Ann Liver Transplant.  2021 Nov;1(2):113-122. 10.52604/alt.21.0018.

Liver transplantation in Wilson’s disease: A systematic review

• Turgut E
• Aydin C
• Kayaalp C
• Yilmaz S

• KMID: 2560860
• DOI: http://doi.org/10.52604/alt.21.0018

Abstract

Wilson’s disease (WD) is a rare genetic disorder affecting the metabolism of copper that may present with neurological symptoms. In cases leading to hepatic failure, liver transplantation (LT) may be necessary. The aim of this paper is to examine the literature on LT cases related to WD in terms of disease characteristics, the neurological conditions of patients and survival rates. The keywords “Wilson and transplant*” were used to perform a title and abstract search across three databases (PubMed, Scopus, Web of Science) up until November 2018. The results from a total of 791 patients, mentioned in 28 studies, were analyzed. Thirty-nine point six percent of the patients received emergency LT and acute liver failure was more predominant in woman patients (61.7%-38.3%; p<0.0001). Live donations accounted for 36.6% of the transplants with none of these patients experiencing recurrence of disease. 36.4% of the patients displayed neurological symptoms prior to the transplant; however, following the operation these symptoms decreased by 74.2%. Survival rates for 1, 2, 3, 5, 10 years were 84%, 82%, 81%, 81%, and 80%, respectively. Further research into the reasons that acute liver failure in patients suffering from WD is more common among women is needed. Moreover, while mortality rates for up to one-year post-transplant are 16%, this rate is only 4% for the following 1–10 years. As neurological symptoms experienced prior to transplantation decrease by 74.2% after the operation, further studies should be carried out into the factors that determine potential neurological improvement.

Keyword

Liver; Transplantation; Review