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.

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