 85, "type": "sym", "entity": "腹泻" } ] }, { "text": "(1)脑型:以惊厥、头痛、反复呕吐、昏迷、血压增高为主,引起这种颅内高压的原因尚不清楚。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "惊厥" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "头痛" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "反复呕吐" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "昏迷" }, { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "血压" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "血压增高" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "颅内" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "颅内高压" } ] }, { "text": "严重者可发生脑水肿、脑疝而发生呼吸衰竭、反复惊厥。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑水肿" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "脑疝" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "呼吸衰竭" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "反复惊厥" } ] }, { "text": "(2)休克型:以循环衰竭为主,常发生在年幼儿、体弱儿,也是毒血症和弥漫血管内凝血过程,表现为面色灰、肢端发凉、皮肤有花纹、血压下降、脉细、心率快。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "循环衰竭" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "毒血症" }, { "start_idx": 46, "end_idx": 48, "type": "sym", "entity": "面色灰" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "肢端发凉" }, { "start_idx": 55, "end_idx": 59, "type": "sym", "entity": "皮肤有花纹" }, { "start_idx": 61, "end_idx": 62, "type": "ite", "entity": "血压" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "血压下降" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "脉细" }, { "start_idx": 69, "end_idx": 70, "type": "ite", "entity": "心率" }, { "start_idx": 69, "end_idx": 71, "type": "sym", "entity": "心率快" } ] }, { "text": "病程中尚可出现多脏器衰竭、休克肺、休克心、休克肾、休克脑、休克肝等。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "多脏器衰竭" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "休克肺" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "休克心" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "休克肾" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "休克脑" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "休克肝" } ] }, { "text": "3.慢性菌痢病程超过2个月以上者诊断为慢性菌痢。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "慢性菌痢" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "慢性菌痢" } ] }, { "text": "当治疗不彻底、不规则时,耐药菌株存在或机体免疫力减低时出现,临床无发热等毒血症表现,粪便性质也不典型,可为消化不良稀便,甚至软便或有黏液,间有少量脓冻、脓血便,次数多少不定。", "entities": [ { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "毒血症" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "粪便" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "消化不良稀便" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "软便" }, { "start_idx": 65, "end_idx": 67, "type": "sym", "entity": "有黏液" }, { "start_idx": 73, "end_idx": 74, "type": "sym", "entity": "脓冻" }, { "start_idx": 76, "end_idx": 78, "type": "sym", "entity": "脓血便" } ] }, { "text": "【并发症】脱水是最常见的,它可引起肾衰竭和死亡。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "脱水" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "当发生休克、毒血症时死亡率可高达20%~50%。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "休克" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "毒血症" } ] }, { "text": "此外,在志贺菌感染时,溶血性尿毒症综合征是较常见的并发症。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "志贺菌感染" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "溶血性尿毒症综合征" } ] }, { "text": "另外,脱肛、中毒性巨结肠、肠穿孔、伪膜性结肠炎、严重营养不良所致结膜炎、虹膜炎、角膜溃疡等为不常见的并发症。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "脱肛" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "中毒性巨结肠" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肠穿孔" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "伪膜性结肠炎" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "严重营养不良" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "结膜炎" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "虹膜炎" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "角膜溃疡" } ] }, { "text": "【诊断】有典型的脓血便,结合临床表现和流行季节,诊断并不困难。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "脓血便" } ] }, { "text": "实验室检查粪便镜检有大量白细胞、脓细胞或红、白细胞。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "粪便镜检" }, { "start_idx": 10, "end_idx": 24, "type": "sym", "entity": "大量白细胞、脓细胞或红、白细胞" } ] }, { "text": "血常规白细胞增加明显,并有核左移,都支持诊断。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "血常规" }, { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "白细胞增加明显" } ] }, { "text": "大便和直肠拭子细菌培养是最好的诊断方法。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "大便" }, { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "直肠拭子" } ] }, { "text": "最近已开展PCR快速诊断法。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "PCR快速诊断法" } ] }, { "text": "【鉴别诊断】具有脓血便,在儿科应与鼠伤寒杆菌肠炎、金黄色葡萄球菌肠炎、真菌性肠炎和出血坏死性小肠炎鉴别。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "脓血便" }, { "start_idx": 13, "end_idx": 14, "type": "dep", "entity": "儿科" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "鼠伤寒杆菌肠炎" }, { "start_idx": 25, "end_idx": 33, "type": "dis", "entity": "金黄色葡萄球菌肠炎" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "真菌性肠炎" }, { "start_idx": 41, "end_idx": 48, "type": "dis", "entity": "出血坏死性小肠炎" } ] }, { "text": "无典型脓血便者,特别是婴幼儿菌痢需与致病性大肠埃希菌肠炎、病毒性肠炎、空肠弯曲菌肠炎鉴别。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "sym", "entity": "脓血便" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "婴幼儿菌痢" }, { "start_idx": 18, "end_idx": 27, "type": "dis", "entity": "致病性大肠埃希菌肠炎" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "病毒性肠炎" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "空肠弯曲菌肠炎" } ] }, { "text": "中毒性菌痢应与暴发型流脑、乙型脑炎、大叶性肺炎及其他病原菌引起的感染性休克鉴别。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "中毒性菌痢" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "暴发型流脑" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "乙型脑炎" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "大叶性肺炎" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "感染性休克" } ] }, { "text": "慢性菌痢应与慢性非特异性溃疡性结肠炎、慢性血吸虫病相鉴别。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "慢性菌痢" }, { "start_idx": 6, "end_idx": 17, "type": "dis", "entity": "慢性非特异性溃疡性结肠炎" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "慢性血吸虫病" } ] }, { "text": "2.控制感染抗生素治疗目前对抗生素耐药的痢疾杆菌株的地理分布是不同的。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "抗生素" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "抗生素" }, { "start_idx": 20, "end_idx": 23, "type": "mic", "entity": "痢疾杆菌" } ] }, { "text": "对敏感株,氨苄西林口服100mg/(kg•d),每天分四次口服即可。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "氨苄西林" } ] }, { "text": "阿莫西林效果不如氨苄西林,但胃肠道吸收好。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "阿莫西林" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "胃肠道" } ] }, { "text": "由于复方磺胺甲唑在我国耐药率较高,它不用做首选用药头孢克肟(cefixime)8mg/(kg•d),口服分两次,共服5天,或口服其他三代头孢菌头孢曲松(cetriaxone)50mg/(kg•d),每天一次,肌内注射或静脉注射2~5天,可作为首选。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "复方磺胺甲唑" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "头孢克肟" }, { "start_idx": 30, "end_idx": 37, "type": "dru", "entity": "cefixime" }, { "start_idx": 68, "end_idx": 74, "type": "dru", "entity": "头孢菌头孢曲松" }, { "start_idx": 76, "end_idx": 85, "type": "dru", "entity": "cetriaxone" }, { "start_idx": 104, "end_idx": 107, "type": "pro", "entity": "肌内注射" }, { "start_idx": 109, "end_idx": 112, "type": "pro", "entity": "静脉注射" } ] }, { "text": "萘啶酸(nalidixic)55mg/(kg•d)分四次给予,共用5天,是另一种替代方法,疗程一般5天。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "萘啶酸" }, { "start_idx": 4, "end_idx": 12, "type": "dru", "entity": "nalidixic" } ] }, { "text": "口服一、二代头孢菌素不能作为二线替代药。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "头孢菌素" } ] }, { "text": "吡哌酸因对小儿骨骼发育有影响,18岁以下慎用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "吡哌酸" } ] }, { "text": "3.中毒性菌痢治疗根据临床不同表现对症治疗。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "中毒性菌痢" } ] }, { "text": "4.慢性菌痢采用支持疗法和抗病原治疗相结合,应2种以上抗生素联合用药和药物保留灌肠。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "慢性菌痢" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "抗生素" } ] }, { "text": "【预防】1.痢疾高发地区应鼓励延长母乳喂养。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "痢疾" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "延长母乳喂养" } ] }, { "text": "2.指导幼儿园、学校的儿童及工作人员及时进行清洁和消毒工作。", "entities": [ { "start_idx": 18, "end_idx": 28, "type": "pro", "entity": "及时进行清洁和消毒工作" } ] }, { "text": "3.作好疫情报告,出现疫情后,立即找出并控制传染源,禁止患者或带菌者从事餐饮业和保育工作。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "作好疫情报告" } ] }, { "text": "第五节肢体保留性治疗长期以来,恶性骨肿瘤的治疗中,截肢一直被推崇为外科治疗的主要手段。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "肢体保留性治疗" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "恶性骨肿瘤" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "截肢" }, { "start_idx": 33, "end_idx": 34, "type": "dep", "entity": "外科" } ] }, { "text": "近10多年来,一种既考虑到恶性骨肿瘤的有效治疗,同时又尽量兼顾保留患肢的治疗方法正在兴起并得到了很多学者的重视和采用。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "恶性骨肿瘤" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "患肢" } ] }, { "text": "这就是肢体保留性治疗肢体保留性治疗(limbsalvage),简称保肢治疗。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "肢体保留性治疗" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "肢体保留性治疗" }, { "start_idx": 18, "end_idx": 28, "type": "pro", "entity": "limbsalvage" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "保肢治疗" } ] }, { "text": "保留有功能而无肿瘤的肢体是保肢治疗的根本。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肢体" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "保肢治疗" } ] }, { "text": "因此,治疗前仔细的计划和肿瘤分期,明确局部病灶的切除范围,包括手术前后的化疗都对治疗的结果有着很重要的影响。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肿瘤" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "手术" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "化疗" } ] }, { "text": "保肢治疗不是适用于所有骨肿瘤病人的通用治疗方法,必须因人而异,根据不同的病人采用各自适用的保肢方法,具体对待不同的分期和类型的肿瘤。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "保肢治疗" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "骨肿瘤" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "保肢方法" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "肿瘤" } ] }, { "text": "施行保肢治疗,在现阶段条件下的确也还存在着许多潜在的危险和并发症,有时要达到一个稳定有功能的肢体,可能需要两次甚至多次的手术。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "保肢治疗" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "肢体" }, { "start_idx": 60, "end_idx": 61, "type": "pro", "entity": "手术" } ] }, { "text": "因此,截肢手术远没有走到应该放弃的地步。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "截肢手术" } ] }, { "text": "施行肢体保留性治疗的条件:①肿瘤处于发病的早期;②肿瘤边缘清楚,可以施行外科切除,并有把握保护好手术中对坐骨神经和其分支等重要结构的损伤;③手术前必须充分考虑好手术后切除部位的皮肤覆盖问题,避免术后无组织局部的皮肤坏死;④有经验的骨科医生。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "肢体保留性治疗" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肿瘤" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "肿瘤" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "外科切除" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "手术" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "坐骨神经" }, { "start_idx": 70, "end_idx": 71, "type": "pro", "entity": "手术" }, { "start_idx": 80, "end_idx": 81, "type": "pro", "entity": "手术" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "皮肤" }, { "start_idx": 105, "end_idx": 108, "type": "dis", "entity": "皮肤坏死" }, { "start_idx": 115, "end_idx": 116, "type": "dep", "entity": "骨科" } ] }, { "text": "保肢治疗的主要方法为:1.肿瘤局部切除手术中广泛切除肿瘤组织和覆盖肿瘤的软组织。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "保肢治疗" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "肿瘤局部切除" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "手术" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "肿瘤组织" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "软组织" } ] }, { "text": "采用髓内或髓外固定,并辅以自体植骨的方法,固定长骨中段的缺损,使切除两端的骨组织重新连接。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "髓内或髓外固定" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "自体植骨" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "长骨" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "骨组织" } ] }, { "text": "2.同种异体骨关节重建肿瘤切除后采用同种异体关节移植重建骨关节功能,但是供体来源的有限、植入体连接困难和骨折以及关节软骨的退化和塌陷大大限制了这种方法的广泛使用。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "同种异体骨关节重建" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "肿瘤切除" }, { "start_idx": 18, "end_idx": 25, "type": "pro", "entity": "同种异体关节移植" }, { "start_idx": 44, "end_idx": 48, "type": "pro", "entity": "植入体连接" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "骨折" }, { "start_idx": 58, "end_idx": 65, "type": "dis", "entity": "软骨的退化和塌陷" } ] }, { "text": "3.人工关节假体功能重建这是肢体保留治疗中采用最多的一种方法。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "pro", "entity": "人工关节假体功能重建" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "肢体保留" } ] }, { "text": "随着人工关节假体材料技术和植入技术的日渐成熟,这种新的治疗方法正在越来越多的显示出其在恶性骨关节肿瘤中的应用优势。", "entities": [ { "start_idx": 2, "end_idx": 16, "type": "pro", "entity": "人工关节假体材料技术和植入技术" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "恶性骨关节肿瘤" } ] }, { "text": "根据患者实际尺寸定制或采用通用型的人工关节,安装在肿瘤切除后的关节进行重建。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "肿瘤切除" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "关节" } ] }, { "text": "对于儿童病例,考虑到肢体的继续生长趋势,目前已有可生长性的假体问世,为这种新的治疗方法带来了希望。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肢体" } ] }, { "text": "第二节慢性肾衰竭慢性肾衰竭(chronicrenalfailure,CRF)是指各种原因造成的慢性进行性肾实质损害,呈进行性不可逆转的肾小球滤过率下降,导致氮质血症、代谢紊乱和各系统受累的临床综合征。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "慢性肾衰竭" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "慢性肾衰竭" }, { "start_idx": 14, "end_idx": 32, "type": "dis", "entity": "chronicrenalfailure" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "CRF" }, { "start_idx": 67, "end_idx": 72, "type": "ite", "entity": "肾小球滤过率" }, { "start_idx": 78, "end_idx": 81, "type": "dis", "entity": "氮质血症" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "代谢紊乱" } ] }, { "text": "当进展到需肾透析或移植方可维持生命时称为终末期肾病(endstagerenaldisease,ESRD)。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "肾透析" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "移植" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "终末期肾病" }, { "start_idx": 26, "end_idx": 45, "type": "dis", "entity": "endstagerenaldisease" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "ESRD" } ] }, { "text": "CRF小儿中的发生率国内尚无确切数据,国外报道为每百万人口中4~5人。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "CRF" } ] }, { "text": "【病因】慢性肾衰竭的病因以各种原发性及继发性肾小球肾炎占首位,其次为泌尿系统先天畸形(如肾发育不良,先天性多囊肾,膀胱输尿管反流等)及遗传性疾病(如遗传性肾炎,肾髓质囊性病,Fanconi综合征等)。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "慢性肾衰竭" }, { "start_idx": 15, "end_idx": 26, "type": "dis", "entity": "原发性及继发性肾小球肾炎" }, { "start_idx": 34, "end_idx": 41, "type": "dis", "entity": "泌尿系统先天畸形" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "肾发育不良" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "先天性多囊肾" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "遗传性肾炎" }, { "start_idx": 80, "end_idx": 85, "type": "dis", "entity": "肾髓质囊性病" }, { "start_idx": 87, "end_idx": 96, "type": "dis", "entity": "Fanconi综合征" } ] }, { "text": "全身性系统疾病中以肾小动脉硬化、高血压及结缔组织病等多见。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "肾小动脉硬化" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "高血压" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "结缔组织病" } ] }, { "text": "近年来肾间质小管损害引起的CRF也逐渐受到人们的重视,糖尿病肾病、自身免疫性与结缔组织疾病及肾损害引起的CRF也有上升趋势。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "肾间质小管损害" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "CRF" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "糖尿病肾病" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "结缔组织疾病" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "肾损害" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "CRF" } ] }, { "text": "Topel统计欧洲37个肾移植中心总结286例<15岁儿童肾移植病例其终末期肾病的分布:慢性肾小球肾炎52.3%,慢性肾盂肾炎20.8%,遗传性肾病8.0%,血管性肾病4.5%,多囊肾3.0%,药物性肾病2.4%,先天性肾发育不全1.6%,其他(包括胱氨酸沉积症、草酸盐沉积症、Alport综合征及溶血尿毒综合征)7.4%。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "肾移植" }, { "start_idx": 29, "end_idx": 31, "type": "pro", "entity": "肾移植" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "终末期肾病" }, { "start_idx": 44, "end_idx": 50, "type": "dis", "entity": "慢性肾小球肾炎" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "慢性肾盂肾炎" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "遗传性肾病" }, { "start_idx": 79, "end_idx": 83, "type": "dis", "entity": "血管性肾病" }, { "start_idx": 89, "end_idx": 91, "type": "dis", "entity": "多囊肾" }, { "start_idx": 97, "end_idx": 101, "type": "dis", "entity": "药物性肾病" }, { "start_idx": 107, "end_idx": 114, "type": "dis", "entity": "先天性肾发育不全" }, { "start_idx": 125, "end_idx": 130, "type": "dis", "entity": "胱氨酸沉积症" }, { "start_idx": 132, "end_idx": 137, "type": "dis", "entity": "草酸盐沉积症" }, { "start_idx": 139, "end_idx": 147, "type": "dis", "entity": "Alport综合征" }, { "start_idx": 149, "end_idx": 155, "type": "dis", "entity": "溶血尿毒综合征" } ] }, { "text": "然而,要注意到,反流性肾病是小儿终末期肾衰的重要原因之一,我院的资料表明,在小儿慢性肾功能不全的病因中,虽然获得性肾小球疾病仍占重要地位(占45.9%),但已与先天性和遗传性肾脏疾病平分秋色(占45.9%)。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "反流性肾病" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "小儿终末期肾衰" }, { "start_idx": 38, "end_idx": 46, "type": "dis", "entity": "小儿慢性肾功能不全" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "获得性肾小球疾病" }, { "start_idx": 80, "end_idx": 94, "type": "dis", "entity": "先天性和遗传性肾脏疾病平分秋色" } ] }, { "text": "其常见病因获得性肾小球疾病比例下降(66.7%→45.9%),先天性和遗传性肾脏疾病比例明显增加(33.3%→45.9%)。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "获得性肾小球疾病" }, { "start_idx": 31, "end_idx": 41, "type": "dis", "entity": "先天性和遗传性肾脏疾病" } ] }, { "text": "结合20世纪70年代中期起的国外统计资料,也发现由获得性肾小球疾病引起的慢性肾功能不全逐渐减少,取而代之占主导地位的是先天性和遗传性肾脏疾病。", "entities": [ { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "获得性肾小球疾病" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "慢性肾功能不全" }, { "start_idx": 59, "end_idx": 69, "type": "dis", "entity": "先天性和遗传性肾脏疾病" } ] }, { "text": "【发生机制】有关慢性肾衰竭的发病机制,历年来先后提出过“尿毒症毒素学说”、“矫枉失衡学说”、“肾小球高滤过学说”、“脂肪代谢紊乱学说”以及“肾小管高代谢学说”等等,晚近又有人提出“蛋白尿学说”、“慢性酸中毒学说”以及高蛋白饮食、肾内低氧对肾功能的影响等。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "慢性肾衰竭" } ] }, { "text": "(一)健存肾单位的血流动力学改变肾单位受损或失用后,剩余健全的肾单位一系列适应性改变即负担起全肾功能性代偿及小球、小管各部分间的适应,部分健存肾单位功能高于正常,引起单个肾单位的肾小球滤过率增高,肾小球毛细血管压力增加,内皮细胞增生,系膜区基质增多,小球体积增大,逐步出现肾小球硬化。", "entities": [ { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "肾" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "肾" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "小球" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "小管" }, { "start_idx": 89, "end_idx": 96, "type": "sym", "entity": "肾小球滤过率增高" }, { "start_idx": 98, "end_idx": 108, "type": "sym", "entity": "肾小球毛细血管压力增加" }, { "start_idx": 110, "end_idx": 115, "type": "sym", "entity": "内皮细胞增生" }, { "start_idx": 117, "end_idx": 123, "type": "sym", "entity": "系膜区基质增多" }, { "start_idx": 125, "end_idx": 130, "type": "sym", "entity": "小球体积增大" }, { "start_idx": 132, "end_idx": 140, "type": "sym", "entity": "逐步出现肾小球硬化" } ] }, { "text": "这一学说认为,CRF时体内某些物质的积聚,并非全部由于肾清除减少所致,而是机体为了纠正代谢失调的一种平衡适应,其结果又导致新的不平衡,如此周而复始,造成了进行性损害,成为CRF患者病情进展的重要原因之一。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "CRF" }, { "start_idx": 85, "end_idx": 87, "type": "dis", "entity": "CRF" } ] }, { "text": "CRF时甲状旁腺素(parathyroidhormone,PTH)升高造成的危害是本学说最好的证据。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "甲状旁腺素" }, { "start_idx": 10, "end_idx": 27, "type": "bod", "entity": "parathyroidhormone" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "PTH" } ] }, { "text": "随着GRF降低,尿磷排泄量减少,引起高磷血症。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "高磷血症" } ] }, { "text": "由于血清中钙磷乘积的升高,一方面使无机盐在各器官(包括肾脏)沉积,出现软组织钙化;另一方面,低钙血症又刺激了PTH的合成和分泌,代偿性促进尿磷排泄并升高血钙。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血清" }, { "start_idx": 5, "end_idx": 8, "type": "ite", "entity": "钙磷乘积" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "无机盐" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肾脏" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "软组织钙化" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "低钙血症" }, { "start_idx": 76, "end_idx": 77, "type": "ite", "entity": "血钙" } ] }, { "text": "但对甲状旁腺的持续性刺激则又导致甲状旁腺的增生及继发性甲状旁腺功能亢进(secondaryhyperparathyroidism,SHP),从而累及骨骼、心血管及造血系统等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "甲状旁腺" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "甲状旁腺的增生" }, { "start_idx": 24, "end_idx": 34, "type": "dis", "entity": "继发性甲状旁腺功能亢进" }, { "start_idx": 36, "end_idx": 63, "type": "dis", "entity": "secondaryhyperparathyroidism" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "SHP" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "骨骼" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "心血管" }, { "start_idx": 81, "end_idx": 84, "type": "bod", "entity": "造血系统" } ] }, { "text": "矫枉失衡学说对于进一步解释各种慢性肾脏疾病进展的原因,加深人们对CRF时钙磷代谢紊乱及SHP发病机制的认识具有重要意义,因此一直为各国学者所推崇。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "慢性肾脏疾病" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "CRF" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "钙磷代谢紊乱" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "SHP" } ] }, { "text": "首先,磷的潴留并非产生SHP的始动因素;只有当肾衰竭进入晚期(GFR<20ml/min)时,患者才出现磷的潴留。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "磷的潴留" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "磷的潴留" } ] }, { "text": "高磷血症不仅可以通过低钙血症,还可以通过其他途径直接或间接促进PTH的分泌。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "高磷血症" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "低钙血症" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "PTH" } ] }, { "text": "磷对甲状旁腺还可能具有直接作用,因为低磷饮食可在血清中钙和1,25-(OH)2D3浓度无变化的情况下,降低PTH及其前体PTHmRNA的水平。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "甲状旁腺" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "血清中钙" }, { "start_idx": 65, "end_idx": 67, "type": "ite", "entity": "PTH" }, { "start_idx": 72, "end_idx": 78, "type": "ite", "entity": "PTHmRNA" } ] }, { "text": "其次,低钙血症也并非引起SHP的唯一直接原因。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "低钙血症" } ] }, { "text": "除了低钙血症外,还有其他重要因素参与了SHP的形成。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "低钙血症" } ] }, { "text": "现已证实SHP的发生和发展最重要的机制是:①1,25-(OH)2D3的缺乏和甲状旁腺对1,25-(OH)2D3的抵抗;②血钙水平对PTH分泌的调控作用减弱,即所谓调控点(set-point,指降低血清PTH水平至50%所需的钙离子浓度)上移,骨骼对PTH提高血钙的调节作用具有抵抗,加重了低钙血症;③肾脏对PTH的降解作用障碍,使血循环中残留的PTH片段增加等。", "entities": [ { "start_idx": 84, "end_idx": 85, "type": "ite", "entity": "血钙" }, { "start_idx": 89, "end_idx": 91, "type": "bod", "entity": "PTH" }, { "start_idx": 122, "end_idx": 126, "type": "ite", "entity": "血清PTH" }, { "start_idx": 136, "end_idx": 138, "type": "ite", "entity": "钙离子" }, { "start_idx": 145, "end_idx": 146, "type": "bod", "entity": "骨骼" }, { "start_idx": 168, "end_idx": 171, "type": "dis", "entity": "低钙血症" }, { "start_idx": 174, "end_idx": 175, "type": "bod", "entity": "肾脏" }, { "start_idx": 177, "end_idx": 179, "type": "bod", "entity": "PTH" }, { "start_idx": 196, "end_idx": 198, "type": "bod", "entity": "PTH" } ] }, { "text": "最近的研究表明口服补充生理剂量的1,25-(OH)2D3并不能完全抑制PTH的分泌,而仅仅在应用1,25-(OH)2D3冲击治疗导致体内超生理浓度时才能完全抑制PTH分泌,因此有学者提出甲状旁腺对1,25-(OH)2D3存在抵抗。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "生理剂" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "PTH" }, { "start_idx": 93, "end_idx": 96, "type": "ite", "entity": "生理浓度" }, { "start_idx": 104, "end_idx": 106, "type": "bod", "entity": "PTH" }, { "start_idx": 117, "end_idx": 120, "type": "bod", "entity": "甲状旁腺" } ] }, { "text": "现已知甲状旁腺的主细胞中存在维生素D特异性受体(vitaminDreceptor,VDR),CRF时这种受体的密度和结合力均降低,使1,25-(OH)2D3作用下降。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "甲状旁腺" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "主细胞" }, { "start_idx": 14, "end_idx": 22, "type": "bod", "entity": "维生素D特异性受体" }, { "start_idx": 24, "end_idx": 39, "type": "bod", "entity": "vitaminDreceptor" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "VDR" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "CRF" } ] }, { "text": "(三)尿毒症毒素目前已知的尿素、多胺类、胍类、中分子量物质及甲状旁腺素在尿毒症期血浓度都增高。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "尿毒症毒素" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "尿素" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "多胺类" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "胍类" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "中分子量物质" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "甲状旁腺素" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "尿毒症期" }, { "start_idx": 40, "end_idx": 42, "type": "ite", "entity": "血浓度" } ] }, { "text": "它们对心脏、促红细胞生成素、Na-K-ATP酶、神经、肌肉以及血小板聚集代谢等均有一定毒性。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心脏" }, { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "促红细胞生成素" }, { "start_idx": 14, "end_idx": 22, "type": "bod", "entity": "Na-K-ATP酶" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "神经" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肌肉" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "血小板" } ] }, { "text": "(四)肾小管间质损伤肾小管间质病变与肾小球疾病进展的关系已受到重视。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "肾小管间质损伤" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "肾小管间质病变" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "肾小球疾病" } ] }, { "text": "这种肾小管间质的形态学上的变化如肾小管萎缩、肾间质细胞浸润及间质纤维化一旦发生后,则进一步通过小管内阻力增加、正常的管球反馈功能丧失以及不能维持正常的渗透梯度等功能改变,加剧肾功能恶化。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "肾小管间质" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "肾小管萎缩" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "肾间质细胞浸润" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "间质纤维化" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "小管" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "管球" } ] }, { "text": "(五)饮食影响膳食中高蛋白摄入可使入球小动脉扩张,加剧肾小球的高灌注损伤,并可加剧蛋白尿。", "entities": [ { "start_idx": 7, "end_idx": 43, "type": "sym", "entity": "膳食中高蛋白摄入可使入球小动脉扩张,加剧肾小球的高灌注损伤,并可加剧蛋白尿" } ] }, { "text": "膳食中盐过高除影响全身血压外,观察到还可致肾小球容积加大和硬化,磷的摄入亦应限制,低磷饮食可防止钙磷盐沉积于血管壁和组织,抑制甲状旁腺的分泌。", "entities": [ { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "肾小球容积加大和硬化" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "血管壁" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "甲状旁腺" } ] }, { "text": "高脂血症除影响内皮细胞外,还刺激肾小球系膜的增生及细胞外基质的积聚,而易发生肾小球硬化。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "高脂血症" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "肾小球" } ] }, { "text": "(六)肾素-血管紧张素系统(reninangiotensinsystem,RAS)在肾脏病进展中,血管紧张素Ⅱ(AⅡ)的作用也受到重视。", "entities": [ { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "肾脏病" }, { "start_idx": 49, "end_idx": 53, "type": "bod", "entity": "血管紧张素" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "AⅡ" } ] }, { "text": "AⅡ可通过以下机制导致或加重肾脏病的进展:①作为一种血管活性物质,优先收缩肾小球出球小动脉刺激肾小球系膜的增生及细胞外基质的积聚,而易发生肾小球硬化系数;③促进水盐重吸收和兴奋肾交感神经;④作为促肾生长因子,除使系膜细胞增生肥大外,还能刺激其他血管活性物及细胞因子产生(如TGF-β1),导致细胞外基质进行性积聚;⑤抑制细胞外基质血管活性物质因引起肾小球高滤过而加重蛋白尿;⑦促进肾小管上皮细胞氨的产生,后者又通过激活补体引起肾损伤;⑧促进肾小管上皮细胞钠的重吸收,增加肾组织氧耗,引起肾组织氧供相对不足,加重肾损害。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "AⅡ" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肾脏病" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肾小球" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "肾小球" }, { "start_idx": 45, "end_idx": 73, "type": "sym", "entity": "刺激肾小球系膜的增生及细胞外基质的积聚,而易发生肾小球硬化" }, { "start_idx": 88, "end_idx": 92, "type": "bod", "entity": "肾交感神经" }, { "start_idx": 98, "end_idx": 102, "type": "bod", "entity": "肾生长因子" }, { "start_idx": 122, "end_idx": 126, "type": "bod", "entity": "血管活性物" }, { "start_idx": 128, "end_idx": 131, "type": "bod", "entity": "细胞因子" }, { "start_idx": 146, "end_idx": 147, "type": "bod", "entity": "细胞" }, { "start_idx": 165, "end_idx": 170, "type": "bod", "entity": "血管活性物质" }, { "start_idx": 174, "end_idx": 176, "type": "bod", "entity": "肾小球" }, { "start_idx": 183, "end_idx": 185, "type": "bod", "entity": "蛋白尿" }, { "start_idx": 190, "end_idx": 197, "type": "bod", "entity": "肾小管上皮细胞氨" }, { "start_idx": 220, "end_idx": 226, "type": "bod", "entity": "肾小管上皮细胞" } ] }, { "text": "【临床表现】(一)电解质、酸碱代谢失常1.水代谢早期由于浓缩功能减退,尿量不减少或反而增多,晚期尿量才有减少,终末期可发展到无尿。", "entities": [ { "start_idx": 9, "end_idx": 18, "type": "sym", "entity": "电解质、酸碱代谢失常" }, { "start_idx": 28, "end_idx": 53, "type": "sym", "entity": "浓缩功能减退,尿量不减少或反而增多,晚期尿量才有减少" }, { "start_idx": 55, "end_idx": 63, "type": "sym", "entity": "终末期可发展到无尿" } ] }, { "text": "患者对水代谢调节能力减退,当水分摄入过多时,易在体内潴留并形成稀释性低钠血症,摄入过少时也易引起体内水分不足。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "水代谢调节能力减退" }, { "start_idx": 13, "end_idx": 37, "type": "sym", "entity": "当水分摄入过多时,易在体内潴留并形成稀释性低钠血症" }, { "start_idx": 39, "end_idx": 53, "type": "sym", "entity": "摄入过少时也易引起体内水分不足" } ] }, { "text": "2.钾代谢有高钾血症趋势,细胞内钾的积聚与Na-KATP酶活力下降有关。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "高钾血症" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "细胞" }, { "start_idx": 21, "end_idx": 28, "type": "bod", "entity": "Na-KATP酶" } ] }, { "text": "高钾血症可随外伤、手术、麻醉、输血、酸中毒及突然更改饮食等而加剧,慢性肾衰时血钾升高是一方面,但总体钾的存储量仍降低,所以保持钾的正常平衡仍是潴留。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "高钾血症" }, { "start_idx": 5, "end_idx": 31, "type": "sym", "entity": "随外伤、手术、麻醉、输血、酸中毒及突然更改饮食等而加剧" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "慢性肾衰" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "血钾" }, { "start_idx": 48, "end_idx": 68, "type": "sym", "entity": "总体钾的存储量仍降低,所以保持钾的正常平衡" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "潴留" } ] }, { "text": "3.钠代谢CRF可以维持钠正常平衡状态相当长时间,这与健存肾单位及利钠激素等体液因子有关。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "CRF" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "利钠激素" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "体液因子" } ] }, { "text": "(1)钠消耗型:盐分丢失型肾病因细胞外液的缩小及低血压等均有钠酸中毒失。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "盐分丢失型肾病" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "酸中毒" } ] }, { "text": "很多疾病可引起盐分丢失,如肾盂肾炎、肾髓质囊性病、肾积水及间质性肾炎等,这类病人的集合管往往不能吸收运输过来足够量的钠盐而出现低钠。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "肾髓质囊性病" }, { "start_idx": 25, "end_idx": 33, "type": "dis", "entity": "肾积水及间质性肾炎" } ] }, { "text": "(2)钠潴留型:当摄入钠过多时,不能正常排泄以致钠潴留,体内细胞外容量增加,发生高血压、肺充血与心脏扩大,甚至心力衰竭。", "entities": [ { "start_idx": 8, "end_idx": 58, "type": "sym", "entity": "当摄入钠过多时,不能正常排泄以致钠潴留,体内细胞外容量增加,发生高血压、肺充血与心脏扩大,甚至心力衰竭" } ] }, { "text": "4.酸碱平衡慢性肾衰病人早期肾小管合成氨的肾盂肾炎力未全丧失,可动员体内其他缓冲系统来代偿代谢性酸中毒,如呼吸系统,组织代偿如骨盐的丢失等。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肾小管" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "呼吸系统" } ] }, { "text": "当病情进展,健存肾单位进一步减少,GFR<20ml/min时肾脏排泄有机酸能力下降,排氨能力减低,引起酸中毒肺充血血pH<7.25时要警惕合并酮症酸中毒。", "entities": [ { "start_idx": 0, "end_idx": 47, "type": "sym", "entity": "当病情进展,健存肾单位进一步减少,GFR<20ml/min时肾脏排泄有机酸能力下降,排氨能力减低" }, { "start_idx": 49, "end_idx": 75, "type": "sym", "entity": "引起酸中毒肺充血血pH<7.25时要警惕合并酮症酸中毒" } ] }, { "text": "5.其他电解质慢性肾衰病人不能充分排泄氯离子,高氯血症与钠浓度成正比;血钙浓度往往降低,慢性肾衰患者常能忍受低血钙而不致搐搦,这些患者的肠道钙的吸收能力下降,口服活性维生素D可提高血钙浓度;当GFR<20ml/min时,血镁可升高,尿血pH减少。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "慢性肾衰病" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "高氯血症" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "钠浓度" }, { "start_idx": 35, "end_idx": 36, "type": "ite", "entity": "血钙" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "慢性肾衰" }, { "start_idx": 55, "end_idx": 56, "type": "ite", "entity": "血钙" }, { "start_idx": 81, "end_idx": 86, "type": "dru", "entity": "活性维生素D" }, { "start_idx": 90, "end_idx": 91, "type": "ite", "entity": "血钙" }, { "start_idx": 110, "end_idx": 111, "type": "ite", "entity": "血镁" }, { "start_idx": 117, "end_idx": 119, "type": "ite", "entity": "血pH" } ] }, { "text": "当血镁较高(>2mmol/L)有临床症状时则可应用排钠利尿剂,促镁排出,纠正脱水,必要时给透析疗法。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "血镁" }, { "start_idx": 25, "end_idx": 29, "type": "dru", "entity": "排钠利尿剂" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "透析疗法" } ] }, { "text": "GFR<20ml搐搦min时,血磷升高较明显,病情进展到肾脏排磷进一步减少。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "搐搦" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "血磷" } ] }, { "text": "(二)血管系统1.高血压常见原因有①GFR下降、NO分泌减少,使VDML血管减低的髓脂质下降,引起细胞外容量增加,心搏出量增加,继而外周阻力增加,血排钠利尿剂②肾素、血管紧张素及醛固酮系统活跃,肾素分泌过多。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "高血压" }, { "start_idx": 74, "end_idx": 78, "type": "dru", "entity": "排钠利尿剂" } ] }, { "text": "2.心包炎尿毒性心包炎似由不明的生化物质、尿酸沉积及代谢异常所引起。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "尿毒性心包炎" } ] }, { "text": "属纤维性心包炎,有渗出、出血,可闻及心包摩擦音,偶发生心包填塞VDML血管减低的髓脂质下降,有不同程度的心肌肥厚,间质纤维化,心肌钙化,草酸盐沉积。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "纤维性心包炎" }, { "start_idx": 8, "end_idx": 22, "type": "sym", "entity": "有渗出、出血,可闻及心包摩擦音" }, { "start_idx": 31, "end_idx": 44, "type": "sym", "entity": "VDML血管减低的髓脂质下降" }, { "start_idx": 46, "end_idx": 55, "type": "sym", "entity": "有不同程度的心肌肥厚" }, { "start_idx": 57, "end_idx": 72, "type": "sym", "entity": "间质纤维化,心肌钙化,草酸盐沉积" } ] }, { "text": "临床表现心脏扩大,心输出量减少,各种心律失肾素。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "sym", "entity": "心脏扩大,心输出量减少" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "各种心律失肾素" } ] }, { "text": "(三)胃肠系统胃纳减退,常见有呕吐及恶心等症状,加重了水、盐代谢及酸碱平衡紊乱,负氮平衡加剧,对钙的吸收下降。", "entities": [ { "start_idx": 7, "end_idx": 53, "type": "sym", "entity": "胃纳减退,常见有呕吐及恶心等症状,加重了水、盐代谢及酸碱平衡紊乱,负氮平衡加剧,对钙的吸收下降" } ] }, { "text": "另外消化道出血偶发生心包填塞于黏膜有弥散性小出血点炎症及溃疡引起。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "偶发生心包填塞" }, { "start_idx": 15, "end_idx": 31, "type": "sym", "entity": "黏膜有弥散性小出血点炎症及溃疡引起" } ] }, { "text": "(四)精神神经症状,乏力、失眠、激惹、压抑、记忆力减退或反抗心理行为尿毒症伴有各种心律失常能亢进时可使脑细胞钙离子浓度增高,出现不正常脑电图。", "entities": [ { "start_idx": 3, "end_idx": 33, "type": "sym", "entity": "精神神经症状,乏力、失眠、激惹、压抑、记忆力减退或反抗心理行为" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "尿毒症" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "各种心律失常" }, { "start_idx": 51, "end_idx": 58, "type": "ite", "entity": "脑细胞钙离子浓度" }, { "start_idx": 67, "end_idx": 69, "type": "ite", "entity": "脑电图" } ] }, { "text": "临床可有谵妄、木僵,甚至昏迷。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "sym", "entity": "谵妄、木僵,甚至昏迷" } ] }, { "text": "周围神经症状如痛性肢体麻痹,深腱反射消失,消化道出血、痉挛甚至感觉消失,被认为与体内中分子物质积聚有关。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "周围神经症状如痛性肢体麻痹,深腱反射消失" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "消化道出血" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "痉挛甚至感觉消失" } ] }, { "text": "(五)血液系统1.贫血呈正血色素、正细胞性记忆力减退随肾功能减退而加剧。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "贫血" }, { "start_idx": 11, "end_idx": 34, "type": "sym", "entity": "呈正血色素、正细胞性记忆力减退随肾功能减退而加剧" } ] }, { "text": "主要由于肾脏产生促红细胞生成素减少有关;其次为红细胞寿命缩短,饮食中铁及叶酸摄入不足也参与一定因素昏迷另外,中性粒细胞趋化性改变,淋巴细胞功能受抑制,免疫功能降低。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肾脏" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "促红细胞生成素减少" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "红细胞寿命缩短" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "昏迷" }, { "start_idx": 54, "end_idx": 80, "type": "sym", "entity": "中性粒细胞趋化性改变,淋巴细胞功能受抑制,免疫功能降低" } ] }, { "text": "2.出血倾向可有鼻出血,损伤后出血不止。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "出血倾向" }, { "start_idx": 6, "end_idx": 18, "type": "sym", "entity": "可有鼻出血,损伤后出血不止" } ] }, { "text": "消化道出血与出血时间延长、血小板功能异常、黏附聚集能力降低及第三因子释放减少有关。", "entities": [ { "start_idx": 0, "end_idx": 28, "type": "sym", "entity": "消化道出血与出血时间延长、血小板功能异常、黏附聚集能力降低" } ] }, { "text": "(六)促红细胞生成素肪代谢障碍CRF时肾脏清除胰岛素能力减退,血中胰岛素升高。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "bod", "entity": "促红细胞生成素" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "CRF" }, { "start_idx": 19, "end_idx": 37, "type": "sym", "entity": "肾脏清除胰岛素能力减退,血中胰岛素升高" } ] }, { "text": "慢性肾衰患者一般都有负氮平衡、血浆及细胞内游离氨基酸谱异常及低白蛋白血症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "慢性肾衰" }, { "start_idx": 10, "end_idx": 35, "type": "sym", "entity": "负氮平衡、血浆及细胞内游离氨基酸谱异常及低白蛋白血症" } ] }, { "text": "血甘油三酯增高,低密度脂蛋白增高,高密度脂蛋白降低,可能与脂蛋白酯酶及肝酯酶活性下降有关。", "entities": [ { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "血甘油三酯增高,低密度脂蛋白增高,高密度脂蛋白降低" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "脂蛋白酯酶" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "肝酯酶" } ] }, { "text": "(七)其他GFR降到一定程度时可有高尿素血症及高尿酸血症,皮肤有瘙痒CRF色素沉着,身上散发一股尿毒症臭味,与尿素分泌增加排出减少有关。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "GFR" }, { "start_idx": 17, "end_idx": 27, "type": "sym", "entity": "高尿素血症及高尿酸血症" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "皮肤有瘙痒" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "CRF" }, { "start_idx": 42, "end_idx": 52, "type": "sym", "entity": "身上散发一股尿毒症臭味" } ] }, { "text": "CRF患者由于营养不良,免疫功能低下,易罹患各种感染。", "entities": [ { "start_idx": 7, "end_idx": 25, "type": "sym", "entity": "营养不良,免疫功能低下,易罹患各种感染" } ] }, { "text": "小儿由于摄入不足及内分泌紊乱等因素高密度脂蛋白降低迟缓,或发生肾性佝偻病。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "小儿由于摄入不足及内分泌紊乱" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "高密度脂蛋白降低" }, { "start_idx": 25, "end_idx": 35, "type": "sym", "entity": "迟缓,或发生肾性佝偻病" } ] }, { "text": "【诊断与鉴别诊断】慢性肾衰到晚期各种症状明显时容易诊断,重要的是认识早期的慢性肾衰竭,设法延缓肾功能进行性恶化。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "慢性肾衰" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "慢性肾衰竭" } ] }, { "text": "慢性肾衰分期:①肾功能不全代偿期,血肌酐为110~177μmol/L(1.2~2mg/dl),GFR剩余50%~80内分泌紊乱临床症状;②肾功能不全失代偿期(氮质血症期):血肌酐为178~445μmol/L(2~5mg/dl),GFR剩余25%~50%,可有轻度贫血、酸中毒、夜尿及乏力;③肾衰竭期(尿毒症期):Cr为446~707μmol/L(5~8mg/dl),GFR剩余10%~25%,有明显消化道症状及贫血肾功能不全失代偿期酸中毒及钙、磷代谢异常;④终末期肾病:Cr大于等于708μmol/L(8mg/dl),GFR剩余小于10%,有各种尿毒症症状,包括消化、神经及心血管各系统功能异Cr,水、盐代谢紊乱,酸碱失衡明显,严重贫血。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "肾功能不全代偿期" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "血肌酐" }, { "start_idx": 47, "end_idx": 49, "type": "ite", "entity": "GFR" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "内分泌紊乱" }, { "start_idx": 69, "end_idx": 77, "type": "dis", "entity": "肾功能不全失代偿期" }, { "start_idx": 114, "end_idx": 116, "type": "ite", "entity": "GFR" }, { "start_idx": 127, "end_idx": 142, "type": "sym", "entity": "可有轻度贫血、酸中毒、夜尿及乏力" }, { "start_idx": 145, "end_idx": 148, "type": "dis", "entity": "肾衰竭期" }, { "start_idx": 150, "end_idx": 152, "type": "dis", "entity": "尿毒症" }, { "start_idx": 183, "end_idx": 185, "type": "ite", "entity": "GFR" }, { "start_idx": 196, "end_idx": 203, "type": "sym", "entity": "有明显消化道症状" }, { "start_idx": 207, "end_idx": 215, "type": "dis", "entity": "肾功能不全失代偿期" }, { "start_idx": 216, "end_idx": 226, "type": "sym", "entity": "酸中毒及钙、磷代谢异常" }, { "start_idx": 229, "end_idx": 233, "type": "dis", "entity": "终末期肾病" }, { "start_idx": 235, "end_idx": 236, "type": "ite", "entity": "Cr" }, { "start_idx": 259, "end_idx": 261, "type": "ite", "entity": "GFR" }, { "start_idx": 296, "end_idx": 297, "type": "dis", "entity": "Cr" }, { "start_idx": 271, "end_idx": 317, "type": "sym", "entity": "各种尿毒症症状,包括消化、神经及心血管各系统功能异Cr,水、盐代谢紊乱,酸碱失衡明显,严重贫血" } ] }, { "text": "目前临床上多使用慢性肾脏疾病(chronickidneydisease,CKD)概念,CKD的定义:①肾损害(病理、血、尿及影像学异常)≥3个月;②GFR<60ml/(min•1.73m2),持续时间≥3个月。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "慢性肾脏疾病" }, { "start_idx": 15, "end_idx": 34, "type": "dis", "entity": "chronickidneydisease" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "CKD" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "CKD" }, { "start_idx": 51, "end_idx": 71, "type": "sym", "entity": "肾损害(病理、血、尿及影像学异常)≥3个月" }, { "start_idx": 74, "end_idx": 109, "type": "sym", "entity": "GFR<60ml/(min•1.73m2),持续时间≥3个月" } ] }, { "text": "具有以上两条的任何一条者,就可以诊断为CKD。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "CKD" } ] }, { "text": "CKD分期为:1期GFR>90ml/(min•1.73m2);3期GFR30~59ml/(min•1.73m2);4期GFR15~29ml/(min•1.73m2);5期GFR<15ml/(min•1.73m2)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "CKD" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "1期GFR" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "CKD" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "2期GFR" }, { "start_idx": 70, "end_idx": 74, "type": "dis", "entity": "3期GFR" }, { "start_idx": 102, "end_idx": 106, "type": "dis", "entity": "4期GFR" }, { "start_idx": 134, "end_idx": 138, "type": "dis", "entity": "5期GFR" } ] }, { "text": "5期即为尿毒症期。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "尿毒症期" } ] }, { "text": "引起CRF病因多种,如由肾小球疾病引起者多有水肿,尿液异常者较易诊断。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "CRF" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "肾小球疾病" }, { "start_idx": 22, "end_idx": 33, "type": "sym", "entity": "水肿,尿液异常者较易诊断" } ] }, { "text": "某些症状如纳差、不爱活动、夜尿或遗尿等症状无特异性。", "entities": [ { "start_idx": 5, "end_idx": 24, "type": "sym", "entity": "纳差、不爱活动、夜尿或遗尿等症状无特异性" } ] }, { "text": "也有因贫血待查、难治性佝偻病5期GFR育迟缓以及多饮多尿而来就诊者,则需经仔细的体检、尿液检查(包括比重)及血生化肾功能等测定以及时检出CRF,并尽量寻找病因。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "5期GFR" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "多饮多尿" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "CRF" } ] }, { "text": "如由泌尿系先天性畸形的肾发育不良、多囊肾及遗传性疾病如Alport综合征引起的肾衰,发病年夜尿早。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "泌尿系先天性畸形" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肾发育不良" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "多囊肾" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 27, "end_idx": 35, "type": "dis", "entity": "Alport综合征" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "肾衰" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "夜尿" } ] }, { "text": "常无水肿,以身材矮小及肾性骨病较多见。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "常无水肿,以身材矮小及肾性骨病较多见" } ] }, { "text": "肾小球疾病引起的CRF多见于较大儿童,常>5岁,可伴贫血、高血压及水肿,有中等量蛋白尿、血尿及低比重尿,或合并继发性尿路感染。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肾小球疾病" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "CRF" }, { "start_idx": 24, "end_idx": 61, "type": "sym", "entity": "可伴贫血、高血压及水肿,有中等量蛋白尿、血尿及低比重尿,或合并继发性尿路感染" } ] }, { "text": "肾衰的急性肾发育不良与急性肾衰竭相鉴别。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "肾发育不良" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "两者的临床表现相似,病因及诱因也有部分相同,但大多数急性肾衰预后良好,少部分患者恢复期后可逐渐发展到CRF。", "entities": [ { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "CRF" } ] }, { "text": "由于先天性或遗传性肾脏疾病而致慢性肾功能不全的,小儿明显多于成人,并且小儿以有中等量蛋白尿、血尿及低比重尿人的先天性或遗传性肾脏疾病则主要见于先天性多囊肾。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "先天性或遗传性肾脏疾病" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "慢性肾功能不全" }, { "start_idx": 38, "end_idx": 52, "type": "sym", "entity": "有中等量蛋白尿、血尿及低比重尿" }, { "start_idx": 59, "end_idx": 65, "type": "dis", "entity": "遗传性肾脏疾病" }, { "start_idx": 71, "end_idx": 76, "type": "dis", "entity": "先天性多囊肾" } ] }, { "text": "【治疗】虽然造成慢性肾功能不全的一些原发病尚无特异治疗,但有相当一部分因素引起的肾功能损害是可逆的,如感染、尿路梗阻、脱水及有效循环血量的减少等,及时去除诱因,肾功能仍有部分或全部恢复的可能。", "entities": [ { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "感染" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "尿路梗阻" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "脱水" }, { "start_idx": 62, "end_idx": 70, "type": "sym", "entity": "有效循环血量的减少" }, { "start_idx": 75, "end_idx": 78, "type": "pro", "entity": "去除诱因" } ] }, { "text": "有些治疗能延缓慢性肾功能不全的发展。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "慢性肾功能不全" } ] }, { "text": "鉴于经济的原因,目前国内仅少数单位开展肾脏替代治疗,对于小儿慢性肾衰竭的治疗,多为对症处理,因此,重点应做到早期诊断,明确病因,纠正代谢紊乱,防治感染,避免引起肾功能急剧恶化的诱因发生等。", "entities": [ { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "小儿慢性肾衰竭" }, { "start_idx": 73, "end_idx": 74, "type": "sym", "entity": "感染" } ] }, { "text": "(一)饮食疗法低蛋白摄入为传统疗法,因肾功能减退到一定程度时不能有效排出蛋白分解产物,高蛋白饮食必然加重氮质血症。", "entities": [ { "start_idx": 7, "end_idx": 16, "type": "pro", "entity": "低蛋白摄入为传统疗法" } ] }, { "text": "主食以麦淀粉、红薯、芋艿及土豆等含蛋白较低的食物替代部分米、面,有利于促进肠道内尿素氮的吸附,后由大便排出。", "entities": [ { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "尿素氮" } ] }, { "text": "有高钾血症时避免水果过分摄入。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "高钾血症" } ] }, { "text": "补充必需氨基酸并配合低蛋白饮食,摄入体内后可利用含氮代谢产物,促进蛋白质合成,减轻氮质血症,维持正氮平衡。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "氨基酸" } ] }, { "text": "常用的口服有肾灵片(含9种必需氨基酸)也称开同片(ketosteril),静脉滴注的有肾必氨(含9种必需氨基酸)注射液。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "肾灵片" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "开同片" }, { "start_idx": 25, "end_idx": 34, "type": "dru", "entity": "ketosteril" }, { "start_idx": 43, "end_idx": 45, "type": "dru", "entity": "肾必氨" } ] }, { "text": "(二)纠正水、电解质紊乱及酸碱平衡失调对有水肿、高血压、心功能差及少尿、无尿者应严格限制摄入量,详见急性肾衰竭一节。", "entities": [ { "start_idx": 3, "end_idx": 18, "type": "pro", "entity": "纠正水、电解质紊乱及酸碱平衡失调" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "水肿" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "高血压" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "心功能差" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "当有吐、泻或消化道失血等脱水、休克现象应即予以纠正肾灵片以保证肾小球的有效肾血流量及滤过率。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "当有吐、泻或消化道失血等脱水、休克现象" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "肾灵片" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "肾小球" }, { "start_idx": 37, "end_idx": 40, "type": "ite", "entity": "肾血流量" }, { "start_idx": 42, "end_idx": 44, "type": "ite", "entity": "滤过率" } ] }, { "text": "血钠<120mmol/L伴有低钠症状时可口服氯化钠2~4g/d,或用氯化钠静脉滴入。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "氯化钠" }, { "start_idx": 34, "end_idx": 40, "type": "pro", "entity": "氯化钠静脉滴入" } ] }, { "text": "常用为3%NaCl,1ml3%NaCl稀释性低钠血症mol,先给总量的1/2,以后根据血压、心脏及复查血钠决定是否再补。", "entities": [ { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "稀释性低钠血症" } ] }, { "text": "常用药物有10%葡萄糖酸钙每次0.5~1ml/kg,静脉缓注,或5%碳酸氢钠每次3~5ml/kg,静脉滴注。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "葡萄糖酸钙" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "静脉缓注" }, { "start_idx": 32, "end_idx": 52, "type": "pro", "entity": "5%碳酸氢钠每次3~5ml/kg,静脉滴注" } ] }, { "text": "当血钾>6.5mmol/L,或心电图有高血钾心肌损害时需给透析治疗。", "entities": [ { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "高血钾心肌损害" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "透析治疗" } ] }, { "text": "轻度酸中毒不予处理。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "轻度酸中毒" } ] }, { "text": "当TCO2尿毒症sub><13mmol/L伴临床症状时应予治疗。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "尿毒症" } ] }, { "text": "口服Shohl氏溶液(枸橼酸70g加枸橼酸钠50g,以蒸馏水冲到500ml,1ml含1mmolNa,按钠2~3mmol/(kg•d)给予。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dru", "entity": "Shohl氏溶液" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "枸橼酸" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "枸橼酸钠" } ] }, { "text": "或用5%NaHCO心电图/sub>静脉滴注,按下面公式(30-缓注实测得的TCO2数)×0.5×kg体重=所需的5%NaHCO3毫升数。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "心电图" } ] }, { "text": "高磷血症应限制磷的摄入和使用结合剂,常用药物为碳酸钙。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "高磷血症" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "碳酸钙" } ] }, { "text": "适当补充铁、锌,避免铝的摄入。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "pro", "entity": "适当补充铁、锌,避免铝的摄入" } ] }, { "text": "(三)各系统症状处理1.肾性骨病定期监测血钙、血磷,并防止甲状腺功能过度亢进及骨骼外钙化治疗。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "肾性骨病" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "血钙" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "血磷" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "甲状腺" }, { "start_idx": 39, "end_idx": 45, "type": "pro", "entity": "骨骼外钙化治疗" } ] }, { "text": "控制高血磷,使用磷结合剂。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "pro", "entity": "控制高血磷,使用磷结合剂" } ] }, { "text": "补充钙盐,如碳酸钙、乳酸钙或萄糖酸钙,同时加用活性维生素D3,常用有双氢速固醇,或1,25-(OH)2D3(Rocaltrol),剂量每日1次0.25μg/片,逐血钙过渡到隔日1次或每周2次口服。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "补充钙盐" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "碳酸钙" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "乳酸钙" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "萄糖酸钙" }, { "start_idx": 23, "end_idx": 35, "type": "dru", "entity": "活性维生素D3" }, { "start_idx": 40, "end_idx": 44, "type": "dru", "entity": "双氢速固醇" }, { "start_idx": 47, "end_idx": 70, "type": "dru", "entity": "1,25-(OH)2D3" }, { "start_idx": 72, "end_idx": 80, "type": "dru", "entity": "Rocaltrol" }, { "start_idx": 99, "end_idx": 100, "type": "ite", "entity": "血钙" } ] }, { "text": "每2周随访血钙,当血钙达11mg/dl(2.75mmol/L)时应减量葡萄糖酸钙。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "ite", "entity": "血钙" }, { "start_idx": 35, "end_idx": 39, "type": "dru", "entity": "葡萄糖酸钙" } ] }, { "text": "2.控制高血压慢性肾衰高血压的基本处理原则为延缓肾衰的进展,其多数为容量依赖性,故需限制钠的摄入和使用利尿剂。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "控制高血压" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "慢性肾衰高血压" }, { "start_idx": 51, "end_idx": 52, "type": "dru", "entity": "利尿" } ] }, { "text": "常用药物有双氯噻嗪、氯噻酮及肼屈嗪等。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "双氯噻嗪" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "氯噻酮" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "肼屈嗪" } ] }, { "text": "当Ccr<15ml/(min•1.73m2)时,一般利尿药往往疗效不高,可应用呋塞米,剂量由小到大,逐渐递增。", "entities": [ { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "利尿药" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "呋塞米" } ] }, { "text": "降压药常用为血管紧张素转换酶抑制剂(ACEI)中的蒙诺(福辛普利fosinopril)或贝那普利(benazepril),此类药可扩张出入双氯噻嗪,但出球小动脉扩张更明显,从而使肾小球内压力降低,有利于延缓肾小球病变的进展,减少蛋白尿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "降压药" }, { "start_idx": 6, "end_idx": 16, "type": "dru", "entity": "血管紧张素转换酶抑制剂" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "ACEI" }, { "start_idx": 25, "end_idx": 26, "type": "dru", "entity": "蒙诺" }, { "start_idx": 28, "end_idx": 41, "type": "dru", "entity": "福辛普利fosinopril" }, { "start_idx": 44, "end_idx": 47, "type": "dru", "entity": "贝那普利" }, { "start_idx": 49, "end_idx": 58, "type": "dru", "entity": "benazepril" }, { "start_idx": 69, "end_idx": 72, "type": "dru", "entity": "双氯噻嗪" } ] }, { "text": "β受体阻滞剂通过抑制肾素而减少醛固酮分泌和水、钠潴留,起到降血压作用;临床应用的药物有普萘洛尔及阿替洛尔(苯氧蒙诺)等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "β受体阻滞剂" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肾素" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "醛固酮" }, { "start_idx": 43, "end_idx": 46, "type": "dru", "entity": "普萘洛尔" }, { "start_idx": 48, "end_idx": 51, "type": "dru", "entity": "阿替洛尔" }, { "start_idx": 53, "end_idx": 56, "type": "dru", "entity": "苯氧蒙诺" } ] }, { "text": "钙拮抗剂是使L型钙通道活性降低,抑制钙离子进入血管平滑肌细胞,使血管平滑肌张力降低,全身动脉扩张,血压下降;临床常用药物有硝苯地平(心痛定)及维拉帕米等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "钙拮抗剂" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "L型钙通道" }, { "start_idx": 23, "end_idx": 29, "type": "bod", "entity": "血管平滑肌细胞" }, { "start_idx": 61, "end_idx": 64, "type": "dru", "entity": "硝苯地平" }, { "start_idx": 66, "end_idx": 68, "type": "dru", "entity": "心痛定" }, { "start_idx": 71, "end_idx": 74, "type": "dru", "entity": "维拉帕米" } ] }, { "text": "已证明控制了β受体阻滞剂肾脏病患者其GFR下降速度低于未控制血压的患者。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "β受体阻滞剂" } ] }, { "text": "3.贫血与出血自从20世纪80年代应用重组人红细胞生成素(γHuEPO)治疗CRF患者的慢性贫血以来,基本上可使大多数病人不再接受输血。", "entities": [ { "start_idx": 22, "end_idx": 27, "type": "dru", "entity": "红细胞生成素" }, { "start_idx": 29, "end_idx": 34, "type": "dru", "entity": "γHuEPO" } ] }, { "text": "剂量为50~100U/(kg•次),隔天一次皮下心痛定。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "心痛定" } ] }, { "text": "血细胞压积上升到35%时减为每周2次,使其维持在35%~40%左右,注意该药可使血黏度增加,血压升高。", "entities": [ { "start_idx": 40, "end_idx": 42, "type": "ite", "entity": "血黏度" }, { "start_idx": 46, "end_idx": 47, "type": "ite", "entity": "血压" } ] }, { "text": "及时供应铁剂、叶酸及维生素B12等。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dru", "entity": "铁剂" }, { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "叶酸" }, { "start_idx": 10, "end_idx": 21, "type": "dru", "entity": "维生素B12" } ] }, { "text": "最近发现一种新的红细胞生成刺激蛋白(novelerythropoiesisstimulatingprotein,NESP),为一糖蛋白,半衰期是促红细胞生成素的3倍,治疗慢性肾衰中贫血,可更有效地血压患者的血红蛋白浓度。", "entities": [ { "start_idx": 8, "end_idx": 16, "type": "dru", "entity": "红细胞生成刺激蛋白" }, { "start_idx": 18, "end_idx": 54, "type": "dru", "entity": "novelerythropoiesisstimulatingprotein" }, { "start_idx": 56, "end_idx": 59, "type": "dru", "entity": "NESP" }, { "start_idx": 64, "end_idx": 66, "type": "dru", "entity": "糖蛋白" }, { "start_idx": 72, "end_idx": 78, "type": "dru", "entity": "促红细胞生成素" }, { "start_idx": 85, "end_idx": 91, "type": "dis", "entity": "慢性肾衰中贫血" }, { "start_idx": 98, "end_idx": 99, "type": "ite", "entity": "血压" }, { "start_idx": 103, "end_idx": 108, "type": "ite", "entity": "血红蛋白浓度" } ] }, { "text": "透析疗法可改善血小板功能和血小板第三因子的释放,有助于减少出血。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "透析疗法" } ] }, { "text": "严重出血时可酌用抗纤溶止血剂。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dru", "entity": "抗纤溶止血剂" } ] }, { "text": "4.防止小管、间质损伤肾小管受损重要原因之一是氨产生增加一糖蛋白激活C3直接引起肾间质炎性反应。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肾小管受损" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "一糖蛋白" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "肾间质炎性" } ] }, { "text": "给予重碳酸钠碱性药物时则尿中产氨下降,尿蛋白减少,理论上碱性药物有保护小管、间质受损的作用。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dru", "entity": "重碳酸钠碱性药物" }, { "start_idx": 12, "end_idx": 23, "type": "sym", "entity": "尿中产氨下降,尿蛋白减少" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "小管" } ] }, { "text": "晚期尿毒症到终末期Ccr<5%时,内科治疗不能见效只能通过透析疗法维持生命,以达最终肾移植目的。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "晚期尿毒症" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "透析疗法" } ] }, { "text": "第二节肾功能检查肾脏的主要生理功能有:①清除机体的最终代谢产物以及药物和毒物;②调节水、电解质及酸碱平衡;③产生重要的生物活性物质,如肾素、1,25(OH)2-D3和促红细胞生成素等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "肾功能检查" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肾脏" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "代谢产物" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "水" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "电解质" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "活性物质" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "肾素" }, { "start_idx": 72, "end_idx": 81, "type": "bod", "entity": "25(OH)2-D3" }, { "start_idx": 84, "end_idx": 89, "type": "bod", "entity": "红细胞生成素" } ] }, { "text": "一、分侧肾功能检查由于肾脏强大的储备能力,一侧肾功能受损甚至丧失后对肾脏总的生理功能影响极小。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "分侧肾功能检查" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肾脏" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "肾功能受损" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肾脏" } ] }, { "text": "此时,需了解两侧肾脏各自的功能状况。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肾脏" } ] }, { "text": "能分别反映两侧肾功能的方法有:①双肾放射性核素检查:肾动态显像并可测定分肾肾小球流过率(GFR);②静脉肾盂造影:也可大致了解分侧肾功能;③其他:采用肾动脉或输尿管插管方法检查单个肾脏功能等。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肾" }, { "start_idx": 16, "end_idx": 24, "type": "pro", "entity": "双肾放射性核素检查" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "肾" }, { "start_idx": 35, "end_idx": 42, "type": "ite", "entity": "分肾肾小球流过率" }, { "start_idx": 44, "end_idx": 46, "type": "ite", "entity": "GFR" }, { "start_idx": 50, "end_idx": 55, "type": "pro", "entity": "静脉肾盂造影" }, { "start_idx": 65, "end_idx": 65, "type": "bod", "entity": "肾" }, { "start_idx": 75, "end_idx": 85, "type": "pro", "entity": "肾动脉或输尿管插管方法" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "肾脏" } ] }, { "text": "参考文献1.严勤.主动脉狭窄//丁文祥,苏肇伉.小儿心脏外科学.济南:山东科技出版社,2000:2862.王惠玲.冠状动脉畸形//杨思源.小儿心脏病学.第2版.北京:人民卫生出版社,1994:2903.朱铭,王荣发,高伟.冠状血管畸形.见:周爱卿主编.心导管术-先天性心脏病的诊断与治疗.山东:山东科技出版社,1997.4164.BalzerDT,SprayTL,McMufflinD,etal.Endarteritisassociatedwithaclinicallysilentpatentductusarteriosus.AmHeartJ,1993,125:1192-11925.BezerraAJ,DiDioLJ,PratesJC,etal.Variationsoftheareaandshapeoftheleftatrioventricularvalveanditscuspsandleaflets.SurgRadiolAnat,1994,16(3):277-2776.BilkisAA,AlwiM,HasriS,etal.TheAmplatzerductoccluder:Experiencein209patients.JAmCollCardiol,2001,37:258-2617.BurnJ,BrennanP,LitteJ,etal.Recurrenceriskinoffspringofadultswithmajorheartdefect:resultsfromfirstcohortofBritishcollaborativestudy.Lancet,1998,351:311-3118.CheungYF,ClementSWChiu,TCYung,etal.Impactofpreoperativeaorticcuspprolapseonlongtermoutcomeaftersurgicalclosureofsubarterialventricularseptaldefect.AnnThoracSurg,2002,73:622-6279.CobanogluA,MenasheVD.Totalanomalouspulmonaryvenousconnectioninneonatesandyounginfants:repairinthecurrentera.AnnThoracSurg,1993,55:43-4810.CopeJT,BanksD,McDanielNL,etal.Isverticalveinligationnecessaryinrepairoftotalanomalouspulmonaryvenousconnection?", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "主动脉狭窄" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "心脏" }, { "start_idx": 28, "end_idx": 29, "type": "dep", "entity": "外科" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "冠状动脉畸形" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "小儿心脏病" }, { "start_idx": 111, "end_idx": 116, "type": "dis", "entity": "冠状血管畸形" }, { "start_idx": 126, "end_idx": 128, "type": "bod", "entity": "心导管" }, { "start_idx": 131, "end_idx": 136, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "五、肺中叶综合征肺中叶综合征(middlelobesyndrome)以肺中叶局限性慢性炎症和肺不张为特征。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "肺中叶综合征" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "肺中叶综合征" }, { "start_idx": 15, "end_idx": 32, "type": "dis", "entity": "middlelobesyndrome" }, { "start_idx": 35, "end_idx": 44, "type": "dis", "entity": "肺中叶局限性慢性炎症" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "肺不张" } ] }, { "text": "绝大多数发生在右侧,故又称右肺中叶综合征。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "右侧" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "右肺中叶综合征" } ] }, { "text": "主要病因为非特异性感染,如反复发作的亚急性或慢性中叶肺炎、支气管黏膜炎症狭窄或闭塞、痰栓堵塞;或支气管淋巴结肿大、尤其是肺门结核压迫导致支气管狭窄,发生阻塞性肺不张。", "entities": [ { "start_idx": 18, "end_idx": 27, "type": "dis", "entity": "亚急性或慢性中叶肺炎" }, { "start_idx": 29, "end_idx": 37, "type": "dis", "entity": "支气管黏膜炎症狭窄" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "闭塞" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "痰栓堵塞" }, { "start_idx": 48, "end_idx": 55, "type": "dis", "entity": "支气管淋巴结肿大" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "肺门结核压迫" }, { "start_idx": 68, "end_idx": 72, "type": "dis", "entity": "支气管狭窄" }, { "start_idx": 76, "end_idx": 81, "type": "dis", "entity": "阻塞性肺不张" } ] }, { "text": "部分可发生于哮喘急性发作或急性哮喘性支气管炎。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "哮喘急性发作" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "急性哮喘性支气管炎" } ] }, { "text": "主要症状为长期反复咳嗽、咳黏液痰或脓痰、呼吸困难、发热、胸痛、喘息等,重者有发绀,偶有咯血。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "长期反复咳嗽" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "咳黏液痰" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "脓痰" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "发热" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "胸痛" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "喘息" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "发绀" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "咯血" } ] }, { "text": "肺部可闻干湿性啰音及哮鸣音。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 3, "end_idx": 12, "type": "sym", "entity": "闻干湿性啰音及哮鸣音" } ] }, { "text": "右肺中叶部位叩诊浊音、呼吸音减弱,少数有杵状指(趾)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "右肺中叶部位" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "叩诊浊音" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "呼吸音减弱" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "杵状指(趾)" } ] }, { "text": "重症者可发生肺纤维化与囊性支气管扩张。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "肺纤维化" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "囊性支气管扩张" } ] }, { "text": "胸片可见三角形均匀一致的致密阴影,其基底与右心缘重叠,右横膈前方上抬。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "胸片" }, { "start_idx": 4, "end_idx": 15, "type": "sym", "entity": "三角形均匀一致的致密阴影" }, { "start_idx": 18, "end_idx": 25, "type": "sym", "entity": "基底与右心缘重叠" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "右横膈前方上抬" } ] }, { "text": "侧位片中叶呈狭窄的梭形阴影,尖端指向肺门,中叶胸膜多与胸壁粘连,少数与横膈粘连。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "侧位片中叶" }, { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "狭窄的梭形阴影" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "尖端指向肺门" }, { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "中叶胸膜多与胸壁粘连" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "少数与横膈粘连" } ] }, { "text": "叶间裂下移、斜裂上移。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "叶间裂下移" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "斜裂上移" } ] }, { "text": "支气管造影可见中叶支气管狭窄、充盈缺损及远端支气管扩张。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "支气管造影" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "中叶支气管狭窄" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "充盈缺损及远端支气管扩张" } ] }, { "text": "支气管镜检查:中叶支气管有狭窄、充血、炎症水肿、黏液栓塞和肉芽肿等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "支气管镜检查" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "中叶支气管有狭窄" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "充血" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "黏液栓塞和肉芽肿" } ] }, { "text": "选用有效抗生素控制感染和体位引流痰液,解除支气管狭窄。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "抗生素" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "感染" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "体位引流痰液" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "支气管狭窄" } ] }, { "text": "必要时进行纤维支气管镜下灌洗。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "pro", "entity": "纤维支气管镜下灌洗" } ] }, { "text": "如内科治疗数月无效,肺炎仍反复发作,且病情严重,中叶肺组织破坏严重,肺功能严重损害的不可逆病例,应在控制感染的基础上,手术切除病肺。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肺炎" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "中叶肺组织" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肺" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "手术" } ] }, { "text": "第二节非感染性口炎一、创伤性口炎机械性或热性刺激可能是此病的主要发病条件。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "非感染性口炎" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "创伤性口炎" } ] }, { "text": "锐利的牙根、残冠,口腔异物,较硬橡皮奶头等机械性因素均可造成黏膜撕裂伤、出血、溃疡或糜烂;过烫的饮料、茶水或食物则引起黏膜烫伤。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "牙根" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "残冠" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "黏膜撕裂伤" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "出血" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "溃疡或糜烂" } ] }, { "text": "治疗为去除病因如拔去残根,磨改锐利牙齿或边缘。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "去除病因如拔去残根" }, { "start_idx": 13, "end_idx": 21, "type": "pro", "entity": "磨改锐利牙齿或边缘" } ] }, { "text": "冰硼散、锡类散及青黛散可局部消炎止痛。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "冰硼散" }, { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "锡类散" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "青黛散" } ] }, { "text": "药物漱口水含漱,多喝凉开水以清洁口腔。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "药物漱口水含漱" }, { "start_idx": 8, "end_idx": 17, "type": "pro", "entity": "多喝凉开水以清洁口腔" } ] }, { "text": "三、血小板释放功能缺陷性疾患血小板释放功能缺陷是一组异质性很大的疾患,也是遗传性疾患,遗传方式有些至今尚未完全清楚。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dis", "entity": "血小板释放功能缺陷性疾患" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "血小板释放功能缺陷" } ] }, { "text": "临床上表现轻-中度的出血,如鼻出血、瘀斑、月经及分娩后出血过多,拔牙及扁桃体摘除等手术后过度出血等,偶有严重出血致死者。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "出血" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "鼻出血" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "瘀斑" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "扁桃体" } ] }, { "text": "实验室检查出血时间大多延长;血小板计数正常或轻度减少,形态正常;凝血因子正常,血小板黏附可降低。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "血小板" }, { "start_idx": 14, "end_idx": 25, "type": "sym", "entity": "血小板计数正常或轻度减少" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "血小板" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "血小板黏附可降低" } ] }, { "text": "提示本组疾患诊断的关键是血小板对ADP或肾上腺素的聚集试验第一波正常,第二波显著降低或缺如;对胶原的聚集亦降低,但在高浓度下正常。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "血小板" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 37, "end_idx": 44, "type": "sym", "entity": "波显著降低或缺如" }, { "start_idx": 46, "end_idx": 54, "type": "sym", "entity": "对胶原的聚集亦降低" } ] }, { "text": "本组疾患进一步又分为两大类,一是储存池病(storagepooldisease,SPD),二是血小板释放障碍性疾患。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "储存池病" }, { "start_idx": 21, "end_idx": 38, "type": "dis", "entity": "storagepooldisease" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "SPD" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "血小板" }, { "start_idx": 47, "end_idx": 56, "type": "sym", "entity": "血小板释放障碍性疾患" } ] }, { "text": "前者为血小板的致密颗粒、α或δ分别或者联合缺乏电镜;后者为血小板内容物正常,但释放障碍,包括花生四烯酸释放缺陷、环氧化酶缺乏症,血栓素A2合成酶缺乏症等。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "血小板" }, { "start_idx": 3, "end_idx": 22, "type": "sym", "entity": "血小板的致密颗粒、α或δ分别或者联合缺乏" }, { "start_idx": 23, "end_idx": 24, "type": "equ", "entity": "电镜" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "血小板" }, { "start_idx": 46, "end_idx": 54, "type": "sym", "entity": "花生四烯酸释放缺陷" }, { "start_idx": 56, "end_idx": 62, "type": "dis", "entity": "环氧化酶缺乏症" }, { "start_idx": 64, "end_idx": 80, "type": "dis", "entity": "血栓素A2合成酶缺乏症" } ] }, { "text": "最后诊断时先要排除继发性血小板释放功能障碍,然后借助检测血小板颗粒内容物、电镜等手段以确诊。", "entities": [ { "start_idx": 9, "end_idx": 20, "type": "dis", "entity": "继发性血小板释放功能障碍" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "血小板" }, { "start_idx": 37, "end_idx": 38, "type": "equ", "entity": "电镜" } ] }, { "text": "我们将重点讨论储存池病。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "储存池病" } ] }, { "text": "(一)δ储存池病(δ-SPD)又称原发性致密体缺乏症。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "δ储存池病" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "δ-SPD" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "原发性致密体缺乏症" } ] }, { "text": "其基本缺陷是血小板的致密颗粒内含物ATP、ADP、钙离子、焦磷酸盐、5-羟色胺等减少,其中ADP的减少较ATP更为显著。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "血小板" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "ATP" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "ADP" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "钙离子" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "焦磷酸盐" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "5-羟色胺" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "ADP的减少" } ] }, { "text": "δ-SPD的血小板对ADP或肾上腺素的诱导缺乏二相聚集波,胶原诱导的聚集反应降低或缺失,花生四烯酸引起聚集反应亦降低,电镜下可见致密颗粒减少。", "entities": [ { "start_idx": 29, "end_idx": 42, "type": "sym", "entity": "胶原诱导的聚集反应降低或缺失" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "花生四烯酸" }, { "start_idx": 44, "end_idx": 57, "type": "sym", "entity": "花生四烯酸引起聚集反应亦降低" }, { "start_idx": 59, "end_idx": 60, "type": "equ", "entity": "电镜" }, { "start_idx": 64, "end_idx": 69, "type": "sym", "entity": "致密颗粒减少" } ] }, { "text": "α及δ颗粒同时发生缺陷者称为α、δ储存池病(αδ-SPD)其中δ颗粒内容物的减少往往更严重,α颗粒内容物的减少为轻到中度,临床及实验室检查类似δ-SPD。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "α、δ储存池病" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "αδ-SPD" } ] }, { "text": "致密体缺陷又可是其他遗传性疾病的一部分,如Hermansky-Pudlak综合征、Chediak-Higashi综合征、Wiskott-Aldrich综合征、成骨不全及血小板减少伴桡骨缺如综合征(TAR综合征)等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "致密体缺陷" }, { "start_idx": 21, "end_idx": 39, "type": "dis", "entity": "Hermansky-Pudlak综合征" }, { "start_idx": 41, "end_idx": 58, "type": "dis", "entity": "Chediak-Higashi综合征" }, { "start_idx": 60, "end_idx": 77, "type": "dis", "entity": "Wiskott-Aldrich综合征" }, { "start_idx": 79, "end_idx": 96, "type": "dis", "entity": "成骨不全及血小板减少伴桡骨缺如综合征" }, { "start_idx": 98, "end_idx": 103, "type": "dis", "entity": "TAR综合征" } ] }, { "text": "1.Hermansky-Pudlak综合征本病属常染色体隐性遗传,特点是眼与皮肤的白化症、单核-巨噬系统内脂质样物质积聚和血小板功能缺陷性出血倾向。", "entities": [ { "start_idx": 2, "end_idx": 20, "type": "dis", "entity": "Hermansky-Pudlak综合征" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "白化症" }, { "start_idx": 45, "end_idx": 51, "type": "bod", "entity": "单核-巨噬系统" }, { "start_idx": 45, "end_idx": 59, "type": "sym", "entity": "单核-巨噬系统内脂质样物质积聚" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "血小板" }, { "start_idx": 61, "end_idx": 72, "type": "sym", "entity": "血小板功能缺陷性出血倾向" } ] }, { "text": "血小板中致密体减少或实际缺如,由于5-羟色胺、钙离子和腺嘌呤核苷酸水平很低,因此血小板对ADP、肾上腺素和凝血酶的诱导缺乏二相聚集波。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "血小板中致密体减少或实际缺如" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "5-羟色胺" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "钙离子" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "腺嘌呤核苷酸" }, { "start_idx": 40, "end_idx": 46, "type": "bod", "entity": "血小板对ADP" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "凝血酶" } ] }, { "text": "2.Chediak-Higashi综合征本病属常染色体隐性遗传,特点是中性粒细胞、单核细胞、成纤维细胞和黑色素细胞中常有异常颗粒形成,对细菌和真菌的易感性增高。", "entities": [ { "start_idx": 2, "end_idx": 19, "type": "dis", "entity": "Chediak-Higashi综合征" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "单核细胞" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "成纤维细胞" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "黑色素细胞" }, { "start_idx": 68, "end_idx": 69, "type": "mic", "entity": "细菌" }, { "start_idx": 71, "end_idx": 72, "type": "mic", "entity": "真菌" } ] }, { "text": "临床常有眼和皮肤的白化病,出血症较轻,检查粒细胞中异常颗粒常可帮助确诊。", "entities": [ { "start_idx": 4, "end_idx": 28, "type": "sym", "entity": "眼和皮肤的白化病,出血症较轻,检查粒细胞中异常颗粒" } ] }, { "text": "3.Wiskott-Aldrich综合征本病属性联隐性遗传疾病,特点是细胞免疫缺陷引起的反复感染、湿疹和血小板减少性出血。", "entities": [ { "start_idx": 2, "end_idx": 19, "type": "dis", "entity": "Wiskott-Aldrich综合征" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "细胞" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "湿疹" }, { "start_idx": 52, "end_idx": 59, "type": "sym", "entity": "血小板减少性出血" } ] }, { "text": "4.TAR综合征本病属常染色体隐性遗传,患儿有多发性畸形如骨骼、心、肾等,多见双侧桡骨缺如。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "TAR综合征" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "骨骼" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "心" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肾" } ] }, { "text": "血小板常减少伴骨髓巨核细胞数量减少或缺如。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "巨核细胞" } ] }, { "text": "(二)α储存池病α储存池(α-SPD)又名灰色血小板综合征,这是因为瑞氏染色涂片上的血小板呈灰色的缘故。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "α储存池病" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "α储存池" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "α-SPD" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "灰色血小板综合征" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "血小板" } ] }, { "text": "电镜观察发现患者的巨核细胞及血小板特异性缺乏α颗粒。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "电镜" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "巨核细胞" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "血小板" } ] }, { "text": "生化研究发现病人血小板α颗粒蛋白质如血小板第四因子(PF4)、血小板球蛋白(β-TG)vWF、血小板凝血酶原敏感蛋白、纤维连接蛋白、因子Ⅴ、高分子量的激肽原、血小板生长因子等降低或缺乏。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "PF4" }, { "start_idx": 31, "end_idx": 36, "type": "bod", "entity": "血小板球蛋白" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "β-TG" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "vWF" }, { "start_idx": 47, "end_idx": 57, "type": "bod", "entity": "血小板凝血酶原敏感蛋白" }, { "start_idx": 59, "end_idx": 64, "type": "bod", "entity": "纤维连接蛋白" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "因子Ⅴ" }, { "start_idx": 70, "end_idx": 77, "type": "bod", "entity": "高分子量的激肽原" }, { "start_idx": 79, "end_idx": 85, "type": "bod", "entity": "血小板生长因子" }, { "start_idx": 79, "end_idx": 91, "type": "sym", "entity": "血小板生长因子等降低或缺乏" } ] }, { "text": "进一步研究揭示α颗粒蛋白合成正常,但储存障碍;本病同时还兼有传导缺陷。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "α颗粒蛋白" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "储存障碍" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "传导缺陷" } ] }, { "text": "病人有轻度的血小板数降低,出血时间延长和出血倾向,血小板对凝血酶介导的聚集和释放反应异常,但对胶原和ADP诱导的聚集反应不变。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "血小板" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "血小板数降低" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "出血时间延长" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "血小板" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "凝血酶" }, { "start_idx": 25, "end_idx": 43, "type": "sym", "entity": "血小板对凝血酶介导的聚集和释放反应异常" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "胶原" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "ADP" } ] }, { "text": "瑞氏染色血片上的灰色血小板常可提示诊断,确诊多依赖电镜观察结果。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "瑞氏染色血片" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "血小板" }, { "start_idx": 25, "end_idx": 26, "type": "equ", "entity": "电镜" } ] }, { "text": "第三节肠旋转不良小肠旋转及固定异常是宫内小肠发育过程受扰而致的解剖学异常,可引发肠梗阻或肠扭转,并可威胁生命,大多在婴儿及儿童期出现症状。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "小肠旋转" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "固定异常" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "小肠" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "肠扭转" } ] }, { "text": "了解小肠胚胎发育对认识及手术治疗该病具有重要意义。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "小肠" } ] }, { "text": "确定中肠旋转及固定异常的具体发病率相当困难,有报道称占存活婴儿的1/6000。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "中肠旋转" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "固定异常" } ] }, { "text": "尸解发现有0.5%存在完全性肠旋转不良,即所有小肠位于腹腔右侧,结肠位于左侧。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "小肠" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "腹腔" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "结肠" } ] }, { "text": "胃肠道钡剂造影检查偶尔发现的无症状发病率为0.2%,见于任何年龄。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "胃肠道钡剂造影" } ] }, { "text": "【胚胎学】孕早期时,胚胎中肠发育快于体腔发育,因此,胎儿发育至第4周时,中肠通常凸出腹腔,形成脐疝;孕10周左右,肠段回纳入腹腔,逐步完成旋转和固定,直至最终形成足月儿形态的小肠与结肠。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "胚胎中肠" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "体腔" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "中肠" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "腹腔" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "脐疝" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "肠段" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "腹腔" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "小肠" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "结肠" } ] }, { "text": "若中肠在旋转过程中受扰,生后即可能出现相应的临床症状。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "中肠" } ] }, { "text": "中肠发育分为3个阶段。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "中肠" } ] }, { "text": "(一)第一阶段脐疝形成孕4周,胎儿中肠开始向腹侧弯曲突起,进入体蒂腔,形成疝。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "脐疝" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "中肠" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "体蒂腔" }, { "start_idx": 37, "end_idx": 37, "type": "dis", "entity": "疝" } ] }, { "text": "疝中轴为肠系膜上动脉(SMA)(图13-13A),并以此为标志,将中肠分为头侧段(动脉前,十二指肠空肠段)与尾侧段(动脉后,盲结肠段)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "疝中轴" }, { "start_idx": 4, "end_idx": 9, "type": "bod", "entity": "肠系膜上动脉" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "SMA" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "中肠" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "动脉" }, { "start_idx": 45, "end_idx": 51, "type": "bod", "entity": "十二指肠空肠段" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "动脉" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "盲结肠段" } ] }, { "text": "第一阶段中肠头侧段发育快于尾侧段。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "中肠" } ] }, { "text": "1.头侧段的发育中肠疝形成并在体腔外继续生长过程中,头侧段被发育中的肝脏与左脐静脉推向下方,逆时针旋转90°至SMA右侧(图13-13B);随着肠腔进一步发育,于第二阶段开始前,头侧段再旋转90°至SMA后方,共计旋转180°(图13-13C)。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "中肠疝" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "体腔" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肝脏" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "左脐静脉" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "SMA" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "肠腔" }, { "start_idx": 99, "end_idx": 101, "type": "bod", "entity": "SMA" } ] }, { "text": "2.尾侧段的发育第一阶段,中肠尾侧段与头侧段平行旋转,回盲部最初位于SMA下方(图13-13A),与头侧段首次90°旋转到达SMA右侧同步,回盲部逆时针旋转90°至SMA左侧(图13-13B)。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "中肠" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "头侧段" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "回盲部" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "SMA" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "头侧段" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "SMA" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "回盲部" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "SMA" } ] }, { "text": "在第10周回纳入腹腔前,回盲部继续旋转90°,此时恰位于SMA腹侧(图13-13C)。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "腹腔" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "回盲部" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "SMA" } ] }, { "text": "(二)第二阶段中肠回纳入腹1.头侧段的发育肠段继续发育,于孕10周(此时胚胎长约40mm)开始返回腹腔,至孕11周完成。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "中肠" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肠段" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "腹腔" } ] }, { "text": "头侧段首先回纳,期间绕SMA继续旋转90°,共计逆时针旋转270°。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "头侧段" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "SMA" } ] }, { "text": "最终,十二指肠空肠连接部经屈氏韧带固定于SMA左侧的后腹壁(图13-4A)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "十二指肠" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "空肠" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "屈氏韧带" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "SMA" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "后腹壁" } ] }, { "text": "2.尾侧段的发育在盲结肠回纳入腹腔过程中,也继续旋转90°至SMA右侧,共计绕SMA逆时针旋转270°(13-4B)。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "盲结肠" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "腹腔" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "SMA" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "SMA" } ] }, { "text": "(三)第三阶段中肠固定从孕12周至出生,如中肠正常旋转,则结肠逐渐完成固定,腹膜形成束带样组织即升、降结肠系膜附着于后腹壁。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "中肠" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "中肠" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "结肠" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "腹膜" }, { "start_idx": 42, "end_idx": 46, "type": "bod", "entity": "束带样组织" }, { "start_idx": 48, "end_idx": 54, "type": "bod", "entity": "升、降结肠系膜" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "后腹壁" } ] }, { "text": "如盲肠和升结肠旋转不彻底,未达到右侧腹,腹膜束带仍会把升结肠连至右结肠沟,此时束带横跨于十二指肠腹侧,压迫肠段造成梗阻。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "盲肠" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "升结肠" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "腹膜束带" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "升结肠" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "右结肠沟" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "束带" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "十二指肠" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "肠段" } ] }, { "text": "【旋转不良分型】旋转不良的解剖异常主要还是根据头侧段和尾侧段来分型,固定异常通常只发生于盲结肠部。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "旋转不良" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "旋转不良" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "盲结肠部" } ] }, { "text": "(一)完全不旋转临床上最为常见的是中肠完全不旋转,即头侧段和尾侧段均不旋转(图13-13)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "完全不旋转" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "中肠完全不旋转" } ] }, { "text": "正常情况下,十二指肠旋转至SMA后方,屈氏韧带恰位于中线左侧胃窦水平。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "十二指肠" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "SMA" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "屈氏韧带" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "胃窦" } ] }, { "text": "如果中肠不旋转,十二指肠长度变短,外观呈螺旋状,完全位于中线右侧,导致十二指肠不全梗阻;而肠段不固定,使中肠易发生扭转。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "中肠不旋转" }, { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "十二指肠长度变短" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "外观呈螺旋状" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "完全位于中线右侧" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "十二指肠不全梗阻" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "肠段不固定" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "中肠易发生扭转" } ] }, { "text": "十二指肠和结肠的肠系膜包绕SMA彼此融合成一系膜蒂,中肠即以此为轴发生扭转。", "entities": [ { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "十二指肠和结肠的肠系膜包绕SMA彼此融合成一系膜蒂" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "中肠" } ] }, { "text": "正常情况下,小肠系膜附着的基底部很宽,从左上腹屈氏韧带直至右下腹回盲部,小肠及系膜一般不可能发生轴性扭转;若近端空肠和远端回肠均位于中腹部,系膜附着部相对较窄,则扭转的可能性大大增加。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "小肠系膜" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "左上腹屈氏韧带" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "右下腹回盲部" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "小肠" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "系膜" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "近端空肠" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "远端回肠" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "中腹部" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "系膜" } ] }, { "text": "图13-2中肠头侧段旋转肠系膜上动脉(SMA)从主动脉右侧角发出,圆盘代表中肠头侧段绕SMA旋转的角度(A)十二指肠空肠段发生时(胚胎5mm大)位于SMA下面或0°;(B)在胚胎10mm时,十二指肠空肠段绕SMA逆时针旋转90°;(C)到胚胎40mm大时,十二指肠空肠段继续旋转,总共旋转180°图13-3中肠尾侧段旋转肠系膜上动脉(SMA)从主动脉右侧角发出,圆盘代表中肠尾侧段绕SMA旋转的角度(A)盲肠发生时(胚胎5mm大)位于SMA下面或0°;(B)在胚胎10mm时,盲肠绕SMA逆时针旋转90°;(C)到胚胎40mm大时,盲肠继续旋转,总共旋转180°,位于SMA腹侧图13-4胚胎时的中肠旋转完成(A)十二指肠空肠连接部的最终位置。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "中肠头侧段旋转" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "肠系膜上动脉" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "SMA" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "主动脉" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "SMA" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "十二指肠" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "肠段" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "SMA" }, { "start_idx": 95, "end_idx": 101, "type": "bod", "entity": "十二指肠空肠段" }, { "start_idx": 103, "end_idx": 105, "type": "bod", "entity": "SMA" }, { "start_idx": 128, "end_idx": 134, "type": "bod", "entity": "十二指肠空肠段" }, { "start_idx": 153, "end_idx": 159, "type": "dis", "entity": "中肠尾侧段旋转" }, { "start_idx": 160, "end_idx": 165, "type": "bod", "entity": "肠系膜上动脉" }, { "start_idx": 167, "end_idx": 169, "type": "bod", "entity": "SMA" }, { "start_idx": 172, "end_idx": 174, "type": "bod", "entity": "主动脉" }, { "start_idx": 185, "end_idx": 189, "type": "bod", "entity": "中肠尾侧段" }, { "start_idx": 191, "end_idx": 193, "type": "bod", "entity": "SMA" }, { "start_idx": 202, "end_idx": 203, "type": "bod", "entity": "盲肠" }, { "start_idx": 217, "end_idx": 219, "type": "bod", "entity": "SMA" }, { "start_idx": 238, "end_idx": 239, "type": "bod", "entity": "盲肠" }, { "start_idx": 241, "end_idx": 243, "type": "bod", "entity": "SMA" }, { "start_idx": 266, "end_idx": 267, "type": "bod", "entity": "盲肠" }, { "start_idx": 284, "end_idx": 286, "type": "bod", "entity": "SMA" }, { "start_idx": 298, "end_idx": 299, "type": "bod", "entity": "中肠" }, { "start_idx": 307, "end_idx": 312, "type": "bod", "entity": "十二指肠空肠" } ] }, { "text": "它发生时位于SMA腹侧,在逆时针旋转270°后,位于SMA左侧;(B)盲肠的最终位置。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "SMA" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "SMA" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "盲肠" } ] }, { "text": "它发生时位于SMA的背侧,在绕SMA逆时针旋转270°后位于SMA右侧图13-5中肠完全不旋转十二指肠空肠连接部和盲肠都不旋转。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "SMA" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "SMA" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "SMA" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "中肠完全不旋转" }, { "start_idx": 47, "end_idx": 62, "type": "sym", "entity": "十二指肠空肠连接部和盲肠都不旋转" } ] }, { "text": "所有的小肠都位于SMA的右侧,而结肠位于SMA的左侧。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "所有的小肠都位于SMA的右侧" }, { "start_idx": 16, "end_idx": 25, "type": "sym", "entity": "结肠位于SMA的左侧" } ] }, { "text": "这种类型在肠旋转不良中最为常见,常可并发肠扭转(二)头侧段旋转异常如果只是头侧段不旋转,而尾侧段旋转和固定正常,亦可因结肠系膜束带的压迫,导致十二指肠梗阻(图13-6)。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "肠扭转" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "头侧段旋转异常" }, { "start_idx": 59, "end_idx": 64, "type": "bod", "entity": "结肠系膜束带" }, { "start_idx": 71, "end_idx": 76, "type": "dis", "entity": "十二指肠梗阻" } ] }, { "text": "但由于十二指肠空肠连接部至回盲部之间的系膜附着部仍相对较宽广,中肠扭转的可能性较小。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "bod", "entity": "十二指肠空肠连接部" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "回盲部" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "系膜附着部" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "中肠" } ] }, { "text": "(三)头侧段反方向旋转导致十二指肠位于SMA前面(正常应位于后方);而尾侧段的逆向旋转则导致横结肠位于SAM后方(图13-13),出现结肠梗阻。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "头侧段反方向旋转" }, { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "十二指肠位于SMA前面" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "横结肠" }, { "start_idx": 67, "end_idx": 70, "type": "dis", "entity": "结肠梗阻" } ] }, { "text": "若尾侧段正常旋转,随着升结肠从左上腹旋转至右下腹,其系膜跨越SMA前方,覆盖头侧段发育而来的小肠,形成疝囊,称十二指肠旁疝(图13-8)。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "升结肠" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "左上腹" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "右下腹" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "系膜" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "SMA" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "小肠" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "疝囊" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "十二指肠旁疝" } ] }, { "text": "(四)头侧段不完全旋转导致十二指肠空肠连接部(屈氏韧带)位置低于其左上腹的正常位置;而尾侧段异常旋转可致中肠扭转。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "头侧段不完全旋转" }, { "start_idx": 13, "end_idx": 40, "type": "sym", "entity": "十二指肠空肠连接部(屈氏韧带)位置低于其左上腹的正常位置" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "中肠扭转" } ] }, { "text": "此外,区分头侧段不旋转或不完全旋转,并无客观标准,一般认为,只要屈氏韧带位于腹部中线右侧,即为不旋转。", "entities": [ { "start_idx": 5, "end_idx": 16, "type": "dis", "entity": "头侧段不旋转或不完全旋转" }, { "start_idx": 32, "end_idx": 43, "type": "sym", "entity": "屈氏韧带位于腹部中线右侧" } ] }, { "text": "(五)尾侧段的旋转异常如果头侧段旋转正常,但尾侧段旋转异常,亦可引起中肠扭转。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "尾侧段的旋转异常" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "中肠扭转" } ] }, { "text": "与完全不旋转相同,此时屈氏韧带与回盲部之间的肠系膜根部附着处很窄。", "entities": [ { "start_idx": 11, "end_idx": 31, "type": "sym", "entity": "屈氏韧带与回盲部之间的肠系膜根部附着处很窄" } ] }, { "text": "(六)尾侧段不完全旋转可能导致结肠固定异常,回盲部的不完全固定可致盲肠扭转;而结肠肝曲固定不全时,则有结肠延伸至右上后腹壁的腹膜束带形成,导致十二指肠部分梗阻。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "尾侧段不完全旋转" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "结肠固定异常" }, { "start_idx": 22, "end_idx": 30, "type": "dis", "entity": "回盲部的不完全固定" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "盲肠扭转" }, { "start_idx": 39, "end_idx": 46, "type": "dis", "entity": "结肠肝曲固定不全" }, { "start_idx": 51, "end_idx": 67, "type": "sym", "entity": "结肠延伸至右上后腹壁的腹膜束带形成" }, { "start_idx": 71, "end_idx": 78, "type": "dis", "entity": "十二指肠部分梗阻" } ] }, { "text": "图13-6十二指肠空肠连接部未旋转而盲肠正常旋转。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "十二指肠" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "空肠" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "盲肠" } ] }, { "text": "在临床上常因结肠系膜束带压迫引起十二指肠梗阻图13-7十二指肠空肠连接部反向旋转,位于SMA腹侧,同时结肠反向旋转,位于SMA背侧。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "结肠" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "十二指肠梗阻" }, { "start_idx": 27, "end_idx": 35, "type": "bod", "entity": "十二指肠空肠连接部" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "SMA" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "结肠" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "SMA" } ] }, { "text": "临床上常引起横结肠梗阻图13-8(A)十二指肠空肠连接部的反向旋转(横过SMA腹侧),但结肠旋转正常;(B)十二指肠旁疝【伴发畸形】中肠不旋转或旋转不全往往是先天性膈疝和腹壁缺损——脐膨出及腹裂的重要并发症。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "横结肠梗阻" }, { "start_idx": 19, "end_idx": 27, "type": "bod", "entity": "十二指肠空肠连接部" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "SMA" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "结肠" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "十二指肠旁疝" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "畸形" }, { "start_idx": 66, "end_idx": 75, "type": "dis", "entity": "中肠不旋转或旋转不全" }, { "start_idx": 79, "end_idx": 83, "type": "dis", "entity": "先天性膈疝" }, { "start_idx": 85, "end_idx": 88, "type": "dis", "entity": "腹壁缺损" }, { "start_idx": 91, "end_idx": 93, "type": "dis", "entity": "脐膨出" }, { "start_idx": 95, "end_idx": 96, "type": "dis", "entity": "腹裂" } ] }, { "text": "有报道称:30%~62%旋转不良患儿有伴发畸形,大多为消化道畸形。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "旋转不良" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "畸形" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "消化道畸形" } ] }, { "text": "50%十二指肠闭锁患儿、1/3空回肠闭锁患者伴有肠旋转不良,原因之一是宫内肠扭转使肠系膜血供受阻,导致肠闭锁。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "十二指肠闭锁" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "空回肠闭锁" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "宫内肠" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "肠系膜" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "肠闭锁" } ] }, { "text": "其他畸形包括:梅克尔憩室、十二指肠瓣膜或狭窄、巨结肠、肛门闭锁、食管闭锁合并食管气管瘘、先天性短肠、胆道闭锁、先天性心脏病、内脏反位、肠系膜囊肿和梨状腹综合征。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "畸形" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "梅克尔憩室" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "十二指肠瓣膜" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "狭窄" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "肛门闭锁" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "食管闭锁" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "食管气管瘘" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "先天性短肠" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "胆道闭锁" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "内脏反位" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "肠系膜囊肿" }, { "start_idx": 73, "end_idx": 78, "type": "dis", "entity": "梨状腹综合征" } ] }, { "text": "有报道发现家族性肠旋转不良、伴发颜面或肢体异常,提示该病可能与遗传有关。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "颜面或肢体异常" } ] }, { "text": "【临床表现】旋转不良可表现为:①中肠扭转;②十二指肠不全或完全梗阻,胆汁性呕吐;③间歇性或长期腹痛,间歇性腹泻或便血;④偶然发现的无症状者。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "旋转不良" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "中肠扭转" }, { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "十二指肠不全或完全梗阻" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "胆汁性呕吐" }, { "start_idx": 41, "end_idx": 48, "type": "sym", "entity": "间歇性或长期腹痛" }, { "start_idx": 50, "end_idx": 57, "type": "sym", "entity": "间歇性腹泻或便血" } ] }, { "text": "(一)中肠扭转中肠扭转(midgutvolvulus)多见于婴儿及儿童,但也可发生在其他年龄段,甚至成年后。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "中肠扭转" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "中肠扭转" }, { "start_idx": 12, "end_idx": 25, "type": "dis", "entity": "midgutvolvulus" } ] }, { "text": "系外科急诊,如果未能及时手术纠治,可因小肠坏死导致短肠与死亡。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "小肠坏死" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "短肠" } ] }, { "text": "典型症状为新生儿突发胆汁性呕吐,呕吐尚与十二指肠折叠成角及腹膜束带压迫导致十二指肠梗阻有关。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "呕吐" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "呕吐" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "十二指肠" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "腹膜" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "十二指肠" } ] }, { "text": "肠梗阻、败血症及出血性颅高压也可引起胆汁性呕吐,需快速做出鉴别诊断。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "败血症" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "出血性颅高压" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "胆汁性呕吐" } ] }, { "text": "一旦出现肠坏死,生存机会大大减少,保留足够长度小肠亦极度困难。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "肠坏死" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "小肠" } ] }, { "text": "所以一旦发现存在旋转不良,即应手术纠治。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "旋转不良" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "手术" } ] }, { "text": "中肠扭转也可出现间歇性的症状,主要见于年长患儿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "中肠扭转" } ] }, { "text": "包括慢性腹痛、间歇性呕吐(有时为非胆汁性)、厌食、体重下降、生长发育不良、肠道吸收障碍及腹泻等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "慢性腹痛" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "厌食" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "体重下降" }, { "start_idx": 30, "end_idx": 35, "type": "sym", "entity": "生长发育不良" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "肠道吸收障碍" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "腹泻" } ] }, { "text": "肠部分扭转者肠系膜静脉和淋巴回流受阻可致营养素吸收障碍、肠腔内蛋白质丢失;动脉供血不足致黏液缺血,出现黑便。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肠部分扭转" }, { "start_idx": 6, "end_idx": 26, "type": "sym", "entity": "肠系膜静脉和淋巴回流受阻可致营养素吸收障碍" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "肠腔内蛋白质丢失" }, { "start_idx": 37, "end_idx": 47, "type": "sym", "entity": "动脉供血不足致黏液缺血" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "黑便" } ] }, { "text": "(二)十二指肠梗阻十二指肠梗阻系头侧段的十二指肠空肠未绕SMA旋转或旋转不彻底,引起十二指肠折叠、扭曲,产生间歇性梗阻。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "十二指肠梗阻" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "十二指肠梗阻" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "十二指肠" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "SMA" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "十二指肠折叠" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "扭曲" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "产生间歇性梗阻" } ] }, { "text": "此外,从升结肠行经十二指肠腹侧至右上后腹壁的先天性束带可压迫十二指肠,引起梗阻。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "升结肠" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "十二指肠" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "后腹壁" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "十二指肠" } ] }, { "text": "典型症状为胆汁性呕吐(偶见非胆汁性)和腹痛,或二者兼有。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "胆汁性呕吐" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "腹痛" } ] }, { "text": "(三)间歇性慢性腹痛旋转不良者可出现间歇性或慢性腹痛,原因很多,且往往并存。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "间歇性慢性腹痛" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "旋转不良" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "慢性腹痛" } ] }, { "text": "间歇性肠扭转或其他原因所致肠梗阻均可引发肠腔扩张,产生痉挛性疼痛及呕吐;肠系膜静脉或淋巴系统部分或间断闭塞,可致肠壁、肠系膜及系膜淋巴结水肿,以上原因也可引起腹痛。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "间歇性肠扭转" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "肠腔扩张" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "痉挛性疼痛" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "呕吐" }, { "start_idx": 36, "end_idx": 52, "type": "sym", "entity": "肠系膜静脉或淋巴系统部分或间断闭塞" }, { "start_idx": 56, "end_idx": 69, "type": "sym", "entity": "肠壁、肠系膜及系膜淋巴结水肿" }, { "start_idx": 79, "end_idx": 80, "type": "sym", "entity": "腹痛" } ] }, { "text": "部分扭转亦可引发慢性动脉供血不足,导致腹泻、慢性腹痛及餐后加重的肠绞痛,或因黏液缺血出现黑便。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "慢性动脉供血不足" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "腹泻" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "慢性腹痛" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "餐后加重的肠绞痛" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "黑便" } ] }, { "text": "(四)无症状患者因其他疾病行腹部手术或上下消化道造影检查时偶可发现无症状肠旋转不良。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "腹部手术" }, { "start_idx": 19, "end_idx": 27, "type": "pro", "entity": "上下消化道造影检查" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "肠旋转不良" } ] }, { "text": "【诊断】凡怀疑旋转不良或中肠扭转,术前需行影像学检查,包括腹部平片、上下消化道造影,或两者兼之。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "旋转不良" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "中肠扭转" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "影像学检查" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "腹部平片" }, { "start_idx": 34, "end_idx": 40, "type": "pro", "entity": "上下消化道造影" } ] }, { "text": "(一)腹部平片每个有胆汁性呕吐的新生儿都应立即接受影像学检查,通常为前后直立位及侧卧位腹部平片。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "腹部平片" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "胆汁性呕吐" }, { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "影像学检查" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "腹部平片" } ] }, { "text": "婴儿呕吐胆汁样物,即使腹部平片表现正常,也不能排除肠旋转不良,需进一步检查明确诊断。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "呕吐胆汁样物" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "腹部平片" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "肠旋转不良" } ] }, { "text": "中肠扭转患者腹部平片肠腔充气可完全正常,也可表现为完全性小肠梗阻,包括多个充气扩张肠袢及液平,此时可能已有肠坏死。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "中肠扭转" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "腹部平片" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "完全性小肠梗阻" }, { "start_idx": 35, "end_idx": 45, "type": "sym", "entity": "多个充气扩张肠袢及液平" }, { "start_idx": 53, "end_idx": 55, "type": "sym", "entity": "肠坏死" } ] }, { "text": "若腹部平片提示肠梗阻,则无需进一步检查,应立即手术。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "腹部平片" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "肠梗阻" } ] }, { "text": "(二)上消化道造影当怀疑有肠旋转不良时,常规应行消化道造影检查,首选钡剂造影,也可选用水溶性造影剂。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "上消化道造影" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 24, "end_idx": 30, "type": "pro", "entity": "消化道造影检查" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "钡剂造影" }, { "start_idx": 43, "end_idx": 48, "type": "pro", "entity": "水溶性造影剂" } ] }, { "text": "经鼻胃管注入造影剂后,即可在透视下行动态观察,以获得更多有用信息。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "equ", "entity": "鼻胃管" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "造影剂" } ] }, { "text": "需要指出,怀疑急性肠扭转时不宜行此检查。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "急性肠扭转" } ] }, { "text": "如果肠旋转不良患儿未发生肠扭转,上消化道造影对确定十二指肠空肠连接部(屈氏韧带)位置具有十分重要的作用。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "肠扭转" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "上消化道造影" }, { "start_idx": 25, "end_idx": 33, "type": "bod", "entity": "十二指肠空肠连接部" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "韧带" } ] }, { "text": "正常情况下,该韧带应位于脊柱左侧胃窦水平,紧贴后腹壁。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "韧带" }, { "start_idx": 12, "end_idx": 19, "type": "bod", "entity": "脊柱左侧胃窦水平" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "后腹壁" } ] }, { "text": "如果有肠旋转不良,十二指肠未绕SMA发生正常旋转,则位于脊柱右侧十二指肠球部下方,且较正常部位更靠近腹侧。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "十二指肠" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "SMA" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "脊柱" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "十二指肠" } ] }, { "text": "腹部平片中未能显示的充满液体的扩张肠段也可使十二指肠空肠连接部下移,造成旋转不良假象,此时可经肛门注入造影剂,以确定回盲部位置。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "腹部平片" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肠段" }, { "start_idx": 22, "end_idx": 30, "type": "bod", "entity": "十二指肠空肠连接部" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "旋转不良" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "肛门" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "造影剂" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "回盲部" } ] }, { "text": "(三)钡剂灌肠尽管钡灌肠是诊断肠旋转不良的方法之一,但同上消化道造影相比,有较大局限性。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 28, "end_idx": 33, "type": "pro", "entity": "上消化道造影" } ] }, { "text": "主要因为15%的正常婴儿回盲部游离或位置较高;而婴儿的结肠相对较长,给回盲部识别和摄片带来困难;更重要的是,肠旋转不良患者回盲部位置可能正常。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "回盲部" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "结肠" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "回盲部" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "回盲部" } ] }, { "text": "虽然存在以上局限,但如果钡灌肠检查发现全部结肠位于左腹部(见于完全不旋转),或(和)升结肠异常缩短且回盲部位于髂骨翼上方,亦可诊断肠旋转不良。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "钡灌肠" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "结肠" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "升结肠" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "回盲部" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "肠旋转不良" } ] }, { "text": "(四)超声作为一种非创伤性检查,通过探查肠系膜上血管位置和走向,超声有助于确诊肠旋转不良。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "超声" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "非创伤性检查" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肠系膜" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "超声" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "肠旋转不良" } ] }, { "text": "但是,超声并不能精确诊断肠旋转不良。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "超声" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "肠旋转不良" } ] }, { "text": "有作者报道,9例手术证实为肠旋转不良的患者,仅有6例B超发现肠系膜血管异常;另一组249例为排除幽门狭窄而接受超声检查患者中,仅9名发现肠系膜血管异常,其中SMV位于SMA左侧的5例均患有肠旋转不良,其余4例SMV位于SMA腹侧,但仅1例证实患有肠旋转不良。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "B超" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "肠系膜" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "狭窄" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "超声" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "肠系膜" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "SMV" }, { "start_idx": 83, "end_idx": 85, "type": "bod", "entity": "SMA" }, { "start_idx": 94, "end_idx": 98, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 104, "end_idx": 106, "type": "bod", "entity": "SMV" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "SMA" }, { "start_idx": 123, "end_idx": 127, "type": "dis", "entity": "肠旋转不良" } ] }, { "text": "因肠旋转不良患者的肠系膜血管位置可能完全正常,而血管位置异常亦不能确定肠旋转不良,故而超声并非诊断肠旋转不良的首选方法,其作用仅限于排除呕吐患儿的幽门狭窄。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肠系膜" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "超声" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "呕吐" }, { "start_idx": 73, "end_idx": 76, "type": "dis", "entity": "幽门狭窄" } ] }, { "text": "【治疗】肠扭转肠梗阻患儿需急诊手术,首先应立即予静脉补液,同时置胃肠减压管、Foley导尿管,血液交叉配型,应用广谱抗生素。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "肠扭转" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "静脉补液" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "胃肠减压管" }, { "start_idx": 38, "end_idx": 45, "type": "pro", "entity": "Foley导尿管" }, { "start_idx": 47, "end_idx": 52, "type": "pro", "entity": "血液交叉配型" }, { "start_idx": 56, "end_idx": 60, "type": "dru", "entity": "广谱抗生素" } ] }, { "text": "时间是能否保留小肠的关键。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "小肠" } ] }, { "text": "如果影像学检查发现肠旋转不良,但无临床症状,不必急诊手术。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "影像学检查" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "肠旋转不良" } ] }, { "text": "手术采用经典的Ladd术式,须遵循以下6个原则:1.探查进腹后,立即将全部肠管托出腹腔,探查是否存在肠扭转或造成肠梗阻的其他原因,辨识回盲部位置。", "entities": [ { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "肠管" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "腹腔" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "肠扭转" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "回盲部" } ] }, { "text": "肠扭转时,腹腔内浑浊腹水提示可能已存在细菌污染,脓性渗出见于缺血性肠坏死;如淋巴回流受阻则出现乳糜样腹水,应常规作腹水培养。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肠扭转" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "腹腔" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "肠坏死" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "腹水培养" } ] }, { "text": "2.扭转复位大多数情况下,肠扭转呈顺时针方向,应行逆时针旋转复位,也就是“把时针往后拨”。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "扭转复位" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肠扭转" } ] }, { "text": "复位后,小肠可能已有充血、水肿及局部坏死,此时可用温水热敷一会儿,观察肠管血运情况。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "小肠" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肠管" } ] }, { "text": "若已存在肠坏死,应行肠切除与造瘘。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "肠坏死" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "肠切除" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "造瘘" } ] }, { "text": "为了尽可能多地保留小肠,对一些暂时无法判断的可疑肠段,应旷置观察24~36小时,再次手术决定是否切除。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "小肠" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肠段" } ] }, { "text": "3.束带松解束带可压迫十二指肠造成反复梗阻,必须松解,通常松解至十二指肠上方的肝门,下方至十二指肠空肠连接部。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "十二指肠" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "反复梗阻" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "十二指肠" }, { "start_idx": 45, "end_idx": 53, "type": "bod", "entity": "十二指肠空肠连接部" } ] }, { "text": "4.加宽肠系膜基底部,松解十二指肠内侧部分的束带,可进一步增加十二指肠和升结肠间距离,最大限度地扩大肠系膜基底部,以减少肠扭转机会。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "肠系膜" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "十二指肠" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "十二指肠" }, { "start_idx": 50, "end_idx": 55, "type": "bod", "entity": "肠系膜基底部" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "肠扭转" } ] }, { "text": "但并不强调回盲部和十二指肠复位至正常解剖位置。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "回盲部" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "十二指肠" } ] }, { "text": "5.解除十二指肠梗阻十二指肠粘连处可形成“螺旋样”结构,从幽门至空肠近端的整个十二指肠,应彻底暴露松解,解除梗阻。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "十二指肠" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "十二指肠" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "十二指肠" } ] }, { "text": "6.顺行切除阑尾后,回盲部最终位于左侧腹,若将来发生阑尾炎,诊断具有困难,故应顺便切除阑尾。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "阑尾" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "回盲部" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "左侧腹" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "阑尾炎" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "阑尾" } ] }, { "text": "小肠放回右侧腹腔,而结肠放在左侧腹。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "小肠" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "腹腔" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "结肠" } ] }, { "text": "【结果】手术治疗肠旋转不良的死亡率为3%~9%,如同时伴有小肠坏死、早产及其他畸形,则死亡率上升。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "小肠坏死" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "早产" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "畸形" } ] }, { "text": "此外,及时辨识肠扭转和肠旋转不良症状、早期诊断与治疗均是改善其预后的重要环节。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "肠扭转" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肠旋转不良" } ] }, { "text": "【并发症】临床上很少复发肠扭转,但术后出现肠梗阻表现时,仍需考虑复发可能。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "肠扭转" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "肠梗阻" } ] }, { "text": "导致肠扭转复发的因素包括:将小肠固定于腹腔右侧、结肠固定于左侧的腹腔内粘连形成不全;或肠系膜基底部不够宽大。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "肠扭转" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "小肠" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "腹腔" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "结肠" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "腹腔" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "肠系膜" } ] }, { "text": "有报告肠扭转的复发率为0~10%。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "肠扭转" } ] }, { "text": "约18%小儿短肠综合征的原因是肠扭转,因此早期及时的诊断与治疗是关键。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "小儿短肠综合征" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "肠扭转" } ] }, { "text": "第二章儿童心血管系统的解剖生理特点第一节心脏的解剖特点在先天性心脏病中,任何的腔室、任何的瓣膜或血管可位于心脏的任何位置。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "心血管系统" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "解剖" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "心脏" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "解剖" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "腔室" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "瓣膜" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "血管" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "心脏" } ] }, { "text": "因此,心腔判别既不能依靠其相对位置(左侧或右侧),也不能单靠其功能进行判断。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心腔" } ] }, { "text": "因为在先天性心脏病中位置和功能变化无常。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "一个心室可位于左侧且为动脉系统,但它可为形态学左心室,亦可为形态学右心室,解剖学形态的判别是先天性心脏病准确诊断的最大难点。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心室" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "动脉系统" }, { "start_idx": 20, "end_idx": 25, "type": "bod", "entity": "形态学左心室" }, { "start_idx": 30, "end_idx": 35, "type": "bod", "entity": "形态学右心室" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "一、心房形态学左右心房的特征(表9-9),每个心房均包含有三个部分:①静脉部,②原始心房部,及③房室通道部。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心房" }, { "start_idx": 4, "end_idx": 10, "type": "bod", "entity": "形态学左右心房" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心房" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "静脉部" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "原始心房部" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "房室通道部" } ] }, { "text": "两心房的静脉部组成不同,但固有心房部均由心耳及小梁组成,房室通道部均由房室瓣及房室隔所组成。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "心房" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "静脉部" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "心房部" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "心耳" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "小梁" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "房室通道部" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "房室瓣" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "房室隔" } ] }, { "text": "表9-2左右心房解剖形态学特征(一)形态学右心房在右心房,静脉部在形态上即是腔静脉窦,由上腔静脉入口,上下腔静脉汇合部静脉汇合部及冠状窦所组成的光滑的腔室。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "左右心房" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "解剖" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "右心房" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "右心房" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "静脉部" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "腔静脉窦" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 51, "end_idx": 58, "type": "bod", "entity": "上下腔静脉汇合部" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "冠状窦" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "腔室" } ] }, { "text": "下腔静脉与腔静脉窦下方相连,上腔静脉则与其上方相接,冠状静脉窦则回流入腔静脉窦中部。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "下腔静脉" }, { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "腔静脉窦下方" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "冠状静脉窦" }, { "start_idx": 35, "end_idx": 40, "type": "bod", "entity": "腔静脉窦中部" } ] }, { "text": "其房隔面为继发隔,房间隔的平面与人体的冠状面呈45°角,故当病人平卧时,房间隔平面并不在水平位置,如需要将房间隔保持水平则需将病人右侧垫高30°~45°。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "房隔面" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "继发隔" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "房间隔" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "房间隔" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "房间隔" } ] }, { "text": "终嵴为与心外界沟相对应的肌性组织,位于下腔静脉入口的侧面,窦房结位于其内膜下方,为腔静脉窦与固有心房的分界线。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "终嵴" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "心外界沟" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "肌性组织" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "下腔静脉" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "窦房结" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "内膜" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "腔静脉窦" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "心房" } ] }, { "text": "外科手术时如果缝到终嵴可引起窦房结动脉栓塞导致窦房结缺血及病窦综合征。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "外科手术" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "终嵴" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "窦房结动脉栓塞" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "窦房结缺血" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "病窦综合征" } ] }, { "text": "TS接近与终嵴垂直,在心导管术时,导管头可能卡在TS下方造成心房穿孔,心房向前突出的部分即为右心耳,其形态为宽底的三角形,位于右心房的前方,将主动脉右侧遮盖,其内面梳状肌丰富。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "TS" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "终嵴" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "心导管术" }, { "start_idx": 17, "end_idx": 19, "type": "equ", "entity": "导管头" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "TS" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "心房" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "心房穿孔" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "心房" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "右心耳" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "右心房" }, { "start_idx": 71, "end_idx": 75, "type": "bod", "entity": "主动脉右侧" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "梳状肌" } ] }, { "text": "心脏手术时,右心耳为手指心内探查及右心房内插管的理想部位。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "心脏手术" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "右心耳" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "手指心内探查" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "右心房内插管" } ] }, { "text": "房室结位于冠状静脉窦口的前方,希氏束则位于冠状窦口与三尖瓣隔前瓣交点(膜部室间隔)的连线上。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "房室结" }, { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "冠状静脉窦口" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "希氏束" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "冠状窦口" }, { "start_idx": 26, "end_idx": 31, "type": "bod", "entity": "三尖瓣隔前瓣" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "膜部室间隔" } ] }, { "text": "房室束紧贴在膜部室间隔下方穿入心室。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "房室束" }, { "start_idx": 6, "end_idx": 12, "type": "bod", "entity": "膜部室间隔下方" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心室" } ] }, { "text": "因此,房室结及房室束位于三尖瓣隔瓣、冠状静脉瓣(Thebesian瓣)及欧氏瓣形成的三角区内(Koch三角)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "房室结" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "房室束" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "三尖瓣隔瓣" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "冠状静脉瓣" }, { "start_idx": 24, "end_idx": 33, "type": "bod", "entity": "Thebesian瓣" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "欧氏瓣" } ] }, { "text": "(二)形态学左心房左心房是四个心腔中最靠后、上且近中线的部分,前方有狭长的指头样突起的左心耳为其特征。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "形态学左心房" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "左心房" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心腔" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "左心耳" } ] }, { "text": "在其内面,房隔面为原发隔,即卵圆孔膜。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "房隔面" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "原发隔" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "卵圆孔膜" } ] }, { "text": "在心房位置正常时,原发隔位于继发隔的左侧,而在心房反位时则位于继发隔的右侧。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "心房" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "原发隔" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "继发隔" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "心房" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "继发隔" } ] }, { "text": "左心房的静脉部由正常情况下四根肺静脉汇合成的共同肺静脉所组成。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "左心房" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "静脉部" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肺静脉" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肺静脉" } ] }, { "text": "三、特异性免疫治疗(脱敏治疗)变应原特异性免疫治疗是通过使用高效、标准化的纯化抗原,使机体对变应原反应性降低,以减轻气道慢性特应性炎症;与成人哮喘相比,呼吸道过敏在儿童哮喘中更为突出,使变应原特异性免疫成为一种重要的治疗儿童过敏性哮喘方法。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "特异性免疫治疗" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "脱敏治疗" }, { "start_idx": 15, "end_idx": 24, "type": "pro", "entity": "变应原特异性免疫治疗" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "抗原" }, { "start_idx": 58, "end_idx": 66, "type": "dis", "entity": "气道慢性特应性炎症" }, { "start_idx": 69, "end_idx": 72, "type": "dis", "entity": "成人哮喘" }, { "start_idx": 76, "end_idx": 80, "type": "dis", "entity": "呼吸道过敏" }, { "start_idx": 82, "end_idx": 85, "type": "dis", "entity": "儿童哮喘" }, { "start_idx": 93, "end_idx": 100, "type": "pro", "entity": "变应原特异性免疫" }, { "start_idx": 110, "end_idx": 116, "type": "dis", "entity": "儿童过敏性哮喘" } ] }, { "text": "这种疗法的唯一缺点是需要多次注射才能达到(个体)最大剂量,而且由于IgE介导的(B细胞抗原决定族引起的)不良反应,每次注射的变应原剂量不能随意增大。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "多次注射" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "IgE" }, { "start_idx": 40, "end_idx": 47, "type": "bod", "entity": "B细胞抗原决定族" }, { "start_idx": 59, "end_idx": 60, "type": "pro", "entity": "注射" } ] }, { "text": "通过对变应原加工,进行化学修饰(如使用甲醛),改变蛋白结构,可以制成类变应原。", "entities": [ { "start_idx": 35, "end_idx": 37, "type": "mic", "entity": "变应原" } ] }, { "text": "理论上使用类变应原可以减少不良反应,延长作用持续时间,减少注射次数。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "注射" } ] }, { "text": "目前认为变应原特异性免疫治疗对下列物质过敏治疗有效:1.花粉引起的哮喘和过敏性鼻炎桦属和桦木科植物花粉、禾本科植物花粉、豚草属植物花粉、Parietaria植物花粉2.屋尘螨引起的哮喘和过敏性鼻炎3.猫皮屑引起的哮喘4.真菌引起的哮喘链格孢属、支孢霉属霉菌现强调治疗应从早期开始,它既可以抑制已形成的变应原过敏状态的进一步发展,还能阻止机体对其他变应原过敏的形成。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "pro", "entity": "变应原特异性免疫治疗" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "花粉引起的哮喘" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "过敏性鼻炎" }, { "start_idx": 84, "end_idx": 91, "type": "dis", "entity": "屋尘螨引起的哮喘" }, { "start_idx": 93, "end_idx": 97, "type": "dis", "entity": "过敏性鼻炎" }, { "start_idx": 100, "end_idx": 107, "type": "dis", "entity": "猫皮屑引起的哮喘" } ] }, { "text": "递增阶段是一个逐渐增加变应原浓度的过程,目的是在减少机体反应性同时,使IgE介导的不良反应降低到最小程度。", "entities": [ { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "IgE" } ] }, { "text": "目前国内主要使用的是螨特异性免疫治疗,并已有舌下螨脱敏制剂开始应用于临床。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "pro", "entity": "螨特异性免疫治疗" }, { "start_idx": 22, "end_idx": 28, "type": "dru", "entity": "舌下螨脱敏制剂" } ] }, { "text": "第十七篇免疫系统疾病第一章小儿免疫系统发育特征一、概述免疫(immune)的最基本含义是识别自己、排斥异己。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "免疫系统疾病" } ] }, { "text": "免疫系统在机体防御感染、清除衰老、死亡、损伤以及突变细胞等方面发挥重要作用。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "感染" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "死亡" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "损伤" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "突变细胞" } ] }, { "text": "免疫系统的各类细胞均来自多能造血干细胞(SC),SC在胚胎期储于卵黄囊或胚肝,最终均存于骨髓。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "细胞" }, { "start_idx": 12, "end_idx": 18, "type": "bod", "entity": "多能造血干细胞" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "SC" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "SC" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "卵黄囊" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "胚肝" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "骨髓" } ] }, { "text": "SC在特殊的微环境中受到特定诱导信号刺激后将向各种细胞方向分化、发育。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "SC" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "细胞" } ] }, { "text": "SC向免疫有关的细胞发育主要有两方面:一方面向免疫效应细胞分化,构成天然免疫系统,如生发中心的树状突细胞、皮肤朗格汉斯细胞(Langerhan)、分泌炎症介质的细胞(肥大细胞、嗜碱性细胞以及嗜酸性细胞等)以及内皮细胞、成纤维细胞、成骨细胞、破骨细胞和肝上皮细胞等天然免疫反应细胞;另一方面分化成两大类淋巴细胞,即T细胞和B细胞,由它们构成了抗原特异性免疫系统。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "SC" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "细胞" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "免疫效应细胞" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "树状突细胞" }, { "start_idx": 53, "end_idx": 60, "type": "bod", "entity": "皮肤朗格汉斯细胞" }, { "start_idx": 62, "end_idx": 70, "type": "bod", "entity": "Langerhan" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "肥大细胞" }, { "start_idx": 88, "end_idx": 92, "type": "bod", "entity": "嗜碱性细胞" }, { "start_idx": 95, "end_idx": 99, "type": "bod", "entity": "嗜酸性细胞" }, { "start_idx": 104, "end_idx": 107, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 109, "end_idx": 113, "type": "bod", "entity": "成纤维细胞" }, { "start_idx": 115, "end_idx": 118, "type": "bod", "entity": "成骨细胞" }, { "start_idx": 120, "end_idx": 123, "type": "bod", "entity": "破骨细胞" }, { "start_idx": 125, "end_idx": 129, "type": "bod", "entity": "肝上皮细胞" }, { "start_idx": 150, "end_idx": 153, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 156, "end_idx": 158, "type": "bod", "entity": "T细胞" }, { "start_idx": 160, "end_idx": 162, "type": "bod", "entity": "B细胞" } ] }, { "text": "成熟淋巴细胞都有自己明确的免疫效能,如通过分泌各类抗体,直接杀伤细胞,分泌效应细胞因子等来发挥特异性免疫功能。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "抗体" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "细胞" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "效应细胞因子" } ] }, { "text": "同时淋巴细胞通过表达各种细胞膜表面分子,由它决定淋巴细胞在免疫网络反应中各自传递信号的形式;通过分泌多种细胞因子来调节免疫系统中细胞间各自的功能状态,从而有机地、协调地发挥免疫系统的完整功能。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "细胞膜" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "细胞因子" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "细胞" } ] }, { "text": "免疫功能的实现主要由五种成分完成:①单核/巨噬细胞(MC/MΦ);②中性粒细胞(PMN);③补体系统;④T淋巴细胞及其分泌的淋巴因子;⑤B淋巴细胞及其分泌的免疫球蛋白(Ig)。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "单核/巨噬细胞" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "MC/MΦ" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "PMN" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "T淋巴细胞" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "淋巴因子" }, { "start_idx": 68, "end_idx": 72, "type": "bod", "entity": "B淋巴细胞" }, { "start_idx": 78, "end_idx": 82, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "Ig" } ] }, { "text": "狭义上讲,由淋巴细胞介导的免疫反应称为特异性免疫,由非淋巴细胞介导的免疫反应为非特异性免疫,事实上淋巴细胞也参与一些非特异性免疫效应,MC/MΦ等也参与一些特异性免疫效应过程。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 67, "end_idx": 71, "type": "bod", "entity": "MC/MΦ" } ] }, { "text": "MC/MΦ作为抗原提呈细胞(APC)吞噬、消化及处理抗原,并将抗原信息递呈给淋巴细胞,另一方面MC/MΦ也分泌细胞因子参与免疫反应。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "MC/MΦ" }, { "start_idx": 7, "end_idx": 12, "type": "bod", "entity": "抗原提呈细胞" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "APC" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "MC/MΦ" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "细胞因子" } ] }, { "text": "Ⅰ类APC分泌IL-12及IL-18,诱导CD4+T淋巴细胞向TH1(辅助性T细胞1)方向分化,TH1细胞分泌白细胞介素-2(IL-2)和干扰素-γ(IFN-γ),促进CD8+T淋巴细胞、毒性杀伤性T细胞(CTL)和自然杀伤细胞(NK)活性,发挥细胞免疫功能;Ⅱ类APC细胞分泌IL-4,诱导CD4+T淋巴向TH2细胞方向分化,TH2细胞分泌IL-4、IL-5、IL-6、IL-8、IL-10、IL-13以及IL-15等细胞因子,辅助B淋巴细胞分化成浆细胞,合成并分泌抗体。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "APC" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "IL-12" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "IL-18" }, { "start_idx": 21, "end_idx": 29, "type": "bod", "entity": "CD4+T淋巴细胞" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "TH1" }, { "start_idx": 35, "end_idx": 41, "type": "bod", "entity": "辅助性T细胞1" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "TH1细胞" }, { "start_idx": 55, "end_idx": 61, "type": "bod", "entity": "白细胞介素-2" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "IL-2" }, { "start_idx": 69, "end_idx": 73, "type": "bod", "entity": "干扰素-γ" }, { "start_idx": 75, "end_idx": 79, "type": "bod", "entity": "IFN-γ" }, { "start_idx": 100, "end_idx": 107, "type": "bod", "entity": "毒性杀伤性T细胞" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "CTL" }, { "start_idx": 114, "end_idx": 119, "type": "bod", "entity": "自然杀伤细胞" }, { "start_idx": 121, "end_idx": 122, "type": "bod", "entity": "NK" }, { "start_idx": 136, "end_idx": 142, "type": "bod", "entity": "Ⅱ类APC细胞" }, { "start_idx": 145, "end_idx": 148, "type": "bod", "entity": "IL-4" }, { "start_idx": 195, "end_idx": 198, "type": "bod", "entity": "IL-4" }, { "start_idx": 200, "end_idx": 203, "type": "bod", "entity": "IL-5" }, { "start_idx": 205, "end_idx": 208, "type": "bod", "entity": "IL-6" }, { "start_idx": 210, "end_idx": 213, "type": "bod", "entity": "IL-8" }, { "start_idx": 215, "end_idx": 219, "type": "bod", "entity": "IL-10" }, { "start_idx": 221, "end_idx": 225, "type": "bod", "entity": "IL-13" }, { "start_idx": 228, "end_idx": 232, "type": "bod", "entity": "IL-15" }, { "start_idx": 234, "end_idx": 237, "type": "bod", "entity": "细胞因子" }, { "start_idx": 239, "end_idx": 245, "type": "bod", "entity": "辅助B淋巴细胞" }, { "start_idx": 249, "end_idx": 251, "type": "bod", "entity": "浆细胞" }, { "start_idx": 258, "end_idx": 259, "type": "bod", "entity": "抗体" } ] }, { "text": "抗体结合抗原形成免疫复合物,并激活补体,补体复合物或效应片段可激活中性粒细胞的趋化、吞噬、氧化和杀菌功能,发生炎症,有利于清除抗原物质,发挥体液免疫功能。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "抗体" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "补体" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "炎症" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "体液" } ] }, { "text": "此外,静息T细胞还可以在转化生长因子-β(TGF-β)诱导下分化成CD4+CD25+调节性T细胞(CD4+CD25+Treg),CD4+CD25+Treg主要发挥免疫抑制作用,调节免疫反应的强度。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "静息T细胞" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "生长因子-β" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "TGF-β" } ] }, { "text": "上述免疫反应网络中,APC与T细胞或B细胞之间的信息传递,除抗原信号之外还需要一些共刺激信号,如T细胞与B细胞或APC之间的CD40配体(CD40L)-CD40;T细胞与B细胞或APC之间的CD28及配体分子(CD80,也称B7)等信号间交联,才能最终完成免疫应答程序。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "APC" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "T细胞" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "B细胞" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "T细胞" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "B细胞" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "APC" }, { "start_idx": 62, "end_idx": 67, "type": "bod", "entity": "CD40配体" }, { "start_idx": 69, "end_idx": 73, "type": "bod", "entity": "CD40L" }, { "start_idx": 76, "end_idx": 79, "type": "bod", "entity": "CD40" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "T细胞" }, { "start_idx": 85, "end_idx": 87, "type": "bod", "entity": "B细胞" }, { "start_idx": 89, "end_idx": 91, "type": "bod", "entity": "APC" }, { "start_idx": 95, "end_idx": 98, "type": "bod", "entity": "CD28" }, { "start_idx": 100, "end_idx": 103, "type": "bod", "entity": "配体分子" }, { "start_idx": 105, "end_idx": 108, "type": "bod", "entity": "CD80" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "B7" } ] }, { "text": "在免疫调节网络中T细胞发挥“中心”作用,免疫网络调节失衡将产生各种临床疾病。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "T细胞" } ] }, { "text": "第六节先天性睾丸发育不全综合征【概述】先天性睾丸发育不全综合征又称Klinefelter综合征,是一种发病率较高的性染色体疾病,在染色体鉴定之前,1942年Klinefelter首先报道了此症,1956年Bradbury等表明患者体细胞内呈女性X染色质,1959年Jacobs和Strong首先发现该病患者的染色体核型为47,XXY。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "先天性睾丸发育不全综合征" }, { "start_idx": 19, "end_idx": 30, "type": "dis", "entity": "先天性睾丸发育不全综合征" }, { "start_idx": 33, "end_idx": 46, "type": "dis", "entity": "Klinefelter综合征" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "性染色体疾病" }, { "start_idx": 115, "end_idx": 125, "type": "sym", "entity": "体细胞内呈女性X染色质" }, { "start_idx": 154, "end_idx": 161, "type": "sym", "entity": "染色体核型为47" } ] }, { "text": "由于性染色体异常导致睾丸发育不全、不育及智能低下等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "性染色体异常" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "睾丸发育不全" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "不育" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "智能低下" } ] }, { "text": "在男性无生育力或性腺发育不良患者中高达30%。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "男性无生育力" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "性腺发育不良" } ] }, { "text": "【病理生理和发病机制】先天性睾丸发育不全综合征是一种先天性睾丸生精发育不全或不发育的疾病,患者常因不育或体检外生殖器不发育而就诊,然后经染色体检查确诊。", "entities": [ { "start_idx": 11, "end_idx": 22, "type": "dis", "entity": "先天性睾丸发育不全综合征" }, { "start_idx": 26, "end_idx": 34, "type": "dis", "entity": "先天性睾丸生精发育" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "不育" } ] }, { "text": "该病不同类型的共同特征是性染色体比正常的XY多一个或一个以上的X染色体。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "性染色体" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "XY" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "X染色体" } ] }, { "text": "多余的X染色体对睾丸及体征均有不对体征影响更甚。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "X染色体" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "睾丸" } ] }, { "text": "X染色体越多,睾丸发育不良程度越明显,症状越严重,智能发育越差,其他畸症状越严重愈多。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "X染色体" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "睾丸" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "畸症状越严重" } ] }, { "text": "由于Y染色体有睾丸决定基因(TDF),该病患者均睾丸决定基因,因此患者表型为男性,但超过正常的X染色体导致不同程度的女性化。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "Y染色体" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "睾丸" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "基因" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "TDF" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "睾丸决定基因" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "X染色体" } ] }, { "text": "先天性睾丸发育不全综合征的染色体核型较多,1987年在国内王德芬等报道的62例中,47,XXY占71.0%,47,XXY/46,XY嵌合体占24.2%,48,XXXY及48,XXYY各约3.2%和1.6%。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dis", "entity": "先天性睾丸发育不全综合征" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "XXY" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "XXY" }, { "start_idx": 64, "end_idx": 68, "type": "bod", "entity": "XY嵌合体" }, { "start_idx": 79, "end_idx": 82, "type": "bod", "entity": "XXXY" }, { "start_idx": 87, "end_idx": 90, "type": "bod", "entity": "XXYY" } ] }, { "text": "这些核型的形成是由于细胞成熟分裂,或受精卵在卵裂中发生的性染色体或性染色单体不分离的结果。", "entities": [ { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "性染色体" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "性染色单体" } ] }, { "text": "染色体基因标记研究提示,卵子细胞的不染色体基因标记的2倍。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "染色体基因" }, { "start_idx": 17, "end_idx": 24, "type": "pro", "entity": "不染色体基因标记" } ] }, { "text": "性染色体畸变,以高龄孕妇妊娠中机会为多,可因卵细胞的衰老、着丝点纵裂动力减弱或纺锤丝迷向的缘故着丝点纵裂动力减弱生殖细胞形成过程中发生了性染色体不分离。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "卵细胞" }, { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "着丝点纵裂动力减弱" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "生殖细胞" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "性染色体" } ] }, { "text": "有分析表明,60%的患者是由于母体染色体不分离,40%是由于父体染色体不分离所致。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "母体染色体" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "父体染色体" } ] }, { "text": "成熟分裂过程父体染色体不分离约有83%的可能发生在第一次减数分裂,17%的可能发生在第二次减数分裂。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "父体染色体" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "父体染色体不分离" } ] }, { "text": "由于X染色体的增多,致使睾丸未发育,阴茎短小,血浆睾酮降低,FSH及LH升高;血浆睾酮降低不足,FSH升高可能是因为支持细胞损伤,分泌抑制素减少之故。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "X染色体" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "睾丸" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "致使睾丸未发育" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "阴茎" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "阴茎短小" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "血浆睾酮" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "血浆睾酮降低" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "FSH" }, { "start_idx": 34, "end_idx": 35, "type": "ite", "entity": "LH" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "血浆睾酮降低不足" }, { "start_idx": 48, "end_idx": 50, "type": "ite", "entity": "FSH" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "细胞" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "抑制素" } ] }, { "text": "支持细胞损伤低,说明该病患者睾丸间质细胞分泌睾酮功能降低,这就必然使LH代偿性升高。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "支持细胞损伤" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "睾丸间质细胞" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "睾酮" } ] }, { "text": "通过电镜观察,患者LH代偿性升高内有异常线粒体和内质网,这可能是干扰睾酮生物合成障碍的物质基础。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "equ", "entity": "电镜" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "LH代偿性升高" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "异常线粒体" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "内质网" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "睾酮生物" } ] }, { "text": "【临床表现】患者表型男性,体格较瘦长,身材较高,指间距大往往增大,乳房女性化约占40%。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "体格较瘦长" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "身材较高" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "指间距大" } ] }, { "text": "青春期发育常延缓,由于无精子,一般青春期发育常延缓有例外)。", "entities": [ { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "青春期发育常延缓" } ] }, { "text": "体格检查发现男性第二性征不明显,无胡须,无喉结,皮肤白皙,睾丸小,可有隐睾或尿道下裂,阴茎亦小。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "胡须" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "无胡须" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "喉结" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "无喉结" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "皮肤" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "皮肤白皙" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "睾丸" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "睾丸小" }, { "start_idx": 34, "end_idx": 41, "type": "sym", "entity": "有隐睾或尿道下裂" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "阴茎" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "阴茎亦小" } ] }, { "text": "患者可有性格孤僻、腼腆、不活跃、胆小。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "性格孤僻" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "腼腆" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "不活跃" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "胆小" } ] }, { "text": "在标准47,XXY核型缺乏男孩性格25%显示中等度智能发育落后,表现为语言和学习障碍。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "XXY" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "缺乏男孩性格" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "智能发育落后" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "语言和学习障碍" } ] }, { "text": "若对智能落后或行为异常的儿童作常规的染色体核型分析进行早期诊断。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "染色体核型分析" } ] }, { "text": "【实验室检查】1.染色体核型分析(1)外周血淋巴细胞染色体核型分析:正常男性体细胞中的性染色体为XY,该病性染色体标准型为XXY,为性染色体三体型。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "染色体核型分析" }, { "start_idx": 19, "end_idx": 32, "type": "pro", "entity": "外周血淋巴细胞染色体核型分析" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "XY" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "XXY" }, { "start_idx": 66, "end_idx": 72, "type": "dis", "entity": "性染色体三体型" } ] }, { "text": "该病80%核型为标准型47,XXY或者标准型核型变型,例如48,XXXY;48,XXYY;49,XXXXY;49,XXXYY。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "XXY" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "XXXY" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "XXYY" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "XXXXY" }, { "start_idx": 57, "end_idx": 61, "type": "bod", "entity": "XXXYY" } ] }, { "text": "15%为嵌合体型,嵌合体有47,XXY/46,XY;47,XXY/46,XX;47,XXY/46,XY/45,X;47,XXY/46,XY/46,XX等。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "XXY" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "XY" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "XXY" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "XX" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "XXY" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "XY" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "X" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "XXY" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "XY" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "XX" } ] }, { "text": "(2)羊水细胞染色体核型分析:为了防止先羊水细胞染色体核型分析性染色体疾病患儿的出生,在妊娠中期第16~20周行羊膜腔穿刺,抽取羊水细胞,经培养后进行胎儿染色体核型分析,发现异常核型及时终止妊娠,可降低出生缺陷。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "pro", "entity": "羊水细胞染色体核型分析" }, { "start_idx": 20, "end_idx": 30, "type": "pro", "entity": "羊水细胞染色体核型分析" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "性染色体疾病" }, { "start_idx": 56, "end_idx": 60, "type": "pro", "entity": "羊膜腔穿刺" }, { "start_idx": 77, "end_idx": 83, "type": "pro", "entity": "染色体核型分析" } ] }, { "text": "2.荧光原位杂交妊娠中期对胎儿染色体核荧光原位杂交论是外周血淋巴细胞还是羊水细胞均需作细胞培养后才能进行核型分析,因此很细胞培养,而荧光原位杂交检测不需细胞培养,可直接对间期细胞进行杂交检测,缩短了诊断时间期细胞于产前诊断则该方法更显出其优势,可直接和绒毛细胞、羊水细胞杂交,1天即可得出结论。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "荧光原位杂交" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "荧光原位杂交" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "细胞培养" }, { "start_idx": 52, "end_idx": 55, "type": "pro", "entity": "核型分析" }, { "start_idx": 60, "end_idx": 63, "type": "pro", "entity": "细胞培养" }, { "start_idx": 66, "end_idx": 73, "type": "pro", "entity": "荧光原位杂交检测" }, { "start_idx": 102, "end_idx": 105, "type": "ite", "entity": "间期细胞" }, { "start_idx": 107, "end_idx": 110, "type": "pro", "entity": "产前诊断" }, { "start_idx": 126, "end_idx": 129, "type": "bod", "entity": "绒毛细胞" }, { "start_idx": 131, "end_idx": 136, "type": "bod", "entity": "羊水细胞杂交" } ] }, { "text": "3.生化检验及其他检验患者血清中睾酮生化检验对下丘脑垂体反馈抑制减弱,垂体促性腺激素黄体生成激素(LH)及促卵泡生长激素(FSH)水平LH高,血清睾酮水平较正常为低;促性腺激素释放激素(LHRH)兴奋试验显示FSH反应增高,LH反应正常;人绒毛HCG)刺激试验睾酮呈低反应。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "血清" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "睾酮" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "生化检验" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "下丘脑垂体" }, { "start_idx": 35, "end_idx": 41, "type": "bod", "entity": "垂体促性腺激素" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "激素" }, { "start_idx": 53, "end_idx": 59, "type": "bod", "entity": "促卵泡生长激素" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "FSH" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "LH" }, { "start_idx": 71, "end_idx": 74, "type": "bod", "entity": "血清睾酮" }, { "start_idx": 83, "end_idx": 87, "type": "bod", "entity": "促性腺激素" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "激素" }, { "start_idx": 93, "end_idx": 96, "type": "bod", "entity": "LHRH" }, { "start_idx": 104, "end_idx": 110, "type": "sym", "entity": "FSH反应增高" }, { "start_idx": 112, "end_idx": 117, "type": "sym", "entity": "LH反应正常" }, { "start_idx": 119, "end_idx": 124, "type": "bod", "entity": "人绒毛HCG" }, { "start_idx": 130, "end_idx": 135, "type": "sym", "entity": "睾酮呈低反应" } ] }, { "text": "患者精液中无精子生成,睾丸活体组织患者精液中无精子生成变性,间质细胞增生。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "精液" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "精子" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "睾丸" }, { "start_idx": 17, "end_idx": 26, "type": "sym", "entity": "患者精液中无精子生成" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "间质细胞" } ] }, { "text": "【诊断】Klinefelter综合征在儿童期可因隐睾或者小睾丸来诊,但多数因症状不严重,缺乏特征性或体格检查疏忽不易被重视缺乏特征性青春发育期,由于睾丸不发育,男性化不全,部分患者有女性乳腺发育或不育而来诊。", "entities": [ { "start_idx": 4, "end_idx": 17, "type": "dis", "entity": "Klinefelter综合征" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "隐睾" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "小睾丸" }, { "start_idx": 61, "end_idx": 65, "type": "sym", "entity": "缺乏特征性" } ] }, { "text": "根据临床特征怀疑为先天性睾丸发育不全综合征的患者可先予生化检查。", "entities": [ { "start_idx": 9, "end_idx": 20, "type": "dis", "entity": "先天性睾丸发育不全综合征" } ] }, { "text": "若血清FSH及LH增高,睾酮水平较正常为低,可对患者作睾酮水平较正常为低型分析。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "sym", "entity": "血清FSH及LH增高" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "睾酮水平较正常为低" }, { "start_idx": 27, "end_idx": 35, "type": "sym", "entity": "睾酮水平较正常为低" } ] }, { "text": "确定X染色体数目,是诊断先天性睾丸发育不全的主要依据。", "entities": [ { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "先天性睾丸发育不全" } ] }, { "text": "FISH技术是快速诊断方法。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "FISH技术" } ] }, { "text": "本症要与青春期发育延迟作鉴别诊断。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "青春期发育延迟" } ] }, { "text": "Klinefelter综合征青春期血促性腺激素明显升高,睾酮水平较低,而青春期发育延迟者处于未发育水平,无促性腺激素升高。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "dis", "entity": "Klinefelter综合征" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "青春期血促性腺激素明显升高" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "睾酮水平较低" }, { "start_idx": 52, "end_idx": 59, "type": "sym", "entity": "无促性腺激素升高" } ] }, { "text": "如对本症患者在无促性腺激素升高活体组织检查,可见曲细精管玻璃样变,其睾丸间质细胞(Legdig细胞)虽有增加,但内分泌【治疗】患者自11~12岁开始应进行雄激素疗法。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "无促性腺激素升高" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "活体组织检查" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "睾丸间质细胞" }, { "start_idx": 41, "end_idx": 48, "type": "bod", "entity": "Legdig细胞" }, { "start_idx": 77, "end_idx": 81, "type": "pro", "entity": "雄激素疗法" } ] }, { "text": "一般应用环戊丙酸酯(cyclopentylproplonateester),开始每次肌注50mg,每3周1次,每隔6~9个月增加剂量50mg,直至达到成人剂量(每3周250mg)。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "环戊丙酸酯" } ] }, { "text": "国内目前较常用的是十一酸睾酮(testosteroneundecanoate)又称安雄(andriol),为睾酮的衍生安雄,有较强的雄激素作用和蛋白同化作用。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "十一酸睾酮" }, { "start_idx": 41, "end_idx": 42, "type": "dru", "entity": "安雄" }, { "start_idx": 59, "end_idx": 60, "type": "dru", "entity": "安雄" } ] }, { "text": "油剂针剂每支250mg,每月注射1次,连续4个月。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "注射" } ] }, { "text": "注射后通过淋巴系统缓慢吸收,血药浓度峰值时间为2天,药效可维持1个月。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "注射" } ] }, { "text": "口服剂型每粒40mg,易被胃肠道吸收,2.5~5小时达高峰,胃肠道小时后恢复原先水平。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "胃肠道" } ] }, { "text": "由于十一酸睾酮通过淋巴系统吸收,不经过肝脏,故对肝功能无影响。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "十一酸睾酮" } ] }, { "text": "自青少年期起辅以雄激素治疗,常可见其学习技能水平提雄激素治疗正常儿差距缩小,变得开朗,自信力增强,但停止治疗后有的又复后退。", "entities": [ { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "雄激素治疗" } ] }, { "text": "【预后】Klinefelter综合征患者根据其染色体核型可初步判断其预后,核型中X染色体越多,预后越差,其次早期诊断,尽早治疗对患儿症状的改善也很有关系。", "entities": [ { "start_idx": 4, "end_idx": 17, "type": "dis", "entity": "Klinefelter综合征" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "X染色体" } ] }, { "text": "第四节言语和语言障碍语言是学习、社会交往、个性发育中一个重要的能力。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "言语和语言障碍" } ] }, { "text": "从广义上来说,儿童言语和语言障碍(languagedisorder)又称沟通障碍。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "言语和语言障碍" } ] }, { "text": "在学龄前儿童中,沟通障碍是最为多见的一个发育问题,约7%~10%的儿童在言语和语言的发育上低于正常标准,而3%~6%的儿童有语言感受或表达障碍,并影响日后的阅读和书写。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "沟通障碍" } ] }, { "text": "语言发育及有关语言的大脑功能存在着性别差异。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "大脑" } ] }, { "text": "最近使用功能性磁共振对语言的研究表明在语言信息处理中,女性较男性在神经系统中有更多的激活;男性大脑的激活具有一侧优势,主要在左脑下额叶角回区,而女性则两侧大脑的相应区域均较活跃。", "entities": [ { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "大脑" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "大脑" } ] }, { "text": "【病因】(一)听力障碍听觉是语言感受的一个重要的渠道,当小儿听力受损害后,不管是传导性的,还是感觉神经性的,都不能正确地察觉声音信号,产生程度不等的语言发育迟缓,其迟缓的严重度受多种因素的影响,诸如听力损害的程度、发生的年龄、矫治听力的年龄、矫治的合适性等。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "听力障碍" } ] }, { "text": "传导性听力障碍伴有反复和长期的中耳炎、同时有渗出,这对早期言语和语言发育可产生不良的影响。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "传导性听力障碍" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "中耳炎" } ] }, { "text": "长期中耳渗出在儿童早期可引起语言表达延迟,在学龄初期出现语言问题。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "长期中耳渗出" } ] }, { "text": "某些染色体和遗传性疾病伴有语言障碍,例如21-三体综合征的儿童有程度不等的语言障碍;脆性X综合征儿童的语言障碍为韵律和语言内容上有特别的形式。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "染色体和遗传性疾病" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "脆性X综合征" } ] }, { "text": "(三)孤独症孤独症的一个重要特征即交流障碍,并伴有交往障碍和刻板的重复性动作。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "孤独症" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "孤独症" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "交流障碍" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "交往障碍" }, { "start_idx": 30, "end_idx": 37, "type": "sym", "entity": "刻板的重复性动作" } ] }, { "text": "孤独症儿童的语言障碍可表现为完全不理解,没有语言,或言语过于刻板、学究式的,并有夸张的韵律。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "孤独症" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "完全不理解" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "没有语言" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "言语过于刻板" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "学究式的" }, { "start_idx": 38, "end_idx": 44, "type": "sym", "entity": "并有夸张的韵律" } ] }, { "text": "语言应用也出现问题,出现回声样语言或非言语的交流,几乎没有眼神交流,面部表情和姿势也很有限。", "entities": [ { "start_idx": 10, "end_idx": 23, "type": "sym", "entity": "出现回声样语言或非言语的交流" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "几乎没有眼神交流" }, { "start_idx": 34, "end_idx": 44, "type": "sym", "entity": "面部表情和姿势也很有限" } ] }, { "text": "(四)神经系统疾病脑性瘫痪儿童因神经运动通路的障碍而影响说话,常出现构音障碍,他们对语言的感受能力比表达好得多。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "神经系统疾病" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "脑性瘫痪" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "构音障碍" } ] }, { "text": "儿童左侧大脑的病变对语言、阅读、书写的影响较右侧大脑病变的影响更大,临床上一些左脑病变的儿童往往保存了原有的语言能力,因为右脑代替了左脑的功能,这说明右脑具有可塑性的功能。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "大脑" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "大脑" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "左脑病变" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "右脑" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "左脑" } ] }, { "text": "近年来,一些少见的神经学因素引起的语言障碍引起人们的关注,这就是获得性失语综合征伴抽搐障碍,或称为Landau-Kleffner综合征。", "entities": [ { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "获得性失语综合征" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "抽搐障碍" }, { "start_idx": 49, "end_idx": 66, "type": "dis", "entity": "Landau-Kleffner综合征" } ] }, { "text": "患儿脑电图表现异常,有两侧的尖慢波,至少2/3患儿有各种类型的癫痫。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "脑电图表现异常" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "有两侧的尖慢波" }, { "start_idx": 20, "end_idx": 32, "type": "sym", "entity": "2/3患儿有各种类型的癫痫" } ] }, { "text": "(五)行为障碍语言障碍和行为问题之间有密切的关系。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "行为障碍" } ] }, { "text": "【临床表现】(一)构音异常即说话不清晰,有的小儿是个别发音的错误,有的则是很多的错误,以致他人听不懂。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "构音异常" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "说话不清晰" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "个别发音的错误" } ] }, { "text": "常见的构音异常有以下几种:1.舌根音化即以舌根音如g、k、h代替大多数语音,例如把“耳朵”说成“耳郭”、“草莓”说成“考莓”、“头发太长”说成“头发盖扛”。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "舌根音化" }, { "start_idx": 20, "end_idx": 36, "type": "sym", "entity": "以舌根音如g、k、h代替大多数语音" } ] }, { "text": "这些儿童常常用舌根磨擦音代替舌前位的发音。", "entities": [ { "start_idx": 4, "end_idx": 19, "type": "sym", "entity": "常常用舌根磨擦音代替舌前位的发音" } ] }, { "text": "2.舌前音化即以舌前音d、t代替某些语音,例如“乌龟”说成“乌堆”、“公园”说成“东园”、“裤子”说成“兔子”。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "舌前音化" }, { "start_idx": 8, "end_idx": 19, "type": "sym", "entity": "舌前音d、t代替某些语音" } ] }, { "text": "3.不送气音化汉语中有许多音如p、t、k、c、s等是送气音。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "不送气音化" } ] }, { "text": "当儿童把送气音用不送气的音作替代,即为错误。", "entities": [ { "start_idx": 4, "end_idx": 15, "type": "sym", "entity": "送气音用不送气的音作替代" } ] }, { "text": "4.省略音化即省略语音的某些部分。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "省略音化" }, { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "省略语音的某些部分" } ] }, { "text": "(二)嗓音问题嗓音问题可以是功能性的,也可以是器质性的,表现为音调、响度、音质共鸣的异常。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "嗓音问题" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "嗓音问题" } ] }, { "text": "声带襞麻痹表现为嗓音柔软或缺如、弱的、喘息样的哭声。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "声带襞麻痹" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "嗓音柔软" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "如、弱的、喘息样的哭声" } ] }, { "text": "共鸣异常表现为鼻音过重或过轻,儿童腭裂、黏膜下腭裂、神经功能障碍影响声门关闭问题造成鼻音过重;而严重上呼吸道感染或鼻炎可造成鼻音过轻。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "共鸣异常" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "鼻音过重或过轻" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "腭裂" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "黏膜下腭裂" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "神经功能障碍" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "鼻音过重" }, { "start_idx": 48, "end_idx": 55, "type": "dis", "entity": "严重上呼吸道感染" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "鼻炎" } ] }, { "text": "2.延长在说某词语时拖长某一声音。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "延长" } ] }, { "text": "3.联带动作当小儿说话不流利时,伴随一些动作如面部扭曲、张大嘴、伸舌、瞪眼、下颌抽搐等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "联带动作" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "面部扭曲" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "张大嘴" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "伸舌" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "瞪眼" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "下颌抽搐" } ] }, { "text": "(二)体格检查一般的体格检查,并注意口腔器官的异常,如畸齿、腭裂、舌系带问题等,口腔运动功能的检查包括下颌的位置是否居中、嘴唇的运动、舌的位置和运动、口的轮替运动、发声情况等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "体格检查" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "体格检查" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "畸齿" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "腭裂" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "舌系带问题" } ] }, { "text": "(三)行为观察行为观察常常在与小儿的游戏中获得信息,观察内容包括游戏的技巧、眼手协调、大运动、注意力、自发语言和沟通技能等,了解儿童认知水平及言语语言能力。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "行为观察" } ] }, { "text": "(四)听力测试儿童构音异常,说话不清晰、迟迟不开口说话均应常规作听力测试,可用声阻抗测听法、耳声发射、脑干诱发电位以排除听力障碍对儿童言语和语言的影响。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "听力测试" } ] }, { "text": "(五)言语评估儿童构音是一定的发育进程的,普通话的音素发育进程见表3-3。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "言语评估" } ] }, { "text": "表3-5普通话的音素发育进程(六)语言评估包括语言理解和语言表达。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "语言评估" } ] }, { "text": "(七)标准化测试1.图片词汇测试该测试于20世纪70年代末期进行了标准化,最初作为儿童智力筛查的工具。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "图片词汇测试" } ] }, { "text": "2.丹佛发育筛查测试这是一项发育筛查,早已有我国的量表,主要用以了解婴幼儿的发育水平,寻找与发育有关的语言发育迟缓,并为语言治疗提供适合发育年龄的可行方案。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "丹佛发育筛查测试" } ] }, { "text": "3.韦氏智力测验包括学龄前和学龄初期、学龄期两种韦氏智力测验。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "韦氏智力测验" }, { "start_idx": 24, "end_idx": 29, "type": "pro", "entity": "韦氏智力测验" } ] }, { "text": "音素水平的治疗:当儿童出现数个错误发音时,治疗总是选择正常儿童最早出现的音(也即最容易的音)入手,这个音称为目标音,首先帮助儿童认识正确发目标音的口形及其他特征,其次进行听觉训练,即区分目标音和另外一个声音,接着让儿童比较自己发目标音和正确目标音之间的差别,建立正确的感知,最后用语音定位法,让儿童看着发目标音时,治疗人员的唇、舌、下颌的运动和口形,让儿童对着镜子模仿发音。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "音素水平的治疗" }, { "start_idx": 85, "end_idx": 88, "type": "pro", "entity": "听觉训练" }, { "start_idx": 140, "end_idx": 144, "type": "pro", "entity": "语音定位法" } ] }, { "text": "音节水平的治疗:一个新的目标音在初学时往往是脆弱而不稳定的,如果不放在音节及其以后水平的治疗中进行强化,就很容易丢失或仍旧回到原来的错误发音。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "音节水平的治疗" } ] }, { "text": "单词水平的治疗:治疗人员在这时把目标音应用到有意义的单词中。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "单词水平的治疗" } ] }, { "text": "句子水平的治疗:治疗人员选择一些符合儿童的句子,采用放慢说话速度、重复说、模仿说、与儿童一起说等方式。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "句子水平的治疗" } ] }, { "text": "2.口功能训练口腔运动功能问题会影响说话的清晰度。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "口功能训练" } ] }, { "text": "因此,临床上发现这类问题的儿童必须进行口功能训练;包括增强口腔黏膜的本体感,即要求每天按压或轻柔快速地弹击儿童的面颊、下颌、唇部;用软硬适中的牙刷或硅胶棒刺激口腔内的舌、牙龈、颊黏膜和硬腭;改善食物质地,从软向硬。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "口腔黏膜" }, { "start_idx": 65, "end_idx": 93, "type": "pro", "entity": "用软硬适中的牙刷或硅胶棒刺激口腔内的舌、牙龈、颊黏膜和硬腭" }, { "start_idx": 95, "end_idx": 105, "type": "pro", "entity": "改善食物质地,从软向硬" } ] }, { "text": "在干预中所用的策略如下:①用单词或叠词作语言刺激,反复应用于环境中,称为“听力轰炸”;②将儿童感兴趣的物品和玩具与单词相匹配;③鼓励儿童用姿势、发声作交流,不必理会其发音不佳;④用最简单的语言与儿童交流;⑤纠正哭叫、发怒、扔物等不良的交流;⑥创造情景,促使儿童与他人交流,并迅速给予应答。", "entities": [ { "start_idx": 13, "end_idx": 23, "type": "pro", "entity": "用单词或叠词作语言刺激" }, { "start_idx": 89, "end_idx": 100, "type": "pro", "entity": "用最简单的语言与儿童交流" }, { "start_idx": 103, "end_idx": 118, "type": "pro", "entity": "纠正哭叫、发怒、扔物等不良的交流" }, { "start_idx": 121, "end_idx": 134, "type": "pro", "entity": "创造情景,促使儿童与他人交流" } ] }, { "text": "表3-6语言发育异常的警告信号【预防】小儿自出生后,应生活在丰富的语言环境中,并且定期进行听力筛查和发育监测,一旦发现异常,立即进行干预。", "entities": [ { "start_idx": 45, "end_idx": 48, "type": "ite", "entity": "听力筛查" }, { "start_idx": 50, "end_idx": 53, "type": "ite", "entity": "发育监测" } ] }, { "text": "第五节磁共振成像技术磁共振成像技术(MRI),计算机断层扫描技术(CT)等现代化的影像技术已成为心脏影像学检查的重要手段,尤其是磁共振成像技术由于其无创、无辐射、较好的软组织成像、三维成像能力及可用于检测功能及生理学信息等优点受到了越来越广泛的应用。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "磁共振成像技术" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "磁共振成像技术" }, { "start_idx": 18, "end_idx": 20, "type": "pro", "entity": "MRI" }, { "start_idx": 23, "end_idx": 31, "type": "pro", "entity": "计算机断层扫描技术" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "CT" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "心脏" }, { "start_idx": 64, "end_idx": 70, "type": "pro", "entity": "磁共振成像技术" } ] }, { "text": "(一)磁共振成像原理磁共振全名为核磁共振显像系统,又称NMR显像系统(nuclearmagneticresonanceimagingsystem)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "磁共振" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "磁共振" }, { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "核磁共振显像系统" }, { "start_idx": 27, "end_idx": 33, "type": "pro", "entity": "NMR显像系统" }, { "start_idx": 35, "end_idx": 71, "type": "pro", "entity": "nuclearmagneticresonanceimagingsystem" } ] }, { "text": "磁共振技术是利用磁共振的原理,测定各组织中运动质子的密度差加以图像显示和判定。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "磁共振技术" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "磁共振" } ] }, { "text": "人体内一般体液或水分,T1和T2几乎相似,T2/T1接近1,因而所得信号也最强,在图像上呈白色。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "体液" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "水分" } ] }, { "text": "而诸如骨质等固体组织,则T2明显小于T1。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "骨质" } ] }, { "text": "肌肉、韧带、内脏等软组织则介于体液与骨骼之间。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肌肉" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "韧带" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "内脏" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "体液" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "骨骼" } ] }, { "text": "流动中的体液,例如脑脊液及血管中血流难以测出信号,故多呈黑色。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "体液" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "脑脊液" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "血管中血流" } ] }, { "text": "(二)患儿准备患儿在进行磁共振检查以前必须检查体内有否手术后金属夹、心脏起搏器或其他金属植入物。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "磁共振检查" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "心脏" } ] }, { "text": "但外科用金属绝大多数由高纯度的合金制成,含铁量较少,不影响磁共振检查。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dep", "entity": "外科" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "磁共振检查" } ] }, { "text": "(三)先天性心脏病的常用检查方案第一图像序列采用T1WSE图像并进行呼吸补偿以获取整个胸腔的基本的解剖信息。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "呼吸补偿" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "胸腔" } ] }, { "text": "随后对感兴趣的部位进行进一步显像,如显示主动脉弓和降主动脉须采用矢状斜切面,而对显示左肺动脉需采用冠状斜切面。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "降主动脉" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "左肺动脉" } ] }, { "text": "此外对特定的心脏畸形需进行特定截面的显像,如四腔面、短轴或左右心室流出道等。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "心脏畸形" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "四腔面" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "短轴" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "左右心室流出道" } ] }, { "text": "(四)心脏解剖结构检查磁共振在显示心血管解剖结构方面尤其在先天性心脏病的具有独特的优势,主要是由于该技术能够明确区分心血管结构和非心血管结构、极高的空间分辨率、可显示任意截面及无视野限制。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "心脏解剖结构检查" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "磁共振" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "心血管" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "心血管结构" } ] }, { "text": "在三个相互垂直的截面进行的增强SE显像可满足绝大多数先天性心脏病诊断的要求。", "entities": [ { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "先天性心脏病" } ] }, { "text": "额状截面可用于显示半月瓣水平主动脉和肺动脉总干的相互空间关系,利于对圆锥动脉干畸形的鉴别诊断。", "entities": [ { "start_idx": 9, "end_idx": 22, "type": "bod", "entity": "半月瓣水平主动脉和肺动脉总干" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "圆锥动脉干畸形" } ] }, { "text": "轴向截面有利于显示肺动脉起源、心房与肺静脉的连接关系及心室与心房的连接关系。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肺动脉" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心房" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肺静脉" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "心室" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "心房" } ] }, { "text": "垂直冠状截面可用于显示左心室流出道,显示气管肺动脉及胸腹腔脏器间的相互关系有助于心脾综合征的诊断。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "左心室流出道" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "气管肺动脉" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "胸腹腔脏器" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "心脾综合征" } ] }, { "text": "矢状截面能理想显示右心室流出道及有助于显示血管环。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "右心室流出道" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "血管环" } ] }, { "text": "矢状斜截面与升主动脉及降主动脉弓走行方向平行,能够清晰显示胸腔内的主动脉,已常规用于主动脉弓的异常。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "bod", "entity": "升主动脉及降主动脉弓" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "胸腔内的主动脉" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "主动脉弓的异常" } ] }, { "text": "直接沿心脏的长短轴所作的截面有利于心室容量及质量的测算或可用于瓣膜疾病或间隔缺损的准确诊断。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "心室" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "瓣膜疾病" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "间隔缺损" } ] }, { "text": "这些截面类似于超声心动图的两腔及四腔切面。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "超声心动图" } ] }, { "text": "对心脏的连续的二维断层切片可以进行三维重建,重建后的三维图像可以外科医师手术时的观察方式显示心内结构的三维空间关系。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "心脏" }, { "start_idx": 32, "end_idx": 33, "type": "dep", "entity": "外科" } ] }, { "text": "(五)磁共振定量测定在先天性心脏病诊断中的应用1.分流量测定运用磁共振速度显像功能,通过多切面多梯度回波显像测定左、右心室每搏量差或可直接通过测定肺总动脉及升主动脉的速度显像,可精确测定体肺循环血流比值(Qp∶Qs)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "磁共振" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "分流量" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "磁共振" }, { "start_idx": 56, "end_idx": 60, "type": "bod", "entity": "左、右心室" }, { "start_idx": 73, "end_idx": 86, "type": "ite", "entity": "肺总动脉及升主动脉的速度显像" }, { "start_idx": 93, "end_idx": 100, "type": "ite", "entity": "体肺循环血流比值" }, { "start_idx": 102, "end_idx": 106, "type": "ite", "entity": "Qp∶Qs" } ] }, { "text": "2.肺动脉压力的定量已有研究表明,肺动脉压力大于70mmHg时,磁共振在收缩期在肺动脉内可测到高密度的信号,信号的密度与肺血管的阻力呈直线相关。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "ite", "entity": "肺动脉压力" }, { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "肺动脉压力" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "磁共振" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "肺动脉" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "肺血管" } ] }, { "text": "运用磁共振血流速度编码技术,可发现肺高压患者的肺动脉的血流峰值速度降低,出现反向血流或湍流。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "磁共振" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "肺高压" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "肺动脉" } ] }, { "text": "因此,在左向右分流先心病患儿,磁共振在无创测量肺动脉压力及血管阻力方面具有潜在的优势。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "左向右分流先心病" }, { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "磁共振" }, { "start_idx": 19, "end_idx": 32, "type": "ite", "entity": "无创测量肺动脉压力及血管阻力" } ] }, { "text": "3.心室容量、射血分数及心肌重量(Mass)的测定双向的X线心血管造影及核素造影可用于测量左心室容量及射血分数,并在一定程度上作为测量的金标准。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "心室容量" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "射血分数" }, { "start_idx": 12, "end_idx": 15, "type": "ite", "entity": "心肌重量" }, { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "Mass" }, { "start_idx": 25, "end_idx": 34, "type": "pro", "entity": "双向的X线心血管造影" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "核素造影" }, { "start_idx": 45, "end_idx": 49, "type": "ite", "entity": "左心室容量" }, { "start_idx": 51, "end_idx": 54, "type": "ite", "entity": "射血分数" } ] }, { "text": "但临床上多应用二维超声心动图进行常规测量。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "二维超声心动图" } ] }, { "text": "当用于右心室或单心室单心室的容量测定时准确性将受到明显的影响。", "entities": [ { "start_idx": 3, "end_idx": 17, "type": "ite", "entity": "右心室或单心室单心室的容量测定" } ] }, { "text": "磁共振具有视野宽、空间分辨率高等优点,可作为心室容量和心肌总量测定的金标准。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "磁共振" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "心室容量" }, { "start_idx": 27, "end_idx": 30, "type": "ite", "entity": "心肌总量" } ] }, { "text": "运用快速屏气K-space截段采样方法,在5分钟之内即可准确测定心室的容量和射血分数。", "entities": [ { "start_idx": 32, "end_idx": 36, "type": "ite", "entity": "心室的容量" }, { "start_idx": 38, "end_idx": 41, "type": "ite", "entity": "射血分数" } ] }, { "text": "同样,磁共振可用于心肌总量的测定。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "磁共振" }, { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "心肌总量" } ] }, { "text": "有研究报道,磁共振可无创检测肥厚性心肌病、高血压患者及运动员的心肌重量。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "磁共振" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "肥厚性心肌病" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "高血压" }, { "start_idx": 31, "end_idx": 34, "type": "ite", "entity": "心肌重量" } ] }, { "text": "第十四节后尿道瓣膜症后尿道瓣膜是男性儿童先天性下尿路梗阻中最常见的疾病。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "后尿道瓣膜症" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "先天性下尿路梗阻" } ] }, { "text": "随着产前超声的广泛应用,新生儿肾积水的发现率逐步提高,后尿道瓣膜的早期发现成为可能。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "产前超声" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "新生儿肾积水" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "后尿道瓣膜" } ] }, { "text": "后尿道瓣膜形成于胚胎的早期,可引起泌尿系统及其他系统的发育异常及功能障碍。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 15, "end_idx": 35, "type": "sym", "entity": "引起泌尿系统及其他系统的发育异常及功能障碍" } ] }, { "text": "胎儿尿是妊娠中、后期羊水的主要来源。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "胎儿尿" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "羊水" } ] }, { "text": "后尿道瓣膜的胎儿因尿道梗阻,排尿少,导致羊水减少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "尿道梗阻" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "排尿少" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "导致羊水减少" } ] }, { "text": "由于尿路梗阻,膀胱压力增高,使输尿管抗反流机制失调形成膀胱输尿管输尿管扩张积水使肾曲管内压力增高而造成肾发育异常上尿路压力增高可破坏肾的集合管系统造成肾小管浓缩功能障碍,即获得性肾性多尿症或肾性糖尿病,尿量增加,从而又加重输尿管扩张,同时也增加了膀胱容量,形成恶性循环。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "尿路梗阻" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "膀胱压力增高" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "输尿管" }, { "start_idx": 14, "end_idx": 24, "type": "sym", "entity": "使输尿管抗反流机制失调" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "膀胱输尿管" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "形成膀胱输尿管" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "输尿管" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "输尿管扩张积水" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "肾曲管" }, { "start_idx": 39, "end_idx": 55, "type": "sym", "entity": "使肾曲管内压力增高而造成肾发育异常" }, { "start_idx": 66, "end_idx": 72, "type": "bod", "entity": "肾的集合管系统" }, { "start_idx": 56, "end_idx": 72, "type": "sym", "entity": "上尿路压力增高可破坏肾的集合管系统" }, { "start_idx": 73, "end_idx": 83, "type": "sym", "entity": "造成肾小管浓缩功能障碍" }, { "start_idx": 86, "end_idx": 93, "type": "dis", "entity": "获得性肾性多尿症" }, { "start_idx": 95, "end_idx": 99, "type": "dis", "entity": "肾性糖尿病" }, { "start_idx": 101, "end_idx": 133, "type": "sym", "entity": "尿量增加,从而又加重输尿管扩张,同时也增加了膀胱容量,形成恶性循环" } ] }, { "text": "后尿道瓣膜的膀胱为克服排尿的阻力,逼尿肌收缩肥厚,随之胶原纤维沉着,反使膀胱逼尿肌收缩功能下降,造成膀胱功能异常。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "膀胱" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "逼尿肌" }, { "start_idx": 6, "end_idx": 23, "type": "sym", "entity": "膀胱为克服排尿的阻力,逼尿肌收缩肥厚" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "随之胶原纤维沉着" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "膀胱逼尿肌" }, { "start_idx": 34, "end_idx": 55, "type": "sym", "entity": "反使膀胱逼尿肌收缩功能下降,造成膀胱功能异常" } ] }, { "text": "【临床表现】由于年龄和后尿道瓣膜梗阻的程度不同,临床表现各异。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "后尿道瓣膜梗阻" } ] }, { "text": "新生儿期可有排尿费力、哭吵及尿滴沥症状,甚至发生急性尿潴留。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "排尿费力" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "哭吵及尿滴沥症状" }, { "start_idx": 20, "end_idx": 28, "type": "sym", "entity": "甚至发生急性尿潴留" } ] }, { "text": "在下腹部常可触及胀大的膀胱,壁很厚因肺发育不良引起呼吸困难及发绀等部分患儿可有尿液性腹水。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "膀胱" }, { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "在下腹部常可触及胀大的膀胱,壁很厚" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肺" }, { "start_idx": 17, "end_idx": 32, "type": "sym", "entity": "因肺发育不良引起呼吸困难及发绀等" }, { "start_idx": 33, "end_idx": 43, "type": "sym", "entity": "部分患儿可有尿液性腹水" } ] }, { "text": "尿液可从肾实质或肾窦渗出,通过有渗透性的腹膜渗入腹腔。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "尿液可从肾实质或肾窦渗出" }, { "start_idx": 13, "end_idx": 25, "type": "sym", "entity": "通过有渗透性的腹膜渗入腹腔" } ] }, { "text": "尿液性腹水可引起水、电解质平衡紊乱,甚至危及生命,但尿液分流至腹腔,减少了肾脏的压力腹膜又可吸收腹水,所以对患儿的预后反有好处。", "entities": [ { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "尿液性腹水可引起水、电解质平衡紊乱,甚至危及生命" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "尿液" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "腹腔" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "肾脏" }, { "start_idx": 25, "end_idx": 41, "type": "sym", "entity": "但尿液分流至腹腔,减少了肾脏的压力" }, { "start_idx": 42, "end_idx": 62, "type": "sym", "entity": "腹膜又可吸收腹水,所以对患儿的预后反有好处" } ] }, { "text": "因呕吐及营养不良可被怀疑为消化系统疾病。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "因呕吐及营养不良可被怀疑为消化系统疾病" } ] }, { "text": "【诊断】对有排尿异常、排尿费力及反复尿路感染的男性儿都应进行超声检查,如发现有双肾积水、膀胱扩张及有小梁小室,随之可进行经会阴部的后尿道超声,有经验的医师可发现瓣膜。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "排尿异常" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "排尿费力" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "反复尿路感染" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "超声检查" }, { "start_idx": 38, "end_idx": 63, "type": "sym", "entity": "有双肾积水、膀胱扩张及有小梁小室,随之可进行经会阴部" }, { "start_idx": 65, "end_idx": 69, "type": "pro", "entity": "后尿道超声" }, { "start_idx": 80, "end_idx": 81, "type": "sym", "entity": "瓣膜" } ] }, { "text": "考虑到后尿道瓣膜即应进行排尿性膀胱尿道造影(VCUG)。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 12, "end_idx": 20, "type": "pro", "entity": "排尿性膀胱尿道造影" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "VCUG" } ] }, { "text": "在胎儿中,羊水量减少是下尿路存在梗阻的一个重要信号。", "entities": [ { "start_idx": 5, "end_idx": 17, "type": "sym", "entity": "羊水量减少是下尿路存在梗阻" } ] }, { "text": "对胎儿期即出现双侧肾积水的新生儿出生后均要进一步做超声检查和VCUG以尽早确诊后尿道瓣膜。", "entities": [ { "start_idx": 1, "end_idx": 15, "type": "sym", "entity": "胎儿期即出现双侧肾积水的新生儿" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "超声检查" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "VCUG" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "后尿道瓣膜" } ] }, { "text": "【治疗】后尿道瓣膜一旦确诊即应保留导尿管,解除下尿路梗阻。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "保留导尿管" }, { "start_idx": 21, "end_idx": 27, "type": "pro", "entity": "解除下尿路梗阻" } ] }, { "text": "先纠正水、电解质失衡,控制感染,随后进行经尿道膀胱镜瓣膜电灼。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "先纠正水、电解质失衡" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "控制感染" }, { "start_idx": 20, "end_idx": 29, "type": "pro", "entity": "经尿道膀胱镜瓣膜电灼" } ] }, { "text": "在比较偏远、交通不发达、又无电切设备的地区,或患儿小、膀胱镜无法进入时可进行膀胱造瘘。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "equ", "entity": "电切设备" }, { "start_idx": 27, "end_idx": 41, "type": "pro", "entity": "膀胱镜无法进入时可进行膀胱造瘘" } ] }, { "text": "但采用不置造瘘管的膀胱皮肤造口术,护理上简单。", "entities": [ { "start_idx": 1, "end_idx": 15, "type": "pro", "entity": "采用不置造瘘管的膀胱皮肤造口术" } ] }, { "text": "部分患儿,因膀胱功能损害严重,瓣膜电切后,仍无法排尿;或肾功能及代谢功能未能改善,也可进行膀胱造瘘。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "膀胱" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "瓣膜电切" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "膀胱造瘘" } ] }, { "text": "对后尿道瓣膜的胎儿进行宫内手术,主要是膀胱造瘘以减压的问题目前有很大的争议,其对母亲和胎儿的影响、所能达到的效果以及并发症的发生情况都在观察之中。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "宫内手术" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "膀胱造瘘" } ] }, { "text": "【预后】后尿道瓣膜的预后并不令人乐观,尤其是新生儿期即出现症状的患儿。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "后尿道瓣膜" } ] }, { "text": "我们在临床上常常看到瓣膜电灼后尿路梗阻已解除,但膀胱内始终有残余尿,肾输尿管积水没有改善或反而恶化,出现肾衰竭的情况。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "瓣膜电灼" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "后尿路梗阻" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "膀胱" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "尿" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "肾输尿管积水" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "因此瓣膜电灼后应定期随访,观察膀胱排空,有无反复泌尿系感染及肾功能恢复情况。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "瓣膜电灼" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "膀胱" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "反复泌尿系感染" } ] }, { "text": "对膀胱输尿管反流,我们一般要在电灼瓣膜后6~12个月以上才考虑手术治疗。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "电灼瓣膜" } ] }, { "text": "对膀胱功能异常,如排尿困难或尿失禁等,要依据膀胱的肌肉收缩状况、膀胱容量以及顺应性分别予以处理。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "膀胱" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "排尿困难或尿失禁" }, { "start_idx": 22, "end_idx": 30, "type": "ite", "entity": "膀胱的肌肉收缩状况" }, { "start_idx": 32, "end_idx": 35, "type": "ite", "entity": "膀胱容量" } ] }, { "text": "可相应地用抗胆碱类药物治疗、间歇性清洁导尿或膀胱扩大术以改善症状。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dru", "entity": "抗胆碱类药物" }, { "start_idx": 14, "end_idx": 31, "type": "pro", "entity": "间歇性清洁导尿或膀胱扩大术以改善症状" } ] }, { "text": "第十七节包茎新生儿出生后在正常情况下其阴茎头完全被包皮覆盖,并且阴茎头和包皮之间存在粘连。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "包茎" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "阴茎头" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "包皮" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "阴茎头" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "包皮" } ] }, { "text": "随着阴茎和阴茎头的发育,以及所产生的分泌物在其间的积聚,粘连的逐渐吸收,加之阴茎勃起的作用,包皮与阴茎头逐渐分离,国外报告在3岁左右,包皮可上翻,阴茎头得以显露。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "阴茎" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "阴茎头" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "分泌物" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "阴茎" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "包皮" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "阴茎头" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "包皮" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "阴茎头" } ] }, { "text": "如果没有排尿困难及没有阴茎头包皮炎,我们认为学龄前儿童包皮覆盖阴茎头,即包皮过长,是正常的生理现象,不必做任何处理。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "阴茎头包皮炎" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "包皮" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "阴茎头" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "包皮过长" } ] }, { "text": "对包茎的定义,我们认为应是在学龄期后包皮口仍狭小,包皮不能翻转显露阴茎头。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "包皮口" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "包皮" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "阴茎头" } ] }, { "text": "但包茎是否一定要手术,尚存争议。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "包茎" } ] }, { "text": "美国对阴茎发育的调查显示,3岁时有90%的包茎自愈;17岁以后,包茎不足1%,因此包皮由不能上翻至完全上翻是一个逐渐发展的过程。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "阴茎" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "包茎" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "包茎" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "包皮" } ] }, { "text": "美国及加拿大的儿科学会已不提倡新生儿包皮环切,当然在有膀胱输尿管反流的情况下另当别论。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "包皮环切" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "膀胱输尿管反流" } ] }, { "text": "嵌顿包茎是包茎的并发症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "嵌顿包茎" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "包茎" } ] }, { "text": "由于包皮口狭小,当包皮被上翻至阴茎头冠状沟上方而又未及时复位后,狭窄的包皮环将阻塞其远端阴茎、尤其是阴茎头的静脉及淋巴回流引起其水肿,反使包皮更不能复原,造成嵌顿包茎待包皮水肿后,包皮狭窄环越来越紧,以至循环阻塞及水肿更加严重,形成恶性循环。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "包皮口狭小" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "包皮" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "阴茎头冠状沟" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "包皮" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "阴茎" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "阴茎头" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "静脉" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "淋巴" }, { "start_idx": 8, "end_idx": 60, "type": "sym", "entity": "当包皮被上翻至阴茎头冠状沟上方而又未及时复位后,狭窄的包皮环将阻塞其远端阴茎、尤其是阴茎头的静脉及淋巴回流" }, { "start_idx": 61, "end_idx": 65, "type": "sym", "entity": "引起其水肿" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "包皮" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "嵌顿包茎" }, { "start_idx": 67, "end_idx": 82, "type": "sym", "entity": "反使包皮更不能复原,造成嵌顿包茎" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "包皮" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "包皮" }, { "start_idx": 83, "end_idx": 98, "type": "sym", "entity": "待包皮水肿后,包皮狭窄环越来越紧" }, { "start_idx": 100, "end_idx": 119, "type": "sym", "entity": "以至循环阻塞及水肿更加严重,形成恶性循环" } ] }, { "text": "临床表现为水肿的包皮翻在阴茎头的冠状沟上方,在水肿的包皮上缘可见到狭窄环,阴茎头呈暗紫色肿大。", "entities": [ { "start_idx": 5, "end_idx": 20, "type": "sym", "entity": "水肿的包皮翻在阴茎头的冠状沟上方" }, { "start_idx": 22, "end_idx": 35, "type": "sym", "entity": "在水肿的包皮上缘可见到狭窄环" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "阴茎头" }, { "start_idx": 37, "end_idx": 45, "type": "sym", "entity": "阴茎头呈暗紫色肿大" } ] }, { "text": "患儿疼痛剧烈,哭闹不止,可有排尿困难。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "患儿疼痛剧烈,哭闹不止,可有排尿困难" } ] }, { "text": "时间过长,嵌顿包皮及阴茎头可发生坏死、脱落。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "阴茎头" }, { "start_idx": 0, "end_idx": 20, "type": "sym", "entity": "时间过长,嵌顿包皮及阴茎头可发生坏死、脱落" } ] }, { "text": "嵌顿包茎应急诊处理,大部分患儿可手法复位。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "嵌顿包茎" } ] }, { "text": "如手法复位失败,要做包皮背侧切开术。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "包皮背侧切开术" } ] }, { "text": "第二节儿童非霍奇金淋巴瘤儿童非霍奇金淋巴瘤(non-Hodgkinlymphomas,NHL)是源于免疫系统器官和细胞的一系列疾病的总称,包括所有未归类于霍奇金病的恶性淋巴瘤。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "儿童非霍奇金淋巴瘤" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "儿童非霍奇金淋巴瘤" }, { "start_idx": 22, "end_idx": 41, "type": "dis", "entity": "non-Hodgkinlymphomas" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "NHL" }, { "start_idx": 50, "end_idx": 58, "type": "bod", "entity": "免疫系统器官和细胞" }, { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "霍奇金病" }, { "start_idx": 84, "end_idx": 86, "type": "dis", "entity": "淋巴瘤" } ] }, { "text": "儿童NHL的现代治疗策略与儿童急性淋巴细胞性白血病(acutelymphoblasticleukemia,ALL)的治疗极其类似。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "儿童NHL" }, { "start_idx": 13, "end_idx": 24, "type": "dis", "entity": "儿童急性淋巴细胞性白血病" }, { "start_idx": 26, "end_idx": 51, "type": "dis", "entity": "acutelymphoblasticleukemia" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "ALL" } ] }, { "text": "此外,免疫系统细胞具多样性,细胞分化为不同功能的多种细胞,以履行各种机体防御的职责。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "免疫系统细胞" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "细胞" } ] }, { "text": "细胞恶变可发生在这些功能截然不同的细胞及其前体细胞之中,因此,儿童NHL的形态学特征、免疫学特征及临床表现均呈现出多样化。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "细胞" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "前体细胞" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "儿童NHL" } ] }, { "text": "组织病理学、免疫学、细胞学及分子生物学的发展已极大地推动了我们对NHL生物学的理解,对正常及恶性淋巴细胞行为学和分子学基础研究的进步也使这些疾病得到了更合理的划分。", "entities": [ { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "NHL" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "同时对恶性淋巴瘤的研究也充分增进了我们对正常淋巴细胞发育的认识。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "恶性淋巴瘤" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "值得注意的是,疗效的明显进步并非源自新型有效药物的开发,而是基于对NHL生物学、免疫学及分子生物学更深刻认知的、更合理的分类系统和相适应的治疗方案的进步和支持治疗的进展。", "entities": [ { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "NHL" } ] }, { "text": "【流行病学及发病机制】儿童淋巴瘤的发病率依年龄不同,在世界不同地区也有显著差异。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "儿童淋巴瘤" } ] }, { "text": "10岁以下的儿童中NHL比霍奇金病更为常见,但霍奇金病的相对发病率在大于10岁的儿童中迅速上升。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "NHL" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "霍奇金病" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "霍奇金病" } ] }, { "text": "此外,特定组织学亚型发病率与年龄有关,如Burkitt及Burkitt样淋巴瘤在5~15岁儿童中多见,而淋巴母细胞性淋巴瘤(lymphoblasticlymphoma,LL)的发病率则在各年龄组中比较恒定。", "entities": [ { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 52, "end_idx": 60, "type": "dis", "entity": "淋巴母细胞性淋巴瘤" }, { "start_idx": 62, "end_idx": 82, "type": "dis", "entity": "lymphoblasticlymphoma" }, { "start_idx": 84, "end_idx": 85, "type": "dis", "entity": "LL" } ] }, { "text": "弥漫性大B细胞淋巴瘤(diffuselargeB-celllymphoma,DLBCL)是一种好发于大龄青少年的疾病,其发病率在整个童年期稳步上升,并在15~19岁年龄组内作为主导地位的组织学亚型达到高峰。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "弥漫性大B细胞淋巴瘤" }, { "start_idx": 11, "end_idx": 36, "type": "dis", "entity": "diffuselargeB-celllymphoma" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "DLBCL" } ] }, { "text": "不同亚型NHL的发病率和相对频率在世界各个地区也有很大差异。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "亚型NHL" } ] }, { "text": "2002~2005年上海市肿瘤登记系统统计结果表明上海市0~14岁组儿童淋巴瘤年发病率为9.9/100万,在儿童肿瘤中占第三位,仅次于白血病和颅内肿瘤,其中近80%为NHL。", "entities": [ { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "儿童淋巴瘤" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "儿童肿瘤" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "白血病" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "颅内肿瘤" }, { "start_idx": 83, "end_idx": 85, "type": "dis", "entity": "NHL" } ] }, { "text": "NHL的病因尚不明确。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "NHL" } ] }, { "text": "经流行病学研究评估,迄今为止产前及产后的暴露研究并未发现各种暴露与患淋巴瘤风险增加明确相关。", "entities": [ { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "淋巴瘤" } ] }, { "text": "遗传或获得性免疫缺陷综合征或接受免疫抑制治疗的病人中,NHL的发病率增高。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "获得性免疫缺陷综合征" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "NHL" } ] }, { "text": "在Burkitt淋巴瘤中,免疫球蛋白基因正常重排程序发生错误,并通过易位导致c-myc基因的功能失调,使细胞的增殖与分化失衡,最终细胞发生癌变。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "dis", "entity": "Burkitt淋巴瘤" }, { "start_idx": 13, "end_idx": 19, "type": "bod", "entity": "免疫球蛋白基因" }, { "start_idx": 38, "end_idx": 44, "type": "bod", "entity": "c-myc基因" }, { "start_idx": 65, "end_idx": 70, "type": "sym", "entity": "细胞发生癌变" } ] }, { "text": "【病理分类】NHL为一组复杂疾病,无论从细胞形态学、临床表现、免疫表型,还是最近的细胞遗传学/分子生物学均有差异较大的变异。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "NHL" } ] }, { "text": "值得注意的是,儿童NHL免疫表型特征与在儿童ALL有明显不同,后者约85%病例为早前B细胞免疫表型,15%源自T细胞,而仅有不到2%的病例是表达表面免疫球蛋白的成熟B细胞表型。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "儿童NHL" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "儿童ALL" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "B细胞" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "T细胞" }, { "start_idx": 72, "end_idx": 78, "type": "bod", "entity": "表面免疫球蛋白" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "B细胞" } ] }, { "text": "纵观所有淋巴系肿瘤,似乎早期B系来源淋巴肿瘤常起源于骨髓,临床上表现为ALL;而T细胞及成熟B细胞肿瘤分别源于胸腺和肠道淋巴组织的生发中心,较前者更可能起源于髓外部位,临床上则表现为NHL为多。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "淋巴系肿瘤" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "淋巴肿瘤" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "骨髓" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "ALL" }, { "start_idx": 40, "end_idx": 50, "type": "dis", "entity": "T细胞及成熟B细胞肿瘤" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "胸腺" }, { "start_idx": 58, "end_idx": 63, "type": "bod", "entity": "肠道淋巴组织" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "髓外" }, { "start_idx": 91, "end_idx": 93, "type": "dis", "entity": "NHL" } ] }, { "text": "大细胞淋巴瘤从形态学和免疫学上均表现有异质性。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "大细胞淋巴瘤" } ] }, { "text": "约1/3的儿童大细胞淋巴瘤为B细胞肿瘤;1/3为T细胞来源,且通常表达胸腺后“激活”T细胞的抗原谱;另30%则来自不确定的谱系(非T、非B)。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "儿童大细胞淋巴瘤" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "B细胞肿瘤" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "T细胞" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "胸腺" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "T细胞" } ] }, { "text": "极少数病例来自组织细胞/巨噬细胞系,此类可被称为“真性组织细胞淋巴瘤”(表11-11)。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "组织细胞" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 25, "end_idx": 33, "type": "dis", "entity": "真性组织细胞淋巴瘤" } ] }, { "text": "表11-15儿童NHL组织病理学、免疫表型、临床特征、细胞遗传学及分子学特征之间的关系以欧洲/美国主导的REAL淋巴肿瘤分类法(REALclassification)将免疫学、分子遗传学及标准形态学结合,并由此形成了WHO造血系统及淋巴肿瘤分类法的基石。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "儿童NHL" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "淋巴肿瘤" }, { "start_idx": 116, "end_idx": 119, "type": "dis", "entity": "淋巴肿瘤" } ] }, { "text": "由此而来的WHO分类法将源自前体(淋巴母)淋巴细胞到成熟淋巴细胞各阶段起源淋巴系肿瘤ALL和NHL均包括在内,基于免疫表型谱(B细胞或T细胞/自然杀伤细胞肿瘤)进一步分类。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "淋巴母" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 37, "end_idx": 44, "type": "dis", "entity": "淋巴系肿瘤ALL" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "NHL" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "B细胞" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "T细胞" }, { "start_idx": 71, "end_idx": 78, "type": "dis", "entity": "自然杀伤细胞肿瘤" } ] }, { "text": "这一分类系统体现了淋巴细胞在不同成熟阶段恶变所形成的NHL和ALL自特定的临床、形态学、免疫学、遗传学特征。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "NHL" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "ALL" } ] }, { "text": "根据WHO2008分类标准,儿童NHL主要有四个重要类型:成熟B细胞肿瘤(包括Burkitt淋巴瘤/成熟B细胞性白血病、未能进一步分类的B细胞淋巴瘤、弥漫大B细胞淋巴瘤及纵隔大B细胞淋巴瘤亚型),成熟(或周围)T细胞及自然杀伤细胞肿瘤(包括ALCL、CD30+、T细胞及裸细胞型),前B细胞肿瘤(包括前体B淋巴母细胞白血病/淋巴瘤),以及前T细胞肿瘤(包括前体T淋巴母细胞白血病/淋巴瘤)。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "儿童NHL" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "B细胞肿瘤" }, { "start_idx": 39, "end_idx": 48, "type": "dis", "entity": "Burkitt淋巴瘤" }, { "start_idx": 50, "end_idx": 58, "type": "dis", "entity": "成熟B细胞性白血病" }, { "start_idx": 68, "end_idx": 73, "type": "dis", "entity": "B细胞淋巴瘤" }, { "start_idx": 75, "end_idx": 83, "type": "dis", "entity": "弥漫大B细胞淋巴瘤" }, { "start_idx": 85, "end_idx": 95, "type": "dis", "entity": "纵隔大B细胞淋巴瘤亚型" }, { "start_idx": 98, "end_idx": 116, "type": "dis", "entity": "成熟(或周围)T细胞及自然杀伤细胞肿瘤" }, { "start_idx": 120, "end_idx": 123, "type": "dis", "entity": "ALCL" }, { "start_idx": 125, "end_idx": 128, "type": "dis", "entity": "CD30" }, { "start_idx": 153, "end_idx": 158, "type": "dis", "entity": "前B细胞肿瘤" }, { "start_idx": 162, "end_idx": 172, "type": "dis", "entity": "前体B淋巴母细胞白血病" }, { "start_idx": 174, "end_idx": 176, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 181, "end_idx": 186, "type": "dis", "entity": "前T细胞肿瘤" }, { "start_idx": 190, "end_idx": 200, "type": "dis", "entity": "前体T淋巴母细胞白血病" }, { "start_idx": 202, "end_idx": 204, "type": "dis", "entity": "淋巴瘤" } ] }, { "text": "(一)Burkitt淋巴瘤Burkitt淋巴瘤在显微镜下肿瘤细胞弥漫性浸润,细胞小,含圆或卵圆形细胞核、1~3个强嗜碱性核仁,以及常含有脂泡的嗜碱性胞质。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "Burkitt淋巴瘤" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "Burkitt淋巴瘤" }, { "start_idx": 28, "end_idx": 36, "type": "sym", "entity": "肿瘤细胞弥漫性浸润" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "细胞小" }, { "start_idx": 42, "end_idx": 61, "type": "sym", "entity": "含圆或卵圆形细胞核、1~3个强嗜碱性核仁" }, { "start_idx": 63, "end_idx": 75, "type": "sym", "entity": "以及常含有脂泡的嗜碱性胞质" } ] }, { "text": "零散的残余正常巨噬细胞散布于恶性细胞之间,呈现特征性的“星空”貌。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "恶性细胞" } ] }, { "text": "从免疫学上来说,Burkitt淋巴瘤及其变异型是生发中心B细胞肿瘤,细胞膜表达κ或λ轻链相关的表面免疫球蛋白(常为IgM),并可表达B系相关抗原CD19、CD20、CD22、CD79a及CD10,但常不表达末端脱氧核苷酸转移酶(TdT),是否表达TdT有助于鉴别Burkitt淋巴瘤与LL。", "entities": [ { "start_idx": 8, "end_idx": 17, "type": "dis", "entity": "Burkitt淋巴瘤" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "B细胞肿瘤" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "细胞膜" }, { "start_idx": 49, "end_idx": 53, "type": "bod", "entity": "免疫球蛋白" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "IgM" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "CD19" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "CD20" }, { "start_idx": 94, "end_idx": 97, "type": "bod", "entity": "CD22" }, { "start_idx": 105, "end_idx": 108, "type": "bod", "entity": "CD79" }, { "start_idx": 117, "end_idx": 120, "type": "bod", "entity": "CD10" }, { "start_idx": 133, "end_idx": 142, "type": "bod", "entity": "末端脱氧核苷酸转移酶" }, { "start_idx": 144, "end_idx": 146, "type": "bod", "entity": "TdT" }, { "start_idx": 153, "end_idx": 155, "type": "bod", "entity": "TdT" }, { "start_idx": 161, "end_idx": 170, "type": "dis", "entity": "Burkitt淋巴瘤" }, { "start_idx": 172, "end_idx": 173, "type": "dis", "entity": "LL" } ] }, { "text": "1%~2%ALL患者有Burkitt淋巴瘤的形态学及免疫学特征(即FAB形态学分类中的L3型),常伴有腹部或其余部位的包块性疾病极晚期Burkitt淋巴瘤,对这类患儿应采用晚期Burkitt淋巴瘤的治疗方案。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "ALL" }, { "start_idx": 11, "end_idx": 20, "type": "dis", "entity": "Burkitt淋巴瘤" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "腹部" }, { "start_idx": 48, "end_idx": 63, "type": "sym", "entity": "常伴有腹部或其余部位的包块性疾病" }, { "start_idx": 64, "end_idx": 76, "type": "dis", "entity": "极晚期Burkitt淋巴瘤" }, { "start_idx": 86, "end_idx": 97, "type": "dis", "entity": "晚期Burkitt淋巴瘤" } ] }, { "text": "另两种变异易位可在15%的Burkitt淋巴瘤病例中观察到,包括t(2;8)(p-11.1;q24.1)及t(8;22)(q24.1;q11.2)。", "entities": [ { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "Burkitt淋巴瘤" } ] }, { "text": "(二)间变性大细胞淋巴瘤间变性大细胞淋巴瘤(ALCL)是儿童大细胞淋巴瘤中最常见的亚型,绝大多数归于成熟T细胞和自然杀伤细胞肿瘤。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "间变性大细胞淋巴瘤" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "间变性大细胞淋巴瘤" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "ALCL" }, { "start_idx": 28, "end_idx": 35, "type": "dis", "entity": "儿童大细胞淋巴瘤" }, { "start_idx": 50, "end_idx": 63, "type": "dis", "entity": "成熟T细胞和自然杀伤细胞肿瘤" } ] }, { "text": "组织学常表现为凝聚性的、奇形怪状的、含丰富胞质的多型性大细胞,包含形状怪异的马蹄形细胞核ALCL表达T细胞抗原。", "entities": [ { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "细胞核" }, { "start_idx": 32, "end_idx": 43, "type": "sym", "entity": "含形状怪异的马蹄形细胞核" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "ALCL" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "T细胞抗原" } ] }, { "text": "该类肿瘤细胞也表达上皮细胞膜抗原(epithelialmembraneantigen,EMA)和CD30(Ki-1)抗原ALCL常存在特征性非随机染色体[t(2;5)(p23;q35)]平衡易位,染色体5q35位上的核磷蛋白基因NPM,与染色体2p23位上的间变性淋巴瘤激酶(anaplasticlymphomakinase,ALK),一种酪氨酸激酶基因融合。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 9, "end_idx": 15, "type": "bod", "entity": "上皮细胞膜抗原" }, { "start_idx": 17, "end_idx": 41, "type": "bod", "entity": "epithelialmembraneantigen" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "EMA" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "CD30" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "Ki-1" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "ALCL" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "染色体" }, { "start_idx": 98, "end_idx": 102, "type": "bod", "entity": "染色体5q" }, { "start_idx": 108, "end_idx": 116, "type": "bod", "entity": "核磷蛋白基因NPM" }, { "start_idx": 119, "end_idx": 121, "type": "bod", "entity": "染色体" }, { "start_idx": 129, "end_idx": 136, "type": "bod", "entity": "间变性淋巴瘤激酶" }, { "start_idx": 138, "end_idx": 161, "type": "bod", "entity": "anaplasticlymphomakinase" }, { "start_idx": 163, "end_idx": 165, "type": "bod", "entity": "ALK" }, { "start_idx": 170, "end_idx": 176, "type": "bod", "entity": "酪氨酸激酶基因" } ] }, { "text": "(三)淋巴母细胞白血病/淋巴瘤WHO将前驱T或B淋巴母细胞型白血病/淋巴瘤归为同一类,前驱T细胞起源者以淋巴瘤为多见,而前驱B细胞起源者以白血病多见。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "淋巴母细胞白血病" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 21, "end_idx": 32, "type": "dis", "entity": "T或B淋巴母细胞型白血病" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "前驱T细胞" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "前驱B细胞" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "白血病" } ] }, { "text": "T系相关抗原表达通常包括UCHL1(CD45RO)、CD1、CD2、CD3、CD4、CD5、CD7、CD8、CD56;B系表达CD19、CD20、CD22、CD79a及CD10,类似B系白血病,不表达细胞膜表达κ或λ轻链相关的表面免疫球蛋白(常为IgM)。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "抗原" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "UCHL1" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "CD45RO" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "CD1" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "CD2" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "CD3" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "CD4" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "CD5" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "CD7" }, { "start_idx": 86, "end_idx": 88, "type": "bod", "entity": "CD8" }, { "start_idx": 96, "end_idx": 99, "type": "bod", "entity": "CD56" }, { "start_idx": 111, "end_idx": 114, "type": "bod", "entity": "CD19" }, { "start_idx": 122, "end_idx": 125, "type": "bod", "entity": "CD20" }, { "start_idx": 133, "end_idx": 136, "type": "bod", "entity": "CD22" }, { "start_idx": 144, "end_idx": 147, "type": "bod", "entity": "CD79" }, { "start_idx": 156, "end_idx": 159, "type": "bod", "entity": "CD10" }, { "start_idx": 169, "end_idx": 173, "type": "dis", "entity": "B系白血病" }, { "start_idx": 178, "end_idx": 180, "type": "bod", "entity": "细胞膜" }, { "start_idx": 191, "end_idx": 197, "type": "bod", "entity": "表面免疫球蛋白" }, { "start_idx": 201, "end_idx": 203, "type": "bod", "entity": "IgM" } ] }, { "text": "前驱T和B淋巴细胞均表达TdT。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "前驱T和B淋巴细胞" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "TdT" } ] }, { "text": "【临床表现】NHL临床表现差异大,一些病人仅有外周淋巴结无痛性肿大几乎无全身症状,因此在活检后即明确诊断。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "NHL" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "淋巴结" }, { "start_idx": 23, "end_idx": 32, "type": "sym", "entity": "外周淋巴结无痛性肿大" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "几乎无全身症状" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "活检" } ] }, { "text": "常见表现有非特异性全身症状,如发热,浅表淋巴结肿大盗汗。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "非特异性全身症状" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "如发热" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "淋巴结" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "浅表淋巴结肿大" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "盗汗" } ] }, { "text": "晚期病人出现消瘦、贫血、出血倾向、发热、肝脾大、浆膜腔积液恶病质等症状和体征。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "消瘦" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "贫血" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "出血倾向" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "发热" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肝脾" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "肝脾大" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "浆膜腔" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "浆膜腔积液" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "恶病质" } ] }, { "text": "1.淋巴母细胞型淋巴瘤原发于纵隔较为多见(70%为前T细胞性),肿块常位于前或中纵隔,巨大肿块可压迫气管、上腔静脉、心脏和肺,有时还合并大量胸水,临床出现胸痛刺激性咳嗽、气促、平卧困难,重者有呼吸困难、发绀、颈头面部及上肢水肿上腔静脉压迫综合征。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "淋巴母细胞型淋巴瘤" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "纵隔" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肿块" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "前或中纵隔" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "肿块" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "气管" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "心脏" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "肺" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "胸水" }, { "start_idx": 77, "end_idx": 77, "type": "bod", "entity": "胸" }, { "start_idx": 77, "end_idx": 78, "type": "sym", "entity": "胸痛" }, { "start_idx": 79, "end_idx": 83, "type": "sym", "entity": "刺激性咳嗽" }, { "start_idx": 85, "end_idx": 86, "type": "sym", "entity": "气促" }, { "start_idx": 88, "end_idx": 91, "type": "sym", "entity": "平卧困难" }, { "start_idx": 96, "end_idx": 99, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 101, "end_idx": 102, "type": "sym", "entity": "发绀" }, { "start_idx": 104, "end_idx": 104, "type": "bod", "entity": "颈" }, { "start_idx": 105, "end_idx": 105, "type": "bod", "entity": "头" }, { "start_idx": 104, "end_idx": 112, "type": "sym", "entity": "颈头面部及上肢水肿" }, { "start_idx": 113, "end_idx": 121, "type": "dis", "entity": "上腔静脉压迫综合征" } ] }, { "text": "胸部X线平片可见中、前纵隔巨大肿块可伴有不等量胸水伯基特型淋巴瘤原发于腹部较为多见(成熟B细胞性,Burkitt),可有腹痛、腹围增大、恶心、呕吐、大便习惯改变、肝脾肿大、腹水。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "胸部X线平片" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "中、前纵隔" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "中、前纵隔巨大肿块" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "胸水" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "可伴有不等量胸水" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "伯基特型淋巴瘤" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "腹部" }, { "start_idx": 59, "end_idx": 61, "type": "sym", "entity": "有腹痛" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "腹围增大" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "恶心" }, { "start_idx": 71, "end_idx": 72, "type": "sym", "entity": "呕吐" }, { "start_idx": 74, "end_idx": 79, "type": "sym", "entity": "大便习惯改变" }, { "start_idx": 81, "end_idx": 84, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 86, "end_idx": 87, "type": "sym", "entity": "腹水" } ] }, { "text": "3.大细胞型淋巴瘤70%大细胞型淋巴瘤来源于T细胞性,20%~30%为B细胞性,尚有部分病人来源于NK细胞或不表达T或B细胞标记的裸细胞。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "大细胞型淋巴瘤" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "大细胞型淋巴瘤" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "NK细胞" }, { "start_idx": 57, "end_idx": 61, "type": "bod", "entity": "T或B细胞" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "裸细胞" } ] }, { "text": "大细胞型NHL临床表现相对复杂,病程相对较长,可有较特殊部位的浸润,如原发于皮肤皮下组织、中枢神经系统、肺、睾丸、骨甚至肌肉等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "大细胞型NHL" }, { "start_idx": 38, "end_idx": 43, "type": "bod", "entity": "皮肤皮下组织" }, { "start_idx": 45, "end_idx": 50, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "肺" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "睾丸" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "骨" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "肌肉" } ] }, { "text": "儿童NHL可在诊断时和病程中出现中枢神经系统浸润,并有相应症状与体征,各型NHL均可发生,与骨髓浸润同时存在较为多见,包括脑膜、脑神经、脑实质、脊髓、脊髓旁及混合性浸润,出现头痛、呕吐等颅高压症状,或面瘫、感觉障碍、肌力改变、截瘫等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "儿童NHL" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "NHL" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "骨髓" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "脑膜" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "脑神经" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "脑实质" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "脊髓" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "脊髓" }, { "start_idx": 85, "end_idx": 97, "type": "sym", "entity": "出现头痛、呕吐等颅高压症状" }, { "start_idx": 100, "end_idx": 101, "type": "sym", "entity": "面瘫" }, { "start_idx": 103, "end_idx": 106, "type": "sym", "entity": "感觉障碍" }, { "start_idx": 108, "end_idx": 111, "type": "sym", "entity": "肌力改变" }, { "start_idx": 113, "end_idx": 114, "type": "sym", "entity": "截瘫" } ] }, { "text": "如不给予中枢浸润预防性措施,病程中中枢浸润机会很高,眼神经与面神经受累机会较多。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "中枢" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "中枢" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "眼神经" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "面神经" } ] }, { "text": "少数病人因中枢浸润所致的临床表现而首诊。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "中枢" } ] }, { "text": "【辅助检查】1.全身的影像学检查以评估疾病浸润范围,见分期检查。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "影像学检查" } ] }, { "text": "2.实验室检查①血清乳酸脱氢酶(LDH)水平与肿瘤成正相关,并和预后相关,因此在治疗前应进行评估;②高肿瘤负荷者可发生心、肝、肾等重要脏器的浸润而致功能不全,治疗前因仔细评估;③高负荷NHL在治疗前、初始治疗的一周内易发生肿瘤细胞溶解综合征,因此在这段时间内应定时进行肾功能、血电解质的监测;④进行增强CT检查前应先核实肾功能情况,有肿瘤细胞溶解综合征或肾功能不良时应避免增强CT,因造影剂可能加重肾功能不全;⑤外周血常规检查如存在贫血、血小板减少常提示为晚期或有骨髓浸润;⑥骨髓涂片可除外骨髓浸润;⑦浆膜腔液体沉渣涂片检查结合免疫表型检查有助于诊断、鉴别诊断和肿瘤浸润状态的评估。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "ite", "entity": "血清乳酸脱氢酶" }, { "start_idx": 16, "end_idx": 18, "type": "ite", "entity": "LDH" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "肿瘤" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "肿瘤" }, { "start_idx": 59, "end_idx": 59, "type": "bod", "entity": "心" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "肝" }, { "start_idx": 63, "end_idx": 63, "type": "bod", "entity": "肾" }, { "start_idx": 92, "end_idx": 94, "type": "dis", "entity": "NHL" }, { "start_idx": 111, "end_idx": 119, "type": "dis", "entity": "肿瘤细胞溶解综合征" }, { "start_idx": 134, "end_idx": 136, "type": "ite", "entity": "肾功能" }, { "start_idx": 138, "end_idx": 141, "type": "ite", "entity": "血电解质" }, { "start_idx": 149, "end_idx": 154, "type": "pro", "entity": "增强CT检查" }, { "start_idx": 160, "end_idx": 162, "type": "ite", "entity": "肾功能" }, { "start_idx": 167, "end_idx": 175, "type": "dis", "entity": "肿瘤细胞溶解综合征" }, { "start_idx": 177, "end_idx": 179, "type": "ite", "entity": "肾功能" }, { "start_idx": 186, "end_idx": 189, "type": "pro", "entity": "增强CT" }, { "start_idx": 199, "end_idx": 199, "type": "bod", "entity": "肾" }, { "start_idx": 206, "end_idx": 210, "type": "pro", "entity": "外周血常规" }, { "start_idx": 216, "end_idx": 217, "type": "sym", "entity": "贫血" }, { "start_idx": 219, "end_idx": 223, "type": "sym", "entity": "血小板减少" }, { "start_idx": 232, "end_idx": 233, "type": "bod", "entity": "骨髓" }, { "start_idx": 238, "end_idx": 241, "type": "pro", "entity": "骨髓涂片" }, { "start_idx": 245, "end_idx": 246, "type": "bod", "entity": "骨髓" }, { "start_idx": 251, "end_idx": 261, "type": "pro", "entity": "浆膜腔液体沉渣涂片检查" }, { "start_idx": 264, "end_idx": 268, "type": "pro", "entity": "免疫表型检" }, { "start_idx": 281, "end_idx": 282, "type": "pro", "entity": "肿瘤" } ] }, { "text": "【诊断标准】NHL的诊断必须依据于病理(细胞)形态学、免疫学和细胞/分子遗传学。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "NHL" } ] }, { "text": "【疾病分期检查及分期标准】在治疗前必须先明确分期,常规分期检查包括以下项目:全身体格检查,眼底检查,骨髓活检及涂片,胸腹盆腔影像学检查(以增强CT检查为主),脑脊液离心甩片找肿瘤细胞,疑有中枢浸润时增强头颅MRI或CT以除外颅内转移,疑有骨骼浸润时全身骨扫描。", "entities": [ { "start_idx": 38, "end_idx": 43, "type": "pro", "entity": "全身体格检查" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "眼底检查" }, { "start_idx": 50, "end_idx": 56, "type": "pro", "entity": "骨髓活检及涂片" }, { "start_idx": 58, "end_idx": 66, "type": "pro", "entity": "胸腹盆腔影像学检查" }, { "start_idx": 69, "end_idx": 74, "type": "pro", "entity": "增强CT检查" }, { "start_idx": 79, "end_idx": 85, "type": "pro", "entity": "脑脊液离心甩片" }, { "start_idx": 87, "end_idx": 90, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "中枢" }, { "start_idx": 99, "end_idx": 108, "type": "pro", "entity": "增强头颅MRI或CT" }, { "start_idx": 119, "end_idx": 120, "type": "bod", "entity": "骨骼" }, { "start_idx": 124, "end_idx": 128, "type": "pro", "entity": "全身骨扫描" } ] }, { "text": "通过以上检查确定肿瘤浸润范围并据此作出临床分期。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肿瘤" } ] }, { "text": "表11-16St.Jude非霍奇金淋巴瘤分期系统注:中枢神经系统浸润定义:1.CSFWBC≥5个/μl,并CSF标本离心发现淋巴瘤细胞;或2.有明确中枢神经系统受累症状或/和体怔,如脑神经瘫痪,并不能用其他原因解释;或3.脊髓浸润;或4.孤立性脑内肿瘤性病变。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "非霍奇金淋巴瘤" }, { "start_idx": 26, "end_idx": 31, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 39, "end_idx": 44, "type": "ite", "entity": "CSFWBC" }, { "start_idx": 53, "end_idx": 55, "type": "ite", "entity": "CSF" }, { "start_idx": 62, "end_idx": 66, "type": "bod", "entity": "淋巴瘤细胞" }, { "start_idx": 74, "end_idx": 79, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 91, "end_idx": 95, "type": "dis", "entity": "脑神经瘫痪" }, { "start_idx": 111, "end_idx": 112, "type": "bod", "entity": "脊髓" }, { "start_idx": 119, "end_idx": 128, "type": "dis", "entity": "孤立性脑内肿瘤性病变" } ] }, { "text": "骨髓受累定义:1.骨髓穿刺涂片见≥5%但<25%的幼稚淋巴细胞;2.或骨髓活检发现局灶性浸润【治疗】(一)整体治疗原则及目标治疗的目标是使疾病获得完全缓解并长期无病生存,同时获得正常的远期生命质量。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "骨髓" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "骨髓穿刺涂片" }, { "start_idx": 25, "end_idx": 30, "type": "ite", "entity": "幼稚淋巴细胞" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "骨髓活检" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "局灶性浸润" } ] }, { "text": "治疗原则上以化疗为主,根据不同分期、形态分型或/及免疫分型采用不同药物联合和强度的治疗方案。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "化疗" } ] }, { "text": "除中枢浸润、脊髓肿瘤压迫症、化疗后局部残留病灶、姑息性治疗等特殊情况外,常规治疗不推荐放疗。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "中枢" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "脊髓肿瘤压迫症" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "化疗" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "姑息性治疗" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "放疗" } ] }, { "text": "手术主要用于下列情况:①手术活检,尽量争取获得组织标本以明确诊断及分型,如肿块较小并为局限性病变,可将肿块完全切除。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "手术活检" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "肿块" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "切除" } ] }, { "text": "估计肿块不能完全切除时应仅做小切口活检术,不推荐肿瘤部分或大部分切除术;②急腹症:出现如肠套叠、完全性肠梗阻、肠穿孔、严重的胃肠道出血等外科急腹症时考虑急诊手术;③二次活检及手术:化疗3个疗程后有稳定残留病灶时,应考虑再次活检(手术),为进一步治疗提供依据。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肿块" }, { "start_idx": 17, "end_idx": 19, "type": "pro", "entity": "活检术" }, { "start_idx": 24, "end_idx": 34, "type": "pro", "entity": "肿瘤部分或大部分切除术" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "急腹症" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "肠套叠" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "完全性肠梗阻" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "肠穿孔" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "胃肠道出血" }, { "start_idx": 68, "end_idx": 69, "type": "dep", "entity": "外科" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "急腹症" }, { "start_idx": 76, "end_idx": 79, "type": "pro", "entity": "急诊手术" }, { "start_idx": 82, "end_idx": 88, "type": "pro", "entity": "二次活检及手术" }, { "start_idx": 90, "end_idx": 91, "type": "pro", "entity": "化疗" }, { "start_idx": 111, "end_idx": 112, "type": "pro", "entity": "活检" }, { "start_idx": 114, "end_idx": 115, "type": "pro", "entity": "手术" } ] }, { "text": "(二)急诊处理儿童NHL临床进展较快,应将之视作急诊,尽快完成各项检查明确诊断。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "急诊" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "儿童NHL" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "急诊" } ] }, { "text": "如为巨大纵隔肿块伴有气道及上腔静脉压迫症状无外周淋巴结肿大骨髓及胸腹腔积液)也不能诊断时,可选择性采取肿块粗针穿刺活检、纵隔镜活检或胸骨旁切口活检。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "纵隔" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "气道" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 2, "end_idx": 20, "type": "sym", "entity": "巨大纵隔肿块伴有气道及上腔静脉压迫症状" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "外周淋巴结" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "无外周淋巴结肿大" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "骨髓" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "胸腹腔积液" }, { "start_idx": 53, "end_idx": 58, "type": "pro", "entity": "粗针穿刺活检" }, { "start_idx": 60, "end_idx": 64, "type": "pro", "entity": "纵隔镜活检" }, { "start_idx": 66, "end_idx": 72, "type": "pro", "entity": "胸骨旁切口活检" } ] }, { "text": "如此时有危及生命的现象,全身麻醉过于危险,临床及影像学检查符合NHL,为抢救生命可给予紧急低剂量化疗,12~24小时后多数病人的压迫症状就可能得到缓解,病情稍稳定后再行活检(24~48小时内),但此时由于受化疗影响组织细胞学判断可能出现困难,因此应尽量避免先治疗后活检。", "entities": [ { "start_idx": 21, "end_idx": 28, "type": "pro", "entity": "临床及影像学检查" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "NHL" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "化疗" }, { "start_idx": 84, "end_idx": 85, "type": "pro", "entity": "活检" }, { "start_idx": 103, "end_idx": 104, "type": "pro", "entity": "化疗" }, { "start_idx": 107, "end_idx": 110, "type": "bod", "entity": "组织细胞" }, { "start_idx": 132, "end_idx": 133, "type": "pro", "entity": "活检" } ] }, { "text": "治疗时间越长病理诊断越困难,因此即使先治疗缓解危及生命的状况,也应积极准备在治疗48小时内进行活检手术。", "entities": [ { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "活检手术" } ] }, { "text": "对已明确诊断的肿瘤负荷较大的患儿,应尽早采用3~7天低强度化疗(如糖皮质激素和长春新碱),同时给予水化2000~3000ml/m2、5%碳酸氢钠5ml/kg碱化尿液、别嘌呤醇10mg/kg抑制过多的尿酸形成,维持水电解质酸碱平衡,避免肿瘤细胞溶解过快造成的肿瘤细胞溶解综合征。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "化疗" }, { "start_idx": 33, "end_idx": 37, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 39, "end_idx": 42, "type": "dru", "entity": "长春新碱" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "水化" }, { "start_idx": 74, "end_idx": 77, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 84, "end_idx": 85, "type": "pro", "entity": "碱化" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "尿液" }, { "start_idx": 89, "end_idx": 92, "type": "dru", "entity": "别嘌呤醇" }, { "start_idx": 105, "end_idx": 106, "type": "bod", "entity": "尿酸" }, { "start_idx": 112, "end_idx": 115, "type": "bod", "entity": "水电解质" }, { "start_idx": 116, "end_idx": 116, "type": "bod", "entity": "酸" }, { "start_idx": 117, "end_idx": 117, "type": "bod", "entity": "碱" }, { "start_idx": 123, "end_idx": 126, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 134, "end_idx": 142, "type": "dis", "entity": "肿瘤细胞溶解综合征" } ] }, { "text": "(三)支持治疗(1)治疗期及治疗结束后1月TMP-SMZ50mg/(kg•d)分两次口服,每周用三天,大剂量MTX前24小时至MTX血浓度降至<0.1μmol/L期间停用。", "entities": [ { "start_idx": 21, "end_idx": 27, "type": "dru", "entity": "TMP-SMZ" }, { "start_idx": 54, "end_idx": 56, "type": "dru", "entity": "MTX" }, { "start_idx": 63, "end_idx": 68, "type": "ite", "entity": "MTX血浓度" } ] }, { "text": "以预防肺孢子虫感染。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "mic", "entity": "肺孢子虫" } ] }, { "text": "(2)当外周血白细胞<1000/cm3或粒细胞绝对计数<500/cm3时应用C-CSF或GM-CSF5μg/(kg•d)至外周血白细胞>2000/cm3。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "ite", "entity": "外周血白细胞" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "粒细胞" }, { "start_idx": 44, "end_idx": 48, "type": "dru", "entity": "C-CSF" }, { "start_idx": 50, "end_idx": 56, "type": "dru", "entity": "GM-CSF5" }, { "start_idx": 67, "end_idx": 72, "type": "ite", "entity": "外周血白细胞" } ] }, { "text": "(3)血小板减少并有活动性出血或血小板<20×109/L输注血小板。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "血小板" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "血小板" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "血小板" } ] }, { "text": "(四)各亚型NHL的方案选择根据病理形态学分型及/或免疫分型,分别采用成熟B细胞型NHL(非淋巴母细胞型)或淋巴母细胞型NHL(免疫表型为前驱T或前驱B)治疗方案,根据分期及分组化疗强度不同。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "亚型NHL" }, { "start_idx": 35, "end_idx": 43, "type": "dis", "entity": "成熟B细胞型NHL" }, { "start_idx": 54, "end_idx": 62, "type": "dis", "entity": "淋巴母细胞型NHL" }, { "start_idx": 89, "end_idx": 90, "type": "pro", "entity": "化疗" } ] }, { "text": "成熟B细胞性NHL(代表性疾病为伯基特淋巴瘤)的化疗方案原则是短程、强烈,以烷化剂和抗代谢性药物(主要是甲氨蝶呤和阿糖胞苷)为主,化疗强度根据临床分组或分期而定。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "B细胞性NHL" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "伯基特淋巴瘤" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "化疗" }, { "start_idx": 52, "end_idx": 55, "type": "dru", "entity": "甲氨蝶呤" }, { "start_idx": 57, "end_idx": 60, "type": "dru", "entity": "阿糖胞苷" }, { "start_idx": 65, "end_idx": 66, "type": "dru", "entity": "化疗" } ] }, { "text": "而对前驱T或B淋巴细胞型NHL(形态为淋巴母细胞型)的化疗方案原则与急性淋巴细胞白血病(ALL)一致。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "前驱T或B淋巴细胞型NHL" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "化疗" }, { "start_idx": 34, "end_idx": 42, "type": "dis", "entity": "急性淋巴细胞白血病" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "ALL" } ] }, { "text": "【疾病状态评估标准和影响预后因素】通常在治疗42~60天时需要评估肿瘤对治疗的反应,以评介治疗的有效性,并根据疗效反应对治疗方案作适当的修正。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "肿瘤" } ] }, { "text": "疗效评介标准包括:①完全缓解(CR):CT、骨扫描、脑脊液、及体检均未发现残留肿瘤迹象,骨髓涂片<5%幼淋巴细胞、或经病理活检证实残留病灶无肿瘤细胞,并维持1个月以上;②部分缓解(PR):肿瘤缩小>50%,但未达CR,无新发或重新进展病灶,骨髓涂片<5%幼淋巴细胞、脑脊液必须无肿瘤细胞,并维持在1个月以上;③好转:所有可检测病灶减少<50%,无新发病灶或重新进展;④进展:原有疾病状态基础上的进展。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "CT" }, { "start_idx": 22, "end_idx": 24, "type": "pro", "entity": "骨扫描" }, { "start_idx": 26, "end_idx": 28, "type": "pro", "entity": "脑脊液" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "体检" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "骨髓涂片" }, { "start_idx": 51, "end_idx": 55, "type": "ite", "entity": "幼淋巴细胞" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "病理活检" }, { "start_idx": 70, "end_idx": 73, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 94, "end_idx": 95, "type": "dis", "entity": "肿瘤" }, { "start_idx": 120, "end_idx": 123, "type": "pro", "entity": "骨髓涂片" }, { "start_idx": 127, "end_idx": 131, "type": "ite", "entity": "幼淋巴细胞" }, { "start_idx": 133, "end_idx": 135, "type": "ite", "entity": "脑脊液" }, { "start_idx": 139, "end_idx": 142, "type": "bod", "entity": "肿瘤细胞" } ] }, { "text": "影响NHL预后的主要因素是初诊时肿瘤的负荷,LDH水平超过正常值2倍、存在中枢浸润和/或骨髓转移时提示肿瘤负荷高,预后相对不良,需要更强烈的治疗。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "NHL" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "肿瘤" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "LDH" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "中枢" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "骨髓" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "肿瘤" } ] }, { "text": "肿瘤对治疗早期的反应也常预示着预后,治疗反应不佳,治疗42~60天未能获得完全缓解者预后不良。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" } ] }, { "text": "但影像学水平残留病灶并不一定代表残留病灶内存在活性肿瘤细胞,部分病例残留灶内及仅为坏死组织、纤维组织等非肿瘤性成分,因此有必要进行再次病理活检,以明确残留灶内是否存在肿瘤细胞,对后续治疗方案的确定十分重要,以避免过度治疗和治疗不足。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "坏死组织" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "纤维组织" }, { "start_idx": 67, "end_idx": 70, "type": "pro", "entity": "病理活检" }, { "start_idx": 83, "end_idx": 86, "type": "bod", "entity": "肿瘤细胞" } ] }, { "text": "第三篇不同年龄儿童的生长发育特点第一章儿童生长发育特点连续不断的生长发育是小儿时期特有的生理现象,生长是由于细胞数量和大小的增加,引起个体体积增大的形态学改变,为“量”的变化。", "entities": [ { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "细胞" } ] }, { "text": "发育则为细胞、组织、器官功能的成熟,为“质”的变化。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "细胞" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "组织" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "器官" } ] }, { "text": "在生长发育中,经常联系到成熟(maturity)的概念,这是指机体的整体和局部,系统或器官在形态上、功能上逐渐达到正常成人的水平。", "entities": [ { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "系统" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "器官" } ] }, { "text": "第一节体格生长特点(一)生长是一个连续过程生长是一个连续过程,但并不匀速,各年龄的生长速率各不相同,年龄越小,生长速率越快。", "entities": [ { "start_idx": 37, "end_idx": 60, "type": "sym", "entity": "各年龄的生长速率各不相同,年龄越小,生长速率越快" } ] }, { "text": "在整个生长期有两个生长高峰,一是婴儿期,到第一年末体重增加到出生体重的3倍,身长则增加到1.5倍。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "ite", "entity": "体重" }, { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "体重" }, { "start_idx": 38, "end_idx": 39, "type": "ite", "entity": "身长" } ] }, { "text": "(二)身体各系统和各部分生长不平衡身体各系统的生长发育先后和快慢各不相同,如神经系统发育较早,生长速度快,大脑在出生时约重390g,1岁时已达900g,8岁时已接近成人重量。", "entities": [ { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "神经系统" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "大脑" } ] }, { "text": "淋巴系统则先快后回缩,生殖系统发育最晚,皮下脂肪在年幼时发育较快,肌肉组织到学龄期才发育加速。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "淋巴系统" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "生殖系统" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "皮下脂肪" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "肌肉组织" } ] }, { "text": "身体各部分的生长也各不相同,在整个生长期头部增长了1倍、躯干增长了2倍、上肢增长了3倍、下肢则增长了4倍。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "头部" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "躯干" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "上肢" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "下肢" } ] }, { "text": "从一个头大、四肢相对短小下肢修长、头部较小的成人体形。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "头" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "四肢" }, { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "头大、四肢相对短小" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "下肢修长、头部较小" } ] }, { "text": "五、呼吸功呼吸运动过程中,气道压力的变化使肺容积产生相应的变化。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "呼吸功" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "气道" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "肺" } ] }, { "text": "呼吸功(WOB)=压力变化(△P)×容积变化(△V)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "呼吸功" }, { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "WOB" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "压力" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "△P" }, { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "容积" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "△V" } ] }, { "text": "呼吸功主要用于克服气道黏性阻力,组织弹性阻力,肺泡表面张力所做的功。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "呼吸功" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "气道" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺泡" } ] }, { "text": "呼吸功主要是指吸气时。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "呼吸功" } ] }, { "text": "足月新生儿呼吸功=1500g•cm/min。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "呼吸功" } ] }, { "text": "肺顺应性降低(如肺表面活性物质缺乏)和胸廓顺应性降低(如肥胖)均可增加弹性功。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "肺顺应性" }, { "start_idx": 8, "end_idx": 14, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 19, "end_idx": 23, "type": "ite", "entity": "胸廓顺应性" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "肥胖" } ] }, { "text": "气道阻力增加(如哮喘),使克服气道的黏性阻力功上升。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "气道" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "哮喘" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "气道" } ] }, { "text": "第四节先天性巨结肠【发病率】先天性巨结肠(Hirschsprung’sdisease,HD)是一种比较常见的小儿消化道畸形,国外统计发病率为1/5000,其中男孩占70%~80%。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "先天性巨结肠" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "先天性巨结肠" }, { "start_idx": 21, "end_idx": 41, "type": "dis", "entity": "Hirschsprung’sdisease" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "HD" }, { "start_idx": 54, "end_idx": 60, "type": "dis", "entity": "小儿消化道畸形" } ] }, { "text": "有一份报道描述了4个家族中的22例受累同胞,其中大多为长段型神经节细胞缺乏症。", "entities": [ { "start_idx": 27, "end_idx": 37, "type": "dis", "entity": "长段型神经节细胞缺乏症" } ] }, { "text": "患有神经节细胞缺乏症的母亲比患该病的父亲更易将此病传给子女,如果父母均患有全结肠神经节细胞缺乏症,则子女患病率为12.5%。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "神经节细胞缺乏症" }, { "start_idx": 37, "end_idx": 47, "type": "dis", "entity": "全结肠神经节细胞缺乏症" } ] }, { "text": "【病因】虽然早在1888年HaraldHirschsprung就首次详尽描述了先天性巨结肠这一疾病,且至今论述其病理生理改变的文章已有500多篇,但病因仍不明确。", "entities": [ { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "先天性巨结肠" } ] }, { "text": "目前比较一致的观点是位于10号染色体的RET基因(常染色体显性)、位于13号染色体的内皮素受体B基因(常染色体隐性)以及位于20号染色体的内皮素3(EDN3)基因(常染色体隐性)与巨结肠的发生有关。", "entities": [ { "start_idx": 10, "end_idx": 23, "type": "bod", "entity": "位于10号染色体的RET基因" }, { "start_idx": 33, "end_idx": 49, "type": "bod", "entity": "位于13号染色体的内皮素受体B基因" }, { "start_idx": 60, "end_idx": 80, "type": "bod", "entity": "位于20号染色体的内皮素3(EDN3)基因" }, { "start_idx": 90, "end_idx": 92, "type": "dis", "entity": "巨结肠" } ] }, { "text": "然而,HD所具有的高散发性特征,同一受累家族中在神经节细胞缺乏症的类型及性别上的差异,均提示HD相关基因可能不止一个,而是多基因的复杂遗传模式。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "HD" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "神经节细胞缺乏症" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "HD" } ] }, { "text": "常染色体不完全显性遗传可能与长段型神经节细胞缺乏症有关,而常染色体隐性或多基因遗传则是短段型HD的遗传方式。", "entities": [ { "start_idx": 14, "end_idx": 24, "type": "dis", "entity": "长段型神经节细胞缺乏症" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "HD" } ] }, { "text": "现已发现,50%的家族性病例和10%~20%的散发病例具有RET酪氨酸酶受体基因突变,但仍不能完全证实遗传缺陷是导致神经嵴细胞增生缺陷和神经节细胞分布异常的根本原因,也就是说,至今仍无法明确巨结肠的病因。", "entities": [ { "start_idx": 29, "end_idx": 41, "type": "dis", "entity": "RET酪氨酸酶受体基因突变" }, { "start_idx": 58, "end_idx": 66, "type": "dis", "entity": "神经嵴细胞增生缺陷" }, { "start_idx": 68, "end_idx": 76, "type": "dis", "entity": "神经节细胞分布异常" }, { "start_idx": 95, "end_idx": 97, "type": "dis", "entity": "巨结肠" } ] }, { "text": "【病理生理学】HD病理生理学的基础包括肠神经节细胞发育不良、神经节细胞缺乏或无神经节细胞,导致肠蠕动不良、肛门内括约肌不能松弛或松弛困难。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "HD" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "肠神经节细胞" }, { "start_idx": 30, "end_idx": 34, "type": "ite", "entity": "神经节细胞" }, { "start_idx": 39, "end_idx": 43, "type": "ite", "entity": "神经节细胞" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "肠蠕动不良" }, { "start_idx": 53, "end_idx": 67, "type": "sym", "entity": "肛门内括约肌不能松弛或松弛困难" } ] }, { "text": "但必须指出,HD患儿神经支配异常仅仅是一种定量概念。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "HD" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "支配异常" } ] }, { "text": "(一)蠕动反射肠蠕动是由位于肠腔内容团块上方的环肌收缩,而位于下端的肠段反射性松弛,以及肠管纵形肌同时收缩并向远端传递所构成。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肠" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肠腔" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "环肌" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肠段" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "肠管纵形肌" } ] }, { "text": "该神经反射由肠管扩张和肠平滑肌中的起搏细胞同步去极化激发,胆碱能神经元将电冲动传至位于黏液下和肌间神经丛中的中间神经元。