Diet principles - GAL

The diet for classical galactosemia patients must be individualized depending on each patient’s nutrient needs. Despite the long experience with classical Galactosemia management, major controversies remain on the optimal strictness and duration of dietary galactose restriction¹.

When patients grow from infancy into childhood, recommendations on dietary restriction of galactose vary widely². In some countries the restriction of galactose extends beyond dairy products, including many fruits, legumes and vegetables, where others recommend more liberal guidelines. This liberal approach is bases on the nutritional benefit of fruits and vegetables, which only create a small increase in daily galactose intake (30-45 mg galactose per day), which is minor relative to the estimated endogenous production of galactose in adult patients (1000 mg per day)³.

Diet principles^4,5

Limit the intake of galactose; of which the main source in the human diet is lactose in milk, milk-products and manufactured foods containing milk
- In some countries only a lactose free diet is recommended
- In some countries intake of galactose containing products, such as fruits, legumes and vegetables is restricted
Adequate energy intake to promote appropriate growth and weight maintenance
Monitor nutritional status and adjust diet as needed
Provision of a palatable, varied and ‘easy to maintain’ diet.
Ensure intake of nutrients connected to bone development and maintenance, such as calcium, vitamin D_3,vitamin K₁in line with recommended Dietary Reference Intakes (DRI)

References

Bosch AM, Bakker HD et al. High tolerance for oral galactose in classical galactosemia: dietary implications. Arch Dis Child. 2004;89:1034-1036
Bosch AM. Classical galactosemia revisted. J Inherit Metab Dis. 2011;34:257-260.
Huidekoper H, Bosch A, et al Short-term exogenous galactose supplementation does not influence rate of appearance of galactose in patients with classical galactosemia. Mol Genet Metab. 2005; 84:265–272.
Dixon M, Macdonald A et al. Disorders of carbohydrate metabolism, In; Shaw V (ed) Clinical paediatric dietetics, 4^th John Wiley & Sons ltd. Sussex, 2015:526-539
Bernstein LE, Calcar A. The diet for Galactosemia. In: Bernstein (ed)Nutrition Management of Inherited Metabolic Diseases 1^stSpringer International Publishing Switzerland 2015:285-293.