Application of targeted high-throughput sequencing as a diagnostic tool for neonatal genetic metabolic diseases following tandem mass spectrometry screening

BackgroundTandem mass spectrometry (MS/MS) is a crucial technique for detecting inborn errors of metabolism (IEM) in newborns. However, the high false positive rate poses challenges in diagnosing specific types of diseases. Therefore, this study aimed to evaluate the role of targeted next-generation...

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Main Authors: Guihua Lai, Qiying Gu, Zhiyong Lai, Haijun Chen, Xiangwen Tu, Junkun Chen, Jungao Huang
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-12-01
Series:Frontiers in Public Health
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Online Access:https://www.frontiersin.org/articles/10.3389/fpubh.2024.1461141/full
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author Guihua Lai
Qiying Gu
Zhiyong Lai
Haijun Chen
Xiangwen Tu
Junkun Chen
Jungao Huang
author_facet Guihua Lai
Qiying Gu
Zhiyong Lai
Haijun Chen
Xiangwen Tu
Junkun Chen
Jungao Huang
author_sort Guihua Lai
collection DOAJ
description BackgroundTandem mass spectrometry (MS/MS) is a crucial technique for detecting inborn errors of metabolism (IEM) in newborns. However, the high false positive rate poses challenges in diagnosing specific types of diseases. Therefore, this study aimed to evaluate the role of targeted next-generation sequencing (NGS) in the accurate diagnosis of positive samples identified through MS/MS screening.MethodsA cohort study of 260,915 newborns was conducted from January 2018 to June 2023 in Ganzhou City, southern China. Heel blood samples were collected within 72 h of birth and subjected to MS/MS analysis. Infants with positive MS/MS results underwent targeted NGS to confirm the diagnosis and identify genetic variants.ResultsAmong 1,265 suspected cases with positive MS/MS results, 73 were confirmed by NGS, and 12 were identified as carriers of recessive diseases. The overall incidence rate was 1 in 3,574, effectively ruling out 94.2% (1,192/1,265) of the MS/MS false-positive. We found 76 variants in 18 genes associated with 15 types of IEM. Among these, 64.47% (49/76) were pathogenic, 10.53% (8/76) were likely pathogenic. Remarkably, 7.89% (6/76) were identified as novel variants. Variants in SLC22A5 (NM_003060.4) gene was most prevalent, accounting for 41% (77/188), with hotspot variants including c.51C > G, c.1400C > G, and c.338G > A.ConclusionTargeted NGS technology can serve as a crucial diagnostic tool for neonatal genetic metabolic diseases following MS/MS screening. Additionally, we identified IEM variant hotspots and some novel variants in our region, which are the underlying causes of disease in patients with IEM.
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spelling doaj-art-4606c0b9d7d14cdabcea34e6d7432dc32024-12-24T06:36:45ZengFrontiers Media S.A.Frontiers in Public Health2296-25652024-12-011210.3389/fpubh.2024.14611411461141Application of targeted high-throughput sequencing as a diagnostic tool for neonatal genetic metabolic diseases following tandem mass spectrometry screeningGuihua LaiQiying GuZhiyong LaiHaijun ChenXiangwen TuJunkun ChenJungao HuangBackgroundTandem mass spectrometry (MS/MS) is a crucial technique for detecting inborn errors of metabolism (IEM) in newborns. However, the high false positive rate poses challenges in diagnosing specific types of diseases. Therefore, this study aimed to evaluate the role of targeted next-generation sequencing (NGS) in the accurate diagnosis of positive samples identified through MS/MS screening.MethodsA cohort study of 260,915 newborns was conducted from January 2018 to June 2023 in Ganzhou City, southern China. Heel blood samples were collected within 72 h of birth and subjected to MS/MS analysis. Infants with positive MS/MS results underwent targeted NGS to confirm the diagnosis and identify genetic variants.ResultsAmong 1,265 suspected cases with positive MS/MS results, 73 were confirmed by NGS, and 12 were identified as carriers of recessive diseases. The overall incidence rate was 1 in 3,574, effectively ruling out 94.2% (1,192/1,265) of the MS/MS false-positive. We found 76 variants in 18 genes associated with 15 types of IEM. Among these, 64.47% (49/76) were pathogenic, 10.53% (8/76) were likely pathogenic. Remarkably, 7.89% (6/76) were identified as novel variants. Variants in SLC22A5 (NM_003060.4) gene was most prevalent, accounting for 41% (77/188), with hotspot variants including c.51C > G, c.1400C > G, and c.338G > A.ConclusionTargeted NGS technology can serve as a crucial diagnostic tool for neonatal genetic metabolic diseases following MS/MS screening. Additionally, we identified IEM variant hotspots and some novel variants in our region, which are the underlying causes of disease in patients with IEM.https://www.frontiersin.org/articles/10.3389/fpubh.2024.1461141/fullgenetic diagnosisinborn errors of metabolismnewborn screeningtargeted sequencingtandem mass spectrometry
spellingShingle Guihua Lai
Qiying Gu
Zhiyong Lai
Haijun Chen
Xiangwen Tu
Junkun Chen
Jungao Huang
Application of targeted high-throughput sequencing as a diagnostic tool for neonatal genetic metabolic diseases following tandem mass spectrometry screening
Frontiers in Public Health
genetic diagnosis
inborn errors of metabolism
newborn screening
targeted sequencing
tandem mass spectrometry
title Application of targeted high-throughput sequencing as a diagnostic tool for neonatal genetic metabolic diseases following tandem mass spectrometry screening
title_full Application of targeted high-throughput sequencing as a diagnostic tool for neonatal genetic metabolic diseases following tandem mass spectrometry screening
title_fullStr Application of targeted high-throughput sequencing as a diagnostic tool for neonatal genetic metabolic diseases following tandem mass spectrometry screening
title_full_unstemmed Application of targeted high-throughput sequencing as a diagnostic tool for neonatal genetic metabolic diseases following tandem mass spectrometry screening
title_short Application of targeted high-throughput sequencing as a diagnostic tool for neonatal genetic metabolic diseases following tandem mass spectrometry screening
title_sort application of targeted high throughput sequencing as a diagnostic tool for neonatal genetic metabolic diseases following tandem mass spectrometry screening
topic genetic diagnosis
inborn errors of metabolism
newborn screening
targeted sequencing
tandem mass spectrometry
url https://www.frontiersin.org/articles/10.3389/fpubh.2024.1461141/full
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