Van Calcar SC. A re-evaluation of life-long severe galactose restriction for the nutrition management of classic galactosemia. Mol Genet Metab. 2014 Jul;112(3):191-197.
Although the need for galactose restriction in infants with classic galactosemia is unquestioned, the value of very severe galactose restriction for older individuals is controversial. Van Calcar et al review current practices and make recommendations based on the available literature. They find that there is considerable heterogeneity in treatment around the world, particularly with respect to allowing minor sources of galactose from dairy (e.g. cheeses) and plant sources. The authors suggest the inclusion in the diet of fruits, vegetables, juices, legumes, and all non-fermented soy based products, as well as specific hard cheeses and the dairy-based additives, calcium and sodium caseinate. They also stress the importance of ensuring adequate calcium and vitamin D intake.
The risks and benefits of liberalising the diet for older/adult patients with galactosemia, as well as the pathophysiology and prevention of long-term complications, remain important and incompletely understood issues that require further study.
Posted by Alina Levtova, MD