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肠管" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "肠平滑肌" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "起搏细胞" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "胆碱能" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "神经元" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "黏液" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "肌间神经丛" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "中间神经元" } ] }, { "text": "该中间神经元是一种非肾上腺素能非胆碱能神经元,通过ATP、血管活性肠肽(VIP)和NO来直接抑制平滑肌细胞。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "中间神经元" }, { "start_idx": 9, "end_idx": 21, "type": "bod", "entity": "非肾上腺素能非胆碱能神经元" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "ATP" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "血管活性肠肽" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "VIP" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "NO" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "平滑肌细胞" } ] }, { "text": "每个肠壁神经丛的神经节含4~6个神经细胞,同时接受胆碱能和肾上腺素能神经支配,而肠壁外神经信号通过肾上腺素能纤维支配的血管实行调节作用,肾上腺素调节胆碱能突触释放乙酰胆胺。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "bod", "entity": "肠壁神经丛的神经节" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "神经细胞" }, { "start_idx": 25, "end_idx": 35, "type": "bod", "entity": "胆碱能和肾上腺素能神经" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "支配" }, { "start_idx": 49, "end_idx": 55, "type": "bod", "entity": "肾上腺素能纤维" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "支配" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "血管" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "胆碱能" }, { "start_idx": 81, "end_idx": 84, "type": "bod", "entity": "乙酰胆胺" } ] }, { "text": "除了以上提到的神经纤维、黏液下和肌间神经丛,星形胶质细胞似乎也有相当重要的肠道肌肉调节作用。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "神经纤维" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "黏液" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "肌间神经丛" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "星形胶质细胞" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "肠道肌肉" } ] }, { "text": "最近有报道NO是介导胃肠道平滑肌松弛的一种神经传导介质,与NADPN-黄递酶相同,可作为HD的诊断标志物。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "NO" }, { "start_idx": 10, "end_idx": 15, "type": "bod", "entity": "胃肠道平滑肌" }, { "start_idx": 29, "end_idx": 37, "type": "bod", "entity": "NADPN-黄递酶" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "HD" } ] }, { "text": "缺乏合成NO的神经元可能是无神经节细胞肠段无法松弛的原因。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "NO" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "神经元" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "神经节细胞肠段" } ] }, { "text": "贮存神经肽如VIP、P物质、脑啡肽、神经激肽A、组胺、异亮氨酸及胃泌素释放肽等的神经元也参与蠕动反射,在HD及相关肠神经性疾病患者中已发现该神经元的缺乏或发育异常。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "VIP" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "P物质" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "脑啡肽" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "神经激肽A" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "组胺" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "异亮氨酸" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "胃泌素释放肽" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "神经元" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "HD" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "肠神经性疾病" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "神经元" } ] }, { "text": "(二)肛门内括约肌内括约肌松弛类似于蠕动反射,开始排便时,位于粪团下方肠段松弛,肛管开放。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "肛门内括约肌" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肠段" } ] }, { "text": "该反射存在即提示机体具有正常的神经传递,可排除HD。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "HD" } ] }, { "text": "调节肛门内括约肌的四种神经机制包括:1.α-肾上腺素能神经,主要存在于下腹下神经节及其纤维内,通过α受体维持括约肌的兴奋节律。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "肛门内括约肌" }, { "start_idx": 20, "end_idx": 28, "type": "bod", "entity": "α-肾上腺素能神经" }, { "start_idx": 35, "end_idx": 40, "type": "bod", "entity": "下腹下神经节" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "α受体" } ] }, { "text": "2.β-肾上腺素能抑制性受体,松弛平滑肌。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "β-肾上腺素能" } ] }, { "text": "3.胆碱能神经元,对括约肌作用尚不清楚。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "胆碱能" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "神经元" } ] }, { "text": "4.非肾上腺素能非胆碱能神经元(NANC-中间神经元),通过NO、VIP和其他神经肽能神经元,松弛内括约肌。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "bod", "entity": "非肾上腺素能非胆碱能神经元" }, { "start_idx": 16, "end_idx": 25, "type": "bod", "entity": "NANC-中间神经元" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "NO" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "VIP" }, { "start_idx": 39, "end_idx": 45, "type": "bod", "entity": "神经肽能神经元" }, { "start_idx": 47, "end_idx": 52, "type": "bod", "entity": "松弛内括约肌" } ] }, { "text": "肠神经系统发育异常如胆碱能神经节缺乏、NANC-中间神经元缺乏、各种神经肽能纤维缺乏和可能存在的肠壁结缔组织缺乏是HD肠功能异常的原因。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "肠神经系统发育异常" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "胆碱能神经节缺乏" }, { "start_idx": 19, "end_idx": 30, "type": "dis", "entity": "NANC-中间神经元缺乏" }, { "start_idx": 32, "end_idx": 41, "type": "dis", "entity": "各种神经肽能纤维缺乏" }, { "start_idx": 43, "end_idx": 55, "type": "dis", "entity": "可能存在的肠壁结缔组织缺乏" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "HD肠功能异常" } ] }, { "text": "骶副交感神经丛发出的胆碱能纤维进入肠壁,直接作用于平滑肌细胞,使之产生同步收缩,神经末梢释放的乙酰胆碱由等量乙酰胆碱酯酶灭活,这也就是乙酰胆碱酯酶染色这一诊断HD有效方法的基本原理。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "骶副交感神经丛" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "胆碱能纤维" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肠壁" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "平滑肌细胞" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "神经末梢" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "乙酰胆碱" }, { "start_idx": 54, "end_idx": 59, "type": "bod", "entity": "乙酰胆碱酯酶" }, { "start_idx": 67, "end_idx": 72, "type": "bod", "entity": "乙酰胆碱酯酶" }, { "start_idx": 73, "end_idx": 74, "type": "pro", "entity": "染色" }, { "start_idx": 79, "end_idx": 80, "type": "dis", "entity": "HD" } ] }, { "text": "但活检组织乙酰胆碱酯酶染色有10%误诊率,尤其是长段型HD。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "ite", "entity": "乙酰胆碱酯酶" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "染色" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "HD" } ] }, { "text": "由于缺乏NANC中间神经元和NO,无神经节细胞肠段持续收缩、不能松弛,但该肠管本身固有弹性产生的一些不协调的肠蠕动,一定程度上仍可使排便成为可能,这也是某些HD患者较晚才被确诊的原因。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "bod", "entity": "NANC中间神经元" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "NO" }, { "start_idx": 17, "end_idx": 28, "type": "sym", "entity": "无神经节细胞肠段持续收缩" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "不能松弛" }, { "start_idx": 37, "end_idx": 56, "type": "sym", "entity": "肠管本身固有弹性产生的一些不协调的肠蠕动" }, { "start_idx": 78, "end_idx": 79, "type": "dis", "entity": "HD" } ] }, { "text": "存在神经细胞黏附分子减少和NADPH-黄递酶活性下降的神经节细胞减少症和肠神经发育不良症,之所以具有相似临床表现,亦因为此。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "sym", "entity": "神经细胞黏附分子减少" }, { "start_idx": 13, "end_idx": 25, "type": "sym", "entity": "NADPH-黄递酶活性下降" }, { "start_idx": 27, "end_idx": 34, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 36, "end_idx": 43, "type": "dis", "entity": "肠神经发育不良症" } ] }, { "text": "此外,直接作用于平滑肌细胞的兴奋性α-受体的肾上腺素能神经纤维正常,也使肛门内括约肌处于持续痉挛状态,不能舒张。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "平滑肌细胞" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "兴奋性α-受体" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "肾上腺素能" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "神经纤维" }, { "start_idx": 36, "end_idx": 41, "type": "bod", "entity": "肛门内括约肌" } ] }, { "text": "(三)神经节细胞减少症在HD无神经节细胞肠段的近端,通常存在一神经节细胞减少区域。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "HD" }, { "start_idx": 15, "end_idx": 19, "type": "ite", "entity": "神经节细胞" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肠段" }, { "start_idx": 30, "end_idx": 39, "type": "sym", "entity": "一神经节细胞减少区域" } ] }, { "text": "而神经节细胞减少症也可独立发病,肠壁神经节细胞数量和神经纤维密度显著减少,乙酰胆碱酯酶染色多为阴性。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 16, "end_idx": 35, "type": "sym", "entity": "肠壁神经节细胞数量和神经纤维密度显著减少" }, { "start_idx": 37, "end_idx": 42, "type": "ite", "entity": "乙酰胆碱酯酶" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "染色" } ] }, { "text": "肠肌间神经丛内的神经细胞数少于正常的50%,神经节间距为正常的2倍,神经节平均面积较正常小3倍。", "entities": [ { "start_idx": 0, "end_idx": 20, "type": "sym", "entity": "肠肌间神经丛内的神经细胞数少于正常的50%" }, { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "神经节间距为正常的2倍" }, { "start_idx": 34, "end_idx": 46, "type": "sym", "entity": "神经节平均面积较正常小3倍" } ] }, { "text": "单纯神经节细胞减少症与HD、肠神经发育不良(IND)伴发神经节细胞减少的组化检查相同。", "entities": [ { "start_idx": 0, "end_idx": 41, "type": "sym", "entity": "单纯神经节细胞减少症与HD、肠神经发育不良(IND)伴发神经节细胞减少的组化检查相同" } ] }, { "text": "神经节细胞减少症通常仅累及一小段结肠,但偶可累及全部肠段。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "结肠" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肠段" } ] }, { "text": "因此,术中冷冻活检中发现少量神经节细胞并不能证明肠段神经发育或功能正常。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "冷冻活检" }, { "start_idx": 12, "end_idx": 18, "type": "bod", "entity": "少量神经节细胞" } ] }, { "text": "神经节细胞减少症往往存在肛门内括约肌松弛功能不全或缺如。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 12, "end_idx": 23, "type": "dis", "entity": "肛门内括约肌松弛功能不全" } ] }, { "text": "(四)神经节细胞不成熟LDH染色可发现未成熟的单极小神经节细胞,胞浆内不含脱氧酶,因此不能与Schwann细胞区分。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "神经节细胞" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "LDH染色" }, { "start_idx": 23, "end_idx": 30, "type": "bod", "entity": "单极小神经节细胞" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "胞浆内不含脱氧酶" }, { "start_idx": 46, "end_idx": 54, "type": "bod", "entity": "Schwann细胞" } ] }, { "text": "琥珀酸脱氢酶(SDH)反应可测定神经节细胞成熟度,正常新生儿生后一周内琥珀酸脱氢酶活性较低;而神经节细胞完全成熟需2~4年。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "琥珀酸脱氢酶" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "SDH" }, { "start_idx": 16, "end_idx": 20, "type": "ite", "entity": "神经节细胞" }, { "start_idx": 35, "end_idx": 40, "type": "bod", "entity": "琥珀酸脱氢酶" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "神经节细胞" } ] }, { "text": "IND或神经节细胞减少症患儿肠段内均可见不成熟神经节细胞。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "IND" }, { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 18, "end_idx": 27, "type": "sym", "entity": "可见不成熟神经节细胞" } ] }, { "text": "神经节细胞减少症和神经节发育不成熟统称神经节发育不全。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "神经节发育不成熟" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "神经节发育不全" } ] }, { "text": "(五)肠神经发育不良(IND)与神经节细胞缺乏症和神经节细胞减少症相同,肠肌间丛神经节细胞发育不良既可并发于典型HD,亦可独立发生,导致肠梗阻,可分为以支配血管的肾上腺素能神经异常为特征的A型,以及以黏液下神经丛受累为主的B型。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "肠神经发育不良" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "IND" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "神经节细胞缺乏症" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 36, "end_idx": 48, "type": "dis", "entity": "肠肌间丛神经节细胞发育不良" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "HD" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 100, "end_idx": 109, "type": "sym", "entity": "黏液下神经丛受累为主" } ] }, { "text": "肠壁内发现LDH染色阳性的巨大神经节是B型IND的诊断依据,多为7~10个,偶尔可达16个。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肠壁" }, { "start_idx": 5, "end_idx": 9, "type": "ite", "entity": "LDH染色" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "巨大神经节" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "IND" } ] }, { "text": "这些巨大神经节占所见全部神经节的60%,可呈异常的树芽状,并有串珠样神经纤维,一般不累及直肠远端。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "巨大神经节" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "神经节" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "神经纤维" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "直肠远端" } ] }, { "text": "此外,黏液肌层与固有层常可发现异位神经细胞或神经节。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "黏液肌层" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "神经细胞" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "神经节" } ] }, { "text": "权威认定必须观察连续40张切片的LDH反应,其中30%~55%切片黏液下层无神经节,1/4切片有巨大神经节,且至少要观察到4个巨大神经节才能确诊。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "黏液" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "神经节" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "巨大神经节" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "巨大神经节" } ] }, { "text": "但是,至今仍无统一标准,因而给IND的诊断带来了困难。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "IND" } ] }, { "text": "4岁以上患儿IND往往与神经节细胞减少症、肌间神经丛发育不良和异位相伴发。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "患儿IND" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 21, "end_idx": 32, "type": "dis", "entity": "肌间神经丛发育不良和异位" } ] }, { "text": "对肛门闭锁患儿的研究显示,先天性肠梗阻部位近端亦存在IND,表现为神经肌肉肥大。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "肛门闭锁" }, { "start_idx": 13, "end_idx": 20, "type": "bod", "entity": "先天性肠梗阻部位" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "IND" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "神经肌肉肥大" } ] }, { "text": "A型罕见,仅占肠神经异常疾病的2%,特点是肠肌间神经丛、动脉血管和黏液肾上腺素能神经缺乏或发育不成熟。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "肠神经异常" }, { "start_idx": 21, "end_idx": 49, "type": "sym", "entity": "肠肌间神经丛、动脉血管和黏液肾上腺素能神经缺乏或发育不成熟" } ] }, { "text": "(六)结肠结缔组织病近年来认为结肠结缔组织病是导致神经节细胞异位的原因之一,表现为肠管肌层的结缔组织完全或部分缺损,导致肠道蠕动功能受损。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "结肠结缔组织病" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "结肠结缔组织病" }, { "start_idx": 25, "end_idx": 29, "type": "ite", "entity": "神经节细胞" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "肠管" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "结缔组织" }, { "start_idx": 60, "end_idx": 67, "type": "sym", "entity": "肠道蠕动功能受损" } ] }, { "text": "(七)获得性神经节细胞损害获得性无神经节细胞症和神经节细胞减少症的原因可分为血管性或非血管性:非血管性疾病包括美洲锥虫病(Chagasdisease,恰加斯病)、维生素B1缺乏症以及慢性感染(如结核病等)。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "获得性神经节细胞损害" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "获得性无神经节细胞症" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "非血管性疾病" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "美洲锥虫病" }, { "start_idx": 61, "end_idx": 73, "type": "dis", "entity": "Chagasdisease" }, { "start_idx": 75, "end_idx": 78, "type": "dis", "entity": "恰加斯病" }, { "start_idx": 81, "end_idx": 94, "type": "dis", "entity": "维生素B1缺乏症" }, { "start_idx": 97, "end_idx": 100, "type": "dis", "entity": "慢性感染" }, { "start_idx": 103, "end_idx": 105, "type": "dis", "entity": "结核病" } ] }, { "text": "缺血性神经节细胞损害可由于拖出肠段血供不足引起,如HD根治术拖出肠段有张力,动静脉血管受牵拉,或肠系膜血管受损等。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "缺血性神经节细胞损害" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "肠段血供不足引起" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "HD" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肠段" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "肠段有张力" }, { "start_idx": 38, "end_idx": 45, "type": "sym", "entity": "动静脉血管受牵拉" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "肠系膜血管受损" } ] }, { "text": "Swenson、Duhame和Soave手术均需拖出结肠,因此,术中注意保护血供相当重要。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "Swenson" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "Duhame" }, { "start_idx": 15, "end_idx": 21, "type": "pro", "entity": "Soave手术" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "结肠" } ] }, { "text": "【伴发畸形】11%~30%的HD患儿有伴发畸形。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "HD" } ] }, { "text": "但对HD患儿进行临床遗传学筛查则发现伴发畸形可高达48%。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "HD" } ] }, { "text": "对一组203例患儿调查发现其中11%有相关家族史,35%有伴发畸形,其中泌尿生殖道(11%)最为常见,其次为心血管系统(6%)和胃肠道系统(6%)畸形,另有8%为其他系统,包括白内障、四肢残疾或脑组织缺损等;HD患儿中10%为早产儿。", "entities": [ { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "畸形" }, { "start_idx": 54, "end_idx": 74, "type": "dis", "entity": "心血管系统(6%)和胃肠道系统(6%)畸形" }, { "start_idx": 88, "end_idx": 90, "type": "dis", "entity": "白内障" }, { "start_idx": 92, "end_idx": 95, "type": "dis", "entity": "四肢残疾" }, { "start_idx": 97, "end_idx": 101, "type": "dis", "entity": "脑组织缺损" }, { "start_idx": 104, "end_idx": 105, "type": "dis", "entity": "HD" } ] }, { "text": "HD患者还可能因直肠扩张压迫膀胱颈,导致膀胱颈梗阻、膀胱增大,引起排尿异常。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "HD" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "直肠" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "膀胱颈" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "膀胱颈梗阻" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "膀胱增大" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "排尿异常" } ] }, { "text": "患儿的膀胱功能类似于脊髓损伤患者,术后可能仍需导尿。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "脊髓损伤" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "导尿" } ] }, { "text": "正常人群心血管异常的发生率为0.5%~1%,而HD患者为2%~8%,主要与部分患者伴发Down综合征,常有心内膜垫发育异常有关。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "心血管异常" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "HD" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "Down综合征" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "心内膜垫" } ] }, { "text": "HD患者眼发育异常发生率为12%,包括小眼畸形和无眼畸形。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "HD" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "小眼畸形" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "无眼畸形" } ] }, { "text": "(一)21-三体综合征(Down综合征)据报道3%的HD患儿患有Down综合征,是正常人群发病率的4倍。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "Down综合征" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "HD" } ] }, { "text": "而Down综合征患儿也可因甲状腺功能减退、肌无力或精神发育迟缓导致便秘,给鉴别带来困难。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "Down综合征" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "甲状腺功能减退" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "肌无力" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "精神发育迟缓" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "便秘" } ] }, { "text": "(二)瓦登伯格(Waardenburg)和其他综合征胃肠道交感神经节起源于神经外胚层细胞,神经嵴细胞从神经管迁徙至胃肠道形成肠神经系统(ENS),以来自第4、5体节的为主;位于第3体节尾端的神经嵴则发育为结肠ENS。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "瓦登伯格" }, { "start_idx": 8, "end_idx": 18, "type": "dis", "entity": "Waardenburg" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "胃肠道交感神经节" }, { "start_idx": 37, "end_idx": 43, "type": "bod", "entity": "神经外胚层细胞" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "神经嵴细胞" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "神经管" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "胃肠道" }, { "start_idx": 62, "end_idx": 66, "type": "bod", "entity": "肠神经系统" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "ENS" }, { "start_idx": 95, "end_idx": 97, "type": "bod", "entity": "神经嵴" }, { "start_idx": 102, "end_idx": 106, "type": "bod", "entity": "结肠ENS" } ] }, { "text": "研究表明,ENS前体可能沿腹外侧途径迁徙,而由菱脑神经嵴至第3体节尾端来源的细胞则经背外侧途径迁徙至咽弓。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "ENS" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "菱脑神经嵴" }, { "start_idx": 29, "end_idx": 39, "type": "bod", "entity": "第3体节尾端来源的细胞" } ] }, { "text": "当神经嵴细胞迁徙至胃肠道时,在肠道组织发出信号的指引下,在适当的时间与部位停止迁徙,开始形成神经节。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "神经嵴细胞" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "胃肠道" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "肠道组织" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "神经节" } ] }, { "text": "存在于黏液肌层、浆膜上皮与肠道平滑肌层内的层粘连蛋白可能即是此类信号之一,属于细胞外基质分子。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "黏液肌层" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "浆膜上皮" }, { "start_idx": 13, "end_idx": 25, "type": "bod", "entity": "肠道平滑肌层内的层粘连蛋白" }, { "start_idx": 39, "end_idx": 45, "type": "bod", "entity": "细胞外基质分子" } ] }, { "text": "当神经嵴细胞迁徙至肠道时即获得层粘连蛋白受体,并与层粘连蛋白相互作用,决定神经嵴细胞的停留位置,但究竟如何指导形成神经节细胞,机制尚未明了。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "神经嵴细胞" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肠道" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "层粘连蛋白受体" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "层粘连蛋白" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "神经嵴细胞" }, { "start_idx": 57, "end_idx": 61, "type": "ite", "entity": "神经节细胞" } ] }, { "text": "Waardenburg综合征患者几乎所有的神经嵴细胞均形成黑素细胞,因此临床特征表现为色素沉着异常,此外还有内耳失听及面部发育异常。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "dis", "entity": "Waardenburg综合征" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "神经嵴细胞" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "黑素细胞" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "色素沉着异常" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "内耳失听" }, { "start_idx": 59, "end_idx": 64, "type": "sym", "entity": "面部发育异常" } ] }, { "text": "Shah-Waardenburg综合征是伴发HD的一型Waardenburg综合征,主要由SOX10基因突变所致,可能属于常染色体显性遗传。", "entities": [ { "start_idx": 0, "end_idx": 18, "type": "dis", "entity": "Shah-Waardenburg综合征" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "HD" }, { "start_idx": 27, "end_idx": 40, "type": "dis", "entity": "Waardenburg综合征" }, { "start_idx": 45, "end_idx": 51, "type": "bod", "entity": "SOX10基因" }, { "start_idx": 61, "end_idx": 68, "type": "sym", "entity": "常染色体显性遗传" } ] }, { "text": "(三)肠道伴发畸形HD、神经节细胞缺乏症及IND也可伴有先天性小肠或结肠闭锁、胎粪性腹膜炎或无肛等。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "肠道伴发畸形HD" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "神经节细胞缺乏症" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "IND" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "先天性小肠" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "结肠闭锁" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "胎粪性腹膜炎" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "无肛" } ] }, { "text": "一组伴有小肠闭锁的19例HD患者,其中5例为神经节细胞减少症,2例为IND。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "HD" }, { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "神经节细胞减少症" } ] }, { "text": "52例肛门直肠畸形患者中,一半以上存在ENS异常,9例直肠活体组织检查标本为无神经节细胞症,11例为神经节细胞减少症,4例为B型IND,3例为神经节发育不良,仅2例患者(4%)的瘘管和直肠标本的神经支配正常。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "肛门直肠畸形" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "ENS异常" }, { "start_idx": 27, "end_idx": 34, "type": "pro", "entity": "直肠活体组织检查" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "神经节细胞症" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "B型IND" }, { "start_idx": 71, "end_idx": 77, "type": "dis", "entity": "神经节发育不良" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "瘘管" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "直肠" }, { "start_idx": 97, "end_idx": 100, "type": "dis", "entity": "神经支配" } ] }, { "text": "【临床表现】便秘是巨结肠最主要的症状,但严重程度存在明显的个体差异。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "巨结肠" } ] }, { "text": "有些患儿由于腹胀和粪块积聚导致厌食及恶病质等,出现贫血和低蛋白血症,影响生长发育。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "腹胀" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "粪块积聚" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "厌食及恶病质" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "贫血" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "低蛋白血症" } ] }, { "text": "生后3个月以内诊断为HD的患儿,约1/3常有腹泻,可能与小肠结肠炎有关。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "HD" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "小肠结肠炎" } ] }, { "text": "由于至今尚无HD相关小肠结肠炎的确切定义,有人认为单纯腹泻即是较轻度的小肠结肠炎,而另一些作者则认为只有出现黏液溃疡和败血症才能称之为小肠结肠炎。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "HD" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "小肠结肠炎" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "腹泻" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "小肠结肠炎" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "黏液溃疡" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "败血症" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "小肠结肠炎" } ] }, { "text": "HD患者小肠结肠炎的发生率为12%~58%。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "HD" }, { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "小肠结肠炎" } ] }, { "text": "相关的发病机制有多种假说,如粪便积聚致黏液缺血和细菌侵入与移位、黏蛋白成分变化和黏液防御机制丧失、小肠神经内分泌细胞数量改变、前列腺素E1活性增高和难辨梭状芽孢杆菌或轮状病毒感染等。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "细菌" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "黏蛋白" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "黏液" }, { "start_idx": 49, "end_idx": 57, "type": "bod", "entity": "小肠神经内分泌细胞" }, { "start_idx": 63, "end_idx": 68, "type": "bod", "entity": "前列腺素E1" }, { "start_idx": 80, "end_idx": 87, "type": "bod", "entity": "难辨梭状芽孢杆菌" }, { "start_idx": 89, "end_idx": 94, "type": "bod", "entity": "轮状病毒感染" } ] }, { "text": "小肠结肠炎的致病机制至今未明,有患者甚至在行粪便改道及结肠造瘘术后还有持久性的小肠结肠炎症状。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "小肠结肠炎" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "粪便改道" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "结肠造瘘" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "小肠结肠炎" } ] }, { "text": "【诊断】ENS是小肠的“中枢”,与大脑相似,即使存在某些缺陷,一定程度上仍具有自主功能。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "ENS" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "小肠" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "大脑" } ] }, { "text": "因此,既可以婴儿期肠梗阻表现收治入院,也可以儿童或成人期慢性便秘就诊。", "entities": [ { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "慢性便秘" } ] }, { "text": "(一)影像学检查仰卧位及直立位腹部平片发现结肠有液平,应怀疑HD,常规需行钡剂灌肠检查。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "影像学检查" }, { "start_idx": 8, "end_idx": 25, "type": "sym", "entity": "仰卧位及直立位腹部平片发现结肠有液平" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "HD" }, { "start_idx": 36, "end_idx": 42, "type": "pro", "entity": "行钡剂灌肠检查" } ] }, { "text": "新生儿在钡剂灌肠前不必直肠指检或清洁灌肠,以免出现假阴性结果。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "钡剂灌肠" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "直肠指检" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "清洁灌肠" } ] }, { "text": "怀疑存在胎粪性肠梗阻或者胎粪栓塞综合征时,通常应选用水溶性造影剂取代钡剂以防止加重梗阻。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "胎粪性肠梗阻" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "胎粪栓塞综合征" }, { "start_idx": 26, "end_idx": 31, "type": "dru", "entity": "水溶性造影剂" } ] }, { "text": "经典的X线表现为:直肠口径正常或远端狭窄,移行段呈漏斗型扩张,近端结肠明显扩张。", "entities": [ { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "直肠口径正常或远端狭窄" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "移行段呈漏斗型扩张" }, { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "近端结肠明显扩张" } ] }, { "text": "不能确诊的病例,可在24小时后重复摄片,如结肠中仍有造影剂潴留则有助诊断。", "entities": [ { "start_idx": 21, "end_idx": 30, "type": "sym", "entity": "结肠中仍有造影剂潴留" } ] }, { "text": "有10%HD和29%伴发HD的B型IND患儿,钡剂灌肠检查无法确诊。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "HD" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "HD" }, { "start_idx": 23, "end_idx": 28, "type": "pro", "entity": "钡剂灌肠检查" } ] }, { "text": "全结肠无神经节细胞患儿,结肠可无典型狭窄表现,但若观察到钡剂反流入扩张回肠则有助诊断。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "全结肠无神经节细胞" }, { "start_idx": 12, "end_idx": 19, "type": "sym", "entity": "结肠可无典型狭窄" } ] }, { "text": "小肠结肠炎患儿的腹部平片可见肠壁增厚、黏液不规则以及肠曲明显扩张,由于肠道肌肉功能受炎症破坏,有正常神经支配的移行段结肠一般亦无典型表现。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "肠壁增厚" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "黏液不规则" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "肠曲明显扩张" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "肠道肌肉" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "移行段结肠" } ] }, { "text": "运用放射性标记物评估肠道排空时间,有助于确定肠道病变的最高部位,确定手术切除的长度,判断可能伴发的肠神经元异常,如B型IND或神经节细胞缺乏的临床特征。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "放射性标记物" }, { "start_idx": 10, "end_idx": 15, "type": "ite", "entity": "肠道排空时间" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肠道" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "肠神经元异常" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "B型IND" } ] }, { "text": "该方法也可用于根治术前已行肠造瘘的患儿,明确从远端造口到肛门排泄所需时间。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肠造瘘" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "远端造口" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肛门" } ] }, { "text": "(二)直肠肛管测压直肠肛管测压诊断HD的准确率高达85%。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "直肠肛管测压" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "直肠肛管测压" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "HD" } ] }, { "text": "正常情况下,气囊充盈扩张直肠,可使内括约肌松弛。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "内括约肌" } ] }, { "text": "神经节细胞缺乏患儿可见特异性多节段收缩波形;直肠气囊扩张时,肛管或直肠缺乏抑制性反应,直肠肛管测压直肠肛管测压曲线上升。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "神经节细胞缺乏" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "特异性多节段收缩波形" }, { "start_idx": 30, "end_idx": 41, "type": "sym", "entity": "肛管或直肠缺乏抑制性反应" }, { "start_idx": 43, "end_idx": 48, "type": "pro", "entity": "直肠肛管测压" }, { "start_idx": 49, "end_idx": 58, "type": "sym", "entity": "直肠肛管测压曲线上升" } ] }, { "text": "神经节细胞减少症或IND患者则可能表现为抑制性反应低下或消失。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "IND" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "抑制性反应低下或消失" } ] }, { "text": "但直肠肛管测压一般不用于新生儿,因为即使正常婴儿,直肠括约肌反射需在生后12天才发育完全;而那些胎龄<39周或体重<2.7kg的婴儿则尚未形成该反射。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "直肠肛管测压" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "直肠括约肌" } ] }, { "text": "总体而言,直肠肛管测压是筛查HD及相关疾病一种有效手段。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "直肠肛管测压" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "HD" } ] }, { "text": "(三)直肠活体组织检查直肠活体组织检查可确诊HD。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "pro", "entity": "直肠活体组织检查" }, { "start_idx": 11, "end_idx": 18, "type": "pro", "entity": "直肠活体组织检查" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "HD" } ] }, { "text": "理想的活检标本直径约为3.5mm,且包含黏液下层,但有吸引活检导致出血和穿孔的报道。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "黏液" } ] }, { "text": "新生儿神经节细胞发育不成熟,需采用特殊染色技术(LDH及SDH还原反应),否则可能无法识别;吸引活检标本也可能不能完全反映受累肠段的真实情况,易误诊为神经节细胞缺乏症,导致不必要的肠切除。", "entities": [ { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "特殊染色技术" }, { "start_idx": 24, "end_idx": 26, "type": "pro", "entity": "LDH" }, { "start_idx": 28, "end_idx": 34, "type": "pro", "entity": "SDH还原反应" }, { "start_idx": 46, "end_idx": 49, "type": "pro", "entity": "吸引活检" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "肠段" }, { "start_idx": 75, "end_idx": 82, "type": "dis", "entity": "神经节细胞缺乏症" }, { "start_idx": 90, "end_idx": 90, "type": "bod", "entity": "肠" } ] }, { "text": "若标本仅包含黏液下丛,则可能漏诊神经节细胞减少症。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "黏液" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "神经节细胞减少症" } ] }, { "text": "因此若怀疑神经节细胞减少症或肠肌间神经丛异位,建议应取肠壁全层标本。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "神经节细胞减少症" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "肠肌间神经丛" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "肠壁全层" } ] }, { "text": "【鉴别诊断】临床上往往很难鉴别新生儿胎粪栓塞综合征、左半小结肠综合征、HD及相关ENS病。", "entities": [ { "start_idx": 15, "end_idx": 24, "type": "dis", "entity": "新生儿胎粪栓塞综合征" }, { "start_idx": 26, "end_idx": 33, "type": "dis", "entity": "左半小结肠综合征" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "HD" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "相关ENS病" } ] }, { "text": "巨膀胱-小结肠-肠蠕动不全综合征是导致新生儿慢性肠梗阻的一种罕见而致命的疾病,至今报道不足70例,病理表现为平滑肌细胞退行性变与大量结缔组织增生,但根本病因尚不清楚。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "dis", "entity": "巨膀胱-小结肠-肠蠕动不全综合征" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "新生儿慢性肠梗阻" }, { "start_idx": 54, "end_idx": 71, "type": "sym", "entity": "平滑肌细胞退行性变与大量结缔组织增生" } ] }, { "text": "腹肌松弛和膀胱扩张导致腹胀,可伴有小肠旋转不良和结肠蠕动功能不良。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "腹肌松弛和膀胱扩张" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "腹胀" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "小肠旋转不良" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "结肠蠕动功能不良" } ] }, { "text": "【治疗】包括胃肠减压、造瘘术及根治手术等。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "胃肠减压" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "造瘘术" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "根治手术" } ] }, { "text": "(一)减压有肠梗阻表现的新生儿应立即放置鼻胃管,并置肛管行清洁灌肠,反复刺激排便。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "减压" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "放置鼻胃管" }, { "start_idx": 25, "end_idx": 32, "type": "pro", "entity": "置肛管行清洁灌肠" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "刺激排便" } ] }, { "text": "影像学、直肠测压和活体组织标本组化分析明确诊断后,若有必要,需行结肠造瘘术。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "影像学" }, { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "直肠测压" }, { "start_idx": 9, "end_idx": 16, "type": "pro", "entity": "活体组织标本组化" } ] }, { "text": "(二)结肠造瘘术新生儿HD肠梗阻时,应先考虑行肠造瘘术,一般认为新生儿期以后行根治术效果更佳。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "结肠造瘘术" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "HD肠梗阻" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "肠造瘘术" } ] }, { "text": "造瘘术前必须行清洁灌肠,造瘘口一般位于右侧横结肠,若病变累及全部或几乎全部结肠,必须行回肠造瘘术。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "造瘘术" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "清洁灌肠" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "右侧横结肠" }, { "start_idx": 43, "end_idx": 47, "type": "pro", "entity": "回肠造瘘术" } ] }, { "text": "(三)根治手术巨结肠根治术是最为有效的治疗手段。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "根治手术" } ] }, { "text": "1948年,Swenson和Bill首次施行了无神经节细胞肠段切除术。", "entities": [ { "start_idx": 23, "end_idx": 33, "type": "pro", "entity": "无神经节细胞肠段切除术" } ] }, { "text": "以此为基础,逐步发展了其他四种基本术式:Swenson术式、Duhamel-GROB术式、Rehbein术式和Soave术式。", "entities": [ { "start_idx": 20, "end_idx": 28, "type": "pro", "entity": "Swenson术式" }, { "start_idx": 30, "end_idx": 43, "type": "pro", "entity": "Duhamel-GROB术式" }, { "start_idx": 45, "end_idx": 53, "type": "pro", "entity": "Rehbein术式" }, { "start_idx": 55, "end_idx": 61, "type": "pro", "entity": "Soave术式" } ] }, { "text": "随着微创外科的发展及器械的改良,腹腔镜技术也已应用于巨结肠根治术。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "腹腔镜技术" }, { "start_idx": 26, "end_idx": 31, "type": "pro", "entity": "巨结肠根治术" } ] }, { "text": "在此基础上又出现了经肛门游离直肠和结肠,并将正常段结肠从肛门拖下、成形的方法。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "bod", "entity": "经肛门游离直肠" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肛门" } ] }, { "text": "主要适用于小婴儿和无神经节细胞肠段比较短的患儿,由于不进腹腔,具有手术创伤小、并发症少及恢复快的优点。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "神经节细胞肠段" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "腹腔" } ] }, { "text": "【预后】先天性巨结肠尽管病因不明,但常见的普通型病变在诊断和治疗上已比较定型,效果也比较肯定。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "先天性巨结肠" } ] }, { "text": "长段型和其他一些少见类型的处理尚不尽如人意,巨结肠并发小肠结肠炎仍是临床面临的非常棘手的问题。", "entities": [ { "start_idx": 22, "end_idx": 31, "type": "dis", "entity": "巨结肠并发小肠结肠炎" } ] }, { "text": "三、异体造血干细胞移植供体的选择(一)人类白细胞抗原(HLA)基本理论供受体之间主要组织相容性复合物(majorhistocompatibilitycomplex,MHC)的不同是移植排异反应的基础。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "异体造血干细胞移植" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "白细胞抗原" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "HLA" }, { "start_idx": 41, "end_idx": 50, "type": "bod", "entity": "主要组织相容性复合物" }, { "start_idx": 52, "end_idx": 81, "type": "bod", "entity": "majorhistocompatibilitycomplex" }, { "start_idx": 83, "end_idx": 85, "type": "bod", "entity": "MHC" }, { "start_idx": 91, "end_idx": 96, "type": "dis", "entity": "移植排异反应" } ] }, { "text": "MHC为一组抗原基因,在人类称为HLA系统,位于第6对染色体的p21区。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "MHC" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "抗原基因" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "HLA系统" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "染色体" } ] }, { "text": "此类基因集中在三个区,即Ⅰ、Ⅱ、Ⅲ类抗原区。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "基因" } ] }, { "text": "与移植排斥反应相关的主要是第Ⅰ、Ⅱ类MHC抗原。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "移植排斥反应" }, { "start_idx": 13, "end_idx": 22, "type": "bod", "entity": "第Ⅰ、Ⅱ类MHC抗原" } ] }, { "text": "Ⅰ类MHC抗原包括HLA-A、B、C,近来又发现E、F、G三类。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "Ⅰ类MHC抗原" }, { "start_idx": 9, "end_idx": 17, "type": "bod", "entity": "HLA-A、B、C" } ] }, { "text": "Ⅱ类MHC抗原包括HLA-D、DR、DQ、DP抗原。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "Ⅱ类MHC抗原" }, { "start_idx": 9, "end_idx": 24, "type": "bod", "entity": "HLA-D、DR、DQ、DP抗原" } ] }, { "text": "(二)HLA相配几率及移植物抗宿主病发生率1.同基因异体移植同卵双胎间HLA基因完全相配,基本无移植物抗宿主病(graft-versus-hostdisease,GVHD)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "HLA" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "抗宿主病" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "同基因异体移植" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "HLA基因" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "抗宿主病" }, { "start_idx": 56, "end_idx": 79, "type": "dis", "entity": "graft-versus-hostdisease" }, { "start_idx": 81, "end_idx": 84, "type": "dis", "entity": "GVHD" } ] }, { "text": "2.同胞HLA相合供者同胞间HLA相合机会为25%,其HLA相合可分为两种情况,即HLA表型相合及HLA基因型相合,其中多数为后者。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "HLA" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "HLA" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "HLA" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "HLA" }, { "start_idx": 49, "end_idx": 53, "type": "bod", "entity": "HLA基因" } ] }, { "text": "HLA完全相合者,GVHD较轻。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "HLA" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "GVHD" } ] }, { "text": "3.家族HLA不完全相合供者包括1个、2个或3个位点不合,GVHD发病率与不相合位点成比例,不相合位点越多越易移植失败。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "HLA" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "GVHD" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "移植" } ] }, { "text": "4.无关供者约1万~2万无关供者中可找到1个HLA完全相配者,GVHD发生的严重度一般较同胞相合供体移植重。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "HLA" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "GVHD" }, { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "移植" } ] }, { "text": "第四章白血病第一节儿童急性淋巴细胞白血病儿童急性淋巴细胞白血病(acutelymphoblasticleukemia,ALL)是最常见的儿童肿瘤性疾病。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "白血病" }, { "start_idx": 9, "end_idx": 19, "type": "dis", "entity": "儿童急性淋巴细胞白血病" }, { "start_idx": 20, "end_idx": 30, "type": "dis", "entity": "儿童急性淋巴细胞白血病" }, { "start_idx": 32, "end_idx": 57, "type": "dis", "entity": "acutelymphoblasticleukemia" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "ALL" }, { "start_idx": 68, "end_idx": 74, "type": "dis", "entity": "儿童肿瘤性疾病" } ] }, { "text": "近40年来,特别是20世纪80年代以后,对儿童ALL的基础和临床研究取得了巨大的成就,儿童ALL已成为可以治愈的恶性肿瘤,是当今疗效最好、治愈率最高的恶性肿瘤性疾病之一,给其他儿童恶性肿瘤的治疗带来了信心和合理的临床研究模式。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "儿童ALL" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "儿童ALL" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 75, "end_idx": 81, "type": "dis", "entity": "恶性肿瘤性疾病" }, { "start_idx": 88, "end_idx": 93, "type": "dis", "entity": "儿童恶性肿瘤" } ] }, { "text": "目前小儿ALL的完全缓解(CR)率可达95%以上,5年以上持续完全缓解(CCR)率可达65%~80%。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "小儿ALL" } ] }, { "text": "【发病率及流行病学】在儿童肿瘤性疾病中,ALL发病率最高,发病高峰年龄约为4岁。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "儿童肿瘤性疾病" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "ALL" } ] }, { "text": "根据上海市肿瘤登记系统1986~1992年资料,0~14岁组ALL发病数占儿童肿瘤总发病数的20%,年发病率为20.90/100万,0~14岁累积发病率为312.55/100万,男性发病率高于女性,男∶女为1.5∶1。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "ALL" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "【病因学】可能导致发生儿童白血病的因素包括遗传、环境、病毒感染、免疫缺陷因素,但对每一个白血病患儿来说常不能确定其个体的致病原因。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "儿童白血病" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "病毒感染" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "白血病" } ] }, { "text": "(一)环境因素接触电离辐射有利于白血病的发生,第二次世界大战时日本发生原子弹爆炸后,当地白血病发病率增高即证实这一点。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "白血病" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "白血病" } ] }, { "text": "接触治疗性辐射也增加白血病的发病率。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "白血病" } ] }, { "text": "长期接触苯等有毒化学物品与急性非淋巴细胞白血病有关。", "entities": [ { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "急性非淋巴细胞白血病" } ] }, { "text": "其他与ALL发病可能有关的化学物品有除草剂、杀虫剂、孕妇酗酒、避孕药、烟草及化学溶剂,但这些因素与ALL发病的确切关系尚不肯定。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "ALL" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "避孕药" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "ALL" } ] }, { "text": "(二)病毒感染EB病毒感染可能与L3型ALL相关,也有ALL发病与HIV感染相关的病例报告。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "病毒感染" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "EB病毒感染" }, { "start_idx": 16, "end_idx": 27, "type": "dis", "entity": "L3型ALL" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "ALL" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "HIV感染" } ] }, { "text": "(三)免疫缺陷先天性免疫缺陷者淋巴系统恶性肿瘤的发病率增高。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "先天性免疫缺陷" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "淋巴系统恶性肿瘤" } ] }, { "text": "(四)先天性基因(遗传)因素有报告白血病患儿(包括ALL)同胞的白血病发病率比普通人群高2~4倍。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "白血病" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "ALL" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "白血病" } ] }, { "text": "单卵双胎中一个发生白血病后,另一个发生白血病的机会高达25%;发病年龄越小,另一个发病的机会越高;当发病年龄>7岁时,另一个发病的机会明显减少。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "白血病" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "白血病" } ] }, { "text": "说明白血病的发生可以有先天性遗传因素参与,但确切的基因因素尚未十分明了。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "白血病" } ] }, { "text": "【临床表现】各类型小儿急性白血病临床表现相似,通常表现为进行性苍白、乏力、食欲减退、盗汗、虚弱、低热和出血倾向。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "急性白血病" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "进行性苍白" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "乏力" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "食欲减退" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "盗汗" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "虚弱" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "低热和出血倾向" } ] }, { "text": "从起病到诊断可长达数月,也可以骤然起病,以不规则发热、急速的进行性苍白、明显的出血症状和骨关节疼痛等症贫血、出血、发热和白血病细胞对全身各脏器浸润引起的症状贫血常早期出现,轻重不等,表现为苍白、乏力、气促、心悸、颜面水肿等,可进行性加重,贫血和出血程度常不成比例。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "不规则发热" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "急速的进行性苍白" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "骨关节" }, { "start_idx": 36, "end_idx": 50, "type": "sym", "entity": "明显的出血症状和骨关节疼痛等症" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "贫血" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "出血" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "白血病细胞" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "脏器" }, { "start_idx": 57, "end_idx": 77, "type": "sym", "entity": "发热和白血病细胞对全身各脏器浸润引起的症状" }, { "start_idx": 78, "end_idx": 79, "type": "sym", "entity": "贫血" }, { "start_idx": 94, "end_idx": 95, "type": "sym", "entity": "苍白" }, { "start_idx": 97, "end_idx": 98, "type": "sym", "entity": "乏力" }, { "start_idx": 100, "end_idx": 101, "type": "sym", "entity": "气促" }, { "start_idx": 103, "end_idx": 104, "type": "sym", "entity": "心悸" }, { "start_idx": 106, "end_idx": 110, "type": "sym", "entity": "颜面水肿等" }, { "start_idx": 119, "end_idx": 120, "type": "sym", "entity": "贫血" }, { "start_idx": 122, "end_idx": 123, "type": "sym", "entity": "出血" } ] }, { "text": "(二)出血极大部分患儿均有不同程度的皮肤和黏膜出血,表现为皮肤紫癜、乌青和瘀斑,甚至发生皮下血肿。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "出血" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "皮肤和黏膜出血" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "皮肤紫癜" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "乌青" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "瘀斑" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "皮下血肿" } ] }, { "text": "齿龈出血、鼻出血、口腔黏膜渗血,严重者可出现眼底视网膜出血,导致视力减退、颅内压增高。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "齿龈" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "齿龈出血" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "鼻" }, { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "鼻出血" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "口腔黏膜" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "口腔黏膜渗血" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "视网膜" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "眼底视网膜出血" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "视力减退" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "颅内压增高" } ] }, { "text": "消化道和泌尿道出血,临床表现为便血、呕血和尿血。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "消化道和泌尿道出血" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "便血" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "呕血" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "尿血" } ] }, { "text": "颅内出血时表现为头痛、呕吐、抽搐和昏迷等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅内出血" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "头痛" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "呕吐" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "抽搐" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "昏迷" } ] }, { "text": "(三)发热与感染多数患儿起病时有不同程度发热,可为低热、不规则发热、持续高热或弛张热,暂时性热退时常大汗淋漓。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "发热" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "感染" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "发热" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "低热" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "不规则发热" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "持续高热" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "弛张热" }, { "start_idx": 43, "end_idx": 53, "type": "sym", "entity": "暂时性热退时常大汗淋漓" } ] }, { "text": "发热的原因包括肿瘤性发热和感染性发热,前者用抗生素治疗无效,而用吲哚美辛0.5mg/kg,每8小时口服,热可退净,以此鉴别肿瘤性发热和感染性发热。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "发热" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "肿瘤性发热" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "感染性发热" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "抗生素" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "肿瘤性发热" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "感染性发热" } ] }, { "text": "常见的感染有:呼吸系统感染、败血症、口腔溃疡、皮肤疖肿、肠道感染等,肛周炎也颇为常见。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "呼吸系统感染" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "败血症" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "口腔溃疡" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "皮肤疖肿" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "肠道感染" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "肛周炎" } ] }, { "text": "常见的病原菌为大肠埃希菌、铜绿假单胞菌、副大肠杆菌等革兰阴性杆菌、金黄色葡萄球菌和表皮葡萄球菌等革兰阳性球菌,其他还有粪链球菌、克雷白菌、阴沟杆菌、硝酸盐阴性杆菌、黏质沙雷菌、弗氏枸橼酸杆菌等条件致病菌和厌氧菌。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "mic", "entity": "大肠埃希菌" }, { "start_idx": 13, "end_idx": 18, "type": "mic", "entity": "铜绿假单胞菌" }, { "start_idx": 20, "end_idx": 24, "type": "mic", "entity": "副大肠杆菌" }, { "start_idx": 26, "end_idx": 31, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 33, "end_idx": 39, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 41, "end_idx": 46, "type": "mic", "entity": "表皮葡萄球菌" }, { "start_idx": 48, "end_idx": 53, "type": "mic", "entity": "革兰阳性球菌" }, { "start_idx": 59, "end_idx": 62, "type": "mic", "entity": "粪链球菌" }, { "start_idx": 64, "end_idx": 67, "type": "mic", "entity": "克雷白菌" }, { "start_idx": 69, "end_idx": 72, "type": "mic", "entity": "阴沟杆菌" }, { "start_idx": 74, "end_idx": 80, "type": "mic", "entity": "硝酸盐阴性杆菌" }, { "start_idx": 82, "end_idx": 86, "type": "mic", "entity": "黏质沙雷菌" }, { "start_idx": 88, "end_idx": 94, "type": "mic", "entity": "弗氏枸橼酸杆菌" }, { "start_idx": 98, "end_idx": 100, "type": "mic", "entity": "致病菌" }, { "start_idx": 102, "end_idx": 104, "type": "mic", "entity": "厌氧菌" } ] }, { "text": "此外可有巨细胞包涵体病毒(CMV)、疱疹病毒、EB病毒感染。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "mic", "entity": "巨细胞包涵体病毒" }, { "start_idx": 13, "end_idx": 15, "type": "mic", "entity": "CMV" }, { "start_idx": 18, "end_idx": 21, "type": "mic", "entity": "疱疹病毒" }, { "start_idx": 23, "end_idx": 26, "type": "mic", "entity": "EB病毒" } ] }, { "text": "真菌感染也较常见,有白色念珠菌引起鹅口疮、肛周真菌症、真菌性肠炎和深部真菌感染。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "真菌感染" }, { "start_idx": 10, "end_idx": 14, "type": "mic", "entity": "白色念珠菌" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "鹅口疮" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "肛周真菌症" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "真菌性肠炎" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "真菌感染" } ] }, { "text": "上述各种感染可单独发生也可混合感染,临床常表现为不规则或弛张性发热。", "entities": [ { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "不规则或弛张性发热" } ] }, { "text": "(四)白血病细胞浸润表现ALL易有网状内皮系统的浸润,表现为肝、脾和淋巴结轻至重度肿大。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "白血病" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "ALL" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "网状内皮系统" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "肝" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "脾" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "淋巴结" }, { "start_idx": 30, "end_idx": 42, "type": "sym", "entity": "肝、脾和淋巴结轻至重度肿大" } ] }, { "text": "骨关节浸润表现为持续性并阵发性加剧的骨、关节疼痛或肿痛,行动受碍,多见于膝、胫骨、胸骨、踝、肩、腕、肘关节处,易被误诊为风湿关节炎或骨髓炎。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "骨关节" }, { "start_idx": 8, "end_idx": 26, "type": "sym", "entity": "持续性并阵发性加剧的骨、关节疼痛或肿痛" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "行动受碍" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "膝" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "胫骨" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "胸骨" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "踝" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "肩" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "腕" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "肘关节" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "风湿关节炎" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "骨髓炎" } ] }, { "text": "中枢神经系统浸润时,可表现为颅内压增高,有头痛、呕吐、视神经乳头水肿所致视力模糊,也可引起面瘫等脑神经损害症,甚至发生癫痫样发作,意识障碍等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "头痛" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "呕吐" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "视神经乳头" }, { "start_idx": 27, "end_idx": 39, "type": "sym", "entity": "视神经乳头水肿所致视力模糊" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "面瘫" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "脑神经损害症" }, { "start_idx": 59, "end_idx": 63, "type": "sym", "entity": "癫痫样发作" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "意识障碍" } ] }, { "text": "腮腺浸润时表现为两侧腮腺无痛性增大,质地较硬,无压痛或轻度压痛。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "腮腺" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "腮腺" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "腮腺无痛性增大" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "质地较硬" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "无压痛或轻度压痛" } ] }, { "text": "睾丸浸润时有单侧或双侧睾丸无痛性肿大,质地坚硬,压痛不明显,透光试验呈阴性。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "睾丸" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "睾丸" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "睾丸无痛性肿大" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "质地坚硬" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "压痛不明显" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "透光试验呈阴性" } ] }, { "text": "ALL时肾浸润并不少见,可因水肿、尿量尿色改变而就诊,有时肾脏明显肿大可在两侧腹部触及,腹部B型超声或CT可见肾脏有多发性浸润灶。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "ALL" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "肾" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "水肿" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "尿量尿色改变" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "肾脏明显肿大" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "腹部" }, { "start_idx": 44, "end_idx": 49, "type": "equ", "entity": "腹部B型超声" }, { "start_idx": 51, "end_idx": 52, "type": "equ", "entity": "CT" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "肾脏" } ] }, { "text": "其他如皮肤、胃肠道、肺、胸膜和心脏浸润时,引起相应脏器功能障碍的症状。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "皮肤" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "胃肠道" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肺" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胸膜" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心脏" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "脏器功能障碍" } ] }, { "text": "【实验室检查及临床分型】(一)外周血象ALL时外周血象红系、髓系和巨核系中常有≥2系的异常变化,多数患儿有贫血和血小板减少。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "ALL" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "贫血" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "血小板减少" } ] }, { "text": "低增生性ALL时白细胞数可很低,外周血象类似再生障碍性贫血,三系均降低,也未见幼稚细胞。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "ALL" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "白细胞" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "再生障碍性贫血" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "幼稚细胞" } ] }, { "text": "高增生性时可高至数十万,较多患儿外周血中可见到幼稚细胞。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "外周血" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "幼稚细胞" } ] }, { "text": "少数患儿可因外周血变化不明显或基本正常而被忽略并延误诊断。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "外周血" } ] }, { "text": "(二)骨髓象(细胞形态学检查)骨髓涂片是确诊白血病的主要依据。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "细胞形态学检查" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "骨髓涂片" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "白血病" } ] }, { "text": "除了白血病细胞明显增生外,有时可伴有不同程度的骨髓纤维组织增生,此时抽取骨髓液较为困难,称之为“干抽”现象。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "白血病" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "骨髓纤维组织" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "骨髓液" } ] }, { "text": "ALL骨髓涂片组织化学染色的典型表现为糖原呈阳性或强阳性,过氧化物酶阴性,非特异性酯酶呈阴性。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "ALL" }, { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "骨髓涂片" }, { "start_idx": 19, "end_idx": 27, "type": "sym", "entity": "糖原呈阳性或强阳性" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "过氧化物酶阴性" }, { "start_idx": 37, "end_idx": 45, "type": "sym", "entity": "非特异性酯酶呈阴性" } ] }, { "text": "根据法国、美国和英国(FAB)形态学分类,ALL分为L1、L2、L3型,90%儿童ALL形态分型为L1,细胞形态较小;L3型肿瘤细胞的形态与Burkitt's淋巴瘤细胞相似;L2细胞大小不均,介于L1和L3之间。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "ALL" }, { "start_idx": 59, "end_idx": 61, "type": "dis", "entity": "ALL" }, { "start_idx": 92, "end_idx": 95, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 109, "end_idx": 113, "type": "bod", "entity": "淋巴瘤细胞" } ] }, { "text": "仅依靠骨髓细胞形态学不能鉴别ALL还是非霍奇金淋巴瘤骨髓浸润。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "骨髓细胞" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "ALL" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "非霍奇金淋巴瘤" } ] }, { "text": "(三)免疫分型根据单克隆抗体(McAb)对白血病细胞表面分化抗原、胞质免疫球蛋白链的反应,可将ALL分为T、B二大系列。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "抗体" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "McAb" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "白血病" }, { "start_idx": 33, "end_idx": 40, "type": "bod", "entity": "胞质免疫球蛋白链" } ] }, { "text": "1.T系淋巴细胞型(T-ALL)约占儿童ALL10%~15%,常表达T淋巴细胞分化抗原标志,如CD1、CD2、CD3、CD4、CD5、CD7、CD8以及TdT等。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "T系淋巴细胞型" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "T-ALL" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "T淋巴细胞" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "抗原" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "CD1" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "CD2" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "CD3" }, { "start_idx": 77, "end_idx": 79, "type": "bod", "entity": "CD4" }, { "start_idx": 87, "end_idx": 89, "type": "bod", "entity": "CD5" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "CD7" }, { "start_idx": 107, "end_idx": 109, "type": "bod", "entity": "CD8" }, { "start_idx": 118, "end_idx": 120, "type": "bod", "entity": "TdT" } ] }, { "text": "临床上可伴有纵隔增宽、外周血白细胞计数高。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "纵隔增宽" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "外周血白细胞计数高" } ] }, { "text": "2.B系淋巴细胞型(B-ALL)约占儿童ALL80%~90%,可大致分为未成熟型(以发育成熟过程依次包括B淋巴祖细胞性、早期前B淋巴细胞性和前B淋巴细胞型)和相对成熟型。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "B系淋巴细胞型" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "B-ALL" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "儿童ALL" } ] }, { "text": "胞质免疫球蛋白(CyIg)从无到有,继之细胞膜表面免疫球蛋白(SmIg)的出现反映了B细胞向成熟方向发育的过程。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "胞质免疫球蛋白" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "CyIg" }, { "start_idx": 20, "end_idx": 29, "type": "bod", "entity": "细胞膜表面免疫球蛋白" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "SmIg" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "B细胞" } ] }, { "text": "B系淋巴细胞其他常用的分化抗原标记有TdT、HLA-DR、CD19、CD22、CD10、CD20以及CD24,其中CD20、CD10出现较晚,至前B淋巴细胞型才出现。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "B系淋巴细胞" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "抗原" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "TdT" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "HLA-DR" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "CD19" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "CD22" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "CD10" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "CD20" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "CD24" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "CD20" }, { "start_idx": 74, "end_idx": 77, "type": "bod", "entity": "CD10" }, { "start_idx": 91, "end_idx": 96, "type": "dis", "entity": "B淋巴细胞型" } ] }, { "text": "成熟B淋巴细胞白血病和B细胞性非霍奇金淋巴瘤细胞一样常表达SmIg。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "B淋巴细胞白血病" }, { "start_idx": 11, "end_idx": 21, "type": "dis", "entity": "B细胞性非霍奇金淋巴瘤" } ] }, { "text": "(四)细胞遗传学检查1.染色体数量异常染色体数<46条时称为低二倍体,当染色体<40条时预后较差。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "细胞遗传学检查" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "染色体" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "染色体" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "染色体" } ] }, { "text": "2.染色体结构异常常见的相对成熟B细胞型ALL染色体异常有t(8;14)、t(2;8)、t(8;22),与B细胞性非霍奇金淋巴瘤相同。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "染色体" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "B细胞型ALL" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "染色体" }, { "start_idx": 53, "end_idx": 63, "type": "dis", "entity": "B细胞性非霍奇金淋巴瘤" } ] }, { "text": "B系未成熟型ALL常见的染色体结构异常有t(11;v)、t(9;22)、t(1;19)、t(4;11)、t(12;21)等。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "ALL" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "染色体" } ] }, { "text": "常见的TALL染色体结构异常有t(11;14)、t(8;14)、t(10;14)、t(1;14)t(4;11)等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "TALL" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "染色体" } ] }, { "text": "现代白血病诊断应包含形态学(morphology,M)、免疫学(immunology,I)、细胞遗传学(cytogenetics,C)和分子生物学(molecularbiology,M)即MICM综合诊断。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "白血病" } ] }, { "text": "ALL还应作出临床危险型诊断,以指导临床治疗方案的选择。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "ALL" } ] }, { "text": "(五)脑脊液检查ALL应常规作脑脊液检查,包括脑脊液常规细胞计数及分类、生化、离心甩片找肿瘤细胞。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "ALL" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 23, "end_idx": 34, "type": "pro", "entity": "脑脊液常规细胞计数及分类" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "生化" }, { "start_idx": 39, "end_idx": 47, "type": "pro", "entity": "离心甩片找肿瘤细胞" } ] }, { "text": "美国国立癌症研究所(NCI)儿童ALL中枢神经系统白血病(CNSL)的诊断标准(表11-11)。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "癌症" }, { "start_idx": 14, "end_idx": 27, "type": "dis", "entity": "儿童ALL中枢神经系统白血病" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "CNSL" } ] }, { "text": "表11-7中枢神经系统白血病的诊断标准(六)影像学检查胸部X线平片可发现是否同时伴有纵隔增宽和肺门淋巴结增大。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "白血病" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "影像学检查" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "胸部X线" }, { "start_idx": 42, "end_idx": 53, "type": "sym", "entity": "纵隔增宽和肺门淋巴结增大" } ] }, { "text": "腹部B型超声或CT可发现部分病例有不同程度的肾脏、肝脏的浸润性病变及腹腔淋巴结肿大骨扫描有异常浓集灶,骨X线平片可有虫蚀样病变或骨骺部白血病线。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "腹部B型超声" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "CT" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肾脏" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肝脏" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "腹腔淋巴结" }, { "start_idx": 17, "end_idx": 40, "type": "sym", "entity": "不同程度的肾脏、肝脏的浸润性病变及腹腔淋巴结肿大" }, { "start_idx": 41, "end_idx": 43, "type": "pro", "entity": "骨扫描" }, { "start_idx": 51, "end_idx": 55, "type": "pro", "entity": "骨X线平片" }, { "start_idx": 58, "end_idx": 70, "type": "sym", "entity": "虫蚀样病变或骨骺部白血病线" } ] }, { "text": "(七)临床危险程度分型小儿ALL有明显影响预后的危险因素,国际上公认的因素包括:①诊断时外周血白细胞计数≥50×109/L;②年龄<1岁或>12岁;③诊断时有CNSL;④染色体核型为t(9;22)或t(4;11)异常者;⑤泼尼松诱导试验60mg/M2/d×7天,第8天外周血白血病细胞≥1×109/L(1000/μl),或治疗15~19天时骨髓幼稚淋巴细胞比例仍大于25%。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "小儿ALL" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "白细胞" }, { "start_idx": 91, "end_idx": 93, "type": "bod", "entity": "染色体" }, { "start_idx": 149, "end_idx": 153, "type": "bod", "entity": "白血病细胞" }, { "start_idx": 188, "end_idx": 195, "type": "bod", "entity": "骨髓幼稚淋巴细胞" } ] }, { "text": "具备上述危险因素≥1项者为高危ALL(HR-ALL),提示预后较差,需较强烈的治疗方案;不存在其中任何1项者为标危ALL(SR-ALL),提示预后较好,在合理治疗下,长期无病生存率可达70%~85%。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "ALL" }, { "start_idx": 61, "end_idx": 66, "type": "dis", "entity": "SR-ALL" } ] }, { "text": "近来国际上普遍认可在诱导缓解治疗结束时(诱导28天至35天)骨髓微量白血病残留(MRD)水平低于10-4时预后好。", "entities": [ { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "骨髓" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "白血病" } ] }, { "text": "【鉴别诊断】临床诊断ITP、再生障碍性贫血、粒细胞减少症、传染性单核细胞增多症、各种关节炎、类白血病反应时应想到本病,当不能肯定除外白血病时,即应及时作骨髓穿刺涂片进一步明确诊断。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "ITP" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "再生障碍性贫血" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "粒细胞减少症" }, { "start_idx": 29, "end_idx": 38, "type": "dis", "entity": "传染性单核细胞增多症" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "关节炎" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "白血病" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "白血病" }, { "start_idx": 76, "end_idx": 81, "type": "pro", "entity": "骨髓穿刺涂片" } ] }, { "text": "1.对不明原因的贫血、出血、发热和不能以感染完全解释的发热,以及多脏器浸润症状表现者应考虑本病诊断。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "贫血" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "出血" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "发热" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "发热" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "多脏器浸润症状" } ] }, { "text": "2.对体格检查中发现有与出血程度不相符的贫血、肝、脾、淋巴结肿大者,尤其有腮腺、睾丸和软组织浸润肿大者,以及伴有骨、关节痛明显者应考虑本病的诊断。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "贫血" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "肝" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "脾" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "淋巴结" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "腮腺" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "睾丸" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "软组织" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "骨、关节痛" } ] }, { "text": "3.外周血发现≥2个系列异常或见有幼稚细胞者应考虑到本病的可能,进一步作骨髓涂片检查。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "幼稚细胞" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "骨髓涂片" } ] }, { "text": "【治疗】(一)治疗原则ALL以化学治疗(化疗)为主要手段。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "ALL" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "化学治疗" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "化疗" } ] }, { "text": "化疗的主要原则是按临床危险型选择不同强度的治疗方案,强调早期连续合理强烈化疗和坚持长期持续化疗,同时给予鞘内化疗预防CNSL的发生。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "化疗" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "化疗" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "化疗" }, { "start_idx": 58, "end_idx": 61, "type": "dis", "entity": "CNSL" } ] }, { "text": "化疗过程中应密切观察,进行有效的对症治疗和并发症的预防和治疗,包括瘤细胞性栓塞,肿瘤溶解综合征,水、电解质平衡,贫血,出血,DIC,各脏器特别是心、肝、肾正常功能的维持,各种感染及各种化疗药物毒副反应的防治。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "瘤细胞性栓塞" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "肿瘤溶解综合征" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "水" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "电解质" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "贫血" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "出血" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "DIC" }, { "start_idx": 72, "end_idx": 72, "type": "bod", "entity": "心" }, { "start_idx": 74, "end_idx": 74, "type": "bod", "entity": "肝" }, { "start_idx": 76, "end_idx": 76, "type": "bod", "entity": "肾" }, { "start_idx": 87, "end_idx": 88, "type": "dis", "entity": "感染" }, { "start_idx": 94, "end_idx": 97, "type": "dis", "entity": "药物毒副" } ] }, { "text": "同种异体造血干细胞移植适用于难治性及复发性ALL,宜在CR后进行移植。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "造血干细胞" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "ALL" } ] }, { "text": "(二)化疗ALL化疗基本组成部分包括诱导缓解治疗、缓解后巩固治疗、CNSL预防性治疗、再诱导治疗、和维持治疗。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "化疗" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "ALL" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "化疗" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "诱导缓解治疗" }, { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "缓解后巩固治疗" }, { "start_idx": 33, "end_idx": 41, "type": "pro", "entity": "CNSL预防性治疗" }, { "start_idx": 43, "end_idx": 47, "type": "pro", "entity": "再诱导治疗" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "维持治疗" } ] }, { "text": "1.诱导缓解治疗ALL诱导缓解治疗首选国内外常用的标准方案VDLP方案:即长春新碱(VCR)1.5mg/m2,每周1次×4次;柔红霉素(DNR)30mg/m2,每周1次,共2~3次(HR-ALL用3次,SR-ALL用2次);左旋-门冬酰胺酶(L-Asparaginase,L-ASP)6000~10000U/m2,隔天1次共6~8次(HR-ALL用8次,SR-ALL用6次);泼尼松(Prednisone)每天60mg/m2,分三次口服,共28天,减停7天。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "诱导缓解治疗" }, { "start_idx": 8, "end_idx": 16, "type": "pro", "entity": "ALL诱导缓解治疗" }, { "start_idx": 37, "end_idx": 40, "type": "dru", "entity": "长春新碱" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "VCR" }, { "start_idx": 69, "end_idx": 72, "type": "dru", "entity": "柔红霉素" }, { "start_idx": 74, "end_idx": 76, "type": "dru", "entity": "DNR" }, { "start_idx": 103, "end_idx": 108, "type": "dis", "entity": "HR-ALL" }, { "start_idx": 113, "end_idx": 118, "type": "dis", "entity": "SR-ALL" }, { "start_idx": 124, "end_idx": 131, "type": "dru", "entity": "左旋-门冬酰胺酶" }, { "start_idx": 133, "end_idx": 146, "type": "dru", "entity": "L-Asparaginase" }, { "start_idx": 148, "end_idx": 152, "type": "dru", "entity": "L-ASP" }, { "start_idx": 179, "end_idx": 184, "type": "dis", "entity": "HR-ALL" }, { "start_idx": 189, "end_idx": 194, "type": "dis", "entity": "SR-ALL" }, { "start_idx": 200, "end_idx": 202, "type": "dru", "entity": "泼尼松" }, { "start_idx": 204, "end_idx": 213, "type": "dru", "entity": "Prednisone" } ] }, { "text": "2.缓解后巩固治疗推荐用CAT方案,环磷酰胺(CTX)800~1000/m2第1天,阿糖胞苷(Ara-C)每日100mg/m2×7天,每日分2次(q12h),皮下注射;6-硫鸟嘌呤(6-TG)或6-鸟嘌呤(6-MP),每日75mg/m2,晚间顿服×7日;HR-ALL时可采用中、大剂量Ara-C,1~2g/m2,q12h×4~6次,CTX和6-mp同上。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "缓解后巩固治疗" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "CTX" }, { "start_idx": 42, "end_idx": 45, "type": "dru", "entity": "阿糖胞苷" }, { "start_idx": 47, "end_idx": 51, "type": "dru", "entity": "Ara-C" }, { "start_idx": 79, "end_idx": 82, "type": "pro", "entity": "皮下注射" }, { "start_idx": 84, "end_idx": 89, "type": "dru", "entity": "6-硫鸟嘌呤" }, { "start_idx": 91, "end_idx": 94, "type": "dru", "entity": "6-TG" }, { "start_idx": 97, "end_idx": 101, "type": "dru", "entity": "6-鸟嘌呤" }, { "start_idx": 103, "end_idx": 106, "type": "dru", "entity": "6-MP" }, { "start_idx": 127, "end_idx": 132, "type": "dis", "entity": "HR-ALL" }, { "start_idx": 142, "end_idx": 146, "type": "dru", "entity": "Ara-C" }, { "start_idx": 166, "end_idx": 168, "type": "dru", "entity": "CTX" }, { "start_idx": 170, "end_idx": 173, "type": "dru", "entity": "6-mp" } ] }, { "text": "3.CNSL及其他髓外白血病预防采用头颅放疗预防CNSL者越来越少。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "CNSL" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "髓外白血病" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "头颅放疗" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "CNSL" } ] }, { "text": "HDMTX在巩固治疗休疗结束后开始,每隔10~15天1次,用3次,高危(HR-ALL)共用5~6次,低危(LR-ALL)共用3~5次。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "HDMTX" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "HR-ALL" }, { "start_idx": 53, "end_idx": 58, "type": "dis", "entity": "LR-ALL" } ] }, { "text": "每次MTX剂量为3000~5000mg(HR)/m2,1/6静脉推注15分钟(不超过500mg),余量于24小时内均匀滴入。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "MTX" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "静脉推注" } ] }, { "text": "在推注后30~120分钟之间鞘内注入“三联”化疗(见后)。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "推注" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "化疗" } ] }, { "text": "于治疗起第37小时用四氢叶酸钙(CF)15mg/m2共6~8次,首剂静注,以后可改q6h口服。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "四氢叶酸钙" }, { "start_idx": 16, "end_idx": 17, "type": "dru", "entity": "CF" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "静注" }, { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "口服" } ] }, { "text": "有条件者检测血浆MTX浓度(<0.1mol/L为无毒性浓度),以调整CF应用的次数和剂量。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "血浆MTX" }, { "start_idx": 34, "end_idx": 35, "type": "dru", "entity": "CF" } ] }, { "text": "若44小时时<1mol,68小时时<0.1mol,则CF用6次即可,否则要延长并增加解救剂量。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "dru", "entity": "CF" } ] }, { "text": "预防毒性措施包括水化、碱化,化疗前3天起口服碳酸氢钠0.5~1.0g,每日3次,化疗当天起用5%碳酸氢钠5ml/kg静滴,每天补液1/5张含钠溶液3000ml/m2,24小时内均匀滴入,共4天。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "水化" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "碱化" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "化疗" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "化疗" }, { "start_idx": 48, "end_idx": 51, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 58, "end_idx": 59, "type": "pro", "entity": "静滴" }, { "start_idx": 69, "end_idx": 72, "type": "dru", "entity": "含钠溶液" } ] }, { "text": "用药前肝、肾功能必须正常。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肝" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "肾" } ] }, { "text": "鞘内化疗:诱导治疗开始后2~5天起每周鞘内注射MTX、Ara-c、地塞米松(DX)“三联”化疗1次,共5~6次,以后每3月1次至治疗结束。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "鞘内化疗" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "诱导治疗" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "鞘内注射" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "MTX" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "Ara-c" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "地塞米松" }, { "start_idx": 38, "end_idx": 39, "type": "dru", "entity": "DX" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "化疗" } ] }, { "text": "MTX剂量为12.5mg/m2(最大12.5mg);Ara-C1mg/kg(最大50mg);DX0~2岁为2.5mg,>2岁5mg。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "MTX" }, { "start_idx": 32, "end_idx": 36, "type": "dru", "entity": "Ara-C" }, { "start_idx": 52, "end_idx": 53, "type": "dru", "entity": "DX" } ] }, { "text": "4.再诱导治疗一般在第3次HDMTX+CF10~14天起,HR-ALL的早期强化治疗分2个阶段,第一阶段用VDLP,与诱导治疗的不同之处是DNR和VCR每周1次共2次,泼尼松剂量每天45mg/m2共14天,逐渐减量,7天内停药,口服。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "再诱导治疗" }, { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "HDMTX" }, { "start_idx": 19, "end_idx": 20, "type": "dru", "entity": "CF" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "HR-ALL" }, { "start_idx": 53, "end_idx": 56, "type": "dru", "entity": "VDLP" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "诱导治疗" }, { "start_idx": 69, "end_idx": 71, "type": "dru", "entity": "DNR" }, { "start_idx": 73, "end_idx": 75, "type": "dru", "entity": "VCR" }, { "start_idx": 84, "end_idx": 86, "type": "dru", "entity": "泼尼松" } ] }, { "text": "第二阶段用VP-16每次200mg/m2,Ara-c每次300mg/m2,每3天1次,共3次,静滴。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "VP-16" }, { "start_idx": 21, "end_idx": 25, "type": "dru", "entity": "Ara-c" }, { "start_idx": 47, "end_idx": 48, "type": "pro", "entity": "静滴" } ] }, { "text": "SR-ALL的早期强化只用VDLP。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "SR-ALL" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "VDLP" } ] }, { "text": "5.维持治疗MTX肌注或口服20~30mg/m2每周1次共3周,同时6-巯基嘌呤(6-MP)每天75mg/m2共21天,口服;后接VCR1.5mg/m21次,泼尼松剂量每天45mg/m2共7天;如此每4周1个周期,周而复始,并根据个体外周血白细胞计数调整MTX和6-MP剂量,使白细胞计数维持在(2.8~3.0)×109/L。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "MTX" }, { "start_idx": 40, "end_idx": 45, "type": "dru", "entity": "6-巯基嘌呤" }, { "start_idx": 47, "end_idx": 50, "type": "dru", "entity": "6-MP" }, { "start_idx": 71, "end_idx": 73, "type": "dru", "entity": "VCR" }, { "start_idx": 91, "end_idx": 93, "type": "dru", "entity": "泼尼松" }, { "start_idx": 135, "end_idx": 137, "type": "bod", "entity": "外周血" }, { "start_idx": 138, "end_idx": 140, "type": "bod", "entity": "白细胞" }, { "start_idx": 145, "end_idx": 147, "type": "dru", "entity": "MTX" }, { "start_idx": 149, "end_idx": 152, "type": "dru", "entity": "6-MP" }, { "start_idx": 157, "end_idx": 159, "type": "bod", "entity": "白细胞" } ] }, { "text": "ALL总治疗期限男孩为2.5~3年,女孩2~2.5年。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "ALL" } ] }, { "text": "6.CNSL治疗按剂量“三联”鞘注化疗8次,隔天1次至脑脊液中肿瘤细胞消失(一般鞘注2~3次后脑脊液大多转阴),以后每周2次至总共8次。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "CNSL" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "鞘注化疗" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "脑脊液" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "鞘注" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "脑脊液" } ] }, { "text": "如CNSL发生在骨髓CR期,则需在脑脊液转阴后增加1次全身强化治疗,以避免CNSL后全身复发,然后作全颅放疗(60Co或直线加速器)治疗,总剂量为18Gy,分成15次照射,对已有足够身高的大年龄患儿同时作全脊髓放疗,对小年龄患儿则在全颅放疗的同时增加鞘内化疗每周1次共2次。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "CNSL" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "骨髓" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "脑脊液" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "CNSL" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "全颅放疗" }, { "start_idx": 108, "end_idx": 112, "type": "pro", "entity": "全脊髓放疗" }, { "start_idx": 122, "end_idx": 125, "type": "pro", "entity": "全颅放疗" }, { "start_idx": 131, "end_idx": 134, "type": "pro", "entity": "鞘内化疗" } ] }, { "text": "7.睾丸白血病(TL)治疗睾丸异常肿大,怀疑为TL时,最好能作活检以确诊。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "睾丸白血病" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "TL" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "睾丸" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "TL" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "活检" } ] }, { "text": "如为双侧TL,则作双侧睾丸放疗,总剂量为24~30Gy。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "TL" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "睾丸放疗" } ] }, { "text": "若是单侧TL,可作病侧睾丸手术切除。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "TL" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "病侧睾丸手术" } ] }, { "text": "如起病时已有TL,应按原治疗方案进行全身性诱导、巩固等治疗,在诱导结束后作TL局部治疗。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "TL" }, { "start_idx": 37, "end_idx": 42, "type": "pro", "entity": "TL局部治疗" } ] }, { "text": "若CR中发生TL,在治疗TL的同时,给予VDLDX和VP-16+Ara-c方案各一个疗程作全身治疗,以免由TL引发骨髓复发。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "TL" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "TL" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "VDLDX" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "VP-16" }, { "start_idx": 32, "end_idx": 36, "type": "dru", "entity": "Ara-c" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "TL" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "骨髓" } ] }, { "text": "8.并发症的预防及支持治疗(1)防止肿瘤细胞溶解综合征:淋巴细胞白血病细胞对化疗常十分敏感,在化疗开始时大量的肿瘤细胞被药物杀伤破坏肿瘤细胞溶解综合征发肿瘤细胞溶解综合征,此种情况常发生在化疗刚开始1周内,主要表现为高尿酸血症、高血钾、高血磷、低血钠、低血钙等电解质紊乱,酸碱平衡失调和少尿、无尿、DIC等。", "entities": [ { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "肿瘤细胞溶解综合征" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "白血病细胞" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "化疗" }, { "start_idx": 47, "end_idx": 48, "type": "pro", "entity": "化疗" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 66, "end_idx": 74, "type": "dis", "entity": "肿瘤细胞溶解综合征" }, { "start_idx": 94, "end_idx": 95, "type": "pro", "entity": "化疗" }, { "start_idx": 108, "end_idx": 112, "type": "sym", "entity": "高尿酸血症" }, { "start_idx": 114, "end_idx": 116, "type": "sym", "entity": "高血钾" }, { "start_idx": 118, "end_idx": 120, "type": "sym", "entity": "高血磷" }, { "start_idx": 122, "end_idx": 124, "type": "sym", "entity": "低血钠" }, { "start_idx": 126, "end_idx": 128, "type": "sym", "entity": "低血钙" }, { "start_idx": 130, "end_idx": 134, "type": "sym", "entity": "电解质紊乱" }, { "start_idx": 136, "end_idx": 141, "type": "sym", "entity": "酸碱平衡失调" }, { "start_idx": 143, "end_idx": 144, "type": "sym", "entity": "少尿" }, { "start_idx": 146, "end_idx": 147, "type": "sym", "entity": "无尿" }, { "start_idx": 149, "end_idx": 151, "type": "sym", "entity": "DIC" } ] }, { "text": "为减慢肿瘤细胞溶解的速度,避免肿瘤细胞溶解综合征形成,对于外周血白细胞计数大于50×109/L者初始化疗应相对减弱,如仅给泼尼松和长春新碱,在3~7天后才给予较强的化疗。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 15, "end_idx": 23, "type": "dis", "entity": "肿瘤细胞溶解综合征" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "白细胞" }, { "start_idx": 56, "end_idx": 57, "type": "pro", "entity": "化疗" }, { "start_idx": 67, "end_idx": 69, "type": "dru", "entity": "泼尼松" }, { "start_idx": 71, "end_idx": 74, "type": "dru", "entity": "长春新碱" }, { "start_idx": 88, "end_idx": 89, "type": "dis", "entity": "化疗" } ] }, { "text": "对所有诱导期第1~2周的新病人均应给予3000ml/m2水化、5%碳酸氢钠5ml/kg碱化血液和尿液,监测电解质、尿酸、DIC指标,保证水、电解质平衡,同时服用别嘌呤醇200~300mg/(m2•d),以减少尿酸的形成,防止尿酸性肾小管栓塞所致的肾功能不全。", "entities": [ { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "水化" }, { "start_idx": 39, "end_idx": 42, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "碱化" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "血液" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "尿液" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "电解质" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "尿酸" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "DIC" }, { "start_idx": 74, "end_idx": 74, "type": "bod", "entity": "水" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "电解质" }, { "start_idx": 86, "end_idx": 89, "type": "dru", "entity": "别嘌呤醇" }, { "start_idx": 116, "end_idx": 117, "type": "bod", "entity": "尿酸" }, { "start_idx": 124, "end_idx": 131, "type": "dis", "entity": "尿酸性肾小管栓塞" }, { "start_idx": 135, "end_idx": 139, "type": "dis", "entity": "肾功能不全" } ] }, { "text": "应用SMZco25~50mg/(kg•d),诱导期可全程应用,缓解后每周用3天,防止发生卡氏肺囊虫肺炎。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "SMZco" }, { "start_idx": 44, "end_idx": 50, "type": "dis", "entity": "卡氏肺囊虫肺炎" } ] }, { "text": "静脉应用丙种球蛋白每次200~400mg/kg可能减少某些感染的机会。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "丙种球蛋白" } ] }, { "text": "化疗期间禁止接种活疫苗,以避免疫苗布散感染。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" } ] }, { "text": "加强口腔和肛门护理,及时治疗如龋齿等潜在感染灶以减少内源性感染。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "口腔" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肛门" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "龋齿" } ] }, { "text": "定期进行心、肝、肾功能检查,避免脏器功能不全。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "心" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肝" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肾" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "脏器" } ] }, { "text": "(4)适当应用造血刺激因子缩短骨髓抑制期,可能减少感染机会。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dru", "entity": "造血刺激因子" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "骨髓" } ] }, { "text": "可应用粒-单刺激因子或粒细胞刺激因子(GMCSF或G-CSF),对缓解中病人在强化疗48小时后根据化疗强度适时应用3~5μg/(kg•d)至白细胞>3.0×109/L。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dru", "entity": "粒-单刺激因子" }, { "start_idx": 11, "end_idx": 17, "type": "dru", "entity": "粒细胞刺激因子" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "GMCSF" }, { "start_idx": 25, "end_idx": 29, "type": "dru", "entity": "G-CSF" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "化疗" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "化疗" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "白细胞" } ] }, { "text": "(5)血制品应用:在贫血、出血的预防和治疗中十分重要,具体见第十篇第二章第三节。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "贫血" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "出血" } ] }, { "text": "9.随访与病人管理应将白血病治疗视作为一个系统性工程,随访及病人管理是其中十分重要的部分,以保证按时实施治疗计划。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "白血病" } ] }, { "text": "第三节遗传病的产前诊断绝大多数遗传病和遗传代谢病无有效治疗方法,因此在今后很长一段时间内,预防显得更为重要。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "产前诊断" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "绝大多数遗传病" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "遗传代谢病" } ] }, { "text": "产前诊断(prenataldiagnosis)又称宫内诊断,通过直接或间接对胎儿性别及健康状况进行检测,以防止具有严重遗传病、智力障碍及先天畸形患儿的出生。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "产前诊断" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "宫内诊断" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "严重遗传病" }, { "start_idx": 63, "end_idx": 66, "type": "dis", "entity": "智力障碍" }, { "start_idx": 68, "end_idx": 71, "type": "dis", "entity": "先天畸形" } ] }, { "text": "产前诊断是近20年来由细胞遗传学、生化遗传学、分子遗传学等学科和临床医学实践紧密结合起来形成的一门边缘学科,有很强的实际应用价值,是近代医学的一项重大成就。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "产前诊断" } ] }, { "text": "一、产前诊断现状目前已有2万多种不同的遗传病,包括单基因病、多基因病和染色体病。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "产前诊断" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "遗传病" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "单基因病" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "多基因病" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "染色体病" } ] }, { "text": "这些遗传病可在胎内造成流产,出生后也是造成新生儿死亡的原因之一。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "遗传病" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "流产" } ] }, { "text": "因此,人们希望找到一种早期诊断和治疗这些疾病的方法,产前诊断就是顺应这一要求而发展起来的。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "产前诊断" } ] }, { "text": "自20世纪60年代开始经腹壁羊膜腔穿刺术用于产前诊断以来,产前诊断技术发展很快,1966年羊水细胞培养成功后不久,第一例21-三体综合征及半乳糖血症产前诊断成功,标志着宫内诊断的可行性。", "entities": [ { "start_idx": 12, "end_idx": 19, "type": "pro", "entity": "腹壁羊膜腔穿刺术" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "产前诊断" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "产前诊断" }, { "start_idx": 45, "end_idx": 50, "type": "pro", "entity": "羊水细胞培养" }, { "start_idx": 60, "end_idx": 67, "type": "dis", "entity": "21-三体综合征" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 74, "end_idx": 77, "type": "pro", "entity": "产前诊断" }, { "start_idx": 84, "end_idx": 87, "type": "pro", "entity": "宫内诊断" } ] }, { "text": "70年代以后,这些技术已广泛应用于临床,随着绒毛、羊水细胞培养、高分辨显带染色体以及重组DNA等技术的广泛应用和不断完善,继之可经胎儿镜取胎儿血标本及经宫颈、经腹壁取绒毛做产前诊断。", "entities": [ { "start_idx": 22, "end_idx": 30, "type": "pro", "entity": "绒毛、羊水细胞培养" }, { "start_idx": 32, "end_idx": 39, "type": "pro", "entity": "高分辨显带染色体" }, { "start_idx": 42, "end_idx": 46, "type": "pro", "entity": "重组DNA" }, { "start_idx": 64, "end_idx": 73, "type": "pro", "entity": "经胎儿镜取胎儿血标本" }, { "start_idx": 83, "end_idx": 84, "type": "bod", "entity": "绒毛" }, { "start_idx": 86, "end_idx": 89, "type": "pro", "entity": "产前诊断" } ] }, { "text": "近年来正在发展一种非损伤性产前诊断技术,从孕妇外周血中富集、分离胎儿有核红细胞以及胎儿DNA片段,将来源于胎儿的DNA扩增后进行连锁分析或直接检测突变进行产前基因诊断。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "pro", "entity": "非损伤性产前诊断" }, { "start_idx": 34, "end_idx": 38, "type": "pro", "entity": "有核红细胞" }, { "start_idx": 41, "end_idx": 47, "type": "bod", "entity": "胎儿DNA片段" }, { "start_idx": 64, "end_idx": 67, "type": "pro", "entity": "连锁分析" }, { "start_idx": 71, "end_idx": 74, "type": "pro", "entity": "检测突变" }, { "start_idx": 77, "end_idx": 82, "type": "pro", "entity": "产前基因诊断" } ] }, { "text": "优生知识和遗传知识的普及使遗传病的产前诊断受到了社会的广泛重视,西欧、北美各国相继建立了产前诊断中心。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "遗传病" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "产前诊断" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "产前诊断" } ] }, { "text": "我国有较多省市开展了染色体核型分析,开展了产前诊断。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "染色体核型分析" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "产前诊断" } ] }, { "text": "近年,随着分子生物学技术的发展,北京、上海及广州等地都开展了一些遗传代谢病的产前诊断。", "entities": [ { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "产前诊断" } ] }, { "text": "二、硬膜下出血随着产科实践的改进,近年由产伤所致的硬膜下出血(subduralhemorrhage)的发生率明显下降,但因其临床后果严重,早期诊断和及时干预十分重要。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 9, "end_idx": 10, "type": "dep", "entity": "产科" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 31, "end_idx": 48, "type": "dis", "entity": "subduralhemorrhage" } ] }, { "text": "【病因与发病机制】硬膜下出血主要由小脑幕或大脑镰撕裂所致。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "小脑幕" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "大脑镰" } ] }, { "text": "严重的小脑幕撕裂可以致死,特别是伴直窦或横窦撕裂时,血块可流到后颅凹迅速压迫脑干。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "小脑幕" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "直窦" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "血块" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "后颅凹" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "脑干" } ] }, { "text": "多数为小脑幕轻度撕裂所致的幕上或幕下出血。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "小脑幕" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "出血" } ] }, { "text": "出血也可发生在小脑幕的游离缘,特别是小脑幕和大脑镰的连接处,并向前进一步伸展到蛛网膜腔或脑室系统。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "出血" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "小脑幕" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "小脑幕" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "大脑镰" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "蛛网膜腔" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "脑室系统" } ] }, { "text": "在某些臀位产的患儿,可因枕骨分离伴小脑幕和枕窦撕裂而引起后颅凹大量出血和小脑撕裂。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "臀位" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "枕骨" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "小脑幕" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "枕窦" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "后颅凹" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "大量出血" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "小脑撕裂" } ] }, { "text": "单纯的大脑镰撕裂比小脑幕撕裂常见,出血来源于下矢状窦和胼胝体上方的大脑纵裂池,大脑表面的桥静脉破裂也可引起大脑表面的硬膜下血肿。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "大脑镰撕裂" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "小脑幕撕裂" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "出血" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "下矢状窦" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "胼胝体" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "大脑纵裂池" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "大脑" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "桥静脉破裂" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "硬膜下血肿" } ] }, { "text": "产伤性颅内出血常同时伴有脑挫伤。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "产伤性颅内出血" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "脑挫伤" } ] }, { "text": "【临床表现】(一)小脑幕撕裂伴后颅凹硬膜下出血常见于难产性臀位牵引,临床表现可有3个阶段:①出生数小时内可无任何症状,此时血肿缓慢增大,通常<24小时,也可长达3~4天;②随着颅内压增高,后颅凹脑脊液循环通路受阻,出现前囟饱满、激惹或嗜睡等症状;③随着病情进展,出现脑干受压的体征,包括呼吸节律异常、眼动异常、斜视、面瘫和惊厥。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "小脑幕撕裂" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "后颅凹硬膜下出血" }, { "start_idx": 61, "end_idx": 66, "type": "sym", "entity": "血肿缓慢增大" }, { "start_idx": 88, "end_idx": 90, "type": "ite", "entity": "颅内压" }, { "start_idx": 88, "end_idx": 92, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 94, "end_idx": 99, "type": "bod", "entity": "后颅凹脑脊液" }, { "start_idx": 109, "end_idx": 110, "type": "bod", "entity": "前囟" }, { "start_idx": 109, "end_idx": 118, "type": "sym", "entity": "前囟饱满、激惹或嗜睡" }, { "start_idx": 133, "end_idx": 134, "type": "bod", "entity": "脑干" }, { "start_idx": 143, "end_idx": 148, "type": "sym", "entity": "呼吸节律异常" }, { "start_idx": 150, "end_idx": 153, "type": "sym", "entity": "眼动异常" }, { "start_idx": 155, "end_idx": 156, "type": "sym", "entity": "斜视" }, { "start_idx": 158, "end_idx": 159, "type": "sym", "entity": "面瘫" }, { "start_idx": 161, "end_idx": 162, "type": "sym", "entity": "惊厥" } ] }, { "text": "(二)小脑幕撕裂伴大量幕下出血出生时即可出现中脑及脑桥上部受压的症状,如木僵、斜视、瞳孔不等大和对光反射迟钝、颈项强直和角弓反张等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "小脑幕撕裂" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "大量幕下出血" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "木僵" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "斜视" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "瞳孔" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "瞳孔不等大" }, { "start_idx": 48, "end_idx": 53, "type": "sym", "entity": "对光反射迟钝" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "颈项" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "颈项强直" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "角弓反张" } ] }, { "text": "如血块增大,可在短期内(数分钟至数小时)出现脑干下部受压的体征,从木僵进入昏迷,瞳孔固定和散大、心动过缓和呼吸不规则,最终呼吸停止而死亡。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "血块增大" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "脑干下部" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "脑干下部受压" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "木僵" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "昏迷" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "瞳孔固定和散大" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "心动过缓" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "呼吸不规则" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "呼吸停止" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "死亡" } ] }, { "text": "(三)大脑镰撕裂伴硬膜下出血出生时即可出现双侧弥漫性脑损伤症状,如兴奋、激惹等,如血块伸展到小脑幕下时症状类似于小脑幕撕裂。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "大脑镰撕裂" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "脑损伤" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "弥漫性脑损伤" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "兴奋" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "激惹" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "小脑幕" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "小脑幕撕裂" } ] }, { "text": "(四)大脑表面硬膜下出血轻度出血可无明显的临床症状,或仅表现兴奋、激惹。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "大脑表面硬膜下出血" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "轻度出血" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "兴奋、激惹" } ] }, { "text": "局灶性脑定位体征常开始于生后第2或3天,表现为局灶性惊厥、偏瘫、眼向对侧偏斜。", "entities": [ { "start_idx": 23, "end_idx": 37, "type": "sym", "entity": "局灶性惊厥、偏瘫、眼向对侧偏斜" } ] }, { "text": "当发生小脑幕切迹疝时可有瞳孔散大、对光反应减弱或消失等第3对脑神经受压的表现。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "小脑幕切迹疝" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "瞳孔" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "脑神经" }, { "start_idx": 12, "end_idx": 34, "type": "sym", "entity": "瞳孔散大、对光反应减弱或消失等第3对脑神经受压" } ] }, { "text": "少数病例在新生儿期无任何硬膜下出血的症状、体征,但在数月后发生硬膜下积液。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "硬膜下积液" } ] }, { "text": "【诊断】硬膜下出血的诊断主要依靠临床症状的识别和影像学检查。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "影像学检查" } ] }, { "text": "CT检查可确定硬膜下出血的部位和程度,但对后颅凹硬膜下出血和小脑出血的诊断价值不及MRI。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "CT检查" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "后颅凹硬膜下出血" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "小脑出血" }, { "start_idx": 41, "end_idx": 43, "type": "pro", "entity": "MRI" } ] }, { "text": "头颅超声只能检测到伴中线移位的大脑表面的硬膜下血肿,对幕上出血的诊断不及CT,对幕下出血的诊断不及MRI。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "头颅超声" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "硬膜下血肿" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "幕上出血" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "CT" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "幕下出血" }, { "start_idx": 49, "end_idx": 51, "type": "pro", "entity": "MRI" } ] }, { "text": "枕骨分离和颅骨骨折可通过头颅X线片证实。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "枕骨分离" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "颅骨骨折" }, { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "头颅X线片" } ] }, { "text": "腰椎穿刺对硬膜下出血诊断没有帮助,且有诱发脑疝可能。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "脑疝" } ] }, { "text": "【治疗】(一)止血可用维生素K1、酚磺乙胺、氨甲苯酸等。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "止血" }, { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "维生素K1" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "酚磺乙胺" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "氨甲苯酸" } ] }, { "text": "(二)降低颅内压如颅内压很高,发生脑疝,可适当使用20%甘露醇。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "降低颅内压" }, { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "颅内压" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "脑疝" }, { "start_idx": 25, "end_idx": 30, "type": "dru", "entity": "20%甘露醇" } ] }, { "text": "(三)抗惊厥出现惊厥者应及时止惊,可用地西泮类药物。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "抗惊厥" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "惊厥" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "止惊" }, { "start_idx": 19, "end_idx": 24, "type": "dru", "entity": "地西泮类药物" } ] }, { "text": "(四)外科治疗手术指征取决于出血病灶的大小、颅压增高的体征和是否存在脑疝。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dep", "entity": "外科" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "颅压" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "脑疝" } ] }, { "text": "大脑表面硬膜下出血伴中线移位,特别是临床症状恶化伴小脑幕切迹疝时,均是急诊硬膜下穿刺或切开引流的指征。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "大脑" }, { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "小脑幕切迹疝" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "硬膜下穿刺" } ] }, { "text": "位于后颅凹的大量硬膜下出血也需外科手术。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "后颅凹" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "外科手术" } ] }, { "text": "对于无明显症状的硬膜下出血患儿,外科手术并不能改善其远期预后,但需临床严密观察,若患儿病情稳定,无须手术。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "硬膜下出血" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "外科手术" } ] }, { "text": "轻度出血若能早期诊断和及时治疗,预后较好。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "轻度出血" } ] }, { "text": "严重小脑幕和大脑镰撕裂者病死率较高,存活者常发生脑积水和其他后遗症。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "小脑幕和大脑镰撕裂" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "脑积水" } ] }, { "text": "第六章溶血尿毒综合征详见“第六篇重症监护和急救”之“第八章”。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "溶血尿毒综合征" } ] }, { "text": "二、继发性免疫性血小板减少性紫癜由于免疫介导的继发性血小板减少性紫癜种类较多(见表10-8)。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "dis", "entity": "继发性免疫性血小板减少性紫癜" } ] }, { "text": "本文简要介绍儿科常见的继发性血小板减少性紫癜(secondarythrombocytopenicpurpura)—药物免疫性血小板减少性紫癜(drugsinducedimmunethrombocytopenicpurpura)。", "entities": [ { "start_idx": 11, "end_idx": 21, "type": "dis", "entity": "继发性血小板减少性紫癜" }, { "start_idx": 23, "end_idx": 54, "type": "dis", "entity": "secondarythrombocytopenicpurpura" }, { "start_idx": 57, "end_idx": 69, "type": "dis", "entity": "药物免疫性血小板减少性紫癜" }, { "start_idx": 71, "end_idx": 111, "type": "dis", "entity": "drugsinducedimmunethrombocytopenicpurpura" } ] }, { "text": "【发病机制】临床上通过免疫机制引起血小板减少的药物可达上百种,如退热镇痛药、抗生素、植物碱、镇静剂、利尿剂、强心剂、化疗药物、杀虫剂等等。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "血小板" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "抗生素" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "植物碱" }, { "start_idx": 46, "end_idx": 48, "type": "dru", "entity": "镇静剂" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "利尿剂" }, { "start_idx": 54, "end_idx": 56, "type": "dru", "entity": "强心剂" }, { "start_idx": 58, "end_idx": 61, "type": "dru", "entity": "化疗药物" }, { "start_idx": 63, "end_idx": 65, "type": "dru", "entity": "杀虫剂" } ] }, { "text": "目前所知分子量为500~1000D的药物可作为半抗原与血小板膜的一种或多种蛋白成分相结合,形成抗原并刺激机体产生特异性的抗体。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "血小板膜" } ] }, { "text": "这种抗体对药物-血小板复合物有特异性,可直接与血小板的某些成分如膜糖蛋白GPIb/Ⅸ和GPⅡb/Ⅲa等相结合,被单核-巨噬细胞系统所清除,有的亦可直接激活补体系统引起血小板破坏。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "血小板" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 83, "end_idx": 85, "type": "bod", "entity": "血小板" } ] }, { "text": "【临床表现】从摄入药物到引起免疫性血小板减少,临床上常有一潜伏期。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "血小板" } ] }, { "text": "该期长短不一,奎宁及奎尼丁类可短至数小时,安替比林类约2周,吲哚美辛、金盐等可长达数月。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "奎宁" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "奎尼丁类" }, { "start_idx": 21, "end_idx": 25, "type": "dru", "entity": "安替比林类" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 35, "end_idx": 36, "type": "dru", "entity": "金盐" } ] }, { "text": "临床症状取决于血小板减少程度和机体的反应,出血是在骨髓巨核细胞失代偿后出现,可有皮肤瘀点、瘀斑,鼻出血、牙龈出血等。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "血小板" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "骨髓" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "巨核细胞" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "皮肤" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "瘀点" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "瘀斑" }, { "start_idx": 48, "end_idx": 50, "type": "sym", "entity": "鼻出血" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "牙龈出血" } ] }, { "text": "严重病例全身皮肤发红,继之发热、寒战,严重出血包括口腔黏膜出血大疱、胃肠道出血、血尿、肺出血、颅内出血等;某些服用奎宁的病例兼有微血管病性贫血并发急性肾衰竭。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "全身皮肤发红" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "发热" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "寒战" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "出血" }, { "start_idx": 25, "end_idx": 50, "type": "sym", "entity": "口腔黏膜出血大疱、胃肠道出血、血尿、肺出血、颅内出血" }, { "start_idx": 57, "end_idx": 58, "type": "dru", "entity": "奎宁" }, { "start_idx": 64, "end_idx": 70, "type": "dis", "entity": "微血管病性贫血" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "急性肾衰竭" } ] }, { "text": "【实验室检查】血小板计数降低,重者常<1×109~10×109/L,出血时间延长,血块收缩不良;骨髓巨核细胞数正常或增加并伴有成熟障碍。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "ite", "entity": "血小板计数降低" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "骨髓" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "巨核细胞" } ] }, { "text": "体外有一些测定抗体的办法,例如测定奎宁、奎尼丁等诱导的抗体,可将病人的血清或血浆、正常人血小板、致敏药物混合后进行免疫测定,常用的有血块退缩抑制试验,即病人血清在有相关药物存在条件下抑制了相合血型的血块收缩,表明有与该药物有关的抗体存在。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "dru", "entity": "奎宁" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "奎尼丁" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "血清" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "血浆" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "血小板" }, { "start_idx": 114, "end_idx": 115, "type": "bod", "entity": "抗体" } ] }, { "text": "【诊断】病史中有可疑药物史,临床上以皮肤黏膜自发性出血为主,实验室可见血小板降低,如测得药物有关的抗体即可诊断。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "皮肤" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "黏膜" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "血小板" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "抗体" } ] }, { "text": "【治疗】停用一切可疑药物是治疗本症的关键;糖皮质激素能改善血管完整性而减少出血,根据病情选择口服或静滴。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "糖皮质激素" } ] }, { "text": "具有威胁生命的严重出血时可输注血小板,也可用血浆交换以减少抗体和药物浓度。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "血小板" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血浆" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "抗体" } ] }, { "text": "此外高剂量丙种球蛋白静滴,免疫抑制剂如长春新碱、环磷酰胺的使用都有帮助。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "丙种球蛋白" }, { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "长春新碱" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "环磷酰胺" } ] }, { "text": "金制剂引起者恢复慢,临床可试用二巯基丙醇增加其排泄率。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "金制剂" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "二巯基丙醇" } ] }, { "text": "【预后】停用有关药物后,本病能在数天至数周内恢复,极个别病例可死于严重出血如肺出血、颅内出血。", "entities": [ { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "严重出血" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "肺出血" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "颅内出血" } ] }, { "text": "第二节诊断方法(一)结核杆菌素试验(结素试验)结核杆菌感染后4~8周,身体对于结核蛋白产生过敏状态,此时如果作结核杆菌素试验(包括旧结核杆菌素试验OT和提纯蛋白物质PPD),局部可发生反应,表示受试者已受结核菌感染。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "结核杆菌素试验" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "结素试验" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "结核杆菌感染" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "过敏" }, { "start_idx": 55, "end_idx": 61, "type": "pro", "entity": "结核杆菌素试验" }, { "start_idx": 65, "end_idx": 72, "type": "pro", "entity": "旧结核杆菌素试验" }, { "start_idx": 73, "end_idx": 74, "type": "pro", "entity": "OT" }, { "start_idx": 76, "end_idx": 81, "type": "pro", "entity": "提纯蛋白物质" }, { "start_idx": 82, "end_idx": 84, "type": "pro", "entity": "PPD" }, { "start_idx": 87, "end_idx": 93, "type": "sym", "entity": "局部可发生反应" }, { "start_idx": 102, "end_idx": 106, "type": "dis", "entity": "结核菌感染" } ] }, { "text": "PPD第1次试验液为每0.1ml含1U,第2次试验液为每0.1ml中含5U,注射后48~72小时看结果,阴性者用高一级浓度再试。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "PPD" } ] }, { "text": "阳性标准如下:红晕及硬肿的平均直径5~9mm为+;10~19mm为++;>20mm为+++;除红晕及硬肿外,还有疱疹或坏死,或发热等全身症状为++++。", "entities": [ { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "红晕" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "硬肿" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "疱疹" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "坏死" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "发热" } ] }, { "text": "但BCG接种后,结核菌素试验的诊断价值受到影响。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "BCG" }, { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "接种" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "结核菌素试验" } ] }, { "text": "目前区别自然感染与BCG接种后过敏反应的方法,是根据阳性反应的强度和持久情况。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "BCG" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "接种" } ] }, { "text": "自然感染时,结素反应多为强阳性反应(+++以上),且终生不消失;而BCG接种后多为弱阳性反应(红晕及硬肿平均直径<10mm),且持续时间短,反应逐渐减弱,一般3~5年后完全消失。", "entities": [ { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "BCG" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "接种" }, { "start_idx": 70, "end_idx": 75, "type": "sym", "entity": "反应逐渐减弱" } ] }, { "text": "此外,结素反应仅表示结核杆菌感染,还要根据小儿的年龄、BCG接种史及结核病接触史,判断患儿感染的活动性。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "结素反应" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "结核杆菌感染" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "BCG" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "接种" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "结核病" } ] }, { "text": "(二)血常规检查急性期时,白细胞可增高到10×109~20×109/L,伴有中性多形核粒细胞增高淋巴细胞减少单核细胞增多中性粒细胞核左移出现中毒性颗粒。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "血常规检查" }, { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "白细胞" }, { "start_idx": 50, "end_idx": 57, "type": "bod", "entity": "中性多形核粒细胞" }, { "start_idx": 50, "end_idx": 59, "type": "sym", "entity": "中性多形核粒细胞增高" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 60, "end_idx": 65, "type": "sym", "entity": "淋巴细胞减少" }, { "start_idx": 66, "end_idx": 69, "type": "bod", "entity": "单核细胞" }, { "start_idx": 66, "end_idx": 71, "type": "sym", "entity": "单核细胞增多" }, { "start_idx": 72, "end_idx": 77, "type": "bod", "entity": "中性粒细胞核" }, { "start_idx": 72, "end_idx": 79, "type": "sym", "entity": "中性粒细胞核左移" }, { "start_idx": 80, "end_idx": 86, "type": "sym", "entity": "出现中毒性颗粒" } ] }, { "text": "好转时白细胞数目恢复正常淋巴细胞增加红细胞沉降率多加速血清学检测国内外结核病血清学研究报告很多,目前主要检测结核病特异性抗体水平,尤其是抗结核IgG抗体的检测对诊断有很重要的参考价值。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "白细胞" }, { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "白细胞数目恢复正常" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "淋巴细胞增加" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "红细胞" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "红细胞沉降率多加速" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "血清学检测" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "结核病" }, { "start_idx": 54, "end_idx": 61, "type": "bod", "entity": "结核病特异性抗体" }, { "start_idx": 68, "end_idx": 75, "type": "bod", "entity": "抗结核IgG抗体" } ] }, { "text": "从临床应用角度来看,仍以ELISA法应用最广泛,也较为成熟。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "ELISA法" } ] }, { "text": "(四)寻找结核杆菌1.痰液标本的检测痰涂片找抗酸杆菌,通常几个小时之内就能出结果,但在儿童结核病很少阳性。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "痰液标本的检测" }, { "start_idx": 18, "end_idx": 20, "type": "equ", "entity": "痰涂片" }, { "start_idx": 22, "end_idx": 25, "type": "mic", "entity": "抗酸杆菌" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "儿童结核病" } ] }, { "text": "标本中要有大量的病原菌,即每毫升5000~10000个杆菌痰涂片中才能检测到抗酸杆菌。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "病原菌" }, { "start_idx": 27, "end_idx": 28, "type": "mic", "entity": "杆菌" }, { "start_idx": 13, "end_idx": 28, "type": "sym", "entity": "每毫升5000~10000个杆菌" }, { "start_idx": 29, "end_idx": 31, "type": "equ", "entity": "痰涂片" }, { "start_idx": 38, "end_idx": 41, "type": "mic", "entity": "抗酸杆菌" } ] }, { "text": "如果做培养,只需10~100个杆菌即可得到阳性结果原发结核病的患儿体内病原菌数目很少,因而,即使是活动结核病的患儿,其痰涂片有95%,而培养有60%都是阴性的。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "mic", "entity": "杆菌" }, { "start_idx": 8, "end_idx": 24, "type": "sym", "entity": "10~100个杆菌即可得到阳性结果" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "原发结核病" }, { "start_idx": 35, "end_idx": 37, "type": "mic", "entity": "病原菌" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "活动结核病" }, { "start_idx": 59, "end_idx": 61, "type": "equ", "entity": "痰涂片" } ] }, { "text": "另外,痰涂片对结核杆菌不具有特异性。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "痰涂片" }, { "start_idx": 7, "end_idx": 10, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "一份含有非结核分枝杆菌的标本也可能抗酸杆菌涂片阳性,因而病原菌一定要用标准的培养方法和DNA探针确认。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "mic", "entity": "非结核分枝杆菌" }, { "start_idx": 43, "end_idx": 47, "type": "pro", "entity": "DNA探针" } ] }, { "text": "在成人和儿童,涂片或培养阴性不能除外结核病。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "结核病" } ] }, { "text": "2.胃液或支气管肺泡灌洗液标本的检测由于儿童收集痰标本困难,在小婴幼儿肺结核患者,可通过清晨吸取胃液或通过支气管肺泡灌洗液收集标本。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "胃液" }, { "start_idx": 5, "end_idx": 12, "type": "bod", "entity": "支气管肺泡灌洗液" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "痰" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "肺结核" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "胃液" }, { "start_idx": 53, "end_idx": 60, "type": "bod", "entity": "支气管肺泡灌洗液" } ] }, { "text": "在夜间,呼吸道的纤毛把肺部的分泌物送到大气道,这些分泌物又被吞到胃内。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "呼吸道" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "纤毛" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肺部" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "分泌物" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "大气道" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "分泌物" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "胃内" } ] }, { "text": "清晨患儿未进食或未完全清醒时,其胃没有蠕动,此时下胃管抽取胃液做结核培养可以提供最好的标本。", "entities": [ { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "胃" }, { "start_idx": 24, "end_idx": 26, "type": "pro", "entity": "下胃管" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "胃液" } ] }, { "text": "另一种获取标本的途径是支气管肺泡灌洗液,和胃液的敏感性是一样的。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "bod", "entity": "支气管肺泡灌洗液" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "胃液" } ] }, { "text": "3.肺外结核病的标本检测在肺外结核的患者,由于感染结核的部位相对于肺部来说更难取标本,而且结核杆菌的数目较少,使获得细菌学诊断依据更为困难。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肺外结核病" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "肺外结核" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "结核" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肺部" }, { "start_idx": 45, "end_idx": 48, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "淋巴结结核通常需要针吸或手术切除后活检。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "淋巴结结核" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "针吸" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "手术切除" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "活检" } ] }, { "text": "标本应该送普通细菌培养和分枝杆菌培养。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "分枝杆菌培养" } ] }, { "text": "如果培养阴性,病理染色找抗酸杆菌和肉芽肿病变就更为重要。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "mic", "entity": "抗酸杆菌" } ] }, { "text": "其他部位的结核,如腹膜、骨或皮肤结核,一般需要做活检以取得足够的标本做检查。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "结核" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "腹膜" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "骨" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "皮肤" } ] }, { "text": "肾结核的患者则可以从尿液中找到结核菌。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肾结核" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "尿液" }, { "start_idx": 15, "end_idx": 17, "type": "mic", "entity": "结核菌" } ] }, { "text": "结核性脑膜炎的患者脑脊液的培养经常是阴性的,这是因为脑脊液中结核菌的数目相对较少的缘故。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "脑脊液" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "脑脊液" }, { "start_idx": 30, "end_idx": 32, "type": "mic", "entity": "结核菌" } ] }, { "text": "10ml或更多的脑脊液标本有利于提高检出的几率。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "脑脊液" } ] }, { "text": "即使是在理想条件下,也只有大约40%能从结核病患儿的胃液或支气管肺泡灌洗液标本找到结核杆菌。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "结核病" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "胃液" }, { "start_idx": 29, "end_idx": 36, "type": "bod", "entity": "支气管肺泡灌洗液" }, { "start_idx": 41, "end_idx": 44, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "婴幼儿肺结核培养的阳性率(70%)高于1岁以上的患儿(40%)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "婴幼儿肺结核" } ] }, { "text": "如果患儿结核菌素皮试阳性,具有和结核病一致的临床表现或X线片的改变,则已符合临床诊断结核病的标准。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "结核病" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "X线片" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "结核病" } ] }, { "text": "结核菌素试验阴性结核病的临床表现,并和有传染性的成人结核病患者有接触史者,也认为可以临床诊断。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "结核菌素试验" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "结核菌素试验阴性" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "结核病" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "结核病" } ] }, { "text": "怀疑有结核病的患儿应该进一步取标本做培养,因为培养阳性可以证实临床诊断,而且药物敏感试验的结果对治疗也非常重要。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "结核病" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "培养阳性" } ] }, { "text": "如果和患儿有接触的成人结核病患者培养分离出阳性结果,其药物敏感试验结果可以指导患儿的治疗,因为儿童一般都是由于家庭成员患病或和结核患者共同生活在同一居室内而感染结核病的。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "结核病" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "药物敏感试验" }, { "start_idx": 80, "end_idx": 82, "type": "dis", "entity": "结核病" } ] }, { "text": "4.结核杆菌检测的新方法结核杆菌传统的检测方法存在许多缺点。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 12, "end_idx": 15, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "结核杆菌在培养基中生长缓慢,每18~24小时分离一代。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "传统的结核培养在固体培养基中要8周才能出结果,可能还需要4~8周的时间做药物敏感试验。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "结核" }, { "start_idx": 36, "end_idx": 41, "type": "pro", "entity": "药物敏感试验" } ] }, { "text": "而结核抗酸杆菌涂片在儿科病例阳性率极低且没有特异性。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "pro", "entity": "结核抗酸杆菌涂片" } ] }, { "text": "放射或比色法系统如BACTEC使用Middlebrook7H12液体培养基,在这种培养基中加入了促使结核杆菌生长的物质,使其在1~3周之内即能快速生长,从而得到阳性结果。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "放射或比色法系统" }, { "start_idx": 17, "end_idx": 36, "type": "equ", "entity": "Middlebrook7H12液体培养基" }, { "start_idx": 50, "end_idx": 53, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "传统的固体培养基如改良罗氏培养基(LowenssteinJenssen)和BACTEC液体培养基结合,使菌落的形态学可以被确定。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "equ", "entity": "改良罗氏培养基" }, { "start_idx": 17, "end_idx": 34, "type": "equ", "entity": "LowenssteinJenssen" }, { "start_idx": 37, "end_idx": 47, "type": "equ", "entity": "BACTEC液体培养基" } ] }, { "text": "如果培养阳性,就要用DNA探针鉴别分枝杆菌的类型,确定是否为结核分枝杆菌。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "DNA探针" }, { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "分枝杆菌" }, { "start_idx": 30, "end_idx": 35, "type": "mic", "entity": "结核分枝杆菌" } ] }, { "text": "应用最广泛的是核酸杂交技术和高效液相色谱法。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "核酸杂交技术" } ] }, { "text": "这些技术可以区别出结核杆菌菌属,以及鸟型分枝杆菌、堪萨斯分枝杆菌、戈登分枝杆菌。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "结核杆菌" }, { "start_idx": 18, "end_idx": 23, "type": "mic", "entity": "鸟型分枝杆菌" }, { "start_idx": 25, "end_idx": 31, "type": "mic", "entity": "堪萨斯分枝杆菌" }, { "start_idx": 33, "end_idx": 38, "type": "mic", "entity": "戈登分枝杆菌" } ] }, { "text": "目前的方法还不能鉴定其他分枝杆菌的类型。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "mic", "entity": "分枝杆菌" } ] }, { "text": "一旦培养阳性就要使用这些技术做鉴别,但这种技术不能用于临床标本如痰液或胃液的直接检测。", "entities": [ { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "痰液" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "胃液" } ] }, { "text": "(五)核酸扩增技术核酸扩增技术如DNA快速复制靶序列的DNA或RNA。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "核酸扩增技术" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "核酸扩增技术" }, { "start_idx": 16, "end_idx": 33, "type": "pro", "entity": "DNA快速复制靶序列的DNA或RNA" } ] }, { "text": "由于只需一部分DNA或RNA,正在治疗中的患者体内的非存活菌,以及结核感染后极少量的临床标本,都可以聚合酶链反应(PCR)检测结果阳性。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "DNA" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "RNA" } ] }, { "text": "实验室研究报道,这种技术可以在标本中少到只有10个细菌的情况下得到阳性结果。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "mic", "entity": "细菌" } ] }, { "text": "在抗酸杆菌涂片阳性的标本,PCR的敏感度是95%,特异度是98%。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "equ", "entity": "抗酸杆菌涂片" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "PCR" } ] }, { "text": "在抗酸杆菌涂片阴性,细菌数量少的标本,PCR的特异度仍有95%,但敏感度只有50%。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "equ", "entity": "抗酸杆菌涂片" }, { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "细菌" }, { "start_idx": 19, "end_idx": 21, "type": "pro", "entity": "PCR" } ] }, { "text": "因此,PCR结果阴性仍不能除外结核病。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "PCR" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "结核病" } ] }, { "text": "在儿科的研究表明,PCR的敏感度和培养相同,约为40%;如果PCR和培养结合,并和临床诊断比较,其敏感度可增加到60%。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "PCR" }, { "start_idx": 30, "end_idx": 32, "type": "pro", "entity": "PCR" } ] }, { "text": "如果PCR阳性,可以证实诊断,但PCR阴性绝不能除外结核病的诊断。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "PCR" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "PCR" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "结核病" } ] }, { "text": "在临床怀疑结核病的患者,这一检测方法的敏感度是75%~88%,特异度达100%。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "结核病" } ] }, { "text": "(六)影像学由于至少有50%原发结核的患儿没有临床症状,因此,在结核菌素试验阳性的患儿,无论有无症状,都应拍胸片。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "原发结核" }, { "start_idx": 32, "end_idx": 37, "type": "pro", "entity": "结核菌素试验" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "胸片" } ] }, { "text": "应同时拍胸正位片和侧位片以确认有无肺部浸润拍正位片,淋巴结肿大肺部CT扫描除了有利于发现隐蔽处病灶外,在显示纵隔肺门淋巴结,肺内空洞、钙化,支气管充气像支气管扩张X线检查敏感。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肺部" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "肺部浸润" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "拍正位片" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "淋巴结" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "淋巴结肿大" }, { "start_idx": 31, "end_idx": 36, "type": "pro", "entity": "肺部CT扫描" }, { "start_idx": 54, "end_idx": 60, "type": "bod", "entity": "纵隔肺门淋巴结" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "支气管" }, { "start_idx": 70, "end_idx": 75, "type": "sym", "entity": "支气管充气像" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "支气管" }, { "start_idx": 76, "end_idx": 80, "type": "sym", "entity": "支气管扩张" }, { "start_idx": 81, "end_idx": 84, "type": "pro", "entity": "X线检查" } ] }, { "text": "因此,肺CT对诊断有重要的价值。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "肺CT" } ] }, { "text": "附:美国儿科学会胎儿新生儿委员会对吸入NO治疗的推荐意见1.出现低氧性呼吸衰竭的新生儿应该在(或及时转运到)具有多种呼吸支持救治手段的医疗中心治疗。", "entities": [ { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "吸入NO治疗" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "低氧性呼吸衰竭" }, { "start_idx": 58, "end_idx": 63, "type": "pro", "entity": "呼吸支持救治" } ] }, { "text": "2.给予吸入NO必须参考气体说明提供的针对指征、剂量、给药方式、监测方法等。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "吸入NO" } ] }, { "text": "建议应用多普勒彩超诊断排除先心病。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "pro", "entity": "多普勒彩超诊断" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "先心病" } ] }, { "text": "3.吸入NO应由专门训练的、会使用多种呼吸治疗的医师指导下进行,最好具备包括ECMO支持。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "吸入NO" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "呼吸治疗" }, { "start_idx": 38, "end_idx": 41, "type": "equ", "entity": "ECMO" } ] }, { "text": "4.一般吸入NO须在具备ECMO的医院进行,或先给予NO,同时与ECMO中心保持联系,一旦吸入NO失败,可以迅速转运到ECMO中心治疗。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "吸入NO" }, { "start_idx": 12, "end_idx": 15, "type": "equ", "entity": "ECMO" }, { "start_idx": 32, "end_idx": 35, "type": "equ", "entity": "ECMO" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "吸入NO" }, { "start_idx": 59, "end_idx": 62, "type": "equ", "entity": "ECMO" } ] }, { "text": "转运中必须保持吸入NO治疗NO治疗不中断。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "吸入NO治疗" } ] }, { "text": "5.治疗NO的医院必须长期随访患儿健康和神经经运动发育。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "神经" } ] }, { "text": "6.建立前瞻性资料收集,如治疗时间、不良反应、治疗失败、其他特殊呼吸治疗方式的应用、生存或死亡等。", "entities": [ { "start_idx": 30, "end_idx": 35, "type": "pro", "entity": "特殊呼吸治疗" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "生存" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "死亡" } ] }, { "text": "第二节慢性胰腺炎慢性胰腺炎(chronicpancreatitis)是指胰腺局部或弥漫性的慢性进行性炎症。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "胰腺" } ] }, { "text": "日益加重的胰实质损害导致胰腺内、外分泌功能进行性衰退。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胰腺" } ] }, { "text": "慢性胰腺炎在儿童中很少见。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "【病因】引起慢性胰腺炎的常见原因有三类,具体见表13-13。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "表13-15儿童慢性胰腺炎的病因【病理生理】慢性胰腺炎有两种主要的病理类型:钙化性和阻塞性。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "在儿童中,慢性钙化性胰腺炎见于遗传性胰腺炎和特发性胰腺炎。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "胰腺坚硬,手术时可触及钙石。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胰腺" } ] }, { "text": "当含有多种蛋白如消化酶、黏多糖以及糖蛋白的黏性塞子融合在导管腔内时,就会发生阻塞。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "消化酶" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "黏多糖" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "糖蛋白" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "黏性塞子" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "导管腔" } ] }, { "text": "也有推测有毒的代谢物加重胰腺的损害。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胰腺" } ] }, { "text": "胰石蛋白水平降低以及继发性钙结石的形成,可能是许多原因导致钙化性胰腺炎的共同通路。", "entities": [ { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "先天畸形或后天获得性疾病如肿瘤、纤维变性或损伤性狭窄阻塞主胰管时就会发生阻塞性胰腺炎。", "entities": [ { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "胰腺上皮发生炎症,被纤维组织替代。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胰腺" } ] }, { "text": "【临床表现】许多患者有反复发作的急性胰腺炎史。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "98%的胰腺功能丧失时才会出现外分泌功能不足的表现。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "胰腺" } ] }, { "text": "胰腺外分泌功能不足会导致营养不良、贪食及生长障碍、营养缺乏,尤其是脂溶性维生素、维生素B12以及必需脂肪酸的缺乏。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胰腺" } ] }, { "text": "(一)慢性钙化性胰腺炎1.遗传性胰腺炎是一种常染色体隐性遗传性疾病。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "遗传性胰腺炎的基因定位于7号染色体长臂。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "胰蛋白酶原的第117位的精氨酸被组氨酸替代,导致胰腺的自身消化并诱发胰腺炎。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "胰腺" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "病理发现包括胰腺萎缩、纤维化及钙化,腺泡细胞几乎均萎缩,导管堵塞以及广泛的纤维化。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "胰腺" } ] }, { "text": "体格检查和临床过程与其他原因所致的急性胰腺炎相似,急性期症状在4~8天后缓解。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "每次发作后都会加重胰腺组织损害。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "胰腺" } ] }, { "text": "当发展为慢性胰腺炎时,淀粉酶和脂肪酶在急性发作时可以正常。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "2.青少年热带性(营养性)胰腺炎热带性(营养性)胰腺炎是儿童慢性胰腺炎较常见的原因。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "临床过程与其他类型的慢性胰腺炎类似。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "(二)慢性阻塞性胰腺炎1.胰腺分裂症胰腺分裂症在总人群中的发病率为5%~15%,是胰腺最常见的畸形。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胰腺" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "胰腺" } ] }, { "text": "由于背侧和腹侧胰腺始基不能融合,导致胰尾、胰体和部分胰头通过相对狭窄的副胰管引流,而不是通过主胰管引流。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "胰腺" } ] }, { "text": "许多学者认为胰腺分裂症和复发性胰腺炎有关。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "胰腺" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "ERCP可以诊断胰腺分裂症。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胰腺" } ] }, { "text": "2.腹部外伤腹部受外伤后,胰腺导管的隐性损伤可以导致狭窄、假性囊肿形成以及慢性阻塞。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胰腺" } ] }, { "text": "(三)特发性纤维化胰腺炎特发性纤维化胰腺炎很罕见,可以有腹痛或阻塞性黄疸。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "(四)其他高脂血症Ⅰ、Ⅳ、Ⅴ型患者可以发生胰腺炎。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "胰腺炎时可以出现一过性高脂血症,所以急性胰腺炎时的血脂升高必须在好转后重测。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "【诊断】根据患者有典型的胰腺炎病史,以及影像学上有慢性征象,慢性胰腺炎很容易诊断。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "更典型的是患者有反复发作的腹痛、呕吐及血清淀粉酶升高。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "腹痛" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "呕吐" } ] }, { "text": "3.胰腺功能测试如核素脂肪试验、CCK-促胰液素、BT-PABA试验等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "胰腺" } ] }, { "text": "无任何胰功能试验有足够敏感性可诊断出轻度早期甚至中度胰腺炎。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "4.腹部平片腹部平片见到胰腺钙化,则慢性胰腺炎的诊断成立。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胰腺" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "5.ERCP诊断慢性胰腺炎的敏感性最高。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "【治疗】1.疼痛对于儿童慢性胰腺炎,控制疼痛很重要,但有时却很困难。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "可以用甾体类或非甾体类抗炎药;补充胰酶以抑制胆囊收缩素的分泌;药物治疗失败时,可行内镜下或外科手术解除胰管梗阻。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "胰酶" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "胆囊收缩素" } ] }, { "text": "2.吸收不良慢性胰腺炎时吸收不良常见,但儿童中的发生率尚不清楚。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "第十二节膀胱外翻与尿道上裂膀胱外翻和尿道上裂是由泄殖腔发育异常所导致的一组相互关联的泌尿生殖系畸形,尤其是膀胱外翻,是最为复杂的小儿先天性畸形之一。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "dis", "entity": "膀胱外翻与尿道上裂" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "膀胱外翻和尿道上裂" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "泄殖腔发育异常" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "泌尿生殖系畸形" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "膀胱外翻" }, { "start_idx": 64, "end_idx": 70, "type": "dis", "entity": "小儿先天性畸形" } ] }, { "text": "一、膀胱外翻膀胱外翻是1597年由VonGrafenberg首先描述,1780年Chaussier始用膀胱外翻(bladderexstrophy)一词。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "膀胱外翻" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "膀胱外翻" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "膀胱外翻" }, { "start_idx": 56, "end_idx": 71, "type": "dis", "entity": "bladderexstrophy" } ] }, { "text": "1885年,Nyman在一位5天的膀胱外翻新生儿中进行了膀胱关闭。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "膀胱外翻" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "膀胱关闭" } ] }, { "text": "其后的实践包括试用骨盆截骨使耻骨靠近、回肠扩大膀胱提高膀胱容量等。", "entities": [ { "start_idx": 9, "end_idx": 30, "type": "pro", "entity": "骨盆截骨使耻骨靠近、回肠扩大膀胱提高膀胱容量" } ] }, { "text": "1942年Young和1948年Micbon分别报道首例女性及男性膀胱外翻关闭术后能控制排尿。", "entities": [ { "start_idx": 33, "end_idx": 39, "type": "pro", "entity": "膀胱外翻关闭术" } ] }, { "text": "膀胱外翻并不常见,发病率为1/10000~1/50000,男性为女性1.7~2.3倍,有一定的遗传倾向,如报告膀胱外翻和尿道上裂患者子女的发病率为1/70,是正常人群发病率的500倍。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "膀胱外翻" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "膀胱外翻" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "尿道上裂" } ] }, { "text": "【胚胎发生】胚胎3周时后肠末端和尿囊基部的扩大部分成为泄殖腔,其尾端有一层由内、外胚层组成的薄膜与羊膜腔分隔,称为泄殖腔膜。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肠末端" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "尿囊基部" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "泄殖腔" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "外胚层" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "羊膜腔" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "泄殖腔膜" } ] }, { "text": "胚胎第4~7周泄殖腔被尿直肠膈分为背侧的直肠与腹侧的尿生殖窦,尿直肠膈与泄殖腔膜会合处形成会阴体。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "泄殖腔" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "尿直肠膈" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "直肠" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "尿生殖窦" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "尿直肠膈" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "泄殖腔膜" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "会阴体" } ] }, { "text": "胚胎第4~10周时泄殖腔膜内、外胚层之间有间充质向内生长,发育成下腹部的肌肉和耻骨,构成脐以下的腹壁。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "泄殖腔膜" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "下腹部" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肌肉" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "耻骨" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "腹壁" } ] }, { "text": "泄殖腔膜发育不正常将阻碍间充质组织的移行,影响下腹壁发育。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "泄殖腔膜" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "下腹壁" } ] }, { "text": "如泄殖腔膜有破溃,则其破溃的位置和时间的不同决定了膀胱外翻、尿道上裂系列畸形的各种类型。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "泄殖腔膜" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "膀胱外翻" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "尿道上裂" } ] }, { "text": "其中典型膀胱外翻占50%~60%,尿道上裂约占30%,最严重的泄殖腔发育异常是泄殖腔外翻(cloacalexstrophy),占l0%,其他畸形包括尿道上裂合并重复膀胱等。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "膀胱外翻" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "尿道上裂" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "泄殖腔发育异常" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "泄殖腔外翻" }, { "start_idx": 45, "end_idx": 60, "type": "dis", "entity": "cloacalexstrophy" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "尿道上裂" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "重复膀胱" } ] }, { "text": "【临床表现】膀胱外翻涉及整个下腹部及盆腔脏器的发育异常,包括腹壁肌肉、骨盆骨骼、泌尿生殖系统及直肠肛门。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "膀胱外翻" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "下腹部" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "盆腔脏器" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "腹壁肌肉" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "骨盆骨骼" }, { "start_idx": 40, "end_idx": 45, "type": "bod", "entity": "泌尿生殖系统" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "直肠肛门" } ] }, { "text": "骨骼肌肉异常表现为耻骨联合分离、骨盆外旋、耻骨支外旋及外转。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "骨骼肌肉异常" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "耻骨联合分离" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "骨盆外旋" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "耻骨支外旋及外转" } ] }, { "text": "分离的耻骨之间三角形筋膜缺损由外翻膀胱占据,膀胱上端是脐,位置低于两侧髂嵴连线,脐与肛门之间距离缩短。", "entities": [ { "start_idx": 0, "end_idx": 20, "type": "sym", "entity": "分离的耻骨之间三角形筋膜缺损由外翻膀胱占据" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "膀胱" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "脐" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "肛门" }, { "start_idx": 22, "end_idx": 49, "type": "sym", "entity": "膀胱上端是脐,位置低于两侧髂嵴连线,脐与肛门之间距离缩短" } ] }, { "text": "泌尿系统异常表现为下腹壁缺如,膀胱外翻,可见喷尿的两侧输尿管口。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "泌尿系统异常" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "下腹壁缺如" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "膀胱外翻" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "可见喷尿的两侧输尿管口" } ] }, { "text": "出生时外翻的膀胱黏膜正常,异位肠黏膜或岛状肠襻可位于外翻膀胱表面。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "膀胱黏膜" }, { "start_idx": 13, "end_idx": 31, "type": "sym", "entity": "异位肠黏膜或岛状肠襻可位于外翻膀胱表面" } ] }, { "text": "长期暴露的黏膜可有鳞状上皮化生、炎性水肿及炎性息肉。", "entities": [ { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "长期暴露的黏膜可有鳞状上皮化生、炎性水肿及炎性息肉" } ] }, { "text": "膀胱容量差别很大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "膀胱" } ] }, { "text": "如膀胱过小,严重纤维化,难于做功能性修复。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "sym", "entity": "膀胱过小,严重纤维化" } ] }, { "text": "由于阴茎海绵体附着于耻骨下支,耻骨联合分离使两侧阴茎海绵体分离很宽,阴茎变短,阴茎严重向背侧弯曲。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "阴茎海绵体" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "耻骨下支" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "耻骨" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "阴茎海绵体" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "阴茎" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "阴茎" } ] }, { "text": "阴茎头靠近精阜,尿道板短。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "阴茎头靠近精阜" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "尿道板短" } ] }, { "text": "女性尿道短,阴道口前移并常有狭窄,阴蒂对裂,阴唇阴阜分开。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "尿道" }, { "start_idx": 6, "end_idx": 15, "type": "sym", "entity": "阴道口前移并常有狭窄" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "阴蒂对裂" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "阴唇阴阜分开" } ] }, { "text": "子宫、输卵管及卵巢一般正常,有时副中肾管结构重复。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "子宫" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "输卵管" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "卵巢" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "副中肾管结构重复" } ] }, { "text": "如有肛提肌、坐骨直肠肌以及外括约肌异常,可引起不同程度肛门失禁以及脱肛。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肛提肌" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "坐骨直肠肌" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "外括约肌" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "肛门失禁" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "脱肛" } ] }, { "text": "【治疗】手术重建是唯一的治疗方法,包括修复腹壁和外翻膀胱,在男性重建外观接近正常并且有性功能的阴茎,使能控制排尿,保护肾功能。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "手术重建" }, { "start_idx": 19, "end_idx": 27, "type": "pro", "entity": "修复腹壁和外翻膀胱" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "阴茎" } ] }, { "text": "手术多主张分期完成,新生儿期修复腹壁和外翻膀胱,至2岁时修复尿道上裂,3~4岁时做双侧抗反流输尿管移植、后尿道延长及膀胱颈紧缩成形术。", "entities": [ { "start_idx": 10, "end_idx": 22, "type": "pro", "entity": "新生儿期修复腹壁和外翻膀胱" }, { "start_idx": 25, "end_idx": 33, "type": "pro", "entity": "2岁时修复尿道上裂" }, { "start_idx": 35, "end_idx": 65, "type": "pro", "entity": "3~4岁时做双侧抗反流输尿管移植、后尿道延长及膀胱颈紧缩成形术" } ] }, { "text": "如膀胱容量小,同时进行膀胱扩大。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "膀胱容量小" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "进行膀胱扩大" } ] }, { "text": "在修复腹壁和外翻膀胱时必须做髂骨截骨。", "entities": [ { "start_idx": 1, "end_idx": 17, "type": "pro", "entity": "修复腹壁和外翻膀胱时必须做髂骨截骨" } ] }, { "text": "多数作者主张在生后72小时内做膀胱内翻缝合。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "膀胱内翻缝合" } ] }, { "text": "其优点为:膀胱壁柔软易于复位;尽早使膀胱黏膜不受外界刺激,避免一系列继发改变和废用性膀胱萎缩;有助于排尿控制。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "膀胱壁" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "膀胱黏膜" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "废用性膀胱萎缩" } ] }, { "text": "1998年,美国西雅图儿童医院的Grady和Mitchell报告在新生儿期一期完成腹壁修复、骨盆成形、膀胱成形和尿道成形,16例外观均非常满意,其中11例已接受排尿训练,可以尿不失禁。", "entities": [ { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "腹壁修复" }, { "start_idx": 46, "end_idx": 49, "type": "pro", "entity": "骨盆成形" }, { "start_idx": 51, "end_idx": 59, "type": "pro", "entity": "膀胱成形和尿道成形" } ] }, { "text": "膀胱外翻的一系列修复手术的效果应该说是在逐渐进步中,但还难令人满意,如患儿控制排尿率约在60%左右。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "膀胱外翻" } ] }, { "text": "一部分患儿要做尿流改道。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "尿流改道" } ] }, { "text": "第五章儿童淋巴瘤第一节儿童霍奇金病1832年,霍奇金(Hodgkin)首先对本病在解剖学水平进行描述,因此而命名为霍奇金病(Hodgkindisease,HD),当时认为它是一种脾脏和淋巴结异常性疾病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "儿童淋巴瘤" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "儿童霍奇金病" }, { "start_idx": 57, "end_idx": 60, "type": "dis", "entity": "霍奇金病" }, { "start_idx": 62, "end_idx": 75, "type": "dis", "entity": "Hodgkindisease" }, { "start_idx": 77, "end_idx": 78, "type": "dis", "entity": "HD" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "脾脏" }, { "start_idx": 92, "end_idx": 94, "type": "bod", "entity": "淋巴结" } ] }, { "text": "直至19世纪50年代以后,由于显微镜技术的发展才对本病有了更进一步的了解,镜下观察到巨大畸形的细胞作为霍奇金病的诊断依据。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "equ", "entity": "显微镜" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "巨大畸形的细胞" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "霍奇金病" } ] }, { "text": "Sternberg和Reed分别在1898年和1902年对霍奇金病的组织病理学变化作了全面的定义和说明。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "霍奇金病" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "组织" } ] }, { "text": "霍奇金病的浸润细胞有多样性,多数为形态正常的反应性细胞,其中的R-S细胞由相对成熟的淋巴细胞恶性转化而来。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "霍奇金病" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "浸润细胞" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "R-S细胞" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "【流行病学】根据我国上海市肿瘤登记系统,1986~1992年间,0~14岁组儿童HD的年发病率为2.39/百万,男女比为2.3∶1。", "entities": [ { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "儿童HD" } ] }, { "text": "【组织病理学】病变组织中常有正常淋巴细胞、浆细胞、嗜酸粒细胞、组织细胞反应性浸润,伴有细胞形态异常的R-S细胞。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "浆细胞" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "嗜酸粒细胞" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "组织细胞" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "R-S细胞" } ] }, { "text": "R-S细胞大而畸形,直径≥15~45μm,有丰富的胞质,多核或多叶核,核膜染色深,有细致的染色质网,在核仁周围形成淡染的圈影、核仁大而明显。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "R-S细胞" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "胞质" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "核膜" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "染色质网" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "核仁" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "核仁" }, { "start_idx": 0, "end_idx": 68, "type": "sym", "entity": "R-S细胞大而畸形,直径≥15~45μm,有丰富的胞质,多核或多叶核,核膜染色深,有细致的染色质网,在核仁周围形成淡染的圈影、核仁大而明显" } ] }, { "text": "未见到R-S细胞时很难诊断本病,但在其他一些疾病中如传染性单核细胞增多症、非霍奇金淋巴瘤及其他非淋巴系恶性肿瘤中也可见到类似细胞。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "R-S细胞" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "单核细胞增多症" }, { "start_idx": 37, "end_idx": 43, "type": "dis", "entity": "非霍奇金淋巴瘤" }, { "start_idx": 47, "end_idx": 54, "type": "dis", "entity": "非淋巴系恶性肿瘤" } ] }, { "text": "1966年提出RYE分类系统,将HD分为4个组织学亚型。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "HD" } ] }, { "text": "表11-12HD分型标准变迁及WHO-2008分型标准【临床表现】(一)全身症状非特异性症状包括发热、乏力、厌食、轻度消瘦、瘙痒。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "HD" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "发热" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "乏力" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "厌食" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "轻度消瘦" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "瘙痒" } ] }, { "text": "(二)淋巴结肿大无痛性锁骨上、颈部或其他部位淋巴结肿大淋巴结质硬有橡皮样感觉。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "淋巴结肿大" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "锁骨" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "颈部" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "淋巴结" }, { "start_idx": 8, "end_idx": 26, "type": "sym", "entity": "无痛性锁骨上、颈部或其他部位淋巴结肿大" }, { "start_idx": 27, "end_idx": 37, "type": "sym", "entity": "淋巴结质硬有橡皮样感觉" } ] }, { "text": "肿瘤原发于锁骨上、颈部较多见,腋下、腹股沟、腹腔淋巴结为原发者相对少见。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "锁骨" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "颈部" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "腋下" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "腹股沟" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "腹腔淋巴结" } ] }, { "text": "(三)可合并免疫功能紊乱如合并免疫性溶血性贫血,有贫血、黄疸、网织红细胞升高、Coomb试验阳性。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "免疫功能紊乱" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "免疫性溶血性贫血" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "贫血" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "黄疸" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "网织红细胞升高" } ] }, { "text": "合并免疫性血小板减少症时,有血小板减少、出血倾向、血小板相关抗体增高髓巨核细胞成熟障碍。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "免疫性血小板减少症" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "血小板减少" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "出血倾向" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "抗体" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "血小板相关抗体增高" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "髓巨核细胞成熟障碍" } ] }, { "text": "【实验室检查】(一)血液学检查血常规检查常无特异性异常,偶可见到嗜酸粒细胞或单核细胞增多。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "血液学检查" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "血常规检查" }, { "start_idx": 28, "end_idx": 43, "type": "sym", "entity": "偶可见到嗜酸粒细胞或单核细胞增多" } ] }, { "text": "血沉可增快。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "血沉可增快" } ] }, { "text": "(二)淋巴结活检病理组织形态检查是确诊的必需手段。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "淋巴结活检" }, { "start_idx": 8, "end_idx": 15, "type": "pro", "entity": "病理组织形态检查" } ] }, { "text": "(三)影像学检查可选择性作胸部X线平片、腹部B超、胸部CT、腹部CT,以确定病变的范围。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "影像学检查" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "胸部X线平片" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "腹部B超" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "胸部CT" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "腹部CT" } ] }, { "text": "由于67镓对淋巴组织亲和力高,可作67镓扫描作为补充检查确定肿瘤浸润范围。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "淋巴组织" } ] }, { "text": "(四)骨髓活检HD可发生灶性骨髓转移,因此骨髓活检比骨髓涂片容易发现肿瘤细胞,在治疗前应常规作骨髓活检。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "骨髓活检" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "HD" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "骨髓活检" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "骨髓涂片" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "骨髓活检" } ] }, { "text": "【诊断】完整的诊断必须包括疾病分期,以指导临床治疗与随访,根据体格检查及相关实验室检查可作出分期诊断,较常用的HD分期系统为AnnArbor分期(表11-11)。", "entities": [ { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "HD" } ] }, { "text": "表11-13HD的分期【治疗】主要的治疗手段为化疗和放疗。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "HD" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "化疗" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "放疗" } ] }, { "text": "(一)放疗HD对放疗敏感,20世纪70年代以前,无论年龄、分型和分期的差别均采用放疗。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "放疗" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "放疗" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "放疗" } ] }, { "text": "目前对生长期儿童主要采用联合化疗加肿瘤浸润野低剂量放疗,有试图进一步减少或删除放疗的倾向。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "联合化疗" }, { "start_idx": 19, "end_idx": 26, "type": "pro", "entity": "浸润野低剂量放疗" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "放疗" } ] }, { "text": "对已完全发育的青少年局限性病变采用肿瘤扩大野高剂量放疗。", "entities": [ { "start_idx": 19, "end_idx": 26, "type": "pro", "entity": "扩大野高剂量放疗" } ] }, { "text": "常用的放疗野有以下几个:1.Waldeyer野用于Waldeyer或耳前淋巴结病变。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "放疗" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "Waldeyer" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "耳前淋巴结病变" } ] }, { "text": "上颈部病变并以放疗为单一治疗手段时应同时作Waldeyer野预防性放疗。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "上颈部病变" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "放疗" }, { "start_idx": 21, "end_idx": 29, "type": "dis", "entity": "Waldeyer野" } ] }, { "text": "2.横膈上斗篷样野包括颌下、颏下、颈部、锁骨上下、腋下、纵隔和肺门淋巴结。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "颌下" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "颏下" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "颈部" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "锁骨上下" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "腋下" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "纵隔" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "肺门淋巴结" } ] }, { "text": "3.横膈下野包括脾和主动脉旁淋巴结。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "脾" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "主动脉旁淋巴结" } ] }, { "text": "4.倒“Y”野包括髂总、髂外、腹股沟淋巴结。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "髂总" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "髂外" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "腹股沟淋巴结" } ] }, { "text": "(二)化疗经典联合化疗方案MOPP对成人与儿童的晚期HD有50%的治愈率。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "化疗" }, { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "联合化疗" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "HD" } ] }, { "text": "化疗剂量宜大,但过长的维持治疗并不延长缓解期,根据不同分期以4~6个疗程为宜。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" } ] }, { "text": "表11-14HD化疗方案*MOPP中M为氮芥**COPP中C为环磷酰胺【预后】HD在合理的治疗下预后良好,治愈率可达80%~90%,但反复复发的晚期广泛病变预后仍不良,HD可见远期复发。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "HD" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "化疗" }, { "start_idx": 26, "end_idx": 27, "type": "dru", "entity": "氮芥" }, { "start_idx": 43, "end_idx": 46, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "HD" } ] }, { "text": "常见的与放疗、化疗相关并影响远期生活质量的并发症有放疗部位的软组织、骨骼发育不良及畸形,放疗野内脏器功能障碍,心、肺功能障碍,不育和第二肿瘤等。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "放疗" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "化疗" }, { "start_idx": 30, "end_idx": 42, "type": "dis", "entity": "软组织、骨骼发育不良及畸形" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "脏器功能障碍" }, { "start_idx": 55, "end_idx": 61, "type": "dis", "entity": "心、肺功能障碍" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "不育" }, { "start_idx": 66, "end_idx": 69, "type": "dis", "entity": "第二肿瘤" } ] }, { "text": "第三章小儿急性肺损伤和急性呼吸窘迫综合征【概述】小儿呼吸系统危重症中急性呼吸窘迫综合征(acuterespiratorydistresssyndrome,ARDS)是由于多种原发病和诱因作用下发生的急性呼吸衰竭,以非心源性肺水肿和顽固性低氧血症为特征,表现为肺顺应性降低、严重呼吸困难、呼吸窘迫;其病理特征为肺泡毛细血管损伤、膜通透性增高、肺表面活性物质失去功能、弥漫性肺损伤和蛋白性肺泡水肿液聚集,发病机制本质上是“全身炎症反应综合征在肺部的表现”。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "小儿急性肺损伤" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "急性呼吸窘迫综合征" }, { "start_idx": 24, "end_idx": 32, "type": "dis", "entity": "小儿呼吸系统危重症" }, { "start_idx": 34, "end_idx": 42, "type": "dis", "entity": "急性呼吸窘迫综合征" }, { "start_idx": 44, "end_idx": 75, "type": "dis", "entity": "acuterespiratorydistresssyndrome" }, { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "ARDS" }, { "start_idx": 99, "end_idx": 104, "type": "dis", "entity": "急性呼吸衰竭" }, { "start_idx": 111, "end_idx": 111, "type": "bod", "entity": "肺" }, { "start_idx": 107, "end_idx": 113, "type": "sym", "entity": "非心源性肺水肿" }, { "start_idx": 115, "end_idx": 121, "type": "dis", "entity": "顽固性低氧血症" }, { "start_idx": 129, "end_idx": 129, "type": "bod", "entity": "肺" }, { "start_idx": 129, "end_idx": 134, "type": "sym", "entity": "肺顺应性降低" }, { "start_idx": 136, "end_idx": 141, "type": "sym", "entity": "严重呼吸困难" }, { "start_idx": 143, "end_idx": 146, "type": "sym", "entity": "呼吸窘迫" }, { "start_idx": 154, "end_idx": 155, "type": "bod", "entity": "肺泡" }, { "start_idx": 154, "end_idx": 161, "type": "sym", "entity": "肺泡毛细血管损伤" }, { "start_idx": 163, "end_idx": 168, "type": "sym", "entity": "膜通透性增高" }, { "start_idx": 170, "end_idx": 176, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 170, "end_idx": 180, "type": "sym", "entity": "肺表面活性物质失去功能" }, { "start_idx": 182, "end_idx": 187, "type": "sym", "entity": "弥漫性肺损伤" }, { "start_idx": 192, "end_idx": 193, "type": "bod", "entity": "肺泡" }, { "start_idx": 189, "end_idx": 198, "type": "sym", "entity": "蛋白性肺泡水肿液聚集" }, { "start_idx": 211, "end_idx": 217, "type": "sym", "entity": "炎症反应综合征" }, { "start_idx": 219, "end_idx": 220, "type": "bod", "entity": "肺部" } ] }, { "text": "与新生儿RDS不同的是,ARDS起病多有一段时间,可以由肺内、外多种原因造成,多见于感染、严重外伤等。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "新生儿RDS" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "ARDS" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肺" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "感染" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "严重外伤" } ] }, { "text": "小儿ARDS可被作为重症肺炎、呼吸衰竭进行诊治。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "小儿ARDS" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "重症肺炎" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "ARDS早期肺病变为急性肺损伤(acutelunginjury,ALI),由于病情发展迅速,ALI发展为ARDS往往是不典型的;因此,在儿科危重呼吸疾病中发现临床病例特点为急性发病、肺部感染和高渗出性病变时,要考虑作为ARDS慎重处理。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "ARDS" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肺病" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "急性肺损伤" }, { "start_idx": 16, "end_idx": 30, "type": "dis", "entity": "acutelunginjury" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "ALI" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "ALI" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "ARDS" }, { "start_idx": 68, "end_idx": 75, "type": "dis", "entity": "儿科危重呼吸疾病" }, { "start_idx": 91, "end_idx": 92, "type": "bod", "entity": "肺部" }, { "start_idx": 91, "end_idx": 94, "type": "sym", "entity": "肺部感染" }, { "start_idx": 96, "end_idx": 101, "type": "sym", "entity": "高渗出性病变" }, { "start_idx": 109, "end_idx": 112, "type": "dis", "entity": "ARDS" } ] }, { "text": "【流行病学】有关ARDS在我国儿童人群中的发病率尚不清,分析ARDS在儿科ICU病房年收治病例的比例,发达国家20世纪80~90年代小儿ARDS发病率约占ICU收治数的1%;国内孙波教授主持的中国儿童呼吸衰竭协作组2009年12月结束的26个PICU急性低氧性呼吸衰竭和ARDS的研究表明,PICU中2.7%的危重病例发生了ARDS,肺炎和脓毒症为其主要原因。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "ARDS" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "ARDS" }, { "start_idx": 35, "end_idx": 39, "type": "dep", "entity": "儿科ICU" }, { "start_idx": 66, "end_idx": 71, "type": "dis", "entity": "小儿ARDS" }, { "start_idx": 77, "end_idx": 79, "type": "dep", "entity": "ICU" }, { "start_idx": 100, "end_idx": 103, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 121, "end_idx": 124, "type": "dep", "entity": "PICU" }, { "start_idx": 125, "end_idx": 133, "type": "dis", "entity": "急性低氧性呼吸衰竭" }, { "start_idx": 135, "end_idx": 138, "type": "dis", "entity": "ARDS" }, { "start_idx": 145, "end_idx": 148, "type": "dep", "entity": "PICU" }, { "start_idx": 162, "end_idx": 165, "type": "dis", "entity": "ARDS" }, { "start_idx": 167, "end_idx": 168, "type": "dis", "entity": "肺炎" }, { "start_idx": 170, "end_idx": 172, "type": "dis", "entity": "脓毒症" } ] }, { "text": "目前,儿科患儿ARDS诊断标准主要参考成人,其治疗和预后的研究均有待通过多中心前瞻性临床研究来解答。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dep", "entity": "儿科" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "ARDS" } ] }, { "text": "小儿ARDS病死率和临床转归与基础疾病性质关系非常密切;在农村、内地、边远地区仍多见由感染性肺炎发展为重症肺炎呼吸衰竭和ARDS;在沿海、大城市儿科重症肺炎发病呈渐降趋势,但恶病质病变、依赖免疫抑制疗法的基础疾病中有较高趋势,如白血病、恶性肿瘤、艾滋病、结缔组织病等,且病死率非常高,可以达到80%~90%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "小儿ARDS" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "感染性肺炎" }, { "start_idx": 51, "end_idx": 58, "type": "dis", "entity": "重症肺炎呼吸衰竭" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "ARDS" }, { "start_idx": 72, "end_idx": 73, "type": "dep", "entity": "儿科" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "重症肺炎" }, { "start_idx": 95, "end_idx": 100, "type": "pro", "entity": "免疫抑制疗法" }, { "start_idx": 114, "end_idx": 116, "type": "dis", "entity": "白血病" }, { "start_idx": 118, "end_idx": 121, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 123, "end_idx": 125, "type": "dis", "entity": "艾滋病" }, { "start_idx": 127, "end_idx": 131, "type": "dis", "entity": "结缔组织病" } ] }, { "text": "【病因和发病机制】(一)病因和危险因素小儿ARDS主要致病原因为三大类:感染性肺部损伤、急性创伤性肺损伤以及免疫抑制性肺损伤。", "entities": [ { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "小儿ARDS" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "感染性肺部损伤" }, { "start_idx": 44, "end_idx": 51, "type": "dis", "entity": "急性创伤性肺损伤" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "免疫抑制性肺损伤" } ] }, { "text": "间接性肺损伤如休克、败血症、创伤和烧伤、大量输血、弥散性血管内凝血、药物性伤害、代谢性疾病等成为致病的主要危险因素。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "间接性肺损伤" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "休克" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "败血症" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "创伤" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "烧伤" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "大量输血" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "血管" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "弥散性血管内凝血" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "药物性伤害" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "代谢性疾病" } ] }, { "text": "近年来,脏器移植手术后抗排斥药物和肿瘤化疗导致免疫功能低下并发肺部感染,主要为真菌、卡氏肺囊虫、条件性致病菌和病毒感染发展为ARDS,其预后差,此类免疫剥夺、免疫低下患儿的急性感染性肺损伤和ARDS,成为制约相关学科发展的重要疾病。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "脏器移植手术" }, { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "抗排斥药物" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "肿瘤化疗" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "免疫功能低下" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "肺部感染" }, { "start_idx": 39, "end_idx": 40, "type": "mic", "entity": "真菌" }, { "start_idx": 42, "end_idx": 46, "type": "mic", "entity": "卡氏肺囊虫" }, { "start_idx": 48, "end_idx": 53, "type": "mic", "entity": "条件性致病菌" }, { "start_idx": 55, "end_idx": 56, "type": "mic", "entity": "病毒" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "ARDS" }, { "start_idx": 86, "end_idx": 93, "type": "dis", "entity": "急性感染性肺损伤" }, { "start_idx": 95, "end_idx": 98, "type": "dis", "entity": "ARDS" } ] }, { "text": "(二)病理生理对肺部的直接或间接损伤、感染或非感染性损伤,在ARDS发生、发展过程中,机制上存在异同。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肺部" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "ARDS" } ] }, { "text": "主要病理生理机制有以下几方面:1.炎症细胞的迁移与聚集ARDS的本质目前被认为是“全身炎症反应综合征的肺部表现”。", "entities": [ { "start_idx": 17, "end_idx": 26, "type": "sym", "entity": "炎症细胞的迁移与聚集" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "ARDS" }, { "start_idx": 41, "end_idx": 49, "type": "dis", "entity": "全身炎症反应综合征" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "肺部" } ] }, { "text": "肺部是急性炎症最重要的效应器官之一,创伤、脓毒血症、急性胰腺炎、理化刺激或体外循环等情况下,内毒素脂多糖(LPS)、C5a、白细胞介素-8(IL-8)、黏附分子等因子作用的参与、调控下,中性粒细胞(PMNs)、肺泡巨噬细胞(AMs)在肺毛细血管、肺泡内大量聚集,附壁、黏附并移行到肺间质,释放产物损伤肺。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "急性炎症" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "创伤" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "脓毒血症" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "急性胰腺炎" }, { "start_idx": 46, "end_idx": 51, "type": "bod", "entity": "内毒素脂多糖" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "LPS" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "C5a" }, { "start_idx": 62, "end_idx": 68, "type": "bod", "entity": "白细胞介素-8" }, { "start_idx": 70, "end_idx": 73, "type": "bod", "entity": "IL-8" }, { "start_idx": 76, "end_idx": 79, "type": "bod", "entity": "黏附分子" }, { "start_idx": 93, "end_idx": 97, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 99, "end_idx": 102, "type": "bod", "entity": "PMNs" }, { "start_idx": 105, "end_idx": 110, "type": "bod", "entity": "肺泡巨噬细胞" }, { "start_idx": 112, "end_idx": 114, "type": "bod", "entity": "AMs" }, { "start_idx": 117, "end_idx": 121, "type": "bod", "entity": "肺毛细血管" }, { "start_idx": 123, "end_idx": 124, "type": "bod", "entity": "肺泡" }, { "start_idx": 140, "end_idx": 142, "type": "bod", "entity": "肺间质" }, { "start_idx": 150, "end_idx": 150, "type": "bod", "entity": "肺" } ] }, { "text": "炎症介质释放是启动和推动ARDS“炎症瀑布”、细胞趋化、跨膜迁移和聚集、次级介质释放的重要介导物质;并激活肺部局部凝血机制,导致凝血功能的紊乱,血小板聚集和微栓塞是ARDS常见病理改变(图6-6)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "炎症" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "ARDS" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "细胞" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "肺部" }, { "start_idx": 64, "end_idx": 70, "type": "sym", "entity": "凝血功能的紊乱" }, { "start_idx": 72, "end_idx": 74, "type": "ite", "entity": "血小板" }, { "start_idx": 72, "end_idx": 76, "type": "sym", "entity": "血小板聚集" }, { "start_idx": 78, "end_idx": 80, "type": "sym", "entity": "微栓塞" }, { "start_idx": 82, "end_idx": 85, "type": "dis", "entity": "ARDS" } ] }, { "text": "图6-5机体对于直接或间接肺损伤的反应机制2.肺泡高通透性及液体转运障碍肺泡的屏障作用由肺泡上皮细胞和肺泡毛细血管内皮形成,维持气血交换。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "直接或间接肺损伤" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺泡" }, { "start_idx": 23, "end_idx": 35, "type": "sym", "entity": "肺泡高通透性及液体转运障碍" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肺泡" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 51, "end_idx": 58, "type": "bod", "entity": "肺泡毛细血管内皮" } ] }, { "text": "肺血流量与全身血流量相近,大量血流通过肺部,对于维持肺部和全身液体平衡起到重要作用。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 1, "end_idx": 3, "type": "ite", "entity": "血流量" }, { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "血流量" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血流" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肺部" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肺部" } ] }, { "text": "肺泡上皮细胞和毛细血管形成的屏障保护作用,表现在肺泡上皮细胞(与肺泡巨噬细胞)阻碍外界有害物质进入循环,肺毛细血管网可与血液细胞和血流中有害物质发生反应,并过滤和阻碍循环内有害物质进入肺泡。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "毛细血管" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "肺泡巨噬细胞" }, { "start_idx": 52, "end_idx": 57, "type": "bod", "entity": "肺毛细血管网" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "血液细胞" }, { "start_idx": 92, "end_idx": 93, "type": "bod", "entity": "肺泡" } ] }, { "text": "ARDS的病理、病理生理和临床过程基本上不依赖于特定病因,共同基础是肺泡-毛细血管的急性损伤。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "ARDS" }, { "start_idx": 34, "end_idx": 45, "type": "dis", "entity": "肺泡-毛细血管的急性损伤" } ] }, { "text": "直接和间接损伤以及氧自由基、蛋白酶、细胞因子、花生四烯酸代谢产物以及高荷电产物(如中性粒细胞主要阳离子蛋白)等可以改变膜屏障的通透性;ARDS时,液体清除障碍主要为渗出性肺水肿。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "氧自由基" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "蛋白酶" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "细胞因子" }, { "start_idx": 23, "end_idx": 31, "type": "bod", "entity": "花生四烯酸代谢产物" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "高荷电产物" }, { "start_idx": 41, "end_idx": 52, "type": "bod", "entity": "中性粒细胞主要阳离子蛋白" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "膜屏障" }, { "start_idx": 67, "end_idx": 70, "type": "dis", "entity": "ARDS" }, { "start_idx": 82, "end_idx": 87, "type": "dis", "entity": "渗出性肺水肿" } ] }, { "text": "血管渗出液在肺泡间质和肺泡内积聚,严重影响肺顺应性,降低肺功能余气量(FRC),增加无效腔量。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "血管渗出液" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "肺泡间质" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肺泡" }, { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "血管渗出液在肺泡间质和肺泡内积聚" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肺" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "降低肺功能余气量" } ] }, { "text": "在血管损伤导致大量渗漏,或持续低氧导致细胞代谢障碍,超过了淋巴管的代偿能力,大量液体将在肺内滞留并大量进入肺泡腔内。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "血管损伤" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "持续低氧" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "细胞代谢障碍" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "淋巴管" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "肺" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "肺泡腔" } ] }, { "text": "呼吸膜屏障结构只容许液体通过,使大分子蛋白受阻挡,因此肺泡液和血浆的蛋白比值小于0.5,为静水压水肿漏出液。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "呼吸膜屏障" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "大分子蛋白" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肺泡液" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "血浆" }, { "start_idx": 45, "end_idx": 52, "type": "bod", "entity": "静水压水肿漏出液" } ] }, { "text": "当心功能不全、静水压增大时,蛋白漏出少,肺泡间质的蛋白稀释,胶体渗透压梯度有利于间质液体向血管内转移,也可以促使液体吸收,也起到代偿作用。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "心功能不全" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "静水压增大" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "蛋白" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "蛋白漏出少" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "肺泡间质" }, { "start_idx": 20, "end_idx": 28, "type": "sym", "entity": "肺泡间质的蛋白稀释" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "血管" } ] }, { "text": "直接测定肺内液体的蛋白含量,可以判断出肺泡内渗出的性质。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "肺内液体" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "蛋白" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肺泡" } ] }, { "text": "如果血管和肺泡损伤导致大分子蛋白也可以通过膜屏障,则肺泡液和血浆的蛋白比值大于0.7,为胶体性水肿渗出液。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "血管和肺泡损伤" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "肺泡液" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "血浆" }, { "start_idx": 44, "end_idx": 51, "type": "bod", "entity": "胶体性水肿渗出液" } ] }, { "text": "当血管在低灌流压力下大量漏出,并失去间质低胶体渗透压保护作用时,大量液体开始迅速在肺内积聚,出现ARDS临床危象。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "血管" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "肺" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "ARDS" } ] }, { "text": "在低氧血症时,肺泡上皮细胞钠-钾-ATP酶活性下降,使电解质和水代谢失平衡,也导致肺水肿。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "低氧血症" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "肺泡上皮细胞" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "钠-钾-ATP酶" }, { "start_idx": 7, "end_idx": 24, "type": "sym", "entity": "肺泡上皮细胞钠-钾-ATP酶活性下降" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "电解质" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "水" }, { "start_idx": 27, "end_idx": 36, "type": "sym", "entity": "电解质和水代谢失平衡" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "肺水肿" } ] }, { "text": "3.通气-灌流失调ARDS发生后肺泡萎陷可以导致局部肺泡区通气-灌流失调和肺内分流,严重影响气血交换,出现持续低氧血症。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "通气-灌流失调" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "ARDS" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肺泡" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "肺泡萎陷" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肺泡" }, { "start_idx": 24, "end_idx": 35, "type": "sym", "entity": "局部肺泡区通气-灌流失调" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "肺" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "肺内分流" }, { "start_idx": 44, "end_idx": 49, "type": "sym", "entity": "影响气血交换" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "低氧血症" } ] }, { "text": "正常时肺内动-静脉分流为5%~10%,ARDS时可以达到20%~30%或更高。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "ite", "entity": "肺内动-静脉分流" }, { "start_idx": 3, "end_idx": 17, "type": "sym", "entity": "肺内动-静脉分流为5%~10%" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "ARDS" } ] }, { "text": "测定肺内动静脉分流(Qs/Qt)的公式:Qs/Qt=(CcO2-CaO2)/(CcO2-CvO2)×100%。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "ite", "entity": "肺内动静脉分流" } ] }, { "text": "在不能获得混合静脉血时,可吸纯氧15~20分钟后简略估计:Qs/Qt=(700-PaO2)×5%。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "静脉血" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "吸纯氧" } ] }, { "text": "4.肺血管阻力增加和肺动脉高压持续低氧血症可以导致肺阻力血管平滑肌收缩,增加肺血管阻力,肺血流显著减少,表现为肺动脉高压,进一步加重呼吸功能障碍。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肺血管" }, { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "肺血管阻力增加" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "肺动脉" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "肺动脉高压" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "低氧血症" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肺" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "血管平滑肌" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "肺血管" }, { "start_idx": 44, "end_idx": 46, "type": "ite", "entity": "肺血流" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "肺血流显著减少" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "肺动脉" }, { "start_idx": 55, "end_idx": 59, "type": "sym", "entity": "肺动脉高压" }, { "start_idx": 64, "end_idx": 71, "type": "sym", "entity": "加重呼吸功能障碍" } ] }, { "text": "目前以多普勒彩超技术检查小儿ARDS肺外分流、肺动脉压和肺血管阻力比较安全。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "多普勒彩超" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "小儿ARDS" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肺" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "肺动脉压" }, { "start_idx": 28, "end_idx": 32, "type": "ite", "entity": "肺血管阻力" } ] }, { "text": "如果没有此条件,在机械通气时,吸入氧浓度>0.6时,经皮氧饱和度>80%,应该考虑存在肺内分流。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "机械通气" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "吸入" }, { "start_idx": 17, "end_idx": 17, "type": "dru", "entity": "氧" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "肺" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "肺内分流" } ] }, { "text": "5.肺泡损伤和肺表面活性物质功能低下肺泡上皮细胞分Ⅰ型和Ⅱ型,肺泡内表面>90%为Ⅰ型上皮细胞覆盖,7%为Ⅱ型细胞覆盖。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肺泡" }, { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 2, "end_idx": 17, "type": "sym", "entity": "肺泡损伤和肺表面活性物质功能低下" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "肺泡内表面" }, { "start_idx": 41, "end_idx": 46, "type": "bod", "entity": "Ⅰ型上皮细胞" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "Ⅱ型细胞" } ] }, { "text": "Ⅱ型细胞可以增殖并转化为Ⅰ型细胞,修复损坏的肺泡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "Ⅱ型细胞" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "Ⅰ型细胞" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肺泡" } ] }, { "text": "但Ⅱ型细胞的主要生理功能为合成和分泌肺表面活性物质。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "Ⅱ型细胞" }, { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "肺表面活性物质" } ] }, { "text": "表面活性物质由磷脂和特异蛋白组成,可降低肺泡气液界面表面张力,对于维持肺泡扩张、减少呼吸做功、促进肺液吸收具有重要作用。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "表面活性物质" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "磷脂" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "特异蛋白" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肺泡" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肺泡" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "肺液" } ] }, { "text": "直接急性肺损伤主要作用在气道和肺泡,而缺氧可以导致肺泡上皮细胞代谢障碍,高氧及过氧化导致肺泡上皮细胞变性,细菌毒素和促炎症介质抑制作用,均会影响到表面活性物质合成和代谢。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "直接急性肺损伤" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "气道" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺泡" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 25, "end_idx": 34, "type": "sym", "entity": "肺泡上皮细胞代谢障碍" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 44, "end_idx": 51, "type": "sym", "entity": "肺泡上皮细胞变性" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "细菌毒素" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "促炎症介质" }, { "start_idx": 73, "end_idx": 78, "type": "bod", "entity": "表面活性物质" } ] }, { "text": "肺泡内液可以稀释表面活性物质,改变其降低表面张力的作用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肺泡内液" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "表面活性物质" } ] }, { "text": "此外,肺泡内血浆蛋白、代谢产物、细菌毒素可抑制表面活性物质,导致继发性表面活性物质缺乏和功能低下,肺泡因此萎陷。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肺泡" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "血浆蛋白" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "代谢产物" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "细菌毒素" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "表面活性物质" }, { "start_idx": 35, "end_idx": 40, "type": "bod", "entity": "表面活性物质" }, { "start_idx": 32, "end_idx": 47, "type": "sym", "entity": "继发性表面活性物质缺乏和功能低下" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "肺泡" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "萎陷" } ] }, { "text": "6.血管内皮损伤和血管张力调节功能障碍全身和肺部感染,细菌内毒素可以导致血管扩张和感染性休克。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血管" }, { "start_idx": 2, "end_idx": 18, "type": "sym", "entity": "血管内皮损伤和血管张力调节功能障碍" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "全身和肺部感染" }, { "start_idx": 27, "end_idx": 28, "type": "mic", "entity": "细菌" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "血管" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "血管扩张" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "感染性休克" } ] }, { "text": "持续低氧血症导致大量代谢产物在肺部积聚,造成肺小血管痉挛和阻力增加,肺血流下降,通气-灌流变差,加重低氧血症。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "低氧血症" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺部" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "大量代谢产物在肺部积聚" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "肺小血管" }, { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "肺小血管痉挛和阻力增加" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肺" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "肺血流下降" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "通气-灌流变差" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "低氧血症" }, { "start_idx": 48, "end_idx": 53, "type": "sym", "entity": "加重低氧血症" } ] }, { "text": "创伤导致大量肠源性毒素、脂肪栓、血小板血栓形成,均会导致肺部血管血流障碍和血管内皮损伤,进一步导致血管通透性增加和肺水肿发生。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "创伤" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "肠源性毒素" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "脂肪栓" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "血小板血栓" }, { "start_idx": 4, "end_idx": 22, "type": "sym", "entity": "大量肠源性毒素、脂肪栓、血小板血栓形成" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "肺部血管" }, { "start_idx": 28, "end_idx": 42, "type": "sym", "entity": "肺部血管血流障碍和血管内皮损伤" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "血管" }, { "start_idx": 49, "end_idx": 55, "type": "sym", "entity": "血管通透性增加" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "肺水肿" } ] }, { "text": "近年研究认为肺毛细血管和肺泡上皮细胞等结构细胞也参与炎症免疫反应,在ARDS的次级炎症反应中具有意义。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "肺毛细血管" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "肺泡上皮细胞" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "炎症" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "ARDS" } ] }, { "text": "7.肺部损伤导致肺外脏器损伤和功能障碍肺部大量毒素、炎症介质可以不断释放经循环带到肺外脏器,致心、肝、脾、肾、脑、肠道功能障碍和损伤,最终导致多脏器功能不全。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "肺部损伤" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "肺外脏器" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "肺外脏器损伤和功能障碍" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肺部" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "毒素" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "炎症介质" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "肺外脏器" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "心" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "肝" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "脾" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "肾" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "脑" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "肠道" }, { "start_idx": 57, "end_idx": 65, "type": "sym", "entity": "肠道功能障碍和损伤" }, { "start_idx": 71, "end_idx": 77, "type": "bod", "entity": "多脏器功能不全" } ] }, { "text": "【病理】主要病理表现为弥漫性肺损害,可以在急性期和慢性期先后出现。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "弥漫性肺损害" } ] }, { "text": "根据病情进展,将病理演变可分为以下几期:急性渗出期,在ARDS最初1~4天为渗出性病变;增生期,在ARDS第3~10天;慢性纤维化期,在疾病恢复期,此病变一般在持续2周以上才出现。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "ARDS" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "ARDS" } ] }, { "text": "急性肺损伤(ALI)与急性呼吸窘迫综合征(ARDS)机制和病理上没有本质差异,只是根据改良氧合指数(PaO2/FiO2)进行的区分,ALI是ARDS的早期表现。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "急性肺损伤" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "ALI" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "急性呼吸窘迫综合征" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "ARDS" }, { "start_idx": 45, "end_idx": 48, "type": "ite", "entity": "氧合指数" }, { "start_idx": 88, "end_idx": 90, "type": "sym", "entity": "ALI" }, { "start_idx": 92, "end_idx": 95, "type": "dis", "entity": "ARDS" } ] }, { "text": "【临床表现】小儿起病特点与成人有差别,临床上可先出现肺部感染,或在基础疾病上发生进行性呼吸困难,多见于脓毒症、中毒、严重复合伤等基础疾病。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肺部" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "肺部感染" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "进行性呼吸困难" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "脓毒症" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "中毒" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "严重复合伤" } ] }, { "text": "根据下述诊断标准,可能在基础疾病临床不同时期发现ARDS。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "ARDS" } ] }, { "text": "如果判断尚不明确或属于ARDS早期的急性肺损伤,一般仍作为呼吸衰竭进行处理。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "ARDS" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "急性肺损伤" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "初期患儿在原发病基础上突然呼吸加快、气促加重,肺部多无异常发现,X线胸片显示清晰肺野,或仅有肺纹理增多模糊,为间质液体聚集,动脉血PaO2和PaCO2偏低。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "突然呼吸加快" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "气促加重" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺部" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "肺部多无异常发现" }, { "start_idx": 32, "end_idx": 35, "type": "ite", "entity": "X线胸片" }, { "start_idx": 32, "end_idx": 41, "type": "sym", "entity": "X线胸片显示清晰肺野" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "肺" }, { "start_idx": 46, "end_idx": 52, "type": "sym", "entity": "肺纹理增多模糊" }, { "start_idx": 62, "end_idx": 64, "type": "bod", "entity": "动脉血" }, { "start_idx": 65, "end_idx": 79, "type": "bod", "entity": "PaO2" }, { "start_idx": 81, "end_idx": 96, "type": "bod", "entity": "PaCO2" }, { "start_idx": 62, "end_idx": 98, "type": "sym", "entity": "动脉血PaO2和PaCO2偏低" } ] }, { "text": "动脉血PaO2和PaCO2明显降低,呼吸性碱中毒,氧疗不能改善。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "动脉血" }, { "start_idx": 3, "end_idx": 17, "type": "bod", "entity": "PaO2" }, { "start_idx": 19, "end_idx": 34, "type": "bod", "entity": "PaCO2" }, { "start_idx": 0, "end_idx": 38, "type": "sym", "entity": "动脉血PaO2和PaCO2明显降低" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "呼吸性碱中毒" }, { "start_idx": 47, "end_idx": 48, "type": "pro", "entity": "氧疗" } ] }, { "text": "(一)急性肺损伤期(ALI)可以有明显的突发致损伤因素,出现与原发于肺部的损伤相关的症状,或表现为早期ARDS的呼吸急促困难,经吸氧无法改善,也可能呼吸窘迫的症状并不明显,容易被临床医师所忽略。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "ALI" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肺部" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "ARDS" }, { "start_idx": 56, "end_idx": 61, "type": "sym", "entity": "呼吸急促困难" }, { "start_idx": 64, "end_idx": 65, "type": "pro", "entity": "吸氧" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "呼吸窘迫" } ] }, { "text": "(二)潜伏期出现在原发肺损伤后6~48小时,此阶段可以因机械通气或氧疗,使患儿心血管和呼吸功能、血气均出现暂时稳定或有所改善。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "原发肺损伤" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "机械通气" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "氧疗" }, { "start_idx": 39, "end_idx": 61, "type": "sym", "entity": "心血管和呼吸功能、血气均出现暂时稳定或有所改善" } ] }, { "text": "但X线胸片上已经可以出现两肺细颗粒影,为间质水肿的征象,并可能与临床状况并不一致。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "X线胸片" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肺" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "两肺细颗粒影" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "间质水肿" } ] }, { "text": "(三)急性呼吸衰竭期出现呼吸急促、呼吸困难、持续低氧血症、酸中毒,胸片显示双侧肺部广泛渗出,由于顺应性下降,大多数依赖高氧和高气道压力进行机械通气等。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "低氧血症" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "持续低氧血症" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "酸中毒" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "胸片" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肺部" }, { "start_idx": 37, "end_idx": 44, "type": "sym", "entity": "双侧肺部广泛渗出" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "顺应性下降" }, { "start_idx": 59, "end_idx": 60, "type": "dru", "entity": "高氧" }, { "start_idx": 62, "end_idx": 66, "type": "pro", "entity": "高气道压力" }, { "start_idx": 69, "end_idx": 72, "type": "pro", "entity": "机械通气" } ] }, { "text": "此时出现肺顺应性显著下降(<0.5ml/(cmH2O•kg)),气道阻力显著上升,并出现严重呼吸窘迫症状。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "肺" }, { "start_idx": 4, "end_idx": 11, "type": "sym", "entity": "肺顺应性显著下降" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "气道" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "气道阻力显著上升" }, { "start_idx": 55, "end_idx": 60, "type": "sym", "entity": "严重呼吸窘迫" } ] }, { "text": "(四)严重生理异常期可以表现为持续低氧血症、高碳酸血症、失代偿性酸中毒、长期呼吸机依赖、间质纤维化,对于呼吸机治疗反应差,或突然性恶化、酸中毒和代谢紊乱,最后导致死亡。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "低氧血症" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "失代偿性酸中毒" }, { "start_idx": 15, "end_idx": 34, "type": "sym", "entity": "持续低氧血症、高碳酸血症、失代偿性酸中毒" }, { "start_idx": 38, "end_idx": 40, "type": "equ", "entity": "呼吸机" }, { "start_idx": 36, "end_idx": 42, "type": "sym", "entity": "长期呼吸机依赖" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "间质纤维化" }, { "start_idx": 52, "end_idx": 54, "type": "equ", "entity": "呼吸机" }, { "start_idx": 50, "end_idx": 59, "type": "sym", "entity": "对于呼吸机治疗反应差" }, { "start_idx": 62, "end_idx": 66, "type": "sym", "entity": "突然性恶化" }, { "start_idx": 68, "end_idx": 70, "type": "dis", "entity": "酸中毒" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "代谢紊乱" }, { "start_idx": 81, "end_idx": 82, "type": "dis", "entity": "死亡" } ] }, { "text": "【实验室检查】反复检查动态血气可以判断全身氧和二氧化碳代谢、酸碱平衡情况。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "ite", "entity": "动态血气" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "氧" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "二氧化碳" } ] }, { "text": "初期(或ALI期)多表现为持续低氧和低二氧化碳血症,后期低氧加重,并二氧化碳潴留,代谢性或混合性酸中毒。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "ALI" }, { "start_idx": 15, "end_idx": 24, "type": "dis", "entity": "低氧和低二氧化碳血症" }, { "start_idx": 13, "end_idx": 24, "type": "sym", "entity": "持续低氧和低二氧化碳血症" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "氧" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "低氧加重" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "二氧化碳" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "二氧化碳潴留" }, { "start_idx": 41, "end_idx": 50, "type": "dis", "entity": "代谢性或混合性酸中毒" } ] }, { "text": "根据改良氧合指数即动脉氧分压与吸入氧浓度比值(PaO2/FiO2)<300mmHg提示急性肺损伤的存在,<200表明符合ARDS诊断(尚须结合临床、放射检查和呼吸机参数判断)。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "ite", "entity": "氧合指数" }, { "start_idx": 9, "end_idx": 21, "type": "ite", "entity": "动脉氧分压与吸入氧浓度比值" }, { "start_idx": 23, "end_idx": 53, "type": "ite", "entity": "PaO2/FiO2" }, { "start_idx": 2, "end_idx": 62, "type": "sym", "entity": "改良氧合指数即动脉氧分压与吸入氧浓度比值(PaO2/FiO2)<300mmHg" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "急性肺损伤" }, { "start_idx": 82, "end_idx": 85, "type": "dis", "entity": "ARDS" }, { "start_idx": 101, "end_idx": 103, "type": "equ", "entity": "呼吸机" } ] }, { "text": "如果是鼻导管或头罩,一般实际吸入氧浓度在40%~70%之间。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "鼻导管" }, { "start_idx": 7, "end_idx": 8, "type": "equ", "entity": "头罩" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "吸入" }, { "start_idx": 16, "end_idx": 16, "type": "dru", "entity": "氧" } ] }, { "text": "有条件时可以在鼻旁取样,测定氧浓度,作为判断肺损伤指数(PaO2/FiO2)的依据。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "鼻旁" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "氧浓度" }, { "start_idx": 22, "end_idx": 26, "type": "ite", "entity": "肺损伤指数" }, { "start_idx": 28, "end_idx": 58, "type": "ite", "entity": "PaO2/FiO2" } ] }, { "text": "在持续低氧血症不能纠正时,应该作为气道插管和机械通气治疗的指征。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "低氧血症" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "气道插管" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "机械通气治疗" } ] }, { "text": "放射学检查特点:两肺广泛渗出阴影为急性期的特征,表明肺血管向间质及肺泡渗出过程。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肺" }, { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "两肺广泛渗出阴影" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "肺血管" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "间质" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肺泡" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "肺血管向间质及肺泡渗出" } ] }, { "text": "血管通透性增加时,渗出液先积聚在大血管周围,呈肺纹理增加和微细颗粒状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血管" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "血管通透性增加" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "渗出液" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "大血管" }, { "start_idx": 9, "end_idx": 33, "type": "sym", "entity": "渗出液先积聚在大血管周围,呈肺纹理增加和微细颗粒状" } ] }, { "text": "随病情发展,可以出现大量肺泡渗出,为斑片状和实变,甚至有胸腔积液。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "肺泡" }, { "start_idx": 10, "end_idx": 23, "type": "sym", "entity": "大量肺泡渗出,为斑片状和实变" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "胸腔" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "胸腔积液" } ] }, { "text": "在疾病后期出现纤维化时,出现肺纹理增粗和小囊泡等慢性病变特征。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "肺纹理增粗" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "小囊泡" } ] }, { "text": "对仰卧位出现中背部肺的萎陷,在俯卧位时扩张,可以认为肺萎陷,而不扩张则为实变。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肺" }, { "start_idx": 1, "end_idx": 20, "type": "sym", "entity": "仰卧位出现中背部肺的萎陷,在俯卧位时扩张" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "肺萎陷" } ] }, { "text": "动态放射学检查对于掌握病情发展和治疗效果有非常重要的意义。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "动态放射学检查" } ] }, { "text": "【诊断】(一)诊断标准1994年,北美呼吸病-欧洲危重病学会专家联席评审会议(AECC)发表了ALI/ARDS诊断共识,该标准不把PEEP和肺顺应性列入。", "entities": [ { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "ALI" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "ARDS" }, { "start_idx": 65, "end_idx": 68, "type": "ite", "entity": "PEEP" }, { "start_idx": 70, "end_idx": 70, "type": "bod", "entity": "肺" } ] }, { "text": "1.急性起病有感染、创伤、吸入等诱因。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "急性起病" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "感染" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "创伤" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "吸入" } ] }, { "text": "2.低氧血症PaO2/FiO2<40kPa(300mmHg)提示急性肺损伤的存在,<27kPa(200mmHg)表明有ARDS(不考虑PEEP水平)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "低氧血症" }, { "start_idx": 6, "end_idx": 51, "type": "sym", "entity": "PaO2/FiO2<40kPa(300mmHg)" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "急性肺损伤" }, { "start_idx": 81, "end_idx": 84, "type": "dis", "entity": "ARDS" }, { "start_idx": 89, "end_idx": 92, "type": "ite", "entity": "PEEP" } ] }, { "text": "3.胸部放射检查两肺广泛渗出阴影。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "放射检查" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肺" }, { "start_idx": 2, "end_idx": 15, "type": "sym", "entity": "胸部放射检查两肺广泛渗出阴影" } ] }, { "text": "4.排除左心功能不全[肺毛细血管楔压<2.5kPa(18mmHg),或多普勒彩超检查]。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "左心功能不全" }, { "start_idx": 11, "end_idx": 32, "type": "sym", "entity": "肺毛细血管楔压<2.5kPa(18mmHg)" }, { "start_idx": 35, "end_idx": 41, "type": "pro", "entity": "多普勒彩超检查" } ] }, { "text": "(二)鉴别诊断1.心源性肺水肿有心脏病史或过量快速输液史,一般呼吸困难、肺部啰音出现伴X线心影显著增大。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "心源性肺水肿" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "心脏病" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "肺部啰音出现" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "X线" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "X线心影显著增大" } ] }, { "text": "经氧疗、控制输液量、强心、利尿等措施后,情况会迅速改善。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "氧疗" }, { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "控制输液量" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "强心" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "利尿" } ] }, { "text": "2.呼吸机相关肺炎(VAP)或呼吸机诱发肺损伤(VILI)为继发性肺炎的一种。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "呼吸机相关肺炎" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "VAP" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "呼吸机诱发肺损伤" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "VILI" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "继发性肺炎" } ] }, { "text": "可以在持续呼吸机通气治疗(>3天)时出现X线胸片弥漫渗出阴影,可以有气道分泌物细菌培养阳性。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "equ", "entity": "呼吸机" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "通气" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "X线胸片" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "X线胸片弥漫渗出阴影" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "气道分泌物" }, { "start_idx": 39, "end_idx": 40, "type": "mic", "entity": "细菌" }, { "start_idx": 34, "end_idx": 44, "type": "sym", "entity": "气道分泌物细菌培养阳性" } ] }, { "text": "如果PaO2/FiO2>300,则作为继发性肺炎,<300mmHg,则按急性肺损伤对待。", "entities": [ { "start_idx": 2, "end_idx": 36, "type": "sym", "entity": "PaO2/FiO2>300" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "继发性肺炎" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "急性肺损伤" } ] }, { "text": "气道分泌物细菌培养不作为病因诊断的唯一指标,但应该针对性应用敏感抗生素治疗。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "气道分泌物" }, { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "细菌" }, { "start_idx": 30, "end_idx": 34, "type": "dru", "entity": "敏感抗生素" } ] }, { "text": "【治疗】2006年,中华医学会重症医学分会提出了我国急性肺损伤急性呼吸窘迫综合征诊断和治疗指南。", "entities": [ { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "急性肺损伤" }, { "start_idx": 31, "end_idx": 39, "type": "dis", "entity": "急性呼吸窘迫综合征" } ] }, { "text": "ARDS治疗的关键在于:①积极控制原发病及其病因,如治疗创伤、控制感染是必要措施,降低全身炎症反应综合征;②氧疗是基本手段,及时纠正严重缺氧,PO2达到60~80mmHg,ARDS低氧纠正困难,机械通气支持是有效手段;③采用肺保护性通气策略、肺复张手法等;④防止气压伤、容量伤、生物伤,预防呼吸道继发感染和氧中毒等并发症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "ARDS" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "治疗创伤" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "控制感染" }, { "start_idx": 45, "end_idx": 51, "type": "dis", "entity": "炎症反应综合征" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "氧疗" }, { "start_idx": 64, "end_idx": 69, "type": "pro", "entity": "纠正严重缺氧" }, { "start_idx": 71, "end_idx": 84, "type": "bod", "entity": "PO2" }, { "start_idx": 71, "end_idx": 95, "type": "sym", "entity": "PO2达到60~80mmHg" }, { "start_idx": 97, "end_idx": 100, "type": "dis", "entity": "ARDS" }, { "start_idx": 101, "end_idx": 106, "type": "sym", "entity": "低氧纠正困难" }, { "start_idx": 108, "end_idx": 113, "type": "pro", "entity": "机械通气支持" }, { "start_idx": 123, "end_idx": 130, "type": "pro", "entity": "肺保护性通气策略" }, { "start_idx": 132, "end_idx": 136, "type": "pro", "entity": "肺复张手法" }, { "start_idx": 140, "end_idx": 152, "type": "pro", "entity": "防止气压伤、容量伤、生物伤" }, { "start_idx": 156, "end_idx": 162, "type": "dis", "entity": "呼吸道继发感染" }, { "start_idx": 164, "end_idx": 166, "type": "dis", "entity": "氧中毒" } ] }, { "text": "(一)积极治疗原发疾病(二)氧疗和辅助通气一般FiO2>0.6,PaO2仍<8kPa(60mmHg),SaO2<90%时,应对患者采用呼气末正压通气(PEEP)为主的综合治疗。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "氧疗" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "辅助通气" }, { "start_idx": 23, "end_idx": 37, "type": "bod", "entity": "FiO2" }, { "start_idx": 23, "end_idx": 41, "type": "sym", "entity": "FiO2>0.6" }, { "start_idx": 43, "end_idx": 57, "type": "bod", "entity": "PaO2" }, { "start_idx": 43, "end_idx": 71, "type": "sym", "entity": "PaO2仍<8kPa(60mmHg)" }, { "start_idx": 73, "end_idx": 87, "type": "bod", "entity": "SaO2" }, { "start_idx": 73, "end_idx": 91, "type": "sym", "entity": "SaO2<90%" }, { "start_idx": 100, "end_idx": 106, "type": "pro", "entity": "呼气末正压通气" }, { "start_idx": 108, "end_idx": 111, "type": "pro", "entity": "PEEP" } ] }, { "text": "这些通气模式的特点:在保持通气量时,显著降低平均气道压(MAP)。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "ite", "entity": "平均气道压" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "MAP" }, { "start_idx": 20, "end_idx": 31, "type": "sym", "entity": "降低平均气道压(MAP)" } ] }, { "text": "在ARDS早期经气道插管吸氧和辅助通气有一定好处。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "ARDS" }, { "start_idx": 8, "end_idx": 11, "type": "equ", "entity": "气道插管" }, { "start_idx": 13, "end_idx": 13, "type": "dru", "entity": "氧" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "辅助通气" } ] }, { "text": "首先,可以比较可靠地判断PaO2/FiO2比值,对病情危重程度有正确掌握;其次,便于处理由于分泌物栓塞、吸入等在气道出现的通气障碍病情恶化时,可以给予有效通气和特殊呼吸支持治疗,防止病情恶化。", "entities": [ { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "分泌物" }, { "start_idx": 68, "end_idx": 75, "type": "sym", "entity": "分泌物栓塞、吸入" }, { "start_idx": 97, "end_idx": 100, "type": "pro", "entity": "有效通气" }, { "start_idx": 102, "end_idx": 109, "type": "pro", "entity": "特殊呼吸支持治疗" } ] }, { "text": "(三)无创机械通气(NIV)可避免气管插管和气管切开引起的并发症;近年来,在成人领域得到了广泛的推广应用,但儿童使用的经验较少,年长儿可考虑使用。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "无创机械通气" }, { "start_idx": 10, "end_idx": 12, "type": "pro", "entity": "NIV" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "气管插管" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "气管切开" } ] }, { "text": "应严密监测患者的生命体征及治疗反应,意识不清、休克、气道自洁能力障碍的ALI/ARDS患者不宜应用。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "意识不清" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "休克" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "气道" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "气道自洁能力障碍" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "ALI" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "ARDS" } ] }, { "text": "(四)有创气道正压控制通气ARDS患儿应尽早采用有创机械通气支持。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "有创气道正压控制通气" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "ARDS" }, { "start_idx": 24, "end_idx": 31, "type": "pro", "entity": "有创机械通气支持" } ] }, { "text": "采用肺保护性通气策略:①小潮气量(VT,6~8ml/kg),可采用较快呼吸频率。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "肺保护性通气策略" }, { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "潮气量" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "VT" }, { "start_idx": 12, "end_idx": 28, "type": "sym", "entity": "小潮气量(VT,6~8ml/kg)" }, { "start_idx": 33, "end_idx": 38, "type": "pro", "entity": "较快呼吸频率" } ] }, { "text": "②限制气道峰压(PIP<30~35cmH2O)。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "限制气道峰压" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "PIP" }, { "start_idx": 17, "end_idx": 32, "type": "ite", "entity": "cmH2O" }, { "start_idx": 8, "end_idx": 32, "type": "sym", "entity": "PIP<30~35cmH2O" } ] }, { "text": "③选择最佳呼气末正压(PEEP,0.2~1kPa)以获得理想的顺应性,防止肺泡塌陷;有条件者可采用肺力学曲线测量低位拐点(LIP)和高位拐点(HIP),以LIP+2cmH2O为最佳PEEP;也可根据表6-6进行设置;以HIP-2cmH2O为PIP;④允许性高二氧化碳(使pH>7.2),低于该值可能需要纠酸;颅内高压是其禁忌症。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "pro", "entity": "选择最佳呼气末正压" }, { "start_idx": 11, "end_idx": 14, "type": "ite", "entity": "PEEP" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "肺泡" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "肺泡塌陷" }, { "start_idx": 162, "end_idx": 163, "type": "pro", "entity": "纠酸" }, { "start_idx": 165, "end_idx": 166, "type": "bod", "entity": "颅内" }, { "start_idx": 165, "end_idx": 168, "type": "sym", "entity": "颅内高压" } ] }, { "text": "在ARDS早期,通过调节潮气量、触发频率,获得稳定的分钟通气量和高同步化。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "ARDS" } ] }, { "text": "利用呼吸机内置装置或床旁肺流速仪监测呼吸力学,如供气和排气潮气量,气管漏气、分钟通气量,判断动态顺应性和呼吸阻力变化,从而达到缩短通气时间、降低机械通气致肺损伤的危险性。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "呼吸机" }, { "start_idx": 12, "end_idx": 15, "type": "equ", "entity": "肺流速仪" }, { "start_idx": 24, "end_idx": 31, "type": "ite", "entity": "供气和排气潮气量" }, { "start_idx": 33, "end_idx": 36, "type": "ite", "entity": "气管漏气" }, { "start_idx": 38, "end_idx": 42, "type": "ite", "entity": "分钟通气量" }, { "start_idx": 72, "end_idx": 75, "type": "pro", "entity": "机械通气" }, { "start_idx": 77, "end_idx": 79, "type": "dis", "entity": "肺损伤" } ] }, { "text": "常用的呼吸机治疗参数为:将潮气设置在6~8ml/kg(实际潮气乘以体重),通气频率视体重和病情设定,一般30~40次/分钟,PIP在20~25cmH2O,PEEP根据表6-6设定,保持PaO27~10kPa、PaCO25~7kPa、经皮氧饱和度(SpO2)>90%。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "equ", "entity": "呼吸机" }, { "start_idx": 29, "end_idx": 30, "type": "ite", "entity": "潮气" }, { "start_idx": 138, "end_idx": 143, "type": "ite", "entity": "经皮氧饱和度" } ] }, { "text": "设置PEEP兼顾氧合、顺应性和心功能诸方面。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "PEEP" } ] }, { "text": "调节PEEP与FiO2之间应以SpO2>90%作为基本判断指标,设定相对比较低的PEEP和FiO2水平,同时不应出现心率明显加快或变慢,否则要考虑PEEP水平偏高,导致回心血量减少、心输出量下降、心率代偿性变化。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "PEEP" }, { "start_idx": 62, "end_idx": 65, "type": "ite", "entity": "PEEP" }, { "start_idx": 106, "end_idx": 109, "type": "ite", "entity": "PEEP" } ] }, { "text": "呼气末二氧化碳分压(PetCO2)的动态变化可间接反映PaCO2,并可以判断无效腔量变化(VD/VT=[PetCO2-PaCO2]/PaCO2×100%),<0.3为正常,>0.5一般为严重肺泡通气障碍。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "ite", "entity": "呼气末二氧化碳分压" }, { "start_idx": 10, "end_idx": 26, "type": "ite", "entity": "PetCO2" }, { "start_idx": 67, "end_idx": 93, "type": "ite", "entity": "VD/VT" }, { "start_idx": 170, "end_idx": 177, "type": "dis", "entity": "严重肺泡通气障碍" } ] }, { "text": "有创机械通气时应注意采用半卧位(30°~45°),以预防呼吸机相关肺炎(ventilatorassociatedpneumonia,VAP)的发生。", "entities": [ { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "呼吸机相关肺炎" }, { "start_idx": 36, "end_idx": 64, "type": "dis", "entity": "ventilatorassociatedpneumonia" }, { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "VAP" } ] }, { "text": "并合理采用镇静镇痛技术,确保患儿能够耐受机械通气,并保障高恒定PEEP的实施。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "镇静镇痛技术" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "机械通气" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "耐受机械通气" }, { "start_idx": 31, "end_idx": 34, "type": "ite", "entity": "PEEP" } ] }, { "text": "表6-9最佳PEEP设置表(五)肺腹张手法小潮气量通气往往不利于ARDS塌陷肺泡的膨胀;充分复张塌陷肺泡是纠正ARDS低氧血症和保证PEEP效应的重要手段,目前临床常用的肺复张手法包括控制性肺膨胀、PEEP递增法及压力控制法。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "肺腹张手法" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "ARDS" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "ARDS" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "低氧血症" }, { "start_idx": 85, "end_idx": 89, "type": "pro", "entity": "肺复张手法" }, { "start_idx": 92, "end_idx": 97, "type": "pro", "entity": "控制性肺膨胀" }, { "start_idx": 99, "end_idx": 105, "type": "pro", "entity": "PEEP递增法" }, { "start_idx": 107, "end_idx": 111, "type": "pro", "entity": "压力控制法" } ] }, { "text": "控制性肺膨胀采用恒压通气方式,吸气压为30~45cmH2O,持续时间为30~40秒。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "控制性肺膨胀" } ] }, { "text": "注意肺复张手法可能影响循环功能。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "肺复张手法" } ] }, { "text": "(六)高频振荡通气(HFOV)目前缺乏可靠的HFOV治疗小儿ARDS临床治疗。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "高频振荡通气" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "HFOV" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "HFOV" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "ARDS" } ] }, { "text": "一般高频振荡呼吸机对10kg体重以上小儿效率显著下降。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "equ", "entity": "高频振荡呼吸机" } ] }, { "text": "对于10kg以下的婴幼儿,可以应用以改善持续低氧血症。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "低氧血症" } ] }, { "text": "高频通气优点为将闭陷小气道和肺泡持续扩张,避免肺泡承受高牵张剪切力作用,同时出现呼吸管道内气体的持续对流交换,因此达到高气体通气和换气效率并对肺组织具保护作用。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "小气道" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肺泡" }, { "start_idx": 8, "end_idx": 19, "type": "sym", "entity": "闭陷小气道和肺泡持续扩张" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺泡" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "肺组织" } ] }, { "text": "一般不会对心血管功能产生严重影响。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "心血管" } ] }, { "text": "(七)其他治疗手段1.肺表面活性物质肺泡上皮细胞损伤会影响肺泡Ⅱ型细胞合成分泌表面活性物质及Ⅰ型细胞对水、盐电解质代谢和肺液转运。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "肺泡上皮细胞损伤" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "肺泡Ⅱ型细胞" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "表面活性物质" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "Ⅰ型细胞" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "水" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "盐" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "肺液" }, { "start_idx": 27, "end_idx": 63, "type": "sym", "entity": "影响肺泡Ⅱ型细胞合成分泌表面活性物质及Ⅰ型细胞对水、盐电解质代谢和肺液转运" } ] }, { "text": "肺表面活性物质可以起到降低气液界面表面张力、减少呼吸作功、促进肺液吸收、防止血管通透增加的作用。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "肺表面活性物质" } ] }, { "text": "应用指征为:在气道峰压>2.5kPa(25cmH2O),顺应性<0.5ml/(cmH2O•kg),氧合指数(OI)>10,且持续低氧血症不得改善(>6~12小时),排除由于呼吸机参数调节不当时,可以气道内滴入肺表面活性物质50~200mg/kg,必要时可以间隔6~12小时,再给予2~3次,每次100mg/kg,以获得迅速改善氧合、降低供氧浓度、缩短呼吸机治疗时间、提高存活率等效果。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "气道峰压" }, { "start_idx": 7, "end_idx": 37, "type": "sym", "entity": "气道峰压>2.5kPa(25cmH2O)" }, { "start_idx": 39, "end_idx": 69, "type": "sym", "entity": "顺应性<0.5ml/(cmH2O•kg)" }, { "start_idx": 71, "end_idx": 74, "type": "ite", "entity": "氧合指数" }, { "start_idx": 76, "end_idx": 77, "type": "ite", "entity": "OI" }, { "start_idx": 71, "end_idx": 81, "type": "sym", "entity": "氧合指数(OI)>10" }, { "start_idx": 86, "end_idx": 89, "type": "dis", "entity": "低氧血症" }, { "start_idx": 108, "end_idx": 110, "type": "equ", "entity": "呼吸机" }, { "start_idx": 121, "end_idx": 125, "type": "pro", "entity": "气道内滴入" }, { "start_idx": 126, "end_idx": 132, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 197, "end_idx": 199, "type": "equ", "entity": "呼吸机" } ] }, { "text": "在有肺血管痉挛导致通气-灌流失调时,要考虑扩张肺血管措施。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肺血管痉挛" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "通气-灌流失调" }, { "start_idx": 21, "end_idx": 25, "type": "pro", "entity": "扩张肺血管" } ] }, { "text": "2.吸入一氧化氮(NO)吸入NO具有选择性扩张肺血管、降低肺动脉压和肺血管阻力、增加肺血流、改善通气-灌流作用。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "吸入" }, { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "一氧化氮" }, { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "NO" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "吸入" }, { "start_idx": 14, "end_idx": 15, "type": "dru", "entity": "NO" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "肺血管" }, { "start_idx": 29, "end_idx": 32, "type": "ite", "entity": "肺动脉压" }, { "start_idx": 34, "end_idx": 38, "type": "ite", "entity": "肺血管阻力" }, { "start_idx": 42, "end_idx": 44, "type": "ite", "entity": "肺血流" } ] }, { "text": "ARDS患儿如果并发肺动脉高压,可以用气道吸入NO治疗。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "ARDS" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "吸入" }, { "start_idx": 23, "end_idx": 24, "type": "dru", "entity": "NO" } ] }, { "text": "起始浓度在10~20ppm,一般在5~30分钟内可以将动脉血氧分压提高到>7kPa(52mmHg),经皮氧饱和度>85%,然后用低NO(5~10ppm)持续维持治疗,将呼吸机参数逐渐下调,经1~3天后停止吸入NO,转回常规呼吸机治疗。", "entities": [ { "start_idx": 50, "end_idx": 55, "type": "ite", "entity": "经皮氧饱和度" }, { "start_idx": 102, "end_idx": 103, "type": "pro", "entity": "吸入" }, { "start_idx": 104, "end_idx": 105, "type": "dru", "entity": "NO" } ] }, { "text": "在气道阻塞、大量肺泡内水肿渗出、肺泡严重萎陷时,吸入NO效果差,必要时可以将吸入NO与高频振荡通气、肺表面活性物质气道滴入、改变体位等手段联合应用,可以提高吸入NO治疗的效果。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "吸入" }, { "start_idx": 26, "end_idx": 27, "type": "dru", "entity": "NO" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "吸入" }, { "start_idx": 40, "end_idx": 41, "type": "dru", "entity": "NO" }, { "start_idx": 43, "end_idx": 48, "type": "pro", "entity": "高频振荡通气" }, { "start_idx": 50, "end_idx": 56, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "气道滴入" }, { "start_idx": 62, "end_idx": 65, "type": "pro", "entity": "改变体位" }, { "start_idx": 78, "end_idx": 79, "type": "pro", "entity": "吸入" }, { "start_idx": 80, "end_idx": 81, "type": "dru", "entity": "NO" } ] }, { "text": "吸入NO治疗也适宜在ARDS早期开展,效果快且持久。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "吸入" }, { "start_idx": 2, "end_idx": 3, "type": "dru", "entity": "NO" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "ARDS" } ] }, { "text": "3.糖皮质激素虽然持续的过度炎症反应和肺纤维化是导致晚期病情恶化和治疗困难的重要原因,但目前争论颇多,除了过敏、肾上腺皮质功能不全、感染性休克合并的ARDS,不宜常规使用糖皮质激素治疗,尤其是晚期ARDS。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "炎症" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "肺纤维化" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "过敏" }, { "start_idx": 56, "end_idx": 64, "type": "dis", "entity": "肾上腺皮质功能不全" }, { "start_idx": 66, "end_idx": 70, "type": "dis", "entity": "感染性休克" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "ARDS" }, { "start_idx": 85, "end_idx": 89, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 96, "end_idx": 101, "type": "dis", "entity": "晚期ARDS" } ] }, { "text": "4.俯卧位通气处于平卧位的小儿肺的背部居下方,血流慢,肺泡含气量低,加上胸廓后肋活动限制,使充气困难,出现大部分肺通气-灌流障。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "俯卧位通气" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肺" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "背部" }, { "start_idx": 23, "end_idx": 25, "type": "sym", "entity": "血流慢" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肺泡" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "肺泡含气量低" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "胸廓后肋" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "胸廓后肋活动限制" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "肺" }, { "start_idx": 56, "end_idx": 62, "type": "sym", "entity": "肺通气-灌流障" } ] }, { "text": "如果改为俯卧位,可以有利于背部肺泡扩张,改善通气-灌流,提高血氧合。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "背部肺泡" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "血氧合" } ] }, { "text": "5.营养推荐的热量中葡萄糖不宜太高,因为可导致高碳酸血症。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "高碳酸血症" } ] }, { "text": "在急性期,一般不用脂肪乳剂,因其导致血液黏稠,肺部血流速度下降,影响通气-灌流;而在恢复期,可以采用脂肪乳剂,提高能量摄入,有利于肺组织细胞修复。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "脂肪乳剂" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "血液黏稠" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肺部" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "肺部血流速度下降" }, { "start_idx": 50, "end_idx": 53, "type": "dru", "entity": "脂肪乳剂" } ] }, { "text": "适当摄入维生素A、D、E等可以强化上皮细胞抗过氧化损害、促进增生修复和功能恢复。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dru", "entity": "维生素A、D、E" } ] }, { "text": "在呼吸机治疗中,如果患儿情况稳定,应考虑采用经胃肠道营养支持,加速肺和机体复员。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "equ", "entity": "呼吸机" }, { "start_idx": 23, "end_idx": 29, "type": "pro", "entity": "胃肠道营养支持" } ] }, { "text": "6.补液与输血应严格控制ARDS病例的液体摄入量。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "补液" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "输血" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "ARDS" } ] }, { "text": "补液量保持在常规需要量的70%为宜,血容量和输液的平衡点为能够保持基础营养液量和有效心输出量。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "补液" }, { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "血容量" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "心" } ] }, { "text": "应用胶体液不一定有利于肺液吸收,尤其在血管通透性高时,大量蛋白会漏出到肺间质和肺泡内,吸收水分并妨碍液体清除。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肺液" }, { "start_idx": 19, "end_idx": 23, "type": "ite", "entity": "血管通透性" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "蛋白" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "肺间质" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肺泡" } ] }, { "text": "根据病情可以通过输入红细胞,提高血细胞比容达到32%~34%,或提供25%或5%清蛋白。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "红细胞" }, { "start_idx": 16, "end_idx": 20, "type": "ite", "entity": "血细胞比容" }, { "start_idx": 40, "end_idx": 42, "type": "dru", "entity": "清蛋白" } ] }, { "text": "7.利尿适当应用呋塞米(速尿)类药物利尿,可有利于肺水肿的改善,促进肺液吸收,减轻心脏后负荷。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "利尿" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "呋塞米" }, { "start_idx": 12, "end_idx": 13, "type": "dru", "entity": "速尿" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "肺水肿" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "肺液" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "心脏" } ] }, { "text": "8.连续血液净化技术近年来,连续血液净化技术(包括连续静脉滤过、透析滤过、持续超滤等)在ARDS治疗中的应用逐步得到肯定。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "连续血液净化技术" }, { "start_idx": 14, "end_idx": 21, "type": "pro", "entity": "连续血液净化技术" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "连续静脉滤过" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "透析滤过" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "持续超滤" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "ARDS" } ] }, { "text": "既有利于液体平衡的控制和药物的使用,更有利于降低致炎-抑炎的炎症反应强度,改善全身和肺局部的凝血机制。", "entities": [ { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "肺" } ] }, { "text": "对于肾功能障碍者可以联合应用血液透析或腹膜透析技术,有利于排出代谢废物和细菌毒素,促进肺液吸收。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "血液透析" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "腹膜透析技术" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "代谢废物" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "细菌毒素" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "肺液" } ] }, { "text": "9.液体通气技术(liquidventilation,LV)和体外膜肺技术(extracorporealmembraneoxygenation,ECMO)采用全氟化碳灌注肺部的液体通气技术在动物实验得到良好证实,临床应用也收到明显疗效,但儿童开展经验不多;ECMO技术在严重ARDS病例的使用已经得到证实和逐步推广,可采用VV模式,如合并循环功能不全,采用VA模式。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "液体通气技术" }, { "start_idx": 9, "end_idx": 25, "type": "pro", "entity": "liquidventilation" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "LV" }, { "start_idx": 38, "end_idx": 70, "type": "pro", "entity": "extracorporealmembraneoxygenation" }, { "start_idx": 72, "end_idx": 75, "type": "pro", "entity": "ECMO" }, { "start_idx": 79, "end_idx": 82, "type": "dru", "entity": "全氟化碳" }, { "start_idx": 83, "end_idx": 84, "type": "pro", "entity": "灌注" }, { "start_idx": 88, "end_idx": 93, "type": "pro", "entity": "液体通气技术" }, { "start_idx": 128, "end_idx": 133, "type": "pro", "entity": "ECMO技术" }, { "start_idx": 137, "end_idx": 140, "type": "dis", "entity": "ARDS" }, { "start_idx": 167, "end_idx": 174, "type": "dis", "entity": "合并循环功能不全" } ] }, { "text": "【预后】小儿ARDS如果在感染性重症肺炎合并呼吸衰竭时,治愈的把握较大。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "小儿ARDS" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "感染性重症肺炎" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "但是,经化疗后的白血病患儿、肿瘤恶病质、小婴儿肺炎伴严重营养不良和免疫力低下、存在全身性结缔组织病、组织细胞增生症等,往往在肺部感染后出现ARDS,并难以用单纯呼吸机治疗和抗感染克服。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "白血病" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肿瘤恶病质" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "小婴儿肺炎" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "严重营养不良" }, { "start_idx": 41, "end_idx": 48, "type": "dis", "entity": "全身性结缔组织病" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "组织细胞增生症" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "肺部感染" }, { "start_idx": 69, "end_idx": 72, "type": "dis", "entity": "ARDS" }, { "start_idx": 80, "end_idx": 82, "type": "equ", "entity": "呼吸机" }, { "start_idx": 86, "end_idx": 88, "type": "pro", "entity": "抗感染" } ] }, { "text": "且这些患儿可以因化疗导致骨髓造血干细胞抑制,临床上以外周白细胞明显减少为特点,不同于败血症时的肺部白细胞集聚而引起的外周白细胞减少症。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "化疗" }, { "start_idx": 12, "end_idx": 18, "type": "bod", "entity": "骨髓造血干细胞" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "骨髓造血干细胞抑制" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "白细胞" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "外周白细胞明显减少" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "败血症" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "肺部" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "白细胞" }, { "start_idx": 47, "end_idx": 53, "type": "sym", "entity": "肺部白细胞集聚" }, { "start_idx": 58, "end_idx": 65, "type": "dis", "entity": "外周白细胞减少症" } ] }, { "text": "因此,对于此类患儿重点为早期预防和治疗,并针对肺血管内微血栓形成等机制进行治疗,成为目前救治小儿ARDS的难题,有待攻克。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "肺血管" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "微血栓" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "小儿ARDS" } ] }, { "text": "参考文献1.SwartJF,WulffraatNM.Diagnosticworkupformixedconnectivetissuediseaseinchildhood.IsrMedAssoc,2008,10(8-8):650-6522.RichardJ.Mier,MichaelShishov,GloriaC,etal.Pediatriconsetmixedconnectivetissuedisease.RheumDisClinNAm,2005,31:483-4963.蒋明等.风湿病学.北京:科学出版社,19984.BehrmanRE,KliegmanRM,JensonHB.NelsonTextBookofPediatrics.16thed.Philadelphia:W.B.SaunersCo,20005.杨锡强等.儿童免疫学.北京:人民卫生出版社,2001", "entities": [ { "start_idx": 241, "end_idx": 243, "type": "dis", "entity": "风湿病" } ] }, { "text": "第九章抽动障碍【概述】(一)抽动与抽动障碍的定义1.抽动抽动(tic)一词是从法语Tique演变而来,原意为扁虱,用扁虱去叮咬牛马时出现的急促的皮肌收缩,用于表达原发性三叉神经痛时所伴随的面肌痉挛。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "抽动" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "抽动" }, { "start_idx": 69, "end_idx": 75, "type": "sym", "entity": "急促的皮肌收缩" }, { "start_idx": 84, "end_idx": 87, "type": "bod", "entity": "三叉神经" }, { "start_idx": 94, "end_idx": 97, "type": "sym", "entity": "面肌痉挛" } ] }, { "text": "抽动是一个形象的概念,并不是一个疾病的名称,有许多神经和精神病学者如Meige和Feindel等(1903),很早就开始研究“抽动”,但迄今仍很难相当完善、肯定和清楚地阐明“抽动”这一现象的本质。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "抽动" } ] }, { "text": "Lapouse等(1964)报道纽约的Buffalo区内,6~12岁的儿童有12%患过抽动。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "报道" } ] }, { "text": "抽动被认为是固定或游走性的身体任何部位肌群出现不自主、无目的、重复和快速的收缩动作。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "抽动" }, { "start_idx": 13, "end_idx": 40, "type": "sym", "entity": "身体任何部位肌群出现不自主、无目的、重复和快速的收缩动作" } ] }, { "text": "抽动可以发生于身体某一部位的某一组肌肉,也可同时或先后出现在多个部位的多组肌肉;可以是连续性的天天出现,也可间断性发作。", "entities": [ { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "连续性的天天出现" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "间断性发作" } ] }, { "text": "每一次抽动动作均急速完成,可重复出现,其表现十分类似。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "抽动动作均急速完成" } ] }, { "text": "抽动有时可带有阵挛性,但无强直。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "阵挛性" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "无强直" } ] }, { "text": "其累及范围和频率因人而异,可以有急速挤眉、瞬目、撅嘴、转颈及耸肩等,也可以有躯干的急速抖动或扭转,喉部的抽动出现如清喉时发出“哼”音、其他怪声或秽语。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "急速挤眉" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "瞬目" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "撅嘴" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "转颈" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "耸肩" }, { "start_idx": 38, "end_idx": 47, "type": "sym", "entity": "躯干的急速抖动或扭转" }, { "start_idx": 57, "end_idx": 65, "type": "sym", "entity": "清喉时发出“哼”音" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "怪声" } ] }, { "text": "抽动具有多变性的特点,即抽动并不固定在一个部位,如运动性抽动的分布通常起始于头面部肌肉,可出现眨眼、摇头及扮鬼脸等动作;随着病情进展,抽动逐渐累及身体各部位,分布的模式不同,也无一定规律性。", "entities": [ { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "头" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "面部" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "眨眼" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "摇头" }, { "start_idx": 53, "end_idx": 55, "type": "sym", "entity": "扮鬼脸" } ] }, { "text": "当情绪紧张、焦虑时,抽动频率增加、强度增大;而当注意力集中、意志控制时,抽动可减少或短时间消失。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "情绪紧张" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "焦虑" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "抽动频率增加" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "强度增大" } ] }, { "text": "2.抽动障碍抽动障碍(ticdisorders,TD)首先由Itard(1825)和GillesdelaTourette(1885)描述,20世纪60年代以前一直视为原因不明、罕见的可自愈性疾病。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 11, "end_idx": 22, "type": "dis", "entity": "ticdisorders" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "TD" } ] }, { "text": "然而,近40多年来,抽动障碍的认识发生了戏剧性变化,特别是20世纪90年代以来,普遍认为抽动障碍非常多见,是一种于儿童和青少年时期起病、由遗传缺陷和不良环境因素所致的神经精神发育障碍,具有复杂异质性。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "遗传缺陷" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "不良环境" }, { "start_idx": 83, "end_idx": 90, "type": "dis", "entity": "神经精神发育障碍" } ] }, { "text": "不仅表现有抽动,还有较多的行为和精神症状。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "较多的行为" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "精神症状" } ] }, { "text": "抽动症状可以时轻时重,呈波浪式进展,间或静止一段时间;新的抽动症状可以代替旧的抽动症状,或在原有抽动症状的基础上出现新的抽动症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "抽动症状" } ] }, { "text": "所有形式的抽动都可因应激、焦虑、疲劳、兴奋以及感冒发热而加重,都可因放松、全身心投入某事而减轻,睡眠时可消失。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "应激" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "焦虑" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "疲劳" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "兴奋" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "感冒发热" } ] }, { "text": "抽动障碍伴发的注意缺陷多动障碍(ADHD)、强迫障碍(OCD)、睡眠障碍(SD)、情绪障碍(ED)以及其他心理行为问题,增加了病情的严重性和复杂性,也给治疗带来一定的难度。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "注意缺陷多动障碍" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "ADHD" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "强迫障碍" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "OCD" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "睡眠障碍" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "SD" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "情绪障碍" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "ED" } ] }, { "text": "3.更新观念我国医学界对此病认识仍很混乱,把这些儿童的表现当成“坏毛病”、“沙眼”、“结膜炎”,以及“咽炎”等现象极为普遍;即使能识别者也常因持有“可自愈性”的观点而延误治疗。", "entities": [ { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "结膜炎" } ] }, { "text": "(1)抽动障碍并不少见:抽动障碍实际上是儿童青少年中较为常见的一种心理行为障碍,包括短暂性抽动障碍、慢性抽动障碍和Tourette综合征三种类型。", "entities": [ { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 57, "end_idx": 67, "type": "dis", "entity": "Tourette综合征" } ] }, { "text": "抽动障碍男孩更多见。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "据北京安定医院儿科门诊统计,1997年共有抽动障碍患儿556人次就诊,其中初诊101人,占全年儿科门诊总数的25.5%,较10年前的8.4%增加3倍,已成为儿童精神科和儿科的一种较常见病。", "entities": [ { "start_idx": 78, "end_idx": 82, "type": "dep", "entity": "儿童精神科" }, { "start_idx": 84, "end_idx": 85, "type": "dep", "entity": "儿科" } ] }, { "text": "(2)自愈倾向较低:多年来,许多医生认为抽动障碍是一种可自愈性疾病,不必采用特殊治疗。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "一项由22个国家参加的3500名TS病人的综合研究中,在接受治疗的情况下,19.4%病人16岁以后仍然症状明显,没有自愈性的报道。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "TS" } ] }, { "text": "许多人将暂时性抽动障碍(病程不超过一年)与慢性抽动障碍和TS混为一谈,所以得出错误结论。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "暂时性抽动障碍" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "TS" } ] }, { "text": "(3)精神症状突出:抽动障碍症状从轻到重,复杂多变,不只表现为抽动,而有多种情绪和行为问题。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "精神症状突出" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "抽动" }, { "start_idx": 35, "end_idx": 44, "type": "sym", "entity": "有多种情绪和行为问题" } ] }, { "text": "常导致自身心理困扰并影响他人的日常生活和学习。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "自身心理困扰" } ] }, { "text": "据调查,慢性抽动障碍和TS中,60%合并多动症、59%合并强迫症、15%有品行障碍、23%学习困难、20%心境障碍、18%伴有焦虑、14%有自伤、20%社会技能有问题、25%有睡眠问题,以及37%脾气控制困难。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "TS" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "多动症" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "品行障碍" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "学习困难" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "心境障碍" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "焦虑" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "自伤" }, { "start_idx": 76, "end_idx": 82, "type": "sym", "entity": "社会技能有问题" }, { "start_idx": 88, "end_idx": 91, "type": "sym", "entity": "睡眠问题" }, { "start_idx": 98, "end_idx": 103, "type": "sym", "entity": "脾气控制困难" } ] }, { "text": "因此,抽动障碍患者虽然多去小儿内科或神经科,但无论是国际疾病分类,还是中国疾病分类均将抽动障碍划分在精神科。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 13, "end_idx": 16, "type": "dep", "entity": "小儿内科" }, { "start_idx": 18, "end_idx": 20, "type": "dep", "entity": "神经科" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 50, "end_idx": 52, "type": "dep", "entity": "精神科" } ] }, { "text": "(4)难治性病例增多:难治性抽动障碍是近些年来精神科临床逐渐形成的新概念,用于描述经过常规药物(硫必利、氟哌啶醇及安定类药物)治疗效果不好,病程迁延不愈的抽动障碍病人。", "entities": [ { "start_idx": 23, "end_idx": 25, "type": "dep", "entity": "精神科" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "硫必利" }, { "start_idx": 52, "end_idx": 55, "type": "dru", "entity": "氟哌啶醇" }, { "start_idx": 57, "end_idx": 61, "type": "dru", "entity": "安定类药物" } ] }, { "text": "在2000年,安定医院门诊抽动障碍病例中,难治性占68%,多是到处诊治无效而转诊者,其特点是症状严重和多肌群抽动,合并精神症状者多(其中多动占64%、行为问题占54%、脾气控制问题占52%、强迫占31%、睡眠障碍22%,以及情绪障碍占20%)。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 51, "end_idx": 55, "type": "sym", "entity": "多肌群抽动" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "多动" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "行为问题" }, { "start_idx": 84, "end_idx": 89, "type": "sym", "entity": "脾气控制问题" }, { "start_idx": 95, "end_idx": 96, "type": "sym", "entity": "强迫" }, { "start_idx": 102, "end_idx": 105, "type": "sym", "entity": "睡眠障碍" }, { "start_idx": 112, "end_idx": 115, "type": "sym", "entity": "情绪障碍" } ] }, { "text": "所以,抽动障碍应早期诊断,早期治疗。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "(二)抽动的分类抽动通常被分为运动抽动(motortics)和发声抽动(vocaltics)。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "运动抽动" }, { "start_idx": 20, "end_idx": 28, "type": "dis", "entity": "motortics" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "发声抽动" }, { "start_idx": 36, "end_idx": 44, "type": "dis", "entity": "vocaltics" } ] }, { "text": "运动抽动是指头、面部、颈肩、躯干及四肢肌肉的不自主、突发、快速收缩运动。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "运动抽动" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "头" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "面部" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "颈肩" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "躯干" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "四肢肌肉" }, { "start_idx": 6, "end_idx": 34, "type": "sym", "entity": "头、面部、颈肩、躯干及四肢肌肉的不自主、突发、快速收缩运动" } ] }, { "text": "发声抽动实际上是累及呼吸肌、咽肌、喉肌、口腔肌和鼻肌的抽动。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "发声抽动" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "咽肌" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "喉肌" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "口腔肌" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "鼻肌" }, { "start_idx": 8, "end_idx": 28, "type": "sym", "entity": "累及呼吸肌、咽肌、喉肌、口腔肌和鼻肌的抽动" } ] }, { "text": "鼻、口腔和咽喉的气流产生发声。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "鼻" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "口腔" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "咽喉" } ] }, { "text": "运动抽动或发声抽动均可分为简单和复杂两类,但有时简单与复杂抽动之间的界限是不易分清的。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "运动抽动" }, { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "发声抽动" } ] }, { "text": "简单运动抽动是指突然、迅速、孤立和无意义的运动,如眨眼、摇头、点头、皱额、鼻子抽动、撅嘴、张口、歪颈、耸肩、腹肌抽动、臂动、手动、腿动或脚动等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "简单运动抽动" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "眨眼" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "摇头" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "点头" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "皱额" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "鼻子抽动" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "撅嘴" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "张口" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "歪颈" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "耸肩" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "腹肌抽动" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "手动" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "腿动" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "脚动" } ] }, { "text": "复杂运动抽动是指突然、似有目的性、协调和复杂的运动,如“做鬼脸”、拍手、挥舞上臂、弯腰、扭动躯干、眼球转动、嗅、下蹲、跺脚、蹦、跳、扔、敲打、打自己、修饰发鬓、走路转圈或突然停下来、重复触摸物品或身体某一部位等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "复杂运动抽动" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "做鬼脸" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "拍手" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "挥舞上臂" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "弯腰" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "扭动躯干" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "眼球转动" }, { "start_idx": 54, "end_idx": 54, "type": "sym", "entity": "嗅" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "下蹲" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "跺脚" }, { "start_idx": 62, "end_idx": 62, "type": "sym", "entity": "蹦" }, { "start_idx": 64, "end_idx": 64, "type": "sym", "entity": "跳" }, { "start_idx": 66, "end_idx": 66, "type": "sym", "entity": "扔" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "敲打" }, { "start_idx": 71, "end_idx": 73, "type": "sym", "entity": "打自己" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "修饰发鬓" }, { "start_idx": 80, "end_idx": 83, "type": "sym", "entity": "走路转圈" }, { "start_idx": 85, "end_idx": 89, "type": "sym", "entity": "突然停下来" }, { "start_idx": 91, "end_idx": 103, "type": "sym", "entity": "重复触摸物品或身体某一部位" } ] }, { "text": "复杂发声抽动是由有意义的单词、词组或句子组成,表现为与环境不相适宜的不自主发出音节、单字、词组、短语、短句、唠叨、秽语(coprolalia)、重复言语(palilalia)和模仿言语(echolalia)等。", "entities": [ { "start_idx": 34, "end_idx": 91, "type": "sym", "entity": "不自主发出音节、单字、词组、短语、短句、唠叨、秽语(coprolalia)、重复言语(palilalia)和模仿言语" } ] }, { "text": "(三)抽动障碍的分类1.