[Effect of prepuberal gonadectomy in partial androgen resistance syndrome]

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.