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Prader-Willi syndrome: how does growth hormone affect body composition and physical function?

A L Carrel1, D B Allen

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

Journal of Pediatric Endocrinology & Metabolism : JPEM
|February 12, 2002
PubMed

Insights

Growth hormone (GH) deficiency is common in children with Prader-Willi syndrome (PWS), causing body composition issues. GH treatment improves growth, body fat, and physical abilities in these children.

Area of Science:

  • Pediatric Endocrinology
  • Genetics and Metabolic Disorders
  • Growth Hormone Research

Background:

  • Prader-Willi syndrome (PWS) is characterized by growth failure, reduced lean body mass, and increased adipose tissue.
  • These body composition abnormalities in PWS patients resemble those in growth hormone deficiency (GHD).
  • Evidence suggests true GHD in many children with PWS due to diminished GH response and low IGF-I levels.

Purpose of the Study:

  • To evaluate the impact of growth hormone (GH) treatment on body composition and metabolic parameters in children with PWS.
  • To assess the contribution of GHD to the disabilities observed in pediatric PWS.
  • To determine the clinical significance and benefits of GH therapy in this population.

Main Methods:

  • Assessment of body composition (lean body mass, adipose tissue) in children with PWS.
  • Measurement of growth parameters, including linear growth.
  • Evaluation of metabolic effects, physical strength, and agility following GH treatment.

Main Results:

  • GH treatment led to decreased body fat and increased lean body mass in children with PWS.
  • Significant improvements were observed in linear growth, fat utilization, and energy expenditure.
  • Enhancements in physical strength and agility were noted, particularly during the first year of therapy.

Conclusions:

  • Growth hormone deficiency contributes significantly to the disabilities associated with Prader-Willi syndrome.
  • GH therapy offers clinically significant benefits for children with PWS, improving body composition and metabolic function.
  • While improvements are most pronounced initially, prolonged GH treatment remains beneficial for managing PWS-related challenges.

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