{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:34:33Z","timestamp":1787027673445,"version":"build-2736575974"},"reference-count":43,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2021,3,28]],"date-time":"2021-03-28T00:00:00Z","timestamp":1616889600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biology"],"abstract":"<jats:p>In this cross-sectional study we assessed the vascular alterations in retinal and choriocapillaris perfusion in patients affected by \u03b2-thalassemia, by means of optical coherence tomography angiography (OCTA). A total of 124 eyes of 62 patients (mean age 44.74 \u00b1 5.79 years old) affected by \u03b2-thalassemia (transfusion dependent thalassemia (TDT), non-transfusion dependent thalassemia (NTDT) and minor) were compared to 40 eyes of twenty healthy subjects. We evaluated the vessel density (VD) in superficial capillary plexus, deep capillary plexus, radial peripapillary capillary, choriocapillaris and the foveal avascular zone area. The TDT group showed a statistically significant reduction in retinal and choriocapillaris VD respect to controls and the other groups (p &lt; 0.05). No statistically significant difference was found in OCTA parameters between \u03b2-thalassemia minor and controls. The NTDT group showed a significant reduction in VD in deep capillary plexus respect to controls and \u03b2-thalassemia minor. Significant negative correlations were shown in TDT group between foveal avascular zone and hemoglobin (r = \u22120.437, p = 0.044) and between ferritin levels and VD of choriocapillaris (r = \u22120.431, p = 0.038). The OCTA parameters provided a deeper understanding on retinal and choriocapillaris vascular impairment affected by tissue hypoxia levels and the oxidative stress in different clinical phenotypes of the \u03b2-thalassemia.<\/jats:p>","DOI":"10.3390\/biology10040276","type":"journal-article","created":{"date-parts":[[2021,3,28]],"date-time":"2021-03-28T22:09:01Z","timestamp":1616969341000},"page":"276","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Retinal and Choriocapillaris Vascular Changes in Patients Affected by Different Clinical Phenotypes of \u03b2-Thalassemia: An Optical Coherence Tomography Angiography Study"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4253-1929","authenticated-orcid":false,"given":"Gilda","family":"Cennamo","sequence":"first","affiliation":[{"name":"Eye Clinic, Public Health Department, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1423-9621","authenticated-orcid":false,"given":"Daniela","family":"Montorio","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Reproductive Sciences and Dentistry, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuliano","family":"Mazzella","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Reproductive Sciences and Dentistry, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7361-3308","authenticated-orcid":false,"given":"Paolo","family":"Ricchi","sequence":"additional","affiliation":[{"name":"Rare Blood Cell Disease Unit, \u201cCardarelli\u201d Hospital, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Costantini","sequence":"additional","affiliation":[{"name":"Rare Blood Cell Disease Unit, \u201cCardarelli\u201d Hospital, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Spasiano","sequence":"additional","affiliation":[{"name":"Rare Blood Cell Disease Unit, \u201cCardarelli\u201d Hospital, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aldo","family":"Filosa","sequence":"additional","affiliation":[{"name":"Rare Blood Cell Disease Unit, \u201cCardarelli\u201d Hospital, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Storino","sequence":"additional","affiliation":[{"name":"CEINGE-Biotecnologie Avanzate, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Aquila","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Medical Biotechnology, School of Medicine, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fausto","family":"Tranfa","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Reproductive Sciences and Dentistry, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1664-1657","authenticated-orcid":false,"given":"Michela","family":"Grosso","sequence":"additional","affiliation":[{"name":"CEINGE-Biotecnologie Avanzate, 80131 Naples, Italy"},{"name":"Department of Molecular Medicine and Medical Biotechnology, School of Medicine, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,3,28]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1196\/annals.1345.003","article-title":"Changing patterns of thalassemia worldwide","volume":"1054","author":"Vichinsky","year":"2005","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Liaska, A., Petrou, P., Georgakopoulos, C.D., Diamanti, R., Papaconstantinou, D., Kanakis, M.G., and Georgalas, I. (2016). \u03b2-Thalassemia and ocular implications: A systematic review. BMC Ophthalmol., 16.","DOI":"10.1186\/s12886-016-0285-2"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1111\/j.1751-553X.2007.00946.x","article-title":"Delayed decline of gamma-globin expression in infant age associated with the presence of Ggamma-158 (C-->T) polymorphism","volume":"30","author":"Grosso","year":"2008","journal-title":"Int. J. Lab. Hematol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1111\/bjh.15595","article-title":"MRI multicentre prospective survey in thalassaemia major patients treated with deferasirox versus deferiprone and desferrioxamine","volume":"183","author":"Pepe","year":"2018","journal-title":"Br. J. Haematol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1111\/j.1600-0609.2010.01447.x","article-title":"Combined chelation therapy in thalassemia major with deferiprone and desferrioxamine: A retrospective study","volume":"85","author":"Ricchi","year":"2010","journal-title":"Eur. J. Haematol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1159\/000507369","article-title":"Urinary Metabolic Profile of Patients with Transfusion-Dependent \u03b2-Thalassemia Major Undergoing Deferasirox Therapy","volume":"45","author":"Capolongo","year":"2020","journal-title":"Kidney Blood Press Res."