Amino acids in the blood determine what. Complex analysis for amino acids (32 indicators) (urine). Key Substances

[06-225 ] Blood test for amino acids (32 indicators)

5645 rub.

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Amino acids are important organic substances whose structure contains carboxyl and amine groups. A comprehensive study that determines the content of amino acids and their derivatives in the blood allows us to identify congenital and acquired disorders of amino acid metabolism.

* Composition of the study:

1. Alanine (ALA)
2. Arginine (ARG)
3. Aspartic acid (ASP)
4. Citrulline (CIT)
5. Glutamic acid (GLU)
6. Glycine (GLY)
7. Methionine (MET)
8. Ornithine (ORN)
9. Phenylalanine (PHE)
10. Tyrosine (TYR)
11. Valin (VAL)
12. Leucine (LEU)
13. Isoleucine (ILEU)
14. Hydroxyproline (HPRO)
15. Serin (SER)
16. Asparagine (ASN)
17. Glutamine (GLN)
18. Beta-alanine (BALA)
19. Taurine (TAU)
20. Histidine (HIS)
21. Threonine (THRE)
22. 1-methylhistidine (1MHIS)
23. 3-methylhistidine (3MHIS)
24. Alpha-aminobutyric acid (AABA)
25. Proline (PRO)
26. Cystathionine (CYST)
27. Lysine (LYS)
28. Cystine (CYS)
29. Cysteinic acid (CYSA)

Synonyms Russian

Screening for aminoacidopathy; amino acid profile.

SynonymsEnglish

Amino Acids Profile, Plasma.

Methodresearch

High performance liquid chromatography.

What biomaterial can be used for research?

Venous blood.

How to properly prepare for research?

• Eliminate alcohol from your diet for 24 hours before the test.
• Do not eat for 8 hours before the test; you can drink clean still water.
• Completely avoid taking medications for 24 hours before the test (in consultation with your doctor).
• Avoid physical and emotional stress for 30 minutes before the test.
• Do not smoke for 30 minutes before the test.

General information about the study

Amino acids are organic substances containing carboxyl and amine groups. About 100 amino acids are known, but only 20 are involved in protein synthesis. These amino acids are called “proteinogenic” (standard) and, according to the possibility of synthesis in the body, are classified into replaceable and essential. Essential amino acids include arginine, valine, histidine, isoleucine, leucine, lysine, methionine, threonine, tryptophan, phenylalanine. Nonessential amino acids are alanine, asparagine, aspartate, glycine, glutamate, glutamine, proline, serine, tyrosine, cysteine. Proteinogenic and non-standard amino acids, their metabolites are involved in various metabolic processes in the body. A defect in enzymes at various stages of transformation of substances can lead to the accumulation of amino acids and their transformation products and have a negative effect on the condition of the body.

Disorders of amino acid metabolism can be primary (congenital) or secondary (acquired). Primary aminoacidopathies are usually inherited autosomal recessively or X-linked and manifest in early childhood. Diseases develop due to a genetically determined deficiency of enzymes and/or transport proteins associated with the metabolism of certain amino acids. More than 30 variants of aminoacidopathies are described in the literature. Clinical manifestations can range from mild benign disorders to severe metabolic acidosis or alkalosis, vomiting, mental retardation and growth retardation, lethargy, coma, sudden neonatal death syndrome, osteomalacia, and osteoporosis. Secondary disorders of amino acid metabolism may be associated with diseases of the liver, gastrointestinal tract (for example, ulcerative colitis, Crohn's disease), kidneys (for example, Fanconi syndrome), insufficient or inadequate nutrition, and neoplasms. Early diagnosis and timely treatment can prevent the development and progression of symptoms of the disease.

This study makes it possible to comprehensively determine the concentration in the blood of standard and non-proteinogenic amino acids and their derivatives and assess the state of amino acid metabolism.

Alanine (A.L.A.) can be synthesized in the human body from other amino acids. It is involved in the process of gluconeogenesis in the liver. According to some data, increased levels of alanine in the blood are associated with increased blood pressure, body mass index, etc.

Arginine (A.R.G.) Depending on the age and functional state of the body, it is classified as semi-essential amino acids. Due to the immaturity of enzyme systems, premature babies are not capable of forming it, and therefore need an external source of this substance. An increased need for arginine occurs during stress, surgical treatment, and injuries. This amino acid is involved in cell division, wound healing, hormone release, and the formation of nitric oxide and urea.

Aspartic acid (A.S.P.) can be formed from citrulline and ornithine and is a precursor to some other amino acids. Aspartic acid and asparagine (ASN) participate in gluconeogenesis, the synthesis of purine bases, nitrogen metabolism, and the function of ATP synthetase. In the nervous system, asparagine plays the role of a neurotransmitter.

Citrulline (CIT) can be formed from ornithine or arginine and is an important component of the urea cycle in the liver (ornithine cycle). Citrulline is a component of filaggrin, histones, and plays a role in autoimmune inflammation in rheumatoid arthritis.

Glutamic acid (G.L.U.) – a non-essential amino acid that is of great importance in nitrogen metabolism. Free glutamic acid is used in the food industry as a flavor enhancer. Glutamic acid and glutamate are important excitatory neurotransmitters in the nervous system. Decreased glutamate release is observed in classic phenylketonuria.

Glycine (GLY) is a non-essential amino acid that can be formed from serine under the influence of pyridoxine (vitamin B6). It takes part in the synthesis of proteins, porphyrins, purines and is an inhibitory transmitter in the central nervous system.

Methionine (MET) – an essential amino acid, the maximum content of which is determined in eggs, sesame seeds, cereals, meat, and fish. Homocysteine ​​can be formed from it. Methionine deficiency leads to the development of steatohepatitis.

Ornithine (ORN) is not encoded by human DNA and is not involved in protein synthesis. This amino acid is formed from arginine and plays a key role in the synthesis of urea and the removal of ammonia from the body. Preparations containing ornithine are used to treat cirrhosis and asthenic syndrome.

Phenylalanine (PHE) – an essential amino acid, which is a precursor of tyrosine, catecholamines, melanin. A genetic defect in phenylalanine metabolism leads to the accumulation of the amino acid and its toxic products and the development of aminoacidopathy - phenylketonuria. The disease is associated with mental and physical developmental disorders and seizures.

Tyrosine (TYR) enters the body with food or is synthesized from phenylalanine. It is a precursor of neurotransmitters (dopamine, norepinephrine, adrenaline) and the pigment melanin. With genetic disorders of tyrosine metabolism, tyrosinemia occurs, which is accompanied by damage to the liver, kidneys and peripheral neuropathy. An important differential diagnostic value is the absence of an increase in the level of tyrosine in the blood during phenylketonuria, in contrast to some other pathological conditions.

Valine (VAL), leucine (LEU) And isoleucine (ILEU)– essential amino acids, which are important sources of energy in muscle cells. With fermentopathies that disrupt their metabolism and lead to the accumulation of these amino acids (especially leucine), “maple syrup disease” (leucinosis) occurs. The pathognomonic sign of this disease is the sweet odor of urine, which resembles maple syrup. Symptoms of aminoacidopathy occur early in life and include vomiting, dehydration, lethargy, hypotension, hypoglycemia, seizures and opisthotonus, ketoacidosis and central nervous system pathology. The disease often ends in death.

