Cardiac Involvement in Becker Muscular Dystrophy: Insights from Echocardiographic Analysis.

IF 2 4区 医学 Q3 CLINICAL NEUROLOGY
Jihye You, Mi Jin Kim, Seulgi Cha, Jae Suk Baek, Mi-Sun Yum, Beom Hee Lee, Jeong Jin Yu
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引用次数: 0

Abstract

Becker muscular dystrophy is an infrequent genetic disorder that results from dystrophin gene mutations. Cardiac involvement is a primary manifestation. The time of onset of underlying cardiac functional abnormalities remains largely undefined. This study involved 17 pediatric patients with Becker muscular dystrophy who visited our hospital between January 1, 2002, and December 31, 2018, and underwent echocardiographic imaging analysis. Another set of 17 controls matched for age and sex to the patient cohort was chosen for comparison. Patients with Becker muscular dystrophy demonstrated a decline in echocardiographic measures, especially deformation parameters, compared with the control group. This alteration is influenced by age. Our findings suggest that early echocardiographic monitoring may help identify subclinical cardiac dysfunction, particularly in younger patients (<10 years of age).

贝克尔肌萎缩症的心脏受累:来自超声心动图分析的见解。
贝克肌营养不良症是一种罕见的遗传性疾病,由肌营养不良蛋白基因突变引起。心脏受累是主要表现。潜在心脏功能异常的发病时间在很大程度上仍不明确。本研究纳入了2002年1月1日至2018年12月31日期间到我院就诊的17例贝克尔肌萎缩症患儿,并对其进行了超声心动图成像分析。另一组17名年龄和性别与患者队列相匹配的对照组被选中进行比较。与对照组相比,贝克尔肌营养不良症患者的超声心动图指标下降,尤其是变形参数。这种变化受年龄的影响。我们的研究结果表明,早期超声心动图监测可能有助于识别亚临床心功能障碍,特别是在年轻患者(
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来源期刊
Journal of Child Neurology
Journal of Child Neurology 医学-临床神经学
CiteScore
4.20
自引率
5.30%
发文量
111
审稿时长
3-6 weeks
期刊介绍: The Journal of Child Neurology (JCN) embraces peer-reviewed clinical and investigative studies from a wide-variety of neuroscience disciplines. Focusing on the needs of neurologic patients from birth to age 18 years, JCN covers topics ranging from assessment of new and changing therapies and procedures; diagnosis, evaluation, and management of neurologic, neuropsychiatric, and neurodevelopmental disorders; and pathophysiology of central nervous system diseases.
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