Interpretation of the 2023 American College of Chest Physicians' respiratory management guidelines for patients with neuromuscular diseases_Chinese Journal of Evidence-Based Medicine

Authors：

GUO Yiqun , LUO Yang , SHEN Man , FENG Lijuan , SONG Yurong ,  BAO Lihong

Department of Thoracic Surgery, Tongji Medical College Affiliated Union Hospital, Huazhong University of Science and Technology, Wuhan 430000, P. R. China;

Corresponding author：

BAO Lihong, Email: xhzlzxz4@163.com

Keywords：

Clinical practice guidelines; Neuromuscular diseases; Non-invasive ventilation; Pulmonary function testing; Respiratory failure; Sleep-disordered breathing; Airway clearance techniques

DOI：

10.7507/1672-2531.202402120

Video：

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Abstract Full text Figures/Tables Video References Cited by

Neuromuscular disease (NMD) encompasses a group of disorders that affect motor neurons, peripheral nerves, neuromuscular junctions, and skeletal muscles, potentially leading to respiratory muscle impairment and decline in respiratory function, significantly impacting patients' quality of life. In March 2023, clinical practice guideline titled Respiratory Management of Patients with Neuromuscular Weakness was released by the American College of Chest Physicians. This article summarizes, categorizes, and interprets the contents and key points of the guideline, aiming to provide more targeted guidance for clinical healthcare professionals and ultimately enhance the effectiveness of respiratory management for patients with NMD.

Citation： GUO Yiqun, LUO Yang, SHEN Man, FENG Lijuan, SONG Yurong, BAO Lihong. Interpretation of the 2023 American College of Chest Physicians' respiratory management guidelines for patients with neuromuscular diseases. Chinese Journal of Evidence-Based Medicine, 2025, 25(1): 95-101. doi: 10.7507/1672-2531.202402120 Copy

