Serum fatty acid profiles in cystic fibrosis patients and their parents

Abstract

Fatty acid compositions of the major serum lipid classes from 43 cystic fibrosis (CF) homozygotes (CF patients), 36 obligate heterozygotes (parents of CF patients) and 34 controls were determined by capillary gas chromatography. Fatty acid compositions of the homozygote CF patients were skewed in the direction of relative essential fatty acid deficiency in comparison with the controls. Less pronounced, but similar deviations from normal, were observed in the heterozygotes. Homozygotes with normal fatty acid compositions and heterozygotes with considerably disturbed fatty acid profiles were found.

Plasma fatty acids and lipid hydroperoxides increase after antibiotic therapy in cystic fibrosis

“…low levels of the PL and CE of linoleic acid (C18:2n-6) and PL of linolenic acid (C18:3n-3) [10], and high levels of the PL and CE of n-7 and n-9 FAs [29], respectively. Moreover, in the relevant literature, the levels of arachidonic acid in CF patients have been found to be lower [2,4,30], equivalent [10], or higher [11] than those of controls according to age or to other characteristics. The present results, lower levels of the PL fraction, are in agreement with those of THOMPSON et al [2], FARRELL et al [4] and CHRISTOPHE et al [30,31], although they are in disagreement with those of FREEDMAN et al [11].…”

Molecular Species Compositions of Lung and Pancreas Phospholipids in the cftrtm1HGU/tm1HGU Cystic Fibrosis Mouse

“…Any comparison of membrane phospholipid compositions between mice and people must recognize the lower ⌬5 desaturase activity in human liver (25) leading generally to phospholipids from mouse compared with human tissues having higher relative concentrations of 20:4 n-6 -containing species. Consequently, the decreased concentrations of PC16:0/20:4 and PC18:0/20:4 in plasma from the cftr tm1HGU/tm1HGU mouse may reflect similar metabolic processes to those that cause the decreased concentration of 18:2 n-6 in plasma PtdCho both from patients with CF even after pancreatic lipase supplementation (5) and from CFTR heterozygotic subjects (2). In effect, the contribution of n-6 fatty acids to plasma PtdCho is similarly decreased for both CF mice and CF patients, albeit the 20:4 n-6 chain elongation product for the mice and the 18:2 n-6 dietary precursor for patients.…”

“…For instance, low concentrations of the essential fatty acid (EFA) linoleate (18:2 n-6 ) have been reported in phospholipid, triacylglycerol, esterified, and nonesterified cholesterol fractions of plasma (1,2), in red blood cell membrane phospholipid (3,4), and in total lipid extracts of liver, lungs, and heart muscle from patients with CF (1). Although disease-related impairments of lipid digestion and absorption have largely been corrected by introduction of diets with high energy and fat contents combined with pancreatic lipase supplementation, most evidence suggests that a degree of EFA deficiency remains in CF.…”

“…This observation raised the possibility that dietary supplementation of patients with CF and 22:6 n-3 might both redress a putative imbalance between 22:6 n-3 and the proinflammatory fatty acid arachidonate (20:4 n-6 ) in cftrexpressing cells and lead to clinical benefit. Phospholipid fatty acid analysis of plasma and erythrocytes from children with CF have varied widely, possible as a result of nutritional factors, but have generally reported decreased concentrations of all polyunsaturated fatty acids rather than a specific deficiency of 22:6 n-3 (1)(2)(3)(4)(5).…”

A mechanism accounting for the low cellular level of linoleic acid in cystic fibrosis and its reversal by DHA

“…Decreased LA levels were among the earliest fatty acid abnormalities described in CF (3). Several studies reported the presence of low LA levels in different blood components of CF patients (4)(5)(6)(7). In addition, decreased LA levels were also found in CFTR-expressing tissues of CF patients, such as nasal tissue (8).…”