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Published on: January 7, 2016
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.
Objectives:
To assess body composition of infants with Prader-Willi syndrome (PWS) by using deuterium dilution and investigating the efficacy of early institution of growth hormone (GH) therapy in increasing lean mass (LM) and preventing massive obesity.
Study Design:
One group of 11 children with PWS <2 years before and during 30-month GH therapy (GH group) was compared with 6 infants administered only coenzyme Q(10) for 1 year (Q10 group). LM adjusted for height (LM(Ht)) and relative fat mass (%FM(Age)) standard deviation scores (SDS) were calculated from data of 95 healthy children.
Results:
Initially, LM(Ht) of all patients was below the normal average. LM(Ht) decreased by -0.46 +/- 0.3 SD (P=.03) per year in the Q10 group but rose by 0.25 +/- 0.3 SD (P=.02) per year during GH therapy, normalizing after 30 months (-0.70 +/- 1.0 SD). Despite low to normal weight for height (WfH), %FM(Age) was above the normal average (GH group, 31.0% +/- 4.5%, Q10 group, 32.4% +/- 9.5%). In the Q10 infants, %FM(Age) increased by 0.71 +/- 0.7 SD per year, whereas in the GH group, %FM(Age) remained more stable up to 30 months.
Conclusions:
Diminished LM(Ht) found in infants with PWS further declines during the early years. Early institution of GH therapy lifts LM(Ht) into the normal range and delays fat tissue accumulation.
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