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Titolo:
Long QT syndrome.
Autore:
Lupoglazoff, JM; Denjoy, I; Guicheney, P; Casasoprana, A; Coumel, P;
Indirizzi:
Hop Robert Debre, Serv Cardiol Infantile, F-75019 Paris, France Hop RobertDebre Paris France F-75019 l Infantile, F-75019 Paris, France Hop Lariboisiere, Serv Cardiol, F-75475 Paris, France Hop Lariboisiere Paris France F-75475 erv Cardiol, F-75475 Paris, France Grp Hosp Pitie Salpetriere, INSERM U523, Inst Myol, F-75634 Paris, France Grp Hosp Pitie Salpetriere Paris France F-75634 l, F-75634 Paris, France
Titolo Testata:
ARCHIVES DE PEDIATRIE
fascicolo: 5, volume: 8, anno: 2001,
pagine: 525 - 534
SICI:
0929-693X(200105)8:5<525:LQS>2.0.ZU;2-3
Fonte:
ISI
Lingua:
FRE
Soggetto:
BETA-BLOCKER THERAPY; CARDIAC-ARRHYTHMIA; POTASSIUM CHANNEL; MOLECULAR-BASIS; HERG; MUTATIONS; GENE; INTERVAL; KVLQT1; RISK;
Keywords:
long QT syndrome; death, sudden, cardiac; torsades de pointes; genetics; prevention and control;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
42
Recensione:
Indirizzi per estratti:
Indirizzo: Lupoglazoff, JM Hop Robert Debre, Serv Cardiol Infantile, 48 Blvd Serurier, F-75019 Paris,France Hop Robert Debre 48 Blvd Serurier Paris France F-75019 ce
Citazione:
J.M. Lupoglazoff et al., "Long QT syndrome.", ARCH PED, 8(5), 2001, pp. 525-534

Abstract

Long QT syndrome (LQTS) is a clinically and genetically heterogenous syndrome characterized by a lengthening of the QT interval on the surface ECG and a propensity to severe ventricular arrhythmias such as torsades de pointes and ventricular fibrillation, leading eventually to syncope and sudden death. This rare syndrome with a mendelian inheritance occurs in subjects with otherwise normal cardiac morphological examination. The potentially severe prognosis justifies a presymptomatic diagnosis. The genetic nature of thedisease has been confirmed with the identification of at least six loci and five genes. This syndrome is a perfect illustration of an adrenergic-induced ventricular arrhythmia. The first-line treatment is a beta-blocking agent for all symptomatic patients. In addition, a number of drugs known to lengthen ventricular repolarization must be prohibited. In case of suspicion of LQTS, all family members should be tested both clinically with a surfaceECG and genetically in order to diagnose presymptomatic patients. (C) 2001editions scientifiques et medicales Elsevier SAS.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 06/04/20 alle ore 02:03:56