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心脏 MRI 生物标志物在杜氏肌营养不良症中的应用。

Cardiac MRI biomarkers for Duchenne muscular dystrophy.

机构信息

Department of Radiological Sciences, University of California, Los Angeles, CA 90024, USA.

Department of Bioengineering, University of California, Los Angeles, CA 90095, USA.

出版信息

Biomark Med. 2018 Nov;12(11):1271-1289. doi: 10.2217/bmm-2018-0125. Epub 2018 Nov 30.

Abstract

Duchenne muscular dystrophy (DMD) is a fatal inherited genetic disorder that results in progressive muscle weakness and ultimately loss of ambulation, respiratory failure and heart failure. Cardiac MRI (MRI) plays an increasingly important role in the diagnosis and clinical care of boys with DMD and associated cardiomyopathies. Conventional cardiac MRI biomarkers permit measurements of global cardiac function and presence of fibrosis, but changes in these measures are late manifestations. Emerging MRI biomarkers of myocardial function and structure include the estimation of rotational mechanics and regional strain using MRI tagging; T1-mapping; and T2-mapping, a marker of inflammation, edema and fat. These emerging biomarkers provide earlier insights into cardiac involvement in DMD, improving patient care and aiding the evaluation of emerging therapies.

摘要

杜氏肌营养不良症(DMD)是一种致命的遗传性疾病,会导致进行性肌肉无力,最终丧失行走能力、呼吸衰竭和心力衰竭。心脏磁共振成像(MRI)在 DMD 男孩和相关心肌病的诊断和临床护理中发挥着越来越重要的作用。传统的心脏 MRI 生物标志物可用于测量整体心脏功能和纤维化的存在,但这些指标的变化是晚期表现。心肌功能和结构的新兴 MRI 生物标志物包括使用 MRI 标记估计旋转力学和局部应变;T1 映射;以及 T2 映射,这是炎症、水肿和脂肪的标志物。这些新兴的生物标志物更早地揭示了 DMD 患者的心脏受累情况,改善了患者的护理,并有助于评估新兴的治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e022/6462870/96c4e12a0ade/bmm-12-1271-g1.jpg

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