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Search on : Lown-Ganong-Levine and Syndrome [Words]
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[PMID]: 23299344
[Au] Autor:Kleineberg N; Nia AM; Gassanov N; Er F
[Ad] Address:Klinik III für Innere Medizin, Herzzentrum der Universität zu Köln.
[Ti] Title:53-jährige Patientin mit paroxysmalen Tachykardien. [53-year-old woman with paroxysmal tachycardia].
[So] Source:Dtsch Med Wochenschr;138(3):89-90, 2013 Jan.
[Is] ISSN:1439-4413
[Cp] Country of publication:Germany
[La] Language:ger
[Mh] MeSH terms primary: Electrocardiography/methods
Lown-Ganong-Levine Syndrome/diagnosis
Tachycardia, Paroxysmal/diagnosis
[Mh] MeSH terms secundary: Diagnosis, Differential
Female
Humans
Middle Aged
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1303
[Cu] Class update date: 130109
[Lr] Last revision date:130109
[Js] Journal subset:IM
[Da] Date of entry for processing:130110
[St] Status:MEDLINE
[do] DOI:10.1055/s-0032-1327209

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[PMID]: 22311718
[Au] Autor:Casini S; Postma AV
[Ti] Title:Decreased inward rectification of Kir2.1 channels is a novel mechanism underlying the short QT syndrome.
[So] Source:Cardiovasc Res;93(4):535-6, 2012 Mar 15.
[Is] ISSN:1755-3245
[Cp] Country of publication:England
[La] Language:eng
[Mh] MeSH terms primary: Lown-Ganong-Levine Syndrome/genetics
Lown-Ganong-Levine Syndrome/physiopathology
Mutation/genetics
Potassium Channels, Inwardly Rectifying/genetics
Potassium Channels, Inwardly Rectifying/physiology
[Mh] MeSH terms secundary: Animals
Female
Humans
[Pt] Publication type:COMMENT; EDITORIAL
[Nm] Name of substance:0 (KCNJ2 protein, human); 0 (Kir2.1 channel); 0 (Potassium Channels, Inwardly Rectifying)
[Em] Entry month:1209
[Cu] Class update date: 120302
[Lr] Last revision date:120302
[Js] Journal subset:IM
[Da] Date of entry for processing:120208
[St] Status:MEDLINE
[do] DOI:10.1093/cvr/cvs084

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[PMID]: 22155372
[Au] Autor:Hattori T; Makiyama T; Akao M; Ehara E; Ohno S; Iguchi M; Nishio Y; Sasaki K; Itoh H; Yokode M; Kita T; Horie M; Kimura T
[Ad] Address:Department of Cardiovascular Medicine, Kyoto University Graduate School of Medicine, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan.
[Ti] Title:A novel gain-of-function KCNJ2 mutation associated with short-QT syndrome impairs inward rectification of Kir2.1 currents.
[So] Source:Cardiovasc Res;93(4):666-73, 2012 Mar 15.
[Is] ISSN:1755-3245
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:AIMS: Short-QT syndrome (SQTS) is a recently recognized disorder associated with atrial fibrillation (AF) and sudden death due to ventricular arrhythmias. Mutations in several ion channel genes have been linked to SQTS; however, the mechanism remains unclear. This study describes a novel heterozygous gain-of-function mutation in the inward rectifier potassium channel gene, KCNJ2, identified in SQTS. METHODS AND RESULTS: We studied an 8-year-old girl with a markedly short-QT interval (QT = 172 ms, QTc = 194 ms) who suffered from paroxysmal AF. Mutational analysis identified a novel heterozygous KCNJ2 mutation, M301K. Functional assays displayed no Kir2.1 currents when M301K channels were expressed alone. However, co-expression of wild-type (WT) with M301K resulted in larger outward currents than the WT at more than -30 mV. These results suggest a gain-of-function type modulation due to decreased inward rectification. Furthermore, we analysed the functional significance of the amino acid charge at M301 (neutral) by changing the residue. As with M301K, in M301R (positive), the homozygous channels were non-functional, whereas the heterozygous channels demonstrated decreased inward rectification. Meanwhile, the currents recorded in M301A (neutral) showed normal inward rectification under both homo- and heterozygous conditions. Heterozygous overexpression of WT and M301K in neonatal rat ventricular myocytes exhibited markedly shorter action potential durations than the WT alone. CONCLUSION: In this study, we identified a novel KCNJ2 gain-of-function mutation, M301K, associated with SQTS. Functional assays revealed no functional currents in the homozygous channels, whereas impaired inward rectification demonstrated under the heterozygous condition resulted in larger outward currents, which is a novel mechanism predisposing SQTS.
[Mh] MeSH terms primary: Lown-Ganong-Levine Syndrome/genetics
Lown-Ganong-Levine Syndrome/physiopathology
Mutation/genetics
Potassium Channels, Inwardly Rectifying/genetics
Potassium Channels, Inwardly Rectifying/physiology
[Mh] MeSH terms secundary: Action Potentials/physiology
Animals
Cells, Cultured
Child
Electrocardiography
Female
Genetic Predisposition to Disease/genetics
HEK293 Cells
Heterozygote
Homozygote
Humans
Models, Animal
Myocytes, Cardiac/cytology
Myocytes, Cardiac/physiology
Patch-Clamp Techniques
Pedigree
Rats
Rats, Wistar
Transfection
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Name of substance:0 (KCNJ2 protein, human); 0 (Kir2.1 channel); 0 (Potassium Channels, Inwardly Rectifying)
[Em] Entry month:1209
[Cu] Class update date: 120302
[Lr] Last revision date:120302
[Js] Journal subset:IM
[Da] Date of entry for processing:111214
[St] Status:MEDLINE
[do] DOI:10.1093/cvr/cvr329

