Variceal hemorrhage and adverse liver outcomes in patients with cystic fibrosis cirrhosis

Wen Ye, Michael R. Narkewicz, Daniel H. Leung, Wikrom Karnsakul, Karen F. Murray, Estella M. Alonso, John C. Magee, Sarah Jane Schwarzenberg, Alexander Weymann, Jean P. Molleston

Research output: Contribution to journalArticlepeer-review

33 Scopus citations


Objectives: Cirrhosis occurs in 5% to 10% of cystic fibrosis (CF) patients, often accompanied by portal hypertension. We analyzed 3 adverse liver outcomes, variceal bleeding (VB), liver transplant (LT), and liver-related death (LD), and risk factors for these in CF Foundation Patient Registry subjects with reported cirrhosis. Methods: We determined 10-year incidence rates for VB, LT, LD, and allcause mortality (ACM), and examined risk factors using competing risk models and Cox-proportional hazard regression. Results: From 2003 to 2012, 943 participants (41% females, mean age 18.1 years) had newly reported cirrhosis; 24.7% required insulin, 85% had previous pseudomonas. Seventy-three subjects had reported VB: 38 with first VB and new cirrhosis reported simultaneously and 35 with VB after cirrhosis report. Ten-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 (hazard ratio [HR] 1.10, 95% CI: 0.59, 2.08). CF-related diabetes (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, whereas 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.

Original languageEnglish (US)
Pages (from-to)122-127
Number of pages6
JournalJournal of pediatric gastroenterology and nutrition
Issue number1
StatePublished - Jan 1 2018

Bibliographical note

Publisher Copyright:
Copyright © 2017 ESPGHAN and NASPGHAN.


  • Cirrhosis
  • Cystic fibrosis
  • Liver
  • Portal HTN
  • Variceal bleeding


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