Shribman, S; Webb, G; Taylor, R; Warner, TT; Duckworth, A; Gimson, A; Shenoy, A; Shribman, S; Webb, G; Taylor, R; Warner, TT; Duckworth, A; Gimson, A; Shenoy, A; Griffiths, W; - view fewer (2020) Liver transplantation for late-onset acute liver failure in Wilson’s disease: the UK experience over two decades. JHEP Reports 10.1016/j.jhepr.2020.100096. (In press).

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Abstract

BACKGROUNDS AND AIMS: Acute liver failure as the initial presentation of Wilson’s disease is usually associated with onset in childhood, adolescence or early adulthood. Outcomes after transplantation for late-onset presentations, at or after 40 years, are seldom reported in the literature METHODS: We report a case, review the literature and provide unpublished data from the UK Transplant Registry on late-onset acute liver failure in Wilson’s disease. RESULTS: We describe a 62-year-old man presenting with acute liver failure who was successfully treated with urgent liver transplantation. We identified seven cases presenting at age 40 years or over in the literature where individual outcomes were reported; three were treated with transplantation and two survived. We identified a further eight cases listed for transplantation in the UK between 1995 and 2014; seven were treated with transplantation and six survived. One patient was de-listed for unknown reasons. CONCLUSIONS: We discuss the need to consider Wilson’s disease in older adults presenting with acute liver failure and importance of making the diagnosis prior to transplantation. We suggest that urgent liver transplantation has good outcomes for late-onset presentations and recommend that urgent transplantation should always be considered in Wilson’s disease presenting with acute liver failure.

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