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Pathology
An infant appears normal at birth following an uncomplicated pregnancy and begins to nurse. Within 3 days the infant exhibits vomiting and diarrhea. A week later jaundice is evident, and the infant becomes febrile. On physical examination hepatomegaly is present. Laboratory studies show abnormal reducing substances in the urine, and Escherichia coli is cultured from blood. This infant most likely has a deficiency in which of the following enzymes?
Galactose-1-phosphate uridyltransferase
Glucocerebrosidase
Phenylalanine hydroxylase
Sphingomyelinase
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