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

doi:10.1016/j.ymgme.2014.04.004

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

Mol Genet Metab. 2014 Jul;112(3):191-7. doi: 10.1016/j.ymgme.2014.04.004. Epub 2014 May 2.

Authors

Sandra C Van Calcar¹, Laurie E Bernstein², Frances J Rohr³, Christine H Scaman⁴, Steven Yannicelli⁵, Gerard T Berry⁶

Affiliations

¹ .Division of Genetics and Metabolism, Department of Pediatrics, School of Medicine and Public Health, Waisman Center, University of Wisconsin-Madison, Madison, Wisconsin, USA. Electronic address: vancalcar@pediatrics.wisc.edu.
² Section of Clinical Genetics and Metabolism, Inherited Metabolic Disease Nutrition Department, University of Colorado-Denver School of Medicine, The Children's Hospital Colorado, Aurora, Colorado, USA.
³ Division of Genetics and Genomics, Metabolism Program, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
⁴ Food Nutrition and Health, University of British Columbia, Vancouver, British Columbia, Canada.
⁵ Medical and Scientific Affairs, Nutricia North America, Gaithersburg, Maryland, USA.
⁶ Division of Genetics and Genomics, Metabolism Program, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. Electronic address: Gerard.Berry@childrens.harvard.edu.

PMID: 24857409
DOI: 10.1016/j.ymgme.2014.04.004

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.

Keywords: Diet treatment; Galactose restriction; Galactose-1-phosphate uridyltransferase deficiency; Inborn errors of metabolism; Lactose.

Publication types

Meta-Analysis
Research Support, Non-U.S. Gov't
Review

MeSH terms

Age Factors
Diet, Carbohydrate-Restricted*
Food
Galactose*
Galactosemias / diet therapy*
Humans

Substances

Galactose