Abstract
A 4 year old male (46XY) pseudohermaphrodite with 170Hase deficiency (Ped.Res.13:387A,1979) underwent bilateral orchiectomy 2 days after 3 days of HCG (2,000 u/day IM), during which serum levels of testosterone, dihydrotestosterone,androstenedione, 170H-progesterone (170HP) and estradiol did not increase while progesterone increased from 52 to 69 ng/dl. Teased testicular tissue was incubated with 10uCi (3.3 nmol) of 3H-pregnenolone or 3H-170HP in 3 ml NADP fortified KRB buffer, pH 7.4, for 3 hr. at 37°C with and without 100 IU/ml HCG.
Thus the testes of this patient were unable to metabolize pregnenolone, but were able to metabolize 170HP. The data are consistent with deficiency of 170Hase activity.
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Vargas, A., Reiter, E., Kula, K. et al. 452 DIRECT DETERMINATION OF 17α-HYDROXYLASE (170Hase) DEFICIENCY IN A MALE PSEUDOHERMAPHRODITE BY IN VITRO STUDIES OF TESTICULAR STEROID BIOSYNTHESIS. Pediatr Res 15 (Suppl 4), 515 (1981). https://doi.org/10.1203/00006450-198104001-00464
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DOI: https://doi.org/10.1203/00006450-198104001-00464
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