Cystic Fibrosis: The Mechanisms of Pathogenesis of an Inherited Lung Disorder

Drug Discov Today Dis Mech. 2007;4(2):63-72. doi: 10.1016/j.ddmec.2007.09.001.


Cystic fibrosis patients exhibit lung disease consistent with a failure of innate airway defense mechanisms. The link between abnormal ion transport and disease initiation and progression is not fully understood, but airway mucus dehydration seems paramount in the initiation of CF lung disease. New therapies are currently in development that target the ion transport defects in CF with the intention of rehydrating airway surfaces.