Assessing growth and energy requirements of children with Prader-Willi syndrome: study protocol

Background Prader-Willi Syndrome (PWS) is a complex genetic disorder characterised by neurodevelopmental impairments and distinct nutritional phases, including hyperphagia. If not managed, hyperphagia frequently leads to obesity, the most prevalent and clinically significant complication. Current dietetic management relies on energy restrictions; however, recommended energy intake values vary, and no predictive equations have been developed specifically for estimating requirements in children with PWS. This study aims to address this by developing clinically applicable predictive equations. Methods This cross-sectional observational study will recruit children (2–17 years) with genetically confirmed PWS. Participants will have anthropometric, body composition, and resting energy expenditure (REE) measurements taken. Additional data will include dietary intake, physical activity, hyperphagia-related behaviours, and clinical and growth data. Multivariate regression analyses will be used to develop and validate equations for estimating REE. Associations between REE and body composition, dietary intake, and growth patterns will also be explored. Discussion This study addresses a critical literature gap by developing the first paediatric PWS-specific predictive equations for energy requirements. Improved estimation of REE has the potential to enhance dietary management, reduce obesity risk, and inform clinical practice. Findings may also contribute to a better understanding of metabolic regulation and growth trajectories in children with PWS. Impact Children with PWS experience the development of hyperphagia at a young age, often leading to the development of obesity if left uncontrolled. Dietetic intervention is based on recommendations of energy restriction, however recommended values of energy intake vary, and there are currently no predictive equations available for a paediatric PWS population. This cross-sectional observational study aims to develop novel predictive equations for estimating energy requirements based on anthropometric, bioelectrical impedance, and indirect calorimetry measures. More accurate estimation of energy requirements may assist in reducing the risk of obesity and associated complications in these children.