pmc logo image
Logo of brjopthalBritish Journal of OphthalmologyCurrent TOCInstructions for authors

Formats:

Br J Ophthalmol. 1985 April; 69(4): 283–290.
PMCID: PMC1040584
Clinical trials of vitamin B6 and proline supplementation for gyrate atrophy of the choroid and retina.
S. Hayasaka, T. Saito, H. Nakajima, O. Takahashi, K. Mizuno, and K. Tada
Abstract
Five patients with gyrate atrophy of the choroid and retina were examined ophthalmologically, especially ophthalmoscopically, to evaluate trials of vitamin B6 (pyridoxine) or supplementary proline. The oral administration of vitamin B6 was tried in two patients. The vitamin did not alter the serum ornithine level and the progression of chorioretinal atrophy in one patient (case 2). Despite a reduced serum ornithine level following vitamin B6 administration, chorioretinal atrophy progressed gradually in another patient (case 3). Supplementary proline was tried in four patients. Despite the supplementation the serum proline level did not increase, and the chorioretinal atrophy progressed in one patient (case 2) who received both vitamin B6 and proline. Supplementary proline minimised the progression of gyrate atrophy in the youngest patient (case 1) and halted the progression in two others (cases 4 and 5). Supplementary proline may possibly lessen the progression of chorioretinal lesions in gyrate atrophy.
Full text
Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (2.2M), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.
Images in this article
Click on the image to see a larger version.
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
  • Simell O, Takki K. Raised plasma-ornithine and gyrate atrophy of the choroid and retina. Lancet. 1973 May 12;1(7811):1031–1033. [PubMed]
  • Shih VE, Berson EL, Mandell R, Schmidt SY. 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]
  • Kaiser-Kupfer MI, Valle D, Del Valle LA. A specific enzyme defect in gyrate atrophy. Am J Ophthalmol. 1978 Feb;85(2):200–204. [PubMed]
  • O'Donnell JJ, Sandman RP, Martin SR. Gyrate atrophy of the retina: inborn error of L-ornithin:2-oxoacid aminotransferase. Science. 1978 Apr 14;200(4338):200–201. [PubMed]
  • Weleber RG, Kennaway NG, Buist NR. Vitamin B6 in management of gyrate atrophy of choroid and retina. Lancet. 1978 Dec 2;2(8101):1213. [PubMed]
  • Weleber RG, Kennaway NG. Clinical trial of vitamin B6 for gyrate atrophy of the choroid and retina. Ophthalmology. 1981 Apr;88(4):316–324. [PubMed]
  • Kaiser-Kupfer MI, de Monasterio F, Valle D, Walser M, Brusilow S. Visual results of a long-term trial of a low-arginine diet in gyrate atrophy of choroid and retina. Ophthalmology. 1981 Apr;88(4):307–310. [PubMed]
  • Berson EL, Shih VE, Sullivan PL. Ocular findings in patients with gyrate atrophy on pyridoxine and low-protein, low-arginine diets. Ophthalmology. 1981 Apr;88(4):311–315. [PubMed]
  • Valle D, Walser M, Brusilow S, Kaiser-Kupfer MI, Takki K. Gyrate atrophy of the choroid and retina. Biochemical considerations and experience with an arginine-restricted diet. Ophthalmology. 1981 Apr;88(4):325–330. [PubMed]
  • McInnes RR, Arshinoff SA, Bell L, Marliss EB, McCulloch JC. Hyperornithinaemia and gyrate atrophy of the retina: improvement of vision during treatment with a low-arginine diet. Lancet. 1981 Mar 7;1(8219):513–516. [PubMed]
  • Sipilä I, Rapola J, Simell O, Vannas A. Supplementary creatine as a treatment for gyrate atrophy of the choroid and retina. N Engl J Med. 1981 Apr 9;304(15):867–870. [PubMed]
  • Hayasaka S, Saito T, Nakajima H, Takaku Y, Shiono T, Mizuno K, Ohmura K, Tada K. Gyrate atrophy with hyperornithinaemia: different types of responsiveness to vitamin B6. Br J Ophthalmol. 1981 Jul;65(7):478–483. [PMC free article] [PubMed]
  • Saito T, Omura K, Hayasaka S, Nakajima H, Mizuno K, Tada K. Hyperornithinemia with gyrate atrophy of the choroid and retina: a disturbance in de novo formation of proline. Tohoku J Exp Med. 1981 Dec;135(4):395–402. [PubMed]
  • Hayasaka S, Shiono T, Takaku Y, Mizuno K. Ornithine ketoacid aminotransferase in the bovine eye. Invest Ophthalmol Vis Sci. 1980 Dec;19(12):1457–1460. [PubMed]
  • Shiono T, Hayasaka S, Mizuno K. Presence of ornithine ketoacid aminotransferase in human ocular tissues. Graefes Arch Clin Exp Ophthalmol. 1982;218(1):34–36. [PubMed]
  • Hayasaka S, Matsuzawa T, Shiono T, Mizuno K, Ishiguro I. Enzymes metabolizing ornithine-proline pathway in the bovine eye. Exp Eye Res. 1982 Apr;34(4):635–638. [PubMed]
  • Smith RJ, Phang JM. The importance of ornithine as a precursor for proline in mammalian cells. J Cell Physiol. 1979 Mar;98(3):475–481. [PubMed]
  • Hayasaka S, Mizuno K, Yabata K, Saito T, Tada K. Atypical gyrate atrophy of the choroid and retina associated with iminoglycinuria. Arch Ophthalmol. 1982 Mar;100(3):423–425. [PubMed]
  • Shih VE, Efron ML, Moser HW. Hyperornithinemia, hyperammonemia, and homocitrullinuria. A new disorder of amino acid metabolism associated with myoclonic seizures and mental retardation. Am J Dis Child. 1969 Jan;117(1):83–92. [PubMed]
  • Rinaldi E, Stoppoloni GP, Savastano S, Russo S, Cotticelli L. Gyrate atrophy of choroid associated with hyperornithinaemia: report of the first case in Italy. J Pediatr Ophthalmol Strabismus. 1979 Mar–Apr;16(2):133–135. [PubMed]
  • Hodes DT, Oberholzer VG, Mushin AS, Briddon A, Laurance BM. Hyperornithinaemia with gyrate atrophy of the choroid and retina in two siblings. J R Soc Med. 1980 Aug;73(8):588–591. [PMC free article] [PubMed]
  • Atwood KC, Yu MT, Eicher E, Henderson AS. Feasibility tests for mapping low-multiplicity genes by hybridization in situ. Birth Defects Orig Artic Ser. 1976;12(7):372–375. [PubMed]
  • Giordano C, De Santo NG, Pluvio M, Santinelli R, Stoppoloni G. Lysine in treatment of hyperornithinemia. Nephron. 1978;22(1-3):97–106. [PubMed]
  • Takki KK, Milton RC. The natural history of gyrate atrophy of the choroid and retina. Ophthalmology. 1981 Apr;88(4):292–301. [PubMed]