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Hypoventilation Syndromes

Amanda J. Piper, Brendon J. Yee

10.1002/cphy.c140008

Source: Volume 4, Issue 4, October 2014

Published online: September 2014

Full Article on Wiley Online Library



Abstract

In patients with impaired inspiratory muscle function or altered respiratory system mechanics, an imbalance between load and capacity can arise. The ventilatory control system normally compensates for this by increasing drive to maintain adequate alveolar ventilation levels, thereby keeping arterial CO2 within its normal range. To reduce work of breathing, a pattern of reduced tidal volume and increased respiratory rate occurs. This pattern itself may eventually reduce effective ventilation by increasing dead space ventilation. However, the impact of sleep on breathing and its role in the development of diurnal respiratory failure is often overlooked in this process. Sleep not only reduces respiratory drive, but also diminishes chemoresponsiveness to hypoxia and hypercapnia creating an environment where significant alterations in oxygenation and CO2 can occur. Acute increases in CO2 load especially during rapid eye movement sleep can initiate the process of bicarbonate retention which further depresses ventilatory responsiveness to CO2. Treatment of hypoventilation needs to be directed toward factors underlying its development. Nocturnal noninvasive positive pressure therapy is the most widely used and reliable strategy currently available to manage hypoventilation syndromes. Although this may not consistently alter respiratory muscle strength or the mechanical properties of the respiratory system, it does appear to reset chemosensitivity by reducing bicarbonate, resulting in a more appropriate ventilatory response to CO2 during wakefulness. Not only is diurnal hypoventilation reduced with noninvasive ventilation, but quality of life, functional capacity and survival are also improved. However, close attention to how therapy is set up and used are key factors in achieving clinical benefits. © 2014 American Physiological Society. Compr Physiol 4:1639‐1676, 2014.

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Figure 1. Figure 1. Changes in minute ventilation (V I), tidal volume (V T) and respiratory rate from wakefulness to sleep in patients with nocturnal hypoventilation (Desaturators), patients with eucapnic OSA during effective CPAP, and healthy normal controls. In the nocturnal hypoventilation group, the larger relative falls in V T were not offset by changes in respiratory rate so that an overall reduction in V I occurred. [Adapted, with permission, from Becker et al. 1999; Breathing during sleep in patients with nocturnal desaturation. Am J Respir Crit Care Med, 159:112‐118. Reprinted with permission of the American Thoracic Society. Copyright © 2014 American Thoracic Society. Official Journal of the American Thoracic Society (28.)]
Figure 2. Figure 2. Recording of respiratory muscle activity and chest wall movement from a patient with scoliosis during sleep. There is widespread recruitment of accessory respiratory muscles during NREM sleep. Note also the activation of abdominal muscle activity (EMGABDO) during expiration. With the onset of REM sleep, a generalized reduction in inspiratory muscle activity occurs, with complete loss of abdominal EMG activity. This produces a marked reduction in chest wall movement with reduced airflow. [Adapted, with permission, from Becker et al. 1999; Breathing during sleep in patients with nocturnal desaturation. Am J Respir Crit Care Med, 159:112‐118. Reprinted with permission of the American Thoracic Society. Copyright © 2014 American Thoracic Society. Official Journal of the American Thoracic Society (28.)]
Figure 3. Figure 3. Resting breathing in an obese subject (BMI 42 kg/m2) when seated (left), supine without CPAP (middle) and with CPAP (right). The change in end‐expiratory esophageal baseline pressure is reflected by the horizontal dotted lines (nos 1‐3). There is PEEPi of around 6 cmH2O (vertical broken lines indicate the start of inspiratory flow, with the difference between horizontal line 2 and 4 indicating PEEPi). Zero flow is indicated by the horizontal line. In the right panel the patient is supine breathing with CPAP at 6 cmH2O. Neural respiratory drive to the diaphragm increases when changing posture from sitting to supine and reduces with CPAP; PEEPi is offset by CPAP and pressure swings of Poes and Pdi are smaller. CPAP, continuous positive airway pressure; EMGdi, electromyogram of the diaphragm; Poes, esophageal pressure; Pgas, gastric pressure; Pdi, transdiaphragmatic pressure (= Pgas‐Poes); PEEPi, intrinsic positive end expiratory pressure. [Reproduced, with permission, from Thorax, Steier et al. 64:719‐725, 2009 with permission from the BMJ Publishing Group Ltd (373.)]
Figure 4. Figure 4. High upper airway resistance is present in OHS patients not only when supine but during sitting. Total respiratory resistance (Rrs) at different oscillometry frequencies measured in awake sitting (A: upper panel) and supine (B: lower panel) in normal controls without OSA (Group I), in patients with moderate‐severe OSA and BMI ≤ 35 kg/m2 (Group IIa) or BMI > 35 kg/m2 (Group IIb) and patients with OHS (Group III). In sitting, there was no significant difference in Rrs at any oscillometry frequency between normal subjects and those with eucapnic OSAS, although Rrs at all frequencies was higher in the eucapnic OSAS compared to normal controls when supine (# P < 0.05). In patients with OHS, significantly higher Rrs was seen at all oscillometry frequencies compared to the other three groups in both sitting and when supine (* P < 0.05). [Reprinted from Respir Physiol Neurobiol. Vol 139, Lin et al, Oral airway resistance during wakefulness in eucapnic and hypercapnic sleep apnea syndrome. pg 215‐224, 2004, with permission from Elsevier. (226.)]
Figure 5. Figure 5. Conceptual illustration of the impact of the interapneic period on the accumulation of CO2 during sleep in patients with upper airway obstruction. The accumulation of CO2 during the hypopnea is offset during the interapnea period by sufficient ventilation over a sufficient period to eliminate the previous CO2 build up. However, if more CO2 is accumulated than can be excreted in the interapneic period, a small net rise in CO2 occurs. If this process is repeatedly frequently, a rise in PaCO2 will occur. [Reprinted with permission from Berger et al., 2002. J of Appl Physiol 93:917‐924) (38).]
Figure 6. Figure 6. In patients with a neuromuscular and chest wall disorder, the development of nocturnal hypoventilation portends the development of awake hypercapnia within the next 12 to 24 months. These 10 patients represent a control group allocated to no NIV over a 2 year period. The light‐colored bar represents the period of time the subject remained NIV‐free. By 12 months, 70% of the group required NIV, and by 24 months only one subject did not fulfill the criteria to commence NIV. (Reproduced from Thorax, Ward et al. 60:1019‐1024, 2005 with permission from the BMJ Publishing Group Ltd) (412).
Figure 7. Figure 7. Scatter plots (left‐hand panel) and receiver operator curves (right‐hand panel) for the predictive thresholds for Inspiratory vital capacity for the (A) onset of sleep‐disordered breathing (SDB), (B) continuous nocturnal hypoventilation and (C) diurnal respiratory failure. [Reproduced from Thorax, Ragette et al. 57:724‐728, 2002 (327) with permission from the BMJ Publishing Group Ltd.]
Figure 8. Figure 8. The “dose response” of NPPV showing the change in PaCO2 (left panel) and daytime sleepiness (ESS) (right panel) in patients with neuromuscular and restrictive thoracic disorders after 3 months of therapy. The dotted line represents the threshold below which NPPV does not have a consistent effect on PaCO2 or daytime sleepiness. Abbreviations: ESS—Epworth Sleepiness Scale; Use p.n.—use per night. [Reproduced from Thorax, Nickol et al. 60:754‐760, 2005 (281) with permission from the BMJ Publishing Group Ltd.]
Figure 9. Figure 9. Illustration of overventilation during the spontaneous mode of bilevel ventilation. Note the markedly reduced TcCO2 (right panel). Reducing CO2 below the apneic threshold will create central events which are associated with significant oxygen desaturation. Note the loss of diaphragm EMG activity along with absence of respiratory efforts on the thoracic and abdominal bands. This breathing abnormality occurs commonly at sleep onset or following arousal if excessive levels of pressure support are used in the spontaneous mode.
Figure 10. Figure 10. Examples of the more common asynchronies that can occur during NPPV therapy. Such events may not be apparent during wakefulness but can impact on the quality of ventilation during sleep. Panel A illustrates ineffective efforts caused by high leak. Small upward deflections can be seen in the airflow channel without a corresponding EPAP to IPAP transition on the pressure trace. Inspiratory efforts go unrewarded. Note also the generalized reduction in thoracic band movement during the leak period and a gradual fall in SpO2. The first, third, and fifth breaths on the airflow and pressure channels in Panel B show the machine has cycled twice with a very short expiratory period between the two during a single inspiratory effort (Thoracic and Abdominal bands). This can indicate pressurization is too short compared to inspiratory demand or the level of inspiratory support is too low (406). Panel C illustrates autotriggering—a rapid succession of multiple machine pressurizations clearly above the patient's native respiratory rate (151). This can occur if the inspiratory trigger has been set too low, especially in the presence of leak (406).


Figure 1. Changes in minute ventilation (V I), tidal volume (V T) and respiratory rate from wakefulness to sleep in patients with nocturnal hypoventilation (Desaturators), patients with eucapnic OSA during effective CPAP, and healthy normal controls. In the nocturnal hypoventilation group, the larger relative falls in V T were not offset by changes in respiratory rate so that an overall reduction in V I occurred. [Adapted, with permission, from Becker et al. 1999; Breathing during sleep in patients with nocturnal desaturation. Am J Respir Crit Care Med, 159:112‐118. Reprinted with permission of the American Thoracic Society. Copyright © 2014 American Thoracic Society. Official Journal of the American Thoracic Society (28.)]


Figure 2. Recording of respiratory muscle activity and chest wall movement from a patient with scoliosis during sleep. There is widespread recruitment of accessory respiratory muscles during NREM sleep. Note also the activation of abdominal muscle activity (EMGABDO) during expiration. With the onset of REM sleep, a generalized reduction in inspiratory muscle activity occurs, with complete loss of abdominal EMG activity. This produces a marked reduction in chest wall movement with reduced airflow. [Adapted, with permission, from Becker et al. 1999; Breathing during sleep in patients with nocturnal desaturation. Am J Respir Crit Care Med, 159:112‐118. Reprinted with permission of the American Thoracic Society. Copyright © 2014 American Thoracic Society. Official Journal of the American Thoracic Society (28.)]


Figure 3. Resting breathing in an obese subject (BMI 42 kg/m2) when seated (left), supine without CPAP (middle) and with CPAP (right). The change in end‐expiratory esophageal baseline pressure is reflected by the horizontal dotted lines (nos 1‐3). There is PEEPi of around 6 cmH2O (vertical broken lines indicate the start of inspiratory flow, with the difference between horizontal line 2 and 4 indicating PEEPi). Zero flow is indicated by the horizontal line. In the right panel the patient is supine breathing with CPAP at 6 cmH2O. Neural respiratory drive to the diaphragm increases when changing posture from sitting to supine and reduces with CPAP; PEEPi is offset by CPAP and pressure swings of Poes and Pdi are smaller. CPAP, continuous positive airway pressure; EMGdi, electromyogram of the diaphragm; Poes, esophageal pressure; Pgas, gastric pressure; Pdi, transdiaphragmatic pressure (= Pgas‐Poes); PEEPi, intrinsic positive end expiratory pressure. [Reproduced, with permission, from Thorax, Steier et al. 64:719‐725, 2009 with permission from the BMJ Publishing Group Ltd (373.)]


Figure 4. High upper airway resistance is present in OHS patients not only when supine but during sitting. Total respiratory resistance (Rrs) at different oscillometry frequencies measured in awake sitting (A: upper panel) and supine (B: lower panel) in normal controls without OSA (Group I), in patients with moderate‐severe OSA and BMI ≤ 35 kg/m2 (Group IIa) or BMI > 35 kg/m2 (Group IIb) and patients with OHS (Group III). In sitting, there was no significant difference in Rrs at any oscillometry frequency between normal subjects and those with eucapnic OSAS, although Rrs at all frequencies was higher in the eucapnic OSAS compared to normal controls when supine (# P < 0.05). In patients with OHS, significantly higher Rrs was seen at all oscillometry frequencies compared to the other three groups in both sitting and when supine (* P < 0.05). [Reprinted from Respir Physiol Neurobiol. Vol 139, Lin et al, Oral airway resistance during wakefulness in eucapnic and hypercapnic sleep apnea syndrome. pg 215‐224, 2004, with permission from Elsevier. (226.)]


Figure 5. Conceptual illustration of the impact of the interapneic period on the accumulation of CO2 during sleep in patients with upper airway obstruction. The accumulation of CO2 during the hypopnea is offset during the interapnea period by sufficient ventilation over a sufficient period to eliminate the previous CO2 build up. However, if more CO2 is accumulated than can be excreted in the interapneic period, a small net rise in CO2 occurs. If this process is repeatedly frequently, a rise in PaCO2 will occur. [Reprinted with permission from Berger et al., 2002. J of Appl Physiol 93:917‐924) (38).]


Figure 6. In patients with a neuromuscular and chest wall disorder, the development of nocturnal hypoventilation portends the development of awake hypercapnia within the next 12 to 24 months. These 10 patients represent a control group allocated to no NIV over a 2 year period. The light‐colored bar represents the period of time the subject remained NIV‐free. By 12 months, 70% of the group required NIV, and by 24 months only one subject did not fulfill the criteria to commence NIV. (Reproduced from Thorax, Ward et al. 60:1019‐1024, 2005 with permission from the BMJ Publishing Group Ltd) (412).


Figure 7. Scatter plots (left‐hand panel) and receiver operator curves (right‐hand panel) for the predictive thresholds for Inspiratory vital capacity for the (A) onset of sleep‐disordered breathing (SDB), (B) continuous nocturnal hypoventilation and (C) diurnal respiratory failure. [Reproduced from Thorax, Ragette et al. 57:724‐728, 2002 (327) with permission from the BMJ Publishing Group Ltd.]


Figure 8. The “dose response” of NPPV showing the change in PaCO2 (left panel) and daytime sleepiness (ESS) (right panel) in patients with neuromuscular and restrictive thoracic disorders after 3 months of therapy. The dotted line represents the threshold below which NPPV does not have a consistent effect on PaCO2 or daytime sleepiness. Abbreviations: ESS—Epworth Sleepiness Scale; Use p.n.—use per night. [Reproduced from Thorax, Nickol et al. 60:754‐760, 2005 (281) with permission from the BMJ Publishing Group Ltd.]


