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Wiener klinische Wochenschrift

, Volume 128, Supplement 1, pp 1–36 | Cite as

Nichtinvasive und invasive außerklinische Beatmung beim chronisch respiratorischen Versagen

Konsensus-Report des Arbeitskreises für Beatmung & Intensivmedizin der Österreichischen Gesellschaft für Pneumologie
  • Peter Schenk
  • Ernst Eber
  • Georg-Christian Funk
  • Wilfried Fritz
  • Sylvia Hartl
  • Peter Heininger
  • Eveline Kink
  • Gernot Kühteubl
  • Beatrice Oberwaldner
  • Ulrike Pachernigg
  • Andreas Pfleger
  • Petra Schandl
  • Ingrid Schmidt
  • Markus Stein
ÖGP-Konsensus

Zusammenfassung

Der vorliegende Konsensusreport, der unter der Patronanz der Österreichischen Gesellschaft für Pneumologie (ÖGP) erstellt wurde, soll einen praxisnahen Leitfaden für die außerklinische Beatmung darstellen mit Bedachtnahme auf die spezifisch österreichischen Rahmenbedingungen und gesetzlichen Grundlagen. Der Leitfaden orientiert sich nach einer Konsensusempfehlung der ÖGP zur Ausstattung deslangzeitbeatmeten Patienten aus dem Jahr 2004 und der S2-Leitlinie der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin über die nichtinvasive und invasive Beatmung bei der chronischen respiratorischen Insuffizienz aus 2010, angepasst an nationale Erfahrungen und aktualisiert durch rezente Literatur.

In 11 Kapiteln wird einerseits die Einleitung, Umstellung und Kontrolle der außerklinische Beatmung, die technische Ausstattung sowie der Beatmungszugang, andererseits die verschiedenen Indikationen wie chronisch obstruktive Atemwegserkrankungen, thorakal-restriktive und neuromuskuläre Erkrankungen, das Adipositas-Hypoventilationssyndrom sowie pädiatrische Erkrankungen beschrieben. Weiters wird ausführlich auf die Atemphysiotherapie bei Erwachsenen und Kindern unter invasiver und nichtinvasiver Langzeitbeatmung eingegangen.

Schlüsselwörter

Langzeitbeatmung außerklinisch Konsensusempfehlung Respiratorabhängigkeit Atemphysiotherapie 

Abkürzungsverzeichnis

ASVG

Allgemeines Sozialversicherungsgesetz

APT

Atemphysiotherapie

CO2

Kohlendioxid

COPD

chronic obstructive pulmonary disease (chronisch obstruktive Lungenerkrankung)

EMG

Elektromyografie

FiO2

Fraction of inspired oxygen

FVC

forcierte Vitalkapazität

GOLD

Global Initiative for Chronic Obstruvtive Lung Disease

GuKG

Gesundheits- und Krankenpflegegesetz

HME

heat and moisture exchanger (Wärme- und Feuchtigkeitsaustauscher)

I:E

Verhältnis von Inspirations- zu Expirationsdauer

mbar

millibar

MI:E

mechanical in-exsufflator

NIV

noninvasive ventilation (nichtinvasive Beatmung)

NME

neuromuskuläre Erkrankung

ÖGP

Österreichische Gesellschaft für Pneumologie

OSAS

obstruktives Schlafapnoesyndrom

PaCO2

arterieller Kohlendioxidpartialdruck

PaO2

arterieller Sauerstoffpartialdruck

PCF

peak cough flow (Hustenspitzenfluss)

PEEP

positive endexpiratory pressure (positiver endexspiratorischer Druck)

Pe max

maximaler expiratorischer Druck

Pi max

maximaler inspiratorischer Druck

PSV

pressure support ventilation

PTcCO2

transkutan gemessener, zur Temperatur korrigierter, Kohlendioxidpartialdruck

PVC

Polyvinyl-Chlorid

QoL

quality of life

REM

rapid eye movement (schnelle Augenbewegungen)

Tbc

Tuberkulose

VC

vital capacity (Vitalkapazität)

Non-invasive and invasive out of hospital ventilation in chronic respiratory failure

Consensus report of the working group on ventilation and intensive care medicine of the Austrian Society of Pneumology

Abstract

The current consensus report was compiled under the patronage of the Austrian Society of Pneumology (Österreichischen Gesellschaft für Pneumologie, ÖGP) with the intention of providing practical guidelines for out-of-hospital ventilation that are in accordance with specific Austrian framework parameters and legal foundations. The guidelines are oriented toward a 2004 consensus ÖGP recommendation concerning the setup of long-term ventilated patients and the 2010 German Respiratory Society S2 guidelines on noninvasive and invasive ventilation of chronic respiratory insufficiency, adapted to national experiences and updated according to recent literature. In 11 chapters, the initiation, adjustment, and monitoring of out-of-hospital ventilation is described, as is the technical equipment and airway access. Additionally, the different indications—such as chronic obstructive pulmonary diseases, thoracic restrictive and neuromuscular diseases, obesity hypoventilation syndrome, and pediatric diseases—are discussed. Furthermore, the respiratory physiotherapy of adults and children on invasive and noninvasive long-term ventilation is addressed in detail.

Keywords

Long-term ventilation Out-of-hospital Consensus recommendation Respirator dependence Respiratory physiotherapy 

Notes

Interessenkonflikt

P. Schenk – Referentenhonorar: Boehringer Ingelheim, AstraZeneca, Menarini, InterMune, Takeda, Actelion, Torrex-Chiesi, Linde, Maquet, Novartis; Roche; Advisory Board: Boehringer Ingelheim, Torrex-Chiesi, Actelion, Novartis; Research grants: INOTherapeutics, Lilly, AstraZeneca, Roche; Konsulenten-Tätigkeit: Angelini. Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: keine.

E. Eber – Referentenhonorar: Honorare von Abbott, AOP Orphan Pharmaceuticals, AstraZeneca, Chiesi, MSD, Novartis, Nycomed; Advisory Board: Mitwirkung in Advisory Boards von AOP Orphan Pharmaceuticals, GSK; Research grants: keine; Konsulenten-Tätigkeit: AOP Orphan Pharmaceuticals; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: keine.

G.C. Funk – Referentenhonorar: Orion Pharma, Actelion, B. Braun, Pfizer, Boehringer Ingelheim, Nycomed, Roche Diagnostics, Dräger, Fresenius; Advisory Board: Takeda Pharma; Research grants: keine; Konsulenten-Tätigkeit: keine; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: keine.

W. Fritz – Referentenhonorar: Astra Zeneca, Bayer, Boehringer Ingelheim, Pfizer, Novartis; Advisory Board: nein; Research grants: nein; Konsulenten-Tätigkeit: nein; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: nein.

S. Hartl – Referentenhonorar: Takeda, Boehringer, GSK; Advisory Board: Böhringer, Takeda, Chiesi, Mundipharma, GSK, Teva, Novartis, Almirall; Research grants: Boehringer, GSK, Novartis, MSD, Astra, als Ludwig Boltzmann Institut; Konsulenten-Tätigkeit: keine; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: keine.

P. Heininger – Referentenhonorar: Fa.: Actelion, GSK, AOP Orphan, Pfizer; Advisory Board: nein; Research grants: nein; Konsulenten-Tätigkeit: nein; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: nein.

