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Case Report: Identification of a de novo Microdeletion 1q44 in a Patient With Seizures and Developmental Delay

Objective: 1q44 microdeletion syndrome is difficult to diagnose due to the wide phenotypic spectrum and strong genetic heterogeneity. We explore the correlation between the chromosome microdeletions and phenotype in a child with 1q44 microdeletion syndrome, we collected the clinical features of the...

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Autores principales: Tung, Yiehen, Lu, Haiying, Lin, Wenxin, Huang, Tingting, Kim, Samuel, Hu, Guo, Zhang, Gang, Zheng, Guo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8173053/
https://www.ncbi.nlm.nih.gov/pubmed/34093647
http://dx.doi.org/10.3389/fgene.2021.648351
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author Tung, Yiehen
Lu, Haiying
Lin, Wenxin
Huang, Tingting
Kim, Samuel
Hu, Guo
Zhang, Gang
Zheng, Guo
author_facet Tung, Yiehen
Lu, Haiying
Lin, Wenxin
Huang, Tingting
Kim, Samuel
Hu, Guo
Zhang, Gang
Zheng, Guo
author_sort Tung, Yiehen
collection PubMed
description Objective: 1q44 microdeletion syndrome is difficult to diagnose due to the wide phenotypic spectrum and strong genetic heterogeneity. We explore the correlation between the chromosome microdeletions and phenotype in a child with 1q44 microdeletion syndrome, we collected the clinical features of the patient and combined them with adjacent copy number variation (CNV) regions previously reported. Methods: We collected the full medical history of the patient and summarized her clinical symptoms. Whole-exome sequencing (WES) and CapCNV analysis were performed with DNA extracted from both the patient's and her parents' peripheral blood samples. Fluorescent quantitative PCR (q-PCR) was performed for the use of verification to the CNV regions. Results: A 28.7 KB microdeletion was detected in the 1q44 region by whole-exome sequencing and low-depth whole-genome sequencing. The deleted region included the genes COX20 and HNRNPU. As verification, karyotype analysis showed no abnormality, and the results of qPCR were consistent with that of whole-exome sequencing and CapCNV analysis. Conclusion: The patient was diagnosed with 1q44 microdeletion syndrome with clinical and genetic analysis. Analyzing both whole-exome sequencing and CapCNV analysis can not only improve the diagnostic rate of clinically suspected syndromes that present with intellectual disability (ID) and multiple malformations but also support further study of the correlation between CNVs and clinical phenotypes. This study lays the foundation for the further study of the pathogenesis of complex diseases.
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spelling pubmed-81730532021-06-04 Case Report: Identification of a de novo Microdeletion 1q44 in a Patient With Seizures and Developmental Delay Tung, Yiehen Lu, Haiying Lin, Wenxin Huang, Tingting Kim, Samuel Hu, Guo Zhang, Gang Zheng, Guo Front Genet Genetics Objective: 1q44 microdeletion syndrome is difficult to diagnose due to the wide phenotypic spectrum and strong genetic heterogeneity. We explore the correlation between the chromosome microdeletions and phenotype in a child with 1q44 microdeletion syndrome, we collected the clinical features of the patient and combined them with adjacent copy number variation (CNV) regions previously reported. Methods: We collected the full medical history of the patient and summarized her clinical symptoms. Whole-exome sequencing (WES) and CapCNV analysis were performed with DNA extracted from both the patient's and her parents' peripheral blood samples. Fluorescent quantitative PCR (q-PCR) was performed for the use of verification to the CNV regions. Results: A 28.7 KB microdeletion was detected in the 1q44 region by whole-exome sequencing and low-depth whole-genome sequencing. The deleted region included the genes COX20 and HNRNPU. As verification, karyotype analysis showed no abnormality, and the results of qPCR were consistent with that of whole-exome sequencing and CapCNV analysis. Conclusion: The patient was diagnosed with 1q44 microdeletion syndrome with clinical and genetic analysis. Analyzing both whole-exome sequencing and CapCNV analysis can not only improve the diagnostic rate of clinically suspected syndromes that present with intellectual disability (ID) and multiple malformations but also support further study of the correlation between CNVs and clinical phenotypes. This study lays the foundation for the further study of the pathogenesis of complex diseases. Frontiers Media S.A. 2021-05-20 /pmc/articles/PMC8173053/ /pubmed/34093647 http://dx.doi.org/10.3389/fgene.2021.648351 Text en Copyright © 2021 Tung, Lu, Lin, Huang, Kim, Hu, Zhang and Zheng. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Genetics
Tung, Yiehen
Lu, Haiying
Lin, Wenxin
Huang, Tingting
Kim, Samuel
Hu, Guo
Zhang, Gang
Zheng, Guo
Case Report: Identification of a de novo Microdeletion 1q44 in a Patient With Seizures and Developmental Delay
title Case Report: Identification of a de novo Microdeletion 1q44 in a Patient With Seizures and Developmental Delay
title_full Case Report: Identification of a de novo Microdeletion 1q44 in a Patient With Seizures and Developmental Delay
title_fullStr Case Report: Identification of a de novo Microdeletion 1q44 in a Patient With Seizures and Developmental Delay
title_full_unstemmed Case Report: Identification of a de novo Microdeletion 1q44 in a Patient With Seizures and Developmental Delay
title_short Case Report: Identification of a de novo Microdeletion 1q44 in a Patient With Seizures and Developmental Delay
title_sort case report: identification of a de novo microdeletion 1q44 in a patient with seizures and developmental delay
topic Genetics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8173053/
https://www.ncbi.nlm.nih.gov/pubmed/34093647
http://dx.doi.org/10.3389/fgene.2021.648351
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