Abstract
Background The lung clearance index (LCI) measured by the multiple breath washout (MBW) test is sensitive to early lung disease in children with cystic fibrosis (CF). While LCI worsens during the preschool years in CF, there is limited evidence to clarify whether this continues during the early school age years, and whether the trajectory of disease progression as measured by LCI is modifiable.
Methods A cohort of children (healthy (HC) and CF) previously studied for 12 months as preschoolers were followed during school age (5–10 years). LCI was measured every 3 months for a period of 24 months using the Exhalyzer® D MBW nitrogen washout device. Linear mixed effects regression was used to model changes in LCI over time.
Results A total of 582 MBW measurements in 48 healthy subjects and 845 measurements in 64 CF subjects were available. The majority of children with CF had elevated LCI at the first preschool and first school age visits (57.8% (37/64)), whereas all but six had normal forced expiratory volume in 1 s (FEV1) values at the first school age visit. During school age years, the course of disease was stable (−0.02 units·year−1 (95% CI −0.14; 0.10). LCI measured during preschool years, as well as the rate of LCI change during this time period, were important determinants of LCI and FEV1, at school age.
Conclusion Preschool LCI was a major determinant of school age LCI; these findings further support that the preschool years are critical for early intervention strategies.
Footnotes
This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.
Conflict of interest: Dr. Stanojevic reports grants from CF Canada, grants from US CF Foundaiton, during the conduct of the study.
Conflict of interest: Dr. Davis has nothing to disclose.
Conflict of interest: Dr. Perrem has nothing to disclose.
Conflict of interest: Ms. Shaw has nothing to disclose.
Conflict of interest: Dr. Retsch-Bogart reports grants from Cystic Fibrosis Foundation, during the conduct of the study.
Conflict of interest: Dr. Davis has nothing to disclose.
Conflict of interest: R. Jensen has nothing to disclose.
Conflict of interest: Dr. Clem has nothing to disclose.
Conflict of interest: Ms. Isaac has nothing to disclose.
Conflict of interest: Ms. Guido has nothing to disclose.
Conflict of interest: Dr. Jara has nothing to disclose.
Conflict of interest: Dr. France has nothing to disclose.
Conflict of interest: Dr. McDonald has nothing to disclose.
Conflict of interest: Dr. Solomon reports grants from Vertex Pharmaceutical, grants from Mylene Pharmaceutical, other from Vertex Pharamaceutical, outside the submitted work.
Conflict of interest: Neil Sweezey has nothing to disclose.
Conflict of interest: Dr. Grasemann has nothing to disclose.
Conflict of interest: Dr. Waters has nothing to disclose.
Conflict of interest: Dr. Sanders has nothing to disclose.
Conflict of interest: Dr. Ratjen reports grants and personal fees from Vertex, Calithera, Proteostasis, TranslateBio, Genentech, Bayer and Boehringer Ingelheim, outside the submitted work.
- Received September 3, 2020.
- Accepted December 11, 2020.
- Copyright ©ERS 2021
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