Formula powder for patients with maple sugar urine diseases and preparation method of formula powder

Abstract

The invention relates to formula powder for patients with maple sugar urine diseases and a preparation method of the formula powder. The formula powder for the patients with maple sugar urine diseases is prepared from the following components in parts by weight: 10 to 20 parts of compound amino acid, 50 to 60 parts of protein-free fat powder, 11.1 to 35.5 parts of solid corn syrup, 0.8 to 1.0 part of DHA (docosahexaenoic acid) algae oil powder, 0.5 to 1.5 parts of ARA (arachidonic acid) powder, 0.56 to 0.74 part of compound vitamin, 3.6 to 5.6 parts of compound mineral substances and 0.04 to 0.06 part of nucleotide, the formula powder for the patients with maple sugar urine diseases contains medium and long chain fatty acid triglyceride MLCT, and does not contain leucine, isoleucine and valine. The formula powder is beneficial to nutrition intake and growth and development of patients, anaphylactic reactions are effectively reduced, and intelligence development of child patients is improved.

Description

Technical Field

[0001] The present invention relates to the field of special medical purpose formula foods, and in particular, to a formula powder containing medium-chain fatty acids (MLCT) for maple syrup urine disease patients and a preparation method thereof. Background Art

[0002] Maple syrup urine disease is a branched-chain amino acid metabolism disorder and is an autosomal recessive genetic disease. The incidence rate in newborns is about 1 / 185,000, and may be higher in some people with close relatives. Due to the congenital defect of the branched-chain α-ketoacid dehydrogenase complex in the body, the catabolism of branched-chain amino acids (leucine, isoleucine, and valine) is impaired. The corresponding branched-chain α-ketoacids cannot further catalyze decarboxylation, resulting in increased levels of branched-chain amino acids and branched-chain α-ketoacids in tissues, interfering with the synthesis of neurotransmitters and myelin in the brain, leading to severe nerve damage and even life-threatening acute metabolic disorders. The treatment of maple syrup urine disease is mainly to limit the intake of branched-chain amino acids. Early diagnosis and timely and effective nutritional intervention can help improve the prognosis of the disease.

[0003] At present, the main infant formula for maple syrup urine disease is imported from overseas. However, the price of products obtained through overseas shopping is high, which is beyond the economic affordability of most people, making patients face great challenges in economic burden. The patent with publication number CN107772459A relates to a formula powder for maple syrup urine disease and its preparation method. The formula powder is composed of vegetable oil fat powder, amino acids, fatty acids, algae oil, nucleotides, complex vitamins, and complex minerals. It is prepared by a dry preparation process, and leucine, isoleucine, and valine are removed from the raw materials. On the one hand, the formula powder can ensure the nutritional supplementation of patients, and on the other hand, it can effectively control the content of leucine, isoleucine, and valine in patients; through the scientific ratio of nutritional components, the solubility and stability are good, the preparation method is simple, and it is suitable for large-scale industrial production. However, this formula product has the problem of nutritional imbalance. Summary of the invention

[0004] The following is an overview of the subject matter described in detail herein. This overview is not intended to limit the scope of protection of this application.

[0005] At present, in the field of infant food for special medical purposes such as maple syrup urine disease, there is an urgent need to provide a formula food that can promote growth and development for maple syrup urine disease. The medium-chain fatty acid MLCT is helpful for the growth and development of children with maple syrup urine disease. Specifically, it can effectively promote the growth and development of children with maple syrup urine disease, such as height, weight, and head circumference. Therefore, the technical problem to be solved by the present application is to provide a formula powder that can promote growth and development and has special medical uses, especially for infant maple syrup urine disease, and a preparation method thereof. The formula powder of the present application is beneficial to the patient's nutritional intake and growth and development, effectively reduces allergic reactions, and improves the intellectual development of children.

[0006] In view of the deficiencies of the prior art, the first aspect of the present application provides a formula powder for patients with maple syrup urine disease, comprising the following components: 10-20 parts by weight of compound amino acids, 50-60 parts by weight of protein-free fat powder, Solid corn syrup 11.1-35.5 parts by weight, DHA algae oil powder 0.8-1.0 weight portion, ARA powder 0.5-1.5 parts by weight, 0.56-0.74 parts by weight of multivitamins, 3.6-5.6 parts by weight of composite minerals, Nucleotides 0.04-0.06 parts by weight; The formula powder for patients with maple syrup urine disease contains medium-chain fatty acid triglycerides MLCT, but does not contain leucine, isoleucine, or valine.

[0007] In an exemplary embodiment, the formula powder for patients with maple syrup urine disease consists of the following components: 10-20 parts by weight of compound amino acids, 50-60 parts by weight of protein-free fat powder, Solid corn syrup 11.1-35.5 parts by weight, DHA algae oil powder 0.8-1.0 weight portion, ARA powder 0.5-1.5 parts by weight, 0.56-0.74 parts by weight of multivitamins, 3.6-5.6 parts by weight of composite minerals, Nucleotides 0.04-0.06 parts by weight; The formula powder for patients with maple syrup urine disease contains medium-chain fatty acid triglycerides MLCT, but does not contain leucine, isoleucine, or valine.

[0008] In an exemplary embodiment, the compound amino acids include, by weight: 1.1-1.4 parts of glycine, 0.6-1.0 parts of L-alanine, 1.2-1.6 parts of L-arginine, 1.0-1.4 parts of L-aspartic acid, 0.4-0.6 parts of L-cysteine, 0.05-0.2L-part of glutamine, 0.6-1.0 parts of L-histidine, 1.3-1.7 parts of L-lysine, 0.2-0.6 parts of L-methionine, 0.8-1.2 parts of L-phenylalanine, 1.3-1.8 parts of L-proline, 0.8-1.2L-serine, 0.9-1.2 parts of L-threonine, 0.3-0.6 parts of L-tryptophan, and 0.8-1.2 parts of L-tyrosine.

[0009] In an exemplary embodiment, the protein content of the protein-free fat powder is ≤0.5g / 100g, and the fat content is ≥30g / 100g.

[0010] In an exemplary embodiment, the protein-free fat powder is a fat powder produced using raw materials and auxiliary materials that do not contain protein.

[0011] In an exemplary embodiment, the protein-free fat powder is made from edible vegetable oil as raw material, with the addition of encapsulating materials, with or without the addition of emulsifiers, antioxidants, acidity regulators and other food additives, and is processed through main processes such as batching, homogenization, sterilization, spray drying, and packaging.

