Treatment of gyrate atrophy of the choroid and retina with low arginine diet (2025)

. 1980;78:226–242.

R R McInnes

, S A Arshinoff

, L Bell

, C McCulloch

PMCID: PMC1312141PMID: 7257057

Abstract

In gyrate atrophy the blood ornithine is grossly elevated, due to deficiency of ornithine ketoacid transaminase, which converts ornithine towards glutamic acid. Two patients with gyrate atrophy have been treated with a low arginine diet and their blood ornithine levels have been reduced to near normal. At this level hyperammonemia may result from overtreatment, but this can be quickly cleared by a small dose of arginine. There has also been some improvement in vision, but no clearing of the gyrate areas. Future care with this regimen seems possible and improvements in handling of these patients are likely.

Full text

PDF
Treatment of gyrate atrophy of the choroid and retina with low arginine diet (1)

Images in this article

Treatment of gyrate atrophy of the choroid and retina with low arginine diet (2)FIGURE 2on p.228

Treatment of gyrate atrophy of the choroid and retina with low arginine diet (3)FIGURE 5on p.232

Treatment of gyrate atrophy of the choroid and retina with low arginine diet (4)FIGURE 6on p.233

Treatment of gyrate atrophy of the choroid and retina with low arginine diet (5)FIGURE 7on p.235

Treatment of gyrate atrophy of the choroid and retina with low arginine diet (6)FIGURE 8on p.237

