Table 3.

Pharmacological Therapy for Wilson’s Disease

Drug	Mode of Action	Maintenance Dose	Side effects
Trientine	Induction of urinarycopper excretion by chelating action	750-1,000 mg/day three times a day; children, 20-25 mg/kg/day	Gastritis, in rare cases aplastic anemia and sideroblastic anemia, neurological deterioration during initial phase of treatment (about 26% [130])
D-Penicillamine	Induction of urinary copper excretion by chelating action	750-1,000 mg/day three times a day; children: 20 mg/kg/day	Fever, rash, proteinuria, lupus-like reation, aplastic anemia, leukopenia, thrombocytopenia, nephrotic syndrome, degenerative change in skin, elastosis perforans serpingosa, serous retinitis, hepatotoxicity, neurological deterioration during initial phase of treatment (about 50% [110])
Zinc	Blockage of copper absorption by inducing metallothionein in enterocytes	150 mg/day, three times a day; children: 50-75 mg/day	Gastritis, biochemical pancreatitis, zinc accumulation, possible changes in immune function
Tetrathiomolybdate	Detoxifying copper in plasma and blocking copper absorption by complexation with copper	20 mg, three times with meals and three times between meals [108]	Anemia, neutropenia, hepatotoxicity, neurologic deterioration during initial treatment (about 4% [108])