A case of Wilson's disease with fulminan hepatic form survived with plasma exchange, glucagon-insulin (GI) therapy, prostaglandin E1 (PGE1) and haptoglobin.

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  • 血しょう交換療法，ＰＧＥ１療法，ＧＩ療法併用にて救命し得た劇症肝炎型Ｗｉｌｓｏｎ病の１例

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A 15-year-old girl was admitted to our hospital, because of general malaise and icterus. Based on the data of urinar copper and seum ceruloplasmin as well as Kayser-Fleisher ring in the cornea, this case was diagnosed as Wilson's disease. Although D-pennicillamine was administered, hepatic encephalopathy, ascites and icterus progressed. The maximum level of total bilirubin showed 80.26mg/dl. We treated this case with repeated plasma exchange, GI therapy, PGE1 and haptoglobin, because she presented progressive hepatic failure and hemolytic anemia. By using the combination therapies descrived above, the clinical course of this case showed remarkable improvement after five months from onset.<BR>This report presented a survived case of Wilson's disease with fulminant hepatic form, and pointed out the importance of various kinds of active therapies.

Journal

  • Kanzo

    Kanzo 36 (10), 589-593, 1995

    The Japan Society of Hepatology

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