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.
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References
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) 45–48
Kador, P. F., Zigler, J. S. and Kinoshita, J. H. Alteration of lens protein synthesis in galactosemic rats. Invest. Ophthalmol. Visual Sci. 18 (1979) 696–702
Kador, P. F. and Kinoshita, J. Diabetic and galactosaemic cataracts. In Ciba Foundation Symposium 106, Human Cataract Formation. Pittman, London, 1984, pp. 110–131
Ng, M. C., Tsui, J. Y., Merola, L. O. and Unakar, N. J. Effect of prolactin on galactose cataractogenesis. Ophthalm. Res. 19 (1987) 82–94
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) 355–367
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) 83–89
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) 137–142
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© 1989 SSIEM and Kluwer Academic Publishers
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Brivet, M., Migayron, F., Roger, J., Cheron, G., Lemonnier, A. (1989). Lens Hexitols and Cataract Formation During Lactation in a Woman Heterozygote for Galactosaemia. In: Addison, G.M., Connor, J.M., Harkness, R.A., Pollitt, R.J. (eds) Studies in Inherited Metabolic Disease. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-1069-0_46
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DOI: https://doi.org/10.1007/978-94-009-1069-0_46
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