hereditary fructose intolerance

hereditary fructose intolerance
an autosomal recessive carbohydrate intolerance with onset in infancy, caused by mutation in the ALDOB gene (locus: 9q22.3), which encodes fructose-bisphosphate aldolase, isozyme B; the resulting enzyme deficiency is characterized by hypoglycemia, with variable manifestations of fructosuria, fructosemia, anorexia, vomiting, failure to thrive, jaundice, splenomegaly, and an aversion to fructose-containing foods. If untreated, it may be fatal. See also essential fructosuria, under fructosuria.

Medical dictionary. 2011.

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  • fructose 1-phosphate — A fructose derivative that accumulates in individuals with hereditary fructose intolerance. * * * an intermediate in fructose metabolism …   Medical dictionary

  • intolerance — Abnormal metabolism, excretion, or other disposition of a given substance; term often used to indicate impaired utilization or disposal of dietary constituents. hereditary fructose i. [MIM*229600] a metabolic error due to deficiency …   Medical dictionary

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  • carbohydrate intolerance — inability to properly metabolize one or more carbohydrates, as in glucose intolerance, hereditary fructose intolerance, and the various types of disaccharide intolerance …   Medical dictionary

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