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Deferoxamine-induced electronegative ERG responses

  • Clinical Case Report
  • Published:
Documenta Ophthalmologica Aims and scope Submit manuscript

Abstract

Purpose

To report a case of deferoxamine-induced retinopathy characterized by electroretinography (ERG), optical coherence tomography angiography (OCT-A), and other multimodal imaging.

Methods

This is an observational case report of one patient. Full-field ERG was performed. OCT-A, spectral-domain optical coherence tomography (SD-OCT), color fundus photography, and fundus autofluorescence were used to characterize the retinopathy induced by deferoxamine use.

Results

A 64-year-old man with a history of β-thalassemia intermedia presented with worsening visual acuity, nyctalopia, and electronegative ERG. OCT-A revealed atrophy of the choriocapillaris in areas of hypoautofluorescence, corresponding to regions of retinal atrophy. SD-OCT showed disruption of the ellipsoid zone, granular hyperreflective deposits within the retinal pigment epithelium, thinning of the retinal layers, and extensive choroidal sclerosis and atrophy of the retinal pigment epithelium.

Conclusion

Deferoxamine-induced retinopathy can manifest with electronegative maximal ERG responses, and OCT-A can be used to detect deferoxamine toxicity.

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Acknowledgements

The Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory are supported by the National Institutes of Health [P30EY019007, R01EY018213, R01EY024698, R01EY026682, R21AG050437], National Cancer Institute Core [5P30CA013696], the Research to Prevent Blindness (RPB) Physician-Scientist Award, unrestricted funds from RPB, New York, NY, USA. R. J. is supported by the RPB medical student eye research fellowship. S. H. T. is a member of the RD-CURE Consortium and is supported by the Tistou and Charlotte Kerstan Foundation, the Schneeweiss Stem Cell Fund, New York State [C029572], and the Gebroe Family Foundation. VBM is supported by NIH Grants [R01EY024665, R01EY025225, R01EY024698, R21AG050437, P30EY026877], and Research to Prevent Blindness (RPB).

Author information

Authors and Affiliations

  1. Department of Ophthalmology, Columbia University, New York, NY, USA

    Ruben Jauregui, Karen Sophia Park & Stephen H. Tsang

  2. Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory, New York, NY, USA

    Ruben Jauregui, Karen Sophia Park & Stephen H. Tsang

  3. Weill Cornell Medical College, New York, NY, USA

    Ruben Jauregui

  4. Department of Pediatrics, University of Iowa, Iowa City, IA, USA

    Alexander G. Bassuk

  5. Byers Eye Institute, Omics Laboratory, Department of Ophthalmology, Stanford University School of Medicine, Palo Alto, CA, USA

    Vinit B. Mahajan

  6. Palo Alto Veterans Administration, Palo Alto, CA, USA

    Vinit B. Mahajan

  7. Department of Pathology and Cell Biology, Stem Cell Initiative (CSCI), Institute of Human Nutrition, College of Physicians and Surgeons, Columbia University, New York, NY, USA

    Stephen H. Tsang

  8. Edward S. Harkness Eye Institute, Columbia University Medical Center, New York, NY, 10032, USA

    Stephen H. Tsang

Authors
  1. Ruben Jauregui
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  2. Karen Sophia Park
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  3. Alexander G. Bassuk
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  4. Vinit B. Mahajan
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  5. Stephen H. Tsang
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Contributions

R.J. collected and interpreted patient data and images. R.J. and K.S.P. composed the manuscript. S.H.T., A.G.B., and V.B.M. conceived the experimental design and approved the final interpretation of the data.

Corresponding author

Correspondence to Stephen H. Tsang.

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Conflicts of interest

The authors declare that they have no conflicts of interest.

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The patient has given informed consent for submission of this case report to the journal.

Statement of human rights

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Statement on the welfare of animals

This article does not contain any studies with animals performed by any of the authors.

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Jauregui, R., Park, K.S., Bassuk, A.G. et al. Deferoxamine-induced electronegative ERG responses. Doc Ophthalmol 137, 15–23 (2018). https://doi.org/10.1007/s10633-018-9640-3

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  • DOI: https://doi.org/10.1007/s10633-018-9640-3

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