Gyrate Atrophy patient with defective ornithine aminotransferase will be benefited by
Correct Answer: Arginine free diet
Description: Ans is 'b' i.e. Arginine free diet Gyrate atrophy of the choroid and retina is a slowly progressive, diffuse, bilateral chorioretinal dystrophy with an autosomal recessive mode of inheritance.Gyrate dystrophy is caused by a deficiency of OAT (ornithine aminotransferase) also k/a ornithine keto acid aminotransferase.Deficiency of OAT leads to increased level of omit Bine (hyperomithemia).Ornithine, a nonessential amino acid, is an intermediate compound in the formation of urea. It is formed from Arginine.Arginine is converted to ornithine then to glutamate-y-semialdehyde by OAT.Deficiency of OAT leads to hyperomithemia causing gyrate atrophy.Patients develop nyctalopia during the second to third decade of life. Visual acuity is preserved until later stages. Treatment:Since ornithine is produced from other amino acids, mainly arginine a low protein diet, including near total elimination of arginine with supplementation of essential amino acids has been found to be beneficial in gyrate atrophy.Gene for the enzyme OAT has been mapped to chromosome 10q26.Replacement of the abnormal gene, or genetic engineering to produce a supply of normal enzyme, may result in a reduction of ornithine levels without dietary restrictions.
Category:
Ophthalmology
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