Endocrine Abstracts

JOINT2624

Introduction: Reducing calorie intake to avoid excessive weight gain whilst maintaining nutrient intake and growth is challenging in the management of PWS. General societal misconception of appropriate food portions also impacts care. Guidelines suggest 30% fewer calories are required in PWS, whereas micronutrient requirement is unchanged. No visual, practical guide exists to illustrate such appropriate food portion sizes. We aimed to 1. develop a national pictorial resource defining and illustrating correct portion sizes for children and adults with PWS, and 2. to assess the nutritional adequacy of these proposed portions.

Methods: A national working group with experience in PWS was devised (paediatric endocrinologist, specialist dieticians, therapy assistant, representative from the PWS Association UK and an endocrinology nurse) and linked with Nutrition and Diet Resources UK, a charity who develop nutritional resources. Previously defined national portion sizes for children (Public Health England, 2016) were proportionally reduced by 30% for a selection of commonly consumed foods for 6 age categories (2-3/4-6/7-10/11-13/14-18/19-64 years) and photographs produced. Recommended number of portions of each food group per day were defined from national recommendations, allowing for the development of day meal plans. Patient groups were consulted to include parent/patient feedback. A final resource was recirculated to the patient groups and national stakeholders for peer review. An example proposed 24 hour intake from the resource underwent dietary analysis for each age group for both sexes to validate nutritional adequacy.

Results: Sixty common foods were used to calculate age specific food portions and 348 photographs were taken to produce the resource (https://www.pwsa.co.uk/practicalportions). Less healthy foods were included but highlighted as undesired. Examples of 24 hour intake for each age group were analysed for nutritional value. Caloric intake ranged from 49% (adult males) to 69% (5 year old females) of the EAR. Several micronutrients were lower than recommended in several age groups including iron, zinc and iodine.

Conclusion: Poorly defined dietary guidance in PWS can negatively impact acute and chronic health outcomes. In addition, such an unusual recommended intake can be difficult to define, appreciate and apply. We present a PWS unique resource to provide correct food portion sizes and practically support and address these challenges. Nutritional analysis supports its use to provide a caloric intake suitable for PWS and highlights micronutrients that may require attention. Strengths include user involvement and visual nature reducing language barriers, allowing wide application. Limitations include restriction to 60 foods.