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As per Schwartz's criteria, 31 (36.9%) out of 84 children with Long QTc (8.17% in sample population), scored high points (4.0 to 6.0), proving presence of JLNS.
Key Words: Long QT interval, Jervell and Lange-Nielsen Syndrome (JLNS), Congenital deafness, Electrocardiographs.
Classically, this disease has been described as having apparent autosomal-recessive transmission.7 These patients have the identical clinical presentation as those with RWS, but also have associated sensorineural deafness.[7,17,18] Individuals with JLNS usually have longer QT intervals as compared to individuals with RWS and also have a more malignant course.
GENETICS AND PHYSIOLOGY OF AUTOSOMAL-RECESSIVE LQTS (JLNS)
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