619 results match your criteria Retinopathy Hemoglobinopathies


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
17 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
2 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
3 Reads
1 Citation
2.640 Impact Factor

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
5 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
12 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
5 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
7 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
4 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
1 Read

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
6 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
8 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
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
3 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
5 Reads

[Risk factors for retinal detachment in Togo].

Pan Afr Med J 2017 26;28:74. Epub 2017 Sep 26.

Université de Lomé, FSS, Service d'Ophtalmologie CHU Sylvanus Olympio Lomé-Togo.

Introduction: Retinal detachment poses management problems in developing countries due to the shortage of technical equipment. Prevention passes through knowledge and elimination of risk factors. This study aimed to identify the risk factors for retinal detachment in people living in Togo. Read More

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http://www.panafrican-med-journal.com/content/article/28/74/
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http://dx.doi.org/10.11604/pamj.2017.28.74.13418DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5724729PMC
December 2017
14 Reads

Sickle cell disease and the eye.

Curr Opin Ophthalmol 2017 Nov;28(6):623-628

aUSC Roski Eye Institute, Keck School of Medicine bUSC Institute for Biomedical Therapeutics, University of Southern California, Los Angeles, California, USA.

Purpose Of Review: To review recent literature pertaining to sickle cell retinopathy (SCR) and, in particular, sickle cell maculopathy.

Recent Findings: Several recent studies suggest that macular perfusion abnormalities seen in patients with sickle cell disease of various genotypes may affect both the superficial and deep capillary plexi, with a predilection for the deep capillary plexus. Further, these changes may be associated with areas of macular thinning, as well as with peripheral retinal ischemia, even in individuals without visual symptoms, contrary to what has previously been described in both diabetic retinopathy and retinal vein occlusion. Read More

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http://dx.doi.org/10.1097/ICU.0000000000000423DOI Listing
November 2017
14 Reads

Optical Coherence Tomography Angiography and Ultra-widefield Fluorescein Angiography for Early Detection of Adolescent Sickle Retinopathy.

Am J Ophthalmol 2017 Nov 30;183:91-98. Epub 2017 Aug 30.

Department of Ophthalmology, Columbia University Medical Center, New York, New York. Electronic address:

Purpose: Based on standard screening techniques, sickle retinopathy reportedly occurs in 10% of adolescents with sickle cell disease (SCD). We performed a prospective, observational clinical study to determine if ultra-widefield fluorescein angiography (UWFA), spectral-domain optical coherence tomography (SD-OCT), and optical coherence tomography angiography (OCT-A) detect more-frequent retinopathy in adolescents with SCD.

Design: Cross-sectional study. Read More

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http://dx.doi.org/10.1016/j.ajo.2017.08.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5660939PMC
November 2017
16 Reads

Visual Function in Asymptomatic Patients With Homozygous Sickle Cell Disease and Temporal Macular Atrophy.

JAMA Ophthalmol 2017 10;135(10):1100-1105

Ophthalmology Department, Necker-Enfants Malades University Hospital, APHP, Paris, France.

Importance: Temporal macular involvement in sickle cell disease can now easily be detected by optical coherence tomography (OCT). However, while recent studies have demonstrated its high prevalence, little is known about its potential consequences on visual function.

Objective: To assess the visual function of patients with sickle cell disease with no visual symptoms despite temporal macular atrophy. Read More

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http://dx.doi.org/10.1001/jamaophthalmol.2017.3008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5847113PMC
October 2017
44 Reads

Expression of the angiogenic mediator, angiopoietin-like 4, in the eyes of patients with proliferative sickle retinopathy.

PLoS One 2017 23;12(8):e0183320. Epub 2017 Aug 23.

Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MD, United States of America.

