Skip to main content
SpringerLink
Log in

Gastrointestinal System, Obesity, and Body Composition

  • Chapter
  • First Online:
Management of Prader-Willi Syndrome

Abstract

Obesity or increased body fat is arguably the most characteristic physical feature of Prader-Willi syndrome (PWS). Perhaps for this reason, a vast majority of the publications regarding PWS mention, discuss, and/or explore the topic of obesity. Despite this attention, the diagnosis, pathogenesis, optimal treatment, monitoring, and outcome of this condition in PWS remain controversial and incompletely defined. The paradox of the underweight infant with PWS evolving into an overweight child and adult has led to considerable speculation regarding pathophysiology. In addition, there is an inappropriate interchangeable use of the terms obesity, body mass index (BMI), and weight in the medical and scientific literature.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Pedersen A, Sørensen CE, Proctor GB, Carpenter GH. Salivary functions in mastication, taste and textural perception, swallowing and initial digestion. Oral Dis. 2018;24(8):1399–416.

    Article  PubMed  Google Scholar 

  2. Carpenter GH. The secretion, components, and properties of saliva. Annu Rev Food Sci Technol. 2013;4:267–76.

    Article  CAS  PubMed  Google Scholar 

  3. Hollis JH. The effect of mastication on food intake, satiety and body weight. Physiol Behav. 2018;193(Pt B):242–5.

    Article  CAS  PubMed  Google Scholar 

  4. Pereira LJ, van der Bilt A. The influence of oral processing, food perception and social aspects on food consumption: a review. J Oral Rehabil. 2016;43(8):630–48.

    Article  CAS  PubMed  Google Scholar 

  5. Shaker R, Hogan WJ. Normal physiology of the aerodigestive tract and its effect on the upper gut. Am J Med. 2003;115(Suppl 3A):2S–9S.

    Article  PubMed  Google Scholar 

  6. Ertekin C, Aydogdu I. Neurophysiology of swallowing. Clin Neurophysiol. 2003;114(12):2226–44.

    Article  PubMed  Google Scholar 

  7. Ertekin C. Voluntary versus spontaneous swallowing in man. Dysphagia. 2011;26(2):183–92.

    Article  PubMed  Google Scholar 

  8. Finan DS, Barlow SM. Intrinsic dynamics and mechanosensory modulation of non-nutritive sucking in human infants. Early Hum Dev. 1998;52(2):181–97.

    Article  CAS  PubMed  Google Scholar 

  9. Fucile S, McFarland DH, Gisel EG, Lau C. Oral and nonoral sensorimotor interventions facilitate suck-swallow-respiration functions and their coordination in preterm infants. Early Hum Dev. 2012;88(6):345–50.

    Article  PubMed  Google Scholar 

  10. Gryboski JD. Gastrointestinal function in the infant and young child. Clin Gastroenterol. 1977;6(2):253–65.

    Article  CAS  PubMed  Google Scholar 

  11. Mizuno K, Ueda A. The maturation and coordination of sucking, swallowing, and respiration in preterm infants. J Pediatr. 2003;142(1):36–40.

    Article  PubMed  Google Scholar 

  12. Greenberg F, Elder FF, Ledbetter DH. Neonatal diagnosis of Prader-Willi syndrome and its implications. Am J Med Genet. 1987;28(4):845–56.

    Article  CAS  PubMed  Google Scholar 

  13. Wang P, Zhou W, Yuan W, Huang L, Zhao N, Chen X. Prader-Willi syndrome in neonates: twenty cases and review of the literature in Southern China. BMC Pediatr. 2016;16:124.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Salehi P, Stafford HJ, Glass RP, Leavitt A, Beck AE, McAfee A, et al. Silent aspiration in infants with Prader-Willi syndrome identified by videofluoroscopic swallow study. Medicine (Baltimore). 2017;96(50):e9256.

    Article  Google Scholar 

  15. Fieldstone A, Zipf WB, Schwartz HC, Berntson GG. Food preferences in Prader-Willi syndrome, normal weight and obese controls. Int J Obes Relat Metab Disord. 1997;21(11):1046–52.

    Article  CAS  PubMed  Google Scholar 

  16. Stevenson DA, Heinemann J, Angulo M, Butler MG, Loker J, Rupe N, et al. Deaths due to choking in Prader-Willi syndrome. Am J Med Genet A. 2007;143A(5):484–7.

    Article  PubMed  Google Scholar 

  17. Holm VA, Cassidy SB, Butler MG, Hanchett JM, Greenswag LR, Whitman BY, et al. Prader-Willi syndrome: consensus diagnostic criteria. Pediatrics. 1993;91(2):398–402.

    Article  CAS  PubMed  Google Scholar 

  18. Hart PS. Salivary abnormalities in Prader-Willi syndrome. Ann N Y Acad Sci. 1998;842:125–31.

    Article  CAS  PubMed  Google Scholar 

  19. Boccellino M, Di Stasio D, Serpico R, Lucchese A, Guida A, Settembre G, et al. Analysis of saliva samples in patients with Prader-Willi syndrome. J Biol Regul Homeost Agents. 2018;32(2 Suppl. 1):107–11.

    CAS  PubMed  Google Scholar 

  20. González LC, Villa CG, Cárdenas AC. Prader Willi Syndrome: saliva quantification and culture in 10 patients. Med Oral Patol Oral Cir Bucal. 2008;13(12):E774–7.

    PubMed  Google Scholar 

  21. Saeves R, Reseland JE, Kvam BM, Sandvik L, Nordgarden H. Saliva in Prader-Willi syndrome: quantitative and qualitative characteristics. Arch Oral Biol. 2012;57(10):1335–41.

    Article  PubMed  Google Scholar 

  22. Levin SL, Khaikina LI. Is there neural control over electrolyte reabsorption in the human salivary gland? Clin Sci (Lond). 1987;72(5):541–8.

    Article  CAS  Google Scholar 

  23. Tennese AA, Gee CB, Wevrick R. Loss of the Prader-Willi syndrome protein necdin causes defective migration, axonal outgrowth, and survival of embryonic sympathetic neurons. Dev Dyn. 2008;237(7):1935–43.

    Article  PubMed  Google Scholar 

  24. Munné-Miralvés C, Brunet-Llobet L, Cahuana-Cárdenas A, Torné-Durán S, Miranda-Rius J, Rivera-Baró A. Oral disorders in children with Prader-Willi syndrome: a case control study. Orphanet J Rare Dis. 2020;15(1):43.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Lingström P, Moynihan P. Nutrition, saliva, and oral health. Nutrition. 2003;19(6):567–9.

    Article  PubMed  Google Scholar 

  26. Butler MG, Lee PDK, Whitman BY. Management of Prader-Willi Syndrome, vol. xxiii. 3rd ed. New York: Springer; 2006. p. 550.

    Book  Google Scholar 

  27. Young W, Khan F, Brandt R, Savage N, Razek AA, Huang Q. Syndromes with salivary dysfunction predispose to tooth wear: case reports of congenital dysfunction of major salivary glands, Prader-Willi, congenital rubella, and Sjögren's syndromes. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001;92(1):38–48.

