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... > Nutritional and Metabolic Diseases > Metabolic Diseases > Metabolism, Inborn Errors > Carbohydrate Metabolism, Inborn Errors > Fructose Metabolism, Inborn Errors > Fructose Intolerance
... > Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Metabolism, Inborn Errors > Carbohydrate Metabolism, Inborn Errors > Fructose Metabolism, Inborn Errors > Fructose Intolerance

Preferred term

Fructose Intolerance  

Type

  • Topical Descriptor

Broader concept

Related concepts

Entry terms

  • ALDOB Deficiencies
  • ALDOB Deficiency
  • Aldolase B Deficiencies
  • Aldolase B Deficiency
  • Aldolase Deficiencies, Fructose-1,6-Biphosphate
  • Aldolase Deficiencies, Fructose-1-Phosphate
  • Aldolase Deficiency, Fructose-1,6-Biphosphate
  • Aldolase Deficiency, Fructose-1-Phosphate
  • Deficiencies, ALDOB
  • Deficiencies, Aldolase B
  • Deficiencies, Fructose-1,6-Biphosphate Aldolase
  • Deficiencies, Fructose-1-Phosphate Aldolase
  • Deficiency, ALDOB
  • Deficiency, Aldolase B
  • Deficiency, Fructose-1,6-Biphosphate Aldolase
  • Deficiency, Fructose-1-Phosphate Aldolase
  • Fructose-1,6-Biphosphate Aldolase Deficiencies
  • Fructose 1,6 Biphosphate Aldolase Deficiency
  • Fructose-1,6-Biphosphate Aldolase Deficiency
  • Fructose 1,6 Bisphosphate Aldolase B Deficiency
  • Fructose-1,6-Bisphosphate Aldolase B Deficiency
  • Fructose-1-Phosphate Aldolase Deficiencies
  • Fructose 1 Phosphate Aldolase Deficiency
  • Fructose-1-Phosphate Aldolase Deficiency
  • Fructose Aldolase B Deficiency
  • Fructose Intolerance, Hereditary
  • Fructose Intolerances, Hereditary
  • Fructosemia
  • Fructosemias
  • Hereditary Fructose Intolerance
  • Hereditary Fructose Intolerances

Note

  • an inborn error of fructose metab; do not use /congen & do not coord with INFANT, NEWBORN, DISEASES

Scope note

  • An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.

History note

  • 1991(1975); use FRUCTOSE METABOLISM, INBORN ERRORS 1989-1990; use CARBOHYDRATE METABOLISM, INBORN ERRORS 1964-1988

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URI

http://www.yso.fi/onto/mesh/D005633

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