Abstract
Children with Prader-Willi syndrome (PWS) display impaired growth, reduced lean body mass, and increased fat mass resembling body composition abnormalities observed in growth hormone-deficient (GHD) patients. Diminished growth hormone (GH) responses to provocative stimuli, relatively low insulin-like growth factor 1 levels, excessive curtailing of growth during caloric restriction, and the presence of other hypothalamic abnormalities also support the presence of true GHD in PWS. GH treatment of children with PWS results in normalization of linear growth, decreased adipose tissue, and increased muscle and bone mass, fat oxidation, and energy expenditure. Significant improvements in strength, agility, and respiratory muscle function in GH-treated versus control PWS subjects also occur. Response to GH is reduced after the first year of therapy and prolonged treatment of older children and adolescents with PWS sustains fat losses and promotes additional lean body mass accretion, but does not normalize body composition or physical function. These observations support a contribution of true GHD to disabilities of PWS and a clinically significant benefit of GH treatment in PWS patients and provide impetus for further study of this question.
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