Magazine article Science News

Energy Duo Takes on CF's Chloride Defect

Magazine article Science News

Energy Duo Takes on CF's Chloride Defect

Article excerpt

Two naturally occurring substances appear to correct a cellular defect that may lie at the root of cystic fibrosis (CF). The new findings, although preliminary, hold out the hope of blocking the progressive lung damage wrought by CF and perhaps extending the lives of many children and young adults who suffer from this deadly inherited disease, the researchers suggest.

Cystic fibrosis strikes one in every 2,500 babies born in the United States. The disorders causes epithelial cells lining the lung's airways to absorb too much sodium and secrete too little chloride. This double defect leads to a buildup of thick, sticky mucus that clogs the breathing tubes. The mucus-layered lungs become vulnerable to frequent infections--a process that destroys healthy lung tissue, impairs breathing and usually causes death by age 30.

Last year, researchers at the University of North Carolina at Chapel Hill reported encouraging results in treating CF with an aerosol form of the diuretic drug amiloride (SN: 4/28/90, p.260). The study, which involved 14 patients, suggested that amiloride helps inhibit sodium absorption. Now, in the Aug. 22 NEW ENGLAND JOURNAL OF MEDICINE, the same group reports that two different compounds, both classified as triphosphate nucleotides, attack the other half of the CF equation: the chloride deficit. Together, the findings hint at a double-barreled approach to treatment.

"Ultimately our goal would be to give these drugs [amiloride and triphosphate nucleotides] in combination at a very early age to protect the airways," says Michael R. Knowles, who co-directed the new study. If all goes well, such treatment may prevent the devastating lung damage that leads to premature death for CF victims, he told SCIENCE NEWS.

In the new work, Knowles, Richard C. Boucher and their colleagues studied 12 men and women with CF and a control group of nine men and women in good health. …

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