Efficacies of Living donor liver transplantation for hereditary tyrosinemia type I

Objective To explore the surgical outcomes of living donor liver transplantation (LDLT) for hereditary tyrosinemia type I (HT Ⅰ). Methods From July 2013 to May 2015, 3 boys and 1 girl, diagnosed as HT Ⅰ, underwent LDLT at our center.The Child-Pugh stages were B (n=3) and C (n=1). All grafts were harvested from their parents.Their median age at LT was 4.4 years and median follow-up period was 17 months. Results All operations were successful and all four patients survived.After LDLT, the metabolism of tyrosine was restored to normal in all patients.That is, the serum levels of succinyl acetone (SA), succinyl acetoacetate (SAA), fumaryl acetoacetate (FAA) and maleyl acetoacetate (MAA) became normalized.All metabolic products of tyrosine decreased to undetected levels.Liver function and alpha-fetoprotein level were normal.Renal dysfunction prior to LDLT such as hypocalcaemia, hypophosphatemia and low serum bicarbonate returned to normal after LT and no immunosuppressant-related toxicity was observed.All of them showed catch-up growth after LDLT.All complications at 6 months after discharge, including EBV infection & acute graft rejection (n=1), chylous fistula & intractable ascites (n=1) and hand-foot-mouth infection (n=1), were cured. Conclusions As an effective treatment for HT1, LDLT yields clinical and biochemical improvements with a promising survival rate.However, long-term careful monitoring is necessary for assessing renal function post-LDLT. Key words: Tyrosinemias; Liver transplantation; Treatment effectiveness