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enhanced S cone syndrome (ESCS); Goldmann-Favre syndrome; retinoschisis with early hemeralopia

Pathology: 1) retinopathy 2) retinal degeneration (variable) Genetics: 1) autosomal recessive 2) associated with defects in NR2E3 gene Clinical manifestations: 1) increased sensitivity to blue light S (short wavelength, blue) cones 2) visual loss 3) night blindness occurring from early in life 4) varying degrees of L (long, red)- & M (middle, green)-cone vision

General

genetic syndrome (multisystem disorder) genetic disease of the eye retinopathy

References

  1. OMIM :accession 268100