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C3肾小球病的当前概念

Current concepts in C3 glomerulopathy.

作者信息

Thomas S, Ranganathan D, Francis L, Madhan K, John G T

机构信息

Department of Renal Medicine, Royal Brisbane and Women's Hospital, Brisbane, Australia.

Department of Pathology, Royal Brisbane and Women's Hospital, Brisbane, Australia.

出版信息

Indian J Nephrol. 2014 Nov;24(6):339-48. doi: 10.4103/0971-4065.134089.

Abstract

Complement component 3 glomerulopathy (C3G) is a recently defined entity comprising of dense deposit disease and C3 glomerulonephritis. The key histological feature is the presence of isolated C3 deposits without immunoglobulins. Often masqueradng as some of the common glomerulonephritides this is a prototype disorder occurring from dysregulated alternate complement pathway with recently identified genetic defects and autoantibodies. We review the pathophysiology, clinical features, and diagnostic and treatment strategies.

摘要

补体成分3肾小球病(C3G)是一种最近定义的疾病实体,包括致密物沉积病和C3肾小球肾炎。关键的组织学特征是存在无免疫球蛋白的孤立C3沉积物。这种疾病常伪装成一些常见的肾小球肾炎,是一种由替代补体途径失调以及最近发现的基因缺陷和自身抗体引起的典型疾病。我们回顾了其病理生理学、临床特征以及诊断和治疗策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f1c9/4244712/73a9ef28a8e8/IJN-24-339-g001.jpg

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