Session Type: Poster Session
Date: Monday, June 3, 2019
Session Time: 6:00pm-7:00pm
Presentation Time: 6:00pm-7:00pm
Location: Hall C & D
*Purpose: Wilson’s disease (WD) is a rare genetic disorder of copper metabolism, which leads to the accumulation of copper in the body, causing damage to tissues. In most cases, WD can be managed adequately by chelating agents; however, chronic liver disease that is unable to be managed by chelation therapy is an indication for liver transplantation (LT). LT is also the only effective treatment for acute liver failure associated with WD. The aim of the study is to examine the clinical manifestations of WD patients at the time of LT listing and post-LT outcomes in order to determine the optimal timing for transplantation in WD patients.
*Methods: Data from 18 studies regarding clinical features and outcomes of 792 WD patients who received a liver transplant between 1971 and 2014 were reviewed. Variables including age at diagnosis and LT, admission-transplant interval, model of end-stage liver disease/pediatric end-stage liver disease (MELD/PELD) score, WD prognostic index (WPI), acute liver failure, chronic liver disease, chelation therapy, neurological symptoms, Kayser-Fleischer rings, hepatic encephalopathy, ascites, hemolysis, renal insufficiency, intubation, post-operative complications, and retransplantation were examined. In addition, values including ceruloplasmin, urine copper, liver copper, prothrombin time (PTT), Internationalized Normalized Ratio (INR), aspartate aminotransferase (AST), alanine aminotransferase (ALT), AST/ALT ratio, total bilirubin, alkaline phosphatase (AP), AP/bilirubin ratio, albumin, white blood cell count (WCC), hemoglobin, hematocrit, platelets, and creatinine were evaluated.
*Results: Liver and urinary copper, bilirubin, AST, PTT, INR, albumin, WCC, and creatinine appear to be some of the most important factors in determining the relative need of an LT for a patient with WD. Acute liver failure, neurological symptoms, ascites, hepatic encephalopathy, renal insufficiency, and ventilator use were some of the variables most associated with lower post-operative survival rates.
*Conclusions: For most WD patients, LT is effective at solving the copper metabolism defect and increasing longevity; however, due to the shortage of available donors, as well as the negative side effects associated with continued immunosuppression, LT should be indicated only when necessary. LT listing should have deliberate timing in order to minimize risks, avoid unnecessary transplantations, and maximize patient outcomes.
To cite this abstract in AMA style:Flanagan M, Koohmaraie S, Kueht M, Cotton R, O'Mahony C, Rana A, Goss J, Galvan N. When to Transplant for Wilson’s Disease: A Review [abstract]. Am J Transplant. 2019; 19 (suppl 3). https://atcmeetingabstracts.com/abstract/when-to-transplant-for-wilsons-disease-a-review/. Accessed November 29, 2022.
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