Treatments for Wilson disease
Drug | Mode of action | Neurologic deterioration | Side effects |
---|---|---|---|
D-penicillamine | Chelator, induces cupruria | 10%–20% during initial phase of treatment | Fever, rash, lupus reaction, leukopenia, aplastic anemia, nephrotic syndrome, hepatotoxicity, retinitis |
Trientine | Chelator, induces cupruria | 10%–15% during initial phase of treatment | Gastritis Aplastic anemia |
Tetrathiomolybdate | Aims to reduce level of free serum copper | Developed to protect central nervous system and other tissues vulnerable to free copper | Anemia Hepatotoxicity |
Zinc | Blocks intestinal absorption of copper | No | Gastritis |
Based on information in Roberts EA, Schilsky ML; American Association for Study of Liver Diseases (AASLD). Diagnosis and treatment of Wilson disease: an update. Hepatology 2008; 47:2089–2111.