National Center for Child Health and Development
Two cases of hepatocyte transplantation for urea cycle disorders by using hepatocytes isolated from living donor reduced grafts
Takanobu Shigeta1, Shin Enosawa2, Yoshihiro Hirata1, Kengo Sasaki1, Hajime Uchida1, Hiroyuki Kanazawa1, Akinari Fukuda1, Kazuaki Nakamura3, Reiko Horikawa4, Shunsuke Nosaka5, Mureo Kasahara1.
1Transplantation Center, National Center for Child Health and Development, Tokyo, Japan; 2Advanced Medical Sciences, National Research Institute for Child Health and Development, Tokyo, Japan; 3Pharmacology, National Research Institute for Child Health and Development, Tokyo, Japan; 4Endocrinology and Metabolism, National Center for Child Health and Development, Tokyo, Japan; 5Radiology, National Center for Child Health and Development, Tokyo, Japan
Background: Hepatocyte transplantation (HT) has been indicated in patients with metabolic liver diseases and acute liver failure as a bridge or an alternative to liver transplantation. However, one of the problems of HT is the limited availability of donor tissue. In our center, hepatocytres were obtained from remnant tissue of left lateral segment graft from unrelated donors. Reduction of left lateral segment graft was performed when graft to recipient weight ratio was over 4%. The aim of this study was to report the experience of HT using cryopreserved hepatocytes from resected living donor liver tissue
Materials and Methods: In our center, we experienced two cases of HT using hepatocytes from living donor, isolated from the remnant liver of reduced left lateral segment graft. One case was neonatal ornithine transcarbamylase deficiency (OTCD) and another was neonatal carbamoyl phosphate synthetase 1 deficiency (CPS1D). The age at HT was at 11, 14 days of age in OTCD case, and 18, 20 and 22 days of age in CPS1D case, respectively. Double lumen catheter was inserted into left portal vein via umbilical vein. Portal vein pressure was monitored simultaneously during infusion. Tacrolimus and low dose of steroid were initiated as immunosuppression therapy after HT.
Results: HT was performed successfully in both cases. In OTCD case, total counts and mean viability of transplanted hepatocytes were 7.01 ×107 cells and 85.9%. In CPS1D case, total counts and mean viability of transplanted hepatocytes were 1.76 × 108 cells and 64.6%. Administration of hepatocytes was discontinued for about 5 minutes when portal vein pressure showed above 20 mmHg. After reduction of portal vein pressure, administration of hepatocytes was continued. Although both cases experienced hyperammonemia episode after HT and required continuous hemodialysis and filtration, severe neurological impairment did not presented. Both patients underwent living donor liver transplantation 5 months after HT. Postoperative courses were uneventful. No neurological sequelae related to hyperammonemia have been observed.
Conclusion: HT using the remnant liver of reduced grafts from living donor, a novel source of hepatocytes, was safe and useful.
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|Hepatocyte Transplantation||Two cases of hepatocyte transplantation for urea cycle disorders by using hepatocytes isolated from living donor reduced grafts||Room 111-112|