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抗肾小球基底膜病致肾病综合征一例报告

An unusual case of anti-glomerular basement membrane disease presenting with nephrotic syndrome.

机构信息

Division of Nephrology, Department of Medicine, University of Virginia, Box 800133, Charlottesville, VA 22908, USA.

出版信息

Int Urol Nephrol. 2011 Dec;43(4):1249-53. doi: 10.1007/s11255-010-9862-0. Epub 2010 Nov 18.

Abstract

Anti-glomerular basement membrane (anti-GBM) disease is a vasculitic disease characterized by acute kidney injury, oliguria, hematuria and proteinuria. Proteinuria is rarely in the nephrotic range. A case of anti-GBM disease with proteinuria of 22.5 g/day is discussed. Immunofluorescence showed strong linear IgG deposits while electron microscopy showed widespread visceral epithelial cell foot cell process effacement. No electron dense immune complex-type deposits were identified. Pathology findings were not suggestive of simultaneous presentation of anti-GBM disease and other diseases associated with nephrotic range proteinuria. Anti-GBM disease should be considered in a comprehensive differential diagnosis of severe proteinuria.

摘要

抗肾小球基底膜(anti-GBM)病是一种血管炎疾病,其特征为急性肾损伤、少尿、血尿和蛋白尿。蛋白尿很少达到肾病范围。本文讨论了一例蛋白尿为 22.5 g/天的抗肾小球基底膜病病例。免疫荧光显示强线性 IgG 沉积,而电子显微镜显示广泛的内脏上皮细胞足细胞过程消失。未发现电子致密免疫复合物型沉积物。病理学发现不支持抗肾小球基底膜病和其他与肾病范围蛋白尿相关疾病的同时表现。在严重蛋白尿的综合鉴别诊断中应考虑抗肾小球基底膜病。

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