Comparison of Body Composition, Muscle Strength and Cardiometabolic Profile in Children with Prader-Willi Syndrome and Non-Alcoholic Fatty Liver Disease: A Pilot Study

• Published in: International journal of molecular sciences Vol. 23; no. 23; p. 15115
• Main Authors: Mager, Diana R, MacDonald, Krista, Duke, Reena L, Avedzi, Hayford M, Deehan, Edward C, Yap, Jason, Siminoski, Kerry, Haqq, Andrea M
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 01.12.2022; MDPI
• Subjects: Adipose tissue; Adult; Blood pressure; Body Composition; Body mass; Body Mass Index; Body weight; Child; Children; Chromosomes; Fatty liver; Gastrointestinal surgery; Gene expression; Genotype & phenotype; Genotyping; Habitus; Hand Strength; Health care; Health services; Humans; Insulin; Insulin resistance; Lean body mass; Lipids; Liver; Liver diseases; Metabolism; MicroRNAs; Muscle Strength; Mutation; non-alcoholic fatty liver disease; Non-alcoholic Fatty Liver Disease - etiology; Obesity; Overweight; Physical activity; Pilot Projects; Prader-Willi syndrome; Prader-Willi Syndrome - complications; Quality of Life; Skeletal muscle; Weight control; muscle strength; body composition; non-alcoholic fatty liver disease; Prader-Willi syndrome; children
• DOI: 10.3390/ijms232315115
• ISSN: 1422-0067; 1661-6596

Syndromic and non-syndromic obesity conditions in children, such as Prader-Willi syndrome (PWS) and non-alcoholic fatty liver disease (NAFLD), both lower quality of life and increase risk for chronic health complications, which further increase health service utilization and cost. In a pilot observational study, we compared body composition and muscle strength in children aged 7−18 years with either PWS (n = 9), NAFLD (n = 14), or healthy controls (n = 16). Anthropometric and body composition measures (e.g., body weight, circumferences, skinfolds, total/segmental composition, and somatotype), handgrip strength, six minute-walk-test (6MWT), physical activity, and markers of liver and cardiometabolic dysfunction (e.g., ALT, AST, blood pressure, glucose, insulin, and lipid profile) were measured using standard procedures and validated tools. Genotyping was determined for children with PWS. Children with PWS had reduced lean body mass (total/lower limb mass), lower handgrip strength, 6MWT and increased sedentary activity compared to healthy children or those with NAFLD (p < 0.05). Children with PWS, including those of normal body weight, had somatotypes consistent with relative increased adiposity (endomorphic) and reduced skeletal muscle robustness (mesomorphic) when compared to healthy children and those with NAFLD. Somatotype characterizations were independent of serum markers of cardiometabolic dysregulation but were associated with increased prevalence of abnormal systolic and diastolic blood pressure Z-scores (p < 0.05). Reduced lean body mass and endomorphic somatotypes were associated with lower muscle strength/functionality and sedentary lifestyles, particularly in children with PWS. These findings are relevant as early detection of deficits in muscle strength and functionality can ensure effective targeted treatments that optimize physical activity and prevent complications into adulthood.