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Hemochromatosis


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Hemochromatosis (without specification) or a hemosiderosis generally is determined as surplus in an iron organism by the hereditary reason (primary) , or is a result of other metabolic disorder. However more widely this term is used for designation of excess of iron today in an organism which arises through certain specific reasons, for example, hereditary hemochromatosis.

Hereditary hemochromatosis this autosomal and recessive disease which prevalence frequency 1 person on 500 among other races a disease occurs (among the Caucasian population) less often. The gene which is responsible for hereditary hemochromatosis (HFE) is on the 6th chromosome. Most of patients with hemochromatosis in this gene has mutations which cause a disease. Hereditary hemochromatosis is characterized by the accelerated intestinal absorption of iron and its accumulation in various fabrics. Consequences of this process, as a rule, are shown on the third or fifth decade of life, but can arise also at children. The most widespread manifestation is cirrhosis in combination with a hypopituitarism, a cardiomyopathy, diabetes, arthritis or a hyperpegmentation. Considering that complications which can arise from hemochromatosis in the absence of due treatment very difficult, it is important to establish the diagnosis and to begin treatment even before emergence of the first symptoms of this disorder.

Classification of hemochromatosis

  1. Hereditary (idiopathic, primary) hemochromatosis.
  2. Secondary hemochromatosis, forms:
  • posttransfusion (in chronic anemia in which treatment it is long blood transfusions were performed);
  • alimentary (hemochromatosis of the African tribe to a bow owing to the excess use of iron with food and water; alcoholic cirrhosis; possibly, Kashin-Beck's disease, etc.);
  • metabolic (disturbance of transformation of iron at intermediate V. of a thalassemia, at patients with cirrhosis at development or imposing of a porto-caval anastomosis, at obstruction of a pancreat duct, skin porphyria, etc.);
  • the mixed origin (a big thalassemia, some types of dizeritro-poetic anemia - zhelezorefraktorny, sideroakhrestichesky, sidero-blast).

Diagnosis of hemochromatosis

Results biochemical analyses of blood serum include increase in level of aminotransferases, deviations of indicators of exchange of iron, a hyperglycemia, a purpurinuria.

At a biopsy of a liver the fibrosis and cirrhotic changes are found. Development of a hepatocellular carcinoma is possible.

The most available screening test for existence of an overload iron is definition of the index of saturation of transferrin (% Tf). If % Tf> 45%, then the following step is genetic testing for existence of mutations of a gene of C282Y/H63D.

Hemochromatosis symptoms

Classical triad of the developed hemochromatosis stage: xanthopathy, grayish with a bluish shade, mainly in extremities, the person, a neck and genitals (melanoderma), poor pilosis, diabetes and a hepatomegalia (bronze diabetes, pigmentary cirrhosis) is observed more than at 80% of patients. The liver at patients with hemochromatosis is evenly increased, dense, smooth and quite often painful. Increase in a spleen is observed at later stages of a disease. A final stage of a disease - makronodulyarny cirrhosis. Diabetes often is insulin-dependent, and at some patients insulin resistance can be noted. Also the latent diabetes detected by glucose tolerance test is possible. At 20% of patients the pigmentation not only skins, but also mucous membranes is noted. At 7-19% of patients with hemochromatosis hepatocellular cancer of a liver develops. Pancreas cirrhosis is often observed, than development in hemochromatosis of insulin-dependent diabetes mellitus speaks.

Treatment of hemochromatosis

In genetic hemochromatosis the treatment provides removal of excess of iron from an organism and maintenance of function of the damaged bodies.

Iron is the easiest removed from an organism at weekly single (or once in 2 weeks) bloodletting of 500 ml. In spite of the fact that at the same time in the beginning the volume of eritrotsitny weight decreases a little (approximately up to 350 ml/l), in several weeks it stabilizes. Iron level in plasma remains raised to exhaustion of its stocks. Ferritin level in plasma progressively decreases that reflects gradual reduction of reserves of iron in an organism. As in 500 ml of blood 200 — 250 mg of iron contain, and it is necessary to delete it in number of about 25 g, usually weekly bloodlettings carry out within 2 — 3 years. After normalization of levels of iron and ferritin in plasma of bloodletting make with such intervals to provide maintenance of iron in plasma at the level of lower than 1500 mkg/l. It is usually enough to make on one phlebotomy each 3 months. The adequacy of treatment can be estimated at any time, having determined iron level in plasma, saturation percent by iron of transferrin or concentration of ferritin in serum. These indicators quickly reach patholologically high level at an iron reakkumulyation.

At parenteral administration of chelating agents, such as Deferoxaminum , 10 — 20 mg of iron are removed per day, i.e. it is less than a half of that quantity which will be mobilized by weekly bloodletting. Besides, phlebotomy at treatment of patients with genetic hemochromatosis, as a rule, costs cheaper, is more convenient and safe. However chelating agents are shown when anemia or a hypoproteinemia serves as an obstacle for bloodletting. Hypodermic infusions of Deferoxaminum by means of the portable pump working at small speed are most effective.

Treatment in liver and heart failure and diabetes differs from usual a little, carried out in these diseases. Loss of a libido and change of secondary sexual characteristics are partially compensated by testosterone or gonadotrophins.

The drugs used at treatment of this disease:

Deferoxaminum
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