Fructosuria, Essential, also known as essential fructosuria, is related to fructose intolerance, hereditary and hypoglycemia. An important gene associated with Fructosuria, Essential is KHK (Ketohexokinase), and among its related pathways/superpathways are Metabolism and Glycosaminoglycan metabolism. Affiliated tissues include liver, and related phenotypes are impairment of fructose metabolism and abnormal circulating enzyme concentration or activity