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Retinal atrophy and markers of systemic and cerebrovascular severity in homozygous sickle cell disease.
Eur J Ophthalmol
November 2022
Ophthalmology Department and Rare Ophthalmological Diseases Reference Centre (OPHTARA), 37072Necker-Enfants Malades University Hospital, APHP, Paris, France.
Introduction: While paramacular retinal atrophy (PRA) is known to be found in 48% of eyes of adults and 42% of eyes of children with homozygous SCD (SS-SCD), the aim of this study is to assess the association between PRA and red blood cell (RBC) deformability, hematological markers and brain imaging abnormalities in SS-SCD.
Methods: This study is a subset of , a prospective observational study performed between August 2015 and August 2016. Children (5-17 years) with SS-SCD and no history of large vessel vasculopathy, were included.