Center for Reproductive Medicine, Department of Obstetrics and Gynecology, Peking University Third Hospital, No. 49, North Garden Road, Haidian District, Beijing, 100191, China.
National Clinical Research Center for Obstetrics and Gynecology (Peking University Third Hospital), Beijing, 100191, China.
J Hum Genet. 2023 Dec;68(12):813-821. doi: 10.1038/s10038-023-01188-4. Epub 2023 Aug 18.
Hereditary tumor syndromes have garnered substantial attention due to their adverse effects on both the physical and psychological health of patients, as well as the elevated risk of transmission to subsequent generations. This has prompted a growing interest in exploring preimplantation genetic testing (PGT) as a treatment option to mitigate and eliminate these impacts. Several studies have demonstrated that de novo variants have become a great cause of many hereditary tumor syndromes, which introduce certain difficulties to PGT. In the absence of adequate genetic linkage information (parents and offspring), haplotype construction seems unrealizable. In the study, researchers used single sperm or affected embryos as proband to perform single-nucleotide polymorphism linkage analysis for cases with de novo variants. For complicated variants, the strategy that sperm combined with embryo detection will increase accuracy while avoiding the limitations and potential failures of using a single detection material. The study recruited 11 couples with male de novo carriers, including 3 tumor types and 4 genes. To date, 4 couples have been clinically confirmed as pregnant and three healthy babies have been born. The results of amniocentesis or umbilical cord blood verification were consistent with the results of PGT-M. The study aims to introduce the application of the PGT-M strategy in hereditary tumor syndromes.
遗传性肿瘤综合征因其对患者身心健康的不良影响以及向后代传递的风险增加而引起了广泛关注。这促使人们越来越关注探讨胚胎植入前遗传学检测(PGT)作为一种治疗选择,以减轻和消除这些影响。多项研究表明,新生变异已成为许多遗传性肿瘤综合征的重要原因,这给 PGT 带来了一定的困难。在缺乏充分的遗传连锁信息(父母和后代)的情况下,单倍型构建似乎难以实现。在这项研究中,研究人员使用单个精子或受影响的胚胎作为先证者,对具有新生变异的病例进行单核苷酸多态性连锁分析。对于复杂的变异,精子结合胚胎检测的策略将提高准确性,同时避免使用单一检测材料的局限性和潜在失败。该研究招募了 11 对携带男性新生变异的夫妇,包括 3 种肿瘤类型和 4 个基因。迄今为止,已有 4 对夫妇被临床证实怀孕,3 名健康婴儿已经出生。羊膜穿刺术或脐带血验证的结果与 PGT-M 的结果一致。本研究旨在介绍 PGT-M 策略在遗传性肿瘤综合征中的应用。