title: Liver support--a task for nephrologists? Extracorporeal treatment of a patient with fulminant Wilson crisis.
abstract: BACKGROUND
  Patients with Wilson's disease may present with cirrhosis, acute hepatitis or fulminant hepatic failure. Without urgent orthotopic liver transplantation, a fulminant Wilson crisis has a mortality of 100%. We report on an 18-year-old female patient with fulminant hepatic failure due to Wilson crisis.
  
  
  METHODS
  The molecular adsorbent recirculating system (MARS) was used to eliminate albumin-bound toxins and to bridge waiting until an organ became available.
  
  
  RESULTS
  A total of 18 MARS sessions and 4 plasma exchange sessions were performed. Bilirubin levels and hepatic encephalopathy improved under MARS therapy. A total of 75 mg copper was removed until serum copper levels were within the normal range. Copper elimination was measured in 15 MARS treatments, which removed a total of 12.9 mg copper. Four plasma exchange sessions, with a total exchange of 11 liters of plasma, removed 12 mg copper. Urinary copper elimination with penicillamine was 50 mg.
  
  
  CONCLUSIONS
  MARS was an effective method to stabilize a patient with Wilson crisis, contributed to copper elimination and gained time for liver transplantation. The risk of high-urgency transplantation could be avoided. Liver support was easy in the hands of nephrologists familiar with extracorporeal therapy.
authors.affiliation: Department of Nephrology, Albert Ludwigs University, Freiburg i.Br., Germany.
authors.forename: Tanja
authors.initials: T
authors.lastname: Manz
authors.forename: Andreas
authors.initials: A
authors.lastname: Ochs
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Bisse
authors.forename: Christoph
authors.initials: C
authors.lastname: Strey
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Grotz
mesh_terms: D000293:Adolescent; D001663:Bilirubin / Q000097:blood; D003300:Copper / Q000097:blood / Q000652:urine; D005260:Female; D006501:Hepatic Encephalopathy / Q000628:therapy; D006527:Hepatolenticular Degeneration / Q000628:therapy*; D006801:Humans; D017093:Liver Failure / Q000628:therapy*; D010396:Penicillamine / Q000627:therapeutic use; D010951:Plasma Exchange; D006435:Renal Dialysis
chemical_list: D003300:Copper; D010396:Penicillamine; D001663:Bilirubin