Energy Metabolism Profile in Individuals with Prader-Willi Syndrome and Implications for Clinical Management: A Systematic Review.

Prader-Willi syndrome (PWS) is a rare genetic disorder associated with excessive weight gain. Hyperphagia associated with PWS may result in higher energy intake, but alterations in energy expenditure may also contribute to energy imbalance. The purpose of this critical literature review is to determine the presence of alterations in energy expenditure in individuals with PWS. Ten studies that measured total energy expenditure (TEE), resting energy expenditure (REE), sleep energy expenditure (SEE), activity energy expenditure (AEE), and diet induced thermogenesis (DIT) were included in this review. The studies provided evidence that absolute TEE, REE, SEE, and AEE are lower in individuals with PWS than in age-, sex-, and body mass index-matched individuals without the syndrome. Alterations in lean body mass and lower physical activity amounts appear to be responsible for the lower energy expenditure in PWS rather than metabolic differences. Regardless of the underlying mechanism for lower TEE, the estimation of energy requirements with the use of equations derived for the general population would result in weight gain in individuals with PWS. The determination of energy requirements for weight management in individuals with PWS requires a more comprehensive understanding of energy metabolism. Future studies should aim to comprehensively profile all specific components of energy expenditure in individuals with PWS with the use of appropriately matched controls and gold standard methods to measure energy metabolism and body composition. One component of energy expenditure that is yet to be explored in detail in PWS is DIT. A reduced DIT (despite differences in fat free mass), secondary to hormonal dysregulation, may be present in PWS individuals, leading to a reduced overall energy expenditure. Further research exploring DIT in PWS needs to be conducted. Dietary energy recommendations for weight management in PWS have not yet been clearly established.

[1]  A. Crinò,et al.  Obesity management in Prader–Willi syndrome: current perspectives , 2018, Diabetes, metabolic syndrome and obesity : targets and therapy.

[2]  A. Haqq,et al.  Hormonal and Metabolic Responses to a Single Bout of Resistance Exercise in Prader-Willi Syndrome
 , 2017, Hormone Research in Paediatrics.

[3]  Mario Siervo,et al.  Is Obesity Associated with Altered Energy Expenditure? , 2016, Advances in nutrition.

[4]  A. Haqq,et al.  A review of chemosensory perceptions, food preferences and food-related behaviours in subjects with Prader–Willi Syndrome , 2016, Appetite.

[5]  G. Bedogni,et al.  Assessment of fat-free mass from bioelectrical impedance analysis in obese women with Prader-Willi syndrome , 2015, Annals of human biology.

[6]  M. Gray,et al.  Breakfasts Higher in Protein Increase Postprandial Energy Expenditure, Increase Fat Oxidation, and Reduce Hunger in Overweight Children from 8 to 12 Years of Age. , 2015, The Journal of nutrition.

[7]  W. Colmers,et al.  Progressive postnatal decline in leptin sensitivity of arcuate hypothalamic neurons in the Magel2-null mouse model of Prader-Willi syndrome. , 2015, Human molecular genetics.

[8]  K. Steinbeck,et al.  Postprandial metabolism in adults with prader–willi syndrome , 2015, Obesity.

[9]  A. Haqq,et al.  Hormonal and metabolic responses to endurance exercise in children with Prader-Willi syndrome and non-syndromic obesity. , 2015, Metabolism: clinical and experimental.

[10]  V. Samuel,et al.  A high-protein diet for reducing body fat: mechanisms and possible caveats , 2014, Nutrition & Metabolism.

[11]  Z. Aycan,et al.  Prader-Willi Syndrome and Growth Hormone Deficiency , 2014, Journal of clinical research in pediatric endocrinology.

[12]  K. Clément,et al.  Comparison of body composition, basal metabolic rate and metabolic outcomes of adults with Prader Willi syndrome or lesional hypothalamic disease, with primary obesity , 2013, International Journal of Obesity.

[13]  H. Itabashi,et al.  Testosterone replacement therapy to improve secondary sexual characteristics and body composition without adverse behavioral problems in adult male patients with Prader–Willi syndrome: An observational study , 2013, American journal of medical genetics. Part A.

[14]  J. Emerick,et al.  Endocrine manifestations and management of Prader-Willi syndrome , 2013, International Journal of Pediatric Endocrinology.

[15]  A. Haqq,et al.  Effects of Adiposity and Prader-Willi Syndrome on Postexercise Heart Rate Recovery , 2013, Journal of obesity.

[16]  Klaas R. Westerterp,et al.  Physical activity and physical activity induced energy expenditure in humans: measurement, determinants, and effects , 2013, Front. Physiol..

[17]  D. J. Driscoll,et al.  A reduced-energy intake, well-balanced diet improves weight control in children with Prader-Willi syndrome. , 2013, Journal of human nutrition and dietetics : the official journal of the British Dietetic Association.

[18]  A C Pinheiro Volp,et al.  Energy expenditure: components and evaluation methods. , 2011, Nutricion hospitalaria.

[19]  G. Colditz.  Overview of the Epidemiology Methods and Applications: Strengths and Limitations of Observational Study Designs , 2010, Critical reviews in food science and nutrition.

[20]  S. Heymsfield,et al.  A new total body potassium method to estimate total body skeletal muscle mass in children. , 2007, The Journal of nutrition.

