Müller-Pawlowski H, von Moers A, Raffenberg M, Petri M, Saalfeld S, Lode H
Pneumologie I, Lungenklinik, Heckeshorn, Berlin.
Med Klin (Munich). 1995 Apr;90(1 Suppl 1):35-8.
Patients with hereditary generalized neuromuscular diseases develop respiratory failure and decreased maximal inspiratory pressure (Pi max) due to both, involvement of respiratory muscles in the disease and secondary spine- and thorax-deformity. In recent years mechanical respiratory support was done mostly by hyperbaric pressure ventilation when the patients become hypercapnic during daytime. However, polysomnographic investigations have shown that despite normal ventilation during daytime, severe respiratory failure might be detectable only during sleep.
Fourty patients with hereditary generalized neuromuscular diseases were analyzed using polysomnographic analyses and lung function tests (4-channel-ECG,2-channel-ECG, EOG, respiratory parameters, continuous oxygen saturation measurement and capillary blood gas analyses during sleep). Patients with need of ventilatory support during sleep were treated using bilevel positive airway pressure ventilation (BiPAP).
Twenty patients revealed severe sleep related breathing disorders and were therefore treated by BiPAP. All showed normalisation or substantial improvement during BiPAP-therapy except two patients with persistent daytime symptoms. During follow-up (6 to 38 months) 3 patients died (cardiomyopathy, pulmonary embolism, pneumonia). The most important side effect of BiPAP-therapy was pressure marks due to the masks.
BiPAP is useful for treatment of sleep related respiratory failure in patients with hereditary generalized neuromuscular diseases.
遗传性全身性神经肌肉疾病患者会出现呼吸衰竭，且最大吸气压力（Pi max）降低，这是由于呼吸肌受累于该疾病以及继发的脊柱和胸廓畸形所致。近年来，当患者白天出现高碳酸血症时，机械通气支持主要采用高压通气。然而，多导睡眠图研究表明，尽管白天通气正常，但严重的呼吸衰竭可能仅在睡眠期间才被检测到。
对40例遗传性全身性神经肌肉疾病患者进行了多导睡眠图分析和肺功能测试（睡眠期间进行4通道心电图、2通道心电图、眼电图、呼吸参数、连续血氧饱和度测量和毛细血管血气分析）。对睡眠期间需要通气支持的患者采用双水平气道正压通气（BiPAP）进行治疗。
20例患者出现严重的睡眠相关呼吸障碍，因此接受了BiPAP治疗。除2例白天症状持续的患者外，所有患者在BiPAP治疗期间均恢复正常或有显著改善。在随访（6至38个月）期间，3例患者死亡（心肌病、肺栓塞、肺炎）。BiPAP治疗最重要的副作用是面罩导致的压痕。
BiPAP对治疗遗传性全身性神经肌肉疾病患者的睡眠相关呼吸衰竭有用。
