Wilson's disease is characterized by lesions of the liver and nervous system - in the form of progressive liver cirrhosis and neurological symptoms. The disease is genetically determined, based on clinical signs is a violation of copper metabolism in the body. Normally, the bulk of copper after absorption in the intestine is excreted in the bile and urine.
In Wilson's disease increases the output of copper through the kidneys and therefore decreases its content in the blood. This leads to disruption of the blood exchange oxygen, since copper is part of the blood of specific enzymes responsible for the gas exchange of the body. As a result, due to metabolic disorders of copper, it is accumulated in the liver, kidney, cornea of the eye and brain. In liver cirrhosis because of this - "shrinkage" of liver tissue and proliferation of fibrous tissue, which leads to a violation of the cleaning function of the liver, and this in turn - to the accumulation of toxins in the body. Thus, even in childhood often have episodes of jaundice, revealed enlargement of the liver and spleen. Symptoms of liver disease, the earliest, at a time when neurological symptoms appear only after 20-30 years.
The accumulation of copper in the kidneys followed by the development of glomerulonephritis and neuritis - that is, lesion of renal glomeruli and tubules, and this leads to disruption of ion filtration and protein, and their increased excretion in the urine from the body. The accumulation of copper in the cornea of the eye does not cause blurred vision or movement of the eyeball, but leads to the appearance of specific diagnostic symptom of the disease - yellow circles on the iris, which are visible to the naked eye.
The accumulation of copper in the brain, along with his intoxication due to the slag cleaning impaired liver and kidney function leads to the development of a number of neuropsychiatric symptoms.
The initial manifestations of Wilson's disease may be difficulties in carrying out small-scale movements, slurring of speech. There is shaking hands and head at rest and during stress. There is muscle stiffness, lack of arbitrariness in the movements. Course of disease progression, with episodes of exacerbations and remissions. Frequent death observed in the form of liver disease in children.
In the treatment of Wilson's disease using drugs, normalizing the exchange of copper. Assign them to a strictly individual and depends on the degree of disease activity, and increasing doses under the supervision of the copper concentration in serum. Because these drugs have strong side effects, both prescribed vitamins or bracing means. Patients are encouraged to diet, eliminating foods high in copper (mushrooms, cocoa, chocolate, liver, fish).
