ID | 115572 |
Author |
Adachi, Katsuhito
National Hospital Organization Tokushima Hospital
Hashiguchi, Shuji
National Hospital Organization Tokushima Hospital
Saito, Miho
National Hospital Organization Tokushima Hospital
Kashiwagi, Setsuko
National Hospital Organization Tokushima Hospital
Miyazaki, Tatsushi
National Hospital Organization Tokushima Hospital
Kawai, Hisaomi
Hiwasa Hospital
Yamada, Hirotsugu
Tokushima University
Tokushima University Educator and Researcher Directory
KAKEN Search Researchers
Akaike, Masashi
Tokushima University
Tokushima University Educator and Researcher Directory
KAKEN Search Researchers
Takao, Shoichiro
Tokushima University
Tokushima University Educator and Researcher Directory
KAKEN Search Researchers
Kobayashi, Michio
National Hospital Organization Akita Hospital
Ishizaki, Masatoshi
National Hospital Organization Kumamoto-Minai Hospital
Matsumura, Tuyoshi
National Hospital Organization Toneyama Hospital
Mori-Yoshimura, Madoka
National Center of Neurology and Psychiatry
Kimura, En
National Center of Neurology and Psychiatry
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Keywords | Female dystrophinopathy carrier
Cardiomyopathy
Cardiac magnetic resonance imaging
Late gadolinium enhancement
Speckle tracking echocardiography
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Content Type |
Journal Article
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Description | Regular health checkups for mothers of patients with Duchenne muscular dystrophy have been performed at National Hospital Organization Tokushima Hospital since 1994. Among 43 mothers participated in this study, 28 dystrophinopathy carriers were identified. Skeletal and cardiac muscle functions of these subjects were examined. High serum creatine kinase was found in 23 subjects (82.1%). Obvious muscle weakness was present in 5 (17.8%) and had progressed from 1994 to 2015. Cardiomyopathy was observed in 15 subjects (60.0%), including dilated cardiomyopathy-like damage that was more common in the left ventricular (LV) posterior wall. Late gadolinium enhancement on cardiac MRI was found in 5 of 6 subjects, suggesting fibrotic cardiac muscle. In speckle tracking echocardiography performed seven years later, global longitudinal strain was decreased in these subjects, indicating LV myocardial contractile abnormality. These results suggest that female dystrophinopathy carriers should receive regular checkups for detection and treatment of cardiomyopathy, even if they have no cardiac symptoms.
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Journal Title |
Journal of the Neurological Sciences
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ISSN | 0022510X
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NCID | AA00703265
AA11534024
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Publisher | Elsevier
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Volume | 386
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Start Page | 74
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End Page | 80
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Published Date | 2017-12-21
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Rights | This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
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EDB ID | |
DOI (Published Version) | |
URL ( Publisher's Version ) | |
FullText File | |
language |
eng
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TextVersion |
Publisher
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departments |
University Hospital
Medical Sciences
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