| Case Reports : A case of KearnsSayre syndrome complicated with complete AV block |
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In Hae Park, Jong Kun Lim, Jeong Geun Moon, Bo Yong Joung, Moon Hyong Lee, Sung Soon Kim |
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한림의대 한강성심병원 내과 |
| 증례 : 완전방실차단이 발생한 Kearns-Sayre syndrome 1예 |
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박인혜, Jong Kun Lim, Jeong Geun Moon, Bo Yong Joung, Moon Hyong Lee, Sung Soon Kim |
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| Abstract |
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KearnsSayre syndrome (KSS) is a mitochondrial DNA disorder characterized by the onset before age 20 years, progressive external opthalomoplegia, atypical retinal pigmentation and cardiac conduction disturbance. This report describes a 24-year-old woman who experienced syncope due to complete atrioventricular block complicated by KSS. At 12 years old, she was diagnosed KSS. The consecutive change of EKG shows typical progression of cardiac conduction disturbance of KSS. She was successfully treated with implantation of a pacemaker.(Korean J Med 70:564-568, 2006)
Key Words : Kearns-Sayre syndrome, Complete atrioventricular block, Cardiac pacemaker |
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