BACKGROUND: Children with cystic fibrosis (CF) and pancreatic insufficiency (PI) are at increased risk for essential fatty acid (EFA) deficiency. OBJECTIVES: To investigate serum markers of EFA status in children with CF and PI and their association with growth, body composition, and lung function. PATIENTS AND METHODS: Serum phospholipid fatty acid, growth, and forced expiratory volume at 1 second (FEV1, percentage predicted) status were assessed at baseline and 12 months in 77 children with CF and PI, 7 to 10 years old. Longitudinal mixed-effects models were used to compare associations of the triene:tetraene ratio (ratio of eicosatrienoic acid to arachidonic acid) and serum linoleic acid (as a molar percentage of total serum phospholipid fatty acids, or mol%) with the clinical outcomes. Controls for serum fatty acid were 23 healthy white age- and sex-matched children. RESULTS: Children with CF and PI had higher median triene:tetraene ratio and lower linoleic acid than healthy controls. Depending on the triene:tetraene ratio cutoff point used (0.04 or 0.02), either 17% or 52% of the children with CF had EFA deficiency, respectively. Only linoleic acid was significantly and positively associated with z scores for weight, height, body mass index, upper arm muscle area, and FEV1 at baseline. Children with linoleic acid at 21 mol% or higher had significantly better growth and pulmonary status than those with lower concentrations. CONCLUSIONS: Serum phospholipid linoleic acid at 21 mol% or higher was associated with better growth, body composition, and FEV1. No clinical outcome associations were found with the triene:tetraene ratio. These findings suggest that linoleic acid concentration was a more clinically relevant biomarker of EFA status than the triene:tetraene ratio in children with CF and PI. Further research is warranted to validate this specific percentage of linoleic acid cutoff point as a new recommendation for clinical use.
BACKGROUND:Children with cystic fibrosis (CF) and pancreatic insufficiency (PI) are at increased risk for essential fatty acid (EFA) deficiency. OBJECTIVES: To investigate serum markers of EFA status in children with CF and PI and their association with growth, body composition, and lung function. PATIENTS AND METHODS: Serum phospholipid fatty acid, growth, and forced expiratory volume at 1 second (FEV1, percentage predicted) status were assessed at baseline and 12 months in 77 children with CF and PI, 7 to 10 years old. Longitudinal mixed-effects models were used to compare associations of the triene:tetraene ratio (ratio of eicosatrienoic acid to arachidonic acid) and serum linoleic acid (as a molar percentage of total serum phospholipid fatty acids, or mol%) with the clinical outcomes. Controls for serum fatty acid were 23 healthy white age- and sex-matched children. RESULTS:Children with CF and PI had higher median triene:tetraene ratio and lower linoleic acid than healthy controls. Depending on the triene:tetraene ratio cutoff point used (0.04 or 0.02), either 17% or 52% of the children with CF had EFA deficiency, respectively. Only linoleic acid was significantly and positively associated with z scores for weight, height, body mass index, upper arm muscle area, and FEV1 at baseline. Children with linoleic acid at 21 mol% or higher had significantly better growth and pulmonary status than those with lower concentrations. CONCLUSIONS: Serum phospholipidlinoleic acid at 21 mol% or higher was associated with better growth, body composition, and FEV1. No clinical outcome associations were found with the triene:tetraene ratio. These findings suggest that linoleic acid concentration was a more clinically relevant biomarker of EFA status than the triene:tetraene ratio in children with CF and PI. Further research is warranted to validate this specific percentage of linoleic acid cutoff point as a new recommendation for clinical use.
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