Wilson's disease medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]

Overview

Medical therapy is the mainstay of therapy for treatment patients with Wilson's disease. The treatment option include copper chelators as penicillamine trientine. The treatment also includes Zinc to prevent copper reaccumulation.

Recommendations for the treatment of Wilson's Disease (DO NOT EDIT)

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Initial treatment for symptomatic patients should include a chelating agent (D-penicillamine or trientine). Trientine may be better tolerated. Patients should avoid intake of foods and water with high concentrations of copper, especially during the first year of treatment. Treatment of presymptomatic patients or those on maintenance therapy can be accomplished with a chelating agent or with zinc. Trientine may be better tolerated. Treatment in Specific Clinical Situations Patients with acute liver failure due to WD should be referred for and treated with liver transplantation immediately. Patients with decompensated cirrhosis unresponsive to chelation treatment should be evaluated promptly for liver transplantation. Treatment for WD should be continued during pregnancy, but dosage reduction is advisable for D-penicillamine and trientine. Treatment is lifelong and should not be discontinued, unless a liver transplant has been performed. Treatment Targets and Monitoring of Treatment For routine monitoring, serum copper and ceruloplasmin, liver biochemistries and international normalized ratio, complete blood count and urinalysis (especially for those on chelation therapy), and physical examination should be performed regularly, at least twice annually. Patients receiving chelation therapy require a complete blood count and urinalysis regularly, no matter how long they have been on treatment. The 24-hour urinary excretion of copper while on medication should be measured yearly, or more frequently if there are questions on compliance or if dosage of medications is adjusted. The estimated serum non–ceruloplasmin bound copper may be elevated in situations of non-adherence and extremely low in situations of overtreatment.

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Medical therapy

• The mainstay stay of therapy for treatment of Wilson's disease is the medical therapy.
• The treatment of Wilson's disease is accomplished through two mechanisms:
  • Removing of the excess copper in the body by copper chelators
  • Preventing reaccumulation of the copper
• Removal of the copper:
  • Preferred regimen (1): D-penicillamine 20 mg/kg PO q12h
  • Alterantive regimen (1):  Trientine hydrochloride 500 to 750 mg PO q12h/q6h
• Preventing reaccumulation:
  • Preferred regimen (1): Zinc acetate PO 50 mg q8h