ENDOCRINE STUDIES AND SUCCESSFUL TREATMENT IN A PATIENT WITH TRUE HERMAPHRODITISM

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Abstract
A 12 yr old, 46 XX true hermaphrodite born with genital ambiguity was studied and successfully treated. The serum LH and FSH [lutropin and follitropin] profile resembled that of a pubertal normal individual and LH-RH [luliberin] administration induced a normal LH response. Baseline testosterone serum levels were within the range for normal children. Exogenous hCG [human chorionic gonadotropin] stimulation induced a significant serum testosterone increase up to values similar to those observed in normal post-pubertal males. Surgical examination disclosed the presence of bilateral ovotestis, normal Mullerian derivatives, epididymis and vas deferens. A complete ovotestis with testicular predominance, the testicular portion of the contralateral ovotestis and the Wolffian derivatives were removed. Further hCG stimulation 3 mo. after surgery failed to induce a serum testosterone increase. Spontaneous menarche was observed 6 mo. after surgery and ovulation was well documented. At present the patient has several characteristics of the female sex including those of chromosome complement, gonad, internal and external genitalia, hormone levels and gender identity, demonstrating that treatment was successful and that reproductive function could be obtained. The finding of spontaneous ovulation following removal of the testicular portion suggests normal cyclic gonadotropic release, implying a difference between animal models and man in regard to hypothalamic virilization.