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病例报告:一种罕见的染色体失衡,由父母的臂间倒位导致,存在7号染色体长臂36.3区至末端重复及7号染色体短臂末端至22.3区缺失。

Case Report: A rare chromosomal imbalance with dup 7q36.3-qter and del 7pter-p22.3 arising from parental pericentric inversion.

作者信息

Lin Rongbo, Zhang Wenhui, Huang Mingwei, Shen Yansheng, Liao Jianxiang, Song Ping, Qi Ying, He Jie, Xia Yuanxiang, Duan Jing, Ye Yuanzhen, Yi Qiuwei, Lan Pei, Kong Lingyu, Hu Zhanqi

机构信息

Department of Neurology, Shenzhen Children's Hospital, Shenzhen, China.

Department of Emergency, Shenzhen Children's Hospital, Shenzhen, China.

出版信息

Front Genet. 2025 Jul 17;16:1564711. doi: 10.3389/fgene.2025.1564711. eCollection 2025.

DOI:10.3389/fgene.2025.1564711
PMID:40747102
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12310479/
Abstract

Chromosomal abnormality is a significant cause of neurodevelopmental delay and congenital malformation. Only a few cases of chromosome 7 imbalances with both duplication of the distal long arm (7q) and deletion of the distal short arm (7p) have been reported without a systematic analysis of the genotype-phenotype relationship. We identify a new case of chromosome 7 imbalance with dup 7q36.3-qter and del 7pter-p22.3 and thoroughly characterize the chromosomal abnormality in the patient and related family members using a variety of genetic tests. More importantly, similar cases of 7q duplication and 7p deletion arising from parental pericentric inversion are reviewed to clarify the genotype-phenotype correlation of the disease. In summary, in cases of normal prenatal and early postnatal growth, progressive neurodevelopmental delay, intellectual disability, limited speech, and mild facial dysmorphism, the rare combination of duplication and deletion of distal ends of chromosome 7 may be suspected. Parental pericentric chromosomal inversion is likely a genetic contributor to the duplication-deletion imbalance in the offspring despite normal phenotypes in the inversion carrier, so genetic testing and counseling are recommended for better disease management and prevention.

摘要

染色体异常是神经发育迟缓及先天性畸形的一个重要原因。仅有少数远端长臂(7q)重复且远端短臂(7p)缺失的7号染色体失衡病例被报道,且未对基因型-表型关系进行系统分析。我们鉴定出1例新的7号染色体失衡病例,其7q36.3-qter重复且7pter-p22.3缺失,并使用多种基因检测方法对该患者及相关家庭成员的染色体异常进行了全面表征。更重要的是,对因亲代臂间倒位导致的7q重复和7p缺失的类似病例进行了回顾,以阐明该疾病的基因型-表型相关性。总之,在产前及出生后早期生长正常,但出现进行性神经发育迟缓、智力残疾、言语受限及轻度面部畸形的病例中,可能怀疑存在7号染色体远端重复和缺失的罕见组合。尽管倒位携带者的表型正常,但亲代臂间染色体倒位可能是后代重复-缺失失衡的一个遗传因素,因此建议进行基因检测和遗传咨询,以更好地管理和预防该疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/82dd/12310479/4b49efbaec2e/fgene-16-1564711-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/82dd/12310479/25bd56df7078/fgene-16-1564711-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/82dd/12310479/d1e24a1da31f/fgene-16-1564711-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/82dd/12310479/4b49efbaec2e/fgene-16-1564711-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/82dd/12310479/25bd56df7078/fgene-16-1564711-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/82dd/12310479/d1e24a1da31f/fgene-16-1564711-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/82dd/12310479/4b49efbaec2e/fgene-16-1564711-g003.jpg

相似文献

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本文引用的文献

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7p22.3 microdeletion: a case study of a patient with congenital heart defect, neurodevelopmental delay and epilepsy.7p22.3 微缺失:一名伴有先天性心脏缺陷、神经发育迟缓及癫痫患者的病例研究。
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Optical genome mapping enables constitutional chromosomal aberration detection.光学基因组图谱技术可用于检测染色体结构异常。
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Variants in PRKAR1B cause a neurodevelopmental disorder with autism spectrum disorder, apraxia, and insensitivity to pain.PRKAR1B 变异导致一种神经发育障碍,伴有自闭症谱系障碍、失用症和对疼痛不敏感。
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Prenatal detection of a 7q11.21 microdeletion (517-605 kb): A variant with normal characteristics at birth (STROBE).产前检测到 7q11.21 微缺失(517-605kb):出生时具有正常特征的变异体(STROBE)。
Medicine (Baltimore). 2021 Feb 12;100(6):e24560. doi: 10.1097/MD.0000000000024560.
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Recombinant Chromosomes Resulting From Parental Pericentric Inversions-Two New Cases and a Review of the Literature.源于亲代臂间倒位的重组染色体——两例新病例及文献综述
Front Genet. 2019 Nov 14;10:1165. doi: 10.3389/fgene.2019.01165. eCollection 2019.
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Small 7p22.3 microdeletion: Case report of Snx8 haploinsufficiency and neurological findings.7p22.3小片段微缺失:Snx8单倍体不足及神经学表现的病例报告
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A Chromosome 7 Pericentric Inversion Defined at Single-Nucleotide Resolution Using Diagnostic Whole Genome Sequencing in a Patient with Hand-Foot-Genital Syndrome.利用诊断性全基因组测序在一名患有手足生殖器综合征的患者中以单核苷酸分辨率定义的7号染色体臂间倒位。
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