【非典型溶血性尿毒症综合征诊断与治疗的多学科共识(2025版)】。

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引用次数: 0

摘要

非典型溶血性尿毒症综合征(aHUS)是一种以微血管性溶血性贫血、血小板减少和多器官损害为特征的血栓性微血管疾病,以肾脏为主。aHUS是由补体替代通路失调引起的,由感染、妊娠和手术等因素引发。因此,补体抑制剂通过阻断补体系统的异常激活,改善患者预后,在aHUS的治疗中起着至关重要的作用。本共识由非典型溶血性尿毒症综合征诊疗多学科共识工作组在最新文献和指南的基础上形成,探讨了aHUS的发病机制、诊断与鉴别诊断、治疗策略等,为中国aHUS的规范化诊断和管理提供指导和参考。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Multidisciplinary consensus on the diagnosis and treatment of atypical hemolytic uremic syndrome (2025 version)].

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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