脊髓性肌肉萎缩症带菌者筛查计划:土耳其医护人员的认识和态度。

IF 1.5 Q4 GENETICS & HEREDITY Journal of Community Genetics Pub Date : 2024-10-11 DOI:10.1007/s12687-024-00737-4
Kubra Muge Celik, Canan Ceylan Kose, Derya Kaya, Koray Tekin, Fatma Silan
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引用次数: 0

摘要

脊髓性肌肉萎缩症(SMA)是一种常染色体隐性遗传病,由 SMN1 基因变异引起,导致进行性肌无力。据估计,SMN1 基因变异(包括变异和拷贝数变异)的携带者频率约为每 50 人中有 1 人,而 SMA 的全球患病率为每 10,000 名活产婴儿中有 1-3 人。为应对携带者比例的增加,特别是近亲结婚导致的携带者比例增加,土耳其于2021年启动了SMA携带者筛查计划。值得注意的是,最近在未接受携带者筛查的医护人员的子女中发现了 SMA 病例,这促使我们评估他们对该计划的了解程度。在获得伦理批准后,1,322 名医护人员根据《SMA 携带者筛查指南》完成了一项包含 15 个项目的调查。其中,5.8% 的人不了解 SMA,26% 的人缺乏有关国家筛查计划的信息。与初级医疗保健专业人员相比,二级和三级医疗保健专业人员对筛查计划的认知度明显较低(p
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Spinal muscular atrophy carrier screening program: awareness and attitude of healthcare professionals in Turkey.

Spinal Muscular Atrophy (SMA) is an autosomal recessive disease caused by variants in the SMN1 gene, leading to progressive muscle weakness. The carrier frequency of SMN1 gene variants, including variant and copy number variations, is estimated to be around 1 in 50 people, while the global prevalence of SMA is 1-3 per 10,000 live births. In response to the increasing carrier proportion, especially due to consanguineous marriages, Turkey launched the SMA Carrier Screening Program in 2021. Notably, recent SMA cases have been observed in the children of healthcare workers who did not undergo carrier screening, prompting us to evaluate their awareness of this program. After receiving ethics approval, 1,322 healthcare professionals completed a 15-item survey based on the SMA Carrier Screening Guidelines. Of these, 5.8% were unaware of SMA, and 26% lacked information about the national screening program. Awareness of the screening program was significantly lower among secondary and tertiary healthcare professionals compared to primary healthcare professionals (p < 0.0001) and among non-physician healthcare professionals compared to physicians (p < 0.0001). Additionally, a serious lack of knowledge was observed concerning the parts of the screening covering the pregnancy period. Although there is generally high awareness of the SMA Carrier Screening Program among healthcare professionals, significant knowledge gaps exist. These findings highlight the need for increased efforts to more effectively deliver screening programs and continue the education of healthcare professionals. Education and awareness campaigns can enhance program awareness and effectiveness, reach wider audiences, and contribute to preventive measures for the health of future generations.

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来源期刊
Journal of Community Genetics
Journal of Community Genetics GENETICS & HEREDITY-
CiteScore
3.30
自引率
5.30%
发文量
54
期刊介绍: The Journal of Community Genetics is an international forum for research in the ever-expanding field of community genetics, the art and science of applying medical genetics to human communities for the benefit of their individuals. Community genetics comprises all activities which identify persons at increased genetic risk and has an interest in assessing this risk, in order to enable those at risk to make informed decisions. Community genetics services thus encompass such activities as genetic screening, registration of genetic conditions in the population, routine preconceptional and prenatal genetic consultations, public education on genetic issues, and public debate on related ethical issues. The Journal of Community Genetics has a multidisciplinary scope. It covers medical genetics, epidemiology, genetics in primary care, public health aspects of genetics, and ethical, legal, social and economic issues. Its intention is to serve as a forum for community genetics worldwide, with a focus on low- and middle-income countries. The journal features original research papers, reviews, short communications, program reports, news, and correspondence. Program reports describe illustrative projects in the field of community genetics, e.g., design and progress of an educational program or the protocol and achievement of a gene bank. Case reports describing individual patients are not accepted.
期刊最新文献
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