Determinants of lung disease progression measured by lung clearance index in children with cystic fibrosis

The lung clearance index (LCI) measured by the multiple breath washout (MBW) test is sensitive to early disease in children with cystic fibrosis. While LCI worsens during preschool years fibrosis, there limited evidence clarify whether this continues school age years, and trajectory of progression as modifiable. A cohort (healthy fibrosis) previously studied for 12 months preschoolers were followed (5–10 years). was every 3 a period 24 using Exhalyzer D MBW nitrogen device. Linear mixed effects regression used model changes over time. total 582 measurements 48 healthy subjects 845 64 fibrosis available. majority had elevated at first visits (57.8% (37 out 64)), whereas all but six normal forced expiratory volume 1 s (FEV ) values visit. During course stable (?0.02 units·year ?1 (95% CI ?0.14–0.10). well rate change time period, important determinants FEV 1, age. Preschool major determinant LCI; these findings further support that are critical intervention strategies.