WO2020010896A1 - 一种用于苯丙酮尿症患者的配方粉及其制备方法 - Google Patents

一种用于苯丙酮尿症患者的配方粉及其制备方法 Download PDF

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WO2020010896A1
WO2020010896A1 PCT/CN2019/084420 CN2019084420W WO2020010896A1 WO 2020010896 A1 WO2020010896 A1 WO 2020010896A1 CN 2019084420 W CN2019084420 W CN 2019084420W WO 2020010896 A1 WO2020010896 A1 WO 2020010896A1
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acid
vitamin
formula powder
mass percentage
materials
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PCT/CN2019/084420
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English (en)
French (fr)
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杨国民
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宁波特壹食品有限公司
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    • A—HUMAN NECESSITIES
    • A23—FOODS OR FOODSTUFFS; TREATMENT THEREOF, NOT COVERED BY OTHER CLASSES
    • A23L—FOODS, FOODSTUFFS, OR NON-ALCOHOLIC BEVERAGES, NOT COVERED BY SUBCLASSES A21D OR A23B-A23J; THEIR PREPARATION OR TREATMENT, e.g. COOKING, MODIFICATION OF NUTRITIVE QUALITIES, PHYSICAL TREATMENT; PRESERVATION OF FOODS OR FOODSTUFFS, IN GENERAL
    • A23L33/00—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof
    • A23L33/10—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof using additives
    • A23L33/115—Fatty acids or derivatives thereof; Fats or oils
    • A23L33/12—Fatty acids or derivatives thereof
    • A—HUMAN NECESSITIES
    • A23—FOODS OR FOODSTUFFS; TREATMENT THEREOF, NOT COVERED BY OTHER CLASSES
    • A23L—FOODS, FOODSTUFFS, OR NON-ALCOHOLIC BEVERAGES, NOT COVERED BY SUBCLASSES A21D OR A23B-A23J; THEIR PREPARATION OR TREATMENT, e.g. COOKING, MODIFICATION OF NUTRITIVE QUALITIES, PHYSICAL TREATMENT; PRESERVATION OF FOODS OR FOODSTUFFS, IN GENERAL
    • A23L33/00—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof
    • A—HUMAN NECESSITIES
    • A23—FOODS OR FOODSTUFFS; TREATMENT THEREOF, NOT COVERED BY OTHER CLASSES
    • A23L—FOODS, FOODSTUFFS, OR NON-ALCOHOLIC BEVERAGES, NOT COVERED BY SUBCLASSES A21D OR A23B-A23J; THEIR PREPARATION OR TREATMENT, e.g. COOKING, MODIFICATION OF NUTRITIVE QUALITIES, PHYSICAL TREATMENT; PRESERVATION OF FOODS OR FOODSTUFFS, IN GENERAL
    • A23L33/00—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof
    • A23L33/10—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof using additives
    • A—HUMAN NECESSITIES
    • A23—FOODS OR FOODSTUFFS; TREATMENT THEREOF, NOT COVERED BY OTHER CLASSES
    • A23L—FOODS, FOODSTUFFS, OR NON-ALCOHOLIC BEVERAGES, NOT COVERED BY SUBCLASSES A21D OR A23B-A23J; THEIR PREPARATION OR TREATMENT, e.g. COOKING, MODIFICATION OF NUTRITIVE QUALITIES, PHYSICAL TREATMENT; PRESERVATION OF FOODS OR FOODSTUFFS, IN GENERAL
    • A23L33/00—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof
    • A23L33/10—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof using additives
    • A23L33/115—Fatty acids or derivatives thereof; Fats or oils
    • A—HUMAN NECESSITIES
    • A23—FOODS OR FOODSTUFFS; TREATMENT THEREOF, NOT COVERED BY OTHER CLASSES
    • A23L—FOODS, FOODSTUFFS, OR NON-ALCOHOLIC BEVERAGES, NOT COVERED BY SUBCLASSES A21D OR A23B-A23J; THEIR PREPARATION OR TREATMENT, e.g. COOKING, MODIFICATION OF NUTRITIVE QUALITIES, PHYSICAL TREATMENT; PRESERVATION OF FOODS OR FOODSTUFFS, IN GENERAL
    • A23L33/00—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof
    • A23L33/10—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof using additives
    • A23L33/125—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof using additives containing carbohydrate syrups; containing sugars; containing sugar alcohols; containing starch hydrolysates
    • A—HUMAN NECESSITIES
    • A23—FOODS OR FOODSTUFFS; TREATMENT THEREOF, NOT COVERED BY OTHER CLASSES
    • A23L—FOODS, FOODSTUFFS, OR NON-ALCOHOLIC BEVERAGES, NOT COVERED BY SUBCLASSES A21D OR A23B-A23J; THEIR PREPARATION OR TREATMENT, e.g. COOKING, MODIFICATION OF NUTRITIVE QUALITIES, PHYSICAL TREATMENT; PRESERVATION OF FOODS OR FOODSTUFFS, IN GENERAL
    • A23L33/00—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof
    • A23L33/10—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof using additives
    • A23L33/15—Vitamins
    • A—HUMAN NECESSITIES
    • A23—FOODS OR FOODSTUFFS; TREATMENT THEREOF, NOT COVERED BY OTHER CLASSES
    • A23L—FOODS, FOODSTUFFS, OR NON-ALCOHOLIC BEVERAGES, NOT COVERED BY SUBCLASSES A21D OR A23B-A23J; THEIR PREPARATION OR TREATMENT, e.g. COOKING, MODIFICATION OF NUTRITIVE QUALITIES, PHYSICAL TREATMENT; PRESERVATION OF FOODS OR FOODSTUFFS, IN GENERAL
    • A23L33/00—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof
    • A23L33/10—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof using additives
    • A23L33/15—Vitamins
    • A23L33/155—Vitamins A or D
    • A—HUMAN NECESSITIES
    • A23—FOODS OR FOODSTUFFS; TREATMENT THEREOF, NOT COVERED BY OTHER CLASSES
    • A23L—FOODS, FOODSTUFFS, OR NON-ALCOHOLIC BEVERAGES, NOT COVERED BY SUBCLASSES A21D OR A23B-A23J; THEIR PREPARATION OR TREATMENT, e.g. COOKING, MODIFICATION OF NUTRITIVE QUALITIES, PHYSICAL TREATMENT; PRESERVATION OF FOODS OR FOODSTUFFS, IN GENERAL
    • A23L33/00—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof
    • A23L33/10—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof using additives
    • A23L33/16—Inorganic salts, minerals or trace elements
    • A—HUMAN NECESSITIES
    • A23—FOODS OR FOODSTUFFS; TREATMENT THEREOF, NOT COVERED BY OTHER CLASSES
    • A23L—FOODS, FOODSTUFFS, OR NON-ALCOHOLIC BEVERAGES, NOT COVERED BY SUBCLASSES A21D OR A23B-A23J; THEIR PREPARATION OR TREATMENT, e.g. COOKING, MODIFICATION OF NUTRITIVE QUALITIES, PHYSICAL TREATMENT; PRESERVATION OF FOODS OR FOODSTUFFS, IN GENERAL
    • A23L33/00—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof
    • A23L33/10—Modifying nutritive qualities of foods; Dietetic products; Preparation or treatment thereof using additives
    • A23L33/17—Amino acids, peptides or proteins
    • A23L33/175—Amino acids
    • A—HUMAN NECESSITIES
    • A23—FOODS OR FOODSTUFFS; TREATMENT THEREOF, NOT COVERED BY OTHER CLASSES
    • A23V—INDEXING SCHEME RELATING TO FOODS, FOODSTUFFS OR NON-ALCOHOLIC BEVERAGES AND LACTIC OR PROPIONIC ACID BACTERIA USED IN FOODSTUFFS OR FOOD PREPARATION
    • A23V2002/00—Food compositions, function of food ingredients or processes for food or foodstuffs

Definitions

  • the invention relates to a formula powder and a preparation method thereof, in particular to a formula powder used in patients with phenylketonuria and a preparation method thereof.
