| 先天性长QT综合征的研究进展 |
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| 引用本文： | 夏忆. 先天性长QT综合征的研究进展[J]. 国际儿科学杂志, 2011, 38(4): 353-356. DOI: 10.3760/cma.j.issn.1673-4408.2011.04.014 |
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| 作者姓名： | 夏忆 |
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| 作者单位： | 华中科技大学同济医学院附属协和医院儿科,武汉,430022 |
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| 摘 要： | 先天性长QT综合征(LQTS)是心肌细胞膜离子通道功能异常导致心肌细胞复极时间延长的心脏传导异常疾病.心电图特征为QT间期延长和尖端扭转性室性心动过速.临床表现为反复发生心源性昏厥,甚至猝死.目前分子遗传学研究已经发现与LQTS相关的至少12个突变基因,分别定位于3、4、7、11、12、17、20和21号染色体上,控制...
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| 关 键 词： | 先天性长QT综合征 心律失常 突变位点 基因诊断 |
Research progress of congenital long QT syndrome |
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| XIA Yi. Research progress of congenital long QT syndrome[J]. International Journal of Pediatrics, 2011, 38(4): 353-356. DOI: 10.3760/cma.j.issn.1673-4408.2011.04.014 |
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| Authors: | XIA Yi |
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| Abstract: | Congenital long QT syndrome ( LQTS) is a cardiac ion channel dysfunction, leading to prolonged myocardial repolarization time. It is characterized by the typical ECG QT interval prolongation and torsades de pointes. It shows clinical recurrence of cardiogenic syncope and even lead to sudden death. Molecular genetic studies have revealed a total of 12 forms of congenital LQTS caused by mutations in genes of the potassium, sodium and calcium channels or membrane adapter located on chromosomes 3, 4, 7, 11, 12, 17, 20 and 21. This review summarized the studies of the pathogenesis of LQTS and gene-related treatments. |
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| Keywords: | Congenital long QT syndrome Arhythmia Mutable site Gene diagnosis |
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