溶血性尿毒综合征发病机制和治疗研究进展  

Research Advances on the Pathogenesis and Treatment of Hemolytic Uremic Syndrome——Review

作　　者：刘婉莹 肖毅[1] LIU Wan-Ying;XIAO Yi(Department of Hematology,Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology,Wuhan 430030,Hubei Province,China)

机构地区：[1]华中科技大学同济医学院附属同济医院血液科,湖北武汉430030

出　　处：《中国实验血液学杂志》2022年第2期636-640,共5页Journal of Experimental Hematology

摘　　要：溶血性尿毒综合征(HUS)临床上较为罕见,死亡率和病死率均较高。近年来对HUS的研究不断深入,病理生理学机制的不断完善,目前其发病的主要机制为补体相关基因突变或抗体存在介导的补体替代途径过度激活。治疗方法与策略也不断更新,主要有阻断补体药物如依库丽珠单抗、拉伐珠单抗、Ravulizumab。本文就HUS发病机制和治疗研究的新进展作一概述,为临床治疗HUS提供参考。Hemolytic uremic syndrome(HUS) is clinically rare, with high mortality and case fatality rates. In recent years, the research on HUS has been intensified and the pathophysiological mechanism has been continuously improved. At present, the main mechanism of pathogenesis is the excessive activation of complement alternative pathways mediated by complement-related gene mutations or the existence of antibodies. The treatment methods and strategies are also constantly updated, mainly including complement-blocking drugs such as Eculizumab, Lavalizumab, and Ravulizumab. In this review, the new developments in the pathogenesis and treatment of HUS is summarized, and provide references for the clinical treatment of HUS.

关 键 词：溶血尿毒综合征 志贺毒素 依库丽珠单抗 拉伐珠单抗 Ravulizumab 

分 类 号：R55[医药卫生—血液循环系统疾病]