Albumin dialysis and molecular adsorbents recirculating system (MARS) for acute Wilson’s disease
Open Access
- 1 October 2002
- journal article
- case report
- Published by Wolters Kluwer Health in Liver Transplantation
- Vol. 8 (10) , 962-967
- https://doi.org/10.1053/jlts.2002.35546
Abstract
Wilson’s disease presenting as acute liver failure (ALF) is potentially fatal, and liver transplantation (LTx) is the only option. We report two patients with Wilson’s disease and ALF treated with the Molecular Adsorbents Recirculating System (MARS). Both patients fulfilled criteria for poor prognosis. Because LTx was not available immediately in either case, MARS was used as a bridge to LTx. In Case 1, serum bilirubin decreased from 803 to 425 μmol/L after 3 treatments, but increased to 656 μmol/L during a break, decreasing again to 457 μmol/L with further treatment. Serum copper decreased from 53.7 μmol/L, to 35.8 μmol/L after first treatment session, and 17.4 μmol/L at treatment completion. In Case 2, MARS treatment over 2 weeks reduced serum bilirubin from 1200 to 450 μmol/L and copper from 35 to 13 μmol/L with marked improvement in encephalopathy and reduction in ammonia (59 to 34 μmol/L). Both patients were successfully bridged to LTx (days 9 and 28, respectively). Analysis of albumin-dialysate from the MARS circuit suggested that copper removal occurred mostly in the first few hours of treatment, partly being adsorbed by albumin and partly by the MARSFlux membrane (Teraklin AG, Rostock, Germany). These data suggest that MARS removes copper efficiently and can be used to bridge patients with Wilson’s disease and ALF to LTx.Keywords
This publication has 14 references indexed in Scilit:
- Blood lactate as an early predictor of outcome in paracetamol-induced acute liver failure: a cohort studyThe Lancet, 2002
- Molecular Adsorbent Recycling System (MARS): Clinical Results of a New Membrane‐Based Blood Purification System for Bioartificial Liver SupportArtificial Organs, 1999
- Effective removal of copper by plasma exchange in fulminant Wilson's diseaseTransfusion, 1998
- Efficient Detection of Mutations in Wilson Disease by Manifold SequencingGenomics, 1996
- Liver transplantation for Wilson’s disease: Indications and outcomeHepatology, 1994
- The Wilson disease gene is a putative copper transporting P–type ATPase similar to the Menkes geneNature Genetics, 1993
- Early indicators of prognosis in fulminant hepatic failureGastroenterology, 1989
- Copper distribution among serum proteins in paediatric liver disorders and malignanciesEuropean Journal of Clinical Investigation, 1988
- Fulminant Wilson's disease with haemolysis and renal failure: copper studies and assessment of dialysis regimens.BMJ, 1977
- Acute Intravascular Hemolysis and Acute Liver Failure Associated as a First Manifestation of Wilson's DiseaseAnnals of Internal Medicine, 1977