Variability of clinically measured lung clearance index in children with cystic fibrosis.

Frauchiger, Bettina S; Ramsey, Kathryn A; Usemann, Jakob; Kieninger, Elisabeth; Casaulta, Carmen; Sirtes, Daniel; Yammine, Sophie; Spycher, Ben; Moeller, Alexander; Latzin, Philipp (2023). Variability of clinically measured lung clearance index in children with cystic fibrosis. Pediatric pulmonology, 58(1), pp. 197-205. Wiley-Blackwell 10.1002/ppul.26180

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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 physiologically 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, for 74 patients 399 visits at clinical stability were available. 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 physiologically relevant. These findings will help guide clinical decisions according to LCI changes. This article is protected by copyright. All rights reserved.

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