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Guideline sources The following summarized guidelines for the evaluation and management of adrenal incidentaloma (AI) are prepared by our editorial team based on guidelines from the Canadian Urological Association (CUA 2023), the American Association of Endocrine Surgeons (AAES 2022), the American College of Radiology (ACR 2021), the European Society of Endocrinology (ESE/ENSAT 2016), and the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES 2013). 1 2 3 4 5 6 7 8 9 Definition AI refers to an adrenal mass unexpectedly detected through an imaging procedure performed for reasons unrelated to adrenal pathology. 6 Epidemiology AIs are caused due to hormone excess (adenoma, carcinoma, pheochromocytoma, congenital adrenal hyperplasia, massive macronodular adrenal disease, nodular variant of Cushing's disease), no hormone excess (adenoma, myelolipoma, neuroblastoma, ganglioneuroma, hemangioma, carcinoma, metastasis, cyst, hemorrhage, granuloma, amyloidosis, and infiltrative disease. 7 https://web.pathway.md/diseases/recl3lB15yicFTcCE 1/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway Disease course A cluster of different pathologies in the adrenal gland due to physiological process of aging detected incidentally during an imaging procedure is known as AI. The majority of tumors are non-functioning, but incidentaloma with hormonal and malignancy evolution may cause life-threatening complications. 8 Prognosis and risk of recurrence AIs are not associated with an increase in mortality, except for adrenal metastasis. 9 Guidelines 1. Classification and risk stratification Risk stratification: consider using washout characteristics on an adrenal protocol CT to stratify the risk of malignancy for adrenal nodules when the non-contrast HU is > 10 and other clinical risk factors for malignancy are not present. Recognize that adrenal protocol CT does not improve diagnostic accuracy for nodules with non-contrast HU < 10 nor does it improve evaluation for pheochromocytoma. C Show 2 more 2. Diagnostic investigations History and physical examination: Elicit a focused history and perform a physical examination to identify signs/symptoms of adrenal hormone excess, adrenal malignancy, and/or extra-adrenal malignancy in patients with an AI. E Set a low threshold for a multidisciplinary review by endocrinologists, surgeons, and radiologists when the imaging is not consistent with a benign lesion, there is evidence of hormone hypersecretion, the tumor has grown significantly during follow-up imaging, or adrenal surgery is being considered. E Diagnostic imaging: As per CUA 2023 guidelines: Obtain non-contrast CT as first-line imaging to distinguish benign lesions from those requiring further radiological investigation in patients with an indeterminate incidental adrenal mass. B Obtain washout CT or chemical-shift MRI as second-line imaging in patients continuing to have an indeterminate adrenal mass on non-contrast CT. B As per AAES 2022 guidelines, consider obtaining adrenal protocol CT to determine washout characteristics in order to stratify the risk of malignancy of adrenal nodules. C As per ACR 2021 guidelines, do not obtain imaging for the initial evaluation of patients with no prior history of malignancy and an incidentally detected indeterminate adrenal mass < 1 cm. D https://web.pathway.md/diseases/recl3lB15yicFTcCE 2/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway Show 4 more As per ESE 2016 guidelines, obtain adrenal-specific imaging to determine if the mass is homogeneous and lipid-rich (therefore benign) in all patients with AIs. B Show 2 more Dexamethasone suppression test: As per CUA 2023 guidelines, evaluate for autonomous cortisol secretion in all patients with AIs. B Obtain a 1-mg dexamethasone suppression test to identify autonomous cortisol secretion when clinically appropriate. B As per AAES 2022 guidelines, obtain biochemical testing to identify autonomous cortisol secretion in all patients with an AI 1 cm. B As per ESE 2016 guidelines, obtain a 1-mg overnight dexamethasone suppression test to exclude cortisol excess in all patients with AIs. B Show 2 more Metanephrine assays: As per CUA 2023 guidelines: Obtain plasma or 24-hour urinary metanephrines to evaluate for pheochromocytoma in patients with an AI displaying 10 HUs on non-contrast CT or having signs/symptoms of catecholamine excess. B Avoid obtaining evaluation for pheochromocytoma in patients with unequivocal adrenocortical adenomas confirmed on unenhanced CT (< 10 HUs) and no signs or symptoms of adrenergic excess. D As per AAES 2022 guidelines, obtain biochemical evaluation for pheochromocytoma in patients with non-contrast adrenal CT findings of HU > 10. B As per ESE 2016 guidelines, obtain plasma free metanephrines or urinary fractionated metanephrines to exclude pheochromocytoma. A Aldosterone-to-renin ratio: As per CUA 2023 guidelines, obtain aldosterone-to-renin ratio to evaluate for primary aldosteronism in patients with an AI and hypertension and/or hypokalemia. B As per AAES 2022 guidelines, obtain biochemical evaluation for primary aldosteronism in patients with hypertension or hypokalemia. B As per ESE 2016 guidelines, obtain aldosterone-to-renin ratio to evaluate for primary aldosteronism in patients with concomitant hypertension or unexplained hypokalemia. A Hormonal evaluation: As per CUA 2023 guidelines, obtain serum testing for excess androgen in patients with suspected adrenocortical carcinoma and/or when clinical signs of virilization are present. E As per AAES 2022 guidelines, obtain a directed hormonal evaluation in patients with an adrenal mass regardless of history of extra-adrenal malignancy. B https://web.pathway.md/diseases/recl3lB15yicFTcCE 3/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway As per ESE 2016 guidelines, consider measuring sex hormones and steroid precursors in patients with clinical or imaging features suggestive of adrenocortical carcinoma. B 3. Diagnostic procedures Adrenal mass biopsy: As per CUA 2023 guidelines, do not perform adrenal mass biopsy routinely for the evaluation of an AI. D As per AAES 2022 guidelines, consider performing an image-guided biopsy of a radiographically indeterminate mass, only after confirming a lack of hormone excess, if the results will change overall disease management. B As per ACR 2021 guidelines, perform adrenal gland biopsy or obtain FDG-PET/CT from the skull base to mid-thigh in patients with a history of malignancy and an indeterminate adrenal mass 4 cm without diagnostic benign imaging characteristics on initial images. B As per ESE 2016 guidelines, consider performing adrenal mass biopsy if all of the following criteria are met: the lesion is hormonally inactive (pheochromocytoma has been excluded) the lesion has not been conclusively characterized as benign by imaging determination of histology will affect the management. C 4. Therapeutic procedures Ablation and stereotactic radiation: avoid performing ablation or stereotactic radiation as an alternative to adrenalectomy in patients with adrenal lesions because of inadequate data supporting these modalities. Include surgeons in the decision-making early in the treatment algorithm. D 5. Perioperative care Perioperative corticosteroids: administer perioperative corticosteroids at major surgical stress doses in all patients undergoing surgery for an adrenal tumor when there is evidence of 'possible autonomous cortisol secretion', as evidenced by a lack of cortisol suppression to < 50 nmol/L during the 1-mg dexamethasone overnight suppression test. A 6. Surgical interventions Indications for adrenalectomy, nonfunctioning tumors: As per CUA 2023 guidelines: Consider performing adrenalectomy in patients with AIs growing > 5 mm/year after repeating a functional workup. C https://web.pathway.md/diseases/recl3lB15yicFTcCE 4/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway Ensure shared decision-making for the management (repeat imaging in 3-6 months versus surgical resection) of patients with indeterminate nonfunctioning adrenal lesions. E As per AAES 2022 guidelines, avoid performing resection of a myelolipoma or adrenal cyst with pathognomonic imaging features to improve the patient's quality of life unless there are symptoms of mass effect. D As per ENSAT/ESE 2016 guidelines, do not perform adrenalectomy in patients with an asymptomatic, nonfunctioning unilateral adrenal mass and obvious benign features on imaging. D Indications for adrenalectomy, hormone-secreting tumors: As per CUA 2023 guidelines, perform unilateral adrenalectomy of the affected adrenal gland, with a minimally invasive technique when feasible, in patients with unilateral cortisol-secreting adrenal masses and clinically apparent Cushing's syndrome. E Show 2 more As per ENSAT/ESE 2016 guidelines, perform adrenalectomy as the standard of care in patients with unilateral adrenal tumors with clinically significant hormone excess. Individualize the decision for surgery according to age, degree of cortisol excess, general health, comorbidities, and patient preferences. Confirm that cortisol excess is independent of ACTH. A Indications for adrenalectomy, malignant tumors: As per CUA 2023 guidelines: Consider performing minimally invasive adrenalectomy in patients with suspected adrenocortical carcinomas suitable for safe resection without rupturing the tumor capsule. C Consider performing open adrenalectomy in patients with larger adrenocortical carcinomas or presenting with locally advanced tumors, lymph node metastases, or tumor thrombus in the renal vein/IVC. B As per ENSAT/ESE 2016 guidelines: Perform open adrenalectomy in patients with unilateral adrenal masses with radiological findings suspicious of malignancy and signs of local invasion. B Consider performing laparoscopic adrenalectomy in patients with unilateral adrenal masses with radiological findings suspicious of malignancy and a diameter 6 cm but without evidence of local invasion. C Technical considerations for adrenalectomy: As per AAES 2022 guidelines, perform minimally invasive adrenalectomy over open adrenalectomy for improved perioperative morbidity when patient and tumor characteristics are appropriate. B Show 2 more As per SAGES 2013 guidelines, prefer minimally invasive adrenalectomy over open surgery for all non-primary adrenal cancer pathology due to less postoperative pain, shorter hospital stay, earlier recovery, and similar long-term outcomes. B Show 8 more https://web.pathway.md/diseases/recl3lB15yicFTcCE 5/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway 7. Specific circumstances Pediatric, pregnant and elderly patients: obtain an urgent assessment of the adrenal mass in pediatric patients, adolescent patients, pregnant, and < 40 years old adult patients because of a higher likelihood of malignancy. A Show 2 more Patients with bilateral adrenal incidentalomas (evaluation): assess bilateral adrenal masses separately, according to the same imaging protocol as for unilateral adrenal masses, to establish if either or both masses are benign or malignant. A Show 2 more Patients with bilateral adrenal incidentalomas, management: As per ENSAT/ESE 2016 guidelines, consider applying the same indications for surgery and follow-up identical to the recommendations in patients with unilateral AI. C Show 2 more As per SAGES 2013 guidelines: Consider preferring the posterior retroperitoneal approach to eliminate patient repositioning during bilateral adrenalectomies. C Consider performing laparoscopic cortical-sparing surgery as the procedure of choice in patients requiring bilateral adrenalectomy (such as for hereditary pheochromocytomas). C Patients with autonomous cortisol secretion: As per AAES 2022 guidelines, perform laparoscopic adrenalectomy for anticipated significant improvements in cardiometabolic comorbidities in patients with mild autonomous cortisol secretion secondary to a unilateral adenoma. B Show 3 more As per ESE 2016 guidelines, do not view 'autonomous cortisol secretion' as a condition with a high risk for the development of overt Cushing's syndrome. D Show 3 more Patients with extra-adrenal malignancy: obtain plasma or urinary metanephrines to exclude pheochromocytoma in patients with extra-adrenal malignancy with an indeterminate mass, even if the adrenal mass is likely to be a metastasis. Consider obtaining additional hormonal work-up using an individualized approach. A Show 3 more Patients with adrenal metastases: As per AAES 2022 guidelines, consider offering resection of adrenal metastases, after multidisciplinary review, to improve survival in highly selected patients. C As per SAGES 2013 guidelines, consider using a laparoscopic approach, by a surgeon skilled in advanced laparoscopy and adrenal surgery, in patients with solitary adrenal metastases without evidence of local invasion. C Convert to an open approach if a local invasion is found intraoperatively. B https://web.pathway.md/diseases/recl3lB15yicFTcCE 6/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway 8. Follow-up and surveillance Serial imaging assessment: As per CUA 2023 guidelines, do not obtain further follow-up imaging in patients with benign nonfunctioning adenomas < 4 cm, myelolipomas, and other small masses containing macroscopic fat detected on the initial workup for an AI. D Show 2 more As per AAES 2022 guidelines: Do not obtain routine scheduled follow-up of a nonfunctional adrenal nodule (size < 4 cm) with benign imaging characteristics and non-contrast HU < 10 because the risk of developing malignancy is very low. D Obtain at least one repeated image at 6-12 months to confirm stability in patients with nodules 1-4 cm with indeterminate imaging characteristics (such as non-contrast CT with HU > 10) because of a slightly increased risk of malignancy. B As per ACR 2021 guidelines, obtain non-contrast MRI or non-contrast/contrast-enhanced CT of the abdomen for the 12-month follow-up imaging of patients with no prior history of malignancy and an incidentally detected indeterminate adrenal mass 1-2 cm without diagnostic benign imaging characteristics. B As per ESE 2016 guidelines: Avoid obtaining further imaging for follow-up in patients with an adrenal mass < 4 cm with clear benign features on imaging studies. D Consider obtaining a repeat non-contrast CT or MRI after 6-12 months to exclude significant growth in patients with an indeterminate adrenal mass opting not to undergo adrenalectomy following initial assessment. C Consider performing surgical resection if the lesion enlarges by > 20%m in addition to at least a 5 mm increase in maximum diameter, during this period. C Obtain additional imaging after 6-12 months if there is a growth of the lesion below this threshold. B Serial laboratory assessment: As per CUA 2023 guidelines: Do not obtain further follow-up function testing in patients with benign nonfunctioning adenomas < 4 cm, myelolipomas, and other small masses containing macroscopic fat detected on the initial workup for an AI. D Do not obtain further follow-up function testing in patients with adrenal lesions growing < 3 mm/year on follow-up imaging. D As per AAES 2022 guidelines, consider reevaluating for autonomous cortisol secretion at 2-5- year intervals as it is the most common hormonal excess to develop during surveillance. B As per ESE 2016 guidelines: Avoid obtaining repeated hormonal workup in patients with a normal hormonal workup at the initial evaluation unless new clinical signs of endocrine activity appear or there is a worsening of comorbidities (such as hypertension or T2DM). D https://web.pathway.md/diseases/recl3lB15yicFTcCE 7/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway Consider obtaining annual clinical reassessment for cortisol excess comorbidities potentially related to cortisol excess in patients with 'autonomous cortisol secretion' without signs of overt Cushing's syndrome. Discuss the potential benefit of surgery based on the outcome of this evaluation. C References 1. Fassnacht M, Arlt W, Bancos I et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016 Aug;175 2 G1 G34. Open 2. Linwah Yip, Quan-Yang Duh, Heather Wachtel et al. American Association of Endocrine Surgeons Guidelines for Adrenalectomy: Executive Summary. JAMA Surg. 2022 Aug 17. Open 3. Dimitrios Stefanidis, Melanie Goldfarb, Kent W Kercher et al. SAGES guidelines for minimally invasive treatment of adrenal pathology. Surg Endosc. 2013 Nov;27 11 3960 80. Open 4. Neal E Rowe, Ravi M Kumar, Nicola Schieda et al. Canadian Urological Association guideline: Diagnosis, management, and followup of the incidentally discovered adrenal mass. Can Urol Assoc J. 2023 Feb;17 2 12 24. Open 5. Expert Panel on Urological Imaging, Rekha N Mody, Erick M Remer et al. ACR Appropriateness Criteria Adrenal Mass Evaluation: 2021 Update. J Am Coll Radiol. 2021 Nov;18 11S S251 S267. Open 6. David Aron, Massimo Terzolo, T J Cawood. Adrenal incidentalomas. 2012 Feb;26 1 69 82.2012 Feb;26 1 69 82. Open 7. Lynnette K Nieman. Approach to the patient with an adrenal incidentaloma. 2010 Sep;95 9 4106 13.2010 Sep;95 9 4106 13. Open 8. Lucyna Papierska, Andrzej Cichocki, Artur J Sankowski et al. Adrenal incidentaloma imaging - the first steps in therapeutic management. 2013 Oct;78 4 47 55.2013 Oct;78 4 47 55. Open 9. Jekaterina Patrova, Iwona Jarocka, Hans Wahrenberg et al. CLINICAL OUTCOMES IN ADRENAL INCIDENTALOMA EXPERIENCE FROM ONE CENTER. 2015 Aug;21 8 870 7.2015 Aug;21 8 870 7. Open 10. Lee JM, Kim MK, Ko SH et al. Clinical Guidelines for the Management of Adrenal Incidentaloma. Endocrinol Metab Seoul). 2017 Jun;32 2 200 218. Open 11. Martin Fassnacht, Olaf Dekkers, Tobias Else et al. European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2018 Oct 1;179 4 G1 G46. Open https://web.pathway.md/diseases/recl3lB15yicFTcCE 8/8
Guideline sources The following summarized guidelines for the evaluation and management of adrenocortical carcinoma (ACC) are prepared by our editorial team based on guidelines from the Canadian Urological Association (CUA 2023), the American Association of Endocrine Surgeons (AAES 2022), the European Reference Network on Rare Adult Solid Cancers (EURACAN/ESMO 2020), the European Society of Endocrinology (ESE 2018), the European Society of Endocrine Surgeons (ESES/ENSAT 2017), and the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES 2013). 1 2 3 4 5 6 Calculator Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s TNM class Guidelines 1. Classification and risk stratification https://web.pathway.md/diseases/recuSEdsRdEILoF3w 1/7 6/23/23, 2:10 AM Adrenocortical carcinoma Pathway Risk of malignancy: As per AAES 2022 guidelines, consider using washout characteristics on an adrenal protocol CT to stratify the risk of malignancy for adrenal nodules when the non-contrast HU is > 10 and other clinical risk factors for malignancy are not present. Recognize that adrenal protocol CT does not improve diagnostic accuracy for nodules with non-contrast HU < 10 nor does it improve evaluation for pheochromocytoma. C Show 2 more As per ESES 2017 guidelines, regard adrenal mass to be at increased risk of malignancy in the presence of any of the following: multiple hormonal, steroid precursor, or sex hormone oversecretion intratumoral radiological signs of malignancy and/or a diameter > 6 cm evidence of local invasion, suspected metastatic lymph nodes, distant metastasis, and/or high 18F-FDG-PET uptake. A Staging: As per ESMO 2020 guidelines, use the ENSAT-TNM system for the initial staging of ACC. B As per ESE 2018 guidelines, use the European Network for the Study of Adrenal Tumours staging classification at initial diagnosis of ACC. B Prognosis: Take the following factors into account when assessing prognosis and treatment options at initial diagnosis tumor stage resection status ki67 index (or mitotic count) autonomous cortisol secretion patient's general condition. B Reassess prognosis at each evaluation during follow-up to guide treatment strategy. B 2. Diagnostic investigations Clinical assessment: As per CUA 2023 guidelines, elicit a focused history and perform a physical examination to identify signs/symptoms of adrenal hormone excess, adrenal malignancy, and/or extra-adrenal malignancy in patients with an adrenal incidentaloma. E As per ESMO 2020 guidelines, obtain careful clinical assessment for signs of adrenal hormone excess in all patients with suspected ACC. E Diagnostic imaging: obtain at least abdominal CT or MRI and chest CT (or 18F-FDG-PET/CT) in patients with suspected ACC. B Laboratory tests: https://web.pathway.md/diseases/recuSEdsRdEILoF3w 2/7 6/23/23, 2:10 AM Adrenocortical carcinoma Pathway As per CUA 2023 guidelines, obtain serum testing for excess androgen in patients with suspected ACC. E As per AAES 2022 guidelines: Obtain biochemical testing to identify autonomous cortisol secretion in all patients with an adrenal incidentaloma 1 cm. Obtain biochemical evaluation for primary aldosteronism in patients with hypertension or hypokalemia. B Obtain a directed hormonal evaluation in patients with an adrenal mass regardless of history of extra-adrenal malignancy. B As per ESMO 2020 guidelines, obtain a comprehensive endocrine workup to identify potential autonomous excess of glucocorticoids, sex hormones, mineralocorticoids, and adrenocortical steroid hormone precursors in all patients with suspected ACC. B 3. Diagnostic procedures Biopsy and histopathology: As per AAES 2022 guidelines, consider performing an image-guided biopsy of a radiographically indeterminate mass, only after confirming a lack of hormone excess, if the results will change overall disease management. B As per ESMO 2020 guidelines: Avoid performing biopsies of suspected ACC because they are usually not informative. D Define ACC histopathologically by a Weiss score 3. B Make histological diagnosis by an experienced endocrine pathologist. Assess Ki-67 index to stratify the risk of recurrence. E As per ESE 2018 guidelines, confirm the diagnosis of ACC by histopathology. B Show 5 more 4. Medical management Setting of care: As per AAES 2022 guidelines: Treat patients with clinical and radiographic findings consistent with ACC at high-volume multidisciplinary centers to improve recurrence outcomes. B Perform adrenalectomy preferentially by a high-volume adrenal surgeon to optimize outcomes, including lower rates of morbidity and mortality. B As per ESMO 2020 guidelines, discuss all cases of suspected or proven ACC in a multidisciplinary expert team meeting, at least at the time of initial diagnosis and in case of progressive disease. E As per ESES 2017 guidelines: Manage patients with ACC in referral centers with established multidisciplinary teams consisting of surgeons, endocrinologists, oncologists, radiologists, pathologists, nuclear https://web.pathway.md/diseases/recuSEdsRdEILoF3w 3/7 6/23/23, 2:10 AM Adrenocortical carcinoma Pathway medicine physicians, biologists, and geneticists. B Perform surgeries in patients with ACC by surgeons with expertise in adrenal surgery (open and laparoscopic) and with a volume of > 15 adrenalectomies per year (benign and malignant). B Management of locoregional disease (neoadjuvant chemotherapy): do not use neoadjuvant chemotherapy in patients with resectable ACC. D Management of locoregional disease, surgery: As per CUA 2023 guidelines: Consider performing minimally invasive adrenalectomy in patients with suspected ACCs suitable for safe resection without rupturing the tumor capsule. C Consider performing open adrenalectomy in patients with larger ACCs or presenting with locally advanced tumors, lymph node metastases, or tumor thrombus in the renal vein/IVC. B As per AAES 2022 guidelines, perform resection of the primary tumor in patients with systemic disease if all sites of disease are reasonably amenable to resection or local treatment and if performance status allows. Consider performing surgery also in patients with hormone excess medically refractory to steroidogenic inhibition. B Show 3 more As per EURACAN/ESMO 2020 guidelines, perform complete en bloc resection (by a surgeon experienced in adrenal and oncological surgery) of all adrenal tumors suspected to be ACC as the mainstay of a potentially curative approach. E Show 2 more As per ENSAT/ESES 2017 guidelines, perform complete en bloc resection of the ACC with the peritumoral/periadrenal retroperitoneal fat. Do not perform enucleation and partial adrenal resection in patients with suspected ACC. Avoid causing intraoperative tumor capsule rupture or spillage. B Show 8 more As per SAGES 2013 guidelines, prefer minimally invasive adrenalectomy over open surgery for all non-primary adrenal cancer pathology due to less postoperative pain, shorter hospital stay, earlier recovery, and similar long-term outcomes. B Show 8 more Management of locoregional disease (other therapeutic interventions): avoid using ablation or stereotactic radiation as an alternative to adrenalectomy in patients with adrenal lesions. D Management of locoregional disease, adjuvant chemotherapy: As per EURACAN/ESMO 2020 guidelines: Offer adjuvant mitotane in patients at high risk of recurrence (stage III, or R1-RX resection, and/or Ki-67 index > 10%). B Individualize the decision to offer adjuvant therapy in patients at low risk of recurrence (stage I/II, R0 resection, and Ki-67 index 10%). B As per ESE 2018 guidelines, do not use adjuvant therapy in patients with adrenal tumors with uncertain malignant potential. D https://web.pathway.md/diseases/recuSEdsRdEILoF3w 4/7 6/23/23, 2:10 AM Adrenocortical carcinoma Pathway Show 3 more Management of locoregional disease, adjuvant radiotherapy: As per EURACAN/ESMO 2020 guidelines, consider offering adjuvant radiotherapy only on an individualized basis (in addition to mitotane) in patients with R1 or RX resection or in stage III. C As per ESE 2018 guidelines: Insufficient evidence to recommend adjuvant radiation therapy in patients with ACC. Avoid offering routine radiation therapy in patients with stage I-II and R0 resection. I Initiate adjuvant radiation therapy as soon as clinically possible after surgery, if a decision is made to deliver, at the dose of 50-60 Gy to the previous tumor bed in fractionated doses of approximately 2 Gy each. B Management of advanced/metastatic disease, systemic therapy: As per AAES 2022 guidelines, offer neoadjuvant systemic therapy in patients with advanced ACC if R0 surgical resection is not initially feasible. Perform up-front surgical intervention if R0 resection is possible. B As per EURACAN/ESMO 2020 guidelines: Offer mitotane monotherapy or mitotane plus chemotherapy (EDP-M: mitotane with etoposide, doxorubicin, and cisplatin) as first-line therapy in most patients with metastatic ACC. A Consider offering mitotane monotherapy in selected patients, such as with low tumor burden and/or more indolent disease. E As per ESE 2018 guidelines, offer either mitotane monotherapy or mitotane plus EDP (etoposide, doxorubicin, and cisplatin) depending on prognostic parameters in patients with advanced ACC at the time of diagnosis not qualifying for local treatment. B Management of advanced/metastatic disease, surgery: As per EURACAN/ESMO 2020 guidelines, offer surgery and locoregional therapies in addition to systemic therapy in selected patient populations. E Perforn surgery as the treatment of choice only if all tumoral lesions can be removed. B As per ESE 2018 guidelines: Consider performing surgery in patients with limited intra-abdominal metastases at the time of initial diagnosis, if complete resection of all lesions seems feasible. Consider performing adrenal tumor resection in conjunction with therapy aiming at long-term tumor control of other lesions in patients with limited extra-abdominal lesions. Initiate mitotane therapy as soon as clinically possible in all patients. C Avoid performing routine adrenal surgery in patients with widespread metastatic disease at the time of initial diagnosis. D As per ENSAT/ESES 2017 guidelines, consider performing surgical resection of liver and/or pulmonary metastases in patients with metastatic ACC, if R0 resection is achievable and can be performed with low morbidity and mortality rates. Recognize that the best results are observed in highly selected patients with favorable biological behavior (low Ki-67 index and long disease- free interval). C https://web.pathway.md/diseases/recuSEdsRdEILoF3w 5/7 6/23/23, 2:10 AM Adrenocortical carcinoma Pathway Show 2 more Management of advanced/metastatic disease, other therapeutic interventions: As per EURACAN/ESMO 2020 guidelines, offer local therapies (such as radiotherapy, radiofrequency ablation, cryoablation, microwave ablation, and chemoembolization) in patients with advanced ACC on an individualized basis. B As per ESE 2018 guidelines, consider offering other local therapeutic interventions (such as radiotherapy, radiofrequency ablation, cryoablation, microwave ablation, and chemoembolization) in addition to surgery in patients with advanced ACC. Consider individualizing the choice based on the localization of the tumor lesions, local expertise, prognostic factors, and patient's preference. C Management of progressive/relapsed disease: As per ESE 2018 guidelines, perform surgery or alternatively offer other local therapies in patients with recurrent disease and a disease-free interval of at least 12 months, if a complete resection/ablation seems feasible. B Show 3 more As per ESES 2017 guidelines, perform complete resection of locally recurrent ACC when feasible. Recognize that the best results after surgery for recurrent ACC are seen in patients with delayed recurrence (> 12 months), low Ki-67 status, and R0 complete resection. Do not use a laparoscopic approach for resection in patients with recurrent ACC. B 5. Perioperative care Preoperative evaluation: Elicit clinical history and perform a physical examination to assess the following before adrenalectomy for suspected ACC: symptoms related to hormone excess symptoms related to local compression by a large mass detailed family history for familial forms of cancer. B Show 4 more Perioperative corticosteroid replacement: administer perioperative hydrocortisone replacement in all patients with autonomous cortisol secretion. B 6. Follow-up and surveillance Follow-up: As per ESMO 2020 guidelines, obtain radiological imaging every 3 months for 2 years after complete resection, then every 3-6 months for at least another 3 years. B As per ESES 2017 guidelines, obtain clinical and hormonal evaluation, thoracoabdominal CT, and 18F-FDG-PET every 3 months for the first 2 years and every 4-6 months thereafter based on the risk of recurrence. B https://web.pathway.md/diseases/recuSEdsRdEILoF3w 6/7 6/23/23, 2:10 AM Adrenocortical carcinoma Pathway References 1. S Gaujoux, R Mihai, joint working group of ESES and ENSAT. European Society of Endocrine Surgeons ESES and European Network for the Study of Adrenal Tumours ENSAT recommendations for the surgical management of adrenocortical carcinoma. Br J Surg. 2017 Mar;104 4 358 376. Open 2. M Fassnacht, G Assie, E Baudin et al. Adrenocortical carcinomas and malignant phaeochromocytomas: ESMO EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020 Nov;31 11 1476 1490. Open 3. Martin Fassnacht, Olaf Dekkers, Tobias Else et al. European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2018 Oct 1;179 4 G1 G46. Open 4. Linwah Yip, Quan-Yang Duh, Heather Wachtel et al. American Association of Endocrine Surgeons Guidelines for Adrenalectomy: Executive Summary. JAMA Surg. 2022 Aug 17. Open 5. Neal E Rowe, Ravi M Kumar, Nicola Schieda et al. Canadian Urological Association guideline: Diagnosis, management, and followup of the incidentally discovered adrenal mass. Can Urol Assoc J. 2023 Feb;17 2 12 24. Open 6. Dimitrios Stefanidis, Melanie Goldfarb, Kent W Kercher et al. SAGES guidelines for minimally invasive treatment of adrenal pathology. Surg Endosc. 2013 Nov;27 11 3960 80. Open 7. Expert Panel on Urological Imaging, Rekha N Mody, Erick M Remer et al. ACR Appropriateness Criteria Adrenal Mass Evaluation: 2021 Update. J Am Coll Radiol. 2021 Nov;18 11S S251 S267. Open https://web.pathway.md/diseases/recuSEdsRdEILoF3w 7/7
Guideline sources The following summarized guidelines for the evaluation and management of adult growth hormone deficiency (AGHD) are prepared by our editorial team based on guidelines from the Endocrine Society (ES 2020; 2011) and the American College of Endocrinology (ACE/AACE 2019). 1 2 3 Guidelines 1. Screening and diagnosis Indications for testing: As per AACE 2019 guidelines: Consider screening patients with TBI, subarachnoid hemorrhage, ischemic stroke, and CNS infections for adult GH deficiency. C Obtain GH stimulation testing only after at least 12 months following TBI or subarachnoid hemorrhage, as GH deficiency in these conditions may be transient. C Obtain GH-stimulation tests only based on the appropriate clinical context of each individual patient with a history suggestive of a reasonable clinical suspicion of GH deficiency and with the intent to initiate recombinant human GH replacement if the diagnosis is confirmed. B As per ES 2011 guidelines: https://web.pathway.md/diseases/recfUiFwgJnBHDweg 1/5 6/23/23, 2:11 AM Adult growth hormone deficiency Pathway Consider testing for acquired GH deficiency in adult patients with structural hypothalamic/pituitary disease, surgery or irradiation in these areas, head trauma, or evidence of other pituitary hormone deficiencies. B Retest for GH deficiency in patients with childhood-onset GH deficiency being candidates for GH therapy after adult height achievement unless they have known mutations, embryopathic lesions causing multiple hormone deficits or irreversible structural lesions/damage. A Diagnosis: As per AACE 2019 guidelines, recognize that adults can be diagnosed with GH deficiency in childhood (childhood-onset GH deficiency) and adulthood (adult-onset GH deficiency). B Show 4 more As per ES 2011 guidelines, recognize that idiopathic GH deficiency in adults is very rare and stringent criteria are necessary to make this diagnosis. Consider obtaining two tests before making this diagnosis because in the absence of suggestive clinical circumstances there is a significant false-positive error rate in the response to a single GH stimulation test. Recognize that the presence of a low IGF-1 also increases the likelihood that this diagnosis is correct. B 2. Diagnostic investigations Provocative testing: As per AACE 2019 guidelines, do not obtain random serum GH and IGF-1 levels alone for the diagnosis of adult GH deficiency. Obtain GH stimulation tests to confirm the diagnosis with the exception of certain subpopulations such as patients with: organic hypothalamic-pituitary disease (such as suprasellar mass with previous surgery and cranial irradiation) having multiple ( 3) pituitary hormone deficiencies and low serum IGF-1 levels (< - 2.0 SDS) genetic defects affecting the hypothalamic-pituitary axes hypothalamic-pituitary structural brain defects. D Show 6 more As per ES 2011 guidelines, obtain insulin tolerance test and GH-releasing hormone-arginine test to establish the diagnosis of GH deficiency. Recognize that GH-releasing hormone-arginine testing may be misleading in patients with clearly established, recent (within 10 years) hypothalamic causes of suspected GH deficiency such as irradiation. A Show 5 more Brain magnetic resonance imaging: obtain baseline MRI in patients with any post-surgical tumor remnant in the hypothalamic-pituitary region before initiating recombinant human GH therapy, and obtain periodic MRI during therapy. B Lipid profile: Assess lipid profile at diagnosis to screen for dyslipidemia in adult patients with GH deficiency. B https://web.pathway.md/diseases/recfUiFwgJnBHDweg 2/5 6/23/23, 2:11 AM Adult growth hormone deficiency Pathway Consider obtaining lipid profile and initiating treatment for dyslipidemia and other cardiovascular risk factors in adult patients with GH deficiency associated with hypopituitarism. C Assessment of quality of life: consider assessing baseline quality of life using specific Quality of Life in Adult GH Deficiency Assessment questionnaires before initiating recombinant human GH treatment and at 12-month intervals to determine whether there is a change or sustained impact of recombinant human GH therapy on quality of life. C 3. Medical management Growth hormone therapy, indications: As per ES 2020 guidelines, do not use GH replacement solely to lower LDL-C to reduce cardiovascular risk in adult patients with GH deficiency. D As per ES 2011 guidelines, initiate GH therapy in patients with adult GH deficiency to provide significant clinical benefits in body composition, exercise capacity B and skeletal integrity. B Show 3 more Growth hormone therapy, dosing and duration: As per AACE/ACE 2019 guidelines, insufficient evidence to recommend one commercial recombinant human GH product over another. I Show 8 more As per ES 2011 guidelines, individualize GH dosing regimens taking into account gender, estrogen status and age, starting with low doses and titrating according to clinical response, side effects and IGF-1 levels. A Growth hormone therapy, monitoring: As per AACE/ACE 2019 guidelines, recognize that treatment-related side effects are mainly related to fluid retention effects and are typically seen during initiation and dose escalation of recombinant human GH and generally respond to dose reductions or cessation of therapy. Initiate lower doses of recombinant human GH in patients with obesity and in older patients generally being more susceptible to the side effects of recombinant human GH replacement. A Show 5 more As per ES 2011 guidelines: Consider monitoring thyroid and adrenal function during GH therapy. C Monitor patients at 1- to 2-month intervals during dose titration and semi-annually thereafter with a clinical assessment and an evaluation for adverse effects, IGF-1 levels and other parameters of GH response. B 4. Specific circumstances Pregnant patients: do not use recombinant human GH during pregnancy. D Patients with diabetes mellitus: https://web.pathway.md/diseases/recfUiFwgJnBHDweg 3/5 6/23/23, 2:11 AM Adult growth hormone deficiency Pathway As per AACE 2019 guidelines: Consider using low doses of recombinant human GH and adding and/or adjusting antidiabetic medications in patients developing diabetes mellitus during recombinant human GH therapy or if recombinant human GH therapy is considered in patients with concurrent diabetes mellitus. B Consider initiating or increasing the doses of antidiabetic therapy or discontinuing recombinant human GH therapy and optimizing treatment of diabetes mellitus first before considering resuming recombinant human GH therapy in patients experiencing worsening of preexisting diabetes mellitus while on recombinant human GH therapy. B As per ES 2011 guidelines, consider adjusting antidiabetic medications in patients with diabetes mellitus and adult GH deficiency receiving GH therapy. B Patients with malignancy: do not use recombinant human GH therapy in patients with a history of active malignancy (other than basal-cell or squamous-cell skin cancers) and active proliferative or severe nonproliferative diabetic retinopathy. D Show 3 more 5. Follow-up and surveillance Pediactric transition to adult care: retest for GH deficiency with GH stimulation tests after at least 1 month following discontinuation of recombinant human GH therapy in transition patients with idiopathic isolated GH deficiency with low-normal (between 0 to -2 SDS) or low (< -2 SDS) serum IGF-1 levels, after longitudinal growth is completed. B Show 10 more Monitoring for comorbidities: Monitor cardiovascular parameters, including fasting lipids, systolic and diastolic BP, and HR, at 6- to 12-month intervals in patients with adult GH deficiency. Consider obtaining more detailed examinations, such as ECG, echocardiogram, and carotid echo-Doppler, if clinically indicated. B Consider measuring bone mineral content and bone mineral density in patients with GH deficiency before starting recombinant human GH therapy. Obtain repeated bone DEXA at 2- to 3-year intervals to assess the need for additional bone-treatment modalities if the initial bone DEXA is abnormal. C 6. Quality improvement Laboratory quality control: adopt laboratory standards set by the National Institute for Biological Standards and Control, including reporting serum GH levels in mass units without reliance on conversion factors. B Show 6 more Growth hormone in sports: https://web.pathway.md/diseases/recfUiFwgJnBHDweg 4/5 6/23/23, 2:11 AM Adult growth hormone deficiency Pathway Recognize that detection of recombinant human GH abuse is difficult because GH is a naturally occurring substance with a short half-life after SC and IV injection, is released in a pulsatile fashion and the levels increase after exercise. Do not obtain drug testing with urine sampling as it is not accurate and reliable, whereas repeated blood sampling over 24 hours is neither practical nor feasible in the sports setting. A Do not prescribe recombinant human GH for sports or anti-aging purposes under any circumstances, recognizing that the off-label distribution or marketing of GH for the enhancement of athletic performance or to treat aging or aging-related conditions is illegal in the United States. D References 1. Mark E Molitch, David R Clemmons, Saul Malozowski et al. Evaluation and treatment of adult growth hormone deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011 Jun;96 6 1587 609. Open 2. Kevin C J Yuen, Beverly M K Biller, Sally Radovick et al. AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS AND AMERICAN COLLEGE OF ENDOCRINOLOGY GUIDELINES FOR MANAGEMENT OF GROWTH HORMONE DEFICIENCY IN ADULTS AND PATIENTS TRANSITIONING FROM PEDIATRIC TO ADULT CARE. Endocr Pract. 2019 Nov;25 11 1191 1232. Open 3. Connie B Newman, Michael J Blaha, Jeffrey B Boord et al. Lipid Management in Patients with Endocrine Disorders: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2020 Dec 1;105 12):dgaa674. Open 4. Sklar CA, Antal Z, Chemaitilly W et al. Hypothalamic-Pituitary and Growth Disorders in Survivors of Childhood Cancer: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2018 Aug 1;103 8 2761 2784. Open https://web.pathway.md/diseases/recfUiFwgJnBHDweg 5/5
Guideline sources The following summarized guidelines for the evaluation and management of adult-onset Still's disease (ASD) are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023), the German Society of Rheumatology (DGRh 2022), the European League Against Rheumatism (EULAR 2022), the National Institute for Health and Care Excellence (NICE 2021), the Adult-onset Still's Disease Consensus Group (AOSD-CG 2019), and the Ministry of Health, Labour and Welfare of Japan (MHLW 2018). 1 2 3 4 5 6 Calculator Calculator Fautrel's diagnostic criteria for a Yamaguchi diagnostic criteria fo Guidelines 1. Screening and diagnosis Definition: As per DGRh 2022 guidelines, recognize that ASD is a rare, polygenetic, autoinflammatory disorder. B https://web.pathway.md/diseases/recKXcdSwb7GjDVhL 1/4 6/23/23, 2:12 AM Adult-onset Still's disease Pathway As per AOSD-CG 2019 guidelines: Recognize that ASD and systemic JIA show substantial similarities in terms of clinical manifestations, laboratory features, response to treatment, B and, possibly, genetic background B Consider viewing ASD and systemic JIA as the same disease and attributing differences in the rate of clinical manifestations to the age of onset. C Clinical presentation: as per DGRh 2022 guidelines, recognize that in ASD: arthralgias are very common (> 80%) and arthritis is common (> 50%) a polyarticular course is observed more often than oligo- or monoarticular involvement very commonly involved regions (> 50%) are knees, ankles, and wrists, followed by (> 20%) elbows, shoulders, and fingers. B Show 4 more Diagnostic criteria: Diagnose ASD based on the typical combination of: very common symptoms (> 50%), such as fever > 39 C, rash, arthralgia, arthritis, throat pain, lymphadenopathy, myalgia common symptoms (> 20%), such as splenomegaly, hepatomegaly, weight loss less common characteristic symptoms (< 20%), such as pleuritis, pericarditis, abdominal pain exclusion of alternative rheumatological, hemato-oncological, and infectious diseases. B Consider supporting the clinical diagnosis of ASD by fulfilling the Yamaguchi criteria. C 2. Diagnostic investigations Laboratory testing: As per DGRh 2022 guidelines: Measure serum ferritin levels for diagnosis and follow-up of patients with ASD and to assess disease activity in conjunction with markers of inflammation, such as CRP. Recognize that a markedly elevated serum ferritin level ( 5 ULN) and, if available, markedly reduced fraction of glycosylated ferritin (< 20%) further support the diagnosis of ASD. B Consider measuring IL-18 levels to substantiate the diagnosis and disease activity in patients with ASD. C Evaluation for complications: Evaluate macrophage activation syndrome, a complication of ASD, in case of risk factors such as high clinical disease activity and laboratory markers such as a high ferritin serum level and cytopenia. B Exclude AA amyloidosis, a rare complication of ASD, in case of persistently active disease. B Pretreatment evaluation: screen for HBV infection in all patients eligible for treatment with conventional synthetic, biological, or targeted synthetic DMARDs, immunosuppressants, or corticosteroids (according to dose and duration). B https://web.pathway.md/diseases/recKXcdSwb7GjDVhL 2/4 6/23/23, 2:12 AM Adult-onset Still's disease Pathway Show 3 more 3. Medical management General principles: Ensure rheumatological expertise in diagnosing and treating ASD. B Follow the principles of participatory decision-making within a holistic therapeutic approach including pharmacological therapy alongside accompanying measures such as analgetic and physical therapy, rehabilitative measures, functional training, and the involvement of patient support groups for the treatment of ASD. B Nonsteroidal anti-inflammatory drugs: as per DGRh 2022 guidelines, consider initiating NSAIDs and other analgesics and antipyretics to control symptoms, such as pain and fever. C Corticosteroids: as per DGRh 2022 guidelines, initiate systemic corticosteroids as part of the initial therapy of acute-onset ASD. B Disease-modifying antirheumatic drugs: as per DGRh 2022 guidelines, consider initiating corticosteroid-sparing agents and/or alternative pharmacological therapies to prevent unwanted corticosteroid side effects. C Consider initiating methotrexate or calcineurin inhibitors, especially cyclosporin, as corticosteroid-sparing agents. B Biologics: As per DGRh 2022 guidelines, consider initiating biologics as corticosteroid-sparing agents, such as tocilizumab, B anakinra, B , or canakinumab. B Show 2 more As per NICE 2021 guidelines, initiate anakinra in patients with ASD not responding adequately to 2 conventional DMARDs. E As per AOSD-CG 2019 guidelines: Initiate IL-1 Inhibitors to improve clinical and laboratory parameters and for the significant corticosteroid-sparing effect in patients with ASD refractory to conventional treatment and/or other biologics. B Initiate IL-1 inhibitors for the treatment of ASD-related macrophage activation syndrome, B although cases of macrophage activation syndrome occurring during treatment with these drugs are reported. B 4. Preventative measures Routine immunizations: consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications. C Show 17 more https://web.pathway.md/diseases/recKXcdSwb7GjDVhL 3/4 6/23/23, 2:12 AM Adult-onset Still's disease Pathway Prophylaxis for Pneumocystis jirovecii pneumonia: consider administering prophylaxis against P. jirovecii pneumonia in patients initiating high-dose corticosteroids, especially in combination with immunosuppressants and depending on the risk-benefit ratio. C References 1. Stefan Vordenb umen, Eugen Feist, J rgen Rech et al. Diagnosis and treatment of adult-onset Still's disease: a concise summary of the German society of rheumatology S2 guideline. Z Rheumatol. 2022 Dec 15. Open 2. Toshihide Mimura, Yuya Kondo, Akihide Ohta et al. Evidence-based clinical practice guideline for adult Still's disease. Mod Rheumatol. 2018 Sep;28 5 736 757. Open 3. George E Fragoulis, Elena Nikiphorou, Mrinalini Dey et al. 2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis. 2022 Nov 3;ard-2022 223335. Open 4. Anne R Bass, Eliza Chakravarty, Elie A Akl et al. 2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases. Arthritis Rheumatol. 2023 Jan 4. Online ahead of print. Open 5. NICE. Anakinra for treating Still s disease. NICE. 2021. Open 6. Serena Colafrancesco, Maria Manara, Alessandra Bortoluzzi et al. Management of adult-onset Still's disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts. Arthritis Res Ther. 2019 Dec 11;21 1 275. Open 7. M Yamaguchi, A Ohta, T Tsunematsu et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992 Mar;19 3 424 30. Open 8. Bruno Fautrel, Emmanuel Zing, Jean-Louis Golmard et al. Proposal for a new set of classification criteria for adult-onset still disease. Medicine Baltimore). 2002 May;81 3 194 200. Open https://web.pathway.md/diseases/recKXcdSwb7GjDVhL 4/4
Guideline sources The following summarized guidelines for the evaluation and management of advanced maternal age pregnancy are prepared by our editorial team based on guidelines from the American College of Obstetricians and Gynecologists (ACOG 2022) and the Society of Obstetricians and Gynaecologists of Canada (SOGC 2017). 1 2 3 Guidelines 1. Diagnostic investigations Fertility evaluation: As per SOGC 2017 guidelines, refer females > 35 yeast of age for infertility work-up after 6 months of trying to conceive because of the decline in fertility and the increased time to conception that occur after age 35. B Show 4 more As per SOGC 2017 guidelines, obtain fertility evaluation after 6 months of unprotected intercourse without conception in females 35-37 years of age and earlier in females > 37 years of age. B Screening for multiple gestations: consider obtaining a first-trimester ultrasound to screen for multiple gestations in pregnant patients with anticipated delivery at age 35 years. C https://web.pathway.md/diseases/recMgU3hadUmKg8ET 1/3 6/23/23, 2:16 AM Advanced maternal age pregnancy Pathway Screening for genetic disorders: discuss and offer prenatal genetic screening (serum screening with or without nuchal translucency ultrasound or cell-free DNA screening) and diagnostic testing (chorionic villus sampling or amniocentesis) in all pregnant patients, regardless of age or risk of chromosomal abnormality. Recognize that every patient has the right to pursue or decline prenatal genetic screening and diagnostic testing after review and discussion. A Screening for congenital anomalies: As per ACOG 2022 guidelines, consider obtaining a detailed fetal anatomic ultrasound in pregnant patients with anticipated delivery at age 35 years because of the increased risk of aneuploidy and the potential increased risk of congenital anomalies. C As per SOGC 2017 guidelines, obtain screening for fetal aneuploidy and a detailed second- trimester ultrasound to look for significant fetal birth defects (particularly cardiac defects) in females 35 years of age. B Prenatal fetal monitoring: Consider obtaining an ultrasound for growth assessment in the third trimester in pregnant patients with anticipated delivery at age 40 years because of the increased risk of both large- for-gestational-age and small-for-gestational-age neonates. C Consider obtaining antenatal fetal surveillance in pregnant patients with anticipated delivery at age 40 years because of the increased risk of stillbirth. C 2. Medical management Prevention of preeclampsia: initiate daily low-dose aspirin for the reduction of preeclampsia in pregnant patients aged 35 years in the setting of at least one other moderate risk factor. B 3. Therapeutic procedures Indications for delivery: Initiate delivery in well-dated pregnancies at 39 0/7-39 6/7 weeks of gestation in patients with anticipated delivery at age 40 years because of the increasing rates of neonatal morbidity and stillbirth beyond this gestational age. B Consider counseling patients that vaginal delivery is safe and appropriate if there are no other maternal or fetal indications for Cesarean delivery. Include a discussion of the risks of Cesarean delivery, the patient's comorbidities, and the patient's preferences and goals. Recognize that advancing patient age alone is not an indication for Cesarean delivery. B 4. Patient education Maternal counseling: As per ACOG 2022 guidelines, consider viewing pregnancy with anticipated delivery at age 35 years as a risk factor for adverse maternal, fetal, and neonatal outcomes when counseling https://web.pathway.md/diseases/recMgU3hadUmKg8ET 2/3 6/23/23, 2:16 AM Advanced maternal age pregnancy Pathway patients and determining management plans. Provide nuanced counseling depending on specific age and comorbidities. C As per SOGC 2017 guidelines, recognize that females delaying childbearing are at increased risk of infertility. Inform prospective parents, especially females, that their fecundity and fertility begin to decline significantly after 32 years of age. Inform prospective parents that assisted reproductive technologies cannot guarantee a live birth or completely compensate for age- related decline in fertility. B Show 4 more As per SOGC 2017 guidelines, counsel females in their 20s and 30s about the age-related risk of infertility when other reproductive health issues, such as sexual health or contraception, are addressed as part of their primary well-woman care. Inform reproductive-age females that natural fertility and assisted reproductive technology success (except with egg donation) is significantly lower for females in their late 30s and 40s. B Show 2 more Paternal counseling: As per SOGC 2017 guidelines, counsel males > 40 years of age and their partners that advanced paternal age appears to be associated with an increased risk of spontaneous abortion and increased frequency of some autosomal dominant conditions, autism spectrum disorders, and schizophrenia when they are seeking pregnancy, although the risks remain small. B As per SOGC 2017 guidelines, inform prospective parents that semen quality and male fertility deteriorate with advancing age and that the risk of genetic disorders in offspring increases. B References 1. Kimberly E Liu, Allison Case. No. 346 Advanced Reproductive Age and Fertility. J Obstet Gynaecol Can. 2017 Aug;39 8 685 695. Open 2. No authors listed. Pregnancy at Age 35 Years or Older: ACOG Obstetric Care Consensus No. 11. Obstet Gynecol. 2022 Aug 1;140 2 348 366. Open 3. Jo-Ann Johnson, Suzanne Tough. No-271 Delayed Child-Bearing. J Obstet Gynaecol Can. 2017 Nov;39 11):e500-e515. Open https://web.pathway.md/diseases/recMgU3hadUmKg8ET 3/3
Guideline sources The following summarized guidelines for the evaluation and management of african trypanosomiasis are prepared by our editorial team based on guidelines from the World Health Organization (WHO 2019) and the Infectious Diseases Society of America (IDSA 2008). 1 2 Calculator WHO criteria for staging of Afric Guidelines 1. Diagnostic procedures Lumbar puncture: Perform a lumbar puncture for stratification in patients not meeting the following criteria: low index of suspicion of severe disease based on clinical judgment, and high confidence that the patient will have an appropriate follow-up to detect relapse early. B Consider performing a lumbar puncture in patients rejecting or not tolerating fexinidazole, to decide between pentamidine and nifurtimox-eflornithine combination therapy. C https://web.pathway.md/diseases/recobLels5aCZdsoq 1/3 6/23/23, 2:17 AM African trypanosomiasis Pathway 2. Medical management Setting of care: Consider deciding on outpatient administration of fexinidazole (under daily supervision) in consultation with the patient, family members, and clinicians, taking into account the following factors: convenience to the patient and the family (such as distance and costs) development of side effects interfering with treatment compliance existing comorbidities capacity of the healthcare system for supervised administration as an outpatient. C Hospitalize patients in the following cases: concomitant psychiatric disorders pediatric patients with body weight < 35 kg 100 WBCs in CSF after treatment (exceptionally) with fexinidazole risk of poor compliance with treatment. B Fexinidazole: Consider initiating treatment, preferentially with fexinidazole, omitting lumbar puncture in patients aged 6 years and weighing 20 kg meeting both of the following conditions: low index of suspicion of severe disease based on clinical judgment high confidence that the patient will have an appropriate follow-up to detect relapse early. C Show 2 more Nifurtimox-eflornithine combination therapy: Administer nifurtimox-eflornithine combination therapy in patients requiring lumbar puncture stratification but not undergoing lumbar puncture, and in patients with unreliable lumbar puncture results. B Administer nifurtimox-eflornithine combination therapy in patients with severe second-stage disease. B 3. Specific circumstances Pediatric patients: Administer pentamidine in pediatric patients aged < 6 years or weighing < 20 kg with first-stage disease. Administer nifurtimox-eflornithine combination therapy in patients with second-stage disease. B Administer fexinidazole in pediatric patients aged 6 years or weighing 20 kg with first- B or second-stage disease with WBCs in CSF < 100/ L. B Pregnant patients: consider administering fexinidazole after the first trimester. C Patients with encephalitis: Administer eflornithine, or melarsoprol as an alternative, in patients with Trypanosoma brucei gambiense encephalitis. B https://web.pathway.md/diseases/recobLels5aCZdsoq 2/3 6/23/23, 2:17 AM African trypanosomiasis Pathway Administer melarsoprol in patients with Trypanosoma brucei rhodesiense encephalitis. B References 1. No authors listed. WHO interim guidelines for the treatment of gambiense human African trypanosomiasis. Geneva: World Health Organization; 2019. Open 2. Tunkel AR, Glaser CA, Bloch KC et al. The management of encephalitis: clinical practice guidelines by the Infectious Diseases Society of America. Clin Infect Dis. 2008 Aug 1;47 3 303 27. Open 3. World Health Organization. Control and surveillance of human African trypanosomiasis. World Health Organ Tech Rep Ser. 2013; 984 1 237. Open https://web.pathway.md/diseases/recobLels5aCZdsoq 3/3
Guideline sources The following summarized guidelines for the evaluation and management of alcohol use disorder are prepared by our editorial team based on guidelines from the French Society of Alcohology (SFA/AFEF 2022), the American Association for the Surgery of Trauma (AAST 2022), the United States Department of Defense (DoD/VA 2021), the American Association for the Study of Liver Diseases (AASLD 2020), the American Psychiatric Association (APA 2018), the European Association for the Study of the Liver (EASL 2018), the U.S. Preventive Services Task Force (USPSTF 2018), and the American College of Gastroenterology (ACG 2018). 1 2 3 4 5 6 7 8 9 10 11 12 Definition AUD is defined in DSM-5 as a pattern of alcohol consumption leading to problems associated with two or more potential symptoms of tolerance, withdrawal, increased amount of alcohol consumed https://web.pathway.md/diseases/recK68bWnZmuq5dMC 1/8 6/23/23, 2:17 AM Alcohol use disorder Pathway over time, difficulty in controlling drinking, neglect of activities, ineffective efforts to reduce use, craving, failure to fulfil major role obligations, recurrent alcohol use in hazardous situations, time spent drinking or recovering from alcohol, and drinking despite physical/psychological problems in the past year. 9 Epidemiology AUD is caused due to genetic, psychological, and environmental factors. 10 Disease course The neurobiological disturbances due to habitual drinking cause AUD, which increases the risk of damage to the gastrointestinal, cardiovascular, immune, nervous, and other systems. AUD causes increased morbidity and mortality. 11 Prognosis and risk of recurrence AUD is associated with increased mortality from all causes of death (mortality rate ratio, 3.0-5.2). 12 Calculator Calculator AUDIT C for alcohol use CAGE questionnaire for alcohol Guidelines 1. Screening and diagnosis Definition: use the term AUD (defined by DSM-V criteria) in preference to alcoholic, alcohol abuse, alcohol dependence or risky drinker. A Indications for screening: As per AFEF 2022 guidelines, obtain systematic assessment of alcohol consumption in both general practice and specialist consultations. A Show 3 more As per AASLD 2020 guidelines, obtain routine screening for alcohol use using validated questionnaires in all patients receiving care in primary care and gastroenterology/hepatology outpatient clinics, emergency departments, and inpatient admissions. E As per ACG 2018 guidelines, use the AUDs Inventory Test to identify patients with alcohol use and dependence. E As per EASL 2018 guidelines: Screen patients for harmful alcohol consumption by general practitioners and in emergency departments. B https://web.pathway.md/diseases/recK68bWnZmuq5dMC 2/8 6/23/23, 2:17 AM Alcohol use disorder Pathway Use the AUDs Inventory Test or AUDs Inventory Test-Concise to screen patients for AUD and dependence. A As per USPSTF 2018 guidelines: Screen for unhealthy alcohol use in primary care settings in 18 years old adult patients, including pregnant females. B Insufficient evidence to recommend screening for alcohol use in primary care settings in 12-17 years old adolescent patients. I 2. Diagnostic investigations Initial evaluation: As per ACG 2018 guidelines, obtain LFTs and abdominal ultrasound in patients with harmful alcohol use and/or AUDs. E As per APA 2018 guidelines, consider obtaining physiological biomarkers to identify persistently elevated levels of alcohol consumption in the initial evaluation of patients with AUD or in the treatment of patients requiring ongoing monitoring of alcohol use. C Psychiatric evaluation: Assess for current and past use of tobacco and alcohol and any misuse of other substances, including prescribed or OTC medications and supplements, in the initial psychiatric evaluation of patients with suspected AUD. B Obtain quantitative behavioral measurement to detect alcohol misuse and assess its severity in the initial psychiatric evaluation of patients with suspected AUD. B Screening for alcohol-related liver disease: screen for ALD in high-risk populations, such as patients in alcohol rehabilitation clinics, or patients with harmful drinking identified by their general practitioner. B Show 2 more Screening for comorbidities: As per APA 2018 guidelines, evaluate for co-occurring conditions, including substance use disorders, other psychiatric disorders, and other medical disorders that may influence the selection of pharmacotherapy in patients with AUD. B As per EASL 2018 guidelines, screen patients with AUD for concurrent psychiatric disorders and other addictions. A 3. Medical management General principles: ensure a documented comprehensive and person-centered treatment plan including evidence-based nonpharmacological and pharmacological therapies for patients with AUD. B Goals of treatment: https://web.pathway.md/diseases/recK68bWnZmuq5dMC 3/8 6/23/23, 2:17 AM Alcohol use disorder Pathway Consider setting the initial goals of treatment of AUD (such as abstinence from alcohol use, reduction or moderation of alcohol use, other elements of harm reduction) agreed on between the patient and clinician and documented this agreement in the medical records. C Consider including the following discussions in the initial treatment goals and document these in the medical record: patient's legal obligations, such as abstinence from alcohol use, monitoring of abstinence risks to self from continued use of alcohol, such as physical health, occupational functioning, legal involvement risk to others from continued use of alcohol, such as impaired driving. C Naltrexone: do not use naltrexone in patients with AUD using opioids or having an anticipated need for opioids. D Show 3 more Acamprosate: Consider offering acamprosate in patients with moderate-to-severe AUD: having a goal of reducing alcohol consumption or achieving abstinence preferring pharmacotherapy or not responding to nonpharmacological treatments alone not having contraindications to the use of acamprosate. B Show 2 more Disulfiram: Consider offering disulfiram in patients with moderate-to-severe AUD: having a goal of achieving abstinence preferring disulfiram or intolerant to or not responding to naltrexone and acamprosate capable of understanding the risks of alcohol consumption while taking disulfiram not having contraindications to the use of disulfiram. C Anticonvulsants: Consider offering topiramate or gabapentin in patients with moderate-to-severe AUD: having a goal of reducing alcohol consumption or achieving abstinence preferring topiramate or gabapentin or intolerant to or not responding to naltrexone and acamprosate not having contraindications to the use of these medications. C Benzodiazepines: As per AFEF 2022 guidelines, offer benzodiazepines for the treatment of alcohol withdrawal syndrome until symptoms disappear. A As per ACG 2018 guidelines, offer benzodiazepines as the treatment of choice in patients with severe alcohol withdrawal syndrome and ALD. E As per APA 2018 guidelines, do not use benzodiazepines in patients with AUD unless for the treatment of acute alcohol withdrawal or a co-occurring disorder for which a benzodiazepine is indicated. D As per EASL 2018 guidelines, offer benzodiazepines for the treatment of alcohol withdrawal syndrome but not beyond 10-14 days because of the potential for abuse and/or encephalopathy. https://web.pathway.md/diseases/recK68bWnZmuq5dMC 4/8 6/23/23, 2:17 AM Alcohol use disorder Pathway A Antidepressants: do not use antidepressants in patients with AUD unless there is evidence of a co-occurring disorder for which an antidepressant is indicated. D Baclofen: As per AASLD 2020 guidelines, consider offering acamprosate or baclofen for the treatment of AUD in patients with ALD. E As per ACG 2018 guidelines, consider offering baclofen for the prevention of alcohol relapse in patients with ALD. C 4. Inpatient care Prevention of alcohol withdrawal syndrome in ICU: screen patients within 6-24 hours of discontinuation of alcohol consumption. Consider obtaining blood alcohol level as an initial screen, although subjective scoring systems (such as the AUDs Identification Test, the PAWSS) have higher sensitivity and specificity for the prediction of alcohol withdrawal syndrome. E Show 8 more 5. Nonpharmacologic interventions Brief behavioral intervention: As per AFEF 2022 guidelines, offer brief behavioral intervention in patients identified as alcohol misusers. Ensure that all practitioners are able to carry out this brief intervention. A As per AASLD 2020 guidelines, offer brief intervention in patients engaged in hazardous drinking (AUDIT-C 4, AUDIT > 8, binge drinkers). E As per USPSTF 2018 guidelines: Provide brief behavioral counseling interventions to reduce unhealthy alcohol use in persons engaged in risky or hazardous drinking. B Insufficient evidence to recommend brief behavioral counseling interventions for alcohol use in primary care settings in 12-17 years old adolescent patients. I Mindfulness-based therapies: insufficient evidence to recommend for or against mindfulness- based therapies in patients with AUD. I Technology-based interventions: consider implementing structured telephone-based care as an adjunct to usual care in patients with AUD. C Show 2 more 6. Specific circumstances Pregnant and breastfeeding patients: do not offer pharmacological treatment in pregnant and breastfeeding patients with AUD unless for the treatment of acute alcohol withdrawal with https://web.pathway.md/diseases/recK68bWnZmuq5dMC 5/8 6/23/23, 2:17 AM Alcohol use disorder Pathway benzodiazepines or a co-occurring disorder requiring pharmacotherapy. D 7. Patient education General counseling: counsel patients with heavy alcohol use (> 3 drinks per day in males and > 2 drinks per day in females for > 5 years) that they are at an increased risk for liver disease. B 8. Preventative measures Alcohol restriction: As per AFEF 2022 guidelines, consider advising alcohol consumption of 10 standard drinks per week in the general population to avoid overall health risks. C Show 2 more As per AASLD 2020 guidelines, counsel patients without liver disease about safe levels of alcohol use for males ( 2 standard drinks per 24 hours) and females ( 1 standard drink per 24 hours). E 9. Follow-up and surveillance Monitoring of abstinence: consider obtaining hair or urine ethyl glucuronide for accurate monitoring of abstinence in patients with ALD. B 10. Quality improvement Public health measures: As per AFEF 2022 guidelines, disseminate information to the general population on the risks associated with binge drinking, particularly in terms of cardiovascular morbidity and mortality and physical and psychological injuries. A As per EASL 2018 guidelines, address excess alcohol consumption using pricing-based policies and regulation of availability. A Show 3 more Likelihood Ratios Pertinent positives The following findings increase the probability of alcohol use disorder in adults. -1 Finding LR+ Value Presence of AUDIT or AUDIT-C score 8 6.8-12 (8-12) https://web.pathway.md/diseases/recK68bWnZmuq5dMC 6/8 6/23/23, 2:17 AM Alcohol use disorder Pathway Pertinent negatives The following findings decrease the probability of alcohol use disorder in adults. -1 Finding LR- Value Absence of AUDIT or AUDIT-C score 8 0.46-0.62 References 1. Alexandre Louvet, Jean-Baptiste Trabut, Christophe Moreno et al. Management of alcohol-related liver disease: the French Association for the Study of the Liver and the French Alcohol Society clinical guidelines. Liver Int. 2022 Apr 29. Online ahead of print. Open 2. Reus VI, Fochtmann LJ, Bukstein O et al. The American Psychiatric Association Practice Guideline for the Pharmacological Treatment of Patients With Alcohol Use Disorder. Am J Psychiatry. 2018 Jan 1;175 1 86 90. Open 3. David W Crabb, Gene Y Im, Gyongyi Szabo et al. Diagnosis and Treatment of Alcohol-Associated Liver Diseases: 2019 Practice Guidance From the American Association for the Study of Liver Diseases. Hepatology. 2020 Jan;71 1 306 333. Open 4. European Association for the Study of the Liver. EASL clinical practice guidelines: management of alcohol-related liver disease. J Hepatol. 2018 Jul;69 1 154 181. Open 5. Department of Defense/Veterans Affairs. VA/DoD CLINICAL PRACTICE GUIDELINE FOR THE MANAGEMENT OF SUBSTANCE USE DISORDERS. VA/DoD. 2021 Aug. Open 6. Anupamaa Seshadri, Rachel Appelbaum, Samuel P Carmichael nd et al. Prevention of alcohol withdrawal syndrome in the surgical ICU an American Association for the Surgery of Trauma Critical Care Committee Clinical Consensus Document. Trauma Surg Acute Care Open. 2022 Nov 21;7 1):e001010. Open 7. US Preventive Services Task Force, Curry SJ, Krist AH et al. Screening and Behavioral Counseling Interventions to Reduce Unhealthy Alcohol Use in Adolescents and Adults: US Preventive Services Task Force Recommendation Statement. JAMA. 2018 Nov 13;320 18 1899 1909. Open 8. Singal AK, Bataller R, Ahn J et al. ACG Clinical Guideline: Alcoholic Liver Disease. Am J Gastroenterol. 2018 Feb;113 2 175 194. Open 9. K Witkiewitz, R Z Litten, L Leggio. Advances in the science and treatment of alcohol use disorder. 2019 Sep 25;5 9):eaax4043.2019 Sep 25;5 9):eaax4043. Open 10. Ping Yang, Rui Tao, Chengsen He et al. The Risk Factors of the Alcohol Use Disorders-Through Review of Its Comorbidities. Front Neurosci. 2018 May 11;12 303. Open 11. Robin C Wackernah, Matthew J Minnick, Peter Clapp. Alcohol use disorder: pathophysiology, effects, and pharmacologic options for treatment. Subst Abuse Rehabil. 2014 Jan 23;5 1 12. Open 12. J Westman, K Wahlbeck, T M Laursen et al. Mortality and life expectancy of people with alcohol use disorder in Denmark, Finland and Sweden. 2015 Apr;131 4 297 306.2015 Apr;131 4 297 306. Open 13. Reus VI, Fochtmann LJ, Bukstein O et al. The American Psychiatric Association Practice Guideline for the Pharmacological Treatment of Patients With Alcohol Use Disorder. Am J Psychiatry. 2018 Jan 1;175 1 86 90. Open https://web.pathway.md/diseases/recK68bWnZmuq5dMC 7/8 6/23/23, 2:17 AM Alcohol use disorder Pathway 14. Sheryl Spithoff, Suzanne Turner, Tara Gomes et al. First-line medications for alcohol use disorders among public drug plan beneficiaries in Ontario. Can Fam Physician. 2017 May;63 5):e277-e283. Open 15. Sheryl Spithoff, Suzanne Turner. A systemic failure to address at-risk drinking and alcohol use disorders: the Canadian story. CMAJ. 2015 Apr 21;187 7 479 480. Open 16. J R Stabenau. Implications of family history of alcoholism, antisocial personality, and sex differences in alcohol dependence. Am J Psychiatry. 1984 Oct;141 10 1178 82. Open 17. Andrew T A Cheng, Shur-Fen Gau, Tony H H Chen et al. A 4-year longitudinal study on risk factors for alcoholism. Arch Gen Psychiatry. 2004 Feb;61 2 184 91. Open 18. Fides Sch ckher, Tabita Sellin, Ingemar Engstr m et al. History of childhood abuse is associated with less positive treatment outcomes in socially stable women with alcohol use disorder. BMC Womens Health. 2019 Dec 12;19 1 159. Open 19. K Poikolainen. Risk factors for alcohol dependence: a case-control study. Alcohol Alcohol. Mar-Apr 2000;35 2 190 6. Open 20. Sara M. Nehring, Andrew M. Freeman. Alcohol Use Disorder. In: StatPearls Internet]. Treasure Island FL StatPearls Publishing; 2021 Jan. 2020 Nov 18. Open 21. Henry R Kranzler, Michael Soyka. Diagnosis and Pharmacotherapy of Alcohol Use Disorder: A Review. JAMA. 2018 Aug 28;320 8 815 824. Open 22. K Bush, D R Kivlahan, M B McDonell et al. The AUDIT alcohol consumption questions AUDIT C an effective brief screening test for problem drinking. Ambulatory Care Quality Improvement Project ACQUIP . Alcohol Use Disorders Identification Test. Arch Intern Med. 1998 Sep 14;158 16 1789 95. Open 23. Mareike Augsburger, Esta Kaal, Triin lesoo et al. Effects of a minimal-guided online intervention for alcohol misuse in Estonia: A Randomized Controlled Trial. Addiction. 2022 Jan;117 1 108 117. Open 24. Scott L Friedman, Bruce A Runyon, Kristen M Robson. Clinical manifestations and diagnosis of alcohol- associated fatty liver disease and cirrhosis. UpToDate. 2021. Open 25. Michael P Bogenschutz, Stephen Ross, Snehal Bhatt et al. Percentage of Heavy Drinking Days Following Psilocybin-Assisted Psychotherapy vs Placebo in the Treatment of Adult Patients With Alcohol Use Disorder: A Randomized Clinical Trial. JAMA Psychiatry. 2022 Oct 1;79 10 953 962. Open 26. A R Lingford-Hughes, S Welch, L Peters et al. BAP updated guidelines: evidence-based guidelines for the pharmacological management of substance abuse, harmful use, addiction and comorbidity: recommendations from BAP. J Psychopharmacol. 2012 Jul;26 7 899 952. Open https://web.pathway.md/diseases/recK68bWnZmuq5dMC 8/8
Guideline sources The following summarized guidelines for the evaluation and management of alcohol-related liver disease are prepared by our editorial team based on guidelines from the French Society of Alcohology (SFA/AFEF 2022), the American Association for the Study of Liver Diseases (AASLD 2020), the European Association for the Study of the Liver (EASL 2018), the U.S. Preventive Services Task Force (USPSTF 2018), and the American College of Gastroenterology (ACG 2018). 1 2 3 4 5 6 7 8 9 Definition Alcoholic liver disease encompasses a spectrum of disorders that may take an acute form (alcoholic hepatitis) or present as a chronic disease (steatosis, steatohepatitis, fibrosis, and cirrhosis). 6 Epidemiology Alcoholic liver disease is caused by heavy alcohol use. 7 https://web.pathway.md/diseases/recH96LhA8v1VcAWd 1/9 6/23/23, 2:17 AM Alcohol-related liver disease Pathway Disease course Alcohol abuse results in steatosis followed by steatohepatitis, alcoholic hepatitis, fibrosis, and liver cirrhosis due to inflammation, hepatocellular damage, and liver fibrosis. Disease progression may also cause liver decompensation and HCC. 8 Prognosis and risk of recurrence 1-year mortality of alcoholic liver disease as a single group is around 20%. 9 Calculator AUDIT C for alcohol use Guidelines 1. Screening and diagnosis Screening for alcohol abuse: As per AFEF 2022 guidelines, obtain systematic assessment of alcohol consumption in both general practice and specialist consultations. A Show 3 more As per AASLD 2020 guidelines, obtain routine screening for alcohol use using validated questionnaires in all patients receiving care in primary care and gastroenterology/hepatology outpatient clinics, emergency departments, and inpatient admissions. E As per ACG 2018 guidelines, use the AUDs Inventory Test as a validated tool to identify patients with alcohol use and dependence. E As per EASL 2018 guidelines, screen patients for harmful alcohol consumption by general practitioners and in emergency departments. B Show 2 more Screening for alcohol-related liver disease: As per AFEF 2022 guidelines, integrate screening for ALD in more general liver disease screening, including nonalcoholic fatty liver disease and hepatitis B and C viruses. E As per EASL 2018 guidelines, screen for ALD in high-risk populations, such as patients in alcohol rehabilitation clinics, or patients with harmful drinking identified by their general practitioner. B Show 2 more Diagnosis: As per AASLD 2020 guidelines: https://web.pathway.md/diseases/recH96LhA8v1VcAWd 2/9 6/23/23, 2:17 AM Alcohol-related liver disease Pathway Consider obtaining alcohol biomarkers to aid in diagnosis of AUD and support recovery. Prefer urine and hair ethyl glucuronide, urine ethyl sulfate, and phosphatidylethanol as they are not affected by liver disease. E Diagnose alcoholic hepatitis (definite, probable, possible) using the published consensus criteria. E As per EASL 2018 guidelines: Use the term AUD (defined by DSM-V criteria) in preference to alcoholic, alcohol abuse, alcohol dependence or risky drinker. A Suspect the diagnosis of alcoholic hepatitis in patients with excessive alcohol consumption and a recent onset of jaundice. A 2. Classification and risk stratification Prognostic scores: As per AFEF 2022 guidelines, use the NIAAA classification in the absence of liver biopsy to offer treatment only in patients with probable alcoholic hepatitis. E Show 7 more As per AASLD 2020 guidelines, use lab-based prognostic scores to determine prognosis in alcoholic hepatitis. E Show 3 more As per EASL 2018 guidelines, use available prognostic scores to identify patients with severe forms of alcoholic hepatitis at risk of early mortality. A 3. Diagnostic investigations Initial evaluation: obtain LFTs and an abdominal ultrasound in patients with harmful alcohol use and/or AUD. E Assessment of liver fibrosis: obtain noninvasive assessment (elastography or specialized blood tests) of liver fibrosis in all patients with ALD. A Show 3 more Evaluation for advanced disease: Define a target group meeting the following criteria in order to identify patients with advanced ALD in the general population: age 40-45 years, AUDIT score predictive of hazardous consumption, and/or consumption of 14 standard drinks/week. B Show 2 more Screening for infection: As per AFEF 2022 guidelines, obtain systematic evaluation for infection in patients with severe alcoholic hepatitis. A https://web.pathway.md/diseases/recH96LhA8v1VcAWd 3/9 6/23/23, 2:17 AM Alcohol-related liver disease Pathway As per EASL 2018 guidelines, obtain systematic screening for infection before initiating therapy, during corticosteroid treatment, and during the follow-up period in patients with alcoholic hepatitis. A Screening for cognitive impairment: screen for the presence of cognitive impairments because of their high prevalence. E 4. Diagnostic procedures Liver biopsy: As per AFEF 2022 guidelines: Perform liver biopsy to confirm the clinical suspicion of alcoholic hepatitis in patients being potential candidates for specific treatment. B Perform liver biopsy in patients with ALD in case of doubt concerning the presence of associated chronic liver disease, or in the case of a discordant noninvasive examination casting doubt on the presence of cirrhosis. E As per ACG 2018 guidelines: Avoid performing routine liver biopsy for the diagnosis of alcoholic fatty liver disease. D Consider performing liver biopsy and using noninvasive fibrosis assessment tools in patients with suspected steatohepatitis and/or liver fibrosis. E As per EASL 2018 guidelines, perform liver biopsy if there is diagnostic uncertainty, precise staging is required, or in clinical trials. A 5. Medical management General principles: As per AASLD 2020 guidelines, offer brief intervention, pharmacotherapy, and referral to treatment in patients engaged in hazardous drinking (AUDIT-C 4, AUDIT > 8, binge drinkers). E Show 2 more As per ACG 2018 guidelines, provide multidisciplinary care for ALD with the involvement of addiction specialists. E As per EASL 2018 guidelines, refer patients with ALD identified through screening to a multidisciplinary team. A Show 3 more Management of alcohol use disorder: As per AFEF 2022 guidelines, recognize that long-term outcomes in patients with ALD depend mainly on achieving abstinence. Offer addiction treatment systematically after an episode of alcoholic hepatitis. A Show 6 more https://web.pathway.md/diseases/recH96LhA8v1VcAWd 4/9 6/23/23, 2:17 AM Alcohol-related liver disease Pathway As per AASLD 2020 guidelines, consider offering acamprosate or baclofen for the treatment of AUD in patients with ALD. E As per ACG 2018 guidelines, consider offering baclofen for the prevention of alcohol relapse in patients with ALD. C Management of alcohol withdrawal: As per AFEF 2022 guidelines, offer benzodiazepines for the treatment of alcohol withdrawal syndrome until symptoms disappear. (G1+. As per ACG 2018 guidelines: Manage alcohol withdrawal syndrome according to the Clinical Institute Withdrawal Assessment for Alcohol protocol. E Offer benzodiazepines as the treatment of choice in patients with severe alcohol withdrawal syndrome and ALD. E Landmark trials: Symptom-triggered vs. fixed-dose benzodiazepines for alcohol withdrawal In patients with alcohol dependence entering an alcohol treatment program, symptom- triggered benzodiazepine administration was superior to fixed-schedule benzodiazepine administration with respect to total amount of oxazepam administered. Daeppen JB et al. Arch Intern Med. 2002 May 27. As per EASL 2018 guidelines, offer benzodiazepines for the treatment of alcohol withdrawal syndrome but not beyond 10-14 days because of the potential for abuse and/or encephalopathy. A Corticosteroids: As per AFEF 2022 guidelines, initiate corticosteroid therapy (prednisolone 40 mg/day or methylprednisolone 32 mg/day) to improve short-term survival in patients with severe alcoholic hepatitis. A As per AASLD 2020 guidelines, consider initiating oral prednisolone 40 mg/day, if not contraindicated, to improve 28-day mortality in patients with severe alcoholic hepatitis (MDF 32). E As per EASL 2018 guidelines: Consider initiating corticosteroids (prednisolone 40 mg/day or methylprednisolone 32 mg/day) to reduce short-term mortality in patients with severe alcoholic hepatitis, in the absence of active infection. B Identify patients with early nonresponse (at day 7) to corticosteroids and apply strict rules of cessation therapy. A N-acetylcysteine: https://web.pathway.md/diseases/recH96LhA8v1VcAWd 5/9 6/23/23, 2:17 AM Alcohol-related liver disease Pathway As per AFEF 2022 guidelines, offer adding N-acetylcysteine to corticosteroids in patients with severe alcoholic hepatitis. B As per AASLD 2020 guidelines, consider administering IV N-acetylcysteine in addition to prednisolone (40 mg/day) to improve 30-day survival in patients with severe alcoholic hepatitis. E As per EASL 2018 guidelines, consider administering IV N-acetylcysteine for 5 days combined with corticosteroids in patients with severe alcoholic hepatitis. C Pentoxifylline: As per AFEF 2022 guidelines, do not use pentoxifylline in patients with severe alcoholic hepatitis because of its ineffectiveness. D As per AASLD 2020 guidelines, do not use pentoxifylline for the treatment of alcoholic hepatitis. D 6. Nonpharmacologic interventions Alcohol abstinence: As per AFEF 2022 guidelines, advise patients with cirrhosis and/or HCC to completely and permanently stop all alcohol consumption in order to limit the risk of excess mortality. B As per AASLD 2020 guidelines, advise patients with ALD or other liver diseases, in particular nonalcoholic fatty liver disease, NASH, viral hepatitis, and hemochromatosis to abstain from drinking. E As per ACG 2018 guidelines, advise complete abstinence from alcohol consumption in patients with ALD. E As per EASL 2018 guidelines, advise and encourage patients with ALD cirrhosis to achieve complete abstinence from alcohol to reduce the risk of liver-related complications and mortality. A Brief behavioral intervention: As per AFEF 2022 guidelines, offer brief behavioral intervention in patients identified as alcohol misusers. Ensure that all practitioners are able to carry out this brief intervention. A As per AASLD 2020 guidelines, offer brief intervention in patients engaged in hazardous drinking (AUDIT-C 4, AUDIT > 8, binge drinkers). E As per ACG 2018 guidelines, consider offering brief motivational interventions to reduce alcohol relapse in patients with ALD. C As per EASL 2018 guidelines, offer a brief intervention in patients with ALD identified through screening. A As per USPSTF 2018 guidelines, provide brief behavioral counseling interventions to reduce unhealthy alcohol use in persons engaged in risky or hazardous drinking. B Smoking cessation: https://web.pathway.md/diseases/recH96LhA8v1VcAWd 6/9 6/23/23, 2:17 AM Alcohol-related liver disease Pathway As per AFEF 2022 guidelines, advise smoking cessation in patients with ALD as it increases the risk of fibrosis and HCC in the course of ALD. A As per ACG 2018 guidelines, advise abstaining from cigarette smoking in patients with ALD. B Weight loss: consider offering specific management for obesity/overweight as it accelerates the progression of ALD. E Nutritional support: As per AASLD 2020 guidelines, address and treat malnutrition, preferably with enteral nutrition, in patients with alcoholic hepatitis. E As per EASL 2018 guidelines, obtain careful evaluation of nutritional status and advise patients to achieve a daily energy intake 35-40 kcal/kg body weight and 1.2-1.5 g/kg protein, and to adopt the oral route as first-line intervention. B 7. Perioperative care Pretransplantation care: decide on the duration of abstinence before listing depending on the degree of liver insufficiency in selected patients with a favorable addiction and psychological profile and supportive relatives. A Show 5 more 8. Surgical interventions Liver transplantation: As per AFEF 2022 guidelines, consider offering fast-track liver transplantation in patients at the end of their therapeutic options. Select candidates rigorously and in a multidisciplinary setting in the reference transplant center. B As per AASLD 2020 guidelines, refer patients with decompensated alcohol-associated cirrhosis, CPT class C or MELD-Na 21 for liver transplantation. E Show 2 more As per ACG 2018 guidelines, consider performing liver transplantation while formulating a management plan for patients with end-stage ALD. E As per EASL 2018 guidelines, consider performing liver transplantation in patients with ALD (classified as Child-Pugh C and/or MELD 15) as it confers a survival benefit. B Show 3 more 9. Preventative measures Alcohol restriction: As per AFEF 2022 guidelines, consider advising alcohol consumption of 10 standard drinks per week in the general population to avoid overall health risks. C https://web.pathway.md/diseases/recH96LhA8v1VcAWd 7/9 6/23/23, 2:17 AM Alcohol-related liver disease Pathway Show 2 more As per AASLD 2020 guidelines, counsel patients without liver disease about safe levels of alcohol use for males ( 2 standard drinks per 24 hours) and females ( 1 standard drink per 24 hours). E 10. Follow-up and surveillance Monitoring of abstinence: consider obtaining hair or urine ethyl glucuronide for accurate monitoring of abstinence in patients with ALD. B Monitoring of transplant recipients: As per ACG 2018 guidelines, screen transplant recipients at each visit for use of alcohol and other substances especially tobacco and cannabis. E As per EASL 2018 guidelines, obtain a multidisciplinary evaluation for medical and psychological suitability for transplantation after liver transplantation. A Show 2 more 11. Quality improvement Public health measures: As per AFEF 2022 guidelines, disseminate information to the general population on the risks associated with binge drinking, particularly in terms of cardiovascular morbidity and mortality and physical and psychological injuries. A As per EASL 2018 guidelines, address excess alcohol consumption using pricing-based policies and regulation of availability. A Show 3 more References 1. European Association for the Study of the Liver. EASL clinical practice guidelines: management of alcohol-related liver disease. J Hepatol. 2018 Jul;69 1 154 181. Open 2. David W Crabb, Gene Y Im, Gyongyi Szabo et al. Diagnosis and Treatment of Alcohol-Associated Liver Diseases: 2019 Practice Guidance From the American Association for the Study of Liver Diseases. Hepatology. 2020 Jan;71 1 306 333. Open 3. US Preventive Services Task Force, Curry SJ, Krist AH et al. Screening and Behavioral Counseling Interventions to Reduce Unhealthy Alcohol Use in Adolescents and Adults: US Preventive Services Task Force Recommendation Statement. JAMA. 2018 Nov 13;320 18 1899 1909. Open 4. Alexandre Louvet, Jean-Baptiste Trabut, Christophe Moreno et al. Management of alcohol-related liver disease: the French Association for the Study of the Liver and the French Alcohol Society clinical guidelines. Liver Int. 2022 Apr 29. Online ahead of print. Open https://web.pathway.md/diseases/recH96LhA8v1VcAWd 8/9 6/23/23, 2:17 AM Alcohol-related liver disease Pathway 5. Singal AK, Bataller R, Ahn J et al. ACG Clinical Guideline: Alcoholic Liver Disease. Am J Gastroenterol. 2018 Feb;113 2 175 194. Open 6. Radan Bruha, Karel Dvorak, Jaromir Petrtyl. Alcoholic liver disease. 2012 Mar 27;4 3 81 90.2012 Mar 27;4 3 81 90. Open 7. Koichiro Ohashi, Michael Pimienta, Ekihiro Seki. Alcoholic liver disease: A current molecular and clinical perspective. 2018 Dec;2 4 161 172.2018 Dec;2 4 161 172. Open 8. Eric S Orman, Gemma Odena, Ramon Bataller. Alcoholic liver disease: pathogenesis, management, and novel targets for therapy. 2013 Aug;28 Suppl 1 0 1 77 84.2013 Aug;28 Suppl 1 0 1 77 84. Open 9. Ina Bergheim, Craig J McClain, Gavin E Arteel. Treatment of alcoholic liver disease. 2005;23 3 4 275 84.2005;23 3 4 275 84. Open 10. European Association for the Study of Liver. EASL clinical practical guidelines: management of alcoholic liver disease. J Hepatol. 2012 Aug;57 2 399 420. Open 11. O'Shea RS, Dasarathy S, McCullough AJ et al. Alcoholic liver disease. Hepatology. 2010 Jan;51 1 307 28. Open 12. K Walsh, G Alexander. Alcoholic liver disease. Postgrad Med J. 2000 May;76 895 280 6. Open 13. Natalia A Osna, Terrence M Donohue Jr, Kusum K Kharbanda. Alcoholic Liver Disease: Pathogenesis and Current Management. Alcohol Res. 2017;38 2 147 161. Open 14. Radan Bruha, Karel Dvorak, Jaromir Petrtyl. Alcoholic liver disease. World J Hepatol. 2012 Mar 27;4 3 81 90. Open 15. Christina Hackl, Hans J Schlitt, Philipp Renner et al. Liver surgery in cirrhosis and portal hypertension. World J Gastroenterol. 2016 Mar 7;22 9 2725 35. Open 16. Paul Martin, Andrea DiMartini, Sandy Feng et al. Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology. 2014 Mar;59 3 1144 65. Open 17. Italian Association for the Study of the Liver AISF , Italian Association for the Study of the Liver AISF. AISF position paper on liver disease and pregnancy. Dig Liver Dis. 2016 Feb;48 2 120 37. Open 18. Monika Sarkar, Carla W Brady, Jaquelyn Fleckenstein et al. Reproductive Health and Liver Disease: Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73 1 318 365. Open 19. Alexandre Louvet, Julien Labreuche, Thong Dao et al. Effect of Prophylactic Antibiotics on Mortality in Severe Alcohol-Related Hepatitis: A Randomized Clinical Trial. JAMA. 2023 May 9;329 18 1558 1566. Open 20. Mads Israelsen, Bj rn St hr Madsen, Nikolaj Torp et al. Rifaximin- for liver fibrosis in patients with alcohol-related liver disease GALA RIF a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Gastroenterol Hepatol. 2023 Jun;8 6 523 532. Open https://web.pathway.md/diseases/recH96LhA8v1VcAWd 9/9
Guideline sources The following summarized guidelines for the evaluation and management of alcoholic hepatitis (AH) are prepared by our editorial team based on guidelines from the American College of Gastroenterology (ACG 2018), the European Association for the Study of the Liver (EASL 2012), and the American College of Gastroenterology (ACG/AASLD 2010). 1 2 3 4 4 4 5 Definition AH is a life-threatening form of acute-on-chronic liver failure occurring in patients with sustained heavy alcohol use that is characterized by a rapid onset or worsening of jaundice, liver synthetic dysfunction, and features of hepatic decompensation. 4 Epidemiology AH is caused by intra- and extra-hepatic effects of alcohol. 4 Disease course https://web.pathway.md/diseases/recuYBTsmBvoQCsjt 1/5 6/23/23, 2:17 AM Alcoholic hepatitis Pathway The complex interplay between ethanol metabolism, inflammation, and innate immunity results in liver damage causing AH, which present with clinical manifestations of fever, jaundice, hepatomegaly, ascites, and hepatic encephalopathy. Disease progression leads to liver fibrosis, cirrhosis, and the need for liver transplantation. 5 Prognosis and risk of recurrence Severe AH is associated with 50% 90-day mortality. 4 Calculator Diagnostic criteria for alcoholic Guidelines 1. Screening and diagnosis Diagnostic criteria: Diagnose AH in a patient with: rapid development or worsening of jaundice and liver-related complications serum TBIL > 50 mol/L (> 3 mg/dL) ALT and AST elevated > 1.5 times the ULN but < 400 U/L with the AST/ALT ratio > 1.5 documentation of heavy alcohol use until 8 weeks prior to onset of symptoms exclusion of other liver diseases. E 2. Classification and risk stratification Severity grading: As per ACG 2018 guidelines, identify severe AH in patients with a Maddrey's discriminant function score > 32 or MELD score > 20. E As per EASL 2012 guidelines, stratify patients with severe alcoholic steatohepatitis using available scoring systems to identify patients who are at risk of death within 1-3 months. A As per AASLD 2010 guidelines: Stratify patients presenting with a high clinical suspicion of AH using Maddrey's discriminant function, as well as other available clinical data. B Consider serial calculation of the MELD score to evaluate a patient's condition over time. B 3. Diagnostic investigations https://web.pathway.md/diseases/recuYBTsmBvoQCsjt 2/5 6/23/23, 2:17 AM Alcoholic hepatitis Pathway Screening for infections: assess patients for acute infections as part of the routine workup of patients with AH. E 4. Diagnostic procedures Liver biopsy: As per ACG 2018 guidelines, perform a transjugular liver biopsy in patients with suspected AH when the clinical diagnosis is confounded by another etiology of liver disease, or there is uncertainty on alcohol consumption history. E As per EASL 2012 guidelines, perform liver biopsy if establishing a definite diagnosis of alcoholic steatohepatitis is desired, although the presence of AH can be suspected on clinical and biochemical grounds. A As per AASLD 2010 guidelines, consider performing liver biopsy, after a risk-benefit assessment, in patients with a clinical diagnosis of severe AH for whom medical treatment is contemplated, or for those in whom reasonable uncertainty exists regarding the underlying diagnosis. B 5. Medical management Supportive care: institute appropriate measures to prevent renal injury, such as avoidance of nephrotoxic drugs, judicious use of diuretics, and use a low threshold for expanding circulating blood volume with albumin or saline infusions. E Indications for corticosteroids: As per ACG 2018 guidelines: Treat patients with severe AH (Maddrey's discriminant function score > 32 or MELD score > 20) with corticosteroids (prednisolone 40 mg/day for 28 days, typically followed by discontinuation or a 2-week taper) if there are no contraindications for their use. B Discontinue corticosteroid therapy at 7 days in nonresponders, as defined by a Lille score > 0.45. E Landmark trials: STOPAH In patients with severe alcoholic hepatitis, treatment with pentoxifylline, as compared with placebo, did not reduce 28-day mortality. Treatment with prednisolone, as compared with placebo, was associated with a reduction in 28-day mortality that did not reach significance, with no improvement in outcomes at 90 days or 1 year. Serious infections occurred in 13% of the patients treated with prednisolone versus 7% of those who did not receive prednisolone (p=0.002). Thursz MR et al. N Engl J Med. 2015 Apr 23. https://web.pathway.md/diseases/recuYBTsmBvoQCsjt 3/5 6/23/23, 2:17 AM Alcoholic hepatitis Pathway As per EASL 2012 guidelines, administer corticosteroids as first-line therapy in patients with severe alcoholic steatohepatitis who do not have ongoing sepsis. B As per AASLD 2010 guidelines, consider a four week course of prednisolone (40 mg/day for 28 days, typically followed by discontinuation or a 2-week taper) in patients with severe disease (Maddrey's discriminant function 32, with or without hepatic encephalopathy) who lack contraindications to steroid use. B Indications for pentoxifylline: As per ACG 2018 guidelines, avoid the use of pentoxifylline for patients with severe AH. D As per EASL 2012 guidelines, consider administering pentoxifylline as first-line therapy in patients with severe AH in whom ongoing sepsis is present. B As per AASLD 2010 guidelines, consider pentoxifylline therapy in patients with severe AH (Maddrey's discriminant function > 32), especially if there are contraindications to steroid therapy. B N-acetylcysteine therapy: consider initiating N-acetylcysteine therapy in patients with severe alcoholic steatohepatitis receiving corticosteroids. C 6. Inpatient care Monitoring for infections and renal injury: monitor patients with severe alcoholic steatohepatitis for deterioration of renal function and incidence of new infection. A 7. Nonpharmacologic interventions Nutritional support: as per ACG 2018 guidelines, consider nutritional supplementation in patients with AH to maintain adequate caloric intake and correct specific deficits. C Alcohol use cessation: As per EASL 2012 guidelines, advise total alcohol abstinence in patients with ALD, as persistent alcohol intake is associated with disease progression. A As per AASLD 2010 guidelines, advise strict abstinence in patients with evidence of alcohol- induced liver disease, because continued alcohol use is associated with disease progression. B 8. Surgical interventions Liver transplantation: as per ACG 2018 guidelines, consider liver transplantation for highly selected patients with severe AH. B Landmark trials: Early liver transplantation for severe alcoholic hepatitis https://web.pathway.md/diseases/recuYBTsmBvoQCsjt 4/5 6/23/23, 2:17 AM Alcoholic hepatitis Pathway In patients with no prior episodes of alcoholic hepatitis and had scores of 0.45 according to the Lille model, undergoing early liver transplantation was superior to not undergoing liver transplantation with respect to cumulative survival at 6 months. Mathurin P et al. N Engl J Med. 2011 Nov 10. 9. Follow-up and surveillance Palliative care: consider palliative care for patients with multi-organ failure who are non- responsive to corticosteroids and ineligible for early liver transplantation. E References 1. Singal AK, Bataller R, Ahn J et al. ACG Clinical Guideline: Alcoholic Liver Disease. Am J Gastroenterol. 2018 Feb;113 2 175 194. Open 2. European Association for the Study of Liver. EASL clinical practical guidelines: management of alcoholic liver disease. J Hepatol. 2012 Aug;57 2 399 420. Open 3. O'Shea RS, Dasarathy S, McCullough AJ et al. Alcoholic liver disease. Hepatology. 2010 Jan;51 1 307 28. Open 4. Vikrant Rachakonda, Ramon Bataller, Andres Duarte-Rojo. Recent advances in alcoholic hepatitis. 2020 Feb 10;9 F1000 Faculty Rev-97.2020 Feb 10;9 F1000 Faculty Rev-97. Open 5. Maneerat Chayanupatkul, Suthat Liangpunsakul. Alcoholic hepatitis: a comprehensive review of pathogenesis and treatment. 2014 May 28;20 20 6279 86.2014 May 28;20 20 6279 86. Open 6. O'Shea RS, McCullough AJ. Treatment of alcoholic hepatitis. Clin Liver Dis. 2005 Feb;9 1 103 34. Open 7. Alexandre Louvet, Julien Labreuche, Thong Dao et al. Effect of Prophylactic Antibiotics on Mortality in Severe Alcohol-Related Hepatitis: A Randomized Clinical Trial. JAMA. 2023 May 9;329 18 1558 1566. Open https://web.pathway.md/diseases/recuYBTsmBvoQCsjt 5/5
Guideline sources The following summarized guidelines for the evaluation and management of allergic bronchopulmonary aspergillosis (ABPA) are prepared by our editorial team based on guidelines from the Infectious Diseases Society of America (IDSA 2016) and the American Thoracic Society (ATS 2011). 1 2 Calculator Calculator ISHAM criteria for allergic bronc Rosenberg-Patterson criteria for Guidelines 1. Diagnostic investigations Immunoglobulin E: obtain Aspergillus IgE and total IgE for screening and to establish the diagnosis. A 2. Medical management Antifungal therapy: https://web.pathway.md/diseases/recW9gfrXTTJuzchh 1/2 6/23/23, 2:18 AM Allergic bronchopulmonary aspergillosis Pathway Consider initiating oral itraconazole with therapeutic drug monitoring in symptomatic patients with asthma and bronchiectasis or mucoid impaction despite oral or ICS therapy. C Consider initiating oral itraconazole in patients with cystic fibrosis experiencing frequent exacerbations and/or falling FEV in 1 second to minimize corticosteroid use with therapeutic drug monitoring. Consider initiating other mold-active azole therapies if therapeutic levels cannot be achieved. C Corticosteroids: initiate prednisone (or other corticosteroid equivalent) at a starting dose of 0.5 mg/kg/day, with the dose tapering as indicated by symptom improvement, in patients with ABPA. A Show 3 more Bronchodilators: consider offering bronchodilators in selected patients with mild exacerbations of ABPA. C Leukotriene antagonists: consider offering leukotriene antagonists in selected patients with mild exacerbations of ABPA. C Serial monitoring: obtain regular monitoring of serum IgE levels, serial monitoring of pulmonary function tests, and chest imaging in all patients with ABPA. Adjust the corticosteroid dosing when imaging evidence, such as infiltrates, mucoid impaction, fibrosis, or worsening bronchiectasis, is present. B References 1. Andrew H Limper, Kenneth S Knox, George A Sarosi et al. An official American Thoracic Society statement: Treatment of fungal infections in adult pulmonary and critical care patients. Am J Respir Crit Care Med. 2011 Jan 1;183 1 96 128. Open 2. Patterson TF, Thompson GR rd, Denning DW et al. Practice Guidelines for the Diagnosis and Management of Aspergillosis: 2016 Update by the Infectious Diseases Society of America. Clin Infect Dis. 2016 Aug 15;63 4):e1-e60. Open 3. R Agarwal, A Chakrabarti, A Shah et al. Allergic bronchopulmonary aspergillosis: review of literature and proposal of new diagnostic and classification criteria. Clin Exp Allergy. 2013 Aug;43 8 850 73. Open 4. M Rosenberg, R Patterson, R Mintzer et al. Clinical and immunologic criteria for the diagnosis of allergic bronchopulmonary aspergillosis. Ann Intern Med. 1977 Apr;86 4 405 14. Open https://web.pathway.md/diseases/recW9gfrXTTJuzchh 2/2
Guideline sources The following summarized guidelines for the evaluation and management of allergic conjunctivitis are prepared by our editorial team based on guidelines from the American Academy of Ophthalmology (AAO 2022; 2019), the Japanese Society of Allergology (JSA/JSOA 2022), the Brazilian Pediatric Ophthalmology Society (SBOP 2021), and the Spanish Ocular Surface and Cornea Group (GESOC/SEAIC 2015). 1 2 3 4 5 Calculator DECA criteria for suspicion of all Guidelines 1. Screening and diagnosis Diagnostic criteria: Diagnose allergic conjunctivitis based on family and personal history of atopy, characteristic clinical signs and symptoms, and results of appropriate additional tests. B Use data of https://web.pathway.md/diseases/recSetUMBWeOOBYEY 1/5 6/23/23, 2:18 AM Allergic conjunctivitis Pathway response to topical antihistamines and/or mastocyte stabilizers to support the diagnosis of allergic conjunctivitis. A Consider using the DECA criteria for clinical suspicion of allergic conjunctivitis. C 2. Medical management Topical corticosteroids: As per AAO 2022 guidelines, consider offering topical corticosteroids in patients with acute exacerbations of vernal/atopic conjunctivitis. B As per JSOA 2022 guidelines, consider offering corticosteroid eye drops for the treatment of seasonal or allergic conjunctivitis, C , vernal keratoconjunctivitis, and atopic keratoconjunctivitis. B As per GESOC 2015 guidelines, do not use ocular corticosteroids as first-line therapy in patients with allergic conjunctivitis. Offer less potent corticosteroids (such as fluorometholone, medrysone, loteprednol, or rimexolone) for the treatment of moderate inflammation. Offer betamethasone, dexamethasone, or prednisolone in patients with severe inflammation. D Topical antihistamines: As per AAO 2022 guidelines, offer OTC antihistamines or second-generation topical H1RAs in patients with mild allergic conjunctivitis. B Offer mast cell membrane stabilizers if the condition is frequently recurrent or persistent. B As per GESOC 2015 guidelines, offer second-generation topical antihistamines (levocabastine, emedastine) in patients with allergic conjunctivitis due to their long half-life (4-6 hours) and a good safety and efficacy profile, even in pediatric patients. A Show 2 more Topical vasoconstrictors: As per AAO 2022 guidelines, offer OTC vasoconstrictors in patients with mild allergic conjunctivitis. B As per GESOC 2015 guidelines, do not use vasoconstrictors (naphazoline, oxymetazoline, phenylephrine, tetrahydrozoline) in patients with allergic conjunctivitis because of their short- lasting effect, limited efficacy, and poor tolerance. D Topical cyclosporine: As per AAO 2022 guidelines, consider offering topical cyclosporine in patients with severe vernal/atopic conjunctivitis. B As per JSOA 2022 guidelines: Consider offering cyclosporine eye drops for the treatment of vernal and atopic keratoconjunctivitis. C Consider offering combination therapy with cyclosporine and corticosteroid eye drops for vernal keratoconjunctivitis and atopic keratoconjunctivitis in patients with conjunctival proliferative changes. C https://web.pathway.md/diseases/recSetUMBWeOOBYEY 2/5 6/23/23, 2:18 AM Allergic conjunctivitis Pathway As per AAO 2019 guidelines, consider offering topical cyclosporin in patients with severe allergic/vernal/atopic conjunctivitis, as well as to prevent seasonal recurrences. C Topical tacrolimus: As per JSOA 2022 guidelines: Offer tacrolimus eye drops to improve the epithelial damage and giant papillae in patients with vernal keratoconjunctivitis and atopic keratoconjunctivitis. A Consider offering combination therapy with tacrolimus and corticosteroid eye drops for vernal keratoconjunctivitis and atopic keratoconjunctivitis in patients with severe conjunctival proliferative changes. C As per AAO 2019 guidelines, consider offering topical tacrolimus in patients with severe allergic conjunctivitis. C Topical NSAIDs: consider offering ophthalmic NSAIDs (diclofenac, flurbiprofen, and ketorolac) for conjunctival hyperemia and pruritus. B Intranasal corticosteroids: consider offering intranasal corticosteroids (such as mometasone furoate, and fluticasone furoate) to improve ocular symptoms by diminishing the nasal-ocular reflex in patients with allergic rhinoconjunctivitis. B Oral antihistamines: do not use first-generation H1RAs in patients with allergic conjunctivitis because of their sedative effect and anticholinergic activity. Recognize that second-generation H1RAs (bilastine, cetirizine, desloratadine, ebastine, fexofenadine, levocetirizine, loratadine, mizolastine, or rupatadine) have similar efficacy but a more manageable sedation profile and fewer adverse effects. D Oral leukotriene receptor antagonists: consider offering leukotriene receptor antagonists (mainly montelukast) for nasal symptoms in patients with allergic rhinoconjunctivitis. B Systemic immunosuppressants: consider offering systemic immunosuppressants (such as montelukast, aspirin, interferons, cyclosporine, or tacrolimus) in rare circumstances. C Specific immunotherapy: as per AAO 2022 guidelines, offer allergen-specific immunotherapy in patients with allergic conjunctivitis, recognizing that it is more effective in pediatric than adult patients. B 3. Nonpharmacologic interventions Avoidance of allergens: As per AAO 2022 guidelines, advise environment modifications in patients with vernal/atopic conjunctivitis. B As per GESOC 2015 guidelines, advise general preventative measures including specific actions to reduce exposure to house dust mite, molds, animal dander, and pollen. A Eye hygiene: As per AAO 2022 guidelines, advise applying cold compresses and ocular lubricants in patients with vernal/atopic conjunctivitis. B https://web.pathway.md/diseases/recSetUMBWeOOBYEY 3/5 6/23/23, 2:18 AM Allergic conjunctivitis Pathway As per GESOC 2015 guidelines: Advise applying cold (compresses soaked in water, preservative-free artificial tears, and saline solution) and washing allergens from the conjunctiva to relieve edema and hyperemia. A Consider advising to wear large wraparound sunglasses to prevent contact with aeroallergens and improve photophobia. B 4. Specific circumstances Pediatric patients (nonpharmacological measures): Advise the following nonpharmacological measures: general environmental measures to reduce exposure to allergens (such as elimination of domestic dust, fungi, and pollen) specific actions such as the use of cold-water compresses, preservative-free artificial tears, and local cleansing with saline solution to wash the allergens from the conjunctiva and to contract the conjunctival vessels to relieve edema and hyperemia. A Consider advising to wear sunglasses to prevent contact with suspended allergens and for photophobia relief. B Pediatric patients (pharmacotherapy): avoid offering first-generation topical antihistamines because of poor tolerance and limited effect and potency. D Show 8 more Pediatric patients, immunotherapy: As per AAO 2022 guidelines, offer allergen-specific immunotherapy in pediatric patients with allergic conjunctivitis. B As per SBOP 2021 guidelines, offer specific immunotherapy to improve ocular symptoms in patients with allergic rhinoconjunctivitis. A References 1. M C S nchez-Hern ndez, J Montero, C Rondon et al. Consensus document on allergic conjunctivitis DECA . J Investig Allergol Clin Immunol. 2015;25 2 94 106. Open 2. AAO PPP Cornea/External Disease Panel, Hoskins Center for Quality Eye Care. Cornea/External Disease Summary Benchmarks 2022. AAO. 2022 Dec. Open 3. Divya M Varu, Michelle K Rhee, Esen K Akpek et al. Conjunctivitis Preferred Practice Pattern . Ophthalmology. 2019 Jan;126 1 P94 P169. Open 4. Cristiana Soares Ronconi, Dayane C Issaho, Fabio Ejzenbaum et al. Brazilian guidelines for the monitoring and treatment of pediatric allergic conjunctivitis. Arq Bras Oftalmol. 2021 Nov 29;85 4 415 425. Open 5. Dai Miyazaki, Atsuki Fukushima, Eiichi Uchio et al. Executive summary: Japanese guidelines for allergic conjunctival diseases 2021. Allergol Int. 2022 Oct;71 4 459 471. Open https://web.pathway.md/diseases/recSetUMBWeOOBYEY 4/5 6/23/23, 2:18 AM Allergic conjunctivitis Pathway https://web.pathway.md/diseases/recSetUMBWeOOBYEY 5/5
Guideline sources The following summarized guidelines for the evaluation and management of allergic rhinitis are prepared by our editorial team based on guidelines from the American Academy of Allergy, Asthma & Immunology (AAAAI/ACAAI 2020), the Allergic Rhinitis and its Impact on Asthma (ARIA 2017), and the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF 2015). 1 2 3 4 4 4 5 6 7 Definition Allergic rhinitis is an inflammatory disease of the nasal mucosa characterized by symptoms of sneezing, nasal pruritus, airflow obstruction, and clear nasal discharge. 4 Epidemiology Common allergens that are associated with allergic rhinitis include pollen and mites (67%), animal dander and pollutants (23%), and fungal allergens (21%). 6 Pathophysiology The prevalence of physician-diagnosed allergic rhinitis is estimated at 14% in the United States. 5 Disease course https://web.pathway.md/diseases/rec4ETSKQguw0ZOAi 1/7 6/23/23, 2:18 AM Allergic rhinitis Pathway In patients with allergic rhinitis, exposure to allergens triggers an IgE-mediated hypersensitivity reaction, leading to mucosal inflammation and associated nasal symptoms. Type 2 helper T cells play a role in maintaining persistent mucosal inflammation beyond the time of allergen exposure. 4 Prognosis and risk of recurrence Allergic rhinitis can impair quality of life by interrupting sleep. It is associated with work performance impairment and missed work time. 4 7 Pathway Calculator Allergic rhinitis Diagnostic criteria for allergic rhi Diagnosis and management Guidelines 1. Screening and diagnosis Diagnostic criteria: diagnose allergic rhinitis clinically when patients present with 1 typical symptom(s) (nasal congestion, runny nose, itchy nose, or sneezing), and history and physical examination is consistent with an allergic cause. B 2. Classification and risk stratification Severity assessment: consider using a validated instrument (such as scoring system, scale, or questionnaire) to help determine the severity of rhinitis and to monitor the degree of disease control. C 3. Diagnostic investigations Clinical examination: As per AAAAI 2020 guidelines: Elicit a complete detailed history and perform physical examination in patients presenting with symptoms of rhinitis. B Review all current medications in patients presenting with symptoms of rhinitis to assess whether drug-induced rhinitis may be present. A As per AAO-HNSF 2015 guidelines, assess patients with allergic rhinitis for associated conditions such as asthma, atopic dermatitis, sleep-disordered breathing, conjunctivitis, rhinosinusitis, and otitis media. B IgE allergy testing: https://web.pathway.md/diseases/rec4ETSKQguw0ZOAi 2/7 6/23/23, 2:18 AM Allergic rhinitis Pathway As per AAAAI 2020 guidelines: Obtain aeroallergen skin prick testing or serum-specific IgE to confirm the diagnosis of allergic rhinitis in patients with a history consistent with allergic rhinitis. A Do not obtain food skin prick testing or serum-specific IgE for foods in the routine evaluation of patients presenting with signs and symptoms compatible with the diagnosis of allergic rhinitis. D As per AAO-HNSF 2015 guidelines, obtain specific IgE allergy testing: in patients in whom there is uncertainty regarding the diagnosis of allergic rhinitis in patients in whom knowledge of the specific causative allergen is needed to target therapy in patients with a lack of response to empiric treatment for allergic rhinitis. B Sinonasal imaging: avoid routine sinonasal imaging in patients presenting with symptoms consistent with a diagnosis of allergic rhinitis. D 4. Medical management Intranasal corticosteroids: As per AAAAI 2020 guidelines: Prefer intranasal corticosteroids when choosing monotherapy in patients with persistent allergic rhinitis. A Prefer intranasal corticosteroids over leukotriene receptor antagonists for the initial treatment of 15 years old patients with moderate or severe seasonal allergic rhinitis. A As per ARIA 2017 guidelines, consider offering intranasal corticosteroids, alone or in combination with oral (conditional recommendation, low certainty of evidence) or intranasal antihistamines, in patients with seasonal allergic rhinitis. C Show 4 more Intranasal antihistamines: As per AAAAI 2020 guidelines: Offer intranasal antihistamines for the initial treatment of patients with seasonal allergic rhinitis. A Offer intranasal antihistamines as first-line therapy in patients with intermittent allergic rhinitis. B As per ARIA 2017 guidelines, consider offering intranasal antihistamines in combination with intranasal corticosteroids rather than intranasal antihistamines alone in patients with seasonal allergic rhinitis. C Show 3 more As per AAO-HNSF 2015 guidelines, consider intranasal antihistamines for patients with seasonal, perennial, or episodic allergic rhinitis. C Intranasal decongestants: https://web.pathway.md/diseases/rec4ETSKQguw0ZOAi 3/7 6/23/23, 2:18 AM Allergic rhinitis Pathway Consider offering intranasal decongestants for short-term and for intermittent or episodic therapy of nasal congestion. C Consider offering intranasal decongestants for up to 5 days in patients with severe mucosal edema impairing the delivery of other intranasal agents. C Intranasal anticholinergics: consider offering intranasal ipratropium in patients with perennial allergic rhinitis having rhinorrhea as their main nasal symptom. C Intranasal cromolyn: consider offering intranasal cromolyn just before allergen exposure to reduce symptoms of allergic rhinitis from episodic allergen exposures. C Systemic corticosteroids: Consider offering a short course (5-7 days) of oral corticosteroids in patients with severe or intractable allergic rhinitis. C Avoid administering depot parenteral corticosteroids in patients with very severe or intractable allergic rhinitis. D Oral antihistamines: As per AAAAI 2020 guidelines, do not use first-generation antihistamines in patients with allergic rhinitis. Prefer second-generation antihistamines over first-generation antihistamines when prescribing oral antihistamines. D As per ARIA 2017 guidelines, consider offering either oral antihistamines or leukotriene receptor antagonists in patients with seasonal allergic rhinitis. C Show 3 more As per AAO-HNSF 2015 guidelines, administer oral second-generation/less sedating antihistamines for patients with primary complaints of sneezing and itching. B Oral leukotriene receptor antagonists: As per AAAAI 2020 guidelines, do not use montelukast for the initial treatment of patients with allergic rhinitis. D Show 2 more As per ARIA 2017 guidelines, consider offering either leukotriene receptor antagonists or oral antihistamines in patients with seasonal allergic rhinitis. C As per AAO-HNSF 2015 guidelines, avoid leukotriene receptor antagonists as primary therapy for patients with allergic rhinitis. D Oral decongestants: Be cautious when using oral decongestants in older adult and < 4 years old pediatric patients, and in patients of any age having a history of cardiac arrhythmia, angina pectoris, cerebrovascular disease, uncontrolled hypertension, bladder outlet obstruction, glaucoma, hyperthyroidism, or Tourette's syndrome. B Avoid using oral decongestants during the first trimester of pregnancy. D Combination therapy: As per AAAAI 2020 guidelines, do not use a combination of oral antihistamines and intranasal corticosteroids in preference to monotherapy with an intranasal corticosteroid for the initial https://web.pathway.md/diseases/rec4ETSKQguw0ZOAi 4/7 6/23/23, 2:18 AM Allergic rhinitis Pathway treatment of > 12 years old patients with symptoms of seasonal allergic rhinitis. D Show 7 more As per AAO-HNSF 2015 guidelines, consider combination pharmacologic therapy in patients with allergic rhinitis who do not respond adequately to monotherapy. C Allergen immunotherapy: As per AAAAI 2020 guidelines: Consider offering allergen immunotherapy (SC or sublingual tablets) through shared decision making in patients with moderate/severe allergic rhinitis: not controlled with allergen avoidance and/or pharmacotherapy choosing immunotherapy as the preferred method of treatment (such as due to the desire to avoid the adverse effects, costs, or long-term use of pharmacotherapy) desiring the potential benefit of immunotherapy to prevent or reduce the severity of comorbid conditions, such as asthma. C Consider offering allergen immunotherapy (SC or sublingual tablets) in patients with controlled mild and moderate asthma with coexisting allergic rhinitis. C As per AAO-HNSF 2015 guidelines, refer patients who do not respond adequately to pharmacologic therapy to a clinician who can offer immunotherapy. B 5. Nonpharmacologic interventions Avoidance of allergens: consider advising avoidance of known allergens or environmental control measures (such as removal of pets, air filtration systems, bed covers, and chemical agents formulated to kill dust mites) in patients with allergic rhinitis who have identified allergens that correlate with clinical symptoms. C Herbal products: As per AAAAI 2020 guidelines, insufficient evidence to recommend for or against the use of specific herbal products for the treatment of patients with allergic rhinitis. I As per AAO-HNSF 2015 guidelines, insufficient evidence to make recommendations regarding the use of herbal therapy for patients with allergic rhinitis. Acupuncture: As per AAAAI 2020 guidelines, insufficient evidence to recommend for or against the use of acupuncture for the treatment of patients with allergic rhinitis. I As per AAO-HNSF 2015 guidelines, consider acupuncture in patients with allergic rhinitis who are interested in nonpharmacologic therapy. C 6. Surgical interventions Inferior turbinate reduction: consider performing surgical inferior turbinate reduction in patients with nasal airway obstruction and enlarged inferior turbinates who have failed medical https://web.pathway.md/diseases/rec4ETSKQguw0ZOAi 5/7 6/23/23, 2:18 AM Allergic rhinitis Pathway management. C References 1. Mark S Dykewicz, Dana V Wallace, David J Amrol et al. Rhinitis 2020 A practice parameter update. J Allergy Clin Immunol. 2020 Oct;146 4 721 767. Open 2. Seidman MD, Gurgel RK, Lin SY et al. Clinical practice guideline: Allergic rhinitis. Otolaryngol Head Neck Surg. 2015 Feb;152 1 Suppl):S1 43. Open 3. Jan L Bro ek, Jean Bousquet, Ioana Agache et al. Allergic Rhinitis and its Impact on Asthma ARIA guidelines-2016 revision. J Allergy Clin Immunol. 2017 Oct;140 4 950 958. Open 4. Wheatley LM, Togias A. Clinical practice. Allergic rhinitis. N Engl J Med. 2015 Jan 29;372 5 456 63. Open 5. Meltzer EO, Blaiss MS, Naclerio RM et al. Burden of allergic rhinitis: allergies in America, Latin America, and Asia-Pacific adult surveys. Allergy Asthma Proc. 2012 Sep-Oct;33 Suppl 1 S113 41. Open 6. Passali D, Cingi C, Staffa P et al. The International Study of the Allergic Rhinitis Survey: outcomes from 4 geographical regions. Asia Pac Allergy. 2018 Jan 25;8 1):e7. Open 7. Vandenplas O, Vinnikov D, Blanc PD et al. Impact of Rhinitis on Work Productivity: A Systematic Review. J Allergy Clin Immunol Pract. 2018 Jul Aug;6 4 1274 1286.e9. Open 8. Larenas-Linnemann D, Luna-Pech JA, Rodriguez-Perez N et al. GUIMIT 2019, Mexican Guideline on Immunotherapy. Guideline on the diagnosis of IgE-mediated allergic disease and immunotherapy following the ADAPTE approach]. Rev Alerg Mex. 2019;66 Suppl 1 1 105. Open 9. American Academy of Ophthalmology. Choosing Wisely AAO recommendations. Choosing Wisely. 2015. Open 10. Ahmed Al-Digheari, Bassam Mahboub, Hesham Tarraf et al. The clinical burden of allergic rhinitis in five Middle Eastern countries: results of the SNAPSHOT program. Allergy Asthma Clin Immunol. 2018 Nov 19;14 63. Open 11. Eli O Meltzer, Michael S Blaiss, M Jennifer Derebery et al. Burden of allergic rhinitis: results from the Pediatric Allergies in America survey. J Allergy Clin Immunol. 2009 Sep;124 3 Suppl):S43 70. Open 12. M Fr hlich, M Pinart, T Keller et al. Is there a sex-shift in prevalence of allergic rhinitis and comorbid asthma from childhood to adulthood? A meta-analysis. Clin Transl Allergy. 2017 Dec 5;7 44. Open 13. L M Baumann, K M Romero, C L Robinson et al. Prevalence and risk factors for allergic rhinitis in two resource-limited settings in Peru with disparate degrees of urbanization. Clin Exp Allergy. 2015 Jan;45 1 192 9. Open 14. Lia Restimulia, Dwi Reno Pawarti, Haris Mayaguyang Ekorini. The Relationship between Serum Vitamin D Levels with Allergic Rhinitis Incidence and Total Nasal Symptom Score in Allergic Rhinitis Patients. Open Access Maced J Med Sci. 2018 Aug 10;6 8 1405 1409. Open 15. Lisa M Wheatley, Alkis Togias. Clinical practice. Allergic rhinitis. N Engl J Med. 2015 Jan 29;372 5 456 63. Open https://web.pathway.md/diseases/rec4ETSKQguw0ZOAi 6/7 6/23/23, 2:18 AM Allergic rhinitis Pathway 16. Zhisheng Shao, Jonathan A Bernstein. Occupational Rhinitis: Classification, Diagnosis, and Therapeutics. Curr Allergy Asthma Rep. 2019 Nov 27;19 12 54. Open https://web.pathway.md/diseases/rec4ETSKQguw0ZOAi 7/7
Guideline sources The following summarized guidelines for the evaluation and management of allied disorders of Hirschsprung's disease are prepared by our editorial team based on guidelines from the Ministry of Health, Labour and Welfare of Japan (MHLW 2018). 1 Calculator Calculator Diagnostic criteria for chronic idi Diagnostic criteria for megacysti Guidelines 1. Screening and diagnosis Diagnosis: suspect the diagnosis of megacystis microcolon intestinal hypoperistalsis syndrome in patients with observed microcolon and megalocystis confirmed on cystography, CT, or ultrasound. https://web.pathway.md/diseases/rec0NgqXVyqnFzyMM 1/4 6/23/23, 2:18 AM Allied disorders of Hirschsprung's disease Pathway B Differential diagnosis: differentiate mechanical obstruction and secondary intestinal pseudo- obstruction from chronic idiopathic intestinal pseudo-obstruction in adult patients. B 2. Classification and risk stratification Prognosis: expect long-term survival with appropriate treatment in patients with isolated hypoganglionosis and megacystis microcolon intestinal hypoperistalsis syndrome with appropriate treatment. B Show 2 more 3. Diagnostic investigations History and physical examination: elicit clinical history and perform physical examination to determine the duration of symptoms and bowel obstruction conditions in patients with suspected chronic idiopathic intestinal pseudo-obstruction. B Diagnostic imaging: Obtain diagnostic imaging to identify intestinal dilatation, air-fluid levels, and exclude mechanical obstruction in patients with suspected chronic idiopathic intestinal pseudo-obstruction. B Obtain gastrointestinal series in neonatal patients with suspected megacystis microcolon intestinal hypoperistalsis syndrome presenting with symptoms of bowel obstruction, such as abdominal distension and vomiting, and without organic obstruction. B 4. Diagnostic procedures Biopsy: perform a full-thickness biopsy of the gastrointestinal tract, especially the small intestine and sigmoid colon, for a definitive diagnosis of isolated hypoganglionosis. B Show 3 more 5. Medical management Pharmacologic therapy: Insufficient evidence to support the use of any medication for the management of patients with isolated hypoganglionosis. I Insufficient evidence to support the use of any of the following medications for the management of patients with chronic idiopathic intestinal pseudo-obstruction: prucalopride cisapride antibiotics (erythromycin). I https://web.pathway.md/diseases/rec0NgqXVyqnFzyMM 2/4 6/23/23, 2:18 AM Allied disorders of Hirschsprung's disease Pathway 6. Nonpharmacologic interventions Nutrition: Offer enteral and parenteral nutrition for nutritional management of patients with isolated hypoganglionosis, megacystis microcolon intestinal hypoperistalsis syndrome, and chronic idiopathic intestinal pseudo-obstruction. B Recognize that adequate nutritional support is required for a long period of time in many patients with isolated hypoganglionosis, megacystis microcolon intestinal hypoperistalsis syndrome, and chronic idiopathic intestinal pseudo-obstruction. B Probiotics: insufficient evidence to support the use of Japanese herbal medicine (daikenchuto) for the management of patients with megacystis microcolon intestinal hypoperistalsis syndrome or chronic idiopathic intestinal pseudo-obstruction. I Herbal products: insufficient evidence to support the use of probiotics for the management of patients with megacystis microcolon intestinal hypoperistalsis syndrome or chronic idiopathic intestinal pseudo-obstruction. I 7. Therapeutic procedures Antegrade continence enema: consider performing antegrade continence enema and antidromic enema to improve defecation in patients with chronic idiopathic intestinal pseudo-obstruction. C 8. Surgical interventions Intestinal decompression: consider performing upper jejunostomy in patients with isolated hypoganglionosis. C Show 4 more Radical surgery: consider avoiding radical surgery in patients with isolated hypoganglionosis. D Show 2 more Small bowel transplantation: Consider performing small bowel transplantation in patients with isolated hypoganglionosis in the following cases: discontinuation of parenteral nutrition is difficult even after the maximum use of the native intestinal tract under intestinal rehabilitation the number of central venous access routes is low repeated episodes of sepsis progressive hepatopathy. C Show 4 more References https://web.pathway.md/diseases/rec0NgqXVyqnFzyMM 3/4 6/23/23, 2:18 AM Allied disorders of Hirschsprung's disease Pathway 1. Mitsuru Muto, Hiroshi Matsufuji, Tomoaki Taguchi et al. Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease, 2017. Pediatr Int. 2018 May;60 5 400 410. Open https://web.pathway.md/diseases/rec0NgqXVyqnFzyMM 4/4
Guideline sources The following summarized guidelines for the management of alopecia areata are prepared by our editorial team based on guidelines from the British Photodermatology Group (BPG/BAD 2019), the British Association of Dermatologists (BAD 2012), and the American Association of Family Physicians (AAFP 2009). 1 2 3 Guidelines 1. Medical management Topical therapy: As per BAD 2012 guidelines: Offer potent topical corticosteroids in patients with limited alopecia areata. B Recognize that other topical treatment options include minoxidil B and dithranol. B As per AAFP 2009 guidelines, offer topical corticosteroids applied BID in patients with alopecia areata. B Show 3 more Contact immunotherapy: As per BAD 2012 guidelines, offer contact immunotherapy in patients with extensive alopecia areata or alopecia totalis/universalis. B https://web.pathway.md/diseases/recCA3d9B062j9g6j 1/3 6/23/23, 2:18 AM Alopecia areata Pathway As per AAFP 2009 guidelines, offer topical immunotherapy with diphenylcyclopropenone or squaric acid applied by dermatologist every few weeks in patients with alopecia areata. B Systemic therapy: As per BAD 2012 guidelines, recognize that the following systemic agents have limited roles in the management of patients with alopecia areata: systemic corticosteroids B calcineurin inhibitors B sulfasalazine B methotrexate B isoprinosine B biologic agents. B As per AAFP 2009 guidelines: Offer a 6-week tapering course of oral prednisone starting at 40 mg/day in patients with alopecia areata. B Recognize that oral corticosteroids and cyclosporine have no long-term benefits in patients with alopecia areata. A 2. Nonpharmacologic interventions Wig and hairpiece: Offer wig/hairpiece use in patients with extensive alopecia areata. B Offer wig use in patients with alopecia totalis/universalis. B Alternative and complementary therapies: recognize that aromatherapy and hypnotherapy have limited roles in the management of patients with alopecia areata. B 3. Therapeutic procedures Intralesional corticosteroids: As per BAD 2012 guidelines, consider performing intralesional corticosteroid injections in patients with limited alopecia areata. C As per AAFP 2009 guidelines, consider performing intralesional injections of triamcinolone acetonide 5-10 mg/mL; 0.1 mL injected with a 30-gauge needle into the dermis 1 cm apart to a maximum of 3 mL. Consider repeating every 4-6 weeks. C Phototherapy: As per BPG 2019 guidelines, insufficient evidence to support the use of photodynamic therapy in patients with alopecia areata. I As per BAD 2012 guidelines, recognize that psoralen-UV A therapy has a limited role in the management of patients with alopecia areata. B https://web.pathway.md/diseases/recCA3d9B062j9g6j 2/3 6/23/23, 2:18 AM Alopecia areata Pathway As per AAFP 2009 guidelines, recognize that photodynamic therapy has no long-term benefits in patients with alopecia areata. A Laser therapy: recognize that laser therapy has a limited role in the management of patients with alopecia areata. B 4. Specific circumstances Eyelash alopecia: recognize that prostaglandin F2-alpha analogs (latanoprost and bimatoprost) have limited roles in the management of patients with eyelash alopecia. B References 1. A G Messenger, J McKillop, P Farrant et al. British Association of Dermatologists' guidelines for the management of alopecia areata 2012. Br J Dermatol. 2012 May;166 5 916 26. Open 2. Anne L Mounsey, Sean W Reed. Diagnosing and treating hair loss. Am Fam Physician. 2009 Aug 15;80 4 356 62. Open 3. T H Wong, C A Morton, N Collier et al. British Association of Dermatologists and British Photodermatology Group guidelines for topical photodynamic therapy 2018. Br J Dermatol. 2019 Apr;180 4 730 739. Open 4. Messenger AG, McKillop J, Farrant P et al. British Association of Dermatologists' guidelines for the management of alopecia areata 2012. Br J Dermatol. 2012 May;166 5 916 26. Open 5. Brett King, Manabu Ohyama, Ohsang Kwon et al. Two Phase 3 Trials of Baricitinib for Alopecia Areata. N Engl J Med. 2022 May 5;386 18 1687 1699. Open 6. Brett King, Manabu Ohyama, Ohsang Kwon et al. Two Phase 3 Trials of Baricitinib for Alopecia Areata. N Engl J Med. 2022 May 5;386 18 1687 1699. Open https://web.pathway.md/diseases/recCA3d9B062j9g6j 3/3