[0012] In an exemplary embodiment, the source of the edible vegetable oil is selected from vegetable oil, animal fat, other fats or a combination of several fats, but hydrogenated fats are not used.

[0013] In an exemplary embodiment, the protein-free fat powder is composed of the following by weight: 40-60 parts of glucose syrup, 30-50 parts of edible vegetable blending oil, 8-20 parts of sodium octenyl succinate starch, 1-2 parts of mono- and di-glycerol fatty acid esters, and 0.1-0.8 parts of sodium ascorbate.

[0014] In an exemplary embodiment, the edible vegetable blended oil is composed of the following by weight: 25-45 parts of medium-chain fatty acid triglycerides MLCT, 40-50 parts of sunflower oil, 15-35 parts of low-erucic acid rapeseed oil, 0.01-0.4 parts of mixed tocopherol concentrate, and 0.001-0.1 parts of ascorbyl palmitate.

[0015] In an exemplary embodiment, the DHA algae oil powder is composed of the following by weight: 20-50 parts of glucose syrup, 20-50 parts of sodium starch octenylsuccinate, 22-32 parts of docosahexaenoic acid oil, 1-6 parts of sodium ascorbate, 0.1-4 parts of mono- and diglycerol fatty acid esters, 0.02-3 parts of sodium citrate, 0.1-2 parts of tricalcium phosphate, 0.05-2 parts of phospholipids, and 0.05-2 parts of ascorbyl palmitate.

[0016] In an exemplary embodiment, the ARA powder is composed of the following by weight: 20-50 parts of glucose syrup, 20-50 parts of sodium starch octenylsuccinate, 22-32 parts of arachidonic acid oil, 1-6 parts of sodium ascorbate, 0.1-4 parts of mono- and diglycerol fatty acid esters, 0.02-3 parts of sodium citrate, 0.1-2 parts of tricalcium phosphate, 0.05-2 parts of phospholipids, and 0.05-2 parts of ascorbyl palmitate.

[0017] In an exemplary embodiment, the multivitamin comprises: vitamin A acetate 756250.000~1023161.765 mcg / kg, vitamin D3 16250~21985.294 mcg / kg, mixed tocopherol concentrate 5250.000~7102.941 mg / kg, vitamin K1 81250~109926.471 mcg / kg, vitamin B1 81250-109926.471 mcg / kg, riboflavin 1308375.000-1133595.588mcg / kg, nicotinamide 8471375.000-11461272.059mcg / kg, pantothenic acid 4791875.000-6483125.000mcg / kg, vitamin B6 802000.0000-1085058.824 mcg / kg, D-biotin 30625.000-41433.824mcg / kg, folic acid 138000.000-186705.882mcg / kg, vitamin B 12 2500.000-3382.353 mcg / kg, vitamin C 6617.647-8088.235 mg / kg, choline 158823.529-194117.647mg / kg, iodine 129375.000-175036.765mcg / kg, selenium 25125.000-33992.647mcg / kg, L-carnitine 23823.529-29117.647mg / kg, taurine 53867.647-65838.235mg / kg, inositol 158823.529-194117.647mg / kg.

[0018] In an exemplary embodiment, the complex minerals include: copper 90097.826-110119.565mcg / kg, iron 1213.044-1482.609mg / kg, magnesium 11523.913-14084.783mg / kg, manganese 44551.087-60275.000mg / kg, sodium 23576.087-28815.217mg / kg, zinc 1213.044-1482.609mg / kg, calcium 121304.348-148260.870mg / kg and chlorine 31304.348-38260.870mg / kg.

[0019] In an exemplary embodiment, the nucleotides are composed of the following components by mass percentage: 15%-25% adenosine 5'-monophosphate, 20%-40% disodium 5'-cytidylate, 20%-30% disodium 5'-uridylate, 6%-10% disodium 5'-guanylate, and 10%-20% disodium 5'-inosinate; Preferably, the nucleotides are composed of the following components by mass percentage: 20.3% adenosine 5'-monophosphate, 30% disodium 5'-cytidylate, 24% disodium 5'-uridylate, 6.9% disodium 5'-guanylate, and 13.8% disodium 5'-inosinate.

[0020] In an exemplary embodiment, the molecular weight distribution of the formula powder for maple syrup urine disease patients is 94.4-95.2% for ≤500Da, 99.4%-99.6% for ≤1000Da, and 0.4-0.6% for >1000Da, which can play a role in reducing the risk of food protein allergy.

[0021] In an exemplary embodiment, in the formula powder for patients with maple syrup urine disease, the content of casein, soy protein, and whole milk protein allergens is less than 2.5 mg / kg, the content of β-lactoglobulin is less than 0.167 mg / kg, and the content of β-lactoglobulin fragment is less than 5 mg / kg, which can effectively reduce the risk of food protein allergy.

[0022] The second aspect of the present application provides a method for preparing the above-mentioned formula powder for patients with maple syrup urine disease, comprising the following steps: Step 1) Weighing: Weigh the materials according to the formula, and prepare the materials according to the formula after weighing; Step 2) Premixing: Put the composite minerals, ARA powder, nucleotides, DHA algae oil powder, composite vitamins, and compound amino acids into the premixer in sequence, and put in the protein-free fat powder for premixing; Step 3) Feeding and screening: the premixed materials and the remaining raw materials are fed into the dry mixing tank for screening according to the feeding requirements; Step 4) dry mixing: dry mixing the materials after sieving; Step 5) Metal detection: The material in the dry mixing tank is transported to a metal detector for detection, and after detection of no metal impurities, it is filled; Step 6) Filling: Filling the material according to the set net content to make the product; Step 7) Packing: adding an outer cover to the product, packing it in a box, and sealing it; Step 8) Palletizing: palletizing the products according to palletizing requirements; Step 9) Warehousing: transporting the product to the finished product warehouse for warehousing; Step 10) Shipping: The product will be shipped after passing the inspection.

[0023] In an exemplary embodiment, in step 2), the premixing is eccentric mixing, and the premixing parameters are as follows: premixing time 20 min; premixing speed 12 r / min.

[0024] In an exemplary embodiment, in step 3), the mesh size of the sieving process is 16-20 meshes.

[0025] In an exemplary embodiment, in step 4), the dry mixing is eccentric mixing, and the parameters of the dry mixing are as follows: dry mixing time 30 min; dry mixing time 8 r / min.

[0026] In an exemplary embodiment, in step 4), the total mass of the dry mix is ​​200 kg.

[0027] The third aspect of the present application provides a formula powder for patients with maple syrup urine disease prepared by the above method.

