Determinants of Serum Glycerophospholipid Fatty Acids in Cystic Fibrosis.

Sławomira Drzymała-Czyż,Jan Nowak,Berthold Koletzko,Aleksandra Lisowska,Anna Miśkiewicz-Chotnicka,Jerzy Moczko,Jarosław Walkowiak,Patrycja Krzyżanowska

doi:10.3390/ijms18010185

Abstract

The etiology of altered blood fatty acid (FA) composition in cystic fibrosis (CF) is understood only partially. We aimed to investigate the determinants of serum glycerophospholipids’ FAs in CF with regard to the highest number of FAs and in the largest cohort to date. The study comprised 172 CF patients and 30 healthy subjects (HS). We assessed Fas’ profile (gas chromatography/mass spectrometry), CF transmembrane conductance regulator (CFTR) genotype, spirometry, fecal elastase-1, body height and weight Z-scores, liver disease, diabetes and colonization by Pseudomonas aeruginosa. The amounts of saturated FAs (C14:0, C16:0) and monounsaturated FAs (C16:1n-7, C18:1n-9, C20:1n-9, C20:3n-9) were significantly higher in CF patients than in HS. C18:3n-6, C20:3n-6 and C22:4n-6 levels were also higher in CF, but C18:2n-6, C20:2n-6 and C20:4n-6, as well as C22:6n-3, were lower. In a multiple regression analysis, levels of seven FAs were predicted by various sets of factors that included age, genotype, forced expiratory volume in one second, pancreatic status and diabetes. FA composition abnormalities are highly prevalent in CF patients. They seem to be caused by both metabolic disturbances and independent clinical risk factors. Further research into the influence of CFTR mutations on fat metabolism and desaturases’ activity is warranted.

Highlights

Introduction and AimsThe fatty acid (FA) profiles of serum and blood cells influence a range of metabolic, immune, and other functions [1]
The FA status of patients can be affected by diet or by disease-related changes in FA absorption or metabolism, which is the case in cystic fibrosis (CF) [2,8]
The FA composition of serum glycerophospholipids is a valuable biomarker of FA status in humans [3,27]

Summary

Introduction

The fatty acid (FA) profiles of serum and blood cells influence a range of metabolic, immune, and other functions [1]. The FA status of patients can be affected by diet (e.g., the consumption of fatty fish and their oils) or by disease-related changes in FA absorption or metabolism, which is the case in cystic fibrosis (CF) [2,8]. CF is the most common genetic disorder in the Caucasian population. It is caused by a dysfunction of the CF transmembrane conductance regulator (CFTR) [9]. Many groups have described abnormalities in the FA profile, which include an increased level of saturated fatty acids and C16:1n-7 (palmitoleic acid, POA) and C20:3n-9 (mead acid). The exact causes of these abnormalities are unknown and their disentanglement constitutes a current challenge in CF research [23]

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