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Liver support--a task for nephrologists? Extracorporeal treatment of a patient with fulminant Wilson crisis.

AbstractBACKGROUND:
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.
CONCLUSION:
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.
AuthorsTanja Manz, Andreas Ochs, Emmanuel Bisse, Christoph Strey, Wolfgang Grotz
JournalBlood purification (Blood Purif) Vol. 21 Issue 3 Pg. 232-6 ( 2003) ISSN: 0253-5068 [Print] Switzerland
PMID12784049 (Publication Type: Case Reports, Journal Article)
CopyrightCopyright 2003 S. Karger AG, Basel
Chemical References
  • Copper
  • Penicillamine
  • Bilirubin
Topics
  • Adolescent
  • Bilirubin (blood)
  • Copper (blood, urine)
  • Female
  • Hepatic Encephalopathy (therapy)
  • Hepatolenticular Degeneration (therapy)
  • Humans
  • Liver Failure (therapy)
  • Penicillamine (therapeutic use)
  • Plasma Exchange
  • Renal Dialysis

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