• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

GJB2-R143W 相关听力障碍的筛查:对加纳卫生政策和实践的影响。

Screening for GJB2-R143W-Associated Hearing Impairment: Implications for Health Policy and Practice in Ghana.

机构信息

West African Centre for Cell Biology of Infectious Pathogens (WACCBIP), University of Ghana, Accra, Ghana.

Division of Human Genetics, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa.

出版信息

Public Health Genomics. 2020;23(5-6):184-189. doi: 10.1159/000512121. Epub 2020 Dec 10.

DOI:10.1159/000512121
PMID:33302283
Abstract

Genetic factors significantly contribute to the burden of hearing impairment (HI) in Ghana as there is a high carrier frequency (1.5%) of the connexin 26 gene founder variant GJB2-R143W in the healthy Ghanaian population. GJB2-R143W mutation accounts for nearly 26% of causes in families segregating congenital non-syndromic HI. With HI associated with high genetic fitness, this indicates that Ghana will likely sustain an increase in the number of individuals living with inheritable HI. There is a universal newborn hearing screening (UNHS) program in Ghana. However, this program does not include genetic testing. Adding genetic testing of GJB2-R143W mutation for the population, prenatal and neonatal stages may lead to guiding genetic counseling for individual and couples, early detection of HI for at-risk infants, and improvement of medical management, including speech therapy and audiologic intervention, as well as provision of the needed social service to enhance parenting and education for children with HI. Based on published research on the genetics of HI in Ghana, we recommend that the UNHS program should include genetic screening for the GJB2-R143W gene variant for newborns who did not pass the initial UNHS tests. This will require an upgrade and resourcing of public health infrastructures to implement the rapid and cost-effective GJB2-R143W testing, followed by appropriate genetic and anticipatory guidance for medical care.

摘要

遗传因素在加纳听力障碍(HI)的负担中起着重要作用,因为在健康的加纳人群中,间隙连接蛋白 26 基因的 GJB2-R143W 创始人变体的携带频率很高(1.5%)。GJB2-R143W 突变几乎占家族性先天性非综合征 HI 原因的 26%。由于 HI 与高遗传适应性相关,这表明加纳可能会有更多遗传性 HI 的个体。加纳有一个普遍的新生儿听力筛查(UNHS)计划。然而,该计划不包括基因检测。对人群进行 GJB2-R143W 突变的基因检测,在产前和新生儿阶段,可能会导致为个人和夫妇提供遗传咨询,早期发现高危婴儿的 HI,并改善医疗管理,包括语言治疗和听力干预,以及提供必要的社会服务,以提高 HI 儿童的养育和教育水平。基于加纳 HI 遗传学的已发表研究,我们建议 UNHS 计划应包括对未通过初始 UNHS 测试的新生儿进行 GJB2-R143W 基因突变的基因筛查。这将需要升级和提供公共卫生基础设施资源,以实施快速且具有成本效益的 GJB2-R143W 检测,随后进行适当的遗传和预期指导以进行医疗护理。

相似文献

1
Screening for GJB2-R143W-Associated Hearing Impairment: Implications for Health Policy and Practice in Ghana.GJB2-R143W 相关听力障碍的筛查:对加纳卫生政策和实践的影响。
Public Health Genomics. 2020;23(5-6):184-189. doi: 10.1159/000512121. Epub 2020 Dec 10.
2
Enhancing Genetic Medicine: Rapid and Cost-Effective Molecular Diagnosis for a Founder Mutation for Hearing Impairment in Ghana.增强遗传医学:在加纳快速且具有成本效益的听力障碍致病变异的分子诊断。
Genes (Basel). 2020 Jan 27;11(2):132. doi: 10.3390/genes11020132.
3
GJB2-associated hearing loss undetected by hearing screening of newborns.GJB2 相关听力损失在新生儿听力筛查中漏诊。
Gene. 2013 Dec 10;532(1):41-5. doi: 10.1016/j.gene.2013.08.094. Epub 2013 Sep 6.
4
[Non-invasive screening for GJB2 mutations in buccal smears for the diagnosis of inherited hearing impairment].[用于遗传性听力障碍诊断的口腔拭子中GJB2突变的无创筛查]
Laryngorhinootologie. 2003 Jun;82(6):397-401. doi: 10.1055/s-2003-40538.
5
and Mutations in Non-Syndromic Childhood Hearing Impairment in Ghana.加纳非综合征性儿童听力损失中的突变
Front Genet. 2019 Sep 18;10:841. doi: 10.3389/fgene.2019.00841. eCollection 2019.
6
Newborn hearing concurrent genetic screening for hearing impairment-a clinical practice in 58,397 neonates in Tianjin, China.新生儿听力与遗传性听力障碍联合筛查——中国天津58397例新生儿的临床实践
Int J Pediatr Otorhinolaryngol. 2013 Dec;77(12):1929-35. doi: 10.1016/j.ijporl.2013.08.038. Epub 2013 Sep 8.
7
Does universal newborn hearing screening identify all children with GJB2 (Connexin 26) deafness? Penetrance of GJB2 deafness.新生儿普遍听力筛查能识别出所有患有GJB2(连接蛋白26)耳聋的儿童吗?GJB2耳聋的外显率。
Ear Hear. 2006 Dec;27(6):732-41. doi: 10.1097/01.aud.0000240492.78561.d3.
8
Pattern of connexin 26 (GJB2) mutations causing sensorineural hearing impairment in Ghana.加纳导致感音神经性听力障碍的连接蛋白26(GJB2)突变模式。
Hum Mutat. 2001;18(1):84-5. doi: 10.1002/humu.1156.
9
Public Health Burden of Hearing Impairment and the Promise of Genomics and Environmental Research: A Case Study in Ghana, Africa.听力障碍的公共卫生负担与基因组学和环境研究的前景:以非洲加纳为例。
OMICS. 2017 Nov;21(11):638-646. doi: 10.1089/omi.2017.0145.
10
Structural basis for pathogenic variants of GJB2 and hearing levels of patients with hearing loss.GJB2 致病性变异体的结构基础及耳聋患者的听力水平。
BMC Res Notes. 2024 May 10;17(1):131. doi: 10.1186/s13104-024-06793-w.

引用本文的文献

1
Science and Society: Pathways to Equitable Access and Delivery of Genomics Medicine in Africa.科学与社会:非洲实现基因组医学公平获取与提供的途径
Curr Genet Med Rep. 2025;13(1):1. doi: 10.1007/s40142-024-00211-0. Epub 2025 Feb 24.
2
The Otoacoustic Emissions in the Universal Neonatal Hearing Screening: A Scoping Review Update on the African Data (2004 to 2024).普遍新生儿听力筛查中的耳声发射:非洲数据(2004年至2024年)的范围综述更新
Children (Basel). 2025 Jan 27;12(2):141. doi: 10.3390/children12020141.
3
Is a Major Cause of Non-Syndromic Hearing Impairment in Senegal.
是塞内加尔非综合征性听力障碍的主要原因。
Biology (Basel). 2022 May 23;11(5):795. doi: 10.3390/biology11050795.