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特发性快速进展性肾小球肾炎的演变观察

Observations on the evolution of idiopathic rapidly progressive glomerulonephritis.

作者信息

Davis C A, McEnery P T, Maby S, McAdams A J, West C D

出版信息

Clin Nephrol. 1978 Mar;9(3):91-101.

PMID:346274
Abstract

Distinctive deposits of C3, C5 and properdin were identified in the minimally proliferative glomerular lesions of a patient with idiopathic rapidly progressive glomerulonephritis. Biopsies of her renal allograft at times of recurrences of her disease and of five other patients with progressive renal failure but less severe crescent formation showed deposits identical composition and position, giving evidence of a common pathogenesis. The deposits were subepithelial and located in that part of the basement membrane in apposition to the mesangium (capillary waist). Breaks in the basement membrane were often exclusively in this area suggesting the deposits were causative. Ancillary observations suggest that the subepithelial deposits become unidentifiable after the scarring of severe extracapillary proliferation develops. Three of the patients had, in addition, intramembranous dense deposits but in other ways their disease was not characteristic of membranoproliferative glomerulonephritis Type II. Instead, the evidence indicated that all six patients were in early or lage stages of idiopathic (non-streptococcal) rapidly progressive glomerulonephritis.

摘要

在一名特发性快速进展性肾小球肾炎患者的轻度增殖性肾小球病变中,发现了C3、C5和备解素的特征性沉积物。在其疾病复发时对她的肾移植活检以及对另外五名进行性肾衰竭但新月体形成较轻的患者的活检显示,沉积物的组成和位置相同,这证明了共同的发病机制。沉积物位于上皮下,位于与系膜(毛细血管袢)相邻的基底膜部分。基底膜的断裂通常仅发生在该区域,表明沉积物是病因。辅助观察表明,在严重的毛细血管外增殖形成瘢痕后,上皮下沉积物变得无法识别。此外,三名患者有膜内致密沉积物,但在其他方面,他们的疾病并非II型膜增生性肾小球肾炎的特征。相反,证据表明所有六名患者均处于特发性(非链球菌性)快速进展性肾小球肾炎的早期或晚期阶段。

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