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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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Overview
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]
References
- Kirmse B. Hereditary fructose intolerance. Medline Plus Web site. http://www.nlm.nih.gov/medlineplus/ency/article/000359.htm. Accessed March 17, 2009.
Your Questions Answered
by the Genetic and Rare Diseases Information Center2 question(s) from the public on Hereditary fructose intolerance have been answered. See questions and answers. You can also submit a new question.
On this page
General Information
- Genetics Home Reference (GHR) contains information on Hereditary fructose intolerance. Click on the link to go to GHR and review the information.
- The Mayo Clinic provides additional information on foods to avoid if you have hereditary fructose intolerance.
- MedlinePlus, a Web site designed by the National Library of Medicine to help you research your health questions, provides more information about this topic. Click on the link to view this information.
- Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
- The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge. Click on the link to read information on this topic.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Hereditary fructose intolerance. Click on the link to view a sample search on this topic.
- The The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Hereditary fructose intolerance. Click on the link to go to OMIM and review these resources.