Evidence for airway surface dehydration as the initiating event in CF airway disease
- 3 January 2007
- journal article
- review article
- Published by Wiley in Journal of Internal Medicine
- Vol. 261 (1), 5-16
- https://doi.org/10.1111/j.1365-2796.2006.01744.x
Abstract
Cystic fibrosis (CF) lung disease reflects persistent bacterial infection of airway lumens. Several hypotheses have been advanced to link mutations in the CFTR gene to the failure of the CF lung to defend itself against bacterial infection. Amongst the most productive hypotheses at present is the ''low airway surface liquid (ASL) volume'' or ''dehydration'' hypothesis. This hypothesis predicts that airway surface dehydration produces the mucus adhesion, inflammation, and bacterial biofilm formation characteristic of CF. Clinical trials of inhaled hypertonic saline have demonstrated therapeutic benefit of manoeuvres designed to rehydrate CF airway surfaces.Keywords
This publication has 58 references indexed in Scilit:
- Design, Synthesis, and Structure−Activity Relationships of Novel 2-Substituted Pyrazinoylguanidine Epithelial Sodium Channel Blockers: Drugs for Cystic Fibrosis and Chronic BronchitisJournal of Medicinal Chemistry, 2006
- Genetic adaptation by Pseudomonas aeruginosa to the airways of cystic fibrosis patientsProceedings of the National Academy of Sciences of the United States of America, 2006
- Use of Pseudomonas biofilm susceptibilities to assign simulated antibiotic regimens for cystic fibrosis airway infectionJournal of Antimicrobial Chemotherapy, 2005
- ASSEMBLY OF FUNCTIONAL CFTR CHLORIDE CHANNELSAnnual Review of Physiology, 2005
- Method for detection of respiratory viruses in the sputa of patients with cystic fibrosisEuropean Journal of Clinical Microbiology & Infectious Diseases, 2004
- Pseudomonas aeruginosa Anaerobic Respiration in Biofilms: Relationships to Cystic Fibrosis PathogenesisDevelopmental Cell, 2002
- Phosphatidylinositol 4,5-Bisphosphate (PIP2) Stimulates Epithelial Sodium Channel Activity in A6 CellsOnline Journal of Public Health Informatics, 2002
- Hydrocephalus, Situs Inversus, Chronic Sinusitis, and Male Infertility in DNA Polymerase λ-Deficient Mice: Possible Implication for the Pathogenesis of Immotile Cilia SyndromeMolecular and Cellular Biology, 2002
- Anionic Phospholipids Regulate Native and Expressed Epithelial Sodium Channel (ENaC)Online Journal of Public Health Informatics, 2002
- Cystic Fibrosis Airway Epithelia Fail to Kill Bacteria Because of Abnormal Airway Surface FluidCell, 1996