补体参与儿童肾脏疾病发病机制的再认识  

Re-recognition of the complement involvement in the pathogenesis of kidney diseases in children

作　　者：王培培 张沛(综述) 高春林 夏正坤(审校) WANG Peipei;ZHANG Pei;GAO Chunlin;XIA Zhengkun(Department of Pediatrics,Jinling Hospital,The Eastern Theater General Hospital,Nanjing Medical University,Nanjing 210002,China;Department of Pediatrics,The Eastern Theater General Hospital,Nanjing 210002,China)

机构地区：[1]南京医科大学金陵临床医学院(东部战区总医院)儿科 [2]东部战区总医院儿科,江苏南京210002

出　　处：《临床儿科杂志》2024年第5期467-473,共7页Journal of Clinical Pediatrics

基　　金：江苏省自然科学基金青年基金项目(No.BK20190251);东部战区总医院临床研究专项(No.22LCYY-XH9)。

摘　　要：补体是先天免疫的重要组成部分,也是调节适应性免疫的桥梁,能介导细胞炎症反应和细胞凋亡。补体级联反应受到补体调节蛋白的严格调控,一旦补体过度活化或调节异常,就会导致宿主细胞的损伤。在肾小球微环境中,补体系统调节异常可导致C3肾小球病和非典型溶血尿毒综合征。此外,补体在多种炎性肾脏疾病中过度激活,如抗中性粒细胞胞质抗体相关肾炎、狼疮性肾炎、Ig A肾病、膜性肾病和糖尿病肾病等。另外,它还可能通过在缺血再灌注损伤和排斥反应中发挥作用介导移植肾损伤。现对补体在肾小球疾病发病机制中的作用及相关靶向治疗进行综述。Complement is an important component of innate immunity,as well as a bridge to regulate adaptive immunity.It is an important substance that mediates cellular inflammatory response and induces apoptosis.The cascade of the complement response is stictly regulated by the stringent control of the complennent regulatory proteins.Should the complement overactivated or regulate abnormally,it can cause damage to the host cells.In the glomerular microenvironment,dysregulation of the complement system can lead to C3 glomerulopathy and atypical hemolytic uremic syndrome.In addition,complement is also hyperactivated in many other inflammatory kidney diseases,such as ANCA-associated glomerulonephritis,lupus nephritis,IgA nephropathy,membranous nephropathy,and diabetic nephropathy.In addition,it may mediate renal allograft injury by playing a role in ischemia-reperfusion injury and rejection.This article reviews the role of complement in the pathogenesis of glomerular diseases and related targeted therapy.

关 键 词：补体 肾脏病 靶向治疗 

分 类 号：R726.9[医药卫生—儿科]