A rare, autosomal recessive, pigment dysfunction disease characterized by early formation of cataract, myopia, and progressive chorioretinal degeneration. It is caused by a deficiency in the ornithine aminotransferase (OAT) enzyme and can lead to nyctalopia, circular pigment patches in the mid-peripheral fundus, loss of tunnel vision, and loss of peripheral vision.

Also Known As

  • HOGA
  • Hyperornithinemia
  • OAT Deficiency
  • OKT Deficiency
  • Ornithine Aminotransferase Deficiency
  • Ornithine-Delta-Aminotransferase Deficiency
  • Ornithine Ketoacid Aminotransferase Deficiency
  • Ornithine with Gyrate Atrophy
  • Gyrate Atrophy of the Choroid and Retina