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转移性骨肿瘤的部位也同原发肿瘤生长的部位有关,如乳腺癌的骨转移通常发生在胸椎和肱骨近端,甲状腺癌则常见于颈椎和颅骨,当然有时转移性肿瘤的发生也与原发肿瘤的部位无关。
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骨盆的转移性肿瘤常同时累及髂骨、耻骨和坐骨,脊椎的转移性肿瘤有时同时侵犯邻近的几个椎体和肋骨。
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但有些以骨肿瘤为首发症状的转移性骨肿瘤在诊断上往往要依赖于各种实验室检查。
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很多单发性的骨肿瘤病变,应该尽量地将活体组织检查手术操作同肿瘤组织的手术清除结合起来。
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有时,肿瘤部位很深或周围结构非常紧凑,如脊柱椎体的转移性肿瘤,也要充分考虑到活体组织检查操作本身的风险和操作后的并发症,采用其他的组织检查方法如穿刺活检也不失为一种较好的诊断方法。
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骨骼的病变可以采用手术清除、局部放疗和全身性化学治疗等方法。
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出现骨骼并发症如病理性骨折的病例,要及时治疗出现的并发症。
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主要侵犯主动脉弓,胸、腹主动脉及其分支,60%~70%累及一侧或双侧肾动脉。
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可因为头痛特别是枕部头痛、眩晕、急躁、过度兴奋、不安及疲乏而就医。
[ { "end_idx": 4, "entity": "头痛", "start_idx": 3, "type": "sym" }, { "end_idx": 3, "entity": "头", "start_idx": 3, "type": "bod" }, { "end_idx": 11, "entity": "枕部头痛", "start_idx": 8, "type": "sym" }, { "end_idx": 9, "entity": "枕部", "start_idx": 8, "type": "bod" }, { "end_idx": 10, "entity": "头", "start_idx": 10, "type": "bod" }, { "end_idx": 14, "entity": "眩晕", "start_idx": 13, "type": "sym" }, { "end_idx": 17, "entity": "急躁", "start_idx": 16, "type": "sym" }, { "end_idx": 22, "entity": "过度兴奋", "start_idx": 19, "type": "sym" }, { "end_idx": 25, "entity": "不安", "start_idx": 24, "type": "sym" }, { "end_idx": 28, "entity": "疲乏", "start_idx": 27, "type": "sym" } ]
重症病人可有高血压脑病,有一过性视力障碍及抽搐等,有的可表现为行为异常或好动等。
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X线胸片可见左心室增大,也曾见RVH致全心衰竭者呈全心普遍增大,肺淤血表现。
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易误诊为哮喘,多有反复上呼吸道感染。
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在治疗以前需小心进软食并治疗肺部感染。
[ { "end_idx": 17, "entity": "肺部感染", "start_idx": 14, "type": "dis" } ]
对淋巴管肉瘤无有效治疗方案,肿瘤对放疗不敏感,成人病例多要截肢。
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凡有慢性和先天性淋巴水肿的患者,应定期检查。
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(3)班布特罗(Bambec)1mg/ml,100ml/瓶。
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(2)优喘平:每片400mg。
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紧张、恐惧、激动、睡眠不足、气候变化、噪声、闪光刺激以及某些特殊食物的摄入(如奶酪和巧克力)等因素,均可诱发偏头痛发作。
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小儿偏头痛的临床特点是:①发作持续时间短,但发作次数较频;②双侧头痛多见,偏侧头痛相对少见;③视觉症状及头痛为搏动性较少见;④胃肠道症状突出,常伴有恶心、呕吐及腹痛;⑤有家族遗传史者多见;⑥伴夜尿、夜惊、夜游症及晕车晕船者多见。
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头痛前没有明确的先兆,但常有一些非特异的症状,如嗜睡、疲劳、周身不适以及食欲减退等。
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70%有恶心、呕吐或腹痛等胃肠道症状。
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可分两类:家族性多呈常染色体显性遗传;散发性可表现为经典型、普通型和偏瘫型偏头痛的交替发作。
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有明确的起源于双侧枕叶或脑干的先兆症状,视觉症状如闪光、暗点、视物模糊及黑蒙等,脑干症状如眩晕、复视、眼球震颤、耳鸣、构音障碍、共济失调、双侧肢体麻木及无力等,甚至可出现短暂的意识丧失。
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主要包括良性阵发性眩晕、周期性呕吐(再发性呕吐)、腹型偏头痛、儿童交替性偏瘫以及阵发性斜颈等。
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临床上除可出现贫血外,还可因缺铁而降低许多含铁酶的生物活性,进而影响细胞代谢功能,使机体出现消化道功能紊乱、循环功能障碍、免疫功能低下、精神神经症状以及皮肤黏膜病变等一系列非血液系统的表现。
[ { "end_idx": 8, "entity": "贫血", "start_idx": 7, "type": "dis" }, { "end_idx": 28, "entity": "缺铁而降低许多含铁酶的生物活性", "start_idx": 14, "type": "sym" }, { "end_idx": 15, "entity": "铁", "start_idx": 15, "type": "bod" }, { "end_idx": 23, "entity": "含铁酶", "start_idx": 21, "type": "bod" }, { "end_idx": 39, "entity": "影响细胞代谢功能", "start_idx": 32, "type": "sym" }, { "end_idx": 35, "entity": "细胞", "start_idx": 34, "type": "bod" }, { "end_idx": 52, "entity": "消化道功能紊乱", "start_idx": 46, "type": "dis" }, { "end_idx": 59, "entity": "循环功能障碍", "start_idx": 54, "type": "dis" }, { "end_idx": 66, "entity": "免疫功能低下", "start_idx": 61, "type": "sym" }, { "end_idx": 73, "entity": "精神神经症状", "start_idx": 68, "type": "sym" }, { "end_idx": 71, "entity": "神经", "start_idx": 70, "type": "bod" }, { "end_idx": 81, "entity": "皮肤黏膜病变", "start_idx": 76, "type": "dis" }, { "end_idx": 90, "entity": "非血液系统", "start_idx": 86, "type": "bod" } ]
缺铁与贫血之间是一个循序渐进的过程,当体内出现缺铁时,最早出现的表现是贮存铁的下降,如缺铁持续存在,即可出现铁蛋白下降,此时仍可无血红蛋白下降,临床上也无贫血的表现。
[ { "end_idx": 1, "entity": "缺铁", "start_idx": 0, "type": "sym" }, { "end_idx": 1, "entity": "铁", "start_idx": 1, "type": "bod" }, { "end_idx": 4, "entity": "贫血", "start_idx": 3, "type": "dis" }, { "end_idx": 24, "entity": "缺铁", "start_idx": 23, "type": "sym" }, { "end_idx": 24, "entity": "铁", "start_idx": 24, "type": "bod" }, { "end_idx": 40, "entity": "贮存铁的下降", "start_idx": 35, "type": "sym" }, { "end_idx": 37, "entity": "贮存铁", "start_idx": 35, "type": "bod" }, { "end_idx": 48, "entity": "缺铁持续存在", "start_idx": 43, "type": "sym" }, { "end_idx": 44, "entity": "铁", "start_idx": 44, "type": "bod" }, { "end_idx": 58, "entity": "铁蛋白下降", "start_idx": 54, "type": "sym" }, { "end_idx": 56, "entity": "铁蛋白", "start_idx": 54, "type": "bod" }, { "end_idx": 70, "entity": "血红蛋白下降", "start_idx": 65, "type": "sym" }, { "end_idx": 68, "entity": "血红蛋白", "start_idx": 65, "type": "bod" }, { "end_idx": 78, "entity": "贫血", "start_idx": 77, "type": "dis" } ]
