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Five-Year Follow-Up and Outcomes of Noninvasive Ventilation in Subjects With Neuromuscular Diseases

 Mi Ri Suh  ;  Won Ah Choi  ;  Dong Hyun Kim  ;  Jang Woo Lee  ;  Eun Young Kim  ;  Seong-Woong Kang 
 RESPIRATORY CARE, Vol.63(3) : 274-281, 2018 
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amyotrophic lateral sclerosis ; congenital myopathy ; duchenne muscular dystrophy ; neuromuscular disease ; noninvasive ventilation ; spinal muscular atrophy
INTRODUCTION: The purpose of this study was to investigate the 5-year outcomes of noninvasive ventilation (NIV) application in different neuromuscular disease (NMD) groups. METHODS: We categorized 180 subjects who had initiated NIV between March 2001 and August 2009 into 4 groups and followed them for > 5 y. The NIV maintenance rate and average duration, applying time, and forced vital capacity (FVC) were investigated at the time NIV was initiated and 5 y after NIV initiation in each group. RESULTS: In subjects with amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and spinal muscular atrophy (SMA)-congenital myopathy, the 5-year subjects who continued to use NIV over time were 22.5%, 89.4%, and 91.3%, respectively, and the average NIV maintenance durations were 21.53 ± 19.26 months, 55.22 ± 11.47 months, and 57.48 ± 8.34 months, respectively (P < .001). Median daily applying time changed from 8.0 h to 24.0 h (P < .001), from 8.0 h to 12.0 h (P < .001), and from 8.0 h to 9.0 h (P = .11) in subjects with ALS, DMD, and SMA-congenital myopathy, respectively. FVC decreased significantly after 5 y except in the group with combined SMA-congenital myopathy. CONCLUSIONS: NIV was tolerated long-term without significant increases in daily application time for most subjects with NMD. However, in individuals with ALS, development of severe bulbar symptoms can risk maintaining NIV.
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1. College of Medicine (의과대학) > Dept. of Rehabilitation Medicine (재활의학교실) > 1. Journal Papers
Yonsei Authors
Kang, Seong Woong(강성웅) ORCID logo https://orcid.org/0000-0002-7279-3893
Kim, Eun Young(김은영) ORCID logo https://orcid.org/0000-0001-6622-219X
Suh, Mi Ri(서미리)
Choi, Won Ah(최원아) ORCID logo https://orcid.org/0000-0003-0403-8869
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