{"title":"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","authors":"Giulio Gadaleta, Stefano Pidello, Guido Urbano, Diana Carli, Tiziana Mongini","doi":"10.36185/2532-1900-787","DOIUrl":null,"url":null,"abstract":"<p><strong>Case presentation: </strong>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.</p>","PeriodicalId":93851,"journal":{"name":"Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology","volume":"44 2","pages":"77-80"},"PeriodicalIF":0.0000,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12250588/pdf/","citationCount":"0","resultStr":"{\"title\":\"ZASP/LDB3-related atypical distal myopathy with subtle cardiac impairment unveiled after COVID-19 infection: a short report.\",\"authors\":\"Giulio Gadaleta, Stefano Pidello, Guido Urbano, Diana Carli, Tiziana Mongini\",\"doi\":\"10.36185/2532-1900-787\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Case presentation: </strong>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.</p>\",\"PeriodicalId\":93851,\"journal\":{\"name\":\"Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology\",\"volume\":\"44 2\",\"pages\":\"77-80\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-06-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12250588/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.36185/2532-1900-787\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.36185/2532-1900-787","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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.