年长儿可诉头晕、眼前发黑、耳鸣等。
[ { "end_idx": 6, "entity": "头晕", "start_idx": 5, "type": "sym" }, { "end_idx": 5, "entity": "头", "start_idx": 5, "type": "bod" }, { "end_idx": 11, "entity": "眼前发黑", "start_idx": 8, "type": "sym" }, { "end_idx": 8, "entity": "眼", "start_idx": 8, "type": "bod" }, { "end_idx": 14, "entity": "耳鸣", "start_idx": 13, "type": "sym" }, { "end_idx": 13, "entity": "耳", "start_idx": 13, "type": "bod" } ]
肝、脾、淋巴结可轻度增大,主要原因为髓外造血。
[ { "end_idx": 11, "entity": "肝、脾、淋巴结可轻度增大", "start_idx": 0, "type": "sym" }, { "end_idx": 0, "entity": "肝", "start_idx": 0, "type": "bod" }, { "end_idx": 2, "entity": "脾", "start_idx": 2, "type": "bod" }, { "end_idx": 6, "entity": "淋巴结", "start_idx": 4, "type": "bod" }, { "end_idx": 21, "entity": "髓外造血", "start_idx": 18, "type": "sym" }, { "end_idx": 19, "entity": "髓外", "start_idx": 18, "type": "bod" }, { "end_idx": 21, "entity": "血", "start_idx": 21, "type": "bod" } ]
有时有腹泻或呕吐,皮肤微肿,脉搏加速,心前区可有吹风样收缩期杂音。
[ { "end_idx": 4, "entity": "腹泻", "start_idx": 3, "type": "sym" }, { "end_idx": 3, "entity": "腹", "start_idx": 3, "type": "bod" }, { "end_idx": 7, "entity": "呕吐", "start_idx": 6, "type": "sym" }, { "end_idx": 12, "entity": "皮肤微肿", "start_idx": 9, "type": "sym" }, { "end_idx": 10, "entity": "皮肤", "start_idx": 9, "type": "bod" }, { "end_idx": 17, "entity": "脉搏加速", "start_idx": 14, "type": "sym" }, { "end_idx": 14, "entity": "脉", "start_idx": 14, "type": "bod" }, { "end_idx": 31, "entity": "心前区可有吹风样收缩期杂音", "start_idx": 19, "type": "sym" }, { "end_idx": 21, "entity": "心前区", "start_idx": 19, "type": "bod" } ]
单纯IDA时,白细胞通常在正常范围或稍低。
[ { "end_idx": 4, "entity": "单纯IDA", "start_idx": 0, "type": "dis" }, { "end_idx": 19, "entity": "白细胞通常在正常范围或稍低", "start_idx": 7, "type": "sym" }, { "end_idx": 9, "entity": "白细胞", "start_idx": 7, "type": "bod" } ]
伴有钩虫病患儿可有嗜酸性粒细胞增多。
[ { "end_idx": 4, "entity": "钩虫病", "start_idx": 2, "type": "dis" }, { "end_idx": 16, "entity": "嗜酸性粒细胞增多", "start_idx": 9, "type": "sym" }, { "end_idx": 14, "entity": "嗜酸性粒细胞", "start_idx": 9, "type": "bod" } ]
典型IDA的诊断并不困难,但轻型病例和无贫血缺铁状态的诊断,须进行血清铁、铁结合力、骨髓细胞外铁染色检查及红细胞内游离原卟啉的测定方能作出明确诊断。
[ { "end_idx": 4, "entity": "典型IDA", "start_idx": 0, "type": "dis" }, { "end_idx": 21, "entity": "贫血", "start_idx": 20, "type": "dis" }, { "end_idx": 23, "entity": "缺铁", "start_idx": 22, "type": "sym" }, { "end_idx": 23, "entity": "铁", "start_idx": 23, "type": "bod" }, { "end_idx": 51, "entity": "血清铁、铁结合力、骨髓细胞外铁染色检查", "start_idx": 33, "type": "pro" }, { "end_idx": 64, "entity": "红细胞内游离原卟啉的测定", "start_idx": 53, "type": "pro" } ]
IDA主要与小细胞低色素性贫血鉴别,包括铅中毒、β-地中海贫血、α-地中海贫血、慢性感染引起的贫血等。
[ { "end_idx": 2, "entity": "IDA", "start_idx": 0, "type": "dis" }, { "end_idx": 14, "entity": "小细胞低色素性贫血", "start_idx": 6, "type": "dis" }, { "end_idx": 22, "entity": "铅中毒", "start_idx": 20, "type": "dis" }, { "end_idx": 30, "entity": "β-地中海贫血", "start_idx": 24, "type": "dis" }, { "end_idx": 38, "entity": "α-地中海贫血", "start_idx": 32, "type": "dis" }, { "end_idx": 43, "entity": "慢性感染", "start_idx": 40, "type": "dis" }, { "end_idx": 48, "entity": "贫血", "start_idx": 47, "type": "dis" } ]
对易感儿,应给予预防量铁剂预防。
[ { "end_idx": 12, "entity": "铁剂", "start_idx": 11, "type": "dru" } ]
在钩虫病流行地区,要大力开展消灭寄生虫病的卫生防疫工作,防止病儿重复感染,同时需给予口服铁剂,以预防或治疗贫血。
[ { "end_idx": 3, "entity": "钩虫病", "start_idx": 1, "type": "dis" }, { "end_idx": 19, "entity": "寄生虫病", "start_idx": 16, "type": "dis" }, { "end_idx": 35, "entity": "感染", "start_idx": 34, "type": "dis" }, { "end_idx": 43, "entity": "口服", "start_idx": 42, "type": "pro" }, { "end_idx": 45, "entity": "铁剂", "start_idx": 44, "type": "dru" }, { "end_idx": 54, "entity": "贫血", "start_idx": 53, "type": "dis" } ]
第一种是以神经的病变部位为分类基础,其代表为Bors-Coman分类法,根据脊髓损伤的部位将神经源性膀胱分为上运动神经元型(骶上型)、下运动神经元型(骶下型)以及混合型;第二种以膀胱功能变化为基础,如Wein分类法,根据膀胱排空机能将神经源性膀胱分为贮尿障碍和排空障碍,该方法的优点是较适用于临床应用。
[ { "end_idx": 9, "entity": "神经的病变", "start_idx": 5, "type": "dis" }, { "end_idx": 41, "entity": "脊髓损伤", "start_idx": 38, "type": "dis" }, { "end_idx": 51, "entity": "神经源性膀胱", "start_idx": 46, "type": "dis" }, { "end_idx": 60, "entity": "上运动神经元型", "start_idx": 54, "type": "dis" }, { "end_idx": 64, "entity": "骶上型", "start_idx": 62, "type": "dis" }, { "end_idx": 73, "entity": "下运动神经元型", "start_idx": 67, "type": "dis" }, { "end_idx": 77, "entity": "骶下型", "start_idx": 75, "type": "dis" }, { "end_idx": 83, "entity": "混合型", "start_idx": 81, "type": "dis" }, { "end_idx": 90, "entity": "膀胱", "start_idx": 89, "type": "bod" }, { "end_idx": 111, "entity": "膀胱", "start_idx": 110, "type": "bod" }, { "end_idx": 122, "entity": "神经源性膀胱", "start_idx": 117, "type": "dis" }, { "end_idx": 128, "entity": "贮尿障碍", "start_idx": 125, "type": "dis" }, { "end_idx": 133, "entity": "排空障碍", "start_idx": 130, "type": "dis" } ]
