Testicular Endocrine Function in a Pubertal Boy with 3β-Hydroxysteroid Dehydrogenase Deficiency

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Abstract
Plasma pregnenolone, progesterone, 17-hydroxyprogesterone, testosterone and 5α-dihydrotestosterone were determined in the peripheral and spermatic venous blood of a pubertal boy with 3β-hydroxysteroid dehydrogenase deficiency in order to evaluate his testicular endocrine function. The peripheral testosterone concentration was 0.28 ng/ml, and rose only to 0.60 ng/ml in response to human chorionic gonadotropin (HCG) administration. The spermatic venous testosterone concentration, however, was about 15 times greater than in peripheral blood, which is a clear indication of testicular testosterone secretion in this patient. In addition, testicular secretion of 17-hydroxyprogesterone and 5α-dihydrotestosterone was also evident, thus, the testis has some 3β-hydroxysteroid dehydrogenase activity. In addition to the weak response to HCG administration, a very high spermatic venous pregnenolone concentration as compared to normal males and a slightly elevated plasma luteinizing hormone (LH) concentration suggest that the 3β-hydroxysteroid dehydrogenase activity in the testis tissue of this subject is subnormal.

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