CA2894333A1 - Liquid nutritional formula for tyrosinemia patients - Google Patents
Liquid nutritional formula for tyrosinemia patients Download PDFInfo
- Publication number
- CA2894333A1 CA2894333A1 CA2894333A CA2894333A CA2894333A1 CA 2894333 A1 CA2894333 A1 CA 2894333A1 CA 2894333 A CA2894333 A CA 2894333A CA 2894333 A CA2894333 A CA 2894333A CA 2894333 A1 CA2894333 A1 CA 2894333A1
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- Prior art keywords
- vitamin
- formula
- nutritional formula
- amino acids
- beverage
- Prior art date
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- 201000011296 tyrosinemia Diseases 0.000 title claims abstract description 21
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- 230000036571 hydration Effects 0.000 claims abstract description 38
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- LXNHXLLTXMVWPM-UHFFFAOYSA-N pyridoxine Chemical compound CC1=NC=C(CO)C(CO)=C1O LXNHXLLTXMVWPM-UHFFFAOYSA-N 0.000 claims description 21
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- OVBPIULPVIDEAO-LBPRGKRZSA-N folic acid Chemical compound C=1N=C2NC(N)=NC(=O)C2=NC=1CNC1=CC=C(C(=O)N[C@@H](CCC(O)=O)C(O)=O)C=C1 OVBPIULPVIDEAO-LBPRGKRZSA-N 0.000 claims description 14
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- QIVBCDIJIAJPQS-VIFPVBQESA-N L-tryptophane Chemical compound C1=CC=C2C(C[C@H](N)C(O)=O)=CNC2=C1 QIVBCDIJIAJPQS-VIFPVBQESA-N 0.000 claims description 13
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- ODKSFYDXXFIFQN-BYPYZUCNSA-P L-argininium(2+) Chemical compound NC(=[NH2+])NCCC[C@H]([NH3+])C(O)=O ODKSFYDXXFIFQN-BYPYZUCNSA-P 0.000 claims description 12
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Abstract
Liquid metabolic formulas for dietary management of tyrosinemia, including nutritional formulas and hydration beverages (sport drinks).
Description
LIQUID NUTRITIONAL FORMULA FOR TYROSINEMIA PATIENTS
BACKGROUND
Tyrosinemia is a genetic disorder characterized by elevated blood levels of the amino acid tyrosine. Tyrosinemia is caused by deficiency of tyrosine metabolism, which leads to increased levels of tyrosine and tyrosine products in the body. There are three types of tyrosinemia, classed by the particular enzyme deficiency of the patient. Type 1 tyrosinemia, also known as hepatorenal tyrosinemia, is the most severe form of the disease and is caused by a deficiency of the enzyme fumarylacetoacetate hydrolase, which catalyzes the final step in tyrosine metabolism.
Type 1 tyrosinemia can lead to liver and kidney failure, problems affecting the nervous system and increased risk of liver cancer. Type 2 tyrosinemia is caused by a deficiency of the enzyme aminotransferase and affects the eyes, skin and mental development. Type 3 tyrosinemia is the rarest form and is caused by a deficiency of the enzyme 4-hydroxyphenylpyruvate dioxygenase.
Type 3 tyrosinemia is characterized by intellectual disability, seizures and intermittent ataxia.
Treatment of tyrosinemia typically involves a diet that is low in phenylalanine and low in tyrosine (low Phe/Tyr). However in children, the low Phe/Tyr diet must also be designed to provide enough of the two amino acids to support growth while avoiding excesses of both. This low Phe/Tyr diet must be consumed for life and compliance is an issue for many. Treatment may also include the administration of nitisinone, a medication that blocks the formation of fumarylacetoacetate.
SUMMARY
Nutritional formulation Described herein are metabolic formulas for the dietary management of tyrosinemia. In one embodiment, the metabolic formulation is a nutritional formula (also referred to as a nutritional formulation) that has a balanced amino acid profile suitable for therapy for individuals who have tyrosinemia; they include complete, peptide-based, ready-to-drink (RTD) nutritional formulas, such as shelf-stable, ready-to-drink (liquid), nutritional formulas that have a balanced amino acid profile and are suitable therapy for patients suffering from tyrosinemia. The RTD
nutritional formulas can be packaged using known methods. For example, the container can be a bottle, a carton or a can. In some embodiments, packaging is carried out under aseptic conditions. The RTD formula can be canned (e.g. placed in containers that are then hermetically sealed and sterilzed, such as by heat) or bottled. In some embodiments, the formula is bottled by a hot fill bottling process.
In a second embodiment, the metabolic formula is a hydration beverage (also referred to as a sports drink).
The nutritional formulas have a balanced amino acid profile suitable for therapy for individuals who have tyrosinemia, are low in phenylalanine and tyrosine, have a lower osmolality than a purely synthetic amino acid formula and have an acceptable taste. For example, the osmolality of a nutritional formula described herein is generally about 330 milliosmoles per liter. In contrast, the osmolality of purely synthetic amino acid formulas is generally about 1000 milliosmoles per liter.
The nutritional formula comprises in one embodiment: (a) a protein source that comprises (i) whey protein, such as caseino-glyco-macropeptide (cGMP) and (ii) complementary essential amino acids which are a mixture of arginine, tryptophan, leucine, histidine and cysteine and provides a balanced amino acid profile; (b) a carbohydrate source, which typically includes non-reducing sugars to minimize/reduce browning potential; (c) a fat (lipid/oil) source; and (d) vitamins and minerals in sufficient quantities to meet the daily requirements for each. In alternative embodiments, in (ii) the complimentary essential amino acids are a mixture of arginine, tryptophan, leucine, histidine and methionine or the complimentary essential amino acids are a mixture of arginine, tryptophan, leucine, histidine, cysteine and methionine. The nutritional formula comprises no added tyrosine or phenylalanine; tyrosine and phenylalanine in the nutritional formula are contributed by cGMP.
In addition, the nutritional formula typically, but optionally, includes flavors, which can be natural or artificial or a combination of both; coloring agents, which can be natural or artificial or a combination of both; sweetener, which can be natural or artificial or a combination of both;
gelling agents, thickening agents, stabilizing agents, sequestrants, emulsifiers or a combination of two or more of gelling agents, thickening agents, stabilizing agents, sequestrants, emulsifiers,
BACKGROUND
Tyrosinemia is a genetic disorder characterized by elevated blood levels of the amino acid tyrosine. Tyrosinemia is caused by deficiency of tyrosine metabolism, which leads to increased levels of tyrosine and tyrosine products in the body. There are three types of tyrosinemia, classed by the particular enzyme deficiency of the patient. Type 1 tyrosinemia, also known as hepatorenal tyrosinemia, is the most severe form of the disease and is caused by a deficiency of the enzyme fumarylacetoacetate hydrolase, which catalyzes the final step in tyrosine metabolism.
