Lens Hexitols and Cataract Formation During Lactation in a Woman Heterozygote for Galactosaemia

  • M. Brivet
  • F. Migayron
  • J. Roger
  • G. Cheron
  • A. Lemonnier

Abstract

The occurrence of cataracts in a healthy lactating woman, heterozygous for UDP glucose-hexose-l-phosphate uridyltransferase (GALT; EC 2.7.7.12) deficiency (McKusick 23040), may suggest a possible link to the enzymatic defect of galactose metabolism. As studies in rats (Ng et al., 1987) showed that prolactin accelerates galactose-induced cataractogenesis, i.e. aldose reductase mediated accumulation of galactitol in the lenses, our purpose was to determine the hexitol contents of the patient’s lens tissue.

Keywords

Cataract Formation Lens Protein Cataractous Lens Galactose Metabolism GALT Activity 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Avisar, R. A., Schwartzman, S., Levinsky, H., Allalouf, D., Goldman, J., Ninio, A. and Savir, H. A case of cataract formation during the lactating period associated with galactose-1-phosphate uridyltransferase deficiency. Metab. Pediatr. Syst. Ophthalmol. 6 (1982) 45–48PubMedGoogle Scholar
  2. Kador, P. F., Zigler, J. S. and Kinoshita, J. H. Alteration of lens protein synthesis in galactosemic rats. Invest. Ophthalmol. Visual Sci. 18 (1979) 696–702Google Scholar
  3. Kador, P. F. and Kinoshita, J. Diabetic and galactosaemic cataracts. In Ciba Foundation Symposium 106, Human Cataract Formation. Pittman, London, 1984, pp. 110–131Google Scholar
  4. Ng, M. C., Tsui, J. Y., Merola, L. O. and Unakar, N. J. Effect of prolactin on galactose cataractogenesis. Ophthalm. Res. 19 (1987) 82–94CrossRefGoogle Scholar
  5. Ocken, P. R., Fu, S-C J., Hart, R., White, J. H., Wagner, B. J. and Lewis, K. E. Characterization of lens proteins. I. Identification of additional soluble fractions in rat lenses. Exp. Eye Res. 24 (1977) 355–367PubMedCrossRefGoogle Scholar
  6. Popp-Snijders, C., Lomecky, M. Z. and de Jong, A. P. Determination of sorbitol in erythrocytes of diabetic and healthy subjects by capillary gas chromatography. Clin. Chim. Acta 132 (1983) 83–89PubMedCrossRefGoogle Scholar
  7. Tomana, M., Prchal, J. T., Garner, L. C., Skalka, H. W. and Barker, S. A. Gas chromatographic analysis of lens monosaccharides. J. Lab. Clin. Med. 103 (1984) 137–142PubMedGoogle Scholar

Copyright information

© SSIEM and Kluwer Academic Publishers 1989

Authors and Affiliations

  • M. Brivet
    • 1
  • F. Migayron
    • 1
  • J. Roger
    • 2
  • G. Cheron
    • 3
  • A. Lemonnier
    • 1
  1. 1.Laboratoire de BiochimieHôpital de BicêtreLe Kremlin-Bicêtre CedexFrance
  2. 2.Service de ContactologieFondation RothschildParis Cedex 19France
  3. 3.Départment de PédiatrieHôpital Necker Enfants MaladesParis Cedex 15France

Personalised recommendations