Complex Relation Between Diet and Phospholipid Fatty Acids in Children With Cystic Fibrosis.

Objectives: Altered total plasma n-6 and n-3 fatty acids are common in cystic fibrosis (CF). Whether alterations extend to plasma phosphatidylcholine (PC) and phosphatidylethanolamine (PE) and are explained by diet is unclear. The present study was to describe the dietary intake of a large group of children with CF and to determine whether dietary fat composition explains differences in plasma PC and PE fatty acids between children with and without CF.

Methods: Dietary intake was assessed using a food frequency questionnaire. Venous blood was collected. Plasma PC and PE were separately analyzed for fatty acids.

Results: Children with CF, n = 74, consumed more calories and fat (g/day and % energy), with significantly more saturates mainly from dairy foods and less polyunsaturates including linoleic acid (LA), arachidonic acid (ARA), eicosapentaenoic acid (EPA), and docosahexaenoic acid (DHA) (% fat) than reference children, n = 71. A subset of children with CF, not differing in dietary intake from the larger group, had significantly lower LA and DHA, but higher EPA in plasma PC and had higher LA and lower ARA and DHA in plasma PE, compared to a subset of reference children. In both groups, LA intake and LA in plasma PC and PE were not associated. EPA and DHA intakes were positively associated with EPA and DHA, respectively, in plasma PC, but not PE, in reference children only.

Conclusions: The fatty acid composition of plasma PC and PE is altered in CF. Fatty acid differences between children with and without CF are inconsistent between PC and PE and are not explained by dietary fat.