在中等收入国家克服遗传性神经肌肉诊断陷阱。

IF 4.1 Q1 CLINICAL NEUROLOGY Brain communications Pub Date : 2024-11-14 eCollection Date: 2024-01-01 DOI:10.1093/braincomms/fcae342
Rodrigo Siqueira Soares Frezatti, Pedro José Tomaselli, Christopher J Record, Lindsay A Wilson, Gustavo Maximiano Alves, Natalia Dominik, Stephanie Efthymiou, Krutik Patel, Jana Vandrovcova, Roope Männikkö, Robert D S Pitceathly, Claudia Ferreira da Rosa Sobreira, Robert McFarland, Robert W Taylor, Henry Houlden, Michael G Hanna, Mary M Reilly, Wilson Marques
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引用次数: 0

摘要

神经肌肉疾病影响着全球近 2000 万人。分子诊断技术的进步为人们深入了解神经肌肉疾病提供了宝贵的资料,从而提高了护理标准和有针对性的治疗方法。尽管取得了这一进展,但在巴西等中等收入国家,获得基因组诊断的机会仍然很少,而且不一致。在这一过程中,缺乏可行的基因诊断公共卫生政策以及神经肌肉疾病专家的短缺是主要原因。我们报告了我们在神经肌肉疾病跨洲基因组联盟中的经验,强调了合作努力如何帮助我们克服了诊断患者过程中的各种障碍。我们描述了几个具有挑战性的病例,分为三大主题,强调了基因诊断中的重大差距:(i) 反向表型和变异验证,(ii) 深度表型和确定定制的分子方法,(iii) 探索全外显子测序以外的基因组测试的使用。我们采用基于案例的定性方法,举例说明中等收入国家在基因组诊断中常见的误区。我们的经验表明,建立虚拟的跨洲合作伙伴关系是可行的,它能有效地交流科学经验,为当地的合理决策和专业培训提供指导,并能获得不同的分子诊断策略和功能分析。诸如此类的合作努力有可能克服当地的障碍,加强科研能力,培养多样化的多种族队列,并最终为患者提供更好的治疗。
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Overcoming genetic neuromuscular diagnostic pitfalls in a middle-income country.

Neuromuscular disorders affect almost 20 million people worldwide. Advances in molecular diagnosis have provided valuable insights into neuromuscular disorders, allowing for improved standards of care and targeted therapeutic approaches. Despite this progress, access to genomic diagnosis remains scarce and inconsistent in middle-income countries such as Brazil. The lack of public health policies to enable feasible genetic diagnosis and the shortage of neuromuscular disorders specialists are the main reasons in this process. We report our experience in a transcontinental genomic consortium for neuromuscular disorders highlighting how collaborative efforts have helped overcome various obstacles in diagnosing our patients. We describe several challenging cases categorized into three major themes, underlining significant gaps in genetic diagnosis: (i) reverse phenotyping and variant validation, (ii) deep phenotyping and identifying a bespoke molecular approach, and (iii) exploring the use of genomic tests beyond whole exome sequencing. We applied a qualitative case-based approach to exemplify common pitfalls in genomic diagnosis in a middle-income country. Our experience has shown that establishing a virtual transcontinental partnership is viable, offering effective exchange of scientific experiences, providing both guidance for rational decision-making and specialized training on a local level and access to diverse molecular diagnosis strategies and functional analyses. Collaborative efforts such as these have the potential to overcome local obstacles, strengthen scientific capabilities, foster diverse multi-ethnic cohorts, and ultimately provide improved care for patients.

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期刊最新文献
Mapping sentence comprehension and syntactic complexity: evidence from 131 stroke survivors. Overcoming genetic neuromuscular diagnostic pitfalls in a middle-income country. Compensatory mechanisms amidst demyelinating disorders: insights into cognitive preservation. The Fork sign: a new cortical landmark in the human brain. Glial fibrillary acidic protein in Alzheimer's disease: a narrative review.
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