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Gyrate atrophy of the choroid and retina

(JIE-rayt • AT-ruh-fee • of • the • KOR-oid • and • REH-tuh-nuh)

General Condition Information

Other Names

  • Gyrate atrophy
  • HOGA
  • Hyperornithine with gyrate deficiency
  • OAT deficiency
  • OKT deficiency
  • Ornithine aminotransferase deficiency
  • Ornithine-delta-aminotransferase deficiency

Condition Type

Birth Prevalence

  • It is unknown how many babies are born with this rare condition each year in the United States.

Screening Finding

Increased ornithine

What is gyrate atrophy of the choroid and retina

Gyrate atrophy of the choroid and retina is an inherited (genetic) condition that prevents the breakdown of a substance in the blood called ornithine. Ornithine is an amino acid, one of the building blocks of protein.

Ornithine aminotransferase is an enzyme that helps break down ornithine. Ornithine aminotransferase helps balance amino acids so they can correctly produce proteins.

Without enough working ornithine aminotransferase enzyme, your baby will have trouble breaking down ornithine. High amounts of ornithine can damage your baby’s eyes and other organs. Left untreated, this damage leads to the signs and symptoms of the condition.

Newborn Screening and Follow-Up

Condition Details

Treatment and Management

It is important to talk to your health care provider about which treatment(s) are best for your baby. The goal of treatment is to prevent the health problems caused by this condition.

Treatments may include the following:

  • Diet low in certain proteins
  • Vitamins and other supplements
  • Vision aids, like eyeglasses

Children who receive early and ongoing treatment for Gyrate atrophy of the choroid and retina can lead healthier lives.

Date Last Reviewed: