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孕早期干血样中CFTR基因DeltaF508突变检测:一种囊性纤维化可能的常规产前筛查策略?

CFTR DeltaF508 mutation detection from dried blood samples in the first trimester of pregnancy: a possible routine prenatal screening strategy for cystic fibrosis?

作者信息

Konialis Christopher P, Hagnefelt Birgitta, Kazamia Constantina, Karapanou Sophia, Pangalos Constantinos

机构信息

Department of Molecular Genetics, Diagnostic Genetic Center, Athens, Greece.

出版信息

Fetal Diagn Ther. 2007;22(1):41-4. doi: 10.1159/000095842. Epub 2006 Sep 22.

Abstract

OBJECTIVE

The implementation and evaluation of a proposed wide-scale prenatal screening strategy, based on DNA isolated from dried blood spots in the first trimester of pregnancy, for the early detection of pregnancies at risk for cystic fibrosis (CF).

METHODS

The screening was performed in conjunction with routine biochemical marker screening for Down's syndrome risk in the first trimester of pregnancy. DNA was isolated from 1,233 dried blood spots and analyzed for the presence of the CF transmembrane regulator DeltaF508 mutation. Women carriers were offered and accepted the option for additional full testing of their partners in order to assess the risk for the fetus.

RESULTS

All 1,233 samples were successfully analyzed, identifying 23 DeltaF508 carriers, corresponding to a DeltaF508 carrier rate of approximately 1/55 (1.8%). All partners of the women carriers were further tested without revealing any need for further prenatal testing in this group.

CONCLUSIONS

This study reveals the relatively high frequency of the DeltaF508 CF mutation in the Greek population. More importantly, we demonstrate that the proposed prenatal screening strategy, based on the ease and cost-effectiveness of the analysis for the detection of a single common mutation, can be considered as a feasible and practical approach for wide-scale prenatal screening for CF, following the sequential model. It is applied early on in pregnancy, allowing for the timely management of families at risk for the corresponding genetic disorders. Finally, it can easily be extended to include screening for other common genetic disorders in specific population groups.

摘要

目的

基于妊娠早期干血斑中分离的DNA,实施并评估一项拟议的大规模产前筛查策略,用于早期检测有囊性纤维化(CF)风险的妊娠。

方法

该筛查与妊娠早期唐氏综合征风险的常规生化标志物筛查同时进行。从1233份干血斑中分离DNA,并分析CF跨膜调节因子DeltaF508突变的存在情况。向女性携带者提供并接受了对其伴侣进行额外全面检测的选择,以评估胎儿的风险。

结果

成功分析了所有1233份样本,鉴定出23名DeltaF508携带者,DeltaF508携带率约为1/55(1.8%)。女性携带者的所有伴侣均接受了进一步检测,该组中未发现有进一步产前检测的必要。

结论

本研究揭示了希腊人群中DeltaF508 CF突变的相对高频率。更重要的是,我们证明,基于检测单一常见突变的分析简便性和成本效益的拟议产前筛查策略,可被视为一种可行且实用的大规模CF产前筛查方法,遵循序贯模式。它在妊娠早期应用,可及时管理有相应遗传疾病风险的家庭。最后,它可以很容易地扩展到包括特定人群中其他常见遗传疾病的筛查。

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