TY - JOUR
T1 - Bone Mineral Density and Effects of Growth Hormone Treatment in Prepubertal Children with Prader-Willi Syndrome
T2 - A Randomized Controlled Trial
AU - van Wijngaarden, Roderick F. A. de Lind
AU - Festen, Dederieke A. M.
AU - Otten, Barto J.
AU - van Mil, Edgar G. A. H.
AU - Rotteveel, Joost
AU - Odink, Roelof J.
AU - van Leeuwen, Mariette
AU - Haring, Danny A. J. P.
AU - Bocca, Gianni
AU - Houdijk, E. C. A. Mieke
AU - Hokken-Koelega, Anita C. S.
PY - 2009/10
Y1 - 2009/10
N2 - 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/m(2) . 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), -0.2SDS (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. (J Clin Endocrinol Metab 94: 3763-3771, 2009)
AB - 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/m(2) . 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), -0.2SDS (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. (J Clin Endocrinol Metab 94: 3763-3771, 2009)
KW - X-RAY ABSORPTIOMETRY
KW - BODY-MASS INDEX
KW - DIAGNOSTIC-CRITERIA
KW - SCOLIOSIS
KW - GH
KW - THERAPY
KW - METABOLISM
KW - INFANTS
KW - INSUFFICIENCY
KW - REPLACEMENT
U2 - 10.1210/jc.2009-0270
DO - 10.1210/jc.2009-0270
M3 - Article
SN - 0021-972X
VL - 94
SP - 3763
EP - 3771
JO - Journal of Clinical Endocrinology and Metabolism
JF - Journal of Clinical Endocrinology and Metabolism
IS - 10
ER -