因此,进行尿常规和培养、尿素氮和肌酐以及血钠、钾、氯和二氧化碳结合力等检查对了解神经源性膀胱患儿的上尿路损害程度与全身状况十分重要。
[ { "end_idx": 7, "entity": "尿常规", "start_idx": 5, "type": "ite" }, { "end_idx": 10, "entity": "培养", "start_idx": 9, "type": "pro" }, { "end_idx": 14, "entity": "尿素氮", "start_idx": 12, "type": "ite" }, { "end_idx": 17, "entity": "肌酐", "start_idx": 16, "type": "ite" }, { "end_idx": 36, "entity": "血钠、钾、氯和二氧化碳结合力等检查", "start_idx": 20, "type": "ite" }, { "end_idx": 45, "entity": "神经源性膀胱", "start_idx": 40, "type": "dis" }, { "end_idx": 53, "entity": "上尿路损害", "start_idx": 49, "type": "dis" } ]
放射性核素显像可精确评价患儿分肾功能受损程度。
[ { "end_idx": 6, "entity": "放射性核素显像", "start_idx": 0, "type": "pro" }, { "end_idx": 19, "entity": "肾功能受损", "start_idx": 15, "type": "dis" } ]
因此,将尿流动力学结果与病史、体格检查以及影像学发现进行综合评价,才能对神经源性膀胱患儿作出个体化的全面诊断,从而达到为治疗提供合理指导的目的。
[ { "end_idx": 4, "entity": "尿", "start_idx": 4, "type": "bod" }, { "end_idx": 18, "entity": "体格检查", "start_idx": 15, "type": "pro" }, { "end_idx": 41, "entity": "神经源性膀胱", "start_idx": 36, "type": "dis" } ]
根据Wein分类法决定的神经源性膀胱的基本治疗原则对临床治疗有较好的指导意义。
[ { "end_idx": 17, "entity": "神经源性膀胱", "start_idx": 12, "type": "dis" } ]
非手术治疗包括:①导尿术;②药物治疗;③神经阻滞疗法;④其他方法如生物反馈技术及排尿训练等。
[ { "end_idx": 2, "entity": "手术", "start_idx": 1, "type": "pro" }, { "end_idx": 11, "entity": "导尿术", "start_idx": 9, "type": "pro" }, { "end_idx": 17, "entity": "药物治疗", "start_idx": 14, "type": "pro" }, { "end_idx": 25, "entity": "神经阻滞疗法", "start_idx": 20, "type": "pro" }, { "end_idx": 38, "entity": "生物反馈技术", "start_idx": 33, "type": "pro" }, { "end_idx": 43, "entity": "排尿训练", "start_idx": 40, "type": "pro" } ]
手术治疗包括:①降低膀胱出口阻力的手术,如神经根切断术、经尿道外括约肌/膀胱颈部切开术等;②增加膀胱出口阻力的手术,如膀胱颈悬吊术、特氟隆(Teflon)等生物材料注射手术以及人工括约肌装置等;③增加膀胱容量的手术,如自体或胃(肠)膀胱扩大成形术;④增加膀胱逼尿肌收缩能力的手术,如电刺激治疗以及单层肠浆肌层膀胱加强术等;⑤其他如尿流改道以及并发症的治疗。
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可伴有低热、食欲缺乏及乏力等全身症状。
[ { "end_idx": 4, "entity": "低热", "start_idx": 3, "type": "sym" }, { "end_idx": 9, "entity": "食欲缺乏", "start_idx": 6, "type": "sym" }, { "end_idx": 12, "entity": "乏力", "start_idx": 11, "type": "sym" } ]
可用泼尼松1~2mg/(kg•d),分次口服,或用地塞米松、甲基泼尼松龙静脉滴注,症状缓解后即可停用。
[ { "end_idx": 4, "entity": "泼尼松", "start_idx": 2, "type": "dru" }, { "end_idx": 21, "entity": "口服", "start_idx": 20, "type": "pro" }, { "end_idx": 28, "entity": "地塞米松", "start_idx": 25, "type": "dru" }, { "end_idx": 35, "entity": "甲基泼尼松龙", "start_idx": 30, "type": "dru" }, { "end_idx": 39, "entity": "静脉滴注", "start_idx": 36, "type": "pro" } ]
重症可用免疫抑制剂如环磷酰胺或雷公藤多甙片。
[ { "end_idx": 8, "entity": "免疫抑制剂", "start_idx": 4, "type": "dru" }, { "end_idx": 13, "entity": "环磷酰胺", "start_idx": 10, "type": "dru" }, { "end_idx": 20, "entity": "雷公藤多甙片", "start_idx": 15, "type": "dru" } ]
实体瘤原发于体表部位时,主要的就诊原因是可扪及的无痛性肿块。
[ { "end_idx": 2, "entity": "实体瘤", "start_idx": 0, "type": "dis" }, { "end_idx": 7, "entity": "体表", "start_idx": 6, "type": "bod" }, { "end_idx": 28, "entity": "可扪及的无痛性肿块", "start_idx": 20, "type": "sym" }, { "end_idx": 22, "entity": "可扪及", "start_idx": 20, "type": "bod" } ]
肿瘤原发于纵隔、腹腔、盆腔时,早期常无症状,体检也不易发现肿块,当肿瘤生长至压迫邻近组织、器官使其功能障碍时,出现压迫症状(表11-11)。
[ { "end_idx": 12, "entity": "肿瘤原发于纵隔、腹腔、盆腔", "start_idx": 0, "type": "sym" }, { "end_idx": 1, "entity": "肿瘤", "start_idx": 0, "type": "dis" }, { "end_idx": 6, "entity": "纵隔", "start_idx": 5, "type": "bod" }, { "end_idx": 9, "entity": "腹腔", "start_idx": 8, "type": "bod" }, { "end_idx": 12, "entity": "盆腔", "start_idx": 11, "type": "bod" }, { "end_idx": 30, "entity": "肿块", "start_idx": 29, "type": "sym" }, { "end_idx": 52, "entity": "肿瘤生长至压迫邻近组织、器官使其功能障碍", "start_idx": 33, "type": "sym" }, { "end_idx": 34, "entity": "肿瘤", "start_idx": 33, "type": "dis" }, { "end_idx": 43, "entity": "压迫邻近组织", "start_idx": 38, "type": "bod" }, { "end_idx": 46, "entity": "器官", "start_idx": 45, "type": "bod" }, { "end_idx": 52, "entity": "功能障碍", "start_idx": 49, "type": "dis" }, { "end_idx": 60, "entity": "压迫症状", "start_idx": 57, "type": "sym" } ]
全身播散时出现全身症状如苍白、消瘦、发热、骨关节疼痛、出血,肝、脾、淋巴结肿大等。
[ { "end_idx": 13, "entity": "苍白", "start_idx": 12, "type": "sym" }, { "end_idx": 16, "entity": "消瘦", "start_idx": 15, "type": "sym" }, { "end_idx": 19, "entity": "发热", "start_idx": 18, "type": "sym" }, { "end_idx": 23, "entity": "骨关节", "start_idx": 21, "type": "bod" }, { "end_idx": 25, "entity": "骨关节疼痛", "start_idx": 21, "type": "sym" }, { "end_idx": 28, "entity": "出血", "start_idx": 27, "type": "sym" }, { "end_idx": 28, "entity": "血", "start_idx": 28, "type": "bod" }, { "end_idx": 30, "entity": "肝", "start_idx": 30, "type": "bod" }, { "end_idx": 38, "entity": "肝、脾、淋巴结肿大", "start_idx": 30, "type": "sym" }, { "end_idx": 32, "entity": "脾", "start_idx": 32, "type": "bod" }, { "end_idx": 36, "entity": "淋巴结", "start_idx": 34, "type": "bod" } ]
根据其发病有无前驱症状(腹泻),分为典型HUS和非典型HUS。