按临床特征和病程分类抽动障碍是起病于儿童或青少年时期,以不自主的、反复的、快速的一个或多个部位运动抽动和或发声抽动为主要特征的一组复杂的、慢性神经精神综合征。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 40, "end_idx": 62, "type": "sym", "entity": "不自主的、反复的、快速的一个或多个部位运动抽动" } ] }, { "text": "根据抽动障碍临床症状和病程长短的不同,分为短暂性抽动障碍(transientticdisorders,TTD)、慢性抽动障碍(chronicticdisorders,CTD)和Tourette综合征(Tourettesyndrome,TS)三种类型。", "entities": [ { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 29, "end_idx": 49, "type": "dis", "entity": "transientticdisorders" }, { "start_idx": 56, "end_idx": 61, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 63, "end_idx": 81, "type": "dis", "entity": "chronicticdisorders" }, { "start_idx": 83, "end_idx": 85, "type": "dis", "entity": "CTD" }, { "start_idx": 88, "end_idx": 98, "type": "dis", "entity": "Tourette综合征" }, { "start_idx": 100, "end_idx": 115, "type": "dis", "entity": "Tourettesyndrome" }, { "start_idx": 117, "end_idx": 118, "type": "dis", "entity": "TS" } ] }, { "text": "短暂性抽动障碍可以向慢性抽动障碍转化,而慢性抽动障碍也可以向TS转化。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "TS" } ] }, { "text": "至于不能归于上述三种类型的抽动障碍,被认为是属于其他尚未界定的抽动障碍,如成年期发病的抽动障碍(迟发性抽动障碍)。", "entities": [ { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "将抽动障碍分为短暂性抽动障碍、慢性抽动障碍和TS三种类型,这种分类已被国内外大多数学者所公认。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "TS" } ] }, { "text": "短暂性抽动障碍,又称为暂时性抽动障碍、一过性抽动障碍或习惯性痉挛,是抽动障碍中最多见的一种类型,也是最轻的一型;是指表现有一种或多种运动抽动和(或)发声抽动,可以仅有运动抽动或发声抽动,也可以二者相继出现,病程在一年之内。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "暂时性抽动障碍" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "一过性抽动障碍" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "习惯性痉挛" }, { "start_idx": 66, "end_idx": 69, "type": "dis", "entity": "运动抽动" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "发声抽动" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "运动抽动" }, { "start_idx": 88, "end_idx": 91, "type": "dis", "entity": "发声抽动" } ] }, { "text": "在短暂性抽动障碍中,抽动可能仅局限于某一部位肌群收缩,即仅累及一组肌肉的简单抽动,表现为一种短暂、孤立的急跳(jerk)状运动,或一种简单、无意义、不连续的(unarticulated)发声,诸如眨眼、摇头、嗅鼻及清喉声等,这种反复地单一抽动只是在他人看起来有点别扭,自己觉得有点不自然,往往把这种仅局限于某一组肌肉的简单抽动称为习惯性抽动。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "肌肉的简单抽动" }, { "start_idx": 46, "end_idx": 62, "type": "sym", "entity": "短暂、孤立的急跳(jerk)状运动" }, { "start_idx": 65, "end_idx": 94, "type": "sym", "entity": "一种简单、无意义、不连续的(unarticulated)发声" }, { "start_idx": 98, "end_idx": 99, "type": "sym", "entity": "眨眼" }, { "start_idx": 101, "end_idx": 102, "type": "sym", "entity": "摇头" }, { "start_idx": 104, "end_idx": 105, "type": "sym", "entity": "嗅鼻" }, { "start_idx": 107, "end_idx": 109, "type": "sym", "entity": "清喉声" } ] }, { "text": "慢性抽动障碍,又称为慢性运动或发声抽动障碍,是指仅表现有运动抽动或发声抽动,二者不兼有,病程在一年以上;抽动形式可以是简单抽动或复杂抽动,抽动部位可以是单一的也可以是多种的。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "发声抽动障碍" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "运动抽动" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "发声抽动" } ] }, { "text": "TS又称为GillesdelaTourette综合征、图雷特病(Tourettedisorders)、多发性抽动症、发声与多种运动联合抽动障碍以及妥瑞症等;至于过去国内常称谓的抽动秽语综合征这一病名欠妥,源于秽语的发生率还不足13,秽语症状并非诊断本病所必须具备的条件,而且秽语本身带有很大贬义,建议弃用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "TS" }, { "start_idx": 5, "end_idx": 25, "type": "dis", "entity": "GillesdelaTourette综合征" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "图雷特病" }, { "start_idx": 32, "end_idx": 48, "type": "dis", "entity": "Tourettedisorders" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "多发性抽动症" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "妥瑞症" } ] }, { "text": "TS是抽动障碍中病情相对较重的一型,可由慢性抽动障碍转变而来,指既表现有运动抽动,也兼有发声抽动,但运动抽动和发声抽动不一定同时出现,病程在一年以上。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "TS" }, { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "发声抽动" } ] }, { "text": "这三种类型抽动障碍的区别主要在症状的构成和持续时间。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "症状构成的不同在于:短暂性抽动障碍可以仅有运动抽动或发声抽动,也可以二者兼有;慢性抽动障碍则要求只有一种或多种运动抽动,或只有一种或多种发声抽动,二者不兼有;TS必须有一种或多种运动抽动,兼有一种或多种发声抽动,但二者不一定同时出现。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "运动抽动" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "发声抽动" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 79, "end_idx": 80, "type": "dis", "entity": "TS" }, { "start_idx": 101, "end_idx": 104, "type": "dis", "entity": "发声抽动" } ] }, { "text": "持续时间的不同在于:短暂性抽动障碍的病程在一年之内,而慢性抽动障碍和TS的病程在一年以上。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "TS" } ] }, { "text": "孙圣刚等提出,从临床实践的角度上来看,上述三种类型抽动障碍之间有时是不易分清的,这种人为的分类不是很切合实际,例如在时间概念上,短暂性抽动障碍的抽动要求至少持续2周以上,但连续期不超过1年,而慢性抽动障碍和TS则规定病程超过1年,但在临床实践中这种病人往往在发病2~3个月内被家属觉察异常而送到医院诊治,极少超过1年者,并且药物治疗后疗效显著,势必给分型带来困难。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 64, "end_idx": 70, "type": "dis", "entity": "短暂性抽动障碍" }, { "start_idx": 96, "end_idx": 101, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 103, "end_idx": 104, "type": "dis", "entity": "TS" } ] }, { "text": "他们建议将抽动障碍分为以下两型:(1)简单抽动症(或习惯性痉挛):诊断标准为①以少年儿童最常见,但可持续到成年;②有不自主的、快速、重复无目的单一运动,以反复眨眼、努嘴或头颈部其他肌肉抽动最常见;③抽动能受意志短暂克制,入睡后消失,神经系统性体征。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "简单抽动症" }, { "start_idx": 58, "end_idx": 74, "type": "sym", "entity": "不自主的、快速、重复无目的单一运动" }, { "start_idx": 85, "end_idx": 93, "type": "sym", "entity": "头颈部其他肌肉抽动" }, { "start_idx": 116, "end_idx": 118, "type": "ite", "entity": "神经系" }, { "start_idx": 116, "end_idx": 118, "type": "bod", "entity": "神经系" } ] }, { "text": "(2)TS:诊断标准根据Shapiro(日本)的标准加以修改。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "TS" } ] }, { "text": "1)诊断必需条件为①起病在21岁以前;②有2组或2组以上的肌肉抽动和发声抽动;③症状呈慢性经过,至少持续2个月以上,但可有波动,可由新症状替代旧症状,或在原有症状上增加新症状;④需排除风湿性舞蹈症、肝豆状核变性、癫痫肌阵挛性发作及其他锥体外系疾病。", "entities": [ { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "发声抽动" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "慢性经过" }, { "start_idx": 50, "end_idx": 56, "type": "sym", "entity": "持续2个月以上" }, { "start_idx": 92, "end_idx": 97, "type": "dis", "entity": "风湿性舞蹈症" }, { "start_idx": 99, "end_idx": 104, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 106, "end_idx": 111, "type": "dis", "entity": "癫痫肌阵挛性" }, { "start_idx": 117, "end_idx": 122, "type": "dis", "entity": "锥体外系疾病" } ] }, { "text": "2)有助诊断的条件为①伴有秽语或模仿语言等复杂性发声抽动;②伴有强迫动作及猥亵等行为问题;③对抗多巴胺活动过度的药物有明显疗效。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "秽语" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "强迫动作" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "猥亵" } ] }, { "text": "2.按生理性和病理性分类(1)生理性抽动障碍:如矫揉造作。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "生理性抽动障碍" } ] }, { "text": "(2)病理性抽动障碍1)原发性:散发性包括①短暂性运动抽动或发声抽动(病程少于1年);②慢性运动抽动或发声抽动(病程超过1年);③成年期起病(晚发的)抽动障碍;④TS。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "病理性抽动障碍" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "短暂性运动抽动" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "发声抽动" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "慢性运动抽动" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "发声抽动" }, { "start_idx": 65, "end_idx": 78, "type": "dis", "entity": "成年期起病(晚发的)抽动障碍" }, { "start_idx": 81, "end_idx": 82, "type": "dis", "entity": "TS" } ] }, { "text": "遗传性包括①TS;②亨廷顿舞蹈病;③原发性肌张力不全;④神经棘红细胞病。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "TS" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "亨廷顿舞蹈病" }, { "start_idx": 18, "end_idx": 25, "type": "sym", "entity": "原发性肌张力不全" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "神经棘红细胞病" } ] }, { "text": "2)继发性:抽动障碍可继发于以下各种原因。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "A.感染性:如脑炎、风湿性舞蹈病、神经梅毒及克-雅病。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "脑炎" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "神经梅毒及克-雅病" } ] }, { "text": "B.药物性:某些药物可诱发或加重抽动,如中枢兴奋剂(哌甲酯、匹莫林、安非他明和可卡因)、抗精神病药、抗抑郁药、抗组胺药、抗胆碱药、阿片制剂、抗癫痫药(卡马西平、苯巴比妥和苯妥英钠)以及左旋多巴等。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "中枢兴奋剂" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "哌甲酯" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "匹莫林" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "安非他明" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "可卡因" }, { "start_idx": 44, "end_idx": 48, "type": "dru", "entity": "抗精神病药" }, { "start_idx": 50, "end_idx": 53, "type": "dru", "entity": "抗抑郁药" }, { "start_idx": 60, "end_idx": 63, "type": "dru", "entity": "抗胆碱药" }, { "start_idx": 65, "end_idx": 68, "type": "dru", "entity": "阿片制剂" }, { "start_idx": 70, "end_idx": 73, "type": "dru", "entity": "抗癫痫药" }, { "start_idx": 75, "end_idx": 78, "type": "dru", "entity": "卡马西平" }, { "start_idx": 80, "end_idx": 83, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 92, "end_idx": 95, "type": "dru", "entity": "左旋多巴" } ] }, { "text": "D.发育性:如见于染色体异常、先天性代谢缺陷、精神发育迟滞以及Asperger综合征。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "染色体异常" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "先天性代谢缺陷" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "精神发育迟滞" }, { "start_idx": 31, "end_idx": 41, "type": "dis", "entity": "Asperger综合征" } ] }, { "text": "E.其他:见于脑卒中、精神分裂症、神经皮肤综合征以及颅脑外伤等。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "脑卒中" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "精神分裂症" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "神经皮肤综合征" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "颅脑外伤" } ] }, { "text": "3.按病因分类(1)原发性抽动障碍:包括小儿急性短暂性抽动,慢性抽动障碍,TS,成人起病的抽动障碍,老年抽动障碍等。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "原发性抽动障碍" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "慢性抽动障碍" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "TS" }, { "start_idx": 40, "end_idx": 48, "type": "dis", "entity": "成人起病的抽动障碍" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "老年抽动障碍" } ] }, { "text": "(2)继发性抽动障碍:是与各种神经疾病相关联,分为以下几种类型。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "继发性抽动障碍" } ] }, { "text": "1)遗传性:包括染色体异常(如唐氏综合征,脆性X综合征及其他染色体病),亨廷顿舞蹈病,肌张力障碍(如Meige综合征),Hyperekplexias等。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "唐氏综合征" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "脆性X综合征" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "其他染色体病" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "亨廷顿舞蹈病" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "肌张力障碍" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "Meige综合征" }, { "start_idx": 60, "end_idx": 73, "type": "dis", "entity": "Hyperekplexias" } ] }, { "text": "2)发育性:包括Rett综合征,静止性(static)脑病(如缺氧等),全面发育延迟等。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "Rett综合征" }, { "start_idx": 16, "end_idx": 28, "type": "dis", "entity": "静止性(static)脑病" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "全面发育延迟" } ] }, { "text": "3)变性性:包括神经棘红细胞病,进行性核上性麻痹等。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "神经棘红细胞病" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "进行性核上性麻痹" } ] }, { "text": "4)精神性:包括精神分裂症,OCD等。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "精神分裂症" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "OCD" } ] }, { "text": "5)中毒-代谢性:包括一氧化碳中毒,低血糖等。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "一氧化碳中毒" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "低血糖" } ] }, { "text": "6)药物性:包括精神抑制药(致迟发性抽动),兴奋剂,抗惊厥药,左旋多巴等。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "精神抑制药" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "兴奋剂" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "左旋多巴" } ] }, { "text": "7)感染性:包括风湿性舞蹈病,脑炎,脑炎后帕金森综合征,克-雅病,Rubella综合征等。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "风湿性舞蹈病" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "脑炎" }, { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "脑炎后帕金森综合征" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "克-雅病" }, { "start_idx": 33, "end_idx": 42, "type": "dis", "entity": "Rubella综合征" } ] }, { "text": "8)习惯性身体动作(habitualbodymanipulations):包括吸吮手指,咬指甲,擦眼睛,触摸耳朵,挖鼻孔,触摸外生殖器等。", "entities": [ { "start_idx": 44, "end_idx": 46, "type": "sym", "entity": "咬指甲" }, { "start_idx": 48, "end_idx": 50, "type": "sym", "entity": "擦眼睛" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "触摸耳朵" }, { "start_idx": 57, "end_idx": 59, "type": "sym", "entity": "挖鼻孔" }, { "start_idx": 61, "end_idx": 66, "type": "sym", "entity": "触摸外生殖器" } ] }, { "text": "9)刻板动作:包括点头或击头,摇动身体,手臂抽动(armjerking)等。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "点头" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "击头" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "摇动身体" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "手臂抽动" } ] }, { "text": "(1)原发性抽动障碍:包括TS、慢性复杂运动抽动障碍、慢性复杂发声抽动障碍、慢性单纯运动抽动障碍、慢性单纯发声抽动障碍以及短暂性抽动障碍。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "原发性抽动障碍" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "TS" }, { "start_idx": 16, "end_idx": 25, "type": "dis", "entity": "慢性复杂运动抽动障碍" }, { "start_idx": 27, "end_idx": 36, "type": "dis", "entity": "慢性复杂发声抽动障碍" }, { "start_idx": 38, "end_idx": 47, "type": "dis", "entity": "慢性单纯运动抽动障碍" }, { "start_idx": 49, "end_idx": 58, "type": "dis", "entity": "慢性单纯发声抽动障碍" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "短暂性抽动障碍" } ] }, { "text": "(2)继发性抽动障碍:很多神经科疾病如亨廷顿舞蹈病、神经棘红细胞病、扭转痉挛、染色体异常和其他遗传性疾病等都可引起抽动,这些情况属于继发性抽动障碍。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "继发性抽动障碍" }, { "start_idx": 13, "end_idx": 15, "type": "dep", "entity": "神经科" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "亨廷顿舞蹈病" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "神经棘红细胞病" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "扭转痉挛" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "染色体异常" }, { "start_idx": 45, "end_idx": 51, "type": "sym", "entity": "其他遗传性疾病" }, { "start_idx": 66, "end_idx": 72, "type": "dis", "entity": "继发性抽动障碍" } ] }, { "text": "与原发性抽动障碍不同的是,这些继发性抽动障碍伴发其他运动障碍,如舞蹈和肌张力障碍等。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "原发性抽动障碍" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "继发性抽动障碍" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "运动障碍" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "舞蹈和肌张力障碍" } ] }, { "text": "此外,获得性抽动障碍包括药物性(左旋多巴、抗精神病药物以及抗惊厥药)、外伤引发的抽动障碍,脑炎、风湿性舞蹈病以及皮质纹状体脊髓变性等感染性疾病,发育异常,精神发育异常,孤独症,卒中(中风),累及基底神经节的变性病(如帕金森病和进行性核上性麻痹),中毒(如一氧化碳中毒)。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "获得性抽动障碍" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "左旋多巴" }, { "start_idx": 21, "end_idx": 26, "type": "dru", "entity": "抗精神病药物" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "抗惊厥药" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "脑炎" }, { "start_idx": 48, "end_idx": 53, "type": "dis", "entity": "风湿性舞蹈病" }, { "start_idx": 56, "end_idx": 64, "type": "dis", "entity": "皮质纹状体脊髓变性" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "发育异常" }, { "start_idx": 77, "end_idx": 82, "type": "sym", "entity": "精神发育异常" }, { "start_idx": 84, "end_idx": 86, "type": "sym", "entity": "孤独症" }, { "start_idx": 88, "end_idx": 93, "type": "sym", "entity": "卒中(中风)" }, { "start_idx": 95, "end_idx": 105, "type": "dis", "entity": "累及基底神经节的变性病" }, { "start_idx": 108, "end_idx": 111, "type": "dis", "entity": "帕金森病" }, { "start_idx": 113, "end_idx": 120, "type": "dis", "entity": "进行性核上性麻痹" }, { "start_idx": 127, "end_idx": 132, "type": "sym", "entity": "一氧化碳中毒" } ] }, { "text": "5.Weingarten分类Weingarten于1968年按病因、疾病范围和严重程度将抽动障碍分为以下四型。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "Weingarten" }, { "start_idx": 14, "end_idx": 23, "type": "dis", "entity": "Weingarten" } ] }, { "text": "(1)无器质性病因的抽动障碍:2岁儿童就可起病,6~12岁明显增多,以后随年龄增长而发病率减少。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "无器质性病因的抽动障碍" } ] }, { "text": "在30岁以前,全部抽动障碍的34左右都属于无器质性病因的抽动障碍。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "全部抽动障碍" }, { "start_idx": 21, "end_idx": 31, "type": "dis", "entity": "无器质性病因的抽动障碍" } ] }, { "text": "在40岁以后,本组抽动障碍则占全部抽动障碍病人总数的12左右。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "本组抽动障碍" } ] }, { "text": "本组抽动常累及面部和颈部,使得病人有种不愉快的感觉。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "面部" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "颈部" } ] }, { "text": "在面部的抽动可有眼部抽动(瞬目抽动)、耳朵抽动、鼻部抽动、唇抽动、舌抽动以及喉头抽动等形式,大多较轻,呈单一性,病人不一定就医。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "面部的抽动" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "眼部抽动" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "耳朵抽动" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "鼻部抽动" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "唇抽动" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "舌抽动" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "喉头抽动" } ] }, { "text": "颈部和胸部的抽动可有扭转样发作。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "颈部" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "胸部" } ] }, { "text": "肩、臂、手的抽动常与面部抽动合并发生,但也有单独发生,呈现上臂突然一个甩动。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肩" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "臂" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "手" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "面部" } ] }, { "text": "下肢抽动十分少见,偶尔病人发作时的表现为行走中突然跳一下。", "entities": [ { "start_idx": 20, "end_idx": 27, "type": "sym", "entity": "行走中突然跳一下" } ] }, { "text": "喉部的发声抽动表现为喉中有“哼、噢”等声音。", "entities": [ { "start_idx": 10, "end_idx": 20, "type": "sym", "entity": "喉中有“哼、噢”等声音" } ] }, { "text": "内脏的抽动相当罕见,有食管和呼吸道的抽动。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "食管" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "呼吸道" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "食管和呼吸道的抽动" } ] }, { "text": "(2)TS:系抽动障碍分类中病情相对较重的一型。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "TS" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "抽动障碍" } ] }, { "text": "(3)由锥体外系器质性疾病等原因引起的继发性抽动障碍。", "entities": [ { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "继发性抽动障碍" } ] }, { "text": "1)纹状体性抽动(striataltics):由各种原因的纹状体损害造成。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "纹状体性抽动" } ] }, { "text": "如脑炎后(如昏睡性脑炎)、神经棘红细胞病、脑血管病、颅脑外伤、脑肿瘤、脑软化灶、中毒性损害(一氧化碳中毒和锰中毒)以及神经梅毒等造成纹状体损害时均可出现抽动样运动障碍(tic-likedyskinesias)。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "脑炎" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "昏睡性脑炎" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "神经棘红细胞病" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "脑血管病" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "颅脑外伤" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "脑肿瘤" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "脑软化灶" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "中毒性损害" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "一氧化碳中毒" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "锰中毒" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "神经梅毒" }, { "start_idx": 76, "end_idx": 82, "type": "dis", "entity": "抽动样运动障碍" } ] }, { "text": "2)斜颈:一般认为斜颈为锥体外系疾病,但功能性反射性抽动(reflextic)导致颈部肌肉一侧的抽动,也可产生斜颈。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "锥体外系疾病" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "功能性反射性抽动" }, { "start_idx": 29, "end_idx": 37, "type": "dis", "entity": "reflextic" } ] }, { "text": "吩噻嗪(phenothiazine)治疗精神病时的副作用可为抽动样多动。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "吩噻嗪" } ] }, { "text": "左旋多巴及卡马西平等药物都可引起抽动样多动。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "左旋多巴" }, { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "卡马西平" } ] }, { "text": "4)头颈部疾患所致的继发性抽动障碍:该型包括反射性抽动(reflextic)、偏侧面肌痉挛(hemifacialspasms)和面轻瘫后抽动(ticfollowingfacialparesis),与功能性抽动障碍的区别在于它们只累及面神经支配的肌群,而不影响颈、肩和肢体的肌群。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "继发性抽动障碍" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "反射性抽动" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "偏侧面肌痉挛" }, { "start_idx": 46, "end_idx": 61, "type": "sym", "entity": "hemifacialspasms" }, { "start_idx": 64, "end_idx": 69, "type": "sym", "entity": "面轻瘫后抽动" }, { "start_idx": 99, "end_idx": 105, "type": "dis", "entity": "功能性抽动障碍" }, { "start_idx": 129, "end_idx": 129, "type": "bod", "entity": "颈" }, { "start_idx": 131, "end_idx": 131, "type": "bod", "entity": "肩" }, { "start_idx": 133, "end_idx": 134, "type": "bod", "entity": "肢体" } ] }, { "text": "抽动成丛集性发作,每一次发作持续时间不等(半秒钟到1min),总共持续数秒钟到数分钟。", "entities": [ { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "每一次发作持续时间不等" }, { "start_idx": 31, "end_idx": 41, "type": "sym", "entity": "总共持续数秒钟到数分钟" } ] }, { "text": "通常在睡眠时抽动也不消失,甚至在麻醉的情况下抽动也不消失,且抽动难以通过主观意志来控制。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "睡眠时抽动也不消失" } ] }, { "text": "治疗上,传统的神经安定类药物无效,可局部注射肉毒杆菌毒素。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "注射" }, { "start_idx": 22, "end_idx": 27, "type": "dru", "entity": "肉毒杆菌毒素" } ] }, { "text": "6.Robertson分类1988年Robertson等提出,从临床的角度可将抽动障碍分为以下三类。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "Robertson" } ] }, { "text": "(1)单纯性抽动障碍:临床上主要表现为或几乎表现为运动或发声抽动者。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "单纯性抽动障碍" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "运动或发声抽动者" } ] }, { "text": "(2)全面发展的抽动障碍:除多发性抽动障碍外,可有秽语、模仿或应声性发声或回文现象(paliphenomena,即语句既顺着说又倒着说)。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "全面发展的抽动障碍" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "多发性抽动障碍" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "秽语" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "模仿" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "应声性发声" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "回文现象" } ] }, { "text": "(3)抽动障碍附加症(ticdisordersplus,TD+):指除有抽动障碍外,还伴有ADHD、强迫-冲动症状(OCS)强迫-冲动行为(OCB)或自伤行为等共患病症;抽动障碍伴有抑郁、焦虑、性格改变、社会和学校生活适应困难,甚至有反社会行为者,也可列入此类。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "抽动障碍附加症" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "ADHD" }, { "start_idx": 55, "end_idx": 61, "type": "sym", "entity": "强迫-冲动症状" }, { "start_idx": 67, "end_idx": 73, "type": "sym", "entity": "强迫-冲动行为" }, { "start_idx": 80, "end_idx": 83, "type": "sym", "entity": "自伤行为" }, { "start_idx": 90, "end_idx": 93, "type": "dis", "entity": "抽动障碍" }, { "start_idx": 96, "end_idx": 97, "type": "sym", "entity": "抑郁" }, { "start_idx": 99, "end_idx": 100, "type": "sym", "entity": "焦虑" }, { "start_idx": 102, "end_idx": 105, "type": "sym", "entity": "性格改变" }, { "start_idx": 107, "end_idx": 117, "type": "sym", "entity": "社会和学校生活适应困难" } ] }, { "text": "二、梦魇梦魇即做噩梦,它常常使睡眠者从REM睡眠中醒来,影响REM睡眠。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "梦魇" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "梦魇" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "噩梦" } ] }, { "text": "有关梦魇的发生率各研究的报道不一致。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "梦魇" } ] }, { "text": "3~5岁的儿童中10%~50%有相当多的梦魇,约75%的小儿在童年期至少有一次或几次的梦魇,约50%的成人承认至少有过一次梦魇。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "梦魇" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "梦魇" }, { "start_idx": 61, "end_idx": 62, "type": "dis", "entity": "梦魇" } ] }, { "text": "可能有1%的成人有每周一次或一次以上的频繁梦魇。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "梦魇" } ] }, { "text": "梦魇通常起始于3~6岁,但可发生于任何年龄。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "梦魇" } ] }, { "text": "有研究表明,频繁发作、持续终身的梦魇有家族倾向。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "梦魇" } ] }, { "text": "【病因及易感因素】梦魇的病因不明。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "梦魇" } ] }, { "text": "【临床表现】梦魇的总是长而复杂的梦,从开始到结束其内容越来越恐怖。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "梦魇" } ] }, { "text": "觉醒发生于REM睡眠期,有时不是立即觉醒,而是随之回忆一个非常恐怖的梦,但这种状况在梦魇中并不常见。", "entities": [ { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "梦魇" } ] }, { "text": "恐怖和焦虑是梦魇的主要成分。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "梦魇" } ] }, { "text": "梦魇时很少有讲话、尖叫、行走,这就有别于夜惊和REM睡眠行为障碍。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "梦魇" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "讲话" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "尖叫" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "行走" } ] }, { "text": "父母常常在小儿2~3岁时就发现梦魇,但小儿只有在3~4岁才能描述恐怖的梦。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "梦魇" } ] }, { "text": "梦魇的发作次数常常在几个星期、几个月或几年后明显减少。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "梦魇" } ] }, { "text": "部分儿童的梦魇可持续到青春期甚至成人,这些个体通常成为频繁发作、持续终身的患者。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "梦魇" } ] }, { "text": "【诊断】(一)睡眠障碍的国际分型中梦魇的诊断标准1.至少有一次突然从睡眠中醒来,伴随极度的害怕、焦虑,感觉将有危害降临。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "梦魇" }, { "start_idx": 26, "end_idx": 38, "type": "sym", "entity": "至少有一次突然从睡眠中醒来" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "伴随极度的害怕" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "焦虑" }, { "start_idx": 51, "end_idx": 58, "type": "sym", "entity": "感觉将有危害降临" } ] }, { "text": "2.患者能立即回忆恐怖的梦境内容。", "entities": [ { "start_idx": 4, "end_idx": 15, "type": "sym", "entity": "能立即回忆恐怖的梦境内容" } ] }, { "text": "3.醒来后立即完全清醒,几乎没有混乱或迷惑。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "醒来后立即完全清醒" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "没有混乱或迷惑" } ] }, { "text": "4.至少有以下一个相关特征(1)发作后继续睡眠,但并不是迅速入睡。", "entities": [ { "start_idx": 16, "end_idx": 31, "type": "sym", "entity": "发作后继续睡眠,但并不是迅速入睡" } ] }, { "text": "(2)发生于平时睡眠期的后1/2。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "sym", "entity": "发生于平时睡眠期的后1/2" } ] }, { "text": "5.PSG的特征(1)从已有持续10分钟以上的REM睡眠期突然醒来。", "entities": [ { "start_idx": 11, "end_idx": 32, "type": "sym", "entity": "从已有持续10分钟以上的REM睡眠期突然醒来" } ] }, { "text": "(2)发作时轻度心动过速和呼吸加快。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "sym", "entity": "发作时轻度心动过速和呼吸加快" } ] }, { "text": "(3)没有癫痫活动。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "没有癫痫活动" } ] }, { "text": "6.可以与其他的睡眠障碍,如夜惊和梦游并存。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "夜惊" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "梦游" } ] }, { "text": "临床诊断梦魇至少符合上述标准的1~4项。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "梦魇" } ] }, { "text": "(二)梦魇根据其严重程度可分为三度1.轻度发作少于每周一次,没有心理社交功能损害的表现。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "梦魇" }, { "start_idx": 21, "end_idx": 42, "type": "sym", "entity": "发作少于每周一次,没有心理社交功能损害的表现" } ] }, { "text": "2.中度每周发作一次以上,但不是每夜发生,有轻度的心理社交损害的表现。", "entities": [ { "start_idx": 4, "end_idx": 33, "type": "sym", "entity": "每周发作一次以上,但不是每夜发生,有轻度的心理社交损害的表现" } ] }, { "text": "3.重度每夜发作,有中度或重度心理社交功能损害的表现。", "entities": [ { "start_idx": 4, "end_idx": 25, "type": "sym", "entity": "每夜发作,有中度或重度心理社交功能损害的表现" } ] }, { "text": "【鉴别诊断】(一)夜惊见表3-3。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "夜惊" } ] }, { "text": "表3-7夜惊和梦魇的鉴别诊断(二)REM睡眠行为障碍较常见于中年以上者,在REM睡眠期常有暴力的、暴发性的动作,但并不突然觉醒至完全清醒,较少有害怕和恐惧的表现,可见特征性的PSG。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "梦魇" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "REM睡眠行为障碍" }, { "start_idx": 36, "end_idx": 54, "type": "sym", "entity": "在REM睡眠期常有暴力的、暴发性的动作" }, { "start_idx": 56, "end_idx": 67, "type": "sym", "entity": "但并不突然觉醒至完全清醒" }, { "start_idx": 69, "end_idx": 79, "type": "sym", "entity": "较少有害怕和恐惧的表现" } ] }, { "text": "【治疗】偶发梦魇无需特殊治疗。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "梦魇" } ] }, { "text": "第十节脊髓灰质炎脊髓灰质炎(poliomyelitis,简称灰髓炎)是由脊髓灰质炎病毒引起的急性神经系统传染病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "脊髓灰质炎" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "脊髓灰质炎" }, { "start_idx": 14, "end_idx": 26, "type": "dis", "entity": "poliomyelitis" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "灰髓炎" }, { "start_idx": 36, "end_idx": 42, "type": "mic", "entity": "脊髓灰质炎病毒" }, { "start_idx": 46, "end_idx": 54, "type": "dis", "entity": "急性神经系统传染病" } ] }, { "text": "临床特征为分布不规则和轻重不等的弛缓性肌肉麻痹。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肌肉" }, { "start_idx": 5, "end_idx": 22, "type": "sym", "entity": "分布不规则和轻重不等的弛缓性肌肉麻痹" } ] }, { "text": "重者可于急性期因呼吸、吞咽麻痹而死亡。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "呼吸、吞咽麻痹" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "死亡" } ] }, { "text": "我国推行疫苗预防后发病率已大大下降。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dru", "entity": "疫苗" } ] }, { "text": "【病原和流行病学】脊髓灰质炎病毒(poliovirus,简称灰髓炎病毒)为无包膜的单正链RNA病毒,属小RNA病毒科肠道病毒属。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "mic", "entity": "脊髓灰质炎病毒" }, { "start_idx": 17, "end_idx": 26, "type": "mic", "entity": "poliovirus" }, { "start_idx": 30, "end_idx": 34, "type": "mic", "entity": "灰髓炎病毒" }, { "start_idx": 41, "end_idx": 48, "type": "mic", "entity": "单正链RNA病毒" }, { "start_idx": 51, "end_idx": 56, "type": "mic", "entity": "小RNA病毒" }, { "start_idx": 58, "end_idx": 61, "type": "mic", "entity": "肠道病毒" } ] }, { "text": "3型病毒间无共同抗原,可分别感染人类而致病。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "3型病毒" } ] }, { "text": "病毒可在很多灵长类动物非神经组织的原代或传代细胞中增殖。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" } ] }, { "text": "病毒在外环境中较为稳定,在粪便中能存活6个月,生活污水中存活4个月。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "粪便" } ] }, { "text": "紫外线、漂白粉、过氧化氢等氧化剂、高锰酸钾、甲醛、碘酊和升汞等均能杀灭之。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "过氧化氢" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "氧化剂" }, { "start_idx": 17, "end_idx": 20, "type": "dru", "entity": "高锰酸钾" }, { "start_idx": 22, "end_idx": 23, "type": "dru", "entity": "甲醛" }, { "start_idx": 25, "end_idx": 26, "type": "dru", "entity": "碘酊" }, { "start_idx": 28, "end_idx": 29, "type": "dru", "entity": "升汞" } ] }, { "text": "在潜伏期末,患者从鼻咽分泌物和粪便中排出病毒。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "鼻咽分泌物" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "粪便" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "病毒" } ] }, { "text": "病毒在咽部存在时间一般不超过病后一周。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "咽部" } ] }, { "text": "在粪便中排病毒时间较久,个别可长达3~4个月。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "粪便" }, { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "病毒" } ] }, { "text": "病毒污染用具、玩具、食物或水源,易感者接触或食用后获感染。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" } ] }, { "text": "1994年9月以来我国已成功阻断本土脊灰野病毒传播。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "mic", "entity": "脊灰野病毒" } ] }, { "text": "1995年11月~1996年4月发现4例输入野病毒病例和1999年1例输入野病毒病例后,未再发现野病毒传播的证据。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "mic", "entity": "野病毒" }, { "start_idx": 37, "end_idx": 39, "type": "mic", "entity": "野病毒" }, { "start_idx": 48, "end_idx": 50, "type": "mic", "entity": "野病毒" } ] }, { "text": "【发病机制和病理改变】病毒先在咽部和肠壁的淋巴组织内增殖。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "病毒" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "咽部" }, { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "肠壁的淋巴组织" } ] }, { "text": "机体产生特异性抗体,可阻止病毒增殖,使感染中断,形成隐性感染。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "特异性抗体" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "病毒" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "感染" } ] }, { "text": "在少数患者,病毒可侵入血液循环,形成第一次病毒血症。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "病毒" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血液" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "病毒血症" } ] }, { "text": "病毒到达全身淋巴组织和单核/吞噬细胞内继续增殖,再次入血(第二次病毒血症),此时患者有发热等前驱期症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "淋巴组织" }, { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "单核/吞噬细胞" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "血" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "病毒血症" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "发热" } ] }, { "text": "若产生的特异性抗体使疾病停止发展,则成为顿挫型。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "特异性抗体" } ] }, { "text": "如病毒量多、毒力大,透过血-脑屏障侵犯神经组织,产生瘫痪等症状;若病变轻微,可无瘫痪发生。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "mic", "entity": "病毒" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "血-脑屏障" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "神经组织" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "瘫痪" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "无瘫痪" } ] }, { "text": "病变以脊髓前角的运动神经元损害为主,尤以颈段和腰段损害多见。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "bod", "entity": "脊髓前角的运动神经元" }, { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "脊髓前角的运动神经元损害" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "颈段" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "腰段" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "颈段和腰段损害" } ] }, { "text": "其次受损部位为脑干,病灶呈多发散在性,可见神经细胞内胞质染色质溶解,周围组织水肿、充血和血管周围炎性细胞浸润。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "脑干" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "病灶呈多发散在性" }, { "start_idx": 21, "end_idx": 30, "type": "bod", "entity": "神经细胞内胞质染色质" }, { "start_idx": 21, "end_idx": 32, "type": "sym", "entity": "神经细胞内胞质染色质溶解" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "周围组织水肿、充血" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "血管" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "炎性细胞" }, { "start_idx": 44, "end_idx": 53, "type": "sym", "entity": "血管周围炎性细胞浸润" } ] }, { "text": "严重者见神经细胞坏死和瘢痕形成。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "神经细胞" }, { "start_idx": 4, "end_idx": 14, "type": "sym", "entity": "神经细胞坏死和瘢痕形成" } ] }, { "text": "根据有无神经系统损害及损害程度和部位,又分为顿挫型、无瘫痪型、瘫痪型和脑型。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "神经系统" }, { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "神经系统损害" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "无瘫痪" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "瘫痪" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "脑" } ] }, { "text": "典型病例的临床经过如下:(一)前驱期多有低热或中度发热,伴食欲减退、乏力、全身不适和头痛等一般“感冒”症状,或有腹痛、呕吐、腹泻、便秘等胃肠道症状以及咽痛、咳嗽、流涕等呼吸道症状。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "低热" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "中度发热" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "食欲减退" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "乏力" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "全身不适" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "头痛" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "感冒" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "腹痛" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "呕吐" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "腹泻" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "便秘" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "胃肠道" }, { "start_idx": 68, "end_idx": 72, "type": "sym", "entity": "胃肠道症状" }, { "start_idx": 75, "end_idx": 76, "type": "sym", "entity": "咽痛" }, { "start_idx": 78, "end_idx": 79, "type": "sym", "entity": "咳嗽" }, { "start_idx": 81, "end_idx": 82, "type": "sym", "entity": "流涕" }, { "start_idx": 84, "end_idx": 86, "type": "bod", "entity": "呼吸道" }, { "start_idx": 84, "end_idx": 88, "type": "sym", "entity": "呼吸道症状" } ] }, { "text": "(二)瘫痪前期经2~6天的静止阶段,体温再次升高,进入瘫痪前期。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "瘫痪" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "瘫痪" } ] }, { "text": "患者有全身兴奋状态,面赤、皮肤微红、多汗,可有呕吐和咽痛。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "全身兴奋状态" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "面赤" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "皮肤" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "皮肤微红" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "多汗" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "呕吐" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "咽痛" } ] }, { "text": "全身或四肢肌肉疼痛,感觉过敏,不愿他人抚抱。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "全身或四肢肌肉" }, { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "全身或四肢肌肉疼痛" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "感觉过敏" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "不愿他人抚抱" } ] }, { "text": "颈项强直,弯曲时疼痛。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "颈项" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "颈项强直" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "弯曲时疼痛" } ] }, { "text": "有脑膜刺激征和脑脊液中白细胞数增高。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "脑膜" }, { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "脑膜刺激征" }, { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "脑脊液中白细胞" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "脑脊液中白细胞数增高" } ] }, { "text": "如疾病终止,无瘫痪出现,称为无瘫痪型。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "无瘫痪" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "无瘫痪" } ] }, { "text": "(三)瘫痪期肌肉瘫痪大都于瘫痪前期的第3~4天开始,随发热而加重,大都经过5~10天。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "瘫痪" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肌肉" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "肌肉瘫痪" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "瘫痪" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "发热" } ] }, { "text": "一般热退后瘫痪不再进展。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "瘫痪" } ] }, { "text": "依据主要病变部位分为下列数型:1.脊髓型最常见,为脊髓前角细胞受损所致,表现为分布不对称、不规则的弛缓性肌肉瘫痪,四肢多见,下肢尤甚。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "脊髓" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "脊髓前角细胞" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "脊髓前角细胞受损" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "肌肉" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "四肢" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "下肢" }, { "start_idx": 39, "end_idx": 65, "type": "sym", "entity": "分布不对称、不规则的弛缓性肌肉瘫痪,四肢多见,下肢尤甚" } ] }, { "text": "感觉存在。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "感觉存在" } ] }, { "text": "膀胱肌麻痹时则有尿潴留和尿失禁。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "膀胱肌" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "尿" }, { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "膀胱肌麻痹时则有尿潴留和尿失禁" } ] }, { "text": "2.延髓型(脑干型或球型)出现呼吸深浅不匀、节律不齐和各种异样呼吸;心动过速或过缓、血压下降、循环衰竭;侵犯脑神经则产生各种相应症状。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脑干" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "呼吸深浅不匀" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "节律不齐" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "异样呼吸" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "心动过速或过缓" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "血压下降" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "循环衰竭" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "脑神经" }, { "start_idx": 52, "end_idx": 56, "type": "sym", "entity": "侵犯脑神经" } ] }, { "text": "3.脑炎型偶见。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "脑炎" } ] }, { "text": "急起高热、嗜睡、昏迷和惊厥,可有痉挛性肢体瘫痪。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "高热" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "嗜睡" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "昏迷" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "惊厥" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肢体" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "痉挛性肢体瘫痪" } ] }, { "text": "4.混合型为脊髓型和延髓型同时存在。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脊髓" } ] }, { "text": "(四)恢复期瘫痪后1~2周,病肌逐渐恢复功能,常以足趾为起点,然后上升至胫、股部。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "瘫痪" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "病肌逐渐恢复功能" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "足趾" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "胫" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "股部" } ] }, { "text": "膝腱反射也渐回复。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "膝腱反射也渐回复" } ] }, { "text": "(五)后遗症期由于神经组织损害严重,瘫痪不易恢复,受累肌群萎缩,造成躯肢畸形,如马蹄内翻足、脊柱弯曲等后遗症。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "神经组织" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "神经组织损害严重" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "瘫痪" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "瘫痪不易恢复" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "受累肌群萎缩" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "躯肢" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "躯肢畸形" } ] }, { "text": "【并发症】少数患者在急性期有心肌损害。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "心肌" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "心肌损害" } ] }, { "text": "呼吸肌麻痹和呼吸中枢受累患者因呼吸不畅可并发肺炎、肺不张等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "呼吸肌麻痹" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "呼吸中枢" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "呼吸中枢受累" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "呼吸不畅" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "肺炎" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "肺不张" } ] }, { "text": "尿潴留者易继发泌尿系感染。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "尿潴留" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "泌尿系感染" } ] }, { "text": "严重瘫痪长期卧床者易有褥疮、骨质脱钙,甚至肾结石。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "严重瘫痪" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "褥疮" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "骨质脱钙" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "肾结石" } ] }, { "text": "尚可有麻痹性肠梗阻和胃扩张等胃肠道并发症。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "麻痹性肠梗阻" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "胃扩张" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "胃肠道并发症" } ] }, { "text": "【实验室检查】(一)脑脊液检查前驱期脑脊液正常;瘫痪前期细胞数轻度增加,早期以中性粒细胞为主,后期以淋巴细胞为主;热退后细胞数恢复。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "脑脊液" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "瘫痪" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "细胞数轻度增加" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 57, "end_idx": 64, "type": "sym", "entity": "热退后细胞数恢复" } ] }, { "text": "蛋白质在早期多数正常,瘫痪后第2周蛋白质水平常增高,此时细胞数已正常,呈细胞和蛋白质分离现象,对诊断本病有一定帮助。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "蛋白质" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "瘫痪" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "蛋白质" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "蛋白质水平常增高" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "细胞" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "蛋白质" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "细胞和蛋白质分离" } ] }, { "text": "蛋白质水平到第4~10周恢复正常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "蛋白质" } ] }, { "text": "(二)病毒分离起病1周内可从咽部和粪便内分离出病毒,早期从血液和脑脊液中有时也能检出病毒。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "病毒分离" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "咽部" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "粪便" }, { "start_idx": 23, "end_idx": 24, "type": "mic", "entity": "病毒" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "血液" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "脑脊液" }, { "start_idx": 42, "end_idx": 43, "type": "mic", "entity": "病毒" } ] }, { "text": "需用标准抗血清中和试验鉴定血清型。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "pro", "entity": "抗血清中和试验" } ] }, { "text": "(三)血清学检查前驱期末,特异性IgG抗体出现,补体结合抗体持续时间较短,中和抗体持续较久。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "血清学检查" }, { "start_idx": 13, "end_idx": 20, "type": "bod", "entity": "特异性IgG抗体" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "抗体" } ] }, { "text": "双份血清抗体滴度呈≥4倍以上增高有诊断意义。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "血清抗体" } ] }, { "text": "对顿挫型和无瘫痪型患者,只能依靠病原学检查结果诊断。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "无瘫痪" } ] }, { "text": "瘫痪患者需要和下列疾病鉴别:(一)急性感染性多发性神经根炎表现为四肢弛缓性瘫痪,常从下肢开始,然后发展到上肢、腰背及躯干。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "瘫痪" }, { "start_idx": 17, "end_idx": 28, "type": "dis", "entity": "急性感染性多发性神经根炎" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "四肢" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "四肢弛缓性瘫痪" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "下肢" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "上肢" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "腰背" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "躯干" } ] }, { "text": "病初就有肢体感觉障碍。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肢体" }, { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "肢体感觉障碍" } ] }, { "text": "脑脊液中细胞数正常而蛋白质特高。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑脊液" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "蛋白质" }, { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "脑脊液中细胞数正常而蛋白质特高" } ] }, { "text": "(二)其他肠道病毒所致瘫痪,接受过有效灰髓炎疫苗预防的患者如出现类似本病的瘫痪,应进行病原学检查来确诊。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "mic", "entity": "肠道病毒" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "瘫痪" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "灰髓炎疫苗" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "瘫痪" } ] }, { "text": "最初1周强调呼吸道和消化道隔离。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "呼吸道" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "消化道" } ] }, { "text": "(二)被动免疫暴露前肌内注射人丙种球蛋白0.3~0.5ml/kg,有一定保护作用。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "肌内注射" }, { "start_idx": 14, "end_idx": 19, "type": "dru", "entity": "人丙种球蛋白" } ] }, { "text": "(三)主动免疫按计划普遍服用灰髓炎减毒活疫苗。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "dru", "entity": "灰髓炎减毒活疫苗" } ] }, { "text": "在流行发生时,对周围的易感儿及时施行疫苗预防可中断流行。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "dru", "entity": "疫苗" } ] }, { "text": "(四)急性期的治疗1.前驱期和瘫痪前期治疗患者宜卧床休息以减少麻痹的发生和进展。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "瘫痪" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "卧床休息" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "麻痹" } ] }, { "text": "有肌痛者可作局部湿热敷或口服镇痛剂。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "肌痛" }, { "start_idx": 6, "end_idx": 16, "type": "pro", "entity": "局部湿热敷或口服镇痛剂" } ] }, { "text": "静脉注射适量50%葡萄糖液和维生素C可减少神经系统水肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "静脉注射" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "葡萄糖液" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "维生素C" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "神经系统" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "神经系统水肿" } ] }, { "text": "口服地巴唑可促进神经系统功能的恢复。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "口服" }, { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "地巴唑" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "神经系统" } ] }, { "text": "2.瘫痪期治疗注射加兰他敏可促进肌肉的张力。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "瘫痪" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "注射" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "加兰他敏" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肌肉" } ] }, { "text": "将瘫痪的肢体置于功能位,以防止畸形发生。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "瘫痪" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "畸形" } ] }, { "text": "有呼吸肌麻痹时,应保持呼吸道通畅,并给予氧气吸入和各种辅助呼吸。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "呼吸肌" }, { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "呼吸肌麻痹" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "保持呼吸道通畅" }, { "start_idx": 18, "end_idx": 30, "type": "pro", "entity": "给予氧气吸入和各种辅助呼吸" } ] }, { "text": "呼吸中枢或循环中枢受累时按呼吸衰竭或循环衰竭抢救措施进行。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "呼吸中枢" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "循环中枢" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "呼吸中枢或循环中枢受累" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "呼吸衰竭" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "循环衰竭" } ] }, { "text": "3.后遗症期治疗于体温降至正常、病情进入恢复期时就应加强瘫痪肌群的功能锻炼,并作针刺、推拿、按摩、理疗等康复治疗,以促进瘫痪肌群的恢复。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "体温降至正常" }, { "start_idx": 26, "end_idx": 36, "type": "pro", "entity": "加强瘫痪肌群的功能锻炼" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "针刺" }, { "start_idx": 43, "end_idx": 44, "type": "pro", "entity": "推拿" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "按摩" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "理疗" }, { "start_idx": 58, "end_idx": 66, "type": "pro", "entity": "促进瘫痪肌群的恢复" } ] }, { "text": "遗有畸形时,可手术矫治。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "畸形" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "手术" } ] }, { "text": "十二、腺泡型软组织肉瘤腺泡型软组织肉瘤(alveolarsoftpartsarcoma)是一临床-病理实体,小儿少见,多见于15岁左右的青少年,女性多于男性。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "腺泡型软组织肉瘤" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "腺泡型软组织肉瘤" }, { "start_idx": 20, "end_idx": 42, "type": "dis", "entity": "alveolarsoftpartsarcoma" } ] }, { "text": "肿瘤无明显界限质软和易碎。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "肿瘤无明显界限" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "质软和易碎" } ] }, { "text": "切面呈黄白或灰红色,中央含坏死和出血区,肿瘤光镜下致密的小梁将肿瘤分为大小不同的腔隙腔隙中有界限分明的瘤细胞岛瘤细胞中有坏死和脱落肿瘤在儿童好发于头颈,尤其是眼眶和舌。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "切面呈黄白或灰红色" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "中央含坏死和出血区" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肿瘤" }, { "start_idx": 22, "end_idx": 23, "type": "equ", "entity": "光镜" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "肿瘤" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "腔隙" }, { "start_idx": 20, "end_idx": 41, "type": "sym", "entity": "肿瘤光镜下致密的小梁将肿瘤分为大小不同的腔隙" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "腔隙" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "瘤细胞岛" }, { "start_idx": 42, "end_idx": 54, "type": "sym", "entity": "腔隙中有界限分明的瘤细胞岛" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "瘤细胞" }, { "start_idx": 55, "end_idx": 64, "type": "sym", "entity": "瘤细胞中有坏死和脱落" }, { "start_idx": 65, "end_idx": 66, "type": "dis", "entity": "肿瘤" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "头颈" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "眼眶" }, { "start_idx": 82, "end_idx": 82, "type": "bod", "entity": "舌" } ] }, { "text": "因肿瘤内血管丰富,有时可摸及血管搏动,故一般无症状,而易忽视。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "血管" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "血管" } ] }, { "text": "该肿瘤早期就可向肺、脑、骨骼转移。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肺" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "脑" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "骨骼" } ] }, { "text": "淋巴结转移少见。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "淋巴结" } ] }, { "text": "治疗以根治手术切除原发肿瘤及转移灶。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "手术切除" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "肿瘤" } ] }, { "text": "儿童预后较成人佳,可能因头面部肿瘤易于发现的缘故。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "头面部肿瘤" } ] }, { "text": "二、急性咽炎急性咽炎(acutepharyngitis)是咽黏膜、黏膜下组织和淋巴组织的急性炎症。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "急性咽炎" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "急性咽炎" }, { "start_idx": 12, "end_idx": 27, "type": "dis", "entity": "acutepharyngitis" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "咽黏膜" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "黏膜下组织" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "淋巴组织" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "急性炎症" } ] }, { "text": "单纯性咽炎以学龄前儿童多见,婴儿期较少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "单纯性咽炎" } ] }, { "text": "1.疱疹性咽炎(herpangina)系柯萨奇A组病毒引起。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "疱疹性咽炎" }, { "start_idx": 8, "end_idx": 17, "type": "dis", "entity": "herpangina" }, { "start_idx": 20, "end_idx": 26, "type": "mic", "entity": "柯萨奇A组病毒" } ] }, { "text": "急性起病,表现高热、咽痛、流涎、厌食、呕吐等,咽部充血,咽腭弓、腭垂、软腭等处可伴有2~4mm大小的疱疹,周围有红晕,破溃后形成小溃疡.病程1周左右。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "高热" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "咽痛" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "流涎" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "厌食" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "呕吐" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "咽部充血" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "咽腭弓" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "腭垂" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "软腭" }, { "start_idx": 28, "end_idx": 66, "type": "sym", "entity": "咽腭弓、腭垂、软腭等处可伴有2~4mm大小的疱疹,周围有红晕,破溃后形成小溃疡" } ] }, { "text": "2.咽-结合膜热(pharyngo-conjunctivitis)由腺病毒3、7型引起。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "咽-结合膜热" }, { "start_idx": 9, "end_idx": 31, "type": "dis", "entity": "pharyngo-conjunctivitis" }, { "start_idx": 34, "end_idx": 40, "type": "mic", "entity": "腺病毒3、7型" } ] }, { "text": "以发热、咽炎、结合膜炎为特点,表现高热、咽痛、眼部刺痛、咽部充血、一侧或两侧滤泡性眼结合膜炎,可伴有胃肠道症状、颈部、耳后淋巴结肿大。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "发热" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "咽炎" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "结合膜炎" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "高热" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "咽痛" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "眼部" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "眼部刺痛" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "咽部" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "咽部充血" }, { "start_idx": 38, "end_idx": 45, "type": "dis", "entity": "滤泡性眼结合膜炎" }, { "start_idx": 33, "end_idx": 45, "type": "sym", "entity": "一侧或两侧滤泡性眼结合膜炎" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "胃肠道" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "颈部" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "耳后淋巴结" }, { "start_idx": 56, "end_idx": 65, "type": "sym", "entity": "颈部、耳后淋巴结肿大" } ] }, { "text": "3.链球菌咽炎(streptococcalpharyngitis)以5岁以上高发。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "链球菌咽炎" }, { "start_idx": 8, "end_idx": 31, "type": "dis", "entity": "streptococcalpharyngitis" } ] }, { "text": "早期可伴有颈前淋巴结肿大伴触痛扁桃体广泛充血、软腭表面有瘀点、瘀斑提示链球菌感染。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "颈前淋巴结" }, { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "颈前淋巴结肿大伴触痛" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "扁桃体" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "扁桃体广泛充血" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "软腭" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "软腭表面有瘀点" }, { "start_idx": 31, "end_idx": 39, "type": "sym", "entity": "瘀斑提示链球菌感染" } ] }, { "text": "第四章脑积水脑积水(hydrocephalus)系指脑脊液的分泌、循环或吸收过程发生障碍,导致颅内脑脊液增多,引起脑室和(或)蛛网膜下腔异常扩大过量的脑脊液产生颅内压增高,因而扩大了正常脑脊液所占有的空间脑积水在颅缝闭合之前发生,则头颅增大异常显著脑积水称为先天性脑积水,在出生后有明确病因产生的脑积水称为后天性(获得性)脑积水。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脑积水" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑积水" }, { "start_idx": 10, "end_idx": 22, "type": "dis", "entity": "hydrocephalus" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "脑脊液" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "脑脊液" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "脑室" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 47, "end_idx": 71, "type": "sym", "entity": "颅内脑脊液增多,引起脑室和(或)蛛网膜下腔异常扩大" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "脑脊液" }, { "start_idx": 80, "end_idx": 82, "type": "ite", "entity": "颅内压" }, { "start_idx": 93, "end_idx": 95, "type": "bod", "entity": "脑脊液" }, { "start_idx": 72, "end_idx": 101, "type": "sym", "entity": "过量的脑脊液产生颅内压增高,因而扩大了正常脑脊液所占有的空间" }, { "start_idx": 102, "end_idx": 104, "type": "dis", "entity": "脑积水" }, { "start_idx": 116, "end_idx": 117, "type": "bod", "entity": "头颅" }, { "start_idx": 116, "end_idx": 123, "type": "sym", "entity": "头颅增大异常显著" }, { "start_idx": 124, "end_idx": 126, "type": "dis", "entity": "脑积水" }, { "start_idx": 129, "end_idx": 134, "type": "dis", "entity": "先天性脑积水" }, { "start_idx": 148, "end_idx": 150, "type": "dis", "entity": "脑积水" } ] }, { "text": "由于各种原因引起脑实质本身先发生萎缩而后使脑室和蛛网膜下腔扩大,脑脊液容量相对增加脑积水的范畴。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "脑室" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "蛛网膜" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "脑脊液" }, { "start_idx": 8, "end_idx": 40, "type": "sym", "entity": "脑实质本身先发生萎缩而后使脑室和蛛网膜下腔扩大,脑脊液容量相对增加" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "脑积水" } ] }, { "text": "【流行病学】先天性脑积水是最常见的先天神经系统畸形疾病之一,据WHO对24个国家的统计报告,其发病率为0.87‰,男女无明显差别。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "先天性脑积水" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "先天神经系统畸形" } ] }, { "text": "我国1996—2007年31个省市自治区出生缺陷监测资料表明,先天性脑积水的发病率为0.68‰,仅次于神经管畸形。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "出生缺陷" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "先天性脑积水" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "神经管畸形" } ] }, { "text": "由于后天性脑积水是出生后多种原因引起的一种病理结果,则其确切发病率很难统计。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "后天性脑积水" } ] }, { "text": "随着医学科学技术的进步,先天性或后天性脑积水的发病率呈降低趋势。", "entities": [ { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "先天性或后天性脑积水" } ] }, { "text": "根据我国2004—2007年出生缺陷监测网资料,全国先天性脑积水的发病率有明显下降趋势,下降幅度为16.4%,年下降速率为9.0%,且城市较农村下降明显。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "出生缺陷" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "先天性脑积水" } ] }, { "text": "【病理生理】正常情况下,脑脊液在脑室系统和蛛网膜下腔内不断地循环、代谢,其分泌和吸收速度处于动态平衡,从而维持颅内脑脊液容量的相对稳定。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "脑脊液" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "脑室" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "颅内脑脊液" } ] }, { "text": "脑脊液的产生主要来自各个脑室特别是侧脑室的脉络丛,约占80%~85%,少数由室管膜上皮渗出,在小儿每分钟产生脑脊液0.3~0.35ml(平均每小时20ml)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑脊液" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "脑室" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "侧脑室" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "脉络丛" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "管膜上皮" }, { "start_idx": 54, "end_idx": 56, "type": "ite", "entity": "脑脊液" } ] }, { "text": "脑脊液在脑室生成后,以约1.47kPa(150mmH2O)液体静水压循环流动。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑脊液" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "脑室" } ] }, { "text": "左右两侧侧脑室产生的脑脊液,经室间孔流入第三脑室,与第三脑室产生的脑脊液一起经中脑导水管(又称大脑导水管)流入第四脑室,再与第四脑室产生的脑脊液一起经正中孔和两个侧孔流出而进入蛛网膜下腔的小脑延髓池。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "侧脑室" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "脑脊液" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "室间孔" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "第三脑室" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "第三脑室" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "脑脊液" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "中脑导水管" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "大脑导水管" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "第四脑室" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "第四脑室" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "脑脊液" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "正中孔" }, { "start_idx": 88, "end_idx": 92, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 94, "end_idx": 98, "type": "bod", "entity": "小脑延髓池" } ] }, { "text": "蛛网膜下腔的脑脊液,向上循环到脑表面,最后通过蛛网膜绒毛(颗粒)被动地吸收入硬膜静脉窦(上矢状窦),这是脑脊液吸收的主要途径。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "脑脊液" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "脑表面" }, { "start_idx": 23, "end_idx": 31, "type": "bod", "entity": "蛛网膜绒毛(颗粒)" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "硬膜静脉窦" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "上矢状窦" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "脑脊液" } ] }, { "text": "一小部分的脑脊液进入脊髓蛛网膜下腔,由脊髓静脉的蛛网膜绒毛吸收入血液。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "脑脊液" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "脊髓蛛网膜下腔" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "脊髓静脉" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "蛛网膜绒毛" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "血液" } ] }, { "text": "另有少量脑脊液可通过脑室的室管膜上皮、蛛网膜下腔的毛细血管、脑膜的淋巴管以及颅神经出颅处的蛛网膜鞘等结构吸收。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "脑脊液" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "脑室" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "室管膜上皮" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "毛细血管" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "脑膜" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "淋巴管" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "蛛网膜鞘" } ] }, { "text": "脑脊液经上述途径不断地回到静脉中去,形成了脑脊液的循环。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "脑脊液" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "静脉" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "脑脊液" } ] }, { "text": "产生脑积水可能有三种情况:①脑脊液产生过多,这种情况极少;②脑脊液吸收发生障碍脑脊液循环发生障碍脑积水病例属于这类。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑积水" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "脑脊液" }, { "start_idx": 30, "end_idx": 38, "type": "sym", "entity": "脑脊液吸收发生障碍" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "脑脊液" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "脑脊液循环发生障碍" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "脑积水" } ] }, { "text": "【分类与病因】(一)分类临床上习惯于将脑积水分为交通性(非阻塞性)和非交通性(阻塞性)两种类型,这是根据解剖学的分类。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "脑积水" } ] }, { "text": "由于脑室系统内的循环通路阻塞引起的脑积水称为非交通性脑积水,在临床上以此型脑积水多见。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "脑室系统" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "脑积水" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "非交通性脑积水" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "脑积水" } ] }, { "text": "(二)病因脑积水的原因很多,主要为脑脊液循环通路的阻塞。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "脑积水" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "脑脊液" } ] }, { "text": "常见原因为中脑导水管阻塞、颅内肿瘤的压迫及各种原因引起的蛛网膜粘连先天性脑积水系因脑脊液循环阻塞非交通性脑积水。", "entities": [ { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "蛛网膜" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "蛛网膜粘连" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "先天性脑积水" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "脑脊液" }, { "start_idx": 41, "end_idx": 47, "type": "sym", "entity": "脑脊液循环阻塞" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "非交通性脑积水" } ] }, { "text": "1.脑室系统内的阻塞(1)先天性畸形1)室内孔闭锁。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "脑室系统" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "先天性畸形" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "室内孔闭锁" } ] }, { "text": "2)中脑导水管狭窄、分叉、胶质增生和隔膜形成中脑导水管的阻塞先天性脑积水最常见的原因。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "中脑导水管" }, { "start_idx": 2, "end_idx": 21, "type": "sym", "entity": "中脑导水管狭窄、分叉、胶质增生和隔膜形成" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "中脑导水管" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "中脑导水管的阻塞" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "先天性脑积水" } ] }, { "text": "3)Dandy-Walker畸形:由于第四脑室正中孔及侧孔先天性闭塞而引起脑积水。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "dis", "entity": "Dandy-Walker畸形" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "脑积水" } ] }, { "text": "6)其他:无脑回畸形,软骨发育不良,脑穿通畸形,第五、六脑室囊肿等,均可发生脑积水。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "无脑回畸形" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "软骨发育不良" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "脑穿通畸形" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "第五、六脑室囊肿" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "脑积水" } ] }, { "text": "2.脑室系统外的阻塞(1)出血:如蛛网膜下腔出血后引起的纤维增生。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "脑室系统" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "纤维增生" } ] }, { "text": "(2)炎症:如化脓性、结核性或其他类型脑膜炎,由于增生的纤维组织引起脑底部蛛网膜粘连脑脊液循环通路。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "炎症" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "纤维组织" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "脑底部蛛网膜" }, { "start_idx": 34, "end_idx": 41, "type": "sym", "entity": "脑底部蛛网膜粘连" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "脑脊液" } ] }, { "text": "(3)脑膜癌。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脑膜癌" } ] }, { "text": "(4)颅内手术后。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "颅内手术" } ] }, { "text": "3.脑脊液的分泌和吸收障碍(1)脑脊液产生过多脉络丛乳头状瘤。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "脑脊液" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "脑脊液" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "脑脊液产生过多" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "脉络丛乳头状瘤" } ] }, { "text": "2)维生素缺乏。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "维生素缺乏" } ] }, { "text": "(2)脑脊液吸收障碍静脉窦压力增高静脉窦血栓颈静脉血栓上腔静脉阻塞先天性蛛网膜颗粒发育不良。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "脑脊液" }, { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "脑脊液吸收障碍" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "静脉窦" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "静脉窦压力增高" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "静脉窦" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "静脉窦血栓" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "颈静脉" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "颈静脉血栓" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "上腔静脉阻塞" }, { "start_idx": 33, "end_idx": 44, "type": "dis", "entity": "先天性蛛网膜颗粒发育不良" } ] }, { "text": "【临床表现】临床症状并不一致,与脑积水病理变化出现的年龄、病情的轻重和病程的长短有关。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "脑积水" } ] }, { "text": "(一)头颅改变先天性脑积水。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "头颅" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "头颅改变" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "先天性脑积水" } ] }, { "text": "头颅进行性异常增大,与全身的发育不成比例伴囟门扩大、颅缝裂开以及颅骨变薄变软先天性脑积水的特有体征。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "头颅" }, { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "头颅进行性异常增大,与全身的发育不成比例" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "囟门" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "颅缝" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "颅骨" }, { "start_idx": 20, "end_idx": 37, "type": "sym", "entity": "伴囟门扩大、颅缝裂开以及颅骨变薄变软" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "先天性脑积水" } ] }, { "text": "婴幼儿患脑积水时行头颅透光试验可呈阳性,枕部局限性透照光圈增大提示为Dandy-Walker畸形,头颅广泛透光则见于严重脑积水。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "脑积水" }, { "start_idx": 9, "end_idx": 14, "type": "ite", "entity": "头颅透光试验" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "枕部局限性透照光圈增大" }, { "start_idx": 34, "end_idx": 47, "type": "dis", "entity": "Dandy-Walker畸形" }, { "start_idx": 49, "end_idx": 54, "type": "sym", "entity": "头颅广泛透光" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "严重脑积水" } ] }, { "text": "(二)颅内压增高的表现在婴幼儿,颅内压增高的一般症状多不明显,但可见囟门隆起,张力增高,头皮静脉怒张脑积水进展较快时,亦可出现反复呕吐。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "囟门" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "头皮静脉" }, { "start_idx": 34, "end_idx": 49, "type": "sym", "entity": "囟门隆起,张力增高,头皮静脉怒张" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "脑积水" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "反复呕吐" } ] }, { "text": "其中头痛常在卧床休息较久时加重,故常有早晨头痛而起床活动后消失的现象,可能是活动促使脑脊液通过狭窄部位所造成。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "头痛" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "脑脊液" } ] }, { "text": "当脑积水发展缓慢、脑室扩大颅内压增高较慢时,可以只表现为头痛、个性和情绪的改变,或者出现展神经麻痹而使眼球内斜颅内压增高症。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "脑积水" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "脑室" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "脑室扩大" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 28, "end_idx": 38, "type": "sym", "entity": "头痛、个性和情绪的改变" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "展神经" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "眼球" }, { "start_idx": 44, "end_idx": 54, "type": "sym", "entity": "展神经麻痹而使眼球内斜" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "颅内压增高症" } ] }, { "text": "(三)神经系统功能障碍除继发于部分颅内肿瘤者外,大多数脑积水无明显的神经系统定位体征。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经系统" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "脑积水" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "神经系统" } ] }, { "text": "但随着病情的进展,婴幼儿或儿童可出现运动功能减退等。", "entities": [ { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "运动功能减退" } ] }, { "text": "重度脑积水由于极度扩大的脑室枕角压迫枕叶皮质,或扩大的第三脑室的搏动压迫视交叉,引起视力减退,甚至失明,眼底可见原发性视神经萎缩。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "重度脑积水" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "第三脑室" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "视交叉" }, { "start_idx": 42, "end_idx": 63, "type": "sym", "entity": "视力减退,甚至失明,眼底可见原发性视神经萎缩" } ] }, { "text": "第四脑室扩大明显时,可出现小脑或脑干受累两眼上视障碍及锥体束损害等症状。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "小脑" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "脑干" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "小脑或脑干受累" }, { "start_idx": 20, "end_idx": 31, "type": "sym", "entity": "两眼上视障碍及锥体束损害" } ] }, { "text": "脑积水晚期或病情严重时,则出现生长发育障碍、智力减退、肢体痉挛性瘫痪及意识障碍等,最终往往是由于营养不良、全身衰竭及合并呼吸道感染精神不振、迟钝以及易激惹等,头部因增大过重,则头颈控制力差,一般不能坐及站立,多见于婴幼儿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑积水" }, { "start_idx": 15, "end_idx": 38, "type": "sym", "entity": "生长发育障碍、智力减退、肢体痉挛性瘫痪及意识障碍" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "营养不良" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "全身衰竭" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "呼吸道" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "合并呼吸道感染" }, { "start_idx": 65, "end_idx": 76, "type": "sym", "entity": "精神不振、迟钝以及易激惹" }, { "start_idx": 79, "end_idx": 109, "type": "sym", "entity": "头部因增大过重,则头颈控制力差,一般不能坐及站立,多见于婴幼儿" } ] }, { "text": "【诊断】婴幼儿脑积水,根据其头颅快速增大及其特有的外观形态等特征,可做出临床诊断,尚需进一步做神经影像学检查(头颅CT或MRI)予以确定诊断。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "婴幼儿脑积水" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "头颅" }, { "start_idx": 47, "end_idx": 53, "type": "pro", "entity": "神经影像学检查" }, { "start_idx": 55, "end_idx": 58, "type": "pro", "entity": "头颅CT" }, { "start_idx": 60, "end_idx": 62, "type": "pro", "entity": "MRI" } ] }, { "text": "随着儿童发病年龄的增大或者由于脑积水进展缓慢,头颅改变可能不典型,则需要根据其他临床表现,并借助有关辅助检查进行诊断。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "脑积水" } ] }, { "text": "头颅X线检查可见颅腔扩大、颅骨变薄,脑回压迹加深,颅缝分离、囟门扩大,头与面比例明显增大头颅CT和MRI无创性检查是目前最常用的方法,结果最可靠,既可明确脑积水的诊断,又可进一步了解脑积水的原因、种类、阻塞部位及脑室扩大的程度,以便选择适当的治疗措施。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "头颅X线" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "颅腔" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "颅骨" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "脑回压迹" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "颅缝" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "囟门" }, { "start_idx": 8, "end_idx": 43, "type": "sym", "entity": "颅腔扩大、颅骨变薄,脑回压迹加深,颅缝分离、囟门扩大,头与面比例明显增大" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "头颅CT" }, { "start_idx": 49, "end_idx": 51, "type": "pro", "entity": "MRI" }, { "start_idx": 77, "end_idx": 79, "type": "dis", "entity": "脑积水" }, { "start_idx": 91, "end_idx": 93, "type": "dis", "entity": "脑积水" }, { "start_idx": 106, "end_idx": 107, "type": "bod", "entity": "脑室" } ] }, { "text": "特别是头颅MRI在显示脑脊液通路的阻塞和引起阻塞的原因方面,尤其是中脑导水管和第四脑室附近的畸形如Arnold-Chiari畸形等,有着无可比拟的优越性。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "头颅MRI" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "脑脊液" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "中脑导水管" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "第四脑室" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "畸形" }, { "start_idx": 49, "end_idx": 63, "type": "dis", "entity": "Arnold-Chiari畸形" } ] }, { "text": "而头颅B超可用于胎儿脑积水的宫内诊断,为孕妇是否中止妊娠提供依据。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "头颅B超" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "胎儿脑积水" } ] }, { "text": "【治疗】无论何种原因引起的脑积水均以手术治疗为主,对有进展的脑积水更应及时采取手术治疗。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "脑积水" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "手术" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "脑积水" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "手术" } ] }, { "text": "手术治疗可以去除病因或重建脑脊液循环通路,但目前手术效果尚未达到满意的境地。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "脑脊液" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "手术" } ] }, { "text": "后天性脑积水还需同时进行原发病因的治疗。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "后天性脑积水" } ] }, { "text": "(一)手术治疗1.脑脊液分流术目的是通过重建脑脊液循环通路,以达到脑脊液分流的目的。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "手术" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "脑脊液分流术" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "脑脊液" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "脑脊液" } ] }, { "text": "按分流的终点不同,可分为颅内分流和颅外分流两种。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "颅内" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "颅外" } ] }, { "text": "近年有研究报道在神经内窥镜下行第三脑室底脚间池造瘘微创手术,是一种治疗中脑导水管狭窄性梗阻性脑积水的新方法。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "equ", "entity": "神经内窥镜" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "第三脑室" }, { "start_idx": 35, "end_idx": 48, "type": "dru", "entity": "中脑导水管狭窄性梗阻性脑积水" } ] }, { "text": "晚近香港大学神经外科专家创用脑室-上矢状窦分流术(吻合术),可避免其他分流术的缺点,交通性和非交通性脑积水病例均可采用。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dep", "entity": "神经外科" }, { "start_idx": 14, "end_idx": 23, "type": "pro", "entity": "脑室-上矢状窦分流术" }, { "start_idx": 25, "end_idx": 27, "type": "pro", "entity": "吻合术" }, { "start_idx": 35, "end_idx": 37, "type": "pro", "entity": "分流术" }, { "start_idx": 42, "end_idx": 52, "type": "dis", "entity": "交通性和非交通性脑积水" } ] }, { "text": "分流术效果除取决于手术本身外,与术前小儿脑皮质保留之厚度及有无合并其他畸形有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "分流术" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "手术" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "脑皮质" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "畸形" } ] }, { "text": "术后经常随访,也将有利于及时发现分流管有无不通畅、远端分流管是否足够长或有无继发感染等情况,以便给予相应处理。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "equ", "entity": "分流管" }, { "start_idx": 25, "end_idx": 29, "type": "equ", "entity": "远端分流管" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "继发感染" } ] }, { "text": "最近有研究发现,分流术本身造成的脑损害或术后并发症(如感染及硬脑膜下血肿等)可导致癫痫发作,2岁内行分流术者易发生,发生率高达20%~50%。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "分流术" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "感染" }, { "start_idx": 30, "end_idx": 35, "type": "pro", "entity": "硬脑膜下血肿" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "癫痫" }, { "start_idx": 50, "end_idx": 52, "type": "pro", "entity": "分流术" } ] }, { "text": "2.减少脑脊液产生的手术主要为脉络丛切除术或电灼术。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "脑脊液" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "手术" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "脉络丛切除术" }, { "start_idx": 22, "end_idx": 24, "type": "pro", "entity": "电灼术" } ] }, { "text": "3.去除病因的手术如切除颅内肿瘤及脓肿等占位性病变,恢复脑脊液循环通路。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "手术" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "脑脊液" } ] }, { "text": "至于通过手术能解除先天发育畸形所致阻塞原因者很少,如对Dandy-Walker畸形可行第四脑室正中孔切开术;对Arnold-Chiari畸形可行后颅窝及上颈段椎板切除减压术。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "手术" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "先天发育畸形" }, { "start_idx": 27, "end_idx": 40, "type": "dis", "entity": "Dandy-Walker畸形" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "第四脑室" }, { "start_idx": 47, "end_idx": 52, "type": "pro", "entity": "正中孔切开术" }, { "start_idx": 55, "end_idx": 69, "type": "dis", "entity": "Arnold-Chiari畸形" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "后颅窝" }, { "start_idx": 76, "end_idx": 80, "type": "bod", "entity": "上颈段椎板" }, { "start_idx": 81, "end_idx": 85, "type": "pro", "entity": "切除减压术" } ] }, { "text": "中脑导水管阻塞性病变除先天性隔膜外,手术常造成脑干损伤,很少采用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "中脑导水管" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "手术" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "脑干" } ] }, { "text": "(二)药物治疗目的在于暂时减少脑脊液的分泌或增加机体水分的排出(利尿),降低颅内压。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "脑脊液" } ] }, { "text": "主要使用乙酰唑胺(醋氮酰胺)25~50mg/(kg•d),通过抑制脉络膜丛上皮细胞Na+-K+ATP酶以减少脑脊液的分泌;或用脱水剂如甘露醇、利尿剂如双氢克脲噻等,以增加水分的排出。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "乙酰唑胺" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "醋氮酰胺" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "脑脊液" }, { "start_idx": 63, "end_idx": 65, "type": "dru", "entity": "脱水剂" }, { "start_idx": 67, "end_idx": 69, "type": "dru", "entity": "甘露醇" }, { "start_idx": 71, "end_idx": 73, "type": "dru", "entity": "利尿剂" }, { "start_idx": 75, "end_idx": 79, "type": "dru", "entity": "双氢克脲噻" } ] }, { "text": "对于有蛛网膜粘连地塞米松口服、肌内注射或鞘内注射治疗。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "蛛网膜" }, { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "蛛网膜粘连" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "地塞米松" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "肌内注射" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "鞘内注射" } ] }, { "text": "【预后】脑积水的预后差别较大,主要取决于治疗的及时与否和引起脑积水的病因及病变程度。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "脑积水" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "脑积水" } ] }, { "text": "如能及时手术根治阻塞的原因,则有可能达到临床痊愈;如阻塞原因难以解除,或合并其他先天畸形,则预后不良。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "手术" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "先天畸形" } ] }, { "text": "部分(约1/3)脑积水患儿的病情可以自然静止,不再发展。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "脑积水" } ] }, { "text": "脑积水常见的后遗症为大头畸形、精神发育迟滞、癫痫及失明先天性脑积水患儿,虽然有大约20%可停止发展,脑脊液的分泌和吸收趋于平衡,称为静止性脑积水,但是约半数患儿可在一年半内死亡。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑积水" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "癫痫" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "失明" }, { "start_idx": 10, "end_idx": 26, "type": "sym", "entity": "大头畸形、精神发育迟滞、癫痫及失明" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "先天性脑积水" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "脑脊液" }, { "start_idx": 66, "end_idx": 71, "type": "dis", "entity": "静止性脑积水" } ] }, { "text": "剩下约半数可以继续存活的先天性脑积水患儿,仅约15%智商接近正常,超过2/3有神经功能障碍,如共济失调、痉挛性瘫痪以及感知觉障碍等。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "先天性脑积水" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "神经功能障碍" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "共济失调" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "痉挛性瘫痪" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "感知觉障碍" } ] }, { "text": "有研究认为脑脊液的生化分析有助于判断脑积水的预后,如脑脊液中脂肪酸的浓度与颅内压增高可成比例升高,阻塞性脑积水解除后,脂肪酸浓度下降分流术后仍持续性升高,多提示预后不良。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "脑脊液" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脑积水" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "脑脊液" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "脂肪酸" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "阻塞性脑积水" }, { "start_idx": 59, "end_idx": 63, "type": "ite", "entity": "脂肪酸浓度" }, { "start_idx": 59, "end_idx": 65, "type": "sym", "entity": "脂肪酸浓度下降" }, { "start_idx": 66, "end_idx": 68, "type": "pro", "entity": "分流术" } ] }, { "text": "第四篇儿童营养和营养性疾病第一章儿童营养需要人类的健康主要受遗传和环境这两大因素影响,环境因素中营养则起到了非常重要的作用。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "营养性疾病" } ] }, { "text": "目前已有大量研究显示,在生命早期和生长期的儿童,无论是营养缺乏还是营养过剩,或者营养不均衡,都会引起疾病或影响疾病的预后,并且还与成年期的多种疾病的发生、发展,甚至与死亡率密切相关。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "营养缺乏" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "营养过剩" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "营养不均衡" } ] }, { "text": "为此,用现代科学医学技术对不同年龄阶段、不同疾病状态下的儿童建立合理的临床营养支持是促进我国儿科医学事业发展、提高我国儿童健康的重要保证,也是社会进步和家庭幸福的基础。", "entities": [ { "start_idx": 46, "end_idx": 47, "type": "dep", "entity": "儿科" } ] }, { "text": "二、术前准备肾活检是创伤性检查,其成功率及安全性直接与充分、细致的术前准备及穿刺者的经验有关,切忌掉以轻心,术前应做好下列准备工作:①详细询问病史,特别注意有无出血性疾病史;全面体检,排除出血性疾病、全身性感染和心肺疾患,控制血压。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "肾活检" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "创伤性检查" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "穿刺" }, { "start_idx": 80, "end_idx": 84, "type": "dis", "entity": "出血性疾病" }, { "start_idx": 89, "end_idx": 90, "type": "pro", "entity": "体检" }, { "start_idx": 94, "end_idx": 98, "type": "dis", "entity": "出血性疾病" }, { "start_idx": 100, "end_idx": 104, "type": "dis", "entity": "全身性感染" }, { "start_idx": 106, "end_idx": 109, "type": "dis", "entity": "心肺疾患" }, { "start_idx": 113, "end_idx": 114, "type": "ite", "entity": "血压" } ] }, { "text": "术前肌注小剂量镇静剂。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "肌注小剂量镇静剂" } ] }, { "text": "③训练患儿在俯卧位时呼气和屏气,还要训练床上排尿。", "entities": [ { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "尿" } ] }, { "text": "④测定血红蛋白、出凝血时间、KPTT、凝血酶原时间及血小板计数,血尿素氮、肌酐以及内生肌酐清除率。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "血红蛋白" }, { "start_idx": 8, "end_idx": 12, "type": "ite", "entity": "出凝血时间" }, { "start_idx": 14, "end_idx": 17, "type": "ite", "entity": "KPTT" }, { "start_idx": 19, "end_idx": 24, "type": "ite", "entity": "凝血酶原时间" }, { "start_idx": 26, "end_idx": 30, "type": "ite", "entity": "血小板计数" }, { "start_idx": 32, "end_idx": 35, "type": "ite", "entity": "血尿素氮" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "肌酐" }, { "start_idx": 41, "end_idx": 47, "type": "ite", "entity": "内生肌酐清除率" } ] }, { "text": "鉴定血型,必要时备血。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "血" } ] }, { "text": "⑤肾功能明显损害的患儿行肾活检前必须透析数次,改善一般情况。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "肾功能明显损害" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "肾活检" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "透析" } ] }, { "text": "但注意如行血透者,穿刺前24小时行无肝素透析,并复查试管法凝血时间。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "血透" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "穿刺" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "无肝素透析" }, { "start_idx": 26, "end_idx": 32, "type": "ite", "entity": "试管法凝血时间" } ] }, { "text": "第二十二章肝豆状核变性肝豆状核变性(hepatolenticulardegeneration)是一种常染色体隐性遗传病。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 18, "end_idx": 45, "type": "dis", "entity": "hepatolenticulardegeneration" }, { "start_idx": 50, "end_idx": 58, "type": "dis", "entity": "常染色体隐性遗传病" } ] }, { "text": "1912年KinnerWilson首次对本病的临床表现及病理解剖作了全面的描述,因而本病又称Wilson病(Wilsondisease,WD)。", "entities": [ { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "Wilson病" }, { "start_idx": 54, "end_idx": 66, "type": "dis", "entity": "Wilsondisease" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "WD" } ] }, { "text": "13号染色体编码的铜转运P型ATP酶的缺乏或功能异常引起的铜代谢障碍,使铜在肝脏内逐渐蓄积,当铜超过了肝脏储存能力,随之铜释放入血,引起溶血及组织中铜的沉积,造成组织、器官中毒和功能异常。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "bod", "entity": "铜转运P型ATP酶" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "铜代谢障碍" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "铜" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "肝脏" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "铜" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "肝脏" }, { "start_idx": 60, "end_idx": 60, "type": "bod", "entity": "铜" }, { "start_idx": 64, "end_idx": 64, "type": "bod", "entity": "血" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "溶血" }, { "start_idx": 74, "end_idx": 74, "type": "bod", "entity": "铜" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "组织" }, { "start_idx": 84, "end_idx": 85, "type": "bod", "entity": "器官" } ] }, { "text": "WD的实验室诊断依据包括血清铜蓝蛋白和血铜氧化酶的降低、24小时尿铜增加肝铜含量增加K-F环阳性WD的治疗包括螯合剂(青霉胺及曲恩汀)、锌剂、低铜饮食以及肝脏移植。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "WD" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "血清铜蓝蛋白" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "血铜氧化酶" }, { "start_idx": 28, "end_idx": 33, "type": "ite", "entity": "24小时尿铜" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "24小时尿铜增加" }, { "start_idx": 36, "end_idx": 39, "type": "ite", "entity": "肝铜含量" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "肝铜含量增加" }, { "start_idx": 42, "end_idx": 45, "type": "ite", "entity": "K-F环" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "K-F环阳性" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "WD" }, { "start_idx": 55, "end_idx": 57, "type": "dru", "entity": "螯合剂" }, { "start_idx": 59, "end_idx": 61, "type": "dru", "entity": "青霉胺" }, { "start_idx": 63, "end_idx": 65, "type": "dru", "entity": "曲恩汀" }, { "start_idx": 68, "end_idx": 69, "type": "dru", "entity": "锌剂" }, { "start_idx": 71, "end_idx": 74, "type": "pro", "entity": "低铜饮食" }, { "start_idx": 77, "end_idx": 80, "type": "pro", "entity": "肝脏移植" } ] }, { "text": "随着WD的分子基因机制的不断阐明,基因治疗将是最彻底的治疗手段。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "WD" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "基因治疗" } ] }, { "text": "【流行病学】WD是一影响所有人种的常染色体隐性遗传性疾病,发病率约1/30000,基因携带率约为1/90,种族与地区差别不大,而近亲结婚者患病率明显增高。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "WD" }, { "start_idx": 17, "end_idx": 27, "type": "dis", "entity": "常染色体隐性遗传性疾病" } ] }, { "text": "【发病机制】1985年Frydman等将WD基因定位于13q,Farrer又将其定位于13q14.3。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "WD基因" } ] }, { "text": "1993年WD基因被克隆,并显示能够编码大量阳离子转运P型ATP酶系列。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "WD基因" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "P型ATP酶" } ] }, { "text": "铜是人体重要的微量元素之一,通过胃肠道吸收和胆汁排出得以保持平衡。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "铜" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "胃肠道" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "胆汁" } ] }, { "text": "铜通过胃肠道吸收,并迅速在门脉系统中出现,与白蛋白及氨基酸结合。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "铜" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "胃肠道" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "门脉系统" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "白蛋白" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "氨基酸" } ] }, { "text": "放射性铜研究表明,新吸收的单剂量铜可被肝脏迅速清除,在24小时内10%的铜以铜蓝蛋白的形式出现在血浆中。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "铜" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "铜" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肝脏" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "铜" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "铜蓝蛋白" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "血浆" } ] }, { "text": "动力学研究表明,肝脏在调节铜转运至其他组织如骨骼肌和脑的过程中起关键作用,但其中的机制尚未明确。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肝脏" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "铜" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "脑" } ] }, { "text": "尽管已明确铜的沉积是由于肝细胞内的铜释放入血,而导致基底神经节特异性受损的机制至今未明。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "铜" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肝细胞" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "铜" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "血" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "基底神经节" } ] }, { "text": "肝脏是维持铜在体内平衡的核心器官,有强大的储存和分泌铜的能力。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肝脏" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "铜" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "铜" } ] }, { "text": "肝细胞是肝脏中吸收和蓄积铜的部位,并监测血浆中的铜,根据细胞内铜的浓度来调节铜分泌入胆汁。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肝细胞" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肝脏" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "铜" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "血浆" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "铜" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "细胞" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "铜" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "铜" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "胆汁" } ] }, { "text": "这种调节是通过铜转运P型ATP酶来完成的,ATP酶在肝细胞中大量合成,并局限于滑面内质网。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "bod", "entity": "铜转运P型ATP酶" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "ATP酶" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "肝细胞" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "滑面内质网" } ] }, { "text": "当肝细胞内的铜含量增加,ATP酶便从滑面内质网移入小管膜附近的囊状腔隙。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "肝细胞" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "铜" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "ATP酶" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "滑面内质网" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "小管膜" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "囊状腔隙" } ] }, { "text": "随着铜在囊状腔隙的蓄积,胞浆中铜的下降导致ATP酶的重新分布,重又进入滑面内质网,并将铜分泌入胆汁。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "铜" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "囊状腔隙" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胞浆" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "铜" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "ATP酶" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "滑面内质网" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "铜" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "胆汁" } ] }, { "text": "这个独特的翻译后调控机制,为维持细胞内铜的自身稳定及保证多余的铜能够迅速排出提供了一个快速反应系统。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "细胞" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "铜" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "铜" } ] }, { "text": "血浆铜蓝蛋白是一种单链糖蛋白,在体内含量较多,结合了血浆中95%以上的铜。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "血浆铜蓝蛋白" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "单链糖蛋白" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "血浆" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "铜" } ] }, { "text": "它由肝细胞合成并分泌,是全蛋白,可与6个原子的铜结合,并将铜转运,最终排入胆汁。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肝细胞" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "全蛋白" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "铜" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "铜" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "胆汁" } ] }, { "text": "在WD患者中,由于铜进入腔隙的转运过程受损导致血浆铜蓝蛋白的下降。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "WD" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "铜" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "腔隙" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "血浆铜蓝蛋白" } ] }, { "text": "WD的发病及原发缺陷尚未阐明,曾有多种假说:①胃肠道铜吸收增多铜蓝蛋白合成障碍细胞内异常蛋白存在胆道排铜障碍溶酶体缺陷控制基因异常WD基因突变导致铜蓝蛋白的缺失或功肝细胞内铜自身稳定的破坏,大量铜蓄积于肝脏内当达到一定程度后肝细胞受损铜进入血中造成溶血及组织中铜的蓄积导致各组织器官的中毒功能受损,出现各种临床症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "WD" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "胃肠道" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "铜" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "胃肠道铜吸收增多" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "铜蓝蛋白" }, { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "铜蓝蛋白合成障碍" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "细胞" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "异常蛋白" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "细胞内异常蛋白存在" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "胆道" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "铜" }, { "start_idx": 48, "end_idx": 53, "type": "sym", "entity": "胆道排铜障碍" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "溶酶体" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "溶酶体缺陷" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "控制基因" }, { "start_idx": 59, "end_idx": 64, "type": "sym", "entity": "控制基因异常" }, { "start_idx": 65, "end_idx": 66, "type": "dis", "entity": "WD" }, { "start_idx": 73, "end_idx": 76, "type": "bod", "entity": "铜蓝蛋白" }, { "start_idx": 73, "end_idx": 81, "type": "sym", "entity": "铜蓝蛋白的缺失或功" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "肝细胞" }, { "start_idx": 86, "end_idx": 86, "type": "bod", "entity": "铜" }, { "start_idx": 97, "end_idx": 97, "type": "bod", "entity": "铜" }, { "start_idx": 101, "end_idx": 102, "type": "bod", "entity": "肝脏" }, { "start_idx": 95, "end_idx": 103, "type": "sym", "entity": "大量铜蓄积于肝脏内" }, { "start_idx": 112, "end_idx": 114, "type": "bod", "entity": "肝细胞" }, { "start_idx": 104, "end_idx": 116, "type": "sym", "entity": "当达到一定程度后肝细胞受损" }, { "start_idx": 117, "end_idx": 117, "type": "bod", "entity": "铜" }, { "start_idx": 120, "end_idx": 120, "type": "bod", "entity": "血" }, { "start_idx": 117, "end_idx": 121, "type": "sym", "entity": "铜进入血中" }, { "start_idx": 124, "end_idx": 125, "type": "bod", "entity": "溶血" }, { "start_idx": 130, "end_idx": 130, "type": "bod", "entity": "铜" }, { "start_idx": 122, "end_idx": 133, "type": "sym", "entity": "造成溶血及组织中铜的蓄积" }, { "start_idx": 137, "end_idx": 140, "type": "bod", "entity": "组织器官" }, { "start_idx": 134, "end_idx": 143, "type": "sym", "entity": "导致各组织器官的中毒" }, { "start_idx": 144, "end_idx": 147, "type": "sym", "entity": "功能受损" } ] }, { "text": "但尚有不能解释的地方:遗传性低铜蓝蛋白血症及部分WD患者的亲属长期血清铜蓝蛋白处于低水平WD临床症状。", "entities": [ { "start_idx": 11, "end_idx": 20, "type": "dis", "entity": "遗传性低铜蓝蛋白血症" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "WD" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "血清铜蓝蛋白" }, { "start_idx": 31, "end_idx": 43, "type": "sym", "entity": "长期血清铜蓝蛋白处于低水平" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "WD" } ] }, { "text": "国内学者的研究认为溶酶体在本病的发生、发展中占重要位置,并提示铜在细胞内分布形式与细胞病变密切相关。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "溶酶体" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "铜" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "细胞" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "细胞" } ] }, { "text": "溶酶体无法容纳的过剩的铜经血液循环沉积在脑基底节、大脑、小脑、小脑齿状核、角膜后弹力层、近端肾小管及皮肤。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "溶酶体" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "铜" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "血液循环" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "脑基底节" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "大脑" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "小脑" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "小脑齿状核" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "角膜后弹力层" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "近端肾小管" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "皮肤" } ] }, { "text": "【病理】神经病理检查显示铜在基底节区(尤其是尾状核及豆状核)广泛沉着神经元丧失,基底神经节和皮质脱髓鞘变伴有广泛的神经胶质细胞增生肝损伤的所有阶段都可发生,伴有脂肪变性铜颗粒轻微炎症、Kupffer细胞增大结缔组织增生淋巴细胞浸润电镜下可见线粒体形状、大小不一,基质密度增加,内、外层膜分离和嵴间距增宽改变。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "神经病理检查" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "铜" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "基底节区" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "尾状核" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "豆状核" }, { "start_idx": 12, "end_idx": 33, "type": "sym", "entity": "铜在基底节区(尤其是尾状核及豆状核)广泛沉着" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "神经元丧失" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "基底神经节" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "皮质脱髓" }, { "start_idx": 40, "end_idx": 51, "type": "sym", "entity": "基底神经节和皮质脱髓鞘变" }, { "start_idx": 57, "end_idx": 62, "type": "bod", "entity": "神经胶质细胞" }, { "start_idx": 52, "end_idx": 64, "type": "sym", "entity": "伴有广泛的神经胶质细胞增生" }, { "start_idx": 65, "end_idx": 67, "type": "dis", "entity": "肝损伤" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "脂肪" }, { "start_idx": 80, "end_idx": 83, "type": "sym", "entity": "脂肪变性" }, { "start_idx": 84, "end_idx": 84, "type": "bod", "entity": "铜" }, { "start_idx": 84, "end_idx": 86, "type": "sym", "entity": "铜颗粒" }, { "start_idx": 87, "end_idx": 90, "type": "sym", "entity": "轻微炎症" }, { "start_idx": 92, "end_idx": 100, "type": "bod", "entity": "Kupffer细胞" }, { "start_idx": 92, "end_idx": 102, "type": "sym", "entity": "Kupffer细胞增大" }, { "start_idx": 103, "end_idx": 106, "type": "bod", "entity": "结缔组织" }, { "start_idx": 103, "end_idx": 108, "type": "sym", "entity": "结缔组织增生" }, { "start_idx": 109, "end_idx": 112, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 109, "end_idx": 114, "type": "sym", "entity": "淋巴细胞浸润" }, { "start_idx": 115, "end_idx": 129, "type": "sym", "entity": "电镜下可见线粒体形状、大小不一" }, { "start_idx": 131, "end_idx": 136, "type": "sym", "entity": "基质密度增加" }, { "start_idx": 138, "end_idx": 152, "type": "sym", "entity": "内、外层膜分离和嵴间距增宽改变" } ] }, { "text": "在肝细胞中多囊泡状的圆形颗粒被认为是WD的特征性的改变,通过X线吸收分析,显示铜含量的增加。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "肝细胞" }, { "start_idx": 5, "end_idx": 13, "type": "bod", "entity": "多囊泡状的圆形颗粒" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "WD" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "X线" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "铜含量的增加" } ] }, { "text": "【临床表现】WD的临床表现各异,大多数WD患者在疾病早期有神经精神症状肝病的表现。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "WD" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "WD" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "神经" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "神经精神症状" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "肝病" } ] }, { "text": "在儿童,患者一旦出现不明原因的肝功能异常WD的可能。", "entities": [ { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肝" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "肝功能异常" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "WD" } ] }, { "text": "(一)肝脏损害肝功能异常是儿童期WD最常见的表现,平均发病年龄在10~13岁。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "肝脏损害" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "肝功能异常" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "儿童期WD" } ] }, { "text": "肝损伤可发生在神经系统症状肝脏症状可表现为无症状仅血清转氨酶轻度增高急性肝炎、慢性肝炎以及肝硬化等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肝损伤" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "神经" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肝脏" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "血清转氨酶" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "血清转氨酶轻度增高" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "急性肝炎" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "慢性肝炎" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "肝硬化" } ] }, { "text": "在特殊情况下,首发症状可为急性肝功能衰竭伴大量铜突然释放入血引起溶血性贫血。", "entities": [ { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肝" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "急性肝功能衰竭" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "铜" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "血" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "伴大量铜突然释放入血" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "引起溶血性贫血" } ] }, { "text": "突发这种急性肝脏变性的患者常提示存在病毒性疾病或有外界因素促使铜负荷过重使肝脏受损加剧。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "急性肝脏变性" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "病毒性疾病" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "铜" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "肝脏" } ] }, { "text": "无论首发症状如何,几乎所有患者都有不同程度的肝硬化,这反映了机体对临床症状出现前的肝铜蓄积的一个应答。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "肝硬化" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "肝铜" } ] }, { "text": "(二)神经系统损害中枢神经系统损害仅次于肝脏,以基底神经节受损为主。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "神经系统损害" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "中枢神经系统损害" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肝脏" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "基底神经节" } ] }, { "text": "大年龄儿童肝损不明显,主要以精神神经症状为主,但比例较成人少。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肝损" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "精神神经症状" } ] }, { "text": "早期症状主要为面部表情减少震颤、肌张力障碍吞咽困难、构音障碍、流涎以及舞蹈样动作。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "面部" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "面部表情减少" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "震颤" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "肌" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "肌张力障碍" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "吞咽困难" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "构音障碍" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "流涎" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "舞蹈样动作" } ] }, { "text": "早期出现症状的患者中可通过MRI检测到脑结构的改变铜的沉积螯合剂的治疗可发生可逆性改变。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "MRI检测" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "脑" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "脑结构的改变" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "铜" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "铜的沉积" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "螯合剂" } ] }, { "text": "精神症状可单独发生或者与其他症状伴随出现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "精神症状" } ] }, { "text": "随病情进展出现皮层下型特征的痴呆精神迟钝和惰性,记忆力损害,注意力不集中均很显著。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "皮层" }, { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "皮层下型特征的痴呆" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "精神迟钝和惰性" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "记忆力损害" }, { "start_idx": 30, "end_idx": 39, "type": "sym", "entity": "注意力不集中均很显著" } ] }, { "text": "(三)其他除了常见的肝脏和神经系统表现外,任何器官铜的过量沉积,均可导致该脏器功能障碍。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肝脏" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "神经系统" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "铜" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "脏器" } ] }, { "text": "临床表现包括范可尼综合征伴氨基酸尿、肾结石糖尿、蛋白尿、继发性骨质密度减低、角膜色素环K-F环)、心律失常、关节炎、横纹肌溶解溶血性贫血、血小板减少、皮肤变黑甲状腺功能减低甲状旁腺功能减退继发性闭经。", "entities": [ { "start_idx": 6, "end_idx": 16, "type": "sym", "entity": "范可尼综合征伴氨基酸尿" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肾" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "肾结石" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "糖尿" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 28, "end_idx": 36, "type": "sym", "entity": "继发性骨质密度减低" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "角膜" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "角膜色素环" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "K-F环" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "心律失常" }, { "start_idx": 54, "end_idx": 56, "type": "sym", "entity": "关节炎" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "横纹肌" }, { "start_idx": 58, "end_idx": 62, "type": "sym", "entity": "横纹肌溶解" }, { "start_idx": 63, "end_idx": 67, "type": "sym", "entity": "溶血性贫血" }, { "start_idx": 69, "end_idx": 73, "type": "sym", "entity": "血小板减少" }, { "start_idx": 75, "end_idx": 76, "type": "bod", "entity": "皮肤" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "皮肤变黑" }, { "start_idx": 79, "end_idx": 81, "type": "bod", "entity": "甲状腺" }, { "start_idx": 79, "end_idx": 85, "type": "sym", "entity": "甲状腺功能减低" }, { "start_idx": 86, "end_idx": 89, "type": "bod", "entity": "甲状旁腺" }, { "start_idx": 86, "end_idx": 93, "type": "sym", "entity": "甲状旁腺功能减退" }, { "start_idx": 94, "end_idx": 98, "type": "sym", "entity": "继发性闭经" } ] }, { "text": "随着螯合剂的早期应用,这些症状有可逆性的可能。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "螯合剂" } ] }, { "text": "【诊断】(一)临床表现任何患者如仅出现血清转氨酶升高原因不明的慢性肝炎出现锥体外系症状Coombs试验阴性的溶血性贫血肾小管功能不全血尿或蛋白尿代谢性骨病无法解释的精神症状包括突然行为改变等都应想到WD的诊断。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "血清转氨酶" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "血清转氨酶升高" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "慢性肝炎" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "原因不明的慢性肝炎" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "锥" }, { "start_idx": 35, "end_idx": 42, "type": "sym", "entity": "出现锥体外系症状" }, { "start_idx": 43, "end_idx": 50, "type": "pro", "entity": "Coombs试验" }, { "start_idx": 43, "end_idx": 58, "type": "sym", "entity": "Coombs试验阴性的溶血性贫血" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "肾小管" }, { "start_idx": 59, "end_idx": 65, "type": "sym", "entity": "肾小管功能不全" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "血尿" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "蛋白尿" }, { "start_idx": 66, "end_idx": 71, "type": "sym", "entity": "血尿或蛋白尿" }, { "start_idx": 75, "end_idx": 76, "type": "dis", "entity": "骨病" }, { "start_idx": 72, "end_idx": 76, "type": "sym", "entity": "代谢性骨病" }, { "start_idx": 77, "end_idx": 85, "type": "sym", "entity": "无法解释的精神症状" }, { "start_idx": 90, "end_idx": 93, "type": "sym", "entity": "行为改变" }, { "start_idx": 99, "end_idx": 100, "type": "dis", "entity": "WD" } ] }, { "text": "本病患者肝损害和神经系统均非特异性,角膜K-F环为WD特异性体征,但早期缺乏完整的环。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "肝损害" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "神经系统" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "角膜K-F环" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "WD特异性体征" } ] }, { "text": "铜在脑内蓄积到一定程度出现症状时,在角膜上也有沉积。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "铜" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "脑" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "角膜" } ] }, { "text": "另外,K-F环也见于其他肝病患儿,因此没有K-F环不能排除WD,仅依靠K-F环不能确诊WD。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "K-F环" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肝病" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "K-F环" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "WD" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "K-F环" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "WD" } ] }, { "text": "(二)实验室检查以下实验室检查可明确WD的诊断。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "实验室检查" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "WD" } ] }, { "text": "1.血清铜蓝蛋白及铜氧化酶患者血清铜蓝蛋白降低铜氧化酶活力下降血清铜蓝蛋白均低于200mg/L(免疫扩散法)。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "血清铜蓝蛋白" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "铜氧化酶" }, { "start_idx": 15, "end_idx": 20, "type": "bod", "entity": "血清铜蓝蛋白" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "血清铜蓝蛋白降低" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "铜氧化酶" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "铜氧化酶活力下降" }, { "start_idx": 31, "end_idx": 36, "type": "bod", "entity": "血清铜蓝蛋白" }, { "start_idx": 48, "end_idx": 52, "type": "pro", "entity": "免疫扩散法" } ] }, { "text": "血清铜蓝蛋白为一急性期蛋白的反应,因此5%的患者由于感染或炎症时,血清铜蓝蛋白可在正常范围内。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "血清铜蓝蛋白" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "感染" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "炎症" }, { "start_idx": 33, "end_idx": 38, "type": "pro", "entity": "血清铜蓝蛋白" } ] }, { "text": "另外在严重肝病、营养不良、肾病及6个月以内婴儿,其值可降低。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "严重肝病" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "营养不良" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "肾病" } ] }, { "text": "患者血清铜氧化酶低于0.2光密度。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "血清铜氧化酶" } ] }, { "text": "2.血清铜和24小时尿铜患者血清总铜量降低24小时尿铜排泄量增多血清总铜量低于正常人的1/2,24h尿铜>1.6μmol/24h。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "血清铜" }, { "start_idx": 6, "end_idx": 11, "type": "ite", "entity": "24小时尿铜" }, { "start_idx": 14, "end_idx": 18, "type": "ite", "entity": "血清总铜量" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "血清总铜量降低" }, { "start_idx": 21, "end_idx": 29, "type": "ite", "entity": "24小时尿铜排泄量" }, { "start_idx": 21, "end_idx": 31, "type": "sym", "entity": "24小时尿铜排泄量增多" }, { "start_idx": 32, "end_idx": 36, "type": "ite", "entity": "血清总铜量" }, { "start_idx": 47, "end_idx": 51, "type": "ite", "entity": "24h尿铜" } ] }, { "text": "但需排除肝脏阻塞性疾病。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "肝脏阻塞性疾病" } ] }, { "text": "对可疑病例可用青霉胺,在治疗前后测24h尿铜作诊断性治疗,如口服青霉胺后24h尿铜增加至1000μg即可诊断。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "青霉胺" }, { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "24h尿铜" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "口服青霉胺" }, { "start_idx": 36, "end_idx": 40, "type": "ite", "entity": "24h尿铜" } ] }, { "text": "3.肝铜测定测定肝铜需行肝活体组织检查,如肝铜含量>250μg/g干重,具有诊断意义。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "肝铜测定" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肝铜" }, { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "肝活体组织检查" }, { "start_idx": 21, "end_idx": 24, "type": "ite", "entity": "肝铜含量" } ] }, { "text": "即使在无症状患者中肝铜含量通常也可升高。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "ite", "entity": "肝铜含量" } ] }, { "text": "肝活体组织检查对临床症状不典型、其他检查不能确诊、症状出现前的患者,以及对WD患者家族成员的筛查有诊断意义。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "肝活体组织检查" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "WD" } ] }, { "text": "铜的沉积主要在门静脉区及纤维隔处,因此在进行诊断及治疗后随访时,要考虑铜分布的显著差异。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "铜" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "门静脉区" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "纤维隔处" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "铜" } ] }, { "text": "另外,其他疾病也可导致肝铜含量增高,如慢性活动性肝炎、胆汁淤积性肝硬化及任何原因所致的长时间的肝外胆管阻塞的病人,但血浆铜蓝蛋白正常或增加可与WD相鉴别。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "ite", "entity": "肝铜含量" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "慢性活动性肝炎" }, { "start_idx": 27, "end_idx": 34, "type": "dis", "entity": "胆汁淤积性肝硬化" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "肝外胆管阻塞" }, { "start_idx": 58, "end_idx": 63, "type": "bod", "entity": "血浆铜蓝蛋白" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "WD" } ] }, { "text": "4.影像学检查MRI对WD的诊断优于CT,测定T1及T2迟缓时间能反映WD治疗结果,判断症状改善或变化。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "影像学检查" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "MRI" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "WD" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "CT" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "WD" } ] }, { "text": "MRI改变呈双侧对称一致类型。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "MRI" } ] }, { "text": "T2加权像低信号为本病较具特殊性的改变,其主要改变位于壳核及尾状核,其次为脑萎缩及皮质下白质损害,病变还见于顶盖、大脑导水管周围灰质、红核和小脑深部齿状核,且分布与某些神经体征有很好的相关性。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "壳核" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "尾状核" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "脑萎缩" }, { "start_idx": 47, "end_idx": 53, "type": "dis", "entity": "皮质下白质损害" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "顶盖" }, { "start_idx": 63, "end_idx": 71, "type": "bod", "entity": "大脑导水管周围灰质" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "红核" }, { "start_idx": 76, "end_idx": 82, "type": "bod", "entity": "小脑深部齿状核" } ] }, { "text": "5.基因诊断随着分子生物学的发展,基因诊断成为本病最可靠、最有前途的诊断方法。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "基因诊断" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "基因诊断" } ] }, { "text": "20世纪80年代应用探针确定WD的基因位点在13号染色体长臂13q14-q21,此后经连锁分析,认为本病的基因位点D13S13远端0.4cM、D13S59近端1.2cM处,并提出13q14.3区的D13S31远端0.4cM和D13S59间长约2cM的DNA区是筛选WD后选基因的最佳片段处。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "equ", "entity": "探针" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "WD" }, { "start_idx": 22, "end_idx": 38, "type": "bod", "entity": "13号染色体长臂13q14-q21" }, { "start_idx": 42, "end_idx": 46, "type": "pro", "entity": "经连锁分析" }, { "start_idx": 132, "end_idx": 133, "type": "dis", "entity": "WD" } ] }, { "text": "WD蛋白有3个功能区:第一区为金属离子结合区;第二区为功能区,WD基因突变常涉及此区;第三区为跨膜区。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "WD蛋白" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "金属离子结合区" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "功能区" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "WD基因" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "跨膜区" } ] }, { "text": "WD患者的DNA分析显示也能有500多种突变形式,且大多数患者为复杂的杂合子,可能同时携带2种不同的突变形式,使DNA研究更为复杂。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "WD" }, { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "DNA分析" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "DNA" } ] }, { "text": "另外,WD存在高度异质性,排除了大多数患者在DNA水平进行诊断的可能,但是在不同的人群中对常见的突变形式进行分析仍不失为一诊断手段。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "WD" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "DNA" } ] }, { "text": "①间接基因诊断:在有先证者的情况下,可采用多态标记连锁分析对家系中其他成员进行间接基因诊断。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "间接基因诊断" }, { "start_idx": 21, "end_idx": 28, "type": "pro", "entity": "多态标记连锁分析" }, { "start_idx": 39, "end_idx": 44, "type": "pro", "entity": "间接基因诊断" } ] }, { "text": "目前限制性片段长度多态性连锁分析(RFLP)已用于WD诊断、症状前诊断、产前诊断和携带者的检测。", "entities": [ { "start_idx": 2, "end_idx": 15, "type": "pro", "entity": "限制性片段长度多态性连锁分析" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "RFLP" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "WD诊断" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "症状前诊断" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "产前诊断" }, { "start_idx": 41, "end_idx": 46, "type": "pro", "entity": "携带者的检测" } ] }, { "text": "因此对常规检查不能确诊的患者可采用RFLP,达到早期诊断的目的。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "RFLP" } ] }, { "text": "保证RFLP准确有效的前提是家族中先有WD患者,家系中能提供组织标本成员的数量。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "RFLP" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "WD" } ] }, { "text": "②直接基因诊断:对临床可疑但家系中无先证者的患者,可直接监测ATP7B基因突变。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "直接基因诊断" }, { "start_idx": 30, "end_idx": 36, "type": "bod", "entity": "ATP7B基因" } ] }, { "text": "本病的基因位于13q14.3,我国Wilson病患者的ATP7B基因突变有3个突变热点,即R778L、P992L及T935M,占所有突变的60%左右。", "entities": [ { "start_idx": 27, "end_idx": 33, "type": "bod", "entity": "ATP7B基因" } ] }, { "text": "综上所述,结合家族史、临床表现及上述实验诊断方法可较准确地诊断WD。", "entities": [ { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "WD" } ] }, { "text": "【治疗】治疗的目的是重新维持肝内铜的自身稳定,降低组织内铜的含量,并解除铜的毒性作用。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肝内铜" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "铜" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "铜" } ] }, { "text": "(一)螯合剂青霉胺可通过与铜螯合促进WD患儿尿铜排泄增加铜在症状前患者体内的蓄积。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "螯合剂" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "青霉胺" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "铜螯合" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "WD" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "尿铜" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "尿铜排泄增加" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "铜" } ] }, { "text": "青霉胺治疗的试验性口服剂量开始一般为15~25mg/(kg•d),如果能够耐受逐渐增大剂量至40mg/(kg•d),每日分4次,饭前2小时口服,大多数患者在服药后4个月症状消失。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "青霉胺治疗" } ] }, { "text": "但是由于螯合剂治疗后可引起体内铜的再分布,肝铜入脑,累及基底神经节,从而加重神经系统症状。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "螯合剂治疗" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "铜" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肝铜" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "脑" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "基底神经节" } ] }, { "text": "因此,有报道20%有明显神经系统症状WD患儿接受青霉胺治疗后反而神经系统症状铜含量降低到一定程度(血清游离铜含量<10mg/dl,尿铜排泄<80mg/d),则给予常规治疗剂量的一半终身维持治疗。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "神经系统" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "WD" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "青霉胺治疗" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "神经系统" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 38, "end_idx": 40, "type": "ite", "entity": "铜含量" }, { "start_idx": 49, "end_idx": 55, "type": "ite", "entity": "血清游离铜含量" }, { "start_idx": 65, "end_idx": 68, "type": "ite", "entity": "尿铜排泄" } ] }, { "text": "坚持服药至关重要,因为据报道突然停用青霉胺的患者会导致病情急剧恶化。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "青霉胺" } ] }, { "text": "在应用青霉胺治疗的过程中,需定期监测尿常规和血常规,因为过敏反应如发热、皮疹、淋巴结病以及血恶液质等不良反应较为常见。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "青霉胺治疗" }, { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "尿常规" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "血常规" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "发热" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "皮疹" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "淋巴结病" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "血恶液质" } ] }, { "text": "此外,20%~25%的WD患者服用青霉胺后可出现严重的不良反应如SLE和肾病综合征。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "WD" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "青霉胺" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "SLE" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "肾病综合征" } ] }, { "text": "一旦发生上述青霉胺诱发的自身免疫性病症,需立即停药,并改用曲恩汀(化学名为二盐酸三甲烯羟化四甲胺,triethylenetetra-minedihydrochloride)。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "青霉胺" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "自身免疫性病症" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "曲恩汀" }, { "start_idx": 37, "end_idx": 47, "type": "dru", "entity": "二盐酸三甲烯羟化四甲胺" }, { "start_idx": 49, "end_idx": 84, "type": "dru", "entity": "triethylenetetra-minedihydrochloride" } ] }, { "text": "曲恩汀也是一种络合剂,可促进铜的排出,有时可作为伴有神经系统症状WD患者的一线药物,对各型患者均有效,一般剂量为40~50mg/(kg•d)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "曲恩汀" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "络合剂" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "铜" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "神经系统" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "WD" } ] }, { "text": "某些患者应用后出现血浆铁浓度下降青霉胺同时加用维生素B6,可避免维生素B6缺乏引起的癫痫发作,服用时加用左旋多巴疗效更好。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "血浆铁" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "血浆铁浓度下降" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "青霉胺" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "维生素B6" }, { "start_idx": 38, "end_idx": 42, "type": "dru", "entity": "维生素B6" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "癫痫" }, { "start_idx": 64, "end_idx": 67, "type": "dru", "entity": "左旋多巴" } ] }, { "text": "二巯基丙磺酸钠推荐用于有轻、中、重度肝损害和神经精神症状患者。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "二巯基丙磺酸钠" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "重度肝损害" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "神经精神症状" } ] }, { "text": "(二)锌剂用于WD起始治疗和维持治疗,也用于不能耐受青霉胺患者的替代治疗和螯合剂治疗导致锌缺乏的补充治疗。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "锌剂" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "WD" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "青霉胺" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "螯合剂治疗" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "锌缺乏" } ] }, { "text": "锌剂的作用机制是使WD患者肠道内金属硫因(MT)合成增加,MT对铜有高度亲和力,肠道内MT增加,阻止铜吸收入血。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "锌剂" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "WD" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肠道" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "金属硫因" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "MT" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "MT" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "铜" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "肠道" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "MT" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "铜" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "血" } ] }, { "text": "同时肠道上皮细胞更新加剧,细胞脱落,铜由粪便排出,逐渐形成一个慢性铜负平衡。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "肠道上皮细胞" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "细胞" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "铜" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "铜" } ] }, { "text": "锌剂安全有效、低毒,但起效慢。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "锌剂" } ] }, { "text": "锌剂用法:5~7.5mg/(kg•d),餐前1小时服用,与青霉胺间隔至少1小时。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dru", "entity": "锌剂" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "青霉胺" } ] }, { "text": "(三)低铜饮食在治疗初期,饮食中铜的含量应少于1mg/d,之后随着症状的控制可以增加至1.0~1.5mg/d。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "低铜饮食" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "铜" } ] }, { "text": "含铜量低的食物有精白米面、萝卜、藕、芹菜、小白菜、瘦猪肉、瘦鸡、瘦鸭、牛奶以及马铃薯等,牛奶不仅含铜量低,长期服用尚有轻度排铜作用。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "铜" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "铜" }, { "start_idx": 62, "end_idx": 62, "type": "bod", "entity": "铜" } ] }, { "text": "相反,铜含量高的食物有肥猪肉、动物内脏和小牛肉等肉类;蟹、虾、鱼和贝类等,黄豆、青豆和扁豆等豆类;花生、芝麻和胡桃等坚果类;蘑菇、牡蛎、蜈蚣以及全蝎等。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "铜" } ] }, { "text": "这些含铜量高的食物应禁止食用。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "铜" } ] }, { "text": "(四)肝移植患有进行肝衰竭、螯合剂治疗无效或由暴发性肝炎引起的急性肝衰竭患者,可考虑肝脏移植。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "肝移植" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "进行肝衰竭" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "螯合剂治疗" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "暴发性肝炎" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "急性肝衰竭" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "肝脏移植" } ] }, { "text": "肝脏移植可以在6个月内使铜的自身稳定达到正常水平,并能持续改善临床症状,包括神经和精神性疾病。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "肝脏移植" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "铜" }, { "start_idx": 38, "end_idx": 45, "type": "dis", "entity": "神经和精神性疾病" } ] }, { "text": "由于肝细胞增殖能力有限,很难长时间维持其功能,同时存在的免疫排斥反应,尚需进一步研究。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "肝细胞" } ] }, { "text": "(五)基因治疗随着WD基因研究的进一步深入,基因治疗将为WD病的治疗开拓新的领域。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "基因治疗" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "WD基因" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "WD" } ] }, { "text": "然而,想通过此方法有效治疗WD病,尚需对基因产物的特征有更多的了解,尚需有更有效的方法将基因导入所有肝细胞内。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "WD" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "肝细胞" } ] }, { "text": "中华医学会神经病学分会帕金森病及运动障碍学组及中华医学会神经病学分会神经遗传病学组肝豆状核变性的诊断与治疗指南(2008年):1.推荐症状前患者的治疗以及治疗有效患者的维持治疗,可用络合剂和锌剂。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "神经病" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "帕金森病" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "运动障碍" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "神经病" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 91, "end_idx": 93, "type": "dru", "entity": "络合剂" }, { "start_idx": 95, "end_idx": 96, "type": "dru", "entity": "锌剂" } ] }, { "text": "2.药物治疗的监测开始用药后应检查肝肾功能、24小时尿铜以及血尿常规等,前3个月每月复查一次,病情稳定后3个月检查一次。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "肝肾功能" }, { "start_idx": 22, "end_idx": 27, "type": "ite", "entity": "24小时尿铜" }, { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "血尿常规" } ] }, { "text": "每3~6个月检查一次肝脾B超。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "肝脾B超" } ] }, { "text": "3.除严重肢体痉挛、畸形、严重构音障碍的脑型患者及对青霉胺过敏的患者慎用或不用外,其他类型患者均适用青霉胺。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "严重肢体痉挛" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "畸形" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "严重构音障碍" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "青霉胺" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "青霉胺" } ] }, { "text": "由于青霉胺疗效肯定、药源充足、价格低廉以及使用方便,目前在我国仍作为治疗本病的主要药物。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "青霉胺" } ] }, { "text": "4.对症治疗①静止性且幅度较小的震颤首选苯海索1mg,每日2次,如症状缓解不明显,可加用复方多巴类制剂。", "entities": [ { "start_idx": 7, "end_idx": 17, "type": "sym", "entity": "静止性且幅度较小的震颤" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "苯海索" }, { "start_idx": 44, "end_idx": 50, "type": "dru", "entity": "复方多巴类制剂" } ] }, { "text": "意向性震颤且粗大震颤首选氯硝西泮0.5mg,每日1次或2次。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "意向性震颤且粗大震颤" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "氯硝西泮" } ] }, { "text": "合并严重肌张力增高者可选用氯氮平或奥氮平。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "严重肌张力增高" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "氯氮平" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "奥氮平" } ] }, { "text": "⑥白细胞和血小板减少白细胞药物,仍不能纠正时应减用或停用青霉胺,改用其他驱铜药物。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "白细胞" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "血小板" }, { "start_idx": 1, "end_idx": 9, "type": "sym", "entity": "白细胞和血小板减少" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "白细胞" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "青霉胺" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "铜" } ] }, { "text": "如仍无效,可施行脾切除术。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "脾切除术" } ] }, { "text": "⑦暴发性肝功能衰竭铜(血液透析以及新鲜冰冻血浆进行血浆置换),尽快给予肝脏移植手术。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "肝功能" }, { "start_idx": 1, "end_idx": 8, "type": "sym", "entity": "暴发性肝功能衰竭" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "铜" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "血液透析" }, { "start_idx": 17, "end_idx": 22, "type": "dru", "entity": "新鲜冰冻血浆" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "血浆置换" }, { "start_idx": 35, "end_idx": 40, "type": "pro", "entity": "肝脏移植手术" } ] }, { "text": "第十五章中枢神经系统慢感染中枢神经系统慢感染(slowinfectionsofthecentralnervoussystem)潜伏期很长(一般1年以上),起病后持续发展,缺乏有效治疗手段,预后不良。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "dis", "entity": "中枢神经系统慢感染" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "中枢神经系统慢感染" }, { "start_idx": 23, "end_idx": 61, "type": "dis", "entity": "slowinfectionsofthecentralnervoussystem" } ] }, { "text": "中枢神经系统慢感染可分为两类:①慢病毒感染(slowvirusinfection),由传统的病毒引起;②非传统病毒引起的慢感染(slowinfectionswithunconventionalviruses),病原体很小,但与一般病毒的结构不同,其所引起的疾病统称为可传播性海绵样脑病(transmissiblespongiformencephalopathies),又称为朊蛋白病(priondiseases),包括疯牛(羊)病和人类克-雅病(Creutzfeldt-Jakobdisease,CJD)及库鲁(Kuru)病。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "中枢神经系统慢感染" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "慢病毒感染" }, { "start_idx": 22, "end_idx": 39, "type": "dis", "entity": "slowvirusinfection" }, { "start_idx": 46, "end_idx": 47, "type": "mic", "entity": "病毒" }, { "start_idx": 52, "end_idx": 62, "type": "dis", "entity": "非传统病毒引起的慢感染" }, { "start_idx": 64, "end_idx": 102, "type": "dis", "entity": "slowinfectionswithunconventionalviruses" }, { "start_idx": 105, "end_idx": 107, "type": "mic", "entity": "病原体" }, { "start_idx": 115, "end_idx": 116, "type": "mic", "entity": "病毒" }, { "start_idx": 133, "end_idx": 141, "type": "dis", "entity": "可传播性海绵样脑病" }, { "start_idx": 143, "end_idx": 181, "type": "dis", "entity": "transmissiblespongiformencephalopathies" }, { "start_idx": 187, "end_idx": 190, "type": "dis", "entity": "朊蛋白病" }, { "start_idx": 192, "end_idx": 204, "type": "dis", "entity": "priondiseases" }, { "start_idx": 209, "end_idx": 214, "type": "dis", "entity": "疯牛(羊)病" }, { "start_idx": 216, "end_idx": 221, "type": "dis", "entity": "人类克-雅病" }, { "start_idx": 223, "end_idx": 246, "type": "dis", "entity": "Creutzfeldt-Jakobdisease" }, { "start_idx": 248, "end_idx": 250, "type": "dis", "entity": "CJD" }, { "start_idx": 253, "end_idx": 261, "type": "dis", "entity": "库鲁(Kuru)病" } ] }, { "text": "本组疾病罕见,共同特点包括:①初始病毒或朊蛋白感染,可有典型表现,如麻疹,也可为隐匿性感染;②在一段较长(通常1年以上)的无症状的潜伏期之后再次出现新的症状,通常仅表现为脑病;③感染的影响局限于神经系统发病后病情持续加重,一般经数月至数年最终导致死亡。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "病毒" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "朊蛋白感染" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "麻疹" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "隐匿性感染" }, { "start_idx": 85, "end_idx": 86, "type": "dis", "entity": "脑病" }, { "start_idx": 97, "end_idx": 100, "type": "bod", "entity": "神经系统" }, { "start_idx": 89, "end_idx": 100, "type": "sym", "entity": "感染的影响局限于神经系统" }, { "start_idx": 101, "end_idx": 124, "type": "sym", "entity": "发病后病情持续加重,一般经数月至数年最终导致死亡" } ] }, { "text": "第一节慢病毒感染慢病毒疾病系指一组由普通病毒引起的以慢性进行性脑病为主要表现的综合征,是神经系统慢性持续性病毒感染的结果。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "慢病毒感染" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "慢病毒疾病" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "病毒" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "慢性进行性脑病" }, { "start_idx": 44, "end_idx": 56, "type": "dis", "entity": "神经系统慢性持续性病毒感染" } ] }, { "text": "包括:①亚急性硬化性全脑炎(subacutesclerosingpanencephalitis,SSPE);②进行性风疹全脑炎(progressiverubellapanencephalitis);③进行性多灶性白质脑炎(progressivemultifocalleukoencephalitis);④直接逆转录病毒脑病(directretrovirusencephalopathy)。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "dis", "entity": "亚急性硬化性全脑炎" }, { "start_idx": 14, "end_idx": 46, "type": "dis", "entity": "subacutesclerosingpanencephalitis" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "SSPE" }, { "start_idx": 55, "end_idx": 62, "type": "dis", "entity": "进行性风疹全脑炎" }, { "start_idx": 64, "end_idx": 96, "type": "dis", "entity": "progressiverubellapanencephalitis" }, { "start_idx": 100, "end_idx": 109, "type": "dis", "entity": "进行性多灶性白质脑炎" }, { "start_idx": 111, "end_idx": 148, "type": "dis", "entity": "progressivemultifocalleukoencephalitis" }, { "start_idx": 152, "end_idx": 160, "type": "dis", "entity": "直接逆转录病毒脑病" }, { "start_idx": 162, "end_idx": 191, "type": "dis", "entity": "directretrovirusencephalopathy" } ] }, { "text": "以下主要介绍SSPE。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "SSPE" } ] }, { "text": "【病因和发病机制】SSPE是由麻疹病毒引起的慢病毒感染,是波及全脑的炎症性变性病。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "SSPE" }, { "start_idx": 15, "end_idx": 18, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "慢病毒感染" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "脑" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "炎症性变性病" } ] }, { "text": "有人认为病毒突变是一个重要原因;也有人认为SSPE病人有轻微的免疫缺陷,例如婴儿期患麻疹后发生SSPE的危险性增加,提示免疫功能不成熟可能与之有关。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "mic", "entity": "病毒" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "SSPE" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "麻疹" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "SSPE" } ] }, { "text": "SSPE病人脑中并未找到完整的麻疹病毒颗粒,也未见到其基质蛋白(Mprotein),但是麻疹病毒编码各种蛋白质所需的遗传物质全都存在,且是功能完整的。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "SSPE" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "脑" }, { "start_idx": 15, "end_idx": 18, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "基质蛋白" }, { "start_idx": 32, "end_idx": 39, "type": "bod", "entity": "Mprotein" }, { "start_idx": 44, "end_idx": 47, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "蛋白质" } ] }, { "text": "对此,有的研究提示,基质蛋白虽然已被编码,但由于有突变,故不能与病毒粒子结合,导致不完整麻疹病毒的产生和蓄积,因此病人的抗体和细胞免疫均不能将其清除。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "基质蛋白" }, { "start_idx": 44, "end_idx": 47, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "抗体" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "细胞" } ] }, { "text": "本病神经系统症状大多出现于麻疹病毒感染后7~11年,平均8年。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 13, "end_idx": 16, "type": "mic", "entity": "麻疹病毒" } ] }, { "text": "根据其典型的临床表现,本病可分为4期:第1期(早期)典型表现包括行为改变、嗜睡、疲倦、学校适应困难、非频发性癫痫发作、多动以及性格变化等。", "entities": [ { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "行为改变" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "嗜睡" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "疲倦" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "学校适应困难" }, { "start_idx": 50, "end_idx": 57, "type": "sym", "entity": "非频发性癫痫发作" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "多动" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "性格变化" } ] }, { "text": "通过神经功能不全量表(neurologicdisabilityscale)定量研究发现,此期神经功能下降水平不超过30%。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "equ", "entity": "神经功能不全量表" }, { "start_idx": 11, "end_idx": 35, "type": "equ", "entity": "neurologicdisabilityscale" }, { "start_idx": 46, "end_idx": 49, "type": "ite", "entity": "神经功能" } ] }, { "text": "不同病人此期进展速度各异,取决于灰质脑炎的严重程度以及病变向皮层下发展的快慢。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "灰质脑炎" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "皮层" } ] }, { "text": "当大脑皮层灰质病变恶化并开始向下波及皮层下白质和深部灰质时,肌阵挛逐渐明显,即进展到本病的第2期。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "大脑皮层灰质病变" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "皮层" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "白质" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "灰质" }, { "start_idx": 30, "end_idx": 32, "type": "sym", "entity": "肌阵挛" } ] }, { "text": "第2期肌阵挛是本期的特征性表现,常随病程发展渐渐发生,并逐渐累及全身所有肌群,特别是躯干轴部肌群。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "sym", "entity": "肌阵挛" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肌群" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "躯干轴部肌群" } ] }, { "text": "肌阵挛的特点包括弥漫性、重复性和频发性,大多为对称性出现,常有相对固定的间隔,全身性肌阵挛一般每5~10秒发生一次。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "肌阵挛" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "全身性肌阵挛" } ] }, { "text": "其发生是锥体外系广泛的刺激性病变所致,而非大脑皮层神经元异常放电所致的癫痫发作。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "刺激性病变" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "大脑皮层神经元" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "癫痫" } ] }, { "text": "除了有明显的不自主运动外,出现了中枢神经运动或感觉长束受累癫痫和痴呆也进一步恶化。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "ite", "entity": "中枢神经运动" }, { "start_idx": 16, "end_idx": 28, "type": "sym", "entity": "中枢神经运动或感觉长束受累" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "癫痫" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "痴呆" } ] }, { "text": "第3期开始于病变进展累及皮层下灰质核团和脑干以后,以进行性智力和运动衰退为标志。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "皮层" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "灰质核团" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "脑干" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "进行性智力" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "运动衰退" } ] }, { "text": "代表锥体外系刺激性病变的肌阵挛消失。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "锥体外系刺激性病变" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "肌阵挛" } ] }, { "text": "第4期由于大脑功能丧失脑干、脊髓上段的广泛受累出现严重的自主神经功能异常全身重度弛缓或强直以及自主神经功能衰竭死亡。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "大脑" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "大脑功能丧失" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脑干" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "脊髓上段" }, { "start_idx": 11, "end_idx": 22, "type": "sym", "entity": "脑干、脊髓上段的广泛受累" }, { "start_idx": 28, "end_idx": 33, "type": "ite", "entity": "自主神经功能" }, { "start_idx": 23, "end_idx": 35, "type": "sym", "entity": "出现严重的自主神经功能异常" }, { "start_idx": 36, "end_idx": 44, "type": "sym", "entity": "全身重度弛缓或强直" }, { "start_idx": 47, "end_idx": 52, "type": "ite", "entity": "自主神经功能" }, { "start_idx": 47, "end_idx": 54, "type": "sym", "entity": "自主神经功能衰竭" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "死亡" } ] }, { "text": "SSPE病人发病后大多按上述4期顺序发展,每期持续数月,于2~4年左右死亡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "SSPE" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "死亡" } ] }, { "text": "但有些病例病情进展十分迅速,很快导致死亡,上述各期难以准确区分。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "死亡" } ] }, { "text": "而另一些病人进展很慢,至死亡时尚未进展至第4期。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "死亡" } ] }, { "text": "此外,在SSPE的任何一期都有可能出现病情相对静止,或呈波动性病程。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "SSPE" } ] }, { "text": "分析各期神经功能丧失的程度发现,SSPE的病期和神经功能丧失的程度之间存在非常密切的相关性。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "神经功能丧失" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "SSPE" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "神经功能丧失" } ] }, { "text": "如果将所有4期神经功能丧失的总量定为100%,则在1~4期中神经功能丧失的构成比例依次为0%~30%,31%~55%,55%~80%,81%~100%。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "神经功能丧失" }, { "start_idx": 30, "end_idx": 35, "type": "sym", "entity": "神经功能丧失" } ] }, { "text": "典型SSPE每一病期可持续几个月。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "SSPE" } ] }, { "text": "由于存在较大的个体差异,有人建议根据病情进展的速度将其分为3型:①急性型:发病后3个月以内神经功能至少丧失66%,在6个月以内出现明确的4期和死亡,或神经功能丧失90%以上;②亚急性型:在9个月以内神经功能丧失至少66%,有典型分期;③慢性型:发病9个月以后神经功能丧失不足66%,没有典型的临床分期。", "entities": [ { "start_idx": 45, "end_idx": 48, "type": "ite", "entity": "神经功能" }, { "start_idx": 71, "end_idx": 72, "type": "sym", "entity": "死亡" }, { "start_idx": 75, "end_idx": 80, "type": "sym", "entity": "神经功能丧失" }, { "start_idx": 99, "end_idx": 104, "type": "sym", "entity": "神经功能丧失" }, { "start_idx": 129, "end_idx": 134, "type": "sym", "entity": "神经功能丧失" } ] }, { "text": "慢性型病人的肌阵挛或第2期的其他症状可能大大延续。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "肌阵挛" } ] }, { "text": "【实验室检查与诊断】目前对SSPE的诊断有赖于对病人临床特点和实验室资料的综合分析。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "SSPE" } ] }, { "text": "过去曾提出以下5点作为SSPE的诊断线索:①典型的临床表现;②特征性脑电图式样;③经脑活检或尸解证实的典型组织学改变;④脑脊液球蛋白增高总蛋白量的20%;⑤血清和脑脊液麻疹病毒抗体滴度升高血清中>1∶24,脑脊液中>1∶8(补体结合抗体)。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "SSPE" }, { "start_idx": 34, "end_idx": 36, "type": "pro", "entity": "脑电图" }, { "start_idx": 42, "end_idx": 44, "type": "pro", "entity": "脑活检" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "尸解" }, { "start_idx": 60, "end_idx": 65, "type": "bod", "entity": "脑脊液球蛋白" }, { "start_idx": 60, "end_idx": 67, "type": "sym", "entity": "脑脊液球蛋白增高" }, { "start_idx": 68, "end_idx": 71, "type": "ite", "entity": "总蛋白量" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "血清" }, { "start_idx": 81, "end_idx": 91, "type": "ite", "entity": "脑脊液麻疹病毒抗体滴度" }, { "start_idx": 78, "end_idx": 93, "type": "sym", "entity": "血清和脑脊液麻疹病毒抗体滴度升高" }, { "start_idx": 94, "end_idx": 95, "type": "bod", "entity": "血清" }, { "start_idx": 103, "end_idx": 105, "type": "bod", "entity": "脑脊液" }, { "start_idx": 112, "end_idx": 113, "type": "bod", "entity": "补体" }, { "start_idx": 116, "end_idx": 117, "type": "bod", "entity": "抗体" } ] }, { "text": "CT和MRI技术的发展为本病诊断提供了无创性方法,且可作动态观察,可作为诊断的重要参考。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "CT" }, { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "MRI" } ] }, { "text": "脑脊液麻疹抗体检测迄今仍是诊断本病的特异性方法,因为已经证实脑脊液麻疹病毒抗体只能在中枢神经系统合成。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "pro", "entity": "脑脊液麻疹抗体检测" }, { "start_idx": 30, "end_idx": 38, "type": "bod", "entity": "脑脊液麻疹病毒抗体" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "综上所述,目前对SSPE的诊断只要具备相应的临床表现(不一定十分典型)以及脑脊液麻疹抗体升高两项条件即可建立诊断,如果还具备一些支持条件如麻疹病史或接种史、典型分期、脑电图异常脑脊液球蛋白升高异丙肌苷(isoprinosine)可能增加病人存活时间,对改善某些症状有所益处。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "SSPE" }, { "start_idx": 37, "end_idx": 43, "type": "bod", "entity": "脑脊液麻疹抗体" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "麻疹病" }, { "start_idx": 83, "end_idx": 85, "type": "pro", "entity": "脑电图" }, { "start_idx": 83, "end_idx": 87, "type": "sym", "entity": "脑电图异常" }, { "start_idx": 88, "end_idx": 93, "type": "bod", "entity": "脑脊液球蛋白" }, { "start_idx": 88, "end_idx": 95, "type": "sym", "entity": "脑脊液球蛋白升高" }, { "start_idx": 96, "end_idx": 99, "type": "dru", "entity": "异丙肌苷" }, { "start_idx": 101, "end_idx": 112, "type": "dru", "entity": "isoprinosine" } ] }, { "text": "2.干扰素鞘内注射或静脉注射,可延缓病情进展速度。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "干扰素" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "鞘内注射" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "静脉注射" } ] }, { "text": "近有个例报告表明,鞘内注射IFN-α可能改善临床症状和MRI异常。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "pro", "entity": "鞘内注射IFN-α" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "MRI" } ] }, { "text": "3.对症治疗包括止惊、防治感染、理疗及护理等,可减少并发症,延缓死亡,改善病人及家庭的生活质量。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "对症治疗" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "止惊" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "防治感染" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "理疗" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "护理" } ] }, { "text": "六、氢呼气技术口服乳果糖2.0g/kg,收集呼气标本,测定氢浓度,确定收集到的呼出气体中氢气量突然增加的时间,该时间即为乳果糖到达盲肠的时间——小肠传递时间。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "氢呼气技术" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "乳果糖" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "盲肠" }, { "start_idx": 72, "end_idx": 73, "type": "bod", "entity": "小肠" } ] }, { "text": "由于该检查受较多因素影响,因此,用于判定小肠传递时间仅限于成人。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "小肠" } ] }, { "text": "在小儿较多应用于诊断乳糖酶缺乏和碳水化合物吸收不良。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "乳糖酶缺乏" }, { "start_idx": 16, "end_idx": 24, "type": "dis", "entity": "碳水化合物吸收不良" } ] }, { "text": "六、肾移植后的并发症儿童肾移植后除了急慢性排斥反应外,另一些并发症,包括感染、高血压、高血脂、生长障碍以及恶性肿瘤等与肾病复发,都不同程度地影响着移植的预后。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肾移植" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "急慢性排斥反应" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "感染" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "高血压" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "高血脂" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "生长障碍" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "肾病" }, { "start_idx": 73, "end_idx": 74, "type": "pro", "entity": "移植" } ] }, { "text": "(一)感染儿童肾移植后的感染包括:细菌、真菌、病毒以及原虫等,多发生于尿路、肺部、伤口、动静脉瘘及中枢神经系统,其诱因包括导尿管的持续放置、大剂量激素的应用、粒细胞缺乏、肾周脓肿、尿瘘及糖尿病等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "感染" }, { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "感染" }, { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "细菌" }, { "start_idx": 20, "end_idx": 21, "type": "mic", "entity": "真菌" }, { "start_idx": 23, "end_idx": 24, "type": "mic", "entity": "病毒" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "尿路" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "肺部" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "伤口" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "动静脉瘘" }, { "start_idx": 49, "end_idx": 54, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 61, "end_idx": 63, "type": "equ", "entity": "导尿管" }, { "start_idx": 73, "end_idx": 74, "type": "dru", "entity": "激素" }, { "start_idx": 79, "end_idx": 83, "type": "sym", "entity": "粒细胞缺乏" }, { "start_idx": 85, "end_idx": 88, "type": "sym", "entity": "肾周脓肿" }, { "start_idx": 90, "end_idx": 91, "type": "sym", "entity": "尿瘘" }, { "start_idx": 93, "end_idx": 95, "type": "sym", "entity": "糖尿病" } ] }, { "text": "细菌感染一旦发展为败血症,死亡率为35%~75%,是移植后最常见的死亡原因。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "细菌感染" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "败血症" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "移植" } ] }, { "text": "病毒感染中以疱疹病毒族多见,CMV感染率占8%,也越来越多的引起重视,水痘感染往往是致死性。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "病毒感染" }, { "start_idx": 6, "end_idx": 9, "type": "mic", "entity": "疱疹病毒" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "水痘" } ] }, { "text": "(二)肾病复发常见于局灶节段型肾小球肾炎、IgA肾病、系膜增殖性肾炎、草酸盐沉积症以及溶血尿毒综合征;往往会发生急性肾小管坏死及肾功能减退,但长期随访结果移植肾存活率并不受复发影响。", "entities": [ { "start_idx": 10, "end_idx": 19, "type": "dis", "entity": "局灶节段型肾小球肾炎" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "系膜增殖性肾炎" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "草酸盐沉积症" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "溶血尿毒综合征" }, { "start_idx": 56, "end_idx": 62, "type": "sym", "entity": "急性肾小管坏死" }, { "start_idx": 64, "end_idx": 68, "type": "sym", "entity": "肾功能减退" }, { "start_idx": 79, "end_idx": 79, "type": "bod", "entity": "肾" } ] }, { "text": "二次肾移植复发率略高于首次肾移植,首次肾移植复发后进行二次肾移植有75%~80%复发。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "肾移植" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "肾移植" }, { "start_idx": 19, "end_idx": 21, "type": "pro", "entity": "肾移植" }, { "start_idx": 29, "end_idx": 31, "type": "pro", "entity": "肾移植" } ] }, { "text": "对复发的处理主张血浆置换与免疫抑制剂(CsA、CTX和MTX)联合应用可改善一些肾移植后复发的重症病例的存活率。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "血浆置换" }, { "start_idx": 13, "end_idx": 17, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "CsA" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "CTX" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "MTX" }, { "start_idx": 40, "end_idx": 42, "type": "pro", "entity": "肾移植" } ] }, { "text": "(三)高血压、高血脂及生长迟缓严重影响肾移植患儿的长期生活质量。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "高血压" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "高血脂" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "生长迟缓" }, { "start_idx": 19, "end_idx": 21, "type": "pro", "entity": "肾移植" } ] }, { "text": "高血压常见于术后早期,与继发性肾动脉狭窄、大量糖皮质激素应用及排斥反应有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "高血压" }, { "start_idx": 12, "end_idx": 19, "type": "sym", "entity": "继发性肾动脉狭窄" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "排斥反应" } ] }, { "text": "另有人提出成人供肾在小儿体内灌注不足造成肾素分泌增加而形成移植后高血压。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肾" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肾素" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "移植" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "高血压" } ] }, { "text": "当高血压超过明显时必须使用抗高血压药物,ACEI类以及血管紧张素受体拮抗剂已作为首选控制血压的药物。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "高血压" }, { "start_idx": 13, "end_idx": 18, "type": "dru", "entity": "抗高血压药物" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "ACEI类" }, { "start_idx": 27, "end_idx": 36, "type": "dru", "entity": "血管紧张素受体拮抗剂" }, { "start_idx": 44, "end_idx": 45, "type": "ite", "entity": "血压" } ] }, { "text": "高血脂与高血压成为心血管并发症的重要危险因素。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "高血脂" }, { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "高血压" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "心血管" } ] }, { "text": "通过饮食控制及降脂药物的应用控制血脂成为决定肾移植预后的又一因素。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "降脂药物" }, { "start_idx": 22, "end_idx": 24, "type": "pro", "entity": "肾移植" } ] }, { "text": "国外对生长迟缓的肾移植患儿应用了人类重组生长激素rhGH进行治疗取得了良好效果,尤其在一些移植肾功能低下的患儿,必须应用rhGH才能使生长迟缓得以改善。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "生长迟缓" }, { "start_idx": 8, "end_idx": 10, "type": "pro", "entity": "肾移植" }, { "start_idx": 16, "end_idx": 23, "type": "dru", "entity": "人类重组生长激素" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "rhGH" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "移植" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "肾" }, { "start_idx": 60, "end_idx": 63, "type": "dru", "entity": "rhGH" } ] }, { "text": "但长期rhGH的应用是导致排斥反应的危险因素。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "rhGH" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "排斥反应" } ] }, { "text": "(四)恶性肿瘤儿童肾移植可能并发恶性肿瘤,其中主要是恶性淋巴瘤与皮肤癌,这可能与免疫抑制剂应用导致的免疫紊乱有关。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "恶性淋巴瘤" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "皮肤癌" }, { "start_idx": 40, "end_idx": 44, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "免疫紊乱" } ] }, { "text": "德国报道儿童肾移植有2.6%发生恶性肿瘤。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "儿童肾移植有着良好的前景,在我国的台湾、香港地区已广泛开展,而内地受经济、社会等因素制约尚未广泛开展,某些医疗单位已开始着手儿童肾移植的临床实践与基础研究,积累经验,通过多学科的协作逐步使之得到广泛开展。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "儿童肾移植" }, { "start_idx": 62, "end_idx": 66, "type": "pro", "entity": "儿童肾移植" } ] }, { "text": "四、先天性血小板功能异常性疾病的治疗先天性血小板功能异常性疾病至今仍缺乏特异性治疗药物及措施,但注意护理避免外伤,病儿有不同程度出血时,恰当地给予处理是必要的。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "先天性血小板功能异常性疾病" }, { "start_idx": 18, "end_idx": 30, "type": "dis", "entity": "先天性血小板功能异常性疾病" } ] }, { "text": "(二)局部出血不重时,多可用明胶海绵、凝血酶等压迫止血,青春期月经过多时可采用避孕药如复方炔诺酮以控制月经量。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "凝血酶" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "避孕药" }, { "start_idx": 43, "end_idx": 47, "type": "dru", "entity": "复方炔诺酮" } ] }, { "text": "(三)1-脱氧-8-精氨酸加压素(DDAVP)本药可提高血浆凝血因子Ⅷ活性和抗利尿作用,但对储存池的病人使用亦有效。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "血浆" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "抗利尿" } ] }, { "text": "DDAVP0.2~0.3μg/kg皮下注射或溶于20ml生理盐水中缓慢静注,可使60%~70%的储存池病病人改善临床出血症状,出血时间缩短或恢复正常,但对BSS及GT病人常无效。", "entities": [ { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "生理盐水" } ] }, { "text": "(四)严重出血需输注血小板浓缩制剂,反复输注易产生抗小板抗体而失效,因此有条件者宜做ABO及HLA配型,给予去白细胞的同型血小板制剂,不易引起同种免疫。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dru", "entity": "血小板浓缩制剂" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "血小板" } ] }, { "text": "对于已产生抗血小板抗体的病人,可使用血浆交换以减少抗体后再输同型血小板制剂,有时可静脉给予丙种球蛋白亦有帮助。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "抗血小板抗体" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "血浆" }, { "start_idx": 30, "end_idx": 36, "type": "dru", "entity": "同型血小板制剂" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "静脉" }, { "start_idx": 45, "end_idx": 49, "type": "dru", "entity": "丙种球蛋白" } ] }, { "text": "(五)骨髓移植或基因治疗严重的病儿如能找到合适的供体可进行异基因骨髓移植、脐血干细胞移植,一旦成功即可根治。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "骨髓移植" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "基因治疗" }, { "start_idx": 29, "end_idx": 35, "type": "pro", "entity": "异基因骨髓移植" }, { "start_idx": 37, "end_idx": 43, "type": "pro", "entity": "脐血干细胞移植" } ] }, { "text": "二、发病机制环境因素在成人肿瘤的发病中起有重要作用,这些后天的环境因素包括个人生活习惯、环境污染、特殊感染等。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "成人肿瘤" } ] }, { "text": "在儿童肿瘤的发病机制中涉及更多的先天性因素即基因因素。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "1.胚胎期已由于某些打击存在的DNA多发性突变这些突变并非一定来自亲代,在这些出生时已存在的突变基础上,再次遭受打击时(如病毒感染)引起再次基因突变时(双突变理论)肿瘤的发病机会明显增高,导致临床上低年龄发病,但家族性发病的病例并不多见,可将此情况称为遗传性(或先天性)肿瘤发病倾向。", "entities": [ { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "DNA多发性突变" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "病毒感染" }, { "start_idx": 82, "end_idx": 83, "type": "dis", "entity": "肿瘤" }, { "start_idx": 126, "end_idx": 136, "type": "dis", "entity": "遗传性(或先天性)肿瘤" } ] }, { "text": "2.遗传因素致家族性发病倾向由亲代遗传获得某一致病基因,并由此导致肿瘤的形成。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "致病基因" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "肿瘤" } ] }, { "text": "这一因素在各种肿瘤中的比例不同,如40%视网膜母细胞瘤病例有遗传因素,而在白血病中仅2.5%病例可能有遗传因素参与。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "肿瘤" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "视网膜母细胞瘤" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "白血病" } ] }, { "text": "药物性免疫性溶血性贫血药物性免疫性溶血性贫血(druginducedimmunehemolyticanemia,DIIHA)的临床特征为:①大多为急性溶血;②血红蛋白尿多见;③多数伴有脾大;④Coombs试验阳性。", "entities": [ { "start_idx": 1, "end_idx": 11, "type": "dis", "entity": "药物性免疫性溶血性贫血" }, { "start_idx": 12, "end_idx": 22, "type": "dis", "entity": "药物性免疫性溶血性贫血" }, { "start_idx": 24, "end_idx": 55, "type": "dis", "entity": "druginducedimmunehemolyticanemia" }, { "start_idx": 57, "end_idx": 61, "type": "dis", "entity": "DIIHA" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "急性溶血" }, { "start_idx": 80, "end_idx": 84, "type": "sym", "entity": "血红蛋白尿" }, { "start_idx": 93, "end_idx": 93, "type": "bod", "entity": "脾" }, { "start_idx": 93, "end_idx": 94, "type": "sym", "entity": "脾大" }, { "start_idx": 97, "end_idx": 104, "type": "pro", "entity": "Coombs试验" } ] }, { "text": "可诱发DIIHA的药物很多,主要包括抗感染药物(如青霉素类、头孢霉素类、抗结核药、磺胺类等)、消炎止痛药(安乃近、保泰松、吲哚美辛等)、镇静催眠及抗癫痫药(如苯妥英钠、氯丙嗪、氯氮等)及奎尼丁、奎宁、甲基多巴、左旋多巴、白消安等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "DIIHA" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "青霉素" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "头孢霉素" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "抗结核药" }, { "start_idx": 41, "end_idx": 43, "type": "dru", "entity": "磺胺类" }, { "start_idx": 47, "end_idx": 51, "type": "dru", "entity": "消炎止痛药" }, { "start_idx": 53, "end_idx": 55, "type": "dru", "entity": "安乃近" }, { "start_idx": 57, "end_idx": 59, "type": "dru", "entity": "保泰松" }, { "start_idx": 61, "end_idx": 64, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 73, "end_idx": 76, "type": "dru", "entity": "抗癫痫药" }, { "start_idx": 79, "end_idx": 82, "type": "dru", "entity": "苯妥英钠" }, { "start_idx": 84, "end_idx": 86, "type": "dru", "entity": "氯丙嗪" }, { "start_idx": 88, "end_idx": 89, "type": "dru", "entity": "氯氮" }, { "start_idx": 93, "end_idx": 95, "type": "dru", "entity": "奎尼丁" }, { "start_idx": 97, "end_idx": 98, "type": "dru", "entity": "奎宁" }, { "start_idx": 100, "end_idx": 103, "type": "dru", "entity": "甲基多巴" }, { "start_idx": 105, "end_idx": 108, "type": "dru", "entity": "左旋多巴" }, { "start_idx": 110, "end_idx": 112, "type": "dru", "entity": "白消安" } ] }, { "text": "临床上服药后出现急性溶血性贫血,且Coombs试验阳性者可确诊本病。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "急性溶血性贫血" }, { "start_idx": 17, "end_idx": 26, "type": "sym", "entity": "Coombs试验阳性" } ] }, { "text": "患儿一经确诊,应立刻停用可疑药物,并给予糖皮质激素治疗,严重贫血时,输注洗涤红细胞。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "贫血" }, { "start_idx": 34, "end_idx": 40, "type": "pro", "entity": "输注洗涤红细胞" } ] }, { "text": "在中国,医疗活动中善意的隐瞒(如确诊为恶性肿瘤而不告以实情)曾经被认为是积极的行为,新的《医疗事故处理办法》对告知的具体要求使得上述“积极行为”的合法性受到挑战。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "(五)利益冲突儿科医生要维护患儿的利益,也要维护患儿家庭的利益,而有时患儿的利益和其家庭的利益是不一致的,这种利益冲突造成许多儿科医学上特有的伦理学的问题,也是伦理学上的重要命题。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dep", "entity": "儿科" } ] }, { "text": "第六章组织细胞增生症组织细胞起自骨髓造血干细胞,然后逐步分化成熟为单核-巨噬细胞及树突状细胞,完全分化成熟的组织细胞分布于全身各脏器组织中发挥它们的功能,不同部位的组织细胞各有其特定的名称,如在外周血中即为单核细胞,肝脏中称为库普弗细胞,在骨髓中称巨噬细胞,在皮肤中称朗格汉斯细胞。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "组织细胞增生症" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "组织细胞" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "骨髓造血干细胞" }, { "start_idx": 33, "end_idx": 39, "type": "bod", "entity": "单核-巨噬细胞" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "树突状细胞" }, { "start_idx": 54, "end_idx": 57, "type": "bod", "entity": "组织细胞" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "脏器组织" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "组织细胞" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "外周血" }, { "start_idx": 103, "end_idx": 106, "type": "bod", "entity": "单核细胞" }, { "start_idx": 108, "end_idx": 109, "type": "bod", "entity": "肝脏" }, { "start_idx": 113, "end_idx": 117, "type": "bod", "entity": "库普弗细胞" }, { "start_idx": 120, "end_idx": 121, "type": "bod", "entity": "骨髓" }, { "start_idx": 124, "end_idx": 127, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 130, "end_idx": 131, "type": "bod", "entity": "皮肤" }, { "start_idx": 134, "end_idx": 139, "type": "bod", "entity": "朗格汉斯细胞" } ] }, { "text": "单核-巨噬细胞主要功能为吞噬及杀伤入侵的微生物及处理衰老和变性的细胞,而树状突细胞多作为抗原提呈细胞,在主要组织相容性抗原参与下,将抗原提呈给T、B淋巴细胞,激发体内特异性细胞及体液免疫反应。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "单核-巨噬细胞" }, { "start_idx": 20, "end_idx": 22, "type": "mic", "entity": "微生物" }, { "start_idx": 26, "end_idx": 33, "type": "bod", "entity": "衰老和变性的细胞" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "树状突细胞" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "细胞" }, { "start_idx": 71, "end_idx": 77, "type": "bod", "entity": "T、B淋巴细胞" }, { "start_idx": 83, "end_idx": 87, "type": "bod", "entity": "特异性细胞" } ] }, { "text": "该分类将组织细胞增生症分为Ⅰ、Ⅱ、Ⅲ型,Ⅰ型旧称为组织细胞增生症Ⅹ,病变中的组织细胞主要为郎罕细胞,因此目前已更名为郎罕组织细胞增生症。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "组织细胞增生症" }, { "start_idx": 25, "end_idx": 32, "type": "dis", "entity": "组织细胞增生症Ⅹ" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "组织细胞" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "郎罕细胞" }, { "start_idx": 58, "end_idx": 66, "type": "dis", "entity": "郎罕组织细胞增生症" } ] }, { "text": "组织细胞增生症Ⅲ型为恶性组织细胞疾病,包括急性单核细胞性白血病、恶性组织细胞增生症、组织细胞肉瘤和极少见的恶性朗格汉斯增生症。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "组织细胞增生症" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "恶性组织细胞疾病" }, { "start_idx": 21, "end_idx": 30, "type": "dis", "entity": "急性单核细胞性白血病" }, { "start_idx": 32, "end_idx": 40, "type": "dis", "entity": "恶性组织细胞增生症" }, { "start_idx": 42, "end_idx": 47, "type": "bod", "entity": "组织细胞肉瘤" }, { "start_idx": 53, "end_idx": 61, "type": "dis", "entity": "恶性朗格汉斯增生症" } ] }, { "text": "第一节朗格汉斯细胞性组织细胞增生症朗格汉斯细胞性组织细胞增生症(Langerhan’scellhistiocytosis)为组织细胞增生症Ⅰ型。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "dis", "entity": "朗格汉斯细胞性组织细胞增生症" }, { "start_idx": 17, "end_idx": 30, "type": "dis", "entity": "朗格汉斯细胞性组织细胞增生症" }, { "start_idx": 32, "end_idx": 59, "type": "dis", "entity": "Langerhan’scellhistiocytosis" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "组织细胞增生症" } ] }, { "text": "因其共同病理特点为郎罕组织细胞浸润,故重新命名为朗格汉斯细胞性组织细胞增多症,朗格汉斯细胞组织细胞为一种正常的抗原提呈细胞,但对抗原刺激呈失控性反应。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "组织细胞" }, { "start_idx": 24, "end_idx": 37, "type": "dis", "entity": "朗格汉斯细胞性组织细胞增多症" }, { "start_idx": 39, "end_idx": 48, "type": "bod", "entity": "朗格汉斯细胞组织细胞" } ] }, { "text": "根据发病年龄,病变范围和临床表现,将此类疾病分为三种亚型,包括勒-雪综合征(Letterer-Siwesyndrome),韩-薛-柯综合征(Hand-Schuller-Christiansyndrome)和骨嗜酸细胞性肉芽肿。", "entities": [ { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "勒-雪综合征" }, { "start_idx": 38, "end_idx": 58, "type": "dis", "entity": "Letterer-Siwesyndrome" }, { "start_idx": 61, "end_idx": 68, "type": "dis", "entity": "韩-薛-柯综合征" }, { "start_idx": 70, "end_idx": 100, "type": "dis", "entity": "Hand-Schuller-Christiansyndrome" }, { "start_idx": 103, "end_idx": 111, "type": "dis", "entity": "骨嗜酸细胞性肉芽肿" } ] }, { "text": "(一)勒-雪综合征(急性婴儿型)以内脏病变为主,此型临床表现最严重、也最多见,多于1岁以内发病,起病急而重,以内脏和皮肤受侵害为主。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "勒-雪综合征" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "内脏" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "内脏" } ] }, { "text": "表现为发热、皮疹、咳嗽、苍白、耳流脓、肝、脾很快增大,肺部广泛浸润,淋巴结轻度增大,皮疹较特殊,主要分布于躯干、颈部、头皮和发际,四肢少见。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "发热" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "皮疹" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "咳嗽" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "苍白" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "耳" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "耳流脓" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "肝" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "脾" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肺部" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "肺部广泛浸润" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "淋巴结" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "淋巴结轻度增大" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "皮疹" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "躯干" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "颈部" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "头皮" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "发际" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "四肢" } ] }, { "text": "初起为淡红色斑丘疹,直径1~3mm,继而呈出血性或湿疹样、皮脂溢出样皮疹,以后皮疹表面结痂、脱屑、脱痂后留有白斑或色素沉着。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "初起为淡红色斑丘疹" }, { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "出血性或湿疹样" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "皮脂" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "皮脂溢出样皮疹" }, { "start_idx": 39, "end_idx": 60, "type": "sym", "entity": "皮疹表面结痂、脱屑、脱痂后留有白斑或色素沉着" } ] }, { "text": "各期皮疹可同时存在,常成批发生,一批消退,一批又起,手触摸时有粗糙感。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "皮疹" }, { "start_idx": 11, "end_idx": 33, "type": "sym", "entity": "成批发生,一批消退,一批又起,手触摸时有粗糙感" } ] }, { "text": "出疹前常先发热,出疹同时伴肝增大,呼吸道感染时,症状急剧加重,极易发生肺炎,出现喘憋和发绀,但肺部体征多不明显,因系肺间质性病变,可并发气胸和皮下气肿。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "发热" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肝" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "肝增大" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "肺炎" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "喘憋" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "发绀" }, { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "肺间质性病变" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "气胸" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "皮下气肿" } ] }, { "text": "呼吸衰竭是致死的主要原因。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "此外常见耳流脓、营养不良、腹泻和贫血。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "耳" }, { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "耳流脓" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "营养不良" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "腹泻" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "贫血" } ] }, { "text": "也可同时有溶骨性骨骼病变,但与其他二型相比,相对较少。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "溶骨性骨骼病变" } ] }, { "text": "(二)韩-薛-柯综合征骨损害伴中度其他器官侵犯,又称慢性黄色瘤病,多发生于3~4岁小儿。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "韩-薛-柯综合征" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "骨损害" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "慢性黄色瘤病" } ] }, { "text": "颅骨缺损、突眼、尿崩症是此型的三大特征,这三大特征可先后出现或在病程中只见其中之一或二。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "颅骨" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "颅骨缺损、突眼" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "尿崩症" } ] }, { "text": "初起颅骨损害呈肿块状凸起,硬,有轻度压痛。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "颅骨" }, { "start_idx": 2, "end_idx": 19, "type": "sym", "entity": "颅骨损害呈肿块状凸起,硬,有轻度压痛" } ] }, { "text": "当病变蚀穿颅骨外板后、肿物变软、触之有波动感,常可触及颅骨边缘,压痛不明显,以后肿块逐渐吸收、局部下凹,缺损大者可触及脑,并随脉搏跳动。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "颅骨外板" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "颅骨" }, { "start_idx": 11, "end_idx": 36, "type": "sym", "entity": "肿物变软、触之有波动感,常可触及颅骨边缘,压痛不明显" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "肿块" }, { "start_idx": 59, "end_idx": 59, "type": "bod", "entity": "脑" }, { "start_idx": 40, "end_idx": 66, "type": "sym", "entity": "肿块逐渐吸收、局部下凹,缺损大者可触及脑,并随脉搏跳动" } ] }, { "text": "尿崩症由垂体或下丘脑受累所致,不一定有蝶鞍破坏。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "尿崩症" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "垂体" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "下丘脑" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "蝶鞍破坏" } ] }, { "text": "皮疹呈孤立、稀疏的黄色丘疹,似半个小米粒或黄豆状,突出在皮肤表面,若以玻片压于其上,可见皮疹中央发黄,是慢性黄色瘤病名称的来由。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "皮肤" }, { "start_idx": 0, "end_idx": 31, "type": "sym", "entity": "皮疹呈孤立、稀疏的黄色丘疹,似半个小米粒或黄豆状,突出在皮肤表面" }, { "start_idx": 35, "end_idx": 36, "type": "equ", "entity": "玻片" }, { "start_idx": 44, "end_idx": 49, "type": "sym", "entity": "皮疹中央发黄" }, { "start_idx": 52, "end_idx": 57, "type": "dis", "entity": "慢性黄色瘤病" } ] }, { "text": "其后皮疹开始消退变软、变浅,慢慢吸收。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "皮疹" } ] }, { "text": "此外低热、轻度肝、脾增大,贫血,牙龈齿槽肿胀,发炎、坏死、萎缩、牙齿松动、脱落也常见。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "低热" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肝" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "脾" }, { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "轻度肝、脾增大" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "贫血" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "牙龈" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "牙龈齿槽肿胀" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "发炎" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "坏死" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "萎缩" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "牙齿" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "牙齿松动、脱落" } ] }, { "text": "(三)骨嗜酸细胞肉芽肿为单纯骨损害型,是本症中预后最好的一型,多发于4~7岁小儿,但也可见于婴儿或成人,任何骨骼均可受累,但以颅骨、四肢骨、脊椎、骨盆最常见。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "骨嗜酸细胞肉芽肿" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "骨骼" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "颅骨" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "四肢骨" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "脊椎" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "骨盆" } ] }, { "text": "不少患儿是在偶然情况下或出现病理性骨折时发现。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "病理性骨折" } ] }, { "text": "唯有脊椎病变,椎弓破坏者,常伴神经压迫症状,如肢体麻木、疼痛、无力、瘫痪,甚至大小便失禁成为疾病的主诉而就医。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "脊椎" }, { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "脊椎病变" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "椎弓破坏" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "神经压迫" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肢体" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "肢体麻木" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "疼痛" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "无力" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "瘫痪" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "大小便失禁" } ] }, { "text": "发热、贫血、肝脾大、耳流脓伴典型皮疹时要考虑郎罕细胞性组织细胞增生症Ⅰ型。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "发热" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "贫血" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "肝脾大" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "耳流脓伴" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "皮疹" }, { "start_idx": 22, "end_idx": 33, "type": "dis", "entity": "郎罕细胞性组织细胞增生症" } ] }, { "text": "突眼、尿崩症、颅骨缺损是Ⅱ型综合征典型表现,应疑及本病。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "突眼" }, { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "尿崩症" }, { "start_idx": 7, "end_idx": 16, "type": "dis", "entity": "颅骨缺损是Ⅱ型综合征" } ] }, { "text": "骨骼X线摄片示扁平骨和长骨发生溶骨性骨质破坏,形状不规则,边缘可呈锯齿状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "骨骼" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "扁平骨" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "长骨" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "溶骨性骨质破坏" } ] }, { "text": "脊椎骨受压则呈扁平椎,但椎间隙不狭窄。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脊椎" } ] }, { "text": "上下颌骨破坏可致牙齿脱落,长骨的缺损多为囊状,单发或互相融合成分房状,骨皮质变薄。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "颌骨" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "牙齿" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "长骨" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "骨皮质" } ] }, { "text": "破坏明显处可有层状骨膜增生。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "骨膜" } ] }, { "text": "以上典型的骨X线变化提示郎罕细胞性组织细胞增生症。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "骨X线" }, { "start_idx": 12, "end_idx": 23, "type": "dis", "entity": "郎罕细胞性组织细胞增生症" } ] }, { "text": "皮疹印片或活检、骨病变,郎罕细胞是诊断的依据。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "活检" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "骨" }, { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "骨病变" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "郎罕细胞" } ] }, { "text": "S-100蛋白染色阳性,电镜找到Birbeck颗粒支持本病的诊断。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "equ", "entity": "电镜" } ] }, { "text": "(一)局限病灶手术治疗病变局限的骨嗜酸性肉芽肿应采取手术刮除或切除。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "骨嗜酸性肉芽肿" } ] }, { "text": "比较小的病灶应用局部氢化可的松注射亦可取得与手术刮除同样的效果。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "氢化可的松" } ] }, { "text": "(二)放射治疗适用于孤立的骨骼病变,尤以手术刮除有困难的部位如:眼眶周围、颌骨、乳突或负重后易发生骨折和神经损伤的脊椎等部位,以及早期的垂体病变,尿崩症出现时间较久(如6个月以上),放射治疗大多无效。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "骨骼" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "骨骼病变" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "手术刮除" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "眼眶" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "颌骨" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "乳突" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "骨折" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "神经损伤" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "脊椎" }, { "start_idx": 68, "end_idx": 69, "type": "bod", "entity": "垂体" }, { "start_idx": 68, "end_idx": 71, "type": "sym", "entity": "垂体病变" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "尿崩症" }, { "start_idx": 91, "end_idx": 94, "type": "pro", "entity": "放射治疗" } ] }, { "text": "皮肤病变对放疗亦不敏感。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "皮肤病变" } ] }, { "text": "(三)药物治疗肾上腺皮质激素为首选药物,且多药联合治疗并不比采用单一药物效果好,但对多脏器受累病人应采用联合化疗。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "脏器" } ] }, { "text": "常用药物:①肾上腺皮质激素:泼尼松45~60mg/(m2•d)或地塞米松8~10mg/(m2•d)口服,分3~4次,6周后减至半量,再用4周,然后逐渐减量,总疗程12周。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "泼尼松" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "地塞米松" } ] }, { "text": "危重病人可静脉滴注氢化可的松250~300mg/M2。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "氢化可的松" } ] }, { "text": "急性症状消失后改为口服,此类药物对全身症状如发热、皮疹和贫血等效果较好。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "发热" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "皮疹" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "贫血" } ] }, { "text": "②长春花碱:每次6mg/m2,一周1次。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "长春花碱" } ] }, { "text": "或长春新碱每次1.4mg/m2,一周1次,静脉注射,连用4~6周,以后改为每月一次或停8~12周后,再给4~6周,并与泼尼松合用。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "长春新碱" }, { "start_idx": 65, "end_idx": 67, "type": "dru", "entity": "泼尼松" } ] }, { "text": "若应用上述药物4~6周后效果不明显,可加用足叶乙甙(Vp16)100/(m2•d),静滴,每日1次,用3~5天,每月一疗程。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "足叶乙甙" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "Vp16" } ] }, { "text": "亦可采用6-巯基嘌呤(6-MP)或6-硫鸟嘌呤(6-TG)60~75mg/(m2•d)口服。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "6-巯基嘌呤" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "6-MP" }, { "start_idx": 17, "end_idx": 22, "type": "dru", "entity": "6-硫鸟嘌呤" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "6-TG" } ] }, { "text": "甲氨蝶呤(MTX)每次15~20mg/m2,每周1次口服或静脉注射。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "甲氨蝶呤" }, { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "MTX" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "静脉注射" } ] }, { "text": "多脏器病变化疗时间一般不短于1年。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "脏器" } ] }, { "text": "若于用药后很快退热,精神食欲好转,1~2周后皮疹消退,肝、脾缩小,肺部症状减轻说明对治疗敏感,但骨骼X线,肺部病变消退较慢。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "皮疹消退" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "肝" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "脾" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "肝、脾缩小" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "肺部" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "骨骼X线" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "肺部" } ] }, { "text": "有些病人在治疗过程中可有反复或发生新的病状,则应改换其他药物,如环磷酰胺10mg/(kg•d),连续3天,每三周1次。", "entities": [ { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "环磷酰胺" } ] }, { "text": "(四)免疫治疗胸腺素或胸腺肽,每次3~5mg静脉或肌肉注射,连用2~3个月,可与化疗联合使用,增加疗效。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "胸腺素" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "胸腺肽" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肌肉" } ] }, { "text": "(五)支持治疗包括用SMZco预防卡氏肺囊虫病及积极控制感染,特别是中耳炎。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "SMZco" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "卡氏肺囊虫病" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "中耳炎" } ] }, { "text": "如合并呼吸道感染导致呼吸衰竭,应及时给氧并需监护。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "呼吸衰竭" } ] }, { "text": "对尿崩症患者应给神经垂体激素治疗。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "尿崩症" } ] }, { "text": "此外预防出血、纠正贫血亦很重要。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "贫血" } ] }, { "text": "【预后】预后决定于发病年龄,受累器官的数目和有无脏器功能衰竭。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "脏器" } ] }, { "text": "参考文献1.BillmireD,VinocurC,RescorlaF,etal.Malignantmediastinalgermcelltumors:anintergroupstudy.JPediatrSurg,2001,36(1):18-182.MannJR,RaafatF,RobinsonK,etal.TheUnitedKingdomChildren'sCancerStudyGroup'ssecondgermcelltumorstudy:carboplatin,etoposide,andbleomycinareeffectivetreatmentforchildrenwithmalignantextracranialgermcelltumors,withacceptabletoxicity.JClinOncol,2000,18(22):3809-38093.PhilipAPizzo,DavidGPoplock.PrincipleandPracticeofPediatricOncology.4rded.Philadelphia.NewYork:LWW.com,2002.1091-10914.RescorlaF,BillmireD,StolarC,etal.Theeffectofcisplatindoseandsurgicalresectioninchildrenwithmalignantgermcelltumorsatthesacrococcygealregion:apediatricintergrouptrial(POG9049/CCG8882).JPediatrSurg,2001,36(1):12-125.VukyJ,BainsM,BacikJ,etal.Roleofpostchemotherapyadjunctivesurgeryinthemanagementofpatientswithnonseminomaarisingfromthemediastinum.JClinOncol,2001,19(3):682-688", "entities": [ { "start_idx": 40, "end_idx": 73, "type": "dis", "entity": "Malignantmediastinalgermcelltumors" }, { "start_idx": 198, "end_idx": 216, "type": "dis", "entity": "secondgermcelltumor" }, { "start_idx": 223, "end_idx": 233, "type": "dru", "entity": "carboplatin" }, { "start_idx": 235, "end_idx": 243, "type": "dru", "entity": "etoposide" }, { "start_idx": 248, "end_idx": 259, "type": "dru", "entity": "bleomycinare" }, { "start_idx": 293, "end_idx": 327, "type": "dis", "entity": "malignantextracranialgermcelltumors" }, { "start_idx": 595, "end_idx": 616, "type": "dis", "entity": "malignantgermcelltumor" } ] }, { "text": "第五节维生素B1缺乏症维生素B1又称硫胺素、抗脚气病因子或抗神经炎因子,它是最早发现的维生素之一。", "entities": [ { "start_idx": 3, "end_idx": 21, "type": "dis", "entity": "维生素B1缺乏症" }, { "start_idx": 22, "end_idx": 37, "type": "bod", "entity": "维生素B1" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "硫胺素" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "抗脚气病因子" }, { "start_idx": 51, "end_idx": 56, "type": "bod", "entity": "抗神经炎因子" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "维生素" } ] }, { "text": "维生素B1在高温、特别是高温碱性溶液中易被破坏,在酸性溶液中,稳定性较好。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "bod", "entity": "维生素B1" } ] }, { "text": "在体内硫胺80%是以硫胺素焦磷酸盐(TPP)的形式存在,10%是以硫胺素三磷酸盐的形式存在,其余的为硫胺素单磷酸盐或游离的硫胺素。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "硫胺" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "硫胺素焦磷酸盐" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "TPP" }, { "start_idx": 33, "end_idx": 39, "type": "bod", "entity": "硫胺素三磷酸盐" }, { "start_idx": 50, "end_idx": 56, "type": "bod", "entity": "硫胺素单磷酸盐" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "硫胺素" } ] }, { "text": "维生素B1缺乏将引起一种典型的疾病,被称为脚气病。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "dis", "entity": "维生素B1缺乏" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "脚气病" } ] }, { "text": "【流行病学】18~19世纪,脚气病在中国、日本(尤其在东南亚一带)广为流行,当时每年约有几十万人死于脚气病。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "脚气病" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "脚气病" } ] }, { "text": "第一个记录脚气病的是1592年荷兰医生JacobBontius。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "脚气病" } ] }, { "text": "1897年,一名驻爪哇的荷兰医生Eijkman以小鸡做实验,发现用精白米饲养小鸡,即出现一种类似脚气病的多发性神经炎,如用糙米饲养小鸡,则能预防这种疾病。", "entities": [ { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "脚气病" }, { "start_idx": 52, "end_idx": 57, "type": "dis", "entity": "多发性神经炎" } ] }, { "text": "1936年,Williams公布了硫胺素的化学结构,它是由含硫噻唑环联结氨基吡啶环组成,并开始人工合成。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "硫胺素" } ] }, { "text": "近年,随着生活水平的提高,人们食不厌精、脍不厌细,使维生素B1缺乏发病率有升高趋势,使乳母及婴幼儿体内的维生素B1严重不足。", "entities": [ { "start_idx": 26, "end_idx": 43, "type": "dis", "entity": "维生素B1缺乏" }, { "start_idx": 63, "end_idx": 78, "type": "bod", "entity": "维生素B1" } ] }, { "text": "许秀举等抽样选择包头郊区4个地区的6~8岁儿童409名,采用荧光法测定其4小时负荷尿中维生素B1的含量,结果缺乏率为14.42%,不足率为18.58%。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "荧光法" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "尿" }, { "start_idx": 43, "end_idx": 58, "type": "bod", "entity": "维生素B1" } ] }, { "text": "新华医院对25例以夜寐不安为主要表现,同时伴有烦躁、食欲缺乏的<2岁小儿进行临床观察和红细胞转酮醇酶活力(TPP效应)的测定,结果显示16%的小儿属边缘型维生素B1缺乏,20%属严重缺乏。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "夜寐不安" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "烦躁" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 43, "end_idx": 51, "type": "ite", "entity": "红细胞转酮醇酶活力" }, { "start_idx": 53, "end_idx": 57, "type": "ite", "entity": "TPP效应" }, { "start_idx": 74, "end_idx": 94, "type": "dis", "entity": "边缘型维生素B1缺乏" } ] }, { "text": "【病因】(一)摄入不足母乳中维生素B1的含量较牛乳低,母乳中的含量为16μg/ml,牛乳中的含量为42μg/ml,但母乳中的维生素B1含量,对婴儿的生长需要已足够。", "entities": [ { "start_idx": 14, "end_idx": 29, "type": "bod", "entity": "维生素B1" }, { "start_idx": 73, "end_idx": 88, "type": "bod", "entity": "维生素B1" } ] }, { "text": "但如果乳母膳食中维生素B1的摄入量缺乏,则会引起母乳中的维生素B1不足,如不及时补充,也将引起婴儿维生素B1缺乏症。", "entities": [ { "start_idx": 8, "end_idx": 23, "type": "bod", "entity": "维生素B1" }, { "start_idx": 39, "end_idx": 54, "type": "bod", "entity": "维生素B1" }, { "start_idx": 71, "end_idx": 89, "type": "dis", "entity": "维生素B1缺乏症" } ] }, { "text": "对于已添加辅食的小儿,如长期使用精白米、面以及淀粉为主食,或煮饭时为增加其黏稠度而加入少量的碱,将破坏维生素B1。", "entities": [ { "start_idx": 51, "end_idx": 66, "type": "dru", "entity": "维生素B1" } ] }, { "text": "(二)吸收障碍如患有消化系统疾病,如慢性腹泻、慢性痢疾、胆囊纤维化、肠道感染等疾病,均可减少维生素B1的吸收。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "消化系统疾病" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "慢性腹泻" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "慢性痢疾" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "胆囊纤维化" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "肠道感染" }, { "start_idx": 46, "end_idx": 61, "type": "bod", "entity": "维生素B1" } ] }, { "text": "肝、肾疾病将影响TPP的合成,造成维生素B1缺乏。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肝" }, { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肾" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "TPP" }, { "start_idx": 17, "end_idx": 34, "type": "dis", "entity": "维生素B1缺乏" } ] }, { "text": "维生素B1缺乏使胃液中酸度降低,从而在胃肠道中维生素B1复合物内的维生素B1释放减少,影响了维生素B1的吸收。", "entities": [ { "start_idx": 0, "end_idx": 17, "type": "dis", "entity": "维生素B1缺乏" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胃液" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "胃肠道" }, { "start_idx": 34, "end_idx": 49, "type": "dru", "entity": "维生素B1" }, { "start_idx": 55, "end_idx": 70, "type": "dru", "entity": "维生素B1" }, { "start_idx": 79, "end_idx": 94, "type": "dru", "entity": "维生素B1" } ] }, { "text": "(三)维生素B1药物的需要量增加儿童生长发育速度较快,需要量也相对较多;如小儿患结核、麻疹、水痘、肺炎以及高热时,或患有如甲状腺功能亢进等代谢率增加的疾病时,维生素B1的消耗增加,如此时未予及时补充,则造成维生素B1的缺乏。", "entities": [ { "start_idx": 3, "end_idx": 18, "type": "bod", "entity": "维生素B1" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "结核" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "麻疹" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "水痘" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "肺炎" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "高热" }, { "start_idx": 72, "end_idx": 78, "type": "dis", "entity": "甲状腺功能亢进" }, { "start_idx": 90, "end_idx": 105, "type": "bod", "entity": "维生素B1" }, { "start_idx": 125, "end_idx": 140, "type": "bod", "entity": "维生素B1" } ] }, { "text": "(四)遗传代谢障碍遗传性维生素B1代谢与功能障碍引起的维生素B1缺乏症,一般具有高度的家族性遗传性疾病史,或父母近亲结婚史。", "entities": [ { "start_idx": 9, "end_idx": 34, "type": "dis", "entity": "遗传性维生素B1代谢与功能障碍" }, { "start_idx": 38, "end_idx": 56, "type": "dis", "entity": "维生素B1缺乏症" }, { "start_idx": 65, "end_idx": 72, "type": "dis", "entity": "家族性遗传性疾病" } ] }, { "text": "【病理生理】在身体中,硫胺80%是以TPP的形式存在,它是丙酮酸氧化脱羧酶系的辅助因子,也是磷酸己糖氧化支路中转羧乙醛酶的辅酶。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "硫胺" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "TPP" }, { "start_idx": 29, "end_idx": 36, "type": "bod", "entity": "丙酮酸氧化脱羧酶" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "转羧乙醛酶" } ] }, { "text": "因此,维生素B1与糖代谢密切相关,其缺乏使糖代谢受阻,能量产生减少,会产生一系列的病理变化。", "entities": [ { "start_idx": 3, "end_idx": 18, "type": "bod", "entity": "维生素B1" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "糖" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "糖" } ] }, { "text": "(一)神经系统尤其是末梢神经受损严重、髓鞘退化及色素沉着。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "神经系统" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "末梢神经" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "髓鞘" } ] }, { "text": "中枢神经系统和周围神经系统的神经纤维的髓鞘发育不良,因此表现为易激惹。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 7, "end_idx": 12, "type": "bod", "entity": "周围神经系统" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "神经纤维" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "髓鞘发育不良" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "易激惹" } ] }, { "text": "重者神经轴被破坏,以坐骨神经及其分支受累较为常见,并且出现较早。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "神经轴" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "坐骨神经及其分支" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "坐骨神经及其分支受累" } ] }, { "text": "其他如前臂神经等亦可累及。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "前臂神经" } ] }, { "text": "(二)心血管系统由于能量缺乏,心肌无力,严重时发生心力衰竭,周围血管平滑肌张力下降,小血管扩张。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "心血管系统" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心肌" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "血管平滑肌" }, { "start_idx": 30, "end_idx": 40, "type": "sym", "entity": "周围血管平滑肌张力下降" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "小血管" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "小血管扩张" } ] }, { "text": "心脏扩张肥厚,尤以右心明显。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心脏" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "右心" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "心脏扩张肥厚,尤以右心明显" } ] }, { "text": "心肌水肿,其心肌纤维粗硬。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心肌" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "心肌纤维" }, { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "心肌水肿,其心肌纤维粗硬" } ] }, { "text": "血管充血,但组织结构正常。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血管" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "血管充血" } ] }, { "text": "(三)组织水肿及浆膜腔积液组织水肿多见于下肢,当体腔浆液渗出时,可见心包腔、胸腔及腹腔积液。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "组织水肿" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "浆膜腔" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "浆膜腔积液" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "组织水肿" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "下肢" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "体腔" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "心包腔" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "胸腔" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "腹腔" }, { "start_idx": 34, "end_idx": 44, "type": "sym", "entity": "心包腔、胸腔及腹腔积液" } ] }, { "text": "(四)肌肉萎缩出现于受累神经支配的肌肉。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "肌肉萎缩" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "受累神经" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肌肉" } ] }, { "text": "镜下可见肌纤维横纹消失、混浊肿胀及脂肪变性。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "肌纤维" }, { "start_idx": 4, "end_idx": 20, "type": "sym", "entity": "肌纤维横纹消失、混浊肿胀及脂肪变性" } ] }, { "text": "(五)消化系统消化道平滑肌张力下降,影响胃肠蠕动,消化功能减弱。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "消化系统" }, { "start_idx": 7, "end_idx": 12, "type": "bod", "entity": "消化道平滑肌" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "消化道平滑肌张力下降" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "胃肠" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "消化功能减弱" } ] }, { "text": "【临床表现】维生素B1缺乏将导致脚气病。", "entities": [ { "start_idx": 6, "end_idx": 23, "type": "dis", "entity": "维生素B1缺乏" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "脚气病" } ] }, { "text": "脚气病是维生素B1摄入不足的最终结果。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脚气病" }, { "start_idx": 4, "end_idx": 19, "type": "dru", "entity": "维生素B1" } ] }, { "text": "本病主要影响心血管和神经系统。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "心血管" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "神经系统" } ] }, { "text": "主要表现为多发性神经炎、肌肉萎缩、组织水肿、心脏扩大、循环失调及胃肠症状。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "多发性神经炎" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肌肉" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "肌肉萎缩" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "组织水肿" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "心脏" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "循环失调" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "胃肠" } ] }, { "text": "消化系统症状:发病初期主要表现为消化系统症状,如食欲缺乏、厌食、恶心、呕吐、腹痛、便秘或腹泻。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "消化系统" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "消化系统" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "厌食" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "恶心" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "呕吐" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "腹痛" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "便秘或腹泻" } ] }, { "text": "神经系统症状:消化道系统症状出现后不久就出现神经系统症状,神经系统症状突出者可分为脑型或神经炎型。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "神经系统" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "消化道系统" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "神经系统" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "神经系统" } ] }, { "text": "跟腱反射和膝反射初期增强,随后减弱,最后消失。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "跟腱" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "膝" }, { "start_idx": 0, "end_idx": 21, "type": "sym", "entity": "跟腱反射和膝反射初期增强,随后减弱,最后消失" } ] }, { "text": "心血管症状:出现心悸、心动过速,婴儿可出现奔马律,呼吸困难,晚期出现发绀、心脏扩大、心力衰竭、肺充血及肝淤血。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "心血管" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "心" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "心悸" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "心" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "心动过速" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "奔马律" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "发绀" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "心脏" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "心" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "肺" }, { "start_idx": 47, "end_idx": 49, "type": "sym", "entity": "肺充血" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "肝" }, { "start_idx": 51, "end_idx": 53, "type": "sym", "entity": "肝淤血" } ] }, { "text": "如不及时治疗,很快死亡。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "死亡" } ] }, { "text": "水肿及浆膜腔积液:水肿可遍及全身,多发生于下肢,浆膜腔积液,可发生于心包腔、胸腔和腹腔。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "水肿" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "浆膜腔" }, { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "浆膜腔积液" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "水肿" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "下肢" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "浆膜腔" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "浆膜腔积液" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "心包腔" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "胸腔" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "腹腔" } ] }, { "text": "由于喉的水肿而出现失声,或出现特殊的喉鸣(脚气病哭声)。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "喉" }, { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "水肿" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "失声" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "特殊的喉鸣" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "脚气病" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "脚气病哭声" } ] }, { "text": "先天性维生素B1代谢缺陷有关的遗传性疾病包括枫糖尿症、婴儿慢性乳酸酸中毒、婴儿及儿童的亚急性坏死性脑病及对维生素B1有反应的巨幼红细胞贫血。", "entities": [ { "start_idx": 0, "end_idx": 22, "type": "dis", "entity": "先天性维生素B1代谢缺陷" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "遗传性疾病" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "枫糖尿症" }, { "start_idx": 38, "end_idx": 46, "type": "dis", "entity": "婴儿慢性乳酸酸中毒" }, { "start_idx": 54, "end_idx": 61, "type": "dis", "entity": "亚急性坏死性脑病" }, { "start_idx": 64, "end_idx": 79, "type": "bod", "entity": "维生素B1" }, { "start_idx": 84, "end_idx": 90, "type": "dis", "entity": "巨幼红细胞贫血" } ] }, { "text": "1.枫糖尿症枫糖尿病的病因是由于缺乏支链α-酮酸脱氢酶复合物,患者的相应α-酮酸不能通过氧化脱羧作用而降解,而引起支链氨基酸(亮氨酸、异亮氨酸、缬氨酸)代谢异常。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "枫糖尿症" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "枫糖尿病" }, { "start_idx": 18, "end_idx": 29, "type": "bod", "entity": "支链α-酮酸脱氢酶复合物" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "α-酮酸" } ] }, { "text": "此病是常染色体隐性遗传性疾病,可出现精神及身体发育延迟、嗜睡、喂养困难、注意力减退、肌张力交替性升高和减弱。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "常染色体隐性遗传性疾病" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "精神及身体发育延迟" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "嗜睡" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "喂养困难" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "注意力减退" }, { "start_idx": 42, "end_idx": 52, "type": "sym", "entity": "肌张力交替性升高和减弱" } ] }, { "text": "给予口服大剂量维生素B1治疗,可减轻临床症状,血清支链氨基酸水平恢复正常,如停止给予维生素B1时,血清支链氨基酸水平再度升高。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "口服" }, { "start_idx": 7, "end_idx": 22, "type": "dru", "entity": "维生素B1" }, { "start_idx": 53, "end_idx": 68, "type": "dru", "entity": "维生素B1" } ] }, { "text": "2.婴儿慢性乳酸酸中毒主要以乳酸和丙酮酸酸中毒、神经性异常以及发育迟缓为特征。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "婴儿慢性乳酸酸中毒" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "乳酸和丙酮酸酸中毒" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "神经性异常" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "发育迟缓" } ] }, { "text": "对大剂量维生素B1治疗有效者考虑为维生素B1代谢有缺陷,对维生素B1无效者可能为丙酮酸脱羧酶缺少。", "entities": [ { "start_idx": 4, "end_idx": 19, "type": "dru", "entity": "维生素B1" }, { "start_idx": 28, "end_idx": 43, "type": "bod", "entity": "维生素B1" }, { "start_idx": 28, "end_idx": 48, "type": "sym", "entity": "维生素B1代谢有缺陷" }, { "start_idx": 51, "end_idx": 66, "type": "dru", "entity": "维生素B1" }, { "start_idx": 73, "end_idx": 80, "type": "dis", "entity": "丙酮酸脱羧酶缺少" } ] }, { "text": "但有文献报道,丙酮酸脱羧酶缺少的婴儿,接受大剂量维生素B1治疗后好转。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "丙酮酸脱羧酶缺少" }, { "start_idx": 24, "end_idx": 39, "type": "dru", "entity": "维生素B1" } ] }, { "text": "3.婴儿及儿童的亚急性坏死型脑病(Leighs脑病)是婴儿期和儿童发育早期的一种致命性疾病,伴有虚弱、厌食、说话和眼球震颤、抽搐、瘫痪及复合感觉障碍,甚至生长停止。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "亚急性坏死型脑病" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "Leighs脑病" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "虚弱" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "厌食" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "说话和眼球震颤" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "抽搐" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "瘫痪" }, { "start_idx": 68, "end_idx": 73, "type": "sym", "entity": "复合感觉障碍" }, { "start_idx": 77, "end_idx": 80, "type": "sym", "entity": "生长停止" } ] }, { "text": "其血中的乳酸和丙酮酸升高,机制目前仍不详,考虑与TPP降低有关。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "血" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "乳酸" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "丙酮酸" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "TPP" } ] }, { "text": "4.对维生素B1有反应的巨幼红细胞贫血是婴儿期和儿童期的一种罕见疾病,其特点是巨幼红细胞性贫血,并伴有感觉神经性耳聋和糖尿病,也可能出现心脏异常。", "entities": [ { "start_idx": 3, "end_idx": 18, "type": "bod", "entity": "维生素B1" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "巨幼红细胞贫血" }, { "start_idx": 50, "end_idx": 57, "type": "dis", "entity": "巨幼红细胞性贫血" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "感觉神经性耳聋" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "糖尿病" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "心脏异常" } ] }, { "text": "此病与继发于维生素B1在细胞内的转运和吸收障碍所引起的维生素B1缺乏状态有关。", "entities": [ { "start_idx": 6, "end_idx": 21, "type": "bod", "entity": "维生素B1" }, { "start_idx": 38, "end_idx": 55, "type": "dis", "entity": "维生素B1缺乏" } ] }, { "text": "【维生素B1营养水平评价】评价维生素B1的营养状况,可通过测定维生素B1负荷前后的尿维生素B1排泄量,血清维生素B1水平、红细胞转酮醇酶(ETK)活性及空腹一次测定尿液中维生素B1/肌酐比率进行评价。", "entities": [ { "start_idx": 1, "end_idx": 16, "type": "dru", "entity": "维生素B1" }, { "start_idx": 26, "end_idx": 41, "type": "dru", "entity": "维生素B1" }, { "start_idx": 53, "end_idx": 68, "type": "dru", "entity": "维生素B1" }, { "start_idx": 74, "end_idx": 93, "type": "ite", "entity": "尿维生素B1排泄量" }, { "start_idx": 95, "end_idx": 114, "type": "ite", "entity": "血清维生素B1水平" }, { "start_idx": 116, "end_idx": 129, "type": "ite", "entity": "红细胞转酮醇酶(ETK)活性" }, { "start_idx": 137, "end_idx": 138, "type": "bod", "entity": "尿液" }, { "start_idx": 140, "end_idx": 160, "type": "ite", "entity": "维生素B1/肌酐比率" } ] }, { "text": "(一)维生素B1负荷前后的尿维生素B1排泄量摄入过多的维生素B1会从尿中排出,故可利用测定尿中的维生素B1来估计体内维生素B1的状态,因为维生素B1的需要量与其尿排泄量之间具有一定的关系,因此维生素B1负荷试验可以测定维生素B1的营养状况。", "entities": [ { "start_idx": 3, "end_idx": 18, "type": "dru", "entity": "维生素B1" }, { "start_idx": 24, "end_idx": 43, "type": "ite", "entity": "尿维生素B1排泄量" }, { "start_idx": 49, "end_idx": 64, "type": "dru", "entity": "维生素B1" }, { "start_idx": 67, "end_idx": 67, "type": "bod", "entity": "尿" }, { "start_idx": 78, "end_idx": 78, "type": "bod", "entity": "尿" }, { "start_idx": 81, "end_idx": 96, "type": "bod", "entity": "维生素B1" }, { "start_idx": 102, "end_idx": 117, "type": "bod", "entity": "维生素B1" }, { "start_idx": 124, "end_idx": 139, "type": "bod", "entity": "维生素B1" }, { "start_idx": 146, "end_idx": 149, "type": "ite", "entity": "尿排泄量" }, { "start_idx": 162, "end_idx": 181, "type": "ite", "entity": "维生素B1负荷试验" }, { "start_idx": 186, "end_idx": 201, "type": "bod", "entity": "维生素B1" } ] }, { "text": "通常用荧光法或微生物法进行维生素B1的测定,被测者于清晨排尿后禁食,给维生素B1(口服5mg或肌注1mg),然后饮水200ml,收集4小时尿,测定尿中维生素B1量,若在100μg以上者为正常,脚气患病常低于50μg。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "荧光法" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "微生物法" }, { "start_idx": 13, "end_idx": 28, "type": "bod", "entity": "维生素B1" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "尿" }, { "start_idx": 46, "end_idx": 61, "type": "dru", "entity": "维生素B1" }, { "start_idx": 63, "end_idx": 64, "type": "pro", "entity": "口服" }, { "start_idx": 69, "end_idx": 70, "type": "pro", "entity": "肌注" }, { "start_idx": 91, "end_idx": 91, "type": "bod", "entity": "尿" }, { "start_idx": 95, "end_idx": 95, "type": "bod", "entity": "尿" }, { "start_idx": 97, "end_idx": 112, "type": "bod", "entity": "维生素B1" }, { "start_idx": 129, "end_idx": 132, "type": "sym", "entity": "脚气患病" } ] }, { "text": "(二)血清维生素B1水平因为血中的游离维生素B1及其磷酸盐的含量很低,故测血中的维生素B1水平作为维生素B1营养状况的指标一直未被广泛采用,但是,最近采用灵敏的高效液相色谱法,此方法简单而可靠,易于标准化,但因其参考值幅度较广,血中含量不稳定,不能及时反映早期缺乏状况,故临床很少采用。", "entities": [ { "start_idx": 3, "end_idx": 22, "type": "ite", "entity": "血清维生素B1水平" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "血" }, { "start_idx": 30, "end_idx": 45, "type": "bod", "entity": "维生素B1" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "磷酸盐" }, { "start_idx": 59, "end_idx": 59, "type": "bod", "entity": "血" }, { "start_idx": 62, "end_idx": 77, "type": "bod", "entity": "维生素B1" }, { "start_idx": 82, "end_idx": 97, "type": "bod", "entity": "维生素B1" }, { "start_idx": 124, "end_idx": 130, "type": "ite", "entity": "高效液相色谱法" }, { "start_idx": 158, "end_idx": 158, "type": "bod", "entity": "血" } ] }, { "text": "正常参考值为103~306nmol/L(3.1~9.2μg/dl),如血清维生素B1水平<100nmol/L(3μg/dl),则提示维生素B1缺乏。", "entities": [ { "start_idx": 35, "end_idx": 54, "type": "ite", "entity": "血清维生素B1水平" }, { "start_idx": 77, "end_idx": 94, "type": "sym", "entity": "维生素B1缺乏" } ] }, { "text": "(三)红细胞转酮醇酶(ETK)活性这是测定维生素B1营养状况的特异性指标,也是评价维生素B1营养状况的最有效指标。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "ite", "entity": "红细胞转酮醇酶(ETK)活性" }, { "start_idx": 21, "end_idx": 36, "type": "bod", "entity": "维生素B1" }, { "start_idx": 52, "end_idx": 67, "type": "bod", "entity": "维生素B1" } ] }, { "text": "在临床维生素B1缺乏的症状出现之前,ETK已有改变,故称为亚临床诊断或边缘状态的检查。", "entities": [ { "start_idx": 3, "end_idx": 20, "type": "dis", "entity": "维生素B1缺乏" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "ETK" } ] }, { "text": "通过测定溶解的红细胞中戊糖消失率或己糖出现率来测量ETK活性。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "红细胞" }, { "start_idx": 25, "end_idx": 29, "type": "ite", "entity": "ETK活性" } ] }, { "text": "采用体外不加(基础)或加入TPP(刺激)后测定ETK的活性,通常以基础活性(ETKA)或以刺激后活性与基础活性之差占基础活性的百分率(ETK-AC活性系数或TPP效应)来表示。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "TPP" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "ETK" }, { "start_idx": 38, "end_idx": 41, "type": "ite", "entity": "ETKA" }, { "start_idx": 67, "end_idx": 76, "type": "ite", "entity": "ETK-AC活性系数" }, { "start_idx": 78, "end_idx": 82, "type": "ite", "entity": "TPP效应" } ] }, { "text": "硫胺素缺乏与ETKA的降低与ETK-AC的增加有联系;ETK-AC值越高,则维生素B1缺乏越严重。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "硫胺素缺乏" }, { "start_idx": 6, "end_idx": 9, "type": "ite", "entity": "ETKA" }, { "start_idx": 14, "end_idx": 19, "type": "ite", "entity": "ETK-AC" }, { "start_idx": 27, "end_idx": 32, "type": "ite", "entity": "ETK-AC" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "维生素B1缺乏" } ] }, { "text": "TPP效应的正常参考值为0%~15%,维生素B1低水平时为16%~20%,缺乏时>20%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "TPP效应" }, { "start_idx": 19, "end_idx": 34, "type": "bod", "entity": "维生素B1" } ] }, { "text": "(四)空腹一次测定尿液中的维生素B1/肌酐比率其正常值为176μg/g肌酐,幼儿如低于120μg/g肌酐,4~12岁小儿低于60μg/g肌酐则为维生素B1缺乏。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "尿液" }, { "start_idx": 13, "end_idx": 33, "type": "ite", "entity": "维生素B1/肌酐比率" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "肌酐" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "肌酐" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "肌酐" }, { "start_idx": 83, "end_idx": 100, "type": "dis", "entity": "维生素B1缺乏" } ] }, { "text": "【诊断】依靠病史、临床症状和体征、实验室检查和实验性维生素B1治疗可作出可靠诊断。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "体征" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "实验室检查" }, { "start_idx": 23, "end_idx": 43, "type": "pro", "entity": "实验性维生素B1治疗" } ] }, { "text": "(一)病史患儿是否有维生素B1摄入不足,已添加辅食的小儿,是否有长期食用精白米、面及有无偏食。", "entities": [ { "start_idx": 10, "end_idx": 25, "type": "dru", "entity": "维生素B1" } ] }, { "text": "有无妨碍维生素B1吸收和利用的疾病,如慢性消耗疾病、胃肠道疾病、肝胆系统疾病等。", "entities": [ { "start_idx": 4, "end_idx": 19, "type": "dru", "entity": "维生素B1" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "慢性消耗疾病" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "胃肠道疾病" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "肝胆系统疾病" } ] }, { "text": "患者是否存在硫胺素需要量增加的因素,如生长发育阶段、发热及甲状腺功能亢进等。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "硫胺素" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "发热" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "甲状腺功能亢进" } ] }, { "text": "(二)临床特点有无周围神经炎的表现,如肌肉萎缩、感觉异常、跟腱及膝反射异常。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "周围神经炎" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肌肉" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "肌肉萎缩" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "感觉异常" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "跟腱" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "膝" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "跟腱及膝反射异常" } ] }, { "text": "有无进行性水肿、心脏扩张肥厚、心率增加、脉压加大。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "进行性水肿" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心脏" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "心脏扩张肥厚" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "心率" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "心率增加" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "脉压" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "脉压加大" } ] }, { "text": "能除外其他心脏病的心力衰竭。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "心脏病" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "心力衰竭" } ] }, { "text": "有无其他营养缺乏的征象。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "营养缺乏" } ] }, { "text": "(三)实验室检验可通过测定维生素B1负荷前后尿维生素B1排泄量、血清维生素B1水平、红细胞转酮醇酶(ETK)活性及空腹一次测定尿液中的维生素B1/肌酐比率等实验室检查帮助诊断。", "entities": [ { "start_idx": 13, "end_idx": 28, "type": "dru", "entity": "维生素B1" }, { "start_idx": 33, "end_idx": 52, "type": "ite", "entity": "尿维生素B1排泄量" }, { "start_idx": 54, "end_idx": 73, "type": "ite", "entity": "血清维生素B1水平" }, { "start_idx": 75, "end_idx": 88, "type": "ite", "entity": "红细胞转酮醇酶(ETK)活性" }, { "start_idx": 96, "end_idx": 97, "type": "bod", "entity": "尿液" }, { "start_idx": 100, "end_idx": 120, "type": "ite", "entity": "维生素B1/肌酐比率" }, { "start_idx": 122, "end_idx": 126, "type": "pro", "entity": "实验室检查" } ] }, { "text": "【治疗和预防】(一)去除病因仔细询问病史,查明缺乏维生素B1的原因,治疗造成维生素B1缺乏的原发性疾病,如发热、感染、甲状腺功能亢进等。", "entities": [ { "start_idx": 25, "end_idx": 40, "type": "bod", "entity": "维生素B1" }, { "start_idx": 49, "end_idx": 66, "type": "sym", "entity": "维生素B1缺乏" }, { "start_idx": 75, "end_idx": 76, "type": "dis", "entity": "发热" }, { "start_idx": 78, "end_idx": 79, "type": "dis", "entity": "感染" }, { "start_idx": 81, "end_idx": 87, "type": "dis", "entity": "甲状腺功能亢进" } ] }, { "text": "(二)饮食增加含维生素B1丰富的食物的摄入量,并注意合理配合。", "entities": [ { "start_idx": 8, "end_idx": 23, "type": "dru", "entity": "维生素B1" } ] }, { "text": "如果乳母维生素B1缺乏,应及时予以补充,避免婴儿发生维生素B1缺乏症。", "entities": [ { "start_idx": 4, "end_idx": 21, "type": "sym", "entity": "维生素B1缺乏" }, { "start_idx": 37, "end_idx": 55, "type": "dis", "entity": "维生素B1缺乏症" } ] }, { "text": "未精制的粮谷类中维生素B1丰富,故碾磨精度不宜过度。", "entities": [ { "start_idx": 8, "end_idx": 23, "type": "dru", "entity": "维生素B1" } ] }, { "text": "豆类、坚果类、瘦肉及内脏维生素B1也较为丰富。", "entities": [ { "start_idx": 12, "end_idx": 27, "type": "dru", "entity": "维生素B1" } ] }, { "text": "蛋类、绿叶菜(芹菜叶、莴笋叶)等也是维生素B1的良好来源,应充分加以利用。", "entities": [ { "start_idx": 18, "end_idx": 33, "type": "dru", "entity": "维生素B1" } ] }, { "text": "(三)应用维生素B1治疗小儿症状较轻,一般维生素B1的剂量为5mg/d;重症则需10mg/d静脉注射,每天2次,如症状缓解,则可改为口服。", "entities": [ { "start_idx": 5, "end_idx": 20, "type": "dru", "entity": "维生素B1" }, { "start_idx": 32, "end_idx": 47, "type": "dru", "entity": "维生素B1" }, { "start_idx": 68, "end_idx": 71, "type": "pro", "entity": "静脉注射" }, { "start_idx": 88, "end_idx": 89, "type": "pro", "entity": "口服" } ] }, { "text": "用维生素B1治疗,神经症状一般于24小时内缓解,心脏症状一般于24~48小时缓解,而水肿则需48~72小时缓解,运动无力的恢复一般时间较长,需1~3个月。", "entities": [ { "start_idx": 1, "end_idx": 16, "type": "dru", "entity": "维生素B1" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "神经" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "心脏" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "水肿" }, { "start_idx": 67, "end_idx": 70, "type": "sym", "entity": "运动无力" } ] }, { "text": "如口服有严重不能耐受的不良反应;长期腹泻、呕吐或大部分小肠切除后需要全肠外营养维持者可通过肠外途径予以补充。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "口服" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "长期腹泻、呕吐" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "小肠" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "全肠" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "肠" } ] }, { "text": "九、其他微生物感染性肺炎(一)支原体肺炎胸片改变多样化,但以单个的密度增高的大片状阴影为主,边缘模糊,常有胸膜反应,病灶吸收缓慢。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "微生物感染性肺炎" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "支原体肺炎" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "胸片" }, { "start_idx": 30, "end_idx": 42, "type": "sym", "entity": "单个的密度增高的大片状阴影" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "边缘模糊" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "胸膜反应" }, { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "病灶吸收缓慢" } ] }, { "text": "(二)衣原体肺炎常以细小点片状阴影出现,分布在两肺野,大多数两侧对称性分布,新生儿时期常与粟粒性肺结核相混淆,需提高警惕。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "衣原体肺炎" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "细小点片状阴影" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肺野" }, { "start_idx": 20, "end_idx": 36, "type": "sym", "entity": "分布在两肺野,大多数两侧对称性分布" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "粟粒性肺结核" } ] }, { "text": "(三)真菌性肺炎病变弥漫广泛,其形态为小斑片状、结节状、绒毛状不等,边缘尚清,病灶内可出现空洞,有时出现胸膜炎、胸腔积液。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "真菌性肺炎" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "边缘尚清" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "病灶内可出现空洞" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "胸膜炎" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "胸腔积液" } ] }, { "text": "第三节隐球菌病新型隐球菌(cryptococcusneoformans)是隐球菌病的病原菌。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "隐球菌病" }, { "start_idx": 7, "end_idx": 11, "type": "mic", "entity": "新型隐球菌" }, { "start_idx": 13, "end_idx": 34, "type": "mic", "entity": "cryptococcusneoformans" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "隐球菌病" }, { "start_idx": 42, "end_idx": 44, "type": "mic", "entity": "病原菌" } ] }, { "text": "该菌首次由Buschke于1894年在德国从一全身芽生菌病患者身上分离出的。", "entities": [ { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "全身芽生菌病" } ] }, { "text": "主要侵犯肺和中枢神经系统,侵犯皮肤、骨骼等比较少见。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "肺" }, { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "皮肤" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "骨骼" } ] }, { "text": "【临床流行病学】隐球菌属有17个种和9个变种,分布广泛,其中只有新型隐球菌是人类的病原菌。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "隐球菌" }, { "start_idx": 32, "end_idx": 36, "type": "mic", "entity": "新型隐球菌" }, { "start_idx": 41, "end_idx": 43, "type": "mic", "entity": "病原菌" } ] }, { "text": "新型隐球菌为酵母菌,在寄生状态下,可见圆球形带宽阔荚膜的菌体,部分菌体可有出芽,菌体内可见一个或多个反光颗粒。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "新型隐球菌" }, { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "酵母菌" } ] }, { "text": "与非致病隐球菌不同的是,它能在37℃的环境中生长。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "mic", "entity": "非致病隐球菌" } ] }, { "text": "新型隐球菌荚膜的血清型(A~D和AD)与致病性有关,我国有A、B、D共3型存在,且以A型为最多见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "新型隐球菌" } ] }, { "text": "从患者分离到的新型隐球菌多是A型和D型。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "mic", "entity": "新型隐球菌" } ] }, { "text": "此菌是土壤、鸽子粪、牛乳、水果的腐生菌,可从呼吸道、消化道、皮肤等进入人体引起隐球菌病。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "mic", "entity": "腐生菌" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "呼吸道" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "消化道" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "皮肤" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "隐球菌病" } ] }, { "text": "隐球菌分布很广,世界各地均有报道,在我国也有发现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "隐球菌" } ] }, { "text": "尚未证实人与人之间传播的隐球菌感染。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "隐球菌感染" } ] }, { "text": "虽然在免疫正常的儿童可以发生播散型隐球菌病,但感染最常发生在有细胞免疫缺陷的免疫抑制患者,如先天性或获得性免疫缺陷病、器官移植的患者、胶原性血管性疾病、应用皮质类固醇等。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "播散型隐球菌病" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "细胞免疫缺陷" }, { "start_idx": 46, "end_idx": 57, "type": "dis", "entity": "先天性或获得性免疫缺陷病" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "器官移植" }, { "start_idx": 67, "end_idx": 74, "type": "dis", "entity": "胶原性血管性疾病" }, { "start_idx": 78, "end_idx": 82, "type": "dru", "entity": "皮质类固醇" } ] }, { "text": "有报道儿童最常见的原发疾病是急性淋巴细胞白血病、系统性红斑狼疮,以及恶性肿瘤和呼吸道疾病等。", "entities": [ { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "急性淋巴细胞白血病" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "呼吸道疾病" } ] }, { "text": "【发病机制和病理生理】存在于土壤及鸽粪中的隐球菌,可随尘埃一起被人吸入。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "隐球菌" } ] }, { "text": "隐球菌直径仅1μm,能进入肺泡。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "隐球菌" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肺泡" } ] }, { "text": "隐球菌在体外无荚膜包裹,进入体内后很快形成荚膜,带荚膜的隐球菌具有致病力。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "隐球菌" }, { "start_idx": 28, "end_idx": 30, "type": "mic", "entity": "隐球菌" } ] }, { "text": "侵入人体的隐球菌不一定致病,细胞免疫在防止隐球菌感染中起主要的作用。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "mic", "entity": "隐球菌" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "隐球菌感染" } ] }, { "text": "免疫功能低下的各种疾病如白血病、淋巴瘤、结节病、糖尿病等患者,均易继发隐球菌病。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "白血病" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "结节病" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "糖尿病" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "隐球菌病" } ] }, { "text": "隐球菌侵入肺部后,局部病变进展缓慢,常无临床症状,但可由此经血进入中枢神经系统,导致隐球菌病中最为常见的脑膜炎。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "隐球菌" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "肺部" }, { "start_idx": 33, "end_idx": 38, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "隐球菌病" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "颅底软脑膜病变较显著,蛛网膜下腔有广泛的渗出物积聚,内含单核细胞、淋巴细胞及隐球菌等。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "颅底软脑膜病变较显著" }, { "start_idx": 11, "end_idx": 24, "type": "sym", "entity": "蛛网膜下腔有广泛的渗出物积聚" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "单核细胞" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 38, "end_idx": 40, "type": "mic", "entity": "隐球菌" } ] }, { "text": "也可形成局限性肉芽肿,后者是机体反应较强的表现,由组织细胞、巨细胞、淋巴样细胞及成纤维细胞组成;隐球菌较少发现,大多存在于巨细胞和组织细胞内,引起脑干的血管炎,导致局部脑组织缺血和软化,脑实质内亦可形成肉芽肿。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "局限性肉芽肿" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "组织细胞" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "巨细胞" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "淋巴样细胞" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "成纤维细胞" }, { "start_idx": 48, "end_idx": 50, "type": "mic", "entity": "隐球菌" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "巨细胞" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "组织细胞" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "脑干" }, { "start_idx": 76, "end_idx": 78, "type": "dis", "entity": "血管炎" }, { "start_idx": 82, "end_idx": 91, "type": "sym", "entity": "局部脑组织缺血和软化" }, { "start_idx": 93, "end_idx": 95, "type": "bod", "entity": "脑实质" }, { "start_idx": 101, "end_idx": 103, "type": "dis", "entity": "肉芽肿" } ] }, { "text": "隐球菌也可在血管周围间隙中增殖并在灰质内形成许多肉眼可见的囊肿,囊肿内充满隐球菌。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "隐球菌" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血管" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "灰质" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "肉眼可见的囊肿" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "囊肿内充满隐球菌" } ] }, { "text": "肺部病变可以很轻微,仅有少量淋巴细胞浸润。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "慢性病灶常有巨噬细胞浸润,但很少形成化脓灶或呈纤维化者,少数病例可表现为局灶性或广泛性小肉芽肿。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "慢性病灶常有巨噬细胞浸润" }, { "start_idx": 14, "end_idx": 25, "type": "sym", "entity": "很少形成化脓灶或呈纤维化" }, { "start_idx": 28, "end_idx": 46, "type": "sym", "entity": "少数病例可表现为局灶性或广泛性小肉芽肿" } ] }, { "text": "皮肤病变有肉芽肿及胶质性损害两种类型,后者的组织反应较少,内含大量隐球菌。", "entities": [ { "start_idx": 33, "end_idx": 35, "type": "mic", "entity": "隐球菌" } ] }, { "text": "正常人血浆中α2和γ球蛋白内含抗隐球菌生长因子,具有抑制新型隐球菌生长的能力。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "血浆" }, { "start_idx": 21, "end_idx": 28, "type": "bod", "entity": "抗隐球菌生长因子" }, { "start_idx": 34, "end_idx": 38, "type": "mic", "entity": "新型隐球菌" } ] }, { "text": "因此只有在机体抵抗力下降时,血清抗隐球菌能力下降时,病原菌才易进入人体致病。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "血清" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "隐球菌" } ] }, { "text": "【临床表现】(一)肺部感染肺部隐球菌病为亚急性或慢性过程,而且常无症状,可能在偶然胸部X线检查中被发现。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "肺部感染" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "肺部隐球菌病" }, { "start_idx": 41, "end_idx": 46, "type": "pro", "entity": "胸部X线检查" } ] }, { "text": "有报道新型隐球菌可在肺部定植,但不侵袭肺实质。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "mic", "entity": "新型隐球菌" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肺部" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "肺实质" } ] }, { "text": "明显的肺部病变引起的症状有咳嗽、咯血和胸痛而且伴有全身症状。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "咳嗽" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "咯血" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "胸痛" } ] }, { "text": "胸部X线最常见的有3种类型:①孤立病灶,类似于肿瘤;②散在的小结节病灶,与其他真菌感染、粟粒性肺结核、结节病相似;③浸润性病灶,与浸润性肺结核类似,要与卡氏肺孢子虫鉴别。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "胸部X线" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "孤立病灶" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "肿瘤" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "散在的小结节病灶" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "真菌感染" }, { "start_idx": 44, "end_idx": 49, "type": "dis", "entity": "粟粒性肺结核" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "结节病" }, { "start_idx": 58, "end_idx": 62, "type": "sym", "entity": "浸润性病灶" }, { "start_idx": 65, "end_idx": 70, "type": "dis", "entity": "浸润性肺结核" }, { "start_idx": 76, "end_idx": 81, "type": "mic", "entity": "卡氏肺孢子虫" } ] }, { "text": "尽管有的病例可出现空洞,但钙化和纤维化罕见。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "钙化" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "纤维化" } ] }, { "text": "肺部病变在诊断中作为播散性病变的原发病灶比单独感染灶更为常见。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" } ] }, { "text": "(二)神经系统表现中枢神经系统是临床上最常见的感染部位。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "长期颅压高可有明显的视神经乳头水肿,晚期视神经继发性萎缩而致视力减退,脑神经受累可出现复视、视野缺损、眼肌麻痹或眼球震颤等表现。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "颅压高" }, { "start_idx": 18, "end_idx": 27, "type": "dis", "entity": "晚期视神经继发性萎缩" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "视力减退" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "脑神经" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "复视" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "视野缺损" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "眼肌麻痹" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "眼球震颤" } ] }, { "text": "但有10%的病例完全不出现神经系统症状。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "神经系统" } ] }, { "text": "隐球菌肉芽肿发生在大脑,相应的体征与肿瘤、脓肿、硬膜下血肿或脑疝相似,也可发生脑积水。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "隐球菌肉芽肿" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "肿瘤" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "脓肿" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "硬膜下血肿" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "脑疝" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "脑积水" } ] }, { "text": "中枢神经系统隐球菌病常被误诊为结核性脑膜炎而延误治疗造成死亡。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "中枢神经系统隐球菌病" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "结核性脑膜炎" } ] }, { "text": "脑脊液检查与结核性脑膜炎不易区分,70%隐球菌性脑膜炎患者脑脊液压力增高。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "隐球菌性脑膜炎" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "脑脊液压力增高" } ] }, { "text": "慢性患者可在正常范围内,外观清澈、透明或微混,白细胞计数轻至中度增多,早期以中性粒细胞为主,中期以淋巴细胞为主,可达88%~90%,主要为淋巴细胞、单核细胞等。", "entities": [ { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "外观清澈、透明或微混" }, { "start_idx": 23, "end_idx": 33, "type": "sym", "entity": "白细胞计数轻至中度增多" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 69, "end_idx": 72, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 74, "end_idx": 77, "type": "bod", "entity": "单核细胞" } ] }, { "text": "糖和氯化物早期变化不明显,中后期明显下降,尤其是糖。", "entities": [ { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "糖和氯化物早期变化不明显,中后期明显下降,尤其是糖" } ] }, { "text": "蛋白常在2g/L以上。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "蛋白常在2g/L以上" } ] }, { "text": "隐球菌脑膜炎的鉴别包括其他真菌引起的脑膜炎、结核性脑膜炎、布鲁菌病、淋巴瘤和淋巴肉瘤。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "隐球菌脑膜炎" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "真菌" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "布鲁菌病" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "淋巴肉瘤" } ] }, { "text": "(三)皮肤表现是其他部位隐球菌的一个局部表现,也可经直接接触感染而患病。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "mic", "entity": "隐球菌" } ] }, { "text": "多为血行播散的结果,播散性病变患者中有10%出现隐球菌性皮肤损害。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "播散性病变" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "隐球菌性皮肤损害" } ] }, { "text": "从骨骼病灶直接扩散到皮肤也可发生。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "骨骼" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "皮肤" } ] }, { "text": "皮损初为软疣样或粉刺状皮疹,结节或脓肿,边界清楚,无红晕,继而中央破溃,溃疡有隆起的乳头瘤样边缘,流出少量带黏液血性的脓液,内含隐球菌。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "皮损" }, { "start_idx": 4, "end_idx": 12, "type": "sym", "entity": "软疣样或粉刺状皮疹" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "边界清楚" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "无红晕" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "中央破溃" }, { "start_idx": 36, "end_idx": 47, "type": "sym", "entity": "溃疡有隆起的乳头瘤样边缘" }, { "start_idx": 49, "end_idx": 60, "type": "sym", "entity": "流出少量带黏液血性的脓液" }, { "start_idx": 64, "end_idx": 66, "type": "mic", "entity": "隐球菌" } ] }, { "text": "头面部和头皮是最常见的皮损部位,但全身其他部位均可受累。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "头面部" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "头皮" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "皮损" } ] }, { "text": "(四)脉络膜视网膜炎主要发生在成人,多为播散性疾病的一种征象。