Hepatic encephalopathy and orotic aciduria associated with hepatocellular carcinoma in a noncirrhotic liver

Authors

  • Lennox J. Jeffers,

    1. Division of Hepatology, Departments of Internal Medicine and Surgery, Center for Liver Diseases, University of Miami School of Medicine, Veterans Administration Medical Center, Miami, Florida 33125
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  • Richard A. Dubow,

    1. Division of Hepatology, Departments of Internal Medicine and Surgery, Center for Liver Diseases, University of Miami School of Medicine, Veterans Administration Medical Center, Miami, Florida 33125
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  • Leslie Zieve,

    1. Division of Hepatology, Departments of Internal Medicine and Surgery, Center for Liver Diseases, University of Miami School of Medicine, Veterans Administration Medical Center, Miami, Florida 33125
    Current affiliation:
    1. Hennepin County Medical Center, Minneapolis, Minnesota
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  • K. Rajender Reddy,

    1. Division of Hepatology, Departments of Internal Medicine and Surgery, Center for Liver Diseases, University of Miami School of Medicine, Veterans Administration Medical Center, Miami, Florida 33125
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  • Alan S. Livingstone,

    1. Division of Hepatology, Departments of Internal Medicine and Surgery, Center for Liver Diseases, University of Miami School of Medicine, Veterans Administration Medical Center, Miami, Florida 33125
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  • Sidney Neimark,

    1. Division of Hepatology, Departments of Internal Medicine and Surgery, Center for Liver Diseases, University of Miami School of Medicine, Veterans Administration Medical Center, Miami, Florida 33125
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    • Dr. Neimark is a practicing gastroenterologist in West Palm Beach, Florida.

  • Manuel Viamonte,

    1. Division of Hepatology, Departments of Internal Medicine and Surgery, Center for Liver Diseases, University of Miami School of Medicine, Veterans Administration Medical Center, Miami, Florida 33125
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  • Eugene R. Schiff M.D.

    Corresponding author
    1. Division of Hepatology, Departments of Internal Medicine and Surgery, Center for Liver Diseases, University of Miami School of Medicine, Veterans Administration Medical Center, Miami, Florida 33125
    • Division of Hepatology, Veterans Administration Medical Center, 1201 N.W. 16th Street, Miami, Florida 33125
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Abstract

A 40-yr-old man presented with encephalopathy and was found to have hepatocellular carcinoma without cirrhosis. A large vascular hepatic mass was defined by CT scan and angiography; laparoscopy with biopsy confirmed the absence of chronic liver disease. A definitive tissue diagnosis of hepatocellular carcinoma was made at laparotomy; the tumor was unresectable. Peripheral arterial and selective portal and hepatic venous ammonia levels were high, and this finding suggested that the encephalopathy was nitrogenous and hepatic in origin. The proposed mechanisms of the encephalopathy are generation of ammonia from tumor breakdown and por-tosystemic shunting, a result of partial tumor occlusion of the hepatic veins. An unusually high urinary excretion of orotic acid was found similar to that seen in hereditary orotic aciduria.

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