title: Surgical and metabolic aspects of liver transplantation for tyrosinemia.
abstract: Tyrosinemia is the diagnosis of a very small percentage of patients undergoing liver transplantation worldwide. Tyrosinemia is endemic within our referral area however, and fully one-third of the liver transplantations at our institution are done for this disease. Since 1986, 15 patients with tyrosinemia (TYR) and 31 patients with various other indications (non-TYR) have undergone a total of 51 liver transplantations. The 36-month actuarial survival for TYR patients is 87%, compared with 74% for non-TYR patients. Liver transplantation for hereditary tyrosinemia and other metabolic disorders without portal hypertension or previous portohepatic operations is notably easier to perform. Intraoperative blood loss was less, length of hospital stay was shorter, and incidence of infections was lower in TYR than in non-TYR patients. Less than 10% of TYR patients had foci of hepatocellular carcinoma at the time of transplantation. For this reason, and while most patients with tyrosinemia will eventually require liver transplantation, our results do not support systematic early transplantation before the age of two years.
authors.affiliation: Department of Surgery, Hôpital Sainte-Justine, Université de Montréal, Quebec, Canada.
authors.forename: F I
authors.initials: FI
authors.lastname: Luks
authors.forename: D
authors.initials: D
authors.lastname: St-Vil
authors.forename: B J
authors.initials: BJ
authors.lastname: Hancock
authors.forename: J M
authors.initials: JM
authors.lastname: Laberge
authors.forename: A L
authors.initials: AL
authors.lastname: Bensoussan
authors.forename: P
authors.initials: P
authors.lastname: Russo
authors.forename: G
authors.initials: G
authors.lastname: Mitchell
authors.forename: M
authors.initials: M
authors.lastname: Lambert
authors.forename: H
authors.initials: H
authors.lastname: Blanchard
mesh_terms: D000293:Adolescent; D000592:Amino Acid Metabolism, Inborn Errors / Q000378:metabolism* / Q000401:mortality / Q000601:surgery*; D001656:Biliary Atresia / Q000401:mortality / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D007223:Infant; D016031:Liver Transplantation / Q000502:physiology; D008297:Male; D011792:Quebec / Q000453:epidemiology; D015996:Survival Rate; D014443:Tyrosine / Q000097:blood* / Q000378:metabolism
chemical_list: D014443:Tyrosine