摘要目的 探讨需长期气管切开.机械通气治疗(TMV)的肌萎缩侧索硬化症(ALS)患者,使用无创双水平正压(BiPAP)呼吸机替代有创呼吸机进行长期家庭治疗的可行性.方法 选择2002年1月至2008年3月在我院进行TMV的ALS患者16例,其中男12例,女4例,年龄25～75岁,平均59岁.在经抗感染等综合治疗后病情稳定,将BiPAP呼吸机的呼吸管道经平台呼气阀和转接管直接与气管切开导管连接(命名为"向氏"连接法),进行机械通气并持续家庭应用.观察使用有创呼吸机时、改用无创呼吸机后及出院前的动脉血气;患者出院前,必须对其家属进行尤创呼吸机应用相关知识的培训;随访患者无创呼吸机的使用情况、患者耐受情况和生存率.采用单因素方差分析进行统计学分析.结果 使用无创呼吸机前及使用无创呼吸机后2 h、1 d及患者出院前的PaCO2分别为(36±10)、(42±10)、(41±10)及(42±11)mm Hg(1 mm Hg=0.133 kPa),PaO2分别为(84±11)、(80±12)、(87±14)及(86±12)mm Hg,SaO2分别为(96.7±1.3)%、(96.5±0.8)%、(96.8 ±1.2)%及(96.5±1.0)%,各时间段差异均无统计学意义(F值分别为1.21、0.59、0.97、0.41,均P>0.05);患者均无明显不适主诉,未发现有不能耐受无创呼吸机的现象,也未发生呼吸机故障等问题.截至2008年7月31日随访结束,16例患者中除1例在治疗13个月后猝死外,其余15例存活至今,使用无创呼吸机的平均时间为39个月(4～66个月).结论 对于需长期TMV的ALS患者,临床情况稳定后采用无创BiPAP呼吸机连接气管切开导管进行家庭机械通气可能是一种安全有效的方法.

abstractsObjective To study the feasibility of the bilevel positive airway pressure ( BiPAP) non- invasive ventilator used in home mechanical ventilation for long-term tracheostomy-mechanical ventilation (TMV) in patients with amyotrophic lateral sclerosis (ALS). Methods Sixteen patients(12 men and 4 women,mean age 59 years) with ALS were selected for this study at Respiratory Department of the Shougang Hospital, Peking University from January 2002 to March 2008. After the disease had been controlled by anti-infective therapy and comprehensive treatment,the patients received TMV,through the improved ( "Xiang's" connection) non-invasive BiPAP ventilator connected with tracheotomy tube,and on-going home mechanical ventilation ( HMV). The blood gas was evaluated during invasive ventilation and non-invasive ventilation before discharge . Family members of the patients were trained for the use of non-invasive ventilators. The use of ventilators and the patients' condition were regularly followed and the survival rate calculated. Statistical analysis was carried out by using one-way ANOVA. Results There was no statistical difference in the blood gas before the use of non-invasive ventilator,2 h and 1 d after the use of non-invasive ventilator, and before discharge, PaCO2[ (36 ± 10) ,(42 ± 11) , (41 ± 10) , (42 ± 11) mm Hg( 1 mm Hg =0.133 kPa) ] , PaO2 [(84 ±11), (81 ± 12), (87 ±14), (86 ±12) mm Hg], SaO2[(96.7 ± 1. 3 )% , (96. 5 ± 0. 8)% , (96.8±1.2)%,(96.5 ±1.0)%] respectively,( F = 1.21, 0.59, 0.97, 0. 41, respectively, all P> 0.05). All patients had no complaint of uncomfortable use, no intolerance to ventilators, and no ventilator breakdown. Fifteen patients were alive at the end of the follow-up ( July 31 ,2008). The mean time of using non-invasive ventilator was 39 months ( range 4 to 66 months). Conclusion For ALS patients who need long-term ventilation support, the use of BiPAP non-invasive ventilators is a safe and effective alternative for invasive ventilators.