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薄基底膜肾病所致的家族性血尿

Familial haematuria due to thin basement membrane nephropathy.

作者信息

Bailey R R

机构信息

Department of Nephrology, Christchurch Hospital.

出版信息

N Z Med J. 1990 Jul 11;103(893):312-3.

PMID:2371004
Abstract

Persistent microscopic haematuria, especially when present in young individuals and associated with proteinuria, is generally of glomerular origin. Important causes include glomerulonephritis and Alport's syndrome. A new type of benign familial haematuria has now been reported characterised by little or no proteinuria, no deafness or hypertension and no deterioration of renal function. Examination by electron microscopy of renal biopsy material shows thinning or attenuation of the glomerular basement membrane. A family with thin basement membrane nephropathy is reported to illustrate this newly recognised entity. This disorder should be considered in any patient with persistent microscopic haematuria. Pathologists who assess renal biopsy material should pay greater attention to an ultrastructural morphometric analysis of the glomerular basement membranes of at least two glomeruli.

摘要

持续性镜下血尿,尤其是在年轻人中出现且伴有蛋白尿时,通常起源于肾小球。重要病因包括肾小球肾炎和阿尔波特综合征。现已报道一种新型良性家族性血尿,其特征为很少或无蛋白尿、无耳聋或高血压且肾功能无恶化。对肾活检材料进行电子显微镜检查显示肾小球基底膜变薄或萎缩。报道了一个薄基底膜肾病家族以说明这一新发现的疾病实体。任何持续性镜下血尿患者均应考虑此病。评估肾活检材料的病理学家应更加重视对至少两个肾小球的肾小球基底膜进行超微结构形态计量分析。

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