Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Original Article
  • Published:

Clinical Studies and Practice

Comparison of body composition, basal metabolic rate and metabolic outcomes of adults with Prader Willi syndrome or lesional hypothalamic disease, with primary obesity

International Journal of Obesity volume 37, pages 1198–1203 (2013)Cite this article

Subjects

Abstract

Context:

The care of patients with hypothalamic obesity is challenging.

Objective:

To compare body composition, basal metabolic rate (BMR) and metabolic outcomes of adults, with lesional or genetic hypothalamic obesity, with obese patients suffering from primary obesity, once matched for body mass index (BMI).

Design and patients:

Adults with hypothalamic obesity of genetic origin (Prader Willi syndrome (PWS)) or acquired hypothalamic damage (HD), such as craniopharygioma, were compared with obese control candidates awaiting bariatric surgery (C), with a BMI between 35 and 65 kg m−2, and aged between 18 and 50 years.

Main outcome measures:

Body composition measured by whole-body dual-energy X-ray absorptiometry scanning, BMR using indirect calorimetry, hormonal and metabolic assessments.

Results:

A total of 27 adults with a genetic diagnosis of PWS, 15 obese subjects with HD and 206 obese controls with similar BMI were studied. Compared with the control group, PWS patients had an increased percentage of fat mass (FM), and a decreased percentage of android FM. The BMR of PWS patients was significantly lower than controls and highly correlated with lean body mass in PWS and C patients. Body composition of HD was similar with those of obese patients. A trend toward an increased prevalence of diabetes in HD patients and of cytolysis in PWS was observed in comparison with primary obese patients.

Conclusion:

Genetic and lesional hypothalamic obesities have different consequences for phenotypic features such as body composition or BMR compared with primary obese patients. The mechanisms of adipose tissue development and metabolic complications may be different between genetic and lesional obesities.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Similar content being viewed by others

References

  1. Hochberg I, Hochberg Z . Expanding the definition of hypothalamic obesity. Obes Rev 2010; 11: 709–721.

    Article  CAS  Google Scholar 

  2. Bulow B, Attewell R, Hagmar L, Malmstrom P, Nordstrom CH, Erfurth EM . Postoperative prognosis in craniopharyngioma with respect to cardiovascular mortality, survival, and tumor recurrence. J Clin Endocrinol Metab 1998; 83: 3897–3904.

    CAS  PubMed  Google Scholar 

  3. Muller HL, Emser A, Faldum A, Bruhnken G, Etavard-Gorris N, Gebhardt U et al. Longitudinal study on growth and body mass index before and after diagnosis of childhood craniopharyngioma. J Clin Endocrinol Metab 2004; 89: 3298–3305.

    Article  Google Scholar 

  4. Gautier A, Godbout A, Grosheny C, Tejedor I, Coudert M, Courtillot C et al. Markers of recurrence and long-term morbidity in craniopharyngioma: a systematic analysis of 171 patients. J Clin Endocrinol Metab 2012; 97: 1258–1267.

    Article  CAS  Google Scholar 

  5. Holmer H, Pozarek G, Wirfalt E, Popovic V, Ekman B, Bjork J et al. Reduced energy expenditure and impaired feeding-related signals but not high energy intake reinforces hypothalamic obesity in adults with childhood onset craniopharyngioma. J Clin Endocrinol Metab 2010; 95: 5395–5402.

    Article  CAS  Google Scholar 

  6. Srinivasan S, Ogle GD, Garnett SP, Briody JN, Lee JW, Cowell CT . Features of the metabolic syndrome after childhood craniopharyngioma. J Clin Endocrinol Metab 2004; 89: 81–86.

    Article  CAS  Google Scholar 

  7. Goldstone AP, Holland AJ, Hauffa BP, Hokken-Koelega AC, Tauber M . Recommendations for the diagnosis and management of Prader-Willi syndrome. J Clin Endocrinol Metab 2008; 93: 4183–4197.

    Article  CAS  Google Scholar 

  8. Sode-Carlsen R, Farholt S, Rabben KF, Bollerslev J, Schreiner T, Jurik AG et al. Body composition, endocrine and metabolic profiles in adults with Prader-Willi syndrome. Growth Horm IGF Res 2010; 20: 179–184.

