Literature DB >> 27186691

Ocular findings in a patient with Cockayne syndrome with two mutations in the ERCC6 gene.

Yuan Wu1, Yajie Zheng1, Xiaoming Yan1, Yu Huang2, Yuwu Jiang3, Haili Li1.   

Abstract

BACKGROUND: Cockayne syndrome is a rare, autosomal recessive, multisystem disorder that causes a senile appearance. Ophthalmic abnormalities are frequently present. Here, we report a wide range of ocular findings in a child with Cockayne syndrome.
MATERIALS AND METHODS: The systemic and ocular findings were reviewed. A mutation analysis was performed in the patient and her parents.
RESULTS: The patient underwent a complete ocular examination. Both eyes had low visual acuity, corneal epithelial degeneration, punctate opacities of the lens, and retina disorders. The systemic findings included growth deficiency and a senile appearance. Gene analysis showed mutations in exons 4 and 18 of the ERCC6 gene.
CONCLUSION: Multiple ocular abnormalities were observed in a patient with Cockayne syndrome. A detailed ophthalmic evaluation of children with Cockayne syndrome is advised.

Entities:  

Keywords:  Cockayne syndrome; ocular abnormalities; optical coherence tomography

Mesh:

Substances:

Year:  2016        PMID: 27186691     DOI: 10.3109/13816810.2016.1171881

Source DB:  PubMed          Journal:  Ophthalmic Genet        ISSN: 1381-6810            Impact factor:   1.803


  4 in total

1.  Cockayne syndrome in adults: complete retinal dysfunction exploration of two case reports.

Authors:  Marc Figueras-Roca; Vanessa Budi; Montserrat Morató; Anna Camós-Carreras; José Esteban Muñoz; Bernardo Sánchez-Dalmau
Journal:  Doc Ophthalmol       Date:  2019-02-28       Impact factor: 2.379

2.  Two heterozygous mutations in the ERCC6 gene associated with Cockayne syndrome in a Chinese patient.

Authors:  Qin Zhang; Minjuan Liu; Yinghua Liu; Hui Tang; Ting Wang; Hong Li; Jingjing Xiang
Journal:  J Int Med Res       Date:  2019-09-26       Impact factor: 1.671

3.  Pseudopapilledema in Cockayne syndrome.

Authors:  Michael C Brodsky; Deborah L Renaud
Journal:  Am J Ophthalmol Case Rep       Date:  2021-02-18

Review 4.  UV Protection in the Cornea: Failure and Rescue.

Authors:  Thomas Volatier; Björn Schumacher; Claus Cursiefen; Maria Notara
Journal:  Biology (Basel)       Date:  2022-02-10
  4 in total

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