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首页> 外文期刊>Journal of the American Academy of Nurse Practitioners. >Current understanding and management of dilated cardiomyopathy in Duchenne and Becker muscular dystrophy.
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Current understanding and management of dilated cardiomyopathy in Duchenne and Becker muscular dystrophy.

机译：Duchenne和Becker肌营养不良症中扩张心肌病的现有理解和管理。

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PURPOSE: To review the current understanding of the pathophysiology of dilated cardiomyopathy (DCM) in patients with Duchenne and Becker muscular dystrophies, assessment of cardiac dysfunction for these patients, and the recommended pharmacological treatment options and ongoing research directions. DATA SOURCES: Reviews and original research articles from scholarly journals and books. CONCLUSIONS: Duchenne and Becker muscular dystrophies are debilitating neuromuscular disorders, both caused by mutations in the dystrophin gene. Most patients develop DCM as part of the disease course; in fact, DCM is the leading cause of death among these patients. Cardiac surveillance, including routine monitoring of electrocardiograms, echocardiograms, and appropriate blood biomarkers, may detect early DCM development. Although previous studies have shown that early administration of cardiac medications may delay the development of DCM, current standard of care does not emphasize cardiac surveillance and timely treatment. This, in turn, limits clinicians, including advanced practice nurses, to be optimally engaged in providing the most aggressive and proactive patient care. IMPLICATIONS FOR PRACTICE: Implementing a routine cardiac assessment and timely pharmacological treatment in primary or specialty care settings is highlighted as an important step to optimize cardiac health among patients with Duchenne and Becker muscular dystrophies.
机译：目的：审查目前对Duchenne和Becker肌营养不良患者的扩张心肌病（DCM）病理生理学的理解，对这些患者的心脏功能障碍评估，以及推荐的药理学治疗方案和正在进行的研究方向。数据来源：来自学术期刊和书籍的评论和原创研究文章。结论：Duchenne和Becker肌肉营养不良是衰弱的神经肌肉障碍，既由营养不良素基因突变引起。大多数患者作为疾病课程的一部分发展DCM;事实上，DCM是这些患者中死亡的主要原因。心脏监测，包括心电图，超声心动图和适当的血液生物标志物的常规监测，可能会检测到早期的DCM发育。虽然之前的研究表明，早期的心脏药物施用可能会推迟DCM的发展，目前的护理标准并不强调心脏监测和及时治疗。反过来，这限制了临床医生，包括高级练习护士，以最佳地从事提供最具侵略性和积极主动的患者护理。对实践的影响：实施常规心脏评估和在初级或特种护理环境中的及时药理学治疗是优化Duchenne和Becker肌营养不良症患者心脏健康的重要步骤。

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