Mureo Kasahara

Living-related liver transplantation for type II citrullinemia using a graft from heterozygote donor.

BACKGROUND Type II citrullinemia (CTLN2) characterized by a liver-specific argininosuccinate synthetase deficiency is an adult onset genetical disorder caused by the mutation of SLC25A13 gene, which results in fulminant hyperammonemia often with poor prognosis. METHODS A 16-year-old Japanese boy presented fulminant hyperammonemia and encephalopathy and recovered after aggressive medical treatment. The patient was diagnosed as CTLN2 by plasma amino acid pattern and detection of the mutated SLC25A13 gene. We performed living-related liver transplantation (LRLT) using a graft from the genetically proven heterozygote father. RESULTS Serum amino acid concentration was normalized within a day after transplantation without protein restriction and medication. The patients postoperative course was natural. The patient is back in school 6 months after surgery. CONCLUSIONS Living-related liver transplantation using a graft from genetically proven heterozygote donors might be a permissible treatment modality for CTLN2. Long-term observation may be necessary to make a definite conclusion possible.

Posttransplant Bilioportal Fistula With Portal Vein Thrombosis: A Case Report

An 8-year-old female patient, known to have post-Kasai biliary atresia with mild intrapulmonary shunting, underwent living donor liver transplantation because of recurrent cholangitis. After the treatment of postoperative biliary stricture with percutaneous transhepatic biliary drainage, the patient subsequently developed hematochezia with portal vein thrombosis. The intraoperative findings showed portal vein thrombosis with a bilioportal fistula. We performed closure of the bilioportal fistula and reconstruction of the portal vein with a native internal jugular vein interposition graft. A bilioportal fistula due to percutaneous hepatobiliary procedures is a reportedly a rare complication following liver transplantation. The patient is currently doing well after a successful surgical intervention.

Usefulness of Continuous Machine Perfusion Preservation with Rewarming Method for Donation after Cardiac Death Liver Graft: 1308

Shigeta T.1,2, Matsuno N.1, Obara H.3, Mizunuma H.3, Hirano T.4, Tanaka H.1, Kanazawa H.1, Sakamoto S.1, Kasahara M.1, Uemoto S.2, Enosawa S.1 1National Center for Child Health and Development, Tokyo, Japan, 2Kyoto University Graduate School of Medicine, Division of HepatoBiliary-Pancreatic and Transplant Surgery, Kyoto, Japan, 3Tokyo Metropolitan University, Mechanical Engineering, Tokyo, Japan, 4Tokyo University of Pharmacy and Life Sciences, Department of Clinical Pharmacology, School of Pharmacy, Tokyo, Japan