Fructosamine is an autosomal recessive genetic condition characterized by the violation metabolism fruktozy. Refers to hereditary enzymopathies (see), caused by a deficiency of the enzyme fructose-1-phosphate-aldolase. The disease was first described in 1956, the Deficiency of the enzyme fructose-1-phosphate-aldolase leads to accumulation in blood and tissues of the body undigested fructose-1-phosphate and increased its allocation of urine.
The clinical symptoms of the disease appear since the appointment of child fruit juices, cow's milk, cereals, sugar-sweetened. In children celebrated vomiting, diarrhea, decreased appetite, develops hypotrophy, sometimes increased liver and jaundice appears, can develop hypoglycemic coma. Screening determines the increased excretion of fructose, protein, amino acids and sometimes leukocytes.
Diagnosis of fructosamine is based on chromatographic allocation of fructose from the urine and research glycemic curves after load fructose. Fructosamine must be differentiated from the galactosemia (see). Unlike galactosemia, children suffering from fructosamina tolerate milk, they have not observed the development of cataracts, and mental retardation.
Treatment of fructosamine is the elimination of food products containing fructose (honey, sugar, fruit, jams, marmalade, sugar beets, carrots, cocoa, chicory, turnips). It should also appoint medicines containing fructose (various syrups). With timely diagnosis and rational diet forecast is favorable.