Is Cystic Fibrosis Lung Disease Caused by Abnormal Ion Composition or Abnormal Volume?

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Is Cystic Fibrosis Lung Disease Caused by Abnormal Ion Composition or Abnormal Volume?

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© 2001 The Rockefeller University Press The most common lethal genetic disease in the Caucasian population is cystic fibrosis (CF). In 1970, the median life expectancy was 8 yr; today, with the concerted effort of scientists and clinicians, the median survival age has risen to 30 yr. About 1 in 23 people carry a single defective copy of the CF gene in the United States population and 40,000 people in the US have cystic fibrosis. The disease is characterized by a generalized exocrine dysfunction, and lung disease is the leading cause of death in CF patients (Davis et al. 1996). The lungs develop reoccurring (requiring hospitalization) and persistent infections often with Pseudomonas aeruginosa or Burkholderia cepacia, both of which are fairly innocuous in the normal population. The genesis of lung infection is not well understood, and has led to controversies in the CF field. The two prevalent theories of CF lung infection are termed the “high salt hypothesis” and the “low volume hypoth

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