如何将当前的基因治疗科学转化为血友病护理并付诸实施?

IF 3.4 3区 医学 Q2 HEMATOLOGY
Therapeutic Advances in Hematology Pub Date : 2023-01-12 eCollection Date: 2023-01-01 DOI:10.1177/20406207221145627
Cedric Hermans, Yves Gruel, Laurent Frenzel, Evelien Krumb
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引用次数: 0

摘要

基因疗法为血友病(PWH)患者的治疗开辟了一种全新的模式,通过转移旨在替代血友病患者自身缺陷基因的功能基因,使第八因子(FVIII)或第九因子(FIX)能够持续表达,从而为血友病患者提供功能性治愈的可能性。近年来,基因疗法取得了重大进展,使凝血因子的活性接近正常水平,这体现在以前的重症患者只需服用一次腺相关病毒(AAV)载体就能实现 "零出血率"。虽然这一新方法代表了一项重大进步,但在将该技术应用于临床实践之前,仍有几个问题必须解决。首先,必须进一步加强对基因疗法的治疗潜力和模式的认识、交流和教育。为此,必须在患者组织的支持下,以适当的方式和易懂的语言,分享客观、公正、透明和定期更新的信息。其次,医疗服务提供者应采取以患者为中心的方法,因为在考虑基因疗法时,"一刀切 "的方法是不恰当的。取而代之的是,必须从患者的整体角度出发,考虑他们的身体和心理状况,以及未表达的期望和偏好。第三,必须改进同意程序,确保最大限度地保护病人的利益。最后,基因治疗很可能首先在少数几个中心进行,这些中心在这一领域拥有最丰富的专业知识和经验。因此,病人的管理应采用中心辐射模式,同时考虑到基因疗法成功的关键在于血友病中心内部以及血友病中心之间在国际登记框架内分享经验时的最佳沟通与合作。本综述介绍了最近的进展,并解释了必须解决的突出障碍,以促进这种改变模式的新疗法的实施。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

How to translate and implement the current science of gene therapy into haemophilia care?

How to translate and implement the current science of gene therapy into haemophilia care?

How to translate and implement the current science of gene therapy into haemophilia care?

How to translate and implement the current science of gene therapy into haemophilia care?

Gene-based therapy opens an entirely new paradigm in managing people with haemophilia (PWH), offering them the possibility of a functional cure by enabling continuous expression of factor VIII (FVIII) or factor IX (FIX) after transfer of a functional gene designed to replace the PWH's own defective gene. In recent years, significant advances in gene therapy have been made, resulting in clotting factor activity attaining near-normal levels, as reflected by 'zero bleeding rates' in previously severely inflicted patients following a single administration of adeno-associated viral (AAV) vectors. While this new approach represents a major advancement, there are still several issues that must be resolved before applying this technology in clinical practice. First, awareness, communication, and education about the therapeutic potential and modalities of gene therapy must be further strengthened. To this end, objective, unbiased, transparent, and regularly updated information must be shared, in an appropriate way and understandable language with the support of patients' organizations. Second, healthcare providers should adopt a patient-centred approach, as the 'one size fits all' approach is inappropriate when considering gene therapy. Instead, a holistic patient view taking into account their physical and mental dimensions, along with unexpressed expectations and preferences, is mandatory. Third, the consent procedure must be improved, ensuring that patients' interests are maximally protected. Finally, gene therapy is likely to be first delivered in a few centres, with the highest expertise and experience in this domain. Thus, patients should be managed based on a hub-and-spoke model, taking into account that the key to gene therapy's success lies in an optimal communication and collaboration both within and between haemophilia centres sharing their experiences in the frame of international registries. This review describes recent progress and explains outstanding hurdles that must be tackled to ease the implementation of this paradigm-changing new therapy.

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来源期刊
CiteScore
4.30
自引率
0.00%
发文量
54
审稿时长
7 weeks
期刊介绍: Therapeutic Advances in Hematology delivers the highest quality peer-reviewed articles, reviews, and scholarly comment on pioneering efforts and innovative studies across all areas of hematology. The journal has a strong clinical and pharmacological focus and is aimed at clinicians and researchers in hematology, providing a forum in print and online for publishing the highest quality articles in this area.
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