A case of KearnsSayre syndrome complicated with complete AV block.
- Author:
In Hae PARK
1
;
Jong Kun LIM
;
Jeong Geun MOON
;
Bo Yong JOUNG
;
Moon Hyong LEE
;
Sung Soon KIM
Author Information
1. Department of Internal Medicine and Research Institute, Yonsei Universitiy College of Medicine, Seoul, Korea. cby6908@yumc.yonsei.ac.kr
- Publication Type:Case Report
- Keywords:
Kearns-Sayre syndrome;
Complete atrioventricular block;
Cardiac pacemaker
- MeSH:
Atrioventricular Block*;
Child;
DNA, Mitochondrial;
Electrocardiography;
Female;
Humans;
Kearns-Sayre Syndrome;
Pigmentation;
Retinaldehyde;
Syncope;
Young Adult
- From:Korean Journal of Medicine
2006;70(5):564-568
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
