660 results match your criteria Retinopathy Hemoglobinopathies


Ocular Manifestations of Sickle Cell Disease in Different Genotypes.

Ophthalmic Epidemiol 2020 Aug 6:1-6. Epub 2020 Aug 6.

Department of Special Surgery, The University of Jordan Hospital, The University of Jordan , Amman, Jordan.

Background: Sickle cell disease (SCD) is a multisystemic disorder with variable systemic involvement which varies according to genotype. In this study, our aim is to compare ocular complications between HbSS, HbSC, HbS/β+ thalassemia, HbS/β0 thalassemia, SS alpha thalassemia, and S/β0 + alpha thalassemia genotypes.

Methods: Data of patients included in this study was recruited from the Cooperative Study of Sickle Cell Disease (CSSCD). Read More

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http://dx.doi.org/10.1080/09286586.2020.1801762DOI Listing

Sight threatening vitreous haemorrhage and retinal detachment in a patient with sickle cell disease.

Pan Afr Med J 2020 2;35. Epub 2020 Jan 2.

Department of Paediatrics and Adolescent Health, Faculty of Medicine, University of Botswana, Botswana.

We report a case of sight threatening vitreous haemorrhage and retinal detachment as complication of sickle cell disease (SCD). A 35 years old female Nigerian patient had presented to ophthalmology clinic of Princess Marina Hospital, Botswana, with two weeks history of poor vision in the left eye. The loss of vision was due to vitreous haemorrhage and retinal detachment which was confirmed by direct and indirect ophthalmoscopy and B-Scan ultrasound. Read More

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http://dx.doi.org/10.11604/pamj.2020.35.1.17098DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7026545PMC

Ocular Manifestations of Sickle Cell Disease: Signs, Symptoms and Complications.

Ophthalmic Epidemiol 2020 08 3;27(4):259-264. Epub 2020 Feb 3.

Department of Urology, Jordanian Royal Medical Services , Amman, Jordan.

Background: Sickle cell disease is an inherited hematological disorder that can affect any organ in the body including the eyes (1-6). Previous studies on ocular manifestations of sickle cell disease generally included samples of less than 100 patients. In this study, we aim to assess the frequency of different ocular signs, symptoms and complications among sickle cell disease patients. Read More

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http://dx.doi.org/10.1080/09286586.2020.1723114DOI Listing
August 2020
1.271 Impact Factor

Optical coherence tomography angiography findings in young β-thalassemia patients.

Eur J Ophthalmol 2020 May 5;30(3):600-607. Epub 2020 Jan 5.

Department of Ophthalmology, Bursa City Hospital, Bursa, Turkey.

Purpose: The purpose was to evaluate retinal vascular parameters by optical coherence tomography angiography in β-thalassemia major patients.

Methods: Thirty-three patients with β-thalassemia major (study group) and 29 healthy children (control group) were enrolled in the study. All subjects underwent a complete ocular examination. Read More

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http://dx.doi.org/10.1177/1120672119899375DOI Listing

Curing Hemoglobinopathies: Challenges and Advances of Conventional and New Gene Therapy Approaches.

Mediterr J Hematol Infect Dis 2019 1;11(1):e2019067. Epub 2019 Nov 1.

Department of Pediatrics, Division of Hematology, Children's Hospital of Philadelphia, Philadelphia, PA, United States.

Inherited hemoglobin disorders, including beta-thalassemia (BT) and sickle-cell disease (SCD), are the most common monogenic diseases worldwide, with a global carrier frequency of over 5%.1 With migration, they are becoming more common worldwide, making their management and care an increasing concern for health care systems. BT is characterized by an imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and compensatory hemopoietic expansion. Read More

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http://dx.doi.org/10.4084/MJHID.2019.067DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6827604PMC
November 2019

Awareness of Ocular Manifestations, Complications, and Treatment of Sickle Cell Disease in the Eastern Province of Saudi Arabia: A Cross-Sectional Study.

Middle East Afr J Ophthalmol 2019 Apr-Jun;26(2):89-94. Epub 2019 Aug 26.

Department of Statistics, Assistant Professor, King Abdulaziz University, Jeddah, Saudi Arabia.

Aim: This study aims to assess the level of awareness about the effect of sickle cell disease (SCD) on the eye and vision and factors influencing SCD awareness.

