Objective To investigate the effect of living donor liver transplantation plus domino auxiliary liver transplantation in the treatment of metabolic liver disease in children. Methods The first patient with ornithine aminotransferase deficiency（OTCD）received living donor liver transplantation（left lateral liver）and the patient's right lobe was procured for domino auxiliary donor liver transplantation. At the same time，the recipient of domino auxiliary liver transplantation was type Ⅰ crigler-najjar syndrome. The right half of the liver with the middle hepatic vein was resected，The domino liver was retained for vascular and biliary reconstruction. Results Recipie nt who received living donor liver transplantation had normal and sustained liver function and normal blood ammonia， the patient who received domino auxiliary liver transplantation had normal bilirubin and blood ammonia，Abdominal CT examination followed up one year after the transplantation was normal. Conclusion Non-sclerosing metabolic liver diseases can be treated by liver transplantation, and their livers can be used as domino donor livers，this type of liver graft can be successfully applied to auxiliary liver transplantation of different metabolic liver diseases，thus it provids new ideas for patients with metabolic liver diseases to expand the source of donor livers.

目的 探讨亲体肝移植加多米诺辅助肝移植治疗儿童代谢性肝病的疗效。方法 多米诺供肝 受者为鸟氨酸氨甲酰基转移酶缺乏（ornithine aminotransferase deficiency，OTCD）患者，接受亲属活体肝移 植（左外叶供肝），切取的右半肝病肝用于多米诺辅助供肝肝移植。同期接受多米诺辅助供肝肝移植受者为 Ⅰ型 Crigler-Najjar 综合征，其切除带肝中静脉的右半肝，留取左半肝。多米诺供肝进行修整后进行血管及 胆道重建。结果 接受亲体肝移植的受者肝功能恢复正常且持续稳定，血氨恢复正常及接受多米诺辅助肝 移植受者胆红素及血氨恢复正常。术后 1 年随访腹部 CT 检查提示多米诺辅助肝移植受者肝脏形态及血流未 见异常。结论 对于非硬化性代谢性肝脏疾病可通过肝移植治疗，其病肝可以作为多米诺供肝，并可成功 应用于不同代谢肝脏疾病的辅助肝移植，从而为代谢性肝脏疾病患者扩大供肝来源提供新的思路。