Abstract

We examined nutritional and psychosocial factors associated with adherence to the recommended diet (< or = 30% of energy from fat and < 10% from saturated fat) in children with familial hypercholesterolemia. Ninety-eight boys and 74 girls aged 7-17 y treated for > or = 18 mo responded to a quantitative food-frequency questionnaire that was self- (ages 13-17 y) or dietitian-(ages 7-12 y) administered. One hundred nine subjects also completed a weighed food record. Psychosocial assessments included the Child Behavior Checklist, Youth Self Report, and Children's Global Assessment Scale. The weighed record showed better adherence to dietary guidelines than the food-frequency questionnaire, but energy intake was underestimated. Low energy reporters had a healthier diet than the rest with the weighed record. According to the questionnaire, energy intake was underreported in only 9% of subjects and was not associated with a healthier diet, thus, further analyses were based on the questionnaire. Intakes of vitamin C (P = 0.0001), folate (P = 0.0001), riboflavin (P = 0.03), thiamine (P = 0.0001), and magnesium (P = 0.0001) per megajoule increased as quartile of total fat intake (as a % of total energy) decreased, reflecting increased intakes of cereals (P = 0.002), pasta (P = 0.01), fruit (P = 0.0001), pure meat (not minced or meat products; P = 0.047), skim milk (P = 0.0001), and skim cheese (P = 0.005). Energy and sugar (% of total energy) intakes were not significantly different across all fat intakes; energy density decreased with decreasing fat quartile. Overall psychosocial function score and parental educational level were associated with lower fat intake in multivariate analysis, explaining 11% of the variance in fat intake. We conclude that adherence to fat restriction among children treated for familial hypercholesterolemia is associated with increased micronutrient density, decreased energy density, and psychosocial factors that facilitate adherence.

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