Subjects And Methods: The study design was cross-sectional and was carried out in 2018 among the general population in the Eastern province of Saudi Arabia. It was conducted using an online, validated questionnaire, after obtaining consent from the participants. Read More

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http://dx.doi.org/10.4103/meajo.MEAJO_200_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6737790PMC
November 2019

Retinal Thickness and Microvascular Changes in Children With Sickle Cell Disease Evaluated by Optical Coherence Tomography (OCT) and OCT Angiography.

Am J Ophthalmol 2020 01 29;209:88-98. Epub 2019 Aug 29.

Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. Electronic address:

Purpose: To compare the severity of macular vascular changes in children with sickle cell disease (SCD) vs age- and race-matched controls.

Design: Cross-sectional study.

Methods: Children (<18 years old) with HbSS and HbS variant (HbSC and HbS thalassemia) genotypes, and their age- and race-matched controls, were recruited between January 2017 and December 2018. Read More

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http://dx.doi.org/10.1016/j.ajo.2019.08.019DOI Listing
January 2020
1 Read

Identification and Characterization of Hematopoietic Stem Cell Transplant Candidates in a Sickle Cell Disease Cohort.

Biol Blood Marrow Transplant 2019 10 20;25(10):2103-2109. Epub 2019 Jun 20.

Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Serviço de Hematologia, Hemoterapia e Terapia Celular, São Paulo, Brazil; Department of Haematology, University of Oxford, Churchill Hospital, NHS-BT, Oxford, United Kingdom.

Sickle cell disease (SCD) is associated with significant morbidity, and allogeneic hematopoietic stem cell transplantation (HSCT) remains the primary curative treatment. Recently, the Brazilian Ministry of Health released a regulation that required the publically funded healthcare system to pay for HSCT for SCD patients with defined indications. We used an existing 2794-member SCD cohort established during 2013 to 2015 to characterize candidates for HSCT and estimate the number of possible donors. Read More

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http://dx.doi.org/10.1016/j.bbmt.2019.06.013DOI Listing
October 2019
12 Reads

Knowledge, beliefs and practices regarding sickle cell eye disease of patients at the sickle cell unit, Jamaica.

Pan Afr Med J 2019 19;32:84. Epub 2019 Feb 19.

Sickle Cell Unit, Caribbean Institute for Health Research, University of the West Indies, Mona Campus Kingston 7, Jamaica.

Introduction: Sickle cell disease can result in visually threatening eye disease (proliferative sickle cell retinopathy). This can be prevented with timely eye screening. It is important for patients to understand their role. Read More

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http://www.panafrican-med-journal.com/content/article/32/84/
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http://dx.doi.org/10.11604/pamj.2019.32.84.14742DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6560980PMC
July 2019
17 Reads

CRAO in Moyamoya Syndrome Associated With Southampton Hemoglobinopathy.

Ophthalmic Surg Lasers Imaging Retina 2019 05;50(5):e166-e170

The authors present the first case of central retinal artery occlusion (CRAO) resulting from moyamoya syndrome secondary to Southampton hemoglobinopathy. A 12-year-old Hispanic girl with a history of Southampton hemoglobinopathy with moyamoya syndrome presented with amaurosis fugax in her left eye that resolved within hours except for an inferior paracentral scotoma. She had left ophthalmic artery occlusion on magnetic resonance angiogram. Read More

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http://dx.doi.org/10.3928/23258160-20190503-17DOI Listing
May 2019
4 Reads

Angiopoietin-2 as a Marker of Retinopathy in Children and Adolescents With Sickle Cell Disease: Relation to Subclinical Atherosclerosis.

J Pediatr Hematol Oncol 2019 07;41(5):361-370

Departments of Pediatric.

Objectives: Angiopoietin-2 (Ang-2) is a multifaceted cytokine that functions in both angiogenesis and inflammation. A proangiogenic state has been found in adults with sickle cell disease (SCD), mainly because of elevated Ang-2 levels. We determined Ang-2 level in 40 children and adolescents with SCD compared with 40 healthy controls and assessed its relation to retinopathy as well as carotid intimamedia thickness (CIMT). Read More

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http://dx.doi.org/10.1097/MPH.0000000000001486DOI Listing
July 2019
16 Reads

Morphine promotes neovascularizing retinopathy in sickle transgeneic mice.

Blood Adv 2019 04;3(7):1073-1083

Vascular Biology Center, and.