    Article  CAS  PubMed  Google Scholar 

  28. Rao RS, Akula R, Satyanarayana TSV, Indugu V. Recent advances of pacemakers in treatment of xerostomia: a systematic review. J Int Soc Prev Community Dent. 2019;9(4):311–5.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Olczak-Kowalczyk D, Korporowicz E, Gozdowski D, Lecka-Ambroziak A, Szalecki M. Oral findings in children and adolescents with Prader-Willi syndrome. Clin Oral Investig. 2019;23(3):1331–9.

    Article  PubMed  Google Scholar 

  30. Bots CP, Schueler YT, Brand HS, van Nieuw AA. A patient with Prader-Willi syndrome. Characteristics, oral consequences and treatment options. Ned Tijdschr Tandheelkd. 2004;111(2):55–8.

    CAS  PubMed  Google Scholar 

  31. Uemura Y, Oka A, Kurosaka H, Yamashiro T. Comprehensive orthodontic treatment of a patient with Prader-Willi Syndrome. Cleft Palate Craniofac J. 2020;58:1459. https://doi.org/10.1177/1055665620977375.

    Article  PubMed  Google Scholar 

  32. Vandenplas Y, Rudolph CD, Di Lorenzo C, Hassall E, Liptak G, Mazur L, et al. Pediatric gastroesophageal reflux clinical practice guidelines: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN). J Pediatr Gastroenterol Nutr. 2009;49(4):498–547.

    Article  PubMed  Google Scholar 

  33. Nelson SP, Chen EH, Syniar GM, Christoffel KK. Prevalence of symptoms of gastroesophageal reflux during childhood: a pediatric practice-based survey. Pediatric Practice Research Group. Arch Pediatr Adolesc Med. 2000;154(2):150–4.

    Article  CAS  PubMed  Google Scholar 

  34. Poets CF. Gastroesophageal reflux: a critical review of its role in preterm infants. Pediatrics. 2004;113(2):e128–32.

    Article  PubMed  Google Scholar 

  35. Hornby PJ. Central neurocircuitry associated with emesis. Am J Med. 2001;111(Suppl 8A):106S–12S.

    Article  PubMed  Google Scholar 

  36. Alexander RC, Greenswag LR, Nowak AJ. Rumination and vomiting in Prader-Willi syndrome. Am J Med Genet. 1987;28(4):889–95.

    Article  CAS  PubMed  Google Scholar 

  37. Swaab DF, Purba JS, Hofman MA. Alterations in the hypothalamic paraventricular nucleus and its oxytocin neurons (putative satiety cells) in Prader-Willi syndrome: a study of five cases. J Clin Endocrinol Metab. 1995;80(2):573–9.

    CAS  PubMed  Google Scholar 

  38. Martin A, State M, Anderson GM, Kaye WM, Hanchett JM, McConaha CW, et al. Cerebrospinal fluid levels of oxytocin in Prader-Willi syndrome: a preliminary report. Biol Psychiatry. 1998;44(12):1349–52.

    Article  CAS  PubMed  Google Scholar 

  39. Sloan TB, Kaye CI. Rumination risk of aspiration of gastric contents in the Prader-Willi syndrome. Anesth Analg. 1991;73(4):492–5.

    Article  CAS  PubMed  Google Scholar 

  40. Wharton RH, Wang T, Graeme-Cook F, Briggs S, Cole RE. Acute idiopathic gastric dilation with gastric necrosis in individuals with Prader-Willi syndrome. Am J Med Genet. 1997;73(4):437–41.

    Article  CAS  PubMed  Google Scholar 

  41. Blat C, Busquets E, Gili T, Caixàs A, Gabau E, Corripio R. Gastric dilatation and abdominal compartment syndrome in a child with Prader-Willi Syndrome. Am J Case Rep. 2017;18:637–40.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Stevenson DA, Heinemann J, Angulo M, Butler MG, Loker J, Rupe N, et al. Gastric rupture and necrosis in Prader-Willi syndrome. J Pediatr Gastroenterol Nutr. 2007;45(2):272–4.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Butler JV, Whittington JE, Holland AJ, 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(4):248–55.

    Article  CAS  PubMed  Google Scholar 

  44. Kuhlmann L, Joensson IM, Froekjaer JB, Krogh K, Farholt S. A descriptive study of colorectal function in adults with Prader-Willi Syndrome: high prevalence of constipation. BMC Gastroenterol. 2014;14:63.

    Article  PubMed  PubMed Central  Google Scholar 

  45. Bhargava SA, Putnam PE, Kocoshis SA, Rowe M, Hanchett JM. Rectal bleeding in Prader-Willi syndrome. Pediatrics. 1996;97(2):265–7.

    Article  CAS  PubMed  Google Scholar 

  46. Campeotto F, Naudin C, Viot G, Dupont C. Rectal self-mutilation, rectal bleeding and Prader-Willi syndrome. Arch Pediatr. 2001;8(10):1075–7.

    Article  CAS  PubMed  Google Scholar 

  47. Salehi P, Lee D, Ambartsumyan L, Sikka N, Scheimann AO. Rectal picking masquerading as inflammatory bowel disease in Prader-Willi Syndrome. J Pediatr Gastroenterol Nutr. 2018;67(1):59–63.

    Article  PubMed  Google Scholar 

  48. Xanthakos SA, Lavine JE, Yates KP, Schwimmer JB, Molleston JP, Rosenthal P, et al. Progression of fatty liver disease in children receiving standard of care lifestyle advice. Gastroenterology. 2020;159(5):1731–51.e10.

    Article  CAS  PubMed  Google Scholar 

  49. Fintini D, Inzaghi E, Colajacomo M, Bocchini S, Grugni G, Brufani C, et al. Non-Alcoholic Fatty Liver Disease (NAFLD) in children and adolescents with Prader-Willi Syndrome (PWS). Pediatr Obes. 2016;11(3):235–8.

    Article  CAS  PubMed  Google Scholar 

  50. Jalali M, Rahimlou M, Mahmoodi M, Moosavian SP, Symonds ME, Jalali R, et al. The effects of metformin administration on liver enzymes and body composition in non-diabetic patients with non-alcoholic fatty liver disease and/or non-alcoholic steatohepatitis: an up-to date systematic review and meta-analysis of randomized controlled trials. Pharmacol Res. 2020;159:104799.

    Article  CAS  PubMed  Google Scholar 

  51. Hashizume K, Nakajo T, Kawarasaki H, Iwanaka T, Kanamori Y, Tanaka K, et al. Prader-Willi syndrome with del(15)(q11,q13) associated with hepatoblastoma. Acta Paediatr Jpn. 1991;33(6):718–22.

    Article  CAS  PubMed  Google Scholar 

  52. Takayasu H, Motoi T, Kanamori Y, Kitano Y, Nakanishi H, Tange T, et al. Two case reports of childhood liver cell adenomas harboring beta-catenin abnormalities. Hum Pathol. 2002;33(8):852–5.

    Article  PubMed  Google Scholar 

  53. Yamashita M, Koishi K, Yamaya R, Tsubo T, Matsuki A, Oyama T. Anaesthetic considerations in the Prader-Willi syndrome: report of four cases. Can Anaesth Soc J. 1983;30(2):179–84.

