Autosomal recessive condition affecting copper excretion; originally known as progressive lenticular degeneration
Leads to progressive liver damage and cirrhosis, commonly associated ...
Typically presents in children between ages 9 and 13 years with signs of liver disease
Older patients between ages 15 and 21 years are more likely to present with neurologic manifestat...
Avoid copper-rich foods (2)[C].
Check home water for copper content (2).
D-penicillamine: 250 to 500 mg/day initially. Increase by 250 mg every 4 to 7 day...
24-hour urinary copper excretion (annually or more frequent if questioning compliance): 200 to...
Suspect Wilson disease in a patient with liver disease of unknown origin, coexisting neuropsychologic symptoms, or with atypical autoimmune hepatitis/nonalcoholic steatohepatitis that ...
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