SURGICAL AND METABOLIC ASPECTS OF LIVER-T RANSPLANTATION FOR TYROSINEMIA

Tyrosinemia represents a very small percentage of patients undergoing liver transplantation world-wide. This disease is endemic within our r eferral area however, one-third of the liver transplantations at our i nstitution are done for this disease. Sine 1986, 16 patients with tyro sinemia and 34 patients with various other indications (non-tyrosinemi c) have undergone a total of 55 liver transplantation. The survival ra te for tyrosinemic patients is 87 %, compared to 75 % for non-tyrosine mic patients. Liver transplantation for hereditary tyrosinemia and oth er metabolic disorders without portal hypertension or previous portohe patic operations is notably easier to perform. Intraoperative blood lo ss was less, length of hospital stay was shorter and incidence of infe ctions was lower in tyrosinemic than in non-tyrosinemic patients. Less than 10 % of tyrosinemic patients had foci of hepatocellular carcinom a at the time of transplantation. For this reason, and while most pati ents with tyrosinemia will eventually require liver transplantation, o ur results do not support systematic early transplantation.