An eye on sickle cell retinopathy

Melo, Mônica Barbosa de

doi:10.1016/j.bjhh.2014.07.020

Cited by 14 publications

(26 citation statements)

References 20 publications

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“…Compared to HbSS patients, HbSC patients had significantly higher rates of retinopathy (44% vs. 25%, P = 0Á0013) and PSR (86% vs. 72%, P = 0Á0002, Table I). This is consistent with what is already reported in the literature (Friberg et al, 1986;De Melo, 2014).…”

Section: Resultssupporting

confidence: 94%

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

et al. 2018

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Summary 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. We aimed to investigate the effect of hydroxycarbamide use and HbF level on sickle cell retinopathy development in adults. We performed a retrospective cross‐sectional study and collected demographics, comorbidities, and ocular and haematological data from 300 adult sickle cell subjects examined at the Henkind Eye Institute at Montefiore Medical Center during a 5‐year period, from October 2012 to November 2017. The cohort was comprised mainly of Black and Hispanic subjects with all SCD genotypes, aged 18–71 years. Results show that in HbSS patients treated with hydroxycarbamide, those with retinopathy had significantly lower HbF levels compared to patients without retinopathy (P = 0·018). Our study identified the optimal HbF cut‐off point for retinopathy protection to be 14·87%. Thus, a HbF level of 15% appears to be the threshold above which the odds for developing retinopathy in SS patients are reduced by 50%.

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Section: Resultssupporting

confidence: 94%

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

et al. 2018

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“…5,7,12 Retinal changes characterize sickle cell retinopathy, which may be non-proliferative or proliferative and is divided into five stages. 1,10,12,13 Among the forms of sickle cell anemia, SS patients present a more severe systemic clinical picture than those with type SC. On the other hand, occlusive ocular effects are more predominant in SC patients, who present only moderate anemia and higher blood viscosity.…”

Section: Ocular Manifestationsmentioning

confidence: 99%

“…9 Sickle cell retinopathy, the subject of this study, develops in up to 42% of sickle cell individuals in the second decade of life. 10 The systemic manifestations of sickle cell disease may be neurological, ophthalmologic, cardiac, pulmonary, gastrointestinal/hepatobiliary, renal/genitourinary, splenic, muscular/skeletal, and growth and developmental disorders. 11 They are more severe in homozygotes for cell disease (SS) than in heterozygotes with sickle hemoglobin C (SC), and yet visual loss due to proliferative retinopathy is more common in the latter.…”

Section: Introductionmentioning

confidence: 99%

Sickle cell retinopathy: A literature review

Ribeiro

Jucá

Alves

et al. 2017

Rev. Assoc. Med. Bras.

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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. Therefore, periodic ophthalmologic monitoring of these patients is important for early diagnosis and adequate therapeutic management, which can be done localy by treating the lesions in the eyes, or systemically.

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“…These rigid erythrocytes have a tendency to stack and occlude small blood vessels of nearly any organ, including the retinal capillaries. Clinical presentations of non-proliferative sickle cell retinopathy include hemorrhages, venous tortuosity, and enlargement of the foveal avascular zone [60]. On the other hand, proliferative sickle cell retinopathy reflects more advanced disease and is characterized by retinal neovascularization in response to ischemia following occlusion, especially in the peripheral retina.…”

Section: Reviewmentioning

confidence: 99%

Human retinal microvascular imaging using adaptive optics scanning light ophthalmoscopy

Chui

Krawitz

et al. 2016

Int J Retin Vitr

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BackgroundRetinal microvascular imaging is an especially promising application of high resolution imaging since there are increasing options for therapeutic intervention and need for better structural and functional biomarkers to characterize ocular and systemic vascular diseases.Main bodyAdaptive optics scanning light ophthalmoscopy (AOSLO) is an emerging technology for improving in vivo imaging of the human retinal microvasculature, allowing unprecedented visualization of retinal microvascular structure, measurements of blood flow velocity, and microvascular network mapping. This high resolution imaging technique shows significant potential for studying physiological and pathological conditions of the retinal microvasculature noninvasively.ConclusionThis review will briefly summarize the abilities of in vivo human retinal microvasculature imaging in healthy controls, as well as patients with diabetic retinopathy, retinal vein occlusion, and sickle cell retinopathy using AOSLO and discuss its potential contribution to scientific research and clinical applications.

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An eye on sickle cell retinopathy

Cited by 14 publications

References 20 publications

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

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

Sickle cell retinopathy: A literature review

Human retinal microvascular imaging using adaptive optics scanning light ophthalmoscopy

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