Cystic Fibrosis: The Mechanisms of Pathogenesis of an Inherited Lung Disorder.
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.
Clunes
Mark T
MT
For The Virtual Lung Project, Cystic Fibrosis/Pulmonary Research and Treatment Center, University of North Carolina at Chapel Hill, North Carolina 27599.
Boucher
Richard C
RC