2-Alkyl-4-quinolone quorum sensing molecules are biomarkers for culture-independent Pseudomonas aeruginosa burden in adults with cystic fibrosis

Zain, Nur Masirah M.; Webb, Karmel; Stewart, Iain; Halliday, Nigel; Barrett, David A.; Nash, Edward F.; Whitehouse, Joanna L.; Honeybourne, David; Smyth, Alan R.; Forrester, Douglas L.; Knox, Alan J.; Williams, Paul; Fogarty, Andrew; Cámara, Miguel; Bruce, Kenneth D.; Barr, Helen L.

doi:10.1099/jmm.0.001420

All Outputs (7)

CF Tummy Tracker: A Cystic Fibrosis–Specific Patient-Reported Outcome Measure for Daily Gastrointestinal Symptom Burden (2025)
Journal Article
Calthorpe, R. J., Saumtally, H. A., Howells, L. M., Goodchild, N. J., Evans, B. C., Elliott, Z., Hayee, B. H., Carr, S. B., Elston, C. M., Horsley, A. A., Peckham, D. G., Barr, H. L., Major, G. A., Stewart, I. D., Thomas, K. S., & Smyth, A. R. (2025). CF Tummy Tracker: A Cystic Fibrosis–Specific Patient-Reported Outcome Measure for Daily Gastrointestinal Symptom Burden. Mayo Clinic Proceedings: Digital Health, 3(2), Article 100203. https://doi.org/10.1016/j.mcpdig.2025.100203

Objective
To develop a cystic fibrosis (CF)–specific patient-reported outcome measure (PROM) to measure the daily burden of gastrointestinal symptoms for people with cystic fibrosis (pwCF) aged 12 years and older and address the lack of validated ou... Read More about CF Tummy Tracker: A Cystic Fibrosis–Specific Patient-Reported Outcome Measure for Daily Gastrointestinal Symptom Burden.

A longitudinal study assessing the impact of elexacaftor/tezacaftor/ivacaftor on gut transit and function in people with cystic fibrosis using magnetic resonance imaging (MRI) (2024)
Journal Article
Yule, A., Ng, C., Recto, A., Lockwood, F., Dellschaft, N. S., Hoad, C. L., Zagoya, C., Mainz, J. G., Major, G., Barr, H. L., Gowland, P. A., Stewart, I., Marciani, L., Spiller, R. C., & Smyth, A. R. (2024). A longitudinal study assessing the impact of elexacaftor/tezacaftor/ivacaftor on gut transit and function in people with cystic fibrosis using magnetic resonance imaging (MRI). Journal of Cystic Fibrosis, 23(5), 984-990. https://doi.org/10.1016/j.jcf.2024.08.001

Background

Gastrointestinal (GI) symptoms in cystic fibrosis (CF) are common and disruptive. The effect of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on the GI tract is not fully understood. The aim was to use magnetic r... Read More about A longitudinal study assessing the impact of elexacaftor/tezacaftor/ivacaftor on gut transit and function in people with cystic fibrosis using magnetic resonance imaging (MRI).

A randomised crossover trial of tezacaftor-ivacaftor for gut dysfunction in cystic fibrosis with magnetic resonance imaging (MRI) outcomes: a pilot study (2024)
Journal Article
Ng, C., Dellschaft, N. S., Hoad, C., Marciani, L., Spiller, R., Crooks, C., Hill, T., Menys, A., Mainz, J. G., Barr, H., Gowland, P. A., Major, G., & Smyth, A. R. (2024). A randomised crossover trial of tezacaftor-ivacaftor for gut dysfunction in cystic fibrosis with magnetic resonance imaging (MRI) outcomes: a pilot study. NIHR Open Research, 3, Article 65. https://doi.org/10.3310/nihropenres.13510.2

Background
People with cystic fibrosis (CF) can experience recurrent chest infections, pancreatic exocrine insufficiency and gastrointestinal symptoms. New cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs improve lung funct... Read More about A randomised crossover trial of tezacaftor-ivacaftor for gut dysfunction in cystic fibrosis with magnetic resonance imaging (MRI) outcomes: a pilot study.