  • Phenylketonuria is a common disorder of amino acid metabolism. Due to enzyme defects in the phenylalanine (PA) metabolic pathway, phenylalanine cannot be converted into tyrosine, leading to benzene Alanine and its keto acids accumulate and are excreted in large amounts from the urine.
  • PA phenylalanine
  • Phenylketonuria is more common in hereditary disorders of amino acid metabolism, with an incidence of 1 in 10,000. Its inheritance is autosomal recessive. The clinical manifestations are uneven, and the main clinical features are mental retardation, neuropsychiatric symptoms, eczema, skin scratch signs, pigment loss, and rat odor, and abnormal EEG.
  • phenylketonuria can be diagnosed and treated early, the aforementioned clinical manifestations may not occur, intelligence is normal, and EEG abnormalities can be recovered.
  • phenylalanine is contained in general food, milk powder and breast milk, in order to cope with phenylketonuria, it is necessary to control the diet of patients and limit the intake of phenylalanine.
  • the purpose of the present invention is to provide a formula powder for patients with phenylketonuria and a preparation method thereof. Aiming at the shortcomings of the prior art, it does not produce the accumulation of phenylalanine and its keto acids, and also guarantees the amino acid intake of patients. To maintain the body's normal metabolism of amino acids.
  • the present invention provides a formula powder for phenylketonuria patients, which is characterized in that it is prepared from the following components in mass percentage:
  • Vegetable oil fat powder 60-90%, complex amino acids 8-20%, fructooligosaccharide (Fos) 0-6.45%, galactooligosaccharide (Gos) 0-6.45%, nucleotides 0.012-0.058%, complex vitamins 0.06- 0.41% and composite minerals 1.059-4.717%, the formula powder does not contain phenylalanine.
  • the composite amino acid is composed of the following amino acids: L-alanine; L-arginine and / or L-arginine hydrochloride, L-aspartic acid and / or L- Magnesium aspartate; one or more combinations of L-cystine, L-cysteine, and L-cysteine hydrochloride; L-glutamic acid, L-potassium potassium, L- One or more combinations of glutamine; glycine; L-histidine and / or L-histidine hydrochloride; L-isoleucine, L-leucine; L-lysine hydrochloride and / Or L-lysine acetate; L-methionine and / or L-acetyl-methionine; L-proline; L-serine; L-threonine; L-tryptophan; L-tyrosine; L-valine, and L-tyrosine constitutes 7-11% by weight of the following amino acids: L-a
  • the composite amino acid is composed of the following mass percentage components: L-alanine 0.2-0.4%, L-arginine 0.2-0.5%, and L-aspartic acid 0.7- 1.0%, L-cystine 0.15-0.35%, L-glutamine 1.6-2.0%, glycine 0.1-0.3%, L-histidine 0.3-0.5%, L-isoleucine 0.8-1.1%, L-leucine 1.4-1.6%, L-lysine hydrochloride 0.8-1.2%, L-methionine 0.2-0.4%, L-proline 0.8-1.1%, L-serine 0.3-0.6%, L-threonine 0.5-0.7%, L-tryptophan 0.2-0.4%, L-tyrosine 0.8-1.2%, and L-valine 0.8-1.2%.
  • the vegetable oil fat powder is made of solid glucose syrup, pregelatinized starch, fatty acid, and emulsifier by spray drying.
  • Vegetable fat powder contains fats and carbohydrates to provide energy for the body.
  • the nucleotide is composed of adenylic acid, uridine, guanylic acid, cytidylic acid, and inosine acid, and its mass ratio is 2.9: 3.5: 1.0: 5.1: 2.0.
  • the multivitamin is composed of the following mass percentage components: Vitamin A 0.00029-0.0009%, Vitamin C 0.0525-0.357%, Vitamin D 0.0000525-0.0000126%, Vitamin E0.00252 -0.0252%, Vitamin K10.000021-0.0001365%, Vitamin B10.000294-0.001512%, Vitamin B20.000399-0.002499%, Vitamin B60.0001785-0.000945%, Vitamin B120.000000525-0.00000756%, Niacin 0.00147-0.00756% , Folic acid 0.000525-0.000252%, pantothenic acid 0.002-0.01%, biotin 0.000084-0.0000504%, inositol 0.021-0.1995%, choline 0.0357-0.252%, and taurine 0-0.063%. Multivitamins provide patients with the vitamins they need to participate in various biochemical reactions in the body.
  • the composite mineral is composed of the following mass percentage components: sodium 0.105-0.294%, chlorine 0.252-0.798%, potassium 0.294-0.903%, calcium 0.252-0.735%, phosphorus 0.126- 0.504%, iron 0.0021-0.00756%, zinc 0.00252-0.00756%, manganese 0.000252-0.000504%, copper 0.0001785-0.000609%, magnesium 0.0252-0.0756%, selenium 0.00001-0.0000399%, and iodine 0.000525-0.000294%.
  • Minerals are important constituents of the body's tissues; maintain the body's acid-base balance and osmotic pressure of tissue cells; maintain neuromuscular excitability and cell membrane permeability.
  • the formula powder further comprises 0.12-0.16% by weight of a compound unsaturated fatty acid, the compound unsaturated fatty acid is composed of DHA and ARA, and a weight ratio of DHA and ARA is 1: 1.8.
  • Formulated powder containing compound unsaturated fatty acids can be used for children aged 0-10 years. Long chain unsaturated fatty acids can promote brain development and improve cognitive ability; promote physical development and ensure healthy physique; ensure vision development and reduce vision defects; Take care of premature babies and improve their health; support immune development and improve respiratory health. Formulated powders for patients over 10 years may not need to contain the complex unsaturated fatty acids.
  • the formula powder further comprises 0.0-0.5% of the essence by weight.
  • the flavor is one of chocolate, banana, strawberry, orange, grapefruit and other fruit flavors. The addition of flavor can provide a unique flavor to the formula powder of the present invention to suit different patients.
  • the present invention provides a method for preparing a formula powder for patients with phenylketonuria, which is characterized in that it includes the following steps:
  • Feeding Transfer the weighed raw and auxiliary materials to the mixer through the feeding station; pay attention to placing relatively low-quality raw materials such as vitamins and minerals in the middle of the bulk raw materials to prevent small materials from sticking. Affect uniformity on the surface of the equipment;
  • the mixed materials will be transferred to the powder storage for temporary storage, and the inspection department will take samples for process product inspection;
  • Packaging product packaging using automatic packaging machines
  • the method for preparing the formula powder is characterized in that, in the mixing and stirring step, a three-dimensional mixer is recommended to determine an appropriate mixing time to ensure uniformity of various indexes of the product.
  • the formula powder for phenylketonuria patients and the preparation method thereof have a basic composition of fat, carbohydrates, amino acids, vitamins, minerals, etc., and are scientifically matched, which can satisfy infants.
  • the formula powder for phenylketonuria patients of the present invention is prepared from the following mass percentage components: vegetable oil fat powder 82.26%, compound amino acid 14%, nucleotide 0.03%, docosahexaenoic acid (DHA ) 0.05%, eicosatetraenoic acid (ARA) 0.09%, flavor 0.1%, complex vitamin 0.57% and complex mineral 2.9%, the formula powder does not contain phenylalanine.
  • the composite amino acid is composed of the following mass percentage components: L-alanine 0.2-0.4%, L-arginine 0.2-0.5%, L-aspartic acid 0.7-1.0%, and L-cystine 0.15 -0.35%, L-glutamine 1.6-2.0%, glycine 0.1-0.3%, L-histidine 0.3-0.5%, L-isoleucine 0.8-1.1%, L-leucine 1.4-1.6% , L-lysine hydrochloride 0.8-1.2%, L-methionine 0.2-0.4%, L-proline 0.8-1.1%, L-serine 0.3-0.6%, L-threonine 0.5-0.7% , L-tryptophan 0.2-0.4%, L-tyrosine 0.8-1.2%, and L-valine 0.8-1.2%.
