GONADOTROPHINS, TESTOSTERONE AND OESTROGEN LEVELS IN RELATION TO OVARIAN MORPHOLOGY IN 11β-HYDROXYLASE DEFICIENCY

Abstract

Two siblings, aged 6 years 2 months and 4 years 2 months respectively, with the hypertensive form of congenital virilizing adrenal hyperplasia due to 11β-hydroxylase deficiency are presented. The blood levels of FSH were in the pubertal range and the LH levels were low. Testosterone-like-substances (TLS) were in the normal range for adult males; urinary oestrogens were increased and the bone ages were 15 and 13 years. Pubertal development had begun although suppression therapy was never started, as shown by the pubertal levels of FSH, and the rapid increase of both FSH and LH when the adrenal secretion was suppressed. Polycystic ovaries were observed in both cases. The role of testosterone and derivatives as well as of FSH in the development of polycystic ovaries is discussed.