Pregnancy-exaggerated galactosemia and congenital cataracts

Abstract

One child in a family and two children in another family had galactosemia and congenital cataract. Two of them had total soft cataracts while in one, cataract was less soft. In addition, they had mild lactosuria. The mothers of the affected children had significant lactosuria and mild galactosuria without cataracts. Fathers did not have galactosuria or lactosuria. Clinically unaffected siblings in one family had mild galactosuria and lactosuria. Pregnancy-exaggerated galactosemia was suspected in these two mothers who gave birth to children with congenital cataract. As an extension of this work, 5001 pregnant women were screened for galactose in urine just before the delivery of babies. Mild galactosuria was present in 54 (1.08%). Three children had congenital cataract and one had changes in posterior pole and cornea. Restriction of lactose by reducing intake of milk and milk products during pregnancy by mothers with galactosuria is recommended to avoid the birth of children with congenital cataract.

Naturally occurring inhibitors against the formation of advanced glycation end-products

“…128 A study also observed that inositol displayed a potent antiglycative property (57-67% decrease of glycation of human eye lens proteins with glucose) due to the scavenging of glucose. 129 In addition, alterations of rat tail tendon collagen induced by glycation can be prevented by arginine and arginine-lysine. 130 In the presence of amino acids arginine and lysine, glucose incorporation into rat tail tendon fibers as well as Amadori product formation decreased significantly, which was mainly due to the competitive attachment of these two amino acids to glucose.…”