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Jervell and Lange-Nielsen syndrome

Pronunciation: yer-vel' lahng'e nel'sen

Definition: a prolonged Q-T interval recorded in the electrocardiogram of certain congenitally deaf children subject to attacks of unconsciousness resulting from Adams-Stokes seizures and ventricular fibrillation; autosomal recessive inheritance, caused by homozygosity for a mutation in the potassium channel gene (KVLQT1) on chromosome 11 or minimal potassium ion channel gene (KCNE1) on 21.

Synonym(s): surdocardiac syndrome

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© Copyright 2017 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.
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