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三姐妹患薄基底膜病合并IgA肾病一例。

A case of IgA nephropathy in three sisters with thin basement membrane disease.

作者信息

Yoshida K, Suzuki J, Suzuki S, Kume K, Suzuki H, Hujiki T

机构信息

Department of Padiatrics, Fakushima Medical College, Fukushima, Japan.

出版信息

Am J Nephrol. 1998;18(5):422-4. doi: 10.1159/000013387.

Abstract

IgA nephropathy associated with thin basement membrane disease is reported in a 9-year-old female. The diagnosis of IgA nephropathy was made by means of an immunofluorescence investigation, which showed generalized diffuse mesangial deposits. Thin basement membrane disease was identified by electron-microscopic investigations, which disclosed thinning of the basement membrane of several capillary loops and prominence of the lamina densa. Her father, elder sister and younger sister were also found to have hematuria and her sisters were diagnosed as having thin basement membrane disease by renal biopsy. Patients with IgA nephropathy have focal thinning of the glomerular basement membrane, but we consider that urinalysis of the family needs to be done for the diagnosis of familial thin basement membrane disease, when diffuse thinning of the glomerular basement membrane is detected in such patients.

摘要

一名9岁女性被报道患有与薄基底膜病相关的IgA肾病。IgA肾病的诊断通过免疫荧光检查做出,结果显示为弥漫性系膜沉积。薄基底膜病通过电子显微镜检查确定,结果显示多个毛细血管袢的基底膜变薄且致密层突出。她的父亲、姐姐和妹妹也被发现有血尿,她的妹妹经肾活检被诊断为患有薄基底膜病。IgA肾病患者有肾小球基底膜局灶性变薄,但当在这类患者中检测到肾小球基底膜弥漫性变薄时,我们认为对其家族进行尿液分析对于诊断家族性薄基底膜病是必要的。

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