Type 1 tyrosinemia can lead to liver and kidney failure, problems affecting the nervous system and increased risk of liver cancer. Type 2 tyrosinemia is caused by a deficiency of the enzyme aminotransferase and affects the eyes, skin and mental development. Type 3 tyrosinemia is the rarest form and is caused by a deficiency of the enzyme 4-hydroxyphenylpyruvate dioxygenase.
Type 3 tyrosinemia is characterized by intellectual disability, seizures and intermittent ataxia.
Treatment of tyrosinemia typically involves a diet that is low in phenylalanine and low in tyrosine (low Phe/Tyr). However in children, the low Phe/Tyr diet must also be designed to provide enough of the two amino acids to support growth while avoiding excesses of both. This low Phe/Tyr diet must be consumed for life and compliance is an issue for many. Treatment may also include the administration of nitisinone, a medication that blocks the formation of fumarylacetoacetate.
SUMMARY
Nutritional formulation Described herein are metabolic formulas for the dietary management of tyrosinemia. In one embodiment, the metabolic formulation is a nutritional formula (also referred to as a nutritional formulation) that has a balanced amino acid profile suitable for therapy for individuals who have tyrosinemia; they include complete, peptide-based, ready-to-drink (RTD) nutritional formulas, such as shelf-stable, ready-to-drink (liquid), nutritional formulas that have a balanced amino acid profile and are suitable therapy for patients suffering from tyrosinemia. The RTD
nutritional formulas can be packaged using known methods. For example, the container can be a bottle, a carton or a can. In some embodiments, packaging is carried out under aseptic conditions. The RTD formula can be canned (e.g. placed in containers that are then hermetically sealed and sterilzed, such as by heat) or bottled. In some embodiments, the formula is bottled by a hot fill bottling process.
In a second embodiment, the metabolic formula is a hydration beverage (also referred to as a sports drink).
The nutritional formulas have a balanced amino acid profile suitable for therapy for individuals who have tyrosinemia, are low in phenylalanine and tyrosine, have a lower osmolality than a purely synthetic amino acid formula and have an acceptable taste. For example, the osmolality of a nutritional formula described herein is generally about 330 milliosmoles per liter. In contrast, the osmolality of purely synthetic amino acid formulas is generally about 1000 milliosmoles per liter.
The nutritional formula comprises in one embodiment: (a) a protein source that comprises (i) whey protein, such as caseino-glyco-macropeptide (cGMP) and (ii) complementary essential amino acids which are a mixture of arginine, tryptophan, leucine, histidine and cysteine and provides a balanced amino acid profile; (b) a carbohydrate source, which typically includes non-reducing sugars to minimize/reduce browning potential; (c) a fat (lipid/oil) source; and (d) vitamins and minerals in sufficient quantities to meet the daily requirements for each. In alternative embodiments, in (ii) the complimentary essential amino acids are a mixture of arginine, tryptophan, leucine, histidine and methionine or the complimentary essential amino acids are a mixture of arginine, tryptophan, leucine, histidine, cysteine and methionine. The nutritional formula comprises no added tyrosine or phenylalanine; tyrosine and phenylalanine in the nutritional formula are contributed by cGMP.
In addition, the nutritional formula typically, but optionally, includes flavors, which can be natural or artificial or a combination of both; coloring agents, which can be natural or artificial or a combination of both; sweetener, which can be natural or artificial or a combination of both;
gelling agents, thickening agents, stabilizing agents, sequestrants, emulsifiers or a combination of two or more of gelling agents, thickening agents, stabilizing agents, sequestrants, emulsifiers,
-2-4088596.1 each of which can be natural or artificial or a combination of both.
In one embodiment, the nutritional formula comprises: water, sucrose, whey protein isolate, vitamin and mineral blend (dicalcium phosphate, calcium lactate, dipotassium phosphate, choline bitartrate, magnesium citrate, sodium ascorbate and ascorbic acid, ferrous sulfate, zinc sulfate, niacinamide, vitamin E dl-alphatocopheryl acetate, calcium d-pantothenate, manganese sulfate, vitamin A palmitate, vitamin B6 pyridoxine, riboflavin, thiamin hydrochloride, copper gluconate, folic acid, potassium iodide, vitamin K 1 phytonadione, sodium selenite, sodium molybdate, chromium chloride, biotin, vitamin D3 cholecalciferol, vitamin B12 cyanocobalamin), food starch modified, leucine, maltodextrin, cocoa butter, canola oil, arginine, cellulose gel and carboxymethylcellulose sodium, natural flavor (propylene glycol, ethyl alcohol, water, polysorbate 80 potassium sorbate), histidine, one or more food grade polyphosphate chelator, such as sodium hexametaphosphate, cysteine, tryptophan, carrageenan, sodium stearoyl lactylate.
Also described are methods for producing the nutritional formula described herein. One embodiment of the method is represented schematically in Figure 1. In one embodiment, the method comprises: (a) kitting together dry ingredients; (b) kitting together a mineral mixture; (c) producing an oil incorporation mixture; (d) combining, in order, the mixtures produced in steps (a) to (c) in a high shear blender under conditions under which a stable emulsion formula is formed; (e) adding vitamin C to the formula (emulsion); (f) sterilizing the formula by ultra-high temperature aseptic treatment of the emulsion under conditions sufficient to sterilize the formula, for example at 140 C for 5-6 seconds; (g) optionally, storing the sterilized formula in an aseptic storage tank; and (h) aseptically packaging the formula.
Hydration beverage In some embodiments, the metabolic formula is a hydration beverage or sports drink for dietary management of individuals who have tyrosinemia. The hydration beverage or sports drink comprises: (a) a protein source that comprises (i) caseino-glyco-macropeptide (cGMP) and (ii) complementary essential amino acids which are a mixture of arginine, tryptophan, leucine, histidine and cysteine, and provides a balanced amino acid profile; (b) a carbohydrate source, which typically includes non-reducing sugars to minimize/reduce browning potential; and (c)
In one embodiment, the nutritional formula comprises: water, sucrose, whey protein isolate, vitamin and mineral blend (dicalcium phosphate, calcium lactate, dipotassium phosphate, choline bitartrate, magnesium citrate, sodium ascorbate and ascorbic acid, ferrous sulfate, zinc sulfate, niacinamide, vitamin E dl-alphatocopheryl acetate, calcium d-pantothenate, manganese sulfate, vitamin A palmitate, vitamin B6 pyridoxine, riboflavin, thiamin hydrochloride, copper gluconate, folic acid, potassium iodide, vitamin K 1 phytonadione, sodium selenite, sodium molybdate, chromium chloride, biotin, vitamin D3 cholecalciferol, vitamin B12 cyanocobalamin), food starch modified, leucine, maltodextrin, cocoa butter, canola oil, arginine, cellulose gel and carboxymethylcellulose sodium, natural flavor (propylene glycol, ethyl alcohol, water, polysorbate 80 potassium sorbate), histidine, one or more food grade polyphosphate chelator, such as sodium hexametaphosphate, cysteine, tryptophan, carrageenan, sodium stearoyl lactylate.
