COVID-19感染后发现的ZASP/ ldb3相关非典型远端肌病伴轻微心脏损害:简短报告

Giulio Gadaleta, Stefano Pidello, Guido Urbano, Diana Carli, Tiziana Mongini
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引用次数: 0

摘要

病例介绍:一名34岁男性先天性内翻足,运动后横纹肌溶解,中年心脏性猝死家族史,因严重横纹肌溶解需要多次血液透析而被评估。临床评估显示轻度远端肌病征象,CK水平约3000 IU/L,肌肉活检显示desmin和dystrophin阳性的细胞质蛋白聚集/纤维分裂。55岁轻微的SARS-CoV-2感染后,通过心脏MRI观察到轻微的心肌病迹象,没有心肌炎的迹象。随后,NGS分析确定了LDB3基因的变异,可能与临床-组织学-放射学图像相关,从而拓宽了LDB3相关远端肌病的表型谱。此外,病毒感染与其他微妙的心肌病的恶化之间可能存在联系。在高血凝和阳性家族史的背景下,不明原因的心脏异常,广谱NGS检测和心脏MRI在选定的情况下,应考虑及时诊断和干预。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
ZASP/LDB3-related atypical distal myopathy with subtle cardiac impairment unveiled after COVID-19 infection: a short report.

Case presentation: A 34-year-old male with congenital clubfoot, post-exertional rhabdomyolysis, and a family history of sudden cardiac death in mid-life was evaluated for a severe rhabdomyolysis requiring multiple hemodialyses. Clinical evaluation showed mild distal myopathy signs, with CK levels around 3000 IU/L and muscle biopsy revealing desmin- and dystrophin-positive cytosolic protein aggregates/fibre splitting. After a minor SARS-CoV-2 infection at 55, modest signs of cardiomyopathy were observed via cardiac MRI, without patterns indicative of myocarditis. Subsequently, NGS analysis identified a variant in the LDB3 gene, potentially correlated with the clinical-histological-radiological picture, thus broadening the phenotypic spectrum of LDB3-related distal myopathies. Additionally, a possible link was suggested between the viral infection and the exacerbation of the otherwise subtle cardiomyopathy. In the context of hyperCKemia and positive family history for unexplained cardiac abnormalities, broad-spectrum NGS testing, and cardiac MRI in selected cases, should be considered for timely diagnosis and interventions.

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