The current pathophysiological model of cystic fibrosis lungdisease assumes that defective expression, trafficking, or functionof the cystic fibrosis transmembrane regulator (CFTR) proteinleads to impaired epithelial chloride secretion and sodium hyperabsorption.This process, in turn, results in the depletion of airway surfaceliquid and abnormal mucociliary transport. Retention of mucusis thought to favor bacterial overgrowth, which then triggersa cycle of repeated or chronic infections associated with intenseneutrophilic airway inflammation.
Defective mucociliary transport itself can cause airway inflammation,as has been shown in mice that have an overexpression of thebeta subunit of the epithelial sodium . . . [Full Text of this Article]
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From the Hospital for Sick Children and the University of Toronto, Toronto.
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Hypertonic Saline for Cystic Fibrosis
Aziz I., Kastelik J. A., Zarogiannis S., Hatzoglou C., Gourgoulianis K., Kuver R., Lee S. P., Bye P. T.P., Elkins M. R., Donaldson S. H., Tarran R., Boucher R. C., Ratjen F.
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N Engl J Med 2006;
354:1848-1851, Apr 27, 2006.
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