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Hereditary fructose intolerance - Overview

Alternative Names

Fructosemia; Fructose intolerance; Fructose aldolase B-deficiency; Fructose 1, 6 bisphosphate aldolase deficiency

Definition of Hereditary fructose intolerance:

Hereditary fructose intolerance is a disorder of metabolism in which a person lacks the protein needed to break down fructose. Fructose is a fruit sugar that naturally occurs in the body. Man-made fructose is used as a sweetener in many foods, including baby food and drinks.

Causes, incidence, and risk factors:

This condition occurs when the body is missing a substance called aldolase B. This substance is needed to break down fructose.

If a person without this substance eats fructose and sucrose (cane or beet sugar, table sugar), complicated chemical changes occur in the body. The body cannot change its energy storage material, glycogen, into glucose. As a result, the blood sugar falls and dangerous substances build up in the liver.

Hereditary fructose intolerance is inherited, which means it is passed down through families. It may be as common as 1 in 20,000 in some European countries.

  • Reviewed last on: 5/2/2007
  • Brian Kirmse, MD, Mount Sinai School of Medicine, Department of Human Genetics, New York, NY. Review provided by VeriMed Healthcare Network.