Aim: To compare body composition in children with Prader-Willi syndrome (PWS) not naïve to growth hormone (GH) with obese and lean controls. Methods: Participants included 12 children with PWS, 12 children with obesity (body fat percentage >95th percentile for age and sex) and 12 lean children (body fat percentage <85th percentile for age and sex) matched by age and height. Fat mass, lean mass, bone mineral content (BMC), bone mineral density (BMD) and BMD z-score for total body, hips and lumbar spine were obtained through dual X-ray absorptiometry. Results: PWS had higher fat percentage in the legs (p = 0.04) but similar leg fat mass (p = 1.00) compared to obese. PWS exhibited lower lean mass in the body (p = 0.04) and legs (p = 0.02) than obese, but similar to lean (p = 1.00 and p = 0.89, respectively). PWS had lower hip BMC (p < 0.01), BMD (p < 0.01) and BMD z-score (p < 0.01) compared to obese but similar to lean. No other differences were found between PWS and obese (p > 0.05 for all). Conclusions: Children with PWS not naïve to GH present differences in fat and lean mass distribution compared to obese controls. BMC and BMD appear unaffected by PWS, except at the hips.

Nutritional intakes in children with Prader - Willi syndrome and non-congenital obesity

During childhood, varying exercise modalities are recommended to stimulate normal growth, development, and health. This project investigated hormonal and metabolic responses triggered by a resistance exercise protocol in lean children (age: 9.3 ± 1.4 y, body fat: 18.3 ± 4.9%), obese children (age: 9.6 ± 1.3 y, body fat: 40.3 ± 5.2%) and lean adults (age: 23.3 ± 2.4 y, body fat: 12.7 ± 2.9%). The protocol consisted of stepping onto a raised platform (height = 20% of stature) while wearing a weighted vest (resistance = 50% of lean body mass). Participants completed 6 sets of 10 repetitions per leg with a 1-min rest period between sets. Blood samples were obtained at rest preexercise, immediately postexercise and 2 times throughout the 1-hr recovery to analyze possible changes in hormones and metabolites. Children-adult differences included a larger exercise-induced norepinephrine increase in adults vs. children and a decrease in glucagon in children but not adults. Similarities between adults and children were observed for GH-IGF-1 axis responses. Metabolically, children presented with lower glycolytic and increased fat metabolism after exercise than adults did. Obesity in childhood negatively influenced GH, insulin, and glucose concentrations. While adults occasionally differed from children, amount of activated lean mass, not maturation, likely drove these dissimilarities.

The aim of the study was to determine if physical activity (PA) is associated with bone health in children with Prader-Willi syndrome (PWS).Participants included 23 children with PWS (age: 11.0±2.0 years). PA, measured by accelerometry, was categorized into light, moderate, vigorous and moderate plus vigorous intensities. Hip, total body minus the head (body), bone mineral content (BMC), bone mineral density (BMD) and BMD z-score (BMDModerate PA and select covariates explained the most variance in hip BMC (84.0%), BMD (61.3%) and BMDPA of at least moderate intensity appears important for BMC and BMD in children with PWS.