Management of Pulmonary Disease in Patients with Cystic Fibrosis

Abstract excerpt

Cystic fibrosis is the most common life-shortening autosomal recessive disorder in the white population, affecting approximately 30,000 persons in the United States.1 It is caused by mutations in a single gene on the long arm of chromosome 7 that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) (Figure 1).2 CFTR has multiple functions involving fluid balance across epithelial cells. It acts...

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