Literature review for - Flourish Paediatrics

Galactosaemia in Infants
Search results
The initial search of the databases included 18 references on galactosaemia in breastfed and
formula fed infants. Data were extracted from three narrative reviews but the evidence was
not strong enough to develop a body of evidence statement. The information gathered from
the three reviews have been summarised below.
Notes on galactosaemia in infants
In infants with galactosaemia, galactose must be excluded from the diet early in life to avoid
cirrhosis of the liver, mental retardation, cataracts and hypoglycaemia. Breastmilk contains
3.7 g of galactose per 100 ml therefore breastfeeding must be ceased (Thompson,
Arrowsmith et al. 2003; Leung and Sauve 2005).
Galactosaemia (galactose-1-phosphate uridyltransferase deficiency) is an indication for the
use of lactose free soy formulae. It should be noted that some soy protein formulae contain
raffinose and stachyose that are cleaved in the digestive tract under the action of bacterial
galactosidases, leading to the liberation of 1,4 galactose that may contribute to elevated
galactose-1-P values in erythrocytes of galactosaemic patients (Thompson, Arrowsmith et al.
2003; Agostoni, Axelsson et al. 2006).
The working group of Australasia Society of Inborn Errors of Metabolism (ASIEM)
handbook written in 1998 recommends the following foods be avoided when infant
commence on solid foods: milk and milk products (except for mature cheese), lactose (used
as an ingredient and carrier for flavour in foods e.g. in extruded cheese snacks, take away
foods, and some medications), chickpeas, beans, gram flour, fermented soy products. Other
foods which may contain some free galactose are recommended in small quantities only
(Thompson, Arrowsmith et al. 2003). The ASIEM Galactosaemia handbook is currently
being revised and a draft copy is currently available (Thompson and Netting 2010).
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