essential fructosuria

(redirected from fructokinase deficiency)


the presence of fructose in the urine.
essential fructosuria a benign, autosomal recessive disorder of carbohydrate metabolism due to a defect in fructokinase and manifested only by fructose in the blood and urine.

es·sen·tial fruc·to·su·ri·a

a benign, asymptomatic inborn error of metabolism due to deficiency of fructokinase, the first enzyme in the specific fructose pathway; fructose appears in the blood and urine, but is simply excreted unchanged; autosomal recessive inheritance. A fructokinase deficiency.
See also: hereditary fructose intolerance.