Hereditary Fructose Intolerance
Alternate Names : Fructose Aldolase B-Deficiency, Fructose Intolerance, Fructosemia
Definition Hereditary fructose intolerance is a metabolic disease caused by the absence of an enzyme, 1-phosphofructaldolase.
Overview, Causes, & Risk Factors
Fructose is a naturally-occuring fruit sugar. Man-made fructose is used as a sweetener in many foods (including baby food) and drinks. Hereditary fructose intolerance is 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 relatively mild or very severe disease. In the severe form, even eliminating fructose and sucrose from the diet may not prevent progressive liver disease.
|