  • the vegetable oil fat powder is made of solid glucose syrup, pre-gelatinized starch, fatty acid and emulsifier by spray drying.
  • the nucleotide is composed of adenylic acid, uridine, guanylic acid, cytidylic acid, and inosine acid, and its mass ratio is 2.9: 3.5: 1.0: 5.1: 2.0.
  • the compound vitamin is composed of the following mass percentage components: vitamin A 0.00029-0.0009%, vitamin C 0.0525-0.357%, vitamin D 0.0000525-0.0000126%, vitamin E 0.00252-0.0252%, vitamin K10.000021- 0.0001365%, Vitamin B10.000294-0.001512%, Vitamin B20.000399-0.002499%, Vitamin B60.0001785-0.000945%, Vitamin B120.000000525-0.00000756%, Niacin 0.00147-0.00756%, Folic acid 0.000525-0.000252%, Pantothenic acid 0.002 -0.01%, biotin 0.000084-0.0000504%, inositol 0.021-0.1995%, choline 0.0357-0.252%, and taurine 0-0.063%.
  • the composite mineral is composed of the following mass percentage components: sodium 0.105-0.294%, chlorine 0.252-0.798%, potassium 0.294-0.903%, calcium 0.252-0.735%, phosphorus 0.126-0.504%, iron 0.0021-0.00756%, Zinc is 0.00252-0.00756%, manganese is 0.000252-0.000504%, copper is 0.001785-0.000609%, magnesium is 0.0252-0.0756%, selenium is 0.00001-0.0000399%, and iodine is 0.000525-0.000294%.
  • the flavor is one of chocolate, banana, strawberry, orange, grapefruit and other fruit flavors.
  • the addition of flavor can provide a unique flavor to the formula powder of the present invention to suit different patients.
  • the composite unsaturated fatty acid is composed of DHA and ARA, and the weight ratio of DHA to ARA is 1: 1.8.
  • Feeding The weighed raw and auxiliary materials are transferred to the three-dimensional mixer through the feeding station; when feeding, pay attention to placing relatively small raw materials such as vitamins and minerals in the middle of the bulk materials to prevent small materials from sticking. Attached to the surface of the equipment to affect uniformity;
  • Packaging product packaging using automatic packaging machines
  • the formula powder for phenylketonuria patients of the present invention is prepared from the following components in mass percentage:
  • Vegetable oil fat powder 79.883%, compound amino acids 14%, fructooligosaccharide (Fos) 0.1%, galactooligosaccharide (Gos) 0.9%, nucleotides 0.05%, docosahexaenoic acid (DHA) 0.0525%, two Deca-tetraenoic acid (ARA) 0.0945%, flavor 0.3%, complex vitamin 0.12% and complex mineral 4.5%, the formula powder does not contain phenylalanine.
  • the composite amino acid is composed of the following mass percentage components: L-alanine 0.2-0.4%, L-arginine 0.2-0.5%, L-aspartic acid 0.7-1.0%, and L-cystine 0.15 -0.35%, L-glutamine 1.6-2.0%, glycine 0.1-0.3%, L-histidine 0.3-0.5%, L-isoleucine 0.8-1.1%, L-leucine 1.4-1.6% , L-lysine hydrochloride 0.8-1.2%, L-methionine 0.2-0.4%, L-proline 0.8-1.1%, L-serine 0.3-0.6%, L-threonine 0.5-0.7% L-tryptophan 0.2-0.4%, L-tyrosine 0.8-1.2%, and L-valine 0.8-1.2%.
  • the vegetable oil fat powder is made of solid glucose syrup, pre-gelatinized starch, fatty acid and emulsifier by spray drying.
  • the nucleotide is composed of adenylic acid, uridine, guanylic acid, cytidylic acid, and inosine acid, and its mass ratio is 2.9: 3.5: 1.0: 5.1: 2.0.
  • the compound vitamin is composed of the following mass percentage components: vitamin A 0.00029-0.0009%, vitamin C 0.0525-0.357%, vitamin D 0.0000525-0.0000126%, vitamin E 0.00252-0.0252%, vitamin K10.000021- 0.0001365%, Vitamin B10.000294-0.001512%, Vitamin B20.000399-0.002499%, Vitamin B60.0001785-0.000945%, Vitamin B120.000000525-0.00000756%, Niacin 0.00147-0.00756%, Folic acid 0.000525-0.000252%, Pantothenic acid 0.002 -0.01%, biotin 0.000084-0.0000504%, inositol 0.021-0.1995%, choline 0.0357-0.252%, and taurine 0-0.063%.
  • the composite mineral is composed of the following mass percentage components: sodium 0.105-0.294%, chlorine 0.252-0.798%, potassium 0.294-0.903%, calcium 0.252-0.735%, phosphorus 0.126-0.504%, iron 0.0021-0.00756%, Zinc is 0.00252-0.00756%, manganese is 0.000252-0.000504%, copper is 0.001785-0.000609%, magnesium is 0.0252-0.0756%, selenium is 0.00001-0.0000399%, and iodine is 0.000525-0.000294%.
  • the flavor is one of chocolate, banana, strawberry, orange, grapefruit and other fruit flavors.
  • the addition of flavor can provide a unique flavor to the formula powder of the present invention to suit different patients.
  • the composite unsaturated fatty acid is composed of DHA and ARA, and the weight ratio of DHA to ARA is 1: 1.8.
  • Feeding The weighed raw and auxiliary materials are transferred to the three-dimensional mixer through the feeding station; when feeding, pay attention to placing relatively small raw materials such as vitamins and minerals in the middle of the bulk materials to prevent small materials from sticking. Attached to the surface of the equipment to affect uniformity;
  • the mixed materials will be transferred to the powder storage for temporary storage, and the inspection department will take samples for process product inspection;
  • Packaging product packaging using automatic packaging machines
  • the formula powder for phenylketonuria patients of the present invention is prepared from the following components in mass percentage:
  • Vegetable oil fat powder 88.296%, compound amino acids 8%, nucleotides 0.035%, docosahexaenoic acid (DHA) 0.045%, eicosatetraenoic acid (ARA) 0.081%, flavor 0.5%, multivitamin 0.087% And compound minerals, 2.956%, the formula powder does not contain phenylalanine.
  • the composite amino acid is composed of the following mass percentage components: L-alanine 0.2-0.4%, L-arginine 0.2-0.5%, L-aspartic acid 0.7-1.0%, and L-cystine 0.15 -0.35%, L-glutamine 1.6-2.0%, glycine 0.1-0.3%, L-histidine 0.3-0.5%, L-isoleucine 0.8-1.1%, L-leucine 1.4-1.6% , L-lysine hydrochloride 0.8-1.2%, L-methionine 0.2-0.4%, L-proline 0.8-1.1%, L-serine 0.3-0.6%, L-threonine 0.5-0.7% L-tryptophan 0.2-0.4%, L-tyrosine 0.8-1.2%, and L-valine 0.8-1.2%.
  • the vegetable oil fat powder is made of solid glucose syrup, pre-gelatinized starch, fatty acid and emulsifier by spray drying.
  • the nucleotide is composed of adenylic acid, uridine, guanylic acid, cytidylic acid, and inosine acid, and its mass ratio is 2.9: 3.5: 1.0: 5.1: 2.0.
  • the compound vitamin is composed of the following mass percentage components: vitamin A 0.00029-0.0009%, vitamin C 0.0525-0.357%, vitamin D 0.0000525-0.0000126%, vitamin E 0.00252-0.0252%, vitamin K10.000021- 0.0001365%, Vitamin B10.000294-0.001512%, Vitamin B20.000399-0.002499%, Vitamin B60.0001785-0.000945%, Vitamin B120.000000525-0.00000756%, Niacin 0.00147-0.00756%, Folic acid 0.000525-0.000252%, Pantothenic acid 0.002 -0.01%, biotin 0.000084-0.0000504%, inositol 0.021-0.1995%, choline 0.0357-0.252%, and taurine 0-0.063%.