    Article  CAS  Google Scholar 

  9. Schuster DP, Osei K, Zipf WB . Characterization of alterations in glucose and insulin metabolism in Prader-Willi subjects. Metabolism 1996; 45: 1514–1520.

    Article  CAS  Google Scholar 

  10. Ciangura C, Bouillot JL, Lloret-Linares C, Poitou C, Veyrie N, Basdevant A et al. Dynamics of change in total and regional body composition after gastric bypass in obese patients. Obesity (Silver Spring) 2010; 18: 760–765.

    Article  Google Scholar 

  11. Talebizadeh Z, Butler MG . Insulin resistance and obesity-related factors in Prader-Willi syndrome: comparison with obese subjects. Clin Genet 2005; 67: 230–239.

    Article  CAS  Google Scholar 

  12. Frankenfield D, Roth-Yousey L, Compher C . Comparison of predictive equations for resting metabolic rate in healthy nonobese and obese adults: a systematic review. J Am Diet Assoc 2005; 105: 775–789.

    Article  Google Scholar 

  13. Alberti KG, Zimmet PZ . Definition, diagnosis and classification of diabetes mellitus and its complications. Part 1: diagnosis and classification of diabetes mellitus provisional report of a WHO consultation. Diabet Med 1998; 15: 539–553.

    Article  CAS  Google Scholar 

  14. Graham I, Atar D, Borch-Johnsen K, Boysen G, Burell G, Cifkova R et al. European guidelines on cardiovascular disease prevention in clinical practice: full text. Fourth Joint Task Force of the European Society of Cardiology and other societies on cardiovascular disease prevention in clinical practice (constituted by representatives of nine societies and by invited experts). Eur J Cardiovasc Prev Rehabil 2007; 14 (Suppl 2): S1–113.

    Article  Google Scholar 

  15. National Institute of Health Publications. National Cholesterol Education Program. Detection, Evaluation and Treatment of High Blood Cholesterol in Adults (Adult Treatment Panel III). No. 02-5215, September 2002.

  16. Matthews DR, Hosker JP, Rudenski AS, Naylor BA, Treacher DF, Turner RC . Homeostasis model assessment: insulin resistance and beta-cell function from fasting plasma glucose and insulin concentrations in man. Diabetologia 1985; 28: 412–419.

    Article  CAS  Google Scholar 

  17. Angulo P . Nonalcoholic fatty liver disease. N Engl J Med 2002; 346: 1221–1231.

    Article  CAS  Google Scholar 

  18. 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: 1369–1374.

    Article  CAS  Google Scholar 

  19. van Mil EG, Westerterp KR, Gerver WJ, Van Marken Lichtenbelt WD, Kester AD, Saris WH . Body composition in Prader-Willi syndrome compared with nonsyndromal obesity: relationship to physical activity and growth hormone function. J Pediatr 2001; 139: 708–714.

    Article  CAS  Google Scholar 

  20. 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: 372–376.

    Article  Google Scholar 

  21. Theodoro MF, Talebizadeh Z, Butler MG . Body composition and fatness patterns in Prader-Willi syndrome: comparison with simple obesity. Obesity 2006; 14: 1685–1690.

    Article  Google Scholar 

  22. 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: 468–475.

    Article  CAS  Google Scholar 

  23. Marzullo P, Marcassa C, Campini R, Eleuteri E, Minocci A, Priano L et al. The impact of growth hormone/insulin-like growth factor-I axis and nocturnal breathing disorders on cardiovascular features of adult patients with Prader-Willi syndrome. J Clin Endocrinol Metab 2005; 90: 5639–5646.

    Article  CAS  Google Scholar 

  24. Kennedy L, Bittel DC, Kibiryeva N, Kalra SP, Torto R, Butler MG . Circulating adiponectin levels, body composition and obesity-related variables in Prader-Willi syndrome: comparison with obese subjects. Int J Obes 2006; 30: 382–387.

    Article  CAS  Google Scholar 

  25. Reus L, Zwarts M, van Vlimmeren LA, Willemsen MA, Otten BJ, Nijhuis-van der Sanden MW . Motor problems in Prader-Willi syndrome: a systematic review on body composition and neuromuscular functioning. Neurosci Biobehav Rev 2011; 35: 956–969.

