Jervell and Lange-Nielsen syndrome: Neurologic and cardiologic evaluation

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dc.authorwosidKomsuoglu, Sezer Sener/ABE-3464-2021
dc.contributor.authorIlhan, A
dc.contributor.authorTuncer, C
dc.contributor.authorKomsuoglu, SS
dc.contributor.authorKali, S
dc.date.accessioned2024-08-04T20:11:59Z
dc.date.available2024-08-04T20:11:59Z
dc.date.issued1999
dc.departmentİnönü Üniversitesien_US
dc.description.abstractRecurrent syncope, malignant ventricular arrhythmias, and sudden death are complications of the long QT syndrome (LQTS), Two well-known syndromes with long QT intervals are known. The Jervell and Lange-Nielsen syndrome (JLNS) is characterized by prolongation of the QT interval, deafness, and autosomal-recessive inheritance, and the Romano-Ward syndrome is characterized by a prolonged QT interval, autosomal-dominant inheritance, and no deafness. In the present study assessment was performed of the diagnostic importance of the ventricular derepolarization parameters, clinical features, and prevalence of JLNS among 132 children with congenital hearing loss (CHL), In the CHL group the mean QT, QTc, JT, and JTc intervals and the dispersion values (QT-d, JT-d, QTc-d, and JTc-d) were significantly longer than those of control subjects (n = 96) (P < 0.05). Patients with CHL and JLNS (n = 5) had significantly longer mean values of QT, QTc, JT, and JTc intervals and dispersion values than those of CHL without JLNS (n = 127) and control subjects (P < 0.05). The results suggest that assessment of ventricular derepolarization parameters in children with CHL will be helpful in the early detection of JLNS because infants with CHL cannot accurately describe the symptoms of syncope, (C) 1999 by Elsevier Science Inc. All rights reserved.en_US
dc.identifier.doi10.1016/S0887-8994(99)00100-9
dc.identifier.endpage813en_US
dc.identifier.issn0887-8994
dc.identifier.issue5en_US
dc.identifier.pmid10593671en_US
dc.identifier.scopus2-s2.0-0032698401en_US
dc.identifier.scopusqualityN/Aen_US
dc.identifier.startpage809en_US
dc.identifier.urihttps://doi.org/10.1016/S0887-8994(99)00100-9
dc.identifier.urihttps://hdl.handle.net/11616/93146
dc.identifier.volume21en_US
dc.identifier.wosWOS:000083810400006en_US
dc.identifier.wosqualityQ2en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherElsevier Science Incen_US
dc.relation.ispartofPediatric Neurologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectLong-Qt Syndromeen_US
dc.subjectPotassium Channel Geneen_US
dc.subjectCardiac-Arrhythmiaen_US
dc.subjectDispersionen_US
dc.subjectHeterogeneityen_US
dc.subjectIntervalsen_US
dc.subjectMutationen_US
dc.subjectRecoveryen_US
dc.titleJervell and Lange-Nielsen syndrome: Neurologic and cardiologic evaluationen_US
dc.typeArticleen_US

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