Objectives: To assess fatty acid (FA) profiles in whole blood of 90 cystic fibrosis patients (CF) and 30 control subjects (C) and to correlate FA changes to the severity of respiratory disease.Methods: Whole blood FA were assessed by GC with a micromethod-based analysis.Results: Saturated and monounsaturated FA are higher, whereas polyunsaturated FA are lower in CF versus C with reduction of total n-6 FA, 22:5n-3 and 22:6n-3 (DHA). The product of linoleic acid (LA) x DHA, proposed as a marker for the disease, is 30% lower in CF than in C. Correlations with the severity of the respiratory disease are present for different FA and for the LA x DHA product. There is a reduction of Delta 5 desaturase activity in CF, greater in severe disease, suggesting a basic metabolic alteration.Conclusions: The micromethod-based analysis of blood FA facilitates the assessment of the FA status while confirming alterations of FA profiles already reported in specific blood compartments of CF. (C) 2010 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

OBJECTIVE:To assess the relationship between dietary intakes, plasma phospholipid (PL) fatty acid profile and clinical parameters in children with cystic fibrosis (CF) in comparison to healthy controls. PATIENTS AND METHODS:A cross-sectional survey including 37 patients with CF (ages 8.0 +/- 2.9 yrs) and a reference group of 68 healthy children (ages 8.0 +/- 0.7 yrs) was carried out by means of a food-frequency questionnaire. At enrollment, all subjects underwent blood sampling for plasma PL fatty acids (FA). In CF patients, pulmonary function tests (forced expiratory volume in 1 second and forced vital capacity), anthropometric measurements and the Shwachman score were also determined. RESULTS:In CF patients, mean z score for weight and height (-0.35 +/- 1.16 and -0.28 +/- 0.99) were lower than controls (0.83 +/- 1.73 and 0.55 +/- 1.11, respectively). Patients with CF showed higher energy intakes (110 +/- 43 kcal/d) compared with controls (75 +/- 22 kcal/d; P < 0.0001), with higher intake of total (saturated and monounsaturated) fats and lower intake of polyunsaturated FA (3.9 +/- 1.0% of total macronutrient intake vs 4.3 +/- 1.2%, P = 0.05). In CF patients, plasma and PL levels of linoleic and docosahexaenoic acids were lower, whereas those of arachidonic acid were similar compared with controls. The Shwachman score showed significant positive associations with plasma PL levels of arachidonic acid and total n-6 long-chain FA (r = 0.32, P = 0.05, and r = 0.35, P = 0.03, respectively). CONCLUSIONS:The data give suggestions that fat intake and CF-associated biomechanisms are bound in a vicious circle, concurring to create the clinical and biochemical picture of CF. The quantity and quality of fat supplementation in CF need careful attention to balance the fat supply with polyunsaturated FA.

It has been suggested that genes other than CFTR could modulate the severity of lung disease in cystic fibrosis (CF). Neutrophil Fcγ receptor II (FcγRII) is involved in host defense against microorganisms and in inflammatory response. We evaluated the association between genetic variability of this gene and both airway infection with Pseudomonas aeruginosa and severity of lung disease in patients with CF. We studied 167 Italian unrelated patients with CF and 50 control subjects. The distribution of FcγRIIA genotypes in CF patients was compared with that in control subjects and the different genotypes were related with the presence or absence of P. aeruginosa infection and markers of disease severity in CF patients. The distribution of FcγRIIA genotypes was not significantly different between CF patients and controls. We observed that in CF patients with the same CFTR genotype (ΔF508/ΔF508), those carrying the R allele of FcγRIIA had an increased risk of acquiring chronic P. aeruginosa infection (P=0.042, R.R.: 4.38; 95% CI: 1.17÷22.4). Moreover, the frequency of R/R genotype in patients with chronic P. aeruginosa infection seems to be higher than that of control subjects and patients without chronic infection. The observation that CF patients carrying the R allele of FcγRIIA are at higher risk of acquiring chronic P. aeruginosa infection suggests that the FcγRII loci genetic variation is contributing to this infection susceptibility.