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Formula powder for glutaric acidemia type I, and preparation method thereof
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A technology of glutaric acidemia and formula powder, applied in the field of biomedicine, can solve the problems such as the preparation method of formula powder without glutaric acidemia type I, achieve good solubility and stability, and ensure normal metabolism. Effect
Inactive Publication Date: 2018-03-02
赤峰赛佰诺制药有限公司
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Problems solved by technology
[0003] In the prior art, there is no formula powder suitable for glutaric acidemia type I and its preparation method
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Embodiment 1
[0049] The invention relates to a formula powder for glutaric acidemia type I, which comprises the following components in parts by weight: 30 parts by weight of vegetable oil and fat powder, 15 parts by weight of amino acid, 0 part by weight of algae oil, 0.01 part by weight of nucleotide, 2 parts by weight of multivitamins, 2 parts by weight of multivitamins, and 0 parts by weight of essence; the amino acid does not include tryptophan and lysine.
[0058] The invention relates to a formula powder for glutaric acidemia type I, which comprises the following components in parts by weight: 85 parts by weight of vegetable oil and fat powder, 60 parts by weight of amino acid, 1 part by weight of algae oil, 0.1 part by weight of nucleotides, 5 parts by weight of multi-vitamin, 5 parts by weight of multi-mineral and 1 part by weight of essence; the amino acid does not include tryptophan and lysine.
[0068] The invention relates to a formula powder for glutaric acidemia type I, which consists of 44 parts by weight of vegetable oil and fat powder, 50 parts by weight of amino acid, 0.5 part by weight of algae oil, 0.05 part by weight of nucleotides, It is composed of 2 parts by weight of vitamins, 1 part by weight of compound minerals and 0.005 parts by weight of essence.
[0070] In parts by weight, the amino acid consists...
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Abstract
The invention provides formula powder for glutaric acidemia type I, and a preparation method thereof, wherein the formula powder comprises vegetable oil fat powder, amino acids, fatty acid, algae oil,compound vitamins and compound minerals, and is prepared through a dry preparation process. According to the present invention, the formula powder contains the amino acids and does not contain tryptophan and lysine, such that the nutritional supplements for patients can be ensured, and the contents of tryptophan and lysine in the bodies of patients can be effectively controlled so as to further reduce the accumulation of harmful metabolites such as glutaric acid, 3-hydroxy glutaric acid and glutaconic acid in vivo and ensure the normal metabolism of patients; the formula powder has advantagesof scientific nutrient matching, good solubility and good stability; and the preparation method is simple and easy to operate, and is suitable for large-scale industrial production.
Description
technical field [0001] The invention relates to the field of biomedicine, in particular to a formula powder for glutaric acidemia type I and a preparation method thereof. Background technique [0002] Glutaric acidemia type I (GAI), a disorder of lysine, hydroxylysine, and tryptophanmetabolism due to glutaryl-CoA dehydrogenase (GCDH) deficiency leads to glutaric acid in the body , 3-Hydroxyglutaric acid, glutaconic acid accumulation, is an autosomal recessive genetic disease. The clinical manifestations are macrocephaly, or hypotonia, feeding difficulties, convulsions and other non-specific neurological symptoms. The principle of treatment for GAI is to avoid acute attacks and control symptoms. Long-term diet control is required to limit the intake of lysine and tryptophan to reduce the accumulation of glutaric acid, 3-hydroxyglutaric acid, and glutaconic acid in the patient's body and maintain them at normal levels. [0003] In the prior art, there is no formula powder ...
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