    Article  CAS  Google Scholar 

  26. Eiholzer U, Grieser J, Schlumpf M, l'Allemand D . Clinical effects of treatment for hypogonadism in male adolescents with Prader-Labhart-Willi syndrome. Horm Res 2007; 68: 178–184.

    CAS  PubMed  Google Scholar 

  27. Crino A, Schiaffini R, Ciampalini P, Spera S, Beccaria L, Benzi F et al. Hypogonadism and pubertal development in Prader-Willi syndrome. Eur J Pediatr 2003; 162: 327–333.

    CAS  PubMed  Google Scholar 

  28. Allen K . Managing Prader-Willi syndrome in families: an embodied exploration. Soc Sci Med 2011; 72: 460–468.

    Article  Google Scholar 

  29. Kim RJ, Shah R, Tershakovec AM, Zemel BS, Sutton LN, Grimberg A et al. Energy expenditure in obesity associated with craniopharyngioma. Childs Nerv Syst 2010; 26: 913–917.

    Article  Google Scholar 

  30. Pinto G, Bussieres L, Recasens C, Souberbielle JC, Zerah M, Brauner R . Hormonal factors influencing weight and growth pattern in craniopharyngioma. Horm Res 2000; 53: 163–169.

    CAS  PubMed  Google Scholar 

  31. Lustig RH, Rose SR, Burghen GA, Velasquez-Mieyer P, Broome DC, Smith K et al. Hypothalamic obesity caused by cranial insult in children: altered glucose and insulin dynamics and reversal by a somatostatin agonist. J Pediatr 1999; 135 (2 Pt 1): 162–168.

    Article  CAS  Google Scholar 

  32. Muller HL . Childhood craniopharyngioma. Recent advances in diagnosis, treatment and follow-up. Horm Res 2008; 69: 193–202.

    PubMed  Google Scholar 

  33. Butler JV, Whittington JE, Holland AJ, McAllister CJ, Goldstone AP . The transition between the phenotypes of Prader-Willi syndrome during infancy and early childhood. Dev Med Child Neurol 2010; 52: e88–e93.

    Article  Google Scholar 

  34. Haqq AM, Farooqi IS, O'Rahilly S, Stadler DD, Rosenfeld RG, Pratt KL et al. Serum ghrelin levels are inversely correlated with body mass index, age, and insulin concentrations in normal children and are markedly increased in Prader-Willi syndrome. J Clin Endocrinol Metab 2003; 88: 174–178.

    Article  CAS  Google Scholar 

  35. van den Berg-Emons R, Festen D, Hokken-Koelega A, Bussmann J, Stam H . Everyday physical activity and adiposity in Prader-Willi syndrome. J Pediatr Endocrinol Metab 2008; 21: 1041–1048.

    Article  Google Scholar 

  36. Haqq AM, Muehlbauer MJ, Newgard CB, Grambow S, Freemark M . The metabolic phenotype of Prader-Willi syndrome (PWS) in childhood: heightened insulin sensitivity relative to body mass index. J Clin Endocrinol Metab 2011; 96: E225–E232.

    Article  CAS  Google Scholar 

  37. Hoffstedt J, Arner E, Wahrenberg H, Andersson DP, Qvisth V, Lofgren P et al. Regional impact of adipose tissue morphology on the metabolic profile in morbid obesity. Diabetologia 2010; 53: 2496–2503.

    Article  CAS  Google Scholar 

  38. Sinnema M, Maaskant MA, van Schrojenstein Lantman-de Valk HM, van Nieuwpoort IC, Drent ML, Curfs LM et al. Physical health problems in adults with Prader-Willi syndrome. Am J Med Genet A 2011; 155A: 2112–2124.

    Article  Google Scholar 

  39. 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: 4330–4338.

    Article  CAS  Google Scholar 

  40. Simoneau-Roy J, O'Gorman C, Pencharz P, Adeli K, Daneman D, Hamilton J . Insulin sensitivity and secretion in children and adolescents with hypothalamic obesity following treatment for craniopharyngioma. Clin Endocrinol 2010; 72: 364–370.

    Article  CAS  Google Scholar 

  41. Sahakitrungruang T, Klomchan T, Supornsilchai V, Wacharasindhu S . Obesity, metabolic syndrome, and insulin dynamics in children after craniopharyngioma surgery. Eur J Pediatr 2011; 170: 763–769.

    Article  CAS  Google Scholar 

  42. Lee M, Korner J . Review of physiology, clinical manifestations, and management of hypothalamic obesity in humans. Pituitary 2009; 12: 87–95.

    Article  CAS  Google Scholar 

  43. Crowley RK, Hamnvik OP, O'Sullivan EP, Behan LA, Smith D, Agha A et al. Morbidity and mortality in patients with craniopharyngioma after surgery. Clin Endocrinol 2010; 73: 516–521.

    CAS  Google Scholar 

  44. Iannuzzi MC, Fontana JR . Sarcoidosis: clinical presentation, immunopathogenesis, and therapeutics. JAMA 2011; 305: 391–399.

    Article  CAS  Google Scholar 

  45. Residori L, Garcia-Lorda P, Flancbaum L, Pi-Sunyer FX, Laferrere B . Prevalence of co-morbidities in obese patients before bariatric surgery: effect of race. Obes Surg 2003; 13: 333–340.

    Article  Google Scholar 

  46. Lionti T, Reid SM, Rowell MM . Prader-Willi syndrome in Victoria: mortality and causes of death. J Paediatr Child Health 2012; 48: 506–511.

    Article  Google Scholar 

  47. Grugni G, Crino A, Bosio L, Corrias A, Cuttini M, De Toni T et al. The Italian National Survey for Prader-Willi syndrome: an epidemiologic study. Am J Med Genet A 2008; 146: 861–872.

    Article  Google Scholar 

  48. Tauber M, Diene G, Molinas C, Hebert M . Review of 64 cases of death in children with Prader-Willi syndrome (PWS). Am J Med Genet A 2008; 146: 881–887.

    Article  Google Scholar 

  49. Vogels A, Van Den Ende J, Keymolen K, Mortier G, Devriendt K, Legius E et al. Minimum prevalence, birth incidence and cause of death for Prader-Willi syndrome in Flanders. Eur J Hum Genet 2004; 12: 238–240.

    Article  CAS  Google Scholar 

Download references

Acknowledgements

We thank Florence Marchelli (Center for Research on Human Nutrition, Pitié-Salpêtrière Hospital, Paris, France), who was involved in data base management of the control group. We sincerely thank patients with hypothalamic obesity (PWS or HD), caregivers and their families for their participation in this study.

Author information

Author notes

  1. C Lloret-Linares and P Faucher: These authors contributed equally to this work.

Authors and Affiliations

  1. Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris, Hôpital Lariboisière, Unit of Therapeutic Research, Paris, France

    C Lloret-Linares

  2. INSERM U705, CNRS UMR 8206, Neuropsychopharmacology of Drug Addiction, Faculty of Pharmacy, University Paris Descartes, Paris, France

    C Lloret-Linares

  3. Nutrition and Endocrinology Department, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, French Reference Center for Prader Willi Syndrome, Paris, France

    P Faucher, M Coupaye, R Alili, A Basdevant, K Clément & C Poitou

  4. CRNH-Ile de France, Paris, France

    P Faucher, M Coupaye, R Alili, A Basdevant, K Clément & C Poitou

  5. Institute of CArdiometabolism and Nutrition (ICAN), Paris, France

    P Faucher, M Coupaye, R Alili, A Basdevant, K Clément & C Poitou

  6. Department of General Medicine, Yorkleigh Surgery, Cheltenham, Gloucestershire, UK

    A Green

  7. INSERM, U872, Team 7 Nutriomique, Cordeliers Research Center, Paris, France

    A Basdevant, K Clément & C Poitou

  8. Université Pierre et Marie Curie-Paris6, UMR S 872, Paris, France

    A Basdevant, K Clément & C Poitou

Authors
  1. C Lloret-Linares
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. P Faucher
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. M Coupaye
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. R Alili
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. A Green
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. A Basdevant
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. K Clément
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. C Poitou
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to C Poitou.

Ethics declarations

Competing interests

The authors declare no conflict of interest.

Additional information

Supplementary Information accompanies this paper on International Journal of Obesity website

Supplementary information

Rights and permissions

Reprints and permissions

About this article

Cite this article

Lloret-Linares, C., Faucher, P., Coupaye, M. 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 37, 1198–1203 (2013). https://doi.org/10.1038/ijo.2012.228

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ijo.2012.228

Keywords

This article is cited by

Search

Advanced search

Quick links