Fructose Intolerance
Synonyms
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 Phosphate Aldolase Deficiency
Fructose 1,6 Biphosphate Aldolase Deficiency
Fructose 1,6 Bisphosphate Aldolase B Deficiency
Fructose Aldolase B Deficiency
Fructose Intolerance, Hereditary
Fructose Intolerances
Fructose Intolerances, Hereditary
Fructose-1,6-Biphosphate Aldolase Deficiencies
Fructose-1,6-Biphosphate Aldolase Deficiency
Fructose-1,6-Bisphosphate Aldolase B Deficiency
Fructose-1-Phosphate Aldolase Deficiencies
Fructose-1-Phosphate Aldolase Deficiency
Fructosemia
Fructosemias
Hereditary Fructose Intolerance
Hereditary Fructose Intolerances
Intolerance, Fructose
Intolerances, Fructose
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.
See Also