title: Living related liver transplantation for acute liver failure in children.
abstract: The mortality rate among children with acute liver failure (ALF) on the waiting list for liver transplantation is high. We present our experience with living related donor liver transplantation (LRD-LT) in children who required urgent transplantation for ALF. Between December 1995 and July 1997, 6 children underwent LRD-LT for ALF. Cause of liver failure, recipient and donor demographics, clinical and laboratory data, surgical details, complications, and 6-month and 2-year graft and patient survival were recorded. Five boys and 1 girl received left lateral segment grafts from their parents. The mean age was 4 +/- 2.8 years (range, 1 to 9 years). ALF was caused by Wilson's disease in 1 patient and sickle cell intrahepatic cholestasis syndrome in 1 patient; in 4 patients, the cause was unknown. All patients had mental status changes; 2 were on life support. Mean pretransplantation liver function test values were: alanine aminotransferase, 972 +/- 565 U/L (normal, 1 to 53 U/L), total bilirubin, 31.3 +/- 12.4 mg/dL (normal, 0.1 to 1.2 mg/dL), prothrombin time, 34.3 +/- 12.4 seconds (normal, 10.8 to 13.3 seconds), international normalized ratio, 8.46 +/- 5.4 (normal < 2), and fibrinogen, 109 +/- 23.9 mg/dL (normal, 175 to 400 mg/dL). The donors were 5 mothers and 1 father. The mean donor age was 32.5 +/- 7.6 years (range, 19 to 40 years). No donor required blood transfusion, and no donor had any early or late postoperative complications. The donors' mean hospital length of stay was 5 days. In five cases, grafts were blood group-compatible; 1 child received a blood group-incompatible graft. All grafts functioned immediately. No patient had hepatic artery or portal vein thrombosis or biliary complications. The child who received a mismatched graft died of infection of the brain caused by Aspergillus spp at 22 days posttransplantation with a functioning graft. The child with ALF caused by sickle cell intrahepatic cholestasis syndrome developed outflow obstruction 3 months posttransplantation and required retransplantation; he eventually died of vascular complications related to his primary disease. Four children are alive at a mean follow-up of 27 months (range, 14 to 36 months). LRD-LT for children with ALF facilitates timely transplantation without drawing on cadaveric donor resources. The established safety record of LRD-LT made this option appealing to both physicians and parental donors.
authors.affiliation: Recanati/Miller Transplantation Institute, Mount Sinai Medical Center, New York, NY 10029, USA.
authors.forename: S
authors.initials: S
authors.lastname: Emre
authors.forename: M E
authors.initials: ME
authors.lastname: Schwartz
authors.forename: B
authors.initials: B
authors.lastname: Shneider
authors.forename: J
authors.initials: J
authors.lastname: Hojsak
authors.forename: L
authors.initials: L
authors.lastname: Kim-Schluger
authors.forename: T M
authors.initials: TM
authors.lastname: Fishbein
authors.forename: S R
authors.initials: SR
authors.lastname: Guy
authors.forename: P A
authors.initials: PA
authors.lastname: Sheiner
authors.forename: N S
authors.initials: NS
authors.lastname: LeLeiko
authors.forename: A
authors.initials: A
authors.lastname: Birnbaum
authors.forename: F J
authors.initials: FJ
authors.lastname: Suchy
authors.forename: C M
authors.initials: CM
authors.lastname: Miller
mesh_terms: D000328:Adult; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D006801:Humans; D007902:Length of Stay / Q000706:statistics & numerical data; D017114:Liver Failure, Acute / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D008111:Liver Function Tests; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D010290:Parents; D013997:Time Factors