A re-evaluation of life-long severe galactose restriction for the nutrition management of classic galactosemia

• Published in: Molecular genetics and metabolism 2014-07, Vol.112 (3), p.191-197
• Main Authors: Van Calcar, Sandra C., Bernstein, Laurie E., Rohr, Frances J., Scaman, Christine H., Yannicelli, Steven, Berry, Gerard T.
• Format: Article
• Language: English
• Subjects: Age Factors; Diet treatment; Diet, Carbohydrate-Restricted; Food; Galactose; Galactose restriction; Galactose-1-phosphate uridyltransferase deficiency; Galactosemias - diet therapy; Humans; Inborn errors of metabolism; Lactose
• ISSN: 1096-7192; 1096-7206
• DOI: 10.1016/j.ymgme.2014.04.004

The galactose-restricted diet is life-saving for infants with classic galactosemia. However, the benefit and extent of dietary galactose restriction required after infancy remain unclear and variation exists in practice. There is a need for evidence-based recommendations to better standardize treatment for this disorder. This paper reviews the association between diet treatment and outcomes in classic galactosemia and evaluates the contribution of food sources of free galactose in the diet. Recommendations include allowing all fruits, vegetables, legumes, soy products that are not fermented, various aged cheeses and foods containing caseinates. Further research directions are discussed. •Variation exists in recommendations for nutrition management of classic galactosemia.•Review of current diet recommendations for treatment of this disorder is needed.•All fruits, vegetables, legumes, soy products that are not fermented, various aged cheeses and foods containing caseinates can be allowed in the diet.•Further investigation is needed to determine the extent of dietary restriction required after infancy.