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机构地区:[1]南京军区南京总医院全军肾脏病研究所,南京210002
出 处:《肾脏病与透析肾移植杂志》2011年第4期357-361,共5页Chinese Journal of Nephrology,Dialysis & Transplantation
摘 要:以往研究证实多种肾小球肾炎发病机制与补体系统,尤其是补体旁路途径调节异常相关。近年有作者发现一组免疫荧光染色单纯补体C3沿肾小球毛细血管袢沉积的肾小球肾炎,不伴或伴少量免疫球蛋白沉积,其发病机制可能与先天或后天获得性补体系统调节异常相关,该作者将这一组疾病统一命名为C3肾小球病,并根据其临床表现及可能的发病机制分为不同类别。本文就这类疾病作一综述,旨在关注补体在疾病发生中的作用机制,并提高对此类肾小球肾炎的认识。Previous studies found that the pathogenesis of a variety of glomernlonephritis were associated with abnormal rePrevious studies found that the pathogenesis of a variety of glomernlonephritis were associated with abnormal regulation of the complement system, specifically,with dysregulation of the alternative pathway of the complement system. In recent years, clinicians have observed a group of glomernlonephritis which is characterized by glomerular deposits of C3, with no or only scanty glomerular deposits of immunoglobulin, and which pathogenesis may be related to genetic and acquired complement dysregulation. This group of glomerulonephritis was named C3 glomernlopathy,and was classified according to their clinical presentation and possible pathogenesis. This article summarized this group of glomernlonephritis and the relationship between dysregulation of the complement system and glomerular inflammation.
关 键 词:补体系统 C3肾小球肾炎 致密物沉积病 补体H因子相关蛋白5肾病 膜增生性肾小球肾炎
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