Neovascularizing retinopathy is a significant complication of sickle cell disease (SCD), occurring more frequently in HbSC than HbSS disease. This risk difference is concordant with a divergence of angiogenesis risk, as identified by levels of pro- vs anti-angiogenic factors in the sickle patient's blood. Because our prior studies documented that morphine promotes angiogenesis in both malignancy and wound healing, we tested whether chronic opioid treatment would promote retinopathy in NY1DD sickle transgenic mice. Read More

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http://www.bloodadvances.org/lookup/doi/10.1182/bloodadvance
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http://dx.doi.org/10.1182/bloodadvances.2018026898DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6457224PMC
April 2019
18 Reads

Sickle cell retinopathy. A focused review.

Graefes Arch Clin Exp Ophthalmol 2019 Jul 20;257(7):1353-1364. Epub 2019 Mar 20.

Vitreoretinal Division, King Khaled Eye Specialist Hospital, Al-Oruba Street, 7191, Riyadh, 11462, Kingdom of Saudi Arabia.

Purpose: To provide a focused review of sickle cell retinopathy in the light of recent advances in the pathogenesis, multimodal retinal imaging, management of the condition, and migration trends, which may lead to increased prevalence of the condition in the Western world.

Methods: Non-systematic focused literature review.

Results: Sickle retinopathy results from aggregation of abnormal hemoglobin in the red blood cells in the retinal microcirculation, leading to reduced deformability of the red blood cells, stagnant blood flow in the retinal precapillary arterioles, thrombosis, and ischemia. Read More

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http://dx.doi.org/10.1007/s00417-019-04294-2DOI Listing
July 2019
13 Reads

Peripheral Vaso-Occlusive Events Following Trauma in Patients With Sickle Cell Trait.

Ophthalmic Surg Lasers Imaging Retina 2019 03;50(3):e84-e87

Sickle cell trait (SCT) is a common hemoglobin variant, particularly in patients of African descent. Patients with SCT have an increased risk of adverse ocular events following trauma with subsequent elevated intraocular pressure (IOP). The authors describe three cases of young males with peripheral vaso-occlusive events following ocular trauma with subsequent elevated IOP. Read More

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http://dx.doi.org/10.3928/23258160-20190301-18DOI Listing
March 2019
1 Read

Conjunctival biomicroscopical findings in the conjunctiva of a patient with drepanocytic proliferative retinopathy.

Arch Soc Esp Oftalmol 2019 May 10;94(5):e33. Epub 2019 Mar 10.

Unidad de Gestión Clínica de Oftalmología, Hospital Universitario Virgen del Rocío, Sevilla, España.

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http://dx.doi.org/10.1016/j.oftal.2019.01.012DOI Listing
May 2019
1 Read

Sickle cell retinopathy: What we now understand using optical coherence tomography angiography. A systematic review.

Blood Rev 2019 05 4;35:32-42. Epub 2019 Mar 4.

Postgraduate course, Bahiana School of Medicine and Public Health, Av. Dom João VI, 275, Brotas, 40290-000 Salvador, BA, Brazil.

For over four decades, efforts have been underway for the evaluation of sickle cell retinopathy (SCR) in an attempt to identify peripheral high-risk vascular abnormalities based on Goldberg's classification (gold-standard) (1971). The macula is an area in the center of the retina that is responsible for high-resolution central vision and is also affected in SCR. With the development of new technologies for retinal imaging, the macula became a main focus of interest in the study of sickle cell disease (SCD). Read More

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http://dx.doi.org/10.1016/j.blre.2019.03.001DOI Listing
May 2019
8 Reads
5.565 Impact Factor

Prevalence and Onset of Pediatric Sickle Cell Retinopathy.

Ophthalmology 2019 07 2;126(7):1000-1006. Epub 2019 Mar 2.

Division of Ophthalmology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Department of Ophthalmology, Scheie Eye Institute, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:

Purpose: Children with sickle cell hemoglobinopathy (SCH) can demonstrate proliferative retinopathy with vision loss, but lack of consensus exists regarding screening regimens. We sought to determine the prevalence, age at onset, and risk factors associated with sickle cell retinopathy (SCR) to inform development of screening guidelines for asymptomatic children.

Design: Retrospective cohort study. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01616420183200
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http://dx.doi.org/10.1016/j.ophtha.2019.02.023DOI Listing
July 2019
21 Reads

Longitudinal Study of Peripapillary Thinning in Sickle Cell Hemoglobinopathies.

Am J Ophthalmol 2019 06 14;202:30-36. Epub 2019 Feb 14.

Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois, USA. Electronic address:

Purpose: To determine the rate of retinal nerve fiber layer (RNFL) thinning in patients with sickle cell hemoglobinopathies.

