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

Abstract

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.

• Authors: Urs Eiholzer, Dagmar L'allemand, Michael Schlumpf, Valentin Rousson, Theo Gasser, Christoph Fusch
• Journal: The Journal of pediatrics (J Pediatr) Vol. 144 Issue 6 Pg. 753-8 (Jun 2004) ISSN: 0022-3476 [Print] United States
• PMID: 15192622 (Publication Type: Clinical Trial, Comparative Study, Controlled Clinical Trial, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Recombinant Proteins
• Human Growth Hormone
• Ubiquinone

Topics

• Body Composition (drug effects)
• Female
• Human Growth Hormone (pharmacology, therapeutic use)
• Humans
• Infant
• Male
• Obesity (prevention & control)
• Prader-Willi Syndrome (drug therapy)
• Recombinant Proteins
• Statistics, Nonparametric
• Ubiquinone (pharmacology, therapeutic use)