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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., …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..

Thinking outside the box: a review of gastrointestinal symptoms and complications in cystic fibrosis (2023)
Journal Article
Yule, A., Sills, D., Smith, S., Spiller, R., & Smyth, A. R. (2023). Thinking outside the box: a review of gastrointestinal symptoms and complications in cystic fibrosis. Expert Review of Respiratory Medicine, 17(7), 547-561. https://doi.org/10.1080/17476348.2023.2228194

Introduction Gastrointestinal (GI)-related symptoms, complications, and comorbidities in cystic fibrosis (CF) are common and research to reduce their burden is a priority for the CF community. To enable future research, this review aimed to summariz... Read More about Thinking outside the box: a review of gastrointestinal symptoms and complications in cystic fibrosis.

A refresh of the top 10 research priorities in cystic fibrosis (2023)
Journal Article
Rowbotham, N. J., Smith, S., Elliott, Z. C., Cupid, B., Allen, L. J., Cowan, K., …Smyth, A. R. (2023). A refresh of the top 10 research priorities in cystic fibrosis. Thorax, 78(8), 840-843. https://doi.org/10.1136/thorax-2023-220100

In 2018 we published the James Lind Alliance (JLA) top 10 priorities for clinical research in cystic fibrosis (CF), chosen jointly by the patient and clinical communities. These priorities have led to new research funding. To establish whether priori... Read More about A refresh of the top 10 research priorities in cystic fibrosis.

A grumbling concern: an international survey of gastrointestinal symptoms in cystic fibrosis in the modulator era [version 1; peer review: awaiting peer review] (2023)
Journal Article
Calthorpe, R. J., Goodchild, N., Gleetus, V., Premakumar, V., Hayee, B., Elliott, Z., …Smyth, A. R. (2023). A grumbling concern: an international survey of gastrointestinal symptoms in cystic fibrosis in the modulator era [version 1; peer review: awaiting peer review]. NIHR Open Research, 3, Article 18. https://doi.org/10.3310/nihropenres.13384.1

Background: Gastrointestinal symptoms in cystic fibrosis (CF) are common and intrusive to daily life. Relieving gastrointestinal symptoms was identified as an important research priority and previously explored in an international survey in 2018. How... Read More about A grumbling concern: an international survey of gastrointestinal symptoms in cystic fibrosis in the modulator era [version 1; peer review: awaiting peer review].

Reporting Standards for Diagnostic Testing: Guidance for Authors From Editors of Respiratory, Sleep, and Critical Care Journals (2023)
Journal Article
Ost, D. E., Feller-Kopman, D. J., Gonzalez, A. V., Grosu, H. B., Herth, F., Mazzone, P., …Smyth, A. (2023). Reporting Standards for Diagnostic Testing: Guidance for Authors From Editors of Respiratory, Sleep, and Critical Care Journals. Journal of Bronchology & Interventional Pulmonology, 30(3), 207-222. https://doi.org/10.1097/LBR.0000000000000920

Diagnostic testing is fundamental to medicine. However, studies of diagnostic testing in respiratory medicine vary significantly in terms of their methodology, definitions, and reporting of results. This has led to often conflicting or ambiguous resu... Read More about Reporting Standards for Diagnostic Testing: Guidance for Authors From Editors of Respiratory, Sleep, and Critical Care Journals.

Complex roles of TGF-β signaling pathways in lung development and bronchopulmonary dysplasia (2023)
Journal Article
Calthorpe, R. J., Poulter, C., Smyth, A. R., Sharkey, D., Bhatt, J., Jenkins, G., & Tatler, A. L. (2023). Complex roles of TGF-β signaling pathways in lung development and bronchopulmonary dysplasia. AJP - Lung Cellular and Molecular Physiology, 324(3), L285-L296. https://doi.org/10.1152/ajplung.00106.2021

As survival of extremely preterm infants continues to improve, there is also an associated increase in bronchopulmonary dysplasia (BPD), one of the most significant complications of preterm birth. BPD development is multifactorial resulting from expo... Read More about Complex roles of TGF-β signaling pathways in lung development and bronchopulmonary dysplasia.

