They are changes emotsyonalno-woleva areas declining mental activity and intelligence. At the same time, there are cases in the current benign gepatotserebralna malnutrition when sick for a long time neurological symptoms is missing or had very light symptoms, which do not violate the compensation. Such patients are normally detected in the random survey of families of patients with a detailed picture of the disease. 3. Psychiatric violations emotional, psychosis, a breach of conduct educational activities. 4. Hematology-gemoliz, anemia, thrombocytopenia, a breach of blood coagulation. The 15% of patients may experience severe phenomenon vnutrisosudistogo hemolysis. Gemoliz usually temporary passes to prior vivid clinical symptoms of liver failure within a few years. Sometimes, it may proceed simultaneously with acute liver failure. The influence of large quantities of free copper in the plasma membranes of cells and haemoglobin. 5. Kidney - kanalzeve violations (partial or complete Fanconi syndrome), reduced clubockova filtering stone. The defeat was kidney perifericescimi circulation, mikrogematuriei, low proteinuriei, increased concentration of serum creatinine blood. As an early symptom may be a macro and mikrogematuria. Most in the urine showed treonin, tirosin, lysine, valin, fenilalanin. 6. Ophthalmic-ring Keizer-fleishera, cataracts (containing copper deposits in aphykia capsule). 7. Endocrine - amenorea, spontaneous abortions, delay sexual development, ginekomastia, girsutizm, obesity, gipoparatiroidizm. 8. Cardiovascular-cardiomyopathy, incredible. 9. Musculoskeletal - osteomalacia, osteoporosis, artropatia, arthralgia. 10. Stomach - cholelithiasis, pancreatitis, spontaneous bacterial peritonitis. 11. Title-blue golf nogtevogo the floor, vascular purpura, hyperpigmentation skin acantosis nigricans. Diagnostics suspicion of the existence of the disease Villson-konovalova should arise : * unspecified etiology of chronic hepatitis and cirrhosis; * Fulminantna liver failure; * Unexplained increase aminotransferaz; * Availability of neurological changes unknown etiology, behavioural change; * Psychological symptoms, combined with signs of liver disease; * Inexplicable acquired hemolytic anemia; * Family history of gepatotserebralna malnutrition. The (screening) tests for the diagnosis of the disease Villson-konovalova : * detection ring Keizer-fleishner : not found in 50-62% of patients with neurological symptoms; Might not be available to the 5% of patients with early signs of central nervous system damage; * Identification reduce ceruloplasmine in the serum to a level <20 mg / m (rule 25-50 mg / m) : level of <5 mg / m = absolute proof Villson-konovalova disease. Moderate decline may meet the geterozigotnykh carriers of a gene in liver cirrhosis another etiology, in the Gulf malabsorbtion, nefroticescom syndrome, etc.; In 10-15% of patients with abdominal form of the disease ceruloplasmine may be within normal limits; * increase in the non-zerouloplazminom copper in the serum (300 µ g / L and>); * increasing the copper content in the organs, particularly in the liver bioptatah (over 250 µ g / g dry weight); * increased copper excretion in urine (more than 200 micrograms per day with an average <70 µ g / day); * D-penitsillaminovy test-improving daily excretion of copper to a level of> 1500 g, the rate of a significant increase in copper excretion in the urine were observed; * the high level of the isotope in copper ceruloplasmin in the rule is the lack of a peak after 48 hours; the t diagnostically meaningful only in patients with normal levels of ceruloplasmine; * genetic research : the popular siblings and other family members probanda. Many mutations ATP7B gene responsible for the disease Villson-konovalova, does not allow to use molekulyarno-genetic What Methods of Screening for diagnosis of the disease, but they can be used for diagnosis of siblings and family members probanda. All sibsa probanda subject to mandatory survey to exclude Wilson disease, the risk of which for them is 25%. There complexity differential diagnosis of asymptomatic homozygous and geterozigotnykh holders ATP7B gene, as the 10-20% can be expected to reduce ceruloplasmine and irregularities in the exchange of copper, and in the course of bessimptomnom disease may not ring Keizer-fleishera and giperkiprurii. To quantify copper in the liver bioptatah use spectrofotometriu, rentgenstrukturny analysis. Also for the diagnosis using liver uptake of radioactive copper. The radioactivity of the liver in 24 and 2 hours after intravenous injection of radioactive copper in the rate of 1.4-9 well, as a Villson-konovalova 0.2-0.3. Geterozigotnye carriers and patients with other liver diseases have ratio of one. The kinetics of radioactive copper to differentiate disease from Villson-konovalova gepatotserebralnogo syndrome and liver diseases.
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