改进法国转甲状腺素淀粉样变性的基因检测途径:挑战与策略。

IF 3.4 2区 医学 Q2 GENETICS & HEREDITY
Bérénice Hebrard, Marie-Lise Babonneau, Philippe Charron, Emilie Consolino, Benjamin Dauriat, Delphine Dupin-Deguine, Dominique Fargeaud, Agnès Farrugia, Anna-Gaëlle Giguet-Valard, Damien Guijarro, Jocelyn Inamo, Julien Jeanneteau, Jean-Michaël Mazzella, Claire-Cécile Michon, Gilles Millat, Frédéric Mouquet, Silvia Oghina, Yann Pereon, Vianney Poinsignon, Julie Pompougnac, Julie Proukhnitzky, Elise Schaefer, Franck Sturtz, Mathilde Trosdorf, Anne Auguste, Giorgia Canali, Alexandre Combes, Benoît Funalot, Thibaud Damy
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引用次数: 0

摘要

转甲状腺素淀粉样变性(ATTR)是一种严重而罕见的疾病,其特征是错误折叠的转甲状腺素蛋白进行性沉积,造成不可逆的器官损伤。转甲状腺素淀粉样变性可表现为遗传性 ATTR 或获得性野生型 ATTR。基因检测对于确定遗传倾向和随后启动适当的家族筛查至关重要。法国对基因检测有严格的规定,旨在保护受 ATTR 等遗传性疾病影响的患者及其家庭。然而,建立有效的基因检测途径仍面临挑战。一个由法国专家组成的多学科小组召开会议,讨论与 ATTR 基因诊断相关的挑战,并提出改进策略。主要挑战包括:缺乏路径标准化、医疗保健专业人员(HCP)与患者之间存在沟通障碍,以及难以满足监管要求。对患者数据安全和外包检测质量的担忧使问题进一步复杂化。建议采取的策略包括:为医疗保健专业人员和患者开发利益相关者图谱工具;开展教育计划以提高对基因检测法规的认识;提高医学遗传学家和遗传咨询师对疾病的认识;以及通过教育材料加强医疗保健专业人员与患者之间的沟通。这些举措旨在简化基因检测途径,提高法规合规性,最终为 ATTR 患者和家庭提供最佳支持。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Improving genetic testing pathways for transthyretin amyloidosis in France: challenges and strategies.

Transthyretin amyloidosis (ATTR) is a severe and rare disease characterized by the progressive deposition of misfolded transthyretin proteins, causing irreversible organ damage. Transthyretin amyloidosis can present as a hereditary ATTR or acquired wild-type ATTR form. Genetic testing is critical for determining a hereditary predisposition and subsequently initiating appropriate family screening. In France, strict regulations govern genetic testing that aim to protect patients and their families affected by hereditary diseases such as ATTR. However, challenges persist in establishing an effective genetic testing pathway. A multidisciplinary group of French experts convened to discuss the challenges associated with an ATTR genetic diagnosis and to propose improvement strategies. Key challenges include the lack of pathway standardization, communication gaps between healthcare professionals (HCPs) and patients, and difficulties in complying with regulatory requirements. Concerns about patient data safety and outsourced testing quality further complicate matters. Proposed strategies included the development of stakeholder mapping tools for HCPs and patients, educational programs to improve literacy on genetic testing regulations, increase disease awareness among medical geneticists and genetic counselors, and strengthening HCP-patient communication through educational materials. These initiatives aim to streamline the genetic testing pathway, enhance compliance with regulations, and ultimately provide optimal support for patients and families with ATTR.

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来源期刊
Orphanet Journal of Rare Diseases
Orphanet Journal of Rare Diseases 医学-医学:研究与实验
CiteScore
6.30
自引率
8.10%
发文量
418
审稿时长
4-8 weeks
期刊介绍: Orphanet Journal of Rare Diseases is an open access, peer-reviewed journal that encompasses all aspects of rare diseases and orphan drugs. The journal publishes high-quality reviews on specific rare diseases. In addition, the journal may consider articles on clinical trial outcome reports, either positive or negative, and articles on public health issues in the field of rare diseases and orphan drugs. The journal does not accept case reports.
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