1.	张鹏, 杨琳, 赵蕾, 等. 新生儿神经肌肉疾病25例病例系列报告. 中国循证儿科杂志, 2020, 15(3): 177-181.
2.	Fay AJ. Neuromuscular diseases of the newborn. Semin Pediatr Neurol, 2019, 32: 100771.
3.	Kang SW, Kang YS, Moon JH, et al. Assisted cough and pulmonary compliance in patients with Duchenne muscular dystrophy. Yonsei Med J, 2005, 46(2): 233-238.
4.	Benditt JO. Respiratory care of patients with neuromuscular disease. Respir Care, 2019, 64(6): 679-688.
5.	Fauroux B, Abel F, Amaddeo A, et al. ERS statement on paediatric long-term noninvasive respiratory support. Eur Respir J, 2022, 59(6): 2101404.
6.	杨琴, 鲍燕敏, 路新国, 等. 儿童神经肌肉疾病患者的睡眠呼吸障碍特征分析. 中国当代儿科杂志, 2021, 23(2): 158-163.
7.	邓通, 汪洋, 黄笛, 等. 临床实践指南制订方法—GRADE方法理论篇. 中国循证心血管医学杂志, 2018, 10(12): 1441-1445, 1449.
8.	Balshem H, Helfand M, Schünemann HJ, et al. GRADE guidelines: 3. Rating the quality of evidence. J Clin Epidemiol, 2011, 64(4): 401-406.
9.	Andrews JC, Schünemann HJ, Oxman AD, et al. GRADE guidelines: 15. Going from evidence to recommendation-determinants of a recommendation's direction and strength. J Clin Epidemiol, 2013, 66(7): 726-735.
10.	Diekemper RL, Patel S, Mette SA, et al. Making the GRADE: CHEST updates its methodology. Chest, 2018, 153(3): 756-759.
11.	Dewidar O, Lotfi T, Langendam MW, et al. Good or best practice statements: proposal for the operationalisation and implementation of GRADE guidance. BMJ Evid Based Med, 2023, 28(3): 189-196.
12.	Chen TH, Hsu JH, Jong YJ. Noninvasive airway approaches for acute neuromuscular respiratory failure in emergency departments. Pediatr Pulmonol, 2017, 52(10): 55-57.
13.	谢德颖, 赵雨意, 左云霞. 新型冠状病毒感染患儿围手术期气道管理及呼吸系统并发症的防治. 国际麻醉学与复苏杂志, 2023, 44(3): 228-231.
14.	张立新. 重症神经肌肉疾病患者的呼吸支持与康复训练. 第三届全国医药研究论坛论文集(三), 2023: 890-896.
15.	张铮铮, 陆国平. 儿童神经肌肉病的呼吸支持. 中国小儿急救医学, 2023, 30(6): 411-416.
16.	曹俊霞, 蒋胤, 王蓉. 不同气道廓清技术在机械通气危重症患者中的应用. 当代护士(中旬刊), 2022, 29(1): 61-64.
17.	中华医学会呼吸病学分会, 中国老年保健医学研究会呼吸病学分会, 中国呼吸医师分会呼吸职业发展委员会呼吸治疗师工作组, 等. 机械气道廓清技术临床应用专家共识. 中华结核和呼吸杂志, 2023, 46(9): 866-879.
18.	张洁晶, 张海邻. 儿童神经肌肉疾病的呼吸系统并发症诊治进展. 2018年浙江省医学会儿科学分会学术年会论文汇编, 2018: 236.
19.	Bianchi C, Carrara R, Khirani S, et al. Independent cough flow augmentation by glossopharyngeal breathing plus table thrust in muscular dystrophy. Am J Phys Med Rehabil, 2014, 93(1): 43-48.
20.	Miller RG, Brooks BR, Swain-Eng RJ, et al. Quality improvement in neurology: amyotrophic lateral sclerosis quality measures. Report of the quality measurement and reporting subcommittee of the American Academy of Neurology. Amyotroph Lateral Scler Frontotemporal Degener, 2014, 15(3-4): 165-168.
21.	Vender RL, Mauger D, Walsh S, et al. Respiratory systems abnormalities and clinical milestones for patients with amyotrophic lateral sclerosis with emphasis upon survival. Amyotroph Lateral Scler, 2007, 8(1): 36-41.
22.	Simonds AK. Recent advances in respiratory care for neuromuscular disease. Chest, 2006, 130(6): 1879-1886.
23.	李慧敏, 董明林, 胡克. 神经肌肉疾病患者的睡眠呼吸障碍. 中华全科医师杂志, 2018, 17(12): 1030-1033.
24.	王嵘峰, 徐文安. 睡眠呼吸障碍与神经肌肉疾病. 国际神经病学神经外科学杂志, 2017, 44(2): 192-195.
25.	中国医师协会儿科医师分会, 中国医师协会儿科医师分会儿童呼吸学组. 脊髓性肌萎缩症呼吸管理专家共识(2022版). 中华实用儿科临床杂志, 2022, 37(6): 401-411.
26.	Georges M, Perez T, Rabec C, et al. Proposals from a French expert panel for respiratory care in ALS patients. Respir Med Res, 2022, 81: 100901.
27.	郝红琳, 戴毅, 黄颜, 等. 以睡眠呼吸障碍为突出表现的先天性肌营养不良1例及文献综述. 中风与神经疾病杂志, 2023, 40(3): 244-246.
28.	Sheehan DW, Birnkrant DJ, Benditt JO, et al. Respiratory management of the patient with duchenne muscular dystrophy. Pediatrics, 2018, 142(Suppl 2): S62-S71.
29.	丁啟迪, 曹照龙, 韩芳. 神经肌肉疾病患者睡眠呼吸障碍的诊治进展. 中国全科医学, 2022, 25(33): 4203-4206.
30.	刘宇飞, 朱炬, 刘娜, 等. 肌萎缩侧索硬化患者临床特征分析. 天津医药, 2023, 51(7): 681-686.
31.	赵熳, 于伟. 呼吸机辅助通气对重症呼吸衰竭的治疗效果. 中国城乡企业卫生, 2024, 39(1): 77-80.
