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Multifocal electroretinogram findings in sickle cell maculopathy

Abstract : BACKGROUND: The aim of the present work was to describe and compare multifocal electroretinogram findings (mfERG) in patients with sickle cell disease (SCD) without clinical sign of maculopathy according to the hemoglobin genotype. METHODS: HbSS (homozygous SCD), HbSC (Hemoglobin SC disease) and HbAA (controls) individuals underwent a full ophthalmologic examination (with a fundoscopy), a spectral domain ocular coherence tomography (SD-OCT) and a mfERG. RESULTS: A total of 86 subjects were included: 54 SCD patients (107 eyes) with 32 HbSS (63 eyes) and 22 HbSC (44 eyes) and 32 controls (64 eyes). None of the eyes showed retinal clinical abnormalities. SD-OCT analysis showed that macular thickness was statistically lower in SCD eyes than in controls. mfERG analysis demonstrated a significant reduction of N1 (initial negative deflection), P1 (positive peak) and N2 (second negative deflection) response amplitude densities of HbSS eyes compared to HbAA eyes from the center (<2°) to the periphery (>15°). Implicit time response was also reduced in the center (<2°). N1 and P1 response amplitude densities of HbSC eyes were significantly lower than those of HbAA eyes from the center (<2°) to the periphery (>15°). N2 response amplitude densities were also significantly reduced in the center (<2°) and in the periphery (>10°). N1 implicit time was statistically reduced in HbSC compared to HbSS eyes. CONCLUSION: Our study is the first one to describe macular electrophysiological dysfunction in SCD patients. Moreover, we confirm that SCD maculopathy is equally frequent in HbSS and HbSC genotypes.
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Submitted on : Thursday, October 31, 2019 - 11:30:34 PM
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Laurence Beral, Marc Romana, Nathalie Lemonne, Yoann Garnier, Marie Billaud, et al.. Multifocal electroretinogram findings in sickle cell maculopathy. Eye, Nature Publishing Group: Open Access Hybrid Model Option B, 2019, ⟨10.1038/s41433-019-0499-7⟩. ⟨hal-02342557⟩



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