title: Liver transplantation for fulminant hepatic failure in infancy: a single center experience.
abstract: FHF is characterized by a high percentage of unknown causes leading to acute liver failure and furthermore by an increased morbidity and mortality prior to and post-Ltx. In different transplant centers, the reasons leading to FHF differ significantly as well as outcome. We report our single center experience with 30 pediatric patients receiving a liver transplant for FHF, out of a total of 83 children presenting with FHF. The time to transfer patients to the transplant center after the diagnosis of FHF was long, with a median of 14 days (Ltx group) and 12 days (controls), respectively. In nearly half of the patients (n = 14) in the Ltx group, we were not able to establish an exact diagnosis prior to Ltx: 50% suffered from encephalopathy, and 13 patients were treated in the intensive care unit prior to transplant. Because of the availability of different surgical techniques, all children received a timely transplant [split (n = 18), living donor (n = 9), whole organ (n = 2), and reduced liver (n = 1)]. Patient survival was 93.4%, and graft survival was 83.4% for at least one yr follow-up. Severe complications following Ltx included three cases with aplastic anemia and one child suffering from systemic mitochondrial depletion syndrome. The survival of patients treated medically was 83%. We conclude that a strong focus should be made on early referral to a specialized center and on improvement of diagnostic tools to timely detect the underlying reason for FHF. Results following Ltx for FHF are good.
authors.affiliation: Department of Pediatrics, Pediatric Hepatology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: Annette
authors.initials: A
authors.lastname: Strauss
authors.forename: Enke
authors.initials: E
authors.lastname: Grabhorn
authors.forename: Marijke
authors.initials: M
authors.lastname: Sornsakrin
authors.forename: Andrea
authors.initials: A
authors.lastname: Briem-Richter
authors.forename: Lutz
authors.initials: L
authors.lastname: Fischer
authors.forename: Bjoern
authors.initials: B
authors.lastname: Nashan
authors.forename: Rainer
authors.initials: R
authors.lastname: Ganschow
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006085:Graft Survival; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D017114:Liver Failure, Acute / Q000628:therapy*; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D011183:Postoperative Complications; D012189:Retrospective Studies; D016896:Treatment Outcome