32.	Berry RB, Budhiraja R, Gottlieb DJ, et al. Rules for scoring respiratory events in sleep: update of the 2007 AASM manual for the scoring of sleep and associated events. Deliberations of the sleep apnea definitions task force of the American academy of sleep medicine. J Clin Sleep Med, 2012, 8(5): 597-619.
33.	Finder JD, Birnkrant D, Carl J, et al. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med, 2004, 170(4): 456-465.
34.	Hull J, Aniapravan R, Chan E, et al. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax, 2012, 67(Suppl 1): 1-40.
35.	Rutgers M, Lucassen H, Kesteren RV, et al. Respiratory insufficiency and ventilatory support. 39th ENMC international workshop, Naarden, the Netherlands, 26-28 January 1996. European consortium on chronic respiratory insufficiency. Neuromuscul Disord, 1996, 6(6): 431-435.
36.	Delorme M, Leotard A, Lebret M, et al. Effect of intensity of home noninvasive ventilation in individuals with neuromuscular and chest wall disorders: a systematic review and meta-analysis of individual participant data. Arch Bronconeumol, 2023, 59(8): 488-496.
37.	Selim BJ, Wolfe L, Coleman JM, et al. Initiation of noninvasive ventilation for sleep related hypoventilation disorders: advanced modes and devices. Chest, 2018, 153(1): 251-265.
38.	Britton D, Hoit JD, Pullen E, et al. Experiences of speaking with noninvasive positive pressure ventilation: a qualitative investigation. Am J Speech Lang Pathol, 2019, 28(2S): 784-792.
39.	Bédard ME, McKim DA. Daytime mouthpiece for continuous noninvasive ventilation in individuals with amyotrophic lateral sclerosis. Respir Care, 2016, 61(10): 1341-1348.
40.	Delorme M, Reveillere C, Devaux C, et al. Quality of life in patients with slowly progressive neuromuscular disorders dependent on mechanical ventilation. Thorax, 2023, 78(1): 92-96.
41.	何世聪. 慢性阻塞性肺疾病抗胆碱能药物的应用进展. 系统医学, 2022, 7(11): 194-198.
42.	黄建湖, 赵恒帅, 韦江平. A型肉毒素治疗唾液腺非肿瘤性疾病研究进展. 中国口腔颌面外科杂志, 2022, 20(6): 607-611.
43.	赵婧, IsaacsonS. B型肉毒杆菌毒素治疗流涎. 中国康复, 2020, 35(7): 371.
44.	Weikamp JG, Schinagl DA, Verstappen CC, et al. Botulinum toxin-A injections vs radiotherapy for drooling in ALS. Acta Neurol Scand, 2016, 134(3): 224-231.
45.	柯键, 刘于, 乐霄. 气道廓清技术在清理呼吸道无效患儿中的应用进展. 中华急危重症护理杂志, 2020, 1(1): 86-90.
46.	Bach JR, Bianchi C, Vidigal-Lopes M, et al. Lung inflation by glossopharyngeal breathing and "air stacking" in Duchenne muscular dystrophy. Am J Phys Med Rehabil, 2007, 86(4): 295-300.
47.	高朝娜, 郭锦丽, 辛海峰, 等. 机械性吸-呼气技术在脊髓损伤呼吸管理中的应用. 实用骨科杂志, 2020, 26(10): 955-960.
48.	巩应军, 汪志平, 王艳妮, 等. 高频胸壁振荡技术在高龄卧床患者中的应用效果及安全性. 中华老年多器官疾病杂志, 2022, 21(2): 120-124.
49.	姜源, 王颖硕, 唐兰芳, 等. 儿童气道廓清技术的应用. 中华儿科杂志, 2020, 58(8): 690-693.
50.	Tattersall R, Murray D, Heverin M, et al. Respiratory measurements and airway clearance device prescription over one year in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener, 2020, 21(1-2): 70-77.
51.	Camela F, Gallucci M, Ricci G. Cough and airway clearance in Duchenne muscular dystrophy. Paediatr Respir Rev, 2019, 31: 35-39.
52.	Homnick DN. Mechanical insufflation-exsufflation for airway mucus clearance. Respir Care, 2007, 52(10): 1296-305.
53.	Miske LJ, Hickey EM, Kolb SM, et al. Use of the mechanical in-exsufflator in pediatric patients with neuromuscular disease and impaired cough. Chest, 2004, 125(4): 1406-1412.
54.	葛慧青, 孙兵, 王波, 等. 重症患者气道廓清技术专家共识. 中华重症医学电子杂志(网络版), 2020, 6(3): 272-282.
55.	Yuan N, Kane P, Shelton K, et al. Safety, tolerability, and efficacy of high-frequency chest wall oscillation in pediatric patients with cerebral palsy and neuromuscular diseases: an exploratory randomized controlled trial. J Child Neurol, 2010, 25(7): 815-821.