Design: This was a prospective cohort study.

Methods: Sixty-seven patients averaging 35. Read More

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http://dx.doi.org/10.1016/j.ajo.2019.02.006DOI Listing
June 2019
2 Reads

Terminologies regarding sickle cell retinopathy and maculopathy.

Clin Hemorheol Microcirc 2019 ;71(1):1-2

Laboratoire d'Excellence GR-Ex ≪ The red cell: from genesis to death ≫, PRES Sorbonne Paris Cité, Paris, France.

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http://dx.doi.org/10.3233/CH-190552DOI Listing
November 2019
4 Reads

Gross anterior segment ischaemia following vitreoretinal surgery for sickle-cell retinopathy.

BMJ Case Rep 2019 Jan 29;12(1). Epub 2019 Jan 29.

Vitreo-retinal Unit, Manchester Royal Eye Hospital, Manchester, UK.

We report the case of a 32-year-old Afrocaribbean man with known stage 3 proliferative sickle-cell retinopathy who presented with a mixed picture of tractional and rhegmatogenous macula off detachment. He underwent left primary 25 g vitrectomy with silicone oil, delamination and endolaser photocoagulation under a general anaesthetic. He, however, presented 48 hours postoperatively with gross anterior segment ischaemia. Read More

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http://dx.doi.org/10.1136/bcr-2018-227541DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352801PMC
January 2019
11 Reads

Association between MTHFR 677C>T polymorphism and vascular complications in sickle cell disease: A meta-analysis.

Transfus Clin Biol 2019 Nov 10;26(4):284-288. Epub 2019 Jan 10.

Sickle Cell Institute Chhattisgarh, Forest Block-2, Jail Road, Raipur 492001, Chhattisgarh India. Electronic address:

Sickle cell disease (SCD) is considered as a vascular disease due to its chronic vascular manifestations such as leg ulcers, priapism, acute chest syndrome (ACS), stroke, retinopathy, renal insufficiency, pulmonary hypertension, avascular necrosis of the femoral head (AVNF) and splenic infarction. Emerging evidence has shown that the MTHFR 677C>T variant allele is associated with vascular complications (VC) in patients with SCD; however, results from individual studies are inconclusive. The aim of this meta-analysis is to evaluate the association between the MTHFR 677C>T polymorphism and the susceptibility for VC in SCD patients. Read More

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http://dx.doi.org/10.1016/j.tracli.2019.01.003DOI Listing
November 2019
8 Reads

[Indications and efficacy of intravitreal injections of anti-VEGF in black people in Abidjan].

J Fr Ophtalmol 2019 Jan 26;42(1):e18-e19. Epub 2018 Dec 26.

UFR des sciences médicales, 01 BP V34, Abidjan 01, Cote d'Ivoire.

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http://dx.doi.org/10.1016/j.jfo.2018.02.026DOI Listing
January 2019
1 Read

Terminologies regarding sickle cell retinopathy.

Clin Hemorheol Microcirc 2019 ;71(4):499-500

Bahiana's School of Medicine and Public Health, Salvador, Brazil.

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http://dx.doi.org/10.3233/CH-180462DOI Listing
November 2019
5 Reads
2.242 Impact Factor

Retinal Angiography Findings in Male Infant With Incontinentia Pigmenti and Sickle Cell Trait.

JAMA Ophthalmol 2018 11 8;136(11):e183140. Epub 2018 Nov 8.

Department of Ophthalmology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, England.

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http://archopht.jamanetwork.com/article.aspx?doi=10.1001/jam
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http://dx.doi.org/10.1001/jamaophthalmol.2018.3140DOI Listing
November 2018
12 Reads

Elevated fetal haemoglobin levels are associated with decreased incidence of retinopathy in adults with sickle cell disease.

Br J Haematol 2018 12 12;183(5):807-811. Epub 2018 Nov 12.

Department of Hematology, Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, NY, USA.

Among the many vascular complications of sickle cell disease (SCD), retinopathy is the most prevalent and represents a leading cause of blindness. Hydroxycarbamide therapy ameliorates many symptoms of SCD, and high fetal haemoglobin (HbF) levels have been shown to protect against the development of retinopathy in children with HbSS. Its effect on adults with SCD, who are at a much higher risk of developing retinopathy, has not been studied. Read More

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http://doi.wiley.com/10.1111/bjh.15617
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http://dx.doi.org/10.1111/bjh.15617DOI Listing
December 2018
18 Reads

Beta-Thalassemia Minor Manifesting as Proliferative Retinopathy.

