Growth hormone treatment for osteoporosis in patients with scoliosis of Prader-Willi syndrome

Abstract

Background Patients with Prader-Willi syndrome (PWS) have fragile bones. Osteoporosis is a major concern in scoliosis surgery. Our aim was to investigate bone mineral density (BMD) in PWS patients and to verify the efficacy of and scoliosis deterioration with growth hormone (GH) administration for osteoporosis.Methods We followed 148 PWS patients who underwent lumbar spine (L2–4) BMD testing. Sixty-four patients had scoliosis, and 84 were non-scoliosis patients. Patients were treated with GH (0.245 mg/kg/week) until they reached a skeletal age of 17 years for males and 15 years for females. We also evaluated the effect of GH treatment on BMD in 101 patients (60 males, 41 females) undergoing BMD testing more than twice. The mean patient age was 5.4 years. The mean duration of GH administration was 54 months.Results Mean lumbar BMD was 0.567 g/cm2. Fifty patients (33.8 %) had osteoporosis and 41 (27.7 %) had osteopenia. There was no significant difference in mean BMD between patients with scoliosis (0.598 g/cm2) and without scoliosis (0.548 g/cm2). GH treatment caused a significant increase in Z score (pre-GH: mean −2.28 vs. post-GH: mean −1.53, P < 0.001). There was no statistical difference in the prevalence of scoliosis between the GH treatment group (45/112, 40.1 %) and non-treatment group (19/36, 52.8 %).Conclusions Among patients with PWS, 61.5 % had low BMDs. GH administration significantly improved the lumbar BMD (Z score). There were no statistically significant differences in the prevalence of scoliosis among patients who received GH treatment compared to patients who did not.