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

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

Mol Genet Metab

Division of Genetics and Genomics, Metabolism Program, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. Electronic address:

Published: July 2014

AI Article Synopsis

Article 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.

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Source
http://dx.doi.org/10.1016/j.ymgme.2014.04.004DOI Listing

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