Dietary and Clinical Implications of FA Status in Children with Cystic Fibrosis

Background: Children with cystic fibrosis (CF) and pancreatic insufficiency (PI) are prone to essential fatty acid (FA) deficiency. This abstract aims to determine if type of dietary fat predicts FA status, and if FA status predicts CF clinical outcomes in two samples. Methods: Children with CF participated in a CF Nutrition Study. Healthy children from a Bone Study were the comparison group. Z scores for height [HAZ], weight [WAZ], body mass index [BMIZ] and mid upper arm circumference [MUACZ], FEV1, dietary intake, fat malabsorption (percent CFA) and serum FA status were obtained at baseline. Linear regression was used to examine dietary intake and serum FA outcomes. Longitudinal mixed effects were used to compare associations of T:T, serum LA and ALA with clinical outcomes. Results: 65 subjects with CF (8.4 plus1.0 years) and 22 controls (8.5 plus1.1 years) were evaluated. Despite greater energy, saturated fat, and LA intake, subjects with CF had lower LA and DHA and higher T:T and ARA:DHA than the comparison group. Depending on the T:T reference value(0.04 or 0.02), 17 percent  and 52 percent  of children with CF had EFA deficiency. Dietary total fat, MUFA, PUFA, LA, total (t) Tn 6PUFA, and ALA intake positively predicted LA concentration. MUFA, total (t) n 3PUFA, and ALA intake positively predicted DHA concentration. Total dietary fat, MUFA, PUFA, t n 3PUFA, LA, and ALA intake negatively predicted serum T:T. The relationship between serum PUFA status and clinical outcomes in 77 subjects (8.4 plus 0.9) was evaluated. Only serum LA was positively associated HAZ, WAZ, BMIZ, MUACZ and FEV1. Children with LA greater than 21 mol percent  had better growth and FEV1 status(p=0.002;p=0.006) than those with lower concentrations. Conclusions: Dietary fat type predicted serum PUFA in CF and PI. LA status predicted important growth and pulmonary outcomes. Type of dietary fat recommendations for patients with CF should be considered to improve FA and clinical outcomes. (Authors abstract)