普拉德-威利综合征患儿静息和睡眠期间的能量消耗可由身体组成来解释。

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

作者信息

van Mil E A, Westerterp K R, Gerver W J, Curfs L M, Schrander-Stumpel C T, Kester A D, Saris W H

机构信息

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

出版信息

Am J Clin Nutr. 2000 Mar;71(3):752-6. doi: 10.1093/ajcn/71.3.752.

DOI:10.1093/ajcn/71.3.752

PMID:10702169

Abstract

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.

摘要

背景

普拉德-威利综合征（PWS）中的肥胖似乎与基础代谢率（BMR）较低有关。此外，PWS中报告的异常睡眠模式可能会影响睡眠代谢率（SMR）。

目的

我们的目的是评估年轻PWS患者经去脂体重校正后的BMR和SMR。

设计

受试者为17名PWS患者（10名女性和7名男性，年龄7.5 - 19.8岁）以及17名按性别和骨龄匹配的肥胖对照受试者。在呼吸室内测量SMR，用通风罩系统测量BMR，通过氘稀释法测量身体成分。

结果

PWS组的BMR和SMR显著低于对照组（分别为5.36±1.18和4.62±1.08 MJ/d，而对照组为6.38±1.55和5.60±1.52 MJ/d；P<0.05）。当在分析中纳入去脂体重时，多元回归显示两组之间的BMR和SMR没有差异。当在分析中纳入体重而非去脂体重时，PWS组的SMR较低。PWS组的去脂体重无论是绝对值还是经身高校正后均较低。