[0028] The fourth aspect of the present application provides an infant formula food comprising the above-mentioned formula powder for patients with maple syrup urine disease.

[0029] In an exemplary embodiment, the infant formula is milk powder.

[0030] A fifth aspect of the present application provides a use of the above-mentioned formula powder or infant formula for patients with maple syrup urine disease in infant food for patients with maple syrup urine disease.

[0031] In an exemplary embodiment, the application includes: the formula powder or infant formula food for patients with maple syrup urine disease is used in baby food to promote the growth and development of children with maple syrup urine disease, reduce eczema, diarrhea, abdominal pain, vomiting, nasal congestion, runny nose caused by allergic reactions, and improve the intellectual development of children.

[0032] Compared with the existing related technologies, this application has the following technical effects: The formula powder for maple syrup urine disease patients in this application uses compound amino acids as the protein source, contains medium-chain fatty acid triglycerides MLCT, and removes leucine, isoleucine, and valine. On the one hand, it can ensure the nutritional needs of patients and promote the growth and development of children with maple syrup urine disease; on the other hand, it can effectively control the leucine, isoleucine, and valine content in the patient's body. The formula of this application is scientifically proportioned and nutritionally balanced, which can meet the growth and development of infants with maple syrup urine disease, and can effectively reduce allergic reactions such as eczema, diarrhea, abdominal pain, vomiting, nasal congestion, runny nose, etc. in infants, and improve the intellectual development of children. The preparation method of this product is simple and easy to operate, and can be mass-produced industrially.

[0033] Other features and advantages of the present application will be described in the following description, and partly become apparent from the description, or be understood by implementing the present application. Other advantages of the present application can be realized and obtained by the schemes described in the description. DETAILED DESCRIPTION

[0034] In order to make the purpose, technical solution and advantages of the present application more clear, the embodiments of the present application will be described in detail below. It should be noted that the embodiments and features in the embodiments of the present application can be combined with each other arbitrarily without conflict.

[0035] The present invention is further described in detail below in conjunction with specific examples, but the examples should not be construed as limiting the present invention.

[0036] In the examples and comparative examples of the present application: Protein-free fat powder was purchased from Tianjin Yinhe Weiye Import and Export Co., Ltd.; Corn syrup solids were purchased from Bowling Biotech Co., Ltd.; DHA algae oil powder was purchased from Guangdong Runke Bioengineering Co., Ltd.; Arachidonic acid ARA powder was purchased from Guangdong Runke Bioengineering Co., Ltd.; Multivitamins were purchased from DSM Vitamins (Shanghai) Co., Ltd.; Complex minerals were purchased from DSM Vitamins (Shanghai) Co., Ltd.; Nucleotides were purchased from DSM Vitamins (Shanghai) Co., Ltd.

[0037] Unless otherwise specified, the raw materials in this application are all conventional products on the market.

[0038] Example 1 A special medical formula powder for infants with maple syrup urine disease is prepared from the following components in parts by weight: 15.5 parts of compound amino acids, 20.7 parts of solid corn syrup, 56.6 parts of protein-free fat powder, 0.9 parts of DHA algae oil powder, 1 part of arachidonic acid ARA powder, 0.65 parts of multivitamins, 4.6 parts of multiminerals, and 0.05 parts of nucleotides; the formula powder contains medium-chain fatty acid triglycerides MLCT, but does not contain leucine, isoleucine, and valine; The protein-free fat powder is composed of the following components by weight: 49.8 parts of glucose syrup, 38.5 parts of edible vegetable blending oil, 10 parts of sodium octenyl succinate starch, 1.5 parts of mono- and di-glycerol fatty acid esters, and 0.2 parts of sodium ascorbate; The edible vegetable blended oil is composed of the following by weight: 35 parts of medium-chain fatty acid triglycerides MLCT, 40 parts of sunflower oil, 25 parts of low-erucic acid rapeseed oil, 0.1 parts of mixed tocopherol concentrate, and 0.01 parts of ascorbyl palmitate; The DHA algae oil powder is composed of the following parts by weight: 35.4 parts of glucose syrup, 30 parts of sodium starch octenylsuccinate, 23 parts of docosahexaenoic acid oil, 5.9 parts of sodium ascorbate, 3.3 parts of mono- and diglycerol fatty acid esters, 1 part of sodium citrate, 0.8 parts of tricalcium phosphate, 0.5 parts of phospholipids, and 0.1 parts of ascorbyl palmitate; The ARA powder is composed of the following by weight: 36 parts of glucose syrup, 30 parts of sodium octenyl succinate starch, 23 parts of arachidonic acid oil, 5.3 parts of sodium ascorbate, 3.3 parts of mono- and di-glycerol fatty acid esters, 1 part of sodium citrate, 0.8 parts of tricalcium phosphate, 0.5 parts of phospholipids, and 0.1 parts of ascorbyl palmitate; The nucleotides are composed of the following components in weight percentage: 20.3% adenosine 5'-monophosphate, 30% disodium 5'-cytidylate, 24% disodium 5'-uridylate, 6.9% disodium 5'-guanylate, and 13.8% disodium 5'-inosinate; The composite amino acid comprises: 1.3 parts by weight of glycine, 0.9 parts by weight of L-alanine, 1.5 parts by weight of L-arginine, 1.3 parts by weight of L-aspartic acid, 0.6 parts by weight of L-cysteine, 0.2 parts by weight of L-glutamine, 0.9 parts by weight of L-histidine, 1.6 parts by weight of L-lysine, 0.5 parts by weight of L-methionine, 1.0 parts by weight of L-phenylalanine, 1.6 parts by weight of L-proline, 1.0 parts by weight of L-serine, 1.1 parts by weight of L-threonine, 0.5 parts by weight of L-tryptophan, and 1.0 parts by weight of L-tyrosine; The multivitamin comprises: vitamin A acetate 889705.8825mcg / kg, vitamin D3 19117.647mcg / kg, mixed tocopherol concentrate 6176.4705mg / kg, vitamin K 195588.2355mcg / kg, vitamin B 195588.2355mcg / kg, riboflavin 1220985.294mcg / kg, nicotinamide 9966323.5295mcg / kg, pantothenic acid 5637500mcg / kg, vitamin B 6943529.412mcg / kg, D-biotin 72058.824mcg / kg, folic acid 162352.941mcg / kg, vitamin B 122941.1765 mcg / kg, vitamin C 7352.941mg / kg, choline 176470.588mg / kg, iodine 152205.8825mcg / kg, selenium 29558.8235mcg / kg, L-carnitine 26470.588mg / kg, taurine 59852.941mg / kg, inositol 176470.588mg / kg; The complex minerals include: 100108.6955mcg / kg copper, 1347.8265mg / kg iron, 12804.348mg / kg magnesium, 52413.0435mg / kg manganese, 26196.0435mg / kg sodium, 1347.8265mg / kg zinc, 134782.609mg / kg calcium and 34782.609mg / kg chlorine.

