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膜增生性肾小球肾炎。

Membranoproliferative glomerulonephritis.

机构信息

Renal Unit, Addenbrooke's Hospital, Cambridge, CB2 0QQ, UK.

出版信息

Pediatr Nephrol. 2010 Aug;25(8):1409-18. doi: 10.1007/s00467-009-1322-7. Epub 2009 Nov 12.

Abstract

Membranoproliferative glomerulonephritis is an uncommon kidney disorder characterized by mesangial cell proliferation and structural changes in glomerular capillary walls. It can be subdivided into idiopathic and secondary forms, which are differentially diagnosed by a review of clinical features, laboratory data, and renal histopathology. Three types-I, II, and III-have been defined by pathologic features. All three types are associated with hypocomplementemia, but they manifest somewhat different mechanisms of complement activation. Type II, also known as "dense deposit disease", is associated with the presence of C3-nephritic factor. Membranoproliferative glomerulonephritis primarily affects children and young adults, with patients presenting with nephrotic or nephritic syndrome or with asymptomatic renal disease. This type of glomerulonephritis often progresses slowly to end-stage renal disease, and it tends to recur after renal transplantation, especially type II. The efficacy of various forms of treatment remains controversial; however, long-term steroid treatment seems to be effective in children with nephrotic-range proteinuria. Improvement in renal outcomes largely relies on the evaluation of more selective agents in carefully controlled studies.

摘要

膜增生性肾小球肾炎是一种不常见的肾脏疾病,其特征为系膜细胞增殖和肾小球毛细血管壁的结构改变。它可以分为特发性和继发性两种,通过回顾临床特征、实验室数据和肾脏组织病理学来进行鉴别诊断。根据病理特征,已经定义了三种类型 - I、II 和 III 型。所有三种类型都与低补体血症相关,但它们表现出不同的补体激活机制。II 型也称为“密沉积病”,与 C3 肾炎因子的存在有关。膜增生性肾小球肾炎主要影响儿童和年轻成年人,患者表现为肾病综合征或肾炎综合征或无症状性肾脏疾病。这种类型的肾小球肾炎通常缓慢进展为终末期肾病,并且在肾移植后往往会复发,尤其是 II 型。各种治疗形式的疗效仍然存在争议;然而,长期使用类固醇治疗似乎对肾病范围蛋白尿的儿童有效。改善肾脏预后在很大程度上依赖于在精心控制的研究中评估更具选择性的药物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2de6/2887509/cb7e7fe32112/467_2009_1322_Fig1_HTML.jpg

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