Long QT Syndrome with Nocturnal Cardiac Events Caused by a KCNH2 Missense Mutation (G604S)
スポンサーリンク
概要
- 論文の詳細を見る
An 8-year-old boy suffered from an unconsciousness attack and torsade de pointes arrhythmia during sleep or at rest. His electrocardiogram showed prolonged QT intervals, but the T wave morphology was atypical for type 1, 2 or 3 congenital long-QT syndrome (LQTS). Intravenous epinephrine slightly prolonged the QT interval whereas mexiletine infusion shortened the QT interval. Although these clinical characteristics might suggest type 3 LQTS, a genetic analysis identified the G604S-KCNH2 mutation (type 2 LQTS). Because mismatches between the genotype and phenotype of LQTS are possible, genetic analysis of LQTS is important to identify the most appropriate therapeutic option and risk stratification.
著者
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Sato Akinori
Division of Cardiology, Niigata University Graduate School of Medical and Dental Sciences
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Furushima Hiroshi
Division Of Cardiology Hemotology And Endocrinology Niigata University Graduate School Of Medical An
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Chinushi Masaomi
School Of Health Science Niigata University School Of Medicine
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Iijima Kenichi
Division Of Cardiology Niigata University Graduate School Of Medical And Dental Science
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Suzuki Hiroshi
Department Of Allergy And Immunology National Research Institute For Child Health & Development
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Watanabe Hiroshi
Division Of Cardiology Hemotology And Endocrinology Niigata University Graduate School Of Medical And Dental Science
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Numano Fujito
Department of Pediatrics, Niigata University Medical and Dental Hospital, Japan
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Hanyu Takanori
Department of Pediatrics, Niigata University Medical and Dental Hospital, Japan
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