Virilizing Male Pseudohermaphroditism. Association with Abnormal Testicular Function1

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Abstract
Two siblings with the diagnosis of male pseudohermaphroditism have been studied. Gender role assignment in childhood was female, but marked virilization occurred at adolescence. Increased urinary 17-ketosteroid excretion was demonstrated, with exaggerated responses to adrenocorticotropin (ACTH) and chorionic gonadotropin (HCG). Following gonadectomy, urinary 17-ketosteroid excretion fell to normal levels with normal responses to ACTH stimulation but no responses to HCG stimulation. Studies of individual urinary ketosteroids by fractionation techniques demonstrated that androsterone and etiocholanolone responses to ACTH and HCG were similar prior to gonadectomy. No excessive excretion of dehydroepiandrosterone (DHEA) was found. Studies following gonadectomy indicate normal adrenal function. It was concluded that, in this syndrome of virilizing male pseudohermaphroditism, presence of the testes was responsible for the major proportion of the 17-ketosteroids excreted. In vitro incubation studies of testicular tissue from these patients demonstrated testosterone synthesis to be present, but conversion of pregnenolone to progesterone could not be demonstrated. The pathway of synthesis appeared to follow the route of pregnenolone, 17-hydroxypregnenolone, DHEA, androstenedione and testosterone.

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