[0039] The preparation steps of the formula powder include: Step 1) Weighing: Weigh the materials according to the formula, and prepare the materials according to the formula after weighing; Step 2) Premixing: Put the composite minerals, ARA powder, nucleotides, DHA algae oil powder, composite vitamins, and composite amino acids into a premixer in sequence, and put in the protein-free fat powder for premixing; the premixing is eccentric mixing, and its parameters are as follows: the premixing time is 20 min, and the premixing speed is 12 r / min; Step 3) Feeding and screening: the premixed materials and other raw materials in step 2) are fed into a dry mixing tank according to the feeding requirements and screened at a mesh size of 16-20; Step 4) Dry mixing: dry mix the sieved materials in step 3) and temporarily store them in a dry mixing tank; in this embodiment, the total mass of the dry mix is ​​200 kg, and the dry mixing parameters are as follows: the dry mixing time is 30 min, and the rotation speed is 8 r / min; Step 5) Metal detection: The materials in the dry mix tank are transported to the metal detector for detection, and after detection without metal impurities, they are filled; Step 6) Filling: Fill the material in step 5) according to the set net content to make the product; Step 7) Packing: Add outer covers to the products, pack them in boxes, and seal them; Step 8) Palletizing: Palletize the products according to the palletizing requirements; Step 9) Warehousing: transport the products to the finished product warehouse for storage; Step 10) Shipping: The product will be shipped after passing the inspection.

[0040] Example 2 A special medical formula powder for infants with maple syrup urine disease is prepared from the following components in parts by weight: 11.3 parts of compound amino acids, 50 parts of protein-free fat powder, 34.2 parts of solid corn syrup, 0.8 parts of DHA algae oil powder, 0.5 parts of arachidonic acid ARA powder, 0.56 parts of multivitamins, 3.6 parts of multiminerals, and 0.04 parts of nucleotides; the formula powder contains medium-chain fatty acid triglycerides MLCT, but does not contain leucine, isoleucine, or valine; The protein-free fat powder is composed of the following components by weight: 57.9 parts of glucose syrup, 32 parts of edible vegetable blending oil, 9 parts of sodium octenyl succinate starch, 1 part of mono- and di-glycerol fatty acid esters, and 0.1 part of sodium ascorbate; The edible vegetable blended oil is composed of the following by weight: 45 parts of medium-chain fatty acid triglycerides MLCT, 50 parts of sunflower oil, 15 parts of low-erucic acid rapeseed oil, 0.1 parts of mixed tocopherol concentrate, and 0.01 parts of ascorbyl palmitate; The DHA algae oil powder is composed of the following parts by weight: 46.78 parts of glucose syrup, 30 parts of sodium starch octenylsuccinate, 220 parts of docosahexaenoic acid oil, 1 part of sodium ascorbate, 0.1 parts of mono- and diglycerol fatty acid esters, 0.02 parts of sodium citrate, 0.1 parts of tricalcium phosphate, 0.05 parts of phospholipids, and 0.05 parts of ascorbyl palmitate; The ARA powder is composed of the following by weight: 46.78 parts of glucose syrup, 30 parts of sodium octenyl succinate starch, 22 parts of arachidonic acid oil, 1 part of sodium ascorbate, 0.1 parts of mono- and di-glycerol fatty acid esters, 0.02 parts of sodium citrate, 0.1 parts of tricalcium phosphate, 0.05 parts of phospholipids, and 0.05 parts of ascorbyl palmitate; The nucleotides are composed of the following components in weight percentage: 20.3% adenosine 5'-monophosphate, 30% disodium 5'-cytidylate, 24% disodium 5'-uridylate, 6.9% disodium 5'-guanylate, and 13.8% disodium 5'-inosinate; The compound amino acid comprises 1.1 parts by weight of glycine, 0.6 parts by weight of L-alanine, 1.2 parts by weight of L-arginine, 1.0 parts by weight of L-aspartic acid, 0.4 parts by weight of L-cysteine, 0.05 parts by weight of L-glutamine, 0.6 parts by weight of L-histidine, 1.3 parts by weight of L-lysine, 0.2 parts by weight of L-methionine, 0.8 parts by weight of L-phenylalanine, 1.3 parts by weight of L-proline, 0.8 parts by weight of L-serine, 0.9 parts by weight of L-threonine, 0.3 parts by weight of L-tryptophan, and 0.8 parts by weight of L-tyrosine.

[0041] The rest is the same as in Example 1.

[0042] Example 3 A special medical formula powder for infants with maple syrup urine disease is prepared from the following components in parts by weight: 16.7 parts of compound amino acids, 60 parts of protein-free fat powder, 14.4 parts of solid corn syrup, 1 part of DHA algae oil powder, 1.5 parts of arachidonic acid ARA powder, 0.74 parts of multivitamins, 5.6 parts of multiminerals, and 0.06 parts of nucleotides; the formula powder contains medium-chain fatty acid triglycerides MLCT, but does not contain leucine, isoleucine, and valine; The protein-free fat powder is composed of the following components by weight: 40.2 parts of glucose syrup, 50 parts of edible vegetable blending oil, 8 parts of sodium octenyl succinate starch, 1 part of mono- and di-glycerol fatty acid esters, and 0.8 parts of sodium ascorbate; The edible vegetable blended oil is composed of the following components by weight: 25 parts of medium-chain fatty acid triglycerides MLCT, 40 parts of sunflower oil, 35 parts of low-erucic acid rapeseed oil, 0.1 parts of mixed tocopherol concentrate, and 0.01 parts of ascorbyl palmitate; The DHA algae oil powder is composed of the following parts by weight: 46.68 parts of glucose syrup, 20 parts of sodium starch octenylsuccinate, 32 parts of docosahexaenoic acid oil, 1 part of sodium ascorbate, 0.1 parts of mono- and di-glycerol fatty acid esters, 0.02 parts of sodium citrate, 0.1 parts of tricalcium phosphate, 0.05 parts of phospholipids, and 0.05 parts of ascorbyl palmitate; The ARA powder is composed of the following by weight: 46.68 parts of glucose syrup, 20 parts of sodium octenyl succinate starch, 32 parts of arachidonic acid oil, 1 part of sodium ascorbate, 0.1 parts of mono- and di-glycerol fatty acid esters, 0.02 parts of sodium citrate, 0.1 parts of tricalcium phosphate, 0.05 parts of phospholipids, and 0.05 parts of ascorbyl palmitate; The amino acids include: 1.4 parts by weight of glycine, 1.0 parts by weight of L-alanine, 1.6 parts by weight of L-arginine, 1.4 parts by weight of L-aspartic acid, 0.6 parts by weight of L-cysteine, 0.2 parts by weight of L-glutamine, 1.0 parts by weight of L-histidine, 1.7 parts by weight of L-lysine, 0.6 parts by weight of L-methionine, 1.2 parts by weight of L-phenylalanine, 1.8 parts by weight of L-proline, 1.2 parts by weight of L-serine, 1.2 parts by weight of L-threonine, 0.6 parts by weight of L-tryptophan, and 1.2 parts by weight of L-tyrosine; The nucleotides are composed of the following components in weight percentage: 20.3% of 5'-adenosine monophosphate, 30% of 5'-cytidylic acid disodium, 24% of 5'-uridylic acid disodium, 6.9% of 5'-guanylic acid disodium, and 13.8% of 5'-inosinic acid disodium.

