Rare-ID: Genomic Diagnosis in Symptomatic Neonates and Young Infants with Complex Clinical Phenotypes: A Descriptive Cohort Study

Background/Objectives: Genomic sequencing can shorten the diagnostic pathway for selected symptomatic neonates and young infants, but evidence from such cohorts should not be extrapolated to population newborn screening. This study describes molecular findings and potential clinical implications in 25 unrelated patients younger than 6 months at referral with heterogeneous, predominantly neurological phenotypes and no established molecular diagnosis. Methods: The first 17 patients underwent whole-exome sequencing (WES), and the subsequent 8 underwent whole-genome sequencing (WGS) under sequential laboratory protocols; allocation was not randomized, and the study was not designed to compare platforms. Results: Pathogenic or likely pathogenic findings providing a definitive or likely molecular diagnosis were identified in 7/25 patients (28.0%; 95% confidence interval [CI], 14.3–47.6), including sequence variants, one 20q13.33 deletion, and mosaic trisomy 9. An additional RANBP2 variant was interpreted as a susceptibility-associated finding in a patient with infection-related encephalitis, yielding clinically relevant findings in 8/25 patients (32.0%; 95% CI, 17.2–51.6). Three definitive diagnoses involved disorders with established disease-specific management considerations; however, patient-level treatment changes, turnaround times, and outcomes were not systematically assessed. Conclusions: These findings support the diagnostic value of genomic testing in selected symptomatic neonates and young infants, while the small, heterogeneous cohort, sequential non-equivalent workflows, and incomplete outcome data preclude conclusions about comparative WES/WGS performance or population newborn screening.

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Publication Details

Journal
Genes
Published
2026-08-14
DOI
https://doi.org/10.3390/genes17080952
Primary Topic
Genomics and Rare Diseases
Type
article
Field-Weighted Citation Impact
0.00

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article

Rare-ID: Genomic Diagnosis in Symptomatic Neonates and Young Infants with Complex Clinical Phenotypes: A Descriptive Cohort Study

Yannis L. Loukas, Konstantinos Tegopoulos, Stefania Antonopoulou, Elina Molou et al.
Genes
Genomics and Rare Diseases
article

Rare-ID: Genomic Diagnosis in Symptomatic Neonates and Young Infants with Complex Clinical Phenotypes: A Descriptive Cohort Study

Yannis L. Loukas, Konstantinos Tegopoulos, Stefania Antonopoulou, Elina Molou, Konstantinos Douros, Argirios Dinopoulos, Georgia Thodi, Tania Siahanidou, Maria Angeli, Vasiliki Koute, Antonia Charitou, Eleftheria Kokkinou, Yannis Dotsikas, Eleni Skouteli, Maria Spanou, Eirini Loukatou, Christos Gavalas, Maria Alvanou, Melpomene Giorgi, Konstantinos Tziouvas, Artemis Stephanede, Roser Pons, Georgios Vartzelis, Katherine Anagnostopoulou, Antigoni Poulopoulou, Maria Nikolaidou, Ioanna Kouri, Eleni Frysira
article en

Abstract

Background/Objectives: Genomic sequencing can shorten the diagnostic pathway for selected symptomatic neonates and young infants, but evidence from such cohorts should not be extrapolated to population newborn screening. This study describes molecular findings and potential clinical implications in 25 unrelated patients younger than 6 months at referral with heterogeneous, predominantly neurological phenotypes and no established molecular diagnosis. Methods: The first 17 patients underwent whole-exome sequencing (WES), and the subsequent 8 underwent whole-genome sequencing (WGS) under sequential laboratory protocols; allocation was not randomized, and the study was not designed to compare platforms. Results: Pathogenic or likely pathogenic findings providing a definitive or likely molecular diagnosis were identified in 7/25 patients (28.0%; 95% confidence interval [CI], 14.3–47.6), including sequence variants, one 20q13.33 deletion, and mosaic trisomy 9. An additional RANBP2 variant was interpreted as a susceptibility-associated finding in a patient with infection-related encephalitis, yielding clinically relevant findings in 8/25 patients (32.0%; 95% CI, 17.2–51.6). Three definitive diagnoses involved disorders with established disease-specific management considerations; however, patient-level treatment changes, turnaround times, and outcomes were not systematically assessed. Conclusions: These findings support the diagnostic value of genomic testing in selected symptomatic neonates and young infants, while the small, heterogeneous cohort, sequential non-equivalent workflows, and incomplete outcome data preclude conclusions about comparative WES/WGS performance or population newborn screening.

GenesVol. 17(8)
Democritus University of Thrace (GR), National and Kapodistrian University of Athens (GR), LETO Maternity Hospital (GR), University Hospital of Larissa (GR), Alexandra Hospital (GR), Children's Hospital Agia Sophia (GR), Mitera Hospital (GR), ELEPAP-Rehabilitation for The Disabled (GR), Panagiotis & Aglaia Kyriakou Children's Hospital (GR), Athens State University (US)
General Secretariat for Research and Technology
Openalex Percentile: Top 9%
Genomics and Rare Diseases
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