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1. Bushby K, Finkel R, Birnkrant DJ, et al., Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management, Lancet Neurol, 2010;9:77-93.

2. LoMauro A, D’Angelo MG, Aliverti A, Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options, Ther Clin Risk Manag, 2015;11:1475-88.

3. Bello L, Gordish-Dressman H, Morgenroth LP, et al., Prednisone/prednisolone and deflazacort regimens in the CINRG Duchenne Natural History Study, Neurology, 2015;85:1048-55.

4. Rall S, Grimm T, Survival in Duchenne muscular dystrophy, Acta Myol, 2012;31:117-20.

5. Van Ruiten HJ, Marini Bettolo C, Cheetham T, et al., Why are some patients with Duchenne muscular dystrophy dying young: An analysis of causes of death in North East England, Eur J Paediatr Neurol, 2016;20:904-9.

6. Mayer OH, Henricson EK, McDonald CM, et al., Advances in Pulmonary Care in Duchenne Muscular Dystrophy, US Neurology, 2017;13:35–41

7. Bushby K, Finkel R, Birnkrant DJ, et al., Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care, Lancet Neurol, 2010;9:177-89.

8. Dohna-Schwake C, Ragette R, Teschler H, et al., Predictors of severe chest infections in pediatric neuromuscular disorders, Neuromuscul Disord, 2006;16:325-8.

9. Simonds AK, Chronic hypoventilation and its management, Eur Respir Rev, 2013;22:325-32.

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;170:456-65.

11. Kinnett K, Rodger S, Vroom E, et al., Imperatives for DUCHENNE MD: a Simplified Guide to Comprehensive Care for Duchenne Muscular Dystrophy, PLoS Curr, 2015;7.

12. Katz SL, Assessment of sleep-disordered breathing in pediatric neuromuscular diseases, Pediatrics, 2009;123 Suppl 4:S222-5.

13. Kravitz RM, Airway clearance in Duchenne muscular dystrophy, Pediatrics, 2009;123 Suppl 4:S231-5.

14. West JB, Luk AM. West’s Respiratory Physiology: The Essentials (Chapter 7). 10th ed. Philadelphia, USA: Lippincott Williams & Wilkins; 2015.

15. Mayer OH, Finkel RS, Rummey C, et al., Characterization of pulmonary function in Duchenne Muscular Dystrophy, Pediatr Pulmonol, 2015;50:487-94.

16. Ishikawa Y, Miura T, Ishikawa Y, et al., Duchenne muscular dystrophy: survival by cardio-respiratory interventions, Neuromuscul Disord, 2011;21:47-51.

17. West JB, Luk AM. West’s Respiratory Physiology: The Essentials (Chapter 1). 10th ed. Philadelphia, USA: Lippincott Williams & Wilkins; 2015.

18. Lo Mauro A, Aliverti A, Physiology of respiratory disturbances in muscular dystrophies, Breathe (Sheff), 2016;12:318-27.

19. Bach JR, Rajaraman R, Ballanger F, et al., Neuromuscular ventilatory insufficiency: effect of home mechanical ventilator use v oxygen therapy on pneumonia and hospitalization rates, Am J Phys Med Rehabil, 1998;77:8-19.

20. Fauroux B, Khirani S, Neuromuscular disease and respiratory physiology in children: putting lung function into perspective, Respirology, 2014;19:782-91.

21. Wagner KR, Lechtzin N, Judge DP, Current treatment of adult Duchenne muscular dystrophy, Biochim Biophys Acta, 2007;1772:229-37.

22. Sharma GD, Pulmonary function testing in neuromuscular disorders, Pediatrics, 2009;123 Suppl 4:S219-21.

23. Parker MJ, Interpreting spirometry: the basics, Otolaryngol Clin North Am, 2014;47:39-53.

24. Mellies U, Goebel C, Optimum insufflation capacity and peak cough flow in neuromuscular disorders, Ann Am Thorac Soc, 2014;11:1560-8.

25. Vaughn BV, Giallanza P, Technical review of polysomnography, Chest, 2008;134:1310-9.

26. Wagner MH, Berry RB, Disturbed sleep in a patient with Duchenne muscular dystrophy, J Clin Sleep Med, 2008;4:173-5.

27. Ganong’s Review of Medical Physiology (Section VII). 23rd ed. McGraw Hill; 2010.

28. (Accessed Jan 2018)

29. (Accessed Jan 2018)

30. (Accessed Jan 2018)

31. 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 anesthesia or sedation, Chest, 2007;132:1977-86.

32. McKim DA, Katz SL, Barrowman N, et al., Lung volume recruitment slows pulmonary function decline in Duchenne muscular dystrophy, Arch Phys Med Rehabil, 2012;93:1117-22.

33. Toussaint M, Pernet K, Steens M, et al., Cough Augmentation in Subjects With Duchenne Muscular Dystrophy: Comparison of Air Stacking via a Resuscitator Bag Versus Mechanical Ventilation, Respir Care, 2016;61:61-7.

34. Wollinsky KH, Kutter B, Geiger PM, Long-term ventilation of patients with Duchenne muscular dystrophy: experiences at the Neuromuscular Centre Ulm, Acta Myol, 2012;31:170-8.

35. Boussaid G, Lofaso F, Santos DB, et al., Impact of invasive ventilation on survival when non-invasive ventilation is ineffective in patients with Duchenne muscular dystrophy: A prospective cohort, Respir Med, 2016;115:26-32.

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