Evaluation of the hypothalamic-pituitary function in a female with hypogonadotropic hypogonadism and anosmia

A case of Kallmann's syndrome in a woman is presented to evaluate the hypothalamic-pituitary function and status of the genital organs. The diagnosis of Kallmann's syndrome was performed under the determination of low circulating level of estradiol-17 beta and gonadotropins and low sensitivity to an olfactometer. Pituitary response to LRH was observed. Plasma LH response to LRH was observed in a lesser degree compared with the less impaired response of FSH. Daily injection of LRH for 13 days increased pituitary response of gonadotropin secretion. Diminished prolactin secretion and the dissociation of response to TRH and chlorpromazine were shown. Laparoscopic findings revealed a hypoplastic uterus and nearly normal sized ovaries. Ovarian response to exogenous gonadotropins was affirmed by increased estradiol-17 beta level. These results indicated disturbed hypothalamic control of gonadotropins and prolactin, and a reserved pituitary function in this case. Low circulating level of estradiol-17 beta might affect the heterogenous response of the hypothalamic-pituitary axis.