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Bone Mineral Density and Effects of Growth Hormone Treatment in Prepubertal Children with Prader-Willi Syndrome: A Randomized Controlled Trial

Dutch Growth Research Foundation (R.F.A.d.L.v.W., D.A.M.F., A.C.S.H.-K.), 3016 AH Rotterdam, The Netherlands; Department of Pediatrics (R.F.A.d.L.v.W., D.A.M.F., A.C.S.H.-K.), Subdivision of Endocrinology, Erasmus University Medical Center Rotterdam, Sophia Children’s Hospital, 3015 GJ Rotterdam, The Netherlands; Department of Pediatrics (B.J.O.), Subdivision of Endocrinology, Radboud University Nijmegen Medical Center, 6525 GA Nijmegen, The Netherlands; Department of Pediatrics (E.G.A.H.v.M.), Hieronymus Bosch Medical Center, 5211 NL ‘s-Hertogenbosch, The Netherlands; Department of Pediatrics (J.R.), Subdivision of Endocrinology, VU University Medical Center, 1081 HV Amsterdam, The Netherlands; Department of Pediatrics (R.J.O.), Catharina Hospital, 5623 EJ Eindhoven, The Netherlands; Department of Pediatrics (M.v.L.), St. Jansdal Hospital, 3844 DG Harderwijk, The Netherlands; Department of Pediatrics (D.A.J.P.H.), Diaconessenhuis, 2334 CK Leiden, The Netherlands; Department of Pediatrics (G.B.), Subdivision of Endocrinology, University Medical Center Groningen/Beatrix Children’s Hospital, 9713 GZ Groningen, The Netherlands; and Department of Pediatrics (E.C.A.M.H.), Subdivision of Endocrinology, Haga Hospitals/Juliana Children’s Hospital, 2566 MJ The Hague, The Netherlands

Address all correspondence and requests for reprints to: Roderick de Lind van Wijngaarden, Clinical Research Fellow, Dutch Growth Research Foundation, Erasmus University Medical Center/Sophia Children’s Hospital, Westzeedijk 106, 3016 AH Rotterdam, The Netherlands. E-mail: r.delindvanwijngaarden{at}erasmusmc.nl.

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 evaluate BMD in 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: Baseline total body and lumbar spine BMD SD score (SDS) were normal [mean (SD), –0.2 SDS (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.