title: Liver transplantation for hereditary tyrosinemia: the Quebec experience.
abstract: Sixteen tyrosinemic patients were evaluated in our institution for a possible liver transplantation. All patients showed biochemical and/or radiological evidence of liver dysfunction. Renal involvement was found to be more abnormal than expected. Seven patients have been transplanted, with two patients receiving a combined liver-kidney transplant. Hepatocarcinoma was detected in two of eight patients in whom the whole liver was examined. Six (37.5%) of the initial 16 patients have died since evaluation, one of the six dying after combined liver-kidney transplantation. Posttransplantation survival was 86%, with normal liver function, normal growth, and no recurrence of neurological crises on a normal diet.
authors.affiliation: Department of Pediatrics and Radiology, Hôpital Sainte-Justine, Montreal, Quebec, Canada.
authors.forename: K
authors.initials: K
authors.lastname: Paradis
authors.forename: A
authors.initials: A
authors.lastname: Weber
authors.forename: E G
authors.initials: EG
authors.lastname: Seidman
authors.forename: J
authors.initials: J
authors.lastname: Larochelle
authors.forename: L
authors.initials: L
authors.lastname: Garel
authors.forename: C
authors.initials: C
authors.lastname: Lenaerts
authors.forename: C C
authors.initials: CC
authors.lastname: Roy
mesh_terms: D000293:Adolescent; D000592:Amino Acid Metabolism, Inborn Errors / Q000235:genetics / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D008297:Male; D011379:Prognosis; D011792:Quebec; D014443:Tyrosine / Q000097:blood*
chemical_list: D014443:Tyrosine