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1135","DOI":"10.1056\/NEJMra050436","article-title":"Beta-thalassemia","volume":"353","author":"Rund","year":"2005","journal-title":"N. Engl. J. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"125","DOI":"10.4103\/0301-4738.60083","article-title":"Multiple transfused thalassemia major: Ocular manifestations in a hospital-based population","volume":"58","author":"Taneja","year":"2010","journal-title":"Indian J. Ophthalmol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1186\/1471-2415-14-88","article-title":"Deferoxamine retinopathy:spectral domain-optical coherence tomography findings","volume":"2","author":"Wu","year":"2014","journal-title":"BMC Ophthalmol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1136\/bjo.70.5.373","article-title":"Ocular toxicity of desferrioxamine: Light microscopic histochemical and ultrastructural findings","volume":"70","author":"Rahi","year":"1986","journal-title":"Br. J. Ophthalmol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1136\/bjo.68.12.873","article-title":"Ocular changes in patients undergoing long-term desferrioxamine treatment","volume":"68","author":"Arden","year":"1984","journal-title":"Br. J. Ophthalmol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1182\/blood.V99.1.30","article-title":"Elastic tissue abnormalities resembling pseudoxanthoma elasticum in beta thalassemia and the sickling syndromes","volume":"99","author":"Aessopos","year":"2002","journal-title":"Blood"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1016\/S0037-1963(01)90028-9","article-title":"Pathophysiology of alpha- and beta-thalassemia: Therapeutic implications","volume":"38","author":"Rund","year":"2001","journal-title":"Semin. Hematol"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"583","DOI":"10.3109\/08880018.2014.937884","article-title":"Guidelines for diagnosis and management of Beta-thalassemia intermedia","volume":"31","author":"Karimi","year":"2014","journal-title":"Pediatr. Hematol. Oncol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.blre.2012.12.003","article-title":"Regulation of erythropoiesis by hypoxia-inducible factors","volume":"27","author":"Haase","year":"2013","journal-title":"Blood Rev."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.cccn.2003.07.010","article-title":"Oxidant, antioxidant status and metabolic data in patients with betathalassemia","volume":"338","author":"Laradi","year":"2003","journal-title":"Clin. Chim. Acta"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2196","DOI":"10.1097\/IAE.0000000000000635","article-title":"In vivo characterization of retinal vascularization morphology using optical coherence tomography angiography","volume":"35","author":"Savastano","year":"2015","journal-title":"Retina"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1016\/j.ajo.2016.07.023","article-title":"Choriocapillaris Flow Features Follow a Power Law Distribution: Implications for Characterization and Mechanisms of Disease Progression","volume":"170","author":"Spaide","year":"2016","journal-title":"Am. J. Ophthalmol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1787","DOI":"10.1007\/s00417-017-3709-7","article-title":"Optical coherence tomography angiography in pre-perimetric open-angle glaucoma","volume":"255","author":"Cennamo","year":"2017","journal-title":"Graefes Arch Clin. Exp. Ophthalmol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"4710","DOI":"10.1364\/OE.20.004710","article-title":"Split spectrum amplitude-decorrelation angiography with optical coherence tomography","volume":"20","author":"Jia","year":"2012","journal-title":"Opt. Express"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1159\/000442770","article-title":"Optical Coherence Tomography Angiography Using the Optovue Device","volume":"56","author":"Huang","year":"2016","journal-title":"Dev. Ophthalmol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/j.ajo.2016.08.030","article-title":"Regional Comparisons of Optical Coherence Tomography Angiography Vessel Density in Primary Open-Angle Glaucoma","volume":"171","author":"Rao","year":"2016","journal-title":"Am. J. Ophthalmol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"2277","DOI":"10.1097\/IAE.0000000000001902","article-title":"Optical coherence tomography angiography to detect macular capillary ischemia in patients with inner retinal changes after resolved diabetic","volume":"38","author":"Moein","year":"2018","journal-title":"Retina"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1111\/j.1365-2141.2005.05734.x","article-title":"Quantitative evaluation of oxidative stress status on peripheral blood in beta-thalassaemic patients by means of electron paramagnetic resonance spectroscopy","volume":"131","author":"Filosa","year":"2005","journal-title":"Br. J. Haematol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1002\/(SICI)1098-1136(199606)17:2<83::AID-GLIA1>3.0.CO;2-7","article-title":"Relationship of iron to oligodendrocytes and myelination","volume":"17","author":"Connor","year":"1996","journal-title":"Glia"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"740","DOI":"10.3945\/jn.110.131169","article-title":"Early iron deficiency has brain and behavior effects consistent with dopaminergic dysfunction","volume":"141","author":"Lozoff","year":"2011","journal-title":"J. Nutr."