The pathogenesis of cystic fibrosis (CF) airways infection is unknown. Two hypotheses,"
hypotonic [low salt]/defensin" and" isotonic volume transport/mucus clearance," attempt to …

The current pathophysiological model of cystic fibrosis lung disease assumes that defective
expression, trafficking, or function of the cystic fibrosis transmembrane regulator (CFTR) …

The pathogenesis of cystic fibrosis (CF) lung disease is reviewed, focusing on an overview
of the physiologic mechanisms that regulate mucus transport. A major emphasis is placed …

Although there has been impressive progress in the elucidation of the genetic and molecular
basis of cystic fibrosis (CF), the pathogenesis of CF lung disease remains obscure. The …

Cystic fibrosis (CF) is a life-limiting disease characterised by recurrent respiratory infections,
inflammation and lung damage. The volume and composition of the airway surface liquid …

The airways are lined with a film of liquid about 10 µm deep that is in two layers. Around the
cilia is the watery periciliary sol. Over this is a mucous blanket that traps inhaled particles …

Mammalian airways normally regulate the volume of a thin liquid layer, the periciliary liquid
(PCL), to facilitate the mucus clearance component of lung defense. Studies under standard …

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) protein, an epithelial chloride channel expressed in the airways, pancreas, testis …

Cystic fibrosis (CF) lung disease involves chronic bacterial infection of retained airway
secretions (mucus). Recent data suggest that CF lung disease pathogenesis reflects the …

There is controversy over whether abnormalities in the salt concentration or volume of
airway surface liquid (ASL) initiate cystic fibrosis (CF) airway disease. In vivo studies of CF …