Abstract
Precocious puberty (PP) in girls with Mc Cune-Albright syndrome has been attributed to autonomous ovarian function and premature activation of the hypothalamo-pituitary-ovarian axis. We report the clinical and hormonal findings in a 5 2/12 year old girl with Mc Cune-Albright syndrome, large ovarian cysts and PP. She initially presented with extremely elevated E2- (1473 pmol/1) and suppressed LH- (11 ng/ml) and FSH-levels below the detection limit. 8 months after initiation of cyproterone acetate therapy, an intravenous LHRH-test showed LH- (56 and 324 ng/ml, basal and stimulated) and FSH-levels (6.8 and 12.6 ng/ml) well in the pubertal range. 23 days later complete suppression of gonadotropins again was demonstrated. We suggest that the mechanism of PP in girls with Mc Cune-Albright syndrome might involve intermittent activation of the hypothalamo-pituitary axis followed by apparently autonomous ovarian function.
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