Horsley, AR, Belcher, J ORCID: https://orcid.org/0000-0001-6844-0932, Bayfield, K, Bianco, B, Cunningham, S, Fullwood, C, Jones, A, Shawcross, A, Smith, JA, Maitra, A and Gilchrist, FJ ORCID: https://orcid.org/0000-0002-1947-7621 (2021) Longitudinal assessment of lung clearance index to monitor disease progression in children and adults with cystic fibrosis. Thorax.

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Abstract

BACKGROUND: Lung clearance index (LCI) is a valuable research tool in cystic fibrosis (CF) but clinical application has been limited by technical challenges and uncertainty about how to interpret longitudinal change. In order to help inform clinical practice, this study aimed to assess feasibility, repeatability and longitudinal LCI change in children and adults with CF with predominantly mild baseline disease. METHODS: Prospective, 3-year, multicentre, observational study of repeated LCI measurement at time of clinical review in patients with CF >5 years, delivered using a rapid wash-in system. RESULTS: 112 patients completed at least one LCI assessment and 98 (90%) were still under follow-up at study end. The median (IQR) age was 14.7 (8.6-22.2) years and the mean (SD) FEV1 z-score was -1.2 (1.3). Of 81 subjects with normal FEV1 (>-2 z-scores), 63% had raised LCI (indicating worse lung function). For repeat stable measurements within 6 months, the mean (limits of agreement) change in LCI was 0.9% (-18.8% to 20.7%). A latent class growth model analysis identified four discrete clusters with high accuracy, differentiated by baseline LCI and FEV1. Baseline LCI was the strongest factor associated with longitudinal change. The median total test time was under 19 min. CONCLUSIONS: Most patients with CF with well-preserved lung function show stable LCI over time. Cluster behaviours can be identified and baseline LCI is a risk factor for future progression. These results support the use of LCI in clinical practice in identifying patients at risk of lung function decline.

Item Type: Article
Additional Information: The final version of this accepted manuscript and all relevant information related to it, including copyrights, can be found online at; https://thorax.bmj.com/content/early/2021/07/22/thoraxjnl-2021-216928
Subjects: R Medicine > RC Internal medicine > RC705 Diseases of the respiratory system
R Medicine > RJ Pediatrics
R Medicine > RJ Pediatrics > RJ101 Child Health. Child health services
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Depositing User: Symplectic
Date Deposited: 24 Sep 2021 13:11
Last Modified: 24 Sep 2021 13:11
URI: https://eprints.keele.ac.uk/id/eprint/10064

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