Mucociliary clearance in cystic fibrosis

Abstract

Abnormal mucociliary clearance (MCC) is one of the central hypotheses for the development of lung disease in patients with cystic fibrosis (CF). However, attempts to demonstrate this decrease of MCC in vivo have proved to be somewhat less definitive, with the evidence barely favoring impaired clearance. Any apparent disparities are most likely due to the variety of methodologies used by different laboratories to measure MCC. The limitations of the various methodologies are examined in this review, in an attempt to better facilitate comparison of results. A number of physical and pharmacological therapies have been developed to promote mucus clearance from the CF airway. A summary of the results of interventional studies utilizing the measurement of MCC as an outcome measure is presented.