非典型溶血尿毒综合征
重症监护医学
医学
协商一致会议
多学科方法
内科学
免疫学
政治学
抗体
补体系统
法学
出处
期刊:PubMed
[National Institutes of Health]
日期:2025-05-01
卷期号:64 (5): 396-411
标识
DOI:10.3760/cma.j.cn112138-20250219-00095
摘要
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, thrombocytopenia, and multi-organ damage, with the kidneys being predominantly affected. aHUS results from dysregulation of the complement alternative pathway, triggered by factors including infection, pregnancy, and surgery, among others. Therefore, complement inhibitors play a crucial role in the treatment of aHUS by blocking abnormal activation of the complement system and improving patients' prognosis. This consensus, developed by the Multidisciplinary Consensus Working Group on Atypical Hemolytic Uremic Syndrome Diagnosis and Treatment, is based on the latest literature and guidelines, discusses the pathogenesis, diagnosis and differential diagnosis, and treatment strategies for aHUS, and provides a guide and reference for the standardized diagnosis and management of aHUS in China.
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