E. Kink – Referentenhonorar: Dräger, Habel, Vivisol, Cardinal Health, Menarini; Advisory Board: nein; Research grants: nein; Konsulenten-Tätigkeit: nein; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: nein.

G. Kühteubl – Referentenhonorar: Fa.Habel; Advisory Board: nein; Research grants: nein; Konsulenten-Tätigkeit: nein; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: keine.

B. Oberwaldner – Referentenhonorar: nein; Advisory Board: nein; Research grants: nein; Konsulenten-Tätigkeit: nein; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: nein.

U. Pachernigg – Referentenhonorar: nein; Advisory Board: nein; Research grants: nein; Konsulenten-Tätigkeit: nein; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: nein.

A. Pfleger – Referentenhonorar: keine von Firmen; Advisory Board: nein; Research grants: nein; Konsulenten-Tätigkeit: nein; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: keine.

P. Schandl – Referentenhonorar: Smith Medical Care, Heinen und Löwenstein; Advisory Board: nein; Research grants: nein; Konsulenten-Tätigkeit: nein; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: nein.

I. Schmidt – Referentenhonorar: Air Liquide, Astra Zeneca, Boehringer Ingelheim, Chiesi, Dräger, Forest, Heinen & Löwenstein, Linde, Menarini, Novartis, AOP, Vivisol; Advisory Board: Chiesi; Research grants: nein; Konsulenten-Tätigkeit: nein; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: nein.

M. Stein – Referentenhonorar: Boehringer Ingelheim, Bristol-Myers Squibb, GSK, Habel; Advisory Board: nein; Research grants: nein; Konsulenten-Tätigkeit: nein; Aktien- oder sonstiger Besitz an pharmazeutischen bzw. medizintechnischen Unternehmen, Patentrechte etc.: keine.

