LIVING-RELATED LIVER TRANSPLANTATION FOR TYPE II CITRULLINEMIA : THE FIRST SUCCESSFUL CASE IN GUNMA PREFECTURE

Abstract

The first case of living-related liver transplantation (LRLT) in Gunma Prefecture was successfully performed. The recipient was a 16-year-old man with Type II citrullinemia in a stable condition who had recovered from fulminant hyperammonemia and encephalopathy and was being well-managed with protein restriction and phenylacetate administration. His father was finally selected as the donor because he was ABO/HLA compatible, negative for lymphocyte crossmatch, and genetically proven heterozygote, and an optimal graft-to-recipient weight ratio (GRWR ; 0.95%) was estimated with a low rate of hepatic resection (29%) for extended left hepatic lobe graft. The extended left lobe graft with the middle hepatic vein was uneventfully obtained from the donor without hepatic vascular occlusion. The actual graft weight was 370 g and GRWR was 0.86%. After taking out the recipient's whole liver and constructing a temporary portosystemic shunt between the right portal branch and the suprarenal inferior vena cava, the graft was transplanted using the piggyback method. The hepatic artery was reconstructed using a microsurgical technique. Cold and warm ischemic times were 82 and 41 minutes, respectively. The donor was discharged 10 days after surgery, and resumed his job. The recipient was discharged 50 days after transplantation, and is doing well.