GnRH analogues have been used with variable success for the treatment of
precocious puberty in children with
McCune-Albright syndrome (MAS). In general, children with a bone age of less than 13.5 yr have been reported not to have benefitted from
GnRH therapy. In contrast, we have successfully treated a young girl with MAS and--probably secondary--
central precocious puberty using
Decapeptyl, a long acting
GnRH analogue. The girl with MAS presented at the age of six years with café au lait spots, osseous lesions and
precocious puberty. At initial presentation height was 130.7 cm (> 97 percentile), weight 27.5 kg (> 97 percentile), Tanner stage B3, PH3. Bone age was 11 yr. Magnetic resonance imaging of the brain was normal. Endocrine function tests were normal with the exception of biochemical evidence of
central precocious puberty:
LHRH test: LH 0.9/20.3, FSH 4.3/12.7 (mU/ml), E2 15.6 pg/ml.
Therapy was started with 3.75 mg
GnRH analogue i.m. every four weeks and was intensified two years after the beginning of
therapy to 3.75 mg i.m. every three weeks. Three years after the start of treatment bone age was 12 yr and growth velocity was 2.5 cm/year. Tanner stage was B3, PH3 and
LHRH testing revealed biochemical evidence for suppression of
gonadotropins: LH < 0.5/1.0, FSH 1.9/2.5 (mU/ml). We hypothesize that a subgroup of patients with MAS might present with a central form of
precocious puberty. This may be particularly so in children with a bone age greater than or equal to 11 yr.
Central precocious puberty in these children might follow extensive sex
steroid exposure due to the peripheral
precocious puberty induced by the activating mutation of the Gs
protein gene. This central form of
precocious puberty responds to
therapy with
GnRH analogues.