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ENDOCRINE STUDIES IN A CASE OF MALE PSEUDOHERMAPHRODISM: TESTICULAR FEMINIZATION

Sections of Medicine, Biochemistry and Physiology, Mayo Clinic and Mayo Foundation Rochester, Minnesota^*

Adrcnocortical and gonadal functions were studied in a 24-year-old patient with testicular feminization. The control values for plasma 17-hydroxycorticosteroids, urinary formaldehydrogenic steroids and urinary pregnanetriol were normal. The urinary excretion of 17-ketosteroids and pituitary gonadotropins fluctuated at the upper range of normal for males, while the urinary excretion of estrogens was high for a male of comparable age. Fractionation of the urinary 17-ketosteroids revealed normal quantities of etiocholanolone, androsterone, and 11-oxygenated 17-ketosteroids, and only small quantities of dchydroepiandrosterone. The administration of ACTH resulted in the usual increase in the level of plasma 17-hydroxycorticosteroids and all urinary steroids except dchydroepiandrosterone. Administration of dexamethasone resulted in a fall in the level of plasma 17-hydroxycorticosteroids to nearly zero, but the urinary excretion of 17-ketosteroids remained appreciable and averaged 12.5 mg. per twenty-four hours. The administration of chorionic gonadotropin, while endogenous secretion of ACTH was inhibited by dexamethasone, resulted in increased urinary excretion of 17-ketosteroids, pregnanetriol and etiocholanolone. There was a slight increase in the excretion of androsterone and 11-oxygenated 17-ketosteroids. Following orchiectomy, urinary 17-ketosteroids and estrogens virtually disappeared. It is concluded that the patient’s gonads were the source of appreciable quantities of estrogens and 17-ketosteroids and small quantities of pregnanetriol.

^* The Mayo Foundation, Rochester, Minnesota, is a part of the Graduate School of the University of Minnesota.

Received September 15, 1960.