Ophthalmic Surg Lasers Imaging Retina 2018 10;49(10):e161-e164

Beta-thalassemia (β-thalassemia) minor is characterized by a mutation in one of the two β-globin genes (HBB) that produce the β-globin chains in the hemoglobin molecule. Although other hemoglobinopathies have been frequently associated with retinal disease, there are limited reports of retinal pathology with β-thalassemia minor. We report two patients with β-thalassemia minor presenting with decreased vision, vitreous hemorrhage, and proliferative retinopathy. Read More

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https://www.healio.com/doiresolver?doi=10.3928/23258160-2018
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http://dx.doi.org/10.3928/23258160-20181002-22DOI Listing
October 2018
40 Reads

Increased Prevalence of Type 2 Diabetes-Related Complications in Combined Type 2 Diabetes and Sickle Cell Trait.

Diabetes Care 2018 12 16;41(12):2595-2602. Epub 2018 Oct 16.

Inter-university Laboratory of Biology of Motor Function EA7424, Vascular Biology and the Red Blood Cell Team, Claude Bernard University Lyon 1, University de Lyon 1, Villeurbanne, France

Objective: The prevalence of type 2 diabetes (T2D) is rapidly increasing in sub-Saharan Africa, where sickle cell trait (SCT) is also frequent. Although SCT is generally considered a benign condition, evidence suggests that SCT could exaggerate vascular dysfunction in T2D. However, it remains unclear whether SCT could increase the risk of the development of T2D complications. Read More

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http://dx.doi.org/10.2337/dc18-1289DOI Listing
December 2018
8 Reads

Clinical and Ophthalmic Factors Associated With the Severity of Sickle Cell Retinopathy.

Am J Ophthalmol 2019 01 29;197:105-113. Epub 2018 Sep 29.

Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. Electronic address:

Purpose: To identify associations between severity of sickle cell retinopathy (SCR) and other clinical, laboratory, or treatment factors relevant to sickle cell disease (SCD).

Design: Retrospective cohort study.

Methods: We investigated clinical, laboratory, and demographic associations with the severity of SCR in 296 patients seen at both our SCD specialty clinic and our retina clinic. Read More

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http://dx.doi.org/10.1016/j.ajo.2018.09.025DOI Listing
January 2019
3 Reads

ANGIOID STREAKS: A Comprehensive Review From Pathophysiology to Treatment.

Retina 2019 Jan;39(1):1-11

2nd Department of Ophthalmology, University of Athens, Athens, Greece.

Purpose: To stratify the literature on angioid streaks, from pathophysiology to treatment.

Methods: Review of the current literature.

Results: Angioid streaks are crack-like dehiscences of Bruch membrane, which may coexist with systemic diseases, such as pseudoxanthoma elasticum, Paget disease, Ehlers-Danlos syndrome, hemoglobinopathies, or other diseases of the collagen. Read More

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http://Insights.ovid.com/crossref?an=00006982-900000000-9635
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http://dx.doi.org/10.1097/IAE.0000000000002327DOI Listing
January 2019
48 Reads

Angiopoetin-like 4 in sickle cell retinopathy.

Biosci Rep 2018 10 31;38(5). Epub 2018 Oct 31.

Bahiana's School of Medicine and Public Health, Salvador, Brazil.

This correspondence provides a comment on the recent review article by Yang et al. [ (2018) , BSR20180557, https://doi.org/10. Read More

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http://bioscirep.org/lookup/doi/10.1042/BSR20181462
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http://dx.doi.org/10.1042/BSR20181462DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6209607PMC
October 2018
11 Reads
1 Citation
2.640 Impact Factor

[Angioid streaks in a 33-year-old man with β-Thalassemia].

J Fr Ophtalmol 2018 Oct 14;41(8):e355-e357. Epub 2018 Sep 14.

Service d'ophtalmologie, CHRU de Rennes-Pontchaillou, 2, rue Henri-Le-Guilloux, 35000 Rennes, France.

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http://dx.doi.org/10.1016/j.jfo.2018.01.017DOI Listing
October 2018
3 Reads

Pattern dystrophies in patients treated with deferoxamine: report of two cases and review of the literature.

BMC Ophthalmol 2018 Sep 12;18(1):246. Epub 2018 Sep 12.

Department of Ophthalmology, Medical School, University of Patras, 265 04, Patras, Greece.

