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[男性假两性畸形。睾丸女性化综合征]

[Male pseudohermaphroditism. Testicular feminization syndrome].

作者信息

Liu W A

机构信息

Second Affiliated Hospital, Fujian Medical College.

出版信息

Zhonghua Wai Ke Za Zhi. 1992 Mar;30(3):165-6, 190.

PMID:1473390
Abstract

Male pseudohermaphroditism--testicular feminization syndrome is a rare genetic entity with considerably familial predisposition. Of the 9 cases, 7 except 2 children were confirmed surgically. Clinical manifestations of the disorder were primary amenorrhea, infertility and a mass in groin. We suggested that orchiectomy is necessary after puberty. The genetic pattern, clinical characteristics, diagnosis and treatment of the condition were briefly discussed with a review of the literature.

摘要

男性假两性畸形——睾丸女性化综合征是一种罕见的具有明显家族易感性的遗传疾病。9例患者中,除2名儿童外,其余7例经手术确诊。该疾病的临床表现为原发性闭经、不孕和腹股沟肿块。我们建议青春期后行睾丸切除术。结合文献复习,简要讨论了该病的遗传模式、临床特征、诊断和治疗。

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