Bridging use of plasma exchange and continuous hemodiafiltration before living donor liver transplantation in fulminant Wilson's disease.

Abstract

A 15-year-old girl presented with acute hepatic failure showing ascites and hepatic encephalopathy, accompanied by hemolytic anemia. She was diagnosed as having fulminant Wilson's disease (FWD). Plasma exchange (PE), continuous hemodiafiltration (CHDF) and D-penicillamine administration were started immediately. Copper [24,000 microg] was removed by PE and CHDF over three days, which relieved the jaundice and the consciousness disorder. A successful liver transplant followed. FWD progresses rapidly and often liver transplantation is the only possible therapy. In this case, PE and CHDF were an effective therapy bridge until liver transplantation.

• Authors: Yoshiko Nagata, Hirofumi Uto, Satoru Hasuike, Akio Ido, Katsuhiro Hayashi, Toshiharu Eto, Toshiro Hamakawa, Koichi Tanaka, Hirohito Tsubouchi
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 42 Issue 10 Pg. 967-70 (Oct 2003) ISSN: 0918-2918 [Print] Japan
• PMID: 14606709 (Publication Type: Case Reports, Journal Article)

Chemical References

• Chelating Agents
• Penicillamine

Topics

• Adolescent
• Chelating Agents (therapeutic use)
• Female
• Hemodiafiltration (methods)
• Hepatolenticular Degeneration (complications, surgery, therapy)
• Humans
• Liver Failure (etiology, surgery, therapy)
• Liver Transplantation
• Living Donors
• Penicillamine (therapeutic use)
• Plasma Exchange (methods)
• Preoperative Care
• Treatment Outcome