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At the stage of advanced clinical manifestations, histological changes in the liver usually correspond to pigmentary septal or small-nodular cirrhosis with massive deposits of hemosiderin in hepatocytes and less significant in macrophages, bile duct epithelium. Histological examination in the terminal stage of the disease reveals a picture of generalized hemosiderosis with damage to the liver (by the type of mono- and multilobular cirrhosis), heart, pancreas, thyroid, salivary and sweat glands, adrenal glands, pituitary gland and other organs.

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Iron overload has been observed in a number of congenital or acquired conditions from which HHC must be differentiated.

The disease must also be differentiated from blood pathology (thalassemia, sideroblastic anemia, hereditary atransferrinemia, microcytic anemia, tardive cutaneous porphyria), liver diseases (alcoholic liver damage, chronic viral hepatitis, non-alcoholic steatohepatitis). A diet rich in proteins is shown, without foods containing iron.

Bloodletting is the most accessible way to remove excess iron from the body. Usually 300-500 ml of blood is removed with a frequency of 1-2 times a week. The number of phlebotomies is calculated depending on the level of hemoglobin, blood hematocrit, ferritin, and the amount of excess iron. This takes into account that 500 ml of blood contains 200-250 mg of iron, mainly in the hemoglobin of erythrocytes.

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Bleeding continues until the patient develops mild anemia. A modification of this extracorporeal technique is cytapheresis (CA) (removal of the cellular part of the blood with the return of autoplasma in a closed circuit). In addition to the mechanical removal of blood cells, CA has a detoxifying effect and helps to reduce the severity of degenerative-inflammatory processes. Each patient undergoes 8-10 sessions of accutane with a further transition to maintenance therapy using CA or hemoexfusions in the amount of 2-3 sessions for 3 months.

Drug treatment is based on the use of deferoxamine (desferal, desferin), 10 ml of a 10% solution intramuscularly or intravenously by drip. accutane drug has a high specific activity towards Fe3+ ions. At the same time, 500 mg of Desferal are able to remove 42.5 mg of iron from the body. The duration of the course is 20-40 days. At the same time, cirrhosis, diabetes mellitus and heart failure are treated. Frequently observed anemic syndrome in patients with HCH in the presence of hutThe exact content of iron in the liver tissue limits the use of efferent therapy.

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Our clinic has developed a scheme for the use of recombinant erythropoietin against the background of CA.

The drug promotes increased utilization of iron from the body's depot, due to which there is a decrease in the total reserves of the microelement, an increase in hemoglobin levels. Recombinant erythropoietin is administered at a dose of 25 μg/kg of body weight against the background of isotretinoin sessions performed 2 times a week for 10-15 weeks. The forecast is determined by the degree and duration of overloads.

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The course of the disease is long, especially in the elderly. Timely therapy prolongs life by several decades. Survival for 5 years in treated patients is 2.5-3 times higher than in untreated patients. The risk of developing HCC in patients with HCC in the presence of liver cirrhosis increases by 200 times. The most common cause of death is liver failure.

  • Which doctors should be contacted if you have Hemochromatosis.
  • If necessary, the issue of liver transplantation and arthroplasty is decided.
  • Treatment of patients with hemochromatosis is based on diet, administration of deferoxamine, bloodletting, plasmapheresis, hemosorption, and symptomatic therapy.
  • Causes of hemochromatosis Symptoms of hemochromatosis Diagnosis of hemochromatosis Treatment of hemochromatosis Prognosis and prevention Prices for treatment.

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Hemochromatosis is a hereditary polysystemic disease, accompanied by active absorption of iron in the gastrointestinal tract and its subsequent accumulation in internal organs (heart, pancreas, liver, joints, pituitary gland). The clinic of hemochromatosis is characterized by bronze pigmentation of isotretinoin pills and mucous membranes, the development of cirrhosis of the liver, diabetes mellitus, cardiomyopathy, arthralgia, sexual dysfunction, etc. The diagnosis of hemochromatosis is confirmed by determining increased urinary iron excretion, high iron content in blood serum and liver biopsy specimens, as well as with the help of radiography, ultrasound, MRI of internal organs.

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Hemochromatosis (bronze diabetes, pigmentary cirrhosis) is a genetically determined violation of iron metabolism, leading to the deposition of iron-containing pigments in tissues and organs and the development of multiple organ failure. The disease, accompanied by a characteristic symptom complex (skin pigmentation, liver cirrhosis and diabetes mellitus) was described in 1871, and in 1889 it was called hemochromatosis for the characteristic color of the skin and internal organs. The frequency of accutane hemochromatosis in the population is 1.5-3 cases per 1000 population. Men suffer from hemochromatosis 2-3 times more often than women.

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The average age of pathology development is 40-60 years. Due to the polysystem nature of the lesion, various clinical disciplines are engaged in the study of hemochromatosis: gastroenterology, cardiology, endocrinology, rheumatology, etc.
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