有可能成为杜兴氏和贝克氏肌肉萎缩症携带者的妇女掌握遗传信息的能力。

IF 1.5 Q4 GENETICS & HEREDITY Journal of Community Genetics Pub Date : 2024-04-01 Epub Date: 2024-01-02 DOI:10.1007/s12687-023-00695-3
Alice Maria Luderitz Hoefel, Cesar Augusto Weschenfelder, Bruna Faria Rosa, Karina Carvalho Donis, Jonas Alex Morales Saute
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引用次数: 0

摘要

作用于杜兴氏和贝克氏肌肉营养不良症(DBMD)特定分子靶点的疗法的出现,扩大了诊断性 DMD 分析的可及性。然而,目前还不清楚这些进展在多大程度上改善了医疗保健,以及高危女性基因携带者接受基因检测的机会。本研究评估了遗传咨询的过程以及 DBMD 家庭中女性对遗传信息的掌握情况。我们于 2022 年 2 月至 6 月在巴西开展了一项横断面研究。从罕见病参考服务机构和全国性患者权益组织招募的 123 名妇女回答了在线调查,调查项目包括社会人口学数据、家族史、获得医疗服务的途径、生育决定和基因组结果量表。77/123(62.6%)名妇女表示接受过遗传咨询,53.7%的妇女表示进行过 DMD 遗传分析。虽然大多数人都知道携带者患心脏病和肌无力的风险,但只有 35% 的潜在携带者在一生中至少进行过一次心脏检查。国家地区、亲属关系类型、家庭中受影响男性的数量、年龄、遗传风险意识、教育水平以及是否参加宣传小组是影响妇女获得充分医疗保健服务和遗传信息的主要因素。无论是公共政策还是对制药公司诊断项目的监管,对医疗专业人员的教育以及扩大携带者基因检测机会的政策,对于改善巴西DBMD患者家庭的护理都是至关重要的。
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Empowerment of genetic information by women at-risk of being carriers of Duchenne and Becker muscular dystrophies.

The emergence of therapies acting on specific molecular targets for Duchenne and Becker muscular dystrophies (DBMD) led to expanded access of diagnostic DMD analysis. However, it is unclear how much of these advances have also improved healthcare and access to genetic testing for women at-risk of being carriers. This study evaluates the process of genetic counseling and empowerment of genetic information by women from DBMD families. We carried out a cross-sectional study between February and June 2022 in Brazil. The online survey with items regarding sociodemographic data; family history; access to health services; reproductive decisions; and the Genomic Outcome Scale was answered by 123 women recruited from a rare diseases reference service and a nationwide patient advocacy group. Genetic counseling was reported by 77/123 (62.6%) of women and 53.7% reported having performed genetic analysis of DMD. Although the majority knew about the risks for carriers of developing heart disease and muscle weakness, only 35% of potential carriers have had cardiac studies performed at least once in their lives. Country region, type of kinship, number of affected males in the family, age, notion of genetic risk, education level, and participation in advocacy groups were the main factors associated with adequate healthcare access to women and empowerment of genetic information. Education to health professionals and policies to expand access to carrier genetic testing, whether public policies or regulation of pharmaceutical companies' diagnostic programs, is paramount to improve the care of families with DBMD in Brazil.

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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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