冯-威廉氏病:内科医生指南

IF 3.4 4区 医学 Q1 MEDICINE, GENERAL & INTERNAL Cleveland Clinic Journal of Medicine Pub Date : 2024-02-02 DOI:10.3949/ccjm.91a.22033
Varinder Kaur, Omar Elghawy, Saarang Deshpande, David Riley
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引用次数: 0

摘要

冯-威廉氏病(VWD)是最常见的遗传性出血性疾病,是由于患者体内的冯-威廉因子(VWF)不足或有缺陷而导致的。这种疾病的病理生理学非常复杂,但需要通过了解病理生理学来解释诊断测试。大多数患者的症状为轻度至中度,可由普通内科医生给予适当的指导和处理,但有些患者需要咨询血液科医生。我们回顾了 VWD 的病理生理学、其亚型、各亚型的常见表现、诊断测试以及轻度和重度 VWD 临床表现的处理。
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von Willebrand disease: A guide for the internist.
von Willebrand disease (VWD), the most common inherited bleeding disorder, results when patients either do not make enough von Willebrand factor (VWF) or make defective VWF. The pathophysiology of this disorder is complex but needs to be understood to interpret the diagnostic tests. Most patients have mild to moderate symptoms and can be adequately counseled and managed by a general internist, but some need to consult a hematologist. We review the pathophysiology of VWD, its subtypes, common presentations of each subtype, diagnostic testing, and management of mild as well as severe clinical manifestations of VWD.
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来源期刊
Cleveland Clinic Journal of Medicine
Cleveland Clinic Journal of Medicine 医学-医学:内科
CiteScore
3.80
自引率
0.00%
发文量
109
审稿时长
6-12 weeks
期刊介绍: The mission of Cleveland Clinic Journal of Medicine (CCJM) is to provide its readers with up-to-date, practical, clinical information relevant to internal medicine, cardiology, and related fields. Consistent with this mission, CCJM focuses on timely review articles and other content that has a continuing-education orientation rather than on original research or case reports. CCJM authors, drawn from Cleveland Clinic and other top medical institutions throughout the world, are asked to identify new findings that are changing the practice of medicine and to advise readers how to apply them in daily patient care. Authors are chosen for their experience, acquired through caring for patients, teaching other physicians, and researching clinical questions.
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