RESUMO
Hypothalamic-pituitary gonadotropin function was evaluated in two postpubertal XX males. Serum levels of LH and FSH were moderately elevated, and the serum testosterone level was low. A subnormal response by testicular Leydig cells to hCG was observed. The LH and FSH responses to LRH were normal. A significant LH increase was observed after castration. Weekly administration of testosterone enanthate (250 mg) for 10 consecutive weeks caused a reduction (greater than 75%) in gonadotropins and abolishment of the LRH pituitary response. No differences were observed in terms of gonadotropin dynamics compared with other forms of hypergonadotropic hypogonadism. These results indicate that XX males exhibit hypergonadotropic hypogonadism secondary to testicular failure with a preserved androgen responsiveness of the hypothalamic-pituitary unit.