Suppr超能文献

2023年全球脊髓性肌萎缩症新生儿筛查项目

Newborn screening programs for spinal muscular atrophy worldwide in 2023.

作者信息

Vrščaj Eva, Dangouloff Tamara, Osredkar Damjan, Servais Laurent

机构信息

Department of Pediatric Neurology, University Children's Hospital, University Medical Centre Ljubljana, Slovenia.

Division of Child Neurology, Reference Center for Neuromuscular Diseases, Department of Pediatrics, University Hospital Liège & University of Liège, Belgium.

出版信息

J Neuromuscul Dis. 2024 Nov;11(6):1180-1189. doi: 10.1177/22143602241288095.

Abstract

BACKGROUND

Spinal muscular atrophy is a rare, genetic neuromuscular disorder. Disease-modifying therapies, when administered early, have shown improved outcomes, leading to the implementation of numerous newborn screening programs for spinal muscular atrophy.

OBJECTIVE

The aim of this study was to evaluate the progress in implementing newborn screening for spinal muscular atrophy and therapy accessibility worldwide, after the first paper published in 2021.

METHODS

We conducted a survey, contacted experts from 143 countries worldwide, gathered responses from 86 experts from 80 countries.

RESULTS

By 2023, 31 countries reported established programs, 33 in the beginning of the year 2024; identifying approximately 1176 cases of spinal muscular atrophy. Additionally, the availability of disease-modifying therapies has expanded. At least one therapy is now accessible in 62 countries. Challenges, such as lack of governmental support, resource constraints, and varying healthcare priorities continue to impede implementation in some countries.

CONCLUSIONS

The data shows a significant increase in the implementation of newborn screening programs since 2021. The experts are still expressing a strong need for equitable access to standard of care for all the patients globally. Despite all setbacks, collaborative efforts have played a crucial role in newborn screening for spinal muscular atrophy implementation and currently 7% of world newborns are being screened, projections indicate an estimated 18% screening rate by 2028.

摘要

背景

脊髓性肌萎缩症是一种罕见的遗传性神经肌肉疾病。早期使用的疾病改善疗法已显示出更好的治疗效果,这促使许多国家实施了针对脊髓性肌萎缩症的新生儿筛查项目。

目的

本研究旨在评估自2021年发表首篇论文以来,全球范围内脊髓性肌萎缩症新生儿筛查的实施进展以及治疗的可及性。

方法

我们开展了一项调查,联系了全球143个国家的专家,收到了来自80个国家的86位专家的回复。

结果

截至2023年,31个国家报告已建立筛查项目,2024年初有33个国家建立;共确诊约1176例脊髓性肌萎缩症病例。此外,疾病改善疗法的可及性有所扩大。目前至少有一种疗法在62个国家可及。一些国家仍面临政府支持不足、资源受限以及医疗保健重点不同等挑战,这些阻碍了筛查项目的实施。

结论

数据显示自2021年以来,新生儿筛查项目的实施有显著增加。专家们仍强烈表示需要让全球所有患者都能公平获得标准治疗。尽管面临种种挫折,但合作努力在脊髓性肌萎缩症新生儿筛查实施过程中发挥了关键作用,目前全球7%的新生儿正在接受筛查,预计到2028年筛查率将达到18%。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验