Is lactose-free cow's milk infant formula recommended in galactosemia?

• A. Yes
• B. No
• C. Only for infants who cannot tolerate soy
• D. Only with enzyme supplementation

Answer: (B)

Galactosemia requires complete avoidance of galactose in the diet because the body cannot properly metabolize it, leading to harmful accumulation. Lactose-free cow's milk is produced by breaking lactose into glucose and galactose using the enzyme lactase. That hydrolysis creates free galactose in the product, so even though lactose is removed, galactose may still be present and can be harmful for an infant with galactosemia. For this reason, lactose-free cow's milk infant formula is not recommended. The safer approach is a truly galactose-free formula, such as soy-based or other specialized galactose-free formulas, to meet energy and protein needs without introducing galactose. Enzyme supplementation cannot make galactose-containing foods safe for galactosemia, since the restriction is dietary rather than treatable by enzymes.