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新型治疗模式:局灶节段性肾小球硬化症

Novel Treatment Paradigms: Focal Segmental Glomerulosclerosis.

作者信息

de Cos Marina, Meliambro Kristin, Campbell Kirk N

机构信息

Division of Nephrology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, New York, USA.

出版信息

Kidney Int Rep. 2022 Oct 8;8(1):30-35. doi: 10.1016/j.ekir.2022.10.004. eCollection 2023 Jan.

Abstract

Focal segmental glomerulosclerosis (FSGS) is a histologic pattern of injury defined by the presence of sclerosis in some (segmental) of certain glomeruli (focal). On electron microscopy, it is characterized by a variable degree of podocyte foot process effacement and gaps in the coverage of the glomerular basement membrane. The pattern of injury occurs when podocytes, highly differentiated cells with limited regenerative capacity, are reduced in number. The heterogeneity in underlying causes of podocyte loss results in equally variable clinical phenotypes. Recent work acknowledging advances in defining the genetic and immunologic basis of disease has redefined the classification of FSGS. Unprecedented clinical trial activity and efficacy of repurposed agents presents hope for improved therapeutic options. This minireview summarizes recent advances with a focus on novel treatment paradigms in FSGS.

摘要

局灶节段性肾小球硬化(FSGS)是一种损伤的组织学模式,其定义为某些肾小球(局灶性)的部分(节段性)出现硬化。在电子显微镜下,其特征是足细胞足突不同程度的消失以及肾小球基底膜覆盖存在间隙。当足细胞(具有有限再生能力的高度分化细胞)数量减少时,就会出现这种损伤模式。足细胞丢失的潜在原因的异质性导致了同样多变的临床表型。最近在确定该疾病的遗传和免疫基础方面取得的进展重新定义了FSGS的分类。前所未有的临床试验活动以及重新利用药物的疗效为改善治疗选择带来了希望。本综述总结了近期的进展,重点关注FSGS的新型治疗模式。

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