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Updated: Dec 21, 2025
In vivo Evaluation of Mucociliary Clearance in Mice
Published on: December 18, 2020
Changes in mucociliary clearance over time in children with cystic fibrosis
Beth L Laube1, Kathryn A Carson2, Christopher M Evans3
1Department of Pediatrics, Johns Hopkins Medical Institutions, Baltimore, Maryland.
Insights
Mucociliary clearance (MCC) significantly declines in children with cystic fibrosis (CF), increasing Pseudomonas aeruginosa (PA) infection risk and lung damage. Mucus composition impacts MCC decline, highlighting early monitoring importance.
Area of Science:
- Pulmonary Medicine
- Pediatric Respiratory Research
- Cystic Fibrosis Research
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting mucus clearance.
- Mucociliary clearance (MCC) is crucial for airway health in CF patients.
- Understanding MCC changes is vital for managing CF lung disease progression.
Purpose of the Study:
- To quantify longitudinal changes in MCC in children with CF.
- To investigate the relationship between MCC, Pseudomonas aeruginosa (PA) infections, and CF lung disease evolution.
- To explore the role of mucus composition in determining MCC.
Main Methods:
- Longitudinal study involving repeat MCC measurements over 3-10 years.
- Assessed ventilation inhomogeneity (LCI) and lung structure (HRCT).
- Quantified PA+ cultures and mucus dry weight in CF patients and controls.
Main Results:
- MCC declined by 30% over a median of 4.6 years.
- Lower MCC correlated with increased PA infections and higher LCI/HRCT scores.
- CF patients had higher mucus dry weight, associated with lower MCC.
Conclusions:
- Significant MCC decline occurs in pediatric CF, linked to PA infection and mucus properties.
- Early reductions in MCC predict future ventilation inhomogeneity and lung damage.
- Monitoring MCC and mucus composition is critical for CF management.
Objectives:
(a) To quantify changes in mucociliary clearance (MCC) over time in children with cystic fibrosis (CF) and the relationship between MCC and rate of infection with Pseudomonas aeruginosa (PA); (b) to determine the impact of MCC on the evolution of CF lung disease; and (c) to explore the role of mucus composition as a determinant of MCC.
Methods:
Children with CF, who had previously undergone an MCC measurement (visit 1), underwent the following tests 3 to 10 years later: (a) second MCC measurement (visit 2); (b) multiple breath washout to assess ventilation inhomogeneity, expressed as lung clearance index (LCI); (c) high resolution computed tomography lung scan (HRCT); and (d) induced sputum test. Number of PA + cultures/year between visits was documented and mucus dry weight was quantified in the children and adult controls.
Results:
Nineteen children completed both visits. Median time between visits was 4.6 years. Clearance declined 30% between visits. Lower MCC on visit 2 was associated with more PA+ cultures/year between visits. Lower MCC values on visit 1 were associated with higher LCI values and higher HRCT scores on visit 2. Mucus dry weight was significantly higher in children with CF compared with controls. Higher dry weights were associated with lower MCC.
Conclusions:
Mucociliary clearance declines significantly over time in children with CF. The decline is associated with PA infection rate and is affected by mucus composition. Children with early slowing of MCC appear to be at risk for developing ventilation inhomogeneity and parenchymal lung damage when they are older.
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