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贝氏肌营养不良症中的心肌病:概述

Cardiomyopathy in becker muscular dystrophy: Overview.

作者信息

Ho Rady, Nguyen My-Le, Mather Paul

机构信息

Rady Ho, My-Le Nguyen, Paul Mather, Department of Internal Medicine, Thomas Jefferson University Hospital, Philadelphia, PA 19107, United States.

出版信息

World J Cardiol. 2016 Jun 26;8(6):356-61. doi: 10.4330/wjc.v8.i6.356.

Abstract

Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. Cardiac involvement in BMD has been described and cardiomyopathy represents the number one cause of death in these patients. In this paper, the pathophysiology, clinical evaluations and management of cardiomyopathy in patients with BMD will be discussed.

摘要

贝克尔肌营养不良症(BMD)是一种X连锁隐性疾病,涉及肌营养不良蛋白基因的突变。BMD患者的心脏受累情况已有报道,心肌病是这些患者的首要死因。本文将讨论BMD患者心肌病的病理生理学、临床评估和管理。

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