Growth hormone and body composition in children younger than 2 years with Prader-Willi syndrome

Urs Eiholzer1, Dagmar L'allemand, Michael Schlumpf

  • 1Department of Biostatistics, University of Zürich, Switzerland. mail@childgrowth.org

Insights

Infants with Prader-Willi syndrome (PWS) have low lean mass (LM) that declines with age. Early growth hormone (GH) therapy improves LM and helps prevent obesity in children with PWS.

Area of Science:

  • Pediatric endocrinology
  • Genetics
  • Body composition analysis

Background:

  • Prader-Willi syndrome (PWS) is a genetic disorder characterized by developmental abnormalities and hormonal imbalances.
  • Infants with PWS often exhibit diminished lean mass (LM) and a propensity for obesity.
  • Early assessment of body composition is crucial for managing PWS complications.

Purpose of the Study:

  • To evaluate body composition in infants with Prader-Willi syndrome (PWS) using deuterium dilution.
  • To determine the efficacy of early growth hormone (GH) therapy in increasing lean mass (LM) and mitigating excessive fat accumulation in infants with PWS.

Main Methods:

  • A comparative study involving two groups of infants with PWS under 2 years of age.
  • The growth hormone (GH) group (n=11) received GH therapy for 30 months.
  • The coenzyme Q10 (Q10) group (n=6) received only Q10 for 1 year.
  • Lean mass adjusted for height (LM(Ht)) and relative fat mass (%FM(Age)) standard deviation scores (SDS) were calculated using data from 95 healthy children.

Main Results:

  • Initially, all PWS infants had below-average LM(Ht).
  • The Q10 group showed a significant decrease in LM(Ht) (-0.46 SD/year), while the GH group experienced an increase (0.25 SD/year), normalizing after 30 months.
  • %FM(Age) was elevated in both groups, increasing in the Q10 group but remaining more stable with GH therapy.

Conclusions:

  • Infants with Prader-Willi syndrome (PWS) present with reduced lean mass (LM) that deteriorates in early childhood.
  • Early initiation of growth hormone (GH) therapy is effective in normalizing LM and delaying the onset of obesity in infants with PWS.
Abstract

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