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Iron overload (also known as haemochromatosis or hemochromatosis) is the abnormal and increased accumulation of total iron in the body, leading to organ damage. [1] The primary mechanism of organ damage is oxidative stress, as elevated intracellular iron levels increase free radical formation via the Fenton reaction.
Hemosiderin deposition in the liver is a common feature of hemochromatosis and is the cause of liver failure in the disease. Selective iron deposition in the beta cells of pancreatic islets leads to diabetes [4] [2] due to the distribution of transferrin receptor on the beta cells of islets [3] and in the skin leads to hyperpigmentation.
Haemochromatosis is protean in its manifestations, i.e., often presenting with signs or symptoms suggestive of other diagnoses that affect specific organ systems.Many of the signs and symptoms below are uncommon, and most patients with the hereditary form of haemochromatosis do not show any overt signs of disease nor do they have premature morbidity, if they are diagnosed early, but, more ...
Iron metabolism disorders may involve a number of genes including HFE and TFR2. [ 1 ] Hepcidin is the master regulator of iron metabolism and, therefore, most genetic forms of iron overload can be thought of as relative hepcidin deficiency in one way or another [1] .
Transfusion hemosiderosis can cause permanent damage to tissues that may lead to death. [2] Tissue damage can remain even after chelation therapy. [2] Outcomes are usually worse in patients who require blood transfusions compared to those who can have alternative therapies. [2] Cardiomyopathy and cardiac arrhythmia are often a cause of death. [1]
It is this breakdown of heme that is the source of iron deposition that ultimately causes superficial siderosis. [6] The excess free iron is circulated in the cerebrospinal fluid and deposited in neuronal tissues, where it catalyzes the formation of reactive oxygen species which can damage DNA, lipids and proteins, and is otherwise toxic to the ...
Patients with this condition can experience frequent painful bruising around joints and muscles. Because of the rarity of the disorder, there are few methods of support in place for patients. [3] Many patients are labelled with the stigma of having a psychological condition without this having a specifically proven link.
Methemoglobinemia can be due to certain medications, chemicals, or food or it can be inherited. [2] Substances involved may include benzocaine , nitrites , or dapsone . [ 3 ] The underlying mechanism involves some of the iron in hemoglobin being converted from the ferrous [Fe 2+ ] to the ferric [Fe 3+ ] form. [ 3 ]