    Article  CAS  PubMed  Google Scholar 

  54. Forbes GB. The companionship of lean and fat. Basic Life Sci. 1993;60:1–14.

    CAS  PubMed  Google Scholar 

  55. Forbes GB. A distinctive obesity: body composition provides the clue. Am J Clin Nutr. 1997;65(5):1540–1.

    Article  CAS  PubMed  Google Scholar 

  56. Brambilla P, Bosio L, Manzoni P, Pietrobelli A, Beccaria L, Chiumello G. Peculiar body composition in patients with Prader-Labhart-Willi syndrome. Am J Clin Nutr. 1997;65(5):1369–74.

    Article  CAS  PubMed  Google Scholar 

  57. Carrel AL, Allen DB. Effects of growth hormone on body composition and bone metabolism. Endocrine. 2000;12(2):163–72.

    Article  CAS  PubMed  Google Scholar 

  58. Eiholzer U, Blum WF, Molinari L. Body fat determined by skinfold measurements is elevated despite underweight in infants with Prader-Labhart-Willi syndrome. J Pediatr. 1999;134(2):222–5.

    Article  CAS  PubMed  Google Scholar 

  59. Eiholzer U, l'Allemand D, van der Sluis I, Steinert H, Gasser T, Ellis K. Body composition abnormalities in children with Prader-Willi syndrome and long-term effects of growth hormone therapy. Horm Res. 2000;53(4):200–6.

    CAS  PubMed  Google Scholar 

  60. Ellis KJ, Shypailo RJ, Pivarnik JM, Jenks BH, Walzel P, Lee PD. Body composition of HIV-infected male adults with wasting syndrome. Asia Pac J Clin Nutr. 1995;4(1):105–8.

    CAS  PubMed  Google Scholar 

  61. Lindgren AC, Barkeling B, Hägg A, Ritzén EM, Marcus C, Rössner S. Eating behavior in Prader-Willi syndrome, normal weight, and obese control groups. J Pediatr. 2000;137(1):50–5.

    Article  CAS  PubMed  Google Scholar 

  62. Key AP, Jones D, Zengin-Bolatkale H, Roof E, Hunt-Hawkins H. Visual food cue processing in children with Prader-Willi Syndrome. Physiol Behav. 2021;238:113492.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  63. Vijayvargiya P, Chedid V, Wang XJ, Atieh J, Maselli D, Burton DD, et al. Associations of gastric volumes, ingestive behavior, calorie and volume intake, and fullness in obesity. Am J Physiol Gastrointest Liver Physiol. 2020;319(2):G238–G44.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  64. Arenz T, Schwarzer A, Pfluger T, Koletzko S, Schmidt H. Delayed gastric emptying in patients with Prader Willi Syndrome. J Pediatr Endocrinol Metab. 2010;23(9):867–71.

    Article  CAS  PubMed  Google Scholar 

  65. Choe YH, Jin DK, Kim SE, Song SY, Paik KH, Park HY, et al. Hyperghrelinemia does not accelerate gastric emptying in Prader-Willi syndrome patients. J Clin Endocrinol Metab. 2005;90(6):3367–70.

    Article  CAS  PubMed  Google Scholar 

  66. Rehfeld JF. Cholecystokinin and the hormone concept. Endocr Connect. 2021;10(3):R139–R50.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  67. Paik KH, Jin DK, Lee KH, Armstrong L, Lee JE, Oh YJ, et al. Peptide YY, cholecystokinin, insulin and ghrelin response to meal did not change, but mean serum levels of insulin is reduced in children with Prader-Willi syndrome. J Korean Med Sci. 2007;22(3):436–41.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  68. Butler MG, Carlson MG, Schmidt DE, Feurer ID, Thompson T. Plasma cholecystokinin levels in Prader-Willi syndrome and obese subjects. Am J Med Genet. 2000;95(1):67–70.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  69. Tomita T, Greeley G, Watt L, Doull V, Chance R. Protein meal-stimulated pancreatic polypeptide secretion in Prader-Willi syndrome of adults. Pancreas. 1989;4(4):395–400.

    Article  CAS  PubMed  Google Scholar 

  70. Zipf WB, O'Dorisio TM, Cataland S, Dixon K. Pancreatic polypeptide responses to protein meal challenges in obese but otherwise normal children and obese children with Prader-Willi syndrome. J Clin Endocrinol Metab. 1983;57(5):1074–80.

    Article  CAS  PubMed  Google Scholar 

  71. Berntson GG, Zipf WB, O'Dorisio TM, Hoffman JA, Chance RE. Pancreatic polypeptide infusions reduce food intake in Prader-Willi syndrome. Peptides. 1993;14(3):497–503.

    Article  CAS  PubMed  Google Scholar 

  72. Zipf WB, O'Dorisio TM, Berntson GG. Short-term infusion of pancreatic polypeptide: effect on children with Prader-Willi syndrome. Am J Clin Nutr. 1990;51(2):162–6.

    Article  CAS  PubMed  Google Scholar 

  73. Batterham RL, Cohen MA, Ellis SM, Le Roux CW, Withers DJ, Frost GS, et al. Inhibition of food intake in obese subjects by peptide YY3-36. N Engl J Med. 2003;349(10):941–8.

    Article  CAS  PubMed  Google Scholar 

  74. Butler MG, Bittel DC, Talebizadeh Z. Plasma peptide YY and ghrelin levels in infants and children with Prader-Willi syndrome. J Pediatr Endocrinol Metab. 2004;17(9):1177–84.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  75. Bizzarri C, Rigamonti AE, Luce A, Cappa M, Cella SG, Berini J, et al. Children with Prader-Willi syndrome exhibit more evident meal-induced responses in plasma ghrelin and peptide YY levels than obese and lean children. Eur J Endocrinol. 2010;162(3):499–505.

    Article  CAS  PubMed  Google Scholar 

  76. Giménez-Palop O, Giménez-Pérez G, Mauricio D, González-Clemente JM, Potau N, Berlanga E, et al. A lesser postprandial suppression of plasma ghrelin in Prader-Willi syndrome is associated with low fasting and a blunted postprandial PYY response. Clin Endocrinol. 2007;66(2):198–204.

    Article  Google Scholar 

  77. Talebizadeh Z, Kibiryeva N, Bittel DC, Butler MG. Ghrelin, peptide YY and their receptors: gene expression in brain from subjects with and without Prader-Willi syndrome. Int J Mol Med. 2005;15(4):707–11.

    CAS  PubMed  Google Scholar 

  78. Goldstone AP, Unmehopa UA, Bloom SR, Swaab DF. Hypothalamic NPY and agouti-related protein are increased in human illness but not in Prader-Willi syndrome and other obese subjects. J Clin Endocrinol Metab. 2002;87(2):927–37.

    Article  CAS  PubMed  Google Scholar 

  79. Höybye C, Barkeling B, Espelund U, Petersson M, Thorén M. Peptides associated with hyperphagia in adults with Prader-Willi syndrome before and during GH treatment. Growth Hormon IGF Res. 2003;13(6):322–7.

    Article  Google Scholar 

  80. Omokawa M, Ayabe T, Nagai T, Imanishi A, Omokawa A, Nishino S, et al. Decline of CSF orexin (hypocretin) levels in Prader-Willi syndrome. Am J Med Genet A. 2016;170A(5):1181–6.

    Article  PubMed  Google Scholar 

  81. Nevsimalova S, Vankova J, Stepanova I, Seemanova E, Mignot E, Nishino S. Hypocretin deficiency in Prader-Willi syndrome. Eur J Neurol. 2005;12(1):70–2.

