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Coupaye M.,Pitie Salpetriere Hospital | Coupaye M.,Institute of Cardiology Metabolism and Nutrition | Coupaye M.,French Reference Center for Prader Willi Syndrome | Coupaye M.,Childrens Hospital | And 48 more authors.
Journal of Clinical Endocrinology and Metabolism | Year: 2013

Context: Children with Prader-Willi syndrome (PWS) who receive GH treatment have improved growth and body composition; however, data are lacking for adults when treatment is discontinued after completion of growth. Objectives: Our aim was to compare body composition and metabolic status in adults with PWS according to GH treatment in childhood and adolescence. Design: 64 adults (mean age: 25.4 years) with a genetic diagnosis of PWS were evaluated: 20 received GH in childhood (T), which had been discontinued at the time of this study, and 44 did not receive GH (C). Mean duration of treatment in the T group was 4.4 ± 2.7 years, age at baseline was 11.8 ± 2.7 years, mean time between the end of treatment and the current evaluation was 7.0 ± 4.4 years. Main Outcomes Measures: Dual-energy X-ray absorptiometry was used to assess body composition and fasting biological analyses evaluated metabolic status. Results (mean ± SD): Body mass index and percentage of fat mass were significantly lower in the T group (32.4 ± 10.3 vs 41.2 ± 11.1 kg/m2, P = 0.05 and 44.0 ± 9.6 vs 50.1 ± 7.2%, P = 0.02, respectively). Insulinemia and HOMA-IR in non-diabetic subjects were significantly lower in the T group (5.8 ± 5.9 vs 13.9 ± 11.6 μUI/ml, P = 0.03, and 1.6 ± 1.3 vs 2.7 ± 2.1, P = 0.04, respectively). Non-diabetic and diabetic subjects from the T group had a significantly lower HbA1c. Lipid profiles were similar between groups. Conclusions: GH treatment in childhood and adolescence is associated with significantly decreased bodymass index and improved body composition and metabolic status in adults with PWS at several years after discontinuing treatment. Copyright © 2013 by The Endocrine Society.

Lloret-Linares C.,Assistance Publique Hopitaux de Paris | Lloret-Linares C.,University of Paris Descartes | Faucher P.,French Reference Center for Prader Willi Syndrome | Faucher P.,CEA DAM Ile-de-France | And 23 more authors.
International Journal of Obesity | Year: 2013

Context:The care of patients with hypothalamic obesity is challenging.Objective:To compare body composition, basal metabolic rate (BMR) and metabolic outcomes of adults, with lesional or genetic hypothalamic obesity, with obese patients suffering from primary obesity, once matched for body mass index (BMI).Design and patients:Adults with hypothalamic obesity of genetic origin (Prader Willi syndrome (PWS)) or acquired hypothalamic damage (HD), such as craniopharygioma, were compared with obese control candidates awaiting bariatric surgery (C), with a BMI between 35 and 65 kg m - 2, and aged between 18 and 50 years.Main outcome measures:Body composition measured by whole-body dual-energy X-ray absorptiometry scanning, BMR using indirect calorimetry, hormonal and metabolic assessments.Results:A total of 27 adults with a genetic diagnosis of PWS, 15 obese subjects with HD and 206 obese controls with similar BMI were studied. Compared with the control group, PWS patients had an increased percentage of fat mass (FM), and a decreased percentage of android FM. The BMR of PWS patients was significantly lower than controls and highly correlated with lean body mass in PWS and C patients. Body composition of HD was similar with those of obese patients. A trend toward an increased prevalence of diabetes in HD patients and of cytolysis in PWS was observed in comparison with primary obese patients.Conclusion:Genetic and lesional hypothalamic obesities have different consequences for phenotypic features such as body composition or BMR compared with primary obese patients. The mechanisms of adipose tissue development and metabolic complications may be different between genetic and lesional obesities. © 2013 Macmillan Publishers Limited.

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