Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis
| العنوان: | Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis |
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| المؤلفون: | Fayssoil, Abdallah, Ben Yaou, Rabah, Ogna, Adam, Chaffaut, Cendrine, Leturcq, France, Nardi, Olivier, Wahbi, Karim, Duboc, Denis, Lofaso, Frederic, Prigent, Helene, Clair, Bernard, Crenn, Pascal, Nicolas, Guillaume, Laforet, Pascal, Behin, Anthony, Chevret, Sylvie, Orlikowski, David, Annane, Djillali |
| المصدر: | PLoS ONE, Vol 13, Iss 1, p e0190518 (2018) PLoS ONE |
| بيانات النشر: | Public Library of Science (PLoS), 2018. |
| سنة النشر: | 2018 |
| مصطلحات موضوعية: | Adult, Male, Heredity, Genetic Linkage, Bundle-Branch Block, Cardiology, lcsh:Medicine, Duchenne Muscular Dystrophy, Biochemistry, Muscular Dystrophies, Dystrophin, Young Adult, Protein Domains, Diagnostic Medicine, Medicine and Health Sciences, Genetics, Prevalence, Point Mutation, Humans, Genetic Predisposition to Disease, lcsh:Science, Retrospective Studies, Heart Failure, Clinical Genetics, lcsh:R, Biology and Life Sciences, Proteins, Prognosis, Muscular Dystrophy, Duchenne, Cytoskeletal Proteins, Deletion Mutation, Neurology, X-Linked Traits, Sex Linkage, Mutation, Female, lcsh:Q, Research Article |
| الوصف: | Background Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. We designed this study to determine the prevalence of left bundle branch block (LBBB), whether there is a relationship between LBBB and genetic pattern, and to assess predictive factors for acute cardiac events and mortality in adult DMD patients. Methods We reviewed the charts of DMD followed at the Home Mechanical Ventilation Unit of the Raymond Poincare University Hospital. Results A total of 121 patients, aged from 18 to 41 years have been included in our study. Median vital capacity (VC) was 12% [7; 19.5] of predicted. Almost all patients were on home mechanical ventilation (95%). LBBB was present in 15 patients (13%); among them, 10 disclosed exonic deletions. After a median follow up of 6 years, 21 patients (17%) experienced acute heart failure (AHF), 7 patients (6%) supraventricular arrhythmia, 3 patients (2.4%) ventricular tachycardia, 4 patients (3%) significant electrical disturbances. LBBB was significantly associated with cardiac events (OR = 12.7; 95%CI [3.78–42.7]; p |
| اللغة: | English |
| تدمد: | 1932-6203 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::c4f2b64938eddc2299d05103cd6967d9Test http://europepmc.org/articles/PMC5755816?pdf=renderTest |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.pmid.dedup....c4f2b64938eddc2299d05103cd6967d9 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 19326203 |
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