Cuprior

Treatment should only be initiated by specialist physicians with experience in the management of Wilson’s disease. 4). Adult The recommended dose is between 450 mg and 975 mg (3 to 6 and a half film-coated tablets) per day in 2 to 4 divided doses.

Paediatric population The starting dose in paediatrics is lower than for adults and depends on age and may be calculated using body weight. 4). Children and adolescents (≥ 5 years to 18 years) The dose is usually between 225 mg and 600 mg per day (1 and a half to 4 film-coated tablets) in 2 to 4 divided doses.

Children aged < 5 years The safety and efficacy of trientine in children aged < 5 years have not been established. The pharmaceutical form is not suitable for administration to children < 5 years. e. not in mg of the trientine tetrahydrochloride salt).

Special populations Elderly No dose adjustment is required in elderly patients. Renal impairment There is limited information in patients with renal impairment. 4). Method of administration Cuprior is for oral use. The film-coated tablets should be swallowed with water.

The scored film- coated tablet can be divided in two equal halves, if required, to provide a more precise dose or facilitate administration. 5).

Summary of the safety profile The most commonly reported adverse reaction with trientine is nausea. Serious iron deficiency anaemia and severe colitis may occur during treatment. Tabulated list of adverse reactions The following adverse reactions have been reported with the use of trientine for Wilson’s disease.

Frequencies are defined as: very common (≥ 1/10); common (≥ 1/100 to < 1/10); uncommon (≥ 1/1,000 to < 1/100); rare (≥ 1/10,000 to < 1/1,000); very rare (< 1/10,000); not known (cannot be estimated from the available data). System organ class Adverse reactions Blood and lymphatic system disorders Uncommon: sideroblastic anaemia.

Not known: iron deficiency anaemia. Gastrointestinal disorders Common: nausea. Not known: duodenitis, colitis (including severe colitis). Skin and subcutaneous tissue disorder Uncommon: skin rash, pruritus, erythema. Not known: urticaria.

Reporting of suspected adverse reactions Reporting suspected adverse reactions after authorisation of the medicinal product is important. It allows continued monitoring of the benefit/risk balance of the medicinal product. Healthcare professionals are asked to report any suspected adverse reactions via the national reporting system listed in Appendix V.

2). Trientine is a chelating agent which has been found to reduce serum iron levels. 5). The combination of trientine with zinc is not recommended. There are only limited data on concomitant use available and no specific dose recommendations can be made.

In patients who were previously treated with D-penicillamine, lupus-like reactions have been reported during subsequent treatment with trientine, however it is not possible to determine if there is a causal relationship with trientine.

2). The aim of maintenance treatment is to maintain free copper levels in the serum within acceptable limits. The most reliable index for monitoring therapy is the determination of serum free copper which is calculated using the difference between the total copper and the ceruloplasmin-bound copper (normal level of free copper in the serum is usually 100 to 150 microgram/L).

The measurement of copper excretion in the urine may be performed during therapy. Since chelation therapy leads to an increase in urinary copper levels, this may/will not give an accurate reflection of the excess copper load in the body but may be a useful measure of treatment compliance.

4 Worsening of clinical symptoms, including neurological deterioration, may occur at the beginning of chelation therapy due to excess of free serum copper during the initial response to treatment. Close monitoring is required to optimise the dose or to adapt treatment if necessary.

Special populations Overtreatment carries the risk of copper deficiency. 6) and in children where appropriate control of copper levels are required to ensure proper growth and mental development. Patients with renal impairment receiving trientine should remain under regular medical supervision for appropriate control of symptoms and copper levels.

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