Also described are methods for producing the nutritional formula described herein. One embodiment of the method is represented schematically in Figure 1. In one embodiment, the method comprises: (a) kitting together dry ingredients; (b) kitting together a mineral mixture; (c) producing an oil incorporation mixture; (d) combining, in order, the mixtures produced in steps (a) to (c) in a high shear blender under conditions under which a stable emulsion formula is formed; (e) adding vitamin C to the formula (emulsion); (f) sterilizing the formula by ultra-high temperature aseptic treatment of the emulsion under conditions sufficient to sterilize the formula, for example at 140 C for 5-6 seconds; (g) optionally, storing the sterilized formula in an aseptic storage tank; and (h) aseptically packaging the formula.
Hydration beverage In some embodiments, the metabolic formula is a hydration beverage or sports drink for dietary management of individuals who have tyrosinemia. The hydration beverage or sports drink comprises: (a) a protein source that comprises (i) caseino-glyco-macropeptide (cGMP) and (ii) complementary essential amino acids which are a mixture of arginine, tryptophan, leucine, histidine and cysteine, and provides a balanced amino acid profile; (b) a carbohydrate source, which typically includes non-reducing sugars to minimize/reduce browning potential; and (c)
-3-4088596.1 vitamins and minerals. In addition, the hydration beverage or sports drink typically, but optionally, includes preservatives; flavors, which can be natural or artificial or a combination of both; coloring agents, which can be natural or artificial or a combination of both; sweetener, which can be natural or artificial or a combination of both; gelling agents, thickening agents, stabilizing agents, sequestrants, emulsifiers or a combination of two or more of gelling agents, thickening agents, stabilizing agents, sequestrants, emulsifiers, each of which can be natural or artificial or a combination of both. In one specific embodiment, the hydration beverage or sports drink comprises the components listed in Table 3. In one specific embodiment, the hydration beverage or sports drink comprises the components listed in Table 4. In a specific embodiment, the hydration beverage consists essentially of the components listed in Table 3 or consists essentially of the components listed in Table 4.
In a specific embodiment, the hydration beverage or sports drink comprises:
water, sugar (e.g., sucrose), a protein source, such as whey protein isolate (glycomacropeptide), citric acid, amino acids (e.g., leucine, arginine, histidine, cysteine, tryptophan), sodium citrate, natural flavors, (ethyl alcohol, natural flavors, propylene glycol, glycerine, polysorbate 60, citric acid), potassium sorbate (preservative), sodium benzoate (preservative), vitamin/mineral blend (sugar, niacin [vitamin b3], pantothenate [vitamin b5], pyridoxine [vitamin b6], riboflavin [vitamin b2], folic acid, biotin, cyanocobalamin [vitamin b12]), FD&C yellow #5, FD&C red #40. The hydration beverage or sports drink may optionally include flavors, which can be natural or artificial or a combination of both; coloring agents, which can be natural or artificial or a combination of both; sweetener, which can be natural or artificial or a combination of both;
gelling agents, thickening agents, stabilizing agents, sequestrants, emulsifiers or a combination of two or more of gelling agents, thickening agents, stabilizing agents, sequestrants, emulsifiers, each of which can be natural or artificial or a combination of both.
The hydration beverage or sports drink described herein is produced, using methods also described herein and other methods known to those of skill in the art. In one embodiment, the method comprises: (a) kitting together the dry ingredients; (b) blending together with the kitted dry ingredients in the following order (i) preservatives, (ii) a carbohydrate source (such as sucrose), (iii) a protein source (such as cGMP), (iv) amino acids (e.g., leucine, arginine, histidine,
In a specific embodiment, the hydration beverage or sports drink comprises:
water, sugar (e.g., sucrose), a protein source, such as whey protein isolate (glycomacropeptide), citric acid, amino acids (e.g., leucine, arginine, histidine, cysteine, tryptophan), sodium citrate, natural flavors, (ethyl alcohol, natural flavors, propylene glycol, glycerine, polysorbate 60, citric acid), potassium sorbate (preservative), sodium benzoate (preservative), vitamin/mineral blend (sugar, niacin [vitamin b3], pantothenate [vitamin b5], pyridoxine [vitamin b6], riboflavin [vitamin b2], folic acid, biotin, cyanocobalamin [vitamin b12]), FD&C yellow #5, FD&C red #40. The hydration beverage or sports drink may optionally include flavors, which can be natural or artificial or a combination of both; coloring agents, which can be natural or artificial or a combination of both; sweetener, which can be natural or artificial or a combination of both;
gelling agents, thickening agents, stabilizing agents, sequestrants, emulsifiers or a combination of two or more of gelling agents, thickening agents, stabilizing agents, sequestrants, emulsifiers, each of which can be natural or artificial or a combination of both.
The hydration beverage or sports drink described herein is produced, using methods also described herein and other methods known to those of skill in the art. In one embodiment, the method comprises: (a) kitting together the dry ingredients; (b) blending together with the kitted dry ingredients in the following order (i) preservatives, (ii) a carbohydrate source (such as sucrose), (iii) a protein source (such as cGMP), (iv) amino acids (e.g., leucine, arginine, histidine,
-4-4088596.1 cysteine, tryptophan) with buffers and citric acid, (v) flavorings, (vi) coloring, and (vii) sweeteners to form a hydration beverage; (c) titrating the pH of the hydration beverage; (d) optionally, ultra-heat treating the hydration beverage; (e) bottling the hydration beverage by a hot fill bottling process; and, (f) cooling the bottled hydration beverage in a cool water bath. In some embodiments, the bending is performed at ambient temperature (e.g., room temperature). In some embodiments, the blending, pH titrating and ultra-heat treating are performed while the hydration beverage is being continuously mixed. In some embodiments the preservatives are sorbate and benzoate. In some embodiments, the amount of sorbate ranges from about 0.05 g to about 1.0 g per 1030.4158 g of hydration beverage. In some embodiments, the amount of benzoate ranges from about 0.05 g to about 1.0 g per 1030.4158 g of hydration beverage. In some embodiments, the hydration beverage pH is titrated to between about pH
4.0 and about pH
5Ø In some embodiments, the hydration beverage pH is titrated to between about pH 4.3 and about pH 4.4.
BRIEF DESCRIPTION OF THE FIGURES
Figure 1 is a schematic representation of a method by which nutritional formulas are produced.
Figure 2 is a label for TYLACTIN RTD 15 ORIGINAL
Figure 3 is a label for TYLACTIN RESTORE 10 CITRUS
Figure 4 is a label for TYLACTIN RESTORE CITRUS
Figure 5 is a label for TYLACTIN RESTORE (FRENCH) Figure 6 is a label for TYLACTIN RTD ORIGINAL
DETAILED DESCRIPTION
Dietary compliance is the primary issue for tyrosinemia patients whose diets are based on amino acid formulas. Compliance is an issue and there have been many attempts to improve acceptance of foods and beverages for those with tyrosinemia but those efforts have not been successful.
