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Subject:
From:
"Linda L. Shaw MD" <[log in to unmask]>
Reply To:
Lactation Information and Discussion <[log in to unmask]>
Date:
Tue, 21 Jul 1998 09:21:32 EDT
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Most infants are screened for galactosemia by their state screening programs,
so I think that missing a galactosemic by using Lactofree is probably not a
major issue.

However, I don't see much of a use for this product.  Human infants are NOT
lactase deficient.  Lactose is, of course, the major sugar in human milk.
Humans who are lactase-deficient usually develop this deficiency after the age
of weaning and are then unable to process cow milk products when they are
older children and adults.  I don't know whether Lactofree has been studied
for use in galactosemia--traditionally, these infants have been fed one of the
soy formulas which don't contain galactose.

Galactosemia is an inborn error of metabolism in which the infant lacts the
enzyme (Galactokinase) necessary to process galactose (incidence about
1/40,000); because of this, toxic substances will accumulate in the baby and
cause cataracts and other problems.  The treatment is a galactose-free diet.
There are 2 other galactosemia enzyme defects; Galactose-1-Phosphate uridyl
transferase deficiency and uridyl diphosphogalactose-4-epimerase deficiency.
G1PUT deficiency (incidence about 1/50,000) can cause jaundice, hepatomegaly,
vomiting, hypoglycemia, convulsions, lethargy, irritability, feeding
difficulties, poor weight gain, aminoaciduria, cataracts, hepatic cirrhosis,
and mental retardation if not diagnosed in infancy and treated with a
galactose-free diet.  UDG4E deficiency is clinically identical to G1PUT and is
very rare.

Hope this helps.

Linda L. Shaw MD FAAP

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