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RNFL thickness was manually segmented and compared to clinical and imaging parameters including the transfoveal ellipsoid zone (EZ) width, the horizontal diameter of the macular hyperautofluorescent ring. RNFL thickness was compared to 17 age- and sex-matched controls.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>In patients with <jats:italic>RPGR<\/jats:italic>-associated RP, the EZ width (<jats:italic>R<\/jats:italic><jats:sup>2<\/jats:sup>\u2009=\u20090.65), the central hyperautofluorescent ring on AF images (<jats:italic>R<\/jats:italic><jats:sup>2<\/jats:sup>\u2009=\u20090.72), and visual acuity (<jats:italic>R<\/jats:italic><jats:sup>2<\/jats:sup>\u2009=\u20090.68) were negatively correlated with age. In comparison to controls, a significantly (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001) increased global RNFL thickness was identified in <jats:italic>RPGR<\/jats:italic>-associated RP, which was, however, less pronounced in progressed disease as indicated by the EZ width or the diameter of the central hyperautofluorescent ring.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>This study describes retinal characteristics in patients with <jats:italic>RPGR<\/jats:italic>-associated RP including a pronounced peripapillary RNFL thickness compared to healthy controls. These results contribute to\u00a0the knowledge about imaging biomarkers in RP, which might be of interest for therapeutic approaches such as gene replacement therapies.\n<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00417-021-05233-w","type":"journal-article","created":{"date-parts":[[2021,7,21]],"date-time":"2021-07-21T10:04:02Z","timestamp":1626861842000},"page":"3597-3604","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Analysis of imaging biomarkers and retinal nerve fiber layer thickness in RPGR-associated retinitis pigmentosa"],"prefix":"10.1007","volume":"259","author":[{"given":"Theresa H.","family":"Birtel","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7699-2112","authenticated-orcid":false,"given":"Johannes","family":"Birtel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristina","family":"Hess","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amelie C.","family":"Clemens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4416-3421","authenticated-orcid":false,"given":"Moritz","family":"Lindner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philipp","family":"Herrmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank G.","family":"Holz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8215-6655","authenticated-orcid":false,"given":"Martin","family":"Gliem","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,7,21]]},"reference":[{"key":"5233_CR1","doi-asserted-by":"publisher","first-page":"157","DOI":"10.1016\/j.preteyeres.2018.03.005","volume":"66","author":"SK Verbakel","year":"2018","unstructured":"Verbakel SK, van Huet RAC, Boon CJF, den Hollander AI, Collin RWJ, Klaver CCW, Hoyng CB, Roepman R, Klevering BJ (2018) Non-syndromic retinitis pigmentosa. 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The views expressed are those of the authors and not necessarily those of the NHS, the NIHR, or the Department of Health.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Disclaimer"}}]}}