Ethische Standards

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

Literatur

  1. 1.
    Schönhofer B. Nichtinvasive Beatmung zur Therapie der akuten respiratorischen und chronischen ventilatorischen Insuffizienz. Dtsch Med Wochenschr. 2009;134:535–40.Google Scholar
  2. 2.
    Kabitz HJ, Walterspacher S, Walker D, Windisch W. Inspiratory muscle strength in chronic obstructive pulmonary disease depending on disease severity. Clin Sci (Lond). 2007b;113(5):243–9.CrossRefGoogle Scholar
  3. 3.
    Criée CP, Laier-Groeneveld G. Die Atempumpe: Atemmuskulatur und intermittierende Selbstbeatmung. 1. Aufl. New York: Thieme; 1995.Google Scholar
  4. 4.
    Windisch W, Brambring J, Budweiser S, et al. S2-Leitlinie: Nichtinvasive und invasive Beatmung als Therapie der chronischen respiratorischen Insuffizienz. Pneumologie. 2010;64(4):207–40.PubMedCrossRefGoogle Scholar
  5. 5.
    Kabitz H, Windisch W. Respiratory muscle testing: state of the art. Pneumologie. 2007;61(9):582–7.PubMedCrossRefGoogle Scholar
  6. 6.
    Mehta S, Hill NS. Noninvasive ventilation. Am J Respir Crit Care Med. 2001;163(2):540–77.PubMedCrossRefGoogle Scholar
  7. 7.
    Hartl S, Heindl W, Lahrmann H, et al. Provisions for long-term at home ventilated patient: consensus recommendations of the Austrian society for lung diseases and tuberculosis. Wien Klin Wochenschr. 2004;116(Suppl 3):1–20.PubMedGoogle Scholar
  8. 8.
    Bundesgesetz über Gesundheits- und Krankenpflegeberufe (Gesundheits- und Krankenpflegegesetz – GuKG)StF. BGBl. I Nr. 108/1997 (NR: GP XX RV 709 AB 777.S. 82. BR: 5494 AB 5515 S. 629.) (CELEX-Nr.: 377L0452, 377L0453, 389L0048392L0051) http://www.ris.bka.gv.at.
  9. 9.
    Goldberg AI. Noninvasive mechanical ventilation at home: building upon the tradition. Chest. 2002;121:321–4.PubMedCrossRefGoogle Scholar
  10. 10.
    Leger P. Organization of long term mechanical ventilation in Europe. In: Simonds AK, Herausgeber. Non-invasive respiratory support. A practical handbook.Google Scholar
  11. 11.
    Lloyd-Owen SJ, Donaldson GC, Ambrosino N, et al. Patterns of home mechanical use in Europe: results from the Eurovent survey. Eur Respir J. 2005;25:1025–31.PubMedCrossRefGoogle Scholar
  12. 12.
    Simonds AK. Risk management of the home ventilator dependent patient. Thorax. 2006;61(5):369–71.PubMedPubMedCentralCrossRefGoogle Scholar
  13. 13.
    Branthwaite MA. Ethical and medico legal aspects of assisted ventilation. In: Simonds AK, Herausgeber. Non‐invasive respiratory support: a practical handbook. London: Arnold; S. 282–91.Google Scholar
  14. 14.
    Durchführungsempfehlungen zur invasiven außerklinischen Beatmung. Gemeinsame Empfehlung der Deutschen Gesellschaft für Pneumologie, der Deutschen Interdisziplinären Gesellschaft für Außerklinische Beatmung, des medizinischen Dienstes des Spitzenverbandes Bund der Krankenkassen e. V. und des AOK-Bundesverbandes. Pneumologie. 2011;65:72–88.CrossRefGoogle Scholar
  15. 15.
    Hartl S, et al. Long-term invasive mechanical ventilation in the home-2007 revision & update. Respir Care. 2007;52(1):1056–62.Google Scholar
  16. 16.
    Gonzalez-Bermejo J, Laplanche V, Husseini FE, et al. Evaluation of the user-friendliness of 11 home mechanical ventilators. Eur Respir J. 2006;27:1236–43.PubMedCrossRefGoogle Scholar
  17. 17.
    Chatwin M, Heather S, Hanak A, et al. Analysis of home support and ventilator malfunction in 1211 ventilator-dependent patients. Eur Respir J. 2010;35:310–16.PubMedCrossRefGoogle Scholar
  18. 18.
    Lofaso F, Fodil R, Lorino H, et al. Inaccuracy of tidal volume delivered by home mechanical ventilators. Eur Respir J. 2000;15:338–41.PubMedCrossRefGoogle Scholar
  19. 19.
    Luján M, Sogo A, Pomares X, Monsó E, Sales B, Blanch L. Effect of leak and breathing pattern on the accuracy of tidal volume estimation by commercial home ventilators: a bench study. Respir Care. 2013;58(5):770–7.PubMedCrossRefGoogle Scholar
  20. 20.
    Contal O, Vignaux L, Combescure C, et al. Monitoring of noninvasive ventilation by built-in software of home bilevel ventilators: a bench study. Chest. 2012;141(2):469–76.PubMedCrossRefGoogle Scholar
  21. 21.
    Highcock MP, Shneerson JM, Smith IE. Functional differences in bi-level pressure preset ventilators. Eur Respir J. 2001;17:268–73.PubMedCrossRefGoogle Scholar
  22. 22.
    Blakeman TC, Rodriquez D, Hanseman D, et al. Bench evaluation of 7 home-care ventilators. Respir Care. 2011;56(11):1791–98.PubMedCrossRefGoogle Scholar
  23. 23.
    Hess DR. Patient-ventilator interactions during noninvasive ventilation. Respir Care. 2011;56(2):153–65.PubMedCrossRefGoogle Scholar
  24. 24.
    Han J, Liu J. Effect of circuit changes on ventilator-associated pneumonia: a systematic review and meta-analysis. Respir Care. 2010;55(4):467–74.PubMedGoogle Scholar
  25. 25.
    Schönhofer B, Sortor-Leger S. Equipment needs for noninvasive mechanical ventilation. Eur Respir J. 2002;20:1029–36.PubMedCrossRefGoogle Scholar
  26. 26.
    Navalesi P, Fanfulla F, Frigerio P, Gregoretti C, Nava S. Physiologic evaluation of noninvasive mechanical ventilation delivered with three types of masks in patients with chronic hypercapnic respiratory failure. Crit Care Med. 2000;28(6):1785–90.PubMedCrossRefGoogle Scholar
  27. 27.
    Fraticelli AT, Lellouche F, L’her E, Taillé S, Mancebo J, Brochard L. Physiological effects of different interfaces during noninvasive ventilation for acute respiratory failure. Crit Care Med. 2009;37(3):939–45.PubMedCrossRefGoogle Scholar
  28. 28.
    Nava S, Navalesi P, Gregoretti C. Interfaces and humidification for noninvasive mechanical ventilation. Respir Care. 2009;54(1):71–84.PubMedGoogle Scholar
  29. 29.
    Ambrosino N, Goldstein R, Herausgeber. Ventilatory support for chronic respiratory failure. USA: Informa Healthcare; 2008.Google Scholar
  30. 30.
    Esquinas AM, Herausgeber. Noninvasive mechanical ventilation. Theory, equipment, and clinical applications. Berlin: Springer; 2010.Google Scholar
  31. 31.
    Björling G. Long-term tracheotomy care: How to do it; Breathe. 2009 March;5(3).Google Scholar
  32. 32.
    Schönhofer B, Kuhlen R, Neumann P, et al. S3-Leitlinie NIV bei akuter respiratorischer Insuffizienz. Pneumologie. 2008;62(5):449–79.PubMedCrossRefGoogle Scholar
  33. 33.
    AARC Clinical Practice Guideline. Long-Term Invasive Mechanical Ventilation in the Home – 2007 Revision & Update. Respir Care. 2007;52(8):1056–62.Google Scholar
  34. 34.
    Hess DR. Tracheostomy tubes and related appliances. Respir Care. 2005;50(4):497–510.PubMedGoogle Scholar
  35. 35.
    Morán I, Cabello B, Manero E, Mancebo J. Comparison of the effects of two humidifier systems on endotracheal tube resistance. Intensive Care Med. 2011;37:1773–9.PubMedCrossRefGoogle Scholar
  36. 36.
    Hess DR. Facilitating speech in the patient with tracheostomy. Respir Care. 2005;50(4):519–25.PubMedGoogle Scholar
  37. 37.
    Lotti GA, et al. Unfavorable effects of heat and moisture exchangers in ventilated patients. Intensive Care Med. 1997;23:399–405.CrossRefGoogle Scholar
  38. 38.
    Jaber S, et al. Comparison of the effects of heat and moisture exchangers and heated humidifiers on ventilation and gas exchange during non-invasive ventilation. Intensive Care Med. 2002;28:1590–4.PubMedCrossRefGoogle Scholar
  39. 39.
    Kelly M, et al. Heated humidification versus heat and moisture exchangers for ventilated adults and children. Cochrane Database Syst Rev. 2010;(4). doi: 10.1002/14651858.CD004711.
  40. 40.
    Jaber S. Long-term effects of different humidification systems on endotracheal tube patency. Anesthesiology. 2004;100(4):782–8.PubMedCrossRefGoogle Scholar
  41. 41.
    AARC Evidence-Based Clinical Practice Guidelines. Care of the ventilator circuit and its relation to ventilator-associated pneumonia. Respir Care. 2003;48(9):869–79. (Hess DR et al)Google Scholar
  42. 42.
    AARC Clinical Practice Guideline. Selection of device, administration of bronchodilator, and evaluation of response to therapy in mechanically ventilated patients. Respir Care. 1999;44(1):105–13.Google Scholar
