Skip to main content

Table 2 Treatment recommendations for Wilson’s disease [26]

From: National alliance for Wilson’s disease: health policy in Costa Rica

• Initial treatment for symptomatic patients with Wilson’s disease should include a chelating agent (D-penicillamine or trientine). Trientine may be better tolerated • Patients with acute liver failure due to Wilson’s disease should be treated with liver transplantation when the revised King’s score is 11 or higher
GRADE II-1, B, 1 GRADE II-2, B, 1
AASLD Class I, Level B AASLD Class I, Level B
• Zinc may have a role as a line therapy in neurological patients • Patients with decompensated cirrhosis, unresponsive to chelation treatment, should be evaluated promptly for liver transplantation
GRADE II-2, C, 2 GRADE II-2, B, 1
AASLD Class II, Level C AASLD Class I, Level B
• Treatment of presymptomatic patients or those with neurological disease on maintenance therapy can be accomplished with a chelating agent or with zinc • Treatment for Wilson’s disease should be continued during pregnancy, but dosage reduction is advisable for D-penicillamine and trientine
GRADE II-1, B, 1 GRADE II-3, B, 1
AASLD Class I, Level B AASLD Class I, Level C
• Treatment is lifelong and should not be discontinued, unless liver transplantation is performed • For routine monitoring, serum copper and ceruloplasmin, liver enzymes and international normalized ratio, functional parameters, complete blood count and urine analysis as well as physical and neurological examinations should be performed regularly, at least twice annually
GRADE II-1, B, 1 GRADE II-2, B, 1
AASLD Class I, Level B AASLD Class I, Level C
• If zinc is used, careful monitoring of transaminases is needed, with changing to chelators if these laboratory parameters are increasing • The 24-h urinary copper excretion on medication and after 2 days of cessation of therapy should be measured at least yearly. The estimated serum non ceruloplasmin bound copper may be another useful parameter to control therapy
GRADE C1  
AASLD Class I, Level B  
• Patients should avoid intake of foods and water with high concentrations of copper, especially during the year of treatment  
GRADE II-3, B, 2 GRADE II-3, B, 1
AASLD Class I, Level C AASLD Class I, Level C
  1. 8th International Meeting on Wilson’s disease. Leipzig, 2001