Variceal Hemorrhage and Adverse Liver Outcomes in Patients With Cystic Fibrosis Cirrhosis
dc.contributor.author | Ye, Wen | |
dc.contributor.author | Leung, Daniel H. | |
dc.contributor.author | Karnsakul, Wikrom | |
dc.contributor.author | Murray, Karen F. | |
dc.contributor.author | Alonso, Estella M. | |
dc.contributor.author | Magee, John C. | |
dc.contributor.author | Schwarzenberg, Sarah Jane | |
dc.contributor.author | Weymann, Alexander | |
dc.contributor.author | Molleston, Jean P. | |
dc.contributor.author | Narkewicz, Mark R. | |
dc.contributor.department | Pediatrics, School of Medicine | en_US |
dc.date.accessioned | 2019-07-02T17:04:47Z | |
dc.date.available | 2019-07-02T17:04:47Z | |
dc.date.issued | 2018-01 | |
dc.description.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 all-cause 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. | en_US |
dc.eprint.version | Author's manuscript | en_US |
dc.identifier.citation | Ye, W., Narkewicz, M. R., Leung, D. H., Karnsakul, W., Murray, K. F., Alonso, E. M., … CFLDnet research group (2018). Variceal Hemorrhage and Adverse Liver Outcomes in Patients With Cystic Fibrosis Cirrhosis. Journal of pediatric gastroenterology and nutrition, 66(1), 122–127. doi:10.1097/MPG.0000000000001728 | en_US |
dc.identifier.uri | https://hdl.handle.net/1805/19808 | |
dc.language.iso | en_US | en_US |
dc.publisher | Wolters Kluwer | en_US |
dc.relation.isversionof | 10.1097/MPG.0000000000001728 | en_US |
dc.relation.journal | Journal of Pediatric Gastroenterology and Nutrition | en_US |
dc.rights | Publisher Policy | en_US |
dc.source | PMC | en_US |
dc.subject | Variceal bleeding | en_US |
dc.subject | portal HTN | en_US |
dc.subject | Cirrhosis | en_US |
dc.subject | Liver | en_US |
dc.subject | Cystic fibrosis | en_US |
dc.title | Variceal Hemorrhage and Adverse Liver Outcomes in Patients With Cystic Fibrosis Cirrhosis | en_US |
dc.type | Article | en_US |