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[塌陷性肾小球病:一种与肾病综合征和终末期肾衰竭相关的新实体]

[Collapsing glomerulopathy: a new entity associated with nephrotic syndrome and end-stage renal failure].

作者信息

Avila-Casado M C

机构信息

Departamento de Patología, Instituto Nacional de Cardiología Ignacio Chávez, México, D.F.

出版信息

Rev Invest Clin. 1999 Nov-Dec;51(6):367-73.

Abstract

Collapsing glomerulopathy is an aggressive form of glomerular disease defined for its histopathological features of glomerular collapse, visceral epithelial cell damage and tubulointerstitial changes that are characteristic. Patients with collapsing glomerulopathy present with severe nephrotic syndrome, marked proteinuria, generally more than 10 g/day and rapid progression to chronic renal failure, or death due to complications of nephrotic syndrome, despite any form of treatment. Collapsing glomerulopathy presents as de novo or recurrent disease in the renal allograft. There is slight predominance in males and strong predominance in blacks as renal diseases in general. Collapsing glomerulopathy shares several clinical and histopathological features with focal and segmental glomerulosclerosis and HIV-nephropathy; nevertheless, there is enough evidence to support collapsing glomerulopathy as a different entity. It must be mentioned that collapsing glomerulopathy, focal and segmental glomerulosclerosis and HIV-nephropathy may have a similar pathophysiological mechanism of damage to the visceral epithelial cell.

摘要

塌陷性肾小球病是一种侵袭性肾小球疾病,其定义基于肾小球塌陷、脏层上皮细胞损伤和特征性的肾小管间质改变等组织病理学特征。塌陷性肾小球病患者表现为严重肾病综合征、显著蛋白尿,通常每日超过10克,并迅速进展为慢性肾衰竭,或因肾病综合征并发症死亡,无论采用何种治疗方式。塌陷性肾小球病可表现为肾移植中的新发或复发性疾病。总体而言,男性略占优势,黑人中更为常见,如同一般的肾脏疾病。塌陷性肾小球病与局灶节段性肾小球硬化症和HIV相关性肾病具有若干临床和组织病理学特征;然而,有足够的证据支持塌陷性肾小球病是一种不同的疾病实体。必须指出的是,塌陷性肾小球病、局灶节段性肾小球硬化症和HIV相关性肾病可能具有相似的损伤脏层上皮细胞的病理生理机制。

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