Department of Pediatrics and Medicine, Columbia University, New York, New York, USA.
Department of Genetics, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
Am J Med Genet C Semin Med Genet. 2022 Jun;190(2):222-230. doi: 10.1002/ajmg.c.31988. Epub 2022 Jul 15.
In the US, newborn screening (NBS) is a unique health program that supports health equity and screens virtually every baby after birth, and has brought timely treatments to babies since the 1960's. With the decreasing cost of sequencing and the improving methods to interpret genetic data, there is an opportunity to add DNA sequencing as a screening method to facilitate the identification of babies with treatable conditions that cannot be identified in any other scalable way, including highly penetrant genetic neurodevelopmental disorders (NDD). However, the lack of effective dietary or drug-based treatments has made it nearly impossible to consider NDDs in the current NBS framework, yet it is anticipated that any treatment will be maximally effective if started early. Hence there is a critical need for large scale pilot studies to assess if and how NDDs can be effectively screened at birth, if parents desire that information, and what impact early diagnosis may have. Here we attempt to provide an overview of the recent advances in NDD treatments, explore the possible framework of setting up a pilot study to genetically screen for NDDs, highlight key technical, practical, and ethical considerations and challenges, and examine the policy and health system implications.
在美国,新生儿筛查(NBS)是一项独特的健康计划,支持健康公平,并在出生后几乎对每个婴儿进行筛查,自 20 世纪 60 年代以来为婴儿带来了及时的治疗。随着测序成本的降低和解释遗传数据方法的改进,有机会将 DNA 测序添加为一种筛查方法,以促进识别无法以任何其他可扩展方式识别的可治疗疾病的婴儿,包括高度外显的遗传神经发育障碍(NDD)。然而,缺乏有效的饮食或基于药物的治疗方法使得在当前 NBS 框架中几乎不可能考虑 NDD,尽管人们预计,如果及早开始治疗,任何治疗都将是最有效的。因此,迫切需要大规模试点研究来评估如果父母希望获得相关信息,以及早期诊断可能会产生何种影响,是否以及如何在出生时有效筛查 NDD。在这里,我们试图提供 NDD 治疗最新进展的概述,探讨为 NDD 进行基因筛查建立试点研究的可能框架,突出关键的技术、实际和伦理考虑因素和挑战,并研究政策和卫生系统的影响。