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KMID : 0368119920220010160
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Korean Circulation Journal
1992 Volume.22 No. 1 p.160 ~ p.165
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Emery-Dreifuss Muscular Dystrophy with Cardiac Involvement
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박주현
승기배/김종진/노태호/채장성/김재형/홍순조/최규보
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Abstract
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Emery-Dreifuss muscular dystrophy has become recognized as a distinct neuromuscular disorder with features including X-liniked inheritance, insidious onset in childhood of a distinect pattern of muscle contractures and weakness. slow progression
without
loss of ambulation, and occurrence by mid-childhood of atrialconduction defects, which, if untreated, cause sudden death.
We report a case of Emery-Dreifuss dystrophy with cardiac involvement of atrial standstill. The patient was 24 year-old man, who had suffered from dyspnea and bradycardia and was inserted by VVI type permanent pacemaker.
Cardiac involvement usually becomes evident as muscle weakness progress and provided that the diagnosis is made sufficiently early. the inserrtion of a cardiac pacemaker can be life saving.
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KEYWORD
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