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对于因心肌病入院的贝克肌肉萎缩症患者,物理医学与康复咨询的重要性。

The Importance of a PM&R Consultation for Becker Muscular Dystrophy Patients Admitted with Cardiomyopathy.

作者信息

Cherin Neyha, Patel Shivani, Jukic Michelle

机构信息

Department of Physical Medicine and Rehabilitation, Penn State Health Milton S. Hershey Medical Center, Hershey, PA, USA.

出版信息

Case Rep Cardiol. 2024 Mar 22;2024:3145086. doi: 10.1155/2024/3145086. eCollection 2024.

Abstract

Becker muscular dystrophy (BMD) is a rare genetic disorder that is associated with significant cardiac compromise, including heart failure and cardiomyopathy. Given the significant cardiac impact of the disease, patients are commonly hospitalized under the care of cardiologists. While it is imperative to address the acute cardiac challenges these patients face, it is crucial to not disregard the musculoskeletal derangement that occurs from this underlying disease and how acute hospitalization can exacerbate these issues. While literature focuses heavily on providing management protocols to address these acute cardiac complications, it is also important for providers to feel supported in addressing the functional implications that hospitalized BMD patients may face. An early PM&R consultation in the inpatient setting can be useful in identifying and addressing the functional impairments and subsequent comorbidities of BMD patients. PM&R teams can provide oversight from multiple avenues including the psychosocial, neurocognitive, durable medical equipment, and pain management perspectives and assist with transition of care to the postacute rehabilitation setting. The ultimate goal of the PM&R provider is to work alongside the primary service and patient in order to assist with retaining independence, improving patient satisfaction, and most importantly improving quality of life both inside and outside of the hospital setting.

摘要

贝克尔肌营养不良症(BMD)是一种罕见的遗传性疾病,与严重的心脏损害有关,包括心力衰竭和心肌病。鉴于该疾病对心脏的重大影响,患者通常在心脏病专家的护理下住院治疗。虽然必须应对这些患者面临的急性心脏挑战,但至关重要的是不要忽视这种基础疾病导致的肌肉骨骼紊乱以及急性住院如何加剧这些问题。虽然文献主要侧重于提供管理方案来应对这些急性心脏并发症,但对于医疗服务提供者而言,在应对住院的BMD患者可能面临的功能影响方面获得支持也很重要。在住院环境中尽早进行物理医学与康复(PM&R)会诊,有助于识别和解决BMD患者的功能障碍及随后的合并症。PM&R团队可以从多个方面提供监督,包括心理社会、神经认知、耐用医疗设备和疼痛管理等角度,并协助将护理过渡到急性后期康复环境。PM&R医疗服务提供者的最终目标是与主要医疗服务团队和患者合作,以帮助患者保持独立、提高患者满意度,最重要的是提高患者在医院内外的生活质量。

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Psychiatric and neurodevelopmental aspects of Becker muscular dystrophy.贝克型肌营养不良的精神神经发育方面。
Neuromuscul Disord. 2019 Dec;29(12):930-939. doi: 10.1016/j.nmd.2019.09.006. Epub 2019 Sep 18.
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Cardiomyopathy in becker muscular dystrophy: Overview.贝氏肌营养不良症中的心肌病:概述
World J Cardiol. 2016 Jun 26;8(6):356-61. doi: 10.4330/wjc.v8.i6.356.
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Dystrophin-Deficient Cardiomyopathy.肌营养不良性心肌病。
J Am Coll Cardiol. 2016 May 31;67(21):2533-46. doi: 10.1016/j.jacc.2016.02.081.

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