You are reviewing the many different formulas available for infant feeding with a group of pediatric residents.
Of the following, the metabolic disease for which feeding with a commercial soy infant formula is MOST appropriate is
A. Galactosemia
B. Hereditary tyrosinemia type 1
C. Maple syrup urine disease
D. Phenylketonuria
E. Propionic acidemia
Correct option is 'A'
Explanation:
The improvements in the development and manufacture of infant formulas now enable physicians to treat a wide variety of chronic illnesses with specialized formulas. For example, because galactosemia is caused by defective conversion of galactose to glucose, a formula in which galactose is absent (eg, conventional soy-based formula, which does not contain lactose) should be fed to affected infants. In addition, a number of specialized formulas that have modified amino acid compositions have been developed to treat specific metabolic illnesses. These include formulas for phenylketonuria, hereditary tyrosinemia, homocystinuria, maple syrup urine disease, urea cycle disorders, and organic acidemias. Each of these metabolic disorders requires a formula that has a different composition of amino acids.
Specialized formulas also are used to treat atopic disease. Infants who have cow milk protein allergy (allergic colitis or eczema) may be treated with either a casein hydrolysate formula or an amino acid-based formula. Although some infants who have cow milk protein allergy can tolerate a soy protein-based formula, soy formulas generally are not recommended for these infants because of the high incidence of cross-reactivity.
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