6．呼吸ケア - 日本神経学会
Contents
1. 2 Ward A gt 96 6 3 4 HORM Pw eRe ARTS2. lt NTT 177 Web171 2 2
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4. 3X nan SE SCI eI RO CBRL gt 100 6 ZOR 12V
5. E 2 http www nanbyou orjp D 1 SHER 2001 55 334 337 2 2
6. D 1 Duchenne 58 1984 p37 55 2 Bushby K Finkel R Birnkrant DJ et al Diagnosis and management of Duchenne muscular dystrophy part 2 implementation of multidisciplinary care Lancet Neurol 2010 9 177 189 3 2007 BMY A RAT 1 MBICBI SAY IW LY FOE EIR ICO CHM KAODT vr a http www kansensho or jp topics pdf pneumococcus_vaccine pdf 2014 1 9 6 20 r0 1 gt 82 8 Clinical Question 6 6 6
7. 1 4 1 2 L 39 4 4 3 CO6 2 LRU ORE CHE 3 W 4 H
8. 49 4 FARO WB Cid expiratory positive airway pressure EPAP FARO d APCs AK Ba HE positive end expiratory pressure PEEP PARO D WO p87e 0 SINS mechanical insufflation exsufflation MI E
9. ADL activity of daily living D 1 SHEP 9 QOL 1998 p247 2 76 NIPPV QOL 9 QOL 1998 p245 3 HE Fc 2001 55 334 337 4 SHERR 1998 52 679 682 117 20 APO 1 D 93 6 0 NEE Clinical Question 6 1 1 6 HMV
10. 5 A 15kg 35kg C9 gt I0I 0 NEE 4
11. K A 3 non invasive positive pressure ventilation NPPV B 3 19 9 ABH D NPPV B 4 TS NPPV BB 4 ONPPV B 4 Am Duchenne muscular dystrophy DMD 25 DMD
12. gt 92 8 6 SH Cb SpO 4
13. 10 15 3249 4 D 1 Panitch HB The pathophysiology of respiratory impairment in pediatric neuromuscular diseases Pedi atrics 2009 123 Suppl 4 215 8218 2 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 170 456 465 3 Inkley SR Oldenburg FC Vignos PJ Jr Pulmonary function in Duchenne muscular dystrophy related to stage of disease Am J Med 1974 56 297 306 4 Sharma GD Pulmonary function testing in neuromuscular disorders Pediatrics 2009 123 Suppl 4 S219 S221 5 Mallory GB Pulmonary complications of neuromuscular disease Pediatr Pulmonol Suppl 2004 26 138 140 6 Misuri G Lanini B Gigliotti F et al Mechanism of CO 2 retention in patients with neuromuscular dis ease Chest 2000 117 447 453 7 Khan Y Heckmatt JZ Obstructive apnoeas in Duchenne muscular dystrophy Thorax 1994 49 157 161 8 Smith
14. SpO SpO WH HMV D a Finder JD A 2009 perspective on t
15. 4 MI E NPPV JE NPPV NPPV amp 4 5 NPPV SpOs 94 NPPV MI E 3 4 NPPV 7 CO6 7 NPPV MI E MLE 20
16. 4 WCAC RRR CMAP RR Mr PRR D 726 6 ERAN ample 2 33
17. 5 D 1 Smith PE Edwards RH Calverley PM Oxygen treatment of sleep hypoxaemia in Duchenne muscular dystrophy Thorax 1989 44 997 1001 2 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 170 456 465 3 Birnkrant DJ Bushby KM Amin RS et al The respiratory management of patients with Duchenne muscu lar dystrophy a DMD care considerations working group specialty article Pediatr Pulmonol 2010 45 739 748 117 21 r0 0 gt 86 Clinical Question 6 8 6 NPPV Non invasive pos itive pressure ventilation NPPV BB 4 NPPV BARS 1
18. 2013 3 Au B 3 RB 3 CQ 6 3 QOL NPPV 3 Ow 4 1 NPPV 4 NPPV Ba 4 NPPV
19. 4 CPF SMM CHL T 2 720 Wit E BA 0o pj 7 OOZSSA C SEI 3 4 CPF 9 CO 6 3 MIC 3 5 V 4 NPPV 1 2 3 mechanical insufflation exsuf flation MLE MIC NPPV MIC gl
20. 94 a 6 TALIC EELT PLEA RECO mechanical insufflation exsufflation MI E 2013 3 HMV HMV CHE
21. B 4 B 4 Bm 1 Duchenne muscular dystrophy DMD NPPV NPPV NPPV 2 5 NPPV Hd X Tk NPPV
22. 5 2 2003 2010 2 9 4 2002 DMD 457379 DMD DMD 2004 American Thoracic Society consensus statement Cd 5 2010
23. home mechanical ventilation HMV HMV A 5 i B JV 5 2 B 5 HMV ld ACIP EIS ERED LL HMV REKOH
24. 19 40 4 56 6 4 1 EZ gt 99 0 NEE 3 3 1
25. 23 4 2 BREN 7 5 2013 3 50Hz 60Hz
26. 5 x 6 7 3P 3P 2P 5 6
27. C NPPV 9 DMD NPPV 1 NPPV DMD NPPV B 3 7 4 DMD NPPV YY 4 NPPV CO6 9 836 0 SINS CO 6 1
