Overview

Jervell–Lange-Nielsen syndrome


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An autosomal recessive disorder characterized by congenital deafness, prolongation of the QT interval (long QT), ventricular arrhythmias, and a high risk of sudden death. The cause is mutation in the genes for KCNQ1 or KCNE1, which are components of the delayed rectifier potassium channel.

Subjects: Medicine and Health.


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