Yasuhiko Hirami

A novel PRPF31 variant associated with autosomal dominant retinitis pigmentosa in Japanese families

BACKGROUND: PRPF31-associated inherited retinal dystrophy is an autosomal dominant form of retinitis pigmentosa (RP), typically caused by haploinsufficiency. Although affected individuals generally exhibit early-onset nyctalopia and progressive visual field loss, the clinical and...

Association Between PS-OCT Derived Polarimetric Entropy and RPE Cellular Metrics Measured With Adaptive Optics Imaging in Retinitis Pigmentosa

Purpose: The purpose of this study was to investigate the association between entropy values derived from the retinal pigment epithelium (RPE) layer using polarization-sensitive optical coherence tomography (PS-OCT) and RPE cellular metrics obtained by adaptive optics transsclera...