    Article  CAS  PubMed  Google Scholar 

  82. Bittel DC, Kibiryeva N, Sell SM, Strong TV, Butler MG. Whole genome microarray analysis of gene expression in Prader-Willi syndrome. Am J Med Genet A. 2007;143A(5):430–42.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  83. Manzardo AM, Johnson L, Miller JL, Driscoll DJ, Butler MG. Higher plasma orexin a levels in children with Prader-Willi syndrome compared with healthy unrelated sibling controls. Am J Med Genet A. 2016;170(9):2328–33.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  84. Ge Y, Ohta T, Driscoll DJ, Nicholls RD, Kalra SP. Anorexigenic melanocortin signaling in the hypothalamus is augmented in association with failure-to-thrive in a transgenic mouse model for Prader-Willi syndrome. Brain Res. 2002;957(1):42–5.

    Article  CAS  PubMed  Google Scholar 

  85. Chen HY, Trumbauer ME, Chen AS, Weingarth DT, Adams JR, Frazier EG, et al. Orexigenic action of peripheral ghrelin is mediated by neuropeptide Y and agouti-related protein. Endocrinology. 2004;145(6):2607–12.

    Article  CAS  PubMed  Google Scholar 

  86. Sun Y, Ahmed S, Smith RG. Deletion of ghrelin impairs neither growth nor appetite. Mol Cell Biol. 2003;23(22):7973–81.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  87. Cummings DE, Clement K, Purnell JQ, Vaisse C, Foster KE, Frayo RS, et al. Elevated plasma ghrelin levels in Prader Willi syndrome. Nat Med. 2002;8(7):643–4.

    Article  CAS  PubMed  Google Scholar 

  88. DelParigi A, Tschöp M, Heiman ML, Salbe AD, Vozarova B, Sell SM, et al. High circulating ghrelin: a potential cause for hyperphagia and obesity in prader-willi syndrome. J Clin Endocrinol Metab. 2002;87(12):5461–4.

    Article  CAS  PubMed  Google Scholar 

  89. Goldstone AP, Patterson M, Kalingag N, Ghatei MA, Brynes AE, Bloom SR, et al. Fasting and postprandial hyperghrelinemia in Prader-Willi syndrome is partially explained by hypoinsulinemia, and is not due to peptide YY3-36 deficiency or seen in hypothalamic obesity due to craniopharyngioma. J Clin Endocrinol Metab. 2005;90(5):2681–90.

    Article  CAS  PubMed  Google Scholar 

  90. Höybye C. Endocrine and metabolic aspects of adult Prader-Willi syndrome with special emphasis on the effect of growth hormone treatment. Growth Hormon IGF Res. 2004;14(1):1–15.

    Article  Google Scholar 

  91. Tauber M, Coupaye M, Diene G, Molinas C, Valette M, Beauloye V. Prader-Willi syndrome: a model for understanding the ghrelin system. J Neuroendocrinol. 2019;31(7):e12728.

    Article  PubMed  Google Scholar 

  92. Haqq AM, Stadler DD, Rosenfeld RG, Pratt KL, Weigle DS, Frayo RS, et al. Circulating ghrelin levels are suppressed by meals and octreotide therapy in children with Prader-Willi syndrome. J Clin Endocrinol Metab. 2003;88(8):3573–6.

    Article  CAS  PubMed  Google Scholar 

  93. Beauloye V, Diene G, Kuppens R, Zech F, Winandy C, Molinas C, et al. High unacylated ghrelin levels support the concept of anorexia in infants with prader-willi syndrome. Orphanet J Rare Dis. 2016;11(1):56.

    Article  PubMed  PubMed Central  Google Scholar 

  94. Allas S, Caixàs A, Poitou C, Coupaye M, Thuilleaux D, Lorenzini F, et al. AZP-531, an unacylated ghrelin analog, improves food-related behavior in patients with Prader-Willi syndrome: a randomized placebo-controlled trial. PLoS One. 2018;13(1):e0190849.

    Article  PubMed  PubMed Central  Google Scholar 

  95. Butler MG, Bittel DC. Plasma obestatin and ghrelin levels in subjects with Prader-Willi syndrome. Am J Med Genet A. 2007;143A(5):415–21.

    Article  PubMed  PubMed Central  Google Scholar 

  96. Orsso CE, Butler AA, Muehlbauer MJ, Cui HN, Rubin DA, Pakseresht M, et al. Obestatin and adropin in Prader-Willi syndrome and nonsyndromic obesity: associations with weight, BMI-z, and HOMA-IR. Pediatr Obes. 2019;14(5):e12493.

    Article  CAS  PubMed  Google Scholar 

  97. Johnson RD. Opioid involvement in feeding behaviour and the pathogenesis of certain eating disorders. Med Hypotheses. 1995;45(5):491–7.

    Article  CAS  PubMed  Google Scholar 

  98. Margules DL, Inturrisi CE. Beta-endorphin immunoreactivity in the plasma of patients with the Prader-Labhart-Willi syndrome and their normal siblings. Experientia. 1983;39(7):766–7.

    Article  CAS  PubMed  Google Scholar 

  99. Butler MG, Nelson TA, Driscoll DJ, Manzardo AM. Evaluation of plasma substance P and beta-endorphin levels in children with Prader-Willi Syndrome. J Rare Disord. 2015;3(2)

    Google Scholar 

  100. Zipf WB, Berntson GG. Characteristics of abnormal food-intake patterns in children with Prader-Willi syndrome and study of effects of naloxone. Am J Clin Nutr. 1987;46(2):277–81.

    Article  CAS  PubMed  Google Scholar 

  101. Bueno G, Moreno LA, Pineda I, Campos J, Ruibal JL, Juste MG, et al. Serum leptin concentrations in children with Prader-Willi syndrome and non-syndromal obesity. J Pediatr Endocrinol Metab. 2000;13(4):425–30.

    Article  CAS  PubMed  Google Scholar 

  102. Butler MG, Moore J, Morawiecki A, Nicolson M. Comparison of leptin protein levels in Prader-Willi syndrome and control individuals. Am J Med Genet. 1998;75(1):7–12.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  103. Carlson MG, Snead WL, Oeser AM, Butler MG. Plasma leptin concentrations in lean and obese human subjects and Prader-Willi syndrome: comparison of RIA and ELISA methods. J Lab Clin Med. 1999;133(1):75–80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  104. Pietrobelli A, Allison DB, Faith MS, Beccaria L, Bosio L, Chiumello G, et al. Prader-Willi syndrome: relationship of adiposity to plasma leptin levels. Obes Res. 1998;6(3):196–201.

    Article  CAS  PubMed  Google Scholar 

  105. Elimam A, Lindgren AC, Norgren S, Kamel A, Skwirut C, Bang P, et al. Growth hormone treatment downregulates serum leptin levels in children independent of changes in body mass index. Horm Res. 1999;52(2):66–72.

    CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  107. Myers SE, Davis A, Whitman BY, Santiago JV, Landt M. Leptin concentrations in Prader-Willi syndrome before and after growth hormone replacement. Clin Endocrinol. 2000;52(1):101–5.

    Article  CAS  Google Scholar 

  108. Johnson L, Manzardo AM, Miller JL, Driscoll DJ, Butler MG. Elevated plasma oxytocin levels in children with Prader-Willi syndrome compared with healthy unrelated siblings. Am J Med Genet A. 2016;170(3):594–601.

