| Literature DB >> 24857409 |
Sandra C Van Calcar1, Laurie E Bernstein2, Frances J Rohr3, Christine H Scaman4, Steven Yannicelli5, Gerard T Berry6.
Abstract
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.Entities:
Keywords: Diet treatment; Galactose restriction; Galactose-1-phosphate uridyltransferase deficiency; Inborn errors of metabolism; Lactose
Mesh：
Substances：
Year: 2014 PMID： 24857409 DOI： 10.1016/j.ymgme.2014.04.004
Source DB: PubMed Journal: Mol Genet Metab ISSN： 1096-7192 Impact factor: 4.797