Fructose Intolerance

**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.

**Tree numbers:** - C16.320.565.202.251.271 - C18.452.648.202.251.271

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