Guideline sources The following summarized guidelines for the evaluation and management of alpha-1 antitrypsin deficiency (AATD) are prepared by our editorial team based on guidelines from the National Comprehensive Cancer Network (NCCN 2022), the Global Initiative for Chronic Obstructive Lung Disease (GOLD 2022), the European Respiratory Society (ERS 2017), the Alpha-1 Foundation (Alpha-1 2016), the American Association for the Study of Liver Diseases (AASLD 2014), and the Canadian Thoracic Society (CTS 2012). 1 2 3 4 5 6 7 Guidelines 1. Screening and diagnosis Clinical presentation: Recognize that lung disease in AAT deficiency generally presents at a younger age than usual COPD and may be misdiagnosed as asthma. E Recognize that patients with AAT deficiency are more likely to have basal emphysema than patients with usual COPD. E Screening of family relatives: https://web.pathway.md/diseases/recnIrSPaCPOnucJJ 1/5 6/23/23, 2:19 AM Alpha-1 antitrypsin deficiency Pathway As per ERS 2017 guidelines, consider offering testing for AAT deficiency after appropriate counseling in family relatives of identified patients. E As per Alpha-1 2016 guidelines, provide genetic counseling and offer testing for AAT deficiency in adult siblings of patients with an abnormal AAT gene, whether heterozygote or homozygote. B Show 2 more Indications for testing, lung disease: As per ERS 2017 guidelines, consider testing for AAT deficiency in patients with bronchiectasis in the presence of basal emphysema or early onset airflow obstruction. As per ERS 2017 guidelines, test for AAT deficiency in all patients with COPD or adult-onset asthma. E As per Alpha-1 2016 guidelines: Test for AAT deficiency in all patients with COPD, regardless of age or ethnicity. B Test for AAT deficiency in all patients with unexplained bronchiectasis. B As per CTS 2012 guidelines: Consider obtaining targeted testing for AAT deficiency in patients with COPD diagnosed before 65 years of age or with a smoking history of < 20 pack-years. C Avoid obtaining targeted testing for AAT deficiency in patients with bronchiectasis or asthma. D Indications for testing (liver disease): test for AAT deficiency in all patients with unexplained chronic liver disease. B Indications for testing (vasculitis): test for AAT deficiency in all patients with granulomatosis with polyangiitis. B Indications for testing (panniculitis): test for AAT deficiency in all patients with necrotizing panniculitis. B 2. Diagnostic investigations AAT levels: As per ERS 2017 guidelines, obtain quantitative measurement of AAT levels in the blood to identify AAT deficiency. E As per Alpha-1 2016 guidelines, measure AAT level for advanced or confirmatory testing. Protein electrophoresis: As per ERS 2017 guidelines, obtain protein phenotyping by isoelectric focusing to identify variants where AAT is present in the sample including the rarer variants F, I, and P. E As per Alpha-1 2016 guidelines, obtain Pi-typing for advanced or confirmatory testing. Genetic testing: https://web.pathway.md/diseases/recnIrSPaCPOnucJJ 2/5 6/23/23, 2:19 AM Alpha-1 antitrypsin deficiency Pathway As per ERS 2017 guidelines, obtain genetic testing for AAT deficiency to support quantitative testing. E Show 2 more As per Alpha-1 2016 guidelines, obtain genotyping for at least the S and Z alleles for diagnostic testing of symptomatic patients. Obtain expanded genotyping for advanced or confirmatory testing. Pulmonary function testing: obtain initial complete lung function testing in all patients with AAT deficiency. B Chest imaging: obtain a baseline chest CT in newly diagnosed patients being symptomatic and/or having abnormal pulmonary function testing. B 3. Medical management Setting of care: manage patients with AAT deficiency in supervision by reference centers of excellence at a national or regional level. E Augmentation therapy: As per GOLD 2022 guidelines, consider initiating IV augmentation therapy to slow down the progression of emphysema in patients with AAT deficiency and COPD (with never or ex- smokers with an FEV in 1 second of 35-60% of predicted and patients with severe hereditary AAT deficiency with established emphysema being the most suitable candidates). C As per ERS 2017 guidelines: Initiate IV augmentation therapy to reduce the progression of emphysema in patients with severe AAT deficiency. E Insufficient evidence to support the use of augmentation therapy in PiSZ or PiMZ subjects or current smokers of any protein phenotype. I As per Alpha-1 2016 guidelines, initiate IV augmentation therapy in patients with AAT deficiency and an FEV in 1 second in the range of 30-65% of predicted. A Show 10 more As per CTS 2012 guidelines, consider initiating AAT augmentation therapy in non-smoking or ex-smoking patients with COPD with a FEV in 1 second in the range of 25-80% of predicted attributable to emphysema and documented low levels of AAT, i.e. 11 mcmol/L, receiving optimal pharmacological and nonpharmacological therapies, including comprehensive case management and pulmonary rehabilitation, for the benefits in CT lung density C and mortality. C 4. Nonpharmacologic interventions Smoking cessation: advise smoking cessation and avoidance of exposure to tobacco in patients with AAT deficiency. A https://web.pathway.md/diseases/recnIrSPaCPOnucJJ 3/5 6/23/23, 2:19 AM Alpha-1 antitrypsin deficiency Pathway 5. Surgical interventions Lung volume reduction surgery: As per ERS 2017 guidelines, consider performing surgical volume reduction and endobronchial valve placement in selected patients with AAT deficiency, with a careful appraisal of risks and benefits assessed by a multidisciplinary team experienced in lung volume reduction and AAT deficiency. E As per Alpha-1 2016 guidelines, do not perform lung volume reduction surgery in patients with COPD related to AAT deficiency. D Lung transplantation: recognize that patients with AAT deficiency generally have improved quality of life following lung transplantation, however insufficient evidence regarding the survival benefit of lung transplantation. E Liver transplantation: As per AASLD 2014 guidelines: Offer liver transplantation in patients with decompensated cirrhosis due to AAT deficiency. B Obtain pulmonary function testing and chest imaging to exclude lung disease in patients with AAT deficiency being evaluated for liver transplantation. B 6. Follow-up and surveillance Surveillance for lung disease: As per ERS 2017 guidelines, obtain annual pulmonary function testing including post- bronchodilator FEV in 1 second and gas transfer to monitor for disease progression. E Show 3 more As per Alpha-1 2016 guidelines: Obtain annual spirometry during follow-up of adult patients with AAT deficiency with normal baseline spirometry. B Do not obtain serial chest CT to monitor the progression of the disease. D Surveillance for liver disease: Obtain the following at annual intervals (or more frequently as indicated by clinical circumstances) to monitor for liver disease in patients with AAT deficiency: physical examination, including a focused examination for signs of liver disease liver ultrasound laboratory monitoring of AST, ALT, gamma-glutamyltransferase, albumin, bilirubin, INR, and platelets. B Surveillance for hepatocellular carcinoma: obtain surveillance for HCC in patients with AAT deficiency and cirrhosis. B References https://web.pathway.md/diseases/recnIrSPaCPOnucJJ 4/5 6/23/23, 2:19 AM Alpha-1 antitrypsin deficiency Pathway 1. Al B Benson, Michael I D'Angelica, Daniel E Abbott et al. Hepatobiliary Cancers, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology. NCCN. 2022 Jul 15. Open 2. Robert A Sandhaus, Gerard Turino, Mark L Brantly et al. The Diagnosis and Management of Alpha-1 Antitrypsin Deficiency in the Adult. Chronic Obstr Pulm Dis. 2016 Jun 6;3 3 668 682. Open 3. Darcy D Marciniuk, P Hernandez, M Balter et al. Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: a Canadian Thoracic Society clinical practice guideline. Can Respir J. 2012 Mar- Apr;19 2 109 16. Open 4. Marc Miravitlles, Asger Dirksen, Ilaria Ferrarotti et al. European Respiratory Society statement: diagnosis and treatment of pulmonary disease in 1 antitrypsin deficiency. Eur Respir J. 2017 Nov 30;50 5 1700610. Open 5. Paul Martin, Andrea DiMartini, Sandy Feng et al. Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology. 2014 Mar;59 3 1144 65. Open 6. Polverino E, Goeminne PC, McDonnell MJ et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J. 2017 Sep 9;50 3 . pii: 1700629. Open 7. Alvar Agusti, Richard Beasley, Bartolome R. Celli et al. Global Strategy for the Diagnosis, Management, and Prevention of Chronic Obstructive Pulmonary Disease: 2022 Report. GOLD. 2022. Open https://web.pathway.md/diseases/recnIrSPaCPOnucJJ 5/5
Guideline sources The following summarized guidelines for the evaluation and management of Alzheimer's disease are prepared by our editorial team based on guidelines from the European Neurological Society (ENS/EAN/EFNS 2015), the American Association of Family Physicians (AAFP 2011), and the European Federation of Neurological Societies (EFNS 2010). 1 2 3 Guidelines 1. Screening and diagnosis Screening for comorbidities: assess patients with Alzheimer's disease for comorbidity, both at the time of diagnosis and throughout the course of the illness E and always consider it as a possible cause of behavioral and psychological symptoms of dementia. B 2. Diagnostic investigations https://web.pathway.md/diseases/recUuM8OqQfI3m3Ej 1/5 6/23/23, 2:19 AM Alzheimer's disease Pathway History and physical examination: elicit a clinical history supplemented by an informant. A Show 2 more Cognitive assessment: assess for impairment of activities of daily living due to cognitive decline in the diagnostic evaluation as it is an essential part of the diagnostic criteria for dementia. A Show 4 more Laboratory studies: Obtain the following tests in patients with Alzheimer's disease at the time of diagnosis: complete blood cell count LFTs renal function tests folate vitamin B12 thyroid stimulating hormone calcium glucose. E Consider serological testing for the following disorders in patients with atypical presentation of Alzheimer's disease or clinical features suggestive of these disorders: syphilis borelia HIV. E Imaging: consider CT and MRI to exclude treatable causes of dementia. Consider multislice CT and coronal MRI to assess hippocampal atrophy to support a clinical diagnosis of Alzheimer's disease. C Show 3 more Electroencephalography: consider electroencephalography in the differential diagnosis of atypical clinical presentations of Alzheimer's disease (good practice point) and when Creutzfeldt- Jakob disease or transient epileptic amnesia is suspected. C Genetic testing: Consider screening for known pathogenic mutations in patients with appropriate phenotype or a family history of an autosomal dominant dementia. Do not routinely obtain Apolipoprotein E genotyping in patients with Alzheimer's disease. 3. Diagnostic procedures Cerebrospinal fluid analysis: Obtain routine CSF analysis in the differential diagnosis of atypical clinical presentations of Alzheimer's disease. E https://web.pathway.md/diseases/recUuM8OqQfI3m3Ej 2/5 6/23/23, 2:19 AM Alzheimer's disease Pathway Obtain CSF 14-3-3 or total tau protein measurements for the identification of Creutzfeldt-Jakob disease in patients with rapidly progressive dementia. Alterations in CSF total tau, phospho-tau and Ab42 support diagnosis of Alzheimer's disease. B 4. Medical management Cholinesterase inhibitors: As per AAFP 2011 guidelines, offer cholinesterase inhibitors for patients with mild-to-moderate Alzheimer's disease based on their modest effectiveness, although limited by their adverse effects. A As per EFNS 2010 guidelines, consider cholinesterase inhibitors (donepezil, galantamine, or rivastigmine) in patients with Alzheimer's disease at the time of diagnosis, taking into account expected therapeutic benefits and potential safety issues. Benefits on cognitive and non- cognitive symptoms have been demonstrated in patients with mild, moderate and severe disease. B Memantine: As per EFNS 2015 guidelines, consider memantine in patients with moderate-to-severe Alzheimer's disease, taking into account expected therapeutic benefits and potential safety issues B . Benefits on cognitive and non-cognitive symptoms are apparent, some non-cognitive symptoms - agitation, delusions - may respond better than others. C As per AAFP 2011 guidelines, consider combination therapy with a cholinesterase inhibitor and memantine in patients with moderate-to-severe Alzheimer's disease. C Antipsychotics: As per AAFP 2011 guidelines, consider atypical antipsychotics to improve some behavioral manifestations in patients with Alzheimer's disease, but take into account that these agents are associated with increased mortality in older patients. C As per EFNS 2010 guidelines, reserve antipsychotics only for patients with moderate-to-severe behavioral and psychological symptoms of dementia causing significant distress which have either not responded to other treatments (such as nonpharmacological measures or cholinesterase inhibitors) or when other treatments are not appropriate. A Show 2 more Other medications: As per AAFP 2011 guidelines, do not prescribe NSAIDs, vitamin E, testosterone, estrogen, statins, and insulin sensitizers for the treatment of patients with Alzheimer's disease. D As per EFNS 2010 guidelines, do not use aspirin for the treatment of patients with Alzheimer's disease , though it can be used in patients with Alzheimer's disease who also have other indications for its use (for example, to prevent cardiovascular events). (Level A). D Show 2 more https://web.pathway.md/diseases/recUuM8OqQfI3m3Ej 3/5 6/23/23, 2:19 AM Alzheimer's disease Pathway 5. Nonpharmacologic interventions Cognitive stimulation: consider cognitive stimulation or rehabilitation in patients with mild-to- moderate Alzheimer's disease. E Occupational therapy: consider occupational therapy in patients with Alzheimer's disease to improve patients functioning and reduce need for informal care. C Addressing behavioral and psychological symptoms of dementia: carefully search for triggers and causative factors for behavioral and psychological symptoms of dementia (such as physical illness). Initiate treatment with nonpharmacological measures, where possible. B 6. Specific circumstances Patients with depression: offer SSRIs rather than TCAs for the treatment of depression in patients with Alzheimer's disease. B 7. Patient education Diagnosis disclosure: disclose the diagnosis of Alzheimer's disease to patient (and caregivers as appropriate), which should be individually tailored and accompanied by information and counseling, as well as useful contacts such as Alzheimer's disease patient organizations. B Patient and caregiver education: Provide education and support to patients with Alzheimer's disease and their caregivers. A Discuss realistic expectations for treatment effects and potential side effects with patients with Alzheimer's disease and their caregivers. E Addressing patient needs and preferences: Consider driving, medico-legal issues and the need for other support services in patients with Alzheimer's disease. E Consider encouraging patients with Alzheimer's disease, if possible, to draw up advance directives containing future treatment and care preferences. E 8. Preventative measures Prevention: Insufficient evidence to support the use of any drugs purely for the primary prevention of dementia. I Insufficient evidence to support the use of anti-inflammatory drugs, nootropics (including piracetam, nicergoline), selegiline, estrogens, pentoxyphylin, statins, EGb 761 and Cerebrolysin for the prevention of Alzheimer's disease. I https://web.pathway.md/diseases/recUuM8OqQfI3m3Ej 4/5 6/23/23, 2:19 AM Alzheimer's disease Pathway 9. Follow-up and surveillance Discontinuation of medical therapy: consider discontinuing treatment for patients with Alzheimer's disease who continue to experience decline despite maximal therapy. C Follow-up: Regular patient follow-up, which should include scales like the MMSE to monitor response to treatment and disease progression, should be an integral part of management. E Consider follow-up with serial MRI in a clinical setting to document disease progression. E References 1. Bradford T Winslow, Mary K Onysko, Christian M Stob et al. Treatment of Alzheimer Disease. Am Fam Physician. 2011 Jun 15;83 12 1403 12. Open 2. J Hort, J T O'Brien, G Gainotti et al. EFNS guidelines for the diagnosis and management of Alzheimers disease. Eur J Neurol. 2010 Oct;17 10 1236 48. Open 3. R Schmidt, E Hofer, F H Bouwman et al. EFNS ENS/EAN Guideline on concomitant use of cholinesterase inhibitors and memantine in moderate to severe Alzheimer's disease. Eur J Neurol. 2015 Jun;22 6 889 98. Open 4. APA Work Group on Alzheimer's Disease and other Dementias, Rabins PV, Blacker D et al. American Psychiatric Association practice guideline for the treatment of patients with Alzheimer's disease and other dementias. Second edition. Am J Psychiatry. 2007 Dec;164 12 Suppl):5 56. Open 5. American College of Medical Genetics. Choosing Wisely ACMG recommendations. Choosing Wisely. 2015. Open 6. Jeff Sevigny, Ping Chiao, Thierry Bussi re et al. The antibody aducanumab reduces A plaques in Alzheimer's disease. Nature. 2016 Sep 1;537 7618 50 6. Open 7. S Budd Haeberlein, P S Aisen, F Barkhof et al. Two Randomized Phase 3 Studies of Aducanumab in Early Alzheimer's Disease. J Prev Alzheimers Dis. 2022;9 2 197 210. Open 8. Christopher H van Dyck, Chad J Swanson, Paul Aisen et al. Lecanemab in Early Alzheimer's Disease. N Engl J Med. 2023 Jan 5;388 1 9 21. Open https://web.pathway.md/diseases/recUuM8OqQfI3m3Ej 5/5
Guideline sources The following summarized guidelines for the evaluation and management of amblyopia are prepared by our editorial team based on guidelines from the American Association for Pediatric Ophthalmology and Strabismus (AAPOS 2018) and the U.S. Preventive Services Task Force (USPSTF 2017). 1 2 3 3 3 4 5 Definition Amblyopia is a neurological disease characterized by cortical visual impairment, with loss or lack of development of central vision in one eye that is unrelated to primary ocular pathology. 3 Epidemiology Abnormal or inadequate stimulation of the visual system during its early developmental period results in amblyopia. Common etiologies include strabismus, refractive errors (anisometropia, and high hyperopia), and structural ocular abnormalities (ptosis, corneal opacity, vitreous hemorrhage, and congenital cataract). 3 Pathophysiology The prevalence of amblyopia in the United States is estimated to be less than 2000 cases per 100,000 individuals. 4 Disease course https://web.pathway.md/diseases/rec5LUyQBaHsEUOlH 1/3 6/23/23, 2:34 AM Amblyopia Pathway The resulting alterations in the development of visual pathways cause clinical manifestations of visual impairment and permanent vision loss of the affected eye. 3 Prognosis and risk of recurrence Amblyopia is not associated with an increase in mortality. 5 Guidelines 1. Screening and diagnosis Indications for screening: as per USPSTF 2017 guidelines, screen pediatric patients 3-5 years of age at least once for amblyopia or its risk factors. B 2. Classification and risk stratification Correction of refractive error: treat refractive error as the initial step in the care of children 0 to 17 years of age with amblyopia. B Patching: consider using patching as first-line therapy after refractive correction in children with moderate amblyopia - 20/40 to 20/80 - B with a prescribed dose of 2 hours of daily patching or weekend atropine. C Show 2 more Filters: consider using filters for the treatment of children with mild amblyopia who do not improve with eyeglasses alone. B Pharmacological treatment: consider using pharmacological treatment that produces cycloplegia of the nonamblyopic eye for the treatment of children who do not improve with eyeglasses alone. B 3. Follow-up and surveillance Follow-up assessment: schedule a follow-up examination 2-3 months after initiation of treatment in most patients, but timing will vary according to the intensity of the treatment and the age of the child. References 1. Wallace DK, Repka MX, Lee KA et al. Amblyopia Preferred Practice Pattern. Ophthalmology. 2018 Jan;125 1 P105 P142. Open 2. US Preventive Services Task Force, Grossman DC, Curry SJ et al. Vision Screening USPSTF in Children Aged 6 Months to 5 Years: US Preventive Services Task Force Recommendation Statement. JAMA. 2017 Sep 5;318 9 836 844. Open https://web.pathway.md/diseases/rec5LUyQBaHsEUOlH 2/3 6/23/23, 2:34 AM Amblyopia Pathway 3. Papageorgiou E, Asproudis I, Maconachie G et al. The treatment of amblyopia: current practice and emerging trends. Graefes Arch Clin Exp Ophthalmol. 2019 Jun;257 6 1061 1078. Open 4. Friedman DS, Repka MX, Katz J et al. Prevalence of amblyopia and strabismus in white and African American children aged 6 through 71 months the Baltimore Pediatric Eye Disease Study. Ophthalmology. 2009 Nov;116 11 2128 34.e1 2. Open 5. van de Graaf ES, van Kempen-du Saar H, Looman CW et al. Utility analysis of disability caused by amblyopia and/or strabismus in a population-based, historic cohort. Graefes Arch Clin Exp Ophthalmol. 2010 Dec;248 12 1803 7. Open https://web.pathway.md/diseases/rec5LUyQBaHsEUOlH 3/3
Guideline sources The following summarized guidelines for the evaluation and management of amenorrhea are prepared by our editorial team based on guidelines from the Endocrine Society (ES 2020; 2018; 2017; 2013; 2011), the American College of Obstetricians and Gynecologists (ACOG 2018; 2017), the Society of Obstetricians and Gynaecologists of Canada (SOGC 2018), the American Society for Reproductive Medicine (ASRM 2017), the European Society of Human Reproduction and Embryology (ESHRE 2016), the Royal College of Obstetricians and Gynaecologists (RCOG 2014), the American Association of Family Physicians (AAFP 2013), and the American College of Sports Medicine (ACSM 2007). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 Calculator Revised Rotterdam diagnostic cr Guidelines 1. Diagnostic investigations Exclusion of pregnancy: exclude pregnancy in all patients presenting with amenorrhea. B Evaluation for etiology, functional hypothalamic amenorrhea: https://web.pathway.md/diseases/recpFmHfW5s3URMrr 1/9 6/23/23, 2:34 AM Amenorrhea Pathway gy yp As per ES 2017 guidelines, consider obtaining diagnostic evaluation for functional hypothalamic amenorrhea in adolescent and female patients if the menstrual cycle interval persistently exceeds 45 days and/or presenting with amenorrhea for 3 months. C Show 9 more As per ACSM 2007 guidelines, exclude other causes of amenorrhea to diagnose functional hypothalamic amenorrhea. B Evaluation for etiology (pimary ovarian insufficiency): exclude premature ovarian insufficiency in < 40 years old patients with amenorrhea/oligomenorrhea or estrogen-deficiency symptoms. B Evaluation for etiology (hyperprolactinemia): obtain a single measurement of serum prolactin for the diagnosis of hyperprolactinemia and confirm the diagnosis with a level above the ULN as long as the serum sample is obtained without excessive venipuncture stress. A Evaluation for etiology (polycystic ovary syndrome): Consider diagnosing PCOS in patients meeting 2 of the following criteria, in the absence of disorders mimicking PCOS (such as thyroid disease, hyperprolactinemia, non-classic congenital adrenal hyperplasia, primarily 21-hydroxylase deficiency by serum 17-hydroxyprogesterone): androgen excess ovulatory dysfunction polycystic ovaries. C Show 2 more Evaluation for etiology, congenital adrenal hyperplasia: As per ES 2018 guidelines: Consider obtaining early morning 17-hydroxyprogesterone levels in the follicular phase (or on a random day) to screen for nonclassical congenital adrenal hyperplasia due to 21- hydroxylase deficiency in patients with hyperandrogenemia and amenorrhea or infrequent menses. C Consider obtaining early morning 17-hydroxyprogesterone levels to screen for nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency in patients with hirsutism at high risk of congenital adrenal hyperplasia (positive family history, member of a high-risk ethnic group), even if serum total and free testosterone are normal. C As per ES 2017 guidelines, obtain total testosterone and DHEA sulfate in patients with clinical hyperandrogenism and 8 AM 17-hydroxyprogesterone levels when suspecting late-onset congenital adrenal hyperplasia. A Evaluation for etiology, M llerian tract anomalies: As per ACOG 2018 guidelines, recognize that patients with M llerian agenesis usually are identified during evaluations for primary amenorrhea with otherwise typical growth and pubertal development. E https://web.pathway.md/diseases/recpFmHfW5s3URMrr 2/9 6/23/23, 2:34 AM Amenorrhea Pathway As per ES 2017 guidelines, consider performing a physical examination, administering a progestin challenge test, and obtaining abdominal or transvaginal ultrasound and/or MRI, depending on the context and patient preferences, for the evaluation of M llerian tract anomalies in patients with primary amenorrhea. C Evaluation of primary ovarian insufficiency (karyotyping and genetic testing): obtain chromosomal analysis in all patients with non-iatrogenic premature ovarian insufficiency. B Show 2 more Evaluation of primary ovarian insufficiency (adrenocortical and thyroid antibodies): Obtain screening for 21-hydroxylase autoantibodies, or alternatively adrenocortical antibodies, in patients with premature ovarian insufficiency of unknown cause or if an immune disorder is suspected. B Refer patients with premature ovarian insufficiency with positive 21-hydroxylase autoantibodies/adrenocortical antibodies to an endocrinologist for testing of adrenal function and to rule out Addison's disease. Obtain screening for anti-TPO antibodies in patients with premature ovarian insufficiency of unknown cause or if an immune disorder is suspected. B Measure TSH annually in patients with positive thyroid antibodies. Evaluation of primary ovarian insufficiency (cardiac evaluation): assess cardiovascular risk in patients diagnosed with premature ovarian insufficiency. Monitor at least BP, weight, and smoking status annually and assess other risk factors if indicated. B Show 2 more Evaluation of primary ovarian insufficiency (bone mineral testing): recognize that premature ovarian insufficiency is associated with reduced bone mineral density, B and reduced bone mineral density is very likely to indicate that premature ovarian insufficiency is associated with an increased risk of fracture later in life. B Show 3 more Evaluation of polycystic ovary syndrome, cardiac evaluation: As per ES 2020 guidelines, obtain a fasting lipid panel at diagnosis to assess cardiovascular risk in patients with PCOS. B As per ACOG 2018 guidelines, assess cardiovascular risks by determination of BMI, fasting lipid and lipoprotein levels, and metabolic syndrome risk factors in patients with PCOS. B As per RCOG 2014 guidelines: Recognize that conventional cardiovascular risk calculators are not validated for patients with PCOS. B Assess CVD risk in all patients with PCOS at the time of initial diagnosis by individual CVD risk factors (obesity, lack of physical activity, cigarette smoking, family history of T2DM, dyslipidemia, hypertension, impaired glucose tolerance, T2DM). B As per ES 2013 guidelines, screen for CVD risk factors, including a family history of early CVD, cigarette smoking, impaired glucose tolerance/T2DM, hypertension, dyslipidemia, obstructive sleep apnea, and obesity, especially increased abdominal adiposity, in adolescent and adult https://web.pathway.md/diseases/recpFmHfW5s3URMrr 3/9 6/23/23, 2:34 AM Amenorrhea Pathway patients with PCOS. B Assess for increased adiposity by BMI calculation and measurement of waist circumference in adolescent and adult patients with PCOS. B Evaluation of polycystic ovary syndrome, screening for diabetes: As per ACOG 2018 guidelines, screen for T2DM and impaired glucose tolerance with a fasting glucose level followed by a 2-hour glucose level after a 75-g glucose load in patients with PCOS. B As per RCOG 2014 guidelines: Obtain a 2-hour post 75 g oral glucose tolerance test in patients presenting with PCOS with overweight (BMI 25 kg/m ) or with normal weight (BMI < 25 kg/m ) but having additional risk factors, such as advanced age (> 40 years), personal history of gestational diabetes, or family history of T2DM. B Obtain oral glucose tolerance test annually in patients with impaired fasting glucose (fasting plasma glucose level 6.1-6.9 mmol/L) or impaired glucose tolerance (plasma glucose of 7.8 mmol/L but < 11.1 mmol/L after a 2-hour oral glucose tolerance test). B Evaluation of polycystic ovary syndrome, screening for endometrial cancer: As per RCOG 2014 guidelines, recognize that oligomenorrhea or amenorrhea may predispose to endometrial hyperplasia and later carcinoma in patients with PCOS. Consider initiating progesterone to induce withdrawal bleeding at least every 3-4 months. B Show 4 more As per ES 2013 guidelines, avoid obtaining routine ultrasound screening for endometrial thickness in patients with polycystic ovarian syndrome. D Evaluation of polycystic ovary syndrome, psychosocial assessment: As per RCOG 2014 guidelines, screen for depression and/or anxiety routinely in patients with PCOS. Obtain further assessment, provide appropriate counseling, and offer intervention by a qualified professional in patients with depression and/or anxiety. A As per ES 2013 guidelines, consider screening for depression and anxiety by history in adolescents with PCOS and, if identified, provide appropriate referral and/or treatment. C Bone mineral density testing: Consider obtaining a baseline bone mineral density measurement by DEXA in all adolescent and adult patients with 6 months of amenorrhea, and earlier in patients with a history or suspicion of severe nutritional deficiency, other energy deficit states, and/or skeletal fragility. C Consider obtaining clinical monitoring for hyperresponse in patients treated with exogenous gonadotropins for infertility. C 2. Medical management Management of functional hypothalamic amenorrhea (general principles): Consider educating patients about various menstrual patterns occurring during the recovery phase once the diagnosis of functional hypothalamic amenorrhea is established. Consider https://web.pathway.md/diseases/recpFmHfW5s3URMrr 4/9 6/23/23, 2:34 AM Amenorrhea Pathway informing patients that irregular menses do not require immediate evaluation and that menstrual irregularity does not preclude conception. C Evaluate patients with functional hypothalamic amenorrhea and severe bradycardia, hypotension, orthostasis, and/or electrolyte imbalance for inpatient treatment. B Management of functional hypothalamic amenorrhea, correction of energy imbalance: As per ES 2017 guidelines, offer increased caloric consumption (often weight gain is required), and/or improved nutrition, and/or decreased exercise activity to correct the energy imbalance in order to improve hypothalamic-pituitary-ovarian axis function in adolescent and adult patients with functional hypothalamic amenorrhea. B As per AAFP 2013 guidelines, offer nutritional rehabilitation and decreased exercise to restore weight as primary treatment in patients with functional hypothalamic amenorrhea, especially with the female athlete triad. B Management of functional hypothalamic amenorrhea, hormonal therapy: As per ES 2017 guidelines, consider offering short-term transdermal estradiol therapy with cyclic oral progestin (not oral contraceptives or ethinyl estradiol) in adolescent and adult patients with no return of menses after a reasonable trial of nutritional, psychological, and/or modified exercise intervention. C Show 2 more As per AAFP 2013 guidelines, do not use combined oral contraceptives for the sole purpose of improving bone density in patients with functional hypothalamic amenorrhea. D Management of functional hypothalamic amenorrhea (psychological support): consider offering psychological support, such as CBT, in adolescent and adult patients with functional hypothalamic amenorrhea. C Management of functional hypothalamic amenorrhea (management of infertility): Consider offering the following in patients with functional hypothalamic amenorrhea wishing to conceive, after a complete fertility workup: treatment with pulsatile GnRH as first-line therapy, followed by gonadotropin therapy and induction of ovulation when GnRH is not available C cautious use of gonadotropin therapy, C a trial of treatment with clomiphene citrate for ovulation induction if the patient has a sufficient endogenous estrogen level C do not use kisspeptin and leptin for the treatment of infertility C consider offering a trial of CBT to restore ovulatory cycles and fertility. C Consider inducing ovulation only in patients with functional hypothalamic amenorrhea having a BMI of at least 18.5 kg/m and only after attempts to normalize energy balance, due to the increased risk for fetal loss, small-for-gestational-age babies, preterm labor, and delivery by C- section for extremely low weight. C Management of functional hypothalamic amenorrhea (management of osteoporosis): Avoid using bisphosphonates, denosumab, testosterone, or leptin to improve bone mineral density in adolescents and adult patients with functional hypothalamic amenorrhea. D https://web.pathway.md/diseases/recpFmHfW5s3URMrr 5/9 6/23/23, 2:34 AM Amenorrhea Pathway Consider administering short-term teriparatide as an option for delayed fracture healing and very low bone mineral density in rare adult cases of functional hypothalamic amenorrhea. C Management of primary ovarian insufficiency: initiate systemic hormone therapy, if not contraindicated, to treat hypoestrogenism symptoms and mitigate long-term health risks in patients with primary ovarian insufficiency. E Show 3 more Management of hyperprolactinemia (medical therapy): initiate dopamine agonist therapy to lower prolactin levels, decrease tumor size, and restore gonadal function in patients with symptomatic prolactin-secreting microadenomas or macroadenomas. A Show 8 more Management of hyperprolactinemia (radiotherapy and surgery): Consider performing transsphenoidal surgery in symptomatic patients with prolactinomas not tolerating high doses of cabergoline or not responsive to dopamine agonist therapy. C Consider offering radiation therapy in patients with aggressive or malignant prolactinomas or when surgery fails. C Management of polycystic ovary syndrome, lifestyle modifications: As per ACOG 2018 guidelines: Advise exercising and dietary changes to reduce diabetes risk in patients with PCOS. A Advise weight loss to improve pregnancy rates, decrease hirsutism, and improve glucose tolerance and lipid levels in patients with PCOS. B As per SOGC 2018 guidelines: Consider advising weight loss through exercise and lifestyle modifications with a target of BMI < 35 kg/m and/or 5-10% of body weight if overweight for restoring ovulatory cycles and achieving pregnancy in patients with overweight and PCOS. B Refer patients with morbid obesity (BMI 40 kg/m ) to qualified providers for expert advice about safe weight loss strategies and pregnancy risk in this condition. B As per RCOG 2014 guidelines, advise lifestyle changes, including diet, exercise, and weight loss, preceding and/or accompanying pharmacological treatment, for improvement of long-term outcomes in patients with PCOS. B Management of polycystic ovary syndrome, hormonal contraceptives: As per ACOG 2018 guidelines, initiate combination low-dose hormonal contraceptives, frequently used for the long term, as primary treatment of menstrual disorders. B As per ES 2013 guidelines, initiate hormonal contraceptives (such as oral contraceptives, patches, or vaginal rings) as first-line therapy for menstrual abnormalities and hirsutism/acne in patients with polycystic ovarian syndrome. B Show 3 more Management of polycystic ovary syndrome, insulin sensitizers: As per ACOG 2018 guidelines, consider initiating insulin-sensitizing agents for improving insulin sensitivity to decrease circulating androgen levels, improve ovulation rate, and improve glucose https://web.pathway.md/diseases/recpFmHfW5s3URMrr 6/9 6/23/23, 2:34 AM Amenorrhea Pathway tolerance in patients with PCOS. B As per SOGC 2018 guidelines: Recognize that metformin combined with clomiphene citrate may increase ovulation and pregnancy rates but does not significantly improve the live birth rate over that of clomiphene citrate alone. A Consider adding metformin to clomiphene citrate in patients with clomiphene resistance aged > 28 years and having visceral obesity (waist-to-hip ratio > 0.85). B As per ASRM 2017 guidelines, consider initiating metformin to increase the ovulation rate in patients with polycystic ovarian syndrome B , recognizing that metformin alone is less effective than clomiphene citrate alone for the achievement of ovulation induction, clinical pregnancy, and live birth. C As per AAFP 2013 guidelines, consider offering metformin to improve abnormal menstruation in patients with PCOS. B As per ES 2013 guidelines, initiate metformin in patients with PCOS and T2DM or impaired glucose tolerance failed lifestyle modification. B Consider initiating metformin if the goal is to treat impaired glucose tolerance/metabolic syndrome. B Show 3 more Management of polycystic ovary syndrome, ovulation induction: As per ACOG 2018 guidelines, consider initiating letrozole as first-line therapy for ovulation induction because of the increased live birth rate compared with clomiphene citrate in patients with PCOS. B Show 2 more As per SOGC 2018 guidelines, consider initiating clomiphene citrate where available as first-line medical therapy for ovulation induction in patients with PCOS. Inform patients regarding the increased risk of twin pregnancy or higher order multiples with ovulation induction using clomiphene citrate. B Show 2 more As per ASRM 2017 guidelines, consider initiating letrozole as first-line therapy for ovulation induction in patients with polcystic ovary syndrome. C As per ES 2013 guidelines, initiate clomiphene citrate (or comparable estrogen modulators such as letrozole) as first-line therapy for anovulatory infertility in patients with PCOS. B Management of polycystic ovary syndrome, statin therapy: As per ES 2020 guidelines, avoid using lipid-lowering therapies for the treatment of hyperandrogenism or infertility in patients with PCOS. D As per RCOG 2014 guidelines, do not initiate routine lipid-lowering treatment in patients with PCOS. Prescribe only by a specialist. D As per ES 2013 guidelines: Avoid using statins for the treatment of hyperandrogenism and anovulation in patients with PCOS. D https://web.pathway.md/diseases/recpFmHfW5s3URMrr 7/9 6/23/23, 2:34 AM Amenorrhea Pathway Consider initiating statins in patients with PCOS meeting current indications for statin therapy. C 3. Specific circumstances Female athletes (evaluation): screen for female athlete triad at the preparticipation or annual health screening examination. Assess athletes with one component of the triad for the others. B Show 2 more Female athletes, management: As per AAFP 2013 guidelines, offer nutritional rehabilitation and decreased exercise to restore weight as primary treatment in patients with the female athlete triad. B As per ACSM 2007 guidelines, include a physician (or another healthcare professional), a registered dietitian, and, for athletes with disordered eating or an eating disorder, a mental health practitioner for multidisciplinary treatment of the female athlete triad. B Show 4 more References 1. Catherine M Gordon, Kathryn E Ackerman, Sarah L Berga et al. Functional Hypothalamic Amenorrhea: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2017 May 1;102 5 1413 1439. Open 2. No authors listed. Committee Opinion No. 698 Hormone Therapy in Primary Ovarian Insufficiency. Obstet Gynecol. 2017 May;129 5):e134-e141. Open 3. Practice Committee of the American Society for Reproductive Medicine. Role of metformin for ovulation induction in infertile patients with polycystic ovary syndrome PCOS a guideline. Fertil Steril. 2017 Sep;108 3 426 441. Open 4. European Society for Human Reproduction and Embryology ESHRE Guideline Group on POI, Webber L, Davies M et al. ESHRE Guideline: management of women with premature ovarian insufficiency. Hum Reprod. 2016 May;31 5 926 37. Open 5. Royal College of Obstetricians and Gynaecologists. Long-term Consequences of Polycystic Ovary Syndrome. Green-top Guideline No. 33. RCOG. 2014 Nov. Open 6. David A Klein, Merrily A Poth. Amenorrhea: an approach to diagnosis and management. Am Fam Physician. 2013 Jun 1;87 11 781 8. Open 7. Melmed S, Casanueva FF, Hoffman AR et al. Diagnosis and treatment of hyperprolactinemia: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011 Feb;96 2 273 88. Open 8. American College of Obstetricians and Gynecologists' Committee on Practice Bulletins Gynecology. ACOG Practice Bulletin No. 194 Polycystic Ovary Syndrome. Obstet Gynecol. 2018 Jun;131 6):e157-e171. Open 9. Martin KA, Anderson RR, Chang RJ et al. Evaluation and Treatment of Hirsutism in Premenopausal Women: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2018 Apr 1;103 4 1233 https://web.pathway.md/diseases/recpFmHfW5s3URMrr 8/9 6/23/23, 2:34 AM Amenorrhea Pathway 1257. Open 10. Legro RS, Arslanian SA, Ehrmann DA et al. Diagnosis and treatment of polycystic ovary syndrome: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2013 Dec;98 12 4565 92. Open 11. Committee on Adolescent Health Care. ACOG Committee Opinion No. 728 M llerian Agenesis: Diagnosis, Management, And Treatment. Obstet Gynecol. 2018 Jan;131 1):e35-e42. Open 12. Connie B Newman, Michael J Blaha, Jeffrey B Boord et al. Lipid Management in Patients with Endocrine Disorders: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2020 Dec 1;105 12):dgaa674. Open 13. Aurelia Nattiv, Anne B Loucks, Melinda M Manore et al. American College of Sports Medicine position stand. The female athlete triad. Med Sci Sports Exerc. 2007 Oct;39 10 1867 82. Open 14. David S Smithson, Tannys D R Vause, Anthony P Cheung. No. 362 Ovulation Induction in Polycystic Ovary Syndrome. J Obstet Gynaecol Can. 2018 Jul;40 7 978 987. Open 15. American Society for Reproductive Medicine. Choosing Wisely ASRM recommendations. Choosing Wisely. 2015. Open https://web.pathway.md/diseases/recpFmHfW5s3URMrr 9/9
Guideline sources The following summarized guidelines for the evaluation and management of ampullary cancer are prepared by our editorial team based on guidelines from the European Society of Gastrointestinal Endoscopy (ESGE 2021) and the American Society for Gastrointestinal Endoscopy (ASGE 2021; 2013). 1 2 3 Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s Guidelines 1. Classification and risk stratification Staging: Obtain EUS and abdominal MRCP for the staging of ampullary tumors. B Consider using the latest TNM classification for the staging of ampullary tumors. C https://web.pathway.md/diseases/rec3CEGnDJZsVsVtK 1/4 6/23/23, 2:34 AM Ampullary cancer Pathway 2. Diagnostic procedures Upper gastrointestinal endoscopy: consider using the cap-assisted method when the papilla is not seen during forward-viewing endoscopy. C Show 2 more Endoscopic ultrasound: obtain EUS in patients with suspected ampullary adenocarcinoma if the EUS findings or positive FNA results would change management. B Intraductal ultrasound: consider obtaining intraductal ultrasound in selected patients with ampullary tumors; however, balance the routine use against training, costs, and risk of pancreatitis. C Biopsy and histopathology: Perform endoscopic biopsies for histological confirmation in the case of low-grade dysplasia adenoma before initiating any treatment. B Avoid obtaining immunohistochemistry, K-ras and p53 evaluation, PCR, and MSI testing of ampullary tumor biopsies to inform prognosis and/or potential response to treatment. D Evaluation after initial negative histopathology: Obtain follow-up with EUS, side-viewing endoscopy, and further sampling with repeated biopsies for an enlarged papilla without clinical or biochemical signs in the case of initial negative histopathology. B Consider obtaining further investigations, at first including endoscopic US-guided FNA/biopsy and then limited sphincterotomy with repeated biopsies, in patients with suspected obstructive ampullary tumor with initial negative histopathology. B 3. Therapeutic procedures Endoscopic biliary stenting: perform retrograde cholangiopancreatography with self-expandable metal stent insertion in patients with ampullary tumors and biliary obstruction in palliative settings. A Show 2 more Ablative techniques: consider using complementary techniques (thermal ablation by cystotome, or radiofrequency ablation) with temporary biliary stenting in patients with ampullary adenoma with 20 mm intraductal extension, and performed in expert centers. C 4. Surgical interventions Endoscopic papillectomy (indications): do not perform diagnostic/therapeutic papillectomy when adenoma has not been proven. D Show 4 more https://web.pathway.md/diseases/rec3CEGnDJZsVsVtK 2/4 6/23/23, 2:34 AM Ampullary cancer Pathway Endoscopic papillectomy (technical considerations): individualize the decision for prophylactic endoscopic hemostasis and the type of technique. B Show 5 more Endoscopic papillectomy (prevention and management of postprocedural complications): perform direct snare resection without submucosal injection for endoscopic papillectomy. B Show 4 more Surgical papillectomy: consider offering surgical treatment of ampullary adenomas when endoscopic resection is not feasible for technical reasons (such as diverticulum, size > 4 cm), and in the case of intraductal involvement (of > 20 mm). C Pancreaticoduodenectomy: perform pancreaticoduodenectomy (including lymphadenectomy) for malignant lesions of the ampulla of stage T1 or higher, including cases where pathology results following an endoscopic papillectomy or surgical transduodenal ampullectomy reveal ampullary T1 adenocarcinoma. B 5. Specific circumstances Patients with gastric outlet obstruction: consider placing self-expandable metallic stents or performing surgical gastrojejunostomy in patients with incurable malignant gastric outlet obstruction undergoing palliative intervention. Decide between these approaches based on patient characteristics and preferences, multidisciplinary input, and local expertise. C Show 2 more 6. Follow-up and surveillance Postoperative surveillance: obtain long-term monitoring of patients after endoscopic papillectomy or surgical ampullectomy, based on duodenoscopy with biopsies of the scar and of any abnormal area, within the first 3 months, at 6 and 12 months, and thereafter yearly for at least 5 years. B Management of recurrent disease: Obtain a careful assessment of local tumoral extent using endoscopic examination and biopsies, and EUS and MRCP before any treatment in patients with recurrence after endoscopic papillectomy. B Consider offering endoscopic treatment including ablative techniques and endoscopic mucosal resection in patients with benign residual or recurrent lesions. C References 1. Geoffroy Vanbiervliet, Marin Strijker, Marianna Arvanitakis et al. Endoscopic management of ampullary tumors: European Society of Gastrointestinal Endoscopy ESGE Guideline. Endoscopy. 2021 Apr;53 4 429 448. Open https://web.pathway.md/diseases/rec3CEGnDJZsVsVtK 3/4 6/23/23, 2:34 AM Ampullary cancer Pathway 2. Anderson MA, Appalaneni V, Ben-Menachem T et al. The role of endoscopy in the evaluation and treatment of patients with biliary neoplasia. Gastrointest Endosc. 2013 Feb;77 2 167 74. Open 3. ASGE Standards of Practice Committee, Terry L Jue, Andrew C Storm et al. ASGE guideline on the role of endoscopy in the management of benign and malignant gastroduodenal obstruction. Gastrointest Endosc. 2021 Feb;93 2 309 322.e4. Open https://web.pathway.md/diseases/rec3CEGnDJZsVsVtK 4/4
Guideline sources The following summarized guidelines for the evaluation and management of amyotrophic lateral sclerosis are prepared by our editorial team based on guidelines from the American College of Chest Physicians (ACCP 2023), the Infectious Diseases Society of America (IDSA/ACR/AAN 2021), the ALS Society of Canada (ALSSC 2020), the European Federation of Neurological Societies (EFNS 2012), and the American Academy of Neurology (AAN 2009). 1 2 3 4 5 6 7 7 8 9 9 9 Definition ALS is a progressive neurological disorder characterized by degeneration of upper motor and lower motor neurons. 7 Epidemiology ALS is familial (10%) and sporadic (90%) in origin. The pathological hallmark of ALS is the presence of inclusion bodies (aggregation of cytoplasmic proteins) in motor neurons. 9 9 Pathophysiology In the United States, the estimated prevalence of ALS is 5.2 persons per 100,000 persons. 8 https://web.pathway.md/diseases/recoWND9TN3ZBmenp 1/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway Disease course Spinal motor neuron degeneration results in muscular paralysis, including respiratory muscle paralysis. Motor neuron degeneration can also occur in the frontal cortex, causing frontotemporal degeneration and dementia. 9 Prognosis and risk of recurrence The overall survival of patients with ALS is approximately 3 years after the time of diagnosis. 7 Calculator Calculator Revised Amyotrophic Lateral Scl Revised El Escorial diagnostic cr Guidelines 1. Screening and diagnosis Diagnosis: Confirm the diagnosis of ALS by a neurologist or physical medicine and rehabilitation specialist with training and expertise in ALS. E Refer patients with an initial diagnosis of ALS to an ALS specialty clinic for confirmation of diagnosis within 4 weeks. E Differential diagnosis: As per IDSA 2021 guidelines, do not test for Lyme disease routinely in patients with typical ALS. D As per EFNS 2012 guidelines: Refer patients with suspected ALS to an experienced neurologist. E Review the diagnosis of ALS if there is no evidence of typical progression or the patient develops atypical features. E Communication of diagnosis: As per ALSSC 2020 guidelines, tailor the approach of communicating the diagnosis to the patient's individual needs. E As per EFNS 2012 guidelines, communicate the diagnosis to patients by a consultant with a good knowledge of the patient. E Show 8 more 2. Diagnostic investigations https://web.pathway.md/diseases/recoWND9TN3ZBmenp 2/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway History and physical examination: obtain prompt detailed clinical and paraclinical evaluations in all patients with suspected ALS. Consider obtaining additional investigations in selected patients, and repeating investigations if initial tests are equivocal. E Evaluation of respiratory function: As per ACCP 2023 guidelines, obtain pulmonary function testing to assist with management decisions in patients with neuromuscular disease at risk for respiratory complications. E Show 3 more As per ALSSC 2020 guidelines, obtain regular respiratory monitoring at baseline and every 3 months, or as clinically indicated, in patients with ALS, including: review of symptoms including dyspnea, orthopnea, and morning headaches B measurement of sitting FVC or slow vital capacity B sniff nasal inspiratory pressure, supine FVC, and/or maximal inspiratory pressure B arterial blood gases, venous blood gas, or transcutaneous CO when hypercapnia is suspected or when bulbar impairment precludes accurate testing B peak cough flow measurement to assess cough effectiveness B nocturnal oximetry or overnight polysomnography when symptomatic sleep-disordered breathing is suspected and other daytime indications for noninvasive ventilation initiation are not present. B As per EFNS 2012 guidelines, assess symptoms or signs of respiratory insufficiency, including symptoms of nocturnal hypoventilation, at each visit. E Show 3 more As per AAN 2009 guidelines, consider obtaining nocturnal oximetry to detect hypoventilation regardless of the FVC. C Show 2 more Cognitive evaluation: As per ALSSC 2020 guidelines: Screen for cognitive and behavioral impairment in patients with ALS early in their disease. B Obtain specific assessments with the patient and their family members or caregivers, as appropriate, if there is concern about cognition or behavior at any point. E As per EFNS 2012 guidelines, recognize that frontotemporal syndrome occurs in up to half of patients with ALS B and is associated with a poorer prognosis. Recognize that symptoms of cognitive dysfunction may appear before or after the onset of motor symptoms. Show 6 more As per AAN 2009 guidelines, consider screening for cognitive and behavioral impairment in patients with ALS, as many patients with ALS demonstrate cognitive impairment and may meet the criteria for dementia. C As per AAN 2009 guidelines: Consider screening for cognitive and behavioral impairment in patients with ALS. C https://web.pathway.md/diseases/recoWND9TN3ZBmenp 3/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway Consider obtaining screening tests of executive function to detect cognitive impairment in patients with ALS before confirmation with formal neuropsychological evaluation. C Genetic testing: obtain genetic testing only in patients with a known family history of ALS and in sporadic ALS cases with the characteristic phenotype of the recessive D90A mutation. Do not obtain genetic testing in patients with sporadic ALS with a typical classical ALS phenotype. E Show 3 more Screening for venous thrombosis: recognize that there is an increased risk of VTE in patients with ALS and the risk appears heightened in cases with leg onset and in patients with poor mobility. E Show 2 more 3. Respiratory support Supplemental oxygen: As per ALSSC 2020 guidelines, do not administer oxygen routinely for chronic respiratory insufficiency. Assess the etiology of the hypoxia and consider administering supplemental oxygen if hypoxemia remains after optimal noninvasive ventilation pressure is provided. D As per EFNS 2012 guidelines, avoid using oxygen monotherapy because of the risk of exacerbating CO retention and oral dryness. Administer oxygen only in the presence of symptomatic hypoxia. D Noninvasive ventilation: As per ACCP 2023 guidelines, initiate noninvasive ventilation in patients with chronic respiratory failure. B Show 4 more As per ALSSC 2020 guidelines, intitiate noninvasive ventilation as the standard of care for the treatment of respiratory insufficiency in patients with ALS, both to lengthen survival and treat symptoms. B Show 9 more As per EFNS 2012 guidelines: Prefer noninvasive positive pressure ventilation over invasive mechanical ventilation in patients with symptoms or signs of respiratory insufficiency. E Recognize that noninvasive positive pressure ventilation can prolong survival for many months A and improve quality of life. B Consider providing active management of secretions and cough-assist devices to increase the effectiveness of assisted ventilation in patients with ALS. E As per AAN 2009 guidelines, consider initiating noninvasive ventilation for the treatment of respiratory insufficiency in ALS, both to lengthen survival and to slow the rate of FVC decline. C Invasive ventilation: https://web.pathway.md/diseases/recoWND9TN3ZBmenp 4/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway As per ACCP 2023 guidelines, consider offering invasive home mechanical ventilation via tracheostomy as an alternative to noninvasive ventilation in patients failing or intolerant of noninvasive ventilation (including extended daytime noninvasive ventilation use), worsening bulbar function, frequent aspiration, insufficient cough, episodes of chest infection despite adequate secretion management, or declining lung function. C As per ALSSC 2020 guidelines: Consider initiating invasive ventilation as an option in carefully selected patients when respiratory impairment cannot be effectively managed by noninvasive ventilation. E Consider performing a tracheostomy for upper airway obstruction with vocal cord paresis. Consider initiating long-term invasive ventilation. E As per EFNS 2012 guidelines, ensure an early discussion of end-of-life issues, coordination with palliative care teams, and appropriate advance directives to avoid unplanned (emergency) invasive mechanical ventilation. Initiate invasive mechanical ventilation only after informed discussion. E Weaning from ventilatory support: withdraw continuous ventilatory support only after consultation and planning with a healthcare professional with expertise in ventilation withdrawal and palliative sedation. E Show 2 more 4. Medical management General principles: As per ALSSC 2020 guidelines, refer patients with ALS to specialized ALS multidisciplinary clinics for optimized healthcare delivery. B Show 5 more As per EFNS 2012 guidelines, provide multidisciplinary care in all patients with ALS. Recognize that attendance at multidisciplinary clinics may extend survival, decrease medical complications, B and improve quality of life. B Show 3 more As per AAN 2009 guidelines, consider referring patients with ALS to multidisciplinary clinics to optimize healthcare delivery and prolong survival. C Riluzole: As per ALSSC 2020 guidelines, offer riluzole for improving survival in patients with ALS. A Initiate riluzole therapy soon after the diagnosis of ALS. Obtain regular monitoring of potential adverse effects. E As per EFNS 2012 guidelines, offer riluzole 50 mg BID in patients with ALS. A Show 3 more As per AAN 2009 guidelines, offer riluzole to slow disease progression in patients with ALS. A https://web.pathway.md/diseases/recoWND9TN3ZBmenp 5/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway Edaravone: consider offering IV edaravone to slow the decline in the ALS Functional Rating Scale-Revised score in selected patients with ALS. C Insufficient evidence regarding the benefit of IV edaravone at other stages of ALS. Take into careful consideration and discuss individualized goals, risks, and benefits of IV edaravone before administration. E Management of sialorrhea, pharmacotherapy: As per ACCP 2023 guidelines, consider administering a therapeutic trial of an anticholinergic medication as first-line therapy, with continued use only if there are perceived benefits compared to side effects, for the management of sialorrhea in patients with neuromuscular diseases. C As per ALSSC 2020 guidelines, offer anticholinergic agents as first-line therapy for sialorrhea. Tailor individual medication choices to patient factors. Consider switching to another anticholinergic medication if one anticholinergic medication is ineffective. E As per EFNS 2012 guidelines, offer amitriptyline, oral or transdermal hyoscine, or sublingual atropine drops for the management of sialorrhea in patients with ALS. E Management of sialorrhea (oral suction): consider offering oral suction as an adjunct therapy. E Management of sialorrhea, botulinum toxin injection: As per ACCP 2023 guidelines, consider offering botulinum toxin therapy to salivary glands in patients with sialorrhea having an inadequate response or intolerant of the side effects of anticholinergic therapy. C As per ALSSC 2020 guidelines, consider offering botulinum toxin injections as second-line therapy B and after feeding tube insertion because of the theoretical risk of worsening swallowing or airway integrity. E As per EFNS 2012 guidelines, consider offering botulinum toxin injections into the parotid and/or submandibular gland in patients with refractory sialorrhea. C As per AAN 2009 guidelines, consider offering botulinum toxin B injection for the treatment of refractory sialorrhea. C Management of sialorrhea, radiotherapy: As per ACCP 2023 guidelines, consider offering salivary gland radiotherapy in patients with sialorrhea having an inadequate response or intolerant of the side effects of anticholinergic therapy. C As per ALSSC 2020 guidelines, consider offering focal salivary gland radiation as a second- or third-line therapy option in patients with sialorrhea. C As per EFNS 2012 guidelines, consider offering irradiation of the salivary glands when pharmacological treatment fails. E As per AAN 2009 guidelines, consider offering low-dose radiotherapy to the salivary glands for the treatment of refractory sialorrhea. C Management of sialorrhea (surgery): do not offer surgical interventions for the management of sialorrhea. D https://web.pathway.md/diseases/recoWND9TN3ZBmenp 6/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway Management of dyspnea: Relieve anxiety and offer lorazepam (0.5-2.5 mg sublingually) for the management of short dyspneic bouts. Offer morphine (2.5 mg PO or SC) for longer phases of dyspnoea (> 30 minutes). E Offer morphine (2.5 mg PO 4-6 times daily) for the management of chronic dyspnea. Administer morphine SC or as IV infusion (started at 0.5 mg/hour, and titrated afterward) in patients with severe dyspnea. Add midazolam (2.5-5 mg) or diazepam if needed for nocturnal symptom control and to relieve anxiety. E Management of excessive bronchial secretions, manually assisted cough: As per ACCP 2023 guidelines, consider offering manually assisted cough techniques, independently or adding to other modalities (such as lung volume recruitment), in patients with reduced cough effectiveness. C As per ALSSC 2020 guidelines, offer manual-assisted coughing in patients reporting difficulty clearing airway secretions. E As per EFNS 2012 guidelines, teach patients and carers how to assist expiratory movements using a manual-assisted cough. E Management of excessive bronchial secretions, lung volume recruitment: As per ACCP 2023 guidelines: Consider offering glossopharyngeal breathing for lung volume recruitment and airway clearance in patients with hypoventilation. C Consider offering regular lung volume recruitment (breath stacking) using a handheld resuscitation bag or mouthpiece in patients with reduced lung function or cough effectiveness. C As per ALSSC 2020 guidelines, offer lung volume recruitment strategies in patients reporting difficulty clearing airway secretions. B Management of excessive bronchial secretions, mechanical insufflation-exsufflation: As per ACCP 2023 guidelines, consider adding regular mechanical insufflation-exsufflation (cough assist device) in patients with reduced cough effectiveness not adequately improved with alternative techniques. C As per ALSSC 2020 guidelines: Consider offering mechanical insufflation-exsufflation BID for secretion clearance in patients with reduced peak cough flow (< 270 L/min). Increase mechanical insufflation-exsufflation frequency during acute chest infections. E Provide in-home respiratory support of mechanical insufflation-exsufflation for education, titration, and troubleshooting. E As per EFNS 2012 guidelines, consider offering mechanical insufflator-exsufflator, particularly in the setting of an acute respiratory infection. E As per AAN 2009 guidelines, consider offering mechanical insufflation-exsufflation to clear secretions in patients with ALS having reduced peak cough flow, particularly during an acute https://web.pathway.md/diseases/recoWND9TN3ZBmenp 7/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway chest infection. C Management of excessive bronchial secretions, high-frequency chest wall oscillation: As per ACCP 2023 guidelines, consider offering high-frequency chest wall oscillation for secretion mobilization in patients with difficulties with secretion clearance. Consider combining high-frequency chest wall oscillation with airway clearance therapies, such as cough assistance or lung volume recruitment. C As per AAN 2009 guidelines, insufficient evidence to assess the risks and benefits of high- frequency chest wall oscillation for clearing airway secretions in patients with ALS. I Management of excessive bronchial secretions (home suction device and room humidifier): consider advising using a portable home suction device and a room humidifier. E Management of excessive bronchial secretions, pharmacotherapy: As per ALSSC 2020 guidelines, consider offering mucolytics (guaifenesin or N-acetylcysteine), -receptor antagonists (metoprolol or propranolol), nebulized saline, or nebulized ipratropium in patients with difficulties clearing airway secretions. E As per EFNS 2012 guidelines: Consider offering mucolytic agents (such as N-acetylcysteine 200-400 mg TID) for the management of excessive bronchial secretions in patients with ALS. E Consider offering combinations of -blockers and a nebulizer with saline and/or anticholinergic bronchodilators and/or mucolytics and/or furosemide. Offer mucolytics only if sufficient cough flow is present. E Management of pseudobulbar affect: As per ALSSC 2020 guidelines, counsel patients and family members that pseudobulbar affect is a symptom of ALS and does not necessarily represent a symptom of depression or impaired cognition. E Show 3 more As per EFNS 2012 guidelines, counsel patients and relatives that emotional lability is not a sign of an additional mood disorder but is because of the effects of ALS on the brain. E Show 2 more As per AAN 2009 guidelines, consider offering dextromethorphan plus quinidine for the treatment of pseudobulbar affect. C Management of fasciculations: do not offer medication management for fasciculations in most patients with ALS. D Consider offering gabapentin if fasciculations cause substantial distress. D Management of spasticity: As per ALSSC 2020 guidelines, consider offering stretching for the management of muscle spasticity. C Show 2 more As per EFNS 2012 guidelines: https://web.pathway.md/diseases/recoWND9TN3ZBmenp 8/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway Consider offering regular physical therapy and antispastic drugs (such as baclofen and tizanidine) to relieve significant spasticity in patients with ALS. Consider offering hydrotherapy with exercises in warm pools (32-34 C) and cryotherapy. E Consider administering intrathecal baclofen in patients with severe spasticity despite oral medications. E Management of muscle cramps: As per ALSSC 2020 guidelines: Differentiate muscle cramps from other causes of pain. E Consider offering tonic water, gabapentin, and baclofen as first-line therapy for muscle cramps. Consider offering quinine, levetiracetam, and mexiletine as second-line treatment options. E As per EFNS 2012 guidelines: Consider offering levetiracetam for the management of muscle cramps in patients with ALS. Consider offering quinine sulfate (200 mg BID) if levetiracetam is unsuccessful or in case of side effects. E Consider offering physiotherapy, physical exercise, and/or hydrotherapy in the management of muscle cramps in patients with ALS. E Management of dysarthria: obtain regular monitoring in patients with dysarthria by a speech- language pathologist to ensure timely communication interventions. E Show 3 more Management of depression and anxiety: As per ALSSC 2020 guidelines: Treat depression in patients with ALS as it has a substantial impact on patient wellbeing. Consider offering SSRIs or SNRIs. Consider offering nonpharmacologic supportive measures, such as psychology, social work, psychiatry, or spiritual care. E Treat anxiety in patients with ALS as it has a substantial impact on patient wellbeing. Determine whether anxiety is related to respiratory insufficiency and treat it appropriately. Offer SSRIs in patients with concurrent depression. Recognize that benzodiazepines can exacerbate respiratory insufficiency. Consider offering nonpharmacologic supportive measures, such as psychology, social work, psychiatry, or spiritual care. E As per EFNS 2012 guidelines: Offer appropriate antidepressants (such as amitriptyline, SSRIs, or mirtazapine) for the management of depression in patients with ALS. Consider preferring SSRIs in elderly or cognitively impaired patients. E Offer bupropion or benzodiazepines (such as diazepam or lorazepam) for the management of anxiety in patients with ALS. E Management of insomnia and fatigue: As per ALSSC 2020 guidelines, obtain appropriate evaluation for insomnia as it may have multiple causes, such as respiratory insufficiency and depression. Consider obtaining respiratory investigations and sleep studies to determine the type and cause of insomnia. E https://web.pathway.md/diseases/recoWND9TN3ZBmenp 9/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway Show 4 more As per EFNS 2012 guidelines: Offer amitriptyline, mirtazapine, or appropriate hypnotics (such as zolpidem) for the management of insomnia in patients with ALS. E Consider offering modafinil for the management of debilitating fatigue in patients with ALS. B As per AAN 2009 guidelines, consider withholding riluzole in patients experiencing fatigue while taking riluzole. C Management of cognitive impairment: insufficient evidence to recommend pharmacologic agents for the management of cognitive or behavioral impairment in patients with ALS. Show 3 more Management of of venous thrombosis: As per ALSSC 2020 guidelines: Initiate anticoagulation as per standard VTE guidelines for the management of VTE in patients with ALS. E Do not administer VTE prophylaxis in nonhospitalized patients with ALS. D As per EFNS 2012 guidelines: Initiate anticoagulant therapy for the treatment of DVT. E Manage risk factors for venous thrombosis. Offer physiotherapy, limb elevation, and compression stockings. E Insufficient evidence to support the prophylactic use of anticoagulants. Therapies with no evidence for benefit: As per EFNS 2012 guidelines, insufficient evidence to support the use of vitamins, testosterone, antioxidants (such as co-enzyme Q-10 and ginkgo biloba), IVIG, cyclosporin, interferons, glatiramer acetate, KDI tripeptide, neurotrophic factors (including brain-derived neurotrophic factor, IGF-1, and mecasermin rinfabate), ceftriaxone, creatine, gabapentin, minocycline, stem cells, or lithium. I As per AAN 2009 guidelines, do not use high-dose vitamin E in the treatment of patients with ALS. D Insufficient evidence to support the use of low-dose vitamin E for the treatment of ALS D Show 2 more Palliative care: As per ALSSC 2020 guidelines, consider providing palliative care in patients with ALS throughout the disease course by ALS clinic staff, palliative care practitioners, and family physicians. E Show 9 more As per EFNS 2012 guidelines, offer input from a palliative care team early in the course of the disease whenever possible. Show 3 more End-of-life care: https://web.pathway.md/diseases/recoWND9TN3ZBmenp 10/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway As per ALSSC 2020 guidelines, refer patients to a physician or nurse practitioner for discussions about medical assistance in dying, abiding by regional guidelines. E Show 3 more As per EFNS 2012 guidelines, initiate discussions on end-of-life decisions when the patient asks or provides an opportunity for discussion on the provision of end of life information and/or interventions. Show 5 more 5. Nonpharmacologic interventions Exercise: consider advising regular moderate-intensity exercise for improved function and quality of life in the early phases of ALS. C Consider offering a personalized exercise program, including strength with submaximal effort for resistance and aerobic training. E Show 2 more Nutritional support: As per ALSSC 2020 guidelines, monitor nutritional status by weight and BMI every 3 months, or as clinically indicated. Consider assessing total daily energy expenditure. B Show 10 more As per EFNS 2012 guidelines, assess bulbar dysfunction and nutritional status (including body weight) at each visit. Recognize that difficulty drinking tap water is frequently the first sign of significant dysphagia. E Show 7 more As per AAN 2009 guidelines, consider performing placement of a percutaneous endoscopic gastrostomy in order to provide enteral nutrition and stabilize body weight in patients with ALS with impaired oral food intake. C 6. Patient education General counseling: discuss treatment options and research opportunities in patients with ALS. E Show 4 more 7. Follow-up and surveillance Indications for referral: refer patients with suspected ALS to an experienced neurologist. E Show 2 more Follow-up: As per ALSSC 2020 guidelines, review medications regularly and consider discontinuing any nonessential medications. Continue symptom management medications. Discontinue primary https://web.pathway.md/diseases/recoWND9TN3ZBmenp 11/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway prevention medications if the duration of effect is longer than the expected survival. Consider discontinuing statins based on the patient's expected survival and cardiovascular risks. E As per EFNS 2012 guidelines: Monitor patients every 2-3 months (more frequently in the months following diagnosis or in the later stages of the disease, and less frequently if the disease is progressing slowly). Maintain regular contact with the patient and relatives between visits. E Ensure to follow the patient by the same neurologist liaising closely with the patient's primary care physician. E References 1. Christen Shoesmith, Agessandro Abrahao, Tim Benstead et al. Canadian best practice recommendations for the management of amyotrophic lateral sclerosis. CMAJ. 2020 Nov 16;192 46 E1453 E1468. Open 2. EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis:, Andersen PM, Abrahams S et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis MALS) revised report of an EFNS task force. Eur J Neurol. 2012 Mar;19 3 360 75. Open 3. Paul M Lantos, Jeffrey Rumbaugh, Linda K Bockenstedt et al. Clinical Practice Guidelines by the Infectious Diseases Society of America IDSA , American Academy of Neurology AAN , and American College of Rheumatology ACR 2020 Guidelines for the Prevention, Diagnosis, and Treatment of Lyme Disease. Arthritis Care Res Hoboken). 2021 Jan;73 1 1 9. Open 4. Miller RG, Jackson CE, Kasarskis EJ et al. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009 Oct 13;73 15 1218 26. Open 5. Akram Khan, Lindsy Frazer-Green, Reshma Amin et al. Respiratory Management of Patients with Neuromuscular Weakness: An American College of Chest Physicians Clinical Practice Guideline and Expert Panel Report. Chest. 2023 Mar 13;S0012 3692 23 00353 7. Open 6. Miller RG, Jackson CE, Kasarskis EJ et al. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009 Oct 13;73 15 1227 33. Open 7. Salameh JS, Brown RH Jr, Berry JD. Amyotrophic Lateral Sclerosis: Review. Semin Neurol. 2015 Aug;35 4 469 76. Open 8. Mehta P, Kaye W, Raymond J et al. Prevalence of Amyotrophic Lateral Sclerosis United States, 2015. MMWR Morb Mortal Wkly Rep. 2018 Nov 23;67 46 1285 1289. Open 9. Brown RH, Al-Chalabi A. Amyotrophic Lateral Sclerosis. N Engl J Med. 2017 Jul 13;377 2 162 172. Open 10. J M Cedarbaum, N Stambler, E Malta et al. The ALSFRS R a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group Phase III . J Neurol Sci. 1999 Oct 31;169 1 2 13 21. Open https://web.pathway.md/diseases/recoWND9TN3ZBmenp 12/13 6/23/23, 2:35 AM Amyotrophic lateral sclerosis Pathway 11. B R Brooks, R G Miller, M Swash et al. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000 Dec;1 5 293 9. Open https://web.pathway.md/diseases/recoWND9TN3ZBmenp 13/13
Guideline sources The following summarized guidelines for the evaluation and management of anal cancer are prepared by our editorial team based on guidelines from the European Society of Medical Oncology (ESMO 2021), the American Society of Colon and Rectal Surgeons (ASCRS 2018), and the British HIV Association (BHIVA 2014). 1 2 3 Calculator Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s TNM class Guidelines https://web.pathway.md/diseases/recxPhkt7YVf0q1hh 1/6 6/23/23, 2:35 AM Anal cancer Pathway 1. Screening and diagnosis Indications for screening: Consider screening for anal squamous cancer in patients at high risk as part of a comprehensive screening program, using anal cytology or anal Pap test. C Consider screening for anal squamous cancer in patients at high risk, using high-resolution anoscopy, performed by clinicians with appropriate training in the procedure. C 2. Diagnostic investigations Clinical history and physical examination: As per ESMO 2021 guidelines: Perform digital anorectal examination for the detection of lesions in the anal area. A Perform clinical examination including DRE (and vaginal examination in female patients) and palpation of the inguinal lymph nodes for the assessment of tumor extenet. B As per ASCRS 2018 guidelines: Elicit history, perform physical examination and obtain laboratory testing to identify patients at increased risk for anal squamous neoplasms, such as human immunodeficiency virus-positive individuals, MSM, and females with a history of cervical dysplasia. B Elicit a disease-specific history and perform physical examination, emphasizing symptoms, risk factors, and signs of advanced disease. B Diagnostic imaging: As per ESMO 2021 guidelines, obtain high-resolution T2-weighted MRI for optimal assessment of primary tumor and lymph nodes. B Show 4 more As per ASCRS 2018 guidelines, obtain radiologic evaluation to determine tumor extension and assess for metastatic disease. B HPV testing: As per ESMO 2021 guidelines, consider obtaining HPV testing for the prediction of treatment response. C As per ASCRS 2018 guidelines, consider obtaining HPV testing as an adjunct to screening for anal cancer. C HIV testing: consider obtaining human immunodeficiency virus testing in at-risk patients C and in patients with recurrent or multifocal anal intraepithelial neoplasia. B Screening for other intraepithelial neoplasia: screen female patients with anal intraepithelial neoplasia for synchronous cervical, vulvar and vaginal intraepithelial neoplasia. A 3. Diagnostic procedures https://web.pathway.md/diseases/recxPhkt7YVf0q1hh 2/6 6/23/23, 2:35 AM Anal cancer Pathway Endoscopy: perform endoscopic evaluation to determine tumor extension and assess for metastatic disease. B Fine needle aspiration: consider performing US-guided FNA for further characterization of enlarged inguinal lymph nodes when confirmatory features of malignancy are not evident on either MRI or PET-CT. C Biopsy and pathology: As per ESMO 2021 guidelines, perform mandatory biopsy to confirm the diagnosis of SCC of the anus. A Show 2 more As per ASCRS 2018 guidelines: Report pathology specimens using a standardized nomenclature with a 2-tiered system. B Use biomarkers including p16 selectively to clarify equivocal high-grade lesions. B 4. Medical management General principles: As per ESMO 2021 guidelines, refer and discuss all patients with anal tumors in a multidisciplinary team meeting with a pre-specified interest in anal cancer. B As per ASCRS 2018 guidelines: Offer chemoradiotherapy as the primary treatment in all patients with squamous cell cancer of the anal canal, and in most patients with perianal squamous cell cancer. A Offer a combination of 5-fluorouracil and mitomycin C in conjunction with radiotherapy as first- line multimodal therapy in patients with SCC of the anus. A Management of in-situ lesions: Consider offering topical imiquimod, fluorouracil, trichloroacetic acid and cidofovir with close long-term follow-up for the treatment of patients with low-grade or high-grade squamous intraepithelial lesion. C Consider performing ablative therapy with conventional anoscopy or high-resolution anoscopy in patients with high-grade squamous intraepithelial lesion. C Management of local/locoregional disease, indications: As per ESMO 2021 guidelines, offer radiotherapy with concomitant 5-fluorouracil and mitomycin C as standard of care in patients with localized SCC of the anus. A Show 3 more As per ASCRS 2018 guidelines, offer multimodal therapy involving chemotherapy combined with radiotherapy rather than radiotherapy alone for better locoregional control. A Management of local/locoregional disease, radiotherapy techniques and doses: As per ESMO 2021 guidelines, administer chemoradiotherapy with a radiotherapy dose of > 50 Gy in patients with locally advanced anal cancer. Insufficient evidence to recommend optimal radiotherapy doses for different tumor stages. B https://web.pathway.md/diseases/recxPhkt7YVf0q1hh 3/6 6/23/23, 2:35 AM Anal cancer Pathway Show 3 more As per ASCRS 2018 guidelines, do not administer radiation doses of > 59 Gy. D Management of anal margin involvement: As per ESMO 2021 guidelines: Offer chemoradiotherapy in patients with anal margin cancer (T1N0M0) if the margin is 1 mm. B Consider performing local excision for definite treament of patients with early anal margin cancer (cT1N0M0) aiming to achieve histological clearance of > 1 mm without damaging the anal sphincter muscle. C As per ASCRS 2018 guidelines, perform wide-local excision (with 1 cm margins of resection) in patients with well-differentiated, node-negative, T1 perianal squamous cancers. B Show 2 more Management of advanced/metastatic disease: As per ESMO 2021 guidelines, consider offering carboplatin in combination with paclitaxel as standard of care in patients with chemotherpy-na ve advanced anal cancer. B Show 2 more As per ASCRS 2018 guidelines: Consider offering systemic chemotherapy in patients with distant metastatic disease. C Consider performing metastasectomy, radiation, and radiofrequency ablation in selected patients with distant metastatic disease. C 5. Therapeutic procedures Endoscopic ablation: consider performing ablative therapy with conventional anoscopy or high- resolution anoscopy in patients with high-grade squamous intraepithelial lesions. C Colostomy: consider performing pre-chemoradiotherapy colostomy in patients with locally advanced anal cancers with (or anticipated) anorectal pain or fecal incontinence and rectovaginal fistula. Counsel patients regarding the likelihood that their colostomy will be permanent. C 6. Surgical interventions Wide-local excision: As per ESMO 2021 guidelines, consider performing local excision for definite treament of patients with early anal margin cancer (cT1N0M0) aiming to achieve histological clearance of > 1 mm without damaging the anal sphincter muscle. C As per ASCRS 2018 guidelines, perform wide-local excision (with 1 cm margins of resection) in patients with well-differentiated, node-negative, T1 perianal squamous cancer. B Abdominoperineal resection: https://web.pathway.md/diseases/recxPhkt7YVf0q1hh 4/6 6/23/23, 2:35 AM Anal cancer Pathway As per ESMO 2021 guidelines, consider performing radical abdominoperineal resection instead of chemoradiotherapy as the primary treatment in uncommon scenarios, such as previous pelvic radiotherapy. C As per ASCRS 2018 guidelines, perform abdominoperineal resection as salvage therapy in patients with persistent or recurrent disease. B 7. Specific circumstances Patients with HIV/AIDS: As per ASCRS 2018 guidelines, treat patients with human immunodeficiency virus infection who present with anal cancer as the first manifestation of their immunosuppression according to the same regimens as immunocompetent patients, provided they are not medically deconditionned. B As per ESMO 2014 guidelines, offer chemoradiotherapy with 5-fluorouracil and mitomycin C in patients with anal cancer and human immunodeficiency virus infection. A Show 4 more 8. Patient education Counseling on treatment adherence: counsel patients on avoiding missed treatments, because they are strongly associated with poor disease control. B 9. Preventative measures HPV vaccination: Offer HPV immunization in < 26 years old males and females in order to prevent anal squamous cell cancer. B Do not offer immunization in individuals with anal dysplasia for the secondary prevention of dysplasia and cancer. D 10. Follow-up and surveillance Assessment of treatment response: assess tumor response after chemoradiotherapy in 26 weeks as an optimum time point. B Show 3 more Surveillance for toxicity: Assess patients for skin and hematological toxicity during chemoradiotherapy. B Inform patients about expected late effects, including changes in anorectal and sexual function, menopause and risk of infertility. B https://web.pathway.md/diseases/recxPhkt7YVf0q1hh 5/6 6/23/23, 2:35 AM Anal cancer Pathway Follow-up: As per ESMO 2021 guidelines, consider obtaining follow-up within a protocol-driven program by anal cancer multidisciplinary team in all patients with anal cancer. C Show 5 more As per ASCRS 2018 guidelines, start disease surveillance 8-12 weeks from the completion of chemoradiotherapy. B Show 3 more Patients with locally recurrent or residual disease: consider performing salvage surgery in patients with locally recurrent or residual anal cancer after chemoradiotherapy. C Show 6 more References 1. S Rao, M G Guren, K Khan et al. Anal cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2021 Jun 24;S0923 7534 21 02064 0. Open 2. Stewart DB, Gaertner WB, Glasgow SC et al. The American Society of Colon and Rectal Surgeons Clinical Practice Guidelines for Anal Squamous Cell Cancers Revised 2018 . Dis Colon Rectum. 2018 Jul;61 7 755 774. Open 3. Bower M, Palfreeman A, Alfa-Wali M et al. British HIV Association guidelines for HIV-associated malignancies 2014. HIV Med. 2014 Mar;15 Suppl 2 1 92. Open 4. Joel M Palefsky, Jeannette Y Lee, Naomi Jay et al. Treatment of Anal High-Grade Squamous Intraepithelial Lesions to Prevent Anal Cancer. N Engl J Med. 2022 Jun 16;386 24 2273 2282. Open https://web.pathway.md/diseases/recxPhkt7YVf0q1hh 6/6
Guideline sources The following summarized guidelines for the evaluation and management of anal fissure are prepared by our editorial team based on guidelines from the American Society of Colon and Rectal Surgeons (ASCRS 2023; 2017), the World Society of Emergency Surgery (WSES/AAST 2021), the American College of Gastroenterology (ACG 2021), and the Association of Coloproctology of Great Britain and Ireland (ACPGBI 2008). 1 2 3 4 5 6 6 6 7 7 8 Definition Anal fissures refer to an anorectal disorder characterized by a tear in the squamous epithelium of the anus, distal to the dentate line. Fissures are defined as acute if present for less than 6 weeks, and they are defined as chronic if present for more than 6 weeks. 6 7 Epidemiology Various risk factors increase the risk of anal fissures, mediating their effects via mechanical tissue trauma, ischemia of the anal canal, impaired sphincter structure or function, or anal hypertonicity. 6 Pathophysiology https://web.pathway.md/diseases/recq5R49DVcBid2l3 1/5 6/23/23, 2:35 AM Anal fissure Pathway The annual incidence of anal fissures in the United States is estimated at 110 cases per 100,000 person-years. 8 Disease course Anal fissures may cause clinical manifestations of anal pain, spasm, and/or bleeding with defecation. 6 Prognosis and risk of recurrence Medical treatment is associated with over 70% success rates. Surgical treatment, which is typically reserved for patients in whom medical therapy fails, yields excellent outcomes. 7 Guidelines 1. Screening and diagnosis Etiology: recognize that anal fissures associated with internal anal sphincter hypertonia are probably ischemic in nature. B Diagnosis: diagnose anal fissures based on medical history and physical examination findings. B Differential diagnosis: As per WSES 2021 guidelines, consider eliciting a focused medical history, performing a complete physical examination and obtaining laboratory tests based on the suspected associated illness, to rule out other causes in patients with atypical acute anal fissures. C As per ASCRS 2017 guidelines, recognize that less common causes of anal fissures include Crohn's disease, sexually transmitted diseases, and low-pressure fissures. B 2. Diagnostic investigations Diagnostic imaging: Insufficient evidence to recommend obtaining imaging investigations in patients with typical acute anal fissures. Consider obtaining endoscopy, CT, or endoanal ultrasound in patients with atypical acute anal fissures only in case of suspected concomitant IBD, anal or colorectal cancer, or occult perianal sepsis. C Laboratory tests: insufficient evidence to recommend obtaining biochemical investigations in patients with typical acute anal fissures. I 3. Medical management Indications for non-operative management: https://web.pathway.md/diseases/recq5R49DVcBid2l3 2/5 6/23/23, 2:35 AM Anal fissure Pathway As per ASCRS 2023 guidelines, offer nonoperative management as first-line therapy in patients with acute anal fissures. B As per WSES 2021 guidelines, offer nonoperative management as first-line therapy in patients with acute anal fissures. B As per ACPGBI 2008 guidelines, recognize that conservative management will heal a proportion of acute anal fissures. A Topical nitrates: As per ASCRS 2023 guidelines, consider offering topical nitrates in patients with anal fissures, although headache symptoms may limit their efficacy. B As per ACPGBI 2008 guidelines, offer topical glyceryl trinitrate in patients with anal fissures. A Topical calcium channel blockers: As per ASCRS 2023 guidelines, offer CCBs as first-line therapy in patients with chronic anal fissures. B As per ACG 2021 guidelines, offer local CCBs as first-line medical treatment in patients with chronic anal fissures. B As per ACPGBI 2008 guidelines, offer topical diltiazem as first-line therapy in patients with anal fissures. Inform patients about pruritis ani. A Topical antibiotics: consider offering topical antibiotics in patients with acute anal fissures in case of potentially reduced therapeutic compliance or poor genital hygiene. C Pain management: consider offering topical anesthetics and common painkillers in patients with acute anal fissures in case of inadequate pain control. C 4. Nonpharmacologic interventions Dietary modifications: advise dietary and lifestyle changes with increased fiber and water intake in patients with acute anal fissures. B 5. Therapeutic procedures Botulinum toxin injections: As per ASCRS 2023 guidelines: Administer botulinum toxin injection as first-line therapy in patients with chronic anal fissures and as second-line therapy following failed treatment with topical therapies. B Insufficient evidence to recommend repeat botulinum toxin injection in patients with recurrent anal fissures, although short-term outcomes have shown good healing rates with a low risk of fecal incontinence. I As per WSES 2021 guidelines, insufficient evidence to recommend botulinum injections in patients with acute anal fissures. I https://web.pathway.md/diseases/recq5R49DVcBid2l3 3/5 6/23/23, 2:35 AM Anal fissure Pathway As per ACG 2021 guidelines, consider performing botulinum toxin A injections in patients failed CCBs or as an alternative to CCBs. C As per ACPGBI 2008 guidelines, consider administering botulinum toxin injection in patients with anal fissures resistant to topical glyceryl trinitrate or diltiazem. B Anal dilatation: As per WSES 2021 guidelines: Do not perform manual dilatation in patients with acure anal fissures. D Insufficient evidence to recommend for or against controlled anal dilatation in patients with acute anal fissures. I As per ACPGBI 2008 guidelines, do not perform anal dilatation for the management of patients with anal fissures. D 6. Surgical interventions Indications for surgery: Avoid performing surgery in patients with acute anal fissures. D Consider performing surgery in patients with anal fissures in the chronic phase not responding after 8 weeks of nonoperative management. B Lateral internal sphincterotomy: As per ASCRS 2023 guidelines, consider performing lateral internal sphincterotomy in selected pharmacologically na ve patients with chronic anal fissures. B Show 4 more As per ACG 2021 guidelines, perform lateral internal sphincterotomy as the surgical treatment of choice in patients with chronic anal fissures failed to heal with nonoperative management. A As per ACPGBI 2008 guidelines, reserve lateral sphincterotomy for patients failed medical treatment. A Anocutaneous flap: As per ASCRS 2023 guidelines: Consider performing anocutaneous flap surgery as an alternative to lateral internal sphincterotomy in patients with chronic anal fissures with a decreased risk of fecal incontinence compared with lateral internal sphincterotomy and comparable healing rates. C Consider performing anocutaneous flap surgery in addition to botulinum toxin injections or lateral internal sphincterotomy to decrease postoperative pain and allow for primary wound healing. C As per ACPGBI 2008 guidelines, perform anal advancement flap surgery in patients with a low resting anal pressure A . Consider performing rotational or V-Y flap techniques. B 7. Specific circumstances https://web.pathway.md/diseases/recq5R49DVcBid2l3 4/5 6/23/23, 2:35 AM Anal fissure Pathway Pediatric patients: offer conservative management initially in pediatric patients with anal fissures. Offer local glyceryl trinitrate or CCBs if conservative management fails. Reserve lateral sphincterotomy or fissurectomy for cases failing to heal with medical treatment. B References 1. K L R Cross, E J D Massey, A L Fowler et al. The management of anal fissure: ACPGBI position statement. Colorectal Dis. 2008 Nov;10 Suppl 3 1 7. Open 2. Antonio Tarasconi, Gennaro Perrone, Justin Davies et al. Anorectal emergencies: WSES AAST guidelines. World J Emerg Surg. 2021 Sep 16;16 1 48. Open 3. Arnold Wald, Adil E Bharucha, Berkeley Limketkai et al. ACG Clinical Guidelines: Management of Benign Anorectal Disorders. Am J Gastroenterol. 2021 Oct 1;116 10 1987 2008. Open 4. Jennifer S Davids, Alexander T Hawkins, Anuradha R Bhama et al. The American Society of Colon and Rectal Surgeons Clinical Practice Guidelines for the Management of Anal Fissures. Dis Colon Rectum. 2023 Feb 1;66 2 190 199. Open 5. Stewart DB Sr, Gaertner W, Glasgow S et al. Clinical Practice Guideline for the Management of Anal Fissures. Dis Colon Rectum. 2017 Jan;60 1 7 14. Open 6. Zaghiyan KN, Fleshner P. Anal fissure. Clin Colon Rectal Surg. 2011 Mar;24 1 22 30. Open 7. Latif A, Ansar A, Butt MQ. Morbidity associated with treatment of chronic anal fissure. Pak J Med Sci. 2013 Sep;29 5 1230 5. Open 8. Mapel DW, Schum M, Von Worley A. The epidemiology and treatment of anal fissures in a population- based cohort. BMC Gastroenterol. 2014 Jul 16;14 129. Open https://web.pathway.md/diseases/recq5R49DVcBid2l3 5/5
Guideline sources The following summarized guidelines for the evaluation and management of anaphylaxis are prepared by our editorial team based on guidelines from the Wilderness Medical Society (WMS 2022), the European Academy of Allergy and Clinical Immunology (EAACI 2022; 2014), the American Academy of Allergy, Asthma & Immunology (AAAAI/ACAAI 2020; 2014), and the American Heart Association (AHA 2020). 1 2 3 4 5 6 Guidelines 1. Screening and diagnosis Diagnostic criteria: consider using clinical criteria, including rapid onset of multiple symptoms and signs, for identifying anaphylaxis in an acute context. C https://web.pathway.md/diseases/recZK0DO9cy9R1bWR 1/7 6/23/23, 2:35 AM Anaphylaxis Pathway 2. Diagnostic investigations Serum tryptase: consider measuring serum tryptase half to two hours after the start of the reaction and baseline tryptase at least 24 hours after complete resolution of symptoms to support the diagnosis of anaphylaxis. C 3. Respiratory support Supplemental oxygen: administer high-flow oxygen by face mask in all patients with anaphylaxis. B 4. Medical management General principles: As per AAAAI 2020 guidelines, consider incorporating severity of anaphylaxis presentation and/or the administration of > 1 dose of epinephrine for the treatment of initial anaphylaxis as a guide to determining patient's risk for developing biphasic anaphylaxis. C As per EAACI 2014 guidelines: Call for help promptly and assess the patient simultaneously. B Use an anaphylaxis management plan from the time of diagnosis to prevent future reactions, and aid recognition and treatment of any further reactions. B Epinephrine, clinician-delivered: As per EAACI 2022 guidelines, administer intramuscularly epinephrine in the mid-thigh area promptly as first-line management of anaphylaxis. B As per WMS 2022 guidelines, administer epinephrine as the essential and primary treatment once anaphylaxis has been diagnosed. A Show 5 more As per ACAAI/AAAAI 2020 guidelines, administer epinephrine as first-line therapy in patients with uniphasic and/or biphasic anaphylaxis. B Show 2 more As per EAACI 2014 guidelines, administer epinephrine promptly as first-line therapy for the emergency management of patients with anaphylaxis. B Show 5 more Epinephrine, patient-delivered: As per EAACI 2022 guidelines, advise self-administering of epinephrine autoinjectors for first- line management of anaphylaxis in the community. B Show 2 more As per WMS 2022 guidelines, take into consideration cost, operator training, and safety for both patient and first responder when choosing an epinephrine delivery device. B Show 4 more https://web.pathway.md/diseases/recZK0DO9cy9R1bWR 2/7 6/23/23, 2:35 AM Anaphylaxis Pathway As per EAACI 2014 guidelines, prescribe at least one epinephrine auto-injector in patients with any of the following: previous anaphylaxis triggered by food, latex, or aeroallergens previous exercise-induced anaphylaxis previous idiopathic anaphylaxis coexisting unstable or moderate-to-severe, persistent asthma and a food allergy (excluding pollen food syndrome) venom allergy in adult patients with previous systemic reactions (not receiving maintenance hymenoptera venom immunotherapy) and pediatric patients with more than cutaneous/mucosal systemic reactions underlying mast cell disorders or elevated baseline serum tryptase concentrations together with any previous systemic allergic reactions to insect stings, even in hymenoptera venom immunotherapy-treated patients. B Show 2 more Corticosteroids and antihistamines: As per WMS 2022 guidelines: Consider administering empiric corticosteroids given the potential for benefit paired with a low side-effect profile. Administer corticosteroids for anaphylaxis with a respiratory component in asthmatic patients. B Consider administering antihistamines early with epinephrine to help blunt the overall severity of anaphylaxis. B Consider preferring nonsedating antihistamines in the field care to help keep the patient alert and potentially able to walk. C Consider adding an H2RA to an H2RA. C As per AAAAI 2020 guidelines, avoid administering corticosteroids or antihistamines for the prevention of biphasic anaphylaxis. D Show 3 more As per EAACI 2014 guidelines: Consider administering oral H1RAs and H2RAs to relieve cutaneous symptoms of anaphylaxis. B Consider administering systemic corticosteroids to reduce the risk of late-phase respiratory symptoms. Consider administering high-dose nebulized corticosteroids for upper airway obstruction. C Intravenous fluids: As per WMS 2022 guidelines, consider administering IV crystalloids with additional doses of intramuscularly epinephrine for hypotension after epinephrine administration. B As per EAACI 2014 guidelines, administer IV fluids (crystalloids, boluses of 20 ml/kg) in patients experiencing cardiovascular instability. B Inhaled beta-2 agonists: As per WMS 2022 guidelines, consider administering inhaled -agonists as adjunctive treatment for wheezing, especially in patients with a history of asthma. B https://web.pathway.md/diseases/recZK0DO9cy9R1bWR 3/7 6/23/23, 2:35 AM Anaphylaxis Pathway As per EAACI 2014 guidelines, administer inhaled short-acting -2 agonists additionally to relieve symptoms of bronchoconstriction. B Glucagon: As per WMS 2022 guidelines, consider administering glucagon in patients on long-term - blockers with refractory hypotension. C As per EAACI 2014 guidelines, consider administering parenteral glucagon in patients with anaphylaxis unresponsive to epinephrine, particularly in patients taking -blockers. C 5. Nonpharmacologic interventions Removal of allergen: As per WMS 2022 guidelines, separate the patient from the inciting allergen, if possible, but do not induce vomiting to eliminate a food-based allergen. B As per EAACI 2014 guidelines, remove the trigger of the anaphylaxis episode. B Patient positioning: Keep patients with anaphylaxis still and position them according to their clinical condition: Situation Guidance Supine position with elevated lower extremities Circulatory instability Sitting up Respiratory distress Recovery position Unconscious Semirecumbent on the left side with elevated lower extremities. B Pregnancy Avoid sudden abrupt change to a more upright posture. D Evacuation: proceed to medical evacuation In the field after treatment of anaphylaxis. Consider deciding on the evacuation (also on the timing and method of evacuation) based on case-specific factors, such as geography, weather, field capabilities, and patient characteristics and response to treatment. B 6. Specific circumstances Patients with food-dependent exercise-induced anaphylaxis: Suspect food-dependent exercise-induced anaphylaxis when ingestion of causal food and tempPO related exercise result in symptoms of anaphylaxis. Recognize that symptoms only occur with ingestion of the causal food proximate to exercise and that ingestion of the food in the absence of exercise will not result in anaphylaxis. B https://web.pathway.md/diseases/recZK0DO9cy9R1bWR 4/7 6/23/23, 2:35 AM Anaphylaxis Pathway Advise avoiding food ingestion within 2-4 hours of exercise for the prevention of symptoms in patients with food-dependent exercise-induced anaphylaxis, and initiate prompt treatment with the onset of symptoms. B Patients in cardiac arrest: undertake standard resuscitative measures and administer immediate epinephrine in patients in cardiac arrest secondary to anaphylaxis. B Show 7 more 7. Patient education Patient education: As per EAACI 2022 guidelines, provide structured, comprehensive training to improve knowledge and use of epinephrine autoinjectors in people at risk of anaphylaxis, in addition to basic instructions about autoinjector use. B As per AAAAI 2020 guidelines, educate all patients with anaphylaxis regarding anaphylaxis, including avoidance of identified triggers, presenting signs and symptoms, biphasic anaphylaxis, treatment with epinephrine, and the use of epinephrine autoinjectors. B As per EAACI 2014 guidelines, offer training for the recognition and management of anaphylaxis in all patients and caregivers of pediatric patients at risk of anaphylaxis, ideally from the time of diagnosis. B Show 6 more 8. Preventative measures Premedication: Insufficient evidence to recommend for or against the use of premedication with antihistamines to prevent anaphylaxis. I Consider administering premedication with SC epinephrine to prevent anaphylaxis when snake bite anti-venom is given to a patient. C Allergen immunotherapy: offer SC venom immunotherapy in patients with venom-allergy with a previous episode of anaphylaxis and in adult patients with systemic cutaneous reactions. A Desensitization: consider offering desensitization to Hymenoptera venom and peanuts in patients with a history of anaphylactic reactions to these antigens. B 9. Follow-up and surveillance Clinical observation: As per WMS 2022 guidelines, obtain clinical observation after treatment of anaphylaxis with a length of observation depending on the severity of the initial reaction and risk factors for a biphasic reaction. B Show 2 more https://web.pathway.md/diseases/recZK0DO9cy9R1bWR 5/7 6/23/23, 2:35 AM Anaphylaxis Pathway As per AAAAI 2020 guidelines: Keep all patients under observation after diagnosis and treatment of anaphylaxis in a setting capable of managing anaphylaxis until symptoms have fully resolved. B Consider obtaining extended clinical observation in a setting capable managing anaphylaxis (to detect a biphasic reaction) in patients with resolved severe anaphylaxis and/or requiring > 1 dose of epinephrine. C As per EAACI 2014 guidelines, monitor patients closely for a duration according to their presentation: respiratory compromise: at least 6-8 hours circulatory instability: 12-24 hours. B Discharge from hospital: As per WMS 2022 guidelines, provide patients before discharge from a medical center with an epinephrine prescription and advise to follow-up for allergy testing and consideration of immunotherapy. B As per EAACI 2014 guidelines: Assess the risk of future reactions and prescribe an epinephrine auto-injector before discharging patients at risk of recurrence. B Provide a discharge advice sheet, including allergen avoidance measures (if possible) and instructions for the use of the epinephrine auto-injector. Offer specialist and food allergy specialist dietitian (in food anaphylaxis) follow-up, and contact information for patient support groups. B Indications for allergist referral: refer all patients with anaphylaxis to an allergist. B 10. Quality improvement Health professional training: consider using simulation training and visual prompts to improve healthcare professionals' recognition and management of anaphylaxis in emergency situations. C School policies: consider implementing school policies reflecting anaphylaxis guidelines. C Austere or disaster conditions: consider disassembling an epinephrine auto-injector after intramuscularly administration to obtain another epinephrine dose when no other source is available in austere or disaster conditions. C Show 2 more References 1. A Muraro, G Roberts, M Worm et al. Anaphylaxis: guidelines from the European Academy of Allergy and Clinical Immunology. Allergy. 2014 Aug;69 8 1026 45. Open https://web.pathway.md/diseases/recZK0DO9cy9R1bWR 6/7 6/23/23, 2:35 AM Anaphylaxis Pathway 2. Flavio G Gaudio, David E Johnson, Kelly DiLorenzo et al. Wilderness Medical Society Clinical Practice Guidelines on Anaphylaxis. Wilderness Environ Med. 2022 Mar;33 1 75 91. Open 3. Antonella Muraro, Margitta Worm, Cherry Alviani et al. EAACI guidelines: Anaphylaxis 2021 update). Allergy. 2022 Feb;77 2 357 377. Open 4. Marcus S Shaker, Dana V Wallace, David B K Golden et al. Anaphylaxis-a 2020 practice parameter update, systematic review, and Grading of Recommendations, Assessment, Development and Evaluation GRADE analysis. J Allergy Clin Immunol. 2020 Apr;145 4 1082 1123. Open 5. Hugh A Sampson, Seema Aceves, S Allan Bock et al. Food allergy: a practice parameter update-2014. J Allergy Clin Immunol. 2014 Nov;134 5 1016 25.e43. Open 6. Ashish R Panchal, Jason A Bartos, Jos G Caba as et al. Part 3 Adult Basic and Advanced Life Support: 2020 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Circulation. 2020 Oct 20;142 16_suppl_2 S366 S468. Open 7. Johannes Ring, Kirsten Beyer, Tilo Biedermann et al. Guideline (S2k) on acute therapy and management of anaphylaxis: 2021 update: S2k-Guideline of the German Society for Allergology and Clinical Immunology DGAKI , the Medical Association of German Allergologists AeDA , the Society of Pediatric Allergology and Environmental Medicine GPA , the German Academy of Allergology and Environmental Medicine DAAU , the German Professional Association of Pediatricians BVKJ , the Society for Neonatology and Pediatric Intensive Care GNPI , the German Society of Dermatology DDG , the Austrian Society for Allergology and Immunology GAI , the Swiss Society for Allergy and Immunology SGAI , the German Society of Anaesthesiology and Intensive Care Medicine DGAI , the German Society of Pharmacology DGP , the German Respiratory Society DGP , the patient organization German Allergy and Asthma Association DAAB , the German Working Group of Anaphylaxis Training and Education AGATE . Allergo J Int. 2021;30 1 1 25. Open https://web.pathway.md/diseases/recZK0DO9cy9R1bWR 7/7
Guideline sources The following summarized guidelines for the evaluation and management of androgenetic alopecia are prepared by our editorial team based on guidelines from the Japanese Dermatological Association (JDA 2018), the European Dermatology Forum (EDF 2018), and the American Association of Family Physicians (AAFP 2009). 1 2 3 Pathway Androgenetic alopecia Management Guidelines 1. Diagnostic investigations Laboratory evaluation: obtain targeted endocrine evaluation (testosterone, DHEA sulfate, and prolactin levels) in female patients presenting with hair loss associated with abnormal menses, history of infertility, hirsutism, unresponsive cystic acne, virilization, or galactorrhea. B https://web.pathway.md/diseases/recTxvwyqLJm31lBs 1/5 6/23/23, 2:35 AM Androgenetic alopecia Pathway 2. Medical management Topical minoxidil, male-pattern hair loss: As per EDF 2018 guidelines, offer topical minoxidil 2-5% solution 1 mL or half a cap of 5% foam BID to improve or to prevent the progression of androgenetic alopecia in > 18 years old male patients with mild-to-moderate disease, Hamilton-Norwood IIv-V. A Consider offering topical minoxidil 5% solution or half a cap of 5% foam for greater efficacy. B Show 4 more As per JDA 2018 guidelines, offer topical minoxidil 5% for initial treatment of male-pattern hair loss. A As per AAFP 2009 guidelines: Offer topical minoxidil 2% for hair regrowth in patients with male-pattern hair loss. A Recognize that discontinuation of minoxidil results in loss of any positive effects of treatment (hair growth) in 6 months. A Topical minoxidil, female-pattern hair loss: As per EDF 2018 guidelines, offer topical minoxidil 2% solution 1 mL BID or half a cap of 5% minoxidil topical foam once daily to improve or to prevent the progression of androgenetic alopecia in > 18 years old female patients. A Show 3 more As per JDA 2018 guidelines, offer topical minoxidil 1% for initial treatment of female-pattern hair loss. A As per AAFP 2009 guidelines: Offer topical minoxidil 2% for hair regrowth in patients with female-pattern hair loss. B Recognize that discontinuation of minoxidil results in loss of any positive effects of treatment (hair growth) in 12 months. A Other topical therapies: offer topical adenosine in patients with male-pattern hair loss. B Show 2 more Oral minoxidil: do not use oral minoxidil for the treatment of androgenetic alopecia. D 5-alpha-reductase inhibitors, male-pattern hair loss: As per EDF 2018 guidelines, offer oral finasteride 1 mg/day to improve or to prevent the progression of androgenetic alopecia in > 18 years old male patients with mild-to-moderate disease, Hamilton-Norwood IIv-V. A Show 3 more As per JDA 2018 guidelines, offer oral finasteride or dutasteride in patients with male-pattern hair loss. A As per AAFP 2009 guidelines: Offer oral finasteride to promote hair regrowth in patients with male-pattern hair loss. A Recognize that discontinuation of finasteride results in loss of any positive effects of treatment (hair growth) in 12 months. A https://web.pathway.md/diseases/recTxvwyqLJm31lBs 2/5 6/23/23, 2:35 AM Androgenetic alopecia Pathway 5-alpha-reductase inhibitors, female-pattern hair loss: As per EDF 2018 guidelines: Avoid using oral finasteride in postmenopausal patients with female-pattern hair loss. D Insufficient evidence to recommend for or against the use of oral finasteride in patients with female-pattern hair loss. I As per JDA 2018 guidelines, do not use oral finasteride or dutasteride in patients with female- pattern hair loss. D Hormonal therapy (male-pattern hair loss): do not use oral estrogens or androgen receptor antagonists to improve or to prevent the progression of androgenetic alopecia in male patients. D Show 2 more Hormonal therapy (female-pattern hair loss): insufficient evidence to support the use of oral anti-androgens (chlormadinone acetate, cyproterone acetate, drospirenone, spironolactone, flutamide) to improve or to prevent the progression of androgenetic alopecia in normoandrogenic female patients. Show 3 more Therapies with no evidence for benefit: As per EDF 2018 guidelines, insufficient evidence to recommend for or against the use of the following topical therapies in the treatment of patients with androgenetic alopecia: biochanin A I polysorbate 60 I curcuma aeruginosa I ketoconazole I roxithromycin I zinc pyrithione I glyceroloxyesters and silicium I niacin derivates I viprostol or latanoprost I cysteine or histidine I adenosine I hibiscus I melatonin I proanthocyanidins I red ginseng I tretinoin I valproic acid. I Show 3 more As per JDA 2018 guidelines, do not offer the following therapies in patients with pattern hair loss: https://web.pathway.md/diseases/recTxvwyqLJm31lBs 3/5 6/23/23, 2:35 AM Androgenetic alopecia Pathway bimatoprost latanoprost growth factors cell transplantation therapy. D 3. Nonpharmacologic interventions Use of wigs: consider advising the use of wigs as an option for hair loss. C 4. Therapeutic procedures Laser therapy: As per EDF 2018 guidelines: Consider offering low-level laser therapy (with devices using energy levels shown to be effective in randomized-controlled clinical trials) as an ancillary therapy in patients with androgenetic alopecia. C Insufficient evidence to recommend for or against treatment for > 6 months with low-level laser therapy. I As per JDA 2018 guidelines, offer irradiation with LED and low-level lasers in patients with pattern hair loss. B 5. Surgical interventions Self-hair transplantation, male-pattern hair loss: As per EDF 2018 guidelines: Consider offering follicular unit transplantation in male patients with androgenetic alopecia with sufficient donor hair. C Consider combining follicular unit transplantation with finasteride 1 mg/day to achieve a better clinical outcome. C As per JDA 2018 guidelines, offer self-hair transplantation in patients with male-pattern hair loss. B Self-hair transplantation, female-pattern hair loss: As per EDF 2018 guidelines, consider offering follicular unit transplantation in female patients with sufficient donor hair. C As per JDA 2018 guidelines, consider offering self-hair transplantation in patients with female- pattern hair loss. C Prosthetic hair transplantation: do not offer prosthetic hair transplantation in patients with pattern hair loss. D https://web.pathway.md/diseases/recTxvwyqLJm31lBs 4/5 6/23/23, 2:35 AM Androgenetic alopecia Pathway References 1. Manabe M, Tsuboi R, Itami S et al. Guidelines for the diagnosis and treatment of male-pattern and female-pattern hair loss, 2017 version. J Dermatol. 2018 Sep;45 9 1031 1043. Open 2. Anne L Mounsey, Sean W Reed. Diagnosing and treating hair loss. Am Fam Physician. 2009 Aug 15;80 4 356 62. Open 3. V Kanti, A Messenger, G Dobos et al. Evidence-based S3 guideline for the treatment of androgenetic alopecia in women and in men - short version. J Eur Acad Dermatol Venereol. 2018 Jan;32 1 11 22. Open https://web.pathway.md/diseases/recTxvwyqLJm31lBs 5/5
Guideline sources The following summarized guidelines for the evaluation and management of anemia of chronic kidney disease are prepared by our editorial team based on guidelines from the Japanese Society of Nephrology (JSN 2019), the United Kingdom Kidney Association (UKKA 2017), the British Committee for Standards In Haematology (BCSH 2013), and the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2012). 1 2 3 4 5 6 6 7 Definition Anemia of CKD is usually normocytic, normochromic, and hypoproliferative, which increases as the kidney disease progresses. 5 Epidemiology Anemia of CKD is mostly caused by a relative deficiency in EPO (EPO) production. Other causes include inflammation, iron deficiency, and accumulation of uremic toxins. 6 Disease course Decreased production of EPO due to worsening kidney function results in anemia of CKD, which causes clinical manifestations of decreased quality of life, cognitive impairment, sleep disturbances, CKD progression, and cardiovascular comorbidities. 6 https://web.pathway.md/diseases/recj8yk723oBn05WO 1/8 6/23/23, 2:35 AM Anemia of chronic kidney disease Pathway Prognosis and risk of recurrence Anemia of CKD among diabetics is associated with higher mortality (HR 3.61, 95% CI 2.48-5.26). 7 Guidelines 1. Screening and diagnosis Indications for screening: As per UKKA 2017 guidelines, consider measuring Hgb levels at least at following intervals to screen for anemia in patients with CKD: stage 3: once per year stage 4-5, non-dialysis: twice per year. C As per KDIGO 2012 guidelines, measure Hgb concentration in patients with CKD without anemia when clinically indicated and at least at the following intervals according to the stage of CKD: Situation Guidance Once per year Stage 3 Twice per year Stage 4-5, non-dialysis Every 3 months. Stage 5, on hemodialysis or peritoneal dialysis Diagnostic criteria: As per UKKA 2017 guidelines, suspect CKD as a possible cause of anemia when the GFR is < 60 mL/min/1.73m , especially if < 30 mL/min/1.73m (or < 45 mL/min/1.73m in patients with diabetes) and no other cause such as blood loss, folic acid or vitamin B12 deficiency is identified. B As per KDIGO 2012 guidelines: Diagnose anemia in 15 years old adolescent and adult patients with CKD based on the Hgb concentration < 13.0 g/dL in males and < 12.0 g/dL in females. Diagnose anemia in < 15 years old pediatric patients with CKD based on the age group- specific Hgb concentration: Situation Guidance < 11.0 g/dL 0.5-5 years < 11.5 g/dL 5-12 years https://web.pathway.md/diseases/recj8yk723oBn05WO 2/8 6/23/23, 2:35 AM Anemia of chronic kidney disease Pathway 12-15 years < 12.0 g/dL. 2. Diagnostic investigations Initial evaluation: As per UKKA 2017 guidelines, evaluate all patients with chronic anemia associated with CKD for the cause and possible treatment in case of Hgb levels < 110 g/L (< 105 g/L in patients aged < 2 years) or symptoms attributable to anemia, irrespective of the grade of kidney disease or requirement for RRT. A Show 4 more As per KDIGO 2012 guidelines, obtain the following tests as part of the initial evaluation of anemia in patients with CKD, regardless of age and CKD stage: CBC absolute reticulocyte count serum ferritin level serum transferrin saturation serum vitamin B12 and folate levels. Erythropoietin measurement: avoid obtaining routine measurements of EPO levels for the diagnosis or management of anemia in patients with CKD. D Evaluation for functional iron deficiency: measure mean cell volume and mean cell Hgb values at diagnosis of iron deficiency and for assessing trends over periods of weeks or months, but not for assessing acute changes in iron availability secondary to therapy with ESAs. B Show 9 more 3. Medical management Iron therapy: As per JSN 2019 guidelines, consider administering iron therapy in patients with CKD and iron deficiency anemia. C As per UKKA 2017 guidelines, ensure iron repletion (defined as a percentage of hypochromic RBCs < 6%, reticulocyte Hgb count > 29 pg, ferritin > 100 g/L, and transferrin saturation > 20%) to achieve and maintain target Hgb whether receiving ESAs or not. B Show 6 more As per KDIGO 2012 guidelines, balance the potential benefits of iron therapy (avoiding or minimizing blood transfusions, ESA therapy, and anemia-related symptoms) against potential risks of harm (anaphylactoid reactions, other unknown long-term risks) when prescribing iron therapy. Show 7 more https://web.pathway.md/diseases/recj8yk723oBn05WO 3/8 6/23/23, 2:35 AM Anemia of chronic kidney disease Pathway Erythropoiesis-stimulating agents, initiation: As per UKKA 2017 guidelines, initiate ESAs in patients with anemia of CKD likely to benefit in terms of quality of life and physical function and to avoid blood transfusion, especially in patients deemed suitable for transplantation. B Show 2 more As per KDIGO 2012 guidelines, address all correctable causes of anemia including iron de ciency and in ammatory states before initiating ESAs. Show 6 more Erythropoiesis-stimulating agents, choice of agent: As per UKKA 2017 guidelines, decide on the choice of ESAs based on the local availability of ESAs. B As per KDIGO 2012 guidelines: Decide on the choice of an ESA based on the balance of pharmacodynamics, safety information, clinical outcome data, costs, and availability. B Consider choosing only ESAs approved by an independent regulatory agency. Choose true biosimilar products for 'copy' versions of ESAs. C Erythropoiesis-stimulating agents, route of administration: As per UKKA 2017 guidelines, consider determining the route of ESA administration by the CKD grade, treatment setting, efficacy, safety and class of ESA used. Prefer the SC route in non- dialysis patients and the IV route in hemodialysis patients because of convenience. C As per KDIGO 2012 guidelines: Consider using either IV or SC route of administration of ESAs in patients with CKD stage 5 on hemodialysis and in patients on hemofiltration or hemodia ltration therapy. C Consider using the SC route of administration of ESAs in patients with non-dialysis CKD and in patients with CKD stage 5 on peritoneal dialysis. C Erythropoiesis-stimulating agents, dosing: As per UKKA 2017 guidelines, determine the initial ESA dose based on the patient's Hgb level, target Hgb level, the observed rate of increase in Hgb level, and clinical circumstances. B Show 2 more As per KDIGO 2012 guidelines, determine the initial ESA dose based on the patient's Hgb concentration, body weight, and clinical circumstances. B Show 4 more Erythropoiesis-stimulating agents, maintenance: As per JSN 2019 guidelines, consider reducing or terminating ESAs if the Hgb level is > 12 g/dL in patients with a past history or complication of severe CVD or any other medical indications. C As per UKKA 2017 guidelines, continue ESA therapy during acute illness, surgical procedures, or any other cause of hospitalization in ESA-dependent patients unless there is a clear contraindication, such as accelerated hypertension. B https://web.pathway.md/diseases/recj8yk723oBn05WO 4/8 6/23/23, 2:35 AM Anemia of chronic kidney disease Pathway Landmark trials: CHOIR In patients with anemia of CKD, high-Hgb was inferior to low-Hgb with respect to death, myocardial infarction, hospitalization for congestive HF, or stroke Singh AK et al. N Engl J Med. 2006 Nov 16. As per KDIGO 2012 guidelines, avoid using ESAs to maintain Hgb concentration > 11.5 g/dL in adult patients with CKD. D Show 2 more Treatment targets: As per JSN 2019 guidelines, target Hgb levels of 11 g/dL and < 13 g/dL for ESA use in patients with non-dialysis CKD. B As per UKKA 2017 guidelines: Do not exceed serum ferritin level > 800 g/L in patients treated with iron therapy and review treatment when ferritin level is > 500 g/L. D Consider achieving Hgb targets of 100-120 g/L in adult patients (and pediatric patients aged > 2 years) with CKD on ESA therapy. C Adjuvant therapies: Do not use androgens as an adjuvant to ESAs. D Avoid using vitamin C, vitamin D, vitamin E, folic acid, L-carnitine, and pentoxifylline as adjuvants to ESAs. D 4. Therapeutic procedures Red blood cell transfusion: As per UKKA 2017 guidelines, avoid administering RBC transfusion where possible to minimize the risk of allosensitisation in patients with anemia of CKD, especially if renal transplantation is an option. D Show 2 more As per KDIGO 2012 guidelines, avoid administering red cell transfusions when possible to minimize the general risks related to their use when managing chronic anemia. D Show 4 more 5. Specific circumstances Pediatric patients (iron therapy): Administer oral iron (or IV iron in patients with hemodialysis-dependent CKD) when transferrin saturation is < 20% and ferritin is < 100 ng/mL in pediatric patients with anemia of CKD not on iron or ESA therapy. B https://web.pathway.md/diseases/recj8yk723oBn05WO 5/8 6/23/23, 2:35 AM Anemia of chronic kidney disease Pathway Administer oral iron (or IV iron in patients with hemodialysis-dependent CKD) to maintain transferrin saturation > 20% and ferritin > 100 ng/mL in pediatric patients with CKD on ESA therapy not receiving iron therapy. B Pediatric patients, ESA therapy: As per UKKA 2017 guidelines: Consider adjusting ESA doses when Hgb is < 105 or > 115 g/L in 2 years old pediatric patients to balance the benefit and safety to patients. Do not wait until Hgb levels are outside the aspirational range before adjusting treatment, instead take action when Hgb levels are within 5 g/L of the range's limits to keep the Hgb level within the aspirational range in < 2 years old pediatric patients. As per KDIGO 2012 guidelines, balance potential bene ts (improvement in quality of life, school attendance/performance, and avoidance of transfusion) against potential harms when selecting Hgb concentration for ESA therapy initiating in individual patients with CKD. B Pediatric patients, treatment targets: As per UKKA 2017 guidelines, aim for a target ferritin level > 100 g/L in pediatric patients with dialysis-dependent CKD and in patients with CKD not receiving ESA therapy. As per KDIGO 2012 guidelines, consider targeting Hgb concentration in the range of 11.0-12.0 g/dL in pediatric patients with CKD receiving ESA therapy. C Renal transplant recipients: treat anemia in renal transplant patients similar to patients with non- dialysis CKD. B Patients with ESA-induced PRCA: As per UKKA 2017 guidelines, do not obtain routine screening for anti-EPO antibodies in patients with CKD regularly treated with ESAs. D Show 3 more As per KDIGO 2012 guidelines, evaluate for possible antibody-mediated pure red cell aplasia in patients receiving ESAs for > 8 weeks and developing all of the following: sudden rapid decrease in Hgb concentration (at the rate of 0.5-1.0 g/dL per week, or requirement for transfusions at the rate of approximately 1-2 per week) normal platelet and white cell counts absolute reticulocyte count < 10, 000/mL. Show 2 more 6. Follow-up and surveillance Serial hemoglobin monitoring (patients not on ESA therapy): Measure Hgb concentration in patients with anemia of CKD when clinically indicated and at least at the following intervals according to the stage of CKD: stage 3-5, non-dialysis or peritoneal dialysis: every 3 months stage 5, on hemodialysis: every month. https://web.pathway.md/diseases/recj8yk723oBn05WO 6/8 6/23/23, 2:35 AM Anemia of chronic kidney disease Pathway Serial hemoglobin monitoring, patients on ESA therapy: As per UKKA 2017 guidelines: Consider monitoring Hgb concentration every 2-4 weeks in the correction phase and every 1-3 months in stable patients in the maintenance phase. C Consider obtaining more frequent monitoring depending on clinical circumstances. C As per KDIGO 2012 guidelines, measure Hgb concentration at least monthly during the initiation phase of ESA therapy. Show 2 more Serial iron status monitoring: As per UKKA 2017 guidelines, monitor iron status every 1-3 months in patients receiving IV iron to avoid toxicity. B Consider viewing serum ferritin consistently > 800 g/L with no evidence of inflammation, such as normal CRP, as suggestive of iron overload. B As per KDIGO 2012 guidelines: Obtain serum ferritin and transferrin saturation to assess iron status at least every 3 months during ESA therapy, and initiate or continue iron therapy accordingly. Assess iron status (ferritin and transferrin saturation) more frequently when initiating or increasing ESA dose, when there is blood loss, when monitoring response after a course of IV iron, and in other circumstances where iron stores may become depleted. Serial blood pressure monitoring: monitor BP in all patients receiving ESAs and, treat hypertension, if present, by volume removal and/or antihypertensive drugs. A Management of ESA hyporesponsiveness: As per JSN 2019 guidelines, determine and correct the causes of ESA hyporesponsiveness. Avoid overdosing ESAs. B As per UKKA 2017 guidelines: Define inadequate response ('resistance') to ESA therapy as failure to reach the target Hgb level despite SC epoetin dose > 300 IU/kg/week (450 IU/kg/week IV epoetin) or darbepoetin dose > 1.5 g/kg/week. A Obtain clinical assessment and evaluation for other common causes of anemia in patients with hyporesponsiveness with iron repletion. A As per KDIGO 2012 guidelines, classify patients as having ESA hyporesponsiveness if there is no increase in Hgb concentration from baseline after the first month of ESA treatment at appropriate weight-based dosing. Show 5 more References 1. KDIGO Executive Committee. KDIGO Clinical Practice Guideline for Anemia in Chronic Kidney Disease. KDIGO. 2012 Aug. Open https://web.pathway.md/diseases/recj8yk723oBn05WO 7/8 6/23/23, 2:35 AM Anemia of chronic kidney disease Pathway 2. Mikhail A, Brown C, Williams JA et al. Renal association clinical practice guideline on Anaemia of Chronic Kidney Disease. BMC Nephrol. 2017 Nov 30;18 1 345. Open 3. Japanese Society of Nephrology. Essential points from Evidence-based Clinical Practice Guidelines for Chronic Kidney Disease 2018. Clin Exp Nephrol. 2019 Jan;23 1 1 15. Open 4. Thomas DW, Hinchliffe RF, Briggs C et al. Guideline for the laboratory diagnosis of functional iron deficiency. Br J Haematol. 2013 Jun;161 5 639 48. Open 5. Jodie L Babitt, Herbert Y Lin. Mechanisms of anemia in CKD. 2012 Oct;23 10 1631 4.2012 Oct;23 10 1631 4. Open 6. Melissa E Stauffer, Tao Fan. Prevalence of anemia in chronic kidney disease in the United States. 2014 Jan 2;9 1):e84943.2014 Jan 2;9 1):e84943. Open 7. C Yang, J W Wang, Y Z Yang et al. Impact of anemia and chronic kidney disease on the risk of cardiovascular disease and all-cause mortality among diabetic patients]. 2018 Jun 18;50 3 495 500.2018 Jun 18;50 3 495 500. Open https://web.pathway.md/diseases/recj8yk723oBn05WO 8/8
Guideline sources The following summarized guidelines for the evaluation and management of ankle sprain are prepared by our editorial team based on guidelines from the Dutch Orthopaedic Society (DOS 2018). 1 2 2 2 2 Definition An ankle sprain is an injury to the lateral ligament complex of the ankle joint. 2 Epidemiology A traumatic inversion and adduction of the plantar flexed foot is the most common cause of an ankle sprain. Risk factors include a previous ankle sprain, a history of ligament hyperlaxity syndrome, and anatomical misalignments (crus varum and pes cavo-varus). 2 Disease course The inversion and supination of plantar flexed foot result in an ankle sprain, which causes clinical manifestations of pain, intermittent swelling localized to the lateral side of the affected ankle, mechanical instability, and stiffness. Persistent or recurrent instability can lead to degenerative changes in the cartilage. 2 Prognosis and risk of recurrence https://web.pathway.md/diseases/recwviXlMJ8BdmmWG 1/3 6/23/23, 2:36 AM Ankle sprain Pathway An ankle sprain is not associated with an increase in mortality. 2 Calculator Ottawa ankle rule Guidelines 1. Diagnostic investigations Clinical evaluation: identify modifiable risk factors such as deficiencies in proprioception and ROM when treating patients with an acute lateral ankle sprain. B Show 2 more 2. Medical management Nonsteroidal anti-inflammatory drugs: consider administering NSAIDs to reduce pain and swelling in patients with an acute lateral ankle sprain, balancing these benefits with the risks of drug-induced adverse events and possible delayed tissue healing. C 3. Nonpharmacologic interventions Rest, ice, compression and elevation: avoid using only rest, ice, compression, and elevation (RICE) in the management of ankle sprains, as there is no evidence that this combination of interventions alone has a positive influence on pain, swelling or patient function. D Functional support: advise exercise therapy with functional support, rather than joint immobilization, as this has been associated better outcomes compared with immobilization. Maintain immobilization for a maximum of 10 days, if used to treat pain or edema, after which functional treatment should be initiated. B Special shoe wear: insufficient evidence to make a conclusive recommendation regarding special shoe wear for the management of ankle sprains. I Exercise therapy: Advise exercise therapy in patients with lateral ankle sprains, in order to optimise recovery of joint functionality. A Insufficient evidence to recommend whether exercise therapy should be supervised or not. I 4. Surgical interventions https://web.pathway.md/diseases/recwviXlMJ8BdmmWG 2/3 6/23/23, 2:36 AM Ankle sprain Pathway Ligament repair: Implement nonoperative treatment as first-line therapy in most patients with ankle sprains, as not all patients require surgical treatment. A Avoid operative treatment in acute ankle sprains, as there is no strong evidence to support their effectiveness in this setting. D 5. Preventative measures Rehabilitation programs: refer patients with an acute lateral ankle sprain in whom deficiencies in propriorception and ROM have been identified to a prevention and/or rehabilitation programme to mitigate the risk for recurrent sprains. B Taping and bracing: consider both tape and brace for the prevention of recurrent lateral ankle sprains, despite a limited understanding of the mechanisms that leads to their beneficial effects. B References 1. Vuurberg G, Hoorntje A, Wink LM et al. Diagnosis, treatment and prevention of ankle sprains: update of an evidence-based clinical guideline. Br J Sports Med. 2018 Aug;52 15 956. Open 2. Peter Aa Struijs, Gino Mmj Kerkhoffs. Ankle sprain. 2010 May 13;2010 1115.2010 May 13;2010 1115. Open 3. Martin RL, Davenport TE, Paulseth S et al. Ankle stability and movement coordination impairments: ankle ligament sprains. J Orthop Sports Phys Ther. 2013 Sep;43 9 A1 40. Open https://web.pathway.md/diseases/recwviXlMJ8BdmmWG 3/3