[0043] The rest is the same as in Example 1.

[0044] Comparative Example 1 Infant formula powder for special medical purposes for maple syrup urine disease is prepared from the following components in parts by weight: 9 parts of compound amino acids, 56.6 parts of protein-free fat powder, 27.2 parts of solid corn syrup, 0.9 parts of DHA algae oil powder, 1 part of arachidonic acid ARA powder, 0.65 parts of multivitamins, 4.6 parts of multiminerals, and 0.05 parts of nucleotides; the formula powder does not contain leucine, isoleucine, and valine; The protein-free fat powder is composed of the following components in parts by weight: 66.15 parts of glucose syrup, 25 parts of edible vegetable blending oil, 8 parts of sodium octenyl succinate starch, 0.8 parts of mono- and di-glycerol fatty acid esters, and 0.05 parts of sodium ascorbate; The edible vegetable blended oil does not contain medium-chain fatty acid MLCT, and is composed of the following by weight: 50 parts of sunflower oil, 50 parts of low-erucic acid rapeseed oil, 0.1 parts of mixed tocopherol concentrate, and 0.01 parts of ascorbyl palmitate; The DHA algae oil powder is composed of the following parts by weight: 58.68 parts of glucose syrup, 20 parts of sodium starch octenylsuccinate, 20 parts of docosahexaenoic acid oil, 1 part of sodium ascorbate, 0.1 parts of mono- and di-glycerol fatty acid esters, 0.02 parts of sodium citrate, 0.1 parts of tricalcium phosphate, 0.05 parts of phospholipids, and 0.05 parts of ascorbyl palmitate; The ARA powder is composed of the following by weight: 58.68 parts of glucose syrup, 20 parts of sodium octenyl succinate starch, 20 parts of arachidonic acid oil, 1 part of sodium ascorbate, 0.1 parts of mono- and di-glycerol fatty acid esters, 0.02 parts of sodium citrate, 0.1 parts of tricalcium phosphate, 0.05 parts of phospholipids, and 0.05 parts of ascorbyl palmitate; The nucleotides are composed of the following components in weight percentage: 20.3% adenosine 5'-monophosphate, 30% disodium 5'-cytidylate, 24% disodium 5'-uridylate, 6.9% disodium 5'-guanylate, and 13.8% disodium 5'-inosinate; The composite amino acid comprises: 0.9 weight part of glycine, 0.5 weight part of L-alanine, 1.0 weight part of L-arginine, 0.9 weight part of L-aspartic acid, 0.3 weight part of L-cysteine, 0.03 weight part of L-glutamine, 0.5 weight part of L-histidine, 1.2 weight parts of L-lysine, 0.1 weight part of L-methionine, 0.6 weight part of L-phenylalanine, 1.2 weight parts of L-proline, 0.5 weight part of L-serine, 0.5 weight part of L-threonine, 0.2 weight part of L-tryptophan, and 0.57 weight part of L-tyrosine.

[0045] The rest is the same as in Example 1.

[0046] Comparative Example 2 Infant formula powder for special medical purposes for maple syrup urine disease is prepared from the following components in parts by weight: 22 parts of compound amino acids, 56.6 parts of protein-free fat powder, 20.7 parts of solid corn syrup, 0.9 parts of DHA algae oil powder, 1 part of arachidonic acid ARA powder, 0.65 parts of multivitamins, 4.6 parts of multiminerals, and 0.05 parts of nucleotides; the formula powder does not contain leucine, isoleucine, and valine; The protein-free fat powder is composed of the following components by weight: 38.15 parts of glucose syrup, 55 parts of edible vegetable blending oil, 6 parts of sodium octenyl succinate starch, 0.8 parts of mono- and di-glycerol fatty acid esters, and 0.05 parts of sodium ascorbate; The edible vegetable blended oil is composed of the following components by weight: 50 parts of medium-chain fatty acid triglycerides MLCT, 40 parts of sunflower oil, 10 parts of low-erucic acid rapeseed oil, 0.1 parts of mixed tocopherol concentrate, and 0.01 parts of ascorbyl palmitate; The DHA algae oil powder is composed of the following parts by weight: 9.68 parts of glucose syrup, 55 parts of sodium starch octenylsuccinate, 34 parts of docosahexaenoic acid oil, 1 part of sodium ascorbate, 0.1 parts of mono- and di-glycerol fatty acid esters, 0.02 parts of sodium citrate, 0.1 parts of tricalcium phosphate, 0.05 parts of phospholipids, and 0.05 parts of ascorbyl palmitate; The ARA powder is composed of the following by weight: 9.68 parts of glucose syrup, 55 parts of sodium octenyl succinate starch, 34 parts of arachidonic acid oil, 1 part of sodium ascorbate, 0.1 parts of mono- and di-glycerol fatty acid esters, 0.02 parts of sodium citrate, 0.1 parts of tricalcium phosphate, 0.05 parts of phospholipids, and 0.05 parts of ascorbyl palmitate; The nucleotides are composed of the following components in weight percentage: 20.3% adenosine 5'-monophosphate, 30% disodium 5'-cytidylate, 24% disodium 5'-uridylate, 6.9% disodium 5'-guanylate, and 13.8% disodium 5'-inosinate; The composite amino acid comprises: 1.9 parts by weight of glycine, 1.5 parts by weight of L-alanine, 1.9 parts by weight of L-arginine, 1.6 parts by weight of L-aspartic acid, 0.8 parts by weight of L-cysteine, 0.5 parts by weight of L-glutamine, 1.5 parts by weight of L-histidine, 1.8 parts by weight of L-lysine, 1.6 parts by weight of L-methionine, 1.8 parts by weight of L-phenylalanine, 1.9 parts by weight of L-proline, 1.5 parts by weight of L-serine, 1.3 parts by weight of L-threonine, 0.8 parts by weight of L-tryptophan, and 1.6 parts by weight of L-tyrosine.

