Body composition, endocrine and metabolic profiles in adults with Prader-Willi syndrome

• Published in: Growth hormone & IGF research Vol. 20; no. 3; pp. 179 - 184
• Main Authors: Sode-Carlsen, Rasmus, Farholt, Stense, Rabben, Kai Fr, Bollerslev, Jens, Schreiner, Thomas, Jurik, Anne Grethe, Christiansen, Jens Sandahl, Höybye, Charlotte
• Format: Journal Article
• Language: English
• Published: Scotland Elsevier Ltd 01-06-2010
• Subjects: Adolescent; Adult; Adults; Advanced Basic Science; Body composition; Body Composition - drug effects; Body Composition - physiology; Body Weights and Measures; Endocrine System - drug effects; Endocrine System - metabolism; Endocrine System - physiology; Endocrinology & Metabolism; Female; Human Growth Hormone - pharmacology; Humans; Hypogonadism - complications; Hypogonadism - metabolism; Hypogonadism - physiopathology; Intra-Abdominal Fat - drug effects; Intra-Abdominal Fat - physiology; Lipids - blood; Male; Medicin och hälsovetenskap; Metabolism; Metabolome - drug effects; Metabolome - physiology; Middle Aged; Prader-Willi Syndrome - complications; Prader-Willi Syndrome - metabolism; Prader-Willi Syndrome - physiopathology; PWS; Subcutaneous Fat - drug effects; Subcutaneous Fat - physiology; Young Adult; Adults; Metabolism; PWS; Body composition
• ISSN: 1096-6374; 1532-2238; 1532-2238
• DOI: 10.1016/j.ghir.2009.12.004

Abstract Objective Prader-Willi syndrome (PWS) is a complex genetic disease associated with hypothalamic-pituitary dysfunction and severe obesity. The aim of the present study was to describe the relationships between body composition, metabolic and hormonal profiles in PWS adults. Method Forty six adults with genetically verified PWS, 25 women and 21 men, median age 28 years were studied. Body composition was evaluated by standard anthropometric procedures and with computed tomography (CT) of the abdomen and at the mid-femur level. CT of abdomen was compared to 22 healthy, unmatched adults. Circulating lipids were measured and oral glucose tolerance test (OGTT) and hormonal screening including GH secretory capacity (GHRH/arginine test) was carried out. Results Median body mass index (BMI) was 27.2 kg/m2 , with women being more obese than men. Sixteen patients had dyslipidaemia, 10 impaired glucose tolerance and seven had diabetes. Fifty percent were hypogonadal and six fulfilled BMI related criteria for growth hormone deficiency (GHD). Visceral to subcutaneous abdominal fat ratio was reduced in PWS. Visceral abdominal fat fraction correlated with both subcutaneous fat, BMI and peak GH-response. Thigh muscle volume was about half of the thigh fat volume. Beneficial effects of sex-steroid replacement on body composition were not observed. Conclusions Body fat was primarily located subcutaneously and metabolic consequences of obesity limited. The abnormal body composition similar to that in non-PWS GHD adults increases the interest of GH treatment in the prevention of obesity in adults with PWS.