Forskning
Udskriv Udskriv
Switch language
Region Hovedstaden - en del af Københavns Universitetshospital
E-pub ahead of print

Prospective longitudinal association between repeated multiple breath washout measurements and computed tomography scores in children with cystic fibrosis

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

DOI

  1. In vivo demonstration of Pseudomonas aeruginosa biofilms as independent pharmacological microcompartments

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  2. Lung clearance index-triggered intervention in children with cystic fibrosis - A randomised pilot study

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  3. Pseudomonas aeruginosa antibody response in cystic fibrosis decreases rapidly following lung transplantation

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  4. The impact of chest computed tomography and chest radiography on clinical management of cystic fibrosis lung disease

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  1. National, clinical cohort study of late effects among survivors of acute lymphoblastic leukaemia: the ALL-STAR study protocol

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  2. Bacterial persisters in long-term infection: Emergence and fitness in a complex host environment

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  3. Patients with cystic fibrosis and advanced lung disease benefit from lumacaftor/ivacaftor treatment

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  4. Extended Screening for Cystic Fibrosis-related Liver Disease Including Elastography in Children and Adolescents

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  5. Lung clearance index-triggered intervention in children with cystic fibrosis - A randomised pilot study

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

Vis graf over relationer

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 (N 2MBW) 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 N 2MBW 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 N 2MBW 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 N 2MBW and CT assess different aspects of CF lung disease. However, stable outcomes from N 2MBW were associated with stable structural lung disease.

OriginalsprogEngelsk
Artikelnummer2121
TidsskriftJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Sider (fra-til)2121
ISSN1569-1993
DOI
StatusE-pub ahead of print - 4 okt. 2021

Bibliografisk note

Copyright © 2020 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

ID: 62064023