Jervell和Lange-Nielsen综合征。家庭病例(临床观察)

T. Tolstikova, L. Bregel, S.V. Czurkan, T. Marchuk, A. V. Dudnik
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引用次数: 1

摘要

本文报告2例儿童Jervell - Lange-Nielsen综合征的临床家庭病例。长QT综合征是儿童心源性猝死的主要原因之一。Jervell和Lange-Nielsen综合征是长QT综合征中最严重和罕见的类型之一。该病的症状出现在婴儿期;其特点是心电图QT间期延长,危及生命的室性心动过速和心室颤动导致晕厥,并伴有先天性双侧听力损失。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Jervell and Lange-Nielsen syndrome. Family case (clinical observation)
A clinical family case of Jervell and Lange-Nielsen syndrome in 2 children is presented. Long QT syndrome is one of the leading causes of sudden cardiac death in children. Jervell and Lange-Nielsen syndrome is one of the most severe and rare types of long QT syndrome. Symptoms of the disease appear in infancy; they are characterized by a lengthening of QT interval on ECG, syncope as a result of life-threatening ventricular tachycardia and ventricular fibrillation, combined with congenital bilateral hearing loss.
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