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Wilson's disease
Adult: Determine the optimal dose to obtain a negative copper balance initially by measuring the 24-hour urinary copper excretion and subsequently by monitoring free copper in serum. Initially, 1,500-2,000 mg daily in divided doses, adjusted according to response. Once disease control is achieved, reduce to the maintenance dose of 750-1,000 mg daily. Maintenance dose of 2,000 mg daily must not be continued for >1 year. Alternative dosing recommendation: 750-1,500 mg daily in divided doses, continued for 3 months if there are no adverse reactions. Max: 2,000 mg daily. If patients cannot tolerate a 1,000 mg daily starting dose, give an initial dose of 250 mg daily then increase gradually to provide better control and to reduce adverse effects. Individualise dosage and use the lowest effective dose to achieve disease control. Dosage recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
Elderly: Up to 20 mg/kg daily in divided doses; adjust to the lowest effective dose to achieve disease control.
Child: ≤12 years 20 mg/kg daily in 2-3 divided doses; >12 years Initially, 20 mg/kg daily in 2-3 divided doses, then maintenance dose of 750-1,000 mg daily. Individualise dosage and use the lowest effective dose to achieve disease control. Dosage recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
Elderly: Up to 20 mg/kg daily in divided doses; adjust to the lowest effective dose to achieve disease control.
Child: ≤12 years 20 mg/kg daily in 2-3 divided doses; >12 years Initially, 20 mg/kg daily in 2-3 divided doses, then maintenance dose of 750-1,000 mg daily. Individualise dosage and use the lowest effective dose to achieve disease control. Dosage recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
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Lead poisoning
Adult: 1,000-1,500 mg daily in divided doses until urinary lead levels are stabilised at <0.5 mg daily. Individualise dosage and use the lowest effective dose. Treatment recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
Elderly: 20 mg/kg daily in divided doses until urinary lead levels are stabilised at <0.5 mg daily. Individualise dosage and use the lowest effective dose. Treatment recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
Child: In patients whose blood lead levels are <45 mcg/dL: 15-20 mg/kg daily in 2-3 divided doses. Individualise dosage and use the lowest effective dose. Treatment recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
Elderly: 20 mg/kg daily in divided doses until urinary lead levels are stabilised at <0.5 mg daily. Individualise dosage and use the lowest effective dose. Treatment recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
Child: In patients whose blood lead levels are <45 mcg/dL: 15-20 mg/kg daily in 2-3 divided doses. Individualise dosage and use the lowest effective dose. Treatment recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
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Severe active rheumatoid arthritis
Adult: In patients who failed to respond to adequate conventional treatment: Initially, 125-250 mg daily for 4 weeks, then increase in increments of 125-250 mg daily at 4- to 12-week intervals until remission occurs. Maintenance: 500-750 mg daily in divided doses, but doses of up to 1,500 mg daily may be required in some patients. Discontinue treatment if there is no improvement within 12 months or after 3-4 months of therapy with 1,000-1,500 mg daily doses. If remission is sustained for 6 months, reducing the daily dose gradually in decrements of 125-250 mg every 12 weeks may be attempted. Individualise dosage and use the lowest effective maintenance dose to achieve symptom suppression. Dosage recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
Elderly: Initially, 125 mg daily for the 1st month, then increase in increments of 125 mg daily at 4- to 12-week intervals until the lowest effective maintenance dose is achieved. Max: 1,000 mg daily.
Elderly: Initially, 125 mg daily for the 1st month, then increase in increments of 125 mg daily at 4- to 12-week intervals until the lowest effective maintenance dose is achieved. Max: 1,000 mg daily.
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Chronic active hepatitis
Adult: For maintenance therapy after the disease has been controlled by corticosteroids: Initially, 500 mg daily in divided doses, then gradually increased over 3 months to the maintenance dose of 1,250 mg daily. Concurrently, gradually reduce the dose of corticosteroids over a 3-month period. Individualise dosage and use the lowest effective dose. Treatment recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
Elderly: Not recommended. Treatment recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
Elderly: Not recommended. Treatment recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
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Cystinuria
Adult: Treatment: 1,000-4,000 mg daily in 4 divided doses; adjust to maintain urinary cystine concentration of <200 mg/L. Usual dose: 2,000 mg daily. If 4 equal doses are not feasible, give the larger portion at bedtime. An initial dose of 250 mg daily, then increased gradually may provide better control and reduce adverse effects. Prophylaxis: 500-1,000 mg daily at bedtime; maintain urinary cystine concentration of <300 mg/L. Individualise dosage and use the lowest effective dose to maintain the required urinary cystine levels. Dosage recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
Elderly: Use the lowest effective dose to maintain urinary cystine <200 mg/L.
Child: 20-30 mg/kg daily in 2-3 divided doses; adjust to maintain urinary cystine concentration of <200 mg/L. Alternative dosing recommendation: 30 mg/kg daily in 4 divided doses. If 4 equal doses are not feasible, give the larger portion at bedtime. Individualise dosage and use the lowest effective dose to maintain the required urinary cystine levels. Dosage recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
Elderly: Use the lowest effective dose to maintain urinary cystine <200 mg/L.
Child: 20-30 mg/kg daily in 2-3 divided doses; adjust to maintain urinary cystine concentration of <200 mg/L. Alternative dosing recommendation: 30 mg/kg daily in 4 divided doses. If 4 equal doses are not feasible, give the larger portion at bedtime. Individualise dosage and use the lowest effective dose to maintain the required urinary cystine levels. Dosage recommendations may vary among individual products and between countries (refer to detailed product or local guidelines).
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