Figure 9. Illustration of overventilation during the spontaneous mode of bilevel ventilation. Note the markedly reduced TcCO2 (right panel). Reducing CO2 below the apneic threshold will create central events which are associated with significant oxygen desaturation. Note the loss of diaphragm EMG activity along with absence of respiratory efforts on the thoracic and abdominal bands. This breathing abnormality occurs commonly at sleep onset or following arousal if excessive levels of pressure support are used in the spontaneous mode.


Figure 10. Examples of the more common asynchronies that can occur during NPPV therapy. Such events may not be apparent during wakefulness but can impact on the quality of ventilation during sleep. Panel A illustrates ineffective efforts caused by high leak. Small upward deflections can be seen in the airflow channel without a corresponding EPAP to IPAP transition on the pressure trace. Inspiratory efforts go unrewarded. Note also the generalized reduction in thoracic band movement during the leak period and a gradual fall in SpO2. The first, third, and fifth breaths on the airflow and pressure channels in Panel B show the machine has cycled twice with a very short expiratory period between the two during a single inspiratory effort (Thoracic and Abdominal bands). This can indicate pressurization is too short compared to inspiratory demand or the level of inspiratory support is too low (406). Panel C illustrates autotriggering—a rapid succession of multiple machine pressurizations clearly above the patient's native respiratory rate (151). This can occur if the inspiratory trigger has been set too low, especially in the presence of leak (406).
References
 1.ATS/ERS. Statement on respiratory muscle testing. Am J Respir Crit Care Med 166: 518‐624, 2002.
 2. Aboussouan LS , Lewis RA . Sleep, respiration and ALS. J Neuro Sci 164: 1‐2, 1999.
 3. Adler D , Bridevaux PO , Contal O , Georges M , Dupuis‐Lozeron E , Claudel E , Pépin JL , Janssens JP . Pulse wave amplitude reduction: A surrogate marker of micro‐arousals associated with respiratory events occurring under non‐invasive ventilation? Respir Med 107: 2053‐2060, 2013.
 4. Adler D , Perrig S , Takahashi H , Espa F , Rodenstein D , Pepin JL , Janssens JP . Polysomnography in stable COPD under non‐invasive ventilation to reduce patient‐ventilator asynchrony and morning breathlessness. Sleep Breath 16: 1081‐1090, 2012.
 5. Al‐Kattan K , Simonds A , Chung KF , Kaplan DK . Kyphoscoliosis and Bronchial Torsion. Chest 111: 1134‐1137, 1997.
 6. Altose MD , McCauley WC , Kelsen SG , Cherniack NS . Effects of hypercapnia and inspiratory flow‐resistive loading on respiratory activity in chronic airways obstruction. J Clin Invest 59: 500‐507, 1977.
 7. Ambrogio C , Lowman X , Kuo M , Malo J , Prasad A , Parthasarathy S . Sleep and non‐invasive ventilation in patients with chronic respiratory failure. Intensive Care Med 35: 306‐313, 2009.
 8. Annane D , Quera‐Salva MA , Lofaso F , Vercken JB , Lesieur O , Fromageot C , Clair B , Gajdos P , Raphael JC . Mechanisms underlying effects of nocturnal ventilation on daytime blood gases in neuromuscular diseases. Eur Respir J 13: 157‐162, 1999.
 9. Anttalainen U , Saaresranta T , Vahlberg T , Polo O . Short‐term medroxyprogesterone acetate in postmenopausal women with sleep‐disordered breathing: A placebo‐controlled, randomized, double‐blind, parallel‐group study. Menopause 21: 361‐8, 2013.
 10. Arnulf I , Similowski T , Salachas F , Garma L , Meheri S , Attali V , Behin‐Bellhesen V , Meininger V , Derenne J . Sleep disorders and diaphragmatic function in patients with Amyotrophic Lateral Sclerosis. Am J Respir Crit Care Med 161: 849‐856, 2000.
 11. Atkeson AD , RoyChoudhury A , Harrington‐Moroney G , Shah B , Mitsumoto H , Basner RC . Patient‐ventilator asynchrony with nocturnal noninvasive ventilation in ALS. Neurology 77: 549‐555, 2011.
 12. Aubier M , Murciano D , Fournier M , Milic‐Emili J , Pariente R , Derenne JP . Central respiratory drive in acute respiratory failure of patients with chronic obstructive pulmonary disease. Am Rev Respir Dis 122: 191‐199, 1980.
 13. Ayappa I , Berger KI , Norman RG , Oppenheimer BW , Rapoport DM , Goldring RM . Hypercapnia and ventilatory periodicity in obstructive sleep apnea syndrome. Am J Respir Crit Care Med 166: 1112‐1115, 2002.
 14. Bach JR . Mechanical insufflation‐exsufflation. Comparison of peak expiratory flows with manually assisted and unassisted coughing techniques. Chest 104: 1553‐1562, 1993.
 15. Bach JR . Amyotrophic lateral sclerosis: Prolongation of life by noninvasive respiratory AIDS. Chest 122: 92‐98, 2002.
 16. Bach JR , Alba AS , Saporito LR . Intermittent positive pressure ventilation via the mouth as an alternative to tracheostomy for 257 ventilator users. Chest 103: 174‐182, 1993.
 17. Bach JR , Ishikawa Y , Kim H . Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest 112: 1024‐1028, 1997.
 18. Bach JR , Martinez D . Duchenne muscular dystrophy: Continuous noninvasive ventilatory support prolongs survival. Respiry Care 56: 744‐750, 2011.
 19. Bach JR , Rajaraman R , Ballanger F , Tzeng AC , Ishikawa Y , Kulessa R , Bansal T . Neuromuscular ventilatory insufficiency: Effect of home mechanical ventilator use v oxygen therapy on pneumonia and hospitalization rates. Am J Phys Med Rehabil 77: 8‐19, 1998.
 20. Bach JR , Saporito LR . Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure: A different approach to weaning. Chest 110: 1566‐1571, 1996.
 21. Ballard RD , Clover CW , Suh BY . Influence of sleep on respiratory function in emphysema. Am J Respir Crit Care Med 151: 945‐951, 1995.
 22. Banerjee D , Yee BJ , Piper AJ , Zwillich CW , Grunstein RR . Obesity hypoventilation syndrome: Hypoxemia during continuous positive airway pressure. Chest 131: 1678‐1684, 2007.
 23. Barbe F , Quera‐Salva MA , McCann C , Gajdos P , Raphael JC , de Lattre J , Agusti AG . Sleep‐related respiratory disturbances in patients with Duchenne muscular dystrophy. Eur Respir J 7: 1403‐1408, 1994.
 24. Basoglu OK , Tasbakan MS . Comparison of clinical characteristics in patients with obesity hypoventilation syndrome and obese obstructive sleep apnea syndrome: A case‐control study. Clil Respir J 8: 167‐174, 2014.
 25. Baydur A . Respiratory muscle strength and control of ventilation in patients with neuromuscular disease. Chest 99: 330‐338, 1991.
 26. Baydur A , Layne E , Aral H , Krishnareddy N , Topacio R , Frederick G , Bodden W . Long term non‐invasive ventilation in the community for patients with musculoskeletal disorders: 46 year experience and review. Thorax 55: 4‐11, 2000.
 27. Bayliss DA , Millhorn DE . Central neural mechanisms of progesterone action: Application to the respiratory system. J Appl Physiol 73: 393‐404, 1992.
 28. Becker HF , Piper AJ , Flynn WE , McNamara SG , Grunstein RR , Peter JH , Sullivan CE . Breathing during sleep in patients with nocturnal desaturation. Am J Respir Crit Care Med 159: 112‐118, 1999.
 29. Bednarek M , Plywaczewski R , Jonczak L , Zielinski J . There is no relationship between chronic obstructive pulmonary disease and obstructive sleep apnea syndrome: A population study. Respiration 72: 142‐149, 2005.
 30. Bégin P , Grassino A . Inspiratory muscle dysfunction and chronic hypercapnia in chronic obstructive pulmonary dDisease. Am Rev Respiry Dis 143: 905‐912, 1991.
 31. Begin P , Mathieu J , Almirall J , Grassino A . Relationship between chronic hypercapnia and inspiratory‐muscle weakness in myotonic dystrophy. Am J Respir Crit Care Med 156: 133‐139, 1997.
 32. Begin R , Bureau MA , Lupien L , Lemieux B . Control and modulation of respiration in Steinert's myotonic dystrophy. Am Rev Respir Dis 121: 281‐289, 1980.
 33. Begin R , Bureau MA , Lupien L , Lemieux B . Control of breathing in Duchenne's muscular dystrophy. Am J Med 69: 227‐234, 1980.
 34. Benditt JO . Full‐time noninvasive ventilation: Possible and desirable. Respir Care 51: 1005‐1012.
 35. Bennett JR , Dunroy HM , Corfield DR , Hart N , Simonds AK , Polkey MI , Morrell MJ . Respiratory muscle activity during REM sleep in patients with diaphragm paralysis. Neurology 62: 134‐137, 2004.
 36. Berg G , Delaive K , Manfreda J , Walld R , Kryger MH . The use of health‐care resources in obesity‐hypoventilation syndrome. Chest 120: 377‐383, 2001.
 37. Berger KI , Ayappa I , Chatr‐Amontri B , Marfatia A , Sorkin IB , Rapoport DM , Goldring RM . Obesity hypoventilation syndrome as a spectrum of respiratory disturbances during sleep. Chest 120: 1231‐1238, 2001.
 38. Berger KI , Ayappa I , Sorkin IB , Norman RG , Rapoport DM , Goldring RM . Postevent ventilation as a function of CO(2) load during respiratory events in obstructive sleep apnea. J Appl Physiol 93: 917‐924, 2002.
 39. Berger KI , Goldring RM , Rapoport DM . Obesity hypoventilation syndrome. Semin Respir Crit Care Med 30: 253‐261, 2009.
 40. Bergofsky EH . Respiratory failure in disorders of the thoracic cage. Am Rev Respir Dis 119: 643‐669, 1979.
 41. Bergofsky EH , Turino GM , Fishman AP . Cardiorespiratory failure in kyphoscoliosis. Medicine (Baltimore) 38: 263‐317, 1959.
 42. Berlowitz DJ , Detering K , Schachter L . A retrospective analysis of sleep quality and survival with domiciliary ventilatory support in motor neuron disease. Amyotroph Lateral Scler 7: 100‐106, 2006.
 43. Berry R , Chediak A , Brown L , Finder J , Gozal D , Iber C , Kushida C , Morgenthaler T , Rowley J , Davidson‐Ward S . Best clinical practices for the sleep center adjustment of noninvasive positive pressure ventilation (NPPV) in stable chronic alveolar hypoventilation syndromes. J Clin Sleep Med 6: 491‐509, 2010.
 44. Berry RB . Noninvasive positive pressure ventilation titration and treatment initiation for chronic hypoventilation syndromes. Sleep Med Clin 5: 485‐505, 2010.
 45. Berthon‐Jones M , Sullivan CE . Time course of change in ventilatory response to CO2 with long‐term CPAP therapy for Obstructive sleep apnea. Am Rev Respir Dis 135: 144‐147, 1987.
 46. Berthon‐Jones M , Sullivan CE . Ventilation and arousal responses to hypercapnia in normal sleeping humans. J Appl Physiol 57: 59‐67, 1984.
 47. Berthon‐Jones M , Sullivan CE . Ventilatory and arousal responses to hypoxia in sleeping humans. Am Rev Respir Dis 125: 632‐639, 1982.
 48. Birchfield RI , Sieker HO , Heyman A . Alterations in respiratory function during natural sleep. J Lab Clin Med 54: 216‐222, 1959.
 49. Borel JC , Sabil A , Janssens J‐P , Couteau M , Boulon L , Lévy P , Pépin J‐L . Intentional leaks in industrial masks have a significant impact on efficacy of bilevel noninvasive ventilation: A bench test study. Chest 135: 669‐677, 2009.
 50. Borel JC , Wuyam B , Chouri‐Pontarollo N , Deschaux C , Levy P , Pepin JL . During exercise non‐invasive ventilation in chronic restrictive respiratory failure. Respir Med 102: 711‐719, 2008.
 51. Borg G . Psychophysical bases of perceived exertion. Med Sci Sports Exerc 14: 377‐381, 1982.
 52. Bourke SC , Gibson GJ . Sleep and breathing in neuromuscular disease. Eur Respir J 19: 1194‐1201, 2002.
 53. Bourke SC , Tomlinson M , Williams TL , Bullock RE , Shaw PJ , Gibson GJ . Effects of non‐invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: A randomised controlled trial. Lancet Neurol 5: 140‐147, 2006.
 54. Boyer J , Amin N , Taddonio R , Dozor AJ . Evidence of airway obstruction in children with idiopathic scoliosis. Chest 109: 1532‐1535, 1996.
 55. Braun NMT , Arora NS , Rochester DF . Respiratory muscle and pulmonary function in polymyositis and other proximal myopathies. Thorax 38: 616‐623, 1983.
 56. Bredin CP . Pulmonary function in long‐term survivors of thoracoplasty. Chest 95: 18‐20, 1989.
 57. Brijker F , van den Elshout FJ , Heijdra YF , Folgering HT . Underestimation of nocturnal hypoxemia due to monitoring conditions in patients with COPD. Chest 119: 1820‐1826, 2001.
 58. Budweiser S , Heinemann F , Fischer W , Dobroschke J , Wild PJ , Pfeifer M . Impact of ventilation parameters and duration of ventilator use on non‐invasive home ventilation in restrictive thoracic disorders. Respiration 73: 488‐494, 2006.
