Home > Encyclopedia of Children's Health > Hereditary Fructose Intolerance

Definition

Hereditary fructose intolerance is a metabolic disorder in which the small intestine cannot process fructose (fruit sugar) into a source of energy because of an enzyme deficiency that prevents fructose absorption.

Description

Fructose is a simple sugar found naturally in fruits, vegetables, and honey. Synthetic fructose (in the form of corn syrup) is used as a sweetener in many foods, including baby food, and sweetened beverages. Other simple sugars include glucose (the form in which sugar circulates in the blood) and galactose (produced by the digestion of milk). Simple sugars can be absorbed by the small intestine.

Digestion of food begins in the mouth, moves to the stomach, and then into the small intestine. Along the way, specific enzymes are needed to process different...