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Journal of the Korean Academy of Rehabilitation Medicine 2010;34(3):347-354.
A Research on the Management of Ventilatory Insufficiency in Patients with Neuromuscular Diseases.
Kim, Dong Hyun , Kang, Seong Woong , Choi, Wonah , Moon, Jae Ho , Baek, Jong Hoon , Choi, Seung Ho , Park, Jung Hyun , Shin, Yong Beom , Seo, Jung Hwan
1Department of Rehabilitation Medicine and Pulmonary Rehabilitation Center, Yonsei University College of Medicine, Korea. kswoong@yuhs.ac
2Department of Rehabilitation Medicine, Daejeon Eulji University Hospital, Eulji University College of Medicine, Korea.
3Department of Rehabilitation Medicine, Pusan National University School of Medicine, Korea.
4Department of Rehabilitation Medicine, Chonbuk National University Medical School, Korea.
희귀ㆍ난치성 신경근육계 질환자의 호흡부전 관리 실태 조사
김동현, 강성웅, 최원아, 문재호, 백종훈, 최승호, 박중현1, 신용범2, 서정환3
연세대학교 의과대학 재활의학교실 및 호흡재활센터, 1을지의과대학교 대전 을지병원 재활의학과, 2부산대학교 의학전문대학원 재활의학교실, 3전북대학교 의학전문대학원 재활의학교실
To investigate the real condition of pulmonary rehabilitation for patients with advanced neuromuscular diseases (NMDs) on mechanical ventilation in Korea.
In order to estimate current state of pulmonary rehabilitative management, chart review and pulmonary function evaluation were conducted in a total of 267 NMD patients who had applied mechanical home ventilator in our center from March 2001 to December 2008.
Total 267 patients were included: 95 with Duchenne muscular dystrophy, 69 with other types of myopathy, 83 with amyotrophic lateral sclerosis (ALS), 20 with spinal muscular atrophy. Among them, 18 who were previously intubated and 17 patients who had undergone tracheostomy were switched into volume-limited non-invasive ventilation (NIV). At the time of hospital discharge, 234 patients were applied NIV successfully. Twenty other patients who once used continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP) ventilators inappropriately were switched into volume-limited NIV. However, 20 patients who had successfully applied NIV first underwent tracheostomy due to exacerbation of underlying disease.
Adequate pulmonary management is the only promising method to prevent lethal complications, and to prolong life span of advanced NMD patients. We assume that more NMD patients can improve their quality of life and prolong their life through proper pulmonary rehabilitation including regular pulmonary function check-ups and ventilatory state monitoring as well as early NIV application. (J Korean Acad Rehab Med 2010; 34: 347-354)
Key Words: Neuromuscular disease, Non-invasive ventilation, Pulmonary rehabilitation, Mechanical ventilation


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