| Theme |
Debatable Issues in Living-Donor Liver Transplantation |
| Title |
Living Donor Liver Transplantation for Congenital Liver Disease |
| Author |
Naoki Kawagishi |
Division of Advanced Surgical Science and Technology, Graduate School of Medicine, Tohoku University |
| Author |
Kazushige Satoh |
Division of Advanced Surgical Science and Technology, Graduate School of Medicine, Tohoku University |
| Author |
Yoshitaka Enomoto |
Division of Advanced Surgical Science and Technology, Graduate School of Medicine, Tohoku University |
| Author |
Yorihiro Akamatsu |
Division of Advanced Surgical Science and Technology, Graduate School of Medicine, Tohoku University |
| Author |
Satoshi Sekiguchi |
Division of Advanced Surgical Science and Technology, Graduate School of Medicine, Tohoku University |
| Author |
Susumu Satomi |
Division of Advanced Surgical Science and Technology, Graduate School of Medicine, Tohoku University |
| [ Summary ] |
Congenital liver diseases, which are indicated for liver transplantation are represented as cholestatic and metabolic diseases. There are many liver diseases and we must treat them in a variety of ways. Particularly metabolic diseases often have genetic back ground, we must also check the genetic status of the donor before transplantation. In this article we describe congenital liver diseases for which we have performed living donor liver transplantation (LDLT) in Japan. Biliary atresia is the most frequent disease requiring LDLT and the number of these cases treated with LDLT in Japan totals 1,000. The most frequent indications for LDLT were failed Kasai operations and developmental retardation. However, the indications became wider when considering the quality of daily life for the patient. Other diseases such as Wilson's disease, familial amyloid polyneuropathy and citrullinemia are described. We also describe our experiences with two homozygous for familial hypercholesterolemia (FH) who received LDLT from their parents, who were heterozygous for FH. |