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完全性雄激素不敏感综合征或睾丸女性化:基于一例病例报告的文献综述

Complete androgen insensitivity syndrome or testicular feminization: review of literature based on a case report.

作者信息

Souhail Regragui, Amine Slaoui, Nadia Abounouh, Tarik Karmouni, Khalid El Khader, Abdellatif Koutani, Ahmed Ibn Attya

机构信息

Department of Urology B, Ibn Sina University Hospital, Rabat, Morocco.

Department of Gynecology Obstetrics, Maternity ibn Sina, Rabat, Morocco.

出版信息

Pan Afr Med J. 2016 Nov 28;25:199. doi: 10.11604/pamj.2016.25.199.10758. eCollection 2016.

Abstract

Testicular feminization, or the androgen insensitivity syndrome, is a rare disease. Because of various abnormalities of the X chromosome, a male, genetically XY, has some physical characteristics of a woman or a full female phenotype. Indeed the androgen insensitivity syndrome occurs because of a resistance to the actions of the androgen hormones, which in turn switches the development towards the aspect of a woman. We report a case of complete androgen insensitivity syndrome in a 30 years old woman who presented primary amenorrhea. We aim to improve our knowledge of this illness from the data that provides us this study, and a review of the literature.

摘要

睾丸女性化,即雄激素不敏感综合征,是一种罕见疾病。由于X染色体存在各种异常,一名基因上为XY的男性具有一些女性的身体特征或完全女性化的表型。事实上,雄激素不敏感综合征的发生是由于对雄激素作用产生抵抗,这进而使发育转向女性化方向。我们报告一例30岁原发性闭经女性的完全性雄激素不敏感综合征病例。我们旨在通过本研究提供的数据及文献综述来增进对该疾病的了解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c31e/5326263/4a746181b1b3/PAMJ-25-199-g001.jpg

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