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "脉络膜视网膜炎" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "播散性疾病" } ] }, { "text": "眼部症状有视力急性减退、眼痛、悬物浮动感、畏光等,检查常有脉络膜炎伴或不伴视网膜炎,而视网膜玻璃体肿块和前葡萄膜炎不多见。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "眼部" }, { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "视力急性减退" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "眼痛" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "悬物浮动感" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "畏光" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "脉络膜炎" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "视网膜炎" }, { "start_idx": 43, "end_idx": 50, "type": "dis", "entity": "视网膜玻璃体肿块" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "前葡萄膜炎" } ] }, { "text": "(五)其他部位的感染播散性病变10%有骨损害,可出现疼痛和肿胀,X线表现与骨源性肉瘤易混淆。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "播散性病变" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "骨损害" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "疼痛" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "肿胀" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "X线" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "骨源性肉瘤" } ] }, { "text": "可能出现隐球菌病的其他组织器官有心脏、肾上腺、肝、肾、淋巴结和关节等。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "隐球菌病" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "心脏" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "肾上腺" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "肝" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肾" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "淋巴结" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "关节" } ] }, { "text": "【实验室检查】(一)病原体检查1.真菌检查墨汁染色最敏感,显微镜下见隐球菌圆形孢子,直径5~20μm,内有反光颗粒,外围一层厚膜,有时可见出芽孢子,但无菌丝。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "真菌检查" }, { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "墨汁染色最敏感" }, { "start_idx": 29, "end_idx": 40, "type": "sym", "entity": "显微镜下见隐球菌圆形孢子" } ] }, { "text": "取脑脊液3~5ml,离心沉淀后检查最容易发现出芽的真菌,所需的酵母菌的数量至少为103cuf/ml。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "脑脊液" }, { "start_idx": 31, "end_idx": 33, "type": "mic", "entity": "酵母菌" } ] }, { "text": "如怀疑肺部隐球菌病,应做痰液、支气管肺泡灌洗液或肺组织的真菌检查。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "肺部隐球菌病" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "痰液" }, { "start_idx": 15, "end_idx": 22, "type": "bod", "entity": "支气管肺泡灌洗液" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肺组织" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "真菌检查" } ] }, { "text": "2.活组织检查和真菌培养活检组织标本做嗜银染色、爱茜蓝染色、黏蛋白胭脂红染色和嗜碘酸-锡夫染色也可确定病原体。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "活组织检查" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "真菌培养" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "嗜银染色" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "爱茜蓝染色" }, { "start_idx": 30, "end_idx": 37, "type": "pro", "entity": "黏蛋白胭脂红染色" }, { "start_idx": 39, "end_idx": 46, "type": "pro", "entity": "嗜碘酸-锡夫染色" } ] }, { "text": "溶解离心技术能增加血培养中酵母菌的检出率。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "溶解离心技术" }, { "start_idx": 13, "end_idx": 15, "type": "mic", "entity": "酵母菌" } ] }, { "text": "3.动物接种小白鼠最敏感,将脑脊液或其他标本进行腹腔注射、尾静脉注射或颅内注射,小白鼠在2~8周内死亡,脑内可见大量的隐球菌。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "动物接种" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "脑脊液" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "腹腔注射" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "尾静脉注射" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "颅内注射" }, { "start_idx": 59, "end_idx": 61, "type": "mic", "entity": "隐球菌" } ] }, { "text": "(二)血清学检查1.抗原检测目前选择的乳胶凝集试验可检测隐球菌的多糖荚膜抗原。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "血清学检查" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "抗原检测" }, { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "乳胶凝集试验" }, { "start_idx": 28, "end_idx": 30, "type": "mic", "entity": "隐球菌" } ] }, { "text": "隐球菌脑膜炎的脑脊液中抗原检测的敏感性至少为90%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "隐球菌脑膜炎" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "脑脊液" } ] }, { "text": "对诊断肺部隐球菌病患者出现血清隐球菌抗原检测阳性,应高度怀疑有播散性感染的可能。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "肺部隐球菌病" }, { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "血清隐球菌抗原检测阳性" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "播散性感染" } ] }, { "text": "2.抗体检测由于患者血清中可测到的抗体不多,因此检测抗体的阳性率不高,且特异性不强,假阳性太高。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "抗体检测" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "血清" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "抗体" }, { "start_idx": 24, "end_idx": 46, "type": "sym", "entity": "检测抗体的阳性率不高,且特异性不强,假阳性太高" } ] }, { "text": "但是抗体检测仍可作为辅助诊断,如抗体检测为阳性,而抗原阴性,说明病情较轻或药物疗效良好。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "抗体检测" }, { "start_idx": 16, "end_idx": 28, "type": "sym", "entity": "抗体检测为阳性,而抗原阴性" } ] }, { "text": "【诊断】确诊新型隐球菌感染必须从体液或组织中分离出病原菌。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "新型隐球菌感染" }, { "start_idx": 15, "end_idx": 27, "type": "sym", "entity": "从体液或组织中分离出病原菌" } ] }, { "text": "经多次血培养和尿培养,有1/3的非艾滋病患者可作出播散性疾病的诊断。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "血培养" }, { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "尿培养" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "艾滋病" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "播散性疾病" } ] }, { "text": "对中枢神经系统隐球菌病作出早期正确诊断时,应注意:凡未确诊的脑膜炎、脑炎及脑占位病变,在脑脊液检查时应常规进行真菌检查;结核性脑膜脑炎,抗结核治疗无效应时应怀疑本病;血液性疾病、肿瘤、系统性红斑狼疮、器官移植等,在病程中突然出现头痛、恶心、呕吐应怀疑中枢神经系统隐球菌感染。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "dis", "entity": "中枢神经系统隐球菌病" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "脑炎" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "脑占位病变" }, { "start_idx": 44, "end_idx": 48, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 55, "end_idx": 58, "type": "pro", "entity": "真菌检查" }, { "start_idx": 60, "end_idx": 66, "type": "dis", "entity": "结核性脑膜脑炎" }, { "start_idx": 68, "end_idx": 72, "type": "pro", "entity": "抗结核治疗" }, { "start_idx": 83, "end_idx": 87, "type": "dis", "entity": "血液性疾病" }, { "start_idx": 89, "end_idx": 90, "type": "dis", "entity": "肿瘤" }, { "start_idx": 92, "end_idx": 98, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 100, "end_idx": 103, "type": "dis", "entity": "器官移植" }, { "start_idx": 114, "end_idx": 115, "type": "sym", "entity": "头痛" }, { "start_idx": 117, "end_idx": 118, "type": "sym", "entity": "恶心" }, { "start_idx": 120, "end_idx": 121, "type": "sym", "entity": "呕吐" }, { "start_idx": 125, "end_idx": 135, "type": "dis", "entity": "中枢神经系统隐球菌感染" } ] }, { "text": "【治疗】应注意,抗真菌治疗的一个重要的辅助治疗是减少或停用免疫抑制药物,特别是肾上腺皮质类固醇。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "抗真菌治疗" }, { "start_idx": 24, "end_idx": 34, "type": "pro", "entity": "减少或停用免疫抑制药物" }, { "start_idx": 39, "end_idx": 46, "type": "dru", "entity": "肾上腺皮质类固醇" } ] }, { "text": "(一)抗真菌药物1.两性霉素B静脉滴注用于中枢神经系统隐球菌病或其他内脏隐球菌病。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 21, "end_idx": 30, "type": "dis", "entity": "中枢神经系统隐球菌病" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "其他内脏隐球菌病" } ] }, { "text": "用药前必须以5%的葡萄糖稀释至5~10mg/dl,缓缓静脉滴注,隔日一次,每次滴注时间应不少于6小时,滴注速度过快可发生抽搐、心室纤颤、过敏性反应、血压降低和心跳停止。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "葡萄糖" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "滴注" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "滴注" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "抽搐" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "心室纤颤" }, { "start_idx": 68, "end_idx": 72, "type": "sym", "entity": "过敏性反应" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "血压降低" }, { "start_idx": 79, "end_idx": 82, "type": "sym", "entity": "心跳停止" } ] }, { "text": "反应重者,于静脉滴入氢化可的松或地塞米松,或减少本药的剂量,延长疗程,亦不影响治疗效果。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "静脉滴入" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "氢化可的松" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "地塞米松" } ] }, { "text": "非HIV感染伴有中枢神经系统或其他症状的播散性隐球菌感染患者可用两性霉素B和氟胞嘧啶联合治疗。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "HIV感染" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "播散性隐球菌感染" }, { "start_idx": 32, "end_idx": 36, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "氟胞嘧啶" } ] }, { "text": "两性霉素B总量应超过15mg/kg,氟胞嘧啶50~150mg/(kg•d)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "氟胞嘧啶" } ] }, { "text": "HIV感染患者播散性隐球菌感染时因复发率较高,需要诱导和维持疗法,最好的方案是两性霉素B伴或不伴氟胞嘧啶。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "HIV感染" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "播散性隐球菌感染" }, { "start_idx": 25, "end_idx": 31, "type": "pro", "entity": "诱导和维持疗法" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 48, "end_idx": 51, "type": "dru", "entity": "氟胞嘧啶" } ] }, { "text": "本药毒性大,适当治疗后即可停药,停药原则是:脑脊液墨汁涂片检查转阴3~4周;临床上可恢复的主要症状体征已消失;总剂量已达1.5~3g;疗程至少6周。", "entities": [ { "start_idx": 22, "end_idx": 36, "type": "sym", "entity": "脑脊液墨汁涂片检查转阴3~4周" } ] }, { "text": "椎管内注射:限于治疗中枢神经系统隐球菌病。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "椎管内注射" }, { "start_idx": 10, "end_idx": 19, "type": "dis", "entity": "中枢神经系统隐球菌病" } ] }, { "text": "适用于病情严重或静脉注射未成功者。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "静脉注射" } ] }, { "text": "注射时,将药物与腰椎穿刺引流出的脑脊液3~5ml混匀后,缓缓注入蛛网膜下腔。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "注射" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "脑脊液" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "蛛网膜下腔" } ] }, { "text": "注意给药过程中的不良反应,如尿潴留甚至暂时性截瘫等,一般立即停药即可缓解。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "尿潴留" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "暂时性截瘫" } ] }, { "text": "鞘内注射时加地塞米松每次1~2mg。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "鞘内注射" }, { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "地塞米松" } ] }, { "text": "2.氟胞嘧啶连续应用具有抗隐球菌的作用,但因耐药性出现很快而较少单独使用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "氟胞嘧啶" }, { "start_idx": 13, "end_idx": 15, "type": "mic", "entity": "隐球菌" } ] }, { "text": "在组织中均可达到较高浓度,口服剂量50~100mg/(kg•d),分3~4次服。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "口服" } ] }, { "text": "3.氟康唑有很好的血-脑屏障通透性,能达到极佳的脑脊液浓度。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "氟康唑" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "血-脑屏障" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "脑脊液" } ] }, { "text": "但氟康唑耐药菌株出现应予重视。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "氟康唑" } ] }, { "text": "该药副作用较轻,少数患者可出现恶心、皮疹、肝酶升高和血钾降低等。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "恶心" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "皮疹" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "肝酶升高" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "血钾降低" } ] }, { "text": "4.伊曲康唑是广谱三唑类抗真菌药,不易通过血-脑屏障进入脑脊液,但在脑组织中有较高的浓度。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "伊曲康唑" }, { "start_idx": 7, "end_idx": 15, "type": "dru", "entity": "广谱三唑类抗真菌药" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "血-脑屏障" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "脑脊液" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "脑组织" } ] }, { "text": "在神经系统隐球菌病的治疗中,主张与两性霉素B合用或作为脑脊液转阴后的维持治疗。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "神经系统隐球菌病" }, { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "脑脊液" } ] }, { "text": "副作用:少数患者出现恶心、呕吐、皮疹和肝酶升高等,但一般不影响治疗。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "恶心" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "呕吐" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "皮疹" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "肝酶升高" } ] }, { "text": "经过1~2周治疗后,脑脊液中或原发感染部位的隐球菌培养转阴,说明治疗有效。", "entities": [ { "start_idx": 10, "end_idx": 28, "type": "sym", "entity": "脑脊液中或原发感染部位的隐球菌培养转阴" } ] }, { "text": "初次治疗应持续至少6周,微生物学检查阴性后最少再治疗4周。", "entities": [ { "start_idx": 12, "end_idx": 19, "type": "sym", "entity": "微生物学检查阴性" } ] }, { "text": "提示隐球菌预后不良的因素有:①治疗前血清或脑脊液隐球菌抗原滴度≥1∶32;②脑脊液中糖降低;③脑脊液中白细胞<20/μl;④脑脊液压力增高;⑤治疗前墨汁染色阳性;⑥治疗后血清或脑脊液隐球菌抗原滴度≥1∶8。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "隐球菌" }, { "start_idx": 15, "end_idx": 35, "type": "sym", "entity": "治疗前血清或脑脊液隐球菌抗原滴度≥1∶32" }, { "start_idx": 38, "end_idx": 59, "type": "sym", "entity": "脑脊液中糖降低;③脑脊液中白细胞<20/μl" }, { "start_idx": 62, "end_idx": 68, "type": "sym", "entity": "脑脊液压力增高" }, { "start_idx": 71, "end_idx": 79, "type": "sym", "entity": "治疗前墨汁染色阳性" }, { "start_idx": 82, "end_idx": 101, "type": "sym", "entity": "治疗后血清或脑脊液隐球菌抗原滴度≥1∶8" } ] }, { "text": "骨骼感染时,除全身抗真菌药外一般均需外科清创;皮肤感染在诊断上需外科活检,药物治疗;脉络膜视网膜炎治疗同样需要全身抗真菌治疗,可用氟康唑或氟胞嘧啶,此两药在玻璃体中可获得高浓度。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "骨骼感染" }, { "start_idx": 7, "end_idx": 12, "type": "dru", "entity": "全身抗真菌药" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "外科清创" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "皮肤感染" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "外科活检" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "脉络膜视网膜炎" }, { "start_idx": 55, "end_idx": 61, "type": "pro", "entity": "全身抗真菌治疗" }, { "start_idx": 65, "end_idx": 67, "type": "dru", "entity": "氟康唑" }, { "start_idx": 69, "end_idx": 72, "type": "dru", "entity": "氟胞嘧啶" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "玻璃体" } ] }, { "text": "(二)其他疗法局限性病灶如皮肤、胸部肉芽肿及脓疡,或肺部肉芽肿及空洞等在未合并中枢神经系统隐球菌病的情况下,可以考虑手术切除。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "局限性病灶" }, { "start_idx": 13, "end_idx": 23, "type": "dis", "entity": "皮肤、胸部肉芽肿及脓疡" }, { "start_idx": 26, "end_idx": 33, "type": "dis", "entity": "肺部肉芽肿及空洞" }, { "start_idx": 39, "end_idx": 48, "type": "dis", "entity": "中枢神经系统隐球菌病" }, { "start_idx": 58, "end_idx": 61, "type": "pro", "entity": "手术切除" } ] }, { "text": "于术前术后均需用两性霉素B,以控制隐球菌感染。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "隐球菌感染" } ] }, { "text": "【预后】隐球菌脑膜炎虽然偶见有两年以上仍反复发作而迁延不愈者,但如不治疗多在3个月至半年左右趋于恶化,出现一系列运动障碍如偏瘫、失语、共济失调等;精神错乱以致抽风昏迷,最后因呼吸衰竭死亡。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "隐球菌脑膜炎" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "偏瘫" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "失语" }, { "start_idx": 67, "end_idx": 70, "type": "sym", "entity": "共济失调" }, { "start_idx": 73, "end_idx": 76, "type": "sym", "entity": "精神错乱" }, { "start_idx": 79, "end_idx": 82, "type": "sym", "entity": "抽风昏迷" }, { "start_idx": 87, "end_idx": 90, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "近40%的患者留有神经系统后遗症,如视神经炎、动眼神经损害,部分有人格和脑功能的改变。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "神经系统后遗症" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "视神经炎" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "动眼神经损害" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "人格和脑功能的改变" } ] }, { "text": "隐球菌性脑膜炎发生在有基础疾病时的病死率为55%,无基础性疾病的病死率为25%。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "隐球菌性脑膜炎" } ] }, { "text": "长期应用糖皮质激素及其他免疫抑制剂影响预后,易复发。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "【预防】新型隐球菌患者无需隔离,目前尚无有效的控制措施,但免疫损伤者应避免接触有鸽子粪便污染的区域。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "mic", "entity": "新型隐球菌" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "隔离" }, { "start_idx": 35, "end_idx": 48, "type": "pro", "entity": "避免接触有鸽子粪便污染的区域" } ] }, { "text": "参考文献1.FischM.Surgeryinrhabdomyosarcomaofthebladder,prostateandvagina.WorldJUrol,1995,13(4):213-2132.GerLP.Cancerpatients'knowledgeoftheirdiagnoses.JFormosMedAssoc,1996,95(8):605-6113.IczkowskiKA.Inflammatorypseudotumorandsarcomaofurinarybladder:differentialdiagnosisandoutcomeinthirtyeightspindlecellneoplasmas.ModPathol,2001,14(10):1043-10434.JulianMoraM.Conservativetreatmentofbladderandprostaticrhabdomyosarcomainchildhood:possibilitiesfornon-radicalsurgery.ArchEspUrol,1991,44(6):719-7195.KamiiY.Primarychemotherapyforchildrenwithrhabdomyosarcomaofthespecialpelvicsites:ispreservationofthebladderpossible?", "entities": [ { "start_idx": 13, "end_idx": 49, "type": "dis", "entity": "Surgeryinrhabdomyosarcomaofthebladder" }, { "start_idx": 195, "end_idx": 217, "type": "dis", "entity": "Inflammatorypseudotumor" }, { "start_idx": 221, "end_idx": 243, "type": "dis", "entity": "sarcomaofurinarybladder" }, { "start_idx": 534, "end_idx": 553, "type": "dis", "entity": "rhabdomyosarcomaofth" } ] }, { "text": "JPediatrSurg,1994,29(3):461-4646.RaneyRBJr.Primarychemotherapywithorwithoutradiationtherapyand/orsurgeryforchildrenwithlocalizedsarcomaofthebladder,prostate,vagina,uterus,andcervix.AcomparisonoftheresultsinIntergroupRhabdomyosarcomaStudiesⅠandⅡ.Cancer,1990,66(10):2072-20727.RaneyBJr.Sequelaeoftreatmentin109patientsfollowedfor5to15yearsafterdiagnosisofsarcomaofthebladderandprostate.AreportfromtheIntergroupRhabdomyosarcomaStudyCommittee.Cancer,1993,71(7):2387-23878.SuzukiY.Hemangiomaofthebladderwithextravesicalextension.UrolInt,1997,59(2):125-1259.SweetserPM.Pheochromocytomaoftheurinarybladder.Surgery,1991,109(5):677-68110.WuTT.Invertedpapillomaoftheurinarybladder.ZhonghuaYiXueZaZhi(Taipei),1996,57(1):59-63", "entities": [ { "start_idx": 140, "end_idx": 146, "type": "bod", "entity": "bladder" }, { "start_idx": 148, "end_idx": 155, "type": "bod", "entity": "prostate" }, { "start_idx": 157, "end_idx": 162, "type": "bod", "entity": "vagina" }, { "start_idx": 164, "end_idx": 169, "type": "bod", "entity": "uterus" }, { "start_idx": 174, "end_idx": 179, "type": "bod", "entity": "cervix" }, { "start_idx": 439, "end_idx": 444, "type": "dis", "entity": "Cancer" }, { "start_idx": 524, "end_idx": 530, "type": "dep", "entity": "UrolInt" }, { "start_idx": 599, "end_idx": 605, "type": "dep", "entity": "Surgery" } ] }, { "text": "第二节肺的通气-灌流(一)肺通气-灌流比值肺泡从吸入空气获得氧,从血液获得二氧化碳(图8-6)。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "肺" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "肺泡" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "血液" } ] }, { "text": "理想状态下整个肺或各个肺泡通气单位的肺泡通气和血液灌流比值应相当(V/Q=1)。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肺" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肺泡" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肺泡" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "V/Q" } ] }, { "text": "在肺泡和组织部分的氧和二氧化碳交换速率应该相等。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "肺泡" } ] }, { "text": "如果气体弥散没有障碍,则肺泡和血液间的气体分压趋于平衡,各个气体分压总和为大气压。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肺泡" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "血液" } ] }, { "text": "从表8-4中看到,理想状态下肺泡内和动脉气体分压是一致的。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肺泡" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "动脉" } ] }, { "text": "在直立状态,上部肺泡含气量多,血流量低,V/Q比值≥1;下部肺泡含气量少,血流量高,V/Q比值<0.8;仅中部肺组织V/Q接近0.8。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "ite", "entity": "肺泡含气量" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "血流量" }, { "start_idx": 30, "end_idx": 34, "type": "ite", "entity": "肺泡含气量" }, { "start_idx": 37, "end_idx": 39, "type": "ite", "entity": "血流量" }, { "start_idx": 42, "end_idx": 44, "type": "ite", "entity": "V/Q" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "肺组织" }, { "start_idx": 58, "end_idx": 60, "type": "ite", "entity": "V/Q" } ] }, { "text": "在病变肺,某些肺组织因实变、水肿、不张等病变导致V/Q<0.8,局部Qs/Qt可以显著上升。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肺组织" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "实变" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "水肿" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "不张" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "V/Q" }, { "start_idx": 34, "end_idx": 38, "type": "ite", "entity": "Qs/Qt" } ] }, { "text": "图8-6肺通气-灌流示意图A.正常的通气-灌流平衡;B.通气障碍:肺泡通气障碍导致氧合受阻,产生功能性分流;C.通气障碍时血液灌流的代偿性改变:通气差的肺泡其血液灌流相应减少,可改善动脉血氧合;D.血液灌流障碍:灌流差的肺泡尽管通气良好,只相当于增大的死腔表8-4海平面大气、肺泡、动静脉血液和组织的气体分压kPa(mmHg)(二)右向左分流右向左分流也称为静脉-动脉分流(Qs/Qt),在健康人大约有3%的混合静脉血不经肺毛细血管直接进入体循环的解剖分流。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "肺" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "通气障碍" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "肺泡通气障碍" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "功能性分流" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "通气障碍" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "血液" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "肺泡" }, { "start_idx": 91, "end_idx": 93, "type": "bod", "entity": "动脉血" }, { "start_idx": 99, "end_idx": 100, "type": "bod", "entity": "血液" }, { "start_idx": 99, "end_idx": 104, "type": "sym", "entity": "血液灌流障碍" }, { "start_idx": 110, "end_idx": 111, "type": "bod", "entity": "肺泡" }, { "start_idx": 138, "end_idx": 139, "type": "bod", "entity": "肺泡" }, { "start_idx": 141, "end_idx": 145, "type": "bod", "entity": "动静脉血液" }, { "start_idx": 166, "end_idx": 170, "type": "sym", "entity": "右向左分流" }, { "start_idx": 171, "end_idx": 175, "type": "sym", "entity": "右向左分流" }, { "start_idx": 179, "end_idx": 185, "type": "sym", "entity": "静脉-动脉分流" }, { "start_idx": 187, "end_idx": 191, "type": "sym", "entity": "Qs/Qt" }, { "start_idx": 206, "end_idx": 208, "type": "bod", "entity": "静脉血" }, { "start_idx": 211, "end_idx": 215, "type": "bod", "entity": "肺毛细血管" }, { "start_idx": 220, "end_idx": 222, "type": "bod", "entity": "体循环" } ] }, { "text": "如果患儿为右向左分流的先天性心血管病,或肺部因实变、水肿、不张等病变,流经肺毛细血管的混合静脉血未与肺泡气交换而进入静脉,为肺内分流。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "右向左分流" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "先天性心血管病" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "肺部" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "实变" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "水肿" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "不张" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "肺毛细血管" }, { "start_idx": 43, "end_idx": 47, "type": "bod", "entity": "混合静脉血" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "肺泡" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "静脉" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "肺内" } ] }, { "text": "如果分流量增加到>15%,可以出现低氧血症。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "低氧血症" } ] }, { "text": "当分流量>30%,为严重低氧血症,只有吸入高浓度氧(FiO2>0.5)才能维持。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "低氧血症" } ] }, { "text": "四、血小板减少性紫癜血小板减少(<100×109/L)是新生儿出血的主要原因之一,新生儿血小板减少的病因很多,发病机制较复杂,临床表现多样。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "血小板减少性紫癜" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "血小板" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "血小板" } ] }, { "text": "【病因与发病机制】根据病因和发病机制不同,新生儿血小板减少性紫癜可分为以下几种:1.同族免疫性血小板减少性紫癜发病机制与新生儿Rh或ABO血型不合溶血病相似,为母婴血小板抗原性不合所致。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "血小板" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "紫癜" }, { "start_idx": 42, "end_idx": 54, "type": "dis", "entity": "同族免疫性血小板减少性紫癜" }, { "start_idx": 60, "end_idx": 75, "type": "dis", "entity": "新生儿Rh或ABO血型不合溶血病" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "血小板" }, { "start_idx": 82, "end_idx": 89, "type": "sym", "entity": "血小板抗原性不合" } ] }, { "text": "2.先天性被动免疫性血小板减少性紫癜因母亲患特发性血小板减少性紫癜,其血中抗血小板抗体通过胎盘进入胎儿血循环,破坏胎儿血小板,如母亲患系统性红斑狼疮,也可产生血小板抗体,通过胎盘,破坏胎儿血小板。", "entities": [ { "start_idx": 2, "end_idx": 17, "type": "dis", "entity": "先天性被动免疫性血小板减少性紫癜" }, { "start_idx": 22, "end_idx": 32, "type": "dis", "entity": "特发性血小板减少性紫癜" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "抗血小板抗体" }, { "start_idx": 67, "end_idx": 73, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 79, "end_idx": 83, "type": "dis", "entity": "血小板抗体" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "胎盘" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "血小板" } ] }, { "text": "3.新生儿溶血病合并血小板减少性紫癜患儿同时存在红细胞和血小板同族免疫抗体。", "entities": [ { "start_idx": 2, "end_idx": 17, "type": "dis", "entity": "新生儿溶血病合并血小板减少性紫癜" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "红细胞" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "血小板" } ] }, { "text": "4.药物性血小板减少性紫癜分为两种:①先天性:孕母多为过敏体质,在孕后期用过某种药物而被致敏,当再用同一药物时,会产生大量IgG抗体,通过胎盘进入胎儿,破坏血小板;②后天性:出生后新生儿用某些药物,如磺胺、地高辛、吲哚美辛等,产生抗血小板抗体,破坏血小板。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "药物性血小板减少性紫癜" }, { "start_idx": 61, "end_idx": 65, "type": "bod", "entity": "IgG抗体" }, { "start_idx": 78, "end_idx": 80, "type": "bod", "entity": "血小板" }, { "start_idx": 100, "end_idx": 101, "type": "dru", "entity": "磺胺" }, { "start_idx": 103, "end_idx": 105, "type": "dru", "entity": "地高辛" }, { "start_idx": 107, "end_idx": 110, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 115, "end_idx": 120, "type": "bod", "entity": "抗血小板抗体" }, { "start_idx": 124, "end_idx": 126, "type": "bod", "entity": "血小板" } ] }, { "text": "5.感染性血小板减少性紫癜宫内感染以巨细胞病毒、弓形体、风疹、疱疹病毒较为常见,生后感染以细菌感染为主,如败血症、肺炎、细菌性脑膜炎、尿路感染等。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "感染性血小板减少性紫癜" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "宫内感染" }, { "start_idx": 18, "end_idx": 22, "type": "mic", "entity": "巨细胞病毒" }, { "start_idx": 24, "end_idx": 26, "type": "mic", "entity": "弓形体" }, { "start_idx": 28, "end_idx": 29, "type": "mic", "entity": "风疹" }, { "start_idx": 31, "end_idx": 34, "type": "mic", "entity": "疱疹病毒" }, { "start_idx": 42, "end_idx": 43, "type": "mic", "entity": "感染" }, { "start_idx": 45, "end_idx": 48, "type": "mic", "entity": "细菌感染" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "败血症" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "肺炎" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "细菌性脑膜炎" }, { "start_idx": 67, "end_idx": 70, "type": "dis", "entity": "尿路感染" } ] }, { "text": "感染导致血小板减少感染可产生血小板抗体、抑制骨髓产生血小板、毒素破坏等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "感染" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "血小板" }, { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "血小板减少" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "感染" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "血小板抗体" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "骨髓" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "血小板" } ] }, { "text": "6.先天性巨核细胞增生不良骨髓巨核细胞减少或缺如,导致血小板减少。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "先天性巨核细胞增生不良" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "骨髓" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "巨核细胞" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "血小板" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "血小板减少" } ] }, { "text": "7.遗传性血小板减少性紫癜主要是Wiskott-Aldrich综合征,是一种X连锁隐性遗传病。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "遗传性血小板减少性紫癜" }, { "start_idx": 16, "end_idx": 33, "type": "dis", "entity": "Wiskott-Aldrich综合征" }, { "start_idx": 38, "end_idx": 45, "type": "dis", "entity": "X连锁隐性遗传病" } ] }, { "text": "【临床表现】全身皮肤可出现出血点、瘀点、瘀斑、血肿,严重病例出现内脏出血,如颅内出血、消化道出血。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "全身皮肤" }, { "start_idx": 6, "end_idx": 24, "type": "sym", "entity": "全身皮肤可出现出血点、瘀点、瘀斑、血肿" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "颅内出血" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "消化道出血" } ] }, { "text": "【治疗】根据导致血小板减少的病因,进行病因治疗。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "血小板" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "血小板减少" } ] }, { "text": "如血小板严重减少,应输新鲜血小板。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "血小板" }, { "start_idx": 1, "end_idx": 7, "type": "sym", "entity": "血小板严重减少" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "血小板" } ] }, { "text": "第八节肺静脉异位连接肺静脉异位连接可分为完全性肺静脉异位连接及部分性肺静脉异位连接两类。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "肺静脉异位连接" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "肺静脉异位连接" }, { "start_idx": 20, "end_idx": 29, "type": "dis", "entity": "完全性肺静脉异位连接" }, { "start_idx": 31, "end_idx": 40, "type": "dis", "entity": "部分性肺静脉异位连接" } ] }, { "text": "一、完全性肺静脉异位连接完全性肺静脉异位连接是指所有的肺静脉直接或借道体静脉间接入右心房,占所有心血管畸形的1.5%~2.6%。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "完全性肺静脉异位连接" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "完全性肺静脉异位连接" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肺静脉" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "借道体静脉" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "右心房" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "心血管畸形" } ] }, { "text": "【病理解剖】2/3病人单独发生完全性肺静脉异位连接,不伴有其他畸形;1/3病例同时伴有其他心血管畸形,极少数伴内脏综合征。", "entities": [ { "start_idx": 15, "end_idx": 24, "type": "dis", "entity": "完全性肺静脉异位连接" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "畸形" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "其他心血管畸形" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "内脏综合征" } ] }, { "text": "(一)心上型此类型最常见,占完全性肺静脉异位连接的1/2。", "entities": [ { "start_idx": 14, "end_idx": 23, "type": "dis", "entity": "完全性肺静脉异位连接" } ] }, { "text": "左、右肺静脉在左心房后面先汇合成静脉总干,然后再向上同无名静脉或上腔静脉相连。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "左、右肺静脉" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "左心房" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "静脉" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "无名静脉" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "上腔静脉" } ] }, { "text": "大部分这类病儿的静脉总干通过垂直静脉与左无名静脉相通。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "静脉总干" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "垂直静脉" }, { "start_idx": 19, "end_idx": 23, "type": "bod", "entity": "左无名静脉" } ] }, { "text": "少数病例连于奇静脉。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "奇静脉" } ] }, { "text": "(二)心内型静脉总干直接连于右心房或冠状静脉窦。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "静脉总干" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "右心房" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "冠状静脉窦" } ] }, { "text": "(三)心下型占完全性肺静脉异位连接的10%。", "entities": [ { "start_idx": 7, "end_idx": 16, "type": "dis", "entity": "完全性肺静脉异位连接" } ] }, { "text": "静脉总干向下穿过膈肌的食管裂孔注入静脉,常见的为门脉系统,其次为静脉导管、肝静脉、下腔静脉。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "静脉总干" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "膈肌" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "食管裂孔" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "静脉" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "静脉导管" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "肝静脉" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "下腔静脉" } ] }, { "text": "图9-23完全性肺静脉异位连接A.心上型:通过上升的垂直静脉连接到无名静脉;B.心内型:通过下降的垂直静脉连接到门静脉;C.心下型:连接到冠状窦肺静脉梗阻可发生在各种类型的本病中,常见静脉总干自身狭窄,在心上型、心下型病例中亦可见外部压迫引起的梗阻。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "dis", "entity": "完全性肺静脉异位连接" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "垂直静脉" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "无名静脉" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "垂直静脉" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "门静脉" }, { "start_idx": 69, "end_idx": 71, "type": "bod", "entity": "冠状窦" }, { "start_idx": 72, "end_idx": 76, "type": "dis", "entity": "肺静脉梗阻" }, { "start_idx": 92, "end_idx": 95, "type": "bod", "entity": "静脉总干" }, { "start_idx": 122, "end_idx": 123, "type": "dis", "entity": "梗阻" } ] }, { "text": "如心下型,静脉回流通道穿过膈肌时可受外界压迫,狭窄的静脉导管及肝血窦也可使肺静脉血液回流受阻,这种类型梗阻较多见;心上型,垂直静脉上行时夹于左肺动脉与左支气管之间、或引流入右上腔静脉的静脉总干夹于右肺动脉与气管之间,均可受压变窄,大约半数病人会发生这种情况;心脏型患者梗阻最少见。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "静脉" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "膈肌" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "静脉导管" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "肝血窦" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "肺静脉血液" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "梗阻" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "垂直静脉" }, { "start_idx": 70, "end_idx": 73, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "左支气管" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "右上腔静脉" }, { "start_idx": 92, "end_idx": 95, "type": "bod", "entity": "静脉总干" }, { "start_idx": 98, "end_idx": 101, "type": "bod", "entity": "右肺动脉" }, { "start_idx": 103, "end_idx": 104, "type": "bod", "entity": "气管" }, { "start_idx": 129, "end_idx": 130, "type": "bod", "entity": "心脏" }, { "start_idx": 134, "end_idx": 135, "type": "dis", "entity": "梗阻" } ] }, { "text": "梗阻还可发生在心房水平,限制性的房间交通使进入左心房的血流减少,右心房压力增高,继而影响肺静脉回流。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "梗阻" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心房" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "左心房" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "右心房" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "肺静脉" } ] }, { "text": "右心房、右心室、肺动脉因血流量增多而扩大,而左心房、左心室则因充盈减少,容积缩小。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心房" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "右心室" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "肺动脉" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "左心房" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "左心室" } ] }, { "text": "【病理生理】本病体循环静脉的低氧血液和肺静脉含氧量多的动脉血在右心房汇合,其混合血液量的多少与心房间交通的大小有关。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "静脉" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "肺静脉" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "动脉血" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "右心房" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "心房" } ] }, { "text": "若房缺很小,血液流入左心房受限,引起体循环灌注量不足,同时右心房压力增高,体静脉及肺静脉回流受阻;若房缺够大,两心房间血流通畅,自右心房分别向右心室或左心房的血流量则取决于心房、心室的顺应性和体肺循环的阻力。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血液" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "左心房" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "右心房" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "体静脉" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "肺静脉" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "心房" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "右心房" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "右心室" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "左心房" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "心房" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "心室" } ] }, { "text": "本病的病理生理与是否存在肺静脉梗阻密切相关。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "肺静脉梗阻" } ] }, { "text": "(一)无梗阻型随着生后肺血管阻力逐渐下降,动静脉混合血液进入肺循环的流量相应增加,其血流动力学与大型房间隔缺损类似。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "肺血管" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "动静脉混合血液" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "大型房间隔缺损" } ] }, { "text": "右心室容量负荷过大,引起右室扩大、心肌肥厚,肺动脉压力明显升高,静脉总干梗阻部位和肺小静脉中膜肥厚、内膜增生,肺部淋巴液增多,淋巴管扩张,引起肺动脉反射性收缩,中膜肥厚,肺水肿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "右室" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "心肌" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肺动脉" }, { "start_idx": 12, "end_idx": 30, "type": "sym", "entity": "右室扩大、心肌肥厚,肺动脉压力明显升高" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "静脉总干" }, { "start_idx": 41, "end_idx": 46, "type": "bod", "entity": "肺小静脉中膜" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "内膜" }, { "start_idx": 55, "end_idx": 59, "type": "bod", "entity": "肺部淋巴液" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "淋巴管" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "肺动脉" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "中膜" }, { "start_idx": 85, "end_idx": 85, "type": "bod", "entity": "肺" } ] }, { "text": "肺动脉收缩又可引起继发性肺动脉高压,右心室压力增高,最终导致右心衰竭。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "肺动脉" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "继发性肺动脉高压" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "右心室" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "右心衰竭" } ] }, { "text": "右心室搏动明显。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "右心室搏动明显" } ] }, { "text": "肺动脉瓣区第二心音亢进,呈宽的固定分裂。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "肺动脉瓣区第二心音亢进,呈宽的固定分裂" } ] }, { "text": "第三心音可及。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "第三心音可及" } ] }, { "text": "右心房、右心室血流量增多,引起肺动脉瓣相对狭窄,在胸骨左上缘可闻及柔和的收缩期喷射样杂音。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心房" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "右心室" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "胸骨左上缘" }, { "start_idx": 0, "end_idx": 43, "type": "sym", "entity": "右心房、右心室血流量增多,引起肺动脉瓣相对狭窄,在胸骨左上缘可闻及柔和的收缩期喷射样杂音" } ] }, { "text": "胸骨左下缘常可闻及三尖瓣相对狭窄时出现的舒张期杂音。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "胸骨左下缘常可闻及三尖瓣相对狭窄时出现的舒张期杂音" } ] }, { "text": "如果异位引流静脉连于左无名静脉,则在心底部两侧可听到连续性静脉杂音静脉杂音。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "异位引流静脉" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "左无名静脉" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "心底部" }, { "start_idx": 18, "end_idx": 36, "type": "sym", "entity": "心底部两侧可听到连续性静脉杂音静脉杂音" } ] }, { "text": "肝脏增大亦可作为右心衰竭的一个凭据。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肝脏" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "肝脏增大" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "右心衰竭" } ] }, { "text": "(二)梗阻型这类患儿出生后数天内即可出现临床症状:迅速加重的呼吸困难、发绀、喂养困难。", "entities": [ { "start_idx": 25, "end_idx": 41, "type": "sym", "entity": "迅速加重的呼吸困难、发绀、喂养困难" } ] }, { "text": "与无梗阻型患儿相比,此类型小儿心血管体征不显著。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "心血管" } ] }, { "text": "由于心排出量下降,外周血管搏动减弱。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "心" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "外周血管" } ] }, { "text": "心脏不扩大,右心室搏动不明显,杂音通常缺。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心脏" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "心脏不扩大" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "右心室" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "右心室搏动不明显" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "杂音通常缺" } ] }, { "text": "肺动脉瓣区第二心音明显,存在分裂,但分裂时限不宽。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "肺动脉瓣区第二心音明显,存在分裂,但分裂时限不宽" } ] }, { "text": "如有严重呼吸窘迫时,由于肺水肿,肺部可及湿啰音。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "肺水肿" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "肺部可及湿啰音" } ] }, { "text": "腹部检查可触及肝脏肿大。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "腹部检查" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肝脏" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "腹部检查可触及肝脏肿大" } ] }, { "text": "【辅助检查】(一)胸部X线1.无梗阻型右心房、右心室增大,肺血增多。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "胸部X线" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "右心房" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "右心室" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "肺血" }, { "start_idx": 19, "end_idx": 32, "type": "sym", "entity": "右心房、右心室增大,肺血增多" } ] }, { "text": "如连于左无名静脉,左上心缘可见扩张的垂直静脉和左无名静脉,右侧可见上腔静脉,使心影呈典型的8字形或雪人样,这种表现多出现在年长儿,出生数月的小儿这种影像不明显。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "左无名静脉" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "左上心缘" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "垂直静脉" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "左无名静脉" }, { "start_idx": 9, "end_idx": 27, "type": "sym", "entity": "左上心缘可见扩张的垂直静脉和左无名静脉" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "右侧可见上腔静脉" }, { "start_idx": 38, "end_idx": 51, "type": "sym", "entity": "使心影呈典型的8字形或雪人样" } ] }, { "text": "2.梗阻型其胸片特征为肺间质弥漫性斑点网状阴影,由肺门向周围放射。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "胸片" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "肺间质" }, { "start_idx": 11, "end_idx": 22, "type": "sym", "entity": "肺间质弥漫性斑点网状阴影" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肺门" }, { "start_idx": 24, "end_idx": 31, "type": "sym", "entity": "由肺门向周围放射" } ] }, { "text": "肺间质及肺泡水肿严重时,可产生毛玻璃样改变。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "肺间质及肺泡水肿" } ] }, { "text": "心缘不清晰,但心脏多不增大(图9-9)。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心脏" } ] }, { "text": "X线只有在新生儿期即出现心衰但心影不大的患儿中,才具诊断意义。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "X线" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "心衰" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "心影不大" } ] }, { "text": "图9-24胸部X片显示:心下型完全性肺静脉异位引流由于肺静脉阻塞导致两肺野模糊(二)心电图电轴右偏,右心室肥大。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "胸部X片" }, { "start_idx": 12, "end_idx": 22, "type": "dis", "entity": "心下型完全性肺静脉异位" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肺静脉" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "肺野" }, { "start_idx": 42, "end_idx": 44, "type": "pro", "entity": "心电图" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "右心室" }, { "start_idx": 50, "end_idx": 54, "type": "sym", "entity": "右心室肥大" } ] }, { "text": "没有肺静脉梗阻的患儿,可伴右心房增大,表现为Ⅱ导联及右胸导联P波高尖。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肺静脉梗阻" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "右心房" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "右心房增大" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "右胸" } ] }, { "text": "(三)超声心动图当检查时发现右心房容量负荷过重、卵圆孔或房缺处有右向左分流、左心房内没有看到正常回流的肺静脉、左心房、左心室小、房间隔向左膨出时,需高度怀疑完全性肺静脉异位连接。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "超声心动图" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "右心房" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "左心房" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "肺静脉" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "左心房" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "左心室" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "房间隔" }, { "start_idx": 78, "end_idx": 87, "type": "dis", "entity": "完全性肺静脉异位连接" } ] }, { "text": "心脏超声检查的目的在于明确异位肺静脉的数量、梗阻部位和肺动脉压力。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "心脏超声检查" }, { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "异位肺静脉" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "梗阻" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "肺动脉" } ] }, { "text": "当肺动脉流速高时可见肺动脉干扩张。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "肺动脉" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "肺动脉干" } ] }, { "text": "1.心上型剑突下冠状切面可见左右肺静脉汇集腔与左房相隔。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "bod", "entity": "左右肺静脉汇集腔" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "左房" } ] }, { "text": "向上行走的垂直静脉在胸骨上窝区最易看到(图9-9a),脉冲Doppler可探测到垂直静脉内正向血流频谱及上腔静脉内负向血流。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "垂直静脉" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "胸骨上窝区" }, { "start_idx": 27, "end_idx": 35, "type": "pro", "entity": "脉冲Doppler" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "垂直静脉" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "上腔静脉" } ] }, { "text": "若垂直静脉行走于左肺动脉与左支气管间,可通过剑突下矢状切面观察。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "静脉" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "左支气管" } ] }, { "text": "2.心内型剑突下冠状切面可看到肺静脉各自或汇总后进入右心房。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肺静脉" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "右心房" } ] }, { "text": "如肺静脉异位引流入冠状静脉窦,该切面还可看到冠状窦膨大(图9-9b),这需要与大型房间隔缺损鉴别(原发房间隔缺损位于更靠前的一个切面)。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "肺静脉" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "冠状静脉窦" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "冠状窦" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "大型房间隔缺损" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "3.心下型左右肺静脉汇集腔同样在通过剑突下冠状切面看到,即而以剑突下矢状切面可见一静脉向下穿过膈肌。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "bod", "entity": "左右肺静脉汇集腔" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "静脉" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "膈肌" } ] }, { "text": "通过彩色Doppler显像示静脉总干在穿行食管裂孔处狭窄,追踪血流见汇入门静脉或肝静脉(图9-9c)。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "彩色Doppler显像" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "静脉总干" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "食管裂孔处" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "门静脉" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "肝静脉" } ] }, { "text": "门静脉常扩张。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "门静脉" } ] }, { "text": "最常见的混合型完全性肺静脉异位连接为右肺静脉直接入右心房,左肺静脉通过垂直静脉与左无名静脉连接。", "entities": [ { "start_idx": 4, "end_idx": 16, "type": "dis", "entity": "混合型完全性肺静脉异位连接" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "右肺静脉" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "右心房" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "左肺静脉" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "垂直静脉" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "左无名静脉" } ] }, { "text": "肺静脉梗阻情况可通过连续Doppler及彩色Doppler显像来估计。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肺静脉梗阻" }, { "start_idx": 10, "end_idx": 18, "type": "pro", "entity": "连续Doppler" }, { "start_idx": 20, "end_idx": 30, "type": "pro", "entity": "彩色Doppler显像" } ] }, { "text": "梗阻部位血流流速增快,无时相性血流图谱(图9-9d)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "梗阻" } ] }, { "text": "但如果右心室流出道梗阻,肺静脉血流量不足,上述现象可能会被掩盖,常见于复杂型青紫性心脏病。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "右心室流出道梗阻" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肺静脉" }, { "start_idx": 35, "end_idx": 43, "type": "dis", "entity": "复杂型青紫性心脏病" } ] }, { "text": "给予前列腺素E可予以鉴别。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "前列腺素E" } ] }, { "text": "用三尖瓣反流检测仪可测得三尖瓣两侧压力阶差,进而明确肺动脉压力。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "equ", "entity": "三尖瓣反流检测仪" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "肺动脉" } ] }, { "text": "(四)心导管造影检查对大多数病人,二维心脏超声和Doppler技术可了解肺静脉解剖结构及梗阻部位等细节问题,通常很少再行心导管检查。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "心导管造影检查" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "二维心脏超声" }, { "start_idx": 24, "end_idx": 32, "type": "pro", "entity": "Doppler技术" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "肺静脉" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "梗阻" }, { "start_idx": 60, "end_idx": 64, "type": "pro", "entity": "心导管检查" } ] }, { "text": "心导管术仅用于复杂病例和狭窄血管的精确定位上。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "心导管术" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "血管" } ] }, { "text": "1.血氧饱和度肺静脉异位入口处血氧饱和度明显升高。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "ite", "entity": "血氧饱和度" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肺静脉" }, { "start_idx": 15, "end_idx": 19, "type": "ite", "entity": "血氧饱和度" } ] }, { "text": "2.压力当心房间交通很小时,右心房压力常高于左心房,不过如没有此压力上的区别,也不能排除限制性心房内交通的存在。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心房" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "右心房" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "左心房" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "心房" } ] }, { "text": "右心室及肺动脉压力稍高于体循环。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "右心室" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "肺动脉" } ] }, { "text": "3.造影选择性肺静脉造影对明确肺静脉异常开口位置具有诊断意义。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "造影" }, { "start_idx": 4, "end_idx": 11, "type": "pro", "entity": "选择性肺静脉造影" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肺静脉" } ] }, { "text": "在梗阻型患儿中,可显示血管狭窄,造影剂排空时间延长。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "梗阻" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血管" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "造影剂" } ] }, { "text": "但是,由于在肺静脉回流通道中放置心导管,可进一步增加梗阻的程度,此检查在肺静脉梗阻同时有肺动脉高压的患婴中危险性较大。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肺静脉" }, { "start_idx": 16, "end_idx": 18, "type": "equ", "entity": "心导管" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "梗阻" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "肺静脉梗阻" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "肺动脉高压" } ] }, { "text": "(五)磁共振成像异位连接的肺静脉和回流静脉狭窄部位还可以通过磁共振来显示。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "磁共振成像" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肺静脉" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "回流静脉" }, { "start_idx": 30, "end_idx": 32, "type": "pro", "entity": "磁共振" } ] }, { "text": "但是,将患婴置于磁共振仪器上有一定的危险性,且目前尚无研究表明磁共振显像优于上述诊断方法。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "equ", "entity": "磁共振仪器" }, { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "磁共振" } ] }, { "text": "【治疗】婴幼儿一旦病情稳定,即应该实施根治性手术。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "手术" } ] }, { "text": "有严重肺水肿、低氧血症、低心排量的患婴,早期应予正压通气插管、强心剂、利尿剂及纠正代谢性酸中毒以稳定病情,等待手术时机。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "严重肺水肿" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "低氧血症" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "低心排量" }, { "start_idx": 24, "end_idx": 29, "type": "pro", "entity": "正压通气插管" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "强心剂" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "利尿剂" }, { "start_idx": 39, "end_idx": 46, "type": "pro", "entity": "纠正代谢性酸中毒" }, { "start_idx": 55, "end_idx": 56, "type": "pro", "entity": "手术" } ] }, { "text": "如果可能,则尽量避免行心导管术,以防耽误手术时间、出现其他并发症。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "心导管术" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "手术" } ] }, { "text": "现已不再认为球囊房隔造口术是必要的姑息性手术。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "pro", "entity": "球囊房隔造口术" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "姑息性手术" } ] }, { "text": "如异位肺静脉直接入右心房,可打开右心房,切开房间隔,用补片将左右肺静脉全部隔入左心房;如异位开口于冠状窦,切开左心房与冠状窦之间的房间隔组织,随即缝合冠状窦开口以及房间隔缺损,使冠状窦直接引流入左心房;对于心上型或心下型肺静脉异位连接者,将肺静脉总干与左心房之间做大的侧-侧吻合,关闭房间隔缺损,而是否结扎肺静脉回流通道仍存在争议。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "异位肺静脉" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "右心房" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "右心房" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "房间隔" }, { "start_idx": 27, "end_idx": 28, "type": "equ", "entity": "补片" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "左右肺静脉" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "左心房" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "冠状窦" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "左心房" }, { "start_idx": 59, "end_idx": 61, "type": "bod", "entity": "冠状窦" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "房间隔" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "冠状窦" }, { "start_idx": 82, "end_idx": 86, "type": "bod", "entity": "房间隔缺损" }, { "start_idx": 89, "end_idx": 91, "type": "bod", "entity": "冠状窦" }, { "start_idx": 97, "end_idx": 99, "type": "bod", "entity": "左心房" }, { "start_idx": 103, "end_idx": 116, "type": "dis", "entity": "心上型或心下型肺静脉异位连接" }, { "start_idx": 120, "end_idx": 124, "type": "bod", "entity": "肺静脉总干" }, { "start_idx": 126, "end_idx": 128, "type": "bod", "entity": "左心房" }, { "start_idx": 142, "end_idx": 146, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 153, "end_idx": 155, "type": "bod", "entity": "肺静脉" } ] }, { "text": "一些主张开放回流静脉者认为,开放垂直静脉可预防术后早期左心偏小、肺静脉回流受阻情况。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "回流静脉" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "垂直静脉" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "左心" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "肺静脉" } ] }, { "text": "且有报道称,结扎下行的静脉通道后,会引起急性肝细胞坏死,故一些外科医生建议不缝合该静脉。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "静脉" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "急性肝细胞坏死" }, { "start_idx": 31, "end_idx": 32, "type": "dep", "entity": "外科" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "静脉" } ] }, { "text": "图9-25超声心动图显示:肺静脉异常引流入(箭头所示为血流方向)CS:冠状窦PV:肺静脉SVC:上腔静脉VV:垂直静脉【预后】若不行手术治疗,本病预后差。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "超声心动图" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肺静脉" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "冠状窦" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "肺静脉" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "上腔静脉" }, { "start_idx": 55, "end_idx": 58, "type": "bod", "entity": "垂直静脉" }, { "start_idx": 66, "end_idx": 69, "type": "pro", "entity": "手术治疗" } ] }, { "text": "手术治疗的效果已有明显改善,患儿死亡率从20世纪70年代末期的30%降至80年代末90年代初的不到10%。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" } ] }, { "text": "手术死亡率与患儿术前病情状况密切相关。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" } ] }, { "text": "心下型患儿、术前需用呼吸机、有代谢性酸中毒患儿术后死亡率较高。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "equ", "entity": "呼吸机" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "肺静脉梗阻小儿术后早期可出现肺高压,故对于这种患儿,吸入NO有助于扩张肺动脉,降低死亡率。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肺静脉梗阻" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肺高压" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "肺动脉" } ] }, { "text": "偶尔也可发生晚期肺静脉梗阻,可能与术后组织反应或吻合口狭窄有关,但再次手术、球囊扩张术或用stent补片却易造成该处血液反流。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "晚期肺静脉梗阻" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "再次手术" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "球囊扩张术" }, { "start_idx": 45, "end_idx": 51, "type": "equ", "entity": "stent补片" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "血液" } ] }, { "text": "少数患儿后期可出现心律失常,包括窦性心动过缓、房扑及室上性心动过速,其中房性心律失常最为常见。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "心律失常" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "窦性心动过缓" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "房扑" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "房性心律失常" } ] }, { "text": "第九章功能性消化不良功能性消化不良(functionaldyspepsia,FD)是指有持续存在或反复发作的上腹痛腹胀早饱、嗳气、厌食、胃灼热泛酸、恶心及呕吐等消化功能障碍症状,经各项检查排除器质性疾病的一组小儿消化内科最常见的临床综合征。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 18, "end_idx": 36, "type": "dis", "entity": "functionaldyspepsia" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "FD" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "上腹" }, { "start_idx": 54, "end_idx": 56, "type": "sym", "entity": "上腹痛" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "腹" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "腹胀" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "早饱" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "嗳气" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "厌食" }, { "start_idx": 68, "end_idx": 68, "type": "bod", "entity": "胃" }, { "start_idx": 68, "end_idx": 70, "type": "sym", "entity": "胃灼热" }, { "start_idx": 71, "end_idx": 72, "type": "sym", "entity": "泛酸" }, { "start_idx": 74, "end_idx": 75, "type": "sym", "entity": "恶心" }, { "start_idx": 80, "end_idx": 85, "type": "dis", "entity": "消化功能障碍" }, { "start_idx": 96, "end_idx": 100, "type": "dis", "entity": "器质性疾病" }, { "start_idx": 104, "end_idx": 109, "type": "dep", "entity": "小儿消化内科" } ] }, { "text": "功能性消化不良的患儿主诉各异,又缺乏肯定的特异病理生理基础,因此,对这一部分患者,曾有许多命名,主要有功能性消化不良、非溃疡性消化不良(nonulcerdyspepsia,NUD)、特发性消化不良(idiopathicdyspepsia)、原发性消化不良(essentialdyspepsia)、胀气性消化不良(flatulentdyspepsia)以及上腹不适综合征(epigastricdistresssyndrome)等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 51, "end_idx": 57, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 59, "end_idx": 66, "type": "dis", "entity": "非溃疡性消化不良" }, { "start_idx": 68, "end_idx": 84, "type": "dis", "entity": "nonulcerdyspepsia" }, { "start_idx": 91, "end_idx": 97, "type": "dis", "entity": "特发性消化不良" }, { "start_idx": 99, "end_idx": 117, "type": "dis", "entity": "idiopathicdyspepsia" }, { "start_idx": 120, "end_idx": 126, "type": "dis", "entity": "原发性消化不良" }, { "start_idx": 128, "end_idx": 145, "type": "dis", "entity": "essentialdyspepsia" }, { "start_idx": 148, "end_idx": 154, "type": "dis", "entity": "胀气性消化不良" }, { "start_idx": 156, "end_idx": 173, "type": "dis", "entity": "flatulentdyspepsia" }, { "start_idx": 177, "end_idx": 183, "type": "dis", "entity": "上腹不适综合征" }, { "start_idx": 185, "end_idx": 210, "type": "dis", "entity": "epigastricdistresssyndrome" } ] }, { "text": "目前国际上多采用前三种命名,而“功能性消化不良”尤为大多数学者所接受。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "【流行病学】FD发病十分普遍,美国东北部郊区507名社区青少年调查发现,5%~10%的受调查者具有典型的消化不良症状。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "FD" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "消化不良" } ] }, { "text": "意大利北部校园儿童研究表明3.5%存在溃疡样消化不良的表现,3.7%存在动力障碍样消化不良,但本研究中未纳入12岁以上的青少年,所以患病率低。", "entities": [ { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "溃疡样消化不良" }, { "start_idx": 36, "end_idx": 44, "type": "dis", "entity": "动力障碍样消化不良" } ] }, { "text": "一项在儿科消化专科门诊进行的研究表明,4~9岁功能性胃肠病患儿中,13.5%被诊断为消化不良,10~18岁中有10.2%有消化不良。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dep", "entity": "儿科" }, { "start_idx": 5, "end_idx": 8, "type": "dep", "entity": "消化专科" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "功能性胃肠病" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "消化不良" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "消化不良" } ] }, { "text": "在我国此病有逐年上升的趋势,以消化不良为主诉的成人患者约占普通内科门诊的11%、占消化专科门诊的53%。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "消化不良" }, { "start_idx": 29, "end_idx": 32, "type": "dep", "entity": "普通内科" }, { "start_idx": 41, "end_idx": 44, "type": "dep", "entity": "消化专科" } ] }, { "text": "国内儿科患者中功能性消化不良的发病率尚无规范的统计。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dep", "entity": "儿科" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "【病因及发病机制】FD的病因不明,其发病机制亦不清楚。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "FD" } ] }, { "text": "这些因素包括了饮食和环境、胃酸分泌、幽门螺旋杆菌感染、消化道运动功能异常、心理因素以及一些其他胃肠功能紊乱性疾病,如胃食管反流性疾病(GERD)、吞气症及肠易激综合征等。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胃酸" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "幽门螺旋杆菌感染" }, { "start_idx": 27, "end_idx": 35, "type": "dis", "entity": "消化道运动功能异常" }, { "start_idx": 47, "end_idx": 55, "type": "dis", "entity": "胃肠功能紊乱性疾病" }, { "start_idx": 58, "end_idx": 65, "type": "dis", "entity": "胃食管反流性疾病" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "GER" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "吞气症" }, { "start_idx": 77, "end_idx": 82, "type": "dis", "entity": "肠易激综合征" } ] }, { "text": "(一)饮食与环境因素FD患者的症状往往与饮食有关,许多患者常常主诉一些含气饮料、咖啡、柠檬或其他水果以及油炸类食物会加重消化不良。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "FD" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "消化不良" } ] }, { "text": "虽然双盲法食物诱发试验对食物诱因的意义提出了质疑,但许多患儿仍在避免上述食物并平衡了膳食结构后感到症状有所减轻。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "双盲法食物诱发试验" } ] }, { "text": "(二)胃酸部分FD的患者会出现溃疡样症状,如饥饿痛,在进食后渐缓解,腹部有指点压痛制酸剂或抑酸药物症状可在短期内缓解。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "FD" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "溃疡" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "饥饿痛" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "腹部" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "腹部有指点压痛" }, { "start_idx": 41, "end_idx": 43, "type": "dru", "entity": "制酸剂" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "抑酸药物" } ] }, { "text": "这些都提示这类患者的发病与胃酸有关。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胃酸" } ] }, { "text": "然而绝大多数研究证实FD患者基础胃酸和最大胃酸分泌量没有增加,胃酸分泌与溃疡样症状无关,症状程度与最大胃酸分泌也无相关性。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "FD" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "胃酸" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "溃疡" }, { "start_idx": 49, "end_idx": 54, "type": "ite", "entity": "最大胃酸分泌" } ] }, { "text": "所以,胃酸在功能性消化不良发病中的作用仍需进一步研究。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "(三)慢性胃炎与十二指肠炎功能性消化不良患者中大约有30%~50%经组织学检查证实为胃窦胃炎,欧洲不少国家将慢性胃炎视为功能性消化不良,认为慢性胃炎可能通过神经及体液因素影响胃的运动功能,也有作者认为非糜烂性十二指肠炎也属于功能性消化不良。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "十二指肠炎" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 42, "end_idx": 45, "type": "dis", "entity": "胃窦胃炎" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 60, "end_idx": 66, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 70, "end_idx": 73, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 78, "end_idx": 79, "type": "bod", "entity": "神经" }, { "start_idx": 81, "end_idx": 82, "type": "bod", "entity": "体液" }, { "start_idx": 87, "end_idx": 87, "type": "bod", "entity": "胃" }, { "start_idx": 100, "end_idx": 108, "type": "dis", "entity": "非糜烂性十二指肠炎" }, { "start_idx": 112, "end_idx": 118, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "应当指出的是,功能性消化不良症状的轻重并不与胃黏膜炎症病变相互平行。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "胃黏膜炎症" } ] }, { "text": "(四)幽门螺杆菌感染幽门螺杆菌是一种革兰阴性细菌,一般定植于胃的黏液层表面。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 10, "end_idx": 14, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 18, "end_idx": 23, "type": "mic", "entity": "革兰阴性细菌" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "胃" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "黏液层" } ] }, { "text": "幽门螺杆菌感染与功能性消化不良关系的研究结果差异很大,有些研究认为幽门螺杆菌感染是FD的病理生理因素之一,因为在成人中,功能性消化不良患者的胃黏膜内常可发现幽门螺杆菌,检出率在40%~70%之间。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "FD" }, { "start_idx": 60, "end_idx": 66, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 78, "end_idx": 82, "type": "mic", "entity": "幽门螺杆菌" } ] }, { "text": "但大量的研究却表明:FD患者的幽门螺杆菌感染率并不高于正常健康人,阳性幽门螺杆菌和阴性幽门螺杆菌者的胃肠运动和胃排空功能无明显差异,且幽门螺杆菌阳性的FD患者经根除幽门螺杆菌治疗后其消化不良症状并不一定随之消失,进一步研究证实幽门螺杆菌特异性抗原与FD无相关性,甚至其特异血清型CagA与任何消化不良症状或任何原发性功能性上腹不适症状均无关系。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "FD" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 35, "end_idx": 39, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 43, "end_idx": 47, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "胃肠" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "胃" }, { "start_idx": 67, "end_idx": 71, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 75, "end_idx": 76, "type": "dis", "entity": "FD" }, { "start_idx": 91, "end_idx": 94, "type": "dis", "entity": "消化不良" }, { "start_idx": 113, "end_idx": 117, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 124, "end_idx": 125, "type": "dis", "entity": "FD" }, { "start_idx": 146, "end_idx": 149, "type": "dis", "entity": "消化不良" }, { "start_idx": 155, "end_idx": 164, "type": "dis", "entity": "原发性功能性上腹不适" } ] }, { "text": "目前国内学者的共识意见为幽门螺杆菌感染为慢性活动性胃炎的主要病因,有消化不良症状的幽门螺杆菌感染者可归属于FD范畴。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "慢性活动性胃炎" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "消化不良" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "FD" } ] }, { "text": "(五)胃肠运动功能障碍许多的研究都认为FD其实是胃肠道功能紊乱的一种。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "胃肠运动功能障碍" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "FD" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "胃肠道功能紊乱" } ] }, { "text": "它与其他胃肠功能紊乱性疾病有着相似的发病机制。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "dis", "entity": "胃肠功能紊乱性疾病" } ] }, { "text": "近年来随着对胃肠功能疾病在生理学(运动-感觉)、基础学(脑-肠作用)及精神社会学等方面的进一步了解,并基于其所表现的症状及解剖位置,罗马委员会制定了新的标准,即罗马Ⅲ标准。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "胃肠功能疾病" } ] }, { "text": "罗马Ⅲ标准不仅包括诊断标准,亦对胃肠功能紊乱的基础生理、病理、神经支配及胃肠激素、免疫系统做了详尽的叙述,同时在治疗方面也提出了指导性意见。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "胃肠功能紊乱" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "免疫系统" } ] }, { "text": "因此罗马Ⅲ标准是目前世界各国用于功能性胃肠疾病诊断、治疗的一个共识文件。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "功能性胃肠疾病" } ] }, { "text": "该标准认为:胃肠道运动在消化期与消化间期有不同的形式和特点。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "胃肠道" } ] }, { "text": "空腹状态下由胃至末端回肠存在一种周期性运动形式,称为消化间期移行性综合运动(MMC)。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "胃" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "末端回肠" } ] }, { "text": "大约在正常餐后4~6小时,这种周期性、特征性的运动起于近端胃,并缓慢传导到整个小肠。", "entities": [ { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "小肠" } ] }, { "text": "每个MMC由4个连续时相组成:Ⅰ相为运动不活跃期;Ⅱ相的特征是间断性蠕动收缩;Ⅲ相时胃发生连续性蠕动收缩,每个慢波上伴有快速发生的动作电位(峰电位),收缩环中心闭合而幽门基础压力却不高,处于开放状态,故能清除胃内残留食物;Ⅳ相是Ⅲ相结束回到Ⅰ相的恢复期。", "entities": [ { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "胃" }, { "start_idx": 83, "end_idx": 84, "type": "bod", "entity": "幽门" }, { "start_idx": 104, "end_idx": 104, "type": "bod", "entity": "胃" } ] }, { "text": "与之相对应,在Ⅲ期还伴有胃酸分泌、胰腺和胆汁分泌。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "胃酸" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "胰腺" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "胆汁" } ] }, { "text": "空腹状态下,十二指肠最大收缩频率为12次/分,从十二指肠开始MMC向远端移动速度为5~10cm/min,90分钟后达末端回肠,其作用是清除肠腔内不被消化的颗粒。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "腹" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "十二指肠" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "末端回肠" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "肠腔" } ] }, { "text": "进餐打乱了消化间期的活动,出现一种特殊的运动类型:胃窦-十二指肠协调收缩。", "entities": [ { "start_idx": 25, "end_idx": 31, "type": "bod", "entity": "胃窦-十二指肠" } ] }, { "text": "胃底出现容受性舒张,远端胃出现不规则时相性收缩,持续数分钟后进入较稳定的运动模式,即3次/分的节律性蠕动性收缩,并与幽门括约肌的开放和十二指肠协调运动,推动食物进入十二指肠。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "胃" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "胃" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "幽门括约肌" }, { "start_idx": 67, "end_idx": 70, "type": "bod", "entity": "十二指肠" } ] }, { "text": "此时小肠出现不规则、随机的收缩运动,并根据食物的大小和性质,使得这种运动模式可维持2.5~8小时。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "小肠" } ] }, { "text": "此后当食物从小肠排空后,又恢复消化间期模式。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "小肠" } ] }, { "text": "在长期的对FD患者的研究中发现:约50%FD患者存在餐后胃排空延迟,可以是液体或(和)固体排空障碍。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "FD" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "FD" }, { "start_idx": 37, "end_idx": 48, "type": "dis", "entity": "液体或(和)固体排空障碍" } ] }, { "text": "小儿FD中有61.53%胃排空迟缓胃运动异常的综合表现,胃近端张力减低胃窦运动减弱胃电紊乱胃排空功能。", "entities": [ { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "胃" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "胃排空迟缓" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "胃运动异常" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "胃" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "胃近端张力减低" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "胃窦" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "胃窦运动减弱" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "胃" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "胃电紊乱" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "胃" } ] }, { "text": "胃内压力测定发现,25%功能性消化不良胃窦运动功能减弱胃窦无收缩。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "胃内压力测定" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胃窦" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "胃窦运动功能减弱" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "胃窦无收缩" } ] }, { "text": "儿童中,FD患儿胃窦收缩幅度明显低于健康儿胃容量-压力关系曲线和电子恒压器检查发现患者胃近端容纳舒张功能受损胃顺应性降低近端胃壁张力下降FD患者有小肠运动障碍,以近端小肠为主,胃窦-十二指肠测压发现胃窦十二指肠运动不协调十二指肠运动紊乱,约有1/3的FD存在肠易激综合征。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "FD" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胃窦" }, { "start_idx": 8, "end_idx": 20, "type": "sym", "entity": "胃窦收缩幅度明显低于健康儿" }, { "start_idx": 21, "end_idx": 26, "type": "ite", "entity": "胃容量-压力" }, { "start_idx": 32, "end_idx": 38, "type": "pro", "entity": "电子恒压器检查" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "胃" }, { "start_idx": 43, "end_idx": 53, "type": "sym", "entity": "胃近端容纳舒张功能受损" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "胃" }, { "start_idx": 54, "end_idx": 59, "type": "sym", "entity": "胃顺应性降低" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "近端胃壁" }, { "start_idx": 60, "end_idx": 67, "type": "sym", "entity": "近端胃壁张力下降" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "FD" }, { "start_idx": 73, "end_idx": 78, "type": "dis", "entity": "小肠运动障碍" }, { "start_idx": 81, "end_idx": 84, "type": "bod", "entity": "近端小肠" }, { "start_idx": 99, "end_idx": 100, "type": "bod", "entity": "胃窦" }, { "start_idx": 101, "end_idx": 104, "type": "bod", "entity": "十二指肠" }, { "start_idx": 99, "end_idx": 109, "type": "sym", "entity": "胃窦十二指肠运动不协调" }, { "start_idx": 110, "end_idx": 117, "type": "dis", "entity": "十二指肠运动紊乱" }, { "start_idx": 125, "end_idx": 126, "type": "dis", "entity": "FD" }, { "start_idx": 129, "end_idx": 134, "type": "dis", "entity": "肠易激综合征" } ] }, { "text": "(六)内脏感觉异常许多功能性消化不良的患者对生理或轻微有害刺激的感受异常或过于敏感。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "一些患者对灌注酸和盐水的敏感性提高;一些患者即使在使用了H2受体拮抗剂阻断酸分泌的情况下,静脉注射五肽胃泌素仍会发生疼痛。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "灌注酸" }, { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "盐水" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "酸" }, { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "静脉注射" }, { "start_idx": 55, "end_idx": 59, "type": "dru", "entity": "五肽胃泌素" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "疼痛" } ] }, { "text": "一些研究报道,球囊在近端胃膨胀时,功能性消化不良患者的疼痛往往会加重,他们疼痛发作时球囊膨胀的水平显著低于对照组内脏感觉的异常在功能性消化不良中可能起到了一定作用。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "球囊" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "胃" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "疼痛往往会加重" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "球囊" }, { "start_idx": 37, "end_idx": 55, "type": "sym", "entity": "疼痛发作时球囊膨胀的水平显著低于对照组" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "内脏" }, { "start_idx": 64, "end_idx": 70, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "但这种感觉异常的基础尚不清楚,初步研究证实功能性消化不良患者存在两种内脏传入功能障碍,一种是不被察觉的反射传入信号,另一种为感知信号。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "感觉异常" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 34, "end_idx": 41, "type": "dis", "entity": "内脏传入功能障碍" } ] }, { "text": "当胃肠道机械感受器感受扩张刺激后,受试者会因扩张容量的逐渐增加而产生感知、不适及疼痛,从而获得不同状态的扩张容量,功能性消化不良患者感知阈明显低于正常人,表明患者感觉过敏。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "bod", "entity": "胃肠道机械感受器" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "(七)心理社会因素心理学因素是否与功能性消化不良的发病有关一直存在着争议。", "entities": [ { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "国内有学者曾对186名FD患者的年龄、性别、生活习惯以及文化程度等进行了解,并做了焦虑及抑郁的评定,结果发现FD患者以年龄偏大的女性多见,它的发生与焦虑及抑郁有较明显的关系。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "FD" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "焦虑" }, { "start_idx": 44, "end_idx": 44, "type": "dis", "entity": "抑" }, { "start_idx": 44, "end_idx": 44, "type": "pro", "entity": "抑" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "结果" }, { "start_idx": 70, "end_idx": 71, "type": "dis", "entity": "的发" }, { "start_idx": 73, "end_idx": 74, "type": "dis", "entity": "与焦" } ] }, { "text": "但目前尚无确切的证据表明功能性消化不良症状与精神异常或慢性应激有关。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "精神异常" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "慢性应激" } ] }, { "text": "功能性消化不良患者重大生活应激事件的数量也不一定高于其他人群,但很可能这些患者对应激的感受程度要更高。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "重大生活应激" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "应激" } ] }, { "text": "(八)其他胃肠功能紊乱性疾病1.胃食管反流性疾病(GERD)胃灼热和反流是胃食管反流的特异性症状,但是许多GERD患者并无此明显症状,有些患者主诉既有胃灼热消化不良。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "胃肠功能紊乱性疾病" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "胃食管反流性疾病" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "GERD" }, { "start_idx": 30, "end_idx": 32, "type": "sym", "entity": "胃灼热" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "反流" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "GERD" }, { "start_idx": 75, "end_idx": 75, "type": "bod", "entity": "胃" }, { "start_idx": 75, "end_idx": 77, "type": "sym", "entity": "胃灼热" }, { "start_idx": 78, "end_idx": 81, "type": "dis", "entity": "消化不良" } ] }, { "text": "目前有许多学者已接受了以下看法:有少数GERD患者并无食管炎,许多GERD患者具有复杂的消化不良病史,而不仅是单纯胃灼热酸反流症状。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "GERD" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "食管炎" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "GERD" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "消化不良" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "胃" }, { "start_idx": 57, "end_idx": 59, "type": "sym", "entity": "胃灼热" }, { "start_idx": 60, "end_idx": 62, "type": "sym", "entity": "酸反流" } ] }, { "text": "用食管24小时pH监测研究发现:约有20%的功能性消化不良患者和反流性疾病有关。", "entities": [ { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "反流性疾病" } ] }, { "text": "最近Sandlu等报告,20例小儿厌食中,12例(60%)有胃食管反流胃食管反流性疾病和某些功能性消化不良的病例有关。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "小儿厌食" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "胃食管" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "胃食管反流" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "胃食管反流性疾病" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "2.吞气症许多患者常下意识地吞入过量的空气,导致腹胀饱胀和嗳气,这种情况也常继发于应激或焦虑。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "吞气症" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "腹" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "腹胀" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "饱胀" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "嗳气" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "应激" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "焦虑" } ] }, { "text": "3.肠易激综合征(IBS)功能性消化不良与其他胃肠道紊乱之间常常有许多重叠。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "肠易激综合征" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "IBS" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "胃肠道紊乱" } ] }, { "text": "约有1/3的IBS患者有消化不良症状;功能性消化不良患者中有IBS症状的比例也近似。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "IBS" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "消化不良症状" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "IBS" } ] }, { "text": "【临床表现及分型】临床症状主要包括上腹痛腹胀早饱、嗳气、厌食、胃灼热泛酸、恶心和呕吐。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "上腹" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "上腹痛" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "腹" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "腹胀" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "早饱" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "嗳气" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "厌食" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "胃" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "胃灼热" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "泛酸" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "恶心" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "呕吐" } ] }, { "text": "病程多在2年内,症状可反复发作,也可在相当一段时间内无症状。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "病程多在2年内" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "症状可反复发作" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "一段时间内无症状" } ] }, { "text": "可以某一症状为主,也可有多个症状的叠加。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "sym", "entity": "某一症状为主" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "多个症状的叠加" } ] }, { "text": "1989年,美国芝加哥FD专题会议将功能性消化不良分为5个亚型:反流样消化不良(refluxlikedyspepsia)、运动障碍样消化不良(dysmotilitylikedyspepsia)、溃疡样消化不良(ulcerlikedyspepsia)、吞气症(aerophagia)及特发性消化不良(idiopathicdyspepsia)。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "FD" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "反流样消化不良" }, { "start_idx": 40, "end_idx": 58, "type": "dis", "entity": "refluxlikedyspepsia" }, { "start_idx": 61, "end_idx": 69, "type": "dis", "entity": "运动障碍样消化不良" }, { "start_idx": 71, "end_idx": 94, "type": "dis", "entity": "dysmotilitylikedyspepsia" }, { "start_idx": 97, "end_idx": 103, "type": "dis", "entity": "溃疡样消化不良" }, { "start_idx": 105, "end_idx": 122, "type": "dis", "entity": "ulcerlikedyspepsia" }, { "start_idx": 125, "end_idx": 127, "type": "dis", "entity": "吞气症" }, { "start_idx": 129, "end_idx": 138, "type": "dis", "entity": "aerophagia" }, { "start_idx": 141, "end_idx": 147, "type": "dis", "entity": "特发性消化不良" }, { "start_idx": 149, "end_idx": 167, "type": "dis", "entity": "idiopathicdyspepsia" } ] }, { "text": "(一)运动障碍样消化不良此型患者的表现以腹胀早饱及嗳气为主。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "运动障碍样消化不良" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "腹" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "腹胀" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "早饱" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "嗳气" } ] }, { "text": "症状多在进食后加重。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "症状多在进食后加重" } ] }, { "text": "过饱时会出现腹痛恶心甚至呕吐反流样消化不良突出的表现是胸骨后痛胃灼热反流。", "entities": [ { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "腹" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "过饱时会出现腹痛恶心甚至呕吐" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "反流样消化不良" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "胸骨" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "胸骨后痛" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "胃" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "胃灼热" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "反流" } ] }, { "text": "内镜检查未发现食管炎,但24小时pH监测可发现部分患者有胃食管酸反流食管对酸敏感性增加有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "内镜检查" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "食管炎" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "胃食管" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "胃食管酸反流" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "食管" } ] }, { "text": "(三)溃疡样消化不良主要表现与十二指肠溃疡特点相同,夜间痛,饥饿痛,进食或服抗酸剂能缓解,可伴有反酸,少数患者伴胃灼热症状呈慢性周期性。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "溃疡样消化不良" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "十二指肠溃疡" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "夜间痛" }, { "start_idx": 30, "end_idx": 32, "type": "sym", "entity": "饥饿痛" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "抗酸剂" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "反酸" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "胃" }, { "start_idx": 56, "end_idx": 58, "type": "sym", "entity": "胃灼热" }, { "start_idx": 59, "end_idx": 66, "type": "sym", "entity": "症状呈慢性周期性" } ] }, { "text": "内镜检查未发现溃疡和糜烂性炎症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "内镜检查" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "溃疡" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "糜烂性炎症" } ] }, { "text": "(四)非特异型消化不良消化不良表现不能归入上述类型者。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "非特异型消化不良" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "消化不良" } ] }, { "text": "常合并肠易激综合征。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "肠易激综合征" } ] }, { "text": "【诊断及鉴别诊断】(一)诊断对于功能性消化不良的诊断,首先应排除器质性消化不良。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "器质性消化不良" } ] }, { "text": "除了仔细询问病史及全面体检外,应进行以下的器械及实验室检查:①血常规;②粪隐血试验;③上消化道内镜;④肝胆胰超声;⑤肝肾功能;⑥血糖;⑦甲状腺功能;⑧胸部X检查。", "entities": [ { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "血常规" }, { "start_idx": 36, "end_idx": 40, "type": "pro", "entity": "粪隐血试验" }, { "start_idx": 43, "end_idx": 48, "type": "pro", "entity": "上消化道内镜" }, { "start_idx": 51, "end_idx": 55, "type": "pro", "entity": "肝胆胰超声" }, { "start_idx": 58, "end_idx": 61, "type": "pro", "entity": "肝肾功能" }, { "start_idx": 64, "end_idx": 65, "type": "pro", "entity": "血糖" }, { "start_idx": 68, "end_idx": 72, "type": "pro", "entity": "甲状腺功能" }, { "start_idx": 75, "end_idx": 79, "type": "pro", "entity": "胸部X检查" } ] }, { "text": "其中①~④为第一线检查,⑤~⑧为可选择性检查,多数根据第一线检查即可基本确定功能性消化不良的诊断。", "entities": [ { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "此外,近年来开展的胃食管24小时pH监测、超声或放射性核素胃排空检查以及胃肠道压力测定等多种胃肠道动力检查手段,在FD的诊断与鉴别诊断上也起到了十分重要的作用。", "entities": [ { "start_idx": 9, "end_idx": 19, "type": "pro", "entity": "胃食管24小时pH监测" }, { "start_idx": 21, "end_idx": 33, "type": "pro", "entity": "超声或放射性核素胃排空检查" }, { "start_idx": 36, "end_idx": 42, "type": "pro", "entity": "胃肠道压力测定" }, { "start_idx": 46, "end_idx": 52, "type": "pro", "entity": "胃肠道动力检查" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "FD" } ] }, { "text": "许多原因不明的腹痛恶心及呕吐患者往往经胃肠道压力检查找到了病因,这些检查也逐渐开始应用于儿科患者。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "腹" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "腹痛" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "恶心" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "呕吐" }, { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "胃肠道压力检查" }, { "start_idx": 44, "end_idx": 45, "type": "dep", "entity": "儿科" } ] }, { "text": "(二)功能性消化不良通用的诊断标准2.内镜检查未发现胃及十二指肠溃疡、糜烂和肿瘤等器质性病变,未发现食管炎,也无上述疾病史。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "内镜检查" }, { "start_idx": 26, "end_idx": 33, "type": "dis", "entity": "胃及十二指肠溃疡" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "糜烂" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "肿瘤" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "器质性病变" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "食管炎" } ] }, { "text": "3.实验室、B超及X线检查排除肝、胆、胰疾病。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "实验室、B超及X线检查" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "肝、胆、胰疾病" } ] }, { "text": "4.无糖尿病、结缔组织病、肾脏疾病及精神病史。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "糖尿病" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "肾脏疾病" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "精神病" } ] }, { "text": "5.无腹部手术史。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "腹部手术" } ] }, { "text": "(三)儿童功能性消化不良的罗马Ⅲ诊断标准必须包括以下所有项:1.持续或反复发作的上腹部(脐上)疼痛或不适排便后不能缓解,或症状发作与排便频率或粪便性状的改变无关肠易激综合征)。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "腹" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "脐" }, { "start_idx": 32, "end_idx": 51, "type": "sym", "entity": "持续或反复发作的上腹部(脐上)疼痛或不适" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "排便后不能缓解" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "粪便" }, { "start_idx": 61, "end_idx": 79, "type": "sym", "entity": "症状发作与排便频率或粪便性状的改变无关" }, { "start_idx": 80, "end_idx": 85, "type": "dis", "entity": "肠易激综合征" } ] }, { "text": "(四)鉴别诊断1.胃食管反流胃食管反流性疾病功能性消化不良中的反流亚型与其鉴别困难。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "胃食管反流性疾病" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "胃食管反流性疾病具有典型或不典型反流症状,内镜证实有不同程度的食管炎症改变,24小时食管pH监测有酸反应,无内镜下食管炎表现的患者属于反流样消化不良或胃食管反流性疾病不易确定,但两者在治疗上是相同的。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "胃食管反流性疾病" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "内镜" }, { "start_idx": 67, "end_idx": 73, "type": "dis", "entity": "反流样消化不良" }, { "start_idx": 75, "end_idx": 82, "type": "dis", "entity": "胃食管反流性疾病" } ] }, { "text": "2.具有溃疡样症状的器质性消化不良包括:十二指肠溃疡、十二指肠炎、幽门管溃疡、幽门前区溃疡、糜烂性胃窦炎。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "溃疡" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "器质性消化不良" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "十二指肠溃疡" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "十二指肠炎" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "幽门管溃疡" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "幽门前区溃疡" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "糜烂性胃窦炎" } ] }, { "text": "在诊断功能性消化不良溃疡亚型前,必须进行内镜检查以排除以上器质性病变。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "功能性消化不良溃疡亚型" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "内镜检查" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "器质性病变" } ] }, { "text": "3.胃轻瘫许多全身性的或消化道疾病均可引起胃排空功能的障碍,造成胃轻瘫。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "胃轻瘫" }, { "start_idx": 7, "end_idx": 16, "type": "dis", "entity": "全身性的或消化道疾病" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "胃" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "胃轻瘫" } ] }, { "text": "较常见的原因有糖尿病、尿毒症及结缔组织病。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "糖尿病" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "尿毒症" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "结缔组织病" } ] }, { "text": "在诊功能性消化不良运动障碍亚型时,应仔细排除其他原因所致的胃轻瘫。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "功能性消化不良运动障碍亚型" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "胃轻瘫" } ] }, { "text": "4.慢性难治性腹痛(CIPA)CIPA患者70%为女性,多有身体或心理创伤史。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "慢性难治性腹痛" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "CIPA" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "CIPA" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "心理创伤" } ] }, { "text": "患者常常主诉有长期腹痛(超过6个月),且腹痛弥漫,多伴有腹部以外的症状。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "腹痛弥漫" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "腹部以外的症状" } ] }, { "text": "这类患者多数有严重的潜在的心理疾患,包括抑郁、焦虑和躯体形态的紊乱。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "心理疾患" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "焦虑" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "躯体形态的紊乱" } ] }, { "text": "对这类患者应提供多种方式的心理、行为和药物联合治疗。", "entities": [ { "start_idx": 13, "end_idx": 24, "type": "pro", "entity": "心理、行为和药物联合治疗" } ] }, { "text": "【预防】并非所有的功能性消化不良的患儿均需接受药物治疗。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "如建立良好的生活习惯,避免心理紧张因素和刺激性食物,避免服用非甾体类消炎药。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "pro", "entity": "建立良好的生活习惯" }, { "start_idx": 11, "end_idx": 24, "type": "pro", "entity": "避免心理紧张因素和刺激性食物" }, { "start_idx": 30, "end_idx": 36, "type": "dru", "entity": "非甾体类消炎药" } ] }, { "text": "对于无法停药者应同时应用胃黏膜保护剂或H2受体拮抗剂。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dru", "entity": "胃黏膜保护剂" } ] }, { "text": "【治疗】(一)一般治疗一般说来,治疗中最重要的是在医生和患者之间建立一种牢固的治疗关系。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "一般治疗" } ] }, { "text": "经过诊断性检查之后,应告诉患者功能性消化不良的诊断,同时向他们进行宣教、消除疑虑,抑制“过分检查”的趋势,将重点从寻找症状的原因转移到帮助患者克服这些症状。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "诊断性检查" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "改变他们的生活环境是不太可能的,应指导患者减轻应激反应的措施,如体育锻炼和良好的饮食睡眠习惯。", "entities": [ { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "体育锻炼" }, { "start_idx": 37, "end_idx": 45, "type": "pro", "entity": "良好的饮食睡眠习惯" } ] }, { "text": "(二)药物治疗对于功能性消化不良,药物治疗的效果不太令人满意。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "药物治疗" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "而且,症状的改善也可能与自然病程中症状的时轻时重有关,或者是安慰剂的作用。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "安慰剂" } ] }, { "text": "在症状加重时,药物治疗可能会有帮助,但应尽量减少用量,只有在有明确益处时才可长期使用。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "药物治疗" } ] }, { "text": "下面介绍一下治疗功能性消化不良的常用药物:1.抗酸剂和制酸剂(1)抗酸剂:抗酸剂(碳酸氢钠、氢氧化铝、氧化镁、三硅酸镁):在我国常用的有碳酸钙口服液、复方氢氧化铝片及胃达。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "抗酸剂" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "制酸剂" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "抗酸剂" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "抗酸剂" }, { "start_idx": 41, "end_idx": 44, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 46, "end_idx": 49, "type": "dru", "entity": "氢氧化铝" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "氧化镁" }, { "start_idx": 68, "end_idx": 73, "type": "dru", "entity": "碳酸钙口服液" }, { "start_idx": 75, "end_idx": 81, "type": "dru", "entity": "复方氢氧化铝片" }, { "start_idx": 83, "end_idx": 84, "type": "dru", "entity": "胃达" } ] }, { "text": "这类药物对于缓解饥饿痛、反酸及胃灼热不良反应。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "饥饿痛" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "反酸" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "胃" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "胃灼热" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "不良反应" } ] }, { "text": "(2)抑酸剂:抑酸剂主要指H2受体拮抗剂和质子泵抑制剂。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "抑酸剂" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "抑酸剂" }, { "start_idx": 27, "end_idx": 32, "type": "dru", "entity": "质子泵抑制剂" } ] }, { "text": "H2受体拮抗剂治疗功能性消化不良的报道很多,药物的疗效在统计学上显著优于安慰剂。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "安慰剂" } ] }, { "text": "它们抑制胃酸的分泌,无论对溃疡亚型和反流亚型都有明显的效果。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "胃酸" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "溃疡亚型" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "反流亚型" } ] }, { "text": "质子泵抑制剂奥美拉唑,可抑制壁细胞H+-K+-ATP酶,抑制酸分泌作用强,持续时间长,适用于H2受体拮抗剂治疗无效的患者。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "质子泵抑制剂" }, { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "奥美拉唑" }, { "start_idx": 14, "end_idx": 26, "type": "bod", "entity": "壁细胞H+-K+-ATP酶" } ] }, { "text": "2.促动力药物根据有对照组的临床验证,现已肯定甲氧氯普胺(胃复安)、多潘立酮(吗丁啉)及西沙比利对消除功能性消化不良诸症状确有疗效。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "促动力药物" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "甲氧氯普胺" }, { "start_idx": 29, "end_idx": 31, "type": "dru", "entity": "胃复安" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "多潘立酮" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "吗丁啉" }, { "start_idx": 44, "end_idx": 47, "type": "dru", "entity": "西沙比利" }, { "start_idx": 51, "end_idx": 57, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "儿科多潘立酮应用较多。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dep", "entity": "儿科" }, { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "多潘立酮" } ] }, { "text": "(1)甲氧氯普胺:有抗中枢和外周多巴胺作用,同时兴奋5-HT4受体,促进内源性乙酰胆碱释放,增加胃窦-十二指肠协调运动,促进胃排空。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "甲氧氯普胺" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "中枢" }, { "start_idx": 14, "end_idx": 18, "type": "bod", "entity": "外周多巴胺" }, { "start_idx": 26, "end_idx": 32, "type": "bod", "entity": "5-HT4受体" }, { "start_idx": 36, "end_idx": 42, "type": "bod", "entity": "内源性乙酰胆碱" }, { "start_idx": 48, "end_idx": 54, "type": "bod", "entity": "胃窦-十二指肠" }, { "start_idx": 62, "end_idx": 62, "type": "bod", "entity": "胃" } ] }, { "text": "因不良反应较多,故临床应用逐渐减少。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "不良反应" } ] }, { "text": "(2)多潘立酮:为外周多巴胺受体阻抗剂,可促进固体和液体胃排空,抑制胃容纳舒张,协调胃窦-十二指肠运动,松弛幽门,从而缓解消化不良症状。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "多潘立酮" }, { "start_idx": 9, "end_idx": 18, "type": "dru", "entity": "外周多巴胺受体阻抗剂" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "胃" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "胃" }, { "start_idx": 42, "end_idx": 48, "type": "bod", "entity": "胃窦-十二指肠" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "幽门" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "消化不良" } ] }, { "text": "1岁以下儿童由于血脑屏障功能发育尚未完全,故不宜服用。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "血脑屏障" } ] }, { "text": "(3)西沙比利:通过促进胃肠道肌层神经丛副交感神经节后纤维末梢乙酰胆碱的释放,增强食管下端括约肌张力,加强食管、胃、小肠和结肠的推进性运动。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "西沙比利" }, { "start_idx": 12, "end_idx": 34, "type": "bod", "entity": "胃肠道肌层神经丛副交感神经节后纤维末梢乙酰胆碱" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "食管" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "括约肌" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "食管" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "胃" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "小肠" }, { "start_idx": 61, "end_idx": 62, "type": "bod", "entity": "结肠" } ] }, { "text": "对胃的作用主要有增加胃窦收缩,改善胃窦-十二指肠协调运动。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "胃" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "胃窦" }, { "start_idx": 17, "end_idx": 23, "type": "bod", "entity": "胃窦-十二指肠" } ] }, { "text": "降低幽门时相性收缩频率,使胃电活动趋于正常,从而加速胃排空。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "幽门" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "胃" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "胃" } ] }, { "text": "临床研究发现该药能明显改善消化不良症状,但因心脏的副作用,故应用受到限制。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "消化不良" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "心脏" } ] }, { "text": "(4)红霉素:虽为抗生素,也是胃动素激动剂,可增加胃近端和远端收缩活力,促进胃推进性蠕动,加速空腹和餐后胃排空,可用于FD小儿。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "红霉素" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "抗生素" }, { "start_idx": 15, "end_idx": 20, "type": "dru", "entity": "胃动素激动剂" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "胃" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "胃" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "胃" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "FD" } ] }, { "text": "3.胃黏膜保护剂这类药物主要有硫糖铝、米索前列醇、恩前列素及蒙脱石散等。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "胃黏膜保护剂" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "硫糖铝" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "米索前列醇" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "恩前列素" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "蒙脱石散" } ] }, { "text": "临床上这类药物的应用主要是由于功能性消化不良的发病可能与慢性胃炎有关,患者可能存在胃黏膜屏障功能的减弱。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "胃黏膜屏障" } ] }, { "text": "4.5-HT3受体拮抗剂和阿片类受体激动剂这两类药物促进胃排空的作用很弱,用于治疗功能性消化不良患者的原理是调节内脏感觉阈。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "5-HT3" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "受体拮抗剂" }, { "start_idx": 13, "end_idx": 20, "type": "dru", "entity": "阿片类受体激动剂" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "胃" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "但此类药在儿科中尚无用药经验。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dep", "entity": "儿科" } ] }, { "text": "5.抗焦虑药国内有人使用小剂量多虑平和多潘立酮结合心理疏导治疗功能性消化不良患者,发现对上腹痛嗳气等症状有明显的缓解作用,较之不使用多虑平的患者有明显提高。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "抗焦虑药" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "多虑平" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "多潘立酮" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "心理疏导" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "功能性消化不良" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "腹" }, { "start_idx": 44, "end_idx": 46, "type": "sym", "entity": "上腹痛" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "嗳气" }, { "start_idx": 66, "end_idx": 68, "type": "dru", "entity": "多虑平" } ] }, { "text": "因此,在对FD的治疗中,利用药物对心理障碍进行治疗有一定的临床意义。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "FD" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "心理障碍" } ] }, { "text": "第五章癫痫癫痫(epilepsy)是由多种病因引起的慢性脑部疾患,以脑部神经元过度放电所致的突然、反复和短暂的中枢神经系统功能失常为特征。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "癫痫" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "癫痫" }, { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "epilepsy" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "慢性脑部疾患" }, { "start_idx": 34, "end_idx": 67, "type": "sym", "entity": "脑部神经元过度放电所致的突然、反复和短暂的中枢神经系统功能失常为特征" } ] }, { "text": "根据所侵犯神经元的部位和发放的范围,可表现为运动、感觉、意识、行为及自主神经功能等不同脑功能障碍。", "entities": [ { "start_idx": 22, "end_idx": 47, "type": "sym", "entity": "运动、感觉、意识、行为及自主神经功能等不同脑功能障碍" } ] }, { "text": "2005年国际抗癫痫联盟(ILAE)对癫痫推荐的定义为:癫痫是一种脑部疾患,其特点是持续存在能产生癫痫发作的脑部持久性改变,并出现相应的神经生物学、认知、心理学以及社会学等方面的后果。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "癫痫" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "癫痫" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "脑部疾患" }, { "start_idx": 68, "end_idx": 90, "type": "sym", "entity": "神经生物学、认知、心理学以及社会学等方面的后果" } ] }, { "text": "【流行病学】我国癫痫的年发病率30/10万,以此推断,每年我国新发癫痫在40万例左右;我国癫痫的患病率(又称现患率)一般在4‰~7‰左右,由此推算,我国应有600万左右的癫痫患者。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "癫痫" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "癫痫" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "癫痫" }, { "start_idx": 85, "end_idx": 86, "type": "dis", "entity": "癫痫" } ] }, { "text": "据世界各国流行病学调查,癫痫发病率差异很大,多数结果表明癫痫的年发病率为24/10万~53/10万之间,多数发展中国家癫痫发病率高于发达国家;世界卫生组织估计,全球大约有5000万癫痫患者。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "癫痫" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "癫痫" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "癫痫" }, { "start_idx": 90, "end_idx": 91, "type": "dis", "entity": "癫痫" } ] }, { "text": "所以癫痫是一世界范围常见病和多发病,也是小儿神经系统的常见病。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "癫痫" } ] }, { "text": "【病因】癫痫的病因复杂多样,构成癫痫发作的因素包括遗传因素、脑内致痫性损伤因素以及诱发性因素等,不同的年龄往往有不同的病因范围。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "癫痫" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "癫痫" }, { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "脑内致痫性损伤" } ] }, { "text": "EEG背景波正常,呈特定部位局限性或双侧对称同步痫样放电。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" } ] }, { "text": "原发性癫痫是癫痫遗传学研究的主要对象,现在的研究显示,特发性癫痫多为中枢神经系统的离子通道异常。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "原发性癫痫" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "特发性癫痫" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "(二)症状性(symptomatic)指能找到明确病因的癫痫,包括脑结构异常或者影响脑功能的各种因素。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "癫痫" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "脑" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "脑结构异常" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "脑" }, { "start_idx": 40, "end_idx": 49, "type": "sym", "entity": "影响脑功能的各种因素" } ] }, { "text": "这类癫痫可有多种形式的临床发作,除有局限性脑电异常外,EEG背景波多异常,并有大量的痫样发电。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "癫痫" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "有局限性脑电异常" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "EEG" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "大量的痫样发电" } ] }, { "text": "随着医学的进步与检查手段的不断发展和丰富,能够寻找到病因的癫痫病例越来越多。", "entities": [ { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "癫痫" } ] }, { "text": "【发病机制】癫痫的发病机制虽然有许多进展,但没有一种能解释全部的癫痫发作,多数认为不同癫痫有着不同的发病机制。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "癫痫" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "癫痫" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "癫痫" } ] }, { "text": "神经元的高度同步化发放是癫痫发作的特征,其产生的条件涉及一系列生化、免疫以及遗传等方面的变化。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "神经元的高度同步化发放" } ] }, { "text": "(一)生化方面如引起神经元去极化而发生兴奋性突触后电位的兴奋性氨基酸(谷氨酸、天冬氨酸及其受体激动剂N甲基天冬氨酸、红藻氨酸和使君子氨酸等)活力增加;引起神经元超级化而发生抑制性突触后电位的抑制性氨基酸(γ-氨基丁酸、牛磺酸、甘氨酸、5-羟色胺及去甲肾上腺素等)活力减弱,γ-氨基丁酸受体减少均可使细胞兴奋性增强;脑部活性自由基(、QH-、H2O2及NO等)增多对机体细胞的毒性作用;钙通道开放致Ca2+异常内流以及细胞内Ca2+结合蛋白减少等,使细胞内Ca2+积蓄,造成细胞坏死。", "entities": [ { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "谷氨酸" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "天冬氨酸" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "受体" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "激动剂" }, { "start_idx": 50, "end_idx": 56, "type": "bod", "entity": "N甲基天冬氨酸" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "红藻氨酸" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "使君子氨酸" }, { "start_idx": 95, "end_idx": 100, "type": "bod", "entity": "抑制性氨基酸" }, { "start_idx": 102, "end_idx": 107, "type": "bod", "entity": "γ-氨基丁酸" }, { "start_idx": 109, "end_idx": 111, "type": "bod", "entity": "牛磺酸" }, { "start_idx": 113, "end_idx": 115, "type": "bod", "entity": "甘氨酸" }, { "start_idx": 117, "end_idx": 121, "type": "bod", "entity": "5-羟色胺" }, { "start_idx": 123, "end_idx": 128, "type": "bod", "entity": "去甲肾上腺素" }, { "start_idx": 136, "end_idx": 143, "type": "bod", "entity": "γ-氨基丁酸受体" }, { "start_idx": 200, "end_idx": 203, "type": "bod", "entity": "机体细胞" }, { "start_idx": 232, "end_idx": 250, "type": "sym", "entity": "细胞内Ca2+结合蛋白减少" }, { "start_idx": 254, "end_idx": 262, "type": "sym", "entity": "细胞内Ca2+积蓄" }, { "start_idx": 266, "end_idx": 269, "type": "sym", "entity": "细胞坏死" } ] }, { "text": "(二)免疫方面免疫的异常如细胞免疫功能低下;体液免疫中IgA等的缺乏,抗脑抗体的产生均是癫痫发作的潜在原因。", "entities": [ { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "细胞免疫功能低下" }, { "start_idx": 22, "end_idx": 33, "type": "sym", "entity": "体液免疫中IgA等的缺乏" } ] }, { "text": "(三)遗传方面遗传因素是导致癫痫、尤其是经典的特发性癫痫的重要原因。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "癫痫" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "特发性癫痫" } ] }, { "text": "分子遗传学研究发现,大部分遗传性癫痫的分子机制为离子通道或相关分子的结构或功能改变。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "遗传性癫痫" } ] }, { "text": "到目前为止部分单基因及多基因遗传性癫痫的致病基因已明确(详见癫痫综合征内容介绍)。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "遗传性癫痫" } ] }, { "text": "【癫痫发作分类】对癫痫发作进行分类,有助于临床上对抗癫痫药物的选择以及对不同发作药物疗效的评估;有助于研究发作症状学与脑结构系统之间的关系。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "癫痫" } ] }, { "text": "癫痫的分类一直繁多,目前神经科沿用的分类是国际抗癫痫联盟(ILAE)1981年提出的“癫痫发作分类”,依据临床发作形式和脑电图改变分类;1989年“癫痫与癫痫综合征的分类”,除依据临床发作形式及脑电图改变外,还结合发病年龄、病因及转归。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "癫痫" }, { "start_idx": 12, "end_idx": 14, "type": "dep", "entity": "神经科" }, { "start_idx": 60, "end_idx": 62, "type": "pro", "entity": "脑电图" }, { "start_idx": 74, "end_idx": 75, "type": "dis", "entity": "癫痫" }, { "start_idx": 77, "end_idx": 81, "type": "dis", "entity": "癫痫综合征" }, { "start_idx": 97, "end_idx": 99, "type": "pro", "entity": "脑电图" } ] }, { "text": "2001年国际抗癫痫联盟关于癫痫发作和对癫痫诊断的建议,其中关于对癫痫发作的类型(表16-4),癫痫和癫痫综合征新的分类(表16-16)。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "癫痫" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "癫痫" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "癫痫综合征" } ] }, { "text": "表16-2癫痫发作分类(1981年)表16-3癫痫与癫痫综合征分类(1989年)表16-4癫痫发作类型(2001年)表16-5癫痫综合征分类(2001年)【临床表现】(一)部分性发作(partialseizures)部分性发作的临床与脑电图异常放电局限在脑某一部位或从某一局部开始。", "entities": [ { "start_idx": 117, "end_idx": 123, "type": "sym", "entity": "脑电图异常放电" } ] }, { "text": "发作时不伴意识障碍为简单部分性发作;伴有意识障碍为复杂部分性发作;部分性发作也可泛化为全面性发作,而且脑电图由局部放电演变为全脑性放电。", "entities": [ { "start_idx": 20, "end_idx": 31, "type": "sym", "entity": "意识障碍为复杂部分性发作" } ] }, { "text": "1.简单部分性发作(simplepartialseizures)发作开始意识多不丧失,最初发作表现可反映癫痫起源的脑区。", "entities": [ { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "癫痫" } ] }, { "text": "(1)运动性症状:包括:①仅为局灶性运动症状,多为阵挛性发作,任何部位都可以出现局灶性抽搐;②Jackson发作,即发作从一侧口角开始,依次波及手、臂和肩等;③偏转性发作,眼、头甚至躯干向一侧偏转;④姿势性发作,表现为某种特殊姿势,如击剑样姿势;⑤抑制性运动发作,发作时动作停止,语言中断,意识不丧失;⑥发音性发作,表现为重复语言或言语中断;⑦半侧发作(2)感觉症状:包括:①躯体感觉性发作(麻木及疼痛等);②特殊感觉异常(视、听、嗅和味)及幻觉;③眩晕性发作。", "entities": [ { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "阵挛性发作" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "局灶性抽搐" }, { "start_idx": 86, "end_idx": 97, "type": "sym", "entity": "眼、头甚至躯干向一侧偏转" }, { "start_idx": 135, "end_idx": 138, "type": "sym", "entity": "动作停止" }, { "start_idx": 140, "end_idx": 143, "type": "sym", "entity": "语言中断" }, { "start_idx": 145, "end_idx": 149, "type": "sym", "entity": "意识不丧失" }, { "start_idx": 161, "end_idx": 164, "type": "sym", "entity": "重复语言" }, { "start_idx": 166, "end_idx": 169, "type": "sym", "entity": "言语中断" }, { "start_idx": 188, "end_idx": 194, "type": "sym", "entity": "躯体感觉性发作" }, { "start_idx": 196, "end_idx": 197, "type": "sym", "entity": "麻木" }, { "start_idx": 199, "end_idx": 200, "type": "sym", "entity": "疼痛" }, { "start_idx": 205, "end_idx": 210, "type": "sym", "entity": "特殊感觉异常" }, { "start_idx": 221, "end_idx": 222, "type": "sym", "entity": "幻觉" }, { "start_idx": 225, "end_idx": 229, "type": "sym", "entity": "眩晕性发作" } ] }, { "text": "(3)自主神经性症状:包括:胃部不适症状、潮红、苍白、冷汗、心悸、竖毛肌收缩以及瞳孔散大(4)精神症状:常见于复杂部分性发作,包括认知障碍、记忆力障碍、情感问题(恐惧和愤怒)、错觉(视物变大和变小)及幻觉。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "胃部" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "潮红" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "苍白" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "冷汗" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "心悸" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "竖毛肌收缩" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "瞳孔散大" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "认知障碍" }, { "start_idx": 70, "end_idx": 74, "type": "sym", "entity": "记忆力障碍" }, { "start_idx": 76, "end_idx": 86, "type": "sym", "entity": "情感问题(恐惧和愤怒)" }, { "start_idx": 88, "end_idx": 89, "type": "sym", "entity": "错觉" }, { "start_idx": 100, "end_idx": 101, "type": "sym", "entity": "幻觉" } ] }, { "text": "2.复杂部分性发作(complexpartialseizures)有意识障碍、发作性感知觉障碍以及梦游状态等。", "entities": [ { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "意识障碍" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "发作性感知觉障碍" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "梦游" } ] }, { "text": "常有“自动症”,是意识障碍下的不自主动作,表现为口咽自动症、姿势自动症、手部自动症、行走自动症和言语自动症。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "“自动症”" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "不自主动作" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "口咽自动症" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "姿势自动症" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "手部自动症" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "行走自动症" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "言语自动症" } ] }, { "text": "复杂部分性发作可从单纯部分性发作开始,随后出现意识障碍,也可从开始即有意识障碍。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "意识障碍" } ] }, { "text": "可见于颞叶或额叶起源的癫痫。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "颞叶" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "额叶" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "癫痫" } ] }, { "text": "EEG在发作时有颞、额区局灶性放电。", "entities": [ { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "颞" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "额" } ] }, { "text": "3.部分性发作继发为全身性发作小婴儿部分性发作时由于难以确定婴儿发作时的意识水平,往往表现为:①反应性降低:动作突然减少或停止,无动性凝视或茫然,有人称为“颞叶假性失神”(temporalpseudoabsence)或“额叶失神”(frontalabsence),但不是真正的失神发作。", "entities": [ { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "反应性降低" }, { "start_idx": 54, "end_idx": 62, "type": "sym", "entity": "动作突然减少或停止" }, { "start_idx": 64, "end_idx": 68, "type": "sym", "entity": "无动性凝视" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "茫然" } ] }, { "text": "②自动症:常见为口部的简单自动症(如咂嘴、咀嚼、吞咽及吸吮等较原始的动作);或躯干肢体无目的不规则运动,与正常运动很相似。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "自动症" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "咂嘴" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "咀嚼" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "吞咽" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "吸吮" }, { "start_idx": 39, "end_idx": 50, "type": "sym", "entity": "躯干肢体无目的不规则运动" } ] }, { "text": "2001年的癫痫发作分类不同于1981年的发作分类,要点包括:①将癫痫发作分为自限性和持续性,在这两种发作的范畴内,又分为全面性和局灶性两类;②在局灶性发作中不再分为单纯性和复杂性;③在“局灶性感觉性发作”及“局灶性运动性发作”,不再承认有“自主神经症状”,自主神经症状多为癫痫发作伴随现象;④发作的类型明显增多。", "entities": [ { "start_idx": 137, "end_idx": 138, "type": "dis", "entity": "癫痫" } ] }, { "text": "(二)全身性发作全身性常有意识障碍,运动性症状是对称性的,脑电图上表现两侧大脑半球广泛性放电。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "pro", "entity": "脑电图" } ] }, { "text": "1.强直-阵挛性发作(tonic-clonicseizures)发作时突然意识丧失,瞳孔散大,全身肌肉强直或阵挛或强直-阵挛性收缩。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "强直-阵挛性发作" }, { "start_idx": 11, "end_idx": 30, "type": "dis", "entity": "tonic-clonicseizures" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "意识丧失" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "瞳孔散大" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "全身肌肉强直" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "阵挛" }, { "start_idx": 57, "end_idx": 64, "type": "sym", "entity": "强直-阵挛性收缩" } ] }, { "text": "阵挛发作是指肢体及躯干呈有节律性重复的收缩为特征。", "entities": [ { "start_idx": 6, "end_idx": 20, "type": "sym", "entity": "肢体及躯干呈有节律性重复的收缩" } ] }, { "text": "EEG特征表现为背景活动正常或非特异性异常,发作间期异常波在两半球可见棘波、尖波、棘慢波和多棘波等;发作期EEG强直期以10~20Hz节律性棘波发放开始,波幅渐高而频率渐慢;发作结束后可见弥漫性慢波活动,逐渐恢复背景活动。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "棘波" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "尖波" }, { "start_idx": 53, "end_idx": 55, "type": "pro", "entity": "EEG" }, { "start_idx": 77, "end_idx": 85, "type": "sym", "entity": "波幅渐高而频率渐慢" } ] }, { "text": "2.肌阵挛发作(myoclonicseizures)表现为某个或某组肌肉或肌群快速有力的收缩,不超过0.2秒,抽动后肢体或躯干立即恢复原来的姿势(状态),屈肌比伸肌更易受累,上肢明显。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肌阵挛发作" }, { "start_idx": 8, "end_idx": 24, "type": "dis", "entity": "myoclonicseizures" }, { "start_idx": 29, "end_idx": 45, "type": "sym", "entity": "某个或某组肌肉或肌群快速有力的收缩" } ] }, { "text": "婴儿期肌阵挛的特点有2种:①全身性粗大肌阵挛,表现为躯干、颈部以及四肢近端突然猛烈抽动,动作幅度大、孤立的或连续的。", "entities": [ { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "全身性粗大肌阵挛" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "躯干" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "颈部" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "四肢近端" }, { "start_idx": 26, "end_idx": 42, "type": "sym", "entity": "躯干、颈部以及四肢近端突然猛烈抽动" } ] }, { "text": "EEG表现为高波幅多棘慢波爆发,或突然广泛低电压。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" }, { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "高波幅多棘慢波爆发" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "突然广泛低电压" } ] }, { "text": "②散在游走性肌阵挛,表现为四肢远端、面部小组肌群幅度较小的抽动,多部位游走性,EEG为持续性弥漫性慢波多灶性棘波、尖波。", "entities": [ { "start_idx": 13, "end_idx": 30, "type": "sym", "entity": "四肢远端、面部小组肌群幅度较小的抽动" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "多部位游走性" }, { "start_idx": 39, "end_idx": 41, "type": "pro", "entity": "EEG" }, { "start_idx": 43, "end_idx": 58, "type": "sym", "entity": "持续性弥漫性慢波多灶性棘波、尖波" } ] }, { "text": "3.失张力发作表现为突然发生的肌张力减低或丧失,不能维持原来的姿势,导致突然跌倒或姿势不稳。", "entities": [ { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "肌张力减低或丧失" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "不能维持原来的姿势" }, { "start_idx": 36, "end_idx": 44, "type": "sym", "entity": "突然跌倒或姿势不稳" } ] }, { "text": "有时发作时间短暂,在未摔倒在地时意识已恢复,可立即站起;长时间的失张力发作可持续一至数分钟,表现全身松软,凝视,但无运动性症状。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "时间短暂" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "全身松软" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "凝视" } ] }, { "text": "EEG发作间期和发作期可表现为全导棘慢波或多棘慢波发放;发作期还可表现为低波幅或高波幅快活动和弥漫性低电压。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "全导棘慢波或多棘慢波发放" }, { "start_idx": 36, "end_idx": 45, "type": "sym", "entity": "低波幅或高波幅快活动" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "弥漫性低电压" } ] }, { "text": "4.失神发作分为典型失神和不典型失神,典型失神主要见于儿童失神癫痫和青少年失神癫痫;不典型失神主要见于Lennox-Gastaut综合征,也可见于其他儿童癫痫综合征。", "entities": [ { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "儿童失神癫痫" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "青少年失神癫痫" }, { "start_idx": 51, "end_idx": 67, "type": "dis", "entity": "Lennox-Gastaut综合征" }, { "start_idx": 75, "end_idx": 81, "type": "dis", "entity": "儿童癫痫综合征" } ] }, { "text": "(三)癫痫综合征不同年龄段常见的癫痫综合征的诊断要点介绍如下。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "癫痫综合征" } ] }, { "text": "1.良性家族性新生儿惊厥为常染色体显性遗传,往往有惊厥家族史,基因定位多位于20q13.2,少数定位于8q染色体上,致病基因为KCNQ2和KCNQ3。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "良性家族性新生儿惊厥" } ] }, { "text": "生后2~3天内发病,惊厥形式以阵挛为主,可以表现为某一肢体或面部抽动,也可表现为全身阵挛;少数表现为广泛性强直。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "阵挛" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "某一肢体或面部抽动" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "阵挛" }, { "start_idx": 50, "end_idx": 54, "type": "sym", "entity": "广泛性强直" } ] }, { "text": "有时表现为呼吸暂停,发作频繁,发作持续时间较短。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "频繁" } ] }, { "text": "从病史及体格检查中找不到病因,脑电图无特殊异常,生化检查及神经影像学检查均正常。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "pro", "entity": "脑电图" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "神经影像学" } ] }, { "text": "2.良性新生儿惊厥本病遗传不明显。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "良性新生儿惊厥" } ] }, { "text": "脑电图在发作间期常可见尖型θ波。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "可见尖型θ波" } ] }, { "text": "3.早发性肌阵挛脑病生后第1天或数天以内起病;主要表现为难治性频繁的肌阵挛发作;脑电图也表现为暴发抑制波形;本病可能与遗传代谢障碍有关,而无明显的神经影像学异常;本病预后不良,多数早期死亡。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "早发性肌阵挛脑病" }, { "start_idx": 28, "end_idx": 38, "type": "sym", "entity": "难治性频繁的肌阵挛发作" }, { "start_idx": 73, "end_idx": 76, "type": "pro", "entity": "神经影像" } ] }, { "text": "4.大田原综合征生后3个月以内发病,多在1个月之内起病;主要为强直痉挛性发作;脑电图表现为暴发抑制波形;常见病因为脑部结构异常,也有隐源性病因。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "大田原综合征" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "强直痉挛性发作" }, { "start_idx": 39, "end_idx": 41, "type": "pro", "entity": "脑电图" } ] }, { "text": "5.婴儿痉挛又称为West综合征,较常见的严重的癫痫综合征。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "婴儿痉挛" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "West综合征" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "癫痫综合征" } ] }, { "text": "EEG表现为高度失律,各导联见到不规则、杂乱、不对称、高波幅慢波、棘波、尖波及多棘慢波。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "高度失律" }, { "start_idx": 11, "end_idx": 25, "type": "sym", "entity": "各导联见到不规则、杂乱、不对称" } ] }, { "text": "引起本病的继发性原因多种多样,如脑发育障碍所致的各种畸形、宫内感染、围生期脑损伤,核黄疸、免疫缺陷、代谢异常、生后感染、窒息以及染色体异常等因素,均可引起本病。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "畸形" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "宫内感染" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "围生期脑损伤" }, { "start_idx": 41, "end_idx": 43, "type": "sym", "entity": "核黄疸" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "免疫缺陷" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "代谢异常" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "生后感染" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "窒息" }, { "start_idx": 64, "end_idx": 68, "type": "sym", "entity": "染色体异常" } ] }, { "text": "其中,10%为结节性硬化。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "结节性硬化" } ] }, { "text": "本病常合并严重的智力倒退或运动发育落后,多数病儿转变为其他形式的发作,特别以Lennox-Gastaut综合征最为多见。", "entities": [ { "start_idx": 38, "end_idx": 54, "type": "dis", "entity": "Lennox-Gastaut综合征" } ] }, { "text": "6.婴儿良性肌阵挛癫痫6个月~2岁间发病,患儿神经发育正常;发作表现为全身肌阵挛;EEG发作期表现为弥漫性棘慢波或多棘慢波,发作间期常无异常放电;以后良好。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "婴儿良性肌阵挛癫痫" } ] }, { "text": "7.婴儿重症肌阵挛癫痫1978年Dravet首次描述本病,目前明确其致病基因为SCN1A。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "婴儿重症肌阵挛癫痫" } ] }, { "text": "8.Lennox-Gastaut综合征1~8岁发病,临床发作形式多样性是本综合征的特点,如强直发作、不典型失神、失张力发作和肌阵挛发作,患儿可同时存在几种发作形式,也可由一种形式转变为另一种形式;EEG在发作间期表现为全导0.5~2.5Hz慢的棘慢波。", "entities": [ { "start_idx": 2, "end_idx": 18, "type": "dis", "entity": "Lennox-Gastaut综合征" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "强直发作" }, { "start_idx": 50, "end_idx": 54, "type": "sym", "entity": "不典型失神" }, { "start_idx": 56, "end_idx": 60, "type": "sym", "entity": "失张力发作" }, { "start_idx": 62, "end_idx": 66, "type": "sym", "entity": "肌阵挛发作" }, { "start_idx": 98, "end_idx": 100, "type": "pro", "entity": "EEG" } ] }, { "text": "9.肌阵挛-站立不能发作癫痫又称Doose综合征,都有遗传因素。", "entities": [ { "start_idx": 2, "end_idx": 13, "type": "dis", "entity": "肌阵挛-站立不能发作癫痫" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "Doose综合征" } ] }, { "text": "临床发作以肌阵挛-站立不能发作为特征性表现,表现为点头、弯腰以及两臂上举,常有跌倒,不能站立。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "点头" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "弯腰" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "两臂上举" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "跌倒" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "不能站立" } ] }, { "text": "EEG在发作期或发作期间均可见到不规则棘慢波或多棘慢波,背景波正常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" } ] }, { "text": "10.儿童良性癫痫伴有中央-颞区棘波是小儿癫痫中常见的一种类型,多在5~10岁间发病,本病与遗传有关,往往有癫痫家族史。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "儿童良性癫痫" } ] }, { "text": "表现为口咽部感觉异常及运动性发作,随后出现半侧面部肌肉抽搐及同侧上下肢抽动,有时可发展为全身性抽动。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "口咽部感觉异" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "运动性发作" }, { "start_idx": 21, "end_idx": 28, "type": "sym", "entity": "半侧面部肌肉抽搐" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "同侧上下肢抽动" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "全身性抽动" } ] }, { "text": "11.具有枕区放电的小儿癫痫发病年龄多见于4~8岁,男孩略多于女孩。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "具有枕区放电的小儿癫痫" } ] }, { "text": "发作可在清醒或入睡时,惊厥表现为半侧阵挛发作或扩展为全身强直-阵挛发作。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "半侧阵挛发作" }, { "start_idx": 23, "end_idx": 34, "type": "sym", "entity": "扩展为全身强直-阵挛发作" } ] }, { "text": "惊厥前部分病儿出现视觉症状,如一过性视力丧失,视野出现暗点及幻视等。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "一过性视力丧失" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "视野出现暗点" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "幻视" } ] }, { "text": "1/3病例发作后有头痛、恶心及呕吐。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "头痛" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "恶心" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "呕吐" } ] }, { "text": "EEG在发作间期表现为枕部和后颞部出现一侧或双侧高波幅棘波或尖波,这种异常放电睁眼时消失,闭眼后1~20秒重复出现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "枕部" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "后颞部" }, { "start_idx": 11, "end_idx": 31, "type": "sym", "entity": "枕部和后颞部出现一侧或双侧高波幅棘波或尖波" } ] }, { "text": "12.获得性失语性癫痫又称为Landau-Kleffner综合征,4~7岁发病最多,男孩多于女孩,发病前语言功能正常,听觉失认为特征,失语表现为能听见声音,但不能理解语言的含意,逐渐发展为语言表达障碍。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "获得性失语性癫痫" }, { "start_idx": 14, "end_idx": 31, "type": "dis", "entity": "Landau-Kleffner综合征" }, { "start_idx": 72, "end_idx": 87, "type": "sym", "entity": "能听见声音,但不能理解语言的含意" }, { "start_idx": 94, "end_idx": 99, "type": "sym", "entity": "语言表达障碍" } ] }, { "text": "大约有一半病人首发症状是失语,另1/2病人首发症状为惊厥,惊厥为部分性发作或全身性发作;约有17%~25%病儿没有惊厥发作;2/3病人有明显的行为异常。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "失语" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "惊厥" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "惊厥" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "部分性发作" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "全身性发作" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "惊厥" } ] }, { "text": "EEG背景波正常,一侧或双侧颞区阵发性高幅棘波、尖波或棘慢波,睡眠时异常放电明显增多。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" }, { "start_idx": 9, "end_idx": 29, "type": "sym", "entity": "一侧或双侧颞区阵发性高幅棘波、尖波或棘慢波" }, { "start_idx": 31, "end_idx": 41, "type": "sym", "entity": "睡眠时异常放电明显增多" } ] }, { "text": "13.慢波睡眠中持续棘慢波的癫痫发病为年龄依赖性,多在3~10岁发病,临床上存在获得性认知功能障碍,80%~90%的患者有部分性或全面性发作。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "慢波睡眠中持续棘慢波的癫痫" } ] }, { "text": "EEG呈现慢波睡眠中持续性癫痫样放电。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" } ] }, { "text": "14.儿童失神癫痫4~8岁起病,6~7岁发病最多,女孩多于男孩。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "儿童失神癫痫" } ] }, { "text": "EEG表现为双侧对称、弥漫性高波幅每秒3次棘慢波。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "双侧对称" }, { "start_idx": 11, "end_idx": 23, "type": "sym", "entity": "弥漫性高波幅每秒3次棘慢波" } ] }, { "text": "15.青少年失神癫痫青春期左右发病,7~17岁起病,发病年龄高峰在10~12岁,男女性别无差异,失神发作频率较少,不一定每天均有发作,多伴有全身强直-阵挛发作。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "青少年失神癫痫" }, { "start_idx": 70, "end_idx": 76, "type": "sym", "entity": "全身强直-阵挛" } ] }, { "text": "EEG表现为对称的棘慢波,每秒3.5~4次,额部占优势。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "对称的棘慢波" } ] }, { "text": "16.少年肌阵挛癫痫青春期前后发病,男女性别无大差异。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "少年肌阵挛癫痫" } ] }, { "text": "发作时主要表现为肌阵挛,突然发生肩外展、肘屈曲、屈髋、屈膝以及跌倒,常伴膈肌收缩,发作多在醒后不久发生。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "肌阵挛" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "肩外展" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "肘屈曲" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "屈髋" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "屈膝" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "跌倒" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "膈肌收缩" } ] }, { "text": "也可能单个的发作或重复发作最后转为全身强直-阵挛发作。", "entities": [ { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "全身强直-阵挛" } ] }, { "text": "EEG为弥漫的每秒3~6次的棘慢波或多棘慢波。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "EEG" } ] }, { "text": "17.觉醒时全身强直-阵挛癫痫多发生在10~20岁之间,16~17岁为高峰,本病有遗传倾向,大约10%病例有癫痫家族史。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "dis", "entity": "觉醒时全身强直-阵挛癫痫" } ] }, { "text": "EEG可见弥漫性异常放电,表现为棘慢合并失神棘慢波。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "合并失神" } ] }, { "text": "18.肌阵挛性失神癫痫多有遗传背景,目前多考虑特发性的原因。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "肌阵挛性失神癫痫" } ] }, { "text": "出生后数月以至青春期都可发病,发病高峰在7岁左右,以肌阵挛性失神为特征性表现,常伴有强直性收缩。", "entities": [ { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "肌阵挛性失神" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "强直性收缩" } ] }, { "text": "19.Rsmussen综合征是一特殊的、主要影响一侧大脑半球伴有难治性部分性癫痫,进行性严重认知障碍与偏瘫发生,神经影像学早期正常,以后出现一侧大脑半球进行性萎缩,EEG呈现背景活动不对称慢波活动,一侧为主的癫痫样放电。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "Rsmussen综合征" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "难治性部分性癫痫" }, { "start_idx": 70, "end_idx": 80, "type": "sym", "entity": "一侧大脑半球进行性萎缩" }, { "start_idx": 82, "end_idx": 84, "type": "pro", "entity": "EEG" } ] }, { "text": "20.全面性癫痫伴热性惊厥附加症为常染色体显性遗传方式,是一多个基因受累(致病基因包括SCN1B、SCN1A、SCN2A和GABAG2)的单基因遗传癫痫。", "entities": [ { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "全面性癫痫伴热性惊厥附加症" }, { "start_idx": 69, "end_idx": 75, "type": "dis", "entity": "单基因遗传癫痫" } ] }, { "text": "家族成员中存在热性惊厥或多种发作形式,如热性惊厥附加症、失神发作、肌阵挛发作以及部分性发作等,每个受累者可以有一种或多种发作形式。", "entities": [ { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "热性惊厥附加症" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "失神发作" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "肌阵挛发作" } ] }, { "text": "21.边缘叶癫痫和新皮层癫痫内侧颞叶癫痫为边缘叶癫痫,外侧颞叶癫痫、额叶癫痫、顶叶癫痫以及枕叶癫痫属于新皮层癫痫。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "边缘叶癫痫" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "新皮层癫痫" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "内侧颞叶癫痫" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "边缘叶癫痫" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "外侧颞叶癫痫" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "额叶癫痫" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "顶叶癫痫" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "枕叶癫痫" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "新皮层癫痫" } ] }, { "text": "(四)癫痫持续状态是指癫痫发作持续30分钟以上,或反复发作,且发作间期意识不能恢复。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "癫痫" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "癫痫" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "反复发作" }, { "start_idx": 31, "end_idx": 40, "type": "sym", "entity": "发作间期意识不能恢复" } ] }, { "text": "1.惊厥性癫痫持续状态是指阵发性或连续强直和(或)阵挛运动性发作,意识不恢复者,伴有两侧性脑电图的痫性放电,持续时间超过30分钟。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "惊厥性癫痫" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "阵发性或连续强直" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "阵挛运动性发作" } ] }, { "text": "全身性惊厥持续状态往往是儿科急诊,全面性强直-阵挛性发作、阵挛性发作、强直性发作以及肌阵挛发作均可持续癫痫持续状态;部分性惊厥发作也可呈局灶性惊厥癫痫持续状态。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dep", "entity": "儿科" }, { "start_idx": 17, "end_idx": 27, "type": "sym", "entity": "全面性强直-阵挛性发作" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "阵挛性发作" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "强直性发作" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "肌阵挛发作" }, { "start_idx": 68, "end_idx": 78, "type": "sym", "entity": "局灶性惊厥癫痫持续状态" } ] }, { "text": "2.非惊厥性癫痫持续状态是指持续发作的不同程度意识障碍、认知与行为异常,不伴有惊厥发生的脑功能障碍,伴有脑电图监护异常,行为异常、脑电图监护异常,持续时间大于30分钟者。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "非惊厥性癫痫" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "行为异常" }, { "start_idx": 65, "end_idx": 69, "type": "pro", "entity": "脑电图监护" } ] }, { "text": "非惊厥性癫痫持续状态主要包括典型失神性癫痫状态、非典型失神癫痫状态或精神运动性癫痫状态,可由全身性与部分性发作发展而来,其共同的特点为意识模糊、精神错乱及行为的改变,发作期EEG脑电背景活动变慢,同时伴有痫性放电,而发作间期EEG脑电活动增快。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "非惊厥性癫痫" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "典型失神性癫痫" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "非典型失神癫痫" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "精神运动性癫痫" }, { "start_idx": 67, "end_idx": 70, "type": "sym", "entity": "意识模糊" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "精神错乱" }, { "start_idx": 77, "end_idx": 81, "type": "sym", "entity": "行为的改变" }, { "start_idx": 86, "end_idx": 88, "type": "pro", "entity": "EEG" }, { "start_idx": 89, "end_idx": 96, "type": "sym", "entity": "脑电背景活动变慢" }, { "start_idx": 102, "end_idx": 105, "type": "sym", "entity": "痫性放电" }, { "start_idx": 112, "end_idx": 114, "type": "pro", "entity": "EEG" }, { "start_idx": 115, "end_idx": 120, "type": "sym", "entity": "脑电活动增快" } ] }, { "text": "非惊厥性癫痫状态可导致永久性认知和记忆功能障碍。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "永久性认知" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "记忆功能障碍" } ] }, { "text": "此外,对反复发作性症状的患儿,还应根据临床及脑电图检查鉴别其他非癫痫发作的疾病,如屏气发作、睡眠障碍、晕厥、习惯性阴部摩擦、多发性抽动以及心因性发作等。", "entities": [ { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "屏气发作" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "睡眠障碍" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "晕厥" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "习惯性阴部摩擦" }, { "start_idx": 62, "end_idx": 66, "type": "sym", "entity": "多发性抽动" }, { "start_idx": 69, "end_idx": 73, "type": "sym", "entity": "心因性发作" } ] }, { "text": "(一)临床资料癫痫的诊断主要结合病史,临床表现各种形式的发作,具突然发生、反复发作以及自行缓解的特点。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "癫痫" } ] }, { "text": "(二)脑电图检查EEG检查对癫痫的诊断和分类有很大价值,可出现各种阵发性活动,如尖波、棘波、尖慢波、棘慢波、多棘波以及多棘慢波等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "脑电图检查" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "EEG检查" } ] }, { "text": "一般常规脑电图阳性率接近50%左右;加上过度换气、闪光刺激及睡眠脑电图诱发试验可提高20%阳性率;一些多功能脑电图描记仪,Hoter脑电图仪,视屏智能化脑电图监测仪,观察与临床同步的痫性放电,使之阳性率提高至85%以上。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "pro", "entity": "脑电图" }, { "start_idx": 54, "end_idx": 59, "type": "equ", "entity": "脑电图描记仪" }, { "start_idx": 61, "end_idx": 69, "type": "equ", "entity": "Hoter脑电图仪" }, { "start_idx": 71, "end_idx": 81, "type": "equ", "entity": "视屏智能化脑电图监测仪" } ] }, { "text": "做脑电图时注意,原服的抗癫痫药物不需停用,以免诱发癫痫发作;脑电图阴性也不能完全排除癫痫,但仅有脑电图的痫样放电而无临床发作不能诊断为癫痫。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "pro", "entity": "脑电图" }, { "start_idx": 48, "end_idx": 50, "type": "pro", "entity": "脑电图" } ] }, { "text": "(三)辅助检查各种实验室检查或神经影像学检查帮助寻找癫痫的病因和评价预后。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "实验室检查" }, { "start_idx": 15, "end_idx": 21, "type": "pro", "entity": "神经影像学检查" } ] }, { "text": "(四)神经系统功能评价在儿童癫痫的诊断中还应关注神经系统其他方面异常的诊断及全身各系统并发疾病的诊断。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "儿童癫痫" } ] }, { "text": "【治疗】癫痫的治疗目的是控制癫痫发作,提高患儿生活质量。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "癫痫" } ] }, { "text": "癫痫的综合治疗包括药物治疗(以抗癫痫药物治疗为主)和非药物治疗(预防危险因素、心理治疗、外科治疗、酮源性饮食治疗及病因治疗等)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "癫痫" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "药物治疗" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "抗癫痫药物" }, { "start_idx": 26, "end_idx": 30, "type": "pro", "entity": "非药物治疗" }, { "start_idx": 32, "end_idx": 37, "type": "pro", "entity": "预防危险因素" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "心理治疗" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "外科治疗" }, { "start_idx": 49, "end_idx": 55, "type": "pro", "entity": "酮源性饮食治疗" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "病因治疗" } ] }, { "text": "2.根据发作类型选药药物选择目前主要根据癫痫的发作类型或癫痫综合征的类型选药,不合适的选药甚或加重癫痫发作(表16-16)。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "癫痫" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "癫痫综合征" } ] }, { "text": "3.提倡单药治疗尽量采用单一的抗癫痫药物,80%病例单药治疗满意,剂量从小至大,达到有效治疗剂量,特别是卡马西平、氯硝西泮、扑痫酮及新的抗癫痫药拉莫三嗪、妥吡酯等,可减少不良反应。", "entities": [ { "start_idx": 52, "end_idx": 55, "type": "dru", "entity": "卡马西平" }, { "start_idx": 57, "end_idx": 60, "type": "dru", "entity": "氯硝西泮" }, { "start_idx": 62, "end_idx": 64, "type": "dru", "entity": "扑痫酮" }, { "start_idx": 72, "end_idx": 75, "type": "dru", "entity": "拉莫三嗪" }, { "start_idx": 77, "end_idx": 79, "type": "dru", "entity": "妥吡酯" } ] }, { "text": "联合用药注意药物间相互作用,如肝酶诱导剂有苯妥英钠、卡马西平、苯巴比妥以及扑痫酮;肝酶抑制剂有丙戊酸钠,联合用药或从合用方案中撤除某一药物可引起错综复杂的血药浓度的变化,了解药物之间相互作用对指导癫痫治疗以及调整药物剂量甚为重要。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "苯妥英钠" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "卡马西平" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "扑痫酮" }, { "start_idx": 47, "end_idx": 50, "type": "dru", "entity": "丙戊酸钠" } ] }, { "text": "有待评估;*首选用药7.疗程要长,停药要慢一般停止发作后需继续服用3~4年,脑电图监测正常后,经过1~2年逐渐减药至停药。", "entities": [ { "start_idx": 44, "end_idx": 46, "type": "pro", "entity": "脑电图" } ] }, { "text": "当然不同病因、不同发作类型的癫痫服药疗程则不相同:失神发作控制后1~2年;新生儿癫痫控制后1/2年;脑炎、脑外伤继发癫痫,发作停止后1年;复杂部分性、失张力性发作或器质性病变引起全身性大发作者3~4年。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "癫痫" } ] }, { "text": "(二)预防复发寻找患者癫痫的病因和诱发因素,应避免各种诱发因素,如感染、外伤、过度兴奋、睡眠剥夺以及有害的感光刺激等,减少癫痫复发的几率。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "感染" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "外伤" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "过度兴奋" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "睡眠剥夺" }, { "start_idx": 50, "end_idx": 56, "type": "sym", "entity": "有害的感光刺激" } ] }, { "text": "(三)外科治疗其适应证主要是长期药物治疗无效的难治性癫痫以及症状性部分性癫痫。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "癫痫" } ] }, { "text": "掌握手术的适应证并进行术前各种检查如脑电图、硬膜下脑电图、SPECT及PET明确异常的部位,癫痫的起源;头部CT及MRI明确脑部结构改变;特别是新进开展的FMRI和IAP检查既可判断病灶的位置,还可确定脑部重要的皮层功能,对于手术的选择很有帮助。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "pro", "entity": "脑电图" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "硬膜下脑电图" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "SPECT" }, { "start_idx": 35, "end_idx": 37, "type": "pro", "entity": "PET" }, { "start_idx": 52, "end_idx": 55, "type": "pro", "entity": "头部CT" }, { "start_idx": 57, "end_idx": 59, "type": "pro", "entity": "MRI" }, { "start_idx": 77, "end_idx": 80, "type": "pro", "entity": "FMRI" }, { "start_idx": 82, "end_idx": 84, "type": "pro", "entity": "IAP" }, { "start_idx": 101, "end_idx": 102, "type": "bod", "entity": "脑部" } ] }, { "text": "至于手术种类常见有大脑半球切除术、皮层切除术、胼胝体切除术、立体定向手术及颞叶切除术等,以达到切除病灶或阻断癫痫放电通路。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "大脑半球切除术" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "皮层切除术" }, { "start_idx": 23, "end_idx": 28, "type": "pro", "entity": "胼胝体切除术" }, { "start_idx": 30, "end_idx": 35, "type": "pro", "entity": "立体定向手术" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "颞叶切除术" } ] }, { "text": "(四)癫痫持续状态治疗惊厥性癫痫持续状态急救治疗见下章(小儿惊厥),是防治的重点;非惊厥性癫痫持续状态虽不会导致危及生命的全身并发症,但临床仍应积极处理,可用氯硝西泮等治疗。", "entities": [ { "start_idx": 79, "end_idx": 82, "type": "dru", "entity": "氯硝西泮" } ] }, { "text": "钙离子拮抗剂(尼莫地平和氟桂利嗪)可以抑制钙离子内流,保护受损神经细胞,同时可预防血管痉挛及防治其引起的脑局部缺血缺氧;辅以使用自由基清除剂及维生素E,具有稳定细胞膜作用;根据癫痫的神经免疫损伤机制,有人主张静脉注射丙种球蛋白添加治疗婴儿痉挛与Lennox-Gastaut综合征[0.4g/(kg•d)×5天/疗程]取得一定疗效。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "钙离子拮抗剂" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "尼莫地平" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "氟桂利嗪" }, { "start_idx": 64, "end_idx": 69, "type": "dru", "entity": "自由基清除剂" }, { "start_idx": 71, "end_idx": 74, "type": "dru", "entity": "维生素E" }, { "start_idx": 104, "end_idx": 107, "type": "pro", "entity": "静脉注射" }, { "start_idx": 108, "end_idx": 112, "type": "dru", "entity": "丙种球蛋白" }, { "start_idx": 117, "end_idx": 120, "type": "dis", "entity": "婴儿痉挛" }, { "start_idx": 122, "end_idx": 138, "type": "dis", "entity": "Lennox-Gastaut综合征" } ] }, { "text": "②此外,部分癫痫患儿伴有不同程度的脑损害,对癫痫小儿发育迟缓、心理障碍、行为异常及学习教育研究已成为日渐关注的问题。", "entities": [ { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "小儿发育迟缓" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "心理障碍" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "行为异常" } ] }, { "text": "第三章甲状腺功能亢进症【概述】甲状腺功能亢进症(hyperthyroidism,甲亢)是指由于甲状腺激素分泌过多所致的临床综合征,常伴有甲状腺肿大、眼球外突及基础代谢率增高等表现。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "甲状腺功能亢进症" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "甲状腺功能亢进症" }, { "start_idx": 24, "end_idx": 38, "type": "dis", "entity": "hyperthyroidism" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "甲亢" }, { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "甲状腺激素分泌过多" }, { "start_idx": 68, "end_idx": 85, "type": "sym", "entity": "甲状腺肿大、眼球外突及基础代谢率增高" } ] }, { "text": "患有Graves病孕妇的胎儿约有2%在出生后会呈现甲亢症状,这是由于母体内高浓度的促甲状腺素受体刺激性抗体经胎盘进入胎儿所致,新生儿甲亢通常在生后3个月左右逐渐缓解。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "Graves病" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "甲亢" }, { "start_idx": 41, "end_idx": 52, "type": "ite", "entity": "促甲状腺素受体刺激性抗体" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "胎盘" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "新生儿甲亢" } ] }, { "text": "【流行病学】根据一项20年回顾性统计,甲亢在成年女性中的发病率约1∶1000/年。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "甲亢" } ] }, { "text": "15岁以下儿童甲亢约占总甲亢发生率5%,多见于青少年。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "甲亢" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "甲亢" } ] }, { "text": "【病理生理和发病机制】弥漫性毒性甲状腺肿是一种自身免疫性疾病,约15%患者亲属中患有同样疾病,近半数亲属中呈现抗甲状腺抗体阳性。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "弥漫性毒性甲状腺肿" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 55, "end_idx": 62, "type": "sym", "entity": "抗甲状腺抗体阳性" } ] }, { "text": "患者及其亲属HLA的某些类型的等位基因分布频率增高。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "HLA" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "等位基因" } ] }, { "text": "国内外资料都已证实本病与HLA-Ⅱ类抗原的某些等位基因类型及自身免疫有关。", "entities": [ { "start_idx": 12, "end_idx": 19, "type": "bod", "entity": "HLA-Ⅱ类抗原" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "等位基因" } ] }, { "text": "在白种人中,Graves病与HLA-B8和HLA-DR3有关,后者发生甲亢的危险增加7倍。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "Graves病" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "HLA-B8" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "HLA-DR3" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "甲亢" } ] }, { "text": "该病还可并发其他与之相关的疾病,例如Addison病、重症肌无力、1型糖尿病、全身性红斑狼疮、类风湿性关节炎、白癜风、特发性血小板减少性紫癜和恶性贫血等。", "entities": [ { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "Addison病" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "重症肌无力" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "1型糖尿病" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "全身性红斑狼疮" }, { "start_idx": 47, "end_idx": 53, "type": "dis", "entity": "类风湿性关节炎" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "白癜风" }, { "start_idx": 59, "end_idx": 69, "type": "dis", "entity": "特发性血小板减少性紫癜" }, { "start_idx": 71, "end_idx": 74, "type": "dis", "entity": "恶性贫血" } ] }, { "text": "患者的甲状腺功能状态与甲状腺自身抗体关系密切,可在体内测到多种甲状腺自身抗体。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "甲状腺" }, { "start_idx": 11, "end_idx": 17, "type": "bod", "entity": "甲状腺自身抗体" }, { "start_idx": 31, "end_idx": 37, "type": "bod", "entity": "甲状腺自身抗体" } ] }, { "text": "据报道,80%~100%的患者可测到TSH受体抗体,此抗体为甲状腺刺激免疫球蛋白,能产生刺激甲状腺功能作用,使甲状腺对碘的摄取增加,cAMP介导的甲状腺激素合成和甲状腺球蛋白合成增加,促进蛋白质合成与细胞生长。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "TSH受体抗体" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "抗体" }, { "start_idx": 30, "end_idx": 39, "type": "bod", "entity": "甲状腺刺激免疫球蛋白" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "甲状腺" }, { "start_idx": 54, "end_idx": 64, "type": "sym", "entity": "使甲状腺对碘的摄取增加" }, { "start_idx": 66, "end_idx": 69, "type": "bod", "entity": "cAMP" }, { "start_idx": 73, "end_idx": 77, "type": "bod", "entity": "甲状腺激素" }, { "start_idx": 81, "end_idx": 86, "type": "bod", "entity": "甲状腺球蛋白" }, { "start_idx": 92, "end_idx": 103, "type": "sym", "entity": "促进蛋白质合成与细胞生长" } ] }, { "text": "甲亢经治疗后随着TSH受体阻断抗体的升高,疾病也逐步缓解。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "甲亢" }, { "start_idx": 8, "end_idx": 16, "type": "ite", "entity": "TSH受体阻断抗体" } ] }, { "text": "在部分甲亢病例中可发现一些其他抗甲状腺的抗体,如甲状腺球蛋白抗体(TGAb)及甲状腺过氧化物酶抗体(TPOAb)。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "甲亢" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "甲状腺" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "抗体" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "甲状腺球蛋白抗体" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "TGAb" }, { "start_idx": 39, "end_idx": 48, "type": "dis", "entity": "甲状腺过氧化物酶抗体" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "TPOAb" } ] }, { "text": "这些抗体在部分正常人中也可存在,其特异性不如TSH受体抗体。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "抗体" } ] }, { "text": "【病理】Graves病的甲状腺腺体呈对称性肿大,滤泡细胞增多,由立方形变为柱状,滤泡内胶质丧失或仅少量染色极浅的胶质,在上皮及胶质间有大量排列成行的空泡,血管明显增多,淋巴组织也增多,有大量淋巴细胞浸润。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "Graves病" }, { "start_idx": 12, "end_idx": 100, "type": "pro", "entity": "甲状腺腺体呈对称性肿大,滤泡细胞增多,由立方形变为柱状,滤泡内胶质丧失或仅少量染色极浅的胶质,在上皮及胶质间有大量排列成行的空泡,血管明显增多,淋巴组织也增多,有大量淋巴细胞浸润" } ] }, { "text": "在电镜下可见滤泡细胞内高尔基体肥大,内浆网和核蛋白体增多,微绒毛数量增多而且变长,呈分泌活跃的表现。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "equ", "entity": "电镜" }, { "start_idx": 6, "end_idx": 14, "type": "bod", "entity": "滤泡细胞内高尔基体" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "内浆网" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "核蛋白体" }, { "start_idx": 0, "end_idx": 48, "type": "sym", "entity": "在电镜下可见滤泡细胞内高尔基体肥大,内浆网和核蛋白体增多,微绒毛数量增多而且变长,呈分泌活跃的表现" } ] }, { "text": "致密的淋巴样集合物内以辅助T细胞(CD4+)为主,在细胞密度较低的区域内则以细胞毒性T细胞(CD8+)为主。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "辅助T细胞" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "CD4+" }, { "start_idx": 32, "end_idx": 35, "type": "ite", "entity": "细胞密度" }, { "start_idx": 44, "end_idx": 50, "type": "bod", "entity": "细胞毒性T细胞" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "CD8+" } ] }, { "text": "甲状腺内浸润的活化B淋巴细胞的百分率高于在周围血管中者。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "甲状腺内" }, { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "活化B淋巴细胞" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "血管中" } ] }, { "text": "推测是由于T抑制细胞的功能障碍,使得T辅助细胞得以表达,被TSH抗原所激活,然后与B细胞发生反应。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "T抑制细胞" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "T辅助细胞" }, { "start_idx": 29, "end_idx": 33, "type": "bod", "entity": "TSH抗原" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "B细胞" } ] }, { "text": "这些细胞分化成为浆细胞,产生促甲状腺激素受体刺激抗体。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "细胞" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "浆细胞" }, { "start_idx": 14, "end_idx": 25, "type": "bod", "entity": "促甲状腺激素受体刺激抗体" } ] }, { "text": "目前认为Graves病浸润性突眼发生机制是抗甲状腺抗体和抗眼眶肌肉抗体与眼外肌和眼眶内成纤维细胞结合,产生毒性反应。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "Graves病" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "浸润性突眼" }, { "start_idx": 21, "end_idx": 26, "type": "bod", "entity": "抗甲状腺抗体" }, { "start_idx": 28, "end_idx": 34, "type": "bod", "entity": "抗眼眶肌肉抗体" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "眼外肌" }, { "start_idx": 40, "end_idx": 47, "type": "bod", "entity": "眼眶内成纤维细胞" } ] }, { "text": "亦有人认为浸润性突眼是眼眶肌肉内沉积甲状腺球蛋白-抗甲状腺球蛋白免疫复合物,引起免疫复合物的炎性反应。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "浸润性突眼" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "眼眶肌肉内" }, { "start_idx": 18, "end_idx": 36, "type": "bod", "entity": "甲状腺球蛋白-抗甲状腺球蛋白免疫复合物" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "免疫复合物" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "炎性反应" } ] }, { "text": "能引起儿童甲状腺功能亢进的其他病因有慢性淋巴性甲状腺炎、亚急性甲状腺炎、甲状腺腺瘤、McCuneAlbright综合征、甲状腺癌、碘过多诱发甲亢、TSH分泌过多、垂体性腺瘤、下丘脑性甲亢以及医源性甲亢等。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "儿童甲状腺功能亢进" }, { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "慢性淋巴性甲状腺炎" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "亚急性甲状腺炎" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "甲状腺腺瘤" }, { "start_idx": 42, "end_idx": 58, "type": "dis", "entity": "McCuneAlbright综合征" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "甲状腺癌" }, { "start_idx": 73, "end_idx": 79, "type": "dis", "entity": "TSH分泌过多" }, { "start_idx": 81, "end_idx": 85, "type": "dis", "entity": "垂体性腺瘤" }, { "start_idx": 87, "end_idx": 92, "type": "dis", "entity": "下丘脑性甲亢" }, { "start_idx": 95, "end_idx": 99, "type": "dis", "entity": "医源性甲亢" } ] }, { "text": "典型的症状与体征有以下表现:1.交感神经兴奋性增加,基础代谢率增加如消瘦、多汗、怕热、低热及食欲增加,但体重下降,大便次数增多,睡眠障碍和易于疲乏等。", "entities": [ { "start_idx": 16, "end_idx": 32, "type": "sym", "entity": "交感神经兴奋性增加,基础代谢率增加" }, { "start_idx": 34, "end_idx": 72, "type": "sym", "entity": "消瘦、多汗、怕热、低热及食欲增加,但体重下降,大便次数增多,睡眠障碍和易于疲乏" } ] }, { "text": "因交感神经系统过于兴奋,出现心率加快、脾气急躁,大龄儿童常感到心悸,严重病例可出现心律紊乱,心房颤动。", "entities": [ { "start_idx": 0, "end_idx": 49, "type": "sym", "entity": "因交感神经系统过于兴奋,出现心率加快、脾气急躁,大龄儿童常感到心悸,严重病例可出现心律紊乱,心房颤动" } ] }, { "text": "两手常有细微而迅速的震颤。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "两手常有细微而迅速的震颤" } ] }, { "text": "2.所有患儿都有甲状腺肿大肿大程度不一,一般为左右对称,质地柔软,表面光滑,边界清楚,可随吞咽动作上、下移动。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "甲状腺" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "甲状腺肿大" }, { "start_idx": 13, "end_idx": 53, "type": "sym", "entity": "肿大程度不一,一般为左右对称,质地柔软,表面光滑,边界清楚,可随吞咽动作上、下移动" } ] }, { "text": "在肿大的甲状腺上有时可听到收缩期杂音或者扪及震颤。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "甲状腺" }, { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "在肿大的甲状腺上有时可听到收缩期杂音或者扪及震颤" } ] }, { "text": "结节性肿大者可扪及大小不一、质硬、单个或多个结节。", "entities": [ { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "结节性肿大者可扪及大小不一、质硬、单个或多个结节" } ] }, { "text": "有时患者表现有颈部不适,压迫感,吞咽困难。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "颈部" }, { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "有时患者表现有颈部不适,压迫感,吞咽困难" } ] }, { "text": "3.眼部变化是甲亢特有表现,由于眼球突出常作凝视状,不常瞬目,上眼睑挛缩,眼向下看时上眼睑不能随眼球立即下落,上眼睑外翻困难。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "眼部变化" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "甲亢" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "眼球" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "上眼睑" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "上眼睑" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "眼球" }, { "start_idx": 55, "end_idx": 57, "type": "bod", "entity": "上眼睑" }, { "start_idx": 16, "end_idx": 61, "type": "sym", "entity": "眼球突出常作凝视状,不常瞬目,上眼睑挛缩,眼向下看时上眼睑不能随眼球立即下落,上眼睑外翻困难" } ] }, { "text": "4.其他可有青春期性发育缓慢,月经紊乱,闭经及月经过少等。", "entities": [ { "start_idx": 6, "end_idx": 26, "type": "sym", "entity": "青春期性发育缓慢,月经紊乱,闭经及月经过少" } ] }, { "text": "【实验室检查】主要测定血清FT3、FT4及超敏感TSH浓度。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "ite", "entity": "血清FT3" }, { "start_idx": 23, "end_idx": 25, "type": "ite", "entity": "FT4" }, { "start_idx": 33, "end_idx": 40, "type": "ite", "entity": "超敏感TSH浓度" } ] }, { "text": "患者FT4、FT3浓度都升高。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "ite", "entity": "FT4" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "FT3" } ] }, { "text": "甲亢疾病初期,临床症状轻微时,常先出现FT3升高,以后再出现FT4增高,并出现典型临床症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "甲亢" } ] }, { "text": "甲亢复发早期亦常见FT3先升高,后再出现FT4升高的情况。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "甲亢" } ] }, { "text": "甲亢治疗中症状尚未完全控制时,亦可只见FT3升高。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "甲亢" } ] }, { "text": "认识T3型甲亢,对甲亢早期诊断和甲亢的复发监测具有重要意义。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "甲亢" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "甲亢" } ] }, { "text": "甲亢时TSH降低,TSH水平受抑制而低于正常。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "甲亢" }, { "start_idx": 3, "end_idx": 21, "type": "sym", "entity": "TSH降低,TSH水平受抑制而低于正常" } ] }, { "text": "在多数新近被诊断为Graves病的患者中,可测出TSH受体刺激抗体(TRSAb),这种抗体的消失预告本病的缓解。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "Graves病" }, { "start_idx": 24, "end_idx": 32, "type": "bod", "entity": "TSH受体刺激抗体" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "TRSAb" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "抗体" } ] }, { "text": "测定抗甲状腺球蛋白抗体(TGAb)及抗甲状腺微粒体抗体(TMAb)以便明确是否为桥本病引致甲亢。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "bod", "entity": "抗甲状腺球蛋白抗体" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "TGAb" }, { "start_idx": 18, "end_idx": 26, "type": "bod", "entity": "抗甲状腺微粒体抗体" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "TMAb" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "桥本病" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "甲亢" } ] }, { "text": "甲状腺B超可以显示甲状腺大小,显示结节及囊肿等,必要时进行甲状腺同位素扫描。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "甲状腺B超" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "甲状腺" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "结节" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "囊肿" }, { "start_idx": 29, "end_idx": 36, "type": "pro", "entity": "甲状腺同位素扫描" } ] }, { "text": "【诊断及鉴别诊断】甲亢典型者根据临床症状、实验室检查发现总T3和FT3增高而TSH水平低下可确立诊断,TRSAb的存在可确定弥漫性毒性甲状腺肿的原因。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "甲亢" }, { "start_idx": 63, "end_idx": 67, "type": "bod", "entity": "TRSAb" }, { "start_idx": 74, "end_idx": 82, "type": "dis", "entity": "弥漫性毒性甲状腺肿" } ] }, { "text": "淋巴细胞性甲状腺炎(桥本病)在病程早期可呈现甲亢症状,但多数是一过性的,经随访可区别,检测TGAb和TPOAb有助于与弥漫性毒性甲状腺肿鉴别,但无法区别两者同时并存的患儿。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "淋巴细胞性甲状腺炎" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "桥本病" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "甲亢" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "TGAb" }, { "start_idx": 50, "end_idx": 54, "type": "bod", "entity": "TPOAb" }, { "start_idx": 59, "end_idx": 67, "type": "dis", "entity": "弥漫性毒性甲状腺肿" } ] }, { "text": "当甲状腺可触及结节或血清T3值极度增高时,应进行甲状腺B超和(或)同位素扫描检查,以正确诊断结节性甲状腺肿和鉴别癌肿;对甲状腺轻度肿大和甲亢症状轻微的患儿应考虑亚急性甲状腺炎(病毒感染所致)的可能性,必要时可以考虑同位素扫描检查和细针穿刺细胞学检查。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "sym", "entity": "甲状腺可触及结节" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "甲状腺B超" }, { "start_idx": 39, "end_idx": 45, "type": "pro", "entity": "同位素扫描检查" }, { "start_idx": 52, "end_idx": 58, "type": "dis", "entity": "结节性甲状腺肿" }, { "start_idx": 62, "end_idx": 63, "type": "dis", "entity": "癌肿" }, { "start_idx": 66, "end_idx": 72, "type": "sym", "entity": "甲状腺轻度肿大" }, { "start_idx": 74, "end_idx": 79, "type": "sym", "entity": "甲亢症状轻微" }, { "start_idx": 86, "end_idx": 92, "type": "dis", "entity": "亚急性甲状腺炎" }, { "start_idx": 94, "end_idx": 97, "type": "sym", "entity": "病毒感染" }, { "start_idx": 113, "end_idx": 119, "type": "pro", "entity": "同位素扫描检查" }, { "start_idx": 121, "end_idx": 129, "type": "pro", "entity": "细针穿刺细胞学检查" } ] }, { "text": "极少数是由于TSH受体基因激活性突变引起。", "entities": [ { "start_idx": 6, "end_idx": 17, "type": "sym", "entity": "TSH受体基因激活性突变" } ] }, { "text": "多数新生儿甲亢在出生时即有症状,表现为突眼、甲状腺肿大、烦躁、多动、心动过速、呼吸急促,严重可出现心力衰竭,血T3、T4升高,TSH下降。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "新生儿甲亢" } ] }, { "text": "这些症状经6~12周后,随体内甲状腺刺激免疫球蛋白水平下降而缓解。", "entities": [ { "start_idx": 12, "end_idx": 31, "type": "sym", "entity": "随体内甲状腺刺激免疫球蛋白水平下降而缓解" } ] }, { "text": "单纯性甲状腺肿多发生在青春期,心率正常,大便次数正常,血FT3、FT4正常。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "单纯性甲状腺肿" } ] }, { "text": "【治疗】小儿甲亢的治疗不同于成人,在口服药、手术切除及同位素碘治疗三者中,首选为口服药,一般需口服治疗2~3年;桥本病导致者可缩短些。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "小儿甲亢" }, { "start_idx": 18, "end_idx": 20, "type": "pro", "entity": "口服药" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "手术切除" }, { "start_idx": 27, "end_idx": 32, "type": "pro", "entity": "同位素碘治疗" }, { "start_idx": 40, "end_idx": 42, "type": "pro", "entity": "口服药" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "口服治疗" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "桥本病" } ] }, { "text": "疗法的选择应根据患儿年龄、病程、甲亢类型、甲状腺大小、药物反应、有无桥本病以及家长能否坚持治疗等。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "甲亢" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "甲状腺" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "桥本病" } ] }, { "text": "仅在药物治疗无效时才考虑手术或用同位素碘疗法。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "手术" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "同位素碘疗法" } ] }, { "text": "2.甲巯咪唑(又称他巴唑)本药能阻抑碘与酪氨酸结合,抑制甲状腺激素的合成,口服后奏效快而作用时间较长(半衰期为6~8小时),可按每日0.3~0.5mg/kg,分2次口服。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "甲巯咪唑" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "他巴唑" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "酪氨酸" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "甲状腺激素" } ] }, { "text": "用药1~3个月后病情基本得到控制,心率降到80~90次/分,血T3、T4亦降到正常时可减量1/3~1/2,如仍稳定,逐步减至维持量,一般用药2~3年为宜。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "心率" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "血T3" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "T4" } ] }, { "text": "少数小儿用药后可能发生暂时性白细胞减少症或皮疹,停药即消失,严重者可发生粒细胞减少、肝损害、肾小球肾炎及脉管炎等,虽属罕见,在使用中仍须仔细观察。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "暂时性白细胞减少症" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "皮疹" }, { "start_idx": 30, "end_idx": 54, "type": "sym", "entity": "严重者可发生粒细胞减少、肝损害、肾小球肾炎及脉管炎" } ] }, { "text": "粒细胞缺乏症多发生在服药开始几周或几个月,常伴有发热,故在治疗最初期间,应经常复查血常规,一旦白细胞低于4×109/L,应减少或停服抗甲状腺药物,并给予升白细胞药物(如鲨肝醇、利血生及MG-CSF等)治疗。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "粒细胞缺乏症" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "发热" }, { "start_idx": 41, "end_idx": 43, "type": "pro", "entity": "血常规" }, { "start_idx": 47, "end_idx": 49, "type": "ite", "entity": "白细胞" }, { "start_idx": 72, "end_idx": 77, "type": "dru", "entity": "抗甲状腺药物" }, { "start_idx": 82, "end_idx": 87, "type": "dru", "entity": "升白细胞药物" }, { "start_idx": 90, "end_idx": 92, "type": "dru", "entity": "鲨肝醇" }, { "start_idx": 94, "end_idx": 96, "type": "dru", "entity": "利血生" }, { "start_idx": 98, "end_idx": 103, "type": "dru", "entity": "MG-CSF" } ] }, { "text": "皮疹一般经抗过敏药治疗可好转,严重的皮疹可试用糖皮质激素。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "皮疹" }, { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "抗过敏药" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "皮疹" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "糖皮质激素" } ] }, { "text": "3.丙基硫氧嘧啶(PTU)除抑制甲状腺激素的合成外,同时还减少在外周组织的T4转化成T3,毒性与甲巯咪唑类相同,初始剂量为每日4~6mg/kg,因其半衰期较甲巯咪唑短,故需分3次服用。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "丙基硫氧嘧啶" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "PTU" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "甲状腺激素" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "外周组织" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "T4" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "T3" }, { "start_idx": 60, "end_idx": 64, "type": "dru", "entity": "甲巯咪唑类" }, { "start_idx": 90, "end_idx": 93, "type": "dru", "entity": "甲巯咪唑" } ] }, { "text": "PTU被吸收后大多在血循环中与蛋白质结合,极少通过胎盘,不致损伤胎儿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "PTU" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "血循环" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "蛋白质" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "胎盘" } ] }, { "text": "13岁以上的病人、男孩以及甲状腺肿较小和甲状腺激素水平轻度升高者,症状可能较早缓解。", "entities": [ { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "甲状腺肿较小" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "甲状腺激素水平轻度升高" } ] }, { "text": "5.治疗过程中若出现甲低、甲状腺肿大或者突眼更明显者,应加服甲状腺素,并酌情减少甲巯咪唑用量。", "entities": [ { "start_idx": 10, "end_idx": 24, "type": "sym", "entity": "甲低、甲状腺肿大或者突眼更明显" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "甲状腺素" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "甲巯咪唑" } ] }, { "text": "6.对有药物过敏、粒细胞减少、甲状腺肿瘤、甲状腺明显肿大且服药后缩小不明显、服药后复发不愈者等,则有甲状腺手术切除治疗适应证。", "entities": [ { "start_idx": 2, "end_idx": 44, "type": "sym", "entity": "对有药物过敏、粒细胞减少、甲状腺肿瘤、甲状腺明显肿大且服药后缩小不明显、服药后复发不愈" }, { "start_idx": 50, "end_idx": 56, "type": "pro", "entity": "甲状腺手术切除" } ] }, { "text": "术前应用抗甲状腺药物2~3个月使甲状腺功能正常。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "抗甲状腺药物" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "甲状腺" } ] }, { "text": "术前服复方碘溶液1~2周防止术中出血。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "复方碘溶液" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "术中出血" } ] }, { "text": "自术前4日至术后7日,口服普萘洛尔1~2mg/kg,每6小时1次。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "普萘洛尔" } ] }, { "text": "手术后甲低发生率为50%,少数出现暂时性或永久性甲状旁腺功能减低。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "甲低" }, { "start_idx": 13, "end_idx": 31, "type": "sym", "entity": "少数出现暂时性或永久性甲状旁腺功能减低" } ] }, { "text": "7.近来不少学者推荐甲亢用同位素碘治疗,认为简单、有效、经济且无致癌危险。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "甲亢" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "同位素碘治疗" } ] }, { "text": "治疗后甲状腺可缩小35%~54%,但远期甲低发生率可高达92%。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "甲状腺" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "甲低" } ] }, { "text": "8.新生儿甲亢轻者不必用药,症状明显的可用丙基硫氧嘧啶,重症加服普萘洛尔及对症治疗,必要时输液、加用抗生素及皮质激素等。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "新生儿甲亢" }, { "start_idx": 21, "end_idx": 26, "type": "dru", "entity": "丙基硫氧嘧啶" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "普萘洛尔" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "输液" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "抗生素" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "皮质激素" } ] }, { "text": "二、髓母细胞瘤髓母细胞瘤(medulloblastoma)本病为常见的中枢神经系统肿瘤,属于恶性程度很高的肿瘤,起源于小脑蚓部或第四脑室顶的后髓帆原始胚胎残留组织。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "髓母细胞瘤" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "髓母细胞瘤" }, { "start_idx": 13, "end_idx": 27, "type": "dis", "entity": "medulloblastoma" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "中枢神经系统肿瘤" }, { "start_idx": 59, "end_idx": 62, "type": "bod", "entity": "小脑蚓部" }, { "start_idx": 64, "end_idx": 68, "type": "bod", "entity": "第四脑室顶" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "后髓帆" } ] }, { "text": "占儿童原发性脑瘤的10%~20%,后颅窝肿瘤的40%。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "儿童原发性脑瘤" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "后颅窝肿瘤" } ] }, { "text": "肿瘤细胞可随脑脊液播散至蛛网膜下腔或转移至颅外,可达11%~43%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "脑脊液" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "颅外" } ] }, { "text": "瘤组织呈灰红或紫红色,质软、易碎,边界不清。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "瘤组织" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "瘤组织呈灰红或紫红色" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "质软、易碎,边界不清" } ] }, { "text": "镜下肿瘤细胞密集,核呈圆形或椭圆形,深染,多有核分裂,胞质及间质较少,有的呈假菊形团排列。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "镜下肿瘤细胞密集" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "核呈圆形或椭圆形" }, { "start_idx": 18, "end_idx": 43, "type": "sym", "entity": "深染,多有核分裂,胞质及间质较少,有的呈假菊形团排列" } ] }, { "text": "主要表现为颅内压增高和共济失调等小脑症状,侵及脑干者常有复视及多种脑神经障碍,小脑扁桃体疝时常有颈强直、斜颈表现。", "entities": [ { "start_idx": 5, "end_idx": 19, "type": "sym", "entity": "颅内压增高和共济失调等小脑症状" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "脑干" }, { "start_idx": 28, "end_idx": 37, "type": "dis", "entity": "复视及多种脑神经障碍" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "小脑扁桃体疝" }, { "start_idx": 48, "end_idx": 55, "type": "sym", "entity": "颈强直、斜颈表现" } ] }, { "text": "辅助检查CT及MRI可清楚地显示肿瘤。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "检查CT" }, { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "MRI" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "肿瘤" } ] }, { "text": "CT见肿瘤为高密度或等密度影,类圆形,边界清楚,周围水肿常为低密度。MRI瘤体呈长T1与长T2信号,注射Gd-DTPA(顺磁剂)后明显均一强化,少数可见厚薄不均的环状强化。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "CT" }, { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肿瘤" }, { "start_idx": 0, "end_idx": 32, "type": "sym", "entity": "CT见肿瘤为高密度或等密度影,类圆形,边界清楚,周围水肿常为低密度" }, { "start_idx": 34, "end_idx": 36, "type": "pro", "entity": "MRI" }, { "start_idx": 34, "end_idx": 84, "type": "sym", "entity": "MRI瘤体呈长T1与长T2信号,注射Gd-DTPA(顺磁剂)后明显均一强化,少数可见厚薄不均的环状强化" } ] }, { "text": "术后放疗是十分重要的措施,除局部照射外,应包括全脑和椎管照射,以防止播散肿瘤发展。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "放疗" } ] }, { "text": "并加用化学治疗。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "化学治疗" } ] }, { "text": "早年髓母细胞瘤预后极差,近年来,随着放疗及化疗的改进,生存期已大大延长,有报道5年生存期可达85%。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "髓母细胞瘤" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "放疗" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "化疗" } ] }, { "text": "二、实验室检查主要包括病毒标志物和特异性抗体的检查。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "实验室检查" }, { "start_idx": 11, "end_idx": 24, "type": "pro", "entity": "病毒标志物和特异性抗体的检查" } ] }, { "text": "(一)病毒学检查(即病毒标志物检查)1.病毒颗粒检查用电镜或免疫电镜直接检测样本中病毒颗粒。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "病毒学检查" }, { "start_idx": 10, "end_idx": 16, "type": "pro", "entity": "病毒标志物检查" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "病毒颗粒检查" }, { "start_idx": 27, "end_idx": 28, "type": "equ", "entity": "电镜" }, { "start_idx": 30, "end_idx": 33, "type": "equ", "entity": "免疫电镜" }, { "start_idx": 41, "end_idx": 44, "type": "mic", "entity": "病毒颗粒" } ] }, { "text": "2.病毒分离取样本接种于适宜细胞,培养分离病毒,常根据细胞病变或病毒标志物检查或利用其生物学特性如红细胞吸附、空斑形成等予以鉴定。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "病毒分离" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "细胞" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "病毒" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "细胞" }, { "start_idx": 32, "end_idx": 38, "type": "pro", "entity": "病毒标志物检查" }, { "start_idx": 49, "end_idx": 58, "type": "sym", "entity": "红细胞吸附、空斑形成" } ] }, { "text": "此法能客观反映所感染病毒的存在及其种类,故被称为“黄金标准”。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "病毒" } ] }, { "text": "标准的病毒分离和电镜检查可用于检测未知病毒或发现新的病毒株。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "病毒分离" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "电镜检查" }, { "start_idx": 19, "end_idx": 20, "type": "mic", "entity": "病毒" }, { "start_idx": 26, "end_idx": 28, "type": "mic", "entity": "病毒株" } ] }, { "text": "但此法费时、耗材、耗力,标本所含病毒数量极少时会出现假阴性。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "病毒" } ] }, { "text": "病毒复制不全、产生前病毒或与宿主细胞DNA整合时不能分离成功。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "病毒" }, { "start_idx": 14, "end_idx": 20, "type": "bod", "entity": "宿主细胞DNA" } ] }, { "text": "有些病毒如乙型肝炎病毒(HBV)、丙型肝炎病毒(HCV)、庚型肝炎病毒(HGV)等目前尚缺乏敏感的培养细胞。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "病毒" }, { "start_idx": 5, "end_idx": 10, "type": "mic", "entity": "乙型肝炎病毒" }, { "start_idx": 12, "end_idx": 14, "type": "mic", "entity": "HBV" }, { "start_idx": 17, "end_idx": 22, "type": "mic", "entity": "丙型肝炎病毒" }, { "start_idx": 24, "end_idx": 26, "type": "mic", "entity": "HCV" }, { "start_idx": 29, "end_idx": 34, "type": "mic", "entity": "庚型肝炎病毒" }, { "start_idx": 36, "end_idx": 38, "type": "mic", "entity": "HGV" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "细胞" } ] }, { "text": "3.病毒抗原检测用免疫标记技术直接检测体液和组织中病毒抗原是快速而实用的方法。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "病毒抗原检测" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "免疫标记技术" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "体液" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "组织" }, { "start_idx": 25, "end_idx": 28, "type": "mic", "entity": "病毒抗原" } ] }, { "text": "这一方法要求标本含有一定量的抗原和高质量的特异性抗体。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "特异性抗体" } ] }, { "text": "4.病毒基因检测应用核酸杂交法或聚合酶链反应(PCR),通过检测病毒特异性核酸(DNA或RNA)片段来发现相关病毒。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "病毒基因检测" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "核酸杂交法" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "聚合酶链反应" }, { "start_idx": 23, "end_idx": 25, "type": "pro", "entity": "PCR" }, { "start_idx": 32, "end_idx": 38, "type": "mic", "entity": "病毒特异性核酸" }, { "start_idx": 55, "end_idx": 56, "type": "mic", "entity": "病毒" } ] }, { "text": "此法灵敏度高,如PCR法能测出fg(10~15)水平的核酸,较杂交法测出pg(10~12)水平更为敏感。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "PCR法" }, { "start_idx": 31, "end_idx": 33, "type": "pro", "entity": "杂交法" } ] }, { "text": "此法能发现不完整病毒如潜伏病毒DNA,故对疱疹类病毒如人巨细胞病毒(HCMV),单独检查病毒DNA不能区别活动性感染和潜伏感染。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "mic", "entity": "病毒" }, { "start_idx": 13, "end_idx": 17, "type": "mic", "entity": "病毒DNA" }, { "start_idx": 21, "end_idx": 25, "type": "mic", "entity": "疱疹类病毒" }, { "start_idx": 27, "end_idx": 32, "type": "mic", "entity": "人巨细胞病毒" }, { "start_idx": 34, "end_idx": 37, "type": "mic", "entity": "HCMV" }, { "start_idx": 44, "end_idx": 48, "type": "mic", "entity": "病毒DNA" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "活动性感染" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "潜伏感染" } ] }, { "text": "此外,新生儿娩出时,脐血中可混入母血,比血细胞更小的病毒核酸更易污染脐血。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "脐血" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "母血" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "血细胞" }, { "start_idx": 26, "end_idx": 27, "type": "mic", "entity": "病毒" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "脐血" } ] }, { "text": "因此,用敏感的PCR法从脐血中检测病毒基因尚难确定系新生儿自身的感染。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "PCR法" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "脐血" }, { "start_idx": 17, "end_idx": 18, "type": "mic", "entity": "病毒" } ] }, { "text": "5.其他标志物检查如感染细胞内病毒颗粒聚集形成的包涵体(如疱疹类病毒)、内基小体(狂犬病毒)等特殊结构,常为活动性病毒感染的诊断依据。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "其他标志物检查" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "感染细胞" }, { "start_idx": 15, "end_idx": 18, "type": "mic", "entity": "病毒颗粒" }, { "start_idx": 24, "end_idx": 26, "type": "mic", "entity": "包涵体" }, { "start_idx": 29, "end_idx": 33, "type": "mic", "entity": "疱疹类病毒" }, { "start_idx": 36, "end_idx": 39, "type": "mic", "entity": "内基小体" }, { "start_idx": 41, "end_idx": 44, "type": "mic", "entity": "狂犬病毒" }, { "start_idx": 54, "end_idx": 60, "type": "dis", "entity": "活动性病毒感染" } ] }, { "text": "(二)血清学检查狭义的血清学检查是指特异性抗体测定。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "血清学检查" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "血清学检查" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "特异性抗体测定" } ] }, { "text": "1.特异性IgG检测特异性IgG抗体多出现较晚,一般持续时间较长,乃至终生,单项阳性提示既往或正在感染该病毒。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "特异性IgG检测" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "特异性IgG抗体" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "单项阳性" }, { "start_idx": 52, "end_idx": 53, "type": "mic", "entity": "病毒" } ] }, { "text": "取急性期和恢复期双份血清检测特异性IgG,若抗体滴度≥4倍增高可回顾性诊断近期相应病毒感染。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "血清检测" }, { "start_idx": 14, "end_idx": 19, "type": "ite", "entity": "特异性IgG" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "抗体滴度" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "病毒感染" } ] }, { "text": "但也有例外,如原发EBV感染时,抗VCAIgG峰值在急性期,故不能使用上述诊断标准。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "原发EBV感染" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "抗VCAIgG" } ] }, { "text": "IgG能通过胎盘,在评估6个月以下婴儿的IgG抗体时应考虑被动抗体的存在。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "IgG" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "胎盘" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "IgG抗体" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "抗体" } ] }, { "text": "在严重免疫缺陷患者,IgG抗体水平低下或缺如,可出现假阴性结果。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "严重免疫缺陷" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "IgG抗体" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "假阴性" } ] }, { "text": "2.特异性IgM检测机体在病毒初次感染早期或潜伏病毒活化时产生特异性IgM抗体(后者IgM抗体水平常较前者低),一般持续数月后消失,常作为急性(或近期)感染或活动性感染的诊断指标。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "特异性IgM检测" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "病毒初次感染早期" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "病毒" }, { "start_idx": 31, "end_idx": 38, "type": "ite", "entity": "特异性IgM抗体" }, { "start_idx": 42, "end_idx": 46, "type": "ite", "entity": "IgM抗体" }, { "start_idx": 69, "end_idx": 77, "type": "sym", "entity": "急性(或近期)感染" }, { "start_idx": 79, "end_idx": 83, "type": "sym", "entity": "活动性感染" } ] }, { "text": "受高滴度IgG抗体或类风湿因子等因素干扰可出现假阳性,采用IgM抗体捕捉ELISA或先去除IgG等方法可避免之。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "bod", "entity": "高滴度IgG抗体" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "类风湿因子" }, { "start_idx": 23, "end_idx": 25, "type": "sym", "entity": "假阳性" }, { "start_idx": 29, "end_idx": 40, "type": "pro", "entity": "IgM抗体捕捉ELISA" }, { "start_idx": 42, "end_idx": 47, "type": "pro", "entity": "先去除IgG" } ] }, { "text": "免疫抑制者和幼小婴儿因免疫反应低下易出现假阴性。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "免疫抑制" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "免疫反应低下" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "假阴性" } ] }, { "text": "3.特异性IgA检测某些病毒感染后,特异性IgA检出率较高,有助于诊断,如原发性带状疱疹病毒(VZV)感染和急性呼吸道合胞病毒(RSV)感染时。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "特异性IgA检测" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "病毒感染" }, { "start_idx": 18, "end_idx": 23, "type": "ite", "entity": "特异性IgA" }, { "start_idx": 37, "end_idx": 52, "type": "dis", "entity": "原发性带状疱疹病毒(VZV)感染" }, { "start_idx": 54, "end_idx": 69, "type": "dis", "entity": "急性呼吸道合胞病毒(RSV)感染" } ] }, { "text": "EB病毒(EBV)相关性鼻咽癌时,抗早期抗原IgA和抗病毒衣壳抗原IgA水平增高可用于早期诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "EB病毒" }, { "start_idx": 5, "end_idx": 7, "type": "mic", "entity": "EBV" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "鼻咽癌" }, { "start_idx": 17, "end_idx": 24, "type": "ite", "entity": "抗早期抗原IgA" }, { "start_idx": 26, "end_idx": 37, "type": "ite", "entity": "抗病毒衣壳抗原IgA水平" } ] }, { "text": "第七节特发性高钙尿症特发性高钙尿症(idiopathichypercalciuria,IH)是指病因不明尿钙排出增多而血钙正常而血钙正常的一组疾病。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "特发性高钙尿症" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "特发性高钙尿症" }, { "start_idx": 18, "end_idx": 41, "type": "dis", "entity": "idiopathichypercalciuria" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "IH" }, { "start_idx": 52, "end_idx": 53, "type": "ite", "entity": "尿钙" }, { "start_idx": 59, "end_idx": 60, "type": "ite", "entity": "血钙" }, { "start_idx": 52, "end_idx": 62, "type": "sym", "entity": "尿钙排出增多而血钙正常" }, { "start_idx": 64, "end_idx": 65, "type": "ite", "entity": "血钙" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "血钙正常" } ] }, { "text": "【病因与发病机制】肾脏和钙代谢关系非常密切,从肾小球滤过的钙50%~70%在近端肾小管被重吸收,30%~40%在远端肾小管重吸收,尿中排出的钙,离子钙占20%,复合钙占80%,以枸橼酸钙为主。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肾脏" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "钙" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "肾小球" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "钙" }, { "start_idx": 38, "end_idx": 42, "type": "bod", "entity": "近端肾小管" }, { "start_idx": 56, "end_idx": 60, "type": "bod", "entity": "远端肾小管" }, { "start_idx": 65, "end_idx": 65, "type": "bod", "entity": "尿" }, { "start_idx": 70, "end_idx": 70, "type": "bod", "entity": "钙" }, { "start_idx": 72, "end_idx": 74, "type": "bod", "entity": "离子钙" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "复合钙" }, { "start_idx": 89, "end_idx": 92, "type": "bod", "entity": "枸橼酸钙" } ] }, { "text": "钙的重吸收和钠相似,是通过主动转运完成的,且受PTH和活性维生素D的调节。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "钙" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "钠" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "PTH" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "活性维生素D" } ] }, { "text": "特发性高钙尿症病因不明,此病有明显的家族史,可能系常染色体显性遗传,但也有认为家族中同一饮食或环境因素引起。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "特发性高钙尿症" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "常染色体显性遗传" } ] }, { "text": "发病机制可能与以下机制有关:(一)肠钙吸收亢进(吸收型)主要由于空肠对钙选择性吸收过多,使血钙短暂升高致肾小球滤过钙增多及甲状旁腺分泌抑制而使肾小管重吸收钙减少。", "entities": [ { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "肠钙吸收亢进" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "空肠" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "钙" }, { "start_idx": 32, "end_idx": 42, "type": "sym", "entity": "空肠对钙选择性吸收过多" }, { "start_idx": 45, "end_idx": 46, "type": "ite", "entity": "血钙" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "血钙短暂升高" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "肾小球" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "钙" }, { "start_idx": 52, "end_idx": 59, "type": "sym", "entity": "肾小球滤过钙增多" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "甲状旁腺" }, { "start_idx": 61, "end_idx": 68, "type": "sym", "entity": "甲状旁腺分泌抑制" }, { "start_idx": 71, "end_idx": 73, "type": "bod", "entity": "肾小管" }, { "start_idx": 77, "end_idx": 77, "type": "bod", "entity": "钙" }, { "start_idx": 71, "end_idx": 79, "type": "sym", "entity": "肾小管重吸收钙减少" } ] }, { "text": "肠吸收钙亢进原因尚不明,可能系维生素D合成增多及调节功能障碍所致。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "肠吸收钙亢进" }, { "start_idx": 15, "end_idx": 29, "type": "sym", "entity": "维生素D合成增多及调节功能障碍" } ] }, { "text": "也有人认为与原发性肾失磷,致1,25(OH2D3合成增加,肠吸收钙增多所致。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "原发性肾失磷" }, { "start_idx": 14, "end_idx": 40, "type": "bod", "entity": "1,25(OH2D3" }, { "start_idx": 14, "end_idx": 44, "type": "sym", "entity": "1,25(OH2D3合成增加" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "肠吸收钙增多" } ] }, { "text": "(二)肾小管重吸收钙障碍(肾漏型)由于肾小管重吸收钙缺陷致尿钙漏出增多,刺激甲状旁腺的分泌及1,25(OH)2D3合成增多,引起继发性肠钙吸收亢进并维持血钙正常。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肾小管" }, { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "肾小管重吸收钙障碍" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "肾小管" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "钙" }, { "start_idx": 19, "end_idx": 27, "type": "sym", "entity": "肾小管重吸收钙缺陷" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "尿钙漏出增多" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "甲状旁腺" }, { "start_idx": 46, "end_idx": 68, "type": "bod", "entity": "1,25(OH)2D3" }, { "start_idx": 46, "end_idx": 72, "type": "sym", "entity": "1,25(OH)2D3合成增多" }, { "start_idx": 88, "end_idx": 89, "type": "ite", "entity": "血钙" }, { "start_idx": 76, "end_idx": 91, "type": "sym", "entity": "继发性肠钙吸收亢进并维持血钙正常" } ] }, { "text": "一般认为高钙尿达10天以上会出现血尿,高钙尿可能损伤肾脏而出现血尿,但也并非高钙尿都有血尿。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "高钙尿" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "血尿" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "高钙尿" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肾脏" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "损伤肾脏" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "血尿" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "高钙尿" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "血尿" } ] }, { "text": "【临床表现】(一)血尿主要为镜下血尿,肉眼血尿一般为反复发作性,有时可见血丝。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "血尿" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "镜下血尿" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "反复发作性" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "血丝" } ] }, { "text": "尿中红细胞形态为非肾小球性。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "尿" }, { "start_idx": 2, "end_idx": 6, "type": "ite", "entity": "红细胞形态" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "尿中红细胞形态为非肾小球性" } ] }, { "text": "(二)泌尿系症状少数病例有尿频、尿急、尿痛、排尿困难、遗尿及肾绞痛等症状。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "尿频" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "尿急" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "尿痛" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "排尿困难" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "遗尿" }, { "start_idx": 30, "end_idx": 32, "type": "sym", "entity": "肾绞痛" } ] }, { "text": "(三)尿路结石小儿肾结石中仅2%~5%系由本病引起。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "尿路结石" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "小儿肾结石" } ] }, { "text": "(四)少数病人身体矮小,体重不增,肌无力,骨质稀疏等。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "身体矮小" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "体重不增" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "肌无力" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "骨质稀疏" } ] }, { "text": "【实验室检查】血钙正常,血磷有时可降低;粪钙、磷减少;血碱性磷酸酶增高。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "血钙" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "血钙正常" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "血磷" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "血磷有时可降低" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "粪钙" }, { "start_idx": 23, "end_idx": 23, "type": "ite", "entity": "磷" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "粪钙、磷减少" }, { "start_idx": 27, "end_idx": 32, "type": "ite", "entity": "血碱性磷酸酶" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "血碱性磷酸酶增高" } ] }, { "text": "尿钙增多(尿钙/尿肌酐>0.18;24小时尿钙定量>0.1mmol/kg);X线及B型超声等影像学检查应常规进行。", "entities": [ { "start_idx": 38, "end_idx": 44, "type": "pro", "entity": "X线及B型超声" } ] }, { "text": "不能完全确诊,长期伴有血尿者可考虑做肾活体组织检查。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "血尿" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "肾活体组织检查" } ] }, { "text": "【诊断】(一)随意尿Ca/Cr比值一般采用早餐后2小时随意尿标本测定,当尿Ca/Cr比值>0.21者,提示有高钙尿可能。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "ite", "entity": "尿Ca/Cr比值" }, { "start_idx": 36, "end_idx": 43, "type": "ite", "entity": "尿Ca/Cr比值" }, { "start_idx": 36, "end_idx": 48, "type": "sym", "entity": "尿Ca/Cr比值>0.21" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "高钙尿" } ] }, { "text": "因儿童收集24小时尿较为困难,经实验证明,可用随意尿Ca/Cr比值来做筛查。", "entities": [ { "start_idx": 25, "end_idx": 32, "type": "ite", "entity": "尿Ca/Cr比值" } ] }, { "text": "(二)24小时尿钙测定定量当尿Ca>4mg/(kg•d),可诊断高钙尿症,但尿钙排出量是受多种因素影响,如饮食中钙、钠、磷及蛋白质的含量,维生素D的摄入量等,一般上述检查应重复2次以上,排除各种已知病因引起的高钙尿后,可诊断为特发性高钙尿。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "ite", "entity": "24小时尿钙测定定量" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "尿Ca" }, { "start_idx": 14, "end_idx": 27, "type": "sym", "entity": "尿Ca>4mg/(kg•d)" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "高钙尿症" }, { "start_idx": 38, "end_idx": 42, "type": "ite", "entity": "尿钙排出量" }, { "start_idx": 104, "end_idx": 106, "type": "dis", "entity": "高钙尿" }, { "start_idx": 113, "end_idx": 118, "type": "dis", "entity": "特发性高钙尿" } ] }, { "text": "(三)钙负荷试验可做钙负荷试验进一步分型:给患儿低钙低钠饮食1周(停服乳品及钙剂,钙<250~300mg/d);试验前晚餐后禁食,于晚9时及午夜各饮水5~10ml/kg,试验日清晨7~9时留尿测空腹尿Ca/Cr比值;正常早餐后,口服葡萄糖酸钙糖浆(含钙1g/1.73m2或元素钙15~20mg/kg,),收集上午9时至下午1时共4小时尿,再测尿Ca/Cr比值。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "钙负荷试验" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "钙负荷试验" }, { "start_idx": 97, "end_idx": 106, "type": "ite", "entity": "空腹尿Ca/Cr比值" }, { "start_idx": 116, "end_idx": 122, "type": "dru", "entity": "葡萄糖酸钙糖浆" }, { "start_idx": 182, "end_idx": 189, "type": "ite", "entity": "尿Ca/Cr比值" } ] }, { "text": "如为吸收型IH,则空腹尿Ca/Cr比值正常,钙负荷后增高(>0.28),肾性IH则不受限钙影响,空腹尿Ca/Cr>0.21。", "entities": [ { "start_idx": 9, "end_idx": 18, "type": "ite", "entity": "空腹尿Ca/Cr比值" }, { "start_idx": 9, "end_idx": 20, "type": "sym", "entity": "空腹尿Ca/Cr比值正常" }, { "start_idx": 48, "end_idx": 55, "type": "ite", "entity": "空腹尿Ca/Cr" }, { "start_idx": 48, "end_idx": 60, "type": "sym", "entity": "空腹尿Ca/Cr>0.21" } ] }, { "text": "【鉴别诊断】本病主要要与其他原因所致肾结石,如原发性甲状旁腺功能亢进症、肾小管酸中毒、维生素D中毒以及手术后制动等相鉴别;尚应注意排出其他病因所致的高钙尿症,如髓质海绵肾、结节病、肝豆状核变性、糖尿病以及长期皮质醇治疗,慢性镉/铅中毒、Wilson病及幼年类风湿关节炎等;可根据各原发病特点进行鉴别。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "肾结石" }, { "start_idx": 23, "end_idx": 34, "type": "dis", "entity": "原发性甲状旁腺功能亢进症" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "肾小管酸中毒" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "维生素D中毒" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "高钙尿症" }, { "start_idx": 80, "end_idx": 84, "type": "dis", "entity": "髓质海绵肾" }, { "start_idx": 86, "end_idx": 88, "type": "dis", "entity": "结节病" }, { "start_idx": 90, "end_idx": 95, "type": "dis", "entity": "肝豆状核变性" }, { "start_idx": 97, "end_idx": 99, "type": "dis", "entity": "糖尿病" }, { "start_idx": 102, "end_idx": 108, "type": "pro", "entity": "长期皮质醇治疗" }, { "start_idx": 110, "end_idx": 116, "type": "dis", "entity": "慢性镉/铅中毒" }, { "start_idx": 118, "end_idx": 124, "type": "dis", "entity": "Wilson病" }, { "start_idx": 126, "end_idx": 133, "type": "dis", "entity": "幼年类风湿关节炎" } ] }, { "text": "(二)噻嗪类利尿剂对肾漏型者噻嗪类利尿剂治疗有效,可促进远端肾小管重吸收钙,使尿钙恢复正常,并调节甲状旁腺及1,25(OH)2D3至正常水平,使肠钙吸收正常。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "噻嗪类利尿剂" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾漏型" }, { "start_idx": 14, "end_idx": 19, "type": "dru", "entity": "噻嗪类利尿剂" }, { "start_idx": 28, "end_idx": 32, "type": "bod", "entity": "远端肾小管" }, { "start_idx": 39, "end_idx": 40, "type": "ite", "entity": "尿钙" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "尿钙恢复正常" }, { "start_idx": 49, "end_idx": 52, "type": "ite", "entity": "甲状旁腺" }, { "start_idx": 54, "end_idx": 81, "type": "ite", "entity": "1,25(OH)2D3" }, { "start_idx": 49, "end_idx": 86, "type": "sym", "entity": "甲状旁腺及1,25(OH)2D3至正常水平" }, { "start_idx": 89, "end_idx": 94, "type": "sym", "entity": "肠钙吸收正常" } ] }, { "text": "(三)磷酸纤维素钠为一种不被肠道吸收的离子交换树脂,能减少肠道钙的吸收,从而减少尿钙排出,对吸收型有效。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "磷酸纤维素钠" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "减少肠道钙的吸收" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "尿钙" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "减少尿钙排出" } ] }, { "text": "不良反应为能影响肠道镁的吸收,可致血镁降低,应注意补充镁。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "影响肠道镁的吸收" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "血镁降低" }, { "start_idx": 27, "end_idx": 27, "type": "dru", "entity": "镁" } ] }, { "text": "(四)口服锌或铁剂可减少钙的吸收而降低尿钙,适用于低锌血症或缺铁性贫血患儿。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "dru", "entity": "锌" }, { "start_idx": 7, "end_idx": 8, "type": "dru", "entity": "铁剂" } ] }, { "text": "(五)有人报告用未加工的麦麸服用治疗,能使80%患儿尿钙减少,其中近半数尿钙达正常水平。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dru", "entity": "麦麸" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "尿钙" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "尿钙减少" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "尿钙" } ] }, { "text": "其机制可能是影响肠钙吸收,故更适用于因肠吸收钙过多者。", "entities": [ { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "肠吸收钙过多" } ] }, { "text": "第七章气管、支气管疾病第一节先天性气道畸形一、气管软化气管软化(tracheomalacia)因气管软骨先天发育不良所致,可同时伴有喉软化(laryngomalacia),两者是先天性喉喘鸣的主要原因。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "气管、支气管疾病" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "先天性气道畸形" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "气管软化" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "气管软化" }, { "start_idx": 33, "end_idx": 46, "type": "dis", "entity": "tracheomalacia" }, { "start_idx": 49, "end_idx": 58, "type": "dis", "entity": "气管软骨先天发育不良" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "喉软化" }, { "start_idx": 71, "end_idx": 84, "type": "dis", "entity": "laryngomalacia" }, { "start_idx": 90, "end_idx": 95, "type": "dis", "entity": "先天性喉喘鸣" } ] }, { "text": "偶伴支气管软化(bronchomalacia)。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "支气管软化" }, { "start_idx": 8, "end_idx": 21, "type": "dis", "entity": "bronchomalacia" } ] }, { "text": "动物实验表明,多柔比星诱发的食管闭锁常伴气管软化,说明两者可能存在共同的致病因素。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "多柔比星" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "食管闭锁" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "气管软化" } ] }, { "text": "易并发呼吸道感染。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "呼吸道感染" } ] }, { "text": "25%患儿伴有反应性气道疾病。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "反应性气道疾病" } ] }, { "text": "婴儿持续喘鸣应考虑到本病可能。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "喘鸣" } ] }, { "text": "气管镜检查可做出诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "气管镜检查" } ] }, { "text": "在气管镜直视下可看到气管的前后壁随着呼吸运动而相互贴近,深呼吸时更为明显。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "equ", "entity": "气管镜" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "气管" } ] }, { "text": "当气管镜探入隆突的下方时,由于支气管受到支撑,可使呼吸困难有所缓解。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "equ", "entity": "气管镜" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "支气管" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "喂养时奶孔不宜过大,必要时胃管喂养。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "equ", "entity": "胃管" } ] }, { "text": "安静时喘鸣一般在5岁内消失。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "喘鸣" } ] }, { "text": "多发于冬春两季,呈散发性或流行性发病,后者称为流行性毛细支气管炎,又因该病是以喘憋为主要特征的一种特殊类型的肺炎,故又称喘憋性肺炎。", "entities": [ { "start_idx": 23, "end_idx": 31, "type": "dis", "entity": "流行性毛细支气管炎" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "喘憋" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "肺炎" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "喘憋性肺炎" } ] }, { "text": "本病主要由病毒引起,呼吸道合胞病毒占50%以上,其次为副流感病毒3型、肺炎支原体、腺病毒等。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "病毒" }, { "start_idx": 10, "end_idx": 16, "type": "mic", "entity": "呼吸道合胞病毒" }, { "start_idx": 27, "end_idx": 33, "type": "mic", "entity": "副流感病毒3型" }, { "start_idx": 35, "end_idx": 39, "type": "mic", "entity": "肺炎支原体" }, { "start_idx": 41, "end_idx": 43, "type": "mic", "entity": "腺病毒" } ] }, { "text": "多数患儿先有上呼吸道感染症状,伴发热、食欲缺乏。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "上呼吸道感染" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "发热" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "食欲缺乏" } ] }, { "text": "逐渐出现呼吸困难、阵发性喘憋,发作时呼吸快而浅,并伴有呼气性喘鸣,明显鼻扇及三凹征。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "阵发性喘憋" }, { "start_idx": 18, "end_idx": 31, "type": "sym", "entity": "呼吸快而浅,并伴有呼气性喘鸣" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "鼻" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "明显鼻扇" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "三凹征" } ] }, { "text": "严重病例有明显梗阻性肺气肿,常有极度烦躁不安、面色苍白及发绀。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "梗阻性肺气肿" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "极度烦躁不安" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "面色苍白" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "发绀" } ] }, { "text": "胸部叩诊呈过清音,毛细支气管接近完全梗阻时,呼吸音明显减低或完全听不到,或仅有呼气延长及哮鸣音,喘憋时常听不到湿啰音,趋于缓解时则可有弥漫性中小水泡音、捻发音。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "胸部叩诊" }, { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "过清音" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "毛细支气管" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "完全梗阻" }, { "start_idx": 22, "end_idx": 46, "type": "sym", "entity": "呼吸音明显减低或完全听不到,或仅有呼气延长及哮鸣音" }, { "start_idx": 48, "end_idx": 57, "type": "sym", "entity": "喘憋时常听不到湿啰音" }, { "start_idx": 59, "end_idx": 78, "type": "sym", "entity": "趋于缓解时则可有弥漫性中小水泡音、捻发音" } ] }, { "text": "胸片可见不同程度的梗阻性肺气肿,可伴支气管周围炎影像,部分患儿可有散在点片状阴影。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "胸片" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "梗阻性肺气肿" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "支气管周围炎" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "点片状阴影" } ] }, { "text": "周围血白细胞总数及分类多属正常;鼻咽分泌物病毒抗原检测、病毒分离或病毒核酸检测可阳性。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "周围血白细胞总数" }, { "start_idx": 16, "end_idx": 26, "type": "ite", "entity": "鼻咽分泌物病毒抗原检测" }, { "start_idx": 28, "end_idx": 38, "type": "ite", "entity": "病毒分离或病毒核酸检测" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "阳性" } ] }, { "text": "亦可通过测定血清特异IgM或双份血清抗体效价得出病原学诊断。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "ite", "entity": "血清特异IgM" }, { "start_idx": 14, "end_idx": 19, "type": "ite", "entity": "双份血清抗体" } ] }, { "text": "根据发病年龄偏小,发病初期即出现明显的喘憋,体格检查及X线检查在初期即出现明显肺气肿,诊断不难。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "喘憋" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "体格检查" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "X线检查" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "明显肺气肿" } ] }, { "text": "本病应与哮喘相鉴别。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "哮喘" } ] }, { "text": "应放置在冷湿化环境中,或冷湿化给氧,以缓解低氧血症、减少不显性失水。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "pro", "entity": "冷湿化给氧" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "低氧血症" } ] }, { "text": "避免使用镇静剂。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "镇静剂" } ] }, { "text": "有些患儿可发展成为反应性气道疾病。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "反应性气道疾病" } ] }, { "text": "多发生于儿童期,临床经过多样,儿童患者常表现为隐性感染和轻微的上呼吸道炎症。", "entities": [ { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "上呼吸道炎症" } ] }, { "text": "接触含有病毒的唾液是本病的主要传播方式。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "mic", "entity": "病毒" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "唾液" } ] }, { "text": "偶可经输血传播。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "输血" } ] }, { "text": "自身抗体是导致血液系统异常变化的因素之一。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "血液系统" } ] }, { "text": "病理改变为淋巴结滤泡增多增大,生发中心增大,其核心见母细胞、组织细胞和淋巴细胞。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "淋巴结滤泡" }, { "start_idx": 5, "end_idx": 13, "type": "sym", "entity": "淋巴结滤泡增多增大" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "生发中心" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "生发中心增大" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "母细胞" }, { "start_idx": 30, "end_idx": 33, "type": "bod", "entity": "组织细胞" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "脾脏增大2~3倍,充血,伴局灶性出血,脾包膜和小梁水肿、增厚伴淋巴样细胞浸润。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脾脏" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "脾脏增大2~3倍,充血" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "局灶性出血" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "脾包膜" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "小梁" }, { "start_idx": 19, "end_idx": 29, "type": "sym", "entity": "脾包膜和小梁水肿、增厚" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "淋巴样细胞浸润" } ] }, { "text": "神经系统病变包括神经元变性,血管周围出血和星状细胞增生,大脑皮层、基底节、小脑或脊髓等处小单核细胞浸润。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "神经系统" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "神经元变性" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "血管" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "血管周围出血和星状细胞增生" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "大脑皮层" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "基底节" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "小脑" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "脊髓" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "小单核细胞" }, { "start_idx": 28, "end_idx": 50, "type": "sym", "entity": "大脑皮层、基底节、小脑或脊髓等处小单核细胞浸润" } ] }, { "text": "显性表现常较轻微,如上呼吸道感染、扁桃体炎、持续发热伴或不伴淋巴结肿大。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "上呼吸道感染" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "扁桃体炎" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "持续发热" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "淋巴结" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "不伴淋巴结肿大" } ] }, { "text": "纵隔淋巴结肿大可引起咳嗽和气促。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "淋巴结" }, { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "淋巴结肿大" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "咳嗽" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "气促" } ] }, { "text": "常发生致死性单核细胞增多症、继发性低或无免疫球蛋白血症、恶性多克隆源性淋巴瘤、再生障碍性贫血、慢性淋巴细胞性间质性肺炎等。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "致死性单核细胞增多症" }, { "start_idx": 14, "end_idx": 26, "type": "dis", "entity": "继发性低或无免疫球蛋白血症" }, { "start_idx": 28, "end_idx": 37, "type": "dis", "entity": "恶性多克隆源性淋巴瘤" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "再生障碍性贫血" }, { "start_idx": 47, "end_idx": 58, "type": "dis", "entity": "慢性淋巴细胞性间质性肺炎" } ] }, { "text": "还可用免疫标记技术检测样本中病毒抗原,如EBNA、潜伏膜抗原(LYDMA成分之一)。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "免疫标记技术" }, { "start_idx": 14, "end_idx": 17, "type": "mic", "entity": "病毒抗原" }, { "start_idx": 20, "end_idx": 23, "type": "mic", "entity": "EBNA" } ] }, { "text": "遇有发热、扁桃体炎、咽峡炎和颈淋巴结肿大时应与链球菌性扁桃体炎和咽炎相鉴别,后者血象中性粒细胞增多,咽拭培养可检出细菌,青霉素治疗有效。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "发热" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "扁桃体炎" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "咽峡炎" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "颈淋巴结" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "颈淋巴结肿大" }, { "start_idx": 23, "end_idx": 30, "type": "dis", "entity": "链球菌性扁桃体炎" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "咽炎" }, { "start_idx": 42, "end_idx": 46, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 40, "end_idx": 48, "type": "sym", "entity": "血象中性粒细胞增多" }, { "start_idx": 60, "end_idx": 62, "type": "dru", "entity": "青霉素" } ] }, { "text": "如表现为传单(80%~95%由EBV所致),应与HCMV、弓形虫、腺病毒、风疹病毒和HAV等其他病原所致传单样综合征相鉴别,后者嗜异性抗体阴性,主要靠病原学检查区别。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "传单" }, { "start_idx": 15, "end_idx": 17, "type": "mic", "entity": "EBV" }, { "start_idx": 24, "end_idx": 27, "type": "mic", "entity": "HCMV" }, { "start_idx": 29, "end_idx": 31, "type": "mic", "entity": "弓形虫" }, { "start_idx": 33, "end_idx": 35, "type": "mic", "entity": "腺病毒" }, { "start_idx": 37, "end_idx": 40, "type": "mic", "entity": "风疹病毒" }, { "start_idx": 42, "end_idx": 44, "type": "mic", "entity": "HAV" }, { "start_idx": 52, "end_idx": 57, "type": "dis", "entity": "传单样综合征" } ] }, { "text": "脾大者恢复期应避免明显身体活动或运动,以防脾破裂;脾破裂时应紧急处理。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "脾" }, { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "脾大" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "脾" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "脾破裂" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "脾" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "脾破裂" } ] }, { "text": "因深部上呼吸道炎症致完全呼吸道梗阻时宜行气管插管。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "上呼吸道炎症" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "呼吸道" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "呼吸道梗阻" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "气管插管" } ] }, { "text": "初步研究显示,对严重EBV诱导的淋巴增生性疾病使用抗B细胞单抗和照射过的移植供体白细胞,同时减少免疫抑制剂用量有一定疗效。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "mic", "entity": "EBV" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "淋巴增生性疾病" } ] }, { "text": "尤其当胃肠道功能不成熟或者因疾病或手术前后不能耐受正常喂养时,往往营养素的需求是得不到满足的,可迅速发展为蛋白质-热能营养不良。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "胃肠道" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "手术" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "营养素" }, { "start_idx": 53, "end_idx": 62, "type": "dis", "entity": "蛋白质-热能营养不良" } ] }, { "text": "此时,可通过肠道内管饲或经肠外营养输注进行营养干预。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "管饲" }, { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "经肠外营养输注" } ] }, { "text": "肿瘤好发于下肢,膝关节最多见,其他依次为足、踝、髋;上肢主要发生于腕、肩、肘和手的关节。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "下肢" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "膝关节" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "足" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "踝" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "髋" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "上肢" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "腕" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "肩" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "肘和手的关节" } ] }, { "text": "头颈部肿瘤可能引起吞咽或呼吸困难。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "头颈部肿瘤" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "吞咽或呼吸困难" } ] }, { "text": "一般主张切除肿瘤,还要切除肿瘤附近的肌群或做截肢,区域淋巴结清扫。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "切除肿瘤" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "肿瘤" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "肌群" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "截肢" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "淋巴结清扫" } ] }, { "text": "患儿一般出生体重较低,胎盘常常很小,多为单侧脐动脉。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "患儿一般出生体重较低" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "胎盘常常很小" } ] }, { "text": "降低这类患儿的出生是优生优育的关键,取羊水细胞进行染色体检查是常用的产前诊断方法,核型分析类同外周血淋巴细胞染色体检查。", "entities": [ { "start_idx": 18, "end_idx": 29, "type": "pro", "entity": "取羊水细胞进行染色体检查" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "产前诊断" }, { "start_idx": 41, "end_idx": 58, "type": "pro", "entity": "核型分析类同外周血淋巴细胞染色体检查" } ] }, { "text": "对于染色体易位型患儿,则需检查其父母的染色体,以确定他们之一是否是平衡易位的携带者。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "染色体易位型" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "染色体" } ] }, { "text": "外周血白细胞计数常明显升高,伴核左移。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "外周血白细胞计数" } ] }, { "text": "确诊必须依靠特殊的化验检查,如应用特殊培养基从呼吸道标本或血、胸水中分离出病菌;应用免疫荧光或免疫酶法测定上述标本中的军团菌抗原或血清标本中的特异抗体。", "entities": [ { "start_idx": 23, "end_idx": 27, "type": "ite", "entity": "呼吸道标本" }, { "start_idx": 29, "end_idx": 29, "type": "ite", "entity": "血" }, { "start_idx": 31, "end_idx": 32, "type": "ite", "entity": "胸水" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "免疫荧光" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "免疫酶法" }, { "start_idx": 59, "end_idx": 63, "type": "mic", "entity": "军团菌抗原" }, { "start_idx": 65, "end_idx": 68, "type": "mic", "entity": "血清标本" } ] }, { "text": "首选大环内酯类,如红霉素及阿奇霉素、克拉霉素、罗红霉素等,疗程为2~3周。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "大环内酯类" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "红霉素" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "阿奇霉素" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "克拉霉素" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "罗红霉素" } ] }, { "text": "可加用利福平。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "利福平" } ] }, { "text": "喹诺酮类和氨基糖苷类虽有较好的抗菌活性,但儿童期尤其是年幼儿童禁用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "喹诺酮类" }, { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "氨基糖苷类" } ] }, { "text": "在病毒感染的基础上,易继发细菌感染,常见的致病菌为肺炎链球菌、流感嗜血杆菌等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "病毒感染" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "细菌感染" }, { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "致病菌" }, { "start_idx": 25, "end_idx": 29, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 31, "end_idx": 36, "type": "mic", "entity": "流感嗜血杆菌" } ] }, { "text": "多数患儿先有上呼吸道感染症状,3~4天后逐渐出现明显的咳嗽。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "上呼吸道感染" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "咳嗽" } ] }, { "text": "重者可有发热、头痛、乏力、胸骨后不适或胸前疼痛、腹痛、呕吐、腹泻等消化道症状。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "发热" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "头痛" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "乏力" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "胸骨后" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "胸骨后不适" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胸前" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "胸前疼痛" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "腹痛" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "呕吐" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "腹泻" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "消化道" } ] }, { "text": "开始为干咳,数天后咳嗽有痰,呈白色黏液痰或黄色脓痰,一般持续7~10天。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "干咳" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "咳嗽有痰" }, { "start_idx": 15, "end_idx": 24, "type": "sym", "entity": "白色黏液痰或黄色脓痰" } ] }, { "text": "如不及时治疗,可向下蔓延导致肺炎。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肺炎" } ] }, { "text": "胸部听诊可闻不固定干啰音及大、中水泡音,咳嗽或体位变化后可减少或消失。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "胸部听诊" }, { "start_idx": 6, "end_idx": 18, "type": "sym", "entity": "不固定干啰音及大、中水泡音" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "咳嗽" } ] }, { "text": "以化痰治疗为主。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "化痰治疗" } ] }, { "text": "感染、生理和心理因素已被鉴定为CVS的触发因素。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "CVS" } ] }, { "text": "CRF的清晨峰值也可解释CVS多于清晨发作的原因。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "CRF" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "CVS" } ] }, { "text": "CVS发病时许多症状如苍白、发热、嗜睡、恶心、呕吐及过量流涎等都为自主神经功能紊乱症状。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "CVS" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "苍白" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "嗜睡" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "恶心" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "呕吐" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "过量流涎" }, { "start_idx": 33, "end_idx": 40, "type": "dis", "entity": "自主神经功能紊乱" } ] }, { "text": "临床以会阴及肛门附近瘙痒为特征。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "会阴" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肛门" }, { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "会阴及肛门附近瘙痒" } ] }, { "text": "雌虫有时亦可侵入阴道、输卵管及腹腔而引起阴道炎、盆腔腹膜炎,侵入尿道可引起尿道炎,侵入阑尾引起阑尾炎。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "雌虫" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "阴道" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "输卵管" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "腹腔" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "阴道炎" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "盆腔腹膜炎" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "尿道" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "尿道炎" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "阑尾" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "阑尾炎" } ] }, { "text": "有时可见恶心、呕吐、腹泻、腹痛、食欲缺乏等症状。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "恶心" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "呕吐" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "腹泻" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "腹痛" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "食欲缺乏" } ] }, { "text": "可有不安、夜惊、精神易激动及其他神经症状。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "不安" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "夜惊" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "精神易激动" } ] }, { "text": "亦有鼻蛲虫的报道,主要表现为鼻部瘙痒、鼻出血、鼻塞、流涕,查体可见鼻前庭皮肤增厚、慢性出血、鼻中隔黏膜糜烂、溃疡、渗血。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "mic", "entity": "鼻蛲虫" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "鼻部" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "鼻部瘙痒" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "鼻" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "鼻出血" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "鼻塞" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "流涕" }, { "start_idx": 29, "end_idx": 30, "type": "ite", "entity": "查体" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "鼻前庭皮肤增厚" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "慢性出血" }, { "start_idx": 46, "end_idx": 50, "type": "bod", "entity": "鼻中隔黏膜" }, { "start_idx": 46, "end_idx": 58, "type": "sym", "entity": "鼻中隔黏膜糜烂、溃疡、渗血" } ] }, { "text": "近年来被明确归入功能性胃肠道疾病,目前公认的定义为3次或反复多次的发作性顽固的恶心和呕吐,每次发作持续数小时至数日,2次发作间期有长达数周至数日的完全无症状间隙期。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "功能性胃肠道疾病" }, { "start_idx": 25, "end_idx": 80, "type": "sym", "entity": "3次或反复多次的发作性顽固的恶心和呕吐,每次发作持续数小时至数日,2次发作间期有长达数周至数日的完全无症状间隙期" } ] }, { "text": "双侧肺发育不全或不发育常无法存活,生后迅速出现严重呼吸困难和呼吸衰竭。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "dis", "entity": "双侧肺发育不全或不发育" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "无法存活" }, { "start_idx": 19, "end_idx": 33, "type": "sym", "entity": "迅速出现严重呼吸困难和呼吸衰竭" } ] }, { "text": "单侧肺发育不全或不发育以左侧多见,特异症状少,仅1/3患儿生前得到诊断;部分于新生儿期出现肺活量不足表现,常伴有持续肺动脉高压。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "dis", "entity": "单侧肺发育不全或不发育" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "肺活量不足" }, { "start_idx": 56, "end_idx": 62, "type": "sym", "entity": "持续肺动脉高压" } ] }, { "text": "体格检查显示患侧肺部叩诊浊音,呼吸音减低,尤以腋下及肺底部为明显,气管和心脏向患侧移位。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "体格检查" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "患侧肺部叩诊浊音" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "呼吸音减低" } ] }, { "text": "支气管镜及支气管造影检查可确定诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "支气管镜" }, { "start_idx": 5, "end_idx": 11, "type": "ite", "entity": "支气管造影检查" } ] }, { "text": "CT和MRI可准确反映气道整体情况和血管解剖畸形。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "CT" }, { "start_idx": 3, "end_idx": 5, "type": "ite", "entity": "MRI" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "气道" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "血管" } ] }, { "text": "可出现过度兴奋、喊叫乃至全身惊厥等神经系统表现。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "过度兴奋" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "喊叫" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "全身惊厥" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "神经系统" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "神经系统表现" } ] }, { "text": "可先后出现肾小管性蛋白尿,全氨基酸尤以赖氨酸和酸氨酸明显,还可出现高磷尿症而致血磷降低,引起抗维生素D性佝偻病或骨质疏松,肾小管性酸中毒也较常见,而糖尿往往不明显,多无低血钾以及多尿,即使有也表现轻微。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "肾小管性蛋白尿" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "全氨基酸" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "赖氨酸" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "酸氨酸" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "高磷尿症" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "血磷降低" }, { "start_idx": 46, "end_idx": 54, "type": "dis", "entity": "抗维生素D性佝偻病" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "骨质疏松" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "肾小管性酸中毒" }, { "start_idx": 74, "end_idx": 75, "type": "sym", "entity": "糖尿" }, { "start_idx": 83, "end_idx": 90, "type": "sym", "entity": "无低血钾以及多尿" } ] }, { "text": "有高钙尿症者还需口服氢氯噻嗪,降低尿钙,以免形成肾脏钙化及肾结石。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "高钙尿症" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "氢氯噻嗪" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "降低尿钙" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "肾脏钙化" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "肾结石" } ] }, { "text": "幽门螺杆菌是慢性活动性胃炎的主要致病因素,是消化性溃疡发病的重要因素。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "慢性活动性胃炎" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "消化性溃疡" } ] }, { "text": "幽门螺杆菌的长期感染也与胃腺癌和胃黏膜相关淋巴组织(MALT)淋巴瘤的发生相关。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "胃腺癌" }, { "start_idx": 16, "end_idx": 33, "type": "dis", "entity": "胃黏膜相关淋巴组织(MALT)淋巴瘤" } ] }, { "text": "临床研究发现,根除幽门螺杆菌可明显降低消化性溃疡病的复发率,还可使胃MALT淋巴瘤病程发生逆转,甚至消失;并可改善一部分慢性胃炎患者的顽固性消化不良症状。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "消化性溃疡病" }, { "start_idx": 33, "end_idx": 40, "type": "dis", "entity": "胃MALT淋巴瘤" }, { "start_idx": 60, "end_idx": 63, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 67, "end_idx": 73, "type": "dis", "entity": "顽固性消化不良" } ] }, { "text": "在西方发达国家地区儿童与青少年中一般很少有幽门螺杆菌定植,与此相反的是在发展中国家幽门螺杆菌感染率较高,10岁即有一半感染幽门螺杆菌。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 41, "end_idx": 45, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 61, "end_idx": 65, "type": "mic", "entity": "幽门螺杆菌" } ] }, { "text": "此外最近报道在人的一生中儿童期较成人期容易获得幽门螺杆菌感染,出生较早的较出生迟的个体具有更容易获得幽门螺杆菌感染的危险。", "entities": [ { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "幽门螺杆菌感染" } ] }, { "text": "但幽门螺杆菌是怎样从人到人之间的传播尚不清楚,目前较多的依据是支持口-口与粪-口途径传播。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "口-口" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "粪-口" } ] }, { "text": "有人提出母亲喂小孩饭前的咀嚼食物或吃饭时共用一个饭碗可能是发展中国家幽门螺杆菌感染的传播机制。", "entities": [ { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "幽门螺杆菌感染" } ] }, { "text": "有不少文献提示,儿童幽门螺杆菌可以导致铁的缺乏,若在补充铁剂同时给予根除幽门螺杆菌治疗甚至是单独采用根除方案,病情即可恢复。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 28, "end_idx": 29, "type": "dru", "entity": "铁剂" }, { "start_idx": 36, "end_idx": 40, "type": "mic", "entity": "幽门螺杆菌" } ] }, { "text": "欧洲幽门螺杆菌研究小组宣布的MaastrichtⅢ共识提出,对所有缺铁性贫血患者在检查完其他原因外应行幽门螺杆菌检测和根除治疗。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "缺铁性贫血" }, { "start_idx": 51, "end_idx": 57, "type": "pro", "entity": "幽门螺杆菌检测" }, { "start_idx": 59, "end_idx": 62, "type": "pro", "entity": "根除治疗" } ] }, { "text": "幽门螺杆菌感染缺铁性贫血发病机制目前尚不清楚,可能是影响了铁的吸收,或增加了铁的消耗及流失,导致铁的储备不够。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "缺铁性贫血" } ] }, { "text": "此外,还可提供细菌的药物敏感试验,指导临床选用药物,尤其是治疗失败者或生活在幽门螺杆菌耐药性很高的国家与地区。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "药物敏感试验" }, { "start_idx": 38, "end_idx": 42, "type": "mic", "entity": "幽门螺杆菌" } ] }, { "text": "除常规的组织学检查外,还有免疫化学和免疫荧光方法,但需使用免疫荧光镜和免疫抗体,多用于实验室研究。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "组织学检查" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "免疫化学" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "免疫荧光方法" } ] }, { "text": "福建三强生化有限公司生产的CPUT试剂盒属于这种方法,可半定量指示幽门螺杆菌感染的程度(+、++、+++、++++)。", "entities": [ { "start_idx": 13, "end_idx": 19, "type": "equ", "entity": "CPUT试剂盒" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "幽门螺杆菌感染" } ] }, { "text": "呼气试验具有快速、可靠、安全及无痛苦的优点,适合大规模流行病学调查,表明目前是否有幽门螺杆菌感染,优于血清学检查。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "呼气试验" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 51, "end_idx": 55, "type": "pro", "entity": "血清学检查" } ] }, { "text": "管儿童是幽门螺杆菌的易感人群,已有的资料也显示中国儿童人群中幽门螺杆菌感染率很高,感染后所造成的结局相差有悬殊;因根除治疗困难以及抗生素的长期应用会产生不良反应等因素的存在,所以对所有幽门螺杆菌感染者都给予根除治疗显然是不可能也不现实的。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 30, "end_idx": 34, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "根除治疗" }, { "start_idx": 92, "end_idx": 98, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 103, "end_idx": 106, "type": "pro", "entity": "根除治疗" } ] }, { "text": "中国儿科消化学组制定的共识,对儿童目前主要用于幽门螺杆菌感染性胃炎和消化性溃疡,胃MALT患儿以及消化不良症状严重且反复发生幽门螺杆菌的患儿。", "entities": [ { "start_idx": 23, "end_idx": 32, "type": "dis", "entity": "幽门螺杆菌感染性胃炎" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "胃MALT" }, { "start_idx": 62, "end_idx": 66, "type": "mic", "entity": "幽门螺杆菌" } ] }, { "text": "急性胰腺炎在儿童中少见,其发病原因多种多样,临床上常有急性发作的上腹部剧痛伴恶心、呕吐及血尿淀粉酶增高,疾病初期常易被忽视或误诊。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "轻型胰腺炎多见,仅有轻度的胰腺功能障碍,去除发作的病因后多不会再有发作,病情呈自限性,一般病程在1~2周,胰腺的形态和功能亦恢复正常。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "胰腺功能障碍" } ] }, { "text": "重型胰腺炎少见,有器官衰竭或坏死、脓肿、假性囊肿等局部并发症存在,病情急重,病死率高。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "成人急性胰腺炎的病因主要是胆石症及酗酒。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "其中流行性腮腺炎病毒引起的胰腺炎是较常见的。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "mic", "entity": "流行性腮腺炎病毒" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "在亚洲地区寄生虫感染也是一种多见的原因,如蛔虫及肝吸虫引起的上行性感染、梗阻可导致急性胰腺炎的发生。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "寄生虫感染" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "上行性感染" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "梗阻" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "细菌感染如沙门菌、痢疾杆菌、弯曲菌及钩端螺旋体感染可伴急性胰腺炎,但多是由于其毒素引起。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "mic", "entity": "沙门菌" }, { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "痢疾杆菌" }, { "start_idx": 14, "end_idx": 16, "type": "mic", "entity": "弯曲菌" }, { "start_idx": 18, "end_idx": 22, "type": "mic", "entity": "钩端螺旋体" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "此类疾病因血管炎累及胰腺和其他脏器的血管,引起血管壁的炎症、坏死、血栓形成而致坏死性胰腺炎。", "entities": [ { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "患者常在幼年开始发生典型的急性胰腺炎,以后转为慢性反复发作,逐渐导致胰腺的钙化、糖尿病和脂肪泻。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "急性水肿型胰腺炎通常呈自限性,预后一般较好。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "急性坏死性胰腺炎在儿童少见。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "用于评估成人胰腺炎严重性的体系通常不适用于儿科患者。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "剧烈腹痛是胰腺炎的主要症状,但小儿有时仅表现为阵发性哭闹,而小婴儿可无腹痛,仅有反应差及面色灰等表现。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "典型胰腺炎往往左上腹痛,而小儿腹痛常为全腹性甚至伴明显肌卫,易并发腹膜炎。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "有文献报道急性胰腺炎如具有以下症状之一者即应拟诊急性坏死性胰腺炎:①急性胰腺炎经内科治疗4~72小时,病情无改善或加重,表现为高热、末梢血白细胞计数明显增高及脏器功能衰竭(肾衰竭及休克肺等);②腹胀明显,肠鸣音减弱或出现麻痹性肠梗阻;③出现腹水,尤其是血性腹水,其淀粉酶升高;④发生休克。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "75%急性胰腺炎患者血清淀粉酶增高达正常的3倍并持续数日,一般在症状发作后2~12小时即增高,24小时最高峰,48小时后高峰下降而尿淀粉酶升高,尿淀粉酶可持续1~2周。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "1)禁食:急性胰腺炎的患者应绝对禁食,直到腹痛消失可开始进少量流质。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "脂肪乳剂有利于补充代谢需要,有利于重型胰腺炎的恢复,故现主张可予以适量的脂肪乳剂。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "在成人的急性坏死性胰腺炎的治疗中,许多专家学者强调要晚期手术,但国内儿科较多主张一旦确诊为急性坏死性胰腺炎时,即应做手术治疗。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "1/3病例可有先天性心血管畸形。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "先天性心血管畸形" } ] }, { "text": "发育明显落后,2岁时才会坐,4岁时才会走,出现一种痉挛性步态。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "发育明显落后" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "2岁时才会坐" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "4岁时才会走" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "出现一种痉挛性步态" } ] }, { "text": "患儿通常有臀位难产史,临床症状大多开始于生后2天之内,以后很快出现脑干受压症状,如木僵、昏迷、脑神经异常、呼吸暂停、心动过缓或角弓反张等。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "臀位难产" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "脑干" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "脑干受压" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "木僵" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "昏迷" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "脑神经异常" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "心动过缓" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "角弓反张" } ] }, { "text": "小脑出血的诊断主要靠临床医师高度警惕,确诊可通过CT或MRI,有时头颅超声也可证实小脑出血,但阴性结果不能排除本病。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "小脑出血" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "CT" }, { "start_idx": 27, "end_idx": 29, "type": "pro", "entity": "MRI" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "头颅超声" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "小脑出血" } ] }, { "text": "带菌妇女所分娩的婴儿约有1%~2%发生早发GBS感染。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "GBS感染" } ] }, { "text": "极低出生体重儿GBS感染的发病率为8‰。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "GBS感染" } ] }, { "text": "早发GBS感染的发病率受一些产科危险因素的影响,如早产、低出生体重(体重≤2500g)、胎膜早破(>18小时)、母亲有产褥期发热、母亲分娩时为阴道GBS带菌者和母亲有绒毛膜羊膜炎或子宫内膜炎等均可增加感染的发生率。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "GBS感染" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "发热" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "阴道" }, { "start_idx": 73, "end_idx": 75, "type": "mic", "entity": "GBS" }, { "start_idx": 83, "end_idx": 88, "type": "dis", "entity": "绒毛膜羊膜炎" }, { "start_idx": 90, "end_idx": 94, "type": "dis", "entity": "子宫内膜炎" } ] }, { "text": "主要(90%)由GBSⅢ型引起。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "mic", "entity": "GBSⅢ型" } ] }, { "text": "晚发GBS感染骨髓炎的病理生理改变不典型,可以由早发的无症状的菌血症引起,很少有全身症状。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "GBS感染骨髓炎" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "菌血症" } ] }, { "text": "脑膜炎的发生率低于10%,可以有惊厥、嗜睡、昏迷、前囟隆起等表现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "惊厥" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "嗜睡" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "昏迷" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "前囟隆起" } ] }, { "text": "晚发GBS感染主要临床表现为脑膜炎。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "GBS感染" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "常见隐匿起病,最初可表现为发热、昏睡、呕吐、囟门饱满等。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "发热" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "昏睡" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "呕吐" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "囟门饱满" } ] }, { "text": "化脓性关节炎常累及髋、膝、踝关节,症状可持续数天。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "化脓性关节炎" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "髋" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "膝" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "踝关节" } ] }, { "text": "GBS蜂窝组织炎或淋巴结炎常不对称,可累及面部或下颌部,腹股沟、阴囊和髌骨前也可受累。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "GBS蜂窝组织炎" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "淋巴结炎" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "面部" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "下颌部" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "腹股沟" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "阴囊" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "髌骨" } ] }, { "text": "细菌学检查是诊断GBS感染的基本手段,依靠从血液、脑脊液或感染病灶(如骨、关节、脓肿液等)中分离出病原菌即可诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "细菌学检查" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "GBS感染" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血液" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "脑脊液" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "骨" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "关节" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "脓肿液" } ] }, { "text": "快速抗原检测的方法可以从体液中直接检测GBS抗原,常用的方法有乳胶凝集试验(LA)、协同凝集试验(COA)、对流免疫电泳(CIE)等,其敏感性比培养低,但对于产前已应用了抗生素治疗的母亲或婴儿及非菌血症、败血症的检测是非常有用的。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "快速抗原检测" }, { "start_idx": 19, "end_idx": 21, "type": "mic", "entity": "GBS" }, { "start_idx": 31, "end_idx": 36, "type": "pro", "entity": "乳胶凝集试验" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "LA" }, { "start_idx": 42, "end_idx": 47, "type": "pro", "entity": "协同凝集试验" }, { "start_idx": 49, "end_idx": 51, "type": "pro", "entity": "COA" }, { "start_idx": 54, "end_idx": 59, "type": "pro", "entity": "对流免疫电泳" }, { "start_idx": 61, "end_idx": 63, "type": "pro", "entity": "CIE" }, { "start_idx": 85, "end_idx": 89, "type": "pro", "entity": "抗生素治疗" }, { "start_idx": 98, "end_idx": 100, "type": "dis", "entity": "菌血症" }, { "start_idx": 102, "end_idx": 104, "type": "dis", "entity": "败血症" } ] }, { "text": "但抗原检测可以有假阳性,因此抗原检测的阳性结果只能提示可能有GBS感染,同时应结合感染的临床表现来综合判断。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "抗原检测" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "抗原检测" }, { "start_idx": 30, "end_idx": 34, "type": "pro", "entity": "GBS感染" } ] }, { "text": "对青霉素耐药,可选择氨苄西林、氨基糖苷类或头孢类抗生素。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "青霉素" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "氨基糖苷类" }, { "start_idx": 21, "end_idx": 26, "type": "dru", "entity": "头孢类抗生素" } ] }, { "text": "脑膜炎的治疗应用大剂量的青霉素,也可用氨苄西林加庆大霉素,疗程2~3周。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "青霉素" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "庆大霉素" } ] }, { "text": "支持治疗、治疗休克、纠正通气不良、控制颅内高压对于改善GBS感染的预后尤为重要。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "支持治疗" }, { "start_idx": 10, "end_idx": 15, "type": "pro", "entity": "纠正通气不良" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "控制颅内高压" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "GBS感染" } ] }, { "text": "辅助应用静脉注射免疫球蛋白(IVIG)可增加吞噬细胞的杀菌力,应用500~800mg/kg的剂量,对于新生儿败血症是安全的。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "pro", "entity": "静脉注射免疫球蛋白" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "IVIG" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "吞噬细胞" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "新生儿败血症" } ] }, { "text": "病程长者,可影响局部发育出现小颌畸形;累及喉杓(环状软骨及杓状软骨)可致声哑、喉喘鸣和饮食困难。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "小颌畸形" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "喉杓" }, { "start_idx": 24, "end_idx": 32, "type": "bod", "entity": "环状软骨及杓状软骨" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "声哑" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "喉喘鸣" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "饮食困难" } ] }, { "text": "部分患儿晨起关节活动障碍,但病变关节可不发红,即晨僵。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "关节" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "活动障碍" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "关节" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "不发红" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "晨僵" } ] }, { "text": "偶见个别幼儿颌关节炎后导致口腔活动障碍,面部不对称而需要外科手术纠正。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "面部不对称" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "外科手术" } ] }, { "text": "而在JIA总体阳性率不足15%,主要出现在多关节型JIA之中,RF阴性并不能除外诊断JIA。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "JIA" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "多关节型JIA" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "RF阴性" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "JIA" } ] }, { "text": "各型患儿活动期HIgM-RF均值高于缓解期,并与病情活动性有关。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "HIgM-RF" } ] }, { "text": "256例JIA患儿检测ANA阳性结果分析与发病年龄偏小、不对称性关节炎及虹膜睫状体炎的发生有关。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "JIA" }, { "start_idx": 11, "end_idx": 13, "type": "ite", "entity": "ANA" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "虹膜睫状体炎" } ] }, { "text": "因此建议将APF作为JIA诊断指标。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "pro", "entity": "APF" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "JIA" } ] }, { "text": "研究发现抗Sa抗体与RF、RA3、SSA、SSB、RNP、Sm、Jo-1及Scl-70等多种自身抗体无交叉反应性,Sa抗体对JIA的诊断价值罕见报告。", "entities": [ { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "Sa抗体" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "JIA" } ] }, { "text": "各种NSAIDs药理机制及副作用基本相似,因此不能两种NSAIDs联合使用。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "NSAIDs" }, { "start_idx": 27, "end_idx": 32, "type": "dru", "entity": "NSAIDs" } ] }, { "text": "尽管为成人类风湿关节炎(RA)指南,对儿童亦有较好的指导作用,该指南第一项提出即一经诊断RA即可早期使用DMARDs治疗。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "成人类风湿关节炎" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "RA" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "RA" }, { "start_idx": 52, "end_idx": 57, "type": "dru", "entity": "DMARDs" } ] }, { "text": "CSA可用于全身型JIA,尤其是合并巨噬细胞活化综合征患儿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "CSA" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "全身型JIA" }, { "start_idx": 16, "end_idx": 26, "type": "dis", "entity": "合并巨噬细胞活化综合征" } ] }, { "text": "糖皮质激素治疗RA价值有争议,成人RA的“强化治疗”理念认为“应依据病情活动度制订个体化的早期联合治疗方案,此后密切随访,根据疗效及时调整用药,使患者的病情活动度能在最快时间内达临床缓解,防止关节破坏及关节外损伤”。