Progressive bilateral nuclear cataracts associated with cerebellar-facial-dental syndrome: case report, literature review, and identification of a new genetic variant.


Journal

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
ISSN: 1528-3933
Titre abrégé: J AAPOS
Pays: United States
ID NLM: 9710011

Informations de publication

Date de publication:
12 2021
Historique:
received: 20 05 2021
revised: 14 07 2021
accepted: 18 07 2021
pubmed: 11 10 2021
medline: 18 3 2022
entrez: 10 10 2021
Statut: ppublish

Résumé

Cerebellar-facial-dental syndrome (CFDS) is a newly described autosomal recessive genetic disorder characterized by mutations in the BRF1 gene. CFDS is clinically associated with dysmorphic facial features and cerebellar hypoplasia. We report visually significant progressive bilateral nuclear cataracts in a child with CFDS and identify a new causative genetic variant.

Identifiants

pubmed: 34628026
pii: S1091-8531(21)00544-9
doi: 10.1016/j.jaapos.2021.07.006
pii:
doi:

Substances chimiques

BRF1 protein, human 0
TATA-Binding Protein Associated Factors 0

Types de publication

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

Langues

eng

Sous-ensembles de citation

IM

Pagination

370-373

Informations de copyright

Copyright © 2021 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

Auteurs

Brianna Pandey (B)

Creighton University School of Medicine, Omaha, Nebraska.

Newell Belnap (N)

Translational Genomics Research Institute, Neurogenomics Division, Center for Rare Childhood Disorders, Phoenix, Arizona.

Chris Balak (C)

Department of Cellular and Molecular Medicine, University of California San Diego, School of Medicine, La Jolla, California.

Matt Huentelman (M)

Translational Genomics Research Institute, Neurogenomics Division, Center for Rare Childhood Disorders, Phoenix, Arizona.

Keri Ramsey (K)

Translational Genomics Research Institute, Neurogenomics Division, Center for Rare Childhood Disorders, Phoenix, Arizona.

Vinodh Narayanan (V)

Translational Genomics Research Institute, Neurogenomics Division, Center for Rare Childhood Disorders, Phoenix, Arizona.

James Plotnik (J)

Department of Ophthalmology, Phoenix Children's Hospital, Phoenix, Arizona. Electronic address: jplotnik@phoenixchildrens.com.

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Classifications MeSH