Zusammenfassung

Journal of Pediatrics 2004; 144: pp. 753-758

Growth hormone and body composition in children younger than two years with Prader-Willi syndrome
Urs Eiholzer, Dagmar l'Allemand, Michael Schlumpf, Valentin Rousson, Theo Gass, Christoph Fusch

OBJECTIVES:
The study was aimed at assessing body composition of infants with PWS using deuterium dilution and investigating the efficacy of early institution of 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 to 6 infants administered only coenzyme Q10 for 1 year (Q10 group). LM adjusted for height (LMHt) and relative fat mass (%FMAge) standard deviation scores (SDS) were calculated from data of 95 healthy children.

RESULTS:
Initially LMHt of all patients was below the normal average. LMHt decreased by -0.46±0.3SD, p=0.03 per year in the Q10 group, but rose by 0.25±0.3SD, p=0.02 per year during GH therapy, normalising after 30 months (-0.70±1.0SD). Despite low to normal weight for height (WfH), %FMAge was above the normal average (GH group 31.0%±4.5, Q10 group 32.4%±9.5). In the Q10 infants, %FMAge increased by 0.71SD±0.7 per year, while in the GH group, %FMAge remained more stable up to 30 months.

CONCLUSIONS:
Diminished LMHt found in infants with PWS further declines during the early years. Early institution of GH therapy lifts LMHt into the normal range and delays fat tissue accumulation.