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Hemochromatosis disease and types

Hemochromatosis disease

Hemochromatosis is a disorder which is caused by increased iron accumulation in the organs, resulting in the occurrence of damage to them. Most often affect the liver, heart, pancreas, joints, pituitary gland. The disease is quite rare – 1/200 to 1/2000 of Caucasians. Usually, men suffer more often and more severely.

There are essentially two forms of the disease – primary and secondary.

Primary hemochromatosis is idiopathic, ie her usual cause of the disease is unknown. Most often it is a congenital disease that develops as a result of abnormally increased intestinal absorption of iron. Unlike normal subjects patients with this type of hemochromatosis absorb 3-4 times more iron than normal. Since the body is adapted for processing such a amount of iron, it is most often accumulate in certain organs, with the result that they become damaged.

The secondary hemochromatosis is observed most often in or diseases that can cause inefficient formation of erythrocytes, or other chronic liver disease (alcoholic and viral hepatitis).

In principle, the illness is asymptomatic to 4-5 decade. The earliest symptoms of hemochromatosis are too unspecific – fatigue, drowsiness, joint pain, loss of weight. Subsequently occurring menstrual irregularities, loss of libido, light weight in the right side of ribs. Typical symptoms of the disease include liver disease, skin hyperpigmentation (increased formation and deposition of pigment in the skin) and diabetes. These complaints, however, are late-onset disease and unfortunately occur in a minority of patients. Liver damage ranging from barely detectable hepatomegaly (enlarged liver) to clinically overt liver cirrhosis with the development of portal hypertension and esophageal varices.

Diabetes mellitus often precedes the onset of liver damage. The reason for his appearance are toxic iron accumulation in the pancreatic cells secreting insulin. As a result, the cells occurs desktruktsiya and develop diabetes mellitus. Relatively rare symptoms of hemochromatosis may also be manifested by other bodies that are toxic damage from excessive accumulation of iron in them. On the one hand this can be damage to the heart, most often a manifestation of a different beat-conduction disturbances; other times it is a arthritic manifestations. Fairly often observed typical darkening of the skin, which gradually becomes grayish-brown, hair disappears. Patients with hemochromatosis are at increased risk of developing liver cancer. In addition, they are relatively frequent, and infection, which further worsens the condition.


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