Abstract
SCHWARTZ, HOLZEL and KOMROWER have demonstrated high levels of galactose-1-phosphate in tissues of newborns deficient in galactose-1-phosphate uridyl transferase activity. Following their recommendations to maintain a known heterozygotic pregnant mother on a galactose free diet, we examined the cord blood of a galactosemic infant. The levels of galactose-1-phosphate were found to be approximately four times normal. At age of ten days, the infant's red cell content of galactose-1-phosphate had increased to seven times normal, even though he was on a galactose free diet. Over the ensuing five weeks, the levels slightly decreased. Intravenous administration of galactose-1-phosphate uridyl transferase in 35 ml of packed normal red cells resulted in prompt disappearance of galactose-1-phosphate and decrease in liver size. Studies of maternal galactose levels in another pregnant woman as well as evaluation of maternal and newborn lactation are in process. (SPR)
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Harris, R., Isbell, S. 78 Galactose-1-Phosphate Accumulation in Red Cells of a Neonate. Pediatr Res 1, 220 (1967). https://doi.org/10.1203/00006450-196705000-00085
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DOI: https://doi.org/10.1203/00006450-196705000-00085