1. A Functional Anatomic Defect of the Cystic Fibrosis Airway

    A Functional Anatomic Defect of the Cystic Fibrosis Airway

    Rationale: The mechanisms underlying cystic fibrosis (CF) lung disease pathogenesis are unknown. Objective: To establish mechanisms linking anion transport with the functional microanatomy, we evaluated normal and CF piglet trachea, as well as adult swine trachea in the presence of selective anion inhibitors. Methods: We investigated airway functional microanatomy using micro-optical coherence tomography, a new imaging modality that concurrently quantifies multiple functional parameters of airway epithelium in a co-localized fashion. Main Results: Tracheal explants from wild type swine demonstrated a direct link between periciliary liquid (PCL) hydration and mucociliary transport (MCT) rates, a relationship frequently invoked but never experimentally confirmed ...

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