OBJECTIVE: To measure activity related energy expenditure in Prader ± Willi syndrome (PWS) corrected for body size. SUBJECTS: 17 PWS subjects (10 females, seven males, age 7.5 ± 19.8 y) and 17 obese controls, matched for gender and bone age. MEAUREMENTS: Basal metabolic rate (BMR) was measured by ventilated hood and average daily metabolic rate (ADMR) was measured with doubly labelled water. Activity induced energy expenditure (AEE) was calculated as 0.9ADMR 7 BMR. Activity related energy expenditure was corrected for body size using the following measures: AEE per kg body weight (AEEakg), ADMRaBMR (PAL), and the residual of the regression of ADMR on BMR (rADMR). Group differences were analyzed by analysis of covariance adjusting for bone age, fat mass (FM) and gender. RESULTS: ADMR, AEE and PAL were lower (P`0.01) in the PWS group compared with the control group (7.14 AE 1.72, 1.07 AE 0.69 and 1.33 AE 0.15 MJaday compared with 9.94 AE 2.64, 2.56 AE 1.03 and 1.55 AE 0.12 MJaday respectively). The variance of AEEakg and PAL was signi®cantly explained by gender and PWS, while AEE was additionally explained by FM. The variance of rADMR was explained by PWS and not by FM or gender. CONCLUSION: Activity related energy expenditure is decreased in PWS compared with controls adjusted for bone age, FM and gender.
International Journal of Obesity (2000) 24, 429±434Keywords: Prader ± Willi syndrome; obesity; child; doubly labelled water; physical activity; energy; metabolism
IntroductionPrader ± Willi syndrome (PWS) is known as the most common human genetic disorder linked to obesity. 1 This complex, multisystem disorder is characterized by perinatal and neonatal hypotonia, followed by a childhood obese phase. 2,3 The obesity is likely to be caused by a combination of a low energy expenditure and a high energy intake. 4,5 Eating problems, characterized by hyperphagia, and often combined with food stealing are well reported problems in PWS, 6,4 however, the actual energy intake is dif®cult to measure with current methods. The doubly labelled water technique offers a valid method to measure the individual's average daily metabolic rate (ADMR) 7 which consists of basal metabolic rate (BMR), activity induced energy expenditure (AEE) and diet induced thermogenesis. 8,9 In a previous report we have demonstrated that the low energy expenditure in rest as well as during sleep could be explained by a relative low fat-free mass (FFM) as one of the major components of the pathophysiological background of PWS. 10 Schoeller et al 5 demonstrated that the average daily metabolic rate (ADMR) in PWS patients as well as the level of physical activity were signi®cantly lower compared with obese controls. Other investigators 11 were unable to con®rm the decreased ADMR in patients with this syndrome. One of the problems of comparing physical activity between subjects with large differences in body weight is the correction for body size. The relatively low fat-free mass, resulting in a high adiposity level in PWS, is an extra complication to th...