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Paramacular temporal atrophy in sickle cell disease occurs early in childhood

British Journal of Ophthalmology Aug 26, 2018

Martin GC, et al. - Researchers described paramacular temporal atrophy in children affected with sickle cell disease (SCD). The occurrence of paramacular temporal atrophy was seen to be early in the course of SCD, which suggests distinct mechanisms from those of peripheral retinopathy. In 11% of children, peripheral retinopathy was found, with a significant association between the HbSC genotype and the severity of the retinopathy. Using a specific temporal pattern, 53% of eyes demonstrated retinal atrophy with no significant difference in the prevalence between HbSS and HbSC genotype, and no impact of age (mean 12.3 years vs 11.9).
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