Aldolase B Deficiency
An autosomal recessive Fructose Metabolism disorder due to deficient Fructose-1-phosphate Aldolase (EC 18.104.22.168) 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.