 59. Budweiser S , Heinemann F, Fischer W , Dobroschke J , Pfeifer M . Long‐term reduction of hyperinflation in stable COPD by non‐invasive nocturnal home ventilation. Respir Med 99: 976‐984, 2005.
 60. Budweiser S , Hitzl AP , Jorres RA , Heinemann F , Arzt M , Schroll S , Pfeifer M . Impact of noninvasive home ventilation on long‐term survival in chronic hypercapnic COPD: A prospective observational study. Intern J Clin Pract 61: 1516‐1522, 2007.
 61. Budweiser S , Jorres RA , Criee CP , Langer V , Heinemann F , Hitzl AP , Schmidbauer K , Windisch W , Pfeifer M . Prognostic value of mouth occlusion pressure in patients with chronic ventilatory failure. Respir Med 101: 2343‐2351, 2007.
 62. Budweiser S , Jorres RA , Riedl T , Heinemann F , Hitzl AP , Windisch W , Pfeifer M . Predictors of survival in COPD patients with chronic hypercapnic respiratory failure receiving noninvasive home ventilation. Chest 131: 1650‐1658, 2007.
 63. Burki NK , Baker RW . Ventilatory regulation in eucapnic morbid obesity. Am Rev Respir Dis 129: 538‐543, 1984.
 64. Buyse B , Meersseman W , Demedts M . Treatment of chronic respiratory failure in kyphoscoliosis: Oxygen or ventilation? Eur Respir J 22: 525‐528, 2003.
 65. Bye PT , Ellis ER , Issa FG , Donnelly PM , Sullivan CE . Respiratory failure and sleep in neuromuscular disease. Thorax 45: 241‐247, 1990.
 66. Calderini E , Confalonieri M , Puccio PG , Francavilla N , Stella L , Gregoretti C . Patient‐ventilator asynchrony during noninvasive ventilation: The role of expiratory trigger. Intensive Care Med 25: 662‐667, 1999.
 67. Campo A , Fruhbeck G , Zulueta JJ , Iriarte J , Seijo LM , Alcaide AB , Galdiz JB , Salvador J . Hyperleptinemia, respiratory drive and hypercapnic response in obese patients. Eur Respir J 30: 223‐231, 2007.
 68. Carlucci A , Pisani L , Ceriana P , Malovini A , Nava S . Patient‐ventilator asynchronies: May the respiratory mechanics play a role? Crit Care 17: R54, 2013.
 69. Carlucci A , Schreiber A , Mattei A , Malovini A , Bellinati J , Ceriana P , Gregoretti C . The configuration of bi‐level ventilator circuits may affect compensation for non‐intentional leaks during volume‐targeted ventilation. Intensive Care Med 39: 59‐65, 2013.
 70. Cejudo P , López‐Márquez I , Lopez‐Campos J , Ortega F , Bernal CC , Márquez E , Tallón R , Sánchez‐Riera H, Barrot E . Factors associated with quality of life in patients with chronic respiratory failure due to kyphoscoliosis. Disabil Rehabil 31: 928‐934, 2009.
 71. Chan CS , Bye PT , Woolcock AJ , Sullivan CE . Eucapnia and hypercapnia in patients with chronic airflow limitation. The role of the upper airway. Am Rev Respir Dis 141: 861‐865, 1990.
 72. Chaouat A , Weitzenblum E , Kessler R , Charpentier C , Ehrhart M , Levi‐Valensi P , Zielinski J , Delaunois L , Cornudella R , Moutinho dos Santos J . Sleep‐related O2 desaturation and daytime pulmonary haemodynamics in COPD patients with mild hypoxaemia. Eur Respir J 10: 1730‐1735, 1997.
 73. Chaouat A , Weitzenblum E , Krieger J , Ifoundza T , Oswald M , Kessler R . Association of chronic obstructive pulmonary disease and sleep apnea syndrome. Am J Respir Crit Care Med 151: 82‐86, 1995.
 74. Chaouat A , Weitzenblum E , Krieger J , Sforza E , Hammad H , Oswald M , Kessler R . Prognostic value of lung function and pulmonary haemodynamics in OSA patients treated with CPAP. Eur Respir J 13: 1091‐1096, 1999.
 75. Chatwin M , Ross E , Hart N , Nickol AH , Polkey MI , Simonds AK . Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness. Eur Respir J 21: 502‐508, 2003.
 76. Chau EH , Lam D , Wong J , Mokhlesi B , Chung F . Obesity hypoventilation syndrome: A review of epidemiology, pathophysiology, and perioperative considerations. Anesthesiology 117: 188‐205, 2012.
 77. Chin K , Hirai M , Kuriyama T , Fukui M , Kuno K , Sagawa Y , Ohi M . Changes in the arterial PCO2 during a single night's sleep in patients with obstructive sleep apnea. Intern Med 36: 454‐460, 1997.
 78. Chlif M , Keochkerian D , Choquet D , Vaidie A , Ahmaidi S . Effects of obesity on breathing pattern, ventilatory neural drive and mechanics. Respir Physiol Neurobiol 168: 198‐202, 2009.
 79. Chouri‐Pontarollo N , Borel JC , Tamisier R , Wuyam B , Levy P , Pepin JL . Impaired objective daytime vigilance in obesity‐hypoventilation syndrome: Impact of noninvasive ventilation. Chest 131: 148‐155, 2007.
 80. Chung SA , Yuan H , Chung F . A systemic review of obstructive sleep apnea and its implications for anesthesiologists. Anesth Analg 107: 1543‐1563, 2008.
 81. Clini E , Sturani C , Rossi A , Viaggi S , Corrado A , Donner CF , Ambrosino N . The Italian multicentre study on noninvasive ventilation in chronic obstructive pulmonary disease patients. Eur Respir J 20: 529‐538, 2002.
 82. Collet F , Mallart A , Bervar JF , Bautin N , Matran R , Pattou F , Romon M , Perez T . Physiologic correlates of dyspnea in patients with morbid obesity. Int J Obes 31: 700‐706, 2006.
 83. Collins LC , Hoberty PD , Walker JF , Fletcher EC , Peiris AN . The effect of body fat distribution on pulmonary function tests. Chest 107: 1298‐1302, 1995.
 84. Contal O , Adler D , Borel JC , Espa F , Perrig S , Rodenstein D , Pepin JL , Janssens JP . Impact of different back‐up respiratory rates on the efficacy of non‐invasive positive pressure ventilation in obesity hypoventilation syndrome: A randomized trial. Chest 143: 37‐46, 2013.
 85. Contal O , Carnevale C , Borel J‐C , Sabil A , Tamisier R , Lévy P , Janssens J‐P , Pépin J‐L . Pulse transit time as a measure of respiratory effort under noninvasive ventilation. Eur Respiry J 41: 346‐353, 2013.
 86. Contal O , Janssens JP , Dury M , Delguste P , Aubert G , Rodenstein D . Sleep in ventilatory failure in restrictive thoracic disorders. Effects of treatment with non invasive ventilation. Sleep Med 12: 373‐377, 2011.
 87. Contal O , Vignaux L , Combescure C , Pepin J , Jolliet P , Janssens J . Monitoring of noninvasive ventilation by built‐in software of home bilevel ventilators: A bench study. Chest 141: 469‐476, 2012.
 88. Cooper KR , Phillips BA . Effect of short‐term sleep loss on breathing. J Appl Physiol 53: 855‐858, 1982.
 89. Crescimanno G , Canino M , Marrone O . Asynchronies and sleep disruption in neuromuscular patients under home noninvasive ventilation. Respir Med 106: 1478‐1485, 2012.
 90. Crescimanno G , Marrone O , Vianello A . Efficacy and comfort of volume‐guaranteed pressure support in patients with chronic ventilatory failure of neuromuscular origin. Respirology 16: 672‐679, 2011.
 91. Crummy F , Piper AJ , Naughton MT . Obesity and the lung: 2. Obesity and sleep‐disordered breathing. Thorax 63: 738‐746, 2008.
 92. Cuvelier A , Grigoriu B , Molano LC , Muir JF . Limitations of transcutaneous carbon dioxide measurements for assessing long‐term mechanical ventilation. Chest 127: 1744‐1748, 2005.
 93. de Lucas‐Ramos P , de Miguel‐Díez J , Santacruz‐Siminiani A , González‐Moro JMR , Buendía‐García MJ , Izquierdo‐Alonso JL . Benefits at 1 year of nocturnal intermittent positive pressure ventilation in patients with obesity‐hypoventilation syndrome. Respir Med 98: 961‐967, 2004.
 94. De Miguel Diez J , De Lucas Ramos P , Perez Parra JJ , Buendia Garcia MJ , Cubillo Marcos JM , Gonzalez‐Moro JM . Analysis of withdrawal from noninvasive mechanical ventilation in patients with obesity‐hypoventilation syndrome. Medium term results. Arch Bronconeumol 39: 292‐297, 2003.
 95. De Troyer A , Borenstein S , Cordier R . Analysis of lung volume restriction in patients with respiratory muscle weakness. Thorax 35: 603‐610, 1980.
 96. De Troyer A , Leeper J , McKenzie D , Gandevia S . Neural drive to the diaphragm in patients with severe COPD. Am J Respir Crit Care Med 155: 1335‐1340, 1997.
 97. Dean AC , Graham BA , Dalakas M , Sato S . Sleep apnea in patients with postpolio syndrome. Ann Neurol 43: 661‐664, 1998.
 98. Dellborg C , Olofson J , Hamnegard CH , Skoogh BE , Bake B . Ventilatory response to CO2 re‐breathing before and after nocturnal nasal intermittent positive pressure ventilation in patients with chronic alveolar hypoventilation. Respir Med 94: 1154‐1160, 2000.
 99. Dellweg D , Schonhofer B , Haidl PM , Barchfeld T , Wenzel MD , Appelhans P , Kohler D . Short‐term effect of controlled instead of assisted noninvasive ventilation in chronic respiratory failure due to chronic obstructive pulmonary disease. Respir Care 52: 1734‐1740, 2007.
 100. Dematteis M , Pepin JL , Jeanmart M , Deschaux C , Labarre‐Vila A , Levy P . Charcot‐Marie‐Tooth disease and sleep apnoea syndrome: A family study. Lancet 357: 267‐272, 2001.
 101. Diaz O , Begin P , Andresen M , Prieto ME , Castillo C , Jorquera J , Lisboa C . Physiological and clinical effects of diurnal noninvasive ventilation in hypercapnic COPD. Eur Respir J 26: 1016‐1023, 2005.
 102. Diaz O , Begin P , Torrealba B , Jover E , Lisboa C . Effects of noninvasive ventilation on lung hyperinflation in stable hypercapnic COPD. Eur Respir J 20: 1490‐1498, 2002.
 103. Dick CR , Liu Z , Sassoon CS , Berry RB , Mahutte CK . O2‐induced change in ventilation and ventilatory drive in COPD. Am J Respir Crit Care Med 155: 609‐614, 1997.
 104. Dohna‐Schwake C , Ragette R , Teschler H , Voit T , Mellies U . Predictors of severe chest infections in pediatric neuromuscular disorders. Neuromuscul Disord 16: 325‐328, 2006.
 105. Douglas NJ , Calverley PMA , Leggett RJE , Brash HM , Flenley DC , Brezinova V . Transient hypoxaemia during sleep in chronic bronchitis and emphysema. Lancet i: 1‐4, 1979.
 106. Douglas NJ , White DP , Pickett CK , Weil JV , Zwillich CW . Respiration during sleep in normal man. . Thorax 37: 840‐844, 1982.
 107. Douglas NJ , White DP , Weil JV , Pickett CK , Martin RJ , Hudgel DW , Zwillich CW . Hypoxic ventilatory response decreases during sleep in normal men. Am Rev Respir Dis 125: 286‐289, 1982.
 108. Douglas NJ , White DP , Weil JV , Pickett CK , Zwillich CW . Hypercapnic ventilatory response in sleeping adults. Am Rev Respir Dis 126: 758‐762, 1982.
 109. Dowell AR , Heyman A , Sieker HO , Tripathy K . Effect of aminophylline on respiratory‐center sensitivity in Cheynestokes respiration and in pulmonary emphysema. N Engl J Med 273: 1447‐1453, 1965.
 110. Dreher M , Ekkernkamp E , Walterspacher S , Walker D , Schmoor C , Storre JH , Windisch W . Noninvasive ventilation in copd: Impact of inspiratory pressure levels on sleep quality. Chest 140: 939‐945, 2011.
 111. Dreher M , Rauter I , Storre JH , Geiseler J , Windisch W . When should home mechanical ventilation be started in patients with different neuromuscular disorders? Respirology 12: 749‐753, 2007.
 112. Dreher M , Storre JH , Schmoor C , Windisch W . High‐intensity versus low‐intensity non‐invasive ventilation in patients with stable hypercapnic COPD: A randomised crossover trial. Thorax 65: 303‐308, 2010.
 113. Duiverman ML , Bladder G , Meinesz AF , Wijkstra PJ . Home mechanical ventilatory support in patients with restrictive ventilatory disorders: A 48‐year experience. Respir Med 100: 56‐65, 2006.
 114. Eagle M , Baudouin SV , Chandler C , Giddings DR , Bullock R , Bushby K . Survival in Duchenne muscular dystrophy: Improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromuscul Disord 12: 926‐929, 2002.
 115. El‐Gamal H , Khayat A , Shikora S , Unterborn JN . Relationship of dyspnea to respiratory drive and pulmonary function tests in obese patients before and after weight loss. Chest 128: 3870‐3874, 2005.
 116. Elliott M , Simonds A . Nocturnal assisted ventilation using bilevel positive airway pressure: The effect of expiratory positive airway pressure. Eur Respiy J 8: 436‐440, 1995.
 117. Elliott MW . Domiciliary non‐invasive ventilation in stable COPD? Thorax 64: 553‐556, 2009.
 118. Elliott MW . Non‐invasive ventilation during sleep: Time to define new tools in the systematic evaluation of the technique. Thorax 66: 82‐84, 2011.
 119. Elliott MW , Mulvey DA , Moxham J , Green M , Branthwaite MA . Domiciliary nocturnal nasal intermittent positive pressure ventilation in COPD: Mechanisms underlying changes in arterial blood gas tensions. Eur Respir J 4: 1044‐1052, 1991.
 120. Ellis ER , Bye PTP , Bruderer JW , Sullivan CE . Treatment of respiratory failure during sleep in patients with neuromuscular disease. Am Rev Respir Dis 135: 148‐152, 1987.
