- Neonatal hemochromatosis
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Neonatal hemochromatosis Classification and external resources OMIM 231100 DiseasesDB 34508 Neonatal Hemochromatosis is a rare and severe liver disease. Its characteristics are similar to hereditary hemochromatosis, where iron deposition causes damage to the liver and other organs and tissues.
Contents
Causes
The causes of neonatal hemochromatosis are still unknown, however recent research has led to the hypothesis that it is an alloimmune disease (see alloimmunity). Evidence supporting this hypothesis includes the high recurrence rate within sibships (>80%). This evidence along with other research indicates that Neonatal Hemochromatosis could be classified as a Congenital Alloimmune Hepatitis.[1]
Treatment
Effective treatment of the disease has been confined to liver transplants. An antioxidant chelation cocktail has also been reported as having some success though its effectiveness cannot be confirmed.
Based on the alloimmune cause hypothesis, a new treatment involving high-dose immunoglobulin to pregnant mothers who have had a previous pregnancy with a confirmed neonatal hemochromatosis outcome, has provided very encouraging results.[2]
Related conditions
Neonatal hemochromatosis is sometimes confused with juvenile hemochromatosis, which is a hereditary hemochromatosis, caused by mutations of a gene called hemojuvelin. While the symptoms and outcomes from these two diseases are similar, the causes appear to be different.
References
- ^ Whitington PF (August 2007). "Neonatal hemochromatosis: a congenital alloimmune hepatitis". Semin Liver Dis 27 (3): 243–250. doi:10.1055/s-2007-985069. PMID 17682971.
- ^ Whitington PF, Hibbard JU (2004 November 6-12). "High-dose immunoglobulin during pregnancy for recurrent neonatal haemochromatosis". The Lancet 364 (9446): 1690–8. doi:10.1016/S0140-6736(04)17356-X. PMID 15530630.
External links
- Neonatal Hemochromatosis Information Center (NHIC)
- Gestational treatment to prevent recurrent lethal neonatal hemochromatosis
- Neonatal Hemochromatosis: a family's experience
- Neonatal Hemochromatosis: an inspirational story
- American Hemochromatosis Society
- International Bioiron Society
- Canadian Hemochromatosis Society
- Haemochromatosis page
- "The Bronze Killer" Mary Warder's book on Hemochromatosis
- Iron Toxicity, What you don't know
- Andrews N (1999). "Disorders of iron metabolism". New England Journal of Medicine 341 (26): 1986–95. doi:10.1056/NEJM199912233412607. PMID 10607817. link
- Haemochromatosis Society, UK
- Haemochromatosis Society Australia Inc
- Hemachromatosis page at the National Center for Biotechnology Information
Categories:- Iron metabolism
- Genetic disorders with OMIM but no gene
- Hepatology
- Rare diseases
- Genetic disorder stubs
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