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[PMID]: 21955341
[Au] Autor:McGrath BM; Moore J
[Ad] Address:Department of Medicine, Dalhousie University, Halifax, NS. brent .mcgrath @dal.ca
[Ti] Title:Country cardiograms case 41.
[So] Source:Can J Rural Med;16(4):133, answer pg 141-2, 2011.
[Is] ISSN:1488-237X
[Cp] Country of publication:Canada
[La] Language:eng
[Mh] MeSH terms primary: Electrocardiography
Lown-Ganong-Levine Syndrome/diagnosis
[Mh] MeSH terms secundary: Adolescent
Female
Humans
Tachycardia/etiology
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1201
[Cu] Class update date: 110929
[Lr] Last revision date:110929
[Js] Journal subset:IM
[Da] Date of entry for processing:110930
[St] Status:MEDLINE

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[PMID]: 19859765
[Au] Autor:Shabanian R; Kiani A; Rad EM; Eslamiyeh H
[Ad] Address:Division of Cardiology, Department of Pediatrics, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
[Ti] Title:Lown-Ganong-Levine syndrome in a 3-month-old infant with isolated left ventricular noncompaction.
[So] Source:Pediatr Cardiol;31(2):274-6, 2010 Feb.
[Is] ISSN:1432-1971
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:This report describes a 3-month-old boy with isolated left ventricular noncompaction admitted to a medical facility due to heart failure and dysrhythmia. His electrocardiogram showed a short PR interval and a normal QRS complex after abortion of supraventricular tachycardia in favor of Lown-Ganong-Levine syndrome or enhanced atrioventricular nodal conduction.
[Mh] MeSH terms primary: Isolated Noncompaction of the Ventricular Myocardium
Lown-Ganong-Levine Syndrome
[Mh] MeSH terms secundary: Electrocardiography
Humans
Infant
Isolated Noncompaction of the Ventricular Myocardium/physiopathology
Lown-Ganong-Levine Syndrome/physiopathology
Male
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1005
[Cu] Class update date: 170922
[Lr] Last revision date:170922
[Js] Journal subset:IM
[Da] Date of entry for processing:091028
[St] Status:MEDLINE
[do] DOI:10.1007/s00246-009-9564-5

