cyproterone and Fibrous-Dysplasia-of-Bone

We have studied the endocrinological pattern in a girl with McCune-Albright syndrome. The young patient showed: normal prepubertal serum levels of gonadotropins, fluctuating estrogen concentrations, which sometimes were similar to the levels in adult women of fertile age, hyperprolactinemia with galactorrhea, ovarian cysts. The effects of treatment with antiandrogen drug, cyproterone acetate, and of a LHRH agonist, buserelin (less than D-Ser[TBU(8)6-des-gly NH2.10 greater than LHRH ethylamide), were studied. Cyproterone acetate with or without buserelin did not fully suppress estradiol concentrations. On the other hand, surgical resection of these cysts resulted in both clinical and endocrinological remission. It is likely that in this case of McCune-Albright syndrome precocious puberty was a result of ovarian estrogen secretion, while pubertal activation of the hypothalamus-pituitary axis was absent. Hyperprolactinemia, which appeared after the beginning of the combined therapy with buserelin and cyproterone acetate, was probably due to the elevated estrogen levels.

Topics: Buserelin; Child, Preschool; Cyproterone; Cyproterone Acetate; Estradiol; Estrogens; Female; Fibrous Dysplasia of Bone; Fibrous Dysplasia, Polyostotic; Follicle Stimulating Hormone; Galactorrhea; Gonadotropin-Releasing Hormone; Humans; Lactation Disorders; Luteinizing Hormone; Ovary; Prolactin; Puberty, Precocious

In a 6 1/2-month-old girl with McCune-Albright syndrome, gonadotropin-independent isosexual precocity and recurrent ovarian cysts, the short-term effects of surgical therapy, cyproterone acetate (120 mg/m2/d), combined medroxyprogesterone acetate (10 mg/d), and spironolactone (50-75 mg/d) treatment, and testolactone (40 mg/kg/d) were evaluated sequentially. No significant reduction of cyst frequency was achieved with any of the medical treatments. The rate of bone maturation (delta BA/delta CA) was increased and the height standard deviation score (SDS) for bone age as a potential indicator of final height was decreased with surgical treatment alone and combined medroxyprogesterone acetate and spironolactone. Both parameters normalized with cyproterone acetate and testolactone. Height velocity SDS, however, was higher with testolactone (0.97 vs. 0.45).

Topics: Androgen Antagonists; Body Height; Bone Development; Combined Modality Therapy; Cyproterone; Cyproterone Acetate; Drug Therapy, Combination; Female; Fibrous Dysplasia of Bone; Fibrous Dysplasia, Polyostotic; Follow-Up Studies; Humans; Infant; Medroxyprogesterone; Medroxyprogesterone Acetate; Ovarian Cysts; Ovarian Function Tests; Spironolactone; Testolactone