1例糖原累积综合征Ⅲa型患者行活体肝脏移植的围手术期护理

doi:10.3761/j.issn.0254-1769.2025.21.003

摘要/Abstract

摘要：

总结1例糖原累积综合征Ⅲa型患者行活体肝脏移植的围手术期护理经验。护理要点如下：强化营养支持与血糖调控，维持术前代谢稳定；评估心肌及骨骼肌功能，多维度预防跌倒；术后开展多维度精准监测调控，促进移植肝脏功能重建；进行阶梯式营养支持，维持糖代谢稳态，满足多元营养需求；开展以心功能为核心的综合评估，实施个性化分阶段康复训练；建立全程健康档案，落实随访管理。患者术后顺利出院，出院后随访3个月，恢复良好。

关键词: 肝移植, 糖原累积综合征, 肝硬化, 围手术期护理

Abstract:

This report summarizes the perioperative nursing care of a patient with glycogen storage disease（GSD） type Ⅲa who underwent living donor liver transplantation. Key nursing strategies included：enhanced nutritional support and blood glucose control to maintain metabolic stability before surgery；comprehensive preoperative assessment of myocardial and skeletal muscle function with multidimensional fall prevention measures. Postoperatively，multi-dimensional precise monitoring and management were implemented to support graft function recovery. A stepwise nutritional intervention was adopted to sustain glucose homeostasis and meet diverse nutritional needs. Cardiac function-guided rehabilitation planning enabled individualized phased rehabilitation training. A comprehensive health record was established to ensure continuity of care through structured and continuous follow-up. The patient recovered well and was discharged without complications. At 3-month follow-up，the recovery remained satisfactory.

Key words: Liver Transplantation, Glycogen Storage Disease, Liver Cirrhosis, Perioperative Nursing

苏倩, 李越, 陈红爽, 潘新伟, 冯璐晶, 吴宇, 徐雪蕾. 1例糖原累积综合征Ⅲa型患者行活体肝脏移植的围手术期护理[J]. 中华护理杂志, 2025, 60(21): 2579-2583.

SU Qian, LI Yue, CHEN Hongshuang, PAN Xinwei, FENG Lujing, WU Yu, XU Xuelei. Perioperative nursing care of a patient with glycogen storage disease type Ⅲa under-going living donor liver transplantation[J]. Chinese Journal of Nursing, 2025, 60(21): 2579-2583.

参考文献 15

[1]	Wicker C, Cano A, Decostre V, et al. French recommendations for the management of glycogen storage disease type Ⅲ[J]. Eur J Med Res, 2023, 28(1):253.
[2]	Hijazi G, Paschall A, Young SP, et al. A retrospective longitudinal study and comprehensive review of adult patients with glycogen storage disease type Ⅲ[J]. Mol Genet Metab Rep, 2021, 29:100821.
[3]	Halaby CA, Young SP, Austin S, et al. Liver fibrosis during clinical ascertainment of glycogen storage disease type Ⅲ:a need for improved and systematic monitoring[J]. Genet Med, 2019, 21(12):2686-2694.
[4]	Gümüş E, Özen H. Glycogen storage diseases:an update[J]. World J Gastroenterol, 2023, 29(25):3932-3963.
[5]	《糖原累积病中国三级预防指南》制订组. 糖原累积病中国三级预防指南[J]. 国际神经病学神经外科学杂志, 2024, 51(5):14-22.
	Working Group on Chinese Guidelines for the Primary,Secondary,and Tertiary Prevention of Glycogen Storage Disease. Chinese guidelines for the primary,secondary,and tertiary prevention of glycogen storage disease[J]. J Int Neurol Neurosurg, 2024, 51(5):14-22.
[6]	杜彩琪, 梁雁. 肝糖原贮积症的营养管理[J]. 中华实用儿科临床杂志, 2020, 35(20):1546-1548.
	Du CQ, Liang Y. The nutritional management of hepatic glycogen storage diseases[J]. Chin J Appl Clin Pediatr, 2020, 35(20):1546-1548.
[7]	中华医学会心血管病学分会, 中国医师协会心血管内科医师分会, 中国医师协会心力衰竭专业委员会, 等. 中国心力衰竭诊断和治疗指南2024[J]. 中华心血管病杂志, 2024, 52(3):235-275.
	Chinese Society of Cardiology,, Chinese College of Cardiology Physicians, Chinese Society of Heart Failure. et al. Chinese guidelines for the diagnosis and treatment of heart failure 2024[J]. Chin J Cardiol, 2024, 52(3):235-275.
[8]	中华医学会神经病学分会, 中华医学会神经病学分会神经肌肉病学组, 中华医学会神经病学分会肌电图与临床神经生理学组. 中国糖原累积性肌病诊治指南[J]. 中华神经科杂志, 2016, 49(1):8-16.
	Chinese Society of Neurology, Neuromuscular Disease Group of Chinese Society of Neurology, Electromyography and Clinical Neurophysiology Group of Chinese Society of Neurology. Guidelines for the diagnosis and treatment of glycogen storage myopathy in China[J]. Chin J Neurol, 2016, 49(1):8-16.
[9]	孙慧慧, 张丙良, 娄连玉, 等. 肝移植患者术前预康复方案的构建[J]. 中华护理杂志, 2023, 58(18):2195-2202. DOI
	Sun HH, Zhang BL, Lou LY, et al. Construction of a preoperative prehabilitation program for liver transplant recipients[J]. Chin J Nurs, 2023, 58(18):2195-2202. DOI
[10]	Masuda Y, Yoshizawa K, Ohno Y, et al. Small-for-size syndrome in liver transplantation:definition,pathophysiology and management[J]. Hepatobiliary Pancreat Dis Int, 2020, 19(4):334-341.
[11]	朱志军. 小肝综合征的防治策略与进展[J]. 中国普外基础与临床杂志, 2017, 24(8):923-926.
	Zhu ZJ. Prevention and treatment strategies and progress of small liver syndrome[J]. Chin J Bases Clin Gen Surg, 2017, 24(8):923-926.
[12]	杨龙, 张雅敏. 门静脉压力变化对肝再生影响的研究进展[J]. 中华肝胆外科杂志, 2019, 25(8):633-636.
	Yang L, Zhang YM. Research progress about the effect of portal vein pressure on liver regeneration[J]. Chin J Hepatobiliary Surg, 2019, 25(8):633-636.
[13]	毛一雷, 卢欣, 桑新亭, 等. 外科术后患者允许性摄入不足的前瞻、随机、对照临床研究[J]. 中华普通外科杂志, 2005, 20(10):612-615.
	Mao YL, Lu X, Sang XT, et al. Permissive underfeeding in post-operative patients:results of a prospective,randomized,controlled clinical trial[J]. Chin J Gen Surg, 2005, 20(10):612-615.
[14]	周惠玲, 杨志玉, 严靖雯, 等. 肝移植患者体力活动现状及影响因素研究[J]. 中华护理杂志, 2024, 59(10):1212-1218. DOI
	Zhou HL, Yang ZY, Yan JW, et al. Status and influencing factors of physical activity among liver transplant patients[J]. Chin J Nurs, 2024, 59(10):1212-1218. DOI
[15]	丁思, 韩连书. 遗传代谢病患者肝移植及预后的研究进展[J]. 中华医学杂志, 2022, 102(8):602-604.
	Ding S, Han LS. Research progress on liver transplantation and prognosis of patients with genetic metabolic diseases[J]. Natl Med J Chin, 2022, 102(8):602-604.