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[微小病变肾病继发于偶然发现的IgA肾病——病例报告]

[MCNS, which secondary developed into incidental IgA nephropathy--a case report].

作者信息

Tsunehisa N, Takemura T, Inui T, Kamiya H

机构信息

Department of pediatrics Mie National Hospital, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 1992 Oct;34(10):1095-9.

PMID:1289609
Abstract

There have been a number of case reports on nephrotic syndrome with histological findings of minimal change on light microscopy and mesangial IgA deposition on fluorescent microscopy. The pathogenesis of these cases is, however, yet to be clarified. Here, we report a case of minimal change nephrotic syndrome (MCNS) associated with IgA nephropathy, which developed later in the course of MCNS. The patient was 18 years old male with steroid-responsive nephrotic syndrome. First episode of proteinuria occurred when he was 4 years old. On the fourth episode of proteinuria, renal biopsy revealed minimal change on light microscopy and no evidence of deposition of immunoglobulins or complements on immunofluorescent and electron microscopy. On the fifth relapse of MCNS, microhematuria developed concomitantly with massive proteinuria. Renal biopsy, then, showed light microscopic findings of mild focal segmental glomurulonephritis. Significant mesangial IgA deposition was observed on immunofluorescence study. Electron microscopy revealed electron dense deposit in the mesangial and paramesangial area. The patient was well-responsive to steroid although microhematuria persisted after disappearance of proteinuria. We concluded that IgA nephropathy may have developed subsequently in the course of MCNS in our case.

摘要

已有多篇病例报告描述了肾病综合征,其光镜组织学表现为微小病变,荧光显微镜检查显示系膜IgA沉积。然而,这些病例的发病机制尚待阐明。在此,我们报告一例微小病变肾病综合征(MCNS)合并IgA肾病的病例,该病例在MCNS病程后期出现。患者为18岁男性,患有类固醇反应性肾病综合征。蛋白尿首次发作于他4岁时。在蛋白尿第四次发作时,肾活检光镜显示微小病变,免疫荧光和电子显微镜检查未发现免疫球蛋白或补体沉积证据。在MCNS第五次复发时,镜下血尿与大量蛋白尿同时出现。此时肾活检光镜显示轻度局灶节段性肾小球肾炎。免疫荧光研究观察到显著的系膜IgA沉积。电子显微镜检查显示系膜和系膜旁区域有电子致密沉积物。尽管蛋白尿消失后镜下血尿仍持续存在,但患者对类固醇治疗反应良好。我们得出结论,在我们的病例中,IgA肾病可能在MCNS病程中随后发生。

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