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.	張鵬, 楊琳, 趙蕾, 等. 新生兒神經肌肉疾病25例病例系列報告. 中國循證兒科雜志, 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.	楊琴, 鮑燕敏, 路新國, 等. 兒童神經肌肉疾病患者的睡眠呼吸障礙特征分析. 中國當代兒科雜志, 2021, 23(2): 158-163.
7.	鄧通, 汪洋, 黃笛, 等. 臨床實踐指南制訂方法—GRADE方法理論篇. 中國循證心血管醫學雜志, 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.	謝德穎, 趙雨意, 左云霞. 新型冠狀病毒感染患兒圍手術期氣道管理及呼吸系統并發癥的防治. 國際麻醉學與復蘇雜志, 2023, 44(3): 228-231.
14.	張立新. 重癥神經肌肉疾病患者的呼吸支持與康復訓練. 第三屆全國醫藥研究論壇論文集(三), 2023: 890-896.
15.	張錚錚, 陸國平. 兒童神經肌肉病的呼吸支持. 中國小兒急救醫學, 2023, 30(6): 411-416.
16.	曹俊霞, 蔣胤, 王蓉. 不同氣道廓清技術在機械通氣危重癥患者中的應用. 當代護士(中旬刊), 2022, 29(1): 61-64.
17.	中華醫學會呼吸病學分會, 中國老年保健醫學研究會呼吸病學分會, 中國呼吸醫師分會呼吸職業發展委員會呼吸治療師工作組, 等. 機械氣道廓清技術臨床應用專家共識. 中華結核和呼吸雜志, 2023, 46(9): 866-879.
18.	張潔晶, 張海鄰. 兒童神經肌肉疾病的呼吸系統并發癥診治進展. 2018年浙江省醫學會兒科學分會學術年會論文匯編, 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. 神經肌肉疾病患者的睡眠呼吸障礙. 中華全科醫師雜志, 2018, 17(12): 1030-1033.
24.	王嶸峰, 徐文安. 睡眠呼吸障礙與神經肌肉疾病. 國際神經病學神經外科學雜志, 2017, 44(2): 192-195.
25.	中國醫師協會兒科醫師分會, 中國醫師協會兒科醫師分會兒童呼吸學組. 脊髓性肌萎縮癥呼吸管理專家共識(2022版). 中華實用兒科臨床雜志, 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.	郝紅琳, 戴毅, 黃顏, 等. 以睡眠呼吸障礙為突出表現的先天性肌營養不良1例及文獻綜述. 中風與神經疾病雜志, 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, 曹照龍, 韓芳. 神經肌肉疾病患者睡眠呼吸障礙的診治進展. 中國全科醫學, 2022, 25(33): 4203-4206.
30.	劉宇飛, 朱炬, 劉娜, 等. 肌萎縮側索硬化患者臨床特征分析. 天津醫藥, 2023, 51(7): 681-686.
31.	趙熳, 于偉. 呼吸機輔助通氣對重癥呼吸衰竭的治療效果. 中國城鄉企業衛生, 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.	何世聰. 慢性阻塞性肺疾病抗膽堿能藥物的應用進展. 系統醫學, 2022, 7(11): 194-198.
42.	黃建湖, 趙恒帥, 韋江平. A型肉毒素治療唾液腺非腫瘤性疾病研究進展. 中國口腔頜面外科雜志, 2022, 20(6): 607-611.
43.	趙婧, IsaacsonS. B型肉毒桿菌毒素治療流涎. 中國康復, 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.	柯鍵, 劉于, 樂霄. 氣道廓清技術在清理呼吸道無效患兒中的應用進展. 中華急危重癥護理雜志, 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, 郭錦麗, 辛海峰, 等. 機械性吸-呼氣技術在脊髓損傷呼吸管理中的應用. 實用骨科雜志, 2020, 26(10): 955-960.
48.	鞏應軍, 汪志平, 王艷妮, 等. 高頻胸壁振蕩技術在高齡臥床患者中的應用效果及安全性. 中華老年多器官疾病雜志, 2022, 21(2): 120-124.
49.	姜源, 王穎碩, 唐蘭芳, 等. 兒童氣道廓清技術的應用. 中華兒科雜志, 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, 孫兵, 王波, 等. 重癥患者氣道廓清技術專家共識. 中華重癥醫學電子雜志(網絡版), 2020, 6(3): 272-282.