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[PMID]: 19218660
[Au] Autor:Krenke R; Nasilowski J; Przybylowski T; Chazan R
[Ad] Address:Department of Internal Medicine, Pneumology and Allergology, Warsaw Medical University, Warsaw, Poland. rafalkrenke@interia.pl
[Ti] Title:Electrocardiographic changes in patients with spontaneous pneumothorax.
[So] Source:J Physiol Pharmacol;59 Suppl 6:361-73, 2008 Dec.
[Is] ISSN:1899-1505
[Cp] Country of publication:Poland
[La] Language:eng
[Ab] Abstract:The aim of the study was to evaluate the prevalence of electrocardiography (ECG) abnormalities in subjects with spontaneous pneumothorax. Forty consecutive patients (mean age 43.7 +/-19.1 years) with spontaneous pneumothorax participated in the study. There were 22 cases of left-sided and 18 cases of right-sided pneumothorax. The mean relative volume of pneumothorax was 51.4 +/-24.7% according to the Light's index and 53.5 +/-22.9% according to the Rhea method. Heart rate was significantly higher in patients with pneumothorax than after lung re-expansion (91 +/-20 bpm vs. 72 +/-16 bpm; P<0.001). Abnormal left axis deviation was found in 3 patients with left-sided and in 1 with right-sided pneumothorax, while abnormal right axis deviation was found in 2 patients with left-sided pneumothorax. Relevant QRS abnormalities (incomplete RBBB and T-wave inversion) were found in 4 patients (10%). QRS amplitude in V2-V6 leads was significantly decreased in left-sided pneumothorax, while the right-sided pneumothorax was associated with an increase in QRS amplitude in V5-V6. We conclude that ECG in subjects with pneumothorax often reveals significant abnormalities. The most significant abnormalities were seen in patients with massive right-sided pneumothorax.
[Mh] MeSH terms primary: Electrocardiography
Pneumothorax/physiopathology
[Mh] MeSH terms secundary: Adolescent
Adult
Aged
Aged, 80 and over
Arrhythmias, Cardiac/etiology
Arrhythmias, Cardiac/physiopathology
Female
Humans
Hypoxia/etiology
Hypoxia/physiopathology
Lown-Ganong-Levine Syndrome/etiology
Lown-Ganong-Levine Syndrome/physiopathology
Male
Middle Aged
Pneumothorax/pathology
Respiratory Mechanics/physiology
Young Adult
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:0904
[Cu] Class update date: 161124
[Lr] Last revision date:161124
[Js] Journal subset:IM
[Da] Date of entry for processing:090217
[St] Status:MEDLINE

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[PMID]: 15654149
[Au] Autor:Dreifus LS; Pollak SJ
[Ad] Address:Department of Cardiovascular Disease, Florida Hospital, Orlando, FL,USA. ldmd@dreifus.com
[Ti] Title:Ablation therapy of supraventricular tachycardia in elderly persons.
[So] Source:Am J Geriatr Cardiol;14(1):20-5, 2005 Jan-Feb.
[Is] ISSN:1076-7460
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Ablation of supraventricular tachycardia in elderly persons presents a challenging problem to electrophysiologists. Friable cardiac structures, prone to catheter perforation, comorbid cardiovascular disease, and the propensity to develop atrial fibrillation and thromboembolic complications, place these patients at high risk. Newer techniques for cardiac mapping and ablation, the establishment of precise ablation lines, and safer approaches to the ablation mechanism (particularly for atrial fibrillation) are presented in this review. In addition, a novel rapid ablation method to permanently cure atrial ventricular nodal re-entry tachycardia is demonstrated.
[Mh] MeSH terms primary: Catheter Ablation
Tachycardia, Atrioventricular Nodal Reentry/surgery
Tachycardia, Supraventricular/surgery
[Mh] MeSH terms secundary: Electrocardiography
Humans
Lown-Ganong-Levine Syndrome/surgery
Wolff-Parkinson-White Syndrome/surgery
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:0505
[Cu] Class update date: 050117
[Lr] Last revision date:050117
[Js] Journal subset:IM
[Da] Date of entry for processing:050118
[St] Status:MEDLINE

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[PMID]: 14662232
[Au] Autor:Eichholz A; Whiting RB; Artal R
[Ad] Address:Department of Obstetrics and Gynecology, St. Mary's Health Center, Saint Louis University School of Medicine, St. Louis, Missouri 63117, USA.
[Ti] Title:Lown-Ganong-Levine syndrome in pregnancy.
[So] Source:Obstet Gynecol;102(6):1393-5, 2003 Dec.
[Is] ISSN:0029-7844
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Lown-Ganong-Levine syndrome is characterized by paroxysmal supraventricular tachycardia, a short PR interval, and normal QRS. CASE: A gravida 3, para 2 was diagnosed with Lown-Ganong-Levine syndrome at 16 weeks' gestation after an episode of chest pain and shortness of breath. She was hospitalized and treated with digoxin, and her symptoms subsided. The remainder of her pregnancy was uneventful. CONCLUSION: Lown-Ganong-Levine Syndrome is a rare complication most commonly seen in young to middle-aged women. This syndrome can be treated and controlled medically with a good outcome.
[Mh] MeSH terms primary: Lown-Ganong-Levine Syndrome
Pregnancy Complications, Cardiovascular
[Mh] MeSH terms secundary: Female
Humans
Lown-Ganong-Levine Syndrome/diagnosis
Lown-Ganong-Levine Syndrome/drug therapy
Pregnancy
Pregnancy Complications, Cardiovascular/diagnosis
Pregnancy Complications, Cardiovascular/drug therapy
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Em] Entry month:0401
[Cu] Class update date: 091026
[Lr] Last revision date:091026
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:031210
[St] Status:MEDLINE

