Author(s): Carrel AL, Allen DB
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Abstract Children with Prader-Willi syndrome (PWS) display diminished growth, reduced muscle mass (lean body mass), and increased adipose tissue-body composition abnormalities resembling those seen in growth hormone (GH) deficiency. Diminished GH responses to various provocative agents, low insulin-like growth factor-I levels, and the presence of other hypothalamic dysfunction support the presence of true GH deficiency (GHD) in many children with PWS. GH treatment in these children decreases body fat, and increases linear growth, muscle mass, fat utilization and energy expenditure. Strength and agility are also improved. These improvements are most dramatic during the first year of GH therapy, and prolonged treatment still does not 'normalize' these parameters. The metabolic effects, including changes in physical strength and agility, may be the most important features for this particular pediatric population. These observations support a contribution of GHD to disabilities of children with PWS, and a clinically significant benefit of GH treatment.
This article was published in J Pediatr Endocrinol Metab
and referenced in Journal of Nutrition & Food Sciences