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "RA" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "RA" }, { "start_idx": 96, "end_idx": 97, "type": "bod", "entity": "关节" }, { "start_idx": 101, "end_idx": 102, "type": "bod", "entity": "关节" } ] }, { "text": "HGA是由于嗜吞噬细胞无形体感染引起的经蜱传播的立克次体病,主要侵犯人中性粒细胞,临床以发热伴白细胞、血小板减少和多脏器功能损害为主要临床表现。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "HGA" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "嗜吞噬细胞" }, { "start_idx": 6, "end_idx": 15, "type": "sym", "entity": "嗜吞噬细胞无形体感染" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "立克次体病" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "人中性粒细胞" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "发热" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "白细胞" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "血小板" }, { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "白细胞、血小板减少" }, { "start_idx": 57, "end_idx": 63, "type": "sym", "entity": "多脏器功能损害" } ] }, { "text": "我国已有报道黑龙江、蒙古及新疆等地的全沟硬蜱中检测到人粒细胞无形体核酸。", "entities": [ { "start_idx": 26, "end_idx": 34, "type": "bod", "entity": "人粒细胞无形体核酸" } ] }, { "text": "免疫缺陷患者及应用糖皮质激素治疗者感染本病后病情多较危重。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "免疫缺陷" }, { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "糖皮质激素" } ] }, { "text": "尿常规可出现蛋白尿、血尿、管型尿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "尿常规" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "血尿" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "管型尿" } ] }, { "text": "血生化检查可出现肝、肾功能异常,心肌酶谱升高,少数患者出现血淀粉酶、尿淀粉酶和血糖升高。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "血生化检查" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "肝" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "肾" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "肝、肾功能异常" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "心肌酶谱升高" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "血淀粉酶" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "尿淀粉酶" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "血糖" }, { "start_idx": 29, "end_idx": 42, "type": "sym", "entity": "血淀粉酶、尿淀粉酶和血糖升高" } ] }, { "text": "全血或血细胞标本PCR检测嗜吞噬细胞无形体特异性核酸阳性,或分离到病原体。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "PCR检测" }, { "start_idx": 13, "end_idx": 25, "type": "bod", "entity": "嗜吞噬细胞无形体特异性核酸" }, { "start_idx": 8, "end_idx": 27, "type": "sym", "entity": "PCR检测嗜吞噬细胞无形体特异性核酸阳性" } ] }, { "text": "按临床表现又可分为少尿型与非少尿型以及高分解型。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "少尿型" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "非少尿型" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "高分解型" } ] }, { "text": "败血症也可引起弥散性血管内凝血(DIC),导致急性肾衰。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "败血症" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "弥散性血管内凝血" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "DIC" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "急性肾衰" } ] }, { "text": "中毒性ATN的病变限于近端小管,呈局灶性分布,坏死的肾小管基膜完整,小管上皮再生良好。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "中毒性ATN" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "近端小管" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "局灶性分布" }, { "start_idx": 23, "end_idx": 32, "type": "sym", "entity": "坏死的肾小管基膜完整" }, { "start_idx": 34, "end_idx": 41, "type": "sym", "entity": "小管上皮再生良好" } ] }, { "text": "而缺血性ATN病变可涉及各段肾小管,呈弥漫性分布,坏死的小管基底膜断裂,上皮细胞再生较差。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "缺血性ATN病变" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肾小管" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "弥漫性分布" }, { "start_idx": 25, "end_idx": 34, "type": "sym", "entity": "坏死的小管基底膜断裂" }, { "start_idx": 36, "end_idx": 43, "type": "sym", "entity": "上皮细胞再生较差" } ] }, { "text": "②氮质血症:血BUN及Cr增高,并出现由于毒素在体内储积而引起的全身各系统中毒症状,如厌食、恶心、呕吐、呕血、嗜睡、烦躁及贫血等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "氮质血症" }, { "start_idx": 6, "end_idx": 9, "type": "ite", "entity": "血BUN" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "Cr" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "厌食" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "恶心" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "呕吐" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "呕血" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "嗜睡" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "烦躁" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "贫血" } ] }, { "text": "③水钠潴留:全身水肿、血压升高,并可出现肺水肿、脑水肿及心力衰竭等表现。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "水钠潴留" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "全身水肿" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "血压升高" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "并可出现肺水肿" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "脑水肿" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "心力衰竭等表现" } ] }, { "text": "高磷及低钙血症,可出现手足搐搦及惊厥等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "高磷及低钙血症" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "手足搐搦" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "惊厥" } ] }, { "text": "⑤代谢性酸中毒:表现为疲乏、嗜睡、面色潮红、恶心、呕吐、呼吸深大,甚至昏迷、休克等。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "疲乏" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "嗜睡" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "面色潮红" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "恶心" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "呕吐" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "呼吸深大" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "昏迷" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "休克" } ] }, { "text": "2.防治感染。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "防治感染" } ] }, { "text": "3.加强营养,纠正贫血。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "加强营养" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "贫血" } ] }, { "text": "胸水苏丹乙醇染色可见红色脂肪颗粒。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胸水" }, { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "苏丹乙醇" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "脂肪颗粒" } ] }, { "text": "胸水乙醚试验:胸水加少量乙醚振荡均匀静置片刻,胸水变清亮。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "胸水乙醚试验" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "胸水" }, { "start_idx": 12, "end_idx": 13, "type": "dru", "entity": "乙醚" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "胸水" } ] }, { "text": "反复胸腔穿刺可减轻压迫症状,但乳糜可迅速漏出重新积聚,并导致热量、蛋白质和淋巴细胞的丢失,继发免疫功能缺陷,如低免疫球蛋白血症和细胞免疫功能异常。", "entities": [ { "start_idx": 18, "end_idx": 25, "type": "pro", "entity": "迅速漏出重新积聚" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "蛋白质" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 55, "end_idx": 62, "type": "dis", "entity": "低免疫球蛋白血症" }, { "start_idx": 64, "end_idx": 71, "type": "dis", "entity": "细胞免疫功能异常" } ] }, { "text": "多数学者主张乳糜胸患儿应给予低脂肪、高蛋白质、高热量饮食,适当限制盐的摄入。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "乳糜胸" }, { "start_idx": 12, "end_idx": 36, "type": "pro", "entity": "给予低脂肪、高蛋白质、高热量饮食,适当限制盐的摄入" } ] }, { "text": "若内科保守治疗无效,可行手术治疗,如胸导管结扎、胸腹腔引流术。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "pro", "entity": "内科保守治疗" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "胸导管结扎" }, { "start_idx": 24, "end_idx": 29, "type": "pro", "entity": "胸腹腔引流术" } ] }, { "text": "此外,文献报道还与单纯疱疹和带状疱疹病毒,流感A和B、流行性腮腺炎、麻疹、柯萨奇、甲型和乙型肝炎病毒,天花和人类免疫缺陷病毒等感染有关。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "单纯疱疹" }, { "start_idx": 14, "end_idx": 19, "type": "mic", "entity": "带状疱疹病毒" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "流感A和B" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "流行性腮腺炎" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "麻疹" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "柯萨奇" }, { "start_idx": 41, "end_idx": 49, "type": "mic", "entity": "甲型和乙型肝炎病毒" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "天花" }, { "start_idx": 54, "end_idx": 61, "type": "mic", "entity": "人类免疫缺陷病毒" } ] }, { "text": "目前空肠弯曲杆菌及GBS的相关性引起广泛关注,空肠弯曲杆菌(CJ)是引起急性胃肠炎的主要病原,也是最常见的GBS的前驱感染源。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "mic", "entity": "空肠弯曲杆菌" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "GBS" }, { "start_idx": 23, "end_idx": 28, "type": "mic", "entity": "空肠弯曲杆菌" }, { "start_idx": 30, "end_idx": 31, "type": "mic", "entity": "CJ" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "急性胃肠炎" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "GBS" } ] }, { "text": "各亚型的临床及病理特征各异,但最主要的病理改变为周围神经中单核细胞浸润和节段性脱髓鞘。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "单核细胞" }, { "start_idx": 36, "end_idx": 41, "type": "bod", "entity": "节段性脱髓鞘" } ] }, { "text": "严重者24~48小时内发生呼吸肌麻痹,需立即机械通气。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "呼吸肌麻痹" } ] }, { "text": "感觉障碍包括麻木感、蚁行感、针刺感,以及烧灼感。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "麻木感" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "蚁行感" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "针刺感" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "烧灼感" } ] }, { "text": "(4)抗Ga1NAc-GD1a抗体:此抗体与前驱空肠弯曲杆菌感染相关,研究表明伴有此抗体的GBS患者可出现快速进展,非常严重的肌无力(以远端肌群为主)。", "entities": [ { "start_idx": 3, "end_idx": 16, "type": "bod", "entity": "抗Ga1NAc-GD1a抗体" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "抗体" }, { "start_idx": 22, "end_idx": 31, "type": "dis", "entity": "前驱空肠弯曲杆菌感染" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "抗体" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "GBS" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "严重的肌无力" }, { "start_idx": 68, "end_idx": 71, "type": "bod", "entity": "远端肌群" } ] }, { "text": "8)变异型:①神经症状发生时发热。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "神经症状" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "发热" } ] }, { "text": "最重要的是观察呼吸肌的力量,最方便的床旁方法是测肺活量,对高危患者应每隔2小时监测一次肺活量,当肺活量下降至15ml/kg时,即使患者未出现低氧血症,也需进行机械通气。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "观察呼吸肌的力量" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "测肺活量" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "肺活量" }, { "start_idx": 48, "end_idx": 50, "type": "ite", "entity": "肺活量" }, { "start_idx": 70, "end_idx": 73, "type": "dis", "entity": "低氧血症" }, { "start_idx": 79, "end_idx": 82, "type": "pro", "entity": "机械通气" } ] }, { "text": "因此对那些发病5天内不能吞咽的患者需给予营养支持。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "营养支持" } ] }, { "text": "勤翻身可避免褥疮及因长期卧床导致的深静脉栓塞及肺栓塞等并发症。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "褥疮" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "深静脉栓塞" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "肺栓塞" } ] }, { "text": "疼痛是GBS常见的症状,可能与多种因素有关,如神经根炎及神经炎,不能活动等造成的肌肉疼痛和痛觉过敏。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "疼痛" }, { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "GBS" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "神经根炎" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "神经炎" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "肌肉疼痛" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "痛觉过敏" } ] }, { "text": "短期应用大剂量肾上腺皮质激素有时也有效。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dru", "entity": "肾上腺皮质激素" } ] }, { "text": "患者在入院后的1~2天内即可进行理疗,肢体做被动锻炼,但应避免骨折。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肢体" } ] }, { "text": "据报道大剂量静注免疫球蛋白应用于重症GBS,可以降低气管插管及机械通气的需要,缩短患者在ICU的时间,以及促进其功能恢复。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "大剂量静注免疫球蛋白" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "重症GBS" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "气管插管" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "机械通气" }, { "start_idx": 44, "end_idx": 46, "type": "dep", "entity": "ICU" } ] }, { "text": "有学者认为大剂量肾上腺皮质激素冲击疗效好,能抑制B细胞产生抗体,同时减轻神经组织水肿,方法为甲泼尼龙,开始剂量为15mg/(kg•d),3~5天后改为口服泼尼松,4周后减量,总疗程为6~7周。", "entities": [ { "start_idx": 5, "end_idx": 18, "type": "pro", "entity": "大剂量肾上腺皮质激素冲击疗效" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "B细胞" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "抗体" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "神经组织水肿" }, { "start_idx": 46, "end_idx": 49, "type": "dru", "entity": "甲泼尼龙" }, { "start_idx": 77, "end_idx": 79, "type": "dru", "entity": "泼尼松" } ] }, { "text": "有报道指出肾上腺皮质激素与静脉注射丙球蛋白联合应用疗效显著。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 13, "end_idx": 20, "type": "pro", "entity": "静脉注射丙球蛋白" } ] }, { "text": "总之,GBS的治疗以综合疗法为宜。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "GBS" } ] }, { "text": "此病变很少见于婴儿,手术切除后又可重新长出,且许多先天性心脏病手术后可长出主动脉瓣下隔膜,所以有人认为此系后天长出,但为先天的局部组织异常所致。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "手术" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 37, "end_idx": 43, "type": "bod", "entity": "主动脉瓣下隔膜" } ] }, { "text": "此外,二尖瓣的许多畸形如降落伞样二尖瓣等亦可有左心室流出道的梗阻。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 12, "end_idx": 18, "type": "bod", "entity": "降落伞样二尖瓣" }, { "start_idx": 23, "end_idx": 31, "type": "dis", "entity": "左心室流出道的梗阻" } ] }, { "text": "临床上主动脉瓣下与瓣膜狭窄不易鉴别,左心室造影和超声对诊断有决定意义。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "瓣膜" }, { "start_idx": 18, "end_idx": 22, "type": "pro", "entity": "左心室造影" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "超声" } ] }, { "text": "重者需手术矫治。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "手术矫治" } ] }, { "text": "患儿头小,前额后缩,前囟大及骨缝宽,颅顶头皮有溃疡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "患儿头小" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "前额后缩" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "前囟大" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "骨缝宽" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "颅顶头皮有溃疡" } ] }, { "text": "睑裂呈水平线,可见不同程度的小眼至无眼,眼距宽,有白内障、虹膜缺损及视网膜发育异常。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "睑裂呈水平线" }, { "start_idx": 9, "end_idx": 18, "type": "sym", "entity": "不同程度的小眼至无眼" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "眼距宽" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "白内障" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "虹膜" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "虹膜缺损" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "视网膜" }, { "start_idx": 34, "end_idx": 40, "type": "sym", "entity": "视网膜发育异常" } ] }, { "text": "可见独眼畸形及小下颌。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "独眼畸形" }, { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "小下颌" } ] }, { "text": "耳位低,耳轮较平而界限不清,且有耳聋。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "耳位低" }, { "start_idx": 4, "end_idx": 12, "type": "sym", "entity": "耳轮较平而界限不清" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "耳聋" } ] }, { "text": "面、前额或颈背可有一个或多个血管瘤。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "面" }, { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "前额" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "颈背" }, { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "面、前额或颈背可有一个或多个血管瘤" } ] }, { "text": "第12肋骨发育不良或缺如。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "肋骨发育不良或缺如" } ] }, { "text": "80%病例有先天性心脏病,主要为室间隔缺损、动脉导管未闭及房间隔缺损等。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "消化道畸形可见结肠旋转不良、胰腺或脾组织异位等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "消化道畸形" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "结肠旋转不良" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "胰腺" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "脾组织异位" } ] }, { "text": "常见六指(趾)畸形,指甲过度凸出。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "指" }, { "start_idx": 2, "end_idx": 8, "type": "sym", "entity": "六指(趾)畸形" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "指甲过度凸出" } ] }, { "text": "30%~60%患儿有泌尿系畸形,可见多囊肾、肾盂积水、双肾及双输尿管。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "泌尿系畸形" }, { "start_idx": 18, "end_idx": 20, "type": "sym", "entity": "多囊肾" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "肾盂积水" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肾" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "双肾及双输尿管" } ] }, { "text": "男性80%有隐睾,见阴囊畸形。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "男性80%有隐睾" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "阴囊畸形" } ] }, { "text": "女性可有双角子宫、阴蒂肥大及双阴道。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "女性可有双角子宫" }, { "start_idx": 9, "end_idx": 11, "type": "sym", "entity": "阴蒂肥" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "双阴道" } ] }, { "text": "核型分析类同外周血淋巴细胞染色体检查。", "entities": [ { "start_idx": 6, "end_idx": 17, "type": "pro", "entity": "外周血淋巴细胞染色体检查" } ] }, { "text": "约占所有先天性心脏病的1%~4%。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "心脏病" } ] }, { "text": "Collett及Edwards依据体、肺循环状况将之分为四型:①Ⅰ型:有短段的肺总动脉存在并发出肺动脉分支;②Ⅱ型:无肺总动脉段,左、右肺动脉分别发自动脉干,但两者起源部分紧贴;③Ⅲ型:无肺总动脉段,左、右肺动脉分别发自动脉干,但两者起源部分远离;④Ⅳ型:称假性永存动脉干,肺循环由降主动脉发出侧支循环供应。", "entities": [ { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "体、肺循环" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "肺总动脉" }, { "start_idx": 48, "end_idx": 52, "type": "bod", "entity": "肺动脉分支" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "肺总动脉段" }, { "start_idx": 65, "end_idx": 70, "type": "bod", "entity": "左、右肺动脉" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "动脉干" }, { "start_idx": 94, "end_idx": 98, "type": "bod", "entity": "肺总动脉段" }, { "start_idx": 100, "end_idx": 105, "type": "bod", "entity": "左、右肺动脉" }, { "start_idx": 110, "end_idx": 112, "type": "bod", "entity": "动脉干" }, { "start_idx": 129, "end_idx": 135, "type": "dis", "entity": "假性永存动脉干" }, { "start_idx": 137, "end_idx": 139, "type": "bod", "entity": "肺循环" }, { "start_idx": 141, "end_idx": 144, "type": "bod", "entity": "降主动脉" } ] }, { "text": "患儿多有共同干瓣膜的异常增厚,并可伴有结节。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "干瓣膜的异常增厚" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "结节" } ] }, { "text": "30%的患儿可有共同干瓣膜的狭窄,50%患儿可伴有共同干瓣膜的反流,两者将直接影响该病的预后。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "干瓣膜的狭窄" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "干瓣膜的反流" } ] }, { "text": "65%~70%的共同干瓣膜为三瓣,四瓣畸形的发生率在9%~24%之间,6%~23%可为二瓣畸形,已有单瓣畸形的报道。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "干瓣膜" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "四瓣畸形" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "二瓣畸形" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "单瓣畸形" } ] }, { "text": "永存动脉干的冠状动脉畸形将影响外科手术的矫治。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "永存动脉干" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "冠状动脉畸形" }, { "start_idx": 15, "end_idx": 16, "type": "dep", "entity": "外科" } ] }, { "text": "13%~18%的患儿可为单支冠状动脉畸形。", "entities": [ { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "单支冠状动脉畸形" } ] }, { "text": "永存动脉干的最常见合并畸形是主动脉弓异常。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "永存动脉干" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "畸形" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "主动脉弓异常" } ] }, { "text": "右位主动脉弓、主动脉弓中断的发生率分别为33%和19%。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "右位主动脉弓、主动脉弓中断" } ] }, { "text": "此外,永存动脉干中有近12%合并有左上腔静脉残存。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "永存动脉干" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "左上腔静脉" } ] }, { "text": "其他尚可合并部分性肺静脉异位引流、三尖瓣闭锁及房室间隔缺损等。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "肺静脉异位引流" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "三尖瓣闭锁" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "房室间隔缺损" } ] }, { "text": "临床上与室间隔缺损合并动脉导管未闭或主肺动脉间隔缺损合并室间隔缺损相似。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "主肺动脉间隔缺损" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "如有共同干反流则可及舒张早期杂音,如共同干与肺动脉分支间存在压力阶差时还可闻及连续性杂音。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "共同干反流" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "舒张早期杂音" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "共同干" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "肺动脉分支" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "连续性杂音" } ] }, { "text": "对于2岁以上的患儿应常规测量肺动脉阻力,当肺动脉阻力高于8单位/平方米时不宜进行手术。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "ite", "entity": "肺动脉阻力" }, { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "肺动脉阻力" } ] }, { "text": "内科的抗心力衰竭治疗包括地高辛及利尿剂的应用;在伴有主动脉弓中断时还应临时应用前列腺素E1,以保持动脉导管开放直至手术。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "抗心力衰竭治疗" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "地高辛" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "利尿剂" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "主动脉弓中断" }, { "start_idx": 39, "end_idx": 44, "type": "dru", "entity": "前列腺素E1" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "动脉" } ] }, { "text": "手术的危险因素包括共同干瓣膜反流、合并主动脉弓中断、冠状动脉异常及手术年龄。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "干瓣膜反流" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "合并主动脉弓中断" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "冠状动脉异常" } ] }, { "text": "出生30天后肺动脉高压发生率高,患儿如果出生后100天以上手术,手术死亡率将明显增高。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "肺动脉高压" } ] }, { "text": "患儿在百日咳病程中突然发热、气急,呼吸增快与体温不成比例,严重者可出现呼吸困难、发绀。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "百日咳" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "发热" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "气急" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "呼吸增快" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "体温不成比例" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "发绀" } ] }, { "text": "肺部可闻及细湿啰音,或出现实变体征。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "细湿啰音" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "实变体征" } ] }, { "text": "剧烈咳嗽有时可造成肺泡破裂引起气胸、纵隔气肿或皮下气肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "剧烈咳嗽" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肺泡" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "气胸" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "纵隔气肿" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "皮下气肿" } ] }, { "text": "继发细菌感染者应送检痰培养及血培养。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "ite", "entity": "痰培养" }, { "start_idx": 14, "end_idx": 16, "type": "ite", "entity": "血培养" } ] }, { "text": "治疗首选红霉素,10~14天为一疗程。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "红霉素" } ] }, { "text": "必要时加用氨苄西林或利福平等。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "利福平" } ] }, { "text": "本病为小儿所特有,发生于手指或足趾,有复发倾向。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "手指" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "足趾" } ] }, { "text": "纤维结节仅发生于指或趾,手指较足趾的发病率要高,女孩略多于男孩,无家族遗传史。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "纤维结节" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "指" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "趾" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "手指" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "足趾" } ] }, { "text": "结节呈圆顶型,基底较宽,表面光滑,覆盖的皮肤呈淡红色,体积小,很少超过2cm。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "结节" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "皮肤" } ] }, { "text": "治疗以手术为主,手术切除容易,有60%的病例复发。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "手术" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "手术切除" } ] }, { "text": "多在新生儿期出现呼吸困难及发绀。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "发绀" } ] }, { "text": "体格检查可见胸部呼吸运动减弱,心率加快,心前区易听到收缩期杂音。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "体格检查" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "胸部呼吸运动减弱" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "心率加快" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "心前区易听到收缩期杂音" } ] }, { "text": "胸部X线摄片示肺野网状或颗粒状细小斑点影,偶可出现一侧肺异常透亮。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "胸部X线摄片" }, { "start_idx": 7, "end_idx": 19, "type": "sym", "entity": "肺野网状或颗粒状细小斑点影" }, { "start_idx": 21, "end_idx": 31, "type": "sym", "entity": "偶可出现一侧肺异常透亮" } ] }, { "text": "肠套叠分为原发性和继发性两种。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肠套叠" } ] }, { "text": "随着病情进展,腹痛间歇可出现淡漠、嗜睡。", "entities": [ { "start_idx": 7, "end_idx": 18, "type": "sym", "entity": "腹痛间歇可出现淡漠、嗜睡" } ] }, { "text": "体格检查,早期生命体征平稳,腹痛发作时,可听到亢进的肠鸣音。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "早期生命体征平稳" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "腹痛" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "亢进的肠鸣音" } ] }, { "text": "肠套叠初期,结肠蠕动增加,肠腔内压升高,患儿排出少量正常粪便;后期粪便中出现血迹,随之因肠缺血坏死而排暗红色血块或果酱样大便。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "结肠" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "粪便" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "粪便" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "血" } ] }, { "text": "发作间歇期触诊可有右下腹平坦空虚感,这是由于盲肠和回盲部套入横结肠至右上腹所致;此外还可触及部位不固定的包块。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "盲肠" } ] }, { "text": "梗阻时间过长的患儿可能出现脱水及菌血症,导致心动过速和发热,偶见低血容量性或感染性休克。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "梗阻" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "脱水" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "菌血症" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "心动过速" }, { "start_idx": 32, "end_idx": 42, "type": "dis", "entity": "低血容量性或感染性休克" } ] }, { "text": "若全身情况良好,可即刻行空气或液体(钡剂)灌肠。", "entities": [ { "start_idx": 12, "end_idx": 22, "type": "pro", "entity": "空气或液体(钡剂)灌肠" } ] }, { "text": "目前国内已普遍采用空气灌肠复位法,复位成功率为75%~94%。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "空气灌肠复位法" } ] }, { "text": "有的将有流出道狭窄的称为梗阻性心肌病。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "梗阻性心肌病" } ] }, { "text": "如左右心室都肥厚的称为对称性,否则称为非对称性。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "左右心室" } ] }, { "text": "一般对称性多数为非梗阻性,不对称多数为梗阻性,但也有左心室壁与室间隔肥厚,右心室壁不肥厚而左心室流出道不狭窄的,即只有不对称而无梗阻的。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "左心室壁" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "室间隔" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "右心室壁" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "左心室" } ] }, { "text": "有的患儿室间隔特别肥厚,突入到左心室腔间,尤其在主动脉瓣下,表现为左心室流出道狭窄称为特发性肥厚性主动脉瓣下狭窄。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "室间隔" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "左心室" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "主动脉" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "左心室流出道狭窄" }, { "start_idx": 43, "end_idx": 55, "type": "dis", "entity": "特发性肥厚性主动脉瓣下狭窄" } ] }, { "text": "HCM通常以常染色体显性方式遗传,目前已知多个基因与典型的家族性肥厚性心肌病有关,这些基因均编码肌节蛋白,如β肌凝蛋白重链等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "HCM" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "肥厚性心肌病" } ] }, { "text": "年小儿可表现为生长落后,心力衰竭的发生率较年长儿高。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "生长落后" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "体格检查部分病例在心尖可闻及全收缩期杂音,并向左腋下放射,此杂音是由于二尖瓣反流所致。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "心尖可闻及全收缩期杂音" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "左腋下" } ] }, { "text": "在婴儿,偶可应用心内膜心肌活体组织检查来确定病因,如线粒体肌病、糖原累积病等。", "entities": [ { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "线粒体肌病" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "糖原累积病" } ] }, { "text": "不过现今骨骼肌活体组织检查更方便,且创伤更小。", "entities": [ { "start_idx": 4, "end_idx": 12, "type": "pro", "entity": "骨骼肌活体组织检查" } ] }, { "text": "β受体阻滞剂普萘洛尔(propranolol)为本病治疗的主要药物,它减慢心率,降低心肌收缩力,从而减轻左心室流出道梗阻;且可减低心肌的张力,使氧需量减少,缓解心绞痛心绞痛;此外,普萘洛尔尚有一定的抗心律失常作用。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dru", "entity": "β受体阻滞剂普萘洛尔" }, { "start_idx": 11, "end_idx": 21, "type": "dru", "entity": "propranolol" }, { "start_idx": 37, "end_idx": 38, "type": "ite", "entity": "心率" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "心肌" }, { "start_idx": 52, "end_idx": 57, "type": "bod", "entity": "左心室流出道" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "心肌" }, { "start_idx": 80, "end_idx": 82, "type": "sym", "entity": "心绞痛" }, { "start_idx": 90, "end_idx": 93, "type": "dru", "entity": "普萘洛尔" }, { "start_idx": 100, "end_idx": 101, "type": "ite", "entity": "心律" } ] }, { "text": "其他临床上应用的选择性β受体阻滞剂有阿替洛尔(atenolol)、美托洛尔(metoprolol)等。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dru", "entity": "β受体阻滞剂" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "阿替洛尔" }, { "start_idx": 23, "end_idx": 30, "type": "dru", "entity": "atenolol" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "美托洛尔" }, { "start_idx": 38, "end_idx": 47, "type": "dru", "entity": "metoprolol" } ] }, { "text": "维拉帕米(verapamil)主要用于成人HCM患者。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "维拉帕米" }, { "start_idx": 5, "end_idx": 13, "type": "dru", "entity": "verapamil" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "HCM" } ] }, { "text": "其他手术方式有二尖瓣换置术及心尖主动脉管道,但因疗效不确切,且并发症多、在儿科均极少应用。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "二尖瓣换置术" }, { "start_idx": 14, "end_idx": 20, "type": "pro", "entity": "心尖主动脉管道" }, { "start_idx": 37, "end_idx": 38, "type": "dep", "entity": "儿科" } ] }, { "text": "心脏移植是另一治疗手段。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "心脏移植" } ] }, { "text": "目前已知此类疾病多达40余种,如神经纤维瘤病、结节性硬化症、脑面血管瘤病、色素失调症、伊藤色素减少症、面部半侧萎缩症、神经皮肤鱼鳞病、着色性干皮病、小脑视网膜血管瘤病、皮肤脑脊膜脊髓血管瘤病、黑棘皮病以及线状皮脂痣等。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "神经纤维瘤病" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "结节性硬化症" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "脑面血管瘤病" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "色素失调症" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "伊藤色素减少症" }, { "start_idx": 51, "end_idx": 57, "type": "dis", "entity": "面部半侧萎缩症" }, { "start_idx": 59, "end_idx": 65, "type": "dis", "entity": "神经皮肤鱼鳞病" }, { "start_idx": 67, "end_idx": 72, "type": "dis", "entity": "着色性干皮病" }, { "start_idx": 74, "end_idx": 82, "type": "dis", "entity": "小脑视网膜血管瘤病" }, { "start_idx": 84, "end_idx": 94, "type": "dis", "entity": "皮肤脑脊膜脊髓血管瘤病" }, { "start_idx": 96, "end_idx": 99, "type": "dis", "entity": "黑棘皮病" }, { "start_idx": 102, "end_idx": 106, "type": "dis", "entity": "线状皮脂痣" } ] }, { "text": "这些疾病多数属于常染色体显性遗传,有一个较高的、不完全的外显率。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "常染色体显性遗传" } ] }, { "text": "NFⅡ主要临床特点为双侧听神经瘤。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "NFⅡ" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "双侧听神经瘤" } ] }, { "text": "通常好发于躯干,随年龄增长有增多、扩大的趋势。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "好发于躯干" }, { "start_idx": 8, "end_idx": 21, "type": "sym", "entity": "随年龄增长有增多、扩大的趋势" } ] }, { "text": "皮肤纤维瘤和纤维软瘤主要分布于躯干、面部,也累及四肢。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "皮肤纤维瘤" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "纤维软瘤" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "躯干" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "面部" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "四肢" } ] }, { "text": "部分累及脊髓和周围神经干。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "脊髓" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "周围神经干" } ] }, { "text": "血管系统可见到肾动脉或颈动脉狭窄等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "血管系统" }, { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "肾动脉或颈动脉狭窄" } ] }, { "text": "此外,肾上腺、心、肺、消化道及纵隔等均可发生肿瘤。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "肾上腺" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "心" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肺" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "消化道" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "纵隔" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "肿瘤" } ] }, { "text": "一般肿瘤症状在青春期或青春期以后出现,如前庭及耳蜗神经症状表现为耳鸣、眩晕和听力丧失(开始时往往是单侧);邻近颅神经受损症状表现为面部疼痛、面肌抽搐、面部感觉减退以及周围性轻面瘫等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 6, "end_idx": 17, "type": "sym", "entity": "在青春期或青春期以后出现" }, { "start_idx": 20, "end_idx": 28, "type": "sym", "entity": "前庭及耳蜗神经症状" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "耳鸣" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "眩晕" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "听力丧失" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "开始时往往是单侧" }, { "start_idx": 53, "end_idx": 59, "type": "dis", "entity": "邻近颅神经受损" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "面部疼痛" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "面肌抽搐" }, { "start_idx": 75, "end_idx": 80, "type": "sym", "entity": "面部感觉减退" }, { "start_idx": 83, "end_idx": 88, "type": "sym", "entity": "周围性轻面瘫" } ] }, { "text": "异物刚进入时,患儿除表现为呛咳憋气、作呕、面色苍白、呼吸困难外,喘鸣音是特征性体征,并可见吸气性三凹征。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "呛咳憋气" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "作呕" }, { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "面色" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "面色苍白" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "呼吸" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 32, "end_idx": 34, "type": "sym", "entity": "喘鸣音" }, { "start_idx": 45, "end_idx": 50, "type": "dis", "entity": "吸气性三凹征" } ] }, { "text": "异物较大时可造成呼吸道阻塞窒息甚至死亡;异物较小可进一步进入支气管和细支气管,听诊时可发现一侧呼吸音减轻,同时,由于异物的刺激可出现肺炎体征,炎症如未能控制,还可出现肺不张,气胸、纵隔气肿等。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "呼吸道阻塞窒息" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "死亡" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "支气管" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "细支气管" }, { "start_idx": 39, "end_idx": 40, "type": "pro", "entity": "听诊" }, { "start_idx": 45, "end_idx": 51, "type": "sym", "entity": "一侧呼吸音减轻" }, { "start_idx": 66, "end_idx": 67, "type": "dis", "entity": "肺炎" }, { "start_idx": 83, "end_idx": 85, "type": "sym", "entity": "肺不张" }, { "start_idx": 87, "end_idx": 88, "type": "sym", "entity": "气胸" }, { "start_idx": 90, "end_idx": 93, "type": "sym", "entity": "纵隔气肿" } ] }, { "text": "通常分为两大类:①原发性者:包括芬兰型先天性肾病综合征、弥漫性系膜硬化、微小病变及局灶节段性硬化;②继发性者:可继发于感染(先天梅毒、先天性毒浆原虫病、先天性巨细胞包涵体病、风疹、肝炎、疟疾及艾滋病等)、汞中毒、婴儿系统性红斑狼疮、溶血尿毒综合征、甲髌综合征、Drash综合征及肾静脉血栓形成等。", "entities": [ { "start_idx": 16, "end_idx": 26, "type": "dis", "entity": "芬兰型先天性肾病综合征" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "弥漫性系膜硬化" }, { "start_idx": 36, "end_idx": 47, "type": "sym", "entity": "微小病变及局灶节段性硬化" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "感染" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "先天梅毒" }, { "start_idx": 67, "end_idx": 74, "type": "dis", "entity": "先天性毒浆原虫病" }, { "start_idx": 76, "end_idx": 85, "type": "dis", "entity": "先天性巨细胞包涵体病" }, { "start_idx": 87, "end_idx": 88, "type": "dis", "entity": "风疹" }, { "start_idx": 90, "end_idx": 91, "type": "dis", "entity": "肝炎" }, { "start_idx": 93, "end_idx": 94, "type": "dis", "entity": "疟疾" }, { "start_idx": 96, "end_idx": 98, "type": "dis", "entity": "艾滋病" }, { "start_idx": 102, "end_idx": 104, "type": "dis", "entity": "汞中毒" }, { "start_idx": 106, "end_idx": 114, "type": "dis", "entity": "婴儿系统性红斑狼疮" }, { "start_idx": 116, "end_idx": 122, "type": "dis", "entity": "溶血尿毒综合征" }, { "start_idx": 124, "end_idx": 128, "type": "dis", "entity": "甲髌综合征" }, { "start_idx": 130, "end_idx": 137, "type": "dis", "entity": "Drash综合征" }, { "start_idx": 139, "end_idx": 143, "type": "dis", "entity": "肾静脉血栓" } ] }, { "text": "免疫荧光检查病早期无Ig和补体沉着。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "免疫荧光检查病" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "Ig" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "早期无Ig和补体沉着" } ] }, { "text": "电镜示内皮细胞肿胀,上皮细胞足突融合,基膜皱缩等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "电镜" }, { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "内皮细胞肿胀" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "上皮细胞足突融合" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "基膜" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "基膜皱缩" } ] }, { "text": "出生后常见特殊的外貌,如:鼻梁低、眼距宽、低位耳、颅缝宽、前囟和后囟宽大,还常见髋、膝及肘部呈屈曲畸形。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "鼻梁低" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "眼距宽" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "低位耳" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "颅缝宽" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "前囟" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "后囟" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "前囟和后囟宽大" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "髋" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "膝" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "肘部" }, { "start_idx": 40, "end_idx": 50, "type": "sym", "entity": "髋、膝及肘部呈屈曲畸形" } ] }, { "text": "其后常见腹胀、腹水及脐疝。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "腹胀" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "腹水" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "脐疝" } ] }, { "text": "小婴儿有不能解释的肾病综合征伴外生殖器异常,则应考虑到Drash综合征。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "外生殖器" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "外生殖器异常" }, { "start_idx": 27, "end_idx": 34, "type": "dis", "entity": "Drash综合征" } ] }, { "text": "对严重低白蛋白血症,或伴低血容量表现者可输注无盐白蛋白。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "严重低白蛋白血症" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "低血容量" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "输注" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "无盐白蛋白" } ] }, { "text": "芬兰有主张自生后4周即静脉输白蛋白,维持其血浆蛋白在15g/L以上,则此时一般可无水肿且生长发育接近正常。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "静脉" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "白蛋白" }, { "start_idx": 21, "end_idx": 24, "type": "ite", "entity": "血浆蛋白" }, { "start_idx": 40, "end_idx": 42, "type": "sym", "entity": "无水肿" }, { "start_idx": 44, "end_idx": 51, "type": "sym", "entity": "生长发育接近正常" } ] }, { "text": "继发性CNS随着其病因的不同而有着不同的预后,如感染所致者,采用强有力的抗感染治疗,病情常明显好转。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "继发性CNS" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "感染" }, { "start_idx": 36, "end_idx": 40, "type": "pro", "entity": "抗感染治疗" } ] }, { "text": "因在胸片上右肺下野见一特征性新月形阴影,故又可称“弯刀综合征”。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "胸片" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "右肺" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "弯刀综合征" } ] }, { "text": "伴有房间隔缺损者,体检以房缺体征为主。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "体检" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "房缺" } ] }, { "text": "若肺循环∶体循环大于2∶1,则需手术纠正。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肺" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "手术纠正" } ] }, { "text": "右上肺静脉异位引流伴静脉窦型房缺患儿,术后30%~40%可发生病态窦房结综合征。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "右上肺静脉异位引流" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "静脉窦型房缺" }, { "start_idx": 31, "end_idx": 38, "type": "dis", "entity": "病态窦房结综合征" } ] }, { "text": "几乎全部出现贫血症状,可有面色苍白、乏力、心率增快,心前区可闻及吹风样收缩期杂音。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "出现贫血症状" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "面色苍白" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "乏力" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "心率增快" }, { "start_idx": 26, "end_idx": 39, "type": "sym", "entity": "心前区可闻及吹风样收缩期杂音" } ] }, { "text": "严重者可出现心力衰竭和呼吸衰竭。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "呼吸衰竭" } ] }, { "text": "注意排除继发性肺含铁血黄素沉着症,如继发于二尖瓣狭窄以及任何原因引起的左心衰竭,或肺内毛细血管压力长期增高的心脏病患者及结缔组织疾病如结节性多发性动脉炎、类风湿病、过敏性紫癜。", "entities": [ { "start_idx": 4, "end_idx": 15, "type": "dis", "entity": "继发性肺含铁血黄素沉着症" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "左心衰竭" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "肺" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "毛细血管" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "心脏病" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "结缔组织" }, { "start_idx": 67, "end_idx": 75, "type": "dis", "entity": "结节性多发性动脉炎" }, { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "类风湿病" }, { "start_idx": 82, "end_idx": 86, "type": "dis", "entity": "过敏性紫癜" } ] }, { "text": "本病尚有两种特殊类型:①Goodpasture综合征:是一种免疫复合物病,具体病因不明,病变同时累及肺和肾脏,病情严重,可见发热、咳嗽、咯血,常发生呼吸困难,有显著贫血,尿中有蛋白质、红细胞、管型。", "entities": [ { "start_idx": 12, "end_idx": 25, "type": "dis", "entity": "Goodpasture综合征" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "肺" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "肾脏" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "发热" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "咳嗽" }, { "start_idx": 68, "end_idx": 69, "type": "sym", "entity": "咯血" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 80, "end_idx": 83, "type": "sym", "entity": "显著贫血" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "蛋白质" }, { "start_idx": 92, "end_idx": 94, "type": "bod", "entity": "红细胞" }, { "start_idx": 96, "end_idx": 97, "type": "bod", "entity": "管型" } ] }, { "text": "重症或急性期以静脉给药为主,如甲基泼尼松龙、氢化可的松、地塞米松等,病情好转后减量口服,以泼尼松维持,疗程至少3个月,一般为半年至1年,反复发作者可适当延长。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "dru", "entity": "甲基泼尼松龙" }, { "start_idx": 22, "end_idx": 26, "type": "dru", "entity": "氢化可的松" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "地塞米松" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "泼尼松" } ] }, { "text": "肾上腺皮质激素治疗无效者可试用免疫抑制剂,如硫唑嘌呤、环磷酰胺,疗程一般3个月,可与肾上腺皮质激素联合应用。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 42, "end_idx": 48, "type": "dru", "entity": "肾上腺皮质激素" } ] }, { "text": "输血和铁剂治疗虽可改善贫血,但由于可能增加肺内铁沉积,故应慎用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "输血" }, { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "铁剂治疗" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "贫血" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "肺" } ] }, { "text": "代谢性碱中毒无特征性临床表现。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "代谢性碱中毒" } ] }, { "text": "轻度代碱可无明显症状,重症者表现为呼吸抑制,精神萎靡。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "呼吸抑制" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "精神萎靡" } ] }, { "text": "国外学者认为心肌炎的发生率通常被低估。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "心肌炎" } ] }, { "text": "在特发性扩张性心肌病成人患者,心肌炎的发生率为3%~63%。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "心肌炎" } ] }, { "text": "有时病毒可同时侵犯其他系统如,肌肉、大脑等,并出现相应症状及体征。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "病毒" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肌肉" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "大脑" } ] }, { "text": "现分述病毒性心肌炎各期主要症状、体征。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "病毒性心肌炎" } ] }, { "text": "轻型症状以乏力为主,其次有多汗、苍白、心悸、气短、胸闷、头晕、精神食欲缺乏等。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "苍白" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "心悸" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "气短" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "胸闷" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "头晕" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "精神食欲缺乏" } ] }, { "text": "起病较急,除前述症状外,乏力突出,年长儿常诉心前区疼痛。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "心前区疼痛" } ] }, { "text": "起病较急者可伴恶心、呕吐。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "恶心" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "呕吐" } ] }, { "text": "患儿极度乏力、头晕、烦躁、呕吐、心前区疼痛或压迫感。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "乏力" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "头晕" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "烦躁" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "呕吐" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "心前区疼痛或压迫感" } ] }, { "text": "有的发生急性左心力衰竭、肺水肿。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "急性左心力衰竭" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肺" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "肺水肿" } ] }, { "text": "若血清中CK-MB明显增高则多提示心肌受累,与CK总活性相比,对判断心肌损伤有较高的特异性和敏感性。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "血清" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "心肌" } ] }, { "text": "处理原则与一般心力衰竭及心源性休克相似,包括洋地黄的应用、血管扩张剂、磷酸二酯酶抑制剂、利尿剂及扩容纠正酸中毒等,但在洋地黄应用时应注意在病毒性心肌炎急性期,心肌对洋地黄敏感,易出现毒性反应,应避免快饱和,用药剂量也应适当减少。", "entities": [ { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "心肌" } ] }, { "text": "室上性心动过速洋地黄治疗有效,室性心动过速可用利多卡因或胺碘酮静滴。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "室上性心动过速" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "洋地黄" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "室性心动过速" } ] }, { "text": "如室性心律失常虽经积极治疗仍快速进展至室性纤颤(这种情况在小婴儿更易发生)应即刻予以直流电复律。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "室性心律失常" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "室性纤颤" }, { "start_idx": 42, "end_idx": 46, "type": "pro", "entity": "直流电复律" } ] }, { "text": "严重的二尖瓣梗阻将出现外周循环灌注不良,脉搏微弱。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "严重的二尖瓣梗阻" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "外周循环灌注不良" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "脉搏" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "脉搏微弱" } ] }, { "text": "与风湿性心瓣膜病相反,绝大多数的患儿第一心音柔和,无二尖瓣开放音。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "风湿性心瓣膜病" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "第一心音柔和" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "无二尖瓣开放音" } ] }, { "text": "二维超声心动图及多普勒超声心动图可清晰显示肺静脉解剖及血流正常,肺静脉回流至副房并通过其与真左房间隔膜的狭窄开口进入真左房。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "二维超声心动图" }, { "start_idx": 8, "end_idx": 15, "type": "pro", "entity": "多普勒超声心动图" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺静脉" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "肺静脉" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "副房" }, { "start_idx": 45, "end_idx": 50, "type": "bod", "entity": "真左房间隔膜" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "真左房" } ] }, { "text": "彩色多普勒超声心动图可提供二尖瓣舒张期正向湍流的彩色直观显示,并可对狭窄或反流的位置进行定位。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "彩色多普勒超声心动图" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "二尖瓣" } ] }, { "text": "有时尚需进行吸氧或肺血管扩张试验,以判断肺血管床病理改变。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "吸氧" }, { "start_idx": 9, "end_idx": 13, "type": "pro", "entity": "肺血管扩张" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "肺血管床" } ] }, { "text": "当患儿有明显肺动脉高压表现时应进行心血管造影。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "心血管造影" } ] }, { "text": "患儿高热,中毒症状明显,可伴有多种器官损害,如肺炎、肝炎、肾炎、心肌炎及关节炎等,甚至发生循环衰竭。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "高热" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "中毒症状明显" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "伴有多种器官损害" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "肺炎" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "肝炎" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "肾炎" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "心肌炎" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "关节炎" } ] }, { "text": "皮疹迅速融合成片,成水肿样,其上形成水疱、大疱,疱破后形成糜烂面,似Ⅱ度烧伤,渗出大量浆液或出血性浆液,若无继发感染,1~4周后结痂脱屑,有色素沉着,不留瘢痕。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "皮疹" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "水肿" }, { "start_idx": 18, "end_idx": 31, "type": "sym", "entity": "水疱、大疱,疱破后形成糜烂面" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "Ⅱ度烧伤" }, { "start_idx": 39, "end_idx": 50, "type": "sym", "entity": "渗出大量浆液或出血性浆液" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "结痂脱屑" }, { "start_idx": 69, "end_idx": 73, "type": "sym", "entity": "有色素沉着" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "不留瘢痕" } ] }, { "text": "若存活期长,还易发生肿瘤和自身免疫性疾病。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "自身免疫性疾病" } ] }, { "text": "也可见全身感染,如脓毒血症、败血症、脑膜炎和骨、关节炎。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "全身感染" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "脓毒血症" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "败血症" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "骨、关节炎" } ] }, { "text": "B细胞淋巴瘤最多见,T细胞和霍奇金病也可发生。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "B细胞淋巴瘤" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "T细胞" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "霍奇金病" } ] }, { "text": "临床发现伴发淋巴瘤的PID常为SCID、嘌呤核苷磷酸化酶(PNP)缺乏、XLA、X连锁淋巴组织增生症、高IgM血症(HM)、IgA缺乏症、IgG亚类缺陷、常见变异型免疫缺陷病(CVID)、湿疹血小板减少伴免疫缺陷(WAS)、毛细血管扩张共济失调综合征(AT)以及胸腺发育不全等。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "PID" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "SCID" }, { "start_idx": 20, "end_idx": 34, "type": "dis", "entity": "嘌呤核苷磷酸化酶(PNP)缺乏" }, { "start_idx": 40, "end_idx": 49, "type": "dis", "entity": "X连锁淋巴组织增生症" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "高IgM血症" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "HM" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "IgA缺乏症" }, { "start_idx": 69, "end_idx": 75, "type": "dis", "entity": "IgG亚类缺陷" }, { "start_idx": 79, "end_idx": 86, "type": "dis", "entity": "变异型免疫缺陷病" }, { "start_idx": 88, "end_idx": 91, "type": "dis", "entity": "CVID" }, { "start_idx": 94, "end_idx": 100, "type": "dis", "entity": "湿疹血小板减少" }, { "start_idx": 102, "end_idx": 105, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 107, "end_idx": 109, "type": "dis", "entity": "WAS" }, { "start_idx": 112, "end_idx": 124, "type": "dis", "entity": "毛细血管扩张共济失调综合征" }, { "start_idx": 126, "end_idx": 127, "type": "dis", "entity": "AT" }, { "start_idx": 131, "end_idx": 136, "type": "dis", "entity": "胸腺发育不全" } ] }, { "text": "也有伴发白血病、胶质瘤、肝胆管瘤以及横纹肌肉瘤的报道。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "白血病" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "胶质瘤" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "肝胆管瘤" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "有一定抗体反应者可考虑给予死疫苗接种,细胞免疫缺陷病人不宜接种存活的疫苗如口服灰髓炎疫苗,以免感染患儿。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "抗体" }, { "start_idx": 34, "end_idx": 35, "type": "dru", "entity": "疫苗" }, { "start_idx": 39, "end_idx": 43, "type": "dru", "entity": "灰髓炎疫苗" } ] }, { "text": "已确诊为T细胞缺陷患儿不宜输新鲜血制品,以防发生移植物抗宿主反应(GVHR),必需输血或新鲜血制品时,应先用射线(2000~3000rad)处理,血制品还要严格筛查CMV,以防血源性CMV感染。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "T细胞缺陷" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "输新鲜血制品" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "移植物抗宿主反应" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "GVHR" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "输血" }, { "start_idx": 44, "end_idx": 48, "type": "dru", "entity": "新鲜血制品" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "射线" }, { "start_idx": 73, "end_idx": 75, "type": "dru", "entity": "血制品" }, { "start_idx": 80, "end_idx": 84, "type": "pro", "entity": "筛查CMV" }, { "start_idx": 88, "end_idx": 95, "type": "dis", "entity": "血源性CMV感染" } ] }, { "text": "大约80%PID患儿伴有不同程度IgG或其他抗体缺乏,因此补充IgG(IVIG)是最常见的替代治疗措施。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "PID" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "IgG" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "抗体缺乏" }, { "start_idx": 29, "end_idx": 33, "type": "pro", "entity": "补充IgG" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "IVIG" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "替代治疗" } ] }, { "text": "其他替代治疗包括特异性免疫血清,输白细胞,细胞因子(转移因子,胸腺素)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "替代治疗" }, { "start_idx": 8, "end_idx": 14, "type": "dru", "entity": "特异性免疫血清" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "输白细胞" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "细胞因子" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "转移因子" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "胸腺素" } ] }, { "text": "约50%的LQTS为家族性:Romano-Ward综合征为常染色体显性遗传,可伴有先天性耳聋;Jirvell-Lange-Nielsen综合征为常染色体隐性遗传。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "LQTS" }, { "start_idx": 14, "end_idx": 27, "type": "dis", "entity": "Romano-Ward综合征" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "染色体" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "先天性耳聋" }, { "start_idx": 47, "end_idx": 70, "type": "dis", "entity": "Jirvell-Lange-Nielsen综合征" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "染色体" } ] }, { "text": "遗传学研究显示心脏的钾、钠通道突变。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心脏" } ] }, { "text": "药物可直接(terfenadine,阿司咪唑)或通过抑制代谢(红霉素,酮康唑)来延长QT间期。", "entities": [ { "start_idx": 6, "end_idx": 16, "type": "dru", "entity": "terfenadine" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "阿司咪唑" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "红霉素" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "酮康唑" } ] }, { "text": "LQTS的临床表现最常见的为晕厥发作,多由运动或惊吓引起。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "LQTS" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "晕厥发作" } ] }, { "text": "患儿也可表现为抽搐、前晕厥及心悸,约10%的患儿一开始即表现为心搏骤停。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "抽搐" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "晕厥" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "心悸" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "心搏骤停" } ] }, { "text": "LQTS的诊断主要基于心电图及临床标准。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "LQTS" }, { "start_idx": 11, "end_idx": 13, "type": "pro", "entity": "心电图" } ] }, { "text": "LQTS的治疗包括β受体阻滞剂,其剂量为能降低运动时的心率反应为度。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "LQTS" }, { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "β受体阻滞剂" }, { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "心率" } ] }, { "text": "如仍有症状且心率慢者可安置起搏器。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "心率" }, { "start_idx": 13, "end_idx": 15, "type": "equ", "entity": "起搏器" } ] }, { "text": "手术切除左星状交感神经节也是治疗方法之一。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "pro", "entity": "手术切除左星状交感神经节" } ] }, { "text": "引起发热的原因可分为感染性与非感染性两方面。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "发热" } ] }, { "text": "由下丘脑所调控的体内正常体温调节平衡会由于下列因素而发生改变:感染、疫苗接种、生物学因素(如粒细胞-巨噬细胞集落刺激因子、干扰素、白细胞介素、肿瘤坏死因子)、组织损伤(如血管闭塞、肺栓塞、外伤、肌内注射、烧伤)、恶性肿瘤(白血病、淋巴肿瘤、肝癌、转移瘤)、药物(如药物热、可卡因、两性霉素B)、风湿性疾病(如系统性红斑狼疮、类风湿关节炎)、炎症性疾病(如炎性结肠病)、肉芽肿病(如结节病)、内分泌疾病(如甲状腺毒症、嗜铬细胞瘤)、代谢性疾病(如痛风、尿毒症、Ⅰ型高脂血症)以及原因不明或知之甚少的病变(如家族性地中海热)。", "entities": [ { "start_idx": 46, "end_idx": 53, "type": "bod", "entity": "粒细胞-巨噬细胞" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "干扰素" }, { "start_idx": 65, "end_idx": 69, "type": "bod", "entity": "白细胞介素" }, { "start_idx": 71, "end_idx": 76, "type": "bod", "entity": "肿瘤坏死因子" } ] }, { "text": "发热可增高颅内压,使大脑皮质过度兴奋(烦躁、头痛或高热惊厥)或高度抑制,对6个月~5岁年龄组的儿童增加良性热性惊厥发生的危险性,而对有原发癫痫的患儿则会增加其癫痫的发作次数。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "发热" }, { "start_idx": 2, "end_idx": 62, "type": "sym", "entity": "可增高颅内压,使大脑皮质过度兴奋(烦躁、头痛或高热惊厥)或高度抑制,对6个月~5岁年龄组的儿童增加良性热性惊厥发生的危险性" }, { "start_idx": 67, "end_idx": 70, "type": "dis", "entity": "原发癫痫" }, { "start_idx": 75, "end_idx": 85, "type": "sym", "entity": "会增加其癫痫的发作次数" } ] }, { "text": "发热时消化道分泌减少,消化酶活力降低,胃肠运动缓慢,可引起食欲缺乏、腹胀和便秘等症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "发热" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "消化道" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "消化酶" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "胃肠" }, { "start_idx": 3, "end_idx": 41, "type": "sym", "entity": "消化道分泌减少,消化酶活力降低,胃肠运动缓慢,可引起食欲缺乏、腹胀和便秘等症状" } ] }, { "text": "新生儿可能是社区获得性感染或新生儿脓毒败血症。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "社区获得性感染" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "新生儿脓毒败血症" } ] }, { "text": "3个月以内婴儿的发热常提示严重细菌感染性疾病(约10%~15%)或自限性非特异性病毒感染。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "发热" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "严重细菌感染性疾病" }, { "start_idx": 33, "end_idx": 43, "type": "dis", "entity": "自限性非特异性病毒感染" } ] }, { "text": "前者包括脓毒症、脑膜炎、泌尿道感染、胃肠炎、骨关节炎和化脓性关节炎。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "脓毒症" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "泌尿道感染" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "胃肠炎" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "骨关节炎" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "化脓性关节炎" } ] }, { "text": "约5%的发热婴儿有菌血症,其病原包括李斯特菌、β链球菌(可导致新生儿后期脓毒症、脑膜炎)以及社区获得性病原菌,如沙门菌属、大肠埃希菌、脑膜炎奈瑟菌、肺炎链球菌、b型流感嗜血杆菌和金黄色葡萄球菌。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "发热" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "菌血症" }, { "start_idx": 18, "end_idx": 21, "type": "mic", "entity": "李斯特菌" }, { "start_idx": 23, "end_idx": 26, "type": "mic", "entity": "β链球菌" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "脓毒症" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 46, "end_idx": 53, "type": "mic", "entity": "社区获得性病原菌" }, { "start_idx": 56, "end_idx": 59, "type": "mic", "entity": "沙门菌属" }, { "start_idx": 61, "end_idx": 65, "type": "mic", "entity": "大肠埃希菌" }, { "start_idx": 67, "end_idx": 72, "type": "mic", "entity": "脑膜炎奈瑟菌" }, { "start_idx": 74, "end_idx": 78, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 80, "end_idx": 87, "type": "mic", "entity": "b型流感嗜血杆菌" }, { "start_idx": 89, "end_idx": 95, "type": "mic", "entity": "金黄色葡萄球菌" } ] }, { "text": "此外,这个年龄组还可能发生中耳炎、肺炎、脐炎、乳腺炎和皮肤、软组织感染。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "中耳炎" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "肺炎" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "脐炎" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "乳腺炎" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "皮肤" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "软组织" } ] }, { "text": "冬季常见呼吸道合胞病毒和流感病毒感染,肠道病毒感染多发生在夏秋季节。", "entities": [ { "start_idx": 4, "end_idx": 17, "type": "dis", "entity": "呼吸道合胞病毒和流感病毒感染" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "肠道病毒感染" } ] }, { "text": "病因包括感染性疾病、结缔组织病、恶性肿瘤和其他疾病,其中前三类疾病为FUO的主要病因。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "可由细菌、病毒、支原体、立克次体、螺旋体、寄生虫感染所致。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "细菌" }, { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "病毒" }, { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "支原体" }, { "start_idx": 12, "end_idx": 15, "type": "mic", "entity": "立克次体" }, { "start_idx": 17, "end_idx": 19, "type": "mic", "entity": "螺旋体" }, { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "寄生虫" } ] }, { "text": "传染性单核细胞增多症、感染性心内膜炎,以及病程迁延的普通病毒感染性疾病、肝炎、真菌病等也是儿童发热待查的常见病因。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "传染性单核细胞增多症" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 26, "end_idx": 34, "type": "dis", "entity": "普通病毒感染性疾病" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "肝炎" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "真菌病" } ] }, { "text": "此外应注意慢性咽炎、扁桃体炎或鼻窦炎、淋巴结炎引起的长期低热。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "慢性咽炎" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "扁桃体炎" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "淋巴结炎" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "低热" } ] }, { "text": "约有3%儿童由隐匿性细菌性肺炎发展为细菌性脑膜炎。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "隐匿性细菌性肺炎" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "细菌性脑膜炎" } ] }, { "text": "在流行地区,应考虑伤寒、副伤寒、布鲁菌病、疟疾、血吸虫病、肺吸虫病、钩端螺旋体病及旋毛虫病等。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "伤寒" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "副伤寒" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "布鲁菌病" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "疟疾" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "血吸虫病" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "肺吸虫病" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "钩端螺旋体病" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "旋毛虫病" } ] }, { "text": "结缔组织疾病中少年类风湿关节炎、系统性红斑狼疮为其主要病因。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "结缔组织疾病" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "少年类风湿关节炎" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "系统性红斑狼疮" } ] }, { "text": "川畸病、坏死性肉芽肿性血管炎较为常见。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "川畸病" }, { "start_idx": 4, "end_idx": 13, "type": "dis", "entity": "坏死性肉芽肿性血管炎" } ] }, { "text": "其他包括皮肌炎、结节性脂膜炎等。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "结节性脂膜炎" } ] }, { "text": "小于3岁儿童FUO应主要考虑感染性疾病、先天性疾病及恶性肿瘤。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "先天性疾病" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "6岁以下儿童应考虑呼吸道或泌尿生殖系统感染、局部感染(脓肿、骨髓炎)、幼年类风湿关节炎。", "entities": [ { "start_idx": 9, "end_idx": 20, "type": "dis", "entity": "呼吸道或泌尿生殖系统感染" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "脓肿" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "骨髓炎" }, { "start_idx": 35, "end_idx": 42, "type": "dis", "entity": "幼年类风湿关节炎" } ] }, { "text": "在发热伴瘀点性皮疹中2%~8%为严重的细菌性感染,最常见的是奈瑟脑膜炎球菌。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "瘀点性皮疹" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "细菌性感染" }, { "start_idx": 30, "end_idx": 36, "type": "mic", "entity": "奈瑟脑膜炎球菌" } ] }, { "text": "发热伴疱疹多见于肺炎链球菌、链球菌、疟疾和立克次体感染的患儿。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 14, "end_idx": 16, "type": "mic", "entity": "链球菌" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "疟疾" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "立克次体感染" } ] }, { "text": "发热伴淋巴结肿大,可见于传染性单核细胞增多症、白血病、恶性淋巴瘤、转移癌和淋巴结结核等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "淋巴结肿大" }, { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "传染性单核细胞增多症" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "白血病" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "恶性淋巴瘤" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "转移癌" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "淋巴结结核" } ] }, { "text": "发热伴肝、脾肿大,可见于传染性单核细胞增多症、疟疾、黑热病、急性血吸虫病、结缔组织疾病、白血病及恶性淋巴瘤等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "肝、脾肿大" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "传染性单核细胞增多症" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "疟疾" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "黑热病" }, { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "急性血吸虫病" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "结缔组织疾病" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "白血病" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "恶性淋巴瘤" } ] }, { "text": "发热患儿长时间无汗提示脱水、中枢性或肾性尿崩症、外胚层汗腺发育不良、家族性自主神经功能异常、阿托品中毒等。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "脱水" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "中枢性或肾性尿崩症" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "外胚层汗腺发育不良" }, { "start_idx": 34, "end_idx": 44, "type": "sym", "entity": "家族性自主神经功能异常" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "阿托品中毒" } ] }, { "text": "发热伴眼睛发红、流泪可能是结缔组织病的体征,尤其是结节性多发性动脉炎。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "眼睛发红" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "流泪" }, { "start_idx": 25, "end_idx": 33, "type": "dis", "entity": "结节性多发性动脉炎" } ] }, { "text": "葡萄膜炎提示结节病、幼年类风湿关节炎、系统性红斑狼疮、川崎病、Behçet综合征和脉管炎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "葡萄膜炎" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "结节病" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "幼年类风湿关节炎" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "川崎病" }, { "start_idx": 31, "end_idx": 39, "type": "dis", "entity": "Behçet综合征" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "脉管炎" } ] }, { "text": "视网膜脉络膜炎提示巨细胞病毒感染、弓形虫病或梅毒。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "视网膜脉络膜炎" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "巨细胞病毒感染" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "弓形虫病" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "梅毒" } ] }, { "text": "眼球突出则有可能为眶内肿瘤、甲状腺毒症、转移肿瘤(神经母细胞瘤)、眶内感染、坏死性肉芽肿性血管炎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "眼球突出" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "眶内肿瘤" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "甲状腺毒症" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "转移肿瘤" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "眶内感染" }, { "start_idx": 38, "end_idx": 47, "type": "dis", "entity": "坏死性肉芽肿性血管炎" } ] }, { "text": "发热伴瞳孔不能收缩则提示下丘脑功能不全。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "发热" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "瞳孔不能收缩" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "下丘脑功能不全" } ] }, { "text": "口腔白色念珠菌感染是各种免疫系统疾病的征兆。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "口腔白色念珠菌感染" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "免疫系统疾病" } ] }, { "text": "反复寒战伴高热在各种病原引起的败血症中常见,尤其是与肾脏疾病、肝胆疾病、心内膜炎、疟疾、布鲁菌病、鼠咬热或局灶性多发性脓肿有关的败血症中多见。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "反复寒战" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "高热" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "败血症" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "肾脏疾病" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "肝胆疾病" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "疟疾" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "布鲁菌病" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "鼠咬热" }, { "start_idx": 53, "end_idx": 60, "type": "dis", "entity": "局灶性多发性脓肿" }, { "start_idx": 64, "end_idx": 66, "type": "dis", "entity": "败血症" } ] }, { "text": "骨骼的点状软化灶提示骨髓炎或骨髓的肿瘤性浸润。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "骨骼" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "骨骼的点状软化灶" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "骨髓炎" }, { "start_idx": 14, "end_idx": 21, "type": "dis", "entity": "骨髓的肿瘤性浸润" } ] }, { "text": "斜方肌上方的软化提示膈下脓肿,全身肌肉软弱提示皮肌炎、旋毛虫病、多发性动脉炎、川崎病、支原体或虫媒病毒感染。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "斜方肌上方的软化" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "膈下脓肿" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "全身肌肉软弱" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "旋毛虫病" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "多发性动脉炎" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "川崎病" }, { "start_idx": 43, "end_idx": 52, "type": "dis", "entity": "支原体或虫媒病毒感染" } ] }, { "text": "如隐血试验阳性则提示肉芽肿性结肠炎或溃疡性结肠炎。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "ite", "entity": "隐血试验阳性" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "肉芽肿性结肠炎" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "溃疡性结肠炎" } ] }, { "text": "深反射亢进提示不明原因发热的病因可能是甲状腺毒症。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "深反射亢进" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "甲状腺毒症" } ] }, { "text": "血氧饱和度测定在小婴儿隐匿性细菌性肺炎的诊断中至关重要,由于婴幼儿肺炎中3%缺乏典型的呼吸道症状及肺部体征。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "ite", "entity": "血氧饱和度测定" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "隐匿性细菌性肺炎" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "呼吸道症状" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "肺部体征" } ] }, { "text": "对不明原因发热不伴其他局部症状、体征的患者,胃肠道X线检查对诊断炎性肠病有意义。", "entities": [ { "start_idx": 22, "end_idx": 28, "type": "ite", "entity": "胃肠道X线检查" } ] }, { "text": "骨髓检查有助于诊断白血病、转移瘤、分枝杆菌、真菌或寄生虫感染及组织细胞增多症、嗜红细胞现象。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "骨髓检查" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "白血病" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "转移瘤" }, { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "分枝杆菌" }, { "start_idx": 22, "end_idx": 23, "type": "mic", "entity": "真菌" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "寄生虫感染" }, { "start_idx": 31, "end_idx": 37, "type": "dis", "entity": "组织细胞增多症" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "嗜红细胞现象" } ] }, { "text": "血清学检查(包括抗链球菌溶血素“O”测定、黏蛋白、二苯胺、类风湿因子、肥达反应、冷凝集试验、嗜异性凝集试验、甲胎蛋白测定、补体结合试验、血凝抑制试验、抗核抗体、梅毒凝集反应、酶的测定等)有助于诊断风湿热、传染性单核细胞增多症、巨细胞病毒感染、弓形虫病、沙门菌病、兔热病、布鲁菌病、钩端螺旋体病,有时对幼年类风湿关节炎、恶性肿瘤也有诊断意义。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "血清学检查" }, { "start_idx": 8, "end_idx": 19, "type": "ite", "entity": "抗链球菌溶血素“O”测定" }, { "start_idx": 21, "end_idx": 23, "type": "ite", "entity": "黏蛋白" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "二苯胺" }, { "start_idx": 29, "end_idx": 33, "type": "ite", "entity": "类风湿因子" }, { "start_idx": 35, "end_idx": 38, "type": "ite", "entity": "肥达反应" }, { "start_idx": 40, "end_idx": 44, "type": "ite", "entity": "冷凝集试验" }, { "start_idx": 46, "end_idx": 52, "type": "ite", "entity": "嗜异性凝集试验" }, { "start_idx": 54, "end_idx": 59, "type": "ite", "entity": "甲胎蛋白测定" }, { "start_idx": 61, "end_idx": 66, "type": "ite", "entity": "补体结合试验" }, { "start_idx": 68, "end_idx": 73, "type": "ite", "entity": "血凝抑制试验" }, { "start_idx": 75, "end_idx": 78, "type": "ite", "entity": "抗核抗体" }, { "start_idx": 80, "end_idx": 85, "type": "ite", "entity": "梅毒凝集反应" }, { "start_idx": 87, "end_idx": 90, "type": "ite", "entity": "酶的测定" }, { "start_idx": 98, "end_idx": 100, "type": "dis", "entity": "风湿热" }, { "start_idx": 102, "end_idx": 111, "type": "dis", "entity": "传染性单核细胞增多症" }, { "start_idx": 113, "end_idx": 119, "type": "dis", "entity": "巨细胞病毒感染" }, { "start_idx": 121, "end_idx": 124, "type": "dis", "entity": "弓形虫病" }, { "start_idx": 126, "end_idx": 129, "type": "dis", "entity": "沙门菌病" }, { "start_idx": 131, "end_idx": 133, "type": "dis", "entity": "兔热病" }, { "start_idx": 135, "end_idx": 138, "type": "dis", "entity": "布鲁菌病" }, { "start_idx": 140, "end_idx": 145, "type": "dis", "entity": "钩端螺旋体病" }, { "start_idx": 150, "end_idx": 157, "type": "dis", "entity": "幼年类风湿关节炎" }, { "start_idx": 159, "end_idx": 162, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "泌尿系感染中核素锝-99扫描对肾盂肾炎的确诊率为60%~80%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "泌尿系感染" }, { "start_idx": 6, "end_idx": 13, "type": "pro", "entity": "核素锝-99扫描" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "肾盂肾炎" } ] }, { "text": "超声心动有助于亚急性感染性心内膜炎的诊断。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "超声心动" }, { "start_idx": 7, "end_idx": 16, "type": "dis", "entity": "亚急性感染性心内膜炎" } ] }, { "text": "超声波检查可分辨腹腔内肝脓肿、膈下脓肿、盆腔脓肿和脾脏脓肿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "超声波检查" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "腹腔内肝脓肿" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "膈下脓肿" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "盆腔脓肿" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "脾脏脓肿" } ] }, { "text": "前者用30%乙醇擦浴、温水棉球(非乙醇)擦浴,可用来降低由于感染而发热或外界环境所导致的体温过高(如热休克)。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dru", "entity": "乙醇" } ] }, { "text": "对乙酰氨基酚10~15mg/kg,每4小时一次,很少有不良反应发生。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "对乙酰氨基酚" } ] }, { "text": "布洛芬5~10mg/kg,6~8小时一次,其退热持续时间长,安全有效,不良反应少。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "布洛芬" } ] }, { "text": "致死性粒细胞缺乏为其最严重不良反应。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "致死性粒细胞缺乏" } ] }, { "text": "以下肢大关节炎为主要表现者常误诊为JIA少关节型。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "下肢大关节炎" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "JIA少关节型" } ] }, { "text": "JAS以年长儿居多,鉴于相当多的JAS病人伴有周围关节病变,因而也时常称为脊柱关节病综合征。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "JAS" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "JAS" }, { "start_idx": 37, "end_idx": 44, "type": "dis", "entity": "脊柱关节病综合征" } ] }, { "text": "骶髂关节炎是JAS的特征性病变,但它可能在周围关节病发生后很长时间才得到证实。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "骶髂关节炎" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "JAS" } ] }, { "text": "国内第五届儿科免疫学术会议提出为了早期诊断JAS,建议将JRA少关节Ⅱ型病例中的年长男孩,伴肌腱附着处炎症,HLA-B27阳性,下肢关节炎为主,早期侵犯髋关节,有强直性脊柱炎家族史的患儿诊断为早期JAS,一旦有骶髂关节炎证据时即可确诊JAS。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "JAS" }, { "start_idx": 28, "end_idx": 35, "type": "dis", "entity": "JRA少关节Ⅱ型" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "肌腱附着处炎症" }, { "start_idx": 54, "end_idx": 62, "type": "dis", "entity": "HLA-B27阳性" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "下肢关节炎" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "髋关节" }, { "start_idx": 81, "end_idx": 86, "type": "dis", "entity": "强直性脊柱炎" }, { "start_idx": 105, "end_idx": 109, "type": "dis", "entity": "骶髂关节炎" }, { "start_idx": 117, "end_idx": 119, "type": "dis", "entity": "JAS" } ] }, { "text": "在以下几个方面JAS不同于JRA:即典型的骶髂关节炎,腰骶部病变,年长男孩占绝大多数,有家族史特征,RF阴性,HLA-B27阳性等。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "JAS" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "JRA" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "骶髂关节炎" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "腰骶部病变" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "RF阴性" }, { "start_idx": 55, "end_idx": 63, "type": "sym", "entity": "HLA-B27阳性" } ] }, { "text": "骶髂关节炎是明确诊断JAS的关键条件。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "骶髂关节炎" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "JAS" } ] }, { "text": "JAS早期可能发现因腰骶关节病变所致腰椎前突消失,限制了脊柱下部前弯(Schober征阳性)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "JAS" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "腰骶关节病变" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "腰椎前突消失" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "限制了脊柱下部前弯" }, { "start_idx": 35, "end_idx": 44, "type": "sym", "entity": "Schober征阳性" } ] }, { "text": "若有肋椎关节病变会使胸部扩张度减小。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "肋椎关节病变" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "胸部扩张度减小" } ] }, { "text": "成人AS病人长期发作后可能发生主动脉炎或主动脉扩张,JAS主动脉病变发生率低于成年人,偶尔有报告JAS出现主动脉瓣关闭不全。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "AS" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "主动脉炎" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "主动脉扩张" }, { "start_idx": 26, "end_idx": 33, "type": "dis", "entity": "JAS主动脉病变" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "JAS" }, { "start_idx": 53, "end_idx": 60, "type": "sym", "entity": "主动脉瓣关闭不全" } ] }, { "text": "放射影像改变的特征是骶髂关节面硬化、糜烂或关节间隙增宽,继而发展到关节间隙变窄和僵直。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "放射影像" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "关节间隙变窄和僵直" } ] }, { "text": "CT和MRI扫描会比普通X光片更早发现骶髂关节炎,MRI能更为敏感地发现慢性炎症。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "CT和MRI扫描" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "X光片" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "骶髂关节炎" }, { "start_idx": 25, "end_idx": 27, "type": "pro", "entity": "MRI" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "慢性炎症" } ] }, { "text": "在AS晚期X线还可以发现韧带骨赘形成,关节突融合、形成“竹节样”脊柱。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "AS晚期" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "X线" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "韧带骨赘形成" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "关节突融合" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "形成“竹节样”脊柱" } ] }, { "text": "多数人认为HLA-B27阳性仍是早期鉴别诊断幼年类风湿性关节炎与JAS的重要线索。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "sym", "entity": "HLA-B27阳性" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "JAS" } ] }, { "text": "有些JAS病人对吲哚美辛治疗反应良好。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "JAS" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "吲哚美辛" } ] }, { "text": "有人用1-1羟化维生素D3治疗18例JAS,发现该药可降低CD4/CD8比例,血清IgG下降,骨质疏松减轻。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dru", "entity": "1-1羟化维生素D3" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "JAS" }, { "start_idx": 39, "end_idx": 43, "type": "bod", "entity": "血清IgG" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "骨质疏松减轻" } ] }, { "text": "儿童慢性或反复有痰咳嗽常提示潜在肺部疾病或有全身疾病基础,应特别注意有无哮喘、免疫缺陷、被动吸烟、解剖畸形、慢性鼻-鼻窦炎、支气管扩张、囊性纤维化、纤毛功能障碍、环境污染等。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "慢性或反复有痰咳嗽" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肺部" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "全身疾病" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "哮喘" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "被动吸烟" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "解剖畸形" }, { "start_idx": 54, "end_idx": 60, "type": "dis", "entity": "慢性鼻-鼻窦炎" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "支气管扩张" }, { "start_idx": 68, "end_idx": 72, "type": "dis", "entity": "囊性纤维化" }, { "start_idx": 74, "end_idx": 79, "type": "dis", "entity": "纤毛功能障碍" } ] }, { "text": "多数先有上呼吸道感染症状,起病缓慢。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "呼吸道感染症状" } ] }, { "text": "主要表现为气急、呼吸困难、心率增快、发绀、干咳、体重减轻、无力和食欲减退。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "气急" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "心率增快" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "发绀" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "干咳" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "体重减轻" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "无力" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "食欲减退" } ] }, { "text": "X线胸片显示肺野片状模糊阴影,或弥漫性阴影,呈毛玻璃样,基底部尤为明显。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "X线胸片" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "肺野片状模糊阴影" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "弥漫性阴影" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "呈毛玻璃样" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "基底部尤为明显" } ] }, { "text": "肾上腺皮质激素可使临床及X线改变好转。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "肾上腺皮质激素" } ] }, { "text": "无效者可选用免疫抑制剂或氯喹10mg/(kg•d)。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 12, "end_idx": 13, "type": "dru", "entity": "氯喹" } ] }, { "text": "已公认肺炎衣原体是5岁以上儿童肺炎的重要病原。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "mic", "entity": "肺炎衣原体" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "儿童肺炎" } ] }, { "text": "如遇到不能以病毒、细菌或支原体解释的年长儿肺炎,应想到本病。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "mic", "entity": "病毒" }, { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "细菌" }, { "start_idx": 12, "end_idx": 14, "type": "mic", "entity": "支原体" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "年长儿肺炎" } ] }, { "text": "目前这类头痛是小儿非器质性头痛中较常见的类型,其终身患病率为37%~78%。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "头痛" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "小儿非器质性头痛" } ] }, { "text": "PPHN指生后肺血管阻力持续性增高,肺动脉压超过体循环动脉压,使由胎儿型循环过渡至正常“成人”型循环发生障碍,而引起的心房及(或)动脉导管水平血液的右向左分流,临床出现严重低氧血症等症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "PPHN" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "肺血管" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "肺血管阻力持续性增高" }, { "start_idx": 18, "end_idx": 29, "type": "sym", "entity": "肺动脉压超过体循环动脉压" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "心房" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "动脉导管" }, { "start_idx": 59, "end_idx": 78, "type": "sym", "entity": "心房及(或)动脉导管水平血液的右向左分流" }, { "start_idx": 86, "end_idx": 89, "type": "dis", "entity": "低氧血症" } ] }, { "text": "PPHN多见于足月儿、近足月或过期产儿,但是早产儿亦可出现肺血管阻力的异常增高。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "PPHN" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "肺血管" }, { "start_idx": 29, "end_idx": 38, "type": "sym", "entity": "肺血管阻力的异常增高" } ] }, { "text": "肺实质性疾病,常见有呼吸窘迫综合征(RDS)、胎粪吸入综合征(MAS)和肺炎等,它们可因低氧而出现肺血管收缩、肺动脉高压。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "呼吸窘迫综合征" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "RDS" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "胎粪吸入综合征" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "MAS" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "肺炎" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "低氧" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "肺血管收缩" }, { "start_idx": 55, "end_idx": 59, "type": "sym", "entity": "肺动脉高压" } ] }, { "text": "生后除短期内有窘迫外,在生后24小时内可发现有发绀,如有肺部原发性疾病,患儿可出现气急、三凹征或呻吟,动脉血气显示严重低氧,二氧化碳分压相对正常。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "发绀" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肺部" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "气急" }, { "start_idx": 44, "end_idx": 46, "type": "sym", "entity": "三凹征" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "呻吟" }, { "start_idx": 51, "end_idx": 54, "type": "bod", "entity": "动脉血气" } ] }, { "text": "高氧吸入试验的目的是将PPHN或发绀型先天性心脏病与肺部疾病所致的发绀进行鉴别。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "PPHN" }, { "start_idx": 16, "end_idx": 24, "type": "dis", "entity": "发绀型先天性心脏病" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肺部" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "发绀" } ] }, { "text": "(5)其他:以多普勒测定左或右肺动脉平均血流速度,流速降低提示肺血管阻力增加、肺动脉高压,系列动态观察对评估PPHN的治疗效果有意义。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "多普勒" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肺动脉" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "肺血管阻力增加" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "肺动脉高压" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "PPHN" } ] }, { "text": "经典(传统)的治疗手段有人工呼吸机的高通气、纠正酸中毒或碱化血液、纠正体循环低血压或给以正性肌力药物或液体扩容。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "equ", "entity": "人工呼吸机" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "酸中毒" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "碱化血液" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "体循环" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "低血压" } ] }, { "text": "新型的治疗方法(如血管扩张剂、抗氧化剂治疗等)仍在不断的探索中,并有一定的前景。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "血管扩张剂" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "抗氧化剂" } ] }, { "text": "如氧合改善不理想时,可试用高频震荡人工呼吸机(HFOV)。", "entities": [ { "start_idx": 13, "end_idx": 21, "type": "equ", "entity": "高频震荡人工呼吸机" }, { "start_idx": 23, "end_idx": 26, "type": "equ", "entity": "HFOV" } ] }, { "text": "PPHN伴有肺实质性疾病时,呼吸治疗应考针对原发病而采取不同的策略,而高频通气常用于严重肺实质性疾病所致的呼吸衰竭。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "PPHN" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肺" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "呼吸" }, { "start_idx": 44, "end_idx": 44, "type": "bod", "entity": "肺" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "呼吸衰竭" } ] }, { "text": "目前,临床和实验研究主要集中在对调节肺血管张力的三条途径进行探索,包括一氧化氮、前列环素及内皮素在肺血管张力的调节及相关类似物或阻滞剂的应用。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肺血管" }, { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "一氧化氮" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "前列环素" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "内皮素" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "肺血管" } ] }, { "text": "(2)妥拉苏林有胃肠道出血、体循环低血压等副作用,已较少用于PPHN。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "妥拉苏林" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "胃肠道" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "胃肠道出血" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "体循环" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "体循环低血压" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "PPHN" } ] }, { "text": "(4)肺表面活性物质:成功的PPHN治疗取决于呼吸机应用时保持肺的最佳扩张状态。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "肺" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "PPHN" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "肺" } ] }, { "text": "PDE-5抑制剂西地那非(sildenafil)[或称万艾可(Viagra)]被试用于新生儿PPHN,且显示出能较选择性地作用于肺血管床的作用。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dru", "entity": "PDE-5抑制剂西地那非" }, { "start_idx": 13, "end_idx": 22, "type": "dru", "entity": "sildenafil" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "万艾可" }, { "start_idx": 31, "end_idx": 36, "type": "dru", "entity": "Viagra" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "PPHN" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "肺血管" } ] }, { "text": "(7)内皮素拮抗剂:内皮素为强烈的血管收缩剂,在PPHN患者血浆内皮素(ET-1)水平增高,在成人肺动脉高压,口服内皮素受体拮抗剂波生坦(bosentan)已用于临床。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "内皮素拮抗剂" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "内皮素" }, { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "血管收缩剂" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "PPHN" }, { "start_idx": 30, "end_idx": 44, "type": "sym", "entity": "血浆内皮素(ET-1)水平增高" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "肺动脉高压" }, { "start_idx": 57, "end_idx": 59, "type": "dru", "entity": "内皮素" }, { "start_idx": 65, "end_idx": 67, "type": "dru", "entity": "波生坦" }, { "start_idx": 69, "end_idx": 76, "type": "dru", "entity": "bosentan" } ] }, { "text": "可由细菌或病毒引起,也可为混合感染。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "细菌" }, { "start_idx": 5, "end_idx": 6, "type": "mic", "entity": "病毒" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "混合感染" } ] }, { "text": "病毒感染所致者,症状与一般咽炎相似,有咽痛、低热和其他轻度全身症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "病毒感染" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "咽炎" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "咽痛" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "低热" } ] }, { "text": "急性化脓性扁桃体炎起病急,局部及全身症状均较重,咽痛剧烈,吞咽困难,疼痛常向耳部放射。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "急性化脓性扁桃体炎" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "耳部" }, { "start_idx": 24, "end_idx": 41, "type": "sym", "entity": "咽痛剧烈,吞咽困难,疼痛常向耳部放射" } ] }, { "text": "下颌角淋巴结肿大。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "下颌角淋巴结" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "下颌角淋巴结肿大" } ] }, { "text": "检查可见扁桃体弥漫性红肿,隐窝口可有滤泡状黄白色脓点,并可连成假膜,但不超出扁桃体范围,易于拭去,不留出血创面,此点可与白喉鉴别。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "扁桃体" }, { "start_idx": 4, "end_idx": 11, "type": "sym", "entity": "扁桃体弥漫性红肿" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "扁桃体" }, { "start_idx": 13, "end_idx": 54, "type": "sym", "entity": "隐窝口可有滤泡状黄白色脓点,并可连成假膜,但不超出扁桃体范围,易于拭去,不留出血创面" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "白喉" } ] }, { "text": "链球菌感染所致者多大于2岁。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "链球菌感染" } ] }, { "text": "上呼吸道感染的并发症在婴幼儿较为多见。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "上呼吸道感染" } ] }, { "text": "可波及邻近器官,或向下蔓延,引起中耳炎、鼻窦炎、咽后壁脓肿、颈淋巴结炎、喉炎、气管炎、支气管肺炎等。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "中耳炎" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "咽后壁脓肿" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "颈淋巴结炎" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "喉炎" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "气管炎" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "支气管肺炎" } ] }, { "text": "年长儿若患链球菌性上感,可由于变态反应引起急性肾炎、风湿热等。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "链球菌性上感" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "急性肾炎" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "风湿热" } ] }, { "text": "病毒感染者血白细胞计数正常或偏低,细菌感染者血白细胞常增高,以中性粒细胞为主。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "病毒感染" }, { "start_idx": 5, "end_idx": 10, "type": "ite", "entity": "血白细胞计数" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "细菌感染" }, { "start_idx": 22, "end_idx": 25, "type": "ite", "entity": "血白细胞" }, { "start_idx": 31, "end_idx": 35, "type": "ite", "entity": "中性粒细胞" } ] }, { "text": "咽拭子培养可有病原菌生长;链球菌引起者血中抗O抗体滴度可增高。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "咽拭子培养" }, { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "病原菌" }, { "start_idx": 13, "end_idx": 15, "type": "mic", "entity": "链球菌" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "血" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "抗O抗体" } ] }, { "text": "病毒分离和双份血清抗体反应有助于病毒感染的诊断,但由于费时较长,操作复杂,难以在临床中应用。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "病毒分离" }, { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "双份血清抗体反应" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "病毒感染" } ] }, { "text": "近年来免疫荧光、酶联免疫等方法的开展,有利于病毒感染的早期诊断。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "免疫荧光" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "酶联免疫" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "病毒感染" } ] }, { "text": "根据临床表现,急性上感不难诊断,但需与流行性感冒鉴别。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "急性上感" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "流行性感冒" } ] }, { "text": "腹痛明显者应与急性阑尾炎鉴别。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "腹痛" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "急性阑尾炎" } ] }, { "text": "后者腹痛常先于发热,腹痛部位以右下腹为主,呈持续性,有腹肌紧张和固定压痛点;血白细胞及中性粒细胞增高。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "腹痛" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "发热" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "腹" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "右下腹" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "腹肌" }, { "start_idx": 26, "end_idx": 36, "type": "sym", "entity": "有腹肌紧张和固定压痛点" }, { "start_idx": 38, "end_idx": 41, "type": "ite", "entity": "血白细胞" }, { "start_idx": 43, "end_idx": 47, "type": "ite", "entity": "中性粒细胞" } ] }, { "text": "此外,上感可作为各种传染病,如麻疹、流行性脑脊髓膜炎、猩红热等的前驱症状,因而应结合流行病史、临床表现及实验室资料综合分析,并观察病情演变加以鉴别。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "上感" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "传染病" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "麻疹" }, { "start_idx": 18, "end_idx": 25, "type": "dis", "entity": "流行性脑脊髓膜炎" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "猩红热" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "流行病" } ] }, { "text": "病毒感染所致者以对症支持治疗为主,注意休息、多饮水。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "病毒感染" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "注意休息" }, { "start_idx": 22, "end_idx": 24, "type": "pro", "entity": "多饮水" } ] }, { "text": "高热患儿以物理降温为主,酌情结合口服药物降温。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "高热" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "物理降温" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "口服药物降温" } ] }, { "text": "咽痛者可含服咽喉片,严重者适当应用对乙酰氨基酚或布洛芬;如发生高热惊厥可予镇静、止惊等处理。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "咽痛" }, { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "含服" }, { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "咽喉片" }, { "start_idx": 17, "end_idx": 22, "type": "dru", "entity": "对乙酰氨基酚" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "布洛芬" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "高热惊厥" }, { "start_idx": 37, "end_idx": 41, "type": "pro", "entity": "镇静、止惊" } ] }, { "text": "酌情使用抗病毒药物如利巴韦林(病毒唑),疗程为3~5日。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "抗病毒药物" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "利巴韦林" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "病毒唑" } ] }, { "text": "细菌感染者,或病情重、有继发细菌感染可能,或有并发症者可选用抗菌药物,常用青霉素,疗程3~5日。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "细菌感染" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "继发细菌感染" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "抗菌药物" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "青霉素" } ] }, { "text": "青霉素过敏,或青霉素治疗无效者可应用含β-内酰胺酶抑制剂的复合β-内酰胺类,或头孢霉素、大环内酯类抗生素。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "青霉素过敏" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "青霉素治疗无效" }, { "start_idx": 19, "end_idx": 27, "type": "dru", "entity": "β-内酰胺酶抑制剂" }, { "start_idx": 29, "end_idx": 36, "type": "dru", "entity": "复合β-内酰胺类" }, { "start_idx": 39, "end_idx": 42, "type": "dru", "entity": "头孢霉素" }, { "start_idx": 44, "end_idx": 51, "type": "dru", "entity": "大环内酯类抗生素" } ] }, { "text": "羟氨苄青霉素每日一次疗法可能成为青霉素治疗链球菌咽炎的替代方案。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "羟氨苄青霉素" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "青霉素" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "链球菌咽炎" } ] }, { "text": "应注意呼吸道隔离,预防并发症。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "呼吸道" } ] }, { "text": "其临床表现以阴离子间隙正常的高氯性代谢性酸中毒、肾钙化及肾结石为特征。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "阴离子间隙正常" }, { "start_idx": 14, "end_idx": 22, "type": "dis", "entity": "高氯性代谢性酸中毒" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "肾钙化" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肾结石" } ] }, { "text": "原发性为常染色体显性或隐性遗传,继发性者常是由于其他疾病影响到肾小管功能所致,可见于高丙种球蛋白血症、原发性甲状旁腺功能亢进、维生素D中毒、移植肾排斥反应、髓质海绵肾、梗阻性肾病、特发性高钙尿症肾钙化、Wilson病、失盐性先天性肾上腺皮质增生症、药物及毒素导致肾损害(如锂、两性霉素B、甲苯及地高辛等)。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "sym", "entity": "常染色体显性或隐性遗传" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "肾小管" }, { "start_idx": 42, "end_idx": 49, "type": "dis", "entity": "高丙种球蛋白血症" }, { "start_idx": 51, "end_idx": 61, "type": "dis", "entity": "原发性甲状旁腺功能亢进" }, { "start_idx": 63, "end_idx": 68, "type": "dis", "entity": "维生素D中毒" }, { "start_idx": 70, "end_idx": 76, "type": "dis", "entity": "移植肾排斥反应" }, { "start_idx": 78, "end_idx": 82, "type": "dis", "entity": "髓质海绵肾" }, { "start_idx": 84, "end_idx": 88, "type": "dis", "entity": "梗阻性肾病" }, { "start_idx": 90, "end_idx": 99, "type": "dis", "entity": "特发性高钙尿症肾钙化" }, { "start_idx": 101, "end_idx": 107, "type": "dis", "entity": "Wilson病" }, { "start_idx": 109, "end_idx": 122, "type": "dis", "entity": "失盐性先天性肾上腺皮质增生症" }, { "start_idx": 131, "end_idx": 133, "type": "dis", "entity": "肾损害" } ] }, { "text": "原发性dRTA的基因突变有两种。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "原发性dRTA" } ] }, { "text": "常染色质隐性遗传则涉及质子泵B亚基的缺陷(ATP6B1)。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "常染色质隐性遗传" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "质子泵B亚基的缺陷" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "ATP6B1" } ] }, { "text": "后者常在2岁后出现症状,以女性多见,为染色体显性遗传。", "entities": [ { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "染色体显性遗传" } ] }, { "text": "不完全型dRTA可无酸中毒表现而仅出现低钾、肌无力或肾钙化。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "不完全型dRTA" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "酸中毒" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "低钾" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "肌无力" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "肾钙化" } ] }, { "text": "血钾降低也是dRTA的重要表现,甚至为不完全性dRTA的唯一表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "血钾降低" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "dRTA" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "不完全性dRTA" } ] }, { "text": "一般情况下可使用Shohl合剂或单用10%枸橼酸钾口服,剂量2~4ml/(kg•d);有低钙血症者可适当补充钙剂,如10%葡萄糖酸钙2ml/(kg•d),总量<20ml/d。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dru", "entity": "Shohl合剂" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "枸橼酸钾" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "低钙血症" }, { "start_idx": 50, "end_idx": 55, "type": "pro", "entity": "适当补充钙剂" }, { "start_idx": 61, "end_idx": 65, "type": "dru", "entity": "葡萄糖酸钙" } ] }, { "text": "对高钙尿症,可服用上述枸橼酸制剂治疗,必要时还可加双氢克尿噻,2mg/(kg•d)口服,常可减轻高钙尿症,并促进溶石与排石。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "高钙尿症" }, { "start_idx": 7, "end_idx": 17, "type": "pro", "entity": "服用上述枸橼酸制剂治疗" }, { "start_idx": 25, "end_idx": 29, "type": "dru", "entity": "双氢克尿噻" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "高钙尿症" } ] }, { "text": "内源性感染的放线菌多为构成人和动物黏膜,特别是口腔黏膜固有菌群中的兼性厌氧菌。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "内源性感染" }, { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "放线菌" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "口腔黏膜" }, { "start_idx": 33, "end_idx": 37, "type": "mic", "entity": "兼性厌氧菌" } ] }, { "text": "临床上的放线菌病、泪小管炎、牙周炎和龋齿均由这类菌诱发引起。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "放线菌病" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "泪小管炎" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "牙周炎" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "龋齿" } ] }, { "text": "主要致病菌有诺卡菌属、马杜拉放线菌和链霉菌属中的一些种。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "mic", "entity": "诺卡菌属" }, { "start_idx": 11, "end_idx": 16, "type": "mic", "entity": "马杜拉放线菌" }, { "start_idx": 18, "end_idx": 20, "type": "mic", "entity": "链霉菌" } ] }, { "text": "临床上一般将广义的放线菌病因损害的部位不同分为以下几型:颈面型放线菌病、胸部型放线菌病、腹部型放线菌病、皮肤型放线菌病、脑型放线菌病、其他组织的放线菌病、放线菌性足菌病。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "mic", "entity": "放线菌" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "颈面型放线菌病" }, { "start_idx": 36, "end_idx": 42, "type": "dis", "entity": "胸部型放线菌病" }, { "start_idx": 44, "end_idx": 50, "type": "dis", "entity": "腹部型放线菌病" }, { "start_idx": 52, "end_idx": 58, "type": "dis", "entity": "皮肤型放线菌病" }, { "start_idx": 60, "end_idx": 65, "type": "dis", "entity": "脑型放线菌病" }, { "start_idx": 67, "end_idx": 75, "type": "dis", "entity": "其他组织的放线菌病" }, { "start_idx": 77, "end_idx": 83, "type": "dis", "entity": "放线菌性足菌病" } ] }, { "text": "脑型放线菌病的临床表现与细菌性脑部病变感染略似,有以下两型:①局限性脑脓肿型:多见于大脑半球、少数发生在第三脑室和颅后窝。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "脑型放线菌病" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "细菌性脑部病变" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "局限性脑脓肿" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "大脑半球" }, { "start_idx": 52, "end_idx": 55, "type": "bod", "entity": "第三脑室" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "颅后窝" } ] }, { "text": "主要表现为脑部占位性病变的体征,如颅压增高、脑神经损害、头痛、恶心、呕吐、复视、视神经乳头水肿等,常无发热,白细胞总数及分类正常。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "脑部占位性病变" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "颅压增高" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "脑神经损害" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "头痛" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "恶心" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "呕吐" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "复视" }, { "start_idx": 40, "end_idx": 44, "type": "bod", "entity": "视神经乳头" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "视神经乳头水肿" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "发热" }, { "start_idx": 54, "end_idx": 56, "type": "ite", "entity": "白细胞" }, { "start_idx": 54, "end_idx": 63, "type": "sym", "entity": "白细胞总数及分类正常" } ] }, { "text": "当青霉素过敏、无效或其他原因不能耐受时,可选用红霉素、林可霉素、克林霉素、头孢氨苄霉素、氯霉素、链霉素等。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dru", "entity": "青霉素" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "红霉素" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "林可霉素" }, { "start_idx": 32, "end_idx": 35, "type": "dru", "entity": "克林霉素" }, { "start_idx": 37, "end_idx": 42, "type": "dru", "entity": "头孢氨苄霉素" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "氯霉素" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "链霉素" } ] }, { "text": "颈面部放线菌病预后良好,其他类型若能适当治疗,可减少废用、畸形等后遗症。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "颈面部放线菌病" } ] }, { "text": "尤因肉瘤可以发生在全身各个骨骼器,但半数以上的肿瘤发生在肢体的长骨,特别是股骨。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尤因肉瘤" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "骨骼器" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "肿瘤" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肢体" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "长骨" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "股骨" } ] }, { "text": "骨盆、腓骨、胫骨、肱骨、肋骨、锁骨也经常累及。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "骨盆" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "腓骨" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "胫骨" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肱骨" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肋骨" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "锁骨" } ] }, { "text": "疼痛可以是间歇性的,但随着病情的发展多转为持续性,部分病例早期有原因不明的间歇性发热,肿瘤广泛累及骨骼后甚至会有病理性骨折的发生,但不常见。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "疼痛可以是间歇性的" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "间歇性发热" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "肿瘤" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "骨骼" }, { "start_idx": 55, "end_idx": 63, "type": "sym", "entity": "有病理性骨折的发生" } ] }, { "text": "特别是当骨肿瘤存在时,早期检查肺部对于治疗方案的制定有重要的意义。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "骨肿瘤" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肺部" } ] }, { "text": "尤因肉瘤的X线表现为肿瘤多数位于骨干的中部,呈混合性骨破坏影像,皮质骨上可以有斑点状的骨破坏。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尤因肉瘤" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "X线" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 22, "end_idx": 30, "type": "sym", "entity": "呈混合性骨破坏影像" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "皮质骨" } ] }, { "text": "CT和MRI检查对于肿瘤浸润周围软组织可以有较明确的描述。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "CT" }, { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "MRI检查" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "软组织" } ] }, { "text": "当肿瘤组织呈现为半液体状态时,应该同慢性骨髓炎做鉴别,必要的时候要做组织的细菌培养。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "慢性骨髓炎" } ] }, { "text": "过去,放射治疗应用较多,原因是高度增长扩大的尤文肿瘤,对于放射线有着较为敏感的反应,甚至有时简单的X线摄片都会引起肿瘤生长的暂时性停顿。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "放射治疗" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "尤文肿瘤" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "X线摄片" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "肿瘤" } ] }, { "text": "但近年来的研究越来越多地表明,单纯依赖肿瘤组织对于放射的敏感反应尚不能达到满意的疗效,很多病例,经过化疗后,应及时采取手术肿瘤切除的方法,患者长期生存率的提高比单纯接受放射治疗要好。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "肿瘤组织" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "放射" }, { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "化疗" }, { "start_idx": 59, "end_idx": 64, "type": "pro", "entity": "手术肿瘤切除" }, { "start_idx": 84, "end_idx": 85, "type": "pro", "entity": "放射" } ] }, { "text": "双胎输血综合征的供血者出现贫血,受血者则表现为:多血,红细胞增多,血黏度增高,心、肝、肾、胰及肾上腺增大,羊水多,高胆红素血症,充血性心力衰竭等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "双胎输血综合征" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "贫血" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "多血" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "红细胞增多" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "血黏度增高" }, { "start_idx": 39, "end_idx": 51, "type": "sym", "entity": "心、肝、肾、胰及肾上腺增大" }, { "start_idx": 53, "end_idx": 55, "type": "sym", "entity": "羊水多" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "高胆红素血症" }, { "start_idx": 64, "end_idx": 70, "type": "sym", "entity": "充血性心力衰竭" } ] }, { "text": "对贫血较重者,需输血。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "贫血" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "输血" } ] }, { "text": "体液免疫紊乱也可引起再障的发生,部分再障患儿血浆中可有抗造血细胞抗体存在。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "体液免疫紊乱" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "再障" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "再障" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血浆" }, { "start_idx": 27, "end_idx": 33, "type": "bod", "entity": "抗造血细胞抗体" } ] }, { "text": "8.非血液学综合征如Down,Dubowitz,Seckel综合征等。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "非血液学综合征" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "Down" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "Dubowitz" }, { "start_idx": 24, "end_idx": 32, "type": "dis", "entity": "Seckel综合征" } ] }, { "text": "4.当慢性再障在病程中病情恶化临床表现、血象及骨髓象与急性再障相同时,称为重型再障Ⅱ型(SAA-Ⅱ)。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "慢性再障" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "血象" }, { "start_idx": 23, "end_idx": 25, "type": "pro", "entity": "骨髓象" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "急性再障" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "重型再障Ⅱ型" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "SAA-Ⅱ" } ] }, { "text": "网织红细胞<1%;白细胞总数大多降低,但也有正常者,此时常出现淋巴细胞相对值增高。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "网织红细胞" }, { "start_idx": 9, "end_idx": 13, "type": "ite", "entity": "白细胞总数" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 31, "end_idx": 39, "type": "sym", "entity": "淋巴细胞相对值增高" } ] }, { "text": "儿童再障以后两型多见。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "再障" } ] }, { "text": "3.血清铁、镁、锌升高。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血清" }, { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "血清铁、镁、锌升高" } ] }, { "text": "4.血清EPO、游离红细胞原卟啉(FEP)增加。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "血清EPO" }, { "start_idx": 8, "end_idx": 15, "type": "bod", "entity": "游离红细胞原卟啉" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "FEP" }, { "start_idx": 2, "end_idx": 22, "type": "sym", "entity": "血清EPO、游离红细胞原卟啉(FEP)增加" } ] }, { "text": "鉴别的主要依据为骨髓涂片、骨髓活检及相应的细胞和分子生物学检查。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "骨髓涂片" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "骨髓活检" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "细胞" } ] }, { "text": "因此,积极预防和治疗感染是降低死亡率的重要措施。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "感染" } ] }, { "text": "一旦出现感染,应及早使用强力有效的抗生素。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "感染" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "抗生素" } ] }, { "text": "在没有明确病原体感染之前,通常需要广谱抗生素、抗真菌药及抗病毒药联合应用。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "病原体感染" }, { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "广谱抗生素" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "抗真菌药" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "抗病毒药" } ] }, { "text": "上述方案主要用于急性或重型再障的治疗。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "急性或重型再障" } ] }, { "text": "用药一周末至两周内可发生血清病,出现发热、皮疹(荨麻疹、麻疹样或猩红热样)、淋巴结增大、关节酸痛,严重表现有面部及四肢水肿、少尿、喉头水肿、哮喘、末梢神经炎、头痛、谵妄,甚至惊厥。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "血清病" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "发热" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "皮疹" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "荨麻疹" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "麻疹样或猩红热样" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "淋巴结" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "淋巴结增大" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "关节" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "关节酸痛" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "面部" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "四肢" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "面部及四肢水肿" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "少尿" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "喉头" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "喉头水肿" }, { "start_idx": 70, "end_idx": 71, "type": "dis", "entity": "哮喘" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "末梢神经炎" }, { "start_idx": 79, "end_idx": 80, "type": "sym", "entity": "头痛" }, { "start_idx": 82, "end_idx": 83, "type": "sym", "entity": "谵妄" }, { "start_idx": 87, "end_idx": 88, "type": "sym", "entity": "惊厥" } ] }, { "text": "一旦出现上述任何表现者均应严密监护,仅有皮疹者则可给予异丙嗪、止痒洗剂等对症处理,较重表现者则可给予甲基泼尼松龙10mg/(kg•d)一次静注,连用3~4日。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "皮疹" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "异丙嗪" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "止痒洗剂" }, { "start_idx": 50, "end_idx": 55, "type": "dru", "entity": "甲基泼尼松龙" } ] }, { "text": "(4)抗T淋巴细胞单克隆抗体(单抗)。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "bod", "entity": "抗T淋巴细胞单克隆抗体" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "单抗" } ] }, { "text": "(5)大剂量丙种球蛋白。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dru", "entity": "丙种球蛋白" } ] }, { "text": "(6)异基因造血干细胞移植:适用于重型再障,病程早期进行移植成活率极高。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "pro", "entity": "异基因造血干细胞移植" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "重型再障" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "移植" } ] }, { "text": "慢性再障治疗与急性再障治疗有所区别,急性再障以免疫抑制剂为主,而慢性再障则以雄性激素为主的综合疗法。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "慢性再障" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "急性再障" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "急性再障" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "慢性再障" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "雄性激素" } ] }, { "text": "(4)莨菪浸膏片:每次10mg,每日3次,口服,每日递增10~20mg至每次240~300mg,30日为一疗程,休7日后重复。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "莨菪浸膏片" } ] }, { "text": "急性再障预后甚差,如未能得到有效治疗者,绝大多数一年内死亡,有的甚至2~3月内夭亡。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "急性再障" } ] }, { "text": "如髋关节内翻、膝关节内翻或外翻等。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "髋关节" }, { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "如髋关节内翻" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "膝关节" }, { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "膝关节内翻或外翻" } ] }, { "text": "近年来,除了传统的手术活组织检查,穿刺活组织检查,特别是超声波或放射线引导下的微创性组织活检技术得到了很大的发展。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "手术活组织检查" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "穿刺活组织检查" }, { "start_idx": 39, "end_idx": 45, "type": "pro", "entity": "微创性组织活检" } ] }, { "text": "是一种地方性寄生虫病,由并殖吸虫寄生于人体所引起。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "地方性寄生虫病" }, { "start_idx": 12, "end_idx": 15, "type": "mic", "entity": "并殖吸虫" } ] }, { "text": "约经1~3周后绝大多数幼虫由腹腔穿过横膈进入胸腔(主要在右侧沿肝表面向上移行),可引起胸膜炎症状。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "腹腔" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "横膈" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "胸腔" }, { "start_idx": 31, "end_idx": 31, "type": "bod", "entity": "肝" }, { "start_idx": 43, "end_idx": 45, "type": "sym", "entity": "胸膜炎" } ] }, { "text": "根据虫体寄生部位的不同,有不同的症状,但以胸部、腹部、脑部为重点。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "胸部" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "腹部" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "脑部" } ] }, { "text": "初期即发生胸膜炎者,有咳嗽、胸闷,胸痛及上腹痛明显,可痰中带血。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "胸膜炎" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "咳嗽" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "胸闷" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "胸痛" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "上腹痛" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "痰中带血" } ] }, { "text": "肺内囊肿、脓肿可穿破至胸腔,发生胸水、脓胸或脓气胸等体征。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "肺" }, { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "囊肿" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "脓肿" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "胸腔" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "胸水" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "脓胸" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "脓气胸" } ] }, { "text": "胸X线片显示肺浸润、囊状或结节状阴影或薄壁空洞,有时呈液平面。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "胸X线片" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "肺" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "肺浸润" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "囊状或结节状阴影" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "薄壁空洞" }, { "start_idx": 27, "end_idx": 29, "type": "sym", "entity": "液平面" } ] }, { "text": "此外尚可累及腹腔内其他器官,而可出现黄疸、脓尿、血尿、肝脾肿大等症状,有时甚至与急腹症不易鉴别。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "腹腔" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "黄疸" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "脓尿" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "血尿" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "急腹症" } ] }, { "text": "此外尚可有癔症症状,表现为精神失常,记忆力差,有幻觉、幻视等。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "癔症" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "精神失常" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "记忆力差" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "幻觉" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "幻视" } ] }, { "text": "必要时可做气脑、脑室造影及肺部断层摄影。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "气脑" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "脑室造影" }, { "start_idx": 13, "end_idx": 18, "type": "pro", "entity": "肺部断层摄影" } ] }, { "text": "散发性甲低是由于先天性甲状腺发育不良、异位或甲状腺激素合成途径缺陷所致;地方性甲低多见于甲状腺肿流行的地区,系由于地区性水、土和食物中碘缺乏所致。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "散发性甲低" }, { "start_idx": 8, "end_idx": 32, "type": "sym", "entity": "先天性甲状腺发育不良、异位或甲状腺激素合成途径缺陷" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "地方性甲低" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "甲状腺肿" } ] }, { "text": "吞咽反射不协调,贲门括约肌松弛,胃容量小,早产儿容易发生溢乳和呛咳。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "贲门括约肌" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "胃" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "溢乳和呛咳" } ] }, { "text": "早产儿发生败血症和脑膜炎的机会是足月新生儿的4倍,败血症死亡率高达30%。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "败血症" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "败血症" } ] }, { "text": "尿常规有蛋白尿及管型尿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "尿常规" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "蛋白尿" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "管型尿" } ] }, { "text": "血浆β-血栓球蛋白和Ⅷ因子相关抗原测定有助于随访疗效以及反映血管炎活动程度。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "血浆β-血栓球蛋白" }, { "start_idx": 10, "end_idx": 16, "type": "bod", "entity": "Ⅷ因子相关抗原" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "血管炎" } ] }, { "text": "B组链球菌(GBS)则是新生儿肺炎的主要病原。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "B组链球菌" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "GBS" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "新生儿肺炎" } ] }, { "text": "3型vWD患儿可有关节、软组织出血,极少数因颅内出血而危及生命。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "关节" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "软组织" } ] }, { "text": "反复出血患儿易导致缺铁性贫血。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "缺铁性贫血" } ] }, { "text": "2型又分4种亚型:血浆中大和中等大小的多聚体缺乏者为2A型,缺乏大多聚体且与血小板GPⅠb亲和性增加为2B型,vWF与血小板GPⅠb结合力降低为2M型,与因子Ⅷ结合力降低者为2N型。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "血浆" } ] }, { "text": "该类药物可单纯使用或与DDAVP合用;②口服避孕药:用于治疗月经过多;③肾上腺皮质激素:对产生vWF抗体者更为有效;④因慢性缺铁引起缺铁性贫血者,应给予铁剂治疗。", "entities": [ { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "月经过多" }, { "start_idx": 36, "end_idx": 42, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "慢性缺铁" }, { "start_idx": 66, "end_idx": 70, "type": "dis", "entity": "缺铁性贫血" }, { "start_idx": 76, "end_idx": 77, "type": "dru", "entity": "铁剂" } ] }, { "text": "补充磷及维生素D对有佝偻病表现的患者有较好的效果。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "dru", "entity": "磷" }, { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "维生素D" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "佝偻病" } ] }, { "text": "Depape-Brigger和Goldman试用二硫苏糖醇(dithiotreitol,DIT)治疗2例也未获效果。", "entities": [ { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "二硫苏糖醇" }, { "start_idx": 30, "end_idx": 42, "type": "dru", "entity": "dithiotreitol" }, { "start_idx": 44, "end_idx": 46, "type": "dru", "entity": "DIT" } ] }, { "text": "其不良反应主要有恶心、呕吐、腐臭味较难忍受以及血清病样反应等。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "恶心" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "呕吐" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "腐臭味" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "血清病样" } ] }, { "text": "上肢缺血引起肢体无力和麻木。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "上肢" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肢体" }, { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "肢体无力和麻木" } ] }, { "text": "胸部X线片可显示主动脉钙化或主动脉增宽,超声波检查可显示周围动脉或主动脉等狭窄部位及程度,动脉造影和MRI可显示狭窄或扩张的部位及程度,以及血流减少的程度。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "主动脉" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "主动脉" }, { "start_idx": 20, "end_idx": 24, "type": "pro", "entity": "超声波检查" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "动脉" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "主动脉" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "动脉造影" }, { "start_idx": 50, "end_idx": 52, "type": "pro", "entity": "MRI" } ] }, { "text": "经多代学者的研究努力,肾母细胞瘤的低存活率迅速得到提高,其生存率1940年为25%,至今已上升至90%左右。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肾母细胞瘤" } ] }, { "text": "肾母细胞瘤可能由于未分化形成小管和小球的后肾胚芽异常增生所致。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肾母细胞瘤" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肾胚芽" } ] }, { "text": "有时同时有先天性白内障,还可有中枢神经异常,如小头畸形、头面异形、耳廓异常、泌尿系统畸形和智能迟缓等,近年对虹膜缺如合并肾母细胞瘤患儿的细胞遗传学研究表明,其均有11号染色体短臂移位或部分缺如的现象。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "先天性白内障" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "中枢神经异常" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "小头畸形" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "头面异形" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "耳廓异常" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "泌尿系统畸形" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "智能迟缓" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "虹膜" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "肾母细胞瘤" }, { "start_idx": 84, "end_idx": 88, "type": "bod", "entity": "染色体短臂" } ] }, { "text": "此外,近年来还发现肾母细胞瘤伴外生殖器雌雄难辨的两性畸形患儿,Raifer报道10例,其中7例发生在单侧肾母细胞瘤,3例双侧肾母细胞瘤。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "肾母细胞瘤" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "生殖器" }, { "start_idx": 50, "end_idx": 56, "type": "bod", "entity": "单侧肾母细胞瘤" }, { "start_idx": 60, "end_idx": 66, "type": "bod", "entity": "双侧肾母细胞瘤" } ] }, { "text": "肾母细胞瘤淋巴结转移并不多见,且大多限于局部淋巴结,但血源性扩散甚为多见,首先是经肾静脉,其中常有肿瘤栓子,可漫延至下腔静脉,甚至到右心房,血行性扩散80%到肺部,有时到肝,偶尔到骨骼。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肾母细胞瘤" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "淋巴结" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "肾静脉" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "肿瘤栓子" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "下腔静脉" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "右心房" }, { "start_idx": 79, "end_idx": 79, "type": "bod", "entity": "肺" }, { "start_idx": 85, "end_idx": 85, "type": "bod", "entity": "肝" }, { "start_idx": 90, "end_idx": 91, "type": "bod", "entity": "骨骼" } ] }, { "text": "患儿往往有含糊不清的消化道症状,如恶心、呕吐和食欲减退等。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "sym", "entity": "有含糊不清的消化道症状" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "恶心" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "呕吐" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "食欲减退" } ] }, { "text": "近年还有人从肾母细胞瘤的浸出液中作肾素的定量分析,其量较正常肾皮质所含高得多。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "肾母细胞瘤" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肾素" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "肾皮质" } ] }, { "text": "尿显微镜检查不少有血尿和蛋白尿,但尿中多不能找到癌细胞。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "尿显微镜检查" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "血尿" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "尿" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "癌细胞" } ] }, { "text": "肿瘤主要经血流转移,故向肺转移最为多见,转移后鲜有咳嗽、咯血等症状,故X线肺部检查至为重要。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肺" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "鲜有咳嗽、咯血" }, { "start_idx": 35, "end_idx": 40, "type": "pro", "entity": "X线肺部检查" } ] }, { "text": "Ⅱ期肿瘤已扩散至肾周组织,但能完整切除。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "肾周组织" } ] }, { "text": "Ⅲ期腹部残留有非血源性肿瘤,并有下列一种或多种情况:①肾门和腹主动脉旁淋巴结浸润;②弥漫性腹腔播散,术前或术中肿瘤散落;③切除面镜检或肉眼示有肿瘤残留;④腹膜有肿瘤种植;⑤由于局部浸润粘连,肿瘤无法被完整切除。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "腹部" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "非血源性肿瘤" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肾门" }, { "start_idx": 30, "end_idx": 37, "type": "bod", "entity": "腹主动脉旁淋巴结" }, { "start_idx": 55, "end_idx": 56, "type": "dis", "entity": "肿瘤" }, { "start_idx": 61, "end_idx": 65, "type": "pro", "entity": "切除面镜检" }, { "start_idx": 71, "end_idx": 72, "type": "dis", "entity": "肿瘤" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "腹膜" }, { "start_idx": 80, "end_idx": 81, "type": "dis", "entity": "肿瘤" }, { "start_idx": 95, "end_idx": 96, "type": "dis", "entity": "肿瘤" } ] }, { "text": "Ⅳ期有血源性转移至肺、肝、骨和脑。", "entities": [ { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肺" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "肝" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "骨" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "脑" } ] }, { "text": "双侧肾母细胞瘤术前应仔细考虑手术方案,一般作较大肿瘤患侧肾切除,较小的对侧作半肾切除或仅作肿瘤摘除。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "双侧肾母细胞瘤" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "手术" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "肿瘤" }, { "start_idx": 28, "end_idx": 30, "type": "pro", "entity": "肾切除" }, { "start_idx": 38, "end_idx": 41, "type": "pro", "entity": "半肾切除" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "肿瘤摘除" } ] }, { "text": "1.放线菌素D(AMD)术后当日开始应用,每日静脉注射15μg/kg,连续5日,总剂量75μg/kg,为1疗程。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "放线菌素D" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "AMD" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "静脉注射" } ] }, { "text": "放线菌素D可引起恶心、呕吐、口腔炎、腹泻、脱发和骨髓抑制等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "放线菌素D" } ] }, { "text": "目前了解的分子病理改变有垂体加压素基因(AVP-NPⅡ)。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "bod", "entity": "垂体加压素基因" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "AVP-NPⅡ" } ] }, { "text": "部分家族性单纯性尿崩症患者发现AVP-NPⅡ基因有突变,大多为基因点突变,且突变类型及位点具有一定的异质性,有的呈现常染色体显性遗传,也有常染色体隐性遗传。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "家族性单纯性尿崩症" }, { "start_idx": 15, "end_idx": 23, "type": "bod", "entity": "AVP-NPⅡ基因" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "突变" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "基因点突变" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "突变" } ] }, { "text": "确需吸入氧气治疗(如发生呼吸暂停),应监测和控制用氧浓度吸入时间,同时监测血氧饱和度,使其保持在88%~93%为宜。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "吸入氧气治疗" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 24, "end_idx": 27, "type": "ite", "entity": "用氧浓度" }, { "start_idx": 37, "end_idx": 41, "type": "ite", "entity": "血氧饱和度" } ] }, { "text": "氧气吸入可能和早产儿视网膜眼病(ROP)的发生有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "氧气吸入" }, { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "早产儿视网膜眼病" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "ROP" } ] }, { "text": "环境被蛔虫卵污染,是造成小儿感染蛔虫病的主要来源。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "蛔虫" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "蛔虫病" } ] }, { "text": "人体蛔虫病由于雌虫产卵量极大和具有对恶劣环境条件的抵抗力而广泛流行,虫卵在泥土中生存数月仍具有感染力,在5~10℃较冷气候条件下可生存2年。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "蛔虫病" }, { "start_idx": 7, "end_idx": 8, "type": "mic", "entity": "雌虫" }, { "start_idx": 34, "end_idx": 35, "type": "mic", "entity": "虫卵" } ] }, { "text": "因此蛔虫病可在温暖季节传播,有时经年都可传播。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "蛔虫病" } ] }, { "text": "肠道蛔虫可无任何症状,或有食欲不佳和腹痛、腹胀、恶心、呕吐、轻泻或便秘。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肠道" }, { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "蛔虫" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "食欲不佳" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "腹痛" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "腹胀" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "恶心" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "呕吐" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "轻泻" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "便秘" } ] }, { "text": "由于虫体代谢产物或崩解物被吸收,可引起低热和出现精神、神经系症状,如精神委靡或兴奋不安、头痛、易怒、睡眠不安、易惊。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "mic", "entity": "虫体代谢产物或崩解物" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "低热" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "精神委靡" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "兴奋不安" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "头痛" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "易怒" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "睡眠不安" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "易惊" } ] }, { "text": "蚴虫到达肺,表现为咳嗽、咳血丝痰,肺部体征常不明显,而X线检查可显示病灶淡影,有时类似支气管肺炎或粟粒性肺结核,但阴影游走或很快消失,称为蛔蚴性肺炎或蛔虫性嗜酸性粒细胞肺炎。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "蚴虫" }, { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "肺" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "咳嗽" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "咳血丝痰" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "X线检查" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "病灶淡影" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "支气管肺炎" }, { "start_idx": 49, "end_idx": 54, "type": "dis", "entity": "粟粒性肺结核" }, { "start_idx": 57, "end_idx": 65, "type": "sym", "entity": "阴影游走或很快消失" }, { "start_idx": 69, "end_idx": 73, "type": "dis", "entity": "蛔蚴性肺炎" }, { "start_idx": 75, "end_idx": 85, "type": "dis", "entity": "蛔虫性嗜酸性粒细胞肺炎" } ] }, { "text": "蚴虫移行到身体其他器官可引起相应的症状,如脑膜炎、癫痫、视网膜炎、眼睑肿胀及尿的改变等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "蚴虫" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "癫痫" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "视网膜炎" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "眼睑肿胀" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "尿" } ] }, { "text": "多见于1~6岁年龄,突然起病,剧烈的腹部绞痛和呕吐,甚至吐出胆汁、蛔虫,病情进展快。", "entities": [ { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "腹部绞痛" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "呕吐" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "吐出胆汁、蛔虫" } ] }, { "text": "完全性肠梗阻历时过久,可发生肠壁坏死、穿孔及腹膜炎。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "完全性肠梗阻" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "肠壁坏死、穿孔" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "此病进展迅速,容易发生坏死、穿孔,形成腹膜炎。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "坏死、穿孔" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "蛔虫还可引起脓胸、肺脓肿、急性出血性胰腺炎、泌尿系感染。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "蛔虫" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "脓胸" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "肺脓肿" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "急性出血性胰腺炎" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "泌尿系感染" } ] }, { "text": "手术切除后预后良好。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "手术切除" } ] }, { "text": "疑为蛔虫引起的嗜酸性肺炎时,痰中找到蛔虫幼虫可确诊。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "mic", "entity": "蛔虫" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "嗜酸性肺炎" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "痰" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "蛔虫" } ] }, { "text": "长期预防蛔虫病的最有效措施是对人粪必须进行无害化处理后再当肥料使用,以及提供对污水处理的卫生设施。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "蛔虫病" }, { "start_idx": 14, "end_idx": 25, "type": "pro", "entity": "对人粪必须进行无害化处理" } ] }, { "text": "血友病丙为常染色体显性或不完全性隐性遗传,男女均可发病或是传递者。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "血友病丙" } ] }, { "text": "因子Ⅷ基因很大,长186kb,常见的突变方式是点突变、基因缺失、插入异常片段及内含子22倒位,由于因子Ⅷ基因缺陷导致血友病的发生,重型患儿中约50%发病与内含子22倒位有关。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "因子Ⅷ基因" }, { "start_idx": 49, "end_idx": 53, "type": "bod", "entity": "因子Ⅷ基因" }, { "start_idx": 58, "end_idx": 60, "type": "dis", "entity": "血友病" } ] }, { "text": "缺乏因子Ⅷ或因子Ⅸ时,凝血活酶生成减少,纤维蛋白凝块形成延迟,凝血时间延长,引起出血症状。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "因子Ⅷ" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "因子Ⅸ" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "凝血活酶" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "纤维蛋白" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "出血症状" } ] }, { "text": "因子Ⅸ由肝脏合成,属于依赖维生素K的凝血因子,半寿期18~24小时,血浆活性80%~120%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "因子Ⅸ" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肝脏" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "凝血因子" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "血浆" } ] }, { "text": "血友病甲多在婴儿开始学爬、学走时发病,生后9个月内发病者少,偶见新生儿断脐时出血不止,轻症患儿可至成年后才发现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "血友病甲" }, { "start_idx": 35, "end_idx": 41, "type": "sym", "entity": "断脐时出血不止" } ] }, { "text": "血友病肌肉出血和血肿以下肢、前臂、臀部多见。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "血友病" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肌肉" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "血友病肌肉出血" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "血肿" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "下肢" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "前臂" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "臀部" } ] }, { "text": "深部血肿有相应部位疼痛、压迫症状。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "血肿" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "部位疼痛、压迫" } ] }, { "text": "如出血量多,可引起休克、贫血、黄疸及全身发热。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "出血量多" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "休克" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "贫血" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "黄疸" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "全身发热" } ] }, { "text": "皮下、齿龈、口腔及鼻黏膜易于受伤故为出血多发部位,但皮肤黏膜出血并非为本病的特征,皮肤瘀点、瘀斑少见。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "皮下" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "齿龈" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "口腔" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "鼻黏膜" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "皮肤黏膜" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "皮肤" }, { "start_idx": 41, "end_idx": 49, "type": "sym", "entity": "皮肤瘀点、瘀斑少见" } ] }, { "text": "对伴有剧烈头痛的血友病患儿应警惕颅内出血或硬膜下出血的可能。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "剧烈头痛" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "血友病" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "颅内" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "颅内出血" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "硬膜" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "硬膜下出血" } ] }, { "text": "血友病丙纯合子患儿有出血倾向,出血较轻,多发生在手术后或外伤后,自发性出血少见;偶有皮肤黏膜出血,青春期女性可有月经过多,出血程度与因子Ⅺ浓度无明显关系,患儿常合并因子Ⅴ、因子Ⅶ等凝血因子缺乏。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "血友病丙" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "出血" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "出血" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "手术" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "外伤" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "自发性出血" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "皮肤黏膜" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "皮肤黏膜出血" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "月经过多" } ] }, { "text": "自发性出血少见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "自发性出血" } ] }, { "text": "血友病甲、乙、丙的鉴别可用凝血酶原消耗试验和凝血活酶生成试验的纠正试验来鉴别。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "血友病甲、乙、丙" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "凝血活酶" } ] }, { "text": "本病需注意与vWD相鉴别,后者因vWF质或量的异常引起血小板功能障碍,可借助于阿司匹林耐量试验、血小板对瑞斯托霉素的诱导无凝集反应及vWF因子抗原(vWF∶Ag)测定等鉴别。", "entities": [ { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "血小板" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "血小板功能障碍" }, { "start_idx": 39, "end_idx": 42, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "血小板" }, { "start_idx": 52, "end_idx": 56, "type": "bod", "entity": "瑞斯托霉素" } ] }, { "text": "经常使用血液制品,使患儿易并发肝炎、艾滋病。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "肝炎" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "艾滋病" } ] }, { "text": "高反应者的治疗包括①持续性输入因子Ⅷ,每次100u/kg,每日2次,疗程7~10天;②输入凝血酶原复合物(75u/kg,每日2次)或活化凝血酶原复合物(75u/kg,6~12小时1次),可改善止血功能且止血效果与因子Ⅷ抗体效价无关,但有诱发高凝和血栓的危险;③猪因子Ⅷ浓缩剂,每日20~100u/kg静滴,疗程2~4周;④重组因子Ⅶa,可与组织因子共同作用激活Ⅹ因子,促进凝血活酶的形成。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "因子Ⅷ" }, { "start_idx": 45, "end_idx": 51, "type": "dru", "entity": "凝血酶原复合物" }, { "start_idx": 68, "end_idx": 74, "type": "bod", "entity": "凝血酶原复合物" }, { "start_idx": 106, "end_idx": 110, "type": "bod", "entity": "因子Ⅷ抗体" }, { "start_idx": 120, "end_idx": 121, "type": "dis", "entity": "高凝" }, { "start_idx": 123, "end_idx": 124, "type": "dis", "entity": "血栓" }, { "start_idx": 130, "end_idx": 136, "type": "dru", "entity": "猪因子Ⅷ浓缩剂" }, { "start_idx": 161, "end_idx": 166, "type": "dru", "entity": "重组因子Ⅶa" }, { "start_idx": 186, "end_idx": 189, "type": "bod", "entity": "凝血活酶" } ] }, { "text": "按70~100μg/kg,每2~4小时静脉给药1次;⑤免疫抑制剂,如环磷酰胺。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "静脉给药" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "环磷酰胺" } ] }, { "text": "氨甲环酸(AMCA)5mg/kg,每日3次口服;静脉注射5mg/kg,每日1~2次。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "氨甲环酸" }, { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "AMCA" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "静脉注射" } ] }, { "text": "1938年Wilmer首先提出了融合肾的概念,1957年McDonald和McClellan对其作了修正,并扩展至包括伴或不伴融合的交叉异位肾、孤立交叉异位肾和双侧交叉异位肾。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "融合肾" }, { "start_idx": 66, "end_idx": 70, "type": "dis", "entity": "交叉异位肾" }, { "start_idx": 72, "end_idx": 78, "type": "dis", "entity": "孤立交叉异位肾" }, { "start_idx": 80, "end_idx": 86, "type": "dis", "entity": "双侧交叉异位肾" } ] }, { "text": "2.乙状或S形肾(图12-12B),是第二位常见的融合肾畸形。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "乙状或S形肾" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "融合肾畸形" } ] }, { "text": "4.前后融合肾,也称L型融合(图12-12A)。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "前后融合肾" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "L型融合" } ] }, { "text": "交叉和融合畸形的肾脏,其伴发畸形的发生率不高,最常见的是无肛,其次分别为骨骼畸形、肌肉畸形和心血管畸形。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "bod", "entity": "交叉和融合畸形的肾脏" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "无肛" }, { "start_idx": 41, "end_idx": 50, "type": "sym", "entity": "肌肉畸形和心血管畸形" } ] }, { "text": "由于肾脏位置及其血供异常,其集合系统引流常受影响,易出现尿路感染和结石。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "肾脏" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "出现尿路感染和结石" } ] }, { "text": "早期表现为多尿、烦渴、便秘、厌食和呕吐,多见于5岁以下小儿。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "多尿" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "烦渴" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "便秘" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "厌食" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "呕吐" } ] }, { "text": "(2)远端肾小管缺陷。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "bod", "entity": "远端肾小管" }, { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "远端肾小管缺陷" } ] }, { "text": "(4)髓袢升支粗段缺陷。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "bod", "entity": "髓袢升支粗段" }, { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "髓袢升支粗段缺陷" } ] }, { "text": "(5)为膜缺陷的一部分。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "膜缺陷" } ] }, { "text": "本病临床表现复杂多样,以低血钾症状为主。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "低血钾" } ] }, { "text": "儿童型最常见症状为生长延缓(占51%),其次为肌乏力(41%),还有消瘦(31%)、多尿(28%)、抽搐(26%)以及烦渴(26%)等。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "生长延缓" }, { "start_idx": 23, "end_idx": 25, "type": "sym", "entity": "肌乏力" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "消瘦" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "多尿" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "抽搐" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "烦渴" } ] }, { "text": "成人型最常见症状为肌乏力(40%),其次为疲劳(21%)及抽搐(26%),其他较少见症状有轻瘫,感觉异常,遗尿,夜间多尿,便秘,恶心,呕吐甚至肠梗阻,嗜盐、醋或酸味腌菜,直立性低血压,身材矮小,智力障碍,痛风,高钙尿症,肾钙化,进行性肾衰竭,佝偻病,镁缺乏,红细胞增多症等。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "sym", "entity": "肌乏力" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "疲劳" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "抽搐" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "轻瘫" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "感觉异常" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "遗尿" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "夜间多尿" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "便秘" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "恶心" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "呕吐" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 75, "end_idx": 83, "type": "sym", "entity": "嗜盐、醋或酸味腌菜" }, { "start_idx": 85, "end_idx": 90, "type": "sym", "entity": "直立性低血压" }, { "start_idx": 92, "end_idx": 95, "type": "sym", "entity": "身材矮小" }, { "start_idx": 97, "end_idx": 100, "type": "sym", "entity": "智力障碍" }, { "start_idx": 102, "end_idx": 103, "type": "sym", "entity": "痛风" }, { "start_idx": 105, "end_idx": 108, "type": "dis", "entity": "高钙尿症" }, { "start_idx": 110, "end_idx": 112, "type": "dis", "entity": "肾钙化" }, { "start_idx": 114, "end_idx": 119, "type": "dis", "entity": "进行性肾衰竭" }, { "start_idx": 121, "end_idx": 123, "type": "dis", "entity": "佝偻病" }, { "start_idx": 125, "end_idx": 127, "type": "sym", "entity": "镁缺乏" }, { "start_idx": 129, "end_idx": 134, "type": "dis", "entity": "红细胞增多症" } ] }, { "text": "胎儿期Bartter综合征表现为间歇性发作的多尿,致孕22~24周出现羊水过多,需反复抽羊水,以阻止早产。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "dis", "entity": "胎儿期Bartter综合征" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "间歇性发作的多尿" }, { "start_idx": 26, "end_idx": 38, "type": "sym", "entity": "孕22~24周出现羊水过多" } ] }, { "text": "另有报道血、尿前列腺素增高,缓激肽和肾血管舒缓素排泄增加,尿为低渗性,pH为碱性。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "血" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "尿前列腺素" }, { "start_idx": 4, "end_idx": 12, "type": "sym", "entity": "血、尿前列腺素增高" }, { "start_idx": 14, "end_idx": 27, "type": "sym", "entity": "缓激肽和肾血管舒缓素排泄增加" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "尿" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "尿为低渗性" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "pH为碱性" } ] }, { "text": "有些病人还可出现高血钙、低血磷、低血镁、红细胞内钠浓度增加和钠外流减少,偶有高钙尿症。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "高血钙" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "低血磷" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "低血镁" }, { "start_idx": 20, "end_idx": 28, "type": "sym", "entity": "红细胞内钠浓度增加" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "钠外流减少" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "高钙尿症" } ] }, { "text": "肾活体组织检查可见膜增生性肾小球肾炎、间质性肾炎及肾钙化等病理学改变。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 9, "end_idx": 17, "type": "dis", "entity": "膜增生性肾小球肾炎" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "间质性肾炎" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "肾钙化" } ] }, { "text": "从这些细胞上可见到肾素合成增加的所有征象。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "肾素合成增加" } ] }, { "text": "电镜检查可见粗面内质网和高尔基复合体肥大,并见可能为肾素沉着,肾素合成增加。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "电镜" }, { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "粗面内质网" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "高尔基复合体" }, { "start_idx": 6, "end_idx": 19, "type": "sym", "entity": "粗面内质网和高尔基复合体肥大" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肾素" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "肾素沉着" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肾素" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "肾素合成增加" } ] }, { "text": "免疫细胞化学已确认致密斑细胞萎缩,明显扁平。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "斑细胞" }, { "start_idx": 9, "end_idx": 20, "type": "sym", "entity": "致密斑细胞萎缩,明显扁平" } ] }, { "text": "临床上可按图12-12所示Bartter综合征诊断步骤来逐步确诊该病。", "entities": [ { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "Bartter综合征" } ] }, { "text": "最常见手指呈腊肠样肿胀,但不伴明显硬化,肿胀不过腕,狼疮样皮疹,网状青斑,上眼睑有紫红斑。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "手指" }, { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "手指呈腊肠样肿胀" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "肿胀不过腕" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "狼疮样皮疹" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "网状青斑" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "上眼睑" }, { "start_idx": 37, "end_idx": 43, "type": "sym", "entity": "上眼睑有紫红斑" } ] }, { "text": "半数病人病初即出现雷诺现象,有时为最早出现的症状。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "雷诺现象" } ] }, { "text": "近端肌肉痛、肌肉压痛,乏力。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "近端肌肉" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肌肉" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "近端肌肉痛、肌肉压痛,乏力" } ] }, { "text": "很少见的病例肿瘤可以发生在骨盆和腓骨,发生在肢体远端者较为罕见。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肿瘤" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "骨盆" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "腓骨" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "肢体远端" } ] }, { "text": "根据肿瘤细胞在骨组织中浸润和骨化的特性,成骨肉瘤可以分为成骨性骨肉瘤、成软骨性骨肉瘤和成纤维性骨肉瘤。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "骨组织" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "成骨肉瘤" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "成骨性骨肉瘤" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "成软骨性骨肉瘤" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "成纤维性骨肉瘤" } ] }, { "text": "肿瘤多位于长管状骨的干骺端,边缘不清,骨小梁破坏,肿瘤组织密度增高,穿破骨皮质后肿瘤进一步将骨膜顶起,产生该病具有特征性的X线征象——“Codman三角”,虽然这种现象在部分骨髓炎和尤因肉瘤病人中也可见到,但在成骨肉瘤中则是非常典型的。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "干骺端" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "骨小梁" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "肿瘤组织" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "骨皮质" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "肿瘤" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "骨膜" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "骨髓炎" }, { "start_idx": 91, "end_idx": 94, "type": "dis", "entity": "尤因肉瘤" }, { "start_idx": 105, "end_idx": 108, "type": "dis", "entity": "成骨肉瘤" } ] }, { "text": "虽然,成骨肉瘤目前仍是儿童和青少年中恶性肿瘤死亡率很高的疾病,但早期发现和及时治疗已经从很大程度上提高了该病的生存率。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "成骨肉瘤" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "比较一致的观点是,成骨肉瘤一旦经病理证实,就应该立即开始前期的化学治疗。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "成骨肉瘤" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "化学治疗" } ] }, { "text": "切除肿瘤组织是成骨肉瘤治疗中最为重要和迫切的任务。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "切除肿瘤组织" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "成骨肉瘤" } ] }, { "text": "目前多数学者强调在先期化学治疗之后进行,过去常规采用截肢的方法,近年来,随着骨肿瘤外科技术的提高和人工骨关节植入物研究的发展,以保存肢体为目的的综合疗法显示了较好的应用前景。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "化学治疗" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "截肢" }, { "start_idx": 38, "end_idx": 42, "type": "dep", "entity": "骨肿瘤外科" }, { "start_idx": 49, "end_idx": 56, "type": "pro", "entity": "人工骨关节植入物" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "肢体" } ] }, { "text": "一般在手术治疗施行后继续进行,并做好随访工作,定期检查特别是检查有否转移的发生,并根据病情及时调整治疗方案,必要时也可第二次施行肿瘤的清除手术。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "手术" }, { "start_idx": 64, "end_idx": 65, "type": "dis", "entity": "肿瘤" }, { "start_idx": 69, "end_idx": 70, "type": "pro", "entity": "手术" } ] }, { "text": "由于婴幼儿正处于快速生长阶段,如过多的脂肪摄入和累积会同时增加脂肪细胞的体积和数目,很易导致儿童肥胖,乃至成人期的肥胖病。", "entities": [ { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "儿童肥胖" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "肥胖病" } ] }, { "text": "本症可由多种病因或诱因引起,如婴幼儿气道解剖生理特点、感染(尤其是呼吸道合胞病毒)、过敏体质等。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "气道" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "感染" }, { "start_idx": 33, "end_idx": 39, "type": "mic", "entity": "呼吸道合胞病毒" } ] }, { "text": "临床特点包括:多见于3岁以下小儿,常有湿疹或其他过敏史和家族史;喘息症状明显,可闻及少许中水泡音;易复发,且多与感染有关,但大多预后良好,一般至入学前,复发次数逐渐减少而痊愈,也有少数患儿反复发作,发展成为支气管哮喘;可伴低热,抗生素治疗效果不显著。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "湿疹" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "喘息" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "少许中水泡音" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "感染" }, { "start_idx": 103, "end_idx": 107, "type": "dis", "entity": "支气管哮喘" }, { "start_idx": 111, "end_idx": 112, "type": "sym", "entity": "低热" }, { "start_idx": 114, "end_idx": 116, "type": "dru", "entity": "抗生素" } ] }, { "text": "针对免疫功能低下、过敏体质等诱因可给予免疫调节剂或抗过敏治疗。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "免疫调节剂" }, { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "抗过敏治疗" } ] }, { "text": "以往曾命名为心内膜垫缺损,房室管畸形,共同房室通道等。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "心内膜垫缺损" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "房室管畸形" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "共同房室通道" } ] }, { "text": "房室间隔缺损的形态变异较大,分型方法依据心脏间隔缺损的部位、大小,房室瓣口的形态以及房室瓣附着形态等,不同类型实际为同一病理改变的不同程度。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "房室间隔缺损" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "心脏间隔缺损" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "房室瓣口" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "房室瓣" } ] }, { "text": "均衡型:房室瓣均衡与左、右心室连接;左或右优势:一侧心室发育不良。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "房室瓣" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "左、右心室" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "心室" } ] }, { "text": "2.左或右心房双流出道及房间隔前部偏移造成的心房流出道梗阻,为房间隔和室间隔对位不良所致。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "bod", "entity": "左或右心房双流出道" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "房间隔前部" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "心房流出道梗阻" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "房间隔" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "室间隔" } ] }, { "text": "腱束、瓣叶附着异常、漏斗隔向后移位、异常乳头肌向流出道延伸等均可造成左心室流出道梗阻。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "腱束" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "瓣叶" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "漏斗隔" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "乳头肌" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "流出道" }, { "start_idx": 34, "end_idx": 41, "type": "dis", "entity": "左心室流出道梗阻" } ] }, { "text": "手术治疗的目的是关闭心房内交通,重建左侧房室瓣功能。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "手术治疗" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "左侧房室瓣" } ] }, { "text": "体检可发现心前区搏动明显且弥散,第一心音减弱。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "心前区搏动明显且弥散" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "心音减弱" } ] }, { "text": "单纯的二尖瓣脱垂有可能导致二尖瓣反流,马方综合征时可见二尖瓣黏液样变及脱垂。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "二尖瓣脱垂" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "二尖瓣反流" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "马方综合征" }, { "start_idx": 27, "end_idx": 36, "type": "sym", "entity": "二尖瓣黏液样变及脱垂" } ] }, { "text": "小儿中度二尖瓣反流的治疗仍然以内科治疗为主,包括利尿剂、地高辛及减轻后负荷的药物——血管紧张素转换酶抑制剂的应用。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "中度二尖瓣反流" }, { "start_idx": 15, "end_idx": 16, "type": "dep", "entity": "内科" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "利尿剂" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "地高辛" }, { "start_idx": 42, "end_idx": 52, "type": "dru", "entity": "血管紧张素转换酶抑制剂" } ] }, { "text": "有些患儿还需应用抗心律失常药物。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dru", "entity": "抗心律失常药物" } ] }, { "text": "3.间变大细胞型NHL也可借用本方案。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "间变大细胞型NHL" } ] }, { "text": "有意识障碍时,对外界刺激的反应减低或消失,根据轻重程度可分为嗜睡、意识模糊、昏睡和昏迷等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "意识障碍" }, { "start_idx": 7, "end_idx": 19, "type": "sym", "entity": "对外界刺激的反应减低或消失" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "嗜睡" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "意识模糊" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "昏睡" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "昏迷" } ] }, { "text": "对于有意识障碍的小儿要注意生命体征的改变。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "意识障碍" } ] }, { "text": "精神状态要注意有无烦躁不安、激惹、谵妄或迟钝、抑郁、幻觉及定向障碍等。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "激惹" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "谵妄" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "迟钝" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "抑郁" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "幻觉" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "定向障碍" } ] }, { "text": "头围过大见于脑积水、巨脑症以及某些脑脂质沉积症等,也可能有家族特征。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "头围过大" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑积水" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "巨脑症" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "脑脂质沉积症" } ] }, { "text": "囟门闭合过晚或囟门过大可见于脑积水及软骨营养不良等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "囟门闭合过晚" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "囟门过大" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "脑积水" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "软骨营养不良" } ] }, { "text": "严重的乳头水肿时可伴网膜出血、水肿及渗出。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "乳头水肿" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "网膜出血、水肿及渗出" } ] }, { "text": "网膜周边部有色素变性可见于一些遗传代谢性疾病。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "网膜周边部" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "色素变性" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "遗传代谢性疾病" } ] }, { "text": "三叉神经运动支受刺激时,咀嚼肌强直,出现牙关紧闭,见于破伤风等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "三叉神经运动支" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "咀嚼肌强直" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "牙关紧闭" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "破伤风" } ] }, { "text": "中枢性面神经麻痹时,表现为病变对侧下部面肌麻痹,即只有鼻唇沟变浅,而眼裂变大不能闭眼情况不明显。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "中枢性面神经麻痹" }, { "start_idx": 13, "end_idx": 22, "type": "sym", "entity": "病变对侧下部面肌麻痹" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "鼻唇沟变浅" }, { "start_idx": 34, "end_idx": 46, "type": "sym", "entity": "眼裂变大不能闭眼情况不明显" } ] }, { "text": "感觉性共济失调步态是跨步大,举腿高,落足用力过度,但视觉可以协助平衡,见于脊髓或末梢神经病变。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "跨步大" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "举腿高" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "落足用力过度" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "视觉" }, { "start_idx": 37, "end_idx": 45, "type": "dis", "entity": "脊髓或末梢神经病变" } ] }, { "text": "痉挛性步态见于上运动神经元性瘫痪,下肢伸肌张力高,步态僵直,作拖曳状。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "痉挛性步态" }, { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "上运动神经元性瘫痪" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "下肢伸肌张力高" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "步态僵直" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "作拖曳状" } ] }, { "text": "肌病时骨盆带肌无力,行走慢,左右摇摆如鸭,称“鸭步”。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肌病" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "骨盆带肌无力" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "行走慢" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "左右摇摆如鸭" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "鸭步" } ] }, { "text": "不自主运动主要见于锥体外系疾病,在情绪紧张时或试图保持某一姿势、完成某一动作时加重,入睡后消失。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "锥体外系疾病" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "情绪紧张" } ] }, { "text": "深反射减弱或消失是由于反射弧损伤,也可见于锥体束急性损害或小脑病变。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "深反射减弱或消失" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "反射弧损伤" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "锥体束急性损害" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "小脑病变" } ] }, { "text": "此外,还可见于低钾血症、深睡、昏迷、休克以及用大量镇静药等。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "低钾血症" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "深睡" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "昏迷" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "休克" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "镇静药" } ] }, { "text": "若表现为趾背曲,其他四趾呈扇形分开,称为巴氏征阳性,或伸直性跖反射。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "趾背曲" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "其他四趾呈扇形分开" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "巴氏征阳性" } ] }, { "text": "若趾伸直背曲则为病理反射,意义同巴氏征。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "趾" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "巴氏征" } ] }, { "text": "Cosio等对1078例肾活体组织检查标本的回顾性分析发现,TBMN占成人肾活检患者的5%。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "TBMN" } ] }, { "text": "在成人血尿患者中,TBMN占31%。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "血尿" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "TBMN" } ] }, { "text": "在小儿中,TBMN的发生率更高,它分别占小儿肾活体组织检查患者和小儿血尿患者的9%和50%。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "TBMN" }, { "start_idx": 22, "end_idx": 28, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "小儿血尿" } ] }, { "text": "绝大部分患者以血尿为主要临床表现,其中大部分表现为持续性镜下血尿,少部分患者在上呼吸道感染或剧烈运动后可出现发作性肉眼血尿。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "血尿" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "持续性镜下血尿" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "发作性肉眼血尿" } ] }, { "text": "部分患者在血尿同时伴有轻、中度蛋白尿,偶为肾病范围的大量蛋白尿,还有极少部分患者表现为孤立性蛋白尿。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "血尿" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "轻、中度蛋白尿" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "肾病" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "大量蛋白尿" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "孤立性蛋白尿" } ] }, { "text": "呼吸性酸中毒时常伴有低氧血症及呼吸困难。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "呼吸性酸中毒" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "低氧血症" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "高碳酸血症可引起血管扩张,颅内血流增加,致头痛及颅内压增高,严重高碳酸血症可出现中枢抑制,血pH降低。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "血管扩张" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "颅内血流增加" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "头痛" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "高碳酸血症" }, { "start_idx": 45, "end_idx": 47, "type": "ite", "entity": "血pH" }, { "start_idx": 40, "end_idx": 49, "type": "sym", "entity": "中枢抑制,血pH降低" } ] }, { "text": "肿瘤多生长在皮下组织内,可达肌肉。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "皮下组织" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "肌肉" } ] }, { "text": "可发生于身体任何部位,以四肢、躯干多见。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "四肢" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "躯干" } ] }, { "text": "目前对海绵状血管瘤的有效疗法主要是手术和激素疗法。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "海绵状血管瘤" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "手术" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "激素疗法" } ] }, { "text": "若胸膜腔内同时有脓液存在则称为脓气胸(pyopneumothorax)。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "胸膜腔" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "有脓液存在" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "脓气胸" }, { "start_idx": 19, "end_idx": 33, "type": "dis", "entity": "pyopneumothorax" } ] }, { "text": "根据病因可分为原发性气胸和继发性气胸两大类。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "原发性气胸" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "继发性气胸" } ] }, { "text": "原发性气胸原因不明,多发生于青少年和成人,尤其是体型高而瘦的男性。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "原发性气胸" } ] }, { "text": "小儿气胸多急性起病,一般在原发病的基础上突然出现烦躁、咳嗽、气急及呼吸困难等症状,或原有的呼吸困难等症状突然加重;年长儿可诉胸闷、胸痛。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "小儿气胸" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "烦躁" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "咳嗽" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "气急" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 42, "end_idx": 55, "type": "sym", "entity": "原有的呼吸困难等症状突然加重" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "胸闷" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "胸痛" } ] }, { "text": "张力性气胸时,由于大量气体积聚,不但肺组织受压,而且纵隔严重移位,导致腔静脉回流障碍,易引起严重的心肺功能障碍,表现烦躁、发绀、全身冷汗、脉搏细速、血压下降等休克症状,甚至出现意识不清、昏迷等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "张力性气胸" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肺组织" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "纵隔" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "心肺" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "烦躁" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "发绀" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "全身冷汗" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "脉搏细速" }, { "start_idx": 74, "end_idx": 77, "type": "sym", "entity": "血压下降" }, { "start_idx": 79, "end_idx": 80, "type": "sym", "entity": "休克" }, { "start_idx": 93, "end_idx": 94, "type": "sym", "entity": "昏迷" } ] }, { "text": "典型体征为患侧胸部饱满、呼吸运动减弱或消失、叩诊呈鼓音、触觉语颤及听诊呼吸音减弱或消失,气管及纵隔移向对侧。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "患侧胸部饱满" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "呼吸运动减弱或消失" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "叩诊呈鼓音" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "触觉语颤" }, { "start_idx": 33, "end_idx": 42, "type": "sym", "entity": "听诊呼吸音减弱或消失" }, { "start_idx": 44, "end_idx": 52, "type": "sym", "entity": "气管及纵隔移向对侧" } ] }, { "text": "脓气胸者可有明显的中毒症状,体格检查患侧叩诊呈鼓音或浊音,且随体位的变化而有变化。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脓气胸" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "中毒" }, { "start_idx": 14, "end_idx": 17, "type": "ite", "entity": "体格检查" } ] }, { "text": "本病常根据胸部X线所见做出诊断。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "ite", "entity": "胸部X线" } ] }, { "text": "本病应与肺大疱、大叶性肺气肿、先天性含气肺囊肿等鉴别。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "肺大疱" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "大叶性肺气肿" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "先天性含气肺囊肿" } ] }, { "text": "少量闭合性或开放性气胸、肺压缩程度<20%者,可让患儿卧床休息,气体大多在2~4周内被吸收。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "闭合性或开放性气胸" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "肺" } ] }, { "text": "维生素D不足将导致维生素D缺乏性佝偻病,这是一种慢性营养缺乏病,主要见于3岁以下婴幼儿。", "entities": [ { "start_idx": 9, "end_idx": 18, "type": "dis", "entity": "维生素D缺乏性佝偻病" } ] }, { "text": "它以维生素D缺乏导致的钙、磷代谢紊乱和骨骼的钙化障碍为主要特征。", "entities": [ { "start_idx": 11, "end_idx": 25, "type": "sym", "entity": "钙、磷代谢紊乱和骨骼的钙化障碍" } ] }, { "text": "小儿胆汁淤积、胆总管扩张、先天性胆道狭窄或闭锁、脂肪泻、胰腺炎、难治性腹泻等疾病均可影响维生素D、钙、磷的吸收而患佝偻病。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "小儿胆汁淤积" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "胆总管扩张" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "先天性胆道狭窄或闭锁" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "脂肪泻" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "难治性腹泻" } ] }, { "text": "8~9个月以上的患儿头颅常呈方形,前囟大及闭合延迟,严重者18个月时前囟尚未闭合。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "头颅" }, { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "患儿头颅常呈方形" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "前囟" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "前囟大及闭合延迟" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "前囟尚未闭合" } ] }, { "text": "患儿的肌肉韧带松弛无力,因腹部肌肉软弱而使腹部膨大,平卧时呈“蛙状腹”,因四肢肌肉无力,学会坐、站、走的年龄都较晚,因两腿无力容易跌跤。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "肌肉韧带" }, { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "肌肉韧带松弛无力" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "腹部肌肉" }, { "start_idx": 13, "end_idx": 24, "type": "sym", "entity": "腹部肌肉软弱而使腹部膨大" }, { "start_idx": 44, "end_idx": 56, "type": "sym", "entity": "学会坐、站、走的年龄都较晚" } ] }, { "text": "大脑皮层功能异常,条件反射形成缓慢,患儿表情淡漠,语言发育迟缓,免疫力低下,易并发感染、贫血。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "大脑皮层" }, { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "大脑皮层功能异常,条件反射形成缓慢" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "患儿表情淡漠" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "语言发育迟缓" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "免疫力低下" }, { "start_idx": 38, "end_idx": 45, "type": "sym", "entity": "易并发感染、贫血" } ] }, { "text": "反复气道吸引可能降低MAS临床危重程度,但是经反复吸引的MAS发展为依赖呼吸机治疗的情况仍比较普遍。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "气道吸引" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "MAS" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "MAS" }, { "start_idx": 36, "end_idx": 38, "type": "equ", "entity": "呼吸机" } ] }, { "text": "如果自主呼吸比较强烈,有烦躁不安,也可以用SIMV+PSV或PSV模式通气,可以降低平均气道压(MAP),可以减少肺泡压力差剧烈变化导致的气胸。", "entities": [ { "start_idx": 42, "end_idx": 46, "type": "ite", "entity": "平均气道压" }, { "start_idx": 48, "end_idx": 50, "type": "ite", "entity": "MAP" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "肺泡" }, { "start_idx": 69, "end_idx": 70, "type": "dis", "entity": "气胸" } ] }, { "text": "Findlay等应用牛肺肺表面活性物质制剂(Survanta)随机对照治疗40例MAS得到显著临床效果。", "entities": [ { "start_idx": 10, "end_idx": 20, "type": "dru", "entity": "牛肺肺表面活性物质制剂" }, { "start_idx": 22, "end_idx": 29, "type": "dru", "entity": "Survanta" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "MAS" } ] }, { "text": "部分患儿由于母体的自身抗体,生后即出现短暂的皮肤及血液改变和持续的心脏异常等。", "entities": [ { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "皮肤" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "血液" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "心脏" } ] }, { "text": "此外,患儿常伴有心内膜弹力纤维增生症和其他先天性心脏病如动脉导管未闭以及大动脉转位等。", "entities": [ { "start_idx": 8, "end_idx": 17, "type": "dis", "entity": "心内膜弹力纤维增生症" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "大动脉转位" } ] }, { "text": "血液系统可出现暂时性的白细胞减少,于出生时即存在,可持续数日至数周。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "血液" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "白细胞" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "白细胞减少" } ] }, { "text": "有时仅出现皮肤出血点,胃肠道出血偶有发生。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "胃肠道" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "胃肠道出血偶有发生" } ] }, { "text": "此外,还可有肝脏肿大、转氨酶增高和胆汁淤滞性黄疸。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "肝脏" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "肝脏肿大" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "转氨酶增高" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "胆汁淤滞性黄疸" } ] }, { "text": "伴心脏损害者病死率5%~30%。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "心脏" } ] }, { "text": "新生儿期出现严重心动过缓,应使用起搏器。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "严重心动过缓" }, { "start_idx": 16, "end_idx": 18, "type": "equ", "entity": "起搏器" } ] }, { "text": "小儿口炎在婴幼儿较多见,可以单独发病,也可继发于腹泻、营养不良、急性感染和久病体弱等全身性疾病。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "小儿口炎" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "腹泻" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "营养不良" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "急性感染" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "久病体弱" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "全身性疾病" } ] }, { "text": "根据致病因素分为感染性口炎和非感染性口炎。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "感染性口炎" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "非感染性口炎" } ] }, { "text": "但当内外环境发生变化,身体防御能力下降时,如感冒、发热、感染、滥用抗生素及(或)肾上腺皮质激素、化疗和放疗等,口腔内细菌增殖活跃,毒力增强,菌群关系失调,就可发病。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "身体防御能力下降" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "感冒" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "发热" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "感染" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "抗生素" }, { "start_idx": 40, "end_idx": 46, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 48, "end_idx": 49, "type": "pro", "entity": "化疗" }, { "start_idx": 51, "end_idx": 52, "type": "pro", "entity": "放疗" }, { "start_idx": 55, "end_idx": 63, "type": "sym", "entity": "口腔内细菌增殖活跃" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "毒力增强" }, { "start_idx": 70, "end_idx": 75, "type": "sym", "entity": "菌群关系失调" } ] }, { "text": "致病菌主要包括链球菌、金黄色葡萄球菌及肺炎球菌等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "致病菌" }, { "start_idx": 7, "end_idx": 9, "type": "mic", "entity": "链球菌" }, { "start_idx": 11, "end_idx": 17, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 19, "end_idx": 22, "type": "mic", "entity": "肺炎球菌" } ] }, { "text": "涂片可见大量葡萄球菌,细菌培养可明确诊断。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "涂片可见大量葡萄球菌" } ] }, { "text": "全身症状明显,常并发有链球菌性咽炎。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "全身症状明显,常并发有链球菌性咽炎" } ] }, { "text": "苔膜涂片或细菌培养检查发现链球菌,即可确诊。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "苔膜涂片或细菌培养检查发现链球菌" } ] }, { "text": "溃疡好发于牙龈和颊黏膜,形态不定,大小多在1cm左右,表浅,披以污秽的、灰白色苔膜,擦去此苔膜时,出现溢血的溃疡面,但不久又再被覆以同样的苔膜,周围黏膜有明显充血水肿,触痛明显,并有特别强烈的坏死组织臭味。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "牙龈" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "颊黏膜" }, { "start_idx": 42, "end_idx": 56, "type": "sym", "entity": "擦去此苔膜时,出现溢血的溃疡面" }, { "start_idx": 59, "end_idx": 101, "type": "sym", "entity": "不久又再被覆以同样的苔膜,周围黏膜有明显充血水肿,触痛明显,并有特别强烈的坏死组织臭味" } ] }, { "text": "此病确诊的依据为特殊性口臭,苔膜与小溃疡,涂片中找到大量梭形杆菌与奋森螺旋体。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "特殊性口臭" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "苔膜与小溃疡" }, { "start_idx": 28, "end_idx": 31, "type": "mic", "entity": "梭形杆菌" }, { "start_idx": 33, "end_idx": 37, "type": "mic", "entity": "奋森螺旋体" } ] }, { "text": "全身抗感染治疗可给予广谱抗生素如青霉素、红霉素及交沙霉素等。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "广谱抗生素" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "青霉素" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "红霉素" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "交沙霉素" } ] }, { "text": "①肺动脉狭窄:肺动脉狭窄是四联症最重要的病变,狭窄范围自右心室漏斗部入口至左、右肺动脉分支。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "四联症" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "右心室" }, { "start_idx": 37, "end_idx": 42, "type": "bod", "entity": "左、右肺动脉" } ] }, { "text": "其中以漏斗部及肺动脉瓣复合狭窄多见,狭窄的严重程度差异颇大,严重者肺动脉闭锁,可同时伴动脉导管未闭或主动脉与肺动脉间侧支循环血管;②室间隔缺损:为大型、对位不良型室间隔缺损;③主动脉骑跨:主动脉根部粗大且顺钟向旋转右移并骑跨在室间隔缺损上,骑跨范围在15%~95%;④右心室肥厚:属继发性病变。", "entities": [ { "start_idx": 3, "end_idx": 14, "type": "sym", "entity": "漏斗部及肺动脉瓣复合狭窄" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "肺动脉闭锁" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "动脉导管未闭" }, { "start_idx": 50, "end_idx": 63, "type": "sym", "entity": "主动脉与肺动脉间侧支循环血管" }, { "start_idx": 66, "end_idx": 70, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 81, "end_idx": 85, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 88, "end_idx": 92, "type": "dis", "entity": "主动脉骑跨" }, { "start_idx": 94, "end_idx": 96, "type": "bod", "entity": "主动脉" }, { "start_idx": 113, "end_idx": 117, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 134, "end_idx": 138, "type": "dis", "entity": "右心室肥厚" } ] }, { "text": "在动脉导管关闭前,肺循环血流量减少程度较轻,青紫可不明显,随着动脉导管的关闭和漏斗部狭窄的逐渐加重,青紫日益明显,并出现杵状指(趾)。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "动脉" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "青紫" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "动脉" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "漏斗部" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "青紫" }, { "start_idx": 60, "end_idx": 65, "type": "sym", "entity": "杵状指(趾)" } ] }, { "text": "多见于毛细血管丰富的浅表部位,如唇、指(趾)甲床、球结合膜等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "毛细血管" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "唇" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "指(趾)甲床" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "球结合膜" } ] }, { "text": "因血氧含量下降,活动耐力差,稍一活动如啼哭、情绪激动、体力劳动、寒冷等,即可出现气急及青紫加重。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "ite", "entity": "血氧含量" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "啼哭" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "情绪激动" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "体力劳动" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "寒冷" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "气急" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "青紫" } ] }, { "text": "纠正酸中毒,给予5%碳酸氢钠15~50ml/kg静注,以往常有缺氧发作者,可口服普萘洛尔1~2mg/(kg•d)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "酸中毒" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "缺氧" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "普萘洛尔" } ] }, { "text": "年龄过小的婴幼儿可先行姑息分流手术,对重症的患儿也宜先行姑息手术,待年长后一般情况改善,肺血管发育好转后,再作根治术。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "姑息分流手术" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "姑息手术" }, { "start_idx": 44, "end_idx": 46, "type": "bod", "entity": "肺血管" } ] }, { "text": "根据出血部位的不同,可将消化道出血分为上消化道出血及下消化道出血。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "出血" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "消化道出血" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "上消化道出血" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "下消化道出血" } ] }, { "text": "下消化道出血常见原因:坏死性小肠结肠炎、肠重复畸形、肠套叠以及先天性巨结肠。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "下消化道出血" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "坏死性小肠结肠炎" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "肠重复畸形" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "肠套叠" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "先天性巨结肠" } ] }, { "text": "下消化道:细菌性肠炎、炎症性肠道疾病、息肉及痔。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "下消化道" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "细菌性肠炎" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "炎症性肠道疾病" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "息肉" }, { "start_idx": 22, "end_idx": 22, "type": "dis", "entity": "痔" } ] }, { "text": "黑便代表出血来自上消化道或小肠,大便颜色呈黑色、柏油样,黑便颜色受血液在肠道内停留时间长短影响,当出血量较大、出血速度较快及肠蠕动亢进时,粪便可呈暗红色甚至鲜红色,酷似下消化道出血;相反,空、回肠出血,如出血量不多、在肠内停留时间长,也可表现为黑便。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "出血" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "上消化道" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "小肠" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "大便" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "血液" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肠道" }, { "start_idx": 62, "end_idx": 62, "type": "bod", "entity": "肠" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "粪便" }, { "start_idx": 84, "end_idx": 89, "type": "dis", "entity": "下消化道出血" }, { "start_idx": 94, "end_idx": 99, "type": "dis", "entity": "空、回肠出血" }, { "start_idx": 109, "end_idx": 109, "type": "bod", "entity": "肠" }, { "start_idx": 122, "end_idx": 123, "type": "bod", "entity": "黑便" } ] }, { "text": "便血是指大便呈鲜红或深红褐色,出血部位多位于结肠,但是在上消化道大量出血时,由于血液有轻泻作用,会缩短排泄时间,使得大便呈鲜红色。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "大便" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "结肠" }, { "start_idx": 28, "end_idx": 35, "type": "dis", "entity": "上消化道大量出血" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "血液" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "大便" } ] }, { "text": "如为药物引起的消化道黏膜病变应及时停用药物;维生素K缺乏出血症应补充维生素K;如门脉高压症、溃疡病合并穿孔等应及早手术治疗;血液系统疾病应给予纠正出、凝血障碍机制药,如立止血及冻干凝血酶原复合物。", "entities": [ { "start_idx": 22, "end_idx": 30, "type": "dis", "entity": "维生素K缺乏出血症" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "溃疡病" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "穿孔" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "血液系统疾病" } ] }, { "text": "硬化剂治疗:是目前已建立的最好的治疗食道静脉曲张破裂出血治疗方法,该方法的安全性及有效性已被证实,且费用低廉,适用范围广,操作简单。", "entities": [ { "start_idx": 18, "end_idx": 27, "type": "dis", "entity": "食道静脉曲张破裂出血" } ] }, { "text": "近年来,随着我国围产医学及新生儿重症监护技术的飞速进展,早产儿的成活率越来越高,脑瘫的发生率亦有增加趋势,早产儿脑白质损伤日益受到重视。", "entities": [ { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "脑瘫" }, { "start_idx": 53, "end_idx": 60, "type": "dis", "entity": "早产儿脑白质损伤" } ] }, { "text": "非囊性PVL是目前早产儿脑白质损伤的主要类型,可导致脑瘫和认知功能障碍,而弥漫性脑白质病变相对损伤较轻。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "非囊性PVL" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "脑瘫" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "认知功能障碍" } ] }, { "text": "新生儿期可有下肢肌张力降低、颈部伸肌张力增高、呼吸暂停和心率缓慢发作、激惹和喂养困难等,部分患儿可出现惊厥。", "entities": [ { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "下肢肌张力降低" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "颈部伸肌张力增高" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "心率缓慢" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "激惹" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "喂养困难" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "惊厥" } ] }, { "text": "病变累及近三角区、枕角视放射区和颞角听放射区时常表现为视觉和听觉功能障碍。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "近三角区" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "枕角视放射区" }, { "start_idx": 16, "end_idx": 21, "type": "bod", "entity": "颞角听放射区" }, { "start_idx": 27, "end_idx": 35, "type": "sym", "entity": "视觉和听觉功能障碍" } ] }, { "text": "严重弥漫性脑白质病变不仅累及运动功能,还可因皮质及皮质下神经元受损、星形胶质细胞迁移障碍,导致认知缺陷,感觉功能障碍。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "弥漫性脑白质病变" }, { "start_idx": 22, "end_idx": 32, "type": "sym", "entity": "皮质及皮质下神经元受损" }, { "start_idx": 34, "end_idx": 39, "type": "bod", "entity": "星形胶质细胞" }, { "start_idx": 34, "end_idx": 43, "type": "sym", "entity": "星形胶质细胞迁移障碍" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "认知缺陷" }, { "start_idx": 52, "end_idx": 57, "type": "sym", "entity": "感觉功能障碍" } ] }, { "text": "手术完全切除是唯一的治疗方法。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "手术完全切除" } ] }, { "text": "有50%在幼儿期复发,需再手术,但从不发生转移。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "手术" } ] }, { "text": "我国有不同地区的学者通过对正常新生儿血清胆红素水平的动态监测,证实我国正常新生儿生理性黄疸时其血清胆红素峰值高于传统的诊断水平,故需要进行更大样本的前瞻性研究,才能得出我国新生儿生理性黄疸的诊断标准。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "血清胆红素" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "生理性黄疸" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "血清胆红素" }, { "start_idx": 89, "end_idx": 93, "type": "dis", "entity": "生理性黄疸" } ] }, { "text": "多在前额、上眼睑、鼻孔、枕等部位,压迫可褪色,不会自然消失。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "鼻孔" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "枕" } ] }, { "text": "近年来,国外多采用手术治疗,如面积大,可分期手术,并可植皮。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "手术治疗" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "植皮" } ] }, { "text": "好发于面、臂、手和躯干上部,压迫中央可使蜘蛛痣消失。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "面" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "臂" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "手" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "躯干" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "蜘蛛痣" } ] }, { "text": "通常在生后不久出现,肿瘤常高于皮肤,鲜红色,分叶,形如草莓。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "皮肤" } ] }, { "text": "好发于面、头皮和颈部。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "面" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "头皮" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "颈部" } ] }, { "text": "也可用手术、冷冻、硬化剂、低电压、短距离放疗等方法。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "手术" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "冷冻" }, { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "硬化剂" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "低电压" }, { "start_idx": 17, "end_idx": 21, "type": "pro", "entity": "短距离放疗" } ] }, { "text": "临床特征为应用脂肪乳剂期间,患儿出现头痛、呕吐、贫血、血小板下降、凝血酶原时间延长、自发性出血、DIC及肝功能损害(表现为肝大、黄疸和血GPT升高)等,有作者称上述表现为脂肪超载综合征。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "头痛" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "呕吐" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "贫血" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "血小板下降" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "凝血酶原时间延长" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "自发性出血" }, { "start_idx": 48, "end_idx": 50, "type": "sym", "entity": "DIC" }, { "start_idx": 52, "end_idx": 56, "type": "sym", "entity": "肝功能损害" }, { "start_idx": 61, "end_idx": 72, "type": "sym", "entity": "肝大、黄疸和血GPT升高" }, { "start_idx": 85, "end_idx": 91, "type": "dis", "entity": "脂肪超载综合征" } ] }, { "text": "②禁食作用:PNAC的发生率随禁食时间的延长而增加,多数病例在PN进行2~10周后发生。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "禁食作用" } ] }, { "text": "常由b型流感嗜血杆菌引起。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "mic", "entity": "b型流感嗜血杆菌" } ] }, { "text": "喉镜检查可发现肿胀增大的樱红色会厌,杓会厌皱襞,杓状软骨和喉室带均充血水肿。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "喉镜检查" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "肿胀增大的樱红色会厌" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "杓会厌皱襞" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "杓状软骨" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "喉室带" }, { "start_idx": 18, "end_idx": 36, "type": "sym", "entity": "杓会厌皱襞,杓状软骨和喉室带均充血水肿" } ] }, { "text": "多数患儿存在菌血症,应立即给予肠道外抗生素治疗,如头孢噻肟、头孢曲松、氨苄西林/舒巴坦等,抗生素持续应用7~10天。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "菌血症" }, { "start_idx": 15, "end_idx": 20, "type": "dru", "entity": "肠道外抗生素" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "头孢噻肟" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "头孢曲松" }, { "start_idx": 35, "end_idx": 42, "type": "dru", "entity": "氨苄西林/舒巴坦" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "抗生素" } ] }, { "text": "除镇静、及时吸氧外,为减轻会厌部水肿,缓解呼吸困难,可立即静脉注射地塞米松等激素,必要时肾上腺素、布地奈德雾化吸入。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "pro", "entity": "镇静" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "吸氧" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "会厌部" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "会厌部水肿" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "静脉注射" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "地塞米松" }, { "start_idx": 44, "end_idx": 47, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 49, "end_idx": 52, "type": "dru", "entity": "布地奈德" }, { "start_idx": 53, "end_idx": 56, "type": "pro", "entity": "雾化吸入" } ] }, { "text": "本病常为败血症的一部分或继发于败血症,但也可作为一种局部感染而存在。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "败血症" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "败血症" } ] }, { "text": "主要发生在儿童时期,是常见的危害生命的感染性疾病之一,迄今仍具有较高的死亡率与致残率。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "感染性疾病" }, { "start_idx": 32, "end_idx": 41, "type": "sym", "entity": "较高的死亡率与致残率" } ] }, { "text": "院内感染的细菌主要有克雷伯杆菌、沙门杆菌、肠杆菌、绿脓杆菌、黄质菌以及沙雷菌等。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "mic", "entity": "克雷伯杆菌" }, { "start_idx": 16, "end_idx": 19, "type": "mic", "entity": "沙门杆菌" }, { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "肠杆菌" }, { "start_idx": 25, "end_idx": 28, "type": "mic", "entity": "绿脓杆菌" }, { "start_idx": 30, "end_idx": 32, "type": "mic", "entity": "黄质菌" }, { "start_idx": 35, "end_idx": 37, "type": "mic", "entity": "沙雷菌" } ] }, { "text": "2006年在复旦大学附属儿童医院进行的化脑病原学流行病学研究,最后提出肺炎链球菌、B型流感嗜血杆菌及脑膜炎奈瑟菌仍是上海地区化脑儿童的主要病原菌。", "entities": [ { "start_idx": 35, "end_idx": 39, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 41, "end_idx": 48, "type": "mic", "entity": "B型流感嗜血杆菌" }, { "start_idx": 50, "end_idx": 55, "type": "mic", "entity": "脑膜炎奈瑟菌" }, { "start_idx": 69, "end_idx": 71, "type": "mic", "entity": "病原菌" } ] }, { "text": "大多数脑膜炎病例是由血行播散引起。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "也可由脑脊膜膨出、神经管缺损、先天性窦道、胎儿头皮采血标本穿透伤或因胎内心电图监测致邻近播散所引起。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "脑脊膜膨出" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "神经管缺损" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "先天性窦道" }, { "start_idx": 21, "end_idx": 31, "type": "sym", "entity": "胎儿头皮采血标本穿透伤" }, { "start_idx": 34, "end_idx": 38, "type": "pro", "entity": "胎内心电图" } ] }, { "text": "另外少数是由病原菌直接侵入脑膜引起,如肺炎链球菌脑膜炎。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "病原菌" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "肺炎链球菌脑膜炎" } ] }, { "text": "特殊表现有:①神志改变:烦躁易激惹、惊跳、突然尖叫和嗜睡、神萎等;②颅内压增高:前囟紧张、饱满或隆起、骨缝分离,由于新生儿颈肌发育很差,颈项强直较少见;③惊厥:表现不典型,可仅见双眼凝视、斜视、眼球上翻及眼睑抽动,面肌小抽如吸吮状,也可阵发性青紫及呼吸暂停,一侧或局部肢体抽动;④败血症的表现如黄疸、肝大、腹胀及休克等。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "神志改变" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "烦躁易激惹" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "惊跳" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "突然尖叫" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "嗜睡" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "神萎" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "前囟紧张" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "饱满" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "隆起" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "骨缝分离" }, { "start_idx": 77, "end_idx": 78, "type": "sym", "entity": "惊厥" }, { "start_idx": 89, "end_idx": 92, "type": "sym", "entity": "双眼凝视" }, { "start_idx": 94, "end_idx": 95, "type": "sym", "entity": "斜视" }, { "start_idx": 97, "end_idx": 100, "type": "sym", "entity": "眼球上翻" }, { "start_idx": 102, "end_idx": 105, "type": "sym", "entity": "眼睑抽动" }, { "start_idx": 107, "end_idx": 110, "type": "sym", "entity": "面肌小抽" }, { "start_idx": 112, "end_idx": 114, "type": "sym", "entity": "吸吮状" }, { "start_idx": 118, "end_idx": 122, "type": "sym", "entity": "阵发性青紫" }, { "start_idx": 124, "end_idx": 127, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 129, "end_idx": 137, "type": "sym", "entity": "一侧或局部肢体抽动" }, { "start_idx": 140, "end_idx": 142, "type": "dis", "entity": "败血症" }, { "start_idx": 147, "end_idx": 148, "type": "sym", "entity": "黄疸" }, { "start_idx": 150, "end_idx": 151, "type": "sym", "entity": "肝大" }, { "start_idx": 153, "end_idx": 154, "type": "sym", "entity": "腹胀" }, { "start_idx": 156, "end_idx": 157, "type": "sym", "entity": "休克" } ] }, { "text": "2岁以上小儿出现①发热、头痛;②惊厥、意识改变;③脑膜刺激征或神经局灶症状,均应考虑化脑的可能。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "发热" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "头痛" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "惊厥" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "意识改变" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "脑膜刺激征" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "神经局灶症状" } ] }, { "text": "临床可有以下表现:化脓性脑膜炎患儿经常规治疗后,疗效和化验结果不见好转;病情危重,频繁惊厥,出现呼吸衰竭或脑疝;脑脊液培养出少见细菌(大肠杆菌、流感杆菌,以及变形杆菌等);颅内压增高,已排除硬脑膜下积液及化脓性脑膜炎复发者。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "化脓性脑膜炎" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "病情危重" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "频繁惊厥" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "呼吸衰竭" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "脑疝" }, { "start_idx": 56, "end_idx": 58, "type": "bod", "entity": "脑脊液" }, { "start_idx": 67, "end_idx": 70, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 72, "end_idx": 75, "type": "mic", "entity": "流感杆菌" }, { "start_idx": 79, "end_idx": 82, "type": "mic", "entity": "变形杆菌" }, { "start_idx": 86, "end_idx": 90, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 95, "end_idx": 100, "type": "dis", "entity": "硬脑膜下积液" }, { "start_idx": 102, "end_idx": 107, "type": "dis", "entity": "化脓性脑膜炎" } ] }, { "text": "确诊必须行脑室穿刺术取脑脊液", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "穿刺术" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "脑脊" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脑脊" } ] }, { "text": "根据目前国内检出病原(肺炎链球菌、脑膜炎双球菌及流感杆菌为主),首选头孢三嗪或头孢噻肟,头孢三嗪[100mg/(kg•d),分2次],具有广谱、高效、半衰期长、对革兰阴性杆菌作用效果好以及使用方便等优点,已成为治疗婴幼儿化脓性脑膜炎的常用药物,但其可与胆红素竞争白蛋白,有增加核黄疸的危险,在新生儿黄疸时少用。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 17, "end_idx": 22, "type": "mic", "entity": "脑膜炎双球菌" }, { "start_idx": 24, "end_idx": 27, "type": "mic", "entity": "流感杆菌" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "头孢三嗪" }, { "start_idx": 39, "end_idx": 42, "type": "dru", "entity": "头孢噻肟" }, { "start_idx": 44, "end_idx": 47, "type": "dru", "entity": "头孢三嗪" }, { "start_idx": 81, "end_idx": 86, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 110, "end_idx": 115, "type": "dis", "entity": "化脓性脑膜炎" }, { "start_idx": 138, "end_idx": 140, "type": "dis", "entity": "核黄疸" }, { "start_idx": 149, "end_idx": 150, "type": "dis", "entity": "黄疸" } ] }, { "text": "对其过敏者,用美罗培南替代治疗。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "美罗培南" } ] }, { "text": "建议①流感嗜血杆菌脑膜炎推荐使用;②大于6周龄的肺炎链球菌脑膜炎患儿,权衡利弊再考虑使用;③由其他病菌引起的脑膜炎,不建议常规使用高剂量地塞米松;④部分治疗后脑膜炎,耐β内酰胺酶的肺炎链球菌脑膜炎以及小于6周龄的化脑均不宜使用糖皮质激素治疗。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "流感嗜血杆菌脑膜炎" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "肺炎链球菌脑膜炎" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 68, "end_idx": 71, "type": "dru", "entity": "地塞米松" }, { "start_idx": 79, "end_idx": 81, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 90, "end_idx": 91, "type": "dis", "entity": "肺炎" }, { "start_idx": 92, "end_idx": 97, "type": "dis", "entity": "链球菌脑膜炎" }, { "start_idx": 113, "end_idx": 117, "type": "dru", "entity": "糖皮质激素" } ] }, { "text": "静脉输注丙种球蛋白的不良反应有皮肤潮红、恶心、呕吐、头痛以及呼吸短促等过敏反应,通常发生在输液早期,而且与静注速度有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "静脉输注" }, { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "丙种球蛋白" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "皮肤潮红" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "恶心" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "呕吐" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "头痛" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "呼吸短促" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "过敏反应" } ] }, { "text": "外周血异型淋巴细胞(简称异淋)增多,脑脊液蛋白增高和血清肝酶增高。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "异淋" }, { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "外周血异型淋巴细胞(简称异淋)增多" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "脑脊液蛋白" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "脑脊液蛋白增高" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "血清肝酶" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "血清肝酶增高" } ] }, { "text": "部分患儿在婴儿期出现感觉神经性耳聋和神经肌肉功能障碍,如肌张力低下、瘫痪和癫痫发作等。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "感觉神经性耳聋" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "神经肌肉" }, { "start_idx": 18, "end_idx": 25, "type": "sym", "entity": "神经肌肉功能障碍" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "瘫痪" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "癫痫" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "癫痫发作" } ] }, { "text": "HCMV还可致畸,以腹股沟疝最多见,其他如腭裂、胆道闭锁、心血管畸形和多囊肾等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "HCMV" }, { "start_idx": 7, "end_idx": 7, "type": "dis", "entity": "畸" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "腹股沟疝" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "腭裂" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "胆道闭锁" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "心血管畸形" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "多囊肾" } ] }, { "text": "约90%有后遗症,包括智力障碍、耳聋、神经缺陷和眼部异常等。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "智力障碍" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "耳聋" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "神经缺陷" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "眼部异常" } ] }, { "text": "亦可有肺炎征象,甚至呼吸衰竭。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肺炎" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "肺炎征象" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "呼吸衰竭" } ] }, { "text": "由于HCMV致病力弱,免疫正常时无论原发或再发感染,绝大多数无症状,故在免疫正常个体应谨慎诊断HCMV疾病。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "mic", "entity": "HCMV" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "原发或再发感染" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "HCMV疾病" } ] }, { "text": "在CID时,应与其他宫内感染如先天性风疹,弓形虫、梅毒螺旋体、单纯疱疹病毒等感染相鉴别。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "宫内感染" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "先天性风疹" }, { "start_idx": 21, "end_idx": 23, "type": "mic", "entity": "弓形虫" }, { "start_idx": 25, "end_idx": 29, "type": "mic", "entity": "梅毒螺旋体" }, { "start_idx": 31, "end_idx": 36, "type": "mic", "entity": "单纯疱疹病毒" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "感染" } ] }, { "text": "HCMV引起的传染性单核细胞增多症应与其他病原,特别是EBV引起者鉴别。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "HCMV" }, { "start_idx": 7, "end_idx": 16, "type": "dis", "entity": "传染性单核细胞增多症" }, { "start_idx": 27, "end_idx": 29, "type": "mic", "entity": "EBV" } ] }, { "text": "后者咽痛、渗出性咽炎和淋巴结肿大更为多见,嗜异性抗体常为阳性。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "咽痛" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "渗出性咽炎" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "淋巴结" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "淋巴结肿大" } ] }, { "text": "常先有急性鼻咽炎病史,继而骤起高热、吞咽困难、拒食、呼吸困难、流涎、烦躁。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "急性鼻咽炎" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "骤起高热" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "吞咽困难" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "拒食" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "流涎" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "烦躁" } ] }, { "text": "哭声含糊不清;睡眠时打鼾、呼吸不畅。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "哭声含糊不清" }, { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "睡眠时打鼾、呼吸不畅" } ] }, { "text": "本病应与喉气管支气管炎引起的喉喘鸣、颈椎结核等相鉴别。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "喉气管支气管炎" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "喉喘鸣" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "颈椎结核" } ] }, { "text": "应给予适当的抗生素控制感染,首选耐酶青霉素类,亦可应用克林霉素或含β-内酰胺酶抑制剂的复合青霉素类。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "抗生素" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "感染" }, { "start_idx": 16, "end_idx": 21, "type": "dru", "entity": "耐酶青霉素类" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "克林霉素" }, { "start_idx": 32, "end_idx": 41, "type": "dru", "entity": "含β-内酰胺酶抑制剂" }, { "start_idx": 43, "end_idx": 48, "type": "dru", "entity": "复合青霉素类" } ] }, { "text": "脓肿成熟时应切开引流。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "脓肿" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "切开引流" } ] }, { "text": "临床上多种疾病可引起脱水和酸中毒,如腹泻和酮症中毒等。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "脱水" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "酸中毒" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "腹泻" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "酮症中毒" } ] }, { "text": "起病大多缓慢,表现为发热,咳嗽、进行性呼吸困难、胸痛,咳恶臭痰是本病的特征。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "发热" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "咳嗽" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "进行性呼吸困难" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "胸痛" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "咳恶臭痰" } ] }, { "text": "本病表现为坏死性肺炎,常发生肺脓疡和脓胸、脓气胸。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "坏死性肺炎" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "肺脓疡" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "脓胸" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "脓气胸" } ] }, { "text": "化验检查常有外周血白细胞计数和中性粒细胞比例的升高。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "ite", "entity": "外周血白细胞计数" }, { "start_idx": 15, "end_idx": 19, "type": "ite", "entity": "中性粒细胞" } ] }, { "text": "抗生素可选用青霉素G、克林霉素、甲硝唑等。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "青霉素G" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "克林霉素" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "甲硝唑" } ] }, { "text": "脓胸者需及时开放引流。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "脓胸" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "开放引流" } ] }, { "text": "本病的主要临床特征为面部血管纤维瘤、癫痫发作和智力低下。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "面部血管纤维瘤" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "癫痫发作" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "智力低下" } ] }, { "text": "90%的患儿在出生时即可发现数目多少不等的皮肤色素脱失斑,白色,与周围皮肤界限清楚,呈椭圆形或其他形状,大小不等,长径从1cm至数厘米。", "entities": [ { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "皮肤色素脱失斑" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "白色" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "周围皮肤界限清楚" }, { "start_idx": 42, "end_idx": 50, "type": "sym", "entity": "呈椭圆形或其他形状" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "大小不等" }, { "start_idx": 57, "end_idx": 66, "type": "sym", "entity": "长径从1cm至数厘米" } ] }, { "text": "有些病人还可见到成簇的、数目较多、形状不规则以及面积较小的似纸屑状的小块色素脱失斑。", "entities": [ { "start_idx": 8, "end_idx": 40, "type": "sym", "entity": "成簇的、数目较多、形状不规则以及面积较小的似纸屑状的小块色素脱失斑" } ] }, { "text": "70%~80%的病人有面部血管纤维瘤,以往称为皮脂腺瘤,为TS所特有的体征,由血管及结缔组织所组成,表现为面颊鼻翼两侧一些小的、粉红色、质硬的乳头状丘疹,隆起于皮肤,表面光滑,无渗出或分泌物。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "面部血管纤维瘤" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "皮脂腺瘤" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "TS" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "血管" }, { "start_idx": 42, "end_idx": 45, "type": "bod", "entity": "结缔组织" }, { "start_idx": 53, "end_idx": 75, "type": "sym", "entity": "面颊鼻翼两侧一些小的、粉红色、质硬的乳头状丘疹" }, { "start_idx": 77, "end_idx": 81, "type": "sym", "entity": "隆起于皮肤" }, { "start_idx": 83, "end_idx": 86, "type": "sym", "entity": "表面光滑" }, { "start_idx": 88, "end_idx": 94, "type": "sym", "entity": "无渗出或分泌物" } ] }, { "text": "15%~20%的病人有指(趾)甲纤维瘤,在指(趾)甲下面,像一小块肉状的小结节。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "指(趾)甲纤维瘤" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "指" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "趾" }, { "start_idx": 21, "end_idx": 38, "type": "sym", "entity": "指(趾)甲下面,像一小块肉状的小结节" } ] }, { "text": "20%~30%的病人有鲨鱼皮样斑,微微隆起于皮肤,边界不规则,表面粗糙,呈灰褐色,单发或多发,大小不等,每块直径约几毫米至5~6cm。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "鲨鱼皮样斑" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "微微隆起于皮肤" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "边界不规则" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "表面粗糙" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "呈灰褐色" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "单发或多发" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "大小不等" }, { "start_idx": 52, "end_idx": 65, "type": "sym", "entity": "每块直径约几毫米至5~6cm" } ] }, { "text": "多见于躯干背部及腰骶部皮肤,青春期后出现。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "躯干" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "背部" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "腰骶部" } ] }, { "text": "发作形式多样,多为难治性癫痫。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "癫痫" } ] }, { "text": "初起多为婴儿痉挛,以后可转为Lennox-Gastaut综合征,或呈全身性发作、简单部分性发作及复杂部分性发作。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "婴儿痉挛" }, { "start_idx": 14, "end_idx": 30, "type": "dis", "entity": "Lennox-Gastaut综合征" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "全身性发作" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "简单部分性发作" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "复杂部分性发作" } ] }, { "text": "其中室管膜下结节发生在约80%的病人,常位于侧脑室边缘,双侧多发,易钙化,可发展为巨细胞星形细胞瘤,约占6%,很少恶变,阻塞脑室孔可引起脑积水。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "室管膜" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "侧脑室边缘" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "易钙化" }, { "start_idx": 38, "end_idx": 48, "type": "sym", "entity": "发展为巨细胞星形细胞瘤" }, { "start_idx": 60, "end_idx": 70, "type": "sym", "entity": "阻塞脑室孔可引起脑积水" } ] }, { "text": "本病是最常见的小儿肾脏疾病,据1982年全国105所医院儿科住院病人统计,APSGN占同期住院泌尿系统疾病病人的53%。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "小儿肾脏疾病" }, { "start_idx": 28, "end_idx": 29, "type": "dep", "entity": "儿科" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "APSGN" }, { "start_idx": 47, "end_idx": 52, "type": "dis", "entity": "泌尿系统疾病" } ] }, { "text": "临床表现轻重不一,典型表现为水肿、尿少及高血压。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "水肿" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "尿少" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "高血压" } ] }, { "text": "由皮肤感染引起的肾炎则以49型为主,少数为2、55、57和60型,侵袭率可达25%。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "皮肤感染" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肾炎" } ] }, { "text": "光镜下可见肾小球肿大,内皮细胞及系膜细胞增生(称为毛细血管内增生),中性多形核白细胞和单核细胞在肾小球内浸润,使毛细血管壁狭窄乃至闭塞,但毛细血管壁通常无坏死。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "光镜" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "肾小球肿大" }, { "start_idx": 11, "end_idx": 32, "type": "sym", "entity": "内皮细胞及系膜细胞增生(称为毛细血管内增生)" }, { "start_idx": 34, "end_idx": 53, "type": "sym", "entity": "中性多形核白细胞和单核细胞在肾小球内浸润" }, { "start_idx": 55, "end_idx": 66, "type": "sym", "entity": "使毛细血管壁狭窄乃至闭塞" }, { "start_idx": 68, "end_idx": 78, "type": "sym", "entity": "但毛细血管壁通常无坏死" } ] }, { "text": "初期以眼睑及颜面为主,渐下行至四肢,呈非凹陷性,合并腹水及胸水都极为少见。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "眼睑" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "颜面" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "四肢" } ] }, { "text": "表现为明显水肿、持续少尿乃至无尿,心慌气促、烦躁、不能平卧、发绀、两肺湿啰音、心音低钝、心率增快、奔马律和肝脏进行性增大。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "明显水肿" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "持续少尿" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "无尿" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "心慌气促" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "烦躁" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "不能平卧" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "发绀" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "两肺湿啰音" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "心音低钝" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "心率增快" }, { "start_idx": 49, "end_idx": 59, "type": "sym", "entity": "奔马律和肝脏进行性增大" } ] }, { "text": "2.表现为肾病综合征的急性肾小球肾炎,蛋白尿明显的急性肾炎可出现低蛋白血症、高脂血症和凹陷性水肿。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "急性肾小球肾炎" }, { "start_idx": 19, "end_idx": 21, "type": "ite", "entity": "蛋白尿" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "急性肾炎" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "低蛋白血症" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "高脂血症" } ] }, { "text": "通过尿检动态观察及血清补体检测可与肾炎性肾病综合征相鉴别。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "尿检" }, { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "血清补体检测" }, { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "肾炎性肾病综合征" } ] }, { "text": "鉴别困难时需行肾活体组织检查。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "pro", "entity": "肾活体组织检查" } ] }, { "text": "上述治疗无效时可用血液滤过、血液透析或腹膜透析治疗。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "血液滤过" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "血液透析" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "腹膜透析" } ] }, { "text": "对于持续大量蛋白尿者或临床病理有慢性化趋势的患儿,可口服泼尼松(prednisone)治疗,剂量1~2mg/(kg•d),并逐步减量,疗程以1~2个月为宜。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "泼尼松" }, { "start_idx": 32, "end_idx": 41, "type": "dru", "entity": "prednisone" } ] }, { "text": "对于肾活组织检查有大量新月体的病人可先以甲泼尼龙(methylprednisolone)每次20~30mg/kg冲击治疗,然后改为泼尼松口服治疗。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "肾活组织检查" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "甲泼尼龙" }, { "start_idx": 25, "end_idx": 42, "type": "dru", "entity": "methylprednisolone" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "冲击治疗" }, { "start_idx": 65, "end_idx": 67, "type": "dru", "entity": "泼尼松" } ] }, { "text": "绝大多数患儿2~4周内肉眼血尿消失,尿量增多,水肿消退,血压逐渐恢复,残余少量蛋白尿及镜下血尿多于6个月内消失,少数重症病人可迁延1~3年甚至发展成慢性肾炎或慢性肾功能不全。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "尿量" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "水肿" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "血尿" }, { "start_idx": 74, "end_idx": 77, "type": "dis", "entity": "慢性肾炎" }, { "start_idx": 79, "end_idx": 85, "type": "dis", "entity": "慢性肾功能不全" } ] }, { "text": "肿瘤主要发生于体腔浆膜,如胸膜、腹膜、心包膜、睾丸鞘膜等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "体腔浆膜" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胸膜" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "腹膜" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "心包膜" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "睾丸鞘膜" } ] }, { "text": "儿童患者的胸膜最多见,占85%左右。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "胸膜" } ] }, { "text": "胸腔病变多有胸痛和呼吸困难,可能出现气胸和胸腔积液。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胸腔" }, { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "胸痛和呼吸困难" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "气胸和胸腔积液" } ] }, { "text": "如肿瘤发生于腹腔或睾丸鞘膜则多可能扪及肿块。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "腹腔" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "睾丸鞘膜" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肿块" } ] }, { "text": "肿瘤有局部严重浸润的倾向,沿浆膜面广泛扩展,儿童患者有血行转移趋向,至肺、脑等部位。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "浆膜" }, { "start_idx": 35, "end_idx": 35, "type": "bod", "entity": "肺" }, { "start_idx": 37, "end_idx": 37, "type": "bod", "entity": "脑" } ] }, { "text": "化疗对本瘤无效果。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" } ] }, { "text": "1967年日本的川崎博士总结了自1961年到1967年之间50例有持续性发热、皮疹、淋巴结炎等特征性表现的病例后,将本病命名为皮肤黏膜淋巴结综合征而首先报道。", "entities": [ { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "持续性发热" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "皮疹" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "淋巴结炎" }, { "start_idx": 63, "end_idx": 72, "type": "dis", "entity": "皮肤黏膜淋巴结综合征" } ] }, { "text": "此后,随即发现川崎病并非是一种良性的疾病,许多患儿由于并发心血管疾病而导致死亡。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "川崎病" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "心血管" } ] }, { "text": "虽然川崎病在全世界均有发病,但最多见于日本及具有日本血统的儿童。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "川崎病" } ] }, { "text": "鉴于这种自限性疾病所表现出的发热、皮疹、结膜充血、颈淋巴结肿大以及好发于儿童、暴发流行时明显的地域分布都提示其发病与感染有关。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "发热" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "皮疹" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "结膜" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "结膜充血" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "颈" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "颈淋巴结肿大" } ] }, { "text": "相反,对患有川崎病的儿童的免疫系统所进行的观察发现,这些儿童都存在较严重的免疫紊乱。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "川崎病" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "免疫紊乱" } ] }, { "text": "Ⅱ期:12~25天,小血管炎减轻,冠状动脉主要分支等中等大小动脉全层血管炎(内膜、外膜、中膜均有炎性细胞浸润)突出,伴有坏死、水肿,血管弹力纤维和肌层断裂,出现冠状动脉扩张,易发生冠状动脉瘤及血栓。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "血管炎" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "小血管炎减轻" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "动脉" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "血管炎" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "内膜" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "外膜" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "中膜" }, { "start_idx": 48, "end_idx": 51, "type": "bod", "entity": "炎性细胞" }, { "start_idx": 38, "end_idx": 53, "type": "sym", "entity": "内膜、外膜、中膜均有炎性细胞浸润" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "坏死" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "水肿" }, { "start_idx": 66, "end_idx": 71, "type": "bod", "entity": "血管弹力纤维" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "肌层" }, { "start_idx": 66, "end_idx": 76, "type": "sym", "entity": "血管弹力纤维和肌层断裂" }, { "start_idx": 80, "end_idx": 83, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 80, "end_idx": 85, "type": "sym", "entity": "冠状动脉扩张" }, { "start_idx": 90, "end_idx": 94, "type": "dis", "entity": "冠状动脉瘤" }, { "start_idx": 96, "end_idx": 97, "type": "sym", "entity": "血栓" } ] }, { "text": "早期严重心肌炎、中后期动脉瘤破裂与血管栓塞是本病死亡的主要危险。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "心肌炎" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "动脉瘤破裂" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "血管栓塞" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "死亡" } ] }, { "text": "在最近修订的标准中,由于许多患儿会较快地发生冠状动脉瘤,故只需4项表现即可诊断。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "冠状动脉瘤" } ] }, { "text": "越来越多的病人虽未符合诊断标准但因为有以上临床表现而被诊断为川崎病,并接受静脉免疫球蛋白治疗。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "川崎病" }, { "start_idx": 37, "end_idx": 43, "type": "pro", "entity": "静脉免疫球蛋白" } ] }, { "text": "急性期通常持续1~2周,主要特征是发热,结膜充血,口咽部的改变、四肢末梢红肿、皮疹、淋巴结炎、无菌性脑膜炎、腹泻和肝功能受损。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "发热" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "结膜" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "结膜充血" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "口咽部" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "口咽部的改变" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "四肢" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "四肢末梢红肿" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "皮疹" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "淋巴结炎" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "无菌性脑膜炎" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "腹泻" }, { "start_idx": 57, "end_idx": 61, "type": "sym", "entity": "肝功能受损" } ] }, { "text": "另一方面,如果及时静脉使用免疫球蛋白和大剂量的阿司匹林,发热常在1~2天内缓解。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "pro", "entity": "静脉使用免疫球蛋白" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "发热" } ] }, { "text": "在发热24~48小时后常出现双侧结膜充血。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "发热" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "结膜" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "双侧结膜充血" } ] }, { "text": "裂隙灯检查可发现前葡萄膜炎。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "裂隙灯检查" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "前葡萄膜炎" } ] }, { "text": "口咽部的改变也见于热起后24~48小时。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "口咽部" } ] }, { "text": "最典型的是舌乳头增生,即草莓舌。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "舌乳头" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "舌乳头增生" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "舌" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "草莓舌" } ] }, { "text": "口腔及咽部明显充血,但不伴有溃疡和分泌物。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "口腔" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "咽部" }, { "start_idx": 0, "end_idx": 8, "type": "sym", "entity": "口腔及咽部明显充血" } ] }, { "text": "通常在起病后3~5天出现手掌及足底发红,双手足硬肿。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "手掌" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "足底" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "手掌及足底发红" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "双手" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "足" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "双手足硬肿" } ] }, { "text": "热起后10~20天手足硬肿与泛红趋于消退,进入亚急性期,指趾末端开始脱皮,进而累及整个手掌与足底。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "手足" }, { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "手足硬肿与泛红趋于消退" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "指趾" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "指趾末端开始脱皮" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "手掌" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "足底" } ] }, { "text": "川崎病起病后1~2月,在指甲上可出现横沟(Beau线)。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "川崎病" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "指甲" }, { "start_idx": 12, "end_idx": 19, "type": "sym", "entity": "指甲上可出现横沟" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "Beau线" } ] }, { "text": "腹股沟的皮疹和脱皮时有发生。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "腹股沟" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "皮疹" } ] }, { "text": "以上这些均发生于急性期,较指甲端脱皮发生早。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "指甲" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "指甲端脱皮" } ] }, { "text": "比较而言,其他的症状可见于90%以上的川崎病患儿,而颈淋巴结炎仅见于近50%~70%的患儿。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "川崎病" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "颈淋巴结炎" } ] }, { "text": "约有25%的患儿脑脊液中有单核细胞增多,蛋白质含量正常或轻度升高,糖含量正常。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "脑脊液" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "单核细胞" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "脑脊液中有单核细胞增多" }, { "start_idx": 20, "end_idx": 31, "type": "sym", "entity": "蛋白质含量正常或轻度升高" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "糖含量正常" } ] }, { "text": "在急性期,小关节可有关节炎的表现,而大关节受累多在起病后第二和第三周。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "关节炎" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "关节" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "大关节受累" } ] }, { "text": "不典型川崎病多发生于小婴儿,且这些症状不易被发现。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "川崎病" } ] }, { "text": "另外,一些伴发症状如呕吐、腹泻、体重下降、咽喉疼痛、头痛、假性脑膜炎比较多见。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "呕吐" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "腹泻" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "体重下降" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "咽喉疼痛" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "头痛" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "假性脑膜炎" } ] }, { "text": "更重要的是,年长儿更易发生冠状动脉畸形。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "冠状动脉畸形" } ] }, { "text": "需与其鉴别的有细菌性感染如猩红热,葡萄球菌引起的皮肤症状,中毒性休克,风湿热,洛基山斑疹热和细螺旋体病。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "细菌性感染" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "猩红热" }, { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "葡萄球菌" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "中毒性休克" }, { "start_idx": 35, "end_idx": 37, "type": "sym", "entity": "风湿热" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "洛基山斑疹热" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "细螺旋体病" } ] }, { "text": "病毒感染也要与川崎病鉴别,包括麻疹,EB病毒及腺病毒感染。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "病毒感染" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "川崎病" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "麻疹" }, { "start_idx": 18, "end_idx": 21, "type": "mic", "entity": "EB病毒" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "腺病毒感染" } ] }, { "text": "非感染性疾病如Stevens-Johnson综合征、药物反应和幼年型类风湿性关节炎。", "entities": [ { "start_idx": 7, "end_idx": 24, "type": "dis", "entity": "Stevens-Johnson综合征" }, { "start_idx": 31, "end_idx": 40, "type": "dis", "entity": "幼年型类风湿性关节炎" } ] }, { "text": "冠状动脉瘤是川崎病中最严重的并发症。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "冠状动脉瘤" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "川崎病" } ] }, { "text": "约有近20%~25%的患儿有冠状动脉畸形,包括弥漫性扩张和动脉瘤。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "冠状动脉畸形" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "弥漫性扩张" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "动脉瘤" } ] }, { "text": "血管造影发现,55%的冠状动脉瘤可能持续10~21年。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "血管造影" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "冠状动脉瘤" } ] }, { "text": "冠状动脉表现为内皮功能紊乱、低顺应性、血管壁增厚,而以上这些是否会增加早期动脉硬化症的发病率尚不明确。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "内皮功能紊乱" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "低顺应性" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "血管壁" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "血管壁增厚" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "动脉硬化症" } ] }, { "text": "42%的有持续性动脉瘤的患儿可发生冠状动脉狭窄。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "冠状动脉狭窄" } ] }, { "text": "最严重的类型是发生巨大的动脉瘤(直径>8mm)。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "动脉瘤" } ] }, { "text": "儿童心肌梗死的表现不典型,可表现为恶心、呕吐、苍白、出汗、哭吵,年长儿常诉胸痛或腹痛。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "心肌梗死" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "恶心" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "呕吐" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "苍白" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "出汗" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "哭吵" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "胸痛" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "腹痛" } ] }, { "text": "某些临床表现提示有发生冠心病的危险,包括发热持续16天以上,反复发热之间间隔48小时以上,除了有Ⅰ°心传导阻滞以外的其他心律失常,小于1岁发病,心脏扩大,血小板计数﹑血清清蛋白及血细胞计数低。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "冠心病" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "发热" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "发热" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "Ⅰ°心传导阻滞" }, { "start_idx": 60, "end_idx": 61, "type": "ite", "entity": "心律" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "心律失常" }, { "start_idx": 72, "end_idx": 75, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 77, "end_idx": 81, "type": "ite", "entity": "血小板计数" }, { "start_idx": 83, "end_idx": 87, "type": "ite", "entity": "血清清蛋白" }, { "start_idx": 89, "end_idx": 93, "type": "ite", "entity": "血细胞计数" }, { "start_idx": 77, "end_idx": 94, "type": "sym", "entity": "血小板计数﹑血清清蛋白及血细胞计数低" } ] }, { "text": "约有50%的患儿有心肌炎,常表现为心动过速并有心电图的改变。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "心动过速" }, { "start_idx": 23, "end_idx": 25, "type": "pro", "entity": "心电图" } ] }, { "text": "约有25%的病人有渗出性心包炎。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "渗出性心包炎" } ] }, { "text": "有2%没有治疗的病人发生全身性动脉瘤,通常这些患者亦有冠状动脉瘤。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "全身性动脉瘤" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "冠状动脉瘤" } ] }, { "text": "最常受累的动脉有腋动脉、髂动脉、肾动脉和肠系膜动脉。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "动脉" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "腋动脉" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "髂动脉" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "肾动脉" }, { "start_idx": 20, "end_idx": 24, "type": "bod", "entity": "肠系膜动脉" } ] }, { "text": "而广泛动脉受累导致血管收缩引起四肢末梢坏疽较罕见。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "动脉" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "动脉受累" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "血管收缩" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "四肢末梢坏疽" } ] }, { "text": "使用前列腺素E及系统的阿司匹林治疗并用甲基泼尼松龙冲击治疗可获得意想不到的疗效。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dru", "entity": "前列腺素E" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 19, "end_idx": 24, "type": "dru", "entity": "甲基泼尼松龙" } ] }, { "text": "在急性期白细胞总数正常或升高,多形核白细胞也升高。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "白细胞" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "白细胞" }, { "start_idx": 15, "end_idx": 23, "type": "sym", "entity": "多形核白细胞也升高" } ] }, { "text": "川崎病的患儿几乎没有白细胞减少症。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "川崎病" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "白细胞减少症" } ] }, { "text": "正细胞性贫血较常见。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "正细胞性贫血" } ] }, { "text": "肝酶在急性期有所升高,而胆红素的升高较少见。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肝酶" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "胆红素" } ] }, { "text": "由于川崎病患者有多克隆B细胞的活化,所以抗核抗体和类风湿因子可阴性。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "川崎病" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "多克隆B细胞" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "抗核抗体" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "类风湿因子" } ] }, { "text": "心肌酶谱的升高提示有心肌梗死的存在。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "心肌酶谱的升高" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "心肌梗死" } ] }, { "text": "胸部X线片检查一般无临床意义。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "ite", "entity": "胸部X线片检查" } ] }, { "text": "在有巨大动脉瘤的患儿,胸部X线片检查只能在晚期提示动脉瘤的钙化影。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "巨大动脉瘤" }, { "start_idx": 11, "end_idx": 17, "type": "ite", "entity": "胸部X线片检查" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "钙化影" } ] }, { "text": "对于心脏超声不能明确的冠状动脉狭窄及冠状动脉末梢的损伤,选择性动脉造影均可清晰的显现(图9-39)。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "心脏超声" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "冠状动脉狭窄" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 28, "end_idx": 34, "type": "ite", "entity": "选择性动脉造影" } ] }, { "text": "最近,在少数患川崎病的青少年及青年进行的磁共振冠脉造影被证实可确诊冠脉瘤。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "川崎病" }, { "start_idx": 20, "end_idx": 26, "type": "ite", "entity": "磁共振冠脉造影" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "冠脉瘤" } ] }, { "text": "口服阿司匹林及大剂量的静脉应用免疫球蛋白是治疗的基础。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "免疫球蛋白" } ] }, { "text": "如有因血栓所致的心肌梗死,溶栓治疗是必要的。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "血栓" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "心肌梗死" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "溶栓治疗" } ] }, { "text": "在急性期,阿司匹林的用量是口服80~100mg/(kg•d),每日4次。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "阿司匹林" } ] }, { "text": "当热度消退或起病14天后,阿司匹林剂量为3~5mg/(kg•d),一天1次能减少血栓的形成。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "血栓" } ] }, { "text": "如果在起病后6~8周没有发现冠状动脉瘤,血小板计数及血沉正常,阿司匹林可停药。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "冠状动脉瘤" }, { "start_idx": 20, "end_idx": 24, "type": "ite", "entity": "血小板计数" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "血沉" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "阿司匹林" } ] }, { "text": "联合应用阿司匹林和静脉免疫球蛋白的效果相当迅速。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "免疫球蛋白" } ] }, { "text": "2/3的患儿在使用免疫球蛋白后的24小时内即热退,90%的在48小时内热退,若48小时后体温仍较高,可考虑加用一次静脉免疫球蛋白1g/kg。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "免疫球蛋白" }, { "start_idx": 59, "end_idx": 63, "type": "dru", "entity": "免疫球蛋白" } ] }, { "text": "约有10%的川崎病患者尽管使用了免疫球蛋白但仍有持续发热。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "川崎病" }, { "start_idx": 16, "end_idx": 20, "type": "dru", "entity": "免疫球蛋白" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "发热" } ] }, { "text": "一项研究表明CRP的增高,LDH的增高及血红蛋白的降低是导致免疫球蛋白治疗无效的原因。", "entities": [ { "start_idx": 30, "end_idx": 34, "type": "dru", "entity": "免疫球蛋白" } ] }, { "text": "虽然如此,在日本的早期资料显示对免疫球蛋白治疗无效的患者,肾上腺皮质激素治疗可增加冠状动脉瘤及心肌梗死的发病率。", "entities": [ { "start_idx": 29, "end_idx": 37, "type": "pro", "entity": "肾上腺皮质激素治疗" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "冠状动脉瘤" }, { "start_idx": 47, "end_idx": 50, "type": "dis", "entity": "心肌梗死" } ] }, { "text": "如实验室检查均正常,且没有冠状动脉损伤,阿司匹林可停药。