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Titolo:
Clinical implications for affected parents and siblings of probands with long-QT syndrome
Autore:
Kimbrough, J; Moss, AJ; Zareba, W; Robinson, JL; Hall, J; Benhorin, J; Locati, EH; Medina, A; Napolitano, C; Priori, S; Schwartz, PJ; Timothy, K; Towbin, JA; Vincent, GM; Zhang, L;
Indirizzi:
Univ Rochester, Med Ctr, Heart Res Follow Up Program, Dept Med,Cardiol Unit, Rochester, NY 14642 USA Univ Rochester Rochester NY USA 14642 rdiol Unit, Rochester, NY 14642 USA Univ Rochester, Med Ctr, Dept Biostat, Rochester, NY 14642 USA Univ Rochester Rochester NY USA 14642 pt Biostat, Rochester, NY 14642 USA Vanderbilt Univ, Med Ctr, Dept Med, Nashville, TN USA Vanderbilt Univ Nashville TN USA v, Med Ctr, Dept Med, Nashville, TN USA Bikur Cholim Hosp, Dept Cardiol, Jerusalem, Israel Bikur Cholim Hosp Jerusalem Israel osp, Dept Cardiol, Jerusalem, Israel Univ Perugia, Cardiol Sect, I-06100 Perugia, Italy Univ Perugia Perugia Italy I-06100 Cardiol Sect, I-06100 Perugia, Italy Univ Pavia, Dept Cardiol, I-27100 Pavia, Italy Univ Pavia Pavia Italy I-27100 Pavia, Dept Cardiol, I-27100 Pavia, Italy IRCCS, Policlin San Matteo, Pavia, Italy IRCCS Pavia ItalyIRCCS, Policlin San Matteo, Pavia, Italy IRCCS Fdn Maugeri, Mol Cardiol Lab, Pavia, Italy IRCCS Fdn Maugeri PaviaItaly dn Maugeri, Mol Cardiol Lab, Pavia, Italy Latter Day St Hosp, Dept Med, Salt Lake City, UT 84143 USA Latter Day St Hosp Salt Lake City UT USA 84143 lt Lake City, UT 84143 USA Texas Childrens Hosp, Baylor Coll Med, Dept Pediat, Houston, TX 77030 USA Texas Childrens Hosp Houston TX USA 77030 t Pediat, Houston, TX 77030 USA
Titolo Testata:
CIRCULATION
fascicolo: 5, volume: 104, anno: 2001,
pagine: 557 - 562
SICI:
0009-7322(20010731)104:5<557:CIFAPA>2.0.ZU;2-T
Fonte:
ISI
Lingua:
ENG
Soggetto:
CARDIAC-ARRHYTHMIA; MUTATIONS; GENE; FORMS; HERG;
Keywords:
electrocardiography; long-QT syndrome; risk factors; sex; genetics;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
16
Recensione:
Indirizzi per estratti:
Indirizzo: Moss, AJ Univ Rochester, Med Ctr, Heart Res Follow Up Program, Dept Med,Cardiol Unit, Box 653, Rochester, NY 14642 USA Univ Rochester Box 653 Rochester NY USA 14642 ester, NY 14642 USA
Citazione:
J. Kimbrough et al., "Clinical implications for affected parents and siblings of probands with long-QT syndrome", CIRCULATION, 104(5), 2001, pp. 557-562

Abstract

Background-Whenever a proband is identified with long-QT syndrome (LQTS), his or her parents and siblings should be evaluated regarding the possibility of carrying, the disorder. In the majority of cases, one of the proband's parents and one or more siblings are affected. The aim of this study was (1) to determine whether the clinical severity of LQTS in the proband is useful in identifying first-degree family members at high risk for cardiac events, and (2) to evaluate the clinical course of affected parents and siblings of LQTS probands. Methods and Results-The clinical and ECG characteristics of 211 LQTS probands and 791 first-degree relatives (422 parents and 369 siblings) were studied to determine if the clinical profile of the proband is useful in determining the clinical severity of LQTS in affected parents and siblings. Affected female parents of an LQTS proband had a greater cumulative risk for a first cardiac event than affected male parents. The probability of a parent or sibling having a first cardiac event was not significantly influenced bythe severity of the proband's clinical symptoms. Female sex and QT(c) duration were risk factors for cardiac events among affected parents, and QT(c)was the only risk factor for cardiac events in affected siblings. Conclusions-The severity profile of LQTS in a proband was not found to be useful in identifying the clinical severity of LQTS in affected first-degree relatives of the proband.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 09/04/20 alle ore 13:18:10