{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T15:29:19Z","timestamp":1759332559615,"version":"3.40.5"},"reference-count":24,"publisher":"S. Karger AG","issue":"1","license":[{"start":{"date-parts":[[2021,9,1]],"date-time":"2021-09-01T00:00:00Z","timestamp":1630454400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2021,9,1]],"date-time":"2021-09-01T00:00:00Z","timestamp":1630454400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Ophthalmologica"],"published-print":{"date-parts":[[2022]]},"abstract":"<b><i>Purpose:<\/i><\/b> The aim of this study was to evaluate microvascular abnormalities of patients with Alport syndrome (AS) using optical coherence tomography angiography (OCT-A) quantitative biomarkers. <b><i>Methods:<\/i><\/b> This was cross sectional, prospective evaluation of consecutive patients with AS and healthy subjects. AS diagnosis was performed by the genetic test. All participants underwent a retinal vasculature evaluation by spectral-domain optical coherence tomography (SD-OCT) and OCT-A of the macula. Quantitative analysis included whole vascular density, foveal avascular zone area, fractal dimension (FD), and lacunarity (LAC). <b><i>Results:<\/i><\/b> Ninety-four eyes were included in this study, 45 eyes from patients with AS and 49 eyes from healthy subjects. The pathogenic mutation in the <i>COL4A5<\/i> gene on the chromosome X was found in 14 patients; the pathogenic autosomal recessive mutations in the <i>COL4A3<\/i> gene were found in 9 patients. Quantitative evaluation demonstrated a significant difference between AS and healthy subjects on LAC of the superficial capillary plexus and deep capillary plexus (DCP) (<i>p<\/i> &#x3c; 0.001 and <i>p<\/i> &#x3c; 0.001, respectively) and on FD in the DCP (<i>p<\/i> &#x3c; 0.001). <b><i>Conclusion:<\/i><\/b> The DCP Alport patients have a higher vessel nonuniformity than DCP of healthy subjects. We hypothesize that endothelial cell lesion in the setting of low resistance at the DCP circuit could lead to long-term structural disorganization. <\/jats:p>","DOI":"10.1159\/000519242","type":"journal-article","created":{"date-parts":[[2021,9,1]],"date-time":"2021-09-01T21:00:32Z","timestamp":1630530032000},"page":"41-48","source":"Crossref","is-referenced-by-count":1,"title":["Quantitative Optical Coherence Tomography Angiography Biomarkers for Alport Syndrome"],"prefix":"10.1159","volume":"245","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8519-8786","authenticated-orcid":false,"given":"Ana Maria","family":"Cunha","sequence":"first","affiliation":[]},{"given":"Daniela","family":"Teixeira","sequence":"additional","affiliation":[]},{"given":"Diogo","family":"Cabral","sequence":"additional","affiliation":[]},{"given":"S\u00e9rgio","family":"Estrela-Silva","sequence":"additional","affiliation":[]},{"given":"Fernando","family":"Falc\u00e3o-Reis","sequence":"additional","affiliation":[]},{"given":"\u00c2ngela","family":"Carneiro","sequence":"additional","affiliation":[]}],"member":"127","published-online":{"date-parts":[[2021,9,1]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.1.3454.504"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1002\/humu.9349"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1038\/nrneph.2012.259"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1172\/JCI119431"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1985.63"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-008-0759-4"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.08-3323"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.2009.158089"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1364\/oe.15.004083"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1080\/17469899.2016.1209409"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.3390\/app10248865"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajo.2015.10.008"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keab258"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1016\/j.oret.2018.04.016"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2011.01.028"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.61.2.5"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.15-18793"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-018-28530-7"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.bioeng.6.040803.140100"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1016\/j.physd.2007.09.017"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1002\/humu.21401"},{"key":"ref22","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.10581014"},{"key":"ref23","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2016.09.016"},{"key":"ref24","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.2008.142869"}],"container-title":["Ophthalmologica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/519242","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/519242","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T15:23:40Z","timestamp":1745421820000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000519242"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,9,1]]},"references-count":24,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2022,3,1]]},"published-print":{"date-parts":[[2022,3,8]]}},"URL":"https:\/\/doi.org\/10.1159\/000519242","archive":["Portico"],"relation":{},"ISSN":["0030-3755","1423-0267"],"issn-type":[{"type":"print","value":"0030-3755"},{"type":"electronic","value":"1423-0267"}],"subject":[],"published":{"date-parts":[[2021,9,1]]}}}