Maria Dettoraki1, Antonis Kattamis2, Ioannis Ladas1, Konstantinos Maragkos3, Chryssanthi Koutsandrea1, Klio Chatzistefanou1, Konstantinos Laios4, Dimitrios Brouzas1, Marilita M Moschos5. 1. First Department of Ophthalmology, "G. Gennimatas" General Hospital, Medical School, National and Kapodistrian University of Athens, 154 Mesogion Avenue, Athens, 11527, Greece. 2. Thalassemia Unit, First Department of Pediatrics, Aghia Sofia Children's Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece. 3. Thalassemia Unit, "Hippocratio" General Hospital, Athens, Greece. 4. Department of History of Medicine, Medical School, National and Kapodistrian University of Athens, Athens, Greece. 5. First Department of Ophthalmology, "G. Gennimatas" General Hospital, Medical School, National and Kapodistrian University of Athens, 154 Mesogion Avenue, Athens, 11527, Greece. moschosmarilita@yahoo.fr.
Abstract
PURPOSE: The purpose of this study was to assess the role of various diagnostic tests in early detection of retinal changes in β-thalassemia major patients. METHODS: Thirty-eight visually asymptomatic β-thalassemia major patients receiving regular blood transfusions and iron-chelation therapy with deferoxamine (group A, n = 13), deferasirox (group B, n = 11) or deferoxamine with deferiprone (group C, n = 14) and fourteen age- and sex- matched healthy individuals were included in the study. All participants underwent ophthalmoscopy, full-field electroretinography (ERG), visual evoked potentials (VEP), multifocal electroretinography (mfERG), fundus autofluorescence (FAF) imaging and optical coherence tomography (OCT) scans. RESULTS: Retinal pigment epithelium changes were present in two cases. Scotopic ERG demonstrated decreased a-wave amplitude in groups A, B and C (p = 0.03, p = 0.002 and p = 0.002, respectively) and decreased b-wave amplitude in groups B and C (p = 0.002 and p = 0.01, respectively) compared to controls. Photopic ERG showed delayed b-wave latency in groups A and C (p = 0.03 and p = 0.03, respectively) ERG maximal combined response and VEP response did not differ between groups. MfERG showed reduced retinal response density in ring 1 in groups A, B, C (p < 0.001, p < 0.001, p = 0.001, respectively) and ring 2 in group B (p = 0.02) and delayed latency in ring 5 in groups A and B (p = 0.04 and p = 0.04, respectively). Abnormal FAF images appeared in three cases and OCT abnormalities in one case, whereas no changes were observed in controls (p = 0.55 and p = 1.00, respectively). CONCLUSIONS: Full-field ERG and mfERG are more sensitive tools for detecting early retinal changes in β-thalassemia patients compared with ophthalmoscopy, VEP, FAF imaging and OCT scans.
PURPOSE: The purpose of this study was to assess the role of various diagnostic tests in early detection of retinal changes in β-thalassemia major patients. METHODS: Thirty-eight visually asymptomatic β-thalassemia major patients receiving regular blood transfusions and iron-chelation therapy with deferoxamine (group A, n = 13), deferasirox (group B, n = 11) or deferoxamine with deferiprone (group C, n = 14) and fourteen age- and sex- matched healthy individuals were included in the study. All participants underwent ophthalmoscopy, full-field electroretinography (ERG), visual evoked potentials (VEP), multifocal electroretinography (mfERG), fundus autofluorescence (FAF) imaging and optical coherence tomography (OCT) scans. RESULTS: Retinal pigment epithelium changes were present in two cases. Scotopic ERG demonstrated decreased a-wave amplitude in groups A, B and C (p = 0.03, p = 0.002 and p = 0.002, respectively) and decreased b-wave amplitude in groups B and C (p = 0.002 and p = 0.01, respectively) compared to controls. Photopic ERG showed delayed b-wave latency in groups A and C (p = 0.03 and p = 0.03, respectively) ERG maximal combined response and VEP response did not differ between groups. MfERG showed reduced retinal response density in ring 1 in groups A, B, C (p < 0.001, p < 0.001, p = 0.001, respectively) and ring 2 in group B (p = 0.02) and delayed latency in ring 5 in groups A and B (p = 0.04 and p = 0.04, respectively). Abnormal FAF images appeared in three cases and OCT abnormalities in one case, whereas no changes were observed in controls (p = 0.55 and p = 1.00, respectively). CONCLUSIONS: Full-field ERG and mfERG are more sensitive tools for detecting early retinal changes in β-thalassemia patients compared with ophthalmoscopy, VEP, FAF imaging and OCT scans.
Authors: E Angelucci; G M Brittenham; C E McLaren; M Ripalti; D Baronciani; C Giardini; M Galimberti; P Polchi; G Lucarelli Journal: N Engl J Med Date: 2000-08-03 Impact factor: 91.245
Authors: J Vernon Odom; Michael Bach; Mitchell Brigell; Graham E Holder; Daphne L McCulloch; Alma Patrizia Tormene Journal: Doc Ophthalmol Date: 2009-10-14 Impact factor: 2.379
Authors: Donald C Hood; Michael Bach; Mitchell Brigell; David Keating; Mineo Kondo; Jonathan S Lyons; Michael F Marmor; Daphne L McCulloch; Anja M Palmowski-Wolfe Journal: Doc Ophthalmol Date: 2011-10-30 Impact factor: 2.379
Authors: Francesco Viola; Giulio Barteselli; Laura Dell'arti; Diego Vezzola; Edoardo Villani; Chiara Mapelli; Laura Zanaboni; Maria D Cappellini; Roberto Ratiglia Journal: Ophthalmology Date: 2012-04-04 Impact factor: 12.079
Authors: Constantine D Georgakopoulos; Foteini Tsapardoni; Elli V Kostopoulou; Olga E Makri Journal: BMC Ophthalmol Date: 2018-09-12 Impact factor: 2.209