BAG3基因突变致肌原纤维肌病患者机械通气脱机困难的肺康复护理

doi:10.3761/j.issn.2096-7446.2026.08.016

中华急危重症护理杂志 ›› 2026, Vol. 7 ›› Issue (8): 982-985.doi: 10.3761/j.issn.2096-7446.2026.08.016

BAG3基因突变致肌原纤维肌病患者机械通气脱机困难的肺康复护理

马燕欣¹(), 王国英¹, 虎于丁¹, 刘会玲²^,^*()

¹ 河北医科大学第二医院重症医学科石家庄市 050000
² 河北医科大学第二医院护理部石家庄市 050000

收稿日期:2025-08-14 出版日期:2026-08-10 发布日期:2026-08-04
通讯作者: *刘会玲，E-mail：26900555@hebmu.edu.cm
作者简介:马燕欣：女，硕士，主管护师，E-mail：249177576@qq.com
作者贡献声明
马燕欣、虎于丁：康复方案实施、数据收集、循证分析、论文撰写；王国英：论文修改、护理工作指导；刘会玲：论文指导、护理工作指导

Pulmonary rehabilitation nursing for a patient with myofibrillar myopathy caused by a BAG3 gene mutation who had difficulty weaning from mechanical ventilation

MA Yanxin¹(), WANG Guoying¹, HU Yuding¹, LIU Huiling²^,^*()

¹ Intensive Care Unit， the Second Hospital of Hebei Medical University， Shijiazhuang 050000， China
² Nursing Department， the Second Hospital of Hebei Medical University， Shijiazhuang 050000， China

Received:2025-08-14 Online:2026-08-10 Published:2026-08-04
Contact: *LIU Huiling，E-mail：26900555@hebmu.edu.cm

摘要/Abstract

摘要：

总结1例BAG3基因突变致肌原纤维肌病患者机械通气脱机困难的肺康复护理体会。针对该罕见病所致肌无力及呼吸肌受累，以及患者焦虑抑郁问题，成立多学科团队，制订并实施四级运动方案、分阶段脱机训练、超声指导下的精准化膈肌锻炼，并融入积极心理学理论进行心理干预。经过33 d系统化肺康复护理，患者成功脱机并顺利出院。

关键词: 肌原纤维肌病, 机械通气, 肺康复, 脱机困难, 危重病护理

Abstract:

To summarize the experience of pulmonary rehabilitation nursing for a patient with myofibrillar myopathy caused by BAG3 gene mutation who had difficulty in weaning from mechanical ventilation. In response to the muscle weakness and respiratory muscle involvement caused by this rare disease，as well as the patient’s anxiety and depression issues，a multidisciplinary team was established to develop and implement an early activity plan centered on four-level movement，staged weaning training，personalized diaphragm exercise under ultrasound guidance，and psychological intervention incorporating positive psychology theories. After 33 days of systematic pulmonary rehabilitation nursing，the patient successfully weaned off mechanical ventilation and was discharged smoothly.

Key words: Myofibrillar Myopathy, Mechanical Ventilation, Pulmonary Rehabilitation, Early Mobilization, Critical Care Nursing

马燕欣, 王国英, 虎于丁, 刘会玲. BAG3基因突变致肌原纤维肌病患者机械通气脱机困难的肺康复护理[J]. 中华急危重症护理杂志, 2026, 7(8): 982-985.

MA Yanxin, WANG Guoying, HU Yuding, LIU Huiling. Pulmonary rehabilitation nursing for a patient with myofibrillar myopathy caused by a BAG3 gene mutation who had difficulty weaning from mechanical ventilation[J]. Chinese Journal of Emergency and Critical Care Nursing, 2026, 7(8): 982-985.

