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Hemochromatosis

Hemochromatosis is a disorder that interferes with iron metabolism, which results in too much iron in the body. After all, the producers of everything from breakfast cereals to vitamin tonics tell us that iron builds rich, red blood. In the body, iron becomes part of hemoglobin, a molecule in the blood that transports oxygen from the lungs to all body tissues. This disease causes extra iron to gradually build up in the body's tissues and organs, a term called iron overload. If this iron buildup is untreated, it can, over many years, damage the body's organs. That's because hereditary hemochromatosis causes your body to absorb too much iron from the food you eat. The excess is stored in your organs, especially your liver, heart and pancreas. Sometimes the stored iron damages these organs, leading to life-threatening conditions such as cancer, heart problems and liver disease. Signs and symptoms of hereditary hemochromatosis usually appear in midlife, although they may occur earlier. People with hemochromatosis absorb more than the body needs. Though not always easy to diagnose, hereditary hemochromatosis can be effectively treated by removing blood from your body to lower the level of iron.

Hemochromatosis is the abnormal accumulation of iron in parenchymal organs, leading to organ toxicity. Undiagnosed and untreated HHC can develop into diseases such as diabetes, heart trouble, arthritis, cirrhosis of the liver, neurological problems, depression, impotence, infertility, and liver cancer. HHC/Iron overload generally manifests in a male's early thirties and is diagnosed in a male's mid to late fifties. The iron overload can cause many health problems, most frequently a form of diabetes that's often resistant to insulin treatment The most common complaint is joint pain, but hereditary hemochromatosis can also cause a number of other symptoms, including fatigue, abdominal pain and impotence. Some people with the disease develop symptoms by age 20, although signs of the condition usually appear between ages 40 and 60, when iron in the body has reached damaging levels. However, hereditary hemochromatosis should not be considered a disease of older people or men. Iron buildup from the disease is often present and silently causing problems long before symptoms occur - in men, women, adolescents, and, in rare cases, children.

Causes of Hemochromatosis

The common Causes of Hemochromatosis :

  • Certain anemias, such as thalassemia and aplastic anemia .
  • Chronic liver disease, such as hepatitis, alcoholic liver disease, or nonalcoholic steatohepatitis (“steato” means “fatty”)
  • Atransferrinemia and aceruloplasminemia, both rare inherited diseases.
  • HGE, a specific gene for hemochromatosis, has been identified.
  • A condition called African iron overload, which is a combination of an inherited disorder and diet.
  • The outcome is increased intestinal iron absorption and predominantly hepatocellular accumulation of hepatic iron.

Symptoms of Hemochromatosis

Some Symptoms of Hemochromatosis :

  • Weakness
  • Fatigue
  • Testicular atrophy
  • Weight loss
  • Loss of body hair
  • Abdominal pain
  • Joint pain
  • Loss of sexual desire
  • Lack of energy

Treatment of Hemochromatosis

  • The goal of treatment is to remove excess iron from the body and to give supportive treatment to damaged organs.
  • Iron supplements should be avoided.
  • Patients should limit alcohol consumption and should not eat raw oysters.
  • Once diagnosed, hemochromatosis is treated by phlebotomy to rid the body of excess iron and to maintain normal iron stores.
  • Surgical arthroplasty is considered if joint destruction becomes severe despite medical therapy.
  • Diet modification .
  • Treatment of the resulting diseases or conditions.
  • Loss of sexual desire and change in secondary sexual characteristics are improved with testosterone therapy .

 


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