{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T22:19:30Z","timestamp":1792016370050,"version":"4.4.0"},"reference-count":48,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2016,4,1]],"date-time":"2016-04-01T00:00:00Z","timestamp":1459468800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","aaojournal.org","clinicalkey.es","clinicalkey.com.au","clinicalkey.fr","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Ophthalmology"],"published-print":{"date-parts":[[2016,4]]},"DOI":"10.1016\/j.ophtha.2015.12.009","type":"journal-article","created":{"date-parts":[[2016,1,16]],"date-time":"2016-01-16T21:52:37Z","timestamp":1452981157000},"page":"817-828","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":152,"title":["The Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) Studies"],"prefix":"10.1016","volume":"123","clinical-trial-number":[{"clinical-trial-number":"nct01278277","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct01367444","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct01736592","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct01920867","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct01977846","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct02402660","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct02445612","registry":"10.18810\/clinical-trials-gov"}],"author":[{"given":"Rupert W.","family":"Strauss","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1418-4459","authenticated-orcid":false,"given":"Alex","family":"Ho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beatriz","family":"Mu\u00f1oz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Artur V.","family":"Cideciyan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos\u00e9-Alain","family":"Sahel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janet S.","family":"Sunness","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David G.","family":"Birch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul S.","family":"Bernstein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Michaelides","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elias I.","family":"Traboulsi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eberhart","family":"Zrenner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"SriniVas","family":"Sadda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ann-Margret","family":"Ervin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheila","family":"West","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hendrik P.N.","family":"Scholl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ophtha.2015.12.009_bib1","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1007\/BF01961301","article-title":"Ueber familiaere, progressive Degeneration in der Makulagegend des Auges","volume":"71","author":"Stargardt","year":"1909","journal-title":"Graefes Arch Clin Exp Ophthalmol"},{"issue":"3","key":"10.1016\/j.ophtha.2015.12.009_bib2","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1038\/ng0397-236","article-title":"A\u00a0photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Stargardt macular dystrophy","volume":"15","author":"Allikmets","year":"1997","journal-title":"Nat Genet"},{"key":"10.1016\/j.ophtha.2015.12.009_bib3","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1097\/IAE.0000000000000659","article-title":"Insights into autosomal dominant Stargardt-like macular dystrophy through multimodality diagnostic imaging","volume":"36","author":"Palejwala","year":"2016","journal-title":"Retina"},{"key":"10.1016\/j.ophtha.2015.12.009_bib4","doi-asserted-by":"crossref","first-page":"1151","DOI":"10.1016\/S0161-6420(03)00333-6","article-title":"Visual acuity loss and clinical observations in a large series of patients with Stargardt disease","volume":"110","author":"Rotenstreich","year":"2003","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib5","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1136\/bjophthalmol-2013-304270","article-title":"Predictors of visual acuity and genotype-phenotype correlates in a cohort of patients with Stargardt disease","volume":"98","author":"Miraldi Utz","year":"2014","journal-title":"Br J Ophthalmol"},{"key":"10.1016\/j.ophtha.2015.12.009_bib6","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1016\/j.ophtha.2014.08.012","article-title":"Clinical and molecular characteristics of childhood-onset Stargardt disease","volume":"122","author":"Fujinami","year":"2015","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib7","doi-asserted-by":"crossref","first-page":"1189","DOI":"10.1016\/S0161-6420(80)35106-3","article-title":"Retinal pigment epithelial abnormalities in fundus flavimaculatus: a light