The associations between diet and physical activity with body composition and walking a timed distance in adults with Prader–Willi syndrome

  • Susan G. Woods University of Central Oklahoma
  • Allen Knehans
  • Sandra Arnold
  • Carol Dionne
  • Leah Hoffman
  • Peggy Turner
  • Jonathan Baldwin
DOI: https://doi.org/10.29219/fnr.v62.1343
Keywords: Prader–Willi syndrome, diet, exercise, body composition, BMI, body fat, developmental disability

Abstract

Background: Research on aging in Prader–Willi syndrome (PWS) is limited, although people with PWS are living longer. Individuals with PWS present with high fat mass, low lean mass, and low levels of physical activity (PA). Previous reports in children and young adults with PWS show inadequate nutrient intake and body fat percentage indicating obesity. Previous studies in PWS rarely included individuals beyond young adulthood, especially studies conducted in the United States. This study includes adults from 18 to 62 years of age, and includes 19 of the estimated 60 adult individuals with PWS in Oklahoma. Because individuals with PWS are living longer, information must be provided on aging with PWS. This study is a report of the initial data for a planned longitudinal study on aging with PWS. Objective: Determine associations between body composition, diet, PA, and a timed walk for adults with PWS, and to assess adequacy of dietary intake for those individuals aging with PWS.

Design: This cross-sectional investigation determined dietary habits, PA, and body composition of adults with PWS, and tested associations between these variables.

Results: Participants ranged in age from 18 to 62 years. They had healthier body composition, at 26.8% body fat, than previously reported. Mean body mass index (BMI) was in the overweight range at 26.7. Those who consumed higher amounts of fat (as a percent of total kilocalories) had statistically significant lower body fat percentage, but this may simply reflect that individuals with lower body fat percentages felt freer to consume fat. Mean steps taken per day was 7631.7 steps but only 16% of participants met healthy PA recommendations despite participating in daily structured exercise. All participants’ diets met Dietary Guidelines for macronutrient distribution, but 80% were deficient in calcium, 100% were deficient in dietary vitamin D, and 87% were deficient in fiber. Sample size was small, so it was difficult to reach statistical significance, despite seeing clinical significance.

Conclusions: Recommend working toward healthy PA recommendations for all age groups by decreasing time in sedentary activity. Recommend increasing vitamin A and D fortified dairy products and high-fiber foods, and consider dietary supplementation, especially for calcium, vitamin D, and fiber.

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References


  1. Prader A. Die Nebennieren-Krankheiten Im Kindesalter. Ann Paediatr 1956; 186: 94–100.

  2. Cassidy S, Schwartz S, Miller J, Driscoll D. Prader-Willi syndrome. Genet Med 2012; 14(1): 10–26.

  3. Goldstone A, Holland A, Hauffa B, Hokken-Koelega A, Tauber M. Recommendations for the diagnosis and management of Prader-Willi syndrome. J Clin Endocrinol Metab 2008; 93: 4183–97.

  4. Whittington J, Holland A, Webb T, Butler J, Clarke D, Boer H. Cognitive abilities and genotype in a population-based sample of people with Prader-Willi syndrome. J Intellect Disabil Res 2004; 48: 172–87.

  5. McAllister C, Whittington J. A short clinical overview of Prader-Willi syndrome. Clin Obes 2011; 1: 184–8.

  6. Irizarry K, Miller M, Freemark M, Haqq A. Prader-Willi syndrome: Genetics, metabolomics, hormonal function, and new approaches to therapy. Adv Ped 2016; 63: 47–77.

  7. Lindgren A, Lindberg A. Growth hormone treatment completely normalizes adult height and improves body composition in Prader-Willi syndrome: experience from KIGS. Horm Res 2008; 70(3): 182–7.

  8. Lloret-Linares C, Faucher P, Coupaye M, Alilli R, Green A, Basedevant A, 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. Int J Obes 2013; 37: 1198–203.

  9. Reus L, Zwarts M, van Vlimmeren LA, Willemsen MA, Otten BJ, Nijhuis-van der Sanden MWG. Motor problems in Prader-Willi syndrome: a systematic review on body composition and neuromuscular functioning. Neurosci Biobehav Rev 2011; 35: 956–69.