[ { "end_idx": 10, "entity": "前驱症状", "start_idx": 7, "type": "sym" }, { "end_idx": 13, "entity": "腹泻", "start_idx": 12, "type": "sym" }, { "end_idx": 12, "entity": "腹", "start_idx": 12, "type": "bod" }, { "end_idx": 22, "entity": "典型HUS", "start_idx": 18, "type": "dis" }, { "end_idx": 29, "entity": "非典型HUS", "start_idx": 24, "type": "dis" } ]
细菌感染(如大肠埃希菌、志贺痢疾杆菌、肺炎链球菌和沙门菌)及病毒感染[包括柯萨奇病毒、埃可病毒、流感病毒、人类免疫缺陷病毒(HIV)]均可诱发HUS。
[ { "end_idx": 3, "entity": "细菌感染", "start_idx": 0, "type": "dis" }, { "end_idx": 10, "entity": "大肠埃希菌", "start_idx": 6, "type": "mic" }, { "end_idx": 17, "entity": "志贺痢疾杆菌", "start_idx": 12, "type": "mic" }, { "end_idx": 23, "entity": "肺炎链球菌", "start_idx": 19, "type": "mic" }, { "end_idx": 27, "entity": "沙门菌", "start_idx": 25, "type": "mic" }, { "end_idx": 33, "entity": "病毒感染", "start_idx": 30, "type": "dis" }, { "end_idx": 41, "entity": "柯萨奇病毒", "start_idx": 37, "type": "mic" }, { "end_idx": 46, "entity": "埃可病毒", "start_idx": 43, "type": "mic" }, { "end_idx": 51, "entity": "流感病毒", "start_idx": 48, "type": "mic" }, { "end_idx": 60, "entity": "人类免疫缺陷病毒", "start_idx": 53, "type": "mic" }, { "end_idx": 64, "entity": "HIV", "start_idx": 62, "type": "mic" }, { "end_idx": 73, "entity": "HUS", "start_idx": 71, "type": "dis" } ]
有资料表明,出血性大肠埃希菌(EHEC)O157:H7是引起一些地区流行性感染性腹泻相关的HUS的主要病原,O157:H7主要存在于家畜肠道、未煮熟透的肉类和未经消毒的牛奶。
[ { "end_idx": 26, "entity": "出血性大肠埃希菌(EHEC)O157:H7", "start_idx": 6, "type": "mic" }, { "end_idx": 41, "entity": "流行性感染性腹泻", "start_idx": 34, "type": "sym" }, { "end_idx": 40, "entity": "腹", "start_idx": 40, "type": "bod" }, { "end_idx": 47, "entity": "HUS", "start_idx": 45, "type": "dis" }, { "end_idx": 60, "entity": "O157:H7", "start_idx": 54, "type": "mic" } ]
腹痛严重者伴腹肌紧张,酷似急腹症;腹泻可为水样便,多见血便和黏液便。
[ { "end_idx": 3, "entity": "腹痛严重", "start_idx": 0, "type": "sym" }, { "end_idx": 0, "entity": "腹", "start_idx": 0, "type": "bod" }, { "end_idx": 9, "entity": "腹肌紧张", "start_idx": 6, "type": "sym" }, { "end_idx": 6, "entity": "腹", "start_idx": 6, "type": "bod" }, { "end_idx": 15, "entity": "急腹症", "start_idx": 13, "type": "dis" }, { "end_idx": 23, "entity": "腹泻可为水样便", "start_idx": 17, "type": "sym" }, { "end_idx": 17, "entity": "腹", "start_idx": 17, "type": "bod" }, { "end_idx": 23, "entity": "水样便", "start_idx": 21, "type": "bod" }, { "end_idx": 28, "entity": "血便", "start_idx": 27, "type": "sym" }, { "end_idx": 27, "entity": "血", "start_idx": 27, "type": "bod" }, { "end_idx": 28, "entity": "便", "start_idx": 28, "type": "bod" }, { "end_idx": 32, "entity": "黏液便", "start_idx": 30, "type": "sym" }, { "end_idx": 31, "entity": "黏液", "start_idx": 30, "type": "bod" }, { "end_idx": 32, "entity": "便", "start_idx": 32, "type": "bod" } ]
前驱期后经数天无症状期进入急性期,出现溶血性贫血、急性肾衰竭和血小板减少。
[ { "end_idx": 23, "entity": "溶血性贫血", "start_idx": 19, "type": "dis" }, { "end_idx": 29, "entity": "急性肾衰竭", "start_idx": 25, "type": "dis" }, { "end_idx": 35, "entity": "血小板减少", "start_idx": 31, "type": "sym" }, { "end_idx": 33, "entity": "血小板", "start_idx": 31, "type": "bod" } ]
初期可屡有溶血危象发生,于数小时内血色素下降30~50g/L;急性肾功能减退临床表现轻重不一,轻者仅短暂尿量减少,肾功能轻度减退,但多数患儿呈少尿性急性肾衰竭,少尿可持续达2周甚至2周以上,同时有氮质血症、代谢性酸中毒、高血钾等其他急性肾衰竭的表现,并可由于贫血、高血容量和电解质紊乱等引发充血性心力衰竭;血小板减少致出血倾向,以消化道出血为主,可见皮肤瘀斑,偶见硬脑膜下或视网膜出血。
[ { "end_idx": 8, "entity": "溶血危象", "start_idx": 5, "type": "sym" }, { "end_idx": 6, "entity": "血", "start_idx": 6, "type": "bod" }, { "end_idx": 21, "entity": "血色素下降", "start_idx": 17, "type": "sym" }, { "end_idx": 17, "entity": "血", "start_idx": 17, "type": "bod" }, { "end_idx": 37, "entity": "急性肾功能减退", "start_idx": 31, "type": "sym" }, { "end_idx": 33, "entity": "肾", "start_idx": 33, "type": "bod" }, { "end_idx": 55, "entity": "短暂尿量减少", "start_idx": 50, "type": "sym" }, { "end_idx": 52, "entity": "尿", "start_idx": 52, "type": "bod" }, { "end_idx": 63, "entity": "肾功能轻度减退", "start_idx": 57, "type": "sym" }, { "end_idx": 57, "entity": "肾", "start_idx": 57, "type": "bod" }, { "end_idx": 78, "entity": "少尿性急性肾衰竭", "start_idx": 71, "type": "dis" }, { "end_idx": 93, "entity": "少尿可持续达2周甚至2周以上", "start_idx": 80, "type": "sym" }, { "end_idx": 81, "entity": "尿", "start_idx": 81, "type": "bod" }, { "end_idx": 101, "entity": "氮质血症", "start_idx": 98, "type": "dis" }, { "end_idx": 108, "entity": "代谢性酸中毒", "start_idx": 103, "type": "dis" }, { "end_idx": 112, "entity": "高血钾", "start_idx": 110, "type": "dis" }, { "end_idx": 120, "entity": "急性肾衰竭", "start_idx": 116, "type": "dis" }, { "end_idx": 130, "entity": "贫血", "start_idx": 129, "type": "dis" }, { "end_idx": 135, "entity": "高血容量", "start_idx": 132, "type": "sym" }, { "end_idx": 133, "entity": "血", "start_idx": 133, "type": "bod" }, { "end_idx": 141, "entity": "电解质紊乱", "start_idx": 137, "type": "dis" }, { "end_idx": 151, "entity": "充血性心力衰竭", "start_idx": 145, "type": "dis" }, { "end_idx": 157, "entity": "血小板减少", "start_idx": 153, "type": "sym" }, { "end_idx": 155, "entity": "血小板", "start_idx": 153, "type": "bod" }, { "end_idx": 162, "entity": "出血倾向", "start_idx": 159, "type": "sym" }, { "end_idx": 160, "entity": "血", "start_idx": 160, "type": "bod" }, { "end_idx": 169, "entity": "消化道出血", "start_idx": 165, "type": "dis" }, { "end_idx": 178, "entity": "皮肤瘀斑", "start_idx": 175, "type": "sym" }, { "end_idx": 176, "entity": "皮肤", "start_idx": 175, "type": "bod" }, { "end_idx": 191, "entity": "硬脑膜下或视网膜出血", "start_idx": 182, "type": "dis" } ]
