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Clinical Implications of Routine Monitoring of Pulmonary Function and Ventilation in Patients with Duchenne Muscular Dystrophy

 Han Eol Cho  ;  Jang Woo Lee  ;  Won Ah Choi  ;  Seong-Woong Kang 
 YONSEI MEDICAL JOURNAL, Vol.63(6) : 578-584, 2022-06 
Journal Title
Issue Date
Humans ; Lung ; Muscular Dystrophy, Duchenne* ; Noninvasive Ventilation* / methods ; Respiration ; Retrospective Studies
Muscular dystrophy ; Duchenne ; hypercapnia ; mechanical ventilations ; noninvasive ventilation ; respiratory function tests ; respiratory insufficiency
Purpose: To investigate the effect of regular monitoring of pulmonary function and ventilatory status on the initiation of non-invasive ventilation (NIV) between patients who were routinely monitored before receiving NIV and those who were not.

Materials and methods: This retrospective cohort study included subjects with Duchenne muscular dystrophy (DMD) who first received NIV between 2010 and 2019. The subjects were assigned to either the regular-follow-up (REG) group or the non-REG group, according to their follow-up status, before initiating NIV. We compared the number of emergent cases, the results of nocturnal ventilatory monitoring, and the pulmonary function of each group at initial ventilatory support.

Results: In total, 73 subjects were enrolled in the REG group and 47 subjects in the non-REG group. There were significantly more emergency cases due to respiratory insufficiency in the non-REG group (12/47, 25.5%) than in the REG group (3/73, 4.1%). At the time of initial ventilatory support, hypoventilatory symptoms were more common and relatively severe in the non-REG group (37/47, 78.7%) than in the REG group (18/73, 24.7%). The average age at initial ventilatory support of the non-REG group was 2.15 years older than that of the subjects in the REG group. Moreover, subjects who were not regularly monitored exhibited greater deterioration in pulmonary function compared to those who were regularly followed up.

Conclusion: Regular evaluation of pulmonary function and ventilatory status before the onset of ventilatory insufficiency is crucial to reduce the risk of patients with DMD requiring emergency care due to ventilatory insufficiency.
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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
Cho, Han Eol(조한얼) ORCID logo https://orcid.org/0000-0001-5625-3013
Choi, Won Ah(최원아) ORCID logo https://orcid.org/0000-0003-0403-8869
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