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. 1982 Dec;66(12):767–770. doi: 10.1136/bjo.66.12.767

Combined vitamin A and E therapy prevents retinal electrophysiological deterioration in abetalipoproteinaemia.

S Bishara, S Merin, M Cooper, E Azizi, G Delpre, R J Deckelbaum
PMCID: PMC1039925  PMID: 7171526

Abstract

Eight patients with abetalipoproteinaemia had the typical ocular, systemic, and laboratory findings of this disease. Combined therapy with vitamins A and E was administered, starting as early as the first day of life and as late as 26 years of age. The patients were followed up for 2-6 years. Electroretinography was undertaken in all cases and electrooculography in some. After initiation of vitamin A and E therapy no progression of disturbed visual function could be detected in any patient. These objective tests of retinal function demonstrated that the combined vitamin A and E therapy may be useful in arresting retinal deterioration in abetalipoproteinaemia.

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Selected References

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

  1. ARDEN G. B., KELSEY J. H. Changes produced by light in the standing potential of the human eye. J Physiol. 1962 May;161:189–204. doi: 10.1113/jphysiol.1962.sp006881. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Azizi E., Zaidman J. L., Eshchar J., Szeinberg A. Abetalipoproteinemia treated with parenteral and oral vitamins A and E, and with medium chain triglycerides. Acta Paediatr Scand. 1978 Nov;67(6):796–801. doi: 10.1111/j.1651-2227.1978.tb16264.x. [DOI] [PubMed] [Google Scholar]
  3. BASSEN F. A., KORNZWEIG A. L. Malformation of the erythrocytes in a case of atypical retinitis pigmentosa. Blood. 1950 Apr;5(4):381–387. [PubMed] [Google Scholar]
  4. Bjornson L. K., Kayden H. J., Miller E., Moshell A. N. The transport of alpha-tocopherol and beta-carotene in human blood. J Lipid Res. 1976 Jul;17(4):343–352. [PubMed] [Google Scholar]
  5. Campbell D. A., Tonks E. L. BIOCHEMICAL FINDINGS IN HUMAN RETINITIS PIGMENTOSA WITH PARTICULAR RELATION TO VITAMIN A DEFICIENCY. Br J Ophthalmol. 1962 Mar;46(3):151–164. doi: 10.1136/bjo.46.3.151. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Carr R. E. Abetalipoproteinemia and the eye. Birth Defects Orig Artic Ser. 1976;12(3):385–408. [PubMed] [Google Scholar]
  7. Cottrill C., Glueck C. J., Leuba V., Millett F., Puppione D., Brown W. V. Familial homozygous hypobetalipoproteinemia. Metabolism. 1974 Aug;23(8):779–791. doi: 10.1016/0026-0495(74)90010-9. [DOI] [PubMed] [Google Scholar]
  8. DRUEZ G., LAMY M., FREZAL J., POLONOVSKI J., REY J. [Acanthocytosis. Its relations to the congenital absence of beta-lipoproteins]. Presse Med. 1961 Jul 15;69:1546–1548. [PubMed] [Google Scholar]
  9. Goodman D. S. Vitamin A metabolism. Fed Proc. 1980 Aug;39(10):2716–2722. [PubMed] [Google Scholar]
  10. Gotto A. M., Levy R. I., John K., Fredrickson D. S. On the protein defect in abetalipoproteinemia. N Engl J Med. 1971 Apr 15;284(15):813–818. doi: 10.1056/NEJM197104152841503. [DOI] [PubMed] [Google Scholar]
  11. Gouras P., Carr R. E., Gunkel R. D. Retinitis pigmentosa in abetalipoproteinemia: Effects of vitamin A. Invest Ophthalmol. 1971 Oct;10(10):784–793. [PubMed] [Google Scholar]
  12. Hayes K. C. Retinal degeneration in monkeys induced by deficiencies of vitamin E or A. Invest Ophthalmol. 1974 Jul;13(7):499–510. [PubMed] [Google Scholar]
  13. Katz M. L., Stone W. L., Dratz E. A. Fluorescent pigment accumulation in retinal pigment epithelium of antioxidant-deficient rats. Invest Ophthalmol Vis Sci. 1978 Nov;17(11):1049–1058. [PubMed] [Google Scholar]
  14. Kayden H. J. Abetalipoproteinemia. Annu Rev Med. 1972;23:285–296. doi: 10.1146/annurev.me.23.020172.001441. [DOI] [PubMed] [Google Scholar]
  15. Merin S., Auerbach E. Retinitis pigmentosa. Surv Ophthalmol. 1976 Mar-Apr;20(5):303–346. doi: 10.1016/s0039-6257(96)90001-6. [DOI] [PubMed] [Google Scholar]
  16. Muller D. P., Lloyd J. K., Bird A. C. Long-term management of abetalipoproteinaemia. Possible role for vitamin E. Arch Dis Child. 1977 Mar;52(3):209–214. doi: 10.1136/adc.52.3.209. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Muller D. R., Harries J. T., Lloyd J. K. Vitamin E therapy in abetalipoproteinaemia. Arch Dis Child. 1970 Oct;45(243):715–715. doi: 10.1136/adc.45.243.715-a. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Robison W. G., Jr, Kuwabara T., Bieri J. G. Deficiencies of vitamins E and A in the rat. Retinal damage and lipofuscin accumulation. Invest Ophthalmol Vis Sci. 1980 Sep;19(9):1030–1037. [PubMed] [Google Scholar]
  19. SALT H. B., WOLFF O. H., LLOYD J. K., FOSBROOKE A. S., CAMERON A. H., HUBBLE D. V. On having no beta-lipoprotein. A syndrome comprising a-beta-lipoproteinaemia, acanthocytosis, and steatorrhoea. Lancet. 1960 Aug 13;2(7146):325–329. doi: 10.1016/s0140-6736(60)91478-1. [DOI] [PubMed] [Google Scholar]
  20. Sperling M. A., Hiles D. A., Kennerdell J. S. Electroretinographic responses following vitamin A therapy in A-beta-lipoproteinemia. Am J Ophthalmol. 1972 Mar;73(3):342–351. doi: 10.1016/0002-9394(72)90063-3. [DOI] [PubMed] [Google Scholar]
  21. WOLFF O. H., LLOYD J. K., TONKS E. L. A-BETA-LIPOPROTEINAEMIA WITH SPECIAL REFERENCE TO THE VISUAL DEFECT. Exp Eye Res. 1964 Dec;3:439–442. doi: 10.1016/s0014-4835(64)80055-5. [DOI] [PubMed] [Google Scholar]

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