Guideline sources The following summarized guidelines for the evaluation and management of ankylosing spondylitis (AS) are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023), the Assessment of SpondyloArthritis international Society (ASAS/EULAR 2022), the European League Against Rheumatism (EULAR 2022), the American College of Radiology (ACR 2021; 2017), the Spondyloarthritis Research and Treatment Network (SPARTAN/ACR/SAA 2019), and the British Society for Rheumatology (BSR/BHPR 2017). 1 2 3 4 5 6 7 8 8 8 9 10 11 Definition AS is a chronic inflammatory disease characterized by arthritis of the spine and the sacroiliac joints. 8 Epidemiology https://web.pathway.md/diseases/recBFY65QPWugVBdu 1/9 6/23/23, 2:36 AM Ankylosing spondylitis Pathway AS is strongly associated with the HLA-B27 genotype, although most persons with this genotype do not develop the disease. The etiology of joint inflammation in patients with AS involves the interaction of genetic predisposition and environmental factors with alterations in immune cell function, and cytokine regulation. 8 10 Pathophysiology In the United States, the annual incidence and prevalence of AS are 3.1 cases per 100,000 person- years and 197 persons per 100,000 population, respectively. 9 Disease course Clinical manifestations of AS include arthritis, dactylitis, ankylosis, osteoporosis of the spine and peripheral bones, uveitis, and IBD. 8 Prognosis and risk of recurrence Patients with AS have an increased risk of mortality, with an estimated standardized mortality ratio of 1.61. CVD is the most frequent cause of death (40%), followed by malignant (27%) and infectious (23%) diseases. 11 Guidelines 1. Classification and risk stratification Severity assessment: measure BASDAI on 2 occasions at least 4 weeks apart. B Define the disease as active if BASDAI and spinal pain Visual Analogue Scale score is 4 despite standard therapy. B 2. Diagnostic investigations Diagnostic imaging: As per ACR 2021 guidelines, obtain radiography of sacroiliac joints or sacroiliac joints and spine area of interest as the initial imaging of suspected axial spondyloarthritis. B Show 3 more As per ACR 2017 guidelines: Obtain radiography (often showing characteristic osseous findings) as initial imaging in patients with suspected seronegative spondyloarthropathy. E Obtain ultrasound or MRI to complement radiography by showing synovitis and identifying additional erosions. E Screening for osteoporosis: Consider screening for osteopenia/osteoporosis with dual X-ray absorptiometry. C https://web.pathway.md/diseases/recBFY65QPWugVBdu 2/9 6/23/23, 2:36 AM Ankylosing spondylitis Pathway Consider screening for osteoporosis/osteopenia with dual X-ray absorptiometry of the spine and hips over dual X-ray absorptiometry solely of the hip or other non-spine sites in adult patients with syndesmophytes or spinal fusion. C Screening for heart diseases: do not screen for cardiac conduction defects (with ECG) or VHDs (with echocardiogram). D Screening for chronic infections: screen for HBV infection in all patients eligible for treatment with conventional synthetic, biological, or targeted synthetic DMARDs, immunosuppressants, or corticosteroids (according to dose and duration). B Show 3 more 3. Medical management General principles: As per ASAS 2022 guidelines, individualize treatment of patients with axial spondyloarthritis according to the current signs and symptoms of the disease (axial, peripheral, extra- musculoskeletal manifestations) and the patient characteristics including comorbidities and psychosocial factors. Guide treatment according to a predefined treatment target. E As per ACR 2019 guidelines, avoid using a treat-to-target strategy with a target of ASDAS < 1.3 (or 2.1) over a treatment strategy based on physician assessment in adult patients with active AS. D Management of active AS, NSAIDs: As per EULAR/ASAS 2022 guidelines, initiate NSAIDs up to the maximum dose as first-line pharmacotherapy, taking risks and benefits into account, in patients experiencing pain and stiffness. Continue treatment if needed to control symptoms in patients responding well to NSAIDs. E As per SPARTAN/SAA/ACR 2019 guidelines: Initiate NSAIDs in adult patients with AS. B Consider preferring continuous over on-demand treatment with NSAIDs. B Insufficient evidence to recommend any particular NSAID as the preferred choice. I Landmark trials: PRECISION In patients at increased cardiovascular risk in whom NSAIDs were indicated for OA or rheumatoid arthritis, celecoxib was noninferior to ibuprofen or naproxen with regard to cardiovascular safety. The risk of gastrointestinal events was significantly lower with celecoxib than with naproxen and ibuprofen, and the risk of renal events was significantly lower with celecoxib than with ibuprofen. Nissen SE et al. N Engl J Med. 2016 Dec 29. Management of active AS, DMARDs: https://web.pathway.md/diseases/recBFY65QPWugVBdu 3/9 6/23/23, 2:36 AM Ankylosing spondylitis Pathway As per EULAR/ASAS 2022 guidelines, avoid using conventional synthetic DMARDs in patients with purely axial disease. Consider initiating sulfasalazine in patients with peripheral arthritis. D As per SPARTAN/SAA/ACR 2019 guidelines, consider initiating sulfasalazine, methotrexate, or tofacitinib in adult patients with active AS despite treatment with NSAIDs. Consider initiating sulfasalazine or methotrexate only in patients with prominent peripheral arthritis or when TNF inhibitors are not available. C Show 2 more Management of active AS, biologic agents: As per EULAR/ASAS 2022 guidelines, consider initiating TNF inhibitors, IL-17 inhibitors, or JAK inhibitors in patients with persistently high disease activity despite conventional treatments. E Show 3 more As per SPARTAN/SAA/ACR 2019 guidelines, consider initiating TNF inhibitors over tofacitinib in adult patients with active AS despite treatment with NSAIDs. C Show 9 more As per BHPR/BSR 2017 guidelines, verify that worsening symptoms, radiological changes, and raised inflammatory markers are due to axial spondyloarthritis and not due to other pathologies, such as malignancy or infection, before initiating biologic treatment. B Show 6 more Management of active AS, corticosteroids: As per EULAR/ASAS 2022 guidelines, do not use long-term systemic corticosteroids in patients with axial disease. D As per SPARTAN/SAA/ACR 2019 guidelines, do not use systemic corticosteroids in adult patients with active AS. D Management of stable AS: As per EULAR 2022 guidelines, consider tapering biological DMARDs in patients with sustained remission. E As per ACR 2019 guidelines, consider prescribing on-demand over continuous NSAIDs in patients with stable AS. C Show 6 more Management of pain: Initiate NSAIDs up to the maximum dose as first-line pharmacotherapy, taking risks and benefits into account, in patients experiencing pain and stiffness. Continue treatment if needed to control symptoms in patients responding well to NSAIDs. E Consider initiating analgesics, such as acetaminophen and opioid-(like) drugs, for residual pain after previously recommended treatments have failed, are contraindicated, and/or poorly tolerated. E Management of uveitis: As per ASAS 2022 guidelines, prefer monoclonal antibodies against TNF in patients with a history of recurrent uveitis. E https://web.pathway.md/diseases/recBFY65QPWugVBdu 4/9 6/23/23, 2:36 AM Ankylosing spondylitis Pathway As per ACR 2019 guidelines, treat adult patients with acute iritis by an ophthalmologist to decrease the severity, duration or complications of episodes. B Show 2 more 4. Nonpharmacologic interventions Smoking cessation: advise smoking cessation in patients with AS. E Physical therapy: As per ASAS 2022 guidelines, encourage patients with AS to exercise on a regular basis. Consider offering physical therapy. E As per ACR 2019 guidelines, offer physical therapy in adult patients with active AS. B Show 4 more Spinal manipulation: do not offer spinal manipulation in adult patients with spinal fusion or advanced spinal osteoporosis. D 5. Therapeutic procedures Local corticosteroid injections: As per ASAS 2022 guidelines, consider administering corticosteroid injections directed to the local site of musculoskeletal inflammation. E As per ACR 2019 guidelines, consider administering local parenteral corticosteroids in adult patients with isolated active sacroiliitis despite treatment with NSAIDs. C Show 2 more 6. Surgical interventions Spinal osteotomy: As per EULAR 2022 guidelines, consider performing spinal corrective osteotomy in patients with severe disabling deformity in specialized centers. E As per ACR 2019 guidelines, avoid performing elective spinal osteotomy in adult patients with severe kyphosis. D Total hip replacement: As per ASAS 2022 guidelines, consider performing total hip arthroplasty in patients with refractory pain or disability and radiographic evidence of structural damage, irrespective of age. E As per ACR 2019 guidelines, perform total hip arthroplasty in adult patients with advanced hip arthritis. B https://web.pathway.md/diseases/recBFY65QPWugVBdu 5/9 6/23/23, 2:36 AM Ankylosing spondylitis Pathway 7. Specific circumstances Patients with inflammatory bowel disease: As per ASAS 2022 guidelines, prefer monoclonal antibodies against TNF in patients with active IBD. E As per ACR 2019 guidelines: Insufficient evidence to recommend any particular NSAID as the preferred choice to decrease the risk of worsening of IBD symptoms in adult patients with IBD. I Consider initiating TNF inhibitor monoclonal antibodies over other biologic agents in adult patients with IBD. C 8. Patient education General counseling: As per ASAS 2022 guidelines, educate patients with axial spondyloarthritis about their condition. E As per ACR 2019 guidelines: Consider advising participation in formal group or individual self-management education in adult patients with active or stable AS. C Consider obtaining fall evaluation and counseling in adult patients with active or stable AS. C 9. Preventative measures Routine immunizations: consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications. C Show 17 more Prophylaxis for Pneumocystis jirovecii pneumonia: consider administering prophylaxis against P. jirovecii pneumonia in patients initiating high-dose corticosteroids, especially in combination with immunosuppressants and depending on the risk-benefit ratio. C 10. Follow-up and surveillance Monitoring of disease activity: As per ASAS 2022 guidelines, assess patient-reported outcomes and clinical findings, obtain laboratory tests and imaging, all with the appropriate instruments and relevant to the clinical presentation, for disease monitoring of patients with axial spondyloarthritis. Decide on the frequency of monitoring on an individual basis depending on symptoms, severity, and treatment. E https://web.pathway.md/diseases/recBFY65QPWugVBdu 6/9 6/23/23, 2:36 AM Ankylosing spondylitis Pathway As per ACR 2021 guidelines, obtain radiography of sacroiliac joints +/- spine area of interest as the follow-up imaging for treatment response or disease progression in patients with axial spondyloarthritis. B As per ACR 2019 guidelines, consider obtaining a validated AS disease activity measurement for regular-interval use and monitoring. C Show 4 more Assessment of treatment response: As per ASAS 2022 guidelines: Obtain prompt re-evaluation of the diagnosis in the absence of treatment response and take into consideration the presence of comorbidities. E Suspect causes other than inflammation, such as spinal fracture, and obtain an appropriate evaluation, including imaging, if a significant change in the course of the disease occurs. E As per BSR 2017 guidelines, assess initial efficacy response following 3-6 months of anti-TNF therapy and reassess it every 6 months thereafter. B Show 2 more Likelihood Ratios Pertinent positives The following findings increase the probability of ankylosing spondylitis in adults. -1 Finding LR+ Value Presence of sacroiliitis 9.0 Presence of HLA-B27 9.0 Presence of anterior uveitis 7.3 History of alternating buttock pain 4.0 Show 4 more Pertinent negatives The following findings decrease the probability of ankylosing spondylitis in adults. -1 Finding LR- Value Absence of HLA-B27 0.1 Absence of sacroiliitis 0.1 No history of inflammatory back pain 0.3 serum CRP not increased 0.6 Show 4 more References https://web.pathway.md/diseases/recBFY65QPWugVBdu 7/9 6/23/23, 2:36 AM Ankylosing spondylitis Pathway 1. Sofia Ramiro, Elena Nikiphorou, Alexandre Sepriano et al. ASAS EULAR recommendations for the management of axial spondyloarthritis: 2022 update. Ann Rheum Dis. 2022 Oct 21;ard-2022 223296. Open 2. Michael M Ward, Atul Deodhar, Lianne S Gensler et al. 2019 Update of the American College of Rheumatology/Spondylitis Association of America/Spondyloarthritis Research and Treatment Network Recommendations for the Treatment of Ankylosing Spondylitis and Nonradiographic Axial Spondyloarthritis. Arthritis Rheumatol. 2019 Oct;71 10 1599 1613. Open 3. George E Fragoulis, Elena Nikiphorou, Mrinalini Dey et al. 2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis. 2022 Nov 3;ard-2022 223335. Open 4. Hamilton L, Barkham N, Bhalla A et al. BSR and BHPR guideline for the treatment of axial spondyloarthritis (including ankylosing spondylitis) with biologics. Rheumatology Oxford). 2017 Feb;56 2 313 316. Open 5. Expert Panel on Musculoskeletal Imaging, Gregory J Czuczman, Jacob C Mandell et al. ACR Appropriateness Criteria Inflammatory Back Pain: Known or Suspected Axial Spondyloarthritis: 2021 Update. J Am Coll Radiol. 2021 Nov;18 11S S340 S360. Open 6. Anne R Bass, Eliza Chakravarty, Elie A Akl et al. 2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases. Arthritis Rheumatol. 2023 Jan 4. Online ahead of print. Open 7. Expert Panel on Musculoskeletal Imaging:, Jon A Jacobson, Catherine C Roberts et al. ACR Appropriateness Criteria Chronic Extremity Joint Pain-Suspected Inflammatory Arthritis. J Am Coll Radiol. 2017 May;14 5S S81 S89. Open 8. Taurog JD, Chhabra A, Colbert RA. Ankylosing Spondylitis and Axial Spondyloarthritis. N Engl J Med. 2016 Jun 30;374 26 2563 74. Open 9. Wright KA, Crowson CS, Michet CJ et al. Time trends in incidence, clinical features, and cardiovascular disease in ankylosing spondylitis over three decades: a population-based study. Arthritis Care Res Hoboken). 2015 May;67 6 836 41. Open 10. Tsui FW, Tsui HW, Akram A et al. The genetic basis of ankylosing spondylitis: new insights into disease pathogenesis. Appl Clin Genet. 2014 May 22;7 105 15. Open 11. Bakland G, Gran JT, Nossent JC. Increased mortality in ankylosing spondylitis is related to disease activity. Ann Rheum Dis. 2011 Nov;70 11 1921 5. Open 12. Ward MM, Deodhar A, Akl EA et al. American College of Rheumatology/Spondylitis Association of America/Spondyloarthritis Research and Treatment Network 2015 Recommendations for the Treatment of Ankylosing Spondylitis and Nonradiographic Axial Spondyloarthritis. Arthritis Rheumatol. 2016 Feb;68 2 282 98. Open 13. D sir e van der Heijde, Sofia Ramiro, Robert Landew et al. 2016 update of the ASAS EULAR management recommendations for axial spondyloarthritis. Ann Rheum Dis. 2017 Jun;76 6 978 991. Open 14. P Mandl, V Navarro-Comp n, L Terslev et al. EULAR recommendations for the use of imaging in the diagnosis and management of spondyloarthritis in clinical practice. Ann Rheum Dis. 2015 Jul;74 7 1327 39. Open https://web.pathway.md/diseases/recBFY65QPWugVBdu 8/9 6/23/23, 2:36 AM Ankylosing spondylitis Pathway https://web.pathway.md/diseases/recBFY65QPWugVBdu 9/9
Guideline sources The following summarized guidelines for the evaluation and management of anogenital warts are prepared by our editorial team based on guidelines from the International Union Against Sexually Transmitted Infections (IUSTI 2020). 1 Guidelines 1. Diagnostic investigations Physical examination: perform a physical examination using a good light source. B Show 4 more Screening for sexually transmitted infections: screen for sexually-transmitted infections as per local guidelines in patients with a first episode of genital warts. B https://web.pathway.md/diseases/recVOkmw42RORxf77 1/4 6/23/23, 2:36 AM Anogenital warts Pathway Screening for cervical cancer: inform female patients about cervical cytology screening as per local or national guidelines. B Assessment of sexual partners: offer clinical assessment for the presence of warts along with education and advise about HPV infection and screening for other STIs to current partners of patients with anogenital warts. B 2. Diagnostic procedures Colposcopy: consider magnification with a lens or colposcope for small lesions. C Proctoscopy: obtain proctoscopy if anal canal warts are suspected, such as external lesions extending into the anal canal, anal bleeding or discharge. B Biopsy: do not obtain biopsy for typical anogenital warts, unless there is diagnostic uncertainty or suspicion of precancer or cancer. D 3. Medical management First-line patient-applied therapy: Offer one of the following medications as first-line patient-applied therapy: podophyllotoxin 0.5% solution self-applied to lesions BID for 3 days, followed by 4 rest days, for up to 4 or 5 weeks A podophyllotoxin 0.15% cream self-applied to lesions BID for 3 days, followed by 4 rest days, for up to 4 or 5 weeks B imiquimod 5% cream self-applied directly to the warts thrice weekly before normal sleeping hours and washed off with soap and water between 6 and 10 hours later, until wart clearance, or for up to 16 weeks A sinecatechins 10% or 15% ointment self-applied TID until complete clearance, or for up to 16 weeks A Use podophyllotoxin cream in patients with vulval and perianal warts, as clinical experience suggests that for ease of application the cream formulation is preferable for warts at these sites. Trichloracetic acid: offer trichloracetic acid 80-90% solution as first-line clinician-applied therapy applied weekly directly onto the wart surface with either a wooden or cotton tipped applicator. Protect the surrounding skin with petroleum jelly, as excess application may cause scarring. Ensure readily available neutralizing agent (such as 5% sodium bicarbonate) in case of spills. A 5-fluorouracil: consider 5-fluorouracil when other treatments have failed. B Intralesional interferon injections: consider administering intralesional interferon injections for patients with refractory anogenital warts. B Podophyllin: do not use podophyllin preparations for the management of patients with anogenital warts because they contain a variety of compounds some of which may be mutagenic and severe https://web.pathway.md/diseases/recVOkmw42RORxf77 2/4 6/23/23, 2:36 AM Anogenital warts Pathway systemic toxicity after topical use has been described including death, intrauterine death, teratogenicity and neurological complications. 4. Nonpharmacologic interventions Smoking cessation: advise smoking cessation for general health benefit, acknowledging that there is no evidence that smoking cessation improves outcomes of wart treatment. B 5. Therapeutic procedures Photodynamic therapy: insufficient evidence to support photodynamic therapy for the management of patients with anogenital warts. I 6. Surgical interventions First-line clinician-applied therapy: Offer one of the following surgical methods as clinician- applied first-line therapy: surgical excision cryotherapy electrosurgery and electrocautery laser surgery (CO and neodymium:yttrium aluminium garnet laser). A Show 2 more Combination therapies: insufficient evidence to support any combination therapies for the management of patients with anogenital warts. I 7. Specific circumstances Management of internal involvement: treat patients with vaginal warts with either cryotherapy, trichloracetic acid or any surgical treatment modality. B Show 4 more Pregnant patients: avoid topical treatments in pregnant patients. Treat pregnant patients with ablation using cryotherapy, trichloracetic acid or any surgical treatment modality. Liaise with the obstetrician in all cases of pregnant patients. D Show 2 more 8. Patient education Patient education: provide patients with a detailed explanation of their condition, including advice about onward transmission. B https://web.pathway.md/diseases/recVOkmw42RORxf77 3/4 6/23/23, 2:36 AM Anogenital warts Pathway Show 2 more 9. Follow-up and surveillance Follow-up: follow-up patients at regular intervals, for example, every 4 weeks, until warts have resolved, and switch of treatments if an inadequate response is observed. B 10. Quality improvement Local treatment algorithms: develop local treatment algorithms that address the needs of the given patient population and are deliverable with the resources available to the service. B Surgical team safety: perform electrosurgery, electrocautery and laser surgery with the use of surgical masks by the treatment team, and use an extractor fan due to the potential presence of infectious HPV particles in the smoke plume generated by these techniques. B References 1. R Gilson, D Nugent, R N Werner et al. 2019 IUSTI Europe guideline for the management of anogenital warts. J Eur Acad Dermatol Venereol. 2020 Aug;34 8 1644 1653. Open https://web.pathway.md/diseases/recVOkmw42RORxf77 4/4
Guideline sources The following summarized guidelines for the evaluation and management of anorectal abscess are prepared by our editorial team based on guidelines from the American Society of Colon and Rectal Surgeons (ASCRS 2022; 2020), the World Society of Emergency Surgery (WSES/AAST 2021), the American College of Radiology (ACR 2021), the American College of Gastroenterology (ACG 2018), and the Association of Coloproctologists in Germany (BCD/DGAV/DGAV/DDG/DGGG/DGU/DGK/AGUB/CACP 2017). 1 2 3 4 5 6 7 Guidelines 1. Diagnostic investigations Clinical examination: As per ASCRS 2022 guidelines, elicit a disease-specific history and perform a physical examination assessing symptoms, relevant history, location of the abscess and fistula, and the presence of secondary cellulitis in the initial evaluation of patients with anorectal abscess. B As per WSES 2021 guidelines, consider eliciting a focused medical history and perform a complete physical examination, including a DRE, in patients with suspected anorectal abscess. C https://web.pathway.md/diseases/recMcRK6cN06YZbXf 1/4 6/23/23, 2:37 AM Anorectal abscess Pathway As per DGAV 2017 guidelines, elicit a medical history and perform a clinical examination to establish the indication for surgery in patients with anorectal abscess. A Laboratory tests: Consider obtaining the following tests to identify an undetected diabetes mellitus in patients with suspected anorectal abscess: serum glucose hgb A1c urine ketones. B Consider obtaining the following tests to assess the status of patients with suspected anorectal abscess and signs of systemic infection or sepsis: CBC serum creatinine inflammatory markers (such as CRP, procalcitonin, and lactate). C Diagnostic imaging: As per ASCRS 2022 guidelines: Do not obtain routine diagnostic imaging in patients with anorectal abscess. D Consider obtaining imaging in selected patients with occult anorectal abscess, immunosuppression, or anorectal Crohn's disease. B As per WSES 2021 guidelines, consider obtaining imaging investigations (MRI, CT, or anorectal ultrasound depending on the specific clinical scenario and the available skills and resources) in patients with suspected anorectal abscess in case of atypical presentation or suspicion of occult supralevator abscesses, complex anal fistula, or perianal Crohn's disease. C As per ACR 2021 guidelines, consider obtaining pelvic MRI without and with IV contrast or pelvic CT with IV contrast as the initial imaging in patients with suspected anorectal abscess. C As per ACG 2018 guidelines, consider obtaining cross-sectional imaging with MRI of the pelvis and/or EUS to characterize perianal involvement and perirectal abscesses in patients with Crohn's disease. E As per DGAV 2017 guidelines, obtain further investigations (anorectal ultrasound, MRI) only in case of recurrent, complex abscesses and complex fistulas of difficult clinical classification. A 2. Medical management Antibiotic therapy: As per ASCRS 2022 guidelines, reserve antibiotics for patients with anorectal abscess complicated by cellulitis, systemic signs of infection, or underlying immunosuppression. B As per WSES 2021 guidelines, consider administering antibiotics in patients with drained anorectal abscess in the presence of sepsis and/or surrounding soft tissue infection or in case of disturbances of the immune response. C https://web.pathway.md/diseases/recMcRK6cN06YZbXf 2/4 6/23/23, 2:37 AM Anorectal abscess Pathway 3. Surgical interventions Incision and drainage: As per ASCRS 2022 guidelines, perform prompt incision and drainage in patients with acute anorectal abscess. B As per WSES 2021 guidelines, perform incision and drainage in patients with anorectal abscess. B Show 4 more As per DGAV 2017 guidelines, perform surgery, via a transrectal or perianal approach depending on the location of the abscess, aiming for thorough drainage of the infection focus while preserving the sphincter structures in patients with anorectal abscess. E Show 2 more Concomitant fistulotomy: As per ASCRS 2022 guidelines, consider performing concomitant fistulotomy along with abscess drainage in selected patients with simple anal fistula. C As per WSES 2021 guidelines, consider performing fistulotomy at the time of abscess drainage in patients with anorectal abscess and an obvious fistula, only in cases of low fistula not involving sphincter muscle (subcutaneous fistula). C Show 2 more 4. Specific circumstances Patients with Crohn's disease: As per WSES 2021 guidelines, perform adequate surgical drainage of a perianal abscess without searching for an associated fistula in patients with Crohn's disease. B Show 3 more As per ASCRS 2020 guidelines, consider administering antibiotics with or without drainage in patients with penetrating Crohn's disease with abscess formation, followed by interval elective resection or medical therapy depending on the clinical situation and patient preferences. C As per ACG 2018 guidelines, perform prompt surgical drainage of the perianal abscess in patients with Crohn's disease. E Perform abscess drainage, surgically or percutaneously, before treatment of fistulizing Crohn's disease with anti-TNF agents. B References 1. Andreas Ommer, Alexander Herold, Eugen Berg et al. German S3 guidelines: anal abscess and fistula (second revised version). Langenbecks Arch Surg. 2017 Mar;402 2 191 201. Open 2. Antonio Tarasconi, Gennaro Perrone, Justin Davies et al. Anorectal emergencies: WSES AAST guidelines. World J Emerg Surg. 2021 Sep 16;16 1 48. Open https://web.pathway.md/diseases/recMcRK6cN06YZbXf 3/4 6/23/23, 2:37 AM Anorectal abscess Pathway 3. Lichtenstein GR, Loftus EV, Isaacs KL et al. ACG Clinical Guideline: Management of Crohn's Disease in Adults. Am J Gastroenterol. 2018 Apr;113 4 481 517. Open 4. Wolfgang B Gaertner, Pamela L Burgess, Jennifer S Davids et al. The American Society of Colon and Rectal Surgeons Clinical Practice Guidelines for the Management of Anorectal Abscess, Fistula-in-Ano, and Rectovaginal Fistula. Dis Colon Rectum. 2022 Aug 1;65 8 964 985. Open 5. Amy L Lightner, Jon D Vogel, Joseph C Carmichael et al. The American Society of Colon and Rectal Surgeons Clinical Practice Guidelines for the Surgical Management of Crohn's Disease. Dis Colon Rectum. 2020 Aug;63 8 1028 1052. Open 6. Belinda De Simone, Justin Davies, Elie Chouillard et al. WSES AAST guidelines: management of inflammatory bowel disease in the emergency setting. World J Emerg Surg. 2021; 16 23. Open 7. Expert Panel on Gastrointestinal Imaging: Angela D. Levy, MD, Peter S. Liu et al. ACR Appropriateness Criteria Anorectal Disease. ACR. 2021. Open 8. Vogel JD, Johnson EK, Morris AM et al. Clinical Practice Guideline for the Management of Anorectal Abscess, Fistula-in-Ano, and Rectovaginal Fistula. Dis Colon Rectum. 2016 Dec;59 12 1117 1133. Open https://web.pathway.md/diseases/recMcRK6cN06YZbXf 4/4
Guideline sources The following summarized guidelines for the evaluation and management of anorectal fistula are prepared by our editorial team based on guidelines from the American Society of Colon and Rectal Surgeons (ASCRS 2022), the American College of Radiology (ACR 2021), the World Society of Emergency Surgery (WSES/AAST 2021), the American College of Gastroenterology (ACG 2018), and the Association of Coloproctologists in Germany (BCD/DGAV/DGAV/DDG/DGGG/DGU/DGK/AGUB/CACP 2017). 1 2 3 4 5 Guidelines 1. Diagnostic investigations Clinical examination: As per ASCRS 2022 guidelines, elicit a disease-specific history and perform a physical examination assessing symptoms, relevant history, location of the fistula and abscess, and the presence of secondary cellulitis in the initial evaluation of patients with anorectal fistula. B As per DGAV 2017 guidelines, elicit a medical history and perform a clinical examination to establish the indication for surgery in patients with anorectal fistula. A Diagnostic imaging: As per ASCRS 2022 guidelines: https://web.pathway.md/diseases/recjc0smgZlZ1OMzP 1/4 6/23/23, 2:37 AM p Anorectal fistula Pathway g Do not obtain routine diagnostic imaging in patients with anorectal fistula. D Consider obtaining imaging in selected patients with recurrent or complex anal fistula, immunosuppression, or anorectal Crohn's disease. B As per ACR 2021 guidelines, consider obtaining pelvic MRI without and with IV contrast or pelvic CT with IV contrast as the initial imaging in patients with suspected anorectal fistula. C As per DGAV 2017 guidelines, obtain further investigations (anorectal ultrasound, MRI) only in case of recurrent, complex abscesses and complex fistulas of difficult clinical classification. A 2. Therapeutic procedures Cutting seton placement: As per ASCRS 2022 guidelines, consider placing a cutting seton selectively in the management of complex cryptoglandular anal fistulas. C As per DGAV 2017 guidelines: Consider placing a cutting seton to prepare for subsequent definitive treatment of high anal fistulas demonstrated during abscess drainage. C Do not undertake special bowel preparation in case of seton placement. D Fistula plugging: As per ASCRS 2022 guidelines, consider placing an anal fistula plug for the management of patients with fistula-in-ano, recognizing that this method is relatively ineffective. B As per DGAV 2017 guidelines, consider performing fistula plugging for the management of patients with high anal fistula, recognizing that the healing rates are quite low. C Fibrin glue injection: As per ASCRS 2022 guidelines, consider injecting fibrin glue for the management of patients with fistula-in-ano, recognizing that this method is relatively ineffective. B As per DGAV 2017 guidelines, consider applying fibrin glue only in special cases. C Collagen injection: insufficient evidence to recommend collagen injection for the treatment of patients with anal fistula. I Autologous stem cell injection: insufficient evidence to recommend autologous stem cell injection for the treatment of patients with anal fistula. I 3. Perioperative care Postoperative care: advise rinsing anal area regularly, using tap water. Recognize that the use of local antiseptics is associated with a risk of cytotoxicity. Use antibiotics only in exceptional cases. B https://web.pathway.md/diseases/recjc0smgZlZ1OMzP 2/4 6/23/23, 2:37 AM Anorectal fistula Pathway 4. Surgical interventions Fistulotomy: As per ASCRS 2022 guidelines, consider performing lay-open fistulotomy in patients with simple fistula-in-ano and normal anal sphincter function. B As per DGAV 2017 guidelines, perform primary fistulotomy only in patients with superficial fistulas and by experienced surgeons. A Show 3 more Endoanal advancement flaps: as per ASCRS 2022 guidelines, consider performing endorectal advancement flap repair in patients with fistula-in-ano. B Ligation of intersphincteric fistula tract: As per ASCRS 2022 guidelines, consider performing ligation of the intersphincteric fistula tract for the treatment of patients with transsphincteric fistulas. B As per DGAV 2017 guidelines, consider performing ligation of intersphincteric fistula tract for the treatment of patients with complex fistulas. Recognize that healing and continence rates do not differ significantly from those of the flap procedures. B Fistulectomy with primary reconstruction: consider performing fistula excision with primary reconstruction of the sphincter muscle following complete excision of the fistula and its associated inflammatory tissue, primary readaptation of the divided sphincter apparatus in patients with anorectal fistula. B Show 2 more 5. Specific circumstances Patients with concomitant anorectal abscess: As per ASCRS 2022 guidelines, consider performing concomitant fistulotomy along with abscess drainage in selected patients with anorectal abscess and simple anal fistula. C As per WSES 2021 guidelines, consider performing fistulotomy at the time of abscess drainage in patients with anorectal abscess and an obvious fistula, only in cases of low fistula not involving sphincter muscle (subcutaneous fistula). C Show 2 more Patients with Crohn's disease: As per ASCRS 2022 guidelines, manage anorectal fistula associated with Crohn's disease with a combination of surgical and medical approaches. B Show 6 more As per ACG 2018 guidelines, perform abscess drainage (surgically or percutaneously) before initiating anti-TNF agents for fistulizing Crohn's disease. B Show 3 more https://web.pathway.md/diseases/recjc0smgZlZ1OMzP 3/4 6/23/23, 2:37 AM Anorectal fistula Pathway References 1. Andreas Ommer, Alexander Herold, Eugen Berg et al. German S3 guidelines: anal abscess and fistula (second revised version). Langenbecks Arch Surg. 2017 Mar;402 2 191 201. Open 2. Wolfgang B Gaertner, Pamela L Burgess, Jennifer S Davids et al. The American Society of Colon and Rectal Surgeons Clinical Practice Guidelines for the Management of Anorectal Abscess, Fistula-in-Ano, and Rectovaginal Fistula. Dis Colon Rectum. 2022 Aug 1;65 8 964 985. Open 3. Expert Panel on Gastrointestinal Imaging: Angela D. Levy, MD, Peter S. Liu et al. ACR Appropriateness Criteria Anorectal Disease. ACR. 2021. Open 4. Antonio Tarasconi, Gennaro Perrone, Justin Davies et al. Anorectal emergencies: WSES AAST guidelines. World J Emerg Surg. 2021 Sep 16;16 1 48. Open 5. Lichtenstein GR, Loftus EV, Isaacs KL et al. ACG Clinical Guideline: Management of Crohn's Disease in Adults. Am J Gastroenterol. 2018 Apr;113 4 481 517. Open 6. Vogel JD, Johnson EK, Morris AM et al. Clinical Practice Guideline for the Management of Anorectal Abscess, Fistula-in-Ano, and Rectovaginal Fistula. Dis Colon Rectum. 2016 Dec;59 12 1117 1133. Open https://web.pathway.md/diseases/recjc0smgZlZ1OMzP 4/4
Guideline sources The following summarized guidelines for the evaluation and management of anorectal varices are prepared by our editorial team based on guidelines from the World Society of Emergency Surgery (WSES/AAST 2021). 1 Guidelines 1. Diagnostic investigations History and physical examination: consider eliciting a focused medical history and performing a complete physical examination, including a DRE, to rule out other causes of lower gastrointestinal bleeding in patients with suspected bleeding anorectal varices. C Laboratory tests: Consider checking vital signs and obtaining Hgb, hematocrit and coagulation to evaluate the severity of bleeding in patients with suspected anorectal varices. C Consider obtaining blood typing and cross-matching in patients with severe anorectal bleeding. C Diagnostic imaging: consider obtaining EUS with/without color Doppler evaluation as a second- line diagnostic tool in patients with bleeding anorectal varices, especially for deep rectal varices or https://web.pathway.md/diseases/reccbBbny3Q8ooGZ3 1/3 6/23/23, 2:38 AM Anorectal varices Pathway when in doubt. C Show 2 more 2. Diagnostic procedures Lower gastrointestinal endoscopy: consider performing ano-proctoscopy or flexible sigmoidoscopy as first-line diagnostic tool in patients with suspected bleeding anorectal varices. C Show 2 more 3. Medical management General principles: consider ensuring multidisciplinary management for patients with bleeding anorectal varices, early involving the hepatology specialist team and focusing on optimal control of comorbid conditions. C Resuscitation: consider administering IV fluids, blood transfusion if necessary, correction of coagulopathy, and optimal medication for portal hypertension in patients with anorectal varices and mild bleeding. C Show 2 more Beta-blockers: Consider administering nonselective -adrenergic blockers for the prevention/prophylaxis of first and/or recurrent variceal bleeding in patients with anorectal varices. C Consider suspending -blockers temporarily in patients with acute bleeding. C Vasoactive agents: consider administering vasoactive drugs such as terlipressin or octreotide to reduce splanchnic blood flow and portal pressure in patients with bleeding anorectal varices. C Antibiotic prophylaxis: administer a short course of prophylactic antibiotics in patients with bleeding anorectal varices. B 4. Therapeutic procedures Local procedures: Consider performing any of the following local procedures in patients with bleeding anorectal varices to arrest bleeding in the first instance if possible: endoscopic variceal ligation endoscopic band ligation sclerotherapy endoscopic US-guided glue injection. C Angioembolization: Consider performing embolization via interventional radiological techniques for the short-term control of bleeding in patients with bleeding anorectal varices. C https://web.pathway.md/diseases/reccbBbny3Q8ooGZ3 2/3 6/23/23, 2:38 AM Anorectal varices Pathway Insufficient evidence regarding the superiority of one embolization technique over the others in patients with bleeding anorectal varices. I Transjugular intrahepatic portosystemic shunt: consider performing percutaneous TIPS, if not contraindicated, to decompress the portal venous system and to reduce the risk for rebleeding in patients with bleeding anorectal varices and severe portal hypertension. C Doppler-guided hemorrhoidal artery ligation: insufficient evidence to recommend for or against Doppler-guided hemorrhoidal artery ligation in patients with bleeding anorectal varices and failure of medical treatment, local and radiological procedures. I 5. Surgical interventions Stapled anopexy: insufficient evidence to recommend for or against stapled anopexy in patients with bleeding anorectal varices and failure of medical treatment, local and radiological procedures. I References 1. Antonio Tarasconi, Gennaro Perrone, Justin Davies et al. Anorectal emergencies: WSES AAST guidelines. World J Emerg Surg. 2021 Sep 16;16 1 48. Open https://web.pathway.md/diseases/reccbBbny3Q8ooGZ3 3/3
Guideline sources The following summarized guidelines for the evaluation and management of anorexia nervosa are prepared by our editorial team based on guidelines from the American Psychiatric Association (APA 2023), the The Scottish Intercollegiate Guidelines Network (SIGN 2022), the U.S. Preventive Services Task Force (USPSTF 2022), the Canadian Practice Guidelines (CPG 2021; 2020), the German Society for Psychiatry, Psychotherapy and Psychosomatics (DGPPN 2019), the American Academy of Child and Adolescent Psychiatry (AACAP 2015), the American Association of Family Physicians (AAFP 2015), the Royal Australian and New Zealand College of Psychiatrists (RANZCP 2014), and the American College of Gastroenterology (ACG 2013). 1 2 3 4 5 6 7 8 9 10 Calculator DSM 5 diagnostic criteria for an Guidelines https://web.pathway.md/diseases/recRtaTMHhaVTKJg8 1/8 6/23/23, 2:38 AM Anorexia nervosa Pathway 1. Screening and diagnosis Indications for screening: As per APA 2023 guidelines, screen for the presence of an eating disorder as part of an initial psychiatric evaluation. B As per SIGN 2022 guidelines: Consider screening for the presence of eating disorders as part of the routine review of patients with T1DM. C Elicit history of current or past history of eating disorders in females during pregnancy and postnatal period, recognizing the potential barriers for disclosure. A As per USPSTF 2022 guidelines, insufficient evidence to assess the balance of benefits and harms of screening for eating disorders in adolescents and adults. I As per AACAP 2015 guidelines, screen all pediatric and adolescent patients for eating disorders seen by a mental health clinician, followed by a comprehensive diagnostic evaluation (including laboratory tests and imaging studies as indicated) if positive. B As per ACG 2013 guidelines, consider assessing for the presence of eating disorders (including anorexia nervosa) when evaluating a patient for gastroparesis. B 2. Diagnostic investigations Initial evaluation: As per APA 2023 guidelines, assess the following during the initial evaluation of patients with a possible eating disorder: height and weight history (maximum and minimum weight, recent weight changes) presence of, patterns in, and changes in restrictive eating, food avoidance, binge eating, and other eating-related behaviors (rumination, regurgitation, chewing, and spitting) patterns and changes in food repertoire (breadth of food variety, narrowing or elimination of food groups) presence of, patterns in, and changes in compensatory and other weight control behaviors, including dietary restriction, compulsive or driven exercise, purging behaviors (laxative use, self-induced vomiting), and use of medication to manipulate weight percentage of time preoccupied with food, weight, and body shape prior treatment and response to treatment for an eating disorder psychosocial impairment secondary to eating or body image concerns or behaviors family history of eating disorders, other psychiatric illnesses, and other medical conditions (obesity, IBD, diabetes mellitus). B Show 2 more As per RANZCP 2014 guidelines: Obtain a detailed assessment of core symptoms including restriction methods, psychological symptoms related to fear of weight gain, weight loss, drive for thinness, and body image https://web.pathway.md/diseases/recRtaTMHhaVTKJg8 2/8 6/23/23, 2:38 AM Anorexia nervosa Pathway disturbance/dissatisfaction, establishing both severity and (where possible) duration of illness. B Perform a detailed physical examination and obtain medical and laboratory evaluations, thereby setting priorities for any specific medical interventions. B Laboratory tests: obtain a CBC and a comprehensive metabolic panel including electrolytes, liver enzymes, and renal function tests in the laboratory assessment of patients with a possible eating disorder. B Electrocardiogram: obtain an ECG in patients with a restrictive eating disorder, severe purging behavior, or taking medications known to prolong the QTc interval. B Screening for comobidities: As per APA 2023 guidelines, assess for co-occurring health conditions, including psychiatric disorders, in the initial psychiatric evaluation of patients with a possible eating disorder. B As per DGPPN 2019 guidelines, assess for comorbid conditions in patients with anorexia nervosa, and take these into consideration when making treatment decisions. B As per AAFP 2015 guidelines, assess for psychiatric comorbidities, including depression and suicide risk, anxiety disorders, and substance use disorders, in patients with an eating disorder. B As per RANZCP 2014 guidelines, obtain detailed evaluations for any comorbid psychiatric diagnoses. B 3. Medical management Multidisciplinary care: As per APA 2023 guidelines, provide a documented, comprehensive, culturally appropriate, and person-centered treatment plan incorporating medical, psychiatric, psychological, and nutritional expertise, commonly via a coordinated multidisciplinary team, in patients with an eating disorder. B As per AACAP 2015 guidelines, ensure a multidisciplinary team approach (developmentally aware, sensitive, and skilled in the care of pediatric and adolescent patients with eating disorders) for the treatment of eating disorders in young patients. B As per AAFP 2015 guidelines, ensure an interdisciplinary team approach, often including a family physician, a psychotherapist or psychiatrist, a dietitian, an eating disorder specialist, and school personnel, for the treatment of patients with anorexia nervosa. B Pharmacotherapy, general principles: As per SIGN 2022 guidelines: Monitor all low-weight patients, particularly closely when psychotropic drugs are prescribed. Consider obtaining a baseline ECG and further monitoring to alert about avoidable dangers. B https://web.pathway.md/diseases/recRtaTMHhaVTKJg8 3/8 6/23/23, 2:38 AM Anorexia nervosa Pathway Obtain careful monitoring of any medication selected for the symptomatic treatment of anorexia nervosa and comorbid conditions. B As per AACAP 2015 guidelines, reserve medications, including complementary and alternative medications, for comorbid conditions and refractory cases. B Pharmacotherapy, antipsychotics: As per SIGN 2022 guidelines, consider offering olanzapine to support recovery, but not as the sole treatment, in adult patients with anorexia nervosa. B As per CPG 2020 guidelines, consider offering olanzapine or aripiprazole with careful monitoring in certain pediatric and adolescent patients with anorexia nervosa. C As per DGPPN 2019 guidelines, do not use neuroleptics for the sole purpose of achieving weight gain in patients with anorexia nervosa. D Show 2 more As per AAFP 2015 guidelines, avoid using antipsychotics for the treatment of patients with anorexia nervosa. D As per RANZCP 2014 guidelines: Consider offering low-dose antipsychotics, such as olanzapine, to reduce anxiety and obsessive thinking. B Be cautious when prescribing psychotropic medications for severe comorbid conditions/symptoms until it is clear that such symptoms are not secondary to starvation. B Pharmacotherapy, antidepressants: As per DGPPN 2019 guidelines, do not use antidepressants for the sole purpose of achieving weight gain, both in the initial treatment and relapse prevention, in patients with anorexia nervosa. D As per RANZCP 2014 guidelines, be cautious when using antidepressants B Do not use SSRIs in the acute or maintenance stages of anorexia nervosa. A 4. Inpatient care Indications for hospitalization: As per CPG 2020 guidelines, provide the least intensive treatment environment (such as family- based treatment or day treatment versus lengthy hospitalizations) especially in pediatric and adolescent patients with anorexia nervosa requiring a first admission to hospital and/or with a duration of illness < 3 years. A As per DGPPN 2019 guidelines, ensure that inpatient treatment facilities used for anorexia nervosa are able to offer specialized multimodal treatment programs. B Show 2 more As per AACAP 2015 guidelines, consider offering psychiatric hospitalization, day programs, partial hospitalization programs, and residential programs for eating disorders in pediatric and https://web.pathway.md/diseases/recRtaTMHhaVTKJg8 4/8 6/23/23, 2:38 AM Anorexia nervosa Pathway adolescent patients only when outpatient interventions have been unsuccessful or are unavailable. C As per AAFP 2015 guidelines, assess medical stability and the need for hospitalization in the initial evaluation of patients with eating disorders. B As per RANZCP 2014 guidelines: Provide treatment in the least restrictive environment possible, usually in an outpatient setting. B Provide outpatient treatment as first-line therapy in adolescent patients withanorexia nervosa. A Reserve hospitalization for medical rescue and management of psychiatric risk. B Admit patients at high risk of life-threatening medical complications, extremely low weights, and other uncontrolled symptoms to the hospital. B 5. Nonpharmacologic interventions Psychotherapy: As per APA 2023 guidelines: Offer eating disorder-focused psychotherapy including normalizing eating and weight control behaviors, restoring weight, and addressing psychological aspects of the disorder (such as fear of weight gain and body image disturbance) in adult patients with anorexia nervosa. B Offer eating disorder-focused family-based treatment including caregiver education aimed at normalizing eating and weight control behaviors and restoring weight in adolescent and emerging adult patients with anorexia nervosa having an involved caregiver. B As per SIGN 2022 guidelines, offer enhanced CBT in patients with eating disorders. Offer other forms of CBT as first-line therapy in adult patients with anorexia nervosa. A Consider offering other therapeutic approaches, such as interpersonal psychotherapy, the Maudsley model, Specialist Supportive Clinical Management, or focal psychodynamic therapy, if CBT is ineffective, unsuitable, or unacceptable. B Show 3 more As per CPG 2020 guidelines: Offer family-based treatment in all pediatric and adolescent patients with anorexia nervosa, especially with a duration of illness < 3 years. A Consider offering multi-family therapy or CBT in pediatric and adolescent patients and adolescent-focused psychotherapy in adolescent patients with anorexia nervosa. C As per DGPPN 2019 guidelines: Address ambivalence and motivation to change as a central task in the treatment of patients with anorexia nervosa, as they are highly ambivalent towards change, and maintain it throughout the whole treatment process. B Offer the following evidence-based psychotherapies, administered by practitioners experienced with eating disorders, as first-line therapy for the outpatient treatment of patients with anorexia nervosa: https://web.pathway.md/diseases/recRtaTMHhaVTKJg8 5/8 6/23/23, 2:38 AM Anorexia nervosa Pathway family-based treatment in pediatric and adolescent patients focal psychodynamic therapy in adult patients enhanced CBT in adult patients Maudsley model in adult patients specialist supportive clinical management in adult patients. B As per AACAP 2015 guidelines, offer outpatient psychosocial interventions as the initial treatment of choice in pediatric and adolescent patients with an eating disorder. B As per AAFP 2015 guidelines, offer Maudsley model family-based treatment in adolescent patients with anorexia nervosa. B As per RANZCP 2014 guidelines, offer psychotherapy as the mainstay of treatment in patients with anorexia nervosa. Offer family-based therapies in younger (< 18 years old) patients living with families. Offer family-based therapy or alternate family therapy as the treatment of choice in most pediatric and adolescent patients with anorexia nervosa. (EBR I). A Show 2 more Technology-based interventions: As per SIGN 2022 guidelines, consider offering psychological assessments and treatment via videoconferencing as an adjunct or alternative to in-person sessions when there are barriers to accessing in-person sessions. C As per CPG 2021 guidelines, consider offering telehealth family-based treatment in pediatric and adolescent patients with anorexia nervosa. C Show 12 more Nutritional management: As per APA 2023 guidelines, set an individualized goal for weekly weight gain and target weight in patients with anorexia nervosa requiring nutritional rehabilitation and weight restoration. B As per SIGN 2022 guidelines: Provide refeeding to an optimized healthy weight (taking the patient's ethnicity and sex into consideration), both as a life-saving measure and also as an adjunct to achieve an optimized weight and reduce relapse, in patients with anorexia nervosa. A Set nutritional goals on a case-by-case basis after a holistic assessment of the patient's general physical and psychological condition, taking into account the patient's ethnicity, biological sex, genetics, personal dietary requirements, sensory sensitivities, and cultural beliefs, and guided by an experienced specialist eating disorder dietitian or suitably qualified alternative healthcare professional. B As per DGPPN 2019 guidelines: Do not provide an initial low caloric energy supply with a gradual increase for the sole purpose of safe weight gain (avoidance of refeeding syndrome) in patients with mild-to-moderate anorexia nervosa, provided that medical monitoring is ensured. D Individualize and obtain continuous monitoring of the energy supply for the expected weight gain, tailored to the treatment phase. B https://web.pathway.md/diseases/recRtaTMHhaVTKJg8 6/8 6/23/23, 2:38 AM Anorexia nervosa Pathway As per AAFP 2015 guidelines, set a minimum weight restoration target of 90% of the average weight expected for the patient's age, height, and sex in patients with anorexia nervosa. B As per RANZCP 2014 guidelines, provide refeeding and weight gain regimes to minimize the risk of refeeding syndrome, underfeeding, and other medical complications arising from increased nutritional intake. B Exercise programs: offer specialist-supervised exercise programs as part of a comprehensive management program including a psychoeducation component in patients with anorexia nervosa. A Show 3 more Yoga: advise practicing, yoga in addition to standard treatments, in medically stable young patients with anorexia nervosa. B 6. Specific circumstances Pregnant patients: elicit history of current or past history of eating disorders in females during pregnancy and postnatal period, recognizing the potential barriers for disclosure. A Show 13 more Patients with diabetes mellitus type 1: consider assessing for the presence of eating disorders as part of the routine review of patients with T1DM. C Show 3 more Patients with osteopenia/osteoporosis: offer weight restoration as part of a holistic program of treatment to improve bone mineral density in patients with low-weight anorexia, regardless of gender identity and age. A Show 4 more References 1. Phillipa Hay, David Chinn, David Forbes et al. Royal Australian and New Zealand College of Psychiatrists clinical practice guidelines for the treatment of eating disorders. Aust N Z J Psychiatry. 2014 Nov;48 11 977 1008. Open 2. Catherine Crone, Laura J Fochtmann, Evelyn Attia et al. The American Psychiatric Association Practice Guideline for the Treatment of Patients With Eating Disorders. Am J Psychiatry. 2023 Feb 1;180 2 167 171. Open 3. James Lock, Maria C La Via, American Academy of Child and Adolescent Psychiatry AACAP Committee on Quality Issues CQI . Practice parameter for the assessment and treatment of children and adolescents with eating disorders. J Am Acad Child Adolesc Psychiatry. 2015 May;54 5 412 25. Open 4. Scottish Intercollegiate Guidelines Network. SIGN 164 Eating disorders. SIGN. 2022 Aug. Open 5. Gaby Resmark, Stephan Herpertz, Beate Herpertz-Dahlmann et al. Treatment of Anorexia Nervosa-New Evidence-Based Guidelines. J Clin Med. 2019 Jan 29;8 2 153. Open https://web.pathway.md/diseases/recRtaTMHhaVTKJg8 7/8 6/23/23, 2:38 AM Anorexia nervosa Pathway 6. US Preventive Services Task Force, Karina W Davidson, Michael J Barry et al. Screening for Eating Disorders in Adolescents and Adults: US Preventive Services Task Force Recommendation Statement. JAMA. 2022 Mar 15;327 11 1061 1067. Open 7. Camilleri M, Parkman HP, Shafi MA et al. Clinical guideline: management of gastroparesis. Am J Gastroenterol. 2013 Jan;108 1 18 37. Open 8. Brian C Harrington, Michelle Jimerson, Christina Haxton et al. Initial Evaluation, Diagnosis, and Treatment of Anorexia Nervosa and Bulimia Nervosa. Am Fam Physician. 2015 Jan 1;91 1 46 52. Open 9. Jennifer Couturier, Leanna Isserlin, Mark Norris et al. Canadian practice guidelines for the treatment of children and adolescents with eating disorders. J Eat Disord. 2020 Feb 1;8 4. Open 10. Jennifer Couturier, Danielle Pellegrini, Catherine Miller et al. The COVID 19 pandemic and eating disorders in children, adolescents, and emerging adults: virtual care recommendations from the Canadian consensus panel during COVID 19 and beyond. J Eat Disord. 2021 Apr 16;9 1 46. Open 11. American Geriatrics Society. Choosing Wisely AGS recommendations. Choosing Wisely. 2015. Open 12. Brian C Harrington, Michelle Jimerson, Christina Haxton et al. Initial evaluation, diagnosis, and treatment of anorexia nervosa and bulimia nervosa. Am Fam Physician. 2015 Jan 1;91 1 46 52. Open 13. Jennifer Couzin-Frankel. Rethinking anorexia. Science. 2020 Apr 10;368 6487 124 127. Open 14. American Psychiatric Association. Treatment of patients with eating disorders,third edition. American Psychiatric Association. Am J Psychiatry. 2006 Jul;163 7 Suppl):4 54. Open 15. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, Text Revision DSM 5 TR ). APA. 2022. Open https://web.pathway.md/diseases/recRtaTMHhaVTKJg8 8/8
Guideline sources The following summarized guidelines for the evaluation and management of anterior cruciate ligament injury are prepared by our editorial team based on guidelines from the European Bone and Joint Infection Society (EBJIS 2023), the American Academy of Orthopaedic Surgeons (AAOS 2022), and the American College of Radiology (ACR 2020). 1 2 3 Guidelines 1. Diagnostic investigations History and physical examination: elicit a relevant history and perform a focused musculoskeletal examination of the lower extremities in the evaluation for an ACL injury. A Knee radiography: obtain knee radiography as initial imaging for the evaluation of a fall or acute twisting trauma to the knee in 5 years old patients with focal tenderness, effusion, or inability to bear weight. B Show 3 more Knee magnetic resonance imaging: https://web.pathway.md/diseases/recV6EtuRC9W22XJi 1/4 6/23/23, 2:38 AM Anterior cruciate ligament injury Pathway Knee magnetic resonance imaging: Obtain knee MRI as the next imaging modality for the evaluation of suspected occult knee fractures or internal derangement after a fall or acute twisting trauma to the knee in adult or pediatric patients (whether skeletally mature or immature) with negative radiographs. B Obtain knee MRI or knee CT angiography as the next imaging modality for the evaluation of suspected additional bone or soft tissue injury after a fall or acute twisting trauma to the knee in 5 years old patients with a radiographic diagnosis of tibial plateau fracture. B Knee computed tomography: Obtain knee radiography or CT angiography of the lower extremity as initial imaging for the evaluation of significant trauma to the knee (such as motor vehicle accident or knee dislocation) in 5 years old patients. B Obtain knee CT angiography or knee MRI as the next imaging modality for the evaluation of suspected additional bone or soft tissue injury after a fall or acute twisting trauma to the knee in 5 years old patients with a radiographic diagnosis of tibial plateau fracture. B 2. Nonpharmacologic interventions Functional knee bracing: do not use functional knee braces routinely in patients after isolated primary ACL reconstruction, as they confer no clinical benefit. D 3. Therapeutic procedures Knee joint aspiration: consider performing knee joint aspiration of painful, tense effusions after a knee injury. E 4. Surgical interventions ACL reconstruction (indications): consider performing ACL reconstruction in order to lower the risk of future meniscus pathology or procedures, particularly in younger and/or more active patients, and to improve long-term pain and function. C ACL reconstruction (timing): prefer early reconstruction when surgical treatment is indicated for an acute isolated ACL tear because the risk of additional cartilage and meniscal injury starts to increase within 3 months. A ACL reconstruction (choice of technique): consider using either single or double bundle techniques in patients undergoing intra-articular ACL reconstruction due to similar outcomes. B ACL reconstruction (choice of graft): consider using an autograft over allograft for articular ACL reconstruction to improve patient outcomes and decrease articular ACL graft failure rate, particularly in young and/or active patients. B Show 2 more https://web.pathway.md/diseases/recV6EtuRC9W22XJi 2/4 6/23/23, 2:38 AM Anterior cruciate ligament injury Pathway MCL reconstruction: offer nonoperative treatment of the medial collateral ligament tears injury in patients with combined ACL and medial collateral ligament tears. Consider offering surgical treatment in selected cases. B Meniscal repair: consider offering a meniscal-preserving strategy to optimize joint health and function in patients with ACL tear and meniscal tear. C 5. Specific circumstances Patients with postoperative septic arthritis: suspect septic arthritis after reconstruction of the ACL in the presence of a delayed ROM recovery, increased warmth or swelling, wound drainage and arthrofibrosis, unusual pain, and systemic symptoms, such as fever and malaise. Confirm the diagnosis in the presence of purulent discharge/aspirate, sinus tract communication with the joint, and intra-articular pus. B Show 4 more 6. Preventative measures Education programs: consider offering training programs designed to prevent injury to reduce the risk of primary ACL injuries in athletes participating in high-risk sports. C Prophylactic knee bracing: avoid offering prophylactic bracing for the prevention of ACL injuries. D 7. Follow-up and surveillance Return to sport activities: consider obtaining functional evaluation, such as the hop test, as one factor to determine return to sport after ACL reconstruction. C Likelihood Ratios Pertinent positives The following findings increase the probability of anterior cruciate ligament injury in adults. 1 1 Finding LR+ Value Positive pivot shift test 20.3 Positive Lachman test 12.4 Positive anterior drawer test 3.7 Pertinent negatives The following findings decrease the probability of anterior cruciate ligament injury in adults. 1 1 Finding LR- Value https://web.pathway.md/diseases/recV6EtuRC9W22XJi 3/4 6/23/23, 2:38 AM Anterior cruciate ligament injury Pathway Negative Lachman test Negative pivot shift test 0.1 0.4 Negative anterior drawer test 0.6 References 1. The American Academy of Orthopaedic Surgeons Board of Directors. Management of Anterior Cruciate Ligament Injuries. AAOS. 2022 Aug 22. Open 2. Expert Panel on Musculoskeletal Imaging, Mihra S Taljanovic, Eric Y Chang et al. ACR Appropriateness Criteria Acute Trauma to the Knee. J Am Coll Radiol. 2020 May;17 5S S12 S25. Open 3. Christen Ravn, Jeroen Neyt, Natividad Benito et al. Guideline for management of septic arthritis in native joints SANJO . J Bone Jt Infect. 2023 Jan 12;8 1 29 37. Open https://web.pathway.md/diseases/recV6EtuRC9W22XJi 4/4
Guideline sources The following summarized guidelines for the evaluation and management of anti-GBM glomerulonephritis are prepared by our editorial team based on guidelines from the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021), the American Society for Apheresis (ASFA 2019), and the Japanese Society of Nephrology (JSN 2016). 1 2 3 4 5 5 6 Definition Anti-GBM disease, previously called Goodpasture's disease, is a systemic autoimmune disorder resulting in progressive crescentic glomerulonephritis and, frequently, diffuse pulmonary alveolar hemorrhage. 4 Epidemiology Anti-GBM disease is caused by an autoimmune response against type IV collagen molecules in the kidneys and lungs triggered by environmental factors in genetically predisposed individuals. 5 Disease course https://web.pathway.md/diseases/reciQgKzcH7k8DcC6 1/5 6/23/23, 2:38 AM Anti-glomerular basement membrane disease Pathway Autoantibodies against type IV collagen molecules in the kidneys and lungs result in anti-GBM disease, which causes clinical manifestations of RPGN with concurrent lung hemorrhage. Disease progression may lead to dialysis-dependent kidney failure, end-stage renal disease necessitating kidney transplantation, and respiratory failure. 5 Prognosis and risk of recurrence Anti-GBM disease is associated with an in-hospital mortality rate of 7.7 per 100 admissions. 6 Guidelines 1. Diagnostic investigations Initial evaluation: obtain prompt evaluation for anti-GBM disease in all patients with suspected RPGN. B Show 3 more 2. Diagnostic procedures Kidney biopsy: As per KDIGO 2021 guidelines, perform kidney biopsy with immunofluorescence at presentation or within 24 hours in patients with suspected RPGN. B As per JSN 2016 guidelines, perform kidney biopsy to aid in determining the treatment strategy for RPGN. B 3. Medical management General principles: Initiate treatment without delay in patients with suspected anti-GBM disease, even before the diagnosis is confirmed. B Consider offering a conservative approach in patients without alveolar hemorrhage but with oliguria and/or advanced kidney failure requiring dialysis, especially in the presence of a very high proportion of crescents (85-100%) on kidney biopsy. C Immunosuppressive therapy: As per KDIGO 2021 guidelines: Initiate immunosuppression with cyclophosphamide and corticosteroid plus plasmapheresis in all patients with anti-GBM glomerulonephritis, except patients receiving dialysis at presentation, having 100% crescents or > 50% global glomerulosclerosis in an adequate biopsy sample, and not having a pulmonary hemorrhage. B Administer cyclophosphamide for 2-3 months and corticosteroids for about 6 months. B https://web.pathway.md/diseases/reciQgKzcH7k8DcC6 2/5 6/23/23, 2:38 AM Anti-glomerular basement membrane disease Pathway As per JSN 2016 guidelines, initiate high-dose corticosteroids with immunosuppressive agents as initial therapy in patients with anti-GBM disease presenting with RPGN. Initiate corticosteroids alone, in combination with plasmapheresis, only if the use of immunosuppressive agents is not desirable. B Show 5 more Intravenous immunoglobulin: consider initiating IVIG in patients with concurrent complications such as severe infections to avoid the standard therapy with high-dose corticosteroids and immunosuppressants. C Maintenance therapy: As per KDIGO 2021 guidelines: Do not initiate maintenance therapy for anti-GBM. D Initiate maintenance therapy in anti-GBM- and ANCA-positive patients as for patients with ANCA-associated vasculitis. B As per JSN 2016 guidelines, consider initiating low-dose corticosteroids as maintenance therapy to improve renal function and survival in patients with anti-GBM disease presenting with RPGN. C Show 2 more Antithrombotic therapy: consider initiating anticoagulant or antiplatelet therapy in the absence of hemorrhagic lesions. C 4. Therapeutic procedures Plasmapheresis: As per KDIGO 2021 guidelines: Initiate plasmapheresis along with immunosuppressive therapy in all patients with anti-GBM glomerulonephritis, except patients receiving dialysis at presentation, having 100% crescents or > 50% global glomerulosclerosis in an adequate biopsy sample, and not having a pulmonary hemorrhage. B Perform plasmapheresis until anti-GBM titers are no longer detectable. B As per JSN 2016 guidelines, initiate plasmapheresis in addition to immunosuppressive therapy for improved renal function and survival in patients with anti-GBM antibody-positive RPGN. B 5. Surgical interventions Kidney transplantation: postpone kidney transplantation in patients with kidney failure due to anti-GBM disease until anti-GBM antibodies remain undetectable for 6 months. B 6. Preventative measures https://web.pathway.md/diseases/reciQgKzcH7k8DcC6 3/5 6/23/23, 2:38 AM Anti-glomerular basement membrane disease Pathway Prophylaxis for Pneumocystis jirovecii pneumonia: initiate TMP-sulfamethoxazole to improve life prognosis in patients with RPGN receiving with immunosuppressive therapy. A 7. Follow-up and surveillance Assessment of treatment response: As per KDIGO 2021 guidelines, monitor kidney function, pulmonary infiltrates, anti-GBM antibody titers, and blood counts. Modify treatment accordingly. B As per JSN 2016 guidelines, consider measuring anti-GBM antibody titers to monitor the disease activity and recurrence. Management of refractory disease: consider initiating rituximab in patients with refractory anti- GBM disease. C Likelihood Ratios Pertinent positives The following findings increase the probability of anti-GBM glomerulonephritis in adults. -1 Finding LR+ Value Positive anti-GBM antibodies 2.82 Pertinent negatives The following findings decrease the probability of anti-GBM glomerulonephritis in adults. -1 Finding LR- Value Negative anti-GBM antibodies 0.69 References 1. Kidney Disease: Improving Global Outcomes KDIGO Glomerular Diseases Work Group. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021 Oct;100 4S S1 S276. Open 2. Yoshihiro Arimura, Eri Muso, Shoichi Fujimoto et al. Evidence-based clinical practice guidelines for rapidly progressive glomerulonephritis 2014. Clin Exp Nephrol. 2016 Jun;20 3 322 41. Open 3. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 4. Andrew L Chan, Samuel Louie, Kevin O Leslie et al. Cutting edge issues in Goodpasture's disease. 2011 Oct;41 2 151 62.2011 Oct;41 2 151 62. Open https://web.pathway.md/diseases/reciQgKzcH7k8DcC6 4/5 6/23/23, 2:38 AM Anti-glomerular basement membrane disease Pathway 5. Stephen P McAdoo, Charles D Pusey. Anti-Glomerular Basement Membrane Disease. 2017 Jul 7;12 7 1162 1172.2017 Jul 7;12 7 1162 1172. Open 6. Wisit Kaewput, Charat Thongprayoon, Boonphiphop Boonpheng et al. Inpatient Burden and Mortality of Goodpasture's Syndrome in the United States: Nationwide Inpatient Sample 2003 2014. 2020 Feb 6;9 2 455.2020 Feb 6;9 2 455. Open 7. KDIGO. Chapter 14 Anti-glomerular basement membrane antibody glomerulonephritis. Kidney Int Suppl 2011 . 2012 Jun;2 2 240 242. Open https://web.pathway.md/diseases/reciQgKzcH7k8DcC6 5/5
Guideline sources The following summarized guidelines for the evaluation and management of antibody-mediated pure red cell aplasia (antibody-mediated PRCA) are prepared by our editorial team based on guidelines from the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2012). 1 2 2 2 3 4 Definition Antibody-mediated PRCA is an immunological disorder seen in patients undergoing treatment with ESAs, which is characterized by the production of antibodies that inhibit the erythropoietic activity of endogenous EPO, leading to a rapidly progressive anemia. 2 Epidemiology Subcutaneous administration of recombinant EPO may lead to production of EPO-neutralizing antibodies, causing a rapid decline in blood Hgb levels and the number of circulating reticulocytes. 3 Pathophysiology In patients taking ESAs, the incidence of antibody-mediated PRCA is estimated at 14-35.8 cases per 100,000 patient-years. 2 Disease course Ther disorder is characterized by the sudden onset of severe and progressive normocytic, normochromic anemia, reticulocytopenia, and absence of erythroid precursor cells in the bone https://web.pathway.md/diseases/rec8088ZxPol53oG4 1/2 6/23/23, 2:38 AM Antibody-mediated pure red cell aplasia Pathway marrow. 2 Prognosis and risk of recurrence Treatment with pegisetanide is associated with a reduction in transfusion requirement, and freedom from transfusion in most patients after 3 months of treatment. 4 Guidelines 1. Screening and diagnosis Clinical presentation: Evaluate for possible antibody-mediated pure red-cell aplasia in patients receiving ESAs for > 8 weeks in whom: hgb concentration falls suddenly and rapidly (at a rate of 0.5 to 1.0 g/dL per week, or requirement for transfusions at a rate of approximately 1 to 2 per week) CBC shows normal platelet and white cell counts absolute reticulocyte count is < 10, 000/mL. 2. Medical management Cessation of ESA therapy: discontinue erythropoiesis-stimulated agents in patients who develop antibody-mediated PRCA. B Peginesatide therapy: administer peginesatide for the treatment of antibody-mediated PRCA. B References 1. KDIGO Executive Committee. KDIGO Clinical Practice Guideline for Anemia in Chronic Kidney Disease. KDIGO. 2012 Aug. Open 2. Macdougall IC, Casadevall N, Locatelli F et al. Incidence of erythropoietin antibody-mediated pure red cell aplasia: the Prospective Immunogenicity Surveillance Registry PRIMS . Nephrol Dial Transplant. 2015 Mar;30 3 451 60. Open 3. Casadevall N. What is antibody-mediated pure red cell aplasia PRCA ?. Nephrol Dial Transplant. 2005 May;20 Suppl 4:iv3 8. Open 4. Macdougall IC, Rossert J, Casadevall N et al. A peptide-based erythropoietin-receptor agonist for pure red-cell aplasia. N Engl J Med. 2009 Nov 5;361 19 1848 55. Open https://web.pathway.md/diseases/rec8088ZxPol53oG4 2/2
Guideline sources The following summarized guidelines for the management of antidromic atrioventricular reentrant tachycardia are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC 2020). 1 Calculator NYHA functional classification f Guidelines 1. Medical management Acute management: Consider administer IV formulations of the following medications for acute management of hemodynamically stable patients with antidromic atrioventricular reentrant tachycardia if vagal maneuvers and adenosine fail: ibutilide procainamide flecainide https://web.pathway.md/diseases/recyPWdx5A3BvHCJF 1/3 6/23/23, 2:39 AM Antidromic atrioventricular reentrant tachycardia Pathway profanenone. C Consider administering IV amiodarone for acute management of hemodynamically stable patients with refractory antidromic atrioventricular reentrant tachycardia. C Ongoing management: Consider initiating the following medications for the management of patients with antidromic atrioventricular reentrant tachycardia and without signs of pre-excitation on resting ECG, if ablation is not desirable or feasible: -blockers nondihydropyridine CCBs (verapamil or diltiazem) in the absence of HFrEF. (Class IIa, Level B) consider initiating the following medications for the management of patients with antidromic atrioventricular reentrant tachycardia and without ischemic or structural heart disease, if ablation is not desirable or feasible: propafenone flecainide. C Do not use the following medications in patients with pre-excited AF: -blockers nondihydropyridine CCBs (verapamil or diltiazem) digoxin amiodarone. D 2. Nonpharmacologic interventions Vagal maneuvers: perform vagal maneuvers, preferably in the supine position with leg elevation, for acute management of hemodynamically stable patients with antidromic atrioventricular reentrant tachycardia. B 3. Therapeutic procedures Synchronized electrical cardioversion: perform synchronized direct current cardioversion for acute management of hemodynamically unstable patients with antidromic atrioventricular reentrant tachycardia. B Show 2 more Catheter ablation: perform catheter ablation of accessory pathways in patients with symptomatic, recurrent antidromic atrioventricular reentrant tachycardia. B References https://web.pathway.md/diseases/recyPWdx5A3BvHCJF 2/3 6/23/23, 2:39 AM Antidromic atrioventricular reentrant tachycardia Pathway 1. Josep Brugada, Demosthenes G Katritsis, Elena Arbelo et al. 2019 ESC Guidelines for the management of patients with supraventricular tachycardia The Task Force for the management of patients with supraventricular tachycardia of the European Society of Cardiology ESC . Eur Heart J. 2020 Feb 1;41 5 655 720. Open https://web.pathway.md/diseases/recyPWdx5A3BvHCJF 3/3
Guideline sources The following summarized guidelines for the management of antimicrobial-resistant Gram-negative infections are prepared by our editorial team based on guidelines from the Infectious Diseases Society of America (IDSA 2023) and the European Society for Microbiology and Infectious Diseases (ESCMID 2021; 2019). 1 2 3 Guidelines 1. Medical management General principles: use optimal antibiotic dosing schemes with attention to side effects, especially with the old antibiotics (polymyxins and aminoglycosides). E Show 3 more Management of Enterobacterales infections (extended-spectrum beta-lactamase- producing): administer nitrofurantoin or TMP-sulfamethoxazole as preferred options for the treatment of uncomplicated cystitis caused by extended-spectrum -lactamase-producing Enterobacterales. Administer ciprofloxacin, levofloxacin, or carbapenems as alternative options. Do not use the latter if nitrofurantoin and TMP-sulfamethoxazole are active. Administer single- dose aminoglycosides or oral fosfomycin (for E. coli only) as another alternative option. E Show 6 more Management of Enterobacterales infections (AmpC beta-lactamase-producing): recognize that E. cloacae complex, K. aerogenes, and C. freundii are the most common Enterobacterales at moderate-to-high risk for clinically significant AmpC production. E Show 7 more https://web.pathway.md/diseases/recbVX0szAPj9Tev5 1/3 6/23/23, 2:39 AM Antimicrobial-resistant Gram-negative infections Pathway Show 7 more Management of Enterobacterales infections (third-generation cephalosporin-resistant): administer a carbapenem (imipenem or meropenem) as targeted therapy for the treatment of bloodstream infections and severe infections caused by third-generation cephalosporin-resistant Enterobacterales. B Show 8 more Management of Enterobacterales infections, carbapenem-resistant: As per IDSA 2023 guidelines, administer nitrofurantoin, TMP-sulfamethoxazole, ciprofloxacin, or levofloxacin as preferred options for the treatment of uncomplicated cystitis caused by carbapenem-resistant Enterobacterales, although the likelihood of susceptibility to any of these agents is low. Administer a single dose of an aminoglycoside, oral fosfomycin (for E. coli only), colistin, ceftazidime-avibactam, meropenem-vaborbactam, imipenem-cilastatin-relebactam, or cefiderocol as alternative options. E Show 9 more As per ESCMID 2021 guidelines, consider administering cefiderocol for the treatment of severe infections caused by carbapenem-resistant Enterobacterales carrying metallo- -lactamases and/or resistant to all other antibiotics, including ceftazidime-avibactam and meropenem- vaborbactam. C Show 8 more Management of Pseudomonas aeruginosa infections (carbapenem-resistant): consider administering ceftolozane-tazobactam, if active in vitro, for the treatment of severe infections caused by carbapenem-resistant P. aeruginosa with difficult-to-treat resistance. C Show 4 more Management of Pseudomonas aeruginosa infections (with difficult-to-treat resistance): administer traditional non-carbapenem -lactam agents (such as piperacillin-tazobactam, ceftazidime, cefepime, aztreonam) over carbapenems if P. aeruginosa isolates test susceptible to them. Consider administerimg a traditional agent as high-dose extended-infusion therapy for the treatment of infections caused by P. aeruginosa isolates not susceptible to any carbapenem agent but susceptible to traditional -lactams, and repeat antimicrobial susceptibility testing. Consider administering a novel -lactam agent testing susceptible (such as ceftolozane-tazobactam, ceftazidime-avibactam, imipenem-cilastatin-relebactam) in critically ill patients or patients with poor source control with P. aeruginosa isolates resistant to carbapenems but susceptible to traditional -lactams. E Show 7 more Management of Acinetobacter baumannii infections, carbapenem-resistant: As per IDSA 2023 guidelines, consider administering high-dose ampicillin-sulbactam (total daily dose of 6-9 g of the sulbactam component) combined with at least one other agent for the treatment of carbapenem-resistant A. baumannii infections. E Show 8 more As per ESCMID 2021 guidelines, consider administering ampicillin-sulbactam for the treatment of hospital-acquired pneumonia/ventilator-associated pneumonia caused by carbapenem- https://web.pathway.md/diseases/recbVX0szAPj9Tev5 2/3 6/23/23, 2:39 AM Antimicrobial-resistant Gram-negative infections Pathway resistant A. baumannii susceptible to sulbactam. C Show 5 more Management of Stenotrophomonas maltophilia infections: Consider administering any of the following regimens for the treatment of S. maltophilia infections: two of the following agents: TMP-sulfamethoxazole, minocycline/tigecycline, cefiderocol, or levofloxacin combination of ceftazidime-avibactam and aztreonam in case of evident significant clinical instability or observed intolerance to or inactivity of other agents. E Show 5 more 2. Preventative measures Decolonization of multidrug-resistant Gram-negative bacteria carriers: do not offer routine decolonization in third-generation cephalosporin-resistant or carbapenem-resistant Enterobacterales carriers. D References 1. Mical Paul, Elena Carrara, Pilar Retamar et al. European Society of clinical microbiology and infectious diseases ESCMID guidelines for the treatment of infections caused by Multidrug-resistant Gram-negative bacilli (endorsed by ESICM European Society of intensive care Medicine). Clin Microbiol Infect. 2021 Dec 16;S1198 743X 21 00679 0. Open 2. Pranita D. Tamma, Samuel L. Aitken, Robert A. Bonomo et al. IDSA 2023 Guidance on the Treatment of Antimicrobial Resistant Gram-Negative Infections. IDSA. 2023 Jun 7. Open 3. E Tacconelli, F Mazzaferri, A M de Smet et al. ESCMID EUCIC clinical guidelines on decolonization of multidrug-resistant Gram-negative bacteria carriers. Clin Microbiol Infect. 2019 Jul;25 7 807 817. Open 4. Pranita D. Tamma, Samuel L. Aitken, Robert A. Bonomo et al. Infectious Diseases Society of America Guidance on the Treatment of Extended-Spectrum -lactamase Producing Enterobacterales ESBL E , Carbapenem-Resistant Enterobacterales CRE , and Pseudomonas aeruginosa with Difficult-to-Treat Resistance DTR P. aeruginosa). IDSA. 2022 Mar 7. Open 5. Pranita D. Tamma, Samuel L. Aitken, Robert A. Bonomo et al. Infectious Diseases Society of America Guidance on the Treatment of AmpC -lactamase- Producing Enterobacterales, Carbapenem-Resistant Acinetobacter baumannii, and Stenotrophomonas maltophilia Infections. IDSA. 2022 Mar 31. Open https://web.pathway.md/diseases/recbVX0szAPj9Tev5 3/3
Guideline sources The following summarized guidelines for the evaluation and management of antiphospholipid syndrome (APS) are prepared by our editorial team based on guidelines from the European League Against Rheumatism (EULAR 2022; 2019), the European Society for Vascular Surgery (ESVS 2021), the American College of Chest Physicians (ACCP 2021), the American College of Rheumatology (ACR 2020), the International Society on Thrombosis and Haemostasis (ISTH 2020), the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2017), the European Association for the Study of the Liver (EASL 2016), the American College of Obstetricians and Gynecologists (ACOG 2012), and the British Society for Haematology (BSH 2012). 1 2 3 4 5 6 7 8 9 10 11 12 13 13 14 14 Definition APS is a systemic autoimmune disease characterized by thrombotic or obstetrical events in the presence of antiphospholipid antibodies. 12 Epidemiology APS is caused by autoantibodies targeting phospholipid-bound proteins, mainly -2-glycoprotein I. 14 https://web.pathway.md/diseases/recoC2V41a1ihCnHc 1/9 6/23/23, 2:39 AM Antiphospholipid syndrome Pathway Pathophysiology The prevalence of antiphospholipid antibodies in the general population is estimated at 1-5%. In the United States, the estimated incidence and prevalence of APS are 5 cases per 100,000 person- years and 40-50 persons per 100,000 populations, respectively. 13 Disease course The interaction of antiphopholipid autoantibodies with phospholipid-binding plasma proteins ( -2- glycoprotein I, prothrombin, thrombomodulin, kininogens, antithrombin III, annexins, protein C, and protein S) results in activation of monocytes, endothelial cells, platelets, and the complement system, inducing a prothrombotic state that leads to inflammation, vasculopathy, venous/arterial thrombosis, and obstetric complications. 14 Prognosis and risk of recurrence APS is associated with morbidity related to stroke (13%), myocardial infarction (11%), deep vein thrombosis (9.5%), and pregnancy (6%). 13 Calculator Calculator Pathway Classification criteria for antipho Classification criteria for catastr Antiphosp Investigatio Guidelines 1. Screening and diagnosis Indications for screening: as per BSH 2012 guidelines, screen women with 3 pregnancy losses before 10 weeks gestation for antiphospholipid antibodies. B Show 3 more 2. Classification and risk stratification Risk stratification: as per EULAR 2019 guidelines, stratify patients with APS into high-risk, medium-risk, and low-risk phenotypes: Situation Guidance Multiple antiphospholipid antibodies High-risk Lupus anticoagulant or persistently high antiphospholipid antibody titers History of thrombotic and/or obstetric APS https://web.pathway.md/diseases/recoC2V41a1ihCnHc 2/9 6/23/23, 2:39 AM Antiphospholipid syndrome Pathway Coexistence of other systemic autoimmune diseases such as SLE And presence of traditional cardiovascular risk factors Anticardiolipin antibody of IgG and/or IgM isotype in serum or plasma present in titers > 40 IgG phospholipid units or > 40 IgM phospholipid units, or >the 99th percentile, measured by a standardised ELISA Medium-risk Anti- -2-glycoprotein I antibody of IgG and/or IgM isotype in serum or plasma in titer > the 99th percentile, measured by a standardised ELISA Isolated anticardiolipin antibody or anti- -2- glycoprotein I antibodies at low-medium titers, particularly if transiently positive. B Low-risk Classification: diagnose antiphospholipid antibody syndrome in patients meeting laboratory criteria on two or more occasions at least 12 weeks apart, in addition to either thrombotic criteria or obstetrical criteria. 3. Diagnostic investigations Initial laboratory testing: obtain lupus anticoagulant testing and testing for IgG antibodies to -2- glycoprotein I (either IgG anticardiolipin ELISA or an IgG anti- -2-glycoprotein I ELISA). An anticardiolipin ELISA may detect antibodies to other phospholipid binding proteins as well as anti- -2-glycoprotein I. B Confirmatory testing: perform a confirmatory test (e.g., using a high phospholipid concentration, platelet neutralizing reagent or a lupus-anticoagulant insensitive reagent) to definitively establish the presence of lupus anticoagulant. A Non-IgG antibodies: Avoid measuring anticardiolipin IgM antibodies in patients with thrombosis, as it does not provide additional information that will impact management. D Avoid measuring anticardiolipin IgA antibodies. D Screening for portal vein thrombosis: consider screening for extrahepatic portal vein obstruction in patients with APS. C 4. Medical management General principles: https://web.pathway.md/diseases/recoC2V41a1ihCnHc 3/9 6/23/23, 2:39 AM Antiphospholipid syndrome Pathway Screen for and manage cardiovascular risk factors appropriately in patients with antiphospholipid antibodies, including smoking cessation counseling; management of hypertension, dyslipidemia and diabetes; and promotion of regular physical activity. B Screen for and manage risk factors for venous thrombosis, and use LMWH in high-risk situations such as surgery, hospitalization, prolonged immobilisation and the puerperium. B Management of arterial thrombosis: As per EULAR 2019 guidelines, administer a vitamin K antagonist, rather than low-dose aspirin only, in patients with definite APS and first arterial thrombosis. B Show 3 more Landmark trials: TRAPS In high-risk patients with thrombotic antiphospholipid syndrome, rivaroxaban was not noninferior to warfarin with respect to vascular death, thromboembolic events, or major bleeding. Pengo V et al. Blood. 2018 Sep 27. As per BSH 2012 guidelines, administer antithrombotic therapy consisting of either antiplatelet therapy or warfarin in patients with APS and ischemic stroke with a single positive antiphospholipid antibody test result. B Show 2 more Management of venous thromboembolism: As per ESVS 2021 guidelines, do not use direct OACs in patients with deep vein thrombosis and APS who are triple positive or have a history of arterial or small vessel thrombosis. D As per EULAR 2019 guidelines, initiate a vitamin K antagonist with a target INR 2-3 in patients with definite APS and first venous thrombosis. B Show 2 more Landmark trials: TRAPS In high-risk patients with thrombotic antiphospholipid syndrome, rivaroxaban was not noninferior to warfarin with respect to vascular death, thromboembolic events, or major bleeding. Pengo V et al. Blood. 2018 Sep 27. Duration of treatment for venous thrombosis: As per EULAR 2019 guidelines, aim for long-term anticoagulation in patients with definite APS and first venous thrombosis, and in patients with unprovoked first venous thrombosis. B Show 2 more https://web.pathway.md/diseases/recoC2V41a1ihCnHc 4/9 6/23/23, 2:39 AM Antiphospholipid syndrome Pathway As per BSH 2012 guidelines: Consider long-term anticoagulation in patients with unprovoked venous thrombosis. Complete a finite duration of treatment for patients with venous thrombosis in the presence of a transient risk factor. Adjustment of vitamin K antagonists: As per ACCP 2021 guidelines, consider adjusting the dose of vitamin K antagonists (target INR 2.5) over initiating direct OAC therapy during the treatment phase in patients with confirmed APS. C As per ESVS 2021 guidelines, consider titrating vitamin K antagonists to maintain a target INR of 2-3 in patients with deep vein thrombosis and triple-positive APS. C As per EULAR 2019 guidelines, target an INR of 2-3 or 3-4 taking into account the patient's risks of bleeding and recurrent thrombosis. B As per BSH 2012 guidelines, target an INR of 2-3 in patients with APS on vitamin K antagonist therapy. A Show 2 more Management of recurrent thrombosis: as per EULAR 2019 guidelines, consider one of the following options in patients with recurrent arterial thrombosis despite adequate treatment with vitamin K antagonist, after evaluating for other potential cause: targeting an INR 3-4 adding low-dose aspirin to current therapy switching to LMWH. C Direct oral anticoagulants: As per ISTH 2020 guidelines, initiate vitamin K antagonists instead of direct OACs for the treatment of thrombotic APS in patients with any of the following (high-risk patients): triple positivity arterial thrombosis small vessel thrombosis or organ involvement heart valve disease according to Sydney criteria. E Show 4 more As per EULAR 2019 guidelines: Do not use direct OACs in patients with definite APS and arterial events due to the high risk of recurrent thrombosis. D Consider initiating direct OACs in patients not able to achieve target INRs despite good adherence to vitamin K antagonists or patients with contraindications to vitamin K antagonists (allergy or intolerance to vitamin K antagonists). C 5. Specific circumstances Considerations for contraception: https://web.pathway.md/diseases/recoC2V41a1ihCnHc 5/9 6/23/23, 2:39 AM Antiphospholipid syndrome Pathway As per ACR 2020 guidelines: Do not use combined estrogen-progestin contraceptives in women with positive antiphospholipid antibodies because estrogen increases the risk of thromboembolism. D Offer intrauterine devices (levonorgestrel or copper) or progestin-only pills in patients with positive antiphospholipid antibodies. A As per ACOG 2012 guidelines, do not use estrogen-containing contraceptives in patients with APS. D Considerations for hormone replacement therapy: offer HRT, if desired and in the absence of contraindications, in postmenopausal patients with positive antiphospholipid antibodies with severe vasomotor symptoms. A Show 4 more Patients undergoing ART procedures: consider administering empiric prophylactic anticoagulation in antiphospholipid antibody-positive patients undergoing IVF because of the concern regarding further increased risk of potentially life-threatening thrombosis from elevated estrogen levels during ovarian stimulation. E Show 3 more Pregnant patients: As per ACR 2020 guidelines, do not initiate prophylactic therapy for the prevention of pregnancy loss in asymptomatic antiphospholipid antibody-positive patients (without pregnancy complications or history of thrombosis). D Show 10 more As per EULAR 2019 guidelines, consider low-dose aspirin in pregnant women with a high-risk antiphospholipid antibodies profile but no history of thrombosis or pregnancy complications. C Show 5 more As per BSH 2012 guidelines, initiate low-dose aspirin and heparin at prophylactic dosages throughout pregnancy in women with recurrent pregnancy loss ( 3). B Show 2 more Pediatric patients (evaluation): Obtain the following tests in pediatric patients with suspected APS: lupus anticoagulant anticardiolipin IgG and IgM anti- -2-glycoprotein-I IgG and IgM. B Show 3 more Pediatric patients (management): consider initiating antiplatelet agents in addition to hydroxychloroquine for primary prevention of thrombosis in patients with childhood-onset SLE and antiphospholipid antibodies. C Show 5 more Patients with catastrophic antiphospholipid syndrome: https://web.pathway.md/diseases/recoC2V41a1ihCnHc 6/9 6/23/23, 2:39 AM Antiphospholipid syndrome Pathway As per EULAR 2019 guidelines, initiate antimicrobial therapy promptly in all antiphospholipid antibodies-positive individuals with infection, in order to decrease the risk of catastrophic APS. B Show 3 more As per BSH 2012 guidelines, insufficient evidence to make a recommendation for the treatment of catastrophic APS. A combination of treatments including anticoagulation with heparin/warfarin and immunomodulatory therapies, including plasmapheresis, IVIGs, corticosteroids, and rituximab, have been used. 6. Patient education General counseling: counsel patients on treatment adherence, INR monitoring in patients treated with vitamin K antagonist, use of perioperative bridging therapy with LMWH in patients on OACs, oral contraceptive use, pregnancy and postpartum considerations, postmenopausal hormone therapy, and general lifestyle recommendations. B 7. Preventative measures Primary thromboprophylaxis: As per EULAR 2022 guidelines: Initiate prophylactic treatment with low-dose aspirin (75-100 mg daily) in asymptomatic antiphospholipid antibody carriers with a high-risk profile with or without traditional risk factors. B Initiate prophylactic treatment with low-dose aspirin in patients with SLE with a high-risk antiphospholipid antibody profile and no history of thrombosis or pregnancy complications. B Consider initiating low-dose aspirin in patients with a low-risk antiphospholipid antibody profile. B As per EULAR 2019 guidelines, initiate prophylactic treatment with low-dose aspirin in asymptomatic antiphospholipid antibodies carriers not fulfilling any vascular or obstetric classification criteria, with a high-risk antiphospholipid antibodies profile with or without traditional risk factors. B Show 3 more As per BSH 2012 guidelines, avoid primary thromboprophylaxis in patients incidentally found to have antiphospholipid antibodies. D References 1. Committee on Practice Bulletins Obstetrics, American College of Obstetricians and Gynecologists. Practice Bulletin No. 132 Antiphospholipid syndrome. Obstet Gynecol. 2012 Dec;120 6 1514 21. Open https://web.pathway.md/diseases/recoC2V41a1ihCnHc 7/9 6/23/23, 2:39 AM Antiphospholipid syndrome Pathway 2. Stavros K Kakkos, Manjit Gohel, Niels Baekgaard et al. Editor's Choice European Society for Vascular Surgery ESVS 2021 Clinical Practice Guidelines on the Management of Venous Thrombosis. Eur J Vasc Endovasc Surg. 2021 Jan;61 1 9 82. Open 3. Lisa R Sammaritano, Bonnie L Bermas, Eliza E Chakravarty et al. 2020 American College of Rheumatology Guideline for the Management of Reproductive Health in Rheumatic and Musculoskeletal Diseases. Arthritis Care Res Hoboken). 2020 Apr;72 4 461 488. Open 4. Tektonidou MG, Andreoli L, Limper M et al. EULAR recommendations for the management of antiphospholipid syndrome in adults. Ann Rheum Dis. 2019 May 15. Open 5. Keeling D, Mackie I, Moore GW et al. Guidelines on the investigation and management of antiphospholipid syndrome. Br J Haematol. 2012 Apr;157 1 47 58. Open 6. Miyakis S, Lockshin MD, Atsumi T et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome APS . J Thromb Haemost. 2006 Feb;4 2 295 306. Open 7. George C Drosos, Daisy Vedder, Eline Houben et al. EULAR recommendations for cardiovascular risk management in rheumatic and musculoskeletal diseases, including systemic lupus erythematosus and antiphospholipid syndrome. Ann Rheum Dis. 2022 Jun;81 6 768 779. Open 8. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol. 2016 Jan;64 1 179 202. Open 9. Noortje Groot, Nienke de Graeff, Tadej Avcin et al. European evidence-based recommendations for diagnosis and treatment of paediatric antiphospholipid syndrome: the SHARE initiative. Ann Rheum Dis. 2017 Oct;76 10 1637 1641. Open 10. St phane Zuily, Hannah Cohen, David Isenberg et al. Use of direct oral anticoagulants in patients with thrombotic antiphospholipid syndrome: Guidance from the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. J Thromb Haemost. 2020 Sep;18 9 2126 2137. Open 11. Scott M Stevens, Scott C Woller, Lisa Baumann Kreuziger et al. Antithrombotic Therapy for VTE Disease: Second Update of the CHEST Guideline and Expert Panel Report. Chest. 2021 Dec;160 6):e545-e608. Open 12. David Garcia, Doruk Erkan. Diagnosis and Management of the Antiphospholipid Syndrome. N Engl J Med. 2018 May 24;378 21 2010 2021. Open 13. Ricard Cervera. Antiphospholipid syndrome. 2017 Mar;151 Suppl 1 S43 S47.2017 Mar;151 Suppl 1 S43 S47. Open 14. Negrini S, Pappalardo F, Murdaca G et al. The antiphospholipid syndrome: from pathophysiology to treatment. Clin Exp Med. 2017 Aug;17 3 257 267. Open 15. Lisa R Sammaritano. Antiphospholipid syndrome. Best Pract Res Clin Rheumatol. 2020 Feb;34 1 101463. Open 16. Joz lio Freire de Carvalho, Sandra Gofinet Pasoto, Simone Appenzeller. Seizures in primary antiphospholipid syndrome: the relevance of smoking to stroke. Clin Dev Immunol. 2012;2012 981519. Open 17. Cervera R. Antiphospholipid syndrome. Thromb Res. 2017 Mar;151 Suppl 1 S43 S47. Open https://web.pathway.md/diseases/recoC2V41a1ihCnHc 8/9 6/23/23, 2:39 AM Antiphospholipid syndrome Pathway 18. Garcia D, Erkan D. Diagnosis and Management of the Antiphospholipid Syndrome. N Engl J Med. 2018 May 24;378 21 2010 2021. Open 19. Ruiz-Irastorza G, Cuadrado MJ, Ruiz-Arruza I et al. Evidence-based recommendations for the prevention and long-term management of thrombosis in antiphospholipid antibody-positive patients: report of a task force at the 13th International Congress on antiphospholipid antibodies. Lupus. 2011 Feb;20 2 206 18. Open 20. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 21. M Bj rck, M Koelemay, S Acosta et al. Editor's Choice Management of the Diseases of Mesenteric Arteries and Veins: Clinical Practice Guidelines of the European Society of Vascular Surgery ESVS . Eur J Vasc Endovasc Surg. 2017 Apr;53 4 460 510. Open https://web.pathway.md/diseases/recoC2V41a1ihCnHc 9/9
Guideline sources The following summarized guidelines for the evaluation and management of anxiety and depression in patients with cancer are prepared by our editorial team based on guidelines from the American Society of Clinical Oncology (ASCO 2023) and the European Society of Medical Oncology (ESMO 2023). 1 2 Calculator Calculator Calculator Generalized anxiety disorder-7 s Hamilton anxiety scale HAM A Hamilton d Guidelines 1. Diagnostic investigations Assessment tools: obtain regular screening and assessment for anxiety in all patients with cancer in all phases of illness. B Show 5 more https://web.pathway.md/diseases/recGxzcZtGxH6C2b0 1/3 6/23/23, 2:39 AM Anxiety and depression in patients with cancer Pathway 2. Medical management Pharmacotherapy: As per ASCO 2023 guidelines: Consider offering pharmacotherapy for depression or anxiety in patients without access to first-line treatment, expressing a preference for pharmacotherapy, or not improving following first-line psychological or behavioral management. C Consider offering pharmacotherapy for depression in patients with a history of treatment response to medications, severe symptoms, or accompanying psychotic features. C As per ESMO 2023 guidelines: Offer antidepressants for symptomatic relief in patients with cancer. B Recognize that SSRIs have few significant drug-drug interactions, with the notable exception of tamoxifen metabolism, which is least affected by escitalopram, sertraline, and venlafaxine. Avoid offering paroxetine in patients taking tamoxifen because of negative clinical outcomes. B 3. Nonpharmacologic interventions General principles: As per ASCO 2023 guidelines, use a stepped-care model (selecting the most effective and least resource-intensive intervention based on symptom severity) when deciding on treatment for anxiety and/or depression, as well as taking into account the following variables to inform the choice of treatment approach: psychiatric history (prior diagnoses with or without treatment) history of substance use prior mental health treatment response functional abilities and/or limitations related to self-care, usual activities, and/or mobility recurrent or advanced cancer presence of other chronic diseases (such as cardiac disease) member of socially and/or economically marginalized group (such as Black race, low socioeconomic status). B Show 2 more As per ESMO 2023 guidelines, offer a combination of psychotherapeutic and psychopharmacological modalities for the treatment of anxiety and depression in patients with cancer. A Psychosocial interventions: As per ASCO 2023 guidelines, derive psychological and psychosocial interventions provided by mental health practitioners from manualized, empirically supported treatments. Recognize that manuals for evidence-based treatments specify content, structure, delivery mode, session https://web.pathway.md/diseases/recGxzcZtGxH6C2b0 2/3 6/23/23, 2:39 AM Anxiety and depression in patients with cancer Pathway number, treatment duration, and related topics. Tailor treatment according to linguistic, cultural, and socioecological contexts. B Show 4 more As per ESMO 2023 guidelines: Consider offering the following therapies in patients with anxiety and depressive symptoms: CBT mindfulness-based therapy psychoeducation supportive-expressive therapies. B Offer meaning-centered therapy and dignity therapy in specific cancer settings (such as end- of-life). A 4. Patient education General counseling: offer information regarding depression and anxiety in all patients with cancer and any patient-identified caregiver, family member, or trusted confidant. Offer resources, such as the providers' contact information for further evaluation and treatment within or external to the facility whenever available. B Show 2 more 5. Follow-up and surveillance Assessment of treatment response: assess treatment response regularly (including pre- treatment, 4 weeks, 8 weeks, and end of treatment) by a mental health professional in patients referred to and receiving psychological treatment. B Show 2 more References 1. Barbara L Andersen, Christina Lacchetti, Kimlin Ashing et al. Management of Anxiety and Depression in Adult Survivors of Cancer: ASCO Guideline Update. J Clin Oncol. 2023 Apr 19;JCO2300293. Open 2. L Grassi, R Caruso, M B Riba et al. Anxiety and depression in adult cancer patients: ESMO Clinical Practice Guideline. ESMO Open. 2023 Apr;8 2 101155. Epub 2023 Mar 14. Open https://web.pathway.md/diseases/recGxzcZtGxH6C2b0 3/3
Guideline sources The following summarized guidelines for the evaluation and management of aortic intramural hematoma are prepared by our editorial team based on guidelines from the American Heart Association (AHA/ACC 2022), the American Association for Thoracic Surgery (AATS 2021), the European Society of Cardiology (ESC 2014), and the Society for Cardiovascular Angiography and Interventions (SCAI/STS/SVM/AATS/SCA/AHA/ACR/ACC/ASA/SIR 2010). 1 2 3 4 Guidelines 1. Diagnostic investigations Diagnostic imaging: consider obtaining MRI for characterizing acute intramural hematomas when CT is equivocal. C 2. Medical management Expectant management: As per AHA 2022 guidelines: Consider offering an initial or expectant approach of medical management in selected patients with uncomplicated acute type A intramural hematoma at increased operative risk without https://web.pathway.md/diseases/rec6nnOUwSSuHR8XM 1/3 6/23/23, 2:39 AM Aortic intramural hematoma Pathway high-risk imaging features. C Offer medical therapy as the initial management strategy in patients with uncomplicated acute type B intramural hematoma. B As per ESC 2014 guidelines: Administer initial medical therapy under careful surveillance in patients with type B aortic intramural hematoma. B Administer medical therapy including pain relief and BP control in all patients with aortic intramural hematoma. B 3. Surgical interventions Open surgery: As per AHA 2022 guidelines, perform urgent repair in patients with complicated acute type A or B aortic intramural hematoma. B Show 4 more As per ESC 2014 guidelines: Perform urgent surgery in patients with type A aortic intramural hematoma. B Consider performing surgery in patients with complicated type B aortic intramural hematoma. C Thoracic endovascular aortic repair: As per AHA 2022 guidelines, consider performing endovascular repair by surgeons with endovascular expertise in patients with type B intramural hematoma with favorable anatomy requiring repair of the distal aortic arch or descending thoracic aorta (zones 2-5). C As per ESC 2014 guidelines, consider performing TEVAR in patients with complicated type B aortic intramural hematoma. C 4. Specific circumstances Patients with penetrating atherosclerotic ulcer: Perform urgent repair in patients with penetrating atherosclerotic ulcer of the ascending aorta with associated intramural hematoma. B Consider performing urgent repair in patients with penetrating atherosclerotic ulcer of the aortic arch, descending thoracic aorta, C or abdominal aorta with associated intramural hematoma. C 5. Follow-up and surveillance Surveillance imaging: As per AHA 2022 guidelines: https://web.pathway.md/diseases/rec6nnOUwSSuHR8XM 2/3 6/23/23, 2:39 AM Aortic intramural hematoma Pathway Obtain surveillance CT or MRI after 1 month, 6 months, and 12 months and then, if stable, annually thereafter in patients experienced acute aortic dissection and intramural hematoma managed with medical therapy alone. B Obtain surveillance CT or MRI after 1 month, 6 months, and 12 months and then, if stable, annually thereafter in patients experienced acute aortic dissection and intramural hematoma treated with either open or endovascular aortic repair and having a residual aortic disease. B As per ESC 2014 guidelines, obtain repetitive imaging (CT or MRI) in patients with uncomplicated type B aortic intramural hematoma. B As per AHA 2010 guidelines, consider obtaining surveillance imaging using the schedules established for aortic dissection in patients with aortic intramural hematoma. C References 1. Erbel R, Aboyans V, Boileau C et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology ESC . Eur Heart J. 2014 Nov 1;35 41 2873 926. Open 2. S Christopher Malaisrie, Wilson Y Szeto, Monika Halas et al. 2021 The American Association for Thoracic Surgery expert consensus document: Surgical treatment of acute type A aortic dissection. J Thorac Cardiovasc Surg. 2021 Sep;162 3 735 758.e2. Open 3. Hiratzka LF, Bakris GL, Beckman JA et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation. 2010 Apr 6;121 13):e266 369. Open 4. Eric M Isselbacher, Ourania Preventza, James Hamilton Black rd et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. Circulation. 2022 Nov 2. Open https://web.pathway.md/diseases/rec6nnOUwSSuHR8XM 3/3
Guideline sources The following summarized guidelines for the evaluation and management of aortic regurgitation are prepared by our editorial team based on guidelines from the American Heart Association (AHA/ACC 2021), the European Society of Cardiology (ESC/EACTS 2021), and the Society of Thoracic Surgeons (STS 2013). 1 2 3 4 5 6 6 7 Definition AR, also known as aortic insufficiency, is a structural heart disease characterized by diastolic flow of blood across the aortic valve, from the aorta into the left ventricle. 4 Epidemiology AR may be due to malfunction of the valve leaflets themselves, dilatation of the aortic root and annulus, or a combination of these factors. 6 Pathophysiology The prevalence of AR in the United States is estimated at 500 persons per 100,000 population. 5 Disease course https://web.pathway.md/diseases/rectZZjnwx7zhIc49 1/5 6/23/23, 2:40 AM Aortic regurgitation Pathway Chronic AR causes volume overload of the left ventricle, with ventricular dilation and systolic dysfunction, and clinical symptoms of HF. Acute AR results in acute HF, and can progress to cardiogenic shock due to an uncompensated decrease in effective stroke volume. 6 Prognosis and risk of recurrence Moderate or severe AR is associated with significant morbidity and mortality. In highly symptomatic patients (NYHA functional class 3-4), annual mortality rates of approximately 25% have been reported. 7 Calculator Calculator Calculator Cardiac Anesthesia Risk Evaluati NYHA functional classification f Revised C Guidelines 1. Diagnostic investigations Transthoracic echocardiogram: Obtain TTE for assessment of the cause and severity of regurgitation, LV size and systolic function, prognosis, and timing of valve intervention in patients with signs and symptoms of AR. B Obtain TTE for evaluation of the presence and severity of AR in patients with bicuspid aortic valve or with known dilation of the aortic sinuses or ascending aorta. B Further imaging: obtain cardiac magnetic resonance, TEE or perform cardiac catheterization for assessment of LV systolic function, systolic and diastolic volumes, aortic size and AR severity in patients with moderate or severe AR and suboptimal TTE images or a discrepancy between clinical and TTE findings. B 2. Medical management Pharmacotherapy: Initiate treatment of hypertension (systolic BP > 140 mmHg) in asymptomatic patients with chronic AR. B Initiate guideline-directed medical therapy for reduced LV ejection fraction with ACEIs, ARBs and/or sacubitril/valsartan in patients with severe AR having symptoms and/or LV systolic dysfunction (stages C2-D) but a prohibitive surgical risk. B 3. Surgical interventions https://web.pathway.md/diseases/rectZZjnwx7zhIc49 2/5 6/23/23, 2:40 AM Aortic regurgitation Pathway Indications for surgery, symptomatic patients: As per AHA/ACC 2021 guidelines, perform aortic valve surgery in symptomatic patients with severe AR (stage D) regardless of LV systolic function. B Show 3 more As per EACTS/ESC 2021 guidelines, perform surgery in symptomatic patients regardless of LV function. B Show 2 more As per STS 2013 guidelines, perform aortic valve replacement or repair in symptomatic patients with severe AR irrespective of LV systolic function. B Show 2 more Indications for surgery, asymptomatic patients: As per AHA/ACC 2021 guidelines, perform aortic valve surgery in asymptomatic patients with chronic severe AR and LV systolic dysfunction (LV ejection fraction 55%) (stage C2), if no other cause for systolic dysfunction is identified. B Show 2 more As per EACTS/ESC 2021 guidelines, perform surgery in asymptomatic patients with LV end- systolic diameter > 50 mm or LV end-systolic diameter > 25 mm/m 2 BSA (in patients with small body size) or resting LV ejection fraction 50%. B Show 3 more As per STS 2013 guidelines, perform aortic valve replacement or repair in asymptomatic patients with chronic severe AR and LV systolic dysfunction (LV ejection fraction 50%) at rest. B Show 3 more 4. Specific circumstances Pregnant patients (before pregnancy): obtain clinical evaluation and TTE in female patients with suspected valve disease contemplating pregnancy. B Show 5 more Pregnant patients (during pregnancy): obtain monitoring of pregnant patients with severe valve disease (stages C-D) in a tertiary-care center with a dedicated heart valve team of cardiologists, surgeons, anesthesiologists, and maternal-fetal medicine obstetricians with expertise in the management of high-risk cardiac conditions during pregnancy. B Show 5 more Patients with aortic stenosis: Perform aortic valve replacement in symptomatic patients with combined aortic stenosis and AR and a peak transvalvular jet velocity of at least 4.0 m/s or a mean transvalvular gradient of at least 40 mmHg. B Perform SAVR in asymptomatic patients with combined aortic stenosis and AR having a jet velocity of 4.0 m/s with an LV ejection fraction < 50%. B https://web.pathway.md/diseases/rectZZjnwx7zhIc49 3/5 6/23/23, 2:40 AM Aortic regurgitation Pathway Patients with coronary artery disease: perform CABG in patients with a primary indication for aortic/mitral valve surgery and coronary artery diameter stenosis 70%. B Show 3 more Patients undergoing non-cardiac surgery: obtain preoperative echocardiography in patients with clinically suspected moderate or greater degrees of valvular regurgitation undergoing non- cardiac surgery. B Show 2 more 5. Follow-up and surveillance Post-procedural follow-up: obtain baseline port-procedural TTE followed by periodic monitoring with TTE depending on type of intervention, length of time after intervention, ventricular function, and concurrent cardiac conditions in asymptomatic patients with any type of valve intervention. B Likelihood Ratios Pertinent positives The following findings increase the probability of aortic regurgitation in adults. -1 Finding LR+ Value Increased volume of murmur during transient arterial occlusion 8.4 (1.3-81) Presence of AR murmur (grade 3) 4.5 (1.6-14) Pertinent negatives The following findings decrease the probability of aortic regurgitation in adults. -1 Finding LR- Value Absence of AR murmur (grade < 1) 0.0 (0-0.9) volume of murmur during transient arterial occlusion not increased 0.3 (0.1-0.8) References 1. Catherine M Otto, Rick A Nishimura, Robert O Bonow et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Thorac Cardiovasc Surg. 2021 Aug;162 2):e183-e353. Open 2. Svensson LG, Adams DH, Bonow RO et al. Aortic valve and ascending aorta guidelines for management and quality measures. Ann Thorac Surg. 2013 Jun;95 6 Suppl):S1 66. Open https://web.pathway.md/diseases/rectZZjnwx7zhIc49 4/5 6/23/23, 2:40 AM Aortic regurgitation Pathway 3. Alec Vahanian, Friedhelm Beyersdorf, Fabien Praz et al. 2021 ESC/EACTS Guidelines for the management of valvular heart disease: Developed by the Task Force for the management of valvular heart disease of the European Society of Cardiology ESC and the European Association for Cardio-Thoracic Surgery EACTS . Eur Heart J. 2021;ehab395. Open 4. Akinseye OA, Pathak A, Ibebuogu UN. Aortic Valve Regurgitation: A Comprehensive Review. Curr Probl Cardiol. 2018 Aug;43 8 315 334. Open 5. Nkomo VT, Gardin JM, Skelton TN et al. Burden of valvular heart diseases: a population-based study. Lancet. 2006 Sep 16;368 9540 1005 11. Open 6. Maurer G. Aortic regurgitation. Heart. 2006 Jul;92 7 994 1000. Open 7. Dujardin KS, Enriquez-Sarano M, Schaff HV et al. Mortality and morbidity of aortic regurgitation in clinical practice. A long-term follow-up study. Circulation. 1999 Apr 13;99 14 1851 7. Open 8. Nishimura RA, Otto CM, Bonow RO et al. 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014 Jun 10;129 23 2440 92. Open 9. Nishimura RA, Otto CM, Bonow RO et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014 Jun 10;63 22):e57 185. Open 10. Nishimura RA, Otto CM, Bonow RO et al. 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2017 Jun 20;135 25):e1159-e1195. Open 11. Baumgartner H, Falk V, Bax JJ et al. 2017 ESC/EACTS Guidelines for the management of valvular heart disease. Eur Heart J. 2017 Sep 21;38 36 2739 2791. Open 12. Niteesh K. Choudhry, MD, Edward E. Etchells et al. Does This Patient Have Aortic Regurgitation?. JAMA. 1999;281 23 2231 2238. Open https://web.pathway.md/diseases/rectZZjnwx7zhIc49 5/5
Guideline sources The following summarized guidelines for the evaluation and management of aortic stenosis (AS) are prepared by our editorial team based on guidelines from the American Heart Association (AHA/ACC 2021) and the European Society of Cardiology (ESC/EACTS 2021). 1 2 3 3 3 3 4 5 6 7 Definition AS is a chronic fibrocalcific disease that results in narrowing of the aortic valve. 3 Epidemiology AS is caused by the interplay of structural (e.g. bicuspid aortic valve), hereditary (e.g. genetic polymorphisms of lipoprotein A gene), and acquired (e.g. progressive age-related calcification, rheumatic valve disease) factors. Turbulent flow through a stenotic aortic valve causes endothelial damage, with subsequent sub-endothelial inflammation and accumulation of oxidized lipoproteins, and leads to deposition of calcium on the valve with progression of stenosis. 3 6 Pathophysiology https://web.pathway.md/diseases/recPKP0FKP30zAZyO 1/8 6/23/23, 2:40 AM Aortic stenosis Pathway Pathophysiology The prevalence of AS in the United States is estimated at 400 persons per 100,000 population. The incidence of AS-related admissions in the United States is estimated at 9.5-26 cases per 100,000 patient-years. 4 5 Disease course AS results in obstruction to the ejection of blood the left ventricle, with resultant signs and symptoms of left-sided HF. 3 Prognosis and risk of recurrence The overall survival of elderly patients with severe AS is 3 years from the onset of symptoms. In patients with severe, symptomatic AS who are at prohibitive risk for aortic valve replacement, the 1- year and 5-year mortality rates are 50% and >90%, respectively. 3 7 Calculator Calculator Calculator Cardiac Anesthesia Risk Evaluati NYHA functional classification f Revised C Guidelines 1. Diagnostic investigations Transthoracic echocardiogram: Obtain TTE for accurate diagnosis of the cause of AS, assessment of hemodynamic severity, measurement of LV size and systolic function, and determination of prognosis and timing of valve intervention in patients with signs or symptoms of AS. A Consider calculating the ratio of the outflow tract to aortic velocity to further define severity in patients with suspected low-flow, low-gradient severe AS with normal or reduced LV ejection fraction (stages D2-D3). C Dobutamine stress testing: consider obtaining low-dose dobutamine stress testing with echocardiographic or invasive hemodynamic measurements to further define severity and assess contractile reserve in patients with suspected low-flow, low-gradient severe AS with reduced LV ejection fraction (stage D2). C Exercise stress testing: Consider obtaining exercise testing to assess physiological changes with exercise and to confirm the absence of symptoms in asymptomatic patients with severe AS (stage C1). C Do not obtain exercise testing in symptomatic patients with severe AS (stage D1, aortic velocity 4.0 m/s or mean pressure gradient 40 mmHg) because of the risk of severe hemodynamic compromise. D https://web.pathway.md/diseases/recPKP0FKP30zAZyO 2/8 6/23/23, 2:40 AM Aortic stenosis Pathway Cardiac computed tomography: consider obtaining CT for the measurement of aortic valve calcium score to further define severity in patients with suspected low-flow, low-gradient severe AS with normal or reduced LV ejection fraction (stages D2 and D3). C 2. Diagnostic procedures Coronary angiography: Obtain coronary angiography before valve surgery in patients with severe VHD and any of the following: history of CVD suspected myocardial ischemia LV systolic dysfunction age > 40 for males postmenopause 1 cardiovascular risk factors. B Consider obtaining coronary CT angiography as an alternative to coronary angiography before valve surgery in patients with severe VHD and low probability of coronary artery disease. C 3. Medical management Antihypertensive therapy: ensure optimization of BP control before measurement of AS severity by TTE, TEE, cardiac catheterization, or cardiac magnetic resonance in patients with suspected low-flow, low-gradient severe AS with normal LV ejection fraction (stage D3). B Show 2 more Statin therapy: Initiate statins for primary and secondary prevention of atherosclerosis on the basis of standard risk scores in all patients with calcific AS. A Do not use statins for the prevention of hemodynamic progression of AS in patients with calcific AS (stages B and C). D Landmark trials: SALTIRE In patients with calcific aortic stenosis, treatment with atorvastatin, as compared with placebo, did not reduce the progression of aortic stenosis, as assessed by the change in aortic-jet velocity and aortic-valve calcium score. Serum LDL cholesterol decreased significantly in the atorvastatin group. Cowell SJ et al. N Engl J Med. 2005 Jun 9. https://web.pathway.md/diseases/recPKP0FKP30zAZyO 3/8 6/23/23, 2:40 AM Aortic stenosis Pathway 4. Therapeutic procedures Percutaneous aortic balloon dilation: As per AHA 2021 guidelines, consider performing percutaneous aortic balloon dilation as a bridge to SAVR or TAVI in critically ill patients with severe AS. C As per ESC 2021 guidelines, consider performing balloon aortic valvotomy as a bridge to SAVR or TAVI in hemodynamically unstable patients and (if feasible) in patients with severe AS requiring urgent high-risk non-cardiac surgery. C 5. Surgical interventions Aortic valve replacement (risk assessment): calculate individual risks for specific surgical and/or transcatheter procedures, using on-line tools when available, in patients with valvular heard disease when an intervention is contemplated, and discuss those risks before the procedure as a part of a shared decision-making process. B Aortic valve replacement, indications: As per AHA/ACC 2021 guidelines, perform aortic valve replacement in adult patients with severe high-gradient AS (stag D1) and symptoms of exertional dyspnea, HF, angina, syncope, or presyncope by history or on exercise testing. A Show 10 more As per EACTS/ESC 2021 guidelines, perform intervention in symptomatic patients with severe, high-gradient AS (mean gradient 40 mmHg, peak velocity 4.0 m/s, and valve area 1.0 cm (or 0.6 cm /m ). B Show 9 more Aortic valve replacement, choice of approach: As per AHA/ACC 2021 guidelines, perform SAVR in symptomatic and asymptomatic patients with severe AS and any indication for aortic valve replacement aged < 65 years or having a life expectancy > 20 years. A Show 6 more As per EACTS/ESC 2021 guidelines, perform aortic valve interventions in heart valve centers declaring their local expertise and outcomes data, having active interventional cardiology and cardiac surgical programs on site, and a structured collaborative heart team approach. B Show 5 more Landmark trials: PARTNER 2 In intermediate risk patients with severe aortic stenosis, TAVR was noninferior to surgery with respect to death from all causes or disabling stroke at 2 years. Leon MB et al. N Engl J Med. 2016 Apr 28. https://web.pathway.md/diseases/recPKP0FKP30zAZyO 4/8 6/23/23, 2:40 AM Aortic stenosis Pathway Aortic valve replacement (choice of valves): choose a prosthetic valve based on a shared decision-making process accounting for the patient's values and preferences and including discussion of the indications for and risks of anticoagulant therapy and the potential need for and risks associated with valve reintervention in patients with an indication for aortic valve replacement. B Show 5 more 6. Specific circumstances Pregnant patients: consider performing valve intervention before pregnancy in asymptomatic female patients with severe AS (aortic velocity 4.0 m/s or mean pressure gradient 40 mmHg, stage C) considering pregnancy. C Show 2 more Patients with atrial fibrillation: As per AHA 2021 guidelines, initiate a non-vitamin K OAC as an alternative to vitamin K antagonists in patients with AF and native valve heart disease or in patients received a bioprosthetic valve > 3 months ago, based on the patient's CHA2DS2-VASc score. A Show 2 more As per ESC 2021 guidelines, initiate direct OACs over vitamin K antagonists for stroke prevention in patients with AS and AF eligible for oral anticoagulation. A Show 2 more Patients with coronary artery disease: As per AHA 2021 guidelines, consider performing SAVR and CABG over TAVI and PCI in patients with significant AS and coronary artery disease (luminal reduction > 70% diameter, fractional flow reserve < 0.8, instantaneous wave-free ratio < 0.89) consisting of complex bifurcation left main and/or multivessel coronary artery disease with a SYNTAX score > 33. C As per ESC 2021 guidelines, perform CABGing in patients with a primary indication for aortic valve surgery and coronary artery diameter stenosis 70%. B Show 2 more Patients undergoing cardiac surgery for other indications: As per AHA 2021 guidelines: Consider performing aortic valve replacement in patients with moderate AS (stage B) undergoing cardiac surgery for other indications. C Perform aortic valve replacement in asymptomatic patients with severe AS (stage C1) undergoing cardiac surgery for other indications. B As per ESC 2021 guidelines: Perform SAVR in patients with severe AS undergoing CABGing or surgical intervention on the ascending aorta or another valve. B Consider performing SAVR in patients with moderate AS undergoing CABGing or surgical intervention on the ascending aorta or another valve after heart team discussion. C https://web.pathway.md/diseases/recPKP0FKP30zAZyO 5/8 6/23/23, 2:40 AM Aortic stenosis Pathway Patients undergoing non-cardiac surgery: obtain preoperative echocardiography in patients with clinically suspected moderate or greater degrees of valvular stenosis undergoing non-cardiac surgery. B Show 2 more 7. Preventative measures Prevention of infective endocarditis: Consider administering antibiotic prophylaxis before dental procedures (involving manipulation of gingival tissue, manipulation of the periapical region of teeth, or perforation of the oral mucosa) in patients with VHD having any of the following: prosthetic cardiac valves, including transcatheter-implanted prostheses and homografts prosthetic material used for cardiac valve repair, such as annuloplasty rings, chords, or clips previous infective endocarditis unrepaired cyanotic congenital heart disease or repaired congenital heart disease, with residual shunts or valvular regurgitation at the site of or adjacent to the site of a prosthetic patch or prosthetic device cardiac transplant with valve regurgitation attributable to a structurally abnormal valve. C Do not administer antibiotic prophylaxis for non-dental procedures (such as TEE, EGD, colonoscopy, or cystoscopy) in the absence of active infection in patients with VHD at high risk of infective endocarditis. D Secondary prevention of rheumatic fever: administer secondary prevention of rheumatic fever in patients with rheumatic heart disease. B 8. Follow-up and surveillance Indications for referral: Evaluate patients with severe VHD by a multidisciplinary heart valve team when intervention is considered. B Consider consulting with or referring to a primary or comprehensive heart valve center when discussing treatment options for: asymptomatic patients with severe VHD patients likely to benefit from valve repair versus valve replacement patients with multiple comorbidities considered for valve intervention. C Post-intervention serial imaging: obtain a baseline postprocedural TTE in asymptomatic patients with any type of valve intervention followed by periodic monitoring with TTE, depending on type of intervention, length of time after intervention, ventricular function, and concurrent cardiac conditions. B https://web.pathway.md/diseases/recPKP0FKP30zAZyO 6/8 6/23/23, 2:40 AM Aortic stenosis Pathway Post-intervention palliative care: offer palliative care after shared decision-making, including discussion of patient preferences and values, in symptomatic patients with severe AS if predicted post-TAVI or post-SAVR survival is < 12 months or if minimal improvement in quality of life is expected. B Likelihood Ratios Pertinent positives The following findings increase the probability of aortic stenosis in adults. -1 Finding LR+ Value Delay in carotid pulse upstroke 9.2 (3.4-24) Presence of systolic murmur radiating to right carotid 8.1 (4-16) Decreased second heart sound 7.5 (3.2-17) Presence of systolic murmur over right clavicle 3.0 (2-4.1) Show 2 more Pertinent negatives The following findings decrease the probability of aortic stenosis in adults. -1 Finding LR- Value Absence of systolic heart murmur 0 Absence of systolic murmur over right clavicle 0.10 (0.02-0.44) Absence of systolic murmur radiating to right carotid 0.29 (0.12-0.57) second heart sound not decreased 0.50 (0.27-0.76) Show 2 more References 1. Catherine M Otto, Rick A Nishimura, Robert O Bonow et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Thorac Cardiovasc Surg. 2021 Aug;162 2):e183-e353. Open 2. Alec Vahanian, Friedhelm Beyersdorf, Fabien Praz et al. 2021 ESC/EACTS Guidelines for the management of valvular heart disease. Eur Heart J. 2021 Aug 28;ehab395. Open 3. Carita P, Coppola G, Novo G et al. Aortic stenosis: insights on pathogenesis and clinical implications. J Geriatr Cardiol. 2016 Sep;13 6 489 98. Open 4. Nkomo VT, Gardin JM, Skelton TN et al. Burden of valvular heart diseases: a population-based study. Lancet. 2006 Sep 16;368 9540 1005 11. Open https://web.pathway.md/diseases/recPKP0FKP30zAZyO 7/8 6/23/23, 2:40 AM Aortic stenosis Pathway 5. Alqahtani F, Aljohani S, Amin AH et al. Effect of Race on the Incidence of Aortic Stenosis and Outcomes of Aortic Valve Replacement in the United States. Mayo Clin Proc. 2018 May;93 5 607 617. Open 6. Thaden JJ, Nkomo VT, Enriquez-Sarano M. The global burden of aortic stenosis. Prog Cardiovasc Dis. 2014 May-Jun;56 6 565 71. Open 7. Bakaeen FG, Rosengart TK, Carabello BA. Aortic Stenosis. Ann Intern Med. 2017 Jan 3;166 1 ITC1 ITC16. Open 8. Nishimura RA, Otto CM, Bonow RO et al. 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014 Jun 10;129 23 2440 92. Open 9. Nishimura RA, Otto CM, Bonow RO et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014 Jun 10;63 22):e57 185. Open 10. Nishimura RA, Otto CM, Bonow RO et al. 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2017 Jun 20;135 25):e1159-e1195. Open 11. Baumgartner H, Falk V, Bax JJ et al. 2017 ESC/EACTS Guidelines for the management of valvular heart disease. Eur Heart J. 2017 Sep 21;38 36 2739 2791. Open 12. Alec Vahanian, Friedhelm Beyersdorf, Fabien Praz et al. 2021 ESC/EACTS Guidelines for the management of valvular heart disease: Developed by the Task Force for the management of valvular heart disease of the European Society of Cardiology ESC and the European Association for Cardio-Thoracic Surgery EACTS . Eur Heart J. 2021;ehab395. Open 13. Edward Etchells, MD, MSc et al. Does This Patient Have an Abnormal Systolic Murmur?. JAMA. 1997;277 7 564 571. Open https://web.pathway.md/diseases/recPKP0FKP30zAZyO 8/8
Guideline sources The following summarized guidelines for the evaluation and management of appendiceal neoplasm are prepared by our editorial team based on guidelines from the National Comprehensive Cancer Network (NCCN 2021) and the American Society of Colon and Rectal Surgeons (ASCRS 2019). 1 2 Calculator Calculator TNM classification for appendic TNM classification for appendic Guidelines 1. Diagnostic investigations Clinical examination: elicit a complete history and perform physical examination in patients with appendiceal neoplasms. B Diagnostic imaging: https://web.pathway.md/diseases/recIrRYbWtlfkgXmh 1/4 6/23/23, 2:40 AM Appendiceal neoplasm Pathway g g g As per NCCN 2021 guidelines, obtain abdominal/pelvic multiphase CT or MRI in patients with appendiceal appendiceal NETs > 2 cm or incomplete resection or positive nodes/margins. B Show 2 more As per ASCRS 2019 guidelines: Do not obtain NET-specific imaging in all patients with appendiceal NETs. D Obtain cross-sectional imaging with CT or MRI at diagnosis of appendiceal epithelial neoplasms and routinely after resection. B Laboratory tests: As per NCCN 2021 guidelines, obtain biochemical testing as clinically indicated in patients with locoregionally advanced or metastatic appendiceal NETs. B As per ASCRS 2019 guidelines: Obtain biochemical testing in patients with localized or metastatic appendiceal NETs to establish baseline measures for future surveillance and disease monitoring. B Obtain tumor markers at diagnosis of appendiceal epithelial neoplasms and routinely after resection. B Preoperative assessment: Include the following in preoperative assessment of patients with appendiceal NETs: history and physical examination CT or MRI of chest, abdomen and pelvis colonoscopy. B 2. Diagnostic procedures Colonoscopy: perform colonoscopy in patients with confirmed or suspected appendiceal neoplasms. B Peritoneal cytology: do not obtain routine peritoneal cytology in patients with appendiceal neoplasms. D 3. Medical management Management of advanced/metastatic disease: As per NCCN 2021 guidelines, observe with imaging or offer octreotide or lanreotide in asymptomatic patients with locoregionally advanced or metastatic appendiceal NETs with low tumor burden. B Show 3 more As per ASCRS 2019 guidelines: Consider administering systemic chemotherapy to improve survival in patients with metastatic high-grade appendiceal mucinous neoplasms. B https://web.pathway.md/diseases/recIrRYbWtlfkgXmh 2/4 6/23/23, 2:40 AM Appendiceal neoplasm Pathway Insufficient evidence to support the use of systemic chemotherapy in patients with low-grade lesions with peritoneal spread. I 4. Therapeutic procedures Hyperthermic intraperitoneal chemotherapy: consider administering intraperitoneal chemotherapy to reduce peritoneal disease recurrence in selected patients with appendiceal epithelial neoplasms. B 5. Surgical interventions Indications for surgery: As per NCCN 2021 guidelines, perform simple appendectomy in patients with appendiceal NETs 2 cm. B Show 3 more As per ASCRS 2019 guidelines, perform cytoreductive surgery in selected patients with appendiceal neoplasms and evidence of peritoneal involvement. B Show 4 more 6. Follow-up and surveillance Follow-up assessment: As per NCCN 2021 guidelines, obtain follow-up assessment in patients with NETs after resection: Situation Guidance History and physical examination 12 weeks to 12 months Biochemical markers (24-hour urine or serum 5-HIAA, testing for Cushing's syndrome) as clinically indicated Abdominal +/- pelvic multiphase CT or MRI as clinically indicated History and physical examination 1-10 years (every 1-2 years) Biochemical markers (24-hour urine or serum 5-HIAA, testing for Cushing's syndrome) as clinically indicated Abdominal +/- pelvic multiphase CT or MRI Obtain surveillance as clinically indicated. B > 10 years https://web.pathway.md/diseases/recIrRYbWtlfkgXmh 3/4 6/23/23, 2:40 AM Appendiceal neoplasm Pathway As per ASCRS 2019 guidelines, include the following in the surveillance after resection of appendiceal NETs with a curative intent: physical examination serial biochemical testing CT or MRI of the chest, abdomen and pelvis. B References 1. Sean C Glasgow, Wolfgang Gaertner, David Stewart et al. The American Society of Colon and Rectal Surgeons, Clinical Practice Guidelines for the Management of Appendiceal Neoplasms. Dis Colon Rectum. 2019 Dec;62 12 1425 1438. Open 2. Manisha H Shah, Whitney S Goldner, Al B Benson et al. Neuroendocrine and Adrenal Tumors, Version 3.2021, NCCN Clinical Practice Guidelines in Oncology. NCCN. 2021 Aug 13. Open https://web.pathway.md/diseases/recIrRYbWtlfkgXmh 4/4
