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
收稿日期: 2025-08-14
网络出版日期: 2026-08-04
Pulmonary rehabilitation nursing for a patient with myofibrillar myopathy caused by a BAG3 gene mutation who had difficulty weaning from mechanical ventilation
Received date: 2025-08-14
Online published: 2026-08-04
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 . DOI: 10.3761/j.issn.2096-7446.2026.08.016
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.
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