CORK study in cystic fibrosis: sustained improvements in ultra-low-dose chest CT scores after CFTR modulation with ivacaftor

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dc.contributor.author Ronan, Nicola J.
dc.contributor.author Einarsson, Gisli G.
dc.contributor.author Twomey, Maria
dc.contributor.author Mooney, Denver
dc.contributor.author Mullane, David
dc.contributor.author NiChroinin, Muireann
dc.contributor.author O'Callaghan, Grace
dc.contributor.author Shanahan, Fergus
dc.contributor.author Murphy, Desmond M.
dc.contributor.author O'Connor, Owen J.
dc.contributor.author Shortt, Cathy A.
dc.contributor.author Tunney, Michael M.
dc.contributor.author Eustace, Joseph A.
dc.contributor.author Maher, Michael M.
dc.contributor.author Elborn, J. Stuart
dc.contributor.author Plant, Barry J.
dc.date.accessioned 2017-11-16T11:41:00Z
dc.date.available 2017-11-16T11:41:00Z
dc.date.issued 2017-10-14
dc.identifier.citation Ronan, N. J., Einarsson, G. G., Twomey, M., Mooney, D., Mullane, D., NiChroinin, M., O’Callaghan, G., Shanahan, F., Murphy, D. M., O’Connor, O. J., Shortt, C. A., Tunney, M. M., Eustace, J. A., Maher, M. M., Elborn, J. S. and Plant, B. J. (2017) ‘CORK study in cystic fibrosis: sustained improvements in ultra-low-dose chest CT scores after CFTR modulation with ivacaftor’, Chest. doi: 10.1016/j.chest.2017.10.005. en
dc.identifier.issn 0012-3692
dc.identifier.uri http://hdl.handle.net/10468/5056
dc.identifier.doi 10.1016/j.chest.2017.10.005
dc.description.abstract Background: Ivacaftor produces significant clinical benefit in patients with cystic fibrosis (CF) with the G551D mutation. Prevalence of this mutation at the Cork CF Centre is 23%. This study assessed the impact of CFTR modulation on multiple modalities of patient assessment. Methods: Thirty-three patients with the G551D mutation were assessed at baseline and prospectively every 3 months for 1 year after initiation of ivacaftor. Change in ultra-low-dose chest CT scans, blood inflammatory mediators, and the sputum microbiome were assessed. Results: Significant improvements in FEV1, BMI, and sweat chloride levels were observed post-ivacaftor treatment. Improvement in ultra-low-dose CT imaging scores were observed after treatment, with significant mean reductions in total Bhalla score (P < .01), peribronchial thickening (P = .035), and extent of mucous plugging (P < .001). Reductions in circulating inflammatory markers, including interleukin (IL)-1β, IL-6, and IL-8 were demonstrated. There was a 30% reduction in the relative abundance of Pseudomonas species and an increase in the relative abundance of bacteria associated with more stable community structures. Posttreatment community richness increased significantly (P = .03). Conclusions: Early and sustained improvements on ultra-low-dose CT scores suggest it may be a useful method of evaluating treatment response. It paralleled improvement in symptoms, circulating inflammatory markers, and changes in the lung microbiota. en
dc.format.mimetype application/pdf en
dc.language.iso en en
dc.publisher Elsevier Inc. on behalf of the American College of Chest Physicians en
dc.rights © 2017, American College of Chest Physicians. Published by Elsevier Inc. All rights reserved. This manuscript version is made available under the CC BY-NC-ND 4.0 license. en
dc.rights.uri http://creativecommons.org/licenses/by-nc-nd/4.0/ en
dc.subject Cystic fibrosis en
dc.subject Ivacaftor en
dc.subject G551D en
dc.subject Low-dose chest CT imaging en
dc.title CORK study in cystic fibrosis: sustained improvements in ultra-low-dose chest CT scores after CFTR modulation with ivacaftor en
dc.type Article (peer-reviewed) en
dc.internal.authorcontactother Barry Plant, Medicine Department, University College Cork, Cork, Ireland. +353-21-490-3000 Email: b.plant@ucc.ie en
dc.internal.availability Full text available en
dc.check.info Access to this item is restricted for 12 months after publication by request of the publisher. en
dc.check.date 2018-10-14
dc.date.updated 2017-11-13T18:18:27Z
dc.description.version Accepted Version en
dc.internal.rssid 418285767
dc.internal.rssid 418031145
dc.contributor.funder Seventh Framework Programme en
dc.description.status Peer reviewed en
dc.identifier.journaltitle Chest en
dc.internal.copyrightchecked Yes en
dc.internal.licenseacceptance Yes en
dc.internal.IRISemailaddress b.plant@ucc.ie en
dc.internal.IRISemailaddress oj.oconnor@ucc.ie en
dc.internal.bibliocheck In Press. Add vol. / issue / page range / supplementary data. Amend citation.
dc.relation.project info:eu-repo/grantAgreement/EC/FP7::SP1::HEALTH/603038/EU/Cystic Fibrosis Microbiome-determined Antibiotic Therapy Trial in Exacerbations: Results Stratified./CFMATTERS en


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© 2017, American College of Chest Physicians. Published by Elsevier Inc. All rights reserved. This manuscript version is made available under the CC BY-NC-ND 4.0 license. Except where otherwise noted, this item's license is described as © 2017, American College of Chest Physicians. Published by Elsevier Inc. All rights reserved. This manuscript version is made available under the CC BY-NC-ND 4.0 license.
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