Inherited abnormalities of fructose metabolism, which include three known autosomal recessive types: hepatic fructokinase deficiency (essential fructosuria), hereditary fructose intolerance, and hereditary fructose-1,6-diphosphatase deficiency. Essential fructosuria is a benign asymptomatic metabolic disorder caused by deficiency in fructokinase, leading to decreased conversion of fructose to fructose-1-phosphate and alimentary hyperfructosemia, but with no clinical dysfunction; may produce a false-positive diabetes test.

DUI

D015318 MeSH Browser

CUI

M0023586

Previous indexing

Carbohydrate Metabolism, Inborn Errors (1966-1988); Fructose (1966-1988)

History note

89; FRUCTOSE INTOLERANCE was see under CARBOHYDRATE METABOLISM, INBORN ERRORS 1967-88 (Prov 1964-66)

Online note

use CARBOHYDRATE METABOLISM, INBORN ERRORS to search FRUCTOSE METABOLISM, INBORN ERRORS 1966-74

Public note

89; FRUCTOSE INTOLERANCE was see under CARBOHYDRATE METABOLISM, INBORN ERRORS 1967-88 (Prov 1964-66)