RESUMO
BACKGROUND: Research on aging in Prader-Willi syndrome (PWS) is limited, although people with PWS are living longer. Individuals with PWS present with high fat mass, low lean mass, and low levels of physical activity (PA). Previous reports in children and young adults with PWS show inadequate nutrient intake and body fat percentage indicating obesity. Previous studies in PWS rarely included individuals beyond young adulthood, especially studies conducted in the United States. This study includes adults from 18 to 62 years of age, and includes 19 of the estimated 60 adult individuals with PWS in Oklahoma. Because individuals with PWS are living longer, information must be provided on aging with PWS. This study is a report of the initial data for a planned longitudinal study on aging with PWS. OBJECTIVE: Determine associations between body composition, diet, PA, and a timed walk for adults with PWS, and to assess adequacy of dietary intake for those individuals aging with PWS. DESIGN: This cross-sectional investigation determined dietary habits, PA, and body composition of adults with PWS, and tested associations between these variables. RESULTS: Participants ranged in age from 18 to 62 years. They had healthier body composition, at 26.8% body fat, than previously reported. Mean body mass index (BMI) was in the overweight range at 26.7. Those who consumed higher amounts of fat (as a percent of total kilocalories) had statistically significant lower body fat percentage, but this may simply reflect that individuals with lower body fat percentages felt freer to consume fat. Mean steps taken per day was 7631.7 steps but only 16% of participants met healthy PA recommendations despite participating in daily structured exercise. All participants' diets met Dietary Guidelines for macronutrient distribution, but 80% were deficient in calcium, 100% were deficient in dietary vitamin D, and 87% were deficient in fiber. Sample size was small, so it was difficult to reach statistical significance, despite seeing clinical significance. CONCLUSIONS: Recommend working toward healthy PA recommendations for all age groups by decreasing time in sedentary activity. Recommend increasing vitamin A and D fortified dairy products and high-fiber foods, and consider dietary supplementation, especially for calcium, vitamin D, and fiber.