Table 3.

Anti-copper drugs used in treatment of Wilson’s disease

	Drugs	Route of administration & Dose	Adverse effects	Special remarks
A	Copper chelators
	a. Dimercaprol (British anti-Lewisite)[68]	Deep intramuscular 5 mg/kg bolus f/b 2.5 mg/kg 1.5 ml (10% suspension in peanut oil) twice a day	Painful Hematoma & sterile abscess at injection site HT & tachycardia (dose dependent) Nausea, vomiting, abdominal pain Headache, paresthesia	BAL is lipid-soluble with best blood-brain barrier permeability amongst all copper-chelators Tachyphylaxis-efficacy reduces with continuous use Not preferred now – May be tried as a 1-month course in combination of DPM in refractory cases
	b. Penicillamine[1,68]	Oral (in 2-3 divided doses) - Start low-go slow policy Adults: Up to 2 g/day Children: 20 mg/kg/day	Paradoxical neurological worsening (10-50% cases) Early drug HSE (<3 weeks): fever, skin rash, thrombocytopenia, leucopenia, lymphadenopathy Late-onset HSE (months to years): lupus, Goodpasture syndrome, myasthenia, skin lesions/Penicillamine dermatopathy, ageusia, bone-marrow suppression, optic neuritis	Safe in pregnancy but avoid breast-feeding Anti-pyridoxine effect: Vit. B6 supplementation especially in pregnancy, acute illness or nutritional deficiencies Take away from meals Psychiatric features less responsive than neurological features
	c. Trientine[68,71]	Oral (in 3 divided doses) - Start low-go slow policy Adults: 750 mg – 2 g/day Children: 20 mg/kg/day	Paradoxical neurological worsening (up to 26% cases) Late-onset HSE: lupus, nephritis Sideroblastic anemia Skin rash; ageusia Pancolitis; hemorrhagic gastritis	Safe in pregnancy but avoid breast-feeding Take away from meals Monitor for iron deficiency
	d. Unithiol (Dimercapto Propane sulfonate)[68]	Oral 200 mg twice daily	Early HSE : fever, leucopenia Nausea, dysgeusia	A sulphonic acid derivative of dimercaprol Only few reports in literature; no reports from India to date
B	Drug reducing gastrointestinal absorption of copper
	Zinc[1,68]	Oral 150 mg (adults) and 75 mg (children) of elemental zinc/day in 2–3 divided doses	Gastric irritation (especially with zinc sulphate salt) Elevation of serum amylase and lipase (asymptomatic) Paradoxical neurological worsening (rare)	Food interferes with absorption: give away from meals Safe in pregnancy Relatively slow to act – primarily used as maintenance therapy after initial therapy with copper chelators
C	Drug which can chelate copper and reduce gastrointestinal absorption of copper
	Tetrathiomolybdate (Undergoing clinical trials – not commercially available)[71]	Oral 20 mg 3 times per day with meals and 3 times without meals	Bone-marrow suppression (reversible) Acute hepatitis Elevated aminotransferases, triglycerides and cholesterol Seizures Paradoxical neurological worsening (less common)	Damage epiphyseal bone-growth in animal studies – dangerous to use in children and adolescents