LIVING-RELATED LIVER-TRANSPLANTATION AND NEUROLOGICAL OUTCOME IN CHILDREN WITH FULMINANT HEPATIC-FAILURE

Background. Fulminant hepatic failure (FHF) in children is associated with high mortality under medical management, Living-related liver tra nsplantation (LRLT) is an accepted measure to treat the children with end-stage liver disease, Reversibility of hepatic encephalopathy is cr ucial for the quality of life among the survivors after transplantatio n Methods. A retrospective review was made of the records of children undergoing LRLT at this hospital between May 1992 and November 1996. R esults, Eleven children with FHF underwent emergency LRLT. The mean ag e was 5 years (range, 2 months to 15 years), The indication for transp lantation was persistent or worsening hepatic encephalopathy and sever e coagulopathy, despite repeated plasma exchanges or exchange transfus ions, The cause of FHF was non-A, non-B hepatitis in seven children, h epatitis B in two children, herpes simplex virus hepatitis in one chil d, and fulminant Wilson's disease with intravascular hemolysis in one child, The grade of hepatic encephalopathy was II in four children, II I in two, and IV in five, The actuarial survival rate was 73% after a mean follow-up of 28 months (range, 13-67 months). Short-term neurolog ical morbidity was present in two children with grade IV encephalopath y who also showed brain edema on cranial computed tomography, Eight su rvivors exhibited no long term neurological deficit; the mean intellig ence or developmental quotient was 97 (range, 86-110) at the end of th e follow-up period, Conclusions. LRLT is an effective option for the t reatment of FHF in children, The long-term neurological status is sati sfactory among survivors.