Changes in adrenocortical function of patients with gonadal dysgenesis after treatment with estrogen

The urinary excretion of pregnanetriol and δ⁵-pregnenetriol (as glucuronides) was measured in 5 untreated patients with gonadal dysgenesis before and after 6 weeks of treatment with stilbestrol (2 mg daily). In all the patients the ratio pregnenetriol/pregnanetriol increased (2 to more than 30 times) after estrogen. Three of the patients had undetectable or very low pregnenetriol excretions and low plasma concentrations of estradiol (35-80 pg/ml) before estrogen treatment. In the other 2 patients who had significant excretion of pregnenetriol in urine before the administration of estrogen the values of estradiol in blood were 150 and 410 pg/ml. These results suggest that both endogenous and exogenous estrogen induce a relative blockade in adrenal 3β-hydroxysteroid dehydrogenase in humans, a phenomenon known to occur in rats. Some of the implications of this finding related to adrenal changes in the newborn period, puberty and polycystic ovarian disease are discussed.