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Topic: Familial long QT syndrome

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	Long QT Syndromes
		Aggravation of congenital long QTc syndrome by astemizole is postulated.
		The congenital long-QT syndrome (LQTS) is characterized by prolonged QT intervals, QT interval lability, and polymorphic ventricular tachycardia.
		BACKGROUND: The long QT syndrome is an inherited disorder with prolonged ventricular repolarization and a propensity to ventricular tachyarrhythmias and sudden arrhythmic death.
	www.pediheart.org /searches/topic/longqt.htm (19169 words)

	Selected Encyclopedia of Heart Diseases and Conditions
		An abnormally long interval between the Q and the T wave (see the section on ECG) called "long QT syndrome," is another common type of arrhythmia and can occur from a variety of both inherited and non-inherited problems.
		Ellis-van Creveld syndrome is characterized by atrial septal defects (see congenital heart defects) which in severe cases result in the affected individual having a single combined atrium.
		This is a form of long QT syndrome (see cardiac arrhythmia and ECG) that is inherited as an autosomal dominant trait.
	www.hhmi.org /biointeractive/vlabs/cardiology/content/cg/encyclopedia.html (1751 words)

	eMedicine - Long QT Syndrome : Article Excerpt by: Wojciech Zareba, MD, PhD, FACC
		Background: Long QT syndrome (LQTS) is a congenital disorder characterized by a prolongation of the QT interval on electrocardiogram (ECG) and a propensity to ventricular tachyarrhythmias, which may lead to syncope, cardiac arrest, or sudden death.
		Pathophysiology: The QT interval on the ECG, measured from the beginning of the QRS complex to the end of the T wave, represents the duration of activation and recovery of the ventricular myocardium.
		QT prolongation in LQTS is due to overload of myocardial cells with positively charged ions during ventricular repolarization.
	www.emedicine.com /med/byname/long-qt-syndrome.htm (502 words)

	UniProtKB/Swiss-Prot entry P51787 [KCNQ1_HUMAN] Potassium voltage-gated channel subfamily KQT member 1
		Long QT syndromes are heart disorders characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias.
		JLNS is an autosomal recessive cardio-auditory syndrome characterized by a prolonged QT interval in the electrocardiogram and congenital deafness.
		Short QT syndromes are heart disorders characterized by idiopathic persistently and uniformly short QT interval on ECG in the absence of structural heart disease in affected individuals.
	www.expasy.org /uniprot/P51787 (1907 words)

	Andersen-Tawil syndrome - Genetics Home Reference
		Anderson-Tawil syndrome is a disorder that causes episodes of muscle weakness (periodic paralysis), changes in heart rhythm (arrhythmia), and developmental abnormalities.
		Long QT syndrome is a heart condition that causes the heart (cardiac) muscle to take longer than usual to recharge between beats.
		Mutations in the KCNJ2 gene cause Andersen-Tawil syndrome.
	ghr.nlm.nih.gov /condition=andersentawilsyndrome (742 words)

	Genomics\|HuGENet\|Reviews\|References\|longQT syndrome\|PubMed ID: 16540748
		A founder mutation of the potassium channel KCNQ1 in long QT syndrome: implications for estimation of disease prevalence and molecular diagnostics.
		Bradycardia-induced long QT syndrome caused by a de novo missense mutation in the S2-S3 inner loop of HERG.
		Long QT syndrome in children: the value of the rate corrected QT interval in children who present with fainting.
	www.cdc.gov /genomics/hugenet/reviews/tables/longQT_Ref.htm (1336 words)

	WHO2
		Familial long QT syndrome is a dominant disorder characterized by prolonged ventricular repolarization, which produces recurrent syncopal attacks and sudden death due to ventricular tachyarrhythmias.
		Before the molecular age, case-finding by screening family members of long QT subjects with electrocardiography was the method of choice.
		Liddle's syndrome is caused by mutations in the carboxyl-terminus of the beta- or gamma-subunits of the renal epithelial sodium channel [101].
	www.medped.org /who/page37.html (546 words)

