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.