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Cystic fibrosis: diagnosis and management

  • NICE guideline
  • Reference number: NG78
  • Published:  25 October 2017
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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?

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