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.

Descriptor ID	D005633
MeSH Number(s)	C16.320.565.202.251.271 C18.452.648.202.251.271
Concept/Terms	Fructose Intolerance Fructose Intolerance Fructose Intolerances Intolerance, Fructose Intolerances, Fructose Fructose Aldolase B Deficiency Fructose Intolerance, Hereditary Fructose Intolerances, Hereditary Hereditary Fructose Intolerances ALDOB Deficiency ALDOB Deficiencies Deficiencies, ALDOB Deficiency, ALDOB 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 Deficiency Fructose-1-Phosphate Aldolase Deficiencies Fructosemia Fructosemias Hereditary Fructose Intolerance Aldolase B Deficiency Aldolase B Deficiencies Deficiencies, Aldolase B Deficiency, Aldolase B 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 Deficiency Fructose-1,6-Biphosphate Aldolase Deficiencies

Below are MeSH descriptors whose meaning is more general than "Fructose Intolerance".

Below are MeSH descriptors whose meaning is related to "Fructose Intolerance".

Below are MeSH descriptors whose meaning is more specific than "Fructose Intolerance".