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

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.

SUBMITTER: Ye W

PROVIDER: S-EPMC5745284 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature

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

Ye Wen W Narkewicz Michael R MR Leung Daniel H DH Karnsakul Wikrom W Murray Karen F KF Alonso Estella M EM Magee John C JC Schwarzenberg Sarah Jane SJ Weymann Alexander A Molleston Jean P JP

Journal of pediatric gastroenterology and nutrition 20180101 1

<h4>Objectives</h4>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.<h4>Methods</h4>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-pro ...[more]

PMID: 28906321

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Project description:Background and Purpose- Cirrhosis-clinically overt, advanced liver disease-is associated with an increased risk of hemorrhagic stroke and poor stroke outcomes. We sought to investigate whether subclinical liver disease, specifically liver fibrosis, is associated with clinical and radiological outcomes in patients with primary intracerebral hemorrhage. Methods- We performed a retrospective cohort study using data from the Virtual International Stroke Trials Archive-Intracerebral Hemorrhage. We included adult patients with primary intracerebral hemorrhage presenting within 6 hours of symptom onset. We calculated 3 validated fibrosis indices-Aspartate Aminotransferase-Platelet Ratio Index, Fibrosis-4 score, and Nonalcoholic Fatty Liver Disease Fibrosis Score-and modeled them as continuous exposure variables. Primary outcomes were admission hematoma volume and hematoma expansion. Secondary outcomes were mortality, and the composite of major disability or death, at 90 days. We used linear and logistic regression models adjusted for previously established risk factors. Results- Among 432 patients with intracerebral hemorrhage, the mean Aspartate Aminotransferase-Platelet Ratio Index, Fibrosis-4, and Nonalcoholic Fatty Liver Disease Fibrosis Score values on admission reflected intermediate probabilities of fibrosis, whereas standard hepatic assays and coagulation parameters were largely normal. After adjusting for potential confounders, Aspartate Aminotransferase-Platelet Ratio Index was associated with hematoma volume (?, 0.20 [95% CI, 0.04-0.36]), hematoma expansion (odds ratio, 1.6 [95% CI, 1.1-2.3]), and mortality (odds ratio, 1.8 [95% CI, 1.1-2.7]). Fibrosis-4 was also associated with hematoma volume (?, 0.27 [95% CI, 0.07-0.47]), hematoma expansion (odds ratio, 1.9 [95% CI, 1.2-3.0]), and mortality (odds ratio, 2.0 [95% CI, 1.1-3.6]). Nonalcoholic Fatty Liver Disease Fibrosis Score was not associated with any outcome. Indices were not associated with the composite of major disability or death. Conclusions- In patients with largely normal liver chemistries, 2 liver fibrosis indices were associated with admission hematoma volume, hematoma expansion, and mortality after intracerebral hemorrhage.

Project description:The results of endoscopic treatment with elastic band ligation for esophageal variceal bleeding in patients with Child-Pugh class C cirrhosis remain poor. In comparison with treatment with elastic band ligation, we have found lower rates of rebleeding and mortality with n-butyl-2-cyanoacrylate injections. Thus, the aim of the current study was to describe our unit's 10 years of experience with injection of n-butyl-2-cyanoacrylate to control esophageal variceal ruptures in patients with Child-Pugh class C cirrhosis.A single-center, retrospective study was conducted. Sixty-three patients with Child-Pugh class C cirrhosis had been admitted to the center with an acute episode of esophageal variceal bleeding. All were treated with injection of n-butyl-2-cyanoacrylate. The patients were assigned to 1 of 2 groups according to their Child-Pugh class C cirrhosis scores: group I (score range, 10 through 13 points) and group II (score, 14 or 15 points). The 3 variables studied were rates of initial failure to control bleeding, failure to prevent rebleeding (secondary prophylaxis), and mortality. Patients in the 2 groups (group I, n = 50; group II, n = 13) had similar characteristics.Bleeding was successfully controlled in almost 75 % of the patients during the first 5 days after treatment, with no significant differences observed between groups I and II. There were no significant differences between the 2 groups with respect to mortality rate for the first 5 days after treatment. Thirty-four patients (54 %) were free of bleeding at 6 weeks after treatment, with a significant difference noted between the groups: group I, 64 %, versus group II, 15.4 % (P < 0.001). The overall mortality rate was 44.4 %, with a significant difference noted between the groups: group I, 34 %, versus group II, 84.6 % (P < 0.001).Endoscopic injection of n-butyl-2-cyanoacrylate is a valid treatment option to control esophageal variceal bleeding in patients with a Child-Pugh class C cirrhosis score in the range of 10 through 13 points.

Dataset Information

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

Publications

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

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