Ready-to-drink, shelf-stable formulae, such as beverages, pose particular challenges, at
4.0 and about pH
5Ø In some embodiments, the hydration beverage pH is titrated to between about pH 4.3 and about pH 4.4.
BRIEF DESCRIPTION OF THE FIGURES
Figure 1 is a schematic representation of a method by which nutritional formulas are produced.
Figure 2 is a label for TYLACTIN RTD 15 ORIGINAL
Figure 3 is a label for TYLACTIN RESTORE 10 CITRUS
Figure 4 is a label for TYLACTIN RESTORE CITRUS
Figure 5 is a label for TYLACTIN RESTORE (FRENCH) Figure 6 is a label for TYLACTIN RTD ORIGINAL
DETAILED DESCRIPTION
Dietary compliance is the primary issue for tyrosinemia patients whose diets are based on amino acid formulas. Compliance is an issue and there have been many attempts to improve acceptance of foods and beverages for those with tyrosinemia but those efforts have not been successful.
Ready-to-drink, shelf-stable formulae, such as beverages, pose particular challenges, at
-5-4088596.1 least in part because the components essential for complete sustenance components must be combined to produce the nutritional formula in such a way that they remain dissolved, suspended or otherwise retained in the formula (beverage). This is a challenge because each ingredient in the nutritional formula has unique requirements for making it stable.
Particularly difficult to incorporate is the caseino-glycomacro-peptide, which contains sialic acid attached to an amino acid residue on the chain. This sialic acid reduces the isoelectric point of the peptide as a function of the fraction of sites occupied by sialic acid. Thus, the isoelectric point can change as the peptide is being processed. Peptides are not soluble at or near their isoelectric pH and tend to precipitate or gel. Described herein is a method by which nutritional formulas described are made, with the result that the formula is ready-to-drink and shelf-stable.
Nutritional Formula Preparation Nutritional formula preparation is carried out using the method represented schematically in the Figure. Kitting of ingredients is carried out as indicated: Kitting of dry ingredients, to produce a dry ingredient mixture, is carried out by combining dry ingredients without food grade polyphosphate chelator, such as sodium hexametaphosphate (SHMP), or mineral blend; kitting together of food grade polyphosphate chelator, , such as SHMP, CuSO4, FeSO4 and, if needed, other minerals; and kitting of ingredients for oil incorporation. The SHMP and minerals are mixed in water separately. Ingredients for oil incorporation are heated and incorporated together.
These include octenyl succinate starch (OSS), sodium stearoyl lactylate (SSL), cocoa butter, vegetable oil and other oil-soluble ingredients. They are subjected to high shear and homogenization. The resulting three combinations (see the Figure) are combined with water and other components as shown in Figure 1 and subjected to the conditions shown, beginning with the step "Sequentially add to high shear blender". The resulting formula is packaged, such as aseptically or by canning. In some embodiments, the dry ingredients include caseino glycomacropeptides (GMP), amino acids, and vitamins.
SHMP is combined with minerals, such as a metal fortification mixture, which comprises at least one type of metallic ions to produce a SHMP-minerals in water SHMP-metal mixture. In some embodiments, the metal fortification mixture comprises two or more types of metallic ions.
In some embodiments, the metallic ions are copper (e.g., Cu2') and/or iron (e.g., Fe2+) and/or
Particularly difficult to incorporate is the caseino-glycomacro-peptide, which contains sialic acid attached to an amino acid residue on the chain. This sialic acid reduces the isoelectric point of the peptide as a function of the fraction of sites occupied by sialic acid. Thus, the isoelectric point can change as the peptide is being processed. Peptides are not soluble at or near their isoelectric pH and tend to precipitate or gel. Described herein is a method by which nutritional formulas described are made, with the result that the formula is ready-to-drink and shelf-stable.
Nutritional Formula Preparation Nutritional formula preparation is carried out using the method represented schematically in the Figure. Kitting of ingredients is carried out as indicated: Kitting of dry ingredients, to produce a dry ingredient mixture, is carried out by combining dry ingredients without food grade polyphosphate chelator, such as sodium hexametaphosphate (SHMP), or mineral blend; kitting together of food grade polyphosphate chelator, , such as SHMP, CuSO4, FeSO4 and, if needed, other minerals; and kitting of ingredients for oil incorporation. The SHMP and minerals are mixed in water separately. Ingredients for oil incorporation are heated and incorporated together.
These include octenyl succinate starch (OSS), sodium stearoyl lactylate (SSL), cocoa butter, vegetable oil and other oil-soluble ingredients. They are subjected to high shear and homogenization. The resulting three combinations (see the Figure) are combined with water and other components as shown in Figure 1 and subjected to the conditions shown, beginning with the step "Sequentially add to high shear blender". The resulting formula is packaged, such as aseptically or by canning. In some embodiments, the dry ingredients include caseino glycomacropeptides (GMP), amino acids, and vitamins.
SHMP is combined with minerals, such as a metal fortification mixture, which comprises at least one type of metallic ions to produce a SHMP-minerals in water SHMP-metal mixture. In some embodiments, the metal fortification mixture comprises two or more types of metallic ions.
In some embodiments, the metallic ions are copper (e.g., Cu2') and/or iron (e.g., Fe2+) and/or
-6-Zinc (Zn+2). In some embodiments, the metallic ions are from sources such as CuSO4 and/or FeSO4 and/or ZnSO4. In some embodiments, a sequestrant or chelator other than a food grade polyphosphate chelator such as SHMP, is combined with minerals. Sequestrants include but are not limited to Calcium disodium ethylene diamine tetra-acetate (E385), Glucono delta-lactone (E575), Potassium gluconate (E577), Sodium gluconate (E576), Sodium triphosphate and EDTA.
An emulsion is prepared by combining and heating oil ingredients, which includes modified starch, sodium stearoyl lactylate (SSL), oils such as cocoa butter and vegetable oil, and other oil soluble ingredients. The modified starch used herein is an amphiphilic starch, which is hydrophilic and hydrophobic in nature. The modified starch is used to bind to fat to prevent the fat from interacting with the proteins by steric hindrance. For example, a normally hydrophilic starch is modified to incorporate hydrophobic moieties such as octenyl groups which binds to fats. The resulting modified starch has an amphiphilic nature and thus surface active properties which are useful in stabilizing oil/water emulsions. In some embodiments, the modified starch is octenyl succinate starch (OSS) or another amphiphilic starch. The hydrophobic octenyl moiety binds the fats in the emulsion assuring that the starch remains attached to the fat globule, preventing the fats from interacting with the proteins by steric hindrance.
The heated and combined oil ingredients are then added to a mixer, such as a high shear mixer, with water and are homogenized to form an emulsion. In some embodiments, the oil ingredients are homogenized with water at about 60-80 C and about 400 psi to about 3000 psi.