    Article  CAS  PubMed  Google Scholar 

  109. Rice LJ, Einfeld SL, Hu N, Carter CS. A review of clinical trials of oxytocin in Prader-Willi syndrome. Curr Opin Psychiatry. 2018;31(2):123–7.

    Article  PubMed  Google Scholar 

  110. Damen L, Grootjen LN, Juriaans AF, Donze SH, Huisman TM, Visser JA, et al. Oxytocin in young children with Prader-Willi syndrome: results of a randomized, double-blind, placebo-controlled, crossover trial investigating 3 months of oxytocin. Clin Endocrinol. 2021;94(5):774–85.

    Article  CAS  Google Scholar 

  111. Holland A, Whittington J, Hinton E. The paradox of Prader-Willi syndrome: a genetic model of starvation. Lancet. 2003;362(9388):989–91.

    Article  CAS  PubMed  Google Scholar 

  112. PDK L. Model for a peripheral signalling defect in Prader-Willi syndrome. In: Eiholzer U LAD, Zipf WB, editor. Prader Willi Syndrome as a model for obesity. Basel: Karger; 2003. p. 70–81.

    Google Scholar 

  113. Schoeller DA, Levitsky LL, Bandini LG, Dietz WW, Walczak A. Energy expenditure and body composition in Prader-Willi syndrome. Metabolism. 1988;37(2):115–20.

    Article  CAS  PubMed  Google Scholar 

  114. Hill JO, Kaler M, Spetalnick B, Reed G, Butler MG. Resting metabolic rate in Prader-Willi Syndrome. Dysmorphol Clin Genet. 1990;4(1):27–32.

    PubMed  PubMed Central  Google Scholar 

  115. van Mil EA, Westerterp KR, Gerver WJ, Curfs LM, Schrander-Stumpel CT, Kester AD, et al. Energy expenditure at rest and during sleep in children with Prader-Willi syndrome is explained by body composition. Am J Clin Nutr. 2000;71(3):752–6.

    Article  PubMed  Google Scholar 

  116. Bekx MT, Carrel AL, Shriver TC, Li Z, Allen DB. Decreased energy expenditure is caused by abnormal body composition in infants with Prader-Willi Syndrome. J Pediatr. 2003;143(3):372–6.

    Article  PubMed  Google Scholar 

  117. Goldstone AP, Brynes AE, Thomas EL, Bell JD, Frost G, Holland A, 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. Am J Clin Nutr. 2002;75(3):468–75.

    Article  CAS  PubMed  Google Scholar 

  118. Nardella MT, Sulzbacher SI, Worthington-Roberts BS. Activity levels of persons with Prader-Willi syndrome. Am J Ment Defic. 1983;87(5):498–505.

    CAS  PubMed  Google Scholar 

  119. Nelson RA, Anderson LF, Gastineau CF, Hayles AB, Stamnes CL. Physiology and natural history of obesity. JAMA. 1973;223(6):627–30.

    Article  CAS  PubMed  Google Scholar 

  120. Purtell L, Viardot A, Sze L, Loughnan G, Steinbeck K, Sainsbury A, et al. Postprandial metabolism in adults with Prader-Willi syndrome. Obesity (Silver Spring). 2015;23(6):1159–65.

    Article  CAS  Google Scholar 

  121. Davies PS, Joughin C. Using stable isotopes to assess reduced physical activity of individuals with Prader-Willi syndrome. Am J Ment Retard. 1993;98(3):349–53.

    CAS  PubMed  Google Scholar 

  122. L'Allemand D, Eiholzer U, Rousson V, Girard J, Blum W, Torresani T, et al. Increased adrenal androgen levels in patients with Prader-Willi syndrome are associated with insulin, IGF-I, and leptin, but not with measures of obesity. Horm Res. 2002;58(5):215–22.

    CAS  PubMed  Google Scholar 

  123. Lee PD. Effects of growth hormone treatment in children with Prader-Willi syndrome. Growth Hormon IGF Res. 2000;10 Suppl B:S75–9.

    Google Scholar 

  124. Schmidt H, Schwarz HP. Premature adrenarche, increased growth velocity and accelerated bone age in male patients with Prader-Labhart-Willi syndrome. Eur J Pediatr. 2001;160(1):69–70.

    Article  CAS  PubMed  Google Scholar 

  125. Crinò A, Grugni G. Update on diabetes mellitus and glucose metabolism alterations in Prader-Willi Syndrome. Curr Diab Rep. 2020;20(2):7.

    Article  PubMed  Google Scholar 

  126. Eiholzer U, Stutz K, Weinmann C, Torresani T, Molinari L, Prader A. Low insulin, IGF-I and IGFBP-3 levels in children with Prader-Labhart-Willi syndrome. Eur J Pediatr. 1998;157(11):890–3.

    Article  CAS  PubMed  Google Scholar 

  127. Mogul HR, Lee PD, Whitman BY, Zipf WB, Frey M, Myers S, et al. Growth hormone treatment of adults with Prader-Willi syndrome and growth hormone deficiency improves lean body mass, fractional body fat, and serum triiodothyronine without glucose impairment: results from the United States multicenter trial. J Clin Endocrinol Metab. 2008;93(4):1238–45.

    Article  CAS  PubMed  Google Scholar 

  128. Zipf WB. Glucose homeostasis in Prader-Willi syndrome and potential implications of growth hormone therapy. Acta Paediatr Suppl. 1999;88(433):115–7.

    Article  CAS  PubMed  Google Scholar 

  129. Goldstone AP, Thomas EL, Brynes AE, Bell JD, Frost G, Saeed N, et al. Visceral adipose tissue and metabolic complications of obesity are reduced in Prader-Willi syndrome female adults: evidence for novel influences on body fat distribution. J Clin Endocrinol Metab. 2001;86(9):4330–8.

    Article  CAS  PubMed  Google Scholar 

  130. Thomas EL, Saeed N, Hajnal JV, Brynes A, Goldstone AP, Frost G, et al. Magnetic resonance imaging of total body fat. J Appl Physiol (1985). 1998;85(5):1778–85.

    Article  CAS  Google Scholar 

  131. Yigit S, Estrada E, Bucci K, Hyams J, Rosengren S. Diabetic ketoacidosis secondary to growth hormone treatment in a boy with Prader-Willi syndrome and steatohepatitis. J Pediatr Endocrinol Metab. 2004;17(3):361–4.

    Article  PubMed  Google Scholar 

  132. American Academy of Pediatrics, Kleinman RE, Greer FR. Pediatric nutrition : policy of the American Academy of Pediatrics. 7th ed. Elk Grove Village: American Academy of Pediatrics; 2014. xxvi, 1477 pages

    Google Scholar 

  133. Holm VA, Pipes PL. Food and children with Prader-Willi syndrome. Am J Dis Child. 1976;130(10):1063–7.

    CAS  PubMed  Google Scholar 

  134. Pipes PL, Holm VA. Weight control of children with Prader-Willi syndrome. J Am Diet Assoc. 1973;62(5):520–4.

    Article  CAS  PubMed  Google Scholar 

  135. Forbes GB. Body fat content influences the body composition response to nutrition and exercise. Ann N Y Acad Sci. 2000;904:359–65.

