Progress in understanding mucus abnormalities in cystic fibrosis airways

Abstract

Normal airways below the carina maintain an essentially sterile environment via a multi-pronged innate defence system that includes mucus clearance via mucociliary clearance and cough, multiple antimicrobials and cellular components including macrophages and neutrophils. In cystic fibrosis (CF), loss of CFTR function compromises these defences, and with present standard of care virtually all people with CF eventually develop mucus accumulation, plugging and chronic infections. This review focuses on how mucus is affected by CFTR loss.

Keywords: CF piglet; Cystic fibrosis; Ferret; HCO(3)(–); Hypertonic saline; Microscopic optical coherence tomography; Mucociliary clearance; Mucus; Submucosal glands; β-ENaC transgenic mouse.