loading page

Changes in lung clearance index in children with cystic fibrosis -- guidance for clinical practice
  • +7
  • Bettina Frauchiger,
  • Kathryn Ramsey,
  • Jakob Usemann,
  • Elisabeth Kieninger,
  • Carmen Casaulta,
  • Daniel Sirtes,
  • Sophie Yammine,
  • Ben Spycher,
  • Alexander Moeller,
  • Philipp Latzin
Bettina Frauchiger
University Childrens Hospital Bern

Corresponding Author:[email protected]

Author Profile
Kathryn Ramsey
University of Bern
Author Profile
Jakob Usemann
University Children's Hospital Basel (UKBB), University of Basel, Basel, Switzerland
Author Profile
Elisabeth Kieninger
Division of Respiratory Medicine, Department of Paediatrics, University Hospital of Bern
Author Profile
Carmen Casaulta
University Hospital of Berne
Author Profile
Daniel Sirtes
Inselspital Universitätsspital Bern
Author Profile
Sophie Yammine
Division of Respiratory Medicine, Department of Paediatrics, University Hospital of Bern
Author Profile
Ben Spycher
University of Bern
Author Profile
Alexander Moeller
University Children's Hospital Zurich
Author Profile
Philipp Latzin
Inselspital Bern
Author Profile


Rationale The lung clearance index (LCI) is increasingly being used in the clinical surveillance of patients with cystic fibrosis (CF). However, there are limited data on long-term variability and clinically relevant changes in LCI during routine clinical surveillance. Objectives To evaluate long-term variability of LCI and propose a threshold for a clinically relevant change. Methods Children with CF aged 4-18 years performed LCI measurements every three months as part of routine clinical surveillance during 2011-2020 in two centers. The variability of LCI during periods of clinical stability was assessed using mixed-effects models and was used to identify thresholds for clinically relevant changes. Results Repeated LCI measurements of acceptable quality (N= 858) were available in 100 patients with CF. Variability of repeated LCI measurements over time expressed as coefficient of variation (CV%) was 7.4%. The upper limit of normal (ULN) for relative changes in LCI between visits was 19%. Conclusion We report the variability of LCI in children and adolescents with CF during routine clinical surveillance. According to our data, a change in LCI beyond 19% may be considered clinically relevant. These findings will help guide clinical decisions according to LCI changes.
14 Mar 2022Submitted to Pediatric Pulmonology
15 Mar 2022Submission Checks Completed
15 Mar 2022Assigned to Editor
16 Mar 2022Reviewer(s) Assigned
08 May 2022Review(s) Completed, Editorial Evaluation Pending
08 May 2022Editorial Decision: Revise Major
29 Aug 20221st Revision Received
01 Sep 2022Submission Checks Completed
01 Sep 2022Assigned to Editor
01 Sep 2022Reviewer(s) Assigned
25 Sep 2022Review(s) Completed, Editorial Evaluation Pending
28 Sep 2022Editorial Decision: Accept