Hydroxyproline (HPRO) is formed by the hydroxylation of proline under the influence of vitamin C. This amino acid ensures the stability of collagen and is its main component. With a deficiency of vitamin C, the synthesis of hydroxyproline is disrupted, the stability of collagen decreases and damage to the mucous membranes occurs - symptoms of scurvy.

Serin (SER) is part of almost all proteins and is involved in the formation of the active centers of many enzymes in the body (for example, trypsin, esterases) and the synthesis of other non-essential amino acids.

Glutamine (GLN) is a partially replaceable amino acid. The need for it increases significantly with injuries, some gastrointestinal diseases, and intense physical activity. It takes part in nitrogen metabolism, purine synthesis, regulation of acid-base balance, and performs a neurotransmitter function. This amino acid accelerates the healing and recovery processes after injuries and surgeries.

Gamma-aminobutyric acid (GABA) synthesized from glutamine and is the most important inhibitory neurotransmitter. GABA drugs are used to treat various neurological disorders.

Beta-aminoisobutyric acid (BAIBA) is a product of the metabolism of thymine and valine. An increase in its level in the blood is observed with a deficiency of beta-aminoisobutyrate-pyruvate aminotransferase, fasting, lead poisoning, radiation sickness and some neoplasms.

Alpha aminobutyric acid (AABA)– a precursor for the synthesis of ophthalmic acid, which is an analogue of glutathione in the lens of the eye.

Beta Alanine (BALA), unlike alpha-alanine, it is not involved in protein synthesis in the body. This amino acid is part of carnosine, which, as a buffer system, prevents the accumulation of acids in muscles during physical activity, reduces muscle pain after training, and accelerates recovery processes after injuries.

Histidine (HIS)– an essential amino acid, which is a precursor of histamine, is part of the active centers of many enzymes, is found in hemoglobin, and promotes tissue repair. A rare genetic defect of histidase causes histidinemia, which can cause hyperactivity, developmental delays, learning difficulties and, in some cases, mental retardation.

Threonine (THRE)– an essential amino acid necessary for protein synthesis and the formation of other amino acids.

1-methylhistidine (1MHIS) is a derivative of anserine. The concentration of 1-methylhistidine in the blood and urine correlates with the consumption of meat foods and increases with deficiency. An increase in the level of this metabolite occurs when there is a deficiency of carosinase in the blood and is observed in Parkinson's disease and multiple sclerosis.

3-methylhistidine (3MHIS) is a product of actin and myosin metabolism and reflects the level of protein breakdown in muscle tissue.

Proline (PRO) synthesized in the body from glutamate. Hyperprolinemia due to a genetic defect of enzymes or due to inadequate nutrition, increased levels of lactic acid in the blood, or liver disease can lead to seizures, mental fatigue and other neurological pathologies.

Lysine (LYS)– an essential amino acid that is involved in the formation of collagen and tissue repair, the function of the immune system, the synthesis of proteins, enzymes and hormones. Lack of glycine in the body leads to asthenia, memory loss and impaired reproductive functions.

Alpha Amino Adipic Acid (AAA)– an intermediate product of lysine metabolism.

Cysteine ​​(CYS) is an essential amino acid for children, the elderly and people with malabsorption of nutrients. In healthy people, this amino acid is synthesized from methionine. Cysteine ​​is part of the keratin of hair and nails, is involved in the formation of collagen, is an antioxidant, a precursor of glutathione and protects the liver from the damaging effects of alcohol metabolites. Cystine is a dimeric cysteine ​​molecule. With a genetic defect in the transport of cystine in the renal tubules and intestinal walls, cystinuria occurs, which leads to the formation of stones in the kidneys, ureters and bladder.

Cystathionine (CYST)– an intermediate product of cysteine ​​metabolism during its synthesis from homocysteine. With hereditary deficiency of the enzyme cystathionase or acquired hypovitaminosis B 6, the level of cystathionine in the blood and urine increases. This condition is described as cystathioninuria, which is benign without obvious pathological signs, but in rare cases it can manifest as intellectual deficit.

Cysteinic acid (CYSA) is formed during the oxidation of cysteine ​​and is a precursor of taurine.

Taurine (TAU) synthesized from cysteine ​​and, unlike amino acids, is a sulfonic acid containing a sulfonic group instead of a carboxyl group. Taurine is part of bile, is involved in the emulsification of fats, is an inhibitory neurotransmitter, improves reparative and energy processes, and has cardiotonic and hypotensive properties.

Amino acids and proteins are widely used in sports nutrition and are used to increase muscle mass. Vegetarians, due to the lack of animal protein in their diet, may experience a deficiency of some essential amino acids. This study allows us to assess the adequacy of such types of nutrition and, if necessary, make their correction.

What is the research used for?

• Diagnosis of hereditary and acquired diseases associated with disorders of amino acid metabolism;
• differential diagnosis of the causes of disorders of nitrogen metabolism, removal of ammonia from the body;
• monitoring compliance with diet therapy and treatment effectiveness;
• nutritional status assessment and dietary modification.

When is the study scheduled?

• If there is a suspicion of a violation of amino acid metabolism in children, including newborns (vomiting, diarrhea, metabolic acidosis, special smell and coloring of diapers, impaired mental development);
• with hyperammonemia (increased levels of ammonia in the blood);
• with a burdened family history, the presence of congenital aminoacidopathies in relatives;
• when monitoring compliance with dietary recommendations, treatment effectiveness;
• when examining athletes (for example, bodybuilders) who consume sports nutrition (proteins and amino acids);
• when examining vegetarians.

What do the results mean?

• Alanine (ALA):

• Arginine (ARG):

• Aspartic Acid (ASP):

• Citrulline (CIT):

• Glutamic acid (GLU):

• Glycine (GLY)

• Methionine (MET)

• Ornithine (ORN)

• Phenylalanine (PHE)

• Tyrosine (TYR)

• Valin (VAL)

• Leucine (LEU)

• Isoleucine (ILEU)

• Hydroxyproline (HPRO)

• Serin (SER)

• Asparagine (ASN)

• Alpha-aminoadipic acid (AAA)

• Glutamine (GLN)

• Beta-alanine (BALA): 0 - 5 µmol/l.
• Taurine (TAU)

• Histidine (HIS)

• Threonine (THRE)

• 1-methylhistidine (1MHIS)

• 3-methylhistidine (3MHIS)

• Gamma-aminobutyric acid (GABA)

• Beta-aminoisobutyric acid (BAIBA)

• Alpha-aminobutyric acid (AABA): 0 - 40 µmol/l.
• Proline (PRO)

• Cystathionine (CYST): 0 - 0.3 µmol/l.
• Lysine (LYS)

• Cystine (CYS)

• Cysteinic acid (CYSA): 0.

Interpretation of results is carried out taking into account age, nutritional habits, clinical condition and other laboratory data.

An increase in the total level of amino acids in the blood is possible with:

• eclampsia;
• impaired fructose tolerance;
• diabetic ketoacidosis;
• renal failure;
• Reye's syndrome.

A decrease in the total level of amino acids in the blood can occur when:

• hyperfunction of the adrenal cortex;
• fever;
• Hartnup's disease;
• Huntington's chorea;
• inadequate nutrition, fasting (kwashiorkore);
• malabsorption syndrome in severe diseases of the gastrointestinal tract;
• hypovitaminosis;
• nephrotic syndrome;
• pappataci fever (mosquito, phlebotomy);
• rheumatoid arthritis.

