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C1q肾病的诊断及治疗
作者姓名:倪兆慧  金海姣  徐维佳
作者单位:1. 200127 上海交通大学医学院附属仁济医院肾脏科
基金项目:国家自然科学基金(81370794); 国家科技支撑计划课题(2011BAI10B08)
摘    要:C1q肾病是一种以系膜增生为主的肾小球疾病,其特点为免疫荧光染色可见系膜区高强度C1q沉积,电镜下可见系膜区电子致密物沉积,根据组织病理学特点主要分为3类,包括微小病变(MCD)、局灶节段性肾小球硬化(FSGS)和免疫介导的增生性肾小球肾炎。C1q肾病的临床表现具有多样性,可表现为肾炎或肾病范围内蛋白尿,伴有或不伴有血尿和肾功能损伤。虽然目前糖皮质激素是治疗C1q肾病的主要方法,但多数研究认为C1q肾病对糖皮质激素治疗反应较差。中西医结合治疗有望提高C1q肾病的疗效。

关 键 词:C1q肾病  临床表现  组织学特点  治疗  

Diagnosis and treatment of C1q nephropathy
Authors:Zhaohui Ni  Haijiao Jin  Weijia Xu
Institution:1. Department of Nephrology, Ren Ji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai 200127, China
Abstract:C1q nephropathy is a glomerular disorder with prominent mesangial proliferation. It is characterized by mesangial dense deposits under electron microscopy and C1q deposits under immunofluorescence microscopy. The histologic pattern of C1q nephropathy can be divided into three kinds: minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), and immune-mediated proliferative glomerulonephritis. Its clinical presentation is heterogenous, ranging from nephritic to nephrotic proteinuria, with or without hematuria and renal insufficiency. Glucocorticoids remain the mainstay of treatment. Most studies indicated poor response of C1q nephropathy to glucocorticoids. The treatment of integrated Chinese and western medicine for C1q nephropathy is promising.
Keywords:C1q nephropathy  Clinical presentation  Histologic pattern  Treatment  
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