title: A re-evaluation of life-long severe galactose restriction for the nutrition management of classic galactosemia.
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.
authors.affiliation: .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.
authors.forename: Sandra C
authors.initials: SC
authors.lastname: Van Calcar
authors.affiliation: 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.
authors.forename: Laurie E
authors.initials: LE
authors.lastname: Bernstein
authors.affiliation: Division of Genetics and Genomics, Metabolism Program, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Frances J
authors.initials: FJ
authors.lastname: Rohr
authors.affiliation: Food Nutrition and Health, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Christine H
authors.initials: CH
authors.lastname: Scaman
authors.affiliation: Medical and Scientific Affairs, Nutricia North America, Gaithersburg, Maryland, USA.
authors.forename: Steven
authors.initials: S
authors.lastname: Yannicelli
authors.affiliation: Division of Genetics and Genomics, Metabolism Program, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. Electronic address: Gerard.Berry@childrens.harvard.edu.
authors.forename: Gerard T
authors.initials: GT
authors.lastname: Berry
mesh_terms: D000367:Age Factors; D050528:Diet, Carbohydrate-Restricted; D005502:Food; D005690:Galactose; D005693:Galactosemias / Q000178:diet therapy*; D006801:Humans
chemical_list: D005690:Galactose
keywords: Diet treatment; Galactose restriction; Galactose-1-phosphate uridyltransferase deficiency; Inborn errors of metabolism; Lactose