Fulminant Wilson's disease treated with postdilution hemofiltration and orthotopic liver transplantation

Jorge Rakela, Stephen B. Kurtz, James T. McCarthy, Jurgen Ludwig, Nancy L. Ascher, Joseph R. Bloomer, Paul L. Claus

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56 Scopus citations

Abstract

A 22-yr-old woman presented with fulminant Wilson's disease. The diagnosis was suspected clinically and was later confirmed with chemical and pathologic studies. She presented with acute hepatic failure, hemolysis, and acute anuric renal failure. Postdilution hemofiltration and continuous arteriovenous hemofiltration with oral d-penicillamine allowed removal of a total of 95,700 μg of copper; 78,665 μg of the total were removed via postdilution hemofiltration alone. On the 57th day, the patient received successful liver and renal transplants. We found that the determination of serum copper was instrumental in the diagnosis of fulminant Wilson's disease, that postdilution hemofiltration allowed a rapid removal of copper in the presence of renal failure, and that, finally, orthotopic liver transplantation should be performed early in the clinical course of these patients. This patient is the longest survivor of this serious condition.

Original languageEnglish (US)
Pages (from-to)2004-2007
Number of pages4
JournalGastroenterology
Volume90
Issue number6
DOIs
StatePublished - Jun 1986

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

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    Rakela, J., Kurtz, S. B., McCarthy, J. T., Ludwig, J., Ascher, N. L., Bloomer, J. R., & Claus, P. L. (1986). Fulminant Wilson's disease treated with postdilution hemofiltration and orthotopic liver transplantation. Gastroenterology, 90(6), 2004-2007. https://doi.org/10.1016/0016-5085(86)90274-X