Department of Obstetrics and Gynaecology, Division Woman and Child, University Hospitals Leuven, Leuven, Belgium.
Faculty of Medicine, KU Leuven, Leuven, Belgium.
Eur J Hum Genet. 2022 Dec;30(12):1323-1330. doi: 10.1038/s41431-022-01147-1. Epub 2022 Jul 27.
Non-invasive prenatal testing has been introduced for the detection of Trisomy 13, 18, and 21. Using genome-wide screening also other "rare" autosomal trisomies (RATs) can be detected with a frequency about half the frequency of the common trisomies in the large population-based studies. Large prospective studies and clear clinical guidelines are lacking to provide adequate counseling and management to those who are confronted with a RAT as a healthcare professional or patient. In this review we reviewed the current knowledge of the most common RATs. We compiled clinical relevant parameters such as incidence, meiotic or mitotic origin, the risk of fetal (mosaic) aneuploidy, clinical manifestations of fetal mosaicism for a RAT, the effect of confined placental mosaicism on placental function and the risk of uniparental disomy (UPD). Finally, we identified gaps in the knowledge on RATs and highlight areas of future research. This overview may serve as a first guide for prenatal management for each of these RATs.
非侵入性产前检测已被用于检测三体 13、18 和 21。使用全基因组筛查,也可以检测到其他“罕见”常染色体三体(RAT),其频率约为基于人群的大型研究中常见三体的频率的一半。缺乏大型前瞻性研究和明确的临床指南,为那些作为医疗保健专业人员或患者面对 RAT 的人提供充分的咨询和管理。在这篇综述中,我们回顾了最常见的 RAT 的现有知识。我们汇编了与临床相关的参数,如发病率、减数分裂或有丝分裂起源、胎儿(嵌合体)非整倍体的风险、RAT 胎儿嵌合体的临床表现、局限胎盘嵌合体对胎盘功能的影响以及单亲二体性(UPD)的风险。最后,我们确定了 RAT 知识中的空白,并强调了未来研究的领域。本综述可作为针对每种 RAT 的产前管理的初步指南。