DIEPTE-study: DNase Inhalation in CF Exacerbations, Peripherally Targeted

Completed

• Conditions: Cystic Fibrosis (CF); Nutritional, Metabolic, Endocrine; Cystic Fibrosis

• Registration Number: ISRCTN50584238
• Lead Sponsor: Erasmus Medical Centre, Sophia Children?s Hospital (Netherlands)

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Study Start: 2006-02-14
• Last Update Posted: 13 January 2015

Recruitment & Eligibility

• Status: Completed
• Sex: All
• Target Recruitment: 40

Inclusion Criteria

1. Age between 6 and 18 years old
2. Diagnosis of CF confirmed by sweat-test and/or DNA analysis and/or electro physiology testing (nasal potential difference measurement)
3. Admission to hospital because of a pulmonary exacerbation requiring treatment with iv antibiotics.
The criteria for a pulmonary exacerbation will be based on the definition of exacerbation by Rosenfeld et al. and will include at least three of the following:
a. Decreased exercise tolerance
b. Increased cough
c. Increased sputum / chest congestion
d. School or work absenteeism
e. Decreased appetite
f. Increased adventitial sounds on lung examination
g. Decrease in FEV1 (% predicted)
4. Enrolment in the study between 1 to 5 days after admission for an exacerbation
5. Routine treatment with rhDNase once daily, started at least two weeks before enrolment in the study
6. Ability to perform lung function tests (assessed by trained lung function technician)
7. Lung function: forced vital capacity (FVC) >/= 30% predicted
8. Signed written informed consent

Exclusion Criteria

1. Inability to follow instructions of the investigator
2. Inability to inhale rhDNase
3. Concomitant medical conditions that effect inhaled treatment (e.g. cleft palate, severe malacia)
4. Pulmonary complications that might put the patient at risk to participate in the study
5. Deterioration primarily related to allergic bronchopulmonary aspergillosis (ABPA)

Study & Design

• Study Type: Interventional
• Study Design: Not specified