28. mechanical insufflation exsufflation MI E B 4 Bm Ba SEPARA bd amp Ine LL JERLSE BUE URE non invasive positive pressure ven tilation NPPV 4 X Tes PAYA 3 max imum insufflation capacity MIC glossopharyngeal breathing GPB CO 6 2 9 I 4 CPF UC 2 4
29. 12 CPFS270L min MLE 4 MEE w mechanically assisted cough MAC 3 4 high frequency chest wall oscillation HFCWO intrapulmonary percussive ventilation IPV iX 4 HFCWO D 76 6 4 PV 1 NPPV
30. 2011 3 11 L KR KE Duchenne muscular dystrophy DMD
31. Au B Duchenne muscular dystrophy DMD SCRA BLOC RRL D 1 1 2m
32. 4 5 3 NPPV NPPV ADL activity of daily living OOL DMD Cli 9 4
33. C cough peak flow CPF 270L min WEZ Eu SB 5 CQ 6 3 BR 2013 3 CQ 6 3 2013 3 CO6 1 7 2 BHA non invasive positi
34. 2009 49 872 876 a 19 3 H b 20 3 http www nanbyou or jp pdf saigai pdf 2014 1 9 c IRYO 2012 66 475 481 d 3 11 2012 2 17 APO 4 102
35. 1 2013 Au 3 B 4 O VC 40 12 CPF 270L min T CPF B 4 9 VC 40 maximum insufflation capacity MIC B 4 Sm B W 4 3 PRPC Eo CRB SADE 6 1 2 4 VC
36. 2002 8 5 59 61 b BA DMD 2 2002 8 6 62 65 c BH DMD 3 2002 8 7 71 75 d RSA WE 2010 16 8 27 30 e OOL 2004 2 301 307 f ALS 2004 g 2004 h 2008 2008 i QOL
37. ALS 2000 p 2012 26 19 E0 2 98 8 Clinical Question 6 13 6 3 B 5 a B Jy 5 9 B 5 9 B 5 Am
38. 95 Y 97 95 MIC NPPV 4 D 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 Bach JR Ishikawa Y Kim H Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy Chest 1997 112 1024 1028 Ishikawa Y Bach JR Komaroff E et al Cough augmentation in Duchenne muscular dystrophy Am J Phys Med Rehabil 2008 87 726 730 Gauld LM Boynton A Relationship between peak cough flow and spirometry in Duchenne muscular dys trophy Pediatr Pulmonol 2005 39 457 460 Brito MF Moreira GA Pradella Hallinan M et al Air stacking and chest compression increase peak cough flow in patients with Duchenne muscular dystrophy J Bras Pneumol 2009 35 973 979 Ishikawa Y Bach JR Physical medicine respiratory muscle aids to avert respiratory complications of pedi atric chest wall a
39. 4 2 4 UE SUCRE TY Dj o CT 4 CT gt 90 6
40. 14 BIO ATIF RAGE A DMD 6 9 vital capacity VC 21 6 26 2 6 FVC FVC 0 NEE PA Wiki MRVL LzZi T S D 1 Smith PE Coakley JH Edwards RH Respiratory muscle training in Duchenne muscular dystrophy Mus cle Nerve 1988 11 784 785 2 Stern LM Martin AJ Jones N et al Respiratory training in Duchenne dystrophy Dev Med Child Neurol 1991 33 649 3 Vilozni D Bar Yishay E Gur I et a
41. 4 CO 6 3 2H NPPV SpO 4 Le Sp RH WAX PY 1 A 3 NPPV OD SpO 4 4 NPPV NPPV 1 SpO
42. TH VC OTK3 ASTE 3025 0C T ARYEN EMO BA CHAT A 4 JERS BJQLXL5 325 0LE 4 70 2013 3 B non invasive positive pressure ventilation NPPV D 7 e 6 CPF 12 CPF gt 160L min CPF gt 270L min T
43. 1 HMV HMV HMV HMV DOSE QE HMV W
44. 4 D 1 Ishikawa Y Miura T Ishikawa Y et al Duchenne muscular dystrophy survival by cardio respiratory interventions Neuromuscul Disord 2011 21 47 51 2 Bach JR Rajaraman R Ballanger F et al Neuromuscular ventilatory insufficiency effect of home mechani cal ventilator use v oxygen therapy on pneumonia and hospitalization rates Am J Phys Med Rehabil 1998 77 8 19 3 Young HK Lowe A Fitzgerald DA et al Outcome of noninvasive ventilation in children with neuromus cular disease Neurology 2007 68 198 201 4 Birnkrant DJ Bushby KM Amin RS et al The respiratory management of patients with duchenne muscu lar dystrophy a DMD care considerations working group specialty article Pediatr Pulmonol 2010 45 739 748 88 6 5 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 170 456 465 6 Bushby K Finkel R Birnkrant DJ et al Diagnosis and management of Duchenne muscular dystrophy part 2 implementation of multidisciplinary care Lancet Neurol 2010 9 177 189 7 Bach JR Martinez D Duchenne muscular dystrophy continuous noninvasive ventilatory support pro longs survival Respir Care 2011 56 744 750 8 Kohler M Clarenbach CF Boni L et al Quality of life physical disability and respiratory impairment in Duchenne muscular dystrophy Am J
