نتایج جستجو برای: sayre

تعداد نتایج: 631  

2016
Mayela Leal Chetan Dhoble Julie Lee Deinymar Lopez Laura Simonne Menéndez

Kearns-Sayre syndrome (KSS) was first described in 1958 as 'a rare neuromuscular disorder defined by a characteristic triad of progressive external ophthalmoplegia, pigmentary retinopathy, atrioventricular block and cerebellar ataxia'. The prevalence rate of KSS is ∼1-3 per 100 000 individuals. Here, we report a rare case of a 17-year-old Venezuelan male with KSS.

2016
Gwenola Tosser-Klopp Philippe Bardou Olivier Bouchez Cédric Cabau Richard Crooijmans Yang Dong Cécile Donnadieu-Tonon André Eggen Henri C. M. Heuven Saadiah Jamli Abdullah Johari Jiken Christophe Klopp Cynthia T. Lawley John McEwan Patrice Martin Carole R. Moreno Philippe Mulsant Ibouniyamine Nabihoudine Eric Pailhoux Isabelle Palhière Rachel Rupp Julien Sarry Brian L. Sayre Aurélie Tircazes Jun Wang Wen Wang Wenguang Zhang

Gwenola Tosser-Klopp, Philippe Bardou, Olivier Bouchez, Cédric Cabau, Richard Crooijmans, Yang Dong, Cécile Donnadieu-Tonon, André Eggen, Henri C. M. Heuven, Saadiah Jamli, Abdullah Johari Jiken, Christophe Klopp, Cynthia T. Lawley, John McEwan, Patrice Martin, Carole R. Moreno, Philippe Mulsant, Ibouniyamine Nabihoudine, Eric Pailhoux, Isabelle Palhière, Rachel Rupp, Julien Sarry, Brian L. Say...

Journal: :Revista espanola de cardiologia 2007
Carolina Hernández-Luis Emilio García-Morán Jerónimo Rubio-Sanz Francisco Fernández-Avilés

Kearns-Sayre syndrome is a rare disease linked to mitochondrial inheritance. The characteristic diagnostic triad consists of progressive external ophthalmoplegia, pigmentary degeneration of the retina, and atrioventricular block.1 It may also be associated with mental retardation, ataxia, deafness, muscle weakness, and endocrine disorders, such as diabetes mellitus or hypothyroidism. We present...

Journal: :Indian Pacing and Electrophysiology Journal 2017

Journal: :JIMD reports 2012
Ingrid van Beynum Eva Morava Marjan Taher Richard J Rodenburg Judit Karteszi Kalman Toth Eszter Szabados

The prognosis of progressive ophthalmoplegia in patients with large-scale mitochondrial DNA deletions is highly variable and almost unpredictable. The risk to develop cardiac involvement and sudden cardiac death is strikingly high, especially in patients with Kearns-Sayre syndrome (KSS). The most typical cardiac complications of the disease are conduction defects, which usually begin with left ...

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