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Genetic and Rare Diseases Information Center (GARD)

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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
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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 progressive liver damage. In addition, blocked processing of fructose will cause a build-up of substances that damage the liver. HFI may be relatively mild or a very severe disease, and 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: 3/17/2009

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 often difficult, but not impossible. People on this diet 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.[2]
Last updated: 3/17/2009

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.

The links entitled  Fructose Free Diet 1 and Fructose Free Diet 2 may be of particular interest to you.
Last updated: 3/17/2009

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 American Dietitian Association can help you locate a dietician in your area.

American Dietetic Association
120 South Riverside Plaza, Suite 2000
Chicago, Illinois 60606-6995
Consumer nutrition information and referrals
Toll-free: 1-800-366-1655
Public Web site:

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:

  * GeneClinics - A searchable directory of US and international genetics and prenatal diagnosis clinics. To locate genetics clinics in the United States, go to the following link and click on 'Clinic Directory' to find a genetic service close to you.  To locate genetics clinics outside of the United States, go the following link, click on 'Clinic Directory', and click on 'International Clinic Directory Search'.

  * ResourceLink - A database of genetics counseling services, searchable by location, name, institution, type of practice, or specialty. Hosted by the National Society of Genetic Counselors.

  * Genetic Centers, Clinics, and Departments - A comprehensive resource list for genetic counseling, including links to genetic centers and clinics, associations, and university genetics departments. Hosted by the University of Kansas Medical Center.

The American Society of Human Genetics (ASHG) is a professional organization of research and clinical geneticists. The ASHG maintains a database of these geneticists, some of which live outside of the United States. If you are interested in obtaining a list of the geneticists in your country, some of which may only be researchers and may not offer medical care, please visit the following hyperlink, enter 'YOUR COUNTRY'S NAME' in the 'Country' search box, and click on 'Click to Begin Search'.
Last updated: 3/17/2009