A randomised crossover trial of tezacaftor-ivacaftor for gut dysfunction in cystic fibrosis with magnetic resonance imaging (MRI) outcomes. (2023)
Journal Article
Ng, C., Dellschaft, N. S., Hoad, C., Marciani, L., Spiller, R., Crooks, C., Hill, T., Menys, A., Mainz, J. G., Barr, H., Gowland, P. A., Major, G., & Smyth, A. R. (2023). A randomised crossover trial of tezacaftor-ivacaftor for gut dysfunction in cystic fibrosis with magnetic resonance imaging (MRI) outcomes. NIHR Open Research, 3(65), 1-16. https://doi.org/10.3310/nihropenres.13510.1

Background

People with cystic fibrosis (CF) can experience recurrent chest infections, pancreatic exocrine insufficiency and gastrointestinal symptoms. New cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs improve lung fun... Read More about A randomised crossover trial of tezacaftor-ivacaftor for gut dysfunction in cystic fibrosis with magnetic resonance imaging (MRI) outcomes..

Porphyromonas pasteri and Prevotella nanceiensis in the sputum microbiota are associated with increased decline in lung function in individuals with cystic fibrosis (2022)
Journal Article
Webb, K., Zain, N. M. M., Stewart, I., Fogarty, A., Nash, E. F., Whitehouse, J. L., Smyth, A. R., Lilley, A. K., Knox, A., Williams, P., Cámara, M., Bruce, K., & Barr, H. L. (2022). Porphyromonas pasteri and Prevotella nanceiensis in the sputum microbiota are associated with increased decline in lung function in individuals with cystic fibrosis. Journal of Medical Microbiology, 71(2), Article 001481. https://doi.org/10.1099/jmm.0.001481

Although anaerobic bacteria exist in abundance in cystic fibrosis (CF) airways, their role in disease progression is poorly understood. We hypothesized that the presence and relative abundance of the most prevalent, live, anaerobic bacteria in sputum... Read More about Porphyromonas pasteri and Prevotella nanceiensis in the sputum microbiota are associated with increased decline in lung function in individuals with cystic fibrosis.

Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial (2021)
Journal Article
Sutharsan, S., McKone, E. F., Downey, D. G., Duckers, J., MacGregor, G., Tullis, E., Van Braeckel, E., Wainwright, C. E., Watson, D., Ahluwalia, N., Bruinsma, B. G., Harris, C., Lam, A. P., Lou, Y., Moskowitz, S. M., Tian, S., Yuan, J., Waltz, D., Mall, M. A., Aurora, P., …Wark, P. (2022). Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial. Lancet Respiratory Medicine, 10(3), 267-277. https://doi.org/10.1016/S2213-2600%2821%2900454-9

Background: Elexacaftor plus tezacaftor plus ivacaftor is a triple-combination cystic fibrosis transmembrane conductance regulator (CFTR) modulator regimen shown to be generally safe and efficacious in people with cystic fibrosis aged 12 years or old... Read More about Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial.

2-Alkyl-4-quinolone quorum sensing molecules are biomarkers for culture-independent Pseudomonas aeruginosa burden in adults with cystic fibrosis (2021)
Journal Article
Zain, N. M. M., Webb, K., Stewart, I., Halliday, N., Barrett, D. A., Nash, E. F., Whitehouse, J. L., Honeybourne, D., Smyth, A. R., Forrester, D. L., Knox, A. J., Williams, P., Fogarty, A., Cámara, M., Bruce, K. D., & Barr, H. L. (2021). 2-Alkyl-4-quinolone quorum sensing molecules are biomarkers for culture-independent Pseudomonas aeruginosa burden in adults with cystic fibrosis. Journal of Medical Microbiology, 70(10), https://doi.org/10.1099/jmm.0.001420

Introduction. Pseudomonas aeruginosa produces quorum sensing signalling molecules including 2-alkyl-4-quinolones (AQs), which regulate virulence factor production in the cystic fibrosis (CF) airways. Hypothesis/Gap statement. Culture can lead to cond... Read More about 2-Alkyl-4-quinolone quorum sensing molecules are biomarkers for culture-independent Pseudomonas aeruginosa burden in adults with cystic fibrosis.