Dietary intake and growth in children with Prader–Willi syndrome

Abstract

Background The management of Prader–Willi Syndrome (PWS) requires strict dietary supervision to prevent obesity, avoid micronutrient deficiencies and ensure optimal growth. The present study aimed to examine the growth and dietary intake of children with PWS. Methods All children with genetically confirmed PWS attending Children's Health Ireland (CHI) at Tallaght (n = 44) were invited to participate. Anthropometry was performed and body composition measured using bioelectrical impedance analysis. Three‐day food diaries were used to evaluate dietary intake and the presence of early feeding issues was assessed. Serum haemoglobin, ferritin and vitamin D levels were measured. Results Nineteen children participated, with a mean (range) age of 7.6 (0.6–18.1) years. Most were female (n = 14, 74%). Twenty‐percent (n = 3) were underweight, 60% (n = 9) were healthy weight, n = 1 was overweight and n = 2 were obese. Mean (range) percentage body fat was 25.7% (10%–40%). Eigthy‐three percent reported early feeding issues. Ninety‐four percent (n = 16) achieved ≤ 100% of estimated average requirement (EAR) for energy. Mean daily energy intake for ≤ 5 years old was 722 kcal (9 kcal cm–1/72–112% EAR); for those ≥ 12 years, it was 1203 kcal (8.3 kcal cm–1/41%–82% EAR). Suboptimal calcium, vitamin D, iron, zinc and fibre intake was evident. Iron deficiency anaemia and vitamin D insufficiency occurred in two children. Conclusions The present study provides the first Irish data for PWS and shows that energy intake does not appear to be excessive, with four in five patients being underweight or of a normal BMI. Suboptimal dietary intake of several micronutrients was evident and biochemical nutrient deficiencies were present.

A Personalized Approach to Determining the Caloric Needs of Children with Prader–Willi Syndrome Treated with Growth Hormone

“…The analysis of the distribution of proteins, carbohydrates, and fats showed that our patients followed a balanced diet within the ranges recommended for healthy children [ 18 ], although there was a trend toward ingesting less carbohydrates and more proteins than recommended. The parents of the children with PWS were generally involved in controlling their diets, and parental involvement likely helped ensure a balanced diet, as has been observed in other studies [ 20 , 24 ]. Miller et al [ 25 ] found that children with PWS who followed a balanced diet achieved better weight control than those who followed a diet only limiting caloric intake, even when the total caloric intake was similar.…”

“…To our knowledge, this was the first study to analyze the dietary intake in children with PWS initiating GH treatment early (median age, 1.4 years); in other studies, the age at initiating GH treatment ranged from 2.7 to 4 years [ 19 , 20 , 21 ]. Moreover, in the current study, the same nutritionist supervised the registry, thus, increasing the internal validity of the study.…”

“…To minimize the impact of age, sex, weight, height, and physical activity, we used the European Food Safety Authority’s equation to estimate total energy requirements for healthy children. The low age of the children in our study might also have influenced the discrepancy in these results, since the percentage of the recommended caloric intake is lower in older children [ 20 ]. Importantly, our findings may not hold true as children with PWS age and grow, and greater caloric restriction will very likely be necessary to maintain normal weight later in life.…”

Associations between Oxidant/Antioxidant Status and Circulating Adipokines in Non-Obese Children with Prader–Willi Syndrome

“…Suboptimal dietary intake of several essential nutrients—such as iron, zinc, tocopherol, and fiber—was described in patients with PWS by many authors [ 47 , 48 , 49 ]. It is suggested that this may result mainly from the reduced energy intake in these patients [ 49 ].…”

“…Suboptimal dietary intake of several essential nutrients—such as iron, zinc, tocopherol, and fiber—was described in patients with PWS by many authors [ 47 , 48 , 49 ]. It is suggested that this may result mainly from the reduced energy intake in these patients [ 49 ]. However, according to Mackenzie et al [ 48 ], adolescents with PWS had similar nutrient intakes to adolescents without PWS despite a lower energy intake.…”