Nutritional Management

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Long-term management of patients with PWS presents unique evolving nutritional challenges. During infancy, muscle hypotonia impairs oral feeding and causes inadequate caloric intake and failure to thrive. The combination of altered body composition (with diminished metabolically active lean mass), growth hormone deficiency, and excessive intake results in obesity unless intake is restricted. Without aggressive nutritional interventions, the life expectancy is shortened for individuals with PWS due to obesity comorbidities.

Prior studies have reported metabolic differences among individuals with PWS. Adults with PWS have a low basal metabolic rate (BMR) dependent on the technique of body composition analysis. Despite differences in body composition, energy expenditure during physical activity is similar to that of controls but their overall activity level is less than that of controls. The combination of diminished BMR and activity level necessitates lower caloric intake to avoid significant obesity.

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