The recent success of therapies directly targeting the angiogenic mediator, vascular endothelial growth factor (VEGF), for the treatment of proliferative diabetic retinopathy has encouraged clinicians to extend the use of anti-VEGF therapies for the treatment of another ischemic retinal vascular disease, proliferative sickle cell retinopathy (PSR), the most common cause of irreversible blindness in patients with sickle cell disease. However, results from case reports evaluating anti-VEGF therapies for PSR have been mixed. This highlights the need to identify alternative therapeutic targets for the treatment of retinal neovascularization in sickle cell patients. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0183320PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5568377PMC
October 2017
11 Reads

[Fortuitous detection of composite heterozygous S/C sickle cell disease].

Pan Afr Med J 2017 7;27:93. Epub 2017 Jun 7.

Service de Biochimie-Toxicologie Hôpital Militaire d'Instruction Mohammed V, Rabat, Maroc.

Composite S/C sickle cell disease accounts for 20%-30% of major sickle cell syndromes. We report a case of fortuitous detection of composite heterozygous S/C sickle cell disease in the context of retinal detachment. The patient had been hospitalized in the Department of Ophthalmology for treatment-resistant decreased visual acuity detected 06 months before. Read More

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http://dx.doi.org/10.11604/pamj.2017.27.93.12724DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5554668PMC
September 2017
9 Reads

Therapeutic use of transferrin to modulate anemia and conditions of iron toxicity.

Blood Rev 2017 11 24;31(6):400-405. Epub 2017 Jul 24.

Department of Intensive Care Medicine, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105AZ Amsterdam, The Netherlands.

As the main iron transporter, transferrin delivers iron to target tissues like the bone marrow for erythropoiesis. Also, by binding free iron, transferrin prevents formation of reactive oxygen species. Transferrin deficiency due to congenital hypotransferrinemia is characterized by anemia as well as oxidative stress related to toxic free iron. Read More

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http://dx.doi.org/10.1016/j.blre.2017.07.005DOI Listing
November 2017
16 Reads

New Ways to Detect Pediatric Sickle Cell Retinopathy: A Comprehensive Review.

J Pediatr Hematol Oncol 2017 11;39(8):618-625

*Departments of Pediatrics †Ophthalmology, Columbia University Medical Center, New York, NY.

Sickle retinopathy reflects disease-related vascular injury of the eye, which can potentially result in visual loss from vitreous hemorrhage or retinal detachment. Here we review sickle retinopathy among children with sickle cell disease, describe the epidemiology, pediatric risk factors, pathophysiology, ocular findings, and treatment. Newer, more sensitive ophthalmological imaging modalities are available for retinal imaging, including ultra-widefield fluorescein angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography. Read More

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http://dx.doi.org/10.1097/MPH.0000000000000919DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5656515PMC
November 2017
12 Reads

Prospective evaluation of chronic organ damage in adult sickle cell patients: A seven-year follow-up study.

Am J Hematol 2017 Oct 29;92(10):E584-E590. Epub 2017 Jul 29.

Department of Hematology, Academic Medical Center, Amsterdam, the Netherlands.

Organ damage in sickle cell disease (SCD) is a crucial determinant for disease severity and prognosis. In a previous study, we analyzed the prevalence of SCD-related organ damage and complications in adult sickle cell patients. We now describe a seven-year follow-up of this cohort. Read More

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http://dx.doi.org/10.1002/ajh.24855DOI Listing
October 2017
12 Reads

Electroretinogram Findings in Early-Stage Sickle Cell Retinopathy According to Hemoglobin Type.

Invest Ophthalmol Vis Sci 2017 06;58(7):3262-3267

Department of Ophthalmology, Centre Hospitalier Intercommunal, Créteil, France.

Purpose: Although extensive clinical research has been performed on structural analysis of sickle cell (SC) retinopathy, functional aspects have been poorly investigated. Our purpose was to report full-field electroretinogram (ffERG) findings in patients with early SC retinopathy according to the following hemoglobin types: HbSS or HbSC (homozygous or heterozygous mutations, respectively).