45. 8 9 HMV amyotrophic lateral sclerosis ALS gt 97 8 0 SINS D a Bj 1
46. 50 CO 45 mmHg ELE NPPV NPPV NPPV NPPV SpOs 94 45 mmHg DE NPPV gt BOS D 1 Gibson B Long term ventilation for patients with Duchenne muscular dystrophy physicians beliefs and practices Chest 2001 119 940 946 2 Boland BJ Silbert PL Groover RV et al Skeletal cardiac and smooth muscle failure in Duchenne muscu lar dystrophy Pediatr Neu
47. SpO2 C NPPV D 4 CO 6 2 LEFTY 4 DMD 4 4 CO 6 7 2 NPPV Xe NPPV 3 A NPPV 30 SpO 94 45 mmHg DLE SpO 92 4 4 apnea hypopnea index AHI 10
48. lt 6 RAE 1 1 OR COS CMT LI AT We 0
49. O peia _ 12V ww oT Baie DE IA AR REK O BRAKE ZEREROE MEE O O O Pae O RABKA mA REE ojassa 0 Oo J Jj 0 Oo Z7 J D oO n s ER OF Ras Sack FR ee BERS
50. 85 4 0 NEE Clinical Question 6 7 6 HE Au D 4 2 Duchenne muscular dystrophy DMD B DMD 2 4 non invasive positive pressure ventilation NPPV CO 6 6 NPPV
51. thesia or sedation Chest 2007 132 1977 1986 56 20 APO 1 78 6 Clinical Question 6 4 6 DMD F overuse J C 4 B 4 Duchenne muscular dystrophy DMD 1980 90 4 3
52. 568 Hull J Aniapravan R Chan E et al Guideline for respiratory management of children with neuromuscu lar weakness Thorax 2012 67 654 655 Chatwin M Mechanical aids for secretion clearance International Journal of Respiratory Care 2009 Autumn Winter 50 53 Crescimanno G Marrone O High frequency chest wall oscillation plus mechanical in exsufflation in Duchenne muscular dystrophy with respiratory complications related to pandemic Influenza A HINI Rev Port Pneumol 2010 16 912 916 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 170 456 465 Bach JR Saporito LR Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure a different approach to weaning Chest 1996 110 1566 1571 Birnkrant DJ Bushby KM Amin RS et al The respiratory management of patients with duchenne muscular dystrophy a DMD care considerations working group specialty article Pediatr Pulmonol 2010 45 739 748 Gomez Merino E Bach JR Duchenne muscular dystrophy prolongation of life by noninvasive ventilation 0 SINS and mechanically assisted coughing Am J Phys Med Rehabil 2002 81 411 415 22 Birnkrant DJ Panitch HB Benditt JO et al American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anes
53. F c 9 4 5 D 1 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 170 456 465 2 Bushby K Finkel R Birnkrant DJ et al Diagnosis and management of Duchenne muscular dystrophy part 2 implementation of multidisciplinary care Lancet Neurol 2010
54. Respir Crit Care Med 2005 172 1032 1036 9 Robert D Willig TN Leger P et al Long term nasal ventilation in neuromuscular disorders report of a consensus conference Eur Respir J 1993 6 599 606 10 Ishikawa Y Ishikawa Y Minami R The effect of nasal IPPV on patients with respiratory failure during sleep due to Duchenne muscular dystrophy Rinsho Shinkeigaku 1993 33 856 861 11 Kohler M Clarenbach CF Boni L et al Quality of life physical disability and respiratory impairment in Duchenne muscular dystrophy Am J Respir Crit Care Med 2005 172 1032 1036 12 Hess DR How to initiate a noninvasive ventilation program bringing the evidence to the bedside Respir Care 2009 54 232 243 discussion 43 45 13 Bach JR A comparison of long term ventilatory support alternatives from the perspective of