Background: Bone mineral density (BMD) is unknown in children with Prader-Willi syndrome (PWS), but is decreased in adults with PWS. In patients with GH deficiency, BMD increases during GH treatment. Objectives: The aim of the study was to evaluateBMDin children with PWS and to study the effects of GH treatment. Design: We conducted a randomized controlled GH trial. Forty-six prepubertal children were randomized into either a GH-treated group (1.0 mg/m2· d) or a control group for 2 yr. At start, 6, 12, and 24 months of study, total body and lumbar spine BMD were measured by dual-energy x-ray absorptiometry, and lumbar spine bone mineral apparent density (BMAD) was calculated. Results: Base line total body and lumbar spine BMDSD score (SDS) were normal [mean(SD),-0.2SDS(1.1) and -0.4 SDS (1.2), respectively]. BMADSDS, which corrects for short stature, was also normal [mean (SD), 0.40 SDS (1.1)]. Total body BMDSDS decreased during the first 6 months of GH (P < 0.0001), but increased during the second year of treatment. After 24 months of study, total body and lumbar spine BMDSDS, and the BMADSDS did not significantly differ between GH-treated children and randomized controls (P = 0.30, P = 0.44, and P = 0.47, respectively). Results were similar when corrected for body mass index SDS. Repeated measurements analysis showed a significant positive association between IGF-I SDS and total body and lumbar spine BMDSDS, but not with BMADSDS. Conclusions: Our results show that prepubertal children with PWS have a normal BMD. GH treatment had no effect on BMD, except for a temporary decrease of total body BMDSDS in the first 6 months. Copyright

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Persistent URL dx.doi.org/10.1210/jc.2009-0270, hdl.handle.net/1765/25376
Citation
de Lind van Wijngaarden, R.F.A, Festen, D.A.M, Otten, B.J, van Mil, E.G.A.H, Rotteveel, J, Odink, R.J.H, … Hokken-Koelega, A.C.S. (2009). Bone mineral density and effects of growth hormone treatment in prepubertal children with Prader-Willi syndrome: A randomized controlled trial. Journal of Clinical Endocrinology and Metabolism, 94(10), 3763–3771. doi:10.1210/jc.2009-0270