加速色素性视网膜炎的基因诊断:香港基因组计划半自动化定制队列分析工作流程的实施。

IF 3.8 2区 生物学 Q2 GENETICS & HEREDITY
Dingge Ying, Jamie Sui Lam Kwok, Annie Tsz Wai Chu, Wei Ma, Helen Ying Fung Tam, Dicky Or, Shirley Pik Ying Hue, Qing Li, Christopher Kai Shun Leung, Brian Hon Yin Chung
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引用次数: 0

摘要

该研究旨在利用下一代测序(NGS)的先进技术,为香港基因组计划(HKGP)中患有或疑似患有视网膜色素变性(RP)的患者开发半自动定制队列分析工作流程(S-BCAW),以提高香港基因组研究所基因变异管理过程的效率。对79例RP患者进行了对比分析,采用传统的手工工作流程和新型的S-BCAW,结合了基于ACMG指南的初始过滤和变异分类,然后进行了详细的手工审查。两种方法的诊断结果是相同的,但是定制的工作流程减少了大约60%的分析时间(1.5小时/样本)。这种效率的提高是由于ACMG规则的自动化应用和支持数据的系统汇总,包括疾病特定信息。该研究报告了25例阳性病例,诊断率为32%,其中包括三种新的变异。S-BCAW显着提高了效率,帮助结束了香港gp患者的诊断过程。这种方法有助于快速评估变异致病性,提高NGS技术临床应用的可行性和及时性,特别是在紧急情况下。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Accelerating genetic diagnostics in retinitis pigmentosa: implementation of a semi-automated bespoke cohort analysis workflow for Hong Kong Genome Project.

The study aims to enhance the efficiency of the genetic variant curation process at the Hong Kong Genome Institute by developing a Semi-Automated Bespoke Cohort Analysis Workflow (S-BCAW) for patients with, or suspected to have, retinitis pigmentosa (RP) in the Hong Kong Genome Project (HKGP), leveraging advances in next-generation sequencing (NGS). A comparative analysis involving 79 RP patients was conducted using both the conventional manual workflow and the novel S-BCAW, which integrates initial filtering and variant classification based on ACMG guidelines, followed by detailed manual review. The diagnostic yields from both methods were identical, but the bespoke workflow reduced analysis time by approximately 60% (1.5 h/sample). This efficiency increase resulted from automated application of ACMG rules and systematic aggregation of supportive data, including disease-specific information. The study reports 25 positive cases with a diagnostic yield of 32%, including three novel variants. The S-BCAW significantly improves efficiency, helping to end the diagnostic odyssey for patients in the HKGP. This approach facilitates rapid assessment of variant pathogenicity, enhancing the feasibility and timeliness of NGS technology for clinical applications, especially in urgent scenarios.

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来源期刊
Human Genetics
Human Genetics 生物-遗传学
CiteScore
10.80
自引率
3.80%
发文量
94
审稿时长
1 months
期刊介绍: Human Genetics is a monthly journal publishing original and timely articles on all aspects of human genetics. The Journal particularly welcomes articles in the areas of Behavioral genetics, Bioinformatics, Cancer genetics and genomics, Cytogenetics, Developmental genetics, Disease association studies, Dysmorphology, ELSI (ethical, legal and social issues), Evolutionary genetics, Gene expression, Gene structure and organization, Genetics of complex diseases and epistatic interactions, Genetic epidemiology, Genome biology, Genome structure and organization, Genotype-phenotype relationships, Human Genomics, Immunogenetics and genomics, Linkage analysis and genetic mapping, Methods in Statistical Genetics, Molecular diagnostics, Mutation detection and analysis, Neurogenetics, Physical mapping and Population Genetics. Articles reporting animal models relevant to human biology or disease are also welcome. Preference will be given to those articles which address clinically relevant questions or which provide new insights into human biology. Unless reporting entirely novel and unusual aspects of a topic, clinical case reports, cytogenetic case reports, papers on descriptive population genetics, articles dealing with the frequency of polymorphisms or additional mutations within genes in which numerous lesions have already been described, and papers that report meta-analyses of previously published datasets will normally not be accepted. The Journal typically will not consider for publication manuscripts that report merely the isolation, map position, structure, and tissue expression profile of a gene of unknown function unless the gene is of particular interest or is a candidate gene involved in a human trait or disorder.
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