 121. Ellis ER , Grunstein RR , Chan S , Bye PT , Sullivan CE . Noninvasive ventilatory support during sleep improves respiratory failure in kyphoscoliosis. Chest 94: 811‐815, 1988.
 122. Estenne M , Derom E , de Troyer A . Neck and abdominal muscle activity in patients with severe thoracic scoliosis. Am J Respir Crit Care Med 158: 452‐457, 1998.
 123. Evans R , Catapano M , Brooks D , Goldstein R , Avendano M . Family caregiver perspectives on caring for ventilator‐assisted individuals at home. Can Respir J 19: 373‐379, 2012.
 124. Fadell EJ , Richman AD , Ward WW , Hendon JR . Fatty infiltration of respiratory muscles in the Pick‐wickian syndrome. N Engl J Med 266: 861‐863, 1962.
 125. Falsaperla R , Wenzel A , Pavone P , Di Mauro C , Vitaliti G . Polysomnographic evaluation of non‐invasive ventilation in children with neuromuscular disease. Respirology 19: 80‐84, 2014.
 126. Fanfulla F , Delmastro M , Berardinelli A , Lupo ND , Nava S . Effects of different ventilator settings on sleep and inspiratory effort in patients with neuromuscular disease. Am J Respir Crit Care Med 172: 619‐624, 2005.
 127. Fanfulla F , Taurino AE , Lupo ND , Trentin R , D'Ambrosio C , Nava S . Effect of sleep on patient/ventilator asynchrony in patients undergoing chronic non‐invasive mechanical ventilation. Respir Med 101: 1702‐1707, 2007.
 128. Farkas GA , Gosselin LE , Zhan WZ , Schlenker EH , Sieck GC . Histochemical and mechanical properties of diaphragm muscle in morbidly obese Zucker rats. J Appl Physiol 77: 2250‐2259, 1994.
 129. Fauroux B , Leroux K , Pépin J‐L , Lofaso F , Louis B . Are home ventilators able to guarantee a minimal tidal volume? Intensive Care Med 36: 1008‐1014, 2010.
 130. Fauroux B , Louis B , Hart N , Essouri S , Leroux K , Clément A , Polkey M , Lofaso F . The effect of back‐up rate during non‐invasive ventilation in young patients with cystic fibrosis. Intensive Care Med 30: 673‐681, 2004.
 131. Fauroux B , Nicot F , Essouri S , Hart N , Clement A , Polkey MI , Lofaso F . Setting of noninvasive pressure support in young patients with cystic fibrosis. Eur Respir J 24: 624‐630, 2004.
 132. Fauroux B , Pigeot J , Polkey MI , Isabey D , Clement A , Lofaso F . In vivo physiologic comparison of two ventilators used for domiciliary ventilation in children with cystic fibrosis. Crit Care Med 29: 2097‐2105, 2001.
 133. Ferguson KA , Ahmad D , George CFP , Strong MJ . Sleep‐disordered breathing in Amyotrophic Lateral Sclerosis. Chest 110: 664‐669, 1996.
 134. Fernandez‐Alvarez R , Rubinos‐Cuadrado G , Cabrera‐Lacalzada C , Galindo‐Morales R , Gullon‐Blanco JA , Gonzalez‐Martin I . Home mechanical ventilation: Dependency and burden of care in the home. Arch Bronconeumol 45: 383‐386, 2009.
 135. Fletcher EC , Miller J , Divine GW , Fletcher JG , Miller T . Nocturnal oxyhemoglobin desaturation in COPD patients with arterial oxygen tensions above 60 mm Hg. Chest 92: 604‐608, 1987.
 136. Fromageot C , Lofaso F , Annane D , Falaize L , Lejaille M , Clair B , Gajdos P , Raphaël JC . Supine fall in lung volumes in the assessment of diaphragmatic weakness in neuromuscular disorders. Arch Phys Med Rehabil 82: 123‐128, 2001.
 137. Fu ES , Downs JB , Schweiger JW , Miguel RV , Smith RA . Supplemental oxygen impairs detection of hypoventilation by pulse oximetry. Chest 126: 1552‐1558, 2004.
 138. Garcia‐Rio F , Pino JM , Ruiz A , Diaz S , Prados C , Villamor J . Accuracy of noninvasive estimates of respiratory muscle effort during spontaneous breathing in restrictive diseases. J Appl Physiol 95: 1542‐1549, 2003.
 139. Garner DJ , Berlowitz DJ , Douglas J , Harkness N , Howard M , McArdle N , Naughton MT , Neill A , Piper A , Yeo A , Young A . Home mechanical ventilation in Australia and New Zealand. Eur Respir J 41: 39‐45, 2013.
 140. Gay PC , Edmonds LC . Severe hypercapnia after low‐flow oxygen therapy in patients with neuromuscular disease and diaphragmatic dysfunction. Mayo Clin Proc 70: 327‐330, 1995.
 141. Gay PC , Westbrook PR , Daube JR , Litchy WJ , Windebank AJ , Iverson R . Effects of alterations in pulmonary function and sleep variables on survival in patients with amyotrophic lateral sclerosis. Mayo Clin Proc 66: 686‐694, 1991.
 142. Gigliotti F , Pizzi A , Duranti R , Gorini M , Iandelli I , Scano G . Control of breathing in patients with limb girdle dystrophy: A controlled study. Thorax 50: 962‐968, 1995.
 143. Gilmartin JJ , Cooper BG , Griffiths CJ , Walls TJ , Veale D , Stone TN , Osselton JW , Hudgson P , Gibson GJ . Breathing during sleep in patients with myotonic dystrophy and non‐myotonic respiratory muscle weakness. Q J Med 78: 21‐31, 1991.
 144. Goldring RM , Turino GM , Heinemann HO . Respiratory‐renal adjustments in chronic hypercapnia in man. Extracellular bicarbonate concentration and the regulation of ventilation. Am J Med 51: 772‐784, 1971.
 145. Gonzalez‐Bermejo J , Perrin C , Janssens JP , Pepin JL , Mroue G , Leger P , Langevin B , Rouault S , Rabec C , Rodenstein D . Proposal for a systematic analysis of polygraphy or polysomnography for identifying and scoring abnormal events occurring during non‐invasive ventilation. Thorax 67: 546‐552, 2012.
 146. Gonzalez‐Bermejo J , Rabec C , Janssens JP , Perrin C , Langevin B , Pepin JL , Rodenstein D . Noninvasive ventilation inefficacy due to technically incompatible ventilator settings. Intensive Care Med 39: 1154‐1156, 2013.
 147. Gonzalez C , Ferris G , Diaz J , Fontana I , Nunez J , Marin J . Kyphoscoliotic ventilatory insufficiency: Effects of long‐term intermittent positive‐pressure ventilation. Chest 124: 857‐862, 2003.
 148. Gould GA , Gugger M , Molloy J , Tsara V , Shapiro CM , Douglas NJ . Breathing pattern and eye movement density during REM sleep in humans. Am Rev Respir Dis 138: 874‐877, 1988.
 149. Grinman S , Whitelaw WA . Pattern of breathing in a case of generalized respiratory muscle weakness. Chest 84: 770‐772, 1983.
 150. Guilleminault C , Kurland G , Winkle R , Miles LE . Severe kyphoscoliosis, breathing, and sleep: The “Quasimodo” syndrome during sleep. Chest 79: 626‐630, 1981.
 151. Guo YF , Sforza E , Janssens JP . Respiratory patterns during sleep in obesity‐hypoventilation patients treated with nocturnal pressure support: A preliminary report. Chest 131: 1090‐1099, 2007.
 152. Gustafson T , Franklin KA , Midgren B , Pehrsson K , Ranstam J , Strom K . Survival of patients with kyphoscoliosis receiving mechanical ventilation or oxygen at home. Chest 130: 1828‐1833, 2006.
 153. Gutierrez M , Beroiza T , Contreras G , Diaz O , Cruz E , Moreno R , Lisboa C . Weekly cuirass ventilation improves blood gases and inspiratory muscle strength in patients with chronic air‐flow limitation and hypercarbia. Am Rev Respir Dis 138: 617‐623, 1988.
 154. Hamada S , Ishikawa Y , Aoyagi T , Ishikawa Y , Minami R , Bach JR . Indicators for ventilator use in Duchenne muscular dystrophy. Respir Med 105: 625‐629, 2011.
 155. Han F , Chen E , Wei H , He Q , Ding D , Strohl KP . Treatment effects on carbon dioxide retention in patients with obstructive sleep apnea‐hypopnea syndrome. Chest 119: 1814‐1819, 2001.
 156. Hannan LM , Dominelli GS , Chen YW , Darlene Reid W , Road J . Systematic review of non‐invasive positive pressure ventilation for chronic respiratory failure. Respir Med 108: 229‐243, 2014.
 157. Hart N , Polkey MI , Sharshar T , Falaize L , Fauroux B , Raphael JC , Lofaso F . Limitations of sniff nasal pressure in patients with severe neuromuscular weakness. J Neurol Neurosurg Psychiatry 74: 1685‐1687, 2003.
 158. Hawrylkiewicz I , Sliwinski P , Gorecka D , Plywaczewski R , Zielinski J . Pulmonary haemodynamics in patients with OSAS or an overlap syndrome. Monaldi Arch Chest Dis 61: 148‐152, 2004.
 159. Heijdra YF , Dekhuijzen PNR , Van Herwaarden CLA , Folgering HTM . Nocturnal saturation and respiratory muscle function in patients with chronic obstructive pulmonary disease. Thorax 50: 610‐612, 1995.
 160. Heinemann F , Budweiser S , Dobroschke J , Pfeifer M . Non‐invasive positive pressure ventilation improves lung volumes in the obesity hypoventilation syndrome. Respir Med 101: 1229‐1235, 2007.
 161. Held M , Walthelm J , Baron S , Roth C , Jany B . Functional impact of pulmonary hypertension due to hypoventilation and changes under noninvasive ventilation. Eur Respir J 43: 156‐165, 2014.
 162. Heritier Barras AC , Adler D , Iancu Ferfoglia R , Ricou B , Gasche Y , Leuchter I , Hurst S , Escher M , Pollak P , Janssens JP . Is tracheostomy still an option in amyotrophic lateral sclerosis? Reflections of a multidisciplinary work group. Swiss Med Wkly 143: w13830, 2013.
 163. Hess DR . Patient‐ventilator interaction during noninvasive ventilation. Respir Care 56: 153‐165; discussion 165‐157, 2011.
 164. Hirabayashi M , Fujiwara C , Ohtani N , Kagawa S , Kamide M . Transcutaneous PCO2 monitors are more accurate than end‐tidal PCO2 monitors. J Anesth 23: 198‐202, 2009.
 165. Hlavac MC , Catcheside PG , McDonald R , Eckert DJ , Windler S , McEvoy RD . Hypoxia impairs the arousal response to external resistive loading and airway occlusion during sleep. Sleep 29: 624‐631, 2006.
 166. Holle RH , Schoene RB , Pavlin EJ . Effect of respiratory muscle weakness on P0.1 induced by partial curarization. J Appl Physiol 57: 1150‐1157, 1984.
 167. Hollier CA , Harmer AR , Maxwell LJ , Menadue C , Willson GN , Unger G , Flunt D , Black DA , Piper AJ . Moderate concentrations of supplemental oxygen worsen hypercapnia in obesity hypoventilation syndrome: A randomised crossover study. Thorax 69: 346‐353, 2013.
 168. Hollier CA , Maxwell LJ , Harmer AR , Menadue C , Piper AJ , Black DA , Willson GN , Alison JA . Validity of arterialised‐venous PCO2, pH and bicarbonate in obesity hypoventilation syndrome. Respir Physiol Neurobiol 188: 165‐171, 2013.
 169. Hsu AA , Staats BA . “Postpolio” sequelae and sleep‐related disordered breathing. Mayo Clin Proc 73: 216‐224, 1998.
 170. Hudgel DW , Martin RJ , Capheart M , Hohnson B , Hill P . Contribution of hypoventilation to sleep oxygen desaturation in chronic obstructive pulmonary disease. J Appl Physiol 55: 669‐677, 1983.
 171. Hudgel DW , Mulholland M , Hendricks C . Neuromuscular and mechanical responses to inspiratory resistive loading during sleep. J Appl Physiol 63: 603‐608, 1987.
 172. Hukins CA , Hillman DR . Daytime predictors of sleep hypoventilation in Duchenne muscular dystrophy. Am J Respir Crit Care Med 161: 166‐170, 2000.
 173. Jackson CE , Rosenfeld J , Moore DH , Bryan WW , Barohn RJ , Wrench M , Myers D , Heberlin L , King R , Smith J , Gelinas D , Miller RG . A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of hypoventilation in ALS/MND patients. J Neurol Sci 191: 75‐78, 2001.
 174. Jager L , Franklin KA , Midgren B , Lofdahl K , Strom K . Increased survival with mechanical ventilation in posttuberculosis patients with the combination of respiratory failure and chest wall deformity. Chest 133: 156‐160, 2008.
 175. Janssens JP , Borel JC , Pepin JL . Nocturnal monitoring of home non‐invasive ventilation: The contribution of simple tools such as pulse oximetry, capnography, built‐in ventilator software and autonomic markers of sleep fragmentation. Thorax 66: 438‐445, 2011.
 176. Janssens JP , Derivaz S , Breitenstein E , De Muralt B , Fitting JW , Chevrolet JC , Rochat T . Changing patterns in long‐term noninvasive ventilation: A 7‐year prospective study in the Geneva Lake area. Chest 123: 67‐79, 2003.
 177. Janssens JP , Metzger M , Sforza E . Impact of volume targeting on efficacy of bi‐level non‐invasive ventilation and sleep in obesity‐hypoventilation. Respir Med 103: 165‐172, 2009.
 178. Janssens JP , Penalosa B , Degive C , Rabeus M , Rochat T . Quality of life of patients under home mechanical ventilation for restrictive lung diseases: A comparative evaluation with COPD patients. Monaldi Arch Chest Dis 51: 178‐184, 1996.
 179. Janssens JP , Perrin E , Bennani I , de Muralt B , Titelion V , Picaud C . Is continuous transcutaneous monitoring of PCO2 (TcPCO2) over 8 h reliable in adults? Respir Med 95: 331‐335, 2001.
 180. Javaheri S , Colangelo G , Corser B , Zahedpour MR . Familial respiratory chemosensitivity does not predict hypercapnia of patients with sleep apnea‐hypopnea syndrome. Am Rev Respir Dis 145: 837‐840, 1992.
