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      • Record: found
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      Is Open Access

      Hemoglobin S/O Arab: Retinal Manifestations of a Rare Hemoglobinopathy

      case-report

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          Abstract

          Hemoglobin S/O<sub>Arab</sub> (Hgb S/O<sub>Arab</sub>) disease is a rare hemoglobinopathy which presents similarly to sickle cell retinopathy, with only three prior reports that describe associated retinal findings. In this report, we present ophthalmic examination findings in 2 patients with Hgb S/O<sub>Arab</sub>. One patient exhibited peripheral ischemia and sunburst lesions without neovascular disease, and the other patient developed proliferative retinopathy of both eyes and multiple posterior-pole branch retinal artery occlusions in one eye. To our knowledge, this is the first case of retinal arterial occlusive disease in Hgb S/O<sub>Arab</sub>, and the first report of fundus autofluorescence and OCT angiography in Hgb/O<sub>Arab</sub> retinopathy.

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          Most cited references12

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          Natural history of untreated proliferative sickle retinopathy.

          Richard Goldberg (1971)
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            Sickle cell retinopathy: improving care with a multidisciplinary approach

            Farid Menaa, Barkat Khan, Bushra Uzair … (2017)
            Sickle cell retinopathy (SCR) is the most representative ophthalmologic complication of sickle cell disease (SCD), a hemoglobinopathy affecting both adults and children. SCR presents a wide spectrum of manifestations and may even lead to irreversible vision loss if not properly diagnosed and treated at the earliest. Over the past decade, multidisciplinary research developments have focused upon systemic, genetic, and ocular risk factors of SCR, enabling the clinician to better diagnose and manage these patients. In addition, newer imaging and testing modalities, such as spectral domain-optical coherence tomography angiography, have resulted in the detection of subclinical retinopathy related to SCD. Innovative therapy includes intravitreal injection of an anti-vascular endothelial growth factor (eg, Lucentis® [ranibizumab] or Eylea® [aflibercept]) which appears comparatively safe and efficient, and may be combined with laser photocoagulation (LPC) for proliferative SCR. The effect of LPC alone does not significantly lead to the regression of advanced SCR, although it helps in avoiding hemorrhage and sight loss. This comprehensive article is based on 10-years retrospective (2007–2017) studies. It aims to present advances and recommendations in SCR theranostics while pointing out the requirement of combinatorial approaches for better management of SCR patients. To reach this goal, we identified and analyzed randomized original and review articles, clinical trials, non-randomized intervention studies, and observational studies using specified keywords in various databases (eg, Medline, Embase, Cochrane, ClinicalTrials.gov).
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              Sickle-cell hemoglobin and its relation to fundus abnormality.

              R B Welch, M F Goldberg (1966)
              Article 0 comments Cited 10 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Case Rep Ophthalmol
                Journal ID (iso-abbrev): Case Rep Ophthalmol
                Journal ID (publisher-id): COP
                Title: Case Reports in Ophthalmology
                Publisher: S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.com )
                ISSN (Electronic): 1663-2699
                Publication date Collection: May-Aug 2020
                Publication date (Electronic): 27 May 2020
                Publication date PMC-release: 27 May 2020
                Volume: 11
                Issue: 2
                Pages: 189-195
                Affiliations
                [1] aHarvey and Bernice Jones Eye Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA
                [2] bCollege of Medicine, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA
                [3] cSouthwest Eye Care, Albuquerque, New Mexico, USA
                Author notes
                *Sami Uwaydat, Harvey and Bernice Jones Eye Institute, University of Arkansas for Medical Sciences, 4301 W Markham St., Slot 523, Little Rock, AR 72205 (USA), SHUwaydat@ 123456uams.edu
                Article
                Publisher ID: cop-0011-0189
                DOI: 10.1159/000507879
                PMC ID: 7315179
                SO-VID: 7838ab08-c0b4-45dc-9827-31888b3cebf5
                Copyright © Copyright © 2020 by S. Karger AG, Basel
                License:

                This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.

                History
                Date received : 22 November 2019
                Date accepted : 14 April 2020
                Date: 2020
                Page count
                Figures: 4, References: 13, Pages: 7
                Categories
                Subject: Case Report

                Keywords: hemoglobin s/oarab,sickle cell retinopathy,hemoglobinopathy,retinal ischemia
                Data availability:
                Keywords: hemoglobin s/oarab, sickle cell retinopathy, hemoglobinopathy, retinal ischemia

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