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Updated: Nov 11, 2025
In vivo Evaluation of Mucociliary Clearance in Mice
Published on: December 18, 2020
Characterizing mucociliary clearance in young children with cystic fibrosis
Beth L Laube1, Kathryn A Carson2, Christopher M Evans3
1Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA. blaube@jhmi.edu.
Insights
Mucociliary clearance (MCC) is stable and reproducible in young children with cystic fibrosis (CF). Lower MCC values correlate with increased lung ventilation inhomogeneity, suggesting MCC could monitor early CF lung disease.
Area of Science:
- Pediatric Pulmonology
- Respiratory Physiology
- Cystic Fibrosis Research
Background:
- Cystic Fibrosis (CF) affects mucociliary clearance (MCC) in the lungs.
- Characterizing MCC in young children with CF is crucial for understanding disease progression.
Purpose of the Study:
- To characterize mucociliary clearance (MCC) in young children (5-7 years) diagnosed with cystic fibrosis (CF).
- To assess the reproducibility and stability of MCC measurements over time.
- To investigate the association between MCC and lung ventilation inhomogeneity.
Main Methods:
- Fourteen children (5-7 years) with CF underwent MCC measurements at baseline and 1-year follow-up.
- Lung clearance index (LCI), a measure of ventilation inhomogeneity, was also assessed.
- Statistical analyses, including Spearman correlation, were used to evaluate reproducibility and associations.
Main Results:
- MCC measurements demonstrated reproducibility over a 2-week period and stability over a 1-year period.
- Lower MCC values in children with CF were significantly associated with higher LCI scores, indicating increased ventilation inhomogeneity.
- No significant changes in MCC were observed over the 1-year study period.
Conclusions:
- MCC measurements are reproducible and stable in 5-7-year-old children with CF.
- Reduced MCC is linked to increased ventilation inhomogeneity, suggesting it reflects early lung pathology.
- MCC measurements show potential as a non-invasive tool for clinical trials and early diagnosis in pediatric CF.
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.
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