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Children's geneticist, children's doctor

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Infringement of an exchange of carbohydrates at children

Alexey Portnov, medical expert
Last reviewed: 23.04.2024

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Disorders of carbohydrate metabolism are a group of common hereditary metabolic disorders. Carbohydrates - one of the main sources of metabolic energy in the cell, among them a special place is occupied by monosugars - galactose, glucose, fructose and polysaccharide - glycogen. The key substratum of energy metabolism is glucose. As a result of glycolysis (conversion of glucose and glycogen to pyruvate) and oxidative phosphorylation in mitochondria, ATP is formed. The constant concentration of glucose is maintained by intake from food, synthesis of de novo in the process of gluconeogenesis and glycogenolysis (cleavage of glycogen). Food sources of glucose are polysaccharides and disaccharides (lactose, maltose, sucrose). Glucose can be synthesized from amino acids, mainly from alanine (gluconeogenesis), but this process requires a long time. Glycogen of the liver is a reserve form of glucose, when it cleaves, the blood glucose level rises rapidly. Glycogen of muscle tissue is necessary to maintain muscle contraction during a period of intense physical activity.

Galactose is formed from lactose (disaccharide present in dairy products); this is the most important source of energy in young children. The main food sources of fructose are sucrose and fructose (fruits, vegetables, honey).

Disorders of carbohydrate metabolism are divided into groups depending on the affected metabolic pathway of glycogen metabolism, fructose metabolism and galactose metabolism. Deficiencies in the transport of glucose and other carbohydrates have been singled out as a separate group.

MKB-10 CODE

E74.0 Diseases of accumulation of glycogen.
E74.1 Violations of fructose metabolism.
E74.2 Disturbances in the metabolism of galactose.
E74.3 Other disorders of carbohydrate absorption in the intestine.
E74.4 Infringements of exchange of pyruvate and gluconeogenesis.
E74.8 Other specified disorders of carbohydrate metabolism.
E74.9 Violation of carbohydrate metabolism, unspecified.

trusted-source [1], [2]