@article{2998294,
    title = "Electrophysiological assessment for early detection of retinal dysfunction in β-thalassemia major patients",
    author = "Dettoraki, M. and Kattamis, A. and Ladas, I. and Maragkos, K. and Koutsandrea, C. and Chatzistefanou, K. and Laios, K. and Brouzas, D. and Moschos, M.M.",
    journal = "Graefe's Archive for Clinical and Experimental Ophthalmology",
    year = "2017",
    volume = "255",
    number = "7",
    pages = "1349-1358",
    publisher = "Springer-Verlag",
    issn = "0721-832X, 1435-702X",
    doi = "10.1007/s00417-017-3650-9",
    keywords = "deferasirox;  deferiprone;  deferoxamine, A wave;  adult;  angioid streak;  Article;  autofluorescence imaging;  B wave;  beta thalassemia;  blood transfusion;  chelation therapy;  clinical article;  confocal scanning laser ophthalmoscope;  controlled clinical trial;  controlled study;  electroretinography;  eye toxicity;  female;  fundus autofluorescence;  human;  imaging system;  latent period;  male;  multifocal electroretinography;  ophthalmoscopy;  optical coherence tomography;  optical coherence tomography device;  priority journal;  retina pigment degeneration;  retinal pigment epithelium;  thalassemia major;  visual evoked potential;  beta thalassemia;  complication;  diagnostic imaging;  early diagnosis;  electroretinography;  eye fundus;  fluorescence angiography;  middle aged;  ophthalmoscopy;  optical coherence tomography;  pathophysiology;  procedures;  retina;  retina disease;  visual acuity;  visual evoked potential, Adult;  beta-Thalassemia;  Early Diagnosis;  Electroretinography;  Evoked Potentials, Visual;  Female;  Fluorescein Angiography;  Fundus Oculi;  Humans;  Male;  Middle Aged;  Ophthalmoscopy;  Retina;  Retinal Diseases;  Tomography, Optical Coherence;  Visual Acuity",
    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. © 2017, Springer-Verlag Berlin Heidelberg."
}