  10. Kuppens R, Bakker N, Siemensma E, Tummers-de Lind van Wijngaarden R, Donze S, Festen D, et al. Beneficial effects of GH in young adults with Prader-Willi syndrome: a 2-year crossover trial. J Clin Endocrinol Metab 2016; 101(11): 4110–16.

  11. Ravussin E, Bogardus C. Relationship of genetics, age, and physical fitness to daily energy expenditure and fuel utilization. Am J Clin Nutr 1980; 49(suppl): 968–75.

  12. Schoeller D, Levitsky L, Bandni L, Dietz W, Walczak A. Energy expenditure and body composition in Prader-Willi syndrome. Metabolism 1988; 37(2): 115–20.

  13. Butler M, Theodoro M, Bittel D, Donnelly J. Energy expenditure and physical activity in Prader-Willi syndrome: comparison with obese subjects. Am J Med Genet A 2007; 143A(5): 449–59.

  14. Lazzer S, Grugni G, Tringali G, Sartorio A. Prediction of basal metabolic rate in patients with Prader-Willi syndrome. Eur J Clin Nutr 2016; 70: 494–8.

  15. Einfeld S, Kavanagh S, Smith A, Evans E, Tonge B, Taffe J. Mortality in Prader-Willi syndrome. Am J Ment Retard 2006; 111: 193–8.

  16. Whittington J, Holland A, Webb T, Butler J, Clarke D, Boer H. Population prevalence and estimated birth incidence and mortality rate for people with Prader-Willi syndrome in one UK Health Region. J Med Genet 2001; 38: 792–8.

  17. Butler J, Whittington J, Holland A, Boer H, Clarke D, Webb T. Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi syndrome: a population-based study. Dev Med Child Neurol 2002; 44: 248–55.

  18. Butler M, Manzardo A, Heinemann J, Loker C, Loker J. Causes of death in Prader-Willi syndrome: Prader-Willi syndrome association (USA) 40-year mortality survey. Genet Med 2017; 19(6): 635–42.

  19. Zipf W. Prader-Willi syndrome: the care and treatment of infants, children, and adults. Adv Pediatr 2004; 51: 409–34.

  20. Butler M, Hanchett J, Thompson T. Clinical findings and natural history of Prader-Willi syndrome. 3rd ed. New York: Springer; 2006.

  21. Holland A, Treasure J, Coskeran P, Dallow J, Milton N, Hillhouse E. Measurement of excessive appetite and metabolic changes in Prader-Willi syndrome. Int J Obes 1993; 17: 527–32.

  22. Hinton E, Holland A, Gellatly M, Soni S, Patterson M, Ghatei M, et al. Neural representations of hunger and satiety in Prader-Willi yndrome. Int J Obes 2006; 30: 313–21.

  23. Goldstone A. Prader-Willi syndrome: advances in genetics, pathophysiology and treatment. Trends Endocrinol Metab 2004; 15: 12–20.

  24. Penner de Lima V, Emerich DR, Guedes de Mesquita ML, Paternez ACAC, Carreiro LRR, de Pina Neto JM, et al. Nutritional intervention with hypocaloric diet for weight contrl in children and adolescents with Prader-Willi syndrome. Eat Behav 2016; 21: 189–92.

  25. Bonfig W, Dokoupil K, Schmidt H. A special, strict, fat-reduced, and carbohydrate-modified diet leads to marked weight reduction even in overweight adolescents with Prader-Willi syndrome (PWS). ScientificWorldJournal 2009; 9: 934–9.

  26. Schmidt H, Pozza S, Bonfig W, Schwarz H, Dokoupil K. Successful early dietary intervention avoids obesity in patients with Prader-Willi syndrome: a ten-year follow-up. J Pediatr Endocrinol Metab 2008; 21(7): 651–5.

  27. Miller J, Lynn C, Shuster J, Driscoll DJ. A reduced-energy intake, well-balanced diet improves weight control in children with Prader-Willi syndrome. J Hum Nutr Diet 2013; 26: 2–9.

