Literature DB >> 27570368

RESTING METABOLIC RATE IN PRADER-WILLI SYNDROME.

James O Hill1, Mary Kaler1, Bennett Spetalnick1, George Reed1, Merlin G Butler1.   

Abstract

Prader-Willi syndrome (PWS) may represent a genetic form of human obesity. The purpose of this study was to determine whether the relationship between resting metabolic rate and body weight/body composition was different in patients with PWS than in obese (body mass index 23-36) and lean (body mass index 15-20) controls. We determined body composition using bioelectrical impedance analysis (BIA) and skinfold thickness measurements in 36 subjects with PWS and in 31 controls (20 nonobese, 11 obese). The BIA measures of percentage of body fat were significantly correlated with those determined from skinfold thicknesses in all three groups. Resting metabolic rate (RMR) was measured in all lean and obese controls and in 22 of the 36 patients. Energy expenditure was compared among groups by comparing the regression relationship between RMR and either body weight or fat-free mass (FFM). The relationship between RMR and body weight and between RMR and FFM was different for patients with PWS than for lean and obese controls, who did not differ from each other. The nature of the relationship was such that patients with PWS had reduced rates of energy expenditure compared to controls, except for patients with the largest body mass and FFM. This suggests that a low energy expenditure exists initially for persons with PWS but may return to normal as obesity becomes greater. These results also suggest that reduced FFM is not the sole explanation for the lower energy expenditure seen in patients with PWS.

Entities:  

Keywords:  bioelectrical impedance analysis (BIA); fat-free mass (FFM); obesity; skinfold thicknesses

Year:  1990        PMID: 27570368      PMCID: PMC5001505     

Source DB:  PubMed          Journal:  Dysmorphol Clin Genet        ISSN: 0893-6633


  15 in total

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Journal:  Am J Clin Nutr       Date:  1989-05       Impact factor: 7.045

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Authors:  F John Meaney; Merlin G Butler
Journal:  Med Anthropol Q       Date:  2009-10-28

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Journal:  Am J Med Genet       Date:  1986-03

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Journal:  Hum Biol       Date:  1988-10       Impact factor: 0.553

6.  Energy expenditure and body composition in Prader-Willi syndrome.

Authors:  D A Schoeller; L L Levitsky; L G Bandini; W W Dietz; A Walczak
Journal:  Metabolism       Date:  1988-02       Impact factor: 8.694

7.  Reliability and validity of bioelectrical impedance in determining body composition.

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Journal:  J Appl Physiol (1985)       Date:  1988-02

8.  The use of skinfold measurements to judge obesity during the early phase of Prader-Labhart-Willi syndrome.

Authors:  M G Butler; R I Butler; F J Meaney
Journal:  Int J Obes       Date:  1988

9.  Prader-Willi syndrome: current understanding of cause and diagnosis.

Authors:  M G Butler
Journal:  Am J Med Genet       Date:  1990-03

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Authors:  H C Lukaski; W W Bolonchuk; C B Hall; W A Siders
Journal:  J Appl Physiol (1985)       Date:  1986-04
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  9 in total

1.  Beta3-adrenergic receptor gene studies in patients with Prader-Willi syndrome.

Authors:  M G Butler; L K Hedges; K S Babe
Journal:  Am J Med Genet       Date:  1997-10-03

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.  Comparison of leptin protein levels in Prader-Willi syndrome and control individuals.

Authors:  M G Butler; J Moore; A Morawiecki; M Nicolson
Journal:  Am J Med Genet       Date:  1998-01-06

4.  Energy expenditure and physical activity in Prader-Willi syndrome: comparison with obese subjects.

Authors:  Merlin G Butler; Mariana F Theodoro; Douglas C Bittel; Joseph E Donnelly
Journal:  Am J Med Genet A       Date:  2007-03-01       Impact factor: 2.802

Review 5.  Prader-Willi Syndrome in Adults: An Update On Nutritional Treatment and Pharmacological Approach.

Authors:  Silvia Savastano; Giovanna Muscogiuri; Luigi Barrea; Claudia Vetrani; Danilo Fintini; Giulia de Alteriis; Filippo Maria Panfili; Sarah Bocchini; Ludovica Verde; Annamaria Colao
Journal:  Curr Obes Rep       Date:  2022-09-05

6.  Prader-Willi Syndrome: Clinical and Genetic Findings.

Authors:  Merlin G Butler; Travis Thompson
Journal:  Endocrinologist       Date:  2000-07

7.  Preliminary observations of mitochondrial dysfunction in Prader-Willi syndrome.

Authors:  Merlin G Butler; Waheeda A Hossain; Robert Tessman; Partha C Krishnamurthy
Journal:  Am J Med Genet A       Date:  2018-10-05       Impact factor: 2.802

8.  Survival trends from the Prader-Willi Syndrome Association (USA) 40-year mortality survey.

Authors:  Ann M Manzardo; James Loker; Janalee Heinemann; Carolyn Loker; Merlin G Butler
Journal:  Genet Med       Date:  2017-07-06       Impact factor: 8.822

9.  Causes of death in Prader-Willi syndrome: Prader-Willi Syndrome Association (USA) 40-year mortality survey.

Authors:  Merlin G Butler; Ann M Manzardo; Janalee Heinemann; Carolyn Loker; James Loker
Journal:  Genet Med       Date:  2016-11-17       Impact factor: 8.822

  9 in total

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