Jervell and Lange-Nielsen syndrome

(redirected from Jervell 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.

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
References in periodicals archive ?
Out of these KCNQ1 which is a potassium channel gene, its heterozygous mutation causes RWS while its homozygous mutation causes a much rarer condition Jervell Lange-Nielsen syndrome in which along with long QT interval deafness also occurs.1-9
Two phenotypes of Long QT Syndrome include Romano-Ward and Jervell Lange-Nielsen syndromes. Romano-Ward Syndrome is the more benign variant with severity ranging from asymptomatic and undiagnosed to sudden cardiac death [7].