  • the composite mineral is composed of the following mass percentage components: sodium 0.105-0.294%, chlorine 0.252-0.798%, potassium 0.294-0.903%, calcium 0.252-0.735%, phosphorus 0.126-0.504%, iron 0.0021-0.00756%, Zinc is 0.00252-0.00756%, manganese is 0.000252-0.000504%, copper is 0.001785-0.000609%, magnesium is 0.0252-0.0756%, selenium is 0.00001-0.0000399%, and iodine is 0.000525-0.000294%.
  • the flavor is one of chocolate, banana, strawberry, orange, grapefruit and other fruit flavors.
  • the addition of flavor can provide a unique flavor to the formula powder of the present invention to suit different patients.
  • the composite unsaturated fatty acid is composed of DHA and ARA, and the weight ratio of DHA to ARA is 1: 1.8.
  • Feeding The weighed raw and auxiliary materials are transferred to the three-dimensional mixer through the feeding station; when feeding, pay attention to placing relatively small raw materials such as vitamins and minerals in the middle of the bulk materials to prevent small materials from sticking. Attached to the surface of the equipment to affect uniformity;
  • the mixed materials will be transferred to the powder storage for temporary storage, and the inspection department will take samples for process product inspection;
  • Packaging product packaging using automatic packaging machines
  • the formula powder for phenylketonuria patients of the present invention is prepared from the following components in mass percentage:
  • Vegetable oil fat powder 81.55%, complex amino acids 8.589%, fructooligosaccharides (Fos) 6.45%, galactooligosaccharides (Gos) 1.006%, nucleotides 0.012%, docosahexaenoic acid (DHA) 0.055%, two Deca-tetraenoic acid (ARA) 0.099%, complex vitamin 0.044% and complex mineral 2.1955%, the formula powder does not contain phenylalanine.
  • the composite amino acid is composed of the following mass percentage components: L-alanine 0.2-0.4%, L-arginine 0.2-0.5%, L-aspartic acid 0.7-1.0%, and L-cystine 0.15 -0.35%, L-glutamine 1.6-2.0%, glycine 0.1-0.3%, L-histidine 0.3-0.5%, L-isoleucine 0.8-1.1%, L-leucine 1.4-1.6% , L-lysine hydrochloride 0.8-1.2%, L-methionine 0.2-0.4%, L-proline 0.8-1.1%, L-serine 0.3-0.6%, L-threonine 0.5-0.7% L-tryptophan 0.2-0.4%, L-tyrosine 0.8-1.2%, and L-valine 0.8-1.2%.
  • the vegetable oil fat powder is made of solid glucose syrup, pre-gelatinized starch, fatty acid and emulsifier by spray drying.
  • the nucleotide is composed of adenylic acid, uridine, guanylic acid, cytidylic acid, and inosine acid, and its mass ratio is 2.9: 3.5: 1.0: 5.1: 2.0.
  • the compound vitamin is composed of the following mass percentage components: vitamin A 0.00029-0.0009%, vitamin C 0.0525-0.357%, vitamin D 0.0000525-0.0000126%, vitamin E 0.00252-0.0252%, vitamin K10.000021- 0.0001365%, Vitamin B10.000294-0.001512%, Vitamin B20.000399-0.002499%, Vitamin B60.0001785-0.000945%, Vitamin B120.000000525-0.00000756%, Niacin 0.00147-0.00756%, Folic acid 0.000525-0.000252%, Pantothenic acid 0.002 -0.01%, biotin 0.000084-0.0000504%, inositol 0.021-0.1995%, choline 0.0357-0.252%, and taurine 0-0.063%.
  • the composite mineral is composed of the following mass percentage components: sodium 0.105-0.294%, chlorine 0.252-0.798%, potassium 0.294-0.903%, calcium 0.252-0.735%, phosphorus 0.126-0.504%, iron 0.0021-0.00756%, Zinc is 0.00252-0.00756%, manganese is 0.000252-0.000504%, copper is 0.001785-0.000609%, magnesium is 0.0252-0.0756%, selenium is 0.00001-0.0000399%, and iodine is 0.000525-0.000294%.
  • the composite unsaturated fatty acid is composed of DHA and ARA, and the weight ratio of DHA to ARA is 1: 1.8.
  • Feeding The weighed raw and auxiliary materials are transferred to the three-dimensional mixer through the feeding station; when feeding, pay attention to placing relatively small raw materials such as vitamins and minerals in the middle of the bulk materials to prevent small materials from sticking. Attached to the surface of the equipment to affect uniformity;
  • the mixed materials will be transferred to the powder storage for temporary storage, and the inspection department will take samples for process product inspection;
  • Packaging product packaging using automatic packaging machines
  • the formula powder for phenylketonuria patients of the present invention is prepared from the following components in mass percentage:
  • Vegetable oil fat powder 74.095%, complex amino acids 13.55%, fructo-oligosaccharides (Fos) 1.0%, galactooligosaccharides (Gos) 6.45%, nucleotides 0.058%, multivitamins 0.13% and complex minerals 4.717%, said formula
  • the powder does not contain phenylalanine.
  • the composite amino acid is composed of the following mass percentage components: L-alanine 0.2-0.4%, L-arginine 0.2-0.5%, L-aspartic acid 0.7-1.0%, and L-cystine 0.15 -0.35%, L-glutamine 1.6-2.0%, glycine 0.1-0.3%, L-histidine 0.3-0.5%, L-isoleucine 0.8-1.1%, L-leucine 1.4-1.6% , L-lysine hydrochloride 0.8-1.2%, L-methionine 0.2-0.4%, L-proline 0.8-1.1%, L-serine 0.3-0.6%, L-threonine 0.5-0.7% L-tryptophan 0.2-0.4%, L-tyrosine 0.8-1.2%, and L-valine 0.8-1.2%.
  • the vegetable oil fat powder is made of solid glucose syrup, pre-gelatinized starch, fatty acid and emulsifier by spray drying.
  • the nucleotide is composed of adenylic acid, uridine, guanylic acid, cytidylic acid, and inosine acid, and its mass ratio is 2.9: 3.5: 1.0: 5.1: 2.0.
  • the compound vitamin is composed of the following mass percentage components: vitamin A 0.00029-0.0009%, vitamin C 0.0525-0.357%, vitamin D 0.0000525-0.0000126%, vitamin E 0.00252-0.0252%, vitamin K10.000021- 0.0001365%, Vitamin B10.000294-0.001512%, Vitamin B20.000399-0.002499%, Vitamin B60.0001785-0.000945%, Vitamin B120.000000525-0.00000756%, Niacin 0.00147-0.00756%, Folic acid 0.000525-0.000252%, Pantothenic acid 0.002 -0.01%, biotin 0.000084-0.0000504%, inositol 0.021-0.1995%, choline 0.0357-0.252%, and taurine 0-0.063%.
  • the composite mineral is composed of the following mass percentage components: sodium 0.105-0.294%, chlorine 0.252-0.798%, potassium 0.294-0.903%, calcium 0.252-0.735%, phosphorus 0.126-0.504%, iron 0.0021-0.00756%, Zinc is 0.00252-0.00756%, manganese is 0.000252-0.000504%, copper is 0.001785-0.000609%, magnesium is 0.0252-0.0756%, selenium is 0.00001-0.0000399%, and iodine is 0.000525-0.000294%.
