Cross-Sectional and Longitudinal Assessment of the Ellipsoid Zone in Childhood-Onset Stargardt Disease.
ABCA4
EZ
OCT
STGD1
Stargardt disease
clinical trials
ellipsoid zone
endpoints
retina
Journal
Translational vision science & technology
ISSN: 2164-2591
Titre abrégé: Transl Vis Sci Technol
Pays: United States
ID NLM: 101595919
Informations de publication
Date de publication:
Mar 2019
Mar 2019
Historique:
received:
18
10
2018
accepted:
12
12
2018
entrez:
6
3
2019
pubmed:
6
3
2019
medline:
6
3
2019
Statut:
epublish
Résumé
To evaluate the reliability of ellipsoid zone (EZ) loss width and area measurements from spectral-domain optical coherence tomography (SD-OCT) images and track disease progression in childhood-onset Stargardt disease (STGD1). Children with molecularly confirmed STGD1 ( The intra- and intergrader reliability of transverse EZ loss and area of EZ loss measurements at baseline for both graders was 0.99. The mean annual rate of transverse EZ loss (±standard deviation) was 279.5 ± 259.9 μm/y. The mean rate of area of EZ loss (±standard deviation) was 1.20 ± 1.29 mm This is a prospective study on the quantification of EZ loss in children with STGD1 and highlights the reliability of SD-OCT in measuring EZ loss. High intra- and intergrader reliability was observed, with good ability to detect changes over time. Measuring the area of EZ loss was more sensitive compared with transverse EZ width loss measurements and will be valuable for natural history studies and clinical trials requiring sensitive and reliable structural endpoints.
Identifiants
pubmed: 30834176
doi: 10.1167/tvst.8.2.1
pii: TVST-18-1188
pmc: PMC6397016
doi:
Types de publication
Journal Article
Langues
eng
Pagination
1Subventions
Organisme : Wellcome Trust
Pays : United Kingdom
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