脊髓性肌萎缩并肺炎患儿13例临床特征分析

Clinical characteristics of 13 children with spinal muscular atrophy combined with pneumonia

目的探讨脊髓性肌萎缩(SMA)合并肺炎住院患儿的临床特点及治疗方案,对比不同治疗方法所致不同结局,以提高SMA合并肺炎的诊疗水平。方法回顾性分析2015年1月至2019年6月因SMA合并肺炎于首都儿科研究所附属儿童医院呼吸内科病房住院的13例患儿临床资料。统计住院SMA患儿的年龄、分型、住院时间、呼吸衰竭类型、并发症等一般资料。描述住院期间SMA患儿的肺部感染病原及治疗情况。总结并分析住院期间及出院后SMA患儿的机械通气方式、时间及转归情况。结果8例1型患儿病情较重,多存在Ⅱ型呼吸衰竭及肺炎并发症,平均住院时间为(39.8±30.3)d,较5例2型患儿平均住院时间[(7.8±2.2)d]长(t=2.318,P=0.041)。选择气管插管及气管切开的6例SMA患儿均存在多重耐药菌感染,需长期并更换使用多种抗生素治疗,平均住院时间为(51.3±25.3)d,较4例使用无创通气患儿[(7.5±2.4)d]长,差异有统计学意义(t=3.391,P=0.009)。3例气管插管的患儿通过积极的呼吸道清理术,病情稳定后(体温正常;空气氧下经皮血氧饱和度>94%;胸片无炎症;吸痰频率少于4 h 1次)成功拔除气管插管过度至无创通气。3例出院时无需白天无创通气的患儿在完善多导睡眠监测后,结果提示存在睡眠呼吸紊乱,出院后长期使用夜间无创通气治疗,均未因重症肺炎再次住院。结论选择气管插管治疗的SMA合并肺炎的患儿,应在病情稳定时尽早拔除气管插管改为无创通气,以避免多重耐药菌感染。出院时已脱离无创通气的患儿应完善多导睡眠监测,若提示存在睡眠呼吸紊乱,出院后应长期使用夜间无创通气,以降低因重症肺部感染再次住院的风险。

Objective To investigate the clinical manifestations of hospitalized patients with spinal muscular atrophy(SMA)combined with pneumonia and compare outcomes of different plans based on the manifestations,so as to improve the diagnosis and treatment of this disease.Methods The clinical data of 13 SMA children with pneumonia hospitalized in the Department of Respiratory in Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2015 to June 2019 were retrospectively analyzed.General information including the age of patients,the classification of their diseases,the type of respiratory failure and complications was collected.For the pneumonia,the pathogens,complications,related respiratory failure type,and the treatments for SMA children during hospitalization were documented.The pathogen of pulmonary infection and treatment were described.The mode,duration,and outcome of mechanical ventilation for the SMA children during hospitalization and after discharge from the hospital were also analyzed.Results There were 8 SMA patients with type 1 suffering from more severe conditions,most of whom had type Ⅱ respiratory failure and pneumonia complications.The average duration of hospitalization for type 1 patients[(39.8±30.3)d]was longer than that of type 2 patients[(7.8±2.2)d](t=2.318,P=0.041).Six SMA children underwent tracheal intubation and tracheotomy,who had multiple drug-resistant bacterial infections and needed long-term treatments with rotated multiple antibiotics.The average duration of hospitalization of these 6 children[(51.3±25.3)d]was longer than that of other 4 children with non-invasive ventilation[(7.5±2.4)d](t=3.391,P=0.009).In contrast,3 patients who underwent tracheal intubation achieved a stable condition(normal body temperature;percutaneous oxygen saturation condition>94% under air condition;chest radiography without inflammation;sputum suction frequency less than 4 hours)after active application of airway clearance technique.They were successfully extubated and shifted to non-invasive ventilation.Three children who got rid of mechanical ventilation in daytime still took long-term application of nighttime non-invasive ventilation after discharge since their polysomnography indicated sleeping disorders.None of those 3 patients were hospitalized again due to severe pneumonia.Conclusions In order to avoid infection of multi-resistant bacteria,SMA children with pneumonia receiving tracheal intubation should be extubated and shift to non-invasive ventilation as soon as their condition is stable.Children who get rid of non-invasive ventilation should take polysomnography before being discharged from hospital.Long-term application of nighttime non-invasive ventilation should be taken at home if the polysomnography suggests sleeping disorders,so as to reduce the risk of re-hospitalization due to recurrent pneumonia.