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一名表现为房室传导阻滞的年轻患者被诊断为强直性肌营养不良。

A Young Patient Presenting with Atrioventricular Block Diagnosed as Myotonic Dystrophy.

作者信息

Onoda Hiroshi, Imamura Teruhiko, Ushijima Ryuichi, Sobajima Mitsuo, Kinugawa Koichiro

机构信息

The Second Department of Internal Medicine, University of Toyama, Japan.

Division of Cardiology, Niigata Prefectural Central Hospital, Japan.

出版信息

Intern Med. 2020 Jun 15;59(12):1531-1533. doi: 10.2169/internalmedicine.4259-19. Epub 2020 Mar 19.

DOI:10.2169/internalmedicine.4259-19
PMID:32188809
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7364259/
Abstract

We encountered a 42-year-old woman with a history of diabetes mellitus and cataracts presenting with repeated syncope whose electrocardiogram showed advanced atrioventricular block. On admission, we excluded major potential differential diagnoses as causes of an atrioventricular block but did not suspect myotonic dystrophy, which was eventually diagnosed by chance based on a suspected weakness of the respiratory muscles followed by a detailed neurological physical examination. Myotonic dystrophy should be suspected as a differential diagnosis when relatively young patients present with conductance disorder.

摘要

我们遇到一名42岁女性,有糖尿病和白内障病史,反复出现晕厥,其心电图显示高度房室传导阻滞。入院时,我们排除了作为房室传导阻滞病因的主要潜在鉴别诊断,但未怀疑强直性肌营养不良,最终因怀疑呼吸肌无力随后进行详细的神经体格检查偶然确诊。当相对年轻的患者出现传导障碍时,应怀疑强直性肌营养不良作为鉴别诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36b5/7364259/f07e831297c9/1349-7235-59-1531-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36b5/7364259/bdbb4c3d4060/1349-7235-59-1531-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36b5/7364259/f393b569cff9/1349-7235-59-1531-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36b5/7364259/f07e831297c9/1349-7235-59-1531-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36b5/7364259/bdbb4c3d4060/1349-7235-59-1531-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36b5/7364259/f393b569cff9/1349-7235-59-1531-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36b5/7364259/f07e831297c9/1349-7235-59-1531-g003.jpg

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本文引用的文献

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Incidence and predictors of sudden death, major conduction defects and sustained ventricular tachyarrhythmias in 1388 patients with myotonic dystrophy type 1.1388 例 1 型肌强直性营养不良患者的猝死、主要传导缺陷和持续性室性心动过速/颤动的发生率和预测因素。
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Myotonic dystrophy and the heart.强直性肌营养不良症与心脏
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Long-term follow-up of arrhythmias in patients with myotonic dystrophy treated by pacing: a multicenter diagnostic pacemaker study.起搏治疗强直性肌营养不良患者心律失常的长期随访:一项多中心诊断性起搏器研究
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