Liver support--a task for nephrologists? Extracorporeal treatment of a patient with fulminant Wilson crisis.
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.
Manz
Tanja
T
Department of Nephrology, Albert Ludwigs University, Freiburg i.Br., Germany.
Ochs
Andreas
A
Bisse
Emmanuel
E
Strey
Christoph
C
Grotz
Wolfgang
W
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
D003300:Copper; D010396:Penicillamine; D001663:Bilirubin