• E A van Mil, K R Westerterp, W J Gerver, L M Curfs, C T Schrander-Stumpel, A D Kester, and W H Saris.
• Departments of Human Biology, Pediatrics, Clinical Genetics, and Methodology and Statistics, Maastricht University, Maastricht, Netherlands. g.vanmil@hb.unimaas.nl
• Am. J. Clin. Nutr. 2000 Mar 1;71(3):752-6.

BackgroundObesity in Prader-Willi syndrome (PWS) seems to be related to a low basal metabolic rate (BMR). In addition, abnormal sleep patterns reported in PWS might affect sleeping metabolic rate (SMR).ObjectiveOur objective was to assess BMR and SMR adjusted for fat-free mass in young PWS patients.DesignSubjects were 17 PWS patients (10 females and 7 males aged 7.5-19.8 y) and 17 obese control subjects matched for sex and bone age. SMR was measured in a respiratory chamber, BMR with a ventilated-hood system, and body composition by deuterium dilution.ResultsBMR and SMR were significantly lower in the PWS group than in the control group (5.36 +/- 1.18 and 4.62 +/- 1.08 MJ/d compared with 6.38 +/- 1.55 and 5.60 +/- 1.52 MJ/d, respectively; P < 0.05). When fat-free mass was included in the analysis, multiple regression showed no differences in BMR and SMR between groups. When weight was included in the analysis instead of fat-free mass, SMR was lower in the PWS group. Fat-free mass was lower in the PWS group both as an absolute value and when adjusted for height.ConclusionBMR and SMR are low in young patients with PWS because of a low fat-free mass.

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