Volume 4, Issue 6 pp. 341-347
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Fulminant hepatic and renal failure complicating Wilson's disease

William G. Rector Jr. M.D.

Corresponding Author

William G. Rector Jr. M.D.

Division of Hepatology, Rancho Los Amigos Hospital, University of Southern California, Downey, California, U.S.A.

Division of Hepatology Rancho Los Amigos Hospital University of Southern California Downey California U.S.A.Search for more papers by this author
Toshikazu Uchida

Toshikazu Uchida

Division of Hepatology, Rancho Los Amigos Hospital, University of Southern California, Downey, California, U.S.A.

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Gary C. Kanel

Gary C. Kanel

Division of Hepatology, Rancho Los Amigos Hospital, University of Southern California, Downey, California, U.S.A.

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Allan G. Redeker

Allan G. Redeker

Division of Hepatology, Rancho Los Amigos Hospital, University of Southern California, Downey, California, U.S.A.

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Telfer B. Reynolds

Telfer B. Reynolds

Division of Hepatology, Rancho Los Amigos Hospital, University of Southern California, Downey, California, U.S.A.

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First published: December 1984
Citations: 18

Abstract

ABSTRACT— We report a case of fatal fulminant liver failure complicating Wilson's disease that is unique in several respects. The illness supervened after several years of medical noncompliance in a patient who had been previously diagnosed and successfully treated with penicillamine. Re-institution of penicillamine therapy 2 weeks prior to the fulminant decompensation failed to prevent it. Renal failure in this patient was apparently secondary to rhabdomyolysis. Addition of penicillamine to a peritoneal dialysis solution allowed chelation and removal of over 14 mg per day of copper but without apparent benefit. Exchange transfusion and high dose dexamethasone therapy (24 mg/day) were equally ineffective in reversing the liver failure. Other reported cases have also been fatal. The best treatment for fulminant Wilson's disease is prevention by diagnosis in a pre-symptomatic stage and institution of carefully supervised lifelong therapy with penicillamine.

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