    Article  CAS  PubMed  Google Scholar 

  136. Bistrian BR, Blackburn GL, Stanbury JB. Metabolic aspects of a protein-sparing modified fast in the dietary management of Prader-Willi obesity. N Engl J Med. 1977;296(14):774–9.

    Article  CAS  PubMed  Google Scholar 

  137. Collier SB, Walker WA. Parenteral protein-sparing modified fast in an obese adolescent with Prader-Willi syndrome. Nutr Rev. 1991;49(8):235–8.

    Article  CAS  PubMed  Google Scholar 

  138. Anderson GH, Moore SE. Dietary proteins in the regulation of food intake and body weight in humans. J Nutr. 2004;134(4):974S–9S.

    Article  CAS  PubMed  Google Scholar 

  139. Buchholz AC, Schoeller DA. Is a calorie a calorie? Am J Clin Nutr. 2004;79(5):899S–906S.

    Article  CAS  PubMed  Google Scholar 

  140. Kohanmoo A, Faghih S, Akhlaghi M. Effect of short- and long-term protein consumption on appetite and appetite-regulating gastrointestinal hormones, a systematic review and meta-analysis of randomized controlled trials. Physiol Behav. 2020;226:113123.

    Article  CAS  PubMed  Google Scholar 

  141. Alsaif M, Triador L, Colin-Ramirez E, Elliott S, Mackenzie ML, Field CJ, et al. Effect of high-protein diet on postprandial energy expenditure in children with Prader-Willi Syndrome: a pilot and feasibility study. Curr Dev Nutr. 2021;5(3):nzab016.

    Article  Google Scholar 

  142. Alsaif M, Pakseresht M, Mackenzie ML, Gaylinn B, Thorner MO, Freemark M, et al. Dietary macronutrient regulation of acyl and desacyl ghrelin concentrations in children with Prader-Willi syndrome (PWS). Clin Endocrinol. 2020;93(5):579–89.

    Article  CAS  Google Scholar 

  143. Felix G, Kossoff E, Barron B, Krekel C, Testa EG, Scheimann A. The modified Atkins diet in children with Prader-Willi syndrome. Orphanet J Rare Dis. 2020;15(1):135.

    Article  PubMed  PubMed Central  Google Scholar 

  144. Mogul HR, Peterson SJ, Weinstein BI, Zhang S, Southren AL. Metformin and carbohydrate-modified diet: a novel obesity treatment protocol: preliminary findings from a case series of nondiabetic women with midlife weight gain and hyperinsulinemia. Heart Dis. 2001;3(5):285–92.

    Article  CAS  PubMed  Google Scholar 

  145. Mogul HR, Peterson SJ, Weinstein BI, Li J, Southren AL. Long-term (2-4 year) weight reduction with metformin plus carbohydrate-modified diet in euglycemic, hyperinsulinemic, midlife women (Syndrome W). Heart Dis. 2003;5(6):384–92.

    Article  CAS  PubMed  Google Scholar 

  146. Kohn Y, Weizman A, Apter A. Aggravation of food-related behavior in an adolescent with Prader-Willi syndrome treated with fluvoxamine and fluoxetine. Int J Eat Disord. 2001;30(1):113–7.

    Article  CAS  PubMed  Google Scholar 

  147. Smathers SA, Wilson JG, Nigro MA. Topiramate effectiveness in Prader-Willi syndrome. Pediatr Neurol. 2003;28(2):130–3.

    Article  PubMed  Google Scholar 

  148. Chan NN, Feher MD, Bridges NA. Metformin therapy for diabetes in Prader-Willi syndrome. J R Soc Med. 1998;91(11):598.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  149. Miller JL, Linville TD, Dykens EM. Effects of metformin in children and adolescents with Prader-Willi syndrome and early-onset morbid obesity: a pilot study. J Pediatr Endocrinol Metab. 2014;27(1–2):23–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  150. Avenell A, Broom J, Brown TJ, Poobalan A, Aucott L, Stearns SC, et al. Systematic review of the long-term effects and economic consequences of treatments for obesity and implications for health improvement. Health Technol Assess. 2004;8(21):iii-iv, 1-182.

    Article  PubMed  Google Scholar 

  151. Wilson DM, Abrams SH, Aye T, Lee PD, Lenders C, Lustig RH, et al. Metformin extended release treatment of adolescent obesity: a 48-week randomized, double-blind, placebo-controlled trial with 48-week follow-up. Arch Pediatr Adolesc Med. 2010;164(2):116–23.

    PubMed  Google Scholar 

  152. Dykens EM, Miller J, Angulo M, Roof E, Reidy M, Hatoum HT, et al. Intranasal carbetocin reduces hyperphagia in individuals with Prader-Willi syndrome. JCI Insight. 2018;3(12):e98333.

    Article  PubMed Central  Google Scholar 

  153. Salehi P, Hsu I, Azen CG, Mittelman SD, Geffner ME, Jeandron D. Effects of exenatide on weight and appetite in overweight adolescents and young adults with Prader-Willi syndrome. Pediatr Obes. 2017;12(3):221–8.

    Article  CAS  PubMed  Google Scholar 

  154. McCandless SE, Yanovski JA, Miller J, Fu C, Bird LM, Salehi P, et al. Effects of MetAP2 inhibition on hyperphagia and body weight in Prader-Willi syndrome: a randomized, double-blind, placebo-controlled trial. Diabetes Obes Metab. 2017;19(12):1751–61.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  155. Kimonis V, Surampalli A, Wencel M, Gold JA, Cowen NM. A randomized pilot efficacy and safety trial of diazoxide choline controlled-release in patients with Prader-Willi syndrome. PLoS One. 2019;14(9):e0221615.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  156. Azevedo CC, Trevizol AP, Gomes JS, Akiba H, Franco RR, Simurro PB, et al. Transcranial direct current stimulation for Prader-Willi Syndrome. J ECT. 2021;37(1):58–63.

    Article  PubMed  Google Scholar 

  157. Anderson AE, Soper RT, Scott DH. Gastric bypass for morbid obesity in children and adolescents. J Pediatr Surg. 1980;15(6):876–81.

    Article  CAS  PubMed  Google Scholar 

  158. Soper RT, Mason EE, Printen KJ, Zellweger H. Gastric bypass for morbid obesity in children and adolescents. J Pediatr Surg. 1975;10(1):51–8.

    Article  CAS  PubMed  Google Scholar 

  159. Fonkalsrud EW, Bray G. Vagotomy for treatment of obesity in childhood due to Prader-Willi syndrome. J Pediatr Surg. 1981;16(6):888–9.

    Article  CAS  PubMed  Google Scholar 

  160. Touquet VL, Ward MW, Clark CG. Obesity surgery in a patient with the Prader-Willi syndrome. Br J Surg. 1983;70(3):180–1.

    Article  CAS  PubMed  Google Scholar 

  161. Laurent-Jaccard A, Hofstetter JR, Saegesser F, Chapuis GG. Long-term result of treatment of Prader-Willi Syndrome by Scopinaro's Bilio-pancreatic diversion. Study of three cases and the effect of Dextrofenfluramine on the postoperative evolution. Obes Surg. 1991;1(1):83–7.

    Article  CAS  PubMed  Google Scholar 

  162. Dousei T, Miyata M, Izukura M, Harada T, Kitagawa T, Matsuda H. Long-term follow-up of Gastroplasty in a patient with Prader-Willi Syndrome. Obes Surg. 1992;2(2):189–93.