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[PMID]: 12685250
[Au] Autor:Undas A; Rys D; Wegrzyn W; Musial J
[Ad] Address:II Katedra Chorób Wewnetrznych, Collegium Medicum UJ Kraków.
[Ti] Title:Nietypowe objawy choroby Fabry'ego--nagla obustronna utrata sluchu, obrzek limfatyczny i zespól Lowna, Ganonga i Levine'a. [Atypical symptoms of Fabry's disease: sudden bilateral deafness, lymphoedema and Lown-Ganong-Levine syndrome].
[So] Source:Pol Arch Med Wewn;108(5):1085-90, 2002 Nov.
[Is] ISSN:1897-9483
[Cp] Country of publication:Poland
[La] Language:pol
[Ab] Abstract:A 40-year-old man with Fabry disease, confirmed by decreased leukocyte alpha-galactosidase A activity in 2001, complained of sudden bilateral deafness, as evidenced by clinical history and audiometry. Magnetic resonance of the brain revealed features typical of Fabry disease. Other clinical manifestations of the disease included: angiokeratoma, mild proteinuria with normal renal function, lymphoedema of the lower limbs, pre-excitation syndrome, myocardial hypertrophy.
[Mh] MeSH terms primary: Fabry Disease/diagnosis
Hearing Loss, Bilateral/complications
Hearing Loss, Sudden/complications
Lown-Ganong-Levine Syndrome/complications
Lymphedema/complications
[Mh] MeSH terms secundary: Adult
Brain/pathology
Diagnosis, Differential
Electrocardiography
Hearing Loss, Bilateral/diagnosis
Hearing Loss, Sudden/diagnosis
Humans
Lower Extremity
Lown-Ganong-Levine Syndrome/diagnosis
Lymphedema/diagnosis
Magnetic Resonance Imaging
[Pt] Publication type:CASE REPORTS; ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:0305
[Cu] Class update date: 170306
[Lr] Last revision date:170306
[Js] Journal subset:IM
[Da] Date of entry for processing:030411
[St] Status:MEDLINE