	Alterations in the long QT syndrome genes KVLQT1 and SCN5A and methods for detecting same (US6342357) (Site not responding. Last check: 2007-10-20)
		Long QT Syndrome (LQTS) is a cardiovascular disorder characterized by prolongation of the QT interval on electrocardiogram and presence of syncope, seizures and sudden death.
		"Long QT Syndrome-associated Mutations in the S4-S5 Linker of KvLQT1 Potassium Channels Modify Gating and Interaction with minK Subunits", J. Biological Chemistry, Jul. 23, 1999; 274(30):21063-21070; J. Biological Chemistry, Aug. 27, 1999; 274(35):25188.
		Vincent, G.M. "The Molecular Genetics of the Long QT Syndrome: Genes Causing Fainting and Sudden Death", Annu.
	www.delphion.com /details?pn=US06342357__ (1461 words)

	Alarm Clocks Deadly For Rare Heart Condition
		In six families with the genetic mutation, five people died unexpectedly in their sleep, including a 22 year old who went into cardiac arrest after an alarm clock went off and a woman who was known to faint at the sound of the doorbell.
		The finding is from a study of 11 families with familial long QT syndrome, a condition characterized by an unusual heart beat and life-threatening heart arrhythmias.
		In a statement, the American College of Cardiology notes that it is important to diagnose long QT syndrome because the potentially fatal disorder can be treated with beta-blocker drugs that reduce the risk of cardiac arrhythmias.
	www.personalmd.com /news/a1999020102.shtml (265 words)

	FAMILION - Cardiac Channelopathies (Site not responding. Last check: 2007-10-20)
		Brugada Syndrome is very similar to Long QT Syndrome, however, the characteristic ECG finding is different.
		In many families, a child is diagnosed with a cardiac channelopathy even though neither parent has ever had symptoms.
		This is a pedigree of a fictional family with LQTS.
	www.familion.com /patients/cardiac_channelopathies.html (1136 words)

	Effects of cardiac sympathetic innervation on regional wall motion abnormality in patients with long QT syndrome -- ...
		with the long QT syndrome was abolished by the injection of a
		Unsuspected echocardiographic abnormality in the long QT syndrome.
		I-metaiodobenzylguanidine scintigraphy in the detection of irregular regional sympathetic innervation in long QT syndrome.
	heart.bmjjournals.com /cgi/content/full/83/3/295 (2973 words)

	GeneDis Congenital Long QT Syndrome Mutation Web Site
		Congenital Long QT Syndrome is a human genetic disease caused due to mutations in the SCN5A, KCNH2, KCNE1 and KCNQ1.
		The long QT syndrome is a familial disease characterized by abnormally prolonged ventricular repolarization and a high risk of malignant ventricular tachyarrhythmias, occurring often, but not always, in the setting of high adrenergic activity, that is, physical or emotional stress (6).
		Defects in SCN5A is the cause of long qt syndrome type 3 (lqt3), an autosomal dominant cardiac disease characterized by recurrent syncope and sudden cardiac death.
	www.tau.ac.il /lifesci/bioinfo/genedis/heart/long_qt.html.old2 (767 words)

	eMedicine - Long QT Syndrome : Article by Wojciech Zareba, MD, PhD, FACC
		Drug-induced QT prolongation also may lead to increased risk of ventricular tachyarrhythmias (eg, torsade de pointes) and sudden cardiac death in a similar ionic mechanism as is observed in congenital LQTS.
		The female predominance may be related to a longer duration of QT corrected for heart rate using the Bazett formula (QTc) in adult females than in males and to a higher mortality rate in young males.
		Secondary (drug-induced) QT prolongation also may have a genetic background, consisting of predisposition of an ion channel to abnormal kinetics caused by gene mutation or polymorphism.
	www.emedicine.com /med/topic1983.htm (4029 words)

	First International Symposium on Long QT Syndrome (Site not responding. Last check: 2007-10-20)
		LQTS is identified by abnormal QT interval prolongation on the ECG.
		The QT prolongation may arise from either a decrease in repolarizing cardiac membrane currents or an increase in depolarizing cardiac currents.
		The association between the magnitude of drug-induced QT prolongation and the risk of malignant ventricular arrhythmias is complex.
	www.lqts-symposium.org (546 words)

