Therapy Treatment is aimed at removal of excess copper from the body to prevent its toxicity. Assign diet No. 5, rich in protein, with the restriction of copper products (sheep, chickens, ducks, sausage, fish, crustaceans, mushrooms, tomatoes, mallow, leeks, radishes, beans, nuts, prunes, chestnuts, chocolate, cocoa, honey, pepper, etc.). Basis therapy is the use of drugs that bind copper and exclude it from the body : * British antiluizit (2,3-dimercaptopropano ) - type injection of 1.25-2.5 mg / kg twice a day for 10-20 days, the gap between the rates 20 days. Another method used : a 200-300 mg twice a day for a few months to obtain. The drug is limited by the pain of injections and the emergence of signs of intoxication in long-term care. * Unitiol 5% in 10 ml every day or every other day for a course 25-30 intramuscular injection. Repeated courses in a couple of months. * D-penitsillamin. Increases down copper with urine : forms complexes, which are easily filtered through renal clubocki. Doses of 0.3-1.3 up to 3-4 grams per day, depending on the value of copper excretion in the urine. Optimal dose drug 0.9-1.2 grams per day. Complications therapy D-penitsillaminom : 1. Hematology-malignant agranulozitos, passing trombo- and lakopenia often been observed in the first 6 weeks of treatment, monitoring blood tests done at three times a week, then once a month. 2. Kidney - principally syndrome is usually detected during the period from 2 months to 2 years after the start of treatment. It can disappear spontaneously or with the application of glucocorticoids. 3. Skin-local or generalized erythema, skin urticarnae, hemorrhagic skin rashes. 4. Allergic to the use of prednisolone. When expressed toxic effects of other helatoobrazutee Connection-trietilentetramin. In rejecting both drugs-tetratiomolibdat. He blocks stripping copper in the gut, as well as links copper in the tissues of the metaboliceski inert form. An alternative method of treatment is far less toxic than D-penitsillamin, zinc sulfate. It inhibits copper absorption in the gut. Dosage : 200 mg three times a day for 30 minutes before eating. Also use vitamins B1 and B6, as well as the plethora of copper blocks the activity. Shown preparations enhance exchange of hepatocytes, antioxidants. Dose drug should be yearly, and in long-term care every two years on the basis of copper with urine, supervisory biopsi liver and the content of copper in bioptatah liver. Clinical improvement under the influence of the treatment is to reduce neurological symptoms, the slowing down of the inflammatory process in the liver. Successful therapy D-penitsillaminom down copper with urine increases by a factor of 3-5. During the first 2-3 weeks of the start of treatment may experience increased neurological symptoms and functional deterioration of the liver, which is then replaced improvement, usually in a few weeks or months. There is a description of the complete disappearance of chronic active hepatitis and cirrhosis of the liver biopsy years after applying the product. Liver transplantation Shown in fulminantna liver failure, progressirovanii liver failure in the face of chronic hepatitis and cirrhosis, with the ineffectiveness of drug therapy. Forecast course disease progression leading to the disease. Forecast improving with the appointment of adequate therapy in the early stages of the disease. Therapy in the late phase does not significantly affect the development of complications. Death occurs predominantly in young age from complications of cirrhosis (bleeding from the veins varikoznorasshirennah oesophagus, liver failure) or fulminantny hepatitis, less frequently, from complications related to the central nervous system injury. Prevention Early detection of the disease. In identifying the defective gene in the homozygous state treatment medhelatirutmi drugs could start in early childhood education. Pregnancy Timely therapy Villson-konovalova disease leads to the full restoration of the liver, the disappearance of the symptoms, the conservation benefits patients, a significant increase in survival, as well as to rehabilitate the reproductive function. Data for pregnant with disease Villson-konovalova showed that the pregnancy is over. This is due to the fact that fruit takes part copper from the mother, as well as possibly raising the level of ceruloplasmine during pregnancy. Damage to liver disease Villson-konovalova, including at the stage of cirrhosis in the absence of warning signs gantries hypertension is not an indication for abortion. The main problem in the conduct of such patients is the need to continue therapy megyeliminiruta during pregnancy, as well as complete its removal may lead to a dangerous to the life of a woman aggravate disease. At the same time, negative effects on the development of a fetus (teratogenic, a violation of the connective tissue) in the application D-penitsillamina completely excluded. The lower dose D-penitsillamina 3-4 times during pregnancy. In recent years, for the maintenance of remission of the disease in pregnant women is the use of drugs zinc. Despite the small experience in the treatment of these drugs during pregnancy, it is safe to use for the fruit.
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