Abstract
Four patients with testicular feminizing syndrome and one patient with pure gonadal dysgenesis constitute the basis of this report. Urinary steroid analyses were performed in one of the patients prior to and after gonadectomy. The most striking feature was the high levels of urinary estrogens which decreased after gonadectomy, suggesting a gonadal origin of the estrogens. However, this could not be confirmed by the in vitro studies of these gonads. A homogenate of the testicular tissue was incubated with 4-C14-progesterone and 7-H3-pregnenolone. Accumulation of 17-OH-progesterone and dehydroepiandrosterone (DHEA) out of progesterone and pregnenolone, respectively, was obtained. Both progesterone and pregnenolone served as substrates for testosterone synthesis.
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