Methods: In this monocentric retrospective observational study, patients affected by nonproliferative SC retinopathy were included from November 2014 to April 2016. Read More

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http://dx.doi.org/10.1167/iovs.16-20719DOI Listing
June 2017
20 Reads

Look into my eyes: An unusual first presentation of sickle cell disease.

Am J Hematol 2017 Sep 9;92(9):968-971. Epub 2017 Jun 9.

Department of Ophthalmology, Johns Hopkins University School of Medicine, Baltimore, Maryland.

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http://dx.doi.org/10.1002/ajh.24787DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5547003PMC
September 2017
5 Reads

Paracentral acute middle maculopathy: precursor to macular thinning in sickle cell retinopathy.

BMJ Case Rep 2017 Apr 26;2017. Epub 2017 Apr 26.

Department of Ophthalmology and Visual Science, Yale University School of Medicine, New Haven, Connecticut, USA.

We present a case of paracentral acute middle maculopathy (PAMM) in a patient with sickle cell disease (SCD). Though gradual capillary loss may contribute to pathophysiology of sickle cell retinopathy as well, our case suggests that PAMM may be the precursor lesion to macular thinning commonly observed in patients with SCD. In addition, fluorescein angiography may be unable to detect these acute vascular events occurring at the deep capillary plexus. Read More

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http://dx.doi.org/10.1136/bcr-2016-216124DOI Listing
April 2017
8 Reads

Ophthalmic Evaluation in Beta-Thalassemia.

Indian J Pediatr 2017 Jul 3;84(7):509-514. Epub 2017 Apr 3.

U B M Institute, Mumbai, Maharashtra, India.

Objectives: To determine the association of ocular manifestations in beta-thalassemia with the patient's age, blood transfusion requirements, average serum ferritin and dose and duration of iron chelation therapy.

Methods: Sixty multi-transfused beta thalassemia patients of 12 to 18 y of age on chelation therapy were included in this cross-sectional analysis. Structural and functional evaluation of the retina was done using Optical coherence tomography (OCT) and Electroretinography (ERG), including flash ERG and Pattern ERG (PERG). Read More

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http://dx.doi.org/10.1007/s12098-017-2339-8DOI Listing
July 2017
7 Reads

Electrophysiological assessment for early detection of retinal dysfunction in β-thalassemia major patients.

Graefes Arch Clin Exp Ophthalmol 2017 Jul 30;255(7):1349-1358. Epub 2017 Mar 30.

First Department of Ophthalmology, "G. Gennimatas" General Hospital, Medical School, National and Kapodistrian University of Athens, 154 Mesogion Avenue, Athens, 11527, Greece.

Purpose: The purpose of this study was to assess the role of various diagnostic tests in early detection of retinal changes in β-thalassemia major patients.

Methods: Thirty-eight visually asymptomatic β-thalassemia major patients receiving regular blood transfusions and iron-chelation therapy with deferoxamine (group A, n = 13), deferasirox (group B, n = 11) or deferoxamine with deferiprone (group C, n = 14) and fourteen age- and sex- matched healthy individuals were included in the study. All participants underwent ophthalmoscopy, full-field electroretinography (ERG), visual evoked potentials (VEP), multifocal electroretinography (mfERG), fundus autofluorescence (FAF) imaging and optical coherence tomography (OCT) scans. Read More

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http://link.springer.com/10.1007/s00417-017-3650-9
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http://dx.doi.org/10.1007/s00417-017-3650-9DOI Listing
July 2017
23 Reads

[Prevalence and risk factors for sickle retinopathy in a sub-Saharan comprehensive Sickle Cell Center].

Rev Med Interne 2017 Sep 23;38(9):572-577. Epub 2017 Feb 23.

