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Wilson disease, also known as hepatolenticular degeneration, is a rare autosomal recessive disorder of copper metabolism affecting multiple systems. Epidemiology It affects 1 in 30,000-40,000 individuals 12. 1 in 90 individuals are a heterozygous carrier 18. Clinical presentation See more The clinical presentation is non-specific and incredibly varied, typically manifesting by early adulthood 5,11,13,15: 1. hepatic manifestations: liver disease ultimately resulting in … See more It is a disorder that results from abnormal ceruloplasmin metabolism, as a result of a variety of mutations in the ATP7Bgene. Total body copper is elevated that has toxic effects on hepatocytes with copper deposition and … See more General treatment is focussed on chelation of the copper, and chelating agents used include zinc sulfate, trientine dihydrochloride, ammonium tetrathiomolybdate and penicillamine 7,18,22. Otherwise, … See more Please see individual articles: 1. Wilson disease: hepatobiliary manifestations 2. Wilson disease: CNS manifestations 3. Wilson disease: musculoskeletal manifestations See more WebThe therapeutic process can be helpful to make sense of your thoughts, identify barriers to change and explore new ways of coping. Therapy can be empowering. (289) 799-4339 Port Hope, ON L1A... on track lease service refugio tx
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