What is Trientine?

Trientine (Trientine) is an oral copper chelator used to treat Wilson's disease (hepatolenticular degeneration), an inherited abnormality in copper metabolism that causes excessive copper accumulation and damage to the liver and brain. The metabolic defects in hepatolenticular degeneration are caused by mutations in ATPase 7B, a liver enzyme responsible for copper transport across membranes and excretion into bile. Metabolic testing results in the accumulation of free copper in the liver and blood, followed by accumulation in other organs, particularly the brain and kidneys. The disease usually presents in childhood or adolescence, with neurological syndromes, signs of advanced liver disease, and hemolytic anemia.

Trientine is one of several copper chelators that reduces copper levels in the blood and tissues, and long-term use may prevent copper accumulation and damage in Wilson's disease. Trientine has a polyamine-like structure that chelates copper by forming a stable complex with four nitrogens in the plantar ring. Trientine bound to copper is excreted in the urine. Trientine was approved for use in the United States in the 1990s, and its current official indication is the treatment of patients with Wilson disease who are intolerant to penicillamine.

Trientine is generally available in capsule form under the brand nameSyprine or Cufence. The recommended dose for adults is 750 to 1250 mg and for children is 500 to 750 mg, initially given in 2 to 4 divided doses per day and increased to a maximum of 2000 mg per day for adults and 1500 mg per day for children. Side effects are usually mild and may include headache, arthralgia, myalgia, nausea, anorexia, diarrhea, rash, and renal insufficiency. Rare but potentially serious adverse events include anaphylaxis, lupus-like syndrome, and pancytopenia.

Reference materials:https://www.ncbi.nlm.nih.gov/books/NBK548119/