Morphology In liver tissue are fat liver hepatocytes, periportalny fibrosis, necrosis submassivee hepatocytes, makronodullarnyi cancer. In the kidney, and fat gidropica dystrophy with copper deposits in proximal bone. Clinical presentation Clinical Manifestations : 1. - Liver cirrhosis, chronic active hepatitis, fulminantnaya liver failure. At the initial stage of changes in the liver non-small and srednecapelnaya muscular dystrophy and necrosis isolated hepatocytes, periportalny fibrosis. The clinic has chronic hepatitis high level of activity with jaundice, high aminotransferaz, gipergammaglobulinem ation. If the-cirrhosis gantries with hypertension and pechenernkletocna failure. Fulminantnaya liver failure is a rare manifestation gepatotserebralna malnutrition. Collaboration among adolescents and young people. Specification of which distinguished it from fulminantnam hepatitis viral etiology : a small increase in transaminaz (with a predominance of a more active ACT), the low level of alkaline phosphatase, it is a low level of albumin in the serum, a high level of direct or indirect cium (vnutrisosudisty gemoliz) gemoglobinurija, high levels of copper in the liver cell and its excretion in urine. Often accompanied by hemolytic anemia associated with the massive release of copper from the liver. The only effective method of treatment is liver transplantation. There abdominalnaya form Kerra-defeated liver prevails throughout the illness and early complicated liver failure. In the debut Development otern-astiticeski syndrome, extent is not in accordance with the appearance of other signs gantries hypertension. The persistence of a large number of untied copper in the serum and increased deposit not only in the liver but also in other organs will damage the brain, eye, kidney, skeletal, gemolizu cells. 2. Neurological - ekstrapiramidnye, zerebelliarnae, psevdobulbarnae violations apnoea seizures. The two main forms of disease-rigidno-aritmogiperkinetical or older, and drojatelnaya significantly differ in their clinical manifestations. The first is characterized by the rapid development of rigidnosti and the irregular giperkinezov atetoidnyi or thorsin-spasticesc th character. Rigidity extends to the back muscles, limbs and the muscles involved in swallowing and speech act. There amimia, disfagia, dizartria. Secondary becomes bound, podpragivatei. Muscle rigidity pristupoobrazno can grow, especially in relation to the arbitrary movements and emotional. Patients often frozen in the most awkward positions. The distal limbs divisions often formed contracture. Increasing rigidity quickly into full obezdwijennosti. This form of the disease begins in childhood, from 7 to 15 years. Viszerle disorders may occur earlier, at the age of 3-5 years. Typically, expressed signs of liver disease, which often precede the development of neurological symptoms. Flexorno-ekstenzorn s tremor. Its severity ranges from barely noticeable drojania hands before tremora entire body. Tremor increases with the excitement and purposeful action. Moderate tremor in some patients can be placed on one side. Tremor elongated fingers typical hands • distonia observed in all patients. Symptoms drojatelno-rigidna of varying extent. Defined gipomimia, gipersalivacia, zatrudnennaya monotonic speech lower intelligence. Akinetiko-rigidnaya form was pronounced rigidnam syndrome affecting different muscle groups. The detailed stage shows giperkinez as typified by slapping wings ", which may be affixed intenzionny component dizartria, disfagia, mozgechkove disorders mioclonii. No specific therapy increased exposure has expressed contrakturam, obezdwijennosti, gross dementia. Patients who are ekstrapiramidna patients can develop pyramid mono and hemiparesis. Such cases are ekstrapiramidno-kork good gepatotserebralna form of malnutrition, which is different from other forms of significant defeat cortical hemispheres. Patients often has epileptic seizures general, and especially jacksonovsky nature, the plight of intelligence gross violations of the individual. Mental violations may occur in patients with other forms of the disease.