Hypertonic saline in treatment of pulmonary disease in cystic fibrosis.
The pathogenesis of lung disease in cystic fibrosis is characterised by decreased airway surface liquid volume and subsequent failure of normal mucociliary clearance. Mucus within the cystic fibrosis airways is enriched in negatively charged matrices composed of DNA released from colonizing bacteria or inflammatory cells, as well as F-actin and elevated concentrations of anionic glycosaminoglycans. Therapies acting against airway mucus in cystic fibrosis include aerosolized hypertonic saline. It has been shown that hypertonic saline possesses mucolytic properties and aids mucociliary clearance by restoring the liquid layer lining the airways. However, recent clinical and bench-top studies are beginning to broaden our view on the beneficial effects of hypertonic saline, which now extend to include anti-infective as well as anti-inflammatory properties. This review aims to discuss the described therapeutic benefits of hypertonic saline and specifically to identify novel models of hypertonic saline action independent of airway hydration.
Respiratory Research Division, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin 9, Ireland. emerreeves@rcsi.ie
Emer P
EP
Reeves
Kevin
K
Molloy
Kerstin
K
Pohl
Noel G
NG
McElvaney
D000293:Adolescent; D000818:Animals; D000890:Anti-Infective Agents / Q000494:pharmacology; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications* / Q000188:drug therapy*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D004548:Elasticity; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007239:Infections; D007249:Inflammation; D008168:Lung / Q000502:physiology; D008171:Lung Diseases / Q000150:complications* / Q000188:drug therapy*; D009079:Mucociliary Clearance / Q000187:drug effects; D009093:Mucus / Q000378:metabolism; D009154:Mutation; D012137:Respiratory System; D012462:Saline Solution, Hypertonic / Q000494:pharmacology*
D000890:Anti-Infective Agents; C505032:CFTR protein, human; D012462:Saline Solution, Hypertonic; D019005:Cystic Fibrosis Transmembrane Conductance Regulator