Journal of pediatric endocrinology & metabolism : JPEM

Treatment of central precocious puberty and early puberty with GnRH analog in girls with Williams-Beuren syndrome.

PMID 26197460


Little has been published on treatment of precocious puberty in girls with Williams-Beuren syndrome (WBS), a condition occurring frequently in this group. We analyzed our own data on growth/age at menarche of now adult female patients with WBS being diagnosed with central precocious puberty or early puberty. Data of patients treated with gonadotropin-releasing hormone (GnRH) analog (n=13) were compared with those not treated (control group, n=11). Longitudinal data on the somatic development of 24 now adult female patients were analyzed. Medium final height was 157.2±6.5 cm compared to 151.4±5.6 cm in the control group. No significant difference could be found in the discrepancy of genetic target height and final height. Prepubertally girls were normal weight in both groups; in adulthood the majority of patients were overweight/obese. Menarche commenced 11 months after cessation of therapy. As already known from other studies, hormonal suppression via GnRH analog was well tolerated.