the patient and care giver Chest 1995 104 1702 1706 14 HullJ Aniapravan R Chan E et al British Thoracic Society guideline for respiratory management of chil dren with neuromuscular weakness Thorax 2012 67 11 140 15 Toussaint M Steens M Wasteels G et al Diurnal ventilation via mouthpiece survival in end stage Duchenne patients Eur Respir J 2006 28 549 555 16 Fauroux B Guillemot N Aubertin G et al Physiologic benefits of mechanical insufflation exsufflation in children with neuromuscular diseases Chest 2008 133 161 168 17 Finsterer J Gelpi E Stollberger C Left ventricular hypertrabeculati
55. 6 Clinical Question 6 1 6 TORE 3 lt A 3 CQ 6 2 CQ 6 6 9 RREK B 3 QOL
56. 9 177 189 3 AIF BEPEUSURE Ze Boro Oak H RA UU Dr A EIFE Z D BEER NPPV 2007 18 94 07 4 76 RI E Duchenne 5 1994 p163 170 5 Saito T Sawabata N Matsumura T et al Tracheo arterial fistula in tracheostomy patients with Duchenne muscular dystrophy Brain Dev 2006 28 223 227 6 leah Ft AMARA ESET ST Str U 2010 30 11 17 7 Vianello A Ragazzi R Mirri L et al Tracheoinnominate fistula in a Duchenne muscular dystrophy patient successful management with an endovascular stent Neuromuscul Disord 2005 15 569 571 8 RL PE Duchenne 1996 36 355 357 9 Matsumura T
57. PE Edwards RH Calverley PM Ventilation and breathing pattern during sleep in Duchenne mus cular dystrophy Chest 1989 96 1546 1351 9 Barbe F Quera Salva MA McCann C et al Sleep related respiratory disturbances in patients with Duchenne muscular dystrophy Eur Respir J 1994 7 1403 1408 10 Kerr SL Kohrman MH Polysomnographic abnormalities in Duchenne muscular dystrophy J Child Neu rol 1994 9 332 334 11 Suresh S Wales P Dakin C et al Sleep related breathing disorder in Duchenne muscular dystrophy dis ease spectrum in the paediatric population J Paediatr Child Health 2005 41 500 503 12 Birnkrant DJ Ashwath ML Noritz GH et al Cardiac and pulmonary function variability in Duchenne Becker muscular dystrophy an initial report J Child Neurol 2010 25 1110 1115 12 21 APO 0 0 NEE Clinical Question 6 2 6 1 vital capacity VO B A 1 VC cough peak flow CPF B 4 VC 40
58. R OZ OORA 3 1992 p111 112 58 21 r0 0 gt 80 Clinical Question 6 5 6 HE B Ax 5 Duchenne muscular dystrophy DMD DMD DMD DMD TIARI OUR PV EO HARDWER
59. Saito T Fujimura H Lung inflation training using a positive end expiratory pressure valve im neuromuscular disorders Intern Med 2012 51 711 716 126 21 150 0 gt 9 0 SINS Clinical Question 6 10 6 5 AU KB 5 CHD 1
60. he 2004 American Thoracic Society statement respiratory care of the patient with Duchenne muscular dystrophy Pediatrics 2009 123 Suppl 4 S239 S241 b A Loa HUE VBI Ez NPPV Xi CSF ay cy AMY ALOT 4 HORS 2009 14 154 158 c Ft 12 2002 p154 d 2008 4 23 26 e Sejerson T Bushby K Standards of care for Duchenne muscular dystrophy brief TREAT NMD recom mendations Adv Exp Med Bio 2009 652 13 21 40 21 r0 0 gt 95 0 NEE Clinical Question 6 12 6 HMV home mechanical ventilation HMV
61. l Computerized respiratory muscle training in children with Duchenne muscular dystrophy Neuromuscul Disord 1994 4 249 255 4 Wanke T Toifl K Merkle M et al Inspiratory muscle training in patients with Duchenne muscular dystro phy Chest 1994 105 475 482 5 Koessler W Wanke T Winkler G et al 2 Years experience with inspiratory muscle training in patients with neuromuscular disorders Chest 2001 120 765 769 6 Gozal D Thiriet P Respiratory muscle training in neuromuscular disease long term effects on strength and load perception Med Sci Sports Exerc 1999 31 1522 1527 7 Topin N Matecki S Le Bris S et al Dose dependent effect of individualized respiratory muscle training