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In the rare infant with congenital lactase deficiency, there may be signs of dehydration and failure to thrive (Altschuler & Liacouras, 1998).
In those rare infants with congenital lactase deficiency, feedings of lactose-free or soy-based formulas are the treatments of choice.
Congenital lactase deficiency, an autosomal recessive condition, is rare and presents immediately after lactose feeding with vomiting, watery stool, dehydration, irritability, acidosis, and lactosuria.

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