Observations on the composition of milk-substitute products for treatment of inborn errors of amino acid metabolism. Comparisons with human milk. A proposal to rationalize nutrient content of treatment products.
We tabulated and compared the stated compositions of four milk-substitute products, now in wide use for the treatment of various inborn errors of amino acid metabolism, and the known composition of human milk. Variations between the treatment products is great not only in the content of amino acids but also in minerals and vitamins, for example. Different source materials and rationales for their manufacture appear to explain these differences. All four products deviate in many ways from the composition of human milk. Although the existing treatment products are quite effective clinically, we propose that a more rational approach to the feeding of young infants whose nutrition is compromised by inborn errors of metabolism would begin with a synthetic product based on the composition of human milk that could be modified specifically to fit the needs and tolerance of the individual patient.
D000592:Amino Acid Metabolism, Inborn Errors / Q000178:diet therapy*; D000596:Amino Acids / Q000032:analysis*; D004041:Dietary Fats / Q000032:analysis; D004044:Dietary Proteins / Q000032:analysis; D005227:Fatty Acids / Q000032:analysis; D005526:Food, Formulated / Q000032:analysis* / Q000592:standards; D006801:Humans; D007223:Infant; D008895:Milk, Human / Q000032:analysis*; D008903:Minerals / Q000032:analysis; D014815:Vitamins / Q000032:analysis
D000596:Amino Acids; D004041:Dietary Fats; D004044:Dietary Proteins; D005227:Fatty Acids; D008903:Minerals; D014815:Vitamins