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Energy expenditure at rest and during sleep in children with Prader-Willi syndrome is explained by body composition^1,2

¹ From the Departments of Human Biology, Pediatrics, Clinical Genetics, and Methodology and Statistics, Maastricht University, Maastricht, Netherlands.

Background: Obesity 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).

Objective: Our objective was to assess BMR and SMR adjusted for fat-free mass in young PWS patients.

Design: Subjects 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.

Results: BMR 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.

Conclusion: BMR and SMR are low in young patients with PWS because of a low fat-free mass.

Key Words: Prader-Willi syndrome • obesity • child • energy metabolism • body composition • bone age • fat-free mass • basal metabolic rate • sleeping metabolic rate

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