It is possible that the main title of the report Fructosuria is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
- Essential Fructosuria
- Hepatic Fructokinase Deficiency
- Ketohexokinase Deficiency
Fructosuria is a rare but benign inherited metabolic disorder. It is characterized by the excretion of fruit sugar (fructose) in the urine. Normally, no fructose is excreted in the urine. This condition is caused by a deficiency of the enzyme fructokinase in the liver. This enzyme is needed for the synthesis of glycogen (the body's form of stored energy) from fructose. The presence of fructose in the blood and urine may lead to an incorrect diagnosis of diabetes mellitus.
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