何凌霄　梁燕

[摘要] 肌萎缩侧索硬化（ALS）患者常存在呼吸肌无力，因肺部感染诱发急性呼吸衰竭和呼吸肌麻痹是ALS患者最常见的死因，对ALS患者进行定期呼吸评估和及时的入院评估尤为重要。本文回顾1例ALS合并急性Ⅱ型呼吸衰竭患者的呼吸支持过程，该患者入院后血气分析提示Ⅱ型呼吸衰竭，基于当前指南建议，根据患者呼吸状态、血气分析结果及个人意愿，先后行无创通气、气管插管辅助通气及入神经科ICU行气管切开，同时给予充分营养支持、抗生素治疗感染、祛痰等对症治疗护理，患者恢复良好；总结呼吸评估及支持经验，认为在入院评估后对符合辅助通气指征的患者，根据病情及个人意愿选择合适的呼吸支持措施并定期调整，有助于患者呼吸功能的改善。

[关键词] 肌萎缩侧索硬化；呼吸衰竭；呼吸支持；机械通气

[中图分类号] R473.74 [文献标识码] A [文章编号] 1673-7210（2018）04（b）-0169-04

[Abstract] ALS patients always occure respiratory muscle weakness， acute respiratory failure and respiratory muscle paralysis caused by pulmonary infection are the most common cause of death in ALS patients. It is very important for ALS patients to be evaluated regularly and timely hospitalized assessment. This article reviews the respiratory support process of an ALS patients with acute type Ⅱ respiratory failure. After the patient admitted to hospital， the blood gas analysis suggested that type Ⅱ respiratory failure， according to the patient's breathing state， blood gas analysis results and personal wishes， he was treated with non-invasive ventilation， tracheal intubation assisted ventilation and tracheotomy after transferred into the neurological ICU by sequence， and given adequate nutritional support， Antibiotic treatment of infection， expectorant and other symptomatic treatment care at the same time. After the systematic treatment， the patient's condition became stable and transferred to long-term carefacility. Generally， in patients with auxiliary ventilation indications， choosing the appropriate respiratory support measures and regular adjustments according to the disease and personal wishes can help improve the patients' respiratory function.

[Key words] Amyotrophic lateral sclerosis； Respiratory failure； Respiratory support； Mechanical ventilation

肌萎縮侧索硬化（Amyotrophic Lateral Sclerosis，ALS）是一种以运动神经元凋亡为特征的进展性、致死性神经变性疾病，绝大多数患者在症状出现2～5年内即死亡；ALS患者的主要特征性表现为肌萎缩、肌无力和锥体束征，同时可伴延髓麻痹、膀胱功能障碍、焦虑抑郁等，由于呼吸肌的进行性萎缩和延髓麻痹所致咳嗽无力、误吸，ALS患者合并呼吸衰竭常见，对患者生存具有极大不利影响；早期监测及识别患者呼吸衰竭迹象，并给予针对性干预，对预防和改善患者急性呼吸衰竭发作，减少院内死亡率方面有重要意义。笔者总结1例典……