由于HUS存在广泛的微血管血栓形成,可导致多系统损害,除胃肠道和肾脏外,尤以中枢神经系统受累多见,是最常见的死因。
[ { "end_idx": 4, "entity": "HUS", "start_idx": 2, "type": "dis" }, { "end_idx": 14, "entity": "微血管血栓", "start_idx": 10, "type": "dis" }, { "end_idx": 25, "entity": "多系统损害", "start_idx": 21, "type": "dis" }, { "end_idx": 30, "entity": "胃肠道", "start_idx": 28, "type": "bod" }, { "end_idx": 33, "entity": "肾脏", "start_idx": 32, "type": "bod" }, { "end_idx": 45, "entity": "中枢神经系统受累", "start_idx": 38, "type": "sym" }, { "end_idx": 43, "entity": "中枢神经系统", "start_idx": 38, "type": "bod" } ]
神经系统症状表现有激惹、嗜睡、焦虑、紧张、幻觉、定向障碍、惊厥和昏迷,部分留有神经系统后遗症,如学习困难、行为异常,严重者可见智力低下或癫痫。
[ { "end_idx": 5, "entity": "神经系统症状", "start_idx": 0, "type": "sym" }, { "end_idx": 3, "entity": "神经系统", "start_idx": 0, "type": "bod" }, { "end_idx": 10, "entity": "激惹", "start_idx": 9, "type": "sym" }, { "end_idx": 13, "entity": "嗜睡", "start_idx": 12, "type": "sym" }, { "end_idx": 16, "entity": "焦虑", "start_idx": 15, "type": "sym" }, { "end_idx": 19, "entity": "紧张", "start_idx": 18, "type": "sym" }, { "end_idx": 22, "entity": "幻觉", "start_idx": 21, "type": "sym" }, { "end_idx": 27, "entity": "定向障碍", "start_idx": 24, "type": "sym" }, { "end_idx": 30, "entity": "惊厥", "start_idx": 29, "type": "dis" }, { "end_idx": 33, "entity": "昏迷", "start_idx": 32, "type": "sym" }, { "end_idx": 45, "entity": "神经系统后遗症", "start_idx": 39, "type": "dis" }, { "end_idx": 51, "entity": "学习困难", "start_idx": 48, "type": "sym" }, { "end_idx": 56, "entity": "行为异常", "start_idx": 53, "type": "sym" }, { "end_idx": 66, "entity": "智力低下", "start_idx": 63, "type": "sym" }, { "end_idx": 69, "entity": "癫痫", "start_idx": 68, "type": "dis" } ]
对疑有免疫因素参与发病机制者,可静脉输注丙种球蛋白。
[ { "end_idx": 19, "entity": "静脉输注", "start_idx": 16, "type": "pro" }, { "end_idx": 24, "entity": "丙种球蛋白", "start_idx": 20, "type": "dru" } ]
具体为:①急性期后临床表现为肾小球肾炎或蛋白尿、血尿者用雷公藤多苷治疗;②急性期后表现为肾病综合征者用泼尼松治疗;③有条件者行肾活检检查,根据病理改变调整治疗方案,如有新月体形成或局灶节段性肾小球硬化者加用甲基泼尼松龙和(或)环磷酰胺冲击治疗;④对治疗无反应、仍呈肾功能进行性减退者停用激素和免疫抑制剂,以对症和肾替代疗法为主。
[ { "end_idx": 18, "entity": "肾小球肾炎", "start_idx": 14, "type": "dis" }, { "end_idx": 22, "entity": "蛋白尿", "start_idx": 20, "type": "sym" }, { "end_idx": 21, "entity": "蛋白", "start_idx": 20, "type": "bod" }, { "end_idx": 22, "entity": "尿", "start_idx": 22, "type": "bod" }, { "end_idx": 25, "entity": "血尿", "start_idx": 24, "type": "sym" }, { "end_idx": 24, "entity": "血", "start_idx": 24, "type": "bod" }, { "end_idx": 25, "entity": "尿", "start_idx": 25, "type": "bod" }, { "end_idx": 32, "entity": "雷公藤多苷", "start_idx": 28, "type": "dru" }, { "end_idx": 48, "entity": "肾病综合征", "start_idx": 44, "type": "dis" }, { "end_idx": 53, "entity": "泼尼松", "start_idx": 51, "type": "dru" }, { "end_idx": 65, "entity": "肾活检", "start_idx": 63, "type": "pro" }, { "end_idx": 88, "entity": "新月体形成", "start_idx": 84, "type": "sym" }, { "end_idx": 86, "entity": "新月体", "start_idx": 84, "type": "bod" }, { "end_idx": 99, "entity": "局灶节段性肾小球硬化", "start_idx": 90, "type": "sym" }, { "end_idx": 97, "entity": "肾小球", "start_idx": 95, "type": "bod" }, { "end_idx": 108, "entity": "甲基泼尼松龙", "start_idx": 103, "type": "dru" }, { "end_idx": 116, "entity": "环磷酰胺", "start_idx": 113, "type": "dru" }, { "end_idx": 120, "entity": "冲击治疗", "start_idx": 117, "type": "pro" }, { "end_idx": 139, "entity": "肾功能进行性减退", "start_idx": 132, "type": "sym" }, { "end_idx": 132, "entity": "肾", "start_idx": 132, "type": "bod" }, { "end_idx": 144, "entity": "激素", "start_idx": 143, "type": "dru" }, { "end_idx": 150, "entity": "免疫抑制剂", "start_idx": 146, "type": "dru" }, { "end_idx": 160, "entity": "肾替代疗法", "start_idx": 156, "type": "pro" } ]
年龄小、有胃肠道前驱症状者,病死率低,肾功能恢复好,终末肾发生率低;而年龄>3岁、无胃肠道前驱症状、无尿期>3天、有神经系统症状者、家族性发病者预后差。
[ { "end_idx": 11, "entity": "胃肠道前驱症状", "start_idx": 5, "type": "sym" }, { "end_idx": 7, "entity": "胃肠道", "start_idx": 5, "type": "bod" }, { "end_idx": 19, "entity": "肾", "start_idx": 19, "type": "bod" }, { "end_idx": 28, "entity": "肾", "start_idx": 28, "type": "bod" }, { "end_idx": 48, "entity": "胃肠道前驱症状", "start_idx": 42, "type": "sym" }, { "end_idx": 44, "entity": "胃肠道", "start_idx": 42, "type": "bod" }, { "end_idx": 51, "entity": "尿", "start_idx": 51, "type": "bod" }, { "end_idx": 63, "entity": "神经系统症状", "start_idx": 58, "type": "sym" }, { "end_idx": 61, "entity": "神经系统", "start_idx": 58, "type": "bod" } ]
约有15%病例发展成慢性肾衰竭、持续高血压或神经系统后遗症。
[ { "end_idx": 14, "entity": "慢性肾衰竭", "start_idx": 10, "type": "dis" }, { "end_idx": 20, "entity": "持续高血压", "start_idx": 16, "type": "dis" }, { "end_idx": 28, "entity": "神经系统后遗症", "start_idx": 22, "type": "dis" } ]
从而引起巨幼红细胞性贫血。
[ { "end_idx": 11, "entity": "巨幼红细胞性贫血", "start_idx": 4, "type": "dis" } ]
