Medical perception of cystic fibrosis (CF) is that the body overproduces a thick mucus in the lungs, plugging up airways and digestive systems; but new research from Wake Forest Baptist Medical Center (Winston-Salem, North Carolina) shows that people with this genetic illness produce too little mucus rather than too much. Normal mucus is thin and slippery and acts as a lubricant. In people with CF, mucus is thick and sticky. It blocks tubes, ducts, and passages in the lungs and, also, in the pancreas and other digestive organs.
In this study, published in the American Journal of Respiratory Cell and Molecular Biology (July 2004), the North Carolina researchers collected sputum samples from 12 CF patients and 11 people without lung disease. The samples from those with CF showed significantly lower amounts of two proteins used to produce mucus (70% and 93%, respectively) than the samples taken from those with healthy lungs. Without those proteins, healthy mucus cannot form. The researchers determined that pus, produced during chronic infection, accounts for the thick stickiness of mucus in CF.
The researchers believe that infection, so common in CF, may decrease if mucus production increases. They are collaborating on an animal study to see if mucus can prevent bacterial infection. Although increasing mucus production would not cure CF, it could increase quality of life.
Bowman, Lee. Researchers gaining new insights into CF. The Herald-Sun (Durham, NC) 1 July 2004
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