Sign in  |  Register

Illustrated Health Encyclopedia
[an error occurred while processing this directive]

Ency. home > Disease > H > Hereditary fructose intolerance

Hereditary fructose intolerance   

Overview | Symptoms | Treatment | Prevention

Alternative names:

Fructosemia; Fructose intolerance; Fructose aldolase B-deficiency

Definition:

A metabolic disease caused by the absence of an enzyme, 1-phosphofructaldolase (i.e., fructose aldolase B).

Causes and Risks

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.

Ency. home > Disease > H > Hereditary fructose intolerance

[an error occurred while processing this directive]