Effects on Growth and Metabolism of Growth Hormone Treatment for 3 Years in 36 Children with Prader-Willi Syndrome
| Autor: | Ana Colmenares, Michel Polak, G. Pinto, Christine Trivin, P Taupin, A Giuseppe, T Odent, K. Laborde, Jean-Claude Souberbielle |
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| Rok vydání: | 2010 |
| Předmět: |
Male
congenital hereditary and neonatal diseases and abnormalities Pediatrics medicine.medical_specialty Endocrinology Diabetes and Metabolism Osteoporosis Scoliosis Biology Short stature Cohort Studies Endocrinology Insulin resistance Bone Density Diabetes mellitus Internal medicine medicine Humans Prospective Studies Insulin-Like Growth Factor I Child Human Growth Hormone Genetic disorder nutritional and metabolic diseases Lipid Metabolism medicine.disease Obesity nervous system diseases Growth hormone treatment Pediatrics Perinatology and Child Health Body Composition Carbohydrate Metabolism Female Insulin Resistance medicine.symptom Prader-Willi Syndrome |
| Zdroj: | Hormone Research in Paediatrics. 75:123-130 |
| ISSN: | 1663-2826 1663-2818 |
| Popis: | Background/Aims: Prader-Willi syndrome (PWS) is a complex genetic disorder whose many manifestations include obesity and short stature. Diabetes, osteoporosis, and scoliosis are common. We evaluated the effects of human growth hormone (hGH). Methods: A prospective cohort study of 36 children (1–15 years of age) with genetically confir med PWS who were given hGH (mean dose 0.033 ± 0.006 mg/kg/day) for 36 months. At baseline and once yearly, we evaluated growth, insulin-like growth factor-1 (IGF-1), body composition, bone mineral density (BMD), glucose tolerance, serum lipids, and spinal radiographs. Results: Height gain over the 3-year period was 1.2 SD score. Lean body mass increased significantly during each treatment year. Total body fat decreased by 5.42 and 1.17% in the 1st and 2nd years, respectively. BMD remained unchanged during therapy. IGF-1 and homeostasis model assessment index of insulin resistance increased, and glucose intolerance was found in 22.7% of patients at baseline and 0% at 3 years. None of the patients had diabetes. Their lipid profile improved. Scoliosis was present in 27.8% of the patients at baseline and 47.2% at 3 years. Conclusion: GH treatment in children with PWS has multiple beneficial effects on growth and body composition. Tolerance is good, with an improvement in glucose metabolism, although IGF-1 levels and insulin resistance parameters should be monitored closely. The high rate of scoliosis warrants monitoring by a pediatric orthopedic surgeon. |
| Databáze: | OpenAIRE |
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