Curtin University, Perth, Australia.
Health Catalyst, Utah, USA.
Sci Rep. 2024 Mar 1;14(1):5056. doi: 10.1038/s41598-024-55424-8.
Rare genetic diseases affect 5-8% of the population but are often undiagnosed or misdiagnosed. Electronic health records (EHR) contain large amounts of data, which provide opportunities for analysing and mining. Data mining, in the form of cluster analysis and visualisation, was performed on a database containing deidentified health records of 1.28 million patients across 3 major hospitals in Singapore, in a bid to improve the diagnostic process for patients who are living with an undiagnosed rare disease, specifically focusing on Fabry Disease and Familial Hypercholesterolaemia (FH). On a baseline of 4 patients, we identified 2 additional patients with potential diagnosis of Fabry disease, suggesting a potential 50% increase in diagnosis. Similarly, we identified > 12,000 individuals who fulfil the clinical and laboratory criteria for FH but had not been diagnosed previously. This proof-of-concept study showed that it is possible to perform mining on EHR data albeit with some challenges and limitations.
罕见遗传病影响 5-8%的人口,但往往未被诊断或误诊。电子健康记录 (EHR) 包含大量数据,为分析和挖掘提供了机会。通过对包含新加坡 3 家大医院的 128 万患者匿名健康记录的数据库进行聚类分析和可视化处理,旨在改进患有未确诊罕见病患者的诊断过程,特别是针对法布瑞病和家族性高胆固醇血症 (FH)。在 4 名患者的基础上,我们发现了另外 2 名可能患有法布瑞病的患者,提示诊断率可能增加 50%。同样,我们发现了超过 12000 名符合 FH 临床和实验室标准但之前未被诊断的个体。这项概念验证研究表明,尽管存在一些挑战和限制,但可以对 EHR 数据进行挖掘。