Cystic fibrosis: diagnosis and management

Research recommendation(s) from an individual piece of guidance

Guidance:

Cystic fibrosis: diagnosis and management

Date issued:

25 October 2017

Research recommendations coming out of this guidance

• Liver disease:- Should all children with meconium ileus receive ursodeoxycholic acid from diagnosis?

• Airway clearance techniques:- How effective are daily airway clearance techniques in maintaining lung function in infants and children with cystic fibrosis?

• Monitoring pulmonary disease:- Is lung clearance index a useful and cost-effective tool for the routine assessment and monitoring of changes in pulmonary status in people with cystic fibrosis?

• Psychological assessment:- What is the most effective measure of psychological functioning to use as a test for thresholds of concern in people with cystic fibrosis?

• Monitoring for cystic-fibrosis-related diabetes:- What is the most effective strategy to detect diabetes in people with cystic fibrosis?

• Mucoactive agents:- What is the most clinically and cost-effective dose of rhDNase (dornase alfa; recombinant human deoxyribonuclease) for people with cystic fibrosis?