总结1例肝脑型线粒体DNA耗竭综合征患儿行肝移植的围手术期护理体会。针对患儿存在能量合成异常及物质代谢障碍、术前易诱发代谢风暴、术中血流动力学不稳定及低体温风险增加、术后并发症复杂多样等问题，采取术前制订合理的禁食策略，术中加强血流动力学监测及实施低体温的预防措施，术后采用小剂量滴定法调整药物剂量、动态调整输液策略管理顽固性腹水、开展个体化随访。经精心治疗与护理，患儿于肝移植术后第38天出院，出院后门诊随访6个月，恢复良好。

To summarize the perioperative nursing experience of a child with liver-brain-type mitochondrial DNA depletion syndrome undergoing liver transplantation. Given the child’s energy synthesis abnormalities and metabolic disorders，the risk of metabolic storm before surgery，instability in hemodynamics and increased risk of hypothermia during the operation，as well as complex and diverse postoperative complications，the following measures were taken. A reasonable fasting strategy was developed preoperatively. Monitoring of hemodynamics was enhanced and hypothermia prevention measures were implemented intraoperatively. Postoperatively，a low-dose titration method for medication was adopted to accurately adjust drug doses and fluid strategy was dynamically adjusted to manage refractory ascites，and the individualized follow-up was conducted. After careful treatment and nursing，the child was discharged on the 38th day after the liver transplantation. After 6 months of outpatient follow-up，he recovered well.