Aerosolized Amiloride as Treatment of Cystic Fibrosis Lung Disease: A Pilot Study
Excessive Na+ absorption1,2 and defective regulation of apical membrane Cl- secretion3–6 likely leads to dehydration and abnormal rheology of CF airway secretions7. These abnormalities contribute to poor clearance of airway secretions, and chronic bacterial infection in CF. Because amiloride inhibits the absorption of Na+ (and liquid) across airway mucosa1,8,9 when applied to the apical surface of CF airway epithelia, we hypo-thesized that inhalation of amiloride may improve rheology and clearance of airway secretions9,10 This hypothesis was tested in a doubleblind crossover study to explore the safety and efficacy of long-term amiloride aerosol in the treatment of cystic fibrosis lung disease11.
KeywordsCystic Fibrosis Force Vital Capacity Cystic Fibrosis Patient Total Lung Capacity Parenteral Antibiotic
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