 181. Jeppesen J , Green A , Steffensen BF , Rahbek J . The Duchenne muscular dystrophy population in Denmark, 1977‐2001: Prevalence, incidence and survival in relation to the introduction of ventilator use. Neuromuscul Disord 13: 804‐812, 2003.
 182. Johnson MW , Remmers JE . Accessory muscle activity during sleep in chronic obstructive pulmonary disease. J Appl Physiol 57: 1011‐1017, 1984.
 183. Jokic R , Zintel T , Sridhar G , Gallagher CG , Fitzpatrick MF . Ventilatory responses to hypercapnia and hypoxia in relatives of patients with the obesity hypoventilation syndrome. Thorax 55: 940‐945, 2000.
 184. Jolley CJ , Luo YM , Steier J , Reilly C , Seymour J , Lunt A , Ward K , Rafferty GF , Polkey MI , Moxham J . Neural respiratory drive in healthy subjects and in COPD. Eur Respir J 33: 289‐297, 2009.
 185. Jonville S , Delpech N , Denjean A . Contribution of respiratory acidosis to diaphragmatic fatigue at exercise. Eur Respir J 19: 1079‐1086, 2002.
 186. Juan G , Calverley P , Talamo C , Schnader J , Roussos C . Effect of carbon dioxide on diaphragmatic function in human beings. N Engl J Med 310: 874‐879, 1984.
 187. Just N , Bautin N , Danel‐Brunaud V , Debroucker V , Matran R , Perez T . The Borg dyspnoea score: A relevant clinical marker of inspiratory muscle weakness in amyotrophic lateral sclerosis. Eur Respir J 35: 353‐360, 2010.
 188. Kafer ER . Respiratory function in paralytic scoliosis. Am Rev Respir Dis 110: 450‐457, 1974.
 189. Kalra SP . Central leptin insufficiency syndrome: An interactive etiology for obesity, metabolic and neural diseases and for designing new therapeutic interventions. Peptides 29: 127‐138, 2008.
 190. Kang SW , Bach JR . Maximum insufflation capacity. Chest 118: 61‐65, 2000.
 191. Katayama M , Naritomi H , Nishio H , Watanabe T , Teramoto S , Kanda F , Hazama A . Long‐term stabilization of respiratory conditions in patients with spinal muscular atrophy type 2 by continuous positive airway pressure: A report of two cases. Kobe J Med Sci 57: E98‐105, 2011.
 192. Kaw R , Hernandez AV , Walker E , Aboussouan L , Mokhlesi B . Determinants of hypercapnia in obese patients with obstructive sleep apnea: A systematic review and metaanalysis of cohort studies. Chest 136: 787‐796, 2009.
 193. Kawata N , Tatsumi K , Terada J , Tada Y , Tanabe N , Takiguchi Y , Kuriyama T . Daytime Hypercapnia in Obstructive Sleep Apnea Syndrome. Chest 132: 1832‐1838, 2007.
 194. Kay A , Trinder J , Kim Y . Progressive changes in airway resistance during sleep. J Appl Physiol 81: 282‐292, 1996.
 195. Kessler R , Chaouat A , Schinkewitch P , Faller M , Casel S , Krieger J , Weitzenblum E . The obesity‐hypoventilation syndrome revisited: A prospective study of 34 consecutive cases. Chest 120: 369‐376, 2001.
 196. Kesten S , Garfinkel SK , Wright T , Rebuck AS . Impaired exercise capacity in adults with moderate scoliosis. Chest 99: 663‐666, 1991.
 197. Khan Y , Heckmatt J . Obstructive apnoeas in Duchenne muscular dystrophy. Thorax 49: 157‐161, 1994.
 198. Khirani S , Louis B , Leroux K , Delord V , Fauroux B , Lofaso F . Harms of unintentional leaks during volume targeted pressure support ventilation. Respir Med 107: 1021‐1029, 2013.
 199. Kimura K , Tachibana N , Kimura J , Shibasaki H . Sleep‐disordered breathing at an early stage of amyotrophic lateral sclerosis. J Neuro Sci 164: 37‐43, 1999.
 200. Kirk VG , Flemons WW , Adams C , Rimmer KP , Montgomery MD . Sleep‐disordered breathing in Duchenne Muscular dystrophy: A preliminary study of the role of portable monitoring. Pediatr Pulmonol 29: 135‐140, 2000.
 201. Kiyan E , Okumus G , Cuhadaroglu C , Deymeer F . Sleep apnea in adult myotonic dystrophy patients who have no excessive daytime sleepiness. Sleep Breath 14: 19‐24, 2009.
 202. Koenig SM . Pulmonary complications of obesity. Am J Med Sci 321: 249‐279, 2001.
 203. Kolodziej MA , Jensen L , Rowe B , Sin D . Systematic review of noninvasive positive pressure ventilation in severe stable COPD. Eur Respir J 30: 293‐306, 2007.
 204. Koo KW , Sax DS , Snider GL . Arterial blood gases and pH during sleep in chronic obstructive pulmonary disease. Am J Med 58: 663‐670, 1975.
 205. Koumbourlis AC . Scoliosis and the respiratory system. Paediatr Respir Rev 7: 152‐160, 2006.
 206. Kress JP , Pohlman AS , Alverdy J , Hall JB . The impact of morbid obesity on oxygen cost of breathing (VO(2RESP)) at rest. Am J Respir Crit Care Med 160: 883‐886, 1999.
 207. Kushida C , Littner M , Morgenthaler TI , Alessi C , Bailey D , Coleman JJ , Friedman L , Hirshkowitz M , Kapen S , Kramer M , Lee‐Chiong T , Loube D , Owens J , Pancer JP , Wise M . Practice parameters for the indications for polysomnography and related procedures: An update for 2005. Sleep 28: 499‐521, 2005.
 208. Labanowski M , Schmidt‐Nowara W , Guilleminault C . Sleep and neuromuscular disease: Frequency of sleep‐disordered breathing in a neuromuscular disease clinic population. Neurology 47: 1173‐1180, 1996.
 209. Ladosky W , Botelho MAM , Albuquerque JP . Chest mechanics in morbidly obese non‐hypoventilated patients. Respir Med 95: 281‐286, 2001.
 210. Lakshminarayan S , Sahn SA , Weil JV . Effect of aminophylline on ventilatory responses in normal man. Am Rev Respir Dis 117: 33‐38, 1978.
 211. Lanini B , Misuri G , Gigliotti F , Iandelli I , Pizzi A , Romagnoli I , Scano G . Perception of dyspnea in patients with neuromuscular disease. Chest 120: 402‐408, 2001.
 212. Laroche CM , Carroll N , Moxham J , Green M . Clinical significance of severe isolated diaphragm weakness. Am Rev Respir Dis 138: 862‐866, 1988.
 213. Laub M , Midgren B . The effects of nocturnal home mechanical ventilation on daytime blood gas disturbances. Clin Physiol Funct Imaging 26: 79‐82, 2006.
 214. Lazarus R , Sparrow D , Weiss ST . Effects of obesity and fat distribution on ventilatory function: The normative aging study. Chest 111: 891‐898, 1997.
 215. Lechtzin N , Shade D , Clawson L , Wiener CM . Supramaximal inflation improves lung compliance in subjects with amyotrophic lateral sclerosis. Chest 129: 1322‐1329, 2006.
 216. Lechtzin N , Wiener CM , Shade DM , Clawson L , Diette GB . Spirometry in the supine position improves the detection of diaphragmatic weakness in patients with amyotrophic lateral sclerosis. Chest 121: 436‐442, 2002.
 217. Lee MY , Lin CC , Shen SY , Chiu CH , Liaw SF . Work of breathing in eucapnic and hypercapnic sleep apnea syndrome. Respiration 77: 146‐153, 2009.
 218. Lee S‐D , Nakano H , Farkas GA . Adenosinergic modulation of ventilation in obese zucker rats. Obes Res 13: 545‐555, 2005.
 219. Lee SK , Kim DH , Choi WA , Won YH , Kim SM , Kang SW . The significance of transcutaneous continuous overnight CO2 monitoring in determining initial mechanical ventilator application for patients with neuromuscular disease. Ann Rehabil Med 36: 126‐132, 2012.
 220. Leger P , Bedicam JM , Cornette A , Reybet‐Degat O , Langevin B , Polu JM , Jeannin L , Robert D . Nasal intermittent positive pressure ventilation. long‐term follow‐up in patients with severe chronic respiratory insufficiency. Chest 105: 100‐105, 1994.
 221. Lewis CA , Eaton TE , Fergusson W , Whyte KF , Garrett JE , Kolbe J . Home overnight pulse oximetry in patients with COPD: More than one recording may be needed. Chest 123: 1127‐1133, 2003.
 222. Lewis CA , Ferguson W , Eaton T , Zeng I , Kolbe J . Isolated nocturnal desaturation in COPD: Prevalence and impact on quality of life and sleep. Thorax 64: 133‐138, 2009.
 223. Lewis MI , LoRusso TJ , Fournier M . Anabolic influences of insulin‐like growth factor I and/or growth hormone on the diaphragm of young rats. J Appl Physiol 82: 1972‐1978, 1997.
 224. Lin CC . Effect of nasal CPAP on ventilatory drive in normocapnic and hypercapnic patients with obstructive sleep apnoea syndrome. Eur Respir J 7: 2005‐2010, 1994.
 225. Lin CC . Comparison between nocturnal nasal positive pressure ventilation combined with oxygen therapy and oxygen monotherapy in patients with severe COPD. Am J Respir Crit Care Med 154: 353‐358, 1996.
 226. Lin CC , Wu KM , Chou CS , Liaw SF . Oral airway resistance during wakefulness in eucapnic and hypercapnic sleep apnea syndrome. Respir Physiol Neurobiol 139: 215‐224, 2004.
 227. Lindgren AC , Hellstrom LG , Ritzen EM , Milerad J . Growth hormone treatment increases CO(2) response, ventilation and central inspiratory drive in children with Prader‐Willi syndrome. Eur J Pediatr 158: 936‐940, 1999.
 228. Lisboa C , Moreno R , Fava M , Ferretti R , Cruz E . Inspiratory muscle function in patients with severe kyphoscoliosis. Am Rev Respir Dis 132: 48‐52, 1985.
 229. Little SA , Elkholy MM , Chalmers GW , Farouk A , Patel KR , Thomson NC . Predictors of nocturnal oxygen desaturation in patients with COPD. Respir Med 93: 202‐207, 1999.
 230. Liu C , Cao Y , Malhotra A , Ling L . Sleep fragmentation attenuates the hypercapnic (but not hypoxic) ventilatory responses via adenosine A1 receptors in awake rats. Respir Physiol Neurobiol 175: 29‐36, 2011.
 231. Lloyd‐Owen SJ , Donaldson GC , Ambrosino N , Escarabill J , Farre R , Fauroux B , Robert D , Schoenhofer B , Simonds AK , Wedzicha JA . Patterns of home mechanical ventilation use in Europe: Results from the Eurovent survey. Eur Respir J 25: 1025‐1031, 2005.
 232. Lo Coco D , Marchese S , Corrao S , Cettina Pesco M , La Bella V , Piccoli F , Lo Coco A . Development of chronic hypoventilation in amyotrophic lateral sclerosis patients. Respir Med 100: 1028‐1036, 2006.
 233. Lofaso F , Fauroux B , Orlikowski D , Prigent H . Daytime predictors of sleep‐disordered breathing in neuromuscular patients to better schedule polysomnography. Eur Respir J 37: 231‐232, 2010.
 234. Lofaso F , Quera‐Salva MA . Polysomnography for the management of progressive neuromuscular disorders. Eur Respir J 19: 989‐990, 2002.
 235. Lopata M , Onal E . Mass loading, sleep apnea, and the pathogenesis of obesity hypoventilation. Am Rev Respir Dis 126: 640‐645, 1982.
 236. Lopes JM , Tabachnik E , Muller NL , Levison H , Bryan AC . Total airway resistance and respiratory muscle activity during sleep. J Appl Physiol 54: 773‐777, 1983.
 237. Louis B , Leroux K , Isabey D , Fauroux B , Lofaso F . Effect of manufacturer‐inserted mask leaks on ventilator performance. Eur Respir J 35: 627‐636, 2010.
 238. Lujan M , Sogo A , Monso E . Home mechanical ventilation monitoring software: Measure more or measure better? Arch Bronconeumol 48: 170‐178, 2012.
 239. Lukacsovits J , Carlucci A , Hill N , Ceriana P , Pisani L , Schreiber A , Pierucci P , Losonczy G , Nava S . Physiological changes during low‐ and high‐intensity noninvasive ventilation. Eur Respir J 39: 869‐875, 2012.
 240. Lukacsovits J , Nava S . Inspiratory pressure during noninvasive ventilation in stable COPD: Help the lungs, but do not forget the heart. Eur Respir J 41: 765‐766, 2013.
 241. Lyall RA , Donaldson N , Polkey MI , Leigh PN , Moxham J . Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis. Brain 124: 2000‐2013, 2001.
 242. Macavei VM , Spurling KJ , Loft J , Makker HK . Diagnostic predictors of obesity‐hypoventilation syndrome in patients suspected of having sleep disordered breathing. J Clin Sleep Med 9: 879‐884, 2013.
 243. Makinodan K , Yoshikawa M , Fukuoka A , Tamaki S , Koyama N , Yamauchi M , Tomoda K , Hamada K , Kimura H . Effect of serum leptin levels on hypercapnic ventilatory response in obstructive sleep apnea. Respiration 75: 257‐264, 2008.
 244. Maniscalco M , Zedda A , Faraone S , Carratu P , Sofia M . Evaluation of a transcutaneous carbon dioxide monitor in severe obesity. Intensive Care Med 34: 1340‐1344, 2008.
 245. Marin JM , Soriano JB , Carrizo SJ , Boldova A , Celli BR . Outcomes in patients with chronic obstructive pulmonary disease and obstructive sleep apnea. The Overlap Syndrome. Am J Respir Crit Care Med 182: 325‐331, 2010.
 246. Marshall NS , Yee BJ , Desai AV , Buchanan PR , Wong KK , Crompton R , Melehan KL , Zack N , Rao SG , Gendreau RM , Kranzler J , Grunstein RR . Two randomized placebo‐controlled trials to evaluate the efficacy and tolerability of mirtazapine for the treatment of obstructive sleep apnea. Sleep 31: 824‐831, 2008.
