Occurrence, outcomes and predictors of portal hypertension in cystic fibrosis: A longitudinal prospective birth cohort study

The impact of liver disease on mortality in cystic fibrosis–A systematic review

“…[36] The studies were based on cohorts in United Kingdom (3), [ 29 , 32 , 36 ] United States (2) [ 26 , 35 ] France (3), [ 16 , 27 , 33 ] Australia (2) [ 28 , 31 ], Ireland (1), [15] Netherlands (1), [17] Italy (1) [14] and 1 combination of cohorts from both Italy and Australia. [34] Over 50% of the studies were single center studies, with 2 from more than one CF center, [ 32 , 34 ] 3 were based on CF registry data [ 17 , 29 , 35 ] and 1 was a national study [15] . As described in the Methods ( Section 2.4 ) studies were divided into groups based on their classification of liver disease with 6/14 (42.8%) studies were classified as Group 1 [ 14 , 16 , 28 , 29 , 31 , 36 ], while 8 (57.1%) were classified as Group 2 [ 15 , 17 , 26 , 27 , 32-35 ].…”

“…In this study, in addition to examining all-cause mortality (combined mortality and transplant) we also examined specific mortality rates for hepatic, pulmonary, other causes of death including the number of transplants (liver, liver and lung, lung and/or other). Apart from in the study of Cipolli et al [34] pulmonary complications accounted for mortality in more than 50% of those with CFLD who died. It is difficult to draw any conclusions from Table 3 as to the role of transplantation (liver, lung) in CFLD management.…”

“…The excess mortality (risk difference) suggests the possibility of an increasing disparity in outcome. In 1991 Scott Jupp [32] reported that the excess risk of mortality was 10.1% (95%CI 1.7-18.4), while in 2020 Cipolli et al reported an excess mortality rate of 18.78% (95%CI 7.9-30.56) [34] ( Table 2 ) for those with clinically significant liver disease. This excess mortality in those with CFLD is also supported by evidence from other studies included in this SR [ 15 , 16 , 29 ].…”

“…A prospective study that followed children diagnosed following newborn screening provides evidence that PWCF with CFLD continue to have much higher all-cause mortality rate (mortality/transplant) compared to those with no liver disease (excess mortality 18.8% 95%CI 6.9-30.6). [34] Using data based on the Dutch CF Registry which included children over 8 years of age and adults, Pals et al demonstrated that the risk of death was greatest in those under 25 years of age. [17] Other studies in this SR originating from paediatric centres [ 28 , 32 ] CFLD contributes to shortened life expectancy in children and adolescents with CF.…”

“…We examined changes in survival since the 1980s. Table 2 shows that for PWCF with no liver disease the crude mortality rate has declined from 16.4% [32] in the 1980's to 4.8% [34] in the second decade of the 21 st century. While there has also been an improvement in mortality rates for those with CFLD since the 1980's, clinically important differences in excess risk remain.…”