Next-generation phenotyping using computer vision algorithms in rare genomic neurodevelopmental disorders.


Journal

Genetics in medicine : official journal of the American College of Medical Genetics
ISSN: 1530-0366
Titre abrégé: Genet Med
Pays: United States
ID NLM: 9815831

Informations de publication

Date de publication:
08 2019
Historique:
received: 02 07 2018
accepted: 03 12 2018
pubmed: 21 12 2018
medline: 7 2 2020
entrez: 21 12 2018
Statut: ppublish

Résumé

The interpretation of genetic variants after genome-wide analysis is complex in heterogeneous disorders such as intellectual disability (ID). We investigate whether algorithms can be used to detect if a facial gestalt is present for three novel ID syndromes and if these techniques can help interpret variants of uncertain significance. Facial features were extracted from photos of ID patients harboring a pathogenic variant in three novel ID genes (PACS1, PPM1D, and PHIP) using algorithms that model human facial dysmorphism, and facial recognition. The resulting features were combined into a hybrid model to compare the three cohorts against a background ID population. We validated our model using images from 71 individuals with Koolen-de Vries syndrome, and then show that facial gestalts are present for individuals with a pathogenic variant in PACS1 (p = 8 × 10 Our results show that analysis of facial photos can be used to detect previously unknown facial gestalts for novel ID syndromes, which will facilitate both clinical and molecular diagnosis of rare and novel syndromes.

Identifiants

pubmed: 30568311
doi: 10.1038/s41436-018-0404-y
pii: S1098-3600(21)01612-9
pmc: PMC6752476
doi:

Substances chimiques

Intracellular Signaling Peptides and Proteins 0
PACS1 protein, human 0
PHIP protein, human 0
Vesicular Transport Proteins 0
PPM1D protein, human EC 3.1.3.16
Protein Phosphatase 2C EC 3.1.3.16

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1719-1725

Subventions

Organisme : Medical Research Council
ID : MR/M014568/1
Pays : United Kingdom

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Auteurs

Roos van der Donk (R)

Princess Máxima Center for Pediatric Oncology, Bilthoven, The Netherlands.
Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.

Sandra Jansen (S)

Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.

Janneke H M Schuurs-Hoeijmakers (JHM)

Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.

David A Koolen (DA)

Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.

Lia C M J Goltstein (LCMJ)

Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.

Alexander Hoischen (A)

Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.

Han G Brunner (HG)

Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.

Patrick Kemmeren (P)

Princess Máxima Center for Pediatric Oncology, Bilthoven, The Netherlands.

Christoffer Nellåker (C)

Nuffield Department of Women's and Reproductive Health, University of Oxford, Oxford, UK.
Institute of Biomedical Engineering, Department of Engineering Science, University of Oxford, Oxford, UK.
Big Data Institute, Li Ka Shing Centre for Health Information and Discovery, University of Oxford, Oxford, UK.

Lisenka E L M Vissers (LELM)

Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.

Bert B A de Vries (BBA)

Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands. Bert.deVries@radboudumc.nl.

Jayne Y Hehir-Kwa (JY)

Princess Máxima Center for Pediatric Oncology, Bilthoven, The Netherlands. J.Y.HehirKwa@prinsesmaximacentrum.nl.

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