Abstract
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 physiologically relevant changes in LCI during routine clinical surveillance. Objectives: To evaluate the long-term variability of LCI and propose a threshold for a physiologically relevant change. Methods: In children aged 4–18 years with CF, LCI was measured every 3 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 physiologically 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. The variability of repeated LCI measurements over time expressed as the 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.
Original language | English |
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Pages (from-to) | 197-205 |
Number of pages | 9 |
Journal | Pediatric Pulmonology |
Volume | 58 |
Issue number | 1 |
DOIs | |
Publication status | Published - Jan 2023 |