Cystic Fibrosis :: Cystic fibrosis produce too little airway mucus

People with cystic fibrosis produce too little airway mucus, rather than too much as has long been thought, U.S. researchers reported.

Researchers at Wake Forest Baptist Medical Center collected sputum — matter ejected from the respiratory system through the mouth — from 12 patients with CF and 11 subjects with healthy lungs. They found CF patients had significantly less — 70 percent and 93 percent less — of two mucus-forming proteins.

“It has always been thought, but never proven, that CF causes the body to produce too much abnormally thick mucus that accumulates in the lungs and intestines,” wrote researcher Bruce Rubin. “However, we have now shown that these patients actually have very little mucus in their airways. This finding could change the way we think about CF treatment.”

CF is a genetic disease affecting about 100,000 people worldwide. It results in frequent respiratory infections, breathing difficulties and, eventually, permanent lung damage. Doctors have long believed the airways fill with mucus because people with CF have a chronic cough.

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