Current medical and surgical therapies of precocious puberty in McCune-Albright syndrome are often unsatisfactory. We used an aromatase inhibitor, testolactone, to treat precocious puberty in a girl with McCune-Albright syndrome. This child was unresponsive to 28 weeks of treatment with the long-acting agonist of LRH, D-trp6-pro9-NEt-LRH. During testolactone therapy, menses ceased, bone age advancement and height velocity diminished, and plasma oestradiol levels were suppressed. Serum gonadotrophin levels remained in the prepubertal range. Testolactone may be an effective therapy of precocious puberty in girls with McCune\x=req-\ Albright syndrome.Precocious puberty in girls with McCune-Albright syndrome is usually due to ovarian oestrogen secre¬ tion that is independent of pubertal hypothalamicpituitary activation. Previous attempts to treat the precocious puberty of McCune-Albright syndrome have met with limited success. Surgical wedge re¬ section of the ovaries or excision of ovarian cysts has resulted only in temporary remission of the symptoms of sexual precocity (Foster et al. 1984a; Scully & McNeely 1975). Agents that suppress pituitary secretion of gonadotrophins, such as the long-acting agonist of LRH, D-trp6-pro9-NEt-LRH, have also failed to achieve remission of menses and secondary sexual development (Foster et al. 1984b;Comité et al. 1981). We have treated a girl with McCune-Albright syndrome with the aromatase inhibitor, testolactone, which inhibits the conversion of androstenedione to oestrone and testoster¬ one to oestradiol (Segaloff et al. 1960). Testolac¬ tone treatment was associated with cessation of menses, decreases in height velocity and the rate of bone age advancement, and suppression of plasma oestradiol levels.
Materials and Methods
SubjectsThe patient, a 2 2/u year old girl, was the 2770 g product of a 24 year old G1 PO mother, who had an uncompli¬ cated pregnancy, labour, and delivery. The child devel¬ oped normally until she had an episode of vaginal bleeding at age 8 months. She continued to have periods every 4-6 weeks and was referred to the National Institutes of Health at the age of 15 months. There was no known exposure to oestrogens. The child's height was 82.5 cm (97th percentile). Height velocity between 11 and 15 months was 34.2 cm/year. (Height velocity of girls between 11 and 15 months is 13.5 ± 2.0 (SD) cm/year (Tanner 1965)). The weight was 10.6 kg (67th percen¬ tile), and the blood pressure was 90/60 mmHg. She had Tanner stage III breasts and no pubic hair. Her external genitalia were normal but her vaginal mucosa appeared oestrogenized and had a white mucoid discharge. The vaginal smear had 100% intermediate cells. There was no abnormal skin pigmentation. The remainder of the phy¬ sical examination was normal. Computed tomography of the head and ultrasonography of the adrenal glands showed no abnormalities. Pelvic ultrasound indicated a