Prospective longitudinal association between repeated multiple breath washout measurements and computed tomography scores in children with cystic fibrosis
Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
Background: Progression of structural lung disease (SLD) is a major risk factor for morbidity in patients with cystic fibrosis (CF). We studied changes in SLD and correlations with spirometry and nitrogen multiple breath washout (N2MBW) outcomes to explore associations in contemporary evolution between structural and functional abnormalities in CF lung disease. Methods: Spirometry-controlled chest-CTs using PRAGMA-CF for scoring extent of SLD, spirometry, and N2MBW were performed at two-year intervals in school-age children with CF. Results: Fifty-seven children aged 6–18 years were included. No significant progression in mean PRAGMA-CF scores was observed. Half of the children showed improvement in the proportion of bronchiectasis (%Bx). Lung Clearance Index (LCI) and the second moment ratio (M2) increased significantly and baseline values correlated significantly with SLD at follow-up (p ≤ 0.0002). The correlation between the change in M2 (∆M2) and the change in total SLD was R = 0.27 (p = 0.048). We found high negative predictive values (100%) for ∆M2<10% to exclude progression in SLD. For stable or improving values of LCI and M2, the predicted probability for progression in SLD was 16% and 14%, respectively (upper 95% confidence limit: 33%). Evolution in N2MBW and CT outcomes was discordant in half of the children. Conclusions: We found no progression in SLD over 2 years in school-age children with CF, in contrast to both LCI and M2, which along with discordant outcomes in half of the children underlines that N2MBW and CT assess different aspects of CF lung disease. However, stable outcomes from N2MBW were associated with stable structural lung disease.
Originalsprog | Engelsk |
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Tidsskrift | Journal of Cystic Fibrosis |
Vol/bind | 20 |
Udgave nummer | 4 |
Sider (fra-til) | 632-640 |
Antal sider | 9 |
ISSN | 1569-1993 |
DOI | |
Status | Udgivet - 2021 |
Bibliografisk note
Funding Information:
The authors wish to thank the patients and their parents for participating, members of the staff at the Pediatric Pulmonary Service of Copenhagen University Hospital for their assistance, and the technicians at the LungAnalysis Core Lab, Pediatric Pulmonology, Sophia Children's Hospital, Erasmus MC, Rotterdam, the Netherlands for PRAGMA-CF image analyses. We also thank the Department of Diagnostic Imaging and Chief Technician Flemming Dalsgaard for setting up the scanning protocol. The spirometric monitoring software was developed by bioengineer Bj?rn Neumann Jensen with inspiration from lung-function technician Sandra Lever at Erasmus University Medical Center in Rotterdam, the Netherlands.
Funding Information:
The authors thank the “Børnelungefonden” and the John & Birthe Meyer Foundation and the Toyota Foundation for financial support that provided equipment and salary for this study. We also thank the “Børnelungefonden” for supporting our CF Center, which has assured a part time salary for the first author.
Publisher Copyright:
© 2020 European Cystic Fibrosis Society
ID: 301139652