7
views
0
recommends
+1 Recommend
1 collections
    0
    shares
      Are you tired of sifting through news that doesn't interest you?
      Personalize your Karger newsletter today and get only the news that matters to you!

      Sign up

      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Desferrioxamine-Related Pseudo-Vitelliform Dystrophy and the Effect of Anti-Vascular Endothelial Growth Factor

      case-report

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          We report a case of a 72-year-old female who developed bilateral pseudo-vitelliform dystrophy after taking desferrioxamine for the treatment of chronic iron overload. The patient then developed a right superior hemiretinal vein occlusion associated with intraretinal fluid in the right eye and was treated with monthly intravitreal aflibercept injections for 3 months followed by as required treatment. In addition to the intraretinal fluid responding to anti-VEGF treatment, there was a reduction in the size of the pseudo-vitelliform subfoveal deposit height, which was not seen in the untreated eye. Our case of an uncommon presentation of desferrioxamine-related maculopathy associated with a vein occlusion and the changes associated with intravitreal anti-VEGF treatment may help with the potential hypotheses of the pathophysiology of desferrioxamine-related pseudo-vitelliform retinal lesions and help with the potential future treatments of the condition.

          Related collections

          Most cited references11

          • Record: found
          • Abstract: found
          • Article: found
          Is Open Access

          Macular dystrophies: clinical and imaging features, molecular genetics and therapeutic options

          Macular dystrophies (MDs) consist of a heterogeneous group of disorders that are characterised by bilateral symmetrical central visual loss. Advances in genetic testing over the last decade have led to improved knowledge of the underlying molecular basis. The developments in high-resolution multimodal retinal imaging have also transformed our ability to make accurate and more timely diagnoses and more sensitive quantitative assessment of disease progression, and allowed the design of optimised clinical trial endpoints for novel therapeutic interventions. The aim of this review was to provide an update on MDs, including Stargardt disease, Best disease, X-linked r etinoschisis, pattern dystrophy, Sorsby fundus dystrophy and autosomal dominant drusen. It highlights the range of innovations in retinal imaging, genotype–phenotype and structure–function associations, animal models of disease and the multiple treatment strategies that are currently in clinical trial or planned in the near future, which are anticipated to lead to significant changes in the management of patients with MDs.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Ocular toxicity of desferrioxamine: light microscopic histochemical and ultrastructural findings.

            This study documents for the first time light and electron microscopical changes in the retinal pigment epithelium (RPE) following treatment with high dose desferrioxamine for systemic iron overload. The changes include loss of microvilli from the apical surface, patchy depigmentation, vacuolation of the cytoplasm, swelling and calcification of mitochondria, and disorganisation of the plasma membrane. In addition, Bruch's membrane overlying degenerate RPE cells appeared abnormally thickened owing to the accumulation of large amounts of mature elastic fibres, pre-elastic oxytalan, and long spacing collagen. The specificity of these changes and the mechanism of toxicity are discussed.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Depletion of trace elements and acute ocular toxicity induced by desferrioxamine in patients with thalassaemia.

              High doses of intravenous desferrioxamine infused over a short period of time induce a large faecal and urinary iron excretion but also produce retinal abnormalities that are characterised by decreased amplitude on electroretinography and defective dark adaptation. This regimen also results in high faecal iron, zinc, and copper excretion, and reduced granulocyte zinc concentrations and alkaline phosphatase activity. The retinal abnormalities may be related to the zinc and copper deficiency and/or iron depletion 'per se' which interferes negatively with critical iron dependent enzymes.
                Bookmark

                Author and article information

                Journal
                COP
                COP
                10.1159/issn.1663-2699
                Case Reports in Ophthalmology
                S. Karger AG
                1663-2699
                2021
                January - April 2021
                07 January 2021
                : 12
                : 1
                : 16-24
                Affiliations
                Sunderland Eye Infirmary, Sunderland, United Kingdom
                Author notes
                *Michael E. Grinton, Ophthalmology Department, Sunderland Eye Infirmary, Queen Alexandra Road, Sunderland SR2 9HP (UK), Michael.Grinton@gmail.com
                Article
                510670 PMC7879262 Case Rep Ophthalmol 2021;12:16–24
                10.1159/000510670
                PMC7879262
                33613246
                74d3c330-d1a2-4353-87b4-d64c68491289
                © 2021 The Author(s). Published by S. Karger AG, Basel

                This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 10 June 2020
                : 01 August 2020
                Page count
                Figures: 3, Tables: 1, Pages: 9
                Categories
                Case Report

                Vision sciences,Ophthalmology & Optometry,Pathology
                Anti-VEGF,Pseudo-vitelliform dystrophy,Desferrioxamine-related maculopathy,Desferrioxamine (desferal, deferoxamine)

                Comments

                Comment on this article