Influence of Pancreatic Status and Sex on Polyunsaturated Fatty Acid Profiles in Cystic Fibrosis

Abstract

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-γ-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.

Whole blood fatty acid analysis with micromethod in cystic fibrosis and pulmonary disease

“…In addition, Coste et al have found lower levels of this product in plasma and RBC from CF according to the pancreatic status [19]. A limitation of the study is the small number (n = 5) of patients with a FEV 1% value below 50; these patients are children or adolescents with an important respiratory deficiency that greatly affects the life expectation, and for this reason the group is poor/limited.…”

“…Strandvik et al [20] have found correlations between an altered FA profile, or levels of selected FA, and genotype, whereas Coste et al [19] found correlations between FA levels and the pancreatic status. Genotype accounts only partially for the severity of CF [25] while FEV 1 is considered the best surrogate measure for survival and therefore the best indicator of the severity of disease.…”

“…An altered FA profile in CF versus C has been described over several years; FA analysis is usually assessed in plasma PL or in red blood cells (RBC), in few cases in plasma total lipids or in the cholesterol ester (CE) fraction [17][18][19]. Whole blood FA analysis is a more practical and useful method to determine the FA status in humans.…”

Persistence of Essential Fatty Acid Deficiency in Cystic Fibrosis Despite Nutritional Therapy

“…Fatty acid composition may be influenced by genetic factors as shown in murine models (10,11) or in humans (12). The role played by pancreatic insufficiency may also be especially relevant because fat malabsorption persists in many patients despite pancreatic enzyme replacement (13).…”

Cystic Fibrosis-Related Oxidative Stress and Intestinal Lipid Disorders

“…However, various studies show the limited impact of this routine therapy on patients' clinical conditions. In fact, a negligible improvement in anthropometric measures, an increase in saturated FAs and monounsaturated FAs, an increase in DHA/AA ratio, and onset of diabetes-type hepatic and metabolic complications have been reported (34,49,77,108). Some authors also mention significantly low levels of vitamin E associated with significantly high levels of oxysterols in patients with pancreatic insufficiency or diabetes.…”