Unified Airway-Cystic Fibrosis.

Do-Yeon Cho, Jessica W Grayson, Bradford A Woodworth

Journal: Otolaryngologic clinics of North America 2022;56(1):125-136

PMID: 36266104

Full Text on Elsevier Science View on PubMed

Abstract

Cystic fibrosis (CF) is a genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The CFTR channel is responsible for the transport of the anions (chloride and bicarbonate) across airway epithelia. Patients with CF have thick mucus, disrupted mucociliary transport, and chronic bacterial infections in the upper and lower airways. In this article, the pathophysiology of CFTR dysfunction and its impact on the united airway are reviewed as well as the treatment strategies for patients with chronic rhinosinusitis-related CF and acquired CFTR dysfunction.

Address: Department of Otolaryngology - Head & Neck Surgery, University of Alabama at Birmingham, 1155 Faculty Office Tower 510 20th Street South, Birmingham, AL 35233, USA; Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA; Department of Surgery, Division of Otolaryngology, Veteran Affairs Medical Center, Birmingham, AL, USA.; Department of Otolaryngology - Head & Neck Surgery, University of Alabama at Birmingham, 1155 Faculty Office Tower 510 20th Street South, Birmingham, AL 35233, USA.; Department of Otolaryngology - Head & Neck Surgery, University of Alabama at Birmingham, 1155 Faculty Office Tower 510 20th Street South, Birmingham, AL 35233, USA; Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA. Electronic address: [email protected].

DOI: 10.1016/j.otc.2022.09.009

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MeSH Terms: Humans, Cystic Fibrosis Transmembrane Conductance Regulator, Cystic Fibrosis, Mucociliary Clearance, Ion Transport, Chlorides