Abstract
Two patients (P1: 1 month, P2: 2 years old) with polar cataract of one eye were observed. P2 underwent cataract surgery to prevent amblyopia. The neurological development corresponded to age.
The enzymatic activities of galactokinase, transferase, epimerase and sorbitdehydrogenase were found to be normal in erythrocytes of P1 and P2, such as cultivated fibroblasts of P1. In both patients normal concentrations of galactose (serum) and galactose-1-phosphate (erythrocytes) were found. During a galactose loading test (1 g/kg b. w.) the increase of galactose and galactose-1-phosphate concentrations was within the normal range. Serum galactitol levels were increased (P1 = 2,65, P2 = 1,08 μmol; normal: 0,09-0,52 μmol) whereas serum sorbitol levels were normal (<3,08 μmol). One and five months after introduction of a galactose-free diet normal serum galactitol levels were found, remaining on the same level now up to one year after termination of the diet by the families (0,29 and 0,3 μmol, respectively). Accumulation of galactitol in the lens possibly causes cataract. The mechanism of the serum galactitol elevation of our patients remains unclear. A transient increase of aldosereductase activity may be discussed.
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Gerding, H., Ullrich, K., Shin, Y. et al. INFANTILE CATARACT WITH TRANSIENT INCREASE OF SERUM GALACTITOL LEVELS. Pediatr Res 26, 515 (1989). https://doi.org/10.1203/00006450-198911000-00097
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DOI: https://doi.org/10.1203/00006450-198911000-00097