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1. 張鵬, 楊琳, 趙蕾, 等. 新生兒神經肌肉疾病25例病例系列報告. 中國循證兒科雜志, 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. 楊琴, 鮑燕敏, 路新國, 等. 兒童神經肌肉疾病患者的睡眠呼吸障礙特征分析. 中國當代兒科雜志, 2021, 23(2): 158-163.
7. 鄧通, 汪洋, 黃笛, 等. 臨床實踐指南制訂方法—GRADE方法理論篇. 中國循證心血管醫學雜志, 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. 謝德穎, 趙雨意, 左云霞. 新型冠狀病毒感染患兒圍手術期氣道管理及呼吸系統并發癥的防治. 國際麻醉學與復蘇雜志, 2023, 44(3): 228-231.
14. 張立新. 重癥神經肌肉疾病患者的呼吸支持與康復訓練. 第三屆全國醫藥研究論壇論文集(三), 2023: 890-896.
15. 張錚錚, 陸國平. 兒童神經肌肉病的呼吸支持. 中國小兒急救醫學, 2023, 30(6): 411-416.
16. 曹俊霞, 蔣胤, 王蓉. 不同氣道廓清技術在機械通氣危重癥患者中的應用. 當代護士(中旬刊), 2022, 29(1): 61-64.
17. 中華醫學會呼吸病學分會, 中國老年保健醫學研究會呼吸病學分會, 中國呼吸醫師分會呼吸職業發展委員會呼吸治療師工作組, 等. 機械氣道廓清技術臨床應用專家共識. 中華結核和呼吸雜志, 2023, 46(9): 866-879.
18. 張潔晶, 張海鄰. 兒童神經肌肉疾病的呼吸系統并發癥診治進展. 2018年浙江省醫學會兒科學分會學術年會論文匯編, 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. 神經肌肉疾病患者的睡眠呼吸障礙. 中華全科醫師雜志, 2018, 17(12): 1030-1033.
24. 王嶸峰, 徐文安. 睡眠呼吸障礙與神經肌肉疾病. 國際神經病學神經外科學雜志, 2017, 44(2): 192-195.
25. 中國醫師協會兒科醫師分會, 中國醫師協會兒科醫師分會兒童呼吸學組. 脊髓性肌萎縮癥呼吸管理專家共識(2022版). 中華實用兒科臨床雜志, 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. 郝紅琳, 戴毅, 黃顏, 等. 以睡眠呼吸障礙為突出表現的先天性肌營養不良1例及文獻綜述. 中風與神經疾病雜志, 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, 曹照龍, 韓芳. 神經肌肉疾病患者睡眠呼吸障礙的診治進展. 中國全科醫學, 2022, 25(33): 4203-4206.
30. 劉宇飛, 朱炬, 劉娜, 等. 肌萎縮側索硬化患者臨床特征分析. 天津醫藥, 2023, 51(7): 681-686.
31. 趙熳, 于偉. 呼吸機輔助通氣對重癥呼吸衰竭的治療效果. 中國城鄉企業衛生, 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. 何世聰. 慢性阻塞性肺疾病抗膽堿能藥物的應用進展. 系統醫學, 2022, 7(11): 194-198.
42. 黃建湖, 趙恒帥, 韋江平. A型肉毒素治療唾液腺非腫瘤性疾病研究進展. 中國口腔頜面外科雜志, 2022, 20(6): 607-611.
43. 趙婧, IsaacsonS. B型肉毒桿菌毒素治療流涎. 中國康復, 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. 柯鍵, 劉于, 樂霄. 氣道廓清技術在清理呼吸道無效患兒中的應用進展. 中華急危重癥護理雜志, 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, 郭錦麗, 辛海峰, 等. 機械性吸-呼氣技術在脊髓損傷呼吸管理中的應用. 實用骨科雜志, 2020, 26(10): 955-960.
48. 鞏應軍, 汪志平, 王艷妮, 等. 高頻胸壁振蕩技術在高齡臥床患者中的應用效果及安全性. 中華老年多器官疾病雜志, 2022, 21(2): 120-124.
49. 姜源, 王穎碩, 唐蘭芳, 等. 兒童氣道廓清技術的應用. 中華兒科雜志, 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.
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A methodological exploration of systematic reviews on disease burden

Chinese Journal of Evidence-Based Medicine

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

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