Abstract
A patient is described with primary amenorrhoea, elevated urinary pregnanediol and pregnanetriol and diminished aldosterone excretion and with clinical signs of hypooestrogenism, but without signs of virilization. Sequential stimulation by large doses of human menopausal and chorionic gonadotrophins resulted in the formation of several Graafian follicles and fresh corpora lutea. These morphological changes were not accompanied by any clinical signs of an increased oestrogen secretion or by an increased urinary oestrogen excretion. Tracer studies revealed that the patient was capable of carrying out a 17α-hydroxylation, but not the removal of the steroid side-chain. It is concluded that ovulation and corpus luteum formation in women are not dependent on a concomitant oestrogen synthesis by the maturing follicle.
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