Literature DB >> 10702169

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, L M Curfs, C T Schrander-Stumpel, A D Kester, W H Saris.   

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.

Entities:  

Mesh:

Year:  2000        PMID: 10702169     DOI: 10.1093/ajcn/71.3.752

Source DB:  PubMed          Journal:  Am J Clin Nutr        ISSN: 0002-9165            Impact factor:   7.045


  13 in total

1.  Adult-onset deletion of the Prader-Willi syndrome susceptibility gene Snord116 in mice results in reduced feeding and increased fat mass.

Authors:  Louise Purtell; Yue Qi; Lesley Campbell; Amanda Sainsbury; Herbert Herzog
Journal:  Transl Pediatr       Date:  2017-04

Review 2.  Energy Metabolism Profile in Individuals with Prader-Willi Syndrome and Implications for Clinical Management: A Systematic Review.

Authors:  Maha Alsaif; Sarah A Elliot; Michelle L MacKenzie; Carla M Prado; Catherine J Field; Andrea M Haqq
Journal:  Adv Nutr       Date:  2017-11-15       Impact factor: 8.701

3.  Body composition and fatness patterns in Prader-Willi syndrome: comparison with simple obesity.

Authors:  Mariana F Theodoro; Zohreh Talebizadeh; Merlin G Butler
Journal:  Obesity (Silver Spring)       Date:  2006-10       Impact factor: 5.002

4.  Prediction of basal metabolic rate in patients with Prader-Willi syndrome.

Authors:  S Lazzer; G Grugni; G Tringali; A Sartorio
Journal:  Eur J Clin Nutr       Date:  2015-09-23       Impact factor: 4.016

5.  Energy metabolism in relation to body composition and gender in adolescents.

Authors:  E G van Mil; K R Westerterp; A D Kester; W H Saris
Journal:  Arch Dis Child       Date:  2001-07       Impact factor: 3.791

6.  Nutritient intake of young children with Prader-Willi syndrome.

Authors:  Marianne Lindmark; Kerstin Trygg; Kaja Giltvedt; Svein O Kolset
Journal:  Food Nutr Res       Date:  2010-03-17       Impact factor: 3.894

7.  Assessment of sleep and breathing in adults with prader-willi syndrome: a case control series.

Authors:  Brendon J Yee; Peter R Buchanan; Sri Mahadev; Dev Banerjee; Peter Y Liu; Craig Phillips; Georgina Loughnan; Kate Steinbeck; Ronald R Grunstein
Journal:  J Clin Sleep Med       Date:  2007-12-15       Impact factor: 4.062

Review 8.  Prader Willi Syndrome: Genetics, Metabolomics, Hormonal Function, and New Approaches to Therapy.

Authors:  Krystal A Irizarry; Mark Miller; Michael Freemark; Andrea M Haqq
Journal:  Adv Pediatr       Date:  2016-08

9.  Snord116 is critical in the regulation of food intake and body weight.

Authors:  Yue Qi; Louise Purtell; Melissa Fu; Nicola J Lee; Julia Aepler; Lei Zhang; Kim Loh; Ronaldo F Enriquez; Paul A Baldock; Sergei Zolotukhin; Lesley V Campbell; Herbert Herzog
Journal:  Sci Rep       Date:  2016-01-04       Impact factor: 4.379

10.  Prader-Willi syndrome in neonates: twenty cases and review of the literature in Southern China.

Authors:  Ping Wang; Wei Zhou; Weiming Yuan; Longguang Huang; Ning Zhao; Xiaowen Chen
Journal:  BMC Pediatr       Date:  2016-08-09       Impact factor: 2.125

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