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一种隐匿性平衡易位(5;17),这是通过对家系进行严格评估以及对由表型提示的候选基因座进行荧光原位杂交(FISH)而揭示出的谜题。

A cryptic balanced translocation (5;17), a puzzle revealed through a critical evaluation of the pedigree and a FISH focused on candidate loci suggested by the phenotype.

作者信息

Primerano A, Colao E, Villella C, Nocera M D, Ciambrone A, Luciano E, D'Antona L, Vismara M F M, Loddo S, Novelli A, Perrotti N, Malatesta Paola

机构信息

Unit of Medical Gentics, University Hospital Mater Domini, Catanzaro, Italy.

Department of "Scienze della Salute", Università UMG di Catanzaro, Catanzaro, Italy.

出版信息

Mol Cytogenet. 2015 Sep 2;8:70. doi: 10.1186/s13039-015-0172-1. eCollection 2015.

Abstract

We report a case of a woman with a cryptic balanced translocation between chromosomes 5 and 17, suspected during genetic counseling. The woman had a history of previous fetal losses attributed to lissencephaly and intra uterine growth retardation (IUGR) and a daughter with dysmorphic features and mental retardation, previously attributed to a small deletion 5pter, detected years ago by a first generation CGH-array. This peculiar combination of personal and family history suggested the opportunity to carry out a FISH approach, focusing on chromosomes 5 and 17, based on the idea that a malsegregation secondary to a balanced translocation, might have escaped the first CGH array. This approach allowed the identification of a balanced translocation in the mother, FISH in the affected child confirmed the partial 5p deletion predicted by the previous CGH array and identified a new 17p duplication that had not been detected before. The described translocation opens the possibility of alternative imbalances that were probably responsible for previous fetal losses. The imbalances were confirmed by a new high resolution SNP array. We conclude that despite the availability of highly effective and sensitive genomic approaches a careful evaluation of medical history is highly recommended since it can suggest clinical hypothesis that can be confirmed by more classical and molecular cytogenetic based approaches.

摘要

我们报告了一例女性病例,该女性在遗传咨询期间被怀疑存在5号和17号染色体之间的隐匿性平衡易位。该女性既往有因无脑回畸形和宫内生长迟缓(IUGR)导致的胎儿丢失史,还有一个女儿,有畸形特征和智力发育迟缓,多年前第一代比较基因组杂交阵列(CGH-array)检测认为这是由5号染色体短臂末端小缺失所致。个人和家族史的这种特殊组合表明,有必要基于这样一种想法进行荧光原位杂交(FISH)检测,即聚焦于5号和17号染色体,因为平衡易位继发的错误分离可能在首次CGH阵列检测中被遗漏。这种方法在母亲中鉴定出了平衡易位,对患病儿童进行的FISH检测证实了先前CGH阵列预测的5号染色体短臂部分缺失,并发现了一个之前未检测到的17号染色体短臂重复。所描述的易位揭示了可能是先前胎儿丢失原因的其他不平衡情况的可能性。这些不平衡情况通过新的高分辨率单核苷酸多态性(SNP)阵列得到了证实。我们得出结论,尽管有高效且灵敏的基因组学方法,但仍强烈建议仔细评估病史,因为它可以提出一些临床假设,而这些假设可以通过更经典的基于分子细胞遗传学的方法得到证实。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/95f1/4557763/2f2f51b87665/13039_2015_172_Fig1_HTML.jpg

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