The role of genetic modifiers, inflammation and CFTR in the pathogenesis of Cystic fibrosis related diabetes

The development of cystic fibrosis-related diabetes (CFRD) often leads to poorer outcomes in patients with cystic fibrosis including increases in pulmonary exacerbations, poorer lung function and early mortality. This review highlights the many factors contributing to the clinical decline seen in patients diagnosed with CFRD, highlighting the important role of nutrition, the direct effect of hyperglycaemia on the lungs, the immunomodulatory effects of high glucose levels and the potential role of genetic modifiers in CFRD.

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Quality of life and psychological distress in patients with cystic fibrosis: The role of (un)favourable illness representations

PsycEXTRA Dataset ◽

10.1037/e524362011-063 ◽

2008 ◽

Author(s):

A. Casier ◽

L. Goubert ◽

D. Huse ◽

M. Theunis ◽

G. Crombez

Keyword(s):

Quality Of Life ◽

Cystic Fibrosis ◽

Psychological Distress ◽

Illness Representations

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Systems Biology and Bile Acid Signalling in Microbiome-Host Interactions in the Cystic Fibrosis Lung

Antibiotics ◽

10.3390/antibiotics10070766 ◽

2021 ◽

Vol 10 (7) ◽

pp. 766

Author(s):

David F. Woods ◽

Stephanie Flynn ◽

Jose A. Caparrós-Martín ◽

Stephen M. Stick ◽

F. Jerry Reen ◽

...

Keyword(s):

Cystic Fibrosis ◽

Bile Acids ◽

Host Response ◽

Signal Molecules ◽

Therapeutic Approaches ◽

Related Factors ◽

Host Interactions ◽

Important Host ◽

Respiratory Microbiota

The study of the respiratory microbiota has revealed that the lungs of healthy and diseased individuals harbour distinct microbial communities. Imbalances in these communities can contribute to the pathogenesis of lung disease. How these imbalances occur and establish is largely unknown. This review is focused on the genetically inherited condition of Cystic Fibrosis (CF). Understanding the microbial and host-related factors that govern the establishment of chronic CF lung inflammation and pathogen colonisation is essential. Specifically, dissecting the interplay in the inflammation–pathogen–host axis. Bile acids are important host derived and microbially modified signal molecules that have been detected in CF lungs. These bile acids are associated with inflammation and restructuring of the lung microbiota linked to chronicity. This community remodelling involves a switch in the lung microbiota from a high biodiversity/low pathogen state to a low biodiversity/pathogen-dominated state. Bile acids are particularly associated with the dominance of Proteobacterial pathogens. The ability of bile acids to impact directly on both the lung microbiota and the host response offers a unifying principle underpinning the pathogenesis of CF. The modulating role of bile acids in lung microbiota dysbiosis and inflammation could offer new potential targets for designing innovative therapeutic approaches for respiratory disease.

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