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Hereditary intolerance to fructose (fructosemia) is a rare genetic disease caused by a mutation in the ALDOB gene, which leads to a deficiency of the aldolase B enzyme, catalyzing the fructose-1,6-bisphosphate and fructose-1-phosphate cleavage to triose (tri-carbon) proteins. Two forms of fructosemia are selected: in the infantile form, the onset of the disease is acute, associated with the first meal, there is fructose or sucrose; late infantile form is a more favorable course with the development of hepatomegaly in the first years of life. Eating containing fructose leads to the accumulation of fructose-1-phosphate in the liver, which, in turn, is manifested by inhibition of gluconeogenesis, the development of hypoglycemia and lactate acidosis, and increased lipid mobilization. Clinically, this is manifested by nausea, vomiting, hypoglycemia, and liver failure. Histological examination of liver tissue determines diffuse steatosis, periportal fibrosis. The main method of treatment is a strict diet with the exception of fructose and sucrose from the diet. Timely diagnosis and diet therapy, provide the normal physical and neuromental development of the child, and biochemical parameters are gradually normalizing. We have presented clinical examples of the late infantile form of fructosemia. Ensuring timely diagnosis and the appointment of diet therapy are factors in the restoration of liver tissue and stabilization of the child’s condition.

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