Decreased energy expenditure is caused by abnormal body composition in infants with Prader-Willi Syndrome

M Tracy Bekx1, Aaron L Carrel, Timothy C Shriver

  • 1Department of Pediatrics, University of Wisconsin Children's Hospital, Madison, Wisconsin 53792, USA. mtbekx@wisc.edu

Insights

Infants with Prader-Willi syndrome (PWS) have higher body fat and lower fat-free mass (FFM), leading to reduced energy expenditure. Energy expenditure per FFM is normal, suggesting FFM drives the lower expenditure in PWS infants.

Area of Science:

  • Pediatrics
  • Endocrinology
  • Metabolism

Background:

  • Prader-Willi syndrome (PWS) infants exhibit hypotonia and underweight prior to developing obesity.
  • Understanding body composition and energy expenditure is crucial for managing PWS in infancy.

Purpose of the Study:

  • To evaluate body composition in infants with PWS.
  • To determine the relationship between body composition and energy expenditure in PWS infants.

Main Methods:

  • Analyzed body composition using dual-energy x-ray absorptiometry and deuterium dilution in 16 PWS infants.
  • Measured energy expenditure via doubly labeled water and indirect calorimetry.
  • Compared findings with age-matched normative data.

Main Results:

  • PWS infants showed significantly increased percent body fat and decreased fat-free mass (FFM) compared to normal infants.
  • Total energy expenditure was significantly lower in PWS infants.
  • The relationship between FFM and total energy expenditure was normal in PWS infants.

Conclusions:

  • Infants with PWS have altered body composition (increased fat, decreased FFM) and reduced energy expenditure.
  • Lower energy expenditure in PWS infants is primarily attributed to decreased FFM.
  • Energy expenditure relative to FFM is comparable to that of infants without PWS.
Abstract

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