Duchenne型肌营养不良心脏损害研究进展

Duchenlie型肌营养不良是儿童致死性肌萎缩疾病，病情进展迅速，如无系统治疗，患儿多于20岁前死于呼吸循环衰竭。Duchenile型肌营养不良对心脏的不良影响日益凸显，推测与dystrophin基因特定外显子缺失密切相关。本文拟对Duchenne型肌营养不良心脏损害的流行病学、与dystrophin基因关系、发生机制、临床表现和病理特征，以及早期预防与治疗进行综述。...

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Bibliographic Details
Published in中国现代神经疾病杂志 Vol. 13; no. 5; pp. 453 - 456
Main Author 张瑶 汤颖 张成
Format Journal Article
LanguageChinese
Published 150001,哈尔滨医科大学附属第一医院神经内科%中山大学附属第一医院神经科,广州,510080 2013
Subjects
心脏病
杜氏
综述
肌营养不良
肌营养不良蛋白
综述
肌营养不良,杜氏
肌营养不良蛋白
心脏病
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ISSN1672-6731
DOI10.3969/j.issn.1672-6731.2013.05.018

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Summary:Duchenlie型肌营养不良是儿童致死性肌萎缩疾病，病情进展迅速，如无系统治疗，患儿多于20岁前死于呼吸循环衰竭。Duchenile型肌营养不良对心脏的不良影响日益凸显，推测与dystrophin基因特定外显子缺失密切相关。本文拟对Duchenne型肌营养不良心脏损害的流行病学、与dystrophin基因关系、发生机制、临床表现和病理特征，以及早期预防与治疗进行综述。
Bibliography:Duchenne muscular dystrophy （DMD） is a fatal muscular disease with rapid progression in children. Most patients die of respiratory and circulatory failure before the age of 20 if there is no systematic treatment. Now the heart problem in this disease has become increasingly prominent, and is thought to be closely associated with certain dystrophin exon deletion. We would like to review the epidemiology, relevance of dystrophin, pathogenesis, clinical manifestations and pathological features, as well as early prevention and treatment of DMD.
Muscular dystrophy, Duchenne; Dystrophin; Heart diseases; Review
ZHANG Yao, TANG Ying, ZHANG Cheng（ 1 Department of Neurology, the First Affiliated Hospital, Harbin Medical University, Harbin 150001, Heilongjiang, China 2Department of Neurology, the First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, Guangdong, China）
12-1363/R
ISSN:1672-6731
DOI:10.3969/j.issn.1672-6731.2013.05.018