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Reliability and Repeatability of Cone Density Measurements in Patients with Congenital Achromatopsia. Adv Exp Med Biol 2016;854:277-83

Date

10/03/2015

Pubmed ID

26427422

Pubmed Central ID

PMC4839591

DOI

10.1007/978-3-319-17121-0_37

Scopus ID

2-s2.0-84943329540   29 Citations

Abstract

Adaptive optics scanning light ophthalmoscopy (AOSLO) allows non-invasive assessment of the cone photoreceptor mosaic. Confocal AOSLO imaging of patients with achromatopsia (ACHM) reveals an altered reflectivity of the remaining cone structure, making identification of the cells more challenging than in normal retinas. Recently, a "split-detector" AOSLO imaging method was shown to enable direct visualization of cone inner segments in patients with ACHM. Several studies have demonstrated gene replacement therapy effective in restoring cone function in animal models of ACHM and human trials have on the horizon, making the ability to reliably assess cone structure increasingly important. Here we sought to examine whether absolute estimates of cone density obtained from split-detector and confocal AOSLO images differed from one another and whether the inter- and intra-observer reliability is significantly different between these modes. These findings provide an important foundation for evaluating the role of these images as tools to assess the efficacy of future gene therapy trials.

Author List

Abozaid MA, Langlo CS, Dubis AM, Michaelides M, Tarima S, Carroll J

Authors

Joseph J. Carroll PhD Director, Professor in the Ophthalmology and Visual Sciences department at Medical College of Wisconsin
Sergey S. Tarima PhD Associate Professor in the Institute for Health and Equity department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Adult
Child
Color Vision Defects
Cyclic Nucleotide-Gated Cation Channels
Female
Humans
Male
Microscopy, Confocal
Middle Aged
Mutation
Ophthalmoscopy
Reproducibility of Results
Retinal Cone Photoreceptor Cells
Sensitivity and Specificity
Young Adult
jenkins-FCD Prod-486 e3098984f26de787f5ecab75090d0a28e7f4f7c0