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一名12岁面部畸形和发育迟缓患者存在一种新的(父系遗传)13q31.3q32.3重复。

A novel (paternally inherited) duplication 13q31.3q32.3 in a 12-year-old patient with facial dysmorphism and developmental delay.

作者信息

Atack E, Fairtlough H, Smith K, Balasubramanian M

机构信息

Sheffield Diagnostic Genetics Service, Sheffield Children's NHS Foundation Trust, Sheffield, UK.

Sheffield Clinical Genetics Service, Sheffield Children's NHS Foundation Trust, Sheffield, UK.

出版信息

Mol Syndromol. 2014 Aug;5(5):245-50. doi: 10.1159/000358538. Epub 2014 Feb 19.

Abstract

We report a 12-year-old boy referred to the Clinical Genetics service in view of facial dysmorphism, learning difficulties and autistic spectrum disorder. 60K arrayCGH revealed an 8.2-Mb duplication on chromosome 13q31.3q32.3, which was paternally inherited. This specific duplication on chromosome 13 has not been previously reported in the medical literature, and there are no familial or de novo patients with the same duplication breakpoints. This region contains 24 OMIM genes, including the glypicans GPC5 and GPC6, and the ZIC2 gene. We discuss the relevance of this chromosome imbalance and discuss the impact of this duplication on our patient's phenotype. Given that the duplication on 13q was paternally inherited, and although initially thought to be of uncertain significance, on exploring the family history further, it became apparent that the father had learning difficulties as a child and previous surgery for congenital diaphragmatic hernia. Here we explore the phenotype in association with this novel duplication on chromosome 13q and add to the existing literature on array findings within this region.

摘要

我们报告了一名12岁男孩,因面部畸形、学习困难和自闭症谱系障碍被转诊至临床遗传学服务部门。60K阵列比较基因组杂交(arrayCGH)显示在染色体13q31.3q32.3上存在一个8.2兆碱基(Mb)的重复,该重复是父系遗传的。此前医学文献中尚未报道过染色体13上的这种特定重复,也没有具有相同重复断点的家族性或新发患者。该区域包含24个在线人类孟德尔遗传数据库(OMIM)基因,包括磷脂酰肌醇蛋白聚糖GPC5和GPC6以及ZIC2基因。我们讨论了这种染色体失衡的相关性,并探讨了这种重复对我们患者表型的影响。鉴于13q上的重复是父系遗传的,尽管最初认为其意义不确定,但在进一步探究家族病史时,发现父亲小时候有学习困难,并且曾接受过先天性膈疝手术。在此,我们探讨与染色体13q上这种新发现的重复相关的表型,并补充该区域内关于阵列检测结果的现有文献。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbc2/4188164/289ee73fe570/msy-0005-0245-g01.jpg

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