[0047] The rest is the same as in Example 1.

[0048] Comparative Example 3 Infant formula powder for special medical purposes for maple syrup urine disease is prepared from the following components in parts by weight: 30 parts of compound amino acids, 56.6 parts of protein-free fat powder, 12.7 parts of solid corn syrup, 0.9 parts of DHA algae oil powder, 1 part of arachidonic acid ARA powder, 0.65 parts of multivitamins, 4.6 parts of multiminerals, and 0.05 parts of nucleotides; the formula powder does not contain leucine, isoleucine, and valine; The protein-free fat powder is composed of the following components by weight: 49.15 parts of glucose syrup, 28 parts of edible vegetable blending oil, 22 parts of sodium octenyl succinate starch, 0.8 parts of mono- and di-glycerol fatty acid esters, and 0.05 parts of sodium ascorbate; The edible vegetable blended oil is composed of the following components by weight: 10 parts of medium-chain fatty acid triglycerides MLCT, 70 parts of sunflower oil, 20 parts of low-erucic acid rapeseed oil, 0.1 parts of mixed tocopherol concentrate, and 0.01 parts of ascorbyl palmitate; The DHA algae oil powder is composed of the following parts by weight: 25.68 parts of glucose syrup, 55 parts of sodium starch octenylsuccinate, 18 parts of docosahexaenoic acid oil, 1 part of sodium ascorbate, 0.1 parts of mono- and di-glycerol fatty acid esters, 0.02 parts of sodium citrate, 0.1 parts of tricalcium phosphate, 0.05 parts of phospholipids, and 0.05 parts of ascorbyl palmitate; The ARA powder is composed of the following by weight: 25.68 parts of glucose syrup, 55 parts of sodium octenyl succinate starch, 18 parts of arachidonic acid oil, 1 part of sodium ascorbate, 0.1 parts of mono- and di-glycerol fatty acid esters, 0.02 parts of sodium citrate, 0.1 parts of tricalcium phosphate, 0.05 parts of phospholipids, and 0.05 parts of ascorbyl palmitate; The nucleotides are composed of the following components in weight percentage: 20.3% adenosine 5'-monophosphate, 30% disodium 5'-cytidylate, 24% disodium 5'-uridylate, 6.9% disodium 5'-guanylate, and 13.8% disodium 5'-inosinate; The composite amino acid comprises: 2.9 parts by weight of glycine, 2.5 parts by weight of L-alanine, 1.9 parts by weight of L-arginine, 2.6 parts by weight of L-aspartic acid, 1.8 parts by weight of L-cysteine, 1.5 parts by weight of L-glutamine, 1.5 parts by weight of L-histidine, 1.8 parts by weight of L-lysine, 2.6 parts by weight of L-methionine, 1.8 parts by weight of L-phenylalanine, 1.9 parts by weight of L-proline, 1.5 parts by weight of L-serine, 2.3 parts by weight of L-threonine, 1.8 parts by weight of L-tryptophan, and 1.6 parts by weight of L-tyrosine.

[0049] The rest is the same as in Example 1.

[0050] Example 4 Detection of Molecular Weight Distribution Specific method: The sample is dissolved in the mobile phase and measured by a high performance liquid chromatography system equipped with a gel permeation chromatography column and a UV detector; the sample components are separated according to their relative molecular weights, and peptides and proteins are measured at 214nm, 220nm or 280nm. Through chromatographic data processing, the different relative molecular weights and distribution ranges are calculated.

[0051] Reagents and standards: Water: Secondary water; Sodium dihydrogen phosphate dihydrate (NaH2PO4): analytical grade; Ammonium chloride (NH4Cl): analytical grade; Acetonitrile (CH3CN): chromatographic grade; Trifluoroacetic acid (TFA): chromatographic grade; Cytochrome C: MW12384; Aprotinin: MW6512; Bacillus enzyme: MW1423; Tyrosine-Tyrosine-Arg: MW451; Tyrosine-tyrosine-tyrosine: MW189; Instruments and equipment: High performance liquid chromatograph; with UV detector; electronic balance: sensitivity 0.1 mg and 0.01 mg; Chromatographic conditions: TSKgel G2000 SWXL300mmx7.8mm chromatographic column or equivalent; Mobile phase: 75% A (0.0375 M sodium dihydrogen phosphate dihydrate + 0.375 M ammonium chloride solution): 25% B (acetonitrile), containing 0.1% trifluoroacetic acid; Detection wavelength: 214 nm; Flow rate: 0.5 mL / min; Column temperature: 30℃.

[0052] Preparation of relative molecular mass calibration curve: Prepare 5 standards with different relative molecular masses with mobile phase, filter through microporous membrane and measure to obtain chromatogram of standard. Curve fitting is performed with relative molecular mass and corresponding retention time to obtain relative molecular mass calibration curve and equation.

[0053] Sample treatment: Weigh an appropriate amount of sample into a volumetric flask, add mobile phase to dissolve, mix well and filter, and measure the filtrate after filtering through a microporous filter membrane.

[0054] Calculation of relative molecular mass distribution: Substitute the chromatographic data retention time of the sample into the calibration curve for calculation to obtain the relative molecular mass of the sample and its distribution range.

[0055] The distribution of different relative molecular mass intervals was calculated using the peak area normalization method, and the calculation was performed as follows:

[0056] Where: X-mass fraction of protein hydrolysate in a certain relative molecular mass range in the sample to the total protein hydrolysate, %; A is the peak area of ​​protein hydrolysate in a certain relative molecular mass range; A The calculation result of the sum of the peak areas in each interval shall be retained to one decimal place.

[0057] Repeatability: The absolute difference between two independent measurement results obtained under repeatability conditions shall not exceed 15% of the arithmetic mean of the two measurement results.

[0058] The molecular weight distribution of the formula powder in the above examples and comparative examples was tested using the above testing method, and the results are shown in Table 1.

