|
People suffering from gyrate atrophy of the choroid (the thin coating of the eye) and retina face a progressive loss of vision, with total blindness usually occurring between the ages of 40 and 60. The disease is an inborn error of metabolism.
|
www.ncbi.nlm.nih.gov/disease/GyrateA.html
www.ncbi.nlm.nih.gov/disease/GyrateA.html
|
|
|
|
|
In gyrate atrophy the blood ornithine is grossly elevated, due to deficiency of ornithine ketoacid transaminase, which converts ornithine towards glutamic ...
|
www.ncbi.nlm.nih.gov/pmc/articles/PMC1312141/
|
|
|
Due to this stabilization, we would recommend a dietary treatment in gyrate atrophy patients. University Eye Hospital, Dept. II, Schleichstr. 12-16, D-72076 Tübingen ; Supported by the DFG (SFB 430, project C2) and fortuene grant Nr. 517 ;
|
www.dog.org/1999/e-abstract99/431.html
|
|
GYRATE ATROPHY (GA) of the choroid and retina (Figure 1) is a rare, autosomal recessive disease causing progressive chorioretinal degeneration resulting in blindness. It is caused by a deficiency of ornithine -aminotransferase (OAT) (1). It was first described as "atypical retinitis pigmentosa" by Jacobsohn in 1888.
|
path.upmc.edu/cases/case423/dx.html
|
|
Deficiency of ornithine-delta-aminotransferase (OAT) in humans results in gyrate atrophy of the choroid and retina (GA), an autosomal recessive disorder characterized by ornithine accumulation and a progressive chorioretinal degeneration of unknown pathogenesis. ... The Medscape Journal ... Allergy & Clinical Immunology...
|
www.medscape.com/medline/abstract/10655512
|
|
OBJECTIVE: To evaluate peripheral nervous system involvement in gyrate atrophy of the choroid and retina with hyperornithinemia (GA). ... OBJECTIVE: To evaluate peripheral nervous system involvement in gyrate atrophy of the choroid and retina with hyperornithinemia (GA). BACKGROUND: GA is an inborn error of amino...
|
www.medscape.com/medline/abstract/12221166
|
|
Gyrate atrophy of the retina and choroid is a rare autosomal recessive inherited disease, characterized by progressive chorioretinal atrophy that results in progressive deterioration of peripheral and night vision and leading to blindness.
|
jmedicalcasereports.com/content/1/1/27
|
|
In a patient with gyrate atrophy of the choroid and retina, an arginine-deficient diet has reduced plasma ornithine concentration fivefold during the past 20 months. Subjective improvement in her visual function was noted approximately 15 months after institution of her diet.
|
www.sciencemag.org/cgi/content/abstract/210/4474/1128
|
|
• 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- -amino-transferase, which results in accumulation of ornithine.
|
archopht.ama-assn.org/cgi/content/abstract/109/11/1539
|
|
