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Duchenne 肌营养不良症扩张型心肌病的药物治疗:系统评价。

Pharmacological management of dilated cardiomyopathy in Duchenne muscular dystrophy: A systematic review.

机构信息

University of Nicosia Medical School, Nicosia, Cyprus.

University of Nicosia Medical School, Nicosia, Cyprus.

出版信息

Hellenic J Cardiol. 2023 Nov-Dec;74:58-64. doi: 10.1016/j.hjc.2023.06.007. Epub 2023 Jul 3.

Abstract

Duchenne muscular dystrophy is a fatal X-linked recessive disease affecting approximately 1 in 3500 births. It is characterized by a genetic lack of dystrophin, which is an essential protein for maintaining muscle integrity. The lack of dystrophin plays a pathophysiological role in the development of dilated cardiomyopathy in Duchenne muscular dystrophy. Currently, no consensus exists on specific pharmacological therapy guidelines for these patients; however, it centers around the guidelines for heart failure management. This systematic review investigated 12 randomized control trials dating back to 2005 in the pharmacotherapy of patients with dilated cardiomyopathy Duchenne muscular dystrophy. This review specifically included angiotensin-converting enzyme inhibitors, aldosterone receptor blockers, angiotensin receptor/neprilysin inhibitors, beta-blockers, and mineralocorticoid receptor antagonists. Despite their limitations, these studies have shown promising effects in improving the overall heart function and prognosis in patients with this condition. However, to attain higher statistical significance, future studies should investigate larger populations and for longer periods.

摘要

杜氏肌营养不良症是一种致命的 X 连锁隐性疾病,影响大约每 3500 名新生儿中的 1 名。它的特征是肌营养不良蛋白的遗传缺失,肌营养不良蛋白是维持肌肉完整性的必需蛋白。肌营养不良蛋白的缺失在杜氏肌营养不良症扩张型心肌病的发展中起着病理生理作用。目前,针对这些患者的具体药物治疗指南尚无共识,但其核心是心力衰竭管理指南。本系统评价调查了 2005 年以来的 12 项随机对照试验,研究了扩张型心肌病杜氏肌营养不良症患者的药物治疗。本综述特别纳入了血管紧张素转换酶抑制剂、醛固酮受体阻滞剂、血管紧张素受体/脑啡肽酶抑制剂、β受体阻滞剂和盐皮质激素受体拮抗剂。尽管存在局限性,但这些研究表明,这些药物在改善此类患者的整体心脏功能和预后方面具有有前景的效果。然而,为了获得更高的统计学意义,未来的研究应该调查更大的人群并延长研究时间。

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