Evaluating Face2Gene as a Tool to Identify Cornelia de Lange Syndrome by Facial Phenotypes.


Journal

International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791

Informations de publication

Date de publication:
04 Feb 2020
Historique:
received: 30 12 2019
revised: 01 02 2020
accepted: 02 02 2020
entrez: 9 2 2020
pubmed: 9 2 2020
medline: 18 11 2020
Statut: epublish

Résumé

Characteristic or classic phenotype of Cornelia de Lange syndrome (CdLS) is associated with a recognisable facial pattern. However, the heterogeneity in causal genes and the presence of overlapping syndromes have made it increasingly difficult to diagnose only by clinical features. DeepGestalt technology, and its app Face2Gene, is having a growing impact on the diagnosis and management of genetic diseases by analysing the features of affected individuals. Here, we performed a phenotypic study on a cohort of 49 individuals harbouring causative variants in known CdLS genes in order to evaluate Face2Gene utility and sensitivity in the clinical diagnosis of CdLS. Based on the profile images of patients, a diagnosis of CdLS was within the top five predicted syndromes for 97.9% of our cases and even listed as first prediction for 83.7%. The age of patients did not seem to affect the prediction accuracy, whereas our results indicate a correlation between the clinical score and affected genes. Furthermore, each gene presents a different pattern recognition that may be used to develop new neural networks with the goal of separating different genetic subtypes in CdLS. Overall, we conclude that computer-assisted image analysis based on deep learning could support the clinical diagnosis of CdLS.

Identifiants

pubmed: 32033219
pii: ijms21031042
doi: 10.3390/ijms21031042
pmc: PMC7038094
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : Spanish Ministry of Health - Fondo de Investigación Sanitaria (FIS)
ID : PI19/01860
Organisme : Spanish Ministry of Science, Innovation and Universities/State Research Agency
ID : RTC-2017-6494-1
Organisme : Spanish Ministry of Science, Innovation and Universities/State Research Agency
ID : RTI2018-094434-B-I00
Organisme : Diputación General de Aragón - FEDER: European Social Fund
ID : Grupo de Referencia B32_17R
Organisme : European JPIAMR-VRI
ID : network "CONNECT"
Organisme : Fondazione Pisa
ID : Fondazione Pisa
Organisme : Medical Faculty of the University of Lübeck
ID : J09-2017
Organisme : German Federal Ministry of Education and Research
ID : CHROMATIN-Net 01GM1520C

Déclaration de conflit d'intérêts

The authors declare no conflicts of interest.

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Auteurs

Ana Latorre-Pellicer (A)

Unit of Clinical Genetics and Functional Genomics, Department of Pharmacology-Physiology, School of Medicine, University of Zaragoza, CIBERER-GCV02 and ISS-Aragon, E-50009 Zaragoza, Spain.

Ángela Ascaso (Á)

Department of Paediatrics, Hospital Clínico Universitario "Lozano Blesa", E-50009 Zaragoza, Spain.

Laura Trujillano (L)

Department of Paediatrics, Hospital Clínico Universitario "Lozano Blesa", E-50009 Zaragoza, Spain.

Marta Gil-Salvador (M)

Unit of Clinical Genetics and Functional Genomics, Department of Pharmacology-Physiology, School of Medicine, University of Zaragoza, CIBERER-GCV02 and ISS-Aragon, E-50009 Zaragoza, Spain.

Maria Arnedo (M)

Unit of Clinical Genetics and Functional Genomics, Department of Pharmacology-Physiology, School of Medicine, University of Zaragoza, CIBERER-GCV02 and ISS-Aragon, E-50009 Zaragoza, Spain.

Cristina Lucia-Campos (C)

Unit of Clinical Genetics and Functional Genomics, Department of Pharmacology-Physiology, School of Medicine, University of Zaragoza, CIBERER-GCV02 and ISS-Aragon, E-50009 Zaragoza, Spain.

Rebeca Antoñanzas-Pérez (R)

Unit of Clinical Genetics and Functional Genomics, Department of Pharmacology-Physiology, School of Medicine, University of Zaragoza, CIBERER-GCV02 and ISS-Aragon, E-50009 Zaragoza, Spain.

Iñigo Marcos-Alcalde (I)

Molecular Modelling Group, Centro de Biología Molecular Severo Ochoa, CBMSO (CSIC-UAM), E-28049 Madrid, Spain.
Bioscience Research Institute, School of Experimental Sciences, Universidad Francisco de Vitoria, UFV, E-28223 Pozuelo de Alarcón, Spain.

Ilaria Parenti (I)

Section for Functional Genetics, Institute of Human Genetics, University of Lübeck, 23562 Lübeck, Germany.
Institute of Science and Technology (IST) Austria, 3400 Klosterneuburg, Austria.

Gloria Bueno-Lozano (G)

Department of Paediatrics, Hospital Clínico Universitario "Lozano Blesa", E-50009 Zaragoza, Spain.

Antonio Musio (A)

Istituto di Ricerca Genetica e Biomedica, Consiglio Nazionale delle Ricerche, I-56124 Pisa, Italy.

Beatriz Puisac (B)

Unit of Clinical Genetics and Functional Genomics, Department of Pharmacology-Physiology, School of Medicine, University of Zaragoza, CIBERER-GCV02 and ISS-Aragon, E-50009 Zaragoza, Spain.

Frank J Kaiser (FJ)

Section for Functional Genetics, Institute of Human Genetics, University of Lübeck, 23562 Lübeck, Germany.
Institute for Human Genetics, University Hospital Essen, University of Duisburg-Essen, 45147 Essen, Germany.

Feliciano J Ramos (FJ)

Unit of Clinical Genetics and Functional Genomics, Department of Pharmacology-Physiology, School of Medicine, University of Zaragoza, CIBERER-GCV02 and ISS-Aragon, E-50009 Zaragoza, Spain.
Department of Paediatrics, Hospital Clínico Universitario "Lozano Blesa", E-50009 Zaragoza, Spain.

Paulino Gómez-Puertas (P)

Molecular Modelling Group, Centro de Biología Molecular Severo Ochoa, CBMSO (CSIC-UAM), E-28049 Madrid, Spain.

Juan Pié (J)

Unit of Clinical Genetics and Functional Genomics, Department of Pharmacology-Physiology, School of Medicine, University of Zaragoza, CIBERER-GCV02 and ISS-Aragon, E-50009 Zaragoza, Spain.

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