Wilson's Disease Research - Treatment, Causes, Symptoms, Medication

Wilson's Disease Research Today is a free monthly online journal that collates and summarizes the latest research about Wilson's Disease, including details on treatment, causes, symptoms, medication.


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The Use Prometheus FPSA System in the Treatment of Acute Liver Failure: Preliminary Results.

Skwarek A, Grodzicki M, Nyckowski P, Kotulski M, Zieniewicz K, Michalowicz B, Patkowski W, Grzelak I, Paczkowska A, Giercuszkiewicz D, Sańko-Resmer J, Paczek L, Krawczyk M

Department of General, Transplant and Liver Surgery, Warsaw Medical University, Warsaw, Poland.

The preliminary outcomes of patients with acute liver failure treated with the Prometheus Fractionated Plasma Separation and Absorption (FPSA) system are presented herein. PATIENTS AND METHODS: The procedures were performed in 13 patients (4, intoxication by Amanita phalloides; 4, unknown reason; 3, acetaminophen intoxication; 1, Wilson disease, and 1, liver insufficiency after hemihepatectomy owing to metastases of colon adenocarcinoma). The patients were qualified for the procedure according to the King's College Hospital criteria. The patients' general status was assessed on basic of GCS, UNOS, and the 4-grade encephalopathy classifications. The procedures were performed with the Prometheus 4008H Fresenius Medical Care unit. RESULTS: The 29 procedures were of mean duration 6.5 hours. There were statistically significant reductions in total bilirubin, ammonia, and aminotransferase levels. In addition, the procedures corrected water, mineral, and carbohydrate disorders. One patient did not require liver transplantation. Seven patients received liver transplants: three patients with positive outcomes; two died due to septicemia within 30 days perioperatively, one died at 6 months after OLT owing to respiratory failure; and one, owing to hemorrhagic diathesis. Four patients did not receive a liver transplant because of lack of a organ, no consent for the surgery, or neoplastic disease with metastases. CONCLUSIONS: The Prometheus FPSA-System was an effective detoxication method for patients with acute liver failure. The system was useful as a symptomatic treatment before liver transplantation allowing a longer wait for a graft.

Published 28 February 2006 in Transplant Proc, 38(1): 209-11.
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Wilson's Disease Research Today Archive:

Volume 1 (2005)
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  Issue 3 (May)
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  Issue 7 (September)
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Volume 2 (2006)
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Volume 3 (2007)
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Volume 4 (2008)
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Pathophysiology: Clinical Concepts of Disease Processes

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