 247. Martí S , Pallero M , Ferrer J , Ríos J , Rodríguez E , Morell F , Muñoz X . Predictors of mortality in chest wall disease treated with noninvasive home mechanical ventilation. Respir Med 104: 1843‐1849, 2010.
 248. Masa JF , Celli BR , Riesco JA , Sanchez de Cos J , Disdier C , Sojo A . Noninvasive positive pressure ventilation and not oxygen may prevent overt ventilatory failure in patients with chest wall diseases. Chest 112: 207‐213, 1997.
 249. Matecki S , Topin N , Hayot M , Rivier F , Echenne B , Prefaut C , Ramonatxo M . A standardized method for the evaluation of respiratory muscle endurance in patients with Duchenne muscular dystrophy. Neuromusc Dis 11: 171‐177, 2001.
 250. McCool FD , Tzelepis GE , Leith DE , Hoppin FG, Jr . Oxygen cost of breathing during fatiguing inspiratory resistive loads. J Appl Physiol 66: 2045‐2055, 1989.
 251. McEvoy RD , Pierce RJ , Hillman D , Esterman A , Ellis EE , Catcheside PG , O'Donoghue FJ , Barnes DJ , Grunstein RR . Nocturnal non‐invasive nasal ventilation in stable hypercapnic COPD: A randomised controlled trial. Thorax 64: 561‐566, 2009.
 252. McKeon JL , Murree‐Allen K , Saunders NA . Prediction of oxygenation during sleep in patients with chronic obstructive lung disease. Thorax 43: 312‐317, 1988.
 253. McKim DA , Griller N , LeBlanc C , Woolnough A , King J . Twenty‐four hour noninvasive ventilation in Duchenne muscular dystrophy: A safe alternative to tracheostomy. Can Respir J 20: e 5‐e 9, 2013.
 254. McNicholas WT . Impact of sleep in respiratory failure. Eur Respir J 10: 920‐933, 1997.
 255. Meecham Jones DJ , Paul EA , Bell JH , Wedzicha JA . Ambulatory oxygen therapy in stable kyphoscoliosis. Eur Respir J 8: 819‐823, 1995.
 256. Meecham Jones DJ , Paul EA , Jones PW , Wedzicha JA . Nasal pressure support ventilation plus oxygen compared with oxygen therapy alone in hypercapnic COPD. Am J Respir Crit Care Med 152: 538‐544, 1995.
 257. Meecham Jones DJ , Wedzicha JA . Comparison of pressure and volume preset nasal ventilator systems in stable chronic respiratory failure. Eur Respir J 6: 1060‐1064, 1993.
 258. Mellies U , Dohna‐Schwake C , Stehling F , Voit T . Sleep disordered breathing in spinal muscular atrophy. Neuromusc Dis 14: 797‐803, 2004.
 259. Mellies U , Ragette R , Dohna Schwake C , Boehm H , Voit T , Teschler H . Long‐term noninvasive ventilation in children and adolescents with neuromuscular disorders. Eur Respir J 22: 631‐636, 2003.
 260. Mermigkis C , Kopanakis A , Foldvary‐Schaefer N , Golish J , Polychronopoulos V , Schiza S , Amfilochiou A , Siafakas N , Bouros D . Health‐related quality of life in patients with obstructive sleep apnoea and chronic obstructive pulmonary disease (overlap syndrome). Intern J Clin Pract 61: 207‐211, 2007.
 261. Meyrignac C , Poirier J , Degos JD . Amyotrophic lateral sclerosis presenting with respiratory insufficiency as the primary complaint. Clinicopathological study of a case. Eur Neurol 24: 115‐120, 1985.
 262. Midgren B . Oxygen desaturation during sleep as a function of the underlying respiratory disease. Am Rev Respir Dis 141: 43‐46, 1990.
 263. Milross MA , Piper AJ , Norman M , Becker HF , Willson GN , Grunstein RR , Sullivan CE , Bye PT . Low‐flow oxygen and bilevel ventilatory support: Effects on ventilation during sleep in cystic fibrosis. Am J Respir Crit Care Med 163: 129‐134, 2001.
 264. Misuri G , Lanini B , Gigliotti F , Iandelli I , Pizzi A , Bertolini MG , Scano G . Mechanism of CO(2) retention in patients with neuromuscular disease. Chest 117: 447‐453, 2000.
 265. Mokhlesi B , Kryger MH , Grunstein RR . Assessment and management of patients with Obesity Hypoventilation Syndrome. Proc Am Thorac Soc 5: 218‐225, 2008.
 266. Mokhlesi B , Tulaimat A , Evans AT , Wang Y , Itani AA , Hassaballa HA , Herdegen JJ , Stepanski EJ . Impact of adherence with positive airway pressure therapy on hypercapnia in obstructive sleep apnea. J Clin Sleep Med 2: 57‐62, 2006.
 267. Mokhlesi B , Tulaimat A , Faibussowitsch I , Wang Y , Evans A . Obesity hypoventilation syndrome: Prevalence and predictors in patients with obstructive sleep apnea. Sleep Breath 11: 117‐124, 2007.
 268. Monneret D , Borel JC , Pepin JL , Tamisier R , Arnol N , Levy P , Faure P . Pleiotropic role of IGF‐I in obesity hypoventilation syndrome. Growth Horm IGF Res 20: 127‐133, 2010.
 269. Montes de Oca M , Celli B . Mouth occlusion pressure, CO2 response and hypercapnia in severe chronic obstructive pulmonary disease. Eur Respir J 12: 666‐671, 1998.
 270. Morgan RK , McNally S , Alexander M , Conroy R , Hardiman O , Costello RW . Use of sniff nasal‐inspiratory force to predict survival in Amyotrophic Lateral Sclerosis. Am J Respir Crit Care Med 171: 269‐274, 2005.
 271. Mulloy E , McNicholas WT . Ventilation and gas exchange during sleep and exercise in severe COPD. Chest 109: 387‐394, 1996.
 272. Munoz X , Crespo A , Marti S , Torres F , Ferrer J , Morell F . Comparative study of two different modes of noninvasive home mechanical ventilation in chronic respiratory failure. Respir Med 100: 673‐681, 2006.
 273. Murphy PB , Brignall K , Moxham J , Polkey MI , Davidson AC , Hart N . High pressure versus high intensity noninvasive ventilation in stable hypercapnic chronic obstructive pulmonary disease: A randomized crossover trial. Int J Chron Obstruct Pulmon Dis 7: 811‐818, 2012.
 274. Murphy PB , Davidson C , Hind MD , Simonds A , Williams AJ , Hopkinson NS , Moxham J , Polkey M , Hart N . Volume targeted versus pressure support non‐invasive ventilation in patients with super obesity and chronic respiratory failure: A randomised controlled trial. Thorax 67: 727‐734, 2012.
 275. Naimark A , Cherniack RM . Compliance of the respiratory system and its components in health and obesity. J Appl Physiol 15: 377‐382, 1960.
 276. Nardi J , Prigent H , Adala A , Bohic M , Lebargy F , Quera‐Salva M‐A , Orlikowski D , Lofaso F . Nocturnal oximetry and transcutaneous carbon dioxide in home‐ventilated neuromuscular patients. Respiratory Care 57: 1425‐1430, 2012.
 277. Nardi J , Prigent Hln , Garnier B , Lebargy Fo , Quera‐Salva M‐A , Orlikowski D , Lofaso Fdr . Efficiency of invasive mechanical ventilation during sleep in Duchenne muscular dystrophy. Sleep Med 13: 1056‐1065, 2012.
 278. Nava S , Fanfulla F , Frigerio P , Navalesi P . Physiologic evaluation of 4 weeks of nocturnal nasal positive pressure ventilation in stable hypercapnic patients with chronic obstructive pulmonary disease. Respiration 68: 573‐583, 2001.
 279. Nava S , Rubini F , Zanotti E , Caldiroli D . The tension‐time index of the diaphragm revisited in quadriplegic patients with diaphragm pacing. Am J Respir Critl Care Med 153: 1322‐1327, 1996.
 280. Nickol A , Hart N , Hopkinson N , Hamnegård C‐H , Moxham J , Simonds A , Polkey M . Mechanisms of improvement of respiratory failure in patients with COPD treated with NIV. Int J Chron Obstruct Pulmon Dis 3: 453‐462, 2008.
 281. Nickol AH , Hart N , Hopkinson NS , Moxham J , Simonds A , Polkey MI . Mechanisms of improvement of respiratory failure in patients with restrictive thoracic disease treated with non‐invasive ventilation. Thorax 60: 754‐760, 2005.
 282. Norman RG , Goldring RM , Clain JM , Oppenheimer BW , Charney AN , Rapoport DM , Berger KI . Transition from acute to chronic hypercapnia in patients with periodic breathing: Predictions from a computer model. J Appl Physiol 100: 1733‐1741, 2006.
 283. Nowbar S , Burkart KM , Gonzales R , Fedorowicz A , Gozansky WS , Gaudio JC , Taylor MRG , Zwillich CW . Obesity‐associated hypoventilation in hospitalized patients: Prevalence, effects, and outcome. Am J Med 116: 1‐7, 2004.
 284. O'Connor TM , O'Riordan DM , Stack M , Bredin CP . Airways obstruction in survivors of thoracoplasty: Reversibility is greater in non‐smokers. Respirology 9: 130‐133, 2004.
 285. O'Donnell CP , Schaub CD , Haines AS , Berkowitz DE , Tankersley CG , Schwartz AR , Smith PL . Leptin prevents respiratory depression in obesity. Am J Respir Crit Care Med 159: 1477‐1484, 1999.
 286. O'Donnell CP , Tankersley CG , Polotsky VP , Schwartz AR , Smith PL . Leptin, obesity, and respiratory function. Respir Physiol 119: 163‐170, 2000.
 287. O'Donoghue FJ , Catcheside PG , Ellis EE , Grunstein RR , Pierce RJ , Rowland LS , Collins ER , Rochford SE , McEvoy RD . Sleep hypoventilation in hypercapnic chronic obstructive pulmonary disease: Prevalence and associated factors. Eur Respir J 21: 977‐984, 2003.
 288. Olson A , Zwillich C . The obesity hypoventilation syndrome. Am J Med 118: 948‐956, 2005.
 289. Ono S , Kurisaki H , Sakuma A , Nagao K . Myotonic dystrophy with alveolar hypoventilation and hypersomnia: A clinicopathological study. J Neurol Sci 128: 225‐231, 1995.
 290. Ono S , Takahashi K , Jinnai K , Kanda F , Fukuoka Y , Kurisaki H , Mitake S , Inagaki T , Yamano T , Shimizu N , Nagao K . Loss of catecholaminergic neurons in the medullary reticular formation in myotonic dystrophy. Neurology 51: 1121‐1124, 1998.
 291. Oscroft NS , Ali M , Gulati A , Davies MG , Quinnell TG , Shneerson JM , Smith IE . A randomised crossover trial comparing volume assured and pressure preset noninvasive ventilation in stable hypercapnic COPD. COPD 7: 398‐403, 2010.
 292. Owange‐Iraka JW , Harrison A , Warner JO . Lung function in congenital and idiopathic scoliosis. Eur J Pediatr 142: 198‐200, 1984.
 293. Ozturk L , Unal M , Tamer L , Celikoglu F . The association of the severity of obstructive sleep apnea with plasma leptin levels. Arch Otolaryng Head Neck Surg 129: 538‐540, 2003.
 294. Paiva R , Krivec U , Aubertin G , Cohen E , Clement A , Fauroux B . Carbon dioxide monitoring during long‐term noninvasive respiratory support in children. Intensive Care Med 35: 1068‐1074, 2009.
 295. Panitch HB . Diurnal hypercapnia in patients with neuromuscular disease. Paediatr Respir Rev 11: 3‐8, 2010.
 296. Pankow W , Hijjeh N , Schuttler F , Penzel T , Becker H , Peter J , von Wichert P . Influence of noninvasive positive pressure ventilation on inspiratory muscle activity in obese subjects. Eur Respir J 10: 2847‐2852, 1997.
 297. Pankow W , Podszus T , Gutheil T , Penzel T , Peter J , Von Wichert P . Expiratory flow limitation and intrinsic positive end‐expiratory pressure in obesity. J Appl Physiol 85: 1236‐1243, 1998.
 298. Parreira VF , Delguste P , Jounieaux V , Aubert G , Dury M , Rodenstein DO . Effectiveness of controlled and spontaneous modes in nasal two‐level positive pressure ventilation in awake and asleep normal subjects. Chest 112: 1267‐1277, 1997.
 299. Parreira VF , Delguste P , Jounieaux V , Aubert G , Dury M , Rodenstein DO . Glottic aperture and effective minute ventilation during nasal two‐level positive pressure ventilation in spontaneous mode. Am J Respir Crit Care Med 154: 1857‐1863, 1996.
 300. Pasquina P , Adler D , Farr P , Bourqui P , Bridevaux PO , Janssens JP . What does built‐in software of home ventilators tell us? An observational study of 150 patients on home ventilation. Respiration 83: 293‐299, 2012.
 301. Passamano L , Taglia A , Palladino A , Viggiano E , D'Ambrosio P , Scutifero M , Rosaria Cecio M , Torre V , DE Luca F , Picillo E , Paciello O , Piluso G , Nigro G , Politano L . Improvement of survival in Duchenne Muscular Dystrophy: Retrospective analysis of 835 patients. Acta Myol 31: 121‐125, 2012.
 302. Pelosi P , Croci M , Ravagnan I , Vicardi P , Gattinoni L . Total respiratory system, lung, and chest wall mechanics in sedated‐paralyzed postoperative morbidly obese patients. Chest 109: 144‐151, 1996.
 303. Perez de Llano LA , Golpe R , Ortiz Piquer M , Veres Racamonde A , Vazquez Caruncho M , Caballero Muinelos O , Alvarez Carro C . Short‐term and long‐term effects of nasal intermittent positive pressure ventilation in patients with obesity‐hypoventilation syndrome. Chest 128: 587‐594, 2005.
 304. Phillips MF , Smith PEM , Carroll N , Edwards RHT , Calverley PMA . Nocturnal oxygenation and prognosis in Duchenne muscular dystrophy. Am J Respir Crit Care Med 160: 198‐202, 1999.
 305. Phillips MS , Miller MR , Kinnear WJ , Gough SE , Shneerson JM . Importance of airflow obstruction after thoracoplasty. Thorax 42: 348‐352, 1987.
