TY - JOUR
T1 - A re-evaluation of life-long severe galactose restriction for the nutrition management of classic galactosemia
AU - Van Calcar, Sandra C.
AU - Bernstein, Laurie E.
AU - Rohr, Frances J.
AU - Scaman, Christine H.
AU - Yannicelli, Steven
AU - Berry, Gerard T.
N1 - Funding Information:
Funding for this project was provided by the Galactosemia Foundation (formerly Parents of Galactosemic Children, USA). We thank Denise Wilburn for serving as the consumer representative on the task force.
PY - 2014/7
Y1 - 2014/7
N2 - 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.
AB - 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.
KW - Diet treatment
KW - Galactose restriction
KW - Galactose-1-phosphate uridyltransferase deficiency
KW - Inborn errors of metabolism
KW - Lactose
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U2 - 10.1016/j.ymgme.2014.04.004
DO - 10.1016/j.ymgme.2014.04.004
M3 - Review article
C2 - 24857409
AN - SCOPUS:84902540273
SN - 1096-7192
VL - 112
SP - 191
EP - 197
JO - Molecular Genetics and Metabolism
JF - Molecular Genetics and Metabolism
IS - 3
ER -