Abstract
Elevated foetal haemoglobin (HbF) levels are protective against some manifestations of sickle cell anaemia but the impact on retinopathy is unknown. We report on 123 children with HbSS, 10.6% of whom developed retinopathy. Independent of hydroxycarbamide, children with a HbF <15% had 7.1-fold (95% confidence interval, 1.5-33.6) higher odds of developing retinopathy. In children treated with hydroxycarbamide, those with retinopathy had lower HbF levels compared to children without retinopathy (9% vs. 16%; P = 0.005). We report a protective benefit of elevated HbF regarding retinopathy, and our data suggests induction of HbF with hydroxycarbamide may prevent retinopathy in children.
© 2013 Blackwell Publishing Ltd.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Adolescent
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Anemia, Sickle Cell / complications*
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Anemia, Sickle Cell / drug therapy
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Antisickling Agents / therapeutic use*
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Child
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Drug Evaluation
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Female
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Fetal Hemoglobin / analysis*
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Fetal Hemoglobin / biosynthesis
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Fetal Hemoglobin / genetics
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Follow-Up Studies
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Gene Expression Regulation / drug effects
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Humans
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Hydroxyurea / therapeutic use*
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Incidence
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Male
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Retinal Diseases / blood
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Retinal Diseases / epidemiology
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Retinal Diseases / etiology
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Retinal Diseases / prevention & control*
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Retrospective Studies
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Risk
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Vitreoretinopathy, Proliferative / blood
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Vitreoretinopathy, Proliferative / epidemiology
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Vitreoretinopathy, Proliferative / etiology
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Vitreoretinopathy, Proliferative / prevention & control
Substances
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Antisickling Agents
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Fetal Hemoglobin
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Hydroxyurea