Centre de recherche et de lutte contre la drépanocytose (CRLD), point G, commune III, 03 BP 186, BKO 03, Bamako, Mali; Service d'hématologie-oncologie médicale du CHU du point G, BP 333, Bamako, Mali. Electronic address:

Introduction: Retinopathy is a chronic complication with severe functional consequences in patients with sickle cell disease. Its prevalence is not well known in sub-Saharan Africa because of the absence of screening. We report here the results of a routine screening for sickle retinopathy in a Comprehensive Sickle Cell Center in Sub-Saharan Africa. Read More

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http://dx.doi.org/10.1016/j.revmed.2017.01.010DOI Listing
September 2017
10 Reads

Evaluation of Macular Vascular Abnormalities Identified by Optical Coherence Tomography Angiography in Sickle Cell Disease.

Am J Ophthalmol 2017 May 14;177:90-99. Epub 2017 Feb 14.

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

Purpose: To evaluate macular vascular flow abnormalities identified by optical coherence tomography angiography (OCT-A) in patients with various sickle cell genotypes.

Design: Prospective, observational case series.

Methods: This is a single-institution case series of adult patients with various sickle cell genotypes. Read More

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http://dx.doi.org/10.1016/j.ajo.2017.02.007DOI Listing
May 2017
12 Reads

A retinopathy in young patient with co-inheritance of heterozygous alpha + -thalassemia and sickle trait: a case report.

BMC Ophthalmol 2017 Jan 18;17(1). Epub 2017 Jan 18.

Private Ophthalmologists, Rabat, Morocco.

Background: The retinopathy is an uncommon complication in individuals with sickle cell trait except for the cases of sickle cell trait associated with systemic arterial hypertension, diabetes mellitus, syphilis, tuberculosis and sarcoidosis.

Case Presentation: A retinopathy in a 16 year-old child with no history of consanguinity in the parents revealed a sickle S trait associated to heterozygous alpha thalassemia. His mother has Sickle cell anaemia (Hb SS) and his father is a carrier of heterozygous alpha-thalassemia status that it was unknown before. Read More

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http://dx.doi.org/10.1186/s12886-017-0402-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5242008PMC
January 2017
36 Reads

CORRELATION OF MULTIMODAL IMAGING IN SICKLE CELL RETINOPATHY.

Retina 2016 Dec;36 Suppl 1:S111-S117

*Department of Ophthalmology, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California; †Eye Research Center, Rassoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran; ‡Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, Maryland; and §Greater Los Angeles VA Healthcare Center, Los Angeles, California.

Purpose: To correlate macular findings on spectral domain optical coherence tomography (SDOCT) and optical coherence tomography angiography (OCTA) with quantitative ischemic index calculations on ultra-wide-field fluorescein angiography (UWFFA) in patients with sickle cell retinopathy.

Methods: In this retrospective case series, SDOCT, OCTA, and UWFFA images of patients with sickle cell retinopathy were evaluated. Eyes were staged based on the Goldberg classification of proliferative sickle cell retinopathy. Read More

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http://dx.doi.org/10.1097/IAE.0000000000001230DOI Listing
December 2016
12 Reads

Expression Pattern of HIF-1α and VEGF Supports Circumferential Application of Scatter Laser for Proliferative Sickle Retinopathy.

Invest Ophthalmol Vis Sci 2016 12;57(15):6739-6746

Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States.

Purpose: Retinal vascular occlusions in sickle cell anemia patients cause tissue ischemia and the release of angiogenic mediators that promote the development of retinal neovascularization, initiating proliferative sickle retinopathy (PSR). Laser photocoagulation (LPC) has emerged as the most common treatment for PSR. Nonetheless, only two randomized controlled clinical trials have evaluated the use of LPC for PSR, and both failed to definitively demonstrate efficacy of this approach. Read More

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http://dx.doi.org/10.1167/iovs.16-19513DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5156513PMC
December 2016
20 Reads

Prognostic value of T786C and G894T eNOS polymorphisms in sickle cell disease.

Nitric Oxide 2017 Jan 18;62:17-23. Epub 2016 Nov 18.

Metropolitan Hospital, Athens, Greece.

