儿童脊髓性肌萎缩症Ⅰ、Ⅱ型合并肺部感染49例临床特征分析
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国家自然科学基金(81600061);南京市医学科技发展资金项目青年人才工程项目(QRX17077)


Analysis of clinical characteristics of 49 children with spinal muscular atrophytype Ⅰ and Ⅱ complicated with pulmonary infection
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    目的:提高临床医生对合并肺部感染的脊髓性肌萎缩症(spinal muscular atrophy,SMA)患儿的呼吸道管理及诊治水平。方法:回顾性分析2013年1月1日—2020年7月31日所收治的49例合并肺部感染的SMA患儿的临床资料。结果:49例中SMAⅠ型35例,SMAⅡ型14例,无SMAⅢ型患者。合并肺部感染的SMAⅠ型患儿较SMAⅡ型患儿发病年龄更早,平均住院日更长(P < 0.05)。SMAⅠ型患儿发生心功能不全及呼吸衰竭的比率显著高于SMAⅡ型患者(P<0.05)。SMAⅡ型咳嗽比率显著高于SMAⅠ型患者(P<0.05)。SMAⅠ型呼吸道病原检测以多重耐药菌及条件致病菌为主,SMAⅡ型以支原体、肺炎链球菌感染为主。SMAⅠ型患儿有创通气比率及碳青霉烯类抗生素使用率均显著高于SMAⅡ型组(P<0.05)。结论:SMA患儿易合并肺部感染。积极促进气道分泌物排出、恰当的呼吸支持及合理抗感染是SMA合并肺部感染的治疗关键。

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    Objective:This study aims to improve the clinicians’ levels of respiratory management and treatment of children with spinal muscular atrophy(SMA)with pulmonary infection. Methods:A retrospective analysis of the clinical data of 49 children with SMA complicated withpulmonary infection admitted from January 1,2013 to July 31,2020. Results:Among the 49 cases,35 cases were type Ⅰ,14 cases were type Ⅱ,and there was no type Ⅲ patient. Compared with type Ⅱ,SMAⅠ children with lung infection had an earlier age of onset,a longer average hospital stays(P < 0.05). The rate of cardiac insufficiency and respiratory failure in children with SMAⅠ type was significantly higher than that of patients with SMAⅡtype(P < 0.05). The cough rate of SMAⅡ type was significantly higher than that of SMAⅠ type patients(P < 0.05). The detection of respiratory pathogens of the SMAⅠ type is mainly multi-drug resistant bacteria and conditional pathogens,and the SMAⅡ type is mainly infected by Mycoplasma and Streptococcus pneumoniae. The rate of invasive ventilation and the use of carbapenem antibioticsin children with SMAⅠ were significantly higher than those in the SMAⅡgroup(P<0.05). Conclusion:Children with SMA are prone to pulmonary infection. Actively promoting the discharge of airway secretions,appropriate respiratory support and reasonable anti-infection are crucial for the treatment of SMA complicated with lung infection.

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张 琴,时 珺,陈 俊,缪红军.儿童脊髓性肌萎缩症Ⅰ、Ⅱ型合并肺部感染49例临床特征分析[J].南京医科大学学报(自然科学版),2022,42(2):222-226

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  • 收稿日期:2021-10-19
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  • 在线发布日期: 2022-03-01
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