In some embodiments, the oil ingredients are homogenized in two steps. In some embodiments, the first emulsion step is performed at between about 60 C and about 80 C and at a pressure between about 1200 psi and 3000 psi. In some embodiments, the second emulsion step is performed at between about 60 C and about 80 C and at a pressure between about 300 psi and about 700 psi. In one embodiment, the oil ingredients is homogenized at about 70 C and about 500 psi to about 2000 psi.
As shown, the mixtures are sequentially added to a blender such as a high shear blender in the following order: the dry ingredient mixture is mixed into metered volume of water, the fortified SHMP mixture is added, the emulsion is incorporated, and vitamin C
is added last. In some embodiments, the vitamin C is sodium ascorbate and/or ascorbic acid. In some
An emulsion is prepared by combining and heating oil ingredients, which includes modified starch, sodium stearoyl lactylate (SSL), oils such as cocoa butter and vegetable oil, and other oil soluble ingredients. The modified starch used herein is an amphiphilic starch, which is hydrophilic and hydrophobic in nature. The modified starch is used to bind to fat to prevent the fat from interacting with the proteins by steric hindrance. For example, a normally hydrophilic starch is modified to incorporate hydrophobic moieties such as octenyl groups which binds to fats. The resulting modified starch has an amphiphilic nature and thus surface active properties which are useful in stabilizing oil/water emulsions. In some embodiments, the modified starch is octenyl succinate starch (OSS) or another amphiphilic starch. The hydrophobic octenyl moiety binds the fats in the emulsion assuring that the starch remains attached to the fat globule, preventing the fats from interacting with the proteins by steric hindrance.
The heated and combined oil ingredients are then added to a mixer, such as a high shear mixer, with water and are homogenized to form an emulsion. In some embodiments, the oil ingredients are homogenized with water at about 60-80 C and about 400 psi to about 3000 psi.
In some embodiments, the oil ingredients are homogenized in two steps. In some embodiments, the first emulsion step is performed at between about 60 C and about 80 C and at a pressure between about 1200 psi and 3000 psi. In some embodiments, the second emulsion step is performed at between about 60 C and about 80 C and at a pressure between about 300 psi and about 700 psi. In one embodiment, the oil ingredients is homogenized at about 70 C and about 500 psi to about 2000 psi.
As shown, the mixtures are sequentially added to a blender such as a high shear blender in the following order: the dry ingredient mixture is mixed into metered volume of water, the fortified SHMP mixture is added, the emulsion is incorporated, and vitamin C
is added last. In some embodiments, the vitamin C is sodium ascorbate and/or ascorbic acid. In some
-7-4088596.1 embodiments, the amount of vitamin C added is between about 120 mg and about 450 mg. In some embodiments, the final amount of vitamin C present in the formula is equal to 50% of the recommended daily allowance (RDA) of vitamin C. The combined mixture is subjected to ultra-high temperature processing (UHT). The resulting formula is subsequently placed/stored in aseptic tank and then aseptically packaged. In some embodiments, the aseptic packaging is a Prisma package. In some embodiments, the aseptic packaging is a can.
Hydration Beverage Preparation Hydration beverage or sports drink preparation is carried out using the method described herein. In one embodiment, the method comprises: (a) kitting together the dry ingredients; (b) blending together with the kitted dry ingredients in the following order (i) preservatives, (ii) a carbohydrate source (e.g., sucrose), (iii) a protein equivalent source (e.g., cGMP), (iv) amino acids (e.g., leucine, arginine, histidine, cysteine, tryptophan) with buffers and citric acid, (v) flavorings, (vi) coloring, and (vii) sweeteners to form a hydration beverage;
(c) titrating the pH of the hydration beverage; (d) ultra-heat treating the hydration beverage; (e) bottling the hydration beverage by a hot fill bottling process; and, (f) cooling the bottled hydration beverage in a cool water bath. In some embodiments, the bending is performed at ambient temperature (e.g., room temperature). In some embodiments, the blending, pH titrating and ultra-heat treating are performed while the hydration beverage is being continuously mixed. In some embodiments, the hydration beverage pH is titrated to between about pH 4.0 and about pH 5Ø In some embodiments, the hydration beverage pH is titrated to between about pH 4.3 and about pH 4.4.
EXAMPLE 1: Preparation of Complete Nutritional Formulation Dry ingredient mixture was prepared by kitting together the dry ingredients without sodium hexametaphosphate (SHMP), vitamins, or mineral. Table 1 lists dry ingredients used, such as whey protein isolate (such as caseino glycomacropeptides (GMP)), amino acids, vitamins, sugar, dextrin, natural flavoring products, natural food coloring, sodium chloride, cellulose gum, carrageenan, and sweeteners. In another step, SHMP is combined with a copper and iron fortification mixture. In another step, vitamins and minerals are mixed together in water
Hydration Beverage Preparation Hydration beverage or sports drink preparation is carried out using the method described herein. In one embodiment, the method comprises: (a) kitting together the dry ingredients; (b) blending together with the kitted dry ingredients in the following order (i) preservatives, (ii) a carbohydrate source (e.g., sucrose), (iii) a protein equivalent source (e.g., cGMP), (iv) amino acids (e.g., leucine, arginine, histidine, cysteine, tryptophan) with buffers and citric acid, (v) flavorings, (vi) coloring, and (vii) sweeteners to form a hydration beverage;
(c) titrating the pH of the hydration beverage; (d) ultra-heat treating the hydration beverage; (e) bottling the hydration beverage by a hot fill bottling process; and, (f) cooling the bottled hydration beverage in a cool water bath. In some embodiments, the bending is performed at ambient temperature (e.g., room temperature). In some embodiments, the blending, pH titrating and ultra-heat treating are performed while the hydration beverage is being continuously mixed. In some embodiments, the hydration beverage pH is titrated to between about pH 4.0 and about pH 5Ø In some embodiments, the hydration beverage pH is titrated to between about pH 4.3 and about pH 4.4.
EXAMPLE 1: Preparation of Complete Nutritional Formulation Dry ingredient mixture was prepared by kitting together the dry ingredients without sodium hexametaphosphate (SHMP), vitamins, or mineral. Table 1 lists dry ingredients used, such as whey protein isolate (such as caseino glycomacropeptides (GMP)), amino acids, vitamins, sugar, dextrin, natural flavoring products, natural food coloring, sodium chloride, cellulose gum, carrageenan, and sweeteners. In another step, SHMP is combined with a copper and iron fortification mixture. In another step, vitamins and minerals are mixed together in water
-8-4088596.1 to provide a vitamin and mineral fortification mixture. Subsequently, the SHMP
with the copper and iron fortification mixture is mixed with the vitamin and mineral fortification mixture, thereby forming a metal/mineral fortified SHMP mixture.