  • Feeding The weighed raw and auxiliary materials are transferred to the three-dimensional mixer through the feeding station; when feeding, pay attention to placing relatively small raw materials such as vitamins and minerals in the middle of the bulk materials to prevent small materials from sticking. Attached to the surface of the equipment to affect uniformity;
  • the mixed materials will be transferred to the powder storage for temporary storage, and the inspection department will take samples for process product inspection;
  • Packaging product packaging using automatic packaging machines

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Abstract

一种用于苯丙酮尿症患者的配方粉，由如下质量百分比的组份制备而成：植物油脂肪粉60-90％，复合氨基酸8-20％，低聚果糖(Fos)0-6.45％，低聚半乳糖(Gos)0-6.45％，核苷酸0.012-0.058％，复合维生素0.06-0.41％和复合矿物质1.059-4.717％，所述配方粉中不包含苯丙氨酸。所述配方粉的制备方法，包括以下步骤：配料、投料、混合搅拌、接料暂存、包装和检验。

Description

一种用于苯丙酮尿症患者的配方粉及其制备方法 技术领域
本发明涉及一种配方粉及其制备方法，特别涉及一种用于苯丙酮尿症患者的配方粉及其制备方法。
背景技术
苯丙酮尿症(phenylketonur ia，PKU)是一种常见的氨基酸代谢障碍疾病，是由于苯丙氨酸(PA)代谢途径中的酶缺陷，使得苯丙氨酸不能转变成为酪氨酸，导致苯丙氨酸及其酮酸蓄积，并从尿中大量排出。
苯丙酮尿症在遗传性氨基酸代谢缺陷疾病中比较常见，发病率为1/10000。其遗传方式为常染色体隐性遗传。临床表现不均一，主要临床特征为智力低下、精神神经症状、湿疹、皮肤抓痕征及色素脱失和鼠气味等、脑电图异常。
苯丙酮尿症如果能得到早期诊断和早期治疗，则前述临床表现可不发生，智力正常，脑电图异常也可得到恢复。
但是，由于一般的食物、奶粉和母乳中都包含有苯丙氨酸，为了应对苯丙酮尿症，需要对患者进行饮食控制，限制苯丙氨酸的摄入。
因此，特别需要一种用于苯丙酮尿症患者的配方粉及其制备方法，以解决上述现有存在的问题。
发明内容
本发明的目的在于提供一种用于苯丙酮尿症患者的配方粉及其制备方法，针对现有技术的不足，既不产生苯丙氨酸及其酮酸的蓄积，又保证患者的氨基酸摄入，维持机体的氨基酸正常代谢。
为了实现上述目的，本发明所解决的技术问题可以采用以下技术方案来实现：
第一方面，本发明提供一种用于苯丙酮尿症患者的配方粉，其特征在于， 它由如下质量百分比的组份制备而成：
植物油脂肪粉60-90％，复合氨基酸8-20％，低聚果糖(Fos)0-6.45％，低聚半乳糖(Gos)0-6.45％，核苷酸0.012-0.058％，复合维生素0.06-0.41％和复合矿物质1.059-4.717％，所述配方粉中不包含苯丙氨酸。
在本发明的一个实施例中，所述复合氨基酸由以下氨基酸组成：L－丙氨酸；L－精氨酸和/或L－盐酸精氨酸、L－天冬氨酸和/或L－天冬氨酸镁；L－胱氨酸、L－半胱氨酸、L－盐酸半胱氨酸中的一种或多种组合；L－谷氨酸、L－谷氨酸钾、L－谷氨酰胺中的一种或多种组合；甘氨酸；L－组氨酸和/或L－盐酸组氨酸；L－异亮氨酸、L－亮氨酸；L－盐酸赖氨酸和/或L－赖氨酸醋酸盐；L－甲硫氨酸和/或L－乙酰－甲硫氨酸；L－脯氨酸；L－丝氨酸；L－苏氨酸；L－色氨酸；L－酪氨酸；L－缬氨酸，并且L－酪氨酸占所述复合氨基酸重量的7-11％。
在本发明的一个实施例中，所述复合氨基酸由以下质量百分比的组分组成：L－丙氨酸0.2-0.4％、L－精氨酸0.2-0.5％、L－天冬氨酸0.7-1.0％、L－胱氨酸0.15-0.35％、L－谷氨酰胺1.6-2.0％、甘氨酸0.1-0.3％、L－组氨酸0.3-0.5％、L－异亮氨酸0.8-1.1％、L－亮氨酸1.4-1.6％、L－盐酸赖氨酸0.8-1.2％、L－甲硫氨酸0.2-0.4％、L－脯氨酸0.8-1.1％、L－丝氨酸0.3-0.6％、L－苏氨酸0.5-0.7％、L－色氨酸0.2-0.4％、L－酪氨酸0.8-1.2％和L－缬氨酸0.8-1.2％。
在本发明的一个实施例中，所述植物油脂肪粉由固体葡萄糖浆、预糊化淀粉、脂肪酸、乳化剂通过喷雾干燥而制成。植物脂肪粉中包含脂肪和碳水化合物，为机体提供能量。
在本发明的一个实施例中，所述核苷酸由腺苷酸、尿苷酸、鸟苷酸、胞苷酸、肌苷酸组成，其质量比为2.9:3.5:1.0:5.1:2.0。
在本发明的一个实施例中，所述复合维生素由以下质量百分比的组分组成：维生素A0.00029-0.0009％、维生素C0.0525-0.357％、维生素D0.00000525-0.0000126％、维生素E0.00252-0.0252％、维生素K10.000021-0.0001365％、维生素B10.000294-0.001512％、维生素B20.000399-0.002499％、维生素B60.0001785-0.000945％、维生素 B120.000000525-0.00000756％、烟酸0.00147-0.00756％、叶酸0.0000525-0.000252％、泛酸0.002-0.01％、生物素0.0000084-0.0000504％、肌醇0.021-0.1995％、胆碱0.0357-0.252％和牛磺酸0-0.063％。复合维生素为患者提供身体所需的维生素，参与身体的各种生化反应。
在本发明的一个实施例中，所述复合矿物质由以下质量百分比的组分组成：钠0.105-0.294％、氯0.252-0.798％、钾0.294-0.903％、钙0.252-0.735％、磷0.126-0.504％、铁0.0021-0.00756％、锌0.00252-0.00756％、锰0.0000252-0.000504％、铜0.0001785-0.000609％、镁0.0252-0.0756％、硒0.00001-0.0000399％和碘0.0000525-0.000294％。矿物质是构成机体组织的重要成份；维持机体的酸碱平衡及组织细胞渗透压；维持神经肌肉兴奋性和细胞膜的通透性。
在本发明的一个实施例中，所述配方粉还包含质量百分比的复合不饱和脂肪酸0.12-0.16％，所述复合不饱和脂肪酸由DHA和ARA组成，DHA与ARA的重量比为1:1.8。含有复合不饱和脂肪酸的配方粉可用于0-10岁的患儿，长链不饱和脂肪酸，促进大脑的发育，提高认知能力；促进身体发育，保证健康体质；保证视力发育，降低视力缺陷；呵护早产婴儿，提高健康程度；支持免疫发育，提高呼吸系统健康。10岁以上的患者的配方粉可不需要包含所述复合不饱和脂肪酸。
在本发明的一个实施例中，所述配方粉还包含质量百分比的香精0.0-0.5％。所述香精为巧克力、香蕉、草莓、橙子、西柚及其他水果味中的一种。香精的添加可为本发明的配方粉提供特有的风味，以适合不同的患者。
第二方面，本发明提供一种用于苯丙酮尿症患者的配方粉的制备方法，其特征在于，它包括以下步骤：
