Revised King's College score for liver transplantation in adult patients with Wilson's disease

Authors

  • Jan Petrasek,

    1. Department of Hepatogastroenterology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
    2. Laboratory of Experimental Hepatology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
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  • Milan Jirsa,

    1. Laboratory of Experimental Hepatology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
    2. Institute of Clinical Biochemistry and Laboratory Diagnostics, 1st Faculty of Medicine, Charles University, Prague, Czech Republic
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  • Jan Sperl,

    1. Department of Hepatogastroenterology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
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  • Libor Kozak,

    1. Center of Molecular Biology and Gene Therapy, Brno, Czech Republic
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  • Pavel Taimr,

    1. Department of Hepatogastroenterology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
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  • Julius Spicak,

    1. Department of Hepatogastroenterology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
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  • Karel Filip,

    1. Department of Hepatogastroenterology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
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  • Pavel Trunecka

    Corresponding author
    1. Department of Hepatogastroenterology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
    • Department of Hepatogastroenterology, Institute for Clinical and Experimental Medicine (IKEM), Videnska 1958, 14000 Prague, Czech Republic
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    • Telephone: 420261364016; FAX: 420261362819


Abstract

Fulminant Wilson's disease (WD) is almost invariably fatal, and liver transplantation is the only life-saving treatment. Decompensated chronic WD usually responds to chelation therapy. Our aim was to validate 3 published scoring systems for deciding between chelation treatment and liver transplantation in patients with chronic decompensated and fulminant WD. Model for end-stage liver disease (MELD) score, as well as WD prognostic index (WPI) and its recently revised version (RWPI) were evaluated as predictors of the safety for chelation therapy. A group of 14 adult patients with decompensated chronic WD who improved on penicillamine treatment were compared with 21 patients with fulminant WD. The diagnosis of WD was based on increased urinary copper excretion and confirmed by elevated liver copper content and/or mutation analysis of the WD gene. The MELD score, WPI, and RWPI were calculated for all patients with WD. The accuracy of the MELD score, WPI, and RWPI for prediction of response to chelation therapy in patients with decompensated chronic WD was 0.968, 0.980, and 0.993, respectively. None of the decompensated chronic WD patients had a MELD score >30, RWPI >11, or WPI >7. RWPI showed the highest accuracy and the lowest false negativity compared with WPI and MELD. In conclusion, our data indicate that RWPI, originally proposed for pediatric patients, is also useful for adults. Liver Transpl, 2007. © 2006 AASLD.

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