  43. 43.
    Medizinische Absauggeräte – Teil 1. Elektrisch betriebene Absauggeräte Sicherheitsanforderungen (ISO 10079-1: 1999); Deutsche Fassung: EN ISO 10079-1: 2009.Google Scholar
  44. 44.
    AARC Clinical Practice Guidelines. Endotracheal suctioning of mechanically ventilated patients with artificial airways. Respir Care. 2010;55(6):758–64.Google Scholar
  45. 45.
    Ram FSF, Picot J, Lightowler J, Wedzicha JA. Non-invasive positive pressure ventilation for treatment of respiratory failure due to exacerbations of chronic obstructive pulmonary disease. Cochrane Database Syst Rev 2004;(3):CD004104.Google Scholar
  46. 46.
    Köhnlein T, Windisch W, Köhler D, et al. Non-invasive positive pressure ventilation for the treatment of severe stable chronic obstructive pulmonary disease: a prospective, multicentre, randomised, controlled clinical trial. Lancet Respir Med. 2014;2(9):698–705.PubMedCrossRefGoogle Scholar
  47. 47.
    Windisch W, Haenel M, Storre JH, Dreher M. High-intensity non-invasive positive pressure ventilation for stable hypercapnic COPD. Int J Med Sci. 2009;6(2):72–6.PubMedPubMedCentralCrossRefGoogle Scholar
  48. 48.
    Dreher M, Storre J, Schmoor C, Windisch W. High-intensity versus low-intensity non-invasive ventilation in stable hypercapnic COPD patients: a randomized cross-over trial. Thorax. 2010;65(4):303–8.PubMedCrossRefGoogle Scholar
  49. 49.
    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. 1995;152(2):538–44.PubMedCrossRefGoogle Scholar
  50. 50.
    Clini E, Sturani C, Rossi A, Viaggi S, et al. The Italian multicentre study on noninvasive ventilation in chronic obstructive pulmonary disease patients. Eur Respir J. 2002;20(3):529–38.PubMedCrossRefGoogle Scholar
  51. 51.
    Budweiser S, Jorres RA, Riedl T, et al. Predictors of survival in COPD patients with chronic hypercapnic respiratory failure receiving noninvasive home ventilation. Chest. 2007;131(6):1650–8.PubMedCrossRefGoogle Scholar
  52. 52.
    Windisch W, Criee CP. Lebensqualität bei Patienten mit häuslicher Beatmung. Pneumologie. 2006;60(9):539–46.PubMedCrossRefGoogle Scholar
  53. 53.
    Tsolaki V, Pastaka C, Karetsi E, et al. One-year non-invasive ventilation in chronic hypercapnic COPD: effect on quality of life. Respir Med. 2008;102(6):904–11.PubMedCrossRefGoogle Scholar
  54. 54.
    Tsolaki V, Pastaka C, Kostikas K, et al. Noninvasive ventilation in chronic respiratory failure: effects on quality of life. Respiration. 2011;81(5):402–10.PubMedCrossRefGoogle Scholar
  55. 55.
    Windisch W, Dreher M, Storre JH, Sorichter S. Nocturnal non-invasive positive pressure ventilation: physiological effects on spontaneous breathing. Respir Physiol Neurobiol. 2006;150(2–3):251–60.PubMedCrossRefGoogle Scholar
  56. 56.
    Oscroft NS, Quinnell TG, Shneerson JM, Smith IE. Long-term non-invasive ventilation to manage persistent ventilatory failure after COPD exacerbation. Respirology. 2010;15(5):818–22.PubMedCrossRefGoogle Scholar
  57. 57.
    Heindl W. Die nichtinvasive Beatmung. Wien Klin Wochenschr. 1999;111(19):784–801.PubMedGoogle Scholar
  58. 58.
    Budweiser S, Heinemann F, Meyer K, Wild PJ, Pfeifer M. Weight gain in cachectic COPD patients receiving noninvasive positive-pressure ventilation. Respir Care. 2006;51(2):126–32.PubMedGoogle Scholar
  59. 59.
    Wijkstra PJ, Lacasse Y, Guyatt GH, et al. A meta-analysis of nocturnal noninvasive positive pressure ventilation in patients with stable COPD. Chest. 2003;124(1):337–43.PubMedCrossRefGoogle Scholar
  60. 60.
    Garrod R, Mikelsons C, Paul EA, Wedzicha JA. Randomized controlled trial of domiciliary noninvasive positive pressure ventilation and physical training in severe chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2000;162(4 Pt 1):1335–41.PubMedCrossRefGoogle Scholar
  61. 61.
    Duiverman ML, Wempe JB, Bladder G, et al. Nocturnal non-invasive ventilation in addition to rehabilitation in hypercapnic patients with COPD. Thorax. 2008;63(12):1052–7.PubMedCrossRefGoogle Scholar
  62. 62.
    Duiverman ML, Wempe JB, Bladder G, et al. Two-year home-based nocturnal noninvasive ventilation added to rehabilitation in chronic obstructive pulmonary disease patients: a randomized controlled trial. Respir Res. 2011;23(12):112.CrossRefGoogle Scholar
  63. 63.
    Dreher M, Storre JH, Windisch W. Noninvasive ventilation during walking in patients with severe COPD: a randomised cross-over trial. Eur Respir J. 2007;29(5):930–6.PubMedCrossRefGoogle Scholar
  64. 64.
    Jones SE, Packham S, Hebden M, Smith AP. Domiciliary nocturnal intermittent positive pressure ventilation in patients with respiratory failure due to severe COPD: long-term follow up and effect on survival. Thorax. 1998;53(6):495–8.PubMedPubMedCentralCrossRefGoogle Scholar
  65. 65.
    Funk GC, Breyer MK, Burghuber OC, et al. Long-term non-invasive ventilation in COPD after acute-on-chronic respiratory failure. Respir Med. 2011;105(3):427–34.PubMedCrossRefGoogle Scholar
  66. 66.
    Heinemann F, Budweiser S, Jörres RA, et al. The role of non-invasive home mechanical ventilation in patients with chronic obstructive pulmonary disease requiring prolonged weaning. Respirology. 2011;16(8):1273–80.PubMedCrossRefGoogle Scholar
  67. 67.
    Windisch W. Impact of home mechanical ventilation on health-related quality of life. Eur Respir J. 2008;32(5):1328–36.PubMedCrossRefGoogle Scholar
  68. 68.
    Dreher M, Kenn K, Windisch W. Nichtinvasive Beatmung und körperliche Belastung bei Patienten mit COPD. Pneumologie. 2008;62(3):162–8.PubMedCrossRefGoogle Scholar
  69. 69.
    McEvoy RD, Pierce RJ, Hillman D, et al. Nocturnal non-invasive nasal ventilation in stable hypercapnic COPD: a randomised controlled trial. Thorax. 2009;64:516–4Google Scholar
  70. 70.
    Fletcher EC, Donner CF, Midgren B, et al. Survival in COPD patients with a daytime PaO2 greater than 60 mm Hg with and without nocturnal oxyhemoglobin desaturation. Chest. 1992;101(3):649–55.PubMedCrossRefGoogle Scholar
  71. 71.
    Andreas S, et al. Tabakentwöhnung bei COPD – S3-Leitlinie herausgegeben von der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin. Pneumologie. 2008;62(5):255–72.PubMedCrossRefGoogle Scholar
  72. 72.
    Lichtenschopf, A. Richtlinien der Tabakentwöhnung Stand 2010. Wien Klin Wochenschr. 2011;123:1–17.CrossRefGoogle Scholar
  73. 73.
    Criner GJ, Brennan K, Travaline JM, Kreimer D. Efficacy and compliance with noninvasive positive pressure ventilation in patients with chronic respiratory failure. Chest. 1999;116(3):667–75.PubMedCrossRefGoogle Scholar
  74. 74.
    Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev. 2013;4:CD002769.PubMedGoogle Scholar
  75. 75.
    Flight WG, Shaw J, Johnson S, et al. Long-term non-invasive ventilation in cystic fibrosis – experience over two decades. J Cyst Fibros. 2012;11(3):187–92.PubMedCrossRefGoogle Scholar
  76. 76.
    Holland AE, Denehy L, Ntoumenopoulos G, Naughton MT, Wilson JW. Non-invasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis. Thorax. 2003;58(10):880–4.PubMedPubMedCentralCrossRefGoogle Scholar
  77. 77.
    Wedzicha JA, Muir JF. Noninvasive ventilation in chronic obstructive pulmonary disease, bronchiectasis and cystic fibrosis. Eur Respir J. 2002;20(3):777–84.PubMedCrossRefGoogle Scholar
  78. 78.
    Leger P, Bedicam JM, Cornette A, et al. Nasal intermittent positive pressure ventilation. Long-term follow-up in patients with severe chronic respiratory insufficiency. Chest. 1994;105(1):100–5.PubMedCrossRefGoogle Scholar
  79. 79.
    Simonds AK, Elliott MW. Outcome of domiciliary nasal intermittent positive pressure ventilation in restrictive and obstructive disorders. Thorax. 1995;50(6):604–9.PubMedPubMedCentralCrossRefGoogle Scholar
  80. 80.
    Midgren B, Olofson J, Harlid R, Dellborg C, Jacobsen E, Norregaard O. Home mechanical ventilation in Sweden, with reference to Danish experiences. Swedish Society of Chest Medicine. Respir Med. 2000;94(2):135–8.PubMedCrossRefGoogle Scholar
  81. 81.
