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

Sandra C. Van Calcar, Laurie E. Bernstein, Frances J. Rohr, Christine H. Scaman, Steven Yannicelli, Gerard T. Berry

Research output: Contribution to journalReview articlepeer-review

31 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)191-197
Number of pages7
JournalMolecular Genetics and Metabolism
Volume112
Issue number3
DOIs
StatePublished - Jul 2014
Externally publishedYes

Keywords

  • Diet treatment
  • Galactose restriction
  • Galactose-1-phosphate uridyltransferase deficiency
  • Inborn errors of metabolism
  • Lactose

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Molecular Biology
  • Genetics
  • Endocrinology

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