Albumin dialysis and Molecular Adsorbents Recirculating System (MARS) for acute Wilson's disease.
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 micromol/L after 3 treatments, but increased to 656 micromol/L during a break, decreasing again to 457 micromol/L with further treatment. Serum copper decreased from 53.7 micromol/L, to 35.8 micromol/L after first treatment session, and 17.4 micromol/L at treatment completion. In Case 2, MARS treatment over 2 weeks reduced serum bilirubin from 1200 to 450 micromol/L and copper from 35 to 13 micromol/L with marked improvement in encephalopathy and reduction in ammonia (59 to 34 micromol/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.
D000208:Acute Disease; D000327:Adsorption; D000328:Adult; D000418:Albumins / Q000627:therapeutic use*; D001663:Bilirubin / Q000097:blood; D003300:Copper / Q000097:blood; D015314:Dialysis Solutions / Q000627:therapeutic use*; D005260:Female; D006501:Hepatic Encephalopathy / Q000209:etiology; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000150:complications* / Q000628:therapy*; D006801:Humans; D017093:Liver Failure / Q000209:etiology*; D016031:Liver Transplantation; D008567:Membranes, Artificial; D006435:Renal Dialysis / Q000379:methods*; D013997:Time Factors
D000418:Albumins; D015314:Dialysis Solutions; D008567:Membranes, Artificial; D003300:Copper; D001663:Bilirubin