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Wilson’s disease — a hereditary syndrome transmitted as an autosomal recessive trait in which a decrease of ceruloplasmin permits accumulation of copper in vari- ous organs (brain, liver, kidney, and cor- nea) associated with increased intestinal absorption of copper. A pigmented ring (Kayser–Fleischer ring) at the outer mar- gin of the cornea is pathognomonic. This syndrome is characterized by degenera- tive changes in the brain, cirrhosis of the liver, hemolysis, splenomegaly, tremor, muscular rigidity, involuntary movements, spastic contractures, psychic disturbances, dysphagia, and progressive weakness and emaciation жүктеу 0,76 Mb. Достарыңызбен бөлісу:

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