  28. Lindmark M, Trygg K, Giltvedt K, Kolset S. Nutritient intake of young children with Prader-Willi syndrome. Food Nutr Res 2010; 54: 2112.

  29. Rubin DA, Nowak J, McLaren E, Patino M, Castner DM, Dumont-Driscoll MC. Nutritional intake in children with Prader-Willi syndrome and non-congenital obesity. Food Nutr Res 2015; 29427.

  30. Nordstrom M, Hansen BH, Paus B, Kolset SO. Accelerometer-determined physical activity and walking capacity in persons with Down syndrome, Williams syndrome and Prader-Willi syndrome. Res Dev Disabil 2013; 34: 4395–403.

  31. Schlumpf M, Eiholzer U, Gygax M, Schmid S, van der Sluis I, I'Allemand D. A daily comprehensive muscle training programme increases lean mass and spontaneous activity in children with Prader-Willi syndrome after 6 months. J Pediatr Endocrinol Metab 2006; 19: 65–74.

  32. Rubin DA, Mowttapa M, Weiss J, Barrera-Ng A. Physical activity in children with Prader-Willi syndrome: a parents' perspective. Calif J Healh Promot 2012; 10(Special Issue: Obesity Prevention & Intervention): 57–66.

  33. Dykens E. Leisure activities in Prader-Willi syndrome: implications for health, cognition and adaptive functioning. J Autism Dev Disord 2014; 44(2): 294–302.

  34. van Mil E, Westerterp K, Gerver W, van Marken Lichtenbelt W, Kester A, Saris W. Body composition in Prader-Willi syndrome compared with nonsyndromal obesity: relationship to physical activity and growth hormone function. J Pediatr 2001; 139: 708–14.

  35. Eiholzer U, Nordmann Y, L'Allemand D, Schlumpf M, Schmid S, Kromeyer-Hauschild K. Improving body composition and physical activity in Prader-Willi syndrome. J Pediatr 2003; 142: 73–8.

  36. Eiholzer U, Schlumpf M, Nordmann Y, I'Allemand D. Early manifestations of Prader-Willi syndrome: influence of growth hormone. J Pediatr Endocrinol Metab 2001; 14(Supp 6): 1441–4.

  37. Hinckson E, Dickinson A, Water T, Sands M, Penman L. Physical activity, dietary habits and overall health in overweight and obese children and youth with intellectual disability or autism. Res Dev Disabil 2013; 34: 1170–8.

  38. American Thoracic Society. ATS Statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med 2002; 166: 111–17.

  39. Rasekaba T, Lee A, Naughton M, Williams TJ, Holland AE. The six-minute walk test: a useful metric for the cardiopulmonary patient. Int Med J 2009; 39(8): 495–501.

  40. Rubin DA, Cano-Sokoloff N, Castnner D, Judelson D, Wright P, Duran A, et al. Update on body composition and bone density in children with Prader-Willi syndrome. Horm Res Paediatr 2013; 79(5): 271–6.

  41. Castner D, Tucker J, Wilson K, Rubin D. Patterns of habitual physical activity in youth with and without Prader-Willi syndrome. Res Dev Disabil 2014; 35(11): 3081–8.

  42. Brambilla P, Bosio L, Manzoni P, Pietrobelli A, Beccaria L, Chiumelo G. Peculiar body composition in patients with Prader-Labhart-Willi syndrome. Am J Clin Nutr 1997; 65: 1369–74.

  43. Orsso CE, Mackenzie M, Alberga AS, Sharma AM, Richer L, Rubin DA, et al. The use of magnetic resonance imaging to characterize abnormal body composition phenotypes in youth with Prader-Willi syndrome. Metab Clin Exp 2017; 69: 67–75.

  44. Theodoro MF, Talebizadeh Z, Butler MG. Body composition and fatness patterns in Prader-Willi syndrome: comparison with simple obesity. Obesity 2006; 14(10): 1685–90.