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "ite", "entity": "实验室检查" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "阿司匹林" } ] }, { "text": "在应用免疫球蛋白治疗后至少6个月不能接受疫苗的接种,因为特殊的抗体可干扰疫苗的免疫应答。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "免疫球蛋白" }, { "start_idx": 20, "end_idx": 24, "type": "pro", "entity": "疫苗的接种" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "抗体" }, { "start_idx": 36, "end_idx": 37, "type": "dru", "entity": "疫苗" } ] }, { "text": "这种划分有利于对患者进行有效的个人化的管理,如长期药物治疗,体格检查来进行诊断。", "entities": [ { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "体格检查" } ] }, { "text": "那些没有冠状动脉受累的患者或仅有急性期暂时性冠状动脉狭窄的患者不需要长期使用阿司匹林。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "冠状动脉受累" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "冠状动脉狭窄" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "阿司匹林" } ] }, { "text": "有冠状动脉持续狭窄或动脉瘤形成的患者,阿司匹林必须长期使用。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "阿司匹林" } ] }, { "text": "若患者感染水痘或流行性感冒,阿司匹林必须暂时停用以防止出现Reye综合征。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "水痘" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "流行性感冒" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "Reye综合征" } ] }, { "text": "在此期间双嘧达莫可替代应用。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "双嘧达莫" } ] }, { "text": "那些有小至中型冠状动脉瘤的患者必须每年复查心脏超声波。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "冠状动脉瘤" }, { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "心脏超声波" } ] }, { "text": "如压力试验提示有冠脉狭窄,就需要进行血管造影。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "冠脉" }, { "start_idx": 18, "end_idx": 21, "type": "ite", "entity": "血管造影" } ] }, { "text": "当然许多儿科的心脏病专家都建议对所有有冠状动脉瘤的患者都进行血管造影。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dep", "entity": "儿科" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "心脏病" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "冠状动脉瘤" }, { "start_idx": 30, "end_idx": 33, "type": "ite", "entity": "血管造影" } ] }, { "text": "除了阿司匹林,华法林治疗也是方法之一。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "华法林" } ] }, { "text": "治疗的手段包括搭桥治疗、球囊扩张及其他一些恢复冠状动脉血流的方法。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "搭桥治疗" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "球囊扩张" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "冠状动脉" } ] }, { "text": "在少数有严重心功能不良及不适合进行冠状动脉成形术的患者,可考虑进行心脏移植。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "心功能不良" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "冠状动脉成形术" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "心脏移植" } ] }, { "text": "分为5型:局限型、囊性型、外部生长型、颈延髓交界型及内部浸润型肿瘤。", "entities": [ { "start_idx": 5, "end_idx": 32, "type": "dis", "entity": "局限型、囊性型、外部生长型、颈延髓交界型及内部浸润型肿瘤" } ] }, { "text": "前4种多为低度胶质瘤,而内部浸润型肿瘤多为高度胶质瘤,并可有转移。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "低度胶质瘤" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "内部浸润型肿瘤" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "高度胶质瘤" } ] }, { "text": "常有相应的脑神经损害症,如口眼歪斜及吞咽发呛(Ⅵ、Ⅶ、Ⅸ脑神经损害)、走路蹒跚及肢体共济失调(小脑损害症)等,颅高压症状相对较少。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "脑神经损害症" }, { "start_idx": 13, "end_idx": 21, "type": "sym", "entity": "口眼歪斜及吞咽发呛" }, { "start_idx": 35, "end_idx": 45, "type": "sym", "entity": "走路蹒跚及肢体共济失调" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "小脑损害症" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "颅高压" } ] }, { "text": "辅助检查MRI对诊断脑干肿瘤最理想,能精确地显示肿瘤的位置、大小及类型等。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "pro", "entity": "MRI" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "脑干肿瘤" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "肿瘤" } ] }, { "text": "对于内部浸润型肿瘤,无法手术,主要靠放射治疗。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "内部浸润型肿瘤" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "手术" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "放射" } ] }, { "text": "化疗对脑干胶质瘤尚未找到有效方案。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "脑干胶质瘤" } ] }, { "text": "病变多仅限于一肺叶,以左上叶最常见,其次为右中叶和右上叶,下叶罕见。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "一肺叶" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "左上叶" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "右中叶" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "右上叶" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "下叶" } ] }, { "text": "应注意与先天性肺囊肿、肺大疱和局限性气胸的鉴别,必要时进行CT检查。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "先天性肺囊肿" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肺大疱" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "局限性气胸" }, { "start_idx": 29, "end_idx": 32, "type": "ite", "entity": "CT检查" } ] }, { "text": "输尿管的先天性畸形是输尿管的胚胎发生异常造成的,其诱导的后肾组织随之发生异常,导致肾脏畸形。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "输尿管的先天性畸形" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "肾脏畸形" } ] }, { "text": "萌出输尿管芽的中肾管肘部以远部分于胚胎第8周逐步吸收成泄殖腔的一部分,形成尿生殖窦,此时开始输尿管及中肾管分别与尿生殖窦独立相连。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "输尿管芽" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "中肾管肘部" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "泄殖腔" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "尿生殖窦" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "输尿管" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "中肾管" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "尿生殖窦" } ] }, { "text": "在发育过程中,输尿管口向头端外侧端迁移,而中肾管则移至远端中线方向,形成含有精阜的后尿道,与膀胱相连,并演变成附睾、输精管和精囊。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "输尿管" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "中肾管" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "后尿道" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "膀胱" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "附睾" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "输精管" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "精囊" } ] }, { "text": "如输尿管芽位置很高,则输尿管口可异位至尿道或中肾管的遗迹,在男性如精阜及输精管等,在女性可位于子宫阔韧带、阴道壁至外阴部。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "输尿管芽" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "输尿管口" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "尿道" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "中肾管" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "精阜" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "输精管" }, { "start_idx": 47, "end_idx": 51, "type": "bod", "entity": "子宫阔韧带" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "阴道壁" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "外阴部" } ] }, { "text": "有人推测是因输尿管口狭窄引起的,但临床上常见输尿管膨出开口并不狭窄的病例。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "输尿管口狭窄" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "输尿管膨出" } ] }, { "text": "食管与胃的交界形成食管贲门角(His角),该角可以防止胃食管反流和食管裂孔疝的发生,婴幼儿His角不明显,黏膜皱襞构成的防护瓣也不明显,因而易发生胃食管反流,使婴儿容易发生呕吐或溢乳。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "食管" }, { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "胃" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "食管贲门角" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "His角" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "食管裂孔疝" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "His角" }, { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "黏膜皱襞" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "胃食管反流" }, { "start_idx": 80, "end_idx": 90, "type": "sym", "entity": "婴儿容易发生呕吐或溢乳" } ] }, { "text": "婴儿胃幽门括约肌发育良好,因自主神经调节功能差,易引起幽门痉挛而出现呕吐。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "胃幽门括约肌" }, { "start_idx": 14, "end_idx": 35, "type": "sym", "entity": "自主神经调节功能差,易引起幽门痉挛而出现呕吐" } ] }, { "text": "由于空、回肠系膜较长,黏膜下组织松弛,活动度大,容易发生肠扭转及肠套叠。", "entities": [ { "start_idx": 0, "end_idx": 22, "type": "sym", "entity": "由于空、回肠系膜较长,黏膜下组织松弛,活动度大" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肠扭转" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "肠套叠" } ] }, { "text": "正常菌群对肠黏液的正常发育有显著影响;同时参与食物的消化和吸收过程,双歧杆菌能分泌各种酶,将不溶性蛋白、脂肪和糖变为可溶性,以利新陈代谢;双歧杆菌促进铁、钙和维生素D吸收,防止佝偻病;双歧杆菌和大肠杆菌还可合成B族和K族维生素;正常菌群不仅是抗原,而且具有调节产生抗体的B细胞和T辅助细胞、巨噬细胞和肥大细胞的功能,产生特异和非特异性免疫功能,对侵入肠道的致病菌有一定的拮抗作用;当然在人体抵抗力下降或消化道功能紊乱时,菌群中许多致病菌大量繁殖进入小肠、胃或者肠管以外脏器,发生内源性感染。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肠黏液" }, { "start_idx": 34, "end_idx": 37, "type": "mic", "entity": "双歧杆菌" }, { "start_idx": 88, "end_idx": 90, "type": "dis", "entity": "佝偻病" }, { "start_idx": 92, "end_idx": 95, "type": "mic", "entity": "双歧杆菌" }, { "start_idx": 97, "end_idx": 100, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 135, "end_idx": 137, "type": "bod", "entity": "B细胞" }, { "start_idx": 139, "end_idx": 143, "type": "bod", "entity": "T辅助细胞" }, { "start_idx": 145, "end_idx": 148, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 150, "end_idx": 153, "type": "bod", "entity": "肥大细胞" }, { "start_idx": 175, "end_idx": 176, "type": "bod", "entity": "肠道" }, { "start_idx": 224, "end_idx": 225, "type": "bod", "entity": "小肠" }, { "start_idx": 227, "end_idx": 227, "type": "bod", "entity": "胃" }, { "start_idx": 230, "end_idx": 231, "type": "bod", "entity": "肠管" }, { "start_idx": 234, "end_idx": 235, "type": "bod", "entity": "脏器" } ] }, { "text": "清创处理后,用抗狂犬病免疫血清或免疫球蛋白滴注伤口深部,并渗入伤口周围或在伤口周围作皮下浸润注射。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dru", "entity": "抗狂犬病免疫血清" }, { "start_idx": 16, "end_idx": 20, "type": "dru", "entity": "免疫球蛋白" }, { "start_idx": 42, "end_idx": 47, "type": "pro", "entity": "皮下浸润注射" } ] }, { "text": "2.被犬咬伤者不论伤在何处,均应立即进行预防注射狂犬疫苗。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "咬伤" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "狂犬疫苗" } ] }, { "text": "免疫功能异常及病毒感染与本病发生有关,即在原发性免疫缺陷基础上诱发感染相关性嗜血细胞综合征。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "免疫功能异常" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "病毒感染" } ] }, { "text": "2.实验室检查伴有肝功能异常、凝血因子特别是纤维蛋白原降低、脂代谢异常如血清甘油三酯及胆固醇升高时应高度怀疑本病。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "ite", "entity": "肝功能" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "肝功能异常" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "凝血因子" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "纤维蛋白原" }, { "start_idx": 15, "end_idx": 28, "type": "sym", "entity": "凝血因子特别是纤维蛋白原降低" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "脂代谢异常" }, { "start_idx": 36, "end_idx": 47, "type": "sym", "entity": "血清甘油三酯及胆固醇升高" } ] }, { "text": "FEL、IAHS的病理细胞学特征相似,随活检的时间而定,病程早期骨髓增生活跃,涂片中可见吞噬红细胞的吞噬细胞增多,而在病程后期,骨髓增生低下并有不同数量的噬血性组织细胞浸润。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "FEL" }, { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "IAHS" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "吞噬红细胞" }, { "start_idx": 50, "end_idx": 53, "type": "bod", "entity": "吞噬细胞" }, { "start_idx": 44, "end_idx": 55, "type": "sym", "entity": "吞噬红细胞的吞噬细胞增多" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "骨髓" }, { "start_idx": 64, "end_idx": 69, "type": "sym", "entity": "骨髓增生低下" }, { "start_idx": 77, "end_idx": 83, "type": "bod", "entity": "噬血性组织细胞" }, { "start_idx": 77, "end_idx": 85, "type": "sym", "entity": "噬血性组织细胞浸润" } ] }, { "text": "3.目前对IAHS尚无特殊的治疗,若有EBV、CMV、腺病毒或其他病毒感染征象,则可考虑使用抗病毒药物如阿昔洛韦及干扰能联合治疗,也可同时考虑以上化疗以抑制病毒感染后异常反应的组织细胞。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "mic", "entity": "EBV" }, { "start_idx": 23, "end_idx": 25, "type": "mic", "entity": "CMV" }, { "start_idx": 27, "end_idx": 29, "type": "mic", "entity": "腺病毒" }, { "start_idx": 52, "end_idx": 55, "type": "dru", "entity": "阿昔洛韦" }, { "start_idx": 78, "end_idx": 81, "type": "dis", "entity": "病毒感染" } ] }, { "text": "可生长于身体的任何部位,但以皮肤和皮下组织多见,其次为口腔黏膜和肌肉,骨骼、肝、脾、颅内也可发生,而消化道、肾、心、肺等罕见。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "皮肤" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "皮下组织" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "口腔黏膜" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肌肉" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "骨骼" }, { "start_idx": 38, "end_idx": 38, "type": "bod", "entity": "肝" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "脾" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "颅内" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "消化道" }, { "start_idx": 54, "end_idx": 54, "type": "bod", "entity": "肾" }, { "start_idx": 56, "end_idx": 56, "type": "bod", "entity": "心" }, { "start_idx": 58, "end_idx": 58, "type": "bod", "entity": "肺" } ] }, { "text": "另一种是肥大细胞学说,因为它能释放或分泌多种生物活性物质,直接刺激内皮细胞增生。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "肥大细胞" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "内皮细胞" } ] }, { "text": "2.海绵状血管瘤和混合型(毛细血管和海绵状)血管瘤。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "海绵状血管瘤" }, { "start_idx": 9, "end_idx": 24, "type": "dis", "entity": "混合型(毛细血管和海绵状)血管瘤" } ] }, { "text": "3.蔓状血管瘤。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "蔓状血管瘤" } ] }, { "text": "它不仅可导致皮肤、黏膜、指(趾)甲的浅部真菌病,在人体抵抗力减低时,还可侵犯胃肠道、肺、心脏、肾和脑等内脏器官,引起深部真菌病。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "皮肤" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "黏膜" }, { "start_idx": 12, "end_idx": 16, "type": "bod", "entity": "指(趾)甲" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "浅部真菌病" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "胃肠道" }, { "start_idx": 42, "end_idx": 42, "type": "bod", "entity": "肺" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "心脏" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "肾" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "脑" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "深部真菌病" } ] }, { "text": "初起局部皮肤潮红、糜烂,形成边界清楚、潮湿鲜红的创面,伴灰白色脱屑和表皮翘起,即擦烂红斑和脱屑性损害。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "皮肤" }, { "start_idx": 2, "end_idx": 10, "type": "sym", "entity": "局部皮肤潮红、糜烂" }, { "start_idx": 14, "end_idx": 25, "type": "sym", "entity": "边界清楚、潮湿鲜红的创面" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "灰白色脱屑" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "表皮翘起" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "擦烂红斑" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "脱屑性损害" } ] }, { "text": "慢性甲沟炎可波及甲板引起甲真菌病,甲沟慢性肉芽组织增生,致甲板变硬、增厚,有时变为灰暗色,指甲表面高低不平,并出现条纹或沟,但仍有光泽。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "慢性甲沟炎" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "甲板" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "甲真菌病" }, { "start_idx": 17, "end_idx": 26, "type": "dis", "entity": "甲沟慢性肉芽组织增生" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "甲板" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "甲板变硬、增厚" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "变为灰暗色" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "指甲" }, { "start_idx": 45, "end_idx": 52, "type": "sym", "entity": "指甲表面高低不平" }, { "start_idx": 55, "end_idx": 60, "type": "sym", "entity": "出现条纹或沟" }, { "start_idx": 64, "end_idx": 66, "type": "sym", "entity": "有光泽" } ] }, { "text": "③肉芽肿型口腔念珠菌病:常并发于皮肤念珠菌性肉芽肿,罕见。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "dis", "entity": "肉芽肿型口腔念珠菌病" }, { "start_idx": 16, "end_idx": 24, "type": "dis", "entity": "皮肤念珠菌性肉芽肿" } ] }, { "text": "皮损以尿布区为主,为大片不规则形红斑,边缘非常清楚,可见有浸软的白色膜状物或脱屑。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "皮损" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "尿布区" }, { "start_idx": 10, "end_idx": 39, "type": "sym", "entity": "大片不规则形红斑,边缘非常清楚,可见有浸软的白色膜状物或脱屑" } ] }, { "text": "大片皮损周围可有散在的斑丘疹、水疱或丘水疱。", "entities": [ { "start_idx": 0, "end_idx": 20, "type": "sym", "entity": "大片皮损周围可有散在的斑丘疹、水疱或丘水疱" } ] }, { "text": "损害可蔓延至邻近的皮肤,包括下腹部、腰部、躯干和下肢等。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "皮肤" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "下腹部" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "腰部" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "躯干" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "下肢" } ] }, { "text": "有时可扩展至腋下、颈部、眼睑、眼结膜和其他部位。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "腋下" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "颈部" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "眼睑" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "眼结膜" } ] }, { "text": "白色念珠菌广泛感染皮肤、黏膜和甲板,但除消化系统外很少累及其他内脏。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "白色念珠菌" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "皮肤" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "黏膜" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "甲板" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "消化系统" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "内脏" } ] }, { "text": "感染可持续数十年,多数患者在出现慢性皮肤黏膜损害后3~10年才显示内分泌异常的表现。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "慢性皮肤黏膜" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "内分泌异常" } ] }, { "text": "患者一般先出现鹅口疮和口角糜烂,然后头面部、四肢末端出现红斑鳞屑性皮疹,有角质增生现象,皮损渐及躯干。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "鹅口疮" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "口角糜烂" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "头面部" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "四肢末端" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "红斑鳞屑性皮疹" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "角质增生" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "躯干" }, { "start_idx": 44, "end_idx": 49, "type": "sym", "entity": "皮损渐及躯干" } ] }, { "text": "部分患者手指末端肿胀,有甲沟炎和念珠菌性甲病。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "手指末端" }, { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "手指末端肿胀" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "甲沟炎" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "念珠菌性甲病" } ] }, { "text": "头发稀疏脱落,前额、鼻部等处可见赘疣状增殖性皮损,富含血管呈棕色蛎壳样或皮角状,有时高达2cm,具特征性。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "头发" }, { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "头发稀疏脱落" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "前额" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "鼻部" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "赘疣状增殖性皮损" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "血管" }, { "start_idx": 25, "end_idx": 38, "type": "sym", "entity": "富含血管呈棕色蛎壳样或皮角状" } ] }, { "text": "皮损好发于面、胸、腹、掌、跖部位,背部极少。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "皮损" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "面" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "胸" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "腹" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "掌" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "跖" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "背部" } ] }, { "text": "患儿常有鹅口疮和甲沟炎,3个月后甲板出现念珠菌性损害。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "鹅口疮" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "甲沟炎" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "甲板" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "念珠菌性损害" } ] }, { "text": "以新生儿最为常见,因其口腔pH低,有利于该菌生长,可引起口腔和牙龈黏膜(有时在舌面和软腭)表面覆盖一层珍珠白色、凝块样斑状物,边缘清楚,周围有红晕。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "口腔" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "口腔pH低" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "口腔" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "牙龈黏膜" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "舌面" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "软腭" } ] }, { "text": "阴茎炎好发于龟头和冠状沟,开始表现为阴茎部位疱疹,随后出现白色乳酪样白斑,可引起严重瘙痒。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "阴茎炎" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "龟头" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "冠状沟" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "阴茎" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "阴茎部位疱疹" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "白色乳酪样白斑" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "严重瘙痒" } ] }, { "text": "临床表现为角膜溃疡、坏死,边缘隆起呈放射状浸润,严重者可以穿孔失明。", "entities": [ { "start_idx": 5, "end_idx": 22, "type": "sym", "entity": "角膜溃疡、坏死,边缘隆起呈放射状浸润" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "穿孔" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "失明" } ] }, { "text": "多在长期应用抗生素的过程中,出现稽留热及剧烈的咳嗽,痰为无色胶冻样,偶带血丝。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "抗生素" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "稽留热" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "剧烈的咳嗽" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "痰" }, { "start_idx": 26, "end_idx": 37, "type": "sym", "entity": "痰为无色胶冻样,偶带血丝" } ] }, { "text": "钡餐可发现食管黏膜不规则或呈鹅卵石样影。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "钡餐" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "食管黏膜" }, { "start_idx": 5, "end_idx": 18, "type": "sym", "entity": "食管黏膜不规则或呈鹅卵石样影" } ] }, { "text": "确诊需通过食管镜检查获得的刮取物或活组织进行染色或培养证明念珠菌的存在。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "食管镜检查" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "刮取物" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "活组织" }, { "start_idx": 29, "end_idx": 31, "type": "mic", "entity": "念珠菌" } ] }, { "text": "严重者可形成黏膜溃疡,排血便甚至发生肠穿孔和腹膜炎。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "黏膜溃疡" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "排血便" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "肠穿孔" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "发病的标志是视网膜呈绒毛状、变白、棉花样改变,病变可扩展到玻璃体。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "视网膜" }, { "start_idx": 6, "end_idx": 21, "type": "sym", "entity": "视网膜呈绒毛状、变白、棉花样改变" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "玻璃体" } ] }, { "text": "表现为充血、虹膜炎、视力模糊、出现盲点和畏光等症状都曾有报道,但多数病例可能没有症状。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "充血" }, { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "虹膜炎" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "视力模糊" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "出现盲点" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "畏光" } ] }, { "text": "持续性粒细胞减少是引起发病的主要原因,抗肿瘤治疗的毒性包括化疗的细胞毒性、严重的黏膜破坏。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "粒细胞" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "持续性粒细胞减少" } ] }, { "text": "经验性地应用广谱抗生素和长期中心静脉插管等因素也参与发病。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dru", "entity": "广谱抗生素" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "静脉插管" } ] }, { "text": "临床表现包括在粒细胞减少症恢复期出现持续发热和腹部不适、白细胞增多和血清碱性磷酸酶增高,患者的肝、脾中可出现微小脓肿,在此期间X线也有相应表现。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "粒细胞减少症" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "持续发热" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "腹部" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "腹部不适" }, { "start_idx": 28, "end_idx": 30, "type": "ite", "entity": "白细胞" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "白细胞增多" }, { "start_idx": 34, "end_idx": 40, "type": "ite", "entity": "血清碱性磷酸酶" }, { "start_idx": 34, "end_idx": 42, "type": "sym", "entity": "血清碱性磷酸酶增高" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "肝" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "脾" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "微小脓肿" }, { "start_idx": 63, "end_idx": 64, "type": "pro", "entity": "X线" } ] }, { "text": "最常发生在膝关节。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "膝关节" } ] }, { "text": "皮损可呈银屑病样、玫瑰糠疹样、脂溢性皮炎样、荨麻疹样、离心性环形红斑样等。", "entities": [ { "start_idx": 0, "end_idx": 34, "type": "sym", "entity": "皮损可呈银屑病样、玫瑰糠疹样、脂溢性皮炎样、荨麻疹样、离心性环形红斑样" } ] }, { "text": "念珠菌引起的内脏过敏反应表现为胃炎、结肠炎、过敏性鼻炎、哮喘、眼色素膜炎等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "念珠菌" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "内脏过敏反应" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "胃炎" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "结肠炎" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "过敏性鼻炎" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "哮喘" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "眼色素膜炎" } ] }, { "text": "诊断念珠菌感染辅助的方法包括对视网膜病变进行眼科检查和超声波检查;对脑、肾脏或肝脾病变作CT或MRI,以及对治疗的反应进行监测。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "念珠菌感染" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "眼科检查" }, { "start_idx": 27, "end_idx": 31, "type": "pro", "entity": "超声波检查" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "脑" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肾脏" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "肝脾" }, { "start_idx": 44, "end_idx": 45, "type": "pro", "entity": "CT" }, { "start_idx": 47, "end_idx": 49, "type": "pro", "entity": "MRI" } ] }, { "text": "另外保持皮肤清洁、干燥是治疗的重要措施。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "保持皮肤清洁、干燥" } ] }, { "text": "(2)氟康唑:本品在16岁以下儿童体内的血浆半衰期与成人不同,故对不同年龄儿童推荐剂量如下:>4周的患儿:深部真菌感染,6mg/(kg•d),每日给药一次;严重威胁生命的感染,12mg/(kg•d),每日给药1次。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "氟康唑" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "血浆" }, { "start_idx": 53, "end_idx": 58, "type": "dis", "entity": "深部真菌感染" } ] }, { "text": "(3)两性霉素B:用于全身性深部念珠菌病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "全身性深部念珠菌病" } ] }, { "text": "(4)氟胞嘧啶:对严重的皮肤念珠菌病,需要全身性抗真菌治疗时可采用。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "氟胞嘧啶" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "皮肤念珠菌病" }, { "start_idx": 21, "end_idx": 26, "type": "dru", "entity": "全身性抗真菌" } ] }, { "text": "全身因素如营养不良、吸收不良综合征、肥胖、甲状腺功能低下、肾上腺功能减退、甲状旁腺功能减退、糖尿病、慢性肺部疾病、肿瘤、肠病性肢端皮炎;局部因素如多汗症、过度的浸渍、局部创伤、鼻饲管、引流管或导管、孕妇外阴阴道炎(致新生儿感染)等。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "肥胖" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "甲状腺功能低下" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "肾上腺功能减退" }, { "start_idx": 37, "end_idx": 44, "type": "dis", "entity": "甲状旁腺功能减退" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "糖尿病" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "慢性肺部疾病" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "肿瘤" }, { "start_idx": 60, "end_idx": 66, "type": "dis", "entity": "肠病性肢端皮炎" }, { "start_idx": 73, "end_idx": 75, "type": "dis", "entity": "多汗症" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "局部创伤" }, { "start_idx": 88, "end_idx": 90, "type": "equ", "entity": "鼻饲管" }, { "start_idx": 92, "end_idx": 94, "type": "equ", "entity": "引流管" }, { "start_idx": 96, "end_idx": 97, "type": "equ", "entity": "导管" }, { "start_idx": 101, "end_idx": 105, "type": "dis", "entity": "外阴阴道炎" } ] }, { "text": "注意保持婴幼儿的皮肤黏膜和口腔卫生,注意清洁母亲的乳头,注意母亲的个人卫生。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "皮肤黏膜" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "口腔" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "乳头" }, { "start_idx": 28, "end_idx": 36, "type": "pro", "entity": "注意母亲的个人卫生" } ] }, { "text": "室速可阵发性或持续性,其病因可为心肌炎、冠状动脉起源异常、致心律失常性右心室发育不良、二尖瓣脱垂、心脏原发肿瘤、心肌病、先天性或获得性QT间期延长、药物、心脏术后(如法洛四联症术后、室间隔缺损术后)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "室速" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "心肌炎" }, { "start_idx": 20, "end_idx": 27, "type": "sym", "entity": "冠状动脉起源异常" }, { "start_idx": 29, "end_idx": 41, "type": "sym", "entity": "致心律失常性右心室发育不良" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "二尖瓣脱垂" }, { "start_idx": 49, "end_idx": 54, "type": "sym", "entity": "心脏原发肿瘤" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "心肌病" }, { "start_idx": 60, "end_idx": 72, "type": "sym", "entity": "先天性或获得性QT间期延长" }, { "start_idx": 77, "end_idx": 79, "type": "pro", "entity": "心脏术" }, { "start_idx": 83, "end_idx": 87, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 91, "end_idx": 95, "type": "dis", "entity": "室间隔缺损" } ] }, { "text": "室速应及时处理,因其可引起低血压或发展成室颤。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "室速" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "低血压" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "室颤" } ] }, { "text": "如血流动力学正常,首选利多卡因。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "利多卡因" } ] }, { "text": "其他可用药物有:普鲁卡因胺、普萘洛尔或胺碘酮。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "普鲁卡因胺" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "普萘洛尔" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "胺碘酮" } ] }, { "text": "心室超速起搏有时十分有效,但偶尔会引起心室颤动。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "心室超速起搏" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "心室颤动" } ] }, { "text": "血培养阴性。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "血培养阴性" } ] }, { "text": "重复畸形输尿管开口的位置遵循Weigert-Meyer定律,引流下肾部的输尿管(下输尿管)口更靠近头端及外侧,引流上肾部的输尿管(上输尿管)口更靠近远端及中线,罕见其开口位于头端中线的。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "重复畸形输尿管开口" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "引流下肾部" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "输尿管" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "下输尿管" }, { "start_idx": 36, "end_idx": 53, "type": "sym", "entity": "输尿管(下输尿管)口更靠近头端及外侧" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "上肾部" }, { "start_idx": 61, "end_idx": 78, "type": "sym", "entity": "输尿管(上输尿管)口更靠近远端及中线" }, { "start_idx": 80, "end_idx": 91, "type": "sym", "entity": "罕见其开口位于头端中线的" } ] }, { "text": "如有输尿管膨出,在男女性均可表现出排尿困难和尿路感染。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "有输尿管膨出" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "排尿困难和尿路感染" } ] }, { "text": "异位开口的输尿管开口处常有狭窄,输尿管和其引流的上部肾常扩张积水,患儿可有腹痛、恶心及呕吐等症状。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "异位开口的输尿管" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "输尿管" }, { "start_idx": 13, "end_idx": 31, "type": "sym", "entity": "狭窄,输尿管和其引流的上部肾常扩张积水" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "腹痛" }, { "start_idx": 40, "end_idx": 47, "type": "sym", "entity": "恶心及呕吐等症状" } ] }, { "text": "引流下部肾的输尿管膀胱连接处结构可因另一输尿管的异位或膨出而被破坏,导致尿液反流的发生,患儿可有腰痛。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "bod", "entity": "引流下部肾的输尿管膀胱连接处" }, { "start_idx": 24, "end_idx": 49, "type": "sym", "entity": "异位或膨出而被破坏,导致尿液反流的发生,患儿可有腰痛" } ] }, { "text": "但如输尿管开口于前尿道或阴道,则要做膀胱镜,且仍很难发现。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "输尿管" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "尿道" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "阴道" }, { "start_idx": 18, "end_idx": 20, "type": "equ", "entity": "膀胱镜" } ] }, { "text": "输尿管重复畸形常常有肾积水和输尿管积水,非常容易被发现;如上部肾小、发育不良,输尿管常常也无扩张,则确诊有一定的难度。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "输尿管重复畸形" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾积水" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "输尿管积水" }, { "start_idx": 29, "end_idx": 47, "type": "sym", "entity": "上部肾小、发育不良,输尿管常常也无扩张" } ] }, { "text": "对有症状的Y型输尿管的处理,如两输尿管的会合点靠近膀胱壁,可切除该Y形连接部,分别做两输尿管与膀胱再吻合术;如反流严重而Y型会合点较高,则做汇合点以下输尿管与膀胱再吻合。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "Y型输尿管" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "输尿管" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "膀胱壁" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "输尿管" }, { "start_idx": 79, "end_idx": 80, "type": "bod", "entity": "膀胱" } ] }, { "text": "如无膀胱输尿管反流,而两输尿管间尿液往反流动严重并有症状,可做输尿管肾盂吻合,或肾盂与肾盂吻合。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "膀胱输尿管" }, { "start_idx": 31, "end_idx": 37, "type": "pro", "entity": "输尿管肾盂吻合" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "肾盂" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "肾盂" } ] }, { "text": "倒Y型重复输尿管的胚胎发生被认为是双输尿管芽的顶端在进入后肾前融合成一根而造成的。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "倒Y型重复输尿管" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "双输尿管芽" } ] }, { "text": "女性表现为阴蒂对裂,阴唇分开,间距增大及耻骨联合分离。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "阴蒂对裂" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "阴唇分开" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "耻骨" } ] }, { "text": "尿道上裂尿道成形的手术方法不及尿道下裂多,手术要点基本相同,充分矫正阴茎背曲畸形,正位尿道开口,但手术却更为复杂。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "pro", "entity": "尿道上裂尿道成形的手术" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "尿道下裂" }, { "start_idx": 30, "end_idx": 39, "type": "pro", "entity": "充分矫正阴茎背曲畸形" }, { "start_idx": 41, "end_idx": 46, "type": "pro", "entity": "正位尿道开口" } ] }, { "text": "主要手术方法包括Thierch-Duplay皮管尿道成形术、阴茎腹侧包皮岛状皮瓣尿道成形术以及Ransley尿道成形术等。", "entities": [ { "start_idx": 8, "end_idx": 28, "type": "pro", "entity": "Thierch-Duplay皮管尿道成形术" }, { "start_idx": 30, "end_idx": 44, "type": "pro", "entity": "阴茎腹侧包皮岛状皮瓣尿道成形术" }, { "start_idx": 47, "end_idx": 58, "type": "pro", "entity": "Ransley尿道成形术" } ] }, { "text": "蛋白尿可同时伴有尿中其他成分的增高,如出现血尿、脓尿、管型尿或尿量的改变,也可出现一些临床症状如水肿、高血压、贫血、腰痛、尿频及尿痛等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "血尿" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "脓尿" }, { "start_idx": 27, "end_idx": 29, "type": "sym", "entity": "管型尿" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "尿量的改变" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "水肿" }, { "start_idx": 51, "end_idx": 53, "type": "sym", "entity": "高血压" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "贫血" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "腰痛" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "尿频" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "尿痛" } ] }, { "text": "儿童期高血压是成年期原发性高血压的危险因素,其检出率有逐渐增加的趋势。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "儿童期高血压" }, { "start_idx": 7, "end_idx": 15, "type": "dis", "entity": "成年期原发性高血压" } ] }, { "text": "有学者推算,经重复测量后,儿童高血压的真正发病率低于5%,且只有不足1%的患者才需要治疗。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "儿童高血压" } ] }, { "text": "继发性高血压的临床表现与原发病有关。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "继发性高血压" } ] }, { "text": "与肾脏疾患相关者,常见生长发育落后,肾功能改变;与中枢有关者有颅内疾病、颅高压;与心脏有关者可及心脏杂音或外周血压改变;与内分泌有关者可出现色素沉着、生长异常、性征异常等。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "肾脏" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "生长发育落后" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "肾功能改变" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "中枢" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "颅内疾病" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "颅高压" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "心脏" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "心脏杂音" }, { "start_idx": 55, "end_idx": 56, "type": "ite", "entity": "血压" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "外周血压改变" }, { "start_idx": 61, "end_idx": 63, "type": "bod", "entity": "内分泌" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "色素沉着" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "生长异常" }, { "start_idx": 80, "end_idx": 83, "type": "sym", "entity": "性征异常" } ] }, { "text": "血压增高时可有头晕、头痛、恶心和呕吐,严重者有眩晕、视力障碍、惊厥、偏瘫、失语等累及眼底、脑和心血管的表现。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "血压" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "血压增高" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "头晕" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "头痛" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "恶心" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "呕吐" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "眩晕" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "视力障碍" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "惊厥" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "偏瘫" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "失语" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "眼底" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "脑" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "心血管" } ] }, { "text": "其他可有心悸、多汗、心动过速、体形变化、多毛、周期性肌张力低下、手足搐搦、多尿、烦渴等。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "心悸" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "多汗" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "心动过速" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "体形变化" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "多毛" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "周期性肌张力低下" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "手足搐搦" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "多尿" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "烦渴" } ] }, { "text": "病情严重者可引起充血性心力衰竭、肾衰竭、高血压脑病和视网膜病等并发症。", "entities": [ { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "高血压脑病" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "视网膜病" } ] }, { "text": "依据眼底检查情况将小儿高血压分为4度:Ⅰ度:正常眼底;Ⅱ度:有局灶性小动脉收缩;Ⅲ度:有渗出伴有或无出血;Ⅳ度:视神经乳头水肿。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "眼底检查" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "小儿高血压" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "正常眼底" }, { "start_idx": 30, "end_idx": 38, "type": "sym", "entity": "有局灶性小动脉收缩" }, { "start_idx": 43, "end_idx": 51, "type": "sym", "entity": "有渗出伴有或无出血" }, { "start_idx": 56, "end_idx": 62, "type": "sym", "entity": "视神经乳头水肿" } ] }, { "text": "疑汞中毒时应行尿汞测定。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "sym", "entity": "汞中毒" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "尿汞测定" } ] }, { "text": "传统的CCB缺乏儿科临床试验资料,新一代CCB中的苯磺酸氨氯地平(amlodipine)和非洛地平(felodipine)已弥补了这一缺憾。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "CCB" }, { "start_idx": 8, "end_idx": 9, "type": "dep", "entity": "儿科" }, { "start_idx": 20, "end_idx": 22, "type": "dep", "entity": "CCB" }, { "start_idx": 25, "end_idx": 31, "type": "dru", "entity": "苯磺酸氨氯地平" }, { "start_idx": 33, "end_idx": 42, "type": "dru", "entity": "amlodipine" }, { "start_idx": 45, "end_idx": 48, "type": "dru", "entity": "非洛地平" }, { "start_idx": 50, "end_idx": 59, "type": "dru", "entity": "felodipine" } ] }, { "text": "高血压急症的治疗,原则是将血压降至安全水平而非迅速降至正常,保证组织器官的灌注,防止器官损害的进一步发展。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "高血压急症" }, { "start_idx": 13, "end_idx": 14, "type": "ite", "entity": "血压" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "组织器官" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "器官" } ] }, { "text": "高血压紧急状态的治疗,目的在于在24小时内使血压降低。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "高血压" }, { "start_idx": 22, "end_idx": 23, "type": "ite", "entity": "血压" } ] }, { "text": "应先将患儿置于光线较暗、体位舒适的地方,30分钟后复测血压,若仍保持较高血压,先给予口服药治疗,如硝苯地平(心痛定)、卡托普利等,应监护患儿1~2小时,以确保治疗有效而无不良反应。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "ite", "entity": "血压" }, { "start_idx": 35, "end_idx": 37, "type": "sym", "entity": "高血压" }, { "start_idx": 42, "end_idx": 46, "type": "pro", "entity": "口服药治疗" }, { "start_idx": 49, "end_idx": 57, "type": "dru", "entity": "硝苯地平(心痛定)" }, { "start_idx": 59, "end_idx": 62, "type": "dru", "entity": "卡托普利" } ] }, { "text": "肿块位于胸锁乳突肌的下半部,右侧多于左侧,组织坚韧,切面呈灰白色,与周围肌肉组织混杂。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "胸锁乳突肌" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "组织" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "肌肉组织" } ] }, { "text": "克罗恩病可累及胃肠道各部位,呈慢性肉芽肿性炎症,以回肠末段及其邻近结肠最常受累。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克罗恩病" }, { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "慢性肉芽肿性炎症" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "回肠" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "结肠" } ] }, { "text": "痉挛性腹痛常于便前及便时发生,便后缓解。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "痉挛性腹痛" } ] }, { "text": "左下腹触痛明显,可有肌紧张或触及硬管状结肠。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "左下腹触痛明显" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "结肠" }, { "start_idx": 9, "end_idx": 20, "type": "sym", "entity": "有肌紧张或触及硬管状结肠" } ] }, { "text": "体重不增和生长发育迟缓亦是小儿UC最早期临床表现。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "UC" } ] }, { "text": "轻度:患者腹泻每日4次以下,便血轻或无,无发热、脉搏加快及贫血,血沉正常。", "entities": [ { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "腹泻每日4次以下" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "便血轻或无" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "无发热、脉搏加快及贫血" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "血沉正常" } ] }, { "text": "暴发型症状严重伴全身中毒性症状,可伴中毒性结肠扩张、肠穿孔及败血症等并发症。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "中毒性结肠扩张" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "肠穿孔" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "败血症" } ] }, { "text": "小儿全结肠炎约占62%。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "小儿全结肠炎" } ] }, { "text": "常见的并发症为肠出血、肠狭窄、肠穿孔、脓毒败血症及中毒性巨结肠。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "肠出血" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肠狭窄" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肠穿孔" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "脓毒败血症" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "中毒性巨结肠" } ] }, { "text": "腹痛是CD最常见的主诉,通常位于脐周,常发生于餐时或餐后,导致患儿不愿进食乃至厌食,只有回肠末端病变的腹痛位于右下腹部。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "腹痛" }, { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "CD" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "脐周" } ] }, { "text": "腹泻常见于90%患儿,可由多种因素所致,如大片肠黏膜功能紊乱、胆盐吸收障碍、细菌过度生长以及炎症性蛋白丢失等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "腹泻" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "黏膜功能紊乱" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "胆盐吸收障碍" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "炎症性蛋白丢失" } ] }, { "text": "上消化道的CD较少见,但也有经内镜与组织学检查证实胃十二指肠病变,往往难以与其他的疾病如胃食道反流、幽门螺杆菌感染及消化性溃疡等相鉴别。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "CD" }, { "start_idx": 15, "end_idx": 22, "type": "ite", "entity": "内镜与组织学检查" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "胃十二指肠病变" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "胃食道反流" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "消化性溃疡" } ] }, { "text": "体重减轻和生长迟缓是CD最常见也是最突出的症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "体重减轻" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "生长迟缓" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "CD" } ] }, { "text": "IBD患儿中生长激素水平是正常的,生长迟缓的原因是由于吸收不良,蛋白质丢失、热量摄入不足、蛋白质分解增加、多种维生素及微量元素缺乏等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "IBD" } ] }, { "text": "肠外表现有关节痛、关节炎、结节性红斑、杵状指、硬化性胆管炎及慢性活动性肝炎等。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "关节痛" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "关节炎" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "结节性红斑" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "杵状指" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "硬化性胆管炎" }, { "start_idx": 30, "end_idx": 36, "type": "dis", "entity": "慢性活动性肝炎" } ] }, { "text": "CD常见的并发症为肠梗阻、消化道出血、瘘管(腹腔内及肛周)、腹腔脓肿及肠穿孔。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "CD" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "消化道出血" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "瘘管" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "腹腔" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "肛周" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "腹腔脓肿" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "肠穿孔" } ] }, { "text": "克罗恩病可根据以下情况进行分型:①根据病变范围分:弥漫性小肠炎型、回肠末端型、回结肠型、结肠型及直肠肛门型。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "克罗恩病" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "弥漫性小肠炎型" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "回肠末端型" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "回结肠型" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "结肠型" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "直肠" } ] }, { "text": "临床表现以血性腹泻为特点,发作与缓解交替,腹泻也可表现为黏液便,可伴腹痛、里急后重、呕吐及厌食。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "血性腹泻" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "发作与缓解交替" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "也可表现为黏液便" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "可伴腹痛" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "里急后重" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "呕吐及厌食" } ] }, { "text": "常有明显的生长迟缓、贫血、发热、低蛋白血症等全身表现以及关节炎、虹膜睫状体炎、肝脾肿大等胃肠道外表现。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "生长迟缓" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "贫血" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "发热" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "低蛋白血症" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "关节炎" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "虹膜睫状体炎" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "肝脾肿大" } ] }, { "text": "UC与多数细菌性肠炎的主要区别在于症状持续时间。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "UC" } ] }, { "text": "溶组织阿米巴肠炎,症状持续数周至数月,大便呈暗红色果酱样,重者可为全血便,结肠镜下表现为灶性、出血性溃疡,中央开口下陷,呈烧瓶样,病灶之间黏膜正常。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "溶组织阿米巴肠炎" }, { "start_idx": 19, "end_idx": 27, "type": "sym", "entity": "大便呈暗红色果酱样" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "全血便" }, { "start_idx": 44, "end_idx": 51, "type": "sym", "entity": "灶性、出血性溃疡" }, { "start_idx": 53, "end_idx": 58, "type": "sym", "entity": "中央开口下陷" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "呈烧瓶样" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "黏膜" } ] }, { "text": "有条件者应作阿米巴血清学试验。", "entities": [ { "start_idx": 6, "end_idx": 13, "type": "ite", "entity": "阿米巴血清学试验" } ] }, { "text": "要排除急性阑尾炎、肠结核、其他慢性感染性肠炎(如耶尔森菌肠炎)、肠道淋巴瘤及溃疡性结肠炎等疾病。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "急性阑尾炎" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "肠结核" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "慢性感染性肠炎" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "耶尔森菌肠炎" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "肠道淋巴瘤" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "溃疡性结肠炎" } ] }, { "text": "回盲部的克罗恩病常常容易与急性阑尾炎混淆。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "克罗恩病" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "急性阑尾炎" } ] }, { "text": "如果患儿同时有肺结核,那么肠结核的诊断不难。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "肺结核" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肠结核" } ] }, { "text": "如有生殖系结核或伴其他器官结核,血中腺苷酸脱氨酶(ADA)活性增高,多考虑肠结核,肠结核的肠壁病变活体组织检查可有干酪样坏死及黏膜下层闭锁。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "生殖系结核" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "其他器官结核" }, { "start_idx": 18, "end_idx": 23, "type": "ite", "entity": "腺苷酸脱氨酶" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "肠结核" }, { "start_idx": 63, "end_idx": 64, "type": "bod", "entity": "黏膜" }, { "start_idx": 41, "end_idx": 68, "type": "sym", "entity": "肠结核的肠壁病变活体组织检查可有干酪样坏死及黏膜下层闭锁" } ] }, { "text": "小肠淋巴瘤的部分症状与克罗恩病也颇为相似,如发热、体重下降、腹泻及腹痛等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "小肠淋巴瘤" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "克罗恩病" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "发热" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "体重下降" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "腹泻及腹痛" } ] }, { "text": "频繁呕吐者应用适量解痉剂,并发感染者加用抗生素如甲硝唑等。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "解痉剂" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "抗生素" }, { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "甲硝唑" } ] }, { "text": "对CD有瘘管形成及脓肿者禁用。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "CD" }, { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "瘘管" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "脓肿" } ] }, { "text": "泼尼松和泼尼松龙:1~2mg/(kg•d),一日2~3次,共2~3周,症状缓解逐渐减量,隔日或间隙疗法[1mg/(kg•d)],持续4~6周,后再逐渐减量至停药,总疗程2~3个月。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "泼尼松" }, { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "泼尼松龙" } ] }, { "text": "4-氨基水杨酸(4-ASA),对UC有效。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dru", "entity": "4-氨基水杨酸" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "UC" } ] }, { "text": "研究最多的是TNF-α,使用TNF-α单抗治疗顽固性CD,取得突出疗效,目前该药已在英美等国批准投放市场。", "entities": [ { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "CD" } ] }, { "text": "有以重组IL-10治疗CD的临床试验的报告,但相继的临床报道不尽如人意。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "CD" } ] }, { "text": "UC患儿10年后有结肠癌的危险性,并逐年上升,故对病程10年以上患儿每6~12个月需行纤维结肠镜检查与活体组织检查。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "UC" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "结肠癌" } ] }, { "text": "本瘤是一边界不清、纤维组织和脂肪组织的混合体,直径一般在3~5cm。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "纤维组织" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "脂肪组织" } ] }, { "text": "本瘤好发于肩、腋窝、背、股和腹股沟等处,为无痛性生长迅速的肿块,质地随脂肪组织和纤维组织比例的变化而不同。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "肩" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "腋窝" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "背" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "股" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "腹股沟" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "肿块" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "脂肪组织" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "纤维组织" } ] }, { "text": "肿块位于皮下,有时和皮肤粘连。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "肿块" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "皮肤" } ] }, { "text": "1947年Jervis对病人进行苯丙氨酸负荷实验,揭示PKU发病的生化基础是肝脏苯丙氨酸代谢障碍。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "苯丙氨酸" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "PKU" }, { "start_idx": 38, "end_idx": 47, "type": "sym", "entity": "肝脏苯丙氨酸代谢障碍" } ] }, { "text": "1953年,德国的Bickel首先报道用低苯丙氨酸奶方治疗PKU病人获得成功。", "entities": [ { "start_idx": 20, "end_idx": 28, "type": "pro", "entity": "低苯丙氨酸奶方治疗" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "PKU" } ] }, { "text": "高浓度的Phe及其异常代谢产物抑制酪氨酸酶,使黑色素合成障碍。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "Phe" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "酪氨酸酶" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "黑色素" } ] }, { "text": "Phe增高影响脑发育,导致智能发育落后及出现小头畸形及抽搐等神经系统症状。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "Phe" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "智能发育落后" }, { "start_idx": 20, "end_idx": 28, "type": "sym", "entity": "出现小头畸形及抽搐" } ] }, { "text": "PKU属常染色体隐性遗传,其特点是:①患儿父母都是致病基因携带者(杂合子);②患儿从父母各得到一个致病基因,是纯合子;③患儿母亲每次生育有1/4可能性为PKU患儿;④近亲结婚的子女发病率较一般人群为高。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "PKU" }, { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "常染色体" }, { "start_idx": 76, "end_idx": 78, "type": "dis", "entity": "PKU" } ] }, { "text": "随着分子生物学技术的发展,北京及上海等地已经开展DNA序列分析等技术对PKU病人进行基因分析,在中国人群中发现了80种以上基因突变(表14-14),发现外显子7和12的突变占的比例相对较高。", "entities": [ { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "PKU" }, { "start_idx": 76, "end_idx": 82, "type": "bod", "entity": "外显子7和12" } ] }, { "text": "2/3患儿有轻微的神经系统体征,如肌张力增高、腱反射亢进及小头畸形等,严重者可有脑性瘫痪。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "神经系统体征" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "肌张力增高" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "腱反射亢进" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "小头畸形" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "脑性瘫痪" } ] }, { "text": "约1/4患儿有癫痫发作,常在18个月以前出现,可表现为婴儿痉挛性发作、点头样发作或其他形式。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "癫痫发作" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "婴儿痉挛性发作" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "点头样发作" } ] }, { "text": "约80%患儿有脑电图异常,异常表现以癫痫样放电为主。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "脑电图异常" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "癫痫样放电" } ] }, { "text": "经治疗后血Phe浓度下降,脑电图亦明显改善。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "sym", "entity": "血Phe浓度下降" } ] }, { "text": "PKU患者除了影响智能发育外,可出现一些行为、性格的异常,如忧郁、多动、自卑及孤僻等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "PKU" }, { "start_idx": 20, "end_idx": 27, "type": "sym", "entity": "行为、性格的异常" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "忧郁" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "多动" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "自卑" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "孤僻" } ] }, { "text": "约1/4病儿有癫痫发作。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "癫痫发作" } ] }, { "text": "诊断主要依靠HPLC测定尿中新蝶呤(N)和生物蝶呤(B)。", "entities": [ { "start_idx": 12, "end_idx": 27, "type": "ite", "entity": "尿中新蝶呤(N)和生物蝶呤(B)" } ] }, { "text": "如为二氢蝶呤还原酶(DHPR)缺乏时,N正常,B明显增加,N/B降低,B%可增高。", "entities": [ { "start_idx": 2, "end_idx": 16, "type": "dis", "entity": "二氢蝶呤还原酶(DHPR)缺乏" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "N正常" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "B明显增加" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "N/B降低" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "B%可增高" } ] }, { "text": "新生儿筛查即是通过测定血苯丙氨酸,在群体中对每个新生儿进行筛检,使PKU患儿在临床症状尚未出现之前,而其生化等方面的改变已比较明显时得以早期诊断和早期治疗,避免智能落后的发生。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "ite", "entity": "血苯丙氨酸" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "PKU" } ] }, { "text": "这两种试验阳性反应也可见于枫糖尿病及胱氨酸血症,故并非为PKU特异性试验,需进一步作血苯丙氨酸测定才能确诊。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "枫糖尿病" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "胱氨酸血症" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "PKU" }, { "start_idx": 42, "end_idx": 48, "type": "ite", "entity": "血苯丙氨酸测定" } ] }, { "text": "PKU患者的智能障碍是由于体内过量的Phe及旁路代谢产物的神经毒性作用而引起,要防止脑损伤,只有减少从食物中摄取苯丙氨酸。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "PKU" }, { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "Phe" } ] }, { "text": "过度治疗将导致苯丙氨酸缺乏,出现嗜睡、厌食贫血、腹泻,甚至死亡。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "苯丙氨酸缺乏" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "嗜睡" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "厌食贫血" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "腹泻" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "死亡" } ] }, { "text": "由于STR多态连锁分析不是直接检测基因突变,因此在应用中必须注意临床诊断的准确性,千万不能将非PAH基因突变的PKU当成PAH突变的病例来进行连锁分析。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "STR多态连锁分析" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "检测基因突变" }, { "start_idx": 46, "end_idx": 57, "type": "dis", "entity": "非PAH基因突变的PKU" }, { "start_idx": 71, "end_idx": 74, "type": "pro", "entity": "连锁分析" } ] }, { "text": "母乳是婴儿最理想的天然食品,对哺乳期病儿在确诊后虽应暂停母乳喂养,但切勿断奶,以便在控制血苯丙氨酸浓度后能及时添加。", "entities": [ { "start_idx": 44, "end_idx": 50, "type": "ite", "entity": "血苯丙氨酸浓度" } ] }, { "text": "血Phe浓度监测在餐后2小时采血。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "血Phe" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "采血" } ] }, { "text": "听诊时啰音不多,1~2周内呼吸困难逐渐加重。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "听诊" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "啰音不多" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "此外,常见症状为呼吸加速、咳嗽、发绀、三凹、鼻扇及腹泻。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "呼吸加速" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "咳嗽" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "发绀" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "三凹" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "鼻扇" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "腹泻" } ] }, { "text": "由于其主要的病理改变是广泛的肾小球新月体形成,因此,RPGN也常从病理角度被叫做“新月体性肾炎”。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "肾小球新月体" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "RPGN" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "新月体性肾炎" } ] }, { "text": "此外,RPGN多在2~3个月内出现肾衰竭,因而从肾衰出现时间上也有时被称为“亚急性肾小球肾炎”。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "RPGN" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "肾衰竭" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "肾衰" }, { "start_idx": 38, "end_idx": 45, "type": "dis", "entity": "亚急性肾小球肾炎" } ] }, { "text": "③Ⅲ型:少或无沉积(寡免疫反应型);包括:a.抗中性粒细胞胞浆抗体阳性,b.抗中性粒细胞抗体阴性。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "寡免疫反应型" }, { "start_idx": 23, "end_idx": 34, "type": "sym", "entity": "抗中性粒细胞胞浆抗体阳性" }, { "start_idx": 38, "end_idx": 47, "type": "sym", "entity": "抗中性粒细胞抗体阴性" } ] }, { "text": "免疫荧光检查可分别出现线状、颗粒状IgG沉积或者无Ig沉积。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "免疫荧光检查" }, { "start_idx": 11, "end_idx": 28, "type": "sym", "entity": "线状、颗粒状IgG沉积或者无Ig沉积" } ] }, { "text": "电镜下可见新月体内除上皮细胞外,尚有纤维素及红细胞,肾小球基底膜断裂及纤维素样沉积,内皮下及系膜区甚至上皮下可见电子致密物。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "equ", "entity": "电镜" }, { "start_idx": 5, "end_idx": 24, "type": "sym", "entity": "新月体内除上皮细胞外,尚有纤维素及红细胞" }, { "start_idx": 26, "end_idx": 40, "type": "sym", "entity": "肾小球基底膜断裂及纤维素样沉积" }, { "start_idx": 42, "end_idx": 60, "type": "sym", "entity": "内皮下及系膜区甚至上皮下可见电子致密物" } ] }, { "text": "1/3~1/2有前驱病史,表现为病前2~3周内出现发热、乏力、关节痛及肌痛等上感症状或非特异性表现。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "发热" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "乏力" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "关节痛" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "肌痛" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "上感症状" } ] }, { "text": "但2~3周后,上述症状不仅不能缓解,反而加剧,出现持续性少尿、严重高血压及循环充血。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "少尿" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "高血压" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "充血" } ] }, { "text": "ANCA可分为C-ANCA及P-ANCA,前者阳性主要见于韦格肉芽肿,后者阳性主要见于显微镜下结节性多动脉炎即所谓特发性RPGN,该病可能是显微镜下结节性多动脉炎的一种特殊形式,仅局限于肾小球毛细血管内。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "ANCA" }, { "start_idx": 7, "end_idx": 12, "type": "ite", "entity": "C-ANCA" }, { "start_idx": 14, "end_idx": 19, "type": "ite", "entity": "P-ANCA" }, { "start_idx": 43, "end_idx": 45, "type": "equ", "entity": "显微镜" }, { "start_idx": 57, "end_idx": 63, "type": "dis", "entity": "特发性RPGN" }, { "start_idx": 70, "end_idx": 72, "type": "equ", "entity": "显微镜" }, { "start_idx": 93, "end_idx": 99, "type": "bod", "entity": "肾小球毛细血管" } ] }, { "text": "通过实验室检查及肾脏病理检查有望明确RPGN的病因。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "实验室检查" }, { "start_idx": 8, "end_idx": 13, "type": "pro", "entity": "肾脏病理检查" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "RPGN" } ] }, { "text": "随着诊治水平的提高,特别是甲泼尼龙冲击疗法及血浆置换等技术的应用,近来疗效已大为提高。", "entities": [ { "start_idx": 13, "end_idx": 20, "type": "pro", "entity": "甲泼尼龙冲击疗法" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "血浆置换" } ] }, { "text": "肾衰竭后还应摄入低蛋白饮食,每日热量230~251kJ/kg(55~60kcal/kg),以维持基础代谢及氮平衡。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "自然缓解少见,但在感染基础上形成抗原抗体复合物的病人,当抗原清除后,可自行缓解。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "感染" } ] }, { "text": "另一类主要为气管纤维性狭窄或闭锁,仅累及较短的一段气管,可同时有气管内隔膜形成。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "气管纤维性狭窄或闭锁" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "气管" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "气管内隔膜" } ] }, { "text": "气管狭窄的表现视其程度而定。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "气管狭窄" } ] }, { "text": "轻度狭窄常无症状,较严重时,可出现气急,发绀,吸气时可闻喘鸣音。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "轻度狭窄" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "气急" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "发绀" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "吸气时可闻喘鸣音" } ] }, { "text": "并发急性呼吸道炎症时,则出现严重呼吸困难、烦躁、鼻扇、口唇及面部发绀、三凹征等。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "急性呼吸道炎症" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "严重呼吸困难" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "烦躁" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "鼻扇" }, { "start_idx": 27, "end_idx": 33, "type": "sym", "entity": "口唇及面部发绀" }, { "start_idx": 35, "end_idx": 37, "type": "sym", "entity": "三凹征" } ] }, { "text": "气管镜下可以直视狭窄的部位、范围及程度。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "equ", "entity": "气管镜" } ] }, { "text": "轻度狭窄易被漏诊,重度狭窄患儿生后即有明显症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "轻度狭窄" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "重度狭窄" } ] }, { "text": "螺旋CT三维重建可清楚地显示气管结构。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "螺旋CT三维重建" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "气管" } ] }, { "text": "轻度狭窄无需治疗,重度狭窄严重影响呼吸及生长发育者,可视其情况进行手术。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "轻度狭窄" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "重度狭窄" }, { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "严重影响呼吸及生长发育" }, { "start_idx": 33, "end_idx": 34, "type": "pro", "entity": "手术" } ] }, { "text": "狭窄段短者行气管狭窄段切除、端端吻合术。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "狭窄段短" }, { "start_idx": 6, "end_idx": 12, "type": "pro", "entity": "气管狭窄段切除" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "端端吻合术" } ] }, { "text": "长段狭窄者可采用自体或人胚胎气管移植、气管成形术、球囊扩张、金属支架等方法。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "长段狭窄" }, { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "胚胎气管移植" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "气管成形术" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "球囊扩张" }, { "start_idx": 30, "end_idx": 33, "type": "pro", "entity": "金属支架" } ] }, { "text": "临床表现为新生儿呼吸困难,反复呼吸道感染和气胸。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "反复呼吸道感染" } ] }, { "text": "体格检查肺呼吸音减弱,伴纵隔向健侧移位。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "体格检查" }, { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "肺呼吸音减弱" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "伴纵隔向健侧移位" } ] }, { "text": "胸片表现囊性肿块伴纵隔移位,如有气液平提示肺脓疡。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "胸片" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "肺脓疡" } ] }, { "text": "治疗为手术切除受累肺叶或段。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "肺叶" }, { "start_idx": 12, "end_idx": 12, "type": "sym", "entity": "段" } ] }, { "text": "目前通过超声波检查在产前即可诊断出本病,从而为产后及时治疗甚至宫内手术提供了可能。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "ite", "entity": "超声波检查" } ] }, { "text": "各种先天性原因所致的脑发育障碍,常有不同程度的大脑皮质萎缩和脑室扩大,可有神经细胞减少和胶质细胞增生。", "entities": [ { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "脑" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "脑发育障碍" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "大脑皮质" }, { "start_idx": 18, "end_idx": 28, "type": "sym", "entity": "不同程度的大脑皮质萎缩" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "脑室" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "脑室扩大" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "神经细胞" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "神经细胞减少" }, { "start_idx": 44, "end_idx": 47, "type": "bod", "entity": "胶质细胞" }, { "start_idx": 44, "end_idx": 49, "type": "sym", "entity": "胶质细胞增生" } ] }, { "text": "②肌张力异常:表现为肌张力亢进、肌强直、肌张力低下及肌张力不协调。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "肌张力异常" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "肌张力亢进" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "肌强直" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "肌张力不协调" } ] }, { "text": "③姿势异常:静止时姿势如紧张性颈反射姿势,四肢强直姿势,角弓反张姿势,偏瘫姿势;活动时姿势异常如舞蹈样手足徐动及扭转痉挛,痉挛性截瘫步态,小脑共济失调步态。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "姿势异常" }, { "start_idx": 6, "end_idx": 19, "type": "sym", "entity": "静止时姿势如紧张性颈反射姿势" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "四肢强直姿势" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "角弓反张姿势" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "偏瘫姿势" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "活动时姿势异常" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "舞蹈样手足徐动" }, { "start_idx": 56, "end_idx": 59, "type": "sym", "entity": "扭转痉挛" }, { "start_idx": 61, "end_idx": 67, "type": "sym", "entity": "痉挛性截瘫步态" }, { "start_idx": 69, "end_idx": 76, "type": "sym", "entity": "小脑共济失调步态" } ] }, { "text": "④反射异常:表现为原始反射延缓消失、保护性反射延缓出现以及Vojta姿势反射样式异常,Vojta姿势反射包括牵拉反射、抬躯反射、Collin水平及垂直反射、立位和倒位及斜位悬垂反射。", "entities": [ { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "原始反射延缓消失" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "保护性反射延缓" }, { "start_idx": 29, "end_idx": 41, "type": "sym", "entity": "Vojta姿势反射样式异常" } ] }, { "text": "上肢屈肌张力增高,表现为肩关节内收,肘关节、腕关节及手指关节屈曲。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "肩关节" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "肩关节内收" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "肘关节" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "腕关节" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "手指关节" } ] }, { "text": "瘫痪侧肢体自发运动减少,行走延迟,偏瘫步态,患肢足尖着地。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "瘫痪侧肢体自发运动减少" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "行走延迟" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "偏瘫步态" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "患肢足尖着地" } ] }, { "text": "约25%的患儿有认知功能异常,智力低下。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "认知功能异常" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "智力低下" } ] }, { "text": "主要病变在锥体外系统,表现为难以用意志控制的不自主运动,当进行有意识运动时,不自主、不协调及无效的运动增多。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "病变在锥体外系统" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "难以用意志控制的不自主运动" }, { "start_idx": 38, "end_idx": 52, "type": "sym", "entity": "不自主、不协调及无效的运动增多" } ] }, { "text": "多有肌张力降低,抬头无力,喂养困难,常有舌伸出口外及流涎。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "肌张力降低" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "抬头无力" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "喂养困难" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "舌" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "舌伸出口外" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "流涎" } ] }, { "text": "1岁后手足徐动逐渐明显,因口肌受累呈显著语言困难,说话时语句含糊,声调调节也受累。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "手足徐动逐渐明显" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "口肌" }, { "start_idx": 13, "end_idx": 23, "type": "sym", "entity": "口肌受累呈显著语言困难" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "语句含糊" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "声调调节也受累" } ] }, { "text": "主要病变在小脑。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "小脑" } ] }, { "text": "临床表现为步态不稳,走路时两足间距加宽,四肢动作不协调,上肢常有意向性震颤,快变转化的动作差,指鼻试验易错误,肌张力低下。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "步态不稳" }, { "start_idx": 10, "end_idx": 18, "type": "sym", "entity": "走路时两足间距加宽" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "四肢动作不协调" }, { "start_idx": 28, "end_idx": 36, "type": "sym", "entity": "上肢常有意向性震颤" }, { "start_idx": 38, "end_idx": 45, "type": "sym", "entity": "快变转化的动作差" }, { "start_idx": 55, "end_idx": 59, "type": "sym", "entity": "肌张力低下" } ] }, { "text": "肌张力低下型(atonia)表现为肌张力低下,四肢呈软瘫状,自主运动很少。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "肌张力低下型" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "atonia" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "四肢呈软瘫状" }, { "start_idx": 30, "end_idx": 35, "type": "sym", "entity": "自主运动很少" } ] }, { "text": "诊断脑性瘫痪应符合以下2个条件:①婴儿时期出现症状(如运动发育落后或各种运动障碍);②需除外进行性疾病(如各种代谢病或变性疾病)所致的中枢性瘫痪及正常小儿一过性发育落后。", "entities": [ { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "运动发育落后" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "各种运动障碍" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "中枢性瘫痪" } ] }, { "text": "主要适应证为痉挛性脑瘫患儿,目的在于矫正畸形、改善肌张力以及改善肢体平衡。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "痉挛性脑瘫" } ] }, { "text": "手术包括肌腱手术、神经手术以及骨关节手术等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "神经手术" }, { "start_idx": 15, "end_idx": 19, "type": "pro", "entity": "骨关节手术" } ] }, { "text": "恶性淋巴管瘤在小儿极其罕见。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "恶性淋巴管瘤" } ] }, { "text": "淋巴管瘤一般采用Wegner分类方法:①毛细淋巴管瘤;②海绵状淋巴管瘤;③囊状淋巴管瘤;④弥漫性淋巴管瘤。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "淋巴管瘤" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "毛细淋巴管瘤" }, { "start_idx": 28, "end_idx": 34, "type": "dis", "entity": "海绵状淋巴管瘤" }, { "start_idx": 37, "end_idx": 42, "type": "dis", "entity": "囊状淋巴管瘤" }, { "start_idx": 45, "end_idx": 51, "type": "dis", "entity": "弥漫性淋巴管瘤" } ] }, { "text": "多见于唇、口腔、舌、头皮、胸壁和外生殖器。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "唇" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "口腔" }, { "start_idx": 8, "end_idx": 8, "type": "bod", "entity": "舌" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "头皮" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "胸壁" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "外生殖器" } ] }, { "text": "手术是唯一的有效疗法。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" } ] }, { "text": "CT检查还有助于观察患侧肺部病变。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "CT检查" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肺部" } ] }, { "text": "如有上述典型症状和体征,结合胸部X线检查,不难做出诊断,但进一步明确胸膜炎的性质,则要通过胸腔穿刺抽出积液,进行实验室检查,后者有助于与化脓性胸膜炎、漏出性胸水、血胸、乳糜胸的鉴别。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "ite", "entity": "胸部X线检查" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "胸膜炎" }, { "start_idx": 51, "end_idx": 52, "type": "bod", "entity": "积液" }, { "start_idx": 56, "end_idx": 60, "type": "ite", "entity": "实验室检查" }, { "start_idx": 68, "end_idx": 73, "type": "dis", "entity": "化脓性胸膜炎" }, { "start_idx": 75, "end_idx": 79, "type": "dis", "entity": "漏出性胸水" }, { "start_idx": 81, "end_idx": 82, "type": "dis", "entity": "血胸" }, { "start_idx": 84, "end_idx": 86, "type": "dis", "entity": "乳糜胸" } ] }, { "text": "对结核性胸膜炎,在抗结核治疗同时可加用肾上腺皮质激素,以减轻中毒症状,促进胸水吸收,减少胸膜增厚和粘连的发生。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "结核性胸膜炎" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "抗结核" }, { "start_idx": 19, "end_idx": 25, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "减轻中毒症状" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "促进胸水吸收" } ] }, { "text": "和其他风湿病相比,血管炎综合征男孩受累多于女孩。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "风湿病" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "血管炎综合征" } ] }, { "text": "切面带黏性,灰白色间隔黄褐色的出血区。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "切面带黏性" }, { "start_idx": 6, "end_idx": 17, "type": "sym", "entity": "灰白色间隔黄褐色的出血区" } ] }, { "text": "治疗以整块肿瘤切除为主或做截肢术。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "肿瘤切除" }, { "start_idx": 13, "end_idx": 15, "type": "pro", "entity": "截肢术" } ] }, { "text": "此外,也可由原虫感染和真菌感染引起。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "原虫感染" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "真菌感染" } ] }, { "text": "早期高凝状态一般无出血症状,但可表现皮肤青紫、低血压,凝血时间缩短。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "出血" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "皮肤青紫" }, { "start_idx": 23, "end_idx": 25, "type": "sym", "entity": "低血压" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "凝血时间缩短" } ] }, { "text": "消耗性低凝状态,常伴出血,逐渐加重;在继发性纤溶时,出血更严重。", "entities": [ { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "常伴出血,逐渐加重" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "出血更严重" } ] }, { "text": "表现为畏寒、发热、黄疸、腰痛、酱油色尿、少尿或无尿、面色进行性苍白、乏力,外周血涂片可见大量红细胞碎片。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "畏寒" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "发热" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "黄疸" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "腰痛" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "酱油色尿" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "少尿" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "无尿" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "面色进行性苍白" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "乏力" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "红细胞" }, { "start_idx": 37, "end_idx": 50, "type": "sym", "entity": "外周血涂片可见大量红细胞碎片" } ] }, { "text": "双嘧达莫(潘生丁)有抗血小板凝聚和扩张血管的作用,在DIC的治疗中也可取得良好效果,用法:10ml/(kg•d),静脉滴注。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "双嘧达莫" }, { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "潘生丁" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "血小板" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "血管" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "DIC" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "糖皮质激素,皮质激素对治疗原发病可能有效,但对DIC的治疗效果目前尚未取得一致意见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "糖皮质激素" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "皮质激素" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "原发病" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "DIC" } ] }, { "text": "根据头痛的严重程度可分为轻度头痛、中度头痛和重度头痛。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "轻度头痛" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "中度头痛" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "重度头痛" } ] }, { "text": "根据病因可分为原发性头痛(如偏头痛和紧张性头痛等)和继发性头痛(如因感染及外伤等所致的头痛)。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "原发性头痛" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "偏头痛" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "紧张性头痛" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "继发性头痛" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "头痛" } ] }, { "text": "③头孢霉素类:以第一代头孢霉素最明显。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "头孢霉素" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "头孢霉素" } ] }, { "text": "⑤四环素族:增加蛋白分解,加重氮质血症。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "增加蛋白分解" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "氮质血症" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "加重氮质血症" } ] }, { "text": "⑥两性霉素B。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dru", "entity": "两性霉素B" } ] }, { "text": "⑦万古霉素以及磺胺类药物。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "万古霉素" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "磺胺类药物" } ] }, { "text": "慢性间质性肾炎可由非类固醇抗炎止痛药以及含马兜铃酸的中草药所引起,用药时间往往长达数月以上。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "慢性间质性肾炎" }, { "start_idx": 9, "end_idx": 17, "type": "dru", "entity": "非类固醇抗炎止痛药" }, { "start_idx": 21, "end_idx": 28, "type": "dru", "entity": "马兜铃酸的中草药" } ] }, { "text": "但表现为肾衰竭的患儿则不宜大量饮水,以免增加容量负荷。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "肾衰竭" } ] }, { "text": "对于表现为肾病综合征或肾炎综合征的药物性肾损害也可酌情使用肾上腺皮质激素。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "肾炎综合征" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "药物性肾损害" }, { "start_idx": 29, "end_idx": 35, "type": "dru", "entity": "肾上腺皮质激素" } ] }, { "text": "呼吸窘迫。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "呼吸窘迫" } ] }, { "text": "也有报道认为FX在男孩中的发病率为1∶1500,在女孩中为1∶2500。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "FX" } ] }, { "text": "不随意运动迟缓、关节过度强直及全身反射亢进等。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "不随意运动迟缓" }, { "start_idx": 8, "end_idx": 13, "type": "sym", "entity": "关节过度强直" }, { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "全身反射亢进" } ] }, { "text": "对遗传代谢病进行早期临床诊断是改善患儿预后的前提,但随着现代研究手段和生化测定技术的不断提高,遗传代谢病的研究也将愈加深入,新生儿疾病筛查的开展,质谱技术及基因诊断技术在临床的应用,促进疾病诊断水平不断提高。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "遗传代谢病" } ] }, { "text": "先天性代谢缺陷有时是全身性的,有时是相对局部性的,这取决于底物分子的大小及理化性质。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "先天性代谢缺陷" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "底物分子" } ] }, { "text": "小分子物质(如苯丙氨酸、有机酸及单糖)因易扩散,故异常堆积后往往会弥漫至全身多种组织、细胞而引起全身性病变,这类遗传代谢病的特点是起病早,如在新生儿期起病者可表现为急性脑病的发作。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "苯丙氨酸" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "有机酸" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "单糖" }, { "start_idx": 25, "end_idx": 44, "type": "sym", "entity": "异常堆积后往往会弥漫至全身多种组织、细胞" }, { "start_idx": 46, "end_idx": 52, "type": "sym", "entity": "引起全身性病变" }, { "start_idx": 82, "end_idx": 85, "type": "dis", "entity": "急性脑病" } ] }, { "text": "大分子物质(如黏多糖、糖原及类脂等)集中在溶酶体内代谢,则不易扩散,因此在酶缺陷时常主要堆积在细胞或细胞器内,引起溶酶体肿胀,其特点常在较大婴儿或者儿童期起病,多呈慢性、进行性病变过程。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "黏多糖" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "糖原" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "类脂" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "溶酶体" }, { "start_idx": 37, "end_idx": 39, "type": "sym", "entity": "酶缺陷" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "细胞" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "细胞器" }, { "start_idx": 57, "end_idx": 61, "type": "sym", "entity": "溶酶体肿胀" } ] }, { "text": "有些遗传代谢病在体检中能嗅到一些不正常的汗味或尿味,可能提示某些遗传病,有异常气味的遗传疾病(表14-14)。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "不正常的汗味或尿味" } ] }, { "text": "尿液三氯化铁试验用于检测尿中的苯丙酮酸,除了对苯丙酮尿症的检测有用外,其他很多化合物也能与三氯化铁反应,生成不同颜色的复合物,由此初步鉴别各种遗传代谢病(表14-10);尿液二硝基苯肼试验是根据二硝基苯肼与α-酮酸反应生成有沉淀的肼,出现黄色荧光而判断为阳性反应。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "尿液三氯化铁试验" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "尿" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "苯丙酮酸" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "苯丙酮尿症" }, { "start_idx": 71, "end_idx": 75, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 85, "end_idx": 93, "type": "pro", "entity": "尿液二硝基苯肼试验" } ] }, { "text": "根据DNPH反应与酮体存在与否可以鉴别一些代谢病(表14-11)。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "DNPH反应" } ] }, { "text": "血、尿常规分析、生化检测,如血糖、血气分析、肝功能、胆红素、血氨、乳酸、酮体、丙酮酸、肌酐、尿素、电解质、钙及磷测定,有助于对遗传代谢病作出初步的判断或者缩小诊断范围。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "pro", "entity": "血" }, { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "尿常规分析" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "生化检测" }, { "start_idx": 14, "end_idx": 15, "type": "ite", "entity": "血糖" }, { "start_idx": 17, "end_idx": 20, "type": "ite", "entity": "血气分析" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "肝功能" }, { "start_idx": 26, "end_idx": 28, "type": "ite", "entity": "胆红素" }, { "start_idx": 30, "end_idx": 31, "type": "ite", "entity": "血氨" }, { "start_idx": 33, "end_idx": 34, "type": "ite", "entity": "乳酸" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "酮体" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "丙酮酸" }, { "start_idx": 43, "end_idx": 44, "type": "ite", "entity": "肌酐" }, { "start_idx": 46, "end_idx": 47, "type": "ite", "entity": "尿素" }, { "start_idx": 49, "end_idx": 51, "type": "ite", "entity": "电解质" }, { "start_idx": 53, "end_idx": 57, "type": "ite", "entity": "钙及磷测定" } ] }, { "text": "遗传代谢病是基因突变引起的终生性疾病,然而由于有时肝肾功能异常或者服用药物也可导致代谢改变,所以对代谢异常的判断需结合临床分析,并且经过多次验证。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "基因突变" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "终生性疾病" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "肝肾功能异常" } ] }, { "text": "基因突变检测能明确遗传学改变,有利于进一步的遗传咨询和产前诊断,值得推广。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "基因突变检测" } ] }, { "text": "对于怀疑遗传代谢病濒临死亡的婴儿,应留取适当的标本,以便进行分析,明确病因,为以后的遗传咨询和产前诊断提供依据。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "遗传咨询" }, { "start_idx": 47, "end_idx": 50, "type": "pro", "entity": "产前诊断" } ] }, { "text": "常用的标本为:①尿液,冰冻保存;②皮肤,保存于组织培养液中,进行成纤维细胞培养;③血液,滴于新生儿筛查滤纸上,或者分离血清和白细胞,-20℃保存;④肝组织,应在死后立即留取,并在-20℃保存;⑤进行尸体解剖,取一些组织用于电镜分析。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "尿液" }, { "start_idx": 17, "end_idx": 18, "type": "ite", "entity": "皮肤" }, { "start_idx": 41, "end_idx": 42, "type": "ite", "entity": "血液" }, { "start_idx": 74, "end_idx": 76, "type": "ite", "entity": "肝组织" }, { "start_idx": 99, "end_idx": 102, "type": "ite", "entity": "尸体解剖" }, { "start_idx": 111, "end_idx": 114, "type": "ite", "entity": "电镜分析" } ] }, { "text": "部分遗传代谢病在新生儿或者婴儿早期即可有临床表现:①胃肠道:喂养困难、食欲差、呕吐、脱水、电解质异常、体重不增及肝大等;②呼吸系统:呼吸困难、酸中毒及过度换气等;③神经肌肉:嗜睡、惊厥、昏迷及肌张力异常等;④其他:皮肤病变、毛发异常、特殊尿味及汗味等。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "胃肠道" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "喂养困难" }, { "start_idx": 35, "end_idx": 37, "type": "sym", "entity": "食欲差" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "呕吐" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "脱水" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "电解质异常" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "体重不增" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "肝大" }, { "start_idx": 61, "end_idx": 64, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 66, "end_idx": 69, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 71, "end_idx": 73, "type": "sym", "entity": "酸中毒" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "过度换气" }, { "start_idx": 82, "end_idx": 85, "type": "bod", "entity": "神经肌肉" }, { "start_idx": 87, "end_idx": 88, "type": "sym", "entity": "嗜睡" }, { "start_idx": 90, "end_idx": 91, "type": "sym", "entity": "惊厥" }, { "start_idx": 93, "end_idx": 94, "type": "sym", "entity": "昏迷" }, { "start_idx": 96, "end_idx": 100, "type": "sym", "entity": "肌张力异常" }, { "start_idx": 107, "end_idx": 110, "type": "sym", "entity": "皮肤病变" }, { "start_idx": 112, "end_idx": 115, "type": "sym", "entity": "毛发异常" }, { "start_idx": 117, "end_idx": 123, "type": "sym", "entity": "特殊尿味及汗味" } ] }, { "text": "遗传代谢病患儿出生早期不易被发觉,常表现有嗜睡、进食少,易误诊为败血症,可因呼吸暂停或呼吸窘迫被注意。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "嗜睡" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "进食少" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "败血症" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "呼吸窘迫" } ] }, { "text": "呼吸暂停提示中枢病变,是代谢性脑病的表现;呼吸过快可能是进行性代谢性酸中毒的表现,如尿素循环缺陷,伴高氨血症昏迷,表现为中枢性过度通气,导致呼吸性碱中毒。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "中枢病变" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "代谢性脑病" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "呼吸过快" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "尿素循环缺陷" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "高氨血症昏迷" }, { "start_idx": 60, "end_idx": 66, "type": "sym", "entity": "中枢性过度通气" }, { "start_idx": 70, "end_idx": 75, "type": "sym", "entity": "呼吸性碱中毒" } ] }, { "text": "呕吐是遗传代谢病的另一突出症状,在较大婴儿中更常见,主要是对蛋白质不耐受所引起。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "呕吐" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "蛋白质" } ] }, { "text": "常有一些呕吐婴儿被误诊为幽门狭窄而行手术治疗,术后才被诊断为遗传代谢病。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "呕吐" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "幽门狭窄" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "手术治疗" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "遗传代谢病" } ] }, { "text": "遗传代谢病婴儿可继发败血症,因此,出现败血症时并不能排除遗传代谢病的可能。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "败血症" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "败血症" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "遗传代谢病" } ] }, { "text": "若不治疗,这些患儿可由嗜睡发展为昏迷,并可出现其他神经系统症状,如抽搐和肌张力增高,有时可出现脑水肿或颅内出血的症状。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "嗜睡" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "昏迷" }, { "start_idx": 23, "end_idx": 30, "type": "sym", "entity": "其他神经系统症状" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "抽搐" }, { "start_idx": 36, "end_idx": 40, "type": "bod", "entity": "肌张力增高" }, { "start_idx": 47, "end_idx": 49, "type": "sym", "entity": "脑水肿" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "颅内出血" } ] }, { "text": "因此,对于任何一种临床表现提示遗传代谢病的婴儿,即使未出现代谢性酸中毒或高氨血症,都应该进行实验室检查,包括全血常规、尿常规分析、血气分析、血电解质、血糖、血氨及乳酸等检查。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "高氨血症" }, { "start_idx": 54, "end_idx": 57, "type": "ite", "entity": "全血常规" }, { "start_idx": 59, "end_idx": 63, "type": "ite", "entity": "尿常规分析" }, { "start_idx": 65, "end_idx": 68, "type": "ite", "entity": "血气分析" }, { "start_idx": 70, "end_idx": 73, "type": "ite", "entity": "血电解质" }, { "start_idx": 75, "end_idx": 76, "type": "ite", "entity": "血糖" }, { "start_idx": 78, "end_idx": 79, "type": "ite", "entity": "血氨" }, { "start_idx": 81, "end_idx": 82, "type": "ite", "entity": "乳酸" } ] }, { "text": "同时还需进行串联质谱和气相色谱质谱的氨基酸、有机酸及肉碱代谢等的检查。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "串联质谱" }, { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "气相色谱质谱" }, { "start_idx": 18, "end_idx": 20, "type": "ite", "entity": "氨基酸" }, { "start_idx": 22, "end_idx": 24, "type": "ite", "entity": "有机酸" }, { "start_idx": 26, "end_idx": 29, "type": "ite", "entity": "肉碱代谢" } ] }, { "text": "在新生儿期表现为急性脑病的常见遗传代谢病实验室检查(表14-14)。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "急性脑病" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "遗传代谢病" } ] }, { "text": "当一个危重婴儿被怀疑患有遗传代谢病,如有机酸血症或尿素循环缺陷时,即使没有确诊,也应开始紧急处理。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "尿素循环缺陷" } ] }, { "text": "对这类患儿的紧急处理可达到两个目的,首先是清除体内沉积的代谢物,如有机酸中间代谢产物或氨。", "entities": [ { "start_idx": 21, "end_idx": 30, "type": "pro", "entity": "清除体内沉积的代谢物" }, { "start_idx": 33, "end_idx": 41, "type": "bod", "entity": "有机酸中间代谢产物" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "氨" } ] }, { "text": "病情危重伴高氨血症的婴儿应进行血液透析。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "血液透析" } ] }, { "text": "腹膜透析、持续动静脉灌注和交换输血,但效果都不如血液透析。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "持续动静脉灌注" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "交换输血" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "血液透析" } ] }, { "text": "对多种羧化酶缺乏症,给予口服或通过鼻饲生物素10mg治疗。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "羧化酶缺乏症" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "口服" }, { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "鼻饲生物素" } ] }, { "text": "若存在酸中毒,应静脉给予足量的碳酸氢钠,因为在这类疾病中有机酸和乳酸代谢产物是进行性增加的。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "sym", "entity": "酸中毒" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "碳酸氢钠" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "有机酸" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "乳酸代谢产物" } ] }, { "text": "治疗期间应经常作血气分析,以便及时调整治疗方案。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "血气分析" } ] }, { "text": "有机酸血症新生儿出现严重酸中毒时,无论是否伴有高氨血症均应考虑透析治疗。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "有机酸血症" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "高氨血症" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "透析治疗" } ] }, { "text": "尿素循环缺陷或对脂肪无不良反应的患儿应静脉给予脂肪乳剂,蛋白质不应长期限制。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "尿素循环缺陷" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "静脉" }, { "start_idx": 23, "end_idx": 26, "type": "dru", "entity": "脂肪乳剂" } ] }, { "text": "任何不明原因的呕吐、嗜睡或其他脑病表现的婴儿均应测定血氨水平。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "呕吐" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "嗜睡" }, { "start_idx": 24, "end_idx": 29, "type": "ite", "entity": "测定血氨水平" } ] }, { "text": "血氨显著升高,常见于尿素循环缺陷及有机酸血症,在新生儿期要与一过性高氨血症相鉴别,对于较大婴儿应考虑到脂肪酸氧化缺陷。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "ite", "entity": "血氨" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "尿素循环缺陷" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "有机酸血症" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "高氨血症" } ] }, { "text": "在新生儿中,血氨达正常值2倍的一过性高氨血症很常见,尤其是早产儿,这些患儿通常无症状,也无长期的神经系统损害。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "一过性高氨血症" } ] }, { "text": "新生儿一过性高氨血症多在生后24小时出现,临床上往往不被认识,无明确的家族遗传史。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "一过性高氨血症" } ] }, { "text": "典型病例可发生在早产儿,也可发生于足月儿,伴有或不伴有呼吸系统症状。", "entities": [ { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "呼吸系统" } ] }, { "text": "肝功能损害、脓毒血症、全身性单纯疱疹病毒感染和围生期窒息可导致血氨升高,但血氨不如遗传代谢病和新生儿一过性高氨血症显著,对于血氨中度升高者应检查肝功能,但是即使是严重肝坏死者,血氨一般很少超过500μmol/L。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肝功能损害" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "脓毒血症" }, { "start_idx": 11, "end_idx": 21, "type": "dis", "entity": "全身性单纯疱疹病毒感染" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "围生期窒息" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "血氨升高" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "血氨" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "一过性高氨血症" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "血氨中度升高" }, { "start_idx": 70, "end_idx": 74, "type": "pro", "entity": "检查肝功能" }, { "start_idx": 88, "end_idx": 89, "type": "bod", "entity": "血氨" } ] }, { "text": "阴离子间隙增加(≥16)可见于很多遗传代谢病及大部分由其他原因引起的代谢性酸中毒的新生儿。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "阴离子间隙增加" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "遗传代谢病" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "相反,阴离子间隙正常的代谢性酸中毒仅限于两种疾病:即腹泻和肾小管性酸中毒。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "sym", "entity": "阴离子间隙正常" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "腹泻" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "肾小管性酸中毒" } ] }, { "text": "在伴有重度代谢性酸中毒的出生缺陷婴儿中最常见的是有机酸血症,包括甲基丙二酸血症、丙酸血症和异戊酸血症。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "重度代谢性酸中毒" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "有机酸血症" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "甲基丙二酸血症" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "丙酸血症" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "异戊酸血症" } ] }, { "text": "通过测定血浆中丙酮酸水平,计算乳酸与丙酮酸的比例,能对这一类疾病进行鉴别,比例正常(≤25)提示丙酮酸脱氢酶(PDH)或葡萄糖异生缺陷,比例升高(≥25)提示丙酮酸羧化酶、呼吸链酶缺陷或线粒体肌病。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "ite", "entity": "测定血浆中丙酮酸水平" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "乳酸" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "丙酮酸" }, { "start_idx": 60, "end_idx": 66, "type": "dis", "entity": "葡萄糖异生缺陷" }, { "start_idx": 79, "end_idx": 91, "type": "dis", "entity": "丙酮酸羧化酶、呼吸链酶缺陷" }, { "start_idx": 93, "end_idx": 97, "type": "dis", "entity": "线粒体肌病" } ] }, { "text": "但是并非所有的危重代谢性疾病都伴有代谢性酸中毒或高氨血症。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "危重代谢性疾病" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "高氨血症" } ] }, { "text": "同样钼辅助因子缺乏症,临床表现与缺氧缺血性脑病相似,无代谢性酸中毒。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "钼辅助因子缺乏症" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "缺氧缺血性脑病" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "代谢性酸中毒" } ] }, { "text": "低血糖临床主要表现为反应差、阵发性发绀、震颤、眼球不正常转动、惊厥、呼吸暂定、嗜睡和拒食,有时出现多汗、苍白。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "反应差" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "阵发性发绀" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "震颤" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "眼球不正常转动" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "惊厥" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "嗜睡" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "拒食" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "多汗" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "苍白" } ] }, { "text": "近年发现,部分低血糖是因脂肪酸氧化酶遗传缺陷所造成。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "低血糖" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "脂肪酸氧化酶遗传缺陷" } ] }, { "text": "低血糖症可单独出现,也可伴有其他生化紊乱,典型的如Reye综合征,可出现高氨血症、代谢性酸中毒和转氨酶升高,有时伴肝肿大。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "低血糖症" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "Reye综合征" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "高氨血症" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "转氨酶升高" }, { "start_idx": 57, "end_idx": 59, "type": "sym", "entity": "肝肿大" } ] }, { "text": "任何有Reye综合征表现的婴儿均应考虑是否有脂肪酸氧化缺陷。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "Reye综合征" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "脂肪酸氧化缺陷" } ] }, { "text": "进行尿有机酸分析、血清肉碱谱分析以及特异性酶的分析对于脂肪酸氧化酶缺陷的确诊十分必要。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "pro", "entity": "尿有机酸分析" }, { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "血清肉碱谱分析" }, { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "特异性酶的分析" }, { "start_idx": 27, "end_idx": 34, "type": "dis", "entity": "脂肪酸氧化酶缺陷" } ] }, { "text": "肾上腺素及生长激素仅用于治疗慢性难处理的低血糖症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "生长激素" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "低血糖症" } ] }, { "text": "如半乳糖血症患儿应完全停止乳类食品,代以不含乳糖食品;亮氨酸过敏婴儿,应限制蛋白质;糖原累积症应昼夜喂奶;先天性果糖不耐受症应限制蔗糖及水果汁等。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "糖原累积症" }, { "start_idx": 53, "end_idx": 61, "type": "dis", "entity": "先天性果糖不耐受症" } ] }, { "text": "红细胞遗传代谢病,例如葡萄糖-6-磷酸脱氢酶缺乏或丙酮酸激酶缺乏不包括在内,这些酶缺乏与溶血性疾病有关。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "红细胞遗传代谢病" }, { "start_idx": 11, "end_idx": 23, "type": "dis", "entity": "葡萄糖-6-磷酸脱氢酶缺乏" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "丙酮酸激酶缺乏" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "酶缺乏" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "溶血性疾病" } ] }, { "text": "这类疾病的黄疸和肝功能受损多呈进行性,常出现在生后第一周末或第二周,伴随呕吐、腹泻、体重增长较慢和白内障形成,可出现低血糖症。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "黄疸" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肝功能受损" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "呕吐" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "腹泻" }, { "start_idx": 42, "end_idx": 47, "type": "sym", "entity": "体重增长较慢" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "白内障形成" }, { "start_idx": 58, "end_idx": 61, "type": "dis", "entity": "低血糖症" } ] }, { "text": "该病初期由于红细胞内半乳糖-1-磷酸水平的升高继发溶血,表现为间接性胆红素升高。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "红细胞" }, { "start_idx": 10, "end_idx": 17, "type": "bod", "entity": "半乳糖-1-磷酸" }, { "start_idx": 31, "end_idx": 38, "type": "bod", "entity": "间接性胆红素升高" } ] }, { "text": "若怀疑半乳糖血症,要用Benedict试剂和葡萄糖氧化酶方法检测尿液,葡萄糖氧化酶方法测定葡萄糖具有特异性,而Benedict试剂能够检测任何还原物质。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "半乳糖血症" }, { "start_idx": 22, "end_idx": 33, "type": "ite", "entity": "葡萄糖氧化酶方法检测尿液" }, { "start_idx": 35, "end_idx": 44, "type": "ite", "entity": "葡萄糖氧化酶方法测定" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "葡萄糖" } ] }, { "text": "尿液化验显示葡萄糖阴性及Benedict试验阳性,提示存在非葡萄糖性还原物质,结合临床表现,这种结果最可能是半乳糖血症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "尿液化验" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "葡萄糖阴性" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "半乳糖血症" } ] }, { "text": "若怀疑半乳糖血症,无论尿中是否发现还原物质,患儿均应立即停服含半乳糖的饮食,用豆奶或其他不含乳糖的配方奶替代。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "半乳糖血症" } ] }, { "text": "新生儿血色素沉着症是最近发现的一种遗传代谢病,它是引起先天性肝硬化的最常见原因,病程发展较快。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "新生儿血色素沉着症" }, { "start_idx": 27, "end_idx": 32, "type": "dis", "entity": "先天性肝硬化" } ] }, { "text": "除出生后即出现严重的肝功能衰竭外,该病还具有肝脏形态学改变和肝组织铁沉积的特点。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "出现严重的肝功能衰竭" } ] }, { "text": "血清铁蛋白和铁升高,而转铁蛋白降低。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "铁蛋白降低" } ] }, { "text": "引起肝功能损害的少见代谢疾病还包括尼曼匹克病C型和糖原累积病Ⅳ型。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肝功能损害" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "尼曼匹克病C型" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "糖原累积病Ⅳ型" } ] }, { "text": "尼曼匹克病C型婴儿早期表现为胆汁淤积性黄疸,可在生后数月消失,在出现退行性神经系统症状及体征疾病之前数月至数年内可表现正常。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "尼曼匹克病C型" }, { "start_idx": 14, "end_idx": 20, "type": "dis", "entity": "胆汁淤积性黄疸" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "退行性神经系统" } ] }, { "text": "糖原累积症Ⅳ型由于糖原分支酶缺陷导致糖原在肝脏沉积,出现进行性肝硬化和肝功能障碍,该型低血糖不显著。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "糖原累积症Ⅳ型" }, { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "糖原分支酶缺陷" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "糖原在肝脏沉积" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "肝功能障碍" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "低血糖不显著" } ] }, { "text": "Zellweger综合征是另一种引起新生儿黄疸和肝功能障碍的代谢疾病,通常由于伴肌张力减低和畸形被发现,该病与过氧化物酶功能障碍有关。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "dis", "entity": "Zellweger综合征" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "新生儿黄疸" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "肝功能障碍" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "代谢疾病" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "肌张力减低" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "畸形" } ] }, { "text": "患儿在无溶血时出现持续性间接胆红素升高提示Criger-Najjar综合征,该病的高胆红素血症是由于葡萄糖醛酸转移酶部分或全部缺乏造成。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "间接胆红素升高" }, { "start_idx": 21, "end_idx": 36, "type": "dis", "entity": "Criger-Najjar综合征" }, { "start_idx": 41, "end_idx": 46, "type": "dis", "entity": "高胆红素血症" }, { "start_idx": 50, "end_idx": 64, "type": "sym", "entity": "葡萄糖醛酸转移酶部分或全部缺乏" } ] }, { "text": "胸部X线检查早期即有肺浸润,呈非典型性肺炎变化。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "胸部X线" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "肺浸润" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "非典型性肺炎" } ] }, { "text": "横纹肌肉瘤占儿童实体肿瘤的15%,软组织肉瘤的50%。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "实体肿瘤" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "软组织肉瘤" } ] }, { "text": "1950年Stobbe报道对头颈部肿瘤加以放疗可提高疗效。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "头颈部肿瘤" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "放疗" } ] }, { "text": "1965年Edland介绍的大剂量放疗成为治疗不能完整切除的肿瘤的标准方法。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "放疗" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "切除" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "肿瘤" } ] }, { "text": "化疗药物的应用包括长春新碱、放线菌素D和环磷酰胺(VCR方案),效果各有差异。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "长春新碱" }, { "start_idx": 14, "end_idx": 18, "type": "dru", "entity": "放线菌素D" }, { "start_idx": 20, "end_idx": 23, "type": "dru", "entity": "环磷酰胺" } ] }, { "text": "如有扩散转移,病人可有发热、厌食、体重下降、疼痛和衰弱等情况。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "发热" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "厌食" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "体重下降" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "疼痛和衰弱" } ] }, { "text": "该肿瘤好发于两个年龄段:2~4岁和12~16岁。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" } ] }, { "text": "发病最常见的部位是头颈部(35%),泌尿生殖系统(26%),四肢(19%)。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "头颈部" }, { "start_idx": 18, "end_idx": 23, "type": "bod", "entity": "泌尿生殖系统" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "四肢" } ] }, { "text": "鼻咽癌和鼻咽部横纹肌肉瘤发病情况很相似,但鼻咽癌好发大龄儿童,颈部淋巴结转移率较高,预后较差。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "鼻咽癌" }, { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "鼻咽部横纹肌肉瘤" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "鼻咽癌" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "颈部淋巴结" } ] }, { "text": "除了原发病,横纹肌肉瘤转移到肺、骨、骨髓、淋巴结、脑、肝和乳腺,可出现相应的症状。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "肺" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "骨" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "骨髓" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "淋巴结" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "脑" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "肝" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "乳腺" } ] }, { "text": "做血常规,肝、肾功能,尿液分析,骨髓穿刺等化验检查。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "pro", "entity": "血常规" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "骨髓穿刺" } ] }, { "text": "CT、MRI可以定位肿物和其向周围侵袭的情况。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "CT" }, { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "MRI" } ] }, { "text": "儿童横纹肌肉瘤尚无特异性血浆或尿标记物。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "儿童横纹肌肉瘤" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "血浆" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "尿" } ] }, { "text": "最常见的横纹肌肉瘤组织类型是胚胎型,由梭状的横纹肌母细胞、具有嗜伊红细胞质和纵行条纹的小圆细胞组成。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "bod", "entity": "横纹肌肉瘤组织" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "横纹肌母细胞" }, { "start_idx": 31, "end_idx": 36, "type": "bod", "entity": "嗜伊红细胞质" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "小圆细胞" } ] }, { "text": "息肉样结构由以圆形和梭状细胞为中心的黏液基质以及围绕的小圆细胞组成,好发年龄小于4岁,预后最好。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "bod", "entity": "圆形和梭状细胞" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "黏液基质" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "小圆细胞" } ] }, { "text": "腺泡型是第二位常见的横纹肌肉瘤,仅次于胚胎型。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "最多见于四肢和躯干,由具有嗜伊红细胞质的大圆细胞组成。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "四肢" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "躯干" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "嗜伊红细胞质" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "大圆细胞" } ] }, { "text": "通过反转录聚合酶链反应(PCR)和荧光原位杂交技术,横纹肌肉瘤的诊断可达到分子遗传学水平,并对治疗有指导意义。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "反转录聚合酶链反应" }, { "start_idx": 12, "end_idx": 14, "type": "pro", "entity": "PCR" }, { "start_idx": 17, "end_idx": 24, "type": "pro", "entity": "荧光原位杂交技术" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "在腺泡型横纹肌肉瘤中发现2、13q35-q14基因位点中断,而在胚胎型横纹肌肉瘤中发现11号染色体上有肿瘤生长抑制基因。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "腺泡型横纹肌肉瘤" }, { "start_idx": 12, "end_idx": 24, "type": "bod", "entity": "2、13q35-q14基因" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "胚胎型横纹肌肉瘤" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "染色体" }, { "start_idx": 51, "end_idx": 58, "type": "bod", "entity": "肿瘤生长抑制基因" } ] }, { "text": "Ⅱ级中39%的患儿为除脑膜外的头颈部肿瘤。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脑膜" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "头颈部肿瘤" } ] }, { "text": "先做活检再行辅助治疗,使肿瘤缩小再做手术比将肿瘤部分切除效果好。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "活检" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肿瘤" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "手术" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "切除" } ] }, { "text": "如果没有完全切除睾丸旁肿瘤,则要取腹膜后淋巴结活检。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "睾丸旁肿瘤" }, { "start_idx": 17, "end_idx": 22, "type": "bod", "entity": "腹膜后淋巴结" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "活检" } ] }, { "text": "对横纹肌肉瘤比较有效的药物有长春新碱、放线菌素D、环磷酰胺、阿霉素、异环磷酰胺、足叶乙甙、顺铂、卡铂、美法仑、氨甲蝶呤、达卡巴嗪以及丝裂霉素C。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "横纹肌肉瘤" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "长春新碱" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "放线菌素D" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "阿霉素" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "异环磷酰胺" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "足叶乙甙" }, { "start_idx": 45, "end_idx": 46, "type": "dru", "entity": "顺铂" }, { "start_idx": 48, "end_idx": 49, "type": "dru", "entity": "卡铂" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "美法仑" }, { "start_idx": 55, "end_idx": 58, "type": "dru", "entity": "氨甲蝶呤" }, { "start_idx": 60, "end_idx": 63, "type": "dru", "entity": "达卡巴嗪" }, { "start_idx": 66, "end_idx": 70, "type": "dru", "entity": "丝裂霉素C" } ] }, { "text": "但除高分级肿瘤外,所有的病例都可采用VCR方案,如加强长春新碱和放线菌素D方案(加强VA),或VAC冲击方案。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "长春新碱" }, { "start_idx": 32, "end_idx": 36, "type": "dru", "entity": "放线菌素D" } ] }, { "text": "加强VA方案对Ⅰ级或Ⅱ级非腺泡型肿瘤有效,Ⅰ级存活率达85%,Ⅱ级达70%;Ⅲ、Ⅳ级病人大多数用冲击VAC方案,大约50%~70%完全敏感,另外20%部分敏感。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "腺泡型肿瘤" } ] }, { "text": "大剂量环磷酰胺连同泌尿系保护药物美司钠一起应用效果不错。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "泌尿系" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "美司钠" } ] }, { "text": "1979年Abramason的研究表明放疗加化疗对眼眶肿瘤是最有效的,从此不再进行眼眶内容物剜除术,其存活率可达90%。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "放疗" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "化疗" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "眼眶肿瘤" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "眼眶" }, { "start_idx": 46, "end_idx": 48, "type": "pro", "entity": "剜除术" } ] }, { "text": "化疗主要是长春新碱和放线菌素D。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "长春新碱" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "放线菌素D" } ] }, { "text": "脑膜旁的横纹肌肉瘤部位较隐蔽,不易诊断,预后较差。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "脑膜" }, { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "横纹肌肉瘤" } ] }, { "text": "强化治疗可提高生存率,其包括VCA、鞘内化疗、整个脑脊髓部的放疗等。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "pro", "entity": "VCA" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "鞘内化疗" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "脑脊髓部" }, { "start_idx": 30, "end_idx": 31, "type": "pro", "entity": "放疗" } ] }, { "text": "大约有50%的胸壁肿瘤是腺泡型横纹肌肉瘤,其他尚有骨骼外Ewing肉瘤和未分化肉瘤也可见这种组织类型。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "胸壁肿瘤" }, { "start_idx": 12, "end_idx": 19, "type": "dis", "entity": "腺泡型横纹肌肉瘤" }, { "start_idx": 25, "end_idx": 34, "type": "dis", "entity": "骨骼外Ewing肉瘤" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "未分化肉瘤" } ] }, { "text": "脊柱旁肿瘤可表现出急性神经压迫症状,需急诊手术减压。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "脊柱旁肿瘤" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "急性神经压迫症状" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "急诊手术" } ] }, { "text": "Ortega对IRSⅠ和Ⅱ的56个脊柱旁横纹肌肉瘤的患者作过总结,大部分发生在腰部和胸腰部,肿瘤直径大于5cm,为未分化型和腺泡型。", "entities": [ { "start_idx": 17, "end_idx": 24, "type": "dis", "entity": "脊柱旁横纹肌肉瘤" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "腰部" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "胸腰部" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "肿瘤" } ] }, { "text": "下肢比上肢多发,远端比近端常见,但预后差别不大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "下肢" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "上肢" } ] }, { "text": "以前膀胱或前列腺横纹肌肉瘤必须行盆腔内肿瘤及组织的根除术,随着综合治疗的开展,该术式已经不再进行。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "dis", "entity": "膀胱或前列腺横纹肌肉瘤" } ] }, { "text": "IRSⅢ提出了加强的放疗和化疗方案,采用阿霉素、顺铂、VAC,在第6周放疗,大大提高了3年存活率(83%),而且60%的膀胱得以保留(表11-11)。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "放疗" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "化疗" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "阿霉素" }, { "start_idx": 24, "end_idx": 25, "type": "dru", "entity": "顺铂" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "VAC" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "放疗" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "膀胱" } ] }, { "text": "膀胱前列腺横纹肌肉瘤如果条件允许可以行部分膀胱切除,或先行病理活检,接受加强化疗和放疗。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "膀胱前列腺横纹肌肉瘤" }, { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "膀胱切除" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "病理活检" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "化疗" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "放疗" } ] }, { "text": "从临床结果来看,早期局部肿瘤尽管可得到控制,但镜下肿瘤残留却可能持续存在,所以大多数病人最终还是做了子宫、阴道切除术。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肿瘤" }, { "start_idx": 23, "end_idx": 23, "type": "equ", "entity": "镜" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "肿瘤残留" }, { "start_idx": 50, "end_idx": 57, "type": "pro", "entity": "子宫、阴道切除术" } ] }, { "text": "确诊后要行睾丸根治切除术。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "pro", "entity": "睾丸根治切除术" } ] }, { "text": "如果以前作过阴囊活检,要行患侧阴囊切除,以免污染阴囊,同时也减少了髂腹股沟和主动脉旁淋巴结转移的机会。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "阴囊活检" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "阴囊切除" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "阴囊" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "髂腹股沟" }, { "start_idx": 39, "end_idx": 44, "type": "bod", "entity": "动脉旁淋巴结" } ] }, { "text": "经上述手术,存活率为50%;如术后化疗,或在有淋巴结转移和肿瘤残留的情况下放疗,存活率可达90%。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "手术" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "化疗" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "淋巴结" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "肿瘤残留" }, { "start_idx": 37, "end_idx": 38, "type": "pro", "entity": "放疗" } ] }, { "text": "对经CT检查无腹膜后淋巴结转移和肿瘤又完全切除者,可不需做腹膜后淋巴结活检。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "CT检查" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "腹膜" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "淋巴结" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "肿瘤" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "完全切除" }, { "start_idx": 29, "end_idx": 36, "type": "pro", "entity": "腹膜后淋巴结活检" } ] }, { "text": "但如怀疑有淋巴结浸润或原发肿瘤切除不完整,还是做系统的腹膜后淋巴结检查为好。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "淋巴结" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "原发肿瘤" }, { "start_idx": 27, "end_idx": 34, "type": "pro", "entity": "腹膜后淋巴结检查" } ] }, { "text": "Heyn等人讨论了86例IRS睾丸旁横纹肌肉瘤的长期疗效。", "entities": [ { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "睾丸旁横纹肌肉瘤" } ] }, { "text": "1/3接受过环磷酰胺治疗的病人患出血性膀胱炎。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "出血性膀胱炎" } ] }, { "text": "治疗一般采用强化疗和放疗,如近距离放疗或术中放疗。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "pro", "entity": "强化疗" }, { "start_idx": 10, "end_idx": 11, "type": "pro", "entity": "放疗" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "近距离放疗" } ] }, { "text": "偶有经二次探查术后转为对治疗敏感的,Ⅲ级病人的存活率为48%。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "二次探查术" } ] }, { "text": "会阴和肛周横纹肌肉瘤少见,通常为腺泡型,对放疗和化疗反应极差,存活率大约为20%。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "会阴和肛周横纹肌肉瘤" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "放疗" }, { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "化疗" } ] }, { "text": "女孩中约80%的中枢性性早熟患儿为特发性性早熟。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "中枢性性早熟" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "特发性性早熟" } ] }, { "text": "下丘脑错构瘤是最常引起真性性早熟的脑部病变之一,这一先天畸形由异位的神经组织所构成,含有分泌GnRH的神经元,并在功能上如同一个附加的GnRH脉冲源。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "下丘脑错构瘤" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "真性性早熟" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "脑部" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "先天畸形" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "神经组织" }, { "start_idx": 46, "end_idx": 49, "type": "bod", "entity": "GnRH" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "神经元" }, { "start_idx": 67, "end_idx": 73, "type": "bod", "entity": "GnRH脉冲源" } ] }, { "text": "其次包括星形细胞瘤、室管膜瘤、视神经胶质瘤及神经纤维瘤Ⅰ型等。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "星形细胞瘤" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "室管膜瘤" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "视神经胶质瘤" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "神经纤维瘤Ⅰ型" } ] }, { "text": "最常见的症状为呼吸道梗阻,呼气时出现喘鸣,常易反复发作急性呼吸道梗阻和呼吸道感染,但不呈现吞咽困难症状。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "呼吸道" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "呼吸道梗阻" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "呼气时出现喘鸣" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "呼吸道" }, { "start_idx": 21, "end_idx": 39, "type": "sym", "entity": "常易反复发作急性呼吸道梗阻和呼吸道感染" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "不呈现吞咽困难" } ] }, { "text": "体格检查可发现右侧肺部呼吸音减弱及肺膨胀不全的表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "体格检查" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "右侧肺部" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "肺" }, { "start_idx": 7, "end_idx": 24, "type": "sym", "entity": "右侧肺部呼吸音减弱及肺膨胀不全的表现" } ] }, { "text": "在气管隆突与食管之间,可见异常左肺动脉的块影,左肺动脉在左肺门发出的分支位置较低,支气管镜或支气管造影可显示并存的气管、支气管异常。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "气管" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "食管" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "左肺门" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "支气管镜" }, { "start_idx": 46, "end_idx": 50, "type": "pro", "entity": "支气管造影" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "气管" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "支气管" } ] }, { "text": "超声心动图可发现左肺动脉异常起源。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "超声心动图" }, { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "左肺动脉" } ] }, { "text": "手术后早期死亡率高,主要致死原因为重度气管、支气管狭窄和左肺动脉因吻合不当发生扭曲和血栓形成。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 17, "end_idx": 26, "type": "dis", "entity": "重度气管、支气管狭窄" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "左肺动脉" }, { "start_idx": 28, "end_idx": 45, "type": "sym", "entity": "左肺动脉因吻合不当发生扭曲和血栓形成" } ] }, { "text": "术后静脉滴注少量地塞米松可减少拔除气管插管后气管黏膜水肿。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "术后静脉滴注" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "地塞米松" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "气管" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "气管黏膜" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "气管黏膜水肿" } ] }, { "text": "可有剧烈的疼痛,并可引起耳鸣。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "可有剧烈的疼痛,并可引起耳鸣" } ] }, { "text": "肿瘤过大,可分期手术,必要时植皮。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "植皮" } ] }, { "text": "肿瘤主要在新生儿期扩大,少数在婴儿期长大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" } ] }, { "text": "治疗只需切除即可。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "切除" } ] }, { "text": "正常情况下主动脉瓣下无圆锥,大血管转位后其瓣下有圆锥部存在,造成与三尖瓣之间无纤维连续。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "大血管转位" }, { "start_idx": 20, "end_idx": 28, "type": "sym", "entity": "其瓣下有圆锥部存在" }, { "start_idx": 32, "end_idx": 42, "type": "sym", "entity": "与三尖瓣之间无纤维连续" } ] }, { "text": "相反,肺动脉瓣与二尖瓣毗邻,两者之间出现纤维连续。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "纤维连续" } ] }, { "text": "大动脉转位最明显的特征是主肺动脉相对位置异常。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "主肺动脉相对位置异常" } ] }, { "text": "常用的分类法是将“面对窦”硬性定为1号窦、2号窦。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "面对窦" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "1号窦" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "2号窦" } ] }, { "text": "大动脉转位除合并卵圆孔、动脉导管未闭外不伴有其他心血管畸形时称为单纯型大动脉转位,半数的大动脉转位的患儿均属于此类型。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "合并卵圆孔" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "动脉导管未闭外" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "心血管畸形" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "单纯型大动脉转位" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "大动脉转位" } ] }, { "text": "另约40%~45%的患儿伴有室间隔缺损,同时伴有室间隔缺损及明显左室流出道梗阻者约占10%。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "室间隔缺损" }, { "start_idx": 24, "end_idx": 38, "type": "sym", "entity": "室间隔缺损及明显左室流出道梗阻" } ] }, { "text": "其他合并畸形尚包括房室瓣异常、主动脉梗阻。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "房室瓣异常" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "主动脉梗阻" } ] }, { "text": "室间隔缺损是最常见的伴发畸形,大动脉转位仅伴有室间隔缺损时,室间隔缺损的位置及大小差异较大,也可为多发性缺损。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 49, "end_idx": 53, "type": "dis", "entity": "多发性缺损" } ] }, { "text": "最常见是膜部缺损及肌部缺损。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "膜部缺损" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "肌部缺损" } ] }, { "text": "左心室流出道梗阻会引起肺动脉狭窄。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "左心室流出道梗阻" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "肺动脉狭窄" } ] }, { "text": "梗阻通常为瓣下型,可由纤维膜、纤维肌性隧道或流出道室间隔向后移位引起。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "梗阻" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "瓣下型" }, { "start_idx": 11, "end_idx": 33, "type": "sym", "entity": "纤维膜、纤维肌性隧道或流出道室间隔向后移位引起" } ] }, { "text": "另一类肺血管异常属于亚临床型,是指肺血流较易进入右肺,为左心室流出道位置异常而引起的,但这种异常的肺血流分布对总的临床症状的影响意义不大。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "肺血管异常" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "肺血流" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "右肺" }, { "start_idx": 28, "end_idx": 37, "type": "sym", "entity": "左心室流出道位置异常" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "异常" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "肺血流" } ] }, { "text": "当存在大的循环间分流,如大的动脉导管未闭或大的室间隔缺损时,因交换血流量较多,则表现为以充血性心力衰竭症状为主,伴有轻度发绀。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "动脉导管未闭" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "室间隔" }, { "start_idx": 33, "end_idx": 35, "type": "ite", "entity": "血流量" }, { "start_idx": 44, "end_idx": 50, "type": "sym", "entity": "充血性心力衰竭" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "发绀" } ] }, { "text": "伴有粗大的动脉导管未闭的患儿通常在生后1周内出现症状,典型的表现为水冲脉,连续性杂音可不明显;伴有大型室间隔缺损的婴儿,通常在生后2~4周出现心力衰竭症状。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "动脉导管未闭" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "水冲脉" }, { "start_idx": 37, "end_idx": 45, "type": "sym", "entity": "连续性杂音可不明显" }, { "start_idx": 49, "end_idx": 55, "type": "dis", "entity": "大型室间隔缺损" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "心力衰竭" } ] }, { "text": "随着肺动脉高压的发展,逐渐出现第三心音、心力衰竭引起的奔马律及肺动脉第二音亢进,在心尖处可闻及因肺静脉血流增加而产生的舒张中期杂音。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "第三心音" }, { "start_idx": 20, "end_idx": 29, "type": "sym", "entity": "心力衰竭引起的奔马律" }, { "start_idx": 31, "end_idx": 38, "type": "sym", "entity": "肺动脉第二音亢进" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "心尖" }, { "start_idx": 47, "end_idx": 64, "type": "sym", "entity": "因肺静脉血流增加而产生的舒张中期杂音" } ] }, { "text": "球囊房隔造口术可作为最初的姑息性手术,该手术在第一章节中详细介绍。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "球囊房隔造口术" } ] }, { "text": "主要有两种术式:Mustard手术和Senning手术。", "entities": [ { "start_idx": 8, "end_idx": 16, "type": "pro", "entity": "Mustard手术" }, { "start_idx": 18, "end_idx": 26, "type": "pro", "entity": "Senning手术" } ] }, { "text": "一些伴有多发孔洞型室间隔缺损的患儿,可先行肺动脉环缩一期姑息手术。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "dis", "entity": "多发孔洞型室间隔缺损" }, { "start_idx": 21, "end_idx": 31, "type": "pro", "entity": "肺动脉环缩一期姑息手术" } ] }, { "text": "近来,对伴有室间隔缺损、左心室流出道梗阻并有严重低氧血症的新生儿,可先行体肺动脉分流术,然后再行Rastelli手术。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 12, "end_idx": 19, "type": "sym", "entity": "左心室流出道梗阻" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "低氧血症" }, { "start_idx": 36, "end_idx": 42, "type": "pro", "entity": "体肺动脉分流术" }, { "start_idx": 48, "end_idx": 57, "type": "pro", "entity": "Rastelli手术" } ] }, { "text": "尽管Rastelli手术的术后的成功率在1~2岁的幼儿较高,但在婴儿期仍可进行。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "pro", "entity": "Rastelli手术" } ] }, { "text": "Rastelli手术的缺点在于将来需做人工管道替换手术。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "Rastelli手术" }, { "start_idx": 19, "end_idx": 26, "type": "pro", "entity": "人工管道替换手术" } ] }, { "text": "此方案避免了将来的人工管道替换术,具有一定的发展潜力,但术后可引起肺动脉反流。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "pro", "entity": "人工管道替换术" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "肺动脉反流" } ] }, { "text": "当有严重水肿或主动脉断离伴主动脉发育不全时,可通过胸部正中切口同时完成大动脉转换术、室间隔缺损修补术及主动脉弓重建术,但手术危险性较大。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "严重水肿" }, { "start_idx": 7, "end_idx": 19, "type": "sym", "entity": "主动脉断离伴主动脉发育不全" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "胸部" }, { "start_idx": 35, "end_idx": 40, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 42, "end_idx": 49, "type": "pro", "entity": "室间隔缺损修补术" }, { "start_idx": 51, "end_idx": 57, "type": "pro", "entity": "主动脉弓重建术" } ] }, { "text": "术后最重要的并发症是晚期猝死,约占2%~8%,房性扑动可能是晚期死亡的高危因素。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "晚期猝死" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "房性扑动" } ] }, { "text": "由于常同时并发心动过速和心动过缓,抗心律失常药应用难度较大。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "心动过速" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "心动过缓" }, { "start_idx": 17, "end_idx": 22, "type": "dru", "entity": "抗心律失常药" } ] }, { "text": "尽管大多患儿并没有相应临床症状,术后有右心室功能不良已有大量的文献报道。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "右心室" } ] }, { "text": "Losay等对1095例大动脉转换术患儿的远期随访结果表明:单纯的大动脉转位患儿术后1年、10年、15年生存率均为92%。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "大动脉转换术" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "大动脉转位" } ] }, { "text": "大动脉转换术后有近80%患儿术后10年无须再次治疗。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "大动脉转换术" } ] }, { "text": "重叠综合征的治疗较治疗单纯某一种疾病困难。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "重叠综合征" } ] }, { "text": "与MCTD相比,重叠综合征的预后更差。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "MCTD" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "重叠综合征" } ] }, { "text": "最早症状为厌食,继之出现体重减轻、低热、精神不振、恶心、呕吐、顽固性便秘、嗜睡、表情淡漠,年长儿诉头痛,重者或晚期可出现高热、多尿、烦躁、脱水、昏迷、抽搐等症状。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "厌食" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "体重减轻" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "低热" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "精神不振" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "恶心" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "呕吐" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "顽固性便秘" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "嗜睡" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "表情淡漠" }, { "start_idx": 49, "end_idx": 50, "type": "sym", "entity": "头痛" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "高热" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "多尿" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "烦躁" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "脱水" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "昏迷" }, { "start_idx": 75, "end_idx": 76, "type": "sym", "entity": "抽搐" } ] }, { "text": "严重者可因高钙血症导致主动脉瓣钙化及狭窄、肾钙化及肾衰竭而致死。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "高钙血症" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "主动脉瓣钙化及狭窄" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "肾钙化" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "肾衰竭" } ] }, { "text": "治疗原则为主要防治胱氨酸结石形成并治疗其并发症。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "胱氨酸结石" } ] }, { "text": "在睡前还可服用醋唑酰胺一次,剂量为5~10mg/kg。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "醋唑酰胺" } ] }, { "text": "本药不良反应常有皮疹、发热、关节痛、骨髓抑制、类狼疮反应以及肾损害(肾病综合征)等,因此,只用于上述一般治疗不能控制以及出现严重胱氨酸结石的病例。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "皮疹" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "发热" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "关节痛" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "骨髓抑制" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "类狼疮" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "肾损害" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "严重胱氨酸结石" } ] }, { "text": "较新的药物如N-乙酰-D-青霉胺(N-acety1-D-penicillamine),毒性较低,有相同效果。", "entities": [ { "start_idx": 6, "end_idx": 15, "type": "dru", "entity": "N-乙酰-D-青霉胺" }, { "start_idx": 17, "end_idx": 40, "type": "dru", "entity": "N-acety1-D-penicillamine" } ] }, { "text": "脊柱X线平片、脊髓碘油造影及CT对诊断有一定价值,MRI检查对诊断尤有帮助。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "脊柱X线平片" }, { "start_idx": 7, "end_idx": 12, "type": "pro", "entity": "脊髓碘油造影" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "CT" }, { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "MRI检查" } ] }, { "text": "放疗需视肿瘤组织类型、恶性程度及切除范围而定,原则上相同于该肿瘤在脑内放疗指征。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "放疗" }, { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "肿瘤" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "肿瘤" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "脑内" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "放疗" } ] }, { "text": "化疗多用于恶性星形细胞瘤和室管膜瘤以及术后残留的低度星形细胞瘤。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "恶性星形细胞瘤" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "室管膜瘤" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "低度星形细胞瘤" } ] }, { "text": "血管痣亦可见于口腔黏膜或颈部、躯干或四肢皮肤。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "口腔黏膜" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "颈部" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "躯干" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "四肢皮肤" } ] }, { "text": "约90%病人有癫痫发作,多表现为血管痣对侧肢体局限性运动性发作,全身大发作少见,部分患儿也可表现为婴儿痉挛、肌阵挛性发作、失张力性发作或复杂部分性发作等。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "癫痫发作" }, { "start_idx": 16, "end_idx": 30, "type": "sym", "entity": "血管痣对侧肢体局限性运动性发作" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "婴儿痉挛" }, { "start_idx": 54, "end_idx": 59, "type": "sym", "entity": "肌阵挛性发作" }, { "start_idx": 61, "end_idx": 66, "type": "sym", "entity": "失张力性发作" }, { "start_idx": 68, "end_idx": 74, "type": "sym", "entity": "复杂部分性发作" } ] }, { "text": "癫痫可用药物控制,如应用抗癫痫药不能控制发作者可行胼胝体离断术或大脑半球切除术,有时也可行肿瘤部分切除术。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "癫痫" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "抗癫痫药" }, { "start_idx": 25, "end_idx": 30, "type": "pro", "entity": "胼胝体离断术" }, { "start_idx": 32, "end_idx": 38, "type": "pro", "entity": "大脑半球切除术" }, { "start_idx": 45, "end_idx": 51, "type": "pro", "entity": "肿瘤部分切除术" } ] }, { "text": "可有面肌无力,而其他脑神经支配的肌群可以不受累。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "面肌无力" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "脑神经" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "肌群" } ] }, { "text": "有些患者可有轻度的外周神经病变,表现为轻度感觉丧失,或者反射减弱。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "轻度的外周神经病变" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "轻度感觉丧失" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "反射减弱" } ] }, { "text": "本病的另一个特征是脑白质髓鞘形成不足。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "sym", "entity": "脑白质髓鞘形成不足" } ] }, { "text": "FCMD的主要临床特征是严重中枢神经系统缺陷,眼部异常和肌肉病变。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "FCMD" }, { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "严重中枢神经系统缺陷" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "眼部异常" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "肌肉病变" } ] }, { "text": "患者血清CK增高;肌肉活体组织检查显示非特异性表现;头颅MRI显示大脑、小脑小多脑回改变,同时可有脑积水和髓鞘发育不全。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "患者血清CK增高" }, { "start_idx": 9, "end_idx": 24, "type": "sym", "entity": "肌肉活体组织检查显示非特异性表现" }, { "start_idx": 26, "end_idx": 43, "type": "sym", "entity": "头颅MRI显示大脑、小脑小多脑回改变" }, { "start_idx": 45, "end_idx": 58, "type": "sym", "entity": "同时可有脑积水和髓鞘发育不全" } ] }, { "text": "临床表现为肌张力低下,运动发育迟缓,但最终能站立和行走,约于5岁左右出现关节挛缩。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "运动发育迟缓" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "能站立和行走" }, { "start_idx": 28, "end_idx": 39, "type": "sym", "entity": "约于5岁左右出现关节挛缩" } ] }, { "text": "严重智能发育迟缓。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "严重智能发育迟缓" } ] }, { "text": "血清CK增高;肌肉病理学检查示肌原性改变;脑组织形态学改变为小多脑回和脑回增厚。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "血清CK增高" }, { "start_idx": 7, "end_idx": 19, "type": "sym", "entity": "肌肉病理学检查示肌原性改变" }, { "start_idx": 21, "end_idx": 38, "type": "sym", "entity": "脑组织形态学改变为小多脑回和脑回增厚" } ] }, { "text": "主要表现为新生儿流涎及吐沫、生后第一次喂养时出现呛咳,甚至发生窒息。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "流涎及吐沫" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "呛咳" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "窒息" } ] }, { "text": "但以后每次喂奶或喂水后均发生同样现象,并导致吸入性肺炎。", "entities": [ { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "吸入性肺炎" } ] }, { "text": "食管下段气管瘘者伴明显腹胀。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "食管下段气管瘘" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "腹胀" } ] }, { "text": "当新生儿生后早期出现上述症状时,应疑及本病,结合X线检查所见,诊断并不困难。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "X线检查" } ] }, { "text": "90%以上的病人可因肿瘤分泌过量的脑脊液而致脑积水、颅高压;另外不同部位肿瘤可有不同表现,四脑室肿瘤多数表现为共济失调症,侧脑室肿瘤多数有癫痫、偏瘫、偏盲等。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "脑脊液" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "脑积水" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "颅高压" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "肿瘤" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "四脑室肿瘤" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "共济失调症" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "侧脑室肿瘤" }, { "start_idx": 69, "end_idx": 70, "type": "dis", "entity": "癫痫" }, { "start_idx": 72, "end_idx": 73, "type": "dis", "entity": "偏瘫" }, { "start_idx": 75, "end_idx": 76, "type": "dis", "entity": "偏盲" } ] }, { "text": "辅助检查CT及MRI检查均有助于肿瘤的诊断。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "CT" }, { "start_idx": 7, "end_idx": 11, "type": "pro", "entity": "MRI检查" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "肿瘤" } ] }, { "text": "最理想的治疗手段为手术将肿瘤全切除。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "手术" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肿瘤" } ] }, { "text": "对于残余肿瘤,如果是脉络丛乳头状瘤,则应再次手术切除;若是脉络丛乳头状癌,可考虑放疗。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "肿瘤" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "脉络丛乳头状瘤" }, { "start_idx": 22, "end_idx": 23, "type": "pro", "entity": "手术" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "脉络丛乳头状癌" }, { "start_idx": 40, "end_idx": 41, "type": "pro", "entity": "放疗" } ] }, { "text": "化疗对脉络丛乳头状癌也有效果,术前化疗尚可减少肿瘤体积及血液供应,有助于手术全切。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "化疗" }, { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "脉络丛乳头状癌" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "化疗" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "肿瘤" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "血液" }, { "start_idx": 36, "end_idx": 37, "type": "pro", "entity": "手术" } ] }, { "text": "本病预后较好,肉眼全切者,生存时间较长,并且可完全治愈。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肉眼" } ] }, { "text": "在HIE病因中产前和产时窒息各占50%和40%,其他原因约占10%。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "HIE" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "窒息" } ] }, { "text": "若缺氧缺血发生在出生前几周或几个月时,患儿在出生时可无窒息,也无神经系统症状,但在数天或数周后出现亚急性或慢性脑病的表现,临床上较难与先天性脑畸形或宫内病毒感染相区别。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "缺氧缺血" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "窒息" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "神经系统" }, { "start_idx": 49, "end_idx": 56, "type": "dis", "entity": "亚急性或慢性脑病" }, { "start_idx": 67, "end_idx": 72, "type": "dis", "entity": "先天性脑畸形" }, { "start_idx": 74, "end_idx": 79, "type": "dis", "entity": "宫内病毒感染" } ] }, { "text": "在HIE急性期,脑水肿比较明显,可能会掩盖脑细胞损伤,并且病情还在变化之中,所以早期影像学检查不能反映预后,需在2~4周后复查。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "HIE" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "脑水肿" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "脑细胞" } ] }, { "text": "本病发展迅速,易并发喉梗阻,应及时治疗。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "喉梗阻" } ] }, { "text": "对Ⅱ度或Ⅱ度以上喉梗阻者应早期应用肾上腺皮质激素,用量要足够大。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "dis", "entity": "Ⅱ度或Ⅱ度以上喉梗阻" }, { "start_idx": 17, "end_idx": 23, "type": "dru", "entity": "肾上腺皮质激素" } ] }, { "text": "及时选用抗生素控制感染,常用青霉素及头孢霉素。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "抗生素" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "感染" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "青霉素" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "头孢霉素" } ] }, { "text": "经上述治疗后,呼吸困难仍不缓解,缺氧继续加重者,应气管切开。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "缺氧继续加重" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "气管" } ] }, { "text": "Ⅳ度喉梗阻患儿则应立即进行气管切开术抢救。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "Ⅳ度喉梗阻" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "气管切开术" } ] }, { "text": "近年来由于抗生素的广泛应用,本病发生率已明显降低。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "抗生素" } ] }, { "text": "婴儿可仅表现为呼吸症状的恶化,病程长者可伴贫血、消瘦、杵状指(趾)等。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "呼吸症状的恶化" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "贫血" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "消瘦" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "杵状指(趾)" } ] }, { "text": "积脓量不多时,可在肺底部一定范围听到湿啰音,或在脓液面上方听到管状呼吸音。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "积脓量不多" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "脓液面" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "管状呼吸音" } ] }, { "text": "胸部X线检查与渗出性胸膜炎相似。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "胸部X线检查" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "渗出性胸膜炎" } ] }, { "text": "胸水检查对诊断及鉴别诊断至关重要。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "胸水检查" } ] }, { "text": "手术目的包括病理活检、根治性肿瘤完全切除术、减负性不完全大部分切除术和解除或减轻症状的姑息性手术。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "病理活检" }, { "start_idx": 11, "end_idx": 20, "type": "pro", "entity": "根治性肿瘤完全切除术" }, { "start_idx": 22, "end_idx": 33, "type": "pro", "entity": "减负性不完全大部分切除术" }, { "start_idx": 43, "end_idx": 47, "type": "pro", "entity": "姑息性手术" } ] }, { "text": "3.儿童处于生长发育期,因此更可能由于化疗药物及放疗对生长发育中的机体器官的损伤造成生长发育障碍及远期的脏器功能不良。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "pro", "entity": "放疗" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "器官" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "生长发育障碍" }, { "start_idx": 52, "end_idx": 57, "type": "dis", "entity": "脏器功能不良" } ] }, { "text": "出生时身长正常,5个月起出现生长减慢,1~2岁明显。", "entities": [ { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "出生时身长正常,5个月起出现生长减慢,1~2岁明显" } ] }, { "text": "伴有垂体其他促激素不足者,多为促性腺激素缺乏,表现为青春发育延缓,男孩小阴茎、小睾丸,女孩乳房不发育,原发闭经;若伴有ACTH缺乏,则常有皮肤色素沉着和严重的低血糖表现;伴有促甲状腺激素不足,则表现为甲状腺功能低下。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "促激素" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "促性腺激素缺乏" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "阴茎" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "睾丸" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "乳房" }, { "start_idx": 26, "end_idx": 54, "type": "sym", "entity": "青春发育延缓,男孩小阴茎、小睾丸,女孩乳房不发育,原发闭经" }, { "start_idx": 59, "end_idx": 64, "type": "sym", "entity": "ACTH缺乏" }, { "start_idx": 67, "end_idx": 83, "type": "sym", "entity": "常有皮肤色素沉着和严重的低血糖表现" }, { "start_idx": 87, "end_idx": 94, "type": "sym", "entity": "促甲状腺激素不足" }, { "start_idx": 100, "end_idx": 102, "type": "bod", "entity": "甲状腺" }, { "start_idx": 100, "end_idx": 106, "type": "sym", "entity": "甲状腺功能低下" } ] }, { "text": "继发性GHD可发生于任何年龄,并伴有原发疾病的相应症状。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "继发性GHD" } ] }, { "text": "当病变是一个进展性的肿瘤时,可有头痛、呕吐、视力障碍、行为异常、癫痫发作、多尿及生长障碍等表现。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "肿瘤" }, { "start_idx": 16, "end_idx": 46, "type": "sym", "entity": "头痛、呕吐、视力障碍、行为异常、癫痫发作、多尿及生长障碍等表现" } ] }, { "text": "生长缓慢出现在神经系统症状体征出现前,尤其多见于颅咽管瘤。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "生长缓慢" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "神经系统" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "颅咽管瘤" } ] }, { "text": "颅咽管瘤的儿童常见有视野缺损、视神经萎缩、视乳头水肿及中枢神经瘫痪。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅咽管瘤" }, { "start_idx": 10, "end_idx": 32, "type": "sym", "entity": "视野缺损、视神经萎缩、视乳头水肿及中枢神经瘫痪" } ] }, { "text": "如病情严重、耐药情况不明,宜首选第三代头孢霉素,如头孢曲松、头孢噻肟等,如为敏感株感染则可用氨苄西林,或SMZ-TMP治疗。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "dru", "entity": "第三代头孢霉素" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "头孢曲松" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "头孢噻肟" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "敏感株感染" }, { "start_idx": 46, "end_idx": 49, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 52, "end_idx": 58, "type": "dru", "entity": "SMZ-TMP" } ] }, { "text": "绝大多数情况下,峡部很庞大,由实质性组织组成,有其固有的血供。", "entities": [ { "start_idx": 0, "end_idx": 29, "type": "sym", "entity": "绝大多数情况下,峡部很庞大,由实质性组织组成,有其固有的血供" } ] }, { "text": "偶然也发现峡部很细、很薄,由纤维组织组成。", "entities": [ { "start_idx": 0, "end_idx": 19, "type": "sym", "entity": "偶然也发现峡部很细、很薄,由纤维组织组成" } ] }, { "text": "马蹄肾血供来源变异很大,可有多支肾动脉供应的情况发生。", "entities": [ { "start_idx": 0, "end_idx": 25, "type": "sym", "entity": "马蹄肾血供来源变异很大,可有多支肾动脉供应的情况发生" } ] }, { "text": "10%的患儿有输尿管重复畸形,半数患儿有膀胱输尿管反流。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "10%的患儿有输尿管重复畸形" }, { "start_idx": 15, "end_idx": 26, "type": "sym", "entity": "半数患儿有膀胱输尿管反流" } ] }, { "text": "当马蹄肾发生肾盂积水、尿路感染以及结石时,可以出现症状,其中最常见的是腹部隐痛,可放射至背部,也可出现Rovsing征,即在脊椎过度伸展时出现腹痛、恶心及呕吐。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "肾盂积水" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "尿路感染" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "结石" }, { "start_idx": 35, "end_idx": 45, "type": "sym", "entity": "腹部隐痛,可放射至背部" }, { "start_idx": 47, "end_idx": 78, "type": "sym", "entity": "也可出现Rovsing征,即在脊椎过度伸展时出现腹痛、恶心及呕吐" } ] }, { "text": "5%~10%的患儿可触及腹部肿块。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "sym", "entity": "患儿可触及腹部肿块" } ] }, { "text": "由于输尿管高位起始于肾盂,可以导致UPJO(肾盂输尿管连接部梗阻);峡部以及异常的肾动脉均可压迫输尿管,也可引起肾盂积水。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "输尿管" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肾盂" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "UPJO" }, { "start_idx": 22, "end_idx": 31, "type": "dis", "entity": "肾盂输尿管连接部梗阻" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "输尿管" }, { "start_idx": 56, "end_idx": 59, "type": "dis", "entity": "肾盂积水" } ] }, { "text": "马蹄肾没有明确症状,诊断常在B超或排泄性尿路造影(IVU)时才能明确,影像学上提示马蹄肾的表现包括:肾脏位置较低,靠近椎体;两肾的纵轴由外上行向内下在肾的下方相交;肾盏朝向背侧,位于肾盂后方;高位输尿管开口,上段输尿管被推向前方腹侧。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "B超" }, { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "排泄性尿路造影" }, { "start_idx": 25, "end_idx": 27, "type": "pro", "entity": "IVU" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "马蹄肾" }, { "start_idx": 50, "end_idx": 60, "type": "sym", "entity": "肾脏位置较低,靠近椎体" }, { "start_idx": 62, "end_idx": 80, "type": "sym", "entity": "两肾的纵轴由外上行向内下在肾的下方相交" }, { "start_idx": 82, "end_idx": 94, "type": "sym", "entity": "肾盏朝向背侧,位于肾盂后方" }, { "start_idx": 96, "end_idx": 115, "type": "sym", "entity": "高位输尿管开口,上段输尿管被推向前方腹侧" } ] }, { "text": "支气管哮喘是由多种细胞,包括炎性细胞(嗜酸性粒细胞、肥大细胞、T淋巴细胞、中性粒细胞等)、气道结构细胞(气道平滑肌细胞和上皮细胞等)和细胞组分参与的气道慢性炎症性疾病。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "支气管哮喘" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "细胞" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "炎性细胞" }, { "start_idx": 19, "end_idx": 24, "type": "bod", "entity": "嗜酸性粒细胞" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "肥大细胞" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "T淋巴细胞" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 45, "end_idx": 50, "type": "bod", "entity": "气道结构细胞" }, { "start_idx": 52, "end_idx": 58, "type": "bod", "entity": "气道平滑肌细胞" }, { "start_idx": 60, "end_idx": 63, "type": "bod", "entity": "上皮细胞" }, { "start_idx": 74, "end_idx": 82, "type": "dis", "entity": "气道慢性炎症性疾病" } ] }, { "text": "这种慢性炎症导致易感个体气道反应性增高,当接触物理、化学、生物等诱发因素时,发生广泛多变的可逆性气流受限,从而引起反复发作的、可逆的喘息、咳嗽、气促、胸闷等症状。", "entities": [ { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "喘息" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "咳嗽" }, { "start_idx": 72, "end_idx": 73, "type": "sym", "entity": "气促" }, { "start_idx": 75, "end_idx": 76, "type": "sym", "entity": "胸闷" } ] }, { "text": "解剖上往往不见输尿管的狭窄存在,但组织学研究显示该段输尿管存在肌肉发育、排列、走向以及纤维类型、分布等的异常;如病变段输尿管的胶原纤维增加、肌肉相对缺乏以及环形肌肉增生等。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "输尿管" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "输尿管" }, { "start_idx": 18, "end_idx": 53, "type": "sym", "entity": "织学研究显示该段输尿管存在肌肉发育、排列、走向以及纤维类型、分布等的异常" }, { "start_idx": 56, "end_idx": 68, "type": "sym", "entity": "病变段输尿管的胶原纤维增加" }, { "start_idx": 70, "end_idx": 84, "type": "sym", "entity": "肌肉相对缺乏以及环形肌肉增生等" } ] }, { "text": "由于尿液的积滞,常致肾盂、肾盏的扩张和积水。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "尿液" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肾盂" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肾盏" } ] }, { "text": "先天性输尿管远端狭窄以及输尿管瓣膜也是原发性梗阻性巨输尿管原因之一。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "先天性输尿管远端狭窄" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "输尿管瓣膜" }, { "start_idx": 19, "end_idx": 28, "type": "dis", "entity": "原发性梗阻性巨输尿管" } ] }, { "text": "它们导致膀胱内压增高、膀胱壁或输尿管远端纤维化,形成膀胱输尿管连接部的梗阻。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "膀胱内压增高" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "膀胱壁" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "输尿管远端" }, { "start_idx": 26, "end_idx": 30, "type": "bod", "entity": "膀胱输尿管" }, { "start_idx": 11, "end_idx": 36, "type": "sym", "entity": "膀胱壁或输尿管远端纤维化,形成膀胱输尿管连接部的梗阻" } ] }, { "text": "有些患儿可仅表现为营养不良或生长发育迟缓等。", "entities": [ { "start_idx": 9, "end_idx": 19, "type": "sym", "entity": "营养不良或生长发育迟缓" } ] }, { "text": "随着超声检查的广泛应用,胎儿巨输尿管的检出率逐渐升高,最高的报道占了超声发现的泌尿系畸形的20%左右。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "超声检查" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "胎儿巨输尿管" }, { "start_idx": 34, "end_idx": 35, "type": "pro", "entity": "超声" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "泌尿系畸形" } ] }, { "text": "神经源性膀胱经药物治疗后反流也可缓解。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "如新生儿输尿管巨大扩张、肾功能很差,或有反复的尿路感染,可选择做输尿管造瘘,待患儿长大后再做输尿管再植。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "输尿管" }, { "start_idx": 1, "end_idx": 26, "type": "sym", "entity": "新生儿输尿管巨大扩张、肾功能很差,或有反复的尿路感染" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "输尿管造瘘" }, { "start_idx": 46, "end_idx": 50, "type": "pro", "entity": "输尿管再植" } ] }, { "text": "吸入后可有短暂的无症状期,但90%以上患儿在吸入后1小时内出现症状,主要表现咳嗽、气急、发热,重者发绀和休克。", "entities": [ { "start_idx": 21, "end_idx": 32, "type": "sym", "entity": "在吸入后1小时内出现症状" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "咳嗽" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "气急" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "发热" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "重者发绀" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "休克" } ] }, { "text": "X线胸片多为两侧广泛肺泡性或网状浸润阴影,部分可伴局灶性实变。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "X线胸片" }, { "start_idx": 6, "end_idx": 19, "type": "sym", "entity": "两侧广泛肺泡性或网状浸润阴影" }, { "start_idx": 21, "end_idx": 29, "type": "sym", "entity": "部分可伴局灶性实变" } ] }, { "text": "应立即清理呼吸道,给氧。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "给氧" } ] }, { "text": "严重者气管内吸引和机械通气。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "机械通气" } ] }, { "text": "既往健康者常继发口腔寄生菌(尤其是厌氧菌)感染,可选用克林霉素或青霉素治疗;住院儿童则易发生大肠埃希菌、肺炎克雷伯杆菌等革兰阴性菌感染,需加用第三代头孢菌素或复合β-内酰胺类等抗生素。", "entities": [ { "start_idx": 8, "end_idx": 22, "type": "dis", "entity": "口腔寄生菌(尤其是厌氧菌)感染" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "克林霉素" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "青霉素" }, { "start_idx": 46, "end_idx": 50, "type": "mic", "entity": "大肠埃希菌" }, { "start_idx": 52, "end_idx": 58, "type": "mic", "entity": "肺炎克雷伯杆菌" }, { "start_idx": 60, "end_idx": 66, "type": "dis", "entity": "革兰阴性菌感染" }, { "start_idx": 71, "end_idx": 77, "type": "dru", "entity": "第三代头孢菌素" }, { "start_idx": 79, "end_idx": 86, "type": "dru", "entity": "复合β-内酰胺类" } ] }, { "text": "根据WHO对1990—1994年期间全球15岁以下儿童1型糖尿病调查作的回顾总结,发病率最高的地区为芬兰和意大利,这2个地区的发病率为36/10万。", "entities": [ { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "儿童1型糖尿病" } ] }, { "text": "1型糖尿病起病多数较急骤,几天内可突然表现明显多饮、多尿,每天饮水量和尿量可达3~5L,易饿多食,但体重下降,称为“三多一少”。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "1型糖尿病" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "多饮" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "多尿" }, { "start_idx": 29, "end_idx": 42, "type": "sym", "entity": "每天饮水量和尿量可达3~5L" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "易饿多食" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "体重下降" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "三多一少" } ] }, { "text": "部分患儿因感染、饮食不当或情绪波动诱发而起病。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "感染" } ] }, { "text": "婴幼儿多饮多尿不易发现,有相当多的病人常以急性酮症酸中毒为首发症状,表现为胃纳减退、恶心、呕吐、腹痛、关节肌肉疼痛、呼吸深快、呼气中带有酮味,神志萎靡、嗜睡、反应迟钝,严重者可出现昏迷。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "多饮多尿" }, { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "急性酮症酸中毒" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "胃纳减退" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "恶心" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "呕吐" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "腹痛" }, { "start_idx": 51, "end_idx": 56, "type": "sym", "entity": "关节肌肉疼痛" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "呼吸深快" }, { "start_idx": 63, "end_idx": 69, "type": "sym", "entity": "呼气中带有酮味" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "神志萎靡" }, { "start_idx": 76, "end_idx": 77, "type": "sym", "entity": "嗜睡" }, { "start_idx": 79, "end_idx": 82, "type": "sym", "entity": "反应迟钝" }, { "start_idx": 90, "end_idx": 91, "type": "sym", "entity": "昏迷" } ] }, { "text": "学龄儿童亦有因夜间遗尿而就诊者。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "夜间遗尿" } ] }, { "text": "在病史较长的年长儿中,消瘦、精神不振及倦怠乏力等体质显著下降颇为突出。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "消瘦" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "精神不振" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "倦怠乏力" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "体质显著下降" } ] }, { "text": "2.糖化血红蛋白(HbA1c)是血中葡萄糖与血红蛋白非酶性结合而产生,其寿命周期与红细胞相同,反映过去3个月的血糖平均水平。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "bod", "entity": "糖化血红蛋白" }, { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "HbA1c" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "葡萄糖" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "红细胞" }, { "start_idx": 55, "end_idx": 56, "type": "ite", "entity": "血糖" } ] }, { "text": "肥胖、高胰岛素血症(黑棘皮病)及家族2型糖尿病史是导致儿童发生该型糖尿病的高危因素。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肥胖" }, { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "高胰岛素血症" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "黑棘皮病" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "家族2型糖尿病" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "糖尿病" } ] }, { "text": "临床特征是发病年龄小于25岁,有三代以上家族糖尿病史,起病后几年内不需要胰岛素治疗。", "entities": [ { "start_idx": 36, "end_idx": 38, "type": "dru", "entity": "胰岛素" } ] }, { "text": "(4)胰岛素的注射方式有较多选择,如注射针、注射笔、无针喷射装置及胰岛素泵等,目前已经有较多青少年1型糖尿病患者采用胰岛素泵持续皮下输注胰岛素(CSII)疗法,用此法与传统的胰岛素注射方案比较,可以增加患者吃主餐和点心的时间灵活性,可以改善代谢,减少严重低血糖的危险。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "胰岛素" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "注射" }, { "start_idx": 18, "end_idx": 20, "type": "equ", "entity": "注射针" }, { "start_idx": 22, "end_idx": 24, "type": "equ", "entity": "注射笔" }, { "start_idx": 26, "end_idx": 31, "type": "equ", "entity": "无针喷射装置" }, { "start_idx": 33, "end_idx": 36, "type": "equ", "entity": "胰岛素泵" }, { "start_idx": 46, "end_idx": 53, "type": "dis", "entity": "青少年1型糖尿病" }, { "start_idx": 58, "end_idx": 61, "type": "equ", "entity": "胰岛素泵" }, { "start_idx": 72, "end_idx": 75, "type": "pro", "entity": "CSII" }, { "start_idx": 87, "end_idx": 91, "type": "pro", "entity": "胰岛素注射" }, { "start_idx": 125, "end_idx": 129, "type": "sym", "entity": "严重低血糖" } ] }, { "text": "(5)胰岛素治疗的并发症有低血糖,应及时加餐或饮含糖饮料。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "胰岛素治疗" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "低血糖" } ] }, { "text": "立克次体是革兰阴性胞内寄生菌,生物学特征介于病毒和细菌之间,在动物宿主包括啮齿动物如鼠类和家畜等贮存宿主体内繁殖,传播途径主要是由节肢动物蜱、螨、蚤、虱等媒介的叮咬或暴露,以及气溶胶方式进行传播。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "mic", "entity": "立克次体" }, { "start_idx": 5, "end_idx": 13, "type": "mic", "entity": "革兰阴性胞内寄生菌" }, { "start_idx": 22, "end_idx": 23, "type": "mic", "entity": "病毒" } ] }, { "text": "传统的立克次体病包括斑疹伤寒、恙虫病、斑点热、战壕热、Q热,其主要致病机制是病原体侵染血管内皮细胞导致小血管炎和血管周围炎。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "立克次体病" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "斑疹伤寒" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "恙虫病" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "斑点热" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "战壕热" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "Q热" }, { "start_idx": 43, "end_idx": 48, "type": "bod", "entity": "血管内皮细胞" }, { "start_idx": 38, "end_idx": 48, "type": "sym", "entity": "病原体侵染血管内皮细胞" }, { "start_idx": 51, "end_idx": 54, "type": "dis", "entity": "小血管炎" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "血管周围炎" } ] }, { "text": "其临床特征是高热和出血性皮疹。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "高热" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "出血性皮疹" } ] }, { "text": "体温度高达39℃以上,多为稽留热,少数为弛张热。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "体温度高达39℃以上" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "稽留热" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "弛张热" } ] }, { "text": "此外,一般症状包括头痛、腰背痛、食欲缺乏、呕吐、腹泻、腹胀、黄疸。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "头痛" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "腰背痛" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "呕吐" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "腹胀" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "黄疸" } ] }, { "text": "此外可见眼部症状,以球结膜充血为主要特点。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "眼部" }, { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "眼部症状" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "球结膜" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "球结膜充血" } ] }, { "text": "重症可加用肾上腺皮质激素。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dru", "entity": "肾上腺皮质激素" } ] }, { "text": "虽有代谢性酸中毒,但与dRTA不同的是尿可为酸性,与pRTA不同的是尿排出较低。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "代谢性酸中毒" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "dRTA" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "尿" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "尿可为酸性" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "pRTA" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "尿" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "尿排出较低" } ] }, { "text": "患儿常有面色苍黄、水肿、皮肤干粗、毛发稀疏。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "面色苍黄" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "水肿" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "皮肤干粗" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "毛发稀疏" } ] }, { "text": "慢性钩虫感染可有低清蛋白血症。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "慢性钩虫感染" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "低清蛋白血症" } ] }, { "text": "一般采用粪便直接涂片法,可于镜下发现虫卵。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "pro", "entity": "粪便直接涂片法" }, { "start_idx": 18, "end_idx": 19, "type": "mic", "entity": "虫卵" } ] }, { "text": "或采用饱和盐水漂浮法、四氯乙烯圆管法和过滤圆管法。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "pro", "entity": "饱和盐水漂浮法" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "四氯乙烯圆管法" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "过滤圆管法" } ] }, { "text": "贫血者应补充铁剂,严重者应考虑少量输血,每次25~50ml,或5~10ml/kg。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "贫血" }, { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "补充铁剂" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "少量输血" } ] }, { "text": "目前尚无疫苗用于预防钩虫,但借助重组DNA技术,通过基因克隆和抗原合成,研制疫苗预防钩虫病是有可能的。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "钩虫" }, { "start_idx": 16, "end_idx": 22, "type": "pro", "entity": "重组DNA技术" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "基因克隆" }, { "start_idx": 31, "end_idx": 34, "type": "pro", "entity": "抗原合成" }, { "start_idx": 38, "end_idx": 39, "type": "dru", "entity": "疫苗" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "钩虫病" } ] }, { "text": "血管壁结构或功能的异常、血小板量和质的异常、凝血功能异常是出血倾向的基本原因。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血管壁" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "血小板量和质的异常" } ] }, { "text": "临床以出血倾向为主要表现的疾病称作出血性疾病,约占血液系统疾病的30%,要对这些疾病作出正确的诊断和鉴别诊断,必须对正常的止血机制有一个全面的了解,现简述于下。", "entities": [ { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "出血性疾病" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "血液系统疾病" } ] }, { "text": "爱-唐(Ehlers-Danlos)综合征属常染色体显性遗传,皮肤弹性及胶原纤维发生数量改变、退行性变和断裂,导致皮肤弹性过强,血管脆性高,而易致出血。", "entities": [ { "start_idx": 0, "end_idx": 20, "type": "dis", "entity": "爱-唐(Ehlers-Danlos)综合征" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "皮肤" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "皮肤" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "血管" } ] }, { "text": "继发于感染等疾病的播散性血管内凝血(DIC)是由于凝血因子、血小板的大量消耗或纤溶亢进,导致严重的出血;使用抗凝药物引起出血者偶或见之。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "感染" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "播散性血管内凝血" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "DIC" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "凝血因子" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "血小板" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "抗凝药物" } ] }, { "text": "本病的初始病原体为病毒(主要为副流感病毒1、2、3型,呼吸道合胞病毒及腺病毒次之),在病毒感染的基础上易继发细菌感染。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "mic", "entity": "病毒" }, { "start_idx": 15, "end_idx": 25, "type": "mic", "entity": "副流感病毒1、2、3型" }, { "start_idx": 27, "end_idx": 33, "type": "mic", "entity": "呼吸道合胞病毒" }, { "start_idx": 35, "end_idx": 37, "type": "mic", "entity": "腺病毒" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "病毒感染" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "细菌感染" } ] }, { "text": "常先有上呼吸道感染症状,1~2天后出现吸气性喉鸣,声音嘶哑和犬吠样咳嗽及鼻扇、三凹征等呼吸困难症状,随炎症向下蔓延,可出现呼气性呼吸困难。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "上呼吸道感染" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "吸气性喉鸣" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "犬吠样咳嗽" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "鼻" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "鼻扇" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "炎症" }, { "start_idx": 61, "end_idx": 67, "type": "dis", "entity": "呼气性呼吸困难" } ] }, { "text": "听诊可闻及喘鸣音和湿啰音,重者因支气管管腔阻塞导致呼吸音减弱或消失。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "听诊" }, { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "喘鸣音和湿啰音" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "支气管管腔" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "支气管管腔阻塞" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "呼吸音减弱或消失" } ] }, { "text": "血气分析可出现低氧血症和高碳酸血症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "血气分析" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "低氧血症" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "高碳酸血症" } ] }, { "text": "必要时行直接喉镜和支气管镜检查,镜下可见喉、气管和支气管黏膜高度红肿,声门及声门下狭窄,气管和支气管内有稠厚分泌物或痂皮阻塞。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "pro", "entity": "喉镜和支气管镜检查" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "喉" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "气管" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "支气管黏膜" }, { "start_idx": 20, "end_idx": 33, "type": "sym", "entity": "喉、气管和支气管黏膜高度红肿" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "声门" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "声门" }, { "start_idx": 35, "end_idx": 42, "type": "sym", "entity": "声门及声门下狭窄" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "气管" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "支气管" }, { "start_idx": 44, "end_idx": 61, "type": "sym", "entity": "气管和支气管内有稠厚分泌物或痂皮阻塞" } ] }, { "text": "值得注意的是,严重缺氧患儿咽喉部检查,甚至用压舌板检查咽部可引起心跳呼吸骤停。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "严重缺氧" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "咽喉部检查" }, { "start_idx": 22, "end_idx": 24, "type": "equ", "entity": "压舌板" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "咽部" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "心跳呼吸骤停" } ] }, { "text": "本病应与细菌性气管炎相鉴别。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "细菌性气管炎" } ] }, { "text": "后者主要病原是金黄色葡萄球菌,亦可由副流感病毒、卡他莫拉菌、不定型流感嗜血杆菌、厌氧菌引起。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 18, "end_idx": 22, "type": "mic", "entity": "副流感病毒" }, { "start_idx": 24, "end_idx": 28, "type": "mic", "entity": "卡他莫拉菌" }, { "start_idx": 33, "end_idx": 38, "type": "mic", "entity": "流感嗜血杆菌" }, { "start_idx": 40, "end_idx": 42, "type": "mic", "entity": "厌氧菌" } ] }, { "text": "症状与喉、气管、支气管炎相似,但中毒症状重,伴高热,一般治疗无效。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "dis", "entity": "喉、气管、支气管炎" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "中毒" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "高热" } ] }, { "text": "气道分泌物呈脓性,周围血白细胞计数明显升高,出现杆状核细胞。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "气道分泌物" }, { "start_idx": 0, "end_idx": 7, "type": "sym", "entity": "气道分泌物呈脓性" }, { "start_idx": 9, "end_idx": 16, "type": "ite", "entity": "周围血白细胞计数" }, { "start_idx": 9, "end_idx": 20, "type": "sym", "entity": "周围血白细胞计数明显升高" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "杆状核细胞" } ] }, { "text": "常需气管切开或插管。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "气管切开" }, { "start_idx": 7, "end_idx": 8, "type": "pro", "entity": "插管" } ] }, { "text": "GSD依其所缺陷的酶12型,多数属分解代谢上的缺陷,使糖原异常堆积。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "酶12型" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "分解代谢上的缺陷" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "糖原异常堆积" } ] }, { "text": "一些患者尽管血糖很低,但无明显的低血糖症状,往往因肝大就诊,经生化检查才发现低血糖。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "血糖很低" }, { "start_idx": 12, "end_idx": 20, "type": "sym", "entity": "无明显的低血糖症状" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "肝" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "肝大" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "低血糖" } ] }, { "text": "饥饿时做肾上腺素或胰高血糖素试验无反应。", "entities": [ { "start_idx": 4, "end_idx": 15, "type": "pro", "entity": "肾上腺素或胰高血糖素试验" }, { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "饥饿时做肾上腺素或胰高血糖素试验无反应" } ] }, { "text": "GSDⅡ型(Pompe病)酸性α-糖苷酶缺陷,全身所有器官都有糖原累积。", "entities": [ { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "酸性α-糖苷酶缺陷" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "糖原" } ] }, { "text": "临床症状和体征:Ⅱ型出生时正常,6个月内出现心脏扩大,随着组织糖原储积增多,心脏重度扩大,可出现心力衰竭,心电图显示P-R间期缩短,T波倒置和巨大QRS波。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "组织糖原" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "组织糖原储积增多" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "心脏" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "心脏重度扩大" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "出现心力衰竭" }, { "start_idx": 53, "end_idx": 55, "type": "pro", "entity": "心电图" } ] }, { "text": "胰高血糖素试验血糖反应正常,尿儿茶酚胺排量正常。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "胰高血糖素试验" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "胰高血糖素试验血糖反应正常" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "尿儿茶酚胺排量正常" } ] }, { "text": "临床症状和体征:中度至重度肝肿大,可无或程度不等的肌张力低下,心脏增大,ECG异常少见,有低血糖。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "中度至重度肝肿大" }, { "start_idx": 17, "end_idx": 29, "type": "sym", "entity": "可无或程度不等的肌张力低下" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "心脏" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "心脏增大" }, { "start_idx": 36, "end_idx": 42, "type": "sym", "entity": "ECG异常少见" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "有低血糖" } ] }, { "text": "GSDⅣ型(Andersen病)分支酶缺陷。", "entities": [ { "start_idx": 0, "end_idx": 20, "type": "dis", "entity": "GSDⅣ型(Andersen病)分支酶缺陷" } ] }, { "text": "临床症状和体征:婴儿期出现肝脾肿大,腹水、肝硬化、肝功能衰竭,生长障碍,智能发育正常,常在儿童期死亡。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "肝脾" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "婴儿期出现肝脾肿大" }, { "start_idx": 18, "end_idx": 29, "type": "sym", "entity": "腹水、肝硬化、肝功能衰竭" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "生长障碍" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "智能发育正常" } ] }, { "text": "GSDⅤ型(McArdle综合征)肌磷酸化酶缺陷。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "GSDⅤ型" }, { "start_idx": 6, "end_idx": 15, "type": "dis", "entity": "McArdle综合征" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "肌磷酸化酶缺陷" } ] }, { "text": "受累组织为骨骼肌,而肝脏和平滑肌正常。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "肝脏" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "平滑肌" } ] }, { "text": "临床症状和体征:运动后骨骼肌无力、肌痛。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 8, "end_idx": 18, "type": "sym", "entity": "运动后骨骼肌无力、肌痛" } ] }, { "text": "缺血运动试验后血乳酸不升高。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "血乳酸" }, { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "缺血运动试验后血乳酸不升高" } ] }, { "text": "GSDⅥ型(Heres病)肝磷酸化酶缺陷,病程较良性,儿童早期开始出现肝大和生长迟缓。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "GSDⅥ型" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "Heres病" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "肝磷酸化酶缺陷" }, { "start_idx": 27, "end_idx": 41, "type": "sym", "entity": "儿童早期开始出现肝大和生长迟缓" } ] }, { "text": "低血糖、高脂血症和酮症较少出现,而且表现轻微。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "低血糖" }, { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "高脂血症" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "酮症" }, { "start_idx": 0, "end_idx": 14, "type": "sym", "entity": "低血糖、高脂血症和酮症较少出现" } ] }, { "text": "GSDⅦ型(Tarui病)肌磷酸果糖激酶缺陷。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "GSDⅦ型" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "Tarui病" }, { "start_idx": 13, "end_idx": 21, "type": "dis", "entity": "肌磷酸果糖激酶缺陷" } ] }, { "text": "受累组织为骨骼肌和红细胞。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "红细胞" } ] }, { "text": "临床症状和体征:运动后骨骼肌疼痛无力,缺血运动试验后血乳酸不升高,智能发育正常,预后好。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "骨骼肌" }, { "start_idx": 8, "end_idx": 17, "type": "sym", "entity": "运动后骨骼肌疼痛无力" }, { "start_idx": 19, "end_idx": 31, "type": "sym", "entity": "缺血运动试验后血乳酸不升高" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "智能发育正常" } ] }, { "text": "受累组织发现大脑糖原增加,电镜显示在大脑神经突触和轴突有糖原堆积形成的α颗粒。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "大脑糖原" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "电镜" }, { "start_idx": 18, "end_idx": 26, "type": "bod", "entity": "大脑神经突触和轴突" }, { "start_idx": 18, "end_idx": 37, "type": "sym", "entity": "大脑神经突触和轴突有糖原堆积形成的α颗粒" } ] }, { "text": "受累组织为肝和肌肉。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "肝" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "肌肉" } ] }, { "text": "临床症状和体征:除肝脏和脾明显增大外,患儿一般尚好,6岁以后有反复性肌肉疼痛,无心脏扩大或低血糖,对胰高血糖素无反应。", "entities": [ { "start_idx": 8, "end_idx": 17, "type": "sym", "entity": "除肝脏和脾明显增大外" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "患儿一般尚好" }, { "start_idx": 26, "end_idx": 37, "type": "sym", "entity": "6岁以后有反复性肌肉疼痛" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "心脏" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "低血糖" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "无心脏扩大或低血糖" }, { "start_idx": 49, "end_idx": 57, "type": "sym", "entity": "对胰高血糖素无反应" } ] }, { "text": "临床症状和体征:酸中毒倾向,生长显著落后,维生素D抵抗性佝偻病,高血脂,尿糖、氨基酸及磷酸排量增高,肾脏大小正常。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "酸中毒倾向" }, { "start_idx": 21, "end_idx": 30, "type": "dis", "entity": "维生素D抵抗性佝偻病" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "高血脂" }, { "start_idx": 36, "end_idx": 37, "type": "ite", "entity": "尿糖" }, { "start_idx": 39, "end_idx": 41, "type": "ite", "entity": "氨基酸" }, { "start_idx": 43, "end_idx": 46, "type": "ite", "entity": "磷酸排量" }, { "start_idx": 36, "end_idx": 48, "type": "sym", "entity": "尿糖、氨基酸及磷酸排量增高" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "肾脏" }, { "start_idx": 50, "end_idx": 55, "type": "sym", "entity": "肾脏大小正常" } ] }, { "text": "6.外周血白细胞DNA分析,进行基因突变检测。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "外周血白细胞DNA分析" }, { "start_idx": 16, "end_idx": 21, "type": "pro", "entity": "基因突变检测" } ] }, { "text": "3.采用低脂饮食预防高脂血症,需注意补充各种微量元素和矿物质。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "高脂血症" } ] }, { "text": "因此对于糖原累积病患者每年进行肝脏超声检查及定期测定血清α-甲胎蛋白。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "ite", "entity": "糖原累积病" }, { "start_idx": 15, "end_idx": 20, "type": "pro", "entity": "肝脏超声检查" }, { "start_idx": 26, "end_idx": 33, "type": "ite", "entity": "血清α-甲胎蛋白" } ] }, { "text": "原发性肾病综合征的病因不清楚,其发病往往因呼吸道感染及过敏反应等而触发,继发性肾病综合征病因则主要有感染、药物、中毒等或继发于肿瘤、遗传及代谢疾病以及全身性系统性疾病之后。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "原发性肾病综合征" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "过敏" }, { "start_idx": 36, "end_idx": 43, "type": "dis", "entity": "继发性肾病综合征" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "感染" }, { "start_idx": 53, "end_idx": 54, "type": "dru", "entity": "药物" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "中毒" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "肿瘤" }, { "start_idx": 66, "end_idx": 72, "type": "dis", "entity": "遗传及代谢疾病" }, { "start_idx": 75, "end_idx": 82, "type": "dis", "entity": "全身性系统性疾病" } ] }, { "text": "儿童肾病综合征以MCN最常见,Glassock报告在1066例儿童肾病中MCN占66%,而在成人病例中仅占21%。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "MCN" }, { "start_idx": 31, "end_idx": 34, "type": "dis", "entity": "儿童肾病" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "MCN" } ] }, { "text": "常见的有呼吸道感染、泌尿道感染、皮肤蜂窝织炎和丹毒及原发性腹膜炎等。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "泌尿道感染" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "皮肤蜂窝织炎" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "丹毒" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "原发性腹膜炎" } ] }, { "text": "病毒感染多发生在接受皮质激素和免疫抑制剂治疗的过程中,多为并发水痘、麻疹及带状疱疹等,病情往往较一般患儿为重。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "病毒感染" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "皮质激素" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "水痘" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "麻疹" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "带状疱疹" } ] }, { "text": "其结果可导致高凝状态,并可发生血栓栓塞并发症,其中以肾静脉血栓形成最为临床重视。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "高凝状态" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "血栓栓塞" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "肾静脉血栓形成" } ] }, { "text": "急性者表现为骤然发作的肉眼血尿和腹痛,检查有脊肋角压痛和肾区肿块,双侧者有急性肾功能减退。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "腹痛" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "脊肋角压痛" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "肾区肿块" }, { "start_idx": 37, "end_idx": 43, "type": "sym", "entity": "急性肾功能减退" } ] }, { "text": "X线检查患肾增大及输尿管有切迹。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "患肾增大" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "输尿管有切迹" } ] }, { "text": "B超有时能检出,必要时肾静脉造影以确诊。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "B超" }, { "start_idx": 11, "end_idx": 15, "type": "pro", "entity": "肾静脉造影" } ] }, { "text": "长期禁盐,过多应用利尿剂以及呕吐、腹泻均可导致低钠血症及低钾血症。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "利尿剂" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "呕吐" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "腹泻" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "低钠血症" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "低钾血症" } ] }, { "text": "当出现厌食、乏力、懒言、嗜睡、血压下降甚至休克、惊厥时应注意有无低钠血症的可能。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "厌食" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "乏力" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "懒言" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "嗜睡" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "血压下降" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "休克" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "惊厥" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "低钠血症" } ] }, { "text": "其原因为:①低血容量,不恰当地大量利尿致肾血液灌注不足,甚至可致肾小管坏死;②严重的肾间质水肿,肾小管为蛋白管型堵塞以致肾小囊及近曲小管内静水压力增高而肾小球滤过减少;③药物引起的肾小管间质病变;④并发双侧肾静脉血栓形成;⑤肾小球严重增生性病变。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "ite", "entity": "血容量" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "利尿" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "肾血液" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "肾小管坏死" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "严重的肾间质水肿" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "肾小管" }, { "start_idx": 60, "end_idx": 68, "type": "bod", "entity": "肾小囊及近曲小管内" }, { "start_idx": 76, "end_idx": 78, "type": "bod", "entity": "肾小球" }, { "start_idx": 48, "end_idx": 82, "type": "sym", "entity": "肾小管为蛋白管型堵塞以致肾小囊及近曲小管内静水压力增高而肾小球滤过减少" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "肾小管" }, { "start_idx": 90, "end_idx": 96, "type": "sym", "entity": "肾小管间质病变" }, { "start_idx": 99, "end_idx": 109, "type": "sym", "entity": "并发双侧肾静脉血栓形成" }, { "start_idx": 112, "end_idx": 114, "type": "bod", "entity": "肾小球" }, { "start_idx": 112, "end_idx": 121, "type": "sym", "entity": "肾小球严重增生性病变" } ] }, { "text": "不应过分低盐以免出现低钠血症,可予盐1~2g/d。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "低钠血症" } ] }, { "text": "也可用黄芪、生地、知母及白术等滋阴补气中药治疗。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dru", "entity": "黄芪" }, { "start_idx": 6, "end_idx": 7, "type": "dru", "entity": "生地" }, { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "知母" }, { "start_idx": 12, "end_idx": 13, "type": "dru", "entity": "白术" } ] }, { "text": "异位的输尿管膨出则可能和其输尿管芽靠近头端,输尿管延迟了从中肾管分离,输尿管末端随之扩张有关。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "输尿管膨出" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "输尿管芽" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "输尿管" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "中肾管" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "输尿管" } ] }, { "text": "由于膀胱颈的括约作用,异位输尿管膨出可引起梗阻。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "膀胱颈" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "异位输尿管膨出" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "梗阻" } ] }, { "text": "输尿管膨出的外层为膀胱黏膜,中间为薄层肌肉和胶原组织,内层为输尿管黏膜。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "输尿管膨出" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "膀胱黏膜" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "薄层肌肉" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "胶原组织" }, { "start_idx": 30, "end_idx": 34, "type": "bod", "entity": "输尿管黏膜" } ] }, { "text": "组织学研究发现输尿管膨出顶部肌细胞小且缺乏,膨出的肌肉中没有厚肌原纤维,因此胚胎发育停滞可能在输尿管膨出形成中起到一定作用。", "entities": [ { "start_idx": 7, "end_idx": 20, "type": "sym", "entity": "输尿管膨出顶部肌细胞小且缺乏" }, { "start_idx": 22, "end_idx": 34, "type": "sym", "entity": "膨出的肌肉中没有厚肌原纤维" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "输尿管膨出" } ] }, { "text": "80%发生于重复肾和输尿管的上肾部输尿管。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "重复肾" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "输尿管" }, { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "上肾部输尿管" } ] }, { "text": "不伴重复肾的异位输尿管膨出罕见,多见于男性。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "重复肾" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "异位输尿管膨出" } ] }, { "text": "异位型输尿管膨出一般较大而开口较小,多位于膀胱基底部近膀胱颈或后尿道内,女孩甚至可脱出尿道,造成尿路梗阻。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "异位型输尿管膨出" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "膀胱基底部" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "膀胱颈" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "后尿道" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "尿道" }, { "start_idx": 48, "end_idx": 51, "type": "dis", "entity": "尿路梗阻" } ] }, { "text": "如效果不满意或出现反流,应做膨出切除及抗反流性输尿管膀胱再吻合术,并同时修复输尿管膨出后侧薄弱的膀胱壁。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "膨出切除" }, { "start_idx": 19, "end_idx": 31, "type": "pro", "entity": "抗反流性输尿管膀胱再吻合术" }, { "start_idx": 36, "end_idx": 50, "type": "pro", "entity": "修复输尿管膨出后侧薄弱的膀胱壁" } ] }, { "text": "反之,若过多地供给水分,超出心肾功能的代偿能力时,则也会引起水中毒,导致水肿、水和电解质紊乱、抽搐和循环衰竭。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "ite", "entity": "心肾功能" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "水中毒" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "水肿" }, { "start_idx": 39, "end_idx": 45, "type": "sym", "entity": "水和电解质紊乱" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "抽搐" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "循环衰竭" } ] }, { "text": "呼气时心率变慢,吸气时心率变快。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "心率" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "心率" } ] }, { "text": "偶尔心率可很慢,并出现交界性逸搏。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "心率" } ] }, { "text": "窦性心律不齐在早产儿常见,特别是心动过缓伴周期性呼吸暂停时。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "窦性心律不齐" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "心动" }, { "start_idx": 24, "end_idx": 25, "type": "ite", "entity": "呼吸" } ] }, { "text": "窦性心动过缓是由于窦房结冲动发放缓慢。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "窦性心动过缓" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "窦房结" } ] }, { "text": "一般情况,1岁以内心率在100次/分以下,1~6岁在80次/分以下,6岁以上在60次/分以下可诊断为窦性心动过缓。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "ite", "entity": "心率" }, { "start_idx": 50, "end_idx": 55, "type": "sym", "entity": "窦性心动过缓" } ] }, { "text": "诊断依靠肺活组织检查。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "ite", "entity": "肺活组织检查" } ] }, { "text": "典型X线胸片改变为弥漫性羽毛状浸润,从肺门弥散到肺周缘。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "ite", "entity": "X线胸片" }, { "start_idx": 6, "end_idx": 16, "type": "sym", "entity": "改变为弥漫性羽毛状浸润" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肺门" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肺周缘" } ] }, { "text": "亦有报道血清抗GM-CSF抗体测定具有很高的敏感度和特异度。", "entities": [ { "start_idx": 4, "end_idx": 14, "type": "ite", "entity": "血清抗GM-CSF抗体" } ] }, { "text": "先天性PAP无特效治疗方法。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "先天性PAP" } ] }, { "text": "如异物较大,阻塞喉腔,可迅速窒息死亡。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "喉腔" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "窒息" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "死亡" } ] }, { "text": "较小尖锐异物,嵌顿于喉部者,出现喉鸣、吸气性呼吸困难、声音嘶哑、疼痛、咯血等。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "喉部" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "喉鸣" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "吸气性呼吸困难" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "疼痛" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "咯血" } ] }, { "text": "如异物阻塞程度较轻,可仅表现喘鸣;不完全阻塞形成活瓣时造成肺气肿;完全阻塞则造成肺不张。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "喘鸣" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "肺气肿" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "肺不张" } ] }, { "text": "此外,如长时间植物(如花生)异物停留,可由于化学刺激而发生植物性支气管炎,后者以咳嗽、败血性发热、呼吸困难为特征。", "entities": [ { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "植物性支气管炎" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "咳嗽" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "败血性发热" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "呼吸困难" } ] }, { "text": "亦可发生慢性化脓性病变。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dis", "entity": "慢性化脓性病变" } ] }, { "text": "根据异物吸入史、典型症状、体征,结合胸部X线检查,一般诊断不难。", "entities": [ { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "胸部X线检查" } ] }, { "text": "必要时做支气管镜检查,以确定诊断。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "pro", "entity": "支气管镜检查" } ] }, { "text": "继发感染者选用适当抗生素。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "感染" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "抗生素" } ] }, { "text": "少数情况下,如植物性异物停留时间过长,可能需要肺叶切除。", "entities": [ { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "肺叶切除" } ] }, { "text": "一般皮肤与70℃热物接触1秒钟即发生水泡,而新生儿却常因50℃的热水袋烫伤。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "皮肤" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "烫伤" } ] }, { "text": "处理时应注意保护创面,保持呼吸道通畅,并迅速建立静脉输液通道,使用镇静和止痛药物。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "保护创面" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "呼吸道" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "静脉输液" }, { "start_idx": 33, "end_idx": 34, "type": "dru", "entity": "镇静" }, { "start_idx": 36, "end_idx": 39, "type": "dru", "entity": "止痛药物" } ] }, { "text": "脑脓肿在任何年龄均可发病,以青壮年最常见。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "新生儿革兰阴性菌和B组溶血性链球菌脑膜炎伴发脑脓肿较多见,婴幼儿脑脓肿相对少见。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "新生儿革兰阴性菌" }, { "start_idx": 9, "end_idx": 19, "type": "dis", "entity": "B组溶血性链球菌脑膜炎" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "在某些高危群体发病率明显增加,如先天性心脏病、免疫缺陷或邻近感染者。", "entities": [ { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "免疫缺陷" } ] }, { "text": "引起脑脓肿的最常见的细菌是链球菌、葡萄球菌、肠道细菌和厌氧菌。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 13, "end_idx": 15, "type": "mic", "entity": "链球菌" }, { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "葡萄球菌" }, { "start_idx": 22, "end_idx": 25, "type": "mic", "entity": "肠道细菌" }, { "start_idx": 27, "end_idx": 29, "type": "mic", "entity": "厌氧菌" } ] }, { "text": "在慢性中耳炎或粒细胞缺乏症的患者,绿脓杆菌感染的发病率增加。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "慢性中耳炎" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "粒细胞缺乏症" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "绿脓杆菌感染" } ] }, { "text": "在先天性或获得性中性粒细胞缺陷、骨髓移植术后或HIV感染的患者,脑脓肿的发生率明显增加,大多数由真菌引起。", "entities": [ { "start_idx": 1, "end_idx": 14, "type": "dis", "entity": "先天性或获得性中性粒细胞缺陷" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "骨髓移植术" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "HIV感染" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 48, "end_idx": 49, "type": "mic", "entity": "真菌" } ] }, { "text": "常见的真菌是念珠菌和曲霉菌;隐球菌通常引起脑膜炎,但也可引起脑脓肿。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "mic", "entity": "念珠菌" }, { "start_idx": 10, "end_idx": 12, "type": "mic", "entity": "曲霉菌" }, { "start_idx": 14, "end_idx": 16, "type": "mic", "entity": "隐球菌" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "芽生菌、组织脑浆菌和球孢子菌等也偶可引起脑脓肿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "芽生菌" }, { "start_idx": 4, "end_idx": 8, "type": "mic", "entity": "组织脑浆菌" }, { "start_idx": 10, "end_idx": 13, "type": "mic", "entity": "球孢子菌" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "其他可引起脑脓肿的致病微生物包括溶组织阿米巴、棘阿米巴、血吸虫、并殖吸虫和弓形体。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 9, "end_idx": 13, "type": "mic", "entity": "致病微生物" }, { "start_idx": 16, "end_idx": 21, "type": "mic", "entity": "溶组织阿米巴" }, { "start_idx": 23, "end_idx": 26, "type": "mic", "entity": "棘阿米巴" }, { "start_idx": 28, "end_idx": 30, "type": "mic", "entity": "血吸虫" }, { "start_idx": 32, "end_idx": 35, "type": "mic", "entity": "并殖吸虫" }, { "start_idx": 37, "end_idx": 39, "type": "mic", "entity": "弓形体" } ] }, { "text": "各种蠕虫蚴体,如粪性圆线虫、旋毛虫以及豚囊虫等也偶可移行至中枢神经系统引起脑脓肿。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "mic", "entity": "粪性圆线虫" }, { "start_idx": 14, "end_idx": 16, "type": "mic", "entity": "旋毛虫" }, { "start_idx": 19, "end_idx": 21, "type": "mic", "entity": "豚囊虫" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "额叶脑脓肿常见病原是微需氧葡萄球菌、厌氧菌和肠杆菌。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "额叶脑脓肿" }, { "start_idx": 10, "end_idx": 16, "type": "mic", "entity": "微需氧葡萄球菌" }, { "start_idx": 18, "end_idx": 20, "type": "mic", "entity": "厌氧菌" }, { "start_idx": 22, "end_idx": 24, "type": "mic", "entity": "肠杆菌" } ] }, { "text": "脑脓肿的发生过程大致可分三期:①急性脑炎期:感染波及脑部引起局灶性化脓性脑炎,局部脑组织出现水肿、坏死或软化灶;②化脓期:炎性坏死和软化灶逐渐扩大、融合,形成较大的脓肿,脓腔外周形成不规则肉芽组织,伴大量中性粒细胞浸润,脓肿周围脑组织重度水肿;③包膜形成期:病变逐渐局限形成包膜,一般在病程1~2周即可初步形成,3~8周形成较完整。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "急性脑炎" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "脑部" }, { "start_idx": 30, "end_idx": 37, "type": "dis", "entity": "局灶性化脓性脑炎" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "脑组织" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "水肿" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "坏死" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "软化灶" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "炎性坏死" }, { "start_idx": 77, "end_idx": 83, "type": "sym", "entity": "形成较大的脓肿" }, { "start_idx": 89, "end_idx": 97, "type": "sym", "entity": "形成不规则肉芽组织" }, { "start_idx": 102, "end_idx": 106, "type": "bod", "entity": "中性粒细胞" }, { "start_idx": 100, "end_idx": 108, "type": "sym", "entity": "大量中性粒细胞浸润" }, { "start_idx": 110, "end_idx": 120, "type": "sym", "entity": "脓肿周围脑组织重度水肿" }, { "start_idx": 129, "end_idx": 138, "type": "sym", "entity": "病变逐渐局限形成包膜" } ] }, { "text": "脑脓肿如破入脑室则形成化脓性脑室炎,引起病情突然恶化,高热、昏迷,甚至死亡。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "化脓性脑室炎" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "恶化" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "高热" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "昏迷" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "死亡" } ] }, { "text": "脑脓肿的临床表现主要包括感染中毒表现、颅内压增高症候和局灶体征。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 12, "end_idx": 17, "type": "sym", "entity": "感染中毒表现" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "颅内压增高症候" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "局灶体征" } ] }, { "text": "在急性脑炎期主要表现为感染中毒症状,常见高热、头痛、呕吐、烦躁、易激惹和惊厥发作。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "急性脑炎" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "感染中毒症状" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "高热" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "头痛" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "呕吐" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "烦躁" }, { "start_idx": 32, "end_idx": 34, "type": "sym", "entity": "易激惹" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "惊厥" } ] }, { "text": "如并发脑膜炎则症状尤著,并有典型脑膜刺激征。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "典型脑膜刺激征" } ] }, { "text": "化脓期和包膜形成期主要表现为颅内压增高症候或局灶体征,体温正常或有低热。", "entities": [ { "start_idx": 14, "end_idx": 20, "type": "sym", "entity": "颅内压增高症候" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "局灶体征" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "体温正常或有低热" } ] }, { "text": "常见剧烈或持续性头痛、喷射性呕吐、意识障碍、血压升高、心率增快、视乳头水肿、头围增大或前囟膨隆以及局灶性惊厥发作等。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "sym", "entity": "剧烈或持续性头痛" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "喷射性呕吐" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "意识障碍" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "血压升高" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "心率增快" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "乳头水肿" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "头围增大" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "前囟膨隆" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "局灶性惊厥" } ] }, { "text": "额叶脓肿常见情感异常、淡漠或性格改变、失语;额顶叶脓肿可有对侧偏瘫或感觉障碍,局灶性惊厥发作常见;小脑脓肿可见共济失调、眼球震颤、眩晕以及肌张力低下等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "额叶" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "额叶脓肿常见情感异常" }, { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "淡漠或性格改变" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "失语" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "额顶叶脓肿" }, { "start_idx": 29, "end_idx": 37, "type": "sym", "entity": "对侧偏瘫或感觉障碍" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "局灶性惊厥发作常见" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "共济失调" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "眼球" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "眼球震颤" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "眩晕" }, { "start_idx": 69, "end_idx": 73, "type": "sym", "entity": "肌张力低下" } ] }, { "text": "C反应蛋白对于鉴别颅内化脓性疾病(如脑脓肿)和非感染性疾病(如肿瘤)有一定的价值。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "dis", "entity": "颅内化脓性疾病" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "非感染性疾病" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "肿瘤" } ] }, { "text": "因此MRI被认为是鉴别颅内化脓性感染的首选诊断性检查。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "pro", "entity": "MRI" } ] }, { "text": "此外,MRI对随诊治疗效果也能提供帮助,获得脑脓肿治疗是否有效的CT信息需1年时间,而MRI的变化在2个月内即可确定。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "MRI" }, { "start_idx": 32, "end_idx": 33, "type": "pro", "entity": "CT" }, { "start_idx": 43, "end_idx": 45, "type": "pro", "entity": "MRI" } ] }, { "text": "隐源性脑脓肿由于缺少上述外周感染史,临床诊断较为困难,确诊仍依赖神经影像学检查。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "隐源性脑脓肿" }, { "start_idx": 32, "end_idx": 38, "type": "pro", "entity": "神经影像学检查" } ] }, { "text": "脑内小脓肿多表现为局灶性癫痫发作,因此对于原因不明的局灶性癫痫患儿,应常规进行增强CT扫描,有条件者行MRI检查,以排除脑内小脓肿的可能性。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "局灶性癫痫" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "CT" }, { "start_idx": 51, "end_idx": 55, "type": "pro", "entity": "MRI检查" }, { "start_idx": 60, "end_idx": 64, "type": "dis", "entity": "脑内小脓肿" } ] }, { "text": "许多颅内感染性疾病的临床和实验室表现与脑脓肿相似,例如脑膜炎、脑炎(大多由病毒引起)、脑外脓肿、(如硬膜下或硬膜外脓肿)以及颅内静脉窦感染。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "颅内感染性疾病" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "脑炎" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "脑外脓肿" }, { "start_idx": 62, "end_idx": 68, "type": "dis", "entity": "颅内静脉窦感染" } ] }, { "text": "颅骨骨髓炎的症状和体征也可与脑脓肿相似。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "中枢神经系统内多发性结核瘤可无症状,也可仅表现为局灶性癫痫发作,与脑内小脓肿相似。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "dis", "entity": "中枢神经系统内多发性结核瘤" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "局灶性癫痫" } ] }, { "text": "近年临床经验表明,头孢三嗪或头孢噻肟加甲硝唑可能是治疗与中耳炎、乳突炎、鼻窦炎或青紫型先天性心脏病有关的脑脓肿的最好的经验性联合用药。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "头孢三嗪" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "头孢噻肟" }, { "start_idx": 19, "end_idx": 21, "type": "dru", "entity": "甲硝唑" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "中耳炎" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "乳突炎" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "鼻窦炎" }, { "start_idx": 40, "end_idx": 48, "type": "dis", "entity": "青紫型先天性心脏病" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "脑脓肿" } ] }, { "text": "如果怀疑葡萄球菌(如头颅穿透伤、脑室腹膜分流术以及瓣膜修复术并发心内膜炎引起的脑脓肿),主张选用万古霉素加第三代头孢菌素(也可用甲硝唑)。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "mic", "entity": "葡萄球菌" }, { "start_idx": 16, "end_idx": 22, "type": "pro", "entity": "脑室腹膜分流术" }, { "start_idx": 25, "end_idx": 29, "type": "pro", "entity": "瓣膜修复术" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "脑脓肿" }, { "start_idx": 48, "end_idx": 51, "type": "dru", "entity": "万古霉素" }, { "start_idx": 53, "end_idx": 59, "type": "dru", "entity": "第三代头孢菌素" }, { "start_idx": 64, "end_idx": 66, "type": "dru", "entity": "甲硝唑" } ] }, { "text": "对于证实有绿脓杆菌感染或有免疫功能缺陷的患者,建议使用头孢噻甲羧肟加万古霉素作为初始的经验治疗。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "绿脓杆菌感染" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "免疫功能缺陷" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "万古霉素" } ] }, { "text": "如果原发病是脑膜炎,由于抗青霉素的肺炎球菌的增多,一般使用万古霉素加头孢三嗪治疗。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 12, "end_idx": 15, "type": "dru", "entity": "抗青霉素" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "万古霉素" }, { "start_idx": 34, "end_idx": 37, "type": "dru", "entity": "头孢三嗪" } ] }, { "text": "如果临床和放射学检查示病情改善较慢,建议全身应用抗生素至少4~6周。", "entities": [ { "start_idx": 24, "end_idx": 26, "type": "dru", "entity": "抗生素" } ] }, { "text": "在高氯性代谢性酸中毒,碳酸氢根的降低被氯离子所替代,而后者可通过血清电解质的测量获得。", "entities": [ { "start_idx": 1, "end_idx": 9, "type": "dis", "entity": "高氯性代谢性酸中毒" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "碳酸氢根" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "氯离子" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "血清" } ] }, { "text": "此外,梗阻也可发生于右心室腔内(右心室双腔),右心室流出道(圆锥部),肺动脉主干以及肺动脉的左右肺内肺外分支。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "梗阻" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "右心室腔" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "右心室双腔" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "右心室流出道" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "圆锥部" }, { "start_idx": 35, "end_idx": 39, "type": "bod", "entity": "肺动脉主干" }, { "start_idx": 42, "end_idx": 53, "type": "bod", "entity": "肺动脉的左右肺内肺外分支" } ] }, { "text": "肺动脉瓣狭窄的发生有家族倾向。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "肺动脉瓣狭窄" } ] }, { "text": "本病的继发病变为右心室向心性的肥厚,室腔可能偏小,心内膜下心肌可有缺血性病变,甚至有右心室心肌梗死。", "entities": [ { "start_idx": 8, "end_idx": 16, "type": "dis", "entity": "右心室向心性的肥厚" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "室腔" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "心内膜下心肌" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "缺血性病变" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "右心室心肌梗死" } ] }, { "text": "亦有患者劳动时感胸痛或上腹痛,可能由于当时心排出量不能相应提高,致使心肌供血不足或心律失常所致,这些都是预后可虑的信号,应着手准备手术。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "sym", "entity": "劳动时感胸痛或上腹痛" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "心" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "心肌供血不足" }, { "start_idx": 41, "end_idx": 44, "type": "dis", "entity": "心律失常" }, { "start_idx": 65, "end_idx": 66, "type": "pro", "entity": "手术" } ] }, { "text": "患儿的生长发育往往正常,甚至有心力衰竭者亦不消瘦,面容往往硕圆(50%),大多无青紫,面颊和指端可能暗红;狭窄严重者可有青紫,大多由于卵圆孔的右向左分流所致,如房间隔缺损很大,可有严重青紫,并有杵状指(趾)及红细胞增多,但有蹲踞者很少见。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "不消瘦" }, { "start_idx": 25, "end_idx": 35, "type": "sym", "entity": "面容往往硕圆(50%)" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "大多无青紫" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "面颊" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "指端" }, { "start_idx": 43, "end_idx": 51, "type": "sym", "entity": "面颊和指端可能暗红" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "狭窄" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "青紫" }, { "start_idx": 67, "end_idx": 69, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 80, "end_idx": 84, "type": "dis", "entity": "房间隔缺损" }, { "start_idx": 90, "end_idx": 93, "type": "sym", "entity": "严重青紫" }, { "start_idx": 104, "end_idx": 106, "type": "bod", "entity": "红细胞" }, { "start_idx": 97, "end_idx": 108, "type": "sym", "entity": "杵状指(趾)及红细胞增多" }, { "start_idx": 111, "end_idx": 117, "type": "sym", "entity": "有蹲踞者很少见" } ] }, { "text": "颈静脉有明显的搏动(a波)者提示狭窄严重,此种收缩期前的搏动在肝区亦可摸到;有心衰时a波则模糊不清,而有右心室收缩时三尖瓣反流的高耸V波。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "颈静脉" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肝区" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "心衰" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "右心室" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "三尖瓣" } ] }, { "text": "心脏多不增大,只有严重狭窄而有心力衰竭者方见心脏扩大;左侧胸骨旁可摸得右心室的抬举搏动。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心脏" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "严重狭窄" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "左侧胸骨" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "右心室" } ] }, { "text": "心音图上示振幅呈渐强后弱的棱形振动,振幅高峰在收缩中期或更晚;频率中或高。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "心音图" } ] }, { "text": "胸部X线检查可发现轻中度狭窄时心脏大小正常,重度狭窄时如心功能尚可,心脏仅轻度增大;如有心力衰竭,心脏则明显增大,甚至大到少见的程度,主要为右心室和右心房扩大。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "胸部X线检查" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "轻中度狭窄" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "心脏" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "重度狭窄" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "心脏" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "心脏" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "右心室" }, { "start_idx": 74, "end_idx": 76, "type": "bod", "entity": "右心房" }, { "start_idx": 70, "end_idx": 78, "type": "sym", "entity": "右心室和右心房扩大" } ] }, { "text": "二维超声可看到肺动脉瓣的厚度和收缩时的开启情况,狭窄后的扩张亦可看到,右心室腔和三尖瓣亦易探得。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "二维超声" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "右心室腔" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "三尖瓣" } ] }, { "text": "如将Bruton病改为X连锁无丙种球蛋白血症(XLA),将瑞士型无丙种球蛋白血症改为严重联合免疫缺陷病(SCID)等。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "Bruton病" }, { "start_idx": 11, "end_idx": 21, "type": "dis", "entity": "X连锁无丙种球蛋白血症" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "XLA" }, { "start_idx": 29, "end_idx": 39, "type": "dis", "entity": "瑞士型无丙种球蛋白血症" }, { "start_idx": 42, "end_idx": 50, "type": "dis", "entity": "严重联合免疫缺陷病" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "SCID" } ] }, { "text": "不同补体成分缺陷有不同临床表现,共同特征是反复感染和易患风湿性疾病。", "entities": [ { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "风湿性疾病" } ] }, { "text": "补体缺陷伴风湿性疾病机制不详,尤其是上游成分C2、C4及C3缺陷,伴风湿性疾病几率高达80%,远高于下游成分缺陷(C5~9)的发病几率(10%),这些主要是系统性红斑狼疮、皮肌炎、硬皮病、过敏性紫癜、血管炎和膜增殖性肾炎。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 78, "end_idx": 84, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 86, "end_idx": 88, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 90, "end_idx": 92, "type": "dis", "entity": "硬皮病" }, { "start_idx": 94, "end_idx": 98, "type": "dis", "entity": "过敏性紫癜" }, { "start_idx": 100, "end_idx": 102, "type": "dis", "entity": "血管炎" }, { "start_idx": 104, "end_idx": 109, "type": "dis", "entity": "膜增殖性肾炎" } ] }, { "text": "雄激素类药物,如stanozolol或danazol可促进C1脂酶抑制物合成,可试用于C1脂酶抑制物缺陷,其他补体成分缺陷治疗主要为对症治疗。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "雄激素类药物" }, { "start_idx": 8, "end_idx": 17, "type": "dru", "entity": "stanozolol" }, { "start_idx": 19, "end_idx": 25, "type": "dru", "entity": "danazol" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "C1脂酶" }, { "start_idx": 43, "end_idx": 46, "type": "bod", "entity": "C1脂酶" } ] }, { "text": "有红斑狼疮体质的人,特别是女性,受到外界的诱因,如紫外线、药物及感染等刺激,引起体内一系列免疫紊乱,导致发病。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "红斑狼疮" } ] }, { "text": "也可有皮肤出血和溃疡。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "皮肤出血" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "溃疡" } ] }, { "text": "②暴发或急性发作出现以下表现之一者:全身极度衰竭伴有剧烈头痛;剧烈腹痛,常类似急腹症;指尖的指甲下或指甲周围出现出血斑;严重口腔溃疡。", "entities": [ { "start_idx": 18, "end_idx": 29, "type": "sym", "entity": "全身极度衰竭伴有剧烈头痛" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "剧烈腹痛" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "急腹症" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "指尖" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "指甲" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "指甲" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "血斑" }, { "start_idx": 60, "end_idx": 65, "type": "sym", "entity": "严重口腔溃疡" } ] }, { "text": "③肾功能进行性下降,伴高血压。", "entities": [ { "start_idx": 1, "end_idx": 1, "type": "bod", "entity": "肾" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "高血压" } ] }, { "text": "④出现狼疮肺炎或肺出血。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "狼疮肺炎" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "肺出血" } ] }, { "text": "近年报道,可应用经颅多普勒超声(TCD)诊断儿童狼疮性脑病,认为TCD有效、简便、无创、价优,有助于长期随访观察SLE病情。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "pro", "entity": "颅多普勒超声" }, { "start_idx": 16, "end_idx": 18, "type": "pro", "entity": "TCD" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "狼疮性脑病" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "TCD" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "SLE" } ] }, { "text": "注意血压,必要时加用血管扩张剂。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "血压" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "血管扩张剂" } ] }, { "text": "可因心肌功能受损或血流动力学负荷过重引起。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "心肌" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "血流" } ] }, { "text": "此外,原发性心肌病如心内膜弹力纤维增生症、先天性心肌病和病毒性心肌炎所致者亦不常见。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "原发性心肌病" }, { "start_idx": 10, "end_idx": 19, "type": "dis", "entity": "心内膜弹力纤维增生症" }, { "start_idx": 21, "end_idx": 26, "type": "dis", "entity": "先天性心肌病" }, { "start_idx": 28, "end_idx": 33, "type": "dis", "entity": "病毒性心肌炎" } ] }, { "text": "高排血量型心力衰竭可能与严重的贫血(Rh同种免疫性疾病、珠蛋白生成障碍性贫血、双胎间输血)或体循环动静脉瘘有关。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "高排血量型心力衰竭" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "贫血" }, { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "Rh同种免疫性疾病" }, { "start_idx": 28, "end_idx": 37, "type": "dis", "entity": "珠蛋白生成障碍性贫血" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "输血" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "体循环动静脉瘘" } ] }, { "text": "心律失常同样可导致心力衰竭。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心律失常" } ] }, { "text": "引起心力衰竭的其他原因,如继发的心肌功能障碍、心律失常如前所述。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "心律失常" } ] }, { "text": "2.小婴儿心力衰竭(出生后2至3个月)左向右分流型的心脏结构畸形多在此时期出现心力衰竭的典型表现,这与生后肺血管阻力降低和肺血流量增加有关。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 19, "end_idx": 31, "type": "sym", "entity": "左向右分流型的心脏结构畸形" }, { "start_idx": 39, "end_idx": 42, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "肺血管" } ] }, { "text": "心力衰竭的药物治疗,可以减轻体循环静脉淤血(利尿剂),改善心肌收缩功能(正性肌力药物)或减轻心脏后负荷(血管扩张剂)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 14, "end_idx": 20, "type": "pro", "entity": "体循环静脉淤血" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "利尿剂" }, { "start_idx": 29, "end_idx": 34, "type": "sym", "entity": "心肌收缩功能" }, { "start_idx": 36, "end_idx": 41, "type": "dru", "entity": "正性肌力药物" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "心脏" }, { "start_idx": 52, "end_idx": 56, "type": "dru", "entity": "血管扩张剂" } ] }, { "text": "临床常用的利尿剂有袢利尿剂、醛固酮拮抗剂和噻嗪类(氯噻嗪、美托拉宗)。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "利尿剂" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "袢利尿剂" }, { "start_idx": 14, "end_idx": 19, "type": "dru", "entity": "醛固酮拮抗剂" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "噻嗪类" }, { "start_idx": 25, "end_idx": 27, "type": "dru", "entity": "氯噻嗪" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "美托拉宗" } ] }, { "text": "袢利尿剂(呋塞米、依他尼酸)常用且有效。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "袢利尿剂" }, { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "呋塞米" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "依他尼酸" } ] }, { "text": "第三代β受体阻滞剂(卡维地洛、布新洛尔)另有血管扩张作用,可有效改善血流动力学。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dru", "entity": "β受体阻滞剂" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "卡维地洛" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "布新洛尔" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "血管" } ] }, { "text": "近年认识到它是引起小儿肾衰竭的重要原因;据估计成年人TIN占急性肾衰竭的5%~15%,进入终末期肾衰中占25%:小儿则分别为5%和6%~8%。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "小儿肾衰竭" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "TIN" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "终末期肾衰" } ] }, { "text": "前者急起,可表现为急性肾衰竭、肾小管功能障碍及尿沉渣异常,组织学上以肾间质水肿和细胞浸润为主;慢性者常呈一不可逆过程,以间质纤维化和小管萎缩为特点。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肾小管" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "肾小管功能障碍" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "尿沉渣异常" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "肾间质" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "肾间质水肿" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "细胞" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "细胞浸润" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "间质" }, { "start_idx": 60, "end_idx": 64, "type": "sym", "entity": "间质纤维化" }, { "start_idx": 66, "end_idx": 67, "type": "bod", "entity": "小管" }, { "start_idx": 66, "end_idx": 69, "type": "sym", "entity": "小管萎缩" } ] }, { "text": "前者如细菌、钩端螺旋体、分枝杆菌、CMV病毒、Hanta病毒以及多瘤病毒等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "细菌" }, { "start_idx": 6, "end_idx": 10, "type": "mic", "entity": "钩端螺旋体" }, { "start_idx": 12, "end_idx": 15, "type": "mic", "entity": "分枝杆菌" }, { "start_idx": 17, "end_idx": 21, "type": "mic", "entity": "CMV病毒" }, { "start_idx": 23, "end_idx": 29, "type": "mic", "entity": "Hanta病毒" }, { "start_idx": 32, "end_idx": 35, "type": "mic", "entity": "多瘤病毒" } ] }, { "text": "儿科最突出的是系统性红斑狼疮,在13%~67%的狼疮病人中肾小管可见免疫复合物沉着,而且TIN是狼疮肾进展和影响预后的重要因素。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "系统性红斑狼疮" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "狼疮" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "肾小管" }, { "start_idx": 29, "end_idx": 40, "type": "sym", "entity": "肾小管可见免疫复合物沉着" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "TIN" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "狼疮肾" } ] }, { "text": "此外TIN也偶见于原发性或梅毒引起的膜性肾病。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "TIN" }, { "start_idx": 13, "end_idx": 14, "type": "mic", "entity": "梅毒" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "膜性肾病" } ] }, { "text": "全身性免疫性紊乱时也可仅间质及小管受累,如肾移植时的排异反应,另一为TINU综合征,即小管间质性肾炎伴眼色素膜炎。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "全身性免疫性紊乱" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "间质" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "小管" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "间质及小管受累" }, { "start_idx": 21, "end_idx": 23, "type": "pro", "entity": "肾移植" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "TINU综合征" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "小管间质性肾炎" }, { "start_idx": 51, "end_idx": 55, "type": "dis", "entity": "眼色素膜炎" } ] }, { "text": "此征1975年始被报道,病人有急性TIN和眼色素膜炎和骨髓肉芽肿,表现有虚弱、厌食、发热、体重下降及多尿。", "entities": [ { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "急性TIN" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "眼色素膜炎" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "骨髓肉芽肿" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "虚弱" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "厌食" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "发热" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "体重下降" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "多尿" } ] }, { "text": "眼部有流泪、眼痛及眼色素膜炎。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "眼部有流泪" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "眼痛" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "眼色素膜炎" } ] }, { "text": "在小儿时期最多见于各种尿路梗阻(UTO)和重度的膀胱输尿管反流(VUR)。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "尿路梗阻" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "UTO" }, { "start_idx": 21, "end_idx": 30, "type": "dis", "entity": "重度的膀胱输尿管反流" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "VUR" } ] }, { "text": "在小儿时期慢性TIN还可由代谢病引起,如①胱氨酸病:见本章第四节。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "慢性TIN" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "胱氨酸病" } ] }, { "text": "②草酸盐过度产生或小肠过度吸收,造成肾排出草酸盐增多,则肾小管内草酸钙结晶沉积,受累小管萎缩,周围炎症细胞浸润和纤维化。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "草酸盐" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "小肠" }, { "start_idx": 1, "end_idx": 14, "type": "sym", "entity": "草酸盐过度产生或小肠过度吸收" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "肾" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "草酸盐" }, { "start_idx": 18, "end_idx": 25, "type": "sym", "entity": "肾排出草酸盐增多" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "肾小管" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "草酸钙" }, { "start_idx": 28, "end_idx": 38, "type": "sym", "entity": "肾小管内草酸钙结晶沉积" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "受累小管" }, { "start_idx": 40, "end_idx": 45, "type": "sym", "entity": "受累小管萎缩" }, { "start_idx": 49, "end_idx": 52, "type": "bod", "entity": "炎症细胞" }, { "start_idx": 47, "end_idx": 58, "type": "sym", "entity": "周围炎症细胞浸润和纤维化" } ] }, { "text": "③高钙血症。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "高钙血症" } ] }, { "text": "④钾不足:严重钾不足时主要为近曲小管受累(上皮空泡变性)。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "sym", "entity": "钾不足" }, { "start_idx": 14, "end_idx": 17, "type": "bod", "entity": "近曲小管" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "近曲小管受累" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "上皮空泡变性" } ] }, { "text": "⑤尿酸盐:尿酸负荷致肾受损,不定形尿酸盐结晶沉于肾间质引起周围巨噬细胞反应,与此同时,在小管及集合管中也有其结晶,最终导致间质纤维化、小管扩张及萎缩,此种损害只发生于血尿酸持续>595~773μmol/L(10~13mg/dl)时。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "尿酸负荷" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肾受损" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "肾间质" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "不定形尿酸盐结晶沉于肾间质" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "巨噬细胞" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "周围巨噬细胞反应" }, { "start_idx": 44, "end_idx": 49, "type": "bod", "entity": "小管及集合管" }, { "start_idx": 44, "end_idx": 55, "type": "sym", "entity": "小管及集合管中也有其结晶" }, { "start_idx": 61, "end_idx": 65, "type": "sym", "entity": "间质纤维化" }, { "start_idx": 67, "end_idx": 73, "type": "sym", "entity": "小管扩张及萎缩" }, { "start_idx": 83, "end_idx": 85, "type": "ite", "entity": "血尿酸" }, { "start_idx": 83, "end_idx": 113, "type": "sym", "entity": "血尿酸持续>595~773μmol/L(10~13mg/dl)" } ] }, { "text": "可表现为急性肾衰竭及肾小管功能障碍,偶见肾病综合征。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "急性肾衰竭" }, { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "肾小管功能障碍" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "肾病综合征" } ] }, { "text": "起病时乏力、厌食、体重下降、腹痛、头痛、苍白及呕吐。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "乏力" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "厌食" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "体重下降" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "腹痛" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "头痛" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "苍白" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "呕吐" } ] }, { "text": "疾病后期表现慢性肾衰竭,伴显著高血压、高血压眼底改变及左心室肥厚,此时常难于区别原发病为肾小球疾病或间质炎症改变。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "慢性肾衰竭" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "高血压" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "高血压眼底改变" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "左心室" }, { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "左心室肥厚" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "肾小球疾病" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "间质" }, { "start_idx": 50, "end_idx": 55, "type": "sym", "entity": "间质炎症改变" } ] }, { "text": "因此时病理上多兼有肾小球硬化和间质纤维化。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "肾小球硬化" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "间质纤维化" } ] }, { "text": "尿沉渣瑞氏染色可检见嗜酸粒细胞,此对本症诊断有助;正常时尿中无嗜酸细胞,当其占尿白细胞中1%~5%,即有诊断意义,由药物引起之急性TIN患者中50%~90%为阳性。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "尿沉渣瑞氏染色" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "嗜酸粒细胞" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "尿" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "嗜酸细胞" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "尿中无嗜酸细胞" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "尿白细胞" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "急性TIN" } ] }, { "text": "对有造成TIN的病因存在、发生肾功能减退以及肾小管功能障碍者应疑及本症,确诊依赖肾活体组织检查。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "TIN" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "肾功能" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "肾功能减退" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肾小管" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "肾小管功能障碍" }, { "start_idx": 40, "end_idx": 46, "type": "pro", "entity": "肾活体组织检查" } ] }, { "text": "在一回顾性研究中,应用泼尼松4~6周者,其ARF恢复时间虽与未用者相似,但8周时治疗组血肌酐水平较对照组为低。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "泼尼松" }, { "start_idx": 42, "end_idx": 45, "type": "ite", "entity": "组血肌酐" } ] }, { "text": "先天性甲状腺功能减低症和急性淋巴细胞性白血病的发生率明显高于正常人群,免疫功能低下,易患感染性疾病。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "dis", "entity": "先天性甲状腺功能减低症" }, { "start_idx": 12, "end_idx": 21, "type": "dis", "entity": "急性淋巴细胞性白血病" }, { "start_idx": 35, "end_idx": 40, "type": "sym", "entity": "免疫功能低下" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "感染性疾病" } ] }, { "text": "如存活至成人期,则常在30岁以后即出现老年性痴呆症状。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "老年性痴呆" } ] }, { "text": "因此,对怀孕早、中期的孕妇开展21-三体综合征筛查,及早采取积极预防措施,对保证妇幼健康水平有一定意义。", "entities": [ { "start_idx": 15, "end_idx": 22, "type": "dis", "entity": "21-三体综合征" } ] }, { "text": "产前筛查血清标志物HCG及AFP测定有一定临床意义,因为它能够减少羊膜穿刺进行产前诊断的盲目性,提示高危孕妇群的存在,使这些孕妇得以作进一步的产前检查和咨询,最大限度地防止21-三体综合征患儿的出生。", "entities": [ { "start_idx": 4, "end_idx": 17, "type": "pro", "entity": "血清标志物HCG及AFP测定" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "羊膜穿刺" }, { "start_idx": 86, "end_idx": 93, "type": "dis", "entity": "21-三体综合征" } ] }, { "text": "亦称心室双入口。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "心室双入口" } ] }, { "text": "动脉干发自原始心球,前者以后发育为主动脉、肺动脉。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "动脉干" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "心球" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "主动脉" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "肺动脉" } ] }, { "text": "最常见的单心室为左心室型单心室、主动脉位于肺动脉左侧、主动脉起自左侧的残余右心室,与纠正型大血管转位相似。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "单心室" }, { "start_idx": 8, "end_idx": 14, "type": "dis", "entity": "左心室型单心室" }, { "start_idx": 16, "end_idx": 25, "type": "dis", "entity": "主动脉位于肺动脉左侧" }, { "start_idx": 27, "end_idx": 39, "type": "dis", "entity": "主动脉起自左侧的残余右心室" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "血管" } ] }, { "text": "有一种特殊的少见类型的单心室称Holmes心,表现为单一左心室双流入道及肺动脉狭窄但不伴有大动脉转位。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "单心室" }, { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "Holmes心" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "单一左心室双流入道" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "肺动脉狭窄" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "大动脉转位" } ] }, { "text": "患儿如不伴有肺动脉狭窄,在婴儿期随着胎儿期肺血管阻力的退化,肺血流逐渐增加,最终形成充血性心力衰竭而往往无法存活。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 21, "end_idx": 25, "type": "ite", "entity": "肺血管阻力" }, { "start_idx": 30, "end_idx": 32, "type": "ite", "entity": "肺血流" }, { "start_idx": 42, "end_idx": 48, "type": "sym", "entity": "充血性心力衰竭" } ] }, { "text": "单心室患儿如伴有肺动脉闭锁,在生后即出现青紫。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "单心室" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "肺动脉闭锁" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "青紫" } ] }, { "text": "如果伴有严重的肺动脉狭窄,则由于明显的青紫而发现更早。", "entities": [ { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "青紫" } ] }, { "text": "通常需在生后6个月至1岁内再次进行心导管造影检查以判定前期处理的效果并确定进一步外科手术纠治的指征。", "entities": [ { "start_idx": 17, "end_idx": 23, "type": "pro", "entity": "心导管造影检查" } ] }, { "text": "异丙肾上腺素是最后可用的方法。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "异丙肾上腺素" } ] }, { "text": "除了有明显可逆性原因外,一般均应作电生理检查。", "entities": [ { "start_idx": 17, "end_idx": 21, "type": "ite", "entity": "电生理检查" } ] }, { "text": "如为WPW综合征,应予消融。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "WPW综合征" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "消融" } ] }, { "text": "如为原因不明或为非可逆性原因引起,应予安置AICD预防猝死。", "entities": [ { "start_idx": 19, "end_idx": 24, "type": "pro", "entity": "安置AICD" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "猝死" } ] }, { "text": "多发生于皮肤、软组织、肝和脾等处。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "皮肤" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "软组织" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "肝" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "脾" } ] }, { "text": "肿瘤以皮肤的发生率最高,其他依次为软组织、乳房、肝、脾、心脏等处。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "皮肤" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "软组织" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "乳房" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "肝" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "脾" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "心脏" } ] }, { "text": "皮肤肿瘤多见于头颈部,其次为上下肢、躯干,表现为高出皮肤的结节,直径1~2cm,表面常见坏死破溃。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "皮肤肿瘤" }, { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "头颈部" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "上下肢" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "躯干" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "皮肤" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "结节" }, { "start_idx": 24, "end_idx": 38, "type": "sym", "entity": "高出皮肤的结节,直径1~2cm" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "坏死破溃" } ] }, { "text": "病理诊断上本瘤易与癌及血管丰富的转移性癌、上皮细胞型滑膜肉瘤、高分化的纤维肉瘤及血管内乳头状内皮增生症等相混淆。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "病理诊断" }, { "start_idx": 9, "end_idx": 9, "type": "dis", "entity": "癌" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血管" }, { "start_idx": 21, "end_idx": 29, "type": "dis", "entity": "上皮细胞型滑膜肉瘤" }, { "start_idx": 31, "end_idx": 38, "type": "dis", "entity": "高分化的纤维肉瘤" }, { "start_idx": 40, "end_idx": 50, "type": "dis", "entity": "血管内乳头状内皮增生症" } ] }, { "text": "免疫组织化学染色有助于其鉴别诊断,如:Ⅷ因子相关抗原由内皮细胞合成及其来源肿瘤的特异标记物。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "免疫组织化学染色" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "抗原" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "内皮细胞" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "肿瘤" } ] }, { "text": "该肿瘤因局部广泛扩散或远处转移,死亡率高。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" } ] }, { "text": "肿瘤常见转移到肺和肝,也可至区域淋巴结,对放疗敏感度低。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "肺" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "肝" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "淋巴结" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "放疗" } ] }, { "text": "只有早期局限的四肢、躯干肿瘤可能获得根治手术和切除。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "四肢、躯干肿瘤" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "手术" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "切除" } ] }, { "text": "除畸胎瘤有完整包膜,为局限性非侵袭性生长,其余肿瘤大多呈侵袭性生长,并可沿脑脊液发生播散性种植或远处转移如骨、肝及淋巴结等。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "畸胎瘤" }, { "start_idx": 23, "end_idx": 24, "type": "dis", "entity": "肿瘤" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "脑脊液" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "骨" }, { "start_idx": 55, "end_idx": 55, "type": "bod", "entity": "肝" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "淋巴结" } ] }, { "text": "辅助检查MRI是诊断该部位肿瘤最有效的检查,由于肿瘤易沿脑脊液播散,因此,必要时还应作脊柱MRI检查及脑脊液细胞学检查。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "pro", "entity": "MRI" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "肿瘤" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "肿瘤" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "脑脊液" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "脊柱" }, { "start_idx": 45, "end_idx": 47, "type": "pro", "entity": "MRI" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "脑脊液" }, { "start_idx": 54, "end_idx": 58, "type": "pro", "entity": "细胞学检查" } ] }, { "text": "由于肿瘤位于脑重要部位,手术死亡率高,因此除畸胎瘤要求完整切除外,其余肿瘤手术目的是部分切除肿瘤,解除脑积水并了解肿瘤性质,或仅做立体定向活检,以利放疗或化疗。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" }, { "start_idx": 6, "end_idx": 6, "type": "bod", "entity": "脑" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "手术" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "畸胎瘤" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "切除" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "肿瘤手术" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "部分切除" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "肿瘤" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "脑积水" }, { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "肿瘤" }, { "start_idx": 65, "end_idx": 70, "type": "pro", "entity": "立体定向活检" }, { "start_idx": 74, "end_idx": 75, "type": "pro", "entity": "放疗" }, { "start_idx": 77, "end_idx": 78, "type": "pro", "entity": "化疗" } ] }, { "text": "放疗是主要治疗手段,松果体区恶性肿瘤如生殖细胞瘤等对放疗敏感,许多作者认为应常规行全脑和脊髓放疗,以防播散。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "放疗" }, { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "松果体区恶性肿瘤" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "生殖细胞瘤" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "放疗" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "全脑" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "脊髓" }, { "start_idx": 46, "end_idx": 47, "type": "pro", "entity": "放疗" } ] }, { "text": "大的、高度恶性的或有下丘脑浸润或转移的肿瘤预后极差,生存期不到1年。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "下丘脑" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "肿瘤" } ] }, { "text": "本病患者近亲发病率也高,国外报道12%SLE患儿近亲中患有同类疾病,其他自身免疫性疾病发病率也高于人群总发病率。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "SLE" } ] }, { "text": "感染诱发SLE也研究较多。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "感染" }, { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "SLE" } ] }, { "text": "T细胞功能紊乱可能源自细胞内信号传递异常,如细胞黏附分子异常,引起细胞间相互识别,黏合,信号传递障碍等,可能在SLE发病机制中具有重要作用。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "T细胞" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "T细胞功能紊乱" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "细胞" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "细胞内信号传递异常" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "细胞黏附分子" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "细胞黏附分子异常" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "细胞" }, { "start_idx": 33, "end_idx": 49, "type": "sym", "entity": "细胞间相互识别,黏合,信号传递障碍" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "SLE" } ] }, { "text": "近年已开始注意有患儿发生冠状动脉炎及心肌梗死的病例。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "冠状动脉炎" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "心肌梗死" } ] }, { "text": "可出现巩膜炎、虹膜炎及视网膜炎等眼部症状。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "巩膜炎" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "虹膜炎" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "视网膜炎" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "眼部症状" } ] }, { "text": "服免疫抑制剂期间尽量不到公共场所,减少感染机会,若发生感染应积极治疗,要避免使用诱发狼疮和肾损害的常用药物(磺胺、肼苯达嗪、普鲁卡因胺、对氨基水杨酸、青霉素及氨基甙类药物);局部皮损若无继发感染,可涂泼尼松软膏。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "感染" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "感染" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "狼疮" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "肾损害" }, { "start_idx": 54, "end_idx": 55, "type": "dru", "entity": "磺胺" }, { "start_idx": 57, "end_idx": 60, "type": "dru", "entity": "肼苯达嗪" }, { "start_idx": 62, "end_idx": 66, "type": "dru", "entity": "普鲁卡因胺" }, { "start_idx": 68, "end_idx": 73, "type": "dru", "entity": "对氨基水杨酸" }, { "start_idx": 75, "end_idx": 77, "type": "dru", "entity": "青霉素" }, { "start_idx": 79, "end_idx": 84, "type": "dru", "entity": "氨基甙类药物" }, { "start_idx": 87, "end_idx": 90, "type": "dis", "entity": "局部皮损" }, { "start_idx": 100, "end_idx": 104, "type": "dru", "entity": "泼尼松软膏" } ] }, { "text": "轻者仅表现为红斑,水疱;重者表现为局部组织坏死、溃疡,可伴有皮肤或其他部位的黏膜损害。