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比利时微阵列产前 (BEMAPRE) 数据库:一个系统的全国性胎儿基因组异常存储库。

The Belgian MicroArray Prenatal (BEMAPRE) database: A systematic nationwide repository of fetal genomic aberrations.

机构信息

Department of Obstetrics and Gynaecology, University Hospital Antwerp, Edegem, Belgium.

Center for Medical Genetics, Universiteit Antwerpen, Antwerp, Belgium.

出版信息

Prenat Diagn. 2018 Dec;38(13):1120-1128. doi: 10.1002/pd.5373. Epub 2018 Nov 14.

Abstract

OBJECTIVE

With the replacement of karyotyping by chromosomal microarray (CMA) in invasive prenatal diagnosis, new challenges have arisen. By building a national database, we standardize the classification and reporting of prenatally detected copy number variants (CNVs) across Belgian genetic centers. This database, which will link genetic and ultrasound findings with postnatal development, forms a unique resource to investigate the pathogenicity of variants of uncertain significance and to refine the phenotypic spectrum of pathogenic and susceptibility CNVs.

METHODS

The Belgian MicroArray Prenatal (BEMAPRE) consortium is a collaboration of all genetic centers in Belgium. We collected data from all invasive prenatal procedures performed between May 2013 and July 2016.

RESULTS

In this three-year period, 13 266 prenatal CMAs were performed. By national agreement, a limited number of susceptibility CNVs and no variants of uncertain significance were reported. Added values for using CMA versus conventional karyotyping were 1.8% in the general invasive population and 2.7% in cases with an ultrasound anomaly. Of the reported CNVs, 31.5% would have remained undetected with non-invasive prenatal test as the first-tier test.

CONCLUSION

The establishment of a national database for prenatal CNV data allows for a uniform reporting policy and the investigation of the prenatal and postnatal genotype-phenotype correlation.

摘要

目的

随着染色体微阵列(CMA)在侵袭性产前诊断中的应用取代核型分析,出现了新的挑战。通过建立一个全国性数据库,我们规范了比利时各遗传中心产前检测到的拷贝数变异(CNVs)的分类和报告。该数据库将遗传和超声发现与产后发育联系起来,形成了一个独特的资源,用于研究具有不确定意义的变异的致病性,并细化致病性和易感性 CNVs 的表型谱。

方法

比利时微阵列产前(BEMAPRE)联盟是比利时所有遗传中心的合作组织。我们收集了 2013 年 5 月至 2016 年 7 月期间所有侵袭性产前程序的数据。

结果

在这三年期间,共进行了 13266 例产前 CMA。根据国家协议,只报告了少数易感性 CNVs 和无意义的变异。与传统核型分析相比,CMA 的附加价值在普通侵袭性人群中为 1.8%,在超声异常病例中为 2.7%。在所报告的 CNVs 中,31.5%如果作为一线测试使用非侵入性产前测试,将无法检测到。

结论

建立产前 CNV 数据的全国性数据库,可实现统一的报告政策,并可进行产前和产后基因型-表型相关性的研究。

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