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Fructose Intolerance

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.

Year introduced: 1991(1975)

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Subheadings:

Tree Number(s): C16.320.565.202.251.271, C18.452.648.202.251.271

MeSH Unique ID: D005633

Entry Terms:

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

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