Bridging use of plasma exchange and continuous hemodiafiltration before living donor liver transplantation in fulminant Wilson's disease.
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.
Nagata
Yoshiko
Y
Department of Internal Medicine II, Miyazaki Medical College, Kiyotake.
Uto
Hirofumi
H
Hasuike
Satoru
S
Ido
Akio
A
Hayashi
Katsuhiro
K
Eto
Toshiharu
T
Hamakawa
Toshiro
T
Tanaka
Koichi
K
Tsubouchi
Hirohito
H
D000293:Adolescent; D002614:Chelating Agents / Q000627:therapeutic use; D005260:Female; D017583:Hemodiafiltration / Q000379:methods*; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000601:surgery / Q000628:therapy*; D006801:Humans; D017093:Liver Failure / Q000209:etiology / Q000601:surgery / Q000628:therapy*; D016031:Liver Transplantation; D019520:Living Donors; D010396:Penicillamine / Q000627:therapeutic use; D010951:Plasma Exchange / Q000379:methods*; D011300:Preoperative Care; D016896:Treatment Outcome
D002614:Chelating Agents; D010396:Penicillamine