Neonatal hemochromatosis
Encyclopedia
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.
). Evidence supporting this hypothesis includes the high recurrence rate within sibship
s (>80%). This evidence along with other research indicates that Neonatal Hemochromatosis could be classified as a Congenital Alloimmune Hepatitis.
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.
, 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.
Liver disease
Liver disease is a broad term describing any single number of diseases affecting the liver.-Diseases:* Hepatitis, inflammation of the liver, caused mainly by various viruses but also by some poisons , autoimmunity or hereditary conditions...
. Its characteristics are similar to hereditary hemochromatosis, where iron deposition causes damage to the liver and other organs and tissues.
Causes
The causes of neonatal hemochromatosis are still unknown, however recent research has led to the hypothesis that it is an alloimmune disease (see alloimmunityAlloimmunity
Alloimmunity is a condition in which the body gains immunity against antigens of another individual of the same species, which are perceived as foreign....
). Evidence supporting this hypothesis includes the high recurrence rate within sibship
Sibship
Sibship is a term used in epidemiology and public health to refer to the group or number of children produced by a pair of parents. Thus, siblings constitute a sibship....
s (>80%). This evidence along with other research indicates that Neonatal Hemochromatosis could be classified as a Congenital Alloimmune Hepatitis.
Treatment
Effective treatment of the disease has been confined to liver transplants. An antioxidantAntioxidant
An antioxidant is a molecule capable of inhibiting the oxidation of other molecules. Oxidation is a chemical reaction that transfers electrons or hydrogen from a substance to an oxidizing agent. Oxidation reactions can produce free radicals. In turn, these radicals can start chain reactions. When...
chelation
Chelation
Chelation is the formation or presence of two or more separate coordinate bonds between apolydentate ligand and a single central atom....
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.
Related conditions
Neonatal hemochromatosis is sometimes confused with juvenile hemochromatosisJuvenile hemochromatosis
Juvenile hemochromatosis is, as its name indicates, a form of hemochromatosis which emerges during youth.There are two forms:* "HFE2A" is associated with hemojuvelin...
, which is a hereditary hemochromatosis, caused by mutations of a gene called hemojuvelin
Hemojuvelin
Hemojuvelin is a membrane-bound and soluble protein in mammals that is responsible for the iron overload condition known as juvenile hemochromatosis in humans, a severe form of hemochromatosis. In humans, the hemojuvelin protein is encoded by the HFE2 gene...
. While the symptoms and outcomes from these two diseases are similar, the causes appear to be different.
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 link
- Haemochromatosis Society, UK
- Haemochromatosis Society Australia Inc
- Hemachromatosis page at the National Center for Biotechnology Information