PT  - JOURNAL ARTICLE
AU  - Rayment, Jonathan H
AU  - Stanojevic, Sanja
AU  - Davis, Stephanie D
AU  - Retsch-Bogart, George
AU  - Ratjen, Felix
TI  - Lung clearance index to monitor treatment response in pulmonary exacerbations in preschool children with cystic fibrosis
AID  - 10.1136/thoraxjnl-2017-210979
DP  - 2018 May 01
TA  - Thorax
PG  - 451--458
VI  - 73
IP  - 5
4099  - http://thorax.bmj.com/content/73/5/451.short
4100  - http://thorax.bmj.com/content/73/5/451.full
SO  - Thorax2018 May 01; 73
AB  - Background Antibiotic treatment for pulmonary symptoms in preschool children with cystic fibrosis (CF) varies among clinicians. The lung clearance index (LCI) is sensitive to early CF lung disease, but its utility to monitor pulmonary exacerbations in young children has not been assessed.Objective We aim to (1) understand how LCI changes during lower respiratory tract symptoms relative to a recent clinically stable measurement, (2) determine whether LCI can identify antibiotic treatment response and (3) compare LCI changes to changes in spirometric indices.Methods LCI and spirometry were measured at quarterly clinic visits over a 12-month period in preschool children with CF. Symptomatic visits were identified and classified as treated or untreated. Treatment response was estimated using propensity score matching methods.Results 104 symptomatic visits were identified in 78 participants. LCI increased from baseline in both treated (mean relative change +23.8% (95% CI 16.2 to 31.4)) and untreated symptomatic visits (mean relative change +11.2% (95% CI 2.4 to 19.9)). A significant antibiotic treatment effect was observed when LCI was used as the outcome measure (average treatment effect −15.5% (95% CI −25.4 to −5.6)) but not for z-score FEV1.Conclusion LCI significantly deteriorated with pulmonary symptoms relative to baseline and improved with antibiotic treatment. These data suggest that LCI may have a role in the routine clinical care of preschool children with CF.