Lu Jiali, Chen Jiangrong, Mei Li, Zhao Jingyu, Wang Changhong, Ma Cuihong, Luan Shanshan, Wan Yang
Prenatal Diagnosis Center (Department of Obstetrics), Fuyang People's Hospital, Fuyang, 236001, Anhui, China.
Clin Lab, BGI Genomics, Shanghai, 201321, China.
Arch Gynecol Obstet. 2025 Jun 24. doi: 10.1007/s00404-025-08100-9.
Expanded carrier screening (ECS) enables proactive identification of at-risk couples (ARCs) and individuals, facilitating informed reproductive decision-making through genetic counseling. This study evaluates the clinical utility of ECS among the population in Anhui Province, China, where its implementation remains understudied. Retrospectively analysis of genetic testing results assessed the carrier frequencies for targeted diseases, identified prevalent pathogenic genes and variants, and ARCs detection rate alongside associated reproductive choices and pregnancy outcomes.
In this single-center retrospective study (June 2020-October 2023), 2,530 reproductive-aged individuals (486 couples; 1,558 individuals) underwent next-generation sequencing (NGS)-based ECS using a customized panel targeting 152 recessive monogenic disorders. Carrier rates, pathogenic variants, ARC detection, and subsequent reproductive outcomes were analyzed.
Overall, 38.50% (974/2,530) of participants carried ≥ 1 pathogenic/likely pathogenic (P/LP) variant. The most prevalent autosomal recessive (AR) disorders included DFNB4 (3.08%), DFNB1A (2.81%), Wilson disease (2.57%), Krabbe disease (2.37%), and phenylketonuria (2.13%). Duchenne muscular dystrophy (DMD, 0.28%) was the most common X-linked (XL) disorder. Twenty ARCs (4.12%, 20/486) were identified, including sixteen pregnant couples. Among these, 56.25% (9/16) opted for invasive prenatal diagnosis, confirming eight unaffected fetuses with healthy live births and one twin pregnancy requiring selective termination of an affected fetus. Five pregnant ARCs declined prenatal diagnosis, four of whom delivered healthy infants, while one pregnancy was terminated due to structural anomalies. Of three non-pregnant ARCs, two pursued preimplantation genetic testing for monogenic disorders (PGT-M), resulting in one healthy birth.
Our study demonstrated that the ECS for reproductive-age individuals can identify couples and individuals at risk of conceiving a child with a recessive genetic disorder and support reproductive choices through the provision of genetic counseling to reduce the likelihood of offspring with congenital anomalies.
扩展携带者筛查(ECS)能够主动识别高危夫妇(ARCs)和个体,通过遗传咨询促进知情的生殖决策。本研究评估了ECS在中国安徽省人群中的临床效用,该地区其实施情况仍未得到充分研究。对基因检测结果进行回顾性分析,评估了目标疾病的携带者频率,确定了常见的致病基因和变异,以及ARCs的检出率,以及相关的生殖选择和妊娠结局。
在这项单中心回顾性研究(2020年6月至2023年10月)中,2530名育龄个体(486对夫妇;1558名个体)接受了基于二代测序(NGS)的ECS,使用定制的检测板针对152种隐性单基因疾病。分析了携带者率、致病变异、ARCs检测情况以及随后的生殖结局。
总体而言,38.50%(974/2530)的参与者携带≥1种致病/可能致病(P/LP)变异。最常见的常染色体隐性(AR)疾病包括DFNB4(3.08%)、DFNB1A(2.81%)、威尔逊病(2.57%)、克拉伯病(2.37%)和苯丙酮尿症(2.13%)。杜氏肌营养不良症(DMD,0.28%)是最常见的X连锁(XL)疾病。识别出20对ARCs(4.12%,20/486),包括16对怀孕夫妇。其中,56.25%(9/16)选择了侵入性产前诊断,确认8名胎儿未受影响并健康出生,1例双胎妊娠需要选择性终止1例受影响胎儿。5对怀孕的ARCs拒绝了产前诊断,其中4例分娩出健康婴儿,1例妊娠因结构异常而终止。在3对未怀孕的ARCs中,2对进行了单基因疾病植入前基因检测(PGT-M),1例健康出生。
我们的研究表明,针对育龄个体的ECS能够识别有生育患有隐性遗传疾病孩子风险的夫妇和个体,并通过提供遗传咨询来支持生殖选择,以降低后代出现先天性异常的可能性。