摘要
C3肾小球病是一类由于先天性遗传变异及获得性自身抗体介导的补体旁路途径异常活化导致的罕见肾小球疾病。肾活检是诊断C3肾小球病的金标准。C3肾小球病主要包括致密物沉积病和C3肾小球肾炎两种类型。免疫荧光染色可见明显的补体C3沉积,可有少量或不伴有免疫球蛋白沉积是其最显著的病理特点。C3肾小球病的临床表现多样化,目前检测手段及治疗方案有限且预后不佳。该文将对近年来关于C3肾小球病的研究进展进行综述,以提高临床医师对C3肾小球病的重视,采取针对性的个体化治疗。
C3 glomerulopathy is a rare disease of glomeruli mediated by abnormal activation of alternative complement pathway secondary to congenital genetic defects and acquired autoantibodies.Renal biopsy is the gold standard for diagnosing C3 glomerulopathy.C3 glomerulopathy encompasses both dense deposit disease and C3 glomerulonephritis.The main glomerular immunofluorescence staining is C3,with few or without immunoglobulins deposition,which is the obvious pathological feature.The clinical manifestations of C3 glomerulopathy are usually various,with limited detection methods and therapies and poor prognosis.This article mainly reviews the progress of C3 glomerulopathy in recent years,in order to improve clinical understanding of C3 glomerulopathy,and choose individualized therapy.
作者
盛爱芹(综述)
傅海东
毛建华(审校)
Sheng Aiqin;Fu Haidong;Mao Jianhua(Department of Nephrology,the Children′s Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,National Children′s Regional Medical Center,Hangzhou 310030,China)
出处
《国际儿科学杂志》
2022年第7期433-437,共5页
International Journal of Pediatrics
基金
国家自然科学基金项目(U20A20351)。
