PITUITARY-OVARIAN RESPONSES TO LEUPROLIDE ACETATE TESTING IN PATIENTSWITH CONGENITAL ADRENAL-HYPERPLASIA DUE TO 21-HYDROXYLASE DEFICIENCY

To assess whether patients with congenital adrenal hyperplasia (CAH) d ue to 21-hydroxylase deficiency exhibit a steroidogenic response to Gn RH agonist consistent with functional ovarian hyperandrogenism (FOH) a nd elucidate the relationship between adrenal and ovarian hyperandroge nism, the LH, FSH, estradiol, 17-hydroxyprogesterone (17-OHP), androst enedione, total testosterone, dehydroepiandrosterone, and 17-hydroxypr egnenolone responses to a sc dose of leuprolide acetate (500 mu g) wer e evaluated in 10 patients with classic CAH (mean age, 18.4 +/- 0.95 y r), 7 of whom had oligomenorrhea, pretreated with dexamethasone (2 mg/ day for 5 days, including the day of the test). The results were compa red with those obtained in 11 patients with FOH (mean age, 18.7 +/- 0. 46 yr) and 17 normal women (mean age, 19.68 +/- 0.59 yr) not pretreate d with dexamethasone. Leuprolide acetate stimulation caused a signific ant augmentation of plasma E(2), 17-OHP, androstenedione, testosterone , and 17-hydroxypregnenolone concentrations in all CAH patients. Howev er, in only 6 (60%) of them, all with oligomenorrhea, was the 17-OHP r esponse (posttest minus pretest value) similar to that of FOH patients and significantly higher than that in controls. In this subset of CAH patients, LH plasma levels after stimulation were significantly highe r than those of CAH subjects with 17-OHP responses in the normal range , controls, and FOH patients, whereas FSH levels were similar to those of controls. In this latter group, plasma FSH concentrations after st imulation were significantly higher than those in FOH. In conclusion, the results of the present study indicate that LH-dependent functional ovarian hyperandrogenism is frequent in patients with classic CAH. As ovarian hyperandrogenism might be partially responsible for the menst rual irregularities that are common complications in such patients, al l classic CAH patients with oligomenorrhea should undergo short term s timulation with GnRH agonists to ascertain the presence of ovarian hyp erandrogenism and receive appropriate treatment.