  45. Klein S, Allison D, Heymsfield S, Kelley D, Leiel R, Nonas C, et al. Waist circumference and cardiometabolic risk: a consensus statement from Shaping America's Health: association for weight management and obesity prevention. Am J Clin Nutr 2007; 85(5): 1197–202.

  46. World Health Organization. Waist circumference and waist-hip ratio: report of a WHO expert consultation, Geneva, 8–11, December 2008. 2011. Contract No.: ISBN: 978 92 4 150149 1.

  47. van den Berg-Emmons R, Festen D, Hokken-Koelega A, Bussman J, Stam H. Everyday physical activity and adiposity in Prader-Willi syndrome. J Pediatr Endocrinol Metab 2008; 21(11): 1041–8.

  48. Faria S, Faria O, Cardeal M, Ito M. Validation study of multi-frequency bioelectrical impedance with dual-energy X-ray absorptiometry among obese patients. Obes Surg 2014; 24: 1476–80.

  49. Donini L, Poggiogalle E, del Balzo V, Lubrano C, Faliva M, Opizzi A, et al. How to estimate fat mass in overweight and obese subjects. In t J Endocrinol 2013; 2013: 285680.

  50. Miller R, Chambers TL, Burns SP, Goddard MP. Validating InBody 570 Multi-frequency bioelectrical impedance analyzer versus DXA for body fat percentage analysis. Med Sci Sports Exerc 2016; 48(5S): 991.

  51. Kyle U, Bosaeus I, De Lorenzo A, Deurenberg P, Elia M, Gomez J, et al. Bioelectrical impedance analysis—part 1: review of principles and methods. Clin Nutr 2004; 23: 1226–43.

  52. Boneva-Asiova Z, Boyanov M. Body composition analysis by leg-to-leg bioelectrical impedance and dual-energy X-ray absorptiometry in non-obese and obese individuals. Diabetes Obes Metab 2008; 10: 1012–18.

  53. Shepherd J, Heymsfield S, Norris S, Redman L, Ward L, Slater C. Measuring body composition in low-resoure settings across the life course. Obesity 2016; 24(5): 985–8.

  54. Lin H, Chen M, Chuang C, Huang C, Niu D, Lin S. Assessment of body composition using bioelectrical impedance analysis in Prader-Willi syndrome. J Formos Med Assoc 2011; 110: 719–23.

  55. FoodWorks. FoodWorks. Nutrient Analysis Software: the professional's choice. Available from: http://nutritionco.com/FoodWorks.htm. Accessed November 2016.

  56. Prader-Willi Syndrome Association (USA). Prader-Willi Syndrome Statistics Available from: https://www.pwsausa.org/pws-statistics/. Accessed December 2016.

  57. United States Department of Agriculture. Recommended Dietary Allowances (RDA). Available from: https://www.nal.usda.gov. Accessed December 2016.

  58. Office of Disease Prevention and Health Promotion. 2015 Dietary Guidelines. Available from: https://www.health.gov/dietaryguidelines 2015. Accessed December 2016.

  59. U.S. Food and Drug Administration. Daily value. Available from: http://www.fda.gov. Accessed January 2017.

  60. Hoybye C, Hilding A, Jacobsson H, Thoren M. Metabolic profile and body composition in adults with Prader-Willi syndrome and severe obesity. J Clin Endocrinol Metab 2002; 87(8): 3590–7.

  61. Carrel A, Myers S, Whitman BY, Allen D. Growth hormone improves body composition, fat utilization, physical strength and agility, and growth in Prader-Willi syndrome: a controlled study. J Pediatr 1999; 134: 215–21.

  62. Tudor-Locke C, Bassett Jr D. How many steps/day are enough? Preliminary pedometer indices for public health. Sports Med 2004; 34(1): 1–8.

Published
2018-06-18
How to Cite
Woods S. G., Knehans A., Arnold S., Dionne C., Hoffman L., Turner P., & Baldwin J. (2018). The associations between diet and physical activity with body composition and walking a timed distance in adults with Prader–Willi syndrome. Food & Nutrition Research, 62. https://doi.org/10.29219/fnr.v62.1343
Issue
Vol 62 (2018)
Section
Original Articles

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