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Liver transplantation for late-onset presentations of acute liver failure in Wilson's disease: The UK experience over 2 decades.


ABSTRACT: Background & 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 Methods:We report a case, review the literature and provide unpublished data from the UK Transplant Registry on late-onset acute liver failure in Wilson's disease.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 the case of a 62-year-old man presenting with acute liver failure who was successfully treated with urgent liver transplantation. We identified 7 cases presenting at age 40 years or over in the literature, for which individual outcomes were reported; 3 were treated with transplantation and 2 survived. We identified a further 8 cases listed for transplantation in the UK between 1995 and 2014; 7 were treated with transplantation and 6 survived. One patient was de-listed for unknown reasons. Conclusions:Wilson's disease should be considered in older adults presenting with acute liver failure. 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 as acute liver failure. Lay summary:Wilson's disease is a rare inherited disease that causes copper accumulation in the liver and brain and usually manifests during childhood, adolescence or early adulthood. We report the case of a 62-year-old who developed acute liver failure and was successfully treated with urgent liver transplantation. We discuss the outcomes of other late-onset cases of acute liver failure due to Wilson's disease in the literature and provide additional data from the UK Transplant Registry.

SUBMITTER: Shribman S 

PROVIDER: S-EPMC7163315 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature

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Liver transplantation for late-onset presentations of acute liver failure in Wilson's disease: The UK experience over 2 decades.

Shribman Samuel S   Webb Gwilym G   Taylor Rhiannon R   Warner Thomas T TT   Duckworth Adam A   Gimson Alexander A   Shenoy Achuth A   Griffiths William W  

JHEP reports : innovation in hepatology 20200309 3


<h4>Background & aims</h4>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.<h4>Methods</h4>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.<h4>Results</h4>We describe the cas  ...[more]

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