Propranolol syrup to tablets change triggers electrical storm in Jervell-Lange-Nielsen syndrome
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DOI: 10.1017/s1047951120003285
Abstract: Abstract A 10-year-old girl with genetically confirmed Jervell-Lange-Nielsen syndrome treated with beta-blocker and developed electrical storm after changing propranolol syrup to tablets. Jervell-Lange-Nielsen is characterised by long QT and congenital sensorineural deafness, with high risk… read more here.
Keywords: electrical storm; syrup tablets; nielsen syndrome; propranolol syrup ... See more keywords
Molecular mechanisms of function deficiencies in KCNQ1 variants associated with Jervell and Lange–Nielsen syndrome
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DOI: 10.1080/19336950.2025.2580177
Abstract: ABSTRACT Jervell and Lange-Nielsen syndrome (JLNS) is characterized by congenital bilateral sensorineural hearing loss, a prolonged QT interval (QTc) on an electrocardiogram (ECG), and a high incidence of sudden death in childhood. More than 90%… read more here.
Keywords: nielsen syndrome; lange nielsen; jervell lange; kcnq1 variants ... See more keywords
A PAS-targeting hERG1 activator reduces arrhythmic events in Jervell and Lange-Nielsen syndrome patient-derived hiPSC-CMs
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DOI: 10.1172/jci.insight.183444
Abstract: The hERG1 potassium channel conducts the cardiac repolarizing current, IKr. hERG1 has emerged as a therapeutic target for cardiac diseases marked by prolonged action potential duration (APD). Unfortunately, many hERG1 activators display off-target and proarrhythmic… read more here.
Keywords: jervell lange; pas; herg1; lange nielsen ... See more keywords