Initial management of Wilson disease in symptomatic adults without acute liver failure

This figure summarizes the general approach to initial therapy for symptomatic patients with Wilson disease in the absence of acute liver failure. This algorithm is intended for use in conjunction with other UpToDate content. Refer to UpToDate's topic on management of Wilson disease including the safety and efficacy of these therapies. Symptoms related to copper accumulation in the liver may include abdominal pain, jaundice, and ascites. Symptoms related to copper accumulation in the brain may include dysarthria, gait abnormalities, dystonia, tremor, and parkinsonism.

HAV: hepatitis A virus; HBV: hepatitis B virus; INR: international normalized ratio.

* Foods with high copper content include shellfish, nuts, chocolate, mushrooms, and organ meats. A detailed list of the mineral content of foods is available on the United States Department of Agriculture website.

¶ Selecting a chelating agent is informed by patient's risk for drug-related toxicity, drug availability, clinician preference, and cost. Patients at risk for D-penicillamine-associated toxicity include those with kidney disease or severe thrombocytopenia (eg, platelet count <40,000/microL).

Δ Patients with Wilson disease require lifelong therapy unless they undergo liver transplantation. Selecting maintenance therapy is informed by the patient's existing drug regimen, drug safety and efficacy, and patient preferences. Options include a lower dose chelating agent (eg, reducing the dose by approximately one-third) or zinc.

◊ Another trientine formulation, trientine tetrahydrochloride, is available for maintenance therapy. Doses of trientine formulations are generally not equivalent, and local product labeling should be consulted.

Graphic 142796 Version 2.0

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Initial management of Wilson disease in symptomatic adults without acute liver failure