[21]  J. Donnelly,et al.  Energy expenditure and physical activity in Prader–Willi syndrome: Comparison with obese subjects , 2007, American journal of medical genetics. Part A.

[22]  M. Butler.  Management of obesity in Prader–Willi syndrome , 2006, Nature Clinical Practice Endocrinology &Metabolism.

[23]  D. J. Driscoll,et al.  Prader-Willi syndrome. , 1984, Current problems in pediatrics.

[24]  D. Allen,et al.  Growth hormone improves mobility and body composition in infants and toddlers with Prader-Willi syndrome. , 2004, The Journal of pediatrics.

[25]  K. Westerterp.  Nutrition & Metabolism BioMed Central Review Diet induced thermogenesis , 2004 .

[26]  C. Höybye.  Endocrine and metabolic aspects of adult Prader-Willi syndrome with special emphasis on the effect of growth hormone treatment. , 2004, Growth hormone & IGF research : official journal of the Growth Hormone Research Society and the International IGF Research Society.

[27]  T. Shriver,et al.  Decreased energy expenditure is caused by abnormal body composition in infants with Prader-Willi Syndrome. , 2003, The Journal of pediatrics.

[28]  A. Haqq,et al.  Effects of growth hormone on pulmonary function, sleep quality, behavior, cognition, growth velocity, body composition, and resting energy expenditure in Prader-Willi syndrome. , 2003, The Journal of clinical endocrinology and metabolism.

[29]  Jimmy D Bell,et al.  Resting metabolic rate, plasma leptin concentrations, leptin receptor expression, and adipose tissue measured by whole-body magnetic resonance imaging in women with Prader-Willi syndrome. , 2002, The American journal of clinical nutrition.

[30]  P. Burman,et al.  Endocrine dysfunction in Prader-Willi syndrome: a review with special reference to GH. , 2001, Endocrine reviews.

[31]  K. Westerterp,et al.  Body composition in Prader-Willi syndrome compared with nonsyndromal obesity: Relationship to physical activity and growth hormone function. , 2001, The Journal of pediatrics.

[32]  D. Allen,et al.  Prader-Willi Syndrome: The Effect of Growth Hormone on Childhood Body Composition , 2000 .

[33]  K. Westerterp,et al.  Energy expenditure at rest and during sleep in children with Prader-Willi syndrome is explained by body composition. , 2000, The American journal of clinical nutrition.

[34]  A Pietrobelli,et al.  Hydration of fat-free body mass: review and critique of a classic body-composition constant. , 1999, The American journal of clinical nutrition.

[35]  P. Deurenberg.  Limitations of the bioelectrical impedance method for the assessment of body fat in severe obesity. , 1996, The American journal of clinical nutrition.

[36]  H. Orskov,et al.  Metabolic effects of growth hormone in humans. , 1995, Metabolism: clinical and experimental.

[37]  J. Burleson,et al.  An evaluation of autonomic nervous system function in patients with Prader-Willi syndrome. , 1994, Pediatrics.

[38]  P. Davies,et al.  Using stable isotopes to assess reduced physical activity of individuals with Prader-Willi syndrome. , 1993, American journal of mental retardation : AJMR.

[39]  Michael T. Shelton,et al.  Resting energy expenditure. Evolution during antibiotic treatment for pulmonary exacerbation in cystic fibrosis. , 1993, Chest.

[40]  E. Ravussin,et al.  Low ratio of fat to carbohydrate oxidation as predictor of weight gain: study of 24-h RQ. , 1990, The American journal of physiology.

[41]  E. Ravussin,et al.  Determinants of 24-hour energy expenditure in man. Methods and results using a respiratory chamber. , 1986, The Journal of clinical investigation.

[42]  D. Schoeller,et al.  ENERGY EXPENDITURE AND BODY COMPOSITION IN PRADER-WILLI SYNDROME , 1984, Pediatric Research.

[43]  A. Gormican,et al.  Out-patient dietary management in the Prader-Willi syndrome. , 1976, Journal of the American Dietetic Association.

[44]  R. Blasco Redondo.  Resting energy expenditure; assessment methods and applications. , 2015, Nutricion hospitalaria.

[45]  David B Allison,et al.  Body-size dependence of resting energy expenditure can be attributed to nonenergetic homogeneity of fat-free mass. , 2002, American journal of physiology. Endocrinology and metabolism.

[46]  Y. Schutz,et al.  Meal-induced thermogenesis and obesity: is a fat meal a risk factor for fat gain in children? , 2001, The Journal of clinical endocrinology and metabolism.

[47]  W. Saris,et al.  Activity related energy expenditure in children and adolescents with Prader–Willi syndrome , 2000, International Journal of Obesity.

[48]  L. Christin,et al.  Energy expenditure in obese women before and during weight loss, after refeeding, and in the weight-relapse period. , 1993, The American journal of clinical nutrition.

[49]  James O. Hill,et al.  RESTING METABOLIC RATE IN PRADER-WILLI SYNDROME. , 1990, Dysmorphology and clinical genetics : official publication of the Center for Birth Defects Information Services, Inc.

[50]  O. Lammert,et al.  Energy expenditure during sleep in men and women: evaporative and sensible heat losses. , 1987, Human nutrition. Clinical nutrition.