Journal
BLOOD PURIFICATION
Volume 28, Issue 2, Pages 102-107Publisher
KARGER
DOI: 10.1159/000218090
Keywords
Albumin dialysis; Fractionated plasma separation and adsorption dialysis; Liver failure; Copper; Liver transplantation
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Background: Wilson's disease (WD) can present in a fulminant form with hepatocellular dysfunction, hemolysis and multiorgan failure (Wilson's crisis). We present a previously healthy young woman with severe WD whose WD severity score was 13. A score >11 indicates a poor chance of survival and liver transplantation will usually be recommended. Methods: Penicillamine and acetylcysteine were initially administered, but the patient deteriorated further, and extracorporeal liver support with the Prometheus (R) FPSA (fractionated plasma separation and adsorption) system was initiated. The patient was treated 6 h daily during 3 consecutive days. Results: Severe hemolysis was reduced to low-grade hemolysis, with no further need for transfusions. The mental state improved and after 4 months practically all biochemical markers were normalized. Conclusions: This is the first report of FPSA albumin dialysis of a patient with Wilson's crisis and the first report in which a patient with a WD score >11 survived without transplantation. Copyright (C) 2009 S. Karger AG, Basel
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