4q 缺失和 3q 重复婴儿的眼部和全身异常:病例报告与文献综述

IF 1 4区 医学 Q4 OPHTHALMOLOGY Journal of Pediatric Ophthalmology & Strabismus Pub Date : 2024-11-01 DOI:10.3928/01913913-20240911-02
Nicholas Householder, Sawyer Vaclaw, Misty Ruppert, Santosh Chavali, Vijay Tonk, Temiloluwa Abikoye
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引用次数: 0

摘要

本病例报告了一名患有两种罕见致病性染色体异常的女婴:3 号染色体部分三体(3q25.2 至 3q29)和 4 号染色体部分单体(4q34.1 至 4q35.2)。通过全面评估和文献查阅,发现该患者的眼部和全身畸形与这两种染色体畸变均无关联。为确认染色体异常,进行了比较基因组杂交。研究人员还进行了全面的文献检索,以便将研究结果与现有的类似染色体畸变知识联系起来。遗传分析显示,3号染色体和4号染色体长臂之间存在不平衡易位,导致3号染色体部分三体(3q25.2至3q29)和4号染色体部分单体(4q34.1至4q35.2)。眼部异常包括眼球震颤、上睑下垂、眼睑内翻、巨角膜、角膜混浊、小儿青光眼、高度近视和视网膜色素沉着。在治疗先天性畸形的过程中,患者将接受双侧下眼睑旋转手术和球镜辅助的腔内小梁切除术。观察到的明显眼部异常代表了三体部分三联症和四体部分单体症在特定断点上的新特征。文献检索揭示了之前记录的与基因易位相关的特征,并证实了我们的患者表型所特有的各种新特征。进一步的研究对于阐明这些眼部表现的遗传机制及其对病例临床治疗的影响至关重要。[2024;61(6):e66-e74.].
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Ocular and Systemic Anomalies in an Infant With 4q Deletion and 3q Duplication: Case Report and Review of Literature.

This is a case report of a female infant with two rare pathogenic chromosomal abnormalities: partial trisomy of chromosome 3 (3q25.2 to 3q29) and partial monosomy of chromosome 4 (4q34.1 to 4q35.2). A comprehensive evaluation and literature review revealed ocular and systemic malformations not previously described in association with either chromosomal aberration. Comparative genomic hybridization was performed to confirm the chromosomal abnormalities. A thorough literature search was conducted to contextualize the findings within existing knowledge of similar chromosomal aberrations. Genetic analysis revealed an unbalanced translocation between the long arms of chromosomes 3 and 4, resulting in partial trisomy of chromosome 3 (3q25.2 to 3q29) and partial monosomy of chromosome 4 (4q34.1 to 4q35.2). Ocular anomalies included nystagmus, epicanthus, lagophthalmos, entropion, megalocornea, corneal clouding, infantile glaucoma, high myopia, and retinal hyperpigmentation. The patient would undergo bilateral lower eyelid rotation and gonioscopy-assisted transluminal trabeculectomy procedures in the management of her congenital malformations. The distinct ophthalmic abnormalities observed represent novel features within the spectrum of partial trisomy three and partial monosomy four at the specified breakpoints. The literature search unveiled the previously documented features associated with genetic translocation and confirmed various novel features specific to our patient's phenotype. Further research will be essential to elucidate the genetic mechanisms underlying these ocular manifestations and their implications for the clinical management of cases. [J Pediatr Ophthalmol Strabismus. 2024;61(6):e66-e74.].

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来源期刊
CiteScore
1.80
自引率
8.30%
发文量
115
审稿时长
>12 weeks
期刊介绍: The Journal of Pediatric Ophthalmology & Strabismus is a bimonthly peer-reviewed publication for pediatric ophthalmologists. The Journal has published original articles on the diagnosis, treatment, and prevention of eye disorders in the pediatric age group and the treatment of strabismus in all age groups for over 50 years.
期刊最新文献
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