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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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.Hereditary fructose intolerance (HFI) is a metabolic disease caused by the absence of an
Last updated: 3/17/2009
- Kirmse B. Hereditary fructose intolerance. Medline Plus Web site. May 2, 2007; http://www.nlm.nih.gov/medlineplus/ency/article/000359.htm. Accessed 3/17/2009.
- Genetics Home Reference (GHR) contains information on Hereditary fructose intolerance. This website is maintained by the National Library of Medicine.
- The Mayo Clinic provides additional information on foods to avoid if you have hereditary fructose intolerance.
- MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
- 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.
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- 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.