Endothelial Nitric Oxide Synthase (eNOS) is crucial for vascular homeostasis. Polymorphisms T786C and G894T affect eNOS regulation and have been related to various diseases. Sickle Cell Disease (SCD), a clinically diverse chronic hemolytic anemia, implies impaired nitric oxide bioavailability. Read More

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http://dx.doi.org/10.1016/j.niox.2016.11.002DOI Listing
January 2017
18 Reads

SPECTRAL DOMAIN VERSUS SWEPT SOURCE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY OF THE RETINAL CAPILLARY PLEXUSES IN SICKLE CELL MACULOPATHY.

Retin Cases Brief Rep 2018 Spring;12(2):87-92

East Bay Retina Consultants Inc, Oakland, California.

Purpose: To compare the spectral domain and swept source optical coherence tomography angiography findings in two cases of sickle cell maculopathy.

Methods: A 53-year-old man and a 24-year-old man both with sickle cell disease (hemoglobin SS) presented with no visual complaints; Humphrey visual field testing demonstrated asymptomatic paracentral scotomas that extended nasally in the involved eyes. Clinical examination and multimodal imaging including spectral domain and swept source optical coherence tomography, and spectral domain optical coherence tomography angiography and swept source optical coherence tomography angiography (Carl Zeiss Meditec Inc, Dublin, CA) were performed. Read More

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http://dx.doi.org/10.1097/ICB.0000000000000448DOI Listing
June 2018
9 Reads

ELECTRORETINOGRAPHIC AND VISUAL-EVOKED POTENTIAL CHANGES IN RELATION TO CHELATION MODALITY IN CHILDREN WITH THALASSEMIA.

Retina 2017 Jun;37(6):1168-1175

Departments of *Ophthalmology and †Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Purpose: To evaluate possible benefits of using electrophysiological investigations for detecting retinal and visual pathway changes and correlating them with chelation modality in children with thalassemia.

Methods: This study included 60 patients on single oral iron chelator (deferasirox) (Group 1), 60 on deferoxamine chelator (Group 2), and 60 controls (Group 3). Participants underwent full ophthalmologic examination, pattern visual-evoked potential, pattern electroretinogram, and multifocal electroretinogram. Read More

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http://dx.doi.org/10.1097/IAE.0000000000001315DOI Listing
June 2017
8 Reads

Impact of a Clinical Pharmacy Service on the Management of Patients in a Sickle Cell Disease Outpatient Center.

Pharmacotherapy 2016 11 3;36(11):1166-1172. Epub 2016 Oct 3.

Comprehensive Sickle Cell Center, Section of Hematology/Oncology, Department of Medicine, University of Illinois at Chicago, Chicago, Illinois.

Ambulatory care clinical pharmacy services have expanded beyond primary care settings, but literature supporting the benefits of clinical pharmacy involvement with patients who have rare diseases such as sickle cell disease (SCD) is lacking. Hydroxyurea is the only agent approved by the U.S. Read More

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http://dx.doi.org/10.1002/phar.1834DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5373798PMC
November 2016
21 Reads

Ophthalmic Manifestations of Sickle Cell Disease.

Authors:
Adrienne W Scott

South Med J 2016 Sep;109(9):542-8

From the Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Sickle cell disease (SCD), the most common inherited blood disorder, is characterized by defective oxygen transport. Every part of the eye can be affected by microvascular occlusions from SCD; however, the major cause of vision loss is proliferative sickle cell retinopathy (PSR). Although individuals with the HbSS genotype of SCD manifest more systemic morbidity and those with the HbSC genotype have a milder clinical course, those with HbSC have an increased risk of developing PSR and resultant vision loss. Read More

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http://dx.doi.org/10.14423/SMJ.0000000000000525DOI Listing
September 2016
2 Reads

Association between Sickle Cell Trait and the Prevalence and Severity of Diabetic Retinopathy.

PLoS One 2016 14;11(7):e0159215. Epub 2016 Jul 14.

Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia.

