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

Abstract

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

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.

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

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