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Diseases

Genetic and Rare Diseases Information Center (GARD)

Hereditary fructose intolerance


Other Names for this Disease
  • ALDOB deficiency
  • Aldolase B deficiency
  • Fructose intolerance, hereditary
  • Fructose-1,6-bisphosphate aldolase B deficiency
  • Fructose-1-phosphate aldolase deficiency
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I have spent hours looking for information about a fructose/sucrose free diet. Everything I have discovered tells you what you can't eat but not much regarding what is permissible. Can you send me a link, diet outline, or any information concerning hereditary fructose intolerance?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is hereditary fructose intolerance (HFI)?

Hereditary fructose intolerance (HFI) is a metabolic disease caused by the absence of an enzyme called aldolase B. In people with HFI, ingestion of fructose (fruit sugar) and sucrose (cane or beet sugar, table sugar) causes severe hypoglycemia (low blood sugar) and the build up of dangerous substances in the liver. HFI may be relatively mild or a very severe disease.[1] The condition is caused by mutations in the ALDOB gene. It is inherited in an autosomal recessive pattern.[2] Treatment involves eliminating fructose and sucrose from the diet. In the severe form, eliminating these sugars from the diet may not prevent progressive liver disease.[1]
Last updated: 8/20/2015

How is hereditary fructose intolerance treated?

Complete elimination of fructose and sucrose from the diet is an effective treatment for most patients with hereditary fructose intolerance.[1] This involves exclusion of anything containing fructose, sucrose, or sorbitol. Implementation and adherence to this diet is challenging. People who following this diet can live normal and healthy lives, although the danger of inadvertent fructose ingestion remain. In extreme cases of life-threatening liver damage, liver transplants have been performed.[3]
Last updated: 8/21/2015

Is there a resource which provides information about what is permitted in a diet for individuals with hereditary fructose intolerance?

The HFI Laboratory at Boston University provides several sample diets for individuals with hereditary fructose intolerance. The following page from their website includes information on what people with HFI can and cannot eat.
http://www.bu.edu/aldolase/HFI/treatment/

The links entitled  Fructose Free Diet 1 and Fructose Free Diet 2 may be of particular interest to you.
Last updated: 8/21/2015

What specialists can help diagnose and treat hereditary fructose intolerance (HFI)?

A dietician can often be very useful in providing guidance regarding the dietary management of HFI. The Academy of Nutrition and Dietetics can help you locate a dietician in your area.

Academy of Nutrition and Dietetics
120 South Riverside Plaza, Suite 2000
Chicago, Illinois 60606-6995
Consumer nutrition information and referrals
Toll-free: 1-800-877-1600
Phone: 312-899-0040 
Email: knowledge@eatright.org
Public Web site: http://www.eatright.org/ 

Since HFI is a genetic condition, we suggest that you contact a genetics clinic to discuss this information with a genetics professional. To find a genetics clinic near you, we recommend contacting your primary doctor for a referral. The following online resources can also help you find a genetics professional in your community:

Last updated: 8/21/2015

References
Other Names for this Disease
  • ALDOB deficiency
  • Aldolase B deficiency
  • Fructose intolerance, hereditary
  • Fructose-1,6-bisphosphate aldolase B deficiency
  • Fructose-1-phosphate aldolase deficiency
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.