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Energy expenditure at rest and during sleep in children with Prader-Willi syndrome is explained by body composition

E A van Mil1, K R Westerterp, W J Gerver

  • 1Departments of Human Biology, Pediatrics, Clinical Genetics, and Methodology and Statistics, Maastricht University, Maastricht, Netherlands. g.vanmil@hb.unimaas.nl

The American Journal of Clinical Nutrition
|March 4, 2000
PubMed

Insights

Young Prader-Willi syndrome (PWS) patients have lower basal metabolic rate (BMR) and sleeping metabolic rate (SMR) due to reduced fat-free mass, not differences in metabolic rate itself.

Area of Science:

  • Endocrinology
  • Metabolism
  • Genetics

Background:

  • Obesity in Prader-Willi syndrome (PWS) is linked to a low basal metabolic rate (BMR).
  • Abnormal sleep patterns in PWS may also impact sleeping metabolic rate (SMR).

Purpose of the Study:

  • To evaluate BMR and SMR, adjusted for fat-free mass, in young PWS patients.
  • To compare metabolic rates between PWS patients and matched obese controls.

Main Methods:

  • 17 PWS patients and 17 matched obese controls (aged 7.5-19.8 years) participated.
  • Body composition was assessed using deuterium dilution.
  • BMR and SMR were measured using ventilated-hood and respiratory chamber systems, respectively.

Main Results:

  • PWS patients exhibited significantly lower BMR and SMR than controls.
  • After adjusting for fat-free mass, no significant differences in BMR or SMR were observed between groups.
  • PWS patients had lower absolute and height-adjusted fat-free mass.

Conclusions:

  • The lower BMR and SMR in young PWS patients are primarily attributed to their reduced fat-free mass.
  • Metabolic rates per unit of fat-free mass do not differ significantly between PWS patients and controls.
Abstract

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