and electron microscopic study","volume":"87","author":"Eagle","year":"1980","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib8","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.1016\/S0161-6420(13)31725-4","article-title":"Histopathology and immunocytochemistry of the neurosensory retina in fundus flavimaculatus","volume":"101","author":"Birnbach","year":"1994","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib9","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.preteyeres.2011.12.001","article-title":"The bisretinoids of retinal pigment epithelium","volume":"31","author":"Sparrow","year":"2012","journal-title":"Prog Retin Eye Res"},{"key":"10.1016\/j.ophtha.2015.12.009_bib10","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1016\/j.ajo.2013.01.018","article-title":"A\u00a0longitudinal study of Stargardt disease: clinical and electrophysiologic assessment, progression, and genotype correlations","volume":"155","author":"Fujinami","year":"2013","journal-title":"Am J Ophthalmol"},{"key":"10.1016\/j.ophtha.2015.12.009_bib11","doi-asserted-by":"crossref","first-page":"931","DOI":"10.1093\/hmg\/ddn421","article-title":"ABCA4 disease progression and a proposed strategy for gene therapy","volume":"18","author":"Cideciyan","year":"2009","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ophtha.2015.12.009_bib12","doi-asserted-by":"crossref","first-page":"5946","DOI":"10.1167\/iovs.15-17698","article-title":"Predicting progression of ABCA4-associated retinal degenerations based on longitudinal measurements of the leading disease front","volume":"56","author":"Cideciyan","year":"2015","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib13","first-page":"227","article-title":"Familial discordance in Stargardt disease","volume":"18","author":"Burke","year":"2012","journal-title":"Mol Vis"},{"key":"10.1016\/j.ophtha.2015.12.009_bib14","doi-asserted-by":"crossref","first-page":"1457","DOI":"10.1364\/JOSAA.24.001457","article-title":"Reduced-illuminance autofluorescence imaging in ABCA4-associated retinal degenerations","volume":"24","author":"Cideciyan","year":"2007","journal-title":"J\u00a0Opt Soc Am A Opt Image Sci Vis"},{"key":"10.1016\/j.ophtha.2015.12.009_bib15","doi-asserted-by":"crossref","unstructured":"Kuehlewein L, Hairi A, Ho A, et\u00a0al. Comparison of manual and semiautomated fundus autofluorescence analysis of macular atrophy in Stargardt disease phenotype. Retina http:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Comparison+of+manual+and+semiautomated+fundus 2015 Nov 18. [Epub ahead of print].","DOI":"10.1097\/IAE.0000000000000870"},{"issue":"5 Suppl","key":"10.1016\/j.ophtha.2015.12.009_bib16","first-page":"741","article-title":"Early Treatment Diabetic Retinopathy Study design and baseline patient characteristics. ETDRS report number 7","volume":"98","year":"1991","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib17","doi-asserted-by":"crossref","first-page":"2112","DOI":"10.1016\/j.ophtha.2010.02.033","article-title":"Evaluation of the age-related eye disease study clinical lens grading system AREDS report no. 31","volume":"117","author":"Chew","year":"2010","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib18","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s10633-014-9473-7","article-title":"ISCEV Standard for full-field clinical electroretinography (2015 update)","volume":"130","author":"McCulloch","year":"2015","journal-title":"Doc Ophthalmol"},{"key":"10.1016\/j.ophtha.2015.12.009_bib19","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.exer.2010.03.021","article-title":"Analysis of autofluorescent retinal images and measurement of atrophic lesion growth in Stargardt disease","volume":"91","author":"Chen","year":"2010","journal-title":"Exp Eye Res"},{"key":"10.1016\/j.ophtha.2015.12.009_bib20","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1186\/1471-2415-5-8","article-title":"Automated analysis of digital fundus autofluorescence images of geographic atrophy in advanced age-related macular degeneration using confocal scanning laser ophthalmoscopy (cSLO)","volume":"5","author":"Deckert","year":"2005","journal-title":"BMC Ophthalmol"},{"key":"10.1016\/j.ophtha.2015.12.009_bib21","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1186\/1741-7015-4-7","article-title":"Revision of visual impairment definitions in the International Statistical Classification of Diseases","volume":"4","author":"Dandona","year":"2006","journal-title":"BMC Med"},{"key":"10.1016\/j.ophtha.2015.12.009_bib22","doi-asserted-by":"crossref","first-page":"772","DOI":"10.1097\/00006982-200509000-00014","article-title":"Outcome measures and their application in clinical