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Cystic fibrosis: an-ion transport issue?

Nature Medicine volume 17, pages 166–167 (2011)Cite this article

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Gerald Pier

The major clinical manifestation of cystic fibrosis—chronic and life-shortening lung infections—could be considered an iatrogenic disease caused by medical intervention. Cystic fibrosis was first identified clinically as a fatal disease of young children resulting from gastrointestinal and secretory organ dysfunction. Defective transepithelial ion transport has long been recognized to be a major contributor to cystic fibrosis symptoms, and the identification of the CFTR gene and protein allowed mechanistic links to be made between nonfunctional or missing CFTR and defects in transport of Cl− and HCO3−. People with cystic fibrosis now survive to 30–40 years of age, as they can be treated with therapies to correct gastrointestinal dysfunction, but they suffer from chronic lung infections for most of their life. This lung disease is also thought to be due to defective transepithelial ion transport of Cl− and HCO3−, as well as of Na+, whose absorption from the airway lumen has been suggested to be affected by CFTR.

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Cystic fibrosis: an-ion transport issue?. Nat Med 17, 166–167 (2011). https://doi.org/10.1038/nm0211-166

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