[Current status of and issues in pediatric liver transplantation in Japan].

Approximately 140 pediatric living donor liver transplantations (PLDLTs) are performed annually in Japan. The most common indication for PLDLT is biliary atresia. The 5-year patient survival rate after PLDLT has reached 84% with advances in surgical techniques, immunosuppressive therapy, and antiinfection therapy in the past two decades. Recently, although the number of PLDLTs performed for the treatment of fulminant hepatic failure and metabolic disease has increased, the optimal timing of surgery for each disease has led to better patient prognosis. However, patient survival rates after fulminant hepatic failure of unknown origin and hepatoblastoma are still poor in comparison with other indications. A multidisciplinary treatment regimen including postoperative medical therapy therefore needs to be established for these conditions. In the outpatient clinic, regular and continuing imaging examination is indispensable for the diagnosis of mid/long-term complications such as vascular anastomotic stenosis and biliary stricture in addition to standard liver function tests. These complications can be treated with interventional radiology if diagnosed in the early stage. Protocol biopsy is also important to confirm graft fibrosis over the long term after PLDLT. Patients with graft fibrosis should receive more potent immunosuppressants to maintain permanent graft function.