Aims Worldwide consensus is that Prader-Willi Syndrome (PWS) children require fewer calories (only 60%) to promote optimal growth as compared with non PWS children. There is no published UK data about adequacy of vitamin and mineral intakes or prevalence of micronutrient deficiencies in these significantly calorie restricted diets. This study assesses if calorie restricted diets in PWS children results in micronutrient intakes below Reference Nutrient Intakes (RNI) and more specifically and clinically relevant, below Lower Reference Nutrient Intakes (LRNI).
Methods PWS children attending a specialist clinic led by a multi-disciplinary team, completed 3 day food diaries (weighed or portion size) as part of their regular assessments. Food diaries were analysed using specialist software (DietPlan); data input and analysis was done by 1 of 2 trained dieticians. The results were expressed as% of RNI and LRNI adjusted for age and sex. Longitudinal data was retrospectively collated from food diaries submitted and analysed from 2002 to 2015.
Results 45 food diaries from 16 PWS children (aged 1.1–16 years; 7 males and 9 females) collected from 2002 to 2015 were reviewed. At least 1 micronutrient was below 100% RNI in 43 of the 45 analyses. The relevant micro-nutrients and proportion of food diaries where the RNI was <100% were Zinc, Selenium, Iron, Niacin and B12 in 76%, 57%, 45%, 32% and 9% respectively.
At least 1 micronutrient was below 100% LRNI in 14 of the 45 analyses. The relevant micro-nutrients and proportion of food diaries where the LRNI was <100% were Zinc, Selenium, Iron, B12 and Niacin in 19.5%, 13.3%, 10.6%, 8.5% and 6.4% respectively. The relevant micro-nutrients<100% LRNI by number of children were Zinc, Iron, Selenium, Niacin and B12 in 5, 4, 4, 3 and 2 children respectively.
Conclusions This study describes the diets of PWS children in UK and likely micronutrient deficiencies in them. Nutrient intakes below RNI are common, but may not always be clinically significant. Although less common, intakes below LRNI are a clinically relevant concern. Particular attention should be paid to zinc, selenium and iron when monitoring calorie restricted diets in PWS children.
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