Journal
JOURNAL OF CYSTIC FIBROSIS
Volume 12, Issue 3, Pages 222-228Publisher
ELSEVIER
DOI: 10.1016/j.jcf.2012.09.004
Keywords
Cystic fibrosis; Lung transplantation; Essential fatty acid; Poly-unsaturated fatty acid
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Background: Cystic fibrosis (CF) can be a devastating disease. Disorders in essential fatty acid state are increasingly reported and various supplementation trials have been performed in an attempt to improve outcomes. However, the mechanisms leading to these disturbances remain elusive. We wanted to investigate the role of the diseased CF lung on fatty acid profiles. Methods: We compared fatty acid profiles in patients with CF after lung transplantation (n=11) to age-matched healthy controls and homozygous F508del patients (n=22 each). Results: Compared to healthy controls, in patients with CF, there are decreased levels of docosahexaenoic, linoleic and arachidonic acid and increased levels of mead acid. In patients that underwent a lung transplantation, levels of docosahexaenoic, linoleic and arachidonic acid were normal. Mead acid did not decrease significantly. Conclusions: The diseased CFTR deficient lung is a major determinant in the disturbed fatty acid profile in CF. (C) 2012 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
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