局灶节段性肾小球硬化致病基因研究进展  

Progress in pathogenic genes of focal segmental glomerulosclerosis

作　　者：吴和燕 王忍 王美秋 彭映潮 高春林 夏正坤 Wu Heyan;Wang Ren;Wang Meiqiu;Peng Yingchao;Gao Chunlin;Xia Zhengkun(Department of Pediatrics,Jinling Hospital,Southern Medical University,Nanjing 210002,China;Department of Pedia-trics,Jinling Hospital,Nanjing Medical University,Nanjing 210002,China)

机构地区：[1]南方医科大学第一临床医学院(东部战区总医院)儿科,南京210002 [2]南京医科大学金陵临床医学院儿科,南京210002

出　　处：《中华实用儿科临床杂志》2020年第17期1358-1360,共3页Chinese Journal of Applied Clinical Pediatrics

基　　金：江苏省重点研发计划-临床前沿技术项目(BE2017719);江苏省儿科医学创新团队项目(CXTDA2017022)。

摘　　要：局灶节段性肾小球硬化(FSGS)以局灶节段分布的肾小球硬化及足细胞的足突融合为特征,是儿童终末期肾脏病的主要原因。其发病机制尚未完全阐明,但近年来随着分子遗传学的发展,已在FSGS患者中定位超过30个致病基因,为足细胞结构和功能的破坏在其发病机制中提供了证据。现将FSGS的常见致病基因研究进展进行综述。Focal segmental glomerulosclerosis(FSGS)is characterized by the fusion of foot processes of podocytes,and can lead to end-stage kidney disease in children.The pathogenesis of FSGS has not been fully clarified,but more than 30 pathogenic genes have been identified in FSGS patients in recent years with the development of molecular genetics.These findings prove that the destruction of the structure and function of podocytes plays a role in the pathogenesis of FSGS.In this paper,the research progress of common pathogenic genes of FSGS was reviewed.

关 键 词：局灶节段性肾小球硬化 致病基因 先天性肾病综合征 激素耐药型肾病综合征 

分 类 号：R726.9[医药卫生—儿科]