},{"key":"ref_27","first-page":"1141","article-title":"Antioxidant status in children with homozygous beta thalassemia","volume":"42","author":"Dhawan","year":"2005","journal-title":"Indian Pediatr."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"22","DOI":"10.3109\/08820538.2013.839811","article-title":"Retinal fiber layerthickness in children with thalessemia major and iron deficiency anemia","volume":"29","author":"Aksoy","year":"2014","journal-title":"Semin. Ophthalmol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1111\/j.1749-6632.2010.05585.x","article-title":"Hepcidin in beta-thalassemia","volume":"1202","author":"Nemeth","year":"2010","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1016\/j.sjopt.2017.10.001","article-title":"Retinal nerve fiber layer thickness in children with \u03b2-thalassemia major","volume":"31","author":"Uzun","year":"2017","journal-title":"Saudi. J. Ophthalmol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"217","DOI":"10.5301\/ejo.5000206","article-title":"Peripapillary retinal nerve fiber layer thickness in children with iron deficiency anemia","volume":"23","author":"Turkyilmaz","year":"2013","journal-title":"Eur. J. Ophthalmol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"2050312116661683","DOI":"10.1177\/2050312116661683","article-title":"Retinal nerve fiber layer thickness and retinal vessel calibers in children with thalassemia minor","volume":"4","author":"Acer","year":"2016","journal-title":"SAGE Open Med."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1016\/j.ijcard.2004.12.025","article-title":"Endothelial dysfunction and inflammatory process in transfusion-dependent patients with beta-thalassemia major","volume":"105","author":"Aggeli","year":"2005","journal-title":"Int. J. Cardiol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"42201","DOI":"10.1038\/srep42201","article-title":"Detailed Vascular Anatomy of the Human Retina by Projection-Resolved Optical Coherence Tomography Angiography","volume":"7","author":"Campbell","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"2188","DOI":"10.1097\/IAE.0000000000000847","article-title":"Correlation of Foveal Avascular Zone Size with Foveal Morphology in Normal Eyes Using Optical Coherence Tomography Angiography","volume":"35","author":"Samara","year":"2015","journal-title":"Retina"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1177\/1120672119899375","article-title":"Optical coherence tomography angiography findings in young \u03b2-thalassemia patients","volume":"30","author":"Korkmaz","year":"2020","journal-title":"Eur. J. Ophthalmol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"3887","DOI":"10.1167\/iovs.19-27291","article-title":"A Pilot Optical Coherence Tomography Angiography Study on Superficial and Deep Capillary Plexus Foveal Avascular Zone in Patients with Beta-Thalassemia Major","volume":"60","author":"Georgalas","year":"2019","journal-title":"Investig. Ophthalmol. Vis. Sci."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1186\/s12348-017-0138-z","article-title":"Pearls and pitfalls of optical coherence tomography angiography in the multimodal evaluation of uveitis","volume":"7","author":"Pichi","year":"2017","journal-title":"J. Ophthalmic. Inflamm. Infect."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"1781","DOI":"10.1097\/IAE.0b013e31825db73d","article-title":"Macular and peripapillary choroidal thickness in diabetic patients","volume":"32","author":"Vujosevic","year":"2012","journal-title":"Retina"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/j.survophthal.2015.08.005","article-title":"Retinal abnormalities in \u03b2-thalassemia major","volume":"6","author":"Bhoiwala","year":"2016","journal-title":"Surv. Ophthalmol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1097\/ICB.0000000000000013","article-title":"Spectral domain optical coherence tomography findings in early deferoxamine maculopathy: Report of two cases","volume":"8","author":"Alami","year":"2014","journal-title":"Retin. Cases Brief Rep."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1428","DOI":"10.1097\/IAE.0000000000000073","article-title":"Multimodal imaging in deferoxamine retinopathy","volume":"34","author":"Viola","year":"2014","journal-title":"Retina"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"1693","DOI":"10.1016\/j.ophtha.2012.01.039","article-title":"Abnormal fundus autofluorescence results of patients in long-term treatment with deferoxamine","volume":"119","author":"Viola","year":"2012","journal-title":"Ophthalmology"}],"container-title":["Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2079-7737\/10\/4\/276\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,13]],"date-time":"2025-10-13T13:33:01Z","timestamp":1760362381000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2079-7737\/10\/4\/276"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,28]]},"references-count":43,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2021,4]]}},"alternative-id":["biology10040276"],"URL":"https:\/\/doi.org\/10.3390\/biology10040276","relation":{},"ISSN":["2079-7737"],"issn-type":[{"value":"2079-7737","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,3,28]]}}}