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中华医学超声杂志（电子版）

中华医学超声杂志（电子版） ›› 2023, Vol. 20 ›› Issue (10) : 1068 -1073. doi: 10.3877/cma.j.issn.1672-6448.2023.10.011

小儿超声影像学

超声对Duchenne肌营养不良儿童膈肌功能的评价
张璇, 马宇童, 苗玉倩, 张云, 吴士文, 党晓楚, 陈颖颖, 钟兆明, 王雪娟, 胡淼, 孙岩峰, 马秀珠, 吕发勤, 寇海燕†()   
  1. 100039　北京，解放军总医院第三医学中心超声科
    100039　北京，解放军总医院第三医学中心神经内科
    100039　北京，解放军总医院第三医学中心儿科
  • 收稿日期:2022-08-01 出版日期:2023-10-01
  • 通信作者: 寇海燕
  • 基金资助:
    海南国际医学港科技创新平台(ZDKJ2019012); 解放军总医院军事医学创新研究项目(CX19025); 军委科技委项目(19-163-12-ZT-006-007-04)

Ultrasound assessment of diaphragm function in pediatric patients with Duchenne muscular dystrophy

Xuan Zhang, Yutong Ma, Yuqian Miao, Yun Zhang, Shiwen Wu, Xiaochu Dang, Yingying Chen, Zhaoming Zhong, Xuejuan Wang, Miao Hu, Yanfeng Sun, Xiuzhu Ma, Faqin Lyu, Haiyan Kou†()   

  1. Ultrasonic Department, the Third Medical Centre of PLA General Hospital, 100039 Beijing, China
    Neurology Department, the Third Medical Centre of PLA General Hospital, 100039 Beijing, China
    Pediatric Department, the Third Medical Centre of PLA General Hospital, 100039 Beijing, China
  • Received:2022-08-01 Published:2023-10-01
  • Corresponding author: Haiyan Kou
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张璇, 马宇童, 苗玉倩, 张云, 吴士文, 党晓楚, 陈颖颖, 钟兆明, 王雪娟, 胡淼, 孙岩峰, 马秀珠, 吕发勤, 寇海燕. 超声对Duchenne肌营养不良儿童膈肌功能的评价[J]. 中华医学超声杂志（电子版）, 2023, 20(10): 1068-1073.

Xuan Zhang, Yutong Ma, Yuqian Miao, Yun Zhang, Shiwen Wu, Xiaochu Dang, Yingying Chen, Zhaoming Zhong, Xuejuan Wang, Miao Hu, Yanfeng Sun, Xiuzhu Ma, Faqin Lyu, Haiyan Kou. Ultrasound assessment of diaphragm function in pediatric patients with Duchenne muscular dystrophy[J]. Chinese Journal of Medical Ultrasound (Electronic Edition), 2023, 20(10): 1068-1073.

目的

探讨超声在评估Duchenne肌营养不良（DMD）儿童膈肌功能中的应用价值。

方法

收集2021年5月1日至2022年3月1日在解放军总医院第三医学中心临床诊断为DMD的患儿21例，同期纳入同年龄段的健康儿童志愿者21例作为对照组。所有纳入对象均行膈肌超声检查，主要检测指标包括膈肌增厚率（DTF）和膈肌移动度（DE）。对比分析2组间膈肌超声表现及DTF和DE的差异。

结果

DMD组21例患儿超声均表现为膈肌较正常儿童明显增厚，回声增强，不均匀，呼吸运动减弱。DMD组右侧腋前线DE为（12.0±5.0）mm，低于对照组（19.0±3.3）mm（t=0.207，P<0.001）；右侧锁骨中线DE为（11.7±4.1）mm，低于对照组（20.0±5.3）mm（t=0.603，P<0.001）。DMD组的右侧腋前线DTF（38.3±19.4）%低于对照组的（50.6±10.9）%（t=0.013，P=0.015）；右侧锁骨中线DTF（32.9±19.0）%低于对照组的（49.7±11.5）%（t=0.079，P=0.002）。

结论

超声可有效评价DMD患儿膈肌功能，定量测量膈肌DTF、DE有助于DMD患儿病情监测。

关键词: Duchenne肌营养不良, 超声检查, 膈肌, 儿童
Objective

To evaluate the value of ultrasonography in assessing diaphragm function in children with Duchenne muscular dystrophy (DMD).

