Respiratory Muscle Rest Therapy Restores Pulmonary Function in Progressive Congenital Myopathy - A Case Report

呼吸肌休息維持漸進性肌肉病變患者之肺功能－病例報告

方曉荷(Hsiao-Ho Fang) ； 王亭貴(Tyng-Guey Wang) ； 陳翠雯(Tze-Wen Chen) ； 連倚南(I-Nan Lien)

《慈濟醫學雜誌》 14卷3期 (2002/06) Pp. 181-186

https://doi.org/10.6440/TZUCMJ.200206.0181

摘要

利用呼吸肌休息的方式來治療慢性肺疾患者已行之有年，然而其治療的效果仍有爭論。本研究報告一名nemaline肌肉病變的患者，因反覆呼吸道感染導致呼吸衰竭而接受非侵入性呼吸器的治療。在急性感染恢復後，病人於夜間利用非侵入性呼吸器於睡眠時休息其呼吸肌，在三年的追蹤期中，患者明顯地較少因呼吸功能障礙住院，血中氣體分壓也維持在較正常的值，甚至肺功能檢查及呼吸肌耐力都比發病初進步。我們認為呼吸肌休息的治對進行性肌肉病變患者除了改善其血液中氣體分壓外，可能也可以協助肺功能的恢復或進步。

關鍵字

呼吸肌休息治療 ； 先天性肌肉病變 ； 胸腔復健 ； 肺功能

並列摘要

The respiratory muscle rest program has been widely used in cases of chronic pulmonary dysfunction. However, the results were quite controversial in various studies. Recently we treated a 33-year-old man with nemaline myopathy who presented with recurrent respiratory tract infection and respiratory failure. He was treated initially with continuous mechanical ventilation, but a few weeks later, he only needed noninvasive ventilator support at night. During 3 years of follow up, he was advised to use a nocturnal ventilator to rest his respiratory muscles during sleep. Fewer hospitalizations and normalized blood gas values were noted. In addition, he had improved lung volume and respiratory muscle endurance. It is postulated that respiratory muscle rest therapy might be indicated and of benefit for patients who have weak respiratory muscles due to congenital myopathy.

並列關鍵字

respiratory muscle rest ； nemaline myopathy ； pulmonary rehabilitation ； lung function