Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Capsule, Oral, as hydrochloride:
Clovique: 250 mg
Syprine: 250 mg
Generic: 250 mg
Mechanism of Action
Trientine is an oral chelating agent structurally dissimilar from penicillamine and other available chelating agents; an effective oral chelator of copper used to induce adequate cupriuresis
Poor (Roberts 2008)
To acetyltrien (chelating activity significantly less than parent) (Roberts 2008)
Urine (1% as parent; 8% as metabolite) (Roberts 2008)
Use: Labeled Indications
Wilson disease: Treatment of patients with Wilson disease who are intolerant to penicillamine.
Limitations of use: Not recommended in cystinuria or rheumatoid arthritis; not indicated for biliary cirrhosis.
Hypersensitivity to trientine or any component of the formulation.
Dosage and Administration
Wilson disease: Oral: Initial: 750 to 1,250 mg/day in divided doses 2 to 4 times daily; increase dose if clinical response not adequate or the concentration of free serum copper is persistently >20 mcg/dL; maximum dose: 2,000 mg/day. AASLD practice guidelines suggest typical doses of 750 to 1,500 mg/day in 2 to 3 divided doses with maintenance therapy of 750 to 1,000 mg/day (Roberts 2008). Optimal long-term maintenance dosage should be determined at 6- to 12-month intervals.
Refer to adult dosing. Use with caution; initiate at lower end of the dosing range.
Wilson disease: Children and Adolescents: Oral: Initial: 20 mg/kg/day (round dose to the nearest 250 mg) in 2 to 3 divided doses; maximum initial daily dose: 1,000 mg/day; titrate dose based on clinical response and free serum copper (non-ceruloplasmin bound copper) concentrations and/or 24-hour urinary copper excretion; usual maintenance dose: 900 to 1,500 mg/day in 2 to 3 divided doses (AASLD [Roberts 2008]; EASL 2012; ESPGHAN [Socha 2018]).
Maximum daily dose:
Children: 1,500 mg/day (AASLD [Roberts 2008]; EASL 2012; ESPGHAN [Socha 2018])
Adolescents: 2,000 mg/day
Administer at least1 hour before or 2 hours after meals and at least 1 hour apart from any drug, food, or milk. Do not open or chew capsule, swallow whole with water; any skin exposed to the contents of a capsule should be promptly washed with water.
Should be taken 1 hour before or 2 hours after meals and at least 1 hour apart from any drug, food, or milk.
Store at 2°C to 8°C (36°F to 46°F).
Carbonic Anhydrase Inhibitor Diuretics: May decrease the serum concentration of Trientine. Monitor therapy
Polyvalent Cation Containing Products: May decrease the serum concentration of Trientine. Management: Avoid concomitant administration of trientine and oral products that contain polyvalent cations. If oral iron supplements are required, separate the administration by 2 hours. If other oral polyvalent cations are needed, separate administration by 1 hour. Consider therapy modification
Frequency not defined.
Central nervous system: Dystonia, myasthenia gravis, neurological deterioration (worsening; European Association for the Study of the Liver 2012)
Endocrine & metabolic: Iron deficiency
Gastrointestinal: Gastritis (Roberts 2008)
Hypersensitivity: Fixed drug eruption (Roberts 2008)
Neuromuscular & skeletal: Muscle spasm, systemic lupus erythematosus
<1%, postmarketing, and/or case reports: Aplastic anemia (Roberts 2008), pancytopenia (Roberts 2008), sideroblastic anemia (reversible; Roberts 2008)
Concerns related to adverse effects:
- Anemia: May cause iron-deficiency anemia; monitor closely, especially women.
- Copper deficiency: Induced by treatment; may lead to hepatic iron overload and/or sideroblastic anemia; reassess dose (Roberts 2008).
- Neurologic worsening: May occur with treatment initiation; less common than with penicillamine (Roberts 2008).
- Hypersensitivity: Not reported with use; however, industrial workers exposed to trientine for prolonged periods have reported asthma, bronchitis, and dermatitis.
Periodic 24-hour urinary copper assessment (every 6 to 12 months); serum non-ceruloplasmin bound copper; LFTs; CBC; INR; urinalysis (Roberts 2008); fever and skin changes during the first month of therapy
Treatment of Wilson disease should be maintained during pregnancy; dose reduction (25% to 50% of prepregnancy dose) should be considered (Roberts 2008).
What is this drug used for?
- It is used to treat Wilson's disease.
Other side effects of this drug: Talk with your doctor right away if you have any of these signs of:
- Lupus like rash on the cheeks or other body parts, sunburn easy, muscle or joint pain, chest pain or shortness of breath, or swelling in the arms or legs
- Difficulty moving
- Muscle spasm
- Severe loss of strength and energy
- Muscle weakness
- Signs of a significant reaction like wheezing; chest tightness; fever; itching; bad cough; blue skin color; seizures; or swelling of face, lips, tongue, or throat.
Note: This is not a comprehensive list of all side effects. Talk to your doctor if you have questions.
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