Disorders of Ornithine and Creatine Metabolism

  • Vivian E. Shih
  • Sylvia Stöckler-Ipsiroglu


Hyperornithinemia due to ornithine aminotransferase (OAT) deficiency is associated with gyrate atrophy (GA) of the choroid and retina. Patients usually become virtually blind by age 55 years. Treatment includes pharmacological doses of pyridoxine (vitamin B6) and/or a low-arginine diet. Preliminary results are encouraging.


Creatine Supplementation Orotic Acid Creatine Monohydrate Chorioretinal Atrophy Gyrate Atrophy 
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Copyright information

© Springer-Verlag Berlin Heidelberg 2000

Authors and Affiliations

  • Vivian E. Shih
  • Sylvia Stöckler-Ipsiroglu

There are no affiliations available

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