	QTsyndrome.ch - Bibliography (Site not responding. Last check: 2007-10-20)
		Keating M, Atkinson D, Dunn C, Timothy K, Vincent GM, Leppert M: Linkage of a cardiac arrhythmia, the long QT syndrome, and the Harvey ras-1 gene.
		Inherited brugada and LQT-3 syndrome mutations of a single residue of the cardiac sodium channel confer distinct channel and clinical phenotypes.
		Vincent, G.M. ; Timothy, K. ; Leppert, M. ; Keating, M.: The spectrum of symptoms and QT intervals in carriers of the gene for the long QT syndrome.
	www.qtsyndrome.ch /biblio.html (681 words)

	Missense Mutation in the Pore Region of HERG Causes Familial Long QT Syndrome -- Benson et al. 93 (10): 1791 -- ... (Site not responding. Last check: 2007-10-20)
		Missense Mutation in the Pore Region of HERG Causes Familial Long QT Syndrome -- Benson et al.
		Missense Mutation in the Pore Region of HERG Causes Familial Long QT Syndrome
		in HERG is the cause of LQT in this family because it segregates
	circ.ahajournals.org /cgi/content/abstract/93/10/1791 (1002 words)

	Prolonged QTc interval as an important factor in sudden infant death syndrome -- WREN 81 (3): 278 -- Archives of ...
		The changes that occur in QT interval with age during infancy are normal and should not be taken as evidence of "tendency
		Risk of cardiac events in family members of patients with long QT syndrome.
		Sudden infant death syndrome: normal QT interval on ECGs of relatives.
	adc.bmjjournals.com /cgi/content/full/81/3/278k (400 words)

	Relation between QT duration and maximal wall thickness in familial hypertrophic cardiomyopathy -- Jouven et al. 88 ...
		QT interval was measured from the onset of the QRS complex to
		Prolonged QT interval in hypertrophic and dilated cardiomyopathy in children.
		QT dispersion and risk factors for sudden cardiac death in patients with hypertrophic cardiomyopathy.
	heart.bmjjournals.com /cgi/content/full/88/2/153 (2682 words)

	Pharmacogenetic Considerations in Diseases of Cardiac Ion Channels -- Anantharam et al. 307 (3): 831 -- Journal of ...
		KCNQ1 and MinK Mutations Cause Inherited Long QT Syndrome and Deafness
		Long QT syndrome-associated mutations in KCNQ1 are dispersed
		(2003) KCNQ1 gain-of-function mutation in familial atrial fibrillation.
	jpet.aspetjournals.org /cgi/content/full/307/3/831 (5097 words)

	Mutation detection in long QT syndrome: a comprehensive set of primers and PCR conditions -- Syrris et al. 38 (10): 705 ...
		Long QT syndrome (LQTS) is an inherited disorder which produces arrhythmia and sudden death.
		Sodium channel abnormalities are infrequent in patients with long QT syndrome: identification of two novel SCN5A mutations.
		Congenital long QT syndrome caused by a novel mutation in a conserved acidic domain of the cardiac Na channel.
	jmg.bmjjournals.com /cgi/content/full/38/10/705 (2890 words)

	long_qt_references
		Wei J, Wang DW, Alings M, Fish F, Wathen M, Roden DM, George AL Jr, Congenital long-QT syndrome caused by a novel mutation in a conserved acidic domain of the cardiac Na+ channel, Circulation 1999 Jun 22;99(24):3165-71.
		Splawski I, Tristani-Firouzi M, Lehmann MH, Sanguinetti MC, Keating MT, Mutations in the hminK gene cause long QT syndrome and suppress IKs function, Nat Genet 1997 Nov;17(3):338-40.
		Saarinen K, Swan H, Kainulainen K, Toivonen L, Viitasalo M, Kontula K, Molecular genetics of the long QT syndrome: two novel mutations of the KVLQT1 gene and phenotypic expression of the mutant gene in a large kindred, Hum Mutat 1998;11(2):158-65.
	www.tau.ac.il /lifesci/bioinfo/genedis/heart/long_qt_references.html (1013 words)

