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家族性肥厚型心肌病:一例MYBPC3基因新突变病例。

Familial hypertrophic cardiomyopathy: A case with a new mutation in the MYBPC3 gene.

作者信息

Hallıoğlu Kılınç Olgu, Giray Dilek, Bişgin Atıl, Tuğ Bozdoğan Sevcan, Karpuz Derya

机构信息

Department of Pediatric Cardiology, Mersin University Faculty of Medicine, Mersin, Turkey.

出版信息

Turk Kardiyol Dern Ars. 2017 Jul;45(5):450-453. doi: 10.5543/tkda.2016.56267.

Abstract

Familial hypertrophic cardiomyopathy is a genetically heterogeneous disease with variable clinical features that is inherited as autosomal dominant with variable penetrance. Recent developments in genetics of hereditary cardiomyopathy have not only enlightened many points about pathogenesis, but have also provided great benefit to diagnostic approaches of clinicians. Heterozygous mutation of c3691-3692insTTCA in MYBPC3 gene was identified in a pediatric patient with diagnosis of hypertrophic cardiomyopathy at clinic. Hypertrophy was observed in sister and father of the patient in echocardiography screening, and it was subsequently determined that they also had same mutation. This mutation has not previously been defined and reported previously in the literature as cause of hypertrophic cardiomyopathy.

摘要

家族性肥厚型心肌病是一种具有遗传异质性、临床特征多样的疾病,以常染色体显性方式遗传,外显率可变。遗传性心肌病遗传学的最新进展不仅在发病机制方面阐明了许多要点,也为临床医生的诊断方法带来了很大益处。在一名临床诊断为肥厚型心肌病的儿科患者中,发现了MYBPC3基因c3691 - 3692insTTCA的杂合突变。在超声心动图筛查中,该患者的姐姐和父亲也观察到心肌肥厚,随后确定他们也有相同的突变。此前该突变在文献中未曾作为肥厚型心肌病的病因被定义和报道过。

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