Guideline sources The following summarized guidelines for the evaluation and management of ascites are prepared by our editorial team based on guidelines from the British Association for the Study of the Liver (BASL/BSG 2021), the American Association for the Study of Liver Diseases (AASLD 2021; 2014), and the European Association for the Study of the Liver (EASL 2018). 1 2 3 4 5 6 6 6 Definition Ascites is a pathologic accumulation of fluid within the peritoneal cavity that usually develops as a result of liver disease, congestive HF, or nephrotic syndrome. 5 Epidemiology Ascites is most frequently caused by portal hypertension occurring in the setting of cirrhosis. Other causes are broadly divided into pre-hepatic (portal vein thrombosis, lymphoma, abdominal lymphatic injury or obstruction, bowel perforation, renal failure, pancreatitis, peritoneal tuberculosis or malignancy with peritoneal implants) and post-hepatic (congestive HF, constrictive pericarditis, Budd-Chiari syndrome, and stricture/web formation in the IVC) causes. 6 https://web.pathway.md/diseases/recs0e0JJ5V8pmQoN 1/8 6/23/23, 3:10 AM Ascites Pathway Disease course Ascites causes clinical manifestations of abdominal distention, abdominal fullness, discomfort, shortness of breath, early satiation, and a sense of reduced mobility. 6 Prognosis and risk of recurrence Ascites is associated with a 1-year mortality of 40%. Refractory ascites has a median survival rate of 6 months. 6 Calculator Calculator Child Pugh score for mortality in Mumtaz readmission risk score i Guidelines 1. Diagnostic investigations Initial evaluation: Obtain the following as part of the initial evaluation of patients with ascites: history and physical examination abdominal ultrasound laboratory assessment of liver and renal functions, serum and urine electrolytes ascitic fluid analysis. Use contrast media cautiously and implement appropriate preventive measures for renal impairment, B although the use of contrast media does not appear to be associated with an increased risk of renal impairment in patients with ascites and preserved renal function. B 2. Diagnostic procedures Diagnostic paracentesis: As per AASLD 2021 guidelines, perform diagnostic paracentesis in all patients with new-onset ascites accessible for sampling. E Show 5 more As per BSG 2021 guidelines, perform diagnostic paracentesis in all patients with new-onset ascites. B Show 6 more As per EASL 2018 guidelines, perform diagnostic paracentesis in all patients with new-onset grade 2 or 3 ascites, and in hospitalized patients with worsening ascites or any complication of https://web.pathway.md/diseases/recs0e0JJ5V8pmQoN 2/8 6/23/23, 3:10 AM Ascites Pathway cirrhosis. B Show 6 more 3. Medical management General principles: Control and treat gastrointestinal hemorrhage, renal impairment, hepatic encephalopathy, hyponatremia, or alterations in serum potassium concentration before initiating diuretic therapy. Be cautious when initiating diuretic therapy and obtain frequent clinical and biochemical assessments. B Identify and treat etiological factors in patients with decompensated cirrhosis, particularly alcohol consumption and hepatitis B or C virus infection. B Diuretic therapy: As per AASLD 2021 guidelines: Initiate diuretics (spironolactone with or without furosemide), along with moderate sodium restriction (2 g or 90 mmol/day), as first-line therapy in patients with cirrhosis and grade 2 ascites. E Attempt to taper the diuretics to the lowest dose necessary to maintain minimal or no ascites to prevent the development of adverse effects after ascites is adequately mobilized. E As per BSG 2021 guidelines, consider initiating spironolactone monotherapy (starting dose 100 mg, increased to 400 mg) in patients with the first presentation of moderate ascites. B Show 6 more As per EASL 2018 guidelines, initiate spironolactone (starting at 100 mg/day PO, with stepwise increases every 72 hours to a maximum of 400 mg/day if there is no response to lower doses) in patients with a first episode of grade 2 ascites. A Show 4 more Intravenous albumin: As per AASLD 2021 guidelines: Consider administering human albumin solution (20-40 g/week) in patients with severe muscle cramps. E Insufficient evidence to support the routine long-term infusion of human albumin solution in patients with cirrhosis and diuretic-responsive ascites. I As per BSG 2021 guidelines, administer albumin (20% or 25% solution) after paracentesis of > 5 L is completed at a dose of 8 g albumin/L of ascites removed. A Show 2 more Other agents: As per AASLD 2021 guidelines, consider administering baclofen (10 mg/day, with a weekly increase of 10 mg/day, up to 30 mg/day) in patients with severe muscle cramps. E https://web.pathway.md/diseases/recs0e0JJ5V8pmQoN 3/8 6/23/23, 3:10 AM Ascites Pathway As per EASL 2018 guidelines, insufficient evidence to support adding clonidine or midodrine to improve the efficacy of diuretic treatment. I Medications to avoid: As per AASLD 2021 guidelines: Avoid using NSAIDs, ACEIs and ARBs in patients with cirrhosis and ascites. D Avoid using aminoglycosides for the treatment of bacterial infections, whenever possible. D As per EASL 2018 guidelines: Avoid NSAIDs in patients with ascites because of the high risk of developing further sodium retention, hyponatremia, and AKI. D Avoid ACEIs, ARBs, or alpha-1 receptor blockers in most patients with ascites, as these drugs have been associated with an increased risk of renal impairment. D Management of hyponatremia: obtain monitoring and advise water restriction in patients with mild hyponatremia (126-135 mEq/L) without symptoms. E Show 6 more 4. Nonpharmacologic interventions Salt restriction: As per AASLD 2021 guidelines, advise moderate sodium restriction (2 g or 90 mmol/day) in patients with cirrhosis and grade 2 ascites. E As per BSG 2021 guidelines: Advise moderate salt restriction with daily salt intake of 5-6.5 g (87-113 mmol sodium), meaning no added salt diet with avoidance of precooked meals, in patients with cirrhosis and ascites. B Provide nutritional counseling on the sodium content in the diet in patients with cirrhosis and ascites. B As per EASL 2018 guidelines, advise moderate restriction of sodium intake (80-120 mmol/day, corresponding to 4.6-6.9 g of salt) in patients with moderate, uncomplicated ascites. A Fluid restriction: do not advise fluid restriction for ascites management unless there is concomitant moderate or severe hyponatremia (serum sodium 125 mmol/L). D Physical activity: insufficient evidence to support prolonged bed rest for the treatment of ascites. I 5. Therapeutic procedures Therapeutic paracentesis: As per AASLD 2021 guidelines, perform large-volume paracentesis as first-line therapy in patients with grade 3 ascites. Advise sodium restriction and initiate diuretics after paracentesis. E https://web.pathway.md/diseases/recs0e0JJ5V8pmQoN 4/8 6/23/23, 3:10 AM Ascites Pathway As per BSG 2021 guidelines: Consider using ultrasound guidance when available during large-volume paracentesis to reduce the risk of adverse events. C Do not obtain routine measurement of the PT and platelet count before therapeutic or diagnostic paracentesis and infusion of blood products. D As per EASL 2018 guidelines, perform large-volume paracentesis, removing ascites completely in a single session, as first-line therapy in patients with large ascites (grade 3 ascites), followed by plasma volume expansion to prevent post-paracentesis circulatory dysfunction. A Show 4 more Transjugular intrahepatic portosystemic shunt: As per BSG 2021 guidelines: Consider performing TIPS placement in patients with refractory ascites. B Be cautious when considering TIPS placement in patients with: age > 70 years serum bilirubin > 50 mcmol/L platelet count < 75 10 /L MELD score 18 current hepatic encephalopathy active infection or hepatorenal syndrome. B As per EASL 2018 guidelines, evaluate patients with refractory or recurrent ascites for TIPS insertion. A Show 5 more Implantable peritoneal pump: As per BSG 2021 guidelines, consider placing an automated low-flow ascites pump only in special circumstances with robust arrangements of clinical governance, audit or research. C As per EASL 2018 guidelines, consider placing an implantable peritoneal pump in experienced centers in patients with refractory ascites not amenable to transhepatic intrajugular portosystemic shunt insertion. Obtain close monitoring because of the high risk of adverse events including renal dysfunction and technical difficulties. B 6. Surgical interventions Liver transplantation: As per AASLD 2021 guidelines, consider referring patients with grade 2 or 3 ascites for evaluation for liver transplantation. E As per EASL 2018 guidelines, evaluate patients with refractory ascites or hydrothorax for liver transplantation. B Show 10 more As per AASLD 2014 guidelines: https://web.pathway.md/diseases/recs0e0JJ5V8pmQoN 5/8 6/23/23, 3:10 AM Ascites Pathway Consider evaluating for liver transplantation once a patient with cirrhosis has experienced an index complication, such as ascites, hepatic encephalopathy, or variceal hemorrhage, or hepatocellular dysfunction results in a MELD score 15. B Treat potentially treatable etiologies and components of hepatic decompensation in liver transplant candidates, such as ascites, hepatic encephalopathy, or variceal hemorrhage. B Elective hernia repair: As per AASLD 2021 guidelines: Perform elective hernia repair in patients with cirrhosis through a multidisciplinary approach after ascites has been controlled and the patient's overall condition, including nutritional status, has been optimized. E Consider performing TIPS placement before elective hernia repair or after an emergent surgery in patients with cirrhosis and uncontrolled ascites. E As per BSG 2021 guidelines, discuss the suitability and timing of surgical repair of umbilical hernia with the patient and multidisciplinary team involving physicians, surgeons, and anesthesiologists. B 7. Specific circumstances Pediatric patients: obtain a comprehensive evaluation of clinical history, physical examination, and diagnostic testing including abdominal ultrasound for the diagnosis of ascites and its cause in pediatric patients. E Show 6 more Patients with bacterascites: Initiate antibiotics in patients with bacterascites (neutrophil count < 250/mm but positive bacterial culture) exhibiting signs of systemic inflammation or infection. B Perform a second paracentesis in patients with bacterascites as an alternative strategy to antibiotic therapy. Initiate treatment if the culture results come back positive again regardless of the neutrophil count. B Patients with hepatic hydrothorax: As per AASLD 2021 guidelines, advise dietary sodium restriction and initiate diuretics plus thoracentesis as required as first-line therapy in patients with hepatic hydrothorax. E Show 3 more As per BSG 2021 guidelines: Consider performing TIPS placement in patients with hepatic hydrothorax, after discussion with the multidisciplinary team. B Consider offering alternative palliative interventions in patients with hepatic hydrothorax not undergoing TIPS placement and/or liver transplant evaluation. B As per EASL 2018 guidelines, exclude cardiopulmonary and primary pleural disease before diagnosing hepatic hydrothorax. B Show 6 more https://web.pathway.md/diseases/recs0e0JJ5V8pmQoN 6/8 6/23/23, 3:10 AM Ascites Pathway 8. Follow-up and surveillance Serial clinical and laboratory assessment: As per AASLD 2021 guidelines, monitor body weight and serum creatinine and sodium regularly to assess response and to detect the development of adverse effects in patients receiving diuretics. E As per EASL 2018 guidelines, advise a maximum weight loss of 0.5 kg/day in patients without edema and 1 kg/day in patients with edema during diuretic therapy. B Show 2 more Post-TIPS care: Continue diuretics and salt restriction after TIPS insertion up to the resolution of ascites. B Continue close clinical follow-up after TIPS insertion. B Management of inadequate response: Initiate the combination of a mineralocorticoid receptor antagonist and furosemide in patients with long-standing or recurrent ascites, increasing the doses sequentially according to clinical response. A Consider initiating torasemide in patients exhibiting a weak response to furosemide. B Management of refractory ascites: As per AASLD 2021 guidelines, advise continued dietary sodium restriction (< 2 g/day) in patients with refractory ascites to reduce the rate of ascites accumulation. E Show 10 more As per BSG 2021 guidelines, do not view refractory ascites as a contraindication to nonselective -blockers. D Show 2 more As per EASL 2018 guidelines, diagnose refractory ascites based on the assessment of the response of ascites to diuretic therapy and salt restriction in stable patients without associated complications, such as bleeding or infection, after ascertaining patient compliance with treatment. B Show 6 more Likelihood Ratios Pertinent positives The following findings increase the probability of ascites in adults. 1 1 Finding LR+ Value Positive fluid wave sign 5.3 (2.9-9.5) History of recent increase in abdominal girth 4.1 (2.3-4.7) History of recent weight gain 3.2 (1.7-6.2) History of ankle swelling 2.8 (1.8-4.3) https://web.pathway.md/diseases/recs0e0JJ5V8pmQoN 7/8 6/23/23, 3:10 AM Ascites Pathway Show 3 more Pertinent negatives The following findings decrease the probability of ascites in adults. 1 1 Finding LR- Value Absence of flank dullness 0.1 No history of ankle swelling 0.10 (0.01-0.67) No history of recent increase in abdominal girth 0.17 (0.05-0.62) No history of recent weight gain 0.42 (0.20-0.87) Show 1 more References 1. Guruprasad P Aithal, Naaventhan Palaniyappan, Louise China et al. Guidelines on the management of ascites in cirrhosis. Gut. 2021 Jan;70 1 9 29. Open 2. European Association for the Study of the Liver. EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis. J Hepatol. 2018 Aug;69 2 406 460. Open 3. Scott W Biggins, Paulo Angeli, Guadalupe Garcia-Tsao et al. Diagnosis, Evaluation, and Management of Ascites, Spontaneous Bacterial Peritonitis and Hepatorenal Syndrome: 2021 Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Aug;74 2 1014 1048. Open 4. Paul Martin, Andrea DiMartini, Sandy Feng et al. Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology. 2014 Mar;59 3 1144 65. Open 5. Ming-Huei Cheng, Ming-Shyen Yen, Kuan-Chong Chao et al. Differential diagnosis of gynecologic organ- related diseases in women presenting with ascites. 2008 Dec;47 4 384 90.2008 Dec;47 4 384 90. Open 6. Christopher M Moore, David H Van Thiel. Cirrhotic ascites review: Pathophysiology, diagnosis and management. 2013 May 27;5 5 251 63.2013 May 27;5 5 251 63. Open 7. de Franchis R, Baveno VI Faculty. Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: Stratifying risk and individualizing care for portal hypertension. J Hepatol. 2015 Sep;63 3 743 52. Open 8. European Association for the Study of the Liver. EASL clinical practice guidelines on the management of ascites, spontaneous bacterial peritonitis, and hepatorenal syndrome in cirrhosis. J Hepatol. 2010 Sep;53 3 397 417. Open 9. Runyon BA, AASLD. Introduction to the revised American Association for the Study of Liver Diseases Practice Guideline management of adult patients with ascites due to cirrhosis 2012. Hepatology. 2013 Apr;57 4 1651 3. Open 10. D L Simel, R A Halvorsen Jr, J R Feussner. Quantitating bedside diagnosis: clinical evaluation of ascites. J Gen Intern Med. Sep-Oct 1988;3 5 423 8. Open https://web.pathway.md/diseases/recs0e0JJ5V8pmQoN 8/8
Guideline sources The following summarized guidelines for the evaluation and management of aspirin overdose are prepared by our editorial team based on guidelines from the Extracorporeal Treatments in Poisoning Workgroup (EXTRIP 2015), the U.S. Department of Health and Human Services (HHS 2007), and the National Poisons Information Service (NPIS 2002). 1 2 2 2 2 3 4 Definition Aspirin overdose is the plasma salicylate concentration of 300-500 mg/L in mild toxicity, 500-700 mg/L in moderate toxicity, and > 750 mg/L in severity toxicity. 2 Epidemiology Aspirin overdose is caused by accidental ingestions in children, suicidal or intentional overdoses in adults. 4 Disease course Acute aspirin overdose results in salicylate poisoning resulting in either mild, moderate, or severe toxicity depending on the plasma salicylate concentration. Mild toxicity presents with burning in the mouth, lethargy, nausea and vomiting, tinnitus, or dizziness. Moderate toxicity includes additional https://web.pathway.md/diseases/recb81eJQsuACVEB7 1/4 6/23/23, 3:12 AM Aspirin overdose Pathway symptoms of tachypnea, hyperpyrexia, sweating, dehydration, loss of coordination, and restlessness. Severe toxicity causes hallucinations, stupor, convulsions, cerebral edema, oliguria, renal failure, cardiovascular failure, metabolic acidosis, and coma. 2 Prognosis and risk of recurrence Severe salicylate poisoning results in 5% mortality. 2 Guidelines 1. Classification and risk stratification Setting of care: refer patients with stated or suspected self-harm or who are the victims of a potentially malicious administration of a salicylate to an emergency department immediately, regardless of the dose reported. B Show 2 more Severity grading: very mild poisoning occurs in patients with a salicylate level < 300 mg/L (adults) or < 200 mg/L (children or elderly). These patients are generally asymptomatic. E Show 3 more 2. Diagnostic investigations Laboratory investigations: Obtain serum salicylate levels, renal function and electrolytes, CBC, and INR in patients with aspirin overdose, including measurements performed at least 4 hours after ingestion. E Obtain an arterial blood gas in patients with symptomatic aspirin overdose. E 3. Medical management General principles: administer oral or intravenous fluid therapy to patients with mild aspirin overdose. E Show 2 more Supportive care: In patients with aspirin overdose who display severe end-organ manifestations (coma, convulsions, acute renal failure pulmonary edema): initiate hemodynamic and respiratory support perform arterial blood gas sampling discuss with poison control centre and toxicology consider hemodialysis - see therapeutic procedures section. E Poison center consultation: ensure follow-up communication with the poison centre at periodic intervals for at least 12 hours after ingestion of non-enteric-coated salicylate products, and for at least 24 hours after the ingestion of enteric-coated aspirin. B https://web.pathway.md/diseases/recb81eJQsuACVEB7 2/4 6/23/23, 3:12 AM Aspirin overdose Pathway Gastric decontamination: As per HHS 2007 guidelines: Avoid intentionally inducing emesis in patients with aspirin overdose. D Consider the out-of-hospital administration of activated charcoal for acute ingestions of a toxic dose if it is immediately available, no contraindications are present, the patient is not vomiting, and local guidelines for its out-of-hospital use are observed. However, do not delay transportation in order to administer activated charcoal. C As per NPIS 2002 guidelines: Perform gastric lavage (ensuring the airway is protected) in patients who have taken an aspirin dose > 500 mg/kg, within < 1 hour before presentation, and the dose and time of ingestion is certain. E Administer oral activated charcoal (ensuring the airway is protected) in the following situations: patients who have taken an unknown dose of aspirin patients who have taken a dose of aspirin 125 mg/kg patients who have taken a dose of aspirin of > 500 mg/kg, > 1 hour ago (as an alternative to gastric lavage). E Urinary alkalinization: Administer 1.26% sodium bicarbonate with 20 to 40 mmol potassium (1,000 mL IV over 3 hours) as the initial strategy for urine alkalinization in adults with aspirin overdose. E Administer 1 ml/kg 8.4% sodium bicarbonate in 10 ml/kg sodium chloride solution with 1 mmol/kg potassium (IV, at a rate of 2 ml/kg/h) as the initial strategy for urine alkalinization in children with aspirin overdose. E Management of metabolic acidosis: Administer 8.4% sodium bicarbonate (1 mL/kg IV) in patients with aspirin overdose and serum pH < 7.3, targeting an increase pH to 7.4. E Consider performing hemodialysis in addition to giving sodium bicarbonate in patients with a serum pH < 7.2 - see therapeutic procedures section. E 4. Inpatient care Monitoring of urinary alkalinization: Measure urine pH every 1 hour, targeting a urine pH of 7.5-8.5; increase the rate of bicarbonate administration if the urine pH remains < 7.5 (note: avoid serum pH > 7.55, closely monitor serum sodium). E Check electrolytes and renal function every 3 hours, and administer supplemental potassium as needed to keep the serum potassium in the range 4.0 to 4.5. E 5. Therapeutic procedures https://web.pathway.md/diseases/recb81eJQsuACVEB7 3/4 6/23/23, 3:12 AM Aspirin overdose Pathway Indications for extracorporeal treatment: ECTR is recommended in severe salicylate poisoning. B Show 11 more Extracorporeal treatment modality: use intermittent hemodialysis as the preferred modality of ECTR in patients with aspirin overdose. B Show 2 more 6. Specific circumstances Patients with dermal exposure: Perform skin washing with soap and water in asymptomatic patients with dermal exposures to salicylates. B Consider discharging asymptomatic patients with dermal exposures to salicylates after skin washing, with observation at home for the development of symptoms. C Patients with ocular exposure: Perform ocular irrigation with room-temperature tap water for 15 minutes in patients with an ocular exposure to salicylates. B Obtain an ophthalmological consultation if ocular pain, decreased visual acuity, or persistent ocular irritation is present after irrigation. B 7. Follow-up and surveillance Discharge from hospital: obtain an assessment of the patient's mental state and risk of repeated episodes of deliberate self harm before discharge, ideally performed by a psychiatrist or psychiatric liaison nurse. E Show 2 more References 1. Peter A Chyka, Andrew R Erdman, Gwenn Christianson et al. Salicylate poisoning: an evidence-based consensus guideline for out-of-hospital management. Clin Toxicol Phila). 2007;45 2 95 131. Open 2. P I Dargan, C I Wallace, A L Jones. An evidence based flowchart to guide the management of acute salicylate (aspirin) overdose. Emerg Med J. 2002 May;19 3 206 9. Open 3. David N Juurlink, Sophie Gosselin, Jan T Kielstein et al. Extracorporeal Treatment for Salicylate Poisoning: Systematic Review and Recommendations From the EXTRIP Workgroup. Ann Emerg Med. 2015 Aug;66 2 165 81. Open 4. Debasish Ghosh, Kenneth M Williams, Garry G Graham et al. Multiple episodes of aspirin overdose in an individual patient: a case report. 2014 Nov 19;8 374.2014 Nov 19;8 374. Open https://web.pathway.md/diseases/recb81eJQsuACVEB7 4/4
Guideline sources The following summarized guidelines for the evaluation and management of assisted human reproduction are prepared by our editorial team based on guidelines from the Society of Obstetricians and Gynaecologists of Canada (SOGC 2014). 1 1 1 2 3 Definition Assisted human reproduction or assisted reproductive technology refers to any procedure that involves handling eggs, sperm, or both outside the human body (in vitro) including IVF and intrauterine insemination, and ovarian stimulation with gonadotropin or medications. 1 Epidemiology Assisted human reproduction is used to address primary or secondary infertility, fertility preservation after gonadotoxic treatments in reproductive-age patients, to enable same-sex couples and single women and men to conceive and have biological children, and to facilitate the services of surrogate gestational carriers. 1 Disease course Reproductive problems or infertility is addressed through assisted human reproduction, which includes IVF (IVF), intratubal transfer of gametes (GIFT), intratubal transfer of zygotes (ZIFT), tubal transfer of preimplantation embryos (TET), gamete or embryo donation, cryopreservation, and micromanipulation. 2 https://web.pathway.md/diseases/recY3AYaTCWq2YHMX 1/4 6/23/23, 3:12 AM Assisted human reproduction Pathway Prognosis and risk of recurrence In assisted human reproduction, early mortality increases multiple pregnancies with each additional fetus corresponding to a relative risk of 2.4 for triplets, 3.3 for quadruplets, and 10.3 for quintuplets. 3 Guidelines 1. Diagnostic investigations Genetic testing: offer genetic/clinical counseling, karyotype assessment for chromosomal abnormalities, and Y-chromosome microdeletion testing to all men with severe oligozoospermia or azoospermia (sperm count < 5 million/hpf) prior to IVF with intracytoplasmic sperm injection. B 2. Surgical interventions Elective single embryo transfer: provide elective single embryo transfer in couples with good prognosis for success. B Cryopreserved embryos fertilized in vitro: there is increasing evidence that pregnancy outcomes are better for cryopreserved embryos fertilized in vitro than for fresh embryo transfers. This finding supports a policy of elective single embryo transfer for women with a good prognosis, and may reassure women who are considering IVF. B Intracytoplasmic sperm injection: Advise women and couples considering assisted human reproduction and concerned about perinatal outcomes that: intracytoplasmic sperm injection does not appear to confer increased adverse perinatal or maternal risk over standard IVF the use of donor oocytes increases successful pregnancy rates in selected women, but even when accounting for maternal age, can increase the risks of low birth weight and preeclampsia. B 3. Patient education Genetic counseling: counsel couples thoroughly about the significant risks of multiple pregnancies associated with all assisted human reproductive treatments. B 4. Follow-up and surveillance Serial imaging assessment: counsel patients being evaluated for any assisted reproductive technology procedure on the slightly increased risk fo congenital structural abnormalities, with emphasis on known confounding factors such as infertility and BMI. B https://web.pathway.md/diseases/recY3AYaTCWq2YHMX 2/4 6/23/23, 3:12 AM Assisted human reproduction Pathway References 1. Okun N, Sierra S. Pregnancy outcomes after assisted human reproduction. J Obstet Gynaecol Can. 2014 Jan;36 1 64 83. Open 2. R Fernandez Pelegrina, A G Kessler, R G Rawlins. Modern approaches to the treatment of human infertility through assisted reproduction. 1991 Aug;10 2 75 81.1991 Aug;10 2 75 81. Open 3. Hamisu M Salihu, Muktar H Aliyu, Dwight J Rouse et al. Potentially preventable excess mortality among higher-order multiples. 2003 Oct;102 4 679 84.2003 Oct;102 4 679 84. Open 4. Joyce Harper, Joep Geraedts, Pascal Borry et al. Current issues in medically assisted reproduction and genetics in Europe: research, clinical practice, ethics, legal issues and policy. Hum Reprod. 2014 Aug;29 8 1603 9. Open 5. Ana Garc a-Blanco, Vicente Diago, David Herv s et al. Anxiety and depressive symptoms, and stress biomarkers in pregnant women after in vitro fertilization: a prospective cohort study. Hum Reprod. 2018 Jul 1;33 7 1237 1246. Open 6. European Society of Human Genetics, European Society of Human Reproduction and Embryology. The need for interaction between assisted reproduction technology and genetics: recommendations of the European Societies of Human Genetics and Human Reproduction and Embryology. Hum Reprod. 2006 Aug;21 8 1971 3. Open 7. Andr Van Steirteghem. Assisted reproductive techniques and human reproduction. Hum Reprod. 2009 Nov;24 11 2675. Open 8. C Gnoth, E Godehardt, P Frank-Herrmann et al. Definition and prevalence of subfertility and infertility. Hum Reprod. 2005 May;20 5 1144 7. Open 9. Satvinder Purewal, Sarah C E Chapman, Olga B A van den Akker. Depression and state anxiety scores during assisted reproductive treatment are associated with outcome: a meta-analysis. Reprod Biomed Online. 2018 Jun;36 6 646 657. Open 10. Bettina Toth, Dunja Maria Baston-B st, Hermann M Behre et al. Diagnosis and Treatment Before Assisted Reproductive Treatments. Guideline of the DGGG, OEGGG and SGGG S2k Level, AWMF Register Number 015 085, February 2019 Part 2, Hemostaseology, Andrology, Genetics and History of Malignant Disease. Geburtshilfe Frauenheilkd. 2019 Dec;79 12 1293 1308. Open 11. Bettina Toth, Dunja Maria Baston-B st, Hermann M Behre et al. Diagnosis and Therapy Before Assisted Reproductive Treatments. Guideline of the DGGG, OEGGG and SGGG S2k Level, AWMF Register Number 015 085, February 2019 Part 1, Basic Assessment of the Woman. Geburtshilfe Frauenheilkd. 2019 Dec;79 12 1278 1292. Open 12. A. Salonia, C. Bettocchi, J. Carvalho et al. EAU Guidelines on Sexual and Reproductive Health. EAU. 2023. Open 13. Shannon M Bates, Ian A Greer, Saskia Middeldorp et al. VTE, thrombophilia, antithrombotic therapy, and pregnancy: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e691S-e736S. Open https://web.pathway.md/diseases/recY3AYaTCWq2YHMX 3/4 6/23/23, 3:12 AM Assisted human reproduction Pathway 14. Monika Sarkar, Carla W Brady, Jaquelyn Fleckenstein et al. Reproductive Health and Liver Disease: Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73 1 318 365. Open https://web.pathway.md/diseases/recY3AYaTCWq2YHMX 4/4
Guideline sources The following summarized guidelines for the evaluation and management of assisted vaginal delivery are prepared by our editorial team based on guidelines from the European Association of Perinatal Medicine (EAPM 2022), the Royal College of Obstetricians and Gynaecologists (RCOG 2020), the American College of Obstetricians and Gynecologists (ACOG 2020), the Society of Obstetricians and Gynaecologists of Canada (SOGC 2019; 2018), and the World Health Organization (WHO 2015). 1 2 3 4 5 6 Guidelines 1. Classification and risk stratification https://web.pathway.md/diseases/recdwpSswFSfKW1N5 1/6 6/23/23, 3:12 AM Assisted vaginal delivery Pathway Classification: use a standard classification system for assisted vaginal delivery to promote safe clinical practice, effective communication between health professionals, and audit of outcomes. B 2. Diagnostic investigations Ultrasound: Obtain ultrasound assessment of the fetal head position before assisted vaginal delivery where uncertainty exists following clinical examination. A Insufficient evidence to recommend routine abdominal or perineal ultrasound for assessment of the station, flexion, and descent of the fetal head in the second stage of labor. I 3. Medical management Antibiotic prophylaxis: As per RCOG 2020 guidelines, administer a single prophylactic dose of IV amoxicillin and clavulanic acid following assisted vaginal delivery. A As per ACOG 2015 guidelines, do not administer routine antibiotic prophylaxis in patients undergoing operative vaginal birth. D Analgesia: offer regular NSAIDs and paracetamol routinely in the absence of contraindications. A Postpartum thromboprophylaxis: reassess patients after assisted vaginal delivery for VTE risk and the need for thromboprophylaxis. B 4. Nonpharmacologic interventions Psychological support: ensure shared decision-making and good communication, and provide positive continuous support during labor and birth to reduce psychological morbidity after birth. B Show 4 more 5. Therapeutic procedures Indications for assisted vaginal delivery: As per RCOG 2020 guidelines, recognize that no indication is absolute and clinical judgment is required in all situations. B As per SOGC 2019 guidelines, consider encouraging the use of safe and effective assisted vaginal delivery by experienced and skilled care providers as a strategy to reduce the rate of primary Cesarean delivery. C https://web.pathway.md/diseases/recdwpSswFSfKW1N5 2/6 6/23/23, 3:12 AM Assisted vaginal delivery Pathway Contraindications for assisted vaginal delivery: as per RCOG 2020 guidelines, recognize that suspected fetal bleeding disorders or a predisposition to fracture are relative contraindications to assisted vaginal delivery. B Show 3 more Choice of instrument: As per RCOG 2020 guidelines, decide on the choice of the instrument most appropriate to the clinical circumstance and the operator's level of skills. B Show 2 more As per SOGC 2019 guidelines, determine the instrument most suitable to the clinical circumstance and physician's level of skill. Recognize that vacuum and forceps are associated with different short- and long-term benefits and risks, and unsuccessful delivery is more likely with the vacuum. A Show 2 more Considerations for assisted vaginal delivery, general principles: As per RCOG 2020 guidelines, ensure a careful assessment of the clinical situation, clear communication with the patient and healthcare personnel, and expertise in the chosen procedure for safe assisted vaginal delivery. B Show 5 more As per SOGC 2019 guidelines: Ensure appropriate expertise in the chosen method, comprehensive assessment of the clinical situation alongside clear communication with the patient, support people, and healthcare personnel for safe and effective assisted vaginal delivery. B Attempt a trial of assisted vaginal delivery performed in a location where immediate recourse to Cesarean delivery is available if assisted vaginal delivery is deemed to have a higher risk of not being successful. B Considerations for assisted vaginal delivery (vacuum extraction): discontinue vacuum- assisted birth if there is no evidence of progressive descent with moderate traction during each pull of a correctly applied instrument by an experienced operator. B Show 7 more Considerations for assisted vaginal delivery (forceps use): discontinue attempted forceps delivery where the forceps cannot be applied easily, the handles do not approximate easily, or if there is a lack of progressive descent with moderate traction. B Show 5 more 6. Surgical interventions Episiotomy: As per EAPM 2022 guidelines, perform episiotomy by indication only, B including in order to shorten the second stage of labor when there is suspected fetal hypoxia, B , prevent obstetric https://web.pathway.md/diseases/recdwpSswFSfKW1N5 3/6 6/23/23, 3:12 AM Assisted vaginal delivery Pathway anal sphincter injury in vaginal operative deliveries, or when obstetric sphincter injury occurred in previous deliveries. B Show 3 more As per RCOG 2020 guidelines, consider performing mediolateral episiotomy, discussed with the patient, as part of the preparation for assisted vaginal delivery. C Show 2 more As per SOGC 2019 guidelines, consider performing mediolateral episiotomy for assisted vaginal delivery, restricted to selected cases. C 7. Patient education Antenatal counseling: inform patients about assisted vaginal delivery in the antenatal period, especially during their first pregnancy, and if they indicate specific restrictions or preferences then explore this with an experienced obstetrician, ideally in advance of labor. B Show 2 more Postpartum counseling: Provide a debrief and support to the patient immediately following an attempted or successful assisted vaginal delivery and, if this is not possible, debrief before hospital discharge including the indication for assisted vaginal delivery, management of any complications, and the prognosis for future deliveries. B Encourage patients to consider spontaneous vaginal birth in a subsequent pregnancy. Individualize care planning and respect patient preferences. B 8. Preventative measures Prevention of assisted vaginal delivery: As per RCOG 2020 guidelines, ensure continuous support during labor to reduce the need for assisted vaginal birth. A Show 8 more As per SOGC 2019 guidelines, consider providing the following to reduce the need for assisted vaginal birth: dedicated and continuous support during labor B oxytocin augmentation of inadequate labor B delayed pushing in patients with an epidural B prolonged pushing in nulliparous patients with an epidural B optimization of fetal head position through manual rotation. B 9. Follow-up and surveillance https://web.pathway.md/diseases/recdwpSswFSfKW1N5 4/6 6/23/23, 3:12 AM Assisted vaginal delivery Pathway Prevention of urinary retention: counsel patients about the risk of urinary retention ensuring that they are aware of the importance of bladder emptying in the postpartum period. B Show 4 more 10. Quality improvement Documentation: Document the following regarding assisted vaginal delivery using a standardized form: detailed information on the assessment decision making and conduct of the procedure a plan for postnatal care sufficient information for counseling in relation to subsequent pregnancies. B Show 2 more Health professional training: As per RCOG 2020 guidelines: Ensure that obstetric trainees achieve expertise in spontaneous vaginal delivery before commencing training in assisted vaginal delivery. B Ensure that obstetric trainees receive appropriate training in vacuum and forceps birth, including theoretical knowledge, simulation training, and clinical training under direct supervision. Ensure competency is demonstrated before conducting unsupervised births. B As per SOGC 2019 guidelines: Ensure that practitioners performing assisted vaginal delivery have the knowledge, skills, and experience necessary to assess the clinical situation, use the selected instrument, and manage complications that may arise from assisted vaginal delivery. B Provide comprehensive training in assisted vaginal delivery in obstetrical trainees and ensure that they are competent before proceeding to independent practice. B References 1. D J Murphy, B K Strachan, R Bahl et al. Assisted Vaginal Birth: Green-top Guideline No. 26. BJOG. 2020 Aug;127 9):e70-e112. Open 2. Committee on Practice Bulletins Obstetrics with the assistance of Alan M. Peaceman, MD. Operative Vaginal Birth: ACOG Practice Bulletin, Number 219. Obstet Gynecol. 2020 Apr;135 4):e149-e159. Open 3. Yvonne M Cargill, Catherine Jane MacKinnon. No. 148 Guidelines for Operative Vaginal Birth. J Obstet Gynaecol Can. 2018 Feb;40 2):e74-e80. Open 4. No authors listed. WHO Recommendations for Prevention and Treatment of Maternal Peripartum Infections. Geneva: World Health Organization; 2015. Open 5. Sebastian Hobson, Krista Cassell, Rory Windrim et al. No. 381 Assisted Vaginal Birth. J Obstet Gynaecol Can. 2019 Jun;41 6 870 882. Open https://web.pathway.md/diseases/recdwpSswFSfKW1N5 5/6 6/23/23, 3:12 AM Assisted vaginal delivery Pathway 6. Katariina Laine, Branka M Yli, Vanessa Cole et al. European guidelines on perinatal care- Peripartum care Episiotomy. J Matern Fetal Neonatal Med. 2022 Dec;35 25 8797 8802. Open https://web.pathway.md/diseases/recdwpSswFSfKW1N5 6/6
Guideline sources The following summarized guidelines for the evaluation and management of asthma are prepared by our editorial team based on guidelines from the Global Initiative for Asthma (GINA 2022), the European Respiratory Society (ERS 2022; 2021; 2017; 2012), the American Thoracic Society (ATS 2021), the British Thoracic Society (BTS 2020), the National Heart, Lung, and Blood Institute (NHLBI 2020), the American Heart Association (AHA 2020), the American College of Chest Physicians (ACCP 2020), the British Thoracic Society (BTS/SIGN 2019), the Canadian Thoracic Society (CTS 2017; 2012), the Infectious Diseases Society of America (IDSA 2016), and the European Respiratory Society (ERS/ATS 2014). 1 2 3 4 5 6 7 8 9 10 12 13 14 15 16 Calculator Calculator Calculator Asthma Control Test ACT Pediatric Asthma Severity Score PRAM sco https://web.pathway.md/diseases/recoid4eeyMVLDPrX 1/26 6/23/23, 3:13 AM Asthma Pathway Guidelines 1. Screening and diagnosis Indications for screening: obtain questionnaire-based identification for surveillance of asthma in all workers at risk of developing work-related asthma. A Show 5 more Diagnostic criteria: As per ERS 2022 guidelines, avoid combining fraction exhaled nitric oxide, blood eosinophils, and serum IgE results to make a diagnosis of asthma. D As per GINA 2022 guidelines, diagnose asthma in adult, adolescent, and pediatric (aged 6-11 years) patients based on characteristic respiratory symptoms and evidence of variable expiratory airflow limitation: Situation Guidance > 1 type of respiratory symptom Wheeze, shortness of breath, chest tightness, and cough Symptoms occurring variably over time and vary in intensity Symptoms often worse at night or on waking Symptoms often triggered by exercise, laughter, allergens, cold air Symptoms often appear or worsen with viral infections Reduced FEV in 1 second/FVC compared with the LLN (> 0.75-0.80 in adults, > 0.90 in children) at a time when FEV in 1 second is reduced Documented expiratory airflow limitation Positive bronchodilator responsiveness test Documented excessive variability in lung function ( 1 of the following) Excessive variability in twice-daily peak expiratory flow over 2 weeks Significant increase in lung function after 4 weeks of anti-inflammatory treatment Positive exercise challenge test Positive bronchial challenge test (usually only for adults) https://web.pathway.md/diseases/recoid4eeyMVLDPrX 2/26 6/23/23, 3:13 AM Asthma Pathway Excessive variation in lung function between visits. As per CTS 2017 guidelines, confirm the diagnosis of asthma based on history and objective measures of lung function in patients old enough to reliably undergo pulmonary function tests. E Show 4 more 2. Classification and risk stratification Severity definitions: define severe asthma as asthma remaining uncontrolled despite optimized treatment with high-dose ICS-long-acting -agonist, or requiring high-dose ICS-long-acting - agonist to prevent it from becoming uncontrolled. Distinguish severe asthma from asthma that is difficult to treat due to inadequate or inappropriate treatment, or persistent problems with adherence or comorbidities, such as chronic rhinosinusitis or obesity. Show 2 more 3. Diagnostic investigations Initial assessment: As per GINA 2022 guidelines, assess symptom control, future risk of adverse outcomes, and treatment issues in adult, adolescent, and pediatric (aged 6-11 years) patients presenting with asthma: assess symptom control over the last 4 weeks identify any other risk factors for exacerbations, persistent airflow limitation, or side-effects measure lung function at diagnosis/initiation of treatment, 3-6 months after starting controller treatment, then periodically (at least once every 1-2 years), but more often in at-risk patients and patients with severe asthma document the patient's current treatment step watch inhaler technique, assess adherence and side-effects check that the patient has a written asthma action plan ask about the patient's attitudes and goals for their asthma and medications assess comorbidities contributing to symptoms and poor quality of life, and sometimes to poor asthma control (such as rhinitis, rhinosinusitis, GERD, obesity, obstructive sleep apnea, depression, and anxiety). As per SIGN 2019 guidelines, obtain a structured clinical assessment to assess the initial probability of asthma based on: history of recurrent episodes of symptoms, ideally corroborated by variable peak flows when symptomatic and asymptomatic symptoms of wheeze, cough, breathlessness, and chest tightness varying over time https://web.pathway.md/diseases/recoid4eeyMVLDPrX 3/26 6/23/23, 3:13 AM Asthma Pathway recorded observation of wheeze heard by a healthcare professional personal/family history of other atopic conditions (atopic dermatitis, allergic rhinitis) no symptoms/signs suggesting alternative diagnoses. B Pulmonary function testing: As per ERS 2022 guidelines: Obtain spirometry as part of the diagnostic work-up in adult patients with suspected asthma. B Avoid recording peak expiratory flow variability as the primary test to make a diagnosis of asthma. D As per GINA 2022 guidelines, obtain pulmonary function testing at diagnosis of asthma or initiation of treatment to assess. B Bronchial challenge testing: As per SIGN 2019 guidelines: Consider obtaining bronchial challenge testing in adult patients with no evidence of airflow obstruction on initial assessment and inconclusive results on other objective tests where asthma remains a possibility. C Obtain bronchial challenge testing to identify eosinophilic inflammation in adult and pediatric patients with an intermediate probability of asthma and normal spirometry results. B As per ERS 2012 guidelines, obtain specific bronchial challenge testing if the diagnosis of occupational asthma is not clear, the cause of occupational asthma is new, or it is necessary for the management of the individual worker. B Show 2 more FeNO testing: As per ERS 2022 guidelines, consider measuring fractional exhaled nitric oxide as part of the diagnostic work-up in adult patients with suspected asthma. C As per ATS 2021 guidelines, consider obtaining fractional exhaled nitric oxide measurement in addition to usual care in patients with asthma considered for treatment. C As per NHLBI 2020 guidelines, consider obtaining fractional exhaled nitric oxide measurement as an adjunct to the evaluation process of 5 years old patients, if the diagnosis of asthma is uncertain based on history, clinical findings, clinical course, and spirometry, including bronchodilator responsiveness testing, or if spirometry cannot be performed. C Show 2 more As per CTS 2017 guidelines: Obtain measurement of fractional exhaled nitric oxide levels where available to help characterize the phenotype in patients with confirmed severe asthma. E Insufficient evidence to support the use of fractional exhaled nitric oxide levels to predict response or responders to omalizumab or anti-IL-5 therapies. I Complete blood count: https://web.pathway.md/diseases/recoid4eeyMVLDPrX 4/26 6/23/23, 3:13 AM Asthma Pathway As per ERS 2022 guidelines, avoid measuring blood eosinophil count to make a diagnosis of asthma. D As per CTS 2017 guidelines: Obtain peripheral eosinophil count to help characterize the phenotype in patients with confirmed severe asthma. E Recognize that blood eosinophil counts may help to identify patients that will experience fewer exacerbations with anti-IL-5 therapies or omalizumab. E Serum immunoglobulin E: As per ERS 2022 guidelines, avoid measuring total serum IgE to make a diagnosis of asthma. D As per CTS 2017 guidelines: Obtain measurement of total serum IgE levels to help characterize the phenotype in patients with confirmed severe asthma. E Recognize that serum IgE does not predict response to anti-IL-5 therapies or omalizumab. E Sputum eosinophils: Obtain sputum eosinophils where available to help characterize the phenotype in patients with confirmed severe asthma. E Recognize that sputum eosinophils may help in identifying responders to anti-IL-5 therapies but not in identifying responders to macrolides. E Plethysmography: avoid measuring specific airway conductance and residual volume/total lung capacity by whole-body plethysmography to make a diagnosis of asthma. D Chest CT: consider obtaining HRCT in adult and pediatric patients with severe asthma only when the presentation is atypical. C Screening for occupational exposure: As per GINA 2022 guidelines, elicit work history and other exposures in all patients with adult- onset asthma. A As per ERS 2012 guidelines, ask adult patients with new, recurrent, or deteriorating symptoms of asthma, COPD, or rhinitis about their job, the materials with which they work, and whether they improve when away from work. A Show 4 more Screening for atopy: assess previous records of skin-prick tests, blood eosinophilia ( 4%), or elevated levels of allergen-specific IgE to corroborate a history of atopic status. Do not obtain these tests routinely as a diagnostic test for asthma. B Screening for food allergy: ask patients with asthma about symptoms associated with any specific foods. B Screening for COPD: assess smokers/former smokers (> 10 pack-year history) for COPD or overlapping features of asthma and COPD (asthma-COPD overlap). B Screening for alpha-1 antitrypsin deficiency: https://web.pathway.md/diseases/recoid4eeyMVLDPrX 5/26 6/23/23, 3:13 AM Asthma Pathway As per ERS 2017 guidelines, test for AAT deficiency in all patients with adult-onset asthma. E As per Alpha-1 2012 guidelines, avoid obtaining targeted testing for AAT deficiency in patients with asthma. D Screening for GERD: Consider suspecting GERD as a possible cause of a dry cough in patients with confirmed asthma, but do not obtain screening for GERD in patients with uncontrolled asthma. B Do not offer anti-reflux therapy in patients with poorly controlled asthma unless symptomatic GERD presents. D 4. Diagnostic procedures Bronchoalveolar lavage: consider performing bronchial challenge testing in secondary care to confirm a diagnosis of asthma in adult patients, if the diagnosis was not previously established in primary care. C 5. Medical management Treatment guidance: As per GINA 2022 guidelines, offer sputum-guided treatment in adult patients with moderate or severe asthma managed in (or referred to) centers experienced in this technique. A Show 2 more As per ERS 2014 guidelines, consider using sputum eosinophil counts, obtained in centers experienced in using this technique, in addition to clinical criteria to guide treatment in adult patients with severe asthma. C Show 2 more Stepwise management: Use the following track as the preferred stepwise approach to the management of asthma in adult and adolescent patients, with as-needed low-dose ICS-formoterol as the preferred reliever in all steps: Situation Guidance Offer as-needed low-dose ICS-formoterol B Steps 1-2. Symptoms < 4-5 days per week Offer low-dose maintenance ICS-formoterol A Step 3. Symptoms most days, or waking with asthma 1 per week Offer medium-dose maintenance ICS- formoterol B ; consider administering a short course of oral corticosteroids in Step 4. Daily symptoms, or waking with asthma 1 per week, and low lung function https://web.pathway.md/diseases/recoid4eeyMVLDPrX 6/26 6/23/23, 3:13 AM Asthma Pathway patients presenting with severely uncontrolled asthma A Offer add-on LAMAs Step 5. Refer for assessment of phenotype Consider offering high-dose maintenance ICS-formoterol Offer anti-IgE, anti-interleukin 5/5 receptor, anti-interleukin 4, and anti-thymic stromal lymphopoietin therapies A Use the following track as the alternative stepwise approach to the management of asthma in adult and adolescent patients, with an as-needed short-acting -agonist as the alternative reliever in all steps: Situation Guidance Offer ICS whenever short-acting -agonist is taken B Step 1. Symptoms < 2 days per month Offer low-dose maintenance ICSs A Step 2. Symptoms 2 days per month, but < 4-5 days per week Offer low-dose maintenance ICS-long-acting -agonist A Step 3. Symptoms most days, or waking with asthma 1 per week Offer medium/high-dose maintenance ICS- long-acting -agonist B ; consider administering a short course of oral corticosteroids in patients presenting with severely uncontrolled asthma A Step 4. Daily symptoms, or waking with asthma 1 per week, and low lung function Offer add-on LAMAs Step 5. Refer for assessment of phenotype Consider offering high-dose maintenance ICS-long-acting -agonist Offer anti-IgE, anti-interleukin 5/5 receptor, anti-interleukin 4, and anti-thymic stromal lymphopoietin therapies A Adjustment of treatment (assessment): review patients with asthma regularly, ideally 1-3 months after starting treatment and every 3-12 months thereafter, and within 1 week after an exacerbation, B to monitor symptom control, risk factors and occurrence of exacerbations, as well as to document the response to any treatment changes. https://web.pathway.md/diseases/recoid4eeyMVLDPrX 7/26 6/23/23, 3:13 AM Asthma Pathway Adjustment of treatment (stepping up): Step up treatment with the following adjustment options when asthma remains uncontrolled despite good adherence and inhaler technique: Situation Guidance Adjusting the number of as-needed doses of ICS-formoterol from day to day according to symptoms (by the patient), if the reliever inhaler is budesonide-formoterol or beclometasone-formoterol (with or without maintenance ICS-formoterol) Day-to-day adjustment Increasing the dose of maintenance ICS for 1-2 weeks (by the patient according to the written asthma action plan or by the healthcare provider), for example, during viral infections or seasonal allergen exposure Short-term step up (for 1-2 weeks) Increasing the low dose of maintenance ICS to medium when the symptoms are confirmed to be due to asthma, inhaler technique and adherence are satisfactory, and modifiable risk factors such as smoking have been addressed. Reduce the dose to the previous level if there is no response after 2-3 months, and consider offering alternative treatments or referring to a specialist center. Sustained step up (for at least 2-3 months) Adjustment of treatment (stepping down): consider stepping down treatment once asthma symptoms are well controlled and lung function has been stable for 3 months. C Show 4 more Landmark trials: SYGMA 2 In patients with mild asthma and were eligible for treatment with regular ICSs, budesonide and formoterol were noninferior to budesonide maintenance with respect to the percentage of patients achieving an improvement in ACQ-5 score. Bateman ED et al. N Engl J Med. 2018 May 17. Management of modifiable risk factors: Address potentially modifiable risk factors to reduce exacerbations in patients with asthma: Situation Guidance https://web.pathway.md/diseases/recoid4eeyMVLDPrX 8/26 6/23/23, 3:13 AM Asthma Pathway Ensure an ICS-containing controller is prescribed A ; prefer a maintenance and reliever therapy with ICS-formoterol to reduce the risk of severe exacerbations A ; provide a written action plan appropriate for the patient's health literacy A ; obtain more frequent monitoring than in low-risk patients A ; check inhaler technique and adherence frequently A ; identify any modifiable risk factors B Any patient with 1 risk factors for exacerbations, including poor symptom control Prefer ICS-formoterol maintenance and reliever regimen to reduce the risk of severe exacerbations A ; consider stepping up treatment if no modifiable risk factors present A ; identify any avoidable triggers for exacerbations B 1 severe exacerbation in last year Encourage smoking cessation by patient/family Exposure to tobacco smoke Provide counseling and resources A ; consider offering higher-dose of ICSs if asthma is poorly controlled B Consider offering a trial of high-dose ICSs for 3 months B ; consider administering oral corticosteroids for 2 weeks, taking short- and long-term risks into account B ; exclude other lung diseases, such as COPD B ; refer for expert advice if there is no improvement B Low FEV in 1 second, especially if < 60% of predicted Offer weight reduction strategies B ; distinguish asthma symptoms from symptoms due to deconditioning, mechanical restriction, and/or sleep apnea B Obesity Obtain mental health assessment Major psychological problems Help the patient to distinguish between symptoms of anxiety and asthma Counsel about the management of panic attacks B Identify the most cost-effective ICS-based regimen B Major socioeconomic problems https://web.pathway.md/diseases/recoid4eeyMVLDPrX 9/26 6/23/23, 3:13 AM Asthma Pathway Confirmed food allergy Advise appropriate food avoidance Prescribe injectable epinephrine A Advise a trial of simple avoidance strategies, taking the cost into account B ; consider stepping up controller treatment B ; consider adding subcutaneous immunotherapy in symptomatic adult house dust mite-sensitive patients with allergic rhinitis despite ICSs, provided FEV in 1 second is > 70% of predicted B Allergen exposure if sensitized Increase ICS dose independent of the level of symptom control A Sputum eosinophilia Management of exacerbation, self-management: Include the following short-term self- management strategies for worsening asthma in the written asthma action plan: low-dose ICS-formoterol as reliever: increase the frequency of as-needed ICS-formoterol A short-acting -agonist as reliever: increase the frequency of short-acting -agonist use; add spacer for pressurized metered-dose inhaler A maintenance and reliever ICS-formoterol: continue maintenance ICS-formoterol and increase reliever ICS-formoterol as needed A maintenance ICS with short-acting -agonist as reliever: increase ICS dose 4 times in adult and adolescent patients B maintenance ICS-formoterol with short-acting -agonist as reliever: increase maintenance ICS- formoterol dose 4 times B maintenance ICS plus other long-acting -blocker with short-acting -agonist as reliever: step up to higher dose formulation of ICS plus other long-acting -agonist B ; consider adding a separate ICS inhaler to increase ICS dose 4 times in adult patients B addition of oral corticosteroids: add oral corticosteroids for severe exacerbations, such as peak expiratory flow or FEV in 1 second < 60% personal best or predicted, or not responding to treatment > 48 hours, preferably with morning dosing A administer prednisolone 40-50 mg/day, usually for 5-7 days in adult patients, and 1-2 mg/kg/day, maximum 40 mg, usually for 3-5 days in pediatric patients aged 6-11 years B do not taper if administered for < 2 weeks B Landmark trials: FAST In adult and adolescent patients with asthma who were receiving ICSs, with or without add-on therapy, and who had at least one exacerbation in the previous 12 months, quadrupling was superior to non-quadrupling with respect to use of systemic corticosteroids. McKeever T et al. N Engl J Med. 2018 Mar 8. https://web.pathway.md/diseases/recoid4eeyMVLDPrX 10/26 6/23/23, 3:13 AM Asthma Pathway Management of exacerbation, primary care setting (evaluation): Elicit brief focused history, perform a relevant physical examination, and obtain objective measurements, concurrently with the prompt initiation of therapy, in patients with asthma exacerbation presenting to primary care: Situation Guidance Timing of onset and cause (if known) of the present exacerbation History Severity of asthma symptoms, including any limiting exercise or disturbing sleep Any symptoms of anaphylaxis Any risk factors for asthma-related death All current reliever and controller medications, including doses and devices prescribed, adherence pattern, any recent dose changes, and response to current therapy Signs of exacerbation severity and vital signs (level of consciousness, temperature, HR, respiratory rate, BP, ability to complete sentences, use of accessory muscles, wheeze) Physical examination Complicating factors (such as anaphylaxis, pneumonia, pneumothorax) Signs of alternative conditions explaining acute breathlessness (such as cardiac failure, inducible laryngeal obstruction, inhaled foreign body, or pulmonary embolism) Pulse oximetry (saturation levels < 90% in signal the need for aggressive therapy in pediatric and adult patients) Objective measurements Peak expiratory flow in > 5 years old patients. Management of exacerbation, primary care setting (oxygen therapy): Administer oxygen therapy titrated against pulse oximetry (if available) to maintain oxygen saturation at 93-95% (94-98% for pediatric patients aged 6-11 years). A Do not withhold oxygen if oximetry is not available, instead monitor patients for deterioration, somnolence, or fatigue because of the risk of hypercapnia and respiratory failure. https://web.pathway.md/diseases/recoid4eeyMVLDPrX 11/26 6/23/23, 3:13 AM Asthma Pathway Management of exacerbation, primary care setting (SABA): Administer inhaled short-acting -agonists up to 4-10 puffs every 20 minutes for the first hour to achieve rapid reversal of airflow limitation in patients with mild-to-moderate exacerbations. A Do not administer additional short-acting -agonists if there is a good response to initial treatment, such as peak expiratory flow > 60-80% of predicted or personal best for 3-4 hours. Recognize that delivery of short-acting -agonists via a pressurized-metered dose inhaler and spacer or a dry-powdered inhaler leads to a similar improvement in lung function as delivery via nebulizer, A although the most cost-effective route of delivery is pressurized-metered dose inhaler and spacer. Management of exacerbation, primary care setting (oral corticosteroids): administer oral corticosteroids (prednisolone 1 mg/kg/day or equivalent up to a maximum of 50 mg/day, usually for 5-7 days in adult patients, and 1-2 mg/kg/day up to a maximum of 40 mg/day, usually for 3-5 days in pediatric patients aged 6-11 years), especially if the patient is deteriorating, or had already increased reliever and controller doses before the presentation. B Management of exacerbation, primary care setting (inhaled corticosteroids): counsel patients already prescribed controller medication about increasing the dose for the next 2-4 weeks. Initiate regular ICS-containing therapy in patients not currently taking controller medication. Management of exacerbation, primary care setting (antibiotics): do not use antibiotics routinely in the treatment of acute asthma exacerbations unless there is strong evidence of lung infection (such as fever and purulent sputum, or radiographic evidence of pneumonia). Management of exacerbation, primary care setting (monitoring and discharge): Obtain close monitoring during exacerbation treatment, and titrate treatment according to the response. Transfer patients immediately to an acute care facility when presenting with signs of a severe or life-threatening exacerbation not responding to treatment or continuing to deteriorate. Include as-needed reliever medication (low dose ICSs-formoterol or short-acting -agonists), a short course of oral corticosteroids, and regular controller treatment in the discharge medications. Do not prescribe short-acting -agonist-only treatment. Review inhaler technique and adherence before discharge. Advise patients to use their reliever inhaler only as-needed, rather than routinely. Schedule a follow-up appointment for 2-7 days later, depending on the clinical and social context. Management of exacerbation, emergency department setting (evaluation): obtain pulmonary function testing in patients with asthma exacerbation presenting to the emergency department. Document peak expiratory flow or FEV in 1 second, if possible and without unduly delaying treatment, before initiating treatment. Monitor lung function at 1 hour and at intervals until a clear response to treatment has occurred or a plateau is reached. Show 3 more Management of exacerbation, emergency department setting (SABA): https://web.pathway.md/diseases/recoid4eeyMVLDPrX 12/26 6/23/23, 3:13 AM Asthma Pathway Administer frequent inhaled short-acting -agonists in patients presenting with acute asthma. Recognize that the most cost-effective and efficient delivery is by pressurized metered-dose inhalers with a spacer. A Insufficient evidence to support the routine use of IV -2 agonists in patients with severe asthma exacerbations. I Management of exacerbation, emergency department setting (systemic corticosteroids): administer systemic corticosteroids in acute care settings to speed resolution of exacerbations and prevent relapses in all adult, adolescent, and pediatric (6-11 years of age) patients with asthma exacerbation, except for the mildest exacerbations. A Show 2 more Management of exacerbation, emergency department setting (inhaled corticosteroids): Administer high-dose ICSs within the first hour of presentation to the emergency department to reduce the need for hospitalization in patients not receiving systemic corticosteroids. A Consider administering ICSs, with or without concomitant systemic corticosteroids, within the first hours of presentation to the emergency department to reduce the risk of hospital admission and the need for systemic corticosteroids in pediatric patients. C Management of exacerbation, emergency department setting (oxygen therapy): administer controlled low-flow oxygen therapy (by nasal cannulae or mask) using pulse oximetry to maintain saturation at 93-95% in patients with severe exacerbations (94-98% in pediatric patients aged 6- 11 years). B Show 3 more Management of exacerbation, emergency department setting (other treatments): consider administering ipratropium in addition to short-acting -agonists for the management of moderate- to-severe exacerbations in the emergency department, to reduce hospitalizations in adult, B adolescent, and pediatric patients C and to improve peak expiratory flow and FEV in 1 second in adolescent and adult patients. B Show 2 more Management of exacerbation, emergency department setting (monitoring and discharge): on discharge home: Prescribe ongoing ICS-containing treatment since the occurrence of a severe exacerbation is a risk factor for future exacerbations , and ICS-containing medications significantly reduce the risk of asthma-related death or hospitalization (Evidence A). short-acting -agonist- only treatment of asthma is no longer recommended. For short-term outcomes such as relapse requiring admission, symptoms, and quality of life, a systematic review found no significant differences when ICSs were added to systemic corticosteroids after discharge. There was some evidence, however, that post-discharge ICSs were as effective as systemic corticosteroids for milder exacerbations, but the confidence limits were wide. (Evidence B). Cost may be a significant factor for patients in the use of high-dose ICSs, and further studies are required to establish their role. B Allergen immunotherapy: As per GINA 2022 guidelines: https://web.pathway.md/diseases/recoid4eeyMVLDPrX 13/26 6/23/23, 3:13 AM Asthma Pathway Consider offering subcutaneous immunotherapy in adult patients with allergic rhinitis and sensitized to house dust mite, with persisting asthma symptoms despite low-to-medium-dose ICS-containing therapy, provided FEV in 1 second is > 70% of predicted. C Weigh potential benefits of subcutaneous immunotherapy against the risk of adverse effects and the inconvenience and cost of the prolonged course of therapy, including the minimum half-hour wait required after each injection. B As per NHLBI 2020 guidelines: Consider offering subcutaneous immunotherapy as an adjunct treatment to standard pharmacotherapy in 5 years old patients with mild-to-moderate allergic asthma controlled at the initiation, build-up, and maintenance phases of immunotherapy. C Avoid using sublingual immunotherapy in patients with persistent allergic asthma. D Long-term macrolides: As per GINA 2022 guidelines, consider offering add-on azithromycin (3 times per week, for at least 6 months) after specialist referral in adult patients with persistent symptomatic asthma despite high dose ICSs-long-term -agonists. Assess sputum for atypical mycobacteria and obtain an ECG for long QTc before initiation (and reassess after a month on treatment), taking into account the risk of increasing antimicrobial resistance. C As per BTS 2020 guidelines: Consider offering oral macrolide therapy for a minimum of 6-12 months to reduce exacerbation frequency in adult (50-70 years) patients with ongoing symptoms despite > 80% adherence to high-dose ICSs (> 800 g/day) and at least one exacerbation requiring oral corticosteroids in the past year. C Do not offer oral macrolide therapy as a way to reduce oral corticosteroid dose, recognizing that in some patients this may result as a consequence of a reduction in exacerbations or symptoms. D Landmark trials: AMAZES In patients with uncontrolled persistent asthma on medium-to-high dose ICS plus a long- acting bronchodilator, oral azithromycin was superior to placebo with respect to total asthma exacerbations. Gibson PG et al. Lancet. 2017 Aug 12. As per CTS 2017 guidelines, consider offering macrolides to reduce the frequency of exacerbations in 18 years old patients with severe asthma. E Show 3 more As per ERS 2014 guidelines, avoid using macrolide antibiotics in adult and pediatric patients with severe asthma. D Biologic therapy: https://web.pathway.md/diseases/recoid4eeyMVLDPrX 14/26 6/23/23, 3:13 AM Asthma Pathway As per GINA 2022 guidelines, offer add-on anti-IgE therapy (omalizumab) in 6 years old patients with moderate-to-severe allergic asthma uncontrolled on step 4-5 treatment. A Show 3 more As per CTS 2017 guidelines, consider offering omalizumab in 6 years old patients with severe asthma meeting the following criteria: inadequate disease control despite high-dose ICSs and at least one other controller sensitization to at least one perennial allergen serum IgE levels of 30-1,300 IU/mL (for children aged 6-11 years) or 30-700 IU/mL (for adults and adolescents aged 12 years). E Show 8 more As per ERS 2014 guidelines, consider offering a therapeutic trial of omalizumab in adult C and pediatric patients with severe allergic asthma. C Landmark trials: EXTRA In patients with inadequately controlled severe asthma who are receiving high-dose ICS and long-acting -agonists, with or without additional controller therapy, omalizumab was superior to placebo with respect to asthma exacerbations at 48 weeks. Hanania NA et al. Ann Intern Med. 2011 May 3. Oral corticosteroids: As per GINA 2022 guidelines: Offer add-on low-dose oral corticosteroids ( 7.5 mg/day prednisone equivalent) as a last resort only in selected adult patients with severe asthma with poor symptom control and/or frequent exacerbations despite good inhaler technique and adherence with step 5 treatment, and after exclusion of other contributory factors and other add-on treatments including biologics (where available and affordable). B Counsel patients about potential side effects, and assess and monitor for risk of adrenal suppression and corticosteroid-induced osteoporosis. Provide relevant lifestyle counseling and prescription of therapy for prevention of osteoporosis (where appropriate) in patients expected to be treated for 3 months. As per SIGN 2019 guidelines, offer daily oral corticosteroids (at the lowest dose providing adequate control) in selected patients not controlled on high-dose therapies. B Immunosuppressants: as per ERS 2014 guidelines, avoid using methotrexate in adult and pediatric patients with severe asthma. D 6. Nonpharmacologic interventions Smoking cessation: https://web.pathway.md/diseases/recoid4eeyMVLDPrX 15/26 6/23/23, 3:13 AM Asthma Pathway As per GINA 2022 guidelines, advise smoking cessation at every visit in smoker patients with asthma. Provide access to counseling and smoking cessation programs if available. A Show 2 more As per SIGN 2019 guidelines, counsel patients with asthma and parents/carers of pediatric patients with asthma about the dangers of smoking and second-hand tobacco smoke exposure, and advise quitting smoking. B Physical activity: encourage patients with asthma to engage in regular physical activity for its general health benefits, A including improvement in cardiopulmonary fitness as well as for a small benefit for asthma control and lung function, including with swimming in young patients with asthma. B Show 3 more Dietary modifications: advise patients with asthma to follow a diet high in fruit and vegetables for its general health benefits. A Show 2 more Weight loss: As per GINA 2022 guidelines: Include weight reduction in the treatment plan of patients with asthma and obesity. B Advise a weight reduction program combined with twice-weekly aerobic and strength exercises for symptom control in adult patients with asthma and obesity. B As per SIGN 2019 guidelines, consider offering weight-loss interventions (including dietary and exercise-based programs) to improve asthma control in adult and pediatric patients with overweight or obesity. C Breathing exercises: As per GINA 2022 guidelines, consider offering breathing exercises as an adjunct to pharmacotherapy to improve symptoms and quality of life, but not for reducing exacerbation risk or improving lung function. B As per SIGN 2019 guidelines, consider offering breathing exercise programs (including face-to- face physiotherapist-delivered methods and audiovisual programs) as an adjuvant to pharmacotherapy to improve quality of life and reduce symptoms in adult patients with asthma. B Stress management: encourage patients to identify goals and strategies to deal with emotional stress if it makes their asthma worse. B Show 2 more Vitamin D supplements: insufficient evidence to support the use of vitamin D supplements for improving disease control or reducing exacerbations in patients with asthma. I Avoidance of indoor allergens: As per GINA 2022 guidelines, do not advise routine allergen avoidance in patients with asthma. D Show 2 more https://web.pathway.md/diseases/recoid4eeyMVLDPrX 16/26 6/23/23, 3:13 AM Asthma Pathway As per NHLBI 2020 guidelines, avoid offering allergen mitigation interventions as part of routine asthma management in patients with asthma not having sensitization to specific indoor allergens or not having symptoms related to exposure to specific indoor allergens. D Show 2 more As per SIGN 2019 guidelines: Do not advise using routine physical or chemical methods of reducing house dust mite levels in the home (including acaricides, mattress covers, vacuum cleaning, heating, ventilation, freezing, washing, air filtration, and ionizers) for the management of asthma. D Do not advise using air ionizers for the treatment of asthma. D Avoidance of outdoor allergens: advise reducing exposure to outdoor allergens in sensitized patients by closing windows and doors, remaining indoors, and using air conditioning when pollen and mold counts are highest. B Avoidance of weather conditions: consider advising staying indoors in a climate-controlled environment and avoiding strenuous outdoor physical activity during unfavorable weather conditions (such as very cold weather or low humidity). Advise avoiding polluted environments during viral infections, if feasible. C Avoidance of air pollution: Advise using non-polluting heating and cooking sources, and vent sources of pollutants outdoors where possible. B Consider advising staying indoors in a climate-controlled environment and avoiding strenuous outdoor physical activity during high air pollution. Advise avoiding polluted environments during viral infections, if feasible. C Avoidance of occupational exposure: As per GINA 2022 guidelines: Identify and eliminate occupational sensitizers as soon as possible and remove any further exposure to these agents in the management of patients with occupational asthma. A Advise using non-powdered low-allergen gloves instead of powdered latex gloves to minimize latex sensitization. A As per SIGN 2019 guidelines, advise relocation away from exposure as soon as the diagnosis of work-related asthma is confirmed, ideally within 12 months of the first work-related symptoms of asthma. B As per ERS 2012 guidelines, inform patients: the risk of work-related asthma is higher in case of atopy or preexisting asthma or pre- employment sensitization B persistence of exposure to the causal agent is likely to result in a deterioration of asthma symptoms and airway obstruction B complete avoidance of exposure is associated with the highest probability of improvement, but may not lead to a complete recovery from asthma. B Show 4 more https://web.pathway.md/diseases/recoid4eeyMVLDPrX 17/26 6/23/23, 3:13 AM Asthma Pathway Avoidance of exacarbating medications: ask patients about asthma and previous reactions always before prescribing NSAIDs, and advise discontinuing them if asthma worsens. B Recognize that aspirin and NSAIDs are not generally contraindicated unless there is a history of previous reactions to these agents. A Show 3 more 7. Therapeutic procedures Bronchial thermoplasty: As per GINA 2022 guidelines, consider offering bronchial thermoplasty, if available, as step 5 in adult patients with uncontrolled asthma despite optimized therapeutic regimens and referral to an asthma specialty center. C As per NHLBI 2020 guidelines, consider offering bronchial thermoplasty in 18 years old patients with persistent asthma placing a low value on harms (short-term worsening symptoms and unknown long-term side effects) and a high value on potential benefits (improvement in quality of life, a small reduction in exacerbations). C As per CTS 2017 guidelines: Insufficient evidence regarding the precise role of bronchial thermoplasty in 18 years old patients with severe asthma. I Perform bronchial thermoplasty in highly specialized centers because of the complexity of the procedure and the occurrence of severe events, such as hospitalizations due to asthma worsening, atelectasis, and pneumonia. E As per ERS 2014 guidelines, offer bronchial thermoplasty in adult patients with severe asthma only in the context of an IRB-approved independent systematic registry or a clinical trial. B 8. Perioperative care Perioperative management: Ensure achieving good asthma control preoperatively in patients scheduled for elective surgery, especially in patients with more severe asthma, uncontrolled symptoms, exacerbation history, or persistent airflow limitation. B
Counsel patients about potential side effects, and assess and monitor for risk of adrenal suppression and corticosteroid-induced osteoporosis. Provide relevant lifestyle counseling and prescription of therapy for prevention of osteoporosis (where appropriate) in patients expected to be treated for 3 months. As per SIGN 2019 guidelines, offer daily oral corticosteroids (at the lowest dose providing adequate control) in selected patients not controlled on high-dose therapies. B Immunosuppressants: as per ERS 2014 guidelines, avoid using methotrexate in adult and pediatric patients with severe asthma. D 6. Nonpharmacologic interventions Smoking cessation: https://web.pathway.md/diseases/recoid4eeyMVLDPrX 15/26 6/23/23, 3:13 AM Asthma Pathway As per GINA 2022 guidelines, advise smoking cessation at every visit in smoker patients with asthma. Provide access to counseling and smoking cessation programs if available. A Show 2 more As per SIGN 2019 guidelines, counsel patients with asthma and parents/carers of pediatric patients with asthma about the dangers of smoking and second-hand tobacco smoke exposure, and advise quitting smoking. B Physical activity: encourage patients with asthma to engage in regular physical activity for its general health benefits, A including improvement in cardiopulmonary fitness as well as for a small benefit for asthma control and lung function, including with swimming in young patients with asthma. B Show 3 more Dietary modifications: advise patients with asthma to follow a diet high in fruit and vegetables for its general health benefits. A Show 2 more Weight loss: As per GINA 2022 guidelines: Include weight reduction in the treatment plan of patients with asthma and obesity. B Advise a weight reduction program combined with twice-weekly aerobic and strength exercises for symptom control in adult patients with asthma and obesity. B As per SIGN 2019 guidelines, consider offering weight-loss interventions (including dietary and exercise-based programs) to improve asthma control in adult and pediatric patients with overweight or obesity. C Breathing exercises: As per GINA 2022 guidelines, consider offering breathing exercises as an adjunct to pharmacotherapy to improve symptoms and quality of life, but not for reducing exacerbation risk or improving lung function. B As per SIGN 2019 guidelines, consider offering breathing exercise programs (including face-to- face physiotherapist-delivered methods and audiovisual programs) as an adjuvant to pharmacotherapy to improve quality of life and reduce symptoms in adult patients with asthma. B Stress management: encourage patients to identify goals and strategies to deal with emotional stress if it makes their asthma worse. B Show 2 more Vitamin D supplements: insufficient evidence to support the use of vitamin D supplements for improving disease control or reducing exacerbations in patients with asthma. I Avoidance of indoor allergens: As per GINA 2022 guidelines, do not advise routine allergen avoidance in patients with asthma. D Show 2 more https://web.pathway.md/diseases/recoid4eeyMVLDPrX 16/26 6/23/23, 3:13 AM Asthma Pathway As per NHLBI 2020 guidelines, avoid offering allergen mitigation interventions as part of routine asthma management in patients with asthma not having sensitization to specific indoor allergens or not having symptoms related to exposure to specific indoor allergens. D Show 2 more As per SIGN 2019 guidelines: Do not advise using routine physical or chemical methods of reducing house dust mite levels in the home (including acaricides, mattress covers, vacuum cleaning, heating, ventilation, freezing, washing, air filtration, and ionizers) for the management of asthma. D Do not advise using air ionizers for the treatment of asthma. D Avoidance of outdoor allergens: advise reducing exposure to outdoor allergens in sensitized patients by closing windows and doors, remaining indoors, and using air conditioning when pollen and mold counts are highest. B Avoidance of weather conditions: consider advising staying indoors in a climate-controlled environment and avoiding strenuous outdoor physical activity during unfavorable weather conditions (such as very cold weather or low humidity). Advise avoiding polluted environments during viral infections, if feasible. C Avoidance of air pollution: Advise using non-polluting heating and cooking sources, and vent sources of pollutants outdoors where possible. B Consider advising staying indoors in a climate-controlled environment and avoiding strenuous outdoor physical activity during high air pollution. Advise avoiding polluted environments during viral infections, if feasible. C Avoidance of occupational exposure: As per GINA 2022 guidelines: Identify and eliminate occupational sensitizers as soon as possible and remove any further exposure to these agents in the management of patients with occupational asthma. A Advise using non-powdered low-allergen gloves instead of powdered latex gloves to minimize latex sensitization. A As per SIGN 2019 guidelines, advise relocation away from exposure as soon as the diagnosis of work-related asthma is confirmed, ideally within 12 months of the first work-related symptoms of asthma. B As per ERS 2012 guidelines, inform patients: the risk of work-related asthma is higher in case of atopy or preexisting asthma or pre- employment sensitization B persistence of exposure to the causal agent is likely to result in a deterioration of asthma symptoms and airway obstruction B complete avoidance of exposure is associated with the highest probability of improvement, but may not lead to a complete recovery from asthma. B Show 4 more https://web.pathway.md/diseases/recoid4eeyMVLDPrX 17/26 6/23/23, 3:13 AM Asthma Pathway Avoidance of exacarbating medications: ask patients about asthma and previous reactions always before prescribing NSAIDs, and advise discontinuing them if asthma worsens. B Recognize that aspirin and NSAIDs are not generally contraindicated unless there is a history of previous reactions to these agents. A Show 3 more 7. Therapeutic procedures Bronchial thermoplasty: As per GINA 2022 guidelines, consider offering bronchial thermoplasty, if available, as step 5 in adult patients with uncontrolled asthma despite optimized therapeutic regimens and referral to an asthma specialty center. C As per NHLBI 2020 guidelines, consider offering bronchial thermoplasty in 18 years old patients with persistent asthma placing a low value on harms (short-term worsening symptoms and unknown long-term side effects) and a high value on potential benefits (improvement in quality of life, a small reduction in exacerbations). C As per CTS 2017 guidelines: Insufficient evidence regarding the precise role of bronchial thermoplasty in 18 years old patients with severe asthma. I Perform bronchial thermoplasty in highly specialized centers because of the complexity of the procedure and the occurrence of severe events, such as hospitalizations due to asthma worsening, atelectasis, and pneumonia. E As per ERS 2014 guidelines, offer bronchial thermoplasty in adult patients with severe asthma only in the context of an IRB-approved independent systematic registry or a clinical trial. B 8. Perioperative care Perioperative management: Ensure achieving good asthma control preoperatively in patients scheduled for elective surgery, especially in patients with more severe asthma, uncontrolled symptoms, exacerbation history, or persistent airflow limitation. B Weigh the risks of performing surgery without achieving good asthma control against the need for immediate surgery in patients requiring emergency surgery. Administer perioperative hydrocortisone in patients on long-term high-dose ICSs or receiving oral corticosteroids for > 2 weeks during the previous 6 months, because of the risk of adrenal crisis in the context of surgery. B 9. Specific circumstances https://web.pathway.md/diseases/recoid4eeyMVLDPrX 18/26 6/23/23, 3:13 AM Asthma Pathway Pediatric patients (diagnosis): do not diagnose asthma solely based on symptoms D or improvement in symptoms after a trial of preventer medication. D Pediatric patients (pulmonary function testing): obtain spirometry as part of the diagnostic evaluation of 5-16 years old patients with suspected asthma. B Show 2 more Pediatric patients (FeNO testing): obtain measurement of fractional exhaled nitric oxide as part of the diagnostic evaluation of 5-16 years old patients with suspected asthma. B Pediatric patients (allergen testing): Do not obtain skin prick tests for aeroallergens in the diagnostic evaluation of asthma. D Do not obtain serum total or specific IgE tests in the diagnostic evaluation of asthma. D Pediatric patients (bronchial challenge tests): Obtain a direct bronchial challenge test using methacholine for the evaluation of asthma in 5-16 years old patients, if asthma diagnosis could not be confirmed with first-line objective tests. B Obtain an indirect bronchial challenge test using a treadmill or a bicycle for the evaluation of asthma with exercise-related symptoms in 5-16 years old patients, if asthma diagnosis could not be confirmed with first-line objective tests. B Pediatric patients (indications for treatment): Consider offering watchful waiting in asymptomatic pediatric patients with an intermediate probability of asthma unable to perform spirometry. C Offer a carefully monitored initiation of treatment in symptomatic pediatric patients with an intermediate probability of asthma unable to perform spirometry. B Pediatric patients, treatment guidance: As per GINA 2022 guidelines, offer fractional exhaled nitric oxide-guided treatment to reduce exacerbation rates in pediatric patients with asthma. A As per BTS/SIGN 2019 guidelines, do not use pulmonary function measurements to guide management in < 5 years old patients with asthma. D As per ERS/ATS 2014 guidelines, consider using clinical criteria alone rather than clinical criteria and sputum eosinophil counts to guide treatment in pediatric patients with severe asthma. C Pediatric patients (stepwise management): Use the following track as the preferred stepwise approach to the management of asthma in pediatric patients aged 6-11 years, with an as-needed short-acting -agonist or low-dose ICS-formoterol as the preferred reliever in all steps: Situation Guidance Offer low-dose ICS whenever short-acting - agonist is taken A Steps 1. Symptoms < 2 days per month Step 2. Symptoms 2 days, but less than daily Offer low-dose ICS daily A Offer low-dose ICS-long-acting -agonist Step 3. Symptoms most days, or waking with asthma 1 per week https://web.pathway.md/diseases/recoid4eeyMVLDPrX 19/26 6/23/23, 3:13 AM Asthma Pathway Offer medium-dose ICS A ; offer very low- dose ICS-formoterol maintenance and reliever B Offer medium-dose maintenance ICS-long- acting -agonist Step 4. Symptoms most days, or waking with asthma 1 per week, and low lung function Offer low-dose ICS-formoterol maintenance and reliever therapy Refer for expert advice Consider administering a short course of oral corticosteroids in patients presenting with severely uncontrolled asthma Refer for phenotypic assessment Step 5. Offer higher-dose ICS-long-acting -agonist Offer add-on therapy with anti-IgE and anti- interleukin 4 receptor therapies A Pregnant patients: recognize that the advantages of actively treating asthma in pregnancy markedly outweigh any potential risks of usual controller and reliever medications. A Show 2 more Perimenstrual patients: consider prescribing oral contraceptives and/or leukotriene receptor antagonists in addition to the usual strategies for asthma treatment in perimenstrual patients. C Athletes: Discuss preventative measures to avoid high exposure to air pollutants, allergens (if sensitized), and chlorine levels in pools, particularly during training periods, with athletes. B Advise avoiding training in extreme cold or pollution. B Patients with exercise-induced bronchoconstriction: As per GINA 2022 guidelines, counsel patients with asthma about the prevention of exercise- induced bronchoconstriction with regular ICSs. A Show 2 more As per SIGN 2019 guidelines: Consider adding any of the following if exercise is a specific problem in otherwise well- controlled patients taking ICSs: leukotriene receptor antagonist long-acting -agonists sodium cromoglicate or nedocromil sodium theophylline. C Offer inhaled short-acting -agonists immediately before exercise. A Patients with aspirin-exacerbated respiratory disease: https://web.pathway.md/diseases/recoid4eeyMVLDPrX 20/26 6/23/23, 3:13 AM Asthma Pathway Advise avoiding aspirin or NSAID-containing products and other medications inhibiting COX-1 in patients with aspirin-exacerbated respiratory disease. Consider preferring a COX-2 inhibitor (such as celecoxib or etoricoxib) or acetaminophen, with appropriate healthcare provider supervision and observation for at least 2 hours after administration, when an NSAID is indicated for other medical conditions. B Administer ICSs as the mainstay of asthma treatment in patients with aspirin-exacerbated respiratory disease, recognizing that oral corticosteroids are sometimes required and leukotriene receptor antagonists may also be useful. B Patients with chronic cough: consider obtaining noninvasive measurement of airway inflammation in adult and adolescent patients with chronic cough due to asthma. Recognize that the presence of eosinophilic airway inflammation is likely to be associated with a more favorable response to corticosteroids. C Show 3 more Patients with allergic bronchopulmonary aspergillosis: As per SIGN 2019 guidelines, consider offering a 4-month trial of itraconazole in adult patients with allergic bronchopulmonary aspergillosis. C As per IDSA 2016 guidelines, consider initiating oral itraconazole with therapeutic drug monitoring in symptomatic patients with asthma and bronchiectasis or mucoid impaction despite oral or ICS therapy. C As per ERS 2014 guidelines: Consider initiating antifungal agents in adult patients with severe asthma and recurrent exacerbations of allergic bronchopulmonary aspergillosis. C Avoid using antifungal agents for the treatment of asthma in adult and pediatric patients with severe asthma without allergic bronchopulmonary aspergillosis, irrespective of sensitization to fungi (positive skin prick test or fungus-specific IgE in serum). D Patients with cardiac arrest: obtain prompt evaluation for tension pneumothorax in patients with asthma with cardiac arrest, sudden elevation in peak inspiratory pressures, or difficulty ventilating. B Show 2 more 10. Patient education Self-management education: as per GINA 2022 guidelines, establish a partnership between the patients with asthma (or the parent/carer) and healthcare providers for effective asthma management, and allow the patients to gain the knowledge, confidence, and skills to assume a major role in the management of their asthma. Recognize that self-management education reduces asthma morbidity in both adult and pediatric patients. A Show 2 more Asthma action plans: As per GINA 2022 guidelines: https://web.pathway.md/diseases/recoid4eeyMVLDPrX 21/26 6/23/23, 3:13 AM Asthma Pathway Include both a step up in ICSs and the addition of oral corticosteroids, and peak expiratory flow-based plans (based on personal best rather than percent predicted peak expiratory flow) in written asthma action plans. A Recognize that the efficacy of self-management education is similar regardless of whether patients self-adjust their medications according to an individual written plan or whether the medication adjustments are made by a doctor. A 11. Preventative measures Avoidance of occupational exposure: eliminate exposure as the best preventive measure to reduce the disease burden of work-related asthma and the preferred approach for primary prevention. A Show 3 more Avoidance of smoke exposure: advise current and prospective parents regarding the many adverse effects of smoking on their children, including increased wheezing in infancy and increased risk of persistent asthma. B Weight loss: Advise weight reduction in patients with obesity to promote general health and to reduce subsequent respiratory symptoms consistent with asthma. B Offer weight-loss programs in pediatric patients with overweight or obesity to reduce the likelihood of respiratory symptoms suggestive of asthma. B Breastfeeding: As per GINA 2022 guidelines: Recognize that breastfeeding decreases wheezing episodes in early life but does not prevent the development of persistent asthma. B Encourage breastfeeding for all of its other positive benefits regardless of its effect on the development of asthma. A As per SIGN 2019 guidelines, encourage breastfeeding for its various benefits, including a potential protective effect in relation to early asthma. B Routine immunizations: offer annual influenza vaccination in patients with moderate-to-severe asthma. B Show 2 more 12. Follow-up and surveillance Indications for specialist referral: as per GINA 2022 guidelines, refer for expert advice in the following cases, where available: Situation Guidance https://web.pathway.md/diseases/recoid4eeyMVLDPrX 22/26 6/23/23, 3:13 AM Asthma Pathway Difficulty confirming the diagnosis of asthma Symptoms of chronic infection, or features suggesting a cardiac or other nonpulmonary cause (immediate referral) Diagnosis is unclear even after a trial of therapy with inhaled or systemic corticosteroids Features of both asthma and COPD, if there is doubt about priorities for treatment Refer for confirmatory testing and identification of sensitizing or irritant agent, specific advice about eliminating exposure, and pharmacological treatment Suspected occupational asthma Uncontrolled symptoms, ongoing exacerbations, or low lung function despite correct inhaler (medium-dose ICS-long- acting -agonist) technique and good adherence (identify and treat modifiable risk factors and comorbidities before referral, depending on the clinical context) Persistent or severely uncontrolled asthma or frequent exacerbations Frequent asthma-related healthcare utilization (such as multiple emergency department visits or urgent primary care visits) Near-fatal asthma attack (ICU admission, or mechanical ventilation for asthma) at any time in the past Any risk factors for asthma-related death Suspected or confirmed anaphylaxis or food allergy Significant side effects from treatment Evidence of, or risk of, significant treatment side effects Need for long-term oral corticosteroid use Frequent courses of oral corticosteroids ( 2 courses per year) Aspirin-exacerbated respiratory disease Symptoms suggesting complications or subtypes of asthma Allergic bronchopulmonary aspergillosis Doubts about the diagnosis of asthma (such as respiratory symptoms not responding well to treatment in a prematurely born child) Additional reasons for referral in pediatric patients aged 6-11 years Symptoms or exacerbations remain uncontrolled despite medium-dose ICSs with https://web.pathway.md/diseases/recoid4eeyMVLDPrX 23/26 6/23/23, 3:13 AM Asthma Pathway correct inhaler technique and good adherence Suspected side effects of treatment (such as growth delay) Concerns about the patient's welfare or well- being. Serial pulmonary function testing: obtain pulmonary function testing after 3-6 months of controller treatment to assess the patient's personal best FEV in 1 second, and periodically thereafter (at least every 1-2 years in adult patients, but more frequently in higher risk patients including with exacerbations and at risk of decline in lung function and in pediatric patients based on asthma severity and clinical course). B Show 2 more Serial FeNO testing: do not obtain routine fractional expiratory nitric oxide testing to monitor asthma in adult or pediatric patients, except in specialist asthma clinics. D Serial sputum testing: do not obtain routine sputum eosinophilia testing to monitor asthma in adult or pediatric patients. D References 1. David Smith, Ingrid Du Rand, Charlotte Louise Addy et al. British Thoracic Society guideline for the use of long-term macrolides in adults with respiratory disease. Thorax. 2020 May;75 5 370 404. Open 2. Global Initiative for Asthma. Global strategy for asthma management and prevention. GINA. 2022. Open 3. Erol A Gaillard, Claudia E Kuehni, Steve Turner et al. European Respiratory Society clinical practice guidelines for the diagnosis of asthma in children aged 5 16 years. Eur Respir J. 2021 Nov 4;58 5 2004173. Open 4. X Baur, T Sigsgaard, T B Aasen et al. Guidelines for the management of work-related asthma. Eur Respir J. 2012 Mar;39 3 529 45. Open 5. British Thoracic Society. SIGN158 British guideline on the management of asthma. BTS. 2019 July. Open 6. Kian Fan Chung, Sally E Wenzel, Jan L Brozek et al. International ERS/ATS guidelines on definition, evaluation and treatment of severe asthma. Eur Respir J. 2014 Feb;43 2 343 73. Open 7. Expert Panel Working Group of the National Heart, Lung, and Blood Institute NHLBI administered and coordinated National Asthma Education and Prevention Program Coordinating Committee NAEPPCC et al. 2020 Focused Updates to the Asthma Management Guidelines: A Report from the National Asthma Education and Prevention Program Coordinating Committee Expert Panel Working Group. J Allergy Clin Immunol. 2020 Dec;146 6 1217 1270. Open 8. FitzGerald J, Lemiere C, M. Lougheed D et al. Recognition and management of severe asthma: A Canadian Thoracic Society position statement. Can J Resp Crit Sleep Med. 2017 Oct;1 4 199 221. Open https://web.pathway.md/diseases/recoid4eeyMVLDPrX 24/26 6/23/23, 3:13 AM Asthma Pathway 9. Sumita B Khatri, Jonathan M Iaccarino, Amisha Barochia et al. Use of Fractional Exhaled Nitric Oxide to Guide the Treatment of Asthma: An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2021 Nov 15;204 10):e97-e109. Open 10. Darcy D Marciniuk, P Hernandez, M Balter et al. Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: a Canadian Thoracic Society clinical practice guideline. Can Respir J. 2012 Mar- Apr;19 2 109 16. Open 11. Graham Roberts, Marta Vazquez-Ortiz, Rebecca Knibb et al. EAACI Guidelines on the effective transition of adolescents and young adults with allergy and asthma. Allergy. 2020 Nov;75 11 2734 2752. Open 12. Ashish R Panchal, Jason A Bartos, Jos G Caba as et al. Part 3 Adult Basic and Advanced Life Support: 2020 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Circulation. 2020 Oct 20;142 16_suppl_2 S366 S468. Open 13. Renaud Louis, Imran Satia, Inigo Ojanguren et al. European Respiratory Society Guidelines for the Diagnosis of Asthma in Adults. Eur Respir J. 2022 Feb 15;2101585. Open 14. Marc Miravitlles, Asger Dirksen, Ilaria Ferrarotti et al. European Respiratory Society statement: diagnosis and treatment of pulmonary disease in 1 antitrypsin deficiency. Eur Respir J. 2017 Nov 30;50 5 1700610. Open 15. Andreanne C t , Richard J Russell, Louis-Philippe Boulet et al. Managing Chronic Cough Due to Asthma and NAEB in Adults and Adolescents: CHEST Guideline and Expert Panel Report. Chest. 2020 Jul;158 1 68 96. Open 16. Patterson TF, Thompson GR rd, Denning DW et al. Practice Guidelines for the Diagnosis and Management of Aspergillosis: 2016 Update by the Infectious Diseases Society of America. Clin Infect Dis. 2016 Aug 15;63 4):e1-e60. Open 17. Timothy S Chang, Robert F Lemanske Jr, Theresa W Guilbert et al. Evaluation of the modified asthma predictive index in high-risk preschool children. J Allergy Clin Immunol Pract. 2013 Mar;1 2 152 6. Open 18. Bert Brunekreef, Jet Smit, Johan de Jongste et al. The prevention and incidence of asthma and mite allergy PIAMA birth cohort study: design and first results. Pediatr Allergy Immunol. 2002;13(s15) 55 60. Open 19. Yoichi Nakamura, Jun Tamaoki, Hiroyuki Nagase et al. Japanese guidelines for adult asthma 2020. Allergol Int. 2020 Oct;69 4 519 548. Open 20. Michael Schatz, Christine A Sorkness, James T Li et al. Asthma Control Test: reliability, validity, and responsiveness in patients not previously followed by asthma specialists. J Allergy Clin Immunol. 2006 Mar;117 3 549 56. Open 21. E D Bateman, S S Hurd, P J Barnes et al. Global strategy for asthma management and prevention: GINA executive summary. GINA. 2021. Open 22. Ioana Agache, Jessica Beltran, Cezmi Akdis et al. Efficacy and safety of treatment with biologicals (benralizumab, dupilumab, mepolizumab, omalizumab and reslizumab) for severe eosinophilic asthma. A systematic review for the EAACI Guidelines - recommendations on the use of biologicals in severe asthma. Allergy. 2020 May;75 5 1023 1042. Open https://web.pathway.md/diseases/recoid4eeyMVLDPrX 25/26 6/23/23, 3:13 AM Asthma Pathway 23. Ioana Agache, Cezmi A Akdis, Mubeccel Akdis et al. EAACI Biologicals Guidelines-Recommendations for severe asthma. Allergy. 2021 Jan;76 1 14 44. Open 24. Ioana Agache, Yang Song, Claudio Rocha et al. Efficacy and safety of treatment with dupilumab for severe asthma: A systematic review of the EAACI guidelines-Recommendations on the use of biologicals in severe asthma. Allergy. 2020 May;75 5 1058 1068. Open 25. Elliot Israel, Juan-Carlos Cardet, Jennifer K Carroll et al. Reliever-Triggered Inhaled Glucocorticoid in Black and Latinx Adults with Asthma. N Engl J Med. 2022 Apr 21;386 16 1505 1518. Open 26. Jan L Bro ek, Jean Bousquet, Ioana Agache et al. Allergic Rhinitis and its Impact on Asthma ARIA guidelines-2016 revision. J Allergy Clin Immunol. 2017 Oct;140 4 950 958. Open 27. American Academy of Pediatrics. Choosing Wisely AAP recommendations. Choosing Wisely. 2020. Open 28. Federico Martin n-Torres, Antonio Rodr guez-N ez, Jose Mar a Martin n-S nchez. Heliox therapy in infants with acute bronchiolitis. Pediatrics. 2002 Jan;109 1 68 73. Open https://web.pathway.md/diseases/recoid4eeyMVLDPrX 26/26
Guideline sources The following summarized guidelines for the evaluation and management of asymptomatic bacteriuria (ASB) are prepared by our editorial team based on guidelines from the European Association of Urology (EAU 2022), the American Urological Association (AUA/CUA/SUFU 2019), the U.S. Preventive Services Task Force (USPSTF 2019), the Infectious Diseases Society of America (IDSA 2019; 2016; 2015; 2010; 2005), the German Society of Urology (GUS 2018), the Spanish Society of Clinical Microbiology and Infectious Diseases (SEIMC 2017), and the Society of Obstetricians and Gynaecologists of Canada (SOGC 2012). 1 2 3 4 5 6 7 8 9 10 11 12 12 13 13 14 Definition ASB is defined as the presence of bacteria in an uncontamined urine sample at a microbial load of 10 CFU/mL, without any associated signs or symptoms of UTI. 12 Epidemiology ASB is most frequently caused by E. coli, followed by P. mirabilis, K. pneumoniae, Enterobacter species, P. aeruginosa, Enterococcus species, S. aureus, S. saprophyticus, and Streptococcus agalactiae. 13 Pathophysiology https://web.pathway.md/diseases/receHyJljqRdbzlPx 1/8 6/23/23, 3:13 AM Asymptomatic bacteriuria Pathway Pathophysiology The prevalence of ASB is 1-2% in healthy men, 1-9% in healthy women, 2-15% in pregnant women, 1-30% in diabetic adults, 2-50% in community-dwelling adults, 14-75% in institutionalized adults, and 9-100% in patients with indwelling catheters. 13 Disease course In patients with ASB, symptoms of UTI are absent for a variety of reasons, including attenuated host immune response, as well as reduced microbial virulence, growth rates, and cell densities. 14 Prognosis and risk of recurrence Treatment of ASB should be avoided in low-risk populations given the absence of demonstrated benefit, potential for adverse drug reactions, and risk of promoting antibiotic-resistance. Select patients who may derive benefit from treatment include pregnant women and patients undergoing certain invasive procedures. 12 Guidelines 1. Screening and diagnosis Indications for screening, pregnant patients: As per EAU 2022 guidelines, screen pregnant females for and treat ASB with standard short- course treatment. B As per IDSA 2019 guidelines, screen pregnant females for ASB. (strong recommendation, moderate-quality evidence. As per USPSTF 2019 guidelines, screen pregnant females for ASB with a midstream, clean- catch urine for culture at the first prenatal visit or at 12-16 weeks of gestation, whichever is earlier. B Indications for screening, catheterized patients: As per IDSA 2019 guidelines: Avoid screening patients with short-term (< 30 days) or long-term indwelling urethral catheters for ASB. D Insufficient evidence to recommend for or against screening for ASB at the time of catheter removal in patients with indwelling catheters. I As per SEIMC 2017 guidelines, avoid screening patients with short-term D or long-term indwelling urethral catheters for ASB. D Indications for screening, patients undergoing urologic surgery: As per EAU 2022 guidelines, screen for and treat ASB before urological procedures breaching the mucosa. A As per IDSA 2019 guidelines: https://web.pathway.md/diseases/receHyJljqRdbzlPx 2/8 6/23/23, 3:13 AM Asymptomatic bacteriuria Pathway Screen for ASB in patients scheduled to undergo endoscopic urologic procedures associated with mucosal trauma. A Avoid screening for ASB in patients scheduled to undergo surgery for an artificial urinary sphincter or penile prosthesis implantation. D As per SEIMC 2017 guidelines: Screen for and treat ASB in patients undergoing TURP A or any other high-risk urological procedure. B Do not screen for ASB in patients undergoing low-risk urological procedures. D Indications for antibiotics, patients with a renal transplant: as per SEIMC 2017 guidelines, screen patients with a renal transplant for ASB only in the first month after transplantation. B Indications for screening (patients with nonrenal transplants): insufficient evidence to recommend for or against screening for and treating ASB in patients with hematopoietic stem cell transplants or nonrenal solid organ transplants. I Indications for screening, patients not to screen: As per EAU 2022 guidelines, do not screen or treat ASB in the following conditions: females without risk factors postmenopausal females elderly institutionalized patients renal transplant recipients well-controlled diabetes mellitus dysfunctional and/or reconstructed lower urinary tracts recurrent UTIs before arthroplasty surgeries. D As per IDSA 2019 guidelines, do not screen for or treat ASB in the following groups: healthy premenopausal, nonpregnant females D healthy postmenopausal females D infants and children D functionally impaired, older, community-dwelling individuals D older individuals residing in long-term care facilities D patients with diabetes D renal transplant recipients undergone renal transplant surgery > 1 month before D patients with non-renal solid organ transplant D patients with spinal cord injury D patients undergoing elective non-urologic surgery. D Show 2 more As per USPSTF 2019 guidelines, do not screen nonpregnant females for ASB. D As per GUS 2018 guidelines, do not screen nonpregnant females without other relevant concomitant diseases for ASB. D https://web.pathway.md/diseases/receHyJljqRdbzlPx 3/8 6/23/23, 3:13 AM Asymptomatic bacteriuria Pathway As per SEIMC 2017 guidelines, do not screen for and/or treat ASB in the following groups: elderly patients living in the community D institutionalized elderly patients D nonpregnant females < 60 years of age D nonpregnant females with diabetes mellitus D patients scheduled to undergo total hip or knee arthroplasty D patients with an orthotopic neobladder D patients with spinal cord injury treated with intermittent urinary catheterization. D Diagnostic criteria, general population: As per SEIMC 2017 guidelines, define bacteriuria in asymptomatic females as two consecutive clean-voided specimens with isolation of the same bacterial species in quantitative counts of 10 CFU/mL, or one positive urine culture with a positive nitrite test in another sample. B Show 2 more As per IDSA 2005 guidelines, diagnose ASB based on results of culture of a urine specimen collected in a manner minimizing contamination. B Show 2 more Diagnostic criteria, catheterized patients: As per SEIMC 2017 guidelines, define catheter-associated ASB in asymptomatic patients with indwelling urinary catheters, or undergoing intermittent catheterization, as a single catheterized urine specimen with one bacterial species isolated in a quantitative count 10 CFU/mL. B Show 2 more As per IDSA 2010 guidelines, define catheter-associated ASB in patients with indwelling urethral or indwelling suprapubic catheters as the presence of 10 CFU/mL of 1 bacterial species in a single catheter urine specimen in a patient without symptoms compatible with UTI. B Show 6 more 2. Diagnostic investigations Urine tests: Do not obtain urine test strips for the detection of ASB. D Obtain an initial urine culture in pregnant females at 12-16th weeks of gestation. A 3. Medical management Indications for antibiotics, pregnant patients: As per IDSA 2019 guidelines, treat ASB in pregnant patients. A As per USPSTF 2019 guidelines, initiate treatment if urine culture shows > 100,000 CFU/mL of a single uropathogen or > 10,000 CFU/mL of group B streptococcus. https://web.pathway.md/diseases/receHyJljqRdbzlPx 4/8 6/23/23, 3:13 AM Asymptomatic bacteriuria Pathway As per SEIMC 2017 guidelines, treat ASB in pregnant patients in order to decrease the risk of pyelonephritis A , preterm labor, and low birth weight infants. B As per IDSA 2015 guidelines, treat ASB in pregnant patients. B As per IDSA 2005 guidelines, complete a 3-7-days of antimicrobial therapy in pregnant patients with ASB. B Indications for antibiotics, catheterized patients: As per IDSA 2019 guidelines: Avoid treating ASB in patients with short-term (< 30 days) or long-term indwelling urethral catheters. D Insufficient evidence to recommend for or against treating ASB at the time of catheter removal. I As per SEIMC 2017 guidelines, treat ASB in females with an indwelling urethral catheter only if ASB persists 48 hours after removal of the catheter. B As per IDSA 2010 guidelines: Consider initiating antimicrobial treatment for catheter-associated ASB persisting 48 hours after short-term indwelling catheter removal in females, to reduce the risk of subsequent catheter-associated UTI. B Insufficient evidence to recommend treatment of persistent catheter-associated ASB in males. I Indications for antibiotics, patients undergoing urologic procedures: As per IDSA 2019 guidelines: Treat ASB in patients scheduled to undergo endoscopic urologic procedures associated with mucosal trauma. A Consider administering a short course of antibiotic therapy (1 or 2 doses) in patients with ASB scheduled to undergo a urologic procedure. C As per SEIMC 2017 guidelines, treat ASB in patients undergoing TURP A or any other high- risk urological procedure. B Show 2 more As per IDSA 2015 guidelines, treat ASB in patients undergoing invasive urological or prostate surgery. B Indications for antibiotics (patients undergoing spinal surgery): treat ASB prior to performing instrumental spinal surgery in patients with urinary catheters, neurogenic bladders or urinary incontinence, in order to reduce the risk of Gram-negative surgical site infections. B Indications for antibiotics, patients with a renal transplant: as per IDSA 2015 guidelines, treat ASB in renal or renal-pancreas transplant recipients within the first year post-transplant. B Indications for antibiotics (patients with nonrenal transplants): do not initiate systemic antifungal therapy for asymptomatic candiduria in transplant patients, except for neutropenic patients or patients scheduled to undergo urological procedures. D https://web.pathway.md/diseases/receHyJljqRdbzlPx 5/8 6/23/23, 3:13 AM Asymptomatic bacteriuria Pathway Indications for antibiotics, patients not to treat: As per IDSA 2019 guidelines, do not treat ASB in the following groups: healthy premenopausal, nonpregnant females D healthy postmenopausal females D infants and children D functionally impaired, older, community-dwelling individuals D older individuals residing in long-term care facilities D patients with diabetes D renal transplant recipients undergone renal transplant surgery > 1 month before D patients with non-renal solid organ transplant D patients with spinal cord injury D patients undergoing elective non-urologic surgery. D Show 2 more As per SUFU/CUA/AUA 2019 guidelines, do not treat nonpregnant patients with ASB not scheduled to undergo invasive urinary tract procedures. D Duration of treatment: administer standard 4-7-day treatment regimens, or a single 3 g dose of fosfomycin tromethamine as an alternative, over short one-day treatments for eradicating bacteriuria. A 4. Specific circumstances Pregnant patients: treat any bacteriuria with colony counts 10 CFU/mL in pregnancy. B Show 3 more Elderly patients: assess for non-urinary pathology as part of the initial evaluation of falls or delirium (acute mental status change, confusion) in older patients with functional and/or cognitive impairment, bacteriuria, and no local genitourinary symptoms or other systemic signs of infection (such as fever or hemodynamic instability). B Catheterized patients: do not screen for catheter-associated ASB except in research studies evaluating interventions designed to reduce the incidence of catheter-associated ASB or catheter- associated UTI and in selected clinical situations, such as pregnant patients and patients undergoing urologic procedures for which visible mucosal bleeding is anticipated. D Show 6 more Patients with asymptomatic candiduria: eliminate predisposing factors, such as indwelling bladder catheters, whenever feasible in patients with asymptomatic candiduria. B Show 3 more 5. Preventative measures https://web.pathway.md/diseases/receHyJljqRdbzlPx 6/8 6/23/23, 3:13 AM Asymptomatic bacteriuria Pathway Routine catheter change: insufficient evidence to recommend routine catheter change (such as every 2-4 weeks) in patients with functional long-term indwelling urethral or suprapubic catheters to reduce the risk of catheter-associated ASB or catheter-associated UTI. I 6. Follow-up and surveillance Post-treatment surveillance: As per SEIMC 2017 guidelines: Obtain a follow-up urine culture in order to verify bacteriuria eradication in pregnant patients. B Obtain subsequent monthly urine cultures until delivery. B As per IDSA 2005 guidelines: Obtain periodic screening for recurrent bacteriuria following therapy in pregnant patients. B Insufficient evidence to recommend for or against repeated screening in culture-negative females in later pregnancy. I References 1. Anger J, Lee U, Ackerman AL et al. Recurrent Uncomplicated Urinary Tract Infections in Women: AUA/CUA/SUFU Guideline. J Urol. 2019 Aug;202 2 282 289. Open 2. de Cueto M, Aliaga L, Al s JI et al. Executive summary of the diagnosis and treatment of urinary tract infection: Guidelines of the Spanish Society of Clinical Microbiology and Infectious Diseases SEIMC . Enferm Infecc Microbiol Clin. 2017 May;35 5 314 320. Open 3. Hooton TM, Bradley SF, Cardenas DD et al. Diagnosis, prevention, and treatment of catheter-associated urinary tract infection in adults: 2009 International Clinical Practice Guidelines from the Infectious Diseases Society of America. Clin Infect Dis. 2010 Mar 1;50 5 625 63. Open 4. US Preventive Services Task Force, Douglas K Owens, Karina W Davidson et al. Screening for Asymptomatic Bacteriuria in Adults: US Preventive Services Task Force Recommendation Statement. JAMA. 2019 Sep 24;322 12 1188 1194. Open 5. Nicolle LE, Bradley S, Colgan R et al. Infectious Diseases Society of America guidelines for the diagnosis and treatment of asymptomatic bacteriuria in adults. Clin Infect Dis. 2005 Mar 1;40 5 643 54. Open 6. Nicolle LE, Gupta K, Bradley SF et al. Clinical Practice Guideline for the Management of Asymptomatic Bacteriuria: 2019 Update by the Infectious Diseases Society of America. Clin Infect Dis. 2019 May 2;68 10):e83-e110. Open 7. Kranz J, Schmidt S, Lebert C et al. The 2017 Update of the German Clinical Guideline on Epidemiology, Diagnostics, Therapy, Prevention, and Management of Uncomplicated Urinary Tract Infections in Adult Patients: Part 1. Urol Int. 2018;100 3 263 270. Open 8. Infectious Diseases Society of America. Choosing Wisely: Recommendations of the Infectious Diseases Society of America. Choosing Wisely. 2015 Feb. Open https://web.pathway.md/diseases/receHyJljqRdbzlPx 7/8 6/23/23, 3:13 AM Asymptomatic bacteriuria Pathway 9. G. Bonkat, R. Bartoletti, F. Bruy re et al. EAU Guidelines on Urological Infections. EAU. 2022. Open 10. Pappas PG, Kauffman CA, Andes DR et al. Clinical Practice Guideline for the Management of Candidiasis: 2016 Update by the Infectious Diseases Society of America. Clin Infect Dis. 2016 Feb 15;62 4):e1 50. Open 11. Victoria M Allen, Mark H Yudin, INFECTIOUS DISEASES COMMITTEE. Management of group B streptococcal bacteriuria in pregnancy. J Obstet Gynaecol Can. 2012 May;34 5 482 486. Open 12. Daniel M, Keller S, Mozafarihashjin M et al. An Implementation Guide to Reducing Overtreatment of Asymptomatic Bacteriuria. JAMA Intern Med. 2018 Feb 1;178 2 271 276. Open 13. Ipe DS, Sundac L, Benjamin WH Jr et al. Asymptomatic bacteriuria: prevalence rates of causal microorganisms, etiology of infection in different patient populations, and recent advances in molecular detection. FEMS Microbiol Lett. 2013 Sep;346 1 1 10. Open 14. Nicolle LE. Asymptomatic bacteriuria. Curr Opin Infect Dis. 2014 Feb;27 1 90 6. Open 15. Gupta K, Hooton TM, Naber KG et al. International clinical practice guidelines for the treatment of acute uncomplicated cystitis and pyelonephritis in women: A 2010 update by the Infectious Diseases Society of America and the European Society for Microbiology and Infectious Diseases. Clin Infect Dis. 2011 Mar 1;52 5):e103 20. Open 16. Infectious Diseases Society of America. Choosing Wisely IDSA recommendations. Choosing Wisely. 2015. Open 17. American Academy of Pediatrics. Choosing Wisely AAP recommendations. Choosing Wisely. 2016. Open 18. American Geriatrics Society. Choosing Wisely AGS recommendations. Choosing Wisely. 2013. Open https://web.pathway.md/diseases/receHyJljqRdbzlPx 8/8
Guideline sources The following summarized guidelines for the evaluation and management of atlanto-occipital dislocation (AOD) are prepared by our editorial team based on guidelines from the American Association of Neurological Surgeons (AANS/CNS 2013). 1 2 2 3 4 Definition AOD is the loss of stability between the atlas and axis (CI-C2) that results in loss of normal articulation of the upper cervical spine to the occiput. 2 Epidemiology AOD is caused due to trauma (head injuries, type II fractures), congenital conditions (Down's syndrome, skeletal dysplasias, Goldenhar syndrome, spondyloepiphyseal dysplasia, Morquio syndrome, occipitalization of atlas), and inflammation (chronic rheumatoid arthritis). 2 Disease course The injury of ligaments between the occiput and upper cervical spine results in AOD, which causes clinical manifestations of severe neck pain, lower cranial nerve deficits, unilateral/bilateral weakness, and quadriplegia, unconsciousness, respiratory arrest, spinal nerve injury, neurogenic shock, and cerebrovascular injury. 3 Prognosis and risk of recurrence AOD is associated with a mortality of 26%. 4 https://web.pathway.md/diseases/rectSjTld3LFDm9Mu 1/2 6/23/23, 3:13 AM Atlanto-occipital dislocation Pathway Guidelines 1. Diagnostic investigations Lateral cervical radiographs: obtain a lateral cervical XR for the diagnosis of AOD. B Computed tomography: obtain CT of the craniocervical junction if there is clinical or radiographic suspicion of AOD. B 2. Therapeutic procedures Traction: avoid traction in the management of patients with AOD, as it is associated with a 10% risk of neurological deterioration. D 3. Surgical interventions Internal fixation: perform internal fixation and fusion for definitive management of AOD. B References 1. Theodore N, Aarabi B, Dhall SS et al. The diagnosis and management of traumatic atlanto-occipital dislocation injuries. Neurosurgery. 2013 Mar;72 Suppl 2 114 26. Open 2. Sun Y Yang, Anthony J Boniello, Caroline E Poorman et al. A review of the diagnosis and treatment of atlantoaxial dislocations. 2014 Aug;4 3 197 210.2014 Aug;4 3 197 210. Open 3. Graham C Hall, Michael J Kinsman, Ryan G Nazar et al. Atlanto-occipital dislocation. 2015 Mar 18;6 2 236 43.2015 Mar 18;6 2 236 43. Open 4. Stephen K Mendenhall, Ahilan Sivaganesan, Akshitkumar Mistry et al. Traumatic atlantooccipital dislocation: comprehensive assessment of mortality, neurologic improvement, and patient-reported outcomes at a Level 1 trauma center over 15 years. 2015 Nov 1;15 11 2385 95.2015 Nov 1;15 11 2385 95. Open https://web.pathway.md/diseases/rectSjTld3LFDm9Mu 2/2