Primary aminoacidopathies

Promotion arginine, glutamine– arginase deficiency.

Promotion arginine succinate, glutamine– arginosuccinase deficiency.

Promotion citrulline, glutamine– citrullinemia.

Promotion cystine, ornithine, lysine– cystinuria.

Promotion valine, leucine, isoleucine– maple syrup disease (leucinosis).

Promotion phenylalanine– phenylketonuria.

Promotion tyrosine– tyrosinemia.

Secondary aminoacidopathies

Promotion glutamine– hyperammonemia.

Promotion alanine– lactic acidosis (lactic acidosis).

Promotion glycine– organic aciduria.

Promotion tyrosine– transient tyrosinemia in newborns.

Literature

• Part 8. Amino Acids. In: Scriver CR, Beaudet AL, Valle D, Sly WS, Childs B, Kinzler KW, Vogelstein B, eds. The Metabolic and Molecular Bases of Inherited Disease. 8th ed. New York, NY: McGraw-Hill, Inc; 2001;1665-2105.
• Part IV. Disorders of amino acid metabolism and transport. Fernandes J, Saudubray J-M, Van den Berghe G, eds. Inborn Metabolic Diseases Diagnosis and Treatment. 3rd ed. New York, NY: Springer; 2000;169-273.
• Part 2. Disorders of amino acid metabolism. Nyhan WL, Barshop BA, Ozand PT, eds. Atlas of Metabolic Diseases. 2nd ed. New York, NY: Oxford University Press Inc; 2005;109-189.
• Blau N, Duran M, Blaskovics ME, Gibson KM, eds. Physician’s Guide to the Laboratory Diagnosis of Metabolic Diseases. 2nd ed. New York, NY: Springer; 2003.
• Human Metabolome Database. Access mode: http://www.hmdb.ca/

At the Doctor Nearby clinic, it makes it possible to determine the content of these beneficial compounds in the body. Today, more than a hundred amino acids are known, but only 32 of them provide protein production. Depending on the possibility of creation in the body, amino acids are divided into essential and non-essential.

Key essential amino compounds:

• Valin;
• Arginine;
• Methionine;
• Phenylalanine;
• Tryptophan;
• Lysine and others.

Key replaceable substances:

• Alanine;
• Acylcarnitine;
• Citrulline;
• Glycine;
• Glutamate;
• Asparagine and others.

These acids are needed for many metabolic processes in the human body. If the enzymes involved in the transformation are disrupted, the concentration of certain amino acids may increase. This negatively affects the functioning of various organs and systems. The pathology of amino acid metabolism can be hereditary or acquired.

Birth defects are inherited in an autosomal recessive manner and usually occur in early childhood. This group of diseases is caused by a lack of transport protein molecules and enzymes involved in the metabolism of amino acids. They can manifest themselves with various symptoms, ranging from minor malaise to severe general impairment, vomiting, coma, delayed physical and intellectual development, osteoporosis and osteomalacia.

In many diseases of internal organs, acquired or secondary metabolic disorders are observed.

Pathological conditions accompanied by a violation of amino acid metabolism include diseases of the digestive system (,), urinary system (Fanconi syndrome), malignant neoplasms, and so on.

Prevention and effective treatment of these diseases makes it possible to slow down their progression and normalize metabolism.

To diagnose these pathological processes, doctors at the Doctor Nearby clinic prescribe a blood test for 32 indicators. This diagnostic procedure makes it possible to conduct a comprehensive assessment of the content of these substances and their derivatives in the blood, as well as determine the state of amino acid metabolism in the body. You can find out the price of a blood test for amino acids on the company’s official website.

Preparing for analysis

To obtain reliable research results, you must adhere to certain preparation rules. During the day before donating blood for analysis, you should stop drinking alcoholic beverages.

For 8 hours before taking biological material, the patient must refuse to eat. During this period, you are only allowed to drink purified water without gas.

In consultation with your doctor, you should stop taking medications the day before donating blood, if possible. For half an hour before the test, the patient is advised to refrain from smoking and try to avoid psycho-emotional and physical overload.

Indications for the study

Doctors at the Doctor Nearby clinic advise taking an amino acid test in the following situations:

• Suspicion of congenital and acquired disorders of amino acid metabolism;
• Differential diagnosis of the pathology of the metabolism of nitrogenous bases and their elimination (with an increase in the level of ammonia in the body);
• Monitoring the effectiveness of diet therapy and therapeutic measures;
• Comprehensive assessment of nutritional status and diet correction;
• Diagnosis of severe congenital disorders of amino acid metabolism in childhood in the presence of vomiting, metabolic acidosis, mental retardation and other characteristic signs;
• Screening in persons with a complicated medical history (congenital disorders of amino acid metabolism in relatives).
• Examination of people involved in professional sports (weightlifters, bodybuilders), especially those taking protein supplements.
• Assessment of amino acid metabolism in vegetarians.

How the research works

Interpretation of amino acid test results

Deciphering the analysis takes one business day. After this, the patient receives a conclusion about the results of the analysis, with which he goes to his doctor. When interpreting the results, the age of the patients, dietary habits, the presence of symptoms of any diseases and other laboratory data are taken into account.

Nomenclature of the Ministry of Health of the Russian Federation (Order No. 804n): B03.016.025.004 "Complex determination of amino acid concentrations (32 indicators) by high performance liquid chromatography in urine"

Biomaterial: Single urine (average portion)

Completion time (in the laboratory): 5 w.d. *

Description

The study is aimed at determining the level of amino acids in urine, their derivatives, and assessing the state of amino acid metabolism.

Amino acids- organic substances involved in protein synthesis. Whenever possible, nonessential and essential amino acids can be synthesized in the body (they are not synthesized in the body and must be supplied with food).

Essential amino acids include: arginine, valine, isoleucine, leucine, methionine, phenylalanine, histidine, threonine, tryptophan.

Essential amino acids include: alanine, aspartic acid, aspartate, glycine, glutamic acid, glutamine, tyrosine, cysteine.

If enzymes are defective at different stages of conversion, an accumulation of amino acids and their conversion products may occur, which can have a negative effect on the body.
There are primary (congenital) and secondary (acquired) disorders of amino acid metabolism. Congenital diseases are caused by a deficiency of enzymes and/or transport proteins that are associated with the metabolism of amino acids.

Clinical manifestations of metabolic disorders can be manifested by various symptoms. Acquired amino acid disorders are associated with diseases of the liver, gastrointestinal tract, kidneys, insufficient or inadequate nutrition, and neoplasms.

The study includes:

1-methylhistidine
3-methylhistidine
a-aminoadipic acid
a-aminobutyric acid
b-alanine
b-aminoisobutyric acid
y-aminobutyric acid
Alanin
Arginine
Asparagine
Aspartic acid
Valin
Hydroxyproline
Histidine
Glycine
Glutamine
Glutamic acid
Isoleucine
Leucine
Lysine
Methionine
Ornithine
Proline
Serin
Taurine
Tyrosine
Threonine
Tryptophan
Phenylalanine
Cystathionine
Cystine
Citrulline

Indications for use

• diagnosis of hereditary and acquired diseases associated with disorders of amino acid metabolism;
• assessment of nutritional status;
• Monitoring diet compliance and treatment effectiveness.

