DEVELOPMENT OF THE COMPONENT COMPOSITION OF AMINO ACID MIXTURES FOR THE NUTRITION OF PATIENTS WITH PHENYLKETONURIA

According to the Ministry of Health in the Republic of Belarus in 2019, there were about 500 patients with phenylketonuria (PKU). Phenylketonuria is a hereditary disease associated with a violation of the metabolism of amino acids, in particular phenylalanine (FA). This disease is accompanied by the accumulation of phenylalanine and its toxic products in the tissues, which leads to severe damage to the central nervous system, manifested in the form of impaired mental development. Many years of world experience shows that for the treatment of such patients, a special diet is prescribed using amino acid mixtures that do not contain phenylalanine or contain it in small amounts, as well as low-protein products based on starch, which are necessary to ensure an adequate energy value of the diet. The article presents the stages of development of the component composition of domestic amino acid mixtures for the nutrition of patients with phenylketonuria, taking into account their age characteristics.

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FEATURES OF THE PRODUCTION TECHNOLOGY LOW-PROTEIN FOODS

Food Industry: Science and Technology ◽

10.47612/2073-4794-2021-14-3(53)-98-104 ◽

2021 ◽

Vol 14 (3(53)) ◽

pp. 98-104

Author(s):

Alexey Iosifovich Grigel

Keyword(s):

Amino Acids ◽

Central Nervous System ◽

Nervous System ◽

Life Support ◽

Hereditary Disease ◽

Bakery Products ◽

Severe Damage ◽

Low Protein ◽

The Central Nervous System ◽

Manufacturing Technologies

Phenylketonuria is a hereditary disease associated with a violation of the metabolism of amino acids, in particular phenylalanine. It is accompanied by the accumulation of phenylalanine and its toxic products in the tissues, which leads to severe damage to the central nervous system, manifested, in particular, in a violation of mental development. An important component of the life support of such patients is the observance of a low-protein diet. The article describes the features of manufacturing technologies for lowprotein products, including such as low-protein pasta and cereals, low-protein dry mixes, small-piece bakery products. The technological process and equipment for the production of low-protein products are described, as well as the characteristics of the intake range of products are given.

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Effect of the Addition of Imbalanced Amino Acid Mixtures to a Low Protein Diet, on Weight Gains and Plasma Amino Acids of Chicks

Journal of Nutrition ◽

10.1093/jn/79.3.296 ◽

1963 ◽

Vol 79 (3) ◽

pp. 296-302 ◽

Cited By ~ 17

Author(s):

D. C. Hill ◽

Ellen M. Olsen

Keyword(s):

Amino Acids ◽

Amino Acid ◽

Protein Diet ◽

Low Protein Diet ◽

Plasma Amino Acids ◽

Low Protein ◽

Weight Gains ◽

Amino Acid Mixtures

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Financial justification of investments into special diet for patients with phenylketonuria

Medicinski pregled ◽

10.2298/mpns1012771k ◽

2010 ◽

Vol 63 (11-12) ◽

pp. 771-774 ◽

Cited By ~ 1

Author(s):

Georgios Konstantinidis ◽

Dobrila Radovanov ◽

Nada Konstantinidis

Keyword(s):

Phenylalanine Hydroxylase ◽

Genetic Disorder ◽

Autosomal Recessive Disorder ◽

First Year ◽

Protein Supplement ◽

Special Diet ◽

Low Protein ◽

The Republic ◽

First Year Of Life ◽

Good Prevention

Introduction. Phenylketonuria is a genetic disorder of metabolism of amino acid phenylalanine, which results in the absence of phenylalanine hydroxylase, an enzyme that catalyzes the conversion of phenylalanine into tyrosine. It is an autosomal recessive disorder. Screening for phenylketonuria in Voivodina started in 2003. Screening data are shown in this paper. Treatment of phenylketonuria is based on a strict, life long, low protein diet with the controlled phenylalanine intake. Diet must start early, in the first weeks of life. The aim of the diet is to reduce natural protein intake and to cover protein needs by special phenylalanine free protein products. There is a big variety of formulas found available on the market for treatment of phenylketonuria. All of them are free of phenylalanine and very expensive. Discussion. Till May 2005 there was no refunding for these products in our country. According to the decision made by the Provincial Secretariat for Health, providing all children with protein supplement in their first year of life started at the Institute for Child and Youth Healthcare. In September 2007 the Republic Fund for Health Insurance started to refund protein supplement and low protein products for all children up to the age of 18 years. Conclusion. Besides all technical and organizational difficulties associated with this work, this paper also shows how, by good prevention of phenylketonuria complications, much more money can be saved than it has been invested, even in countries with low amounts of money allocated for this purpose (in absolute figures).

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