Information about Trientine
Trientine is an oral copper chelating agent used to treat Wilson disease.
Liver safety of Trientine
Trientine has not been associated with worsening of serum enzyme elevations during therapy or with cases of clinically apparent liver injury with jaundice.
Mechanism of action of Trientine
Trientine (trye’ en teen) is an orally available copper chelating agent that is used to treat Wilson disease, an inherited abnormality of copper metabolism that leads to excess copper accumulation and injury to liver and brain. The metabolic defect in Wilson disease is caused by mutations in ATPase7B, a hepatic enzyme responsible for transmembrane transport and excretion of copper into the bile. The metabolic detect leads to accumulation of free copper in liver and blood and secondarily in other organs, particularly brain and kidney. The disease usually presents in childhood or adolescence with neurologic syndromes, signs of advanced liver disease and hemolytic anemia. Trientine is one of several copper chelating agents that lower blood and tissue copper levels and, when given chronically, prevent copper accumulation and injury in Wilson disease. Other copper chelating agents include d-penicillamine and dimercaprol (British anti-Lewisite [BAL]). Trientine has a polyamine-like structure and chelates copper by creating a stable complex with the four nitrogens in a plantar ring. Trientine complexed to copper is excreted in the urine.
FDA approval information for Trientine
Trientine was approved for use in the United States in 1969, and current formal indications are for treatment of patients with Wilson disease who are intolerant of penicillamine. Trientine is available in capsules of 250 mg generically and under the brand name Syprine.
Dosage and administration for Trientine
The recommended dose is 750 to 1250 mg for adults and 500 to 750 mg for children given in 2 to 4 divided doses daily initially, which can be raised to a maximum of 2000 mg daily for adults and 1500 mg daily for children.
Side effects of Trientine
Side effects are generally mild and may include headache, arthralgias, myalgias, nausea, anorexia, diarrhea, rash and renal dysfunction. Uncommon, but potentially severe adverse events include hypersensitivity reactions, lupus-like syndromes and pancytopenia.
Copper Chelators (for Wilson Disease)