Prader-Willi Syndrome as a Model for Obesity: International Symposium, Zurich, October 18-19, 2002Urs Eiholzer, Dagmar l'Allemand Almost fifty years ago, in 1956, three researchers of the University of Zurich, Andrea Prader, Alexis Labhart and Heinrich Willi, first described what is now called the Prader-Willi Syndrome (PWS). The study and the therapy of this syndrome have progressed so rapidly in the past years that the decision was made to share recent advances with the scientific community and to address topics of future research at an international meeting. The results of this meeting are presented in this book. PWS results from a paternally derived deletion or an imprinting defect on chromosome 15. During their first two years of life patients with PWS suffer from muscle weakness, feeding problems and developmental delay. From the age of two years onwards they develop an almost insatiable appetite and suffer from obesity, short stature, hypogonadism and behavior problems. As it is becoming increasingly obvious that PWS is a multisystemic disorder, improving the quality of life of patients and their families requires broad professional support. While growth hormone therapy influences growth and body composition, many other problems such as insufficient satiation, hypoactivity, behavioral difficulties, speech problems and mental retardation remain to be addressed. Parents need psychological support in their daily battle against the eating disorder and the behavioral problems of their child. A comprehensive team approach will yield the best results for both patients and their parents. PWS research may also contribute to basic medical research by providing new insights into the metabolism of obese patients, whose obesity is caused by factors other than PWS. In this way, PWS may be used as a model for obesity. |
Contents
Introduction | 1 |
Central Nervous System and Body Weight Homeostasis | 7 |
Energy Balance in PraderWilli Syndrome Compared to Simple Obesity | 49 |
Comorbidities or Fundamental Defects of Obesity | 86 |
Comprehensive Treatment Approaches | 179 |
Epilogue | 228 |
232 | |
233 | |
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Common terms and phrases
abnormalities adipocytes adipose tissue adolescents AGRP appetite Basel behavior body composition body fat body mass body weight C-peptide child children with Prader-Willi children with PWS cholecystokinin Clin Endocrinol Metab Clin Nutr clinical correlated decreased defect diabetes disorder eating effects of GH Eiholzer energy expenditure energy intake families fat mass food intake genes genetic GH deficiency GH therapy GH treatment ghrelin glucose GnRH gonadotropin growth hormone growth hormone treatment human hyperphagia hypogonadism hypothalamic dysfunction hypotonia increased individuals insulin resistance Karger l’Allemand leptin levels Lindgren meal melanocortin metabolic Model for Obesity neurons neuropeptide non-PWS normal weight obese children obese controls obesity in PWS patients with PWS Pediatr peptide peripheral phenotype physical activity plasma POMC Prader Prader-Willi syndrome Prader-Willi syndrome PWS protein puberty PWS group PWS patients PWS subjects reduced regulation response satiety secretion signals simple obesity sleep apnea studies subjects with PWS Zipf WB eds