Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
Department of Agricultural, Food and Nutritional Science, University of Alberta, Edmonton, Alberta, Canada.
Am J Med Genet A. 2018 Nov;176(11):2309-2317. doi: 10.1002/ajmg.a.40491. Epub 2018 Sep 14.
Dietary management is important to prevent severe obesity in individuals with Prader-Willi syndrome (PWS); however, few studies have examined dietary intake and quality in youth with PWS. Our objective was to estimate intake of essential nutrients and diet quality in youth with PWS compared to those without PWS. Three-day food records were used to estimate intake of energy, nutrients, nutrient-density, foods, and adherence to healthy eating guidelines. Data were presented as medians and interquartile ranges with Mann-Whitney U and Fisher's test used to compare between groups with p < .05 considered significant. Youth with (n = 23) and without (n = 23) PWS were similar in age and sex distribution. The PWS group had a lower energy intake (p ≤ .001), higher nutrient density (p = .003), and better adherence to guidelines (p = .007) compared to the control group. The proportion with nutrient intake from food below Estimated Average Requirement or Adequate Intake were similar between groups. Fiber, vitamin D, calcium, and potassium intake were below recommendations in 50% or more in both groups. The inclusion of supplement intake lowered the proportion below recommendations, except for fiber and potassium. Youth with PWS had a similar nutrient intake as those without PWS despite a lower energy intake, which could be attributed to higher diet quality. However, more than half of youth with PWS were at risk of inadequate fiber, vitamin D, calcium, and potassium intake. A greater emphasis on nutrient-dense foods would improve nutrient intake, but supplements may be warranted in youth with PWS who do not meet recommendations.
饮食管理对于预防普拉德-威利综合征（PWS）患者的严重肥胖症非常重要；然而，很少有研究检查过 PWS 青少年的饮食摄入和质量。我们的目的是评估 PWS 青少年与非 PWS 青少年的必需营养素摄入和饮食质量。使用三天的食物记录来估计能量、营养素、营养素密度、食物摄入量以及遵守健康饮食指南的情况。数据以中位数和四分位距表示，使用 Mann-Whitney U 和 Fisher 检验比较两组间差异，p <.05 认为有统计学意义。患有（n = 23）和未患有（n = 23）PWS 的青少年在年龄和性别分布上相似。与对照组相比，PWS 组的能量摄入较低（p ≤.001），营养素密度较高（p =.003），且更遵守指南（p =.007）。两组的营养素摄入低于估计平均需求量或适宜摄入量的比例相似。两组中超过 50%的人膳食纤维、维生素 D、钙和钾的摄入量低于推荐量。纳入补充剂摄入量后，除了膳食纤维和钾以外，低于推荐量的比例有所降低。尽管 PWS 青少年的能量摄入较低，但他们的营养素摄入与非 PWS 青少年相似，这可能归因于较高的饮食质量。然而，超过一半的 PWS 青少年存在膳食纤维、维生素 D、钙和钾摄入不足的风险。更加强调营养丰富的食物将改善营养素摄入，但对于未能满足推荐量的 PWS 青少年，可能需要补充剂。
