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男性假两性畸形中的肾母细胞瘤和肾病综合征

Wilms tumor and nephrotic syndrome in male pseudohermaphroditism.

作者信息

Koyanagi T, Hirama M, Taniguchi K, Gotoh T, Inoue K, Maru A, Tsuji I

出版信息

Urology. 1984 Dec;24(6):595-600. doi: 10.1016/0090-4295(84)90110-9.

Abstract

A case is reported of a child with male pseudohermaphroditism in whom Wilms tumor developed at age twenty-two months. The tumor was treated accordingly, but the child subsequently died of nephrotic syndrome with renal failure at age thirty-two months. After reviewing the similar concurrence of these disorders described as a syndrome, it was suggested that they may have basic embryologic abnormalities in common and that all of them originate during embryogenesis. The importance of bearing this syndrome in mind in the management of a child with abnormal gonadal differentiation is stressed.

摘要

报告了一例患有男性假两性畸形的儿童,该儿童在22个月大时患上了威尔姆斯瘤。对该肿瘤进行了相应治疗,但该儿童随后在32个月大时死于伴有肾衰竭的肾病综合征。在回顾了被描述为一种综合征的这些疾病的类似并发情况后,有人提出它们可能有共同的基本胚胎学异常,并且所有这些异常都起源于胚胎发生过程。强调了在管理性腺分化异常的儿童时牢记这种综合征的重要性。

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