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无症状性镜下血尿儿童的家族性薄基底膜肾病

Familial thin basement membrane nephropathy in children with asymptomatic microhematuria.

作者信息

Gauthier B, Trachtman H, Frank R, Valderrama E

机构信息

Schneider Children's Hospital of Long Island Jewish Medical Center, New Hyde Park, N.Y.

出版信息

Nephron. 1989;51(4):502-8. doi: 10.1159/000185384.

Abstract

A review of 130 children with persistent microhematuria showed that the most common abnormality was a form of hereditary nephritis for wich we propose the term 'familial thin basement membrane nephropathy' (FTBMN). This lesion is not as severe as that of Alport's syndrome but carries a guarded prognosis. Family histories, urinalyses and audiograms systematically done on parents and other relatives show that FTBMN may be present in as many as 39% of children with persistent microhematuria and that careful family studies identify children likely to have this lesion.

摘要

对130例持续性镜下血尿患儿的回顾性研究表明,最常见的异常是一种遗传性肾炎,我们建议将其命名为“家族性薄基底膜肾病”(FTBMN)。这种病变不如阿尔波特综合征严重,但预后不佳。对父母和其他亲属系统地进行家族史、尿液分析和听力图检查表明,在持续性镜下血尿患儿中,多达39%可能患有FTBMN,仔细的家族研究可识别出可能患有这种病变的儿童。

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