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Hemochromatosis Treatments, Hemochromatosis Diagnosis, Hemochromatosis gicare.com | Hemochromatosis endowsec.com | Hemochromatosis - New Treatments, August 1, 2009 ccspublishing.com |
In medicine, iron overload indicates accumulation of iron in the body due to any cause.
[edit] Terminology[edit] Haemochromatosis or haemosiderosisHistorically, the term haemochromatosis was initially used to refer to what is now more specifically called haemochromatosis type 1 (or HFE-related hereditary haemochromatosis). Currently, haemochromatosis (without further specification) is mostly defined as iron overload with a hereditary/primary cause,[1][2] or originating from a metabolic disorder.[3] However, the term is currently also used more broadly to refer to any form of iron overload, thus requiring specification of the cause, for example hereditary haemochromatosis. The term haemosiderosis is generally used to indicate the pathological effect of iron accumulation in any given organ, which mainly occurs in the form of haemosiderin.[4][5] Sometimes, the simpler term siderosis is used instead. Other definitions distinguishing haemochromatosis or haemosiderosis that are occasionally used include:
[edit] Clinical presentationOrgans commonly affected by haemochromatosis are the liver, heart and endocrine glands.[12] [edit] CausesThe causes can be distinguished between primary cases (hereditary or genetically determined) and less frequent secondary cases (acquired during life).[13] People of Celtic (Ireland, Scotland) origin have a particularly high incidence of whom about 10% are carriers of the gene and 1% sufferers of the condition. [edit] Primary haemochromatosisThe fact that most cases of haemochromatosis were inherited was well known for most of the 20th century, though they were incorrectly assumed to depend on a single gene.[14] The overwhelming majority actually depend on mutations of the HFE gene discovered in 1996, but since then others have been discovered and sometimes are grouped together as "non-classical hereditary haemochromatosis"[15], "non-HFE related hereditary haemochromatosis".[16], or "non-HFE haemochromatosis".[17]
Most types of hereditary haemochromatosis have autosomal recessive inheritance, while type 4 has autosomal dominant inheritance.[18] [edit] Secondary haemochromatosis
[edit] PrognosisA third of those untreated develop hepatocellular carcinoma.[19] [edit] TreatmentRoutine treatment in an otherwise healthy person consists of regularly scheduled phlebotomies (bloodletting). When first diagnosed, the phlebotomies may be fairly frequent, perhaps as often as once a week, until iron levels can be brought to within normal range. Once iron (Fe) and other markers are within the normal range, phlebotomies may be scheduled every other month or every three months depending upon the patient's rate of iron loading. For those unable to tolerate routine blood draws, there is a chelating agent available for use. The drug Deferoxamine binds with iron in the blood stream and enhances its elimination via urine and feces. Typical treatment for chronic iron overload requires subcutaneous injection (SQ) over a period of 8-12 hours daily. [edit] See also[edit] References
[edit] External links
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