Guideline sources The following summarized guidelines for the evaluation and management of atopic dermatitis are prepared by our editorial team based on guidelines from the European Dermatology Forum (EDF 2022), the European Academy of Allergy and Clinical Immunology (EAACI 2021), the European Academy of Dermatology and Venereology (EADV/ETFAD 2020), the European Academy of Dermatology and Venereology (EADV/UEMS/EFA/GA LEN/ETFAD/EDF/EAACI/ESDaP/ESPD 2018), the European Academy of Dermatology and Venereology (EADV/UEMS/EFA/ETFAD/EDF/EAACI/ESDaP/ESPD 2018), and the American Academy of Dermatology (AAD 2014). 1 2 3 4 5 6 7 8 9 10 11 11 11 12 Definition Atopic dermatitis, also known as atopic eczema, is a chronic relapsing inflammatory skin condition characterized by intense itching. 11 Epidemiology Atopic dermatitis is caused by a complex interaction between defects in skin barrier function (caused by mutations in filaggrin gene), immune dysregulation, and environmental and infectious https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 1/12 6/23/23, 3:13 AM Atopic dermatitis Pathway agents (S. aureus). 12 Disease course Skin barrier dysfunction and immune dysfunction results in atopic dermatitis, which causes clinical phases of acute atopic dermatitis (vesicular, weeping, crusting eruption), subacute atopic dermatitis (dry, scaly, erythematous papules and plaques), and chronic atopic dermatitis (lichenification, thickening, from repeated scratching). Atopic dermatitis precedes the development of food allergies, asthma, and allergic rhinitis. The disease decreases productivity and quality of life. 11 Prognosis and risk of recurrence Atopic dermatitis is not associated with an increase in mortality. 11 Guidelines 1. Classification and risk stratification Severity grading: do not use available disease severity or patient quality of life measurement scales in routine clinical practice in the management of patients with atopic dermatitis. D 2. Diagnostic investigations Clinical history: Assess for environmental and food allergies as part of the initial evaluation of patients with atopic dermatitis. B Assess for symptoms of itch, sleep disturbance, impact on daily activity, persistence of disease, and use currently available disease severity scales when practical. B Biomarkers: insufficient evidence to recommend any specific biomarker for diagnosis and/or assessment of disease severity in patients with presumed atopic dermatitis. I Allergy testing: As per EADV 2020 guidelines: Obtain an allergy workup in patients with moderate-to-severe atopic dermatitis, including serum IgE, skin prick tests and patch tests for contact sensitization to ingredients of emollients and topical anti-inflammatory agents, depending on the individual history. Consider obtaining allergy work-up in patients with mild atopic dermatitis depending on clinical suspicion. E Consider setting a low threshold for obtaining patch tests with a standard series and components of topical treatment preparations in patients with atopic dermatitis. Obtain patch testing in patients with recalcitrant disease, atypical localization or aggravation without known cause, especially when systemic intervention is considered. E As per AAD 2014 guidelines, avoid obtaining allergy testing in patients without clinical features suggestive of allergy (such as hives or urticaria). D https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 2/12 6/23/23, 3:13 AM Atopic dermatitis Pathway Show 2 more Specialized tests: consider obtaining skin biopsy specimens or other tests (e.g., serum IgE, potassium hydroxide preparation, patch testing, and/or genetic testing) to rule out other or associated skin conditions. C Screening for associated conditions: assess for conditions associated with atopic dermatitis, such as rhinitis/rhinoconjunctivitis, asthma, food allergy, sleep disturbance, depression, and other neuropsychiatric conditions, and discuss them with the patient as part of the treatment plan when appropriate. B 3. Medical management General principles: As per EDF 2022 guidelines: Ensure shared decision-making by discussing the patient's beliefs, lifestyle, and preferences when deciding on the treatment plan. A Ensure multidisciplinary treatment approach in patients with comorbidities. A As per AAD 2014 guidelines, consider an integrated, multidisciplinary approach to care for patients with atopic dermatitis. C Topical corticosteroids: As per EADV 2020 guidelines: Offer topical corticosteroids according to standard guidelines in combination with many other treatment modalities including patient education. E Offer proactive therapy with topical corticosteroids in patients with moderate-to-severe atopic dermatitis. E As per EAACI 2018 guidelines, offer topical corticosteroids (with an improved risk/benefit ratio) in patients with atopic dermatitis, especially in the acute phase. B Show 3 more As per AAD 2014 guidelines, initiate topical corticosteroids in patients with atopic dermatitis who have failed to respond to good skin care and regular use of emollients alone. A Show 3 more Topical calcineurin inhibitors: As per EADV 2020 guidelines: Offer topical calcineurin inhibitors as first-line therapy for delicate body areas in patients with atopic dermatitis, with preference for pimecrolimus in mild disease, and for tacrolimus in moderate-to-severe disease, and for long-term topical treatment. E Offer proactive therapy with topical calcineurin inhibitors in patients with moderate-to-severe atopic dermatitis. E As per EAACI 2018 guidelines, offer topical calcineurin inhibitors in patients with atopic dermatitis. B https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 3/12 6/23/23, 3:13 AM Atopic dermatitis Pathway Show 4 more As per AAD 2014 guidelines, use topical calcineurin inhibitors, applied to actively affected areas, as a steroid-sparing agent for the initial treatment of atopic dermatitis. A Show 2 more Topical antimicrobials/antiseptics: As per EADV 2020 guidelines, consider offering topical antiseptic drugs and antiseptic baths in addition to an adequate anti-inflammatory treatment with topical corticosteroids and topical calcineurin inhibitors, if clinical signs of strong colonization or bacterial superinfection are present. E Show 3 more As per EAACI 2018 guidelines, do not use long-term topical antibiotics due to the risk of increasing resistances and sensitizations. D Show 2 more As per AAD 2014 guidelines: Consider using bleach baths and intranasal mupirocin to reduce disease severity in patients with moderate-to-severe atopic dermatitis and clinical signs of secondary bacterial infection. C Avoid using any other topical antistaphylococcal treatment in patients with atopic dermatitis. D Topical antihistamines: avoid using topical antihistamines for the treatment of patients with atopic dermatitis, due to the risk of absorption and of contact dermatitis. D Systemic immunomodulatory agents: As per EDF 2022 guidelines, offer any of the following in patients with atopic dermatitis being candidates for systemic treatment: cyclosporin baricitinib upadacitinib. A Show 5 more As per EADV 2020 guidelines, offer classical immunosuppressive therapy with cyclosporine A, methotrexate, mycophenolate mofetil or azathioprine only if topical treatment is not an option. Take into account all relevant patient- and drug-related factors including the patient's comorbidity, expected onset of efficacy, planed duration of treatment and licensing status of the drug when choosing an immunosuppressive agent. E As per EAACI 2018 guidelines, consider offering cyclosporine (for up to 2 years with a continuous regimen) in adult patients with chronic severe atopic dermatitis. Obtain careful monitoring for potential severe side effects. B Show 21 more As per AAD 2014 guidelines, offer systemic immunomodulatory agents in patients with atopic dermatitis in whom optimized topical regimens and/or phototherapy do not adequately control the signs and symptoms of disease. https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 4/12 6/23/23, 3:13 AM Atopic dermatitis Pathway Show 4 more Systemic biologic agents: As per EDF 2022 guidelines: Offer any of the following in patients with atopic dermatitis being candidates for systemic treatment: dupilumab tralokinumab. A Insufficient evidence to support the use of omalizumab in patients with atopic dermatitis. I As per EAACI 2021 guidelines, offer dupilumab to reduce disease activity, rescue and background medication, and improve quality of life in adult and 12-17 years old pediatric patients with uncontrolled atopic dermatitis. A Show 12 more As per EADV 2020 guidelines, offer dupilumab for the treatment of 12 years old patients with moderate-to-severe atopic dermatitis, being candidates for systemic therapy. E As per ETFAD 2018 guidelines, offer dupilumab as a disease-modifying drug in patients with moderate-to-severe atopic dermatitis, if topical treatment is not sufficient and other systemic treatment is not advisable. A Show 3 more Systemic corticosteroids: As per EDF 2022 guidelines: Consider offering systemic corticosteroids only as rescue therapy for acute flares in patients with atopic dermatitis. C Do not use systemic corticosteroids for long term in patients with atopic dermatitis. D As per EAACI 2018 guidelines: Consider offering short-term (up to 1 week) oral corticosteroids for the treatment of an acute flare in exceptional cases of atopic dermatitis. Restrict its use, largely limited to adult patients with severe atopic dermatitis. C Adjust the daily dose of corticosteroids not exceeding 0.5 mg/kg body weight. Do not use oral corticosteroids for long-term. B As per AAD 2014 guidelines, avoid using systemic corticosteroids, if possible, for the treatment of patients with atopic dermatitis. Reserve corticosteroids exclusively for acute, severe exacerbations and as a short-term bridge therapy to other systemic, corticosteroid-sparing therapy. D Systemic antimicrobials: As per EAACI 2018 guidelines: Consider administering a short course of systemic antibiotics, such as cephalosporin, in patients with clinically infected with S. aureus. C Consider offering topical or systemic antifungal therapy in patients with 'head and neck' variant of atopic dermatitis or with demonstrated IgE sensitization to Malassezia species. C https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 5/12 6/23/23, 3:13 AM Atopic dermatitis Pathway As per AAD 2014 guidelines, administer systemic antiviral agents for the treatment of eczema herpeticum. B Show 2 more Systemic antihistamines: Avoid using non-sedating antihistamines as a routine treatment for atopic dermatitis in the absence of urticaria or other atopic conditions such as rhinoconjunctivitis. D Consider short-term, intermittent use of sedating antihistamines in patients with insomnia secondary to symptoms of itch, but avoid substituting them for management of atopic dermatitis with topical therapies. C Management of pruritus: As per EADV 2020 guidelines, do not use first- or second-generation H1RAs for the treatment of pruritus in patients with atopic dermatitis. D Show 2 more As per EAACI 2018 guidelines, offer topical corticosteroids to control pruritus in the initial phase of atopic dermatitis exacerbation. A Show 9 more As per AAD 2018 guidelines, consider offering short-term, intermittent sedating antihistamines in the setting of sleep loss secondary to pruritus. C Allergen immunotherapy: As per EADV 2020 guidelines: Do not offer allergen immunotherapy as a general treatment option in patients with atopic dermatitis. D Consider offering allergen immunotherapy in selected patients with house dust mite, birch or grass pollen sensitization, having severe atopic dermatitis and a history of clinical exacerbation after exposure to the causative allergen or a positive corresponding atopy patch test. E As per EAACI 2018 guidelines: Do not offer allergen-specific immunotherapy as a general treatment option in patients with atopic dermatitis. D Consider offering allergen-specific immunotherapy in selected patients with house dust mite, birch or grass pollen sensitization, having severe atopic dermatitis, and a history of clinical exacerbation after exposure to the causative allergen or a positive corresponding atopy patch test. C As per AAD 2014 guidelines, avoid using sublingual immunotherapy or injection immunotherapy to treat atopic dermatitis in the general atopic dermatitis population. D 4. Nonpharmacologic interventions Moisturizers and emollients: https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 6/12 6/23/23, 3:13 AM Atopic dermatitis Pathway As per EDF 2022 guidelines, consider advising the use of body care products (for example, gentle cleansers) not containing potent irritants or relevant allergens in patients with atopic dermatitis. C Show 4 more As per EADV 2020 guidelines, advise using liberal amounts of emollients daily, at least 30 g/day or 1 kg/month for an adult, preferentially applied in a 'soak-and-seal' technique. E As per EAACI 2018 guidelines, advise using emollients in adequate amounts, liberally and frequently, in a minimum amount of 250 g per week for adults. B Show 2 more As per EAACI 2018 guidelines, consider offering topical application of unsaturated fatty acids as an ingredient in emollients in selected cases. C As per AAD 2014 guidelines: Advise using moisturizers as an integral part of the treatment in patients with atopic dermatitis, as there is strong evidence that their use can reduce disease severity and the need for pharmacologic intervention. A Advise using a limited amount of non-soap cleansers that are neutral to low pH, hypoallergenic, and fragrance-free. B Bathing: As per EDF 2022 guidelines, advise gentle cleansing and bathing procedures in patients with atopic dermatitis, especially in acutely inflamed or superinfected skin. A Show 2 more As per EADV 2020 guidelines, advise regular bathing or showering in warm but not hot water about 2-7 times per week with very mild surfactant and emollient-based wash products in a 'soak-and-seal' approach. E As per EAACI 2018 guidelines, consider adding antiseptics such as sodium hypochlorite to the bathwater in patients with atopic dermatitis. B Advise using emollient bath oils and soap substitutes. C As per AAD 2014 guidelines, consider advising bathing in patients with atopic dermatitis as part of initial and maintenance treatment. Recognize that there is no standard for the frequency or duration of bathing appropriate for patients with atopic dermatitis. C Show 2 more Clothing and laundering: As per EADV 2020 guidelines, consider advising high-quality silver garments in patients with atopic dermatitis tending to strong colonization or infection with S. aureus. E As per AAD 2014 guidelines: Insufficient evidence to support the use of specialized clothing fabrics to treat atopic dermatitis. I Insufficient evidence to recommend the use of specific laundering techniques (such as double rinsing), detergents, or other laundry products for atopic dermatitis treatment. I https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 7/12 6/23/23, 3:13 AM Atopic dermatitis Pathway Wet-wrap therapy: As per EADV 2020 guidelines, offer wet-wrap therapy in patients with atopic dermatitis in flare, or with acute, oozing and erosive lesions. E As per AAD 2014 guidelines, consider offering wet-wrap therapy, with or without a topical corticosteroid, in patients with moderate-to-severe atopic dermatitis, in order to decrease disease severity and water loss during flares. C Allergen avoidance: advise avoiding pollen exposure during the pollen season. B Show 2 more House dust mite covers: consider house dust mite covers to treat patients with atopic dermatitis who are sensitized to dust mites, based on limited evidence. C Food elimination diets: As per EADV 2020 guidelines, advise an elimination diet for atopic dermatitis based on a reliable history, rather than on type I sensitization detected by blood or skin prick tests, followed by elimination and reintroduction of suspected food or an oral food challenge, if this is feasible. E As per EAACI 2018 guidelines, offer a therapeutic diet eliminating foods that elicitated clinical early or late reactions upon controlled oral provocation tests in patients with moderate-to-severe atopic dermatitis. B As per AAD 2014 guidelines, avoid food elimination diets based solely on the findings of food allergy test results for the management of atopic dermatitis. D Dietary supplements: As per EADV 2020 guidelines, do not offer vitamin D supplementation for the treatment of patients with atopic dermatitis. D As per EAACI 2018 guidelines: Do not offer supplementation of unsaturated fatty acids for the treatment of atopic dermatitis. D Insufficient evidence to recommend vitamin supplementation for routine use in patients with atopic dermatitis. I As per AAD 2014 guidelines, insufficient evidence to support the use of fish oils, evening primrose oil, borage oil, multivitamin supplements, zinc, vitamin D, vitamin E, vitamin B12, or vitamin B6 for the treatment of atopic dermatitis. I Probiotics: As per EADV 2020 guidelines, do not offer probiotics for the treatment of patients with atopic dermatitis. D As per AAD 2014 guidelines, avoid probiotics/prebiotics to treat patients with established atopic dermatitis because of inconsistent evidence for effectiveness. D Psychosocial interventions: offer psychosomatic counseling, psychotherapeutical approaches, behavioral therapy techniques, autogenic training, relaxation techniques, psychological and psychosomatic interventions in selected patients with atopic dermatitis. A https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 8/12 6/23/23, 3:13 AM Atopic dermatitis Pathway Alternative and complementary therapies: As per EADV 2020 guidelines, do not offer complementary medicine for the treatment of patients with atopic dermatitis. D As per EAACI 2018 guidelines, consider offering thermal spring water balneotherapy in patients with mild-to-moderate atopic dermatitis. C Show 2 more As per AAD 2014 guidelines, insufficient evidence to recommend the following therapies for the treatment of patients with atopic dermatitis: massage therapy I chinese herbal therapy I aromatherapy I naturopathy I hypnotherapy I acupressure I autologous blood injections. I 5. Therapeutic procedures Phototherapy: As per EADV 2020 guidelines, offer concomitant narrowband UV B or medium-dose UV A in combination with topical corticosteroids or selected systemic therapy for the treatment of patients with atopic dermatitis. E As per EAACI 2018 guidelines, offer medium-dose UV A1 and narrowband UV B for the treatment of adult patients with atopic dermatitis. B Show 5 more As per AAD 2014 guidelines, provide phototherapy as a second-line treatment, in patients with atopic dermatitis who have failed of first-line treatment with emollients, topical steroids, and topical calcineurin inhibitors. B Show 3 more Immunoadsorption: consider offering immunoadsorption in patients with severe atopic dermatitis and high serum IgE levels, if the technology is available. C 6. Patient education General counseling: Educate patients/caregivers in lay language about treating and managing their own condition. A Provide patients/caregivers adequate knowledge, skills, resources, and support for the treatment of atopic dermatitis at home and coping with its impact on life. A https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 9/12 6/23/23, 3:13 AM Atopic dermatitis Pathway Occupational counseling: Develop and optimize preventive strategies to reduce the incidence of occupational dermatitis in patients with atopic dermatitis. E Advise patients with atopic dermatitis early on occupational aspects of their disease and suitable career choices. E Educational programs: As per EADV 2020 guidelines: Offer multimodal education programs to increase coping behavior in patients with atopic dermatitis. Offer relaxation and habit-reversal techniques as a complementary treatment in patients with atopic dermatitis. E Advise participation in a therapeutic education program for atopic dermatitis in all patients with moderate-to-severe atopic dermatitis. E As per EAACI 2018 guidelines, offer patient education programs for atopic dermatitis as an adjunct to conventional therapy in pediatric and adult patients. A As per AAD 2014 guidelines, offer educational programs and "eczema schools" as an adjunct to the conventional therapy of atopic dermatitis. A Counseling on corticosteroids: address patient fears of side effects associated with the use of topical corticosteroids for atopic dermatitis, in order to improve adherence and avoid under- treatment. B Counseling on calcineurin inhibitors: Inform patients of the theoretical risk of cutaneous viral infections with calcineurin inhibitor therapy, although no consistent increases in the prevalence of such viral infections have been documented with continuous or intermittent use of topical calcineurin inhibitors for up to 5 years. B Discuss the black box warning on the use of topical calcineurin inhibitors (stating that the treatments were linked in postmarketing reports with cancer-related adverse events, including lymphoma and skin malignancies) with patients in whom calcineurin inhibitors are being considered. B 7. Preventative measures Routine immunizations: As per EADV 2020 guidelines, follow all regional vaccination programs including the VZV vaccination as recommended. E Show 2 more As per EAACI 2018 guidelines, provide vaccinations in all pediatric patients diagnosed with atopic dermatitis according to the national vaccination plan. B As per EAACI 2018 guidelines: https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 10/12 6/23/23, 3:13 AM Atopic dermatitis Pathway Offer vaccination against VZV in pediatric patients with atopic dermatitis. Encourage parents of atopic children to fully immunize their children. B Initiate systemic antiviral therapy such as systemic aciclovir without delay for the treatment of patients with eczema herpeticum. B Primary prevention: advise exclusive breast milk feeding until 4 months of age for primary prevention of food allergy-associated atopic dermatitis. B Show 3 more 8. Follow-up and surveillance Follow-up: avoid routine monitoring of IgE levels for the assessment of disease severity. D Show 2 more References 1. A Wollenberg, S Barbarot, T Bieber et al. Consensus-based European guidelines for treatment of atopic eczema (atopic dermatitis) in adults and children: part I. J Eur Acad Dermatol Venereol. 2018 May;32 5 657 682. Open 2. A Wollenberg, M Kinberger, B Arents et al. European guideline EuroGuiDerm) on atopic eczema - part II non-systemic treatments and treatment recommendations for special AE patient populations. J Eur Acad Dermatol Venereol. 2022 Sep 3. Open 3. Eichenfield LF, Tom WL, Chamlin SL et al. Guidelines of care for the management of atopic dermatitis: section 1. Diagnosis and assessment of atopic dermatitis. J Am Acad Dermatol. 2014 Feb;70 2 338 51. Open 4. A Wollenberg, S Christen-Z ch, A Taieb et al. ETFAD/EADV Eczema task force 2020 position paper on diagnosis and treatment of atopic dermatitis in adults and children. J Eur Acad Dermatol Venereol. 2020 Dec;34 12 2717 2744. Open 5. A Wollenberg, S Barbarot, T Bieber et al. Consensus-based European guidelines for treatment of atopic eczema (atopic dermatitis) in adults and children: part II. J Eur Acad Dermatol Venereol. 2018 Jun;32 6 850 878. Open 6. Sidbury R, Tom WL, Bergman JN et al. Guidelines of care for the management of atopic dermatitis: Section 4. Prevention of disease flares and use of adjunctive therapies and approaches. J Am Acad Dermatol. 2014 Dec;71 6 1218 33. Open 7. Sidbury R, Davis DM, Cohen DE et al. Guidelines of care for the management of atopic dermatitis: section 3. Management and treatment with phototherapy and systemic agents. J Am Acad Dermatol. 2014 Aug;71 2 327 49. Open 8. Ioana Agache, Cezmi A Akdis, Mubeccel Akdis et al. EAACI Biologicals Guidelines-dupilumab for children and adults with moderate-to-severe atopic dermatitis. Allergy. 2021 Apr;76 4 988 1009. Open 9. A Wollenberg, M Kinberger, B Arents et al. European guideline EuroGuiDerm) on atopic eczema: part I systemic therapy. J Eur Acad Dermatol Venereol. 2022 Sep;36 9 1409 1431. Open https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 11/12 6/23/23, 3:13 AM Atopic dermatitis Pathway 10. Eichenfield LF, Tom WL, Berger TG et al. Guidelines of care for the management of atopic dermatitis: section 2. Management and treatment of atopic dermatitis with topical therapies. J Am Acad Dermatol. 2014 Jul;71 1 116 32. Open 11. Carmela Avena-Woods. Overview of atopic dermatitis. 2017 Jun;23 8 Suppl):S115 S123.2017 Jun;23 8 Suppl):S115 S123. Open 12. Sandeep Kapur, Wade Watson, Stuart Carr. Atopic dermatitis. 2018 Sep 12;14 Suppl 2 52.2018 Sep 12;14 Suppl 2 52. Open 13. Aoki V, Lorenzini D, Orfali RL et al. Consensus on the therapeutic management of atopic dermatitis Brazilian Society of Dermatology. An Bras Dermatol. 2019 Apr;94 2 Suppl 1 67 75. Open 14. American Academy of Dermatology. Choosing Wisely AAD recommendations. Choosing Wisely. 2013. Open 15. W David Boothe, James A Tarbox, Michelle B Tarbox. Atopic Dermatitis: Pathophysiology. Adv Exp Med Biol. 2017;1027 21 37. Open 16. Tiago Torres, Eduarda Os rio Ferreira, Margarida Gon alo et al. Update on Atopic Dermatitis. Acta Med Port. 2019 Sep 2;32 9 606 613. Open 17. Nanette B Silverberg. Typical and atypical clinical appearance of atopic dermatitis. Clin Dermatol. Jul- Aug 2017;35 4 354 359. Open 18. Ioana Agache, Yang Song, Margarita Posso et al. Efficacy and safety of dupilumab for moderate-to- severe atopic dermatitis: A systematic review for the EAACI biologicals guidelines. Allergy. 2021 Jan;76 1 45 58. Open https://web.pathway.md/diseases/rec9qBSnBFgZQE6nw 12/12
Guideline sources The following summarized guidelines for the evaluation and management of atrial fibrillation ablation are prepared by our editorial team based on guidelines from the Latin American Heart Rhythm Society (SOLAECE/ECAS/APHRS/EHRA/HRS 2017). 1 2 2 3 4 Definition AF ablation is the treatment strategy for the maintenance of sinus rhythm and improvement of quality of life in patients with symptomatic AF. 2 Epidemiology AF ablation is indicated in patients with paroxysmal AF and persistent AF. 3 Disease course Catheter ablation of AF goal is to eliminate triggers and/or modify the arrhythmogenic substrate by pulmonary vein isolation in addition to different strategies using various technologies such as electro-anatomical mapping system and cryo-balloon technology. AF ablation maintains sinus rhythm, improves exercise capacity, quality of life, morbidity (stroke), and mortality. 4 Prognosis and risk of recurrence https://web.pathway.md/diseases/reczy5g2bZ5EnxkfY 1/4 6/23/23, 3:15 AM Atrial fibrillation ablation Pathway og os s a d s o ecu e ce AF ablation is associated with lower mortality risk (HR 0.50, 95% CI 0.37-0.62) and stroke/TIA risk (HR 0.62, 95% CI 0.44-0.86). 2 Guidelines 1. Diagnostic investigations Echocardiography: Consider TEE before catheter ablation in patients who are in AF at the time of the procedure and have been receiving therapeutic anticoagulation for 3 weeks or longer. C Consider TEE before catheter ablation in patients who are in sinus rhythm and at the time of the procedure and have not been receiving therapeutic anticoagulation for 3 weeks or longer. C Pre-procedural mapping: insufficient evidence to assess the usefulness of voltage mapping or MRI to identify areas of abnormal myocardial tissue and guide ablation in patients with persistent or long-standing persistent AF. I 2. Medical management Pre-procedural anticoagulation: perform AF catheter ablation without interruption of warfarin or dabigatran in patients who have been anticoagulated with these agents. A Show 2 more Per-procedural anticoagulation: administer heparin prior to or immediately following transseptal puncture during AF catheter ablation procedures, and adjust to achieve and maintain an ACT 300 s. B Post-procedural anticoagulation: adhere to AF anticoagulation guidelines for patients who have undergone an AF ablation procedure, regardless of the apparent success or failure of the procedure. B Management of antiarrythmics: insufficient evidence to assess the usefulness of discontinuation of antiarrhythmic drug therapy before AF ablation in an attempt to improve long- term outcomes. I Management of sleep apnea: consider screening for signs and symptoms of sleep apnea when evaluating a patient for AF ablation, and obtaining a sleep evaluation if sleep apnea is suspected. C 3. Nonpharmacologic interventions Weight loss: consider advising weight loss to patients who are being evaluated to undergo an AF ablation procedure, as part of a comprehensive risk factor management strategy. C https://web.pathway.md/diseases/reczy5g2bZ5EnxkfY 2/4 6/23/23, 3:15 AM Atrial fibrillation ablation Pathway 4. Therapeutic procedures Indications for catheter ablation (prior to initiation of medication): consider catheter ablation for patients with paroxysmal AF prior to initiation of antiarrythmic medication. C Indications for catheter ablation (failure of antiarrhythmic medication): Perform catheter ablation for patients with paroxysmal AF refractory to antiarrythmic medication. A Consider catheter ablation for patients with persistent AF refractory to antiarrythmic medication. C Pulmonary vein isolation: perform electrical isolation of the pulmonary veins during all AF ablation procedures. A Other ablation techniques: perform a linear ablation of the cavotricuspid isthmus in patients with a history of typical atrial flutter, or if typical atrial flutter is induced at the time of AF ablation. B 5. Perioperative care Safety precautions: identify the pulmonary vein ostia carefully to avoid ablation within the pulmonary veins. B Fractionated atrial electrograms: insufficient evidence to assess the usefulness of ablation of complex fractionated atrial electrograms as an initial or repeat ablation strategy in patients with persistent and long-standing AF. I High-dose isoproterenol: consider administration of high-dose isoproterenol to screen for and then ablate non-pulmonary vein triggers during initial or repeat AF ablation procedures in patients with paroxysmal, persistent, or long-standing persistent AF. C 6. Surgical interventions Indications for surgical ablation (prior to initiation of medication): perform surgical ablation in patients with paroxysmal AF undergoing concomitant open cardiac surgery for other indications. B Indications for surgical ablation (failure of antiarrhythmic medication): perform surgical ablation of AF in patients with paroxysmal AF refractory to antiarrythmic medications who are undergoing concomitant open cardiac surgery for other indications. B Indications for surgical ablation (stand-alone procedure): Consider stand-alone surgical ablation for paroxysmal AF in patients who have failed one or more attempts at catheter ablation and also for those who are intolerant or refractory to antiarrhythmic drug therapy and prefer a surgical approach. C Consider stand-alone surgical ablation for persistent AF in patients who have failed one or more attempts at catheter ablation and for those patients who prefer a surgical approach. C https://web.pathway.md/diseases/reczy5g2bZ5EnxkfY 3/4 6/23/23, 3:15 AM Atrial fibrillation ablation Pathway 7. Specific circumstances High-level athletes: consider AF ablation as first-line therapy for high-level athletes with AF, due to the negative effects of medications on athletic performance. C Young patients (<45 years of age): consider using similar indications for AF ablation in young patients with AF < 45 years of age, as in older patients. C Elderly patients (> 75 years of age): consider using similar indications for AF ablation in selected older patients with AF as in younger patients. C Patients with asymptomatic disease: Consider catheter ablation in select patients with asymptomatic, paroxysmal AF. C Consider catheter ablation in select patients with asymptomatic, persistent AF. C Patients with congestive heart failure: consider AF ablation in selected patients with HF using the same indications as in patients without HF. C Patients with hypertrophic cardiomyopathy: consider using similar indications for AF ablation in selected patients with hypertrophic cardiomyopathy as in patients without hypertrophic cardiomyopathy. C Patients with tachy-brady syndrome: consider AF ablation as an alternative to pacemaker implantation in patients with tachycardia-bradycardia syndrome. C References 1. Calkins H, Hindricks G, Cappato R et al. 2017 HRS/EHRA/ECAS/APHRS/SOLAECE expert consensus statement on catheter and surgical ablation of atrial fibrillation: Executive summary. J Arrhythm. 2017 Oct;33 5 369 409. Open 2. Xue-Yuan Guo, Chang-Sheng Ma. Atrial Fibrillation Ablation: Indications, Outcomes, Complications, and Future Directions. 2017 Aug 20;130 16 1891 1893.2017 Aug 20;130 16 1891 1893. Open 3. Nebojsa Mujovic, Milan Marinkovic, Radoslaw Lenarczyk et al. Catheter Ablation of Atrial Fibrillation: An Overview for Clinicians. 2017 Aug;34 8 1897 1917.2017 Aug;34 8 1897 1917. Open 4. Alicia Darge, Matthew R Reynolds, Joseph J Germano. Advances in atrial fibrillation ablation. 2009 May;21 5 247 54.2009 May;21 5 247 54. Open https://web.pathway.md/diseases/reczy5g2bZ5EnxkfY 4/4
Guideline sources The following summarized guidelines for the evaluation and management of atrial fibrillation are prepared by our editorial team based on guidelines from the Society for Cardiovascular Angiography and Interventions (SCAI/HRS 2023), the European Society of Cardiology (ESC/EACTS/EHRA 2021), the American Heart Association (AHA/HRS/ACC 2019), the National Heart Foundation of Australia (NHFA 2018), the Canadian Cardiovascular Society (CCS 2018; 2014), the U.S. Preventive Services Task Force (USPSTF 2018), and the Society of Thoracic Surgeons (STS 2017). 1 2 3 4 5 6 7 8 9 9 10 11 12 Definition AF is a cardiac arrhythmia characterized by a diffuse and abnormal pattern of electrical activity in the atria of the heart. 9 E id i l https://web.pathway.md/diseases/recZrV7qZIxyavdK7 1/20 6/23/23, 3:15 AM Atrial fibrillation Pathway Epidemiology The development of AF is related to structural and electrophysiological abnormalities resulting from comorbid conditions (including hypertension, diabetes mellitus, obesity, obstructive sleep apnea, myocardial infarction, HF), genetics, sex, and other factors. 11 Pathophysiology The prevalence of AF in the United States ranges is estimated at 700-775 cases per 100,000 persons. 10 Disease course In patients with AF, rapid and irregular atrial contractions lead to tachyarrhythmias, which lead to symptoms of palpitations, dyspnea, and an increased risk of HF; as well as stasis of blood in the LA appendage, which increases the risk of stroke and systemic embolism. 12 Prognosis and risk of recurrence AF is estimated to cause 15% of all strokes and is associated with a 5-fold increased risk of stroke and a 2-fold risk for all-cause mortality, respectively. 9 Calculator Calculator Calculator Anticoagulation and risk factors CHA DS VASc score for stroke CHADS s Guidelines 1. Screening and diagnosis Indications for screening: As per ESC 2021 guidelines, consider offering systematic ECG screening to detect AF in individuals 75 years of age, or in patients at high risk of stroke. C Show 4 more As per NHFA 2018 guidelines, obtain opportunistic point-of-care screening for AF in the clinic or community in individuals 65 years of age. B Show 2 more As per USPSTF 2018 guidelines, insufficient evidence to assess the balance of benefits and harms of screening for AF with ECG. I Indications for testing: As per ESC 2021 guidelines: https://web.pathway.md/diseases/recZrV7qZIxyavdK7 2/20 6/23/23, 3:15 AM Atrial fibrillation Pathway Obtain short-term ECG recording for at least the first 24 hours, followed by continuous ECG monitoring for at least 72 hours whenever possible, to monitor for AF in patients with acute ischemic stroke or TIA without previously known AF. B Consider obtaining long-term noninvasive ECG monitoring or placing insertable cardiac monitors for additional monitoring for AF in selected patients with stroke without previously known AF. C As per AHA 2019 guidelines, consider implantation of a cardiac monitor to optimize detection of silent AF in patients with cryptogenic stroke in whom external ambulatory monitoring is inconclusive. C As per CCS 2014 guidelines: Obtain continuous ECG monitoring for at least 24 hours to screen for paroxysmal AF in patients with acute ischemic stroke or TIA potentially eligible for OAC therapy. B Consider obtaining additional ambulatory monitoring (beyond 24 hours) in selected older patients with an acute, non-lacunar, embolic stroke of undetermined source and suspected (but unproven) AF potentially eligible for OAC therapy. C Diagnostic criteria: make the diagnosis of AF based on a standard 12-lead ECG recording or a single-lead ECG tracing of 30 seconds showing heart rhythm with no discernible repeating P waves and irregular RR intervals (when atrioventricular conduction is not impaired). B 2. Classification and risk stratification Structured characterization: consider obtaining structured characterization of AF including clinical assessment of stroke risk, symptom status, burden of AF, and evaluation of substrate in all patients with AF, to streamline the assessment of patients with AF at different healthcare levels, inform treatment decision-making, and facilitate optimal management. C Stroke risk assessment: As per ESC 2021 guidelines, use the CHA2DS2-VASc clinical stroke risk score assessment to initially identify patients at low risk of stroke (CHA2DS2-VASc score of 0 in males and 1 in females). A Show 2 more As per AHA 2019 guidelines: Administer OACs for patients with AF and an elevated CHA2DS2-VASc score of 2 in males or 3 in females. A Use the CHA2DS2-VASc score for assessment of stroke risk in patients with AF, except with moderate-to-severe mitral stenosis or a mechanical heart valve. B As per NHFA 2018 guidelines, use the CHA2DS2-VA score - the sexless CHA2DS2-VASc score - for predicting stroke risk in patients with AF. B As per CCS 2014 guidelines, stratify all patients with AF or atrial flutter, whether paroxysmal or persistent, using a predictive index for stroke risk. A Bleeding risk assessment: https://web.pathway.md/diseases/recZrV7qZIxyavdK7 3/20 6/23/23, 3:15 AM Atrial fibrillation Pathway As per ESC 2021 guidelines, obtain a formal structured risk-score-based bleeding risk assessment to help identify non-modifiable and address modifiable bleeding risk factors in all patients with AF, and to identify patients potentially at high risk of bleeding requiring early and more frequent clinical review and follow-up. B Show 2 more As per NHFA 2018 guidelines, initiate anticoagulant therapy after addressing bleeding and if the stroke risk is believed to exceed the risk of further bleeding. B Show 2 more 3. Diagnostic investigations Clinical assessment: Evaluate AF-related symptoms (including fatigue, tiredness, exertional shortness of breath, palpitations, and chest pain) and quantify the patient symptom status using the modified European Heart Rhythm Association symptom scale before and after initiation of treatment. B Evaluate AF-related symptoms before and after cardioversion of persistent AF to aid rhythm control treatment decisions. B Echocardiography: As per AHA 2019 guidelines, consider obtaining TEE before cardioversion in patients with AF of 48 hours or of unknown duration not received anticoagulation for the preceding 3 weeks. C As per CCS 2018 guidelines, consider obtaining TEE to exclude cardiac thrombus before cardioversion as an alternative to at least 3 weeks of anticoagulation. C As per NHFA 2018 guidelines, obtain TTE in all patients with newly diagnosed AF. B Laboratory testing: As per AHA 2019 guidelines: Determine the INR among patients treated with warfarin at least weekly during initiation of anticoagulant therapy and at least monthly when anticoagulation is stable. A Evaluate renal and hepatic function before initiation of a novel OAC and reevaluate at least annually. B As per NHFA 2018 guidelines, obtain TSH testing in patients with newly diagnosed AF but delay in acutely ill patients. B Evaluation for sleep apnea: obtain polysomnography for the screening of sleep apnea in patients with recurrent symptomatic AF. B 4. Medical management General principles: As per ESC 2021 guidelines, offer an integrated management with a structured multidisciplinary approach including healthcare professionals, patients, and their family/carers in all patients with https://web.pathway.md/diseases/recZrV7qZIxyavdK7 4/20 6/23/23, 3:15 AM Atrial fibrillation Pathway AF to improve clinical outcomes. B Show 4 more As per CCS 2018 guidelines: Implement systematic and strict guideline-adherent management of traditional modifiable cardiovascular risk factors and/or conditions associated with AF, to reduce cardiovascular events. A Consider targeting modifiable risk markers and conditions associated with AF should be applied to prevent recurrence of the arrhythmia and/or decrease its symptom burden. C As per NHFA 2018 guidelines, adopt an integrated care approach aiming to provide patient- centered comprehensive treatment delivered by a multidisciplinary team. A Show 4 more As per STS 2017 guidelines, consider offering multidisciplinary heart team assessment, treatment planning, and long-term follow-up to optimize patient outcomes in the treatment of AF. B Rate control: As per ESC 2021 guidelines, consider setting a resting HR of < 110 bpm (lenient rate control) as the initial HR target for rate control therapy. C Show 4 more As per AHA 2019 guidelines: Consider administration of nondihydropyridine calcium antagonists to slow a rapid ventricular response in patients with acute coronary syndrome and AF only in the absence of significant HF or hemodynamic instability. C Consider administering amiodarone or digoxin to slow a rapid ventricular response in patients with acute coronary syndrome and AF associated with severe LV dysfunction and HF or hemodynamic instability. C As per NHFA 2018 guidelines, administer -blockers or nondihydropyridine CCBs for acute control of ventricular rate in hemodynamically stable patients with AF, although caution is needed if given IV. B Show 7 more Rhythm control (general indications): Administer rhythm control therapy for symptom and quality of life improvement in symptomatic patients with AF. A Perform cardioversion of AF (either pharmacological or electrical) in symptomatic patients with persistent AF as part of rhythm control therapy. B Rhythm control, pharmacological cardioversion: As per EACTS/EHRA/ESC 2021 guidelines, administer pharmacological cardioversion of AF only in a hemodynamically stable patient, after consideration of the thromboembolic risk. B Show 5 more As per NHFA 2018 guidelines, consider administering IV amiodarone for delayed conversion to sinus rhythm in patients with AF and structural heart disease, including patients with HF and https://web.pathway.md/diseases/recZrV7qZIxyavdK7 5/20 6/23/23, 3:15 AM Atrial fibrillation Pathway coronary artery disease. B Rhythm control, maintenance therapy: As per EACTS/EHRA/ESC 2021 guidelines, administer amiodarone for long-term rhythm control in all patients with AF, including those with HFrEF. Consider administering other antiarrhythmic drugs first whenever possible, owing to its extracardiac toxicity. A Show 6 more As per NHFA 2018 guidelines, consider administering -blockers for the maintenance of sinus rhythm in patients with AF. C Show 6 more Anticoagulation, general indications: As per EACTS/EHRA/ESC 2021 guidelines, initiate OACs for stroke prevention in patients with AF and CHA2DS2-VASc score of 2 in males and 3 in females. A Show 4 more As per HRS/ACC/AHA 2019 guidelines, select anticoagulant therapy based on the risk of thromboembolism, irrespective of whether the AF pattern is paroxysmal, persistent, or permanent. B Show 2 more As per HRS/ACC/AHA 2019 guidelines: Consider prescribing an OAC to reduce thromboembolic stroke risk for patients with AF (except with moderate-to-severe mitral stenosis or a mechanical heart valve) and a CHA2DS2-VASc score of 1 in males and 2 in females. C Consider omitting anticoagulant therapy for patients with AF (except with moderate-to-severe mitral stenosis or a mechanical heart valve) and a CHA2DS2-VASc score of 0 in males or 1 in females. C As per CCS 2018 guidelines, initiate oral anticoagulation therapy in most patients with AF aged 65 years or with a CHADS2 score 1. B Landmark trials: AVERROES In patients with AF who were at increased risk for stroke and for whom vitamin K antagonist therapy was unsuitable, apixaban was superior to aspirin with respect to the incidence of stroke or systemic embolism. Connolly SJ et al. N Engl J Med. 2011 Mar 3. As per NHFA 2018 guidelines, initiate OACs for the prevention of stroke and systemic embolism in patients with nonvalvular AF and a CHA2DS2-VA score of 2, unless there are contraindications to anticoagulation. A Show 3 more Anticoagulation, direct oral anticoagulants: https://web.pathway.md/diseases/recZrV7qZIxyavdK7 6/20 6/23/23, 3:15 AM Atrial fibrillation Pathway As per EACTS/EHRA/ESC 2021 guidelines: Prefer direct OACs over vitamin K antagonists (excluding patients with mechanical heart valves or moderate-to-severe mitral stenosis) for stroke prevention in patients with AF eligible for oral anticoagulation. A Consider (re)initiating OACs in patients with AF at high risk of ischemic stroke, with preference for direct OACs over vitamin K antagonists in direct OAC-eligible patients, in consultation with a neurologist/stroke specialist after: a trauma-related ICH acute spontaneous ICH (including subdural, subarachnoid, or intracerebral hemorrhage), after careful consideration of risks and benefits. C As per HRS/ACC/AHA 2019 guidelines: Administer novel OACs (such as dabigatran, rivaroxaban, apixaban, and edoxaban) rather than warfarin in novel OAC-eligible patients with AF (except with moderate-to-severe mitral stenosis or a mechanical heart valve). A Administer a novel OAC for patients with AF (except with moderate-to-severe mitral stenosis or a mechanical heart valve) who are unable to maintain a therapeutic INR level with warfarin. B Updated evidence: INVICTUS In patients with AF and echocardiographically documented rheumatic heart disease, rivaroxaban was not noninferior to vitamin K antagonist with respect to the incidence of composite outcome of stroke, systemic embolism, myocardial infarction, or death from vascular or unknown causes. Stuart J Connolly et al. N Engl J Med. 2022 Sep 15. As per CCS 2018 guidelines, use dabigatran, rivaroxaban, apixaban, or edoxaban in most patients with nonvalvular AF in whom oral anticoagulation is indicated. A Landmark trials: ENGAGE AF-TIMI 48 In patients with moderate-to-high-risk AF, high-dose and low-dose once-daily regimens of edoxaban, as compared with warfarin, were noninferior to warfarin with respect to the prevention of stroke or systemic embolism. The annualized rate of primary end point in high- dose edoxaban vs. warfarin was 1.18% vs. 1.50% (HR 0.79, 97.5% CI 0.63-0.99, p < 0.001 for noninferiority) and in low-dose edoxaban vs. warfarin was 1.61% vs. 1.50% (HR 1.07, 97.5% CI 0.87-1.31, p = 0.005 for noninferiority). There was a favorable trend for high-dose edoxaban vs. warfarin (HR 0.87, 97.5% CI 0.73-1.04, p = 0.08) and an unfavorable trend for low-dose edoxaban vs. warfarin (HR 1.13, 97.5% CI 0.96-1.34, p = 0.10). The annualized rate of major bleeding with high-dose edoxaban vs. warfarin was 2.75% vs. 3.43% (HR 0.80, 95% CI 0.71-0.91, p < 0.001) and with low-dose edoxaban vs. warfarin was 1.61% vs. 3.43% https://web.pathway.md/diseases/recZrV7qZIxyavdK7 7/20 6/23/23, 3:15 AM Atrial fibrillation Pathway (HR 0.47, 95% CI 0.41-0.55, p < 0.001). The corresponding annualized rates of death from cardiovascular causes were 2.74% vs. 3.17% (HR 0.86, 95% CI 0.77-0.97, p = 0.01) and 2.71% vs. 3.17% (HR 0.85, 95% CI 0.76-0.96, p = 0.008). The corresponding rates of the key secondary end point were 3.85% vs. 4.43% (HR 0.87, 95% CI 0.78-0.96, p = 0.005) and 4.23% vs. 4.43% (HR 0.95, 95% CI 0.86-1.05, p = 0.32). Giugliano RP et al. N Engl J Med. 2013 Nov 28. As per NHFA 2018 guidelines, prefer a direct OAC (apixaban, dabigatran, or rivaroxaban) over warfarin in patients with nonvalvular AF requiring oral anticoagulation. B Anticoagulation, vitamin K antagonists: As per EACTS/EHRA/ESC 2021 guidelines: Target an INR of 2-3 if a vitamin K antagonist is used, with individual time in therapeutic range 70%. B Undertake one of the following options in patients taking vitamin K antagonists with low time in INR therapeutic range, such as < 70%: switch to a direct OAC but ensure good adherence and persistence with therapy B attempt to improve time in therapeutic range, such as education/counseling and more frequent INR checks. B As per CCS 2018 guidelines, prefer warfarin over direct OACs, if oral anticoagulation is required, in patients with a mechanical prosthetic valve, rheumatic mitral stenosis, or estimated GFR of 15-30 mL/min/1.73 m . B As per NHFA 2018 guidelines, administer warfarin in patients with valvular AF (mechanical heart valves or moderate-to-severe mitral stenosis). B Show 2 more Anticoagulation, peri-cardioversion: As per EACTS/EHRA/ESC 2021 guidelines, administer effective anticoagulation for a minimum of 3 weeks before cardioversion of AF. B Show 7 more As per HRS/ACC/AHA 2019 guidelines, consider administering heparin, a factor Xa inhibitor, or a direct thrombin inhibitor in patients with AF of < 48 hours duration with a CHA2DS2-VASc score of 0 in males and 1 in females before cardioversion, without the need for post- cardioversion oral anticoagulation. C Show 3 more As per CCS 2018 guidelines, administer therapeutic anticoagulation for 3 weeks before cardioversion in most hemodynamically stable patients with AF or atrial flutter for whom elective electrical or pharmacological cardioversion is planned. B Show 4 more As per NHFA 2018 guidelines, administer anticoagulation at the time of electrical or pharmacological cardioversion, and for at least 4 weeks post-procedurally. B https://web.pathway.md/diseases/recZrV7qZIxyavdK7 8/20 6/23/23, 3:15 AM Atrial fibrillation Pathway Anticoagulation (peri-catheter ablation): initiate pre-procedural anticoagulation in patients with AF and risk factors of stroke not taking OACs for at least 3 weeks before ablation B , or alternatively obtain TEE to exclude LA thrombus before ablation. B Show 2 more Antiplatelet therapy: As per ESC 2021 guidelines, do not administer antiplatelet therapy alone (monotherapy or aspirin in combination with clopidogrel) for stroke prevention in patients with AF. D Show 6 more As per AHA 2019 guidelines, consider initiating double therapy with a P2Y12 inhibitor (such as clopidogrel or ticagrelor) and dose-adjusted vitamin K antagonist, in patients with AF at increased risk of stroke (based on CHA2DS2-VASc risk score of 2) who have undergone PCI with stenting for acute coronary syndrome, to reduce the risk of bleeding as compared with triple therapy. C Show 4 more As per CCS 2018 guidelines, consider using triple therapy (aspirin 81 mg PO once daily, clopidogrel 75 mg PO once daily, and a reduced dose OAC) for patients with AF 65 years of age or with a CHADS2 score 1 undergoing high-risk elective PCI or PCI for acute coronary syndrome. OAC options for triple pathway therapy include: rivaroxaban 2.5 mg PO BID (preferred) warfarin with a target INR 2-2.5. B Show 5 more As per NHFA 2018 guidelines: Administer low-dose aspirin (100 mg) and clopidogrel (75 mg) in patients with AF if dual antiplatelet therapy is required in combination with OACs. Do not use ticagrelor or prasugrel in this situation. B Administer triple therapy (aspirin, P2Y12 inhibitor and OAC) with a duration as short as possible to minimize bleeding, while ensuring coverage of the initial period of high risk of stent thrombosis and/or recurrent coronary ischemia. B 5. Nonpharmacologic interventions Lifestyle modifications: As per ESC 2021 guidelines, advise strict control of risk factors and avoiding triggers as part of a rhythm control strategy. B Show 6 more As per AHA 2019 guidelines, advise weight loss, combined with risk factor modification for overweight and obese patients with AF. B As per NHFA 2018 guidelines: Advise intensive management of weight to a target of 10% body weight loss, aiming for a BMI of < 27 in overweight and obese patients with AF. B https://web.pathway.md/diseases/recZrV7qZIxyavdK7 9/20 6/23/23, 3:15 AM Atrial fibrillation Pathway Offer concomitant management of associated cardiovascular risk factors to target levels in overweight and obese patients with AF. B 6. Therapeutic procedures Electrical cardioversion: Perform emergency electrical cardioversion in patients with AF and acute or worsening hemodynamic instability. B Show 3 more Initiate anticoagulation as soon as possible in patients with AF of > 48 hours duration or unknown duration requiring immediate cardioversion for hemodynamic instability, and continue for at least 4 weeks after cardioversion unless contraindicated. B Decide on long-term anticoagulation therapy based on the thromboembolic and bleeding risk profiles after cardioversion for AF of any duration. B Consider performing immediate electrical cardioversion in patients whose recent-onset AF or atrial flutter is the direct cause of instability with hypotension, acute coronary syndrome, or pulmonary edema. B Perform urgent electrical cardioversion in hemodynamically unstable patients with AF. B Consider performing electrical cardioversion (either as first-line option or when pharmacological rhythm control fails) in hemodynamically stable patients with AF, after consideration of thromboembolic risk. B Catheter ablation: As per ESC 2021 guidelines, take into consideration the procedural risks and the major risk factors for recurrence following the procedure for the decision on catheter ablation of AF, and discuss the risks with the patient. B Show 10 more As per AHA 2019 guidelines, consider performing catheter ablation in selected patients with symptomatic AF and HFrEF to potentially lower mortality rate and reduce hospitalization for HF. C As per NHFA 2018 guidelines, perform catheter ablation of the atrioventricular node after a permanent pacing device has been implanted, if pharmacological rate control fails. B Show 3 more As per CCS 2014 guidelines, perform catheter ablation of AF in patients remaining symptomatic after an adequate trial of antiarrhythmic drug therapy and if a rhythm control strategy remains desired. B Atrioventricular node ablation: consider performing atrioventricular node ablation to control HR in patients with AF unresponsive or intolerant to intensive rate and rhythm control therapy, and not eligible for rhythm control by LA ablation, recognizing that these patients will become pacemaker dependent. C https://web.pathway.md/diseases/recZrV7qZIxyavdK7 10/20 6/23/23, 3:15 AM Atrial fibrillation Pathway 7. Perioperative care Pre-procedural anticoagulation interruption: balance the risks of a thromboembolic event (as indicated by a higher CHADS2 score, mechanical heart valve, or rheumatic heart disease) against a bleeding event (as indicated by a higher HASBLED score and procedures with higher bleeding risks) in patients with atrial flutter/AF to decide on interrupting antithrombotic therapy for an invasive procedure. B Show 6 more Pre-procedural anticoagulation bridging: As per AHA 2019 guidelines: Bridge with UFH or LMWH in patients with AF and a mechanical heart valve undergoing procedures requiring interruption of warfarin. Recognize that decisions on bridging therapy should balance the risks of stroke and bleeding. B Recognize that decisions about bridging therapy (UFH or LMWH) in patients with AF without mechanical heart valves requiring interruption of warfarin for procedures should balance the risks of stroke and bleeding and the duration of time the patient will not be anticoagulated. B As per NHFA 2018 guidelines, avoid bridging with LMWH or UFH in patients treated with warfarin, at low-to-moderate risk of stroke undergoing planned surgical intervention. D Show 2 more As per CCS 2014 guidelines, consider bridging with LMWH or UFH when the INR is below the therapeutic INR target when a decision to interrupt warfarin therapy for an invasive procedure has been made in patients with atrial flutter/AF at high risk of thromboembolic events (CHADS2 score 3, mechanical heart valve, stroke or TIA within 3 months, rheumatic heart disease). C Show 2 more Post-procedural resumption of anticoagulation: Consider restarting therapy once hemostasis is established when warfarin, acetylsalicylic acid, or clopidogrel have been withdrawn for an invasive procedure (usually 24-48 hours for procedures at low risk of bleeding). C Consider restarting therapy after the procedure 1 day after hemostasis is established when apixaban, dabigatran, or rivaroxaban have been withdrawn for an invasive procedure. C 8. Surgical interventions Left atrial appendage closure, indications: As per SCAI/HRS 2023 guidelines, consider performing transcatheter LA appendage closure in patients with nonvalvular AF with high thromboembolic risk unsuitable for long-term oral anticoagulation and having adequate life expectancy (minimum > 1 year) and quality of life to benefit from LA appendage closure. Ensure a patient-provider discussion for shared decision- making. E As per EACTS/EHRA/ESC 2021 guidelines: https://web.pathway.md/diseases/recZrV7qZIxyavdK7 11/20 6/23/23, 3:15 AM Atrial fibrillation Pathway Consider performing LA appendage occlusion for stroke prevention in patients with AF and contraindications for long-term anticoagulant treatment (such as intracranial bleeding without a reversible cause). C Consider performing surgical occlusion or exclusion of the LA appendage for stroke prevention in patients with AF undergoing cardiac surgery. C As per HRS/ACC/AHA 2019 guidelines: Consider performing surgical occlusion of the LA appendage in patients with AF undergoing cardiac surgery S4.4.2-1, as a component of an overall heart team approach to the management of AF. C Consider performing percutaneous LA appendage occlusion in patients with AF at increased risk of stroke who have contraindications to long-term anticoagulation. C As per NHFA 2018 guidelines, consider performing LA appendage occlusion for stroke prevention in patients with nonvalvular AF at moderate to high risk of stroke and with contraindications to OACs. B As per STS 2017 guidelines, consider performing LA appendage excision or exclusion in conjunction with surgical ablation for AF for long-term prevention of morbidity from thromboembolic events. C Left atrial appendage closure (perioperative imaging): obtain baseline TEE or cardiac CT before LA appendage closure. E Show 2 more Left atrial appendage closure, technical considerations: As per SCAI/HRS 2023 guidelines, comply with the testing and labeling of each specific LA appendage closure device for procedural venous access, anticoagulation, transseptal puncture, delivery sheath selection and placement, left atria pressure measurement, and device deployment. E Show 4 more As per CCS 2014 guidelines, avoid using non-approved LA appendage closure devices, except in the context of research, or in systematically documented use protocols in patients at high risk of stroke (CHADS2 score 2) if antithrombotic therapy is contraindicated. D Left atrial appendage closure (management of complications): ensure that operators are familiar with avoidance, recognition, and management of complications associated with LA appendage closure. E Show 4 more Left atrial appendage closure, postoperative anticoagulation: As per SCAI/HRS 2023 guidelines, initiate antithrombotic therapy with warfarin, direct OACs, or dual antiplatelet therapy after LA appendage closure according to the studied regimen and instructions for use for each specific device and tailored to the bleeding risks of each patient. E As per EACTS/EHRA/ESC 2021 guidelines, initiate long-term oral anticoagulation in patients after surgery for AF and appendage closure, based on the patient's thromboembolic risk assessed with the CHA2DS2-VASc score. B https://web.pathway.md/diseases/recZrV7qZIxyavdK7 12/20 6/23/23, 3:15 AM Atrial fibrillation Pathway Surgical ablation: As per ESC 2021 guidelines, consider performing concomitant ablation of AF in patients undergoing cardiac surgery, balancing the benefits of freedom from atrial arrhythmias and the risk factors for recurrence (LA dilatation, years in AF, age, renal dysfunction, and other cardiovascular risk factors). B Show 2 more As per NHFA 2018 guidelines: Consider performing surgical ablation of AF to restore sinus rhythm in the context of concomitant cardiac surgery in patients with symptomatic paroxysmal, persistent, or long- standing persistent AF. B Consider performing stand-alone surgical or hybrid ablation of AF in patients with symptomatic paroxysmal, persistent or long-standing persistent AF if: it is refractory or intolerant to at least one Class I or III antiarrhythmic drug percutaneous ablation failed the likelihood of successful percutaneous ablation is low. C As per STS 2017 guidelines, perform surgical ablation of AF in patients undergoing concomitant operations on the mitral valve. A Show 4 more 9. Specific circumstances Subclinical atrial fibrillation: As per ESC 2021 guidelines, obtain the following in patients with atrial high rate episodes/subclinical AF detected by cardiac implantable electronic devices or insertable cardiac monitor: complete cardiovascular evaluation with ECG recording, clinical risk factors/comorbidity evaluation, and thromboembolic risk assessment using the CHA2DS2-VASc score continued patient follow-up and monitoring (preferably with the support of remote monitoring) to detect progression to clinical AF, monitor the atrial high rate episodes/subclinical AF burden (especially transition to 24 hours), and detect changes in underlying clinical conditions. B As per AHA 2019 guidelines, perform further evaluation to document clinically relevant AF to guide treatment decisions in patients with cardiac implantable electronic devices (such as pacemakers or implanted cardioverter-defibrillators), in case of recorded atrial high-rate episodes. B As per CCS 2018 guidelines, consider initiating oral anticoagulation in patients who are aged 65 years or older or with a CHADS2 score of 1 who have episodes of subclinical AF lasting > 24 continuous hours in duration. High-risk patients (such as those with a recent embolic stroke of unknown source) with shorter-lasting episodes might also be considered for oral anticoagulation. C Postoperative atrial fibrillation (prevention): https://web.pathway.md/diseases/recZrV7qZIxyavdK7 13/20 6/23/23, 3:15 AM Atrial fibrillation Pathway Administer perioperative amiodarone or -blockers for the prevention of postoperative AF after cardiac surgery. A Do not administer -blockers routinely for the prevention of postoperative AF in patients undergoing non-cardiac surgery. D Postoperative atrial fibrillation (anticoagulation): consider initiating long-term OACs for the prevention of thromboembolic events, considering the anticipated net clinical benefit of OACs and informed patient preferences, in patients at risk for stroke with postoperative AF after either non- cardiac C or cardiac surgery. C Patients with active bleeding: As per ESC 2021 guidelines, interrupt OACs in patients with AF and severe active bleeding until the cause of bleeding is identified and active bleeding is resolved. B Show 2 more As per AHA 2019 guidelines: Administer idarucizumab for the reversal of dabigatran in the event of life-threatening bleeding or an urgent procedure. B Consider administering andexanet alfa for the reversal of rivaroxaban and apixaban in the event of life-threatening or uncontrolled bleeding. C As per NHFA 2018 guidelines, administer idarucizumab in patients taking dabigatran experiencing life-threatening bleeding or requiring emergency surgery. B Show 2 more Patients with acute ischemic stroke: As per ESC 2021 guidelines: Initiate long-term oral anticoagulation (with a preference for direct oral coagulants over vitamin K antagonists in direct oral coagulant-eligible patients) for secondary prevention of stroke in patients with AF and an ischemic stroke or TIA, in the absence of strict contraindications to OACs. A Do not administer very early anticoagulation (< 48 hours) using UFH, LMWH, or vitamin K antagonists in patients with AF presenting with acute ischemic stroke. D As per NHFA 2018 guidelines: Do not initiate anticoagulants in the first few days after an ischemic stroke because of the risk of hemorrhage or hemorrhagic transformation of infarction. D Consider delaying the initiation of OACs for 2 weeks, but making the decision before discharge, in patients with ischemic stroke. C Patients with acute coronary syndromes: administer anticoagulation for patients with acute coronary syndrome and AF at increased risk of systemic thromboembolism (based on CHA2DS2- VASc risk score of 2), unless the bleeding risk exceeds the expected benefit. B Show 2 more Patients with valvular heart disease: As per ESC 2021 guidelines, do not use direct OACs in patients with AF and: https://web.pathway.md/diseases/recZrV7qZIxyavdK7 14/20 6/23/23, 3:15 AM Atrial fibrillation Pathway prosthetic mechanical valve D moderate-to-severe mitral stenosis. D As per AHA 2019 guidelines: Administer warfarin in patients with AF who have mechanical heart valves. B Avoid administering the direct thrombin inhibitor dabigatran in patients with AF and a mechanical heart valve. D Patients with coronary/arterial vascular disease: use a direct OAC in preference to warfarin when oral anticoagulation is indicated in the presence of coronary or arterial vascular disease. B Show 2 more Patients with congenital heart disease: consider initiating OACs in all adult patients with intracardiac repair, cyanosis, Fontan palliation, or systemic right ventricle and a history of AF. C Show 5 more Patients with chronic kidney disease: As per AHA 2019 guidelines, avoid administering the direct thrombin inhibitor dabigatran or the factor Xa inhibitors rivaroxaban or edoxaban in patients with AF and end-stage CKD or on dialysis, because of the lack of evidence from clinical trials that benefit exceeds risk. D Show 2 more As per NHFA 2018 guidelines: Individualize the decision to use anticoagulants in patients with AF and severe CKD because there are no prospective data showing benefit in this population. B Administer warfarin if a patient with AF with severe CKD requires anticoagulant therapy. B Pregnant patients (rate control): Administer selective -blockers for rate control in pregnant patients with AF. B Consider administering digoxin or verapamil for rate control in pregnant patients with AF if - blockers fail. C Pregnant patients (cardioversion): perform immediate electrical cardioversion in pregnant patients with AF and hemodynamic instability or pre-excited AF. B Show 2 more Pregnant patients (maintenance therapy): consider administering flecainide, propafenone, or sotalol for the prevention of AF if atrioventricular nodal-blocking drugs fail. C Pregnant patients (anticoagulation): administer therapeutic anticoagulation with heparin or vitamin K antagonist according to the stage of pregnancy in pregnant patients with AF. B 10. Patient education General counseling: As per ESC 2021 guidelines: To optimize shared decision-making about specific treatment options in consideration: https://web.pathway.md/diseases/recZrV7qZIxyavdK7 15/20 6/23/23, 3:15 AM Atrial fibrillation Pathway inform patients about the advantages/limitations and benefit/risks associated with treatment options discuss the potential burden of the treatment and include the patient's perception of treatment burden in the treatment decision. B Counsel patients and strongly emphasize about the importance of adherence and persistence to treatment with direct anticoagulants both before and after cardioversion. B As per NHFA 2018 guidelines: Provide targeted education throughout the continuum of management in patients with AF. A Take into consideration patients' beliefs, values, and preferences in the shared decision- making, with a goal of empowering patients to undertake self-management. B 11. Follow-up and surveillance Serial clinical assessment: obtain regular clinical surveillance for emergent cardiomyopathy or overt HF during long-term follow-up in patients with AF, because HF may develop even in the presence of apparently adequate ventricular rate control. B Monitoring of treatment response: As per ESC 2021 guidelines, collect patient-reported outcomes routinely to measure treatment success and improve patient care. B As per NHFA 2018 guidelines, obtain regular monitoring of treatment adherence and persistence in all patients prescribed pharmacotherapy for the management of AF, including core rhythm control and anticoagulation therapies. Use accessible and patient-centered strategies to support treatment adherence and persistence. B 12. Quality improvement Quality of care: As per ESC 2021 guidelines, consider introducing tools to measure quality of care and identifying opportunities for improved treatment quality and patient outcome. C Show 2 more As per NHFA 2018 guidelines, ensure using eHealth tools and resources by patients and health professionals, to support the integrated management of AF. A References 1. Brieger D, Amerena J, Attia JR et al. National Heart Foundation of Australia and Cardiac Society of Australia and New Zealand: Australian clinical guidelines for the diagnosis and management of atrial fibrillation 2018. Med J Aust. 2018 Oct 15;209 8 356 362. Open https://web.pathway.md/diseases/recZrV7qZIxyavdK7 16/20 6/23/23, 3:15 AM Atrial fibrillation Pathway 2. Gerhard Hindricks, Tatjana Potpara, Nikolaos Dagres et al. 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation developed in collaboration with the European Association for Cardio- Thoracic Surgery EACTS The Task Force for the diagnosis and management of atrial fibrillation of the European Society of Cardiology ESC Developed with the special contribution of the European Heart Rhythm Association EHRA of the ESC. Eur Heart J. 2021 Feb 1;42 5 373 498. Open 3. January CT, Wann LS, Calkins H et al. 2019 AHA/ACC/HRS Focused Update of the 2014 AHA/ACC/HRS Guideline for the Management of Patients With Atrial Fibrillation: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2019 Jul 9;74 1 104 132. Open 4. Jason G Andrade, Atul Verma, L Brent Mitchell et al. 2018 Focused Update of the Canadian Cardiovascular Society Guidelines for the Management of Atrial Fibrillation. Can J Cardiol. 2018 Nov;34 11 1371 1392. Open 5. Badhwar V, Rankin JS, Damiano RJ Jr et al. The Society of Thoracic Surgeons 2017 Clinical Practice Guidelines for the Surgical Treatment of Atrial Fibrillation. Ann Thorac Surg. 2017 Jan;103 1 329 341. Open 6. Verma A, Cairns JA, Mitchell LB et al. 2014 focused update of the Canadian Cardiovascular Society Guidelines for the management of atrial fibrillation. Can J Cardiol. 2014 Oct;30 10 1114 30. Open 7. Jacqueline Saw, David R Holmes, Jo o L Cavalcante et al. SCAI/HRS expert consensus statement on transcatheter left atrial appendage closure. Heart Rhythm. 2023 Mar 20;S1547 5271 23 00011 5. Open 8. US Preventive Services Task Force, Curry SJ, Krist AH et al. Atrial Fibrillation: Screening With Electrocardiography. JAMA. 2018 Aug 7;320 5 478 484. Open 9. Zimetbaum P. Atrial Fibrillation. Ann Intern Med. 2017 Mar 7;166 5 ITC33 ITC48. Open
Show 2 more Patients with acute ischemic stroke: As per ESC 2021 guidelines: Initiate long-term oral anticoagulation (with a preference for direct oral coagulants over vitamin K antagonists in direct oral coagulant-eligible patients) for secondary prevention of stroke in patients with AF and an ischemic stroke or TIA, in the absence of strict contraindications to OACs. A Do not administer very early anticoagulation (< 48 hours) using UFH, LMWH, or vitamin K antagonists in patients with AF presenting with acute ischemic stroke. D As per NHFA 2018 guidelines: Do not initiate anticoagulants in the first few days after an ischemic stroke because of the risk of hemorrhage or hemorrhagic transformation of infarction. D Consider delaying the initiation of OACs for 2 weeks, but making the decision before discharge, in patients with ischemic stroke. C Patients with acute coronary syndromes: administer anticoagulation for patients with acute coronary syndrome and AF at increased risk of systemic thromboembolism (based on CHA2DS2- VASc risk score of 2), unless the bleeding risk exceeds the expected benefit. B Show 2 more Patients with valvular heart disease: As per ESC 2021 guidelines, do not use direct OACs in patients with AF and: https://web.pathway.md/diseases/recZrV7qZIxyavdK7 14/20 6/23/23, 3:15 AM Atrial fibrillation Pathway prosthetic mechanical valve D moderate-to-severe mitral stenosis. D As per AHA 2019 guidelines: Administer warfarin in patients with AF who have mechanical heart valves. B Avoid administering the direct thrombin inhibitor dabigatran in patients with AF and a mechanical heart valve. D Patients with coronary/arterial vascular disease: use a direct OAC in preference to warfarin when oral anticoagulation is indicated in the presence of coronary or arterial vascular disease. B Show 2 more Patients with congenital heart disease: consider initiating OACs in all adult patients with intracardiac repair, cyanosis, Fontan palliation, or systemic right ventricle and a history of AF. C Show 5 more Patients with chronic kidney disease: As per AHA 2019 guidelines, avoid administering the direct thrombin inhibitor dabigatran or the factor Xa inhibitors rivaroxaban or edoxaban in patients with AF and end-stage CKD or on dialysis, because of the lack of evidence from clinical trials that benefit exceeds risk. D Show 2 more As per NHFA 2018 guidelines: Individualize the decision to use anticoagulants in patients with AF and severe CKD because there are no prospective data showing benefit in this population. B Administer warfarin if a patient with AF with severe CKD requires anticoagulant therapy. B Pregnant patients (rate control): Administer selective -blockers for rate control in pregnant patients with AF. B Consider administering digoxin or verapamil for rate control in pregnant patients with AF if - blockers fail. C Pregnant patients (cardioversion): perform immediate electrical cardioversion in pregnant patients with AF and hemodynamic instability or pre-excited AF. B Show 2 more Pregnant patients (maintenance therapy): consider administering flecainide, propafenone, or sotalol for the prevention of AF if atrioventricular nodal-blocking drugs fail. C Pregnant patients (anticoagulation): administer therapeutic anticoagulation with heparin or vitamin K antagonist according to the stage of pregnancy in pregnant patients with AF. B 10. Patient education General counseling: As per ESC 2021 guidelines: To optimize shared decision-making about specific treatment options in consideration: https://web.pathway.md/diseases/recZrV7qZIxyavdK7 15/20 6/23/23, 3:15 AM Atrial fibrillation Pathway inform patients about the advantages/limitations and benefit/risks associated with treatment options discuss the potential burden of the treatment and include the patient's perception of treatment burden in the treatment decision. B Counsel patients and strongly emphasize about the importance of adherence and persistence to treatment with direct anticoagulants both before and after cardioversion. B As per NHFA 2018 guidelines: Provide targeted education throughout the continuum of management in patients with AF. A Take into consideration patients' beliefs, values, and preferences in the shared decision- making, with a goal of empowering patients to undertake self-management. B 11. Follow-up and surveillance Serial clinical assessment: obtain regular clinical surveillance for emergent cardiomyopathy or overt HF during long-term follow-up in patients with AF, because HF may develop even in the presence of apparently adequate ventricular rate control. B Monitoring of treatment response: As per ESC 2021 guidelines, collect patient-reported outcomes routinely to measure treatment success and improve patient care. B As per NHFA 2018 guidelines, obtain regular monitoring of treatment adherence and persistence in all patients prescribed pharmacotherapy for the management of AF, including core rhythm control and anticoagulation therapies. Use accessible and patient-centered strategies to support treatment adherence and persistence. B 12. Quality improvement Quality of care: As per ESC 2021 guidelines, consider introducing tools to measure quality of care and identifying opportunities for improved treatment quality and patient outcome. C Show 2 more As per NHFA 2018 guidelines, ensure using eHealth tools and resources by patients and health professionals, to support the integrated management of AF. A References 1. Brieger D, Amerena J, Attia JR et al. National Heart Foundation of Australia and Cardiac Society of Australia and New Zealand: Australian clinical guidelines for the diagnosis and management of atrial fibrillation 2018. Med J Aust. 2018 Oct 15;209 8 356 362. Open https://web.pathway.md/diseases/recZrV7qZIxyavdK7 16/20 6/23/23, 3:15 AM Atrial fibrillation Pathway 2. Gerhard Hindricks, Tatjana Potpara, Nikolaos Dagres et al. 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation developed in collaboration with the European Association for Cardio- Thoracic Surgery EACTS The Task Force for the diagnosis and management of atrial fibrillation of the European Society of Cardiology ESC Developed with the special contribution of the European Heart Rhythm Association EHRA of the ESC. Eur Heart J. 2021 Feb 1;42 5 373 498. Open 3. January CT, Wann LS, Calkins H et al. 2019 AHA/ACC/HRS Focused Update of the 2014 AHA/ACC/HRS Guideline for the Management of Patients With Atrial Fibrillation: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2019 Jul 9;74 1 104 132. Open 4. Jason G Andrade, Atul Verma, L Brent Mitchell et al. 2018 Focused Update of the Canadian Cardiovascular Society Guidelines for the Management of Atrial Fibrillation. Can J Cardiol. 2018 Nov;34 11 1371 1392. Open 5. Badhwar V, Rankin JS, Damiano RJ Jr et al. The Society of Thoracic Surgeons 2017 Clinical Practice Guidelines for the Surgical Treatment of Atrial Fibrillation. Ann Thorac Surg. 2017 Jan;103 1 329 341. Open 6. Verma A, Cairns JA, Mitchell LB et al. 2014 focused update of the Canadian Cardiovascular Society Guidelines for the management of atrial fibrillation. Can J Cardiol. 2014 Oct;30 10 1114 30. Open 7. Jacqueline Saw, David R Holmes, Jo o L Cavalcante et al. SCAI/HRS expert consensus statement on transcatheter left atrial appendage closure. Heart Rhythm. 2023 Mar 20;S1547 5271 23 00011 5. Open 8. US Preventive Services Task Force, Curry SJ, Krist AH et al. Atrial Fibrillation: Screening With Electrocardiography. JAMA. 2018 Aug 7;320 5 478 484. Open 9. Zimetbaum P. Atrial Fibrillation. Ann Intern Med. 2017 Mar 7;166 5 ITC33 ITC48. Open 10. Morillo CA, Banerjee A, Perel P et al. Atrial fibrillation: the current epidemic. J Geriatr Cardiol. 2017 Mar;14 3 195 203. Open 11. Staerk L, Sherer JA, Ko D et al. Atrial Fibrillation: Epidemiology, Pathophysiology, and Clinical Outcomes. Circ Res. 2017 Apr 28;120 9 1501 1517. Open 12. Xu J, Luc JG, Phan K. Atrial fibrillation: review of current treatment strategies. J Thorac Dis. 2016 Sep;8 9 E886 E900. Open 13. Culebras A, Mess SR, Chaturvedi S et al. Summary of evidence-based guideline update: prevention of stroke in nonvalvular atrial fibrillation: report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology. 2014 Feb 25;82 8 716 24. Open 14. Halvorsen S, Storey RF, Rocca B et al. Management of antithrombotic therapy after bleeding in patients with coronary artery disease and/or atrial fibrillation: expert consensus paper of the European Society of Cardiology Working Group on Thrombosis. Eur Heart J. 2017 May 14;38 19 1455 1462. Open 15. Steffel J, Verhamme P, Potpara TS et al. The 2018 European Heart Rhythm Association Practical Guide on the use of non-vitamin K antagonist oral anticoagulants in patients with atrial fibrillation: executive summary. Europace. 2018 Aug 1;20 8 1231 1242. Open 16. Heart Rhythm Society. Choosing Wisely HRS recommendations. Choosing Wisely. 2014. Open https://web.pathway.md/diseases/recZrV7qZIxyavdK7 17/20 6/23/23, 3:15 AM Atrial fibrillation Pathway 17. Paulus Kirchhof, A John Camm, Andreas Goette et al. Early Rhythm-Control Therapy in Patients with Atrial Fibrillation. N Engl J Med. 2020 Oct 1;383 14 1305 1316. Open 18. James D Douketis, Alex C Spyropoulos, Joanne Duncan et al. Perioperative Management of Patients With Atrial Fibrillation Receiving a Direct Oral Anticoagulant. JAMA Intern Med. 2019 Aug 5;179 11 1469 1478. Open 19. Christine Parsons, MD, Salma I. Patel et al. The CHA2DS2 VASc Score A Predictor of Thromboembolic Events and Mortality In Patients With an Implantable Monitoring Device Without Atrial Fibrillation. Mayo Clin Proc. 2017 Mar; 92 3 360 369. Open 20. Emily C O'Brien, DaJuanicia N Simon, Laine E Thomas et al. The ORBIT bleeding score: a simple bedside score to assess bleeding risk in atrial fibrillation. Eur Heart J. 2015 Dec 7;36 46 3258 64. Open 21. Wengen Zhu, Wenfeng He, Linjuan Guo et al. The HAS BLED Score for Predicting Major Bleeding Risk in Anticoagulated Patients With Atrial Fibrillation: A Systematic Review and Meta-analysis. Clin Cardiol. 2015 Sep;38 9 555 61. Open 22. Margaret C Fang, Alan S Go, Yuchiao Chang et al. A new risk scheme to predict warfarin-associated hemorrhage: The ATRIA Anticoagulation and Risk Factors in Atrial Fibrillation) Study. J Am Coll Cardiol. 2011 Jul 19;58 4 395 401. Open 23. Gareth J Wynn, Derick M Todd, Matthew Webber et al. The European Heart Rhythm Association symptom classification for atrial fibrillation: validation and improvement through a simple modification. Europace. 2014 Jul;16 7 965 72. Open 24. Alec Vahanian, Friedhelm Beyersdorf, Fabien Praz et al. 2021 ESC/EACTS Guidelines for the management of valvular heart disease: Developed by the Task Force for the management of valvular heart disease of the European Society of Cardiology ESC and the European Association for Cardio-Thoracic Surgery EACTS . Eur Heart J. 2021;ehab395. Open 25. Gregory Y H Lip, Robby Nieuwlaat, Ron Pisters et al. Refining clinical risk stratification for predicting stroke and thromboembolism in atrial fibrillation using a novel risk factor-based approach: the euro heart survey on atrial fibrillation. Chest. 2010 Feb;137 2 263 72. Open 26. Wahid Bouida, Kaouthar Beltaief, Mohamed Amine Msolli et al. Low-dose Magnesium Sulfate Versus High Dose in the Early Management of Rapid Atrial Fibrillation: Randomized Controlled Double-blind Study LOMAGHI Study). Acad Emerg Med. 2019 Feb;26 2 183 191. Open 27. Jason G Andrade, Martin Aguilar, Clare Atzema et al. The 2020 Canadian Cardiovascular Society/Canadian Heart Rhythm Society Comprehensive Guidelines for the Management of Atrial Fibrillation. Can J Cardiol. 2020 Dec;36 12 1847 1948. Open 28. Helio P Guimar es, Renato D Lopes, Pedro G M de Barros E Silva et al. Rivaroxaban in Patients with Atrial Fibrillation and a Bioprosthetic Mitral Valve. N Engl J Med. 2020 Nov 26;383 22 2117 2126. Open 29. Masaru Kuroda, Eiji Tamiya, Takahisa Nose et al. Effect of 15-mg Edoxaban on Clinical Outcomes in 3 Age Strata in Older Patients With Atrial Fibrillation: A Prespecified Subanalysis of the ELDERCARE AF Randomized Clinical Trial. JAMA Cardiol. 2022 Jun 1;7 6 583 590. Open 30. Craig T January, L Samuel Wann, Joseph S Alpert et al. 2014 AHA/ACC/HRS guideline for the management of patients with atrial fibrillation: executive summary: a report of the American College of https://web.pathway.md/diseases/recZrV7qZIxyavdK7 18/20 6/23/23, 3:15 AM Atrial fibrillation Pathway Cardiology/American Heart Association Task Force on practice guidelines and the Heart Rhythm Society. Circulation. 2014 Dec 2;130 23 2071 104. Open 31. Nassir F Marrouche, Oussama Wazni, Christopher McGann et al. Effect of MRI Guided Fibrosis Ablation vs Conventional Catheter Ablation on Atrial Arrhythmia Recurrence in Patients With Persistent Atrial Fibrillation: The DECAAF II Randomized Clinical Trial. JAMA. 2022 Jun 21;327 23 2296 2305. Open 32. Stuart J Connolly, Ganesan Karthikeyan, Mpiko Ntsekhe et al. Rivaroxaban in Rheumatic Heart Disease- Associated Atrial Fibrillation. N Engl J Med. 2022 Sep 15;387 11 978 988. Open 33. William J Groh, Deepak Bhakta, Gordon F Tomaselli et al. 2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders. Heart Rhythm. 2022 Oct;19 10):e61-e120. Open 34. Michael J Ackerman, Silvia G Priori, Stephan Willems et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society HRS and the European Heart Rhythm Association EHRA . Heart Rhythm. 2011 Aug;8 8 1308 39. Open 35. C Michael Gibson, Roxana Mehran, Christoph Bode et al. Prevention of Bleeding in Patients with Atrial Fibrillation Undergoing PCI. N Engl J Med. 2016 Dec 22;375 25 2423 2434. Open 36. Christopher P Cannon, Deepak L Bhatt, Jonas Oldgren et al. Dual Antithrombotic Therapy with Dabigatran after PCI in Atrial Fibrillation. N Engl J Med. 2017 Oct 19;377 16 1513 1524. Open 37. Renato D Lopes, Gretchen Heizer, Ronald Aronson et al. Antithrombotic Therapy after Acute Coronary Syndrome or PCI in Atrial Fibrillation. N Engl J Med. 2019 Apr 18;380 16 1509 1524. Open 38. Pascal Vranckx, Marco Valgimigli, Lars Eckardt et al. Edoxaban-based versus vitamin K antagonist- based antithrombotic regimen after successful coronary stenting in patients with atrial fibrillation ENTRUST AF PCI a randomised, open-label, phase 3b trial. Lancet. 2019 Oct 12;394 10206 1335 1343. Open 39. Gregory Y H Lip, Amitava Banerjee, Giuseppe Boriani et al. Antithrombotic Therapy for Atrial Fibrillation: CHEST Guideline and Expert Panel Report. Chest. 2018 Nov;154 5 1121 1201. Open 40. John U Doherty, Ty J Gluckman, William J Hucker et al. 2017 ACC Expert Consensus Decision Pathway for Periprocedural Management of Anticoagulation in Patients With Nonvalvular Atrial Fibrillation: A Report of the American College of Cardiology Clinical Expert Consensus Document Task Force. J Am Coll Cardiol. 2017 Feb 21;69 7 871 898. Open 41. Catharina Jm Klijn, Maurizio Paciaroni, Eivind Berge et al. Antithrombotic treatment for secondary prevention of stroke and other thromboembolic events in patients with stroke or transient ischemic attack and non-valvular atrial fibrillation: A European Stroke Organisation guideline. Eur Stroke J. 2019 Sep;4 3 198 223. Open 42. Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J. 2018 Sep 7;39 34 3165 3241. Open 43. Girish M Nair, David H Birnie, Pablo B Nery et al. Standard vs Augmented Ablation of Paroxysmal Atrial Fibrillation for Reduction of Atrial Fibrillation Recurrence: The AWARE Randomized Clinical Trial. JAMA Cardiol. 2023 May 1;8 5 475 483. Open 44. Satoshi Ogawa, Takeshi Yamashita, Tsutomu Yamazaki et al. Optimal treatment strategy for patients with paroxysmal atrial fibrillation: J RHYTHM Study. Circ J. 2009 Feb;73 2 242 8. Open https://web.pathway.md/diseases/recZrV7qZIxyavdK7 19/20 6/23/23, 3:15 AM Atrial fibrillation Pathway https://web.pathway.md/diseases/recZrV7qZIxyavdK7 20/20
Guideline sources The following summarized guidelines for the evaluation and management of atrial flutter are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC/EACTS/EHRA 2021), the European Society of Cardiology (ESC 2020; 2018), the Canadian Cardiovascular Society (CCS/CHRS 2020), the American Heart Association (AHA/HRS/ACC 2019; 2016; 2014), the Canadian Cardiovascular Society (CCS 2018; 2014), and the Latin American Heart Rhythm Society (SOLAECE/ECAS/APHRS/EHRA/HRS 2017). 1 2 3 4 5 6 7 8 9 10 Guidelines 1. Classification and risk stratification Stroke risk assessment: stratify all patients with atrial flutter, whether paroxysmal or persistent, using a predictive index for stroke risk. A 2. Medical management Acute rhythm control: https://web.pathway.md/diseases/recCej7N2vg1HZE82 1/6 6/23/23, 3:15 AM Atrial flutter Pathway As per ESC 2020 guidelines: Administer IV ibutilide or IV or oral (in-hospital) dofetilide for pharmacological cardioversion in hemodynamically stable patients with macroreentrant atrial arrhythmia. B Do not use propafenone or flecainide for pharmacological cardioversion in hemodynamically stable patients with macroreentrant atrial arrhythmia. D As per AHA 2016 guidelines, administer oral dofetilide or IV ibutilide for acute pharmacological cardioversion in stable patients with atrial flutter. A Show 2 more As per ACC 2014 guidelines: Administer flecainide, dofetilide, propafenone, or IV ibutilide for cardioversion in patients with atrial flutter, in the absence of contraindications. A Do not initiate dofetilide out of hospital. D Acute rate control: As per ESC 2020 guidelines: Consider administering IV -blockers or nondihydropyridine CCBs (verapamil or diltiazem) for control of rapid ventricular rate in hemodynamically stable patients with macroreentrant atrial arrhythmia. C Consider administering IV amiodarone if the above agents are not available or desirable. C As per AHA 2016 guidelines: Administer IV or oral -blockers, diltiazem, or verapamil for acute rate control in hemodynamically stable patients with atrial flutter. B Consider administering IV amiodarone for acute rate control (in the absence of pre-excitation) in patients with atrial flutter and systolic HF, if -blockers are contraindicated or ineffective. C Chronic rhythm control: As per ESC 2020 guidelines: Consider initiating -blockers or nondihydropyridine CCBs (verapamil or diltiazem, in the absence of HFrEF) if ablation is not desirable or feasible. Consider initiating -blockers for the management of patients with recurrent atrial flutter if ablation is not possible or successful. C Consider initiating amiodarone to maintain sinus rhythm if the above agents and/or catheter ablation fail. C As per AHA 2016 guidelines, consider using the following medications for maintaining sinus rhythm in symptomatic patients with recurrent atrial flutter, with the medication choice depending on underlying heart disease and comorbidities: amiodarone dofetilide sotalol. C Show 2 more Chronic rate control: initiate -blockers, diltiazem, or verapamil to control the ventricular rate in patients with hemodynamically tolerated atrial flutter. B https://web.pathway.md/diseases/recCej7N2vg1HZE82 2/6 6/23/23, 3:15 AM Atrial flutter Pathway Antithrombotic therapy: As per ESC 2020 guidelines: Consider initiating anticoagulation in patients with atrial flutter without AF, but the threshold for initiation has not been established. C Initiate anticoagulation in patients with atrial flutter and concomitant AF as for patients with AF. B As per AHA 2019 guidelines, initiate anticoagulant therapy in patients with atrial flutter according to the same risk profile used for AF. B As per CCS 2018 guidelines, avoid initiating antithrombotic therapy for stroke prevention in < 65 years old patients with atrial flutter with no CHADS2 risk factors. D As per AHA 2016 guidelines, initiate acute antithrombotic therapy in patients with atrial flutter to align with recommended antithrombotic therapy for patients with AF. B Show 2 more 3. Therapeutic procedures Electrical cardioversion: As per ESC 2020 guidelines: Perform synchronized direct cuttent cardioversion in hemodynamically unstable patients with macroreentrant atrial arrhythmia. B Perform low-energy (100 J biphasic) electrical cardioversion in hemodynamically stable patients with macroreentrant atrial arrhythmia. B As per CCS 2018 guidelines, consider performing immediate electrical cardioversion if the recent-onset atrial flutter is causing hemodynamic instability with hypotension, acute coronary syndrome, or pulmonary edema. B As per AHA 2016 guidelines, perform synchronized cardioversion for acute management of hemodynamically unstable patients with atrial flutter not responding to pharmacological therapies. B Show 2 more As per ACC 2014 guidelines, perform cardioversion to restore sinus rhythm in patients with atrial flutter. Consider repeating attempts if unsuccessful. B Show 2 more Atrial pacing: As per ESC 2020 guidelines: Perform high-rate atrial pacing for termination of atrial flutter in the presence of an implanted pacemaker or defibrillator in hemodynamically stable patients. B Consider performing invasive or noninvasive high-rate atrial pacing for termination of atrial flutter in hemodynamically stable patients. C As per AHA 2016 guidelines: https://web.pathway.md/diseases/recCej7N2vg1HZE82 3/6 6/23/23, 3:15 AM Atrial flutter Pathway Perform rapid atrial pacing for acute conversion of atrial flutter in patients having pacing wires in place as part of a permanent pacemaker or implantable cardioverter-defibrillator or for temporary atrial pacing after cardiac surgery. B Consider performing atrial pacing to decrease recurrences of atrial flutter in adult patients with congenital heart disease and SND. C Catheter ablation: As per CCS 2020 guidelines, perform catheter ablation of typical RA flutter as a reasonable alternative to pharmacologic rhythm or rate control therapy. B As per HRS 2017 guidelines: Perform a linear ablation of the cavotricuspid isthmus in patients with a history of typical atrial flutter, or if typical atrial flutter is induced at the time of AF ablation. B Insufficient evidence to recommend linear ablation in the absence of macroreentrant atrial flutter. I As per AHA 2016 guidelines, perform catheter ablation of the cavotricuspid isthmus in patients with atrial flutter that is either symptomatic or refractory to pharmacological rate control. B Show 5 more Catheter ablation (ECS): consider performing catheter ablation after the first episode of symptomatic typical atrial flutter. C Show 3 more AV nodal ablation: consider performing atrioventricular nodal ablation with subsequent pacing ("ablate and pace"), either biventricular or His-bundle pacing, if catheter ablation and pharmacological rhythm control fail and the patient has symptomatic persistent macroreentrant atrial arrhythmia with fast ventricular rates. C 4. Perioperative care Pericardioversion thromboprophylaxis: As per ESC 2021 guidelines, administer effective anticoagulation for a minimum of 3 weeks before cardioversion of atrial flutter. B Show 3 more As per AHA 2019 guidelines, consider administering heparin, a factor Xa inhibitor, or a direct thrombin inhibitor in patients with atrial flutter of < 48 hours duration with a CHA2DS2-VASc score of 0 in males and 1 in females before cardioversion, without the need for post- cardioversion oral anticoagulation. C Show 4 more As per CCS 2018 guidelines, initiate therapeutic anticoagulation, in addition to appropriate rate control, for 3 weeks before cardioversion in most hemodynamically stable patients with atrial flutter planned to undergo electrical or pharmacological cardioversion. B Show 4 more https://web.pathway.md/diseases/recCej7N2vg1HZE82 4/6 6/23/23, 3:15 AM Atrial flutter Pathway 5. Surgical interventions Surgical ablation: consider performing surgical ablation of atrial flutter in adult patients with congenital heart disease undergoing planned surgical repair. C 6. Specific circumstances Patients with congenital heart disease: As per ESC 2021 guidelines, consider initiating OACs in all adult patients with intracardiac repair, cyanosis, Fontan palliation, or systemic right ventricle and a history of atrial flutter. C As per ACC 2016 guidelines, initiate acute antithrombotic therapy in patients with atrial flutter and adult congenital heart disease patients to align with recommended antithrombotic therapy for patients with AF. B Pregnant patients: As per ESC 2020 guidelines, consider administering IV ibutilide for termination of atrial flutter in pregnant patients. C As per ESC 2018 guidelines, consider administering ibutilide or flecainide for termination of atrial flutter in stable pregnant patients with structurally normal hearts. C 7. Follow-up and surveillance Follow-up: monitor patients after atrial flutter ablation for the occurrence of AF with opportunistic screening conducted at the time of medical encounters. B References 1. Richard L Page, Jos A Joglar, Mary A Caldwell et al. 2015 ACC/AHA/HRS Guideline for the Management of Adult Patients With Supraventricular Tachycardia: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2016 Apr 5;133 14):e471 505. Open 2. Josep Brugada, Demosthenes G Katritsis, Elena Arbelo et al. 2019 ESC Guidelines for the management of patients with supraventricular tachycardia The Task Force for the management of patients with supraventricular tachycardia of the European Society of Cardiology ESC . Eur Heart J. 2020 Feb 1;41 5 655 720. Open 3. Jason G Andrade, Atul Verma, L Brent Mitchell et al. 2018 Focused Update of the Canadian Cardiovascular Society Guidelines for the Management of Atrial Fibrillation. Can J Cardiol. 2018 Nov;34 11 1371 1392. Open 4. Craig T January, L Samuel Wann, Joseph S Alpert et al. 2014 AHA/ACC/HRS guideline for the management of patients with atrial fibrillation: executive summary: a report of the American College of https://web.pathway.md/diseases/recCej7N2vg1HZE82 5/6 6/23/23, 3:15 AM Atrial flutter Pathway Cardiology/American Heart Association Task Force on practice guidelines and the Heart Rhythm Society. Circulation. 2014 Dec 2;130 23 2071 104. Open 5. Verma A, Cairns JA, Mitchell LB et al. 2014 focused update of the Canadian Cardiovascular Society Guidelines for the management of atrial fibrillation. Can J Cardiol. 2014 Oct;30 10 1114 30. Open 6. Calkins H, Hindricks G, Cappato R et al. 2017 HRS/EHRA/ECAS/APHRS/SOLAECE expert consensus statement on catheter and surgical ablation of atrial fibrillation: Executive summary. J Arrhythm. 2017 Oct;33 5 369 409. Open 7. January CT, Wann LS, Calkins H et al. 2019 AHA/ACC/HRS Focused Update of the 2014 AHA/ACC/HRS Guideline for the Management of Patients With Atrial Fibrillation: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2019 Jul 9;74 1 104 132. Open 8. Jason G Andrade, Martin Aguilar, Clare Atzema et al. The 2020 Canadian Cardiovascular Society/Canadian Heart Rhythm Society Comprehensive Guidelines for the Management of Atrial Fibrillation. Can J Cardiol. 2020 Dec;36 12 1847 1948. Open 9. Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J. 2018 Sep 7;39 34 3165 3241. Open 10. Gerhard Hindricks, Tatjana Potpara, Nikolaos Dagres et al. 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation developed in collaboration with the European Association for Cardio- Thoracic Surgery EACTS The Task Force for the diagnosis and management of atrial fibrillation of the European Society of Cardiology ESC Developed with the special contribution of the European Heart Rhythm Association EHRA of the ESC. Eur Heart J. 2021 Feb 1;42 5 373 498. Open 11. Shah SR, Luu SW, Calestino M et al. Management of atrial fibrillation-flutter: uptodate guideline paper on the current evidence. J Community Hosp Intern Med Perspect. 2018 Oct 15;8 5 269 275. Open 12. Tobe SW, Stone JA, Anderson T et al. Canadian Cardiovascular Harmonized National Guidelines Endeavour C CHANGE guideline for the prevention and management of cardiovascular disease in primary care: 2018 update. CMAJ. 2018 Oct 9;190 40 E1192 E1206. Open 13. Elliott PM, Anastasakis A, Borger MA et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology ESC . Eur Heart J. 2014 Oct 14;35 39 2733 79. Open 14. William J Groh, Deepak Bhakta, Gordon F Tomaselli et al. 2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders. Heart Rhythm. 2022 Oct;19 10):e61-e120. Open https://web.pathway.md/diseases/recCej7N2vg1HZE82 6/6
Guideline sources The following summarized guidelines for the management of atrioventricular nodal reentrant tachycardia are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC 2020) and the American Heart Association (AHA/HRS/ACC 2016). 1 2 Calculator NYHA functional classification f Guidelines 1. Medical management Acute management: Administer IV adenosine (6-18 mg bolus) for acute management of hemodynamically stable patients with AVNRT if vagal maneuvers fail. B Consider administering IV formulations of the following medications for acute management of hemodynamically stable patients with AVNRT if vagal maneuvers and adenosine fail: nondihydropyridine CCBs, i.e. verapamil or diltiazem https://web.pathway.md/diseases/recAWt2YZMRL8w8HW 1/3 6/23/23, 3:15 AM Atrioventricular nodal reentrant tachycardia Pathway -blockers, i.e. esmolol or metoprolol. C Administer adenosine for acute management of patients with AVNRT. B Show 2 more Ongoing management: Consider initiating the following medications for the management of patients with AVNRT if ablation is not desirable or feasible: nondihydropyridine CCBs (verapamil or diltiazem) in the absence of HFrEF -blockers. C Consider offering abstinence from therapy in patients with minimally symptomatic patients with very infrequent, short-lived episodes of AVNRT. C Initiate oral formulations of the following medications for ongoing management of patients with AVNRT not being candidates for, or preferring not to undergo, catheter ablation: verapamil diltiazem -blockers. B Show 3 more 2. Nonpharmacologic interventions Vagal maneuvers: Perform vagal maneuvers, preferably in the supine position with leg elevation, for acute management of hemodynamically stable patients with AVNRT. B Perform vagal maneuvers for acute management of patients with AVNRT. B 3. Therapeutic procedures Synchronized electrical cardioversion: Perform synchronized direct current cardioversion for acute management of hemodynamically unstable patients with AVNRT. B Perform synchronized direct current cardioversion for acute management of hemodynamically stable patients with AVNRT if drug therapy fails to convert or control the tachycardia. B Perform synchronized cardioversion for acute management of hemodynamically unstable patients with AVNRT if adenosine and vagal maneuvers do not terminate the tachycardia or are not feasible. B Perform synchronized cardioversion for acute management of hemodynamically stable patients with AVNRT if pharmacological therapy does not terminate the tachycardia or is contraindicated. B Catheter ablation: https://web.pathway.md/diseases/recAWt2YZMRL8w8HW 2/3 6/23/23, 3:15 AM Atrioventricular nodal reentrant tachycardia Pathway Perform catheter ablation for the management of patients with is recommended for symptomatic recurrent AVNRT. B Perform catheter ablation of the slow pathway in patients with AVNRT. B 4. Follow-up and surveillance Follow-up: consider obtaining clinical follow-up without pharmacological therapy or ablation for ongoing management of minimally symptomatic patients with AVNRT. C References 1. Josep Brugada, Demosthenes G Katritsis, Elena Arbelo et al. 2019 ESC Guidelines for the management of patients with supraventricular tachycardia The Task Force for the management of patients with supraventricular tachycardia of the European Society of Cardiology ESC . Eur Heart J. 2020 Feb 1;41 5 655 720. Open 2. Richard L Page, Jos A Joglar, Mary A Caldwell et al. 2015 ACC/AHA/HRS Guideline for the Management of Adult Patients With Supraventricular Tachycardia: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2016 Apr 5;133 14):e471 505. Open https://web.pathway.md/diseases/recAWt2YZMRL8w8HW 3/3
Guideline sources The following summarized guidelines for the evaluation and management of atrophic gastritis are prepared by our editorial team based on guidelines from the Korean College of Helicobacter and Upper Gastrointestinal Research (KCHUGR 2023), the Italian Society of Digestive Endoscopy (SIDE/SIGE 2022), the American Gastroenterological Association (AGA 2021; 2020), the European Society of Gastrointestinal Endoscopy (ESGE/ESP/SPED/EHMSG 2019), the British Society of Gastroenterology (BSG 2019), and the American Society for Gastrointestinal Endoscopy (ASGE 2015). 1 2 3 4 5 6 7 8 Guidelines 1. Screening and diagnosis Diagnosis: As per AGA 2021 guidelines, recognize that atrophic gastritis is defined as the loss of gastric glands, with or without metaplasia, in the setting of chronic inflammation mainly due to H. pylori infection or autoimmunity. B https://web.pathway.md/diseases/rec75dvWQq2gKXK7H 1/6 6/23/23, 3:16 AM Atrophic gastritis Pathway Show 2 more As per ESGE 2019 guidelines, confirm intestinal metaplasia histologically as the most reliable marker of atrophy in gastric mucosa. A 2. Classification and risk stratification Risk of malignancy: recognize that patients with chronic atrophic gastritis or intestinal metaplasia are at risk for gastric adenocarcinoma, A and patients with advanced stages of gastritis with atrophy and/or intestinal metaplasia affecting both antral and corpus mucosa are at higher risk for gastric adenocarcinoma. B 3. Diagnostic investigations Antibody testing: consider obtaining anti-parietal cell antibodies and anti-intrinsic factor antibodies to assist in the diagnosis of patients with histology compatible with autoimmune gastritis. C Serum pepsinogen levels: assess serum pepsinogen I level and serum pepsinogen I/II ratio to identify patients with advanced stages of atrophic gastritis. B Helicobacter pylori testing: As per AGA 2021 guidelines, obtain testing for H. pylori infection in all patients with atrophic gastritis. B As per AGA 2020 guidelines, obtain testing for H. pylori followed by eradication in patients with gastric intestinal metaplasia. B Evaluation of anemia: As per AGA 2021 guidelines, obtain evaluation for anemia due to vitamin B12 and iron deficiencies in patients with histology compatible with autoimmune gastritis. B As per AGA 2020 guidelines, obtain evaluation for iron and vitamin B12 deficiencies in patients with atrophic gastritis irrespective of etiology, especially if corpus-predominant. B Screening for autoimmune thyroid disease: obtain screening for autoimmune thyroid disease in patients with autoimmune gastritis. B 4. Diagnostic procedures Diagnostic endoscopy: As per KCHUGR 2023 guidelines: Consider performing image-enhanced endoscopy in addition to white light endoscopy to increase the diagnostic accuracy of intestinal metaplasia. C Do not rely on white light endoscopy findings to determine whether to perform a biopsy when considering a biopsy to confirm atrophic gastritis or intestinal metaplasia. D https://web.pathway.md/diseases/rec75dvWQq2gKXK7H 2/6 6/23/23, 3:16 AM Atrophic gastritis Pathway As per AGA 2021 guidelines: Providers should recognize typical endoscopic features of atrophic gastritis, which include pale appearance of gastric mucosa, increased visibility of vasculature due to thinning of the gastric mucosa, and loss of gastric folds, and, if with concomitant intestinal metaplasia, light blue crests and white opaque fields. Because these mucosal changes are often subtle, techniques to optimize evaluation of the gastric mucosa should be performed. B When endoscopic features of atrophic gastritis are present, providers should assess the extent endoscopically. Providers should obtain biopsies from the suspected atrophic/metaplastic areas for histopathological confirmation and risk stratification; at a minimum, biopsies from the body and antrum/incisura should be obtained and placed in separately labeled jars. Targeted biopsies should additionally be obtained from any other mucosal abnormalities. B As per BSG 2019 guidelines, perform a full systematic endoscopy (examination time of a minimum of 7 minutes) of the stomach with clear photographic documentation of gastric regions and pathology in patients at higher risk for gastric adenocarcinoma, including atrophic gastritis and gastric intestinal metaplasia. B Show 6 more As per ESGE 2019 guidelines, perform endoscopy in patients with advanced stages of atrophic gastritis, particularly if H. pylori serology is negative. B Show 2 more Biopsy, general indications: As per AGA 2021 guidelines, perform biopsies of the suspected atrophic/metaplastic areas for histopathological confirmation and risk stratification. Take biopsies from the body and antrum/incisura and place them in separately labeled jars. Perform additional targeted biopsies for any other mucosal abnormalities. B As per SPED/ESP/EHMSG/ESGE 2019 guidelines: Perform gastric biopsies both for H. pylori infection diagnosis and identification of advanced stages of atrophic gastritis during the first diagnostic upper gastrointestinal endoscopy, to adequately stage gastric precancerous conditions. B Take biopsies from at least 2 topographic sites (from both the antrum and the corpus, at the lesser and greater curvature of each) and label them clearly in 2 separate vials. Perform additional biopsies of visible neoplastic suspicious lesions. B Biopsy, patients with anemia: As per AGA 2020 guidelines, do not perform a routine gastric biopsy to diagnose atrophic gastritis in patients with iron deficiency anemia. D As per BSG 2019 guidelines, consider performing a baseline endoscopy with biopsies in 50 years old patients with laboratory evidence of pernicious anemia, defined by vitamin B12 deficiency and either positive anti-parietal cell or anti-intrinsic factor antibodies. Take biopsies from the greater and lesser curves since atrophic gastritis affects the corpus in pernicious anemia. C https://web.pathway.md/diseases/rec75dvWQq2gKXK7H 3/6 6/23/23, 3:16 AM Atrophic gastritis Pathway Biopsy (patients with polyps): consider performing systematic sampling of the surrounding non- polypoid gastric mucosa in the setting of multiple hyperplastic or adenomatous polyps, to assess for H. pylori and metaplastic atrophic gastritis. C Histopathology: As per SIGE 2022 guidelines, obtain immunohistochemical analysis in patients with chronic/atrophic gastritis. B As per ESGE 2019 guidelines, consider using systems for histopathological staging (such as the OLGA and the OLGIM assessment) to identify patients with advanced stages of atrophic gastritis. Consider performing an additional biopsy of the incisura if these systems are used to stratify patients. C 5. Medical management Helicobacter pylori eradication: As per KCHUGR 2023 guidelines, consider initiating H. pylori eradication therapy to improve atrophic gastritis in patients with Helicobacter-positive atrophic gastritis. C As per AGA 2021 guidelines, initiate H. pylori eradication therapy in patients with atrophic gastritis with H. pylori infection and confirm eradication with non-serological testing modalities. B As per BSG 2019 guidelines, initiate H. pylori eradication therapy to reduce the risk of gastric adenocarcinoma development in patients with atrophic gastritis. A As per ESGE 2019 guidelines: Initiate H. pylori eradication therapy to regress atrophic gastritis and to reduce the risk of gastric cancer in patients with atrophic gastritis. A Consider initiating H. pylori eradication therapy to reduce inflammation and atrophy in patients with established intestinal metaplasia. C 6. Specific circumstances Patients with pernicious anemia: Obtain an appropriate diagnostic evaluation for atrophic gastritis in patients with unexplained iron or vitamin B12 deficiency. B Perform endoscopy with topographical biopsies to confirm corpus-predominant atrophic gastritis for risk stratification and to rule out prevalent gastric neoplasia (including NETs) in patients with a new diagnosis of pernicious anemia. B 7. Preventative measures Prevention of progression: https://web.pathway.md/diseases/rec75dvWQq2gKXK7H 4/6 6/23/23, 3:16 AM Atrophic gastritis Pathway Do not use NSAIDs or COX-2 inhibitors to reduce the risk of progression of premalignant lesions of the stomach. D Do not use antioxidants to reduce the prevalence of premalignant gastric lesions. D 8. Follow-up and surveillance Endoscopic surveillance: As per AGA 2021 guidelines, decide on the optimal endoscopic surveillance interval in patients with atrophic gastritis or autoimmune gastritis based on individual risk assessment and shared decision-making. B Show 2 more As per AGA 2020 guidelines: Avoid obtaining routine endoscopic surveillance in patients with gastric intestinal metaplasia. D Avoid performing a routine short-interval repeat endoscopy for risk stratification of patients with gastric intestinal metaplasia. D As per BSG 2019 guidelines: Obtain endoscopic surveillance every 3 years in patients with extensive atrophic gastritis or gastric intestinal metaplasia, defined as affecting the antrum and body. B Do not obtain surveillance in patients with atrophic gastritis or gastric intestinal metaplasia limited to the gastric antrum unless there are additional risk factors, such as a strong family history of gastric cancer or persistent H. pylori infection. Consider obtaining 3-yearly surveillance in these patients. D As per ESGE 2019 guidelines, insufficient evidence to recommend surveillance in patients with mild-to-moderate atrophy restricted to the antrum. I Show 5 more References 1. Pedro Pimentel-Nunes, Diogo Lib nio, Ricardo Marcos-Pinto et al. Management of epithelial precancerous conditions and lesions in the stomach MAPS II European Society of Gastrointestinal Endoscopy ESGE , European Helicobacter and Microbiota Study Group EHMSG , European Society of Pathology ESP , and Sociedade Portuguesa de Endoscopia Digestiva SPED guideline update 2019. Endoscopy. 2019 Apr;51 4 365 388. Open 2. Shailja C Shah, M Blanca Piazuelo, Ernst J Kuipers et al. AGA Clinical Practice Update on the Diagnosis and Management of Atrophic Gastritis: Expert Review. Gastroenterology. 2021 Oct;161 4 1325 1332.e7. Open 3. Seung Joo Kang, Jae Gyu Kim, Hee Seok Moon et al. Clinical Practice Guideline for Gastritis in Korea. J Korean Med Sci. 2023 Apr 3;38 13):e115. Open https://web.pathway.md/diseases/rec75dvWQq2gKXK7H 5/6 6/23/23, 3:16 AM Atrophic gastritis Pathway 4. Banks M, Graham D, Jansen M et al. British Society of Gastroenterology guidelines on the diagnosis and management of patients at risk of gastric adenocarcinoma. Gut. 2019 Sep;68 9 1545 1575. Open 5. Samir Gupta, Dan Li, Hashem B El Serag et al. AGA Clinical Practice Guidelines on Management of Gastric Intestinal Metaplasia. Gastroenterology. 2020 Feb;158 3 693 702. Open 6. ASGE Standards of Practice Committee, John A Evans, Vinay Chandrasekhara et al. The role of endoscopy in the management of premalignant and malignant conditions of the stomach. Gastrointest Endosc. 2015 Jul;82 1 1 8. Open 7. Marco Romano, Antonietta Gerarda Gravina, Leonardo Henry Eusebi et al. Management of Helicobacter pylori infection: Guidelines of the Italian Society of Gastroenterology SIGE and the Italian Society of Digestive Endoscopy SIED . Dig Liver Dis. 2022 Sep;54 9 1153 1161. Open 8. Cynthia W Ko, Shazia M Siddique, Amit Patel et al. AGA Clinical Practice Guidelines on the Gastrointestinal Evaluation of Iron Deficiency Anemia. Gastroenterology. 2020 Sep;159 3 1085 1094. Open https://web.pathway.md/diseases/rec75dvWQq2gKXK7H 6/6
Guideline sources The following summarized guidelines for the evaluation and management of attention deficit and hyperactivity disorder are prepared by our editorial team based on guidelines from the American Academy of Pediatrics (AAP 2019), the American Academy of Neurology (AAN 2016), and the American Association of Family Physicians (AAFP 2012). 1 2 4 5 5 5 6 Definition ADHD is a common psychiatric disorder characterized by pervasive and impairing symptoms of inattention, hyperactivity, and impulsivity according to DSM-V. 5 Epidemiology ADHD is caused by biological (genetics, brain structure and their influence on neuropsychology) and environmental (pregnancy or birth complications, exposure to environmental toxins, chaotic parenting, and diet) factors. 5 Disease course The interplay of environmental, genetic factors, and dysfunction in the frontal cortex, basal ganglia, corpus callosum, and cerebellum results in ADHD, which results in clinical manifestations of inattention, hyperactivity, and impulsivity. ADHD is associated with poor motor coordination, https://web.pathway.md/diseases/recpbVKrWa0Tdeebd 1/4 6/23/23, 3:15 AM Attention deficit and hyperactivity disorder Pathway impaired academic functioning, unintentional physical injury, sleep disturbances, and poor quality of life. 5 Prognosis and risk of recurrence ADHD is not associated with an increased risk of natural-cause mortality. 6 Calculator Calculator DSM 5 diagnostic criteria for AD DSM 5 diagnostic criteria for AD Guidelines 1. Screening and diagnosis Indications for testing: evaluate for ADHD in any pediatric patient 4-18 years old presenting with academic or behavioral problems and symptoms of inattention, hyperactivity, or impulsivity. B Diagnostic criteria: diagnose ADHD according to the DSM-5 criteria, including documentation of symptoms and impairment in 1 major setting (social, academic, or occupational), relying on information drawn primarily from reports from parents or guardians, teachers, other school personnel, and mental health clinicians involved in the patient's care. Rule out any alternative cause. B 2. Diagnostic investigations Screening for comorbid conditions: Assess for other conditions that might coexist with ADHD, including: Situation Guidance Anxiety, depression, oppositional defiant disorder, conduct disorders, substance use Emotional or behavioral conditions Learning and language disorders, autism spectrum disorders Developmental conditions Tics, sleep apnea. B Physical conditions Electroencephalography: inform patients with suspected ADHD and their families that EEG should not be used to confirm an attention deficit hyperactivity disorder diagnosis or to support https://web.pathway.md/diseases/recpbVKrWa0Tdeebd 2/4 6/23/23, 3:15 AM Attention deficit and hyperactivity disorder Pathway further testing after a clinical evaluation, unless such diagnostic assessments take place in the context of a research study. B 3. Medical management General principles: manage pediatric patients with ADHD similar to the care of pediatric patients with special healthcare needs, following the principles of the chronic care model and the medical home. B Medical therapy (indications): Offer United States FDA-approved medications along with parent training in behavior management and/or behavioral classroom intervention, preferably both, in pediatric patients 6- 12 years old (elementary and middle school-aged) with ADHD. B Offer United States FDA-approved medications in adolescent patients 12-18 years old with ADHD. B Medical therapy (dose titration): titrate doses of medications for ADHD to achieve maximum benefit with tolerable side effects. B Medical therapy (methylphenidate): consider offering methylphenidate in pediatric patients 4-5 years old if behavioral interventions do not provide significant improvement and there is moderate- to-severe continued disturbance in the patient's functioning. Weigh the risks of initiating medication before the age of 6 years against the harm of delaying treatment in areas where evidence-based behavioral treatments are not available. C 4. Nonpharmacologic interventions Behavioral therapy: as per AAP 2019 guidelines, offer evidence-based parent training in behavior management and/or behavioral classroom interventions as first-line therapy, if available, in pediatric patients 4-6 years old (preschool-aged) with ADHD. B Show 2 more Educational interventions: Include the following educational interventions and individualized instructional supports in any treatment plan of pediatric patients 6-18 years old with ADHD: school environment class placement instructional placement behavioral supports individualized education program rehabilitation plan (504 plan). B 5. Specific circumstances https://web.pathway.md/diseases/recpbVKrWa0Tdeebd 3/4 6/23/23, 3:15 AM Attention deficit and hyperactivity disorder Pathway Adult patients: recognize that various rating scales are available and are appropriate diagnostic tools for adult patients with ADHD. B Show 3 more 6. Follow-up and surveillance Indications for referral: refer patients with comorbid conditions to an appropriate subspecialist for treatment, or consider initiating treatment if trained or experienced in diagnosing such conditions. Refer patients with possible comorbid conditions to an appropriate subspecialist for diagnosis and treatment if not trained or experienced in diagnosing or treating such conditions. B References 1. Mark L Wolraich, Joseph F Hagan Jr, Carla Allan et al. Clinical Practice Guideline for the Diagnosis, Evaluation, and Treatment of Attention-Deficit/Hyperactivity Disorder in Children and Adolescents. Pediatrics. 2019 Oct;144 4):e20192528. Open 2. Robert E Post, Stuart L Kurlansik. Diagnosis and Management of Attention-Deficit/Hyperactivity Disorder in Adults. Am Fam Physician. 2012 May 1;85 9 890 6. Open 3. Brieger D, Amerena J, Attia JR et al. National Heart Foundation of Australia and Cardiac Society of Australia and New Zealand: Australian clinical guidelines for the diagnosis and management of atrial fibrillation 2018. Med J Aust. 2018 Oct 15;209 8 356 362. Open 4. Gloss D, Varma JK, Pringsheim T et al. Practice advisory: The utility of EEG theta/beta power ratio in ADHD diagnosis: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2016 Nov 29;87 22 2375 2379. Open 5. Ajay Singh, Chia Jung Yeh, Nidhi Verma et al. Overview of Attention Deficit Hyperactivity Disorder in Young Children. 2015 Apr 13;3 2 2115.2015 Apr 13;3 2 2115. Open 6. Vincent Chin-Hung Chen, Hsiang-Lin Chan, Shu-I Wu et al. Attention-Deficit/Hyperactivity Disorder and Mortality Risk in Taiwan. JAMA Netw Open. 2019 Aug 2;2 8):e198714. Open 7. Subcommittee on Attention-Deficit/Hyperactivity Disorder, Steering Committee on Quality Improvement and Management, Wolraich M et al. ADHD clinical practice guideline for the diagnosis, evaluation, and treatment of attention-deficit/hyperactivity disorder in children and adolescents. Pediatrics. 2011 Nov;128 5 1007 22. Open 8. David Coghill, Sarah Seth. Do the diagnostic criteria for ADHD need to change? Comments on the preliminary proposals of the DSM 5 ADHD and Disruptive Behavior Disorders Committee. Eur Child Adolesc Psychiatry. 2011 Feb;20 2 75 81. Open https://web.pathway.md/diseases/recpbVKrWa0Tdeebd 4/4
Guideline sources The following summarized guidelines for the evaluation and management of atypical hemolytic uremic syndrome (aHUS) are prepared by our editorial team based on guidelines from the Korean Working Group on Atypical Hemolytic Uremic Syndrome (KHWG 2016) and the British Society for Haematology (BSH 2010). 1 2 3 4 4 5 Definition AHUS is a complement-mediated disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. 3 Epidemiology AHUS is most commonly caused by dysregulation of the alternative complement pathway. 4 Disease course The failure of complement regulation results in aHUS, which presents with clinical manifestations of microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Extra-renal manifestations include altered consciousness, seizures, focal neurologic deficits, prodromic diarrhea, hypertension, and malaise. Disease progression may lead to end-stage renal disease. 5 https://web.pathway.md/diseases/reciRWE60gPxQD7RF 1/4 6/23/23, 3:15 AM Atypical hemolytic uremic syndrome Pathway Prognosis and risk of recurrence AHUS is associated with an overall mortality rate of 25%. 4 Guidelines 1. Screening and diagnosis Clinical presentation: Suspect atypical HUS in patients with: clinical evidence of thrombotic microangiopathy no evidence of STEC-associated HUS no evidence of TTP (ADAMTS13 activity > 10%). B 2. Diagnostic investigations ADAMTS13 activity: As per KHWG 2016 guidelines, obtain tests for ADAMTS13 activity to exclude TTP in all patients suspected of having atypical HUS. B As per BSH 2010 guidelines, obtain tests for ADAMTS13 activity in all patients with a clinical diagnosis of aHUS. If ADAMTS13 activity is found to be < 10%, specific assays to detect inherited deficiency or anti-ADAMTS13 antibodies should be undertaken. B Complement testing: As per KHWG 2016 guidelines, obtain testing for complement level abnormalities (C3, C4, factor H, factor I, factor B) in all patients with atypical HUS. B As per BSH 2010 guidelines, obtain testing for complement level abnormalities (C3, C4, factor H and factor I) in all patients with atypical HUS, as the results can guide prognosis and transplantation options. B Shiga toxin test: obtain tests for Shiga toxin/enterohemorrhagic E. coli to exclude STEC-HUS for all patients suspected of having atypical HUS. B Anti-factor H antibodies: As per KHWG 2016 guidelines, test for anti-factor H antibodies in all patients with atypical HUS. B As per BSH 2010 guidelines, test for anti-factor H antibodies in all patients with atypical HUS. B Plasma homocysteine, methionine, and methylmalonic acid: obtain tests for plasma homocysteine, methionine, and MMA to exclude cobalamin defect HUS for all patients suspected of having atypical HUS. B Other laboratory tests: consider obtaining other laboratory tests for rare associated conditions in patients with thrombotic microangipathies: DIC panel, autoimmune serology, paroxysmal https://web.pathway.md/diseases/reciRWE60gPxQD7RF 2/4 6/23/23, 3:15 AM Atypical hemolytic uremic syndrome Pathway nocturnal Hgburia screening by flow cytometry, and human immunodeficiency virus screening. B FACS analysis: obtain FACS for expression of CD46 on peripheral blood mononuclear cells in all patients presenting with clinical features compatible with a diagnosis of atypical HUS. B Genetic testing: As per KHWG 2016 guidelines, perform genetic screening for complementary abnormalities in patients with atypical HUS. B As per BSH 2010 guidelines, test for mutations in factor H, factor I, factor B, CD46 and C3 in all patients with atypical HUS. If mutations are found in other genes in the research setting then these should be incorporated into the molecular diagnostic portfolio. B 3. Diagnostic procedures Tissue biopsy: consider performing tissue biopsies on a case-by-case basis in patients with suspected atypical HUS. C 4. Medical management Eculizumab: Administer eculizumab, where available, as first-line treatment for patients with symptomatic atypical HUS. B Provide meningococcal vaccination to all patients scheduled to receive eculizumab, prior to administering the first dose. A 5. Therapeutic procedures Plasma exchange: As per KHWG 2016 guidelines, offer a trial of plasma exchange and/or plasma infusion to all patients who are clinically suspected of having atypical HUS, if eculizumab is not available. B As per BSH 2010 guidelines, offer a trial of plasma exchange and/or plasma infusions to all patients with atypical HUS. B 6. Surgical interventions Kidney/liver transplantation: Avoid performing renal transplantation alone in patients with atypical HUS. D Consider performing isolated liver transplantation or a combined liver and kidney transplantation in patients with a factor H, factor I, factor B, or C3 mutation. C https://web.pathway.md/diseases/reciRWE60gPxQD7RF 3/4 6/23/23, 3:15 AM Atypical hemolytic uremic syndrome Pathway 7. Patient education Trigger avoidance: counsel patients with atypical HUS who have achieved clinical remission to avoid any identifiable trigger factors as much as possible. B References 1. C Mark Taylor, Sam Machin, Stephen J Wigmore et al. Clinical practice guidelines for the management of atypical haemolytic uraemic syndrome in the United Kingdom. Br J Haematol. 2010 Jan;148 1 37 47. Open 2. Hae Il Cheong, Sang Kyung Jo, Sung Soo Yoon et al. Clinical Practice Guidelines for the Management of Atypical Hemolytic Uremic Syndrome in Korea. J Korean Med Sci. 2016 Oct;31 10 1516 28. Open 3. David Kavanagh, Tim H Goodship, Anna Richards. Atypical hemolytic uremic syndrome. 2013 Nov;33 6 508 30.2013 Nov;33 6 508 30. Open 4. Vahid Afshar-Kharghan. Atypical hemolytic uremic syndrome. 2016 Dec 2;2016 1 217 225.2016 Dec 2;2016 1 217 225. Open 5. Kuixing Zhang, Yuxin Lu, Kevin T Harley et al. Atypical Hemolytic Uremic Syndrome: A Brief Review. 2017 Jun 1;9 2 7053.2017 Jun 1;9 2 7053. Open https://web.pathway.md/diseases/reciRWE60gPxQD7RF 4/4
Guideline sources The following summarized guidelines for the evaluation and management of autism spectrum disorder (ASD) are prepared by our editorial team based on guidelines from the British Association for Psychopharmacology (BAP 2018), the U.S. Preventive Services Task Force (USPSTF 2016), and the American Academy of Child and Adolescent Psychiatry (AACAP 2014). 1 2 3 4 4 5 5 6 7 Definition ASD comprises a heterogeneous group of neurodevelopmental disorders characterized by early- onset deficits in social communication as well as restricted and repetitive interests and behaviors. 4 Epidemiology ASD is associated with alterations in brain connectivity resulting from a complex interaction of genetic, epigenetic and environmental factors. 5 Pathophysiology The prevalence of ASD in the United States is estimated at 1250-2410 persons per 100,000 population. 4 https://web.pathway.md/diseases/receYTB3zBBB9w2tb 1/4 6/23/23, 3:16 AM Autism spectrum disorder Pathway Disease course Clinical manifestations include developmental delay, deficits in social interaction, communication, and repetitive/restricted behaviors. Approximately 75% of patients with ASD have comorbid psychiatric conditions including anxiety, depression, attention-deficit hyperactivity disorder, bipolar disorder, and Tourette syndrome. 5 6 Prognosis and risk of recurrence Individuals with ASD have an estimated excess mortality rate ratio of 5.6. Associated medical disorders (including epilepsy with cognitive impairment) and accidents account for most of the deaths. 7 Calculator DSM 5 diagnostic criteria for aut Guidelines 1. Screening and diagnosis Indications for screening: as per USPSTF 2016 guidelines, insufficient evidence to assess the balance of benefits and harms of screening for ASD in young children for whom no concerns of ASD have been raised by their parents or a clinician. I Diagnostic process: Employ a multidisciplinary approach for the diagnosis of ASD. B Perform direct clinical assessment of the individual, and, wherever possible, obtain a detailed interview with the caregiver or other informants, reports from school and employment, and assessment of cognitive and language skills, and a medical examination. B Diagnostic criteria: Obtain a developmental history to support the diagnosis of ASD. B Use established diagnostic criteria, such as the DSM-5 criteria, for diagnosis of ASD. B 2. Diagnostic investigations Clinical assessment: include questions about ASD symptomatology in the developmental assessment of young children and in the psychiatric assessment of all children. B 3. Medical management https://web.pathway.md/diseases/receYTB3zBBB9w2tb 2/4 6/23/23, 3:16 AM Autism spectrum disorder Pathway Pharmacotherapy: As per BAP 2018 guidelines: Insufficient evidence to recommend any pharmacological agent for the routine management of ASD. I Avoid risperidone and aripiprazole in the management of repetitive behaviors in view of potential adverse effects. If used, clinicians should weigh up the risks and benefits and reevaluate these regularly. D As per AACAP 2014 guidelines, consider pharmacotherapy in children with ASD when there is a specific target symptom or comorbid condition. C 4. Nonpharmacologic interventions Alternative medicine: inquire about the use of alternative or complementary treatments and discuss their risk and potential benefits. B Behavioural interventions: assist families in obtaining appropriate, evidence-based, and structured educational and behavioral interventions for children with ASD. B 5. Specific circumstances Patients with depression: Individualize the approach to treatment of depression in pediatric patients with ASD. Follow established guidelines to treat depression. B Individualize the approach to treatment of depression in adult patients with ASD. Follow established guidelines to treat depression. B Patients with anxiety: consider a cautious trial of SSRIs in children with ASD and anxiety, followed by risperidone in patients in whom SSRIs are poorly efficacious. Monitor for worsening of anxiety in some children. C Patients with sleep disorders: Use melatonin, if possible, in combination with a behavioral intervention in children with sleep disorders occurring in the context of ASD. A Use melatonin, if possible, in combination with a behavioral intervention in adults with sleep disorders occurring in the context of ASD. B Patients with irritability: Consider risperidone or aripiprazole for the treatment of irritability in children with ASD only when behavioral or educational approaches have failed. B Consider aripiprazole, risperidone, or a SSRI for the treatment of of irritability in adults with ASD on a case-by-case basis, and only after considering alternatives. C Patients with ADHD: use methylphenidate as first-line therapy, and atomoxetine or alpha2a receptor agonists as second-line therapy, for the management of attention deficit hyperactivity https://web.pathway.md/diseases/receYTB3zBBB9w2tb 3/4 6/23/23, 3:16 AM Autism spectrum disorder Pathway disorder in patients with autism. A Patients with tic disorders and Tourette's syndrome: individualize treatment decisions in patients with ASD and tic disorders or Tourette's syndrome. B 6. Follow-up and surveillance Long-term support: maintain an active role in long-term treatment planning and in the support of the individual and the individual's family. B References 1. Howes OD, Rogdaki M, Findon JL et al. Autism spectrum disorder: Consensus guidelines on assessment, treatment and research from the British Association for Psychopharmacology. J Psychopharmacol. 2018 Jan;32 1 3 29. Open 2. Volkmar F, Siegel M, Woodbury-Smith M et al. Practice parameter for the assessment and treatment of children and adolescents with autism spectrum disorder. J Am Acad Child Adolesc Psychiatry. 2014 Feb;53 2 237 57. Open 3. Siu AL, US Preventive Services Task Force USPSTF , Bibbins-Domingo K et al. Screening USPSTF for Autism Spectrum Disorder in Young Children: US Preventive Services Task Force Recommendation Statement. JAMA. 2016 Feb 16;315 7 691 6. Open 4. Myers SM, Voigt RG, Colligan RC et al. Autism Spectrum Disorder: Incidence and Time Trends Over Two Decades in a Population-Based Birth Cohort. J Autism Dev Disord. 2019 Apr;49 4 1455 1474. Open 5. Sharma SR, Gonda X, Tarazi FI. Autism Spectrum Disorder: Classification, diagnosis and therapy. Pharmacol Ther. 2018 Oct;190 91 104. Open 6. Yenkoyan K, Grigoryan A, Fereshetyan K et al. Advances in understanding the pathophysiology of autism spectrum disorders. Behav Brain Res. 2017 Jul 28;331 92 101. Open 7. Bilder D, Botts EL, Smith KR et al. Excess mortality and causes of death in autism spectrum disorders: a follow up of the 1980s Utah/UCLA autism epidemiologic study. J Autism Dev Disord. 2013 May;43 5 1196 204. Open 8. Schaefer GB, Mendelsohn NJ, Professional Practice and Guidelines Committee. Clinical genetics evaluation in identifying the etiology of autism spectrum disorders: 2013 guideline revisions. Genet Med. 2013 May;15 5 399 407. Open 9. Anne Masi, Marilena M DeMayo, Nicholas Glozier et al. An Overview of Autism Spectrum Disorder, Heterogeneity and Treatment Options. Neurosci Bull. 2017 Apr;33 2 183 193. Open https://web.pathway.md/diseases/receYTB3zBBB9w2tb 4/4