In a separate step, an emulsion is prepared by combining and heating oil ingredients.
The foregoing mixtures are then sequentially added to a blender such as a high shear blender in the following order: the dry ingredient mixture is mixed into metered volume of water, the fortified SHMP mixture is added, the emulsion is incorporated, and Vitamin C (sodium ascorbate and ascorbic acid) is added last. The table below provides the amounts of each ingredient used to prepare the formula. The combined mixture is subjected to ultra-high temperature processing (UHT) at about 140 C for about 5 to about 6 seconds The resulting formula is subsequently stored in aseptic tank and then aseptically packaged using Prisma Packages.
Table 1: Tylactin RTD Original Tylactin RTD Original Number of 250 ml Servings: 4 Weight: 1064 g Range Units % Weight Range Water 635-955 Gram Caseino glycomacropeptides (GMP) 40-60 Gram 3.76-5.64 Vitamin and mineral fortification 16-25 Gram 1.5-2.35 Amino Acids 16-25 Gram 1.5-2.35 Sugar 56-84 Gram 5.26-7.89 dextrin sweetener 8-12 Gram 0.75-1.13 Emulsion 72-108 Gram 6.77-10.15 Natural flavors 2.4-3.6 Gram 0.23-0.34 Natural color 0.4-0.6 Gram 0.04-0.06 sodium hexametaphosphate 1-2 Gram 0.09-0.19 cellulose gum 3.8-6 Gram 0.36-0.56 carrageenan 0.2-0.5 Gram 0.02-0.05 .05-1.0 Gram 0.004-0.09 .05-1.0 Gram 0.004-0.09
with the copper and iron fortification mixture is mixed with the vitamin and mineral fortification mixture, thereby forming a metal/mineral fortified SHMP mixture.
In a separate step, an emulsion is prepared by combining and heating oil ingredients.
The foregoing mixtures are then sequentially added to a blender such as a high shear blender in the following order: the dry ingredient mixture is mixed into metered volume of water, the fortified SHMP mixture is added, the emulsion is incorporated, and Vitamin C (sodium ascorbate and ascorbic acid) is added last. The table below provides the amounts of each ingredient used to prepare the formula. The combined mixture is subjected to ultra-high temperature processing (UHT) at about 140 C for about 5 to about 6 seconds The resulting formula is subsequently stored in aseptic tank and then aseptically packaged using Prisma Packages.
Table 1: Tylactin RTD Original Tylactin RTD Original Number of 250 ml Servings: 4 Weight: 1064 g Range Units % Weight Range Water 635-955 Gram Caseino glycomacropeptides (GMP) 40-60 Gram 3.76-5.64 Vitamin and mineral fortification 16-25 Gram 1.5-2.35 Amino Acids 16-25 Gram 1.5-2.35 Sugar 56-84 Gram 5.26-7.89 dextrin sweetener 8-12 Gram 0.75-1.13 Emulsion 72-108 Gram 6.77-10.15 Natural flavors 2.4-3.6 Gram 0.23-0.34 Natural color 0.4-0.6 Gram 0.04-0.06 sodium hexametaphosphate 1-2 Gram 0.09-0.19 cellulose gum 3.8-6 Gram 0.36-0.56 carrageenan 0.2-0.5 Gram 0.02-0.05 .05-1.0 Gram 0.004-0.09 .05-1.0 Gram 0.004-0.09
-9-4088596.1 Table 2: Tylactin RTD 15 Original TYLACTIN RTD 15 per serving Range % Weight per 100mL
ORIGINAL 8.5 fl oz Range (250mL) Nutrients Calories 200 160-240 80 Calories From Fat 45 36-54 18 Protein Equivalent, g 15 12-18 4.51-6.77 6 Free Amino Acids, g 0 Tyrosine, mg 3 2.4-3.6 <.01% 1 Carbohydrates, g 24 19.2-28.8 7.22-10.83 10 Sugar, g 19 15.2-22.8 5.71-8.57 8 Sugar Alcohols, g 0 0 0 Dietary Fiber, g 1 0.8-1.2 0.30-0.45 0 Fat, g 5 4-6 1.50-2.26 2 Saturated Fat, g 2 1.6-2.4 0.60-0.90 0.8 Trans Fat, g 0 0 0 Cholesterol, mg 0 0 0 Vitamin A, IU (mcg) 900 (495 720-1080 360 mcg) (396¨ <0.01%
594 mcg) Vitamin C, mg 28 22.4-33.6 8.42-12.63 11.2 Vitamin D, IU (mcg) 250 (6.25 200-300 100 <0.01%
mcg) (5-7 mcg) Vitamin E, IU 5 (5 mg) 4-6 (4-6 2 <0.01%
mg) Vitamin K, mcg 30 24-36 <0.01% 12 Thiamin (B1), mg 0.4 0.32-0.48 <0.01% 0.2 Riboflavin (B2), mg 0.4 0.32-0.48 <0.01% 0.2 Niacin (B3), mg 5 4-6 <0.01% 2 Vitamin B6, mg 0.4 0.32-0.48 <0.01% 0.2 Folic acid, mcg 140 112-168 <0.01% 56 Vitamin B12, mcg 0.8 0.64-0.96 <0.01% 0.3 Pantothenic Acid (B5), 1.8 0.7 1.44-2.16 <0.01%
mg Biotin, mcg 8 6.4-9.6 <0.01% 3.2
ORIGINAL 8.5 fl oz Range (250mL) Nutrients Calories 200 160-240 80 Calories From Fat 45 36-54 18 Protein Equivalent, g 15 12-18 4.51-6.77 6 Free Amino Acids, g 0 Tyrosine, mg 3 2.4-3.6 <.01% 1 Carbohydrates, g 24 19.2-28.8 7.22-10.83 10 Sugar, g 19 15.2-22.8 5.71-8.57 8 Sugar Alcohols, g 0 0 0 Dietary Fiber, g 1 0.8-1.2 0.30-0.45 0 Fat, g 5 4-6 1.50-2.26 2 Saturated Fat, g 2 1.6-2.4 0.60-0.90 0.8 Trans Fat, g 0 0 0 Cholesterol, mg 0 0 0 Vitamin A, IU (mcg) 900 (495 720-1080 360 mcg) (396¨ <0.01%
594 mcg) Vitamin C, mg 28 22.4-33.6 8.42-12.63 11.2 Vitamin D, IU (mcg) 250 (6.25 200-300 100 <0.01%
mcg) (5-7 mcg) Vitamin E, IU 5 (5 mg) 4-6 (4-6 2 <0.01%
mg) Vitamin K, mcg 30 24-36 <0.01% 12 Thiamin (B1), mg 0.4 0.32-0.48 <0.01% 0.2 Riboflavin (B2), mg 0.4 0.32-0.48 <0.01% 0.2 Niacin (B3), mg 5 4-6 <0.01% 2 Vitamin B6, mg 0.4 0.32-0.48 <0.01% 0.2 Folic acid, mcg 140 112-168 <0.01% 56 Vitamin B12, mcg 0.8 0.64-0.96 <0.01% 0.3 Pantothenic Acid (B5), 1.8 0.7 1.44-2.16 <0.01%
mg Biotin, mcg 8 6.4-9.6 <0.01% 3.2