[0059] Table 1 Molecular weight distribution

[0060] The results show that in the molecular weight distribution of the formula powders of Examples 1-3, 94.4%-95.2% accounted for ≤500Da, 99.4%-99.6% accounted for ≤1000 Da, and >1000Da accounted for only 0.4-0.6%. From the test results of molecular weight distribution, the lower the content of <1000Da, the more effectively it can reduce the probability of allergic reactions. In the formula powders of Comparative Examples 1-3, the molecular weight >1000Da accounted for 3.2%-4.1%, and the probability of causing allergic reactions was relatively higher.

[0061] Example 5 Allergy Protein Detection The allergenic protein content of the formula powder in the above-mentioned embodiments and comparative examples was analyzed, and the detection was carried out by ELISA sandwich enzyme-linked immunosorbent assay to quantitatively detect casein, soy protein, whole milk protein, β-lactoglobulin, and β-lactoglobulin fragments in the formula powder, and the results are shown in Table 2. Among them, the detection limit of casein, soy protein, and whole milk protein allergens is 2.5 mg / kg; the detection limit of β-lactoglobulin allergen is 0.167 mg / kg; and the detection limit of β-lactoglobulin fragment allergen is 5 mg / kg.

[0062] Table 2 Allergic protein content

[0063] The results showed that the protein allergen content in the formula powders of Examples 1-3 was lower than the detection limit, and the protein allergen content in the formula powders of Comparative Examples 1-3 was higher than the detection limit, indicating that the formula powders in the examples of the present application can play a role in reducing the risk of food protein allergies.

[0064] Example 6 Analysis of the effects of formula powder for maple syrup urine disease patients on growth, intellectual development, and allergic symptoms in children with maple syrup urine disease.

[0065] Test children: 21 children with maple syrup urine disease who were screened and diagnosed were selected. All infants were tested for intelligence level using the Wechsler Children's Intelligence Scale (C-WYCSI). The scores of the infants for language, arithmetic, patterns, etc. were combined as the judgment results. The test evaluation criteria are in line with the diagnostic criteria based on the latest US MSUD Nutrition Management Guidelines and the 2023 Neonatal Milk Protein Allergy Diagnosis and Management Expert Consensus. Among the children in this example, there were 10 males and 11 females; the age of onset was 6 to 9 days, and the average age of diagnosis was 13 days.

[0066] Treatment method: The children were randomly divided into 7 groups, one group took the overseas food 1 normally, three groups took the formula powders of Examples 1 to 3 of the present invention, and the other three groups took the formula powders of Comparative Examples 1-3, and the feeding time was 3 weeks. Medical staff conducted telephone follow-up on growth and development, allergic symptoms, and intellectual development after 1 week, 2 weeks, and 3 weeks of feeding. The growth and development of the children after 3 weeks of consumption were recorded and compared.

[0067] The growth and development after treatment were recorded and compared. Evaluation indicators: height, weight, head circumference, statistical results are shown in Table 3.

[0068] The changes in intelligence development were evaluated by recording and comparing the children's data before and after 3 weeks of consumption. The statistical results of intelligence analysis before and after treatment are shown in Table 4. Evaluation indicators: Observe and record the effect of nutritional treatment on the children. Treatment results were markedly effective: the symptoms of the children improved significantly within one week after treatment; effective: the symptoms of the children improved within two weeks after treatment; ineffective: the symptoms of the children did not change significantly within three weeks after treatment.

[0069] The allergic symptoms of the children are shown in Table 5. The number of allergic reactions such as eczema, diarrhea, abdominal pain, vomiting, nasal congestion, and runny nose were recorded.

[0070] Statistical method: Each variable is expressed as x±s. The t test was used, and P<0.05 was considered to be statistically significant.

[0071] Table 3 Edible growth and development

[0072] Note: The same letters under each indicator indicate no significant difference at the 5% significance level, and different letters indicate significant difference at the 5% significance level.

[0073] Table 4 Observation on the effect of improving the intelligence of children

[0074] Table 5 Statistics of allergic symptoms in children with maple syrup urine disease (times / three weeks; average number of allergies in each group)

[0075] According to Table 3-5, the test results of infants taking the products of Examples 1-3 in growth and development, allergic symptoms and intellectual performance are the best. The main reasons are: compared with the formula of Haitao Food 1 and the formula of Comparative Examples 1-3, the nutritional ingredients in the formula of Examples 1-3 are closer to the required proportion of the human body; α-casein and β-lactoglobulin are the most common causes of milk protein allergic reactions. This application uses a reasonably proportioned compound amino acid and removes leucine, isoleucine, and valine as protein sources, which effectively reduces the allergic reactions such as eczema, diarrhea, abdominal pain, vomiting, nasal congestion, and runny nose shown by infants. The intellectual performance of infants taking the formula powder of Examples 1-3 is also better than that of ordinary formulas, indicating that the formula of this application can effectively reduce the occurrence of allergic reactions while controlling the disease, improve the intellectual development of children, and is conducive to growth and development. Therefore, children have good clinical effects after taking the formula of this application.

[0076] The detailed description of the formula powder for patients with maple syrup urine disease with reference to the above-mentioned embodiments is illustrative rather than restrictive, and several embodiments may be listed according to the limited scope. Therefore, changes and modifications without departing from the overall concept of the present invention should fall within the scope of protection of the present invention.

[0077] Although the embodiments of the present application have been shown and described above, it can be understood that the above embodiments are exemplary and cannot be understood as limitations on the present application. Ordinary technicians in this field can change, modify, replace and modify the above embodiments within the scope of the present application.

Claims

1. A formula powder for patients with maple syrup urine disease, comprising the following components: 10-20 parts by weight of compound amino acids, 50-60 parts by weight of protein-free fat powder, Solid corn syrup 11.1-35.5 parts by weight, DHA algae oil powder 0.8-1.0 weight portion, ARA powder 0.5-1.5 parts by weight, 0.56-0.74 parts by weight of multivitamins, 3.6-5.6 parts by weight of composite minerals, Nucleotides 0.04-0.06 parts by weight; The formula powder for patients with maple syrup urine disease contains medium- and long-chain triglycerides MLCT, but does not contain leucine, isoleucine, or valine.

