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肥厚型心肌病的基因检测与咨询:常见问题解答

Genetic Testing and Counselling in Hypertrophic Cardiomyopathy: Frequently Asked Questions.

作者信息

Girolami Francesca, Gozzini Alessia, Pálinkás Eszter Dalma, Ballerini Adelaide, Tomberli Alessia, Baldini Katia, Marchi Alberto, Zampieri Mattia, Passantino Silvia, Porcedda Giulio, Calabri Giovanni Battista, Bennati Elena, Spaziani Gaia, Crotti Lia, Cecchi Franco, Favilli Silvia, Olivotto Iacopo

机构信息

Pediatric Cardiology Unit, Meyer Children's Hospital IRCCS, 50139 Florence, Italy.

Doctoral School of Clinical Medicine, University of Szeged, 6720 Szeged, Hungary.

出版信息

J Clin Med. 2023 Mar 24;12(7):2489. doi: 10.3390/jcm12072489.

Abstract

Genetic counselling and genetic testing in hypertrophic cardiomyopathy (HCM) represent an integral part of the diagnostic algorithm to confirm the diagnosis, distinguish it from phenocopies, and suggest tailored therapeutic intervention strategies. Additionally, they enable cascade genetic testing in the family. With the implementation of Next Generation Sequencing technologies (NGS), the interpretation of genetic data has become more complex. In this regard, cardiologists play a central role, aiding geneticists to correctly evaluate the pathogenicity of the identified genetic alterations. In the ideal setting, geneticists and cardiologists must work side by side to diagnose HCM as well as convey the correct information to patients in response to their many questions and concerns. After a brief overview of the role of genetics in the diagnosis of HCM, we present and discuss the frequently asked questions by HCM patients throughout our 20-year genetic counselling experience. Appropriate communication between the team and the families is key to the goal of delivering the full potential of genetic testing to our patients.

摘要

肥厚型心肌病(HCM)的遗传咨询和基因检测是诊断流程中不可或缺的一部分,用于确诊疾病、将其与表型相似疾病相区分,并提出针对性的治疗干预策略。此外,它们还能在家族中进行级联基因检测。随着下一代测序技术(NGS)的应用,基因数据的解读变得更加复杂。在这方面,心脏病专家发挥着核心作用,协助遗传学家正确评估所识别基因改变的致病性。在理想情况下,遗传学家和心脏病专家必须并肩合作,诊断HCM,并针对患者的诸多疑问和担忧向他们传达正确信息。在简要概述遗传学在HCM诊断中的作用后,我们根据20年的遗传咨询经验,呈现并讨论HCM患者常见的问题。团队与家庭之间的恰当沟通是充分发挥基因检测对患者潜在作用这一目标的关键。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b66b/10095452/4455a0db07b5/jcm-12-02489-g001.jpg

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