dc.contributor.author |
Ronan, Nicola J. |
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dc.contributor.author |
Einarsson, Gisli G. |
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dc.contributor.author |
Twomey, Maria |
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dc.contributor.author |
Mooney, Denver |
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dc.contributor.author |
Mullane, David |
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dc.contributor.author |
NiChroinin, Muireann |
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dc.contributor.author |
O'Callaghan, Grace |
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dc.contributor.author |
Shanahan, Fergus |
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dc.contributor.author |
Murphy, Desmond M. |
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dc.contributor.author |
O'Connor, Owen J. |
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dc.contributor.author |
Shortt, Cathy A. |
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dc.contributor.author |
Tunney, Michael M. |
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dc.contributor.author |
Eustace, Joseph A. |
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dc.contributor.author |
Maher, Michael M. |
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dc.contributor.author |
Elborn, J. Stuart |
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dc.contributor.author |
Plant, Barry J. |
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dc.date.accessioned |
2017-11-16T11:41:00Z |
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dc.date.available |
2017-11-16T11:41:00Z |
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dc.date.issued |
2017-10-14 |
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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, 153(2), pp. 395-403. doi: 10.1016/j.chest.2017.10.005. |
en |
dc.identifier.volume |
153 |
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dc.identifier.issued |
2 |
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dc.identifier.startpage |
395 |
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dc.identifier.endpage |
403 |
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dc.identifier.issn |
0012-3692 |
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dc.identifier.uri |
http://hdl.handle.net/10468/5056 |
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dc.identifier.doi |
10.1016/j.chest.2017.10.005 |
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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 |
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dc.date.updated |
2017-11-13T18:18:27Z |
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dc.description.version |
Accepted Version |
en |
dc.internal.rssid |
418285767 |
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dc.internal.rssid |
418031145 |
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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.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 |