CFDB - Cystic Fibrosis DataBase

primary studies published RCT

Multicentric trial of rhDNase in patients with cystic fibrosis and severe pulmonary dysfunction.

Study design (if review, criteria of inclusion for studies)

randomized, double-blind, placebo- controlled, multicenter study. After the completion of the randomized study, there was an open-label phase

Participants

The first three aerosolisations were performed in the hospital, after bronchial drainage. 70 patients with cystic fibrosis and severe impairment of pulmonary function, aged 5 to 48 years

Interventions

RCT: aerosolised rhDNase 2.5 mg twice daily for 2 weeks (three aerosolisations were performed in the hospital, after bronchial drainage). Open- label phase for 6 months: all patients received rhDNase.

Outcome measures

Respiratory parameters

Main results

Respiratory parameters improved in both groups during the study period. As compared to baseline values, FVC improved by 13.7% and 12.7%, forced expiratory volume in one second (FEV1) by 6% and 7%, respectively in the placebo and rh DNase groups. No significant difference was detected between the two groups. In the open- label phase FEV1 and FVC further improved in all patients.

Authors' conclusions

We failed to demonstrate for severely ill patients with cystic fibrosis a significant advantage for aerosolised rhDNase compared to placebo for two weeks. The further improvement observed in the open-label phase suggests that more prolonged treatment might be necessary to detect a statistically significant benefit. No significant side effects of the treatment were observed.

Keywords: Adolescent; Adult; Child; Deoxyribonuclease; Airway clearance drugs -expectorants- mucolytic- mucociliary-; Inhalation OR nebulised; pharmacological_intervention; placebo; Respiratory System Agents; Respiratory Tract Diseases; Dornase alpha; Pulmozyme;