Fructosuria
Fructosuria Causes
Essential fructosuria is caused by a hepatic fructokinase deficiency. Without this enzyme, fructose is not further broken down into glucose which the body can use. The unmetabolized fructose remains intact and excreted through urine.
Fructosuria Definition
This disorder is also known as essential fructosuria and hereditary fructosuria often seen in children. It is caused by a hepatic fructokinase deficiency which leads to excretion of fructose in urine. Fructosuria is a benign condition, since fructose is not broken down to glucose; it is simply excreted out of the system in urine. Fructosuria is unlike Fructosemia which means fructose in the blood. The latter is a more serious condition that can cause growth abnormalities and even coma in some severe cases. Unlike fructosuria, fructose in Fructosemia is converted to fructose-1-phosphate which uses up the body's ATP stores. This will prevent the proper release of glucose from glycogen, uses up the free phosphate available in the body, and causes uric acid levels to rise.
Fructosuria Diagnosis
Diagnosis is done through urinalysis where the urine will be positive for fructose.
Fructosuria Symptoms and Signs
Symptoms of Fructosuria include the presence of fructose in the urine. Symptoms of hypoglycemia are also manifested by the individual.
Fructosuria Treatment
Treatment for Fructosuria involves eliminating sources of the sugar from the diet. By reducing and restricting the amount of fructose ingested, the amount of fructose present in the urine will also decrease.