نتایج جستجو برای: jervell and lange nielsen syndrome

تعداد نتایج: 16945764  

Journal: :Circulation. Arrhythmia and electrophysiology 2012
Mira A Coleman J Martijn Bos Jonathan N Johnson Heidi J Owen Claude Deschamps Christopher Moir Michael J Ackerman

BACKGROUND Treatment options for patients with recurrent ventricular arrhythmias refractory to pharmacotherapy and ablation are minimal. Although left cardiac sympathetic denervation (LCSD) is well established in long-QT syndrome, its role in non-long-QT syndrome arrhythmogenic channelopathies and cardiomyopathies is less clear. Here, we report our single-center experience in performing LCSD in...

1993
U.-J Wiese

Iterating renormalization group transformations for lattice fermions the Wilson action is driven to fixed points of the renormalization group. A line of fixed points is found and the fixed point actions are computed analytically. They are local and may be used to improve scaling in lattice QCD. The action at the line's endpoint is chirally invariant and still has no fermion doubling. The Nielse...

Journal: :American journal of physiology. Regulatory, integrative and comparative physiology 2002
Richard Warth Jacques Barhanin

Mutations of the KCNE1 gene (IsK, minK) are related to hereditary forms of cardiac arrhythmias, so-called long QT syndromes (LQT). Here we review the phenotype of a mouse model for the recessive form of LQT known as Jervell and Lange-Nielsen syndrome. KCNE1 knockout mice exhibit an enhanced QT-RR adaptability, which is probably part of the pathophysiological mechanism leading to life-threatenin...

Journal: :Circulation research 1998
M D Drici I Arrighi C Chouabe J R Mann M Lazdunski G Romey J Barhanin

The Jervell and Lange-Nielsen (JLN) syndrome affects the human cardioauditory system, associating a profound bilateral deafness with an abnormally long QT interval on the ECG. It results from mutations in KVLQT1 and ISK genes that encode the 2 subunits forming the K+ channel responsible for the cardiac and inner ear slowly activating component of the delayed rectifier K+ current (IKs). A JLN mo...

2014
Grace Salsbury Emma L. Cambridge Zoe McIntyre Mark J. Arends Natasha A. Karp Christopher Isherwood Carl Shannon Yvette Hooks Ramiro Ramirez-Solis David J. Adams Jacqueline K. White Anneliese O. Speak

Iron homeostasis is a dynamic process that is tightly controlled to balance iron uptake, storage, and export. Reduction of dietary iron from the ferric to the ferrous form is required for uptake by solute carrier family 11 (proton-coupled divalent metal ion transporters), member 2 (Slc11a2) into the enterocytes. Both processes are proton dependent and have led to the suggestion of the importanc...

1995
C. S. Aulakh

We show that self-dual Nielsen Olesen (NO) vortices in 3 dimensions give rise to a class of exact solutions when coupled to Einstein Maxwell Dilaton gravity obeying the Majumdar-Papapetrou(MP) relation between gravitational and Maxwell couplings , provided certain Chern-Simons type interactions are present. The metric may be solved for explicitly in terms of the NO vortex function and becomes d...

Journal: :Indian pediatrics 2009
I Sathyamurthy K Jayanthi Jyothirmaya Dash K N Srinivasan

The Jervell and Lange Nielson syndrome(JLN) is an infrequent form of long QT syndrome (LQTS) in which prolonged QT interval and congenital deafness exist together. We attempted to identify patients with LQTS among 127 children (age 1.2 to 10 years) with congenital hearing loss. The corrected QT interval was measured from 12 lead electrocardiogram(ECG) , using Bazettes and Friedricia formulae.Th...

Journal: :Journal of Ayub Medical College, Abbottabad : JAMC 2011
Ahsan Niaz Syed Faiz-ul-Hassan Rizvi Daniyeh Khurram

BACKGROUND Long QT syndrome is considered a fatal disease because of its association with ventricular arrhythmias and sudden cardiac death. Objectives of study were to determine the prevalence of long QT syndrome and other heart diseases, in deaf-mute children. METHODS A Cross-sectional descriptive study was conducted at Cholistan special education centre and Cardiology department, Sheikh Zay...

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