Purpose: To determine whether Sickle cell trait (SCT) is associated with an increased severity of diabetic retinopathy.

Methods: This was a single center retrospective study case control study of 100 eyes of 100 patients with diabetes mellitus (DM) with SCT (SCT group) and 100 eyes of 100 age-matched patients with DM without SCT (control group). The main outcome measure was the difference in the prevalence of sight threatening DR [here defined as diabetic macular edema (DME) and/or proliferative diabetic retinopathy (PDR)], between the SCT and control groups. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0159215PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4944991PMC
July 2017
6 Reads

Oral Monomethyl Fumarate Therapy Ameliorates Retinopathy in a Humanized Mouse Model of Sickle Cell Disease.

Antioxid Redox Signal 2016 12 22;25(17):921-935. Epub 2016 Aug 22.

1 Department of Biochemistry and Molecular Biology, The Medical College of Georgia at Augusta University , Augusta, Georgia .

Aims: Sickle retinopathy (SR) is a major cause of blindness in sickle cell disease (SCD). The genetic mutation responsible for SCD is known, however; oxidative stress and inflammation also figure prominently in the development and progression of pathology. Development of therapies for SR is hampered by the lack of (a) animal models that accurately recapitulate human SR and (b) strategies for noninvasive yet effective retinal drug delivery. Read More

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http://dx.doi.org/10.1089/ars.2016.6638DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5144884PMC
December 2016
18 Reads

β-Thalassemia and ocular implications: a systematic review.

BMC Ophthalmol 2016 Jul 8;16:102. Epub 2016 Jul 8.

1st Department of Ophthalmology, Athens University, Mesogeion 154, Athens, 11527, Greece.

Background: Beta-thalassemia is a severe genetic blood disorder caused by a mutation in the gene encoding for the beta chains of hemoglobin. Individuals with beta-thalassemia major require regular lifelong Red Blood Cell transfusions to survive. Ocular involvement is quite common and may have serious implications. Read More

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http://dx.doi.org/10.1186/s12886-016-0285-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4938965PMC
July 2016
19 Reads

Gene therapy: Myth or reality?

Authors:
Alain Fischer

C R Biol 2016 Jul-Aug;339(7-8):314-8. Epub 2016 May 31.

Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, 75015 Paris, France; Immunology and Pediatric Hematology Department, Assistance publique-Hôpitaux de Paris, 75015 Paris, France; Inserm UMR 1163, 75015 Paris, France; Collège de France, 75005 Paris, France. Electronic address:

Gene therapy has become a reality, although still a fragile one. Clinical benefit has been achieved over the last 17years in a limited number of medical conditions for which pathophysiological studies determined that they were favorable settings. They include inherited disorders of the immune system, leukodystrophies, possibly hemoglobinopathies, hemophilia B, and retinal dystrophies. Read More

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http://dx.doi.org/10.1016/j.crvi.2016.04.011DOI Listing
February 2017
5 Reads

Cross-Sectional Analysis of Neurocognitive Function, Retinopathy, and Retinal Thinning by Spectral-Domain Optical Coherence Tomography in Sickle Cell Patients.

Middle East Afr J Ophthalmol 2016 Jan-Mar;23(1):79-83

Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, IL, USA.

Purpose: The purpose was to examine the relationship between neurocognitive function and two distinct forms of retinopathy in sickle cell disease.

Materials And Methods: Patients with sickle cell disease (n = 44, age range: 19-56 years, 70% female) were prospectively recruited for this cross-sectional study. Retinopathy was characterized by: (1) Presence of focal retinal thinning on spectral domain optical coherence tomography and (2) determination of the sickle retinopathy stage on funduscopic exam based on Goldberg classification. Read More

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http://dx.doi.org/10.4103/0974-9233.150632DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4759909PMC
August 2016
10 Reads

Capillary nonperfusion by novel technology of OCT angiography in a patient with sickle cell disease with normal fluorescein angiogram.