参考文献 11

[1]	陆玉玲, 罗月贝, 杨欢. 肌原纤维肌病研究进展[J]. 中华神经科杂志, 2018, 51(2):143-150.
	Lu YL, Luo YB, Yang H, et al. Advances in myofibrillar myo-pathies[J]. Chin J Neurol, 2018, 51(2):143-150.
[2]	李清浩. 重症医学科中脱机困难患者危险因素分析及预测模型的建立[D]. 石家庄: 河北医科大学, 2024.
	Li Q. Analysis of risk factors and establishment of predictive models for patients with difficult weaning in intensive care unit[D]. Shijiazhuang: Hebei medical university, 2024.
[3]	吴淼, 倪朝民, 吴鸣, 等. 机械通气患者早期肺康复治疗的临床观察[J]. 中国康复医学杂志, 2018, 33(7):806-811.
	Wu M, Ni C/ZM, Wu M, et al. Clinical research of early pulmonary rehabilitation in patients with mechanical ventila-tion[J]. Chin J Rehabil Med, 2018, 33(7):806-811.
[4]	卞红, 俞萍, 孙芹, 等. 渐进式康复操在ICU机械通气患者中的应用研究[J]. 中华急危重症护理杂志, 2023, 4(8):689-694.
	Bing H, Yu P, Sun Q, et al. Application of progressive rehabi-litation exercise in patients with mechanical ventilation in ICU[J]. Chin J Emerg Crit Care Nurs, 2023, 4(8):689-694.
[5]	孙瑞祥, 江海娇, 汪君, 等. 机械通气ICU获得性衰弱患者早期运动康复的最佳证据总结[J]. 中华危重病急救医学, 2024, 36(7):745-752.
	Sun RX, Wang HJ, Wang J, et al. Summary of the best evi-dence for early exercise rehabilitation in patients with mecha-nically ventilated ICU-acquired weakness[J]. Chin Crit Care Med, 2024, 36(7):745-752.
[6]	Clini EM, Crisafulli E, Antoni FD, et al. Functional recovery fol-lowing physical training in tracheotomized and chronically ventilated patients[J]. Respir Care, 2011, 56(3):306-313. doi: 10.4187/respcare.00956
[7]	Hodgson CL, Stiller K, Needham DM, et al. Expert consensus and recommendations on safety criteria for active mobilization of mechanically ventilated critically ill adults[J]. Crit Care, 2014, 18(6):658. doi: 10.1186/s13054-014-0658-y
[8]	吴月红, 梁红霞, 席芳, 等. 体外膈肌起搏预防无创机械通气患者膈肌功能障碍的效果研究[J]. 中华护理杂志, 2022, 57(9):1029-1034. doi: 10.3761/j.issn.0254-1769.2022.09.001
	Wu YH, Liang HX, Xi F, et al. Effect of external diaphragm pacer on prevention of ventilator-induced diaphragmatic dys-function in patients with ICU non-invasive ventilation[J]. Chin J Nurs, 2022, 57(9):1029-1034.
[9]	郑筱卓. 有创—无创双模通气促进呼吸机相关膈肌功能障碍膈肌康复及机制研究[D]. 重庆: 重庆医科大学, 2024.
	Zheng XZ. Invasive and non-invasive dual-mode ventilation promotes diaphragmatic rehabilitation of ventilator-induced dia-phragmatic dysfunction and its mechanism[D]. Chongqing: Chong-qing medical university, 2024.
[10]	吉莉, 吴慧, 景晨阳, 等. ICU患者呼吸机相关性膈肌功能障碍管理的证据总结[J]. 中华急危重症护理杂志, 2025(1):100-106. doi: 10.3761/j.issn.2096-7446.2025.01.017
	Ji L, Wu H, Jing CY, et al. Evidence summary of management of ventilator-associated diaphragm dysfunction in ICU patients[J]. Chin J Emerg Crit Care Nurs, 2025(1):100-106.
[11]	刘秀, 刘滨滨, 权明桃, 等. ICU机械通气患者膈肌锻炼方案的构建及应用研究[J]. 中华护理杂志, 2023, 58(3):261-267. doi: 10.3761/j.issn.0254-1769.2023.03.001
	Liu X, Liu BB, Quan MT, et al. Construction and application of a diaphragm exercise program in patients with mechanical ventilation in ICU[J]. Chin J Nurs, 2023, 58(3):261-267. doi: 10.3761/j.issn.0254-1769.2023.03.001

