新生儿基因组筛查(NBS)的前景与挑战--公共卫生 NBS 项目的经验教训。

IF 3.1 3区 医学 Q1 PEDIATRICS
Mari Mori, Bimal P Chaudhari, Margie A Ream, Alex R Kemper
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引用次数: 0

摘要

美国的新生儿筛查(NBS)始于 20 世纪 60 年代,目的是检测先天性代谢错误,与出现症状和确诊后的治疗相比,先天性代谢错误可从症状前的治疗中获益。随着时间的推移,它扩展到包括内分泌失调、血液病、免疫缺陷和其他可治疗的疾病,如溶酶体贮积症(LSD)、囊性纤维化、X 连锁肾上腺白质营养不良症和脊髓性肌萎缩症。新技术(如串联质谱法)和新疗法(如酶替代疗法和干细胞移植治疗溶酶体储积症)推动了这一领域的发展。下一代基因测序(NGS)技术的进步使我们能够快速识别更多可能受益于早期症状前干预的疾病。我们回顾了作为诊断测试发展起来的 NGS 技术,并提出了在可能应用于筛查无症状人群之前需要解决的问题。我们说明了选择要筛查的疾病的重要性,并提出了在发现意义不确定的变异时应遵循的建议。我们讨论了有关实现基因组 NBS 敏感性公平性、获得随访和管理(尤其是来自不同背景的人群)的伦理问题以及其他考虑因素。最后,我们讨论了基因组 NBS 对单基因疾病患儿整体健康的潜在利弊。影响:基因组新生儿筛查计划正在全球范围内开展。公众需要讨论是否应将新生儿基因组筛查作为一项公共卫生计划,如果是,应筛查哪些疾病。医疗服务提供者应了解,基因组新生儿筛查对来自非欧洲人群的新生儿的敏感性特别低。甲基化、大型结构变异和重复扩增变异不适合基于新一代测序的新生儿基因组筛查。这篇文章为了解基因组新生儿筛查作为公共卫生项目实施前需要解决的问题提供了全面指导。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Promises and challenges of genomic newborn screening (NBS) - lessons from public health NBS programs.

Newborn screening (NBS) in the United States began in the 1960s to detect inborn errors of metabolism that benefited from presymptomatic treatment compared with treatment after the development of symptoms and diagnosis. Over time, it expanded to include endocrinological disorders, hematological disorders, immunodeficiencies, and other treatable diseases such as lysosomal storage diseases (LSD), cystic fibrosis, X-linked adrenoleukodystrophy, and spinal muscular dystrophy. This expansion has been driven by new technologies (e.g., tandem mass spectrometry) and novel treatments (e.g., enzyme replacement therapy and stem cell transplant for LSDs). Advances in next-generation gene sequencing (NGS) enable rapid identification of many additional conditions that might benefit from early presymptomatic intervention. We review the NGS technologies that evolved as diagnostic testing and suggest issues to be resolved before their potential application to screening the asymptomatic population. We illustrate the importance of selecting diseases to be screened and propose recommendations to follow when variants of uncertain significance are found. We address ethical issues around achieving equity in the sensitivity of genomic NBS, access to follow-up and management, especially for people from diverse backgrounds, and other considerations. Finally, we discuss the potential benefits and harms of genomic NBS to the overall health of children with monogenic diseases. IMPACT: Genomic newborn screening programs are ongoing worldwide. Public discussion is needed as to whether genomic newborn screening should be offered as a public health program and, if so, what conditions should be screened for. Providers should understand that the sensitivity of genomic newborn screening is especially low for newborns from non-European populations. Methylation, large structural variants and repeat expansion variants are not amenable to next-generation sequencing-based genomic newborn screening. The article serves as a comprehensive guide to understanding issues that need to be solved before genomic newborn screening is implemented as a public health program.

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来源期刊
Pediatric Research
Pediatric Research 医学-小儿科
CiteScore
6.80
自引率
5.60%
发文量
473
审稿时长
3-8 weeks
期刊介绍: Pediatric Research publishes original papers, invited reviews, and commentaries on the etiologies of children''s diseases and disorders of development, extending from molecular biology to epidemiology. Use of model organisms and in vitro techniques relevant to developmental biology and medicine are acceptable, as are translational human studies
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