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塌陷性肾小球病:更新。

Collapsing glomerulopathy: update.

机构信息

Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, España.

Servicio de Anatomía Patológica, Hospital Universitario 12 de Octubre, Madrid, España.

出版信息

Med Clin (Barc). 2019 May 3;152(9):361-367. doi: 10.1016/j.medcli.2018.10.021. Epub 2018 Dec 13.

Abstract

Collapsing glomerulopathy (CG) is a rare entity as a glomerular disease. Although it has been considered as a variant of focal segmental glomerulosclerosis, the fact is that the podocyte lesions show different features with respect to the typical focal segmental glomerulosclerosis, an aspect that has been attributed to a type of podocytopathy. In CG, the podocyte lesion is typically characterised by a dysregulated podocyte phenotype, reflected by the loss of expression of mature podocyte markers. CG can be a primary disease or it can be associated with several causal factors that develop a common histopathological entity. The clinical expressiveness of CG is often characterised by the presence of a nephrotic syndrome and a rapid deterioration of the renal function than other variants of the focal segmental glomerulosclerosis. The prognosis of these patients is a rapid progression towards end-stage renal disease with poor response to treatment.

摘要

塌陷性肾小球病 (CG) 作为一种肾小球疾病较为罕见。虽然它被认为是局灶节段性肾小球硬化症的一种变体,但事实上,足细胞病变相对于典型的局灶节段性肾小球硬化症具有不同的特征,这归因于一种足细胞病变。在 CG 中,足细胞病变的特征通常是足细胞表型失调,表现为成熟足细胞标志物表达缺失。CG 可以是一种原发性疾病,也可以与几种因果因素相关,这些因素导致了一种共同的组织病理学实体。CG 的临床特征通常表现为肾病综合征和肾功能迅速恶化,比其他局灶节段性肾小球硬化症的变体更为严重。这些患者的预后是迅速进展为终末期肾病,对治疗反应不佳。

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