Guideline sources The following summarized guidelines for the evaluation and management of autoimmune hemolytic anemia (AIHA) are prepared by our editorial team based on guidelines from the First International Consensus Meeting (FICM 2020), the American Society for Apheresis (ASFA 2019), and the British Society for Haematology (BSH 2017). 1 2 3 4 Guidelines 1. Screening and diagnosis Diagnosis: obtain direct antiglobulin test (C3d+), a cold agglutinin titer and bone marrow examination (at least by histology and flow cytometry) for the diagnosis of cold agglutinin disease. E Show 2 more 2. Classification and risk stratification https://web.pathway.md/diseases/recPvnwIOFm4Eqibw 1/9 6/23/23, 3:16 AM Autoimmune hemolytic anemia Pathway Assessment of thrombotic risk: Take into account the risk of thrombotic events (both venous and arterial), mostly during acute hemolytic flares and in splenectomized patients. E Obtain assessment of general thrombotic risk factors in patients with AIHA: age > 70 years, active cancer, previous VTE, reduced mobility, already known thrombophilic condition, recent trauma and/or surgery, heart and/or respiratory failure, acute infection. E 3. Diagnostic investigations Initial assessment: Assess for signs of acrocyanosis in patients with cold agglutinin disease. E Obtain baseline values of LDH, bilirubin, haptoglobin, reticulocyte count, and review of peripheral smear (looking for spherocytes in warm AIHA, RBC aggregation in cold agglutinin disease, and schiztocytes to exclude thrombotic microangiopethies) in all patients with AIHA. E Direct antiglobulin test: As per FICM 2020 guidelines, obtain direct antiglobulin test with monospecific antisera (anti-IgG, anti-IgA, anti-IgM, anti-C). E Show 2 more As per BSH 2017 guidelines: Include monospecific anti-IgG and anti-C3d in the direct antiglobulin testing. B Retest with a column agglutination direct antiglobulin test method including monospecific anti- IgG, anti-IgA and anti-C3d in patients with unexplained hemolysis and a negative screening direct antiglobulin test. B Consider preparing and investigating a red cell eluate if the latter is also negative. B Direct agglutination test: screen for a cold antibody using a direct agglutination test at room temperature in patients with AIHA and a direct antiglobulin test positive for C3 +/- IgG. B Cold agglutinin test: As per FICM 2020 guidelines, suspect mixed AIHA and obtain cold agglutinin test and titer in patients with direct antiglobulin test positive for IgG and C3 and presence of symptoms of cold agglutinin disease. E As per BSH 2017 guidelines: Obtain further investigation with an antibody titer in a laboratory performing these tests on a regular basis in patients with a positive cold autoantibody screen. B Warm received EDTA- anticoagulated samples to 37 C in a water bath for 1 hour before removing the plasma for testing. B Review all cases of suspected primary cold agglutinin disease by an appropriately constituted hemato-oncology multidisciplinary team. B Donath-Landsteiner test: obtain Donath-Landsteiner biphasic test in an experienced laboratory in case of diagnostic uncertainty after AIHA evaluation in patients with fitting history. E https://web.pathway.md/diseases/recPvnwIOFm4Eqibw 2/9 6/23/23, 3:16 AM Autoimmune hemolytic anemia Pathway Evaluation for underlying cause: As per FICM 2020 guidelines: Evaluate all patients with warm AIHA for underlying causes as follows: Situation Guidance ANA Autoimmune diseases RF Antiphospholipid antibody Flow cytometry of peripheral blood Lymphoproliferative disorders Review of peripheral blood smear Possible CT assessing for lymphadenopathy and splenomegaly SPEP with immunofixation. E Immune deficiency Evaluate all patients with suspected cold agglutinin disease for clonal B-cell disorder with SPEP, immunofixation, peripheral blood and bone marrow flow cytometry, bone marrow biopsy, and, if indicated, CT looking for lymphadenopathy and splenomegaly. E As per BSH 2017 guidelines, keep the clotted sample from patients with suspected cold agglutinin disease for protein electrophoresis and immunofixation at 37 C until the serum has been separated. B Assessment of related conditions: Obtain careful evaluation for Evans syndrome, acute renal failure, infections and multi- treatment, as these conditions are associated with increased risk of death (AIHA-related mortality is 3-4%). E Obtain close monitoring in patients with AIHA with severe anemia at onset, and/or presence of Evans syndrome, since these conditions are associated with an increased risk of multiple relapses/refractoriness to several therapy lines. E 4. Medical management Management of primary warm AIHA, indications: As per FICM 2020 guidelines: Initiate treatment in patients with warm AIHA in the setting of symptomatic anemia. E Consider obtaining monitoring (watchful waiting) in rare patients with mild and stable anemia. E As per BSH 2017 guidelines, do not affect the therapeutic approach to warm AIHA by identification of warm IgA antibodies (concurrent with IgM, IgG or as an isolated cause of AIHA). D Management of primary warm AIHA, first-line therapy: https://web.pathway.md/diseases/recPvnwIOFm4Eqibw 3/9 6/23/23, 3:16 AM Autoimmune hemolytic anemia Pathway As per FICM 2020 guidelines, initiate oral prednisone/prednisolone starting at 1 mg/kg daily as first-line therapy in patients with warm AIHA. E Show 3 more As per BSH 2017 guidelines, initiate prednisolone 1 mg/kg/day as first-line therapy in patients with warm AIHA. B Management of primary warm AIHA, second-line therapy: As per FICM 2020 guidelines, consider initiating second-line therapy in patients not responding to first-line therapy and in patients relapsing as the corticosteroid is weaned. E Show 2 more As per BSH 2017 guidelines: Consider initiating second-line therapy in patients not responding to prednisolone 1 mg/kg/day after 3 weeks and in patients relapsing during or after corticosteroid reduction. C Initiate rituximab as second-line therapy in patients with warm AIHA. B Management of primary warm AIHA, third-line therapy: As per FICM 2020 guidelines, offer azathioprine, cyclosporin, mycophenolate mofetil, and splenectomy as third-line treatment options in patients with warm AIHA, based on the individual assessment of the benefit over risk ratio. E As per BSH 2017 guidelines, offer azathioprine, ciclosporin, danazol, mycophenolate mofetil, and splenectomy as third-line treatment options in patients with warm AIHA. B Management of primary warm AIHA (refractory cases): Refer patients with chronic refractory warm AIHA not responding to at least 3 treatment lines (including splenectomy and or at least one immunosuppressant) to a specialized center and, whenever possible, treat in the setting of a clinical trial. E Consider offering other lines of therapy including cyclophosphamide, continuous low-dose prednisone, danazol, hamatopoietic stem cell transplantation, and bortezomib. E Management of primary warm AIHA, emergency management: As per FICM 2020 guidelines, combine supportive therapies (ESA +/- plasma exchange and/or IVIG), active immunosuppression, and emergency splenectomy/embolization for the management of patients with transfusion-dependent life-threatening warm AIHA. Administer thromboprophylaxis to minimize the risk of venous thrombosis. E As per BSH 2017 guidelines, consider offering IVIG or plasma exchange in patients with severe or life-threatening anemia. C Management of primary cold AIHA (indications): initiate treatment for the indications of symptomatic anemia, severe circulatory symptoms or transfusion dependence. B Management of primary cold AIHA (avoidance of cold): advise patients to avoid cold exposure where possible. B Show 2 more Management of primary cold AIHA, first-line therapy: https://web.pathway.md/diseases/recPvnwIOFm4Eqibw 4/9 6/23/23, 3:16 AM Autoimmune hemolytic anemia Pathway As per FICM 2020 guidelines, initiate rituximab, alone or in combination with bendamustine, as first-line therapy in patients with cold AIHA. E As per BSH 2017 guidelines: Initiate rituximab as first-line therapy in patients with cold AIHA. B Consider adding fludarabine if clonality has been demonstrated. B Management of primary cold AIHA (second-line therapy): consider initiating re-treatment with rituximab as monotherapy or in combination with bendamustine or initiating fludarabine as second-line therapy in patients with cold AIHA. E Management of primary cold AIHA (other therapies): Consider offering other potentially useful agents such as tyrosine kinase- or BCL2-inhibitors in patients with cold AIHA. E Recognize that complement inhibition at various levels of the complement cascade is a promising novel approach for later treatment lines or emergencies. E Management of primary cold AIHA, emergency management: As per FICM 2020 guidelines: Manage patients with severe and life-threatening hemolytic anemia requiring repeated transfusions despite high dose of corticosteroids and rituximab in ICU in a tertiary referral center. E Consider offering either intravenous IgAnd/or plasma exchange as a transfusion-sparing strategy. E As per BSH 2017 guidelines, consider offering plasma exchange or corticosteroids in patients with severe or life-threatening anemia. C Management of mixed AIHA: As per FICM 2020 guidelines: Initiate early aggressive therapy with corticosteroids and rituximab and consider offering participation in clinical trials with new drugs, since mixed AIHA generally has a severe onset and exhibits relapse/refractoriness to several therapies. E Do not perform splenectomy in patents with mixed AIHA. D As per BSH 2017 guidelines, initiate prednisolone 1 mg/kg/day as first-line therapy in patients with mixed AIHA. B Show 2 more Management of paroxysmal cold hemoglobinuria: Encourage cold avoidance and avoid active cooling for fever. B Consider initiating corticosteroids only in patients with severe or persistent disease. C Management of drug-induced AIHA: discontinue the suspected drug. A Show 2 more Management of secondary AIHA, lymphoproliferative disorders: https://web.pathway.md/diseases/recPvnwIOFm4Eqibw 5/9 6/23/23, 3:16 AM Autoimmune hemolytic anemia Pathway As per FICM 2020 guidelines, take into account the stage and activity of chronic lymphocytic leukemia in the management of chronic lymphocytic leukemia-associated warm AIHA must. E Show 4 more As per BSH 2017 guidelines, take into account the lymphoma type and remission status when deciding whether treatment should be anti-lymphoma or AIHA directed. B Show 3 more Management of secondary AIHA (solid organ neoplasms): perform surgical resection as first- line therapy in patients with ovarian teratoma. Do not perform concomitant splenectomy. B Show 2 more Management of secondary AIHA, immune dysregulations: As per FICM 2020 guidelines, conside initiating rituximab for the management of patients with corticosteroid-refractory or dependent SLE-associated warm AIHA. E Show 4 more As per BSH 2017 guidelines, initiate corticosteroids as first-line therapy and azathioprine, danazol, mycophenolate mofetil, or rituximab as second-line therapy in patients with systemic lupus erythematous-associated AIHA. B Show 12 more Management of secondary AIHA (infections): initiate supportive therapy with appropriate antimicrobials, a warm environment and transfusion in symptomatic anemia in patients with cold agglutinin disease secondary to atypical and mycoplasma pneumonia. B Show 5 more Management of Evans syndrome: Initiate corticosteroids or IVIG as first-line therapy in patients with primary Evans syndrome. B Consider initiating azathioprine, ciclosporin, danazol, mycophenolate mofetil, rituximab, vincristine or offering splenectomy as second-line therapy in patients with primary Evans syndrome. C Thromboprophylaxis: As per FICM 2020 guidelines, consider administering VTE prophylaxis in patients with marked hemolysis and associated risk factors. E Show 2 more As per BSH 2017 guidelines, administer thromboprophylaxis with LMWH in inpatients with an acute exacerbation of hemolysis B and consider administering in ambulatory patients during severe exacerbations - Hgb < 85 g/L. B Management of corticosteroid side effects: As per FICM 2020 guidelines, provide dietary and lifestyle advice in all adult patients starting corticosteroids. E Show 3 more As per BSH 2017 guidelines, initiate PPIs in patients receiving corticosteroids at increased risk for peptic ulcer disease (such as concomitant thrombocytopenia, history of history peptic ulcer https://web.pathway.md/diseases/recPvnwIOFm4Eqibw 6/9 6/23/23, 3:16 AM Autoimmune hemolytic anemia Pathway disease, concurrent use of NSAIDs, anticoagulant or antiplatelet agents and age 60 years). B Show 2 more 5. Nonpharmacologic interventions Folic acid supplementation: As per FICM 2020 guidelines: Initiate folic acid supplementation in patients with active hemolysis. E Monitor hematinic in case of reticulocytosis, or if anemia worsens. E As per BSH 2017 guidelines, initiate folic acid supplementation in patients with AIHA. B 6. Therapeutic procedures Blood transfusion: Administer transfusion with ABO, Rh and K matched red cells if anemia is life-threatening in the time required for full compatibility testing. B Consider using a blood warmer for transfusion in patients with cold AIHA (cold agglutinin disease, mixed AIHA and paroxysmal cold Hgburia). C 7. Surgical interventions Splenectomy, indications: As per FICM 2020 guidelines, offer splenectomy as a third-line treatment option in patients with primary warm AIHA, based on the individual assessment of the benefit over risk ratio. E Show 3 more As per BSH 2017 guidelines, offer splenectomy as second-line therapy in patients with primary Evans syndrome. B Splenectomy (preoperative evaluation): Do not obtain radioisotope studies to determine the main site of red cell destruction when considering splenectomy. D Counsel patients on infection risk and be vaccinated at least 2 weeks before splenectomy. B Splenectomy (postoperative care): set a low threshold for investigating patients with postoperative fever, abdominal pain or ileus with Doppler ultrasound to exclude portal or splenic vein thrombosis. B Show 3 more 8. Specific circumstances Pediatric patients: https://web.pathway.md/diseases/recPvnwIOFm4Eqibw 7/9 6/23/23, 3:16 AM Autoimmune hemolytic anemia Pathway As per BSH 2017 guidelines, include LFTs in the evaluation of pediatric patients with AIHA. B Show 2 more As per BSH 2017 guidelines, suspect giant cell hepatitis and perform liver biopsy in pediatric patients with unexplained elevated hepatic transaminases. B Pregnant patients (evaluation): Elicit a history, obtain examination and laboratory testing promptly to exclude AIHA in pregnant patients with a positive direct antiglobulin test. B Obtain serial ultrasound from 20 weeks to assess fetal growth and Doppler ultrasound of the fetal middle cerebral artery to screen for fetal anemia in pregnant patients with AIHA. B Pregnant patients, antenatal care: As per FICM 2020 guidelines, involve both an obstetrician and a hematologist in the antenatal care of pregnant patients with AIHA. E Show 2 more As per BSH 2017 guidelines, provide antenatal care involving joint hematology and obstetric care with access to a specialist in fetal medicine. B Show 4 more Pregnant patients (postnatal care): obtain direct antiglobulin test in cord blood following delivery. Obtain a full blood count, reticulocyte count, bilirubin, direct antiglobulin test and cross-match (in case exchange transfusion is required) in capillary blood sample from the neonate, if neonatal jaundice is present or direct antiglobulin test is positive. B Show 2 more References 1. Quentin A Hill, Robert Stamps, Edwin Massey et al. The diagnosis and management of primary autoimmune haemolytic anaemia. Br J Haematol. 2017 Feb;176 3 395 411. Open 2. Quentin A Hill, Robert Stamps, Edwin Massey et al. Guidelines on the management of drug-induced immune and secondary autoimmune, haemolytic anaemia. Br J Haematol. 2017 Apr;177 2 208 220. Open 3. Ulrich J ger, Wilma Barcellini, Catherine M Broome et al. Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting. Blood Rev. 2020 May;41 100648. Open 4. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 5. David J Kuter, Kerry A Rogers, Michael A Boxer et al. Fostamatinib for the treatment of warm antibody autoimmune hemolytic anemia: Phase 2, multicenter, open-label study. Am J Hematol. 2022 Jun 1;97 6 691 699. Open 6. Alberto Zanella, Wilma Barcellini. Treatment of autoimmune hemolytic anemias. Haematologica. 2014 Oct;99 10 1547 54. Open https://web.pathway.md/diseases/recPvnwIOFm4Eqibw 8/9 6/23/23, 3:16 AM Autoimmune hemolytic anemia Pathway https://web.pathway.md/diseases/recPvnwIOFm4Eqibw 9/9
Guideline sources The following summarized guidelines for the evaluation and management of autoimmune hepatitis are prepared by our editorial team based on guidelines from the American Association for the Study of Liver Diseases (AASLD 2020), the Hellenic Association for the Study of the Liver (HASL 2019), the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN 2018), the American College of Gastroenterology (ACG 2016; 2015), the Italian Association for the Study of the Liver (AISF 2016), and the European Association for the Study of the Liver (EASL 2015). 1 2 3 4 5 6 7 8 9 10 11 Definition AIH is a chronic liver disease of unknown etiology characterized by interface hepatitis, hypergammaglobulinaemia, and circulating autoantibodies. 8 Epidemiology https://web.pathway.md/diseases/recy694asCYrsgEzB 1/12 6/23/23, 3:16 AM Autoimmune hepatitis Pathway The cause of AIH is primarily unknown, but there is a complex interplay of genetic (HLA haplotype) and environmental factors. 9 Disease course The immunological reaction against autologous liver antigens in genetically predisposed individuals results in AIH, which may an asymptomatic, acute, or acute severe (fulminant) presentation. Disease progression may lead to hepatic encephalopathy, cirrhosis, and a need for the liver transplantation. 10 Prognosis and risk of recurrence All-cause mortality/liver transplant rates for AIH over a period of 10 years is 13%. 11 CalculatorHistology activity index HAI Guidelines 1. Screening and diagnosis Clinical presentation: As per AASLD 2020 guidelines, suspect the diagnosis of AIH in all patients presenting with acute or chronic liver disease, including patients with asymptomatic liver test abnormalities, ALF, and autoantibody-negative hepatitis. E As per EASL 2015 guidelines, suspect AIH in any patient with acute or chronic liver disease, particularly in the context of hypergammaglobulinemia. B Show 2 more Diagnostic criteria: As per AASLD 2020 guidelines, diagnose AIH based on compatible histological findings and further support the diagnosis by the following features: elevated serum aminotransferase levels elevated serum IgG level and/or positive serological markers exclusion of viral, hereditary, metabolic, cholestatic, and drug-induced diseases potentially resembling AIH. E Show 4 more As per HASL 2019 guidelines, suspect AIH in any patient with acute or chronic hepatitis, particularly in the presence of high IgG levels, as it has a global distribution at any age in both sexes and in all ethnic groups. B https://web.pathway.md/diseases/recy694asCYrsgEzB 2/12 6/23/23, 3:16 AM Autoimmune hepatitis Pathway Show 6 more As per EASL 2015 guidelines, diagnose AIH based on the presence of autoantibodies, hypergammaglobulinemia and typical or compatible histology. B 2. Classification and risk stratification Classification: Classify the diagnosis of acute AIH as one of the following two clinical forms of presentation: acute worsening of previously undiagnosed or misdiagnosed AIH real (original) acute onset of AIH without chronic lesions on liver histology. B Consider classifying AIH into two types: AIH-1: ANA, smooth muscle antibodies and/or anti-SLA/LP positive AIH-2: anti-LKM1, anti-LKM3 and/or anti-LC1 positive. C 3. Diagnostic investigations Immunoglobulin levels: As per HASL 2019 guidelines, do not exclude the diagnosis of AIH in patients with normal IgG levels, even though a selective high serum IgG is an important AIH characteristic. D As per EASL 2015 guidelines, recognize that: elevated IgG, especially in the absence of cirrhosis, is suggestive of AIH B elevated IgG in the absence of IgA and IgM elevation is particularly suggestive of AIH B normal IgG or gamma-globulin do not preclude the diagnosis of AIH most of patients with normal IgG demonstrate a fall of IgG levels upon treatment. B Autoantibody titers: As per AASLD 2020 guidelines: Obtain ANA and ASMA in adults and ANA, ASMA, and anti-LKM1 in children in the initial serological testing for AIH. E Consider obtaining additional autoantibody tests if warranted to secure the diagnosis. E As per HASL 2019 guidelines, use IDIF, preferably on freshly frozen rodent substrates, for routine screening in the detection of most autoantibodies and use ELISA and immunoblotting as the tests for anti-SLA/LP and anti-LC1 autoantibodies. B As per EASL 2015 guidelines, recognize that circulating non-organ specific antibodies are present in the vast majority of AIH patients. B Show 2 more Cholangiography: As per AASLD 2020 guidelines, obtain cholangiography to evaluate for large-duct PSC for AIH- PSC overlap syndrome in patients with AIH, cholestatic laboratory findings, histological features https://web.pathway.md/diseases/recy694asCYrsgEzB 3/12 6/23/23, 3:16 AM Autoimmune hepatitis Pathway of bile duct injury or loss, and concurrent chronic ulcerative colitis. E As per HASL 2019 guidelines, consider obtaining MRCP in adult patients with AIH only when cholestatic laboratory manifestations are present. C As per ACG 2015 guidelines, obtain MRCP for patients < 25 years of age with AIH who have elevated serum ALP (usually > 2 times the ULN). B As per EASL 2015 guidelines: Consider obtaining magnetic resonance cholangiography to exclude autoimmune sclerosing cholangitis in adult patients with AIH and cholestatic pattern of laboratory abnormalities. C Obtain magnetic resonance cholangiography to exclude autoimmune sclerosing cholangitis in all pediatric patient with AIH. B Screening for drug-induced liver injury: Include drug-induced AIH-like liver injury in the differential diagnosis of AIH. E Withdraw the offending agent and maintain monitoring to ensure laboratory resolution. E Screening for liver cirrhosis: As per AASLD 2020 guidelines: Do not obtain serum-based biomarker panels for hepatic fibrosis in patients with AIH. D Defer obtaining vibration-controlled transient elastography for the detection of advanced fibrosis or cirrhosis for at least 6 months after successful treatment of AIH in order to avoid the confounding effects of hepatic inflammation. E As per HASL 2019 guidelines, assess for cirrhosis at diagnosis of AIH, as almost 33% of adult patients and 50% of pediatric patients with AIH are first diagnosed at the stage of cirrhosis, indicating that they have had subclinical disease for a long time. B Evaluation for concomitant diseases: As per AASLD 2020 guidelines, screen patients with AIH for celiac and thyroid diseases at diagnosis. E Show 2 more As per HASL 2019 guidelines, recognize that coexistence of AIH features and cholestatic diseases can be observed at both diagnosis and follow-up, but their diagnosis may be problematic because of the lack of internationally accepted criteria. B Show 2 more As per EASL 2015 guidelines: Obtain diagnostic tests for primary biliary cirrhosis and PSC in patients showing features of cholestasis. B Recognize that AIH is associated with a wide variety of other autoimmune diseases. B Psychosocial assessment: As per AASLD 2020 guidelines, monitor for manifestations of depression and changes in the quality of life throughout management of AIH. Consider using structured and validated questionnaires for objective assessment. E https://web.pathway.md/diseases/recy694asCYrsgEzB 4/12 6/23/23, 3:16 AM Autoimmune hepatitis Pathway As per EASL 2015 guidelines, address psychosocial needs in the management of patients with AIH, recognizing the associated decreased quality of life. B 4. Diagnostic procedures Liver biopsy: perform liver biopsy in the initial diagnostic work-up of AIH, as it is a prerequisite for the confirmation of diagnosis. B Show 2 more 5. Medical management General principles: As per HASL 2019 guidelines, treat all patients with active disease, even with advanced fibrosis or cirrhosis. A Show 2 more As per EASL 2015 guidelines, initiate treatment in all patients with active AIH. A Show 4 more Treatment targets: As per HASL 2019 guidelines, aim to achieve complete biochemical and histological remission in an attempt to prevent potential disease progression. B Show 2 more As per EASL 2015 guidelines, aim to obtain complete biochemical and histological resolution of the disease in order to prevent further progression of liver disease. B Show 2 more Corticosteroids: As per AASLD 2020 guidelines, initiate corticosteroids in patients with drug-induced AIH-like injury when symptoms or disease activity are severe (fulfilling Hy's law) or if symptoms and laboratory tests fail to improve or worsen after discontinuation of the offending drug. E Show 3 more As per HASL 2019 guidelines, initiate prednisolone 0.5-1 mg/kg/day at one oral dose in the morning, combined with azathioprine at an initial morning dose of 50 mg/day usually after 2 weeks, if bilirubin is < 6 mg/dL, as first-line therapy in patients with AIH. A Show 2 more As per EASL 2015 guidelines, initiate prednisone or prednisolone, followed by the addition of azathioprine after 2 weeks of corticosteroids, as first-line therapy in patients with AIH. A Show 5 more Immunosuppressive agents: As per AASLD 2020 guidelines: https://web.pathway.md/diseases/recy694asCYrsgEzB 5/12 6/23/23, 3:16 AM Autoimmune hepatitis Pathway Consider screening patients with AIH for absent or near-absent TPMT activity before initiating treatment with azathioprine. E Consider initiating a trial of mycophenolate mofetil rather than tacrolimus as the initial second- line agent in patients with AIH. C As per HASL 2019 guidelines, initiate mycophenolate mofetil as second-line therapy in patients intolerant to azathioprine. B Consider initiating mercaptopurine or thioguanine as an alternative. B As per EASL 2015 guidelines, consider initiating azathioprine whenever bilirubin levels are < 6 mg/dl (100 mcmol/L) and ideally 2 weeks after the initiation of corticosteroid treatment. Administer azathioprine at an initial dose of 50 mg/day and increase depending on toxicity and response up until a maintenance dose of 1-2 mg/kg. C Show 3 more Biologics: consider initiating anti-TNF or anti-CD20 therapy as an alternative after first-line and second-line regimens have failed. E Duration of treatment: As per AASLD 2020 guidelines: Consider withdrawing treatment and achieving a long-term treatment-free remission of AIH in patients with normalized serum aminotransferase and IgG levels for at least 2 years. E Consider performing liver tissue examination before drug withdrawal for excluding unsuspected inflammation and reducing the frequency of relapse. E As per HASL 2019 guidelines, perform liver biopsy before treatment discontinuation in patients with AIH in persistent complete biochemical remission for at least the last 2 years of immunosuppression. B Show 2 more As per EASL 2015 guidelines, continue immunosuppressive treatment for 3 years in total and for 2 years following complete normalization of transaminases and IgG. B Show 3 more Maintenance therapy: As per HASL 2019 guidelines, increase azathioprine dose up to 1-2 mg/kg/day (maintenance dose). B Show 7 more As per EASL 2015 guidelines, recognize that only a small minority of patients stay in remission without maintenance therapy. B Show 2 more 6. Surgical interventions Liver transplantation: As per AASLD 2020 guidelines, evaluate patients with AIH for liver transplantation in the following circumstances: https://web.pathway.md/diseases/recy694asCYrsgEzB 6/12 6/23/23, 3:16 AM Autoimmune hepatitis Pathway ALF failed to improve laboratory tests within 1-2 weeks of corticosteroid therapy clinical deterioration within 1-2 weeks of corticosteroid therapy. E Show 4 more As per HASL 2019 guidelines, manage patients with AIH after liver transplantation, either recurrent or de novo, by the basic treatment principles of AIH. B As per EASL 2015 guidelines: List patients with acute severe AIH for emergency liver transplantation after lack of improvement with higher doses of corticosteroids ( 1 mg/kg) within 7 days. B Follow standard management principles for the treatment of AIH (recurrent or de novo) after liver transplantation. B 7. Specific circumstances Pediatric patients, diagnosis: As per HASL 2019 guidelines, obtain at least MRCP in pediatric patients with AIH to exclude autoimmune sclerosing cholangitis. B As per ESPGHAN 2018 guidelines, diagnose autoimmune liver disease in pediatric patients based on presence of serum autoantibodies, elevated IgG, compatible liver histology, positive family history for autoimmune conditions, and exclusion of other causes of pediatric chronic liver disease. E Show 5 more Pediatric patients, treatment: As per HASL 2019 guidelines, transit patients from childhood to adult care based on a multidisciplinary approach by special transition services. B As per ESPGHAN 2018 guidelines, initiate prednisolone/prednisone (2 mg/kg/day, up to 60 mg/day) as first-line therapy in pediatric patients with AIH, weaned down over 6-8 weeks to a maintenance dose of 5-7.5 mg/day, with monitoring of biochemical response (AST/ALT). Avoid using budenoside over the standard treatment as first-line therapy. E Show 8 more Pregnant patients: As per AASLD 2020 guidelines, include the goal of achieving biochemical remission of AIH for 1 year before conception in the family planning. E Show 5 more As per HASL 2019 guidelines, counsel female patients with AIH in remission that they have no contraindication for pregnancy or breastfeeding. B Show 3 more As per ACG 2016 guidelines, continue treatment with corticosteroids and/or azathioprine in pregnant patients with AIH. B https://web.pathway.md/diseases/recy694asCYrsgEzB 7/12 6/23/23, 3:16 AM Autoimmune hepatitis Pathway As per AISF 2016 guidelines, discuss treatment options before pregnancy in patients with AIH. Recognize that the dose of corticosteroids required to maintain remission will likely be lower if the patient is receiving corticosteroid monotherapy. Consider increasing the corticosteroid dose shortly before the expected date of delivery and monitoring liver enzymes and IgG closely in the weeks following delivery as pregnancy-related flare may occur. B Show 2 more As per EASL 2015 guidelines, recognize that controlled AIH is not a contraindication for pregnancy or breastfeeding. B Show 3 more Elderly patients: Initiate treatment in elderly patients with at least moderate necroinflammatory activity. Decide on the type of corticosteroid therapy and the tapering schedule carefully in all ages in the presence of other comorbidities. B Obtain close long-term follow-up in asymptomatic elderly patients with mild interface activity and comorbidities. B Patients with overlap syndromes: As per AASLD 2020 guidelines, consider adding UDCA to prednisone or prednisolone in combination with azathioprine in patients with AIH and overlap syndromes. E As per HASL 2019 guidelines: Initiate a combination of immunosuppression with UDCA in patients with AIH-PBC. Initiate immunosuppression if AIH is the dominant compartment and consider adding UDCA if remission is not achieved. B Consider adding UDCA to immunosuppressive treatment in patients with AIH-PSC. C As per EASL 2015 guidelines, initiate combined therapy with UDCA and immunosuppressants in patients with AIH and features of PBC. B Show 2 more Patients with viral hepatitis: As per AASLD 2020 guidelines: Obtain periodic serological testing (HBsAg, HBV DNA) during conventional therapy with prednisone or prednisolone in conjunction with azathioprine to detect HBV reactivation and the need for on-demand antiviral therapy in patients with AIH being HBsAg-negative/anti-HBcAg- positive during the diagnostic phase of their evaluation. E Consider initiating preemptive antiviral therapy in patients with serological evidence of previous HBV infection treated with high-dose corticosteroids or other immunomodulators, especially B-cell-depleting agents, because they at moderate risk for HBV reactivation. E As per HASL 2019 guidelines, do not screen for anti-liver kidney microsomal antibodies in patients with HCV before direct acting antivirals therapy. D Show 2 more Patients with HIV: individualize treatment in patients with AIH and human immunodeficiency virus, as standard immunosuppression seems to be safe and effective but carries a risk of life- https://web.pathway.md/diseases/recy694asCYrsgEzB 8/12 6/23/23, 3:16 AM Autoimmune hepatitis Pathway threatening infections. B Patients with fatty liver disease: ensure strict adherence to nonalcoholic fatty liver disease/NASH guidelines and use the lowest effective dose of cortiocosteroids for AIH in patients with nonalcoholic fatty liver disease/NASH, recognizing that the impact of nonalcoholic fatty liver disease/NASH on treatment outcome and response in AIH is unknown. B 8. Patient education General counseling: As per AASLD 2020 guidelines, identify potential barriers to long-term medication compliance and address at the start of treatment and monitor thereafter. E As per EASL 2015 guidelines, consider advising UV-protective measures in patients on immunosuppressants. C 9. Preventative measures Routine immunizations: As per AASLD 2020 guidelines: Provide routine immunizations in all susceptible patients with AIH according to the age-specific guidelines of the CDC. E Provide vaccination against HAV and HBV infections in unprotected patients, preferably before immunosuppressive therapy. E As per HASL 2019 guidelines, offer vaccination against hepatitis A, hepatitis B, pneumococcus (particularly in patients with cirrhosis) along with yearly influenza vaccination in all patients with AIH. B As per EASL 2015 guidelines: Provide hepatitis A and B immunization in all patients with AIH. B Provide influenza immunization on an annual basis in all patients with AIH. B Prevention of osteoporosis: As per AASLD 2020 guidelines, obtain bone mineral densitometry at baseline in all adult patients with AIH having risk factors for osteoporosis. Repeat bone mineral densitometry every 2-3 years of continuous corticosteroid treatment. E Show 3 more As per HASL 2019 guidelines, obtain bone density determination at treatment initiation in all patients receiving corticosteroids. Initiate vitamin D supplementation and advise adequate calcium intake. B As per EASL 2015 guidelines: Obtain bone density measurement at the initiation of corticosteroid therapy. B https://web.pathway.md/diseases/recy694asCYrsgEzB 9/12 6/23/23, 3:16 AM Autoimmune hepatitis Pathway Initiate vitamin D supplementation and ensure adequate calcium intake in all patients receiving corticosteroid therapy. B 10. Follow-up and surveillance Assessment of treatment response: As per AASLD 2020 guidelines, reevaluate the accuracy of the original diagnosis and medication adherence in patients with AIH not responding to first-line therapy. E As per EASL 2015 guidelines, reconsider the diagnosis of AIH and reevaluate treatment adherence in patients with failure of adequate response. B Management of incomplete response: As per HASL 2019 guidelines, increase azathioprine dose to 2 mg/kg/day in combination with 5- 10 mg/kg/day prednisolone in patients partially responding to conventional regimen. B Show 8 more As per EASL 2015 guidelines, consider replacing budesonide with prednisone or prednisolone (> 20 mg/day) in patients with incomplete response on budesonide-based regimen. C Show 3 more Monitoring for relapse: As per AASLD 2020 guidelines, obtain close monitoring for relapse with regular laboratory assessments during the first 12 months after treatment withdrawal and annually thereafter to cover for lifelong risk. E As per EASL 2015 guidelines, obtain close follow-up at 3-6-month intervals in patients with (spontaneous) remission. B Show 3 more Management of relapse: As per AASLD 2020 guidelines: Reinstitute original treatment in patients with a relapse until biochemical remission and subsequently switch to a long-term maintenance regimen. E Consider initiating mycophenolate mofetil or tacrolimus to achieve and maintain biochemical remission in patients with AIH with treatment failure, incomplete response, or drug intolerance to first-line agents. C As per HASL 2019 guidelines, recognize that IgG elevation usually precedes transaminase increase in patients with AIH relapse, but do not perform liver biopsy. B Show 2 more As per EASL 2015 guidelines: Consider administering corticosteroids in patients with a relapse or flare with doses similar to the induction regimen. Recognize that earlier detection of relapse allows lower doses of immunosuppressants to re-induce full remission. C https://web.pathway.md/diseases/recy694asCYrsgEzB 10/12 6/23/23, 3:16 AM Autoimmune hepatitis Pathway Keep patients on immunosuppression permanently if they have received adequate immunosuppression and have relapsed during drug withdrawal, or experienced a flare during adequate maintenance therapy. B Surveillance for malignancy: As per AASLD 2020 guidelines, obtain liver ultrasound, with or without serum AFP level, every 6 months for cancer surveillance in patients with cirrhosis, and adhere to standard guidelines for detection of extrahepatic malignancy. E As per HASL 2019 guidelines, obtain liver ultrasound every 6 months for early HCC detection in patients with AIH-related cirrhosis, as in cirrhosis of other etiologies. B As per EASL 2015 guidelines: Obtain liver ultrasound every 6 months for HCC screening in patients with AIH and cirrhosis. B Consider monitoring for non-melanoma skin cancer in patients on immunosuppressants. C References 1. Dalekos GN, Koskinas J, Papatheodoridis GV. Hellenic Association for the Study of the Liver Clinical Practice Guidelines: Autoimmune hepatitis. Ann Gastroenterol. 2019 Jan-Feb;32 1 1 23. Open 2. Cara L Mack, David Adams, David N Assis et al. Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance and Guidelines From the American Association for the Study of Liver Diseases. Hepatology. 2020 Aug;72 2 671 722. Open 3. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Autoimmune hepatitis. J Hepatol. 2015 Oct;63 4 971 1004. Open 4. Giorgina Mieli-Vergani, Diego Vergani, Ulrich Baumann et al. Diagnosis and Management of Pediatric Autoimmune Liver Disease: ESPGHAN Hepatology Committee Position Statement. J Pediatr Gastroenterol Nutr. 2018 Feb;66 2 345 360. Open 5. Tram T Tran, Joseph Ahn, Nancy S Reau. ACG Clinical Guideline: Liver Disease and Pregnancy. Am J Gastroenterol. 2016 Feb;111 2 176 94; quiz 196. Open 6. Lindor KD, Kowdley KV, Harrison ME. ACG Clinical Guideline: Primary Sclerosing Cholangitis. Am J Gastroenterol. 2015 May;110 5 646 59. Open 7. Italian Association for the Study of the Liver AISF , Italian Association for the Study of the Liver AISF. AISF position paper on liver disease and pregnancy. Dig Liver Dis. 2016 Feb;48 2 120 37. Open 8. Luigi Muratori, Paolo Muratori, Alessandro Granito et al. Current topics in autoimmune hepatitis. 2010 Nov;42 11 757 64.2010 Nov;42 11 757 64. Open 9. Dhruv Lowe, Savio John. Autoimmune hepatitis: Appraisal of current treatment guidelines. 2018 Dec 27;10 12 911 923.2018 Dec 27;10 12 911 923. Open 10. Albert J Czaja. Diagnosis and Management of Autoimmune Hepatitis: Current Status and Future Directions. 2016 Mar;10 2 177 203.2016 Mar;10 2 177 203. Open https://web.pathway.md/diseases/recy694asCYrsgEzB 11/12 6/23/23, 3:16 AM Autoimmune hepatitis Pathway 11. Dermot Gleeson. Long-Term Outcomes of Autoimmune Hepatitis. 2019 Aug 2;14 1 24 28.2019 Aug 2;14 1 24 28. Open 12. Elisabeth Sucher, Robert Sucher, Tanja Gradistanac et al. Autoimmune Hepatitis-Immunologically Triggered Liver Pathogenesis-Diagnostic and Therapeutic Strategies. J Immunol Res. 2019 Nov 25;2019 9437043. Open 13. Farhad Sahebjam, John M Vierling. Autoimmune hepatitis. Front Med. 2015 Jun;9 2 187 219. Open 14. Marcial Sebode, Johannes Hartl, Diego Vergani et al. Autoimmune hepatitis: From current knowledge and clinical practice to future research agenda. Liver Int. 2018 Jan;38 1 15 22. Open 15. Jiang Yi Zhu, Ying Han. Autoimmune hepatitis: Unveiling faces. J Dig Dis. 2015 Sep;16 9 483 8. Open 16. Hella Wobser, Tanja Paur, Elisabeth Schnoy et al. Suitability of the simplified autoimmune hepatitis score for the diagnosis of autoimmune hepatitis in a German cohort. United European Gastroenterol J. 2018 Mar;6 2 247 254. Open 17. Paul Martin, Andrea DiMartini, Sandy Feng et al. Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology. 2014 Mar;59 3 1144 65. Open 18. R G Knodell, K G Ishak, W C Black et al. Formulation and application of a numerical scoring system for assessing histological activity in asymptomatic chronic active hepatitis. Hepatology. Sep-Oct 1981;1 5 431 5. Open 19. Monika Sarkar, Carla W Brady, Jaquelyn Fleckenstein et al. Reproductive Health and Liver Disease: Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73 1 318 365. Open https://web.pathway.md/diseases/recy694asCYrsgEzB 12/12
Guideline sources The following summarized guidelines for the evaluation and management of autoimmune pancreatitis (AIP) are prepared by our editorial team based on guidelines from the European Pancreatic Club (EPC/IAP 2017). 1 2 3 4 5 Definition AIP is an IgG4 systemic disease characterized by obstructive jaundice, abdominal pain, and acute pancreatitis. 2 Epidemiology The exact cause of AIP is unknown; however, autoimmune cause (association with HLA haplotype) is postulated. 3 Disease course Th1 type of immune response is predominantly involved in the development of AIP, which presents as type 1 and type 2 subtypes with symptoms of obstructive jaundice, acute pancreatitis, abdominal pain, and IBD. Manifestations of other organ involvement include biliary disease, symptoms of Sjogren's disease (due to salivary gland involvement), lung nodules, interstitial nephritis, and retroperitoneal fibrosis. 4 Prognosis and risk of recurrence The risk of mortality increased with OR 2.07 (p = 0.02; 95% CI 1.07-3.55) in type 1 AIP. 5 https://web.pathway.md/diseases/recoLth7ikLnTajJ8 1/3 6/23/23, 3:16 AM Autoimmune pancreatitis Pathway Pathway Autoimmune pancreatitis Management Guidelines 1. Classification and risk stratification Predictors of relapse: Predictors of relapse include: remarkably high serum IgG4 levels such as > 4 times the ULN before treatment persistently high serum IgG4 levels after steroid treatment diffuse enlargement of the pancreas proximal type of IgG4-related sclerosing cholangitis extensive multi-organ involvement ( 2 other organs involved). B 2. Medical management Indications for treatment (symptomatic patients): treat patients with AIP who are symptomatic due to pancreatic involvement (e.g., obstructive jaundice, abdominal pain, back pain), or other organ involvement (e.g., jaundice due to bile duct stricture). B Indications for treatment (asymptomatic patients): Treat patients with AIP who are asymptomatic but have a persistent pancreatic mass on imaging. B Treat patients with AIP and associated IgG4-related sclerosing cholangitis who are asymptomatic but have persistent liver test abnormalities. B Induction of remission: Initiate corticosteroids as first-line therapy for active AIP in patients who do not have contraindications to their use. A Consider initiating rituximab as first-line therapy for active AIP in patients with contraindications to the use of corticosteroids. C Corticosteroid dosing: Initiate prednisone at a dose of 0.6-1.0 mg/kg/day. A Consider tapering prednisone by 5-10 mg/day every 1-2 weeks until a daily dosage of 20 mg, followed by tapering by 5 mg every 2 weeks. C Maintainance therapy (type 1 AIP): https://web.pathway.md/diseases/recoLth7ikLnTajJ8 2/3 6/23/23, 3:16 AM Autoimmune pancreatitis Pathway Consider maintenance therapy with low-dose corticosteroids or steroid-sparing agents in high- risk patients with type 1 AIP. C Avoid maintenance therapy in patients with type 1 AIP and low disease activity before treatment (focal involvement of the pancreas alone and rapid, complete radiological and biochemical remission in response to steroids). D Maintainance therapy (type 2 AIP): avoid maintenance therapy in patients with with type 2 AIP. D Management of relapse: Consider steroid and steroid-sparing agents (such as immune-modulators or rituximab) for treatment of relapsing AIP. C Consider surgical treatment in selected patients who are refractory to medical therapy. C 3. Specific circumstances Patients with obstructive jaundice: Consider performing biliary drainage in patients with obstructive jaundice in order to prevent biliary infection, obtain endoscopic brushings and cytology to differentiate IgG4-related sclerosing cholangitis from biliary malignancy. C Consider not performing biliary stenting, and attempting steroid treatment alone, in patients with mild jaundice and no signs of infection. D References 1. Okazaki K, Chari ST, Frulloni L et al. International consensus for the treatment of autoimmune pancreatitis. Pancreatology. 2017 Jan Feb;17 1 1 6. Open 2. Kamraan Madhani, James J Farrell. Autoimmune Pancreatitis: An Update on Diagnosis and Management. 2016 Mar;45 1 29 43.2016 Mar;45 1 29 43. Open 3. Iman Zandieh, Michael-F Byrne. Autoimmune pancreatitis: a review. 2007 Dec 21;13 47 6327 32.2007 Dec 21;13 47 6327 32. Open 4. Gyanprakash A Ketwaroo, Sunil Sheth. Autoimmune pancreatitis. 2013 Jul;1 1 27 32.2013 Jul;1 1 27 32. Open 5. Matthew T Huggett, E L Culver, M Kumar et al. Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort. 2014 Oct;109 10 1675 1683.2014 Oct;109 10 1675 1683. Open 6. Isabelle Scheers, Joseph J Palermo, Steven Freedman et al. Recommendations for Diagnosis and Management of Autoimmune Pancreatitis in Childhood: Consensus From INSPPIRE. J Pediatr Gastroenterol Nutr. 2018 Aug;67 2 232 236. Open https://web.pathway.md/diseases/recoLth7ikLnTajJ8 3/3
Guideline sources The following summarized guidelines for the evaluation and management of autosomal dominant polycystic kidney disease (ADPKD) are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2022), the United Kingdom Kidney Association (UKKA/BAPN 2019), the Ministry of Health, Labour and Welfare of Japan (MHLW/JSN 2016), and the Spanish Working Group on Inherited Kidney Diseases (SWG-IKD 2014). 1 2 3 4 Guidelines 1. Screening and diagnosis Indications for screening: As per UKKA 2019 guidelines, decide on screening for ADPKD in asymptomatic children and young individuals at risk of developing ADPKD jointly between health professionals and parents https://web.pathway.md/diseases/recH4dmZ6H9uUUD67 1/7 6/23/23, 3:16 AM Autosomal dominant polycystic kidney disease Pathway or carers and, wherever possible, the young person. B Show 4 more As per SWG-IKD 2014 guidelines, obtain ultrasound as the preferred screening tool for relatives of an affected proband. B Show 5 more 2. Diagnostic investigations Diagnostic imaging: obtain CT in uncertain cases or in patients with suspicion of associated renal disease, such as stones or tumor. B Show 3 more Genetic testing: provide genetic counseling in all patients with ADPKD. B Show 5 more Evaluation for cyst infection: Consider diagnosing cyst infection based on the association of: fever > 38 C local pain in the flank CRP > 5 mg/dL. C Show 3 more Screening for polycystic liver disease: obtain abdominal ultrasound to screen for polycystic liver disease in all patients diagnosed with ADPKD. B Screening for intracranial aneurysm: As per JSN 2016 guidelines: Recognize that: the prevalence of unruptured intracranial aneurysms is higher in patients with ADPKD than in the general population ICH, either cerebral or aneurysmal subarachnoid hemorrhage, confers high risks for mortality and morbidity in patients with ADPKD. B Screen patients with ADPKD for intracranial aneurysms to improve prognosis. B As per SWG-IKD 2014 guidelines, screen for intracranial aneurysms in any of the following situations: family or past personal history of stroke or intracranial aneurysm symptoms suggestive of intracranial aneurysms job or hobby in which loss of consciousness may be lethal preparation for major elective surgery extreme anxiety regarding the risk of having intracranial aneurysms. B Show 3 more Screening for valvular heart disease: consider obtaining TTE only in patients with heart murmur to evaluate the severity of valvular disease. C https://web.pathway.md/diseases/recH4dmZ6H9uUUD67 2/7 6/23/23, 3:16 AM Autosomal dominant polycystic kidney disease Pathway 3. Medical management General principles: assess cardiovascular risk and treat all modifiable cardiovascular risk factors according to the CKD guidelines. Show 2 more Tolvaptan: administer tolvaptan to slow the increase in total kidney volume and the decline in kidney function in patients with ADPKD with a relatively-good renal function with CrCl C60 mL/min by Cock-Croft equation and a total kidney volume 750 mL. B Antihypertensive therapy: As per JSN 2016 guidelines, initiate antihypertensive therapy to slow the deterioration of renal function and to improve the vital prognosis in patients with ADPKD complicated with hypertension. B As per SWG-IKD 2014 guidelines: Target clinic BP in patients with ADPKD similar to other patients with CKD. B Include RAAS inhibitors as the first option in the pharmacological regimens for hypertension. B Management of pain: obtain evaluation for the cause of chronic pain and correct it if possible. B Show 3 more Management of cyst bleeding: As per JSN 2016 guidelines, consider administering tranexamic acid if cystic hemorrhage does not improve by conservative treatment. C As per SWG-IKD 2014 guidelines, offer bed rest, analgesics and hydration to increase the urinary flow rate to 2-3 L/day for the treatment of patients with cyst bleeding. B Show 3 more Management of cyst infection: As per JSN 2016 guidelines, administer newer quinolones for the treatment of cyst infection in patients with ADPKD. B As per SWG-IKD 2014 guidelines, hospitalize patients with ADPKD with symptomatic cyst infection. B Show 4 more Management of kidney stones: Consider offering potassium citrate and urine alkalinization in patients with nephrolithiasis, if hypocitraturia is present. C Consider performing percutaneous nephrolithotomy and extracorporeal shock wave lithotripsy in an individualized setting. C 4. Nonpharmacologic interventions Lifestyle modifications: https://web.pathway.md/diseases/recH4dmZ6H9uUUD67 3/7 6/23/23, 3:16 AM Autosomal dominant polycystic kidney disease Pathway Encourage lifestyle changes (maintenance of ideal body weight, regular aerobic exercise and a diet limited to a maximum intake of 6 g of salt daily) to prevent and to treat hypertension as in patients with essential hypertension. B Advise patients with large polycystic kidneys to avoid contact sports and situations carrying a high risk of abdominal trauma. B Water intake: As per JSN 2016 guidelines, advise 2.5-4 L/day of water intake in patients with ADPKD. B As per SWG-IKD 2014 guidelines, advise high free water intake (2-3 L/day) in patients with stages 1-3 CKD. B Dietary protein restriction: insufficient evidence to recommend for or against dietary protein restriction for inhibiting progression of renal dysfunction in patients with ADPKD. I 5. Therapeutic procedures Cyst aspiration: Do not aspirate renal cysts to improve renal function in patients with ADPKD. D Consider aspirating renal cysts for the management of disease-related chronic pain or abdominal distention, for diagnostic purposes and the treatment of infected cysts. C Transarterial embolization: Perform renal transarterial embolization in patients with end-stage renal disease to reduce the size of enlarged kidneys. B Perform hepatic transarterial embolization in patients with end-stage renal disease to reduce hepatomegaly. B Dialysis: As per JSN 2016 guidelines: Perform peritoneal dialysis in patients with ADPKD. B Consider performing peritoneal dialysis for the improvement of the vital prognosis and quality of life in patients with ADPKD. C As per SWG-IKD 2014 guidelines: Perform peritoneal dialysis or hemodialysis in most patients with end-stage renal disease. B Avoid administering heparin during hemodialysis in patients with recurrent gross hematuria. D 6. Surgical interventions Nephrectomy: as per JSN 2016 guidelines, perform unilateral or bilateral nephrectomy if native kidney enlargement seems sufficiently massive to jeopardize accommodation of the donor kidney. B Nephrectomy (SWGIKD): https://web.pathway.md/diseases/recH4dmZ6H9uUUD67 4/7 6/23/23, 3:16 AM Autosomal dominant polycystic kidney disease Pathway Consider performing elective nephrectomy of native kidneys before renal transplantation if the kidney size prevents adequate placement of the graft. Take into account the experience of each center for timing (pretransplant or simultaneous with transplantation). C Consider performing native kidney nephrectomy for the treatment of complications, such as bleeding or persistent infection. C Kidney transplantation: as per JSN 2016 guidelines, consider performing solitary or simultaneous transplantation of the liver and the kidney for the improvement of the vital prognosis and quality of life in patients with autosomal dominant polycystic kidney. C Kidney transplantation (SWGIKD): perform transplantation as a RRT. Prefer preemptive renal transplantation from living donors. B 7. Specific circumstances Pregnant patients: counsel patients that pregnancy is not recommended in patients with ADPKD with stage 3-5 CKD, excluding transplanted patients. B Show 2 more Pediatric patients: insufficient evidence to recommend for or against screening for ADPKD in pediatric patients at risk due to ethical issues in the absence of a specific available treatment. Show 6 more Patients with cerebral aneurysm: As per JSN 2016 guidelines: Decide on treatment of a cerebral aneurysm by a comprehensive examination of factors such as location, shape, and size of the aneurysm, and general conditions, age, and medical history of the patient. B Consult with a neurosurgeon for decisions regarding treatment advisability and methods. B As per SWG-IKD 2014 guidelines, manage patients with unruptured intracranial aneurysms in collaboration with the neurosurgery department as follows: Situation Guidance Unruptured intracranial aneurysm 12 mm in diameter Treat Symptomatic unruptured intracranial aneurysm Enlarging unruptured intracranial aneurysm Unruptured intracranial aneurysm 7-12 mm in diameter and any of the following features Consider treating Unruptured intracranial aneurysm < 7 mm in diameter in anterior circulation without Do not treat https://web.pathway.md/diseases/recH4dmZ6H9uUUD67 5/7 6/23/23, 3:16 AM Autosomal dominant polycystic kidney disease Pathway family history of subarachnoid hemorrhage and without daughter sac Asymptomatic cavernous internal carotid aneurysms. B Show 2 more Patients with polycystic liver disease: advise avoiding estrogens and drugs stimulating cyclic adenosine monophosphate accumulation (such as caffeine) in patients with moderate-to-severe polycystic liver disease. B Show 6 more 8. Patient education General counseling: provide information on ADPKD inheritance and potential benefits and harms of testing for ADPKD in parents or carers of children at risk of developing ADPKD. B 9. Preventative measures Prevention of kidney stones: Insufficient evidence to recommend any prophylactic medical treatment for urolithiasis in patients with ADPKD. I Consider offering standard prophylactic treatment in patients with metabolic disorder. C 10. Follow-up and surveillance Blood pressure monitoring: obtain ambulatory or home BP monitoring for early diagnosis of hypertension. B Follow-up: schedule follow-up visits according to the CKD stage. B Show 3 more References 1. Shigeo Horie, Toshio Mochizuki, Satoru Muto et al. Evidence-based clinical practice guidelines for polycystic kidney disease 2014. Clin Exp Nephrol. 2016 Aug;20 4 493 509. Open 2. Elisabet Ars, Carmen Bernis, Gloria Fraga et al. Spanish guidelines for the management of autosomal dominant polycystic kidney disease. Nephrol Dial Transplant. 2014 Sep;29 Suppl 4:iv95 105. Open 3. European Association for the Study of the Live. EASL Clinical Practice Guidelines on the management of cystic liver diseases. EASL. 2022 Jun. Open https://web.pathway.md/diseases/recH4dmZ6H9uUUD67 6/7 6/23/23, 3:16 AM Autosomal dominant polycystic kidney disease Pathway 4. Jan Dudley, Paul Winyard, Matko Marlais et al. Clinical practice guideline monitoring children and young people with, or at risk of developing autosomal dominant polycystic kidney disease ADPKD . BMC Nephrol. 2019 Apr 30;20 1 148. Open 5. Japanese Society of Nephrology. Essential points from Evidence-based Clinical Practice Guidelines for Chronic Kidney Disease 2018. Clin Exp Nephrol. 2019 Jan;23 1 1 15. Open https://web.pathway.md/diseases/recH4dmZ6H9uUUD67 7/7
Guideline sources The following summarized guidelines for the evaluation and management of babesiosis are prepared by our editorial team based on guidelines from the Infectious Diseases Society of America (IDSA 2021). 1 Guidelines 1. Diagnostic investigations Laboratory testing: Obtain peripheral blood smear or PCR rather than antibody testing for diagnostic confirmation of acute babesiosis. A Obtain peripheral blood smear or PCR testing in patients with a positive Babesia antibody test to confirm the diagnosis of babesiosis before treatment is considered. A 2. Medical management Antimicrobial therapy: Offer one of the following combinations for the treatment of patients with babesiosis: atovaquone and azithromycin https://web.pathway.md/diseases/recoXt6OwIHzf8QuG 1/3 6/29/23, 3:16 AM Babesiosis Pathway q y clindamycin and quinine. A 3. Therapeutic procedures Red blood cell exchange transfusion: consider performing RBC exchange transfusion in selected patients with severe babesiosis. C 4. Follow-up and surveillance Follow-up: Monitor for Babesia parasitemia using peripheral blood smears in immunocompetent patients during treatment of acute illness. Do not test for parasitemia once symptoms have resolved. A Consider monitoring for Babesia parasitemia using peripheral blood smears in immunocompromised patients even after they become asymptomatic and until blood smears are negative. Consider PCR testing if blood smears have become negative but symptoms persist. C Clinical findings Symptoms Ocular exam Abdominal pain Conjunctival injection Arthralgia Integument exam Chills Cough Jaundice Dyspnea Petechiae Easy bruising Splinter hemorrhages Emotional lability Hematological findings Fatigue Fever DIC Headache Hemolytic anemia Loss of appetite blood leukocyte count Nausea blood platelet count Photophobia Sore throat Sweating Vomiting Weight loss Abdominal exam Hepatomegaly Splenomegaly https://web.pathway.md/diseases/recoXt6OwIHzf8QuG 2/3 6/29/23, 3:16 AM Babesiosis Pathway Lab findings serum ALT serum AST References 1. Peter J Krause, Paul G Auwaerter, Raveendhara R Bannuru et al. Clinical Practice Guidelines by the Infectious Diseases Society of America IDSA 2020 Guideline on Diagnosis and Management of Babesiosis. Clin Infect Dis. 2021 Jan 27;72 2):e49-e64. Open 2. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open https://web.pathway.md/diseases/recoXt6OwIHzf8QuG 3/3
Guideline sources The following summarized guidelines for the evaluation and management of bacterial vaginosis (BV) are prepared by our editorial team based on guidelines from the Center for Disease Control (CDC 2021), the American College of Obstetricians and Gynecologists (ACOG 2020), the U.S. Preventive Services Task Force (USPSTF 2020), the International Union Against Sexually Transmitted Infections (IUSTI/WHO 2018), and the Society of Obstetricians and Gynaecologists of Canada (SOGC 2017; 2015; 2013). 1 2 3 4 5 6 7 8 8 8 9 9 Definition BV, previously referred to as Gardnerella vaginitis, is a disease resulting from overgrowth of normal vaginal bacterial flora. 8 Epidemiology BV is idiopathic; however, sexual transmission has been implicated. It is associated with fluctuations in vaginal microbiota, with a relative increase in Gardnerella vaginalis, Atopobium vaginae, Prevotella species, and Mobiluncus species, in addition, to a decrease in lactobacilli. 8 Pathophysiology https://web.pathway.md/diseases/recjDNg52vuWBkD06 1/5 6/29/23, 3:16 AM Bacterial vaginosis Pathway In the United States, about 7.4 million cases of BV occur yearly. In nonpregnant women with symptomatic vaginal discharge, the overall prevalence of BV is 24.4% based on Nugent's scoring system. 9 Disease course BV is characterized by milky and malodorous vaginal discharge, resulting in vulvovaginal discomfort and irritation. It has a strong association with preterm birth, first-trimester miscarriage in women undergoing IVF, chorioamnionitis, amniotic-fluid infection, endometritis after childbirth or abortion, and infections after hysterectomy. 9 Prognosis and risk of recurrence BV has a high recurrence rate of up to 50% within a year of treatment. 8 Calculator Pathway Calculator Amsel's diagnostic criteria for b Bacterial vaginosis Nugent sc Diagnosis and management Guidelines 1. Screening and diagnosis Indications for screening: Do not obtain screening for BV in pregnant individuals not at increased risk for preterm delivery. D Insufficient evidence to recommend screening for or treating BV in the general pregnant population in order to prevent adverse outcomes, such as preterm birth. Show 2 more Obtain screening for BV at the first obstetric visit of patients with a history of cervical insufficiency. Treat patients with identified BV. A Diagnostic criteria: As per IUSTI 2018 guidelines, use the Hay-Ison criteria during microscopic evaluation for the diagnosis of BV. A As per SOGC 2015 guidelines, diagnose BV using either clinical (Amsel) or laboratory (Gram stain with objective scoring system) criteria. B 2. Medical management Antibiotic therapy: https://web.pathway.md/diseases/recjDNg52vuWBkD06 2/5 6/29/23, 3:16 AM Bacterial vaginosis Pathway As per CDC 2021 guidelines: Offer any of the following regimens as first-line therapy in patients with BV: metronidazole 500 mg PO BID for 7 days metronidazole gel 0.75% one full applicator (5 g) intravaginally, once daily for 5 days clindamycin cream 2% one full applicator (5 g) intravaginally at bedtime for 7 days. E Offer any of the following regimens as second-line therapy in patients with BV: clindamycin 300 mg PO BID for 7 days clindamycin ovules 100 mg intravaginally once at bedtime for 3 days secnidazole 2 g PO granules in a single dose tinidazole 2 g PO once daily for 2 days tinidazole 1 g PO once daily for 5 days. E As per IUSTI 2018 guidelines, offer topical or oral metronidazole for 5-7 days or intravaginal clindamycin for 7 days in patients with uncomplicated BV, depending on personal choice and circumstances. A As per SOGC 2015 guidelines, offer oral metronidazole 500 mg BID for 7 days in patients with symptomatic BV. Offer vaginal metronidazole gel and oral or vaginal clindamycin cream as alternatives. A Alternative therapies: as per SOGC 2015 guidelines, insufficient evidence to support the use of alternative therapies for the treatment of BV, such as probiotics and vitamin C. I Management of sexual partners: do not offer treatment for male sexual partners of patients diagnosed with BV. Offer treatment for female partners if they have BV. D 3. Specific circumstances Pregnant patients: As per IUSTI 2018 guidelines, offer clindamycin for the treatment of BV in pregnant patients. B As per SOGC 2017 guidelines, test for and treat BV for symptom resolution in symptomatic pregnant patients. A Show 4 more 4. Follow-up and surveillance Management of recurrent bacterial vaginosis: As per IUSTI 2018 guidelines, offer intravaginal metronidazole in patients with persistent or recurrent BV. B As per SOGC 2015 guidelines, offer longer courses of antibiotic therapy in patients with documented multiple recurrences of BV. A https://web.pathway.md/diseases/recjDNg52vuWBkD06 3/5 6/29/23, 3:16 AM Bacterial vaginosis Pathway Clinical findings Symptoms Past medical history Fishy vaginal odor Human immunodeficiency virus infection Foul-smelling vaginal discharge Sexually transmitted diseases Foul-smelling vaginal odor Vaginitis Natural lack of lactobacilli bacteria Social history No history of vaginal odor Vaginal discharge Multiple sexual partners Past events Lab findings Douching vaginal fluid pH gardnerella vaginalis deoxyRNA probe test Genitourinary exam vaginal sialidase test White or gray vaginal discharge on vaginal walls Histological findings Clue cells on wet mount Likelihood Ratios Pertinent positives The following findings increase the probability of bacterial vaginosis in adults. 10 12 13 Finding LR+ Value Presence of clue cells on wet mount* 3.4 No history of vaginal odor 2.2 (1.4-3.6) Increased vaginal fluid pH (> 4.5) 1.8 > 20% clue cells Pertinent negatives The following findings decrease the probability of bacterial vaginosis in adults. 10 12 13 Finding LR- Value History of vaginal odor 0.30 (0.24-0.38) vaginal fluid pH not increased ( 4.5) 0.47 Absence of clue cells on wet mount* 0.6 > 20% clue cells https://web.pathway.md/diseases/recjDNg52vuWBkD06 4/5 6/29/23, 3:16 AM Bacterial vaginosis Pathway References 1. van Schalkwyk J, Yudin MH, INFECTIOUS DISEASE COMMITTEE. Vulvovaginitis: screening for and management of trichomoniasis, vulvovaginal candidiasis, and bacterial vaginosis. J Obstet Gynaecol Can. 2015 Mar;37 3 266 274. Open 2. No authors listed. Vaginitis in Nonpregnant Patients: ACOG Practice Bulletin, Number 215. Obstet Gynecol. 2020 Jan;135 1):e1-e17. Open 3. US Preventive Services Task Force, Douglas K Owens, Karina W Davidson et al. Screening for Bacterial Vaginosis in Pregnant Persons to Prevent Preterm Delivery US Preventive Services Task Force Recommendation Statement. JAMA. 2020 Apr 7;323 13 1286 1292. Open 4. Sherrard J, Wilson J, Donders G et al. 2018 European IUSTI/WHO International Union against sexually transmitted infections IUSTI World Health Organisation WHO guideline on the management of vaginal discharge. Int J STD AIDS. 2018 Nov;29 13 1258 1272. Open 5. Mark H Yudin, Deborah M Money. No. 211 Screening and Management of Bacterial Vaginosis in Pregnancy. J Obstet Gynaecol Can. 2017 Aug;39 8):e184-e191. Open 6. Kimberly A Workowski, Laura H Bachmann, Philip A Chan et al. Sexually Transmitted Infections Treatment Guidelines, 2021. MMWR Recomm Rep. 2021 Jul 23;70 4 1 187. Open 7. Brown R, Gagnon R, Delisle MF. Cervical insufficiency and cervical cerclage. J Obstet Gynaecol Can. 2013 Dec;35 12 1115 1127. Open 8. Bagnall P, Rizzolo D. Bacterial vaginosis: A practical review. JAAPA. 2017 Dec;30 12 15 21. Open 9. Paavonen J, Brunham RC. Bacterial Vaginosis and Desquamative Inflammatory Vaginitis. N Engl J Med. 2018 Dec 6;379 23 2246 2254. Open 10. Lowe NK, Neal JL, Ryan-Wenger NA. Accuracy of the clinical diagnosis of vaginitis compared with a DNA probe laboratory standard. Obstet Gynecol. 2009 Jan;113 1 89 95. Open 11. R Hemalatha, Baru Anantha Ramalaxmi, Eluru Swetha et al. Evaluation of vaginal pH for detection of bacterial vaginosis. Indian J Med Res. 2013 Sep;138 3 354 9. Open 12. Bradshaw CS, Morton AN, Garland SM et al. Evaluation of a point-of-care test, BVBlue, and clinical and laboratory criteria for diagnosis of bacterial vaginosis. J Clin Microbiol. 2005 Mar;43 3 1304 8. Open 13. Bornstein J, Lakovsky Y, Lavi I et al. The classic approach to diagnosis of vulvovaginitis: a critical analysis. Infect Dis Obstet Gynecol. 2001;9 2 105 11. Open 14. Simel D, Rennie D. The Rational Clinical Examination: Evidence-Based Clinical Diagnosis. Mayo Clin Proc. 2009 Nov; 84 11 1045. Open 15. US Preventive Services Task Force, Bibbins-Domingo K, Grossman DC et al. Screening USPSTF for Gynecologic Conditions With Pelvic Examination US Preventive Services Task Force Recommendation Statement. JAMA. 2017;317 9 947 953. Open 16. Suzanne Reiter, Susan Kellogg Spadt. Bacterial vaginosis: a primer for clinicians. Postgrad Med. 2019 Jan;131 1 8 18. Open 17. R P Nugent, M A Krohn, S L Hillier. Reliability of diagnosing bacterial vaginosis is improved by a standardized method of gram stain interpretation. J Clin Microbiol. 1991 Feb;29 2 297 301. Open https://web.pathway.md/diseases/recjDNg52vuWBkD06 5/5
Guideline sources The following summarized guidelines for the evaluation and management of Barrett's esophagus (BE) are prepared by our editorial team based on guidelines from the United European Gastroenterology (UEG/ESPEN 2023), the American Gastroenterological Association (AGA 2022; 2012), the American College of Gastroenterology (ACG 2022; 2016; 2013), the American Society for Gastrointestinal Endoscopy (ASGE 2019; 2018), the British Society of Gastroenterology (BSG 2018), and the European Society of Gastrointestinal Endoscopy (ESGE 2017). 1 2 3 4 5 6 7 8 9 10 11 11 11 11 12 Definition BE is a pre-neoplastic condition characterized by intestinal metaplasia of the esophageal squamous mucosa. 11 Epidemiology In patients with BE, cellular damage to the esophageal mucosa is caused by chronic acid-induced mucosal injury associated with GERD. 11 Pathophysiology https://web.pathway.md/diseases/recHRXYnA7BRzX4gP 1/10 6/29/23, 3:16 AM Barrett's esophagus Pathway Pathophysiology In the United States, the prevalence of BE is estimated at 5600 persons per 100,000 population. 12 Disease course Chronic inflammation leads to activation of oncogenes and silencing of tumor suppressor genes, increasing the risk of esophageal adenocarcinoma and esophageal SCC. 11 Prognosis and risk of recurrence The rate of progression of high-grade dysplasia in patients with BE is approximately 6% per year. 11 Calculator Diagnostic criteria for Barrett's e Guidelines 1. Screening and diagnosis Indications for screening, general population: As per ASGE 2019 guidelines, insufficient evidence to recommend screening for BE. I As per ESGE 2017 guidelines, do not obtain endoscopic screening for BE in general population. D As per ACG 2016 guidelines, do not obtain screening for BE in the general population. D Indications for screening, high risk patients: As per ACG 2022 guidelines, consider performing a single screening endoscopy in patients with chronic GERD symptoms and 3 additional risk factors for BE, including: male gender age > 50 year white race tobacco smoking obesity family history of BE or esophageal adenocarcinoma in a first-degree relative. C Show 2 more As per AGA 2022 guidelines, consider obtaining endoscopic screening for BE in patients with 3 of the following risk factors for BE and esophageal adenocarcinoma: male gender https://web.pathway.md/diseases/recHRXYnA7BRzX4gP 2/10 6/29/23, 3:16 AM Barrett's esophagus Pathway non-Hispanic white race age > 50 years history of smoking chronic GERD obesity family history of BE or esophageal adenocarcinoma. C As per ASGE 2019 guidelines, consider adopting a screening strategy for BE, if performed, identifying at-risk population - individuals with a family history of esophageal adenocarcinoma or BE (high risk) or patients with GERD plus at least 1 other risk factor (moderate risk). C As per ESGE 2017 guidelines, consider obtaining endoscopic screening for BE in patients with longstanding GERD (> 5 years) and multiple risk factors, including: male gender age 50 years white race obesity first-degree relative with BE or esophageal adenocarcinoma. C Indications for screening (before bariatric surgery): consider performing upper gastrointestinal endoscopy as a routine diagnostic test before bariatric surgery to rule out Barret's esophagus or esophageal and gastric malignancies. C Technical considerations for screening: consider using nonendoscopic cell-collection devices as an option to screen for BE. C Show 5 more Diagnostic criteria: As per ACG 2022 guidelines: Consider diagnosing BE in the presence of intestinal metaplasia in the tubular esophagus. C Consider diagnosing BE in the presence of columnar mucosa of at least 1 cm in length: in patients with a normal-appearing Z line; do not perform routine endoscopic biopsies in patients with a Z line demonstrating < 1 cm of proximal displacement from the top of the gastric folds in the absence of any visible lesions; do not perform routine endoscopic biopsies. C As per ESGE 2017 guidelines, diagnose BE if the distal esophagus is lined with columnar epithelium with a minimum length of 1 cm (tongues or circular) containing specialized intestinal metaplasia at histopathological examination. B 2. Diagnostic investigations Biomarkers: do not use additional biomarkers for risk stratification of patients with BE. D https://web.pathway.md/diseases/recHRXYnA7BRzX4gP 3/10 6/29/23, 3:16 AM Barrett's esophagus Pathway 3. Diagnostic procedures Upper gastrointestinal endoscopy: As per ACG 2022 guidelines: Consider taking at least 8 endoscopic biopsies in screening examinations with endoscopic findings consistent with possible BE, with the Seattle protocol followed for segments of > 4 cm. C Confirm dysplasia of any grade detected on biopsies of BE by a second pathologist with expertise in gastrointestinal pathology. B As per ASGE 2018 guidelines, consider confirming the diagnosis of BE in patients with low- and high-grade dysplasia being candidates for endoscopic eradication therapy by at least one expert gastrointestinal pathologist or panel of pathologists. C As per ESGE 2017 guidelines, include the following in the endoscopy reports of patients with BE: the extent of BE using the Prague criteria (circumferential extent, C; maximum extent, M) and any separate islands proximal to the maximal extent a description of location (in cm from the incisors and clockwise orientation) of any visible abnormality within the Barrett's epithelium, in addition to lesion size (mm) and macroscopic appearance using the Paris classification the presence or absence of erosive esophagitis using the Los Angeles classification the location of biopsies taken from the Barrett's segment (number of biopsies and location in cm from the incisors) appropriate photo documentation of the landmarks and of all visible Barrett's epithelium and any visible lesions. B Show 2 more As per ACG 2016 guidelines: Document the extent of metaplastic change including circumferential and maximal segment length using the Prague classification. B Document the location of the diaphragmatic hiatus, GEJ, and squamocolumnar junction in the endoscopy report. B Endoscopic ultrasound: As per AGA 2022 guidelines, perform biopsy in patients with erosive esophagitis if concerns of dysplasia or malignancy exist. B Show 3 more As per ACG 2016 guidelines: Insufficient evidence to recommend routine staging of patients with nodular BE with EUS or other imaging modalities before endoscopic mucosal resection. Do not deter from performing endoscopic mucosal resection to stage early neoplasia solely based on EUS findings, given the possibility of over-staging and under-staging. I https://web.pathway.md/diseases/recHRXYnA7BRzX4gP 4/10 6/29/23, 3:16 AM Barrett's esophagus Pathway Consider obtaining EUS to assess and sample regional lymph nodes in patients with known T1b disease, given the increased prevalence of lymph node involvement in these patients compared with less advanced disease. B Transnasal endoscopy: consider performing unsedated transnasal endoscopy as an alternative to conventional upper endoscopy for BE screening. B 4. Medical management Proton pump inhibitors: As per ACG 2022 guidelines: Consider initiating at least once daily PPI therapy in patients with BE without allergy or other contraindication to PPI use. C Insufficient evidence to support the use of combination therapy with aspirin and PPIs in patients with BE. I As per AGA 2022 guidelines, initiate daily PPI therapy in all patients with BE. B As per ACG 2016 guidelines: Initiate once-daily PPI therapy in patients with BE. Do not use twice-daily dosing routinely unless required because of poor control of reflux symptoms or esophagitis. B Set the goal of medical antireflux therapy following complete eradication of intestinal metaplasia to control reflux, as determined by the absence of frequent reflux symptoms (> 1 per week) and/or esophagitis on endoscopic examination. B As per ACG 2013 guidelines, treat symptoms in patients with BE in a similar fashion to patients with GERD not having BE. B Nonsteroidal anti-inflammatory drugs: avoid prescribing aspirin or NSAIDs routinely as an antineoplastic strategy in patients with BE. D Management of esophageal adenocarcinoma: As per ESGE 2017 guidelines: Perform endoscopic resection as the first-choice therapy in patients with T1a esophageal adenocarcinoma. B Decide on the optimal treatment strategy depending on histopathological characteristics of the endoscopic resection specimen in patients with T1b esophageal adenocarcinoma. Consider performing endoscopic resection as a valid alternative to surgery in patients at borderline fit for surgery, if the endoscopic resection specimen meets all of the following criteria: Situation Guidance Limited to < 500 mcm Submucosal invasion Well or moderate Tumor differentiation grade Absent https://web.pathway.md/diseases/recHRXYnA7BRzX4gP 5/10 6/29/23, 3:16 AM Barrett's esophagus Pathway Tumor invasion in lymphatic vessels or blood vessels Absent. B Tumor infiltration in the deep resection margin As per ACG 2016 guidelines, perform esophagectomy, with consideration of neoadjuvant therapy, in patients with esophageal adenocarcinoma with invasion into the submucosa, especially if invasion to the mid- or deep submucosa (T1b, sm2-3) is present. B 5. Therapeutic procedures Endoscopic therapy: As per ACG 2022 guidelines, counsel patients with BE embarking on endoscopic eradication therapy to ensure a clear understanding of the risks and benefits associated with these therapies before the initiation of therapy. Show 5 more As per ASGE 2018 guidelines, consider preferring endoscopic eradication therapy over surveillance in patients with low-grade dysplasia. C Show 5 more As per ESGE 2017 guidelines, do not perform prophylactic endoscopic therapy (such as ablation therapy) for non-neoplastic BE. D Show 3 more 6. Surgical interventions Antireflux surgery: avoid performing antireflux surgery as an antineoplastic measure in patients with BE. D 7. Follow-up and surveillance Indications for referral: As per AGA 2022 guidelines, refer patients with BE-related neoplasia to endoscopists with expertise in advanced imaging, resection, and ablation. B As per ESGE 2017 guidelines: Refer all patients with a BE 10 cm, a confirmed diagnosis of low-grade dysplasia, high-grade dysplasia or early cancer to a BE expert center for surveillance and/or treatment. B Ensure that a BE expert center meets the following requirements: annual case load of 10 new patients with endoscopic treatment for high-grade dysplasia or early carcinoma per BE expert endoscopist https://web.pathway.md/diseases/recHRXYnA7BRzX4gP 6/10 6/29/23, 3:16 AM Barrett's esophagus Pathway endoscopic and histological care is provided by endoscopists and pathologists participated additional training in this field (either by courses or guest visits) and, a minimum of 30 supervised cases of endoscopic resection and 30 cases of endoscopic ablation should be performed to acquire competence in technical skills, management pathways, and complications patients with Barrett's neoplasia are discussed in multidisciplinary meetings with gastroenterologists, surgeons, oncologists and pathologists access to experienced esophageal surgery all patients with BE are registered prospectively in a database. B Surveillance endoscopy, general principles: As per ACG 2022 guidelines, perform both white light endoscopy and chromoendoscopy in patients undergoing endoscopic surveillance of BE. B Show 6 more As per ASGE 2019 guidelines, perform chromoendoscopy, including virtual chromoendoscopy and Seattle protocol biopsy sampling, in patients with BE undergoing surveillance. B Show 3 more As per ESGE 2017 guidelines, obtain endoscopic surveillance in patients with BE. B Show 4 more As per AGA 2012 guidelines, do not obtain surveillance examination within 3 years in patients with BE with a second endoscopy confirming the absence of dysplasia on biopsy. D Surveillance endoscopy, patients without dysplasia: As per AGA 2022 guidelines, perform surveillance endoscopy in 3-5 years in patients with nondysplastic BE. B As per ASGE 2019 guidelines, consider performing surveillance endoscopy in patients with nondysplastic BE. C As per ACG 2016 guidelines: Consider performing repeat endoscopy at 1-2 years to rule out BE in patients with suspected BE and lack of intestinal metaplasia on histology. C Do not perform repeat endoscopy if the initial endoscopy is negative for BE. Perform repeat endoscopy after PPI therapy for 8-12 weeks if endoscopy reveals esophagitis (Los Angeles Classification B, C, or D), to ensure healing of esophagitis and exclude the presence of underlying BE. D Surveillance endoscopy, patients with dysplasia: As per ASGE 2019 guidelines, do not obtain routine EUS to differentiate mucosal versus submucosal disease in patients with BE with high-grade dysplasia/intramucosal cancer or nodules. D As per ASGE 2018 guidelines, consider obtaining surveillance over endoscopic eradication therapy in patients with low-grade dysplasia placing a high value on avoiding adverse events related to endoscopic eradication therapy. C https://web.pathway.md/diseases/recHRXYnA7BRzX4gP 7/10 6/29/23, 3:16 AM Barrett's esophagus Pathway As per BSG 2018 guidelines: Perform a repeat endoscopy at 6 months in patients with low-grade dysplasia. B Offer endoscopic ablation therapy, preferably with radiofrequency ablation and after a review by a specialist multidisciplinary team, in patients with low-grade dysplasia in any of the follow- up endoscopy (confirmed by an expert gastrointestinal pathologist in at least 2 sets of biopsies). Offer 6-monthly surveillance if ablation is not performed. A As per ESGE 2017 guidelines, optimize antireflux therapy and perform repeated endoscopy at 6 months in patients with a diagnosis of "indefinite for dysplasia" confirmed by a second expert gastrointestinal pathologist. Obtain surveillance as in patients with nondysplastic BE if no definite dysplasia is found in subsequent biopsy samples (including if the biopsies are again classified as "indefinite for dysplasia"). B Show 2 more As per ACG 2016 guidelines, obtain endoscopic surveillance at intervals of 3-5 years in patients with BE without dysplasia. B Show 2 more Surveillance endoscopy, post-treatment: As per ACG 2022 guidelines, obtain endoscopic surveillance in patients with BE successfully completed endoscopic eradication therapy. B As per AGA 2022 guidelines: Perform repeat endoscopy after 8 weeks of BID PPI therapy. B Take random biopsies of the esophagogastric junction, gastric cardia, and the distal 2 cm of the neosquamous epithelium and from all visible lesions, independent of the length of the original BE segment, in patients undergoing surveillance after endoscopic eradication therapy. B As per ASGE 2018 guidelines, consider performing surveillance endoscopy in patients with dysplasia and intramural cancer achieved complete eradication of intestinal metaplasia after endoscopic eradication therapy. C As per ACG 2016 guidelines, continue endoscopic surveillance to detect recurrent intestinal metaplasia and/or dysplasia following successful endoscopic therapy and complete eradication of intestinal metaplasia. B Show 3 more Management of recurrent metaplasia or dysplasia: treat recurrent metaplasia and/or dysplasia according to the same guidelines as for the treatment of metaplasia/dysplasia prior to ablation. B Clinical findings Patient demographics Symptoms Age > 50 Chest pain Male sex Dysphagia https://web.pathway.md/diseases/recHRXYnA7BRzX4gP 8/10 6/29/23, 3:16 AM Barrett's esophagus Pathway White race Food regurgitation Heartburn Past medical history Weight loss GERD Family history Obesity Genetic predispositions Social history Lab findings Current cigarette smoking Iron deficiency Histological findings Dysplasia in the distal esophagus Intestinal metaplasia in the distal esophagus References 1. V Raman Muthusamy, Sachin Wani, C Prakash Gyawali et al. AGA Clinical Practice Update on New Technology and Innovation for Surveillance and Screening in Barrett's Esophagus: Expert Review. Clin Gastroenterol Hepatol. 2022 Dec;20 12 2696 2706.e1. Open 2. ASGE STANDARDS OF PRACTICE COMMITTEE, Bashar Qumseya, Shahnaz Sultan et al. ASGE guideline on screening and surveillance of Barrett's esophagus. Gastrointest Endosc. 2019 Sep;90 3 335 359.e2. Open 3. Shaheen NJ, Falk GW, Iyer PG et al. ACG Clinical Guideline: Diagnosis and Management of Barrett's Esophagus. Am J Gastroenterol. 2016 Jan;111 1 30 50. Open 4. Bas Weusten, Raf Bisschops, Emanuel Coron et al. Endoscopic management of Barrett's esophagus: European Society of Gastrointestinal Endoscopy ESGE Position Statement. Endoscopy. 2017 Feb;49 2 191 198. Open 5. Standards of Practice Committee, Sachin Wani, Bashar Qumseya et al. Endoscopic eradication therapy for patients with Barrett's esophagus-associated dysplasia and intramucosal cancer. Gastrointest Endosc. 2018 Apr;87 4 907 931.e9. Open 6. Massimiliano di Pietro, Rebecca C Fitzgerald, BSG Barrett's guidelines working group. Revised British Society of Gastroenterology recommendation on the diagnosis and management of Barrett's oesophagus with low-grade dysplasia. Gut. 2018 Feb;67 2 392 393. Open 7. Nicholas J Shaheen, Gary W Falk, Prasad G Iyer et al. Diagnosis and Management of Barrett's Esophagus: An Updated ACG Guideline. Am J Gastroenterol. 2022 Apr 1;117 4 559 587. Open 8. Katz PO, Gerson LB, Vela MF. Guidelines for the diagnosis and management of gastroesophageal reflux disease. Am J Gastroenterol. 2013 Mar;108 3 308 28. Open 9. American Gastroenterological Association. Choosing Wisely: Recommendations of the American Gastroenterological Association. Choosing Wisely. 2012 Apr. Open https://web.pathway.md/diseases/recHRXYnA7BRzX4gP 9/10 6/29/23, 3:16 AM Barrett's esophagus Pathway 10. Stephan C Bischoff, Johann Ockenga, Ahad Eshraghian et al. Practical guideline on obesity care in patients with gastrointestinal and liver diseases Joint ESPEN/UEG guideline. Clin Nutr. 2023 Apr 10;42 6 987 1024. Open 11. Spechler SJ, Souza RF. Barrett's esophagus. N Engl J Med. 2014 Aug 28;371 9 836 45. Open 12. Kuipers EJ. Barrett esophagus and life expectancy: implications for screening?. Gastroenterol Hepatol N Y . 2011 Oct;7 10 689 91. Open 13. American Gastroenterological Association. Choosing Wisely AGA recommendations. Choosing Wisely. 2012. Open 14. Laura E Targownik, Deborah A Fisher, Sameer D Saini. AGA Clinical Practice Update on De-Prescribing of Proton Pump Inhibitors: Expert Review. Gastroenterology. 2022 Feb 16;S0016 5085 21 04083 X. Open 15. Barbara Farrell, Kevin Pottie, Wade Thompson et al. Deprescribing proton pump inhibitors: Evidence- based clinical practice guideline. Can Fam Physician. 2017 May;63 5 354 364. Open 16. Roos E Pouw, Maximilien Barret, Katharina Biermann et al. Endoscopic tissue sampling Part 1 Upper gastrointestinal and hepatopancreatobiliary tracts. European Society of Gastrointestinal Endoscopy ESGE Guideline. Endoscopy. 2021 Nov;53 11 1174 1188. Open https://web.pathway.md/diseases/recHRXYnA7BRzX4gP 10/10
Guideline sources The following summarized guidelines for the evaluation and management of basal cell carcinoma (BCC) are prepared by our editorial team based on guidelines from the U.S. Preventive Services Task Force (USPSTF 2023; 2018), the American Society of Plastic Surgeons (ASPS 2021), the British Association of Dermatologists (BAD 2021), the American Society for Radiation Oncology (ASTRO 2020), the European Association of Dermato-Oncology (EADO/EDF/EORTC 2019), and the American Academy of Dermatology (AAD 2018). 1 2 3 4 5 6 7 Calculator Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s TNM class https://web.pathway.md/diseases/recwv4t9NOosmhAmc 1/8 6/29/23, 3:16 AM Basal cell carcinoma Pathway Guidelines 1. Screening and diagnosis Indications for screening: As per USPSTF 2023 guidelines, insufficient evidence to assess the balance of benefits and harms of visual skin examination by a clinician to screen for skin cancer in adolescents and adults. I As per USPSTF 2018 guidelines, insufficient evidence to assess the balance of benefits and harms of counseling adult persons about skin self-examination to prevent skin cancer. I Diagnosis: Confirm the diagnosis of BCC histopathologically in patients with ambiguous lesions, large tumors and tumors located in high-risk areas. B Avoid obtaining histological confirmation in patients with superficial and small nodular (< 1 cm) BCCs in low-risk areas, if clearly diagnosed clinically and/or with noninvasive techniques. D 2. Diagnostic investigations Noninvasive evaluation: consider obtaining dermatoscopy, reflectance confocal microscopy and/or optical coherence tomography to improve the diagnostic accuracy in patients with difficult- to-recognize BCCs. B 3. Diagnostic procedures Biopsy: perform a punch, shave, or excisional biopsy, depending on the characteristics of the suspected malignancy (including morphology and location) and the physician's judgment, for the diagnosis of BCC. B Show 4 more 4. Medical management General principles: As per BAD 2021 guidelines, refer all adult patients with high-risk BCC and all adult patients with low-risk BCC to a local skin multidisciplinary team or a specialized skin cancer multidisciplinary team member in the absence of an accredited general practitioner with an enhanced role or if the primary care facility is not suitable for surgery. A As per EDF 2019 guidelines: Decide on potential suitability, indication and technique for difficult-to-treat BCC in a multidisciplinary team. B https://web.pathway.md/diseases/recwv4t9NOosmhAmc 2/8 6/29/23, 3:16 AM Basal cell carcinoma Pathway Avoid using topical or destructive (blind) treatments in patients with BCC at risk of recurrences. D As per AAD 2018 guidelines, provide a treatment plan taking into account recurrence rate, preservation of function, patient expectations, and potential adverse effects. B Topical agents: As per BAD 2021 guidelines, offer topical imiquimod and 5-fluorouracil as treatment options in adult patients with low-risk BCC unsuitable for or declining standard surgical excision. A Show 2 more As per EDF 2019 guidelines, offer topical imiquimod (5%) in patients with primary superficial BCC. B Show 2 more As per AAD 2018 guidelines, consider offering topical therapy with imiquimod B or 5- fluorouracil C in patients with low-risk tumors if surgical therapy is not feasible or preferred. Adjust topical therapy dosing regimen based on side effect tolerance. B Hedgehog pathway inhibitors: As per BAD 2021 guidelines, offer vismodegib as a treatment option in adult patients with advanced BCC unsuitable for Mohs micrographic surgery, standard surgical excision, or radiotherapy, including patients with Gorlin syndrome, following discussion at a multidisciplinary team. A As per EDF 2019 guidelines, offer Hedgehog pathway inhibitors in patients with locally advanced or metastatic BCC. B As per AAD 2018 guidelines: Offer multidisciplinary consultation and smoothened inhibitors in patients with metastatic BCC. B Consider offering systemic therapy with a smoothened inhibitor if surgery and radiation therapy are contraindicated or inappropriate for the treatment of locally advanced BCC, or if residual tumor persists following surgery and/or radiotherapy and further surgery and radiotherapy are contraindicated or inappropriate. B Chemotherapy: As per EDF 2019 guidelines, consider offering chemotherapy in patients with BCC if Hedgehog pathway inhibitors are contraindicated and no clinical trials are available. C As per AAD 2018 guidelines: Offer platinum-based chemotherapy or best supportive care if treatment of metastatic BCC with smoothened inhibitors is not feasible. B Provide with or refer for best supportive and palliative care to optimize symptom management and maximize the quality of life in patients with advanced disease. B Combination therapy: Insufficient evidence to recommend any of the following combinations in patients with BCC: topical diclofenac and calcitriol https://web.pathway.md/diseases/recwv4t9NOosmhAmc 3/8 6/29/23, 3:16 AM Basal cell carcinoma Pathway topical imiquimod and Mohs micrographic surgery intralesional interferon-alpha and standard surgical excision topical photodynamic therapy and Mohs micrographic surgery laser therapy and topical photodynamic therapy. I 5. Therapeutic procedures Curettage and electrodessication: As per BAD 2021 guidelines, do not perform curettage and cautery in adult patients with high- risk BCC unsuitable for or declining Mohs micrographic surgery or standard surgical excision. D As per EDF 2019 guidelines, consider performing curettage and electrodessication as an alternative treatment in patients with small, low-risk BCC on the trunk and extremities. C As per AAD 2018 guidelines, consider performing curettage and electrodessication in patients with low-risk tumors in non-terminal hair-bearing locations. C Cryotherapy: As per BAD 2021 guidelines, offer cryosurgery as a treatment option in adult patients with low- risk BCC unsuitable for or declining standard surgical excision. A Show 2 more As per EDF 2019 guidelines, consider offering cryotherapy as an alternative treatment in patients with small, low-risk BCC on the trunk and extremities. C As per AAD 2018 guidelines, consider offering cryosurgery in patients with low-risk BCC when more effective therapies are contraindicated or impractical. B Definitive radiotherapy, indications: As per BAD 2021 guidelines, offer radiotherapy as a treatment option in adult patients (suggested age 60 years) with low-risk and high-risk BCC unsuitable for or declining Mohs micrographic surgery or standard surgical excision and expressing a preference for radiotherapy, and if the lesion is: a nodular BCC an infiltrative subtype of BCC provided a sufficient planning margin is used an excised BCC with involved margins. A Show 3 more As per ASTRO 2020 guidelines, offer definitive radiotherapy as a curative treatment modality in patients with BCC unable to undergo or declining surgical resection. B Show 4 more As per EORTC/EADO/EDF 2019 guidelines, offer radiotherapy as an alternative to surgery for BCC on the face, including periorbital regions and other anatomical regions, in elderly patients and in patients not suitable for surgery. A As per AAD 2018 guidelines: https://web.pathway.md/diseases/recwv4t9NOosmhAmc 4/8 6/29/23, 3:16 AM Basal cell carcinoma Pathway Consider offering radiotherapy (such as superficial radiation therapy, brachytherapy, external electron beam, or other traditional radiotherapy forms for BCC) in patients with low-risk tumors if surgical therapy is not feasible or preferred. C Insufficient evidence to recommend the routine use of electronic surface brachytherapy in patients with BCC. I Definitive radiotherapy (dosing): use conventional fractionation (180-200 cGy/fraction) for a total of 6,000-6,600 cGy for adjuvant radiotherapy after therapeutic lymphadenectomy. B Show 2 more Postoperative radiotherapy: offer postoperative radiotherapy in patients with clinically or radiologically apparent gross perineural spread. B Show 3 more Photodynamic therapy: As per BAD 2021 guidelines, offer topical photodynamic therapy as a treatment option in adult patients with low-risk BCC unsuitable for or declining standard surgical excision. A Show 2 more As per EDF 2019 guidelines, offer 5-ALA or methyl-5-amino-4-oxopentanoate in combination with red light in patients with superficial and thin nodular BCC. A As per AAD 2018 guidelines, consider offering methyl aminolevulinate- or ALA-photodynamic therapy in patients with low-risk tumors if surgical therapy is not feasible or preferred. B Laser therapy: As per BAD 2021 guidelines, insufficient evidence to recommend CO or pulsed-dye laser therapy in patients with BCC. I As per EDF 2019 guidelines, do not offer laser ablation in patients with BCC. D As per AAD 2018 guidelines, insufficient evidence to recommend laser therapy in patients with BCC. I 6. Surgical interventions Surgical excision: As per BAD 2021 guidelines, perform standard surgical excision as a first-line treatment option in adult patients with low-risk BCC. A Show 12 more As per EDF 2019 guidelines, perform surgical removal for the treatment and histological confirmation in patients with BCC. B Show 5 more As per AAD 2018 guidelines, perform surgical excision with 4-mm clinical margins and histologic margin assessment in patients with low-risk primary BCC. A Show 2 more https://web.pathway.md/diseases/recwv4t9NOosmhAmc 5/8 6/29/23, 3:16 AM Basal cell carcinoma Pathway 7. Specific circumstances Patients with nevoid basal cell carcinoma syndrome: As per BAD 2021 guidelines, offer vismodegib as a treatment option in adult patients with advanced BCC unsuitable for Mohs micrographic surgery, standard surgical excision or radiotherapy, including patients with nevoid BCC syndrome, following discussion at a multidisciplinary team. A As per EDF 2019 guidelines, establish the diagnosis of nevoid BCC syndrome based on clinical criteria. Consider offering genetic testing for germline mutations in the Hedgehog pathway in selected patients. B Show 2 more 8. Patient education General counseling: Provide verbal and written information about BCC in all adult patients with BCC, including the nature and prognosis of BCC, available treatment options and the ongoing need for sun protection and self-surveillance of their skin as part of prevention or early detection of future skin tumors. A Inform all adult patients with BCC declining all treatments that the risk of significant progression of the tumor is at least 25% over 2-5 years. A 9. Preventative measures Primary prevention: Counsel persons with fair skin types aged 6 months to 24 years and parents of young children about minimizing exposure to UV radiation to reduce the risk of skin cancer. B Offer counseling selectively in adults > 24 years with fair skin types about minimizing exposure to UV radiation to reduce the risk of skin cancer, taking into consideration the presence of risk factors for skin cancer. B Secondary prevention: counsel patients with a history of BCC on skin self-examination and sun protection. B Show 3 more 10. Follow-up and surveillance Postoperative evaluation: Do not offer routine follow-up in patients with adequately treated isolated BCC, unless for a postoperative review. D Offer a postoperative review in adult patients with adequately treated BCC by an appropriate healthcare professional, in either secondary or primary care, if possible. E https://web.pathway.md/diseases/recwv4t9NOosmhAmc 6/8 6/29/23, 3:16 AM Basal cell carcinoma Pathway Skin reconstruction after tumor resection (timing): consider performing reconstructive surgery in a delayed (asynchronous) fashion in adult patients after skin cancer resection. C Skin reconstruction after tumor resection (perioperative antibiotics): consider discussing the management of antibiotics with adult patients undergoing reconstruction after skin cancer resection when relevant. C Show 3 more Skin reconstruction after tumor resection (perioperative antithrombotics): consider discussing the management of anticlotting agents with adult patients undergoing reconstruction after skin cancer resection when relevant. C Show 2 more Skin reconstruction after tumor resection (perioperative analgesics): consider discussing the management of pain with adult patients undergoing reconstruction after skin cancer resection when relevant. C Show 2 more Skin reconstruction after tumor resection (follow-up): consider obtaining postoperative follow- up assessment in adult patients undergoing reconstruction after skin cancer resection. C Surveillance for future malignancies: As per BAD 2021 guidelines, offer at least yearly follow-up, if possible, in adult patients with a history of multiple BCC likely to develop further tumors or recurrence within 12 months. E As per EDF 2019 guidelines, follow-up patients with BCC in 3, 6 or 12 monthly intervals according to the risk category. B As per AAD 2018 guidelines, obtain skin cancer screening for new keratinocyte cancers (BCC or cutaneous SCC) and for melanoma on at least an annual basis after diagnosis of first BCC. A Clinical findings Patient demographics Symptoms Elderly Itching Skin growth Past medical history Social history Exposure to radiation therapy Xeroderma pigmentosum Exposure to excessive sun Integument exam Fitzpatrick skin type 1 Nonhealing skin lesion Skin nodule Skin plaque https://web.pathway.md/diseases/recwv4t9NOosmhAmc 7/8 6/29/23, 3:16 AM Basal cell carcinoma Pathway References 1. Work Group, Invited Reviewers, John Y S Kim et al. Guidelines of care for the management of basal cell carcinoma. J Am Acad Dermatol. 2018 Mar;78 3 540 559. Open 2. US Preventive Services Task Force, Grossman DC, Curry SJ et al. Behavioral Counseling to Prevent Skin Cancer: US Preventive Services Task Force Recommendation Statement. JAMA. 2018 Mar 20;319 11 1134 1142. Open 3. Anna Likhacheva, Musaddiq Awan, Christopher A Barker et al. Definitive and Postoperative Radiation Therapy for Basal and Squamous Cell Cancers of the Skin: Executive Summary of an American Society for Radiation Oncology Clinical Practice Guideline. Pract Radiat Oncol. Jan-Feb 2020;10 1 8 20. Open 4. Peris K, Fargnoli MC, Garbe C et al. Diagnosis and treatment of basal cell carcinoma: European consensus-based interdisciplinary guidelines. Eur J Cancer. 2019 Sep;118 10 34. Open 5. Andrew Chen, John G Albertini, Jeremy S Bordeaux et al. Evidence-Based Clinical Practice Guideline: Reconstruction after Skin Cancer Resection. Plast Reconstr Surg. 2021 May 1;147 5 812e-829e. Open 6. US Preventive Services Task Force, Carol M Mangione, Michael J Barry et al. Screening for Skin Cancer: US Preventive Services Task Force Recommendation Statement. JAMA. 2023 Apr 18;329 15 1290 1295. Open 7. I Nasr, E J McGrath, C A Harwood et al. British Association of Dermatologists guidelines for the management of adults with basal cell carcinoma 2021. Br J Dermatol. 2021 Nov;185 5 899 920. Open 8. Lang BM, Balermpas P, Bauer A et al. S2k Guidelines for Cutaneous Basal Cell Carcinoma Part 1 Epidemiology, Genetics and Diagnosis. J Dtsch Dermatol Ges. 2019 Jan;17 1 94 103. Open 9. Lang BM, Balermpas P, Bauer A et al. S2k Guidelines for Cutaneous Basal Cell Carcinoma Part 1 Epidemiology, Genetics and Diagnosis. J Dtsch Dermatol Ges. 2019 Jan;17 1 94 103. Open 10. Alexander G Marzuka, Samuel E Book. Basal cell carcinoma: pathogenesis, epidemiology, clinical features, diagnosis, histopathology, and management. Yale J Biol Med. 2015 Jun 1;88 2 167 79. eCollection 2015 Jun. Open 11. Emi Dika, Federica Scarf , Manuela Ferracin et al. Basal Cell Carcinoma: A Comprehensive Review. Int J Mol Sci. 2020 Aug 4;21 15 5572. Open 12. C A Morton, R M Szeimies, N Basset-Seguin et al. European Dermatology Forum guidelines on topical photodynamic therapy 2019 Part 1 treatment delivery and established indications - actinic keratoses, Bowen's disease and basal cell carcinomas. J Eur Acad Dermatol Venereol. 2019 Dec;33 12 2225 2238. Open https://web.pathway.md/diseases/recwv4t9NOosmhAmc 8/8
Guideline sources The following summarized guidelines for the evaluation and management of behavioral and psychological symptoms of dementia (BPSD) are prepared by our editorial team based on guidelines from the American Geriatrics Society (AGS 2015), the Canadian Geriatrics Society (CGS 2012), and the American Psychiatric Association (APA 2007). 1 2 3 4 5 5 6 Definition BPSD (BPSD) is a heterogeneous range of psychological reactions, psychiatric symptoms, and behaviors occurring in people with dementia of any etiology. 4 Epidemiology BPSD are caused due to a complex interplay of psychological, social, and biological factors including neurochemical, neuropathological and genetic factors. 5 Disease course The complex interaction of psychosocial and biological factors results in BPSD, which have clinical manifestations of agitation, aberrant motor behavior, anxiety, elation, irritability, depression, apathy, disinhibition, delusions, hallucinations, and sleep or appetite changes. Disease progression is associated with poor quality of life, distress among patients and caregivers, long-term hospitalizations, medication misue,a nd increased health-care costs. 5 https://web.pathway.md/diseases/recZCjq4k7lbY7KFO 1/4 6/29/23, 3:16 AM Behavioral and psychological symptoms of dementia Pathway Prognosis and risk of recurrence Moderate and severe BPSD are associated with an increased risk of mortality with HR 1.31 (95% CI, 1.08-1.60) and 1.74 (95% CI 1.44-2.12), respectively. 6 Pathway Behavioral and psychological sy Management Guidelines 1. Diagnostic investigations General principles: undertake a careful evaluation for general medical, psychiatric, environmental, or psychosocial problems that may underlie the disturbance. A 2. Medical management Initial management: evaluate for general medical, psychiatric, environmental, or psychosocial problems that may underlie the behavioral and psychological symptoms, and manage any underlying causes appropriately. A Pharmacologic treatment: As per Choosing Wisely 2015 guidelines, avoid antipsychotics as a first-line therapy for BPSD. D As per APA 2007 guidelines: Consider the judicious use of psychotropic agents in patients with BPSD in whom nonpharmacologic measures are unsuccessful, or if the behaviors are particularly dangerous or distressing. C Implement environmental measures, such as reassurance and redirection, to address persistent symptoms that do not cause significant danger or distress to the patient or others. A Antipsychotic agents: As per CGS 2012 guidelines, consider using risperidone, olanzapine and aripiprazole for the treatment of severe agitation, aggression and psychosis associated with dementia where there is risk of harm to the patient and/or others. B As per APA 2007 guidelines, consider using antipsychotic medications for the treatment of psychosis in patients with dementia. C Show 2 more https://web.pathway.md/diseases/recZCjq4k7lbY7KFO 2/4 6/29/23, 3:16 AM Behavioral and psychological symptoms of dementia Pathway Benzodiazepines: as per APA 2007 guidelines, consider using benzodiazepines in treating patients with prominent anxiety, who may derive a modest benefit from their use. C Other pharmacologic agents: As per CGS 2012 guidelines, insufficient evidence to make a recommendation regarding the use of cholinesterase inhibitors and/or memantine for the treatment of neuropsychiatric symptoms as a primary indication. I As per APA 2007 guidelines, avoid off-label use of anticonvulsants, lithium, or -blockers for the treatment of psychosis or agitation in dementia, except for patients in whom all other treatments have failed. D 3. Nonpharmacologic interventions Use of restraints: Use physical restraints for the management of behavioral symptoms of dementia only in patients who pose an imminent risk of physical harm to themselves or others. A Avoid physical restraints in patients who do not pose an imminent risk of physical harm to themselves or others. D 4. Follow-up and surveillance Re-evaluation of pharmacologic treatment: reevaluate the use of psychotropic agents and document their benefit on an ongoing basis. A Clinical findings Symptoms Neurological exam Aggression Mood alteration Delusions Depression Difficulty sleeping Hallucinations Repetitive behaviour Social inappropriateness Wandering References 1. APA Work Group on Alzheimer's Disease and other Dementias, Rabins PV, Blacker D et al. American Psychiatric Association practice guideline for the treatment of patients with Alzheimer's disease and other dementias. Second edition. Am J Psychiatry. 2007 Dec;164 12 Suppl):5 56. Open https://web.pathway.md/diseases/recZCjq4k7lbY7KFO 3/4 6/29/23, 3:16 AM Behavioral and psychological symptoms of dementia Pathway 2. Gauthier S, Patterson C, Chertkow H et al. Recommendations of the 4th Canadian Consensus Conference on the Diagnosis and Treatment of Dementia CCCDTD4 . Can Geriatr J. 2012 Dec;15 4 120 6. Open 3. American Geriatrics Society. Choosing Wisely: Recommendations of the American Geriatrics Society. Choosing Wisely. 2015 Apr. Open 4. S Shaji, Srija Bose, Shan Kuriakose. Behavioral and psychological symptoms of dementia: A study of symptomatology. 2009 Jan;51 1 38 41.2009 Jan;51 1 38 41. Open 5. J Cerejeira, L Lagarto, E B Mukaetova-Ladinska. Behavioral and psychological symptoms of dementia. 2012 May 7;3 73.2012 May 7;3 73. Open 6. Vanja Bransvik, Eva Granvik, Lennart Minthon et al. Mortality in patients with behavioural and psychological symptoms of dementia: a registry-based study. 2020 Feb 18;1 9.2020 Feb 18;1 9. Open 7. Reus VI, Fochtmann LJ, Eyler AE et al. The American Psychiatric Association Practice Guideline on the Use of Antipsychotics to Treat Agitation or Psychosis in Patients With Dementia. Am J Psychiatry. 2016 May 1;173 5 543 6. Open 8. American Academy of Neurology. Choosing Wisely AAN recommendations. Choosing Wisely. 2014. Open https://web.pathway.md/diseases/recZCjq4k7lbY7KFO 4/4
Guideline sources The following summarized guidelines for the evaluation and management of Beh et's syndrome (BS) are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023) and the European League Against Rheumatism (EULAR 2022; 2018). 1 2 3 Guidelines 1. Diagnostic investigations Pretreatment evaluation: screen for HBV infection in all patients eligible for treatment with conventional synthetic, biological, or targeted synthetic DMARDs, immunosuppressants, or corticosteroids (according to dose and duration). B Show 3 more 2. Medical management Management of mucocutaneous lesions: initiate topical corticosteroids for the treatment of oral and genital ulcers. B Show 4 more https://web.pathway.md/diseases/recDqh7HlDbJG7Aa8 1/4 6/29/23, 3:16 AM Beh et's syndrome Pathway Management of uveitis: Ensure close collaboration with ophthalmologists for the management of uveitis in patients with BS with the ultimate aim of inducing and maintaining remission. Initiate any of following in patients with BS and inflammatory eye disease affecting the posterior segment: azathioprine B cyclosporine A B interferon-alpha B monoclonal anti-TNF antibodies. B Show 4 more Management of venous thrombosis: initiate corticosteroids and immunosuppressive agents, such as azathioprine, cyclophosphamide or cyclosporine A, for the management of acute deep vein thrombosis in patients with BS. B Show 2 more Management of arterial aneurysms: initiate high-dose corticosteroids and cyclophosphamide for the management of pulmonary artery aneurysms. B Show 3 more Management of GI involvement: confirm gastrointestinal involvement of BS by endoscopy and/or imaging. Rule out NSAID ulcers, IBD and infections such as tuberculosis. B Show 3 more Management of CNS involvement: Initiate high-dose corticosteroids followed by slow tapering, together with immunosuppressive agents, such as azathioprine, in patients with acute attacks of parenchymal involvement. Avoid using cyclosporine A. Consider initiating monoclonal anti-TNF antibodies in patients with severe disease as first-line or in patients with refractory disease. B Initiate high-dose corticosteroids followed by tapering for the treatment of the first episode of cerebral venous thrombosis. Consider adding anticoagulants for a short duration. Screen for vascular disease at an extracranial site. B Management of arthritis: initiate colchicine as the initial treatment in patients with BS with acute arthritis. B Show 2 more 3. Preventative measures Routine immunizations: consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications. C Show 17 more Prophylaxis for Pneumocystis jirovecii pneumonia: consider administering prophylaxis against P. jirovecii pneumonia in patients initiating high-dose corticosteroids, especially in combination with immunosuppressants and depending on the risk-benefit ratio. C https://web.pathway.md/diseases/recDqh7HlDbJG7Aa8 2/4 6/29/23, 3:16 AM Beh et's syndrome Pathway Clinical findings Symptoms Past medical history Abdominal pain Budd-chiari syndrome Blurry vision Cerebral venous thrombosis Diarrhea DVT Dysarthria Gastrointestinal fistula Eye pain Intestinal perforation Headache Meningitis Nausea Optic neuritis Personality or behavioral changes Superficial thrombophlebitis Ocular exam Photophobia Visual disturbances Episcleritis Scleritis Neurological exam Uveitis Ataxia Musculoskeletal exam Cranial nerve palsy Hemiparesis Arthritis ICP Polyarticular synovitis Lhermitte's phenomenon Integument exam Head and neck exam Cutaneous lesions Aphtous ulcers Erythema nodosum Oral ulcers Pyoderma gangrenosum Skin papules Genitourinary exam Skin pustules Genital ulcers Genetic testing Lab findings Hla-b51 ESR serum CRP Diagnostic procedures pathergy test References 1. Gulen Hatemi, Robin Christensen, Dongsik Bang et al. 2018 update of the EULAR recommendations for the management of Beh et's syndrome. Ann Rheum Dis. 2018 Jun;77 6 808 818. Open https://web.pathway.md/diseases/recDqh7HlDbJG7Aa8 3/4 6/29/23, 3:16 AM Beh et's syndrome Pathway 2. George E Fragoulis, Elena Nikiphorou, Mrinalini Dey et al. 2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis. 2022 Nov 3;ard-2022 223335. Open 3. Anne R Bass, Eliza Chakravarty, Elie A Akl et al. 2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases. Arthritis Rheumatol. 2023 Jan 4. Online ahead of print. Open 4. Mendes D, Correia M, Barbedo M et al. Behcet's disease a contemporary review. J Autoimmun. 2009 May-Jun;32 3 4 178 88. Open 5. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 6. Expert Panel on Neurological Imaging, Vikas Agarwal, Lubdha M Shah et al. ACR Appropriateness Criteria Myelopathy: 2021 Update. J Am Coll Radiol. 2021 May;18 5S S73 S82. Open https://web.pathway.md/diseases/recDqh7HlDbJG7Aa8 4/4
Guideline sources The following summarized guidelines for the evaluation and management of Bell's palsy are prepared by our editorial team based on guidelines from the French Society of Otorhinolaryngology (SFORL 2020), the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF 2013), and the American Academy of Neurology (AAN 2012). 1 2 3 4 4 5 5 Definition Bell's palsy, also known as idiopathic facial paralysis, is an acute-onset, isolated, unilateral, lower motor neurone facial weakness/paralysis. 4 Epidemiology The exact cause of Bell's palsy is unknown; however, reactivation of herpes virus at the geniculate ganglion of the facial nerve has been postulated. 5 Disease course https://web.pathway.md/diseases/rec9J9Kp4rg2WXZiw 1/6 6/29/23, 3:16 AM Bell's palsy Pathway The likely vascular distension, inflammation, and edema with ischemia of the facial nerve results in Bell's palsy, which presents with clinical manifestations of unilateral weakness/paralysis of upper and lower facial muscles, drooping of ipsilateral eyelids, dry eye due to inability to close eyes completely, epiphora, drooping of the corner of the mouth, ipsilateral impaired/loss of taste sensation, difficulty in eating, dribbling of saliva, altered sensation on the affected side of the face, pain in or behind the ear, hyperacusis on the affected side if stapedius muscle is involved. Spontaneous complete recovery occurs in 70-75% of untreated patients. 4 Prognosis and risk of recurrence Bell's palsy is not associated with an increased risk of mortality. 5 Calculator House-Brackmann facial paralys Guidelines 1. Screening and diagnosis Differential diagnosis: Question the diagnosis of Bell's palsy and screen for tumoral causes in patients with peripheral facial palsy progressing beyond 72 hours after onset or showing fluctuation or recurrence or bilateral involvement. A Question the diagnosis of Bell's palsy in patients with peripheral facial palsy associated with abnormal otoscopy or parotid or cervical lymph node palpation or ipsilateral hearing loss, dizziness or other neurological signs. B 2. Classification and risk stratification Severity assessment: assess the severity of facial involvement on a standardized grading system (House-Brackmann classification) for inclusion in the medical file during initial work-up of patients with Bell's palsy. B 3. Diagnostic investigations History and physical examination: As per SFORL 2020 guidelines: Perform a clinical examination to confirm the peripheral nature of the facial palsy. B https://web.pathway.md/diseases/rec9J9Kp4rg2WXZiw 2/6 6/29/23, 3:16 AM Bell's palsy Pathway Perform a complete clinical neurological and ear, nose and throat examination with otoscopy and parotid and cervical palpation in patients presenting for peripheral facial palsy. Screen for involvement of the superior and inferior facial areas and absence of autonomic-voluntary dissociation to confirm peripheral status. Attempt to rule out involvement of the somatosensory and motor central pathways and other cranial nerves by neurological examination. B As per AAO-HNSF 2013 guidelines, obtain a clinical history and perform a physical examination to identify underlying causes in patients presenting with acute-onset unilateral facial paresis or paralysis. B Laboratory studies: As per SFORL 2020 guidelines, obtain CBC and fasting glucose in patients with Bell's palsy, and possibly HbA1c in patients with diabetes. B Show 2 more As per AAO-HNSF 2013 guidelines, avoid obtaining routine laboratory testing in patients with new-onset Bell's palsy. D Diagnostic imaging: As per SFORL 2020 guidelines, obtain MRI as an emergency examination only for patients with an atypical clinical presentation. B Show 4 more As per AAO-HNSF 2013 guidelines, avoid routine diagnostic imaging for patients with new-onset Bell's palsy. D Electrodiagnostic testing: As per SFORL 2020 guidelines, do not perform electroneuromyography before day 8 to assess prognosis of patients with Bell's palsy, given the poor prognostic value of the results, which are moreover poorly reproducible. D Show 2 more As per AAO-HNSF 2013 guidelines, avoid electrodiagnostic testing in Bell's palsy patients with incomplete facial paralysis. D Audiometry: obtain audiometry in patients with Bell's palsy. B Tympanometry: assess the stapedial reflex in patients with Bell's palsy for prognostic purposes. Question the diagnosis of Bell's palsy and screen for extratemporal cervical or parotid lesions in case of persistent stapedial reflex in patients with severe peripheral facial palsy. B 4. Medical management Corticosteroids: As per SFORL 2020 guidelines: Administer prednisolone or methylprednisolone in patients with Bell's palsy as early as possible - ideally, within 72 hours. A Use one of the following regimens for corticosteroids: https://web.pathway.md/diseases/rec9J9Kp4rg2WXZiw 3/6 6/29/23, 3:16 AM Bell's palsy Pathway 1 mg/kg/day for 7-10 days 2 mg/kg/day for 10 days in severe cases (House-Brackmann grade V or VI) unless contraindicated (Expert opinion). B As per AAO-HNSF 2013 guidelines, administer oral steroids within 72 hours of symptom onset in patients 16 years of age with Bell's palsy. B As per AAN 2012 guidelines, administer steroids to patients with new-onset Bell's palsy to increase the probability of recovery of facial nerve function. A Antiviral therapy: As per SFORL 2020 guidelines: Do not offer antiviral therapy as a sole treatment for patients with Bell's palsy. D Consider antiviral therapy in addition to corticosteroids for the treatment of patients with severe Bell's palsy treated early (within 72 h). C As per AAO-HNSF 2013 guidelines: Avoid administering oral antiviral therapy alone (without corticosteroids), for patients with new- onset Bell's palsy. D Consider antiviral therapy in addition to oral corticosteroids within 72 hours of symptom onset for patients with Bell's palsy. C As per AAN 2012 guidelines, consider offering antiviral agents in combination with steroids to patients with new-onset Bell's palsy, because of the possibility of a modest increase in recovery. C 5. Nonpharmacologic interventions Eye care: As per SFORL 2020 guidelines, advise eye care including local care, nocturnal occlusion and provide patient education, as early as possible. Refer patients with painful red eye to an ophthalmologist. Perform ophthalmologic monitoring for several weeks once recovery has begun. B Implement eye protection for patients with Bell's palsy who demonstrate impaired eye closure. B Acupuncture: As per SFORL 2020 guidelines, do not offer acupuncture to patients with Bell's palsy. D As per AAO-HNSF 2013 guidelines, insufficient evidence to make a recommendation regarding acupuncture for patients with Bell's palsy. Physical therapy: insufficient evidence to make a recommendation regarding physical therapy for the treatment of Bell's palsy. Physical therapy: As per SFORL 2020 guidelines: https://web.pathway.md/diseases/rec9J9Kp4rg2WXZiw 4/6 6/29/23, 3:16 AM Bell's palsy Pathway Refer patients with severe Bell's palsy or with factors for poor recovery to a rehabilitation specialist (speech therapist or physiotherapist, with relevant qualifications). B Do not offer electrotherapy and forced exercise (chewing gum, biofeedback) to patients with Bell's palsy. D 6. Therapeutic procedures Intratympanic corticosteroids: do not offer intratympanic corticosteroids as a sole treatment for patients with Bell's palsy. D Hyperbaric oxygen therapy: do not offer hyperbaric oxygen therapy for patients with Bell's palsy. D 7. Surgical interventions Facial nerve decompression: As per SFORL 2020 guidelines, insufficient evidence to support facial nerve decompression in patients with acute Bell's palsy. I Show 4 more As per AAO-HNSF 2013 guidelines, insufficient evidence to make a recommendation regarding surgical decompression for patients with Bell's palsy. 8. Specific circumstances Patients with Ramsay-Hunt syndrome: complete a 7-day therapy with prednisolone or methylprednisolone in addition to antiviral treatment, initiated as early as possible, in patients with Ramsay-Hunt syndrome. B 9. Follow-up and surveillance Indications for specialist referral: Reassess or refer to a facial nerve specialist patients with Bell's palsy who meet any of the following criteria: new or worsening neurologic findings at any point ocular symptoms developing at any point incomplete facial recovery 3 months after initial symptom onset. B Follow-up surveillance: consider prolonged surveillance when Bell's palsy does not fully resolve to allow treatment, in order for treatment to be adapted to functional progression. C Clinical findings https://web.pathway.md/diseases/rec9J9Kp4rg2WXZiw 5/6 6/29/23, 3:16 AM Bell's palsy Pathway Symptoms Past medical history Ear pain Recent upper respiratory infection Headache Hypersensitivity to sound Ocular exam Inability to close one eye Loss of feeling in the face Lagophthalmos Loss of the taste on the front of the tongue Tearing Neurological exam Dysarthria Lower face paralysis Upper face paralysis Head and neck exam Drooling References 1. M Fieux, V Franco-Vidal, P Devic et al. French Society of ENT SFORL guidelines. Management of acute Bell's palsy. Eur Ann Otorhinolaryngol Head Neck Dis. 2020 Dec;137 6 483 488. Open 2. Baugh RF, Basura GJ, Ishii LE et al. Clinical practice guideline: Bell's palsy. Otolaryngol Head Neck Surg. 2013 Nov;149 3 Suppl):S1 27. Open 3. Gronseth GS, Paduga R, . Evidence-based guideline update: steroids and antivirals for Bell palsy: report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology. 2012 Nov 27;79 22 2209 13. Open 4. Dhruvashree Somasundara, Frank Sullivan. Management of Bell's palsy. 2017 Jun;40 3 94 97.2017 Jun;40 3 94 97. Open 5. N Julian Holland, Jonathan M Bernstein. Bell's palsy. 2014 Apr 9;2014 1204.2014 Apr 9;2014 1204. Open 6. J W House, D E Brackmann. Facial nerve grading system. Otolaryngol Head Neck Surg. 1985 Apr;93 2 146 7. Open https://web.pathway.md/diseases/rec9J9Kp4rg2WXZiw 6/6
Guideline sources The following summarized guidelines for the evaluation and management of benign paroxysmal positional vertigo (BPPV) are prepared by our editorial team based on guidelines from the Society for Academic Emergency Medicine (SAEM 2023), the American Physical Therapy Association (APTA 2022), the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO- HNSF 2017), and the American Academy of Neurology (AAN 2017). 1 1 1 2 3 4 5 5 Definition BPPV (BPPV) is a common disorder of the inner ear characterized by repeated episodes of positional vertigo and nystagmus. 1 Epidemiology BPPV has an unknown origin but is thought to result from degeneration of the macula. Secondary causes include otoconial dislodgement due to otologic and nonotologic surgery, head trauma, or any means by which mechanical force reaches the inner ear, inner ear disorders (vestibular neuritis, Meniere's disease, and sudden sensorineural hearing loss. 5 Disease course https://web.pathway.md/diseases/recG0FmMlhKMvk2Tm 1/4 6/29/23, 3:17 AM Benign paroxysmal positional vertigo Pathway Disease course The aberrant semicircular canal signaling (canalithiasis and cupulolithiasis) results in BPPV, which causes clinical manifestations of dizziness that lasts less than 20 seconds accompanied by a lingering, nonspecific imbalance and characteristic nystagmus with Dix-Hallpike maneuver. Spontaneous resolution occurs in 25% of patients by 1 month and up to 50% at 3 months. 5 Prognosis and risk of recurrence BPPV is not associated with an increase in mortality. 1 Guidelines 1. Screening and diagnosis Differential diagnosis: Differentiate BPPV from other causes of imbalance, dizziness, and vertigo. B Assess patients with BPPV for factors modifying management, including impaired mobility or balance, CNS disorders, a lack of home support, and/or increased risk for falling. B 2. Diagnostic investigations Dix-Hallpike test: perform the Dix-Hallpike maneuver (by bringing the patient from an upright to supine position with the head turned 45 degrees to one side and the neck extended 20 degrees with the affected ear down) to elicit provoked vertigo associated with torsional, upbeating nystagmus for the diagnosis of posterior semicircular canal BPPV. Repeat the maneuver with the opposite ear down if the initial maneuver is negative. B Supine roll test: obtain a supine roll test to assess for lateral semicircular canal BPPV in patients with a history compatible with BPPV and horizontal or no nystagmus during the Dix-Hallpike maneuver. B Diagnostic imaging: As per SAEM 2023 guidelines, avoid obtaining routine MRI or MRA in adult patients with transient-episodic vestibular syndrome presenting to the emergency department and diagnosed with typical posterior canal-BPPV by a positive Dix-Hallpike test with the characteristic nystagmus. D As per AAO-HNSF 2017 guidelines, do not obtain radiographic imaging in patients meeting diagnostic criteria for BPPV without additional signs and/or symptoms warranting imaging. D Vestibular function testing: As per AAN 2017 guidelines, avoid obtaining cervical vestibular evoked myogenic potential testing for the diagnosis of BPPV. D As per AAO-HNSF 2017 guidelines, do not obtain routine vestibular testing in patients meeting diagnostic criteria for BPPV without additional vestibular signs and/or symptoms warranting https://web.pathway.md/diseases/recG0FmMlhKMvk2Tm 2/4 6/29/23, 3:17 AM Benign paroxysmal positional vertigo Pathway testing. D 3. Medical management Watchful waiting: consider offering observation with follow-up as an initial management strategy in patients with BPPV. C Vestibular suppressants: do not use vestibular suppressant medications (such as antihistamines and/or benzodiazepines) routinely in patients with BPPV. D 4. Nonpharmacologic interventions Vestibular rehabilitation: As per APTA 2022 guidelines, offer vestibular physical therapy in patients with acute, subacute, or chronic unilateral vestibular hypofunction. A Show 9 more As per AAO-HNSF 2017 guidelines, consider offering vestibular rehabilitation, either self- administered or administered by a clinician, in the treatment of patients with BPPV. C 5. Therapeutic procedures Canalith repositioning: perform a canalith repositioning procedure for the treatment of patients with posterior canal BPPV. B 6. Patient education General counseling: educate patients regarding the impact of BPPV on their safety, the potential for disease recurrence, and the importance of follow-up. B 7. Follow-up and surveillance Management of unresolved symptoms: evaluate patients with persistent symptoms for unresolved BPPV and/or underlying peripheral vestibular or CNS disorders. B Serial clinical assessment: reassess patients within 1 month after an initial period of observation or treatment in order to document the resolution or persistence of symptoms. B Clinical findings Patient demographics Symptoms https://web.pathway.md/diseases/recG0FmMlhKMvk2Tm 3/4 6/29/23, 3:17 AM Benign paroxysmal positional vertigo Pathway Age > 60 Dizziness Female sex Inner ear hair cell damage Loss of balance Past medical history Nausea No prodromal vertigo Idiopathic Vertigo Migraine Vomiting Neurological exam Past surgical history Acute or recurrent ataxia Recent brain surgery References 1. Bhattacharyya N, Gubbels SP, Schwartz SR et al. Clinical Practice Guideline: Benign Paroxysmal Positional Vertigo Update). Otolaryngol Head Neck Surg. 2017 Mar;156 3_suppl):S1 S47. Open 2. Jonathan A Edlow, Christopher Carpenter, Murtaza Akhter et al. Guidelines for reasonable and appropriate care in the emergency department 3 GRACE 3 Acute dizziness and vertigo in the emergency department. Acad Emerg Med. 2023 May;30 5 442 486. Open 3. Terry D Fife, James G Colebatch, Kevin A Kerber et al. Practice guideline: Cervical and ocular vestibular evoked myogenic potential testing: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2017 Nov 28;89 22 2288 2296. Open 4. Courtney D Hall, Susan J Herdman, Susan L Whitney et al. Vestibular Rehabilitation for Peripheral Vestibular Hypofunction: An Updated Clinical Practice Guideline From the Academy of Neurologic Physical Therapy of the American Physical Therapy Association. J Neurol Phys Ther. 2022 Apr 1;46 2 118 177. Open 5. Peng You, Ryan Instrum, Lorne Parnes. Benign paroxysmal positional vertigo. 2018 Dec 14;4 1 116 123.2018 Dec 14;4 1 116 123. Open 6. Marco Mandal , Lorenzo Salerni, Daniele Nuti. Benign Positional Paroxysmal Vertigo Treatment: a Practical Update. Curr Treat Options Neurol. 2019 Dec 5;21 12 66. Open 7. Takao Imai, Noriaki Takeda, Tetsuo Ikezono et al. Classification, diagnostic criteria and management of benign paroxysmal positional vertigo. Auris Nasus Larynx. 2017 Feb;44 1 1 6. Open 8. D Nuti, M Masini, M Mandala. Benign paroxysmal positional vertigo and its variants. 2016;137 241 56.2016;137 241 56. Open https://web.pathway.md/diseases/recG0FmMlhKMvk2Tm 4/4