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Arshinoff S. A., McCulloch J. C., Matuk Y., Phillips M. J., Gordon B. A., Marliss E. B. Amino-acid metabolism and liver ultrastructure in hyperornithinemia with gyrate atrophy of the choroid and retina. Metabolism. 1979 Oct;28(10):979–988. doi: 10.1016/0026-0495(79)90001-5. [DOI] [PubMed] [Google Scholar]
  2. Berson E. L., Schmidt S. Y., Rabin A. R. Plasma amino-acids in hereditary retinal disease. Ornithine, lysine, and taurine. Br J Ophthalmol. 1976 Feb;60(2):142–147. doi: 10.1136/bjo.60.2.142. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. McCulloch C., Marliss E. B. Gyrate atrophy of the choroid and retina: clinical, ophthalmologic, and biochemical considerations. Trans Am Ophthalmol Soc. 1975;73:153–171. [PMC free article] [PubMed] [Google Scholar]
  4. McCulloch J. C., Arshinoff S. A., Marliss E. B., Parker J. A. Hyperornithinemia and gyrate atrophy of the choroid and retina. Ophthalmology. 1978 Sep;85(9):918–928. doi: 10.1016/s0161-6420(78)35598-6. [DOI] [PubMed] [Google Scholar]
  5. O'Donnell J. J., Sandman R. P., Martin S. R. Deficient L-ornithine: 2-oxoacid aminotransferase activity in cultured fibroblasts from a patient with gyrate atrophy of the retina. Biochem Biophys Res Commun. 1977 Nov 21;79(2):396–399. doi: 10.1016/0006-291x(77)90170-x. [DOI] [PubMed] [Google Scholar]
  6. Phang J. M., Downing S. J., Valle D. A radioisotopic assay for ornithine-delta-transaminase. Anal Biochem. 1973 Sep;55(1):272–277. doi: 10.1016/0003-2697(73)90313-8. [DOI] [PubMed] [Google Scholar]
  7. ROSE W. C., HAINES W. J., WARNER D. T. The amino acid requirements of man. V. The rôle of lysine, arginine, and tryptophan. J Biol Chem. 1954 Jan;206(1):421–430. [PubMed] [Google Scholar]
  8. SNYDERMAN S. E., BOYER A., HOLT L. E., Jr The arginine requirement of the infant. AMA J Dis Child. 1959 Feb;97(2):192–195. doi: 10.1001/archpedi.1959.02070010194006. [DOI] [PubMed] [Google Scholar]
  9. Shih V. E., Berson E. L., Mandell R., Schmidt S. Y. Ornithine ketoacid transaminase deficiency in gyrate atrophy of the choroid and retina. Am J Hum Genet. 1978 Mar;30(2):174–179. [PMC free article] [PubMed] [Google Scholar]
  10. Shih V. E., Schulman J. D. Ornithine-ketoacid transaminase activity in human skin and amniotic fluid cell culture. Clin Chim Acta. 1970 Jan;27(1):73–75. doi: 10.1016/0009-8981(70)90376-1. [DOI] [PubMed] [Google Scholar]
  11. Simell O., Takki K. Raised plasma-ornithine and gyrate atrophy of the choroid and retina. Lancet. 1973 May 12;1(7811):1031–1033. doi: 10.1016/s0140-6736(73)90667-3. [DOI] [PubMed] [Google Scholar]
  12. Simell O., Takki K. Raised plasma-ornithine and gyrate atrophy of the choroid and retina. Lancet. 1973 May 12;1(7811):1031–1033. doi: 10.1016/s0140-6736(73)90667-3. [DOI] [PubMed] [Google Scholar]
  13. Takki K., Simell O. Genetic aspects in gyrate atrophy of the choroid and retina with hyperornithinaemia. Br J Ophthalmol. 1974 Nov;58(11):907–916. doi: 10.1136/bjo.58.11.907. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Trijbels J. M., Sengers R. C., Bakkeren J. A., De Kort A. F., Deutman A. F. L-Ornithine-ketoacid-transaminase deficiency in cultured fibroblasts of a patient with hyperornithinaemia and gyrate atrophy of the choroid and retina. Clin Chim Acta. 1977 Sep 1;79(2):371–377. doi: 10.1016/0009-8981(77)90431-4. [DOI] [PubMed] [Google Scholar]
  15. Valle D., Kaiser-Kupfer M. I., Del Valle L. A. Gyrate atrophy of the choroid and retina: deficiency of ornithine aminotransferase in transformed lymphocytes. Proc Natl Acad Sci U S A. 1977 Nov;74(11):5159–5161. doi: 10.1073/pnas.74.11.5159. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Valle D., Walser M., Brusilow S. W., Kaiser-Kupfer M. Gyrate atrophy of the choroid and retina: amino acid metabolism and correction of hyperornithinemia with an arginine-deficient diet. J Clin Invest. 1980 Feb;65(2):371–378. doi: 10.1172/JCI109680. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Walser M., Batshaw M., Sherwood G., Robinson B., Brusilow S. Nitrogen metabolism in neonatal citrullinaemia. Clin Sci Mol Med. 1977 Aug;53(2):173–181. doi: 10.1042/cs0530173. [DOI] [PubMed] [Google Scholar]
  18. Weleber R. G., Kennaway N. G., Buist N. R. Vitamin B6 in management of gyrate atrophy of choroid and retina. Lancet. 1978 Dec 2;2(8101):1213–1213. doi: 10.1016/s0140-6736(78)92211-0. [DOI] [PubMed] [Google Scholar]
Treatment of gyrate atrophy of the choroid and retina with low arginine diet (2025)

References

Top Articles
Latest Posts
Recommended Articles
Article information

Author: Chrissy Homenick

Last Updated:

Views: 6747

Rating: 4.3 / 5 (54 voted)

Reviews: 85% of readers found this page helpful

Author information

Name: Chrissy Homenick

Birthday: 2001-10-22

Address: 611 Kuhn Oval, Feltonbury, NY 02783-3818

Phone: +96619177651654

Job: Mining Representative

Hobby: amateur radio, Sculling, Knife making, Gardening, Watching movies, Gunsmithing, Video gaming

Introduction: My name is Chrissy Homenick, I am a tender, funny, determined, tender, glorious, fancy, enthusiastic person who loves writing and wants to share my knowledge and understanding with you.