Fructose naturally occurs as fruit sugar. Man made fructose is used as a sweetener in many foods (including baby food) and drinks. Hereditary fructose intolerance is inherited as an autosomal recessive disease. It may be as common as 1 in 20,000 in some European countries.
In fructose-intolerant people, ingestion of fructose (fruit sugar) and sucrose (cane or beet sugar, table sugar) produces complicated chemical changes that cannot be corrected because of the absence of the enzyme 1-phosphofructaldolase. Ingestion of fructose causes profound hypoglycemia (low blood sugar) and progressive liver damage. The body is unable to convert its energy-storage material, glycogen, into glucose. Subsequently, the blood sugar falls (hypoglycemia). In addition, blocks in the metabolic pathway of fructose processing cause a build-up of substances that damage the liver.
Hereditary fructose intolerance can be a relatively mild disease or a very severe disease. In the severe form, even eliminating fructose and sucrose from the diet may not prevent progressive liver disease.
|