(1)配料：将按预定比例称取原辅料，经复核准确后备用；
(2)投料：将已称量好的原辅料经投料站输送至混合机中；投料时注意将维生素、矿物质等质量比较小的原料放在大宗原料的中间投送，防止小料粘附在设备表面影响均匀性；
(3)混合搅拌：待所有的原辅料投送完毕，开启混合机搅拌，搅拌时间一般为8-12分钟(按混合的均匀程度确定混合时间)；
(4)接料暂存：将混合好的物料输送至粉仓中暂存，检验部门取样进行过程产品检验；
(5)包装：采用自动包装机进行产品包装；
(6)检验：检验部门取样进行终产品检测。
在本发明的一个实施例中，所述的配方粉的制备方法，其特征在于，所述混合搅拌步骤，推荐使用三维混合机，确定适宜的混合时间，以确保产品各项指标的均匀性。
本发明的用于苯丙酮尿症患者的配方粉及其制备方法，与现有技术相比，以脂肪、碳水化合物、氨基酸、维生素、矿物质等为基本的组成，配比科学，能满足婴幼儿的生长发育的需要；其中植物油脂肪粉中含有脂肪和碳水化合物，为机体提供能量，复合氨基酸为人体补充生长发育过程中必需的氨基酸，并且不添加苯丙氨酸，既不产生苯丙氨酸及其酮酸的蓄积，又保证患者的氨基酸摄入，维持机体的氨基酸正常代谢，实现本发明的目的。
本发明的特点可参阅本案以下较好实施方式的详细说明而获得清楚地了解。
具体实施方式
为了使本发明实现的技术手段、创作特征、达成目的与功效易于明白了解，下面结合具体实施例进一步阐述本发明。
实施例1
本发明的用于苯丙酮尿症患者的配方粉由如下质量百分比的组份制备而成：植物油脂肪粉82.26％，复合氨基酸14％，核苷酸0.03％，二十二碳六烯酸(DHA)0.05％，二十碳四烯酸(ARA)0.09％,香精0.1％，复合维生素0.57％和复合矿物质2.9％，所述配方粉中不包含苯丙氨酸。
所述复合氨基酸由以下质量百分比的组分组成：L－丙氨酸0.2-0.4％、L－精氨酸0.2-0.5％、L－天冬氨酸0.7-1.0％、L－胱氨酸0.15-0.35％、L－谷氨酰胺1.6-2.0％、甘氨酸0.1-0.3％、L－组氨酸0.3-0.5％、L－异亮氨酸0.8-1.1％、L－亮氨酸1.4-1.6％、L－盐酸赖氨酸0.8-1.2％、L－甲硫氨酸0.2-0.4％、L－脯氨酸0.8-1.1％、L－丝氨酸0.3-0.6％、L－苏氨酸0.5-0.7％、 L－色氨酸0.2-0.4％、L－酪氨酸0.8-1.2％和L－缬氨酸0.8-1.2％。
所述植物油脂肪粉由固体葡萄糖浆、预糊化淀粉、脂肪酸、乳化剂通过喷雾干燥而制成。
所述核苷酸由腺苷酸、尿苷酸、鸟苷酸、胞苷酸、肌苷酸组成，其质量比为2.9:3.5:1.0:5.1:2.0。
所述复合维生素由以下质量百分比的组分组成：维生素A0.00029-0.0009％、维生素C0.0525-0.357％、维生素D0.00000525-0.0000126％、维生素E0.00252-0.0252％、维生素K10.000021-0.0001365％、维生素B10.000294-0.001512％、维生素B20.000399-0.002499％、维生素B60.0001785-0.000945％、维生素B120.000000525-0.00000756％、烟酸0.00147-0.00756％、叶酸0.0000525-0.000252％、泛酸0.002-0.01％、生物素0.0000084-0.0000504％、肌醇0.021-0.1995％、胆碱0.0357-0.252％和牛磺酸0-0.063％。
所述复合矿物质由以下质量百分比的组分组成：钠0.105-0.294％、氯0.252-0.798％、钾0.294-0.903％、钙0.252-0.735％、磷0.126-0.504％、铁0.0021-0.00756％、锌0.00252-0.00756％、锰0.0000252-0.000504％、铜0.0001785-0.000609％、镁0.0252-0.0756％、硒0.00001-0.0000399％和碘0.0000525-0.000294％。
所述香精为巧克力、香蕉、草莓、橙子、西柚及其他水果味中的一种。香精的添加可为本发明的配方粉提供特有的风味，以适合不同的患者。
所述复合不饱和脂肪酸由DHA和ARA组成，DHA与ARA的重量比为1:1.8。
上述用于苯丙酮尿症患者的配方粉的制备方法如下：
(1)配料：将按预定比例称取原辅料，经复核准确后备用；
(2)投料：将已称量好的原辅料经投料站输送至三维混合机中；投料时注意将维生素、矿物质等质量比较小的原料放在大宗原料的中间投送，防止小料粘附在设备表面影响均匀性；
(3)混合搅拌：待所有的原辅料投送完毕，开启混合机搅拌，搅拌时间一般为8-12分钟(按混合的均匀程度确定混合时间)；
(4)接料暂存：将混合好的物料输送至粉仓中暂存，检验部门取样进行 过程产品检验；
(5)包装：采用自动包装机进行产品包装；
(6)检验：检验部门取样进行终产品检测。
实施例2
本发明的用于苯丙酮尿症患者的配方粉由如下质量百分比的组份制备而成：
植物油脂肪粉79.883％，复合氨基酸14％，低聚果糖(Fos)0.1％，低聚半乳糖(Gos)0.9％，核苷酸0.05％，二十二碳六烯酸(DHA)0.0525％，二十碳四烯酸(ARA)0.0945％,香精0.3％，复合维生素0.12％和复合矿物质4.5％，所述配方粉中不包含苯丙氨酸。
所述复合氨基酸由以下质量百分比的组分组成：L－丙氨酸0.2-0.4％、L－精氨酸0.2-0.5％、L－天冬氨酸0.7-1.0％、L－胱氨酸0.15-0.35％、L－谷氨酰胺1.6-2.0％、甘氨酸0.1-0.3％、L－组氨酸0.3-0.5％、L－异亮氨酸0.8-1.1％、L－亮氨酸1.4-1.6％、L－盐酸赖氨酸0.8-1.2％、L－甲硫氨酸0.2-0.4％、L－脯氨酸0.8-1.1％、L－丝氨酸0.3-0.6％、L－苏氨酸0.5-0.7％、L－色氨酸0.2-0.4％、L－酪氨酸0.8-1.2％和L－缬氨酸0.8-1.2％。
所述植物油脂肪粉由固体葡萄糖浆、预糊化淀粉、脂肪酸、乳化剂通过喷雾干燥而制成。
所述核苷酸由腺苷酸、尿苷酸、鸟苷酸、胞苷酸、肌苷酸组成，其质量比为2.9:3.5:1.0:5.1:2.0。
所述复合维生素由以下质量百分比的组分组成：维生素A0.00029-0.0009％、维生素C0.0525-0.357％、维生素D0.00000525-0.0000126％、维生素E0.00252-0.0252％、维生素K10.000021-0.0001365％、维生素B10.000294-0.001512％、维生素B20.000399-0.002499％、维生素B60.0001785-0.000945％、维生素B120.000000525-0.00000756％、烟酸0.00147-0.00756％、叶酸0.0000525-0.000252％、泛酸0.002-0.01％、生物素0.0000084-0.0000504％、肌醇0.021-0.1995％、胆碱0.0357-0.252％和牛磺酸0-0.063％。
所述复合矿物质由以下质量百分比的组分组成：钠0.105-0.294％、氯0.252-0.798％、钾0.294-0.903％、钙0.252-0.735％、磷0.126-0.504％、铁0.0021-0.00756％、锌0.00252-0.00756％、锰0.0000252-0.000504％、铜0.0001785-0.000609％、镁0.0252-0.0756％、硒0.00001-0.0000399％和碘0.0000525-0.000294％。
所述香精为巧克力、香蕉、草莓、橙子、西柚及其他水果味中的一种。香精的添加可为本发明的配方粉提供特有的风味，以适合不同的患者。
所述复合不饱和脂肪酸由DHA和ARA组成，DHA与ARA的重量比为1:1.8。
上述用于苯丙酮尿症患者的配方粉的制备方法如下：
(1)配料：将按预定比例称取原辅料，经复核准确后备用；
