title: Living-related liver transplantation and neurological outcome in children with fulminant hepatic failure.
abstract: BACKGROUND
  Fulminant hepatic failure (FHF) in children is associated with high mortality under medical management. Living-related liver transplantation (LRLT) is an accepted measure to treat the children with end-stage liver disease. Reversibility of hepatic encephalopathy is crucial for the quality of life among the survivors after transplantation.
  
  
  METHODS
  A retrospective review was made of the records of children undergoing LRLT at this hospital between May 1992 and November 1996.
  
  
  RESULTS
  Eleven children with FHF underwent emergency LRLT. The mean age was 5 years (range, 2 months to 15 years). The indication for transplantation was persistent or worsening hepatic encephalopathy and severe coagulopathy, despite repeated plasma exchanges or exchange transfusions. The cause of FHF was non-A, non-B hepatitis in seven children, hepatitis B in two children, herpes simplex virus hepatitis in one child, and fulminant Wilson's disease with intravascular hemolysis in one child. The grade of hepatic encephalopathy was II in four children, III 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 neurological morbidity was present in two children with grade IV encephalopathy who also showed brain edema on cranial computed tomography. Eight survivors exhibited no long-term neurological deficit; the mean intelligence or developmental quotient was 97 (range, 86-110) at the end of the follow-up period.
  
  
  CONCLUSIONS
  LRLT is an effective option for the treatment of FHF in children. The long-term neurological status is satisfactory among survivors.
authors.affiliation: Department of Pediatrics, Kyoto University School of Medicine, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Hattori
authors.forename: Y
authors.initials: Y
authors.lastname: Higuchi
authors.forename: M
authors.initials: M
authors.lastname: Tsuji
authors.forename: Y
authors.initials: Y
authors.lastname: Inomata
authors.forename: S
authors.initials: S
authors.lastname: Uemoto
authors.forename: K
authors.initials: K
authors.lastname: Asonuma
authors.forename: H
authors.initials: H
authors.lastname: Egawa
authors.forename: T
authors.initials: T
authors.lastname: Kiuchi
authors.forename: K
authors.initials: K
authors.lastname: Furusho
authors.forename: Y
authors.initials: Y
authors.lastname: Yamaoka
authors.forename: K
authors.initials: K
authors.lastname: Tanaka
mesh_terms: D001929:Brain Edema / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D006501:Hepatic Encephalopathy / Q000601:surgery*; D006525:Hepatitis, Viral, Human / Q000601:surgery*; D006801:Humans; D007223:Infant; D017093:Liver Failure / Q000601:surgery*; D016031:Liver Transplantation; D019520:Living Donors; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed