Department of Nutrition and Dietetics, Children's Health Ireland at Tallaght, Tallaght University Hospital, Dublin, Ireland.
Children's Health Ireland at Crumlin, Cooley Road, Crumlin, Ireland.
J Hum Nutr Diet. 2021 Oct;34(5):784-791. doi: 10.1111/jhn.12882. Epub 2021 Apr 9.
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.
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.
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 /72-112% EAR); for those ≥ 12 years, it was 1203 kcal (8.3 kcal cm /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.
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.
普拉德-威利综合征（PWS）的管理需要严格的饮食监督，以预防肥胖、避免微量营养素缺乏并确保最佳生长。本研究旨在检查患有 PWS 的儿童的生长和饮食摄入情况。
邀请所有在爱尔兰儿童健康（CHI）塔勒塔特就诊并经基因确认患有 PWS 的儿童（n=44）参加。进行人体测量学和身体成分测量，使用生物电阻抗分析。使用三天的食物日记评估饮食摄入量，并评估早期喂养问题的存在。测量血清血红蛋白、铁蛋白和维生素 D 水平。
19 名儿童参与，平均（范围）年龄为 7.6（0.6-18.1）岁。大多数为女性（n=14，74%）。20%（n=3）体重不足，60%（n=9）体重健康，n=1超重，n=2肥胖。平均（范围）体脂百分比为 25.7%（10%-40%）。83%报告有早期喂养问题。94%（n=16）的能量达到估计平均需求量（EAR）的≤100%。≤5 岁的儿童每日能量摄入量为 722 千卡（9 千卡/cm /72-112%EAR）；≥12 岁的儿童为 1203 千卡（8.3 千卡/cm /41%-82%EAR）。明显存在钙、维生素 D、铁、锌和膳食纤维摄入不足的情况。两名儿童出现缺铁性贫血和维生素 D 不足。
本研究提供了爱尔兰首例 PWS 数据，表明能量摄入似乎不过量，五分之四的患者体重不足或 BMI 正常。明显存在几种微量营养素的饮食摄入不足，且存在生化营养素缺乏。
