Effect of Genotype and Previous GH Treatment on Adiposity in Adults With Prader-Willi Syndrome
| Autor: | Jean-Michel Oppert, Eric Bieth, Laurence Cuisset, Jean-Marc Lacorte, Virginie Laurier, Muriel Coupaye, Maithé Tauber, Karine Clément, Christine Poitou |
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| Rok vydání: | 2016 |
| Předmět: |
Adult
Male 0301 basic medicine congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Adolescent Genotype Endocrinology Diabetes and Metabolism Clinical Biochemistry 030209 endocrinology & metabolism Context (language use) Biology Biochemistry Body Mass Index Cohort Studies Young Adult 03 medical and health sciences chemistry.chemical_compound Absorptiometry Photon 0302 clinical medicine Endocrinology Internal medicine Adipocyte Adipocytes medicine Humans Resting energy expenditure Adiposity Chromosomes Human Pair 15 Biochemistry (medical) nutritional and metabolic diseases Middle Aged Uniparental Disomy medicine.disease Obesity Uniparental disomy Phenotype 030104 developmental biology chemistry Growth Hormone Female Ghrelin Chromosome Deletion Prader-Willi Syndrome Body mass index |
| Zdroj: | The Journal of Clinical Endocrinology & Metabolism. 101:4895-4903 |
| ISSN: | 1945-7197 0021-972X |
| Popis: | Adults with Prader-Willi syndrome (PWS) have an increased proportion of sc fat mass compared with body mass index (BMI)-matched controls, but whether the genotype influences body composition and metabolic profile remains controversial.To assess body composition and metabolic features in adults with PWS, according to genetic subtype. In addition, the effect of previous GH treatment was assessed. Main Outcomes and Measures: Body composition (Dual Energy X-ray Absorptiometry) and metabolic parameters were compared in PWS adults (mean age, 25.5 ± 8.9 y) with deletion (n = 47) or uniparental disomy (UPD) (n = 26), taking into account GH treatment in childhood and/or adolescence. In subgroups, adipocyte size, fasting total ghrelin levels, and resting energy expenditure were measured, and hyperphagia was assessed by the Dykens Hyperphagia Questionnaire.Body composition (Dual Energy X-ray Absorptiometry) and metabolic parameters were compared in PWS adults (mean age, 25.5 ± 8.9 y) with deletion (n = 47) or uniparental disomy (UPD) (n = 26), taking into account GH treatment in childhood and/or adolescence. In subgroups, adipocyte size, fasting total ghrelin levels, and resting energy expenditure were measured, and hyperphagia was assessed by the Dykens Hyperphagia Questionnaire.In the whole sample, the deletion group had a higher BMI compared with UPD (40.9 ± 11.5 vs 34.6 ± 9.6 kg/mA deletion genotype in adults with PWS is associated with increased BMI. GH treatment in childhood and/or adolescence limits this deleterious phenotypic effect with improved adiposity markers. This study suggests relationships between the molecular phenotype of PWS and adipose tissue development as well as sensitivity to GH. |
| Databáze: | OpenAIRE |
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