trials for retinal degenerative diseases: outline, review, and perspective","volume":"25","author":"Fishman","year":"2005","journal-title":"Retina"},{"key":"10.1016\/j.ophtha.2015.12.009_bib23","doi-asserted-by":"crossref","first-page":"918","DOI":"10.1167\/iovs.14-16049","article-title":"Advancing therapeutic strategies for inherited retinal degeneration: recommendations from the Monaciano Symposium","volume":"56","author":"Thompson","year":"2015","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib24","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/j.ophtha.2014.08.032","article-title":"Early-onset Stargardt disease: phenotypic and genotypic characteristics","volume":"122","author":"Lambertus","year":"2015","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib25","doi-asserted-by":"crossref","first-page":"1804","DOI":"10.1016\/j.ophtha.2007.06.047","article-title":"Visual acuity as an outcome measure in clinical trials of retinal diseases","volume":"114","author":"Beck","year":"2007","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib26","doi-asserted-by":"crossref","first-page":"5836","DOI":"10.1167\/iovs.13-12557","article-title":"Relationship between foveal cone structure and clinical measures of visual function in patients with inherited retinal degenerations","volume":"54","author":"Ratnam","year":"2013","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib27","doi-asserted-by":"crossref","first-page":"2399","DOI":"10.1016\/j.ophtha.2014.06.032","article-title":"Macular function and morphologic features in juvenile Stargardt disease: longitudinal study","volume":"121","author":"Testa","year":"2014","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib28","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1167\/iovs.07-1132","article-title":"Report from the NEI\/FDA Ophthalmic Clinical Trial Design and Endpoints Symposium","volume":"49","author":"Csaky","year":"2008","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib29","doi-asserted-by":"crossref","first-page":"2282","DOI":"10.1016\/j.ophtha.2012.05.027","article-title":"The Age-Related Eye Disease Study 2 (AREDS2): study design and baseline characteristics (AREDS2 report number 1)","volume":"119","author":"Chew","year":"2012","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib30","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1016\/j.ophtha.2006.09.016","article-title":"The long-term natural history of geographic atrophy from age-related macular degeneration: enlargement of atrophy and implications for interventional clinical trials","volume":"114","author":"Sunness","year":"2007","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib31","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1007\/978-1-4419-1399-9_45","article-title":"Fundus autofluorescence imaging in age-related macular degeneration and geographic atrophy","volume":"664","author":"Bearelly","year":"2010","journal-title":"Adv Exp Med Biol"},{"key":"10.1016\/j.ophtha.2015.12.009_bib32","doi-asserted-by":"crossref","first-page":"2648","DOI":"10.1167\/iovs.05-0892","article-title":"Correlation between the area of increased autofluorescence surrounding geographic atrophy and disease progression in patients with AMD","volume":"47","author":"Schmitz-Valckenberg","year":"2006","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib33","first-page":"1051","article-title":"Fundus autofluorescence and development of geographic atrophy in age-related macular degeneration","volume":"42","author":"Holz","year":"2001","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib34","doi-asserted-by":"crossref","first-page":"6131","DOI":"10.1167\/iovs.10-5637","article-title":"Treatment of geographic atrophy by the topical administration of OT-551: results of a phase II clinical trial","volume":"51","author":"Wong","year":"2010","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib35","doi-asserted-by":"crossref","first-page":"1884","DOI":"10.1097\/IAE.0b013e3182509778","article-title":"Comparison of color fundus photographs and fundus autofluorescence images in measuring geographic atrophy area","volume":"32","author":"Khanifar","year":"2012","journal-title":"Retina"},{"key":"10.1016\/j.ophtha.2015.12.009_bib36","doi-asserted-by":"crossref","first-page":"8181","DOI":"10.1167\/iovs.13-12104","article-title":"A\u00a0longitudinal study of Stargardt disease: quantitative assessment of fundus autofluorescence, progression, and genotype correlations","volume":"54","author":"Fujinami","year":"2013","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib37","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.ajo.2012.01.019","article-title":"Progression of retinal