[1]房晨鹂,范新荣,蔡琳.Timothy综合征的研究进展[J].心血管病学进展,2016,(1):81-83.[doi:10.16806/j.cnki.issn.1004-3934.2016.01.021]
 FANG Chenli,FAN Xinrong,CAI Lin.Review of Current Research of Timothy Syndrome[J].Advances in Cardiovascular Diseases,2016,(1):81-83.[doi:10.16806/j.cnki.issn.1004-3934.2016.01.021]
点击复制

Timothy综合征的研究进展()
分享到:

《心血管病学进展》[ISSN:51-1187/R/CN:1004-3934]

卷:
期数:
2016年1期
页码:
81-83
栏目:
综述
出版日期:
2016-02-20

文章信息/Info

Title:
Review of Current Research of Timothy Syndrome
作者:
房晨鹂范新荣蔡琳
成都市第三人民医院心内科 成都市心血管病研究所,四川 成都 610041
Author(s):
FANG Chenli FAN Xinrong CAI Lin
Department of Cardiology,The Third People's Hospital of Chengdu,Chengdu Cardiovascular Research Institute,Chengdu 610041,Sichuan,China
关键词:
遗传性长QT综合征 Timothy综合征 L型钙通道 治疗策略
Keywords:
Hereditary long-QT syndrome Timothy syndrome L-type calcium channels Strategy of treatment
分类号:
R596.1
DOI:
10.16806/j.cnki.issn.1004-3934.2016.01.021
文献标志码:
A
摘要:
Timothy综合征,即遗传性长QT综合征的8型,是由于编码L型钙通道的CACNA1C基因发生突变而产生的罕见的多器官功能障碍的遗传性疾病,常导致严重的表型异常以及过早死亡。目前对Timothy综合征的治疗方法包括β受体阻滞剂、安置心脏起搏器、使用埋藏式心脏复律除颤器、左侧颈胸交感神经切除术等,但这些方法的有效性尚缺乏证据。
Abstract:
The long QT syndrome type 8,known as Timothy syndrome,is a rare multiorgan dysfunction caused by a CACNA1C mutation that always accompanied by severe phenotypes and early fatality. To date,several therapies including beta-adrenergic receptor blockers,pacemakers,implantable cardioverter defibrillator and left cervicothoracic sympathectomy are used for these patients,but no data concerning their effectiveness are available.

参考文献/References:

[1] Moss AJ,Schwartz PJ,Crampton RS,et al.The long QT syndrome. Prospective longitudinal study of 328 families[J]. Circulation,1991,84:1136-1134.
[2] Schwartz PJ,Stramba-Badiale M,Crotti L,et al. Prevalence of the congenital long-QT syndrome[J]. Circulation,2009,120:1761-1767.
[3] Schwartz PJ,Ackerman MJ,George AL,et al. Impact of genetics on the clinical management of channelopathies[J]. J Am Coll Cardiol,2013,62:169-180.
[4] Splawski I,Timothy KW,Sharpe LM,et al. Ca(V)1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism[J]. Cell,2004,119(1):19-31.
[5] Splawski I,Timothy KW,Decher N,et al. Severe arrhythmia disorder caused by cardiac L-type calcium channel mutations[J]. Proc Natl Acad Sci USA,2005,102(23):8089-8096.
[6] Catterall WA. Structure and function of voltage-gated sodium and calcium channels[J]. Curr Opin Neurobiol,1991,1:5-13.
[7] Jane G,Elena B,Charles A,et al. Long QT,syndactyly, joint contracutres, stroke and novel CACNA1C mutation: expanding the spectrum of Timothy syndrome[J]. Am J Med Genet A,2012,158A(1):182-187.
[8] Reichenbach H, Meister EM, Theile H. The heart-hand syndrome. A new variant of disorders of heart conduction and syndactylia including osseous changes in hands and feet[J]. Kinderarztl Prax,1992,60: 54-56.
[9] Marks ML, Whisler SL, Clericuzio C, et al. A new form of long QT syndrome associated with syndactyly[J]. J Am Coll Cardiol,1995,25:59-64.
[10] Njoe SM, Wilde AA, van Erven L, et al.Syndactyly and long QT syndrome(CaV1.2 missense mutation G640R)is associated with hypertrophic cardiomyopathy[J]. Heart Rhythm,2005,2:1365-1368.
[11] Fröhler S, Kieslich M, Langnick C, et al. Exome sequencing helped the fine diagnosis two siblings affected with atypical Timothy syndrome(TS2)[J]. BMC Med Genet,2014,15: 48.

备注/Memo

备注/Memo:
作者简介:房晨鹂(1984—),住院医师,硕士,主要从事心血管疾病,尤其是老年心血管疾病、自身免疫性心血管疾病的研究。 Email:fclmonica@sina.com
更新日期/Last Update: 2016-02-20