Trientine Hydrochloride and Iron-deficiency anemia

• Generic Name: trientine
• Dosage Forms: n.a.
• Other Brand Names: Syprine, Clovique

Used to promote excretion of copper in patients with Wilson disease (hepatolenticular degeneration) who are intolerant of penicillamine (designated an orphan drug by FDA for this use). Manufacturer states that trientine should be used when continued treatment with penicillamine is no longer possible (e.g., because of intolerable or life-threatening adverse effects).

American Association for the Study of Liver Diseases (AASLD) recommends a chelating agent (penicillamine or trientine) for initial therapy of symptomatic patients. Penicillamine traditionally used as the chelating agent of choice, but is associated with many adverse effects. Trientine may be better tolerated and should be considered in patients who cannot take penicillamine.

Disease manifestations, particularly neurologic symptoms, may worsen during initial therapy.

Once symptoms and laboratory abnormalities stabilize with initial chelating therapy (typically after 2–6 months, but potentially up to 5 years), patients may continue on a lower dosage of the chelating agent or switch to zinc for maintenance therapy.

Because substantial morbidity and mortality can be prevented by treating asymptomatic/presymptomatic patients, AASLD and other experts recommend that such patients also be treated with a chelating agent (generally at a lower dosage than that used in symptomatic patients) or zinc.

Treatment is lifelong unless a liver transplant is performed. Discontinuance of therapy may result in clinical decompensation and/or death.

Use in conjunction with low copper diet.

Not recommended in the treatment of cystinuria or rheumatoid arthritis due to lack of efficacy in these conditions.

Not indicated for treatment of biliary cirrhosis.