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Previous Volume 335:179-188 July 18, 1996 Number 3 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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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 as a chloride channel activated by cyclic adenosine monophosphate (cAMP)³ and may stimulate other chloride channels⁴ and inhibit sodium absorption by epithelial sodium channels.⁵

View larger version (28K): [in this window] [in a new window]   Figure 1. Gene, Transcript, and Predicted Structure of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) . . . [Full Text of this Article]

 
Conventional Pulmonary Therapy for Cystic Fibrosis

Clearance of Airway Secretions

Bronchodilator Therapy

Reduction in the Viscoelasticity of Sputum

Antibiotic Therapy

Exacerbations of Pulmonary Infection

Maintenance Antibiotic Therapy

Quinolone Therapy

Inhaled Antibiotic Therapy

Gene Therapy

Pharmacologic Modulation of Ion Transport

Inhibition of Sodium Absorption

Activation of Alternative Chloride Channels

Antiinflammatory-Drug Therapy

Conclusions

Source Information

From the Department of Pediatrics, University of Washington School of Medicine, Seattle.

Address reprint requests to Dr. Ramsey at the Cystic Fibrosis Research Center, University of Washington School of Medicine, Department of Pediatrics, Box 359301, Seattle, WA 98195.

References

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