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一名年轻患者中神经肌肉疾病相关性心肌病的快速进展。

Rapid progression of neuromuscular disorder related cardiomyopathy in a young patient.

作者信息

Hayashi Mayuko, Tanaka Hidekazu, Yamamoto Tetsushi, Lee Tomoko, Awano Hiroyuki, Yagi Mariko, Imanishi Takamitsu, Hayashi Nobuhide, Takeshima Yasuhiro, Kawai Hiroya, Hirata Ken-ichi, Kawano Seiji

机构信息

Department of Clinical Laboratory, Kobe University Hospital, Japan.

出版信息

Intern Med. 2013;52(24):2771-5. doi: 10.2169/internalmedicine.52.1000.

Abstract

An 11-year and 3-month-old boy with a neuromuscular disorder was admitted for dyspnea. Echocardiography revealed severe left ventricular dysfunction with an ejection fraction (EF) of 17%. However, the EF had been 57% when the patient was 10 years and 9 months old. The patient's clinical condition became refractory, and he died on the 155th day of hospitalization. Speckle-tracking analysis was retrospectively performed, which demonstrated that the global radial strain was within the normal range; however, the global longitudinal and circumferential strains were lower -than -normal 10 years and 9 months of age. Adult neuromuscular disorder-related secondary cardiomyopathy generally progresses slowly, although progression depends on the age of onset of cardiomyopathy.

摘要

一名11岁3个月大的患有神经肌肉疾病的男孩因呼吸困难入院。超声心动图显示严重的左心室功能障碍,射血分数(EF)为17%。然而,该患者10岁9个月大时EF为57%。患者的临床状况变得难以治疗,于住院第155天死亡。回顾性地进行了斑点追踪分析,结果显示整体径向应变在正常范围内;然而,10岁9个月大时整体纵向和圆周应变低于正常水平。成人神经肌肉疾病相关的继发性心肌病通常进展缓慢,尽管进展情况取决于心肌病的发病年龄。

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