What is the treatment for Wilsons disease?
Early diagnosis and copper chelation therapy are the mainstays of therapy.
The chelation therapy of choice is oral d -penicillamine. d -Penicillamine should be administered concomitantly with pyridoxine to prevent vitamin B6 deficiency.
Side effects include rash, fever, thrombocytopenia, relative eosinophilia with total leukopenia, and reversible lupus-like and myasthenia gravis-like syndromes.
Trientene and zinc acetate are alternative agents with fewer side effects.
Liver transplantation is recommended in patients with fulminant hepatic failure and end-stage liver cirrhosis but is not generally recommended for patients with neurologic disease without pronounced liver involvement.
