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Ophthalmic Surg Lasers Imaging Retina 2018 Oct;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

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

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

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

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

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

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

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

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

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

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

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

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

JAMA Ophthalmol 2017 Oct;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

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

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

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

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

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

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

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.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Hum Gene Ther 2016 Feb;27(2):96-7

Coeditors.

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

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

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

Indian J Ophthalmol 2015 Sep;63(9):710-5

Department of Optometry, School of Paramedical Science, Mashhad University of Medical Sciences, Mashhad, Iran.

Aims: The aim of this study was to assess ocular changes in thalassemia patients who have received multiple transfusions and chelate binding therapy in order to avoid iron accumulation.

Settings And Design: A cross-sectional study.

Subjects And Methods: A total of 54 thalassemia major patients were selected as case group, and 54 age- and sex-matched healthy subjects were regarded as a control group. Read More

Am J Hematol 2016 Feb;91(2):238-42

Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Although hemoglobin SC (HbSC) disease is usually considered less severe than sickle cell anemia (SCA), which includes HbSS and HbS/β(0) -thalassemia genotypes, many patients with HbSC experience severe disease complications, including vaso-occlusive pain, acute chest syndrome, avascular necrosis, retinopathy, and poor quality of life. Fully 20 years after the clinical and laboratory efficacy of hydroxyurea was proven in adult SCA patients, the safety and utility of hydroxyurea treatment for HbSC patients remain unclear. Recent NHLBI evidence-based guidelines highlight this as a critical knowledge gap, noting HbSC accounts for ∼30% of sickle cell patients within the United States. Read More

J Assoc Physicians India 2015 May;63(5):95-6

Gac Med Mex 2015 Nov-Dec;151(6):757-63

Departamento de Investigación Clínica, Grupo Diagnóstico Médico Proa, México, D.F., México.

Hemoglobin S is an abnormal protein that induces morphological changes in erythrocyte in low-oxygen conditions. In Mexico, it is reported that up to 13.7% of the population with mutation in one allele are considered asymptomatic (sickle cell trait). Read More