Background/Aims: Orthotopic liver transplantation (OLT) is life-saving for patients with liver failure from Wilson Disease (WD). Our goal was to evaluate patient and graft survival post-OLT for WD and identify predictors of poor survival using UNOS database.

Methods: Retrospective analysis of UNOS data identified 156 pediatric and 515 adult cases of OLT for WD between 1987 and 2017. Comparison cases were 10442 pediatric and 104874 adult non-WD transplant recipients. Fulminant and chronic cases of WD were also compared. Cases of multiorgan or re-transplantation were excluded. Survivals were calculated using Kaplan-Meier analysis. Recipient, donor, and surgical variables were compared by Cox regression.

Results: The 3yr-, 5-yr, and 10-yr patient survival post-OLT for adults with WD was 87.5% and 85.4% and 80.5%, respectively, which was significantly greater than survival in non-WD, p<0.001. Pediatric (age<18 yr) patient survival at 3-yr, 5-yr, and 10-yr for WD was 90.5%, 89.7%, and 86.5%, not significantly better than non-WD patients. Graft survival in adult and pediatric patient followed a similar trend. When compared by era (87-94, 95-06, 07-17), post-transplant patient and graft survival have improved over the years, but not statistically significant; log rank p-value 0.06. Regression analysis identified older age, and use of life support at the time of transplant as predictors of decreased survival for adults, and older age and high BMI were predictors for poor survival in pediatric recipients. Presentation with acute liver failure was not a significant predictor of survival between WD and non-WD patients. Donor characteristics such as age, gender, weight and cold ischemia time had no effect in survival in adult or pediatric recipients with WD.

Conclusions: Long-term patient and graft survival post-OLT is excellent for WD recipients. Older age, and use of life support predicted poor survival for adult cases of WD. Furthermore, survivals were comparable for adult and pediatric or fulminant and chronic cases of WD. Donor characteristics did not impact survival in adult or pediatric patients.

CITATION INFORMATION: Jalal P., May S., Rana A. Long-Term Outcomes and Predictors of Survival after Liver Transplantation for Wilson Disease (WD) Am J Transplant. 2017;17 (suppl 3).

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