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南京医科大学学报(自然科学版)                                  第42卷第2期
               ·222 ·                     Journal of Nanjing Medical University(Natural Sciences)   2022年2月


             ·临床医学·

              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



              张   琴 ,时    珺,陈 俊,缪红军
                    *
              南京医科大学附属儿童医院重症医学科,江苏 南京                   210008




             [摘    要] 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。
             [关键词] 脊髓性肌萎缩;肺部感染;呼吸道管理
             [中图分类号] R725.6                   [文献标志码] A                        [文章编号] 1007⁃4368(2022)02⁃222⁃06
              doi:10.7655/NYDXBNS20220213



              Analysis of clinical characteristics of 49 children with spinal muscular atrophytype Ⅰ and
              Ⅱ complicated with pulmonary infection

                        *
              ZHANG Qin ,SHI Jun,CHEN Jun,MIAO Hongjun
              Intensive Care Unit,Children’s Hospital of Nanjing Medical University,Nanjing 210008,China


             [Abstract] 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 SMA Ⅰ type was significantly higher than that of patients with SMA Ⅱ type(P < 0.05). The cough rate of SMA Ⅱ 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.
             [Key words] spinal muscular atrophy; pulmonary infection; respiratory tract care
                                                                         [J Nanjing Med Univ,2022,42(02):222⁃226,238]




                                                                     脊髓性肌萎缩症(spinal muscular atrophy,SMA)
                                                                是一种以脊髓前角运动神经元退行性变为主要病
             [基金项目] 国家自然科学基金(81600061);南京市医学科
                                                                理特征的遗传性神经肌肉病,临床表现为进行性对
              技发展资金项目青年人才工程项目(QRX17077)
                                                                称性肢体近端和躯干肌肉无力、萎缩和瘫痪 。由
                                                                                                        [1]
              ∗
               通信作者(Corresponding author),E⁃mail:zhangqin241@aliyun.
              com                                               于存在咳嗽无力及不同程度胃食管反流,SMA患儿
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