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Int. J. Mol. Sci. 2017, 18(1), 185;

Determinants of Serum Glycerophospholipid Fatty Acids in Cystic Fibrosis

Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan University of Medical Sciences, Szpitalna 27/33, 60-572 Poznań, Poland
Ludwig-Maximilians-Universität München, Division of Metabolic and Nutritional Medicine, Dr. von Hauner Children’s Hospital, University of Munich Medical Center, Lindwurmstr. 4, D-80337 Munich, Germany
Department of Computer Science and Statistics, Poznan University of Medical Sciences, Dąbrowskiego 79, 60-529 Poznań, Poland
Author to whom correspondence should be addressed.
Academic Editor: Giovanni Tarantino
Received: 6 December 2016 / Revised: 29 December 2016 / Accepted: 4 January 2017 / Published: 18 January 2017
(This article belongs to the Section Molecular Pathology, Diagnostics, and Therapeutics)
Full-Text   |   PDF [244 KB, uploaded 18 January 2017]


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. View Full-Text
Keywords: phospholipids; polyunsaturated fatty acid; docosahexaenoic acid; eicosapentaenoic acid; arachidonic acid phospholipids; polyunsaturated fatty acid; docosahexaenoic acid; eicosapentaenoic acid; arachidonic acid
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

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Drzymała-Czyż, S.; Krzyżanowska, P.; Koletzko, B.; Nowak, J.; Miśkiewicz-Chotnicka, A.; Moczko, J.A.; Lisowska, A.; Walkowiak, J. Determinants of Serum Glycerophospholipid Fatty Acids in Cystic Fibrosis. Int. J. Mol. Sci. 2017, 18, 185.

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