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塌陷性肾小球病:沙特阿拉伯的情况。31例病例研究及文献综述

Collapsing glomerulopathy, the Saudi Arabian scenario. A study of 31 cases and a review of literature.

作者信息

Husain Sufia

机构信息

Department of Pathology and Laboratory Medicine, College of Medicine, King Khalid University Hospital Medical City, King Saud University, Riyadh, Kingdom of Saudi Arabia. E-mail.

出版信息

Saudi Med J. 2017 May;38(5):509-516. doi: 10.15537/smj.2017.5.19299.

Abstract

To compare the clinico-pathological features of collapsing glomerulopathy (CG) at a tertiary hospital in Saudi Arabia with the world literature. Methods: In a retrospective study, all biopsy-diagnosed cases of CG between 2004-2015 were identified and analyzed, at King Khalid University Hospital, King Saud University, Riyadh. The clinico-pathological findings along with prognosis were reviewed and compared with the reported literature.  Results: Thirty-one CG patients were identified, most were adult males. All the CG cases were idiopathic, all Arabs, none HIV positive, none of African descent, and none with a history of drug abuse. The number of glomeruli with collapsing lesions per biopsy ranged from 1 to 9. Other types of FSGS lesions (not otherwise specified and perihilar) were also noted. There was extensive podocyte effacement. Upon treatment, remission (complete/partial) was noted in almost half the patients; around one fourth did not respond to treatment; and one fourth progressed to end stage kidney disease (ESKD). The median time taken to develop ESKD from the time of biopsy diagnosis was 23 months. Conclusion: The clinico-pathological and prognostic correlates of CG in Saudi Arabia are comparable with that of the world literature. The management protocol at our center is the same as that practiced in different parts of the world, and the prognosis is overall poor.

摘要

比较沙特阿拉伯一家三级医院的塌陷性肾小球病(CG)的临床病理特征与世界文献报道。方法:在一项回顾性研究中,对沙特国王大学利雅得国王哈立德大学医院2004年至2015年期间所有经活检确诊的CG病例进行识别和分析。回顾临床病理结果及预后情况,并与已发表的文献进行比较。结果:共识别出31例CG患者,大多数为成年男性。所有CG病例均为特发性,均为阿拉伯人,无HIV阳性者,无非洲裔,无药物滥用史。每次活检中出现塌陷性病变的肾小球数量为1至9个。还发现了其他类型的FSGS病变(未另行说明和肾小球旁型)。存在广泛的足细胞足突消失。治疗后,近一半患者实现缓解(完全/部分);约四分之一患者对治疗无反应;四分之一患者进展至终末期肾病(ESKD)。从活检诊断时起发展为ESKD的中位时间为23个月。结论:沙特阿拉伯CG的临床病理及预后相关性与世界文献报道相当。我们中心的管理方案与世界不同地区实施的方案相同,且总体预后较差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d054/5447212/b234c82d55e6/SaudiMedJ-38-509-g003.jpg

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