    Windisch W, Dreher M. NIV and chronic respiratory failure secondary to restrictive thoracic disorders. In: Muir JF, Ambrosino N, Simonds AK, Herausgeber Noninvasive ventilation. K. Larsson (Ed. in Chief). European Respiratory Monograph. 2nd. Herausgeber. Sheffield: ERS Journals; S. 240–250.Google Scholar
  82. 82.
    Shneerson JM, Simonds AK. Noninvasive ventilation for chest wall and neuromuscular disorders. Eur Respir J. 2002;20(2):480–7.PubMedCrossRefGoogle Scholar
  83. 83.
    Simonds AK. Home ventilation. Eur Respir J. 2003;47:38–46.CrossRefGoogle Scholar
  84. 84.
    Goldberg A, et al. Clinical indications for noninvasive positive pressure ventilation in chronic respiratory failure due to restrictive lung disease, COPD, and nocturnal hypoventilation – a consensus conference report. Chest. 1999;116(2):521–34.CrossRefGoogle Scholar
  85. 85.
    Nickol AH, Hart N, Hopkinson NS, et al. Mechanisms of improvement of respiratory failure in patients with restrictive thoracic disease treated with non-invasive ventilation. Thorax. 2005;60:754–60.PubMedPubMedCentralCrossRefGoogle Scholar
  86. 86.
    Fauroux B, Lofaso F. Non-invasive mechanical ventilation: when to start for what benefit? Thorax. 2005;60(12):979–80.PubMedPubMedCentralCrossRefGoogle Scholar
  87. 87.
    Perrin C, D’Ambrosio C, White A, Hill NS. Sleep in restrictive and neuromuscular respiratory disorders. Semin Respir Crit Care Med. 2005;26(1):117–30.PubMedCrossRefGoogle Scholar
  88. 88.
    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. 2005;60(12):1019–24.PubMedPubMedCentralCrossRefGoogle Scholar
  89. 89.
    Masa JF, Celli BR, Riesco JA, et al. Noninvasive positive pressure ventilation and not oxygen may prevent overt ventilatory failure in patients with chest wall diseases. Chest. 1997;112(1):207–13.PubMedCrossRefGoogle Scholar
  90. 90.
    Budweiser S, Murbeth RE, Jorres RA, Heinemann F, Pfeifer M. Predictors of long-term survival in patients with restrictive thoracic disorders and chronic respiratory failure undergoing non-invasive home ventilation. Respirology. 2007;12(4):551–9.PubMedCrossRefGoogle Scholar
  91. 91.
    Storre JH, Steurer B, Kabitz H, Dreher M, Windisch W. Transcutaneous PCO2 monitoring during initiation of noninvasive ventilation. Chest. 2007;132(6):1810–6.PubMedCrossRefGoogle Scholar
  92. 92.
    Fletcher EC. Survival in COPD patients with a daytime PaO2 greater than 60 mm Hg with and without nocturnal oxyhemoglobin desaturation. Chest. 1992;101(3):649–55.PubMedCrossRefGoogle Scholar
  93. 93.
    Becker HF. Breathing during sleep in patients with nocturnal desaturation. Am J Respir Crit Care Med. 1999;159(1):112–8.PubMedCrossRefGoogle Scholar
  94. 94.
    Schönhofer 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. 1997;10(1):84–91.Google Scholar
  95. 95.
    Restrick LJ, Fox NC, Braid G, et al. Comparison of nasal pressure support ventilation with nasal intermittent positive pressure ventilation in patients with nocturnal hypoventilation. Eur Respir J. 1993;6(3):364–70.PubMedGoogle Scholar
  96. 96.
    Tejeda M, Boix JH, Alvarez F, Balanza R, Morales M. Comparison of pressure support ventilation and assist-control ventilation in the treatment of respiratory failure. Chest. 1997;111(5):1322–5.PubMedCrossRefGoogle Scholar
  97. 97.
    Windisch W, Storre JH, Sorichter S, Virchow JCJ. Comparison of volume- and pressure-limited NPPV at night: a prospective randomized cross-over trial. Respir Med. 2005;99(1):52–9.PubMedCrossRefGoogle Scholar
  98. 98.
    Tuggey JM, Elliott MW. Randomised crossover study of pressure and volume non-invasive ventilation in chest wall deformity. Thorax. 2005;60(10):859–64.PubMedPubMedCentralCrossRefGoogle Scholar
  99. 99.
    Struik FM, Duiverman ML, Meijer PM, et al. Volume targeted vs. pressure-targeted noninvasive ventilation in patients with chest-wall deformity: a pilot study. Respir Care. 2011;56(10):1522–5.PubMedCrossRefGoogle Scholar
  100. 100.
    Ferris G, Servera-Pieras E, Vergara P, et al. Kyphoscoliosis ventilatory insufficiency: noninvasive management outcomes. Am J Phys Med Rehabil. 2000;79(1):24–9.PubMedCrossRefGoogle Scholar
  101. 101.
    Domenech-Clar R, Nauffal-Manzur D, Perpina-Tordera M, Compte-Torrero L, Macian-Gisbert V. Home mechanical ventilation for restrictive thoracic diseases: effects on patient quality-of-life and hospitalizations. Respir Med. 2003;97(12):1320–7.PubMedCrossRefGoogle Scholar
  102. 102.
    Ellis ER, Grunstein RR, Chan S, Bye PT, Sullivan CE. Noninvasive ventilatory support during sleep improves respiratory failure in kyphoscoliosis. Chest. 1988;94(4):811–5.PubMedCrossRefGoogle Scholar
  103. 103.
    Gonzalez C, Ferris G, Diaz J, et al. Kyphoscoliotic ventilatory insufficiency: effects of long-term intermittent positive-pressure ventilation. Chest. 2003;124(3):857–62.PubMedCrossRefGoogle Scholar
  104. 104.
    Nauffal D, Domenech R, Martinez Garcia MA, et al. Noninvasive positive pressure home ventilation in restrictive disorders: outcome and impact on health-related quality of life. Respir Med. 2002;96(10):777–83.PubMedCrossRefGoogle Scholar
  105. 105.
    Schönhofer B, Wallstein S, Wiese C, Kohler D. Noninvasive mechanical ventilation improves endurance performance in patients with chronic respiratory failure due to thoracic restriction. Chest. 2001;119(5):1371–8.PubMedCrossRefGoogle Scholar
  106. 106.
    Schönhofer 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. 2001;56(7):524–8.PubMedPubMedCentralCrossRefGoogle Scholar
  107. 107.
    Buyse B, Meersseman W, Demedts M. Treatment of chronic respiratory failure in kyphoscoliosis: oxygen or ventilation? Eur Respir J. 2003;22(3):525–8.PubMedCrossRefGoogle Scholar
  108. 108.
    Budweiser S, Heinemann F, Fischer W, et al. Impact of ventilation parameters and duration of ventilator use on non-invasive home ventilation in restrictive thoracic disorders. Respiration. 2006;73(4):488–94.PubMedCrossRefGoogle Scholar
  109. 109.
    Contal O, Janssens JP, Dury M, et al. Sleep in ventilatory failure in restrictive thoracic disorders. Effects of treatment with non invasive ventilation. Sleep Med. 12(2011):373–377.PubMedCrossRefGoogle Scholar
  110. 110.
    Carrey Z, Gottfried SB, Levy RD. Ventilatory muscle support in respiratory failure with nasal positive pressure ventilation. Chest. 1990;97(1):150–8.PubMedCrossRefGoogle Scholar
  111. 111.
    Goldstein RS, De Rosie JA, Avendano MA, Dolmage TE. Influence of noninvasive positive pressure ventilation on inspiratory muscles. Chest. 1991;99(2):408–15.PubMedCrossRefGoogle Scholar
  112. 112.
    Kohler D. Noninvasive ventilation works in all restrictive diseases with hypercapnia whatever the cause. Respiration. 2001;68(5):450–1.PubMedCrossRefGoogle Scholar
  113. 113.
    Annane D, Quera-Salva MA, Lofaso F, et al. Mechanisms underlying effects of nocturnal ventilation on daytime blood gases in neuromuscular diseases. Eur Respir J. 1999;13(1):157–62.PubMedCrossRefGoogle Scholar
  114. 114.
    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. 2000. 94(12):1154–60.PubMedCrossRefGoogle Scholar
  115. 115.
    Estenne M, Gevenois PA, Kinnear W, et al. Lung volume restriction in patients with chronic respiratory muscle weakness: the role of microatelectasis. Thorax. 1993;48(7):698–701.PubMedPubMedCentralCrossRefGoogle Scholar
  116. 116.
    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. 2008;133(1):156–60.PubMedCrossRefGoogle Scholar
  117. 117.
    Gustafson T, Franklin KA, Midgren B. Survival of patients with kyphoscoliosis receiving mechanical ventilation or oxygen at home. Chest. 2006;130(6):1828–33.PubMedCrossRefGoogle Scholar
  118. 118.
    Magnussen H, Kirsten A, Kohler D. Leitlinien zur Langzeit-Sauerstofftherapie. Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin e. V. Pneumologie. 2008;62(12):748–56.PubMedCrossRefGoogle Scholar
  119. 119.
    Janssens JP, Cicotti E, Fitting JW, Rochat T. Non-invasive home ventilation in patients over 75 years of age: tolerance, compliance, and impact on quality of life. Respir Med. 1998;92(12):1311–20.PubMedCrossRefGoogle Scholar
