SUSCEPTIBILITY TO E. COLI INFECTION IN NEONATES WITH GALACTOSEMIA

Among the 10 infants identified with galactosemia by routine newborn screening in Massachusetts, four have had severe infection due to E. coli. Three of these neonates died with bacteremia and meningitis while the fourth had a urinary tract infection and survived with treatment. In each of the three infants who died galactosemia was not identified until after the first week of life whereas galactosemia was detected and treated before the sixth day of life in the other seven infants. Immunologic studies were performed on three of the surviving children. Immunoglobulins were quantitatively normal. In vitro bactericidal activity against E. coli by leucocytes from these children and controls was not significantly inhibited by incubation with galactose in concentrations of 100 - 500 mg/dl. It appears that galactosemic neonates are unusually predisposed to sepsis, a predisposition perhaps limited to E. coli, and that this tends to become manifest if the onset of dietary therapy is delayed beyond the first week of life. It also appears that this susceptibility may be most striking in “classical” galactosemia in which the biochemical abnormalities are most severe.