Future therapies for cystic fibrosis (2023)
Journal Article
Allen, L., Allen, L., Carr, S. B., Davies, G., Downey, D., Egan, M., …Davies, J. C. (2023). Future therapies for cystic fibrosis. Nature Communications, 14(1), Article 693. https://doi.org/10.1038/s41467-023-36244-2

We are currently witnessing transformative change for people with cystic fibrosis with the introduction of small molecule, mutation-specific drugs capable of restoring function of the defective protein, cystic fibrosis transmembrane conductance regul... Read More about Future therapies for cystic fibrosis.

Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis (2023)
Journal Article
Smith, S., Calthorpe, R., Herbert, S., & Smyth, A. R. (2023). Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis. Cochrane Database of Systematic Reviews, 2023(2), Article CD013733. https://doi.org/10.1002/14651858.cd013733.pub2

Background: Improved understanding and treatment of cystic fibrosis (CF) has led to longer life expectancy, which is accompanied by an increasingly complex regimen of treatments.Suboptimal adherence to the treatment plan, in the context of respirator... Read More about Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis.

Prebiotics for people with cystic fibrosis (2022)
Journal Article
Williams, N., Jayaratnasingam, J., Prayle, A. P., Nevitt, S. J., & Smyth, A. R. (2022). Prebiotics for people with cystic fibrosis. Cochrane Database of Systematic Reviews, 2022(12), Article CD015236. https://doi.org/10.1002/14651858.cd015236

Background. Description of the condition. Cystic fibrosis (CF) is a life‐limiting genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It affects approximately 100,000 children and adults worldwi... Read More about Prebiotics for people with cystic fibrosis.

Interventions for the eradication of meticillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis (2022)
Journal Article
Smyth, A. R., Lo, D. K., & Muhlebach, M. S. (2022). Interventions for the eradication of meticillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis. Cochrane Database of Systematic Reviews, 2022(12), Article CD009650. https://doi.org/10.1002/14651858.cd009650.pub5

Background Cystic fibrosis is an inherited recessive disorder of chloride transport that is characterised by recurrent and persistent pulmonary infections from resistant organisms that result in lung function deterioration and early mortality in suf... Read More about Interventions for the eradication of meticillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis.

Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis (2022)
Journal Article
Southern, K. W., Castellani, C., Lammertyn, E., Smyth, A., VanDevanter, D., van Koningsbruggen-Rietschel, S., …Duff, A. (2023). Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis. Journal of Cystic Fibrosis, 22(1), 17-30. https://doi.org/10.1016/j.jcf.2022.10.002

Cystic fibrosis (CF) has entered the era of variant-specific therapy, tailored to the genetic variants in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. CFTR modulators, the first variant-specific therapy available, have transfo... Read More about Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis.

Protocol for establishing a core outcome set for evaluation in studies of pulmonary exacerbations in people with cystic fibrosis (2022)
Journal Article
McLeod, C., Smyth, A. R., Messer, M., Schultz, A., Wood, J., Norman, R., …Snelling, T. L. (2022). Protocol for establishing a core outcome set for evaluation in studies of pulmonary exacerbations in people with cystic fibrosis. BMJ Open, 12(9), Article e056528. https://doi.org/10.1136/bmjopen-2021-056528

INTRODUCTION: Pulmonary exacerbations are associated with increased morbidity and mortality in people with cystic fibrosis (CF). There is no consensus about which outcomes should be evaluated in studies of pulmonary exacerbations or how these outcome... Read More about Protocol for establishing a core outcome set for evaluation in studies of pulmonary exacerbations in people with cystic fibrosis.

Wheeze in the time of COVID-19: overcoming obstacles to an unusual diagnosis (2022)
Journal Article
Barclay, M., Buderi, S., Bush, A., Daniel, M., Jordan, S., Rice, A., …Smyth, A. R. (2022). Wheeze in the time of COVID-19: overcoming obstacles to an unusual diagnosis. Thorax, 77(10), 1050-1053. https://doi.org/10.1136/thoraxjnl-2021-218526

This case is an example of a rare cause of a common clinical presentation (persistent lobar collapse with wheeze). We describe patient management from primary care through to a national thoracic referral centre. We highlight the importance of objecti... Read More about Wheeze in the time of COVID-19: overcoming obstacles to an unusual diagnosis.

