Omega-3 fatty acid supplementation for cystic fibrosis

Watson, Helen; Stackhouse, Caroline

doi:10.1002/14651858.cd002201.pub6

Cited by 24 publications

(23 citation statements)

References 34 publications

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“…A combination of EPA and DHA can reduce induced inflammation in mouse models of asthma ( Fussbroich et al, 2020 ). Treatment of F508del CFTR mice with a diet enriched in ω-3 PUFA reduces lung disease ( Portal et al, 2018 ), however, a recent Cochrane meta-analysis of five limited trials did not indicate that ω-3 PUFA dietary supplementation is beneficial for CF patients ( Watson and Stackhouse, 2020 ).…”

Section: Discussionmentioning

confidence: 99%

CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells

et al. 2021

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A deficiency in cystic fibrosis transmembrane conductance regulator (CFTR) function in CF leads to chronic lung disease. CF is associated with abnormalities in fatty acids, ceramides, and cholesterol, their relationship with CF lung pathology is not completely understood. Therefore, we examined the impact of CFTR deficiency on lipid metabolism and pro-inflammatory signaling in airway epithelium using mass spectrometric, protein array. We observed a striking imbalance in fatty acid and ceramide metabolism, associated with chronic oxidative stress under basal conditions in CF mouse lung and well-differentiated bronchial epithelial cell cultures of CFTR knock out pig and CF patients. Cell-autonomous features of all three CF models included high ratios of ω-6- to ω-3-polyunsaturated fatty acids and of long- to very long-chain ceramide species (LCC/VLCC), reduced levels of total ceramides and ceramide precursors. In addition to the retinoic acid analog fenretinide, the anti-oxidants glutathione (GSH) and deferoxamine partially corrected the lipid profile indicating that oxidative stress may promote the lipid abnormalities. CFTR-targeted modulators reduced the lipid imbalance and oxidative stress, confirming the CFTR dependence of lipid ratios. However, despite functional correction of CF cells up to 60% of non-CF in Ussing chamber experiments, a 72-h triple compound treatment (elexacaftor/tezacaftor/ivacaftor surrogate) did not completely normalize lipid imbalance or oxidative stress.Protein array analysis revealed differential expression and shedding of cytokines and growth factors from CF epithelial cells compared to non-CF cells, consistent with sterile inflammation and tissue remodeling under basal conditions, including enhanced secretion of the neutrophil activator CXCL5, and the T-cell activator CCL17. However, treatment with antioxidants or CFTR modulators that mimic the approved combination therapies, ivacaftor/lumacaftor and ivacaftor/tezacaftor/elexacaftor, did not effectively suppress the inflammatory phenotype.We propose that CFTR deficiency causes oxidative stress in CF airway epithelium, affecting multiple bioactive lipid metabolic pathways, which likely play a role in CF lung disease progression. A combination of anti-oxidant, anti-inflammatory and CFTR targeted therapeutics may be required for full correction of the CF phenotype.

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Section: Discussionmentioning

confidence: 99%

CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells

et al. 2021

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“…However, life history and intake of proinflammatory fatty acids, such as omega-6, are also important factors driving these outcomes ( 141 ). A recent systematic review of omega-3 supplementation for pwCF found mixed evidence of omega-3 supplementation on ppFEV 1 outcomes and the authors emphasized the need for additional randomized-control trials due to the low quality of the current evidence ( 145 ).…”

Section: Drivers Of Microbiota Alterationsmentioning

confidence: 99%

The Gut-Lung Axis in Cystic Fibrosis

Price

O’Toole

2021

J Bacteriol

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Cystic Fibrosis (CF) is a heritable, multi-organ disease that impacts all tissues that normally express CFTR protein. While importance of the airway microbiota has long been recognized, the intestinal microbiota has only recently been recognized as an important player in both intestinal and lung health outcomes for persons with CF (pwCF). Here, we summarize current literature related to the gut-lung axis in CF, with a particular focus on three key ideas: Mechanisms through which microbes influence the gut-lung axis, drivers of microbiota alterations, and the potential for intestinal microbiota remediation.

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“…As PUFAs cannot be synthesised in the human body, they must be obtained from one’s diet. Although the consumption of fatty acids increases their serum concentration [ 36 ], its supplementation is still controversial in CF patients due to poor clinical effects on the disorder course [ 37 , 38 ].…”

Section: Discussionmentioning

confidence: 99%

DeltaF508 CFTR Hetero- and Homozygous Paediatric Patients with Cystic Fibrosis Do Not Differ with Regard to Nutritional Status

Mędza

Kaźmierska²,

Wielgomas

et al. 2021

Nutrients

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The purpose of this study was to compare the nutritional status between deltaF508 CFTR hetero- and homozygous paediatric patients with cystic fibrosis. We assessed the percentage profiles of fatty acids measured in erythrocyte membranes and the serum levels of vitamins A, D3, E and K1 in the studied groups. We also measured the weights and heights and calculated the body mass indexes (BMIs). The studied groups consisted of 34 heterozygous and 30 homozygous patients. No statistically significant differences were found in the serum vitamins or erythrocyte membrane fatty acid profiles between the hetero- and homozygous patient groups, except for heptadecanoic acid (p = 0.038). The mean percentiles of height, weight and BMI did not differ significantly between the two groups. The homozygous and heterozygous paediatric patients with cystic fibrosis were similar in terms of their nutritional statuses.

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Omega-3 fatty acid supplementation for cystic fibrosis

Cited by 24 publications

References 34 publications

CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells

CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells

The Gut-Lung Axis in Cystic Fibrosis

DeltaF508 CFTR Hetero- and Homozygous Paediatric Patients with Cystic Fibrosis Do Not Differ with Regard to Nutritional Status

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