WILSON DISEASE
\wˈɪlsən dɪzˈiːz], \wˈɪlsən dɪzˈiːz], \w_ˈɪ_l_s_ə_n d_ɪ_z_ˈiː_z]\
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A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. Clinical features include LIVER CIRRHOSIS; LIVER FAILURE; SPLENOMEGALY; TREMOR; bradykinesia; DYSARTHRIA; CHOREA; MUSCLE RIGIDITY; Kayser-Fleischer rings (pigmented corneal lesions); ATAXIA; and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years. (From Adams et al., Principles of Neurology, 6th ed pp969-71)
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Platidiam
- An inorganic water-soluble platinum complex. After undergoing hydrolysis, it reacts DNA produce both intra interstrand crosslinks. These crosslinks appear to impair replication and transcription of DNA. The cytotoxicity cisplatin correlates with cellular arrest in G2 phase cell cycle.