Abstract
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 language | English (US) |
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Pages (from-to) | 122-127 |
Number of pages | 6 |
Journal | Journal of pediatric gastroenterology and nutrition |
Volume | 66 |
Issue number | 1 |
DOIs | |
State | Published - Jan 1 2018 |
Funding
This work was financially supported by the CF Foundation grant to Dr Narkewicz, NARKEW07A0 and NIDDK grant DK062456 to Dr Molleston.
Keywords
- Cirrhosis
- Cystic fibrosis
- Liver
- Portal HTN
- Variceal bleeding
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Gastroenterology