Preparing for the study

On the eve of the test, it is not recommended to eat vegetables and fruits that can change the color of urine (beets, carrots, etc.), and not to take diuretics. Before collecting urine, it is necessary to perform a thorough hygienic toilet of the genital organs. Women are not recommended to take a urine test during menstruation.

Interpretation of results/Information for specialists

Interpretation of results is carried out taking into account age, nutritional habits, clinical condition and other laboratory data.

Interpretation of results:

Increasing reference values:
Eclampsia, impaired fructose tolerance, diabetic ketoacidosis, renal failure, Reye's syndrome, phenylketonuria.

Reduction of reference values:
Huntington's chorea, inadequate nutrition, fasting (kwashiorkor), malabsorption syndrome in severe diseases of the gastrointestinal tract; hypovitaminosis, nephrotic syndrome, pappataci fever (mosquito, phlebotomy), rheumatoid arthritis.

Most often ordered with this service

* The website indicates the maximum possible period for completing the study. It reflects the time it takes to complete the study in the laboratory and does not include the time for delivery of the biomaterial to the laboratory.
The information provided is for reference only and is not a public offer. For up-to-date information, contact the Contractor's medical center or call center.

Amino acids- organic compounds that are the main component of proteins (proteins). Disorders of amino acid metabolism are the cause of many diseases (liver and kidneys). Amino acid analysis (urine and blood) is the primary means of assessing dietary protein absorption as well as metabolic imbalances that underlie many chronic disorders.

The biomaterial for a comprehensive analysis of amino acids in the Hemotest Laboratory can be blood or urine.

The following essential amino acids are studied: alanine, arginine, aspartic acid, citrulline, glutamic acid, glycine, methionine, ornithine, phenylalanine, tyrosine, valine, leucine, isoleucine, hydroxyproline, serine, asparagine, α-aminoadipic acid, glutamine, β-alanine, taurine, histidine, threonine, 1-methylhistidine, 3-methylhistidine, γ-aminobutyric acid, β-aminoisobutyric acid, α-aminobutyric acid, proline, cystathionine, lysine, cystine, cysteic acid.

Alanin – an important source of energy for the brain and central nervous system; strengthens the immune system by producing antibodies; actively participates in the metabolism of sugars and organic acids. It can be a raw material for the synthesis of glucose in the body, making it an important source of energy and a regulator of blood sugar levels.

Decreased concentration: chronic kidney disease, ketotic hypoglycemia.

Increased concentrations: hyperalaninemia, citrullinemia (moderate increase), Cushing's disease, gout, hyperorotininemia, histidemia, pyruvate carboxylase deficiency, lysinuric protein intolerance.

Arginine is a conditionally nonessential amino acid. Participates in the cycle of transamination and removal of final nitrogen from the body, that is, the breakdown product of waste proteins. The body’s ability to create urea and cleanse itself of protein waste depends on the power of the cycle (ornithine - citrulline - arginine).

Decreased concentration: 3 days after abdominal surgery, chronic renal failure, rheumatoid arthritis.

Increased concentration: hyperargininemia, in some cases type II hyperinsulinemia.

Aspartic acid is part of proteins, plays an important role in the reactions of the urea cycle and transamination, and participates in the biosynthesis of purines and pyrimidines.

Decreased concentration: 1 day after surgery.

Increased concentration: urine – dicarboxyl aminoaciduria.

Citrulline increases energy supply, stimulates the immune system, and is converted into L-arginine in metabolic processes. Neutralizes ammonia, which damages liver cells.

Increased citrulline concentration: citrullinemia, liver disease, ammonium intoxication, pyruvate carboxylase deficiency, lysinuric protein intolerance.

Urine - citrullinemia, Hartnup disease, argininosuccinate aciduria.

Glutamic acid is a neurotransmitter that transmits impulses in the central nervous system. Plays an important role in carbohydrate metabolism and promotes the penetration of calcium through the blood-brain barrier. Decreased concentration: histidinemia, chronic renal failure.

Increased concentration: pancreatic cancer, gout, glutamic acid, aciduria, rheumatoid arthritis. Urine – dicarboxyl aminoaciduria.

Glycine is a regulator of metabolism, normalizes the processes of excitation and inhibition in the central nervous system, has an anti-stress effect, and increases mental performance.

Decreased concentration: gout, diabetes.

Increased concentration: septicemia, hypoglycemia, type 1 hyperammonemia, severe burns, fasting, propionic acidemia, methylmalonic acidemia, chronic renal failure. Urine – hypoglycemia, cystinuria, Hartnup disease, pregnancy, hyperprolinemia, glycinuria, rheumatoid arthritis.

Methionine an essential amino acid that helps process fats, preventing their deposition in the liver and artery walls. The synthesis of taurine and cysteine ​​depends on the amount of methionine in the body. Promotes digestion, provides detoxification processes, reduces muscle weakness, protects against radiation exposure, is useful for osteoporosis and chemical allergies.

Decreased concentration: homocystinuria, protein nutrition disorder.

Increased concentration: carcinoid syndrome, homocystinuria, hypermethioninemia, tyrosinemia, severe liver disease.

Ornithine Helps release growth hormone, which helps burn fat in the body. Necessary for the immune system, participates in detoxification processes and restoration of liver cells.

Decreased concentration: carcinoid syndrome, chronic renal failure.

Increased concentration: spiral atrophy of the choroid and retina, severe burns, hemolysis.

Phenylalanine - an essential amino acid, in the body it can be converted into tyrosine, which, in turn, is used in the synthesis of two main neurotransmitters: dopamine and norepinephrine. Affects mood, reduces pain, improves memory and learning ability, suppresses appetite.

Increased concentration: transient tyrosinemia of newborns, hyperphenylalaninemia, sepsis, hepatic encephalopathy, viral hepatitis, phenylketonuria.

Tyrosine is a precursor to the neurotransmitters norepinephrine and dopamine. Participates in the regulation of mood; a lack of tyrosine leads to a deficiency of norepinephrine, which leads to depression. Suppresses appetite, reduces fat deposits, promotes the production of melatonin and improves the functions of the adrenal glands, thyroid gland and pituitary gland, and is also involved in phenylalanine metabolism. Thyroid hormones are formed by the addition of iodine atoms to tyrosine.

Decreased concentration: polycystic kidney disease, hypothermia, phenylketonuria, chronic renal failure, carcinoid syndrome, myxedema, hypothyroidism, rheumatoid arthritis.

Increased concentration: hypertyrosinemia, hyperthyroidism, sepsis.

Valin an essential amino acid that has a stimulating effect. Necessary for muscle metabolism, repair of damaged tissues and for maintaining normal nitrogen metabolism in the body, can be used by muscles as a source of energy.

Decreased concentration: hyperinsulinism, hepatic encephalopathy.

Increased concentration: ketoaciduria, hypervalinemia, insufficient protein nutrition, carcinoid syndrome, acute starvation.

Leucine and isoleucine - protect muscle tissue and are sources of energy, and also contribute to the restoration of bones, skin, and muscles. Able to lower blood sugar levels and stimulate the release of growth hormone.

Decreased concentration: acute starvation, hyperinsulinism, hepatic encephalopathy.

Increased concentration: ketoaciduria, obesity, fasting, viral hepatitis.

