Diagnosing and Managing Androgen Insensitivity Syndrome
Diagnosing and Managing Androgen Insensitivity Syndrome作者机构：General Referal National Hospital of N’Djamena N’Djamena Chad Laquintinie Hospital of Douala Douala Cameroon Protestant Hospital of N’Gaoundere N’Gaoundere Cameroon Grand Yoff General Hospital of Dakar Dakar Senegal
出 版 物：《Open Journal of Urology》 (泌尿学期刊（英文）)
年 卷 期：2017年第7卷第11期
页 面：219-225页
学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学]
主 题：Genital Malformation Androgen Genitoplasty Vaginoplasty Chromosomal Sex
摘 要：Introduction: Androgen insensitivity syndrome is a rare congenital abnormality of genital organs revealing a female phenotype in a person with XY chromosomes. The aim of our work is to describe the clinical aspects, to determine the chromosomal sex and to report our therapeutic management. Observation: A 28-year-old woman with female phenotype, feminine voice, normal breast development, normal underarms and pubic hair, absence of menstruation, fusion of small and large vaginal lips leaving a small pertuis to serve as urethral meatus. On the dorsal surface of the large, left lip was a mass the size of a date. Hormonal balance was normal. A feminizing genitoplasty was performed as well as excision of the mass. Histological analysis of the mass concluded that it was a feminizing testicular. Conclusion: Androgen insensitivity syndrome is a rare abnormality of the genitals. Multidisciplinary management is essential both for the designation of the breeding sex and feminizing genitoplasty.
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