-10-Choline, mg 206 164.8- 82.4 0.06-0.09 247.2 Calcium, mg 350 280-420 0.11-0.16 140 Chromium, mcg 13 10.4-15.6 <0.01% 5.2 Copper, mg 0.2 0.16-0.24 <0.01% 0.1 Iodine, mcg 57 45.6-68.4 <0.01% 22.8 Iron, mg 4.5 3.6-5.4 <0.01% 1.8 Magnesium, mg 120 96-144 0.04-0.05 48 Manganese, mg 0.8 0.64-0.96 <0.01% 0.3 Molybdenum, mcg 15 12-18 <0.01% 6 Phosphorus, mg 315 252-378 0.09-0.14 126 Selenium, mcg 20 16-24 <0.01% 8 Zinc, mg 3.3 2.64-3.96 <0.01% 1.3 Potassium, mg 340 272-408 0.10-0.15 136 Sodium, mg 300 240-360 0.09-0.14 120 Chloride, mg 162 129.6- 64.8 0.05-0.07 194.4 Inositol, mg 0 0 0.00-0.00 0 L-alanine mg 700 560-840 0.21-0.32 280 L-arginine mg 1593 1274.4- 637.2 0.48-0.72 1911.6 L-aspartate mg 1000 800-1200 0.30-0.45 400 L-cysteine, mg 162 129-194 0.05-0.07 64.8 L-glutamate, mg 2250 1800- 900 0.68-1.01 L-glycine, mg 148 118.4- 59.2 0.04-0.07 177.6 L-histidine, mg 361 288.8- 144.4 0.11-0.16 433.2 L-isoleucine, mg 1300 1040- 520 0.39-0.59 L-leucine, mg 3000 2400- 1200 0.90-1.35 L-lysine, mg 818 654.4- 327.2 0.25-0.37 981.6 L-methionine, mg 180 144-216 0.05-0.08 72 L-phenylalanine, mg 25 20-30 0.01-0.01 10 L-proline, mg 1400 1120- 0.42-0.63 560
-11-4088596.1 L-serine, mg 800 640-960 0.24-0.36 320 L-threonine, mg 2200 1760- 880 0.66-0.99 L-tryptophan, mg 218 174.4- 87.4 0.07-0.10 261.6 L-tyrosine, mg 3 2.4-3.6 1.2 L-valine, mg 900 720-1080 0.27-0.41 360 Table 3: Tylactin Restore 10 Citrus Tylactin Restore 10 Citrus Number of Servings: 2 (515 g per serving) Weight: 1030 g Range Measure %
Weight Range Water 752- Gram (Vitamins and minerals) 0.06- Gram 0.01-0.01 0.08 Complementary Amino 5.6-8.4 Gram 0.54-0.82 Acids Casein- 12.8- Gram 1.24-1.86 glycomacropeptides 19.2 Table 4: Tylactin Restore 10 Citrus Tylactin Restore 10 Citrus Nutrients per serving Range % Weight per 16.9 fl oz Range 100mL
(500mL) Calories 170 136-204 34 Calories From Fat 1 0.8-1.2 0 Protein Equivalent, g 10 8-10 0.78-1.16 2 Free Amino Acids, g
Weight Range Water 752- Gram (Vitamins and minerals) 0.06- Gram 0.01-0.01 0.08 Complementary Amino 5.6-8.4 Gram 0.54-0.82 Acids Casein- 12.8- Gram 1.24-1.86 glycomacropeptides 19.2 Table 4: Tylactin Restore 10 Citrus Tylactin Restore 10 Citrus Nutrients per serving Range % Weight per 16.9 fl oz Range 100mL
(500mL) Calories 170 136-204 34 Calories From Fat 1 0.8-1.2 0 Protein Equivalent, g 10 8-10 0.78-1.16 2 Free Amino Acids, g
-12-4088596.1 Tylactin Restore 10 Citrus Tyrosine, mg 2 1.6-2.4 <0.01% 0 Carbohydrates, g 31 24-38 2.41-3.61 6 Sugar, g 30 24-36 2.33-3.49 6 Sugar Alcohols, g Dietary Fiber, g 0 0 Fat, g 0 0 Saturated Fat, g 0 0 Trans Fat, g 0 0 Cholesterol, mg 0 0 Vitamin A, IU 0 0 Vitamin C, mg 0 0 Vitamin D, IU
Vitamin E, IU
Vitamin K, mcg Thiamin (B1), mg 0 0 Riboflavin (B2), mg 0.4 0.32-0.48 <0.01% 0.1 Niacin (B3), mg 5 42100 <0.01% 1 Vitamin B6, mg 0.5 0.4-0.6 <0.01% 0.1 Folic acid, mcg 100 80-120 <0.01% 20 Vitamin B12, mcg 1.8 1.44-2.16 <0.01% 0.4 Pantothenic Acid (B5), 2.5 0.5 42038 <0.01%
mg Biotin, mcg 75 60-90 <0.01% 15 Choline, mg Calcium, mg 10 42228 <0.01% 2 Chromium, mcg Copper, mg Iodine, mcg Iron, mg Magnesium, mg 6.4 5.12-7.68 <0.01% 1.3 Manganese, mg Molybdenum, mcg Phosphorus, mg 40 32-48 <0.01% 8 Selenium, mcg Zinc, mg
Vitamin E, IU
Vitamin K, mcg Thiamin (B1), mg 0 0 Riboflavin (B2), mg 0.4 0.32-0.48 <0.01% 0.1 Niacin (B3), mg 5 42100 <0.01% 1 Vitamin B6, mg 0.5 0.4-0.6 <0.01% 0.1 Folic acid, mcg 100 80-120 <0.01% 20 Vitamin B12, mcg 1.8 1.44-2.16 <0.01% 0.4 Pantothenic Acid (B5), 2.5 0.5 42038 <0.01%
mg Biotin, mcg 75 60-90 <0.01% 15 Choline, mg Calcium, mg 10 42228 <0.01% 2 Chromium, mcg Copper, mg Iodine, mcg Iron, mg Magnesium, mg 6.4 5.12-7.68 <0.01% 1.3 Manganese, mg Molybdenum, mcg Phosphorus, mg 40 32-48 <0.01% 8 Selenium, mcg Zinc, mg
-13-Tylactin Restore 10 Citrus Potassium, mg 200 160-240 0.02-0.02 40 Sodium, mg 380 304-456 0.03-0.04 76 Chloride, mg 8 6.4-9.6 <0.01% 1.6 Inositol, mg L-alanine, mg 520 416-624 0.04-0.06 104 L-arginine, mg 1100 880-1320 0.09-0.13 220 L-aspartate, mg 730 584-876 0.06-0.09 146 L-cysteine, mg 107.7 86-129 <0.01% 21.5 L-glutamate, mg 1600 1280-1920 0.12-0.19 320 L-glycine, mg 95 76-114 0.01-0.01 19 L-histidine, mg 250 200-300 0.02-0.03 50 L-isoleucine, mg 900 720-1080 0.07-0.10 180 L-leucine, mg 2110 1688-2532 0.16-0.25 422 L-lysine, mg 520 416-624 0.04-0.06 104 L-methionine, mg 170 136-204 0.01-0.02 34 L-phenylalanine, mg 16 12.8-19.2 <0.01% 3.2 L-proline, mg 1000 800-1200 0.08-0.12 200 L-serine, mg 640 512-768 0.05-0.07 128 L-threonine, mg 1400 1120-1680 0.11-0.16 280 L-tryptophan, mg 150 120-180 0.01-0.02 30 L-tyrosine, mg 2 1.6-2.4 <0.01% 0.4 L-valine, mg 700 560-840 0.05-0.08 140
-14-
Claims (21)
1. A liquid, nutritional formula treating for tyrosinemia comprising: (a) per 8.5 fluid ounces (250ml) or (b) per 100ml
2. The nutritional formula according to claim 1 in which the protein equivalent source provides 20-35% of the energy of the nutritional formula, the fat source provides 20% to 30% of the energy of the nutritional formula, and the carbohydrate source provides 40% to 60% of the energy of the nutritional formula.
3. The nutritional formula according to claim 1 in which complementary amino acids for every gram of protein equivalent (PE) typically comprise:
Tryptophan all percentages being by weight of the complementary amino acids, amino acid esters or salts of amino acids.
Tryptophan all percentages being by weight of the complementary amino acids, amino acid esters or salts of amino acids.
4. A hydration beverage for dietary management of tyrosinemia, comprising:
(a) per 16.9 fluid ounces (500 ml):
or (b) per 100ml
(a) per 16.9 fluid ounces (500 ml):
or (b) per 100ml
5. A liquid nutritional formula for dietary management of tyrosinemia comprising a protein source that includes (i) caseino-glyco-macropeptide (cGMP) and (ii) complementary essential 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.
6. The liquid nutritional formula of claim 5, wherein the complementary essential amino acids are about 1.5% to about 2.35% of the total weight of the formula.
7. The liquid nutritional formula of claim 5 or 6, wherein the complementary essential amino acids comprise at least two of the following: arginine, tryptophan, leucine, histidine and cysteine.
8. The liquid nutritional formula of any one of claims 5-7, wherein the formula contains less than 0.01% by weight of tyrosine.
9. The liquid nutritional formula of any one of claims 5 to 8, further comprising a fat source, wherein the fat source is about 1.5% to about 2.2% of the total weight of the formula.
10. The liquid nutritional formula of any one of claims 5 to 9, further comprising at least one vitamin and at least one mineral, wherein 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.
11. The liquid nutritional formula of any one of claims 5 to 10, wherein the at least one vitamin and/or mineral is selected from the group consisting of: dicalcium phosphate, calcium lactate, dipotassium phosphate, choline bitartrate, magnesium citrate, sodium ascorbate and ascorbic acid, ferrous sulfate, zinc sulfate, niacinamide, vitamin E dl-alphatocopheryl acetate, calcium d-pantothenate, manganese sulfate, vitamin A palmitate, vitamin B6 pyridoxine, riboflavin, thiamin hydrochloride, copper gluconate, folic acid, potassium iodide, vitamin K 1 phytonadione, sodium selenite, sodium molybdate, chromium chloride, biotin, vitamin D3 cholecalciferol, and vitamin B12 cyanocobalamin.
12. A liquid nutritional formula for dietary management of tyrosinemia comprising a protein source that includes (i) caseino-glyco-macropeptide (cGMP) and (ii) complementary essential amino acids which are a mixture of arginine, tryptophan, leucine, histidine, and the protein source provides between about 28% and about 43% of the total calories in the formula.
13. The liquid nutritional formula of claim 12, further comprising a fat source, wherein the fat source provides between about 18% to about 27% of the total calories in the formula.
14. The liquid nutritional formula of claim 12 or 13, wherein the formula contains less than 0.01% by weight of tyrosine.
15. The liquid nutritional formula of any one of claims 12 to 14, further comprising at least one vitamin and/or mineral, wherein the vitamin and/or mineral is present in an amount sufficient to make the formula nutritionally complete.
16. The liquid nutritional formula of any one of claims 12 to 15, wherein the at least one vitamin and/or mineral is selected from the group consisting of: dicalcium phosphate, calcium lactate, dipotassium phosphate, choline bitartrate, magnesium citrate, sodium ascorbate and ascorbic acid, ferrous sulfate, zinc sulfate, niacinamide, vitamin E dl-alphatocopheryl acetate, calcium d-pantothenate, manganese sulfate, vitamin A palmitate, vitamin B6 pyridoxine, riboflavin, thiamin hydrochloride, copper gluconate, folic acid, potassium iodide, vitamin K 1 phytonadione, sodium selenite, sodium molybdate, chromium chloride, biotin, vitamin D3 cholecalciferol, and vitamin B12 cyanocobalamin.
17. A hydration beverage for dietary management of tyrosinemia comprising a protein source that includes (i) caseino-glyco-macropeptide (cGMP) and (ii) complementary essential amino acids which are a mixture of arginine, tryptophan, leucine, histidine, wherein the cGMP is between about 1.2% and about 1.9% of the total weight of the beverage.
18. The hydration beverage of claim 17, wherein the complementary amino acids are between about 0.5% and about 0.9% of the total weight of the beverage.
19. The hydration beverage of claim 17 or 18, wherein the beverage contains less than 0.01% by weight of tyrosine.
20. The hydration beverage of any one of claims 17 to 19, further comprising a carbohydrate source, wherein the carbohydrate source is between about 2.4% to about 3.6% of the total weight of the beverage.
21. The hydration beverage of any one of claims 19 to 22 further comprising at least one vitamin, at least one mineral or at least one vitamin and at least mineral selected from the group consisting of: vitamin B3, vitamin B5, vitamin B6, vitamin B2, folic acid, biotin, and vitamin B12.
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CA2894333A CA2894333C (en) | 2015-06-12 | 2015-06-12 | Liquid nutritional formula for tyrosinemia patients |
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CA2894333A CA2894333C (en) | 2015-06-12 | 2015-06-12 | Liquid nutritional formula for tyrosinemia patients |
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CA2894333A1 true CA2894333A1 (en) | 2016-12-12 |
CA2894333C CA2894333C (en) | 2022-12-13 |
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CA2894333A Active CA2894333C (en) | 2015-06-12 | 2015-06-12 | Liquid nutritional formula for tyrosinemia patients |
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