RÉSUMÉ
Body composition and leptin were studied in 13 young, still underweight and 10 older overweight children with Prader-Labhart-Willi syndrome. Not only the older overweight children but also the young underweight children had elevated skinfold standard deviation scores for body mass index and elevated body mass index adjusted leptin levels, suggesting relatively increased body fat despite underweight. Our data indicate that body composition in Prader-Labhart-Willi syndrome is disturbed already in infancy, long before the onset of obesity. Leptin production appears to be intact.
Sujet(s)
Tissu adipeux , Composition corporelle , Obésité/complications , Syndrome de Prader-Willi/physiopathologie , Protéines/métabolisme , Indice de masse corporelle , Enfant , Enfant d'âge préscolaire , Croissance , Humains , Nourrisson , Leptine , Obésité/métabolisme , Syndrome de Prader-Willi/complications , Épaisseur du pli cutanéSujet(s)
Substances de croissance/sang , Facteur de croissance IGF-I/analyse , Anticorps/analyse , Classification , Substances de croissance/immunologie , Humains , Facteur de croissance IGF-I/biosynthèse , Maladies du foie/complications , Troubles nutritionnels/complications , Récepteur STH/analyse , Récepteur STH/immunologie , Transduction du signal , Syndrome , Terminologie comme sujetRÉSUMÉ
Because patients with uremia have evidence for growth hormone resistance, we investigated whether this resistance can be overcome by administration of recombinant human growth hormone in supraphysiologic doses in children with severe uremia. Nine stunted children with end-stage renal disease (median age 5.8 years, median bone age 2.7 years) were treated with recombinant human growth hormone, 4 IU/m2/day subcutaneously, for a period of 1 year. Median height velocity was increased from 4.4 cm/yr before therapy to 8.0 cm/yr during treatment. Negative values for height velocity standard deviation scores for chronologic age were improved from a median of -2.6 to +1.5 without advancing bone age more than chronologic age. The growth hormone-insulin-like growth factor I resistance may be explained in part by the increased serum concentration of the high molecular weight insulin-like growth factor binding protein despite normal insulin-like growth factor I serum concentration. Treatment with recombinant human growth hormone improved the ratio between the serum concentrations of insulin-like growth factor I and its binding protein, and normalized the somatomedin bioactivity in the growth cartilage bioassay.