in children with Duchenne muscular dystrophy Neuromuscul Disord 2002 12 576 583 8 Takaso M Nakazawa T Imura T et al Surgical management of severe scoliosis with high risk pulmonary dysfunction in Duchenne muscular dystrophy Int Orthop 2010 34 401 406 9 Takaso M Nakazawa T Imura T et al Surgical management of severe scoliosis with high risk pulmonary dysfunction in Duchenne muscular dystrophy patient function quality of life and satisfaction Int Orthop 2010 34 695 702 10 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 170 456 465 11 MERRE
62. l disease patients with daytime normocapnia Thorax 2005 60 1019 1024 Ishikawa Y Miura T Ishikawa Y et al Duchenne muscular dystrophy survival by cardio respiratory interventions Neuromuscul Disord 2011 21 47 51 Benditt JO Initiating noninvasive management of respiratory insufficiency in neuromuscular disease Pediatrics 2009 123 Suppl 4 5236 5238 Simonds AK Muntoni F Heather S et al Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy Thorax 1998 53 949 952 Soudon P Steens M Toussaint M A comparison of invasive versus noninvasive full time mechanical ven tilation in Duchenne muscular dystrophy Chron Respir Dis 2008 5 87 93 Toussaint M Soudon P Kinnear W Effect of non invasive ventilation on respiratory muscle loading and endurance in patients with Duchenne muscular dystrophy Thorax 2008 63 430 434 Birnkrant DJ Bushby KM Amin RS et al The respiratory management of patients with Duchenne muscu lar dystrophy a DMD care considerations working group specialty article Pediatr Pulmonol 2010 45 739 748 Dreher M Rauter I Storre JH et al When should home mechanical ventilation be started in patients with different neuromuscular disorders Respirology 2007 12 749 753 Hamada S Ishikawa Y Aoyagi T et al Indicators for ventilator use in Duchenne muscular dystrophy Respir Med 2011 105 625 629 207 19 APO 2
63. nd vertebral deformity and muscle dysfunction Eur J Phys Rehabil Med 2010 46 581 597 Kang SW Bach JR Maximum insufflation capacity vital capacity and cough flows in neuromuscular dis ease Am J Phys Med Rehabil 2000 79 222 227 Bach JR Bianchi C Vidigal Lopes M et al Lung inflation by glossopharyngeal breathing and air stack ing in Duchenne muscular dystrophy Am J Phys Med Rehabil 2007 86 295 300 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 170 456 465 Kang SW Bach JR Maximum insufflation capacity Chest 2000 118 61 65 Winck JC Goncalves MR Lourenco C et al Effects of mechanical insufflation exsufflation on respiratory parameters for patients with chronic airway secretion encumbrance Chest 2004 126 774 780 Chatwin M Ross E Hart N et al Cough augmentation with mechanical insufflation exsufflation in patients with neuromuscular weakness Eur Respir J 2003 21 502 508 Homnick DN Mechanical insufflation exsufflation for airway mucus clearance Respir Care 2007 52 1296 1305 discussion 306 307 Bach JR Mechanical insufflation exsufflation Comparison of peak expiratory flows with manually assist ed and unassisted coughing techniques Chest 1993 104 1553 1562 Bach JR Goncalves M Ventilator weaning by lung expansion and decannulation Am J Phys Med Rehabil 2004 83 560
64. on noncompaction as a cardiac mani festation of Duchenne muscular dystrophy under non invasive positive pressure ventilation Acta Cardi ol 2005 60 445 448 18 Bach JR Ishikawa Y Kim H Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy Chest 1997 112 1024 1028 19 Gomez Merino E Bach JR Duchenne muscular dystrophy prolongation of life by noninvasive ventilation and mechanically assisted coughing Am J Phys Med Rehabil 2002 81 411 415 20 Birnkrant DJ Panitch HB Benditt JO et al American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anes thesia or sedation Chest 2007 132 1977 1986 21 Bach JR Goncalves MR Hamdani I et al Extubation of patients with neuromuscular weakness a new management paradigm Chest 2010 137 1033 1039 33 20 Atl 0 gt 896 0 NEE Clinical Question 6 9 6 non invasive positive pressure ventilation NPPV NPPV B 5 Jg
