关于非典型溶血尿毒综合征重新命名专家讨论精萃  

A convergence of expert discussion on the atypical hemolytic uremic syndrome nomenclature

作　　者：张倜 李喆 刘志红 ZHANG Ti;LI Zhe;LIU Zhihong(National Clinical Research Center for Kidney Diseases,Jinling Hospital,Nanjing 210016,China)

机构地区：[1]东部战区总医院国家肾脏疾病临床医学研究中心,南京210016

出　　处：《肾脏病与透析肾移植杂志》2024年第6期551-554,共4页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家自然科学基金青年项目(82300770)。

摘　　要：自1970年代中期开始使用非典型溶血尿毒综合征这一术语,相较于具有流行病学特征且表现典型的溶血尿毒综合征,其最初被用于描述家族性或散发性的病例。随着时间的推移,非典型溶血尿毒综合征的定义逐渐演变为非志贺毒素相关的溶血尿毒综合征。多种病因迥异、高度异质的疾病均被命名为非典型溶血尿毒综合征,这不利于疾病的研究以及提供针对性的治疗。为此美国国家肾脏基金会工作组成立血栓性微血管病专家组,采用德尔菲法,对非典型溶血尿毒综合征命名相关的一些关键问题进行定义和探讨。The term atypical hemolytic uremic syndrome(aHUS)has been used since the mid-1970s.It was originally used to describe the familial or sporadic cases,as opposed to the typical hemolytic uremic syndrome with epidemiological characteristics.Over time,the term aHUS has evolved to include any form of hemolytic uremic syndrome that is not associated with Shiga toxin.Diseases with distinct etiologies and high heterogeneity are uniformly referred to as aHUS,which impedes the study of specific diseases and the provision of targeted therapies.Recently,the United States National Kidney Foundation(NKF)working group established a specialty-specific expert group in the thrombotic microangiopathies,utilizing the Delphi approach to define and discuss several key issues related to the nomenclature of aHUS.

关 键 词：溶血尿毒综合征 血栓性微血管病 补体 命名法 

分 类 号：R692.5[医药卫生—泌尿科学]