血液病学家作为血红蛋白病的遗传顾问:他们准备好了吗?

IF 1.2 Q4 HEMATOLOGY
Michael Angastiniotis, Androulla Eleftheriou
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引用次数: 0

摘要

背景/目的:在血液学中,广泛的血液疾病是遗传性的。地中海贫血是一种遗传性贫血,其特点是在公共卫生层面造成很高的疾病负担,对许多卫生系统的资源构成挑战。本综述的重点是地中海贫血,许多国家已为此制定了筛查和预防规划。为了管理这一沉重负担,多年来采取了两种方法。第一个方案的重点是通过适当的非指导性遗传咨询,减少每年受影响的新生儿,使父母有机会就其生殖生活作出知情的选择。第二种方法与早期治疗方法的发展有关,如造血干细胞移植(HSCT),随着持续不断的改进努力,近年来基于基因的整体治疗取得了成功的进展。遗传咨询是成功预防的重要组成部分,旨在告知被发现为携带者的个人和双方都是携带者的夫妇,在隐性孟德尔遗传的情况下,每次怀孕都有25%的风险生下受影响的孩子。问题很多,这可能需要讨论,突出了遗传咨询师在每次咨询会议中期望拥有和适当利用的技能水平。令人关切的是,这种训练有素和熟练的专业人员数量很少,而且没有很好地融入处理这些复杂疾病控制的多学科小组。根据我们的经验,对于血液疾病,咨询很少由合格的科学家提供。我们坚信,有必要将遗传咨询纳入血液学服务的一个组成部分。方法:为了调查当前的做法,我们借鉴了现有项目的经验,以及已发表的文献。结果:目前,在几乎所有的血红蛋白病预防方案中,咨询是由负责临床护理的临床医生提供的,在某些情况下,由诊所的护士或筛查实验室的科学家提供。结论:地中海贫血国际联合会建议并正在制定咨询方面的特殊培训,作为血液学培训的一部分,并为已经在实践中的人员制定专业发展模块。考虑到必须讨论的问题的复杂性,在可能的情况下应考虑采用多学科的咨询方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Haematologists as Genetic Counsellors for Haemoglobinopathies: Are They Prepared?

Background/Objectives: In haematology, a wide range of blood disorders are hereditary. The thalassaemias are hereditary anaemias characterised by a high burden of disease at the public health level, challenging the resources of many health systems. This review focuses on thalassaemias for which many countries have developed screening and prevention programmes. To manage this heavy burden, two approaches were introduced over the years. The first one focused on reducing the annual affected births consequent to appropriate non-directive genetic counselling, offering to the parents the chance to make an informed choice concerning their reproductive lives. The second approach was related to the development of curative treatments such as haematopoietic stem cell transplantation (HSCT) in the early years, with continued ongoing efforts for improvements, followed by successful advances in gene-based holistic cures in more recent years. Genetic counselling is a vital component in successful prevention, aiming at informing individuals who are found to be carriers and couples who are both carriers with a 25% risk at every pregnancy of having an affected child in the case of recessive, Mendelian inheritance. The issues are many, and that may have to be discussed, highlighting the level of skills which a genetic counsellor is expected to possess and utilise appropriately in every counselling session. The concern is that such trained and skilled professionals are few in number and not well integrated into the multidisciplinary groups addressing the control of these complex disorders. It is our experience that for blood disorders, counselling is rarely in the hands of qualified scientists. It is our firm belief that it is necessary to incorporate genetic counselling as an integral part of haematology services. Methods: To investigate current practices we have drawn on the experience of existing programmes, as well as published literature. Results: Currently, in almost all haemoglobinopathy prevention programmes, counselling is offered by the clinicians in charge of clinical care or, in some settings, by the nurse of the clinic or the screening laboratory scientist. Conclusions: The Thalassaemia International Federation suggests and is in the process of developing special training in counselling as part of haematology training, as well as professional development modules for those already in practice. Considering the complexity of the issues that must be discussed, a multidisciplinary approach to counselling should be considered where possible.

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来源期刊
Hematology Reports
Hematology Reports HEMATOLOGY-
CiteScore
0.90
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47
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10 weeks
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