Hydroxyproline found in the tissues of almost the entire body, it is part of collagen, which accounts for most of the protein in the body of mammals. The synthesis of hydroxyproline is impaired by vitamin C deficiency.

Increased concentration: hydroxyprolinemia, uremia, liver cirrhosis.

Serin belongs to the group of non-essential amino acids, participates in the formation of active centers of a number of enzymes, ensuring their function. It is important in the biosynthesis of other non-essential amino acids: glycine, cysteine, methionine, tryptophan. Serine is the starting product of the synthesis of purine and pyrimidine bases, sphingolipids, ethanolamine, and other important metabolic products.

Decreased concentration: phosphoglycerate dehydrogenase deficiency, gout.

Increased serine concentration: protein intolerance. Urine – burns, Hartnup disease.

Asparagine necessary to maintain balance in the processes occurring in the central nervous

system; prevents both excessive excitation and excessive inhibition, participates in the processes of amino acid synthesis in the liver.

Increased concentration: burns, Hartnup disease, cystinosis.

Alpha-aminoadipic acid - metabolite of the main biochemical pathways of lysine.

Increased concentration: hyperlysinemia, alpha-aminoadipic aciduria, alpha-ketoadipic aciduria, Reye's syndrome.

Glutamine performs a number of vital functions in the body: participates in the synthesis of amino acids, carbohydrates, nucleic acids, cAMP and c-GMP, folic acid, enzymes that carry out redox reactions (NAD), serotonin, n-aminobenzoic acid; neutralizes ammonia; converted to aminobutyric acid (GABA); is able to increase the permeability of muscle cells to potassium ions.

Decreased glutamine concentration: rheumatoid arthritis

Increased concentrations: Blood - Hyperammonemia caused by the following reasons: hepatic coma, Reye's syndrome, meningitis, cerebral hemorrhage, urea cycle defects, ornithine transcarbamylase deficiency, carbamoyl phosphate synthase deficiency, citrullinemia, arginine succinic aciduria, hyperornithinemia, hyperammonemia, homocitrullinemia (HHH syndrome), in some cases hyperlysemia type 1, lysinuric protein intolerance. Urine – Hartnup disease, generalized aminoaciduria, rheumatoid arthritis.

β-alanine – is the only beta amino acid, formed from dihydrouracil and carnosine.

Increased concentration: hyper-β-alaninemia.

Taurine - promote the emulsification of fats in the intestines, has anticonvulsant activity, has a cardiotropic effect, improves energy processes, stimulates reparative processes in dystrophic diseases and processes accompanied by metabolic disorders of eye tissue, helps to normalize the function of cell membranes and improve metabolic processes.

Decreased taurine concentration: Blood - Manic-depressive syndrome, depressive neuroses

Increased taurine concentration: Urine - Sepsis, hyper-β-alaninemia, folic acid deficiency (B9), first trimester of pregnancy, burns.

Histidine is part of the active centers of many enzymes and is a precursor in the biosynthesis of histamine. Promotes tissue growth and repair. Contained in large quantities in hemoglobin; used in the treatment of rheumatoid arthritis, allergies, ulcers and anemia. A lack of histidine can cause hearing loss.

Decreased histidine concentration: Rheumatoid arthritis

Increased histidine concentration: Histidinemia, pregnancy, Hartnup disease, generalized

no aminoaciduria.

Threonine is an essential amino acid that helps maintain normal protein metabolism in the body, is important for the synthesis of collagen and elastin, helps the liver, participates in fat metabolism, and stimulates the immune system.

Decreased threonine concentration: Chronic renal failure, rheumatoid arthritis.

Increased threonine concentration: Hartnup disease, pregnancy, burns, hepatolenticular degeneration.

1-methylhistidine main derivative of anserine. The enzyme carnosinase converts anserine into β-alanine and 1-methylhistidine. High levels of 1-methylhistidine tend to inhibit the enzyme carnosinase and increase anserine concentrations. Decreased carnosinase activity also occurs in patients with Parkinson's disease, multiple sclerosis, and post-stroke patients. Vitamin E deficiency can lead to 1-methylhistidinuria due to increased oxidative effects in skeletal muscle.

Increased concentration: chronic renal failure, meat diet.

3-methylhistidine is an indicator of the level of protein breakdown in muscles.

Decreased concentration: fasting, diet.

Increased concentration: chronic renal failure, burns, multiple injuries.

Gamma-aminobutyric acid - found in the central nervous system and takes part in neurotransmitter and metabolic processes in the brain. GABA receptor ligands are considered as potential agents for the treatment of various mental and central nervous system disorders, which include Parkinson’s and Alzheimer’s diseases, sleep disorders (insomnia, narcolepsy), and epilepsy. Under the influence of GABA, the energy processes of the brain are also activated, the respiratory activity of tissues increases, the utilization of glucose by the brain improves, and blood supply improves.

Beta (β) - aminoisobutyric acid - a non-protein amino acid is a product of the catabolism of thymine and valine. Increased concentration: various types of neoplasms, diseases accompanied by increased destruction of nucleic acids in tissues, Down syndrome, protein malnutrition, hyper-beta-alaninemia, beta-aminoisobutyric aciduria, lead poisoning.

Alpha (α) -aminobutyric acid is the main intermediate product of the biosynthesis of ophthalmic acid. Increased concentration: nonspecific aminoaciduria, fasting.

Proline - one of twenty proteinogenic amino acids, is part of all proteins of all organisms.

Decreased concentration: Huntington's chorea, burns

Increased concentration: Blood - hyperprolinemia type 1 (proline oxidase deficiency), hyperprolinemia type 2 (pyrroline-5-carboxylate dehydrogenase deficiency), protein malnutrition in newborns. Urine – hyperproliemia types 1 and 2, Joseph syndrome (severe prolinuria), carcinoid syndrome, iminoglycinuria, Wilson-Konovalov disease (hepatolenticular degeneration).

Cystathionine - sulfur-containing amino acid, participates in the biosynthesis of cysteine, methionine and serine.

Lysine is an essential amino acid that is part of almost any protein, is necessary for growth, tissue repair, the production of antibodies, hormones, enzymes, albumins, has an antiviral effect, maintains energy levels, is involved in the formation of collagen and tissue repair, improves the absorption of calcium from the blood and its transport into bone tissue.

Decreased concentration: carcinoid syndrome, lysinuric protein intolerance.

Increased concentrations: Blood – hyperlysinemia, glutaric acidemia type 2. Urine – cystinuria, hyperlysinemia, first trimester of pregnancy, burns.

Cystine in the body is an important part of proteins such as immunoglobulins, insulin and somatostatin, and strengthens connective tissue. Decreased cystine concentration: protein starvation, burns. Increased cystine concentration: Blood - sepsis, chronic renal failure. Urine – Cystinosis, cystinuria, cystinlysinuria, first trimester of pregnancy.

Cysteinic acid - sulfur-containing amino acid. Intermediate product of cysteine ​​and cystine metabolism. Takes part in transamination reactions and is one of the precursors of taurine.

Only half of the necessary amino acids are synthesized in the human body, and the remaining amino acids - essential (arginine, valine, histidine, isoleucine, leucine, lysine, methionine, threonine, tryptophan, phenylalanine) - must come from food. Exclusion of any essential amino acid from the diet leads to the development of a negative nitrogen balance, clinically manifested by dysfunction of the nervous system, muscle weakness and other signs of metabolic and energy pathology.

