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巴基斯坦儿童肾病综合征的组织病理学谱。

Histopathological spectrum of childhood nephrotic syndrome in Pakistan.

机构信息

Department of Histopathology, Sindh Institute of Urology and Transplantation, Civil Hospital, Karachi, Pakistan.

出版信息

Clin Exp Nephrol. 2009 Dec;13(6):589-93. doi: 10.1007/s10157-009-0216-0. Epub 2009 Jul 28.

Abstract

BACKGROUND

There is no information in international literature on the pattern of glomerulopathies in children with idiopathic nephrotic syndrome (INS) in Pakistan. We undertook this study to determine the pattern of glomerulopathies based on renal biopsies studied by light (LM), immunofluorescence (IF), and electron microscopy (EM).

METHODS

The study was conducted at Sindh Institute of Urology and Transplantation (SIUT), Karachi over 12 years (1996-2008). All children (<or=18 years) with INS in whom renal biopsy was performed were included. Renal biopsies were studied by LM, IF, and EM.

RESULTS

Of 538 children, 347 (64.4%) were male and 191 (35.5%) were female. Mean age was 9.79 +/- 4.59 years. The histopathological lesions comprised: minimal change disease (MCD) and its variants, 43.8%; focal segmental glomerulosclerosis (FSGS), 38.14%; membranous glomerulonephritis (GN) (MGN), 7.96%; mesangioproliferative GN (MesPGN), 4.81%; mesangiocapillary GN (MPGN), 3.14%; IgA nephropathy (IgAN), 1.11%; and other rare lesions.

CONCLUSION

MCD and its variants are the leading cause of overall INS in children, followed by FSGS, which is the predominant pathology in steroid-resistant and adolescent nephrotic syndrome (NS). Our data are in accordance with recent series from around the world with similar biopsy indications. The study defines the true pattern of glomerulopathies in childhood INS for the first time in Pakistan.

摘要

背景

国际文献中尚无关于巴基斯坦特发性肾病综合征(INS)患儿肾小球疾病模式的信息。我们进行这项研究是为了根据光镜(LM)、免疫荧光(IF)和电子显微镜(EM)检查的肾活检来确定肾小球疾病的模式。

方法

该研究于 1996 年至 2008 年在卡拉奇的信德泌尿科和移植研究所(SIUT)进行。所有接受肾活检的 INS 儿童(<或=18 岁)均纳入研究。对肾活检进行 LM、IF 和 EM 检查。

结果

538 例患儿中,男性 347 例(64.4%),女性 191 例(35.5%)。平均年龄为 9.79+/-4.59 岁。组织病理学病变包括:微小病变病(MCD)及其变异型,占 43.8%;局灶节段性肾小球硬化(FSGS),占 38.14%;膜性肾小球肾炎(MGN),占 7.96%;系膜增生性肾小球肾炎(MesPGN),占 4.81%;膜毛细血管性肾小球肾炎(MPGN),占 3.14%;IgA 肾病(IgAN),占 1.11%;以及其他罕见病变。

结论

MCD 及其变异型是儿童总体 INS 的主要原因,其次是 FSGS,FSGS 是类固醇耐药和青少年肾病综合征(NS)的主要病理类型。我们的数据与世界各地具有类似活检指征的最新系列研究结果一致。该研究首次在巴基斯坦确定了儿童 INS 肾小球疾病的真实模式。

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