Hereditary Fructose Intolerance

Fructose is a 6 carbon ketonic sugar that is commonly found in a wide variety of foods. Hereditary fructose intolerance is characterized by severe metabolic disturbances that include hypoglycemia, lactic acidosis, and hypophosphatemia. Hereditary fructose intolerance was originally characterized as an "idiosyncratic reaction to fructose" in a patient that developed violent nausea, abdominal pain, and faintness after consuming sucrose and fructose by Chambers and Fratt in 1956. The hepatic enzyme defect was discovered over the next 4 to 5 years, and the pathophysiology was delineated.