Nitric oxide (NO) that is produced within the airways can be measured in the exhaled air. Concentrations of exhaled NO (FENO) are decreased in cystic fibrosis (CF) and, in cross sectional studies, have been shown to be even lower in patients with more advanced pulmonary disease. This may result from retention and metabolisation of NO within viscous airway secretions. Treatment with recombinant human DNase I (dornase alpha) modifies the rheological properties of airway secretions and thereby improves pulmonary function even in young and apparently healthy patients with CF. We studied FENO and pulmonary function in children with CF with little clinical evidence of lung morbidity in a two-year randomized double-blind placebo-controlled study with nebulized dornase alpha. Mean age at enrollment was 8 years (range 6 to 11 years), mean forced vital capacity (FVC) was 112% (range 86 to 133%), and mean forced expiratory volume in one second (FEV 1) was 109% (range 88 to 128%) of predicted values. In five of six (83%) of the dornase alpha treated patients, FENO changed in parallel to changes in pulmonary function tests while no such correlation was observed in any of the eight patients receiving placebo. This difference between treatment groups was statistically significant for both FVC (P = 0.026, Wilcoxon-test) and FEV1 (P = 0.042). These data suggest that FE NO may serve as a surrogate measure for evaluating the effectiveness of interventions that affect airway clearance in CF.
- Cystic fibrosis
- Dornase alpha
- Long term follow-up
- Nitric oxide
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Pulmonary and Respiratory Medicine