Steroid Metabolism in the Syndrome of Testicular Feminization1

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Abstract
In a patient with the syndrome of testicular feminization, an evaluation of testicular function in vivo and in vitro was attempted. Urinary 17-ketosteroids were fractionated by gradient elution column chromatography. The effect of gonadotropic hormone preparations and of adrenocortical suppression on the steroid patterns was studied. It appeared that the testicular contribution to urinary dehydroepiandro-sterone, androsterone and etiocholanolone was important. Also, a mild anabolic effect was ovserved during treatment with human chorion-ic gonadotropin. The capacity of the testes to synthesize hormones in vitro was studied by incubation of homogenates with radioactive steroids. The synthesis of dehydroepiandrosterone, testosterone and [DELTA]4 androstenedione was demonstrated. These experiments revealed 2 biosynthetic pathways to testosterone, one from [DELTA]5-pregnenolone via 17-hydroxypregnenolone and dehydroepiandrosterone, and a 2nd via progesterone and 17-hydroxyprogesterone. The results are discussed in the light of the pathogenesis of this disorder and are compared with those observed in normal men and women.