(2)投料：将已称量好的原辅料经投料站输送至三维混合机中；投料时注意将维生素、矿物质等质量比较小的原料放在大宗原料的中间投送，防止小料粘附在设备表面影响均匀性；
(3)混合搅拌：待所有的原辅料投送完毕，开启混合机搅拌，搅拌时间一般为8-12分钟(按混合的均匀程度确定混合时间)；
(4)接料暂存：将混合好的物料输送至粉仓中暂存，检验部门取样进行过程产品检验；
(5)包装：采用自动包装机进行产品包装；
(6)检验：检验部门取样进行终产品检测。
实施例3
本发明的用于苯丙酮尿症患者的配方粉由如下质量百分比的组份制备而成：
植物油脂肪粉88.296％，复合氨基酸8％，核苷酸0.035％，二十二碳六烯酸(DHA)0.045％，二十碳四烯酸(ARA)0.081％,香精0.5％，复合维生素0.087％和复合矿物质2.956％，所述配方粉中不包含苯丙氨酸。
所述复合氨基酸由以下质量百分比的组分组成：L－丙氨酸0.2-0.4％、L－精氨酸0.2-0.5％、L－天冬氨酸0.7-1.0％、L－胱氨酸0.15-0.35％、L－谷氨酰胺1.6-2.0％、甘氨酸0.1-0.3％、L－组氨酸0.3-0.5％、L－异亮氨酸 0.8-1.1％、L－亮氨酸1.4-1.6％、L－盐酸赖氨酸0.8-1.2％、L－甲硫氨酸0.2-0.4％、L－脯氨酸0.8-1.1％、L－丝氨酸0.3-0.6％、L－苏氨酸0.5-0.7％、L－色氨酸0.2-0.4％、L－酪氨酸0.8-1.2％和L－缬氨酸0.8-1.2％。
所述植物油脂肪粉由固体葡萄糖浆、预糊化淀粉、脂肪酸、乳化剂通过喷雾干燥而制成。
所述核苷酸由腺苷酸、尿苷酸、鸟苷酸、胞苷酸、肌苷酸组成，其质量比为2.9:3.5:1.0:5.1:2.0。
所述复合维生素由以下质量百分比的组分组成：维生素A0.00029-0.0009％、维生素C0.0525-0.357％、维生素D0.00000525-0.0000126％、维生素E0.00252-0.0252％、维生素K10.000021-0.0001365％、维生素B10.000294-0.001512％、维生素B20.000399-0.002499％、维生素B60.0001785-0.000945％、维生素B120.000000525-0.00000756％、烟酸0.00147-0.00756％、叶酸0.0000525-0.000252％、泛酸0.002-0.01％、生物素0.0000084-0.0000504％、肌醇0.021-0.1995％、胆碱0.0357-0.252％和牛磺酸0-0.063％。
所述复合矿物质由以下质量百分比的组分组成：钠0.105-0.294％、氯0.252-0.798％、钾0.294-0.903％、钙0.252-0.735％、磷0.126-0.504％、铁0.0021-0.00756％、锌0.00252-0.00756％、锰0.0000252-0.000504％、铜0.0001785-0.000609％、镁0.0252-0.0756％、硒0.00001-0.0000399％和碘0.0000525-0.000294％。
所述香精为巧克力、香蕉、草莓、橙子、西柚及其他水果味中的一种。香精的添加可为本发明的配方粉提供特有的风味，以适合不同的患者。
所述复合不饱和脂肪酸由DHA和ARA组成，DHA与ARA的重量比为1:1.8。
上述用于苯丙酮尿症患者的配方粉的制备方法如下：
(1)配料：将按预定比例称取原辅料，经复核准确后备用；
(2)投料：将已称量好的原辅料经投料站输送至三维混合机中；投料时注意将维生素、矿物质等质量比较小的原料放在大宗原料的中间投送，防止小料粘附在设备表面影响均匀性；
(3)混合搅拌：待所有的原辅料投送完毕，开启混合机搅拌，搅拌时间 一般为8-12分钟(按混合的均匀程度确定混合时间)；
(4)接料暂存：将混合好的物料输送至粉仓中暂存，检验部门取样进行过程产品检验；
(5)包装：采用自动包装机进行产品包装；
(6)检验：检验部门取样进行终产品检测。
实施例4
本发明的用于苯丙酮尿症患者的配方粉由如下质量百分比的组份制备而成：
植物油脂肪粉81.55％，复合氨基酸8.589％，低聚果糖(Fos)6.45％，低聚半乳糖(Gos)1.006％，核苷酸0.012％，二十二碳六烯酸(DHA)0.055％，二十碳四烯酸(ARA)0.099％,复合维生素0.044％和复合矿物质2.195％，所述配方粉中不包含苯丙氨酸。
所述复合氨基酸由以下质量百分比的组分组成：L－丙氨酸0.2-0.4％、L－精氨酸0.2-0.5％、L－天冬氨酸0.7-1.0％、L－胱氨酸0.15-0.35％、L－谷氨酰胺1.6-2.0％、甘氨酸0.1-0.3％、L－组氨酸0.3-0.5％、L－异亮氨酸0.8-1.1％、L－亮氨酸1.4-1.6％、L－盐酸赖氨酸0.8-1.2％、L－甲硫氨酸0.2-0.4％、L－脯氨酸0.8-1.1％、L－丝氨酸0.3-0.6％、L－苏氨酸0.5-0.7％、L－色氨酸0.2-0.4％、L－酪氨酸0.8-1.2％和L－缬氨酸0.8-1.2％。
所述植物油脂肪粉由固体葡萄糖浆、预糊化淀粉、脂肪酸、乳化剂通过喷雾干燥而制成。
所述核苷酸由腺苷酸、尿苷酸、鸟苷酸、胞苷酸、肌苷酸组成，其质量比为2.9:3.5:1.0:5.1:2.0。
所述复合维生素由以下质量百分比的组分组成：维生素A0.00029-0.0009％、维生素C0.0525-0.357％、维生素D0.00000525-0.0000126％、维生素E0.00252-0.0252％、维生素K10.000021-0.0001365％、维生素B10.000294-0.001512％、维生素B20.000399-0.002499％、维生素B60.0001785-0.000945％、维生素B120.000000525-0.00000756％、烟酸0.00147-0.00756％、叶酸 0.0000525-0.000252％、泛酸0.002-0.01％、生物素0.0000084-0.0000504％、肌醇0.021-0.1995％、胆碱0.0357-0.252％和牛磺酸0-0.063％。
所述复合矿物质由以下质量百分比的组分组成：钠0.105-0.294％、氯0.252-0.798％、钾0.294-0.903％、钙0.252-0.735％、磷0.126-0.504％、铁0.0021-0.00756％、锌0.00252-0.00756％、锰0.0000252-0.000504％、铜0.0001785-0.000609％、镁0.0252-0.0756％、硒0.00001-0.0000399％和碘0.0000525-0.000294％。
所述复合不饱和脂肪酸由DHA和ARA组成，DHA与ARA的重量比为1:1.8。
上述用于苯丙酮尿症患者的配方粉的制备方法如下：
(1)配料：将按预定比例称取原辅料，经复核准确后备用；
(2)投料：将已称量好的原辅料经投料站输送至三维混合机中；投料时注意将维生素、矿物质等质量比较小的原料放在大宗原料的中间投送，防止小料粘附在设备表面影响均匀性；
(3)混合搅拌：待所有的原辅料投送完毕，开启混合机搅拌，搅拌时间一般为8-12分钟(按混合的均匀程度确定混合时间)；
(4)接料暂存：将混合好的物料输送至粉仓中暂存，检验部门取样进行过程产品检验；
(5)包装：采用自动包装机进行产品包装；
(6)检验：检验部门取样进行终产品检测。
实施例5
本发明的用于苯丙酮尿症患者的配方粉由如下质量百分比的组份制备而成：
植物油脂肪粉74.095％，复合氨基酸13.55％，低聚果糖(Fos)1.0％，低聚半乳糖(Gos)6.45％，核苷酸0.058％,复合维生素0.13％和复合矿物质4.717％，所述配方粉中不包含苯丙氨酸。