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[PMID]: 11279385
[Au] Autor:Tamburrini LR; Fontanelli A; Primossi G
[Ad] Address:Istituto di Clinica Medica Generale e Terapia Medica, Università degli Studi, Trieste, Italy.
[Ti] Title:La pre-eccitazione ventricolare: elettrofisiopatologia, criteri interpretativi e inquadramento clinico. Riferimenti di Geriatria. [Ventricular pre-excitation: electrophysiopathology, criteria for interpretation and clinical diagnosis. References for geriatrics].
[So] Source:Minerva Cardioangiol;49(1):47-73, 2001 Feb.
[Is] ISSN:0026-4725
[Cp] Country of publication:Italy
[La] Language:ita
[Ab] Abstract:The authors review the state-of-the-art on ventricular pre-excitation in medical and arrhythmological literature in order to facilitate the recognition of the various clinical forms, like classic and occult Wolff Parkinson withe syndrome and Lown Ganong Levine syndrome. A historical introduction reviews our electrophysiopathological knowledge of the electrical activation and conduction of ventricular pre-excitation compared to normal, starting from the anatomic discovery of conduction pathways to the possible use of transesophageal electrostimulation and endocavity mapping to study electric potentials. Avantgarde technologies have also been developed to eliminate anomalous pathways firstly by using a direct current dirscharge and secondly radiofrequency. Atrioventricular electric activation has been widely illustrated in normal subjects in order to create a model for comparison with pathological ventricular pre-excitation: the upper left portion of the septum is no longer the first zone to trigger the kinetic mechanism compared to the early fascicular fraying of the homonymous branch. Instead the upper right part of the septum is activated earlier owing to the anomalous fascia connected on this side to the right branch through their septal arborisations. As a result, this new conduction pathway activates the ventricular masses earlier through an anomalous route, given that there is no further contact with the atrioventricular nodes which act as a control. A similar situation is found in the left branch block where the ventriculogram is late with a normal PR, unlike pre-excitation when an early delta wave is present with a short PR. Electric conduction is also illustrated based on new knowledge of the circuit structures and the rings theory. Orthodromic tachycardia is distinguished from the antidromic form, double accessory pathway tachycardia, ectopic reciprocant atrial fibrillation tachycardia and occult bundle tachycardia which is studied using transesophageal stimulation with a time threshold of 70 ms for ventricular-atrial retrograde activation. The stimulation techniques using single or repeated extrastimulus are explained for this purpose, as well as those with serial extrastimulation and the physical characteristics of the circuit based on the ratio between voltage and resistance. The authors also report the practical aims of electrostimulation for determining the electric threshold of the anomalous circuit in terms of refractoriness, electric atrial stability, reciprocity and the occurrence of the macro re-entry. Lastly, the authors describe electric conduction by anomalous pathways based on the criterion of conduction and activation, both of which are analysed and compared on the basis of the intrinsicoid deflection morphology on the surface ECG: the aberrant qRs usually suggests an antidromic ventricular activation and retrograde conduction between atrium and ventricle, while normal intrinsicoid deflection demonstrates that the activation is orthodromic and the conduction anterograde, namely ventricle-atrial. Having been reproduced in a synoptic synthesis, these manifestations show a regular electrophysiological pattern because they are dissimilar from the behaviour of the monophasic bioelectric potential of the cardiac cells specialised in the conduction of the stimulus, whether they represent a normal or pathological electric pathway. The study is rounded off by the analysis of the reciprocant tachycardias and their re-entry varieties related to the type of the anomalous bundles. A number of types of re-entry can be identified: sinusal re-entry (micro re-entry), atrial re-entry, re-entry in the atrio-ventricular node, re-entry tachycardia and the so-called triggered type. The discussion of the electrophysiopathological aspects of pre-excitation is followed by the clinical forms of ventricular pre-excitation that can be divided into 3 main types. The different ECG clinical pictures are set out in the summary table, together with the type of shunt and activation and possible variants, following Rosenbaum s classic paint: the common type B, the rare type A and a last variant, the C type. This section also describes the positional peculiarities of the Kent-Paladino bundle, the left ventricular, septal (anterior and posterior) and the multiple-bundle ones. The authors also illustrate the criterion and meaning of endocavity mapping in the search for anomalous bioelectric potentials that identify the pathway or the location of the endocardiac bundle and/or foci to be eliminated. A new echocardiographic technique is described with a conventional M mode, digitalised 2D and tissular Doppler which has a comparable ability to identify the anomalous pathways of electric conduction using a non-invasive method. (ABSTRACT TRUNCATED)
[Mh] MeSH terms primary: Pre-Excitation Syndromes
[Mh] MeSH terms secundary: Age Factors
Aged
Atrial Fibrillation/physiopathology
Atrial Fibrillation/surgery
Atrial Flutter/physiopathology
Atrial Flutter/surgery
Cardiac Complexes, Premature/physiopathology
Cardiac Complexes, Premature/surgery
Catheter Ablation
Electrocardiography
Electrophysiology
Humans
Lown-Ganong-Levine Syndrome/physiopathology
Lown-Ganong-Levine Syndrome/surgery
Models, Cardiovascular
Pre-Excitation Syndromes/physiopathology
Pre-Excitation Syndromes/surgery
Tachycardia/physiopathology
Tachycardia/surgery
Wolff-Parkinson-White Syndrome/physiopathology
Wolff-Parkinson-White Syndrome/surgery
[Pt] Publication type:COMPARATIVE STUDY; ENGLISH ABSTRACT; JOURNAL ARTICLE; REVIEW
[Em] Entry month:0106
[Cu] Class update date: 061115
[Lr] Last revision date:061115
[Js] Journal subset:IM
[Da] Date of entry for processing:010330
[St] Status:MEDLINE


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