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
See Also: