Liquid nutritional formula for tyrosinemia patients

1. A shelf-stable, liquid nutritional formula for dietary management of tyrosinemia comprising:

(a) a protein source that includes:

(i) caseino-glyco-macropeptide (cGMP); and

(ii) complementary amino acids which are a mixture of arginine, tryptophan, leucine, histidine and cysteine, wherein the cGMP is between about 3% and about 6% of the total weight of the formula, and wherein the protein source provides 20-35% of the energy of the nutritional formula;

(b) a fat source which provides 20% to 30% of the energy of the nutritional formula, wherein the fat source is about 1.5% to about 2.2% of the total weight of the nutritional formula;

(c) a carbohydrate source which provides 40% to 60% of the energy of the nutritional formula,

(d) octenyl succinate starch (OSS); and,

(e) a sodium hexametaphosphate-metal (SHMP-metal) mixture comprising SHMP bound to copper and iron,

wherein the nutritional formula contains less than 0.01% by weight of tyrosine.

2. The shelf-stable, liquid nutritional formula of claim 1 , wherein together the complementary amino acids are about 1.5% to about 2.35% of the total weight of the formula.

3. The shelf-stable, liquid nutritional formula of claim 1 , wherein the formula comprises no added tyrosine or phenylalanine.

4. The shelf-stable, liquid nutritional formula of claim 1 , further comprising at least one vitamin and at least one mineral, wherein together the at least one vitamin and the at least one mineral are between about 1.5% and about 2.35% of the total weight of the formula.

5. The shelf-stable, liquid nutritional formula of claim 4 , wherein the at least one mineral is dicalcium phosphate, calcium lactate, dipotassium phosphate, magnesium citrate, sodium ascorbate, zinc sulfate, calcium d-pantothenate, manganese sulfate, potassium iodide, sodium selenite, sodium molybdate, or chromium chloride, and wherein the at least one vitamin is choline bitartrate, ascorbic acid, niacinamide, vitamin E dl-alphatocopheryl acetate, vitamin A palmitate, vitamin B6 pyridoxine, riboflavin, thiamin hydrochloride, folic acid, vitamin K 1 phytonadione, biotin, vitamin D3 cholecalciferol, or vitamin B12 cyanocobalamin.

6. A shelf-stable, liquid nutritional formula for dietary management of tyrosinemia comprising:

(a) a protein source that includes:

(i) caseino-glyco-macropeptide (cGMP); and

(ii) complementary amino acids which are a mixture of arginine, tryptophan, leucine, and histidine;

(b) a fat source, wherein the fat source provides between about 18% to about 27% of the total calories in the formula,

(c) octenyl succinate starch (OSS); and,

(d) a sodium hexametaphosphate-metal (SHMP-metal) mixture comprising SHMP bound to copper and iron,

wherein the protein source provides between about 28% and about 43% of the total calories in the formula, and the formula contains less than 0.01% by weight of tyrosine.

7. The shelf-stable, liquid nutritional formula of claim 6 , wherein the formula comprises no added tyrosine or phenylalanine.

8. The shelf-stable, liquid nutritional formula of claim 6 , further comprising vitamins and additional minerals, wherein the vitamins and additional minerals are present in an amount sufficient to make the formula nutritionally complete.

9. The shelf-stable, liquid nutritional formula of claim 8 , wherein the additional minerals are dicalcium phosphate, calcium lactate, dipotassium phosphate, magnesium citrate, sodium ascorbate, zinc sulfate, calcium d-pantothenate, manganese sulfate, potassium iodide, sodium selenite, sodium molybdate, and chromium chloride, and wherein the vitamins are choline bitartrate, ascorbic acid, niacinamide, vitamin E dl-alphatocopheryl acetate, vitamin A palmitate, vitamin B6 pyridoxine, riboflavin, thiamin hydrochloride, folic acid, vitamin K 1 phytonadione, biotin, vitamin D3 cholecalciferol, and vitamin B12 cyanocobalamin.

10. The shelf-stable, liquid nutritional formula of claim 3 , wherein the formula comprises between about 2.4 mg and about 3.6 mg tyrosine.

11. The shelf stable, liquid nutritional formula of claim 7 , wherein the formula comprises between about 2.4 mg and about 3.6 mg tyrosine.