Guideline sources The following summarized guidelines for the evaluation and management of benign prostatic hyperplasia (BPH) are prepared by our editorial team based on guidelines from the European Association of Urology (EAU 2022), the Canadian Urological Association (CUA 2022), and the American Urological Association (AUA 2021). 1 3 4 5 Calculator International Prostate Symptom Guidelines 1. Diagnostic investigations https://web.pathway.md/diseases/reclJV1leKHUqIf9D 1/11 6/29/23, 3:17 AM Benign prostatic hyperplasia Pathway History and physical examination: As per EAU 2022 guidelines, elicit a complete medical history in patients with LUTS. A Show 3 more As per AUA 2021 guidelines, elicit a medical history, perform a physical examination, and utilize the AUA-SI and IPSS in the initial evaluation of patients presenting with bothersome LUTS possibly attributed to BPH. B Urinalysis: As per EAU 2022 guidelines, obtain urinalysis (by dipstick or urinary sediment) in the assessment of patients with LUTS. A As per AUA 2021 guidelines, obtain urinalysis in the initial evaluation of patients presenting with bothersome LUTS possibly attributed to BPH. B Renal function tests: assess renal function if renal impairment is suspected based on history and clinical examination, or in the presence of hydronephrosis, or when considering surgical treatment for LUTS. A Prostate-specific antigen: Measure PSA if a diagnosis of prostate cancer will change management. A Measure PSA if it assists in the treatment and/or decision-making process. A Diagnostic imaging: As per EAU 2022 guidelines, obtain ultrasound of the upper urinary tract in patients with LUTS. B Show 3 more As per AUA 2021 guidelines, consider assessing the prostate size and shape via transrectal or abdominal ultrasound, cystoscopy, or cross-sectional imaging (MRI/CT), if available, before interventions for LUTS secondary/attributed to BPH. C Show 3 more Urodynamic testing: As per EAU 2022 guidelines, obtain uroflowmetry in the initial assessment of patients with LUTS. B Show 7 more As per AUA 2021 guidelines: Consider obtaining uroflowmetry before interventions for LUTS secondary/attributed to BPH. C Consider obtaining pressure flow studies before interventions for LUTS secondary/attributed to BPH, if diagnostic uncertainty exists. E 2. Diagnostic procedures Urethrocystoscopy: https://web.pathway.md/diseases/reclJV1leKHUqIf9D 2/11 6/29/23, 3:17 AM Benign prostatic hyperplasia Pathway As per EAU 2022 guidelines, perform urethrocystoscopy in patients with LUTS before minimally invasive/surgical therapies if the findings may change treatment. B As per AUA 2021 guidelines, consider assessing the prostate size and shape via cystoscopy, if available, before interventions for LUTS secondary/attributed to BPH. C 3. Medical management Watchful waiting: offer watchful waiting in patients with mild/moderate symptoms, minimally bothered by their symptoms. A Alpha-blockers: As per CUA 2022 guidelines: Offer alpha-blockers as an excellent first-line therapeutic option in patients with symptomatic BPH. A Consider offering alpha-blocker therapy during the period of catheterization in patients with acute urinary retention secondary to BPH. C As per EAU 2022 guidelines, offer alpha-1 blockers in patients with moderate-to-severe LUTS. A As per AUA 2021 guidelines, offer any of the following alpha-blockers (choice of agent based on patient age and comorbidities, and different adverse event profiles such as ejaculatory dysfunction, changes in BP) as a treatment option in patients with bothersome, moderate-to- severe LUTS secondary/attributed to BPH: alfuzosin doxazosin silodosin tamsulosin terazosin. B Show 4 more 5-alpha reductase inhibitors: As per CUA 2022 guidelines, offer 5-ARIs (dutasteride and finasteride) as an appropriate and effective treatment in patients with LUTS associated with demonstrable prostatic enlargement. A Show 2 more As per EAU 2022 guidelines, offer 5-ARIs in patients with moderate-to-severe LUTS and an increased risk of disease progression (such as prostate volume > 40 mL). Counsel patients about the slow onset of action of 5-ARIs. A As per AUA 2021 guidelines, offer 5-ARI monotherapy as an option for improvement of symptoms in patients with LUTS secondary/attributed to BPH with prostatic enlargement as judged by any of the following: prostate volume of > 30 ml on imaging https://web.pathway.md/diseases/reclJV1leKHUqIf9D 3/11 6/29/23, 3:17 AM Benign prostatic hyperplasia Pathway PSA > 1.5 ng/dL palpable prostate enlargement on digital rectal exam. B Show 3 more As per AUA 2021 guidelines, consider initiating 5-ARIs in patients with refractory hematuria presumably due to prostatic bleeding, after exclusion of other causes of hematuria. E Phosphodiesterase-5 inhibitors: As per CUA 2022 guidelines, offer long-acting PDE5 inhibitors as monotherapy in patients with LUTS/BPH, particularly in patients with both LUTS and erectile dysfunction. B As per EAU 2022 guidelines, offer PDE5 inhibitors in patients with moderate-to-severe LUTS, with or without erectile dysfunction. A As per AUA 2021 guidelines, consider offering tadalafil 5 mg daily as a treatment option in patients with LUTS secondary/attributed to BPH, irrespective of comorbid erectile dysfunction. C Anticholinergic agents: As per CUA 2022 guidelines, consider offering antimuscarinic agents in patients with BPH with predominately storage symptoms. Be cautious when using in patients with significant bladder outlet obstruction and/or an elevated post-void residual volume. C As per EAU 2022 guidelines: Offer antimuscarinic agents in patients with moderate-to-severe LUTS with predominately bladder storage symptoms. A Do not use antimuscarinic overactive bladder medications in patients with a post-void residual volume > 150 mL. D As per AUA 2021 guidelines, consider offering anticholinergic agents, alone or in combination with an alpha-blocker, as a treatment option in patients with moderate-to-severe predominant storage LUTS. C Beta-3 agonists: As per CUA 2022 guidelines, consider offering -3 agonists in patients with BPH with predominately storage symptoms. Be cautious when using in patients with significant bladder outlet obstruction and/or an elevated post-void residual volume. C As per EAU 2022 guidelines, offer -3 agonists in patients with moderate-to-severe LUTS with predominately bladder storage symptoms. B Combination therapy: As per CUA 2022 guidelines, offer combination therapy with an alpha-blocker and a 5-ARI in patients with LUTS associated with prostatic enlargement (> 30 mL). B Show 2 more As per EAU 2022 guidelines, offer combination treatment with an alpha-1 blocker and a 5-ARI in patients with moderate-to-severe LUTS and an increased risk of disease progression (such as prostate volume > 40 mL). A Show 3 more https://web.pathway.md/diseases/reclJV1leKHUqIf9D 4/11 6/29/23, 3:17 AM Benign prostatic hyperplasia Pathway As per AUA 2021 guidelines, consider offering a 5-ARI in combination with an alpha-blocker as a treatment option only in patients with LUTS associated with demonstrable prostatic enlargement as judged by any of the following: prostate volume of > 30 mL on imaging PSA > 1.5 ng/dL palpable prostate enlargement on digital rectal exam. B Show 3 more Management of nocturia (addressing underlying causes): treat underlying causes of nocturia, including behavioral, systemic conditions, sleep disorders, lower urinary tract dysfunction, or a combination of factors. B Management of nocturia (behavioral changes): discuss behavioral changes with the patient to reduce nocturnal urine volume and episodes of nocturia and improve sleep quality. B Management of nocturia, desmopressin: As per CUA 2022 guidelines, offer desmopressin as a therapeutic option in patients with LUTS/BPH with nocturia as a result of nocturnal polyuria. B As per EAU 2022 guidelines, offer desmopressin to decrease nocturia due to nocturnal polyuria in < 65 years old patients. B Show 3 more Management of nocturia (anti-LUTS therapy): offer alpha-1 blockers for the treatment of nocturia in patients with nocturia associated with LUTS. B Show 3 more Management of urinary incontinence (evaluation): elicit a complete medical history including symptoms and comorbidities, medications, and perform a focused physical examination in the evaluation of patients with urinary incontinence. A Show 3 more Management of urinary incontinence (conservative management): offer lifestyle advice to improve urinary incontinence. Inform patients that the evidence for these interventions is lacking. B Show 5 more Management of urinary incontinence (pharmacotherapy): Offer antimuscarinic agents or mirabegron in patients with urge urinary incontinence failed conservative treatment. A Offer duloxetine in patients with stress urinary incontinence. B Inform patients about the possible adverse events of duloxetine. A Management of urinary incontinence (surgical management): do not offer bulking agents in patients with postprostatectomy incontinence. D Show 7 more Management of urinary incontinence (other therapeutic interventions): offer bladder wall injections of onabotulinum toxin A 100 U in patients with overactive bladder/urgency urinary https://web.pathway.md/diseases/reclJV1leKHUqIf9D 5/11 6/29/23, 3:17 AM Benign prostatic hyperplasia Pathway incontinence refractory to medical therapy. B Show 2 more 4. Nonpharmacologic interventions Phytotherapy: As per CUA 2022 guidelines, do not offer phytotherapies as standard treatment in patients with LUTS/BPH. D As per EAU 2022 guidelines, offer hexanic extract of Serenoa repens in patients with LUTS wishing to avoid any potential adverse events especially related to sexual function. B Inform patients that the magnitude of the efficacy of hexanic extract of Serenoa repens may be modest. A 5. Therapeutic procedures Prostatic artery embolization: As per CUA 2022 guidelines, consider offering prostatic artery embolization at centers with urological and radiological collaboration and technical expertise in highly selected, well-informed patients wishing to consider an alternative treatment option. Inform patients about the lack of long-term durability. C As per EAU 2022 guidelines: Offer prostatic artery embolization in patients with moderate-to-severe LUTS wishing to consider minimally invasive treatment options and accept less optimal outcomes compared with TURP. B Perform prostatic artery embolization only in units where the work-up and follow-up are obtained by urologists working collaboratively with trained interventional radiologists for the identification of prostatic artery embolization suitable patients. A As per AUA 2021 guidelines, do not perform prostatic artery embolization for the treatment of patients with LUTS secondary/attributed to BPH outside the context of clinical trials. D Prostatic stenting: consider offering prostatic stenting only as an alternative to catheterization in patients unfit for surgery with a functional detrusor. C Temporary implantable nitinol device: offer implantation of a temporary implantable nitinol device in patients with LUTS interested in preserving ejaculatory function with prostate volume of 30-80 mL. Inform patients about the higher retreatment rate at 3 years. B Intraprostatic botulinum toxin injection: do not offer intraprostatic botulinum toxin-A injection for the treatment of patients with LUTS. D 6. Surgical interventions Indications for surgery: https://web.pathway.md/diseases/reclJV1leKHUqIf9D 6/11 6/29/23, 3:17 AM Benign prostatic hyperplasia Pathway Perform surgery in patients with any of the following: renal insufficiency secondary to BPH refractory urinary retention secondary to BPH recurrent UTIs recurrent bladder stones or gross hematuria due to BPH LUTS secondary/attributed to BPH refractory to or unwilling to use other therapies. B Do not perform surgery solely for the presence of an asymptomatic bladder diverticulum. Consider evaluating for the presence of bladder outlet obstruction in these cases. D Transurethral resection: As per CUA 2022 guidelines, offer monopolar A or bipolar TURP as a standard first-line surgical therapy in patients with moderate-to-severe LUTS/BPH with a prostate volume of 30-80 mL. B As per EAU 2022 guidelines, offer bipolar or monopolar TURP in patients with moderate-to- severe LUTS with a prostate volume of 30-80 mL. A As per AUA 2021 guidelines: Offer TURP as a treatment option in patients with LUTS secondary/attributed to BPH. B Consider using either monopolar or bipolar approach to TURP depending on the clinician's expertise with these techniques. E Transurethral incision: As per CUA 2022 guidelines, offer transurethral incision of the prostate for the treatment of moderate-to-severe LUTS in patients with prostate volume < 30 mL without a middle lobe. Inform patients about the high retreatment rate. B As per EAU 2022 guidelines, offer transurethral incision of the prostate in patients with moderate-to-severe LUTS with prostate volume < 30 mL, without a middle lobe. A As per AUA 2021 guidelines, offer transurethral incision as a treatment option in patients with LUTS secondary/attributed to BPH provided prostate volume is 30 mL. B Transurethral vaporization: As per EAU 2022 guidelines, offer bipolar transurethral vaporization of the prostate as an alternative to TURP in patients with moderate-to-severe LUTS with a prostate volume of 30-80 mL. B As per AUA 2021 guidelines, consider offering bipolar transurethral vaporization of the prostate as a treatment option in patients with LUTS secondary/attributed to BPH. C Transurethral microwave therapy: As per CUA 2022 guidelines, consider offering transurethral microwave therapy as an option in carefully selected, well-informed patients with LUTS/BPH. C As per AUA 2021 guidelines, consider offering transurethral microwave therapy as a treatment option in patients with LUTS secondary/attributed to BPH. C https://web.pathway.md/diseases/reclJV1leKHUqIf9D 7/11 6/29/23, 3:17 AM Benign prostatic hyperplasia Pathway Transurethral needle ablation: do not perform transurethral needle aspiration for the treatment of patients with LUTS secondary/attributed to BPH. D Anatomical endoscopic enucleation: offer anatomic endoscopic enucleation of the prostate (performed by a trained surgeon) as an alternative to TURP or open simple prostatectomy in patients with moderate-to-severe LUTS and any size prostate > 30 mL. Recognize that anatomic endoscopic enucleation of the prostate can be safely performed in patients on anticoagulant/antiplatelet therapy. A Laser enucleation: As per EAU 2022 guidelines, offer laser resection of the prostate using Tm:YAG laser as an alternative to TURP. B Show 4 more As per AUA 2021 guidelines: Consider performing holmium or thulium laser enucleation of the prostate as a treatment option in patients at higher risk of bleeding. E Decide on performing holmium or thulium laser enucleation of the prostate as prostate size- independent treatment options based on the clinician's expertise with these techniques. B Plasmakinetic enucleation: offer bipolar transurethral (plasmakinetic) enucleation of the prostate as an alternative to TURP in patients with moderate-to-severe LUTS. B Photoselective vaporization: As per CUA 2022 guidelines: Offer photoselective vaporization of the prostate as an alternative to monopolar or bipolar TURP in patients with moderate-to-severe LUTS. A Consider offering GreenLight photoselective vaporization therapy as an alternative surgical approach in patients on anticoagulation or with a high cardiovascular risk. C As per EAU 2022 guidelines, offer 80W 532 nm potassium-titanyl-phosphate laser vaporization of the prostate as an alternative to TURP in patients with moderate-to-severe LUTS with a prostate volume of 30-80 mL. A Show 3 more As per AUA 2021 guidelines: Consider offering photoselective vaporization of the prostate as a treatment option in patients at higher risk of bleeding. E Offer photoselective vaporization of the prostate using 120W or 180W platforms as a treatment option in patients with LUTS secondary/attributed to BPH. B Prostatic urethral lift: As per CUA 2022 guidelines: Consider offering prostatic urethral lift as an alternative treatment in patients with LUTS interested in preserving ejaculatory function with prostate volume < 80 mL. C Consider offering prostatic urethral lift in patients with a small-to-moderate median lobe and bothersome LUTS. Inform patients (with or without a median lobe) about the higher https://web.pathway.md/diseases/reclJV1leKHUqIf9D 8/11 6/29/23, 3:17 AM Benign prostatic hyperplasia Pathway retreatment rate at 5 years. C As per EAU 2022 guidelines, offer prostatic urethral lift in patients with LUTS interested in preserving ejaculatory function, with prostate volume < 70 mL and no middle lobe. A As per AUA 2021 guidelines: Consider offering prostatic urethral lift as a treatment option in patients with LUTS secondary/attributed to BPH provided prostate volume 30-80 mL and verified absence of an obstructive middle lobe. C Consider offering prostatic urethral lift as a treatment option in eligible patients desiring preservation of erectile and ejaculatory function. C Water vapor thermal therapy: As per CUA 2022 guidelines, consider offering the Rez m system of convective water vapor energy ablation as an alternative treatment in patients with LUTS interested in preserving ejaculatory function with prostate volume < 80 mL, including patients with a median lobe. C As per AUA 2021 guidelines: Consider offering water vapor thermal therapy as a treatment option in patients with LUTS secondary/attributed to BPH provided prostate volume 30-80 mL. C Consider offering water vapor thermal therapy as a treatment option in eligible patients desiring preservation of erectile and ejaculatory function. C Robotic waterjet ablation: As per CUA 2022 guidelines, consider offering aquablation in patients with LUTS interested in preserving ejaculatory function with prostate volume < 150 mL, with or without a middle lobe. C As per EAU 2022 guidelines, offer aquablation as an alternative to TURP in patients with moderate-to-severe LUTS and a prostate volume of 30-80 mL. B Inform patients about the risk of bleeding and the lack of long-term follow-up data. A As per AUA 2021 guidelines, consider offering robotic waterjet ablation as a treatment option in patients with LUTS secondary/attributed to BPH provided prostate volume 30-80 mL. C Simple prostatectomy: As per CUA 2022 guidelines: Offer open simple prostatectomy as first-line surgical therapy in patients with moderate-to- severe LUTS/BPH and enlarged prostate volume > 80 mL, if anatomic endoscopic enucleation of the prostate is unavailable. A Offer laparoscopic simple prostatectomy or robot-assisted simple prostatectomy as alternative surgical therapies in patients with moderate-to-severe LUTS/BPH and enlarged prostate volume > 80 mL in centers where there are surgeons with high-level expertise in laparoscopy or robotics. B As per EAU 2022 guidelines, offer open prostatectomy in the absence of bipolar transurethral enucleation of the prostate and holmium laser enucleation of the prostate in patients with moderate-to-severe LUTS with prostate volume > 80 mL. A https://web.pathway.md/diseases/reclJV1leKHUqIf9D 9/11 6/29/23, 3:17 AM Benign prostatic hyperplasia Pathway As per AUA 2021 guidelines, consider offering open, laparoscopic, or robot-assisted prostatectomy, depending on the clinician's expertise with these techniques, as treatment options only in patients with large-to-very large prostates. C 7. Patient education General counseling: As per EAU 2022 guidelines, offer lifestyle advice and self-care information before or concurrent with treatment in patients with LUTS. A As per AUA 2021 guidelines, inform patients of the possibility of treatment failure and the need for additional or secondary treatments when considering surgical and minimally-invasive treatments for LUTS secondary to BPH. B As per AUA 2021 guidelines, counsel patients regarding the options for intervention including behavioral/lifestyle modifications, medical therapy and/or referral for discussion of procedural options. E 8. Follow-up and surveillance Follow-up: As per EAU 2022 guidelines: Follow-up all patients receiving conservative, medical, or surgical management. B Define follow-up intervals and examinations according to the specific treatment. B As per AUA 2021 guidelines: Evaluate patients 4-12 weeks after initiating treatment (provided adverse events do not require earlier consultation) to assess response to therapy. Obtain IPSS during reevaluation. Consider obtaining a post-void residual and uroflowmetry during further evaluation. B Obtain further evaluation and consider changing medical management or surgical intervention in patients with bothersome LUTS secondary/attributed to BPH elected initial medical management and not having symptom improvement and/or experiencing intolerable side effects. E Clinical findings Patient demographics Symptoms Male sex Feeling of incomplete bladder emptying Past medical history Nocturia Split urine stream Diabetes mellitus type 2 Terminal dribbling Obesity Urinary frequency https://web.pathway.md/diseases/reclJV1leKHUqIf9D 10/11 6/29/23, 3:17 AM Benign prostatic hyperplasia Pathway Urinary hesitancy Rectal exam Urinary incontinence Enlarged prostate Urinary straining Urinary urgency Weak urine stream Vital signs Hypertension References 1. S. Gravas, J.N. Cornu, M. Gacci et al. EAU Guidelines on Management of Non-Neurogenic Male Lower Urinary Tract Symptoms LUTS , incl. Benign Prostatic Obstruction BPO . EAU. 2022 Mar. Open 2. J Kellogg Parsons, Philipp Dahm, Tobias S K hler et al. Surgical Management of Lower Urinary Tract Symptoms Attributed to Benign Prostatic Hyperplasia: AUA Guideline Amendment 2020. J Urol. 2020 Oct;204 4 799 804. Open 3. Lori B Lerner, Kevin T McVary, Michael J Barry et al. Management of Lower Urinary Tract Symptoms Attributed to Benign Prostatic Hyperplasia: AUA GUIDELINE PART II Surgical Evaluation and Treatment. J Urol. 2021 Oct;206 4 818 826. Open 4. Dean Elterman, M lanie Aub -Peterkin, Howard Evans et al. UPDATE Canadian Urological Association guideline: Male lower urinary tract symptoms/benign prostatic hyperplasia. Can Urol Assoc J. 2022 Aug;16 8 245 256. Open 5. Lori B Lerner, Kevin T McVary, Michael J Barry et al. Management of Lower Urinary Tract Symptoms Attributed to Benign Prostatic Hyperplasia: AUA GUIDELINE PART I Initial Work-up and Medical Management. J Urol. 2021 Oct;206 4 806 817. Open 6. American Urological Association. Choosing Wisely AUA recommendations. Choosing Wisely. 2017. Open 7. Foster HE, Barry MJ, Dahm P et al. Surgical Management of Lower Urinary Tract Symptoms Attributed to Benign Prostatic Hyperplasia: AUA Guideline. J Urol. 2018 Sep;200 3 612 619. Open 8. M J Barry, F J Fowler Jr, M P O'Leary et al. The American Urological Association symptom index for benign prostatic hyperplasia. The Measurement Committee of the American Urological Association. J Urol. 1992 Nov;148 5 1549 57; discussion 1564. Open https://web.pathway.md/diseases/reclJV1leKHUqIf9D 11/11
Guideline sources The following summarized guidelines for the evaluation and management of beta-blocker toxicity are prepared by our editorial team based on guidelines from the American Heart Association (AHA 2020), the American Heart Association (AHA/HRS/ACC 2019), and the American Association of Poison Control Centers (AAPCC/AACT/ACMT 2005). 1 2 3 Guidelines 1. Diagnostic investigations Initial assessment: elicit history to determine the precise ingested dose of a -blocker, the presence of coingestants and comorbidities in patients without evidence of self-harm. B 2. Medical management Admission to emergency department: refer patients with stated or suspected self-harm or victims of a potentially malicious administration of -blocker to an emergency department immediately, guided by local poison center procedures, regardless of the dose reported. B Show 3 more https://web.pathway.md/diseases/recjHXvINa3LCqk5i 1/3 6/29/23, 3:17 AM Beta-blocker toxicity Pathway Gastric decontamination: Do not induce emesis in patients ingested a -blocker exceeding the referral dose. D Consider administering oral activated charcoal, if available and not contraindicated, but not delaying transportation. B Glucagon: As per AHA 2020 guidelines, consider administering IV glucagon in patients with -blocker overdose in refractory shock. C As per ACC 2019 guidelines, consider administering glucagon to increase HR and improve symptoms in patients with bradycardia associated with symptoms or hemodynamic compromise because of -blocker overdose. C High-dose insulin: As per AHA 2020 guidelines, consider administering high-dose insulin with glucose in patients with -blocker overdose in refractory shock. C As per ACC 2019 guidelines, consider administering high-dose insulin to increase HR and improve symptoms in patients with bradycardia associated with symptoms or hemodynamic compromise because of -blocker overdose. C Intravenous calcium: consider administering IV calcium in patients with -blocker overdose in refractory shock. C 3. Inpatient care Inpatient monitoring: Monitor asymptomatic patients referred to healthcare facilities for the following durations after ingestion: 6 hours for an immediate-release product other than sotalol 8 hours for a sustained-release product 12 hours for sotalol. B Do not obtain routine 24-hour inpatient monitoring in asymptomatic patients unintentionally ingested a sustained-release product. D 4. Therapeutic procedures Extracorporeal membrane oxygenation: consider performing ECMO in patients with -blocker overdose in shock refractory to pharmacological therapy. C 5. Follow-up and surveillance Outcome assessment: obtain follow-up calls depending on the specific circumstances to determine outcome at appropriate intervals for up to 12-24 hours based on the judgment of the https://web.pathway.md/diseases/recjHXvINa3LCqk5i 2/3 6/29/23, 3:17 AM Beta-blocker toxicity Pathway poison center staff. B Clinical findings Symptoms Medication history Seizure Beta-blockers Vital signs Neurological exam Bradycardia Altered mental status Hypotension Respiratory exam Cardiac exam Crackles Cardiogenic shock ECG findings Lab findings AV block Hyperkalemia serum glucose serum pH Imaging findings Pulmonary edema References 1. Paul M Wax, Andrew R Erdman, Peter A Chyka et al. beta-blocker ingestion: an evidence-based consensus guideline for out-of-hospital management. Clin Toxicol Phila). 2005;43 3 131 46. Open 2. Ashish R Panchal, Jason A Bartos, Jos G Caba as et al. Part 3 Adult Basic and Advanced Life Support: 2020 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Circulation. 2020 Oct 20;142 16_suppl_2 S366 S468. Open 3. Fred M Kusumoto, Mark H Schoenfeld, Coletta Barrett et al. 2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2019 Aug 20;140 8):e382-e482. Open https://web.pathway.md/diseases/recjHXvINa3LCqk5i 3/3
Guideline sources The following summarized guidelines for the evaluation and management of bicuspid aortic valve (BAV) are prepared by our editorial team based on guidelines from the American Heart Association (AHA/ACC 2022; 2021), the European Society of Cardiology (ESC/EACTS 2021), the European Society of Cardiology (ESC 2014), and the Society of Thoracic Surgeons (STS 2013). 1 2 3 4 5 6 6 7 8 Definition BAV is a common congenital cardiac abnormality consisting of two cusps instead of three that is characterized by dilatation of the thoracic aorta. 6 Epidemiology The exact cause of BAV is unknown; however, genetic causes (Anderson syndrome, NOTCH1 mutation) have been implicated. 7 Disease course https://web.pathway.md/diseases/recRiaA3NnPI5Jmka 1/6 6/29/23, 3:17 AM Bicuspid aortic valve Pathway The fusion of the aortic cusps during valvulogenesis results in the BAV, which can cause clinical manifestations of severe aortic stenosis (symptoms of dyspnea, syncope, and chest pain), AR, aortic incompetence, aortopathy leading to aneurysm and dissection, infective endocarditis to asymptomatic disease. 6 Prognosis and risk of recurrence BAV in patients with definite infective endocarditis of the native aortic valve is associated with a 9% in-hospital mortality. 8 Guidelines 1. Screening and diagnosis Indications for screening: obtain TTE and cardiac MRI at the time of diagnosis to evaluate for BAV, aortic root and ascending aortic dilation, aortic coarctation, and other congenital heart defects in patients with Turner syndrome. B Screening of family relatives: As per ACC 2022 guidelines: Screen all first-degree relatives of patients with BAV and a dilated aortic root or ascending aorta by TTE to evaluate for the presence of BAV, dilation of the aortic root and ascending aorta, or both. Obtain a cardiac-gated CT or MRI of the thoracic aorta if the diameter and morphology of the aortic root, ascending aorta, or both cannot be assessed accurately or completely by TTE. B Consider screening all first-degree relatives of patients with BAV by TTE to evaluate for the presence of a BAV, dilation of the aortic root and ascending aorta, or both. C As per AHA 2021 guidelines, consider obtaining screening with TTE to look for the presence of a BAV or asymptomatic dilation of the aortic sinuses and ascending aorta in first-degree relatives of patients with a known BAV. C As per ESC 2014 guidelines, consider screening first-degree relatives of patients with BAV because of familial occurrence. C 2. Diagnostic investigations Transthoracic echocardiogram: As per ACC 2022 guidelines, obtain TTE to evaluate valve morphology and function, the diameter of the aortic root and ascending aorta, and to evaluate for aortic coarctation and other associated cardiovascular defects in patients with BAV. B As per AHA 2021 guidelines, obtain TTE in patients with a known BAV in order to: evaluate valve morphology measure the severity of aortic stenosis and AR https://web.pathway.md/diseases/recRiaA3NnPI5Jmka 2/6 6/29/23, 3:17 AM Bicuspid aortic valve Pathway assess the shape and diameter of the aortic sinuses and ascending aorta evaluate for the presence of aortic coarctation for prediction of clinical outcome determine the timing of intervention. B As per ESC 2014 guidelines, obtain an initial TTE in patients with known BAV to assess the diameters of the aortic root and ascending aorta. B Computed tomogrpahy/magnetic resonance imaging: As per ACC 2022 guidelines, obtain CT or MRI of the thoracic aorta in patients with BAV when the diameter and morphology of the aortic root, ascending aorta, or both cannot be assessed accurately or completely by TTE. B As per AHA 2021 guidelines, obtain cardiac MRA or CT angiography in patients with BAV if the morphology of the aortic sinuses, sinotubular junction, or ascending aorta cannot be assessed accurately or fully by echocardiography. B As per ESC 2014 guidelines: Obtain cardiac MRI or CT in patients with BAV when the morphology of the aortic root and the ascending aorta cannot be accurately assessed by transthoracic echocardiography. B Obtain cardiac CT or MRI to confirm the measurement in the case of aortic diameter > 5.0 cm or an increase > 3 cm/year measured by echocardiography. B Genetic testing: obtain genetic testing in patients with BAV and either heritable thoracic aortic disease or phenotypic features concerning for Loeys-Dietz syndrome. B 3. Medical management Beta-blockers: consider -blockers in patients with BAV and dilated aortic root > 4.0 cm. C 4. Nonpharmacologic interventions Exercise restrictions: advise avoidance of isometric exercise with a high static load (such as weightlifting) in patients with any elastopathy or BAV with dilated aortic root (> 4.0 cm). B 5. Surgical interventions Indications for aortic replacement or repair: As per ACC 2022 guidelines, perform surgery to replace the aortic root, ascending aorta, or both, in patients with BAV and a diameter of the aortic root, ascending aorta, or both, of 5.5 cm. B Show 4 more As per AHA 2021 guidelines, perform operative intervention to replace the aortic sinuses and/or the ascending aorta in asymptomatic or symptomatic patients with BAV and a diameter of the aortic sinuses or ascending aorta > 5.5 cm. B https://web.pathway.md/diseases/recRiaA3NnPI5Jmka 3/6 6/29/23, 3:17 AM Bicuspid aortic valve Pathway Show 6 more As per ESC 2021 guidelines, perform valve-sparing aortic root replacement in young patients with aortic root dilation, if performed at an experienced center and durable results are expected. B Show 2 more 6. Specific circumstances Pregnant patients: obtain aortic imaging (TTE, MRI or CT, or both as appropriate) before pregnancy to determine aortic diameters in patients with BAV with aortic dilation. B Show 2 more 7. Follow-up and surveillance Follow-up aortic imaging: As per ACC 2022 guidelines, obtain lifelong surveillance imaging of the aortic root and ascending aorta by TTE, CT, or MRI at an interval dependent on the aortic diameter and rate of growth in patients with BAV and a diameter of the aortic root, ascending aorta, or both, of 4.0 cm, B including patients undergone previous aortic valve repair or replacement. B As per AHA 2021 guidelines: Consider obtaining lifelong serial evaluation of the size and morphology of the aortic sinuses and ascending aorta by echocardiography, cardiac magnetic resonance or CT angiography in patients with BAV and a diameter of the aortic sinuses or ascending aorta > 4.0 cm, with an examination interval determined by the degree and rate of the progression of aortic dilation and by family history. C Consider obtaining continued lifelong serial interval imaging of the aorta in patients with BAV undergoing aortic valve replacement, if the diameter of the aortic sinuses or ascending aorta is 4.0 cm. C As per ESC 2014 guidelines: Obtain serial measurement of the aortic root and ascending aorta in every patient with BAV, with an interval depending on aortic size, increase in size and family history. B Obtain annual measurement of aortic diameter in the case of a diameter of the aortic root or the ascending aorta > 4.5 mm or an increase > 0.3 cm/year measured by echocardiography. B Pharmacotherapy after valve repair or replacement: consider initiating postoperative - blockers after bicuspid valve repair. B Show 2 more Clinical findings https://web.pathway.md/diseases/recRiaA3NnPI5Jmka 4/6 6/29/23, 3:17 AM Bicuspid aortic valve Pathway Patient demographics Symptoms Male sex Chest pain on exertion Fainting Past medical history Fatigue Lightheadedness Coarctation of aorta Palpitations Congenital heart disease Ehlers-danlos syndrome Cardiac exam Infective endocarditis Loeys-dietz syndrome Ejection click Marfan syndrome Midsystolic murmur Thoracic aortic aneurysm Thoracic aortic dissection Turner syndrome Williams syndrome Respiratory exam Dyspnea References 1. Eric M Isselbacher, Ourania Preventza, James Hamilton Black rd et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. Circulation. 2022 Nov 2. Open 2. Erbel R, Aboyans V, Boileau C et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology ESC . Eur Heart J. 2014 Nov 1;35 41 2873 926. Open 3. Catherine M Otto, Rick A Nishimura, Robert O Bonow et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Thorac Cardiovasc Surg. 2021 Aug;162 2):e183-e353. Open 4. Alec Vahanian, Friedhelm Beyersdorf, Fabien Praz et al. 2021 ESC/EACTS Guidelines for the management of valvular heart disease: Developed by the Task Force for the management of valvular heart disease of the European Society of Cardiology ESC and the European Association for Cardio-Thoracic Surgery EACTS . Eur Heart J. 2021;ehab395. Open 5. Svensson LG, Adams DH, Bonow RO et al. Aortic valve and ascending aorta guidelines for management and quality measures. Ann Thorac Surg. 2013 Jun;95 6 Suppl):S1 66. Open 6. Ify Mordi, Nikolaos Tzemos. Bicuspid aortic valve disease: a comprehensive review. 2012;2012 196037.2012;2012 196037. Open 7. Stefanos Sakellaropoulos, Muhemin Mohammed, Stefano Svab et al. Causes, Diagnosis, Risk Stratification and Treatment of Bicuspid Aortic Valve Disease: An Updated Review. 2020 Aug;11 4 205 212.2020 Aug;11 4 205 212. Open https://web.pathway.md/diseases/recRiaA3NnPI5Jmka 5/6 6/29/23, 3:17 AM Bicuspid aortic valve Pathway 8. Gokhan Kahveci, Fatih Bayrak, Selcuk Pala et al. Impact of bicuspid aortic valve on complications and death in infective endocarditis of native aortic valves. 2009;36 2 111 6.2009;36 2 111 6. Open 9. Hiratzka LF, Creager MA, Isselbacher EM et al. Surgery for Aortic Dilatation in Patients With Bicuspid Aortic Valves: A Statement of Clarification From the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2016 Feb 16;133 7 680 6. Open https://web.pathway.md/diseases/recRiaA3NnPI5Jmka 6/6
Guideline sources The following summarized guidelines for the evaluation and management of bile acid diarrhea (BAD) are prepared by our editorial team based on guidelines from the Canadian Association of Gastroenterologists (CAG 2020). 1 Guidelines 1. Diagnostic investigations Clinical evaluation: Ask about risk factors (history of terminal ileal resection, cholecystectomy, or abdominal radiotherapy) in the initial assessment of patients presenting with chronic non-bloody diarrhea to identify possible BAD. B Do not use presenting symptoms in the initial assessment in patients presenting with chronic non-bloody diarrhea to identify possible BAD. D Laboratory testing: obtain SeHCAT and serum C4 assay for the diagnosis of BAD in patients with chronic diarrhea, including IBS-D and functional diarrhea. B Show 2 more 2. Medical management https://web.pathway.md/diseases/recCYlHcNlojwbo8r 1/2 6/29/23, 3:17 AM Bile acid diarrhea Pathway Bile acid sequestrants (choice of agent): consider administering cholestyramine for induction of clinical response in patients with BAD. C Show 2 more Bile acid sequestrants (dosing): titrate the daily dose of empiric bile acid sequestrants gradually to minimize side effects in patients with BAD. E Show 2 more Bile acid sequestrants (monitoring): Review concurrent medications to minimize the potential for drug interactions in patients with BAD considered for bile acid sequestrant therapy. E Insufficient evidence to recommend for or against measuring fat-soluble vitamin levels at baseline and annually thereafter in patients with BAD receiving long-term maintenance therapy with bile acid sequestrants. I Anti-diarrheal agents: consider offering alternative anti-diarrheal agents for long-term symptomatic therapy in patients with BAD unable to tolerate bile acid sequestrant therapy. C Management of secondary causes: Treat secondary causes, such as Crohn's disease, microscopic colitis, SIBO, for induction of clinical response in patients with type 1 or 3 BAD. B Consider avoiding the use of bile acid sequestrants in patients with BAD and Crohn's disease with extensive ileal involvement or resection. D 3. Follow-up and surveillance Follow-up: obtain diagnostic reevaluation in patients with BAD and recurrent or worsening symptoms despite stable bile acid sequestrant therapy. E References 1. Daniel C Sadowski, Michael Camilleri, William D Chey et al. Canadian Association of Gastroenterology Clinical Practice Guideline on the Management of Bile Acid Diarrhea. Clin Gastroenterol Hepatol. 2020 Jan;18 1 24 41.e1. Open https://web.pathway.md/diseases/recCYlHcNlojwbo8r 2/2
Guideline sources The following summarized guidelines for the evaluation and management of biliary stricture are prepared by our editorial team based on guidelines from the American College of Gastroenterology (ACG 2023), the European Society of Medical Oncology (ESMO 2023; 2016), the Thai Association for Gastrointestinal Endoscopy (TAGE/T-CAP 2022), the European Society of Gastrointestinal Endoscopy (ESGE 2021; 2020; 2019; 2018), the Thai Association for Gastrointestinal Endoscopy (TAGE/T-CAP/DEST 2020), the British Society of Gastroenterology (BSG 2019), the American College of Radiology (ACR 2019), the Infectious Diseases Society of America (IDSA 2016), the American Society for Gastrointestinal Endoscopy (ASGE 2015), and the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES 2010). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 Guidelines 1. Diagnostic investigations Evaluation of extrahepatic strictures (abdominal ultrasound): consider obtaining abdominal ultrasound in the initial evaluation to identify biliary obstruction in patients with biliary tract cancer. E https://web.pathway.md/diseases/rec5jsaxkrOGmSEpV 1/6 6/29/23, 3:17 AM Biliary stricture Pathway Evaluation of extrahepatic strictures (CT/MRI): consider obtaining contrast-enhanced CT or MRI for the detection and characterization of a mass and to determine the resectability of malignant lesions. B Evaluation of extrahepatic strictures (ERCP): consider performing ERCP to establish the etiology in patients with distal biliary strictures. Perform ERCP-guided tissue acquisition when biliary drainage is required. B Evaluation of extrahepatic strictures, EUS with FNA: As per ACG 2023 guidelines: Perform EUS with fine-needle sampling (aspiration or biopsy) over ERCP to evaluate for malignancy in patients with extrahepatic biliary stricture due to an apparent or suspected pancreatic mass. B Consider performing EUS with fine-needle biopsy or EUS with FNA plus rapid on-site evaluation over FNA without rapid on-site evaluation to evaluate for malignancy in patients with extrahepatic biliary stricture due to an apparent or suspected pancreatic mass. C As per T-CAP/TAGE/DEST 2020 guidelines: Consider performing EUS to establish the etiology in patients with distal biliary strictures. B Perform EUS-guided FNA when cross-sectional imaging reveals a mass associated with the stricture or when endoscopic retrograde cholangio-pancreatography-guided tissue acquisition is unsuccessful. A Evaluation of extrahepatic strictures (cholangioscopy and intraductal ultrasound): consider performing peroral cholangioscopy and intraductal ultrasound to improve the characterization of distal biliary strictures. C Evaluation of perihilar strictures: perform multimodality sampling over brush cytology alone during the index ERCP in patients with a suspected malignant perihilar stricture. B Evaluation of indeterminate strictures: As per TAGE 2022 guidelines, perform direct visualization or targeted biopsy via cholangioscopy to diagnose malignant biliary strictures in patients with an indeterminate biliary stricture. B Consider using image enhancement during cholangioscopy to increase the diagnostic sensitivity of visual impressions of malignant biliary strictures. B Show 3 more As per ASGE 2015 guidelines, consider performing peroral cholangioscopy with directed biopsy as an adjunctive technique for characterizing indeterminate biliary strictures. C Evaluation of primary sclerosing cholangitis: obtain MRCP as the principal imaging modality for the evaluation of patients with suspected PSC. Consider performing ERCP in patients with biliary strictures requiring tissue acquisition (such as cytological brushings). Perform pathological sampling of suspicious strictures in patients undergoing ERCP for dominant strictures. A Evaluation of postoperative biliary obstruction: obtain a specialized multidisciplinary evaluation in patients with biliary tract cancer and postoperative biliary obstruction to determine the location of the obstruction, evaluate for recurrence, and determine the optimal approach to drainage. B https://web.pathway.md/diseases/rec5jsaxkrOGmSEpV 2/6 6/29/23, 3:17 AM Biliary stricture Pathway 2. Therapeutic procedures Biliary drainage, extrahepatic obstruction, benign causes: As per ACG 2023 guidelines, perform placement of a fully covered self-expanding metallic stent over multiple plastic stents in parallel to reduce the procedures required for long-term treatment in patients with extrahepatic stricture caused by a benign condition. B As per T-CAP/TAGE/DEST 2020 guidelines, perform endoscopic biliary drainage for the treatment of cholangitis and relief of cholestasis. Consider performing endoscopic stenting as the primary treatment of benign biliary strictures. A Show 3 more As per ESGE 2019 guidelines, consider performing placement of temporary multiple side-by- side plastic stents or a fully covered self-expandable metal stent in patients with chronic pancreatitis-related benign biliary strictures. C As per ASGE 2015 guidelines, perform ERCP with dilation and stent placement in patients with benign biliary strictures. B Biliary drainage, extrahepatic obstruction, malignant causes: As per ACG 2023 guidelines, avoid performing routine preoperative biliary drainage in patients with extrahepatic stricture caused by resectable pancreatic cancer or cholangiocarcinoma. Perform preoperative biliary drainage in selected patients, including patients with acute cholangitis, severe pruritus, very high serum bilirubin levels, and patients undergoing neoadjuvant therapy or experiencing another anticipated delay to surgery. D Show 2 more As per ESMO 2023 guidelines: Perform biliary drainage and subsequent treatment in patients with biliary tract cancer and biliary obstruction. Perform percutaneous transhepatic drainage when endoscopic access is not possible. B Prefer a metal stent in patients with a life expectancy of > 3 months. B Inform patients of the likely duration of stent patency and symptoms and signs of biliary obstruction or infection. B As per ESGE 2021 guidelines, perform retrograde cholangiopancreatography with self- expandable metal stent placement in patients with ampullary tumors and biliary obstruction in palliative settings. A As per T-CAP/TAGE/DEST 2020 guidelines, perform endoscopic biliary drainage for the treatment of cholangitis and relief of cholestasis. A Show 9 more As per ESGE 2018 guidelines, do not perform preoperative biliary drainage routinely in patients with malignant extrahepatic biliary obstruction. Reserve preoperative biliary drainage for patients with cholangitis, severe symptomatic jaundice (intense pruritus), delayed surgery, or before neoadjuvant chemotherapy in jaundiced patients. D Show 6 more Biliary drainage, perihilar obstruction: https://web.pathway.md/diseases/rec5jsaxkrOGmSEpV 3/6 6/29/23, 3:17 AM Biliary stricture Pathway As per ACG 2023 guidelines, insufficient evidence to recommend for or against ERCP versus percutaneous transhepatic biliary drainage in patients with perihilar stricture due to suspected malignancy. Show 2 more As per ACR 2019 guidelines, perform placement of a percutaneous internal/external biliary catheter in patients with malignant hilar obstruction (such as Klatskin tumor). B As per ESGE 2018 guidelines, perform drainage of malignant hilar strictures in high volume centers with a multidisciplinary hepatobiliary team. B Show 4 more Removal of biliary stents: perform ERCP with planned stent exchange or removal in patients with benign distal biliary strictures. B Show 2 more Adjunctive therapies: recognize that endo-biliary photodynamic therapy, B radiofrequency ablation, B and drug-eluting stents are considered investigational therapies for malignant distal biliary strictures. B 3. Perioperative care Preprocedural antibiotic prophylaxis: As per ESGE 2020 guidelines: Do not administer routine antibiotic prophylaxis before ERCP. D Consider administering antibiotic prophylaxis (with an agent active against Gram-negative bacteria and adapted as much as possible to local epidemiology) before ERCP in case of anticipated incomplete biliary drainage, in severely immunocompromised patients, and when performing cholangioscopy. C As per ASGE 2015 guidelines, administer antibiotic prophylaxis before ERCP in patients with known or suspected biliary obstruction, where there is a possibility of incomplete biliary drainage. Administer antibiotics covering biliary flora, such as enteric gram-negative organisms and enterococci, and continue after the procedure if biliary drainage is incomplete. B Pre-stenting sphincterotomy: avoid performing routine endoscopic biliary sphincterotomy before the insertion of a single plastic or uncovered/partially covered self-expandable metallic stent for relief of biliary obstruction. D 4. Surgical interventions Indications for surgery (hepatic aspergillosis): consider performing surgical intervention in patients with extrahepatic or perihepatic biliary obstruction caused by hepatic aspergillosis. C https://web.pathway.md/diseases/rec5jsaxkrOGmSEpV 4/6 6/29/23, 3:17 AM Biliary stricture Pathway Indications for surgery (sphincter of Oddi dysfunction): consider performing laparoscopic cholecystectomy in patients with symptoms of biliary obstruction without evidence of gallstones but with abnormal gallbladder emptying. C 5. Specific circumstances Patients with an indwelling biliary catheter: recognize that cholangitis in patients with an indwelling biliary stent suggests recurrent biliary obstruction and may require early endoscopic re- intervention. B Show 3 more Clinical findings Symptoms Past medical history Dark urine Autoimmune pancreatitis Fever Cholangiocarcinoma Generalized pruritus Chronic pancreatitis Nausea Gallbladder cancer Pale stools Liver metastasis RUQ pain PSC Vomiting Pancreatic cancer Ocular exam Integument exam Scleral icterus Jaundice Lab findings Imaging findings serum ALP Bile duct stricture serum TBIL Dilated bile ducts References 1. B Joseph Elmunzer, Jennifer L Maranki, Victoria G mez et al. ACG Clinical Guideline: Diagnosis and Management of Biliary Strictures. Am J Gastroenterol. 2023 Mar 1;118 3 405 426. Open 2. Jean-Marc Dumonceau, Andrea Tringali, Ioannis S Papanikolaou et al. Endoscopic biliary stenting: indications, choice of stents, and results: European Society of Gastrointestinal Endoscopy ESGE Clinical Guideline Updated October 2017. Endoscopy. 2018 Sep;50 9 910 930. Open 3. Yousuke Nakai, Hiroyuki Isayama, Hsiu-Po Wang et al. International consensus statements for endoscopic management of distal biliary stricture. J Gastroenterol Hepatol. 2020 Jun;35 6 967 979. Open https://web.pathway.md/diseases/rec5jsaxkrOGmSEpV 5/6 6/29/23, 3:17 AM Biliary stricture Pathway 4. Jean-Marc Dumonceau, Myriam Delhaye, Andrea Tringali et al. Endoscopic treatment of chronic pancreatitis: European Society of Gastrointestinal Endoscopy ESGE Guideline Updated August 2018. Endoscopy. 2019 Feb;51 2 179 193. Open 5. ASGE Standards of Practice Committee, Mouen A Khashab, Krishnavel V Chithadi et al. Antibiotic prophylaxis for GI endoscopy. Gastrointest Endosc. 2015 Jan;81 1 81 9. Open 6. ASGE Standards of Practice Committee, Krishnavel V Chathadi, Vinay Chandrasekhara et al. The role of ERCP in benign diseases of the biliary tract. Gastrointest Endosc. 2015 Apr;81 4 795 803. Open 7. Geoffroy Vanbiervliet, Marin Strijker, Marianna Arvanitakis et al. Endoscopic management of ampullary tumors: European Society of Gastrointestinal Endoscopy ESGE Guideline. Endoscopy. 2021 Apr;53 4 429 448. Open 8. A Vogel, J Bridgewater, J Edeline et al. Biliary tract cancer: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2023 Feb;34 2 127 140. Open 9. Jean-Marc Dumonceau, Christine Kapral, Lars Aabakken et al. ERCP-related adverse events: European Society of Gastrointestinal Endoscopy ESGE Guideline. Endoscopy. 2020 Feb;52 2 127 149. Open 10. Patterson TF, Thompson GR rd, Denning DW et al. Practice Guidelines for the Diagnosis and Management of Aspergillosis: 2016 Update by the Infectious Diseases Society of America. Clin Infect Dis. 2016 Aug 15;63 4):e1-e60. Open 11. Phonthep Angsuwatcharakon, Santi Kulpatcharapong, Jong H Moon et al. Consensus guidelines on the role of cholangioscopy to diagnose indeterminate biliary stricture. HPB Oxford). 2022 Jan;24 1 17 29. Open 12. D Wayne Overby, Keith N Apelgren, William Richardson et al. SAGES guidelines for the clinical application of laparoscopic biliary tract surgery. Surg Endosc. 2010 Oct;24 10 2368 86. Open 13. Chapman MH, Thorburn D, Hirschfield GM et al. British Society of Gastroenterology and UK PSC guidelines for the diagnosis and management of primary sclerosing cholangitis. Gut. 2019 Aug;68 8 1356 1378. Open 14. J W Valle, I Borbath, S A Khan et al. Biliary cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2016 Sep;27(suppl 5):v28-v37. Open 15. Expert Panel on Interventional Radiology:, Alexandra H Fairchild, Eric J Hohenwalter et al. ACR Appropriateness Criteria Radiologic Management of Biliary Obstruction. J Am Coll Radiol. 2019 May;16 5S S196 S213. Open 16. Bing Hu, Bo Sun, Qiang Cai et al. Asia-Pacific consensus guidelines for endoscopic management of benign biliary strictures. Gastrointest Endosc. 2017 Jul. Open https://web.pathway.md/diseases/rec5jsaxkrOGmSEpV 6/6
Guideline sources The following summarized guidelines for the evaluation and management of binge eating disorder (BED) are prepared by our editorial team based on guidelines from the American Psychiatric Association (APA 2023; 2006), the The Scottish Intercollegiate Guidelines Network (SIGN 2022), the U.S. Preventive Services Task Force (USPSTF 2022), the Canadian Practice Guidelines (CPG 2021; 2020), the American College of Endocrinology (ACE/AACE 2016), the American Association of Family Physicians (AAFP 2015), the American Academy of Child and Adolescent Psychiatry (AACAP 2015), and the Royal Australian and New Zealand College of Psychiatrists (RANZCP 2014). 1 2 3 4 5 6 7 8 9 10 Calculator DSM 5 diagnostic criteria for bin Guidelines https://web.pathway.md/diseases/recTfpNVEFkYzkUO6 1/6 6/29/23, 3:17 AM Binge eating disorder Pathway 1. Screening and diagnosis Indications for screening: As per APA 2023 guidelines, screen for the presence of an eating disorder as part of an initial psychiatric evaluation. B As per SIGN 2022 guidelines: Consider screening for the presence of eating disorders as part of the routine review of patients with T1DM. C Elicit history of current or past history of eating disorders in females during pregnancy and postnatal period, recognizing the potential barriers for disclosure. A As per USPSTF 2022 guidelines, insufficient evidence to assess the balance of benefits and harms of screening for eating disorders in adolescents and adults. I As per AACE 2016 guidelines, screen for BED and night eating syndrome in patients with overweight or obesity eligible for weight-loss therapy. B As per AACAP 2015 guidelines, screen all pediatric and adolescent patients for eating disorders seen by a mental health clinician, followed by a comprehensive diagnostic evaluation (including laboratory tests and imaging studies as indicated) if positive. B 2. Diagnostic investigations Initial evaluation: As per APA 2023 guidelines, assess the following during the initial evaluation of patients with a possible eating disorder: height and weight history (maximum and minimum weight, recent weight changes) presence of, patterns in, and changes in restrictive eating, food avoidance, binge eating, and other eating-related behaviors (rumination, regurgitation, chewing, and spitting) patterns and changes in food repertoire (breadth of food variety, narrowing or elimination of food groups) presence of, patterns in, and changes in compensatory and other weight control behaviors, including dietary restriction, compulsive or driven exercise, purging behaviors (laxative use, self-induced vomiting), and use of medication to manipulate weight percentage of time preoccupied with food, weight, and body shape prior treatment and response to treatment for an eating disorder psychosocial impairment secondary to eating or body image concerns or behaviors family history of eating disorders, other psychiatric illnesses, and other medical conditions (obesity, IBD, diabetes mellitus). B Show 2 more As per RANZCP 2014 guidelines, include the following in the assessment of adult patients with possible BED: https://web.pathway.md/diseases/recTfpNVEFkYzkUO6 2/6 6/29/23, 3:17 AM Binge eating disorder Pathway elicit history enquiring into any binge eating, dietary restriction and/or fasting, compulsive or driven exercise, or additional weight control behaviors assess for cognitions of weight and/or shape overvaluation, and body image and eating preoccupations assess for any past history of eating disorders or other psychiatric comorbidities perform a physical examination, including measurement of weight, height, BMI calculation, pulse rate, and BP consider obtaining cardiovascular evaluation and an ECG as clinically indicated consider obtaining serum biochemistry and random glucose as clinically indicated consider involving a general practitioner and/or dentist as appropriate. B Laboratory tests: obtain a CBC and a comprehensive metabolic panel including electrolytes, liver enzymes, and renal function tests in the laboratory assessment of patients with a possible eating disorder. B Electrocardiogram: obtain an ECG in patients with a restrictive eating disorder, severe purging behavior, or taking medications known to prolong the QTc interval. B Screening for comorbidities: As per AAFP 2015 guidelines, assess for psychiatric comorbidities, including depression and suicide risk, anxiety disorders, and substance use disorders, in patients with an eating disorder. B As per RANZCP 2014 guidelines, assess for any past history of eating disorders or other psychiatric comorbidities in patients with BED. B Screening for comobidities: as per APA 2023 guidelines, assess for co-occurring health conditions, including psychiatric disorders, in the initial psychiatric evaluation of patients with a possible eating disorder. B 3. Medical management Multidisciplinary care: As per APA 2023 guidelines, provide a documented, comprehensive, culturally appropriate, and person-centered treatment plan incorporating medical, psychiatric, psychological, and nutritional expertise, commonly via a coordinated multidisciplinary team, in patients with an eating disorder. B As per AACAP 2015 guidelines, ensure a multidisciplinary team approach (developmentally aware, sensitive, and skilled in the care of pediatric and adolescent patients with eating disorders) for the treatment of eating disorders in young patients. B As per AAFP 2015 guidelines, ensure an interdisciplinary team approach, often including a family physician, a psychotherapist or psychiatrist, a dietitian, an eating disorder specialist, and school personnel, for the treatment of patients with bulimia nervosa. B Setting of care: https://web.pathway.md/diseases/recTfpNVEFkYzkUO6 3/6 6/29/23, 3:17 AM Binge eating disorder Pathway As per AACAP 2015 guidelines: Offer outpatient psychosocial interventions as the initial treatment of choice in pediatric and adolescent patients with an eating disorder. B Consider offering psychiatric hospitalization, day programs, partial hospitalization programs, and residential programs for eating disorders in pediatric and adolescent patients only when outpatient interventions have been unsuccessful or are unavailable. C As per AAFP 2015 guidelines, assess medical stability and the need for hospitalization in the initial evaluation of patients with eating disorders. B As per RANZCP 2014 guidelines, consider admitting patients to an inpatient or day program unit if there is an increased risk of non-response to outpatient/community-based care. C Pharmacotherapy, general principles: As per SIGN 2022 guidelines, do not use medications either as an alternative or as an adjunct to psychological treatment in patients with BED. D As per AACAP 2015 guidelines, reserve medications, including complementary and alternative medications, for comorbid conditions and refractory cases. B As per RANZCP 2014 guidelines: Consider offering pharmacotherapy as an adjunctive treatment for an additive benefit. B Offer pharmacological treatment where psychological therapy is not available. A Pharmacotherapy, antidepressants: As per APA 2023 guidelines, offer antidepressants in adult patients with BED preferring medication or not responding to psychotherapy alone. B As per RANZCP 2014 guidelines: Consider offering SSRIs in patients with BED. B Monitor adverse effects of antidepressants and modify use as required. B Pharmacotherapy (stimulants): consider offering lisdexamfetamine in adult patients with BED preferring medication or not responding to psychotherapy alone. C Pharmacotherapy (anticonvulsants): Consider offering topiramate in patients with comorbid obesity. C Monitor adverse effects of antiepileptic agents and modify use as required. B Pharmacotherapy, obesity medications: As per RANZCP 2014 guidelines, consider offering orlistat for weight loss in patients with comorbid obesity. C As per APA 2006 guidelines, consider offering orlistat in addition to a guided self-help CBT program for additional weight reduction. C 4. Nonpharmacologic interventions Psychotherapy: https://web.pathway.md/diseases/recTfpNVEFkYzkUO6 4/6 6/29/23, 3:17 AM Binge eating disorder Pathway As per APA 2023 guidelines, offer eating disorder-focused CBT or interpersonal therapy, in either individual or group formats, in patients with BED. B As per SIGN 2022 guidelines: Consider offering CBT or interpersonal psychotherapy as first-line therapy in adult patients with BED. Consider offering dialectical-based therapy, integrative cognitive-affective therapy, brief strategic therapy, or schema therapy if CBT or interpersonal psychotherapy are ineffective, unsuitable, or unacceptable. C Consider offering dialectical behavior therapy in patients with comorbid substance misuse disorder or emotionally unstable personality disorder. C Consider offering CBT, interpersonal psychotherapy, or family-based interventions in adolescent patients with BED. B As per RANZCP 2014 guidelines, offer individual psychological therapy (therapist-led CBT or enhanced CBT; alternatively, CBT adapted for internet delivery or in guided self-help form) as first-line therapy in adult patients with BED. A Technology-based interventions: As per SIGN 2022 guidelines, consider offering psychological assessments and treatment via videoconferencing as an adjunct or alternative to in-person sessions when there are barriers to accessing in-person sessions. C As per CPG 2021 guidelines, offer internet CBT-based guided self-help and relapse prevention in emerging adult patients with BED. A Show 4 more Yoga: advise practicing, yoga in addition to standard treatments, in medically stable young patients with other specified feeding and eating disorders. B 5. Specific circumstances Pregnant patients: elicit history of current or past history of eating disorders in females during pregnancy and postnatal period, recognizing the potential barriers for disclosure. A Show 13 more Clinical findings Symptoms Past medical history Anxiety Anorexia nervosa Binge eating Bulimia nervosa Body image dissatisfaction Depression Eating unusually large amounts of food Metabolic syndrome Obesity Purging behavior Unable to stop eating https://web.pathway.md/diseases/recTfpNVEFkYzkUO6 5/6 6/29/23, 3:17 AM Binge eating disorder Pathway Social history Dietary restrictions References 1. American Psychiatric Association. Treatment of patients with eating disorders,third edition. American Psychiatric Association. Am J Psychiatry. 2006 Jul;163 7 Suppl):4 54. Open 2. Phillipa Hay, David Chinn, David Forbes et al. Royal Australian and New Zealand College of Psychiatrists clinical practice guidelines for the treatment of eating disorders. Aust N Z J Psychiatry. 2014 Nov;48 11 977 1008. Open 3. Catherine Crone, Laura J Fochtmann, Evelyn Attia et al. The American Psychiatric Association Practice Guideline for the Treatment of Patients With Eating Disorders. Am J Psychiatry. 2023 Feb 1;180 2 167 171. Open 4. Scottish Intercollegiate Guidelines Network. SIGN 164 Eating disorders. SIGN. 2022 Aug. Open 5. Brian C Harrington, Michelle Jimerson, Christina Haxton et al. Initial Evaluation, Diagnosis, and Treatment of Anorexia Nervosa and Bulimia Nervosa. Am Fam Physician. 2015 Jan 1;91 1 46 52. Open 6. US Preventive Services Task Force, Karina W Davidson, Michael J Barry et al. Screening for Eating Disorders in Adolescents and Adults: US Preventive Services Task Force Recommendation Statement. JAMA. 2022 Mar 15;327 11 1061 1067. Open 7. James Lock, Maria C La Via, American Academy of Child and Adolescent Psychiatry AACAP Committee on Quality Issues CQI . Practice parameter for the assessment and treatment of children and adolescents with eating disorders. J Am Acad Child Adolesc Psychiatry. 2015 May;54 5 412 25. Open 8. Jennifer Couturier, Danielle Pellegrini, Catherine Miller et al. The COVID 19 pandemic and eating disorders in children, adolescents, and emerging adults: virtual care recommendations from the Canadian consensus panel during COVID 19 and beyond. J Eat Disord. 2021 Apr 16;9 1 46. Open 9. Jennifer Couturier, Leanna Isserlin, Mark Norris et al. Canadian practice guidelines for the treatment of children and adolescents with eating disorders. J Eat Disord. 2020 Feb 1;8 4. Open 10. W Timothy Garvey, Jeffrey I Mechanick, Elise M Brett et al. AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS AND AMERICAN COLLEGE OF ENDOCRINOLOGY COMPREHENSIVE CLINICAL PRACTICE GUIDELINES FOR MEDICAL CARE OF PATIENTS WITH OBESITY. Endocr Pract. 2016 Jul;22 Suppl 3 1 203. Open 11. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, Text Revision DSM 5 TR ). APA. 2022. Open https://web.pathway.md/diseases/recTfpNVEFkYzkUO6 6/6
Guideline sources The following summarized guidelines for the evaluation and management of BK polyomavirus- associated hemorrhagic cystitis (BKPyV-HC) are prepared by our editorial team based on guidelines from the European Conference on Infections in Leukaemia (ECIL 2018) and the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2009). 1 2 Calculator Grading of hemorrhagic cystitis Guidelines 1. Screening and diagnosis Indications for screening: https://web.pathway.md/diseases/recCrN0g15IjpUo0g 1/4 6/29/23, 3:15 AM BK polyomavirus-associated hemorrhagic cystitis Pathway Do not obtain screening for BKPyV-HC in asymptomatic patients at risk following HSCT, as pre- emptive therapy is not established. D Consider screening all kidney transplant recipients for BK virus infection with quantitative serum NAAT C at least: monthly for the first 3-6 months after transplantation C then every 3 months until the end of the first post-transplant year C whenever there is an unexplained rise in serum creatinine C after treatment for acute rejection. C Diagnostic criteria: Diagnose BKPyV-HC based on the clinical and laboratory triad, while also excluding the significant role of other etiologies: clinical symptoms/signs of cystitis, such as dysuria and lower abdominal pain 2 grade hematuria serum BK polyomavirus loads > 7 log10 gEq/mL. B Consider using plasma BK polyomavirus loads > 1,000 gEq/mL in the management and follow- up of triad-positive patients following allogeneic HSCT. C 2. Respiratory support Hyperbaric oxygen therapy: consider offering hyperbaric oxygen therapy aimed at speeding the healing process of the damaged urothelial lining in patients with BKPyV-HC following HSCT. C 3. Medical management Reduction of immunosuppression: As per ECIL 2018 guidelines, insufficient evidence to provide the risk/benefit ratio of reduction of immunosuppression in patients with BKPyV-HC following HSCT. Balance the reduction of immunosuppression with the risk of worsening or triggering acute GvHD. I As per KDIGO 2009 guidelines, consider reducing immunosuppressive medications when BK virus serum NAAT is persistently > 10,000 copies/mL (107 copies/L) in kidney transplant recipients. C Supportive therapy: offer hyperhydration and perform bladder irrigation and platelet transfusions as needed to reduce bleeding and pain in patients with BKPyV-HC following HSCT. B Antiviral therapy: consider administering IV cidofovir until the availability of safe and effective antiviral treatment, recognizing that there is uncertainty of efficacy, the best dose schedule and the need to balance any benefit against its renal side effects. C https://web.pathway.md/diseases/recCrN0g15IjpUo0g 2/4 6/29/23, 3:15 AM BK polyomavirus-associated hemorrhagic cystitis Pathway Agents with no evidence for benefit: insufficient evidence to support the use of intravesical sodium hyaluronate, IV FXIII concentrate, leflunomide, estrogens, mesenchymal cells, and cellular immunotherapy. I 4. Therapeutic procedures Fibrin glue: consider applying fibrin glue aimed at speeding the healing process of the damaged urothelial lining in patients with BKPyV-HC following HSCT. C 5. Preventative measures Prophylaxis: Consider offering hyperhydration C and performing bladder irrigation for BKPyV-HC prophylaxis aiming to reduce urothelial damage, particularly when using myeloablative conditioning based on cyclophosphamide, busulfan and total body irradiation. C Recognize that specific antiviral prophylaxis is not available. Do not use fluoroquinolones given the lack of significant effects on BK polyomavirus replication and hemorrhagic cystitis severity, and the selection of antibiotic resistance. B Clinical findings Symptoms Lab findings Burning on urination serum BK virus deoxyRNA Dysuria Hematuria Penile pain Suprapubic pain Urinary frequency Urinary incontinence Urinary urgency Endoscopic findings Bladder wall telangiectasia References 1. Simone Cesaro, Tina Dalianis, Christine Hanssen Rinaldo et al. ECIL guidelines for the prevention, diagnosis and treatment of BK polyomavirus-associated haemorrhagic cystitis in haematopoietic stem cell transplant recipients. J Antimicrob Chemother. 2018 Jan 1;73 1 12 21. Open https://web.pathway.md/diseases/recCrN0g15IjpUo0g 3/4 6/29/23, 3:15 AM BK polyomavirus-associated hemorrhagic cystitis Pathway 2. Kidney Disease: Improving Global Outcomes KDIGO Transplant Work Group. KDIGO clinical practice guideline for the care of kidney transplant recipients. Am J Transplant. 2009 Nov;9 Suppl 3 S1 155. Open 3. A Thompson, A Adamson, A Bahl et al. Guidelines for the diagnosis, prevention and management of chemical- and radiation-induced cystitis. J Clin Urol. 2014 Jan;7 1 25 35. Open 4. Jessica H Hannick, Martin A Koyle. Canadian Urological Association Best Practice Report: Pediatric hemorrhagic cystitis. Can Urol Assoc J. 2019 Nov;13 11 E325 E334. Open https://web.pathway.md/diseases/recCrN0g15IjpUo0g 4/4
Guideline sources The following summarized guidelines for the evaluation and management of bladder cancer (BC) are prepared by our editorial team based on guidelines from the European Association of Urology (EAU 2023; 2021), the European Society of Medical Oncology (ESMO 2022; 2014), the American Urological Association (AUA/SUO/ASTRO/ASCO 2017), the American Urological Association (AUA/SUO 2016), and the U.S. Preventive Services Task Force (USPSTF 2011). 1 2 3 4 5 6 7 https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 1/13 6/29/23, 3:18 AM Bladder cancer Pathway Calculator Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s TNM class Guidelines 1. Screening and diagnosis Indications for screening: As per EAU 2023 guidelines, take into account the increased risk of developing BC in patients undergoing external-beam radiation therapy, brachytherapy, or a combination of those, during follow-up. Follow-up closely patients treated with radiation at a young age, as they are at the greatest risk and BC requires time to develop. B As per ESMO 2014 guidelines, do not obtain screening for BC on a population level for improving survival. D As per USPSTF 2011 guidelines, insufficient to recommend screening for BC in asymptomatic adults. A 2. Classification and risk stratification Risk factors: recognize that there are several risk factors connected with the risk of BC, with tobacco smoking as the most important one. B Staging: As per ESMO 2022 guidelines, classify patients with non-muscle-invasive BC into 4 risk categories (low risk, intermediate risk, high risk, and very high risk) based on tumor characteristics for further treatment and follow-up recommendations. B As per EAU 2021 guidelines, classify the depth of invasion (staging) according to the TNM classification. A Show 3 more As per AUA 2016 guidelines, assign a clinical stage and classify patients accordingly as low-, intermediate-, or high-risk at the time of each occurrence/recurrence. B 3. Diagnostic investigations History and physical examination: Use validated questionnaires to assess health-related quality of life in patients with muscle- invasive BC, both at baseline and post-treatment. A https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 2/13 6/29/23, 3:18 AM Bladder cancer Pathway As per ESMO 2022 guidelines, evaluate painless hematuria in all patients as it is the most common presenting symptom in BC. B As per EAU 2021 guidelines, elicit a patient history, focusing on urinary tract symptoms and hematuria. A Elicit a full history and perform physical examination including an examination under anesthesia at the time of transurethral resection of bladder tumor before treatment consideration in patients with suspected invasive cancer. B Urinary tract imaging: As per ESMO 2022 guidelines, obtain cross-sectional upper tract imaging (CT/MRI urography) to screen for synchronous upper urinary tract urothelial carcinoma in cases of high-grade BC. B As per EAU 2021 guidelines: Obtain renal and bladder ultrasound and/or CT-intravenous urography in the initial evaluation of patients with hematuria. A Obtain CT urography in selected patients with bladder tumor (such as tumors located in the trigone, multiple- or high-risk tumors). A As per AUA 2016 guidelines: Obtain upper urinary tract imaging in the initial evaluation of patients with BC. B Consider obtaining upper tract imaging in patients with a history of non-muscle-invasive BC with a normal cystoscopy and positive cytology. E Imaging for staging: As per EAU 2023 guidelines: Obtain CT of the chest, abdomen, and pelvis for staging, including some form of CT urography with designated phases for optimal urothelial evaluation, in patients with confirmed muscle- invasive BC. A Obtain CT urography unless contraindicated for reasons related to contrast administration or radiation dose. Obtain MRI if CT urography is contraindicated. A As per ESMO 2022 guidelines: Obtain further imaging, such as contrast-enhanced CT of the chest-abdomen-pelvis or MRI of the abdomen-pelvis combined with chest CT, for regional and distant staging in patients with invasive disease ( T1). B Consider obtaining FDG-positron emission tomography-CT for the detection of lymph node and distant metastases. C As per AUA 2017 guidelines, obtain chest imaging and cross-sectional imaging of the abdomen and pelvis with IV contrast, if not contraindicated, as part of complete staging evaluation in patients with muscle-invasive BC. B Laboratory tests: obtain a comprehensive metabolic panel (CBC, LFTs, ALP and renal function) in patients with muscle-invasive BC. B https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 3/13 6/29/23, 3:18 AM Bladder cancer Pathway 4. Diagnostic procedures Cystoscopy and ureteroscopy: As per EAU 2023 guidelines: Perform cystoscopy for the diagnosis of BC. A Describe all macroscopic features of the tumor (site, size, number, and appearance) and mucosal abnormalities during cystoscopy. Use a bladder diagram. A As per EAU 2021 guidelines, perform cystoscopy for the diagnosis of BC. A Show 2 more As per AUA 2016 guidelines, perform a thorough cystoscopic examination of the urethra and bladder at the time of resection to evaluate and document tumor size, location, configuration, number and mucosal abnormalities in patients with suspected BC. B Show 3 more Cytology: As per EAU 2023 guidelines, recognize that urinary cytology has high sensitivity in high-grade tumors, including carcinoma in situ. B As per ESMO 2022 guidelines, consider obtaining urine cytology to facilitate the diagnosis of high-grade urothelial carcinoma, but do not use it as the primary method of histological diagnosis. C Use the Paris system for reporting. As per EAU 2021 guidelines, obtain voided urine cytology as an adjunct to cystoscopy to detect high-grade tumor. A Show 3 more Biopsy and histopathology: As per EAU 2023 guidelines, record the following in the pathology report: depth of invasion (categories pT2a and pT2b, pT3a and pT3b, or pT4a and pT4b) margins with special attention paid to the radial margin, prostate, ureter, urethra, peritoneal fat, uterus, and vaginal vault total number of lymph nodes, the number of positive lymph nodes, and extranodal spread lymphovascular invasion presence of carcinoma in situ sampling sites and information on tumor size when providing specimens to the pathologist. A Show 2 more As per ESMO 2022 guidelines, diagnose BC based on cystoscopic examination of the bladder and histological evaluation of tissue obtained either with cold-cup biopsy or transurethral resection of the bladder tumor. Perform complete resection of all tumor tissue when possible. Include muscle tissue in the biopsies except when a Ta/LG is expected. B Show 2 more As per EAU 2021 guidelines, exclude an upper tract urothelial carcinoma, carcinoma in situ in the bladder (by mapping biopsies or photodynamic diagnosis-guided biopsies) and tumor in the https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 4/13 6/29/23, 3:18 AM Bladder cancer Pathway prostatic urethra (by prostatic urethra biopsy) in patients with positive cytology but negative cystoscopy. A As per AUA 2017 guidelines, review the pathology by an experienced genitourinary pathologist if variant histology is suspected or if muscle invasion is equivocal (such as, micropapillary, nested, plasmacytoid, neuroendocrine, sarcomatoid, extensive squamous or glandular differentiation). B As per AUA 2016 guidelines: Consider performing prostatic urethral biopsies and random bladder biopsies in patients with a history of non-muscle-invasive BC with a normal cystoscopy and positive cytology. E Review the pathology of a patient with any doubt in regards to variant or suspected variant histology (such as micropapillary, nested, plasmacytoid, neuroendocrine, sarcomatoid), extensive squamous or glandular differentiation, or the presence/absence of lymphovascular invasion by an experienced genitourinary pathologist. B As per ESMO 2014 guidelines, perform biopsy of the prostatic urethra if the tumor is located at the trigone or bladder neck area, or if there is no bladder tumor and the procedure is performed to study a positive cytology, since the tumor could be located in the urothelium lining the prostatic urethra or the ducts. B Molecular testing: As per EAU 2023 guidelines: Insufficient evidence to support the use of tumor mutational burden, molecular subtypes, immune or other gene expression signatures for the management of patients with urothelial cancer. Evaluate for FGFR2/3 genetic alterations for the potential use of erdafitinib in patients with locally advanced or metastatic urothelial carcinoma progressed after platinum-containing chemotherapy (including within 12 months of neoadjuvant or adjuvant platinum-containing chemotherapy). B As per ESMO 2022 guidelines, do not obtain molecular diagnostics such as The Cancer Genome Atlas classification and PD-L1 status for all tumors. D 5. Medical management Management of non-muscle-invasive cancer (general principles): follow a risk-stratified approach with transurethral resection of the bladder tumor and intravesical chemotherapy or BCG in intermediate- and high-risk patients with non-muscle-invasive BC. A Management of non-muscle-invasive cancer, intravesical chemotherapy: As per EAU 2021 guidelines, offer one immediate chemotherapy instillation in patients with low- risk tumors and in patients with small papillary recurrences (presumably Ta LG/G1) detected > 1 year after previous transurethral resection of the bladder. A Show 8 more https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 5/13 6/29/23, 3:18 AM Bladder cancer Pathway As per SUO/AUA 2016 guidelines, consider administering a single postoperative instillation of intravesical chemotherapy (such as mitomycin C or epirubicin) within 24 hours of transurethral resection of bladder tumor in patients with suspected or known low- or intermediate-risk BC. C Show 4 more Management of non-muscle-invasive cancer, intravesical BCG immunotherapy: As per EAU 2021 guidelines, offer 1-year full-dose BCG treatment (induction plus 3-weekly instillations at 3, 6 and 12 months) or instillations of chemotherapy (the optimal schedule is not known) for a maximum 1 year in patients with intermediate-risk tumors (with or without immediate instillation). Decide on the final treatment option based on individual patient's risk of recurrence and progression and the efficacy and side effects of each treatment modality. A Show 3 more As per SUO/AUA 2016 guidelines, administer a 6-week induction course of BCG in high-risk patients with newly diagnosed carcinoma in situ, high-grade T1, or high-risk Ta urothelial carcinoma. B Show 2 more Management of non-muscle-invasive cancer (salvage therapy): consider offering pembrolizumab or nadofaragene firadenovec in patients unresponsive to BCG and ineligible for or refusing cystectomy. C Offer a multidisciplinary approach in these patients. C Management of muscle-invasive cancer, general principles: As per EAU 2023 guidelines: Differentiate between urachal and non-urachal subtypes of adenocarcinoma for treatment decisions. E Evaluate patients being candidates for curative treatment, such as cystectomy or bladder preservation, by at least an oncologist, a urologist, a radiation oncologist (in case adjuvant radiotherapy or bladder preservation is considered), and a neutral healthcare professional, such as a specialist nurse. E As per ESMO 2022 guidelines, ensure multidisciplinary care via tumor board discussions and/or directed consultations with a medical oncologist, radiation oncologist, and urologist for the optimal management of BC. B As per SUO/ASTRO/ASCO/AUA 2017 guidelines: Discuss curative treatment options with patients with newly diagnosed muscle-invasive BC before determining a plan of therapy based on both patient comorbidity and tumor characteristics. Evaluate patients using a multidisciplinary approach. B Take into account unique clinical characteristics of patients with variant histology that may require divergence from standard evaluation and management for urothelial carcinoma. E Management of muscle-invasive cancer, bladder-preserving therapy: As per EAU 2023 guidelines, do not offer bladder-preserving therapy in patients with carcinoma in situ. D Show 7 more As per ESMO 2022 guidelines: https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 6/13 6/29/23, 3:18 AM Bladder cancer Pathway Consider offering bladder-preserving therapy with radiotherapy as part of multimodal therapy for muscle-invasive BC in patients seeking an alternative to radical cystectomy and as an option in patients medically unfit for surgery. C Utilize a tri-modality combination of transurethral resection of the bladder tumor, radiotherapy, and chemotherapy in contemporary organ-preservation protocols. B As per SUO/ASTRO/ASCO/AUA 2017 guidelines, offer bladder-preserving therapy, if clinically appropriate, in patients with newly diagnosed non-metastatic muscle-invasive BC desiring retaining their bladder and in patients with significant comorbidities when radical cystectomy is not a treatment option. B Show 3 more Management of muscle-invasive cancer, chemotherapy: As per EAU 2023 guidelines, offer neoadjuvant cisplatin-based combination chemotherapy in cisplatin-eligible patients with muscle-invasive BC (T2-T4a, cN0 M0). A Show 4 more As per ESMO 2022 guidelines, offer 3-4 cycles of cisplatin-based neoadjuvant chemotherapy in patients with muscle-invasive BC. A Obtain cross-sectional imaging after chemotherapy before radical cystectomy. B Show 3 more As per SUO/ASTRO/ASCO/AUA 2017 guidelines, offer cisplatin-based neoadjuvant chemotherapy utilizing a multidisciplinary approach in patients eligible for radial cystectomy. B Show 2 more Management of muscle-invasive cancer, radiotherapy: As per EAU 2023 guidelines, do not offer preoperative radiotherapy in patients with operable muscle-invasive BC, as it will only result in downstaging but will not improve survival. D Show 3 more As per ESMO 2022 guidelines, do not offer adjuvant radiotherapy (with or without radiosensitizing chemotherapy) as a standard treatment in patients with muscle-invasive BC. D As per SUO/ASTRO/ASCO/AUA 2017 guidelines, do not offer radiotherapy alone as a curative treatment in patients with muscle-invasive BC. D Management of muscle-invasive cancer, palliative therapy: As per EAU 2023 guidelines, offer radical cystectomy as a palliative treatment in patients with locally-advanced tumors (T4b). B Show 3 more As per ESMO 2022 guidelines, consider offering palliative radiotherapy for palliation (bleeding, pain). C Management of metastatic cancer: As per EAU 2023 guidelines, offer cisplatin-containing combination chemotherapy with gemcitabine and cisplatin regimen or high-dose intensity methotrexate, vinblastine, adriamycin, and cisplatin regimen as first-line therapy in platinum-fit patients with metastatic cancer. A Show 7 more https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 7/13 6/29/23, 3:18 AM Bladder cancer Pathway As per ESMO 2022 guidelines, offer cisplatin-based chemotherapy followed by maintenance avelumab as first-line therapy in patients with advanced or metastatic urothelial carcinoma not progressing on chemotherapy and fit enough to tolerate cisplatin-based combination chemotherapy. A Show 2 more Management of persistent/recurrent disease (general principles): offer pembrolizumab in patients with progression of disease after platinum-based chemotherapy. A Consider offering other immune checkpoint inhibitors, such as atezolizumab. B Show 5 more Management of persistent/recurrent disease, non-muscle-invasive cancer: As per EAU 2021 guidelines, consider performing outpatient fulguration or laser vaporization in patients with small papillary recurrences with a history of TaG1/LG tumors. C As per SUO/AUA 2016 guidelines, consider performing prostatic urethral biopsy and an upper tract evaluation before administration of additional intravesical therapy in intermediate- or high- risk patients with persistent or recurrent disease or positive cytology following intravesical therapy. C Show 6 more As per ESMO 2014 guidelines, perform cystoscopy as the most cost-effective method to detect BC recurrence in patients with non-muscle-invasive BC, despite the fact that it is an invasive and relatively expensive procedure. B Management of persistent/recurrent disease, muscle-invasive cancer: As per EAU 2023 guidelines, offer radiotherapy, chemotherapy and possibly surgery as options of treatment, either alone or in combination, in patients with local recurrence. A As per SUO/ASTRO/ASCO/AUA 2017 guidelines: Offer cystectomy with bilateral pelvic lymphadenectomy in medically fit patients with residual or recurrent muscle-invasive disease after bladder-preserving therapy. B Consider offering either local procedures, such as transurethral resection of bladder tumor with intravesical therapy or radical cystectomy with bilateral pelvic lymphadenectomy in patients with non-muscle-invasive BC recurrence after bladder-preserving therapy. C As per ESMO 2014 guidelines, perform prompt salvage cystectomy in patients received bladder-preserving therapy, when possible, if persistent or recurrent disease is observed at response evaluation or during follow-up (cystoscopy and urinary cytology every 3 months during the first 2 years, and every 6 months thereafter). B Management of persistent/recurrent disease, metastatic cancer: As per EAU 2023 guidelines, offer chemotherapy as the first option in patients with distant recurrence. Consider offering metastasectomy or radiotherapy in case of unique metastasis site. A As per ESMO 2014 guidelines, consider offering vinflunine as second-line therapy in patients progressing after first-line treatment with platinum-containing combination chemotherapy for metastatic disease. B https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 8/13 6/29/23, 3:18 AM Bladder cancer Pathway 6. Nonpharmacologic interventions Smoking cessation: As per EAU 2023 guidelines, counsel patients to stop active smoking and avoid passive smoking. A As per EAU 2021 guidelines, advise smoking cessations in smoker patients with confirmed non- muscle-invasive bladder. A 7. Perioperative care Perioperative care: As per EAU 2023 guidelines, do not offer preoperative bowel preparation. D Show 2 more As per ASTRO 2017 guidelines, attempt to optimize patient performance status in the perioperative setting. E Show 2 more 8. Surgical interventions Transurethral resection of tumor, non-muscle invasive cancer: As per EAU 2021 guidelines, perform transurethral resection of bladder tumor and pathology investigation of the obtained specimens as a diagnostic procedure and initial treatment step in patients with suspected BC. A Show 17 more As per SUO/AUA 2016 guidelines, perform complete visual resection of the bladder tumor at initial diagnosis in patients with BC, if technically feasible. B Show 4 more As per ESMO 2014 guidelines: Perform transurethral resection of the bladder tumor as the treatment of choice in patients with any initial non-muscle-invasive bladder tumor, followed by instillations according to risk stratification of non-muscle-invasive BC. A Consider performing a second transurethral resection of the bladder tumor in patients with high-risk non-muscle-invasive BC, either before intravesical therapy C or thereafter. C Transurethral resection of tumor (muscle invasive cancer): do not perform transurethral resection of bladder tumor alone as a curative treatment option, as most patients will not benefit. D Radical cystectomy, non-muscle-invasive cancer: As per ESMO 2022 guidelines, offer radical cystectomy in patients with very high-risk disease. Perform radical cystectomy in patients with carcinoma in situ or high-grade T1 unresponsive to BCG due to the high risk of progression. B https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 9/13 6/29/23, 3:18 AM Bladder cancer Pathway As per EAU 2021 guidelines: Perform immediate radical cystectomy in patients with very high-risk tumors. Offer intravesical full-dose BCG instillations for 1-3 years in patients with very-high risk tumors refusing or unfit for radical cystectomy. A Offer radical cystectomy in patients with BCG unresponsive tumors. A View treatments other than radical cystectomy as oncologically inferior in patients with BCG unresponsive tumors. B As per SUO/AUA 2016 guidelines, consider offering initial radical cystectomy in patients with variant histology because of the high rate of associated upstaging. E Show 2 more Radical cystectomy, muscle-invasive cancer: As per EAU 2023 guidelines, inform patients of the advantages and disadvantages of open radical cystectomy and robot-assisted radical cystectomy to allow selection of the proper procedure. A Show 7 more As per ESMO 2022 guidelines, offer radical cystectomy with standard pelvic lymph node dissection as first-line treatment in patients with muscle-invasive BC (T2-T4a, N0, M0). A As per SUO/ASTRO/ASCO/AUA 2017 guidelines, perform radical cystectomy as soon as possible following completion of and recovery from neoadjuvant chemotherapy. E Show 3 more Pelvic lymphadenectomy: As per EAU 2023 guidelines, perform lymph node dissection as an integral part of radical cystectomy in patients with muscle-invasive BC. A As per ASTRO 2017 guidelines: Perform bilateral pelvic lymphadenectomy at the time of any surgery with curative intent. B Remove at a minimum the external and internal iliac and obturator lymph nodes (standard lymphadenectomy) during bilateral pelvic lymphadenectomy. B As per ESMO 2014 guidelines, consider performing extended lymphadenectomy in patients with metastasis or micro-metastasis to a few nodes. B Urinary diversion: discuss the type of urinary diversion, taking into account the patient preference, existing comorbidities, tumor variables, and coping abilities. A Show 4 more Transurethral resection of the prostate: offer TURP, followed by intravesical instillation of BCG in patients with carcinoma in situ in the epithelial lining of the prostatic urethra. B Sexual-preserving surgery: As per EAU 2023 guidelines, offer sexual-preserving techniques in eligible male patients very motivated to preserve their sexual function. A Show 3 more https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 10/13 6/29/23, 3:18 AM Bladder cancer Pathway As per ASTRO 2017 guidelines, discuss and offer sexual function-preserving procedures in patients with organ-confined disease and absence of bladder neck, urethra and prostate involvement. B 9. Specific circumstances Elderly patients: screen for frailty and cognitive impairment and provide a Comprehensive Geriatric Assessment where optimization is needed. B Show 2 more Patients with upper urinary tract cancer: offer kidney-sparing management in patients with low- risk upper urinary tract urothelial carcinoma and radical nephroureterectomy with bladder cuff excision in patients with high-risk upper urinary tract urothelial carcinoma. B Show 5 more 10. Patient education General counseling: As per EAU 2023 guidelines, inform patients of the advantages and disadvantages of open radical cystectomy and robot-assisted radical cystectomy to allow selection of the proper procedure. A Show 2 more As per ASTRO 2017 guidelines, counsel patients regarding complications and the implications of treatment on quality of life (such as impact on continence, sexual function, fertility, bowel dysfunction, metabolic problems) before treatment. B Show 3 more 11. Preventative measures Avoidance of carcinogenic substances: Inform workers in potentially hazardous workplaces of the potential carcinogenic effects of a number of recognized substances, including duration of exposure and latency periods. Advise protective measures. A Do not prescribe pioglitazone in patients with active BC or a history of BC. D 12. Follow-up and surveillance Surveillance, non-muscle-invasive cancer: As per EAU 2021 guidelines, use the 2006 EORTC scoring model to predict the risk of tumor recurrence in individual patients not treated with BCG. A Show 9 more https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 11/13 6/29/23, 3:18 AM Bladder cancer Pathway As per SUO/AUA 2016 guidelines, consider obtaining surveillance upper tract imaging at 1-2 year intervals in intermediate- or high-risk patients. E Show 8 more Surveillance, muscle-invasive cancer: As per EAU 2023 guidelines, obtain regular follow-up in patients after curative radical cystectomy. E Show 13 more As per SUO/ASTRO/ASCO/AUA 2017 guidelines, obtain regular surveillance with CT, cystoscopy and urine cytology after completion of bladder-preserving therapy. B Show 3 more Surveillance (metastatic cancer): do not offer preventative brain radiation to avoid brain recurrence in patients with muscle-invasive small cell neuroendocrine variant of bladder urothelial carcinoma. D Clinical findings Symptoms Past medical history Abdominal pain Bladder schistosomiasis Dysuria Bone metastasis Feeling of incomplete bladder emptying Liver metastasis Lung metastasis Flank pain Pelvic radiation therapy Hematuria Prostate cancer Loss of appetite UTI Pelvic pain Vascular exam Urinary frequency Urinary hesitancy Lower extremity edema Urinary straining Urinary urgency Genitourinary exam Weak urine stream Macroscopic hematuria Weight loss Social history Tobacco use Abdominal exam Palpable pelvic mass Integument exam Pallor https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 12/13 6/29/23, 3:18 AM Bladder cancer Pathway References 1. J.A. Witjes Chair), H.M. Bruins, A. Carri n et al. EAU Guidelines on Muscle-invasive and Metastatic Bladder Cancer. EAU. 2023 Mar. Open 2. Sam S Chang, Bernard H Bochner, Roger Chou et al. Treatment of Non-Metastatic Muscle-Invasive Bladder Cancer: AUA/ASCO/ASTRO/SUO Guideline. J Urol. 2017 Sep;198 3 552 559. Open 3. M Moschini, G Gandaglia, F Deh et al. Bladder cancer: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2022 May;33 5 561. Open 4. J Bellmunt, A Orsola, J J Leow et al. Bladder cancer: ESMO Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2014 Sep;25 Suppl 3:iii40 8. Open 5. Sam S Chang, Stephen A Boorjian, Roger Chou et al. Diagnosis and Treatment of Non-Muscle Invasive Bladder Cancer: AUA/SUO Guideline. J Urol. 2016 Oct;196 4 1021 9. Open 6. M. Babjuk, M. Burger, E. Comp rat et al. EAU Guidelines on non-muscle-invasive bladder cancer TaT1 and CIS . EAU Guidelines. 2021 Mar. Open 7. Moyer VA, U.S. Preventive Services Task Force. Screening USPSTF for bladder cancer: U.S. Preventive Services Task Force recommendation statement. Ann Intern Med. 2011 Aug 16;155 4 246 51. Open 8. van Hooft JE, van Halsema EE, Vanbiervliet G et al. Self-expandable metal stents for obstructing colonic and extracolonic cancer: European Society of Gastrointestinal Endoscopy ESGE Clinical Guideline. Endoscopy. 2014 Nov;46 11 990 1053. Open 9. Coleman R, Body JJ, Aapro M et al. Bone health in cancer patients: ESMO Clinical Practice Guidelines. Ann Oncol. 2014 Sep;25 Suppl 3:iii124 37. Open 10. Koren G, Carey N, Gagnon R et al. Cancer chemotherapy and pregnancy. J Obstet Gynaecol Can. 2013 Mar;35 3 263 278. Open 11. Flank J, Robinson PD, Holdsworth M et al. Guideline for the Treatment of Breakthrough and the Prevention of Refractory Chemotherapy-Induced Nausea and Vomiting in Children With Cancer. Pediatr Blood Cancer. 2016 Jul;63 7 1144 51. Open https://web.pathway.md/diseases/recvI2mkZpuJOXOaw 13/13
Guideline sources The following summarized guidelines for the evaluation and management of bladder stones are prepared by our editorial team based on guidelines from the European Association of Urology (EAU 2020). 1 Guidelines 1. Diagnostic investigations Initial evaluation: obtain ultrasound as first-line imaging in patients with symptoms suggestive of a bladder stone. A Show 4 more 2. Medical management https://web.pathway.md/diseases/recbwiiE3D61W3LzU 1/3 6/29/23, 3:18 AM Bladder stones Pathway Oral chemolysis: offer oral chemolysis in adult patients with radiolucent or known uric acid bladder stones. B 3. Therapeutic procedures Transurethral cystolithotripsy: Offer transurethral cystolithotripsy in adult patients with bladder stones, where possible. A Perform transurethral cystolithotripsy with a continuous flow instrument (such as nephroscope or resectoscope) in adult patients, where possible. B Percutaneous cystolithotripsy: offer percutaneous cystolithotripsy if transurethral cystolithotripsy is not possible or advisable. A Shock wave lithotripsy: consider offering open, laparoscopic, and extracorporeal shock wave lithotripsy as alternative treatments in adult patients, if endoscopic treatment is not advisable. C Procedures for bladder outlet obstruction: perform procedures for the stone and underlying bladder outlet obstruction simultaneously, where possible, in adult patients with bladder stones secondary to bladder outlet obstruction. A 4. Surgical interventions Surgical stone removal: consider offering open cystolithotomy as an option in adult patients with very large bladder stones. C 5. Specific circumstances Pediatric patients: offer transurethral cystolithotripsy in pediatric patients with bladder stones, where possible. B Show 4 more 6. Preventative measures Prevention of stone recurrence: offer regular irrigation therapy with saline solution to reduce the risk of stone recurrence in adult and pediatric patients with bladder augmentation, continent cutaneous urinary reservoir or neuropathic bladder dysfunction and no history of autonomic dysreflexia. B 7. Follow-up and surveillance Serial imaging assessment: individualize follow-up imaging for each patient. Recogne that factors affecting follow-up include whether the underlying functional predisposition to stone formation can https://web.pathway.md/diseases/recbwiiE3D61W3LzU 2/3 6/29/23, 3:18 AM Bladder stones Pathway be treated (such as transurethral resection of prostate) and metabolic risk. B Clinical findings Symptoms Past medical history Dysuria Benign prostatic hyperplasia Feeling of incomplete bladder emptying Bladder outlet obstruction Kidney stones Hematuria Imaging findings Penile pain Scrotal pain Bladder stones Sudden voiding stoppage Suprapubic pain Urinary retention Weak urine stream Abdominal exam Bladder distension References 1. Thomas Hughes, Hui Ching Ho, Amelia Pietropaolo et al. EAU Guidelines on Bladder Stones. Turk J Urol. 2020 Nov; 46 Suppl 1 S104 S112. Open 2. Stephen W. Leslie, Hussain Sajjad, Patrick B. Murphy. Bladder Stones. In: StatPearls Internet]. Treasure Island FL StatPearls Publishing; 2020 Jan. 2020 Jun 12. Open 3. F T Hammad, M Kaya, E Kazim. Bladder calculi: did the clinical picture change?. Urology. 2006 Jun;67 6 1154 8. Open https://web.pathway.md/diseases/recbwiiE3D61W3LzU 3/3
Guideline sources The following summarized guidelines for the evaluation and management of bladder trauma are prepared by our editorial team based on guidelines from the European Association of Urology (EAU 2022), the Eastern Association for the Surgery of Trauma (EAST 2019), the World Society of Emergency Surgery (WSES/AAST 2019), the American College of Radiology (ACR 2019), and the American Urological Association (AUA 2014). 1 2 3 4 5 Guidelines 1. Diagnostic investigations Computed tomography: As per ACR 2019 guidelines, obtain pelvic CT with bladder contrast (CT cystography) for initial evaluation of suspected penetrating trauma to the lower urinary tract. B As per EAST 2019 guidelines: Obtain CT cystography for the diagnosis of bladder rupture in moderate-risk (gross hematuria) and high-risk (gross hematuria and pelvic fracture) patients. B Avoid obtaining routine retrograde CT cystography or radiography for the diagnosis of bladder rupture in low-risk patients (microscopic hematuria only). D https://web.pathway.md/diseases/rec7YPY5Fwjw3naVV 1/4 6/29/23, 3:18 AM Bladder trauma Pathway As per WSES 2019 guidelines, recognize that IV contrast-enhanced CT with delayed phase is less sensitive and specific than retrograde cystography in detecting bladder injuries. B Retrograde cystography: As per EAU 2022 guidelines, obtain cystography with active retrograde filling of the bladder with dilute contrast (300-350 mL): in the presence of visible hematuria and pelvic fracture in case of suspected iatrogenic bladder injury in the postoperative setting to rule out bladder injury during retropubic suburethral sling procedures. A As per ACR 2019 guidelines, obtain fluoroscopy retrograde cystography for initial evaluation of suspected penetrating trauma to the lower urinary tract. B As per WSES 2019 guidelines, obtain retrograde cystography (conventional radiography or CT) as the diagnostic procedure of choice in patients with bladder injuries. B Show 2 more As per AUA 2014 guidelines: Obtain retrograde cystography (plain film or CT) in stable patients with gross hematuria and pelvic fracture. B Obtain retrograde cystography in stable patients with gross hematuria and a mechanism concerning for bladder injury and in patients with pelvic ring fractures and clinical indicators of bladder rupture. B 2. Medical management Non-operative management: As per EAU 2022 guidelines: Offer nonoperative management in patients with uncomplicated blunt extraperitoneal bladder injuries. B Offer nonoperative management in patients with small uncomplicated intraperitoneal bladder injuries during endoscopic procedures. B As per EAST 2019 guidelines, consider offering nonoperative management to decrease complications from bladder injury in patients sustaining blunt abdominopelvic trauma with simple extraperitoneal bladder ruptures. C As per WSES 2019 guidelines: Consider observing patients with bladder contusion clinically with no specific treatment. B Consider managing patients with uncomplicated blunt or penetrating extraperitoneal bladder injuries nonoperatively with urinary drainage via a urethral or suprapubic catheter, in the absence of other indications for laparotomy. B 3. Surgical interventions https://web.pathway.md/diseases/rec7YPY5Fwjw3naVV 2/4 6/29/23, 3:18 AM Bladder trauma Pathway Indications for surgery: As per EAU 2022 guidelines: Perform surgery in patients with blunt extraperitoneal bladder injuries in cases of bladder neck involvement and/or associated injuries requiring surgical intervention. A Perform surgical exploration and repair in patients with blunt intraperitoneal injuries. A As per EAST 2019 guidelines: Perform surgery to decrease complications from bladder injury in patients sustaining blunt abdominopelvic trauma with intraperitoneal bladder rupture. B Consider performing an operative repair to decrease complications from the bladder injury in patients with complex extraperitoneal injuries. C As per WSES 2019 guidelines, perform direct inspection of the intraperitoneal bladder, whenever feasible, during emergency laparotomy in patients with suspected bladder injury. Consider using methylene blue or indigo carmine during intraoperative investigation. B Show 7 more As per AUA 2014 guidelines, perform surgical repair of intraperitoneal bladder rupture in patients with blunt or penetrating external trauma. B Show 3 more 4. Follow-up and surveillance Serial imaging assessment: As per EAU 2022 guidelines, obtain cystography to assess bladder wall healing after repair of a complex injury or in case of risk factors for wound healing. A As per EAST 2019 guidelines, avoid obtaining routine follow-up cystography in low-risk patients (operative repair of simple intraperitoneal or extraperitoneal bladder ruptures) in the absence of clinical signs or symptoms concerning for urinary leakage. D Show 2 more As per WSES 2019 guidelines, obtain CT with delayed phase as the choice of follow-up imaging in patients with bladder injuries. B Clinical findings Symptoms Past medical history Difficulty urinating Abdominal trauma Dysuria Kidney trauma Hematuria Pelvic fracture Suprapubic pain Pelvic trauma Sepsis Past events Traumatic injury https://web.pathway.md/diseases/rec7YPY5Fwjw3naVV 3/4 6/29/23, 3:18 AM Bladder trauma Pathway Motor vehicle accident Ureteral injury Genitourinary exam Abdominal exam urine output Abdominal distension Ascites Peritoneal findings Suprapubic tenderness Lab findings serum creatinine References 1. Lawrence L Yeung, Amy A McDonald, John J Como et al. Management of blunt force bladder injuries: A practice management guideline from the Eastern Association for the Surgery of Trauma. J Trauma Acute Care Surg. 2019 Feb;86 2 326 336. Open 2. N.D. Kitrey Chair), F. Campos-Juanatey, P. Hallscheidt et al. EAU Guidelines on Urological Trauma. EAU. 2022. Open 3. Morey AF, Brandes S, Dugi DD rd et al. Urotrauma: AUA guideline. J Urol. 2014 Aug;192 2 327 35. Open 4. Federico Coccolini, Ernest E Moore, Yoram Kluger et al. Kidney and uro-trauma: WSES AAST guidelines. World J Emerg Surg. 2019 Dec 2;14 54. Open 5. Expert Panel on Urological Imaging, Matthew T Heller, Aytekin Oto et al. ACR Appropriateness Criteria Penetrating Trauma-Lower Abdomen and Pelvis. J Am Coll Radiol. 2019 Nov;16 11S S392 S398. Open https://web.pathway.md/diseases/rec7YPY5Fwjw3naVV 4/4
Guideline sources The following summarized guidelines for the management of bleeding in patients on antithrombotics are prepared by our editorial team based on guidelines from the Canadian Cardiovascular Society (CCS 2018) and the British Society for Haematology (BSH 2013). 1 2 3 3 4 4 Definition Bleeding in patients on antithrombotics is a common complication in patients treated with both anticoagulation and antiplatelet therapy. 3 Epidemiology Bleeding in patients on antithrombotics is caused by a complex biological interaction between blood and tissue factors and extensive anticoagulation effect. 4 Disease course The complex biological interaction of blood and tissue factors, in addition to the extensive anticoagulation effect, activates bleeding cascade causing bleeding in patients on antithrombotics, which may be categorized as minor or major bleeding that may increase morbidity and mortality. 4 Prognosis and risk of recurrence Major bleeding is associated with a mortality rate of 11%. 3 https://web.pathway.md/diseases/recbAhTGe1aO1TxVZ 1/3 6/29/23, 3:18 AM Bleeding in patients on antithrombotics Pathway Guidelines 1. Medical management Patients on unfractionated heparin: discontinue heparin infusions and apply general hemostatic measures to stop bleeding in patients on UFH. B Patients on warfarin: administer 4-factor PCC (25-50 units/kg) and vitamin K (5 mg IV, for one dose) in patients with major bleeding while on warfarin. B Show 2 more Patients on danaparoid: discontinue treatment and apply general hemostatic measures to control bleeding, as there is no specific antidote for danaparoid. B Patients on fondaparinux: discontinue treatment and apply general hemostatic measures to control bleeding in patients on fondaparinux, as there is no specific antidote. B Patients on bivalirudin: discontinue treatment and apply general hemostatic measures to control bleeding, as there is no specific antidote for bivalirudin. B Patients on argatroban: discontinue argatroban and implement general hemostatic measures to control bleeding as there is no specific antidote for argatroban. B Patients on dabigatran: As per CCS 2018 guidelines, consider administering idarucizumab for emergency reversal of dabigatran's anticoagulant effect in patients with uncontrollable or potentially life-threatening bleeding and/or in patients who require urgent surgery for which normal hemostasis is necessary. B As per BSH 2013 guidelines: Discontinue treatment and start general hemostatic measures to control bleeding. B Consider administration of PCC, activated PCC and/or recombinant factor VIII in patients with ongoing life-threatening bleeding. C Patients on rivaroxaban: Discontinue treatment and start general hemostatic measures to control bleeding. B Consider administration of PCC, activated PCC and/or recombinant factor VIII in patients with ongoing life-threatening bleeding. C Patients on anti-platelets: decide to withhold antiplatelet drugs or administer pro-hemostatic agents after a careful multidisciplinary evaluation of the associated risks and benefits. B Patients on fibrinolytics: discontinue infusion of fibrinolytic and other antithrombotic drugs in patients with major bleeding within 48 hours of administration of fibrinolytic drugs. B Show 3 more 2. Specific circumstances https://web.pathway.md/diseases/recbAhTGe1aO1TxVZ 2/3 6/29/23, 3:18 AM Bleeding in patients on antithrombotics Pathway Supratherapeutic INR without bleeding: withhold 1-2 doses of warfarin and reduce the maintenance dose in patients with an INR > 5.0 but who are not bleeding. B Show 2 more Warfarin reversal prior to surgery: correct INR by giving intravenous vitamin K for surgery that requires reversal of warfarin and that can be delayed 6-12 hours. B Show 2 more Clinical findings Symptoms Past medical history Hematemesis CVD Hematuria Cancer Melena Diabetes mellitus Liver cirrhosis Social history Ulcer Current cigarette smoking Vital signs Hematological findings Hypotension systolic BP Anemia secondary to blood loss Coagulopathy ECG findings Sinus tachycardia References 1. Makris M, Van Veen JJ, Tait CR et al. Guideline on the management of bleeding in patients on antithrombotic agents. Br J Haematol. 2013 Jan;160 1 35 46. Open 2. Jason G Andrade, Atul Verma, L Brent Mitchell et al. 2018 Focused Update of the Canadian Cardiovascular Society Guidelines for the Management of Atrial Fibrillation. Can J Cardiol. 2018 Nov;34 11 1371 1392. Open 3. M E Gimbel, S C S Minderhoud, J M Ten Berg. A practical guide on how to handle patients with bleeding events while on oral antithrombotic treatment. 2018 Jun;26 6 341 351.2018 Jun;26 6 341 351. Open 4. Petras Stirbys. Review And Insights Into The Bleeding Mechanism Incited By Antithrombotic Therapy: Mechanistic Nuances Of Dual Pro-Hemorrhagic Substrate Incorporating Drug-Induced Microvascular Leakage. 2015 Aug 31;8 2 1254.2015 Aug 31;8 2 1254. Open https://web.pathway.md/diseases/recbAhTGe1aO1TxVZ 3/3
Guideline sources The following summarized guidelines for the evaluation and management of blepharitis are prepared by our editorial team based on guidelines from the College of Optometrists (CO 2023), the American Academy of Ophthalmology (AAO 2022; 2019), and the British Association of Dermatologists (BAD 2021). 1 2 3 4 Guidelines 1. Screening and diagnosis Differential diagnosis: As per AAO 2022 guidelines: Suspect an eyelid tumor in patients with atypical eyelid margin inflammation or disease not responding to medical treatment and obtain a careful re-evaluation in these patients. https://web.pathway.md/diseases/rec6OLM39Q041Xaqa 1/6 6/29/23, 3:18 AM Blepharitis Pathway Refer patients with an inflammatory eyelid lesion appearing suspicious for malignancy to an appropriate specialist. As per AAO 2019 guidelines: Consider distinguishing blepharitis and meibomian gland dysfunction from aqueous deficient dry eye in patients with ocular surface disease. Recognize that worsening of symptoms in the morning is typical of blepharitis, whereas worsening of the symptoms later in the day is typical of aqueous deficient dry eye. E Suspect carcinoma or immune-mediated diseases in patients with blepharitis not responding to therapy, particularly if the blepharitis is associated with a loss of eyelashes and/or conjunctival cicatricial changes. Ensure early diagnosis and appropriate treatment to prevent disfigurement. E 2. Diagnostic investigations History and physical examination: Elicit initial history, including the following: Situation Guidance Redness, irritation, burning, tearing, itching, crusting of eyelashes, loss of eyelashes, eyelid sticking, blurring or fluctuating vision, contact lens intolerance, photophobia, increased frequency of blinking, and recurrent hordeolum Ocular symptoms and signs Time of day when symptoms are worse, duration of symptoms Chronology of symptoms Unilateral versus bilateral presentation Laterality of symptoms Smoke, allergens, wind, contact lenses, low humidity, retinoids, diet, alcohol consumption, and eye makeup Exacerbating conditions Rosacea, atopy, psoriasis, and GvHD Symptoms related to systemic diseases Antihistamines or drugs with anticholinergic effects, or drugs used in the past that might have an effect on the ocular surface, such as isotretinoin Current and previous systemic and topical medications Patient with pediculosis palpebrarum Recent exposure to an infected individual Previous intraocular and eyelid surgery, local trauma, including mechanical, thermal, chemical, and radiation injury, history of Ocular history https://web.pathway.md/diseases/rec6OLM39Q041Xaqa 2/6 6/29/23, 3:18 AM Blepharitis Pathway cosmetic blepharoplasty, history of styes and/or chalazia. Perform initial physical examination, including assessment of visual acuity, external examination of the skin and eyelids, and slit-lamp biomicroscopy (including tear film, anterior eyelid margin, eyelashes, posterior eyelid margin, tarsal conjunctiva, bulbar conjunctiva, and cornea). Conjunctival culture: consider obtaining cultures in patients with recurrent anterior blepharitis with severe inflammation as well as in patients not responding to treatment. 3. Diagnostic procedures Eyelid biopsy: consider performing an eyelid biopsy to exclude the possibility of carcinoma in patients with marked asymmetry, resistance to treatment, or unifocal recurrent chalazia not responding well to treatment. Consult a pathologist before performing the biopsy if sebaceous cell carcinoma is suspected. 4. Medical management Topical antibiotics: As per CO 2023 guidelines, consider offering topical antibiotics in patients with staphylococcal or seborrhoeic blepharitis not controlled by first-line management: antibiotic ointment (such as chloramphenicol) BID; place in eyes or rub into lid margin with fingertip short course of topical azithromycin (off-label use). C As per AAO 2022 guidelines, consider offering topical antibiotics, such as bacitracin or erythromycin, applied 1 times daily or at bedtime on the eyelid margins for a few weeks. As per AAO 2019 guidelines, consider offering topical antibiotic ointments, with or without corticosteroids or oral antibiotics, in patients with blepharitis. E Topical corticosteroids: Consider offering a short course of topical corticosteroids at the minimal effective dose for eyelid or ocular surface inflammation, such as severe conjunctival infection, marginal keratitis, or phlyctenules. Avoid using corticosteroid therapy for long term if possible. Re-evaluate patients receiving corticosteroids within a few weeks of initiation to determine the response to treatment, measure intraocular pressure, and assess treatment compliance. Oral antibiotics: As per CO 2023 guidelines: Consider offering systemic antibiotics (such as doxycycline or minocycline for several weeks or months; or oral erythromycin or azithromycin if tetracyclines are contraindicated) as second- line therapy in patients with posterior blepharitis. C https://web.pathway.md/diseases/rec6OLM39Q041Xaqa 3/6 6/29/23, 3:18 AM Blepharitis Pathway Insufficient evidence to support the use of oral antibiotics in patients with chronic blepharitis. I As per AAO 2022 guidelines, consider offering oral tetracyclines with topical antibiotics in patients with meibomian gland dysfunction with chronic symptoms and signs not adequately controlled with eyelid cleansing or meibomian gland expression. 5. Nonpharmacologic interventions Eyelid cleansing: As per CO 2023 guidelines, advise eyelid cleansing measures to wipe away bacteria and deposits from eyelid margins and to improve signs and symptoms in patients with blepharitis. B As per AAO 2022 guidelines, advise eyelid cleansing and eyelid hygiene for the initial management of patients with blepharitis. Warm compresses: As per CO 2023 guidelines, advise wet warm compresses to loosen collarettes and crusts in patients with anterior blepharitis. Apply dry, warm compresses to the lid skin BID for 5 minutes at 40 C to melt meibum in patients with posterior blepharitis. B As per AAO 2022 guidelines, advise warm compresses for the initial management of patients with blepharitis. 6. Therapeutic procedures Intense pulsed light therapy: insufficient evidence to recommend intense pulsed light therapy for the management of meibomian gland dysfunction. I 7. Specific circumstances Patients with Demodex blepharitis: Suspect Demodex blepharitis in patients with characteristic cylindrical dandruff at the roots of eyelashes or refractory blepharitis. B Insufficient evidence to support the use of tea tree oil in patients with Demodex blepharitis. Use lower concentrations to avoid ocular toxicity when using tea tree oil. I Patients with rosacea (pharmacotherapy): offer topical azelaic acid, topical ivermectin, topical brimonidine, oral doxycycline, or oral isotretinoin for blepharitis in patients with rosacea. B Patients with rosacea (nonpharmacologic management): advise minimizing exposure to aggravating factors (such as air conditioning, excessive central heating, smoky atmospheres, and periocular cosmetics) in patients with ocular rosacea. A Show 4 more https://web.pathway.md/diseases/rec6OLM39Q041Xaqa 4/6 6/29/23, 3:18 AM Blepharitis Pathway Patients with rosacea (indications for referral): Refer patients with ocular rosacea to an ophthalmologist in case of: eye discomfort, sticky eye discharge persisting > 12 months despite frequent (> 6 times daily) topical lubricant use and adequate lid hygiene symptoms keeping the patient awake at night (such as reduced vision, pain on eye movement, and pain). A 8. Patient education General counseling: As per AAO 2022 guidelines, counsel patients about the chronicity and recurrence of the disease process and that symptoms can frequently be improved but are rarely eliminated. As per AAO 2019 guidelines, educate patients that blepharitis is typically a chronic condition that cannot be permanently cured, and successful management is dependent on patient compliance with a treatment regimen. E 9. Follow-up and surveillance Follow-up: arrange follow-up visits to elicit interval history, measure visual acuity, perform external physical examinations, and obtain slit-lamp biomicroscopy. Clinical findings Symptoms Past medical history Dry eyes Chalazion Eye discharge Dry eye disease Eye pain Giant papillary conjunctivitis Foreign body sensation in the eyes IBS Pterygium Gritty eyes Rosacea Itchy eyes Ulcerative colitis Photophobia Ocular exam Puffy eyelids Red eyes Collarettes Watery eyes Conjunctival injection Cylindrical dandruff at the roots of eyelashes Social history Tobacco use Eyelid swelling Use of contact lenses Madarosis Trichiasis https://web.pathway.md/diseases/rec6OLM39Q041Xaqa 5/6 6/29/23, 3:18 AM Blepharitis Pathway References 1. AAO PPP Cornea/External Disease Panel, Hoskins Center for Quality Eye Care. Cornea/External Disease Summary Benchmarks 2022. AAO. 2022 Dec. Open 2. College of Optometrists. Blepharitis Lid Margin Disease). COP. 2022 Jul;v.17. Open 3. Guillermo Amescua, Esen K Akpek, Marjan Farid et al. Blepharitis Preferred Practice Pattern . Ophthalmology. 2019 Jan;126 1 P56 P93. Open 4. P J Hampton, J Berth-Jones, C E Duarte Williamson et al. British Association of Dermatologists guidelines for the management of people with rosacea 2021. Br J Dermatol. 2021 May 15. Open 5. Mary Eberhardt, Guhan Rammohan. Blepharitis. In: StatPearls Internet]. Treasure Island FL StatPearls Publishing; 2021 Jan. 2020 Jul 17. Open 6. Taliana Freitas Bernardes, Adriana Alvim Bonfioli. Blepharitis. Semin Ophthalmol. 2010 May;25 3 79 83. Open 7. Guillermo Amescua, Esen K Akpek, Marjan Farid et al. Blepharitis Preferred Practice Pattern . Ophthalmology. 2019 Jan;126 1 P56 P93. Open 8. College of Optometrists. Keratitis (marginal). COP. 2022 Jan;v.12. Open https://web.pathway.md/diseases/rec6OLM39Q041Xaqa 6/6
Guideline sources The following summarized guidelines for the evaluation of blood gas analysis are prepared by our editorial team based on guidelines from the American Association for Respiratory Care (AARC 2013). 1 2 2 2 3 Definition Blood gas analysis is a common diagnostic tool to correctly interpret most of the respiratory, circulatory, and metabolic derangements through arterial and pulmonary/central venous blood gas and electrolyte analysis. 2 Epidemiology Blood gas analysis is used for the assessment of shunt fraction, pulmonary dead space, and respiratory, hemodynamic and metabolic relationship allowing for diagnosis and monitoring of critically ill patients. 2 Disease course Blood gas analysis is performed either through the arterial, central venous, or dual sampling of blood to assess oxygenation status, ventilatory and respiratory status, metabolic acid-base equilibrium disorders helping in the diagnosis and monitoring of patients in emergency departments and ICUs. 2 Prognosis and risk of recurrence Blood gas analysis is not associated with increased mortality. 3 https://web.pathway.md/diseases/rec8GEnDD8w5MzQF3 1/2 6/29/23, 3:18 AM Blood gas analysis Pathway Guidelines 1. Diagnostic investigations General principles: obtain blood gas analysis and hemoximetry to evaluate a patient's ventilatory, acid-base, and/or oxygenation status. A Show 3 more Assessment of oxygenation: avoid capillary blood gas analysis to determine oxygenation status. D Show 2 more Assessment of ventilation: avoid using venous arterial partial pressure of CO and pH as a substitute for arterial blood measurement of arterial partial pressure of CO and pH. D Clinical findings Symptoms Past medical history Confusion CKD Difficulty breathing Metabolic disorders Nausea Respiratory exam Past events Dyspnea Head injury Lab findings Blood gas analysis Ph of the blood blood CO2 blood oxygen References 1. Davis MD, Walsh BK, Sittig SE et al. AARC clinical practice guideline: blood gas analysis and hemoximetry: 2013. Respir Care. 2013 Oct;58 10 1694 703. Open 2. Luciano Gattinoni, Antonio Pesenti, Michael Matthay. Understanding blood gas analysis. 2018 Jan;44 1 91 93.2018 Jan;44 1 91 93. Open 3. Lora Dukic, Lara Milevoj Kopcinovic, Adrijana Dorotic et al. Blood gas testing and related measurements: National recommendations on behalf of the Croatian Society of Medical Biochemistry and Laboratory Medicine. 2016 Oct 15;26 3 318 336.2016 Oct 15;26 3 318 336. Open https://web.pathway.md/diseases/rec8GEnDD8w5MzQF3 2/2
Guideline sources The following summarized guidelines for the evaluation and management of blunt abdominal trauma are prepared by our editorial team based on guidelines from the American Heart Association (AHA/ACC 2022), the European Association of Urology (EAU 2022), the World Society of Emergency Surgery (WSES 2022; 2020; 2017), the World Society of Emergency Surgery (WSES/AAST 2020; 2019), the Eastern Association for the Surgery of Trauma (EAST 2019; 2012), the American Urological Association (AUA 2014), and the American College of Emergency Physicians (ACEP 2011). 1 2 3 4 5 6 7 8 9 10 11 12 13 Guidelines 1. Diagnostic investigations Initial evaluation: https://web.pathway.md/diseases/recNf2GjVlFbQ9tDb 1/8 6/29/23, 3:19 AM Blunt abdominal trauma Pathway As per WSES 2022 guidelines, obtain a primary survey, extended FAST, perform a physical examination, obtain a secondary survey, blood chemistry, check for vital signs followed by contrast-enhanced abdominal CT in awake and oriented patients with blunt abdominal trauma. A Show 4 more As per WSES 2017 guidelines, decide on diagnostic technique at admission based on the hemodynamic status of the patient. A Show 2 more As per ACEP 2011 guidelines, obtain bedside U/S, when available, as the initial diagnostic modality to identify the need for emergent laparotomy in hemodynamically unstable patients (systolic BP 90 mmHg) with blunt abdominal trauma. B Computed tomography: As per WSES 2022 guidelines, obtain CT in the presence of a seatbelt sign (a high index of suspicion for bowel injury). A Show 3 more As per WSES 2017 guidelines, obtain CT with IV contrast as the gold standard in hemodynamically stable or stabilized patients with trauma. A Show 2 more As per EAST 2012 guidelines, obtain abdominal CT with IV contrast in hemodynamically stable patients with blunt abdominal trauma without peritonitis, to identify and assess the severity of injury to the spleen. B As per ACEP 2011 guidelines, avoid obtaining CT in patients with isolated abdominal trauma suspected for occult abdominal injury, if the following are absent: abdominal tenderness hypotension altered mental status (GCS < 14) costal margin tenderness abnormal CXR hematocrit < 30% hematuria. D Angiography: Consider obtaining angiography in patients with AAST grade > III injuries, presence of a contrast blush, moderate hemoperitoneum, or evidence of ongoing splenic bleeding. C Consider obtaining angiography either as an adjunct to nonoperative management in patients with splenic injury thought to be at high risk for delayed bleeding or as an investigative tool to identify vascular abnormalities such as pseudoaneurysms posing a risk for delayed hemorrhage. C 2. Diagnostic procedures https://web.pathway.md/diseases/recNf2GjVlFbQ9tDb 2/8 6/29/23, 3:19 AM Blunt abdominal trauma Pathway Diagnostic laparoscopy: Consider performing diagnostic laparoscopy in hemodynamically compensated patients with highly sensitive findings of bowel injury on CT. C Consider treating bowel injuries laparoscopically, based on the surgeon experience and logistics of the trauma center, if identified during diagnostic laparoscopy. C Diagnostic peritoneal lavage: consider performing diagnostic peritoneal lavage as an adjunct to a negative laparoscopy to definitively exclude bowel injury, particularly in conjunction with the use of biomarkers. C 3. Medical management Non-operative management: As per ACC 2022 guidelines, initiate anti-impulse therapy, if clinically tolerated, and obtain repeat imaging within 24-48 hours of the initial imaging to reduce the risk of injury progression in patients with grade 1-2 blunt traumatic abdominal aortic injury without malperfusion. B As per EAST 2012 guidelines: Consider offering nonoperative management in patients with splenic injuries only in an environment providing capabilities for monitoring, serial clinical evaluations and an operating room available for urgent laparotomy. C Recognize that the following factors are not contraindications for attempting a trial of nonoperative management in hemodynamically stable patients with splenic injuries: severity of injury (as suggested by CT grade or degree of hemoperitoneum) neurologic status age > 55 presence of associated injuries. B Thromboprophylaxis: consider administering pharmacologic prophylaxis to prevent VTE in patients with isolated blunt splenic injuries without increasing the failure rate of nonoperative management, although insufficient evidence on the optimal timing of safe initiation. C 4. Inpatient care Serial clinical and laboratory assessment: As per WSES 2022 guidelines, admit patients with high-risk mechanisms (handlebar, seatbelt sign) and non-specific CT findings for observation including serial clinical examination. B As per WSES 2017 guidelines, obtain clinical and laboratory observation associated to bed rest in the first 48-72 hours of follow-up in patients with moderate and severe splenic injury. B Serial imaging assessment: As per WSES 2017 guidelines, consider obtaining repeated CT during the admission in patients with splenic injury with: https://web.pathway.md/diseases/recNf2GjVlFbQ9tDb 3/8 6/29/23, 3:19 AM Blunt abdominal trauma Pathway moderate and severe lesions or in decreasing hematocrit in the presence of vascular anomalies or underlying splenic pathology or coagulopathy neurological impairment. B As per EAST 2012 guidelines, use the following to decide on frequency of and need for follow- up imaging in patients with blunt splenic injury: clinical factors, such as persistent systemic inflammatory response increasing/persistent abdominal pain otherwise unexplained drop in Hgb. B As per EAST 2012 guidelines, use the following to decide on frequency of and need for follow- up imaging in patients with blunt splenic injury: clinical factors, such as persistent systemic inflammatory response increasing/persistent abdominal pain otherwise unexplained drop in Hgb. B 5. Therapeutic procedures Resuscitative endovascular balloon occlusion of the aorta: As per ACC 2022 guidelines: Insufficient evidence regarding the usefulness of the routine use of REBOA for hemorrhage control in patients with blunt traumatic abdominal aortic injury, and even it may cause harm in some cases. I Insufficient evidence regarding the usefulness of the routine use of REBOA for hemorrhage control in patients with blunt traumatic abdominal aortic injury, and even it may cause harm in some cases. I 6. Surgical interventions Indications for laparotomy: As per WSES 2022 guidelines, perform prompt surgical exploration in the presence of highly specific CT findings such as extraluminal air, extraluminal oral contrast, or bowel-wall defects. A As per EAST 2012 guidelines: Perform urgent laparotomy in patients with diffuse peritonitis and in hemodynamically unstable patients after blunt abdominal trauma. A Do not perform routine laparotomy in hemodynamically stable patients without peritonitis presenting with an isolated splenic injury. D Vascular injury repair: as per ACC 2022 guidelines, initiate anti-impulse therapy, if clinically tolerated, and obtain repeat imaging within 24-48 hours of the initial imaging to reduce the risk of https://web.pathway.md/diseases/recNf2GjVlFbQ9tDb 4/8 6/29/23, 3:19 AM Blunt abdominal trauma Pathway injury progression in patients with grade 1-2 blunt traumatic abdominal aortic injury without malperfusion. B Show 11 more 7. Specific circumstances Patients with liver trauma: As per WSES 2020 guidelines: Obtain extended FAST for rapid detection of intra-abdominal free fluid. A Obtain CT with IV contrast as the gold standard in hemodynamically stable patients with trauma A , and in patients considered for nonoperative management of liver trauma. B As per EAST 2012 guidelines: Obtain abdominal CT with IV contrast to identify and assess the severity of liver injury in hemodynamically stable patients with blunt abdominal trauma without peritonitis. B Obtain reevaluation with CT in patients with persistent systemic inflammatory response, increasing persistent abdominal pain, jaundice, or an otherwise unexplained drop in Hgb. B Patients with pancreatoduodenal injury: obtain extended FAST for detecting free fluid and solid organ injury. A Show 15 more Patients with kidney injury: As per EAU 2022 guidelines, obtain multiphase CT in patients with traumatic injury and: visible hematuria non-visible hematuria and one episode of hypotension history of rapid deceleration injury and/or significant associated injuries clinical signs suggesting kidney trauma, such as flank pain, abrasions, fractured ribs, abdominal distension, and/or a mass and tenderness. A As per WSES 2019 guidelines, obtain extended FAST to detect intra-abdominal free fluid. A Recognize that extended FAST has low sensitivity and specificity in kidney trauma. B Show 2 more As per AUA 2014 guidelines, obtain IV contrast-enhanced abdominal/pelvic CT with immediate and delayed images in patients with suspected renal injury. B Show 7 more Patients with ureteral injury: As per EAU 2022 guidelines, suspect ureteral injury in patients with deceleration-type blunt trauma. A As per WSES 2019 guidelines: Suspect ureteral injury in patients with high-energy blunt trauma, particularly in deceleration injuries with multi-system involvement. B https://web.pathway.md/diseases/recNf2GjVlFbQ9tDb 5/8 6/29/23, 3:19 AM Blunt abdominal trauma Pathway Obtain IV contrast-enhanced CT with delayed phase in hemodynamically stable or stabilized patients with suspected ureteral injury. B As per AUA 2014 guidelines, obtain IV contrast-enhanced abdominal/pelvic CT with delayed imaging (urogram) in stable patients with trauma with suspected ureteral injuries. B Patients with bladder injury: As per EAST 2019 guidelines, obtain CT cystography for the diagnosis of bladder rupture in moderate-risk (gross hematuria) and high-risk (gross hematuria and pelvic fracture) patients. B Show 4 more As per WSES 2019 guidelines, recognize that IV contrast-enhanced CT with delayed phase is less sensitive and specific than retrograde cystography in detecting bladder injuries. B Show 9 more As per AUA 2014 guidelines, obtain retrograde cystography (plain film or CT) in stable patients with gross hematuria and pelvic fracture. B Show 5 more 8. Follow-up and surveillance Discharge from hospital: discharge clinically stable patients with isolated blunt abdominal trauma after a negative result for abdominal CT with IV contrast, with or without oral contrast. B Follow-up: As per ACC 2022 guidelines: Consider obtaining surveillance imaging at intervals appropriate for the repair approach and location in patients with blunt traumatic aortic injury undergone aortic repair. C Consider obtaining surveillance CT at 1 month, 6 months, and 12 months after the diagnosis and, if stable, at appropriate intervals thereafter (depending on the type and extent of the injury) in patients with blunt traumatic aortic injury not undergone repair. C As per WSES 2022 guidelines, obtain long-term follow-up in patients with blunt abdominal trauma to identify the sequelae of mesenteric injuries. B As per ACEP 2011 guidelines, consider obtaining further observation, close follow-up, and/or imaging in selected patients based on clinical judgment. C Clinical findings Symptoms Vital signs Abdominal pain Hypotension Signs of shock Neurological exam Tachycardia Altered mental status Abdominal exam https://web.pathway.md/diseases/recNf2GjVlFbQ9tDb 6/8 6/29/23, 3:19 AM Blunt abdominal trauma Pathway Abdominal distension Musculoskeletal exam Abdominal guarding Concomitant femur fracture Abdominal tenderness Costal margin tenderness Lab findings Peritoneal findings Microscopic hematuria Rebound tenderness serum lactate Seat belt sign serum base deficit Integument exam Imaging findings Seatbelt sign Free intraperitoneal fluid Hematological findings Pneumoperitoneum WBC count hematocrit Likelihood Ratios Pertinent positives The following findings increase the probability of blunt abdominal trauma in adults. 13 Finding LR+ Value Presence of rebound tenderness 6.5 (1.8-24) Presence of seat belt sign 5.6-9.9 Presence of hypotension 5.2 (3.5-7.5) Presence of abdominal distension 3.8 (1.9-7.6) Show 5 more Pertinent negatives The following findings decrease the probability of blunt abdominal trauma in adults. 13 Finding LR- Value No history of abdominal pain 0.52 (0.34-0.79) Absence of seat belt sign 0.53-0.55 Absence of abdominal tenderness 0.61 (0.46-0.80) Absence of costal margin tenderness 0.74 (0.66-0.84) Show 5 more References 1. Deborah B Diercks, Abhishek Mehrotra, Devorah J Nazarian et al. Clinical policy: critical issues in the evaluation of adult patients presenting to the emergency department with acute blunt abdominal trauma. Ann Emerg Med. 2011 Apr;57 4 387 404. Open https://web.pathway.md/diseases/recNf2GjVlFbQ9tDb 7/8 6/29/23, 3:19 AM Blunt abdominal trauma Pathway 2. Leslie Kobayashi, Raul Coimbra, Adenauer M O Goes Jr et al. American Association for the Surgery of Trauma-World Society of Emergency Surgery guidelines on diagnosis and management of abdominal vascular injuries. J Trauma Acute Care Surg. 2020 Dec;89 6 1197 1211. Open 3. Eric M Isselbacher, Ourania Preventza, James Hamilton Black rd et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. Circulation. 2022 Nov 2. Open 4. Federico Coccolini, Ernest E Moore, Yoram Kluger et al. Kidney and uro-trauma: WSES AAST guidelines. World J Emerg Surg. 2019 Dec 2;14 54. Open 5. N.D. Kitrey Chair), F. Campos-Juanatey, P. Hallscheidt et al. EAU Guidelines on Urological Trauma. EAU. 2022. Open 6. Nicole A Stassen, Indermeet Bhullar, Julius D Cheng et al. Selective nonoperative management of blunt splenic injury: an Eastern Association for the Surgery of Trauma practice management guideline. J Trauma Acute Care Surg. 2012 Nov;73 5 Suppl 4 S294 300. Open 7. Luke Smyth, Cino Bendinelli, Nicholas Lee et al. WSES guidelines on blunt and penetrating bowel injury: diagnosis, investigations, and treatment. 10.1186/s13017 022 00418-y. Open 8. Nicole A Stassen, Indermeet Bhullar, Julius D Cheng et al. Nonoperative management of blunt hepatic injury: an Eastern Association for the Surgery of Trauma practice management guideline. J Trauma Acute Care Surg. 2012 Nov;73 5 Suppl 4 S288 93. Open 9. Federico Coccolini, Giulia Montori, Fausto Catena et al. Splenic trauma: WSES classification and guidelines for adult and pediatric patients. World J Emerg Surg. 2017 Aug 18;12 40. Open 10. Federico Coccolini, Raul Coimbra, Carlos Ordonez et al. Liver trauma: WSES 2020 guidelines. World J Emerg Surg. 2020 Mar 30;15 1 24. Open 11. Federico Coccolini, Leslie Kobayashi, Yoram Kluger et al. Duodeno-pancreatic and extrahepatic biliary tree trauma: WSES AAST guidelines. World J Emerg Surg. 2019 Dec 11;14 56. Open 12. Lawrence L Yeung, Amy A McDonald, John J Como et al. Management of blunt force bladder injuries: A practice management guideline from the Eastern Association for the Surgery of Trauma. J Trauma Acute Care Surg. 2019 Feb;86 2 326 336. Open 13. Morey AF, Brandes S, Dugi DD rd et al. Urotrauma: AUA guideline. J Urol. 2014 Aug;192 2 327 35. Open 14. Nishijima DK, Simel DL, Wisner DH et al. Does this adult patient have a blunt intra-abdominal injury?. JAMA. 2012 Apr 11;307 14 1517 27. Open 15. Expert Panel on Major Trauma Imaging, Jeffrey Y Shyu, Bharti Khurana et al. ACR Appropriateness Criteria Major Blunt Trauma. J Am Coll Radiol. 2020 May;17 5S S160 S174. Open https://web.pathway.md/diseases/recNf2GjVlFbQ9tDb 8/8
Guideline sources The following summarized guidelines for the evaluation and management of blunt cardiac injury (BCI) are prepared by our editorial team based on guidelines from the American College of Radiology (ACR 2020) and the Eastern Association for the Surgery of Trauma (EAST 2012). 1 3 4 4 4 5 Definition BCI refers to an injury sustained due to blunt trauma to the heart, the spectrum of which ranges from a minor bruise to specific post-contusion cardiac conditions such as free-wall rupture. 4 Epidemiology BCI is mostly caused by motor vehicle accidents, falls from heights, direct blow to the chest wall by airbag deployment, crushing incidents, contact sports injuries, acts of violence, or blast trauma. 4 Disease course Clinical manifestations include arrhythmias, myocardial contusion, wall motion abnormalities, myocardial wall rupture, and valve damage. The spectrum of injuries includes commotio cordis, cardiac rupture, coronary artery dissection, indirect cardiac injury, cardiac herniation, pericardial effusion and tamponade, traumatic cardiac arrest, and blunt aortic injury. 4 Prognosis and risk of recurrence https://web.pathway.md/diseases/recdxGUsCMwhmJWIH 1/4 6/29/23, 3:20 AM Blunt cardiac injury Pathway BCI is associated with an overall mortality rate of 13.9%. 5 Calculator NEXUS criteria for chest X-ray in Guidelines 1. Screening and diagnosis Patients at risk: admit patient for continuous ECG monitoring if the admission ECG reveals a new abnormality such as arrhythmias, ST changes, ischemia or heart block. B Indications for testing: avoid implementing cardiac monitoring on the sole basis of a sternal fracture, in patients in whom ECG and troponin I levels are normal. D 2. Diagnostic investigations Electrocardiogram: obtain an admission ECG in all patients in whom BCI is suspected. A Continuous ECG monitoring: admit patients for continuous electrocardiographic monitoring if the admission ECG reveals a new abnormality such as arrhythmia, ST changes, ischemia, heart block, and unexplained ST changes. Compare with a previous ECG to determine need for monitoring in patients with preexisting abnormalities. B Troponin I: measure troponin I routinely for patients with suspected BCI; if elevated, admit patients to a monitored setting and follow troponin I serially, although the optimal timing is unknown. B Creatine kinase: avoid obtaining CK with isoenzyme analysis, because it is not beneficial in predicting which patients have or will have complications related to BCI. D Cardiac imaging: As per ACR 2020 guidelines, obtain a TTE in patients (either hemodynamically stable or unstable) with suspected cardiac injury following blunt chest trauma. B As per EAST 2012 guidelines: Obtain a TTE in patients with hemodynamic instability or persistent new arrhythmia. Obtain a TEE if an optimal TTE cannot be obtained. B Obtain cardiac CT or MRI to help differentiate acute myocardial infarction from BCI in trauma patients with abnormal ECG, cardiac enzymes, and/or abnormal echocardiography. B Chest imaging: https://web.pathway.md/diseases/recdxGUsCMwhmJWIH 2/4 6/29/23, 3:20 AM Blunt cardiac injury Pathway Obtain a CXR and chest CT with IV contrast or chest CT angiography as complementary first- line imaging modalities in hemodynamically stable patients with suspected cardiac injury following blunt chest trauma. B Obtain a CXR, chest CT with IV contrast, chest CT angiography, and cardiac CT with IV contrast as complementary imaging modalities in hemodynamically unstable patients with suspected cardiac injury following blunt chest trauma. B Nuclear medicine studies: avoid routinely obtaining nuclear medicine studies in patients with BCI, as they provide little additional information when compared with echocardiography. D 3. Specific circumstances Patients in need of surgical intervention: consider proceeding with surgery in elderly patients with known cardiac disease, unstable patients, and those with an abnormal admission ECG, provided that they are appropriately monitored. Consider placement of a pulmonary artery catheter in these situations. C Clinical findings Symptoms Past medical history Chest pain DVT Pericardial tamponade Vital signs Traumatic injury Hypotension Vascular exam Cardiac exam Pulse deficit Muffled heart sounds Respiratory exam Murmur Rales ECG findings Imaging findings AF AV block Myocardial rupture Cardiac arrhythmias Pericardial effusion Sinus tachycardia Ventricular septal defect Wall motion abnormality References 1. Clancy K, Velopulos C, Bilaniuk JW et al. Screening for blunt cardiac injury: an Eastern Association for the Surgery of Trauma practice management guideline. J Trauma Acute Care Surg. 2012 Nov;73 5 Suppl 4 S301 6. Open https://web.pathway.md/diseases/recdxGUsCMwhmJWIH 3/4 6/29/23, 3:20 AM Blunt cardiac injury Pathway 2. Mark J Seamon, Elliott R Haut, Kyle Van Arendonk et al. An evidence-based approach to patient selection for emergency department thoracotomy: A practice management guideline from the Eastern Association for the Surgery of Trauma. J Trauma Acute Care Surg. 2015 Jul;79 1 159 73. Open 3. Expert Panels on Cardiac Imaging and Thoracic Imaging, Jadranka Stojanovska, Lynne M Hurwitz Koweek et al. ACR Appropriateness Criteria Blunt Chest Trauma-Suspected Cardiac Injury. J Am Coll Radiol. 2020 Nov;17 11S S380 S390. Open 4. Rayyan Fadel, Ayman El-Menyar, Samir ElKafrawy et al. Traumatic blunt cardiac injuries: An updated narrative review. Int J Crit Illn Inj Sci. Jul-Sep 2019;9 3 113 119. Open 5. Marc Hanschen, Karl-Georg Kanz, Chlodwig Kirchhoff et al. Blunt Cardiac Injury in the Severely Injured A Retrospective Multicentre Study. PLoS One. 2015 Jul 2;10 7):e0131362. Open 6. Rayyan Fadel, Ayman El-Menyar, Samir ElKafrawy et al. Traumatic blunt cardiac injuries: An updated narrative review. Int J Crit Illn Inj Sci. Jul-Sep 2019;9 3 113 119.Jul-Sep 2019;9 3 113 119. Open 7. Marc Hanschen, Karl-Georg Kanz, Chlodwig Kirchhoff et al. Blunt Cardiac Injury in the Severely Injured A Retrospective Multicentre Study. PLoS One. 2015 Jul 2;10 7):e0131362.2015 Jul 2;10 7):e0131362. Open 8. Alexander Levitov, Heidi L Frankel, Michael Blaivas et al. Guidelines for the Appropriate Use of Bedside General and Cardiac Ultrasonography in the Evaluation of Critically Ill Patients-Part II Cardiac Ultrasonography. Crit Care Med. 2016 Jun;44 6 1206 27. Open 9. Heidi L Frankel, Andrew W Kirkpatrick, Mahmoud Elbarbary et al. Guidelines for the Appropriate Use of Bedside General and Cardiac Ultrasonography in the Evaluation of Critically Ill Patients-Part I General Ultrasonography. Crit Care Med. 2015 Nov;43 11 2479 502. Open https://web.pathway.md/diseases/recdxGUsCMwhmJWIH 4/4
