Electroretinographic and Visual-Evoked Potential Changes in Relation to Chelation Modality in Children With Thalassemia

El-Shazly, Amany Abd El-Fattah; Ebeid, Weam Mohamed; Elkitkat, Rania Serag; Deghedy, Marwa Reda

doi:10.1097/iae.0000000000001315

Cited by 8 publications

(9 citation statements)

References 22 publications

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“…Prior studies have evaluated electrophysiologic changes of iron chelation therapy in asymptomatic patients who lacked apparent fundoscopic findings, noting decreased amplitudes compared with controls. 7 Therefore, mfERG may provide a sensitive early diagnostic test for patients with suspected toxicity. 7,9 Management of iron chelator toxicity involves multidisciplinary decision making and weighing the risks and benefits of continued therapy with the need to prevent morbidity and mortality from iron overload.…”

Section: Discussionmentioning

confidence: 99%

Multimodal imaging in deferasirox-mediated retinopathy

Caranfa,

Carrera,

Marmalidou

et al. 2024

European Journal of Ophthalmology

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Introduction Deferasirox is the only iron chelator available in oral formulation and a rare cause of pigmentary retinopathy. We report the first case of multimodal imaging in an adult with deferasirox retinopathy. Methods Case report and literature review, with search terms including deferasirox retinopathy and deferasirox toxicity. Results A 63-year-old man with end stage renal disease and transfusion-dependent anemia on deferasirox for one year presented with asymptomatic pigment epitheliopathy. Optical coherence tomography featured outer retinal and retinal pigment epithelial discontinuity corresponding to hypoautofluorescence on fundus autofluorescence and blocking on fluorescein angiography. Multifocal electroretinography revealed subtle reduction in all amplitudes. Conclusions Retinal examinations should be considered for patients requiring chronic administration of deferasirox.

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Section: Discussionmentioning

confidence: 99%

Multimodal imaging in deferasirox-mediated retinopathy

Caranfa,

Carrera,

Marmalidou

et al. 2024

European Journal of Ophthalmology

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“…That study also found that DFO was more toxic to the macula than oral chelators like DFX. 21 Taneja et al 22 observed ocular changes caused by the different treatment regimens recommended for patients with TM over the course of a year and reported that changes occurred in proportion to the increase in serum ferritin levels. Similarly, Cennamo et al 23 reported, in a study that used OCTA to evaluate vascular changes in retinal and choriocapillaris perfusion, that high serum ferritin levels in TM patients may be significantly associated with choriocapillary hypoperfusion, but Kazancı et al 10 reported that there was no relationship between ferritin levels and ocular changes when comparing TM patients with a control group in a study that also evaluated vascular parameters using OCTA.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of parafoveal and peripapillary vascular densities using optical coherence tomography angiography in children with thalassemia major

et al. 2023

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Background: Thalassemia major (TM) is an inherited anaemia caused by faulty haemoglobin synthesis. Reducing serum iron levels using iron chelating agents is an important step in the treatment of TM, and the effects on the eye of both the disease and these agents can be determined by regular eye examination. Objectives: We evaluated macular and optic nerve vascular densities in children with TM and compared the results with healthy controls using optical coherence tomography angiography (OCTA). Design: This is a prospective study. Methods: A total of 30 children with TM and 30 healthy controls were included in the study. The area of the foveal avascular zone (FAZ) and the vascular densities (VD) of the optic nerve head (ONH), radial peripapillary capillary (RPC) and deep and superficial retinal vascular networks were measured using OCTA. Results: A statistically significant decrease in VD was observed in the whole image and the parafovea, superior hemi, superior and inferior parts of the superficial capillary plexus and in the whole image and the superior regions of the deep capillary plexus in the TM patient group compared with the control group ( p < 0.05). A significant decrease in VD was also observed in the whole image and the inside disc, peripapillary, nasal, inferonasal and temporal regions of the ONH and in the whole image and the inside disc, peripapillary and inferonasal regions of the RPC network in patients with TM ( p < 0.05). A significant positive correlation was observed between both serum ferritin levels and deferasirox dosage, on one hand, and both the superficial ( p = 0.023 and p = 0.002, respectively) and deep FAZs ( p = 0.015 and p = 0.045, respectively), on the other hand. A negative correlation was also found between the deferasirox dosage and the VDs of the superficial ( p = 0.010) and deep ( p = 0.001) foveal plexuses. Conclusion: Retinal VD and FAZ are affected in patients with TM. OCTA, which can noninvasively measure retinal VD in patients with TM, may be a useful tool for the early detection of retinal microvascular changes that may occur during the course of the disease.

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“…Studies have been reported that visual evoked potential values are altered from the normal range in beta-thalassemic individuals due to iron overload and on the other hand neurotoxicity due to DFO is another culprit which causes disrupted visual evoked potential [24][25][27][28] according to some reports.…”

Section: Visual Evoked Potentials (Vep)mentioning

confidence: 99%

Neurological Deficits among Beta-Thalassemia Patients and its Possible Therapeutic Intervention : A Comprehensive Review

Shah

Idrees

Shah

et al. 2020

J Dow Univ Health Sci

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On the other hand, imaging devices like magnetic resonance imaging (MRI) are employed to evaluate the iron chelation therapeutic effects based on signal intensity on T1 and T2 weighted sequences, as lower 8 signal intensity suggests iron overload. Such intervention expedites the assessment of iron levels in the body before the onset of dysfunction of organs and 10,11 hemosiderosis could set in. Many studies showed that beta thalassemia affects the neurological conditions of those individuals who suffer 12,15 from this disease.However, these neurological anomalies have not been fully compiled or discussed yet. In this article, we will discuss these neurological impairments caused by beta-thalassemia and their possible curative therapy to alleviate some of these conditions. EXTRAMEDULLARY HEMATOPOIESISExtramedullary hematopoiesis (EMH) is a homeostatic phenomenon that arises among chronic anemic patients in which hematopoietic foci are formed other than bone marrow to compensate the circulatory 16-18 needs. In 1954, Gatto and their colleagues reported

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Electroretinographic and Visual-Evoked Potential Changes in Relation to Chelation Modality in Children With Thalassemia

Cited by 8 publications

References 22 publications

Multimodal imaging in deferasirox-mediated retinopathy

Multimodal imaging in deferasirox-mediated retinopathy

Evaluation of parafoveal and peripapillary vascular densities using optical coherence tomography angiography in children with thalassemia major

Neurological Deficits among Beta-Thalassemia Patients and its Possible Therapeutic Intervention : A Comprehensive Review

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