    Article  CAS  PubMed  Google Scholar 

  163. Chelala E, Cadiére GB, Favretti F, Himpens J, Vertruyen M, Bruyns J, et al. Conversions and complications in 185 laparoscopic adjustable silicone gastric banding cases. Surg Endosc. 1997;11(3):268–71.

    Article  CAS  PubMed  Google Scholar 

  164. Grugni G, Guzzaloni G, Morabito F. Failure of biliopancreatic diversion in Prader-Willi syndrome. Obes Surg. 2000;10(2):179–81. discussion 82

    Article  CAS  PubMed  Google Scholar 

  165. Marinari GM, Camerini G, Novelli GB, Papadia F, Murelli F, Marini P, et al. Outcome of biliopancreatic diversion in subjects with Prader-Willi Syndrome. Obes Surg. 2001;11(4):491–5.

    Article  CAS  PubMed  Google Scholar 

  166. Braghetto I, Rodríguez A, Debandi A, Brunet L, Papapietro K, Pineda P, et al. Prader-Willi Syndrome (PWS) associated to morbid obesity: surgical treatment. Rev Med Chil. 2003;131(4):427–31.

    PubMed  Google Scholar 

  167. Kobayashi J, Kodama M, Yamazaki K, Morikawa O, Murano S, Kawamata N, et al. Gastric bypass in a Japanese man with Prader-Willi syndrome and morbid obesity. Obes Surg. 2003;13(5):803–5.

    Article  PubMed  Google Scholar 

  168. Musella M, Milone M, Leongito M, Maietta P, Bianco P, Pisapia A. The mini-gastric bypass in the management of morbid obesity in Prader-Willi syndrome: a viable option? J Investig Surg. 2014;27(2):102–5.

    Article  Google Scholar 

  169. Alqahtani AR, Elahmedi MO, Al Qahtani AR, Lee J, Butler MG. Laparoscopic sleeve gastrectomy in children and adolescents with Prader-Willi syndrome: a matched-control study. Surg Obes Relat Dis. 2016;12(1):100–10.

    Article  PubMed  Google Scholar 

  170. Cazzo E, Gestic MA, Utrini MP, Chaim FDM, Cândido EC, Jarolavsky LBDS, et al. Bariatric surgery in individuals with severe cognitive impairment: report of two cases. Sao Paulo Med J. 2018;136(1):84–8.

    Article  PubMed  Google Scholar 

  171. Tripodi M, Casertano A, Peluso M, Musella M, Berardi G, Mozzillo E, et al. Prader-Willi syndrome: role of bariatric surgery in two adolescents with obesity. Obes Surg. 2020;30(11):4602–4.

    Article  PubMed  Google Scholar 

  172. Liu SY, Wong SK, Lam CC, Ng EK. Bariatric surgery for Prader-Willi syndrome was ineffective in producing sustainable weight loss: long term results for up to 10 years. Pediatr Obes. 2020;15(1):e12575.

    Article  PubMed  Google Scholar 

  173. Scheimann AO, Butler MG, Gourash L, Cuffari C, Klish W. Critical analysis of bariatric procedures in Prader-Willi syndrome. J Pediatr Gastroenterol Nutr. 2008;46(1):80–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  174. Saenger P, Dimartino-Nardi J. Premature adrenarche. J Endocrinol Investig. 2001;24(9):724–33.

    Article  CAS  Google Scholar 

  175. LaMoia TE, Shulman GI. Cellular and molecular mechanisms of metformin action. Endocr Rev. 2021;42(1):77–96.

    Article  PubMed  Google Scholar 

  176. Bassali R, Hoffman WH, Chen H, Tuck-Muller CM. Hyperlipidemia, insulin-dependent diabetes mellitus, and rapidly progressive diabetic retinopathy and nephropathy in Prader-Willi syndrome with del(15)(q11.2q13). Am J Med Genet. 1997;71(3):267–70.

    Article  CAS  PubMed  Google Scholar 

  177. Walker JD, Warren RE. Necrobiosis lipoidica in Prader- Willi-associated diabetes mellitus. Diabet Med. 2002;19(10):884–5.

    Article  CAS  PubMed  Google Scholar 

  178. Bedogni G, Grugni G, Nobili V, Agosti F, Saezza A, Sartorio A. Is non-alcoholic fatty liver disease less frequent among women with Prader-Willi syndrome? Obes Facts. 2014;7(1):71–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  179. Butler MG, Meaney FJ. Standards for selected anthropometric measurements in Prader-Willi syndrome. Pediatrics. 1991;88(4):853–60.

    CAS  PubMed  Google Scholar 

  180. Hauffa BP, Schlippe G, Roos M, Gillessen-Kaesbach G, Gasser T. Spontaneous growth in German children and adolescents with genetically confirmed Prader-Willi syndrome. Acta Paediatr. 2000;89(11):1302–11.

    Article  CAS  PubMed  Google Scholar 

  181. Nagai T, Matsuo N, Kayanuma Y, Tonoki H, Fukushima Y, Ohashi H, et al. Standard growth curves for Japanese patients with Prader-Willi syndrome. Am J Med Genet. 2000;95(2):130–4.

    Article  CAS  PubMed  Google Scholar 

  182. Fehlow P, Miosge W, Scholl U, Walther F. Prader-Willi syndrome in association with self-injury behavior and premature craniosynostosis. Klin Padiatr. 1996;208(3):135–6.

    Article  CAS  PubMed  Google Scholar 

  183. Butler MG, Butler RI, Meaney FJ. The use of skinfold measurements to judge obesity during the early phase of Prader-Labhart-Willi syndrome. Int J Obes. 1988;12(5):417–22.

    CAS  PubMed  PubMed Central  Google Scholar 

  184. Hauffa BP, Schlippe G, Gillessen-Kaesbach G. Adiposity indices in German children and adolescents with genetically confirmed Prader-Willi syndrome (PWS). Int J Obes Relat Metab Disord. 2001;25(Suppl 1):S22–5.

    Article  PubMed  Google Scholar 

  185. Wang J, Thornton JC, Kolesnik S, Pierson RN. Anthropometry in body composition. An overview. Ann N Y Acad Sci. 2000;904:317–26.

    Article  CAS  PubMed  Google Scholar 

  186. Ellis KJ. Human body composition: in vivo methods. Physiol Rev. 2000;80(2):649–80.

    Article  CAS  PubMed  Google Scholar 

  187. Wang ZM, Pierson RN, Heymsfield SB. The five-level model: a new approach to organizing body-composition research. Am J Clin Nutr. 1992;56(1):19–28.

    Article  CAS  PubMed  Google Scholar 

  188. Wang ZM, Heshka S, Pierson RN, Heymsfield SB. Systematic organization of body-composition methodology: an overview with emphasis on component-based methods. Am J Clin Nutr. 1995;61(3):457–65.

    Article  CAS  PubMed  Google Scholar 

  189. Lohman TG, Hingle M, Going SB. Body composition in children. Pediatr Exerc Sci. 2013;25(4):573–90.

    Article  PubMed  Google Scholar 

  190. Fields DA, Goran MI, McCrory MA. Body-composition assessment via air-displacement plethysmography in adults and children: a review. Am J Clin Nutr. 2002;75(3):453–67.

