Variability of lung clearance index in clinically stable cystic fibrosis lung disease in school age children

Abstract

LCI within-subject variability was low and comparable to FEV% pred. which strengthen the use of LCI to monitor lung disease progression in CF patients. An increase in LCI >17% compared to previous LCI-measurement in clinically stable CF patients may therefore indicate early lung disease progression.

Cystic fibrosis year in review 2018, part 1

“…year. 86 Measures done during periods of clinical stability showed low within-subject variability; an increase in LCI > 17% was the upper limit of normal variability in this cohort. These studies add to the understanding of variability between subjects, over time and with different assessment systems.…”

“…During the study, systematic difference between two commonly used commercial software systems were not seen, indicating that they could be used interchangeably for both research and for disease monitoring. A second study examining variability of LCI was also assessed in 25 children with CF who performed N 2 MBW and spirometry every 3 months for 1 year . Measures done during periods of clinical stability showed low within‐subject variability; an increase in LCI > 17% was the upper limit of normal variability in this cohort.…”

An 8 week open-label interventional multicenter study to evaluate the lung clearance index as endpoint for clinical trials in cystic fibrosis patients ≥8 years of age, chronically infected with Pseudomonas aeruginosa

“…There is increasing evidence on the clinical utility of LCI. In addition to LCI being used as a clinical endpoint [5,15], it has also been used to assess mucociliary clearance in young children [20] treated with CFTR-modulators [15] and to monitor disease progression [21], or both [22]. However, this study with inhaled tobramycin demonstrated that changes in LCI, FEV 1 and CFU at week 1, 4 and 8 were not statistically significant.…”

An 8 week open-label interventional multicenter study to evaluate the lung clearance index as endpoint for clinical trials in cystic fibrosis patients ≥6 years of age, chronically infected with Pseudomonas aeruginosa

“…There is increasing evidence on the clinical utility of LCI. In addition to LCI being used as a clinical endpoint [5,15], it has also been used to assess mucociliary clearance in young children [17] treated with CFTR-modulators [15] and to monitor disease progression [18], or both [19]. However, this study with inhaled tobramycin demonstrated that changes in LCI, FEV 1 and CFU at week 1, 4 and 8 were not statistically significant.…”