Wilson's disease, the most common inherited disorder of copper metabolism, results from a failure of the copper
excretory pathway. This leads to toxic accumulation of copper in the liver and eventually other organs.1 The worldwide prevalence of Wilson's disease is estimated to be one in 30,000 individuals.2 The gold standard for diagnosis of
Wilson's disease is liver biopsy, although other tests can be a useful guide.3
The condition is effectively treated with a low copper diet and chelating agents that bind copper to facilitate
its excretion from the body. Syprine® (trientine hydrochloride) is a chelating agent indicated
for treatment of patients with Wilson's disease who are intolerant of the first-line treatment, penicillamine.4
Indications and Usage
Syprine® (trientine hydrochloride) is used to treat Wilson's disease in patients who cannot take the medication known as penicillamine. Wilson's disease is a condition where the body stores too much copper. Syprine is not recommended to treat cystinuria (a condition where a protein known as cystine is excreted into the urine), rheumatoid arthritis, or a disease affecting the bile ducts in the liver known as biliary cirrhosis.
Important Safety Information
Do not take Syprine if you are allergic to it or any parts of the formulation.
You should remain under regular medical supervision the entire time you are taking Syprine. Your doctor should regularly check to see
if you have iron deficiency anemia. This is particularly important for women.
Take Syprine on an empty stomach, at least one hour before a meal or two hours after a meal and at least one hour apart from any other drug, food, or milk. The capsules should be swallowed whole with water and should not be opened or chewed. For the first month of treatment, take your temperature every night, and report any symptom such as fever or skin rash to your doctor.
The following adverse reactions have been reported from a clinical study: iron deficiency and a condition affecting the immune system known as systemic lupus erythematosus. In addition,the following adverse reactions have been reported in marketed use: abnormal or uncontrolled muscle contractions, muscle spasm and an immune disease affecting muscles known as myasthenia gravis.
Do not take mineral supplements because they may block the absorption of Syprine.
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References: 1. Gaffney D, Fell GS, O'Reilly St. J. Wilson's disease: acute and presymptomatic laboratory diagnosis and monitoring. J Clin Pathol. 2000;53:807-12.
2. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Wilson’s Disease. J Hepatol. 2012;56:671-685.
3. Brewer GJ. Wilson's disease. In: Kasper DL, Fauci AS, Longo DL, eds. Harrison's Principles of Internal Medicine. New York. 16th ed. McGraw-Hill. 2005:2313-15.
4. http://www.syprine.com/pdfs/Syprine_PI.pdf; Syprine package insert. Lawrenceville, NJ Aton Pharma; 2007.