Methods

A total of 21 children with DMD clinically diagnosed at the Third Medical Center of the PLA General Hospital from May 1, 2021 to March 1, 2022 were collected, and 21 healthy volunteers of the same age group were included as a control group. Diaphragmatic ultrasonography was performed on all subjects, and the main indicators included diaphragmatic thickening rate (DTF) and diaphragmatic mobility (DE). The differences of diaphragm ultrasonography findings, DTF, and DE between the two groups were compared and analyzed.

Results

Compared with normal children, DMD children showed obvious thickening of the diaphragm, enhanced and uneven echo, and weakened respiratory movement. The right anterior axillary line DE in the DMD group was (12.0±5.0) mm, significantly lower than that of the control group (19.0±3.3) mm (t=0.207, P<0.001). The right midclavicular line DE was (11.7±4.1) mm, also significantly lower than that of the control group (20.0±5.3) mm (t=0.603, P<0.001). The DTF [(38.3±19.4)%] in the DMD group was significantly lower than that of the control group [(50.6±10.9)%] (t=0.013, P=0.015). DTF [(32.9±19.0)%] of the right midclavicular line was lower than that of the control group [(49.7±11.5)%] (t=0.079, P=0.002).

Conclusion

Ultrasound can effectively evaluate the diaphragm function in children with DMD, and quantitative measurement of diaphragm DTF and DE is helpful to monitor the condition of children with DMD.

Key words: Duchenne muscular dystrophy, Ultrasonography, Diaphragm, Child
图1 M型超声测量膈肌移动度的图像（箭头所示为膈肌及其运动，双向箭头所示为膈肌移动度）
图2 Duchenne肌营养不良病理图像。图a示肌纤维大小中度不等，偶见坏死纤维，可见较多再生肌纤维，肌内膜及肌束膜轻度增生，血管周围未见炎症细胞浸润（HE ×200）；图b示肌纤维大小显著不等，可见少量坏死纤维及再生肌纤维，肌内膜及肌束膜重度增生，血管周围未见炎症细胞浸润（HE ×200）；图c示肌纤维大小显著不等，少量坏死肌纤维及再生肌纤维，肌内膜及肌束膜重度增生，血管周围未见炎症细胞浸润（HE ×200）
图3 正常儿童与Duchenne肌营养不良膈肌二维超声图像。图a为正常儿童膈肌二维超声图像，膈肌表现为均匀的低回声，中间可见结缔组织形成的条带状高回声线，与胸膜线、腹膜线走行较一致；图b为Duchenne肌营养不良患儿膈肌二维超声图像，膈肌较正常儿童增厚，回声增强、不均匀，肌肉间结缔组织形成的条带状高回声线部分紊乱 注：RL为肝右叶
表1 DMD组、正常对照组膈肌吸气末厚度与呼气末厚度比较（mm，）
组别 例数 右侧腋前线 t值 P值 右侧锁骨中线 t值 P值
吸气末Tdi 呼气末Tdi 吸气末Tdi 呼气末Tdi
DMD组 21 4.26±0.60 4.05±0.59 0.874 0.593 4.62±0.60 4.35±0.59 0.913 0.493
对照组 21 1.72±0.24 1.18±0.21 0.841 0.004 1.69±0.30 0.99±0.20 0.527 0.006
图4 右侧腋前线切面Duchenne肌营养不良患儿与正常儿童膈肌超声图像。图a示Duchenne肌营养不良患儿膈肌运动幅度随呼吸运动变化减弱，接近平直，吸气末与呼气末膈肌的厚度未见明显变化；图b示正常儿童膈肌随呼吸运动变化明显，呈“大波浪”样表现
表2 DMD组与正常对照组DE、DTF对比
组别 例数 右侧锁骨中线DTF（%） 右侧腋前线DTF（%） 右侧锁骨中线DE（mm） 右侧腋前线DE（mm）
DMD组 21 32.9±19.0 38.3±19.4 11.7±4.1 12.0±5.0
对照组 21 49.7±11.5 50.6±10.9 20.0±5.3 19.0±3.3
t值 0.079 0.013 0.603 0.207
P值 0.002 0.015 <0.001 <0.001
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