65. ossopharyngeal breathing GPB eW 4 40 1 Warren RH Alderson SH A pulmonary monitoring and treatment Plan for children with Duchenne type muscular dystrophies J Ark Med Soc 1996 93 333 337 2 Birnkrant DJ Bushby KM Amin RS et al The respiratory management of patients with duchenne muscu lar dystrophy a DMD care considerations working group specialty article Pediatr Pulmonol 2010 45 739 748 3 Toussaint M Steens M Soudon P Lung function accurately predicts hypercapnia in patients with Duchenne muscular dystrophy Chest 2007 131 368 375 4 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 170 456 465 5 Hukins CA Hillman DR Daytime predictors of sleep hypoventilation in Duchenne muscular dystrophy Am J Respir Crit Care Med 2000 161 166 170 6 Bach JR Goncalves MR Paez S et al Expiratory flow maneuvers in patients with neuromuscular diseases Am J Phys Med Rehabil 2006 85 105 111 7 Hahn A Bach JR Delaubier A et al Clinical implications of maximal respiratory pressure determinations for individuals with Duchenne muscular dystrophy Arch Phys Med Rehabil 1997 78 1 6 8 Suarez AA Pes
66. rol 1996 14 7 12 3 Rumbak MJ Walker RM Should patients with neuromuscular disease be denied the choice of the treat ment of mechanical ventilation Chest 2001 119 683 684 4 Kinali M Manzur AY Mercuri E et al UK physicians attitudes and practices in long term non invasive ventilation of Duchenne Muscular Dystrophy Pediatr Rehabil 2006 9 351 364 D 844 5 6 7 8 9 10 11 12 13 14 15 16 17 18 6 Ramelli GP Hammer J Swiss physicians practices of long term mechanical ventilatory support of patients with Duchenne Muscular Dystrophy Swiss Med Wkly 2005 135 599 604 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 170 456 465 Bushby K Finkel R Birnkrant DJ et al Diagnosis and management of Duchenne muscular dystrophy part 2 implementation of multidisciplinary care Lancet Neurol 2010 9 177 189 Hull J Aniapravan R Chan E et al British Thoracic Society quideline for respiratory management of chil dren with neuromuscular weakness Thorax 2012 67 11 140 Bach JR Ishikawa Y Kim H Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy Chest 1997 112 1024 1028 Ward S Chatwin M Heather S et al Randomised controlled trial of non invasive ventilation NIV for nocturnal hypoventilation in neuromuscular and chest wal
67. solano FA Monteiro SG et al Peak flow and peak cough flow in the evaluation of expira tory muscle weakness and bulbar impairment in patients with neuromuscular disease Am J Phys Med Rehabil 2002 81 506 511 9 Kang SW Bach JR Maximum insufflation capacity vital capacity and cough flows in neuromuscular dis ease Am J Phys Med Rehabil 2000 79 222 227 10 Kang SW Bach JR Maximum insufflation capacity Chest 2000 118 61 65 11 Bach JR Bianchi C Vidigal Lopes M et al Lung inflation by glossopharyngeal breathing and air stack ing in Duchenne muscular dystrophy Am J Phys Med Rehabil 2007 86 295 300 32 20 Atl 0 TS 64 0 NEE Clinical Question 6 3 6 vital capacity VC 40 12 cough peak flow CPF 270L min 3s B 4 OVC 40 B 4
68. ve pressure ventilation NPPV CQ 6 6 H8 94 CO 678 CO 10 2 23 1 ik 2 5 gt 8le 0 SINS 2 LE 5 DMD
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