Characterizing mucociliary clearance in young children with cystic fibrosis

Beth L. Laube, Kathryn A. Carson, Christopher M. Evans, Melis A. Aksit, Michael Collaco, Vanessa L. Richardson, Gail Sharpless, Pamela Leslie Zeitlin, Garry R. Cutting, Peter Mogayzel

Research output: Contribution to journalArticlepeer-review

Abstract

Background: This research characterized mucociliary clearance (MCC) in young children with cystic fibrosis (CF). Methods: Fourteen children (5–7 years old) with CF underwent: two baseline MCC measurements (Visits 1 and 2); one MCC measurement approximately 1 year later (Visit 3); and measurements of lung clearance index (LCI), a measure of ventilation inhomogeneity. Results: Median (range) percent MCC through 60 min (MCC60) was similar on Visits 1 and 2 with 11.0 (0.9–33.7) and 12.8 (2.7–26.8), respectively (p = 0.95), and reproducible (Spearman Rho = 0.69; p = 0.007). Mucociliary clearance did not change significantly over 1 year with median percent MCC60 on Visit 3 [12.8 (3.7–17.6)] similar to Visit 2 (p = 0.58). Lower percent MCC60 on Visit 3 was significantly associated with higher LCI scores on Visit 3 (N = 14; Spearman Rho = −0.56; p = 0.04). Conclusions: Tests of MCC were reproducible and reliable over a 2-week period and stable over a 1-year period in 5–7-year-old children with CF. Lower MCC values were associated with increased ventilation inhomogeneity. These results suggest that measurements of MCC could be used in short-term clinical trials of interventions designed to modulate MCC and as a new, non-invasive test to evaluate early lung pathology in children with CF. Impact: This is the first study to characterize mucociliary clearance (MCC) in children with cystic fibrosis (CF) who were 5–7 years old.Measurements of mucociliary clearance were reproducible and reliable over a 2-week period and stable over a 1-year period.Variability in MCC between children was associated with differences in ventilation homogeneity, such that children with lower MCC values had increased ventilation inhomogeneity.These results suggest that measurements of MCC could be used in short-term clinical trials of interventions designed to modulate MCC and as a new, non-invasive test to evaluate early lung pathology in children with CF.

Original languageEnglish (US)
JournalPediatric research
DOIs
StateAccepted/In press - 2021

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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