Abstract:

Objective　To summarize the clinical features of spinal muscular atrophy complicated with pulmonary infection in children，and to improve clinicians’ understanding of the disease and improve the prognosis. Methods　The clinical data of 36 children with SMA complicated with pulmonary infection，who were admitted to Yuying Children’s Hospital of the Second Affiliated Hospital of Wenzhou Medical University from January 1，2008 to December 31，2017，were retrospectively analyzed. Results　Among of the 36 patients，19 were typeⅠ，9 were typeⅡ，and 8 were typeⅢ. The common clinical manifestations and signs were fever，cough，shortness of breath，laborious breathing，three-concave signs，and crackles in the lungs. Respiratory failure occurred in 11 children，including 7 children（63.6%） with typeⅠ SMA，2 children（18.2%） with typeⅡ SMA and 2 children（18.2%） with typeⅢ SMA. Imaging findings showed 5 cases of scoliosis，3 cases being typeⅡ SMA，and 2 being typeⅢ SMA. Pathogenic tests were positive in 18 children：10 cases（55.5%） of type Ⅰ SMA，4 cases（22.2%） of typeⅡ SMA，4 cases（22.2%） of typeⅢ SMA；nosocomial mixed infection with conditioned pathogens was common，among which Burkholderia cepacia was the most common. Three patients died in the hospital，22 patients improved and discharged，and the remaining 11 patients gave up treatment. The number of hospitalizations，the incidence of severe pneumonia and respiratory failure was significantly different between the first 5 years（2008-2012） and the last 5 years（2013-2018）（P＜0.05）.  Conclusion　SMA is verysusceptible to pulmonary infection. We should be alert to opportunistic pathogenic bacteria infection and use mechanical ventilation in time for respiratory failure patients. Active and effective respiratory care can reduce the incidence of pulmonary infection and improve the prognosis of SMA children.

Key words: spinal muscle atrophy, pulmonary infection, clinical features, respiratory tract care, child

摘要：

目的　总结儿童脊髓性肌萎缩合并肺部感染的临床特征，提高临床医生对该病的认识，改善预后。方法　回顾性分析温州医科大学附属第二医院育英儿童医院2008年1月1日至2017年12月31日收治的36例脊髓性肌萎缩症（SMA）合并肺部感染患儿的临床资料。结果　36例中Ⅰ型19例，Ⅱ型9例，Ⅲ型 8例。临床表现和体征以发热、咳嗽、气促或呼吸费力、三凹征、肺部细湿啰音等多见。有11例患儿出现呼吸衰竭，其中Ⅰ型SMA患儿7例（63.6%），Ⅱ型SMA患儿2例（18.2%），Ⅲ型SMA患儿2例（18.2%）。5例SMA患儿影像学检查提示脊柱侧弯，3例为Ⅱ型SMA患儿，2例为Ⅲ型患儿。有18例患儿病原学检测阳性，Ⅰ型SMA患儿10例（55.5%），Ⅱ型SMA患儿4例（22.2%），Ⅲ型SMA患儿4例（22.2%），以院内条件致病菌混合感染多见，其中洋葱伯克霍尔德氏菌最常见。3例患儿在院内死亡，22例患儿好转出院，其余11例患儿放弃治疗。住院治疗的次数，重症肺炎、呼吸衰竭的发生率在前、后5年的比较差异均有统计学意义（P＜0.05）。结论　SMA极易并发肺部感染，要警惕条件致病菌感染，对呼吸衰竭者及时使用机械通气。积极有效的呼吸道护理，对减少肺部感染发生，改善SMA患儿的预后有积极的影响。

关键词: 脊髓性肌萎缩, 肺部感染, 临床特征, 呼吸道护理, 儿童