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한국인 성인발생 및 후기발생 근긴장디스트로피제 1형 환자의 임상 특성

Other Titles
 Clinical Characteristics of the Korean Patients with Adult Onset and Late Onset Myotonic Dystrophy Type 1 
 김현정  ;  김대현  ;  박윤길 
 Journal of the Korean Association of EMG-Electrodiagnostic Medicine (대한근전도 전기진단의학회지), Vol.16(2) : 64-69, 2014 
Journal Title
 Journal of the Korean Association of EMG-Electrodiagnostic Medicine (대한근전도 전기진단의학회지) 
Issue Date
myotonic dystrophy ; complication ; CTG repeats
Objectives: To investigate the clinical characteristics and complications of Korean patients with myotonic dystrophy type 1 (DM1). Method: The medical records of 65 DM1 patients were reviewed retrospectively. We assessed the relationship between the age of onset, muscular impairment rating scale (MIRS) and complications of the DM1 with the number of cytosine-thymine-guanine (CTG) repeats. Results: The mean age of onset was 30.5 ± 10.3 years and the mean disease duration until diagnosis was 6.1 ± 6.0 years. The age of onset was inversely correlated to the number of CTG repeats (P = 0.034). For the systemic complications, 20 of 55 patients (36.4%) had arrhythmia, 18 of 49 patients (36.7%) needed ventilator support, 4 of 52 (7.7%) had diabetes mellitus and 19 of 35 (54.3%) had cataracts. The difference between the number of CTG repeats of patients with these complications and that of patients without complications was not significant. Conclusion: Regular follow-ups of electrocardiogram, pulmonary function test, blood sugar test and ocular examination is recommended for the patients with DM1.
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Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Rehabilitation Medicine (재활의학교실) > 1. Journal Papers
Yonsei Authors
Kim, Dae Hyun(김대현)
Park, Yoon Ghil(박윤길) ORCID logo https://orcid.org/0000-0001-9054-5300
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