pigment epithelial atrophy in Stargardt disease","volume":"154","author":"McBain","year":"2012","journal-title":"Am J Ophthalmol"},{"key":"10.1016\/j.ophtha.2015.12.009_bib38","doi-asserted-by":"crossref","unstructured":"Strauss RW, Munoz BM, Wolfson Y, et\u00a0al. Assessment of estimated retinal atrophy progression in Stargardt macular dystrophy using spectral-domain optical coherence tomography. Br J Ophthalmol 2015 Nov 14. http:\/\/dx.doi.org\/10.1136\/bjophthalmol-2015-307035. [Epub ahead of print].","DOI":"10.1136\/bjophthalmol-2015-307035"},{"key":"10.1016\/j.ophtha.2015.12.009_bib39","doi-asserted-by":"crossref","first-page":"1572","DOI":"10.1016\/j.ophtha.2014.02.023","article-title":"Proposed lexicon for anatomic landmarks in normal posterior segment spectral-domain optical coherence tomography: the IN*OCT consensus","volume":"121","author":"Staurenghi","year":"2014","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib40","doi-asserted-by":"crossref","first-page":"9581","DOI":"10.1167\/iovs.11-8554","article-title":"Transition zones between healthy and diseased retina in choroideremia (CHM) and Stargardt disease (STGD) as compared to retinitis pigmentosa (RP)","volume":"52","author":"Lazow","year":"2011","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib41","doi-asserted-by":"crossref","first-page":"606","DOI":"10.1056\/NEJMoa1110823","article-title":"Enzymatic vitreolysis with ocriplasmin for vitreomacular traction and macular holes","volume":"367","author":"Stalmans","year":"2012","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.ophtha.2015.12.009_bib42","doi-asserted-by":"crossref","first-page":"3953","DOI":"10.1167\/iovs.08-2657","article-title":"A\u00a0comparison of fundus autofluorescence and retinal structure in patients with Stargardt disease","volume":"50","author":"Gomes","year":"2009","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib43","doi-asserted-by":"crossref","first-page":"841","DOI":"10.1167\/iovs.11-8415","article-title":"Macular function in macular degenerations: repeatability of microperimetry as a potential outcome measure for ABCA4-associated retinopathy trials","volume":"53","author":"Cideciyan","year":"2012","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib44","doi-asserted-by":"crossref","first-page":"e12864","DOI":"10.1371\/journal.pone.0012864","article-title":"Structure-function correlation of the human central retina","volume":"5","author":"Charbel Issa","year":"2010","journal-title":"PLoS One"},{"key":"10.1016\/j.ophtha.2015.12.009_bib45","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1016\/S0161-6420(87)33533-X","article-title":"Visual acuity loss in patients with Stargardt\u2019s macular dystrophy","volume":"94","author":"Fishman","year":"1987","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ophtha.2015.12.009_bib46","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1097\/ICU.0b013e32834bbe7a","article-title":"Effects of smoking on ocular health","volume":"22","author":"Galor","year":"2011","journal-title":"Curr Opin Ophthalmol"},{"key":"10.1016\/j.ophtha.2015.12.009_bib47","doi-asserted-by":"crossref","first-page":"9379","DOI":"10.1167\/iovs.11-8319","article-title":"Quantitative measurements of autofluorescence with the scanning laser ophthalmoscope","volume":"52","author":"Delori","year":"2011","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ophtha.2015.12.009_bib48","doi-asserted-by":"crossref","first-page":"5233","DOI":"10.1073\/pnas.0408892102","article-title":"In\u00a0vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa","volume":"102","author":"Cideciyan","year":"2005","journal-title":"Proc Natl Acad Sci U S A"}],"container-title":["Ophthalmology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0161642015014335?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0161642015014335?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T20:32:57Z","timestamp":1654115577000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0161642015014335"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,4]]},"references-count":48,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2016,4]]}},"alternative-id":["S0161642015014335"],"URL":"https:\/\/doi.org\/10.1016\/j.ophtha.2015.12.009","relation":{},"ISSN":["0161-6420"],"issn-type":[{"value":"0161-6420","type":"print"}],"subject":[],"published":{"date-parts":[[2016,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"The Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) Studies","name":"articletitle","label":"Article Title"},{"value":"Ophthalmology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ophtha.2015.12.009","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}