Jervell and Lange-Nielsen syndrome


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

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