ISSN 2097-6046（网络）
ISSN 2096-7446（印刷）
CN 10-1655/R
主管：中国科学技术协会
主办：中华护理学会
中华急危重症护理杂志

中华急危重症护理杂志 ›› 2026, Vol. 7 ›› Issue (4): 459-461.doi: 10.3761/j.issn.2096-7446.2026.04.013

• 重症护理研究 • 上一篇    下一篇

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

汤文秀1(), 应燕1, 诸纪华2,*(), 葛红玲1, 周微微1   

  1. 1 浙江大学医学院附属儿童医院/儿童少年健康与疾病国家临床医学研究中心普外科肝胆中心 杭州市 310052
    2 浙江大学医学院附属儿童医院/儿童少年健康与疾病国家临床医学研究中心护理部 杭州市 310052
  • 收稿日期:2025-06-23 出版日期:2026-04-10 发布日期:2026-04-02
  • 通讯作者: 诸纪华 E-mail:twx9408@zju.edu.cn;jihuazhu@zju.edu.cn
  • 作者简介:汤文秀：女，本科（硕士在读），护师，E-mail：twx9408@zju.edu.cn

Perioperative nursing care of a child with liver-brain-type mitochondrial DNA depletion syndrome undergoing liver transplantation

TANG Wenxiu1(), YING Yan1, ZHU Jihua2,*(), GE Hongling1, ZHOU Weiwei1   

  1. 1 General Surgery Department/Hepatobiliary Surgery Center， Children’s Hospital Zhejiang University School of Medicine/National Clinical Medical Research Center for Children and Adolescents’ Health and Diseases， Hangzhou 310052， China
    2 Nursing Department， Children’s Hospital Zhejiang University School of Medicine/National Clinical Medical Research Center for Children and Adolescents’ Health and Diseases， Hangzhou 310052， China
  • Received:2025-06-23 Online:2026-04-10 Published:2026-04-02
  • Contact: ZHU Jihua E-mail:twx9408@zju.edu.cn;jihuazhu@zju.edu.cn

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摘要：

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。

关键词: 线粒体DNA耗竭综合征, 肝移植, 围手术期护理, 儿科护理学

Abstract:

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.

Key words: Mitochondrial DNA Depletion Syndrome, Liver Transplantation, Perioperative Nursing, Pediatric Nursing