Liver Transplantation for Metabolic Liver Disease Patients: A Single-Center Experience

Abstract

Introduction: Metabolic liver disease (MLD) is life threatening. The efficacy of liver transplantation (LT) for MLD patients are well reported. There are some options of surgical procedures, but a few reports of collecting data was reported from a single-center. Here we present our experiences of LT procedures including deceased donor (DD) LT, living donor (LD) LT and auxiliary partial orthotopic liver transplantation (APOLT), and outcome in those patients. Methods: Medical records of 61 MLD adult (≧18 years old) recipients underwent LT in our institute were reviewed retrospectively. Perioperative factors were investigated. The patient survival rate was calculated. Results: The causes of LT were familial amyloid polyneuropathy in 42, adult-onset type 2 citrullinemia in 17, glycogen storage disease in 1 and Wilson's disease in 1. The median age at LT was 37.1 (range 18.8 to 58.4) years old. All DDLT recipients received whole liver graft (n=7). In all LDLT cases (n=54), the left liver grafts were used. The median volume of graft was 392g that was correspond to 38.0% of the recipient standard liver volume (range 230 (22.1) to 580g (58.8%)). In 18 cases of those, APOLT was selected because of extremely small size of graft or expectation of future gene therapy. The 10-year survival rate of MLD recipients was 86.8%. Conclusions: Even though a graft is relatively small, LT for MLD patients could be successfully achieved with APOLT. The 10-year survival rate of MLD recipient in our institute was thought to be satisfactory.