@article{5b7a98710c0e433ea12f09a6fc8002ed,
title = "Macular spatial distribution of preserved autofluorescence in patients with choroideremia",
abstract = "Background/Aims To better understand the pattern of degeneration progression in cases with choroideremia. Methods A cohort of genotypically confirmed choroideremia cases who underwent optical coherence tomography (OCT) and fundus autofluorescence (FAF) imaging was studied. Using HEYEX review software, the foveal centre was marked on FAF images under guidance of corresponding OCT images, followed by application of an ETDRS grid. The boundaries of preserved autofluorescence (AF) were manually segmented in each individual ETDRS subfield. The regional distribution of preserved AF was assessed by comparing its area among the various subfields. Results A total of 168 eyes from 84 choroideremia cases were enrolled. There was a statistically significant difference in the amount of preserved AF area between inner subfields as determined by one-way analysis of variance (F (3,668)=9.997, p<0.001) and also between outer subfields (F (3,668)=8.348, p<0.001). A Tukey posthoc test revealed that the preserved AF area in the nasal subfields in both the inner and outer subfields was significantly smaller compared with analogue subfields. Conclusion The asymmetric spatial distribution of preserved AF in choroideremia (corresponding to the stellate shaped nature of these regions) suggests that the progression of degeneration has directional preference.",
keywords = "choroideremia, ellipsoid zone, fundus autofluorescence, optical coherence tomography, preserved autofluorescence, retinal pigment epithelium",
author = "Hariri, {Amir H.} and Ip, {Michael S.} and Aniz Girach and Lam, {Byron L.} and Fischer, {M. Dominik} and Sankila, {Eeva Marja} and Pennesi, {Mark Edward} and Holz, {Frank G.} and Maclaren, {Robert E.} and Birch, {David G.} and Hoyng, {Carel B.} and Macdonald, {Ian M.} and Black, {Graeme C.} and Tsang, {Stephen H.} and Bressler, {Neil M.} and Stepien, {Kimberly E.} and Michael Larsen and Gorin, {Michael B.} and Isabelle Meunier and Webster, {Andrew R.} and Srinivas Sadda",
note = "Funding Information: 16Department of Pathology and Cell Biology, Columbia University, New York, New York, USA 17Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA 18Department of Ophthalmology & Visual Sciences, Madison, Wisconsin, USA 19Department of Ophthalmology, Rigshospitalet and University of Copenhagen, Copenhagen, Denmark 20Eye Clinic, H{\^o}pital Robert Debr{\'e}, CHRU de Reims, Reims, France 21Moorfields Eye Hospital NHS Foundation Trust, London, UK 22UCL Institute of Ophthalmology, London, UK Acknowledgements Nightstar Therapeutics is the sponsor of the Natural History of the Progression of Choroideremia (NIGHT) Study Group. Support for the reading center personnel was also provided in part by Beckman Macular Degeneration Research Center and a Research to Prevent Blindness Physician Scientist Award. Funding Information: disclaimer AHH: No financial disclosures. MSI: Thrombogenics (Consultant), Boehringer Ingelheim (Consultant), Omeros (Consultant), Genentech (Consultant), Quark (Consultant). AG: Employee of Nightstar Therapeutics. BLL: Research funding from Nightstar Therapeutics. MDF: Nightstar Therapeutics (Consultant, Financial Support), Casebia Therapeutics (Consultant, Financial Support), Spark Therapeutics (Consultant), Regenxbio (Consultant), Eyeserve GmbH (Consultant), Bayer (Financial Support), Novartis (Financial Support). E-MS: No financial disclosures. MEP: No financial disclosures. FGH: Nightstar Therapeutics (Financial Support), Carl Zeiss Meditec (Financial Support), Optos (Financial Support), Allergan (Consultant, Financial Support), Roche/Genentech (Consultant, Financial Support), Alcon (Consultant), Novartis (Consultant/Financial Support), Bayer (Consultant/Financial Support), Boehringer-Ingelheim (Consultant), Centervue (Financial Support), lin Bioscience (Consultant), Pixium (Consultant/Financial Support), Thrombogenics (Consultant). REM: Nightstar Therapeutics (Financial Support, Consultant, Founder and Director), Spark Therapeutics (Consultant) DGB: Nightstar Therapeutics (Consultant, Financial Support), Ionis Pharmaceuticals (consultant, financial support), Genentech (consultant), Nacuity (consultant), AGTC (consultant, financial support), 4D (financial support). CBH: No financial disclosures. IMM: Allergan (Consultant), Canadian Institutes for Health Research (Grant support), Alberta Innovates (Grant support), Foundation Fighting Blindness (Grant support). GCB: No financial disclosures. SHT: No financial disclosures NMB: Alkeus; Bayer; Chiltern; Nighstar Therapeutics; Novartis; Roche Samsung Bioepis. KES: No financial disclosures. ML: No financial disclosures. MBG: No financial disclosures. IM: No financial disclosures. ARW: No financial disclosures. SS: Carl Zeiss Meditec (Consultant, Financial Support), Optos (Consultant, Financial Support), Allergan (Consultant, Financial Support), Genentech (Consultant, Financial Support), Alcon (Consultant), Novartis (Consultant), Iconic (Consultant). Publisher Copyright: {\textcopyright} Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.",
year = "2019",
month = jul,
day = "1",
doi = "10.1136/bjophthalmol-2018-312620",
language = "English (US)",
volume = "103",
pages = "933--937",
journal = "British Journal of Ophthalmology",
issn = "0007-1161",
publisher = "BMJ Publishing Group",
number = "7",
}