Article
Gyrate atrophy of the choroid and retina. Long-term reduction of ornithine slows retinal degeneration.
Archives of ophthalmology (Chicago, Ill. : 1960) - 1 Nov 1991
Kaiser-Kupfer M I, Caruso R C, Valle D
Abstract excerpt
Gyrate atrophy of the choroid and retina is an autosomal recessive, chorioretinal dystrophy that begins in childhood and leads to blindness in the fourth to seventh decade of life. The primary defect is deficiency of ornithine-delta-amino-transferase, which results in accumulation of ornithine. W...
Topics
- Adult
- Arginine
- Child
- Child, Preschool
- Female
- Follow-Up Studies
- Fundus Oculi
- Genetic Variation
- Gyrate Atrophy
- Humans
- Infant
- Longitudinal Studies
- Male
- Middle Aged
- Ornithine
- Ornithine-Oxo-Acid Transaminase
- Retinal Degeneration
- Treatment Outcome