2. The formula powder for patients with maple syrup urine disease according to claim 1, wherein: The protein-free fat powder is a fat powder produced using raw materials and auxiliary materials that do not contain protein. Optionally, the protein content of the protein-free fat powder is ≤0.5g / 100g, and the fat content is ≥30g / 100g; Optionally, the protein-free fat powder is composed of the following by weight: 40-60 parts of glucose syrup, 30-50 parts of edible vegetable blending oil, 8-20 parts of sodium octenyl succinate starch, 1-2 parts of mono- and di-glycerol fatty acid esters, and 0.1-0.8 parts of sodium ascorbate; Optionally, the edible vegetable blended oil is composed of the following by weight: 25-45 parts of medium-chain fatty acid triglycerides MLCT, 40-50 parts of sunflower oil, 15-35 parts of low-erucic acid rapeseed oil, 0.01-0.4 parts of mixed tocopherol concentrate, and 0.001-0.1 parts of ascorbyl palmitate.

3. The formula powder for patients with maple syrup urine disease according to claim 1 or 2, wherein: The DHA algae oil powder is composed of the following parts by weight: 20-50 parts of glucose syrup, 20-50 parts of sodium starch octenylsuccinate, 22-32 parts of docosahexaenoic acid oil, 1-6 parts of sodium ascorbate, 0.1-4 parts of mono- and diglycerol fatty acid esters, 0.02-3 parts of sodium citrate, 0.1-2 parts of tricalcium phosphate, 0.05-2 parts of phospholipids, and 0.05-2 parts of ascorbyl palmitate; and / or The ARA powder is composed of the following components by weight: 20-50 parts of glucose syrup, 20-50 parts of sodium starch octenylsuccinate, 22-32 parts of arachidonic acid oil, 1-6 parts of sodium ascorbate, 0.1-4 parts of mono- and diglycerol fatty acid esters, 0.02-3 parts of sodium citrate, 0.1-2 parts of tricalcium phosphate, 0.05-2 parts of phospholipids, and 0.05-2 parts of ascorbyl palmitate.

4. The formula powder for patients with maple syrup urine disease according to claim 1 or 2, wherein: The nucleotides are composed of the following components by mass percentage: 15%-25% adenosine 5'-monophosphate, 20%-40% disodium 5'-cytidylate, 20%-30% disodium 5'-uridylate, 6%-10% disodium 5'-guanylate, and 10%-20% disodium 5'-inosinate; Optionally, the nucleotides are composed of the following components by mass percentage: 20.3% adenosine 5'-monophosphate, 30% disodium 5'-cytidylate, 24% disodium 5'-uridylate, 6.9% disodium 5'-guanylate, and 13.8% disodium 5'-inosinate.

5. The formula powder for patients with maple syrup urine disease according to claim 1 or 2, wherein: The composite amino acids, in parts by weight, comprise: 1.1-1.4 parts of glycine, 0.6-1.0 parts of L-alanine, 1.2-1.6 parts of L-arginine, 1.0-1.4 parts of L-aspartic acid, 0.4-0.6 parts of L-cysteine, 0.05-0.2 parts of glutamine, 0.6-1.0 parts of L-histidine, 1.3-1.7 parts of L-lysine, 0.2-0.6 parts of L-methionine, 0.8-1.2 parts of L-phenylalanine, 1.3-1.8 parts of L-proline, 0.8-1.2 parts of L-serine, 0.9-1.2 parts of L-threonine, 0.3-0.6 parts of L-tryptophan, 0.8-1.2 parts of L-tyrosine; and / or The multivitamin comprises: vitamin A acetate 756250.000-1023161.765 mcg / kg, vitamin D3 16250-21985.294 mcg / kg, mixed tocopherol concentrate 5250.000-7102.941 mg / kg, vitamin K1 81250-109926.471 mcg / kg, vitamin B1 81250-109926.471 mcg / kg, riboflavin 1308375.000-1133595.588 mcg / kg, nicotinamide 8471375.000-11461272.059 mcg / kg, pantothenic acid 4791875.000-6483125.000 mcg / kg, vitamin B6 802000.0000-1085058.824 mcg / kg, D-biotin 30625.000-41433.824 mcg / kg, folic acid 138000.000-186705.882 mcg / kg, vitamin B 12 2500.000-3382.353mcg / kg, vitamin C 6617.647-8088.235 mg / kg, choline 158823.529-194117.647mg / kg, iodine 129375.000-175036.765mcg / kg, selenium 25125.000-33992.647mcg / kg, L-carnitine 23823.529-29117.647mg / kg, taurine 53867.647-65838.235mg / kg, inositol 158823.529-194117.647mg / kg; and / or The complex minerals include: copper 90097.826-110119.565mcg / kg, iron 1213.044-1482.609mg / kg, magnesium 11523.913-14084.783mg / kg, manganese 44551.087-60275.000mg / kg, sodium 23576.087-28815.217mg / kg, zinc 1213.044-1482.609mg / kg, calcium 121304.348-148260.870mg / kg and chlorine 31304.348-38260.870mg / kg.

6. The formula powder for patients with maple syrup urine disease according to claim 1 or 2, wherein: The molecular weight distribution of the formula powder for patients with maple syrup urine disease is 94.4%-95.2% for ≤500Da, 99.4%-99.6% for ≤1000Da, and 0.4%-0.6% for >1000Da.

7. A method for preparing a formula powder for patients with maple syrup urine disease according to any one of claims 1 to 6, comprising the following steps: Step 1) Weighing: Weigh the materials according to the formula, and prepare the materials according to the formula after weighing; Step 2) Premixing: Put the composite minerals, ARA powder, nucleotides, DHA algae oil powder, composite vitamins, and composite amino acids into a premixer in sequence, and then put in the protein-free fat powder for premixing; Step 3) Feeding and screening: the premixed materials and the remaining raw materials are fed into a dry mixing tank according to the feeding requirements for screening; optionally, the mesh size of the screening process is 16-20 meshes; Step 4) dry mixing: dry mixing the materials after sieving; Step 5) Metal detection: The material in the dry mixing tank is transported to a metal detector for detection, and after detection of no metal impurities, it is filled; Step 6) Filling: Filling the material according to the set net content to produce the product; Step 7) Packing: adding an outer cover to the product, packing it in a box, and sealing it; Step 8) Palletizing: palletizing the products according to palletizing requirements; Step 9) Warehousing: transporting the product to the finished product warehouse for warehousing; Step 10) Shipping: The product will be shipped after passing the inspection.

8. A formula powder for patients with maple syrup urine disease prepared by the method of claim 7.

9. An infant formula food comprising the formula powder for maple syrup urine disease patients according to any one of claims 1 to 6 and 8.

10. The infant formula food according to claim 9, wherein: The infant formula food is milk powder.

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