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 journalArticle

8 Citations (Scopus)

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 languageEnglish (US)
Pages (from-to)122-127
Number of pages6
JournalJournal of pediatric gastroenterology and nutrition
Volume66
Issue number1
DOIs
StatePublished - Jan 1 2018

Fingerprint

Cystic Fibrosis
Fibrosis
Hemorrhage
Liver
Confidence Intervals
Mortality
Transplants
Portal Hypertension
Pseudomonas
Proportional Hazards Models
Registries
Multivariate Analysis
Insulin
Lung

Keywords

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

PubMed: MeSH publication types

  • Journal Article
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

Cite this

Ye, W., Narkewicz, M. R., Leung, D. H., Karnsakul, W., Murray, K. F., Alonso, E. M., ... Molleston, J. P. (2018). Variceal hemorrhage and adverse liver outcomes in patients with cystic fibrosis cirrhosis. Journal of pediatric gastroenterology and nutrition, 66(1), 122-127. https://doi.org/10.1097/MPG.0000000000001728

Variceal hemorrhage and adverse liver outcomes in patients with cystic fibrosis cirrhosis. / Ye, Wen; Narkewicz, Michael R.; Leung, Daniel H.; Karnsakul, Wikrom; Murray, Karen F.; Alonso, Estella M.; Magee, John C.; Schwarzenberg, Sarah Jane; Weymann, Alexander; Molleston, Jean P.

In: Journal of pediatric gastroenterology and nutrition, Vol. 66, No. 1, 01.01.2018, p. 122-127.

Research output: Contribution to journalArticle

Ye, W, Narkewicz, MR, Leung, DH, Karnsakul, W, Murray, KF, Alonso, EM, Magee, JC, Schwarzenberg, SJ, Weymann, A & Molleston, JP 2018, 'Variceal hemorrhage and adverse liver outcomes in patients with cystic fibrosis cirrhosis', Journal of pediatric gastroenterology and nutrition, vol. 66, no. 1, pp. 122-127. https://doi.org/10.1097/MPG.0000000000001728
Ye, Wen ; Narkewicz, Michael R. ; Leung, Daniel H. ; Karnsakul, Wikrom ; Murray, Karen F. ; Alonso, Estella M. ; Magee, John C. ; Schwarzenberg, Sarah Jane ; Weymann, Alexander ; Molleston, Jean P. / Variceal hemorrhage and adverse liver outcomes in patients with cystic fibrosis cirrhosis. In: Journal of pediatric gastroenterology and nutrition. 2018 ; Vol. 66, No. 1. pp. 122-127.
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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.",
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T1 - Variceal hemorrhage and adverse liver outcomes in patients with cystic fibrosis cirrhosis

AU - Ye, Wen

AU - Narkewicz, Michael R.

AU - Leung, Daniel H.

AU - Karnsakul, Wikrom

AU - Murray, Karen F.

AU - Alonso, Estella M.

AU - Magee, John C.

AU - Schwarzenberg, Sarah Jane

AU - Weymann, Alexander

AU - Molleston, Jean P.

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N2 - 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.

AB - 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.

KW - Cirrhosis

KW - Cystic fibrosis

KW - Liver

KW - Portal HTN

KW - Variceal bleeding

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