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Aldolase Deficiency, Fructose-1-Phosphate

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.