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Graefe's Archive for Clinical and Experimental Ophthalmology
Published in: Graefe's Archive for Clinical and Experimental Ophthalmology 7/2017

01-07-2017 | Retinal Disorders

Electrophysiological assessment for early detection of retinal dysfunction in β-thalassemia major patients

Authors: Maria Dettoraki, Antonis Kattamis, Ioannis Ladas, Konstantinos Maragkos, Chryssanthi Koutsandrea, Klio Chatzistefanou, Konstantinos Laios, Dimitrios Brouzas, Marilita M. Moschos

Published in: Graefe's Archive for Clinical and Experimental Ophthalmology | Issue 7/2017

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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.
Literature
1.
2.
Angelucci E, Brittenham GM, McLaren CE et al (2000) Hepatic iron concentration and total body iron stores in thalassemia major. N Engl J Med 343:327–31CrossRefPubMed
3.
Porter JB, Garbowski M (2014) The pathophysiology of transfusional iron overload. Hematol Oncol Clin North Am 28(683-701):vi
4.
5.
Bhoiwala DL, Dunaief JL (2016) Retinal abnormalities in beta-thalassemia major. Surv Ophthalmol 61:33–50CrossRefPubMed
6.
Di Nicola M, Barteselli G, Dell’Arti L et al (2015) Functional and Structural Abnormalities in Deferoxamine Retinopathy: A Review of the Literature. Biomed Res Int 2015:249617CrossRefPubMedPubMedCentral
7.
Kertes PJ, Lee TK, Coupland SG (2004) The utility of multifocal electroretinography in monitoring drug toxicity: deferoxamine retinopathy. Can J Ophthalmol 39:656–61CrossRefPubMed
8.
9.
10.
11.
Bansal V, Elgarbly I, Ghanchi FD, Atkinson PL (2003) Bull’s eye maculopathy with deferoxamine. Eur J Haematol 70:420–1CrossRefPubMed
12.
13.
McCulloch DL, Marmor MF, Brigell MG et al (2015) ISCEV Standard for full-field clinical electroretinography (2015 update). Doc Ophthalmol 130:1–12CrossRefPubMed
14.
Odom JV, Bach M, Brigell M et al (2010) ISCEV standard for clinical visual evoked potentials (2009 update). Doc Ophthalmol 120:111–9CrossRefPubMed
15.
Hood DC, Bach M, Brigell M et al (2012) ISCEV standard for clinical multifocal electroretinography (mfERG) (2011 edition). Doc Ophthalmol 124:1–13CrossRefPubMed
16.
Viola F, Barteselli G, Dell’arti L et al (2012) Abnormal fundus autofluorescence results of patients in long-term treatment with deferoxamine. Ophthalmology 119:1693–700CrossRefPubMed
17.
Hood DC, Seiple W, Holopigian K, Greenstein V (1997) A comparison of the components of the multifocal and full-field ERGs. Vis Neurosci 14:533–544CrossRefPubMed
18.
19.
Jiang C, Hansen RM, Gee BE et al (1998) Rod and rod mediated function in patients with beta-thalassemia major. Doc Ophthalmol 96:333–45CrossRefPubMed
20.
Marmor MF, Kellner U, Lai TY et al (2011) Revised recommendations on screening for chloroquine and hydroxychloroquine retinopathy. Ophthalmology 118:415–22CrossRefPubMed
21.
Ponjavic V, Andreasson S (2001) Multifocal ERG and full-field ERG in patients on long-term vigabatrin medication. Doc Ophthalmol 102:63–72CrossRefPubMed
22.
Ritter C, Renner AB, Wachtlin J et al (2008) Tamoxifen retinopathy: a case series of clinical and functional data. Ophthalmologe 105:544–9CrossRefPubMed
23.
Dettoraki M, Moschos MM (2016) The Role of Multifocal Electroretinography in the Assessment of Drug-Induced Retinopathy: A Review of the Literature. Ophthalmic Res 56:169–77CrossRefPubMed
24.
Schmidt D, Finke J (2004) Bull’s-Eye Maculopathy with Deferoxamine treatment. Klin Monbl Augenheilkd 221:204–9CrossRefPubMed
25.
El-Fattah El-Shazly AA, Ebeid WM, Elkitkat RS, Deghedy MR (2016) ELECTRORETINOGRAPHIC AND VISUAL-EVOKED POTENTIAL CHANGES IN RELATION TO CHELATION MODALITY IN CHILDREN WITH THALASSEMIA. Retina. doi:10.​1097/​IAE.​0000000000001315​
26.
Koinzer S, Klettner A, Treumer F et al (2012) Correlation of fundus autofluorescence, spectral-domain optical coherence tomography, and microperimetry in late deferoxamine maculopathy. Retin Cases Brief Rep 6:50–5CrossRefPubMed
27.
El Shazly AA, Elkitkat RS, Ebeid WM, Deghedy MR (2016) CORRELATION BETWEEN SUBFOVEAL CHOROIDAL THICKNESS AND FOVEAL THICKNESS IN THALASSEMIC PATIENTS. Retina 36:1767–72CrossRefPubMed
Metadata
Title
Electrophysiological assessment for early detection of retinal dysfunction in β-thalassemia major patients
Authors
Maria Dettoraki
Antonis Kattamis
Ioannis Ladas
Konstantinos Maragkos
Chryssanthi Koutsandrea
Klio Chatzistefanou
Konstantinos Laios
Dimitrios Brouzas
Marilita M. Moschos
Publication date
01-07-2017
Publisher
Springer Berlin Heidelberg
Published in
Graefe's Archive for Clinical and Experimental Ophthalmology / Issue 7/2017
Print ISSN: 0721-832X
Electronic ISSN: 1435-702X
DOI
https://doi.org/10.1007/s00417-017-3650-9

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