Garcia Morales M A, Barron Vallejo J, Limon Luque L
Médicos Adscritos del Departamento de Ginecología y Obstetrica.
Ginecol Obstet Mex. 1996 Mar;64:120-1.
The androgen resistance syndrome, is a dominant and recessive linked to X disorder, with clinically heterogenous manifestations. Reported case. A 17-years patient with primary amenorrhoea and genital ambiguity. Bilateral inguinal herniplastia and probable gonadectomy were performed at 14-months-old. His cariotipe was male 46,XY and the hormonal determinations showed the presence of hypergonadotropic hypogonadismo. The congenital adrenal hyperplasia (deficiency of 21-hidroxilase) was discarded. In the partial androgen resistance syndrome, the prepuberal gonadectomy avoid a progresive virilization of the external genitals. However, the estrogenic hormonal replacement is mandatory.
雄激素抵抗综合征是一种与X染色体相关的显性和隐性疾病，临床表现具有异质性。病例报告。一名17岁原发性闭经且生殖器模糊的患者。14个月大时进行了双侧腹股沟疝修补术及可能的性腺切除术。其染色体核型为男性46,XY，激素测定显示存在高促性腺激素性性腺功能减退。排除先天性肾上腺增生（21-羟化酶缺乏）。在部分雄激素抵抗综合征中，青春期前性腺切除术可避免外生殖器的进行性男性化。然而，雌激素替代治疗是必需的。
