Wilson’s disease in children: Recent update on pathophysiology and management

Table 4 Medications used in Wilson’s disease

Medication	Mechanism of action	Dose	Side effects
D-penicillamine	Cu chelator	Initial: 20 mg/kg/day, 3 times a day (one hour before and two hours after meal). Maintenance: 10-20 mg/kg/day. Oral pyridoxine: 25-50 mg/day. This drug is used as initial and maintenance	Early: Hypersensitivity reaction manifested as fever, rash, lymphadenopathy, pancytopenia. Late: Proteinuria, nephrotic syndrome, drug associated systemic lupus erythematosus, agranulocytosis, thrombocytopenia, Dermatopathy (cutis laxa)
Trientine	Cu chelator	Same as D-penicillamine	Less toxic than D-penicillamine. Toxicity includes sideroblastic anemia, nephrotoxicity, skin and mucosal lesion
Zinc	Inhibit Cu absorption. Stimulate hepatic metallothionein synthesis	< 50 kg-75 mg/day three times daily. > 50 kg-150 mg/day three times daily. It is used as maintenance and adjunctive therapy	Headache, gastrointestinal upset, iron deficiency
Ammonium tetrathiomolybdate	Inhibit Cu absorption. Cu chelator	100-200 mg/day. It is not yet Food and Drug Administration approved	Elevation of aminotransferase