Jer·vell and Lange-Niel·sen syn·drome

(yer-vel' lahng'ĕ nēl'sĕn), [MIM*220440 and MIM*176261]

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

Farlex Partner Medical Dictionary © Farlex 2012

Jervell,

Anton, Norwegian cardiologist, 1901–.

Jervell and Lange-Nielsen syndrome - a prolonged Q-T interval recorded in the electrocardiogram of certain congenitally deaf children subject to Adams-Stokes seizures and ventricular fibrillation. Synonym(s): surdocardiac syndrome

Lange-Nielsen,

F., 20th century Norwegian cardiologist.

Jervell and Lange-Nielsen syndrome - see under Jervell

Medical Eponyms © Farlex 2012

References in periodicals archive

The prevalence of Jervell and Lange-Nielsen syndrome in deaf population has been investigated in different studies.

The Jervell and Lange-Nielsen syndrome: natural history, molecular basis, and clinical outcome.

JERVELL AND LANGE-NIELSEN SYNDROME IN DEAF SCHOOL CHILDREN POPULATION

KCNE1 mutations cause Jervell and Lange-Nielsen syndrome. Nature Genet.

Genotype and Severity of Long QT Syndrome

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