Wilhelm Kevin, Edick Mathew J, Berry Susan A, Hartnett Michael, Brower Amy
Newborn Screening Translational Research Network, American College of Medical Genetics and Genomics, Bethesda, MD, United States.
Graduate Program in Genetics and Genomics, Graduate School of Biological Sciences, Baylor College of Medicine, Houston, TX, United States.
Front Genet. 2022 May 26;13:859837. doi: 10.3389/fgene.2022.859837. eCollection 2022.
With the rapid increase in publicly available sequencing data, healthcare professionals are tasked with understanding how genetic variation informs diagnosis and affects patient health outcomes. Understanding the impact of a genetic variant in disease could be used to predict susceptibility/protection and to help build a personalized medicine profile. In the United States, over 3.8 million newborns are screened for several rare genetic diseases each year, and the follow-up testing of screen-positive newborns often involves sequencing and the identification of variants. This presents the opportunity to use longitudinal health information from these newborns to inform the impact of variants identified in the course of diagnosis. To test this, we performed secondary analysis of a 10-year natural history study of individuals diagnosed with metabolic disorders included in newborn screening (NBS). We found 564 genetic variants with accompanying phenotypic data and identified that 161 of the 564 variants (29%) were not included in ClinVar. We were able to classify 139 of the 161 variants (86%) as pathogenic or likely pathogenic. This work demonstrates that secondary analysis of longitudinal data collected as part of NBS finds unreported genetic variants and the accompanying clinical information can inform the relationship between genotype and phenotype.
随着公开可用测序数据的迅速增加,医疗保健专业人员面临着理解基因变异如何为诊断提供信息以及如何影响患者健康结果的任务。了解基因变异在疾病中的影响可用于预测易感性/保护性,并有助于建立个性化医疗档案。在美国,每年有超过380万新生儿接受几种罕见遗传疾病的筛查,对筛查呈阳性的新生儿进行的后续检测通常涉及测序和变异体的识别。这提供了利用这些新生儿的纵向健康信息来了解诊断过程中鉴定出的变异体影响的机会。为了对此进行测试,我们对一项针对新生儿筛查(NBS)中诊断出的患有代谢紊乱的个体进行的为期10年的自然史研究进行了二次分析。我们发现了564个伴有表型数据的基因变异体,并确定564个变异体中有161个(29%)未包含在ClinVar中。我们能够将161个变异体中的139个(86%)分类为致病或可能致病。这项工作表明,作为NBS一部分收集的纵向数据的二次分析发现了未报告的基因变异体,并且随附的临床信息可以为基因型和表型之间的关系提供信息。