Mucolytic treatment of chronic rhinosinusitis in a murine model of primary ciliary dyskinesia.

ABSTRACT: Background: Genetic defects in motile cilia cause primary ciliary dyskinesia (PCD), a rare disease with no specific therapeutics. Individuals with PCD often have impaired fertility and laterality defects and universally suffer from upper and lower airway diseases. Chronic rhinosinusitis is a universal feature of PCD, and mucus accumulation and subsequent infections of the sinonasal cavity cause significant morbidity in individuals with PCD. Despite this, there are no approved treatments that specifically target mucus. Objective: The goals of this study were to determine whether computed tomography (CT) imaging could be used to quantify mucus accumulation and whether the use of a mucolytic agent to reduce disulfide cross-links present in mucins would improve the effectiveness

SUBMITTER: Yin W 

PROVIDER: S-EPMC10405821 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature