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Pulmonary Rehabilitation in Patients with Neuromuscular Disease

강성웅
연세대학교의과대학 (Yonsei University College of Medicine)|2006.06|Yonsei Medical Journal|제47권 제3호|pp. 307-314

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참고문헌

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초록

In neuromuscular disease (NMD) patients with progressive muscle weakness, respiratory muscles are also affected and hypercapnia can increase gradually as the disease progresses. The fundamental respiratory problems NMD patients experience are decreased alveolar ventilation and coughing ability. For these reasons, it is necessary to precisely evaluate pulmonary function to provide the proper inspiratory and expiratory muscle aids in order to maintain adequate respiratory function. As inspiratory muscle weakening progresses, NMD patients experience hypoventilation. At this point, respiratory support by mechanical ventilator should be initiated to relieve respiratory distress symptoms. Patients with adequate bulbar muscle strength and cognitive function who use a non-invasive ventilation aid, via a mouthpiece or a nasal mask, may have their hypercapnia and associated symptoms resolved. For a proper cough assist, it is necessary to provide additional insufflation to patients with inspiratory muscle weakness before using abdominal thrust. Another effective method for managing airway secretions is a device that performs mechanical insufflation-exsufflation. In conclusion, application of non-invasive respiratory aids, taking into consideration characterization of respiratory pathophysiology, have made it possible to maintain a better quality of life in addition to prolonging the life span of patients with NMD.

Abstract

In neuromuscular disease (NMD) patients with progressive muscle weakness, respiratory muscles are also affected and hypercapnia can increase gradually as the disease progresses. The fundamental respiratory problems NMD patients experience are decreased alveolar ventilation and coughing ability. For these reasons, it is necessary to precisely evaluate pulmonary function to provide the proper inspiratory and expiratory muscle aids in order to maintain adequate respiratory function. As inspiratory muscle weakening progresses, NMD patients experience hypoventilation. At this point, respiratory support by mechanical ventilator should be initiated to relieve respiratory distress symptoms. Patients with adequate bulbar muscle strength and cognitive function who use a non-invasive ventilation aid, via a mouthpiece or a nasal mask, may have their hypercapnia and associated symptoms resolved. For a proper cough assist, it is necessary to provide additional insufflation to patients with inspiratory muscle weakness before using abdominal thrust. Another effective method for managing airway secretions is a device that performs mechanical insufflation-exsufflation. In conclusion, application of non-invasive respiratory aids, taking into consideration characterization of respiratory pathophysiology, have made it possible to maintain a better quality of life in addition to prolonging the life span of patients with NMD.

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관련 논문AI추천

Noninvasive Respiratory Management of Patients With Neuromuscular DiseaseJohn R. Bach대한재활의학회 · Annals of Rehabilitation Medicine2017 · 제41권 제4호Successful Extubation After Weaning Failure by Noninvasive Ventilation in Patients With Neuromuscular Disease: Case Series강성웅, 김선미, 박윤길, 원유휘, 최영철대한재활의학회 · Annals of Rehabilitation Medicine2017 · 제41권 제3호Improvement of Peak Cough Flow After the Application of a Mechanical In-exsufflator in Patients With Neuromuscular Disease and Pneumonia: A Pilot Study강성웅, 박지현, 서미리, 오현준, 이장우, 정지호, 최원아대한재활의학회 · Annals of Rehabilitation Medicine2018 · 제42권 제6호A Comparison of Cough Assistance Techniques in Patients with Respiratory Muscle Weakness강성웅, 김선미, 원유희, 최원아연세대학교의과대학 · Yonsei Medical Journal2016 · 제57권 제6호Comparison of Pulmonary Functions at Onset of Ventilatory Insufficiency in Patients With Amyotrophic Lateral Sclerosis, Duchenne Muscular Dystrophy, and Myotonic Muscular Dystrophy이장우, 조한얼, Hyeonjun Oh, Kil Chan Lee, Seong Woong Kang, Won Ah Choi대한재활의학회 · Annals of Rehabilitation Medicine2016 · 제40권 제1호희귀ㆍ난치성 신경근육계 질환자의 호흡부전 관리 실태 조사강성웅, 김동현, 문재호, 박중현, 백종훈, 서정환, 신용범, 최승호, 최원아대한재활의학회 · Annals of Rehabilitation Medicine2010 · 제34권 제3호

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