Skip navigation

Disease Fructose Intolerance

Name 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-1,6-Biphosphate Aldolase Deficiencies | Fructose 1,6 Biphosphate Aldolase Deficiency | Fructose-1,6-Biphosphate Aldolase Deficiency | Fructose 1,6 Bisphosphate Aldolase B Deficiency | Fructose-1,6-Bisphosphate Aldolase B Deficiency | Fructose-1-Phosphate Aldolase Deficiencies | Fructose 1 Phosphate Aldolase Deficiency | Fructose-1-Phosphate Aldolase Deficiency | Fructose Aldolase B Deficiency | Fructose Intolerance, Hereditary | Fructose Intolerances | Fructose Intolerances, Hereditary | Fructosemia | Fructosemias | Hereditary Fructose Intolerance | Hereditary Fructose Intolerances | Intolerance, Fructose | Intolerances, Fructose
Definition 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.
Categories Genetic disease (inborn) | Metabolic disease
MeSH® ID D005633
OMIM® ID 229600
External Links

Top ↑ Ancestors

1. DiseasesCongenital, Hereditary, and Neonatal Diseases and Abnormalities Has associated chemicals Has associated genes Has associated exposure references Genetic Diseases, Inborn Has associated chemicals Has associated genes Has associated exposure references Metabolism, Inborn Errors Has associated chemicals Has associated genes Has associated exposure references Carbohydrate Metabolism, Inborn Errors Has associated chemicals Has associated genes Fructose Metabolism, Inborn Errors Has associated chemicals Has associated genes Fructose Intolerance Has associated chemicals Has associated genes
2. DiseasesNutritional and Metabolic Diseases Has associated chemicals Has associated genes Has associated exposure references Metabolic Diseases Has associated chemicals Has associated genes Has associated exposure references Metabolism, Inborn Errors Has associated chemicals Has associated genes Has associated exposure references Carbohydrate Metabolism, Inborn Errors Has associated chemicals Has associated genes Fructose Metabolism, Inborn Errors Has associated chemicals Has associated genes Fructose Intolerance Has associated chemicals Has associated genes

Top ↑ Descendants

None.