Indications for the purpose of analysis:

• Diagnosis of diseases associated with amino acid metabolism disorders.
• Assessment of the condition of the human body.

General preparation rules must be followed. Blood must be donated for testing on an empty stomach. At least 8 hours must pass between the last meal and blood collection.

Collect an average morning portion of urine for testing.

Amino acids- these are organic compounds that are building materials for proteins and muscle tissue. Disorders of amino acid metabolism are the cause of many diseases (liver and kidneys). Amino acid analysis (urine and blood) is the primary means of assessing dietary protein absorption as well as metabolic imbalances that underlie many chronic disorders.

Composition of the study:

• 1-methylhistidine (1MHIS).
• 3-methylhistidine (3MHIS).
• a-aminoadipic acid (AAA).
• a-aminobutyric acid (AABA).
• b-alanine (BALA).
• b-aminoisobutyric acid (BAIBA).
• y-aminobutyric acid (GABA).
• Alanine (Ala).
• Arginine (Arg).
• Asparagine (ASN).
• Aspartic acid (Asp).
• Valin (Val).
• Hydroxyproline (HPRO).
• Histidine (HIS).
• Glycine (Gly).
• Glutamine (GLN).
• Glutamic acid (Glu).
• Isoleucine (ILEU).
• Leucine (LEU).
• Lysine (LYS).
• Methionine (Met).
• Ornithine (Orn).
• Proline (PRO).
• Serine (SER).
• Taurine (TAU).
• Tyrosine (Tyr).
• Threonine (THRE).
• Phenylalanine (Phe).
• Cystathionine (CYST).
• Cysteinic acid (CYSA).
• Cystine (CYS).
• Citrulline (Cit).

Arginine- is a conditionally replaceable amino acid, that is, it must constantly be supplied to the body with food. Arginine is involved in the production of nitric oxide, helps accelerate the synthesis of growth hormone and other hormones, accelerates healing and strengthens blood vessels. It is present in the body in free form and as part of proteins. Arginine underlies the synthesis of ornithine.

Ornithine- stimulates the release of insulin and growth hormone. It helps protect the liver from the effects of toxic substances, and also stimulates the regeneration and restoration of liver cells. The extremely important role of ornithine is associated with its participation in the urinary cycle, which is necessary for the excretion of ammonia. Ammonia is formed during the breakdown of proteins and is a toxic substance for the body. Ornithine is involved in its processing to form urea. Urea also has a toxic effect and increases nervous excitability. Thanks to ornithine, these toxins are eliminated from the body.

Aspartic acid- participates in transamination reactions and the urea cycle.

Citrulline- stimulates ammonia detoxification, supports immunity. It plays an important role in the metabolic processes of the body.

Glutamic acid- affects the absorption of calcium, carbohydrate metabolism and is an important neurotransmitter.

Glycine- regulates metabolism, improves brain activity.

Meteonine- prevents the deposition of fats on the walls of blood vessels and in the liver, improves digestion, protects the body from the effects of toxic substances and radiation.

Phenylalanine- participates in the formation of neurotransmitters, norepinephrine and dopamine, improves mental activity, normalizes appetite.

Tyrosine- normalizes the activity of the pituitary gland, thyroid gland, adrenal glands, norepinephrine and dopamine are synthesized from it.

Valin- regulates muscle activity, regenerates damaged tissue. Necessary for maintaining normal nitrogen metabolism in the body, can be used by muscles as a source of energy.

Leucine and isoleucine- participate in the restoration processes of bones, muscles, skin, activate the production of growth hormone, reduce blood sugar levels and are sources of energy. Decreased concentration: acute starvation, hyperinsulinism, hepatic encephalopathy. Increased concentration: ketoaciduria, obesity, fasting, viral hepatitis.

Hydroxyproline- found in the tissues of almost the entire body, it is part of collagen, which accounts for most of the protein in the body of mammals. Hydroxyproline synthesis is impaired in vitamin C deficiency.

Increased concentration: hydroxyprolinemia, uremia, liver cirrhosis.

Serin- belongs to the group of non-essential amino acids, participates in the formation of active centers of a number of enzymes, ensuring their function. Important in the biosynthesis of other non-essential amino acids: glycine, cysteine, methionine, tryptophan. Serine is the initial product of the synthesis of purine and pyrimidine bases, sphingolipids, ethanolamine, and other important metabolic products.

Decreased concentration: phosphoglycerate dehydrogenase deficiency, gout. Increased serine concentration: protein intolerance. Urine - burns, Hartnup's disease.

Asparagine- necessary to maintain balance in the processes occurring in the central nervous system; prevents both excessive excitation and excessive inhibition, participates in the processes of amino acid synthesis in the liver. Increased concentration: burns, Hartnup disease, cystinosis.

Alpha-aminoadipic acid- metabolite of the main biochemical pathways of lysine. Increased concentration: hyperlysinemia, alpha-aminoadipic aciduria, alpha-ketoadipic aciduria, Reye's syndrome.

Glutamine- performs a number of vital functions in the body: participates in the synthesis of amino acids, carbohydrates, nucleic acids, cAMP and c-GMP, folic acid, enzymes that carry out redox reactions (NAD), serotonin, n-aminobenzoic acid; neutralizes ammonia; converted to aminobutyric acid (GABA); is able to increase the permeability of muscle cells to potassium ions.

Decreased glutamine concentration: rheumatoid arthritis

Increased concentration: blood - hyperammonemia caused by the following reasons: hepatic coma, Reye's syndrome, meningitis, cerebral hemorrhage, urea cycle defects, deficiency of ornithine transcarbamylase, carbamoyl phosphate synthase, citrullinemia, arginine succinic aciduria, hyperornithinemia, hyperammonemia, homocitrullinemia (HHH syndrome), in some cases hyperlysemia type 1, lysinuric protein intolerance. Urine - Hartnup disease, generalized aminoaciduria, rheumatoid arthritis.

Beta-alanine- is the only beta amino acid, formed from dihydrouracil and carnosine. Increased concentration: hyper-β-alaninemia.

Taurine- promote the emulsification of fats in the intestines, has anticonvulsant activity, has a cardiotropic effect, improves energy processes, stimulates reparative processes in dystrophic diseases and processes accompanied by metabolic disorders of eye tissue, helps to normalize the function of cell membranes and improve metabolic processes.

Decreased taurine concentration: blood - manic-depressive syndrome, depressive neuroses.

Increased taurine concentration: urine - sepsis, hyper-β-alaninemia, folic acid deficiency (B 9), first trimester of pregnancy, burns.

Histidine- is part of the active centers of many enzymes and is a precursor in the biosynthesis of histamine. Promotes tissue growth and repair. Contained in large quantities in hemoglobin; used in the treatment of rheumatoid arthritis, allergies, ulcers and anemia. A lack of histidine can cause hearing loss.

Decreased histidine concentration: rheumatoid arthritis. Increased histidine concentration: histidinemia, pregnancy, Hartnup disease, generalized aminoaciduria.

Threonine is an essential amino acid that helps maintain normal protein metabolism in the body, is important for the synthesis of collagen and elastin, helps the liver, participates in fat metabolism, and stimulates the immune system.