 306. Phipps PR , Starritt E , Caterson I , Grunstein RR . Association of serum leptin with hypoventilation in human obesity. Thorax 57: 75‐76, 2002.
 307. Pinto A , Almeida JP , Pinto S , Pereira J , Oliveira AG , de Carvalho M . Home telemonitoring of non‐invasive ventilation decreases healthcare utilisation in a prospective controlled trial of patients with amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 81: 1238‐1242, 2010.
 308. Pinto A , de Carvalho M , Evangelista T , Lopes A , Sales‐Luis L . Nocturnal pulse oximetry: A new approach to establish the appropriate time for non‐invasive ventilation in ALS patients. Amyotroph Lateral Scler 4: 31‐35, 2003.
 309. Piper A . Obesity hypoventilation syndrome: Therapeutic implications for treatment. Expert Rev Resp Med 4: 57‐70, 2010.
 310. Piper A . Sleep abnormalities associated with neuromuscular disease: Pathophysiology and evaluation. Semin Respir Crit Care Med 23: 211‐219, 2002.
 311. Piper AJ , Grunstein RR . Big breathing: The complex interaction of obesity, hypoventilation, weight loss, and respiratory function. J Appl Physiol 108: 199‐205, 2010.
 312. Piper AJ , Moran FM . Non‐invasive ventilation and the physiotherapist: Current state and future trends. Phys Ther Rev 11: 37‐43, 2006.
 313. Piper AJ , Parker S , Torzillo PJ , Sullivan CE , Bye PT . Nocturnal nasal IPPV stabilizes patients with cystic fibrosis and hypercapnic respiratory failure. Chest 102: 846‐850, 1992.
 314. Piper AJ , Sullivan CE . Effects of long‐term nocturnal nasal ventilation on spontaneous breathing during sleep in neuromuscular and chest wall disorders. Eur Respir J 9: 1515‐1522, 1996.
 315. Piper AJ , Sullivan CE . Effects of short‐term NIPPV in the treatment of patients with severe obstructive sleep apnea and hypercapnia. Chest 105: 434‐440, 1994.
 316. Piper AJ , Wang D , Yee BJ , Barnes DJ , Grunstein RR . Randomised trial of CPAP vs bilevel support in the treatment of obesity hypoventilation syndrome without severe nocturnal desaturation. Thorax 63: 395‐401, 2008.
 317. Polkey MI , Lyall RA , Green M , Nigel Leigh P , Moxham J . Expiratory muscle function in amyotrophic lateral sclerosis. Am J Respir Crit Care Med 158: 734‐741, 1998.
 318. Polotsky M , Elsayed‐Ahmed AS , Pichard L , Harris CC , Smith PL , Schneider H , Kirkness JP , Polotsky V , Schwartz AR . Effects of leptin and obesity on the upper airway function. J Appl Physiol 112: 1637‐1643, 2012.
 319. Polotsky VY , Wilson JA , Smaldone MC , Haines AS , Hurn PD , Tankersley CG , Smith PL , Schwartz AR , O'Donnell CP . Female gender exacerbates respiratory depression in leptin‐deficient obesity. Am J Respir Crit Care Med 164: 1470‐1475, 2001.
 320. Poulter NR , Chang CL , Farley TM , Meirik O . Risk of cardiovascular diseases associated with oral progestagen preparations with therapeutic indications. Lancet 354: 1610, 1999.
 321. Powers SK , Farkas GA , Demirel H , Coombes J , Fletcher L , Hughes MG , Hodge K , Dodd SL , Schlenker EH . Effects of aging and obesity on respiratory muscle phenotype in Zucker rats. J Appl Physiol 81: 1347‐1354, 1996.
 322. Prinianakis G , Delmastro M , Carlucci A , Ceriana P , Nava S . Effect of varying the pressurisation rate during noninvasive pressure support ventilation. Eur Respir J 23: 314‐320, 2004.
 323. Rabec C , Georges M , Kabeya NK , Baudouin N , Massin F , Reybet‐Degat O , Camus P . Evaluating noninvasive ventilation using a monitoring system coupled to a ventilator: A bench‐to‐bedside study. Eur Respir J 34: 902‐913, 2009.
 324. Rabec C , Rodenstein D , Leger P , Rouault S , Perrin C , Gonzalez‐Bermejo J . Ventilator modes and settings during non‐invasive ventilation: Effects on respiratory events and implications for their identification. Thorax 66: 170‐178, 2011.
 325. Rabkin J , Ogino M , Goetz R , McElhiney M , Marziliano A , Imai T , Atsuta N , Morita M , Tateishi T , Matsumura T , Mitsumoto H . Tracheostomy with invasive ventilation for ALS patients: Neurologists' roles in the US and Japan. Amyotroph Later Scler Frontotemporal Degener 14: 116‐123, 2013.
 326. Radwan L , Maszczyk Z , Koziorowski A , Koziej M , Cieslicki J , Sliwinski P , Zielinski J . Control of breathing in obstructive sleep apnoea and in patients with the overlap syndrome. Eur Respir J 8: 542‐545, 1995.
 327. Ragette R , Mellies U , Schwake C , Voit T , Teschler H . Patterns and predictors of sleep disordered breathing in primary myopathies. Thorax 57: 724‐728, 2002.
 328. Rapoport DM , Garay SM , Epstein H , Goldring RM . Hypercapnia in the obstructive sleep apnea syndrome. A reevaluation of the “Pickwickian syndrome”. Chest 89: 627‐635, 1986.
 329. Raurich JM , Rialp G , Ibanez J , Llompart‐Pou JA , Ayestaran I . Hypercapnic respiratory failure in obesity‐hypoventilation syndrome: CO2 response and acetazolamide treatment effects. Respir Care 55: 1442‐1448, 2010.
 330. Redolfi S , Corda L , La Piana G , Spandrio S , Prometti P , Tantucci C . Long‐term non‐invasive ventilation increases chemosensitivity and leptin in obesity‐hypoventilation syndrome. Respir Med 101: 1191‐1195, 2007.
 331. Resta O , Foschino‐Barbaro MP , Bonfitto P , Talamo S , Legari G , De Pergola G , Minenna A , Giorgino R . Prevalence and mechanisms of diurnal hypercapnia in a sample of morbidly obese subjects with obstructive sleep apnea. Respir Med 94: 240‐246, 2000.
 332. Restrick LJ , Fox NC , Braid G , Ward EM , Paul EA , Wedzicha JA . Comparison of nasal pressure support ventilation with nasal intermittent positive pressure ventilation in patients with nocturnal hypoventilation. Eur Respir J 6: 364‐370, 1993.
 333. Robert D , Argaud L . Non‐invasive positive ventilation in the treatment of sleep‐related breathing disorders. Sleep Med 8: 441‐452, 2007.
 334. Robinson T , Freiberg D , Regnis J , Young IH . The role of hypoventilation and ventilation‐perfusion redistribution in oxygen‐induced hypercapnia during acute exacerbations of chronic obstructive pulmonary disease. Am J Respir Crit Care Med 161: 1524‐1529, 2000.
 335. Rodriguez‐Roisin R , Drakulovic M , Rodriguez DA , Roca J , Barbera JA , Wagner PD . Ventilation‐perfusion imbalance and chronic obstructive pulmonary disease staging severity. J Appl Physiol 106: 1902‐1908, 2009.
 336. Rosenow EC , Engel AG . Acid maltase deficiency in adults presenting as respiratory failure. Am J Med 64: 485‐491, 1978.
 337. Saatci E , Miller DM , Stell IM , Lee KC , Moxham J . Dynamic dead space in face masks used with noninvasive ventilators: A lung model study. Eur Respir J 23: 129‐135, 2004.
 338. Sahebjami H , Gartside PS . Pulmonary function in obese subjects with a normal FEV1/FVC ratio. Chest 110: 1425‐1429, 1996.
 339. Salord N , Mayos M , Miralda RM , Farré A , Carreras M , Sust R , Masuet‐Aumatell C , Rodríguez J , Pérez A . Continuous positive airway pressure in clinically stable patients with mild‐to‐moderate obesity hypoventilation syndrome and obstructive sleep apnoea. Respirology 18: 1135‐1142, 2013.
 340. Samolski D , Tarrega J , Anton A , Mayos M , Marti S , Farrerp E , Guell R . Sleep hypoventilation due to increased nocturnal oxygen flow in hypercapnic COPD patients. Respirology 15: 283‐288, 2010.
 341. Sampson MG , Grassino AE . Load compensation in obese patients during quiet tidal breathing. J Appl Physiol 55: 1269‐1276, 1983.
 342. Sampson MG , Grassino K . Neuromechanical properties in obese patients during carbon dioxide rebreathing. Am J Med 75: 81‐90, 1983.
 343. Sancho J , Servera E , Diaz J , Marin J . Predictors of ineffective cough during a chest infection in patients with stable Amyotrophic Lateral Sclerosis. Am J Respir Crit Care Med 175: 1266‐1271, 2007.
 344. Sancho J , Servera E , Morelot‐Panzini C , Salachas Fo , Similowski T , Gonzalez‐Bermejo J . Non‐invasive ventilation effectiveness and the effect of ventilatory mode on survival in ALS patients. Amyotroph Lateral Scler Frontotemporal Degener 15: 55‐61, 2014.
 345. Sanders MH , Kern NB , Costantino JP , Stiller RA , Strollo PJ, Jr , Studnicki KA , Coates JA , Richards TJ . Accuracy of end‐tidal and transcutaneous PCO2 monitoring during sleep. Chest 106: 472‐483, 1994.
 346. Santos C , Braghiroli A , Mazzini L , Pratesi R , Oliveira LV , Mora G . Sleep‐related breathing disorders in amyotrophic lateral sclerosis. Monaldi Arch Chest Dis 59: 160‐165, 2003.
 347. Sasaki K , Inoue S , Yoshida A , Hayashi F , Masuda Y , Honda Y . Ventilatory response and drive due to carbon dioxide stimulation in idiopathic scoliosis. Tohoku J Experiment Med 137: 145‐151, 1982.
 348. Sawicka EH , Branthwaite MA . Respiration during sleep in kyphoscoliosis. Thorax 42: 801‐808, 1987.
 349. Schonhofer B . Non‐invasive positive pressure ventilation in patients with stable hypercapnic COPD: Light at the end of the tunnel? Thorax 65: 765‐767, 2010.
 350. Schonhofer B , Barchfeld T , Wenzel M , Kohler D . Long term effects of non‐invasive mechanical ventilation on pulmonary haemodynamics in patients with chronic respiratory failure. Thorax 56: 524‐528, 2001.
 351. Schonhofer B , Dellweg D , Suchi S , Kohler D . Exercise endurance before and after long‐term noninvasive ventilation in patients with chronic respiratory failure. Respiration 75: 296‐303, 2008.
 352. Schonhofer B , Geibel M , Sonneborn M , Haidl P , Kohler D . Daytime mechanical ventilation in chronic respiratory insufficiency. Eur Respir J 10: 2840‐2846, 1997.
 353. Schonhofer B , Kohler D . Effect of non‐invasive mechanical ventilation on sleep and nocturnal ventilation in patients with chronic respiratory failure. Thorax 55: 308‐313, 2000.
 354. Schonhofer B , Sonneborn M , Haidl P , Bohrer H , Kohler D . Comparison of two different modes for noninvasive mechanical ventilation in chronic respiratory failure: Volume versus pressure controlled device. Eur Respir J 10: 184‐191, 1997.
 355. Schonhofer B , Sortor‐Leger S . Equipment needs for noninvasive mechanical ventilation. Eur Respir J 20: 1029‐1036, 2002.
 356. Schonhofer B , Wallstein S , Wiese C , Kohler D . Noninvasive mechanical ventilation improves endurance performance in patients with chronic respiratory failure due to thoracic restriction. Chest 119: 1371‐1378, 2001.
 357. Secker‐Walker RH , Ho JE , Gill IS . Observations on regional ventilation and perfusion in kyphoscoliosis. Respiration 38: 194‐203, 1979.
 358. Sharp JT , Druz WS , Kondragunta VR . Diaphragmatic responses to body position changes in obese patients with obstructive sleep apnea. Am Rev Respir Dis 133: 32‐37, 1986.
 359. Shimura R , Tatsumi K , Nakamura A , Kasahara Y , Tanabe N , Takiguchi Y , Kuriyama T . Fat accumulation, leptin, and hypercapnia in obstructive sleep apnea‐hypopnea syndrome. Chest 127: 543‐549, 2005.
 360. Shiraishi K , Sasaki H , Yaekashiwa M , Motomiya M , Nukiwa T . Total respiratory system compliance after thoracoplasty. Respir Med 92: 810‐814, 1998.
 361. Shneerson JM , Simonds AK . Noninvasive ventilation for chest wall and neuromuscular disorders. Eur Respir J 20: 480‐487, 2002.
 362. Similowski T , Attali V , Bensimon G , Salachas F , Mehiri S , Arnulf I , Lacomblez L , Zelter M , Meininger V , Derenne JP . Diaphragmatic dysfunction and dyspnoea in amyotrophic lateral sclerosis. Eur Respir J 15: 332‐337, 2000.
 363. Simon PM , Dempsey JA , Landry DM , Skatrud JB . Effect of sleep on respiratory muscle activity during mechanical ventilation. Am Rev Respir Dis 147: 32‐37, 1993.
 364. Simonds AK , Elliott MW . Outcome of domiciliary nasal intermittent positive pressure ventilation in restrictive and obstructive disorders. Thorax 50: 604‐609, 1995.
 365. Skatrud JB , Dempsey JA , Bhansali P , Irvin C . Determinants of chronic carbon dioxide retention and its correction in humans. J Clin Invest 65: 813‐821, 1980.
 366. Smith IE , Shneerson JM . Secondary failure of nasal intermittent positive pressure ventilation using the Monnal D: Effects of changing ventilator. Thorax 52: 89‐91, 1997.
 367. Smith PE , Edwards RH , Calverley PM . Ventilation and breathing pattern during sleep in Duchenne muscular dystrophy. Chest 96: 1346‐1351, 1989.
 368. Sogo A , Montanya J , Monso E , Blanch L , Pomares X , Lujan M . Effect of dynamic random leaks on the monitoring accuracy of home mechanical ventilators: A bench study. BMC Pulmon Med 13: 75, 2013.