    Article  CAS  PubMed  Google Scholar 

  191. Schoeller D. Hydrometry. In: Roche AFHS, Lohman T, editors. Human body composition. Champaign; 1996. p. 25–44.

    Google Scholar 

  192. Ellis KJ, Shypailo RJ. Whole body potassium measurements independent of body size. Basic Life Sci. 1993;60:371–5.

    CAS  PubMed  Google Scholar 

  193. Murphy AJ, Ellis KJ, Kurpad AV, Preston T, Slater C. Total body potassium revisited. Eur J Clin Nutr. 2014;68(2):153–4.

    Article  CAS  PubMed  Google Scholar 

  194. Guo SM, Roche AF, Chumlea WC, Miles DS, Pohlman RL. Body composition predictions from bioelectric impedance. Hum Biol. 1987;59(2):221–33.

    CAS  PubMed  Google Scholar 

  195. R B. Electrical impedance and total body electrical conductivity. Human Kinetics 1996:79–108.

    Google Scholar 

  196. VanderJagt DJ, Harmatz P, Scott-Emuakpor AB, Vichinsky E, Glew RH. Bioelectrical impedance analysis of the body composition of children and adolescents with sickle cell disease. J Pediatr. 2002;140(6):681–7.

    Article  PubMed  Google Scholar 

  197. Shypailo RJ, Motil KJ. The use of bioimpedance in pediatric health, nutrition, and disease. J Pediatr Gastroenterol Nutr. 2018;67(4):435–6.

    Article  PubMed  Google Scholar 

  198. Kyle UG, Earthman CP, Pichard C, Coss-Bu JA. Body composition during growth in children: limitations and perspectives of bioelectrical impedance analysis. Eur J Clin Nutr. 2015;69(12):1298–305.

    Article  CAS  PubMed  Google Scholar 

  199. Streb AR, Hansen F, Gabiatti MP, Tozetto WR, Del Duca GF. Phase angle associated with different indicators of health-related physical fitness in adults with obesity. Physiol Behav. 2020;225:113104.

    Article  CAS  PubMed  Google Scholar 

  200. Taicher GZ, Tinsley FC, Reiderman A, Heiman ML. Quantitative magnetic resonance (QMR) method for bone and whole-body-composition analysis. Anal Bioanal Chem. 2003;377(6):990–1002.

    Article  CAS  PubMed  Google Scholar 

  201. Tinsley FC, Taicher GZ, Heiman ML. Evaluation of a quantitative magnetic resonance method for mouse whole body composition analysis. Obes Res. 2004;12(1):150–60.

    Article  PubMed  Google Scholar 

  202. Mitchell AD. Validation of quantitative magnetic resonance body composition analysis for infants using piglet model. Pediatr Res. 2011;69(4):330–5.

    Article  PubMed  Google Scholar 

  203. Napolitano A, Miller SR, Murgatroyd PR, Coward WA, Wright A, Finer N, et al. Validation of a quantitative magnetic resonance method for measuring human body composition. Obesity (Silver Spring). 2008;16(1):191–8.

    Article  Google Scholar 

  204. Bosy-Westphal A, Müller MJ. Assessment of fat and lean mass by quantitative magnetic resonance: a future technology of body composition research? Curr Opin Clin Nutr Metab Care. 2015;18(5):446–51.

    Article  PubMed  Google Scholar 

  205. Heard-Lipsmeyer ME, Hull H, Sims CR, Cleves MA, Andres A. Evaluating body composition in infancy and childhood: a comparison between 4C, QMR, DXA, and ADP. Pediatr Obes. 2020;15(6):e12617.

    Article  PubMed  PubMed Central  Google Scholar 

  206. Bredella MA, Gill CM, Keating LK, Torriani M, Anderson EJ, Punyanitya M, et al. Assessment of abdominal fat compartments using DXA in premenopausal women from anorexia nervosa to morbid obesity. Obesity (Silver Spring). 2013;21(12):2458–64.

    Article  CAS  Google Scholar 

  207. Brenner DJ, Elliston CD. Estimated radiation risks potentially associated with full-body CT screening. Radiology. 2004;232(3):735–8.

    Article  PubMed  Google Scholar 

  208. Starck G, Lönn L, Cederblad A, Alpsten M, Sjöström L, Ekholm S. Dose reduction for body composition measurements with CT. Appl Radiat Isot. 1998;49(5–6):561–3.

    Article  CAS  PubMed  Google Scholar 

  209. Seeram E. Computed tomography: a technical review. Radiol Technol. 2018;89(3):279CT-302CT.

    Google Scholar 

  210. Machann J, Bachmann OP, Brechtel K, Dahl DB, Wietek B, Klumpp B, et al. Lipid content in the musculature of the lower leg assessed by fat selective MRI: intra- and interindividual differences and correlation with anthropometric and metabolic data. J Magn Reson Imaging. 2003;17(3):350–7.

    Article  PubMed  Google Scholar 

  211. Schick F, Machann J, Brechtel K, Strempfer A, Klumpp B, Stein DT, et al. MRI of muscular fat. Magn Reson Med. 2002;47(4):720–7.

    Article  PubMed  Google Scholar 

  212. Jacob S, Machann J, Rett K, Brechtel K, Volk A, Renn W, et al. Association of increased intramyocellular lipid content with insulin resistance in lean nondiabetic offspring of type 2 diabetic subjects. Diabetes. 1999;48(5):1113–9.

    Article  CAS  PubMed  Google Scholar 

  213. Treuth MS, Butte NF, Wong WW, Ellis KJ. Body composition in prepubertal girls: comparison of six methods. Int J Obes Relat Metab Disord. 2001;25(9):1352–9.

    Article  CAS  PubMed  Google Scholar 

  214. Wong WW, Hergenroeder AC, Stuff JE, Butte NF, Smith EO, Ellis KJ. Evaluating body fat in girls and female adolescents: advantages and disadvantages of dual-energy X-ray absorptiometry. Am J Clin Nutr. 2002;76(2):384–9.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatrics, Johns Hopkins School of Medicine, Pediatrics, Baltimore, MD, USA

    Ann O. Scheimann

  2. Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA

    Roman Shypailo

  3. Department of Pediatrics, University of Texas Medical Branch, Galveston, TX, USA

    Phillip D. K. Lee

Authors
  1. Ann O. Scheimann
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Roman Shypailo
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Phillip D. K. Lee
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Phillip D. K. Lee .

Editor information

Editors and Affiliations

  1. Departments of Psychiatry & Behavioral Sciences and Pediatrics, University of Kansas Medical Center, Kansas City, KS, USA

    Merlin G. Butler

  2. Department of Pediatrics, University of Texas Medical Branch, Galveston, TX, USA

    Phillip D. K. Lee

  3. Department of Pediatrics, Saint Louis University, St. Louis, MO, USA

    Barbara Y. Whitman

Rights and permissions

Reprints and permissions

Copyright information

© 2022 The Author(s), under exclusive license to Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Scheimann, A.O., Shypailo, R., Lee, P.D.K. (2022). Gastrointestinal System, Obesity, and Body Composition. In: Butler, M.G., Lee, P.D.K., Whitman, B.Y. (eds) Management of Prader-Willi Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-98171-6_6

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-98171-6_6

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-98170-9

  • Online ISBN: 978-3-030-98171-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation