Journal of mucopolysaccharidosis and rare diseases

Association for Research of MPS and Rare Diseases (뮤코다당증연구학회)
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Obesity and Metabolic Syndrome in Adults with Prader-Willi Syndrome

  • Kim, Su Jin (Department of Pediatrics, Myongji Hospital, Seonam University College of Medicine)
  • Received : 2015.11.12
  • Accepted : 2015.11.20
  • Published : 2015.12.31
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Abstract

Body fat distribution in patients with Prader-Willi syndrome (PWS) is characterized by reduce lean body mass (LBM), increased total body fat mass (FM), and lower percentage of visceral adipose tissue (VAT). Individuals with PWS seem to have a lower risk for insulin resistance with high levels of adiponectin, an anti-atherogenic adipocytokine that is decreased in visceral fat hypertrophy subjects compared to simple obese subjects, both in children and in adults. The mechanism of the reduction in visceral adiposity in PWS is still unclear. It might be related to qualitative intrinsic characteristics of adipocyte or novel genetic influences on the control of fat distribution. However, obesity remains a critical problem, and obesity status plays a crucial role in individual metabolic risk clustering and development of metabolic syndrome (Mets) in PWS children and adults. Long-term growth hormone (GH) treatment after cessation of skeletal growth improved body composition, with an increase in lean body mass and a reduction in total body fat and subcutaneous and visceral fat in PWS adults. Thus, the role of GH is important after childhood because it might attenuate obesity and Mets in PWS adult by adipocyte modification.

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References

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