Industry influence in healthcare harms patients: myth or maxim? (2022)
Journal Article
Trayer, J., Rowbotham, N. J., Boyle, R. J., & Smyth, A. R. (2022). Industry influence in healthcare harms patients: myth or maxim?. Breathe, 18(2), Article 220010. https://doi.org/10.1183/20734735.0010-2022

Healthcare is a major global industry accounting for a significant proportion of government spending. Drug and medical device manufacturers are publicly traded companies with a responsibility to their shareholders to maximise profits by increasing sa... Read More about Industry influence in healthcare harms patients: myth or maxim?.

Parenchymal lung abnormalities following hospitalisation for COVID-19 and viral pneumonitis: a systematic review and meta-analysis (2022)
Journal Article
Fabbri, L., Moss, S., Khan, F. A., Chi, W., Xia, J., Robinson, K., …Stewart, I. (2023). Parenchymal lung abnormalities following hospitalisation for COVID-19 and viral pneumonitis: a systematic review and meta-analysis. Thorax, 78(2), 191-201. https://doi.org/10.1136/thoraxjnl-2021-218275

Introduction Persisting respiratory symptoms in COVID-19 survivors may be related to development of pulmonary fibrosis. We assessed the proportion of chest CT scans and pulmonary function tests consistent with parenchymal lung disease in the follow-u... Read More about Parenchymal lung abnormalities following hospitalisation for COVID-19 and viral pneumonitis: a systematic review and meta-analysis.

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., …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.

Daily Bread: Women’s Self-Help Microfinance and the Social Meanings of Money (2022)
Journal Article
Bott, E., Ojha, S., Mini, S., Smyth, A. R., Kamal Kumar, R., Choudhary, S., & Yaron, G. (2023). Daily Bread: Women’s Self-Help Microfinance and the Social Meanings of Money. Sociological Research Online, 28(2), 442-461. https://doi.org/10.1177/13607804211058745

In this article, we explore the impacts and implications of ‘Rojiroti’, a women’s self-help group (SHG) microfinance scheme operating in poor communities in Bihar, India. We focus particularly on how improvements found in women’s circumstances and in... Read More about Daily Bread: Women’s Self-Help Microfinance and the Social Meanings of Money.

Perspectives of patients, family members, health professionals and the public on the impact of COVID-19 on mental health (2022)
Journal Article
Gardiner, E., Baumgart, A., Tong, A., Elliott, J. H., Azevedo, L. C., Bersten, A., …Craig, J. C. (2022). Perspectives of patients, family members, health professionals and the public on the impact of COVID-19 on mental health. Journal of Mental Health, 31(4), 524-533. https://doi.org/10.1080/09638237.2021.2022637

Background The coronavirus (COVID-19) pandemic has seen a global surge in anxiety, depression, post-traumatic stress disorder (PTSD), and stress. Aims This study aimed to describe the perspectives of patients with COVID-19, their family, health... Read More about Perspectives of patients, family members, health professionals and the public on the impact of COVID-19 on mental health.

Working in partnership with the patient community to develop outline trial designs in CF (2021)
Journal Article
Rowbotham, N. J., Smith, S., Davies, G., Leighton, P., Rayner, O., Elliott, Z., …Smyth, A. R. (2022). Working in partnership with the patient community to develop outline trial designs in CF. Journal of Cystic Fibrosis, 21(2), 300-301. https://doi.org/10.1016/j.jcf.2021.12.008

In a collaboration between health professionals and the patient community, we previously identified the Top 10 priorities for clinical research in CF [ [1] ]. Here, we have used a similar approach to explore four of these priorities further and sugge... Read More about Working in partnership with the patient community to develop outline trial designs in CF.

Intestinal function and transit associate with gut microbiota dysbiosis in cystic fibrosis (2021)
Journal Article
Marsh, R., Gavillet, H., Hanson, L., Ng, C., Mitchell-Whyte, M., Major, G., …van der Gast, C. (2022). Intestinal function and transit associate with gut microbiota dysbiosis in cystic fibrosis. Journal of Cystic Fibrosis, https://doi.org/10.1016/j.jcf.2021.11.014

Background: Most people with cystic fibrosis (pwCF) suffer from gastrointestinal symptoms and are at risk of gut complications. Gut microbiota dysbiosis is apparent within the CF population across all age groups, with evidence linking dysbiosis to in... Read More about Intestinal function and transit associate with gut microbiota dysbiosis in cystic fibrosis.