Variceal Hemorrhage and Adverse Liver Outcomes in Patients With Cystic Fibrosis Cirrhosis.

Objectives: Cirrhosis occurs in 5-10% of CF (cystic fibrosis) patients, often accompanied by portal hypertension. We analyzed three adverse liver outcomes, variceal bleeding (VB), liver transplant (LT), and liver death (LD), and risk factors for these in CF Foundation Patient Registry (CFFPR) subjects with reported cirrhosis. Methods: We determined 10-year incidence rates for VB, LT, LD, and all-cause mortality (ACM), and examined risk factors using competing risk models and Cox-proportional hazard regression. Results: From 2003-2012, 943 participants (41% female, mean age 18.1 years) had newly reported cirrhosis; 24.7% required insulin, 85% had prior pseudomonas. Seventy-three subjects had reported VB; 38 with first VB and new cirrhosis reported simultaneously and 35 with VB after cirrhosis report. 10-year cumulative VB, LT, and LD rates were 6.6% (95% Confidence Interval (CI): 4.0, 9.1%), 9.9% (95% CI: 6.6%, 13.2%), and 6.9% (95% CI: 4.0%, 9.8%), respectively, with an ACM of 39.2% (95% CI: 30.8, 36.6%). ACM was not increased in subjects with VB compared to those without (HR 1.10, 95% CI: 0.59, 2.08). CF related diabetes (CFRD (hazard ratio (HR): 3.141, 95% CI:1.56, 6.34) and VB (HR: 4.837, 95% CI: 2.33, 10.0) were associated with higher LT risk while only worse lung function was associated with increased LD in multivariate analysis. Death rate among subjects with VB was 24% with LT and 20.4% with native liver. Conclusions: VB is an uncommon complication of CF cirrhosis and can herald the diagnosis, but does not affect ACM. Adverse liver outcomes and ACM are frequent by 10 years after cirrhosis report. (C) 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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