Background: Prader-Willi syndrome (PWS) is a rare complex genetic disease. Nutritional needs vary depending on life stage, ranging from growth faltering in infancy to obesity from late childhood. Dietetic care is a main pillar of the multidisciplinary team (MDT) approach for PWS management. National Children Hospital (NCH) is the main national centre for PWS in Ireland, with 47 children attending. We sought to explore growth and nutritional status in children with PWS.
Methods: All children with PWS attending NCH were invited to participate (n=44). Growth and nutritional status was assessed using:
1. Weight and height/length
2. Blood tests (full blood count, vitamin D and ferritin levels)
3. 3-day reported food diary
4. Questionnaires assessing feeding practices. Ethical approval was obtained.
Results: Nineteen patients participated (n=19), with 14 being female. Median age was 7.27 years (0.618.2). 13 patients were treated with growth hormone.
Nutrient deficiency: Iron deficiency anaemia was present in two patients and Vitamin D insufficiency was present in two patients.
Supplementation: 11 patients (58%) reported to take vitamin/mineral/nutritional supplements, of these 3 (16%) were taking prescribed iron or vitamin D supplement.
Feeding Issues: 13 patients (70%) required neonatal NG feeding. Average weaning age to solid food was 29.5 weeks. 9 patients (47%) reported food seeking behaviour, with mean age of onset at 4.75 years.
Food diary analysis: 15 patients (79%) were not meeting their recommended daily allowance (RDA) for iron and 9 patients (47%) were not meeting RDA for calcium (Table 1).
|Cole, 1990||WHO, 2007|
Discussion: The results depicts nutritional issues in this group of children with PWS including delayed weaning, food seeking behaviours and the need for ongoing nutrition support in infancy to manage dietary overrestriction as well as prevention of obesity in later childhood. This study highlights the need for a dedicated dietetic service to provide support and evidence-based nutritional advice.
Conclusion: Children with PWS have dynamic nutritional needs and require ongoing nutritional input to optimise growth and nutritional status.
22 - 24 Nov 2017
British Society for Paediatric Endocrinology and Diabetes