 369. Soudon P , Steens M , Toussaint M . A comparison of invasive versus noninvasive full‐time mechanical ventilation in Duchenne muscular dystrophy. Chron Respir Dis 5: 87‐93, 2008.
 370. Sriram KB , Thornton A , Antic R . Spontaneous mode non‐invasive ventilation fails to treat respiratory failure in a patient with Multi‐mincore disease: A case report. Cases J 1: 93, 2008.
 371. Stefanutti D , Benoist MR , Scheinmann P , Chaussain M , Fitting JW . Usefulness of sniff nasal pressure in patients with neuromuscular or skeletal disorders. Am J Respir Crit Care Med 162: 1507‐1511, 2000.
 372. Steier J , Jolley CJ , Seymour J , Kaul S , Luo YM , Rafferty GF , Hart N , Polkey MI , Moxham J . Sleep‐disordered breathing in unilateral diaphragm paralysis or severe weakness. Eur Respir J 32: 1479‐1487, 2008.
 373. Steier J , Jolley CJ , Seymour J , Roughton M , Polkey MI , Moxham J . Neural respiratory drive in obesity. Thorax 64: 719‐725, 2009.
 374. Steier J , Jolley CJ , Seymour J , Teschler H , Luo YM , Polkey MI , Moxham J . Screening for sleep‐disordered breathing in neuromuscular disease using a questionnaire for symptoms associated with diaphragm paralysis. Eur Respir J 37: 400‐405, 2011.
 375. Storre JH , Bohm P , Dreher M , Windisch W . Clinical impact of leak compensation during non‐invasive ventilation. Respir Med 103: 1477‐1483, 2009.
 376. Storre JH , Magnet FS , Dreher M , Windisch W . Transcutaneous monitoring as a replacement for arterial PCO(2) monitoring during nocturnal non‐invasive ventilation. Respir Med 105: 143‐150, 2011.
 377. Storre JH , Seuthe B , Fiechter R , Milioglou S , Dreher M , Sorichter S , Windisch W . Average volume‐assured pressure support in obesity hypoventilation: A randomized crossover trial. Chest 130: 815‐821, 2006.
 378. Storre JH , Steurer B , Kabitz HJ , Dreher M , Windisch W . Transcutaneous PCO2 monitoring during initiation of noninvasive ventilation. Chest 132: 1810‐1816, 2007.
 379. Strom K , Pehrsson K , Boe J , Nachemson A . Survival of patients with severe thoracic spine deformities receiving domiciliary oxygen therapy. Chest 102: 164‐168, 1992.
 380. Struik FM , Lacasse Y , Goldstein R , Kerstjens HM , Wijkstra PJ . Nocturnal non‐invasive positive pressure ventilation for stable chronic obstructive pulmonary disease. Cochrane Database Syst Rev 6: CD002878, 2013.
 381. Suresh S , Wales P , Dakin C , Harris MA , Cooper DG. Sleep‐related breathing disorder in Duchenne muscular dystrophy: Disease spectrum in the paediatric population. J Paediatr Child Health 41: 500‐503, 2005.
 382. Sutton FD , Zwillich CW , Creagh CE , Pierson DJ , Weil JV . Progesterone for outpatient treatment of Pickwickian syndrome. Ann Intern Med 83: 476‐479, 1975.
 383. Tabachnik E , Muller NL , Bryan AC , Levison H . Changes in ventilation and chest wall mechanics during sleep in normal adolescents. J Appl Physiol 51: 557‐564, 1981.
 384. Tankersley CG , O'Donnell C , Daood MJ , Watchko JF , Mitzner W , Schwartz A , Smith P . Leptin attenuates respiratory complications associated with the obese phenotype. J Appl Physiol 85: 2261‐2269, 1998.
 385. Tarrega J , Anton A , Guell R , Mayos M , Samolski D , Marti S , Farrero E , Prats E , Sanchis J . Predicting nocturnal hypoventilation in hypercapnic chronic obstructive pulmonary disease patients undergoing long‐term oxygen therapy. Respiration 82: 4‐9, 2011.
 386. Tatsumi K , Kasahara Y , Kurosu K , Tanabe N , Takiguchi Y , Kuriyama T . Sleep oxygen desaturation and circulating leptin in obstructive sleep apnea‐hypopnea syndrome. Chest 127: 716‐721, 2005.
 387. Tatsumi K , Kimura H , Kunitomo F , Kuriyama T , Watanabe S , Honda Y . Sleep arterial oxygen desaturation and chemical control of breathing during wakefulness in COPD. Chest 90: 68‐73, 1986.
 388. Teppema LJ , Dahan A . Acetazolamide and breathing. Does a clinical dose alter peripheral and central CO2 sensitivity? Am J Respir Crit Care Med 160: 1592‐1597, 1999.
 389. Teschler H , Stampa J , Ragette R , Konietzko N , Berthon‐Jones M . Effect of mouth leak on effectiveness of nasal bilevel ventilatory assistance and sleep architecture. Eur Respir J 14: 1251‐1257, 1999.
 390. Tojima H , Kunitomo F , Kimura H , Tatsumi K , Kuriyama T , Honda Y . Effects of acetazolamide in patients with the sleep apnoea syndrome. Thorax 43: 113‐119, 1988.
 391. Toussaint M , Boitano LJ , Gathot V , Steens M , Soudon P . Limits of effective cough‐augmentation techniques in patients with neuromuscular disease. Respir Care 54: 359‐366, 2009.
 392. Toussaint M , Chatwin M , Soudon P . Review Article: Mechanical ventilation in Duchenne patients with chronic respiratory insufficiency: Clinical implications of 20 years published experience. Chron Respir Dis 4: 167‐177, 2007.
 393. Toussaint M , Steens M , Soudon P . Lung function accurately predicts hypercapnia in patients with Duchenne muscular dystrophy. Chest 131: 368‐375, 2007.
 394. Toussaint M , Steens M , Wasteels G , Soudon P . Diurnal ventilation via mouthpiece: Survival in end‐stage Duchenne patients. Eur Respir J 28: 549‐555, 2006.
 395. Trask CH , Cree EM . Oximeter studies on patients with chronic obstructive emphysema, awake and during sleep. N Engl J Med 266: 639‐642, 1962.
 396. Tsolaki V , Pastaka C , Kostikas K , Karetsi E , Dimoulis A , Zikiri A , Koutsokera A , Gourgoulianis KI . Noninvasive ventilation in chronic respiratory failure: Effects on quality of life. Respiration 81: 402‐410, 2011.
 397. Tsuboi T , Oga T , Machida K , Chihara Y , Matsumoto H , Niimi A , Sumi K , Ohi M , Mishima M , Chin K . Importance of ventilator mode in long‐term noninvasive positive pressure ventilation. Respir Med 103: 1854‐1861, 2009.
 398. Tuggey J , Elliott M . Randomised crossover study of pressure and volume non‐invasive ventilation in chest wall deformity. Thorax 60: 859‐864, 2005.
 399. Tuggey JM , Elliott MW . Titration of non‐invasive positive pressure ventilation in chronic respiratory failure. Respir Med 100: 1262‐1269, 2006.
 400. Ueno Y , Nakanishi N , Oto J , Imanaka H , Nishimura M . A bench study of the effects of leak on ventilator performance during noninvasive ventilation. Respir Care 56: 1758‐1764, 2011.
 401. van der Meche FG , Bogaard JM , van der Sluys JC , Schimsheimer RJ , Ververs CC , Busch HF . Daytime sleep in myotonic dystrophy is not caused by sleep apnoea. J Neurol Neurosurg Psychiatry 57: 626‐628, 1994.
 402. van Noord JA , Cauberghs M , Van de Woestijne KP , Demedts M . Total respiratory resistance and reactance in ankylosing spondylitis and kyphoscoliosis. Eur Respir J 4: 945‐951, 1991.
 403. Veale D , Cooper BG , Gilmartin JJ , Walls TJ , Griffith CJ , Gibson GJ . Breathing pattern awake and asleep in patients with myotonic dystrophy. Eur Respir J 8: 815‐818, 1995.
 404. Ververs CC , Van der Meche FG , Verbraak AF , van der Sluys HC , Bogaard JM . Breathing pattern awake and asleep in myotonic dystrophy. Respiration 63: 1‐7, 1996.
 405. Vianello A , Bevilacqua M , Salvador V , Cardaioli C , Vincenti E . Long‐term nasal intermittent positive pressure ventilation in advanced Duchenne's muscular dystrophy. Chest 105: 445‐448, 1994.
 406. Vignaux L , Vargas F , Roeseler J , Tassaux D , Thille AW , Kossowsky M , Brochard L , Jolliet P . Patient‐ventilator asynchrony during non‐invasive ventilation for acute respiratory failure: A multicenter study. Intensive Care Med 35: 840‐846, 2009.
 407. Vila B , Servera E , Marin J , Diaz J , Gimenez M , Komaroff E , Bach J . Noninvasive ventilatory assistance during exercise for patients with kyphoscoliosis: A pilot study. Am J Phys Med Rehabil 86: 672‐677, 2007.
 408. Vitacca M , Bianchi L , Zanotti E , Vianello A , Barbano L , Porta R , Clini E . Assessment of physiologic variables and subjective comfort under different levels of pressure support ventilation. Chest 126: 851‐859, 2004.
 409. Vitacca M , Nava S , Confalonieri M , Bianchi L , Porta R , Clini E , Ambrosino N . The appropriate setting of noninvasive pressure support ventilation in stable COPD patients. Chest 118: 1286‐1293, 2000.
 410. Vos PJ , Folgering HT , van Herwaarden CL . Predictors for nocturnal hypoxaemia (mean SaO2 < 90%) in normoxic and mildly hypoxic patients with COPD. Eur Respir J 8: 74‐77, 1995.
 411. Wang D , Grunstein R , Teichtahl H . Association between ventilatory response to hypercapnia and obstructive sleep apnea–hypopnea index in asymptomatic subjects. Sleep and Breathing 11: 103‐108, 2007.
 412. Ward S , Chatwin M , Heather S , Simonds AK . Randomised controlled trial of non‐invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia. Thorax 60: 1019‐1024, 2005.
 413. Weinberg J , Klefbeck B , Borg J , Svanborg E . Polysomnography in chronic neuromuscular disease. Respiration 70: 349‐354, 2003.
 414. Weiner P , Waizman J , Weiner M , Rabner M , Magadle R , Zamir D . Influence of excessive weight loss after gastroplasty for morbid obesity on respiratory muscle performance. Thorax 53: 39‐42, 1998.
 415. Weitzenblum E , Chaouat A . Sleep and chronic obstructive pulmonary disease. Sleep Med Rev 8: 281‐294, 2004.
 416. White DP , Douglas NJ , Pickett CK , Zwillich CW , Weil JV . Sleep deprivation and the control of ventilation. Am Rev Respir Dis 128: 984‐986, 1983.
 417. White JE , Drinnan MJ , Smithson AJ , Griffiths CJ , Gibson GJ . Respiratory muscle activity and oxygenation during sleep in patients with muscle weakness. Eur Respir J 8: 807‐814, 1995.
 418. White JE , Drinnan MJ , Smithson AJ , Griffiths CJ , Gibson GJ . Respiratory muscle activity during rapid eye movement (REM) sleep in patients with chronic obstructive pulmonary disease. Thorax 50: 376‐382, 1995.
 419. Whitelaw WA , Derenne JP . Airway occlusion pressure. J Appl Physiol 74: 1475‐1483, 1993.
 420. Whyte KF , Gould GA , Airlie MA , Shapiro CM , Douglas NJ . Role of protriptyline and acetazolamide in the sleep apnea/hypopnea syndrome. Sleep 11: 463‐472, 1988.
 421. Wijesinghe M , Williams M , Perrin K , Weatherall M , Beasley R . The effect of supplemental oxygen on hypercapnia in subjects with obesity‐associated hypoventilation: A randomized, crossover, clinical study. Chest 139: 1018‐1024, 2011.
 422. Windisch W , Freidel K , Schucher B , Baumann H , Wiebel M , Matthys H , Petermann F . Evaluation of health‐related quality of life using the MOS 36‐Item Short‐Form Health Status Survey in patients receiving noninvasive positive pressure ventilation. Intensive Care Med 29: 615‐621, 2003.
 423. Windisch W , Haenel M , Storre JH , Dreher M . High‐intensity non‐invasive positive pressure ventilation for stable hypercapnic COPD. Int J Med Sci 6: 72‐76, 2009.
 424. Windisch W , Kostic S , Dreher M , Virchow JC, Jr , Sorichter S . Outcome of patients with stable COPD receiving controlled noninvasive positive pressure ventilation aimed at a maximal reduction of PaCO2 . Chest 128: 657‐662, 2005.
 425. Windisch W , Storre JH , Sorichter S , Virchow JC, Jr . Comparison of volume‐ and pressure‐limited NPPV at night: A prospective randomized cross‐over trial. Respir Med 99: 52‐59, 2005.
 426. Windisch W , Vogel M, Sorichter S , Hennings E , Bremer H , Hamm H , Matthys H , Virchow JC, Jr . Normocapnia during nIPPV in chronic hypercapnic COPD reduces subsequent spontaneous PaCO2. Respir Med 96: 572‐579, 2002.
 427. Yee BJ , Cheung J , Phipps P , Banerjee D , Piper AJ , Grunstein RR . Treatment of obesity hypoventilation syndrome and serum leptin. Respiration 73: 209‐212, 2006.
 428. Zavorsky GS , Cao J , Mayo NE , Gabbay R , Murias JM . Arterial versus capillary blood gases: A meta‐analysis. Respir Physiol Neurobiol 155: 268‐279, 2007.
 429. Zavorsky GS , Hoffman SL . Pulmonary gas exchange in the morbidly obese. Obes Rev 9: 326‐339, 2008.
 430. Zerah F , Harf A , Perlemuter L , Lorino H , Lorino AM , Atlan G . Effects of obesity on respiratory resistance. Chest 103: 1470‐1476, 1993.
 431. Zwillich CW , Sutton FD , Pierson DJ , Greagh EM , Weil JV . Decreased hypoxic ventilatory drive in the obesity‐hypoventilation syndrome. Am J Med 59: 343‐348, 1975.

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Article Title: Hypoventilation Syndromes
 

How to Cite

Amanda J. Piper, Brendon J. Yee. Hypoventilation Syndromes. Compr Physiol 2014, 4: 1639-1676. doi: 10.1002/cphy.c140008