Study	Reason for exclusion
Belli 1987	Examined the effects of taurine supplementation on the absorption of a fat meal.
Bittner 1991	Evaluated the effect of UDCA in CF and hepatopathy. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.
Colombo 2002	Not a RCT. Prospective observational study on incidence, risk factors and outcome of CFLD.
Colombo 1992	Evaluated the effect of UDCA in CF liver disease. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.
Colombo 1996	Long‐term follow‐up study evaluating UDCA for CFLD. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.
Darling 1985	Examined the effects of taurine supplementation on fat absorption in CF.
Fleet 2000	Case study, not a RCT.
Kapustina 2000	Evaluated the effect of UDCA on lipid metabolism in people with CF. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.
Lamireau 2006	Not a RCT.
Lepage 1997	Examined the effect of UDCA on the hepatic metabolism of essential fatty acid in children with CF. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.
Merli 1994	Examined the effect of a medium dose of UDCA with or without taurine supplementation on the nutritional status of people with CF. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.
Mieles 1991	Not a RCT.
Narkewicz 1994	Evaluated the effect of UDCA for CFLD on indirect tests of hepatic function. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.
Nightingale 2010	A retrospective non‐RCT.
Noble‐Jamieson 1994	Non‐randomised study.
O'Brien 1992	Assessed the effect of UDCA in CFLD. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.
Pozler 2003	Not a RCT.
Pukhalsky 2008	Evaluated anti‐inflammatory treatment on low frequency hepato‐biliary abnormalities in people with CF.
Roland 2011	Not a RCT, a cohort study.
Schuster 1977	Not a RCT, a cohort study.
Smith 1991	Examined the effects of taurine on faecal fatty acid and sterol excretion in CF.
Spray 1998	Prospective study of the role of UDCA on histological changes in children with CFLD. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.
Thomas 1995	Examined the effect of UDCA on malabsorption of vitamin E in CF. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.
Thompson 1987	Assessed protein metabolism in CF, response to malnutrition and taurine supplementation.
Van de Meeberg 1997	Long‐term follow up of low‐dose versus high‐dose UDCA in cholestasis related to CF. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

CF: cystic fibrosis CFLD: cystic fibrosis liver disease RCT; randomised controlled trial UDCA: ursodeoxycholic acid