Related Experiment Videos
Body fat determined by skinfold measurements is elevated despite underweight in infants with Prader-Labhart-Willi
U Eiholzer1, W F Blum, L Molinari
1Foundation Growth Puberty Adolescence, Zurich, Switzerland.
Insights
Children with Prader-Labhart-Willi syndrome exhibit altered body composition and elevated leptin levels, even when underweight. These findings suggest body fat distribution issues begin in infancy, prior to obesity development.
Area of Science:
- Pediatrics
- Endocrinology
- Genetics
Background:
- Prader-Labhart-Willi syndrome (PWS) is a genetic disorder characterized by hyperphagia and obesity.
- Body composition abnormalities and hormonal dysregulation are common in PWS.
- Leptin, a key regulator of appetite and energy expenditure, plays a role in PWS pathophysiology.
Purpose of the Study:
- To investigate body composition and leptin levels in young underweight and older overweight children with PWS.
- To determine if altered body fat distribution is present early in PWS, before obesity onset.
- To assess leptin production in relation to body composition in PWS.
Main Methods:
- Studied body composition using skinfold measurements and body mass index (BMI) standard deviation scores.
- Measured leptin levels adjusted for BMI in two groups of children with PWS: young underweight and older overweight.
- Compared body composition and leptin levels between the two PWS groups.
Main Results:
- Both young underweight and older overweight children with PWS showed elevated BMI-adjusted leptin levels.
- Elevated skinfold standard deviation scores indicated relatively increased body fat in both PWS groups, despite weight status.
- Leptin levels suggest intact leptin production in PWS.
Conclusions:
- Body composition is disturbed in Prader-Labhart-Willi syndrome from infancy, preceding obesity.
- Altered body fat distribution is a key feature of PWS, evident even in underweight individuals.
- Leptin production appears to be intact in children with PWS, suggesting a role in altered fat metabolism.
Abstract:
Body composition and leptin were studied in 13 young, still underweight and 10 older overweight children with Prader-Labhart-Willi syndrome. Not only the older overweight children but also the young underweight children had elevated skinfold standard deviation scores for body mass index and elevated body mass index adjusted leptin levels, suggesting relatively increased body fat despite underweight. Our data indicate that body composition in Prader-Labhart-Willi syndrome is disturbed already in infancy, long before the onset of obesity. Leptin production appears to be intact.