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Get answers to your child's growth, nutrition, and feeding behavior questions.

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Note: This is for informational purposes only. Doctors cannot provide a diagnosis or individual treatment advice via e-mail. Please consult your physician about your specific health care concerns.

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Hereditary fructose intolerance - Treatment

Alternative Names

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

Treatment:

Removing fructose and sucrose from the diet is an effective treatment for most patients. Complications are treated. For example, some patients can take medication to lower the level of uric acid in their blood and decrease their risk for gout.

Expectations (prognosis):

Hereditary fructose intolerance may be mild or severe.

Avoiding fructose and sucrose helps most children with this condition.

A few children with a severe form of the disease will develop severe liver disease. Even removing fructose and sucrose from the diet may not prevent severe liver disease in these children.

How well a person does depends on:

  • How soon the diagnosis is made
  • How soon fructose and sucrose can be removed from the diet
  • How well the enzyme works in the body

Complications:

  • Avoidance of fructose-containing foods due to their effects
  • Bleeding
  • Death
  • Gout
  • Illness from eating foods containing fructose or sucrose
  • Liver failure
  • Low blood sugar (hypoglycemia)
  • Seizures

Calling your health care provider:

Call your health care provider if your child develops symptoms of this condition after feeding starts. If your child has this condition, experts recommend seeing a doctor who specializes in biochemical genetics or metabolism.

  • Reviewed last on: 4/15/2009
  • Chad Haldeman-Englert, MD, Division of Human Genetics, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Steinmann B, Santer R, van den Berghe G. Disorders of Fructose Metabolism. In: Fernandes J, Saudubray JM, van den Berghe G, Walter JH, eds. Inborn Metabolic Diseases. 4th ed. New York, NY:Springer;2006:chap 9.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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