BACKGROUND: Some but not all studies have reported abnormal polyunsaturated fatty acid composition in cystic fibrosis (CF) patients. We investigated the influence of pancreatic status and sex on the fatty acid profile in plasma and erythrocyte membranes in patients with CF.METHODS: After a 1-step transesterification with acetyl chloride on plasma and washed erythrocyte membranes, we quantified fatty acid methyl esters by use of GC-MS in 124 CF patients and 80 age-matched healthy controls. In the CF group, mean (SD) age was 17.5 (11.3) years, and 51.6% were male. Pancreatic insufficiency was diagnosed in 78% of the CF population.RESULTS: A decrease in docosahexaenoic acid concentrations was observed in CF patients independently of pancreatic status. Pancreatic insufficient CF patients displayed lower concentrations of linoleic acid and arachidonic acid and higher concentrations of dihomo-gamma-linolenic acid and eicosatrienoic acid (mead acid) in plasma and erythrocyte membranes compared with healthy controls and pancreatic sufficient CF patients. Male CF patients had significantly lower docosahexaenoic acid and higher eicosatrienoic acid in plasma and erythrocyte membranes compared with female CF patients.CONCLUSIONS: These results support the concept that multiple abnormalities of polyunsaturated fatty acid composition participate in the CF disease phenotype and that pancreatic status plays a major role in such abnormalities. Moreover, patient sex influences the polyunsaturated fatty acid spectrum in CF, with more marked abnormalities in males.
8,11,14-Eicosatrienoic Acid Adolescent