fructosuria meaning in Hindi

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Fructosuria or hepatic fructokinase deficiency is a rare but benign inherited metabolic disorder.
Essential fructosuria is a genetic condition that is inherited in an autosomal recessive manner.
A diagnosis of essential fructosuria is typically made after a positive test for reducing substances in the urine.
Biochemical abnormalities that may lead to the eventual diagnosis of fructosuria are hepatic fructokinase deficiency, levulosuria and ketohexokinase deficiency.
However, urine-reducing substance findings are positive ( fructosuria ) in those with fructose 1-phosphate aldolase deficiency ( fructose intolerance ).
No treatment is indicated for essential fructosuria, while the degree of fructosuria depends on the dietary fructose intake, it does not have any clinical manifestations.
No treatment is indicated for essential fructosuria, while the degree of fructosuria depends on the dietary fructose intake, it does not have any clinical manifestations.
Clinically, patients with hereditary fructose intolerance are much more severely affected than those with essential fructosuria, with elevated uric acid, growth abnormalities and can result in coma if untreated.
In patients with essential fructosuria, metabolism of fructose by hexokinase to fructose-6-phosphate is the primary method of metabolizing dietary fructose; this pathway is not significant in normal individuals.

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