肤色可苍黄,口唇、睑结膜、甲床苍白,头发黄、细、干、稀疏,面水肿。
[ { "end_idx": 4, "entity": "肤色可苍黄", "start_idx": 0, "type": "sym" }, { "end_idx": 0, "entity": "肤", "start_idx": 0, "type": "bod" }, { "end_idx": 16, "entity": "口唇、睑结膜、甲床苍白", "start_idx": 6, "type": "sym" }, { "end_idx": 7, "entity": "口唇", "start_idx": 6, "type": "bod" }, { "end_idx": 11, "entity": "睑结膜", "start_idx": 9, "type": "bod" }, { "end_idx": 14, "entity": "甲床", "start_idx": 13, "type": "bod" }, { "end_idx": 27, "entity": "头发黄、细、干、稀疏", "start_idx": 18, "type": "sym" }, { "end_idx": 19, "entity": "头发", "start_idx": 18, "type": "bod" }, { "end_idx": 31, "entity": "面水肿", "start_idx": 29, "type": "sym" }, { "end_idx": 29, "entity": "面", "start_idx": 29, "type": "bod" } ]
患儿可出现烦躁不安、疲乏无力、表情呆滞、反应迟钝、两眼直视、食欲差、嗜睡等症。
[ { "end_idx": 8, "entity": "烦躁不安", "start_idx": 5, "type": "sym" }, { "end_idx": 13, "entity": "疲乏无力", "start_idx": 10, "type": "sym" }, { "end_idx": 18, "entity": "表情呆滞", "start_idx": 15, "type": "sym" }, { "end_idx": 23, "entity": "反应迟钝", "start_idx": 20, "type": "sym" }, { "end_idx": 28, "entity": "两眼直视", "start_idx": 25, "type": "sym" }, { "end_idx": 26, "entity": "两眼", "start_idx": 25, "type": "bod" }, { "end_idx": 32, "entity": "食欲差", "start_idx": 30, "type": "sym" }, { "end_idx": 35, "entity": "嗜睡", "start_idx": 34, "type": "sym" } ]
心前区可闻及吹风样收缩期杂音。
[ { "end_idx": 2, "entity": "心前区", "start_idx": 0, "type": "bod" }, { "end_idx": 13, "entity": "心前区可闻及吹风样收缩期杂音", "start_idx": 0, "type": "sym" } ]
血小板严重降低时,皮肤可出现瘀点、瘀斑。
[ { "end_idx": 6, "entity": "血小板严重降低", "start_idx": 0, "type": "sym" }, { "end_idx": 2, "entity": "血小板", "start_idx": 0, "type": "bod" }, { "end_idx": 18, "entity": "皮肤可出现瘀点、瘀斑", "start_idx": 9, "type": "sym" }, { "end_idx": 10, "entity": "皮肤", "start_idx": 9, "type": "bod" } ]
可出现动作缓慢、手足无意识运动、头部及肢体颤动。
[ { "end_idx": 6, "entity": "动作缓慢", "start_idx": 3, "type": "sym" }, { "end_idx": 14, "entity": "手足无意识运动", "start_idx": 8, "type": "sym" }, { "end_idx": 8, "entity": "手", "start_idx": 8, "type": "bod" }, { "end_idx": 9, "entity": "足", "start_idx": 9, "type": "bod" }, { "end_idx": 22, "entity": "头部及肢体颤动", "start_idx": 16, "type": "sym" }, { "end_idx": 17, "entity": "头部", "start_idx": 16, "type": "bod" }, { "end_idx": 20, "entity": "肢体", "start_idx": 19, "type": "bod" } ]
震颤初见于手、唇、舌,因反复震颤而舌系带可出现溃疡;继而上肢、头部、甚至全身。
[ { "end_idx": 9, "entity": "震颤初见于手、唇、舌", "start_idx": 0, "type": "sym" }, { "end_idx": 5, "entity": "手", "start_idx": 5, "type": "bod" }, { "end_idx": 7, "entity": "唇", "start_idx": 7, "type": "bod" }, { "end_idx": 9, "entity": "舌", "start_idx": 9, "type": "bod" }, { "end_idx": 15, "entity": "反复震颤", "start_idx": 12, "type": "sym" }, { "end_idx": 24, "entity": "舌系带可出现溃疡", "start_idx": 17, "type": "sym" }, { "end_idx": 19, "entity": "舌系带", "start_idx": 17, "type": "bod" }, { "end_idx": 29, "entity": "上肢", "start_idx": 28, "type": "bod" }, { "end_idx": 32, "entity": "头部", "start_idx": 31, "type": "bod" }, { "end_idx": 37, "entity": "全身", "start_idx": 36, "type": "bod" } ]
重症病例可见四肢屈曲,踝阵挛。
[ { "end_idx": 7, "entity": "四肢", "start_idx": 6, "type": "bod" }, { "end_idx": 9, "entity": "四肢屈曲", "start_idx": 6, "type": "sym" }, { "end_idx": 11, "entity": "踝", "start_idx": 11, "type": "bod" }, { "end_idx": 13, "entity": "踝阵挛", "start_idx": 11, "type": "sym" } ]
长期缺乏未予补充者,可出现智力障碍。
[ { "end_idx": 16, "entity": "智力障碍", "start_idx": 13, "type": "sym" } ]
如喂养不当应予以纠正,慢性腹泻应予以治疗。
[ { "end_idx": 14, "entity": "慢性腹泻", "start_idx": 11, "type": "sym" }, { "end_idx": 13, "entity": "腹", "start_idx": 13, "type": "bod" } ]
治疗期间要适当加服铁剂以供造红细胞所需。
[ { "end_idx": 8, "entity": "加服", "start_idx": 7, "type": "pro" }, { "end_idx": 10, "entity": "铁剂", "start_idx": 9, "type": "dru" }, { "end_idx": 16, "entity": "红细胞", "start_idx": 14, "type": "bod" } ]
严重巨幼红细胞贫血病儿在治疗开始48小时,血钾可突然下降,加之心肌因慢性缺氧,可发生突然死亡,严重巨幼红细胞性贫血患儿,治疗时应同时补充钾盐。
[ { "end_idx": 8, "entity": "严重巨幼红细胞贫血", "start_idx": 0, "type": "dis" }, { "end_idx": 27, "entity": "血钾可突然下降", "start_idx": 21, "type": "sym" }, { "end_idx": 22, "entity": "血钾", "start_idx": 21, "type": "bod" }, { "end_idx": 45, "entity": "心肌因慢性缺氧,可发生突然死亡", "start_idx": 31, "type": "sym" }, { "end_idx": 32, "entity": "心肌", "start_idx": 31, "type": "bod" }, { "end_idx": 56, "entity": "严重巨幼红细胞性贫血", "start_idx": 47, "type": "dis" }, { "end_idx": 69, "entity": "钾盐", "start_idx": 68, "type": "dru" } ]
VLBW的肺动脉缺乏平滑肌,致使肺动脉舒缩困难,容易引发持续性肺动脉高压。
[ { "end_idx": 3, "entity": "VLBW", "start_idx": 0, "type": "dis" }, { "end_idx": 12, "entity": "肺动脉缺乏平滑肌", "start_idx": 5, "type": "sym" }, { "end_idx": 7, "entity": "肺动脉", "start_idx": 5, "type": "bod" }, { "end_idx": 12, "entity": "平滑肌", "start_idx": 10, "type": "bod" }, { "end_idx": 22, "entity": "肺动脉舒缩困难", "start_idx": 16, "type": "sym" }, { "end_idx": 18, "entity": "肺动脉", "start_idx": 16, "type": "bod" }, { "end_idx": 35, "entity": "持续性肺动脉高压", "start_idx": 28, "type": "dis" } ]
出生早期动脉导管处于可开放性状态,容易因缺氧等病理原因造成动脉导管重新开放,甚至在短期内引起充血性心力衰竭。