Background: Deferoxamine (DFO) is one of the most commonly used chelation treatments for transfusional hemosiderosis. Pattern dystrophies constitute a distinct entity of retinal disorders that has been occasionally identified in association with deferoxamine.

Case Presentation: We report two cases of bilateral macular pattern dystrophy in transfusion dependent patients undergoing chronic chelation therapy with deferoxamine due to thalassemias. Read More

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http://dx.doi.org/10.1186/s12886-018-0911-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6134579PMC
September 2018
32 Reads

Recurrent orbital bone sub-periosteal hematoma in sickle cell disease: a case study.

BMC Ophthalmol 2018 Aug 28;18(1):211. Epub 2018 Aug 28.

Department Of Ophthalmology, Faculty of Medicine, Taif University, Taif, Saudi Arabia.

Background: Sickle cell disease is a common inherited hemoglobinopathy and is associated with high morbidity and mortality. Vaso-occlusive crises commonly occur in individuals with SCD that results in high morbidity due to end-organ ischemia and infarction. These include splenic infarction, pulmonary involvement, acute chest syndrome, and orbital compression syndrome. Read More

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https://bmcophthalmol.biomedcentral.com/articles/10.1186/s12
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http://dx.doi.org/10.1186/s12886-018-0884-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6114482PMC
August 2018
42 Reads

Paramacular temporal atrophy in sickle cell disease occurs early in childhood.

Br J Ophthalmol 2019 07 21;103(7):906-910. Epub 2018 Aug 21.

Ophthalmology Department and Rare Ophthalmological Diseases Reference Centre, Necker-Enfants Malades University Hospital, APHP, Paris, France.

Background/aims: Initially reported in a few patients with homozygous sickle cell disease (SCD), atrophic areas of the retina temporal from the macula are now known to be present in about 48% of eyes of adult patients with SS-SCD and in 35% of eyes of adult patients with SC-SCD. The aim of this study is to describe this paramacular atrophy in children affected with SCD.

Methods: In this retrospective series, spectral-domain optical coherence tomography images of 81 children with SCD, acquired with specific patterns including one evaluating the retina temporal to the macula, were reviewed, in order to look for retinal atrophy. Read More

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http://dx.doi.org/10.1136/bjophthalmol-2018-312305DOI Listing
July 2019
2 Reads

Funduscopic examination and SD-OCT in detecting sickle cell retinopathy among pediatric patients.

J AAPOS 2018 10 17;22(5):411. Epub 2018 Aug 17.

Bahia's School of Medicine and Public Health, Salvador, Brazil.

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http://dx.doi.org/10.1016/j.jaapos.2018.07.345DOI Listing
October 2018
6 Reads
1.142 Impact Factor

Retinal thickness measurements in sickle cell patients with HbSS and HbSC genotype.

Can J Ophthalmol 2018 08 11;53(4):420-424. Epub 2017 Dec 11.

Ophthalmology Department, St Thomas' Hospital, London, United Kingdom.

Objective: Temporal macula thinning has been reported in sickle cell patients, but it remains unclear if there is a difference between HbSS and HbSC genotypes. We aimed to quantitatively compare macular thickness between eyes with HbSS and HbSC genotype.

Design: Retrospective descriptive study. Read More

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http://dx.doi.org/10.1016/j.jcjo.2017.10.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6117475PMC
August 2018
33 Reads

The past, present and future management of sickle cell retinopathy within an African context.

Eye (Lond) 2018 08 10;32(8):1304-1314. Epub 2018 Jul 10.

Ophthalmology Department, Central Middlesex Hospital, London North West University Healthcare NHS Trust, London, NW10 7NS, UK.

Sickle cell retinopathy is a potentially blinding condition that affects young people in the working age group. This review looks at the past, present and future management of sickle cell retinopathy within an African context. After Sickle cell disease was first reported in 1910, some reports from Africa were pivotal in describing the retinal changes associated with the disease. Read More

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http://www.nature.com/articles/s41433-018-0162-8
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http://dx.doi.org/10.1038/s41433-018-0162-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6085343PMC
August 2018
19 Reads

Proliferative retinopathy and maculopathy are two independent conditions in sickle cell disease: Is there a role of blood rheology?1.

Clin Hemorheol Microcirc 2019 ;71(3):337-345

Université des Antilles, CHU dePointe-à-Pitre, Guadeloupe, Université Sorbonne Paris Cité, Université Paris Diderot, Inserm, INTS, Unité Biologie Intégrée du Globule Rouge UMR_S1134, Paris, France.