Decreased threonine concentration: chronic renal failure, rheumatoid arthritis. Increased threonine concentration: Hartnup disease, pregnancy, burns, hepatolenticular degeneration.

1-methylhistidine is the main derivative of anserine. The enzyme carnosinase converts anserine into β-alanine and 1-methylhistidine. High levels of 1-methylhistidine tend to inhibit the enzyme carnosinase and increase anserine concentrations. Decreased carnosinase activity also occurs in patients with Parkinson's disease, multiple sclerosis, and post-stroke patients. Vitamin E deficiency can lead to 1-methylhistidinuria due to increased oxidative effects in skeletal muscle.

Increased concentration: chronic renal failure, meat diet.

3-methylhistidine is an indicator of the level of protein breakdown in muscles.

Decreased concentration: fasting, diet. Increased concentration: chronic renal failure, burns, multiple injuries.

Gamma-aminobutyric acid- found in the central nervous system and takes part in neurotransmitter and metabolic processes in the brain. GABA receptor ligands are considered as potential agents for the treatment of various mental and central nervous system disorders, which include Parkinson’s and Alzheimer’s diseases, sleep disorders (insomnia, narcolepsy), and epilepsy. Under the influence of GABA, the energy processes of the brain are also activated, the respiratory activity of tissues increases, the utilization of glucose by the brain improves, and blood supply improves.

Beta-aminoisobutyric (β)- aminoisobutyric acid is a non-protein amino acid, which is a product of the catabolism of thymine and valine. Increased concentration: various types of neoplasms, diseases accompanied by increased destruction of nucleic acids in tissues, Down syndrome, protein malnutrition, hyper-beta-alaninemia, beta-aminoisobutyric aciduria, lead poisoning.

Alpha-aminobutyric (α)- aminobutyric acid is the main intermediate product of the biosynthesis of ophthalmic acid. Increased concentration: nonspecific aminoaciduria, fasting.

Proline- one of twenty proteinogenic amino acids, is part of all proteins of all organisms.

Decreased concentration: Huntington's chorea, burns.

Increased concentration: blood - hyperprolinemia type 1 (proline oxidase deficiency), hyperprolinemia type 2 (pyrroline-5-carboxylate dehydrogenase deficiency), protein deficiency in newborns. Urine - hyperproliemia types 1 and 2, Joseph's syndrome (severe prolinuria), carcinoid syndrome, iminoglycinuria, Wilson-Konovalov disease (hepatolenticular degeneration).

Cystathionine- sulfur-containing amino acid, participates in the biosynthesis of cysteine, methionine and serine.

Lysine- this is an essential amino acid, which is part of almost any proteins, is necessary for growth, tissue repair, the production of antibodies, hormones, enzymes, albumins, has an antiviral effect, maintains energy levels, is involved in the formation of collagen and tissue repair, improves the absorption of calcium from the blood and its transport into bone tissue.

Decreased concentration: carcinoid syndrome, lysinuric protein intolerance.

Increased concentrations: blood - hyperlysinemia, glutaric acidemia type 2. Urine - cystinuria, hyperlysinemia, first trimester of pregnancy, burns.

Cystine in the body- is an important part of proteins such as immunoglobulins, insulin and somatostatin, strengthens connective tissue. Decreased cystine concentration: protein starvation, burns. Increased cystine concentrations: blood - sepsis, chronic renal failure. Urine - cystinosis, cystinuria, cystinlysinuria, first trimester of pregnancy.

Cysteinic acid- sulfur-containing amino acid. Intermediate product of cysteine ​​and cystine metabolism. Takes part in transamination reactions and is one of the precursors of taurine.

Only half of the necessary amino acids are synthesized in the human body, and the remaining amino acids - essential (arginine, valine, histidine, isoleucine, leucine, lysine, methionine, threonine, tryptophan, phenylalanine) - must come from food. The exclusion of any essential amino acid from the diet leads to the development of a negative nitrogen balance, clinically manifested by dysfunction of the nervous system, muscle weakness and other signs of metabolic and energy pathology.

It is impossible to overestimate the role of amino acids in the functioning of the body.

Indications:

• diagnosis of hereditary and acquired diseases associated with disorders of amino acid metabolism;
• differential diagnosis of the causes of disorders of nitrogen metabolism, removal of ammonia from the body;
• monitoring compliance with diet therapy and treatment effectiveness;
• nutritional status assessment and dietary modification.

Collect a strictly morning portion of urine, excreted immediately after sleep. Before collecting urine, it is necessary to perform a thorough hygienic toilet of the external genitalia. When urinating for the first time in the morning, release a small amount of urine (the first 1-2 seconds) into the toilet, then collect the entire portion of urine in a clean container without interrupting urination. Pour approximately 50 ml of urine into a sterile plastic container with a screw cap. When collecting urine, it is advisable not to touch the container to your body. It is necessary to deliver the container with urine to the medical office as soon as possible from the moment the biomaterial is taken.

Interpretation of results
Interpretation of results is carried out taking into account age, nutritional habits, clinical condition and other laboratory data.
Units of measurement - µmol/l.

1. 1-Methylhistidine

• <= 1 года: 17–419
• > 1 year before< 3 лет: 18–1629
• >= 3 years up<= 6 лет: 10–1476
• > 6 years up to<= 8 лет: 19–1435
• > 8 years up< 18 лет: 12–1549
• >= 18 years: 23–1339

• <= 1 года: 88–350
• > 1 year before< 3 лет: 86–330
• >= 3 years up<= 6 лет: 56–316
• > 6 years up to<= 8 лет: 77–260
• > 8 years up< 18 лет: 47–262
• >= 18 years: 70–246

• <= 30 дней: 0–299,7
• > 30 days before< 2 лет: 0–403,1
• >= 2 years before<= 11 лет: 0–211,1
• > 11 years up<= 17 лет: 0–167
• > 17 years: 0–146.7

• <= 1 года: 0–63
• > 1 year before< 3 лет: 0–56
• >= 3 years up<= 6 лет: 0–38
• > 6 years up to<= 8 лет: 0–30
• > 8 years up< 18 лет: 0–31
• >= 18 years: 0–19

• <= 1 года: 0–219
• > 1 year before< 3 лет: 0–92
• >= 3 years up<= 6 лет: 0–25
• > 6 years up to<= 8 лет: 0–25
• > 8 years up< 18 лет: 0–49
• >= 18 years: 0–52

• <= 1 года: 18–3137
• > 1 year before< 3 лет: 0–980
• >= 3 years up<= 6 лет: 15–1039
• > 6 years up to<= 8 лет: 24–511
• > 8 years up< 18 лет: 11–286
• >= 18 years: 0–301

• <= 1 года: 0–25
• > 1 year before< 3 лет: 0–13
• >= 3 years up<= 6 лет: 0–11
• > 6 years up to<= 8 лет: 0–6
• > 8 years up< 18 лет: 0–5
• >= 18 years: 0–5

• <= 1 года: 93–3007
• > 1 year before< 3 лет: 101–1500
• >= 3 years up<= 6 лет: 64–1299
• > 6 years up to<= 8 лет: 44–814
• > 8 years up< 18 лет: 51–696
• >= 18 years: 56–518

• <= 1 года: 10–560
• > 1 year before< 3 лет: 20–395
• >= 3 years up<= 6 лет: 14–240
• > 6 years up to<= 8 лет: 0–134
• > 8 years up< 18 лет: 0–153
• >= 18 years: 0–114

