Nutrition Management of Galactosemia

Classical galactosemia can result in life-threatening complications including failure to thrive, hepatocellular damage, and E. coli sepsis in untreated infants. Initiation of a soy-based infant formula or other lactose-free medical food within the first few days of life can mitigate these complications. Patients with classical galactosemia are at increased risk for developmental delay, speech problems and neurological complications, even with life-long dietary treatment. Dairy products are the primary source of dietary galactose. Galactose is produced endogenously and accounts for a greater contribution to the total galactose pool than the small amounts of galactose found in plant-based foods.