[1]	胥喆, 徐红霞, 封凤, 谢睿, 王飞霞. 右上肢PICC置管患者孤立性永存左上腔静脉的识别及风险管理[J]. 中华急危重症护理杂志, 2026, 7(8): 976-978.
[2]	杨莉, 徐晓滨, 王华芬. 功能性近红外光谱在急危重症患者护理中的应用进展[J]. 中华急危重症护理杂志, 2026, 7(7): 836-840.
[3]	柳小婷, 王萍, 徐亦虹, 范婷婷, 付真真, 熊子菡, 何蓓蕾, 陈晓晖, 章晓明, 舒妍. 巨结肠类缘病术后并发超短肠综合征伴多造口患者的护理[J]. 中华急危重症护理杂志, 2026, 7(7): 849-851.
[4]	邵丽红, 李春艳, 董晓琪, 王巧琳, 叶国凤, 赵巧梅. 口腔多原发癌患者游离皮瓣移植术后多重严重并发症的护理[J]. 中华急危重症护理杂志, 2026, 7(7): 852-855.
[5]	张慧, 何雪花, 王莉, 宫晓艳, 庄一渝. 边缘供心心脏移植术后患者原发性移植物功能障碍的早期预警与护理[J]. 中华急危重症护理杂志, 2026, 7(6): 682-684.
[6]	曾妃. 家族性肥厚型心肌病合并肝功能衰竭患者行心肝同期联合移植的护理[J]. 中华急危重症护理杂志, 2026, 7(6): 685-688.
[7]	羊木英, 范文琪, 胡汉. 维持性血液透析患者透析中脑出血的早期识别与急救护理[J]. 中华急危重症护理杂志, 2026, 7(6): 704-706.
[8]	庄佳妮, 李小珍, 史超强, 王英, 谭丽萍. 颈深部感染致纵隔脓肿患者的围手术期护理[J]. 中华急危重症护理杂志, 2026, 7(6): 707-709.
[9]	李改乐, 潘文彦, 李菁菁, 刘霄, 唐颖嘉, 侯进. 肝移植患者术后并发耶氏肺孢子菌与巨细胞病毒感染的护理[J]. 中华急危重症护理杂志, 2026, 7(6): 710-713.
[10]	王燕, 卢芳燕, 刘元, 王芳. 进行性家族性肝内胆汁淤积症患儿行亲体肝移植的术后护理[J]. 中华急危重症护理杂志, 2026, 7(6): 714-716.
[11]	梅高鹏, 刘军, 杨建兰. 纽卡斯尔喉部高敏感性问卷的汉化及信效度检验[J]. 中华急危重症护理杂志, 2026, 7(6): 717-724.
[12]	蔡莎莎, 帅琴燕, 何杨梦圆, 张美, 项新月, 张明月, 黄丽华. 脓毒症患者机械通气管理的最佳证据总结[J]. 中华急危重症护理杂志, 2026, 7(6): 740-747.
[13]	李常鑫, 陈映雪, 罗陈娟, 郑旺敏, 林如玉, 陈巧玲. 腹式呼吸联合抗阻运动方案对预防机械通气患者谵妄的效果研究[J]. 中华急危重症护理杂志, 2026, 7(5): 524-531.
[14]	王淑君, 鲁虹言, 杨林娜. 《成人吸入性损伤气管切开术后气道内吸引技术》团体标准解读[J]. 中华急危重症护理杂志, 2026, 7(5): 569-573.
[15]	董莲莲, 罗雪莉, 魏文静, 陈玲欣, 任红艳, 宫晓艳. 硫胺素缺乏致湿性脚气病伴多器官功能损害患者的护理[J]. 中华急危重症护理杂志, 2026, 7(5): 591-594.

BAG3基因突变致肌原纤维肌病患者机械通气脱机困难的肺康复护理

Pulmonary rehabilitation nursing for a patient with myofibrillar myopathy caused by a BAG3 gene mutation who had difficulty weaning from mechanical ventilation

RichHTML

PDF下载

可视化

摘要/Abstract

引用本文

使用本文

参考文献 11

相关文章 15

编辑推荐

Metrics

本文评价