Palaniappan SK, Than NN, Thein AW, van Mourik I. Interventions for preventing and managing advanced liver disease in cystic fibrosis. Cochrane Database of Systematic Reviews 2020, Issue 3. Art. No.: CD012056. DOI: 10.1002/14651858.CD012056.pub3.

Randomised controlled trials (RCTs) and quasi-RCTs.

Children and adults of all ages and either gender, diagnosed with CFLD with cirrhosis or liver failure, PHT or variceal bleeding (or both).

1. Pharmacological interventions (e.g. non-selective betablockers) for any duration compared to placebo or no intervention 2. Endoscopic interventions (e.g. band ligation, sclerotherapy) compared to active control 3. TIPSS compared to active control 4. Surgical interventions (e.g. surgical PS shunt, liver transplantation) compared to active control

Primary outcomes 1. Change in variceal bleeding and portal pressure i) development of first bleeding episode (primary prevention) ii) re-bleeding following endoscopic treatment (secondary prevention) 2. Adverse effects i) of non-surgical interventions (e.g. aggravated congestive cardiac failure, respiratory distress, hypotension from non-selective beta-blockers, stenosis, occlusion, thrombosis, worsened encephalopathy after TIPSS, perforation of oesophagus, oesophageal ulceration, oesophageal stricture after sclerotherapy) ii) of surgical interventions (e.g. thrombosis, occlusion, onset or worsening of encephalopathy, infection, rejection) Secondary outcomes 1. Nutritional status 2. Respiratory outcomes.

A comprehensive search of the literature did not identify any published eligible randomised controlled trials.

In order to develop the best source of evidence, there is a need to undertake randomised controlled trials of interventions for preventing and managing advanced liver disease in adults and children with cystic fibrosis.