Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2019 Oct 2;57(10):792-796.
doi: 10.3760/cma.j.issn.0578-1310.2019.10.012.

[Clinical characteristics of non-invasive ventilation treatment in children with spinal muscular atrophy and sleep disordered breathing]

[Article in Chinese]
W H Guo  1 , L Cao, L Chang
Affiliations

[Clinical characteristics of non-invasive ventilation treatment in children with spinal muscular atrophy and sleep disordered breathing]

[Article in Chinese]
W H Guo et al. Zhonghua Er Ke Za Zhi. .

Abstract
in English, Chinese

Objective: To study the short-term and long-term efficacy of the non-invasive ventilation treatment in children with spinal muscular atrophy (SMA) and sleep-disordered breathing. Methods: This was a prospective research to study the effect of night-time non-invasive ventilation in children with SMA and moderate to severe sleep-disordered breathing during March 2016 to January 2018, from the Pulmonary Department of Capital Institute of Pediatrics Affiliated Children's Hospital. Patients were divided into the treated group (with night-time non-invasive ventilation) and the control group (without ventilator). Sleep breathing pressure titration was suggested to the patients who were prepared to receive non-invasive ventilation. All cases were followed up for one year. Parameters'changes in polysomnography were assessed (paired t-test) in titration patients. Frequency of respiratory tract infection during the next year in the patients with and without ventilation was collected and compared (Mann-Whitney U-test). Results: Seventeen cases were recruited. The average age was (5.1±2.9) years, 10 cases were boys and 7 cases were girls. In the titration group (8 patients), after non-invasive ventilation, the average apnea hypopnea index was (3.8±2.5) times/h (t=4.086, P=0.005), hypopnea index was (2.4±1.2) times/h (t=2.779, P=0.027), average oxygen saturation during total sleep time was 0.966±0.007 (t=-5.292, P=0.001), and the minimum oxygen saturation was 0.906±0.023 (t=-3.938, P=0.006). All the above parameters were significantly improved after treatment. Than before, which was (16.6±9.7) times/h, (7.2±4.7) times/h, 0.946±0.015, 0.786±0.092 respectively. Ventilator mode for the 9 children with long time non-invasive ventilation at home was Bi-level positive airway pressure S/T. The positive airway pressure was set at 8-14 cmH(2)O (1 cmH(2)O=0.098 kPa) in inspiratory phase and 4-6 cmH(2)O in expiratory phase. In the treated group (9 patients), the average frequency of upper respiratory tract infection was 1.0 (0, 3.0) times/year (Z=-2.245, P=0.023), the lower respiratory tract infection was 0 (0, 0) times/year (Z=-3.189, P=0.001), hospitalization was 0 (0, 0) times/year (Z=-3.420, P<0.01), and admission to intensive care unit was 0 (0, 0) times/year (Z=-3.353, P=0.029). All the above indexes were significantly decreased compared with the control group (8 patients), which was 3.0 (2.3, 7.0) times/year, 2.0 (1.3, 4.5) times/year, 1.0 (1.0, 4.3) times/year, 0.5 (0, 1.0) times/year respectively. Conclusion: Non-invasive ventilation is efficient to SMA children with sleep-disordered breathing, and also can reduce the incidence of respiratory tract infections for children with SMA.

目的： 探讨无创通气治疗脊髓性肌萎缩（SMA）患儿睡眠呼吸紊乱的有效性和长期作用。 方法： 前瞻性研究2016年3月至2018年1月在首都儿科研究所附属儿童医院呼吸内科就诊，有中重度睡眠呼吸紊乱的17例SMA患儿的夜间无创通气的治疗效果。患儿分为治疗组（使用无创通气）9例和对照组（未使用无创通气），8例拟行无创通气治疗的患儿需完成睡眠呼吸压力滴定。治疗组和对照组患儿均随访1年。采用配对样本t检验，比较完成睡眠呼吸压力滴定的SMA患儿无创通气治疗前后多道睡眠描记术中各参数的变化。采用Mann-Whitney U秩和检验，比较治疗组和对照组患儿1年内发生呼吸道感染频率的差异。 结果： 17例SMA患儿男10例、女7例，就诊时年龄（5.1±2.9）岁。8例患儿完成睡眠呼吸压力滴定后，多道睡眠描记术结果显示，呼吸暂停低通气指数为（3.8±2.5）次/h，与滴定前（16.6±9.7）次/h相比显著减低（t=4.086，P=0.005），滴定后低通气指数为（2.4±1.2）次/h，与滴定前（7.2±4.7）次/h相比显著减低（t=2.779，P=0.027），滴定后平均血氧饱和度为0.966±0.007，与滴定前0.946±0.015相比有显著改善（t=-5.292，P=0.001），滴定后最低血氧饱和度为0.906±0.023，与滴定前0.786±0.092相比有显著改善（t=-3.938，P=0.006）。治疗组9例长期家庭夜间无创通气患儿呼吸机模式均为双水平气道正压通气S/T模式，吸气相气道正压8~14 cmH(2)O（1 cmH(2)O=0.098 kPa），呼气相气道正压4~6 cmH(2)O，1年后患上呼吸道感染1.0（0，3.0）次/年，明显低于8例对照组未使用无创通气患儿的3.0（2.3，7.0）次/年（Z=-2.245，P=0.023），治疗后下呼吸道感染0（0，0）次/年，明显低于对照组的2.0（1.3，4.5）次/年（Z=-3.189，P=0.001），治疗后住院频率0（0，0）次/年，明显低于对照组的1.0（1.0，4.3）次/年（Z=-3.420，P<0.01），治疗后重症监护室住院频率0（0，0）次/年，明显低于对照组的0.5（0，1.0）次/年（Z=-3.353，P=0.029）。 结论： 无创通气可以有效治疗SMA患儿睡眠呼吸紊乱，且长期进行夜间无创通气治疗可减少SMA患儿呼吸道感染的发生。.

Keywords: Polysomnography; Respiratory; Sleep; Spinal muscular atrophies of childhood.

PubMed Disclaimer