  120. 120.
    Piper AJ, Grunstein RR. Current perspectives on the obesity hypoventilation syndrome. Curr Opin Pulm Med. 2007;13(6):490–6.PubMedGoogle Scholar
  121. 121.
    Kessler R, Chaouat A, Schinkewitch P, et al. The obesity hypoventilation syndrome revisited: A prospective study of 34 consecutive cases. Chest. 2001;120(2):369–376.PubMedCrossRefGoogle Scholar
  122. 122.
    Mokhlesi B, Tulaimat A. Recent advances in obesity hypoventilation syndrome. Chest. 2007;132(4):1322–36.PubMedCrossRefGoogle Scholar
  123. 123.
    Banerjee D, Yee BJ, Piper AJ, Zwillich CW, Grunstein RR. Obesity hypoventilation syndrome: hypoxemia during continuous positive airway pressure. Chest. 2007;131(6):1678–84.PubMedCrossRefGoogle Scholar
  124. 124.
    Sullivan CE, Berthon-Jones M, Issa FG. Remission of severe obesity-hypoventilation syndrome after short term treatment during sleep with nasal continuous positive airway pressure. Am Rev Respir Dis. 1983;128(1):177–81.PubMedCrossRefGoogle Scholar
  125. 125.
    Rochester DF, Enson Y. Current concepts in the pathogenesis of the obesity hypoventilation syndrome. Mechanical and circulatory factors. Am J Med. 1974;57(3):402–20.PubMedCrossRefGoogle Scholar
  126. 126.
    Sugerman HJ, Fairman RP, Sood RK, Engle K, Wolfe L, Kellum JM. Long-term effects of gastric surgery for treating respiratory insufficiency of obesity. Am J Clin Nutr. 1992;55(2):597–601.Google Scholar
  127. 127.
    Miller A, Granada M. In-hospital mortality in the Pickwickian syndrome. Am J Med. 1974;56(2):144–50.PubMedCrossRefGoogle Scholar
  128. 128.
    Nowbar S, Burkart KM, Gonzales R, et al. Obesity-associated hypoventilation in hospitalized patients: prevalence, effects, and outcome. Am J Med. 2004;116(1):1–7.PubMedCrossRefGoogle Scholar
  129. 129.
    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. 2008;63(5):395–401.PubMedCrossRefGoogle Scholar
  130. 130.
    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. 2001;120(4):1231–8.PubMedCrossRefGoogle Scholar
  131. 131.
    Murphy PB, Davidson C, Hind MD, et al. Volume targeted versus pressure support non-invasive ventilation in patients with super obesity and chronic respiratory failure: a randomised controlled trial. Thorax. 2012;67:727–34.PubMedCrossRefGoogle Scholar
  132. 132.
    Windisch W, Storre JH. Target volume settings for home mechanical ventilation: great progress or just a gadget? Thorax. 2012;67:663–5.PubMedCrossRefGoogle Scholar
  133. 133.
    Perrin C, Unterborn JN, Ambrosio CD, et al. Pulmonary complications of chronic neuromuscular diseases and their management. Muscle Nerve. 2004;29:5–27.PubMedCrossRefGoogle Scholar
  134. 134.
    Simonds AK. Domiciliary non-invasive ventilation in restrictive disorders and stable neuromuscular disease. In: Simonds AK, Herausgeber. Non-invasive Respiratory Support: a Practical Handbook. London: Arnold; 2001. S. 133–45.Google Scholar
  135. 135.
    Kabitz H, Windisch W. Respiratory muscle testing: state of the art. Pneumologie. 2007;61(9):582–7.PubMedCrossRefGoogle Scholar
  136. 136.
    Bach JR, et al. Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis. Chest. 2004;126(5):1502–7.PubMedCrossRefGoogle Scholar
  137. 137.
    Bach JR, Gonçalves MR, Hamdani I, Winck JC. Extubation of patients with neuromuscular weakness: a new management paradigm. Chest. 2010;137(5):1033–9.PubMedCrossRefGoogle Scholar
  138. 138.
    Simonds AK, Cowie MR. Taboo: crossing the specialty barrier. Eur Resp J. 2005;31:1153–4.CrossRefGoogle Scholar
  139. 139.
    Geiseler J, Karg O. Management of secretion in patients with neuromuscular diseases. Pneumologie. 2008;62(1):43–8.CrossRefGoogle Scholar
  140. 140.
    Carratù P, Spicuzza L, Cassano A, et al. Early treatment with noninvasive positive pressure ventilation prolongs survival in Amyotrophic Lateral Sclerosis patients with nocturnal respiratory insufficiency. Orphanet J Rare Dis. 2009;10:4–10.Google Scholar
  141. 141.
    Lechtzin N, Scott Y, Busse AM, Clawson LL, Kimball R, Wiener CM. Early use of non-invasive ventilation prolongs survival in subjects with ALS. Amyotroph Lateral Scler. 2007;8(3):185–8.PubMedCrossRefGoogle Scholar
  142. 142.
    Miller RG, Jackson CE, Kasarskis EJ, et al. Quality Standards Subcommittee of the American Academy of Neurology. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009;73(15):1227–33.PubMedPubMedCentralCrossRefGoogle Scholar
  143. 143.
    Radunovic A, Annane D, Rafiq MK, Mustafa N. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev. 2013;3. doi: 10.1002/14651858.CD004427.
  144. 144.
    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. 2003;22:631–6.PubMedCrossRefGoogle Scholar
  145. 145.
    Mellies U, Dohna-Schwake C, Ragette R, Teschler H, Voit T. Nächtliche nichtinvasive Beatmung bei Kindern und Jugendlichen mit neuromuskulären Erkrankungen: Einfluss auf Schlaf und Symptome. Wien Klin Wochenschr. 2003;115:855–9.PubMedCrossRefGoogle Scholar
  146. 146.
    Simonds AK, Ward S, Heather S, Bush A, Muntoni F. Outcome of paediatric domiciliary mask ventilation in neuromuscular and skeletal disease. Eur Respir J. 2000;16:476–81.PubMedCrossRefGoogle Scholar
  147. 147.
    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. 2005;60:1019–24.PubMedPubMedCentralCrossRefGoogle Scholar
  148. 148.
    Wallgren-Pettersson C, Bushby K, Mellies U, Simonds AK. ENMC. 117th ENMC workshop: ventilatory support in congenital neuromuscular disorders: congenital myopathies, congenital muscular dystrophies, congenital myotonic dystrophy and SMA (II). Neuromuscul Disord. 2004;14:56–69.PubMedCrossRefGoogle Scholar
  149. 149.
    Finder JD, Birnkrant D, Carl J, et al. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med. 2004;170:456–65.PubMedCrossRefGoogle Scholar
  150. 150.
    Mellies U, Dohna-Schwake C. Neuromuscular disorders. In: Hammer J, Eber E, Herausgeber. Paediatric pulmonary function testing. Progress in respiratory research. Bd. 33. Basel: Karger; 2005. S. 233–46.CrossRefGoogle Scholar
  151. 151.
    Fauroux B, Aubertin G, Lofaso F. NIV and chronic respiratory failure in children. Eur Respir Mon. 2008;41:272–286.Google Scholar
  152. 152.
    Hammer J. Home mechanical ventilation in children: indications and practical aspects. Schweiz Med Wochenschr. 2000;130:1894–902.PubMedGoogle Scholar
  153. 153.
    Holland AE, Denehy L, Ntoumenopoulos G, Naughton MT, Wilson JW. Non-invasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis. Thorax. 2003;58:880–4.PubMedPubMedCentralCrossRefGoogle Scholar
  154. 154.
    Moran F, Bradley JM, Jones AP, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev. 2007;(4):CD002769.Google Scholar
  155. 155.
    Young AC, Wilson JW, Kotsimbos TC, Naughton MT. Randomised placebo controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis. Thorax. 2008;63:72–7.PubMedCrossRefGoogle Scholar
  156. 156.
    Fauroux B, Le Roux E, Ravilly S, Bellis G, Clement A. Long term non-invasive ventilation in patients with cystic fibrosis. Respiration. 2008;76:168–74.PubMedCrossRefGoogle Scholar
  157. 157.
    Flight WG, Shaw J, Johnson S, et al. Long-term non-invasive ventilation in cystic fibrosis – Experience over two decades. J Cyst Fibros. 2012;11:187–92.PubMedCrossRefGoogle Scholar
  158. 158.
    Eber E, Oberwaldner B. Tracheostomy care in the hospital. Paediatr Respir Rev. 2006;7:175–84.PubMedCrossRefGoogle Scholar
  159. 159.
    Trachsel D, Hammer J. Indications for tracheostomy in children. Paediatr Respir Rev. 2006;7:162–8.PubMedCrossRefGoogle Scholar
  160. 160.
    Oberwaldner B, Eber E. Tracheostomy care in the home. Paediatr Respir Rev. 2006;7(3):185–90.PubMedCrossRefGoogle Scholar
  161. 161.
    Dohna-Schwake C, Ragette R, Teschler H, Voit T, Mellies U. Predictors of severe chest infections in pediatric neuromuscular disorders. Neuromuscul Disord. 2006;16:325–8.PubMedCrossRefGoogle Scholar
  162. 162.
    Healy F, Marcus CL. Congenital central hypoventilation syndrome in children. Paediatr Respir Rev. 2011;12:253–63.PubMedCrossRefGoogle Scholar
