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invdup(8)(8q24.13q24.3)-一种复杂的改变及其临床后果。

invdup(8)(8q24.13q24.3)-A Complex Alteration and Its Clinical Consequences.

机构信息

Post-Graduate Program in Pathology, Universidade Federal de Ciências da Saúde de Porto Alegre (UFCSPA), Porto Alegre 90050-170, RS, Brazil.

Medical Genetics Resident, Irmandade da Santa Casa de Misericórdia de Porto Alegre (ISCMPA), Porto Alegre 90020-090, RS, Brazil.

出版信息

Genes (Basel). 2024 Jul 12;15(7):910. doi: 10.3390/genes15070910.

Abstract

Structural variation is a source of genetic variation that, in some cases, may trigger pathogenicity. Here, we describe two cases, a mother and son, with the same partial inverted duplication of the long arm of chromosome 8 [invdup(8)(q24.21q24.21)] of 17.18 Mb, showing different clinical manifestations: microcephaly, dorsal hypertrichosis, seizures and neuropsychomotor development delay in the child, and a cleft lip/palate, down-slanted palpebral fissures and learning disabilities in the mother. The deleterious outcome, in general, is reflected by the gain or loss of genetic material. However, discrepancies among the clinical manifestations raise some concerns about the genomic configuration within the chromosome and other genetic modifiers. With that in mind, we also performed a literature review of research published in the last 20 years about the duplication of the same, or close, chromosome region, seeking the elucidation of at least some relevant clinical features.

摘要

结构变异是遗传变异的来源之一,在某些情况下,可能会引发致病性。在这里,我们描述了两个病例,一个母亲和一个儿子,他们都携带 8 号染色体长臂[invdup(8)(q24.21q24.21)]的 17.18 Mb 部分倒位重复,表现出不同的临床表现:孩子表现为小头畸形、背部多毛症、癫痫和神经精神运动发育迟缓,母亲则表现为唇裂/腭裂、眼睑下斜和学习障碍。有害的结果通常反映在遗传物质的获得或损失上。然而,临床表现的差异引起了人们对染色体内基因组结构和其他遗传修饰物的关注。考虑到这一点,我们还对过去 20 年发表的关于同一或相近染色体区域重复的研究进行了文献回顾,以阐明至少一些相关的临床特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dfc9/11276216/ca7e9bf804b4/genes-15-00910-g001.jpg

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