[ { "end_idx": 7, "entity": "动脉导管", "start_idx": 4, "type": "bod" }, { "end_idx": 21, "entity": "缺氧", "start_idx": 20, "type": "sym" }, { "end_idx": 36, "entity": "动脉导管重新开放", "start_idx": 29, "type": "sym" }, { "end_idx": 32, "entity": "动脉导管", "start_idx": 29, "type": "bod" }, { "end_idx": 52, "entity": "充血性心力衰竭", "start_idx": 46, "type": "dis" } ]
脑室室管膜下生发层在侧脑室周围长得很厚,而此处非常容易引起脑室内室管膜下出血,是脑室内出血好发部位。
[ { "end_idx": 18, "entity": "脑室室管膜下生发层在侧脑室周围长得很厚", "start_idx": 0, "type": "sym" }, { "end_idx": 8, "entity": "脑室室管膜下生发层", "start_idx": 0, "type": "bod" }, { "end_idx": 14, "entity": "侧脑室周围", "start_idx": 10, "type": "bod" }, { "end_idx": 37, "entity": "脑室内室管膜下出血", "start_idx": 29, "type": "dis" }, { "end_idx": 44, "entity": "脑室内出血", "start_idx": 40, "type": "dis" } ]
VLBW可以在经肠道喂养前就比较容易发生坏死性小肠结肠炎(NEC)。
[ { "end_idx": 3, "entity": "VLBW", "start_idx": 0, "type": "dis" }, { "end_idx": 9, "entity": "肠道", "start_idx": 8, "type": "bod" }, { "end_idx": 27, "entity": "坏死性小肠结肠炎", "start_idx": 20, "type": "dis" }, { "end_idx": 31, "entity": "NEC", "start_idx": 29, "type": "dis" } ]
VLBW在生后1~2天内处于一种非少尿性高钾血症状态,并在生后24小时达到高峰,高钾血症的原因可能与肾小球滤过率低、肾小管重吸收能力差以及红细胞Na+-K+-ATP酶的活性较高有关。
[ { "end_idx": 3, "entity": "VLBW", "start_idx": 0, "type": "dis" }, { "end_idx": 23, "entity": "非少尿性高钾血症", "start_idx": 16, "type": "dis" }, { "end_idx": 43, "entity": "高钾血症", "start_idx": 40, "type": "dis" }, { "end_idx": 56, "entity": "肾小球滤过率低", "start_idx": 50, "type": "sym" }, { "end_idx": 52, "entity": "肾小球", "start_idx": 50, "type": "bod" }, { "end_idx": 66, "entity": "肾小管重吸收能力差", "start_idx": 58, "type": "sym" }, { "end_idx": 60, "entity": "肾小管", "start_idx": 58, "type": "bod" }, { "end_idx": 87, "entity": "红细胞Na+-K+-ATP酶的活性较高", "start_idx": 69, "type": "sym" }, { "end_idx": 82, "entity": "红细胞Na+-K+-ATP酶", "start_idx": 69, "type": "bod" } ]
糖耐受量低,容易引起高血糖和糖尿,高血糖可以造成高渗性利尿,从而有引起颅内出血的危险,并可引起呼吸暂停和大脑抑制。
[ { "end_idx": 4, "entity": "糖耐受量低", "start_idx": 0, "type": "sym" }, { "end_idx": 12, "entity": "高血糖", "start_idx": 10, "type": "dis" }, { "end_idx": 15, "entity": "糖尿", "start_idx": 14, "type": "sym" }, { "end_idx": 14, "entity": "糖", "start_idx": 14, "type": "bod" }, { "end_idx": 15, "entity": "尿", "start_idx": 15, "type": "bod" }, { "end_idx": 19, "entity": "高血糖", "start_idx": 17, "type": "dis" }, { "end_idx": 28, "entity": "高渗性利尿", "start_idx": 24, "type": "sym" }, { "end_idx": 28, "entity": "尿", "start_idx": 28, "type": "bod" }, { "end_idx": 38, "entity": "颅内出血", "start_idx": 35, "type": "dis" }, { "end_idx": 50, "entity": "呼吸暂停", "start_idx": 47, "type": "sym" }, { "end_idx": 55, "entity": "大脑抑制", "start_idx": 52, "type": "sym" }, { "end_idx": 53, "entity": "大脑", "start_idx": 52, "type": "bod" } ]
半数以上患儿伴有杓状软骨软化,亦可由于会厌软骨软化或杓状会厌襞过短所致。
[ { "end_idx": 13, "entity": "杓状软骨软化", "start_idx": 8, "type": "dis" }, { "end_idx": 24, "entity": "会厌软骨软化", "start_idx": 19, "type": "dis" }, { "end_idx": 32, "entity": "杓状会厌襞过短", "start_idx": 26, "type": "dis" } ]
多数患儿于新生儿期出现喘鸣症状,有的则于出生后1~2个月,因感冒或腹泻后逐渐出现。
[ { "end_idx": 12, "entity": "喘鸣", "start_idx": 11, "type": "sym" }, { "end_idx": 31, "entity": "感冒", "start_idx": 30, "type": "dis" }, { "end_idx": 34, "entity": "腹泻", "start_idx": 33, "type": "sym" }, { "end_idx": 33, "entity": "腹", "start_idx": 33, "type": "bod" } ]
以吸气性喉鸣为主。
[ { "end_idx": 5, "entity": "吸气性喉鸣", "start_idx": 1, "type": "sym" }, { "end_idx": 4, "entity": "喉", "start_idx": 4, "type": "bod" } ]
直接喉镜检查可确诊,并明确畸形的位置及性质。
[ { "end_idx": 5, "entity": "喉镜检查", "start_idx": 2, "type": "pro" }, { "end_idx": 14, "entity": "畸形", "start_idx": 13, "type": "dis" } ]
本病应注意与喉蹼、婴儿低钙性喉痉挛、喉部肿瘤及继发性喉软骨软化等鉴别。
[ { "end_idx": 7, "entity": "喉蹼", "start_idx": 6, "type": "dis" }, { "end_idx": 16, "entity": "婴儿低钙性喉痉挛", "start_idx": 9, "type": "dis" }, { "end_idx": 21, "entity": "喉部肿瘤", "start_idx": 18, "type": "dis" }, { "end_idx": 30, "entity": "继发性喉软骨软化", "start_idx": 23, "type": "dis" } ]
应强调精心护理和合理喂养,注意避免呛咳。
[ { "end_idx": 18, "entity": "呛咳", "start_idx": 17, "type": "sym" } ]
适当补充钙及维生素D。
[ { "end_idx": 4, "entity": "钙", "start_idx": 4, "type": "dru" }, { "end_idx": 9, "entity": "维生素D", "start_idx": 6, "type": "dru" } ]
极少数症状特别严重者可给予鼻气管插管或气管造口术。
[ { "end_idx": 17, "entity": "鼻气管插管", "start_idx": 13, "type": "pro" }, { "end_idx": 23, "entity": "气管造口术", "start_idx": 19, "type": "pro" } ]
另外14%的患儿主动脉瓣不分叶,因而在增厚的单瓣上有一泪滴样偏心小孔。
[ { "end_idx": 14, "entity": "主动脉瓣不分叶", "start_idx": 8, "type": "sym" }, { "end_idx": 11, "entity": "主动脉瓣", "start_idx": 8, "type": "bod" }, { "end_idx": 33, "entity": "增厚的单瓣上有一泪滴样偏心小孔", "start_idx": 19, "type": "sym" }, { "end_idx": 23, "entity": "单瓣", "start_idx": 22, "type": "bod" }, { "end_idx": 31, "entity": "心", "start_idx": 31, "type": "bod" } ]
由于左心室高压,可有心内膜显著增厚,从而进一步损害左心室功能(继发性心内膜弹力纤维增生症)。