  163. 163.
    Weese-Mayer DE, Berry-Kravis EM, Ceccherini I, Keens TG, Loghmanee DA, Trang H. ATS Congenital Central Hypoventilation Syndrome Subcommittee. An official ATS clinical policy statement: congenital central hypoventilation syndrome: genetic basis, diagnosis, and management. Am J Respir Crit Care Med. 2010;181:626–44.PubMedCrossRefGoogle Scholar
  164. 164.
    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. 2003;21:502–8.PubMedCrossRefGoogle Scholar
  165. 165.
    Fauroux B, Guillemont N, Aubertin G, et al. Physiologic benefits of mechanical insufflation-exsufflation in children with neuromuscular diseases. Chest. 2008;133:161–8.PubMedCrossRefGoogle Scholar
  166. 166.
    Simonds AK. Ethical aspects of home long term ventilation in children with neuromuscular disease. Paediatr Respir Rev. 2005;6:209–14.PubMedCrossRefGoogle Scholar
  167. 167.
    Zeggwagh AA, et al. Weaning from mechanical ventilation: a model extubation. Intensive Care Med. 1999;25(10):1077–83.PubMedCrossRefGoogle Scholar
  168. 168.
    Bach JR, Scaporito LR. Criteria for extubation and trachestomy tube removal for patients with ventilatory failure. A different approach to weaning. Chest. 1996;110:1566–71.PubMedCrossRefGoogle Scholar
  169. 169.
    Evans JA, Whitelaw WA. The assessment of maximal respiratory mouth pressures in adults. Respir Care. 2009;54(10):1348–59.PubMedGoogle Scholar
  170. 170.
    Hautmann H, Hefele S, Schotten K, et al. Maximal inspiratory mouth pressures (PIMAX) in healthy subjects what is the lower limit of normal? Respir Med. 2000;94(7):689–93.PubMedCrossRefGoogle Scholar
  171. 171.
    Turner LA, Tecklenburg-Lund SL, Chapman RF, et al. Inspiratory muscle training lowers the oxygen cost of voluntary hyperpnea. J Appl Physiol. 2012;112(1):127–34.PubMedCrossRefGoogle Scholar
  172. 172.
    Weiner P, Gross D, Meiner Z, et al. Respiratory muscle training in patients with moderate to severe myasthenia gravis. J Neurol Sci. 1998;25(3):236–41.CrossRefGoogle Scholar
  173. 173.
    Hsiao SF, Wu YT, Wu HD, Wang TG. Comparison of effectiveness of pressure threshold and targeted resistance devices for inspiratory muscle training in patients with chronic obstructive pulmonary disease. J Formos Med Assoc. 2003;102(4):240–5.PubMedGoogle Scholar
  174. 174.
    Covey MK, Larson JL, Wirtz SE, et al. High-intensity inspiratory muscle training in patients with chronic obstructive pulmonary disease and severely reduced function. J Cardiopulm Rehabil. 2001;21(4):231–40.PubMedCrossRefGoogle Scholar
  175. 175.
    Troche MS, Okun MS, Rosenbek JC, et al. Aspiration and swallowing in Parkinson disease and rehabilitation with EMST: a randomized trial. Neurology. 2010;75(21):1912–9.PubMedPubMedCentralCrossRefGoogle Scholar
  176. 176.
    Pitts T, Bolser D, Rosenbek J, et al. Impact of expiratory muscle strength training on voluntary cough and swallow function in Parkinson disease. Chest. 2009;135(5):1301–8.PubMedCrossRefGoogle Scholar
  177. 177.
    Fusco C, Zaina F, Atanasio S, et al. Physical exercises in the treatment of adolescent idiopathic scoliosis: an updated systematic review. Physiother Theory Pract. 2011;27(1):80–114.PubMedCrossRefGoogle Scholar
  178. 178.
    Bennell KL, Matthews B, Greig A, et al. Effects of an exercise and manual therapy program on physical impairments, function and quality-of-life in people with osteoporotic vertebral fracture: a randomised, single-blind controlled pilot trial. BMC Musculoskelet Disord. 2010;17:11–36.Google Scholar
  179. 179.
    Weiss HR. The effect of an exercise program on vital capacity and rib mobility in patients with idiopathic scoliosis. Spine. 1991;16:88–93.PubMedCrossRefGoogle Scholar
  180. 180.
    Menadue C, Alison JA, Piper AJ, et al. High- and low-level pressure support during walking in people with severe kyphoscoliosis. Eur Respir J. 2010;36(2):370–8.PubMedCrossRefGoogle Scholar
  181. 181.
    Hornstein S, Inman S, Ledsome JR. Ventilatory muscle training in kyphoscoliosis. Spine. 1987;12:859–63.PubMedCrossRefGoogle Scholar
  182. 182.
    Schmidt I. Assisted cough-physiotherapy to improve expectoration of mucus. Pneumologie. 2008;62(1):23–7.CrossRefGoogle Scholar
  183. 183.
    Aboussouan LS. Mechanisms of exercise limitation and pulmonary rehabilitation for patients with neuromuscular disease. Chron Respir Dis. 2010;6(4):231–49.Google Scholar
  184. 184.
    Dalbello-Haas V, Florence JM, Krivickas LS, et al. Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst Rev. 2008;16(2):CD005229.Google Scholar
  185. 185.
    Pinto AC, Alves M, Nogueira A, et al. Can amyotrophic lateral sclerosis patients with respiratory insuffiency exercise? J Neurol Sci. 1999;169(1–2):69–75.PubMedCrossRefGoogle Scholar
  186. 186.
    Sirithangkul S, Ranganathan S, Robinson PJ, et al. Positive expiratory pressure to enhance cough effectiveness in tracheomalacia. J Med Assoc Thai. 2000;93 Suppl 6:112–8.Google Scholar
  187. 187.
    De Backer LA, Ides K, Daems D, et al. Pulmonary rehabilitation and non-invasive ventilation in COPD Acta. Clin Belg. 2010;65(5):330–5.CrossRefGoogle Scholar
  188. 188.
    Corner E, Garrod R. Does the addition of non-invasive ventilation during pulmonary rehabilitation in patients with chronic obstructive pulmonary disease augment patient outcome in exercise tolerance? A literature review. Physiother Res Int. 2010;15(1):5–15.PubMedGoogle Scholar
  189. 189.
    Gosselink R, De Vos J, van den Heuvel SP, et al. Impact of inspiratory muscle training in patients with COPD: what is the evidence? Eur Respir J. 2011;37(2):416–25.PubMedCrossRefGoogle Scholar
  190. 190.
    Migliori C, Cavazza A, Motta M, Bottino R, Chirico G. Early use of Nasal-BiPAP in two infants with congenital central hypoventilation syndrome. Acta Paediatrica. 2003;92(7):823–6.PubMedCrossRefGoogle Scholar
  191. 191.
    Messineo A, Giusti F, Name S, Mognato G, Antoniello L, Guglielmi M. The safety of home tracheostomy care for children. J Pediatr Surg. 1995;30:1246–8.PubMedCrossRefGoogle Scholar
  192. 192.
    Oberwaldner B. Tracheostoma. In: Rieger C, von der Hardt H, Sennhauser FH, Wahn U, Zach M, Herausgeber. Pädiatrische Pneumologie. Berlin: Springer; 2004. S. 412–417. ISBN 3-540-43627–8.Google Scholar
  193. 193.
    Tearl DK, Hertzog JH. Home discharge of technology-dependent children: evaluation of a respiratory-therapist driven family education program. Respir Care. 2007;52(2):171–6.PubMedGoogle Scholar
  194. 194.
    Dajlby RW, Hogg JC. Effect of breathing dry air on structure and function of airways. J Appl Physiol. 1980;61:312–7.Google Scholar
  195. 195.
    Mirza S, Hopkinson L, Malik TH, Willatt DJ. The use of inhalers in patients with tracheostomas or tracheostomy tubes. J Laryngol Otol. 1999;113:762–4.PubMedCrossRefGoogle Scholar
  196. 196.
    Kerem E, Yatsiv I, Goitein KJ. Effects of endotracheal suctioning on arterial blood gases in children. Intensive Care Med. 1990;7:227–31.Google Scholar
  197. 197.
    Raymond S. Normal saline instillation before suctioning: helpful or harmful? A review of the literature. Am J Crit Care. 1995;4:267–71.PubMedGoogle Scholar
  198. 198.
    Toussaint M, Chatwin M, Soudon P. Mechanical ventilation in Duchenne patients with chronic respiratory insufficiency: clinical implications of 20 years published experience. Chron Respir Dis. 2007;4(3):167–77.PubMedCrossRefGoogle Scholar
  199. 199.
    Toussaint M, Steens M, Wasteels G, Soudon P. Diurnal ventilation via mouthpiece; survival in end-stage Duchenne patients. Eur Resp J. 2006;28(3):549–55.CrossRefGoogle Scholar
  200. 200.
    Wildhaber JH. Grundlagen der Inhalationstherapie. In: Rieger C, von der Hardt H, Sennhauser FH, Wahn U, Zach M, Herausgeber. Pädiatrische Pneumologie. Berlin: Springer; 2004. S. 371–378. ISBN 3-540-43627-8.Google Scholar
  201. 201.
    Oberwaldner B. Physiotherapie. In: Rieger C, von der Hardt H, Sennhauser FH, Wahn U, Zach M, Herausgeber. Pädiatrische Pneumologie. Berlin: Springer; 2004. S. 379–389. ISBN 3-540-43627-8.Google Scholar

Copyright information

© Springer-Verlag Wien 2016

Authors and Affiliations

  • Peter Schenk
    • 1
  • Ernst Eber
    • 2
  • Georg-Christian Funk
    • 3
  • Wilfried Fritz
    • 4
  • Sylvia Hartl
    • 3
  • Peter Heininger
    • 5
  • Eveline Kink
    • 6
  • Gernot Kühteubl
    • 1
  • Beatrice Oberwaldner
    • 7
  • Ulrike Pachernigg
    • 2
  • Andreas Pfleger
    • 2
  • Petra Schandl
    • 8
  • Ingrid Schmidt
    • 3
  • Markus Stein
    • 9
  1. 1.Abteilung für PulmologieLandesklinikum HocheggGrimmensteinÖsterreich
  2. 2.Klinische Abteilung für Pädiatrische Pulmonologie und Allergologie, Universitätsklinik für Kinder- und JugendheilkundeMedizinische Universität GrazGrazÖsterreich
  3. 3.I. Interne Lungenabteilung, Pulmologisches Zentrum, Sozialmedizinisches Zentrum Baumgartner HöheOtto Wagner SpitalWienÖsterreich
  4. 4.Klinische Abteilung für Lungenkrankheiten, Universitätsklinik für Innere MedizinUniversitätsklinikum GrazGrazÖsterreich
  5. 5.Rehabilitationszentrum MünsterMünsterÖsterreich
  6. 6.Abteilung für LungenkrankheitenLandeskrankenhaus Hörgas-EnzenbachEisbachÖsterreich
  7. 7.Medizinische Universität GrazGrazÖsterreich
  8. 8.1. Allgemeine IntensivstationWilhelminenspitalWienÖsterreich
  9. 9.Abteilung für PneumologieLandeskrankenhaus Hochzirl-Natters, Standort NattersNattersÖsterreich

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