Objective: Our study investigated the prevalence of retinopathy and maculopathy in sickle cell patients and tested the association between these two conditions. In addition, we tested whether hematological and hemorheological parameters, as well as genotype, were involved in the development of these two conditions.

Methods: Seventy sickle cell adult patients were recruited: 37 with sickle cell anemia (SCA) and 33 with sickle cell hemoglobin C disease (SCC). Read More

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http://www.medra.org/servlet/aliasResolver?alias=iospress&am
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http://dx.doi.org/10.3233/CH-180412DOI Listing
August 2019
29 Reads

A method for age-matched OCT angiography deviation mapping in the assessment of disease- related changes to the radial peripapillary capillaries.

PLoS One 2018 24;13(5):e0197062. Epub 2018 May 24.

Department of Ophthalmology, New York Eye and Ear Infirmary of Mount Sinai, New York, NY, United States of America.

Purpose: To present a method for age-matched deviation mapping in the assessment of disease-related changes to the radial peripapillary capillaries (RPCs).

Methods: We reviewed 4.5x4. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0197062PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5993123PMC
August 2018
13 Reads

A comparison of 23-gauge and 20-gauge vitrectomy for proliferative sickle cell retinopathy - clinical outcomes and surgical management.

Eye (Lond) 2018 09 22;32(9):1449-1454. Epub 2018 May 22.

Vitreoretinal Service, Moorfields Eye Hospital, 162 City Rd, London, EC1V 2PD, UK.

Aims: To report anatomical and functional outcomes in patients with proliferative sickle retinopathy (PSR) who underwent 23-gauge (23G) and 20-gauge (20G) vitrectomy.

Methods: Retrospective consecutive case series of patients who underwent vitreoretinal intervention for complications of PSR between April 2009 and February 2015. Operations were performed at a tertiary referral centre, Moorfields Eye Hospital. Read More

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http://dx.doi.org/10.1038/s41433-018-0127-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6137034PMC
September 2018
8 Reads

Funduscopic examination and SD-OCT in detecting sickle cell retinopathy among pediatric patients.

J AAPOS 2018 06 4;22(3):197-201.e1. Epub 2018 May 4.

Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania.

Purpose: To compare the results of fundus examination and spectral domain optic coherence tomography (SD-OCT) in detecting retinal changes in pediatric patients with sickle cell disease at a single center.

Methods: In this prospective study, conducted over a period of 19 months, consecutive African American patients with sickle cell disease underwent complete ophthalmologic examination, and SD-OCT images of the maculas of both eyes were obtained.

Results: A total of 69 (37 males) patients aged 5-20 years (mean 12. Read More

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http://dx.doi.org/10.1016/j.jaapos.2017.12.019DOI Listing
June 2018
7 Reads

Retinal Oxygen Delivery and Metabolism in Healthy and Sickle Cell Retinopathy Subjects.

Invest Ophthalmol Vis Sci 2018 04;59(5):1905-1909

Casey Eye Institute, Oregon Health & Science University, Portland, Oregon, United States.

Purpose: Reduction in inner retinal oxygen delivery (DO2) can cause retinal hypoxia and impair inner retinal oxygen metabolism (MO2), leading to vision loss. The purpose of the current study was to establish measurements of DO2 and MO2 in healthy subjects and test the hypothesis that DO2 and MO2 are reduced in sickle cell retinopathy (SCR) subjects.

Methods: Dual wavelength retinal oximetry and Doppler optical coherence tomography were performed in 12 healthy control and 12 SCR subjects. Read More

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http://dx.doi.org/10.1167/iovs.17-23647DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5886143PMC
April 2018
8 Reads

Genetic modifiers of severity in sickle cell disease.

Clin Hemorheol Microcirc 2018 ;68(2-3):147-164

Department of Pediatrics, Division of Hematology/Oncology, Baylor College of Medicine, Houston, TX, USA.

Sickle cell disease (SCD) is one of the most common single disease disorders world-wide. It is remarkable for its clinical heterogeneity, even among individuals with identical genotypes. Some individuals experience morbidity and mortality in early childhood, while others have a relatively mild course, and normal or near normal life expectancy. Read More

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http://dx.doi.org/10.3233/CH-189004DOI Listing
May 2018
14 Reads

Analysis of Retinal Thinning Using Spectral-domain Optical Coherence Tomography Imaging of Sickle Cell Retinopathy Eyes Compared to Age- and Race-Matched Control Eyes.

Am J Ophthalmol 2018 08 17;192:229-238. Epub 2018 Mar 17.

