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[病例报告:肾上腺性征异常综合征中外生殖器的手术重建]

[A case report: surgical reconstruction of the external genitalia in adrenogenital syndrome].

作者信息

Sakamoto W, Kawashima H, Nishijima T, Senju M, Nariyama M, Kishimoto T, Maekawa M

机构信息

Department of Urology, Toyonaka City Hospital.

出版信息

Hinyokika Kiyo. 1987 Dec;33(12):2095-102.

PMID:3329455
Abstract

This is a case report of a 5-year-old girl with congenital adrenal hyperplasia (CAH) who had clitoral hypertrophy and vagina opening at verumontanum between the bladder neck and external urethral sphincter. Female pseudohermaphroditism with CAH is the most common type of intersex problem seen in children. In females with this disorder, the internal genital organs are usually normal, but variable degrees of virilization can be observed externally. We discussed the indication of the surgical correction of ambiguous external genitalia in this syndrome in view of sexual function and cosmetic problems.

摘要

这是一例关于一名5岁先天性肾上腺皮质增生症(CAH)女孩的病例报告,该女孩存在阴蒂肥大以及膀胱颈与尿道外括约肌之间的精阜处有阴道开口。CAH所致的女性假两性畸形是儿童中最常见的两性畸形问题类型。患有这种疾病的女性,其内部生殖器官通常是正常的,但在外部可观察到不同程度的男性化。鉴于性功能和外观问题,我们讨论了该综合征中对外生殖器模糊进行手术矫正的指征。

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