SURGICAL AND METABOLIC ASPECTS OF LIVER-TRANSPLANTATION FOR TYROSINEMIA

Tyrosinemia is the diagnosis of a very small percentage of patients un dergoing liver transplantation worldwide. Tyrosinemia is endemic withi n our referral area however, and fully one-third of the liver transpla ntations at our institution are done for this disease. Since 1986, 15 patients with tyrosinemia (TYR) and 31 patients with various other ind ications (non-TYR) have undergone a total of 51 liver transplantations . The 36-month actuarial survival for TYR patients is 87%, compared wi th 74% for non-TYR patients. Liver transplantation for hereditary tyro sinemia and other metabolic disorders without portal hypertension or p revious portohepatic operations is notably easier to perform. Intraope rative blood loss was less, length of hospital stay was shorter, and i ncidence 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 result s do not support systematic early transplantation before the age of tw o years.