[ { "end_idx": 6, "entity": "左心室高压", "start_idx": 2, "type": "sym" }, { "end_idx": 4, "entity": "左心室", "start_idx": 2, "type": "bod" }, { "end_idx": 16, "entity": "心内膜显著增厚", "start_idx": 10, "type": "sym" }, { "end_idx": 12, "entity": "心内膜", "start_idx": 10, "type": "bod" }, { "end_idx": 29, "entity": "损害左心室功能", "start_idx": 23, "type": "sym" }, { "end_idx": 27, "entity": "左心室", "start_idx": 25, "type": "bod" }, { "end_idx": 43, "entity": "继发性心内膜弹力纤维增生症", "start_idx": 31, "type": "dis" } ]
本病在西方国家颇为多见,可占先心的3%~6%,但我国发病率似较低。
[ { "end_idx": 15, "entity": "先心", "start_idx": 14, "type": "dis" } ]
这也可解释为什么中度主动脉瓣狭窄患儿运动时可出现心绞痛、胸前导联ST段压低及T波倒置。
[ { "end_idx": 15, "entity": "中度主动脉瓣狭窄", "start_idx": 8, "type": "dis" }, { "end_idx": 26, "entity": "心绞痛", "start_idx": 24, "type": "sym" }, { "end_idx": 24, "entity": "心", "start_idx": 24, "type": "bod" }, { "end_idx": 41, "entity": "胸前导联ST段压低及T波倒置", "start_idx": 28, "type": "sym" }, { "end_idx": 31, "entity": "胸前导联", "start_idx": 28, "type": "pro" } ]
主动脉瓣狭窄患儿偶可发生猝死,其原因几乎可肯定为室性纤颤,后者的原因也可能是由缺血或缺血样损害所引起。
[ { "end_idx": 5, "entity": "主动脉瓣狭窄", "start_idx": 0, "type": "dis" }, { "end_idx": 27, "entity": "室性纤颤", "start_idx": 24, "type": "dis" }, { "end_idx": 40, "entity": "缺血", "start_idx": 39, "type": "sym" }, { "end_idx": 40, "entity": "血", "start_idx": 40, "type": "bod" }, { "end_idx": 46, "entity": "缺血样损害", "start_idx": 42, "type": "sym" }, { "end_idx": 43, "entity": "血", "start_idx": 43, "type": "bod" } ]
主动脉瓣狭窄的另一症状为晕厥,通常发生在劳力或长期站立后。
[ { "end_idx": 5, "entity": "主动脉瓣狭窄", "start_idx": 0, "type": "dis" }, { "end_idx": 13, "entity": "晕厥", "start_idx": 12, "type": "sym" } ]
较轻型患者可引起左心室肥厚,但在休息或运动时无缺血症状。
[ { "end_idx": 10, "entity": "左心室", "start_idx": 8, "type": "bod" }, { "end_idx": 12, "entity": "左心室肥厚", "start_idx": 8, "type": "sym" }, { "end_idx": 24, "entity": "缺血", "start_idx": 23, "type": "sym" }, { "end_idx": 24, "entity": "血", "start_idx": 24, "type": "bod" } ]
严重主动脉瓣狭窄患儿生后如卵圆孔关闭,左心房压升高,左心室心输出量可维持,但可出现肺水肿的表现。
[ { "end_idx": 7, "entity": "严重主动脉瓣狭窄", "start_idx": 0, "type": "dis" }, { "end_idx": 17, "entity": "卵圆孔关闭", "start_idx": 13, "type": "sym" }, { "end_idx": 15, "entity": "卵圆孔", "start_idx": 13, "type": "bod" }, { "end_idx": 24, "entity": "左心房压升高", "start_idx": 19, "type": "sym" }, { "end_idx": 21, "entity": "左心房", "start_idx": 19, "type": "bod" }, { "end_idx": 28, "entity": "左心室", "start_idx": 26, "type": "bod" }, { "end_idx": 29, "entity": "心", "start_idx": 29, "type": "bod" }, { "end_idx": 43, "entity": "肺水肿", "start_idx": 41, "type": "dis" } ]
杂音往往为本病的首要体征,婴儿期大多在胸骨左缘中部最响,有时与室缺很难鉴别;其响度决定于左室的排出量,如因心力衰竭而减低,则杂音趋轻柔甚至消失。
[ { "end_idx": 1, "entity": "杂音", "start_idx": 0, "type": "sym" }, { "end_idx": 22, "entity": "胸骨左缘", "start_idx": 19, "type": "bod" }, { "end_idx": 32, "entity": "室缺", "start_idx": 31, "type": "dis" }, { "end_idx": 45, "entity": "左室", "start_idx": 44, "type": "bod" }, { "end_idx": 56, "entity": "心力衰竭", "start_idx": 53, "type": "dis" }, { "end_idx": 63, "entity": "杂音", "start_idx": 62, "type": "sym" } ]
胸部X线表现心脏可不大,或有左心室、左心房增大的表现,升主动脉可示狭窄后扩张。
[ { "end_idx": 3, "entity": "胸部X线", "start_idx": 0, "type": "pro" }, { "end_idx": 7, "entity": "心脏", "start_idx": 6, "type": "bod" }, { "end_idx": 22, "entity": "左心室、左心房增大", "start_idx": 14, "type": "sym" }, { "end_idx": 16, "entity": "左心室", "start_idx": 14, "type": "bod" }, { "end_idx": 20, "entity": "左心房", "start_idx": 18, "type": "bod" }, { "end_idx": 37, "entity": "升主动脉可示狭窄后扩张", "start_idx": 27, "type": "sym" }, { "end_idx": 30, "entity": "升主动脉", "start_idx": 27, "type": "bod" } ]
超声可显示主动脉瓣增厚、开放受限,呈幕顶样运动,两叶主动脉瓣,左心室壁增厚和左心室的等容收缩期延长。
[ { "end_idx": 48, "entity": "超声可显示主动脉瓣增厚、开放受限,呈幕顶样运动,两叶主动脉瓣,左心室壁增厚和左心室的等容收缩期延长", "start_idx": 0, "type": "sym" }, { "end_idx": 1, "entity": "超声", "start_idx": 0, "type": "pro" }, { "end_idx": 8, "entity": "主动脉瓣", "start_idx": 5, "type": "bod" }, { "end_idx": 29, "entity": "两叶主动脉瓣", "start_idx": 24, "type": "bod" }, { "end_idx": 34, "entity": "左心室壁", "start_idx": 31, "type": "bod" }, { "end_idx": 40, "entity": "左心室", "start_idx": 38, "type": "bod" } ]
主动脉根部造影可显示主动脉瓣的活动度,有无反流,瓣环大小和狭窄后的扩张情况。
[ { "end_idx": 6, "entity": "主动脉根部造影", "start_idx": 0, "type": "pro" }, { "end_idx": 13, "entity": "主动脉瓣", "start_idx": 10, "type": "bod" }, { "end_idx": 22, "entity": "反流", "start_idx": 21, "type": "sym" }, { "end_idx": 25, "entity": "瓣环", "start_idx": 24, "type": "bod" }, { "end_idx": 30, "entity": "狭窄", "start_idx": 29, "type": "sym" } ]
急性鼻窦炎以咳嗽和分泌物增多为主要症状。
[ { "end_idx": 4, "entity": "急性鼻窦炎", "start_idx": 0, "type": "dis" }, { "end_idx": 7, "entity": "咳嗽", "start_idx": 6, "type": "sym" }, { "end_idx": 13, "entity": "分泌物增多", "start_idx": 9, "type": "sym" }, { "end_idx": 11, "entity": "分泌物", "start_idx": 9, "type": "bod" } ]
咳嗽、流涕持续超过30天者诊断亚急性鼻窦炎。
[ { "end_idx": 1, "entity": "咳嗽", "start_idx": 0, "type": "sym" }, { "end_idx": 4, "entity": "流涕", "start_idx": 3, "type": "sym" }, { "end_idx": 4, "entity": "涕", "start_idx": 4, "type": "bod" }, { "end_idx": 20, "entity": "亚急性鼻窦炎", "start_idx": 15, "type": "dis" } ]