Gonadotropin dynamics in XX males.

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.

Developmental, cognitive, and behavioral sequelae of child abuse.

There is growing interest in the neurologic, behavioral, and cognitive effects of child abuse and neglect. This article explores the literature on the short and long term sequelae of physically and sexually abused and neglected children, along with controversies generated by the studies themselves. Recommendations are made for swift and ongoing intervention in cases of child abuse to protect young victims from potentially devastating effects.

Ovarian development in 46,XY gonadal dysgenesis.

In humans the XY ovary is degenerative, there being scant evidence of persistence of that organ beyond the perinatal period. Here we describe indications of functional ovarian tissue in a 17-year-old female with male karyotype, H-Y+ cellular phenotype, and some signs of the Turner syndrome. Her gonads were removed after the onset of secondary amenorrhea. Histological examination revealed a degenerative right ovary devoid of germ cells and follicles, and a left streak gonad. There was no trace of testicular development in either side.