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Caring for Hereditary Childhood Retinal Blindness
Journal article   Open access   Peer reviewed

Caring for Hereditary Childhood Retinal Blindness

Ruben Jauregui, Galaxy Y Cho, Vitor K L Takahashi, Julia T Takiuti, Alexander G Bassuk, Vinit B Mahajan and Stephen H Tsang
Asia-Pacific journal of ophthalmology (Philadelphia, Pa.), Vol.7(3), pp.183-191
05/2018
DOI: 10.22608/APO.201851
PMID: 29536675
url
https://doi.org/10.22608/APO.201851View
Published (Version of record) Open Access

Abstract

Inherited retinal diseases (IRDs) are a major cause of incurable familial blindness in the Western world. In the pediatric population, IRDs are a major contributor to the 19 million children worldwide with visual impairment. Unfortunately, the road to the correct diagnosis is often complicated in the pediatric population, as typical diagnostic tools such as fundus examination, electrodiagnostic studies, and other imaging modalities may be difficult to perform in the pediatric patient. In this review, we describe the most significant IRDs with onset during the pediatric years (ie, before the age of 18). We describe the pathogenesis, clinical presentation, and potential treatment of these diseases. In addition, we advocate the use of a pedigree (family medical history), electroretinography, and genetic testing as the 3 most crucial tools for the correct diagnosis of IRDs in the pediatric population.
inherited retinal diseases retinintis pigmentosa pediatrics photoreceptor LCA childhood onset pediatric ophthalmology

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