1. 张鹏, 杨琳, 赵蕾, 等. 新生儿神经肌肉疾病25例病例系列报告. 中国循证儿科杂志, 2020, 15(3): 177-181.
2. Fay AJ. Neuromuscular diseases of the newborn. Semin Pediatr Neurol, 2019, 32: 100771.
3. Kang SW, Kang YS, Moon JH, et al. Assisted cough and pulmonary compliance in patients with Duchenne muscular dystrophy. Yonsei Med J, 2005, 46(2): 233-238.
4. Benditt JO. Respiratory care of patients with neuromuscular disease. Respir Care, 2019, 64(6): 679-688.
5. Fauroux B, Abel F, Amaddeo A, et al. ERS statement on paediatric long-term noninvasive respiratory support. Eur Respir J, 2022, 59(6): 2101404.
6. 杨琴, 鲍燕敏, 路新国, 等. 儿童神经肌肉疾病患者的睡眠呼吸障碍特征分析. 中国当代儿科杂志, 2021, 23(2): 158-163.
7. 邓通, 汪洋, 黄笛, 等. 临床实践指南制订方法—GRADE方法理论篇. 中国循证心血管医学杂志, 2018, 10(12): 1441-1445, 1449.
8. Balshem H, Helfand M, Schünemann HJ, et al. GRADE guidelines: 3. Rating the quality of evidence. J Clin Epidemiol, 2011, 64(4): 401-406.
9. Andrews JC, Schünemann HJ, Oxman AD, et al. GRADE guidelines: 15. Going from evidence to recommendation-determinants of a recommendation's direction and strength. J Clin Epidemiol, 2013, 66(7): 726-735.
10. Diekemper RL, Patel S, Mette SA, et al. Making the GRADE: CHEST updates its methodology. Chest, 2018, 153(3): 756-759.
11. Dewidar O, Lotfi T, Langendam MW, et al. Good or best practice statements: proposal for the operationalisation and implementation of GRADE guidance. BMJ Evid Based Med, 2023, 28(3): 189-196.
12. Chen TH, Hsu JH, Jong YJ. Noninvasive airway approaches for acute neuromuscular respiratory failure in emergency departments. Pediatr Pulmonol, 2017, 52(10): 55-57.
13. 谢德颖, 赵雨意, 左云霞. 新型冠状病毒感染患儿围手术期气道管理及呼吸系统并发症的防治. 国际麻醉学与复苏杂志, 2023, 44(3): 228-231.
14. 张立新. 重症神经肌肉疾病患者的呼吸支持与康复训练. 第三届全国医药研究论坛论文集(三), 2023: 890-896.
15. 张铮铮, 陆国平. 儿童神经肌肉病的呼吸支持. 中国小儿急救医学, 2023, 30(6): 411-416.
16. 曹俊霞, 蒋胤, 王蓉. 不同气道廓清技术在机械通气危重症患者中的应用. 当代护士(中旬刊), 2022, 29(1): 61-64.
17. 中华医学会呼吸病学分会, 中国老年保健医学研究会呼吸病学分会, 中国呼吸医师分会呼吸职业发展委员会呼吸治疗师工作组, 等. 机械气道廓清技术临床应用专家共识. 中华结核和呼吸杂志, 2023, 46(9): 866-879.
18. 张洁晶, 张海邻. 儿童神经肌肉疾病的呼吸系统并发症诊治进展. 2018年浙江省医学会儿科学分会学术年会论文汇编, 2018: 236.
19. Bianchi C, Carrara R, Khirani S, et al. Independent cough flow augmentation by glossopharyngeal breathing plus table thrust in muscular dystrophy. Am J Phys Med Rehabil, 2014, 93(1): 43-48.
20. Miller RG, Brooks BR, Swain-Eng RJ, et al. Quality improvement in neurology: amyotrophic lateral sclerosis quality measures. Report of the quality measurement and reporting subcommittee of the American Academy of Neurology. Amyotroph Lateral Scler Frontotemporal Degener, 2014, 15(3-4): 165-168.
