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Plasma exchange and chelator therapy rescues acute liver failure in Wilson disease without liver transplantation

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Wilson disease (WD) in patients with a New Wilson Index (NWI) score ≥ 11 is fatal, and these patients are good candidates for liver transplantation (LT). However, plasma exchange and… Click to show full abstract

Wilson disease (WD) in patients with a New Wilson Index (NWI) score ≥ 11 is fatal, and these patients are good candidates for liver transplantation (LT). However, plasma exchange and chelator therapy are indispensable and effective even for WD with a score ≥ 11. Moreover, continuous hemodiafiltration (CHDF) with these treatments is essential for acute liver failure (ALF) in WD with hepatic encephalopathy because CHDF can exclude toxic metabolites that may cause damage to the brain. Here, we describe four rescued patients presenting with ALF in WD and discuss the available treatment options.

Keywords: plasma exchange; acute liver; exchange chelator; liver transplantation; chelator therapy; wilson disease

Journal Title: Hepatology Research
Year Published: 2017

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