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强直性肌营养不良中的扩张型心肌病和内脏异常

[Dilated cardiomyopathy and visceral anomalies in myotonic dystrophy].

作者信息

Pentimone F, Del Corso L, Vannini A, Mori L, Moruzzo D

机构信息

Istituto di Clinica Medica II, Università di Pisa, Insegnamento di Geriatria.

出版信息

Minerva Cardioangiol. 1990 May;38(5):231-4.

PMID:2234457
Abstract

In dystrophia myotonica clinical evidence of cardiac involvement usually appears several years after the onset of neuromuscular symptoms. In more than 90% of cases there is damage to the specialized cardiac tissues and in about 7% of cases there are alterations to the myocardium. We report a case characterized by early and spread deterioration of the pump function developing into refractory congestive heart failure. The contemporary involvement of the smooth muscle of gallbladder and colon confirms the hypothesis that dystrophia myotonica is a pan-muscle disease.

摘要

在强直性肌营养不良症中,心脏受累的临床证据通常在神经肌肉症状出现数年之后才出现。超过90%的病例中存在特殊心脏组织受损,约7%的病例中心肌有改变。我们报告了一例以泵功能早期广泛恶化并发展为难治性充血性心力衰竭为特征的病例。胆囊和结肠平滑肌的同时受累证实了强直性肌营养不良症是一种泛肌肉疾病的假说。

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