Department of Ophthalmology, University of Illinois at Chicago, Illinois Eye and Ear Infirmary, Chicago, Illinois, USA.

Purpose: To determine whether the retina is thinner in sickle cell patients than in race- and age-matched controls, and, if it is thinner, whether there is any association with systemic diseases.

Methods: Sickle cell and control (age- and race-matched) patients were prospectively enrolled from a university retina clinic into this observational study. Participants underwent visual acuity testing, slit-lamp biomicroscopy, dilated ophthalmoscopy, and spectral-domain optical coherence tomography imaging. Read More

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http://dx.doi.org/10.1016/j.ajo.2018.03.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6064656PMC
August 2018
4 Reads

Sickle cell maculopathy: Identification of systemic risk factors, and microstructural analysis of individual retinal layers of the macula.

PLoS One 2018 1;13(3):e0193582. Epub 2018 Mar 1.

Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.

Purpose: To identify systemic risk factors for sickle cell maculopathy, and to analyze the microstructure of the macula of Sickle Cell Disease (SCD) patients by using automated segmentation of individual retinal layers.

Methods: Thirty consecutive patients with SCD and 30 matched controls underwent spectral-domain optical coherence tomography (SD-OCT) and automated thickness measurement for each retinal layer; thicknesses for SCD patients were then compared to normal controls. Demographic data, systemic data, and lab results were collected for each SCD patient; multivariate logistic regression analysis was used to identify potential risk factors for sickle cell maculopathy. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0193582PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5832302PMC
June 2018
37 Reads

Sickle cell retinopathy: A literature review.

Rev Assoc Med Bras (1992) 2017 Dec;63(12):1100-1103

Health Science Graduate Program, Universidade Federal de Alagoas, Maceió, AL, Brazil.

Hemoglobinopathies are a group of hereditary diseases that cause quantitative or qualitative changes in the shape, function or synthesis of hemoglobin. One of the most common is sickle cell anemia, which, due to sickling of erythrocytes, causes vaso-occlusive phenomena. Among the possible ocular manifestations, the most representative is retinopathy, which can lead to blindness if left untreated. Read More

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http://dx.doi.org/10.1590/1806-9282.63.12.1100DOI Listing
December 2017
11 Reads

UTILITY OF ULTRA-WIDEFIELD RETINAL IMAGING FOR THE STAGING AND MANAGEMENT OF SICKLE CELL RETINOPATHY.

Retina 2019 May;39(5):836-843

Retina Division, Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, Maryland.

Purpose: To determine whether ultra-widefield (UWF) retinal imaging changes the staging or management of sickle cell retinopathy compared with clinical examination.

Methods: Prospective, observational study including patients with sickle cell disease. All patients underwent dilated fundus examination by a fellowship-trained retina specialist, as well as UWF fundus photography (FF) and fluorescein angiography (FA). Read More

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http://dx.doi.org/10.1097/IAE.0000000000002057DOI Listing
May 2019
4 Reads

Bilateral simultaneous macular infarction with spontaneous visual recovery in genotype ss hemoglobinopathy patient.

Niger J Clin Pract 2017 Dec;20(12):1651-1655

Eye Foundation Center for Prevention of Blindness, Eye Foundation Retina Institute, Lagos, Nigeria.

To report the rare and dramatic event of bilateral macular infarction in a sickle cell hemoglobinopathy (SS genotype) patient, resulting in bilateral severe reduction in visual acuity. Without any intervention, the patient's vision gradually improved over the follow-up period. Central visual field defects however persisted. Read More

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http://dx.doi.org/10.4103/njcp.njcp_95_17DOI Listing
December 2017
18 Reads

Sickle cell retinopathy and other chronic complications of sickle cell anemia: A clinical study of 84 Sub-Saharan African cases (Cameroon).

J Fr Ophtalmol 2018 Jan 30;41(1):50-56. Epub 2017 Dec 30.

Ophthalmology, faculty of medicine and biomedical sciences, university of Yaoundé I, 6066 Yaoundé, Cameroon.

Objective: Sickle retinopathy is a severe complication of sickle cell disease than can lead to blindness. We aim to describe the epidemiology of sickle retinopathy in homozygous sickle cell (SS) African patients and to analyze its association with non-ophthalmologic disease complications of sickle cell anemia.

Methods: We conducted a nested study within the CADRE cohort in Cameroon. Read More

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http://dx.doi.org/10.1016/j.jfo.2017.07.005DOI Listing
January 2018
23 Reads