Eur J Ophthalmol 2016 Aug 4;26(5):e121-3. Epub 2016 Aug 4.

Department of Ophthalmology, University of Florida College of Medicine-Jacksonville, FL - USA.

Purpose: Retinal ischemia is a pathophysiologic feature of sickle cell retinopathy. Inner retinal thinning of retina temporal to the fovea has been reported on spectral-domain optical coherence tomography (SD-OCT) even though clinical examination is normal and fluorescein angiography (FA) does not show any capillary dropout. In a patient with sickle cell disease with temporal inner retinal thinning on SD-OCT and normal FA, the new technology of OCT angiography (OCTA) showed a corresponding area of nonperfusion. Read More

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http://dx.doi.org/10.5301/ejo.5000765DOI Listing
August 2016
9 Reads

Risk factors for visual impairment in patients with sickle cell disease in London.

Eur J Ophthalmol 2016 Aug 29;26(5):431-5. Epub 2016 Feb 29.

Department of Ophthalmology, Guy's and St. Thomas' NHS Foundation Trust, London - UK.

Purpose: Dramatically improved health care in recent years has increased the life expectancy of patients with sickle cell disease (SCD) as well as the prognosis for its ocular complications. We sought to identify risk factors for visual impairment in patients with SCD in London 4 decades after Goldberg's seminal studies.

Methods: Patients 16 years and older with SCD (genotypes HbSS, HbSC, HbSβ-thalassemia) attending hematology and ophthalmology services were offered ocular examination. Read More

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http://dx.doi.org/10.5301/ejo.5000767DOI Listing
August 2016
7 Reads
2 Citations
1.060 Impact Factor

Macular Microangiopathy in Sickle Cell Disease Using Optical Coherence Tomography Angiography.

Am J Ophthalmol 2016 Apr 31;164:137-44.e1. Epub 2015 Dec 31.

University Eye Clinic, Centre Hospitalier Intercommunal de Créteil, University Paris Est, Créteil, France. Electronic address:

Purpose: To characterize the optical coherence tomography angiography (OCTA) appearance of the perifoveal macular microvasculature in visually asymptomatic patients with sickle cell disease, and to compare these findings with those of fluorescein angiography (FA).

Design: Retrospective observational case series.

Methods: Eighteen eyes of 9 consecutive patients with a median age of 41 years (range: 19-54 years) with electrophoretic confirmation of sickle cell disease were included and analyzed. Read More

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http://dx.doi.org/10.1016/j.ajo.2015.12.023DOI Listing
April 2016
8 Reads
13 Citations
3.871 Impact Factor

Temporal Macular Atrophy as a Predictor of Neovascularization in Sickle Cell Retinopathy.

Ophthalmic Surg Lasers Imaging Retina 2016 Jan;47(1):27-34

Background And Objective: To evaluate the association between temporal macular atrophy and the presence of neovascularization in eyes with sickle cell disease (SCD).

Patients And Methods: Retrospective, case-controlled study identifying 64 eyes from 38 consecutive patients with SCD. Dilated funduscopic examination and wide-field fluorescein angiography were used to identify the Goldberg stage of proliferative sickle cell retinopathy. Read More

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http://dx.doi.org/10.3928/23258160-20151214-04DOI Listing
January 2016
3 Reads

Unusual presentation of ocular trauma in sickle cell trait.

Authors:
Nidhi Pandey

Indian J Ophthalmol 2015 Sep;63(9):738-40

Department of Ophthalmology, Pt. Jawahar Lal Nehru Memorial Medical College, Raipur, Chhattisgarh, India.

Sickle cell trait is usually considered as a benign condition. However under certain adverse circumstances, it can give rise to vaso-occlusive features as in sickle cell disease. We present here two cases, both involving healthy young males, who developed retinal vaso-occlusive features following blunt ocular trauma. Read More

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http://dx.doi.org/10.4103/0301-4738.170985DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4705713PMC
September 2015
4 Reads