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一名两岁男孩指甲-髌骨综合征的肾脏组织病理学表现

Renal histopathology of the nail-patella syndrome in a two-year-old boy.

作者信息

Browning M C, Weidner N, Lorentz W B

机构信息

Department of Pediatrics, Bowman Gray School of Medicine, Wake Forest University, Winston-Salem, NC 27103.

出版信息

Clin Nephrol. 1988 Apr;29(4):210-3.

PMID:3365866
Abstract

A two-year-old child with the clinical stigmata of nail-patella syndrome, congenital urinary tract anomalies and proteinuria underwent renal biopsy. Electron microscopy revealed characteristic electron lucent areas and collagen fibril-like deposits in the glomerular basement membrane. Of special interest, electron dense deposits were seen in subendothelial areas of the capillary loops and immunofluorescent staining was striking, particularly for IgM, in a peripheral capillary loop pattern.

摘要

一名患有指甲-髌骨综合征、先天性泌尿系统异常和蛋白尿临床特征的两岁儿童接受了肾活检。电子显微镜检查显示肾小球基底膜有特征性的电子透亮区和胶原纤维样沉积物。特别值得注意的是,在毛细血管袢的内皮下区域可见电子致密沉积物,免疫荧光染色显著,尤其是IgM,呈外周毛细血管袢模式。

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