Guideline sources The following summarized guidelines for the evaluation and management of blunt chest trauma are prepared by our editorial team based on guidelines from the Eastern Association for the Surgery of Trauma (EAST/AOTA 2023), the American Heart Association (AHA/ACC 2022), the European Association of Urology (EAU 2022), the Society of Interventional Radiology (SIR 2020), the American College of Radiology (ACR 2020; 2019), the European Respiratory Society (ERS 2017), the Eastern Association for the Surgery of Trauma (EAST 2017; 2016; 2015; 2012; 2011), the British Thoracic Society (BTS 2017; 2010), the Society of Critical Care Medicine (SCCM 2016), the American Urological Association (AUA 2014), the European Society of Cardiology (ESC 2014), and the Society for Vascular Surgery (SVS 2011). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 Calculator Calculator Blast Lung Injury severity score NEXUS criteria for chest X-ray in https://web.pathway.md/diseases/recgbkpqWjLA9ypLs 1/10 6/29/23, 3:20 AM Blunt chest trauma Pathway Guidelines 1. Diagnostic investigations Evaluation for pneumothorax/hemothorax: consider obtaining trauma ultrasound to identify pneumothorax and pleural effusion. C Evaluation for rib fracture: Obtain a CXR as the initial imaging in patients with suspected rib fractures in the following situations: minor blunt trauma (injury confined to ribs) after CPR pathologic fracture. B Obtain chest CT without IV contrast or whole body technetium-99m bone scan as complementary to the CXR in patients with suspected pathologic rib fracture. B Evaluation for cardiac injury (electrocardiogram): Obtain an ECG on admission in all patients with suspected blunt cardiac injury. A Admit patients with a new abnormality (arrhythmia, ST changes, ischemia, heart block, or unexplained ST changes) for continuous ECG monitoring. Compare the results to a previous ECG to determine the need for monitoring in patients with preexisting abnormalities. B Evaluation for cardiac injury (cardiac biomarkers): obtain troponin I level measurement routinely in patients with suspected blunt cardiac injury. Admit patients to a monitored setting and obtain serial laboratory monitoring if the troponin I level is elevated. B Show 2 more Evaluation for cardiac injury, imaging: As per ACR 2020 guidelines: Obtain a CXR, TTE, and chest CT with IV contrast or chest CT angiography as complementary first-line imaging modalities in hemodynamically stable patients with suspected cardiac injury following blunt chest trauma. B Obtain a CXR, TTE, chest CT with IV contrast, chest CT angiography, and cardiac CT with IV contrast as complementary imaging modalities in hemodynamically unstable patients with suspected cardiac injury following blunt chest trauma. B As per SCCM 2016 guidelines, consider obtaining a bedside TTE to exclude significant pericardial effusion in hemodynamically unstable patients with blunt chest trauma. C As per EAST 2012 guidelines: Obtain a TTE in patients with hemodynamic instability or persistent new arrhythmia. Obtain a TEE if an optimal TTE cannot be obtained. B Obtain cardiac CT or MRI to help differentiate acute myocardial infarction from blunt cardiac injury in trauma patients with abnormal ECG, cardiac enzymes, and/or abnormal https://web.pathway.md/diseases/recgbkpqWjLA9ypLs 2/10 6/29/23, 3:20 AM Blunt chest trauma Pathway echocardiography. B Evaluation for aortic injury: As per EAST 2015 guidelines, obtain contrast-enhanced chest CT in patients with suspected blunt traumatic aortic injury. A As per ESC 2014 guidelines, obtain CT in patients with suspected traumatic aortic injury. B Consider obtaining a TEE if CT is not available. B Evaluation for renal injury: As per EAU 2022 guidelines, obtain multiphase CT in patients with a rib fracture and suspected kidney trauma. A As per AUA 2014 guidelines, obtain IV contrast-enhanced CT in stable patients with trauma with a mechanism of injury or physical examination findings concerning renal injury (such as rapid deceleration, rib fracture, or penetrating injury of the lower chest). B 2. Respiratory support Noninvasive ventilation: As per ERS 2017 guidelines, consider initiating noninvasive ventilation in patients with chest trauma and acute respiratory failure. C Updated evidence: OptiTHO In adult patients admitted to the ICU within 48 hours after high-risk blunt chest trauma, non- severe hypoxemia, and no evidence of acute respiratory failure, early noninvasive ventilation and high-flow nasal oxygen therapy were not superior to late noninvasive ventilation and conventional oxygen therapy with respect to endotracheal intubation for delayed respiratory failure. C dric Carri et al. Crit Care. 2023 Apr 26. As per EAST 2012 guidelines: Consider initiating a trial of mask CPAP in combination with optimal regional anesthesia in alert, compliant patients with marginal respiratory status. C Consider initiating high-frequency oscillatory ventilation in certain cases of blunt chest trauma when other modalities have failed. Consider initiating high-frequency oscillatory ventilation in patients failing conventional ventilatory modes. C Mechanical ventilation: avoid initiating obligatory mechanical ventilation in the absence of respiratory failure solely for the purpose of overcoming chest wall instability. D Show 2 more 3. Medical management https://web.pathway.md/diseases/recgbkpqWjLA9ypLs 3/10 6/29/23, 3:20 AM Blunt chest trauma Pathway Management of pain: As per EAST 2016 guidelines, consider administering epidural analgesia over nonregional modalities of pain control (IV or enteral analgesics, such as opioids, acetaminophen, and NSAIDs) for the management of pain in adult patients with blunt thoracic trauma. C Show 2 more As per EAST 2012 guidelines: Administer optimal analgesia to minimize the likelihood of respiratory failure and ensuing ventilatory support. Use epidural catheter as the preferred mode of analgesia delivery in patients with a severe flail chest injury. B Insufficient evidence regarding the effectiveness of paravertebral analgesia in patients with trauma. Consider administering paravertebral analgesia in certain situations where epidural analgesia is contraindicated. I Management of pneumothorax (oxygen supplementation): Aim at an oxygen saturation of 94-98% in most cases of pneumothorax if the patient is not at risk of hypercapnic respiratory failure. B Use high-concentration oxygen (15 L/min flow rate via reservoir mask) in patients with pneumothorax under hospital observation without drainage unless the patient is at risk of hypercapnic respiratory failure. B Management of pneumothorax (drainage): consider observing stable patients with occult pneumothorax (not seen on CXR), regardless of positive pressure ventilation. C Show 2 more Management of hemothorax: consider draining all hemothoraces, regardless of size. C Show 4 more Management of pulmonary contusion (fluid resuscitation): Administer fluid resuscitation with isotonic crystalloid or colloid solution as necessary to maintain signs of adequate tissue perfusion in patients with pulmonary contusion-flail chest. Avoid administering unnecessary fluid once adequately resuscitated. B Consider placing a pulmonary artery catheter to avoid fluid overload during resuscitation. C Management of pulmonary contusion (respiratory support): consider initiating a trial of mask CPAP in combination with optimal regional anesthesia in alert, compliant patients with marginal respiratory status. C Show 4 more Management of pulmonary contusion (diuretics): consider administering diuretics in the setting of hydrostatic fluid overload, as evidenced by elevated pulmonary capillary wedge pressures, in hemodynamically stable patients or in the setting of known concurrent congestive HF. C Management of pulmonary contusion (corticosteroids): do not use corticosteroids in the management of patients with pulmonary contusion. D Management of pulmonary contusion (analgesics): https://web.pathway.md/diseases/recgbkpqWjLA9ypLs 4/10 6/29/23, 3:20 AM Blunt chest trauma Pathway Administer optimal analgesia to minimize the likelihood of respiratory failure and ensuing ventilatory support. Use epidural catheter as the preferred mode of analgesia delivery in patients with a severe flail chest injury. B Insufficient evidence regarding the effectiveness of paravertebral analgesia in patients with trauma. Consider administering paravertebral analgesia in certain situations where epidural analgesia is contraindicated. I Management of pulmonary contusion (chest physiotherapy): offer aggressive chest physiotherapy to minimize the likelihood of respiratory failure and ensuing ventilatory support. B Management of rib fracture, surgical stabilization: As per EAST 2017 guidelines: Consider performing surgical reduction and internal fixation of rib fracture to decrease mortality, shorten the duration of mechanical ventilation, ICU and hospital length of stay, the incidence of pneumonia, and the need for tracheostomy in adult patients with flail chest after blunt trauma. C Insufficient evidence to recommend surgical reduction and internal fixation of rib fractures in adult patients with non-flail rib fractures after blunt trauma. I As per EAST 2012 guidelines: Consider performing surgical fixation of the flail chest in patients with severe flail chest failing to wean from the ventilator or when thoracotomy is required for other reasons. C Insufficient evidence to recommend any type of proprietary implant for surgical fixation of rib fractures. Consider using rib plating or wrapping devices over intramedullary wires. I Management of rib fracture, analgesics: As per AOTA/EAST 2023 guidelines: Consider administering NSAIDs (such as ketorolac) for pain management in adult patients with a traumatic fracture. C Insufficient evidence to recommend for the preference of selective NSAIDs (COX-2 inhibitors) or nonselective NSAIDs. I As per EAST 2012 guidelines, administer optimal analgesia to minimize the likelihood of respiratory failure and ensuing ventilatory support. Use epidural catheter as the preferred mode of analgesia delivery in patients with a severe flail chest injury. B Management of aortic injury (setting of care): manage and treat patients with blunt traumatic thoracic aortic injury at a trauma center with the facilities and expertise to treat aortic pathology. B Management of aortic injury, expectant management: As per AHA/ACC 2022 guidelines: Offer nonoperative management and follow-up imaging in patients with grade 1 blunt traumatic thoracic aortic injury. B Consider offering nonoperative management and follow-up surveillance imaging in patients with grade 2 blunt traumatic thoracic aortic injury without high-risk imaging features. C https://web.pathway.md/diseases/recgbkpqWjLA9ypLs 5/10 6/29/23, 3:20 AM Blunt chest trauma Pathway As per SVS 2011 guidelines, consider offering expectant management with serial imaging in patients with type I aortic injuries (intimal tear). C Management of aortic injury, anti-impulsive therapy: As per AHA/ACC 2022 guidelines, initiate anti-impulse therapy to reduce the risk of injury extension and rupture in patients with blunt traumatic thoracic aortic injury, except with hypotension or hypovolemic shock. B As per SIR 2020 guidelines, initiate antihypertensive and anti-impulse medications with close symptomatic and/or radiographic follow-up in patients with grade 1-2 aortic injuries (minimal aortic injury). B Management of aortic injury, TEVAR, indications: As per AHA/ACC 2022 guidelines: Perform an aortic intervention in patients with grade 3-4 blunt traumatic thoracic aortic injury and nonprohibitive comorbidities or injuries. B Consider performing an aortic intervention in patients with grade 2 blunt traumatic thoracic aortic injury with high-risk imaging features. C As per SIR 2020 guidelines, perform an aortic intervention in patients with anatomically favorable grade 3-4 aortic injuries. B Management of aortic injury, TEVAR, timing: As per EAST 2015 guidelines, consider performing delayed repair in patients diagnosed with blunt traumatic aortic injury while ensuring effective BP control with antihypertensive medications. C As per SVS 2011 guidelines, consider performing urgent (< 24 hours) repair at the latest before hospital discharge. C Management of aortic injury, TEVAR, choice of approach: As per AHA/ACC 2022 guidelines, perform TEVAR over open repair in patients with blunt traumatic thoracic aortic injury with appropriate anatomy meeting indications for repair. B As per SIR 2020 guidelines, perform emergent TEVAR in patients with anatomically favorable grade 3-4 aortic injuries. B As per EAST 2015 guidelines, perform endovascular repair in patients with blunt traumatic aortic injury without contraindications to endovascular repair. A As per ESC 2014 guidelines, perform TEVAR over open surgery in patients with traumatic aortic injury with suitable anatomy requiring intervention. B As per SVS 2011 guidelines, consider performing endovascular repair over open surgical repair or nonoperative management in patients with traumatic thoracic aortic injuries requiring intervention. Consider performing endovascular repair regardless of age if anatomically suitable. C Management of aortic injury (TEVAR, technical considerations): Consider using the femoral access technique. C https://web.pathway.md/diseases/recgbkpqWjLA9ypLs 6/10 6/29/23, 3:20 AM Blunt chest trauma Pathway Avoid performing routine spinal drainage. D Management of aortic injury (left subclavian artery revascularization): consider performing selective revascularization of the left subclavian artery. C Management of aortic injury (resuscitative thoracotomy): Consider performing emergency department thoracotomy in patients presenting pulseless to the emergency department with signs of life after blunt injury. C Avoid performing emergency department thoracotomy in patients presenting pulseless to the emergency department without signs of life after blunt injury. D Management of aortic injury (surveillance imaging): Consider obtaining surveillance imaging at intervals appropriate for the repair approach and location after aortic repair in patients with blunt traumatic aortic injury. C Consider obtaining surveillance CT at 1 month, 6 months, and 12 months after the diagnosis and, if stable, at appropriate intervals thereafter (depending on the type and extent of the injury) in patients with unrepaired blunt traumatic aortic injury. C 4. Therapeutic procedures Technical considerations for chest drainage (analgesic premedication): Consider administering analgesia as premedication to reduce pain associated with chest drains. E Administer local lidocaine 1% before the procedure, paying particular attention to the skin, periosteum, and pleura. E Technical considerations for chest drainage (antibiotic prophylaxis): consider administering antibiotic prophylaxis in trauma patients requiring chest drains, especially after penetrating trauma. B Technical considerations for chest drainage (insertion technique): perform pleural aspirations and chest drain insertions in a clean area using a full aseptic technique, E including gowns, drapes, sterile gloves, and skin cleansing. B Show 16 more Technical considerations for chest drainage (care of inserted drains): do not clamp the bubbling chest tube. D Show 4 more Technical considerations for chest drainage, follow-up imaging: As per EAST 2011 guidelines, obtain chest CT to determine whether significant undrained fluid exists in patients with persistent opacity on CXR after tube thoracostomy. B As per BTS 2010 guidelines, do not obtain CXRs after a simple pleural aspiration unless air is withdrawn, the procedure is difficult, multiple attempts are required, or the patient becomes symptomatic. D https://web.pathway.md/diseases/recgbkpqWjLA9ypLs 7/10 6/29/23, 3:20 AM Blunt chest trauma Pathway 5. Surgical interventions Indications for surgery: Decide on the surgical intervention based on patient physiology rather than absolute numbers of initial or persistent output. B Consider performing surgical exploration in case of 1,500 mL via a chest tube in any 24-hour period, regardless of mechanism. C Clinical findings Symptoms Past events Chest pain Blunt chest trauma Shortness of breath Motor vehicle accident Vital signs Vascular exam Signs of shock Hypovolemia Tachypnea Respiratory exam O2 saturation Chest wall crepitus Neurological exam Chest wall tenderness Altered mental status Flail chest Paradoxical movement of the chest wall Integument exam Chest wall ecchymosis Lab findings Seatbelt sign serum troponin I ECG findings serum troponin T Cardiac arrhythmias Imaging findings St-segment changes Hemothorax Pericardial effusion Pleural effusion Pneumothorax Rib fracture Studies 2023 OptiTHO In adult patients admitted to the ICU within 48 hours after high-risk blunt chest trauma, non-severe hypoxemia, and no evidence of acute respiratory failure, early noninvasive ventilation and high-flow https://web.pathway.md/diseases/recgbkpqWjLA9ypLs 8/10 6/29/23, 3:20 AM Blunt chest trauma Pathway nasal oxygen therapy were not superior to late noninvasive ventilation and conventional oxygen therapy with respect to endotracheal intubation for delayed respiratory failure. C dric Carri et al. Crit Care. 2023 Apr 26. References 1. Eric M Isselbacher, Ourania Preventza, James Hamilton Black rd et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. Circulation. 2022 Nov 2. Open 2. Rochwerg B, Brochard L, Elliott MW et al. Official ERS/ATS clinical practice guidelines: noninvasive ventilation for acute respiratory failure. Eur Respir J. 2017 Aug 31;50 2 . pii: 1602426. Open 3. Nathan T Mowery, Oliver L Gunter, Bryan R Collier et al. Practice management guidelines for management of hemothorax and occult pneumothorax. J Trauma. 2011 Feb;70 2 510 8. Open 4. Patrick B Murphy, George Kasotakis, Elliott R Haut et al. Efficacy and safety of non-steroidal anti- inflammatory drugs NSAIDs) for the treatment of acute pain after orthopedic trauma: a practice management guideline from the Eastern Association for the Surgery of Trauma and the Orthopedic Trauma Association. Trauma Surg Acute Care Open. 2023 Feb 21;8 1):e001056. Open 5. W Anthony Lee, Jon S Matsumura, R Scott Mitchell et al. Endovascular repair of traumatic thoracic aortic injury: clinical practice guidelines of the Society for Vascular Surgery. J Vasc Surg. 2011 Jan;53 1 187 92. Open 6. Tom Havelock, Richard Teoh, Diane Laws et al. Pleural procedures and thoracic ultrasound: British Thoracic Society Pleural Disease Guideline 2010. Thorax. 2010 Aug;65 Suppl 2:ii61 76. Open 7. Bruce Simon, James Ebert, Faran Bokhari et al. Management of pulmonary contusion and flail chest: an Eastern Association for the Surgery of Trauma practice management guideline. J Trauma Acute Care Surg. 2012 Nov;73 5 Suppl 4 S351 61. Open 8. Fox N, Schwartz D, Salazar JH et al. Evaluation and management of blunt traumatic aortic injury: a practice management guideline from the Eastern Association for the Surgery of Trauma. J Trauma Acute Care Surg. 2015 Jan;78 1 136 46. Open 9. Alexander Levitov, Heidi L Frankel, Michael Blaivas et al. Guidelines for the Appropriate Use of Bedside General and Cardiac Ultrasonography in the Evaluation of Critically Ill Patients-Part II Cardiac Ultrasonography. Crit Care Med. 2016 Jun;44 6 1206 27. Open 10. N.D. Kitrey Chair), F. Campos-Juanatey, P. Hallscheidt et al. EAU Guidelines on Urological Trauma. EAU. 2022. Open 11. Kasotakis G, Hasenboehler EA, Streib EW et al. Operative fixation of rib fractures after blunt trauma: A practice management guideline from the Eastern Association for the Surgery of Trauma. J Trauma Acute Care Surg. 2017 Mar;82 3 618 626. Open 12. Siddharth A Padia, Christopher R Ingraham, John M Moriarty et al. Society of Interventional Radiology Position Statement on Endovascular Intervention for Trauma. J Vasc Interv Radiol. 2020 Mar;31 3 363 369.e2. Open https://web.pathway.md/diseases/recgbkpqWjLA9ypLs 9/10 6/29/23, 3:20 AM Blunt chest trauma Pathway 13. Mark J Seamon, Elliott R Haut, Kyle Van Arendonk et al. An evidence-based approach to patient selection for emergency department thoracotomy: A practice management guideline from the Eastern Association for the Surgery of Trauma. J Trauma Acute Care Surg. 2015 Jul;79 1 159 73. Open 14. Clancy K, Velopulos C, Bilaniuk JW et al. Screening for blunt cardiac injury: an Eastern Association for the Surgery of Trauma practice management guideline. J Trauma Acute Care Surg. 2012 Nov;73 5 Suppl 4 S301 6. Open 15. Morey AF, Brandes S, Dugi DD rd et al. Urotrauma: AUA guideline. J Urol. 2014 Aug;192 2 327 35. Open 16. Erbel R, Aboyans V, Boileau C et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology ESC . Eur Heart J. 2014 Nov 1;35 41 2873 926. Open 17. Expert Panels on Cardiac Imaging and Thoracic Imaging, Jadranka Stojanovska, Lynne M Hurwitz Koweek et al. ACR Appropriateness Criteria Blunt Chest Trauma-Suspected Cardiac Injury. J Am Coll Radiol. 2020 Nov;17 11S S380 S390. Open 18. O'Driscoll BR, Howard LS, Earis J et al. BTS guideline for oxygen use in adults in healthcare and emergency settings. Thorax. 2017 Jun;72 Suppl 1):ii1-ii90. Open 19. Galvagno SM Jr, Smith CE, Varon AJ et al. Pain management for blunt thoracic trauma: A joint practice management guideline from the Eastern Association for the Surgery of Trauma and Trauma Anesthesiology Society. J Trauma Acute Care Surg. 2016 Nov;81 5 936 951. Open 20. Expert Panel on Thoracic Imaging:, Travis S Henry, Edwin F Donnelly et al. ACR Appropriateness Criteria Rib Fractures. J Am Coll Radiol. 2019 May;16 5S S227 S234. Open 21. C dric Carri , Benjamin Rieu, Antoine Benard et al. Early non-invasive ventilation and high-flow nasal oxygen therapy for preventing endotracheal intubation in hypoxemic blunt chest trauma patients: the OptiTHO randomized trial. Crit Care. 2023 Apr 26;27 1 163. Open 22. Heidi L Frankel, Andrew W Kirkpatrick, Mahmoud Elbarbary et al. Guidelines for the Appropriate Use of Bedside General and Cardiac Ultrasonography in the Evaluation of Critically Ill Patients-Part I General Ultrasonography. Crit Care Med. 2015 Nov;43 11 2479 502. Open https://web.pathway.md/diseases/recgbkpqWjLA9ypLs 10/10
Guideline sources The following summarized guidelines for the evaluation and management of bone metastasis are prepared by our editorial team based on guidelines from the European Society of Medical Oncology (ESMO 2018; 2014) and the American Society for Radiation Oncology (ASTRO 2017). 1 2 3 4 4 5 6 Definition Bone metastasis is the development of secondary tumors within the bone of cancer patients characterized by pathological osteolytic, osteoblastic lesions or mixed. 4 Epidemiology Bone metastasis is caused by disseminated tumor cells from various common malignancies including breast, prostate, lung, and kidney cancers, or multiple myeloma. 4 Disease course The spread of tumor cells from the primary tumor to the bone results in pathologic osteolytic, osteoblastic (sclerotic), or mixed bone metastasis, which causes clinical presentation of severe pain, impaired mobility, pathologic fractures, spinal cord compression, bone marrow aplasia, and hypercalcemia. Disease progression is associated with increased morbidity and mortality. 5 https://web.pathway.md/diseases/rec8KskRG3U2FAb2n 1/5 6/29/23, 3:20 AM Bone metastasis Pathway Prognosis and risk of recurrence The 1-year survival of bone metastasis from breast, prostate, and lung cancers are 51%, 35%, and 10%, respectively. 6 Calculator Calculator Pathway Eastern Cooperative Oncology G Karnofsky performance status s Bone meta Managemen Guidelines 1. Screening and diagnosis Populations at risk: bone metastases are especially prevalent in advanced breast and prostate cancer. Pathophysiology: tumor cells home to the hematopoietic stem cell niches in bone, and these calls may remain dormant for long periods of time before progressing to overt metastases. Differential diagnosis: the differential diagnosis of metastatic bone disease includes osteoporosis, degenerative disease, and Paget's disease of bone. Complications of bone metastases: Metastatic bone disease is a major cause of morbidity in cancer. Complications of metastatic bone disease include pain, impaired mobility, pathological fracture, spinal cord compression, hypercalcemia and suppression of bone marrow function. 2. Diagnostic investigations Clinical assessment: assess symptoms and activity status in patients with bone metastases. Biochemical markers: avoid the routine use of biochemical markers of bone metabolism in patients with bone metastasis, although these may provide information on prognosis and response to bone-specific treatments. Bone scan: the isotope bone scan yields little information about the nature of skeletal pathology, even though it is a sensitive test to detect its presence. Computed tomography and magnetic resonance imaging: CT and MRI are the best imaging modalities to obtain structural information on skeletal damage from metastatic bone disease. Positron emission tomography: positron emission tomography scan provides functional information that may aid in diagnosis. https://web.pathway.md/diseases/rec8KskRG3U2FAb2n 2/5 6/29/23, 3:20 AM Bone metastasis Pathway DEXA scan: obtain DEXA scans in patients at risk for cancer treatment-induced accelerated bone loss. Skeletal radiography: skeletal radiography can identify response to treatment, but the information is delayed and the method insensitive. 3. Medical management General principles: provide multidisciplinary management to patients with metastatic bone disease, drawing from relevant expertise in radiation therapy, orthopedic surgery, radiology and palliative care medicine. B Indications for bone-targeted therapy: initiate bone-targeted therapy at diagnosis of metastatic bone disease, and continue such therapy indefinitely throughout the course of the disease. B Choice of antiresorptive agent: Use zoledronic acid as the most effective bisphosphonate for prevention of morbidity from metastatic bone disease. B Use denosumab as a more effective alternative to zoledronic acid for prevention of skeletal morbidity from solid tumours. B Management of bone pain: radium-223 is effective in reducing skeletal-related events, decreasing pain, and improving survival in castrate-resistant prostate cancer patients. A 4. Therapeutic procedures Indications for radiotherapy: offer radiotherapy as the treatment of choice for palliation of localized bone pain. B Dosing of radiotherapy: for patients with previously unirradiated painful bone metastases, high- quality data shows equivalent pain relief between a single 8 Gy fraction, 20 Gy in 5 fractions, 24 Gy in 6 fractions, and 30 Gy in 10 fractions. Inform patients that single fraction radiotherapy is associated with a higher incidence of retreatment to the same painful site than is fractionated treatment. A Radiotherapy for persistent or recurrent pain: consider re-treatment in patients with persistent or recurrent pain for > 1 month following external beam radiation therapy to symptomatic, peripheral bone metastases, while adhering to normal tissue dosing constraints described in the available literature. B Stereotactic body radiation therapy: consider advanced radiation therapy techniques like stereotactic body radiation therapy as the primary treatment for painful spine bone lesions or for spinal compression in the setting of a clinical trial or data registry. B Other procedural interventions: the use of surgery, radionuclides, bisphosphonates, or kyphoplasty/vertebroplasty does not obviate the need for external beam radiation therapy for patients with painful bone metastases. B https://web.pathway.md/diseases/rec8KskRG3U2FAb2n 3/5 6/29/23, 3:20 AM Bone metastasis Pathway 5. Preventative measures Prevention of bone metastases: offer bisphosphonates to postmenopausal women with breast cancer as they reduce the frequency of bone metastases and improve survival. A Prevention of treatment-induced bone loss: offer a bisphosphonate or denosumab to prevent bone loss associated with the use of ovarian suppressants or aromatase inhibitors in early breast cancer, or with the use of androgen deprivation therapy in prostate cancer. B Clinical findings Symptoms Past medical history Bone pain Breast cancer Confusion Cancer Constipation Kidney cancer Fecal incontinence Lung cancer Muscle weakness Lymphoma Nausea Multiple myeloma Urinary incontinence Prostate cancer Vomiting Thyroid cancer Weakness in the legs Lab findings serum calcium References 1. Coleman R, Body JJ, Aapro M et al. Bone health in cancer patients: ESMO Clinical Practice Guidelines. Ann Oncol. 2014 Sep;25 Suppl 3:iii124 37. Open 2. Lutz S, Balboni T, Jones J et al. Palliative radiation therapy for bone metastases: Update of an ASTRO Evidence-Based Guideline. Pract Radiat Oncol. 2017 Jan Feb;7 1 4 12. Open 3. Fallon M, Giusti R, Aielli F et al. Management of cancer pain in adult patients: ESMO Clinical Practice Guidelines. Ann Oncol. 2018 Jul 24. Open 4. Mark Esposito, Theresa Guise, Yibin Kang. The Biology of Bone Metastasis. 2018 Jun 1;8 6):a031252.2018 Jun 1;8 6):a031252. Open 5. Filipa Macedo, Katia Ladeira, Filipa Pinho et al. Bone Metastases: An Overview. 2017 May 9;11 1 321.2017 May 9;11 1 321. Open 6. Elisabeth Svensson, Christian F Christiansen, Sinna P Ulrichsen et al. Survival after bone metastasis by primary cancer type: a Danish population-based cohort study. 2017 Sep 11;7 9):e016022.2017 Sep 11;7 9):e016022. Open https://web.pathway.md/diseases/rec8KskRG3U2FAb2n 4/5 6/29/23, 3:20 AM Bone metastasis Pathway 7. American Academy of Hospice and Palliative Medicine. Choosing Wisely AAHPM recommendations. Choosing Wisely. 2013. Open 8. American Society for Radiation Oncology. Choosing Wisely ASRO recommendations. Choosing Wisely. 2017. Open 9. Handkiewicz-Junak D, Poeppel TD, Bodei L et al. EANM guidelines for radionuclide therapy of bone metastases with beta-emitting radionuclides. Eur J Nucl Med Mol Imaging. 2018 May;45 5 846 859. Open 10. Handkiewicz-Junak D, Poeppel TD, Bodei L et al. EANM guidelines for radionuclide therapy of bone metastases with beta-emitting radionuclides. Eur J Nucl Med Mol Imaging. 2018 May;45 5 846 859. Open 11. Muhammad Umar Jawad, MD and Sean P. Scully, MD et al. In Brief: Classifications in Brief: Mirels Classification: Metastatic Disease in Long Bones and Impending Pathologic Fracture. Clin Orthop Relat Res. 2010 Oct; 468 10 2825 2827. Open https://web.pathway.md/diseases/rec8KskRG3U2FAb2n 5/5
Guideline sources The following summarized guidelines for the evaluation and management of borderline personality disorder are prepared by our editorial team based on guidelines from the National Health and Medical Research Council of Australia (NHMRC 2019). 1 Guidelines 1. Screening and diagnosis Identification and diagnostic assessment (adults): Consider assessment for borderline personality disorder or referral for psychiatric assessment in patients with any of the following: frequent suicidal or self-harming behavior marked emotional instability multiple co-occurring psychiatric conditions nonresponse to established treatments for current psychiatric symptoms a high level of functional impairment. C Show 2 more https://web.pathway.md/diseases/recHQKjxNXMr3WQiR 1/5 6/29/23, 3:20 AM Borderline personality disorder Pathway Identification and diagnostic assessment (children and adolescents): Consider assessment for borderline personality disorder in patients 12-18 years old with any of the following: frequent suicidal or self-harming behavior marked emotional instability multiple co-occurring psychiatric conditions nonresponse to established treatments for current psychiatric symptoms a high level of functional impairment. C Show 3 more 2. Medical management Setting of care: Use community-based mental health services (public and private) for the majority of a patient's treatment for borderline personality disorder. B Offer treatment for borderline personality disorder through a range of services, as appropriate to the individual's current clinical presentation, course of illness, needs and (if applicable) preferences. B Multimodal therapy: Do not routinely add pharmacotherapy to psychological interventions in the treatment of patients with borderline personality disorder. D Consider psychoeducation, family therapy and/or group sessions in addition to one-to-one psychological therapies, as appropriate to the patient's needs. C Pharmacotherapy: do not use medications as primary therapy for borderline personality disorder, because they have only modest and inconsistent effects, and do not change the nature and course of the disorder. D Show 6 more Management of co-occuring health conditions: manage co-occurring mental illness (such as substance use disorder, mood disorder or eating disorder), if present, concurrently with the management of borderline personality disorder. B Show 4 more 3. Inpatient care Indications for inpatient admission: consider acute inpatient admission to provide structured crisis intervention for the treatment of patients who are suicidal or have significant co-occurring mental health conditions. C Show 3 more Family, partner and carer needs: https://web.pathway.md/diseases/recHQKjxNXMr3WQiR 2/5 6/29/23, 3:20 AM Borderline personality disorder Pathway Consider the needs of children and arrange assessment of their mental health and welfare needs if necessary when caring for parents with borderline personality disorder. C Determine whether the patient with borderline personality disorder (particularly during a crisis) has dependent children, and ensure that they are properly cared for (such as refer to a social worker). B 4. Nonpharmacologic interventions Structured psychological therapy: provide structured psychological therapies that are specifically designed for borderline personality disorder to patients with borderline personality disorder, conducted by one or more adequately trained and supervised health professionals. B Show 2 more Dialectical behavior therapy: As per NHMRC 2019 guidelines: Offer comprehensive dialectical behavior therapy in female patients with borderline personality disorder when reduction in self-harm is a treatment goal. B Offer comprehensive dialectical behavior therapy in female patients with borderline personality disorder when reduction in anger, anxiety, or depression is a treatment goal. B 5. Specific circumstances Adolescent patients: offer time-limited structured psychological therapies that are specifically designed for borderline personality disorder to patients 14-18 years old with borderline personality disorder or with clinically significant features of borderline personality disorder. B Show 3 more 6. Follow-up and surveillance Referral for specialized service: consider referring patients with severe and/or enduring borderline personality disorder to a suitable specialized borderline personality disorder service (where available) for assessment and ongoing care, if appropriate. C Supporting family, partners and caregivers: refer families, partners, and carers of patients with borderline personality disorder to support services and/or psychoeducation programs on borderline personality disorder, where available. B Show 5 more Caring for families, partners and caregivers: Support families, partners and carers by referring or directing them to: general family counselling and psychoeducation with a focus on borderline personality disorder structured family programs specific to borderline personality disorder https://web.pathway.md/diseases/recHQKjxNXMr3WQiR 3/5 6/29/23, 3:20 AM Borderline personality disorder Pathway peer support programs such as carer-led programs that educate families/carers on borderline personality disorder respite services. B Show 4 more 7. Quality improvement Health professionals' roles: Health professionals at all levels of the healthcare system and within each type of service setting should: acknowledge that borderline personality disorder treatment is a legitimate use of healthcare services be able to recognize borderline personality disorder presentations be aware of general principles of care for patients with borderline personality disorder and specific effective borderline personality disorder treatments provide appropriate care (including non-specific mental health management, specific treatments for borderline personality disorder and treatment for co-occurring mental illness) according to their level of training and skill refer the patient to a specialized borderline personality disorder service or other services as indicated undertake continuing professional development to maintain and enhance their skills. B Coordinating care: clinicians treating patients with borderline personality disorder should follow a stepped-care approach in which an individual's usual care is based on the least intensive treatment (such as general practice care and regular contact with a community mental health service), and referral to more intensive treatment (such as crisis intervention, a specialized borderline personality disorder service, or specialized borderline personality disorder programs) is provided when indicated. B Show 6 more Supporting healthcare professionals: those responsible for planning or managing services that provide care for patients with borderline personality disorder should ensure that health professionals receive training in borderline personality disorder management. B Show 4 more References 1. Michael H Stone. Clinical Practice Guideline for the Management of Borderline Personality Disorder. Psychodyn Psychiatry. Spring 2019;47 1 5 26. Open 2. James Sall, Lisa Brenner, Amy M Millikan Bell et al. Assessment and Management of Patients at Risk for Suicide: Synopsis of the 2019 U.S. Department of Veterans Affairs and U.S. Department of Defense Clinical Practice Guidelines. Ann Intern Med. 2019 Sep 3;171 5 343 353. Open https://web.pathway.md/diseases/recHQKjxNXMr3WQiR 4/5 6/29/23, 3:20 AM Borderline personality disorder Pathway 3. Cardasis W, Hochman JA, Silk KR. Transitional objects and borderline personality disorder. Am J Psychiatry. 1997 Feb;154 2 250 5. Open https://web.pathway.md/diseases/recHQKjxNXMr3WQiR 5/5
Guideline sources The following summarized guidelines for the evaluation and management of botulism are prepared by our editorial team based on guidelines from the Center for Disease Control (CDC 2021). 1 Guidelines 1. Screening and diagnosis Differential diagnosis: suspect botulism in patients with suspected myasthenia gravis or Guillain- Barr syndrome and in patients with unexplained symmetric cranial nerve palsies, with or without paresis of other muscles. E 2. Diagnostic investigations History and physical examination: Elicit history of possible exposures to well-described sources of botulinum toxin, while keeping in mind that the absence of such exposures does not exclude the possibility of botulism. E https://web.pathway.md/diseases/rec7U2yB1IZOUVnV7 1/4 6/29/23, 3:20 AM Botulism Pathway Perform thorough, serial neurologic examinations to detect neurologic deficits of botulism and assess their progression. E Laboratory testing: discuss specimen collection with the expert consultant from the local or state health department. E Show 2 more Electrodiagnostic studies: consider obtaining electrodiagnostic studies (such as repetitive nerve stimulation, EMG, and nerve conduction studies) when feasible to assist in the diagnosis of suspected botulism. E 3. Medical management Contacting local or state health department: contact the local or state health department's emergency on-call staff immediately when botulism is suspected to arrange an emergency expert clinical consultation and, when indicated, request botulinum antitoxin. E Antitoxin therapy: administer botulinum antitoxin in patients with suspected botulism as early as possible in the course of illness, recognizing that the greatest benefit accrues within the first 2 days of illness onset. Administer botulinum antitoxin in symptomatic patients with suspected botulism on the basis of clinical findings. Do not await laboratory confirmation because results might take several days, and they can be negative in patients with botulism. E Show 6 more Other agents: administer aminoglycosides, magnesium, clindamycin, tetracycline, or calcium in patients with botulism only after careful consideration and with appropriate monitoring. E Supportive care: provide meticulous attention to bladder and bowel care and the prevention of complications, such as UTIs, deep vein thrombosis, and pressure ulcers. E Show 5 more 4. Inpatient care Serial clinical assessment (neurologic deficits): obtain frequent, serial neurologic examinations with an emphasis on cranial nerve palsies, swallowing ability, respiratory status, and extremity strength. Conduct the serial neurologic and other examinations by the same healthcare provider, when possible. E Show 2 more Serial clinical assessment (respiratory compromise): obtain frequent, serial monitoring of respiratory function in patients with botulism. Adjust the frequency of examinations on the basis of signs and symptoms, with very frequent examinations in patients with rapid progression and in patients with respiratory symptoms not required intubation. E Show 3 more Serial clinical assessment (hemodynamic status): Obtain continuous monitoring of cardiac rhythm and frequent measurements of BP. E https://web.pathway.md/diseases/rec7U2yB1IZOUVnV7 2/4 6/29/23, 3:20 AM Botulism Pathway Obtain frequent monitoring for urinary retention. E Serial clinical assessment (other complications): obtain frequent monitoring for constipation or ileus, dry mouth, and dry eyes. E 5. Specific circumstances Pediatric patients: Obtain close monitoring for worsening paralysis in pediatric patients with suspected foodborne botulism treated with botulinum antitoxin therapy according to the weight-based dose described in the package insert. E Consider administering retreatment in patients with a lack of response to the treatment when confidence in the diagnosis of botulism is substantial. E Pregnant patients: administer botulinum antitoxin therapy in pregnant patients with suspected foodborne botulism in the same manner as nonpregnant patients. E Show 3 more Clinical findings Symptoms Past events Abdominal pain Ingestion of home-canned food Blurry vision Ingestion of honey Constipation Ingestion of improperly preserved food Difficulty speaking Diplopia Vital signs Dry eyes Dry mouth Bradycardia Dysarthria Hypotension Dysphagia Tachycardia Facial weakness Respiratory exam Muscle weakness Nausea Respiratory distress Poor feeding Shortness of breath Vomiting Social history IV drug use Nasal cocaine use Neurological exam https://web.pathway.md/diseases/rec7U2yB1IZOUVnV7 3/4 6/29/23, 3:20 AM Botulism Pathway Ataxia Cranial nerve palsy Descending flaccid paralysis Hyporeflexia Mydriasis Ophthalmoplegia Paralysis References 1. Agam K Rao, Jeremy Sobel, Kevin Chatham-Stephens et al. Clinical Guidelines for Diagnosis and Treatment of Botulism, 2021. MMWR Recomm Rep. 2021 May 7;70 2 1 30. Open https://web.pathway.md/diseases/rec7U2yB1IZOUVnV7 4/4
Guideline sources The following summarized guidelines for the evaluation and management of bowel trauma are prepared by our editorial team based on guidelines from the World Society of Emergency Surgery (WSES 2022), the World Society of Emergency Surgery (WSES/AAST 2019), and the Eastern Association for the Surgery of Trauma (EAST 2012). 1 2 3 Guidelines 1. Diagnostic investigations Initial evaluation: obtain a primary survey, extended FAST, perform a physical examination, obtain a secondary survey, blood chemistry, check for vital signs followed by contrast-enhanced abdominal CT in awake and oriented patients with blunt abdominal trauma. A Show 4 more https://web.pathway.md/diseases/recAf3NCIUysPwkxL 1/4 6/29/23, 3:20 AM Bowel trauma Pathway Computed tomography: obtain CT in the presence of a seatbelt sign (a high index of suspicion for bowel injury). A Show 6 more 2. Diagnostic procedures Diagnostic peritoneal lavage: consider performing diagnostic peritoneal lavage as an adjunct to a negative laparoscopy to definitively exclude bowel injury, particularly in conjunction with the use of biomarkers. C Diagnostic laparoscopy: perform local wound exploration or screening laparoscopy to investigate for peritoneal violation, when CT does not identify hard signs of bowel injury, to guide toward a laparotomy or nonoperative management. Discharge patients without peritoneal violation. B Show 2 more 3. Medical management Non-operative management: consider offering nonoperative management at specialized centers in patients with penetrating abdominal trauma if the patient is hemodynamically compensated and cooperative. Recognize that nonoperative management might be more suitable for stab wounds when compared to gunshot wounds. C 4. Inpatient care Serial clinical assessment: Obtain at least 48 hours of serial clinical examinations, performed by consistent specialists or consultants, vital sign monitoring, and serial inflammatory markers testing during nonoperative management. B Admit patients with high-risk mechanisms (handlebar, seatbelt sign) and non-specific CT findings for observation including serial clinical examination. B 5. Perioperative care Perioperative prophylactic antibiotics: administer a single preoperative dose of prophylactic antibiotics with broad-spectrum aerobic and anaerobic coverage in all patients with penetrating abdominal trauma. A Show 3 more 6. Surgical interventions https://web.pathway.md/diseases/recAf3NCIUysPwkxL 2/4 6/29/23, 3:20 AM Bowel trauma Pathway Local wound exploration: perform local wound exploration or screening laparoscopy to investigate for peritoneal violation, when CT does not identify hard signs of bowel injury, to guide toward a laparotomy or nonoperative management. Discharge patients without peritoneal violation. B Indications for laparotomy: perform prompt surgical exploration in the presence of highly specific CT findings such as extraluminal air, extraluminal oral contrast, or bowel-wall defects. A Indications for bowel repair: recognize that the delay in the diagnosis of bowel injury is linked to increased morbidity and mortality. B Show 6 more 7. Specific circumstances Patients with duodenal injury (evaluation): obtain extended FAST for detecting free fluid and solid organ injury. A Show 7 more Patients with duodenal injury (non-operative management): use hemodynamic stability as the key factor in determining management strategy in patients with duodenal injury. B Show 4 more Patients with duodenal injury (operative management): perform immediate operative intervention in hemodynamically unstable (WSES class IV) patients and in patients with peritonitis, bowel evisceration, or impalement. B Show 4 more 8. Follow-up and surveillance Follow-up: obtain long-term follow-up in patients with blunt abdominal trauma to identify the sequelae of mesenteric injuries. B Clinical findings Symptoms Past events Abdominal pain Blunt abdominal trauma Penetrating abdominal trauma Vital signs Abdominal exam Hypotension Signs of shock Abdominal distension Tachycardia Peritoneal findings Imaging findings https://web.pathway.md/diseases/recAf3NCIUysPwkxL 3/4 6/29/23, 3:20 AM Bowel trauma Pathway Pneumoperitoneum References 1. Luke Smyth, Cino Bendinelli, Nicholas Lee et al. WSES guidelines on blunt and penetrating bowel injury: diagnosis, investigations, and treatment. 10.1186/s13017 022 00418-y. Open 2. Federico Coccolini, Leslie Kobayashi, Yoram Kluger et al. Duodeno-pancreatic and extrahepatic biliary tree trauma: WSES AAST guidelines. World J Emerg Surg. 2019 Dec 11;14 56. Open 3. Goldberg SR, Anand RJ, Como JJ et al. Prophylactic antibiotic use in penetrating abdominal trauma: an Eastern Association for the Surgery of Trauma practice management guideline. J Trauma Acute Care Surg. 2012 Nov;73 5 Suppl 4 S321 5. Open https://web.pathway.md/diseases/recAf3NCIUysPwkxL 4/4
Guideline sources The following summarized guidelines for the evaluation and management of bowen disease are prepared by our editorial team based on guidelines from the British Association of Dermatologists (BAD 2023), the U.S. Preventive Services Task Force (USPSTF 2023; 2018), the American Society of Plastic Surgeons (ASPS 2021), the British Photodermatology Group (BPG/BAD 2019), and the European Dermatology Forum (EDF 2019). 1 2 3 4 5 6 Calculator TNM classification for nonmelan Guidelines https://web.pathway.md/diseases/recvKY60ZslrpQZeP 1/5 6/29/23, 3:21 AM Bowen disease Pathway 1. Screening and diagnosis Indications for screening: As per USPSTF 2023 guidelines, insufficient evidence to assess the balance of benefits and harms of visual skin examination by a clinician to screen for skin cancer in adolescents and adults. I As per USPSTF 2018 guidelines, insufficient evidence to assess the balance of benefits and harms of counseling adult persons about skin self-examination to prevent skin cancer. I 2. Diagnostic procedures Biopsy: consider performing a punch biopsy or diagnostic excision when clinical and dermoscopic features are not sufficient for diagnosis of SCC in situ. C Curettage: consider performing curettage for both diagnosis and treatment, depending on the clinical scenario and the operator's judgment. Ensure histology specimens include the deep margin if an assessment of a potentially invasive tumor is required. E 3. Medical management Conservative management: consider offering conservative measures, including moisturizers (preferably urea-based) and skin surveillance, in patients with poor health and multiple SCC in situ lesions, especially on the lower legs, where there is a greater risk of long-term ulceration after treatment. Perform a biopsy if SCC is suspected. E Topical 5-fluorouracil: offer topical 5-fluorouracil (5%) monotherapy, once daily or BID for 3-4 weeks, in patients with small lesions (such as < 2 cm) of SCC in situ in low-risk sites and in patients refusing or unable to undergo alternative treatments. A Show 5 more Topical imiquimod: Consider offering topical imiquimod (5%), thrice weekly for 4 weeks (although prolonged treatment for 12 weeks may be required), in patients with SCC in situ at low-risk sites, if there is no suitable alternative, recognizing that a reduction in efficacy may be observed in immunosuppressed patients. C Consider offering topical imiquimod in patients with SCC in situ lesions located on the lower legs being inappropriate for, having contraindications to, or exhibiting inadequate response to topical 5-fluorouracil (5%), photodynamic therapy, laser, curettage with cautery, or surgery. C Topical tazarotene: insufficient evidence to recommend tazarotene in patients with SCC in situ. I 4. Therapeutic procedures https://web.pathway.md/diseases/recvKY60ZslrpQZeP 2/5 6/29/23, 3:21 AM Bowen disease Pathway Photodynamic therapy: As per BAD 2023 guidelines, offer conventional, red-light photodynamic therapy as a treatment option in patients with SCC in situ, particularly for poorly healing or cosmetically sensitive skin sites, multiple lesions, and large lesions. A Show 2 more As per BPG 2019 guidelines, offer photodynamic therapy as a treatment option in patients with SCC in situ, particularly for poorly healing or cosmetically sensitive skin sites, multiple lesions, and large-area lesions. A Show 2 more As per EDF 2019 guidelines: Offer photodynamic therapy in patients with SCC in situ. A Do not offer photodynamic therapy in patients with invasive SCC. D Cryotherapy: offer cryotherapy as a first-line treatment option in patients with small (< 2 cm) SCC in situ lesions. A Show 3 more Curettage with cautery: perform curettage with cautery as first-line treatment in patients with small SCC in situ lesions, especially if a histological diagnosis is desirable. A Show 2 more Laser therapy: consider offering laser treatment (if available) in patients with SCC in situ when other treatments have failed or are unsuitable. Consider preferring ablative CO laser over nonablative Nd-YAG. C Radiotherapy: consider offering radiotherapy in immunocompetent patients with SCC in situ only where the lesion is recurrent or refractory to other treatments or where surgery is inappropriate or is associated with high morbidity. B Show 4 more 5. Surgical interventions Standard surgical excision: perform standard surgical excision in patients with SCC in situ if there is diagnostic uncertainty regarding an invasive disease. A Show 3 more Mohs micrographic surgery: Consider performing Mohs micrographic surgery in patients with SCC in situ when tissue conservation is important, such as around the eyes and the nail unit. C Consider offering active treatment with any suitable alternative (such as standard surgical excision, curettage with cautery, photodynamic therapy, 5-fluorouracil, imiquimod, laser, or cryotherapy) where Mohs micrographic surgery is not available or not appropriate. Discuss the risk of functional impairment, including that posed by recurrence and its subsequent treatment. E https://web.pathway.md/diseases/recvKY60ZslrpQZeP 3/5 6/29/23, 3:21 AM Bowen disease Pathway 6. Patient education General counseling: provide educational material or a patient information leaflet on SCC in situ and for any proposed treatment modality. E 7. Preventative measures Sun protection: Counsel persons with fair skin types aged 6 months to 24 years and parents of young children about minimizing exposure to UV radiation to reduce the risk of skin cancer. B Offer counseling selectively in adults > 24 years with fair skin types about minimizing exposure to UV radiation to reduce the risk of skin cancer, taking into consideration the presence of risk factors for skin cancer. B 8. Follow-up and surveillance Skin reconstruction after tumor resection (timing): consider performing reconstructive surgery in a delayed (asynchronous) fashion in adult patients after skin cancer resection. C Skin reconstruction after tumor resection (perioperative antibiotics): consider discussing the management of antibiotics with adult patients undergoing reconstruction after skin cancer resection when relevant. C Show 3 more Skin reconstruction after tumor resection (perioperative antithrombotics): consider discussing the management of anticlotting agents with adult patients undergoing reconstruction after skin cancer resection when relevant. C Show 2 more Skin reconstruction after tumor resection (perioperative analgesics): consider discussing the management of pain with adult patients undergoing reconstruction after skin cancer resection when relevant. C Show 2 more Skin reconstruction after tumor resection (follow-up): consider obtaining postoperative follow- up assessment in adult patients undergoing reconstruction after skin cancer resection. C Follow-up: consider discharging most patients with SCC in situ safely following the completion of treatment. Exercise clinical judgment when deciding to offer a follow-up appointment. E Show 2 more Clinical findings Patient demographics Symptoms https://web.pathway.md/diseases/recvKY60ZslrpQZeP 4/5 6/29/23, 3:21 AM Bowen disease Pathway Elderly Itching Past medical history Social history Actinic keratosis Exposure to excessive sun Basal cell carcinoma Exposure to chemotherapy HPV infection Human immunodeficiency virus infection Immunocompromising condition Integument exam Erythematous plaque Fitzpatrick skin type 1 Hyperkeratotic lesion Scaly skin patch Skin plaque References 1. T H Wong, C A Morton, N Collier et al. British Association of Dermatologists and British Photodermatology Group guidelines for topical photodynamic therapy 2018. Br J Dermatol. 2019 Apr;180 4 730 739. Open 2. Ashish Sharma, Andrew J Birnie, Cristina Bordea et al. British Association of Dermatologists guidelines for the management of people with cutaneous squamous cell carcinoma in situ Bowen disease) 2022. Br J Dermatol. 2023 Feb 10;188 2 186 194. Open 3. US Preventive Services Task Force, Grossman DC, Curry SJ et al. Behavioral Counseling to Prevent Skin Cancer: US Preventive Services Task Force Recommendation Statement. JAMA. 2018 Mar 20;319 11 1134 1142. Open 4. Andrew Chen, John G Albertini, Jeremy S Bordeaux et al. Evidence-Based Clinical Practice Guideline: Reconstruction after Skin Cancer Resection. Plast Reconstr Surg. 2021 May 1;147 5 812e-829e. Open 5. C A Morton, R M Szeimies, N Basset-Seguin et al. European Dermatology Forum guidelines on topical photodynamic therapy 2019 Part 1 treatment delivery and established indications - actinic keratoses, Bowen's disease and basal cell carcinomas. J Eur Acad Dermatol Venereol. 2019 Dec;33 12 2225 2238. Open 6. US Preventive Services Task Force, Carol M Mangione, Michael J Barry et al. Screening for Skin Cancer: US Preventive Services Task Force Recommendation Statement. JAMA. 2023 Apr 18;329 15 1290 1295. Open https://web.pathway.md/diseases/recvKY60ZslrpQZeP 5/5
Guideline sources The following summarized guidelines for the evaluation and management of breastfeeding are prepared by our editorial team based on guidelines from the American Academy of Pediatrics (AAP 2022), the American College of Obstetricians and Gynecologists (ACOG 2019; 2018), the American College of Radiology (ACR 2018), the U.S. Preventive Services Task Force (USPSTF 2016), and the Academy of Breastfeeding Medicine (ABM 2013). 1 3 4 5 6 10 15 15 16 17 Definition Breastfeeding is nursing a child with breast milk, which is recommended as the exclusive source of nutrition for the first six months of infants life. 15 Epidemiology The stages of lactation include mammogenesis (growth of breast tissue) and lactogenesis. 15 Disease course Breast milk contains antibodies, enzymes, and hormones and breastfeeding promote infants growth and neurodevelopment, and maternal health too. Breastfed infants have a decreased incidence of gastrointestinal infection, acute otitis media, hypertension, total cholesterol, pain during procedures, atopic dermatitis, asthma, respiratory tract infections, childhood obesity, type 1 and T2DM, leukemia, necrotizing enterocolitis in premature infants, and sudden infant death syndrome. https://web.pathway.md/diseases/rec3ZX5DH7sabgCR8 1/5 6/29/23, 3:21 AM Breastfeeding Pathway Breastfeeding mothers also have decreased incidence of breast cancer, postnatal depression, ovarian cancer, retained gestational weight, T2DM. 16 Prognosis and risk of recurrence Breastfeeding initiation is associated with a significant reduction in total infant mortality with OR 0.81 (95% CI 0.68-0.97). 17 Guidelines 1. Diagnostic investigations Considerations for diagnostic imaging: do not interrupt breastfeeding after gadolinium administration. D Considerations for cancer screening imaging: obtain digital breast tomosynthesis or mammography with minor modifications to address increased mammographic density, increased breast vascularity, and duration of lactation in lactating females. B 2. Nonpharmacologic interventions Duration of breastfeeding: As per AAP 2022 guidelines, encourage exclusive breastfeeding for about six months with complementary food introduction at about 6 months and continued breastfeeding until 2 years or beyond, as mutually desired by the mother and her child. E As per ACOG 2018 guidelines, encourage exclusive breastfeeding for the first 6 months of life, with continued breastfeeding as complementary foods are introduced during the infant's first year or longer, as mutually desired by the mother and her infant. E As per ABM 2013 guidelines: Encourage exclusive breastfeeding for 6 months with continued breastfeeding with complementary foods until at least 24 months and thereafter as long as mutually desired. B Discuss the introduction of solid food at 6 months of age, emphasizing the need for high-iron solids and advise supplementing vitamins, such as vitamin D, K, or A in accordance with published standards. B 3. Patient education Maternal counseling: As per USPSTF 2016 guidelines, offer interventions during pregnancy and after birth to support breastfeeding. B As per ABM 2013 guidelines, introduce the subject of infant feeding in the first trimester and continue to express support of breastfeeding throughout the course of the pregnancy if providing https://web.pathway.md/diseases/rec3ZX5DH7sabgCR8 2/5 6/29/23, 3:21 AM Breastfeeding Pathway antenatal care for the mother. Consider offering a prenatal visit to become acquainted with the family during which the commitment to breastfeeding can be shown. Use open-ended questions, such as "What have you heard about breastfeeding?," to inquire about a feeding plan for this child. Provide educational material that highlights the many ways in which breastfeeding is superior to formula feeding. Identify patients with lactation risk factors such as flat or inverted nipples, history of breast surgery, no increase in breast size during pregnancy, or previous unsuccessful breastfeeding experience to enable individual breastfeeding care for her particular situation. B 4. Follow-up and surveillance Follow-up assessment: in many areas of the world, the first follow-up visit will be done by non- physician healthcare workers. In most European countries midwives care for the mother and infant in the days and weeks after discharge from the hospital. Mothers contact their pediatrician within the first 3 weeks of delivery for the infant's first check-up, which is covered by insurance. In this system, this is the first opportunity the pediatrician has to support breastfeeding. In other countries, such as Australia and New Zealand, routine medical care of infants is undertaken by general practitioners (family physicians), and infants may never visit a pediatrician. In countries such as the United States, where the postpartum care of the mother and infant is done by physicians or physician extenders (such as physician assistants, nurse practitioners), schedule a first infant follow-up visit 48-72 hours after hospital discharge or earlier if breastfeeding-related problems, such as excessive weight loss of > 7% or jaundice, are present at the time of hospital discharge. 5. Quality improvement Coordinated care: as per ABM 2013 guidelines, establish a written breastfeeding-friendly office. Collaborate with colleagues and office staff during development. Inform all new staff about the policy. Provide copies of your practice's policy to hospitals, physicians, and all healthcare professionals covering your practice for you. B Policies: as per ABM 2013 guidelines, set an example for your patients and community. Consider having a written breastfeeding employee policy and provide a lactation room with supplies for your employees who breastfeed or express milk at work. B Clinician training: as per ABM 2013 guidelines, educate clinicians regarding breastfeeding, beginning in the preclinical years. Areas of suggested education include the risks of artificial feeding, the physiology of lactation, management of common breastfeeding problems, and medical contraindications to breastfeeding. Make educational resources available for quick reference by healthcare professionals. Provide staff education and training to all, including front office staff, nurses, and medical assistants. B References https://web.pathway.md/diseases/rec3ZX5DH7sabgCR8 3/5 6/29/23, 3:21 AM Breastfeeding Pathway 1. No authors listed. ACOG Committee Opinion No. 756 Optimizing Support for Breastfeeding as Part of Obstetric Practice. Obstet Gynecol. 2018 Oct;132 4):e187-e196. Open 2. Lisa E Graves, Magali Robert, Victoria M Allen et al. Guideline No. 425b: Cannabis Use Throughout Women's Lifespans Part 2 Pregnancy, the Postnatal Period, and Breastfeeding. J Obstet Gynaecol Can. 2022 Apr;44 4 436 444.e1. Open 3. Grawey AE, Marinelli KA, Holmes AV et al. ABM Clinical Protocol #14 Breastfeeding-friendly physician's office: optimizing care for infants and children, revised 2013. Breastfeed Med. 2013 Apr;8 237 42. Open 4. Expert Panel on Breast Imaging, Roberta M diFlorio-Alexander, Priscilla J Slanetz et al. ACR Appropriateness Criteria Breast Imaging of Pregnant and Lactating Women. J Am Coll Radiol. 2018 Nov;15 11S S263 S275. Open 5. Joan Younger Meek, Lawrence Noble, Section on Breastfeeding. Policy Statement: Breastfeeding and the Use of Human Milk. Pediatrics. 2022 Jul 1;150 1):e2022057988. Open 6. Chirag Jain. ACOG Committee Opinion No. 723 Guidelines for Diagnostic Imaging During Pregnancy and Lactation. Obstet Gynecol. 2019 Jan;133 1 186. Open 7. No authors listed. Guideline: Protecting, Promoting and Supporting Breastfeeding in Facilities Providing Maternity and Newborn Services. Geneva: World Health Organization; 2017. Open 8. No authors listed. Breastfeeding Challenges: ACOG Committee Opinion, Number 820. Obstet Gynecol. 2021 Feb 1;137 2):e42-e53. Open 9. No authors listed. Guideline: Counselling of Women to Improve Breastfeeding Practices. Geneva: World Health Organization; 2018. Open 10. US Preventive Services Task Force, Kirsten Bibbins-Domingo, David C Grossman et al. Primary Care Interventions to Support Breastfeeding: US Preventive Services Task Force Recommendation Statement. JAMA. 2016 Oct 25;316 16 1688 1693. Open 11. No authors listed. Committee Opinion No. 722 Marijuana Use During Pregnancy and Lactation. Obstet Gynecol. 2017 Oct;130 4):e205-e209. Open 12. No authors listed. ACOG Committee Opinion No. 776 Immune Modulating Therapies in Pregnancy and Lactation. Obstet Gynecol. 2019 Apr;133 4):e287-e295. Open 13. No authors listed. Barriers to Breastfeeding: Supporting Initiation and Continuation of Breastfeeding: ACOG Committee Opinion Summary, Number 821. Obstet Gynecol. 2021 Feb 1;137 2 396 397. Open 14. J Mitchell, W Jones, E Winkley et al. Guideline on anaesthesia and sedation in breastfeeding women 2020 Guideline from the Association of Anaesthetists. Anaesthesia. 2020 Nov;75 11 1482 1493. Open 15. Christine M Dieterich, Julia P Felice, Elizabeth O'Sullivan et al. Breastfeeding and health outcomes for the mother-infant dyad. 2013 Feb;60 1 31 48.2013 Feb;60 1 31 48. Open 16. Pat Hoddinott, David Tappin, Charlotte Wright. Breast feeding. 2008 Apr 19;336 7649 881 7.2008 Apr 19;336 7649 881 7. Open 17. Julie L Ware, Aimin Chen, Ardythe L Morrow et al. Associations Between Breastfeeding Initiation and Infant Mortality in an Urban Population. 2019 Sep;14 7 465 474.2019 Sep;14 7 465 474. Open https://web.pathway.md/diseases/rec3ZX5DH7sabgCR8 4/5 6/29/23, 3:21 AM Breastfeeding Pathway 18. American Academy of Neurology. Choosing Wisely AAN recommendations. Choosing Wisely. 2016. Open 19. Pat Hoddinott, David Tappin, Charlotte Wright. Breast feeding. BMJ. 2008 Apr 19;336 7649 881 7. Open 20. Julie L Ware, Aimin Chen, Ardythe L Morrow et al. Associations Between Breastfeeding Initiation and Infant Mortality in an Urban Population. Breastfeed Med. 2019 Sep;14 7 465 474. Open 21. Shannon M Bates, Ian A Greer, Saskia Middeldorp et al. VTE, thrombophilia, antithrombotic therapy, and pregnancy: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e691S-e736S. Open https://web.pathway.md/diseases/rec3ZX5DH7sabgCR8 5/5
Guideline sources The following summarized guidelines for the evaluation and management of bronchiectasis are prepared by our editorial team based on guidelines from the European Respiratory Society (ERS 2021; 2017), the American College of Rheumatology (ACR 2021), the British Thoracic Society (BTS 2020), and the Canadian Thoracic Society (CTS 2012). 1 2 3 4 5 6 7 8 8 9 Definition Bronchiectasis is a chronic lung condition, which presents with permanent and abnormal dilation of the bronchi, and is characterized by mild to moderate airway obstruction and productive cough. 6 Epidemiology Causes of bronchiectasis include sequelae of previous bacterial infection or mycobacterial infection (e.g. following pneumonia, whooping cough, or tuberculosis), as well as allergic bronchopulmonary aspergillosis, rheumatoid arthritis, ulcerative colitis, toxic lung injury, primary ciliary dyskinesia, COPD, and AAT deficiency. Impairment of airway defense mechanisms due to inflammation and persistent infection results in bronchiectasis. 7 8 Di https://web.pathway.md/diseases/recSF4xa2pybKp3wS 1/7 6/29/23, 3:21 AM Bronchiectasis Pathway Disease course Clinical manifestations include productive cough, excessive sputum, hemoptysis, dyspnea, chest pain, and recurrent chest infections. The disease can progress to chronic respiratory failure. 8 Prognosis and risk of recurrence The in-hospital and 1-year mortality in patients with acute exacerbation of bronchiectasis is 9% and 30%, respectively. 9 Guidelines 1. Screening and diagnosis Diagnostic criteria: define clinically significant bronchiectasis as the presence of both permanent bronchial dilatation on CT scanning and the clinical syndrome of cough, sputum production and/or recurrent respiratory infections. 2. Diagnostic investigations Initial investigations: Consider obtaining the following minimum bundle of etiological tests in adults with a new diagnosis of bronchiectasis: CBC serum immunoglobulins (total IgG, IgA and IgM) testing for allergic bronchopulmonary aspergillosis. C Obtain three sequential daily sputum cultures for mycobacterial cultures or a single BAL in patients with radiological features of nontuberculous mycobacterial infection or clinical features such as weight loss, hemoptysis, rapid deterioration or symptoms non-responsive to standard therapy consider screening for evidence of connective tissue disease by history and physical examination, and not routinely testing for autoantibodies in patients without signs and symptoms of connective tissue disease. (Ungraded) consider testing for AAT deficiency in patients with basal emphysema or early onset airflow obstruction. (Ungraded) consider measuring antibody responses to S. pneumoniae 23 valent polysaccharide vaccine (PPV23) in order to identify individuals with specific polysaccharide antibody deficiency (ungraded) consider testing for cystic fibrosis with measurement of sweat chloride, other biomarkers of CFTR-mediated chloride ion transport and CFTR gene mutation analysis in young adults or in patients with specific clinical features of cystic fibrosis, such as upper lobe predominance of bronchiectasis on chest CT, the presence of nasal polyposis and/or chronic rhinosinusitis, recurrent pancreatitis, male primary infertility and/or malabsorption. (ungraded) https://web.pathway.md/diseases/recSF4xa2pybKp3wS 2/7 6/29/23, 3:21 AM Bronchiectasis Pathway consider testing for primary ciliary dyskinesia with nasal nitric oxide, high-speed video analysis, transmission electron microscopy, immunofluorescence and/or genetic testing in patients with several of the following features: persistent wet cough since childhood, situs anomalies, congenital cardiac defects, nasal polyposis and/or chronic rhinosinusitis, chronic middle ear disease with or without hearing loss, a history of neonatal respiratory distress or neonatal intensive care admittance in term infants. (ungraded). Testing for alpha-1 antitrypsin deficiency: avoid obtaining targeted testing for AAT deficiency in patients with bronchiectasis. D 3. Medical management Inhaled bronchodilators: Consider using long-acting bronchodilators on an individual basis for patients with significant breathlessness. C Consider avoiding routine long-acting bronchodilators for adult patients with bronchiectasis. D Inhaled corticosteroids: consider avoiding routine treatment with ICSs to adults with bronchiectasis. D Pseudomonas eradication: consider offering antibiotic eradication therapy to adults with bronchiectasis in whom P. aeruginosa is newly isolated in sputum cultures. C Long-term inhaled antibiotics: Consider initiating long-term treatment with an inhaled antibiotic for adults with bronchiectasis and chronic P. aeruginosa infection who have three or more exacerbations per year, we suggest. C Consider initiating long-term treatment with an inhaled antibiotic for adults with bronchiectasis not infected with P. aeruginosa in whom oral antibiotic prophylaxis is contraindicated, not tolerated or ineffective. C Long-term oral antibiotics: As per BTS 2020 guidelines: Consider offering the following options for long-term macrolide treatment, for a minimum of 6 months, to reduce exacerbations in patients with 3 exacerbations per year: B azithromycin 250 mg daily; consider starting azithromycin at 250 mg thrice weekly to minimize side effect risk with subsequent titration according to clinical response azithromycin 500 mg thrice weekly erythromycin ethylsuccinate 400 mg BID. C Consider offering long-term macrolides for a longer period of therapy (such as 1 year) with the aim of improving quality of life in patients with bronchiectasis. C As per ERS 2017 guidelines, consider offering long-term antibiotic treatment for adults with bronchiectasis who have three or more exacerbations per year. C https://web.pathway.md/diseases/recSF4xa2pybKp3wS 3/7 6/29/23, 3:21 AM Bronchiectasis Pathway Landmark trials: BAT In adult patients with non-cystic fibrosis bronchiectasis and 3 lower respiratory tract infections in the preceding year, azithromycin was superior to placebo with respect to exacerbation, at least 1. Altenburg J et al. JAMA. 2013 Mar 27. Mucoactive agents: consider offering long-term mucoactive treatment ( 3 months) in adult patients with bronchiectasis who have difficulty in expectorating sputum and poor quality of life, where standard airway clearance techniques have failed to control symptoms. C Statin therapy: avoid offering statins to treat bronchiectasis. D Management of acute exacerbations: consider treating acute exacerbations of bronchiectasis with 14 days of antibiotics. C 4. Nonpharmacologic interventions Airway clearance techniques: refer patients with chronic productive cough or difficulty expectorating sputum to a trained respiratory physiotherapist who can teach airway clearance techniques. B Pulmonary rehabilitation programs: refer adult patients with bronchiectasis and impaired exercise capacity to a pulmonary rehabilitation program, and advise regular exercise. Tailor interventions to the patient's symptoms, physical capability, and disease characteristics. A 5. Surgical interventions Indications for surgery: consider avoiding surgical treatments for adult patients with bronchiectasis with the exception of patients with localized disease and a high exacerbation frequency despite optimisation of all other aspects of their bronchiectasis management. D 6. Specific circumstances Patients with Sj gren's disease: Manage patients with Sj gren's disease and clinically relevant bronchiectasis similarly to primary or secondary bronchiectasis of other etiologies and consider using any of the following: mucolytic agents/expectorants nebulized saline or hypertonic saline oscillatory positive expiratory pressure postural drainage mechanical high-frequency chest wall oscillation therapies chronic macrolides in those without non-tuberculous mycobacterium colonization or infection. B https://web.pathway.md/diseases/recSF4xa2pybKp3wS 4/7 6/29/23, 3:21 AM Bronchiectasis Pathway Pediatric patients (evaluation): Consider obtaining a minimum panel of the following tests in pediatric/adolescent patients with suspected or confirmed bronchiectasis: chest CT (to diagnose bronchiectasis) sweat test pulmonary function tests CBC immunological tests (total IgG, IgA, IgM, IgE, specific antibodies to vaccine antigens) lower airway bacteriology. C Show 5 more Pediatric patients (nutrition and exercising): Consider optimizing nutrition including vitamin D status in pediatric/adolescent patients with bronchiectasis. C Consider encouraging exercising on an ongoing basis in pediatric/adolescent patients with bronchiectasis, recognizing that short periods of exercise training are unlikely to have a long- term effect. C Pediatric patients (counseling): teach and provide regular airway clearance techniques or maneuvers in pediatric/adolescent patients with bronchiectasis. B Show 3 more Pediatric patients (inhaled corticosteroids): avoid using routine corticosteroids with or without long-acting -agonists in either the short or long-term, irrespective of stability or exacerbation, in pediatric/adolescent patients with bronchiectasis. D Pediatric patients (antibiotics): administer a systemic course of an appropriate antibiotic for 14 days in pediatric/adolescent patients with bronchiectasis and an acute respiratory exacerbation. B Show 2 more Pediatric patients (mucoactive agents): Do not use recombinant human deoxyRNAase routinely in pediatric/adolescent patients with bronchiectasis. D Avoid using bromhexine, inhaled mannitol or hypertonic saline routinely in pediatric/adolescent patients with bronchiectasis. D Pediatric patients (surgical interventions): Take into account the following factors when considering surgery in pediatric/adolescent patients with bronchiectasis: age symptoms and disease burden localization of the bronchiectatic areas on chest CT underlying etiology (influencing recurrence of disease) facility where surgery is undertaken (surgical expertise and availability of pre- and postoperative care) https://web.pathway.md/diseases/recSF4xa2pybKp3wS 5/7 6/29/23, 3:21 AM Bronchiectasis Pathway optimization of the patient's clinical state. B Pediatric patients (routine immunizations): consider ensuring that pediatric/adolescent patients with bronchiectasis are fully immunized according to their national immunization programs, including pneumococcal and annual seasonal influenza vaccines if these are not part of this program. C Pediatric patients (monitoring): consider reviewing pediatric/adolescent patients with bronchiectasis every 3-6 months in outpatient clinics to monitor their general wellbeing, respiratory status, including lung function when age appropriate and to detect any complications. C Show 5 more Clinical findings Symptoms Past medical history Acute cough Allergic bronchopulmonary aspergillosis Chest pain Aspiration pneumonia Chills Cilia abnormalities Coughing up yellow or green mucus daily Cystic fibrosis Dyspnea IBD Fatigue Immunocompromising condition Fever Measles Hemoptysis Recurrent pneumonia Sputum production Repeated infections Rheumatoid arthritis Respiratory exam Tuberculosis Whooping cough Dyspnea Wheezing Integument exam Digital clubbing Studies 2022 LDPEEPPC In patients undergoing selective lung resection surgery, PEEP titration to the lowest driving pressure was superior to conventional low level of PEEP with respect to the rate of development of 4 Melbourne Group Scale variables within 3 postoperative days. Junjie Yu et al. Anaesth Crit Care Pain Med. 2022 Sep 19. 2014 EMBRACE (azithromycin) https://web.pathway.md/diseases/recSF4xa2pybKp3wS 6/7 6/29/23, 3:21 AM Bronchiectasis Pathway In adult patients with non-cystic fibrosis bronchiectasis with 1 pulmonary exacerbation requiring antibiotic treatment in the past year, azithromycin was superior to placebo with respect to a event- based exacerbations. Gladstone DJ et al. N Engl J Med. 2014 Jun 26. Show 2 more References 1. Anne B Chang, Rebecca Fortescue, Keith Grimwood et al. European Respiratory Society guidelines for the management of children and adolescents with bronchiectasis. Eur Respir J. 2021 Aug 26;58 2 2002990. Open 2. Polverino E, Goeminne PC, McDonnell MJ et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J. 2017 Sep 9;50 3 . pii: 1700629. Open 3. David Smith, Ingrid Du Rand, Charlotte Louise Addy et al. British Thoracic Society guideline for the use of long-term macrolides in adults with respiratory disease. Thorax. 2020 May;75 5 370 404. Open 4. Darcy D Marciniuk, P Hernandez, M Balter et al. Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: a Canadian Thoracic Society clinical practice guideline. Can Respir J. 2012 Mar- Apr;19 2 109 16. Open 5. Augustine S Lee, R Hal Scofield, Katherine Morland Hammitt et al. Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sj gren's. Chest. 2021 Feb;159 2 683 698. Open 6. Paul T King. The pathophysiology of bronchiectasis. Int J Chron Obstruct Pulmon Dis. 2009;4 411 9. Open 7. No authors listed. Bronchiectasis. Breathe Sheff). 2018 Mar;14 1 73 80.2018 Mar;14 1 73 80. Open 8. Maeve P Smith. Diagnosis and management of bronchiectasis. CMAJ. 2017 Jun 19;189 24 E828 E835.2017 Jun 19;189 24 E828 E835. Open 9. James D Finklea, Gul Khan, Sheree Thomas et al. Predictors of mortality in hospitalized patients with acute exacerbation of bronchiectasis. Respir Med. 2010 Jun;104 6 816 21. Open 10. Hill AT, Sullivan AL, Chalmers JD et al. British Thoracic Society Guideline for bronchiectasis in adults. Thorax. 2019 Jan;74 Suppl 1 1 69. Open 11. Maeve P Smith. Diagnosis and management of bronchiectasis. CMAJ. 2017 Jun 19;189 24 E828 E835. Open 12. No authors listed. Bronchiectasis. Breathe Sheff). 2018 Mar;14 1 73 80. Open 13. Paul T King. The pathophysiology of bronchiectasis. Int J Chron Obstruct Pulmon Dis. 2009;4 411 9.2009;4 411 9. Open 14. James D Finklea, Gul Khan, Sheree Thomas et al. Predictors of mortality in hospitalized patients with acute exacerbation of bronchiectasis. Respir Med. 2010 Jun;104 6 816 21.2010 Jun;104 6 816 21. Open https://web.pathway.md/diseases/recSF4xa2pybKp3wS 7/7
Guideline sources The following summarized guidelines for the management of bronchiolitis obliterans syndrome (BOS) are prepared by our editorial team based on guidelines from the British Thoracic Society (BTS 2020) and the European Respiratory Society (ERS/ATS/ISHLT 2014). 1 2 3 3 4 5 Definition BOS is a form of chronic lung allograft dysfunction characterized by progressive airflow obstruction in the absence of acute rejection, infection, or other coexistent conditions. 3 Epidemiology BOS is caused by immune-mediated injury (alloimmune T-cell reactivity, humoral immunity, autoimmunity, pulmonary innate immunity) and environmental insults. 3 Disease course BOS manifests as progressive dyspnea and cough, with imaging features of constrictive bronchiolitis, airway distortion, and subepithelial fibrosis. 4 Prognosis and risk of recurrence Severe BOS is associated with a mortality of 66.6%. 5 https://web.pathway.md/diseases/recIgNOy9NrPRdTwU 1/3 6/29/23, 3:21 AM Bronchiolitis obliterans syndrome Pathway Guidelines 1. Medical management High-dose corticosteroids: consider avoiding long-term, high-dose corticosteroids for lung transplant recipients who develop a decline in FEV in 1 second consistent with the onset of BOS. D Tacrolimus: consider switching from cyclosporine to tacrolimus in lung transplant recipients who develop BOS while receiving chronic immunosuppression with a regimen that includes cyclosporine. C Azithromycin: As per ERS 2020 guidelines, consider offering low-dose azithromycin (250 mg alternate days for a trial period of 3 months) for the treatment of bronchiolitis obliterans in lung transplant recipients. C As per ISHLT 2014 guidelines, consider a trial of azithromycin for lung transplant recipients who develop a decline in FEV in 1 second consistent with the onset of BOS. C 2. Surgical interventions Fundoplication: consider referral to an experienced surgeon to evaluate for potential fundoplication of the gastro-oesophageal junction in lung transplant recipients who develop a decline in FEV in 1 second consistent with the onset of BOS and have confirmed gastro- oesophageal reflux. C Re-transplantation: refer lung transplant recipients with refractory, end-stage BOS to a transplant surgeon to be evaluated for re-transplantation. B 3. Preventative measures Primary prevention: As per ERS 2020 guidelines, consider offering low-dose, long-term azithromycin (250 mg thrice weekly) for the prevention of BOS after lung transplantation. C As per ISHLT 2014 guidelines, consider augmented immunosuppression with a course of systemic steroids to prevent the development of BOS in lung transplant recipients who have non-minimal acute cellular rejection (grade 2) or lymphocytic bronchitis on transbronchial lung biopsy specimens. C Clinical findings Symptoms Past medical history https://web.pathway.md/diseases/recIgNOy9NrPRdTwU 2/3 6/29/23, 3:21 AM Bronchiolitis obliterans syndrome Pathway Asymptomatic Chronic disease Dry cough Inflammation Dyspnea No asthma Fatigue Imaging findings Flu-like symptoms Infection Lung consolidation No cold Symptoms flare up with exercising Respiratory exam Dyspnea Wheezing References 1. Meyer KC, Raghu G, Verleden GM et al. An international ISHLT/ATS/ERS clinical practice guideline: diagnosis and management of bronchiolitis obliterans syndrome. Eur Respir J. 2014 Dec;44 6 1479 503. Open 2. David Smith, Ingrid Du Rand, Charlotte Louise Addy et al. British Thoracic Society guideline for the use of long-term macrolides in adults with respiratory disease. Thorax. 2020 May;75 5 370 404. Open 3. Jamie L Todd, Scott M Palmer. Bronchiolitis obliterans syndrome: the final frontier for lung transplantation. Chest. 2011 Aug;140 2 502 508. Open 4. Patrick R Aguilar, Andrew P Michelson, Warren Isakow. Obliterative Bronchiolitis. Transplantation. 2016 Feb;100 2 272 83.2016 Feb;100 2 272 83. Open 5. M T Parada, A Alba, C Sep lveda. Bronchiolitis obliterans syndrome development in lung transplantation patients. Transplant Proc. Jan-Feb 2010;42 1 331 2. Open 6. Patrick R Aguilar, Andrew P Michelson, Warren Isakow. Obliterative Bronchiolitis. Transplantation. 2016 Feb;100 2 272 83. Open 7. Jamie L Todd, Scott M Palmer. Bronchiolitis obliterans syndrome: the final frontier for lung transplantation. Chest. 2011 Aug;140 2 502 508.2011 Aug;140 2 502 508. Open 8. M T Parada, A Alba, C Sepulveda. Bronchiolitis obliterans syndrome development in lung transplantation patients. Transplant Proc. Jan-Feb 2010;42 1 331 2.Jan-Feb 2010;42 1 331 2. Open 9. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open https://web.pathway.md/diseases/recIgNOy9NrPRdTwU 3/3
Guideline sources The following summarized guidelines for the evaluation and management of bronchiolitis are prepared by our editorial team based on guidelines from the French Group for Pediatric Intensive Care and Emergencies (GFRUP 2023), the National Advisory Committee on Immunization (NACI 2022), the Canadian Paediatric Society (CPS 2021), the American Academy of Pediatrics (AAP 2020; 2018; 2014), the Paediatric Research in Emergency Departments International Collaborative (PREDICT 2019), the American College of Chest Physicians (ACCP 2018), the The Thoracic Society of Australia and New Zealand (TSANZ 2018), the Royal Australasian College of Physicians (RACP 2017), and the Society of Hospital Medicine (SHP 2013). 1 2 3 4 5 6 7 8 9 10 11 12 Calculator Calculator https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 1/13 6/29/23, 3:21 AM Bronchiolitis Pathway Modified Wood's Clinical Asthm Wang Respiratory Score WRS Guidelines 1. Screening and diagnosis Diagnosis: As per CPS 2021 guidelines, diagnose bronchiolitis clinically based on history and physical examination. E As per PREDICT 2019 guidelines, consider diagnosing bronchiolitis in infant patients with an upper respiratory tract infection followed by the onset of respiratory distress with fever and 1 of the following: cough tachypnea respiratory retractions diffuse crackles or wheeze on auscultation. C As per AAP 2014 guidelines, diagnose bronchiolitis and assess disease severity on the basis of history and physical examination. B 2. Classification and risk stratification Risk assessment: As per PREDICT 2019 guidelines, regard the following as risk factors for more serious illness: gestational age < 37 weeks chronological age at presentation < 10 weeks indigenous ethnicity breastfeeding for < 2 months exposure to cigarette smoke failure to thrive chronic pulmonary, heart, or neurological disease. B As per AAP 2014 guidelines, assess the following risk factors for severe disease when making decisions about the evaluation and management of patients with bronchiolitis: age < 12 weeks history of prematurity immunodeficiency underlying cardiopulmonary disease. B https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 2/13 6/29/23, 3:21 AM Bronchiolitis Pathway 3. Diagnostic investigations Clinical assessment: consider using clinical scores (such as the modified Wood's Clinical Asthma Score, the Wang score, and the Critical Bronchiolitis Score) for the initial assessment and follow- up. E Chest X-ray: As per GFRUP 2023 guidelines, avoid obtaining routine CXRs for the diagnosis of bronchiolitis. Obtain CXRs in patients with clinical signs of ventilatory complications or for the search for differential diagnosis. D As per CPS 2021 guidelines, do not obtain CXRs in typical cases of bronchiolitis. D As per PREDICT 2019 guidelines, do not obtain routine CXRs in infant patients with simple bronchiolitis. D As per RACP 2017 guidelines, do not obtain CXRs routinely for diagnosis of bronchiolitis in pediatric patients. D As per AAP 2014 guidelines, do not obtain CXRs routnely in patients with bronchiolitis diagnosed on the basis of history and physical examination. D As per SHP 2013 guidelines, do not obtain CXRs in pediatric patients with uncomplicated bronchiolitis. D Chest ultrasound: consider obtaining a lung ultrasound as an alternative to a CXR. E Laboratory tests: As per GFRUP 2023 guidelines, avoid obtaining virological tests in patients with bronchiolitis. D Show 2 more As per CPS 2021 guidelines, do not obtain blood tests and viral/bacterial cultures in typical cases of bronchiolitis. D As per PREDICT 2019 guidelines, do not obtain blood tests in infant patients presenting to the hospital and hospitalized with bronchiolitis. Do not obtain routine bacteriological testing of blood and urine. D Show 2 more As per AAP 2014 guidelines, do not obtain routne laboratory tests in patients with bronchiolitis diagnosed on the basis of history and physical examination. D 4. Respiratory support Oxygen monitoring: As per GFRUP 2023 guidelines, consider monitoring the trend of respiratory parameters and/or clinical scores to assess disease evolution. Avoid obtaining systematic monitoring of partial pressure of CO (and transcutaneous partial pressure of CO ). E As per CPS 2021 guidelines, obtain oxygen saturation monitoring in 1-24 months old hospitalized patients with bronchiolitis. Consider obtaining continuous saturation monitoring in https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 3/13 6/29/23, 3:21 AM Bronchiolitis Pathway high-risk patients in the acute phase of illness and intermittent monitoring or spot checks in lower-risk patients and clinically improving patients. E As per PREDICT 2019 guidelines, do not obtain routine continuous pulse oximetry in non- hypoxic (oxygen saturation 92%) infant patients not receiving oxygen or stable patients receiving oxygen. D As per AAP 2014 guidelines, avoid obtaining continuous pulse oximetry in infant and pediatric patients with bronchiolitis. D As per SHP 2013 guidelines, do not obtain continuous pulse oximetry routinely in pediatric patients with acute respiratory illness unless on supplemental oxygen. D Supplemental oxygen: As per PREDICT 2019 guidelines: Consider administering supplemental oxygen in the treatment of hypoxic (oxygen saturations < 92%) infant patients with bronchiolitis. C Administer oxygen supplementation in patients with uncomplicated bronchiolitis if the oxygen saturation level is sustained at a level < 92%. Discontinue oxygen therapy at oxygen saturation levels of 92%. B As per AAP 2014 guidelines, avoid administering supplemental oxygen if the oxyHgb saturation is > 90%. D Noninvasive ventilation: As per GFRUP 2023 guidelines, consider using a noninvasive ventilatory support protocol. E Consider initiating noninvasive ventilatory support over invasive ventilation as first-line treatment to reduce the work of breathing and improve clinical respiratory parameters. C Show 6 more Landmark trials: PARIS-2 In children aged 1-4 years requiring hospital admission for acute hypoxemic respiratory failure, high-flow oxygen therapy was inferior to standard oxygen therapy with respect to median length of hospital stay. Donna Franklin et al. JAMA. 2023 Jan 17. As per PREDICT 2019 guidelines: Consider administering high-flow nasal cannula oxygen in infant patients with bronchiolitis with hypoxia (oxygen saturation < 92%) in an inpatient setting. C Consider administering nasal CPAP therapy in the management of infant patients with bronchiolitis. C Invasive ventilation: insufficient evidence regarding the choice of invasive ventilation mode in infant patients with bronchiolitis. I https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 4/13 6/29/23, 3:21 AM Bronchiolitis Pathway 5. Medical management Setting of care (hospital admission): decide on hospital admission based on clinical judgment, factoring in the risk for progression to severe disease, respiratory status, ability to maintain adequate hydration and the family's ability to cope at home. E Setting of care (PICU admission): Consider using the following severity factors when deciding on pediatric ICU admission: presence of apneas hypercapnic acidosis with venous partial pressure of CO > 60 mmHg and/or pH < 7.30, if blood gas testing is available altered alertness and/or hypotonia hypoxemia with SpO < 92% under standard oxygen therapy (nasal cannulas at low-flow up to 2 L/min) significant increase in clinical work of breathing or respiratory fatigue. E Show 2 more Supportive care: As per GFRUP 2023 guidelines, consider preferring gastric enteral nutrition (or oral nutrition when possible) over IV hydration, regardless of the type of ventilatory support, in infant (< 12 months old) patients with bronchiolitis. Avoid thickening oral or enteral nutrition. E Show 6 more As per CPS 2021 guidelines: Initiate supportive care including hydration, minimal handling, gentle nasal suctioning and oxygen therapy in 1-24 months old patients with bronchiolitis. E Administer an isotonic solution (0.9% NaCl/5% dextrose) and monitor serum sodium routinely when using IV fluids for hydration in 1-24 months old patients with bronchiolitis. E As per PREDICT 2019 guidelines, initiate supplemental hydration with isotonic fluids, either via a nasogastric or IV route, B in infant patients unable to maintain hydration PO. E As per AAP 2014 guidelines, administer nasogastric or IV fluids in infant patients with bronchiolitis unable to maintain oral hydration. B Nebulized hypertonic saline: As per GFRUP 2023 guidelines, avoid using nebulized hypertonic saline routinely in infant patients with bronchiolitis. D As per CPS 2021 guidelines: Insufficient evidence to support the use of hypertonic 3% saline in an ambulatory setting. I Consider administering hypertonic 3% saline in 1-24 months old patients hospitalized > 3 days. E As per PREDICT 2019 guidelines, do not use nebulized hypertonic saline in infant patients presenting to the hospital or hospitalized with bronchiolitis. D As per AAP 2014 guidelines: https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 5/13 6/29/23, 3:21 AM Bronchiolitis Pathway Do not use nebulized hypertonic saline in infant patients with bronchiolitis in the emergency department. D Consider administering nebulized hypertonic saline in hospitalized infant and pediatric patients with bronchiolitis. C Nebulized epinephrine: As per CPS 2021 guidelines: Do not use routine epinephrine in 1-24 months old patients with bronchiolitis. D Continue ongoing epinephrine in 1-24 months old patients with bronchiolitis if a trial of epinephrine inhalation was attempted in the emergency department and there are clear signs of clinical improvement. E As per PREDICT 2019 guidelines, do not use epinephrine in infant patients presenting to the hospital or hospitalized with bronchiolitis. D As per AAP 2014 guidelines, do not use epinephrine in infant and pediatric with bronchiolitis. D Bronchodilators: As per GFRUP 2023 guidelines, avoid using inhaled -2 agonists and/or inhaled epinephrine routinely in infant patients with bornchiolitis. Consider obtaining a therapeutic test to avoid intubation in selected cases. D Show 2 more As per CPS 2021 guidelines, do not use routine salbutamol in 1-24 months old patients with bronchiolitis. D As per PREDICT 2019 guidelines, do not use -2 agonists in 12 months old patients presenting to the hospital or hospitalized with bronchiolitis, D including those with a personal or family history of atopy D As per RACP 2017 guidelines, do not use salbutamol routinely for the treatment of pediatric patients with bronchiolitis. D As per AAP 2014 guidelines, do not use albuterol or salbutamol in infant and pediatric patients with bronchiolitis. D As per SHP 2013 guidelines, do not use bronchodilators routinely in pediatric patients with bronchiolitis. D Corticosteroids: As per GFRUP 2023 guidelines, avoid using corticosteroids, whether inhaled or systemic, in infant patients with brinchiolitis. D As per CPS 2021 guidelines, do not use routine corticosteroids in 1-24 months old patients with bronchiolitis. D As per PREDICT 2019 guidelines, do not use systemic or local corticosteroids in infant patients presenting to the hospital or hospitalized with bronchiolitis. D As per RACP 2017 guidelines, do not use systemic corticosteroids routinely for the treatment of pediatric patients with bronchiolitis. D https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 6/13 6/29/23, 3:21 AM Bronchiolitis Pathway As per AAP 2014 guidelines, do not use systemic corticosteroids in infant patients with bronchiolitis in any setting. D As per SHP 2013 guidelines, do not use systemic corticosteroids in < 2 years old patients with an uncomplicated lower respiratory tract infection. D Antibiotics: As per GFRUP 2023 guidelines, avoid using antibiotics routinely in infant patients with bronchiolitis. Reserve antibiotics for suspected pulmonary superinfection or bacterial co- infection. D As per CPS 2021 guidelines, do not use antibiotics in 1-24 months old patients with bronchiolitis unless there is suspicion of an underlying bacterial infection. D As per PREDICT 2019 guidelines, do not use antibiotics for the treatment of infant patients with bronchiolitis. D Do not use azithromycin in hospitalized infant patients with bronchiolitis, D including those at risk of developing bronchiectasis. D As per AAP 2018 guidelines, do not use antibiotics in pediatric patients with viral respiratory illnesses including bronchiolitis. D As per AAP 2014 guidelines, do not use antibiotics in infant and pediatric patients with bronchiolitis unless there is a strongly suspected or confirmed concomitant bacterial infection. D Therapies with no evidence for benefit: Avoid using helium, ribavirin, deoxyribonuclease, anti-leukotrienes, magnesium sulfate, and inhaled nitric oxide in infant patients with bronchiolitis. D Avoid using caffeine routinely in infant patients presenting with apnea. D 6. Nonpharmacologic interventions Chest physiotherapy: As per GFRUP 2023 guidelines, avoid offering routine respiratory physiotherapy in infant patients with bronchiolitis. D As per CPS 2021 guidelines, do not offer chest physiotherapy in 1-24 months old patients with bronchiolitis. D As per AAP 2020 guidelines, do not offer airway clearance therapy routinely in pediatric patients with bronchiolitis. D As per PREDICT 2019 guidelines, do not offer chest physiotherapy routinely in infant patients with bronchiolitis. D As per AAP 2014 guidelines, do not offer chest physiotherapy in infant and pediatric patients with bronchiolitis. D Nasal suctioning: https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 7/13 6/29/23, 3:21 AM Bronchiolitis Pathway Do not perform nasal suctioning routinely in infant patients with bronchiolitis. Consider performing superficial nasal suctioning to assist feeding in patients with moderate disease. D Do not perform deep nasal suctioning for the management of bronchiolitis. D Do not use nasal saline drops routinely in patients with bronchiolitis. Consider administering a trial of intermittent saline drops at time of feeding. D 7. Patient education General counseling: ask about the exposure of the infant or child to tobacco smoke when assessing infants and children for bronchiolitis. B Counsel caregivers about exposing the infant or child to environmental tobacco smoke and smoking cessation when assessing infants and children for bronchiolitis. B Show 2 more 8. Preventative measures Palivizumab prophylaxis, prematurity: As per NACI 2022 guidelines, offer palivizumab prophylaxis in infants born at < 30 weeks 0 days gestation and aged < 6 months at the onset of or during the RSV season. B Show 3 more As per AAP 2014 guidelines, offer palivizumab prophylaxis in the first year of life in infants born before 29 weeks 0 days of gestation. E Show 2 more Palivizumab prophylaxis (multiple birth): do not offer palivizumab prophylaxis in infants or siblings of multiple births not otherwise qualifying for prophylaxis. D Palivizumab prophylaxis, congenital heart disease: As per NACI 2022 guidelines, offer palivizumab prophylaxis in infants with hemodynamically significant congenital heart disease (as assessed by a pediatric cardiologist) aged > 1 year at the onset of the RSV season. A Show 3 more As per AAP 2014 guidelines, consider offering palivizumab prophylaxis in the first year of life in certain infants with hemodynamically significant heart disease. E Palivizumab prophylaxis, immunodeficiency: As per NACI 2022 guidelines, consider offering palivizumab prophylaxis in < 24 months old patients with severe immunocompromise. B As per AAP 2014 guidelines, consider offering palivizumab prophylaxis in < 24 months old patients expected to be profoundly immunocompromised during the RSV season. E Palivizumab prophylaxis, cystic fibrosis: As per NACI 2022 guidelines: https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 8/13 6/29/23, 3:21 AM Bronchiolitis Pathway Do not offer palivizumab prophylaxis routinely in < 24 months old patients with cystic fibrosis. D Consider offering palivizumab prophylaxis in < 24 months old patients with cystic fibrosis with severe chronic lung disease (defined as requiring ongoing supplemental oxygen in the 6 months before the onset of or during the RSV season). B As per AAP 2014 guidelines, insufficient evidence to support the use of palivizumab prophylaxis in pediatric patients with cystic fibrosis. I Palivizumab prophylaxis, Down syndrome: As per NACI 2022 guidelines, do not offer palivizumab prophylaxis routinely in < 24 months old patients with Down's syndrome. D Offer palivizumab prophylaxis in pediatric patients with Down's syndrome qualifying for prophylaxis because of hemodynamically significant congenital heart disease, chronic lung disease, prematurity, or immunodeficiency. D As per AAP 2014 guidelines, insufficient evidence to support the use of palivizumab prophylaxis in pediatric patients with Down syndrome. I Palivizumab prophylaxis, other comorbidities: As per NACI 2022 guidelines, consider offering palivizumab prophylaxis in < 24 months old patients with severe chronic lung disease of other etiology (such as congenital cystic lung disease, chronic ILD, congenital lung malformations, congenital airway abnormalities, or neuromuscular conditions affecting the ability to clear airway secretions) or requiring home respiratory support (such as supplemental oxygen, mechanical ventilation, CPAP, tracheostomy) if requiring ongoing supplemental oxygen or assisted ventilation in the 6 months before the onset of or during the RSV season. B As per AAP 2014 guidelines, consider offering palivizumab prophylaxis in the first year of life in patients with a pulmonary abnormality or neuromuscular disease impairing the ability to clear secretions from the upper airways. E Palivizumab prophylaxis (prevention of wheezing or asthma): do not offer palivizumab prophylaxis for the prevention of recurrent wheezing or asthma in the absence of other indications. D Palivizumab prophylaxis, prevention of healthcare-associated RSV infection: As per NACI 2022 guidelines, do not offer routine palivizumab prophylaxis to control or prevent RSV infections in neonatal intensive care or other hospital units. D Consider offering palivizumab prophylaxis when all other measures to control an RSV outbreak in a neonatal ICU have failed. D As per AAP 2014 guidelines, do not use palivizumab prophylaxis for the prevention of healthcare-associated RSV disease. D Palivizumab prophylaxis (remote communities): Offer palivizumab prophylaxis in infants aged < 6 months born < 36 weeks of gestation and living in remote northern Inuit communities who would require air transport for hospitalization. B Do not offer palivizumab prophylaxis routinely in healthy full-term infants living in remote northern Inuit communities. A Consider offering palivizumab prophylaxis in healthy full-term https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 9/13 6/29/23, 3:21 AM Bronchiolitis Pathway infants aged < 6 months at the onset of or during the RSV season living in remote northern Inuit communities with documented very high RSV hospitalization rates for term infants. B Consider offering palivizumab prophylaxis in infants aged < 6 months born < 36 weeks of gestation and living in other remote communities with documented high rates of hospitalization for RSV who would require air transport for hospitalization. B Palivizumab prophylaxis, regimens: As per NACI 2022 guidelines, administer palivizumab prophylaxis at a dose of 15 mg/kg by IM injection. Administer the first dose at the onset of the current RSV season, as determined by local laboratory data or pediatric hospitalization data. Consider pre-determining the start date based on dates of previous local RSV seasons if local data are not available in a timely fashion. Administer the second dose 21-28 days after the first dose, and at 28-35-day intervals for further doses. B Show 4 more As per AAP 2014 guidelines, consider administering a maximum of 5 monthly doses of palivizumab (15 mg/kg per dose) during the RSV season in infants qualifying for prophylaxis in the first year of life. Recognize that qualifying infants born during the RSV season may require fewer doses. E Show 2 more Hand hygiene: As per PREDICT 2019 guidelines, ensure proper hand hygiene to reduce hospital-acquired infections. B As per AAP 2014 guidelines: Ensure that all people disinfect their hands before and after direct contact with patients, after contact with inanimate objects in the direct vicinity of the patient, and after removing gloves. B Ensure that all people use alcohol-based rubs for hand decontamination when caring for children with bronchiolitis. Ensure that individuals wash their hands with soap and water if alcohol-based rubs are not available. B 9. Follow-up and surveillance Discharge from hospital: As per PREDICT 2019 guidelines: Take into account oxygen saturations, adequacy of feeding, age (< 8 weeks), and lack of social support at the time of discharge as a risk for representation. E Consider discharging infant patients at low risk for severe bronchiolitis, after a period of observation, on home oxygen with a clear return-to-hospital advice. C As per TSANZ 2018 guidelines, do not delay discharge from an inpatient admission based on oxygen saturations alone if saturations are 90% in pediatric patients with bronchiolitis without other comorbidities. D https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 10/13 6/29/23, 3:21 AM Bronchiolitis Pathway Management of chronic cough: avoid using asthma medications or inhaled osmotic agents in pediatric patients with chronic cough (> 4 weeks) after acute viral bronchiolitis. Clinical findings Patient demographics Symptoms Age < 2 Cough Fever Past medical history Poor feeding Rhinorrhea Acute otitis media UTI Vital signs Ocular exam Tachycardia Tachypnea Conjunctivitis O2 saturation Microbiological findings Respiratory exam RSV RNA Apnea Crackles Grunting Intercostal retractions Nasal flaring Wheezing Imaging findings Atelectasis Patchy pulmonary infiltrates Pulmonary hyperinflation Reticulonodular pulmonary infiltrates Studies 2023 AReSVi-006 In adults 60 years of age, RSVPreF3 OA vaccine was superior to placebo vaccine with respect to RSV-related lower respiratory tract disease. Alberto Papi et al. N Engl J Med. 2023 Feb 16. 2023 PARIS-2 https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 11/13 6/29/23, 3:21 AM Bronchiolitis Pathway In children aged 1-4 years requiring hospital admission for acute hypoxemic respiratory failure, high- flow oxygen therapy was inferior to standard oxygen therapy with respect to median length of hospital stay. Donna Franklin et al. JAMA. 2023 Jan 17. Show 2 more References 1. Sharon O'Brien, Meredith L Borland, Elizabeth Cotterell et al. Australasian bronchiolitis guideline. J Paediatr Child Health. 2019 Jan;55 1 42 53. Open 2. Christophe Mil si, Florent Baudin, Philippe Durand et al. Clinical practice guidelines: management of severe bronchiolitis in infants under 12 months old admitted to a pediatric critical care unit. Intensive Care Med. 2023 Jan;49 1 5 25. Open 3. National Advisory Committee on Immunization. An Advisory Committee Statement ACS National Advisory Committee on Immunization NACI Recommended use of palivizumab to reduce complications of respiratory syncytial virus infection in infants. NACI. 2022 Jun. Open 4. Ralston SL, Lieberthal AS, Meissner HC et al. Clinical practice guideline: the diagnosis, management, and prevention of bronchiolitis. Pediatrics. 2014 Nov;134 5):e1474 502. Open 5. American Academy of Pediatrics Committee on Infectious Diseases, American Academy of Pediatrics Bronchiolitis Guidelines Committee. Updated guidance for palivizumab prophylaxis among infants and young children at increased risk of hospitalization for respiratory syncytial virus infection. Pediatrics. 2014 Aug;134 2 415 20. Open 6. Jeremy N Friedman, Michael J Rieder, Jennifer M Walton et al. Bronchiolitis: Recommendations for diagnosis, monitoring and management of children one to 24 months of age. CPS. 2021 Nov 30. Open 7. SHP. Choosing Wisely SHP recommendations. SHP. 2013. Open 8. American Academy of Pediatrics. Choosing Wisely AAP recommendations. Choosing Wisely. 2020. Open 9. RACP. Choosing Wisely RACP recommendations. RACP. 2017. Open 10. Anne B Chang, John J Oppenheimer, Bruce K Rubin et al. Chronic Cough Related to Acute Viral Bronchiolitis in Children: CHEST Expert Panel Report. Chest. 2018 Aug;154 2 378 382. Open 11. TSANZ. Choosing Wisely TSANZ recommendations. TSANZ. 2018. Open 12. American Academy of Pediatrics. Choosing Wisely AAP recommendations. Choosing Wisely. 2018. Open 13. Society of Hospital Medicine. Choosing Wisely SHM recommendations. Choosing Wisely. 2013. Open 14. Donna Franklin, Franz E Babl, Luregn J Schlapbach et al. A Randomized Trial of High-Flow Oxygen Therapy in Infants with Bronchiolitis. N Engl J Med. 2018 Mar 22;378 12 1121 1131. Open 15. Avraham Beigelman, M.D., Mythili Srinivasan et al. Azithromycin to Prevent Recurrent Wheeze Following Severe Respiratory Syncytial Virus Bronchiolitis. NEJM Evid. 2022;1 4 . Open https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 12/13 6/29/23, 3:21 AM Bronchiolitis Pathway 16. Federico Martin n-Torres, Antonio Rodr guez-N ez, Jose Mar a Martin n-S nchez. Heliox therapy in infants with acute bronchiolitis. Pediatrics. 2002 Jan;109 1 68 73. Open 17. E E Wang, R A Milner, L Navas et al. Observer agreement for respiratory signs and oximetry in infants hospitalized with lower respiratory infections. Am Rev Respir Dis. 1992 Jan;145 1 106 9. Open https://web.pathway.md/diseases/recSVEmgKrGNI4oBu 13/13
Guideline sources The following summarized guidelines for the evaluation and management of bronchopulmonary neuroendocrine tumors are prepared by our editorial team based on guidelines from the American Association for Thoracic Surgery (AATS/ESTS 2022), the European Society of Medical Oncology (ESMO 2021), the European Neuroendocrine Tumor Society (ENETS 2015), the American College of Chest Physicians (ACCP 2013), and the Endocrine Society (ES 2012). 1 2 3 4 5 Guidelines 1. Screening and diagnosis Indications for screening: Obtain chest CT or MRI every 1-2 years to detect bronchopulmonary carcinoid tumors in patients with MEN1. B Do not obtain biochemical evaluation with urinary 5-HIAA or chromogranin A. D Diagnosis: Recognize that: https://web.pathway.md/diseases/recNaF7ifeeiz8JWz 1/6 6/29/23, 3:21 AM Bronchopulmonary neuroendocrine tumors Pathway pulmonary carcinoids as a whole are well-differentiated NETs as opposed to poorly differentiated small-cell lung carcinoma and large-cell neuroendocrine carcinoma and include low-grade malignant tumors (typical carcinoids) and intermediate-grade malignant tumors (atypical carcinoids) typical carcinoid is closest to the G1 gastroenteropancreatic NETs, and atypical carcinoid is closest to the G2 gastroenteropancreatic NETs small cell lung carcinoma and large-cell neuroendocrine carcinoma generally correspond to the neuroendocrine carcinoma category of the gastrointestinal tract according to the current WHO classification diagnostic criteria rely primarily on histology. B 2. Classification and risk stratification Prognostic assessment: use the WHO and pTNM classifications for prognostic classification. B Show 2 more 3. Diagnostic investigations Diagnostic imaging: obtain contrast-enhanced chest CT for the diagnosis of bronchopulmonary NETs. B Consider obtaining HRCT if contrast administration is contraindicated. B Show 3 more Laboratory tests: Obtain liver and renal function tests, calcium, glucose, and plasma chromogranin A measurements in patients with bronchopulmonary NETs. B Obtain laboratory testing for paraneoplastic syndrome based on clinical symptoms and features, including 24-hour urine 5-HIAA, ACTH, and GH-releasing hormone as appropriate. B Evaluation for MEN1: Recognize that pulmonary carcinoids may be associated with MEN1 in < 5% of patients. Elicit family history and obtain a clinical assessment and minimal laboratory screening for the evaluation of MEN1. B Obtain genetic testing for MEN1 in patients with a family history suggestive of a MEN1 or a second MEN1 feature. B Imaging for staging: As per ESMO 2021 guidelines, obtain contrast-enhanced cross-sectional conventional imaging, including liver late arterial phase and positron emission tomography-CT with 68-galium-labeled somatostatin analogs, for staging. B As per ENETS 2015 guidelines, obtain chest and abdomen CT for preoperative staging. B Show 2 more https://web.pathway.md/diseases/recNaF7ifeeiz8JWz 2/6 6/29/23, 3:21 AM Bronchopulmonary neuroendocrine tumors Pathway 4. Diagnostic procedures Diagnostic bronchoscopy: Consider performing a bronchoscopy, preferably flexible bronchoscopy, for preoperative staging and assessment of central airway tumors. B Consider preferring rigid bronchoscopy for obtaining biopsy specimens in patients at high risk for bleeding. C Insufficient evidence regarding the added value of new bronchoscopic techniques to increase the sensitivity of detection of primary tumors or recurrence. I Biopsy and histopathology: obtain pathological diagnosis of any pulmonary NET. B Show 8 more 5. Medical management Management of local/locoregional disease, watchful waiting: As per ESMO 2021 guidelines, consider offering initial imaging follow-up without treatment to determine the tumor growth rate in patients with cT1N0 lung carcinoid within the setting of diffuse pulmonary neuroendocrine cell hyperplasia or MEN1, multifocal primaries, or comorbid conditions. C As per ENETS 2015 guidelines, consider offering watchful waiting in a subgroup of asymptomatic patients mainly with typical carcinoids or atypical carcinoids of low proliferative index. C Management of local/locoregional disease, surgery and locoregional procedures: As per ESMO 2021 guidelines: Perform anatomic pulmonary resection (segmentectomy, lobectomy, or bilobectomy) or bronchoplastic procedures (sleeve resections) along with lymph node dissection in patients with localized lung carcinoids. Avoid performing pneumonectomy, where possible. B Consider performing sublobar surgical resection (including wedge resection) or offering nonsurgical locoregional therapeutic procedures as an alternative to anatomic pulmonary resection to preserve lung function in patients with cT1N0 lung carcinoid with functioning syndromes or morphological progression. C As per ENETS 2015 guidelines, offer surgery, transarterial embolization, or radiofrequency as locoregional treatment options for primary tumor in patients with slow progressive pulmonary carcinoids. B Show 5 more As per ES 2012 guidelines, perform curative surgery, where possible, as the treatment of choice in patients with bronchial carcinoid tumors. A Management of local/locoregional disease, systemic therapy: As per ESMO 2021 guidelines: Consider offering somatostatin analogs as an alternative to anatomic pulmonary resection to preserve lung function in patients with cT1N0 lung carcinoid with functioning syndromes or https://web.pathway.md/diseases/recNaF7ifeeiz8JWz 3/6 6/29/23, 3:21 AM Bronchopulmonary neuroendocrine tumors Pathway morphological progression. C Do not offer routine adjuvant therapy in patients with lung carcinoids. Consider offering cytotoxic chemotherapy (dacarbazine/temozolomide- or oxaliplatin-based chemotherapy), with or without radiotherapy, in selected fit patients with a particularly high risk of relapse (atypical carcinoid N2) after multidisciplinary discussion. D As per ENETS 2015 guidelines: Offer peptide receptor radiotargeted therapy in patients with tumors demonstrating strong expression of somatostatin receptors. B Insufficient evidence to recommend adjuvant therapy after complete resection of pulmonary carcinoids. Consider offering adjuvant therapy in patients with atypical lung carcinoids with a high proliferative index. I Management of advanced/metastatic disease (watchful waiting): consider offering watchful follow-up in asymptomatic patients with slowly radiologically progressing lung carcinoids. C Management of advanced/metastatic disease, systemic therapy: As per ESMO 2021 guidelines, discuss systemic options for patients with advanced carcinoids with bulky or progressing tumors in an interdisciplinary setting. Show 7 more As per ENETS 2015 guidelines, recognize that cytotoxic treatment has been the standard for aggressive metastatic pulmonary carcinoids, although the available chemotherapy regimens demonstrate a limited effect. B Consider offering temozolomide monotherapy. B Offer a combination of cisplatinum and etoposide in patients with high-proliferating pulmonary carcinoids. B Show 2 more As per ES 2012 guidelines, consider offering radiotherapy and chemotherapy in patients with advanced diseases where curative surgery is impossible. C Management of advanced/metastatic disease, surgery and locoregional procedures: As per ESMO 2021 guidelines: Consider offering palliative surgery or locoregional procedures (radiofrequency ablation, cryoablation, endobronchial treatment) of the primary tumor in patients with advanced disease at risk of local events or refractory carcinoid syndrome. C Offer locoregional therapies, including surgery (with or without somatostatin analogs), as first- line therapy to decrease the tumor burden, control hormonal secretions, and prevent local complications in patients with advanced, slowly progressing lung carcinoids. B As per ENETS 2015 guidelines: Offer surgery, transarterial embolization, or radiofrequency as locoregional treatment options for metastases in patients with slow progressive pulmonary carcinoids. B Perform resection of liver metastases whenever possible in patients planned for curative-intent treatment and in syndromic patients when > 90% of tumor burden can be removed. View the following as the minimal requirements for curative intent: resectable typical carcinoid and low-grade atypical carcinoid https://web.pathway.md/diseases/recNaF7ifeeiz8JWz 4/6 6/29/23, 3:21 AM Bronchopulmonary neuroendocrine tumors Pathway < 5% mortality absence of right HF absence of unresectable lymph node and extra-abdominal metastases absence of unresectable peritoneal carcinomatosis. B Management of functioning syndromes: As per ESMO 2021 guidelines, consider controlling functioning syndromes before any invasive therapeutic intervention in patients with local/locoregional disease. B Show 4 more As per ENETS 2015 guidelines, offer somatostatin analogs as first-line therapy in patients with carcinoid syndrome and acromegaly. B Show 2 more 6. Perioperative care Preoperative cardiorespiratory evaluation: Obtain functional respiratory tests to assess the surgical risk and the association with chronic obstructive airway disease and to screen for bronchostenosis. B Obtain echocardiography in patients with carcinoid syndrome. Assess left and right-side valves in patients with pulmonary carcinoids. B Perioperative thromboprophylaxis (lobectomy/segmentectomy): consider administering parenteral anticoagulation with subcutaneous LMWH or UFH for VTE prevention in patients undergoing lobectomy or segmentectomy. C Consider preferring LMWH over UFH. C Show 7 more Perioperative thromboprophylaxis (pneumonectomy): consider administering parenteral anticoagulation with subcutaneous LMWH or UFH for VTE prevention in patients undergoing pneumonectomy or extended lung resection. C Consider preferring LMWH over UFH. C Show 6 more 7. Follow-up and surveillance Follow-up: As per ESMO 2021 guidelines, obtain life-long follow-up in patients with lung carcinoids since recurrences remain very common. B Obtain life-long follow-up with low-radiation imaging and increasing interval of time, adjusted to prognostic factors, after radical resection of lung carcinoids. B Show 3 more As per ENETS 2015 guidelines, obtain long-term follow-up after primary surgery in patients with typical or atypical carcinoids. B As per ACCP 2013 guidelines, involve the original treating physicians in decision-making during the follow-up and surveillance of patients with bronchopulmonary carcinoid tumors treated with https://web.pathway.md/diseases/recNaF7ifeeiz8JWz 5/6 6/29/23, 3:21 AM Bronchopulmonary neuroendocrine tumors Pathway curative-intent therapy. B Show 2 more Clinical findings Symptoms Past medical history Chest pain Acromegaly Cough Carcinoid syndrome Hemoptysis Cushing's syndrome Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia Family history Recurrent pneumonia MEN1 Respiratory exam Imaging findings Wheezing Atelectasis Hilar lymphadenopathy Lung mass Lung nodule Mediastinal enlargement Mediastinal lymphadenopathy Mediastinal mass Pleural effusion References 1. M E Caplin, E Baudin, P Ferolla et al. Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids. Ann Oncol. 2015 Aug;26 8 1604 20. Open 2. E Baudin, M Caplin, R Garcia-Carbonero et al. Lung and thymic carcinoids: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2021 Apr;32 4 439 451. Open 3. Rajesh V Thakker, Paul J Newey, Gerard V Walls et al. Clinical practice guidelines for multiple endocrine neoplasia type 1 MEN1 . J Clin Endocrinol Metab. 2012 Sep;97 9 2990 3011. Open 4. Frank C Detterbeck, Sandra Zelman Lewis, Rebecca Diekemper et al. Executive Summary: Diagnosis and management of lung cancer, 3rd ed: American College of Chest Physicians evidence-based clinical practice guidelines. Chest. 2013 May;143 5 Suppl):7S 37S. Open 5. Yaron Shargall, Wojtek Wiercioch, Alessandro Brunelli et al. Joint 2022 European Society of Thoracic Surgeons and The American Association for Thoracic Surgery guidelines for the prevention of cancer- associated venous thromboembolism in thoracic surgery. Eur J Cardiothorac Surg. 2022 Dec 2;63 1):ezac488. Open https://web.pathway.md/diseases/recNaF7ifeeiz8JWz 6/6
Guideline sources The following summarized guidelines for the evaluation and management of brugada syndrome (BS) are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC 2022; 2018), the American Heart Association (AHA/HRS/ACC 2018), the Asia Pacific Heart Rhythm Society (APHRS/EHRA/HRS 2013), and the European Heart Rhythm Association (EHRA/HRS 2011). 1 2 3 4 5 Calculator NYHA functional classification f Guidelines 1. Screening and diagnosis Diagnosis: https://web.pathway.md/diseases/recoFegDbEM4Q3Udl 1/5 6/29/23, 3:21 AM Brugada syndrome Pathway As per ESC 2022 guidelines, diagnose BS in patients with spontaneous type 1 Brugada ECG pattern and no other heart disease. B Show 3 more As per EHRA 2013 guidelines: Diagnose BS in patients with ST-segment elevation with type 1 morphology 2 mm in 1 lead among the right precordial leads V1, V2, positioned in the 2nd, 3rd, or 4th intercostal space occurring either spontaneously or after provocative drug test with IV administration of class I antiarrhythmic drugs. B Diagnose BS in patients with type 2 or type 3 ST-segment elevation in 1 lead among the right precordial leads V1, V2 positioned in the 2nd, 3rd, or 4th intercostal space when a provocative drug test with IV administration of class I antiarrhythmic drugs induces a type I ECG morphology. B 2. Diagnostic investigations Drug challenge testing: As per ESC 2022 guidelines, do not obtain sodium channel blocker testing in patients with a prior type 1 Brugada pattern. D As per AHA 2018 guidelines, consider obtaining pharmacological challenge testing with a sodium channel blocker in patients with suspected BS in the absence of spontaneous type 1 Brugada ECG pattern. C Genetic testing: As per ESC 2022 guidelines, obtain genetic testing for SCN5A gene mutation in probands with BS. B As per AHA 2018 guidelines: Consider providing genetic counseling and obtaining genetic testing to facilitate cascade screening of relatives in patients with suspected or established BS. C Provide genetic counseling and obtaining mutation-specific genetic testing in first-degree relatives of patients with a causative mutation for BS. B As per HRS 2011 guidelines, obtain mutation-specific genetic testing in family members and appropriate relatives following the identification of BS-causative mutation in an index case. A Show 2 more 3. Diagnostic procedures Implantable loop recorder: consider implanting a loop recorder in patients with BS with unexplained syncope. C Electrophysiology study: https://web.pathway.md/diseases/recoFegDbEM4Q3Udl 2/5 6/29/23, 3:21 AM Brugada syndrome Pathway As per ESC 2022 guidelines, consider performing programmed electrical stimulation in asymptomatic patients with a spontaneous type I BS ECG pattern. C As per AHA 2018 guidelines, consider obtaining an electrophysiological study with programmed ventricular stimulation using single and double extra stimuli for further risk stratification in patients with asymptomatic BS and spontaneous type 1 Brugada ECG pattern. C As per ESC 2018 guidelines, consider obtaining an invasive electrophysiology study in patients with Brugada ECG pattern and syncope of suspected arrhythmic etiology. C 4. Medical management Avoidance of aggravating factors: As per ESC 2022 guidelines, avoid using drugs likely to induce ST-segment elevation in right precordial leads in patients with BS. D Show 2 more As per EHRA 2013 guidelines, avoid using drugs likely to induce or aggravate ST-segment elevation in right precordial leads in patients with BS. D Show 2 more Watchful waiting: offer observation without therapy in asymptomatic patients with only inducible type 1 Brugada ECG pattern. B Quinidine: As per ESC 2022 guidelines, consider initiating quinidine in patients with BS qualifying for an ICD but having a contraindication, declining, or having recurrent ICD shocks. C As per AHA 2018 guidelines: Offer intensification of therapy with quinidine or catheter ablation in patients with BS experiencing recurrent ICD shocks for polymorphic VT. B Initiate quinidine or offer catheter ablation in patients with spontaneous type 1 Brugada ECG pattern and symptomatic ventricular arrhythmia either not eligible for or declining ICD placement. B As per ESC 2013 guidelines, consider initiating quinidine in patients with BS and a history of arrhythmic storms defined as > 2 episodes of VT/VF in 24 hours. C Show 2 more Isoproterenol infusion: As per ESC 2022 guidelines, consider administering isoproterenol infusion in patients with BS in electrical storm. C As per EHRA 2013 guidelines, consider administering isoproterenol infusion for the management of arrhythmic storms in patients with BS. C 5. Therapeutic procedures https://web.pathway.md/diseases/recoFegDbEM4Q3Udl 3/5 6/29/23, 3:21 AM Brugada syndrome Pathway Implantable cardioverter-defibrillator: As per ESC 2022 guidelines, perform ICD placement in patients with BS with a history of any of the following: aborted cardiac arrest documented spontaneous sustained VT. B Show 2 more As per AHA 2018 guidelines: Perform ICD in patients with a cardiac channelopathy and sudden cardiac arrest, if the expected meaningful survival is > 1 year. B Perform ICD in patients with BS with spontaneous type 1 Brugada ECG pattern survived cardiac arrest, sustained ventricular arrhythmia, or with a recent history of syncope presumed due to ventricular arrhythmia, if the expected meaningful survival is > 1 year. B As per ESC 2018 guidelines: Consider performing ICD placement in patients with Brugada ECG pattern and syncope of suspected arrhythmic etiology. C Do not perform ICD placement in patients with Brugada ECG pattern and reflex-mediated syncope in the absence of other risk factors. D As per EHRA 2013 guidelines, perform ICD placement in patients with BS with any of the following: cardiac arrest documented spontaneous sustained VT with or without syncope. A Show 3 more Catheter ablation: As per ESC 2022 guidelines: Consider performing catheter ablation of triggering premature ventricular contractions and/or RV outflow tract epicardial substrate in patients with BS with recurrent appropriate ICD shocks refractory to drug therapy. C Do not perform catheter ablation in asymptomatic patients with BS. D As per AHA 2018 guidelines: Perform catheter ablation or offer intensification of therapy with quinidine in patients with BS experiencing recurrent ICD shocks for polymorphic VT. B Perform catheter ablation or initiate quinidine in patients with spontaneous type 1 Brugada ECG pattern and symptomatic ventricular arrhythmia either not eligible for or declining ICD placement. B As per EHRA 2013 guidelines, consider performing catheter ablation in patients with BS and a history of arrhythmic storms or repeated appropriate ICD shocks. C Clinical findings https://web.pathway.md/diseases/recoFegDbEM4Q3Udl 4/5 6/29/23, 3:21 AM Brugada syndrome Pathway Symptoms Cardiac exam Chest discomfort Cardiac arrest Dyspnea Genetic testing Palpitations Seizure Scn5a gene mutation Syncope ECG findings St-segment elevation VF VT References 1. Sana M Al-Khatib, William G Stevenson, Michael J Ackerman et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2018 Sep 25;138 13):e272-e391. Open 2. Silvia G Priori, Arthur A Wilde, Minoru Horie et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013 Dec;10 12 1932 63. Open 3. Katja Zeppenfeld, Jacob Tfelt-Hansen, Marta de Riva et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Aug 26;ehac262. Open 4. Michele Brignole, Angel Moya, Frederik J de Lange et al. 2018 ESC Guidelines for the diagnosis and management of syncope. Eur Heart J. 2018 Jun 1;39 21 1883 1948. Open 5. Michael J Ackerman, Silvia G Priori, Stephan Willems et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society HRS and the European Heart Rhythm Association EHRA . Heart Rhythm. 2011 Aug;8 8 1308 39. Open https://web.pathway.md/diseases/recoFegDbEM4Q3Udl 5/5