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Nearly half a century after its first description, cystic fibrosis has been linked with a physiological defect.
The discovery, from two groups of American scientists, was reported at the Ninth International Cystic Fibrosis Congress, held in Brighton, England. Whether the physiological defect is the product of the abnormal gene that causes cystic fibrosis remains to be determined. Nonetheless, the discovery of the defect might lead to an effective treatment or even a cure for cystic fibrosis, desperately needed since cystic fibrosis is the most common lethal genetic disease among white persons.
Cystic fibrosis has traditionally been thought of as a disease of the exocrine glands, especially of the submucosal glands bordering the airways, of the sweat glands, and of the pancreas, because the major pathological characteristics, such as the formation of excessively thick mucus that is poorly cleared from the airways, the abnormal chemical composition of perspiration, and pancreatic enzyme
Arehart-Treichel J. Cystic fibrosis linked to chloride ions' inability to cross certain cells. JAMA. 1984;252(18):2519–2521. doi:10.1001/jama.1984.03350180001001