Pfeiffer G, Winkler G, Neunzig P, Wolf W, Thayssen G, Kunze K
Neurologische Klinik, Universitätskrankenhaus Hamburg, Germany.
Intensive Care Med. 1996 Dec;22(12):1406-9. doi: 10.1007/BF01709559.
To describe how patients cope with the proposal of treatment with intermittent artificial ventilation after acute respiratory failure due to progressive respiratory muscle weakness.
Case series, follow-up study.
Neurological intensive care unit (ICU).
7 consecutive patients with metabolic myopathy treated for acute respiratory failure between 1983 and 1992.
Intermittent positive pressure ventilation (IPPV) via tracheostomy.
Symptoms of chronic hypoventilation preceded acute respiratory failure for months. With one exception, patients were mainly disabled from respiratory muscle weakness and sleep-related breathing disorders. IPPV was recommended to prevent recurrent respiratory failure. Two of three patients who accepted home IPPV returned to full-time jobs. One patient, who decided against IPPV, died from CO2 narcosis several months after discharge. All patients adhered to the respiratory regimen once instituted.
Acute respiratory failure in chronic myopathy is heralded by daytime drowsiness. IPPV, or at least regular monitoring of waking and sleeping partial pressure of carbon dioxide, is highly recommended even if weaning is successful. IPPV improved quality of life. The treatment strategy at discharge from the ICU should be optimal, as patients are reluctant to modify regimens.
描述因进行性呼吸肌无力导致急性呼吸衰竭的患者如何应对间歇性人工通气治疗方案。
病例系列随访研究。
神经重症监护病房（ICU）。
1983年至1992年间连续7例因急性呼吸衰竭接受治疗的代谢性肌病患者。
通过气管切开进行间歇性正压通气（IPPV）。
慢性通气不足症状在急性呼吸衰竭前数月出现。除1例外，患者主要因呼吸肌无力和睡眠相关呼吸障碍而致残。建议采用IPPV以预防呼吸衰竭复发。接受家庭IPPV治疗的3例患者中有2例恢复了全职工作。1例决定不接受IPPV的患者在出院数月后死于二氧化碳麻醉。所有患者一旦开始执行呼吸方案，均严格遵守。
慢性肌病患者出现急性呼吸衰竭的先兆是日间嗜睡。即使撤机成功，也强烈建议采用IPPV，或至少定期监测清醒和睡眠时的二氧化碳分压。IPPV改善了生活质量。由于患者不愿改变治疗方案，ICU出院时的治疗策略应达到最佳。
