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[肾母细胞瘤、假性两性畸形与肾小球肾病的关联。该综合征的部分及完全形式]

[Association of Wilms' tumor, pseudohermaphroditism and glomerular nephropathy. Partial and complete forms of the syndrome].

作者信息

Chattás A J, Delgado N, Gallo G E, Schvartzman E, Canepa C, Sojo E T

出版信息

Bol Med Hosp Infant Mex. 1979 Nov-Dec;36(6):1203-12.

PMID:226110
Abstract

Four patients, followed at the Buenos Aires Cildren's Hospital are reported: one with a nephrotic syndrome and male pseudohermaphroditism, the second with nephrotic syndrome, male pseudohermaphroditism and Wilms' tumor, the third with pseudohermaphroditism and Wilms' tumor and the fourth with a nephrotic syndrome and Wilms' tumor. The cases presenting nephrotic syndrome, all had an early onset and a rapid and fatal course leading to death in renal failure or secondary to a related bacterial infection. The renal histology in all of the cases with nephrotic syndrome, was very similar: diffuse involvement of all glomeruli consisting in a severe increase of the mesangial matrix, with scarce mesengial proliferation.

摘要

报告了在布宜诺斯艾利斯儿童医院接受随访的4例患者:1例患有肾病综合征和男性假两性畸形,第2例患有肾病综合征、男性假两性畸形和肾母细胞瘤,第3例患有假两性畸形和肾母细胞瘤,第4例患有肾病综合征和肾母细胞瘤。出现肾病综合征的病例均起病早,病程迅速且致命,最终死于肾衰竭或继发于相关细菌感染。所有肾病综合征病例的肾脏组织学表现非常相似:所有肾小球弥漫受累,表现为系膜基质严重增多,系膜细胞增生较少。

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