Literature DB >> 19027585

High incidence of electrocardiogram abnormalities in young patients with duchenne muscular dystrophy.

Yuichi Takami1, Yasuhiro Takeshima, Hiroyuki Awano, Yoh Okizuka, Mariko Yagi, Masafumi Matsuo.   

Abstract

Electrocardiogram abnormalities are reported to be complicated in Duchenne muscular dystrophy. Although Duchenne muscular dystrophy can be genetically diagnosed in young patients, extensive electrocardiogram studies have not been reported. Here, electrocardiogram abnormalities were examined in Duchenne muscular dystrophy cases with dystrophin gene mutations. Sixty-nine patients, aged </=18 years, received 136 electrocardiogram examinations. Sixty-four patients (91.3%) displayed one or more abnormalities. Furthermore, patients adolescent <10 years (84.8% of patients) displayed electrocardiogram abnormalities, and the most common abnormality was deep Q-waves. Remarkably, the abnormality incidence of both deep Q-waves and low RV5 + SV1 (R-wave V5 + S-wave V1) were significantly high in adolescent patients. Although the patterns or positions of dystrophin gene mutations were compared with electrocardiogram abnormalities, no predisposing mutation was disclosed. These results indicate that electrocardiogram abnormalities in Duchenne muscular dystrophy are a result of dystrophin deficiency, regardless of types of gene mutations. The disease can be divided into two types: age-dependent and age-independent. Deep Q-waves and low RV5 + SV1 are proposed as markers of age-dependent cardiac complications.

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Year:  2008        PMID: 19027585     DOI: 10.1016/j.pediatrneurol.2008.08.006

Source DB:  PubMed          Journal:  Pediatr Neurol        ISSN: 0887-8994            Impact factor:   3.372


  15 in total

1.  Age-matched comparison reveals early electrocardiography and echocardiography changes in dystrophin-deficient dogs.

Authors:  Deborah M Fine; Jin-Hong Shin; Yongping Yue; Dietrich Volkmann; Stacey B Leach; Bruce F Smith; Mark McIntosh; Dongsheng Duan
Journal:  Neuromuscul Disord       Date:  2011-05-13       Impact factor: 4.296

2.  Regulation of the calpain and ubiquitin-proteasome systems in a canine model of muscular dystrophy.

Authors:  Kristine M Wadosky; Luge Li; Jessica E Rodríguez; Jin-Na Min; Dan Bogan; Jason Gonzalez; Cam Patterson; Joe N Kornegay; Monte Willis
Journal:  Muscle Nerve       Date:  2011-08-08       Impact factor: 3.217

3.  Leaky RyR2 trigger ventricular arrhythmias in Duchenne muscular dystrophy.

Authors:  Jérémy Fauconnier; Jérôme Thireau; Steven Reiken; Cécile Cassan; Sylvain Richard; Stefan Matecki; Andrew R Marks; Alain Lacampagne
Journal:  Proc Natl Acad Sci U S A       Date:  2010-01-04       Impact factor: 11.205

4.  Low human dystrophin levels prevent cardiac electrophysiological and structural remodelling in a Duchenne mouse model.

Authors:  Gerard A Marchal; Maaike van Putten; Arie O Verkerk; Simona Casini; Kayleigh Putker; Shirley C M van Amersfoorth; Annemieke Aartsma-Rus; Elisabeth M Lodder; Carol Ann Remme
Journal:  Sci Rep       Date:  2021-05-07       Impact factor: 4.379

5.  A Five-Repeat Micro-Dystrophin Gene Ameliorated Dystrophic Phenotype in the Severe DBA/2J-mdx Model of Duchenne Muscular Dystrophy.

Authors:  Chady H Hakim; Nalinda B Wasala; Xiufang Pan; Kasun Kodippili; Yongping Yue; Keqing Zhang; Gang Yao; Brittney Haffner; Sean X Duan; Julian Ramos; Joel S Schneider; N Nora Yang; Jeffrey S Chamberlain; Dongsheng Duan
Journal:  Mol Ther Methods Clin Dev       Date:  2017-07-27       Impact factor: 6.698

6.  The evolution of electrocardiographic changes in patients with Duchenne muscular dystrophies.

Authors:  Woo Hyun Yoo; Min-Jung Cho; Peter Chun; Kwang Hun Kim; Je Sang Lee; Yong Beom Shin
Journal:  Korean J Pediatr       Date:  2017-06-22

Review 7.  Cardiac Involvement Classification and Therapeutic Management in Patients with Duchenne Muscular Dystrophy.

Authors:  Abdallah Fayssoil; Soumeth Abasse; Katy Silverston
Journal:  J Neuromuscul Dis       Date:  2017

8.  Intronic Alternative Polyadenylation in the Middle of the DMD Gene Produces Half-Size N-Terminal Dystrophin with a Potential Implication of ECG Abnormalities of DMD Patients.

Authors:  Abdul Qawee Mahyoob Rani; Tetsushi Yamamoto; Tatsuya Kawaguchi; Kazuhiro Maeta; Hiroyuki Awano; Hisahide Nishio; Masafumi Matsuo
Journal:  Int J Mol Sci       Date:  2020-05-18       Impact factor: 5.923

Review 9.  Advances in Stem Cell Modeling of Dystrophin-Associated Disease: Implications for the Wider World of Dilated Cardiomyopathy.

Authors:  Josè Manuel Pioner; Alessandra Fornaro; Raffaele Coppini; Nicole Ceschia; Leonardo Sacconi; Maria Alice Donati; Silvia Favilli; Corrado Poggesi; Iacopo Olivotto; Cecilia Ferrantini
Journal:  Front Physiol       Date:  2020-05-12       Impact factor: 4.566

10.  Cardiac Dysfunction in Duchenne Muscular Dystrophy Is Less Frequent in Patients With Mutations in the Dystrophin Dp116 Coding Region Than in Other Regions.

Authors:  Tetsushi Yamamoto; Hiroyuki Awano; Zhujun Zhang; Mio Sakuma; Shoko Kitaaki; Masaaki Matsumoto; Masashi Nagai; Itsuko Sato; Takamitsu Imanishi; Nobuhide Hayashi; Masafumi Matsuo; Kazumoto Iijima; Jun Saegusa
Journal:  Circ Genom Precis Med       Date:  2018-01
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