	Sudden Arrhythmia Death Syndromes (SADS) Foundation.
		Cost-effectiveness analysis of genetic testing for familial long QT syndrome in symptomatic index cases.
		Genetic testing in the long QT syndrome: development and validation of an efficient approach to genotyping in clinical practice.
		The Role of Genetic Testing in the Diagnosis and Treatment of Long QT Syndrome
	www.sads.org /Genetics/resources.htm (111 words)

	Cogprints - The Long QT Syndrome (Site not responding. Last check: 2007-10-20)
		The Long QT syndrome (LQTS) can be inherited or acquired and is of particular interest and concern at present.
		Vincent GM, Timothy KW, Leppert M, Keating MT: The spectrum of symptoms and QT Intervals in the carriers of the gene for the long-QT syndrome.
		Kahn JK, Sisson J, Vinik A: QT interval prolongation and sudden cardiac death in diabetic autonomic neuropathy.
	cogprints.org /4302 (1897 words)

	UniProtKB/Swiss-Prot entry Q12809 [KCNH2_HUMAN] Potassium voltage-gated channel subfamily H member 2 (Site not responding. Last check: 2007-10-20)
		Benson D.W. MacRae C.A. Vesely M.R. Walsh E.P. Seidman J.G. Seidman C.E. Satler C.A. "Missense mutation in the pore region of HERG causes familial long QT syndrome.";
		Chen J. Zou A. Splawski I. Keating M.T. Sanguinetti M.C. "Long QT syndrome-associated mutations in the Per-Arnt-Sim (PAS) domain of HERG potassium channels accelerate channel deactivation.";
		Hayashi K. Shimizu M. Ino H. Yamaguchi M. Mabuchi H. Hoshi N. Higashida H. "Characterization of a novel missense mutation E637K in the pore-S6 loop of HERG in a patient with long QT syndrome.";
	www.expasy.org /uniprot/Q12809 (1570 words)

	Genaissance - Research and Development - Long QT Syndrome (Site not responding. Last check: 2007-10-20)
		These conditions are caused by structural abnormalities in the potassium and sodium channels of the heart and predispose affected individuals to an abnormal heart rhythm (dysrrhythmia) that can result in sudden cardiac death.
		They can be inherited (familial) or they can be acquired.
		Genaissance has intellectual property rights relating to the five genes that have been identified as explaining the majority of two cardiac channelopathies, familial Long QT (LQT) Syndrome and Brugada Syndrome, with each of the genes having multiple causative mutations.
	www.genaissance.com /research_development/longqtsyndrome.html (291 words)

	SBF Glossary: LQ to l8r
		An infrequently occurring familial disorder in which affected members have QT prolongation and a propensity to syncope and fatal ventricular arrhythmias.
		The long delay (until 1995) in publishing the diaries has been reported to have something to do with the fact that his book casts a critical eye on language abuse not only by the Nazis but also by the Soviet occupying authority.
		A syndrome in which the follicle develops into the corpus luteum without releasing the egg.
	www.plexoft.com /SBF/L04.html (9485 words)

	Internal Ventricular Defibrillation (Site not responding. Last check: 2007-10-20)
		A 12-year-old boy with the familial long-QT syndrome had an automatic cardioverter-defibrillator implanted after resuscitation from cardiac arrest.
		The device was programmed to wait 7.5 seconds before discharging in response to ventricular tachycardia or ventricular fibrillation.
		Analysis of the cardioverter-defibrillator revealed the following sequence of events: normal sinus rhythm with a corrected QT interval of 0.66 second (top tracing) was followed by torsade de pointes (middle tracing) and ventricular fibrillation (bottom tracing), prompting discharge of the cardioverter-defibrillator (arrow) and a return to sinus rhythm within 600 msec after discharge.
	www.rjmatthewsmd.com /Definitions/pop/62fig.htm (178 words)

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