One of the fundamental pathophysiologic defects in CF is abnormal mucociliary clearance (MCC), a basic host defense mechanism. This deficiency in the CF lung leads to mucostasis, chronic bacterial infection and progressive inflammation, ultimately resulting in diminished lung function and shortened lifespan in patients with CF. As defective MCC is common among all patients with CF, this pathway represents a target for treatment that would be beneficial for all patients, regardless of genotype. Moreover, accumulating data suggests that mucus obstruction alone results in inflammation, regardless of the presence of bacteria. This symposium session will provide an overview of the mechanisms involved in mucociliary clearance and the therapeutic targets associated with them.
Distinguish the pathophysiologic phenotypes of CF mucus obstruction, and compare and contrast with other muco-obstructive disorders.
Discuss important therapeutic approaches that address abnormal mucociliary clearance in CF.
Examine novel targets for therapy to slow the progression of CF lung disease.
Note: Speaker David Stoltz's presentation was not recorded at his request.