• <= 30 дней: 0–2100,3
• > 30 days before< 2 лет: 0–1328,9
• >= 2 years before<= 11 лет: 0–687,8
• > 11 years up<= 17 лет: 0–913,9
• > 17 years: 0–454.2

• <= 1 года: 0–64
• > 1 year before< 3 лет: 0–56
• >= 3 years up<= 6 лет: 0–30
• > 6 years up to<= 8 лет: 0–9
• > 8 years up< 18 лет: 0–11
• >= 18 years: 0–10

• <= 1 года: 11–211
• > 1 year before< 3 лет: 11–211
• >= 3 years up<= 6 лет: 0–139
• > 6 years up to<= 8 лет: 16–91
• > 8 years up< 18 лет: 0–75
• >= 18 years: 11–61

• <= 1 года: 0–2536
• > 1 year before< 3 лет: 0–89
• >= 3 years up<= 6 лет: 0–46
• > 6 years up to<= 8 лет: 0–19
• > 8 years up< 18 лет: 0–22
• >= 18 years: 0–15

• <= 1 года: 145–3833
• > 1 year before< 3 лет: 427–3398
• >= 3 years up<= 6 лет: 230–2635
• > 6 years up to<= 8 лет: 268–2147
• > 8 years up< 18 лет: 134–1983
• >= 18 years: 81–1128

• <= 1 года: 362–18614
• > 1 year before< 3 лет: 627–6914
• >= 3 years up<= 6 лет: 412–5705
• > 6 years up to<= 8 лет: 449–4492
• > 8 years up< 18 лет: 316–4249
• >= 18 years: 229–2989

• <= 30 дней: 0–2279,4
• > 30 days before< 2 лет: 0–4544,3
• >= 2 years before<= 11 лет: 0–1920,6
• > 11 years up<= 17 лет: 0–822
• > 17 years: 0–1756.2

• <= 1 года: 0–243
• > 1 year before< 3 лет: 12–128
• >= 3 years up<= 6 лет: 0–76
• > 6 years up to<= 8 лет: 0–39
• > 8 years up< 18 лет: 0–62
• >= 18 years: 0–34

• <= 1 года: 0–86
• > 1 year before< 3 лет: 0–78
• >= 3 years up<= 6 лет: 0–62
• > 6 years up to<= 8 лет: 0–34
• > 8 years up< 18 лет: 0–28
• >= 18 years: 0–22

• <= 1 года: 0–200
• > 1 year before< 3 лет: 15–167
• >= 3 years up<= 6 лет: 12–100
• > 6 years up to<= 8 лет: 13–73
• > 8 years up< 18 лет: 0–62
• >= 18 years: 0–51

• <= 1 года: 19–1988
• > 1 year before< 3 лет: 25–743
• >= 3 years up<= 6 лет: 14–307
• > 6 years up to<= 8 лет: 17–276
• > 8 years up< 18 лет: 10–240
• >= 18 years: 15–271

• <= 1 года: 0–41
• > 1 year before< 3 лет: 0–41
• >= 3 years up<= 6 лет: 0–25
• > 6 years up to<= 8 лет: 0–23
• > 8 years up< 18 лет: 0–20
• >= 18 years: 0–16

• <= 1 года: 0–265
• > 1 year before< 3 лет: 0–70
• >= 3 years up<= 6 лет: 0–44
• > 6 years up to<= 8 лет: 0–17
• > 8 years up< 18 лет: 0–18
• >= 18 years: 0–25

• <= 1 года: 28–2029
• > 1 year before< 3 лет: 0–119
• >= 3 years up<= 6 лет: 0–78
• > 6 years up to<= 8 лет: 0–20
• > 8 years up< 18 лет: 0–28
• >= 18 years: 0–26

• <= 1 года: 18–4483
• > 1 year before< 3 лет: 284–1959
• >= 3 years up<= 6 лет: 179–1285
• > 6 years up to<= 8 лет: 153–765
• > 8 years up< 18 лет: 105–846
• >= 18 years: 97–540

• <= 1 года: 37–8300
• > 1 year before< 3 лет: 64–3255
• >= 3 years up<= 6 лет: 76–3519
• > 6 years up to<= 8 лет: 50–2051
• > 8 years up< 18 лет: 57–2235
• >= 18 years: 24–1531

• <= 1 года: 39–685
• > 1 year before< 3 лет: 38–479
• >= 3 years up<= 6 лет: 23–254
• > 6 years up to<= 8 лет: 22–245
• > 8 years up< 18 лет: 12–208
• >= 18 years: 15–115

• <= 1 года: 25–1217
• > 1 year before< 3 лет: 55–763
• >= 3 years up<= 6 лет: 30–554
• > 6 years up to<= 8 лет: 25–456
• > 8 years up< 18 лет: 37–418
• >= 18 years: 31–278

• <= 1 года: 14–315
• > 1 year before< 3 лет: 14–315
• >= 3 years up<= 6 лет: 10–303
• > 6 years up to<= 8 лет: 10–303
• > 8 years up< 18 лет: 15–229
• >= 18 years old: 18–114

• <= 1 года: 14–280
• > 1 year before< 3 лет: 34–254
• >= 3 years up<= 6 лет: 20–150
• > 6 years up to<= 8 лет: 21–106
• > 8 years up< 18 лет: 11–111
• >= 18 years: 13–70

• <= 1 года: 0–302
• > 1 year before< 3 лет: 0–56
• >= 3 years up<= 6 лет: 0–26
• > 6 years up to<= 8 лет: 0–18
• > 8 years up< 18 лет: 0–44
• >= 18 years: 0–30

• <= 1 года: 12–504
• > 1 year before< 3 лет: 11–133
• >= 3 years up<= 6 лет: 0–130
• > 6 years up to<= 8 лет: 0–56
• > 8 years up< 18 лет: 0–104
• >= 18 years: 10–98

• <= 1 года: 0–72
• > 1 year before< 3 лет: 0–57
• >= 3 years up<= 6 лет: 0–14
• > 6 years up to<= 8 лет: 0–9
• > 8 years up< 18 лет: 0–14
• >= 18 years: 0–12

• eclampsia;
• impaired fructose tolerance;
• diabetic ketoacidosis;
• renal failure;
• Reye's syndrome.

• hyperfunction of the adrenal cortex;
• fever;
• Hartnup's disease;
• Huntington's chorea;
• inadequate nutrition, fasting (kwashiorkore);
• malabsorption syndrome in severe diseases of the gastrointestinal tract;
• hypovitaminosis;
• nephrotic syndrome;
• pappataci fever (mosquito, phlebotomy);
• rheumatoid arthritis.

• increased arginine, glutamine - arginase deficiency;
• increased arginine succinate, glutamine - arginosuccinase deficiency;
• increased citrulline, glutamine - citrullinemia;
• increased cystine, ornithine, lysine - cystinuria;
• increased valine, leucine, isoleucine - maple syrup disease (leucinosis);
• increased phenylalanine - phenylketonuria;
• increased tyrosine - tyrosinemia.

• increased glutamine - hyperammonemia;
• increased alanine - lactic acidosis (lactic acidosis);
• increased glycine - organic aciduria;
• increased tyrosine - transient tyrosinemia in newborns.