21. Vender RL, Mauger D, Walsh S, et al. Respiratory systems abnormalities and clinical milestones for patients with amyotrophic lateral sclerosis with emphasis upon survival. Amyotroph Lateral Scler, 2007, 8(1): 36-41.
22. Simonds AK. Recent advances in respiratory care for neuromuscular disease. Chest, 2006, 130(6): 1879-1886.
23. 李慧敏, 董明林, 胡克. 神经肌肉疾病患者的睡眠呼吸障碍. 中华全科医师杂志, 2018, 17(12): 1030-1033.
24. 王嵘峰, 徐文安. 睡眠呼吸障碍与神经肌肉疾病. 国际神经病学神经外科学杂志, 2017, 44(2): 192-195.
25. 中国医师协会儿科医师分会, 中国医师协会儿科医师分会儿童呼吸学组. 脊髓性肌萎缩症呼吸管理专家共识(2022版). 中华实用儿科临床杂志, 2022, 37(6): 401-411.
26. Georges M, Perez T, Rabec C, et al. Proposals from a French expert panel for respiratory care in ALS patients. Respir Med Res, 2022, 81: 100901.
27. 郝红琳, 戴毅, 黄颜, 等. 以睡眠呼吸障碍为突出表现的先天性肌营养不良1例及文献综述. 中风与神经疾病杂志, 2023, 40(3): 244-246.
28. Sheehan DW, Birnkrant DJ, Benditt JO, et al. Respiratory management of the patient with duchenne muscular dystrophy. Pediatrics, 2018, 142(Suppl 2): S62-S71.
29. 丁啟迪, 曹照龙, 韩芳. 神经肌肉疾病患者睡眠呼吸障碍的诊治进展. 中国全科医学, 2022, 25(33): 4203-4206.
30. 刘宇飞, 朱炬, 刘娜, 等. 肌萎缩侧索硬化患者临床特征分析. 天津医药, 2023, 51(7): 681-686.
31. 赵熳, 于伟. 呼吸机辅助通气对重症呼吸衰竭的治疗效果. 中国城乡企业卫生, 2024, 39(1): 77-80.
32. Berry RB, Budhiraja R, Gottlieb DJ, et al. Rules for scoring respiratory events in sleep: update of the 2007 AASM manual for the scoring of sleep and associated events. Deliberations of the sleep apnea definitions task force of the American academy of sleep medicine. J Clin Sleep Med, 2012, 8(5): 597-619.
33. Finder JD, Birnkrant D, Carl J, et al. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med, 2004, 170(4): 456-465.
34. Hull J, Aniapravan R, Chan E, et al. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax, 2012, 67(Suppl 1): 1-40.
35. Rutgers M, Lucassen H, Kesteren RV, et al. Respiratory insufficiency and ventilatory support. 39th ENMC international workshop, Naarden, the Netherlands, 26-28 January 1996. European consortium on chronic respiratory insufficiency. Neuromuscul Disord, 1996, 6(6): 431-435.
36. Delorme M, Leotard A, Lebret M, et al. Effect of intensity of home noninvasive ventilation in individuals with neuromuscular and chest wall disorders: a systematic review and meta-analysis of individual participant data. Arch Bronconeumol, 2023, 59(8): 488-496.
37. Selim BJ, Wolfe L, Coleman JM, et al. Initiation of noninvasive ventilation for sleep related hypoventilation disorders: advanced modes and devices. Chest, 2018, 153(1): 251-265.
