Nutritient intake of young children with Prader–Willi syndrome

BACKGROUND: Prader–Willi syndrome (PWS) is a rare genetic disorder resulting in obesity. The diets for young children with PWS must balance the importance of preventing development of obesity with the need to supply sufficient energy and essential nutrients. OBJECTIVE: To investigate the nutritional intake for children with PWS 2, 3, and 4 years of age and compare it with Nordic Nutritional Recommendations (NNR) and intake of healthy controls. DESIGN: Assessments of food intake for six children 2–4 years of age were performed twice a year. At the age of 2 and 3 years data was obtained by using food recall interviews and at 4 year of age a pre-coded food-diary was used. RESULTS: The energy intake for the 2-year-old children was 3.25 MJ/day (SD 0.85) and for the 3- and 4-year olds 3.62 MJ/day (SD 0.73) and 4.07 MJ/day (SD 0.39 MJ), respectively. These intakes are 61%, 68%, and 77% of the estimated energy requirements in NNR for healthy 2-, 3- and 4-year-old children, respectively, and 60% and 66% of the energy intakes of 2- and 4-year-old children in reference populations. The children's BMI-for-age score and length growth was within the normal range during the study period. The intake of fat was about 25 E% in all age groups and reduced when compared with reference populations. In 25% of the assessments the fat intake was 20 E% or below. The intake of iron was below recommendations in all age groups both with and without supplementation. The mean intake of vitamin D and tocopherol was below recommendations when intakes were determined excluding dietary supplementations. CONCLUSIONS: More large-scale investigations on nutritional intake are needed to further investigate dietary challenges for this patient group.