Medical foods for the nutritional support of child/adult metabolic diseases

A composition of matter which is in powdered form and may be reconstituted and orally administered for use in the nutritional support of individuals having inherited metabolic diseases, said composition comprising a mixture of (a) a premix base in physical admixture with (b) a blend of synthetic alpha amino acids, wherein said premix base consists essentially of fats, carbohydrates, minerals, vitamins, trace elements, taurine and antioxidants, and wherein said fat content comprises 25 to 35% by dry weight of said premix base, said carbohydrate content comprises 55 to 70% by dry weight of said premix base, said vitamin content comprises 0.1 to 1.0% by dry weight of said premix base, said taurine content comprises 0.05 to 1.0% by dry weight of said premix base, and said mineral content comprises 5 to 15% by dry weight of said premix base, said premix base comprising not less than about 55% and not more than about 90% by dry weight of said composition, said fat content consisting of about 12% to about 25% by dry weight of said composition, said alpha amino acid blend consists of about 10% to about 45% by dry weight of said composition, and wherein said fat is a blend of at least two fat sources selected from the group consisting of pork lard, beef tallow, herring, menhaden, pilchard, sardine, babassu, caster, coconut, corn, cottonseed, jojoba, linseed, oiticica, olive, palm, palm kernel, peanut, rice bran, rapeseed, safflower, sesame, soy, sunflower, tall and tung oils, and wherein the sole source of amino acids in said composition is said amino acid blend which comprises up to eighteen individual synthetic amino acids selected from the group consisting of L-alanine, L-arginine, L-aspartic acid, L-cystine, L-glutamic acid, L-glutamine, glycine, L-histidine, L-isoleucine, L-leucine, L-lysine, L-methionine, L-proline, L-phenylalanine, -L-serine, L-thronine, L-typtophan, L-tyropsine and L-valine.