38. Britton D, Hoit JD, Pullen E, et al. Experiences of speaking with noninvasive positive pressure ventilation: a qualitative investigation. Am J Speech Lang Pathol, 2019, 28(2S): 784-792.
39. Bédard ME, McKim DA. Daytime mouthpiece for continuous noninvasive ventilation in individuals with amyotrophic lateral sclerosis. Respir Care, 2016, 61(10): 1341-1348.
40. Delorme M, Reveillere C, Devaux C, et al. Quality of life in patients with slowly progressive neuromuscular disorders dependent on mechanical ventilation. Thorax, 2023, 78(1): 92-96.
41. 何世聪. 慢性阻塞性肺疾病抗胆碱能药物的应用进展. 系统医学, 2022, 7(11): 194-198.
42. 黄建湖, 赵恒帅, 韦江平. A型肉毒素治疗唾液腺非肿瘤性疾病研究进展. 中国口腔颌面外科杂志, 2022, 20(6): 607-611.
43. 赵婧, IsaacsonS. B型肉毒杆菌毒素治疗流涎. 中国康复, 2020, 35(7): 371.
44. Weikamp JG, Schinagl DA, Verstappen CC, et al. Botulinum toxin-A injections vs radiotherapy for drooling in ALS. Acta Neurol Scand, 2016, 134(3): 224-231.
45. 柯键, 刘于, 乐霄. 气道廓清技术在清理呼吸道无效患儿中的应用进展. 中华急危重症护理杂志, 2020, 1(1): 86-90.
46. Bach JR, Bianchi C, Vidigal-Lopes M, et al. Lung inflation by glossopharyngeal breathing and "air stacking" in Duchenne muscular dystrophy. Am J Phys Med Rehabil, 2007, 86(4): 295-300.
47. 高朝娜, 郭锦丽, 辛海峰, 等. 机械性吸-呼气技术在脊髓损伤呼吸管理中的应用. 实用骨科杂志, 2020, 26(10): 955-960.
48. 巩应军, 汪志平, 王艳妮, 等. 高频胸壁振荡技术在高龄卧床患者中的应用效果及安全性. 中华老年多器官疾病杂志, 2022, 21(2): 120-124.
49. 姜源, 王颖硕, 唐兰芳, 等. 儿童气道廓清技术的应用. 中华儿科杂志, 2020, 58(8): 690-693.
50. Tattersall R, Murray D, Heverin M, et al. Respiratory measurements and airway clearance device prescription over one year in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener, 2020, 21(1-2): 70-77.
51. Camela F, Gallucci M, Ricci G. Cough and airway clearance in Duchenne muscular dystrophy. Paediatr Respir Rev, 2019, 31: 35-39.
52. Homnick DN. Mechanical insufflation-exsufflation for airway mucus clearance. Respir Care, 2007, 52(10): 1296-305.
53. Miske LJ, Hickey EM, Kolb SM, et al. Use of the mechanical in-exsufflator in pediatric patients with neuromuscular disease and impaired cough. Chest, 2004, 125(4): 1406-1412.
54. 葛慧青, 孙兵, 王波, 等. 重症患者气道廓清技术专家共识. 中华重症医学电子杂志(网络版), 2020, 6(3): 272-282.
55. Yuan N, Kane P, Shelton K, et al. Safety, tolerability, and efficacy of high-frequency chest wall oscillation in pediatric patients with cerebral palsy and neuromuscular diseases: an exploratory randomized controlled trial. J Child Neurol, 2010, 25(7): 815-821.

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Interpretation of the 2023 American College of Chest Physicians' respiratory management guidelines for patients with neuromuscular diseases

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