Which metabolic abnormality is associated with E. coli sepsis?

• A. Biotinidase deficiency
• B. Glycogen storage disease
• C. Phenylketonuria
• D. Galactosemia

Answer: (D)

Neonatal sepsis with E. coli is a classic clue for galactosemia. In classic galactosemia, deficiency of the enzyme that metabolizes galactose from milk prevents proper processing of galactose-1-phosphate. This leads to toxic metabolite buildup in the liver and other tissues, causing jaundice, hepatomegaly, hypoglycemia, and immune distrubances that make newborns particularly vulnerable to severe infections, including E. coli sepsis. Because of this strong link, recognizing galactosemia early is crucial, and management involves stopping galactose-containing feeds (lactose-free, galactose-free formula) and initiating appropriate supportive care. The other metabolic disorders listed do not have the same well-established association with newborn E. coli sepsis.