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Hemochromatosis


Chronic hereditary disease characterized by a violation of iron metabolism, its increased content in the blood and the accumulation of hemosiderin in the cells of the liver, pancreas, heart and other internal organs with the development of fibrosis in them, as well as in the skin with a change in its color.
Symptoms and course:
Clinically manifested by the following triad: cirrhosis (the earliest and persistent symptom), diabetes mellitus - increased blood sugar and the appearance of sugar in the urine, dark brown skin color. The disease develops slowly and gradually, the first signs can be liver damage (its increase), general weakness, gradually darkens the skin, signs of diabetes mellitus join, heart damage (dyspnea, swelling, changes on the ECG). The change in skin coloration may precede the complete picture of the disease over many years. As the progression in the clinic, the symptoms of liver cirrhosis predominate: dense, large, ascites (fluid in the abdominal cavity). The disease lasts for many years.
Recognition:
The amount of iron exceeds the normal values ​​many times (2-3 times) even before the appearance of a brownish color of the skin. Increase of sugar in the blood and its presence in the urine with the adherence of diabetes. When ultrasound examination of the abdominal cavity, scintigraphy signs of cirrhosis. When a biopsy specimen (pieces) of a liver and skin is examined under a microscope, the hemosiderin substance is detected in the cells.
The prognosis without treatment is unfavorable: patients die from hepatic or diabetic coma, heart failure, bleeding from varicose-esophageal veins of the esophagus.
Treatment:
Diet N 5 with the restriction of products containing iron. Repeated bleeding at 300-500 ml of blood. Taking desferol or injecting deferoxamine to remove iron from the body. Insulin therapy of diabetes mellitus. Vitaminotherapy. It should be remembered that prolonged uncontrolled intake of iron preparations may lead to secondary hemosiderosis, but usually without diabetes mellitus and cirrhosis of the liver.