所述复合氨基酸由以下质量百分比的组分组成：L－丙氨酸0.2-0.4％、L－精氨酸0.2-0.5％、L－天冬氨酸0.7-1.0％、L－胱氨酸0.15-0.35％、L－谷氨酰胺1.6-2.0％、甘氨酸0.1-0.3％、L－组氨酸0.3-0.5％、L－异亮氨酸 0.8-1.1％、L－亮氨酸1.4-1.6％、L－盐酸赖氨酸0.8-1.2％、L－甲硫氨酸0.2-0.4％、L－脯氨酸0.8-1.1％、L－丝氨酸0.3-0.6％、L－苏氨酸0.5-0.7％、L－色氨酸0.2-0.4％、L－酪氨酸0.8-1.2％和L－缬氨酸0.8-1.2％。
所述植物油脂肪粉由固体葡萄糖浆、预糊化淀粉、脂肪酸、乳化剂通过喷雾干燥而制成。
所述核苷酸由腺苷酸、尿苷酸、鸟苷酸、胞苷酸、肌苷酸组成，其质量比为2.9:3.5:1.0:5.1:2.0。
所述复合维生素由以下质量百分比的组分组成：维生素A0.00029-0.0009％、维生素C0.0525-0.357％、维生素D0.00000525-0.0000126％、维生素E0.00252-0.0252％、维生素K10.000021-0.0001365％、维生素B10.000294-0.001512％、维生素B20.000399-0.002499％、维生素B60.0001785-0.000945％、维生素B120.000000525-0.00000756％、烟酸0.00147-0.00756％、叶酸0.0000525-0.000252％、泛酸0.002-0.01％、生物素0.0000084-0.0000504％、肌醇0.021-0.1995％、胆碱0.0357-0.252％和牛磺酸0-0.063％。
所述复合矿物质由以下质量百分比的组分组成：钠0.105-0.294％、氯0.252-0.798％、钾0.294-0.903％、钙0.252-0.735％、磷0.126-0.504％、铁0.0021-0.00756％、锌0.00252-0.00756％、锰0.0000252-0.000504％、铜0.0001785-0.000609％、镁0.0252-0.0756％、硒0.00001-0.0000399％和碘0.0000525-0.000294％。
上述用于苯丙酮尿症患者的配方粉的制备方法如下：
(1)配料：将按预定比例称取原辅料，经复核准确后备用；
(2)投料：将已称量好的原辅料经投料站输送至三维混合机中；投料时注意将维生素、矿物质等质量比较小的原料放在大宗原料的中间投送，防止小料粘附在设备表面影响均匀性；
(3)混合搅拌：待所有的原辅料投送完毕，开启混合机搅拌，搅拌时间一般为8-12分钟(按混合的均匀程度确定混合时间)；
(4)接料暂存：将混合好的物料输送至粉仓中暂存，检验部门取样进行过程产品检验；
(5)包装：采用自动包装机进行产品包装；
(6)检验：检验部门取样进行终产品检测。
以上显示和描述了本发明的基本原理、主要特征和本发明的优点。本行业的技术人员应该了解，本发明不受上述实施例的限制，上述实施例和说明书中描述的只是本发明的原理，在不脱离本发明精神和范围的前提下本发明还会有各种变化和改进，这些变化和改进都落入要求保护的本发明的范围内。本发明要求的保护范围由所附的权利要求书及其等同物界定。

Claims (10)

  1. 一种用于苯丙酮尿症患者的配方粉，其特征在于，它由如下质量百分比的组份制备而成：
    植物油脂肪粉60-90％，复合氨基酸8-20％，低聚果糖(Fos)0-6.45％，低聚半乳糖(Gos)0-6.45％，核苷酸0.012-0.058％，复合维生素0.06-0.41％和复合矿物质1.059-4.717％，所述配方粉中不包含苯丙氨酸。
  2. 根据权利要求1所述的配方粉，其特征在于，所述复合氨基酸由以下氨基酸组成：L－丙氨酸；L－精氨酸和/或L－盐酸精氨酸、L－天冬氨酸和/或L－天冬氨酸镁；L－胱氨酸、L－半胱氨酸、L－盐酸半胱氨酸中的一种或多种组合；L－谷氨酸、L－谷氨酸钾、L－谷氨酰胺中的一种或多种组合；甘氨酸；L－组氨酸和/或L－盐酸组氨酸；L－异亮氨酸、L－亮氨酸；L－盐酸赖氨酸和/或L－赖氨酸醋酸盐；L－甲硫氨酸和/或L－乙酰－甲硫氨酸；L－脯氨酸；L－丝氨酸；L－苏氨酸；L－色氨酸；L－酪氨酸；L－缬氨酸，并且L－酪氨酸占所述复合氨基酸重量的7-11％。
  3. 根据权利要求1所述的配方粉，其特征在于，所述复合氨基酸由以下质量百分比的组分组成：L－丙氨酸0.2-0.4％、L－精氨酸0.2-0.5％、L－天冬氨酸0.7-1.0％、L－胱氨酸0.15-0.35％、L－谷氨酰胺1.6-2.0％、甘氨酸0.1-0.3％、L－组氨酸0.3-0.5％、L－异亮氨酸0.8-1.1％、L－亮氨酸1.4-1.6％、L－盐酸赖氨酸0.8-1.2％、L－甲硫氨酸0.2-0.4％、L－脯氨酸0.8-1.1％、L－丝氨酸0.3-0.6％、L－苏氨酸0.5-0.7％、L－色氨酸0.2-0.4％、L－酪氨酸0.8-1.2％和L－缬氨酸0.8-1.2％。
  4. 根据权利要求1所述的配方粉，其特征在于，所述植物油脂肪粉由固体葡萄糖浆、预糊化淀粉、脂肪酸、乳化剂通过喷雾干燥而制成。
  5. 根据权利要求1所述的配方粉，其特征在于，所述核苷酸由腺苷酸、尿苷酸、鸟苷酸、胞苷酸、肌苷酸组成，其质量比为2.9:3.5:1.0:5.1:2.0。
  6. 根据权利要求1所述的配方粉，其特征在于，所述复合维生素由以下质量百分比的组分组成：维生素A0.00029-0.0009％、维生素C0.0525-0.357％、维生素D0.00000525-0.0000126％、维生素E0.00252-0.0252％、维生素K10.000021-0.0001365％、维生素B10.000294-0.001512％、维生素 B20.000399-0.002499％、维生素B60.0001785-0.000945％、维生素B120.000000525-0.00000756％、烟酸0.00147-0.00756％、叶酸0.0000525-0.000252％、泛酸0.002-0.01％、生物素0.0000084-0.0000504％、肌醇0.021-0.1995％、胆碱0.0357-0.252％和牛磺酸0-0.063％。
  7. 根据权利要求1所述的配方粉，其特征在于，所述复合矿物质由以下质量百分比的组分组成：钠0.105-0.294％、氯0.252-0.798％、钾0.294-0.903％、钙0.252-0.735％、磷0.126-0.504％、铁0.0021-0.00756％、锌0.00252-0.00756％、锰0.0000252-0.000504％、铜0.0001785-0.000609％、镁0.0252-0.0756％、硒0.00001-0.0000399％和碘0.0000525-0.000294％。
  8. 根据权利要求1所述的配方粉，其特征在于，所述配方粉还包含质量百分比的复合不饱和脂肪酸0.12-0.16％，所述复合不饱和脂肪酸由DHA和ARA组成，DHA与ARA的重量比为1:1.8。
  9. 根据权利要求1所述的配方粉，其特征在于，所述配方粉还包含质量百分比的香精0.0-0.5％；所述香精为巧克力、香蕉、草莓、橙子、西柚及其他水果味中的一种。
  10. 一种用于苯丙酮尿症患者的配方粉的制备方法，其特征在于，它包括以下步骤：
    (1)配料：将按预定比例称取原辅料，经复核准确后备用；
    (2)投料：将已称量好的原辅料经投料站输送至混合机中；投料时注意将维生素、矿物质等质量比较小的原料放在大宗原料的中间投送，防止小料粘附在设备表面影响均匀性；
    (3)混合搅拌：待所有的原辅料投送完毕，开启混合机搅拌，搅拌时间一般为8-12分钟(按混合的均匀程度确定混合时间)；
    (4)接料暂存：将混合好的物料输送至粉仓中暂存，检验部门取样进行过程产品检验；
    (5)包装：采用自动包装机进行产品包装；
    (6)检验：检验部门取样进行终产品检测。
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