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "红斑" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "水疱" }, { "start_idx": 12, "end_idx": 25, "type": "sym", "entity": "重者表现为局部组织坏死、溃疡" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "皮肤" }, { "start_idx": 27, "end_idx": 41, "type": "sym", "entity": "可伴有皮肤或其他部位的黏膜损害" } ] }, { "text": "主要是去除致敏物质和抗过敏治疗。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "去除致敏物质" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "抗过敏治疗" } ] }, { "text": "抗过敏药物有盐酸苯海拉明及氯苯那敏。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "抗过敏药物" }, { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "盐酸苯海拉明" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "氯苯那敏" } ] }, { "text": "必要时可用泼尼松及地塞米松。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "泼尼松" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "地塞米松" } ] }, { "text": "对症治疗包括局部止痛和抗感染等。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "dru", "entity": "包括局部止痛" }, { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "抗感染" } ] }, { "text": "在第二肿瘤中,AML是一种较常见的类型,主要与先前烷化剂如环磷酰胺、氮芥、马利兰等暴露有关,常发生于第一肿瘤4~5年后,可先表现为骨髓增生异常综合征(MDS)再发展为AML,但在10~12年后AML的发病机会减少。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "第二肿瘤" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "AML" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "第一肿瘤" }, { "start_idx": 65, "end_idx": 73, "type": "dis", "entity": "骨髓增生异常综合征" }, { "start_idx": 75, "end_idx": 77, "type": "dis", "entity": "MDS" }, { "start_idx": 83, "end_idx": 85, "type": "dis", "entity": "AML" }, { "start_idx": 96, "end_idx": 98, "type": "dis", "entity": "AML" } ] }, { "text": "除此以外,鬼臼毒素类药物VP-16的暴露与第二肿瘤性AML的发病有关,其发病时间常较烷化剂诱发者早。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "鬼臼毒素" }, { "start_idx": 12, "end_idx": 16, "type": "dru", "entity": "VP-16" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "第二肿瘤性AML" } ] }, { "text": "与ALL不同的是M3型临床有更严重的出血倾向,在治疗前及刚开始治疗时易发生DIC,而M5型齿龈浸润较多见。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "ALL" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "更严重的出血倾向" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "DIC" }, { "start_idx": 45, "end_idx": 48, "type": "sym", "entity": "齿龈浸润" } ] }, { "text": "体格检查除不同程度面色苍白、出血点、紫癜外,半数以上病人有肝、脾、淋巴结不同程度肿大;皮肤、皮下组织浸润时扪及结节,眼眶部浸润时可有眼球突出,这些浸润性肿块(肿瘤部分)切面可因肿瘤细胞所含的髓过氧化酶作用而转化为绿色,因此又将其称为“绿色瘤”。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "面色苍白" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "出血点" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "紫癜" }, { "start_idx": 29, "end_idx": 41, "type": "sym", "entity": "肝、脾、淋巴结不同程度肿大" }, { "start_idx": 43, "end_idx": 56, "type": "sym", "entity": "皮肤、皮下组织浸润时扪及结节" }, { "start_idx": 58, "end_idx": 69, "type": "sym", "entity": "眼眶部浸润时可有眼球突出" }, { "start_idx": 79, "end_idx": 80, "type": "dis", "entity": "肿瘤" }, { "start_idx": 88, "end_idx": 91, "type": "bod", "entity": "肿瘤细胞" }, { "start_idx": 95, "end_idx": 99, "type": "bod", "entity": "髓过氧化酶" } ] }, { "text": "外周血检查表现为红细胞、血红蛋白不同程度的下降;白细胞计数可明显升高并出现幼稚细胞,或反而减少,此时又称低增生性白血病;血小板一般均中重度减少,偶见正常者。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "外周血检查" }, { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "红细胞" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 24, "end_idx": 26, "type": "ite", "entity": "白细胞" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "幼稚细胞" }, { "start_idx": 52, "end_idx": 58, "type": "dis", "entity": "低增生性白血病" }, { "start_idx": 60, "end_idx": 62, "type": "ite", "entity": "血小板" } ] }, { "text": "起病较急,常见表现有突发头痛,呕吐,偏瘫,失语,惊厥发作,视物模糊或偏盲,感觉障碍,血压、心率及呼吸改变,意识障碍等。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "突发头痛" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "呕吐" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "偏瘫" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "失语" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "惊厥" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "视物模糊" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "偏盲" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "感觉障碍" }, { "start_idx": 42, "end_idx": 51, "type": "sym", "entity": "血压、心率及呼吸改变" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "意识障碍" } ] }, { "text": "血肿破入蛛网膜下腔者常有明显的脑膜刺激征。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "脑膜刺激征" } ] }, { "text": "脑室出血常表现为深昏迷,四肢软瘫,早期高热,双瞳孔缩小,去脑强直样发作。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "脑室出血" }, { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "深昏迷" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "四肢软瘫" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "早期高热" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "双瞳孔缩小" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "去脑强直样发作" } ] }, { "text": "因动脉瘤以及动静脉畸形等血管异常所致者以6岁以上年长儿较多见,且有随年龄增长而逐渐增多的趋势。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "动静脉畸形" } ] }, { "text": "常起病急剧,主要表现为血液刺激或容量增加所致的脑膜刺激征和颅内高压征,如颈项强直、剧烈头痛以及喷射性呕吐等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "起病急剧" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "血液刺激" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "容量增加" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "脑膜刺激征" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "颅内高压征" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "颈项强直" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "剧烈头痛" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "喷射性呕吐" } ] }, { "text": "半数以上病例出现意识障碍、面色苍白和惊厥发作。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "意识障碍" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "面色苍白" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "惊厥发作" } ] }, { "text": "硬膜下出血所形成的血肿大多发生于大脑顶部,多数为双侧,但出血程度可不对称。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "硬膜下出血" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "大脑顶部" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "出血程度可不对称" } ] }, { "text": "脑室周围-脑室内出血主要发生于胎龄较小的未成熟儿,源于室管膜下的生发层毛细血管破裂所致,多于生后24~48小时内发病,多数起病急骤,进行性恶化,生后不久即出现深昏迷、去脑强直与惊厥,多于数小时内死亡;但少数开始时症状亦可不典型,可有意识障碍、局限性“微小型”惊厥、眼球运动障碍以及肢体功能障碍等,症状起伏,时轻时重,多能存活,但易并发脑积水。", "entities": [ { "start_idx": 27, "end_idx": 40, "type": "sym", "entity": "室管膜下的生发层毛细血管破裂" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "起病急骤" }, { "start_idx": 66, "end_idx": 70, "type": "sym", "entity": "进行性恶化" }, { "start_idx": 79, "end_idx": 81, "type": "sym", "entity": "深昏迷" }, { "start_idx": 83, "end_idx": 86, "type": "sym", "entity": "去脑强直" }, { "start_idx": 88, "end_idx": 89, "type": "sym", "entity": "惊厥" }, { "start_idx": 97, "end_idx": 98, "type": "sym", "entity": "死亡" }, { "start_idx": 116, "end_idx": 119, "type": "sym", "entity": "意识障碍" }, { "start_idx": 121, "end_idx": 130, "type": "sym", "entity": "局限性“微小型”惊厥" }, { "start_idx": 132, "end_idx": 137, "type": "sym", "entity": "眼球运动障碍" }, { "start_idx": 140, "end_idx": 145, "type": "sym", "entity": "肢体功能障碍" }, { "start_idx": 167, "end_idx": 169, "type": "sym", "entity": "脑积水" } ] }, { "text": "约10%合并其他结缔组织病,如JRA、SLE及硬皮病等,少数合并恶性肿瘤。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "JRA" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "SLE" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "硬皮病" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "多种感染,尤其是病毒感染,特别是柯萨奇病毒与皮肌炎发病有关。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "mic", "entity": "病毒" }, { "start_idx": 16, "end_idx": 20, "type": "mic", "entity": "柯萨奇病毒" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "皮肌炎" } ] }, { "text": "10%患儿咽喉肌受累,导致吞咽困难;5%患儿面肌和眼外肌受累导致面部表情少及眼睑外翻。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "咽喉肌" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "咽喉肌受累" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "面肌" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "眼外肌" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "面部" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "面部表情少" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "眼睑" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "眼睑外翻" } ] }, { "text": "晚期有肌肉萎缩和关节挛缩。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "肌肉" }, { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "肌肉萎缩" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "关节" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "关节挛缩" } ] }, { "text": "皮疹轻重程度及持续时间不等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "皮疹" } ] }, { "text": "皮疹消退后可留有色素沉着。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "皮疹" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "色素沉着" } ] }, { "text": "常继发于细菌性肺炎,亦可为吸入性或血源性感染。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "吸入性" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "血源性感染" } ] }, { "text": "外周血白细胞计数和中性粒细胞升高,结合X线后前位及侧位胸片,诊断多不困难。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "外周血白细胞计数" }, { "start_idx": 9, "end_idx": 13, "type": "ite", "entity": "中性粒细胞" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "X线" } ] }, { "text": "痰培养、血培养可明确病原。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "痰培养" }, { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "血培养" } ] }, { "text": "怀疑金葡菌者宜首选苯唑西林或万古霉素;厌氧菌感染给予青霉素G、克林霉素、哌拉西林钠、甲硝唑等。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "金葡菌" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "苯唑西林" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "万古霉素" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "厌氧菌感染" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "青霉素G" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "克林霉素" }, { "start_idx": 36, "end_idx": 40, "type": "dru", "entity": "哌拉西林钠" }, { "start_idx": 42, "end_idx": 44, "type": "dru", "entity": "甲硝唑" } ] }, { "text": "房颤时心房激动紊乱,节律快于房扑(300~700次/分),心室律及脉搏不规则。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "房颤" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "心房" }, { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "心房激动紊乱" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "心室" }, { "start_idx": 33, "end_idx": 34, "type": "ite", "entity": "脉搏" }, { "start_idx": 29, "end_idx": 37, "type": "sym", "entity": "心室律及脉搏不规则" } ] }, { "text": "房颤偶尔也可见于心房内手术后,继发于左心房室瓣关闭不全的左心房扩大,WPW综合征等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "房颤" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心房" }, { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "左心房室瓣" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "左心房" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "左心房扩大" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "WPW综合征" } ] }, { "text": "治疗房颤的首选药物为洋地黄,它可使心室率恢复正常,但此时房颤通常持续(WPW综合征患儿不可应用洋地黄)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "房颤" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "洋地黄" }, { "start_idx": 17, "end_idx": 19, "type": "ite", "entity": "心室率" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "房颤" }, { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "WPW综合征" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "洋地黄" } ] }, { "text": "此后可用Ⅰ类抗心律失常药如奎尼丁、普鲁卡因胺或直流电复律来转律。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "ite", "entity": "心律" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "奎尼丁" }, { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "普鲁卡因胺" } ] }, { "text": "淹溺的分类大多根据淹溺时间及缺氧程度、淹溺液体性质(淡水、海水、污水等)、出现并发症等情况而划分。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "淹溺" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "淹溺" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "淹溺" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "并发症" } ] }, { "text": "淹溺(near-drowning)一般指发生淹溺后存活24小时以上;溺死(drowning)指因淹溺窒息于24小时内死亡。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "淹溺" }, { "start_idx": 3, "end_idx": 15, "type": "dis", "entity": "near-drowning" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "淹溺" } ] }, { "text": "在意外死亡原因排位中,淹溺位居第1~2位,部分地区报道儿童淹溺发病率为10/10万,其中幼儿期发病率最高。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "淹溺" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "淹溺" } ] }, { "text": "淹溺亦可发生在家中。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "淹溺" } ] }, { "text": "航船意外引起溺水的原因多为船只倾覆后遇险者不会游泳、寒冷、体能耗竭和缺乏救护支援和救生用品。", "entities": [ { "start_idx": 21, "end_idx": 44, "type": "sym", "entity": "不会游泳、寒冷、体能耗竭和缺乏救护支援和救生用品" } ] }, { "text": "淹溺后气道内无吸入液体称为“干溺”。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "气道" } ] }, { "text": "有人推测干溺者在溺水时上呼吸道亦有水进入,但由于刺激了咽喉产生了咳嗽及吞咽反射,反射性地引起喉痉挛、声门关闭和窒息。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "上呼吸道" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "咽喉" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "喉" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "声门" }, { "start_idx": 46, "end_idx": 56, "type": "sym", "entity": "喉痉挛、声门关闭和窒息" } ] }, { "text": "低温对淹溺者的危害亦很大,是导致淹溺死亡的重要原因之一。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "淹溺" } ] }, { "text": "水温过低引起体温下降,低体温则可引起游泳动作失常、肌肉痉挛强直、感觉麻木以及屏气能力下降。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "肌肉" }, { "start_idx": 18, "end_idx": 43, "type": "sym", "entity": "游泳动作失常、肌肉痉挛强直、感觉麻木以及屏气能力下降" } ] }, { "text": "当中枢温度<32℃时,可导致心律失常、低血压甚至心跳停止。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "心律失常" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "低血压" } ] }, { "text": "淹溺经复苏后可考虑作相应辅助检查以了解病情程度和并发症情况。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "淹溺" } ] }, { "text": "最重要的化验是血气分析。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "血气分析" } ] }, { "text": "必须的化验检查还包括全血常规、红细胞比容、血电解质、血糖(低温可引起低血糖或高血糖)、血渗透压、肝肾功能、诊断DIC的各项指标(包括凝血酶原时间、部分凝血活酶时间、血小板计数、血浆纤维蛋白原、纤维蛋白降解产物)、尿常规等。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "ite", "entity": "全血常规" }, { "start_idx": 15, "end_idx": 19, "type": "ite", "entity": "红细胞比容" }, { "start_idx": 21, "end_idx": 24, "type": "ite", "entity": "血电解质" }, { "start_idx": 26, "end_idx": 27, "type": "ite", "entity": "血糖" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "低血糖" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "高血糖" }, { "start_idx": 43, "end_idx": 46, "type": "ite", "entity": "血渗透压" }, { "start_idx": 48, "end_idx": 48, "type": "bod", "entity": "肝" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "肾" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "DIC" }, { "start_idx": 66, "end_idx": 69, "type": "bod", "entity": "凝血酶原" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "凝血活酶" }, { "start_idx": 82, "end_idx": 84, "type": "bod", "entity": "血小板" }, { "start_idx": 88, "end_idx": 94, "type": "bod", "entity": "血浆纤维蛋白原" }, { "start_idx": 96, "end_idx": 99, "type": "bod", "entity": "纤维蛋白" }, { "start_idx": 106, "end_idx": 108, "type": "ite", "entity": "尿常规" } ] }, { "text": "对怀疑存在骨折、颈椎或头颅外伤者,可给予骨骼摄片。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "骨折" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "颈椎" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "头颅" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "颈椎或头颅外伤" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "骨骼摄片" } ] }, { "text": "淹溺9分钟以上和复苏时间超过25分钟者提示预后不良。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "淹溺" } ] }, { "text": "收治ICU指征为:体温低于32℃;昏迷;持续内环境紊乱;需气管插管或CPAP;心血管功能不稳定;症状体征急剧恶化。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "体温低于32℃" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "昏迷" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "持续内环境紊乱" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "气管插管" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "CPAP" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "心血管" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "心血管功能不稳定" }, { "start_idx": 48, "end_idx": 55, "type": "sym", "entity": "症状体征急剧恶化" } ] }, { "text": "对支气管痉挛者可采用支气管舒张剂,如沙丁胺醇溶液雾化吸入。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "支气管" }, { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "痉挛" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "支气管" }, { "start_idx": 18, "end_idx": 23, "type": "dru", "entity": "沙丁胺醇溶液" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "雾化吸入" } ] }, { "text": "碘缺乏病除了较为典型的地方性甲状腺肿、地方性克汀病以外,尚存在大量亚临床患者。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "碘缺乏病" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "地方性甲状腺肿" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "地方性克汀病" } ] }, { "text": "碘缺乏病是当今世界上严重的公共卫生问题之一,全世界约有10亿人生活在缺碘地区。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "碘缺乏病" } ] }, { "text": "在我国的西安、宝鸡、石泉及蓝田等地区,饮水中的碘含量较低,甲状腺肿的发病率也较高。", "entities": [ { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "甲状腺肿" } ] }, { "text": "如碘的缺乏时发生在胚胎脑组织发育的关键时期(从妊娠开始至出生后2岁),则主要影响智力发育,并有身体发育及性发育障碍,即为克汀病。", "entities": [ { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "克汀病" } ] }, { "text": "Ⅱ度:由于甲状腺肿大,脖根明显变粗,大于本人1/3个拳头到相当于2/3个拳头,特点是“脖根粗”。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "甲状腺" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "脖根" } ] }, { "text": "Ⅲ度:颈部失去正常形状,甲状腺大于本人2/3个拳头到相当于一个拳头,特点是“颈变形”。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "颈部" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "甲状腺" } ] }, { "text": "Ⅳ度:甲状腺大于本人一个拳头,多带有结节。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "甲状腺" } ] }, { "text": "根据甲状腺肿中是否有结节,临床上又可分为三型:①弥漫型:甲状腺均匀增大,摸不到结节;②结节型:在甲状腺上摸到一个或几个结节;③混合型:在弥漫肿大的甲状腺上,摸到一个或几个结节。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "甲状腺肿" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "甲状腺" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "甲状腺" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "甲状腺" } ] }, { "text": "甲状腺如肿大明显,可压迫气管引起咳嗽和呼吸困难,压迫食管引起咽下困难,压迫喉返神经引起声音嘶哑,胸骨后甲状腺肿可使头部、颈部、上肢静脉回流受阻,表现为面部青紫、水肿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "甲状腺" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "气管" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "咳嗽" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "咽下困难" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "喉返神经" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 48, "end_idx": 54, "type": "dis", "entity": "胸骨后甲状腺肿" }, { "start_idx": 57, "end_idx": 58, "type": "bod", "entity": "头部" }, { "start_idx": 60, "end_idx": 61, "type": "bod", "entity": "颈部" }, { "start_idx": 63, "end_idx": 66, "type": "bod", "entity": "上肢静脉" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "面部青紫" }, { "start_idx": 80, "end_idx": 81, "type": "sym", "entity": "水肿" } ] }, { "text": "并作如下规定:如有可疑甲状腺功能低下、可疑智力低下或两者均有,只要有其中一项,则考虑为类甲状腺功能减退症。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "甲状腺" }, { "start_idx": 43, "end_idx": 51, "type": "dis", "entity": "类甲状腺功能减退症" } ] }, { "text": "应该广泛切除肿瘤,但一般不可能。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "切除肿瘤" } ] }, { "text": "术前放疗可使肿瘤体积变小,以便手术切除。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "pro", "entity": "术" }, { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "放疗" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肿瘤" }, { "start_idx": 15, "end_idx": 16, "type": "pro", "entity": "手术" } ] }, { "text": "术后局部复发多见,以血行转移为主,至脑、肺、肝和骨骼。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "pro", "entity": "术" }, { "start_idx": 18, "end_idx": 18, "type": "bod", "entity": "脑" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "肺" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肝" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "骨骼" } ] }, { "text": "其发生是由于大脑神经元的异常放电引起。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "bod", "entity": "大脑神经元" } ] }, { "text": "临床上多表现为突然意识丧失,全身骨骼肌群阵挛性或强直性或局限性抽搐,一般经数秒至数分钟后缓解,若惊厥时间超过30分钟或频繁惊厥中间无清醒者,称之为惊厥持续状态。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "突然意识丧失" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "全身骨骼肌群阵挛性" }, { "start_idx": 24, "end_idx": 32, "type": "sym", "entity": "强直性或局限性抽搐" }, { "start_idx": 48, "end_idx": 49, "type": "dis", "entity": "惊厥" }, { "start_idx": 61, "end_idx": 62, "type": "dis", "entity": "惊厥" }, { "start_idx": 73, "end_idx": 74, "type": "dis", "entity": "惊厥" } ] }, { "text": "常有发热与感染伴随症状、颅内压增高或脑实质受损症状。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "发热" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "感染" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "脑实质受损症状" } ] }, { "text": "细菌性脑膜炎、病毒性脑膜炎及病毒性脑炎常急性起病;结核性脑膜炎多亚急性起病,但婴幼儿时期可急性起病,进展迅速,颅神经常常受累;隐球菌脑膜炎慢性起病,头痛明显并逐渐加重;脑寄生虫病特别是脑囊虫病往往以反复惊厥为主要表现。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "细菌性脑膜炎" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "病毒性脑膜炎" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "病毒性脑炎" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "结核性脑膜炎" }, { "start_idx": 55, "end_idx": 61, "type": "sym", "entity": "颅神经常常受累" }, { "start_idx": 63, "end_idx": 68, "type": "dis", "entity": "隐球菌脑膜炎" }, { "start_idx": 74, "end_idx": 82, "type": "sym", "entity": "头痛明显并逐渐加重" }, { "start_idx": 84, "end_idx": 88, "type": "dis", "entity": "脑寄生虫病" }, { "start_idx": 92, "end_idx": 95, "type": "dis", "entity": "脑囊虫病" }, { "start_idx": 101, "end_idx": 102, "type": "dis", "entity": "惊厥" } ] }, { "text": "脑脊液及脑电图等检查异常帮助诊断,特别是脑脊液检查、病原学检测、免疫学及分子生物学检查帮助明确可能的病原。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "脑脊液" }, { "start_idx": 4, "end_idx": 6, "type": "pro", "entity": "脑电图" }, { "start_idx": 20, "end_idx": 24, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 26, "end_idx": 30, "type": "pro", "entity": "病原学检测" }, { "start_idx": 32, "end_idx": 42, "type": "pro", "entity": "免疫学及分子生物学检查" } ] }, { "text": "热性惊厥是指婴幼儿时期发热38℃以上的惊厥,而无中枢神经系统感染、水及电解质紊乱等异常病因所致者。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "热性惊厥" }, { "start_idx": 11, "end_idx": 20, "type": "sym", "entity": "发热38℃以上的惊厥" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "水及电解质紊乱" } ] }, { "text": "目前仍使用1983年全国小儿神经病学专题讨论会诊断标准(自贡会议):好发年龄为4个月~3岁,复发年龄不超过5~6岁;惊厥发作在体温骤升24小时内,发作次数为1次;表现为全身性抽搐,持续时间在10~15分钟内;可伴有呼吸道或消化道等急性感染,热性惊厥也可发生在预防接种后。", "entities": [ { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "惊厥" }, { "start_idx": 84, "end_idx": 88, "type": "sym", "entity": "全身性抽搐" }, { "start_idx": 107, "end_idx": 109, "type": "bod", "entity": "呼吸道" }, { "start_idx": 111, "end_idx": 113, "type": "bod", "entity": "消化道" }, { "start_idx": 107, "end_idx": 118, "type": "sym", "entity": "呼吸道或消化道等急性感染" }, { "start_idx": 120, "end_idx": 123, "type": "dis", "entity": "热性惊厥" } ] }, { "text": "神经系统无异常体征,脑脊液检查无异常,脑电图2周内恢复正常,精神运动发育史正常,多有家族病史。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 19, "end_idx": 21, "type": "pro", "entity": "脑电图" } ] }, { "text": "以上典型发作又称之为单纯性热性惊厥。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "单纯性热性惊厥" } ] }, { "text": "部分高热惊厥临床呈不典型发作表现,称之为复杂性高热惊厥:24小时内反复多次发作;发作惊厥持续时间超过15分钟以上;发作呈局限性,或左右明显不对称。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "高热惊厥" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "复杂性高热惊厥" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "反复多次发作" }, { "start_idx": 40, "end_idx": 55, "type": "sym", "entity": "发作惊厥持续时间超过15分钟以上" }, { "start_idx": 60, "end_idx": 62, "type": "sym", "entity": "局限性" }, { "start_idx": 65, "end_idx": 71, "type": "sym", "entity": "左右明显不对称" } ] }, { "text": "惊厥的发生为脑缺氧、缺血、水肿或细菌毒素直接作用等多因素所致。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "惊厥" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "脑缺氧" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "缺血" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "水肿" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "细菌毒素" } ] }, { "text": "其他还有颅内出血(产伤、窒息、外伤或维生素缺乏史),颅脑损伤(外伤史),脑血管畸形,颅内肿瘤,脑发育异常(脑积水、颅脑畸形),神经皮肤综合征,脑炎后遗症及脑水肿等。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "颅内出血" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "产伤" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "窒息" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "外伤" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "维生素缺乏" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "颅脑损伤" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "脑血管畸形" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "颅内" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "颅内肿瘤" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "脑" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "脑发育异常" }, { "start_idx": 53, "end_idx": 55, "type": "sym", "entity": "脑积水" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "颅脑畸形" }, { "start_idx": 63, "end_idx": 69, "type": "dis", "entity": "神经皮肤综合征" }, { "start_idx": 71, "end_idx": 75, "type": "dis", "entity": "脑炎后遗症" }, { "start_idx": 77, "end_idx": 79, "type": "dis", "entity": "脑水肿" } ] }, { "text": "积极治疗颅内感染;纠正代谢失常;对复杂性热性惊厥可预防性用药,每日口服苯巴比妥3mg/kg,或口服丙戊酸钠每日20~40mg/kg,疗程数月至1~2年,以免复发;对于癫痫患者强调规范用药。", "entities": [ { "start_idx": 35, "end_idx": 38, "type": "dru", "entity": "苯巴比妥" }, { "start_idx": 49, "end_idx": 52, "type": "dru", "entity": "丙戊酸钠" } ] }, { "text": "呈严重的慢性进行性贫血,需靠输血维持生命,血红蛋白常<30g/L;特殊面容,表现为头大,颧骨略高,鼻梁低陷,眼距增宽,表情呆钝;肝、脾日渐增大,以脾大为主,可达盆腔。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "贫血" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "输血" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "头大" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "颧骨" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "颧骨略高" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "鼻梁" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "鼻梁低陷" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "眼距增宽" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "表情呆钝" }, { "start_idx": 64, "end_idx": 64, "type": "bod", "entity": "肝" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "脾" }, { "start_idx": 64, "end_idx": 70, "type": "sym", "entity": "肝、脾日渐增大" }, { "start_idx": 73, "end_idx": 73, "type": "bod", "entity": "脾" }, { "start_idx": 72, "end_idx": 76, "type": "sym", "entity": "以脾大为主" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "盆腔" } ] }, { "text": "主要见于新生儿和小婴儿,常有以下诱因:①广谱抗生素的大量应用或联合应用;②医源性因素如气管插管、血管插管、人工呼吸机等的应用;③先天性或获得性免疫功能缺陷,如营养不良、白血病、恶性淋巴瘤、长期使用皮质激素或免疫抑制剂等。", "entities": [ { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "气管插管" }, { "start_idx": 48, "end_idx": 51, "type": "pro", "entity": "血管插管" }, { "start_idx": 53, "end_idx": 57, "type": "equ", "entity": "人工呼吸机" }, { "start_idx": 79, "end_idx": 82, "type": "dis", "entity": "营养不良" }, { "start_idx": 84, "end_idx": 86, "type": "dis", "entity": "白血病" }, { "start_idx": 88, "end_idx": 92, "type": "dis", "entity": "恶性淋巴瘤" }, { "start_idx": 98, "end_idx": 101, "type": "dru", "entity": "皮质激素" }, { "start_idx": 103, "end_idx": 107, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "如致病菌株产生超广谱β-内酰胺酶(ESBL),应选用头孢霉素类、复合β-内酰胺类,严重者选用碳青霉烯类抗生素如亚胺培南。", "entities": [ { "start_idx": 7, "end_idx": 15, "type": "mic", "entity": "超广谱β-内酰胺酶" }, { "start_idx": 17, "end_idx": 20, "type": "mic", "entity": "ESBL" }, { "start_idx": 26, "end_idx": 30, "type": "dru", "entity": "头孢霉素类" }, { "start_idx": 32, "end_idx": 39, "type": "dru", "entity": "复合β-内酰胺类" }, { "start_idx": 46, "end_idx": 53, "type": "dru", "entity": "碳青霉烯类抗生素" }, { "start_idx": 55, "end_idx": 58, "type": "dru", "entity": "亚胺培南" } ] }, { "text": "此药可引起锥体外系不良反应,表现为动作减少、减慢和肌张力增强,有伸舌、张口困难、歪颈等,加用等量苯海索(安坦)可抵消这种不良反应。", "entities": [ { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "动作减少" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "减慢" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "肌张力增强" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "伸舌" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "张口困难" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "歪颈" }, { "start_idx": 48, "end_idx": 50, "type": "dru", "entity": "苯海索" } ] }, { "text": "其他三环抗抑郁剂(丙米嗪)和可乐定也用于抽动-秽语综合征伴发注意缺陷伴多动症的治疗,特别是可乐定被视为中枢兴奋剂的一个安全替代药物。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dru", "entity": "三环抗抑郁剂" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "丙米嗪" }, { "start_idx": 14, "end_idx": 16, "type": "dru", "entity": "可乐定" }, { "start_idx": 45, "end_idx": 47, "type": "dru", "entity": "可乐定" }, { "start_idx": 51, "end_idx": 55, "type": "dru", "entity": "中枢兴奋剂" } ] }, { "text": "其他如氯米帕明(氯丙米嗪,起始剂量25mg,睡前服1次)和氟伏沙明治疗抽动-秽语综合征伴发强迫观念和行为有效的报道。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "氯米帕明" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "氟伏沙明" } ] }, { "text": "不同种族CAH的发病率有很大差别。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "CAH" } ] }, { "text": "21-羟化酶缺乏症杂合子发生率约1/60。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "21-羟化酶缺乏症" } ] }, { "text": "犹太人中CAH发生率较高,达1/21。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "CAH" } ] }, { "text": "出生时外生殖器似正常,少数有轻度的阴茎增大,阴囊色素沉着。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "外生殖器" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "阴茎增大" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "阴囊色素沉着" } ] }, { "text": "这些患儿随着年龄增大,往往2岁后出现明显的雄激素过多的体征,阴茎粗大,但由于雄激素增高并非促性腺激素分泌增加所致,故睾丸并无增大,这与真性性早熟完全不同,后者伴睾丸明显发育。", "entities": [ { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "雄激素过多" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "阴茎粗大" }, { "start_idx": 38, "end_idx": 42, "type": "sym", "entity": "雄激素增高" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "促性腺激素" }, { "start_idx": 58, "end_idx": 63, "type": "sym", "entity": "睾丸并无增大" }, { "start_idx": 67, "end_idx": 71, "type": "sym", "entity": "真性性早熟" }, { "start_idx": 80, "end_idx": 85, "type": "sym", "entity": "睾丸明显发育" } ] }, { "text": "女性可表现为阴蒂肥大,伴或不伴阴唇融合,严重者阴唇完全融合似阴囊,阴蒂肥大似阴茎,尿道开口于肥大的阴蒂下(似尿道下裂),外观似男性外生殖器但未能触及睾丸,而内生殖器仍为女性。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "阴蒂肥大" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "伴或不伴阴唇融合" }, { "start_idx": 23, "end_idx": 31, "type": "sym", "entity": "阴唇完全融合似阴囊" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "阴蒂肥大似阴茎" }, { "start_idx": 41, "end_idx": 51, "type": "sym", "entity": "尿道开口于肥大的阴蒂下" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "似尿道下裂" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "外生殖器" }, { "start_idx": 74, "end_idx": 75, "type": "bod", "entity": "睾丸" }, { "start_idx": 78, "end_idx": 81, "type": "bod", "entity": "内生殖器" } ] }, { "text": "患者多在生后1~4周出现失盐症状,又由于同时伴有皮质醇合成障碍,往往出现不同程度的肾上腺皮质功能不足表现,如呕吐、腹泻、脱水和严重的代谢性酸中毒,难以纠正的低血钠和高血钾症,如不及时诊治则导致血容量降低、血压下降及休克,循环功能衰竭。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "失盐" }, { "start_idx": 24, "end_idx": 30, "type": "sym", "entity": "皮质醇合成障碍" }, { "start_idx": 41, "end_idx": 49, "type": "sym", "entity": "肾上腺皮质功能不足" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "呕吐" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "腹泻" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "脱水" }, { "start_idx": 66, "end_idx": 71, "type": "sym", "entity": "代谢性酸中毒" }, { "start_idx": 78, "end_idx": 80, "type": "sym", "entity": "低血钠" }, { "start_idx": 82, "end_idx": 85, "type": "sym", "entity": "高血钾症" }, { "start_idx": 96, "end_idx": 100, "type": "sym", "entity": "血容量降低" }, { "start_idx": 102, "end_idx": 105, "type": "sym", "entity": "血压下降" }, { "start_idx": 107, "end_idx": 108, "type": "sym", "entity": "休克" }, { "start_idx": 110, "end_idx": 115, "type": "sym", "entity": "循环功能衰竭" } ] }, { "text": "男孩可有性早熟、生长加速及骨龄超前。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "性早熟" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "生长加速" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "骨龄超前" } ] }, { "text": "因11β-OH缺乏而导致DOC增加,可使部分患儿出现高血钠、低血钾、碱中毒及高血容量,导致高血压症状;又因皮质醇合成减少引起肾上腺雄激素水平增高,出现类似21-羟化酶缺乏的高雄激素症状和体征。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "sym", "entity": "11β-OH缺乏" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "DOC" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "高血钠" }, { "start_idx": 30, "end_idx": 32, "type": "sym", "entity": "低血钾" }, { "start_idx": 34, "end_idx": 36, "type": "sym", "entity": "碱中毒" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "高血容量" }, { "start_idx": 45, "end_idx": 47, "type": "sym", "entity": "高血压" }, { "start_idx": 53, "end_idx": 59, "type": "sym", "entity": "皮质醇合成减少" }, { "start_idx": 62, "end_idx": 71, "type": "sym", "entity": "肾上腺雄激素水平增高" }, { "start_idx": 77, "end_idx": 84, "type": "dis", "entity": "21-羟化酶缺乏" }, { "start_idx": 86, "end_idx": 89, "type": "sym", "entity": "高雄激素" } ] }, { "text": "因CAH是常染色体隐性遗传病,每生育一胎就有1/4概率为CAH患者,因此,对家族中有本病先证者的孕妇要在妊娠中期抽取羊水或者早期取绒毛膜抽提DNA,进行产前基因分析和诊断。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "CAH" }, { "start_idx": 5, "end_idx": 13, "type": "dis", "entity": "常染色体隐性遗传病" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "CAH" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "羊水" }, { "start_idx": 65, "end_idx": 67, "type": "bod", "entity": "绒毛膜" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "DNA" }, { "start_idx": 76, "end_idx": 81, "type": "pro", "entity": "产前基因分析" } ] }, { "text": "以张口呼吸、持续鼻塞和流涕为特征。", "entities": [ { "start_idx": 1, "end_idx": 12, "type": "sym", "entity": "张口呼吸、持续鼻塞和流涕" } ] }, { "text": "张口呼吸以平卧时明显,睡眠时发出鼾声,严重者白天亦出现张口呼吸,口腔黏膜干燥。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "sym", "entity": "睡眠时发出鼾声" }, { "start_idx": 22, "end_idx": 30, "type": "sym", "entity": "白天亦出现张口呼吸" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "口腔黏膜" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "口腔黏膜干燥" } ] }, { "text": "由于长期张口呼吸,致使面骨发育障碍,上颌骨变长,腭骨高,牙列不齐,上切牙突出,缺乏表情,出现所谓“腺样体”面容。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "张口呼吸" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "面骨发育障碍" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "上颌骨" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "上颌骨变长" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "腭骨" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "腭骨高" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "牙列不齐" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "上切牙" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "上切牙突出" }, { "start_idx": 39, "end_idx": 42, "type": "sym", "entity": "缺乏表情" }, { "start_idx": 49, "end_idx": 51, "type": "bod", "entity": "腺样体" } ] }, { "text": "分泌物向下流并刺激呼吸道黏膜,常引起阵咳,易并发下呼吸道感染;由于咽鼓管咽口受阻,易并发非化脓性中耳炎,导致听力减退和耳鸣。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "bod", "entity": "呼吸道黏膜" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "阵咳" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "下呼吸道感染" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "咽鼓管咽口" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "咽鼓管咽口受阻" }, { "start_idx": 44, "end_idx": 50, "type": "dis", "entity": "非化脓性中耳炎" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "听力减退和耳鸣" } ] }, { "text": "少数严重患儿张口呼吸困难,可出现三凹征、阻塞性呼吸暂停,甚至肺动脉高压。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "张口呼吸困难" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "三凹征" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "阻塞性呼吸暂停" }, { "start_idx": 30, "end_idx": 34, "type": "dis", "entity": "肺动脉高压" } ] }, { "text": "全身发育和营养状况较差,并有夜惊,遗尿、反应迟钝、注意力不集中等神经症状。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "全身发育和营养状况较差" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "夜惊" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "遗尿" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "反应迟钝" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "注意力不集中" } ] }, { "text": "患儿张口呼吸、睡眠鼾声、持续鼻塞和反复鼻窦炎应怀疑本病。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "张口呼吸" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "睡眠鼾声" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "持续鼻塞" }, { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "鼻窦炎" } ] }, { "text": "通过前鼻镜或纤维鼻咽镜检查,可发现鼻咽顶部和后壁表面纵行裂隙的分叶状淋巴组织。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "前鼻镜或纤维鼻咽镜检查" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "鼻咽顶部" }, { "start_idx": 22, "end_idx": 37, "type": "bod", "entity": "后壁表面纵行裂隙的分叶状淋巴组织" } ] }, { "text": "但同时具有以上三方面症状的典型患者不多,尤其2岁以下婴儿往往以全身感染症状为主,仅少数患儿有栓塞症状和(或)心脏杂音。", "entities": [ { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "全身感染" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "栓塞" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "心脏杂音" } ] }, { "text": "内脏栓塞可出现脾大、腹痛、血尿、便血,有时脾大很显著;肺栓塞可出现胸痛、咳嗽、咯血、肺部啰音等;脑动脉栓塞则有头痛、呕吐、偏瘫、失语、抽搐甚至昏迷等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "内脏栓塞" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "脾大" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "腹痛" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "便血" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "脾大" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "肺栓塞" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "胸痛" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "咳嗽" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "咯血" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "肺部啰音" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "脑动脉栓塞" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "头痛" }, { "start_idx": 58, "end_idx": 59, "type": "sym", "entity": "呕吐" }, { "start_idx": 61, "end_idx": 62, "type": "sym", "entity": "偏瘫" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "失语" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "抽搐" }, { "start_idx": 71, "end_idx": 72, "type": "sym", "entity": "昏迷" } ] }, { "text": "治疗不满意或对青霉素过敏者选用头孢菌素类或万古霉素:40~60mg/(kg•d),分2~3次静脉滴注,疗程6~8周。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "青霉素" }, { "start_idx": 15, "end_idx": 19, "type": "dru", "entity": "头孢菌素类" }, { "start_idx": 21, "end_idx": 24, "type": "dru", "entity": "万古霉素" } ] }, { "text": "③革兰阴性杆菌或大肠杆菌:选用氨苄西林300mg/(kg•d),分4次,每6小时1次静脉滴注,疗程4~6周,或用头孢氧哌唑或头孢噻肟三嗪200mg/(kg•d),分4次,每6小时1次静脉滴注,疗程4~6周,加用庆大霉素2周。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "mic", "entity": "革兰阴性杆菌" }, { "start_idx": 8, "end_idx": 11, "type": "mic", "entity": "大肠杆菌" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 56, "end_idx": 60, "type": "dru", "entity": "头孢氧哌唑" }, { "start_idx": 62, "end_idx": 67, "type": "dru", "entity": "头孢噻肟三嗪" }, { "start_idx": 105, "end_idx": 108, "type": "dru", "entity": "庆大霉素" } ] }, { "text": "绿脓杆菌感染可加用羟苄青霉素200~400mg/(kg•d),分4次,每6小时1次静脉滴注。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "绿脓杆菌感染" }, { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "羟苄青霉素" } ] }, { "text": "约有半数患儿可发生各种并发症如充血性心力衰竭、脑栓塞、肺栓塞、心脏瓣膜破坏、腱索断裂、动脉瘤形成等,残留严重瓣膜损伤者,需进行瓣膜修复或置换术。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "脑栓塞" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "肺栓塞" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "心脏瓣膜破坏" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "腱索断裂" }, { "start_idx": 43, "end_idx": 47, "type": "sym", "entity": "动脉瘤形成" }, { "start_idx": 63, "end_idx": 66, "type": "pro", "entity": "瓣膜修复" }, { "start_idx": 68, "end_idx": 70, "type": "pro", "entity": "置换术" } ] }, { "text": "5.建立哮喘发作时的计划。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "哮喘" } ] }, { "text": "病变多局限于脏层胸膜,胸膜面粗糙而无光泽,一般无渗出液或很少渗出液,迅速吸收后留存纤维素层,形成粘连,可能逐渐吸收。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "脏层" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胸膜" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "胸膜" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "粗糙而无光泽" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "一般无渗出液" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "很少渗出液" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "形成粘连" } ] }, { "text": "主要症状为胸痛,可牵涉到腹部、肩部和背部。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "胸痛" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "腹部" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肩部" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "背部" } ] }, { "text": "患儿喜患侧卧位,患侧呼吸运动受限制、听诊呼吸音减弱。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "喜患侧卧位" }, { "start_idx": 8, "end_idx": 16, "type": "sym", "entity": "患侧呼吸运动受限制" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "听诊呼吸音减弱" } ] }, { "text": "腹痛明显者,尚须排除急性肠系膜淋巴结炎、阑尾炎。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "腹痛" }, { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "急性肠系膜淋巴结炎" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "阑尾炎" } ] }, { "text": "可适当给镇痛剂止痛。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "镇痛剂" } ] }, { "text": "环境因素对尿道发育形成影响也主要是通过母亲和胎儿的内分泌系统。", "entities": [ { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "内分泌系统" } ] }, { "text": "本病的狭窄程度决定临床严重性;大多患儿无症状,胸骨左缘上部有一喷射性收缩期杂音,并向腋下及背面传导;如伴有收缩早期喀喇音,提示有肺动脉瓣狭窄同时存在,但肺动脉瓣关闭音可增强,提示瓣膜狭窄后还有梗阻存在。", "entities": [ { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "胸骨左缘上部" }, { "start_idx": 23, "end_idx": 38, "type": "sym", "entity": "胸骨左缘上部有一喷射性收缩期杂音" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "腋下" }, { "start_idx": 41, "end_idx": 48, "type": "sym", "entity": "向腋下及背面传导" }, { "start_idx": 53, "end_idx": 59, "type": "sym", "entity": "收缩早期喀喇音" }, { "start_idx": 64, "end_idx": 69, "type": "dis", "entity": "肺动脉瓣狭窄" }, { "start_idx": 76, "end_idx": 79, "type": "bod", "entity": "肺动脉瓣" }, { "start_idx": 89, "end_idx": 92, "type": "dis", "entity": "瓣膜狭窄" }, { "start_idx": 96, "end_idx": 97, "type": "dis", "entity": "梗阻" } ] }, { "text": "胸部X线上多属正常,但有时可见某侧或某段肺野血管影特少;心电图上右心室肥厚的程度可反映狭窄的严重性;二维超声可显示肺动脉主干及其近支的解剖。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "胸部X线" }, { "start_idx": 20, "end_idx": 24, "type": "pro", "entity": "肺野血管影" }, { "start_idx": 28, "end_idx": 30, "type": "pro", "entity": "心电图" }, { "start_idx": 32, "end_idx": 34, "type": "bod", "entity": "右心室" }, { "start_idx": 50, "end_idx": 53, "type": "pro", "entity": "二维超声" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "肺动脉" } ] }, { "text": "治疗可用球囊导管予以扩张,支架的应用可防止扩张后再狭窄,局部的严重狭窄可以进行手术治疗。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "equ", "entity": "球囊导管" }, { "start_idx": 13, "end_idx": 14, "type": "equ", "entity": "支架" }, { "start_idx": 39, "end_idx": 42, "type": "pro", "entity": "手术治疗" } ] }, { "text": "主要症状为咳嗽伴气急。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "咳嗽伴气急" } ] }, { "text": "当血清胆红素超过生理性黄疸的水平,临床诊断为高胆红素血症(高胆)。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "血清胆红素" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "生理性黄疸" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "高胆红素血症" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "高胆" } ] }, { "text": "分为早发型(母乳喂养性黄疸)和晚发型(母乳性黄疸)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "早发型" }, { "start_idx": 6, "end_idx": 12, "type": "dis", "entity": "母乳喂养性黄疸" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "晚发型" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "母乳性黄疸" } ] }, { "text": "喂奶最好在每天10次以上,血清胆红素达到光疗指征时可光疗。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "bod", "entity": "血清胆红素" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "光疗" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "光疗" } ] }, { "text": "主要有新生儿肝炎和胆道闭锁。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "新生儿肝炎" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "胆道闭锁" } ] }, { "text": "3个月后可逐渐发展至肝硬化。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "肝硬化" } ] }, { "text": "如感染伴有溶血,则可出现贫血。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "溶血" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "贫血" } ] }, { "text": "治疗主要是积极控制感染,加强支持疗法。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "pro", "entity": "积极控制感染" }, { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "加强支持疗法" } ] }, { "text": "其临床表现多种多样,以血尿最为常见。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "血尿" } ] }, { "text": "IgA肾病可分为原发性和继发性两种类型,后者常继发于肝硬化、肠道疾病、关节炎以及疱疹性皮炎等疾病,也以肾小球系膜区显著的IgA沉积为特点。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "肝硬化" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "肠道疾病" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "关节炎" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "疱疹性皮炎" }, { "start_idx": 51, "end_idx": 64, "type": "sym", "entity": "肾小球系膜区显著的IgA沉积" } ] }, { "text": "原发性IgA肾病在世界许多地方被认为是一种最常见的肾小球肾炎,而且是导致终末期肾衰的常见原因之一。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "原发性IgA肾病" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "肾小球肾炎" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "终末期肾衰" } ] }, { "text": "另一种形式的IgA称为分泌型IgA(SIgA),存在于人的外分泌物中,如唾液、眼泪、肠内分泌物以及初乳中。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "IgA" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "分泌型IgA" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "SIgA" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "外分泌物" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "唾液" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "眼泪" }, { "start_idx": 42, "end_idx": 46, "type": "bod", "entity": "肠内分泌物" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "初乳" } ] }, { "text": "IgA分子结构的这些特性在IgA肾病的发生上有重要意义。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "IgA" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "近年报道,肾小球基底膜超微结构也有变化,10%左右的IgA肾病有基底膜变薄,究竟是合并薄基底膜病还是属于IgA肾病的继发改变尚不清楚。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "bod", "entity": "肾小球基底膜" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "基底膜变薄" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "合并薄基底膜病" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "临床表现多样化,从仅有镜下血尿到肾病综合征,均可为起病时的表现,各临床表现型间也可在病程中相互转变,但在病程中其临床表现可相互转变。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "镜下血尿" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "肾病综合征" } ] }, { "text": "80%的儿童IgA肾病以肉眼血尿为首发症状,北美及欧洲的发生率高于亚洲,常和上呼吸道感染有关(Berger病);与上呼吸道感染间隔很短时间(24~72小时),偶可数小时后即出现血尿。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "肉眼血尿" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 47, "end_idx": 53, "type": "dis", "entity": "Berger病" }, { "start_idx": 57, "end_idx": 62, "type": "dis", "entity": "上呼吸道感染" }, { "start_idx": 88, "end_idx": 89, "type": "sym", "entity": "血尿" } ] }, { "text": "且多存在扁桃体肿大,扁桃体切除后多数患者肉眼血尿停止发作。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "扁桃体肿大" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "扁桃体切除" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "肉眼血尿" } ] }, { "text": "以肾病综合征为表现的IgA肾病约占15%~30%,三高一低表现突出,起病前也往往很少合并呼吸道感染。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "肾病综合征" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "呼吸道感染" } ] }, { "text": "亦有部分病例表现为肾炎综合征,除血尿外,还有高血压及肾功能不全。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "肾炎综合征" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "血尿" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "高血压" }, { "start_idx": 26, "end_idx": 28, "type": "sym", "entity": "肾功能" } ] }, { "text": "高血压好发于年龄偏大者,成人占20%,儿童仅5%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "高血压" } ] }, { "text": "不足5%的IgA肾病患者表现为急进性肾炎。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "急进性肾炎" } ] }, { "text": "Ⅴ级:与Ⅳ级相似但更严重,节段和/或球性硬化、玻璃样变以及球囊粘连,50%以上的肾小球有新月体,称之为弥漫硬化性肾小球肾炎。", "entities": [ { "start_idx": 13, "end_idx": 32, "type": "sym", "entity": "节段和/或球性硬化、玻璃样变以及球囊粘连" }, { "start_idx": 34, "end_idx": 46, "type": "sym", "entity": "50%以上的肾小球有新月体" }, { "start_idx": 51, "end_idx": 60, "type": "dis", "entity": "弥漫硬化性肾小球肾炎" } ] }, { "text": "IgA肾病从病理变化到临床表现都有很大差异,预后也有很大区别,因此,治疗措施必须做到个体化。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "IgA肾病" } ] }, { "text": "短期抗生素治疗对于控制急性期症状也有一定作用。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "pro", "entity": "抗生素治疗" } ] }, { "text": "对于合并水肿、高血压的患儿,应相应给予利尿消肿,降压药物治疗,并采用低盐、低蛋白饮食。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "水肿" }, { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "高血压" }, { "start_idx": 19, "end_idx": 22, "type": "pro", "entity": "利尿消肿" }, { "start_idx": 24, "end_idx": 29, "type": "pro", "entity": "降压药物治疗" } ] }, { "text": "日本曾作全国范围多中心对照研究,采用泼尼松及免疫抑制治疗IgA肾病的患儿,其远期肾功能不全的比例要明显低于使用一般性治疗的患儿。", "entities": [ { "start_idx": 18, "end_idx": 20, "type": "dru", "entity": "泼尼松" }, { "start_idx": 22, "end_idx": 27, "type": "pro", "entity": "免疫抑制治疗" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "肾功能不全" } ] }, { "text": "最近,Nagaoka等报道一种新型免疫抑制剂——咪唑立宾(mizoribine),用于儿童IgA肾病治疗,该药安全、易耐受,可长期服用,并能显著减少蛋白尿和血尿程度,重复肾活体组织检查证实肾组织病变程度减轻。", "entities": [ { "start_idx": 17, "end_idx": 21, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "咪唑立宾" }, { "start_idx": 29, "end_idx": 38, "type": "dru", "entity": "mizoribine" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "儿童IgA肾病" }, { "start_idx": 74, "end_idx": 76, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 78, "end_idx": 79, "type": "sym", "entity": "血尿" }, { "start_idx": 85, "end_idx": 91, "type": "pro", "entity": "肾活体组织检查" }, { "start_idx": 94, "end_idx": 98, "type": "sym", "entity": "肾组织病变" } ] }, { "text": "中医中药治疗IgA肾病也有一定疗效,对于中等程度的蛋白尿,使用雷公藤多甙片1mg/(kg•d)治疗3个月,可获明显疗效。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dru", "entity": "中药" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 25, "end_idx": 27, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 31, "end_idx": 36, "type": "dru", "entity": "雷公藤多甙片" } ] }, { "text": "临床主要特征是丑陋面容、骨骼异常及运动受限、肝脾肿大和智能低下。", "entities": [ { "start_idx": 7, "end_idx": 30, "type": "sym", "entity": "丑陋面容、骨骼异常及运动受限、肝脾肿大和智能低下" } ] }, { "text": "目前各型黏多糖病均发现有基因突变,包括无义突变、错义突变、剪接位点突变和缺失或插入。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "黏多糖病" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "基因突变" }, { "start_idx": 19, "end_idx": 40, "type": "sym", "entity": "无义突变、错义突变、剪接位点突变和缺失或插入" } ] }, { "text": "由于各型病情轻重不一,又有各自的临床特征,在诊断中需鉴别,主要表现为身材矮小和特殊面容,表情淡漠,头大、面部丑陋,眼裂小,眼距宽,鼻梁低平,鼻孔大,唇厚,前额和双颧突出,毛发多而发际低,颈短,大部分有角膜混浊。", "entities": [ { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "眼" }, { "start_idx": 65, "end_idx": 66, "type": "bod", "entity": "鼻梁" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "鼻孔" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "前额" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "双颧" }, { "start_idx": 85, "end_idx": 86, "type": "bod", "entity": "毛发" }, { "start_idx": 34, "end_idx": 103, "type": "sym", "entity": "身材矮小和特殊面容,表情淡漠,头大、面部丑陋,眼裂小,眼距宽,鼻梁低平,鼻孔大,唇厚,前额和双颧突出,毛发多而发际低,颈短,大部分有角膜混浊" } ] }, { "text": "临床有智能低下,面容丑陋,肝脾肿大,骨骼病变,心血管病变,角膜混浊和耳聋。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "肝脾" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "骨骼" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "心血管" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "角膜" }, { "start_idx": 3, "end_idx": 35, "type": "sym", "entity": "智能低下,面容丑陋,肝脾肿大,骨骼病变,心血管病变,角膜混浊和耳聋" } ] }, { "text": "ⅢA型为乙酰肝素-N-硫酸酯酶缺乏,ⅢB型为α-N-乙酰氨基葡萄糖苷酶缺乏,ⅢC型为α-氨基葡萄糖乙酰转移酶缺乏,ⅢD型为N-乙酰氨基葡萄糖硫酸酯酶缺乏。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "ⅢA型" }, { "start_idx": 4, "end_idx": 16, "type": "sym", "entity": "乙酰肝素-N-硫酸酯酶缺乏" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "ⅢB型" }, { "start_idx": 22, "end_idx": 36, "type": "sym", "entity": "α-N-乙酰氨基葡萄糖苷酶缺乏" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "ⅢC型" }, { "start_idx": 42, "end_idx": 55, "type": "sym", "entity": "α-氨基葡萄糖乙酰转移酶缺乏" }, { "start_idx": 57, "end_idx": 59, "type": "dis", "entity": "ⅢD型" }, { "start_idx": 61, "end_idx": 75, "type": "sym", "entity": "N-乙酰氨基葡萄糖硫酸酯酶缺乏" } ] }, { "text": "临床上患儿在1岁内发育尚正常,以后逐渐出现语言、行为障碍,生长发育落后,在儿童期神经系统退行性病变较明显,有肝脾肿大,疝气,面容粗陋,关节强直等。", "entities": [ { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "肝脾" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "关节" }, { "start_idx": 3, "end_idx": 71, "type": "sym", "entity": "患儿在1岁内发育尚正常,以后逐渐出现语言、行为障碍,生长发育落后,在儿童期神经系统退行性病变较明显,有肝脾肿大,疝气,面容粗陋,关节强直等" } ] }, { "text": "有明显的生长障碍,骨骼畸形,X线呈典型的黏多糖病表现,脊椎有鸟嘴样突出改变,椎骨扁平,飘带肋骨,鸡胸等,面容丑陋,鼻矮、口大、牙齿发育不良,角膜混浊。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "sym", "entity": "明显的生长障碍" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "骨骼畸形" }, { "start_idx": 14, "end_idx": 15, "type": "pro", "entity": "X线" }, { "start_idx": 27, "end_idx": 36, "type": "sym", "entity": "脊椎有鸟嘴样突出改变" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "椎骨扁平" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "飘带肋骨" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "鸡胸" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "面容丑陋" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "鼻矮" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "口大" }, { "start_idx": 63, "end_idx": 68, "type": "sym", "entity": "牙齿发育不良" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "角膜混浊" } ] }, { "text": "青春发育可正常,随年龄增长出现脊髓压迫症状,晚期出现压迫性截瘫和呼吸麻痹。", "entities": [ { "start_idx": 15, "end_idx": 20, "type": "sym", "entity": "脊髓压迫症状" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "压迫性截瘫" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "呼吸麻痹" } ] }, { "text": "黏多糖病Ⅳ型有两种亚型,黏多糖病Ⅳ型A为半乳糖胺-6-硫酸酯酶缺乏,黏多糖病Ⅳ型B为β-半乳糖苷酶缺乏,导致硫酸角质素和硫酸软骨素降解障碍,导致这些物质在细胞与组织中积聚。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "黏多糖病Ⅳ型" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "黏多糖病Ⅳ型A" }, { "start_idx": 20, "end_idx": 32, "type": "sym", "entity": "半乳糖胺-6-硫酸酯酶缺乏" }, { "start_idx": 34, "end_idx": 40, "type": "dis", "entity": "黏多糖病Ⅳ型B" }, { "start_idx": 42, "end_idx": 50, "type": "sym", "entity": "β-半乳糖苷酶缺乏" }, { "start_idx": 54, "end_idx": 58, "type": "bod", "entity": "硫酸角质素" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "硫酸软骨素" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "细胞" }, { "start_idx": 80, "end_idx": 81, "type": "bod", "entity": "组织" } ] }, { "text": "最近基因工程生产的重组特异性酶的问世为黏多糖病的治疗带来了希望,MPSⅠ型、MPSⅡ型以及MPSⅥ型已有药物上市,酶的替代治疗取得了较好的效果。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "重组特异性酶" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "黏多糖病" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "MPSⅠ型" }, { "start_idx": 38, "end_idx": 42, "type": "dis", "entity": "MPSⅡ型" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "MPSⅥ型" }, { "start_idx": 57, "end_idx": 57, "type": "bod", "entity": "酶" } ] }, { "text": "①上消化道梗阻:食管气管瘘、食管闭锁、食管裂孔疝、胃扭转、幽门肥厚性狭窄、环状胰腺、先天性膈疝等;②下消化道梗阻:如肠旋转不良、小肠重复畸形、肠狭窄、肠闭锁、先天性巨结肠、肛门闭锁等。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "上消化道梗阻" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "食管气管瘘" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "食管闭锁" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "食管裂孔疝" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "胃扭转" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "幽门肥厚性狭窄" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "环状胰腺" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "先天性膈疝" }, { "start_idx": 50, "end_idx": 55, "type": "dis", "entity": "下消化道梗阻" }, { "start_idx": 58, "end_idx": 62, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 64, "end_idx": 69, "type": "dis", "entity": "小肠重复畸形" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "肠狭窄" }, { "start_idx": 75, "end_idx": 77, "type": "dis", "entity": "肠闭锁" }, { "start_idx": 79, "end_idx": 84, "type": "dis", "entity": "先天性巨结肠" }, { "start_idx": 86, "end_idx": 89, "type": "dis", "entity": "肛门闭锁" } ] }, { "text": "喂养不当呕吐时,新生儿一般情况较好,改进喂养方法后呕吐可停止。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "呕吐" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "呕吐" } ] }, { "text": "许多患儿无临床呕吐表现,而发生呼吸暂停、心动过缓、反复吸入甚至猝死。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "呕吐" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "心动过缓" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "反复吸入" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "猝死" } ] }, { "text": "如生后数天排便很少,或胎粪排空时间延迟,患儿可出现呕吐,呕吐物为黄绿色,常伴有腹胀,腹壁可见肠型,用生理盐水灌肠排出胎粪后,呕吐即可缓解。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "呕吐" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "呕吐" } ] }, { "text": "有些患儿出现类似螃蟹吐泡沫,插胃管时胃管受阻折返。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "equ", "entity": "胃管" }, { "start_idx": 18, "end_idx": 19, "type": "equ", "entity": "胃管" } ] }, { "text": "对吞咽功能不全、食管气管瘘可行碘油造影。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "碘油造影" } ] }, { "text": "对幽门肥厚性狭窄,可做腹部超声检查。", "entities": [ { "start_idx": 1, "end_idx": 7, "type": "dis", "entity": "幽门肥厚性狭窄" }, { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "腹部超声检查" } ] }, { "text": "对肠道炎症、感染、低位肠梗阻,可摄腹部X线平片。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "肠道炎症" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "感染" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "低位肠梗阻" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "腹部X线平片" } ] }, { "text": "对结肠疾病如先天性巨结肠,可做钡剂灌肠造影检查。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "结肠" }, { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "先天性巨结肠" }, { "start_idx": 15, "end_idx": 22, "type": "pro", "entity": "钡剂灌肠造影检查" } ] }, { "text": "外周血白细胞计数大多正常。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "ite", "entity": "外周血白细胞计数" } ] }, { "text": "治疗应采用兼治原型和L型菌的抗生素,如氨苄西林或头孢霉素类加大环内酯类。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "兼治原型" }, { "start_idx": 10, "end_idx": 16, "type": "dru", "entity": "L型菌的抗生素" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "头孢霉素" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "环内酯类" } ] }, { "text": "在以免疫复合物介导的疾病中,补体下降往往提示疾病活动,免疫复合物介导的肾炎尤其如此,如狼疮肾炎、血清病、链球菌感染后肾炎、冷球蛋白血症及部分与风湿性疾病相关的肾炎(如多动脉炎肾炎)。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "免疫复合物介导的疾病" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "补体下降" }, { "start_idx": 27, "end_idx": 36, "type": "dis", "entity": "免疫复合物介导的肾炎" }, { "start_idx": 43, "end_idx": 46, "type": "dis", "entity": "狼疮肾炎" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "血清病" }, { "start_idx": 52, "end_idx": 59, "type": "dis", "entity": "链球菌感染后肾炎" }, { "start_idx": 61, "end_idx": 66, "type": "dis", "entity": "冷球蛋白血症" }, { "start_idx": 71, "end_idx": 75, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 79, "end_idx": 80, "type": "dis", "entity": "肾炎" }, { "start_idx": 83, "end_idx": 88, "type": "dis", "entity": "多动脉炎肾炎" } ] }, { "text": "成人类风湿关节炎可见80%RF阳性,而幼年特发性关节炎(JIA)患儿阳性率仅约5%~15%,因此不能依靠它诊断JIA。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "类风湿关节炎" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "RF" }, { "start_idx": 19, "end_idx": 26, "type": "dis", "entity": "幼年特发性关节炎" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "JIA" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "JIA" } ] }, { "text": "因此建议将APF作为JIA诊断指标。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "APF" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "JIA" } ] }, { "text": "最近的报道显示成人DCM患者中47%为特发性,12%与心肌炎有关,11%与冠状动脉病变有关,另有30%为其他原因。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "DCM" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "冠状动脉" } ] }, { "text": "在另外两个不同年龄儿童DCM的研究表明其中2%~15%有活体组织检查证实的心肌炎,其余85%~90%的患儿原因不明。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "DCM" }, { "start_idx": 28, "end_idx": 33, "type": "equ", "entity": "活体组织检查" } ] }, { "text": "其他可应用的利尿剂包括依他尼酸、布美他尼。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "利尿剂" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "依他尼酸" }, { "start_idx": 16, "end_idx": 19, "type": "dru", "entity": "布美他尼" } ] }, { "text": "有效的口服降低后负荷制剂包括ACE抑制剂。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "dru", "entity": "ACE抑制剂" } ] }, { "text": "在儿科,最常用的为卡托普利及依那普利。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dep", "entity": "儿科" }, { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "卡托普利" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "依那普利" } ] }, { "text": "因而这些患儿应考虑应用华法林等类抗凝剂。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dru", "entity": "华法林" }, { "start_idx": 16, "end_idx": 18, "type": "dru", "entity": "抗凝剂" } ] }, { "text": "如已明确有心腔内血栓,应积极以肝素治疗,最终过渡到长期华法林治疗。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "心腔" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "血栓" }, { "start_idx": 15, "end_idx": 16, "type": "dru", "entity": "肝素" }, { "start_idx": 27, "end_idx": 29, "type": "dru", "entity": "华法林" } ] }, { "text": "如确定系心动过速诱导的心肌病,应予以抗心律失常药物治疗。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "心肌病" }, { "start_idx": 19, "end_idx": 20, "type": "ite", "entity": "心律" } ] }, { "text": "普鲁卡因胺及β受体阻滞剂是有效的抗心律失常药物,但因其有负性肌力作用,在这组患儿应慎用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "普鲁卡因胺" }, { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "β受体阻滞剂" } ] }, { "text": "咳嗽多较严重,初为干咳,很快转为顽固性剧咳,有时表现为百日咳样咳嗽,咳少量黏痰,偶见痰中带血丝或血块。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "咳嗽" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "干咳" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "顽固性剧咳" }, { "start_idx": 27, "end_idx": 32, "type": "sym", "entity": "百日咳样咳嗽" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "咳少量黏痰" }, { "start_idx": 40, "end_idx": 49, "type": "sym", "entity": "偶见痰中带血丝或血块" } ] }, { "text": "早期患儿可用PCR法检测患儿痰等分泌物中MP-DNA,亦可从痰、鼻分泌物、咽拭子中分离培养出MP。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "ite", "entity": "PCR法" }, { "start_idx": 20, "end_idx": 25, "type": "mic", "entity": "MP-DNA" } ] }, { "text": "对难治性患儿应关注并发症如胸腔积液、阻塞性甚至坏死性肺炎的可能,及时进行胸腔穿刺或胸腔闭锁引流,必要时进行纤维支气管镜下支气管灌洗治疗。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "胸腔积液" }, { "start_idx": 18, "end_idx": 27, "type": "dis", "entity": "阻塞性甚至坏死性肺炎" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "胸腔穿刺" }, { "start_idx": 41, "end_idx": 46, "type": "pro", "entity": "胸腔闭锁引流" }, { "start_idx": 53, "end_idx": 66, "type": "pro", "entity": "纤维支气管镜下支气管灌洗治疗" } ] }, { "text": "在我国乙型脑炎主要发生于夏秋季节(7~9月份),与其主要传媒——库蚊的繁殖季节相关。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "乙型脑炎" } ] }, { "text": "国外报道约70%的病毒性脑炎和脑膜炎发生于6~11月份,儿童发病者约占50%,男孩发病稍多。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "病毒性脑炎" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "1960年以前,腮腺炎和脊髓灰质炎病毒感染约占中枢神经系统病毒感染的35%,而近年来在实施了此两种病毒计划免疫的国家,发病者明显减少。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "腮腺炎" }, { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "脊髓灰质炎病毒感染" }, { "start_idx": 23, "end_idx": 32, "type": "dis", "entity": "中枢神经系统病毒感染" } ] }, { "text": "但由于在病理解剖研究中比较容易准确判断骑跨的大动脉瓣与下方室间隔的相互连接关系,因此其诊断标准为当两根大动脉根部一半以上的周边均连接至同一心室时即诊断为右室双出口。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "大动脉瓣" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "室间隔" }, { "start_idx": 51, "end_idx": 55, "type": "bod", "entity": "大动脉根部" }, { "start_idx": 69, "end_idx": 70, "type": "bod", "entity": "心室" }, { "start_idx": 76, "end_idx": 80, "type": "dis", "entity": "右室双出口" } ] }, { "text": "绝大多数的右室双出口伴有室间隔缺损,室间隔缺损可以是膜周型、肌部、远离大动脉或双动脉下。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "右室双出口" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肌部" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "大动脉" }, { "start_idx": 39, "end_idx": 41, "type": "bod", "entity": "双动脉" } ] }, { "text": "几乎3/4的右室双出口均有不同程度的肺动脉狭窄甚至闭锁,多为圆锥隔组织导致的肺动脉瓣下狭窄。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "右室双出口" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "肺动脉狭窄" } ] }, { "text": "由于法洛四联症通常由其特殊的流出道形态作出诊断,但该特殊形态在法洛四联症及右室双出口均可存在,因此,右室双出口与法洛四联症较难明确区分。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "流出道" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "法洛四联症" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "右室双出口" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "右室双出口" }, { "start_idx": 56, "end_idx": 60, "type": "dis", "entity": "法洛四联症" } ] }, { "text": "右室双出口可能合并的心脏节段连接异常包括:房室连接不一致、心室双入口或房室瓣闭锁,在以上情况下,外科的纠治方案完全不同。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "右室双出口" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心脏" }, { "start_idx": 21, "end_idx": 27, "type": "sym", "entity": "房室连接不一致" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "房室瓣闭锁" }, { "start_idx": 48, "end_idx": 49, "type": "dep", "entity": "外科" } ] }, { "text": "右室双出口常见的合并畸形包括房室瓣异常、左右流出道梗阻、主动脉弓中断、水肿及房室间隔缺损。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "右室双出口" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "畸形" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "房室瓣" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "水肿" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "房室间隔缺损" } ] }, { "text": "在法四型右室双出口,如果存在严重的肺血供不足,可在新生儿期即有青紫表现。", "entities": [ { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "青紫" } ] }, { "text": "伴主动脉下室间隔缺损的右室双出口的典型临床表现是在出生近1月时充血性心力衰竭而无青紫表现,与单纯大型室间隔缺损临床表现相似,如果生后早期出现心力衰竭则应考虑是否同时伴有水肿。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "主动脉" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 11, "end_idx": 15, "type": "dis", "entity": "右室双出口" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "充血性心力衰竭" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "青紫" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 84, "end_idx": 85, "type": "sym", "entity": "水肿" } ] }, { "text": "不同程度的肺动脉狭窄可伴有不同程度的青紫,可在生后1岁以内即出现。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "青紫" } ] }, { "text": "当狭窄严重时,早期即可出现青紫、乏力、活动后气促、蹲踞及高血红蛋白血症。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "狭窄" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "青紫" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "乏力" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "活动后气促" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "蹲踞" } ] }, { "text": "体格检查可发现有青紫和杵状指(趾),心前区搏动弥散,左侧胸骨旁2~4肋间可及4/6~5/6级的喷射性收缩期杂音,高位左侧胸骨旁可及震颤。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "体格检查" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "青紫" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "杵状指(趾)" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "心前区" }, { "start_idx": 26, "end_idx": 54, "type": "sym", "entity": "左侧胸骨旁2~4肋间可及4/6~5/6级的喷射性收缩期杂音" }, { "start_idx": 56, "end_idx": 66, "type": "sym", "entity": "高位左侧胸骨旁可及震颤" } ] }, { "text": "在伴有水肿时,婴儿早期即可有心功能衰竭、青紫和股动脉搏动减弱或消失。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "水肿" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "心功能衰竭" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "青紫" }, { "start_idx": 23, "end_idx": 32, "type": "sym", "entity": "股动脉搏动减弱或消失" } ] }, { "text": "体格检查可发现典型的青紫和杵状指(趾),身高、体重明显落后于同龄人。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "体格检查" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "青紫" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "杵状指(趾)" } ] }, { "text": "心前区隆起且心尖搏动弥散,在左侧胸骨旁可及高调的2~3级收缩期杂音,当伴有肺动脉狭窄时则可及3~4级响亮的收缩期杂音,第二心音通常响亮而单一。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "心前区隆起且心尖搏动弥散" }, { "start_idx": 13, "end_idx": 32, "type": "sym", "entity": "在左侧胸骨旁可及高调的2~3级收缩期杂音" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "肺动脉狭窄" }, { "start_idx": 44, "end_idx": 57, "type": "sym", "entity": "可及3~4级响亮的收缩期杂音" }, { "start_idx": 59, "end_idx": 69, "type": "sym", "entity": "第二心音通常响亮而单一" } ] }, { "text": "左侧胸骨旁可及3/6~4/6级的全收缩期杂音伴震颤,心尖区可及舒张期杂音及第三心音。", "entities": [ { "start_idx": 0, "end_idx": 24, "type": "sym", "entity": "左侧胸骨旁可及3/6~4/6级的全收缩期杂音伴震颤" }, { "start_idx": 26, "end_idx": 40, "type": "sym", "entity": "心尖区可及舒张期杂音及第三心音" } ] }, { "text": "一般手术方式的选择:①伴主动脉下室缺不伴有右室流出道狭窄,可以根据室间隔缺损的大小,临床症状及一般情况选择姑息术(肺动脉Banding术)或根治术;②伴主动脉下室间隔缺损伴有右室流出道狭窄可行体-肺分流术;③伴肺动脉下室间隔缺损常合并有主动脉弓缩窄,早期易发生肺动脉梗阻,可行姑息术(房间隔造口术)+肺动脉Banding术;④伴远离大动脉室间隔缺损的手术方式基本与伴主动脉下室间隔缺损合并或不合并右室流出道狭窄的手术方式相同。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "主动脉下室缺" }, { "start_idx": 21, "end_idx": 27, "type": "dis", "entity": "右室流出道狭窄" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "室间隔缺损" }, { "start_idx": 53, "end_idx": 55, "type": "pro", "entity": "姑息术" }, { "start_idx": 57, "end_idx": 67, "type": "pro", "entity": "肺动脉Banding术" }, { "start_idx": 70, "end_idx": 72, "type": "pro", "entity": "根治术" }, { "start_idx": 76, "end_idx": 84, "type": "dis", "entity": "主动脉下室间隔缺损" }, { "start_idx": 87, "end_idx": 93, "type": "dis", "entity": "右室流出道狭窄" }, { "start_idx": 96, "end_idx": 101, "type": "pro", "entity": "体-肺分流术" }, { "start_idx": 105, "end_idx": 113, "type": "dis", "entity": "肺动脉下室间隔缺损" }, { "start_idx": 118, "end_idx": 123, "type": "dis", "entity": "主动脉弓缩窄" }, { "start_idx": 130, "end_idx": 134, "type": "dis", "entity": "肺动脉梗阻" }, { "start_idx": 138, "end_idx": 140, "type": "pro", "entity": "姑息术" }, { "start_idx": 142, "end_idx": 147, "type": "pro", "entity": "房间隔造口术" }, { "start_idx": 150, "end_idx": 160, "type": "pro", "entity": "肺动脉Banding术" }, { "start_idx": 164, "end_idx": 173, "type": "dis", "entity": "远离大动脉室间隔缺损" }, { "start_idx": 183, "end_idx": 191, "type": "dis", "entity": "主动脉下室间隔缺损" }, { "start_idx": 198, "end_idx": 204, "type": "dis", "entity": "右室流出道狭窄" } ] }, { "text": "近年来根据尿红细胞形态改变等改变,常将血尿分为肾小球性血尿及非肾小球性血尿,有利于临床诊断。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "ite", "entity": "尿红细胞形态" }, { "start_idx": 5, "end_idx": 12, "type": "sym", "entity": "尿红细胞形态改变" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "血尿" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "肾小球性血尿" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "非肾小球性血尿" } ] }, { "text": "5.先天性肾及血管畸形如多囊肾、膀胱憩室,动静脉瘘,血管瘤等。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "先天性肾及血管畸形" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "多囊肾" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "膀胱憩室" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "动静脉瘘" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "血管瘤" } ] }, { "text": "多见于接近足月儿和足月儿,有剖宫产、羊水吸入、母亲产前应用大量镇静剂等病史。", "entities": [ { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "镇静剂" } ] }, { "text": "临床症状类似早产儿RDS,一般主要表现为气促,60~100次/分,可以出现吸气性凹陷征,肺内有湿啰音。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "RDS" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "气促" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "肺内" }, { "start_idx": 37, "end_idx": 49, "type": "sym", "entity": "吸气性凹陷征,肺内有湿啰音" } ] }, { "text": "研究亦显示产前给予糖皮质激素和出生后给予肺表面活性物质可以产生增强效果,更有利于预防RDS。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 20, "end_idx": 26, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "RDS" } ] }, { "text": "在RDS恢复阶段,可以考虑补充氨基酸、脂肪乳剂等。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "RDS" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "氨基酸" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "脂肪乳剂" } ] }, { "text": "应用肺表面活性物质治疗RDS可以减少BPD的发生,主要在于肺表面活性物质可以显著减少患儿对机械通气和氧疗的依赖时间,并降低机械通气压力和吸入氧浓度。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "RDS" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "BPD" }, { "start_idx": 29, "end_idx": 35, "type": "bod", "entity": "肺表面活性物质" }, { "start_idx": 45, "end_idx": 48, "type": "pro", "entity": "机械通气" }, { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "氧疗" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "机械通气" } ] }, { "text": "早产儿贫血的病因仍未明确,可能与下列因素有关:①早产儿红细胞寿命40~60天,比足月儿(70~90天)更短;②促红细胞生成素(EPO)水平低下,由于早产儿发育未成熟,生后前几周产生EPO的部位主要在肝脏,而肝脏对缺氧的敏感性不如肾脏,因此产生EPO较少;③早产儿生长迅速,血液稀释;④疾病因素:早产儿易患许多疾病,加重贫血;⑤医源性失血:早产儿病情重,需取血做各种检查;⑥营养因素。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "早产儿贫血" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "红细胞" }, { "start_idx": 55, "end_idx": 61, "type": "bod", "entity": "促红细胞生成素" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "EPO" }, { "start_idx": 90, "end_idx": 92, "type": "bod", "entity": "EPO" }, { "start_idx": 99, "end_idx": 100, "type": "bod", "entity": "肝脏" }, { "start_idx": 103, "end_idx": 104, "type": "bod", "entity": "肝脏" }, { "start_idx": 121, "end_idx": 123, "type": "bod", "entity": "EPO" }, { "start_idx": 136, "end_idx": 137, "type": "bod", "entity": "血液" }, { "start_idx": 159, "end_idx": 160, "type": "dis", "entity": "贫血" }, { "start_idx": 163, "end_idx": 167, "type": "sym", "entity": "医源性失血" } ] }, { "text": "前者见于非裔美国人,酶活性在15%以下;后者见于地中海地区国家如意大利、希腊,和中东、非洲及亚洲诸国的人群,酶活性在5%~40%之间,也称G-6-PD地中海型。", "entities": [ { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "酶" } ] }, { "text": "常见外源性诱因包括细菌、病毒等感染(脐炎、败血症、腹泻等)或药物(抗疟药、磺胺类、萘类等)所致,后者可因新生儿用药或母亲服药通过胎盘或母乳进入患儿体内引起。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "感染" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "脐炎" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "败血症" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "腹泻" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "抗疟药" }, { "start_idx": 37, "end_idx": 39, "type": "dru", "entity": "磺胺类" }, { "start_idx": 41, "end_idx": 42, "type": "dru", "entity": "萘类" } ] }, { "text": "部分病儿也可因注射维生素K制剂或接触樟脑丸而发生溶血。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dru", "entity": "维生素K制剂" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "溶血" } ] }, { "text": "内源性因素可能与生理性NADPH还原酶和谷胱甘肽过氧化氢酶活性较低,血糖和维生素E水平低以及缺氧、酸中毒等有关。", "entities": [ { "start_idx": 8, "end_idx": 18, "type": "bod", "entity": "生理性NADPH还原酶" }, { "start_idx": 20, "end_idx": 28, "type": "bod", "entity": "谷胱甘肽过氧化氢酶" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "血糖" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "维生素E" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "缺氧" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "酸中毒" } ] }, { "text": "临床上主要表现为持续轻至中度贫血和黄疸,脾脏多增大,血清胆红素不同程度的增高,网织红细胞持续增高。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "中度贫血" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "黄疸" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "脾脏多增大" }, { "start_idx": 26, "end_idx": 37, "type": "sym", "entity": "血清胆红素不同程度的增高" }, { "start_idx": 39, "end_idx": 47, "type": "sym", "entity": "网织红细胞持续增高" } ] }, { "text": "重症者可在短期内出现溶血危象,血红蛋白可下降60%~70%,常有畏寒、发热、恶心、呕吐、腹痛和背痛等,同时出现血红蛋白尿,尿呈酱油色、浓茶色或暗红色。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "溶血危象" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "畏寒" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "发热" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "恶心" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "呕吐" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "腹痛" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "背痛" }, { "start_idx": 55, "end_idx": 59, "type": "dis", "entity": "血红蛋白尿" }, { "start_idx": 61, "end_idx": 73, "type": "sym", "entity": "尿呈酱油色、浓茶色或暗红色" } ] }, { "text": "轻症者仅有轻度溶血,而不伴血红蛋白尿或黄疸。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "溶血" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "血红蛋白尿" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "黄疸" } ] }, { "text": "药物引起的溶血性贫血临床表现与蚕豆病相似,其主要临床特点是服药1天~4天后出现血管内溶血。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "溶血性贫血临床" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "溶血" } ] }, { "text": "网织红细胞增高。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "网织红细胞" } ] }, { "text": "此时可有发热、倦怠、巩膜黄染、腹或背痛。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "发热" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "倦怠" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "巩膜黄染" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "腹或背痛" } ] }, { "text": "Coombs试验阴性。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "Coombs试验" } ] }, { "text": "临床上按病因及病理变化的不同,分为急性单纯性胃炎、急性糜烂性胃炎、急性腐蚀性胃炎及急性化脓性胃炎,其中临床上以急性单纯性胃炎最为常见,而由于抗生素广泛应用,急性化脓性胃炎已罕见。", "entities": [ { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "急性单纯性胃炎" }, { "start_idx": 25, "end_idx": 31, "type": "dis", "entity": "急性糜烂性胃炎" }, { "start_idx": 33, "end_idx": 39, "type": "dis", "entity": "急性腐蚀性胃炎" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "急性化脓性胃炎" }, { "start_idx": 55, "end_idx": 61, "type": "dis", "entity": "急性单纯性胃炎" }, { "start_idx": 70, "end_idx": 72, "type": "dru", "entity": "抗生素" }, { "start_idx": 78, "end_idx": 84, "type": "dis", "entity": "急性化脓性胃炎" } ] }, { "text": "细菌毒素以金黄色葡萄球菌为多见,偶为肉毒杆菌毒素。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "mic", "entity": "金黄色葡萄球菌" }, { "start_idx": 18, "end_idx": 23, "type": "mic", "entity": "肉毒杆菌毒素" } ] }, { "text": "近年发现幽门螺杆菌也是引起急性胃炎的一种病原菌。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "急性胃炎" }, { "start_idx": 20, "end_idx": 22, "type": "mic", "entity": "病原菌" } ] }, { "text": "厌食、恶心、呕吐,若伴有肠炎,可有腹泻。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "厌食" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "恶心" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "呕吐" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "肠炎" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "腹泻" } ] }, { "text": "腹泻者,粪便常规检查有少量黏液及红、白细胞。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "腹泻" }, { "start_idx": 4, "end_idx": 9, "type": "ite", "entity": "粪便常规检查" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "黏液" }, { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "红、白细胞" } ] }, { "text": "肾异位的位置包括:同侧的盆腔、髂骨、腹部、胸腔,或对侧的上述部位。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肾异位" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "盆腔" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "髂骨" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "腹部" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "胸腔" } ] }, { "text": "盆腔肾与骶骨相对,位于主动脉分叉水平以下;髂位肾靠骶骨岬,位于髂窝,在髂血管的前方;腹腔肾一般位于髂嵴的上方,靠近第二腰椎。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "盆腔肾" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "骶骨" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "主动脉分叉" }, { "start_idx": 21, "end_idx": 27, "type": "bod", "entity": "髂位肾靠骶骨岬" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "髂窝" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "髂血管" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "腹腔肾" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "髂嵴" }, { "start_idx": 59, "end_idx": 60, "type": "bod", "entity": "腰椎" } ] }, { "text": "由于肾旋转不完全,肾盂常位于肾皮质的前方,56%的异位肾可以出现集合系统积水,其中一半是由肾盂输尿管连接部梗阻或膀胱输尿管连接部狭窄引起的。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肾" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "肾盂" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肾皮质" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "异位肾" }, { "start_idx": 32, "end_idx": 37, "type": "sym", "entity": "集合系统积水" }, { "start_idx": 45, "end_idx": 54, "type": "dis", "entity": "肾盂输尿管连接部梗阻" }, { "start_idx": 56, "end_idx": 65, "type": "dis", "entity": "膀胱输尿管连接部狭窄" } ] }, { "text": "异位肾的输尿管多有扭曲,但多于输尿管起始侧进入膀胱,膀胱内开口于正常的位置。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "异位肾" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "膀胱" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "膀胱" } ] }, { "text": "男孩可出现隐睾、重复尿道及尿道下裂。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "隐睾" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "重复尿道" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "尿道下裂" } ] }, { "text": "绝大多数异位肾无症状;少数患儿可因肾盂或输尿管的梗阻而出现腹痛,也可有尿路感染或盆腔肿块。", "entities": [ { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "肾盂或输尿管的梗阻" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "腹痛" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "尿路感染" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "盆腔肿块" } ] }, { "text": "腹泻病是多病因、多因素引起的一组疾病,是儿童时期发病率最高的疾病之一,是世界性公共卫生问题。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "腹泻病" } ] }, { "text": "心电图表现ST段下移,T波压低、平坦、双相、倒置,出现U波,P-R间期和Q-T间期延长。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "心电图" }, { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "ST段下移" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "T波压低" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "平坦" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "双相" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "倒置" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "出现U波" }, { "start_idx": 30, "end_idx": 42, "type": "sym", "entity": "P-R间期和Q-T间期延长" } ] }, { "text": "原有营养不良及佝偻病时更易出现,少数患儿可出现低镁血症,表现为手足震颤,舞蹈病样不随意运动,易受刺激,烦躁不安,严重者可发生惊厥,补充钙剂后症状无改善。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "营养不良" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "佝偻病" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "低镁血症" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "手足" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "手足震颤" }, { "start_idx": 36, "end_idx": 44, "type": "sym", "entity": "舞蹈病样不随意运动" }, { "start_idx": 46, "end_idx": 49, "type": "sym", "entity": "易受刺激" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "惊厥" }, { "start_idx": 67, "end_idx": 68, "type": "dru", "entity": "钙剂" } ] }, { "text": "常伴脱水和酸中毒。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "脱水" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "酸中毒" } ] }, { "text": "本病为自限性疾病,病程3~8天,少数较长,大便镜检偶见少量白细胞。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "pro", "entity": "大便镜检" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "白细胞" } ] }, { "text": "常伴有呕吐,多无发热和全身症状。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "常伴有呕吐" }, { "start_idx": 6, "end_idx": 14, "type": "sym", "entity": "多无发热和全身症状" } ] }, { "text": "主要表现水、电解质紊乱。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "水" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "电解质" }, { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "水、电解质紊乱" } ] }, { "text": "临床症状与细菌性痢疾较难区别,需做大便培养鉴别。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "细菌性痢疾" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "大便培养鉴别" } ] }, { "text": "伴腹痛。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "腹痛" } ] }, { "text": "可伴发溶血尿毒综合征和血小板减少性紫癜。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "溶血尿毒综合征" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "血小板减少性紫癜" } ] }, { "text": "以6个月~2岁婴幼儿发病率最高,家畜、家禽是主要的感染源,经粪-口途径,动物→人或人→人传播。", "entities": [ { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "粪-口" } ] }, { "text": "起病急,症状与细菌性痢疾相似。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "细菌性痢疾" } ] }, { "text": "本病可并发肠系膜淋巴结炎、结节性红斑、反应性关节炎、败血症、心肌炎、急性肝炎、肝脓肿、结膜炎、脑膜炎、尿道炎或急性肾炎等。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "肠系膜淋巴结炎" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "结节性红斑" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "反应性关节炎" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "败血症" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "心肌炎" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "急性肝炎" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "肝脓肿" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "结膜炎" }, { "start_idx": 47, "end_idx": 49, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "尿道炎" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "急性肾炎" } ] }, { "text": "多数为2岁以下婴幼儿,易在儿科病房发生流行。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dep", "entity": "儿科" } ] }, { "text": "常伴显著的低蛋白血症,水、电解质紊乱,全身软弱呈慢性消耗状。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "低蛋白血症" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "水、电解质紊乱" }, { "start_idx": 19, "end_idx": 28, "type": "sym", "entity": "全身软弱呈慢性消耗状" } ] }, { "text": "人和动物的卡氏肺孢子虫感染率很高,但通常仅少数虫体寄生于肺泡内(隐性感染),如遇到虚弱乳幼儿、未成熟儿、先天性免疫缺陷及用免疫抑制剂治疗的白血病等免疫功能低下、尤其是T淋巴细胞功能缺陷的病儿,可引起本病。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "mic", "entity": "虫体" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "肺泡" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "隐性感染" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "虚弱乳幼儿" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "未成熟儿" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "先天性免疫缺陷" }, { "start_idx": 61, "end_idx": 65, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 69, "end_idx": 71, "type": "dis", "entity": "白血病" }, { "start_idx": 83, "end_idx": 91, "type": "dis", "entity": "T淋巴细胞功能缺陷" } ] }, { "text": "临床表现可分为两个类型:①婴儿型:主要发生于1~4个月虚弱婴儿及未成熟儿,起病缓慢,全身症状突出,主要表现为吃奶不好、烦躁不安,早期出现呼吸加快和发绀,1~2周内逐渐加重,出现咳嗽、鼻扇及三凹征,但肺部几乎听不到啰音。", "entities": [ { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "吃奶不好" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 73, "end_idx": 74, "type": "sym", "entity": "发绀" }, { "start_idx": 88, "end_idx": 89, "type": "sym", "entity": "咳嗽" }, { "start_idx": 91, "end_idx": 92, "type": "sym", "entity": "鼻扇" }, { "start_idx": 94, "end_idx": 96, "type": "sym", "entity": "三凹征" }, { "start_idx": 99, "end_idx": 107, "type": "sym", "entity": "肺部几乎听不到啰音" } ] }, { "text": "②儿童型:主要发生于获得性免疫功能低下和应用大量免疫抑制剂者。", "entities": [ { "start_idx": 10, "end_idx": 18, "type": "dis", "entity": "获得性免疫功能低下" }, { "start_idx": 20, "end_idx": 29, "type": "dis", "entity": "应用大量免疫抑制剂者" } ] }, { "text": "起病急骤,常见症状为发热、咳嗽、呼吸加快、发绀、鼻扇及腹泻等,但肺部亦多无啰音。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "发热" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "咳嗽" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "呼吸加快" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "发绀" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "鼻扇" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "腹泻" }, { "start_idx": 32, "end_idx": 38, "type": "sym", "entity": "肺部亦多无啰音" } ] }, { "text": "次日肺内迅速出现广泛融合小片影、肺透亮度减低,可见支气管充气征、泡性肺气肿、病变密度不均匀,肺门影不大。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "肺" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "小片影" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "肺透亮度减低" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "支气管充气征" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "泡性肺气肿" }, { "start_idx": 38, "end_idx": 44, "type": "sym", "entity": "病变密度不均匀" }, { "start_idx": 46, "end_idx": 50, "type": "sym", "entity": "肺门影不大" } ] }, { "text": "在早产婴儿、新生儿和先天或后天性免疫缺陷或抑制的患儿,如出现重度呼吸困难而肺部体征极少,X线胸片出现上述改变时,应考虑为本病。", "entities": [ { "start_idx": 10, "end_idx": 22, "type": "dis", "entity": "先天或后天性免疫缺陷或抑制" }, { "start_idx": 30, "end_idx": 35, "type": "sym", "entity": "重度呼吸困难" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "肺部体征极少" }, { "start_idx": 44, "end_idx": 47, "type": "ite", "entity": "X线胸片" } ] }, { "text": "亦可用PCR法检测痰及气道分泌物中卡氏肺孢子虫的DNA。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "PCR法检测" }, { "start_idx": 9, "end_idx": 9, "type": "ite", "entity": "痰" }, { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "分泌物" }, { "start_idx": 17, "end_idx": 22, "type": "mic", "entity": "卡氏肺孢子虫" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "DNA" } ] }, { "text": "缺铁不仅会引起小细胞低色素性的贫血,不利于儿童正常的行为和生长发育,影响胃肠道的消化吸收功能和机体免疫功能,还易导致铅元素的吸收增加。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "缺铁" }, { "start_idx": 4, "end_idx": 16, "type": "sym", "entity": "会引起小细胞低色素性的贫血" }, { "start_idx": 18, "end_idx": 32, "type": "sym", "entity": "不利于儿童正常的行为和生长发育" }, { "start_idx": 34, "end_idx": 52, "type": "sym", "entity": "影响胃肠道的消化吸收功能和机体免疫功能" }, { "start_idx": 54, "end_idx": 65, "type": "sym", "entity": "还易导致铅元素的吸收增加" } ] }, { "text": "缺锌表现为生长发育落后,性发育障碍,情绪冷漠,厌食、味觉异常和异食癖,皮肤易感染,伤口愈合延迟,母孕期如缺乏锌会引起胎儿畸形等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "缺锌" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "生长发育落后" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "性发育障碍" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "情绪冷漠" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "厌食" }, { "start_idx": 26, "end_idx": 33, "type": "sym", "entity": "味觉异常和异食癖" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "皮肤易感染" }, { "start_idx": 41, "end_idx": 46, "type": "sym", "entity": "伤口愈合延迟" }, { "start_idx": 48, "end_idx": 62, "type": "sym", "entity": "母孕期如缺乏锌会引起胎儿畸形等" } ] }, { "text": "铬缺乏时会引起生长发育迟缓,糖耐量下降和血脂增高等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "铬缺乏" }, { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "生长发育迟缓" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "糖耐量下降" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "血脂增高" } ] }, { "text": "根据Wood临床评分标准可以对哮喘的病情做出判断(表8-8)。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "哮喘" } ] }, { "text": "硫酸镁通过抑制钙离子介导的平滑肌收缩,扩张支气管;可用于6岁以上,对其他平喘治疗无效患儿。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "硫酸镁" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "平滑肌" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "支气管" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "平喘治疗" } ] }, { "text": "病情缓解后全身用糖皮质激素应逐渐减量,可继续吸入普米克令舒。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "普米克令舒" } ] }, { "text": "体格检查可见咽侧壁膨出。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "咽侧壁" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "咽侧壁膨出" } ] }, { "text": "急性Q热起病似流感样症状,可有间质性肺炎或肉芽肿肝炎表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "急性Q热" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "间质性肺炎" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "肉芽肿肝炎" } ] }, { "text": "慢性Q热表现为心内膜炎,但血培养阴性。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "慢性Q热" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "心内膜炎" }, { "start_idx": 13, "end_idx": 15, "type": "ite", "entity": "血培养" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "血培养阴性" } ] }, { "text": "约2/3患者伴有肺部病变,症状轻重不等。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肺部" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "肺部病变" } ] }, { "text": "肺部有时无体征,或呼吸音减低,可听到细小水泡音。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "肺部有时无体征" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "呼吸音减低" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "细小水泡音" } ] }, { "text": "少数病例肝脾肿大,并发心肌炎、脑炎或脑膜炎。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "肝脾" }, { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "肝脾肿大" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "心肌炎" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "脑炎" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脑膜炎" } ] }, { "text": "急性Q热数月或数年后可能出现瓣膜损害的心内膜炎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "急性Q热" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "心内膜炎" }, { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "瓣膜损害的心内膜炎" } ] }, { "text": "慢性Q热的其他表现还有血管受累、动脉瘤、骨髓炎、长期发热、肺炎、肝炎或紫癜样皮疹。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "慢性Q热" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "血管" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "血管受累" }, { "start_idx": 16, "end_idx": 18, "type": "sym", "entity": "动脉瘤" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "骨髓炎" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "长期发热" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "肺炎" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "肝炎" }, { "start_idx": 35, "end_idx": 39, "type": "sym", "entity": "紫癜样皮疹" } ] }, { "text": "经典的病理分类将NB分成3型,即神经母细胞瘤、神经节母细胞瘤、神经节细胞瘤,这三个类型反映了NB的分化、成熟过程。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "NB" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "神经母细胞瘤" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "神经节母细胞瘤" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "神经节细胞瘤" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "NB" } ] }, { "text": "65%患儿肿瘤原发于腹腔,大年龄儿童中肾上腺原发占40%,而在婴儿中只占25%。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "腹腔" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "上腺" } ] }, { "text": "最常见的症状为不同部位的肿块。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肿块" } ] }, { "text": "NB主要转移途径为淋巴及血行。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "NB" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "淋巴" } ] }, { "text": "在局限性病变病人中约35%有局部淋巴结浸润,血行转移主要发生于骨髓、骨、肝和皮肤,终末期或复发时可有脑和肺转移,但较少见。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "淋巴结" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "骨髓" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "骨" }, { "start_idx": 36, "end_idx": 36, "type": "bod", "entity": "肝" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "皮肤" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "脑" }, { "start_idx": 52, "end_idx": 52, "type": "bod", "entity": "肺" } ] }, { "text": "婴儿病例就诊时局限性病变、局限性病变伴有局部淋巴结转移、播散性病变分别为39%、18%和25%;但在大年龄儿童中分别为19%、13%和68%,也即大年龄患儿就诊时多数已处疾病晚期。", "entities": [ { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "淋巴结" } ] }, { "text": "为确定病变范围及临床分期,应作骨髓活检或涂片,选择性骨骼平片、胸片、骨扫描,胸、腹部CT或MRI。", "entities": [ { "start_idx": 15, "end_idx": 21, "type": "pro", "entity": "骨髓活检或涂片" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "骨骼平片" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "胸片" }, { "start_idx": 34, "end_idx": 36, "type": "pro", "entity": "骨扫描" }, { "start_idx": 38, "end_idx": 47, "type": "pro", "entity": "胸、腹部CT或MRI" } ] }, { "text": "影像学所示肿块中常有钙化灶,原发于胸腔时多见于后纵隔脊柱两侧,原发于腹腔时多见于肾上腺或后腹膜脊柱两侧。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "胸腔" }, { "start_idx": 23, "end_idx": 27, "type": "bod", "entity": "后纵隔脊柱" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "腹腔" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "肾上腺" }, { "start_idx": 44, "end_idx": 48, "type": "bod", "entity": "后腹膜脊柱" } ] }, { "text": "NB时血LDH可升高,并与肿瘤负荷成正比。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "NB" }, { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "血LDH" }, { "start_idx": 13, "end_idx": 16, "type": "ite", "entity": "肿瘤负荷" } ] }, { "text": "可用荧光原位杂交法(FISH)检测肿瘤细胞N-MYC的扩增情况,如大于10倍,常提示预后不良。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "pro", "entity": "荧光原位杂交法" }, { "start_idx": 10, "end_idx": 13, "type": "pro", "entity": "FISH" }, { "start_idx": 17, "end_idx": 25, "type": "bod", "entity": "肿瘤细胞N-MYC" } ] }, { "text": "Ⅰ期:肿瘤局限于原发器官。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肿瘤" } ] }, { "text": "Ⅱ期:肿瘤超出原发器官,但未超过中线,同侧淋巴结可能受累。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肿瘤" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "淋巴结" } ] }, { "text": "Ⅲ期:肿瘤超过中线,双侧淋巴结可能受累。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肿瘤" }, { "start_idx": 10, "end_idx": 14, "type": "bod", "entity": "双侧淋巴结" } ] }, { "text": "Ⅵs期:<1岁,原发灶为Ⅰ、Ⅱ期,但又局限于肝、皮肤、骨髓的转移灶。", "entities": [ { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肝" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "皮肤" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "骨髓" } ] }, { "text": "美国CCSG协作组报告晚期NB在接受自身骨髓移植后4年无进展性疾病生存率为38%,各项处理方案结果未显示有差别。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "NB" } ] }, { "text": "对Ⅳ期具有其他预后不良因素者(如N-myc扩增,年龄>2岁,诱导治疗未获缓解者),自身骨髓移植组预后要比常规治疗好。", "entities": [ { "start_idx": 16, "end_idx": 20, "type": "bod", "entity": "N-myc" }, { "start_idx": 43, "end_idx": 46, "type": "pro", "entity": "骨髓移植" } ] }, { "text": "NB对放疗敏感,但全身放疗在干细胞移植预处理方案中的应用尚有争论。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "NB" }, { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "放疗" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "放疗" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "干细胞移植" } ] }, { "text": "NB的原发部位复发机会较高,因此对Ⅲ、Ⅳ期病人仍有主张化疗同时采用局部放疗,但其有效性不明确。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "NB" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "局部放疗" } ] }, { "text": "包囊携带者和慢性患者为主要传染源,通过粪-口传播。", "entities": [ { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "粪" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "口" } ] }, { "text": "少数表现似细菌性痢疾,发热,腹痛,体温达39℃,大便带血,每日10次以上,数天后急性症状缓解,10~20天后自行痊愈,也可转为慢性病变。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "细菌性痢疾" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "发热" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "腹痛" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "体温达39℃" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "大便" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "大便带血" } ] }, { "text": "虽然无菌性脑膜炎也可由其他病原体感染或某些非感染性疾病引起,但多数病例系病毒所致。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "无菌性脑膜炎" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "病原体感染" }, { "start_idx": 19, "end_idx": 26, "type": "sym", "entity": "某些非感染性疾病" } ] }, { "text": "其中肠道病毒最为常见,HSV-2次之,其他如腺病毒、VZV、CMV、EBV、风疹病毒、麻疹病毒以及轮状病毒等也均有报道。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "mic", "entity": "肠道病毒" }, { "start_idx": 22, "end_idx": 24, "type": "mic", "entity": "腺病毒" }, { "start_idx": 26, "end_idx": 28, "type": "mic", "entity": "VZV" }, { "start_idx": 30, "end_idx": 32, "type": "mic", "entity": "CMV" }, { "start_idx": 34, "end_idx": 36, "type": "mic", "entity": "EBV" }, { "start_idx": 38, "end_idx": 41, "type": "mic", "entity": "风疹病毒" }, { "start_idx": 43, "end_idx": 46, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 49, "end_idx": 52, "type": "mic", "entity": "轮状病毒" } ] }, { "text": "主要临床表现包括发热、头痛、呕吐和颈项强直。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "发热" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "头痛" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "呕吐" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "颈项强直" } ] }, { "text": "部分病例可伴发轻微脑实质受累而出现不同程度的意识障碍,如易激惹、嗜睡或昏睡等。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "轻微脑实质受累" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "意识障碍" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "易激惹" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "嗜睡" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "昏睡" } ] }, { "text": "早期可出现惊厥发作。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "惊厥发作" } ] }, { "text": "例如,腮腺炎病毒脑膜炎常伴发唾液腺肿痛;肠道病毒感染可伴有皮疹;如病情较重,伴淋巴结肿大或轻度肝区触痛及皮疹,应注意EB病毒感染。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "dis", "entity": "腮腺炎病毒脑膜炎" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "唾液腺肿痛" }, { "start_idx": 20, "end_idx": 25, "type": "dis", "entity": "肠道病毒感染" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "皮疹" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "伴淋巴结肿大" }, { "start_idx": 45, "end_idx": 50, "type": "sym", "entity": "轻度肝区触痛" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "皮疹" } ] }, { "text": "Mollaret脑膜炎的主要表现是反复发作性的发热、头痛、疲劳及脑膜刺激征。", "entities": [ { "start_idx": 0, "end_idx": 10, "type": "dis", "entity": "Mollaret脑膜炎" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "发热" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "头痛" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "疲劳" }, { "start_idx": 32, "end_idx": 36, "type": "sym", "entity": "脑膜刺激征" } ] }, { "text": "在疾病极期可有轻度颅压增高。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "sym", "entity": "轻度颅压增高" } ] }, { "text": "脑电图检查常见弥漫性慢波增多,个别可见痫样放电,随病情好转脑电图异常也逐渐恢复,在并发癫痫的病例仍可见到痫样放电。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "脑电图检查" }, { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "弥漫性慢波增多" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "痫样放电" }, { "start_idx": 29, "end_idx": 31, "type": "pro", "entity": "脑电图" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "痫样放电" } ] }, { "text": "病毒分离和血清学试验是明确病因的基本方法。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "病毒分离" }, { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "血清学试验" } ] }, { "text": "血清学试验一般采用双份血清法,分别于发病早期及恢复期取血或脑脊液送检,抗体滴度如有4倍以上升高则可确诊。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "血清学试验" } ] }, { "text": "包括:①维持水电解质平衡和适当的营养;②控制高热;③镇静剂与止惊剂的应用,适用于出现过度兴奋、多动或惊厥者;④病情监护,如出现昏迷或更严重的神经症状体征,则应按病毒性脑炎治疗。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "pro", "entity": "维持水电解质平衡" }, { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "适当的营养" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "控制高热" }, { "start_idx": 26, "end_idx": 28, "type": "dru", "entity": "镇静剂" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "止惊剂" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "过度兴奋" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "多动" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "惊厥" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "昏迷" }, { "start_idx": 66, "end_idx": 75, "type": "sym", "entity": "更严重的神经症状体征" }, { "start_idx": 80, "end_idx": 84, "type": "dis", "entity": "病毒性脑炎" } ] }, { "text": "确诊或高度怀疑疱疹病毒或其他DNA病毒感染者,应尽早给予无环鸟苷治疗;每次剂量为5~10mg/kg,于1小时内静脉注射,每8小时1次,疗程1~2周。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "疱疹病毒" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "无环鸟苷" } ] }, { "text": "Cater及Evans的研究认为,只要父母一方有过NTDs病史,则其后代NTDs的发病率为3%,明显高于一般人群。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "NTDs" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "NTDs" } ] }, { "text": "因而NTDs的发生是多基因遗传所致,至于遗传因素对于NTDs发生有多大作用,则尚未定论。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "NTDs" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "NTDs" } ] }, { "text": "与NTDs有关联的常见环境因素包括母亲孕早期叶酸和其他多种维生素缺乏,锌和其他微量元素缺乏,严重妊娠反应,病毒感染,服用某些药物,吸烟,酗酒,电离辐射,以及接触某些化学物质等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "NTDs" }, { "start_idx": 35, "end_idx": 44, "type": "sym", "entity": "锌和其他微量元素缺乏" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "严重妊娠反应" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "病毒感染" } ] }, { "text": "母亲孕早期锌缺乏也是引起胎儿发生NTDs的一种环境因素。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "sym", "entity": "锌缺乏" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "NTDs" } ] }, { "text": "至于严重妊娠反应诱发NTDs的原因,可能是因为严重呕吐使水分丢失引起一时性脱水,造成微量元素(如锌)或维生素(如叶酸)缺乏所致。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "妊娠反应" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "NTDs" } ] }, { "text": "妊娠早期弓形体感染也可能导致NTDs。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "弓形体感染" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "NTDs" } ] }, { "text": "可伴有身体其他部位畸形,如腭裂、颈部脊柱裂、胸腔狭小、上下肢比例失调、胫骨和指缺如等。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "腭裂" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "颈部脊柱裂" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "胸腔狭小" }, { "start_idx": 27, "end_idx": 33, "type": "dis", "entity": "上下肢比例失调" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "胫骨" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "指缺如" } ] }, { "text": "②神经系统症状:轻者无明显神经系统症状,重者与发生的部位及受损的程度有关,可表现智力低下、抽搐和不同程度的上运动神经元瘫痪等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "神经系统" }, { "start_idx": 13, "end_idx": 16, "type": "bod", "entity": "神经系统" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "智力低下" }, { "start_idx": 45, "end_idx": 61, "type": "sym", "entity": "抽搐和不同程度的上运动神经元瘫痪等" } ] }, { "text": "部分患儿成年后有慢性腰痛。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "慢性腰痛" } ] }, { "text": "脊髓脊膜膨出可有不同程度的双下肢瘫痪及大小便失禁等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "脊髓脊膜膨出" }, { "start_idx": 13, "end_idx": 17, "type": "dis", "entity": "双下肢瘫痪" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "大小便失禁" } ] }, { "text": "关于脑膜膨出或脑脑膜膨出的诊断,根据囊性包块的部位、大小和外观,透光试验阳性,加上相应的病史及临床表现,一般作出正确诊断并不难。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "脑膜膨出" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "脑脑膜膨出" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "囊性包块" }, { "start_idx": 32, "end_idx": 35, "type": "ite", "entity": "透光试验" } ] }, { "text": "脊膜膨出与脊髓脊膜膨出的诊断是依据患儿出生后即发现背部中线有膨胀性的包块,并随着年龄增长而扩大,以及相应的神经损害症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "脊膜膨出" }, { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "脊髓脊膜膨出" }, { "start_idx": 19, "end_idx": 58, "type": "sym", "entity": "出生后即发现背部中线有膨胀性的包块,并随着年龄增长而扩大,以及相应的神经损害症状" } ] }, { "text": "脊膜膨出和脊髓脊膜膨出的治疗原则是早期手术治疗。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "脊膜膨出" }, { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "脊髓脊膜膨出" }, { "start_idx": 19, "end_idx": 20, "type": "pro", "entity": "手术" } ] }, { "text": "切除脊膜膨出囊和修补软组织缺损,单纯性脊膜膨出经此手术可以治愈。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "软组织缺损" }, { "start_idx": 16, "end_idx": 22, "type": "dis", "entity": "单纯性脊膜膨出" } ] }, { "text": "增补叶酸方法是妇女从怀孕前1个月至怀孕后3个月每日服用一粒叶酸增补剂,可以减少70%以上NTDs的发生。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dru", "entity": "叶酸" }, { "start_idx": 29, "end_idx": 33, "type": "dru", "entity": "叶酸增补剂" } ] }, { "text": "当前市场上叶酸制剂有两类:一类是单纯的叶酸制剂,为孕妇生产的每片含400μg,如斯利安片。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "叶酸制剂" }, { "start_idx": 19, "end_idx": 22, "type": "dru", "entity": "叶酸制剂" }, { "start_idx": 40, "end_idx": 43, "type": "dru", "entity": "斯利安片" } ] }, { "text": "如玛特纳片,其中包括叶酸在内,共含有13种维生素和另外13种矿物质及微量元素。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "玛特纳片" }, { "start_idx": 10, "end_idx": 11, "type": "dru", "entity": "叶酸" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "维生素" } ] }, { "text": "胎儿有NTDs可使羊水AFP水平明显升高,同时母亲血清AFP水平也升高。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "NTDs" } ] }, { "text": "而脑脑膜膨出一般均合并有神经功能障碍、智力低下和其他部位畸形,手术不能解决此类问题,预后差。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "脑脑膜膨出" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "手术" } ] }, { "text": "而脊髓脊膜膨出者,手术治疗疗效差,一般预后不良,即使病人能够存活下来,也是终身残疾。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "脊髓脊膜膨出" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "手术" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "残疾" } ] }, { "text": "阴道出血应与“假月经”鉴别。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "阴道出血" } ] }, { "text": "对消化道出血者,要暂时禁食,从肠道外补充营养。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "消化道" }, { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "消化道出血" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肠道" } ] }, { "text": "位于幕上者,主要为间变性星形细胞瘤及多形性胶质母细胞瘤,少见的有少突胶质细胞瘤、神经节胶质瘤及星形细胞、少突胶质细胞混合型瘤。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "幕上" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "间变性星形细胞瘤" }, { "start_idx": 18, "end_idx": 26, "type": "dis", "entity": "多形性胶质母细胞瘤" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "少突胶质细胞瘤" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "神经节胶质瘤" }, { "start_idx": 47, "end_idx": 61, "type": "dis", "entity": "星形细胞、少突胶质细胞混合型瘤" } ] }, { "text": "肿瘤呈浸润性生长,约25%~50%的肿瘤可向肺、淋巴结、肝及骨等远处转移。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 18, "end_idx": 19, "type": "dis", "entity": "肿瘤" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "肺" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "淋巴结" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肝" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "骨" } ] }, { "text": "临床表现及辅助检查相似于低度胶质瘤。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "低度胶质瘤" } ] }, { "text": "因肿瘤恶性程度高,且呈浸润性生长,很难获得全切除,原则上应在不造成严重神经功能障碍前提下尽可能多的切除肿瘤。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" }, { "start_idx": 21, "end_idx": 23, "type": "pro", "entity": "全切除" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "神经" }, { "start_idx": 49, "end_idx": 52, "type": "pro", "entity": "切除肿瘤" } ] }, { "text": "术后辅以放疗。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "pro", "entity": "术" }, { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "放疗" } ] }, { "text": "目前多主张行局部放疗,全脑放疗对生存率并无明显影响。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "局部放疗" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "全脑放疗" } ] }, { "text": "根据出血部位的不同,ICH可分为脑出血、蛛网膜下腔出血和硬膜下出血等。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "ICH" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "脑出血" }, { "start_idx": 20, "end_idx": 22, "type": "bod", "entity": "蛛网膜" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "蛛网膜下腔出血" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "硬膜" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "硬膜下出血" } ] }, { "text": "先天性脑血管畸形包括血管瘤和动静脉瘘,前者系因血管壁中层发育缺陷所致,见于末梢小动脉分叉处,直径达6~15mm的动脉瘤易发生破裂出血;后者系因动、静脉系统间毛细血管发育缺陷使动、静脉间直接吻合而成短路,以致病区动脉扩大而成动脉瘤样畸形,并压迫其周围脑组织,易破裂出血,以Galen静脉畸形多见。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "先天性脑血管畸形" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "血管瘤" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "动静脉瘘" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "血管壁" }, { "start_idx": 23, "end_idx": 31, "type": "sym", "entity": "血管壁中层发育缺陷" }, { "start_idx": 37, "end_idx": 44, "type": "bod", "entity": "末梢小动脉分叉处" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 71, "end_idx": 74, "type": "bod", "entity": "动、静脉" }, { "start_idx": 71, "end_idx": 85, "type": "sym", "entity": "动、静脉系统间毛细血管发育缺陷" }, { "start_idx": 87, "end_idx": 90, "type": "bod", "entity": "动、静脉" }, { "start_idx": 105, "end_idx": 106, "type": "bod", "entity": "动脉" }, { "start_idx": 103, "end_idx": 116, "type": "sym", "entity": "病区动脉扩大而成动脉瘤样畸形" }, { "start_idx": 119, "end_idx": 126, "type": "sym", "entity": "压迫其周围脑组织" }, { "start_idx": 128, "end_idx": 132, "type": "sym", "entity": "易破裂出血" }, { "start_idx": 135, "end_idx": 143, "type": "dis", "entity": "Galen静脉畸形" } ] }, { "text": "感染性脑动静脉畸形如颅内细菌性或真菌性动脉瘤,系感染性心内膜炎的感染栓子所致;人类免疫缺陷病毒感染也可导致小儿颅内动脉瘤的发生。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "感染性脑动静脉畸形" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "颅内细菌性" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "真菌性动脉瘤" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "栓子" }, { "start_idx": 39, "end_idx": 48, "type": "dis", "entity": "人类免疫缺陷病毒感染" }, { "start_idx": 53, "end_idx": 59, "type": "dis", "entity": "小儿颅内动脉瘤" } ] }, { "text": "外伤性脑动静脉畸形较少见,仅发生于海绵窦,因颈内动脉位于此处,故外伤可致颈动脉-海绵窦瘘。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "外伤性脑动静脉畸形" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "海绵窦" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "颈内动脉" }, { "start_idx": 36, "end_idx": 43, "type": "dis", "entity": "颈动脉-海绵窦瘘" } ] }, { "text": "其他类型的脑血管畸形有毛细血管扩张、海绵状血管瘤、软脑膜静脉及毛细血管的畸形、脑底异常血管网(Moyamoya病)等。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "脑血管畸形" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "毛细血管" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "毛细血管扩张" }, { "start_idx": 18, "end_idx": 23, "type": "sym", "entity": "海绵状血管瘤" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "软脑膜静脉" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "毛细血管" }, { "start_idx": 25, "end_idx": 37, "type": "sym", "entity": "软脑膜静脉及毛细血管的畸形" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "脑底异常血管网" }, { "start_idx": 47, "end_idx": 55, "type": "dis", "entity": "Moyamoya病" } ] }, { "text": "小儿特发性血小板减少性紫癜病例中发生ICH者占10%。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "dis", "entity": "小儿特发性血小板减少性紫癜" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "ICH" } ] }, { "text": "其他如白血病、再生障碍性贫血、溶血性贫血、弥散性血管内凝血、凝血障碍等血液病,以及抗凝疗法的并发症,均可发生ICH。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "白血病" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "再生障碍性贫血" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "弥散性血管内凝血" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "凝血障碍" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "血液病" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "抗凝疗法" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "ICH" } ] }, { "text": "找不到病因的脑出血称为小儿特发性脑出血。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑出血" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "小儿特发性脑出血" } ] }, { "text": "继发性缺乏可由吸收障碍或使用药物等引起,消耗过多和代谢活动增加也可引起缺乏。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "吸收障碍" } ] }, { "text": "溃疡好发于十二指肠和胃,但也可发生于食管、小肠及胃肠吻合口处,极少数发生于异位的胃黏膜,如Meckel憩室。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "溃疡" }, { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "十二指肠" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "胃" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "食管" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "小肠" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "胃肠吻合口" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "胃黏膜" } ] }, { "text": "呕血一般见于胃溃疡,吐出物呈咖啡样,而黑便较多见于十二指肠溃疡。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "胃溃疡" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "吐出物呈咖啡样" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "黑便" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "十二指肠溃疡" } ] }, { "text": "当出血量较多时,任何一种溃疡可同时表现呕血与黑便,在小儿胃内引流物呈血性多提示胃出血;但引流物阴性者,不能排除十二指肠溃疡合并出血的可能(因为血液可不经幽门反流入胃)。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "溃疡" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "呕血与黑便" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "胃出血" }, { "start_idx": 55, "end_idx": 60, "type": "dis", "entity": "十二指肠溃疡" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "出血" }, { "start_idx": 71, "end_idx": 72, "type": "bod", "entity": "血液" }, { "start_idx": 76, "end_idx": 77, "type": "bod", "entity": "幽门" }, { "start_idx": 81, "end_idx": 81, "type": "bod", "entity": "胃" } ] }, { "text": "幽门螺杆菌感染与NSAIDs/ASA诱发的胃炎是消化性溃疡的两大潜在因素,所以对幽门螺杆菌阳性的溃疡患者亦予以幽门螺杆菌根除疗法;如果可能,停用ASA/NSAIDs。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "幽门螺杆菌感染" }, { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "NSAIDs" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "ASA" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "胃炎" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "幽门螺杆菌阳性" }, { "start_idx": 55, "end_idx": 63, "type": "pro", "entity": "幽门螺杆菌根除疗法" }, { "start_idx": 72, "end_idx": 74, "type": "dis", "entity": "ASA" }, { "start_idx": 76, "end_idx": 81, "type": "dis", "entity": "NSAIDs" } ] }, { "text": "(2)特异性胆碱能神经阻滞剂:哌仑西平(pirenzepine)50~100mg每日2次,治疗4~6周,PU愈合率70%~94%(成人)。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dru", "entity": "特异性胆碱能神经阻滞剂" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "哌仑西平" }, { "start_idx": 20, "end_idx": 30, "type": "dru", "entity": "pirenzepine" }, { "start_idx": 52, "end_idx": 53, "type": "dis", "entity": "PU" } ] }, { "text": "(1)西咪替丁(cimetidine):儿童20~40mg/(kg•d),3~4次/日,亦有主张2次/日。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "西咪替丁" }, { "start_idx": 8, "end_idx": 17, "type": "dru", "entity": "cimetidine" } ] }, { "text": "(4)其他:尼扎替丁、罗沙替丁。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "尼扎替丁" }, { "start_idx": 11, "end_idx": 14, "type": "dru", "entity": "罗沙替丁" } ] }, { "text": "(3)胶体铋制剂:为溃疡隔离剂,保护黏膜,促进前列腺素合成,与表皮生长因子形成复合物,聚集于溃疡部位,促进上皮的再生和溃疡愈合,此外有杀灭幽门螺杆菌及抑制胃蛋白酶活性的作用。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "胶体铋制剂" }, { "start_idx": 10, "end_idx": 14, "type": "dru", "entity": "溃疡隔离剂" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "黏膜" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "前列腺素" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "溃疡" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "上皮" }, { "start_idx": 59, "end_idx": 60, "type": "dis", "entity": "溃疡" }, { "start_idx": 69, "end_idx": 73, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 77, "end_idx": 80, "type": "bod", "entity": "胃蛋白酶" } ] }, { "text": "(4)前列腺素E(PGE):人工合成的类似物有米索前列醇(misoprostol)等。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "前列腺素E" }, { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "PGE" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "米索前列醇" }, { "start_idx": 29, "end_idx": 39, "type": "dru", "entity": "misoprostol" } ] }, { "text": "肿瘤多位于鞍内及鞍上,球形或不规则形,边界清楚,实质性及部分囊性,囊液黄褐色,并有胆固醇结晶,囊壁及实质性部分可有钙化。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "鞍内" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "鞍上" }, { "start_idx": 41, "end_idx": 45, "type": "bod", "entity": "胆固醇结晶" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "囊壁" } ] }, { "text": "颅咽管瘤组织学上属良性肿瘤,但由于在鞍区以及对周围重要结构的浸润,临床上呈现为恶性肿瘤的表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "颅咽管瘤" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "鞍区" } ] }, { "text": "化学治疗目前尚无肯定疗效的药物。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "化学治疗" } ] }, { "text": "囊性肿瘤的预后比实质肿瘤或囊性、实质性混合肿瘤好,肿瘤完整切除比部分切除预后好,部分切除的肿瘤若辅以放疗,仍能获得较长的生存期。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "囊性肿瘤" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "实质肿瘤" }, { "start_idx": 13, "end_idx": 22, "type": "dis", "entity": "囊性、实质性混合肿瘤" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "肿瘤" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "肿瘤" }, { "start_idx": 50, "end_idx": 51, "type": "pro", "entity": "放疗" } ] }, { "text": "有人统计完整切除肿瘤10年生存率达24%~100%;部分切除肿瘤仅达31%~52%;若辅以放疗则可达到62%~84%。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肿瘤" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "肿瘤" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "放疗" } ] }, { "text": "该肿瘤好发于颈部,特别是颈后三角,其次可见于腋窝。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "颈部" }, { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "颈后三角" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "腋窝" } ] }, { "text": "还有人报道用OK-432,系一种经青霉素G钾盐处理而取得的人源性A簇链球菌Ⅲ型低毒菌株的冻干培养混合物。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dru", "entity": "OK-432" }, { "start_idx": 17, "end_idx": 22, "type": "dru", "entity": "青霉素G钾盐" }, { "start_idx": 29, "end_idx": 42, "type": "mic", "entity": "人源性A簇链球菌Ⅲ型低毒菌株" } ] }, { "text": "(2)上消化道出血:原因不明的黑便,呕血。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "上消化道出血" }, { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "原因不明的黑便,呕血" } ] }, { "text": "(4)消化性溃疡病:食管溃疡、胃溃疡及十二指肠溃疡。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "消化性溃疡病" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "食管溃疡" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "胃溃疡" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "十二指肠溃疡" } ] }, { "text": "(6)原因不明贫血:尤为低血色素小细胞性贫血。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "贫血" }, { "start_idx": 12, "end_idx": 21, "type": "sym", "entity": "低血色素小细胞性贫血" } ] }, { "text": "食管炎在胃镜下表现充血、水肿、糜烂及溃疡,根据其主要表现,在镜下可分为三类:卡他型、糜烂型与溃疡型。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "食管炎" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "充血" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "水肿" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "糜烂" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "溃疡" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "卡他型" }, { "start_idx": 42, "end_idx": 44, "type": "dis", "entity": "糜烂型" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "溃疡型" } ] }, { "text": "3)黏液增多:黏膜与黏液附着牢固,若用水冲去,可见黏膜表面发红或糜烂剥脱。", "entities": [ { "start_idx": 2, "end_idx": 35, "type": "sym", "entity": "黏液增多:黏膜与黏液附着牢固,若用水冲去,可见黏膜表面发红或糜烂剥脱" } ] }, { "text": "内镜部分同样要求明确炎症的部分(全胃炎、胃窦炎及胃体胃炎);对内镜所见进行分级,并根据其异常表现将胃炎分成7种基本类型:即充血渗出型、平坦糜烂型、隆起糜烂型、萎缩型、出血型、反流型以及皱襞增生型。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "全胃炎" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "胃窦炎" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "胃体胃炎" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "胃炎" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "充血渗出型" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "平坦糜烂型" }, { "start_idx": 73, "end_idx": 77, "type": "dis", "entity": "隆起糜烂型" }, { "start_idx": 79, "end_idx": 81, "type": "dis", "entity": "萎缩型" }, { "start_idx": 83, "end_idx": 85, "type": "dis", "entity": "出血型" }, { "start_idx": 87, "end_idx": 89, "type": "dis", "entity": "反流型" }, { "start_idx": 92, "end_idx": 96, "type": "dis", "entity": "皱襞增生型" } ] }, { "text": "HSPN好发于学龄儿童,男多于女,是儿童最常见的继发性肾小球疾病。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "HSPN" }, { "start_idx": 24, "end_idx": 31, "type": "dis", "entity": "继发性肾小球疾病" } ] }, { "text": "其基本病变是肾小球系膜区IgA沉积、系膜细胞增生伴或不伴新月体形成。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "肾小球系膜区" }, { "start_idx": 6, "end_idx": 16, "type": "sym", "entity": "肾小球系膜区IgA沉积" }, { "start_idx": 18, "end_idx": 21, "type": "bod", "entity": "系膜细胞" }, { "start_idx": 18, "end_idx": 32, "type": "sym", "entity": "系膜细胞增生伴或不伴新月体形成" } ] }, { "text": "肾损害多发生于出现皮肤紫癜的3个月内(95%),尽管有报道肾损害可出现在皮肤紫癜之前以及在皮肤紫癜1年之后,但在6个月后出现肾损害一般不应轻易视之为紫癜性肾炎。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肾损害" }, { "start_idx": 9, "end_idx": 12, "type": "dis", "entity": "皮肤紫癜" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "肾损害" }, { "start_idx": 36, "end_idx": 39, "type": "dis", "entity": "皮肤紫癜" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "皮肤紫癜" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "肾损害" }, { "start_idx": 74, "end_idx": 78, "type": "dis", "entity": "紫癜性肾炎" } ] }, { "text": "补体系统的激活,产生一系列炎症介质,导致局部炎性改变,继之发生凝血和纤溶系统障碍,出现小血管内血栓形成和纤维蛋白的沉积,最终导致肾小球损伤。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "补体系统" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "局部炎性改变" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "凝血" }, { "start_idx": 34, "end_idx": 37, "type": "bod", "entity": "纤溶系统" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "纤溶系统障碍" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "小血管" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "小血管内血栓形成" }, { "start_idx": 52, "end_idx": 58, "type": "sym", "entity": "纤维蛋白的沉积" }, { "start_idx": 64, "end_idx": 68, "type": "dis", "entity": "肾小球损伤" } ] }, { "text": "皮疹初为鲜红色,略高出皮面,可伴痒感及风团,并反复成批出现。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "皮疹初为鲜红色" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "略高出皮面" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "痒感及风团" }, { "start_idx": 23, "end_idx": 28, "type": "sym", "entity": "反复成批出现" } ] }, { "text": "3.急性肾炎型。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "急性肾炎型" } ] }, { "text": "4.肾病综合征型。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "肾病综合征型" } ] }, { "text": "6.慢性肾炎型。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "慢性肾炎型" } ] }, { "text": "由于HSPN在急性期有特征性出血性皮疹、腹痛、肠出血、关节炎和肾炎等特点,因此不难诊断。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "HSPN" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "特征性出血性皮疹" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "腹痛" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "肠" }, { "start_idx": 23, "end_idx": 25, "type": "sym", "entity": "肠出血" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "关节炎" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "肾炎" } ] }, { "text": "当临床表现不典型时,应与急性肾小球肾炎、IgA肾病、狼疮性肾炎和急性间质性肾炎相鉴别。", "entities": [ { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "急性肾小球肾炎" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "狼疮性肾炎" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "急性间质性肾炎" } ] }, { "text": "HSP肾炎和IgA肾病尽管免疫发病机制相似,但临床上有明显区别,IgA肾病缺乏HSP肾炎的肾外表现。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "HSP肾炎" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 39, "end_idx": 43, "type": "dis", "entity": "HSP肾炎" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "肾" } ] }, { "text": "HSP肾炎呈急性发病过程,临床表现轻重不一,病程较短,其肾损伤的程度取决于肾小球新月体的多少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "HSP肾炎" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肾损伤" } ] }, { "text": "而IgA肾病呈慢性持续性发展,较易发展为肾功能不全,新月体形成不甚明显,而节段性肾小球硬化较为突出。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "IgA肾病" }, { "start_idx": 20, "end_idx": 22, "type": "ite", "entity": "肾功能" }, { "start_idx": 26, "end_idx": 34, "type": "sym", "entity": "新月体形成不甚明显" }, { "start_idx": 37, "end_idx": 44, "type": "bod", "entity": "节段性肾小球硬化" } ] }, { "text": "无明确过敏原者,应注意有无感染或隐性感染,可同时抗过敏治疗。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "感染" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "隐性感染" }, { "start_idx": 24, "end_idx": 28, "type": "pro", "entity": "抗过敏治疗" } ] }, { "text": "一般用于紫癜性肾炎临床上大量蛋白尿的病人,对于病理Ⅲ级以上病人也应积极应用肾上腺皮质激素治疗,可显著改善预后,可选用泼尼松,剂量为2mg/(kg•d),疗程1~2周。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "紫癜性肾炎" }, { "start_idx": 14, "end_idx": 16, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 37, "end_idx": 43, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 58, "end_idx": 60, "type": "dru", "entity": "泼尼松" } ] }, { "text": "维生素E也有部分治疗作用,Ⅳ~Ⅴ期病人还可试用血浆置换及免疫吸附,有望获得肾功能的改善。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "维生素E" }, { "start_idx": 23, "end_idx": 26, "type": "pro", "entity": "血浆置换" }, { "start_idx": 28, "end_idx": 31, "type": "pro", "entity": "免疫吸附" }, { "start_idx": 37, "end_idx": 39, "type": "ite", "entity": "肾功能" } ] }, { "text": "临床上表现为肾病综合征和/或肾炎综合征的患儿,病理上Ⅲb级以上患儿,常遗留下持续的肾损害,并最终导致肾功能不全。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肾病" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "肾病综合征" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肾炎" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "肾炎综合征" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "肾损害" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "肾功能不全" } ] }, { "text": "近年的研究认为幽门螺杆菌的胃内感染是引起慢性胃炎最重要的因素,其产生的机制与黏膜的破坏和保护因素之间失去平衡有关。", "entities": [ { "start_idx": 7, "end_idx": 16, "type": "dis", "entity": "幽门螺杆菌的胃内感染" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "黏膜" } ] }, { "text": "感染幽门螺杆菌后,胃部病理形态改变主要是胃窦黏膜小结节,小颗粒隆起,组织学显示淋巴细胞增多,淋巴滤泡形成,用药物将幽门螺杆菌清除后胃黏膜炎症明显改善。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 20, "end_idx": 26, "type": "sym", "entity": "胃窦黏膜小结节" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "小颗粒隆起" }, { "start_idx": 39, "end_idx": 44, "type": "sym", "entity": "淋巴细胞增多" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "淋巴滤泡形成" }, { "start_idx": 57, "end_idx": 61, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "胃黏膜炎症" } ] }, { "text": "此外成人健康志愿者口服幽门螺杆菌证实可引发胃黏膜的慢性炎症,并出现上腹部痛、恶心及呕吐等症状;用幽门螺杆菌感染动物的动物模型也获得了成功,因此幽门螺杆菌是慢性胃炎的一个重要病因。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "慢性炎症" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "上腹部痛" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "恶心" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "呕吐" }, { "start_idx": 48, "end_idx": 52, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 71, "end_idx": 75, "type": "mic", "entity": "幽门螺杆菌" }, { "start_idx": 77, "end_idx": 80, "type": "dis", "entity": "慢性胃炎" } ] }, { "text": "有报道40%的慢性扁桃腺炎患者其胃内有卡他性改变。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "慢性扁桃腺炎" }, { "start_idx": 16, "end_idx": 23, "type": "sym", "entity": "胃内有卡他性改变" } ] }, { "text": "急性胃炎之后胃黏膜损伤经久不愈,反复发作亦可发展为慢性胃炎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "急性胃炎" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "胃黏膜损伤" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "慢性胃炎" } ] }, { "text": "胆盐可减低胃黏膜屏障对氢离子的通透性,并使胃窦部G细胞释放胃泌素,增加胃酸分泌,氢离子通过损伤的黏膜屏障并弥散进入胃黏膜引起炎症变化、血管扩张及炎性渗出增多,使慢性胃炎持续存在。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "胆盐" }, { "start_idx": 3, "end_idx": 17, "type": "sym", "entity": "减低胃黏膜屏障对氢离子的通透性" }, { "start_idx": 21, "end_idx": 31, "type": "sym", "entity": "胃窦部G细胞释放胃泌素" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "增加胃酸分泌" }, { "start_idx": 45, "end_idx": 51, "type": "bod", "entity": "损伤的黏膜屏障" }, { "start_idx": 57, "end_idx": 59, "type": "bod", "entity": "胃黏膜" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "炎症变化" }, { "start_idx": 67, "end_idx": 70, "type": "sym", "entity": "血管扩张" }, { "start_idx": 72, "end_idx": 77, "type": "sym", "entity": "炎性渗出增多" }, { "start_idx": 80, "end_idx": 83, "type": "dis", "entity": "慢性胃炎" } ] }, { "text": "幼儿腹痛可仅表现不安和正常进食行为改变,年长儿症状似成人,常诉上腹痛,其次有嗳气、早饱、恶心、上腹部不适及泛酸。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "腹痛" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "表现不安" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "正常进食行为改变" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "上腹痛" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "嗳气" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "早饱" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "恶心" }, { "start_idx": 47, "end_idx": 51, "type": "sym", "entity": "上腹部不适" }, { "start_idx": 53, "end_idx": 54, "type": "sym", "entity": "泛酸" } ] }, { "text": "部分患儿可有食欲缺乏、乏力、消瘦及头晕,伴有胃糜烂者可出现黑便。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "乏力" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "消瘦" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "头晕" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "胃糜烂" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "黑便" } ] }, { "text": "虽然过去多数放射学者认为,胃紧张度的障碍、蠕动的改变及空腹胃内的胃液,可作为诊断胃炎的依据,但近年胃镜检查发现,这种现象系胃动力异常而并非胃炎所致。", "entities": [ { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "胃炎" }, { "start_idx": 49, "end_idx": 52, "type": "equ", "entity": "胃镜检查" }, { "start_idx": 61, "end_idx": 61, "type": "bod", "entity": "胃" }, { "start_idx": 69, "end_idx": 70, "type": "dis", "entity": "胃炎" } ] }, { "text": "根据有无腺体萎缩诊断为慢性浅表性胃炎或慢性萎缩性胃炎。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "腺体" }, { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "慢性浅表性胃炎" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "慢性萎缩性胃炎" } ] }, { "text": "鉴别诊断:在慢性胃炎发作期时,可通过胃镜、B超、24小时pH监测综合检查,排除肝、胆、胰、消化性溃疡及反流性食管炎。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "慢性胃炎" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "胃镜" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "B超" }, { "start_idx": 24, "end_idx": 35, "type": "pro", "entity": "24小时pH监测综合检查" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "消化性溃疡" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "反流性食管炎" } ] }, { "text": "在胃炎发作期,应注意与胃穿孔或阑尾炎早期鉴别。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "胃炎" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "胃穿孔" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "阑尾炎" } ] }, { "text": "慢性胃炎尚无特殊疗法,无症状者无需治疗。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "慢性胃炎" } ] }, { "text": "3.有腹胀、恶心、呕吐者,给予胃动力药物,如多潘立酮及西沙比利等。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "腹胀" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "恶心" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "呕吐" }, { "start_idx": 22, "end_idx": 25, "type": "dru", "entity": "多潘立酮" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "西沙比利" } ] }, { "text": "有头晕、乏力、衰弱、食欲减退和长达数月至数年的贫血症,部分病例体重减轻。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "头晕" }, { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "乏力" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "衰弱" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "食欲减退" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "贫血症" } ] }, { "text": "并发症以出血和感染多见,在未转变为急性白血病的病例中,大多死于这两个原因,两者的发生率约分别为20%和39%。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "出血" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "感染" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "急性白血病" } ] }, { "text": "感染中以下呼吸道感染为多见,约占60%~70%,其他可表现为肛门、会阴部感染,脓疱症和败血症等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "感染" }, { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "呼吸道感染" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "肛门" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "脓疱症" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "败血症" } ] }, { "text": "MDS有原发和继发于治疗相关MDS之分,儿童原发性MDS可进一步分为难治性血细胞减少症(RC)、难治性贫血伴幼稚细胞增多(RAEB)、难治性贫血伴幼稚细胞增多向白细胞转化(RAEBT)。", "entities": [ { "start_idx": 34, "end_idx": 42, "type": "dis", "entity": "难治性血细胞减少症" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "RC" }, { "start_idx": 48, "end_idx": 52, "type": "dis", "entity": "难治性贫血" }, { "start_idx": 54, "end_idx": 59, "type": "sym", "entity": "幼稚细胞增多" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "RAEB" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "难治性贫血" }, { "start_idx": 73, "end_idx": 76, "type": "bod", "entity": "幼稚细胞" }, { "start_idx": 80, "end_idx": 82, "type": "bod", "entity": "白细胞" } ] }, { "text": "皮肤损害症状明显,特别是面部皮疹是常见而重要的体征之一,多数患儿脾大,部分患儿肝脏和淋巴结增大。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "皮肤" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "面部" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "皮疹" } ] }, { "text": "外周血白细胞和单核细胞增多,贫血,血小板减少,常见幼稚红细胞和幼稚粒细胞,骨髓呈增生性特征。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "外周血" }, { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "白细胞" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "单核细胞" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "贫血" }, { "start_idx": 31, "end_idx": 35, "type": "bod", "entity": "幼稚粒细胞" } ] }, { "text": "造血干细胞移植是目前唯一可以根治MDS的治疗方法。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "造血干细胞移植" } ] }, { "text": "不良反应为皮肤黏膜干燥,ALT增高,颅压增高等。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "皮肤黏膜干燥" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "ALT增高" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "颅压增高" } ] }, { "text": "目前认为,HHV-6是该病的主要病因,但并不是唯一的病原。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "mic", "entity": "HHV-6" } ] }, { "text": "此期除有食欲减退、不安或轻咳外,体征不明显,仅有咽部和扁桃体轻度充血和头颈部浅表淋巴结轻度肿大。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "食欲减退" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "不安" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "轻咳" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "咽部" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "扁桃体" }, { "start_idx": 24, "end_idx": 33, "type": "sym", "entity": "咽部和扁桃体轻度充血" }, { "start_idx": 35, "end_idx": 42, "type": "bod", "entity": "头颈部浅表淋巴结" }, { "start_idx": 35, "end_idx": 46, "type": "sym", "entity": "头颈部浅表淋巴结轻度肿大" } ] }, { "text": "首现于躯干,然后迅速波及颈、上肢、脸和下肢。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "躯干" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "颈" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "上肢" }, { "start_idx": 17, "end_idx": 17, "type": "bod", "entity": "脸" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "下肢" } ] }, { "text": "皮疹持续24~48小时很快消退,无色素沉着,也不脱皮。", "entities": [ { "start_idx": 0, "end_idx": 25, "type": "sym", "entity": "皮疹持续24~48小时很快消退,无色素沉着,也不脱皮" } ] }, { "text": "也可随后出现白细胞总数增多。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "ite", "entity": "白细胞总数" }, { "start_idx": 6, "end_idx": 12, "type": "sym", "entity": "白细胞总数增多" } ] }, { "text": "确诊有赖于肺活检。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "肺活检" } ] }, { "text": "病毒主要通过空气飞沫传播。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" } ] }, { "text": "常有高热、中度中毒征象和流涕。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "高热" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "中毒" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "中度中毒征象" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "流涕" } ] }, { "text": "此外,可见腹泻和皮疹。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "腹泻" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "皮疹" } ] }, { "text": "肺部可闻干啰音。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "肺部可闻干啰音" } ] }, { "text": "偶有鼻出血。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "鼻" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "偶有鼻出血" } ] }, { "text": "1/3患者出现腹泻水样便。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "1/3患者出现腹泻水样便" } ] }, { "text": "甲、乙型流感临床症状相似,但后者全身症状轻,鼻及眼部症状明显。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "甲、乙型流感" }, { "start_idx": 22, "end_idx": 22, "type": "bod", "entity": "鼻" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "眼部" } ] }, { "text": "个别患者发生急性肌炎,以腓肠肌和比目鱼肌受累多见,常发生于病后1周,临床症状改善不久。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "急性肌炎" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "腓肠肌" }, { "start_idx": 16, "end_idx": 19, "type": "bod", "entity": "比目鱼肌" }, { "start_idx": 12, "end_idx": 23, "type": "sym", "entity": "腓肠肌和比目鱼肌受累多见" } ] }, { "text": "X线检查双肺有散在絮状或结节状阴影,由肺门向四周扩散。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "X线检查" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "肺" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肺门" }, { "start_idx": 4, "end_idx": 25, "type": "sym", "entity": "双肺有散在絮状或结节状阴影,由肺门向四周扩散" } ] }, { "text": "多于5~10日内因呼吸与循环衰竭而死亡,病死率高达80%以上。", "entities": [ { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "呼吸与循环衰竭" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "死亡" } ] }, { "text": "3.扎那米韦(zanamivir)为NA抑制剂。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "扎那米韦" }, { "start_idx": 7, "end_idx": 15, "type": "dru", "entity": "zanamivir" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "NA抑制剂" } ] }, { "text": "对高热烦躁者给予解热镇静剂,避免使用阿司匹林(因其可能诱发Reye综合征)。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "sym", "entity": "高热烦躁" }, { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "解热镇静剂" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "Reye综合征" } ] }, { "text": "在新生儿或小婴儿时期,如先天性(原始)反射不出现,或表现不对称,或3~4个月以上仍持续存在,均提示可能存在神经系统异常。", "entities": [ { "start_idx": 53, "end_idx": 56, "type": "bod", "entity": "神经系统" } ] }, { "text": "二度房室传导阻滞:部分心房激动不能传至心室。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "二度房室传导阻滞" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "心房" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心室" } ] }, { "text": "莫氏Ⅱ型有发生晕厥的可能,并有可能进展。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "晕厥" } ] }, { "text": "毒蛇咬伤后的症状常因被咬者的年龄、体质、被咬部位、毒液量多少以及毒蛇的种类等因素决定,一般在15~20分钟内出现症状。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "咬伤" } ] }, { "text": "我国常见的毒蛇有眼镜王蛇、银环蛇、金环蛇、竹叶青蛇、蝮蛇等,毒蛇咬伤多发生在夏季并以南方地区多见。", "entities": [ { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "咬伤" } ] }, { "text": "神经毒素早期主要表现局部症状轻微,不易引起重视,以后发展头晕、嗜睡、无力、吞咽困难、声音嘶哑、肌肉麻痹、四肢瘫痪、呼吸麻痹、昏迷和死亡。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "头晕" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "嗜睡" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "无力" }, { "start_idx": 37, "end_idx": 40, "type": "sym", "entity": "吞咽困难" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "肌肉" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "肌肉麻痹" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "四肢" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "四肢瘫痪" }, { "start_idx": 57, "end_idx": 58, "type": "ite", "entity": "呼吸" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "呼吸麻痹" }, { "start_idx": 62, "end_idx": 63, "type": "sym", "entity": "昏迷" }, { "start_idx": 65, "end_idx": 66, "type": "sym", "entity": "死亡" } ] }, { "text": "尽快排毒,紧急情况下可用口吸吮,边吸边吮,并用清水漱口。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "可用口吸吮" } ] }, { "text": "作十字切口并用吸引器吸出毒液,扩创排毒。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "equ", "entity": "吸引器" } ] }, { "text": "也可用各种蛇药外敷,切不可用酸类或碘烧灼伤口。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dru", "entity": "蛇药" } ] }, { "text": "常表现为颅内压增高症状,如烦躁、恶心、呕吐、嗜睡、食欲减退、复视、视神经乳头水肿,囟门未闭者则囟门饱满。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "颅内压增高" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "烦躁" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "恶心" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "呕吐" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "嗜睡" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "食欲减退" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "复视" }, { "start_idx": 33, "end_idx": 37, "type": "bod", "entity": "视神经乳头" }, { "start_idx": 33, "end_idx": 39, "type": "sym", "entity": "视神经乳头水肿" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "囟门饱满" } ] }, { "text": "发病的同时常伴有荨麻疹、多形红斑、血管性水肿或关节肿痛。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "荨麻疹" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "多形红斑" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "血管性水肿" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "关节肿痛" } ] }, { "text": "临床表现为双下肢大小不等的皮肤瘀点、瘀斑,多自发发生和消退,很少有其他部位的出血;除了部分患者毛细血管脆性试验阳性外,其余出凝血检查及血小板计数等均正常。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "皮肤" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "皮肤瘀点" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "瘀斑" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "毛细血管" }, { "start_idx": 67, "end_idx": 71, "type": "pro", "entity": "血小板计数" } ] }, { "text": "本病初期仅见毛囊周围的瘀点、瘀斑,齿龈肿胀、出血,病情进展时可出现鼻出血、血尿、黑便,肌肉及关节腔出血等。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "毛囊" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "瘀点" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "瘀斑" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "齿龈" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "齿龈肿胀" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "出血" }, { "start_idx": 33, "end_idx": 33, "type": "bod", "entity": "鼻" }, { "start_idx": 33, "end_idx": 35, "type": "sym", "entity": "鼻出血" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "血尿" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "黑便" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "肌肉" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "关节腔" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "肌肉及关节腔出血" } ] }, { "text": "本病束臂试验阳性,凝血功能检查正常,X线及活体组织电镜检查有助确诊。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "equ", "entity": "电镜" } ] }, { "text": "血片中可见血小板成堆及巨大血小板,骨髓巨核细胞增生。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "血小板" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "血小板" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "骨髓" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "巨核细胞" } ] }, { "text": "本病少见,须与慢性粒细胞白血病、真性红细胞增多症、骨髓纤维化及继发性血小板增多症等鉴别。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "dis", "entity": "慢性粒细胞白血病" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "真性红细胞增多症" }, { "start_idx": 31, "end_idx": 39, "type": "dis", "entity": "继发性血小板增多症" } ] }, { "text": "实验室检查可见血小板数量和形态正常,出血时间(Ivy法)延长或阿司匹林耐量试验阳性,血小板黏附率、Ⅷ∶C降低或正常、APTT延长或正常,血小板对瑞斯托霉素诱导的聚集反应降低,vWF的抗原(vWF∶Ag)减低或正常(正常者需进一步检查是否变异型)。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "血小板" }, { "start_idx": 23, "end_idx": 26, "type": "ite", "entity": "Ivy法" }, { "start_idx": 31, "end_idx": 34, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 42, "end_idx": 47, "type": "ite", "entity": "血小板黏附率" }, { "start_idx": 49, "end_idx": 51, "type": "ite", "entity": "Ⅷ∶C" }, { "start_idx": 58, "end_idx": 61, "type": "ite", "entity": "APTT" }, { "start_idx": 68, "end_idx": 70, "type": "bod", "entity": "血小板" }, { "start_idx": 72, "end_idx": 76, "type": "dru", "entity": "瑞斯托霉素" }, { "start_idx": 87, "end_idx": 89, "type": "bod", "entity": "vWF" } ] }, { "text": "早发型多在生后24小时内发病,经典型于生后2~3天发病,晚发型多于生后1个月后发病,临床可见鼻出血、脐残端出血、尿血、便血和颅内出血等。", "entities": [ { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "鼻" }, { "start_idx": 46, "end_idx": 48, "type": "sym", "entity": "鼻出血" }, { "start_idx": 50, "end_idx": 50, "type": "bod", "entity": "脐" }, { "start_idx": 50, "end_idx": 54, "type": "sym", "entity": "脐残端出血" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "尿血" }, { "start_idx": 59, "end_idx": 60, "type": "sym", "entity": "便血" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "颅内" }, { "start_idx": 62, "end_idx": 65, "type": "sym", "entity": "颅内出血" } ] }, { "text": "DIC首先出现短时间的高凝状态,消耗大量凝血因子和血小板,形成广泛的微小血栓;接着呈现消耗性低凝状态和继发性纤溶亢进;临床表现为出血、栓塞、溶血及微循环障碍等,病势凶险。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "DIC" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "凝血因子" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "血小板" }, { "start_idx": 29, "end_idx": 37, "type": "sym", "entity": "形成广泛的微小血栓" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "消耗性低凝状态" }, { "start_idx": 51, "end_idx": 57, "type": "sym", "entity": "继发性纤溶亢进" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "出血" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "栓塞" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "溶血" }, { "start_idx": 73, "end_idx": 77, "type": "sym", "entity": "微循环障碍" } ] }, { "text": "继发性EH是指有某些病理因素所致的EH,可见于早产、缺氧缺血性脑病、颅内出血、高胆红素血症、化脓性脑膜炎以及维生素A缺乏症等。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "继发性EH" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "EH" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "缺氧缺血性脑病" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "颅内出血" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "高胆红素血症" }, { "start_idx": 46, "end_idx": 51, "type": "dis", "entity": "化脓性脑膜炎" }, { "start_idx": 54, "end_idx": 60, "type": "dis", "entity": "维生素A缺乏症" } ] }, { "text": "有颅内压增高者,可用碳酸酐酶抑制剂如乙酰唑胺(diamox),或脱水剂如甘露醇,以降低颅内压。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dru", "entity": "碳酸酐酶抑制剂" }, { "start_idx": 18, "end_idx": 21, "type": "dru", "entity": "乙酰唑胺" }, { "start_idx": 23, "end_idx": 28, "type": "dru", "entity": "diamox" }, { "start_idx": 32, "end_idx": 34, "type": "dru", "entity": "脱水剂" }, { "start_idx": 36, "end_idx": 38, "type": "dru", "entity": "甘露醇" } ] }, { "text": "不主张采用前囟穿刺放液或脑脊液分流术。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "前囟穿刺" }, { "start_idx": 12, "end_idx": 17, "type": "pro", "entity": "脑脊液分流术" } ] }, { "text": "如有频繁惊厥发作者,可适当短期内给予抗癫痫药物。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "惊厥" }, { "start_idx": 18, "end_idx": 22, "type": "dru", "entity": "抗癫痫药物" } ] }, { "text": "少部分患儿可能发展成为交通性脑积水。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "交通性脑积水" } ] }, { "text": "近年有学者追踪随访,发现少数患儿可出现轻度的神经精神发育障碍,如运动发育迟缓和语言发育迟缓等,值得进一步研究。", "entities": [ { "start_idx": 22, "end_idx": 29, "type": "dis", "entity": "神经精神发育障碍" }, { "start_idx": 32, "end_idx": 37, "type": "dis", "entity": "运动发育迟缓" }, { "start_idx": 39, "end_idx": 44, "type": "dis", "entity": "语言发育迟缓" } ] }, { "text": "出生后感染性肺炎的病理改变以支气管肺炎和间质性肺炎为主,病变分散,影响一叶或数叶,有时融合成大片病灶,肺不张和肺气肿较易发生。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "出生后感染性肺炎" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "支气管肺炎" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "间质性肺炎" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "肺不张" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "肺气肿" } ] }, { "text": "金黄色葡萄球菌肺炎在新生儿室中常有发生,并可引起流行;患儿中毒症状重,易并发化脓性脑膜炎、脓胸、脓气胸、肺大疱等。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "mic", "entity": "金黄色葡萄球菌肺炎" }, { "start_idx": 38, "end_idx": 43, "type": "dis", "entity": "化脓性脑膜炎" }, { "start_idx": 45, "end_idx": 46, "type": "dis", "entity": "脓胸" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "脓气胸" }, { "start_idx": 52, "end_idx": 54, "type": "dis", "entity": "肺大疱" } ] }, { "text": "大肠埃希菌肺炎时患儿有神萎、不吃、不哭、体温低、呼吸窘迫等,脓胸的液体黏稠,有臭味。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "大肠埃希菌肺炎" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "神萎" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "不吃" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "不哭" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "体温低" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "呼吸窘迫" }, { "start_idx": 30, "end_idx": 31, "type": "dis", "entity": "脓胸" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "液体黏稠" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "臭味" } ] }, { "text": "X线胸片有时表现为弥漫性、深浅不一的模糊影,也可表现为两肺广泛点状或大小不一的浸润影,常伴肺气肿、肺不张,偶见大叶实变伴脓胸、脓气胸或肺大疱。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "X线胸片" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "肺气肿" }, { "start_idx": 49, "end_idx": 51, "type": "dis", "entity": "肺不张" }, { "start_idx": 60, "end_idx": 61, "type": "dis", "entity": "脓胸" }, { "start_idx": 63, "end_idx": 65, "type": "dis", "entity": "脓气胸" }, { "start_idx": 67, "end_idx": 69, "type": "dis", "entity": "肺大疱" } ] }, { "text": "重症并发呼吸衰竭者,可用持续正压呼吸或气管插管后机械通气。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "呼吸衰竭" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "正压呼吸" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "机械通气" } ] }, { "text": "病毒性肺炎可用利巴韦林或干扰素治疗,单纯疱疹病毒可用阿昔洛韦静脉滴注。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "病毒性肺炎" }, { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "利巴韦林" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "干扰素" }, { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "疱疹病毒" }, { "start_idx": 26, "end_idx": 33, "type": "pro", "entity": "阿昔洛韦静脉滴注" } ] }, { "text": "衣原体肺炎首选红霉素治疗。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "衣原体肺炎" }, { "start_idx": 7, "end_idx": 9, "type": "dru", "entity": "红霉素" } ] }, { "text": "只具备内脏易位和支气管扩张者,称为不全性Kartagener综合征。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "内脏易位" }, { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "支气管扩张" }, { "start_idx": 17, "end_idx": 32, "type": "dis", "entity": "不全性Kartagener综合征" } ] }, { "text": "主要症状为随年龄增加而加重的咳嗽、咳痰和咯血,以早晨更明显,伴头昏、流涕等。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "咳嗽" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "咳痰" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "咯血" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "头昏" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "流涕" } ] }, { "text": "易患感冒及肺炎,出现呼吸困难、发绀、说话带鼻音,体格检查可发现杵状指趾、肺部啰音等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "感冒" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肺炎" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "发绀" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "说话带鼻音" }, { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "体格检查" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "指趾" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "杵状指趾" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "肺部" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "肺部啰音" } ] }, { "text": "胸片及支气管造影可协助诊断。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "胸片" }, { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "支气管造影" } ] }, { "text": "断层X线片有时可见到支气管扩张和变形。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "断层X线片" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "支气管" }, { "start_idx": 10, "end_idx": 17, "type": "sym", "entity": "支气管扩张和变形" } ] }, { "text": "应鼓励咳嗽,肺部物理治疗有助于排痰。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "咳嗽" }, { "start_idx": 6, "end_idx": 11, "type": "pro", "entity": "肺部物理治疗" } ] }, { "text": "随着分子生物学的进展,LGMD有了新的分类,主要分为两类:LGMD1为常染色体显性遗传,LGMD2为常染色体隐性遗传(表16-16)。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "LGMD" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "LGMD1" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "LGMD2" } ] }, { "text": "腓肠肌肥大是常染色体隐性遗传型的LGMD常见的表现。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "腓肠肌肥大" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "LGMD" } ] }, { "text": "行走困难者可以使用轮椅,在有些伴有危及生命的心肌病患者中,需安装心脏起搏器,甚至心脏移植以挽救生命。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "equ", "entity": "轮椅" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "心肌病" }, { "start_idx": 32, "end_idx": 36, "type": "bod", "entity": "心脏起搏器" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "心脏移植" } ] }, { "text": "基因治疗LGMD的方法很多,与dystrophin缺陷相比,本病基因治疗有潜在的优势,例如在肌聚糖病中,基因较小且较易转入载体,重组腺病毒载体(rAAV)系统由于其能在非分裂细胞中有效地、长期地表达,因此具有很大的优势。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "基因治疗" }, { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "LGMD" }, { "start_idx": 15, "end_idx": 26, "type": "dis", "entity": "dystrophin缺陷" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "肌聚糖病" }, { "start_idx": 64, "end_idx": 70, "type": "bod", "entity": "重组腺病毒载体" }, { "start_idx": 72, "end_idx": 75, "type": "bod", "entity": "rAAV" }, { "start_idx": 87, "end_idx": 88, "type": "bod", "entity": "细胞" } ] }, { "text": "恙虫病在我国古代称为沙虱热,在西方国家常称为丛林斑疹伤寒。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "恙虫病" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "沙虱热" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "丛林斑疹伤寒" } ] }, { "text": "起病后患儿出现咽痛、高热、吞咽困难,常言语不清。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "咽痛" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "高热" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "吞咽困难" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "言语不清" } ] }, { "text": "偶可导致斜颈。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "斜颈" } ] }, { "text": "年幼患儿需住院治疗。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "住院治疗" } ] }, { "text": "抗生素首选青霉素,常需切开引流,否则可发生破裂。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "抗生素" }, { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "青霉素" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "切开引流" } ] }, { "text": "颅内出血进行头颅B超或CT检查。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "颅内出血" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "头颅" }, { "start_idx": 8, "end_idx": 9, "type": "pro", "entity": "B超" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "CT检查" } ] }, { "text": "心脏骤停患儿,小儿呼吸、心搏骤停多由于气道阻塞和缺氧,先引起呼吸骤停,继而心搏骤停。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "心脏骤停" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "呼吸、心搏骤停" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "气道阻塞" }, { "start_idx": 24, "end_idx": 25, "type": "dis", "entity": "缺氧" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "呼吸骤停" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "心搏骤停" } ] }, { "text": "从小儿呼吸心搏骤停的发生年龄来看,3~6个月小婴儿心搏骤停多见于窒息、呼吸道阻塞和婴儿猝死综合征,1~5岁多由心脏疾患、呼吸系统和中枢神经系统疾患引起,7~8岁多为意外事故,特别常见于外伤。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "呼吸心搏骤停" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "心搏骤停" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "窒息" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "呼吸道阻塞" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "婴儿猝死综合征" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "心脏疾患" }, { "start_idx": 60, "end_idx": 72, "type": "dis", "entity": "呼吸系统和中枢神经系统疾患" }, { "start_idx": 92, "end_idx": 93, "type": "dis", "entity": "外伤" } ] }, { "text": "分为上尿路和下尿路感染。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "上尿路和下尿路感染" } ] }, { "text": "前者指肾盂肾炎,后者指膀胱炎和尿道炎。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "肾盂肾炎" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "膀胱炎" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "尿道炎" } ] }, { "text": "有器械操作诱发尿感的细菌可为肠道细菌和绿脓杆菌。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "尿感" }, { "start_idx": 14, "end_idx": 17, "type": "mic", "entity": "肠道细菌" }, { "start_idx": 19, "end_idx": 22, "type": "mic", "entity": "绿脓杆菌" } ] }, { "text": "在泌尿道梗阻、结构异常、尿路结石、膀胱输尿管反流和神经源性膀胱的基础上并发的尿感可为一种以上细菌的混合感染。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "泌尿道梗阻" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "结构异常" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "尿路结石" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "尿感" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "感染" } ] }, { "text": "病毒感染,特别是腺病毒可引起出血性膀胱炎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "病毒感染" }, { "start_idx": 8, "end_idx": 10, "type": "mic", "entity": "腺病毒" }, { "start_idx": 14, "end_idx": 19, "type": "dis", "entity": "出血性膀胱炎" } ] }, { "text": "真菌感染可能继发于糖尿病的留置导尿、免疫缺陷病或类固醇、广谱抗生素或其他免疫抑制剂的治疗过程中。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "真菌感染" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "糖尿病" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "留置导尿" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "免疫缺陷病" }, { "start_idx": 28, "end_idx": 32, "type": "dru", "entity": "广谱抗生素" }, { "start_idx": 36, "end_idx": 40, "type": "dru", "entity": "免疫抑制剂" } ] }, { "text": "同时还有全身不适、精神萎靡、面色苍黄、呕吐、恶心及轻度腹泻,年长儿述胁肋部或腰痛,肾区叩击痛。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "全身不适" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "精神萎靡" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "面色苍黄" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "呕吐" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "恶心" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "轻度腹泻" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "胁肋部或腰" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "胁肋部或腰痛" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "肾区" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "肾区叩击痛" } ] }, { "text": "新生儿表现如败血症,有体重下降、喂养困难、黄疸、激惹、发热或体温不升。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "败血症" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "体重下降" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "喂养困难" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "黄疸" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "激惹" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "发热" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "体温不升" } ] }, { "text": "对<5岁的第一次尿感应做排泄性膀胱尿道造影,以发现膀胱输尿管反流及后尿道瓣膜等尿感诱因。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "尿感" }, { "start_idx": 12, "end_idx": 20, "type": "pro", "entity": "排泄性膀胱尿道造影" }, { "start_idx": 25, "end_idx": 29, "type": "bod", "entity": "膀胱输尿管" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "膀胱输尿管反流" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "后尿道" }, { "start_idx": 33, "end_idx": 37, "type": "sym", "entity": "后尿道瓣膜" }, { "start_idx": 39, "end_idx": 40, "type": "dis", "entity": "尿感" } ] }, { "text": "上尿路感染者还应做泌尿系统B超检查,<5岁患儿应做(尿感控制后2~3周)排泄性膀胱造影,以了解有无UVR或尿道瓣膜致尿流淤滞的尿感诱因。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "上尿路感染" }, { "start_idx": 9, "end_idx": 16, "type": "pro", "entity": "泌尿系统B超检查" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "尿感" }, { "start_idx": 36, "end_idx": 42, "type": "pro", "entity": "排泄性膀胱造影" }, { "start_idx": 49, "end_idx": 51, "type": "sym", "entity": "UVR" }, { "start_idx": 53, "end_idx": 54, "type": "bod", "entity": "尿道" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "尿道瓣膜" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "尿流淤滞" }, { "start_idx": 63, "end_idx": 64, "type": "dis", "entity": "尿感" } ] }, { "text": "婴幼儿急性肾盂肾炎常以急性感染中毒症状为主要表现,而缺乏泌尿系统的特殊症状,故在发热性疾病的诊断过程中应警惕尿感的可能,并注意与其他系统的急性感染作鉴别。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "婴幼儿急性肾盂肾炎" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "急性感染中毒" }, { "start_idx": 11, "end_idx": 18, "type": "sym", "entity": "急性感染中毒症状" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "尿感" }, { "start_idx": 69, "end_idx": 72, "type": "dis", "entity": "急性感染" } ] }, { "text": "除尿感外,急性肾小球肾炎病程中可有暂时性尿白细胞增多,但有血尿、水肿和高血压;急性间质性肾炎和狼疮性肾炎亦有白细胞尿,均应结合临床症状和相关检查作鉴别诊断。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "尿感" }, { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "急性肾小球肾炎" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "尿白细胞" }, { "start_idx": 17, "end_idx": 25, "type": "sym", "entity": "暂时性尿白细胞增多" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "血尿" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "水肿" }, { "start_idx": 35, "end_idx": 37, "type": "sym", "entity": "高血压" }, { "start_idx": 39, "end_idx": 45, "type": "dis", "entity": "急性间质性肾炎" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "狼疮性肾炎" }, { "start_idx": 54, "end_idx": 57, "type": "sym", "entity": "白细胞尿" } ] }, { "text": "蛲虫病和无良好卫生护理的儿童,包茎及会阴炎症亦可出现尿频及尿急症状,但尿白细胞正常或只略为增多,尿培养结果不符合尿感。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "蛲虫病" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "包茎" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "会阴炎症" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "尿频" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "尿急" }, { "start_idx": 35, "end_idx": 38, "type": "bod", "entity": "尿白细胞" }, { "start_idx": 35, "end_idx": 46, "type": "sym", "entity": "尿白细胞正常或只略为增多" }, { "start_idx": 48, "end_idx": 50, "type": "ite", "entity": "尿培养" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "尿感" } ] }, { "text": "本病属血小板聚集功能障碍性疾病,为常染色体隐性遗传。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "血小板" } ] }, { "text": "这种血小板能正常地黏附到受损的血管内皮下组织,但不能进一步地正常延伸和形成血小板聚集。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "血小板" }, { "start_idx": 15, "end_idx": 21, "type": "bod", "entity": "血管内皮下组织" } ] }, { "text": "临床上可分为三个亚型:Ⅰ型:GPⅡb/Ⅲa复合物的量少于正常的5%;Ⅱ型:相当于正常的5%~25%;Ⅲ型:相当于正常的40%~100%,量无明显减少而结构异常,属变异型。", "entities": [ { "start_idx": 14, "end_idx": 23, "type": "bod", "entity": "GPⅡb/Ⅲa复合物" } ] }, { "text": "杂合子出血症不明显,纯合子则可表现皮肤、黏膜的自发出血甚至内脏出血,如皮肤瘀点、瘀斑、鼻出血、牙龈出血、月经过多、血尿、胃肠道出血等,罕见颅内出血。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "出血症不明显" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "皮肤" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "黏膜" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "自发出血" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "内脏出血" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "皮肤瘀点" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "瘀斑" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "鼻" }, { "start_idx": 43, "end_idx": 45, "type": "sym", "entity": "鼻出血" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "牙龈" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "牙龈出血" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "月经过多" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "血尿" }, { "start_idx": 60, "end_idx": 62, "type": "bod", "entity": "胃肠道" }, { "start_idx": 60, "end_idx": 64, "type": "sym", "entity": "胃肠道出血" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "颅内出血" } ] }, { "text": "血小板对ADP、胶原、肾上腺素、凝血酶诱导的聚集反应缺如或降低,但对瑞士托霉素和vWF的诱导的聚集反应正常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "ADP" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "胶原" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "凝血酶" }, { "start_idx": 34, "end_idx": 38, "type": "bod", "entity": "瑞士托霉素" }, { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "vWF" } ] }, { "text": "2.自幼有出血症状,表现为中度或重度皮肤、黏膜出血,可有月经过多,外伤手术后出血不止。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "出血" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "皮肤" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "黏膜" }, { "start_idx": 13, "end_idx": 24, "type": "sym", "entity": "中度或重度皮肤、黏膜出血" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "月经过多" }, { "start_idx": 33, "end_idx": 36, "type": "pro", "entity": "外伤手术" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "外伤手术后出血不止" } ] }, { "text": "4.血小板聚集试验:加ADP、肾上腺素、胶原、凝血酶、花生四烯酸均不引起聚集;少数加胶原、花生四烯酸、凝血酶有聚集反应。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "血小板" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "ADP" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "肾上腺素" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "胶原" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "凝血酶" }, { "start_idx": 27, "end_idx": 31, "type": "bod", "entity": "花生四烯酸" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "胶原" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "花生四烯酸" }, { "start_idx": 51, "end_idx": 53, "type": "bod", "entity": "凝血酶" } ] }, { "text": "肿瘤多生长于皮下组织或横纹肌内,多见于下肢,尤其股部和腹股沟、头面部、纵隔、腹膜后和骨盆内也可发生。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "皮下组织" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "横纹肌" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "下肢" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "股部" }, { "start_idx": 27, "end_idx": 29, "type": "bod", "entity": "腹股沟" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "头面部" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "纵隔" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "腹膜" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "骨盆" } ] }, { "text": "肿瘤为缓慢生长的无痛性肿块,血管丰富,故局部温度可略有增高,有时有微弱搏动,但上述情况很少引起注意,肿瘤可伴发低血糖、男性化表现,原因不明。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "肿块" }, { "start_idx": 14, "end_idx": 15, "type": "bod", "entity": "血管" }, { "start_idx": 20, "end_idx": 28, "type": "sym", "entity": "局部温度可略有增高" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "有时有微弱搏动" }, { "start_idx": 50, "end_idx": 51, "type": "dis", "entity": "肿瘤" }, { "start_idx": 53, "end_idx": 63, "type": "sym", "entity": "伴发低血糖、男性化表现" } ] }, { "text": "对恶性的手术后要放、化疗,但其疗效无统一意见。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "手术" }, { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "放、化疗" } ] }, { "text": "临床特征为骨骼肌活动后容易疲劳,休息或使用胆碱酯酶抑制剂可以缓解。", "entities": [ { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "骨骼肌活动后容易疲劳" }, { "start_idx": 16, "end_idx": 31, "type": "sym", "entity": "休息或使用胆碱酯酶抑制剂可以缓解" } ] }, { "text": "本病可见于任何年龄,既往认为有两个高峰年龄,第一个高峰年龄为20~40岁,女性多见;第二个高峰年龄在40~60岁,以男性多见,多合并胸腺瘤。", "entities": [ { "start_idx": 66, "end_idx": 68, "type": "dis", "entity": "胸腺瘤" } ] }, { "text": "其临床特征为唾液肿大,尤以腮腺肿大最常见,可并发脑膜脑炎、睾丸炎、胰腺炎和其他腺体受累。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "唾液" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "唾液肿大" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "腮腺" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "腮腺肿大" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "脑膜脑炎" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "睾丸炎" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "腺体" } ] }, { "text": "腮腺导管口红肿有助诊断。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "腮腺导管口" }, { "start_idx": 0, "end_idx": 6, "type": "sym", "entity": "腮腺导管口红肿" } ] }, { "text": "发热多为中度,可为高热,热程一般3~7天,约20%体温正常。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "发热多为中度" }, { "start_idx": 9, "end_idx": 28, "type": "sym", "entity": "高热,热程一般3~7天,约20%体温正常" } ] }, { "text": "表现为发热、头痛、呕吐、颈项强直,很少出现惊厥。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "发热" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "头痛" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "呕吐" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "颈项" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "颈项强直" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "惊厥" } ] }, { "text": "患者突起发热、寒战、头痛、恶心、呕吐和下腹痛。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "发热" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "寒战" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "头痛" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "恶心" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "呕吐" }, { "start_idx": 19, "end_idx": 21, "type": "sym", "entity": "下腹痛" } ] }, { "text": "睾丸肿胀、疼痛和变硬,随着体温消退疼痛和肿胀消失,而坚硬可持续较久。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "睾丸" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "睾丸肿胀、疼痛和变硬" }, { "start_idx": 11, "end_idx": 32, "type": "sym", "entity": "随着体温消退疼痛和肿胀消失,而坚硬可持续较久" } ] }, { "text": "患者突起上腹疼痛和紧张感,伴发热、寒战、软弱、反复呕吐。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "上腹" }, { "start_idx": 2, "end_idx": 11, "type": "sym", "entity": "突起上腹疼痛和紧张感" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "发热" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "寒战" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "软弱" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "反复呕吐" } ] }, { "text": "还可见甲状腺炎、乳腺炎、泪腺炎、关节炎、肝炎、间质性肺炎、肾炎、心肌炎和神经炎等表现。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "甲状腺炎" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "乳腺炎" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "泪腺炎" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "关节炎" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肝炎" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "间质性肺炎" }, { "start_idx": 29, "end_idx": 30, "type": "dis", "entity": "肾炎" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "心肌炎" }, { "start_idx": 36, "end_idx": 38, "type": "dis", "entity": "神经炎" } ] }, { "text": "病菌从腮腺口侵入,患者有高热,腮腺部位红肿、压痛,挤压后可见脓液自腮腺口流出。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "腮腺口" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "高热" }, { "start_idx": 15, "end_idx": 18, "type": "bod", "entity": "腮腺部位" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "脓液" }, { "start_idx": 33, "end_idx": 35, "type": "bod", "entity": "腮腺口" }, { "start_idx": 15, "end_idx": 37, "type": "sym", "entity": "腮腺部位红肿、压痛,挤压后可见脓液自腮腺口流出" } ] }, { "text": "急性期注意休息,补充水分和营养,给予流质和软食,避免摄入酸性饮食;高热者给以退热剂或物理降温;腮腺肿痛时,给予镇痛剂,也可局部温敷(因人而异)。", "entities": [ { "start_idx": 16, "end_idx": 22, "type": "pro", "entity": "给予流质和软食" }, { "start_idx": 24, "end_idx": 31, "type": "pro", "entity": "避免摄入酸性饮食" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "高热" }, { "start_idx": 38, "end_idx": 40, "type": "dru", "entity": "退热剂" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "物理降温" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "腮腺" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "腮腺肿痛" }, { "start_idx": 55, "end_idx": 57, "type": "dru", "entity": "镇痛剂" }, { "start_idx": 61, "end_idx": 64, "type": "pro", "entity": "局部温敷" } ] }, { "text": "咯血是本病的重要症状,少数可咯出咖啡色颗粒状物,常为曲菌球脱落的碎片,此时镜检可找到菌丝。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "咯血" }, { "start_idx": 11, "end_idx": 22, "type": "sym", "entity": "少数可咯出咖啡色颗粒状物" }, { "start_idx": 26, "end_idx": 27, "type": "mic", "entity": "曲菌" }, { "start_idx": 37, "end_idx": 43, "type": "sym", "entity": "镜检可找到菌丝" } ] }, { "text": "特征性的临床过程是咳黏液栓性痰、咯血、间断性发热、胸痛、反复肺炎。", "entities": [ { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "痰" }, { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "咳黏液栓性痰" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "咯血" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "间断性发热" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "胸痛" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "反复肺炎" } ] }, { "text": "X线表现为肺上野有短暂的浸润影,常伴有典型的黏液栓形成的分支状的阴影(指套征)和中心性支气管扩张征象。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "X线" }, { "start_idx": 5, "end_idx": 14, "type": "sym", "entity": "肺上野有短暂的浸润影" }, { "start_idx": 19, "end_idx": 33, "type": "sym", "entity": "典型的黏液栓形成的分支状的阴影" }, { "start_idx": 40, "end_idx": 47, "type": "sym", "entity": "中心性支气管扩张" } ] }, { "text": "分为急性和慢性,急性和慢性肺部曲霉菌病的发生与机体免疫状态和基础疾病有关。", "entities": [ { "start_idx": 8, "end_idx": 18, "type": "dis", "entity": "急性和慢性肺部曲霉菌病" } ] }, { "text": "主要表现为发热、咳嗽,病变广泛或严重时可出现呼吸困难。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "发热" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "咳嗽" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "呼吸困难" } ] }, { "text": "慢性肺部曲霉菌病病程可长达数月甚至数年。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "慢性肺部曲霉菌病" } ] }, { "text": "两性霉素B脂质体毒性较小,每日剂量可增到4mg/(kg•d)。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "两性霉素B" } ] }, { "text": "如窦率显著缓慢又无结性逸搏出现,可出现眩晕或晕厥。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "ite", "entity": "窦率" }, { "start_idx": 11, "end_idx": 12, "type": "ite", "entity": "逸搏" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "眩晕" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "晕厥" } ] }, { "text": "有时窦缓可与室上速交替出现(快慢综合征),引起心悸、运动不耐受或眩晕。", "entities": [ { "start_idx": 0, "end_idx": 12, "type": "sym", "entity": "有时窦缓可与室上速交替出现" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "快慢综合征" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "心悸" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "运动不耐受" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "眩晕" } ] }, { "text": "如有些快慢综合征的患儿用药物(普萘洛尔、奎尼丁、普鲁卡因胺)控制心动过速时可出现症状性心动过缓。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "sym", "entity": "快慢综合征" }, { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "普萘洛尔" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "奎尼丁" }, { "start_idx": 24, "end_idx": 28, "type": "dru", "entity": "普鲁卡因胺" }, { "start_idx": 32, "end_idx": 33, "type": "ite", "entity": "心动" }, { "start_idx": 40, "end_idx": 46, "type": "sym", "entity": "症状性心动过缓" } ] }, { "text": "此时可能需药物治疗的同时安置起搏器。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "equ", "entity": "起搏器" } ] }, { "text": "在年长儿,房扑通常发生在有先天性心脏病的基础上。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "房扑" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "心脏病" } ] }, { "text": "房扑可发生在急性感染期间,但最常见于心房扩大患儿,如二尖瓣或三尖瓣的长期关闭不全,三尖瓣闭锁,Ebstein畸形或风湿性二尖瓣狭窄。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "房扑" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "感染" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "心房扩大" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "二尖瓣" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "三尖瓣" }, { "start_idx": 41, "end_idx": 45, "type": "sym", "entity": "三尖瓣闭锁" }, { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "Ebstein畸形" }, { "start_idx": 57, "end_idx": 64, "type": "sym", "entity": "风湿性二尖瓣狭窄" } ] }, { "text": "房扑也可发生在姑息性及纠治性房内手术后。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "房扑" } ] }, { "text": "提高迷走张力的方法(如颈动脉窦压迫、将脸浸于冰水)或给予腺苷通常可使心率暂时减慢。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "颈动脉窦压迫" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "将脸浸于冰水" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "给予腺苷" }, { "start_idx": 34, "end_idx": 35, "type": "ite", "entity": "心率" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "心率暂时减慢" } ] }, { "text": "Ⅲ类药物如胺碘酮、索他洛尔可用于对Ⅰ类无效的患儿。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "胺碘酮" }, { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "索他洛尔可" } ] }, { "text": "如药物治疗无效,可予以射频消融或外科消融治疗。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "射频消融" }, { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "外科消融" } ] }, { "text": "心脏正常的新生儿患儿,如对地高辛有效,应用药6~12个月,然后停药。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心脏" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "地高辛" } ] }, { "text": "本病是儿童时期常见的继发性肾小球疾病之一,以肾病综合征或蛋白尿、血尿为突出表现,病理上则以膜性肾病最为多见。", "entities": [ { "start_idx": 10, "end_idx": 17, "type": "dis", "entity": "继发性肾小球疾病" }, { "start_idx": 22, "end_idx": 26, "type": "sym", "entity": "肾病综合征" }, { "start_idx": 28, "end_idx": 30, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "血尿" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "膜性肾病" } ] }, { "text": "以往本病的命名不统一,如乙型肝炎相关性肾炎、乙肝免疫复合物肾炎以及乙肝病毒抗原相关性肾炎等,自1989年《中华内科杂志》召开乙肝肾炎座谈会后开始统一命名为“乙型肝炎病毒相关肾炎”。", "entities": [ { "start_idx": 12, "end_idx": 20, "type": "dis", "entity": "乙型肝炎相关性肾炎" }, { "start_idx": 22, "end_idx": 30, "type": "dis", "entity": "乙肝免疫复合物肾炎" }, { "start_idx": 33, "end_idx": 43, "type": "dis", "entity": "乙肝病毒抗原相关性肾炎" }, { "start_idx": 55, "end_idx": 56, "type": "dep", "entity": "内科" }, { "start_idx": 62, "end_idx": 65, "type": "dis", "entity": "乙肝肾炎" }, { "start_idx": 78, "end_idx": 87, "type": "dis", "entity": "乙型肝炎病毒相关肾炎" } ] }, { "text": "膜性肾病是儿童HBV-GN最常见的病理类型。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "膜性肾病" }, { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "HBV-GN" } ] }, { "text": "对表现为大量蛋白尿的HBV-GN,有人试用泼尼松短程疗法以减轻蛋白尿,但近年来研究认为HBV-GN对皮质激素多无任何效应,而且远期临床观察表明肾上腺皮质激素有弊无利,因此,应禁止单用肾上腺皮质激素治疗。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "HBV-GN" }, { "start_idx": 21, "end_idx": 27, "type": "pro", "entity": "泼尼松短程疗法" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "HBV-GN" }, { "start_idx": 50, "end_idx": 53, "type": "dru", "entity": "皮质激素" }, { "start_idx": 71, "end_idx": 77, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 91, "end_idx": 99, "type": "pro", "entity": "肾上腺皮质激素治疗" } ] }, { "text": "据统计,全世界每年约有100万~200万新生儿死于新生儿肺炎。", "entities": [ { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "新生儿肺炎" } ] }, { "text": "国内可能仍以大肠杆菌为主要致病菌。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "mic", "entity": "大肠杆菌" } ] }, { "text": "另外,克雷伯杆菌、李斯特菌也可引起新生儿宫内、出生时感染性肺炎。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "mic", "entity": "克雷伯杆菌" }, { "start_idx": 9, "end_idx": 12, "type": "mic", "entity": "李斯特菌" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "感染性肺炎" } ] }, { "text": "巨细胞病毒、单纯疱疹病毒、风疹病毒等,以及原虫(如弓形虫)、衣原体、支原体(如解脲支原体)等也可引起新生儿感染性肺炎。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "巨细胞病毒" }, { "start_idx": 6, "end_idx": 11, "type": "mic", "entity": "单纯疱疹病毒" }, { "start_idx": 13, "end_idx": 16, "type": "mic", "entity": "风疹病毒" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "原虫" }, { "start_idx": 25, "end_idx": 27, "type": "mic", "entity": "弓形虫" }, { "start_idx": 30, "end_idx": 32, "type": "mic", "entity": "衣原体" }, { "start_idx": 34, "end_idx": 36, "type": "mic", "entity": "支原体" }, { "start_idx": 39, "end_idx": 43, "type": "mic", "entity": "解脲支原体" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "感染性肺炎" } ] }, { "text": "生后立即进行胃液涂片查找白细胞与抗原;或取血样、气管分泌物等进行涂片、培养、对流免疫电泳等检测,有助于病原学诊断。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "胃液" }, { "start_idx": 12, "end_idx": 14, "type": "bod", "entity": "白细胞" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "血样" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "气管分泌物" } ] }, { "text": "新生儿出生后一旦发现呼吸增快即开始抗生素治疗:大肠埃希菌等肠道杆菌肺炎可用氨苄西林和阿米卡星,耐药者可选用第三代头孢菌素;GBS肺炎可选用青霉素和氨苄西林治疗3天,以后用大剂量青霉素,疗程10~14天;李斯特菌肺炎可选用氨苄西林;衣原体肺炎首选红霉素;单纯疱疹病毒性肺炎可用阿昔洛韦静脉滴注。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "抗生素" }, { "start_idx": 23, "end_idx": 27, "type": "dru", "entity": "大肠埃希菌" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "肠道杆菌肺炎" }, { "start_idx": 37, "end_idx": 40, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 42, "end_idx": 45, "type": "dru", "entity": "阿米卡星" }, { "start_idx": 56, "end_idx": 59, "type": "dru", "entity": "头孢菌素" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "GBS肺炎" }, { "start_idx": 69, "end_idx": 71, "type": "dru", "entity": "青霉素" }, { "start_idx": 73, "end_idx": 76, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 88, "end_idx": 90, "type": "dru", "entity": "青霉素" }, { "start_idx": 101, "end_idx": 106, "type": "dis", "entity": "李斯特菌肺炎" }, { "start_idx": 110, "end_idx": 113, "type": "dru", "entity": "氨苄西林" }, { "start_idx": 115, "end_idx": 119, "type": "dis", "entity": "衣原体肺炎" }, { "start_idx": 122, "end_idx": 124, "type": "dru", "entity": "红霉素" }, { "start_idx": 126, "end_idx": 134, "type": "dis", "entity": "单纯疱疹病毒性肺炎" }, { "start_idx": 137, "end_idx": 140, "type": "dru", "entity": "阿昔洛韦" }, { "start_idx": 141, "end_idx": 144, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "导致肾盂输尿管连接部内源性梗阻的其他原因尚包括:①瓣膜样的黏膜皱襞;②胎儿期输尿管扭曲折叠的持续存在;③输尿管起始段息肉。", "entities": [ { "start_idx": 2, "end_idx": 14, "type": "dis", "entity": "肾盂输尿管连接部内源性梗阻" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "黏膜皱襞" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "输尿管" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "输尿管" } ] }, { "text": "患儿可出现血尿,或阵发性腹痛,在UPJO中发现的比率有升高之势,从随访来看,术后少有复发。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "血尿" }, { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "阵发性腹痛" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "UPJO" } ] }, { "text": "由迷走血管导致的PUJO的发生率在15%~52%之间,在成人比较多见,小儿很少。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "迷走血管" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "PUJO" } ] }, { "text": "有报道在手术中采用高选择性动脉造影,将其供应动脉栓塞,使病变逐渐萎缩。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "手术" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "动脉" } ] }, { "text": "断续进展型其次,症状在数小时至数天内断续进展,神志异常或呆滞或激惹,肌张力低下,动作减少,呼吸不规则。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "断续进展型" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "神志异常" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "呆滞" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "激惹" }, { "start_idx": 34, "end_idx": 38, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "动作减少" }, { "start_idx": 45, "end_idx": 49, "type": "sym", "entity": "呼吸不规则" } ] }, { "text": "急剧恶化型最为少见,但临床症状最严重,患儿可在数分钟至数小时内迅速恶化,出现意识障碍、呼吸困难或暂停、抽搐、瞳孔光反射消失、四肢肌张力低下、前囟紧张,伴失血性贫血、血压下降、心动过缓。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "急剧恶化型" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "意识障碍" }, { "start_idx": 43, "end_idx": 49, "type": "sym", "entity": "呼吸困难或暂停" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "抽搐" }, { "start_idx": 54, "end_idx": 55, "type": "bod", "entity": "瞳孔" }, { "start_idx": 54, "end_idx": 60, "type": "sym", "entity": "瞳孔光反射消失" }, { "start_idx": 62, "end_idx": 63, "type": "bod", "entity": "四肢" }, { "start_idx": 62, "end_idx": 68, "type": "sym", "entity": "四肢肌张力低下" }, { "start_idx": 70, "end_idx": 71, "type": "bod", "entity": "前囟" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "前囟紧张" }, { "start_idx": 76, "end_idx": 80, "type": "dis", "entity": "失血性贫血" }, { "start_idx": 82, "end_idx": 83, "type": "ite", "entity": "血压" }, { "start_idx": 87, "end_idx": 88, "type": "ite", "entity": "心动" }, { "start_idx": 82, "end_idx": 90, "type": "sym", "entity": "血压下降、心动过缓" } ] }, { "text": "可连续腰椎穿刺以防止脑室的迅速扩大,但往往效果不理想。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "腰椎穿刺" } ] }, { "text": "如腰椎穿刺治疗无效,可考虑暂时直接脑室外部引流,少数患儿由于脑脊液吸收旁路重建而得以恢复。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "腰椎穿刺" }, { "start_idx": 17, "end_idx": 22, "type": "pro", "entity": "脑室外部引流" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "脑脊液" } ] }, { "text": "1986年在美国,化脑的平均发病年龄为15个月。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "化脑" } ] }, { "text": "有密切接触史、居住拥挤、贫穷、小婴儿缺乏母乳喂养都是诱发因素。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "母乳" } ] }, { "text": "传播方式是经接触呼吸道分泌物和飞沫传播,脾功能不全如镰状细胞贫血、无脾的患者易患肺炎链球菌脑膜炎,有时也易患Hib脑膜炎。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "呼吸道分泌物" }, { "start_idx": 20, "end_idx": 20, "type": "bod", "entity": "脾" }, { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "脾功能不全" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "镰状细胞" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "镰状细胞贫血" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "脾" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "无脾" }, { "start_idx": 40, "end_idx": 47, "type": "dis", "entity": "肺炎链球菌脑膜炎" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "Hib脑膜炎" } ] }, { "text": "化脑一般发病急,有高热、头痛、呕吐、食欲缺乏及精神委靡等症状。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "化脑" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "高热" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "头痛" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "呕吐" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "食欲缺乏" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "精神委靡" } ] }, { "text": "起病时神志一般清醒,病情进展可发生嗜睡、谵妄、惊厥和昏迷。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "sym", "entity": "神志一般清醒" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "嗜睡" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "谵妄" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "惊厥" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "昏迷" } ] }, { "text": "体检可见意识障碍、昏迷、颈强直、克氏征与布氏征阳性。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "意识障碍" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "昏迷" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "颈" }, { "start_idx": 12, "end_idx": 14, "type": "sym", "entity": "颈强直" }, { "start_idx": 16, "end_idx": 24, "type": "sym", "entity": "克氏征与布氏征阳性" } ] }, { "text": "如未及时治疗,颈强直加重、头后仰、背肌僵硬甚至角弓反张。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "颈" }, { "start_idx": 7, "end_idx": 9, "type": "sym", "entity": "颈强直" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "头" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "头后仰" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "背肌" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "背肌僵硬" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "角弓反张" } ] }, { "text": "婴幼儿期化脑起病急缓不一。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "婴幼儿期化脑" } ] }, { "text": "常先以易激惹、烦躁不安、面色苍白、食欲减低开始,然后出现发热及呼吸系统或消化系统症状,如呕吐、腹泻、轻微咳嗽,继之嗜睡、头向后仰、感觉过敏、哭声尖锐、眼神发呆、双目凝视,有时用手打头、摇头。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "sym", "entity": "易激惹" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "面色苍白" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "食欲减低" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "发热" }, { "start_idx": 31, "end_idx": 34, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "消化系统" }, { "start_idx": 31, "end_idx": 41, "type": "sym", "entity": "呼吸系统或消化系统症状" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "呕吐" }, { "start_idx": 47, "end_idx": 48, "type": "sym", "entity": "腹泻" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "轻微咳嗽" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "嗜睡" }, { "start_idx": 60, "end_idx": 60, "type": "bod", "entity": "头" }, { "start_idx": 60, "end_idx": 63, "type": "sym", "entity": "头向后仰" }, { "start_idx": 65, "end_idx": 68, "type": "sym", "entity": "感觉过敏" }, { "start_idx": 70, "end_idx": 73, "type": "sym", "entity": "哭声尖锐" }, { "start_idx": 75, "end_idx": 78, "type": "sym", "entity": "眼神发呆" }, { "start_idx": 80, "end_idx": 83, "type": "sym", "entity": "双目凝视" }, { "start_idx": 92, "end_idx": 93, "type": "sym", "entity": "摇头" } ] }, { "text": "往往在发生惊厥后才引起家长注意和就诊。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "惊厥" } ] }, { "text": "较常见的情况是出生时婴儿正常,数日后出现肌张力低下、少动、哭声微弱、吸吮力差、拒食、呕吐、黄疸、发绀、呼吸不规则等非特异性症状。", "entities": [ { "start_idx": 20, "end_idx": 24, "type": "sym", "entity": "肌张力低下" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "少动" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "哭声微弱" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "吸吮力差" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "拒食" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "呕吐" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "黄疸" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "发绀" }, { "start_idx": 51, "end_idx": 55, "type": "sym", "entity": "呼吸不规则" } ] }, { "text": "有些病儿直到尸检时才发现其为化脑。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "化脑" } ] }, { "text": "此时须争取及早作脑脊液检查,以免延误诊断。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "脑脊液检查" } ] }, { "text": "糖、氯化物均明显减低。", "entities": [ { "start_idx": 0, "end_idx": 0, "type": "bod", "entity": "糖" }, { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "氯化物" }, { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "糖、氯化物均明显减低" } ] }, { "text": "脑脊液留膜涂片可找到结核杆菌。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "脑脊液留膜涂片" }, { "start_idx": 10, "end_idx": 13, "type": "mic", "entity": "结核杆菌" } ] }, { "text": "化脑的疗程根据病原菌而异。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "化脑" } ] }, { "text": "肺炎链球菌脑膜炎和流感嗜血杆菌脑膜炎,疗程2~3周,或热退后继续用药10~14天。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "肺炎链球菌脑膜炎" }, { "start_idx": 9, "end_idx": 17, "type": "dis", "entity": "流感嗜血杆菌脑膜炎" } ] }, { "text": "体格检查见前囟膨隆、颅骨缝裂开、头围增大、叩诊有破壶音。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "前囟膨隆" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "颅骨缝裂开" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "头围增大" } ] }, { "text": "主要临床表现为呼吸不规则、瞳孔不等大或扩大、视神经乳头水肿、前囟隆起或紧张、高血压、昏迷、惊厥和四肢张力明显增高、呕吐、头痛等。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "呼吸不规则" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "瞳孔" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "瞳孔不等大或扩大" }, { "start_idx": 22, "end_idx": 26, "type": "bod", "entity": "视神经乳头" }, { "start_idx": 22, "end_idx": 28, "type": "sym", "entity": "视神经乳头水肿" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "前囟隆起" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "紧张" }, { "start_idx": 38, "end_idx": 40, "type": "sym", "entity": "高血压" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "昏迷" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "惊厥" }, { "start_idx": 48, "end_idx": 49, "type": "bod", "entity": "四肢" }, { "start_idx": 48, "end_idx": 55, "type": "sym", "entity": "四肢张力明显增高" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "呕吐" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "头痛" } ] }, { "text": "当化脑患儿有以下情况出现应考虑有脑室膜炎存在的可能:病情危重、频繁惊厥、呼吸衰竭、按正规治疗疗效不佳;脑超声波或CT检查有明显脑室扩大等。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "化脑" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "脑室膜炎" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "惊厥" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "呼吸衰竭" }, { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "脑超声波" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "CT检查" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "脑室扩大" } ] }, { "text": "对暂时性脑积水可进行内科治疗,包括限制饮水或给予利尿剂等。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "暂时性脑积水" }, { "start_idx": 10, "end_idx": 11, "type": "dep", "entity": "内科" } ] }, { "text": "暴发型流脑可伴发DIC、休克。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "暴发型流脑" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "DIC" }, { "start_idx": 12, "end_idx": 13, "type": "dis", "entity": "休克" } ] }, { "text": "肺炎链球菌所致化脑病死率可达15%~25%,且易于复发、再发,甚至出现智力低下、耳聋及肢体瘫痪等后遗症。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "化脑" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "智力低下" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "耳聋" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "肢体瘫痪" } ] }, { "text": "化脓性脑膜炎可发生神经系统后遗症,年幼者更常见,多因延误诊断和治疗不当所致。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "化脓性脑膜炎" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "神经系统" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "神经系统后遗症" } ] }, { "text": "化脑急性期如有严重抽搐;长时间神志不清和其他明显脑损伤表现,均有可能发生后遗症。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "化脑" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "严重抽搐" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "长时间神志不清" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "脑" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "脑损伤" } ] }, { "text": "根据其发生原因分为先天性(婴儿多为支气管软骨发育缺陷,年长儿多为支气管肌肉及弹力纤维发育缺陷)和后天性两类,后者多由慢性肺部感染引起,如麻疹、百日咳和重症肺炎等。", "entities": [ { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "支气管软骨发育缺陷" }, { "start_idx": 32, "end_idx": 45, "type": "dis", "entity": "支气管肌肉及弹力纤维发育缺陷" }, { "start_idx": 58, "end_idx": 63, "type": "dis", "entity": "慢性肺部感染" }, { "start_idx": 68, "end_idx": 69, "type": "dis", "entity": "麻疹" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "百日咳" }, { "start_idx": 75, "end_idx": 78, "type": "dis", "entity": "重症肺炎" } ] }, { "text": "免疫缺陷的患儿,尤其是低免疫球蛋白血症者反复细菌性肺炎和支气管炎后可发生支气管扩张。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "免疫缺陷" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "低免疫球蛋白血症" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "细菌性肺炎" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "支气管炎" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "支气管扩张" } ] }, { "text": "其确切机制尚不明确,一般认为感染和支气管阻塞是支气管扩张的两个根本致病因素,感染后剧烈咳嗽、管腔内分泌物的淤滞等可促使损伤软化的支气管壁扩张。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "感染" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "支气管阻塞" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "支气管扩张" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "感染" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "剧烈咳嗽" }, { "start_idx": 46, "end_idx": 54, "type": "sym", "entity": "管腔内分泌物的淤滞" }, { "start_idx": 64, "end_idx": 69, "type": "dis", "entity": "支气管壁扩张" } ] }, { "text": "根据支气管扩张的形态分为4型:①圆柱状:较局限,常见于轻症;②囊状:分布范围较广,多为重症;③核状:介于两者之间;④混合型:兼有以上两种形态,较常见。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "支气管扩张" } ] }, { "text": "主要症状为咳嗽,多由变换体位时引起,急性感染时伴大量黏液脓痰。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "咳嗽" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "急性感染" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "大量黏液脓痰" } ] }, { "text": "发热少见。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "发热少见" } ] }, { "text": "杵状指(趾)的出现提示病程常在1年以上。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "杵状指(趾)" } ] }, { "text": "轻症患儿胸片仅见肺纹理增粗,病变明显时可见中下肺大小不等的环状透光阴影,呈卷发状或蜂窝状,常伴肺段、肺叶不张影及周围炎性浸润影。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "胸片" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "肺纹理增粗" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "中下肺" }, { "start_idx": 21, "end_idx": 43, "type": "sym", "entity": "中下肺大小不等的环状透光阴影,呈卷发状或蜂窝状" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "肺段" }, { "start_idx": 50, "end_idx": 51, "type": "bod", "entity": "肺叶" }, { "start_idx": 47, "end_idx": 62, "type": "sym", "entity": "肺段、肺叶不张影及周围炎性浸润影" } ] }, { "text": "根据上述典型症状和X线表现,结合支气管造影不难诊断。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "X线" }, { "start_idx": 16, "end_idx": 20, "type": "pro", "entity": "支气管造影" } ] }, { "text": "近年来支气管造影在很大程度上已被CT、尤其是高分辨CT替代。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "pro", "entity": "支气管造影" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "CT" }, { "start_idx": 22, "end_idx": 26, "type": "pro", "entity": "高分辨CT" } ] }, { "text": "柱状支气管扩张症,CT表现为“轨道征”或“印戒征”。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "柱状支气管扩张症" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "CT" }, { "start_idx": 15, "end_idx": 17, "type": "sym", "entity": "轨道征" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "印戒征" } ] }, { "text": "囊状支气管扩张症,呈簇集蜂窝状分布,诊断准确率较高。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "囊状支气管扩张症" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "簇集蜂窝状分布" } ] }, { "text": "支气管扩张患儿的治疗应重视体位引流,以排除气道分泌物;雾化吸入疗法对稀化黏痰有良好效果。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "支气管扩张" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "体位引流" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "气道分泌物" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "雾化吸入" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "黏痰" } ] }, { "text": "急性恶化期全身应用抗生素2~3周,囊性纤维化患儿适当延长疗程,但盲目延长可能会增加耐药性和耐药菌株的产生。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "抗生素" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "囊性纤维化" } ] }, { "text": "最好根据痰培养和药物敏感试验结果选择相应抗生素。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "ite", "entity": "痰培养" }, { "start_idx": 8, "end_idx": 13, "type": "ite", "entity": "药物敏感试验" }, { "start_idx": 20, "end_idx": 22, "type": "dru", "entity": "抗生素" } ] }, { "text": "若一侧或一叶肺组织大部分或全部被多发的囊肿占据,称为多囊肺。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "肺组织" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "多囊肺" } ] }, { "text": "囊肿大者可压迫气管或主支气管引起阵发性咳嗽、气促、喘息甚至发绀等症状,伴反复肺部感染。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "气管" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "主支气管" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "阵发性咳嗽" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "气促" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "喘息" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "发绀" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "反复肺部感染" } ] }, { "text": "肺部叩诊鼓音或实音,听诊呼吸音减低或消失。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "鼓音或实音" }, { "start_idx": 12, "end_idx": 19, "type": "sym", "entity": "呼吸音减低或消失" } ] }, { "text": "胸部X线检查是诊断本病的主要依据。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "pro", "entity": "胸部X线检查" } ] }, { "text": "本病治疗以手术切除为主。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "手术切除" } ] }, { "text": "较大囊肿应尽早切除,早期手术可预防气胸、肺动脉高压、呼吸道感染等并发症。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "较大囊肿" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "尽早切除" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "气胸" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "呼吸道感染" } ] }, { "text": "如有胎儿肺囊肿伴纵隔移位,有必要进行胎儿胸腔穿刺,防止心血管功能不全。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "胎儿肺囊肿" }, { "start_idx": 18, "end_idx": 23, "type": "pro", "entity": "胎儿胸腔穿刺" } ] }, { "text": "因此,如轻度溶血时,外周血中的红细胞数能被骨髓造血功能的增加所完全代偿,此时,临床上虽有溶血存在但并不发生贫血;如果红细胞的破坏超过了骨髓造血的代偿能力,则发生溶血性贫血。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "轻度溶血" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "外周血" }, { "start_idx": 15, "end_idx": 18, "type": "ite", "entity": "红细胞数" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "骨髓" }, { "start_idx": 44, "end_idx": 45, "type": "dis", "entity": "溶血" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "贫血" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "红细胞" }, { "start_idx": 67, "end_idx": 68, "type": "bod", "entity": "骨髓" }, { "start_idx": 80, "end_idx": 84, "type": "dis", "entity": "溶血性贫血" } ] }, { "text": "(3)遗传性口形细胞增多症。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "遗传性口形细胞增多症" } ] }, { "text": "(4)遗传性畸形细胞增多症。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "遗传性畸形细胞增多症" } ] }, { "text": "2)磷酸葡萄糖异构酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "磷酸葡萄糖异构酶缺乏" } ] }, { "text": "3)腺苷三磷酸酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dis", "entity": "腺苷三磷酸酶缺乏" } ] }, { "text": "4)谷胱甘肽还原酶缺乏。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "谷胱甘肽还原酶缺乏" } ] }, { "text": "(2)镰状细胞贫血。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "镰状细胞贫血" } ] }, { "text": "(3)血红蛋白H病。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "血红蛋白H病" } ] }, { "text": "(2)新生儿溶血症(Rh、ABO不相合)。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "新生儿溶血症" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "Rh" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "ABO不相合" } ] }, { "text": "3)与免疫现象有关的贫血(移植物排斥,免疫复合物等)。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "贫血" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "移植物排斥" } ] }, { "text": "(3)热损伤(如烧伤、烫伤等)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "sym", "entity": "热损伤" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "烧伤" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "烫伤" } ] }, { "text": "(4)弥漫性血管内凝血(DIC)。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血管" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "凝血" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "DIC" } ] }, { "text": "(2)无β脂蛋白血症。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "无β脂蛋白血症" } ] }, { "text": "可有黄疸、气喘、心率加快、食欲缺乏、头昏、怕冷等症。", "entities": [ { "start_idx": 2, "end_idx": 22, "type": "sym", "entity": "黄疸、气喘、心率加快、食欲缺乏、头昏、怕冷" } ] }, { "text": "急性溶血时,患儿可突然发病,感胸闷、腰酸、发热、背痛、头痛,甚至发生周围循环衰竭,出现少尿或无尿,乃至急性肾衰竭。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "急性溶血" }, { "start_idx": 14, "end_idx": 55, "type": "sym", "entity": "感胸闷、腰酸、发热、背痛、头痛,甚至发生周围循环衰竭,出现少尿或无尿,乃至急性肾衰竭" } ] }, { "text": "慢性溶血时,患儿可出现黄疸及肝、脾大。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "慢性溶血" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "黄疸" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "肝、脾大" } ] }, { "text": "3.血清间接胆红素增高。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血清" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "胆红素" } ] }, { "text": "4.粪胆原定量增加。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "粪胆原" } ] }, { "text": "6.含铁血黄素尿试验阳性。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "pro", "entity": "尿试验" } ] }, { "text": "2.血清结合珠蛋白减少。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "bod", "entity": "血清" }, { "start_idx": 4, "end_idx": 8, "type": "bod", "entity": "结合珠蛋白" } ] }, { "text": "有溶血诱因者应尽早尽快去除诱因,如冷抗体型自身免疫性溶血性贫血,应立即保暖,特别是使四肢温暖;由蚕豆病引起的溶血性贫血应避免食用蚕豆和氧化性药物;某药引起的溶血性贫血应立即停止用该药,输血引起溶血者应立即停止输血;感染引起的溶血性贫血应积极控制感染等。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "溶血" }, { "start_idx": 17, "end_idx": 30, "type": "dis", "entity": "冷抗体型自身免疫性溶血性贫血" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "四肢" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "蚕豆病" }, { "start_idx": 54, "end_idx": 58, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 67, "end_idx": 71, "type": "dru", "entity": "氧化性药物" }, { "start_idx": 78, "end_idx": 82, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 92, "end_idx": 93, "type": "pro", "entity": "输血" }, { "start_idx": 104, "end_idx": 105, "type": "pro", "entity": "输血" }, { "start_idx": 107, "end_idx": 108, "type": "sym", "entity": "感染" }, { "start_idx": 112, "end_idx": 116, "type": "dis", "entity": "溶血性贫血" }, { "start_idx": 122, "end_idx": 123, "type": "sym", "entity": "感染" } ] }, { "text": "肾上腺皮质激素能抑制免疫反应,对免疫性溶血性贫血有效。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "bod", "entity": "肾上腺皮质激素" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "免疫性溶血性贫血" } ] }, { "text": "雄激素或蛋白合成激素能刺激造血,增加代偿功能。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "激素" } ] }, { "text": "免疫抑制剂对部分自身免疫性溶血性贫血有效,但应在肾上腺皮质激素使用无效时试用。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 8, "end_idx": 17, "type": "dis", "entity": "自身免疫性溶血性贫血" }, { "start_idx": 24, "end_idx": 30, "type": "bod", "entity": "肾上腺皮质激素" } ] }, { "text": "疟疾的主要病理变化是贫血,大量的红细胞被破坏。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "疟疾" }, { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "贫血" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "红细胞" } ] }, { "text": "间日疟和卵形疟隔日发作一次;三日疟每隔二日发作一次;如属重复感染,可每天发作一次;恶性疟呈稽留热,弛张热,或不规则热型,无明显的寒战和出汗期。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "间日疟" }, { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "卵形疟" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "三日疟" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "恶性疟" }, { "start_idx": 49, "end_idx": 51, "type": "sym", "entity": "弛张热" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "不规则热型" }, { "start_idx": 64, "end_idx": 65, "type": "sym", "entity": "寒战" } ] }, { "text": "目前已经研发的复方青蒿素有:复方蒿甲醚、双氢青蒿素哌喹、复方萘酚喹和复方青蒿素片。", "entities": [ { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "复方青蒿素" }, { "start_idx": 14, "end_idx": 18, "type": "dru", "entity": "复方蒿甲醚" }, { "start_idx": 20, "end_idx": 26, "type": "dru", "entity": "双氢青蒿素哌喹" }, { "start_idx": 28, "end_idx": 32, "type": "dru", "entity": "复方萘酚喹" }, { "start_idx": 34, "end_idx": 39, "type": "dru", "entity": "复方青蒿素片" } ] }, { "text": "B超检查的阳性率超过90%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "B超检查" } ] }, { "text": "手术采用幽门环肌切开术。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "pro", "entity": "幽门环肌切开术" } ] }, { "text": "幽门环肌切开术后多见偶发性呕吐,大多无需处理,按计划喂养。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "幽门环肌切开术" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "偶发性呕吐" } ] }, { "text": "异位起搏点可位于心房、房室交界或心室的任何部位。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "异位起搏点" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "心房" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "房室" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "心室" } ] }, { "text": "房性期前收缩在儿童常见,甚至可出现在无心脏病变的患儿。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "房性期前收缩" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "心脏" } ] }, { "text": "房性期前收缩的QRS波群可正常,或延长(差异传导),或缺乏,这主要取决于房性期前收缩提前的程度(联律间期)。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "房性期前收缩" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "房性期前收缩" } ] }, { "text": "室性期前收缩可起源于心室的任何部位,特征为提前出现的、增宽的、畸变的QRS波群,其前无P波。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "室性期前收缩" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "心室" } ] }, { "text": "如室性期前收缩的形态一致,则称之为单源性室性期前收缩。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "室性期前收缩" }, { "start_idx": 17, "end_idx": 25, "type": "dis", "entity": "单源性室性期前收缩" } ] }, { "text": "如期前收缩频发,有时可表现为固定的节律,如期前收缩与正常搏动交替(二联律),或两个正常搏动后一个期前收缩(三联律)。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "期前收缩" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "期前收缩" } ] }, { "text": "单个期前收缩发生时,大多数患儿感觉不到,但有些患儿可感到心前区跳动感。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "期前收缩" }, { "start_idx": 28, "end_idx": 33, "type": "sym", "entity": "心前区跳动感" } ] }, { "text": "焦虑、热性疾病、某些药物或刺激性物质的摄入可引起室性期前收缩。", "entities": [ { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "室性期前收缩" } ] }, { "text": "正常原始生殖腺的移行常沿着躯体中轴进行,因此除原发于卵巢和睾丸外,生殖细胞瘤多数发生于中线部位。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "bod", "entity": "原始生殖腺" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "躯体" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "卵巢" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "睾丸" }, { "start_idx": 33, "end_idx": 37, "type": "dis", "entity": "生殖细胞瘤" } ] }, { "text": "(2)非生殖细胞性:上皮性肿瘤;性索-基质性肿瘤。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "上皮性肿瘤" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "性索-基质性肿瘤" } ] }, { "text": "本病的发病部位较为多处,多见于骶尾部、睾丸、卵巢、盆腔、后腹膜、纵隔和颅内。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "骶尾部" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "睾丸" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "卵巢" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "盆腔" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "后腹膜" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "纵隔" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "颅内" } ] }, { "text": "本瘤起源于成纤维细胞,广泛浸润肌肉,沿筋膜表面实质,边缘不清楚,切除后有强烈的复发倾向。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "成纤维细胞" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肌肉" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "筋膜" } ] }, { "text": "瘤体坚韧,呈灰白色,大小不一,多见于腹壁、胸壁、肩胛等处。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "腹壁" }, { "start_idx": 21, "end_idx": 22, "type": "bod", "entity": "胸壁" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "肩胛" } ] }, { "text": "如腹壁部的肿瘤可发生在前腹壁除白线以外的任何部位,但多位于脐下。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "腹壁" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肿瘤" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "前腹壁" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "脐下" } ] }, { "text": "这些并发症可导致晕厥或猝死。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "晕厥" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "猝死" } ] }, { "text": "当患儿患缓慢性心律失常时,可予以植入起搏器。", "entities": [ { "start_idx": 5, "end_idx": 10, "type": "dis", "entity": "慢性心律失常" }, { "start_idx": 18, "end_idx": 20, "type": "equ", "entity": "起搏器" } ] }, { "text": "对于位于脑重要结构区而未能全切除的婴幼儿肿瘤,主张化疗,以避免放疗或利于延期放疗。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "脑" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "肿瘤" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "化疗" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "放疗" } ] }, { "text": "低度肿瘤预后较好,肿瘤的性质、级别、部位及病人的年龄、切除程度不同,预后各不相同。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "低度肿瘤" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "肿瘤" } ] }, { "text": "本瘤为逐渐增大的体表肿块,伴不同程度的疼痛。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "sym", "entity": "逐渐增大的体表肿块" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "伴不同程度的疼痛" } ] }, { "text": "恶性神经鞘瘤是一种侵袭性很强的肿瘤,切除后易局部复发,远处转移主要到肺,其次到肝和骨。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "恶性神经鞘瘤" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "肿瘤" }, { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "切除" }, { "start_idx": 34, "end_idx": 34, "type": "bod", "entity": "肺" }, { "start_idx": 39, "end_idx": 39, "type": "bod", "entity": "肝" }, { "start_idx": 41, "end_idx": 41, "type": "bod", "entity": "骨" } ] }, { "text": "流出部室间隔是表面光滑的圆锥隔,当其与肌小梁部的交界缘口偏歪不对线(malalignment)会引起主动脉骑跨;若这种不对线发生在左心室流出道室间隔,会引起主动脉弓梗阻;若发生在右心室会导致肺动脉下梗阻,如法洛四联症。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "bod", "entity": "流出部室间隔" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "肌小梁部" }, { "start_idx": 50, "end_idx": 54, "type": "dis", "entity": "主动脉骑跨" }, { "start_idx": 65, "end_idx": 73, "type": "bod", "entity": "左心室流出道室间隔" }, { "start_idx": 78, "end_idx": 83, "type": "dis", "entity": "主动脉弓梗阻" }, { "start_idx": 89, "end_idx": 91, "type": "bod", "entity": "右心室" }, { "start_idx": 95, "end_idx": 100, "type": "dis", "entity": "肺动脉下梗阻" }, { "start_idx": 103, "end_idx": 107, "type": "dis", "entity": "法洛四联症" } ] }, { "text": "大型室间隔缺损可引起肺动脉高压;当缺损很大,缺口不能限制左心室的分流来血,使左、右室压力几乎接近,此时分流量决定于体、肺两个循环的阻力。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "大型室间隔缺损" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 17, "end_idx": 18, "type": "dis", "entity": "缺损" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "左心室" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "左、右室" }, { "start_idx": 59, "end_idx": 59, "type": "bod", "entity": "肺" } ] }, { "text": "因左心室超容,心前区搏动明显,年长儿可看到明显心前区隆起和哈里森(Harrison)沟。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "左心室" }, { "start_idx": 23, "end_idx": 27, "type": "sym", "entity": "心前区隆起" }, { "start_idx": 29, "end_idx": 42, "type": "sym", "entity": "哈里森(Harrison)沟" } ] }, { "text": "有中至大型左向右分流,产生心力衰竭的婴儿,当可能出现缺损部分或完全自然关闭时,也可最初以药物治疗:①利尿剂降低心脏负荷和体循环静脉的充血状况。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "缺损" }, { "start_idx": 44, "end_idx": 47, "type": "pro", "entity": "药物治疗" }, { "start_idx": 50, "end_idx": 52, "type": "dru", "entity": "利尿剂" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "心脏" }, { "start_idx": 60, "end_idx": 64, "type": "bod", "entity": "体循环静脉" } ] }, { "text": "螺内酯有保钾作用,同时使用呋塞米和螺内酯,无须额外补钾。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "螺内酯" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "钾" }, { "start_idx": 13, "end_idx": 15, "type": "dru", "entity": "呋塞米" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "螺内酯" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "钾" } ] }, { "text": "②可以用地高辛,但在小婴儿最初出现负荷加重时一般不用。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dru", "entity": "地高辛" } ] }, { "text": "出现艾森门格综合征者,只能行心脏移植术。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "艾森门格综合征" }, { "start_idx": 14, "end_idx": 18, "type": "pro", "entity": "心脏移植术" } ] }, { "text": "一般将其分为三类:①腹膜后和腹腔内(大网膜、肠系膜、胃肠道和其他脏器);②皮肤和皮下组织;③脉管源性平滑肌肉瘤。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "腹膜后" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "腹腔内" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "大网膜" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肠系膜" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "胃肠道" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "脏器" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "皮肤" }, { "start_idx": 40, "end_idx": 43, "type": "bod", "entity": "皮下组织" }, { "start_idx": 46, "end_idx": 54, "type": "dis", "entity": "脉管源性平滑肌肉瘤" } ] }, { "text": "胃肠道平滑肌肉瘤可分布于从胃至直肠的各个部位,可有多发现象。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "胃肠道平滑肌肉瘤" }, { "start_idx": 13, "end_idx": 13, "type": "bod", "entity": "胃" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "直肠" } ] }, { "text": "肿瘤可导致肠梗阻、肠套叠。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "肠套叠" } ] }, { "text": "治疗为完全切除肿瘤,但范围要广,如胃肠道原发需切除其近端和远端至少5cm。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "完全切除" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "肿瘤" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "胃肠道" } ] }, { "text": "发作时心率大多超过180次/分,偶尔可达300次/分。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "心率" } ] }, { "text": "婴幼儿室上速的诊断比较困难。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "室上速" } ] }, { "text": "婴幼儿室上速就诊时常伴心力衰竭,因为室上速可发作数小时而未被发现。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "室上速" }, { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "室上速" } ] }, { "text": "发作时心率多在200~300次/分之间。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "ite", "entity": "心率" } ] }, { "text": "如发作持续6~24小时或更长,且心率极快,患儿可显得极度病态,脸色灰,烦躁不安,呼吸急促、肝大,可以有发热及白细胞增多。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "心率" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "显得极度病态" }, { "start_idx": 31, "end_idx": 33, "type": "sym", "entity": "脸色灰" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 40, "end_idx": 41, "type": "ite", "entity": "呼吸" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "肝" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "肝大" }, { "start_idx": 51, "end_idx": 52, "type": "sym", "entity": "发热" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "白细胞" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "白细胞增多" } ] }, { "text": "如室上速在胎儿发生,可引起严重心力衰竭及胎儿水肿。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "室上速" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "胎儿水肿" } ] }, { "text": "室上速与室性心动过速(以下简称室速)的鉴别十分重要,因为地高辛可促使室速的患儿发生室性颤动。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "室上速" }, { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "室性心动过速" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "室速" }, { "start_idx": 28, "end_idx": 30, "type": "dru", "entity": "地高辛" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "室速" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "室性颤动" } ] }, { "text": "预激综合征的患儿60%~70%可发生室上速,其典型心电图表现在未发作时通常可见,主要表现为PR间期缩短、δ波及宽QRS波群。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "预激综合征" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "室上速" }, { "start_idx": 25, "end_idx": 27, "type": "ite", "entity": "心电图" } ] }, { "text": "床边食管调搏可用于室上速的诊断、鉴别诊断及药物治疗疗效的评价。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "equ", "entity": "食管" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "室上速" } ] }, { "text": "在难治性病例,可在心导管实验室里进行更详尽的电生理检查。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "equ", "entity": "心导管" }, { "start_idx": 22, "end_idx": 26, "type": "ite", "entity": "电生理检查" } ] }, { "text": "如患儿稳定,首选腺苷快速静脉推注,因为腺苷起效快,对心肌收缩性影响小。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "pro", "entity": "腺苷快速静脉推注" }, { "start_idx": 19, "end_idx": 20, "type": "dru", "entity": "腺苷" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "心肌" } ] }, { "text": "去氧肾上腺素也可应用,通过压力反射来增加迷走张力。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dru", "entity": "去氧肾上腺素" } ] }, { "text": "此外,还可应用抗心律失常药物如奎尼丁、普鲁卡因胺及普萘洛尔。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "ite", "entity": "心律" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "奎尼丁" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "普鲁卡因胺" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "普萘洛尔" } ] }, { "text": "在年长儿,还可应用钙通道阻滞剂维拉帕米来终止发作。", "entities": [ { "start_idx": 15, "end_idx": 18, "type": "dru", "entity": "维拉帕米" } ] }, { "text": "在1岁以下婴儿,维拉帕米可减少心输出量、发生低血压及心搏停止,因而维拉帕米在1岁以下年龄禁用。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "维拉帕米" }, { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "心" }, { "start_idx": 22, "end_idx": 24, "type": "sym", "entity": "低血压" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "心搏停止" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "维拉帕米" } ] }, { "text": "在危重情况下,如已发生严重心力衰竭,首先推荐直流同步电复律。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 22, "end_idx": 28, "type": "pro", "entity": "直流同步电复律" } ] }, { "text": "在无旁道前传的患儿,地高辛或普萘洛尔是主要的治疗手段。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "地高辛" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "普萘洛尔" } ] }, { "text": "如有预激综合征,地高辛或钙通道阻滞剂可增加旁道前传,应予避免。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "预激综合征" }, { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "地高辛" }, { "start_idx": 12, "end_idx": 17, "type": "dru", "entity": "钙通道阻滞剂" } ] }, { "text": "这些患儿可长期口服普萘洛尔。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "dru", "entity": "普萘洛尔" } ] }, { "text": "在顽固的患儿,还可应用普鲁卡因胺、奎尼丁、氟卡因、普罗帕酮、索他洛尔或胺碘酮。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "普鲁卡因胺" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "奎尼丁" }, { "start_idx": 21, "end_idx": 23, "type": "dru", "entity": "氟卡因" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "普罗帕酮" }, { "start_idx": 30, "end_idx": 33, "type": "dru", "entity": "索他洛尔" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "胺碘酮" } ] }, { "text": "心脏正常的患儿如因长期心动过速而发生心力衰竭,心律转为窦性后心功能通常恢复正常,但这一过程可能需几天至几周。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心脏" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "心动过速" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "心力衰竭" } ] }, { "text": "如需应用几种药物控制发作,或药物的副作用不能耐受,药物的治疗效果差,可选择射频消融。", "entities": [ { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "射频消融" } ] }, { "text": "在部分旁道患儿,也可选用外科消融。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "外科消融" } ] }, { "text": "如用单一药物不能控制,可进行射频消融,国外报道成功率可达90%以上。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "射频消融" } ] }, { "text": "初期仅有轻度咳嗽、胸闷、胸痛及咳痰。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "轻度咳嗽" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "胸闷" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "胸痛" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "咳痰" } ] }, { "text": "叩诊可呈浊音,听诊多能闻及啰音或呼吸音粗糙。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "浊音" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "啰音或呼吸音粗糙" } ] }, { "text": "外周血白细胞计数正常或稍高,嗜酸性粒细胞增高,血沉增快。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "ite", "entity": "白细胞计数" }, { "start_idx": 14, "end_idx": 19, "type": "ite", "entity": "嗜酸性粒细胞" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "血沉" } ] }, { "text": "胸部X线摄片特征性的表现为小指甲乃至拇指甲大小、界限鲜明的结节状阴影1~2个,见于下肺野,多伴有环状透亮影,亦有呈毛囊状影,有时阴影直径可达5~6cm。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "胸部X线摄片" }, { "start_idx": 41, "end_idx": 43, "type": "bod", "entity": "下肺野" } ] }, { "text": "在流行地区对临床症状不明显的患者,应做皮内试验和补体结合试验以助诊断。", "entities": [ { "start_idx": 19, "end_idx": 22, "type": "ite", "entity": "皮内试验" }, { "start_idx": 24, "end_idx": 29, "type": "ite", "entity": "补体结合试验" } ] }, { "text": "亦可酌情选用硫氯酚、硝氯酚等。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dru", "entity": "硫氯酚" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "硝氯酚" } ] }, { "text": "肺部可有湿啰音。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 4, "end_idx": 6, "type": "sym", "entity": "湿啰音" } ] }, { "text": "部分患儿有新生儿期患结合膜炎的病史。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "结合膜炎" } ] }, { "text": "如病变侵犯细支气管,可出现喘息,偶见呼吸暂停。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "细支气管" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "喘息" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "呼吸暂停" } ] }, { "text": "鼻咽拭子可分离到沙眼衣原体,经酶联免疫吸附试验和微量免疫荧光试验可检测沙眼衣原体抗体。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "鼻咽拭子" }, { "start_idx": 15, "end_idx": 22, "type": "ite", "entity": "酶联免疫吸附试验" }, { "start_idx": 24, "end_idx": 31, "type": "ite", "entity": "微量免疫荧光试验" }, { "start_idx": 35, "end_idx": 41, "type": "mic", "entity": "沙眼衣原体抗体" } ] }, { "text": "PCR或DNA杂交技术可直接检测沙眼衣原体DNA,或通过ELISA等方法检测衣原体抗原。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "ite", "entity": "PCR" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "杂交技术" }, { "start_idx": 16, "end_idx": 23, "type": "mic", "entity": "沙眼衣原体DNA" }, { "start_idx": 28, "end_idx": 32, "type": "ite", "entity": "ELISA" } ] }, { "text": "治疗首选大环内酯类抗生素。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dru", "entity": "大环内酯类抗生素" } ] }, { "text": "重症或不能口服者静脉给药。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "静脉给药" } ] }, { "text": "本病第一胎即可发病,如果产前发病可导致脑积水、脑囊肿、癫痫等后遗症。", "entities": [ { "start_idx": 19, "end_idx": 21, "type": "dis", "entity": "脑积水" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "脑囊肿" }, { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "癫痫" } ] }, { "text": "至今SMA尚无特异的有效治疗,主要治疗措施为预防或治疗各种严重肌无力产生的并发症,如肺炎、营养不良、骨骼畸形、行动障碍和精神社会性问题等。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "SMA" }, { "start_idx": 29, "end_idx": 33, "type": "dis", "entity": "严重肌无力" }, { "start_idx": 42, "end_idx": 43, "type": "dis", "entity": "肺炎" }, { "start_idx": 45, "end_idx": 48, "type": "dis", "entity": "营养不良" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "骨骼畸形" }, { "start_idx": 55, "end_idx": 58, "type": "dis", "entity": "行动障碍" }, { "start_idx": 60, "end_idx": 66, "type": "dis", "entity": "精神社会性问题" } ] }, { "text": "5q13区存在2个SMN等位基因:SMN1和SMN2,只有SMN1基因的纯合缺失才会导致SMA,而SMN2基因的纯合缺失则出现在5%的正常人群中。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "5q13区" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "SMN" }, { "start_idx": 17, "end_idx": 20, "type": "dis", "entity": "SMN1" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "SMN2" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "SMN1基因" }, { "start_idx": 44, "end_idx": 46, "type": "dis", "entity": "SMA" }, { "start_idx": 49, "end_idx": 54, "type": "bod", "entity": "SMN2基因" } ] }, { "text": "96%的SMA患者提示有SMN1基因突变,而4%并非与5q13连锁。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "SMA" }, { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "SMN1基因" }, { "start_idx": 27, "end_idx": 32, "type": "bod", "entity": "5q13连锁" } ] }, { "text": "如果这些研究被进一步证实将为了解SMA的发病机制迈出重要一步。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "SMA" } ] }, { "text": "大多数患者为SMA-Ⅰ型,其次为Ⅱ型,Ⅲ型发病率最低。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "SMA-Ⅰ型" } ] }, { "text": "本病在儿童晚期或青春期出现症状,表现为神经元性近端肌萎缩,容易和肢带型肌营养不良相混淆。", "entities": [ { "start_idx": 19, "end_idx": 27, "type": "dis", "entity": "神经元性近端肌萎缩" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "肢带型肌营养不良" } ] }, { "text": "患儿常有磷酸肌酸激酶增高。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "sym", "entity": "磷酸肌酸激酶增高" } ] }, { "text": "应用PCR限制性内切酶方法,进行SMN基因外显子7、8的缺失检测,可快速诊断儿童型SMA。", "entities": [ { "start_idx": 2, "end_idx": 12, "type": "pro", "entity": "PCR限制性内切酶方法" }, { "start_idx": 16, "end_idx": 24, "type": "bod", "entity": "SMN基因外显子7" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "SMA" } ] }, { "text": "此外PCR-SSCP分析以及单体型连锁分析法也是诊断SMA的有效方法,三者联合使用可相互验证,互为补充,提高产前基因诊断的准确率。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "pro", "entity": "PCR-SSCP分析" }, { "start_idx": 14, "end_idx": 21, "type": "pro", "entity": "单体型连锁分析法" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "SMA" }, { "start_idx": 56, "end_idx": 59, "type": "pro", "entity": "基因诊断" } ] }, { "text": "有学者应用PCR和PCR内切酶法检测SMA患者基因缺失情况,结果显示SMA-Ⅰ型和Ⅱ型可通过SMN基因第7、8外显子的检测进行确诊,方法简便可靠。", "entities": [ { "start_idx": 5, "end_idx": 15, "type": "pro", "entity": "PCR和PCR内切酶法" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "SMA" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "SMA-Ⅰ型" }, { "start_idx": 46, "end_idx": 57, "type": "bod", "entity": "SMN基因第7、8外显子" } ] }, { "text": "并发症包括肺炎、营养不良、骨骼畸形、行动障碍和精神社会性问题。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "肺炎" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "营养不良" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "骨骼畸形" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "行动障碍" }, { "start_idx": 23, "end_idx": 29, "type": "dis", "entity": "精神社会性问题" } ] }, { "text": "一旦有效肺活量(FVC)下降,即使肢体或躯干的肌力无明显改变,发生肺炎的危险性也会增高。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "ite", "entity": "有效肺活量" }, { "start_idx": 8, "end_idx": 10, "type": "ite", "entity": "FVC" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "肢体" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "躯干" }, { "start_idx": 23, "end_idx": 24, "type": "ite", "entity": "肌力" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "肺炎" } ] }, { "text": "患儿由于负氮平衡会导致肌无力和疲劳加重,尤其是婴儿。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "肌无力" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "疲劳" } ] }, { "text": "部分患者发生慢性营养不良,表现为易于疲劳和储备下降;部分患者由于营养摄入不足导致有机酸尿症;故需要有专业的营养师对其进行喂养指导、调整喂养方案、喂养姿势以及食物结构,以最大限度地增加热量摄入。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "慢性营养不良" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "易于疲劳" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "储备下降" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "有机酸尿症" } ] }, { "text": "另外由于行动障碍,有些患者会发生便秘,但通过增加液体和纤维摄入,可减轻便秘。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "行动障碍" }, { "start_idx": 16, "end_idx": 17, "type": "dis", "entity": "便秘" }, { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "增加液体" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "纤维摄入" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "便秘" } ] }, { "text": "脊柱侧弯是SMA最为严重的骨骼畸形,不能行走的患者会更早地发展为脊柱畸形,大部分的畸形位于胸腰部。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "脊柱侧弯" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "SMA" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "骨骼畸形" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "脊柱畸形" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "胸" }, { "start_idx": 46, "end_idx": 46, "type": "bod", "entity": "腰" } ] }, { "text": "为了防止脊柱融合术后力量或功能的丧失,以及防止呼吸道的并发症,在术前术后需有积极的物理治疗。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "脊柱融合术" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "呼吸道" }, { "start_idx": 41, "end_idx": 44, "type": "pro", "entity": "物理治疗" } ] }, { "text": "畸形足是婴儿型SMA的一个表现,但较为少见,一般不需要外科矫正。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "畸形足" }, { "start_idx": 4, "end_idx": 9, "type": "dis", "entity": "婴儿型SMA" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "外科矫正" } ] }, { "text": "其他更常见的畸形有由于行动障碍导致的屈曲挛缩,迅速累及髋、膝和踝部,适当的锻炼可以预防挛缩的发生。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "行动障碍" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "屈曲挛缩" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "髋" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "膝" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "踝部" }, { "start_idx": 43, "end_idx": 44, "type": "dis", "entity": "挛缩" } ] }, { "text": "临床上多数风湿性疾病活动期都有ESR加快,下列情况也可引起ESR加快,如贫血、药物(肝素及某些避孕药)、妊娠及高免疫球蛋白血症(多发性骨髓瘤,Waldenstroms巨球蛋白血症),甚至性别、年龄、种族和肥胖都会影响血沉结果。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "风湿性疾病" }, { "start_idx": 15, "end_idx": 17, "type": "ite", "entity": "ESR" }, { "start_idx": 29, "end_idx": 31, "type": "ite", "entity": "ESR" }, { "start_idx": 36, "end_idx": 37, "type": "dis", "entity": "贫血" }, { "start_idx": 39, "end_idx": 40, "type": "dru", "entity": "药物" }, { "start_idx": 42, "end_idx": 43, "type": "dru", "entity": "肝素" }, { "start_idx": 47, "end_idx": 49, "type": "dru", "entity": "避孕药" }, { "start_idx": 55, "end_idx": 62, "type": "dis", "entity": "高免疫球蛋白血症" }, { "start_idx": 64, "end_idx": 69, "type": "dis", "entity": "多发性骨髓瘤" }, { "start_idx": 71, "end_idx": 88, "type": "dis", "entity": "Waldenstroms巨球蛋白血症" }, { "start_idx": 108, "end_idx": 109, "type": "ite", "entity": "血沉" } ] }, { "text": "猫和猫科动物为其终末宿主和传染源,猫在最初感染后由粪便排出大量卵囊,由于卵囊具有高度感染性并可持续存在于温暖潮湿的泥土中达一年或更久,因此,食入未洗的被猫粪污染的食物,或接触沙堆或其他这样受污染的地方可致感染,另外,输血或输白细胞、器官移植和实验室意外也可传播。", "entities": [ { "start_idx": 108, "end_idx": 109, "type": "pro", "entity": "输血" }, { "start_idx": 111, "end_idx": 114, "type": "pro", "entity": "输白细胞" }, { "start_idx": 116, "end_idx": 119, "type": "pro", "entity": "器官移植" } ] }, { "text": "在妊娠最初3个月内获得和传播的感染常引起婴儿出生时伴有严重的眼和中枢神经系统表现,如脉络膜视网膜炎、脑积水和颅内钙化。", "entities": [ { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "眼" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 42, "end_idx": 48, "type": "dis", "entity": "脉络膜视网膜炎" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "脑积水" }, { "start_idx": 54, "end_idx": 57, "type": "dis", "entity": "颅内钙化" } ] }, { "text": "发热性疾病、感冒、消化障碍以及过度疲劳等均可为诱因。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "发热性疾病" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "感冒" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "消化障碍" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "过度疲劳" } ] }, { "text": "在疱疹出现前2~3天(潜伏期)患儿常有烦躁、拒食、发热与局部淋巴结肿大。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "疱疹" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "烦躁" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "拒食" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "发热" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "局部淋巴结肿大" } ] }, { "text": "2~3天后体温下降,但口腔症状加重,病损最初表现为弥漫性黏膜潮红,在24小时内渐次出现密集成群的针尖大小水疱,呈圆形或椭圆形,周围环绕红晕,水疱很快破溃,暴露出表浅小溃疡或溃疡相互融合成大溃疡,表面覆有黄白色分泌物。", "entities": [ { "start_idx": 0, "end_idx": 16, "type": "sym", "entity": "2~3天后体温下降,但口腔症状加重" }, { "start_idx": 18, "end_idx": 31, "type": "sym", "entity": "病损最初表现为弥漫性黏膜潮红" }, { "start_idx": 33, "end_idx": 68, "type": "sym", "entity": "在24小时内渐次出现密集成群的针尖大小水疱,呈圆形或椭圆形,周围环绕红晕" }, { "start_idx": 70, "end_idx": 75, "type": "sym", "entity": "水疱很快破溃" }, { "start_idx": 77, "end_idx": 106, "type": "sym", "entity": "暴露出表浅小溃疡或溃疡相互融合成大溃疡,表面覆有黄白色分泌物" } ] }, { "text": "本病为自限性,1~2周内口腔黏膜恢复正常,溃疡愈合后不留瘢痕。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "bod", "entity": "口腔黏膜" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "溃疡" } ] }, { "text": "疱底细胞、病毒分离和血清学实验可帮助诊断。", "entities": [ { "start_idx": 0, "end_idx": 14, "type": "pro", "entity": "疱底细胞、病毒分离和血清学实验" } ] }, { "text": "口腔护理是必要的,包括保持口腔清洁、勤喂水,禁用刺激性、腐蚀性、酸性或过热的食品、饮料及药物。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "口腔护理" }, { "start_idx": 11, "end_idx": 16, "type": "pro", "entity": "保持口腔清洁" }, { "start_idx": 18, "end_idx": 20, "type": "pro", "entity": "勤喂水" } ] }, { "text": "对DMD的基因检测技术包括DNA印记法杂交、限制性片段长度多态性的连锁分析及缺失热点外显子的聚合酶链反应(PCR),进行DMD的基因诊断,但DMD基因庞大,自发突变率高,因此对于点突变型DMD的诊断尚缺乏系统的研究。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "DMD" }, { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "基因检测" }, { "start_idx": 13, "end_idx": 20, "type": "pro", "entity": "DNA印记法杂交" }, { "start_idx": 22, "end_idx": 36, "type": "pro", "entity": "限制性片段长度多态性的连锁分析" }, { "start_idx": 38, "end_idx": 51, "type": "pro", "entity": "缺失热点外显子的聚合酶链反应" }, { "start_idx": 53, "end_idx": 55, "type": "pro", "entity": "PCR" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "DMD" }, { "start_idx": 64, "end_idx": 65, "type": "bod", "entity": "基因" }, { "start_idx": 70, "end_idx": 72, "type": "dis", "entity": "DMD" }, { "start_idx": 73, "end_idx": 74, "type": "bod", "entity": "基因" }, { "start_idx": 89, "end_idx": 95, "type": "dis", "entity": "点突变型DMD" } ] }, { "text": "利用肾上腺皮质激素和联苯双酯等可降低血清酶水平。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "联苯双酯" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "血清酶" } ] }, { "text": "有人用别嘌呤醇治疗本病,患者的临床症状有所好转,血清CK下降。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "pro", "entity": "别嘌呤醇治疗" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "血清CK下降" } ] }, { "text": "有人提出早期给予乳酸钠,可增强患者的肌力。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "乳酸钠" }, { "start_idx": 18, "end_idx": 19, "type": "ite", "entity": "肌力" } ] }, { "text": "因此,常需早期进行光照治疗,严重者则需要换血,以防核黄疸的发生。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "光照治疗" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "换血" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "核黄疸" } ] }, { "text": "发生于幼儿或儿童期者,可轻重不一,轻者可终生无症状,重者可发生严重贫血伴有苍白、黄疸、乏力及运动耐力下降。", "entities": [ { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "苍白" }, { "start_idx": 40, "end_idx": 41, "type": "dis", "entity": "黄疸" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "乏力" }, { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "运动耐力下降" } ] }, { "text": "但也有严重者可出现高输出量心功能衰竭和循环衰竭、严重缺氧甚至死亡。", "entities": [ { "start_idx": 9, "end_idx": 17, "type": "dis", "entity": "高输出量心功能衰竭" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "循环衰竭" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "严重缺氧" } ] }, { "text": "虽然胆石症可发生于早至4~5岁的患儿,但大多见于年长儿童;如未行脾切除者,约有50%的病例将来可合并胆石症。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "胆石症" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "脾" }, { "start_idx": 50, "end_idx": 52, "type": "dis", "entity": "胆石症" } ] }, { "text": "血涂片显微镜检查可见数目不等的球形红细胞,MCV、MCH多数正常,MCHC增加(360~380g/L),红细胞直径缩短,但体积正常。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "pro", "entity": "显微镜" }, { "start_idx": 15, "end_idx": 19, "type": "bod", "entity": "球形红细胞" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "MCV" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "MCH" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "MCHC" }, { "start_idx": 52, "end_idx": 54, "type": "bod", "entity": "红细胞" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "直径缩短" }, { "start_idx": 61, "end_idx": 64, "type": "sym", "entity": "体积正常" } ] }, { "text": "8.单链构象多态性分析(SSCP)、PCR-核苷酸测序等可确定基因突变点。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "pro", "entity": "单链构象多态性分析" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "SSCP" }, { "start_idx": 18, "end_idx": 26, "type": "pro", "entity": "PCR-核苷酸测序" } ] }, { "text": "HS须与其他有球形红细胞的溶血性贫血病症鉴别。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "HS" }, { "start_idx": 7, "end_idx": 11, "type": "bod", "entity": "球形红细胞" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "溶血性贫血病" } ] }, { "text": "其他类型的免疫性贫血如自身免疫性溶血性贫血、药物引起溶血性贫血、输血后溶血反应等,这些病例可因Coombs试验阳性确诊,但HS则Coombs试验阴性。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dis", "entity": "免疫性贫血" }, { "start_idx": 11, "end_idx": 20, "type": "dis", "entity": "自身免疫性溶血性贫血" }, { "start_idx": 22, "end_idx": 30, "type": "dis", "entity": "药物引起溶血性贫血" }, { "start_idx": 32, "end_idx": 36, "type": "dis", "entity": "输血后溶血" }, { "start_idx": 47, "end_idx": 54, "type": "pro", "entity": "Coombs试验" }, { "start_idx": 61, "end_idx": 62, "type": "dis", "entity": "HS" }, { "start_idx": 64, "end_idx": 71, "type": "pro", "entity": "Coombs试验" } ] }, { "text": "但它们均存在原发病因,一般不难鉴别,必要时可作红细胞膜蛋白电泳分析,继发HS者应无连接蛋白、锚蛋白、蛋白3等的缺乏。", "entities": [ { "start_idx": 23, "end_idx": 32, "type": "pro", "entity": "红细胞膜蛋白电泳分析" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "继发HS" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "连接蛋白" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "锚蛋白" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "蛋白3" } ] }, { "text": "发生严重溶血或溶血危象时应输注红细胞,发生再障危象时需输注红细胞,必要时加输血小板。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "溶血危象" }, { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "输注" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "红细胞" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "再障危象" }, { "start_idx": 27, "end_idx": 28, "type": "pro", "entity": "输注" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "红细胞" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "血小板" } ] }, { "text": "用于治疗隐睾的激素,主要有绒毛膜促性腺激素(HCG)、黄体生成激素释放激素(LHRH)和促性腺激素释放激素(GnRH)。", "entities": [ { "start_idx": 13, "end_idx": 20, "type": "dru", "entity": "绒毛膜促性腺激素" }, { "start_idx": 22, "end_idx": 24, "type": "dru", "entity": "HCG" }, { "start_idx": 27, "end_idx": 36, "type": "dru", "entity": "黄体生成激素释放激素" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "LHRH" }, { "start_idx": 44, "end_idx": 52, "type": "dru", "entity": "促性腺激素释放激素" }, { "start_idx": 54, "end_idx": 57, "type": "dru", "entity": "GnRH" } ] }, { "text": "人的一生中,都可能在某个特定时期,特定条件下出现这样那样的SID,因而早期诊断和治疗对SID具有重大意义。", "entities": [ { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "SID" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "SID" } ] }, { "text": "这种情况常伴有小型室间隔缺损,后者临床上可不明显,或已自发性关闭。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "小型室间隔缺损" } ] }, { "text": "继发于左心室显著肥厚的弥漫性室间隔肥厚可突入右室或流出道,因而造成梗阻(Bernheim效应)。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "左心室" }, { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "弥漫性室间隔肥厚" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "右室" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "梗阻" }, { "start_idx": 36, "end_idx": 45, "type": "dis", "entity": "Bernheim效应" } ] }, { "text": "这些表现的存在应怀疑肺动脉瓣下狭窄的存在,但明确诊断有赖于超声、心导管及心血管造影术。", "entities": [ { "start_idx": 10, "end_idx": 16, "type": "dis", "entity": "肺动脉瓣下狭窄" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "超声" }, { "start_idx": 32, "end_idx": 34, "type": "pro", "entity": "心导管" }, { "start_idx": 36, "end_idx": 41, "type": "pro", "entity": "心血管造影术" } ] }, { "text": "急性中毒是儿科的常见急症之一,儿童以食入中毒最多见,年龄多见于1~5岁。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "急性中毒" }, { "start_idx": 5, "end_idx": 6, "type": "dep", "entity": "儿科" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "食入中毒" } ] }, { "text": "因此,在遇到急性中毒时,家长应尽可能提供毒物;另外,即使对于可疑中毒者,亦应及早给予治疗处理,争取抢救时间,避免中毒进一步加重,降低和减少病死率及后遗症。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dis", "entity": "急性中毒" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "中毒" }, { "start_idx": 56, "end_idx": 57, "type": "dis", "entity": "中毒" } ] }, { "text": "毒物进入消化道后,毒物对肠道的直接刺激以及破坏消化道局部组织,可引起腹痛、恶心、呕吐和腹泻等症状。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "消化道" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肠道" }, { "start_idx": 23, "end_idx": 29, "type": "bod", "entity": "消化道局部组织" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "腹痛" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "恶心" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "呕吐" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "腹泻" } ] }, { "text": "消化道症状严重者常会伴随发生脱水、酸中毒、电解质紊乱等症状。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "消化道" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "消化道症状" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "脱水" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "酸中毒" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "电解质紊乱" } ] }, { "text": "其中部分患儿在急性中毒时出现致死性的心力衰竭和休克。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "急性中毒" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "致死性的心力衰竭" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "休克" } ] }, { "text": "原因有两种:一种为毒物直接作用于心肌,引起心肌功能障碍和心力衰竭;另一种为毒物通过对血管及神经系统的作用,抑制氧摄取和氧代谢,导致严重心律失常、低血压或电解质代谢紊乱,最终引起继发性心力衰竭。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "心肌" }, { "start_idx": 21, "end_idx": 26, "type": "sym", "entity": "心肌功能障碍" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "血管" }, { "start_idx": 45, "end_idx": 48, "type": "bod", "entity": "神经系统" }, { "start_idx": 65, "end_idx": 70, "type": "sym", "entity": "严重心律失常" }, { "start_idx": 72, "end_idx": 74, "type": "sym", "entity": "低血压" }, { "start_idx": 76, "end_idx": 82, "type": "sym", "entity": "电解质代谢紊乱" }, { "start_idx": 88, "end_idx": 94, "type": "sym", "entity": "继发性心力衰竭" } ] }, { "text": "中毒患儿可出现刺激性呛咳、呼吸困难、发绀、肺水肿及呼吸节律不整,严重者导致呼吸中枢抑制或呼吸肌麻痹以及呼吸衰竭。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "中毒" }, { "start_idx": 7, "end_idx": 11, "type": "sym", "entity": "刺激性呛咳" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "发绀" }, { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "肺" }, { "start_idx": 21, "end_idx": 23, "type": "sym", "entity": "肺水肿" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "呼吸节律不整" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "呼吸中枢抑制" }, { "start_idx": 44, "end_idx": 48, "type": "sym", "entity": "呼吸肌麻痹" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "呼吸衰竭" } ] }, { "text": "当神经系统受到毒素直接损害或中毒后的缺血缺氧损伤后,可继而发生神经功能失调,严重者出现脑器质性破坏和功能衰竭。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "bod", "entity": "神经系统" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "中毒" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "缺血缺氧损伤" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "神经功能失调" }, { "start_idx": 43, "end_idx": 43, "type": "bod", "entity": "脑" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "脑器质性破坏" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "功能衰竭" } ] }, { "text": "临床相关症状有烦躁、惊厥、瘫痪、昏迷、去大脑强直以及中枢性呼吸衰竭和神经源性休克。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "烦躁" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "惊厥" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "瘫痪" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "昏迷" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "大脑" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "去大脑强直" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "中枢性呼吸衰竭" }, { "start_idx": 34, "end_idx": 39, "type": "sym", "entity": "神经源性休克" } ] }, { "text": "在度过急性中毒急性期后,部分患儿可遗留后遗症,如腐蚀性毒物中毒引起的消化道变形和狭窄,影响正常饮食;脑部中毒损害或严重缺氧后发生精神运动功能障碍等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "急性中毒" }, { "start_idx": 34, "end_idx": 41, "type": "sym", "entity": "消化道变形和狭窄" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "脑部中毒" }, { "start_idx": 64, "end_idx": 71, "type": "sym", "entity": "精神运动功能障碍" } ] }, { "text": "对诊断一时不明确且伴昏迷者,应与下列疾病进行鉴别:①低血糖;②酮症酸中毒;③颅内出血;④中枢感染;⑤肝性脑病;⑥尿毒症;⑦电解质紊乱。", "entities": [ { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "低血糖" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "酮症酸中毒" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "颅内出血" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "中枢感染" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "肝性脑病" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "尿毒症" }, { "start_idx": 61, "end_idx": 65, "type": "dis", "entity": "电解质紊乱" } ] }, { "text": "急性中毒伴有下列表现时,提示病情危重:①深昏迷;②休克或血压不稳定;③高热或体温不升;④呼吸衰竭;⑤心力衰竭或严重心律失常;⑥惊厥持续状态;⑦肾衰竭;⑧DIC;⑨血钠高于150mmol/L或低于120mmol/L。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "急性中毒" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "深昏迷" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "休克" }, { "start_idx": 28, "end_idx": 32, "type": "sym", "entity": "血压不稳定" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "高热" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "体温不升" }, { "start_idx": 44, "end_idx": 47, "type": "sym", "entity": "呼吸衰竭" }, { "start_idx": 50, "end_idx": 53, "type": "sym", "entity": "心力衰竭" }, { "start_idx": 55, "end_idx": 60, "type": "sym", "entity": "严重心律失常" }, { "start_idx": 63, "end_idx": 68, "type": "sym", "entity": "惊厥持续状态" }, { "start_idx": 71, "end_idx": 73, "type": "sym", "entity": "肾衰竭" }, { "start_idx": 76, "end_idx": 78, "type": "sym", "entity": "DIC" }, { "start_idx": 81, "end_idx": 82, "type": "ite", "entity": "血钠" }, { "start_idx": 81, "end_idx": 105, "type": "sym", "entity": "血钠高于150mmol/L或低于120mmol/L" } ] }, { "text": "有毒气体(氯气、一氧化碳等)中毒者应首先脱离中毒环境,加强通风,积极吸氧,以排除呼吸道内残留毒气。", "entities": [ { "start_idx": 40, "end_idx": 42, "type": "bod", "entity": "呼吸道" } ] }, { "text": "食入水溶性毒物者可用胃肠道脱毒方法,包括催吐、洗胃、导泻和胃肠内吸附毒物。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "胃肠道脱毒" }, { "start_idx": 20, "end_idx": 21, "type": "pro", "entity": "催吐" }, { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "洗胃" }, { "start_idx": 26, "end_idx": 27, "type": "pro", "entity": "导泻" }, { "start_idx": 29, "end_idx": 35, "type": "pro", "entity": "胃肠内吸附毒物" } ] }, { "text": "透析疗法为中毒治疗中的重要措施之一。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "透析疗法" }, { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "中毒" } ] }, { "text": "如经初步治疗后症状无好转或继续加重,可考虑做腹膜透析或血液透析治疗。", "entities": [ { "start_idx": 22, "end_idx": 25, "type": "pro", "entity": "腹膜透析" }, { "start_idx": 27, "end_idx": 30, "type": "pro", "entity": "血液透析" } ] }, { "text": "惊厥者给予地西泮、肾上腺皮质激素,并注意呼吸支持。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dru", "entity": "地西泮" }, { "start_idx": 9, "end_idx": 15, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "呼吸支持" } ] }, { "text": "严重者可给予透析治疗。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "透析" } ] }, { "text": "重者可予透析治疗。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "透析治疗" } ] }, { "text": "重者加用肾上腺皮质激素,透析治疗,并给予生命支持。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 12, "end_idx": 13, "type": "pro", "entity": "透析" }, { "start_idx": 20, "end_idx": 23, "type": "pro", "entity": "生命支持" } ] }, { "text": "解磷定15~30mg/kg,每4~12小时静滴一次,或氯解磷定10~15mg/kg,肌内注射,2~3小时后可重复1次,直至血胆碱酯酶恢复正常。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "解磷定" }, { "start_idx": 21, "end_idx": 22, "type": "pro", "entity": "静滴" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "氯解磷定" }, { "start_idx": 42, "end_idx": 45, "type": "pro", "entity": "肌内注射" }, { "start_idx": 61, "end_idx": 65, "type": "bod", "entity": "血胆碱酯酶" } ] }, { "text": "伤口扩创,用依地酸钙钠、过氧化氢、呋喃西林溶液、冷开水或1∶5000高锰酸钾溶液冲洗,反复吸引毒液;无条件扩创可用火焰烧灼伤口,破坏毒素;皮下注射多价或特异性抗蛇毒血清,口服或注射蛇药;补液利尿,重者给予肾上腺皮质激素。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "扩创" }, { "start_idx": 50, "end_idx": 54, "type": "pro", "entity": "无条件扩创" }, { "start_idx": 57, "end_idx": 62, "type": "pro", "entity": "火焰烧灼伤口" }, { "start_idx": 69, "end_idx": 72, "type": "pro", "entity": "皮下注射" }, { "start_idx": 73, "end_idx": 74, "type": "dru", "entity": "多价" }, { "start_idx": 76, "end_idx": 83, "type": "dru", "entity": "特异性抗蛇毒血清" }, { "start_idx": 88, "end_idx": 89, "type": "pro", "entity": "注射" }, { "start_idx": 90, "end_idx": 91, "type": "dru", "entity": "蛇药" }, { "start_idx": 102, "end_idx": 108, "type": "dru", "entity": "肾上腺皮质激素" } ] }, { "text": "扩创后局部可注射3%依米丁1ml(稀释至4~9ml),或用蛇药外敷;输液利尿;重者给予肾上腺皮质激素,防治过敏性休克及肺水肿,生命功能支持。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "扩创" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "注射" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "依米丁" }, { "start_idx": 29, "end_idx": 30, "type": "dru", "entity": "蛇药" }, { "start_idx": 31, "end_idx": 32, "type": "pro", "entity": "外敷" }, { "start_idx": 43, "end_idx": 49, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "过敏性休克" }, { "start_idx": 59, "end_idx": 59, "type": "bod", "entity": "肺" }, { "start_idx": 59, "end_idx": 61, "type": "sym", "entity": "肺水肿" }, { "start_idx": 63, "end_idx": 68, "type": "pro", "entity": "生命功能支持" } ] }, { "text": "近年来选择性冠状动脉造影和超声检查的广泛开展,冠状动脉畸形更加引起临床界的注意。", "entities": [ { "start_idx": 3, "end_idx": 11, "type": "pro", "entity": "选择性冠状动脉造影" }, { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "超声检查" }, { "start_idx": 23, "end_idx": 28, "type": "dis", "entity": "冠状动脉畸形" } ] }, { "text": "患有本畸形的患儿常有阵发性烦躁不安及哭吵,似与疼痛有关,并伴有苍白与出汗,但更常见的为喂养困难、呼吸急促、呼吸道症状及其他左心衰竭的表现。", "entities": [ { "start_idx": 10, "end_idx": 19, "type": "sym", "entity": "阵发性烦躁不安及哭吵" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "疼痛" }, { "start_idx": 29, "end_idx": 35, "type": "sym", "entity": "伴有苍白与出汗" }, { "start_idx": 43, "end_idx": 46, "type": "sym", "entity": "喂养困难" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 53, "end_idx": 57, "type": "sym", "entity": "呼吸道症状" }, { "start_idx": 61, "end_idx": 64, "type": "dis", "entity": "左心衰竭" } ] }, { "text": "心脏常严重扩大,由于二尖瓣环扩张或乳头肌梗死,可出现二尖瓣关闭不全的杂音。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "心脏" }, { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "二尖瓣环扩张" }, { "start_idx": 17, "end_idx": 21, "type": "sym", "entity": "乳头肌梗死" }, { "start_idx": 26, "end_idx": 35, "type": "sym", "entity": "二尖瓣关闭不全的杂音" } ] }, { "text": "胸片可显示心脏扩大、慢性肺静脉充血。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "胸片" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "心脏扩大" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "慢性肺静脉充血" } ] }, { "text": "心内膜弹力纤维增生症、心肌炎、糖原累积病累及心脏可有类似表现。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "心内膜弹力纤维增生症" }, { "start_idx": 15, "end_idx": 19, "type": "dis", "entity": "糖原累积病" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "心脏" } ] }, { "text": "目前治疗方法主要有两种:左冠状动脉重新植入主动脉或主动脉冠状动脉间搭桥。", "entities": [ { "start_idx": 12, "end_idx": 23, "type": "pro", "entity": "左冠状动脉重新植入主动脉" }, { "start_idx": 25, "end_idx": 34, "type": "pro", "entity": "主动脉冠状动脉间搭桥" } ] }, { "text": "如这些方法行不通时,可结扎异常的左冠状动脉,这样不仅能消除分流,还可通过侧支血管更好地供应存活心肌。", "entities": [ { "start_idx": 11, "end_idx": 20, "type": "pro", "entity": "结扎异常的左冠状动脉" }, { "start_idx": 36, "end_idx": 39, "type": "bod", "entity": "侧支血管" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "心肌" } ] }, { "text": "可有心肌缺血症状或猝死。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "心肌缺血" }, { "start_idx": 9, "end_idx": 10, "type": "dis", "entity": "猝死" } ] }, { "text": "诊断依赖于心血管造影,但目前无特殊治疗方法。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "pro", "entity": "心血管造影" } ] }, { "text": "1959年证实该病因为性染色体X呈单体性所致。", "entities": [ { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "性染色体X" } ] }, { "text": "Turner综合征的表型是女性,其发生率低是因为X单体的胚胎不易存活,99%的病例发生流产。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 24, "end_idx": 29, "type": "bod", "entity": "X单体的胚胎" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "流产" } ] }, { "text": "患者为女性表型,生长缓慢,成年期身高约135~140cm。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "生长缓慢" } ] }, { "text": "颈短,50%有颈蹼,后发际低,两乳头距离增宽,随年龄增长而乳头色素变深。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "颈短" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "颈蹼" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "后发际低" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "两乳头距离增宽" }, { "start_idx": 23, "end_idx": 34, "type": "sym", "entity": "随年龄增长而乳头色素变深" } ] }, { "text": "还有肘外翻及皮肤多痣等症状。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "肘外翻" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "皮肤多痣" } ] }, { "text": "约35%患儿伴有心脏畸形,以主动脉缩窄为多见。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "心脏畸形" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "主动脉缩窄" } ] }, { "text": "患者多因生长迟缓、青春期无性征发育以及原发性闭经等而就诊,其血清FSH及LH在婴儿期即已升高,而雌二醇水平很低。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "生长迟缓" }, { "start_idx": 9, "end_idx": 16, "type": "sym", "entity": "青春期无性征发育" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "原发性闭经" }, { "start_idx": 30, "end_idx": 37, "type": "ite", "entity": "血清FSH及LH" }, { "start_idx": 30, "end_idx": 45, "type": "sym", "entity": "血清FSH及LH在婴儿期即已升高" }, { "start_idx": 48, "end_idx": 52, "type": "ite", "entity": "雌二醇水平" }, { "start_idx": 48, "end_idx": 54, "type": "sym", "entity": "雌二醇水平很低" } ] }, { "text": "有时伴有一些先天畸形,构成各种综合征,但无颈短、后发际低及肘外翻等Turner综合征的表现。", "entities": [ { "start_idx": 21, "end_idx": 21, "type": "bod", "entity": "颈" }, { "start_idx": 33, "end_idx": 41, "type": "dis", "entity": "Turner综合征" }, { "start_idx": 20, "end_idx": 41, "type": "sym", "entity": "无颈短、后发际低及肘外翻等Turner综合征" } ] }, { "text": "主要病理表现结缔组织炎性细胞浸润,血管内膜增生,血管壁萎缩、纤维化,结果造成管腔狭窄或闭塞。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "bod", "entity": "结缔组织" }, { "start_idx": 6, "end_idx": 15, "type": "sym", "entity": "结缔组织炎性细胞浸润" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "血管内膜" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "血管内膜增生" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "血管壁" }, { "start_idx": 24, "end_idx": 28, "type": "sym", "entity": "血管壁萎缩" }, { "start_idx": 30, "end_idx": 32, "type": "sym", "entity": "纤维化" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "管腔" }, { "start_idx": 38, "end_idx": 44, "type": "sym", "entity": "管腔狭窄或闭塞" } ] }, { "text": "系统性硬化症患儿发生雷诺现象。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "系统性硬化症" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "雷诺现象" } ] }, { "text": "系统性硬化症的预后主要依据受累的系统。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "系统性硬化症" } ] }, { "text": "维生素K缺乏症是由于维生素K缺乏引起的凝血障碍性疾病。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "维生素K缺乏症" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "维生素K" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "凝血障碍性疾病" } ] }, { "text": "各地众多有关婴儿维生素K缺乏性出血症的研究表明,维生素K缺乏是世界性婴儿发病和死亡的重要原因。", "entities": [ { "start_idx": 8, "end_idx": 17, "type": "dis", "entity": "维生素K缺乏性出血症" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "维生素K缺乏" } ] }, { "text": "1995年,Sutor等报道,该病病死率为19%~33%,21%~67%的患者遗留神经系统后遗症。", "entities": [ { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "神经系统后遗症" } ] }, { "text": "迟发型者约90%以上见于单纯母乳喂养儿,单纯母乳喂养儿维生素K缺乏性出血的机会是人工喂养儿的15~20倍,如合并腹泻、使用抗生素、肝胆疾病和长期禁食患儿更易发生,常见急性或亚急性颅内出血,以蛛网膜下腔、硬膜下、硬膜外出血为多见,脑室、脑实质出血少见,临床上有严重的中枢神经系统功能失常及颅内高压的表现,表现为高声尖叫、频繁呕吐、反复抽搐,严重的患儿可出现昏迷。", "entities": [ { "start_idx": 27, "end_idx": 35, "type": "sym", "entity": "维生素K缺乏性出血" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "腹泻" }, { "start_idx": 61, "end_idx": 63, "type": "dru", "entity": "抗生素" }, { "start_idx": 65, "end_idx": 68, "type": "dis", "entity": "肝胆疾病" }, { "start_idx": 83, "end_idx": 92, "type": "sym", "entity": "急性或亚急性颅内出血" }, { "start_idx": 95, "end_idx": 109, "type": "sym", "entity": "蛛网膜下腔、硬膜下、硬膜外出血" }, { "start_idx": 114, "end_idx": 121, "type": "sym", "entity": "脑室、脑实质出血" }, { "start_idx": 132, "end_idx": 141, "type": "sym", "entity": "中枢神经系统功能失常" }, { "start_idx": 143, "end_idx": 146, "type": "sym", "entity": "颅内高压" }, { "start_idx": 154, "end_idx": 157, "type": "sym", "entity": "高声尖叫" }, { "start_idx": 159, "end_idx": 162, "type": "sym", "entity": "频繁呕吐" }, { "start_idx": 164, "end_idx": 167, "type": "sym", "entity": "反复抽搐" }, { "start_idx": 177, "end_idx": 178, "type": "sym", "entity": "昏迷" } ] }, { "text": "迟发性新生儿出血症,大多表现为颅内出血、烦躁不安、脑性尖叫、拒奶、嗜睡。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "迟发性新生儿出血症" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "颅内出血" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "脑性尖叫" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "拒奶" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "嗜睡" } ] }, { "text": "体检发现前囟饱满,颅缝增宽,Moro反射、觅食反射消失。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "体检" }, { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "前囟饱满" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "颅缝增宽" }, { "start_idx": 14, "end_idx": 26, "type": "sym", "entity": "Moro反射、觅食反射消失" } ] }, { "text": "进行B超、CT及MRI检查有助于诊断,不仅可确定出血部位、范围,还可随访疗效,进行预后判断。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "B超" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "CT" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "MRI检查" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "出血" } ] }, { "text": "幕上肿瘤尚可伴有癫痫及局部脑损害症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "幕上肿瘤" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "癫痫" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "脑损害" } ] }, { "text": "幕下肿瘤可有眼震颤、肢体共济运动差等小脑损害症状及低位脑神经(Ⅵ、Ⅶ、Ⅷ、Ⅸ、Ⅹ)损害症,呕吐也很常见,多与肿瘤刺激第四脑室底的延髓呕吐中枢有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "幕下肿瘤" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "眼震颤" }, { "start_idx": 10, "end_idx": 16, "type": "sym", "entity": "肢体共济运动差" }, { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "小脑损害症状" }, { "start_idx": 25, "end_idx": 43, "type": "dis", "entity": "低位脑神经(Ⅵ、Ⅶ、Ⅷ、Ⅸ、Ⅹ)损害症" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "呕吐" }, { "start_idx": 54, "end_idx": 55, "type": "dis", "entity": "肿瘤" }, { "start_idx": 58, "end_idx": 62, "type": "bod", "entity": "第四脑室底" }, { "start_idx": 64, "end_idx": 69, "type": "bod", "entity": "延髓呕吐中枢" } ] }, { "text": "另外,随着维生素A强化食品的发展或大量滥用维生素A制剂而导致维生素A中毒的现象也有增多的趋势。", "entities": [ { "start_idx": 30, "end_idx": 35, "type": "dis", "entity": "维生素A中毒" } ] }, { "text": "维生素D缺乏可致佝偻病、骨软化和骨质疏松;而维生素D中毒则表现为:高钙血症、高尿钙症和软组织内的钙沉积(肌肉乏力、关节疼痛),临床上还表现为消化道症状和烦躁等。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "维生素D缺乏" }, { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "佝偻病" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "骨软化" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "骨质疏松" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "维生素D中毒" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "高钙血症" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "高尿钙症" }, { "start_idx": 43, "end_idx": 45, "type": "bod", "entity": "软组织" }, { "start_idx": 43, "end_idx": 50, "type": "sym", "entity": "软组织内的钙沉积" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "肌肉乏力" }, { "start_idx": 57, "end_idx": 60, "type": "sym", "entity": "关节疼痛" }, { "start_idx": 70, "end_idx": 72, "type": "bod", "entity": "消化道" }, { "start_idx": 70, "end_idx": 74, "type": "sym", "entity": "消化道症状" }, { "start_idx": 76, "end_idx": 77, "type": "sym", "entity": "烦躁" } ] }, { "text": "其保护血管内皮屏障、改善微循环的作用,有利于预防动脉粥样硬化及相关的心血管疾病;另外,还能保持红细胞膜的完整性和抑制血栓的形成,并有一定的抗风湿和抗癌作用;其抗氧化作用与硒相互协同,共同防止多不饱和脂肪酸被氧化成过氧化脂质。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "bod", "entity": "血管内皮" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "动脉粥样硬化" }, { "start_idx": 34, "end_idx": 38, "type": "dis", "entity": "心血管疾病" }, { "start_idx": 47, "end_idx": 50, "type": "bod", "entity": "红细胞膜" }, { "start_idx": 58, "end_idx": 59, "type": "dis", "entity": "血栓" }, { "start_idx": 70, "end_idx": 71, "type": "dis", "entity": "风湿" } ] }, { "text": "病程发展缓慢,直到成年后因肺纤维增生可出现咳嗽、气短,严重者当心肺功能不全时出现呼吸困难、发绀及杵状指(趾)。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "肺纤维" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "咳嗽" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "气短" }, { "start_idx": 31, "end_idx": 36, "type": "dis", "entity": "心肺功能不全" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "发绀" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "杵状" } ] }, { "text": "少数患者有反复呼吸道感染史。", "entities": [ { "start_idx": 7, "end_idx": 12, "type": "dis", "entity": "呼吸道感染史" } ] }, { "text": "确诊有赖于肺活检。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "肺活检" } ] }, { "text": "但典型X线胸片、高分辨CT、支气管肺泡灌洗有较高的诊断价值。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "ite", "entity": "X线胸片" }, { "start_idx": 8, "end_idx": 12, "type": "ite", "entity": "高分辨CT" }, { "start_idx": 14, "end_idx": 20, "type": "ite", "entity": "支气管肺泡灌洗" } ] }, { "text": "病毒主要通过空气飞沫传播。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" } ] }, { "text": "成人型CML的药物治疗也可用于JCML治疗。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "成人型CML" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "JCML" } ] }, { "text": "异基因骨髓移植是唯一有可能获得长期无病生存的治疗方法。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "异基因骨髓移植" } ] }, { "text": "因某种原因使这种活瓣样功能受损时,尿液即倒流入输尿管,严重时到达肾脏,这种现象称膀胱输尿管反流。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "尿液" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "输尿管" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肾脏" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "膀胱输尿管反流" } ] }, { "text": "膀胱输尿管反流分为原发性和继发性两种。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "原发性" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "继发性" } ] }, { "text": "前者系活瓣机能先天性发育不全,后者继发于下尿路梗阻,如后尿道瓣膜及神经源性膀胱等。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "活瓣机能先天性发育不全" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "下尿路梗阻" }, { "start_idx": 27, "end_idx": 31, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "1958年Politano-Leadbetter首先报道采用抗反流的输尿管膀胱再吻合术来解决膀胱输尿管反流。", "entities": [ { "start_idx": 30, "end_idx": 42, "type": "pro", "entity": "抗反流的输尿管膀胱再吻合术" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "膀胱输尿管反流" } ] }, { "text": "胎儿肾积水中,由反流引起的也很常见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "胎儿肾积水" } ] }, { "text": "种族与原发性膀胱输尿管反流也有关,如白人女孩是黑人女孩的10倍,但一旦发生,其程度和自然缓解的可能性没有区别。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "原发性膀胱输尿管反流" } ] }, { "text": "原发性膀胱输尿管反流与遗传之间的关系也有报告。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "原发性膀胱输尿管反流" } ] }, { "text": "故现提倡凡直系亲属有反流病史的均应接受排尿性膀胱尿道造影(VCUG)筛查。", "entities": [ { "start_idx": 19, "end_idx": 27, "type": "pro", "entity": "排尿性膀胱尿道造影" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "VCUG" } ] }, { "text": "此外,输尿管旁憩室、输尿管开口于膀胱憩室内、异位输尿管口以及膀胱功能紊乱,也可造成膀胱输尿管反流。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "输尿管" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "输尿管" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "膀胱" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "异位输尿管口" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "膀胱" }, { "start_idx": 41, "end_idx": 47, "type": "dis", "entity": "膀胱输尿管反流" } ] }, { "text": "1959年Hodson首先发现肾瘢痕多见于泌尿系感染反复发作的小儿,并观察到有肾瘢痕的小儿中97%有膀胱输尿管反流,因此提出“反流性肾病”这一概念。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "泌尿系感染" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 50, "end_idx": 56, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 63, "end_idx": 67, "type": "dis", "entity": "反流性肾病" } ] }, { "text": "高血压的发生与肾瘢痕有关,肾瘢痕越多,发生高血压的危险越高,患双侧严重肾瘢痕的小儿随访20年以上,20%有高血压,单侧病变者为8%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "高血压" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 13, "end_idx": 28, "type": "sym", "entity": "肾瘢痕越多,发生高血压的危险越高" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "高血压" } ] }, { "text": "因此凡超声发现的肾积水都应行VCUG,以排除反流。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "超声" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "VCUG" } ] }, { "text": "由于相当一部分患儿是无症状反流,在高危人群中用超声进行反流筛查有实际意义。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "超声" } ] }, { "text": "因某种原因使这种活瓣样功能受损时,尿液即倒流入输尿管,严重时到达肾脏,这种现象称膀胱输尿管反流。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "尿液" }, { "start_idx": 23, "end_idx": 25, "type": "bod", "entity": "输尿管" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肾脏" }, { "start_idx": 40, "end_idx": 46, "type": "dis", "entity": "膀胱输尿管反流" } ] }, { "text": "膀胱输尿管反流分为原发性和继发性两种。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "膀胱输尿管反流" }, { "start_idx": 9, "end_idx": 11, "type": "dis", "entity": "原发性" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "继发性" } ] }, { "text": "前者系活瓣机能先天性发育不全,后者继发于下尿路梗阻,如后尿道瓣膜及神经源性膀胱等。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "dis", "entity": "活瓣机能先天性发育不全" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "下尿路梗阻" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "尿道瓣膜" }, { "start_idx": 33, "end_idx": 38, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "1958年Politano-Leadbetter首先报道采用抗反流的输尿管膀胱再吻合术来解决膀胱输尿管反流。", "entities": [ { "start_idx": 30, "end_idx": 42, "type": "pro", "entity": "抗反流的输尿管膀胱再吻合术" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "膀胱输尿管反流" } ] }, { "text": "胎儿肾积水中,由反流引起的也很常见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "胎儿肾积水" } ] }, { "text": "种族与原发性膀胱输尿管反流也有关,如白人女孩是黑人女孩的10倍,但一旦发生,其程度和自然缓解的可能性没有区别。", "entities": [ { "start_idx": 3, "end_idx": 12, "type": "dis", "entity": "原发性膀胱输尿管反流" } ] }, { "text": "原发性膀胱输尿管反流与遗传之间的关系也有报告。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "原发性膀胱输尿管反流" } ] }, { "text": "故现提倡凡直系亲属有反流病史的均应接受排尿性膀胱尿道造影(VCUG)筛查。", "entities": [ { "start_idx": 19, "end_idx": 27, "type": "pro", "entity": "排尿性膀胱尿道造影" }, { "start_idx": 29, "end_idx": 32, "type": "pro", "entity": "VCUG" } ] }, { "text": "高血压的发生与肾瘢痕有关,肾瘢痕越多,发生高血压的危险越高,患双侧严重肾瘢痕的小儿随访20年以上,20%有高血压,单侧病变者为8%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "高血压" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "高血压" }, { "start_idx": 35, "end_idx": 37, "type": "dis", "entity": "肾瘢痕" }, { "start_idx": 53, "end_idx": 55, "type": "dis", "entity": "高血压" } ] }, { "text": "因此凡超声发现的肾积水都应行VCUG,以排除反流。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dis", "entity": "肾积水" } ] }, { "text": "由于相当一部分患儿是无症状反流,在高危人群中用超声进行反流筛查有实际意义。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "pro", "entity": "超声" } ] }, { "text": "尿路感染在儿童中更多的表现是非特异性的,包括发热、嗜睡、无力、厌食、恶心、呕吐和生长障碍等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "尿路感染" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "发热" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "嗜睡" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "无力" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "厌食" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "恶心" }, { "start_idx": 37, "end_idx": 38, "type": "sym", "entity": "呕吐" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "生长障碍" } ] }, { "text": "采用膀胱镜于输尿管开口旁注射某些固体物质如Teflon或胶原蛋白等,阻止尿液反流,是当前欧美地区应用比较多的一种非手术治疗方法,尤其是注射用的固体物质的研究,开展得相当多。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "equ", "entity": "膀胱镜" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "输尿管" }, { "start_idx": 21, "end_idx": 26, "type": "dru", "entity": "Teflon" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "胶原蛋白" } ] }, { "text": "发生率为1/1500,临床上较常见,占所有先心病的6%~10%,以女性多见,男女比例约为2∶1。", "entities": [ { "start_idx": 21, "end_idx": 23, "type": "dis", "entity": "先心病" } ] }, { "text": "最近Benson等发现部分家族性房间隔缺损5p染色体可有基因突变。", "entities": [ { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "家族性房间隔缺损" }, { "start_idx": 21, "end_idx": 25, "type": "bod", "entity": "5p染色体" } ] }, { "text": "③冠状窦口型房间隔缺损:此型罕见。", "entities": [ { "start_idx": 1, "end_idx": 10, "type": "dis", "entity": "冠状窦口型房间隔缺损" } ] }, { "text": "伴有中等量左向右分流的患儿多无症状,即使有症状,也多为轻度的乏力和气促。", "entities": [ { "start_idx": 27, "end_idx": 31, "type": "sym", "entity": "轻度的乏力" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "气促" } ] }, { "text": "髓鞘化障碍是婴幼儿神经系统疾病,尤其是神经变性病和代谢病的主要表现之一。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "髓鞘化障碍" }, { "start_idx": 6, "end_idx": 14, "type": "dis", "entity": "婴幼儿神经系统疾病" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "神经变性病" }, { "start_idx": 25, "end_idx": 27, "type": "dis", "entity": "代谢病" } ] }, { "text": "研究已发现700多种基因突变与CF有关,但均定位于第7号染色体长臂单一位点。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "基因突变" }, { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "CF" }, { "start_idx": 25, "end_idx": 30, "type": "bod", "entity": "第7号染色体" } ] }, { "text": "新生儿期可因胎粪性肠梗阻、腹膜炎而引起注意并得到诊断。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "dis", "entity": "胎粪性肠梗阻" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "腹膜炎" } ] }, { "text": "持续咳嗽是最常见的症状,开始为干咳,以后伴黏稠痰或脓痰,不易咳出。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "持续咳嗽" }, { "start_idx": 12, "end_idx": 31, "type": "sym", "entity": "开始为干咳,以后伴黏稠痰或脓痰,不易咳出" } ] }, { "text": "婴儿可表现广泛喘鸣。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "喘鸣" } ] }, { "text": "随病情进展,出现气急、活动耐力差、生长发育障碍等。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "气急" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "活动耐力差" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "生长发育障碍" } ] }, { "text": "常见并发症包括肺不张、咯血、气胸、肺动脉高压、呼吸衰竭等。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "肺不张" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "咯血" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "气胸" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "肺动脉高压" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "呼吸衰竭" } ] }, { "text": "早期可出现肺气肿体征,散在或局部粗大啰音,伴杵状指(趾)。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "肺气肿" }, { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "散在或局部粗大啰音" }, { "start_idx": 24, "end_idx": 24, "type": "bod", "entity": "指" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "趾" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "杵状指(趾)" } ] }, { "text": "晚期可出现发绀。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "发绀" } ] }, { "text": "长期以来根据汗液试验进行确诊。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "汗液试验" } ] }, { "text": "CF患儿的治疗应着重于对呼吸道感染的防治。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "CF" }, { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "呼吸道感染" } ] }, { "text": "有黏液气道阻塞者可进行气管支气管吸引,或在纤维支气管镜下用生理盐水或黏液溶解剂进行灌洗。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "dis", "entity": "黏液气道阻塞" }, { "start_idx": 11, "end_idx": 17, "type": "pro", "entity": "气管支气管吸引" }, { "start_idx": 21, "end_idx": 26, "type": "equ", "entity": "纤维支气管镜" }, { "start_idx": 29, "end_idx": 32, "type": "dru", "entity": "生理盐水" }, { "start_idx": 34, "end_idx": 38, "type": "dru", "entity": "黏液溶解剂" }, { "start_idx": 41, "end_idx": 42, "type": "pro", "entity": "灌洗" } ] }, { "text": "机会感染(隐球菌病和曲菌病)和恶性肿瘤(Kaposi肉瘤)均可引起儿童和成人AIDS的心脏疾患。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "dis", "entity": "隐球菌病" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "曲菌病" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "Kaposi肉瘤" }, { "start_idx": 38, "end_idx": 41, "type": "dis", "entity": "AIDS" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "心脏" } ] }, { "text": "临床上可见充血性心力衰竭、心包填塞、非细菌性血栓性心内膜炎、传导紊乱和猝死等。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 13, "end_idx": 16, "type": "dis", "entity": "心包填塞" }, { "start_idx": 18, "end_idx": 28, "type": "dis", "entity": "非细菌性血栓性心内膜炎" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "传导紊乱" }, { "start_idx": 35, "end_idx": 36, "type": "sym", "entity": "猝死" } ] }, { "text": "亦可出现肾炎和肾病表现。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "肾炎" }, { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "肾病" } ] }, { "text": "软组织肉瘤的临床表现是肿块,但肿块本身没有功能,故只有肿块增大压迫周围组织时才产生症状。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "软组织肉瘤" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "肿块" }, { "start_idx": 15, "end_idx": 16, "type": "bod", "entity": "肿块" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肿块" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "组织" } ] }, { "text": "其确诊靠病理切片,但没有特征性的组织化学或免疫学、生物学标记。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "pro", "entity": "病理切片" } ] }, { "text": "软组织肉瘤的治疗主要是手术切除,恶性程度相差较大,放疗和化疗的效果对各具体肿瘤不尽相同。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "软组织肉瘤" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "手术切除" }, { "start_idx": 25, "end_idx": 26, "type": "pro", "entity": "放疗" }, { "start_idx": 28, "end_idx": 29, "type": "pro", "entity": "化疗" }, { "start_idx": 37, "end_idx": 38, "type": "dis", "entity": "肿瘤" } ] }, { "text": "先天性及婴儿纤维肉瘤细胞有丝分裂比较多见,可有淋巴细胞散在。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "纤维肉瘤细胞" }, { "start_idx": 23, "end_idx": 26, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "主要是足、踝和小腿,故上肢以手、腕和前臂较多,躯干、腹膜后和腮腺、口腔黏膜、扁桃体、乳突等也可生长此瘤。", "entities": [ { "start_idx": 3, "end_idx": 3, "type": "bod", "entity": "足" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "踝" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "小腿" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "上肢" }, { "start_idx": 14, "end_idx": 14, "type": "bod", "entity": "手" }, { "start_idx": 16, "end_idx": 16, "type": "bod", "entity": "腕" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "前臂" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "躯干" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "腹膜后" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "腮腺" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "口腔黏膜" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "扁桃体" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "乳突" } ] }, { "text": "治疗应广泛彻底切除肿瘤,至少包括瘤周围3cm的组织。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "pro", "entity": "切除肿瘤" }, { "start_idx": 23, "end_idx": 24, "type": "bod", "entity": "组织" } ] }, { "text": "另外,胃灼热是GERD的又一主要症状。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "sym", "entity": "胃灼热" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "GERD" } ] }, { "text": "当反流已引起食管黏膜损伤甚至溃疡时,患者会诉吞咽痛,体检可发现剑突下压痛。", "entities": [ { "start_idx": 0, "end_idx": 35, "type": "sym", "entity": "当反流已引起食管黏膜损伤甚至溃疡时,患者会诉吞咽痛,体检可发现剑突下压痛" } ] }, { "text": "如果长期反流,食管黏膜则会发生糜烂、溃疡、纤维组织增生及瘢痕形成等一系列改变,最后食管壁的顺应性下降,导致食管狭窄,患者逐渐出现吞咽困难。", "entities": [ { "start_idx": 7, "end_idx": 16, "type": "sym", "entity": "食管黏膜则会发生糜烂" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "溃疡" }, { "start_idx": 21, "end_idx": 37, "type": "sym", "entity": "纤维组织增生及瘢痕形成等一系列改变" }, { "start_idx": 41, "end_idx": 49, "type": "sym", "entity": "食管壁的顺应性下降" }, { "start_idx": 51, "end_idx": 56, "type": "sym", "entity": "导致食管狭窄" }, { "start_idx": 58, "end_idx": 67, "type": "sym", "entity": "患者逐渐出现吞咽困难" } ] }, { "text": "另一并发症是Barrett食管,下端食管的鳞状上皮被化生的柱状上皮所代替。", "entities": [ { "start_idx": 6, "end_idx": 14, "type": "dis", "entity": "Barrett食管" }, { "start_idx": 16, "end_idx": 35, "type": "sym", "entity": "下端食管的鳞状上皮被化生的柱状上皮所代替" } ] }, { "text": "另外,反复的呼吸道感染、呛咳、声音嘶哑、屏气,年长儿支气管哮喘发作等都与之有关。", "entities": [ { "start_idx": 3, "end_idx": 10, "type": "sym", "entity": "反复的呼吸道感染" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "呛咳" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "屏气" }, { "start_idx": 23, "end_idx": 32, "type": "sym", "entity": "年长儿支气管哮喘发作" } ] }, { "text": "在新生儿及婴幼儿中,GERD极易引起吸入性肺炎,有时甚至导致吸入性窒息、早产儿或婴儿猝死综合征的严重后果。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "GERD" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "吸入性肺炎" }, { "start_idx": 30, "end_idx": 34, "type": "sym", "entity": "吸入性窒息" }, { "start_idx": 36, "end_idx": 46, "type": "sym", "entity": "早产儿或婴儿猝死综合征" } ] }, { "text": "如少量多餐,避免高脂肪及巧克力等可能降低LES张力、延缓胃排空的食物;婴儿可进食黏稠食物,休息时保持头抬高30°的俯卧位等。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "少量多餐" }, { "start_idx": 6, "end_idx": 33, "type": "pro", "entity": "避免高脂肪及巧克力等可能降低LES张力、延缓胃排空的食物" }, { "start_idx": 35, "end_idx": 43, "type": "pro", "entity": "婴儿可进食黏稠食物" }, { "start_idx": 45, "end_idx": 60, "type": "pro", "entity": "休息时保持头抬高30°的俯卧位等" } ] }, { "text": "Nissan术应用至今已有40余年,仍被认为是最安全有效的方法,能迅速有效地解除GERD的症状。", "entities": [ { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "GERD" } ] }, { "text": "小儿颅内肿瘤发生部位与成人不同,40%~60%发生在幕下,并且以胚胎残余组织发生的肿瘤为主,胶质瘤相对较少。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "小儿颅内肿瘤" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "幕下" }, { "start_idx": 32, "end_idx": 37, "type": "bod", "entity": "胚胎残余组织" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "肿瘤" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "胶质瘤" } ] }, { "text": "而成人多发生在幕上,以胶质瘤为主。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "幕上" }, { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "胶质瘤" } ] }, { "text": "在化学因素中,多环芳香碳氢化合物和硝酸化合物,如甲基胆蒽、苯并比、甲基亚硝脲、亚硝基哌啶,在一些动物实验中都可诱发脑瘤。", "entities": [ { "start_idx": 57, "end_idx": 58, "type": "dis", "entity": "脑瘤" } ] }, { "text": "较有影响的是Kernohan提出将胶质瘤,包括星形细胞瘤、少突胶质细胞瘤、室管膜瘤和神经源肿瘤等,按其分化程度分为Ⅰ~Ⅳ级,根据这个分类似乎能够容易地判断肿瘤的发展和病人的预后,因此受到临床医师欢迎,在国内国际上使用多年。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "胶质瘤" }, { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "星形细胞瘤" }, { "start_idx": 29, "end_idx": 35, "type": "dis", "entity": "少突胶质细胞瘤" }, { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "室管膜瘤" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "神经源肿瘤" } ] }, { "text": "癫痫的发生主要由肿瘤的类型、生长速度及部位而定,生长缓慢的位置表浅的胶质瘤最易诱发癫痫,其发生率可达50%。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "癫痫" }, { "start_idx": 8, "end_idx": 9, "type": "dis", "entity": "肿瘤" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "胶质瘤" }, { "start_idx": 41, "end_idx": 42, "type": "dis", "entity": "癫痫" } ] }, { "text": "另外,10%~20%的病人,尤其是难治性的复杂性部分性癫痫,早期CT检查可呈阴性结果,因此必要时需重复影像学检查。", "entities": [ { "start_idx": 27, "end_idx": 28, "type": "dis", "entity": "癫痫" }, { "start_idx": 32, "end_idx": 35, "type": "pro", "entity": "CT检查" }, { "start_idx": 51, "end_idx": 55, "type": "pro", "entity": "影像学检查" } ] }, { "text": "在临床上,仅依靠病史和常规检查不足以排除导致小儿突然死亡的其他疾病(如先天性心脏病、先天性脑畸形、儿童虐待等),故在怀疑死者为婴儿猝死综合征时,通常需进行尸检进行鉴别。", "entities": [ { "start_idx": 35, "end_idx": 40, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "先天性脑畸形" }, { "start_idx": 63, "end_idx": 69, "type": "dis", "entity": "婴儿猝死综合征" } ] }, { "text": "在美国,除小儿先天畸形、早产、低出生体重外,SIDS目前是婴儿死亡最常见的原因。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "先天畸形" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "SIDS" } ] }, { "text": "各国SIDS的发病率相差甚远。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "SIDS" } ] }, { "text": "SIDS的发病与发病前2周所患的疾病、就诊次数增加、伴有消化道疾病以及精神不振有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "SIDS" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "消化道" } ] }, { "text": "患儿常有反复喂养困难和睡眠时多汗,但这些症状较难用已知的疾病来解释。", "entities": [ { "start_idx": 0, "end_idx": 15, "type": "sym", "entity": "患儿常有反复喂养困难和睡眠时多汗" } ] }, { "text": "内容包括对父母和看护者进行培训,使之熟悉SIDS的疾病过程和危险因素,并进行一些有益于预防的措施如给用安慰奶嘴、避免俯卧位等见表6-6,但母婴同床是否对预防SIDS有利仍有争论。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "dis", "entity": "SIDS" }, { "start_idx": 78, "end_idx": 81, "type": "dis", "entity": "SIDS" } ] }, { "text": "年长儿脑脓疡的发病率较高,引起此类疾病的原因是由于心房水平右向左分流,体循环中的细菌可直接到达脑部繁殖,产生脑脓疡。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脑脓疡" }, { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "心房" }, { "start_idx": 35, "end_idx": 37, "type": "bod", "entity": "体循环" }, { "start_idx": 40, "end_idx": 41, "type": "mic", "entity": "细菌" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "脑部" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "脑脓疡" } ] }, { "text": "若患有感染性心内膜炎,细菌栓子亦可进入脑部。", "entities": [ { "start_idx": 3, "end_idx": 9, "type": "dis", "entity": "感染性心内膜炎" }, { "start_idx": 11, "end_idx": 12, "type": "mic", "entity": "细菌" } ] }, { "text": "所以,对于任何大于2岁的青紫型先心患儿,当出现头痛、呕吐、神经定位症状时,尚需考虑脑部疾病的存在。", "entities": [ { "start_idx": 12, "end_idx": 16, "type": "dis", "entity": "青紫型先心" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "头痛" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "呕吐" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "神经定位" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "脑部" } ] }, { "text": "右心房造影,造影剂经房间交通入左心房,同时可逆流入下腔静脉、肝静脉。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "右心房造影" }, { "start_idx": 6, "end_idx": 8, "type": "equ", "entity": "造影剂" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "房间" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "左心房" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "下腔静脉" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "肝静脉" } ] }, { "text": "在行Fontan手术前,还应通过造影明确是否存在左上腔静脉及桥静脉。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "pro", "entity": "Fontan手术" }, { "start_idx": 16, "end_idx": 17, "type": "pro", "entity": "造影" }, { "start_idx": 24, "end_idx": 28, "type": "bod", "entity": "左上腔静脉" }, { "start_idx": 30, "end_idx": 32, "type": "bod", "entity": "桥静脉" } ] }, { "text": "在年长儿中,可作Fontan手术直接将体静脉血回流入肺循环,达到体肺循环分离。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "pro", "entity": "Fontan手术" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "体静脉" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "肺循环" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "体肺循环" } ] }, { "text": "如卵圆孔太小,心排出量降低,可行球囊房间隔造口术;对大婴儿及年长儿,可行球囊扩张加房间隔撕裂术。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "心排出量" }, { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "球囊房间隔造口术" }, { "start_idx": 36, "end_idx": 46, "type": "pro", "entity": "球囊扩张加房间隔撕裂术" } ] }, { "text": "静注NaHCO3纠正代谢性酸中毒。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "静注NaHCO3" } ] }, { "text": "此类型小儿及有进行性低氧血症或频繁缺氧发作的婴儿需行体-肺静脉分流术。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "进行性低氧血症" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "缺氧" } ] }, { "text": "小于6个月的婴儿,可行改良的Blalock-Taussig分流术,即以Goretex管放于锁骨下动脉与同侧肺动脉之间,两端均作端侧吻合。", "entities": [ { "start_idx": 14, "end_idx": 31, "type": "pro", "entity": "Blalock-Taussig分流术" }, { "start_idx": 35, "end_idx": 42, "type": "equ", "entity": "Goretex管" }, { "start_idx": 45, "end_idx": 49, "type": "bod", "entity": "锁骨下动脉" }, { "start_idx": 53, "end_idx": 55, "type": "bod", "entity": "肺动脉" } ] }, { "text": "Fontan术后4%~13%并发失蛋白性肠病,胃肠道蛋白质极度流失能导致低蛋白血症和水肿。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "Fontan术" }, { "start_idx": 16, "end_idx": 21, "type": "dis", "entity": "失蛋白性肠病" }, { "start_idx": 23, "end_idx": 28, "type": "bod", "entity": "胃肠道蛋白质" }, { "start_idx": 36, "end_idx": 40, "type": "dis", "entity": "低蛋白血症" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "水肿" } ] }, { "text": "临床上患儿一般情况良好,除有轻度贫血貌外,无其他阳性体征。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "轻度贫血貌" } ] }, { "text": "值得注意的是,因诊断和监护的需要而进行反复静脉穿刺采血,可以成为患儿贫血的主要原因,需进行输血治疗。", "entities": [ { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "静脉穿刺采血" }, { "start_idx": 34, "end_idx": 35, "type": "dis", "entity": "贫血" }, { "start_idx": 45, "end_idx": 46, "type": "pro", "entity": "输血" } ] }, { "text": "严重贫血时,可输红细胞悬液,每次10~15ml/kg,体重<1250g的未成熟儿必须尽量采用同一个供血者的血输注。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "严重贫血" }, { "start_idx": 8, "end_idx": 12, "type": "dru", "entity": "红细胞悬液" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "血" }, { "start_idx": 54, "end_idx": 55, "type": "pro", "entity": "输注" } ] }, { "text": "维生素A缺乏症是一种因体内维生素A缺乏引起的疾病,常伴随蛋白质-能量营养不良。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "维生素A缺乏症" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "维生素A" }, { "start_idx": 28, "end_idx": 37, "type": "sym", "entity": "蛋白质-能量营养不良" } ] }, { "text": "据WHO报道,因维生素A缺乏,全世界每年有50万名学龄前儿童患有活动性角膜溃疡。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "维生素A缺乏" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "活动性角膜溃疡" } ] }, { "text": "据报道,我国为儿童亚临床型维生素A缺乏的国家,城市学龄前儿童亚临床型维生素A缺乏发生率约20%,农村约45%。", "entities": [ { "start_idx": 9, "end_idx": 18, "type": "dis", "entity": "亚临床型维生素A缺乏" }, { "start_idx": 30, "end_idx": 39, "type": "dis", "entity": "亚临床型维生素A缺乏" } ] }, { "text": "母乳中的维生素A含量丰富,一般母乳喂养的小儿不会发生维生素A缺乏症。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dru", "entity": "维生素A" }, { "start_idx": 26, "end_idx": 32, "type": "dru", "entity": "维生素A缺乏症" } ] }, { "text": "如果维生素A持续缺乏,将发生角膜干燥症,伴有畏光,随后发生视物变形。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "维生素A" }, { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "角膜干燥症" }, { "start_idx": 22, "end_idx": 23, "type": "sym", "entity": "畏光" }, { "start_idx": 29, "end_idx": 32, "type": "sym", "entity": "视物变形" } ] }, { "text": "睑板腺肿大,并且沿着睑缘出现一串特征性的水泡,表面上皮的连续性遭到破坏,伴有非炎症性的溃疡形成和基质浸润,引起角膜软化、变性、溃疡甚至穿孔等损害,晶状体、虹膜脱出,造成整个眼睛的损害,通常为双侧性的,单侧发病少见。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "睑板腺" }, { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "睑板腺肿大" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "睑缘" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "水泡" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "溃疡" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "基质浸润" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "角膜" }, { "start_idx": 55, "end_idx": 58, "type": "sym", "entity": "角膜软化" }, { "start_idx": 60, "end_idx": 61, "type": "sym", "entity": "变性" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "溃疡" }, { "start_idx": 67, "end_idx": 68, "type": "sym", "entity": "穿孔" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "晶状体" }, { "start_idx": 77, "end_idx": 78, "type": "bod", "entity": "虹膜" }, { "start_idx": 86, "end_idx": 87, "type": "bod", "entity": "眼睛" } ] }, { "text": "维生素A缺乏也可引起皮肤的改变,开始时皮肤较正常干燥,以后由于毛囊上皮角化,发生角化过度的毛囊性丘疹,主要分布在大腿前外侧、上臂后侧,后逐渐扩展到上下肢伸侧、肩和下腹部,很少累及胸、背和臀。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "sym", "entity": "维生素A缺乏" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "皮肤" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "皮肤较正常干燥" }, { "start_idx": 31, "end_idx": 36, "type": "sym", "entity": "毛囊上皮角化" }, { "start_idx": 45, "end_idx": 49, "type": "dis", "entity": "毛囊性丘疹" }, { "start_idx": 40, "end_idx": 49, "type": "sym", "entity": "角化过度的毛囊性丘疹" }, { "start_idx": 56, "end_idx": 60, "type": "bod", "entity": "大腿前外侧" }, { "start_idx": 62, "end_idx": 65, "type": "bod", "entity": "上臂后侧" }, { "start_idx": 73, "end_idx": 75, "type": "bod", "entity": "上下肢" }, { "start_idx": 79, "end_idx": 79, "type": "bod", "entity": "肩" }, { "start_idx": 81, "end_idx": 83, "type": "bod", "entity": "下腹部" }, { "start_idx": 89, "end_idx": 89, "type": "bod", "entity": "胸" }, { "start_idx": 91, "end_idx": 91, "type": "bod", "entity": "背" }, { "start_idx": 93, "end_idx": 93, "type": "bod", "entity": "臀" } ] }, { "text": "维生素A缺乏可使小儿的免疫力低下,容易反复出现感染;容易有精神障碍,甚至出现脑积水。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "维生素A缺乏" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "免疫力低下" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "脑积水" } ] }, { "text": "用维生素A治疗维生素A缺乏症,疗效迅速而有效。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dru", "entity": "维生素A" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "维生素A缺乏症" } ] }, { "text": "早产儿应适当早期添加维生素A。", "entities": [ { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "维生素A" } ] }, { "text": "脑水肿的处理可用肾上腺皮质激素,如颅内压增高较明显可静脉推注脱水剂或利尿剂。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑水肿" }, { "start_idx": 8, "end_idx": 14, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "颅内压增高较明显" }, { "start_idx": 26, "end_idx": 29, "type": "pro", "entity": "静脉推注" }, { "start_idx": 30, "end_idx": 32, "type": "dru", "entity": "脱水剂" }, { "start_idx": 34, "end_idx": 36, "type": "dru", "entity": "利尿剂" } ] }, { "text": "蛛网膜下腔的血液和血凝块可引起脑动脉的炎症反应和脑水肿,可释放促血管痉挛物质而引起血管痉挛。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "血液" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "血凝块" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "脑动脉" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "炎症" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "脑水肿" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "血管" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "血管" }, { "start_idx": 29, "end_idx": 44, "type": "sym", "entity": "释放促血管痉挛物质而引起血管痉挛" } ] }, { "text": "动脉瘤和动静脉畸形(AVMs)采用外科或血管内闭塞治疗对于许多病人来说是非常有效的,但是放射外科学针对儿童AVMs病灶太小或很难用外科手术方法解决的病例,应用越来越多。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 4, "end_idx": 8, "type": "dis", "entity": "动静脉畸形" }, { "start_idx": 17, "end_idx": 18, "type": "dep", "entity": "外科" }, { "start_idx": 44, "end_idx": 47, "type": "dep", "entity": "放射外科" }, { "start_idx": 51, "end_idx": 56, "type": "dis", "entity": "儿童AVMs" } ] }, { "text": "数个较大的回顾性研究报道,放射外科学是非常安全而且对于治疗儿童AVMs是明显有效的。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "dep", "entity": "放射外科" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "儿童AVMs" } ] }, { "text": "对于血小板减少症患者应及时输注血小板或新鲜血,避免服用阿司匹林或其他抗血小板药物,或是避免可能导致头部外伤的刺激。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "血小板" }, { "start_idx": 27, "end_idx": 30, "type": "dru", "entity": "阿司匹林" }, { "start_idx": 34, "end_idx": 39, "type": "dru", "entity": "抗血小板药物" }, { "start_idx": 49, "end_idx": 50, "type": "bod", "entity": "头部" }, { "start_idx": 51, "end_idx": 52, "type": "dis", "entity": "外伤" } ] }, { "text": "对于血友病患者应输注Ⅷ因子,晚发性维生素K缺乏应输注维生素K和凝血因子复合物或新鲜血等。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "Ⅷ因子" }, { "start_idx": 26, "end_idx": 29, "type": "dru", "entity": "维生素K" }, { "start_idx": 31, "end_idx": 37, "type": "dru", "entity": "凝血因子复合物" }, { "start_idx": 39, "end_idx": 41, "type": "dru", "entity": "新鲜血" } ] }, { "text": "心电图同样有助于内脏位置的判断,P波电轴朝向左下,提示心房正位;P波电轴朝向右侧提示内脏反位。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "心电图" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "内脏" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "心房" } ] }, { "text": "超声心动图可对内脏位置及心内畸形进行准确的诊断,检查内容包括:腹部内脏位置、体静脉与心脏连接、房室连接、心室解剖、心室动脉连接及主动脉、肺动脉的解剖。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "超声心动图" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "内脏" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "心内畸形" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "心脏" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "房室" }, { "start_idx": 57, "end_idx": 60, "type": "bod", "entity": "心室动脉" } ] }, { "text": "最常见的合并畸形为房室连接不一致(心室左襻),约占50%。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "畸形" }, { "start_idx": 9, "end_idx": 21, "type": "sym", "entity": "房室连接不一致(心室左襻)" } ] }, { "text": "1/4的患儿伴有单心室,大多数患儿(60%)合并有室间隔缺损。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "sym", "entity": "单心室" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "室间隔缺损" } ] }, { "text": "伴发畸形主要有为房室连接不一致及大血管转位,但明显较右位心伴内脏正位少见。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "sym", "entity": "畸形" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "房室连接不一致" }, { "start_idx": 17, "end_idx": 18, "type": "bod", "entity": "血管" }, { "start_idx": 16, "end_idx": 20, "type": "sym", "entity": "大血管转位" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "右位心" }, { "start_idx": 30, "end_idx": 31, "type": "bod", "entity": "内脏" } ] }, { "text": "我国多数报道活产婴儿窒息发生率约为5%~10%。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "窒息" } ] }, { "text": "新生儿窒息多为产前或产时因素所致,产后因素较少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "新生儿窒息" } ] }, { "text": "血游离脂肪酸增加,促进钙离子与蛋白结合而致低钙血症。", "entities": [ { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "低钙血症" } ] }, { "text": "常见并发症有如下几种:①中枢神经系统:缺氧缺血性脑病和颅内出血;②呼吸系统:胎粪吸入综合征、呼吸窘迫综合征及肺出血;③心血管系统:缺氧缺血性心肌损害(三尖瓣闭锁不全、心力衰竭、心源性休克);④泌尿系统:肾功能不全或衰竭及肾静脉血栓形成等;⑤代谢方面:低血糖、低钙及低钠血症等;⑥消化系统:应激性溃疡和坏死性小肠结肠炎等。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 19, "end_idx": 25, "type": "dis", "entity": "缺氧缺血性脑病" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "颅内出血" }, { "start_idx": 33, "end_idx": 36, "type": "bod", "entity": "呼吸系统" }, { "start_idx": 38, "end_idx": 44, "type": "dis", "entity": "胎粪吸入综合征" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "呼吸窘迫综合征" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "肺出血" }, { "start_idx": 59, "end_idx": 63, "type": "bod", "entity": "心血管系统" }, { "start_idx": 65, "end_idx": 73, "type": "dis", "entity": "缺氧缺血性心肌损害" }, { "start_idx": 75, "end_idx": 81, "type": "dis", "entity": "三尖瓣闭锁不全" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "心力衰竭" }, { "start_idx": 88, "end_idx": 92, "type": "dis", "entity": "心源性休克" }, { "start_idx": 96, "end_idx": 99, "type": "bod", "entity": "泌尿系统" }, { "start_idx": 101, "end_idx": 105, "type": "dis", "entity": "肾功能不全" }, { "start_idx": 107, "end_idx": 108, "type": "dis", "entity": "衰竭" }, { "start_idx": 110, "end_idx": 114, "type": "dis", "entity": "肾静脉血栓" }, { "start_idx": 125, "end_idx": 127, "type": "dis", "entity": "低血糖" }, { "start_idx": 129, "end_idx": 130, "type": "sym", "entity": "低钙" }, { "start_idx": 132, "end_idx": 135, "type": "dis", "entity": "低钠血症" }, { "start_idx": 139, "end_idx": 142, "type": "bod", "entity": "消化系统" }, { "start_idx": 144, "end_idx": 148, "type": "dis", "entity": "应激性溃疡" }, { "start_idx": 150, "end_idx": 157, "type": "dis", "entity": "坏死性小肠结肠炎" } ] }, { "text": "原发肿瘤诊断明确并经治疗后转移至骨骼,一般较易发现。", "entities": [ { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "骨骼" } ] }, { "text": "但原发肿瘤部位和症状隐匿,以转移性骨肿瘤作为主要就诊主诉时,诊断上往往容易混淆,甚至将转移性的骨肿瘤当作骨原发的肿瘤进行诊断和治疗。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "bod", "entity": "转移性骨肿瘤" }, { "start_idx": 43, "end_idx": 49, "type": "bod", "entity": "转移性的骨肿瘤" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "肿瘤" } ] }, { "text": "肿瘤的骨骼转移中,静脉系统、特别是椎静脉系统起着主要的作用。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "骨骼" }, { "start_idx": 9, "end_idx": 12, "type": "bod", "entity": "静脉系统" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "椎静脉系统" } ] }, { "text": "儿童中最易产生肿瘤骨骼转移的是神经母细胞瘤、肺癌、甲状腺癌、乳腺癌。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "肿瘤骨骼" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "神经母细胞瘤" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "肺癌" }, { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "甲状腺癌" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "乳腺癌" } ] }, { "text": "转移性骨肿瘤好发于躯干骨,其次是股骨和肱骨的近端,发生在股骨和肱骨远端的较少。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "转移性骨肿瘤" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "躯干骨" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "股骨" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肱骨" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "股骨" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "肱骨" } ] }, { "text": "转移性骨肿瘤的部位也同原发肿瘤生长的部位有关,如乳腺癌的骨转移通常发生在胸椎和肱骨近端,甲状腺癌则常见于颈椎和颅骨,当然有时转移性肿瘤的发生也与原发肿瘤的部位无关。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "转移性骨肿瘤" }, { "start_idx": 24, "end_idx": 26, "type": "dis", "entity": "乳腺癌" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "胸椎" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "肱骨近端" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "甲状腺癌" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "颈椎" }, { "start_idx": 55, "end_idx": 56, "type": "bod", "entity": "颅骨" }, { "start_idx": 62, "end_idx": 66, "type": "dis", "entity": "转移性肿瘤" } ] }, { "text": "骨盆的转移性肿瘤常同时累及髂骨、耻骨和坐骨,脊椎的转移性肿瘤有时同时侵犯邻近的几个椎体和肋骨。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "骨盆" }, { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "转移性肿瘤" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "髂骨" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "耻骨" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "坐骨" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "脊椎" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "转移性肿瘤" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "椎体" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "肋骨" } ] }, { "text": "但有些以骨肿瘤为首发症状的转移性骨肿瘤在诊断上往往要依赖于各种实验室检查。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "骨肿瘤" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "转移性骨肿瘤" } ] }, { "text": "很多单发性的骨肿瘤病变,应该尽量地将活体组织检查手术操作同肿瘤组织的手术清除结合起来。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "骨肿瘤病变" }, { "start_idx": 18, "end_idx": 25, "type": "pro", "entity": "活体组织检查手术" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "肿瘤组织" }, { "start_idx": 34, "end_idx": 37, "type": "pro", "entity": "手术清除" } ] }, { "text": "有时,肿瘤部位很深或周围结构非常紧凑,如脊柱椎体的转移性肿瘤,也要充分考虑到活体组织检查操作本身的风险和操作后的并发症,采用其他的组织检查方法如穿刺活检也不失为一种较好的诊断方法。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "肿瘤" }, { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "脊柱椎体" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "转移性肿瘤" }, { "start_idx": 38, "end_idx": 43, "type": "pro", "entity": "活体组织检查" }, { "start_idx": 72, "end_idx": 75, "type": "pro", "entity": "穿刺活检" } ] }, { "text": "骨骼的病变可以采用手术清除、局部放疗和全身性化学治疗等方法。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "骨骼的病变" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "手术清除" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "局部放疗" }, { "start_idx": 19, "end_idx": 25, "type": "pro", "entity": "全身性化学治疗" } ] }, { "text": "出现骨骼并发症如病理性骨折的病例,要及时治疗出现的并发症。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "病理性骨折" } ] }, { "text": "主要侵犯主动脉弓,胸、腹主动脉及其分支,60%~70%累及一侧或双侧肾动脉。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "主动脉弓" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "胸" }, { "start_idx": 11, "end_idx": 18, "type": "bod", "entity": "腹主动脉及其分支" }, { "start_idx": 2, "end_idx": 18, "type": "sym", "entity": "侵犯主动脉弓,胸、腹主动脉及其分支" }, { "start_idx": 29, "end_idx": 36, "type": "bod", "entity": "一侧或双侧肾动脉" }, { "start_idx": 27, "end_idx": 36, "type": "sym", "entity": "累及一侧或双侧肾动脉" } ] }, { "text": "可因为头痛特别是枕部头痛、眩晕、急躁、过度兴奋、不安及疲乏而就医。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "头痛" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "枕部头痛" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "眩晕" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "急躁" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "过度兴奋" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "不安" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "疲乏" } ] }, { "text": "重症病人可有高血压脑病,有一过性视力障碍及抽搐等,有的可表现为行为异常或好动等。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "高血压脑病" }, { "start_idx": 13, "end_idx": 19, "type": "dis", "entity": "一过性视力障碍" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "抽搐" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "行为异常" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "好动" } ] }, { "text": "X线胸片可见左心室增大,也曾见RVH致全心衰竭者呈全心普遍增大,肺淤血表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "X线胸片" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "左心室增大" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "RVH" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "全心衰竭" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "全心普遍增大" }, { "start_idx": 32, "end_idx": 34, "type": "sym", "entity": "肺淤血" } ] }, { "text": "易误诊为哮喘,多有反复上呼吸道感染。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "哮喘" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "上呼吸道感染" } ] }, { "text": "在治疗以前需小心进软食并治疗肺部感染。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "肺部感染" } ] }, { "text": "对淋巴管肉瘤无有效治疗方案,肿瘤对放疗不敏感,成人病例多要截肢。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "淋巴管肉瘤" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "肿瘤" }, { "start_idx": 17, "end_idx": 18, "type": "pro", "entity": "放疗" }, { "start_idx": 29, "end_idx": 30, "type": "pro", "entity": "截肢" } ] }, { "text": "凡有慢性和先天性淋巴水肿的患者,应定期检查。", "entities": [ { "start_idx": 2, "end_idx": 11, "type": "dis", "entity": "慢性和先天性淋巴水肿" } ] }, { "text": "(3)班布特罗(Bambec)1mg/ml,100ml/瓶。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "班布特罗" }, { "start_idx": 8, "end_idx": 13, "type": "dru", "entity": "Bambec" } ] }, { "text": "(2)优喘平:每片400mg。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "优喘平" } ] }, { "text": "紧张、恐惧、激动、睡眠不足、气候变化、噪声、闪光刺激以及某些特殊食物的摄入(如奶酪和巧克力)等因素,均可诱发偏头痛发作。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "紧张" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "恐惧" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "激动" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "睡眠不足" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "噪声" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "闪光刺激" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "偏头痛" } ] }, { "text": "小儿偏头痛的临床特点是:①发作持续时间短,但发作次数较频;②双侧头痛多见,偏侧头痛相对少见;③视觉症状及头痛为搏动性较少见;④胃肠道症状突出,常伴有恶心、呕吐及腹痛;⑤有家族遗传史者多见;⑥伴夜尿、夜惊、夜游症及晕车晕船者多见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "小儿偏头痛" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "发作持续时间短" }, { "start_idx": 22, "end_idx": 27, "type": "sym", "entity": "发作次数较频" }, { "start_idx": 30, "end_idx": 35, "type": "sym", "entity": "双侧头痛多见" }, { "start_idx": 37, "end_idx": 44, "type": "sym", "entity": "偏侧头痛相对少见" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "视觉症状" }, { "start_idx": 52, "end_idx": 60, "type": "sym", "entity": "头痛为搏动性较少见" }, { "start_idx": 63, "end_idx": 69, "type": "sym", "entity": "胃肠道症状突出" }, { "start_idx": 74, "end_idx": 75, "type": "sym", "entity": "恶心" }, { "start_idx": 77, "end_idx": 78, "type": "sym", "entity": "呕吐" }, { "start_idx": 80, "end_idx": 81, "type": "sym", "entity": "腹痛" }, { "start_idx": 96, "end_idx": 97, "type": "sym", "entity": "夜尿" }, { "start_idx": 99, "end_idx": 100, "type": "sym", "entity": "夜惊" }, { "start_idx": 102, "end_idx": 104, "type": "sym", "entity": "夜游症" }, { "start_idx": 106, "end_idx": 110, "type": "sym", "entity": "晕车晕船者" } ] }, { "text": "头痛前没有明确的先兆,但常有一些非特异的症状,如嗜睡、疲劳、周身不适以及食欲减退等。", "entities": [ { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "嗜睡" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "疲劳" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "周身不适" }, { "start_idx": 36, "end_idx": 39, "type": "sym", "entity": "食欲减退" } ] }, { "text": "70%有恶心、呕吐或腹痛等胃肠道症状。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "恶心" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "呕吐" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "腹痛" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "胃肠道症状" } ] }, { "text": "可分两类:家族性多呈常染色体显性遗传;散发性可表现为经典型、普通型和偏瘫型偏头痛的交替发作。", "entities": [ { "start_idx": 5, "end_idx": 17, "type": "sym", "entity": "家族性多呈常染色体显性遗传" } ] }, { "text": "有明确的起源于双侧枕叶或脑干的先兆症状,视觉症状如闪光、暗点、视物模糊及黑蒙等,脑干症状如眩晕、复视、眼球震颤、耳鸣、构音障碍、共济失调、双侧肢体麻木及无力等,甚至可出现短暂的意识丧失。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "双侧枕叶" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "脑干" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "闪光" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "暗点" }, { "start_idx": 31, "end_idx": 34, "type": "sym", "entity": "视物模糊" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "黑蒙" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "眩晕" }, { "start_idx": 48, "end_idx": 49, "type": "sym", "entity": "复视" }, { "start_idx": 51, "end_idx": 54, "type": "sym", "entity": "眼球震颤" }, { "start_idx": 56, "end_idx": 57, "type": "sym", "entity": "耳鸣" }, { "start_idx": 59, "end_idx": 62, "type": "sym", "entity": "构音障碍" }, { "start_idx": 64, "end_idx": 67, "type": "sym", "entity": "共济失调" }, { "start_idx": 69, "end_idx": 77, "type": "sym", "entity": "双侧肢体麻木及无力" }, { "start_idx": 83, "end_idx": 91, "type": "sym", "entity": "出现短暂的意识丧失" } ] }, { "text": "主要包括良性阵发性眩晕、周期性呕吐(再发性呕吐)、腹型偏头痛、儿童交替性偏瘫以及阵发性斜颈等。", "entities": [ { "start_idx": 4, "end_idx": 10, "type": "sym", "entity": "良性阵发性眩晕" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "周期性呕吐" }, { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "再发性呕吐" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "腹型偏头痛" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "儿童交替性偏瘫" }, { "start_idx": 40, "end_idx": 44, "type": "sym", "entity": "阵发性斜颈" } ] }, { "text": "临床上除可出现贫血外,还可因缺铁而降低许多含铁酶的生物活性,进而影响细胞代谢功能,使机体出现消化道功能紊乱、循环功能障碍、免疫功能低下、精神神经症状以及皮肤黏膜病变等一系列非血液系统的表现。", "entities": [ { "start_idx": 7, "end_idx": 8, "type": "dis", "entity": "贫血" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "缺铁" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "含铁酶" }, { "start_idx": 17, "end_idx": 28, "type": "sym", "entity": "降低许多含铁酶的生物活性" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "细胞" }, { "start_idx": 46, "end_idx": 52, "type": "sym", "entity": "消化道功能紊乱" }, { "start_idx": 54, "end_idx": 59, "type": "sym", "entity": "循环功能障碍" }, { "start_idx": 61, "end_idx": 66, "type": "sym", "entity": "免疫功能低下" }, { "start_idx": 68, "end_idx": 73, "type": "sym", "entity": "精神神经症状" }, { "start_idx": 76, "end_idx": 81, "type": "sym", "entity": "皮肤黏膜病变" }, { "start_idx": 86, "end_idx": 90, "type": "bod", "entity": "非血液系统" } ] }, { "text": "缺铁与贫血之间是一个循序渐进的过程,当体内出现缺铁时,最早出现的表现是贮存铁的下降,如缺铁持续存在,即可出现铁蛋白下降,此时仍可无血红蛋白下降,临床上也无贫血的表现。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "缺铁" }, { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "贫血" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "缺铁" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "缺铁" }, { "start_idx": 54, "end_idx": 56, "type": "bod", "entity": "铁蛋白" }, { "start_idx": 65, "end_idx": 68, "type": "bod", "entity": "血红蛋白" }, { "start_idx": 77, "end_idx": 78, "type": "dis", "entity": "贫血" } ] }, { "text": "年长儿可诉头晕、眼前发黑、耳鸣等。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "头晕" }, { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "眼前发黑" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "耳鸣" } ] }, { "text": "肝、脾、淋巴结可轻度增大,主要原因为髓外造血。", "entities": [ { "start_idx": 0, "end_idx": 11, "type": "sym", "entity": "肝、脾、淋巴结可轻度增大" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "髓外造血" } ] }, { "text": "有时有腹泻或呕吐,皮肤微肿,脉搏加速,心前区可有吹风样收缩期杂音。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "腹泻" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "呕吐" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "皮肤微肿" }, { "start_idx": 14, "end_idx": 17, "type": "sym", "entity": "脉搏加速" }, { "start_idx": 19, "end_idx": 31, "type": "sym", "entity": "心前区可有吹风样收缩期杂音" } ] }, { "text": "单纯IDA时,白细胞通常在正常范围或稍低。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "bod", "entity": "白细胞" } ] }, { "text": "伴有钩虫病患儿可有嗜酸性粒细胞增多。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "钩虫病" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "嗜酸性粒细胞" } ] }, { "text": "典型IDA的诊断并不困难,但轻型病例和无贫血缺铁状态的诊断,须进行血清铁、铁结合力、骨髓细胞外铁染色检查及红细胞内游离原卟啉的测定方能作出明确诊断。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "IDA" }, { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "贫血" }, { "start_idx": 33, "end_idx": 51, "type": "pro", "entity": "血清铁、铁结合力、骨髓细胞外铁染色检查" }, { "start_idx": 53, "end_idx": 64, "type": "pro", "entity": "红细胞内游离原卟啉的测定" } ] }, { "text": "IDA主要与小细胞低色素性贫血鉴别,包括铅中毒、β-地中海贫血、α-地中海贫血、慢性感染引起的贫血等。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "IDA" }, { "start_idx": 6, "end_idx": 14, "type": "dis", "entity": "小细胞低色素性贫血" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "铅中毒" }, { "start_idx": 24, "end_idx": 30, "type": "dis", "entity": "β-地中海贫血" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "α-地中海贫血" }, { "start_idx": 40, "end_idx": 43, "type": "sym", "entity": "慢性感染" }, { "start_idx": 47, "end_idx": 48, "type": "dis", "entity": "贫血" } ] }, { "text": "对易感儿,应给予预防量铁剂预防。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "dru", "entity": "铁剂" } ] }, { "text": "在钩虫病流行地区,要大力开展消灭寄生虫病的卫生防疫工作,防止病儿重复感染,同时需给予口服铁剂,以预防或治疗贫血。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "dis", "entity": "钩虫病" }, { "start_idx": 16, "end_idx": 19, "type": "dis", "entity": "寄生虫病" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "感染" }, { "start_idx": 42, "end_idx": 43, "type": "pro", "entity": "口服" }, { "start_idx": 44, "end_idx": 45, "type": "dru", "entity": "铁剂" }, { "start_idx": 53, "end_idx": 54, "type": "dis", "entity": "贫血" } ] }, { "text": "第一种是以神经的病变部位为分类基础,其代表为Bors-Coman分类法,根据脊髓损伤的部位将神经源性膀胱分为上运动神经元型(骶上型)、下运动神经元型(骶下型)以及混合型;第二种以膀胱功能变化为基础,如Wein分类法,根据膀胱排空机能将神经源性膀胱分为贮尿障碍和排空障碍,该方法的优点是较适用于临床应用。", "entities": [ { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "脊髓" }, { "start_idx": 54, "end_idx": 60, "type": "dis", "entity": "上运动神经元型" }, { "start_idx": 62, "end_idx": 64, "type": "dis", "entity": "骶上型" }, { "start_idx": 67, "end_idx": 73, "type": "dis", "entity": "下运动神经元型" }, { "start_idx": 75, "end_idx": 77, "type": "dis", "entity": "骶下型" }, { "start_idx": 81, "end_idx": 83, "type": "dis", "entity": "混合型" }, { "start_idx": 89, "end_idx": 90, "type": "bod", "entity": "膀胱" }, { "start_idx": 110, "end_idx": 111, "type": "bod", "entity": "膀胱" }, { "start_idx": 117, "end_idx": 122, "type": "dis", "entity": "神经源性膀胱" }, { "start_idx": 125, "end_idx": 128, "type": "dis", "entity": "贮尿障碍" }, { "start_idx": 130, "end_idx": 133, "type": "dis", "entity": "排空障碍" } ] }, { "text": "因此,进行尿常规和培养、尿素氮和肌酐以及血钠、钾、氯和二氧化碳结合力等检查对了解神经源性膀胱患儿的上尿路损害程度与全身状况十分重要。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "ite", "entity": "尿常规" }, { "start_idx": 12, "end_idx": 14, "type": "ite", "entity": "尿素氮" }, { "start_idx": 16, "end_idx": 17, "type": "ite", "entity": "肌酐" }, { "start_idx": 20, "end_idx": 36, "type": "ite", "entity": "血钠、钾、氯和二氧化碳结合力等检查" }, { "start_idx": 40, "end_idx": 45, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "放射性核素显像可精确评价患儿分肾功能受损程度。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "放射性核素显像" } ] }, { "text": "因此,将尿流动力学结果与病史、体格检查以及影像学发现进行综合评价,才能对神经源性膀胱患儿作出个体化的全面诊断,从而达到为治疗提供合理指导的目的。", "entities": [ { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "根据Wein分类法决定的神经源性膀胱的基本治疗原则对临床治疗有较好的指导意义。", "entities": [ { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "神经源性膀胱" } ] }, { "text": "非手术治疗包括:①导尿术;②药物治疗;③神经阻滞疗法;④其他方法如生物反馈技术及排尿训练等。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "pro", "entity": "导尿术" }, { "start_idx": 14, "end_idx": 17, "type": "pro", "entity": "药物治疗" }, { "start_idx": 20, "end_idx": 25, "type": "pro", "entity": "神经阻滞疗法" }, { "start_idx": 33, "end_idx": 38, "type": "pro", "entity": "生物反馈技术" }, { "start_idx": 40, "end_idx": 43, "type": "pro", "entity": "排尿训练" } ] }, { "text": "手术治疗包括:①降低膀胱出口阻力的手术,如神经根切断术、经尿道外括约肌/膀胱颈部切开术等;②增加膀胱出口阻力的手术,如膀胱颈悬吊术、特氟隆(Teflon)等生物材料注射手术以及人工括约肌装置等;③增加膀胱容量的手术,如自体或胃(肠)膀胱扩大成形术;④增加膀胱逼尿肌收缩能力的手术,如电刺激治疗以及单层肠浆肌层膀胱加强术等;⑤其他如尿流改道以及并发症的治疗。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "手术治疗" }, { "start_idx": 8, "end_idx": 18, "type": "pro", "entity": "降低膀胱出口阻力的手术" }, { "start_idx": 21, "end_idx": 26, "type": "pro", "entity": "神经根切断术" }, { "start_idx": 28, "end_idx": 42, "type": "pro", "entity": "经尿道外括约肌/膀胱颈部切开术" }, { "start_idx": 46, "end_idx": 56, "type": "pro", "entity": "增加膀胱出口阻力的手术" }, { "start_idx": 59, "end_idx": 64, "type": "pro", "entity": "膀胱颈悬吊术" }, { "start_idx": 66, "end_idx": 68, "type": "pro", "entity": "特氟隆" }, { "start_idx": 70, "end_idx": 75, "type": "pro", "entity": "Teflon" }, { "start_idx": 78, "end_idx": 85, "type": "pro", "entity": "生物材料注射手术" }, { "start_idx": 88, "end_idx": 94, "type": "pro", "entity": "人工括约肌装置" }, { "start_idx": 98, "end_idx": 106, "type": "pro", "entity": "增加膀胱容量的手术" }, { "start_idx": 109, "end_idx": 122, "type": "pro", "entity": "自体或胃(肠)膀胱扩大成形术" }, { "start_idx": 125, "end_idx": 138, "type": "pro", "entity": "增加膀胱逼尿肌收缩能力的手术" }, { "start_idx": 141, "end_idx": 158, "type": "pro", "entity": "电刺激治疗以及单层肠浆肌层膀胱加强术" }, { "start_idx": 165, "end_idx": 168, "type": "pro", "entity": "尿流改道" } ] }, { "text": "可伴有低热、食欲缺乏及乏力等全身症状。", "entities": [ { "start_idx": 3, "end_idx": 17, "type": "sym", "entity": "低热、食欲缺乏及乏力等全身症状" } ] }, { "text": "可用泼尼松1~2mg/(kg•d),分次口服,或用地塞米松、甲基泼尼松龙静脉滴注,症状缓解后即可停用。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dru", "entity": "泼尼松" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "地塞米松" }, { "start_idx": 30, "end_idx": 35, "type": "dru", "entity": "甲基泼尼松龙" }, { "start_idx": 36, "end_idx": 39, "type": "pro", "entity": "静脉滴注" } ] }, { "text": "重症可用免疫抑制剂如环磷酰胺或雷公藤多甙片。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 15, "end_idx": 20, "type": "dru", "entity": "雷公藤多甙片" } ] }, { "text": "实体瘤原发于体表部位时,主要的就诊原因是可扪及的无痛性肿块。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "实体瘤" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "体表" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "肿块" }, { "start_idx": 20, "end_idx": 28, "type": "sym", "entity": "可扪及的无痛性肿块" } ] }, { "text": "肿瘤原发于纵隔、腹腔、盆腔时,早期常无症状,体检也不易发现肿块,当肿瘤生长至压迫邻近组织、器官使其功能障碍时,出现压迫症状(表11-11)。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "dis", "entity": "肿瘤" }, { "start_idx": 5, "end_idx": 6, "type": "bod", "entity": "纵隔" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "腹腔" }, { "start_idx": 11, "end_idx": 12, "type": "bod", "entity": "盆腔" } ] }, { "text": "全身播散时出现全身症状如苍白、消瘦、发热、骨关节疼痛、出血,肝、脾、淋巴结肿大等。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "苍白" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "消瘦" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "发热" }, { "start_idx": 21, "end_idx": 23, "type": "bod", "entity": "骨关节" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "骨关节疼痛" }, { "start_idx": 27, "end_idx": 28, "type": "sym", "entity": "出血" }, { "start_idx": 30, "end_idx": 30, "type": "bod", "entity": "肝" }, { "start_idx": 32, "end_idx": 32, "type": "bod", "entity": "脾" }, { "start_idx": 34, "end_idx": 36, "type": "bod", "entity": "淋巴结" }, { "start_idx": 30, "end_idx": 38, "type": "sym", "entity": "肝、脾、淋巴结肿大" } ] }, { "text": "根据其发病有无前驱症状(腹泻),分为典型HUS和非典型HUS。", "entities": [ { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "腹泻" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "典型HUS" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "非典型HUS" } ] }, { "text": "细菌感染(如大肠埃希菌、志贺痢疾杆菌、肺炎链球菌和沙门菌)及病毒感染[包括柯萨奇病毒、埃可病毒、流感病毒、人类免疫缺陷病毒(HIV)]均可诱发HUS。", "entities": [ { "start_idx": 6, "end_idx": 10, "type": "mic", "entity": "大肠埃希菌" }, { "start_idx": 12, "end_idx": 17, "type": "mic", "entity": "志贺痢疾杆菌" }, { "start_idx": 19, "end_idx": 23, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 25, "end_idx": 27, "type": "mic", "entity": "沙门菌" }, { "start_idx": 37, "end_idx": 41, "type": "mic", "entity": "柯萨奇病毒" }, { "start_idx": 43, "end_idx": 46, "type": "mic", "entity": "埃可病毒" }, { "start_idx": 48, "end_idx": 51, "type": "mic", "entity": "流感病毒" }, { "start_idx": 53, "end_idx": 60, "type": "mic", "entity": "人类免疫缺陷病毒" }, { "start_idx": 62, "end_idx": 64, "type": "mic", "entity": "HIV" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "HUS" } ] }, { "text": "有资料表明,出血性大肠埃希菌(EHEC)O157:H7是引起一些地区流行性感染性腹泻相关的HUS的主要病原,O157:H7主要存在于家畜肠道、未煮熟透的肉类和未经消毒的牛奶。", "entities": [ { "start_idx": 6, "end_idx": 26, "type": "mic", "entity": "出血性大肠埃希菌(EHEC)O157:H7" }, { "start_idx": 40, "end_idx": 41, "type": "sym", "entity": "腹泻" }, { "start_idx": 45, "end_idx": 47, "type": "dis", "entity": "HUS" }, { "start_idx": 54, "end_idx": 60, "type": "mic", "entity": "O157:H7" } ] }, { "text": "腹痛严重者伴腹肌紧张,酷似急腹症;腹泻可为水样便,多见血便和黏液便。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "腹痛严重者伴腹肌紧张" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "急腹症" }, { "start_idx": 17, "end_idx": 32, "type": "sym", "entity": "腹泻可为水样便,多见血便和黏液便" } ] }, { "text": "前驱期后经数天无症状期进入急性期,出现溶血性贫血、急性肾衰竭和血小板减少。", "entities": [ { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "溶血性贫血" }, { "start_idx": 25, "end_idx": 35, "type": "sym", "entity": "急性肾衰竭和血小板减少" } ] }, { "text": "初期可屡有溶血危象发生,于数小时内血色素下降30~50g/L;急性肾功能减退临床表现轻重不一,轻者仅短暂尿量减少,肾功能轻度减退,但多数患儿呈少尿性急性肾衰竭,少尿可持续达2周甚至2周以上,同时有氮质血症、代谢性酸中毒、高血钾等其他急性肾衰竭的表现,并可由于贫血、高血容量和电解质紊乱等引发充血性心力衰竭;血小板减少致出血倾向,以消化道出血为主,可见皮肤瘀斑,偶见硬脑膜下或视网膜出血。", "entities": [ { "start_idx": 0, "end_idx": 29, "type": "sym", "entity": "初期可屡有溶血危象发生,于数小时内血色素下降30~50g/L" }, { "start_idx": 47, "end_idx": 55, "type": "sym", "entity": "轻者仅短暂尿量减少" }, { "start_idx": 57, "end_idx": 63, "type": "sym", "entity": "肾功能轻度减退" }, { "start_idx": 66, "end_idx": 78, "type": "sym", "entity": "多数患儿呈少尿性急性肾衰竭" }, { "start_idx": 80, "end_idx": 93, "type": "sym", "entity": "少尿可持续达2周甚至2周以上" }, { "start_idx": 95, "end_idx": 123, "type": "sym", "entity": "同时有氮质血症、代谢性酸中毒、高血钾等其他急性肾衰竭的表现" }, { "start_idx": 129, "end_idx": 130, "type": "sym", "entity": "贫血" }, { "start_idx": 132, "end_idx": 135, "type": "sym", "entity": "高血容量" }, { "start_idx": 137, "end_idx": 141, "type": "sym", "entity": "电解质紊乱" }, { "start_idx": 145, "end_idx": 151, "type": "dis", "entity": "充血性心力衰竭" }, { "start_idx": 153, "end_idx": 155, "type": "bod", "entity": "血小板" }, { "start_idx": 153, "end_idx": 157, "type": "sym", "entity": "血小板减少" }, { "start_idx": 165, "end_idx": 167, "type": "bod", "entity": "消化道" }, { "start_idx": 165, "end_idx": 169, "type": "sym", "entity": "消化道出血" }, { "start_idx": 175, "end_idx": 176, "type": "bod", "entity": "皮肤" }, { "start_idx": 182, "end_idx": 184, "type": "bod", "entity": "硬脑膜" }, { "start_idx": 187, "end_idx": 189, "type": "bod", "entity": "视网膜" }, { "start_idx": 173, "end_idx": 191, "type": "sym", "entity": "可见皮肤瘀斑,偶见硬脑膜下或视网膜出血" } ] }, { "text": "由于HUS存在广泛的微血管血栓形成,可导致多系统损害,除胃肠道和肾脏外,尤以中枢神经系统受累多见,是最常见的死因。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "HUS" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "微血管" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "微血管血栓" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "胃肠道" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "肾脏" }, { "start_idx": 38, "end_idx": 43, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "神经系统症状表现有激惹、嗜睡、焦虑、紧张、幻觉、定向障碍、惊厥和昏迷,部分留有神经系统后遗症,如学习困难、行为异常,严重者可见智力低下或癫痫。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "神经系统" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "激惹" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "嗜睡" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "焦虑" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "紧张" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "幻觉" }, { "start_idx": 24, "end_idx": 27, "type": "sym", "entity": "定向障碍" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "惊厥" }, { "start_idx": 32, "end_idx": 33, "type": "sym", "entity": "昏迷" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "神经系统" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "学习困难" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "行为异常" }, { "start_idx": 58, "end_idx": 69, "type": "sym", "entity": "严重者可见智力低下或癫痫" } ] }, { "text": "对疑有免疫因素参与发病机制者,可静脉输注丙种球蛋白。", "entities": [ { "start_idx": 16, "end_idx": 19, "type": "pro", "entity": "静脉输注" }, { "start_idx": 20, "end_idx": 24, "type": "dru", "entity": "丙种球蛋白" } ] }, { "text": "具体为:①急性期后临床表现为肾小球肾炎或蛋白尿、血尿者用雷公藤多苷治疗;②急性期后表现为肾病综合征者用泼尼松治疗;③有条件者行肾活检检查,根据病理改变调整治疗方案,如有新月体形成或局灶节段性肾小球硬化者加用甲基泼尼松龙和(或)环磷酰胺冲击治疗;④对治疗无反应、仍呈肾功能进行性减退者停用激素和免疫抑制剂,以对症和肾替代疗法为主。", "entities": [ { "start_idx": 14, "end_idx": 18, "type": "dis", "entity": "肾小球肾炎" }, { "start_idx": 20, "end_idx": 22, "type": "sym", "entity": "蛋白尿" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "血尿" }, { "start_idx": 28, "end_idx": 32, "type": "dru", "entity": "雷公藤多苷" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "肾病综合征" }, { "start_idx": 51, "end_idx": 53, "type": "dru", "entity": "泼尼松" }, { "start_idx": 63, "end_idx": 65, "type": "pro", "entity": "肾活检" }, { "start_idx": 84, "end_idx": 88, "type": "sym", "entity": "新月体形成" }, { "start_idx": 90, "end_idx": 99, "type": "sym", "entity": "局灶节段性肾小球硬化" }, { "start_idx": 103, "end_idx": 108, "type": "dru", "entity": "甲基泼尼松龙" }, { "start_idx": 113, "end_idx": 116, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 132, "end_idx": 139, "type": "sym", "entity": "肾功能进行性减退" }, { "start_idx": 143, "end_idx": 144, "type": "dru", "entity": "激素" }, { "start_idx": 146, "end_idx": 150, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 156, "end_idx": 160, "type": "pro", "entity": "肾替代疗法" } ] }, { "text": "年龄小、有胃肠道前驱症状者,病死率低,肾功能恢复好,终末肾发生率低;而年龄>3岁、无胃肠道前驱症状、无尿期>3天、有神经系统症状者、家族性发病者预后差。", "entities": [ { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "胃肠道" }, { "start_idx": 19, "end_idx": 19, "type": "bod", "entity": "肾" }, { "start_idx": 28, "end_idx": 28, "type": "bod", "entity": "肾" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "胃肠道" }, { "start_idx": 58, "end_idx": 61, "type": "bod", "entity": "神经系统" } ] }, { "text": "约有15%病例发展成慢性肾衰竭、持续高血压或神经系统后遗症。", "entities": [ { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "慢性肾衰竭" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "持续高血压" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "神经系统后遗症" } ] }, { "text": "从而引起巨幼红细胞性贫血。", "entities": [ { "start_idx": 4, "end_idx": 11, "type": "dis", "entity": "巨幼红细胞性贫血" } ] }, { "text": "肤色可苍黄,口唇、睑结膜、甲床苍白,头发黄、细、干、稀疏,面水肿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "肤色可苍黄" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "口唇" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "睑" }, { "start_idx": 6, "end_idx": 11, "type": "sym", "entity": "口唇、睑结膜" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "甲床" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "甲床苍白" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "头发" }, { "start_idx": 18, "end_idx": 27, "type": "sym", "entity": "头发黄、细、干、稀疏" }, { "start_idx": 29, "end_idx": 29, "type": "bod", "entity": "面" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "面水肿" } ] }, { "text": "患儿可出现烦躁不安、疲乏无力、表情呆滞、反应迟钝、两眼直视、食欲差、嗜睡等症。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "烦躁不安" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "疲乏无力" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "表情呆滞" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "反应迟钝" }, { "start_idx": 25, "end_idx": 28, "type": "sym", "entity": "两眼直视" }, { "start_idx": 30, "end_idx": 32, "type": "sym", "entity": "食欲差" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "嗜睡" } ] }, { "text": "心前区可闻及吹风样收缩期杂音。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "心前区" }, { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "心前区可闻及吹风样收缩期杂音" } ] }, { "text": "血小板严重降低时,皮肤可出现瘀点、瘀斑。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 9, "end_idx": 10, "type": "bod", "entity": "皮肤" }, { "start_idx": 9, "end_idx": 18, "type": "sym", "entity": "皮肤可出现瘀点、瘀斑" } ] }, { "text": "可出现动作缓慢、手足无意识运动、头部及肢体颤动。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "sym", "entity": "动作缓慢" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "手足" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "手足无意识运动" }, { "start_idx": 16, "end_idx": 17, "type": "bod", "entity": "头部" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肢体" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "头部及肢体颤动" } ] }, { "text": "震颤初见于手、唇、舌,因反复震颤而舌系带可出现溃疡;继而上肢、头部、甚至全身。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "震颤" }, { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "手" }, { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "唇" }, { "start_idx": 9, "end_idx": 9, "type": "bod", "entity": "舌" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "反复震颤" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "舌系带" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "溃疡" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "上肢" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "头部" }, { "start_idx": 36, "end_idx": 37, "type": "bod", "entity": "全身" } ] }, { "text": "重症病例可见四肢屈曲,踝阵挛。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "四肢" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "四肢屈曲" }, { "start_idx": 11, "end_idx": 11, "type": "bod", "entity": "踝" }, { "start_idx": 11, "end_idx": 13, "type": "sym", "entity": "踝阵挛" } ] }, { "text": "长期缺乏未予补充者,可出现智力障碍。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "智力障碍" } ] }, { "text": "如喂养不当应予以纠正,慢性腹泻应予以治疗。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "dis", "entity": "慢性腹泻" } ] }, { "text": "治疗期间要适当加服铁剂以供造红细胞所需。", "entities": [ { "start_idx": 9, "end_idx": 10, "type": "dru", "entity": "铁剂" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "红细胞" } ] }, { "text": "严重巨幼红细胞贫血病儿在治疗开始48小时,血钾可突然下降,加之心肌因慢性缺氧,可发生突然死亡,严重巨幼红细胞性贫血患儿,治疗时应同时补充钾盐。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "巨幼红细胞贫血" }, { "start_idx": 21, "end_idx": 22, "type": "ite", "entity": "血钾" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "心肌" }, { "start_idx": 34, "end_idx": 37, "type": "sym", "entity": "慢性缺氧" }, { "start_idx": 49, "end_idx": 56, "type": "dis", "entity": "巨幼红细胞性贫血" }, { "start_idx": 68, "end_idx": 69, "type": "dru", "entity": "钾盐" } ] }, { "text": "VLBW的肺动脉缺乏平滑肌,致使肺动脉舒缩困难,容易引发持续性肺动脉高压。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "肺动脉" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "平滑肌" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "肺动脉" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "肺动脉舒缩困难" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "肺动脉" }, { "start_idx": 28, "end_idx": 35, "type": "sym", "entity": "持续性肺动脉高压" } ] }, { "text": "出生早期动脉导管处于可开放性状态,容易因缺氧等病理原因造成动脉导管重新开放,甚至在短期内引起充血性心力衰竭。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "动脉导管" }, { "start_idx": 29, "end_idx": 36, "type": "sym", "entity": "动脉导管重新开放" }, { "start_idx": 46, "end_idx": 52, "type": "dis", "entity": "充血性心力衰竭" } ] }, { "text": "脑室室管膜下生发层在侧脑室周围长得很厚,而此处非常容易引起脑室内室管膜下出血,是脑室内出血好发部位。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "脑室室管膜" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "侧脑室" }, { "start_idx": 0, "end_idx": 18, "type": "sym", "entity": "脑室室管膜下生发层在侧脑室周围长得很厚" }, { "start_idx": 29, "end_idx": 37, "type": "dis", "entity": "脑室内室管膜下出血" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "脑室内出血" } ] }, { "text": "VLBW可以在经肠道喂养前就比较容易发生坏死性小肠结肠炎(NEC)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肠道" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "坏死性小肠结肠炎" }, { "start_idx": 29, "end_idx": 31, "type": "dis", "entity": "NEC" } ] }, { "text": "VLBW在生后1~2天内处于一种非少尿性高钾血症状态,并在生后24小时达到高峰,高钾血症的原因可能与肾小球滤过率低、肾小管重吸收能力差以及红细胞Na+-K+-ATP酶的活性较高有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "VLBW" }, { "start_idx": 16, "end_idx": 23, "type": "dis", "entity": "非少尿性高钾血症" }, { "start_idx": 40, "end_idx": 43, "type": "dis", "entity": "高钾血症" }, { "start_idx": 50, "end_idx": 52, "type": "bod", "entity": "肾小球" }, { "start_idx": 50, "end_idx": 56, "type": "sym", "entity": "肾小球滤过率低" }, { "start_idx": 58, "end_idx": 60, "type": "bod", "entity": "肾小管" }, { "start_idx": 58, "end_idx": 66, "type": "sym", "entity": "肾小管重吸收能力差" }, { "start_idx": 69, "end_idx": 82, "type": "bod", "entity": "红细胞Na+-K+-ATP酶" } ] }, { "text": "糖耐受量低,容易引起高血糖和糖尿,高血糖可以造成高渗性利尿,从而有引起颅内出血的危险,并可引起呼吸暂停和大脑抑制。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "sym", "entity": "糖耐受量低" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "高血糖" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "糖尿" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "高血糖" }, { "start_idx": 24, "end_idx": 28, "type": "dis", "entity": "高渗性利尿" }, { "start_idx": 35, "end_idx": 38, "type": "dis", "entity": "颅内出血" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 52, "end_idx": 53, "type": "bod", "entity": "大脑" }, { "start_idx": 52, "end_idx": 55, "type": "sym", "entity": "大脑抑制" } ] }, { "text": "半数以上患儿伴有杓状软骨软化,亦可由于会厌软骨软化或杓状会厌襞过短所致。", "entities": [ { "start_idx": 8, "end_idx": 13, "type": "dis", "entity": "杓状软骨软化" }, { "start_idx": 19, "end_idx": 24, "type": "dis", "entity": "会厌软骨软化" }, { "start_idx": 26, "end_idx": 32, "type": "dis", "entity": "杓状会厌襞过短" } ] }, { "text": "多数患儿于新生儿期出现喘鸣症状,有的则于出生后1~2个月,因感冒或腹泻后逐渐出现。", "entities": [ { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "喘鸣" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "感冒" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "腹泻" } ] }, { "text": "以吸气性喉鸣为主。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "dis", "entity": "吸气性喉鸣" } ] }, { "text": "直接喉镜检查可确诊,并明确畸形的位置及性质。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "pro", "entity": "喉镜检查" }, { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "畸形" } ] }, { "text": "本病应注意与喉蹼、婴儿低钙性喉痉挛、喉部肿瘤及继发性喉软骨软化等鉴别。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "喉蹼" }, { "start_idx": 9, "end_idx": 16, "type": "dis", "entity": "婴儿低钙性喉痉挛" }, { "start_idx": 18, "end_idx": 21, "type": "dis", "entity": "喉部肿瘤" }, { "start_idx": 23, "end_idx": 30, "type": "dis", "entity": "继发性喉软骨软化" } ] }, { "text": "应强调精心护理和合理喂养,注意避免呛咳。", "entities": [ { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "呛咳" } ] }, { "text": "适当补充钙及维生素D。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "dru", "entity": "钙" }, { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "维生素D" } ] }, { "text": "极少数症状特别严重者可给予鼻气管插管或气管造口术。", "entities": [ { "start_idx": 13, "end_idx": 17, "type": "pro", "entity": "鼻气管插管" }, { "start_idx": 19, "end_idx": 23, "type": "pro", "entity": "气管造口术" } ] }, { "text": "另外14%的患儿主动脉瓣不分叶,因而在增厚的单瓣上有一泪滴样偏心小孔。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "单瓣" } ] }, { "text": "由于左心室高压,可有心内膜显著增厚,从而进一步损害左心室功能(继发性心内膜弹力纤维增生症)。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "左心室" }, { "start_idx": 10, "end_idx": 12, "type": "bod", "entity": "心内膜" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "左心室" }, { "start_idx": 34, "end_idx": 43, "type": "dis", "entity": "心内膜弹力纤维增生症" } ] }, { "text": "本病在西方国家颇为多见,可占先心的3%~6%,但我国发病率似较低。", "entities": [ { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "先心" } ] }, { "text": "这也可解释为什么中度主动脉瓣狭窄患儿运动时可出现心绞痛、胸前导联ST段压低及T波倒置。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "中度主动脉瓣狭窄" }, { "start_idx": 24, "end_idx": 26, "type": "sym", "entity": "心绞痛" }, { "start_idx": 28, "end_idx": 41, "type": "sym", "entity": "胸前导联ST段压低及T波倒置" } ] }, { "text": "主动脉瓣狭窄患儿偶可发生猝死,其原因几乎可肯定为室性纤颤,后者的原因也可能是由缺血或缺血样损害所引起。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "主动脉瓣狭窄" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "室性纤颤" } ] }, { "text": "主动脉瓣狭窄的另一症状为晕厥,通常发生在劳力或长期站立后。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "主动脉瓣狭窄" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "晕厥" } ] }, { "text": "较轻型患者可引起左心室肥厚,但在休息或运动时无缺血症状。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "bod", "entity": "左心室" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "左心室肥厚" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "缺血" } ] }, { "text": "严重主动脉瓣狭窄患儿生后如卵圆孔关闭,左心房压升高,左心室心输出量可维持,但可出现肺水肿的表现。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "严重主动脉瓣狭窄" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "卵圆孔" }, { "start_idx": 13, "end_idx": 17, "type": "sym", "entity": "卵圆孔关闭" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "左心房" }, { "start_idx": 19, "end_idx": 24, "type": "sym", "entity": "左心房压升高" }, { "start_idx": 26, "end_idx": 28, "type": "bod", "entity": "左心室" }, { "start_idx": 26, "end_idx": 35, "type": "sym", "entity": "左心室心输出量可维持" }, { "start_idx": 39, "end_idx": 46, "type": "sym", "entity": "出现肺水肿的表现" } ] }, { "text": "杂音往往为本病的首要体征,婴儿期大多在胸骨左缘中部最响,有时与室缺很难鉴别;其响度决定于左室的排出量,如因心力衰竭而减低,则杂音趋轻柔甚至消失。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "杂音" }, { "start_idx": 19, "end_idx": 22, "type": "bod", "entity": "胸骨左缘" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "室缺" }, { "start_idx": 44, "end_idx": 45, "type": "bod", "entity": "左室" }, { "start_idx": 53, "end_idx": 56, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "胸部X线表现心脏可不大,或有左心室、左心房增大的表现,升主动脉可示狭窄后扩张。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "胸部X线" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "心脏" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "心脏可不大" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "左心室" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "左心房" }, { "start_idx": 13, "end_idx": 25, "type": "sym", "entity": "有左心室、左心房增大的表现" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "升主动脉" }, { "start_idx": 27, "end_idx": 37, "type": "sym", "entity": "升主动脉可示狭窄后扩张" } ] }, { "text": "超声可显示主动脉瓣增厚、开放受限,呈幕顶样运动,两叶主动脉瓣,左心室壁增厚和左心室的等容收缩期延长。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 5, "end_idx": 10, "type": "sym", "entity": "主动脉瓣增厚" }, { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "开放受限" }, { "start_idx": 17, "end_idx": 22, "type": "sym", "entity": "呈幕顶样运动" }, { "start_idx": 26, "end_idx": 29, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 24, "end_idx": 29, "type": "sym", "entity": "两叶主动脉瓣" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "左心室" }, { "start_idx": 38, "end_idx": 40, "type": "bod", "entity": "左心室" }, { "start_idx": 31, "end_idx": 48, "type": "sym", "entity": "左心室壁增厚和左心室的等容收缩期延长" } ] }, { "text": "主动脉根部造影可显示主动脉瓣的活动度,有无反流,瓣环大小和狭窄后的扩张情况。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "主动脉根部造影" }, { "start_idx": 10, "end_idx": 13, "type": "bod", "entity": "主动脉瓣" }, { "start_idx": 24, "end_idx": 25, "type": "bod", "entity": "瓣环" } ] }, { "text": "急性鼻窦炎以咳嗽和分泌物增多为主要症状。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "急性鼻窦炎" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "咳嗽" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "分泌物增多" } ] }, { "text": "咳嗽、流涕持续超过30天者诊断亚急性鼻窦炎。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "咳嗽" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "流涕" }, { "start_idx": 15, "end_idx": 20, "type": "dis", "entity": "亚急性鼻窦炎" } ] }, { "text": "根据病史、症状、鼻腔检查及鼻窦华氏位摄影等检查,即可做出诊断。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "鼻腔检查" }, { "start_idx": 13, "end_idx": 19, "type": "pro", "entity": "鼻窦华氏位摄影" } ] }, { "text": "必要时进行CT检查。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "CT检查" } ] }, { "text": "对慢性顽固性鼻窦炎患儿可采用内镜鼻窦手术治疗。", "entities": [ { "start_idx": 1, "end_idx": 8, "type": "dis", "entity": "慢性顽固性鼻窦炎" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "内镜鼻窦手术" } ] }, { "text": "当人、畜接触疫水时,尾蚴很快(短至2~10秒钟)从皮肤或黏膜钻入体内,脱落尾部变为童虫。", "entities": [ { "start_idx": 25, "end_idx": 26, "type": "bod", "entity": "皮肤" }, { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "黏膜" } ] }, { "text": "潜伏期内40%~75%的患者于接触疫水部位出现尾蚴性皮炎。", "entities": [ { "start_idx": 23, "end_idx": 27, "type": "dis", "entity": "尾蚴性皮炎" } ] }, { "text": "胸部X线片一般仅见肺纹增粗、肺门阴影增宽,可有点状、云雾状或雪花状浸润性阴影,重者有粟粒状改变,3个月左右可自行吸收,不留痕迹。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "胸部X线片" }, { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "肺纹增粗" }, { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "肺门阴影增宽" }, { "start_idx": 23, "end_idx": 37, "type": "sym", "entity": "点状、云雾状或雪花状浸润性阴影" }, { "start_idx": 42, "end_idx": 46, "type": "sym", "entity": "粟粒状改变" } ] }, { "text": "无症状型起病常无明显症状而呈慢性经过,仅于普查时或因其他疾病就医时偶然发现;有症状型可表现腹胀,大便有时稀、次数增多,偶见黏液血便,食欲缺乏。", "entities": [ { "start_idx": 48, "end_idx": 57, "type": "sym", "entity": "大便有时稀、次数增多" }, { "start_idx": 59, "end_idx": 64, "type": "sym", "entity": "偶见黏液血便" }, { "start_idx": 66, "end_idx": 69, "type": "sym", "entity": "食欲缺乏" } ] }, { "text": "微波ELISA是ELISA检测方法的改进,是一种快速、高效的血吸虫病免疫学诊断方法,具有实际应用价值。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "pro", "entity": "微波ELISA" }, { "start_idx": 8, "end_idx": 14, "type": "pro", "entity": "ELISA检测" }, { "start_idx": 30, "end_idx": 38, "type": "pro", "entity": "血吸虫病免疫学诊断" } ] }, { "text": "巨脾、腹水等要与其他病因所致肝硬化鉴别。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "sym", "entity": "巨脾" }, { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "腹水" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "肝硬化" } ] }, { "text": "晚期血吸虫病则用60mg/kg(儿童70mg/kg)分3天服用。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "血吸虫病" } ] }, { "text": "消化系统症状有腹痛、恶心、腹胀、腹泻、口干。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "消化系统" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "腹痛" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "恶心" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "腹胀" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "腹泻" }, { "start_idx": 19, "end_idx": 20, "type": "sym", "entity": "口干" } ] }, { "text": "多数患儿除咳嗽及轻度呼吸困难外,缺乏一般症状。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "咳嗽" }, { "start_idx": 8, "end_idx": 14, "type": "sym", "entity": "轻度呼吸困难外" } ] }, { "text": "一般无发热。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "sym", "entity": "无发热" } ] }, { "text": "急性期应进行体位引流及气管吸引,排出油剂。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "pro", "entity": "体位引流" }, { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "气管吸引" } ] }, { "text": "必要时进行纤维支气管镜下吸引。", "entities": [ { "start_idx": 5, "end_idx": 13, "type": "pro", "entity": "纤维支气管镜下吸引" } ] }, { "text": "多发生于左肺下叶,少数位于右下叶。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "bod", "entity": "左肺下叶" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "右下叶" } ] }, { "text": "肺外型及与支气管不相通的肺内型肺隔离症,一般不出现症状,常由于并发症或其他原因进行肺X线检查时才发现有阴影而疑及本病。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "肺外型" }, { "start_idx": 5, "end_idx": 7, "type": "bod", "entity": "支气管" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "肺内型肺隔离症" }, { "start_idx": 41, "end_idx": 45, "type": "ite", "entity": "肺X线检查" }, { "start_idx": 50, "end_idx": 52, "type": "sym", "entity": "有阴影" } ] }, { "text": "胸部X线检查往往显示病变区出现大片致密阴影,其间可见单个或多个囊性透光区,囊壁厚薄不等,周围常有炎性浸润。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "ite", "entity": "胸部X线检查" }, { "start_idx": 10, "end_idx": 20, "type": "sym", "entity": "病变区出现大片致密阴影" }, { "start_idx": 22, "end_idx": 35, "type": "sym", "entity": "其间可见单个或多个囊性透光区" }, { "start_idx": 37, "end_idx": 42, "type": "sym", "entity": "囊壁厚薄不等" }, { "start_idx": 44, "end_idx": 51, "type": "sym", "entity": "周围常有炎性浸润" } ] }, { "text": "X线侧位片或CT能更清楚地显示病变的确切部位和范围,以及与邻近组织器官的关系,并可排除局灶性慢性脓胸等其他肺部疾病。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "ite", "entity": "X线侧位片" }, { "start_idx": 6, "end_idx": 7, "type": "ite", "entity": "CT" }, { "start_idx": 29, "end_idx": 34, "type": "bod", "entity": "邻近组织器官" }, { "start_idx": 43, "end_idx": 49, "type": "dis", "entity": "局灶性慢性脓胸" } ] }, { "text": "当主要侵犯鼻、鼻咽和咽部时,常诊断为急性鼻咽炎、急性咽炎、急性扁桃体炎等,也可统称为上呼吸道感染。", "entities": [ { "start_idx": 5, "end_idx": 5, "type": "bod", "entity": "鼻" }, { "start_idx": 7, "end_idx": 8, "type": "bod", "entity": "鼻咽" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "咽部" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "急性鼻咽炎" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "急性咽炎" }, { "start_idx": 29, "end_idx": 34, "type": "dis", "entity": "急性扁桃体炎" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "上呼吸道感染" } ] }, { "text": "病毒、细菌和肺炎支原体等均可引起,但原发性上感以病毒引起者最为多见,约占90%以上,主要有呼吸道合胞病毒、流感病毒、副流感病毒、腺病毒、鼻病毒、柯萨奇病毒、埃可病毒、冠状病毒等。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "mic", "entity": "病毒" }, { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "细菌" }, { "start_idx": 6, "end_idx": 10, "type": "mic", "entity": "肺炎支原体" }, { "start_idx": 18, "end_idx": 22, "type": "dis", "entity": "原发性上感" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "病毒" }, { "start_idx": 45, "end_idx": 51, "type": "mic", "entity": "呼吸道合胞病毒" }, { "start_idx": 53, "end_idx": 56, "type": "mic", "entity": "流感病毒" }, { "start_idx": 58, "end_idx": 62, "type": "mic", "entity": "副流感病毒" }, { "start_idx": 64, "end_idx": 66, "type": "mic", "entity": "腺病毒" }, { "start_idx": 68, "end_idx": 70, "type": "mic", "entity": "鼻病毒" }, { "start_idx": 72, "end_idx": 76, "type": "mic", "entity": "柯萨奇病毒" }, { "start_idx": 78, "end_idx": 81, "type": "mic", "entity": "埃可病毒" }, { "start_idx": 83, "end_idx": 86, "type": "mic", "entity": "冠状病毒" } ] }, { "text": "病毒感染后易继发细菌感染,形成混合感染。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "病毒感染" }, { "start_idx": 8, "end_idx": 11, "type": "dis", "entity": "细菌感染" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "混合感染" } ] }, { "text": "常见于溶血性链球菌、肺炎链球菌、流感嗜血杆菌等。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "mic", "entity": "溶血性链球菌" }, { "start_idx": 10, "end_idx": 14, "type": "mic", "entity": "肺炎链球菌" }, { "start_idx": 16, "end_idx": 21, "type": "mic", "entity": "流感嗜血杆菌" } ] }, { "text": "以鼻塞、喷嚏、流涕、干咳、咽痛、发热等为主要症状。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "sym", "entity": "鼻塞" }, { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "喷嚏" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "流涕" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "干咳" }, { "start_idx": 13, "end_idx": 14, "type": "sym", "entity": "咽痛" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "发热" } ] }, { "text": "婴幼儿局部症状不显著而全身症状重,可骤然起病,高热、咳嗽、食欲差,可伴有呕吐、腹泻、烦躁,甚至高热惊厥。", "entities": [ { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "高热" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "咳嗽" }, { "start_idx": 29, "end_idx": 31, "type": "sym", "entity": "食欲差" }, { "start_idx": 36, "end_idx": 37, "type": "sym", "entity": "呕吐" }, { "start_idx": 39, "end_idx": 40, "type": "sym", "entity": "腹泻" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "烦躁" }, { "start_idx": 47, "end_idx": 50, "type": "sym", "entity": "高热惊厥" } ] }, { "text": "体格检查可见咽部充血,扁桃体肿大,颌下淋巴结肿大、触痛等。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "咽部" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "咽部充血" }, { "start_idx": 11, "end_idx": 13, "type": "bod", "entity": "扁桃体" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "扁桃体肿大" }, { "start_idx": 17, "end_idx": 21, "type": "bod", "entity": "颌下淋巴结" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "颌下淋巴结肿大" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "触痛" } ] }, { "text": "病程约3~5天,如体温持续不退或病情加重,应考虑合并细菌感染或出现并发症。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "sym", "entity": "体温持续不退" }, { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "细菌感染" } ] }, { "text": "我国新中国成立前每年约100万新生儿死于破伤风,建国后发病率和死亡率显著下降,但在边远农村、山区及私自接生者新生儿破伤风仍不罕见。", "entities": [ { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "破伤风" } ] }, { "text": "呼吸肌与喉肌痉挛引起呼吸困难、青紫、窒息;咽肌痉挛使唾液充满口腔;膀胱及直肠括约肌痉挛可导致尿潴留和便秘。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "呼吸肌与喉肌痉挛引起呼吸困难" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "青紫" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "窒息" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "咽肌痉挛" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "唾液充满口腔" }, { "start_idx": 33, "end_idx": 42, "type": "sym", "entity": "膀胱及直肠括约肌痉挛" }, { "start_idx": 46, "end_idx": 48, "type": "sym", "entity": "尿潴留" }, { "start_idx": 50, "end_idx": 51, "type": "sym", "entity": "便秘" } ] }, { "text": "NICH预后与其出血类型有关。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "NICH" } ] }, { "text": "小脑出血预后差,出生后不久即死亡。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "小脑" }, { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "小脑出血" } ] }, { "text": "(1)两性霉素B:适应证为曲菌、念珠菌、隐球菌和组织胞质菌感染。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 13, "end_idx": 30, "type": "dis", "entity": "曲菌、念珠菌、隐球菌和组织胞质菌感染" } ] }, { "text": "(1)氟康唑:适应证为隐球菌和念珠菌感染,对曲菌感染无效。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dru", "entity": "氟康唑" }, { "start_idx": 11, "end_idx": 19, "type": "dis", "entity": "隐球菌和念珠菌感染" }, { "start_idx": 22, "end_idx": 25, "type": "dis", "entity": "曲菌感染" } ] }, { "text": "本品在16岁以下儿童体内的血浆半衰期与成人不同,故对不同年龄儿童推荐剂量如下:>4周的患儿:深部真菌感染,6mg/(kg•d),每日给药一次;严重威胁生命的感染,12mg/(kg•d),每日给药1次。", "entities": [ { "start_idx": 46, "end_idx": 51, "type": "sym", "entity": "深部真菌感染" } ] }, { "text": "(2)伊曲康唑:适应证为曲菌、念珠菌、隐球菌和组织胞质菌感染,对镰刀霉菌活性低,对毛霉菌无效。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "伊曲康唑" }, { "start_idx": 12, "end_idx": 29, "type": "dis", "entity": "曲菌、念珠菌、隐球菌和组织胞质菌感染" }, { "start_idx": 32, "end_idx": 35, "type": "mic", "entity": "镰刀霉菌" }, { "start_idx": 41, "end_idx": 43, "type": "mic", "entity": "毛霉菌" } ] }, { "text": "(3)伏立康唑:适应证为曲菌、念珠菌以及镰刀霉菌感染,对接合菌无活性。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "伏立康唑" }, { "start_idx": 12, "end_idx": 25, "type": "dis", "entity": "曲菌、念珠菌以及镰刀霉菌感染" } ] }, { "text": "应注意原发病的诊断,如胱氨酸储积病者,眼裂隙灯检查可见角膜有胱氨酸结晶沉着,骨髓或血白细胞中胱氨酸含量增加并见到胱氨酸结晶,对本病确切诊断十分重要。", "entities": [ { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "胱氨酸储积病" }, { "start_idx": 19, "end_idx": 22, "type": "equ", "entity": "眼裂隙灯" }, { "start_idx": 27, "end_idx": 28, "type": "bod", "entity": "角膜" }, { "start_idx": 27, "end_idx": 36, "type": "sym", "entity": "角膜有胱氨酸结晶沉着" }, { "start_idx": 38, "end_idx": 39, "type": "bod", "entity": "骨髓" }, { "start_idx": 41, "end_idx": 44, "type": "bod", "entity": "血白细胞" }, { "start_idx": 38, "end_idx": 60, "type": "sym", "entity": "骨髓或血白细胞中胱氨酸含量增加并见到胱氨酸结晶" } ] }, { "text": "临床上溶血症状较轻,常伴有雷诺症状,可发生肢端坏死、溃疡。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "sym", "entity": "溶血" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "肢端坏死" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "溃疡" } ] }, { "text": "如以专抗IgG或IgM的抗血清作Coombs试验时,直接试验阴性,而抗补体Coombs试验则阳性。", "entities": [ { "start_idx": 16, "end_idx": 23, "type": "pro", "entity": "Coombs试验" }, { "start_idx": 37, "end_idx": 44, "type": "pro", "entity": "Coombs试验" } ] }, { "text": "糖皮质激素对部分患儿有效,反复发作者可应用免疫抑制剂如苯丁酸氮芥、环磷酰胺、硫唑嘌呤等,有一定的疗效;本病可应用血浆分离法(plasmapheresis)去除冷凝抗体,有一定的短期疗效;此外,脾切除、输注洗涤红细胞等也有一定的疗效。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "糖皮质激素" }, { "start_idx": 21, "end_idx": 25, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "苯丁酸氮芥" }, { "start_idx": 33, "end_idx": 36, "type": "dru", "entity": "环磷酰胺" }, { "start_idx": 38, "end_idx": 41, "type": "dru", "entity": "硫唑嘌呤" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "血浆" }, { "start_idx": 104, "end_idx": 106, "type": "bod", "entity": "红细胞" } ] }, { "text": "皮肤散在的自发性瘀点、瘀斑多为血小板减少性紫癜;肌肉血肿伴关节腔出血者多为血友病。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "sym", "entity": "自发性瘀点" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "瘀斑" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "血小板减少性紫癜" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "肌肉血肿" }, { "start_idx": 29, "end_idx": 33, "type": "sym", "entity": "关节腔出血" }, { "start_idx": 37, "end_idx": 39, "type": "dis", "entity": "血友病" } ] }, { "text": "血小板减少者应观察骨髓巨核细胞分化和产血小板情况,测定PAIg。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "血小板" }, { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "骨髓巨核细胞" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "血小板" }, { "start_idx": 27, "end_idx": 30, "type": "bod", "entity": "PAIg" } ] }, { "text": "当PCT正常、APTT、PT和TT均延长时,应属纤维蛋白生成障碍,涉及因子Ⅰ、Ⅻ异常或抗凝物质过多,可进一步测定纤维蛋白原量或作甲苯胺蓝纠正试验。", "entities": [ { "start_idx": 1, "end_idx": 3, "type": "ite", "entity": "PCT" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "APTT" }, { "start_idx": 12, "end_idx": 13, "type": "ite", "entity": "PT" }, { "start_idx": 15, "end_idx": 16, "type": "ite", "entity": "TT" }, { "start_idx": 24, "end_idx": 27, "type": "bod", "entity": "纤维蛋白" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "因子Ⅰ、Ⅻ异常" }, { "start_idx": 56, "end_idx": 59, "type": "bod", "entity": "纤维蛋白" }, { "start_idx": 64, "end_idx": 67, "type": "bod", "entity": "甲苯胺蓝" } ] }, { "text": "播散性血管内凝血(DIC)引起的出血涉及多方面的原因,需作血小板计数、PT、血浆纤维蛋白原测定、血浆鱼精蛋白副凝固(3P)试验,血浆纤维蛋白降解产物(FDP)、D-二聚体测定和乙醇胶试验等确诊。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "bod", "entity": "凝血" }, { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "DIC" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "出血" }, { "start_idx": 29, "end_idx": 31, "type": "bod", "entity": "血小板" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "PT" }, { "start_idx": 38, "end_idx": 44, "type": "bod", "entity": "血浆纤维蛋白原" }, { "start_idx": 48, "end_idx": 56, "type": "bod", "entity": "血浆鱼精蛋白副凝固" }, { "start_idx": 58, "end_idx": 59, "type": "bod", "entity": "3P" }, { "start_idx": 64, "end_idx": 69, "type": "bod", "entity": "血浆纤维蛋白" }, { "start_idx": 75, "end_idx": 77, "type": "bod", "entity": "FDP" }, { "start_idx": 80, "end_idx": 84, "type": "bod", "entity": "D-二聚体" }, { "start_idx": 88, "end_idx": 90, "type": "bod", "entity": "乙醇胶" } ] }, { "text": "在存在中等流量的左向右分流时,早产儿比足月儿更早出现心力衰竭。", "entities": [ { "start_idx": 26, "end_idx": 29, "type": "dis", "entity": "心力衰竭" } ] }, { "text": "肺毛细血管通透性增加可致肺水肿。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "bod", "entity": "肺毛细血管" }, { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "肺水肿" } ] }, { "text": "早产儿动脉导管未闭的发病率极高。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "动脉导管未闭" } ] }, { "text": "在1750g以下的早产儿约有半数伴有动脉导管未闭,而在体重不到1200g者发生率可达80%左右。", "entities": [ { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "动脉导管未闭" } ] }, { "text": "最初可在胸骨左缘第2肋间闻及短促、柔和的收缩期杂音,随着左向右分流的增加,周围血管搏动增强,心前区搏动活跃,杂音增强且延长至舒张期。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "胸骨" }, { "start_idx": 39, "end_idx": 40, "type": "bod", "entity": "血管" }, { "start_idx": 46, "end_idx": 48, "type": "bod", "entity": "心前区" } ] }, { "text": "动脉导管中等或较大的婴儿,因肺血管阻力逐渐降低,左向右分流增加,生后1~2月可出现心力衰竭症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "动脉导管" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肺血管" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "心力衰竭" } ] }, { "text": "此类患儿喂养困难,多汗,吸奶时呼吸短促,体重增长较慢,身长亦低于正常儿。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "sym", "entity": "喂养困难" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "多汗" }, { "start_idx": 12, "end_idx": 18, "type": "sym", "entity": "吸奶时呼吸短促" }, { "start_idx": 20, "end_idx": 25, "type": "sym", "entity": "体重增长较慢" }, { "start_idx": 27, "end_idx": 34, "type": "sym", "entity": "身长亦低于正常儿" } ] }, { "text": "可有呼吸急促及肋间隙凹陷等呼吸系统体征,大量左向右分流时可见哈里森沟。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 7, "end_idx": 18, "type": "sym", "entity": "肋间隙凹陷等呼吸系统体征" }, { "start_idx": 20, "end_idx": 33, "type": "sym", "entity": "大量左向右分流时可见哈里森沟" } ] }, { "text": "由于脉压增大,婴儿可表现为周围血管搏动增强,而年长儿则表现为搏动减弱。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "ite", "entity": "脉压" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "周围血管搏动增强" }, { "start_idx": 30, "end_idx": 33, "type": "sym", "entity": "搏动减弱" } ] }, { "text": "少数未经治疗而存活的动脉导管粗大的患儿,由于肺血管阻力增加,将发生不可逆的肺血管病变。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "dis", "entity": "动脉导管粗大" }, { "start_idx": 22, "end_idx": 24, "type": "bod", "entity": "肺血管" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "肺血管病变" } ] }, { "text": "其他先天性心脏病,如肺动脉闭锁、大动脉转位等伴发的动脉导管未闭可通过胸骨上矢状切面得到理想地显示。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "先天性心脏病" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "肺动脉闭锁" }, { "start_idx": 16, "end_idx": 20, "type": "dis", "entity": "大动脉转位" }, { "start_idx": 25, "end_idx": 30, "type": "dis", "entity": "动脉导管未闭" }, { "start_idx": 34, "end_idx": 35, "type": "bod", "entity": "胸骨" } ] }, { "text": "对于生后体重低于1000g的早产儿在出生10天内静脉滴注吲哚美辛可有效关闭动脉导管。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "静脉滴注" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "吲哚美辛" }, { "start_idx": 37, "end_idx": 40, "type": "bod", "entity": "动脉导管" } ] }, { "text": "经治疗心功能不全不能纠正者,需手术结扎动脉导管。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "心功能不全" }, { "start_idx": 15, "end_idx": 22, "type": "pro", "entity": "手术结扎动脉导管" } ] }, { "text": "手术并发症并不多见,可有导管撕裂、膈神经麻痹、乳糜胸、误扎左肺动脉和降主动脉,故术后检查股动脉搏动十分必要。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "导管撕裂" }, { "start_idx": 17, "end_idx": 21, "type": "dis", "entity": "膈神经麻痹" }, { "start_idx": 23, "end_idx": 25, "type": "dis", "entity": "乳糜胸" }, { "start_idx": 27, "end_idx": 37, "type": "dis", "entity": "误扎左肺动脉和降主动脉" }, { "start_idx": 40, "end_idx": 48, "type": "pro", "entity": "术后检查股动脉搏动" } ] }, { "text": "胸腔镜手术已成功应用于儿童动脉导管的关闭。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "胸腔镜手术" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "儿童动脉导管" } ] }, { "text": "常见促使发病的因素包括早产儿、新生儿、营养不良及久病虚弱,慢性消耗性疾病如恶性肿瘤,影响免疫功能的单核-吞噬细胞系统疾患及血液病,代谢性疾病及肾衰竭,长期使用肾上腺皮质激素及其他免疫抑制剂,先天性免疫功能缺陷,长期使用广谱抗生素等。", "entities": [ { "start_idx": 37, "end_idx": 40, "type": "dis", "entity": "恶性肿瘤" }, { "start_idx": 49, "end_idx": 59, "type": "dis", "entity": "单核-吞噬细胞系统疾患" }, { "start_idx": 61, "end_idx": 63, "type": "dis", "entity": "血液病" }, { "start_idx": 65, "end_idx": 69, "type": "dis", "entity": "代谢性疾病" }, { "start_idx": 71, "end_idx": 73, "type": "dis", "entity": "肾衰竭" }, { "start_idx": 79, "end_idx": 85, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 89, "end_idx": 93, "type": "dru", "entity": "免疫抑制剂" }, { "start_idx": 95, "end_idx": 103, "type": "dis", "entity": "先天性免疫功能缺陷" }, { "start_idx": 109, "end_idx": 113, "type": "dru", "entity": "广谱抗生素" } ] }, { "text": "真菌性肺炎的症状和体征无特异性,但可有以下特点:①持续高热不退;②明显烦躁不安;③咳痰无色透明、黏稠;④肺部可闻及粗细不等湿啰音,也可引起脓胸或肺实变体征;⑤同时伴有其他部位真菌感染的表现,如鹅口疮、大便呈豆渣样、肛周有白膜等;⑥病程迁延不愈,抗生素治疗无效,且病情日益加重。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "真菌性肺炎" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "持续高热不退" }, { "start_idx": 33, "end_idx": 38, "type": "sym", "entity": "明显烦躁不安" }, { "start_idx": 41, "end_idx": 49, "type": "sym", "entity": "咳痰无色透明、黏稠" }, { "start_idx": 52, "end_idx": 63, "type": "sym", "entity": "肺部可闻及粗细不等湿啰音" }, { "start_idx": 69, "end_idx": 70, "type": "sym", "entity": "脓胸" }, { "start_idx": 72, "end_idx": 74, "type": "sym", "entity": "肺实变" }, { "start_idx": 83, "end_idx": 90, "type": "sym", "entity": "其他部位真菌感染" }, { "start_idx": 96, "end_idx": 98, "type": "sym", "entity": "鹅口疮" }, { "start_idx": 100, "end_idx": 105, "type": "sym", "entity": "大便呈豆渣样" }, { "start_idx": 107, "end_idx": 111, "type": "sym", "entity": "肛周有白膜" }, { "start_idx": 115, "end_idx": 120, "type": "sym", "entity": "病程迁延不愈" }, { "start_idx": 122, "end_idx": 128, "type": "sym", "entity": "抗生素治疗无效" } ] }, { "text": "治疗方法为停止使用抗生素及肾上腺皮质激素,酌情选择抗真菌药物治疗。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "dru", "entity": "抗生素" }, { "start_idx": 13, "end_idx": 19, "type": "dru", "entity": "肾上腺皮质激素" }, { "start_idx": 25, "end_idx": 29, "type": "dru", "entity": "抗真菌药物" } ] }, { "text": "两性霉素B对绝大多数真菌均有较强的抗菌活性,多年来广泛用于治疗各种真菌感染,具有较好的临床疗效。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 10, "end_idx": 11, "type": "mic", "entity": "真菌" }, { "start_idx": 33, "end_idx": 34, "type": "mic", "entity": "真菌" } ] }, { "text": "但不良反应多,如发热、氮质血症、低钾血症、低镁血症、血栓性静脉炎等。", "entities": [ { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "发热" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "氮质血症" }, { "start_idx": 16, "end_idx": 19, "type": "sym", "entity": "低钾血症" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "低镁血症" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "血栓性静脉炎" } ] }, { "text": "伏立康唑、卡泊芬净、伊曲康唑等对曲菌有良好的疗效。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dru", "entity": "伏立康唑" }, { "start_idx": 5, "end_idx": 8, "type": "dru", "entity": "卡泊芬净" }, { "start_idx": 10, "end_idx": 13, "type": "dru", "entity": "伊曲康唑" }, { "start_idx": 16, "end_idx": 17, "type": "mic", "entity": "曲菌" } ] }, { "text": "手术彻底切除肿瘤是唯一的治疗方法。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "肿瘤" } ] }, { "text": "该肿瘤对放疗无效,化疗很少能完全治疗,肿瘤多向肺转移,达80%左右,其次为淋巴结,也可能转移至肝、脾、胰、脑等部位,总之未能手术完全切除者,预后极差。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "肿瘤" }, { "start_idx": 4, "end_idx": 5, "type": "pro", "entity": "放疗" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "化疗" }, { "start_idx": 19, "end_idx": 20, "type": "dis", "entity": "肿瘤" }, { "start_idx": 23, "end_idx": 23, "type": "bod", "entity": "肺" }, { "start_idx": 37, "end_idx": 39, "type": "bod", "entity": "淋巴结" }, { "start_idx": 47, "end_idx": 47, "type": "bod", "entity": "肝" }, { "start_idx": 49, "end_idx": 49, "type": "bod", "entity": "脾" }, { "start_idx": 51, "end_idx": 51, "type": "bod", "entity": "胰" }, { "start_idx": 53, "end_idx": 53, "type": "bod", "entity": "脑" }, { "start_idx": 62, "end_idx": 63, "type": "pro", "entity": "手术" } ] }, { "text": "病程第2周若无有效治疗,有10%~40%患儿在行B型超声或冠状动脉造影时见各种冠状动脉病变(动脉扩张,动脉瘤),冠状动脉扩张好发部位依次为左冠脉主干、左前降支及右冠状动脉近端(轻度扩张直径>3mm而≤4mm、中度为瘤样扩张4~7mm、重度扩张为巨大冠脉瘤≥8mm)。", "entities": [ { "start_idx": 24, "end_idx": 27, "type": "pro", "entity": "B型超声" }, { "start_idx": 29, "end_idx": 34, "type": "pro", "entity": "冠状动脉造影" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "冠状动脉" }, { "start_idx": 46, "end_idx": 49, "type": "dis", "entity": "动脉扩张" }, { "start_idx": 51, "end_idx": 53, "type": "dis", "entity": "动脉瘤" }, { "start_idx": 56, "end_idx": 61, "type": "dis", "entity": "冠状动脉扩张" }, { "start_idx": 69, "end_idx": 73, "type": "bod", "entity": "左冠脉主干" }, { "start_idx": 75, "end_idx": 78, "type": "bod", "entity": "左前降支" }, { "start_idx": 80, "end_idx": 86, "type": "bod", "entity": "右冠状动脉近端" } ] }, { "text": "其特征为呕吐、便秘,常有呼吸道感染,血钙特高,弱智,额突,斜视,眉嵴及下颌较突出,上唇短吊,有称此为William综合征者。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "sym", "entity": "呕吐、便秘" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "呼吸道感染" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "血钙特高" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "弱智" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "额突" }, { "start_idx": 29, "end_idx": 30, "type": "sym", "entity": "斜视" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "眉嵴" }, { "start_idx": 35, "end_idx": 36, "type": "bod", "entity": "下颌" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "眉嵴及下颌较突出" }, { "start_idx": 41, "end_idx": 42, "type": "bod", "entity": "上唇" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "上唇短吊" }, { "start_idx": 50, "end_idx": 59, "type": "dis", "entity": "William综合征" } ] }, { "text": "X线胸片无特征性表现。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "X线胸片" } ] }, { "text": "心电图可显示左心室肥厚,如狭窄严重则左胸导联可出现T波倒置。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "心电图" }, { "start_idx": 6, "end_idx": 8, "type": "bod", "entity": "左心室" }, { "start_idx": 6, "end_idx": 10, "type": "sym", "entity": "左心室肥厚" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "左胸" } ] }, { "text": "心导管及心血管造影术可证实诊断及确定严重程度。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "心导管及心血管造影术" } ] }, { "text": "一般认为跨狭窄段压差>50mmHg有手术指征。", "entities": [ { "start_idx": 18, "end_idx": 19, "type": "pro", "entity": "手术" } ] }, { "text": "呼吸性碱中毒临床主要出现原发疾病所致的相应症状及体征。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "呼吸性碱中毒" } ] }, { "text": "同时可伴呕吐、胸痛、吞咽困难、消化道出血及肠梗阻症状。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "sym", "entity": "呕吐" }, { "start_idx": 7, "end_idx": 8, "type": "sym", "entity": "胸痛" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "吞咽困难" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "消化道出血" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "肠梗阻症状" } ] }, { "text": "新生儿期出现呼吸困难及阵发性发绀时,应考虑到本病可能。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 11, "end_idx": 15, "type": "sym", "entity": "阵发性发绀" } ] }, { "text": "X线检查是确诊先天性膈疝的依据。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "ite", "entity": "X线检查" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "先天性膈疝" } ] }, { "text": "有文献报道对危重症患儿,术前先采取体外膜肺(ECMO)、允许性高碳酸血症、NO吸入、表面活性物质等控制持续肺动脉高压,改善氧合,病情稳定后再手术可改善预后。", "entities": [ { "start_idx": 17, "end_idx": 26, "type": "pro", "entity": "体外膜肺(ECMO)" }, { "start_idx": 28, "end_idx": 35, "type": "pro", "entity": "允许性高碳酸血症" }, { "start_idx": 37, "end_idx": 40, "type": "pro", "entity": "NO吸入" }, { "start_idx": 42, "end_idx": 47, "type": "pro", "entity": "表面活性物质" }, { "start_idx": 53, "end_idx": 57, "type": "dis", "entity": "肺动脉高压" } ] }, { "text": "有人甚至用水汽雾化的方法,提高暖箱内的湿度达到100%。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "pro", "entity": "水汽雾化" }, { "start_idx": 15, "end_idx": 16, "type": "equ", "entity": "暖箱" } ] }, { "text": "密切观察有无呼吸暂停的发作,对已经出现呼吸暂停的新生儿可以使用咖啡因或氨茶碱进行治疗,应以前者为主。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "咖啡因" }, { "start_idx": 35, "end_idx": 37, "type": "dru", "entity": "氨茶碱" } ] }, { "text": "对于因PDA引起的充血性心力衰竭,可以使用洋地黄类药物治疗,但要注意对其血浓度的监测。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "PDA" }, { "start_idx": 9, "end_idx": 15, "type": "sym", "entity": "充血性心力衰竭" }, { "start_idx": 21, "end_idx": 26, "type": "dru", "entity": "洋地黄类药物" }, { "start_idx": 36, "end_idx": 38, "type": "ite", "entity": "血浓度" } ] }, { "text": "病变发生于直接受损部位,多见于舌的侧缘,也可发生于唇、颊及他处黏膜,可表现为红肿、出血或溃疡,伴有局部疼痛,如继发感染,则可引起局部淋巴结肿大。", "entities": [ { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "舌" }, { "start_idx": 25, "end_idx": 25, "type": "bod", "entity": "唇" }, { "start_idx": 27, "end_idx": 27, "type": "bod", "entity": "颊" }, { "start_idx": 31, "end_idx": 32, "type": "bod", "entity": "黏膜" }, { "start_idx": 38, "end_idx": 39, "type": "sym", "entity": "红肿" }, { "start_idx": 41, "end_idx": 42, "type": "sym", "entity": "出血" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "溃疡" }, { "start_idx": 47, "end_idx": 52, "type": "sym", "entity": "伴有局部疼痛" }, { "start_idx": 54, "end_idx": 58, "type": "sym", "entity": "如继发感染" }, { "start_idx": 64, "end_idx": 70, "type": "dis", "entity": "局部淋巴结肿大" } ] }, { "text": "患儿血小板数量少,形态小并有致密颗粒减少,临床上多在婴幼儿期发病,出血重,多死于反复感染和出血。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "bod", "entity": "血小板" }, { "start_idx": 33, "end_idx": 34, "type": "sym", "entity": "出血" }, { "start_idx": 42, "end_idx": 43, "type": "sym", "entity": "感染" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "出血" } ] }, { "text": "此外还可出现体重下降或生长发育不良。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "体重下降" }, { "start_idx": 11, "end_idx": 16, "type": "sym", "entity": "生长发育不良" } ] }, { "text": "在明确为其他疾病前,1岁以内患儿发生胆汁性呕吐应首先考虑肠旋转不良。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "sym", "entity": "胆汁性呕吐" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "肠旋转不良" } ] }, { "text": "中肠扭转目前无法预测何时或在何种情况下会发生,故对胆汁性呕吐患儿,必须积极诊治,绝不允许只作观察而任其发展至绞窄性肠梗阻。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "中肠扭转" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "胆汁性呕吐" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "绞窄性肠梗阻" } ] }, { "text": "除了胆汁性呕吐,患儿可有腹胀、脱水和激惹等;绞窄性肠梗阻患儿则有意识淡漠及感染性休克表现;其他临床表现包括腹壁潮红、腹膜炎、酸中毒、血小板减少、白细胞增多或减少,以及由肠黏液局部缺血所至肠道出血和(或)黑便。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "sym", "entity": "胆汁性呕吐" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "腹胀" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "脱水" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "激惹" }, { "start_idx": 22, "end_idx": 27, "type": "dis", "entity": "绞窄性肠梗阻" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "意识淡漠" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "感染性休克" }, { "start_idx": 53, "end_idx": 56, "type": "sym", "entity": "腹壁潮红" }, { "start_idx": 58, "end_idx": 60, "type": "sym", "entity": "腹膜炎" }, { "start_idx": 62, "end_idx": 64, "type": "sym", "entity": "酸中毒" }, { "start_idx": 66, "end_idx": 70, "type": "sym", "entity": "血小板减少" }, { "start_idx": 72, "end_idx": 79, "type": "sym", "entity": "白细胞增多或减少" }, { "start_idx": 84, "end_idx": 90, "type": "sym", "entity": "肠黏液局部缺血" }, { "start_idx": 93, "end_idx": 96, "type": "sym", "entity": "肠道出血" }, { "start_idx": 101, "end_idx": 102, "type": "sym", "entity": "黑便" } ] }, { "text": "中肠扭转影像学表现有:①胃出口梗阻,可见扩张的胃泡,远端气体减少;②典型的双泡征提示十二指肠梗阻。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "中肠扭转" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "胃出口梗阻" }, { "start_idx": 18, "end_idx": 24, "type": "sym", "entity": "可见扩张的胃泡" }, { "start_idx": 26, "end_idx": 31, "type": "sym", "entity": "远端气体减少" }, { "start_idx": 42, "end_idx": 47, "type": "dis", "entity": "十二指肠梗阻" } ] }, { "text": "中肠扭转最典型表现是十二指肠第2、3段出现“鸟嘴样”改变;十二指肠部分梗阻则可呈“螺旋样”改变。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "中肠扭转" }, { "start_idx": 10, "end_idx": 27, "type": "sym", "entity": "十二指肠第2、3段出现“鸟嘴样”改变" }, { "start_idx": 29, "end_idx": 46, "type": "sym", "entity": "十二指肠部分梗阻则可呈“螺旋样”改变" } ] }, { "text": "正常情况下,肠系膜上静脉(SMV)位于SMA右侧,若位于SMA前方或左侧,提示可能存在肠旋转不良。", "entities": [ { "start_idx": 6, "end_idx": 11, "type": "bod", "entity": "肠系膜上静脉" }, { "start_idx": 13, "end_idx": 15, "type": "bod", "entity": "SMV" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "SMA" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "SMA" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "肠旋转不良" } ] }, { "text": "肠旋转不良松解术后,常见胃肠道蠕动功能紊乱;而假性神经性肠梗阻提示小肠存在内在神经支配缺陷可能。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肠旋转不良" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "小肠" } ] }, { "text": "其他术后并发症包括:绞窄性肠梗阻、迟发性肠梗阻及手术相关出血,另外肠扭转复位可致小肠再灌注损伤。", "entities": [ { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 17, "end_idx": 22, "type": "dis", "entity": "迟发性肠梗阻" }, { "start_idx": 33, "end_idx": 35, "type": "dis", "entity": "肠扭转" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "小肠" } ] }, { "text": "天然变应性原制剂疗法有几十年的历史,是IgE介导的过敏疾患的唯一对因疗法。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "pro", "entity": "天然变应性原制剂疗法" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "IgE" } ] }, { "text": "但具体开始治疗年龄还要考虑治疗的安全性,目前多在5岁以后才开始考虑进行变应原特异性免疫治疗,治疗之前应进行特异性变应原诊断试验,以明确机体对什么过敏,以及过敏的强度,特异性诊断试验包括皮肤试验、变应原支气管激发试验、血清变应原特异性IgE测定等方法。", "entities": [ { "start_idx": 35, "end_idx": 44, "type": "pro", "entity": "变应原特异性免疫治疗" }, { "start_idx": 83, "end_idx": 89, "type": "pro", "entity": "特异性诊断试验" }, { "start_idx": 92, "end_idx": 95, "type": "pro", "entity": "皮肤试验" }, { "start_idx": 97, "end_idx": 106, "type": "pro", "entity": "变应原支气管激发试验" }, { "start_idx": 108, "end_idx": 120, "type": "pro", "entity": "血清变应原特异性IgE测定" } ] }, { "text": "一般而言,免疫反应是一种生理性保护反应,一旦这种适度、有益的保护性反应失衡,即可导致异常免疫反应,产生变态反应性疾病、自身免疫性疾病、免疫缺陷病及肿瘤。", "entities": [ { "start_idx": 51, "end_idx": 57, "type": "dis", "entity": "变态反应性疾病" }, { "start_idx": 59, "end_idx": 65, "type": "dis", "entity": "自身免疫性疾病" }, { "start_idx": 67, "end_idx": 71, "type": "dis", "entity": "免疫缺陷病" }, { "start_idx": 73, "end_idx": 74, "type": "dis", "entity": "肿瘤" } ] }, { "text": "在47,XYY及48,XXYY核型,具有两个Y染色体患者高体型表现更为明显。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "bod", "entity": "XYY" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "XXYY" }, { "start_idx": 22, "end_idx": 25, "type": "bod", "entity": "Y染色体" } ] }, { "text": "我国近年来已开展了儿童言语和语言障碍的临床诊治。", "entities": [ { "start_idx": 11, "end_idx": 17, "type": "dis", "entity": "言语和语言障碍" } ] }, { "text": "大脑的损伤或肿瘤使儿童产生获得性失语症,即在儿童发展了说话成句的语言能力后,因为大脑的病灶致使语言损害。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "dis", "entity": "大脑的损伤或肿瘤" }, { "start_idx": 13, "end_idx": 18, "type": "dis", "entity": "获得性失语症" }, { "start_idx": 40, "end_idx": 41, "type": "bod", "entity": "大脑" } ] }, { "text": "临床上出现不同类型的失语症,例如,儿童听觉理解障碍但言语流利的,称为感觉性失语症;对目标物不能命名的称为命名性失语症;难以找到适当词语表达的称为表达性失语症;言语不流利且费力的称为运动性失语症。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "失语症" }, { "start_idx": 34, "end_idx": 39, "type": "dis", "entity": "感觉性失语症" }, { "start_idx": 52, "end_idx": 57, "type": "dis", "entity": "命名性失语症" }, { "start_idx": 72, "end_idx": 77, "type": "dis", "entity": "表达性失语症" }, { "start_idx": 90, "end_idx": 95, "type": "dis", "entity": "运动性失语症" } ] }, { "text": "例如选择性缄默症是一种较少的语言障碍,通常在5岁前发病,患儿在某些特定的情境中如学校等不说话。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "选择性缄默症" } ] }, { "text": "最常见的音质问题是声音嘶哑,持久的或进行性的声音嘶哑,特别是伴有喘鸣或可听得见的呼吸音,需要进一步用纤维镜检查,以发现咽乳头状瘤、先天性声门蹼或声带结节。", "entities": [ { "start_idx": 9, "end_idx": 12, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 22, "end_idx": 25, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 59, "end_idx": 63, "type": "dis", "entity": "咽乳头状瘤" }, { "start_idx": 65, "end_idx": 70, "type": "dis", "entity": "先天性声门蹼" }, { "start_idx": 72, "end_idx": 75, "type": "dis", "entity": "声带结节" } ] }, { "text": "儿童声带结节常常因为大声说话或不停地说话所致。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "儿童声带结节" } ] }, { "text": "儿童腺样增殖体肥大可出现慢性的无鼻音的发声。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "腺样增殖体肥大" } ] }, { "text": "目前国内已利用电脑的多媒体功能,采用临床医学软件作为一种治疗手段,结合个体治疗中的其他方法如改变响度、喉部按摩、半吞咽、改变舌位、减少硬起音、放松、呼吸训练等,达到治疗效果。", "entities": [ { "start_idx": 51, "end_idx": 54, "type": "pro", "entity": "喉部按摩" }, { "start_idx": 74, "end_idx": 77, "type": "pro", "entity": "呼吸训练" } ] }, { "text": "羊水过少妨碍胎儿胸廓的正常活动及肺在子宫内的扩张,造成肺发育不良,生后患儿常有呼吸困难、发绀、呼吸窘迫综合征、气胸及纵隔气肿,可死于呼吸衰竭。", "entities": [ { "start_idx": 0, "end_idx": 23, "type": "sym", "entity": "羊水过少妨碍胎儿胸廓的正常活动及肺在子宫内的扩张" }, { "start_idx": 25, "end_idx": 31, "type": "sym", "entity": "造成肺发育不良" }, { "start_idx": 33, "end_idx": 42, "type": "sym", "entity": "生后患儿常有呼吸困难" }, { "start_idx": 44, "end_idx": 45, "type": "sym", "entity": "发绀" }, { "start_idx": 47, "end_idx": 53, "type": "sym", "entity": "呼吸窘迫综合征" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "气胸" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "纵隔气肿" }, { "start_idx": 63, "end_idx": 69, "type": "sym", "entity": "可死于呼吸衰竭" } ] }, { "text": "后尿道瓣膜导致严重尿路梗阻的新生儿可有严重的泌尿系感染、尿毒症、脱水及电解质紊乱。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 5, "end_idx": 39, "type": "sym", "entity": "导致严重尿路梗阻的新生儿可有严重的泌尿系感染、尿毒症、脱水及电解质紊乱" } ] }, { "text": "至婴儿期的患儿可有生长发育迟缓及尿路感染,甚至败血症。", "entities": [ { "start_idx": 1, "end_idx": 19, "type": "sym", "entity": "婴儿期的患儿可有生长发育迟缓及尿路感染" }, { "start_idx": 21, "end_idx": 25, "type": "sym", "entity": "甚至败血症" } ] }, { "text": "学龄期儿童主要表现为排尿时间延长,尿线细,排尿费力,往往有残余尿而导致尿失禁及遗尿。", "entities": [ { "start_idx": 10, "end_idx": 15, "type": "sym", "entity": "排尿时间延长" }, { "start_idx": 17, "end_idx": 19, "type": "sym", "entity": "尿线细" }, { "start_idx": 21, "end_idx": 24, "type": "sym", "entity": "排尿费力" }, { "start_idx": 26, "end_idx": 40, "type": "sym", "entity": "往往有残余尿而导致尿失禁及遗尿" } ] }, { "text": "VCUG可见前列腺部尿道扩张,其远端尿道极细,膀胱边缘不光滑,有小梁小室形成,可有不同程度的膀胱输尿管反流。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "VCUG" }, { "start_idx": 6, "end_idx": 13, "type": "sym", "entity": "前列腺部尿道扩张" }, { "start_idx": 15, "end_idx": 21, "type": "sym", "entity": "其远端尿道极细" }, { "start_idx": 23, "end_idx": 29, "type": "sym", "entity": "膀胱边缘不光滑" }, { "start_idx": 31, "end_idx": 37, "type": "sym", "entity": "有小梁小室形成" }, { "start_idx": 39, "end_idx": 52, "type": "sym", "entity": "可有不同程度的膀胱输尿管反流" } ] }, { "text": "后尿道瓣膜的胎儿首先表现为肾积水,但有其特点,常为双侧肾与输尿管积水、膀胱壁增厚及羊水量减少。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "后尿道瓣膜" }, { "start_idx": 13, "end_idx": 15, "type": "sym", "entity": "肾积水" }, { "start_idx": 25, "end_idx": 33, "type": "sym", "entity": "双侧肾与输尿管积水" }, { "start_idx": 35, "end_idx": 45, "type": "sym", "entity": "膀胱壁增厚及羊水量减少" } ] }, { "text": "对不能经尿道放入内镜的患儿可经膀胱造瘘处放入,顺行电灼瓣膜。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "尿道" }, { "start_idx": 8, "end_idx": 9, "type": "equ", "entity": "内镜" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "膀胱造瘘" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "电灼瓣膜" } ] }, { "text": "由于儿童非霍奇金淋巴瘤涉及游走于全身各处的淋巴细胞,其在发病部位和蔓延速度上类似于儿童白血病,倾向于归类为全身性疾病。", "entities": [ { "start_idx": 2, "end_idx": 10, "type": "dis", "entity": "儿童非霍奇金淋巴瘤" }, { "start_idx": 21, "end_idx": 24, "type": "bod", "entity": "淋巴细胞" }, { "start_idx": 41, "end_idx": 45, "type": "dis", "entity": "儿童白血病" } ] }, { "text": "儿童NHL治疗的进展是过去20年中最为成功疾病之一。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "儿童NHL" } ] }, { "text": "在美国和发达国家,恶性淋巴瘤(包括NHL及霍奇金病)是继白血病和脑肿瘤之后第三个常见的儿童恶性肿瘤,在小于20岁的少儿中占儿童恶性肿瘤的15%。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "恶性淋巴瘤" }, { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "NHL" }, { "start_idx": 21, "end_idx": 24, "type": "dis", "entity": "霍奇金病" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "白血病" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "脑肿瘤" }, { "start_idx": 43, "end_idx": 48, "type": "dis", "entity": "儿童恶性肿瘤" }, { "start_idx": 61, "end_idx": 66, "type": "dis", "entity": "儿童恶性肿瘤" } ] }, { "text": "15~19岁之间的儿童霍奇金病发病率几乎是NHL的两倍。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "儿童霍奇金病" } ] }, { "text": "NHL发病率因年龄有显著差异;5岁以内儿童NHL并不常见,仅占所有肿瘤的3%,NHL的发病率在整个人生中持续上升,在10岁以上的儿童肿瘤中占8%~9%。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "NHL" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "儿童NHL" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "肿瘤" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "NHL" }, { "start_idx": 64, "end_idx": 67, "type": "dis", "entity": "儿童肿瘤" } ] }, { "text": "自从对正常免疫系统有了更深的认识,免疫诊断技术也应用于NHL的诊断,NHL是淋巴细胞在分化成熟过程中某个时期细胞恶变的结果。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "bod", "entity": "免疫系统" }, { "start_idx": 17, "end_idx": 20, "type": "pro", "entity": "免疫诊断" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "NHL" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "NHL" }, { "start_idx": 38, "end_idx": 41, "type": "bod", "entity": "淋巴细胞" } ] }, { "text": "大约40%~50%儿童NHL都起自T细胞系;同等比例的病例则来自表达表面免疫球蛋白的B细胞肿瘤。", "entities": [ { "start_idx": 9, "end_idx": 13, "type": "dis", "entity": "儿童NHL" }, { "start_idx": 17, "end_idx": 20, "type": "bod", "entity": "T细胞系" }, { "start_idx": 34, "end_idx": 40, "type": "bod", "entity": "表面免疫球蛋白" }, { "start_idx": 42, "end_idx": 46, "type": "dis", "entity": "B细胞肿瘤" } ] }, { "text": "仅有不到10%的儿童NHL为缺乏表面免疫球蛋白的早前B细胞肿瘤。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "dis", "entity": "儿童NHL" }, { "start_idx": 16, "end_idx": 22, "type": "bod", "entity": "表面免疫球蛋白" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "B细胞肿瘤" } ] }, { "text": "NHL的现代分类方案包含了淋巴细胞性白血病,从而确认了NHL与ALL之间的紧密关系,并认识到从骨髓浸润的程度区别ALL和NHL并不合理。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "NHL" }, { "start_idx": 13, "end_idx": 20, "type": "dis", "entity": "淋巴细胞性白血病" }, { "start_idx": 27, "end_idx": 29, "type": "dis", "entity": "NHL" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "ALL" }, { "start_idx": 47, "end_idx": 48, "type": "bod", "entity": "骨髓" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "ALL" }, { "start_idx": 60, "end_idx": 62, "type": "dis", "entity": "NHL" } ] }, { "text": "增殖抗原Ki-67高表达。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "bod", "entity": "增殖抗原Ki-67" } ] }, { "text": "绝大多数Burkitt淋巴瘤存在非随机染色体易位[t(8;14)(q24;q32)],结果是8号染色体上的MYC原癌基因与位于14号染色体的免疫球蛋白重链基因融合。", "entities": [ { "start_idx": 4, "end_idx": 13, "type": "dis", "entity": "Burkitt淋巴瘤" }, { "start_idx": 19, "end_idx": 21, "type": "bod", "entity": "染色体" }, { "start_idx": 48, "end_idx": 50, "type": "bod", "entity": "染色体" }, { "start_idx": 53, "end_idx": 59, "type": "bod", "entity": "MYC原癌基因" }, { "start_idx": 66, "end_idx": 68, "type": "bod", "entity": "染色体" }, { "start_idx": 70, "end_idx": 78, "type": "bod", "entity": "免疫球蛋白重链基因" } ] }, { "text": "ALCL约占儿童NHL的8%~12%,或儿童大细胞淋巴瘤的30%~40%。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "ALCL" }, { "start_idx": 6, "end_idx": 10, "type": "dis", "entity": "儿童NHL" }, { "start_idx": 20, "end_idx": 27, "type": "dis", "entity": "儿童大细胞淋巴瘤" } ] }, { "text": "同一系列(T或B)的白血病或淋巴瘤在病理/细胞形态学、免疫学、生物遗传学均无法鉴别白血病还是淋巴瘤,但临床上前者骨髓原发,而后者骨髓外局部原发。", "entities": [ { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "白血病" }, { "start_idx": 41, "end_idx": 43, "type": "dis", "entity": "白血病" }, { "start_idx": 46, "end_idx": 48, "type": "dis", "entity": "淋巴瘤" }, { "start_idx": 56, "end_idx": 57, "type": "bod", "entity": "骨髓" }, { "start_idx": 64, "end_idx": 66, "type": "bod", "entity": "骨髓外" } ] }, { "text": "有时可表现为肠套叠、胃肠道出血、阑尾炎样表现、甚至少数病人发生肠穿孔等急腹症。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "肠套叠" }, { "start_idx": 10, "end_idx": 14, "type": "dis", "entity": "胃肠道出血" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "阑尾炎" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "肠穿孔" } ] }, { "text": "右下腹肿块较多见,需与炎性阑尾包块、阑尾炎鉴别。", "entities": [ { "start_idx": 2, "end_idx": 2, "type": "bod", "entity": "腹" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "炎性阑尾包块" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "阑尾炎" } ] }, { "text": "鼻咽部也是较多见的原发部位,可表现为鼻塞、打鼾、血性分泌物及吸气性呼吸困难。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "鼻咽部" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "鼻塞" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "打鼾" }, { "start_idx": 24, "end_idx": 36, "type": "sym", "entity": "血性分泌物及吸气性呼吸困难" } ] }, { "text": "病理(细胞)形态学满足NHL的基本诊断,免疫学已成为当今NHL诊断分型的必要手段,有条件时应尽可能进行相关亚型的分子生物学特征检测,如伯基特淋巴瘤常存在t(8;14)及其变异,而间变大细胞淋巴瘤常存在t(2;5)及其变异,使诊断更为可靠。", "entities": [ { "start_idx": 11, "end_idx": 13, "type": "dis", "entity": "NHL" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "NHL" }, { "start_idx": 53, "end_idx": 64, "type": "pro", "entity": "亚型的分子生物学特征检测" }, { "start_idx": 67, "end_idx": 72, "type": "dis", "entity": "伯基特淋巴瘤" }, { "start_idx": 89, "end_idx": 96, "type": "dis", "entity": "间变大细胞淋巴瘤" } ] }, { "text": "有胸膜腔积液或心包积液时可引流改善症状。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "bod", "entity": "胸膜腔积液" }, { "start_idx": 7, "end_idx": 10, "type": "bod", "entity": "心包积液" }, { "start_idx": 0, "end_idx": 10, "type": "sym", "entity": "有胸膜腔积液或心包积液" } ] }, { "text": "刚开始治疗时,输入液体多时可致原有的胸腹腔积液增多,必要时可留置引流。", "entities": [ { "start_idx": 18, "end_idx": 22, "type": "bod", "entity": "胸腹腔积液" } ] }, { "text": "与HD有关的其他染色体异常还有:常染色体2、10和13缺失症,11、22部分三体综合征等。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "HD" }, { "start_idx": 16, "end_idx": 29, "type": "dis", "entity": "常染色体2、10和13缺失症" }, { "start_idx": 31, "end_idx": 42, "type": "dis", "entity": "11、22部分三体综合征" } ] }, { "text": "同时伴发HD、小头畸形、精神发育延滞及面部异常(眼距过宽、球形角膜、眉毛过浓及耳前倾等),是一类与EDNRB基因305位点色氨酸突变有关的综合征;伴发HD的先天性通气不足综合征(原发性肺泡换气不足,Haddad综合征)可能与GDVF和RET基因突变的不完全外显率相关,文献报道161例原发性肺泡换气不足综合征患儿有27%伴发HD;Ⅱ型多发性内分泌瘤(MEN-Ⅱ)同时发生甲状腺髓样癌、嗜铬细胞瘤及多发性黏液瘤,偶有伴发HD。", "entities": [ { "start_idx": 4, "end_idx": 5, "type": "dis", "entity": "HD" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "小头畸形" }, { "start_idx": 12, "end_idx": 17, "type": "dis", "entity": "精神发育延滞" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "面部异常" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "眼距过宽" }, { "start_idx": 29, "end_idx": 32, "type": "dis", "entity": "球形角膜" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "眉毛过浓" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "耳前倾" }, { "start_idx": 48, "end_idx": 71, "type": "dis", "entity": "与EDNRB基因305位点色氨酸突变有关的综合征" }, { "start_idx": 75, "end_idx": 76, "type": "dis", "entity": "HD" }, { "start_idx": 78, "end_idx": 87, "type": "dis", "entity": "先天性通气不足综合征" }, { "start_idx": 99, "end_idx": 107, "type": "dis", "entity": "Haddad综合征" }, { "start_idx": 112, "end_idx": 115, "type": "bod", "entity": "GDVF" }, { "start_idx": 117, "end_idx": 121, "type": "bod", "entity": "RET基因" }, { "start_idx": 117, "end_idx": 123, "type": "sym", "entity": "RET基因突变" }, { "start_idx": 142, "end_idx": 153, "type": "dis", "entity": "原发性肺泡换气不足综合征" }, { "start_idx": 162, "end_idx": 163, "type": "dis", "entity": "HD" }, { "start_idx": 165, "end_idx": 173, "type": "dis", "entity": "Ⅱ型多发性内分泌瘤" }, { "start_idx": 175, "end_idx": 179, "type": "dis", "entity": "MEN-Ⅱ" }, { "start_idx": 185, "end_idx": 190, "type": "dis", "entity": "甲状腺髓样癌" }, { "start_idx": 192, "end_idx": 196, "type": "dis", "entity": "嗜铬细胞瘤" }, { "start_idx": 198, "end_idx": 203, "type": "dis", "entity": "多发性黏液瘤" }, { "start_idx": 209, "end_idx": 210, "type": "dis", "entity": "HD" } ] }, { "text": "大多数HD患儿在新生儿期即出现肠梗阻或严重便秘,主要表现为生后24小时内不能排出胎粪、腹胀和呕吐,某些婴儿甚至可出现完全性肠梗阻;也有患儿在生后几周至几个月内几乎没有症状,以后才出现顽固性便秘,尤其在调整饮食结构,如停母乳改为牛乳或添加固体食物时,便秘加重。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "dis", "entity": "HD" }, { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "严重便秘" }, { "start_idx": 36, "end_idx": 41, "type": "sym", "entity": "不能排出胎粪" }, { "start_idx": 43, "end_idx": 44, "type": "sym", "entity": "腹胀" }, { "start_idx": 46, "end_idx": 47, "type": "sym", "entity": "呕吐" }, { "start_idx": 58, "end_idx": 63, "type": "dis", "entity": "完全性肠梗阻" }, { "start_idx": 91, "end_idx": 95, "type": "sym", "entity": "顽固性便秘" } ] }, { "text": "那些确诊较晚的患儿,往往因长期便秘,导致严重腹胀,出现蛙状腹,触诊可及多个粪块,且多见小肠结肠炎。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "长期便秘" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "严重腹胀" }, { "start_idx": 25, "end_idx": 29, "type": "sym", "entity": "出现蛙状腹" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "多个粪块" } ] }, { "text": "小肠结肠炎是最严重的巨结肠并发症,可致全身性中毒,危及生命。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "小肠结肠炎" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "巨结肠" }, { "start_idx": 17, "end_idx": 23, "type": "sym", "entity": "可致全身性中毒" } ] }, { "text": "临床表现以发热、胆汁样呕吐、喷射样腹泻、腹胀、脱水和休克为特点。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "发热" }, { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "胆汁样呕吐" }, { "start_idx": 14, "end_idx": 18, "type": "sym", "entity": "喷射样腹泻" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "腹胀" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "脱水" }, { "start_idx": 26, "end_idx": 27, "type": "sym", "entity": "休克" } ] }, { "text": "节细胞缺乏段近端肠管黏液溃疡和缺血性坏死可致败血症、肠腔积气症和肠穿孔,有报告自发性穿孔率为3%,无神经节细胞肠段长度与穿孔发生率密切相关。", "entities": [ { "start_idx": 0, "end_idx": 13, "type": "sym", "entity": "节细胞缺乏段近端肠管黏液溃疡" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "缺血性坏死" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "败血症" }, { "start_idx": 26, "end_idx": 30, "type": "dis", "entity": "肠腔积气症" }, { "start_idx": 32, "end_idx": 34, "type": "dis", "entity": "肠穿孔" }, { "start_idx": 49, "end_idx": 56, "type": "bod", "entity": "无神经节细胞肠段" } ] }, { "text": "肛指检查可发现肛门括约肌痉挛与直肠壶腹部空虚感。", "entities": [ { "start_idx": 7, "end_idx": 13, "type": "sym", "entity": "肛门括约肌痉挛" }, { "start_idx": 15, "end_idx": 22, "type": "sym", "entity": "直肠壶腹部空虚感" } ] }, { "text": "肛指检查、插入肛管或体温计、或清洁灌肠时出现粪汁及大量气体喷出,应高度怀疑小肠结肠炎。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "肛指检查" }, { "start_idx": 5, "end_idx": 12, "type": "pro", "entity": "插入肛管或体温计" }, { "start_idx": 15, "end_idx": 18, "type": "pro", "entity": "清洁灌肠" }, { "start_idx": 20, "end_idx": 30, "type": "sym", "entity": "出现粪汁及大量气体喷出" } ] }, { "text": "通常排便排气后,短时间内症状缓解,但不久又再次出现腹胀。", "entities": [ { "start_idx": 8, "end_idx": 15, "type": "sym", "entity": "短时间内症状缓解" }, { "start_idx": 18, "end_idx": 26, "type": "sym", "entity": "不久又再次出现腹胀" } ] }, { "text": "确诊HD手段包括影像学检查、直肠肛管测压和直肠黏液活检组化分析等。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "HD" }, { "start_idx": 8, "end_idx": 12, "type": "pro", "entity": "影像学检查" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "直肠肛管测压" }, { "start_idx": 21, "end_idx": 30, "type": "pro", "entity": "直肠黏液活检组化分析" } ] }, { "text": "另外,新生儿败血症和脑损伤也可致胎粪排出延迟。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "新生儿败血症" }, { "start_idx": 10, "end_idx": 12, "type": "dis", "entity": "脑损伤" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "胎粪排出延迟" } ] }, { "text": "但是,如果发现新生儿或婴儿存在肠梗阻或小肠结肠炎症状,应首先怀疑HD及相关ENS疾病,行钡剂灌肠,直肠吸引活检以及直肠测压等检查,以明确诊断。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "小肠结肠炎" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "HD" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "ENS疾病" }, { "start_idx": 43, "end_idx": 47, "type": "pro", "entity": "行钡剂灌肠" }, { "start_idx": 49, "end_idx": 54, "type": "pro", "entity": "直肠吸引活检" }, { "start_idx": 57, "end_idx": 60, "type": "pro", "entity": "直肠测压" } ] }, { "text": "饮食习惯不当、心理障碍、药物性肠蠕动不良、代谢或内分泌疾病(如尿毒症或甲状腺功能减低)等均可能引起慢性便秘与肠梗阻;其他可致便秘的因素包括内源性肠神经功能疾病(糖尿病或家族性自主神经功能异常)、中枢神经系统病变和平滑肌功能失调等;收缩蛋白异常和结缔组织病(如硬皮病或皮肌炎)同样可致持久性便秘。", "entities": [ { "start_idx": 21, "end_idx": 28, "type": "dis", "entity": "代谢或内分泌疾病" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "尿毒症" }, { "start_idx": 35, "end_idx": 41, "type": "dis", "entity": "甲状腺功能减低" }, { "start_idx": 49, "end_idx": 52, "type": "dis", "entity": "慢性便秘" }, { "start_idx": 54, "end_idx": 56, "type": "dis", "entity": "肠梗阻" }, { "start_idx": 69, "end_idx": 78, "type": "dis", "entity": "内源性肠神经功能疾病" }, { "start_idx": 80, "end_idx": 82, "type": "dis", "entity": "糖尿病" }, { "start_idx": 84, "end_idx": 94, "type": "dis", "entity": "家族性自主神经功能异常" }, { "start_idx": 97, "end_idx": 104, "type": "dis", "entity": "中枢神经系统病变" }, { "start_idx": 106, "end_idx": 112, "type": "dis", "entity": "平滑肌功能失调" }, { "start_idx": 115, "end_idx": 120, "type": "dis", "entity": "收缩蛋白异常" }, { "start_idx": 122, "end_idx": 126, "type": "dis", "entity": "结缔组织病" }, { "start_idx": 129, "end_idx": 131, "type": "dis", "entity": "硬皮病" }, { "start_idx": 133, "end_idx": 135, "type": "dis", "entity": "皮肌炎" }, { "start_idx": 141, "end_idx": 145, "type": "sym", "entity": "持久性便秘" } ] }, { "text": "钡剂造影可发现小结肠,超声或静脉尿路造影可发现肾盂积水和巨膀胱,以此与HD鉴别。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "钡剂造影" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "小结肠" }, { "start_idx": 11, "end_idx": 12, "type": "pro", "entity": "超声" }, { "start_idx": 14, "end_idx": 19, "type": "pro", "entity": "静脉尿路造影" }, { "start_idx": 23, "end_idx": 26, "type": "dis", "entity": "肾盂积水" }, { "start_idx": 28, "end_idx": 30, "type": "dis", "entity": "巨膀胱" }, { "start_idx": 35, "end_idx": 36, "type": "dis", "entity": "HD" } ] }, { "text": "外周血的异常变化不能作为白血病的诊断依据,当临床怀疑白血病时,需及时作骨髓穿刺涂片以明确诊断。", "entities": [ { "start_idx": 12, "end_idx": 14, "type": "dis", "entity": "白血病" }, { "start_idx": 26, "end_idx": 28, "type": "dis", "entity": "白血病" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "骨髓穿刺" } ] }, { "text": "绝大多数白血病骨髓涂片表现为有核细胞增生活跃、明显活跃或极度活跃,5%~10%的急性白血病骨髓增生低下,称之为低增生性白血病。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "白血病" }, { "start_idx": 7, "end_idx": 10, "type": "ite", "entity": "骨髓涂片" }, { "start_idx": 40, "end_idx": 44, "type": "dis", "entity": "急性白血病" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "骨髓" }, { "start_idx": 55, "end_idx": 61, "type": "dis", "entity": "低增生性白血病" } ] }, { "text": "诊断ALL的主要依据是骨髓有核细胞中原始和幼稚淋巴细胞总和>30%,此时正常的红系、巨核细胞系、粒系常明显受抑甚至消失。", "entities": [ { "start_idx": 2, "end_idx": 4, "type": "dis", "entity": "ALL" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "骨髓有核细胞" }, { "start_idx": 18, "end_idx": 26, "type": "bod", "entity": "原始和幼稚淋巴细胞" }, { "start_idx": 39, "end_idx": 45, "type": "bod", "entity": "红系、巨核细胞" } ] }, { "text": "染色体>46条时称为超二倍体,而>50条的超二倍体者预后较好。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "bod", "entity": "染色体" } ] }, { "text": "推荐用大剂量甲氨蝶呤(HDMTX)和鞘内化疗进行CNSL预防。", "entities": [ { "start_idx": 6, "end_idx": 9, "type": "dru", "entity": "甲氨蝶呤" }, { "start_idx": 11, "end_idx": 15, "type": "dru", "entity": "HDMTX" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "鞘内化疗" }, { "start_idx": 24, "end_idx": 27, "type": "dis", "entity": "CNSL" } ] }, { "text": "(2)预防感染:注意食品及环境卫生,减少感染机会。", "entities": [ { "start_idx": 20, "end_idx": 21, "type": "dis", "entity": "感染" } ] }, { "text": "(3)应用L-ASP时宜低脂饮食,减少合并急性胰腺炎的机会;注意血白蛋白水平,明显低下时及时补充,以避免低蛋白血症加重感染。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "L-ASP" }, { "start_idx": 21, "end_idx": 25, "type": "dis", "entity": "急性胰腺炎" }, { "start_idx": 32, "end_idx": 35, "type": "bod", "entity": "血白蛋白" }, { "start_idx": 52, "end_idx": 56, "type": "dis", "entity": "低蛋白血症" } ] }, { "text": "自由基以及抗氧化剂缺乏在慢性胰腺炎的形成和发展中都起了很重要的作用。", "entities": [ { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "在钙化性胰腺炎,随着胰腺的钙化,疼痛减轻,但随之出现内、外分泌功能不足。", "entities": [ { "start_idx": 4, "end_idx": 6, "type": "dis", "entity": "胰腺炎" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "胰腺" } ] }, { "text": "严重时可出现脂肪泻,患儿粪便量显著增多,粪酸臭或恶臭。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "脂肪泻" } ] }, { "text": "遗传性胰腺炎起病年龄多见于5~10岁。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "胰腺炎" } ] }, { "text": "患者有严重的腹痛伴恶心、呕吐。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "腹痛" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "恶心" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "呕吐" } ] }, { "text": "生殖系统异常在男性表现为尿道背侧壁缺如。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "生殖系统异常" }, { "start_idx": 7, "end_idx": 18, "type": "sym", "entity": "男性表现为尿道背侧壁缺如" } ] }, { "text": "肛门直肠异常表现为会阴短、平,肛门前移紧靠尿生殖膈,可伴有肛门狭窄、直肠会阴瘘或直肠阴道瘘。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "肛门直肠异常" }, { "start_idx": 15, "end_idx": 24, "type": "sym", "entity": "肛门前移紧靠尿生殖膈" }, { "start_idx": 26, "end_idx": 32, "type": "sym", "entity": "可伴有肛门狭窄" }, { "start_idx": 34, "end_idx": 44, "type": "sym", "entity": "直肠会阴瘘或直肠阴道瘘" } ] }, { "text": "双侧髂骨截骨可使耻骨联合易于对合,可减小闭合腹壁缺损的张力;可把尿道放入骨盆环内,达到解剖复位;可使尿生殖膈及肛提肌靠拢,协助排尿控制。", "entities": [ { "start_idx": 0, "end_idx": 28, "type": "pro", "entity": "双侧髂骨截骨可使耻骨联合易于对合,可减小闭合腹壁缺损的张力" }, { "start_idx": 30, "end_idx": 46, "type": "pro", "entity": "可把尿道放入骨盆环内,达到解剖复位" }, { "start_idx": 48, "end_idx": 66, "type": "pro", "entity": "可使尿生殖膈及肛提肌靠拢,协助排尿控制" } ] }, { "text": "Reed对本病中的巨型多核细胞作了仔细的描述,并否认了这些细胞来自变异型结核的观点,以后这些畸形巨型细胞被命名为Reed-Sternberg细胞(R-S细胞)。", "entities": [ { "start_idx": 9, "end_idx": 14, "type": "bod", "entity": "巨型多核细胞" }, { "start_idx": 29, "end_idx": 30, "type": "bod", "entity": "细胞" }, { "start_idx": 46, "end_idx": 51, "type": "bod", "entity": "畸形巨型细胞" }, { "start_idx": 56, "end_idx": 71, "type": "bod", "entity": "Reed-Sternberg细胞" }, { "start_idx": 73, "end_idx": 77, "type": "bod", "entity": "R-S细胞" } ] }, { "text": "流行病学调查提示疱疹病毒6、巨细胞包涵体病毒、EB病毒感染可能与发病有关。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "mic", "entity": "疱疹病毒6" }, { "start_idx": 14, "end_idx": 21, "type": "mic", "entity": "巨细胞包涵体病毒" }, { "start_idx": 23, "end_idx": 26, "type": "mic", "entity": "EB病毒" } ] }, { "text": "原因不明38℃以上发热或周期性发热、6个月内体重减轻10%以上、大量盗汗时应想到本病。", "entities": [ { "start_idx": 15, "end_idx": 16, "type": "dis", "entity": "发热" }, { "start_idx": 18, "end_idx": 30, "type": "dis", "entity": "6个月内体重减轻10%以上" }, { "start_idx": 32, "end_idx": 35, "type": "dis", "entity": "大量盗汗" } ] }, { "text": "约2/3的病人就诊时有不同程度的纵隔浸润,引起咳嗽等气管、支气管受压症状。", "entities": [ { "start_idx": 11, "end_idx": 19, "type": "sym", "entity": "不同程度的纵隔浸润" } ] }, { "text": "目前,儿科ARDS在儿科危重病房平均病死率为50%~70%,治疗转归与机械通气治疗策略、时间、反应、肺部感染类型、年龄、是否控制多脏器系统功能衰竭、并发症处理效果等因素有关。", "entities": [ { "start_idx": 3, "end_idx": 8, "type": "dis", "entity": "儿科ARDS" }, { "start_idx": 10, "end_idx": 15, "type": "dep", "entity": "儿科危重病房" }, { "start_idx": 50, "end_idx": 53, "type": "dis", "entity": "肺部感染" }, { "start_idx": 64, "end_idx": 72, "type": "dis", "entity": "多脏器系统功能衰竭" } ] }, { "text": "对肺部的损伤可以是直接或间接的;直接损伤如细菌、真菌、病毒感染、溺水、有害气体或液体吸入、肺栓塞、挫伤等;近年,随ICU和外科救治技术应用,持续气道正压通气、心血管手术导致肺缺血再灌注等成为医源性直接肺损伤的重要原因。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "肺部" }, { "start_idx": 21, "end_idx": 22, "type": "mic", "entity": "细菌" }, { "start_idx": 24, "end_idx": 25, "type": "mic", "entity": "真菌" }, { "start_idx": 27, "end_idx": 30, "type": "dis", "entity": "病毒感染" }, { "start_idx": 32, "end_idx": 33, "type": "dis", "entity": "溺水" }, { "start_idx": 35, "end_idx": 43, "type": "sym", "entity": "有害气体或液体吸入" }, { "start_idx": 45, "end_idx": 45, "type": "bod", "entity": "肺" }, { "start_idx": 45, "end_idx": 47, "type": "sym", "entity": "肺栓塞" }, { "start_idx": 49, "end_idx": 50, "type": "dis", "entity": "挫伤" }, { "start_idx": 57, "end_idx": 59, "type": "dep", "entity": "ICU" }, { "start_idx": 61, "end_idx": 62, "type": "dep", "entity": "外科" }, { "start_idx": 70, "end_idx": 77, "type": "pro", "entity": "持续气道正压通气" }, { "start_idx": 79, "end_idx": 83, "type": "pro", "entity": "心血管手术" }, { "start_idx": 86, "end_idx": 88, "type": "dis", "entity": "肺缺血" }, { "start_idx": 95, "end_idx": 102, "type": "dis", "entity": "医源性直接肺损伤" } ] }, { "text": "ARDS/ALI的主要临床表现为急性呼吸困难、呼吸急促、严重低氧血症、胸片异常和肺静态顺应性降低。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "ARDS" }, { "start_idx": 5, "end_idx": 7, "type": "dis", "entity": "ALI" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "急性呼吸困难" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 30, "end_idx": 33, "type": "dis", "entity": "低氧血症" }, { "start_idx": 35, "end_idx": 36, "type": "ite", "entity": "胸片" }, { "start_idx": 35, "end_idx": 38, "type": "sym", "entity": "胸片异常" }, { "start_idx": 40, "end_idx": 40, "type": "bod", "entity": "肺" }, { "start_idx": 40, "end_idx": 47, "type": "sym", "entity": "肺静态顺应性降低" } ] }, { "text": "随后呼吸窘迫,吸气费力、发绀,常伴烦躁、焦虑不安,两肺广泛间质浸润,可伴奇静脉扩张、胸膜反应或有少量积液。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "呼吸窘迫" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "吸气费力" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "发绀" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "烦躁" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "焦虑不安" }, { "start_idx": 26, "end_idx": 26, "type": "bod", "entity": "肺" }, { "start_idx": 25, "end_idx": 32, "type": "sym", "entity": "两肺广泛间质浸润" }, { "start_idx": 36, "end_idx": 38, "type": "bod", "entity": "奇静脉" }, { "start_idx": 36, "end_idx": 40, "type": "sym", "entity": "奇静脉扩张" }, { "start_idx": 42, "end_idx": 43, "type": "bod", "entity": "胸膜" }, { "start_idx": 42, "end_idx": 45, "type": "sym", "entity": "胸膜反应" }, { "start_idx": 48, "end_idx": 51, "type": "sym", "entity": "少量积液" } ] }, { "text": "后期呼吸窘迫和发绀加重,肺部浸润可发展成“白肺”,二氧化碳潴留,心脏停搏或多器官衰竭。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "sym", "entity": "呼吸窘迫" }, { "start_idx": 7, "end_idx": 10, "type": "sym", "entity": "发绀加重" }, { "start_idx": 12, "end_idx": 13, "type": "bod", "entity": "肺部" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "白肺" }, { "start_idx": 12, "end_idx": 23, "type": "sym", "entity": "肺部浸润可发展成“白肺”" }, { "start_idx": 25, "end_idx": 28, "type": "bod", "entity": "二氧化碳" }, { "start_idx": 25, "end_idx": 30, "type": "sym", "entity": "二氧化碳潴留" }, { "start_idx": 32, "end_idx": 33, "type": "bod", "entity": "心脏" }, { "start_idx": 32, "end_idx": 35, "type": "sym", "entity": "心脏停搏" }, { "start_idx": 37, "end_idx": 41, "type": "sym", "entity": "多器官衰竭" } ] }, { "text": "1995年,全国危重急救学学术会议(庐山)提出我国ARDS分期诊断标准。", "entities": [ { "start_idx": 25, "end_idx": 28, "type": "dis", "entity": "ARDS" } ] }, { "text": "有些患儿可有不典型表现,如单侧肺弥漫性渗出或肺部影像学特征轻、临床低氧血症和感染症状明显,也可以考虑早期ARDS。", "entities": [ { "start_idx": 15, "end_idx": 15, "type": "bod", "entity": "肺" }, { "start_idx": 13, "end_idx": 20, "type": "sym", "entity": "单侧肺弥漫性渗出" }, { "start_idx": 22, "end_idx": 23, "type": "bod", "entity": "肺部" }, { "start_idx": 22, "end_idx": 29, "type": "sym", "entity": "肺部影像学特征轻" }, { "start_idx": 33, "end_idx": 36, "type": "dis", "entity": "低氧血症" }, { "start_idx": 38, "end_idx": 39, "type": "dis", "entity": "感染" }, { "start_idx": 31, "end_idx": 43, "type": "sym", "entity": "临床低氧血症和感染症状明显" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "ARDS" } ] }, { "text": "有条件时可以采用CT扫描,尤其对于发现肺泡萎陷、实变有帮助。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "pro", "entity": "CT扫描" }, { "start_idx": 19, "end_idx": 20, "type": "bod", "entity": "肺泡" }, { "start_idx": 19, "end_idx": 25, "type": "sym", "entity": "肺泡萎陷、实变" } ] }, { "text": "在ARDS的早期,首先应考虑通过面罩或气道插管,提供持续气道正压通气(CPAP)或压力支持通气(PSV)或容量支持通气(VSV)。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "dis", "entity": "ARDS" }, { "start_idx": 16, "end_idx": 22, "type": "pro", "entity": "面罩或气道插管" }, { "start_idx": 26, "end_idx": 33, "type": "pro", "entity": "持续气道正压通气" }, { "start_idx": 35, "end_idx": 38, "type": "pro", "entity": "CPAP" }, { "start_idx": 41, "end_idx": 46, "type": "pro", "entity": "压力支持通气" }, { "start_idx": 48, "end_idx": 50, "type": "pro", "entity": "PSV" }, { "start_idx": 53, "end_idx": 58, "type": "pro", "entity": "容量支持通气" }, { "start_idx": 60, "end_idx": 62, "type": "pro", "entity": "VSV" } ] }, { "text": "一般预计病情能够短期缓解的早期ALI/ARDS患者可考虑应用,合并免疫功能低下的ALI/ARDS患儿者早期可首先试用。", "entities": [ { "start_idx": 15, "end_idx": 17, "type": "dis", "entity": "ALI" }, { "start_idx": 19, "end_idx": 22, "type": "dis", "entity": "ARDS" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "ALI" }, { "start_idx": 44, "end_idx": 47, "type": "dis", "entity": "ARDS" } ] }, { "text": "某些气质特征与频发的梦魇有关。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "dis", "entity": "梦魇" } ] }, { "text": "相当大部分(20%~40%)的患者有精神分裂样人格、临界人格障碍、精神分裂样人格障碍或精神分裂症。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "精神分裂样人格" }, { "start_idx": 26, "end_idx": 31, "type": "dis", "entity": "临界人格障碍" }, { "start_idx": 33, "end_idx": 41, "type": "dis", "entity": "精神分裂样人格障碍" }, { "start_idx": 43, "end_idx": 47, "type": "dis", "entity": "精神分裂症" } ] }, { "text": "频繁发作的梦魇患者容易得精神病。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "dis", "entity": "梦魇" } ] }, { "text": "各种的紧张、特异的创伤均使梦魇的频率和严重度增加。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "dis", "entity": "梦魇" } ] }, { "text": "某些药物,包括L-多巴及类似的药物、β肾上腺素能阻滞剂、REM睡眠抑制药物的撤退可导致或增加梦魇的发生。", "entities": [ { "start_idx": 7, "end_idx": 10, "type": "dru", "entity": "L-多巴" }, { "start_idx": 18, "end_idx": 26, "type": "dru", "entity": "β肾上腺素能阻滞剂" }, { "start_idx": 46, "end_idx": 47, "type": "dis", "entity": "梦魇" } ] }, { "text": "大多数(10%~50%)儿童在3~6岁出现梦魇,其发作是逐渐的。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "梦魇" } ] }, { "text": "对于梦魇发作频繁或较严重者,首先寻找可能影响梦魇发生的一些因素,如各种紧张、焦虑情绪以及某些药物的应用等,有针对性地进行对因治疗。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "梦魇" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "梦魇" } ] }, { "text": "同时对2岁以下的儿童可以在发作的时候用肢体语言安抚,而年长儿则可以尽可能用安抚语言使其平静,并可以在孩子睡眠时为他开一盏灯或将他卧室的门打开,让他充分感觉自己是安全的。", "entities": [ { "start_idx": 18, "end_idx": 24, "type": "pro", "entity": "用肢体语言安抚" }, { "start_idx": 36, "end_idx": 44, "type": "pro", "entity": "用安抚语言使其平静" } ] }, { "text": "成人以下肢多见。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "bod", "entity": "下肢" } ] }, { "text": "术后化疗、放疗联合应用。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "化疗" }, { "start_idx": 5, "end_idx": 6, "type": "pro", "entity": "放疗" } ] }, { "text": "由于肿瘤无症状且生长缓慢,诊断多过晚,预后不良。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "肿瘤" } ] }, { "text": "主要由病毒引起,亦可由A组溶血性链球菌或支原体引起。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "mic", "entity": "病毒" }, { "start_idx": 11, "end_idx": 18, "type": "mic", "entity": "A组溶血性链球菌" }, { "start_idx": 20, "end_idx": 22, "type": "mic", "entity": "支原体" } ] }, { "text": "如患儿同时伴有结膜炎、鼻炎、咳嗽、声音嘶哑或溃疡、皮疹、腹泻等,常提示病毒性咽炎。", "entities": [ { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "结膜炎" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "鼻炎" }, { "start_idx": 14, "end_idx": 15, "type": "dis", "entity": "咳嗽" }, { "start_idx": 17, "end_idx": 20, "type": "sym", "entity": "声音嘶哑" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "溃疡" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "皮疹" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "腹泻" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "病毒性咽炎" } ] }, { "text": "年长儿常以头痛、腹痛、呕吐等非特异性症状起病,伴高热。", "entities": [ { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "头痛" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "腹痛" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "呕吐" }, { "start_idx": 24, "end_idx": 25, "type": "sym", "entity": "高热" } ] }, { "text": "数小时后出现咽痛。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "咽痛" } ] }, { "text": "后者轻重不一,重者可造成吞咽困难。", "entities": [ { "start_idx": 12, "end_idx": 15, "type": "sym", "entity": "吞咽困难" } ] }, { "text": "但咽部检查只有三分之一患儿发现典型扁桃体肿大、渗出和咽部充血。", "entities": [ { "start_idx": 1, "end_idx": 4, "type": "pro", "entity": "咽部检查" }, { "start_idx": 17, "end_idx": 19, "type": "bod", "entity": "扁桃体" }, { "start_idx": 17, "end_idx": 24, "type": "sym", "entity": "扁桃体肿大、渗出" }, { "start_idx": 26, "end_idx": 29, "type": "sym", "entity": "咽部充血" } ] }, { "text": "年长儿急性咽炎伴发热,但缺乏上呼吸道卡他症状,应首先考虑本病。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "急性咽炎" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "发热" }, { "start_idx": 14, "end_idx": 21, "type": "sym", "entity": "上呼吸道卡他症状" } ] }, { "text": "小儿脑积水多为先天性和炎症性病变所致。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "小儿脑积水" } ] }, { "text": "脑室系统与蛛网膜下腔畅通,由于脑脊液的分泌亢进或吸收障碍引起的脑积水称为交通性脑积水。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "bod", "entity": "蛛网膜下腔" }, { "start_idx": 15, "end_idx": 17, "type": "bod", "entity": "脑脊液" }, { "start_idx": 31, "end_idx": 33, "type": "dis", "entity": "脑积水" }, { "start_idx": 36, "end_idx": 41, "type": "dis", "entity": "交通性脑积水" } ] }, { "text": "脑积水还有其他很多分类方法,目前尚不统一。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "脑积水" } ] }, { "text": "按照致病原因,可分为先天性和后天性脑积水;按照发病的速度,可分为急性和慢性脑积水;按照颅内压的增高与否,分为高压力性脑积水和正常压力性脑积水;按照发生的部位不同,分为内脑积水和外脑积水。", "entities": [ { "start_idx": 10, "end_idx": 19, "type": "dis", "entity": "先天性和后天性脑积水" }, { "start_idx": 32, "end_idx": 39, "type": "dis", "entity": "急性和慢性脑积水" }, { "start_idx": 43, "end_idx": 45, "type": "ite", "entity": "颅内压" }, { "start_idx": 54, "end_idx": 60, "type": "dis", "entity": "高压力性脑积水" }, { "start_idx": 62, "end_idx": 69, "type": "dis", "entity": "正常压力性脑积水" }, { "start_idx": 83, "end_idx": 86, "type": "dis", "entity": "内脑积水" }, { "start_idx": 88, "end_idx": 91, "type": "dis", "entity": "外脑积水" } ] }, { "text": "4)Arnold-Chiari畸形:因小脑扁桃体、延髓及第四脑室疝入椎管内,使脑脊液循环受阻引起脑积水,常并发脊柱裂及脊膜膨出。", "entities": [ { "start_idx": 2, "end_idx": 16, "type": "dis", "entity": "Arnold-Chiari畸形" }, { "start_idx": 19, "end_idx": 23, "type": "sym", "entity": "小脑扁桃体" }, { "start_idx": 25, "end_idx": 26, "type": "sym", "entity": "延髓" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "第四脑室" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "椎管" }, { "start_idx": 39, "end_idx": 41, "type": "sym", "entity": "脑脊液" }, { "start_idx": 48, "end_idx": 50, "type": "dis", "entity": "脑积水" }, { "start_idx": 55, "end_idx": 57, "type": "dis", "entity": "脊柱裂" }, { "start_idx": 59, "end_idx": 62, "type": "dis", "entity": "脊膜膨出" } ] }, { "text": "5)扁平颅底:常合并Arnold-Chiari畸形,阻塞第四脑室出口或环池,引起脑积水。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "扁平颅底" }, { "start_idx": 10, "end_idx": 24, "type": "dis", "entity": "Arnold-Chiari畸形" }, { "start_idx": 28, "end_idx": 31, "type": "bod", "entity": "第四脑室" }, { "start_idx": 40, "end_idx": 42, "type": "dis", "entity": "脑积水" } ] }, { "text": "在囟门和颅缝已闭合的较大儿童脑积水,常表现为颅内压增高症(头痛、呕吐和视乳头水肿)。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "bod", "entity": "囟门" }, { "start_idx": 4, "end_idx": 5, "type": "bod", "entity": "颅缝" }, { "start_idx": 14, "end_idx": 16, "type": "dis", "entity": "脑积水" }, { "start_idx": 29, "end_idx": 39, "type": "sym", "entity": "头痛、呕吐和视乳头水肿" } ] }, { "text": "部分病人有抽搐发作。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "抽搐发作" } ] }, { "text": "如第三脑室前部和下视丘、漏斗部受累,可出现各种内分泌功能紊乱,例如青春早熟或落后和生长矮小等。", "entities": [ { "start_idx": 33, "end_idx": 44, "type": "sym", "entity": "青春早熟或落后和生长矮小" } ] }, { "text": "原发症征候,如松果体肿瘤的上视不能,小脑蚓部肿瘤的共济失调等。", "entities": [ { "start_idx": 7, "end_idx": 28, "type": "sym", "entity": "松果体肿瘤的上视不能,小脑蚓部肿瘤的共济失调" } ] }, { "text": "对于早期、发展缓慢或不适于手术治疗的脑积水患儿,则以药物治疗为主,可酌情选用脱水或利尿药。", "entities": [ { "start_idx": 13, "end_idx": 14, "type": "pro", "entity": "手术" }, { "start_idx": 18, "end_idx": 20, "type": "dis", "entity": "脑积水" }, { "start_idx": 38, "end_idx": 39, "type": "pro", "entity": "脱水" }, { "start_idx": 41, "end_idx": 43, "type": "dru", "entity": "利尿药" } ] }, { "text": "小儿脑积水分流术的开展,不仅增加了脑积水患儿的存活率,并使70%的患儿保持智力基本正常。", "entities": [ { "start_idx": 17, "end_idx": 19, "type": "dis", "entity": "脑积水" } ] }, { "text": "颅内脑脊液分流术适用于阻塞性脑积水,如侧脑室-小脑延髓池分流术以及第三脑室造瘘术等。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "颅内脑脊液分流术" }, { "start_idx": 11, "end_idx": 16, "type": "dis", "entity": "阻塞性脑积水" }, { "start_idx": 19, "end_idx": 30, "type": "pro", "entity": "侧脑室-小脑延髓池分流术" }, { "start_idx": 33, "end_idx": 39, "type": "pro", "entity": "第三脑室造瘘术" } ] }, { "text": "颅外脑脊液分流术适用于各型脑积水,方法很多,包括将脑脊液引流至心血管的手术及引流至其他脏器或体腔的手术,前者常用脑室-心房分流术,后者常用侧脑室-腹腔分流术。", "entities": [ { "start_idx": 0, "end_idx": 7, "type": "pro", "entity": "颅外脑脊液分流术" }, { "start_idx": 13, "end_idx": 15, "type": "dis", "entity": "脑积水" }, { "start_idx": 25, "end_idx": 27, "type": "bod", "entity": "脑脊液" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "心血管" }, { "start_idx": 35, "end_idx": 36, "type": "pro", "entity": "手术" }, { "start_idx": 43, "end_idx": 44, "type": "bod", "entity": "脏器" }, { "start_idx": 46, "end_idx": 47, "type": "bod", "entity": "体腔" }, { "start_idx": 49, "end_idx": 50, "type": "pro", "entity": "手术" }, { "start_idx": 56, "end_idx": 63, "type": "pro", "entity": "脑室-心房分流术" }, { "start_idx": 69, "end_idx": 77, "type": "pro", "entity": "侧脑室-腹腔分流术" } ] }, { "text": "分流术后尽管头围停止过快增长而进入正常曲线,仍需用头颅CT或MRI定期观察脑室大小及脑皮质厚度,以防持续存在的轻度颅内高压压迫脑皮质而造成智力发育障碍。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "pro", "entity": "分流术" }, { "start_idx": 25, "end_idx": 28, "type": "pro", "entity": "头颅CT" }, { "start_idx": 30, "end_idx": 32, "type": "pro", "entity": "MRI" }, { "start_idx": 37, "end_idx": 38, "type": "bod", "entity": "脑室" }, { "start_idx": 42, "end_idx": 44, "type": "bod", "entity": "脑皮质" }, { "start_idx": 63, "end_idx": 65, "type": "bod", "entity": "脑皮质" }, { "start_idx": 69, "end_idx": 74, "type": "sym", "entity": "智力发育障碍" } ] }, { "text": "有癫痫发作者,给予抗癫痫药物治疗。", "entities": [ { "start_idx": 1, "end_idx": 2, "type": "dis", "entity": "癫痫" }, { "start_idx": 9, "end_idx": 13, "type": "dru", "entity": "抗癫痫药物" } ] }, { "text": "经过手术治疗的脑积水患儿,存活率至少在90%,大约2/3智商正常或接近正常。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "pro", "entity": "手术" }, { "start_idx": 7, "end_idx": 9, "type": "dis", "entity": "脑积水" } ] }, { "text": "当然,脑积水患儿的神经功能障碍与脑积水的严重程度成正比,如大脑皮质厚度小于1cm,即使脑积水得到控制,也会有神经功能障碍及智力发育障碍。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脑积水" }, { "start_idx": 9, "end_idx": 14, "type": "dis", "entity": "神经功能障碍" }, { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "脑积水" }, { "start_idx": 29, "end_idx": 32, "type": "bod", "entity": "大脑皮质" }, { "start_idx": 43, "end_idx": 45, "type": "dis", "entity": "脑积水" }, { "start_idx": 54, "end_idx": 59, "type": "dis", "entity": "神经功能障碍" }, { "start_idx": 61, "end_idx": 66, "type": "dis", "entity": "智力发育障碍" } ] }, { "text": "WD儿童期的表现主要包括慢性肝炎、无症状性肝硬化或急性肝衰竭;青年期的表现以神经精神症状为主,包括肌张力障碍、震颤、性格改变及认知障碍。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "WD儿童期" }, { "start_idx": 12, "end_idx": 15, "type": "dis", "entity": "慢性肝炎" }, { "start_idx": 17, "end_idx": 23, "type": "dis", "entity": "无症状性肝硬化" }, { "start_idx": 25, "end_idx": 29, "type": "dis", "entity": "急性肝衰竭" }, { "start_idx": 49, "end_idx": 53, "type": "sym", "entity": "肌张力障碍" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "震颤" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "性格改变" }, { "start_idx": 63, "end_idx": 66, "type": "sym", "entity": "认知障碍" } ] }, { "text": "肝细胞癌变在WD中较为罕见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "肝细胞癌变" }, { "start_idx": 6, "end_idx": 7, "type": "dis", "entity": "WD" } ] }, { "text": "晚期有持久性、全身性扭转痉挛,病程进展较成年型明显加快。", "entities": [ { "start_idx": 3, "end_idx": 13, "type": "sym", "entity": "持久性、全身性扭转痉挛" } ] }, { "text": "精神症状包括行为异常、人格改变、抑郁和认知障碍等精神分裂症状。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "sym", "entity": "精神症状" }, { "start_idx": 6, "end_idx": 9, "type": "sym", "entity": "行为异常" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "人格改变" }, { "start_idx": 16, "end_idx": 22, "type": "sym", "entity": "抑郁和认知障碍" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "精神分裂症状" } ] }, { "text": "由于这些症状由铜沉积于中枢神经系统所致,因此我们必须对所有疑为WD的患者进行检查,早期诊断、早期治疗,从而使病情快速、全面恢复。", "entities": [ { "start_idx": 7, "end_idx": 7, "type": "bod", "entity": "铜" }, { "start_idx": 11, "end_idx": 16, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 31, "end_idx": 32, "type": "dis", "entity": "WD" } ] }, { "text": "大多数有神经系统症状的儿童有K-F环,但以肝损为表现的儿童可以无K-F环。", "entities": [ { "start_idx": 4, "end_idx": 9, "type": "sym", "entity": "神经系统症状" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "K-F环" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "肝损" }, { "start_idx": 31, "end_idx": 35, "type": "sym", "entity": "无K-F环" } ] }, { "text": "WD基因的产物(ATP7B)是一种含有1411个氨基酸的铜转运P型ATP酶(即WD蛋白)。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "bod", "entity": "WD基因" }, { "start_idx": 8, "end_idx": 12, "type": "bod", "entity": "ATP7B" }, { "start_idx": 24, "end_idx": 26, "type": "bod", "entity": "氨基酸" }, { "start_idx": 28, "end_idx": 36, "type": "bod", "entity": "铜转运P型ATP酶" }, { "start_idx": 39, "end_idx": 42, "type": "bod", "entity": "WD蛋白" } ] }, { "text": "曲恩汀的不良反应小,主要毒性是骨髓抑制、肾毒性、皮肤黏膜病变及缺铁性贫血。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dru", "entity": "曲恩汀" }, { "start_idx": 15, "end_idx": 18, "type": "dis", "entity": "骨髓抑制" }, { "start_idx": 20, "end_idx": 22, "type": "dis", "entity": "肾毒性" }, { "start_idx": 24, "end_idx": 29, "type": "dis", "entity": "皮肤黏膜病变" }, { "start_idx": 31, "end_idx": 35, "type": "dis", "entity": "缺铁性贫血" } ] }, { "text": "其他排铜药物:二巯基丙醇(因副作用大已少用)、二巯基丁二酸钠、二巯基丁二酸胶囊以及二巯基丙磺酸钠等重金属离子鳌合剂。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "排铜药物" }, { "start_idx": 7, "end_idx": 11, "type": "dru", "entity": "二巯基丙醇" }, { "start_idx": 23, "end_idx": 29, "type": "dru", "entity": "二巯基丁二酸钠" }, { "start_idx": 31, "end_idx": 38, "type": "dru", "entity": "二巯基丁二酸胶囊" }, { "start_idx": 41, "end_idx": 47, "type": "dru", "entity": "二巯基丙磺酸钠" }, { "start_idx": 49, "end_idx": 56, "type": "dru", "entity": "重金属离子鳌合剂" } ] }, { "text": "②肌张力障碍,可选用苯海索。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "肌张力障碍" }, { "start_idx": 10, "end_idx": 12, "type": "dru", "entity": "苯海索" } ] }, { "text": "③舞蹈样动作和手足徐动症可选用地西泮,也可选用小量氟哌啶醇合用苯海索。", "entities": [ { "start_idx": 1, "end_idx": 5, "type": "sym", "entity": "舞蹈样动作" }, { "start_idx": 7, "end_idx": 11, "type": "dis", "entity": "手足徐动症" }, { "start_idx": 15, "end_idx": 17, "type": "dru", "entity": "地西泮" }, { "start_idx": 25, "end_idx": 28, "type": "dru", "entity": "氟哌啶醇" }, { "start_idx": 31, "end_idx": 33, "type": "dru", "entity": "苯海索" } ] }, { "text": "扭转痉挛、强直或痉挛性斜颈为主者,除上述药物外,还可选用氯硝西泮或巴氯芬5mg,每日2次开始,逐渐加至10~20mg,每日3次。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "dis", "entity": "扭转痉挛" }, { "start_idx": 5, "end_idx": 12, "type": "dis", "entity": "强直或痉挛性斜颈" }, { "start_idx": 28, "end_idx": 31, "type": "dru", "entity": "氯硝西泮" }, { "start_idx": 33, "end_idx": 35, "type": "dru", "entity": "巴氯芬" } ] }, { "text": "④精神症状可选用奋乃静或利培酮。", "entities": [ { "start_idx": 8, "end_idx": 10, "type": "dru", "entity": "奋乃静" }, { "start_idx": 12, "end_idx": 14, "type": "dru", "entity": "利培酮" } ] }, { "text": "⑤绝大多数患者需要长期护肝治疗。", "entities": [ { "start_idx": 11, "end_idx": 14, "type": "pro", "entity": "护肝治疗" } ] }, { "text": "病原体可侵犯体内各种器官,但病理改变主要见于中枢神经系统,通常表现为进行性加重的慢性或亚急性脑病。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "mic", "entity": "病原体" }, { "start_idx": 10, "end_idx": 11, "type": "bod", "entity": "器官" }, { "start_idx": 22, "end_idx": 27, "type": "bod", "entity": "中枢神经系统" }, { "start_idx": 34, "end_idx": 47, "type": "dis", "entity": "进行性加重的慢性或亚急性脑病" } ] }, { "text": "自1969年首次证实本病系麻疹病毒所致以来,经大量研究目前临床表现也已得到充分的认识,已经确定麻疹病毒是SSPE的病原,但麻疹病毒在脑内持续存在的原因尚不完全清楚。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 47, "end_idx": 50, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 52, "end_idx": 55, "type": "dis", "entity": "SSPE" }, { "start_idx": 61, "end_idx": 64, "type": "mic", "entity": "麻疹病毒" }, { "start_idx": 66, "end_idx": 66, "type": "bod", "entity": "脑" } ] }, { "text": "由于引起SSPE的并不是完整的麻疹病毒颗粒,所以一般不具有传染性。", "entities": [ { "start_idx": 4, "end_idx": 7, "type": "dis", "entity": "SSPE" }, { "start_idx": 15, "end_idx": 18, "type": "mic", "entity": "麻疹病毒" } ] }, { "text": "由于损毁性病变而引起的特征性锥体外系症状,如舞蹈手足徐动症等开始出现。", "entities": [ { "start_idx": 14, "end_idx": 19, "type": "sym", "entity": "锥体外系症状" }, { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "舞蹈手足徐动症" } ] }, { "text": "出现明显的长束性感觉和运动障碍,智力明显恶化,提示大脑皮层灰质开始了破坏性变化。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "sym", "entity": "出现明显的长束性感觉" }, { "start_idx": 11, "end_idx": 14, "type": "sym", "entity": "运动障碍" }, { "start_idx": 16, "end_idx": 21, "type": "sym", "entity": "智力明显恶化" } ] }, { "text": "儿童组织细胞增生症是一组临床表现差异极大、发病率又较低的疾病。", "entities": [ { "start_idx": 0, "end_idx": 8, "type": "dis", "entity": "儿童组织细胞增生症" } ] }, { "text": "临床诊断及治疗较为困难,其中大部分为非肿瘤性疾病,但习惯上这一组疾病由血液/肿瘤科医生诊治。", "entities": [ { "start_idx": 18, "end_idx": 23, "type": "dis", "entity": "非肿瘤性疾病" }, { "start_idx": 35, "end_idx": 40, "type": "dep", "entity": "血液/肿瘤科" } ] }, { "text": "组织细胞增生症Ⅱ型中的组织细胞主要为噬血性巨噬细胞,又称噬血性组织细胞增生症,包括家族性(原发性)和继发性两大类。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "组织细胞增生症" }, { "start_idx": 11, "end_idx": 14, "type": "bod", "entity": "组织细胞" }, { "start_idx": 18, "end_idx": 24, "type": "bod", "entity": "噬血性巨噬细胞" }, { "start_idx": 28, "end_idx": 37, "type": "dis", "entity": "噬血性组织细胞增生症" } ] }, { "text": "1953年Lichtenstein将一组组织细胞浸润为主的疾病命名为组织细胞增生症X,X的含义为“不知原因”。", "entities": [ { "start_idx": 20, "end_idx": 23, "type": "bod", "entity": "组织细胞" }, { "start_idx": 34, "end_idx": 41, "type": "dis", "entity": "组织细胞增生症X" } ] }, { "text": "肺部受累重者可见气喘和发绀。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "肺部" }, { "start_idx": 8, "end_idx": 9, "type": "sym", "entity": "气喘" }, { "start_idx": 11, "end_idx": 12, "type": "sym", "entity": "发绀" } ] }, { "text": "受累器官少的、即使年龄较小亦有自然痊愈的可能,尤其是单纯皮肤浸润,对化疗反应好的预后亦佳。", "entities": [ { "start_idx": 28, "end_idx": 29, "type": "bod", "entity": "皮肤" } ] }, { "text": "婴儿型脚气病多发生于数个月的婴儿,发病急、突然,较成人型难以捉摸,可出现多种临床表现,但以心血管症状占优势。", "entities": [ { "start_idx": 0, "end_idx": 5, "type": "dis", "entity": "婴儿型脚气病" }, { "start_idx": 45, "end_idx": 47, "type": "bod", "entity": "心血管" } ] }, { "text": "脑型表现主要为发作型哭叫似腹痛状,烦躁不安,前囟饱满,头后仰。", "entities": [ { "start_idx": 7, "end_idx": 29, "type": "sym", "entity": "发作型哭叫似腹痛状,烦躁不安,前囟饱满,头后仰" } ] }, { "text": "严重者可发生脑充血、颅内高压、昏迷而死亡。", "entities": [ { "start_idx": 6, "end_idx": 8, "type": "dis", "entity": "脑充血" }, { "start_idx": 10, "end_idx": 13, "type": "dis", "entity": "颅内高压" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "昏迷" }, { "start_idx": 18, "end_idx": 19, "type": "sym", "entity": "死亡" } ] }, { "text": "神经炎主要表现为周围性瘫痪,早期表现为四肢无力,其后症状加重,同时足趾的背屈运动受限。", "entities": [ { "start_idx": 8, "end_idx": 12, "type": "sym", "entity": "周围性瘫痪" }, { "start_idx": 19, "end_idx": 22, "type": "sym", "entity": "四肢无力" }, { "start_idx": 33, "end_idx": 34, "type": "bod", "entity": "足趾" }, { "start_idx": 33, "end_idx": 41, "type": "sym", "entity": "足趾的背屈运动受限" } ] }, { "text": "软腭反射障碍,吃奶出现呛咳,吞咽困难。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "bod", "entity": "软腭" }, { "start_idx": 0, "end_idx": 17, "type": "sym", "entity": "软腭反射障碍,吃奶出现呛咳,吞咽困难" } ] }, { "text": "当病变自肺播散到中枢神经系统时才被检出。", "entities": [ { "start_idx": 4, "end_idx": 4, "type": "bod", "entity": "肺" }, { "start_idx": 8, "end_idx": 13, "type": "bod", "entity": "中枢神经系统" } ] }, { "text": "体格检查较少出现典型的肺炎体征。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 11, "end_idx": 12, "type": "dis", "entity": "肺炎" } ] }, { "text": "隐球菌感染发生在肺门或纵隔淋巴结的不常见。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "隐球菌感染" }, { "start_idx": 8, "end_idx": 9, "type": "bod", "entity": "肺门" }, { "start_idx": 11, "end_idx": 15, "type": "bod", "entity": "纵隔淋巴结" } ] }, { "text": "尽管隐球菌脑膜炎可急性发作,但更常见的是出现无症状期,病程缓慢,以后逐渐出现症状。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "隐球菌脑膜炎" }, { "start_idx": 27, "end_idx": 30, "type": "sym", "entity": "病程缓慢" } ] }, { "text": "可能有脑膜炎、脑膜脑炎、脑或脊髓肉芽肿或占位性病变的特点。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "dis", "entity": "脑膜炎" }, { "start_idx": 7, "end_idx": 10, "type": "dis", "entity": "脑膜脑炎" }, { "start_idx": 12, "end_idx": 18, "type": "dis", "entity": "脑或脊髓肉芽肿" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "占位性病变" } ] }, { "text": "接受肾上腺皮质类固醇和化疗的恶性肿瘤患者可能病情进展较快。", "entities": [ { "start_idx": 2, "end_idx": 9, "type": "dru", "entity": "肾上腺皮质类固醇" }, { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "恶性肿瘤" } ] }, { "text": "最常见的症状有发热,热型不规则;头痛,为额颞部头痛,偶有枕部疼痛,初为间歇性,逐渐发展为持续性并加重;伴恶心和呕吐、精神状态的改变、惊厥、颅压增高的体征;出现脑膜刺激征。", "entities": [ { "start_idx": 7, "end_idx": 14, "type": "sym", "entity": "发热,热型不规则" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "头痛" }, { "start_idx": 20, "end_idx": 24, "type": "dis", "entity": "额颞部头痛" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "枕部疼痛" }, { "start_idx": 33, "end_idx": 49, "type": "sym", "entity": "初为间歇性,逐渐发展为持续性并加重" }, { "start_idx": 52, "end_idx": 53, "type": "sym", "entity": "恶心" }, { "start_idx": 55, "end_idx": 56, "type": "sym", "entity": "呕吐" }, { "start_idx": 58, "end_idx": 64, "type": "sym", "entity": "精神状态的改变" }, { "start_idx": 66, "end_idx": 67, "type": "sym", "entity": "惊厥" }, { "start_idx": 69, "end_idx": 72, "type": "sym", "entity": "颅压增高" } ] }, { "text": "其疗效与两性霉素B相同而毒性较小,可与两性霉素B联合应用,是治疗隐球菌性脑膜炎的金标准治疗方案,可减少隐球菌的耐药性。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 19, "end_idx": 23, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 32, "end_idx": 38, "type": "dis", "entity": "隐球菌性脑膜炎" }, { "start_idx": 51, "end_idx": 53, "type": "mic", "entity": "隐球菌" } ] }, { "text": "两性霉素B的剂量较常用剂量减少1/3~1/2,一般疗程为6周或3个月以上。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dru", "entity": "两性霉素B" } ] }, { "text": "病初阶段与两性霉素B联合应用能更快使脑脊液转阴,并减少两性霉素B的用量和毒副作用,是治疗中枢神经系统隐球菌病的首选药物之一。", "entities": [ { "start_idx": 5, "end_idx": 9, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 18, "end_idx": 20, "type": "bod", "entity": "脑脊液" }, { "start_idx": 27, "end_idx": 31, "type": "dru", "entity": "两性霉素B" }, { "start_idx": 44, "end_idx": 53, "type": "dis", "entity": "中枢神经系统隐球菌病" } ] }, { "text": "艾滋病患者并发隐球菌性脑膜炎者,预后极差。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "艾滋病" }, { "start_idx": 7, "end_idx": 13, "type": "dis", "entity": "隐球菌性脑膜炎" } ] }, { "text": "完全性肺静脉异位连接病人除了肺静脉开口异常外,还包括心房水平的右向左分流,如卵圆孔未闭、房间隔缺损。", "entities": [ { "start_idx": 0, "end_idx": 9, "type": "dis", "entity": "完全性肺静脉异位连接" }, { "start_idx": 14, "end_idx": 16, "type": "bod", "entity": "肺静脉" }, { "start_idx": 26, "end_idx": 27, "type": "bod", "entity": "心房" }, { "start_idx": 44, "end_idx": 48, "type": "dis", "entity": "房间隔缺损" } ] }, { "text": "在出生后数周,由于肺血流增多,开始出现心功能不全症状,如喂养困难、呼吸急促、体重不增、反复肺部感染。", "entities": [ { "start_idx": 9, "end_idx": 11, "type": "bod", "entity": "肺血流" }, { "start_idx": 19, "end_idx": 23, "type": "dis", "entity": "心功能不全" }, { "start_idx": 28, "end_idx": 31, "type": "sym", "entity": "喂养困难" }, { "start_idx": 33, "end_idx": 36, "type": "sym", "entity": "呼吸急促" }, { "start_idx": 38, "end_idx": 41, "type": "sym", "entity": "体重不增" }, { "start_idx": 45, "end_idx": 46, "type": "bod", "entity": "肺部" }, { "start_idx": 43, "end_idx": 48, "type": "sym", "entity": "反复肺部感染" } ] }, { "text": "体格检查示小儿瘦小、易激惹。", "entities": [ { "start_idx": 0, "end_idx": 3, "type": "pro", "entity": "体格检查" }, { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "小儿瘦小" }, { "start_idx": 10, "end_idx": 12, "type": "sym", "entity": "易激惹" } ] }, { "text": "心下型患者,吮吸、哭吵用力时腹内压升高,肺静脉血流受阻加剧,青紫及呼吸困难加重。", "entities": [ { "start_idx": 6, "end_idx": 18, "type": "sym", "entity": "吮吸、哭吵用力时腹内压升高" }, { "start_idx": 30, "end_idx": 38, "type": "sym", "entity": "青紫及呼吸困难加重" } ] }, { "text": "合并肺静脉梗阻或存在限制性心房水平交通者,甚至可于出生后数周内夭折。", "entities": [ { "start_idx": 2, "end_idx": 6, "type": "dis", "entity": "肺静脉梗阻" }, { "start_idx": 13, "end_idx": 14, "type": "bod", "entity": "心房" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "夭折" } ] }, { "text": "手术后期疗效大多良好。", "entities": [ { "start_idx": 0, "end_idx": 1, "type": "pro", "entity": "手术" } ] }, { "text": "目前采用较多的是4型分类:①运动障碍样型;②反流样型;③溃疡样型;④非特异型。", "entities": [ { "start_idx": 14, "end_idx": 17, "type": "dis", "entity": "运动障碍" }, { "start_idx": 22, "end_idx": 23, "type": "dis", "entity": "反流" }, { "start_idx": 28, "end_idx": 29, "type": "dis", "entity": "溃疡" } ] }, { "text": "有些患儿根据医生诊断得知无病及检查结果亦属正常后,可通过改变生活方式与调整食物种类来预防。", "entities": [ { "start_idx": 28, "end_idx": 33, "type": "pro", "entity": "改变生活方式" }, { "start_idx": 35, "end_idx": 40, "type": "pro", "entity": "调整食物种类" } ] }, { "text": "经过初步检查之后,应与患者讨论鉴别诊断,包括功能性消化不良的可能。", "entities": [ { "start_idx": 22, "end_idx": 28, "type": "dis", "entity": "功能性消化不良" } ] }, { "text": "主要有西咪替丁、雷尼替丁及法莫替丁等。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dru", "entity": "西咪替丁" }, { "start_idx": 8, "end_idx": 11, "type": "dru", "entity": "雷尼替丁" }, { "start_idx": 13, "end_idx": 16, "type": "dru", "entity": "法莫替丁" } ] }, { "text": "我国儿童癫痫年发病率的报道较少,多数儿童病例在10岁之前发病,其中生后头1年发病率最高,随着年龄的增长,发病率有所下降。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dis", "entity": "儿童癫痫" } ] }, { "text": "原发性癫痫可表现为全身性发作或部分性发作,但全身性癫痫的遗传性因素高于部分性癫痫。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "原发性癫痫" }, { "start_idx": 9, "end_idx": 13, "type": "sym", "entity": "全身性发作" }, { "start_idx": 15, "end_idx": 19, "type": "sym", "entity": "部分性发作" }, { "start_idx": 22, "end_idx": 26, "type": "dis", "entity": "全身性癫痫" }, { "start_idx": 35, "end_idx": 39, "type": "dis", "entity": "部分性癫痫" } ] }, { "text": "小儿症状性癫痫常见病因有脑发育异常如脑回畸形及灰质异位;各种原因导致的脑损伤如围生期损伤、中枢神经系统感染或后遗症、头部外伤、中毒、水电解质紊乱、内分泌功能紊乱、低血糖以及维生素缺乏等;脑血管病变如颅内出血、血管内膜炎、血栓、梗死和血管畸形等;以及其他代谢性、脑变性和全身性疾病;另外一些与遗传有关的代谢性疾病及综合征常合并癫痫如神经皮肤综合征(常见结节性硬化、多发性神经纤维瘤病和脑三叉神经血管瘤病)、Rett综合征、Angelman综合征、线粒体脑病以及假性甲状旁腺功能低下等均可有癫痫发作。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "小儿症状性癫痫" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "脑" }, { "start_idx": 12, "end_idx": 16, "type": "sym", "entity": "脑发育异常" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "脑回畸形" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "灰质异位" }, { "start_idx": 35, "end_idx": 37, "type": "sym", "entity": "脑损伤" }, { "start_idx": 39, "end_idx": 43, "type": "sym", "entity": "围生期损伤" }, { "start_idx": 45, "end_idx": 52, "type": "sym", "entity": "中枢神经系统感染" }, { "start_idx": 54, "end_idx": 56, "type": "sym", "entity": "后遗症" }, { "start_idx": 58, "end_idx": 61, "type": "sym", "entity": "头部外伤" }, { "start_idx": 63, "end_idx": 64, "type": "sym", "entity": "中毒" }, { "start_idx": 66, "end_idx": 71, "type": "sym", "entity": "水电解质紊乱" }, { "start_idx": 73, "end_idx": 79, "type": "sym", "entity": "内分泌功能紊乱" }, { "start_idx": 81, "end_idx": 83, "type": "sym", "entity": "低血糖" }, { "start_idx": 86, "end_idx": 90, "type": "sym", "entity": "维生素缺乏" }, { "start_idx": 93, "end_idx": 97, "type": "dis", "entity": "脑血管病变" }, { "start_idx": 99, "end_idx": 102, "type": "sym", "entity": "颅内出血" }, { "start_idx": 104, "end_idx": 108, "type": "dis", "entity": "血管内膜炎" }, { "start_idx": 110, "end_idx": 111, "type": "dis", "entity": "血栓" }, { "start_idx": 113, "end_idx": 114, "type": "dis", "entity": "梗死" }, { "start_idx": 116, "end_idx": 119, "type": "dis", "entity": "血管畸形" }, { "start_idx": 126, "end_idx": 138, "type": "dis", "entity": "代谢性、脑变性和全身性疾病" }, { "start_idx": 150, "end_idx": 154, "type": "dis", "entity": "代谢性疾病" }, { "start_idx": 156, "end_idx": 158, "type": "dis", "entity": "综合征" }, { "start_idx": 162, "end_idx": 163, "type": "dis", "entity": "癫痫" }, { "start_idx": 165, "end_idx": 171, "type": "dis", "entity": "神经皮肤综合征" }, { "start_idx": 175, "end_idx": 179, "type": "dis", "entity": "结节性硬化" }, { "start_idx": 181, "end_idx": 189, "type": "dis", "entity": "多发性神经纤维瘤病" }, { "start_idx": 191, "end_idx": 199, "type": "dis", "entity": "脑三叉神经血管瘤病" }, { "start_idx": 202, "end_idx": 208, "type": "dis", "entity": "Rett综合征" }, { "start_idx": 210, "end_idx": 220, "type": "dis", "entity": "Angelman综合征" }, { "start_idx": 222, "end_idx": 226, "type": "dis", "entity": "线粒体脑病" }, { "start_idx": 229, "end_idx": 238, "type": "sym", "entity": "假性甲状旁腺功能低下" }, { "start_idx": 243, "end_idx": 244, "type": "dis", "entity": "癫痫" } ] }, { "text": "③自主神经症状:呼吸暂停、呼吸节律改变、发绀、面色苍白、潮红、流涎及呕吐。", "entities": [ { "start_idx": 8, "end_idx": 11, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 13, "end_idx": 18, "type": "sym", "entity": "呼吸节律改变" }, { "start_idx": 20, "end_idx": 21, "type": "sym", "entity": "发绀" }, { "start_idx": 23, "end_idx": 26, "type": "sym", "entity": "面色苍白" }, { "start_idx": 28, "end_idx": 29, "type": "sym", "entity": "潮红" }, { "start_idx": 31, "end_idx": 32, "type": "sym", "entity": "流涎" }, { "start_idx": 34, "end_idx": 35, "type": "sym", "entity": "呕吐" } ] }, { "text": "④惊厥性症状:表现为眨眼、眼球震颤或口角抽动、扭转或姿势性强直、局部肢体轻微阵挛,与年长儿相比,发作较轻。", "entities": [ { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "眨眼" }, { "start_idx": 13, "end_idx": 16, "type": "sym", "entity": "眼球震颤" }, { "start_idx": 18, "end_idx": 21, "type": "sym", "entity": "口角抽动" }, { "start_idx": 23, "end_idx": 24, "type": "sym", "entity": "扭转" }, { "start_idx": 26, "end_idx": 30, "type": "sym", "entity": "姿势性强直" }, { "start_idx": 32, "end_idx": 39, "type": "sym", "entity": "局部肢体轻微阵挛" } ] }, { "text": "有时表现为轴性强直,头、颈后仰,躯干极度伸展呈角弓反张;有时表现为“球样强直发作”,低头、弯腰、双上臂举起及屈肘,持续2~3秒,站立时发作会摔倒;有时轻微的强直发作,表现为眼球上转、眨眼或眼球震颤,称为“强直性眼球震颤”。", "entities": [ { "start_idx": 5, "end_idx": 8, "type": "sym", "entity": "轴性强直" }, { "start_idx": 10, "end_idx": 10, "type": "bod", "entity": "头" }, { "start_idx": 12, "end_idx": 12, "type": "bod", "entity": "颈" }, { "start_idx": 10, "end_idx": 14, "type": "sym", "entity": "头、颈后仰" }, { "start_idx": 16, "end_idx": 26, "type": "sym", "entity": "躯干极度伸展呈角弓反张" }, { "start_idx": 33, "end_idx": 40, "type": "sym", "entity": "“球样强直发作”" }, { "start_idx": 70, "end_idx": 71, "type": "sym", "entity": "摔倒" }, { "start_idx": 86, "end_idx": 89, "type": "sym", "entity": "眼球上转" }, { "start_idx": 91, "end_idx": 92, "type": "sym", "entity": "眨眼" }, { "start_idx": 94, "end_idx": 97, "type": "sym", "entity": "眼球震颤" }, { "start_idx": 101, "end_idx": 109, "type": "sym", "entity": "“强直性眼球震颤”" } ] }, { "text": "预后良好,多于1~2个月内消失,大约10%~14%小儿转为其他类型癫痫。", "entities": [ { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "癫痫" } ] }, { "text": "惊厥多表现为阵挛发作,有时伴有呼吸暂停,发作频繁,有时可呈癫痫持续状态。", "entities": [ { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "阵挛" }, { "start_idx": 15, "end_idx": 18, "type": "sym", "entity": "呼吸暂停" }, { "start_idx": 20, "end_idx": 23, "type": "sym", "entity": "发作频繁" }, { "start_idx": 28, "end_idx": 34, "type": "sym", "entity": "呈癫痫持续状态" } ] }, { "text": "多在3~10个月发病;临床以频繁的强直痉挛发作为特征,可分为屈曲型、伸展型及混合型。", "entities": [ { "start_idx": 14, "end_idx": 22, "type": "sym", "entity": "频繁的强直痉挛发作" }, { "start_idx": 30, "end_idx": 32, "type": "dis", "entity": "屈曲型" }, { "start_idx": 34, "end_idx": 36, "type": "dis", "entity": "伸展型" }, { "start_idx": 38, "end_idx": 40, "type": "dis", "entity": "混合型" } ] }, { "text": "屈曲型表现为点头、弯腰、屈肘及屈髋等动作。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "屈曲型" }, { "start_idx": 6, "end_idx": 7, "type": "sym", "entity": "点头" }, { "start_idx": 9, "end_idx": 10, "type": "sym", "entity": "弯腰" }, { "start_idx": 12, "end_idx": 13, "type": "sym", "entity": "屈肘" }, { "start_idx": 15, "end_idx": 16, "type": "sym", "entity": "屈髋" } ] }, { "text": "伸展型表现为头后仰、两臂伸直以及伸膝等动作。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "dis", "entity": "伸展型" }, { "start_idx": 6, "end_idx": 8, "type": "sym", "entity": "头后仰" }, { "start_idx": 10, "end_idx": 13, "type": "sym", "entity": "两臂伸直" }, { "start_idx": 16, "end_idx": 17, "type": "sym", "entity": "伸膝" } ] }, { "text": "混合表现为部分肢体为伸展,部分肢体为屈曲。", "entities": [ { "start_idx": 5, "end_idx": 11, "type": "sym", "entity": "部分肢体为伸展" }, { "start_idx": 13, "end_idx": 19, "type": "sym", "entity": "部分肢体为屈曲" } ] }, { "text": "一般在5~6个月时出现第一次惊厥,往往伴有发热或在惊厥前有感染或预防接种史,初起发作形式为阵挛或强直-阵挛,以后才呈肌阵挛发作,形式多样,可为全身抽动或某个肢体抽动,发作时常摔倒。", "entities": [ { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "发热" }, { "start_idx": 45, "end_idx": 46, "type": "sym", "entity": "阵挛" }, { "start_idx": 48, "end_idx": 52, "type": "sym", "entity": "强直-阵挛" }, { "start_idx": 58, "end_idx": 60, "type": "sym", "entity": "肌阵挛" }, { "start_idx": 71, "end_idx": 74, "type": "sym", "entity": "全身抽动" }, { "start_idx": 76, "end_idx": 81, "type": "sym", "entity": "某个肢体抽动" } ] }, { "text": "神经影像学检查正常。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "pro", "entity": "神经影像学" } ] }, { "text": "癫痫持续状态可能的原因和诱因包括脑外伤、颅内占位性病变、中枢感染、中毒以及代谢性疾病等。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "dis", "entity": "脑外伤" }, { "start_idx": 20, "end_idx": 26, "type": "dis", "entity": "颅内占位性病变" }, { "start_idx": 28, "end_idx": 31, "type": "dis", "entity": "中枢感染" }, { "start_idx": 33, "end_idx": 34, "type": "dis", "entity": "中毒" }, { "start_idx": 37, "end_idx": 41, "type": "dis", "entity": "代谢性疾病" } ] }, { "text": "①必要的实验室检查如血生化检查(血钙、血糖、电解质及其他生化物质等)、脑脊液检查、先天性遗传及代谢疾病血液与尿液筛查试验,神经免疫功能检查,染色体分析和基因定位检查、皮肤及肌肉活体组织检查;②影像学检查如头颅CT、MRI、MRA及DSA了解脑部结构异常;PET及SPECT了解大脑功能改变及帮助癫痫定位;FMRI(功能性MRI)、MEG(脑磁图)及IAP(颈内动脉异戊巴比妥试验)等检查,了解脑的结构与功能的关系。", "entities": [ { "start_idx": 4, "end_idx": 8, "type": "pro", "entity": "实验室检查" }, { "start_idx": 10, "end_idx": 14, "type": "pro", "entity": "血生化检查" }, { "start_idx": 35, "end_idx": 39, "type": "pro", "entity": "脑脊液检查" }, { "start_idx": 41, "end_idx": 59, "type": "pro", "entity": "先天性遗传及代谢疾病血液与尿液筛查试验" }, { "start_idx": 61, "end_idx": 68, "type": "pro", "entity": "神经免疫功能检查" }, { "start_idx": 70, "end_idx": 74, "type": "ite", "entity": "染色体分析" }, { "start_idx": 76, "end_idx": 81, "type": "pro", "entity": "基因定位检查" }, { "start_idx": 83, "end_idx": 93, "type": "pro", "entity": "皮肤及肌肉活体组织检查" }, { "start_idx": 96, "end_idx": 100, "type": "pro", "entity": "影像学检查" }, { "start_idx": 102, "end_idx": 105, "type": "pro", "entity": "头颅CT" }, { "start_idx": 107, "end_idx": 109, "type": "pro", "entity": "MRI" }, { "start_idx": 111, "end_idx": 113, "type": "pro", "entity": "MRA" }, { "start_idx": 115, "end_idx": 117, "type": "pro", "entity": "DSA" }, { "start_idx": 127, "end_idx": 129, "type": "pro", "entity": "PET" }, { "start_idx": 131, "end_idx": 135, "type": "pro", "entity": "SPECT" }, { "start_idx": 152, "end_idx": 155, "type": "pro", "entity": "FMRI" }, { "start_idx": 165, "end_idx": 167, "type": "pro", "entity": "MEG" }, { "start_idx": 169, "end_idx": 171, "type": "pro", "entity": "脑磁图" }, { "start_idx": 174, "end_idx": 176, "type": "pro", "entity": "IAP" }, { "start_idx": 178, "end_idx": 188, "type": "pro", "entity": "颈内动脉异戊巴比妥试验" } ] }, { "text": "儿童甲亢主要见于弥漫性毒性甲状腺肿(Graves病)。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "甲亢" }, { "start_idx": 8, "end_idx": 16, "type": "dis", "entity": "弥漫性毒性甲状腺肿" }, { "start_idx": 18, "end_idx": 24, "type": "dis", "entity": "Graves病" } ] }, { "text": "除了Graves病外,有少数病例甲状腺内有结节(包括腺瘤),称结节性毒性甲状腺肿伴功能亢进。", "entities": [ { "start_idx": 2, "end_idx": 8, "type": "dis", "entity": "Graves病" }, { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "甲状腺" }, { "start_idx": 21, "end_idx": 22, "type": "sym", "entity": "结节" }, { "start_idx": 26, "end_idx": 27, "type": "dis", "entity": "腺瘤" }, { "start_idx": 31, "end_idx": 39, "type": "dis", "entity": "结节性毒性甲状腺肿" }, { "start_idx": 41, "end_idx": 44, "type": "sym", "entity": "功能亢进" } ] }, { "text": "儿童甲亢临床过程个体差异很大,症状逐渐加重,症状开始到确诊时间一般在6~12个月。", "entities": [ { "start_idx": 2, "end_idx": 3, "type": "dis", "entity": "甲亢" } ] }, { "text": "本症初发病时症状不甚明显、进展缓慢,常先呈现情绪不稳定,上课思想不集中,易激惹、多动和注意力不集中等轻微行为改变。", "entities": [ { "start_idx": 2, "end_idx": 55, "type": "sym", "entity": "初发病时症状不甚明显、进展缓慢,常先呈现情绪不稳定,上课思想不集中,易激惹、多动和注意力不集中等轻微行为改变" } ] }, { "text": "甲状腺“危象”是甲状腺功能亢进症的一种类型,表现为急性发病、高热、严重的心动过速和不安,可迅速发展为谵妄、昏迷以至死亡。", "entities": [ { "start_idx": 0, "end_idx": 6, "type": "dis", "entity": "甲状腺“危象”" }, { "start_idx": 8, "end_idx": 15, "type": "dis", "entity": "甲状腺功能亢进症" }, { "start_idx": 25, "end_idx": 58, "type": "sym", "entity": "急性发病、高热、严重的心动过速和不安,可迅速发展为谵妄、昏迷以至死亡" } ] }, { "text": "新生儿甲亢较少见,大多属暂时性,常见于患有甲亢的孕妇。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "新生儿甲亢" }, { "start_idx": 21, "end_idx": 22, "type": "dis", "entity": "甲亢" } ] }, { "text": "避免外来的刺激和压力,饮食应富有蛋白质、糖类及维生素等。", "entities": [ { "start_idx": 16, "end_idx": 18, "type": "bod", "entity": "蛋白质" }, { "start_idx": 20, "end_idx": 21, "type": "bod", "entity": "糖类" }, { "start_idx": 23, "end_idx": 25, "type": "dru", "entity": "维生素" } ] }, { "text": "根据统计,治疗后弥漫性毒性甲状腺肿每2年只有25%的缓解率,因此药物治疗可能必须维持达5年或更久。", "entities": [ { "start_idx": 8, "end_idx": 16, "type": "dis", "entity": "弥漫性毒性甲状腺肿" } ] }, { "text": "4.如心血管症状明显者可加用肾上腺素能受体阻断药普萘洛尔作为辅助药物,减轻交感神经过度兴奋所致的心律快、多汗及震颤等症状,用量为1~2mg/(kg•d),分3次口服。", "entities": [ { "start_idx": 3, "end_idx": 5, "type": "bod", "entity": "心血管" }, { "start_idx": 14, "end_idx": 17, "type": "dru", "entity": "肾上腺素" }, { "start_idx": 24, "end_idx": 27, "type": "dru", "entity": "普萘洛尔" }, { "start_idx": 35, "end_idx": 56, "type": "sym", "entity": "减轻交感神经过度兴奋所致的心律快、多汗及震颤" } ] }, { "text": "主要是手术加放疗和化疗。", "entities": [ { "start_idx": 3, "end_idx": 4, "type": "pro", "entity": "手术" }, { "start_idx": 6, "end_idx": 7, "type": "pro", "entity": "放疗" }, { "start_idx": 9, "end_idx": 10, "type": "pro", "entity": "化疗" } ] }, { "text": "系儿童单纯性血尿的常见原因之一。", "entities": [ { "start_idx": 3, "end_idx": 7, "type": "dis", "entity": "单纯性血尿" } ] }, { "text": "高钙尿引起血尿的机制尚未肯定,有人认为系X线不能发现的细微钙结晶引起尿路损伤所致,亦有人提出和肾间质炎症有关。", "entities": [ { "start_idx": 0, "end_idx": 2, "type": "sym", "entity": "高钙尿" }, { "start_idx": 5, "end_idx": 6, "type": "sym", "entity": "血尿" }, { "start_idx": 20, "end_idx": 21, "type": "ite", "entity": "X线" }, { "start_idx": 34, "end_idx": 37, "type": "dis", "entity": "尿路损伤" }, { "start_idx": 47, "end_idx": 51, "type": "dis", "entity": "肾间质炎症" } ] }, { "text": "成人特发性高钙尿者最终可有40%~60%发生肾结石,而儿童仅有2%~5%可出现肾结石。", "entities": [ { "start_idx": 2, "end_idx": 7, "type": "dis", "entity": "特发性高钙尿" }, { "start_idx": 22, "end_idx": 24, "type": "dis", "entity": "肾结石" }, { "start_idx": 39, "end_idx": 41, "type": "dis", "entity": "肾结石" } ] }, { "text": "易并发尿路感染,也有病例出现多尿和多饮。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "dis", "entity": "尿路感染" }, { "start_idx": 14, "end_idx": 15, "type": "sym", "entity": "多尿" }, { "start_idx": 17, "end_idx": 18, "type": "sym", "entity": "多饮" } ] }, { "text": "儿童中以手术后制动、先天性肾小管功能紊乱及糖皮质激素引起较为常见。", "entities": [ { "start_idx": 10, "end_idx": 19, "type": "dis", "entity": "先天性肾小管功能紊乱" }, { "start_idx": 21, "end_idx": 25, "type": "dru", "entity": "糖皮质激素" } ] }, { "text": "常用氢氯噻嗪1~2mg/(kg•d),疗程一般小于4个月,可取得较显著效果,尚应注意药物不良反应。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "dru", "entity": "氢氯噻嗪" } ] }, { "text": "由于气管软骨支撑作用不足,吸气时随着气道内压力的改变导致气管壁的塌陷,引起功能性气道狭窄阻塞,出现呼吸困难、喘鸣、发绀等。", "entities": [ { "start_idx": 2, "end_idx": 5, "type": "bod", "entity": "气管软骨" }, { "start_idx": 18, "end_idx": 19, "type": "bod", "entity": "气道" }, { "start_idx": 28, "end_idx": 30, "type": "bod", "entity": "气管壁" }, { "start_idx": 37, "end_idx": 45, "type": "dis", "entity": "功能性气道狭窄阻塞" }, { "start_idx": 49, "end_idx": 52, "type": "sym", "entity": "呼吸困难" }, { "start_idx": 54, "end_idx": 55, "type": "sym", "entity": "喘鸣" }, { "start_idx": 57, "end_idx": 58, "type": "sym", "entity": "发绀" } ] }, { "text": "支气管软化主要表现为喘鸣,如病变发生在一侧(左侧多),可发生病侧喘鸣音更响、气体进入延迟。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "支气管软化" }, { "start_idx": 10, "end_idx": 11, "type": "sym", "entity": "喘鸣" }, { "start_idx": 30, "end_idx": 36, "type": "sym", "entity": "病侧喘鸣音更响" }, { "start_idx": 38, "end_idx": 43, "type": "sym", "entity": "气体进入延迟" } ] }, { "text": "Finder报道17例原发性支气管软化,均在6个月内发病,以喘鸣为主,均有左主支气管受累,两侧支气管受累1例,伴喉软化1例,轻度气管软化2例。", "entities": [ { "start_idx": 11, "end_idx": 18, "type": "dis", "entity": "原发性支气管软化" }, { "start_idx": 30, "end_idx": 31, "type": "sym", "entity": "喘鸣" }, { "start_idx": 37, "end_idx": 41, "type": "bod", "entity": "左主支气管" }, { "start_idx": 47, "end_idx": 49, "type": "bod", "entity": "支气管" }, { "start_idx": 56, "end_idx": 58, "type": "dis", "entity": "喉软化" }, { "start_idx": 62, "end_idx": 67, "type": "dis", "entity": "轻度气管软化" } ] }, { "text": "本症亦可继发于其他原因,如气管插管时间过长、损伤、肿瘤和淋巴结压迫等。", "entities": [ { "start_idx": 13, "end_idx": 16, "type": "pro", "entity": "气管插管" }, { "start_idx": 25, "end_idx": 26, "type": "dis", "entity": "肿瘤" }, { "start_idx": 28, "end_idx": 32, "type": "dis", "entity": "淋巴结压迫" } ] }, { "text": "本病以保守疗法为主,包括增强营养、适当补充维生素D及钙剂,保持呼吸道通畅。", "entities": [ { "start_idx": 3, "end_idx": 6, "type": "pro", "entity": "保守疗法" }, { "start_idx": 12, "end_idx": 15, "type": "pro", "entity": "增强营养" }, { "start_idx": 17, "end_idx": 27, "type": "pro", "entity": "适当补充维生素D及钙剂" }, { "start_idx": 31, "end_idx": 33, "type": "bod", "entity": "呼吸道" } ] }, { "text": "支气管软化者应注意体位引流,可应用色甘酸、溴化异丙托品,但应避免使用β受体激动剂。", "entities": [ { "start_idx": 0, "end_idx": 4, "type": "dis", "entity": "支气管软化" }, { "start_idx": 9, "end_idx": 12, "type": "pro", "entity": "体位引流" }, { "start_idx": 17, "end_idx": 19, "type": "dru", "entity": "色甘酸" }, { "start_idx": 21, "end_idx": 26, "type": "dru", "entity": "溴化异丙托品" }, { "start_idx": 34, "end_idx": 39, "type": "dru", "entity": "β受体激动剂" } ] }, { "text": "对严重呼吸困难、常规治疗无效、尤其是气管切开后不能拔管者,可采用大血管固定、气管悬吊术、气囊扩张金属支架等方法。", "entities": [ { "start_idx": 1, "end_idx": 6, "type": "sym", "entity": "严重呼吸困难" }, { "start_idx": 18, "end_idx": 21, "type": "pro", "entity": "气管切开" }, { "start_idx": 32, "end_idx": 36, "type": "pro", "entity": "大血管固定" }, { "start_idx": 38, "end_idx": 42, "type": "pro", "entity": "气管悬吊术" }, { "start_idx": 44, "end_idx": 51, "type": "pro", "entity": "气囊扩张金属支架" } ] } ]